Articles de revues : « Survival – Miscellanea – Juvenile literature »

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TÖTEMEYER, ANDRÉE-JEANNE. « Desert Survival and Wilderness Adventures Juvenile Literature for a Young Namibian Nation ? » Matatu 17-18, n^o 1 (26 avril 1997) : 119–36. http://dx.doi.org/10.1163/18757421-90000220.

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Marschall, Elizabeth A., et Larry B. Crowder. « Density-dependent survival as a function of size in juvenile salmonids in streams ». Canadian Journal of Fisheries and Aquatic Sciences 52, n^o 1 (1 janvier 1995) : 136–40. http://dx.doi.org/10.1139/f95-013.

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The bioenergetic mechanism of density-dependent survival in juvenile stream salmonids was modeled using the relationship between density, food availability, and body size for a food-limited stream system. With a consumption-rate function estimated from laboratory experiments reported in the literature, we developed a function to predict maximum density of stream-resident juvenile salmonids of different sizes. This function can thus predict effective density based on both numbers and sizes of fish. Density predictions from this mechanistic function did not differ from predictions in the literature based on an empirically derived function describing the relationship between minimum territory size and body size in age-0 salmonids in streams, but it did suggest a bioenergetic mechanism for these relationships, linking size and consumption rate to the available food. Finally, we proposed a model of per capita survival rate as a function of the numbers and sizes in a cohort of stream-resident juvenile salmonids.

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Hemerik, Lia, et Chris Klok. « Conserving declining species using incomplete demographic information : what help can we expect from the use of matrix population models ? » Animal Biology 56, n^o 4 (2006) : 519–33. http://dx.doi.org/10.1163/157075606778967865.

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AbstractDue to human activity, many species have strongly declined in number and are currently threatened with extinction. Management directed at conservation of these species can benefit from the use of simple population models such as matrix models. However, for many species, data on survival and reproduction are scarce. Therefore, we set up a general framework based on a matrix model with three parameters: reproduction, juvenile (=first year survival) and adult survival in which incomplete data can be analysed. This framework is applicable to species that can mature after their first year of life. The point in a calendar year at which the population size is determined, i.e. the census time, is varied. We discuss the differences and the similarities between matrices modelling the same population at different census times. The population growth rate and the elasticity of the survival and reproduction parameters have been determined analytically. From these we made, as a visual diagnostic tool (general framework), plots of the growth rate and the elasticity pattern and their dependence on actual values of the reproduction parameter and the juvenile and adult survival. To illustrate the use of this framework we plot and discuss literature data on survival and/or reproduction of a few bird species with a juvenile stage of one year in the light of our modelling results.

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Kanda, L. Leann, et Todd K. Fuller. « Demographic responses of Virginia opossums to limitation at their northern boundary ». Canadian Journal of Zoology 82, n^o 7 (1 juillet 2004) : 1126–34. http://dx.doi.org/10.1139/z04-089.

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The precise response of a population at its distributional edge to the limiting extrinsic factor should be mediated by the demography of the species. We applied this principle to understanding the northern distributional potential of the Virginia opossum (Didelphis virginiana Kerr, 1792). We reviewed the literature for demographic data that we then used to build model populations. Juvenile over-winter survival was adjusted to determine the survival necessary for a stable population. To put the results in the context of life-history strategy and ecological niche, we built models for two other medium-sized mammals with similar distributions, the raccoon (Procyon lotor (L., 1758)) and the muskrat (Ondatra zibethicus (L., 1766)). Northern raccoon populations may sustain juvenile winter survival rates of <0.50 because adult females live to reproduce in multiple years. Muskrat juveniles may need a winter survival rate of only 0.40 in average years because reproduction is very high. In contrast, young northern opossums need a survival rate of 0.81 over winter to compensate for low prewinter survival. Raccoons and muskrats, through different life-history strategies, should be able to expand their northern distribution to the winter-induced physiological limit. However, opossum populations should fail before the average individual physiological limit is reached.

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Valkov, Iliya V., Mladen E. Ovcharov, Milan N. Mladenovski, Nikolay V. Vasilev et Iliya I. Duhlenski. « Radical Resection of Cerebellar Juvenile Pilocytic Astrocytoma - A 22-Year Survival : A Case Report ». Journal of Biomedical and Clinical Research 13, n^o 1 (1 septembre 2020) : 59–62. http://dx.doi.org/10.2478/jbcr-2020-0009.

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Summary Juvenile pilocytic astrocytoma (JPA) is a low-grade glioma, a most common astrocytoma in young patients. It is a tumor with relatively well-defined margins. Pilocytic astrocytomas (PA) comprise approximately 5-6% of all gliomas. Gross total resection ensures a radical cure of patients and long-time survival. In the literature, the data on the survival rate of more than 20 years is scarce. A 5.5-year old boy with a history of 3-month complaints of headache, dizziness, and vomiting was diagnosed after CT to harbour a hypodense cerebellar tumor mass, situated in the midline-right hemisphere. The compression of the fourth ventricle resulted in rostral hydrocephalus with transependymal resorption. Within a week, a VP shunt was applied, followed by a radical Nafziger-Town operation. Gross total resection of the tumor was achieved. Profound clinical improvement was observed immediately after the operation. Postoperative CT scans, including the ones 22 years after the operation, remained practically normal. The patient is now 28-year old and is a perspective economist now. He leads a healthy working life. In general, the prognosis is excellent. If the tumor is completely removed by surgery, the chances of being “cured” are very high. Pilocytic astrocytoma has a five-year survival rate in over 96 percent in children and young adults, which is one of the highest survival rates of any brain tumor. However, there is even a small percent possibility for malignant transformation (1-4%).

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Wallner, Kent E., Michael F. Gonzales, Michael S. B. Edwards, William M. Wara et Glenn E. Sheline. « Treatment results of juvenile pilocytic astrocytoma ». Journal of Neurosurgery 69, n^o 2 (août 1988) : 171–76. http://dx.doi.org/10.3171/jns.1988.69.2.0171.

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✓ Treatment results for 36 patients with juvenile pilocytic astrocytoma treated from 1942 through 1985 at the University of California, San Francisco, were reviewed. Twenty-two tumors were located in the posterior fossa, 10 were in the hypothalamic region, and four were in the cerebral hemispheres. Twenty-eight patients were less than 18 years of age. The overall survival rate was 83% and 70% at 10 and 20 years, respectively. All 12 patients who had total tumor resection remain disease-free; only two of the 12 received postoperative irradiation. The 10- and 20-year freedom-from-progression for the 19 patients who had incomplete resection and received at least 40 Gy of postoperative irradiation was 74% and 41%, respectively. All patients who failed treatment had local recurrence. One patient developed diffuse meningeal seeding, after four local recurrences in the posterior fossa over a 23-year period. Six patients failed treatment and had a repeat biopsy at the time of recurrence or at postmortem examination, and three showed histological progression of the tumor to an anaplastic astrocytoma. Based on this study and others in the literature, a protocol has been adopted whereby patients who have total tumor resection are not treated with postoperative irradiation. Patients who have incomplete tumor resection and are older than 3 years of age are currently treated with postoperative partial-brain irradiation, to a dose of 45 to 60 Gy. In general, young children with incomplete resection are followed closely with computerized tomography or magnetic resonance imaging and are treated with chemotherapy or irradiation if tumor progression is documented.

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Knutson, Melinda G., Randy K. Hines, Larkin A. Powell, Mary A. Friberg et Gerald J. Niemi. « An Assessment of Bird Habitat Quality Using Population Growth Rates ». Condor 108, n^o 2 (1 mai 2006) : 301–14. http://dx.doi.org/10.1093/condor/108.2.301.

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Abstract Survival and reproduction directly affect population growth rate (λ), making λ a fundamental parameter for assessing habitat quality. We used field data, literature review, and a computer simulation to predict annual productivity and λ for several species of landbirds breeding in floodplain and upland forests in the Midwestern United States. We monitored 1735 nests of 27 species; 760 nests were in the uplands and 975 were in the floodplain. Each type of forest habitat (upland and floodplain) was a source habitat for some species. Despite a relatively low proportion of regional forest cover, the majority of species had stable or increasing populations in all or some habitats, including six species of conservation concern. In our search for a simple analog for λ, we found that only adult apparent survival, juvenile survival, and annual productivity were correlated with λ; daily nest survival and relative abundance estimated from point counts were not. Survival and annual productivity are among the most costly demographic parameters to measure and there does not seem to be a low-cost alternative. In addition, our literature search revealed that the demographic parameters needed to model annual productivity and λ were unavailable for several species. More collective effort across North America is needed to fill the gaps in our knowledge of demographic parameters necessary to model both annual productivity and λ. Managers can use habitat-specific predictions of annual productivity to compare habitat quality among species and habitats for purposes of evaluating management plans.

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Lyubeznova, N. V. « Dynamics of development of juvenile plants in the mountain-tundra belt of the Khibiny Mountains ». Проблемы ботаники Южной Сибири и Монголии 22, n^o 1 (3 juillet 2023) : 208–12. http://dx.doi.org/10.14258/pbssm.2023040.

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Germination of seeds and survival of seedlings are important characteristics of the functioning of communities. There are few observations in the literature on the dynamics of seed germination in untouched alpine communities. Severe abiotic environmental factors characteristic of high mountains make it difficult for juvenile plants to take root, and they show high mortality in the first year of life. For 9 years (2011-2019), all juvenile plants were recorded on 5 m2 of the mountain-tundra community of the Khibiny Mountains and their further life was traced. For most plants of the alpine tundra community, low germination and high mortality of juvenile plants in the first year of life were confirmed. Seeds of some species of undisturbed plots, did not germinate during the observation period, most of the rest they did not survive. In species of slightly disturbed places, the number of seedlings was proportional to the number of species in the community, with the exception of species that do not have vegetative propagation. In the latter, seed germination was higher. A surge in the number of seedlings was observed in 2016 and 2019. The surviving individuals did not pass into the generative age state for 7-9 years of observations, which confirms the duration of the life cycle of alpine species.

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Carrasco-Rueda, Farah, et Raúl Bello. « Demographic dynamics of Peruvian black-faced spider monkeys (<i>Ateles chamek</i>) reintroduced in the Peruvian Amazon ». Neotropical Primates 25, n^o 1 (1 décembre 2019) : 1–10. http://dx.doi.org/10.62015/np.2019.v25.86.

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Reintroductions of animals are important conservation tools for different taxa around the world. A reintroduction program in the Peruvian Amazon is focusing on black-faced spider monkeys (Ateles chamek). We investigated life-history parameters such as stage-specific survival and female fertility rates using a capture-mark-recapture framework and data from the literature. We estimated growth rate and probability of extinction for a reintroduced group using matrix models, as well as testing whether population growth depends more on survival of juvenile females or adult females. Our results suggest the population of the reintroduced group is decreasing. After projecting the group size for the next 25 years using different scenarios, we found that in order for the group to persist, survival rate of the female adult stage needs to exceed 79 %. Given that group growth rate is more sensitive to the survival of adult females, management measures actions that target this demographic are required to guarantee survival of the group. Extrapolations of our results are subject to restrictions imposed by the small sample size and the conditions specific to this reintroduction program. However, this study may provide valuable lessons for reintroduction programs attempting the recovery of wild populations of similar species.

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Kalantri, Siddhesh Arun, Saikat Datta, Vijaykumar Shirure, Subham Bhattacharya et Maitreyee Bhattacharyya. « Azacytidine in Juvenile Myelomonocytic Leukaemia : A Single Centre Experience ». Blood 132, Supplement 1 (29 novembre 2018) : 5524. http://dx.doi.org/10.1182/blood-2018-99-112033.

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Abstract Background Juvenile myelomonocytic leukemia (JMML) is very rare and aggressive disease of infancy and childhood. This disorder is characterized by features of both myeloproliferative and myelodysplastic disorders. Allogeneic stem cell transplant is the only curative option available for this disease. The median survival time of children who do not receive an allograft can be as short as 10 to 12 months. Recent studies have highlighted the importance of epigenetic aberrations (aberrant DNA methylation) in JMML. Hence, azacytidine an epigenetic modifier can be good potential therapeutic option in these group of patients. There are very few cases reported in literature. Rarity of this disease makes prospective randomized trials with this agent difficult. Here we report favorable outcome for three patients diagnosed with this fatal condition. Case Series Three patients diagnosed JMML as per WHO 2016 Criteria since March 2017 were enrolled in this study after obtaining informed consent. Details of baseline patient characteristics are shown in table 1. All the patients received azacytidine at 75mg/m2/ day for seven consecutive days every 28 days. All the patients have refused option of allogenic stem cell transplant and hence they are planned to be kept on same protocol till progression. Two children are continuing treatment. First patient enrolled in this study has completed 13 cycles. Second patient discontinued treatment after completion of 5 cycles of azacytidine (parental preference). Third patient in this study has completed 9 cycles. Bone marrow aspiration and cytogenetic evaluation was planned after 6 cycles of azacytidine. Results All the children enrolled in this study are maintaining stable course and are free from transfusion requirement. The second patient who discontinued therapy after 5 cycles of azacytidine is also doing well as per telephonic confirmation with parents and is free from any transfusion requirement at 14 months since he was enrolled in study. At a median follow up of 13 months since the date of diagnosis, all three children are surviving (Range 10-16 months) which is already higher than median overall survival reported for patients not receiving stem cell transplant. No patient has shown evidence of clinical progressive disease as per response criteria of JMML International Symposium (December 2013). As per these criteria one patient had clinical complete remission, another patient had clinical partial remission with progressive genetic disease and the patient who lost to follow up is considered to have clinically stable disease (since we could not complete assessment for this patient). Table 2 summarizes response to treatment for different parameters assessed. We documented a new cytogenetic abnormality (appearance of 20 q deletion) in one patient which was not documented at baseline. Therapy was well tolerated and no major toxicities (grade III-IV) were documented except episode of febrile neutropenia in one patient requiring hospitalization. Other adverse events included thrombocytopenia in between cycles but none were grade III-IV after initial platelet response. Discussion and conclusion Though the sample size is small, results are encouraging. To note all patients in our study had platelet count less than 33 thousand and two patients had haemoglobin F percentage higher than that for age which are poor prognostic clinical variable as reported by EWOG-MDS study group. Our results are similar to those reported by EWOG MDS study group which comprised of nine treatment naive patients. Azacytidine was used for bridge to transplant in this group. However, in resource poor setting like India where most families cannot afford transplant it would be prudent to evaluate if azacytidine can prolong survival or alter the natural history of this fatal disease. Long term study with more number of patients are required to know whether azacytidine can be a suitable alternative in patients where stem cell transplant is not an option. Disclosures No relevant conflicts of interest to declare.

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Kasule, Sabirah N., Michael Apolinario, Christopher Saling, Janis E. Blair, Lisa Speiser et Holenarasipur R. Vikram. « 692. Coccidioides sp. Infective Endocarditis : A Review of the Literature ». Open Forum Infectious Diseases 8, Supplement_1 (1 novembre 2021) : S448. http://dx.doi.org/10.1093/ofid/ofab466.889.

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Abstract Background Despite the endemic nature of Coccidioides sp. to the American Southwest, the incidence Coccidioides sp. infective endocarditis (CIE) is rare. Following successful treatment of a patient with CIE at our institution, we reviewed the literature to identify trends in disease presentation, patient characteristics, and outcomes. Methods We reviewed all cases of CIE reported since 1938. Details including patient demographics, underlying immunodeficiency, time to diagnosis, treatment, and outcome were collected for analysis of diagnostic challenges and survival. Results Including ours, we identified 11 published cases of CIE. The majority (7) occurred in men. 5 patients were of either African American or Hispanic descent. Of the 10 patients with reported ages, the median age was 35.5 years (range 3 weeks – 61 years). 5 patients had a previous diagnosis of coccidioidomycosis and only 3 had an immunocompromising condition. These comprised pregnancy, heart transplant, and juvenile inflammatory arthritis. Three cases had multi-valvular involvement, but the majority affected the mitral (5) and the aortic (4) valves. Only 2 of the 11 cases involved a prosthetic valve. Of the 8 cases with reported blood cultures, only 2 were positive. Ten of the 11 cases had extra-cardiac disease. Complement fixation (CF) titers were heterogenous with a median of 1:32 and a range of 1:1 to 1:2048. There was no obvious correlation between a patient’s CF titer and their survival. Average time to diagnosis was 3.5 months (range 2.5 – 36 months). Diagnosis was made post-mortem in 4 of the 11 cases. 6 patients (54%) did not survive. Notably, 2 of the fatal cases preceded the discovery of amphotericin B (1969) and 4 occurred prior to the discovery of fluconazole (1990). Of the five patients that survived, four required surgical intervention in addition to azole therapy. Conclusion CIE is a diagnostic and therapeutic challenge. The diagnosis itself is rare, culture incubation times are long, and the symptoms are often non-specific thus delaying definitive therapy. The introduction of azole therapy appears to have had significant impact on rates of survival. Despite this, successful management of CIE still requires concurrent surgical intervention with aggressive, indefinite anti-fungal therapy. Disclosures All Authors: No reported disclosures

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Musselman, W. Chris, Thomas A. Worthington, Joshua Mouser, Desiree M. Williams et Shannon K. Brewer. « Passive Integrated Transponder Tags : Review of Studies on Warmwater Fishes With Notes on Additional Species ». Journal of Fish and Wildlife Management 8, n^o 2 (1 juin 2017) : 353–64. http://dx.doi.org/10.3996/122016-jfwm-091.

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Abstract Although numerous studies have assessed retention and survival of passive integrated transponder (PIT) tags, data are scattered and information gaps remain for many diminutive fishes. Our study objectives were to 1) systematically review PIT tag studies and summarize retention, growth, and survival data for warmwater fishes; and 2) conduct a laboratory study to evaluate the retention, survival, and growth effects of intracoelomic-placed, half duplex PIT tags on six small-bodied species common to warmwater streams. Our systematic review suggested small sample sizes were common within PIT tag retention and survival studies (39% with n ≤ 20) and that many experiments (15%, 14 of 97) failed to use control fish as part of their evaluations. Studies focused primarily on short-term changes (15 d to 2 y) in tag retention and survival. Tag retention was equal to or greater than 90% in 85% of the experiments reviewed and median survival was 92%. Growth was reported by fishes in the majority of reviewed studies. We found similar results after PIT tagging (peritoneum tagging using 12- or 23-mm half duplex tags) adult Cardinal Shiner Luxilus cardinalis, Central Stoneroller Campostoma annomalum, Greenside Darter Etheostoma blennioides, Orangethroat Darter Etheostoma spectabile, Slender Madtom Noturus exilis, and juvenile Smallmouth Bass Micropterus dolomieu. Tag retention for all species was high, with only one tag loss recorded after 60 d. Survival was also high (≥88%) for all of our species with the exception of Orangethroat Darter (56% survival). No significant difference in mean growth between treatment and control groups was found. Both our results and the findings of the literature review suggested generally high tag retention and low mortality in tagged fishes (across 31 species reviewed). However, within our study (e.g., Orangethroat Darter) and from the literature, examples of negative effects of PIT tagging on fishes were apparent, suggesting methodological testing is prudent before using PIT tags in field studies. We suggest future studies would benefit from addressing the behavioral implications that may be associated with tagging and examination of longer-term tag retention. Furthermore, standard reporting (i.e., sample sizes) in PIT tag studies would be beneficial, and use of control subjects or groups for statistical comparisons is needed.

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Erickson, Russell J., David R. Mount, Terry L. Highland, J. Russell Hockett, Edward N. Leonard, Vincent R. Mattson, Timothy D. Dawson et Kevin G. Lott. « Effects of copper, cadmium, lead, and arsenic in a live diet on juvenile fish growth ». Canadian Journal of Fisheries and Aquatic Sciences 67, n^o 11 (novembre 2010) : 1816–26. http://dx.doi.org/10.1139/f10-098.

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The effects of diet-borne copper, cadmium, lead, and arsenic on juvenile fish were evaluated using a live diet consisting of the oligochaete Lumbriculus variegatus . In 30 d exposures, no effects were observed on the growth and survival of rainbow trout ( Oncorhynchus mykiss ), fathead minnow ( Pimephales promelas ), and channel catfish ( Ictalurus punctatus ) fed diets contaminated with copper [130–310 µg Cu·(g dm)−1], cadmium [90–540 µg Cd·(g dm)−1], and lead [850–1000 µg Pb·(g dm)−1]. However, rainbow trout growth was reduced in a dose-dependent manner for diets contaminated with arsenic [26–77 µg As·(g dm)−1]. These effects of arsenic on fish growth were accompanied by slower feeding rate, reduced food conversion efficiency, liver cell abnormalities, and fecal matter changes suggestive of digestive effects, and occurred to a similar extent whether the diet was exposed to arsenate or arsenite. Effects from these dietary levels of arsenic, and the absence of effects from these dietary levels of metals, were generally consistent with literature reports using laboratory diets amended with toxicant salts. These results also indicated that reported growth effects on rainbow trout fed diets of invertebrates collected from mining-contaminated areas of the Clark Fork River (Montana, USA) or exposed in the laboratory to Clark Fork River sediments are likely more attributable to the arsenic than the metals in those diets.

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Pierini, Alessio, Maria Criscuolo, Roberta Caccamo, Enrico Bottero, Andrea Campanile, Guido Pisani, Veronica Marchetti et Elena Benvenuti. « A case series of urinary bladder rhabdomyosarcoma in seven dogs ». Open Veterinary Journal 13, n^o 11 (2023) : 1498. http://dx.doi.org/10.5455/ovj.2023.v13.i11.15.

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Background: Juvenile urinary bladder rhabdomyosarcoma is a known entity, however literature regarding its clinical behavior and endoscopic features is scarce. The aim of this study was to describe clinical, endoscopic features and outcome of urinary bladder rhabdomyosarcoma in dogs. Case Description: Dogs undergoing transurethral endoscopy and with a histological diagnosis of urinary bladder rhabdomyosarcoma were retrospectively collected. Seven dogs with a median age of 18 months (range 6-32 months) were included in this retrospective, multicenter, descriptive study. Median tumor size was 58 mm (range 30-65 mm), and tumor location was bladder neck in three cases, trigone in two cases and bladder body in two cases. Two dogs had monolateral ureteral obstruction. Two dogs presented with regional lymphadenopathy and one dog had lung lesions suggestive of metastatic disease. A grape-like mass was reported in four cases and solid in two, with variable consistency (two friables, two firms, two not reported). Tumor treatments included surgery in three cases, surgery and adjuvant doxorubicin in one case, and palliative therapy in three cases. Overall median survival time was 45 days. Survival times were shorter (range 20-45 days) for dogs treated with palliative care than for dogs treated with curative-intent treatment (range 70-120 days). Conclusion: Urinary bladder rhabdomyosarcoma should be considered as a differential diagnosis in young dogs presenting with bladder masses. In this study, urinary bladder rhabdomyosarcoma confirmed its aggressive clinical behavior. Surgery and chemotherapy seem to increase survival times but prognosis remains poor.

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Whooley, Maximillian, Beau Hsia, Darby Keirns, Andrew Luker, Peter T. Silberstein et Xinxin Wu. « Juvenile myelomonocytic leukemia : A national analysis of demographic features. » Journal of Clinical Oncology 41, n^o 16_suppl (1 juin 2023) : e19086-e19086. http://dx.doi.org/10.1200/jco.2023.41.16_suppl.e19086.

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e19086 Background: Juvenile Myelomonocytic Leukemia (JMML) is a rare hematological cancer originating in the bone marrow. It is a type of myelodysplastic syndrome (MDS) characterized by abnormal production of myeloid progenitor cells and monocytes. This malignancy is predominant in males, has a median age of diagnosis of 2 years, and carries a poor prognosis. It only accounts for 1% of all pediatric leukemias but constitutes 20-40% of pediatric MDS cases, making it the most common MDS subtype in children. Hematopoietic stem cell transplant (HSCT) is the only known cure for JMML, but its efficacy is just 50%. Without treatment, patients survive for a median of 10-12 months. Given the rarity of JMML, an analysis of demographic trends could offer valuable epidemiological insight. The National Cancer Database (NCDB) database was used to examine major demographic features of JMML. Methods: A retrospective analysis of all patients diagnosed with JMML between 2004 – 2020 in the NCDB (N = 242) was performed. Demographic factors including sex, race, Hispanic ethnicity, household income status, insurance status, urban/rural status, and Charlson-Deyo score were examined by descriptive statistics. Treatment statistics were also analyzed. Linear regression was used to determine incidence trends. Results: A total of 242 patients were identified with a histologically-confirmed diagnosis of JMML. Most patients were male (67.4%). The average age of diagnosis was 2 years (SD = 3.69, range = 0-33). The average incidence was 14.2 patients diagnosed per year (SD = 3.7, range = 9 – 21, R2 = 0.131). 93.8% had a Charlson-Deyo comorbidity score of 0. The majority of patients were White (74.4%), followed by Black (11.6%). 16.1% of patients were of Hispanic ethnicity. The distribution of median household income was 27.4% in the top quartile, 26.9% in the second, 26.9% in the third, and 18.7% in the fourth. Most patients were either privately insured (49.6%) or insured by Medicaid (42.6%). 75.5% of patients lived in a county with 250,000 people or more. Most patients received treatment (84.6%), while some only received active surveillance (8.7%) or no treatment (6.7%). Conclusions: This is the first NCDB analysis describing the demographic trends of JMML. Patients were twice as likely to be male than female. They were most likely to be White, with an average age of 2 years. This data reflects the expected age group and sex/race predominance described in the literature. The proportion of Hispanic JMML patients was comparable to Hispanics in the general US population. JMML appeared to be nearly evenly distributed among the top three household income quartiles. The lowest income quartile and rural inhabitants were least affected by this disease, which may indicate underdiagnosis in these populations. Further studies are needed to better define the relevance of these individual factors in the diagnosis, treatment, and survival of patients with JMML.

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Grassano, Maurizio, Giorgia Brodini, Giovanni De Marco, Federico Casale, Giuseppe Fuda, Paolina Salamone, Maura Brunetti et al. « Phenotype Analysis of Fused in Sarcoma Mutations in Amyotrophic Lateral Sclerosis ». Neurology Genetics 8, n^o 5 (12 septembre 2022) : e200011. http://dx.doi.org/10.1212/nxg.0000000000200011.

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Background and ObjectivesPathogenic variations in fused in sarcoma (FUS) are among the most common genetic causes of amyotrophic lateral sclerosis (ALS) worldwide. They are supposedly characterized by a homogeneous pure motor phenotype with early-onset and short disease duration. However, a few FUS-mutated cases with a very late disease onset and slow progression have been reported. To analyze genotype-phenotype correlations and identify the prognostic factors in FUS-ALS cases.MethodsWe identified and cross-sectionally analyzed 22 FUS-ALS patient histories from a single-center cohort of 2,615 genetically tested patients and reviewed 289 previously published FUS-ALS cases. Survival analysis was performed by Kaplan-Meier survival curves, followed by the log-rank test and multivariate Cox analysis.ResultsSurvival of FUS-ALS is age-dependent: In our cohort, early-onset cases had a rapid disease progression and short survival (p = 0.000003) while the outcome of FUS-mutated patients with mid-to-late onset did not differ from non–FUS-ALS patients (p = 0.437). Meta-analysis of literature data confirmed this trend (p = 0.00003). This survival pattern is not observed in other ALS-related genes in our series. We clustered FUS-ALS patients in 3 phenotypes: (1) axial ALS, with upper cervical and dropped-head onset in mid-to-late adulthood; (2) benign ALS, usually with a late-onset and slow disease progression; and (3) juvenile ALS, often with bulbar onset and preceded by learning disability or mild mental retardation. Those phenotypes arise from different mutations.DiscussionWe observed specific genotype-phenotype correlations of FUS-ALS and identified age at onset as the most critical prognostic factor. Our results demonstrated that FUS mutations underlie a specific subtype of ALS and enable a careful stratification of newly diagnosed FUS-ALS cases for clinical course and potential therapeutic windows. This will be crucial in the light of incoming gene-specific therapy.

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Syrovatka, N., I. Hrytsyniak et D. Syrovatka. « Use of hulless oats in feeding of pedigree 1+ carp as an effective method for ensuringthe physiological needs of fish ». Ribogospodarsʹka nauka Ukraïni., n^o 4(58) (22 décembre 2021) : 87–100. http://dx.doi.org/10.15407/fsu2021.04.087.

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Purpose. To study the effect of addition of hulless oats during the growing period on the qualitative and quantitative indicators of replacement-brood stock of carp. Methodology. The replacement-brood stock of Lubin intra-breed type of Ukrainian scaly carp strain, which were kept in ponds of State Enterprise Experimental Fish Farm of Lviv Research Station of the Institute of Fisheries of the National Academy of Agrarian Sciences of Ukraine (IFNAASU) was used for the study. The stocking density was 1000 fish/ha. The experiment was based on four experimental groups. Carps of the first experimental group were fed during the entire growing period with a feed mixture with the addition of hulles oats in the amount of 10 %, while carps of the second group were fed with a feed with the addition of 30 % of hulless oats. The third experimental group received a feed mixture in June – July, and hulless oats in August. The control group of carp was fed with the feed mixture throughout the entire growing period. The stocking density of fish was 1000 fish/ha. Piscicultural studies were conducted in accordance with current methods. Sampling of water for chemical analysis and its processing in the laboratory was carried out according to O. Alokin according to the Standard of the Ministry of Agrarian Policy of Ukraine. Haemoglobin content was determined by the haemoglobin-cyanide method. Blood erythrocytes were counted in a Goryaev chamber. Abundance and weight of natural feeds in ponds were determined according to I.A. Kyseliov, V.I. Zhadin. Zooplankton biomass was determined using tables of individual weights of F.D. Mordukhai-Boltovskoi. Analytical processing of the material was performed using MS Excel. The criteria for the analysis of indicators were their mean (M) and mean deviation (m). Findings. Peculiarities of growth, development of carp and efficiency of feed usage with the addition of hulless oats on the background of similar satisfactory growing conditions were studied. It was found that additional feeding of carp with hulless oats at an amount of 30% was effective. In particular, its addition resulted in an increase in individual weight by 31.1% and survival rate by 2.33%. Studies of the physiological functions of the body of experimental fish during the growing period after adding the hulless oats into the main diet provided an opportunity to recommend its use in the feeding process of age–1+carp in order to increase fish productivity. Originality. The advisability of using hulless oats in the feeding of replacement-brood stock of juvenile carp was studied in Ukraine for the first time. The analysis of productive, physiological and economic indicators has been carried out. Practical value. The results of a comprehensive assessment of piscicultura; and biological parameters provide recommendations for the use of hulless oats in the feeding of replacement-brood stock of juvenile carp. Key words: carp, replacement-brood juvenile stock, feed, feed additives, normalized feeding, physiological needs, fish productivity, feed costs, natural food supply.

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Tyndall, Alan. « Successes and Failures of Stem Cell Transplantation in Autoimmune Diseases ». Hematology 2011, n^o 1 (10 décembre 2011) : 280–84. http://dx.doi.org/10.1182/asheducation-2011.1.280.

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Abstract Over the past 15 years, more than 1500 patients have received HSCT, mostly autologous, as treatment for a severe autoimmune disease (AD). More than 1000 of these have been registered in the European Group for Bone Marrow Transplantation (EBMT) and European League Against Rheumatism (EULAR) combined database. A recent retrospective analysis of 900 patients showed that the majority had multiple sclerosis (MS; n = 345) followed by systemic sclerosis (SSc; n = 175), systemic lupus erythematosus (SLE; n = 85), rheumatoid arthritis (RA; n = 89), juvenile idiopathic arthritis (JIA; n = 65), and idiopathic cytopenic purpura (ITP; n = 37). An overall 85% 5-year survival and 43% progression-free survival was seen, with 100-day transplantation-related mortality (TRM) ranging between 1% (RA) and 11% (SLE and JIA). Approximately 30% of patients in all disease subgroups had a complete response, often durable despite full immune reconstitution. In many patients, such as in those with SSc, morphological improvement such as reduction of skin collagen and normalization of microvasculature was documented beyond any predicted known effects of intense immunosuppression alone. The high TRM was in part related to conditioning intensity, comorbidity, and age, but until the results of the 3 prospective randomized trials are known, an evidence-based modification of the conditioning regimen will not be possible.1 In recent years, multipotent mesenchymal stromal cells (MSCs) have been tested in various AD, exploiting their immune-modulating properties and apparent low acute toxicity. Despite encouraging small phase 1/2 studies, no positive data from randomized, prospective studies are as yet available in the peer-reviewed literature.

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Evseeva, N. V. « Characteristics of fucus algae cenopopulations near the Popov island of the White Sea ». Trudy VNIRO 192 (14 août 2023) : 26–38. http://dx.doi.org/10.36038/2307-3497-2023-192-26-38.

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The aim: Determination of the biological characteristics of fucus algae cenopopulations for the development of recommendations for their rational exploitation. Method: The original data obtained in 2020–2022 according to the recommended methods of fisheries research were used. Novelty: The size, age and sex structures of cenopopulations and reproduction of three species of fucus algaeof the intertidal zone of the White Sea are analyzed. Result: Studies in 2020–2022 in the intertidal zone of Popov Island confirmed the stable state of cenopopulations of commercial fucus algae. Fucus algae settlements in the littoral is a fishing area with dense thickets, an estimated specific stock of 630.3 t/km. Extended sporulation of F. vesiculosus in the area of Popov Island from June to the end of October allows the formation of several age generations of seedlings. Together with vegetative reproduction, this significantly increases the survival of the cenopopulation and its resistance to climatic influences. The age of fertility in the studied areas is less than according to the literature data. In terms of resistance to environmental impact and survival, the dominant species can be put in the following order based on the characteristics of reproduction, area and density of juvenile settlements, and abundance in the intertidal zone: Fucus vesiculosus → Ascophyllum nodosum → Fucus distichus. Practical significance: Based on the data on the reproduction of commercial fucus algae near the Karelian coast of the Onega Bay, it is recommended to start harvesting algae in the littoral no earlier than July 1 after the end of the generative period in the most vulnerable species.

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Lee, Sangwoo, Cheolmin Kim, Xiaoshan Liu, Saeram Lee, Younglim Kho, Woo-Keun Kim, Pilje Kim et Kyungho Choi. « Ecological Risk Assessment of Amoxicillin, Enrofloxacin, and Neomycin : Are Their Current Levels in the Freshwater Environment Safe ? » Toxics 9, n^o 8 (23 août 2021) : 196. http://dx.doi.org/10.3390/toxics9080196.

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Veterinary pharmaceuticals may cause unexpected adverse effects on non-target aquatic species. While these pharmaceuticals were previously identified as priority compounds in ambient water, their ecological risks are relatively unknown. In this study, a series of chronic toxicity tests were conducted for these pharmaceuticals using algae, two cladocerans, and a fish. After a 21-d exposure to amoxicillin, enrofloxacin, and neomycin, no observed effect concentration (NOEC) for the reproduction of Daphnia magna was detected at 27.2, 3.3, and 0.15 mg/L, respectively. For the survival of juvenile Oryzias latipes following the 40-d exposure, NOEC was found at 21.8, 3.2, and 0.87 mg/L, respectively. Based on the results of the chronic toxicity tests and those reported in the literature, predicted no-effect concentrations (PNECs) were determined at 0.078, 4.9, and 3.0 µg/L for amoxicillin, enrofloxacin, and neomycin, respectively. Their hazard quotients (HQs) were less than 1 at their average levels of occurrence in ambient freshwater. However, HQs based on the maximum detected levels of amoxicillin and enrofloxacin were determined at 21.2 and 6.1, respectively, suggesting potential ecological risks. As the potential ecological risks of these veterinary pharmaceuticals at heavily contaminated sites cannot be ignored, hotspot delineation and its management are required.

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Manubens, Joan, Oriol Comas, Núria Valls et Lluís Benejam. « First Captive Breeding Program for the Endangered Pyrenean Sculpin (Cottus hispaniolensis Bacescu-Master, 1964) ». Water 12, n^o 11 (24 octobre 2020) : 2986. http://dx.doi.org/10.3390/w12112986.

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The strong decline of freshwater fish species in Europe implies that further ex-situ conservation plans should be implemented in the near future. The present study reflects our experience with the Pyrenean sculpin (Cottus hispaniolensis Bacescu-Mester, 1964)—a small cottid endemic to the Hispano-French Garona River basin. In recent years, the Spanish Pyrenean sculpin population has reached a limit situation. Because of that, the non-profit association ADEFFA—with support from the public administration—started the first captive breeding program for this species in 2006. Fourteen years later, this study presents the results and evaluates the different steps of the program, with the aim of discussing and improving the ex-situ conservation plans for this and other cold freshwater species. There is a description and a comparison between six consecutive phases during the captive breeding process: nesting behaviour, courtship, egg fixation, parental care (incubation), hatching and survival during juvenile development. The purposes of this project are to: (1) identify the most determining phases for a successful captive breeding; (2) identify the factors that had a major influence to the success of the critical phases; and (3) increase the number of the offspring. This study is based on thirty-three wild individuals collected from Garona River (Val d’Aran, Spanish Pyrenees). During the program, twelve couples spawned in captive conditions, with around 2300 eggs laid. Eight couples bred successfully, with 751 hatched individuals and 608 juveniles reared. The analysis of each step of the captive breeding does not reveal significant differences between phases, so it can be concluded that they are all critical at the same level. In the literature, similar study-cases of captive breeding programs identify incubation and survival phases as the most critical. Consequently, the management made for this project has probably allowed to overcome in part the main impediments described in other similar programs.

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Kozlova, O. P., M. M. Kostik, M. D. Kuznetsova, M. F. Dubko, L. S. Snegireva, A. L. Shavkin, E. A. Ligostaeva et al. « Invasive fungal infections in children with rheumatic diseases ». Journal Infectology 12, n^o 5 (21 janvier 2021) : 48–55. http://dx.doi.org/10.22625/2072-6732-2020-12-5-48-55.

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Introduction. In children with rheumatic diseases, severe fungal infections (invasive mycoses – IM) are not well understood.Objectives. To analyze risk factors, disease course of IM in children with systemic rheumatic diseases.Materials and methods. For diagnosis of IM were used criteria EORTC/MSGERC, 2019. We reviewed the literature over the past 15 years on IM in children with rheumatic diseases from the international databases Pubmed and Web of Science.Results. In retrospective multicenter study were included 8 children with IM and systemic rheumatic diseases: ANCA-associated vasculitis (n=4), systemic lupus erythematosus (n=3), juvenile rheumatoid arthritis (n=1). Median age was 13,5 (8-17) y., boys – 67%. Invasive aspergillosis was diagnosed in 5 patients and invasive candidiasis – 3. The risk factors of invasive mycoses were high rheumatic disease activity (100%), corticosteroids (prednisolone ≥ 0,3 mg/kg/d) use for ≥21 d (87,5%), immunosuppressive therapy (87,5%), recent (≤ 2 weeks) pulse steroid therapy (75%), hemophagocytic lymphohistiocytosis (62,5%), prolonged (≥ 10 days) severe neutropenia (≤ 0,5х109/l) (62,5%), and prolonged (≥10 days lymphopenia (≤ 1,0х109/l) (37,5%). In patients with invasive aspergillosis the involved organ was the lung, in patients with invasive candidiasis a candidemia was diagnoses. All patients received antifungal therapy. The overall 30 days survival rate was 37,5%.Сonclusions. Children with high rheumatic diseases activity and intensive treatment with immunosuppressive agents should be considered as patients with a high risk of invasive mycoses with a high mortality.

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d’Angelo, Debora Mariarita, Marina Attanasi, Giulia Di Donato, Giuseppe Lapergola, Mariarosaria Flacco, Francesco Chiarelli, Emma Altobelli et Luciana Breda. « The Role of Serum Calprotectin in Defining Disease Outcomes in Non-Systemic Juvenile Idiopathic Arthritis : A Pilot Study ». International Journal of Molecular Sciences 24, n^o 2 (14 janvier 2023) : 1671. http://dx.doi.org/10.3390/ijms24021671.

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Serum calprotectin (MRP8/14) is currently being studied as a promising biomarker of disease activity and outcome in patients with juvenile idiopathic arthritis (JIA) but the data in the literature are conflicting. The aim of our study was to investigate the potential role of serum calprotectin as biomarker of disease activity and flare/remission in a group of nsJIA patients during a follow-up period of 18 months. In this prospective longitudinal study, two groups of patients with ns-JIA (55 active patients and 56 patients in remission according to Wallace’s criteria) and a control group (50 children) were recruited at baseline from January 2020 to September 2021. JIA patients were followed for up to 18 months at four timepoints: 3 months (T1), 6 months (T2), 12 months (T3) and 18 months (T4). At each timepoint, the following were recorded: JADAS27, blood counts, ESR, CRP, albumin, ferritin and serum calprotectin. To illustrate the performance of calprotectin, Kaplan–Meier curves were constructed from baseline to relapse/remission, dichotomizing patients at baseline in positive/negative on the basis progressive calprotectin cut-offs. Associations between baseline factors and relapse were determined using Cox regression models. Multivariate models were constructed to analyze the effect of covariates. Comparing baseline clinical and laboratory data of the three groups (active vs. inactive JIA vs. controls), only serum calprotectin reached statistical significance (active patients vs. inactive (p = 0.0016) and vs. controls (p = 0.0012)). In the inactive group, during the 18 months of follow up, 31 patients (55.3%) had a relapse. Comparing the baseline data of relapsers vs. non-relapsers, serum calprotectin showed higher levels (p = 0.001) in relapsers. In survival analysis, a log rank test showed significant differences of up to 12 ng/mL (p = 0.045). Multivariate Cox regression confirmed that only baseline calprotectin levels were independently associated with disease recurrence. In the active group, in the 12 months of follow-up, 19 patients (38%) entered remission of the disease. In addition, in this group, the only statistical difference at the baseline was the value of MPR8/14 (p = 0.0001). Log rank test showed significant differences up to 10 ng/mL (p = 0.003). In the multivariate Cox regression, serum calprotectin levels at baseline were independently associated with remission. In conclusion, our study would suggest a dual role for calprotectin in predicting future relapse and treatment response in patients with nsJIA, thus influencing therapeutic decisions and management of these patients during follow up.

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Cuevas, Octavio Aragon. « P022 Anakinra for use in non- juvenile idiopathic arthritis (JIA) related haemophagocytic lymphohistiocytosis (HLH) : evidence base and funding ». Archives of Disease in Childhood 104, n^o 7 (19 juin 2019) : e2.26-e2. http://dx.doi.org/10.1136/archdischild-2019-nppc.32.

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BackgroundNon JIA related HLH is a life-threatening complication that is increasingly recognised in paediatric patients, particularly in those who are unwell in the paediatric intensive care unit (PICU). Untreated or insufficiently treated HLH has a significant mortality rate (up to 53%).1AimTo review the evidence base for the use of anakinra in paediatric patients with non-JIA HLH refractory to systemic corticosteroids in patients who are not fit for treatment as per HLH 2004 protocol.MethodsA PubMed search with words ‘anakinra’ and ‘hemophagocytic lymphohistiocytosis’ was carried out on July 2018 to find out the evidence base with regards to the use of anakinra in non-JIA related HLH. Any published peer reviewed clinical studies or trials (including but not limited to retrospective or prospective controlled trials, comparative studies and observational/cohort studies) were considered. Case reports and series were considered if better evidence studies were not available. A recent case study from a tertiary paediatric centre will be used to illustrate the pathway followed to diagnose non-JIA related HLH and funding options.ResultsAlthough a protocol exists for primary HLH treatment (HLH 2004), including chemotherapy and stem cell transplantation,2 there is no consensus on how to treat secondary HLH. The literature mainly showed case reports and small case series,3 describing the use of anakinra collectively for 35 patients (median age 14 to 48 years) who met the HLH 2004 diagnostic criteria with an overall survival rate of up to 88% at time of discharge from the PICU3. Anakinra was used at standard doses always in combination with corticosteroids. Some patients also received intravenous immunoglobulin (IVIG) and ciclosporin at the discretion of the medical teams.ConclusionThe evidence for use of anakinra in non JIA secondary HLH is limited to retrospective observational studies and mostly restricted to adult populations. Despite this caveat, these studies have demonstrated that anakinra therapy alongside other non-etoposide immunomodulatory therapies is associated with an improvement in short term survival. In patients with multi-organ dysfunction, who are too unstable to receive the existing etoposide based HLH-2004 treatment regimen due to concerns regarding significant treatment toxicity, personalised non-etoposide therapies including dexamethasone, IVIG, ciclosporin and anakinra may be better tolerated and provide a bridge to future more standardised treatment. Evidence to date shows that relapse of secondary HLH is possible with ciclosporin therapy. In JIA related HLH, anakinra was considered better than ciclosporin at inducing remission and having a lower incidence of adverse effects,4 and NHS England granted funding for the treatment based on these findings. The available evidence did not show any serious adverse events related to anakinra.RecommendationsThis tertiary centre approved the use of anakinra for this patient and future patients with this indication despite lack of reimbursement from NHS England for the drug. An urgent interim policy review will be put together by a team of the British Society of Paediatric and Adolescent Rheumatology (BSPAR) and presented to the NHS England commissioners to seek funding for anakinra for paediatric patients with this indicationReferencesMiettunen, et al. Successful treatment of severe paediatric rheumatic disease-associated macrophage activation syndrome with interleukin-1 inhibition following conventional immunosuppressive therapy: case series with 12 patients. Rheumatology (Oxford) 2011;50:417–9Henter JI, Horne A, Arico M, Egeler RM, Filipovich AH, Imashuku S, et al. HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer 2007;48:124–31.Kumar B, Aleem S, Saleh H, Petts J, Ballas ZK. A Personalized Diagnostic and Treatment Approach for Macrophage Activation Syndrome and Secondary Hemophagocytic Lymphohistiocytosis in Adults. J Clin Immunol 2017;37:638–643Boom V, et al. Evidence-based diagnosis and treatment of macrophage activation syndrome in systemic juvenile idiopathic arthritis. Pediatr Rheumatol Online J 2015;13:55.

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Morantz, Robert A. « Radiation Therapy in the Treatment of Cerebral Astrocytoma ». Neurosurgery 20, n^o 6 (1 juin 1987) : 975–82. http://dx.doi.org/10.1227/00006123-198706000-00028.

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Abstract With the advent of more sensitive diagnostic techniques, we are encountering an increasing number of young patients harboring cerebral astrocytoma. The great danger in such patients is that the astrocytoma cells will undergo dedifferentiation to a higher state of malignancy. An essential question is whether the use of postoperative adjuvant radiation therapy can decrease the incidence of this event. Because a prospective, randomized study has never been carried out, it is extremely difficult to ascertain whether radiation therapy should be given to these patients. This article reviews the main retrospective clinical studies in an attempt to determine whether the addition of radiation therapy increases the length or quality of survival in patients with astrocytoma. Based on this literature review, the following tentative conclusions have been reached: (a) All reported studies are inconclusive; therefore, dogmatic statements as to whether radiation therapy should be used are not warranted. (b) One should try to obtain pathological confirmation of the precise nature of all tumor-like cerebral lesions that have been detected on neuroradiological studies. (c) Consistent with sound neurosurgical judgment, every attempt should be made to carry out a gross total removal of the hemispheric astrocytoma. (d) In the case of such a gross total surgical removal and even in its absence in the case of a juvenile pilocytic astrocytoma, radiation therapy may be withheld and the patient carefully followed for tumor recurrence. (e) In those cases where total removal cannot be accomplished, postoperative radiation therapy seems warranted. (f) Such radiation therapy should be given in a conventional fractionated schedule to a maximum of 5500 rads. The radiation therapy should not be given to the whole brain, but rather to the tumor plus a limited surrounding margin as determined by computed tomography/magnetic resonance imaging. Such a treatment regimen may reasonably be expected to lead to a 5-year survival rate of approximately 40%.

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Keerty, Dinesh, Sindhu Priya Devarashetty et Philip A. Haddad. « The impact of adjuvant platinum-based chemotherapy in early adult granulosa cell tumor of the ovaries : A meta-analysis of comparative studies. » Journal of Clinical Oncology 41, n^o 16_suppl (1 juin 2023) : 5547. http://dx.doi.org/10.1200/jco.2023.41.16_suppl.5547.

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5547 Background: Adult granulosa cell tumors (AGCT) are the most common type of malignant ovarian sex chord stromal tumors but only comprise 2 to 5 percent of all malignant ovarian neoplasms. Most granulosa cell tumors have an indolent growth pattern, and prognosis depends on the disease stage at diagnosis and the presence of residual disease after surgery. Though these tumors have metastatic potential, their tendency for late relapses is well documented. Despite the lack of supporting data, the National Cancer Centers Network recommends platinum-based adjuvant chemotherapy (AC) for Stage I with intermediate and high-risk features. Most studies have failed to show any benefit of AC with disease-free or overall survival. However, several of these studies were small, and some had short median follow-ups. Others included advanced stages and the juvenile variant of the disease. Therefore, we conducted this meta-analysis to evaluate the impact of AC on disease recurrence in a stage I enriched AGCT. Methods: A review of the medical literature was conducted using online databases. Inclusion criteria consisted of English language, diagnosis of AGCT, studies with a preponderance of stage I, comparative studies of AC versus none, and studies that reported recurrence rates. Studies that had a preponderance of advanced stages or juvenile variants were excluded. A meta-analysis using the fixed effects and random effects models was conducted. Results: Seven retrospective comparative studies with a total of 500 patients were included. The average median age was 47 years, and the average median follow-up was 58 months. Approximately 79% of the sample were stage I, and 79% of stage I were IC. Most AC regimens used were BEP and EP, though it varied among the regions. Platinum-based AC in early-stage AGCT failed to impact recurrence rates compared to clinical observation (HR=1.39, 95%CI 0.86-2.25, I2=48%, p=0.18). Conclusions: This is the first meta-analysis to show that adding platinum-based adjuvant chemotherapy to surgery does not improve the recurrence rate in early AGCT. Therefore, in the absence of evidence supporting any benefit of AC in this disease, recommendations to use AC should be re-evaluated, especially since the risk of platinum-based AC with or without Bleomycin is well-documented and carry potentially serious side effects.

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Nevmerzhytska, N. M., et V. V. Orzheshkovskyi. « Fahr’s disease ». Likarska sprava, n^o 1-2 (26 mars 2019) : 35–42. http://dx.doi.org/10.31640/jvd.1-2.2019(6).

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The scientific review based on an analysis of the literature examines key points in the etiology, pathomorphology and clinical picture of basal ganglia calcification. It also involves the so-called physiological calcification of the central nervous system. Juvenile and senile forms of a disease and frequency of occurrence of this nosological form are described. The historical information and modes of inheritance are briefly provided. The article considers the numerous synonyms of this disease and the causes of secondary calcification of the brain (Fahr’s syndrome). Four genes are described associated with primary calcification of the basal ganglia: SLC20A2 and XPR1 coding transmembrane conveyors of inorganic phosphate; PDGFB and PDGFRB which are involved in integrity of a blood-brain barrier and survival of pericytes. Pathogenetic mechanisms of clinical displays of a disease are presented. The article displays the features of macro- and microscopic changes in the brain with this nosology. The characteristic signs of the initial and advanced forms of the disease are described in detail, taking into account the age of the debut of calcification of the basal ganglia. The main and auxiliary instrumental methods for diagnosing this disease are also considered, the results of positron emission tomography and magnetic resonance spectroscopy are described, which confirm the pathophysiological mechanism of neurological manifestations of the disease associated with the disorganization of the front-striatal pathways in the area of calcified basal ganglia. A number of additional general clinical laboratory and functional studies are listed to confirm or exclude the diagnosis of primary family idiopathic ferrocalcinosis (Fahr’s diseases). The main directions in the treatment of the described pathology are given.

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Polishchuk, O. « Results of pikeperch (Sander lucioperca Linnaeus, 1758) cultivation with pool technology ». Ribogospodarsʹka nauka Ukraïni., n^o 4(62) (30 décembre 2022) : 55–72. http://dx.doi.org/10.15407/fsu2022.04.055.

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Purpose.To investigate the peculiarities of pikeperch fingerlings (Sander lucioperca Linnaeus, 1758) cultivated with the use of pool technology and different stoking densities, also to provide a comprehensive fishery-biological assessment of the produced fish. Methodology. The material for the study were juvenile and young-of-the-year pikeperch. They were cultivated in 17.3 m3 pools (in the first series of the experiment) and 2.16 m3 pools (in the second series of the experiment). According to the physiological needs of pikeperch at the studied stage of ontogenesis, its diet consisted of salt brine shrimp (Artemia salina Linnaeus, 1758) nauplii and combined feed. They were fed according to a scheme with a sequential change of 3 stages: 1 – feeding exclusively with brine shrimp nauplii; 2 – combined feeding (brine shrimp nauplii and combined feed were mixed); 3 – feeding exclusively with combined feed. Also, 3 options of stocking density were used at each of the above-mentioned growing stages. Namely: at the 1st stage ‒ 40, 60 and 80 thousand individuals/m3; in the II stage ‒ 10, 15 and 20 thousand individuals/m3, and in the III stage ‒ 5, 7, 10 thousand individuals/m3. During the entire growing process, according to the schedule, test catches were carried out, during which weighing was carried out, based on the results of which absolute and relative weight gains were calculated. Statistical processing of the received data was carried out using a package of standard Microsoft Office software. Findings. The study established that the highest growth rates were found in fish that were grown at the stocking density: at first 40 thousand individuals/m3 (I stage), and then 10 thousand individuals/m3 (II stage) and 5 (III stage) individuals/m3 after that — experimental group No 1. The individual weights in the fish from this experimental group were 1.7–1.8 g at the first stage of rearing, and at the second stage of rearing, the intensity of weight accumulation in them increased almost twice (2.4–2.2 g). The survival rate of fish from the experimental group No 1 was 37.5–41.0% at the I stage of rearing and 74.0–78.0% at the II stage. Under such scheme (feeding and stocking density as in experimental group No 1) of pikeperch juveniles and young-of-the-year cultivation in pools, the fish productivity of the latter ranged from 21 to 35.8 kg/m3 (depending on the initial size of the pools for rearing). Originality.For the first time in the conditions of Ukraine, a comparative analysis of young-of-the-pikeperch cultivation using intensive technology was carried out, and practical recommendations were given to improve the existing technology. Practical Value.The use of the pool method of young-of-the-pikeperch cultivation allows expanding the existing technologies of aquaculture for this fish species and increase the market of its additional products. Keywords: pikeperch (Sander lucioperca Linnaeus, 1758), juveniles, fingerlings, pool technology of aquaculture, planting densities, weight, survival.

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Edea, Géoffroy Olayèmi, Lambert Cloud Hinvi, Youssouf Abou et Armand Bienvenu Gbangboche. « Growth and Body Development of Oreochromis niloticus (Linnaeus, 1758) Fattened in Floating Cages Based on Commercial Feed in Benin ». Journal of Biology and Life Science 11, n^o 1 (25 septembre 2019) : 36. http://dx.doi.org/10.5296/jbls.v11i1.15259.

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This work evaluates the growth and body development of Oreochromis niloticus in floating cages in the Toho Lake of Benin. Thus, 6000 juvenile monosex male with an average initial weight of 8.87 ± 4.89 g and average initial total length of 7.87 ± 1.43 cm were randomly distributed in two floating cages (5 x 5 x 2.5 m3) at the stocking density of 3000 fish/cage. The fish were hand-fed to apparent satiation, three times daily, using 45-32% crude protein commercial pelleted floating feed Skretting®. The physico-chemical parameters of lake water recorded every 72 hours during the experiment were within the suitable ranges for fish culture and were as follows: temperature (27.78 ± 0.41 °C), pH (7.55 ± 0.22), dissolved oxygen (4.03 ± 0.96 mg/l), ammonium (0.31 ± 0.18 mg/l), nitrite (0.29 ± 0.07 mg/l) and nitrate (0.27 ± 0.12 mg/l). The variables studied at the end of the 215 days of rearing were as follows: final mean total length (26.61 ± 2.99 cm), final mean standard length (22.40 ± 2.74 cm), final mean predorsal length (6.93 ± 0.94 cm), final mean head length (3.45 ± 0.58 cm), final mean dorsal fin base length (13.55 ± 2.96 cm), final mean inter-orbital width (2.97 ± 0.37 cm), final mean body height (8.57 ± 1.56 cm) and final mean caudal peduncle height (3.27 ± 0.39 cm). The zootechnical growth parameters evaluated were as follows: survival rate (91.5%), final mean body weight (402.18 ± 137.05 g), average daily weight gain (1.83 ± 0.08 g), specific growth rate (0.77 ± 0.03%/day), feed conversion ratio (1.74 ± 0.09%) and protein efficiency ratio (1.62 ± 0.06). These results compared to the literature indicate interesting growth and body development and it would be important to promote in-cage farming of Oreochromis niloticus.

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KURAKBAYEV, Ye, B. TURDALIYEVA, L. MANZHUOVA et V. SCHUKIN. « RISK FACTORS AND EARLY SIGNS OF CRITICAL CONDITIONS IN CHILDREN WITH ACUTE LYMPHOBLASTIC LEUKEMIA ADMITTED TO THE INTENSIVE CARE UNIT ». Oncologia i radiologia Kazakhstana 69, n^o 3 (10 octobre 2023) : 38–46. http://dx.doi.org/10.52532/2663-4864-2023-3-69-38-46.

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Relevance: Acute lymphoblastic leukemia (ALL) is the most common cancer among children, accounting for nearly a quarter of all childhood cancers. The study aimed to determine the risk factors and signs of critical conditions in children with acute lymphoblastic leukemia admitted to an intensive care unit (ICU). Methods: The approach used was a systematic review. Data was collected from sources published in 2019-2023. Four cohort studies, four retrospective analyses, two literature reviews, one case-control study, and one case study were included in this systematic review. Results: The prognosis in pediatric ALL depends on the initial number of blast cells in the peripheral blood. Patients with B-cell precursor acute lymphoblastic leukemia (BCP ALL) and low blast cell numbers survived better than patients with T-cell acute lymphoblastic leukemia (T-ALL) and low cell count. IL1B and NLRP1 genetic polymorphisms enhanced ALL risk and reduced infectious comorbidity. However, these gene polymorphisms must be confirmed in juvenile leukemia. KRAS, FLT3, NRAS, PTPN11, KMT2D, PTEN, and NOTCH1 gene mutations affected pediatric ALL patient features and treatment results. These mutations demonstrate the relevance of genetic profiling in risk classification and tailored management. Gene variations and availability of effective medication contributed. Pediatric BCP-ALL patients with the PAX5P80R mutation had worse 5-year overall survival, higher white blood cell counts, male preponderance, and more genetic abnormalities. Pediatric BCP-ALL focused on genetic analysis and risk stratification. Children of African American and European American ancestry showed varied incidence, recurrence, and outcome rates for ALL. African American children exhibited lower incidence but greater recurrence rates and poorer prognosis than European American children. Conclusion: Risk factors for these patients’ admission to ICU include comorbidities, infectious diseases, hypoxia, and hemodynamic instability, as well as age and baseline white blood cell count at diagnosis.

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KURAKBAYEV, Ye, B. TURDALIYEVA, L. MANZHUOVA et V. SCHUKIN. « RISK FACTORS AND EARLY SIGNS OF CRITICAL ; CONDITIONS IN CHILDREN WITH ACUTE ; LYMPHOBLASTIC LEUKEMIA ADMITTED ; TO THE INTENSIVE CARE UNIT ». Oncologia i radiologia Kazakhstana 69, n^o 3 (30 septembre 2023) : 38–46. http://dx.doi.org/10.52532/2521-6414-2023-3-69-38-46.

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Relevance: Acute lymphoblastic leukemia (ALL) is the most common cancer among children, accounting for nearly a quarter of all childhood cancers. The study aimed to determine the risk factors and signs of critical conditions in children with acute lymphoblastic leukemia admitted to an intensive care unit (ICU). Methods: The approach used was a systematic review. Data was collected from sources published in 2019-2023. Four cohort studies, four retrospective analyses, two literature reviews, one case-control study, and one case study were included in this systematic review. Results: The prognosis in pediatric ALL depends on the initial number of blast cells in the peripheral blood. Patients with B-cell precursor acute lymphoblastic leukemia (BCP ALL) and low blast cell numbers survived better than patients with T-cell acute lymphoblastic leukemia (T-ALL) and low cell count. IL1B and NLRP1 genetic polymorphisms enhanced ALL risk and reduced infectious comorbidity. However, these gene polymorphisms must be confirmed in juvenile leukemia. KRAS, FLT3, NRAS, PTPN11, KMT2D, PTEN, and NOTCH1 gene mutations affected pediatric ALL patient features and treatment results. These mutations demonstrate the relevance of genetic profiling in risk classification and tailored management. Gene variations and availability of effective medication contributed. Pediatric BCP-ALL patients with the PAX5P80R mutation had worse 5-year overall survival, higher white blood cell counts, male preponderance, and more genetic abnormalities. Pediatric BCP-ALL focused on genetic analysis and risk stratification. Children of African American and European American ancestry showed varied incidence, recurrence, and outcome rates for ALL. African American children exhibited lower incidence but greater recurrence rates and poorer prognosis than European American children. Conclusion: Risk factors for these patients’ admission to ICU include comorbidities, infectious diseases, hypoxia, and hemodynamic instability, as well as age and baseline white blood cell count at diagnosis.

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Nizyaev, S. A. « Ecological aspects of the perennial distribution of the Red King Crab Paralithodes camtschaticus in Aniva Bay (Sakhalin Island) ». Geosystems of Transition Zones 6, n^o 4 (2022) : 388–404. http://dx.doi.org/10.30730/gtrz.2022.6.4.388-404.

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Regularity of localization and distribution of clusters of commercial individuals of the Red King Crab Paralithodes camtschaticus in Aniva Bay (Sakhalin Island) and possible reasons for the transformation of the range of this species in the bay are considered in the interannual aspect. The prospects of restoring this species in the bay have been assessed, the changes in habitat conditions being taken into account. Available reporting (SakhNIRO) and literature data on trawl (trap) catches of the Red King Crab in this area are analyzed and summarized in order to solve these problems. The analysis of the location of the main concentrations of larvae during the period of the highest number of the object is performed. The functional structure of the Red King Crab settlements in Aniva Bay is analyzed according to literary sources, and an assessment of possible problems in the implementation of the reproductive strategy proper to the Red King Crab by the population is carried out. Significant negative changes in the distribution and functional structure of this crab species in Aniva Bay are noted. It is shown that the aggregations of commercial individuals of the Red King Crab have been widespread in both the western and eastern parts of Aniva Bay until 2001. Both parts of the bay were self-sufficient in terms of reproduction, with each showing the signs of juvenile production processes. First of all, this can be seen by the distribution of the places of catching of non-migratory juveniles and larvae. From 2004 to the present, even single catches of the Red King Crab have disappeared in the eastern part of Aniva Bay. At the same time, the dynamics of its reserve recovery is observed in the west of the bay, although in general this process is noticeably slow. We believe that the reason for the deterioration of living conditions and reproduction of the Red King Crab in Aniva Bay is a critical transformation of substrates in the eastern part of the bay, namely, a sharp reduction in the area of soils suitable for the development of epifauna, the presence of which is one of the conditions for the survival of crab juveniles. This transformation became possible due to a significant expansion (up to 20 %) of silty soils in the bay caused by soil dumping in 2003–2006 during the construction of a liquefied natural gas plant.

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Vencovský, J., K. Pisarczyk, R. Leff, E. Park, K. Palaniswamy et L. Long. « AB1277 POOR LONG-TERM OUTCOMES AND SUBSTANTIAL BURDEN OF COMORBIDITIES IN EUROPEAN PATIENTS WITH DERMATOMYOSITIS/POLYMYOSITIS : RESULTS FROM A SYSTEMATIC LITERATURE REVIEW ». Annals of the Rheumatic Diseases 81, Suppl 1 (23 mai 2022) : 1746.2–1746. http://dx.doi.org/10.1136/annrheumdis-2022-eular.756.

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BackgroundDermatomyositis (DM) and polymyositis (PM) are rare heterogenous systemic autoimmune disorders of the skin, muscles and other organs that may have a devastating impact on patients’ lives. Despite various therapies used in clinical practice, a notable proportion of patients seem not to achieve sustainable remission. There is a need to better understand long-term outcomes and comorbidities in patients with DM/PM in Europe.ObjectivesTo systematically review and summarize evidence on clinical burden of disease in patients with DM/PM in Europe.MethodsA systematic literature review (SLR) was conducted in MEDLINE and Embase databases to identify studies in children and adults with DM/PM, published in the English language between 2011 and 2021. Primary studies enrolling 10 or more patients were included, irrespective of country or region. Each eligible article was independently reviewed by two reviewers. The title and study abstracts were reviewed to assess eligibility for full-text review. The current abstract summarizes SLR results on the long-term outcomes and comorbidities in patients with DM/PM in Europe.ResultsA total of 2,967 non-duplicated publications were retrieved from medical databases and analyzed against pre-defined study selection criteria. There were 2,574 records excluded at title and abstract screening. Remaining 393 records were analyzed in the full text with 208 papers considered relevant. Additional 21 papers were identified from searching reference list of relevant studies and conference proceedings. In total, 222 studies (229 publications) were included in data abstraction. Among 43 studies conducted across 14 European countries, 23 studies evaluated long-term outcomes of disease and burden of comorbidities. There were 14 longitudinal cohort studies, 7 cross-sectional analyses and 2 case-control studies. Only 29-44% of DM/PM adults achieved remission. Between 38% and 62% of adults with DM/PM required at least 3 different medications, suggesting inadequate response to initial and subsequent regimens. During a mean follow-up of 13.9 years, 69% patients with JDM had polycyclic or chronic disease characterized by periods of remission and recurrence or by permanently active disease for more than 2 years after diagnosis. A notable proportion of children (60.4%) showed evidence of damage in at least one body organ and 13-20% of patients had complications such as calcinosis, lipodystrophy, or muscle atrophy. Adults with DM/PM often suffered from interstitial lung disease (16-66%), serious infections leading to hospitalization or death (21-26.7%), and cardiovascular disorders (CVDs) (19-71%). Compared to age and gender matched controls without DM, patients with DM had 16-times higher risk of venous thromboembolism (HR=16.44, 95% CI: 7.54-35.86). Incidence of coronary heart disease, hemorrhagic stroke, or ischemic stroke during 10-year follow-up was approximately 1.5- to 2-times higher in DM/PM adults relative to general population. Multiple studies reported high rates of any malignancy in DM adults (7.5-33%) and in PM adults (8-11.7%). During approximately 24,000 patient-years of follow-up, adults with DM/PM had more than 4-times higher incidence of lung cancer compared to general population that also was one of the highest incidences among multiple other autoimmune diseases. Long-term prognosis in DM/PM was poor with 20-year survival below 70%. The most common causes of deaths were pulmonary-related, malignancies, and cardiac complications. Mortality due to lung cancer was more than 4-times higher than in general population (standardized mortality ratio=4.17, 95% CI: 3.03-5.60).ConclusionEuropean patients with DM/PM suffer from substantial burden of comorbidities including serious infections, malignancies and CVDs that lead to poor long-term outcomes. A notable proportion of adult and juvenile patients do not achieve sustainable remission or experience relapse indicating high unmet need.Disclosure of InterestsJiří Vencovský Speakers bureau: Abbvie, Biogen, Boehringer, Eli Lilly, Gilead, MSD, Novartis, Pfizer, Roche, Sanofi, UCB, Werfen, Consultant of: Abbvie, Argenx, Boehringer, Eli Lilly, Gilead, Octapharma, Pfizer, UCB, Grant/research support from: Abbvie, Konrad Pisarczyk Consultant of: Kezar Life Sciences, Richard Leff Shareholder of: Kezar Life Sciences, Consultant of: Kezar Life Sciences, Eunmi Park Shareholder of: Kezar Life Sciences, Employee of: Kezar Life Sciences, Kiruthi Palaniswamy Shareholder of: Kezar Life Sciences, Employee of: Kezar Life Sciences, Li Long Shareholder of: Kezar Life Sciences, Employee of: Kezar Life Sciences.

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Auger, Sophie, Genevieve Margueritte, Renaud Tichit, Basheer Khalil, Philippe Quittet, Nathalie Fegueux, Patrice Ceballos et Jean-Francois Rossi. « Multiple Myeloma Is Exceptional in Paediatrics : Report of One Case From 1200 Patients in a Single Institution. » Blood 114, n^o 22 (20 novembre 2009) : 4957. http://dx.doi.org/10.1182/blood.v114.22.4957.4957.

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Abstract Abstract 4957 Multiple myeloma (MM), a disease usually observed in elderly patients, is extremely rare below 30 years of age. We present a case of a MM in a 10-year-old boy who has been admitted in September 2007 to the paediatric unit from the university hospital in Montpellier, with a fracture of his left femoral bone after a rugby match. In his history, he was known to present a juvenile myelomonocytic leukaemia (JMML) when he was 4-month-old in December 1998. For this diagnosis, he has been treated with aracytine and hydroxyurea for 4 years and he got a complete response (CR) since July 2005. At admission, surprisingly the radiography showed two lytic bone lesions. At MRI, it was found proximal and distal medullar metadiaphyseal spreading associated to a fracture, with no clinical symptom. The histology of the two tissue biopsies showed large dystrophic plasma cells, MI 15 positive with no clear evidence of a monoclonality by using light chain immunostaining. The bone marrow biopsy showed an interstitial infiltrate of dystrophic plasma cells, with only lambda light chain expression. Five percent of dystrophic plasma cells were observed on bone marrow smears. The monoclonal component IgG Lamda was 3.56 G/dL. Free kappa and lambda light chain dosages were respectively 5.65 mg/L and 766 mg/L, with a kappa lambda ratio under 0.01. Proteinuria was 0.64 g/day, haemoglobin was 106 G/L, and Beta2 microglobulin was 2.6mg/L. There was no hypercalcaemia and serum albumin and creatinin clearance were normal. Plasma cell labelling index (PCLI) was 1.16 % in the bone marrow and 6.6 circulating plasma cells/μL were counted in peripheral blood. Unfortunately, gene expression profiling analysis failed due to the low number of cells. PET scan found multiple uptakes in femoral, vertebral costal and sternal bones. So, this boy presented a multiple myeloma with stage IIIA according to Durie Salmon staging and ISS (International staging system) I. He underwent nine cycles of bortezomib (1.3 mg/m2 D1, D4, D8, D11) and dexamethazone (40mg/D, D1 to D4) to reach a complete response. A myeloablative allogenic stem cell transplantation was performed from his sister the 11th of September 2008, with a regimen based on cyclophosphamide (60mg/Kg, D1, D2) and TBI 12Gy. The immunosuppressive regimen associated methotrexate (D1, D3, D6) and cyclosporine. The graft contained 4.14 ×108 MNC/kg, 4.19 106 CD34/Kg and 6.16 107 CD3/Kg. At Day 120, a full donor chimerism was obtained, with no GVHd, but the monoclonal component reappeared. He received only a single cycle of bortezomib and dexamethazone because of severe peripheral neuropathy and gastro-intestinal intolerance. A second CR has been obtained in June 2009. Minimal residual disease by flow cytometry will be soon performed in order to discuss donor lymphocyte infusions. We report a case of MM during the childhood that is extremely rare. Very few cases have been reported in the literature. In this particular case, the patient has been also treated for a JMML that may have a relationship with the MM. Unfortunately, no cytogenetic or DNA profiling has been performed. To our knowledge, it is the first time that such feature is reported. The overall survival (OS) reported by the Mayo clinic in a series of 10 children was 87 months that may suggests a better OS as compared to adults (Blade J, Kyle RA, Greipp PR. Multiple myeloma in patients younger than 30 years - Report of 10 cases and review of the literature. Arch Intern Med. 1996;156:1463-8). Disclosures No relevant conflicts of interest to declare.

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Coltoff, Alexander, Deidra Smith, James A. Davis, Kelly J. Gaffney, Praneeth Baratam et Kimberly Michelle Green. « Impact of PTPN11 Mutations in De Novo, Secondary and Relapsed/Refractory Acute Myeloid Leukemia ». Blood 142, Supplement 1 (28 novembre 2023) : 5991. http://dx.doi.org/10.1182/blood-2023-182407.

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Background Somatic mutations in the protein phosphatase non-receptor type 11 ( PTPN11) gene occur frequently in children with juvenile myelomonocytic leukemia and less commonly in adults with AML, being present in less than 5% of reported cases. Uncertainty exists regarding the prognostic impact of PTPN11 mutations in AML, largely owing to their observed rarity. Some retrospective studies have identified PTPN11 mutations as deleterious prognostic markers in AML, though others have noted that their negative impact is mitigated by concurrent NPM1 mutations, which frequently co-occur. We analyzed the outcomes of patients at our institution with AML and PTPN11 mutations in order to add to the literature regarding the relevance of this mutation. Methods We conducted a retrospective case series of patients at a single-center, tertiary-care institution who were diagnosed with AML between January 1 st, 2020 and June 1 st, 2023. Inclusion criteria included: 18 years of age or older, primary follow-up at the study institution, documented diagnosis of AML and identification of a PTPN11 mutation by NGS. Outcomes of interest included cytogenetic and molecular profiles, response to induction or salvage therapy, progression-free survival (PFS) and overall survival (OS). Results Twenty-two (12 men and 10 women) patients with PTPN11 mutant AML were identified. The median age at diagnosis was 60 years (range, 31-89 years). Nineteen patients had a PTPN11 mutation identified at diagnosis and 3 had a PTPN11 mutation appear at the time of relapse. Nine patients with de novo AML had PTPN11 mutations identified at diagnosis. Six were female and the average age at diagnosis was 60 years (range, 44-89 years). The most common co-occurring mutations were NPM1 (8 patients), DNMT3A (3 patients), TET2 (2 patients) and FLT3-TKD (2 patients). Cytogenetics were normal or non-adverse in 8 patients, and complex/adverse-risk in 1 patient. Seven patients received treatment - 4 with intensive chemotherapy and 3 with a hypomethylating agent (HMA) + venetoclax. Treatment response was CR/CRi in 6 patients (85.7%). At a median follow-up of 195 days, median PFS and OS were not reached. Two patients relapsed at 76 and 139 days after response. No patients received an allogeneic transplant in first remission. Ten patients with therapy-related or secondary-AML (t/s-AML) were identified, all with PTPN11 mutations noted at the time of AML diagnosis. Two patients had preceding MDS and 3 had preceding MDS/MPN. Median age was 63 years (range, 31-70 years). The most common co-occurring mutations were RUNX1 (5 patients), FLT3-ITD (2 patients) and NRAS/KRAS (3 patients). Chromosome 7 abnormalities were present in 4 of 5 patients with RUNX1 mutations. Cytogenetics were complex or adverse-risk in 9 patients. Eight patients received treatment - 6 with intensive chemotherapy and 2 with HMA + venetoclax. Treatment response was CR/CRi in 3 patients (37.5%). Median PFS was 183 days and median OS in treated patients was 307 days. The only patient with an ongoing response at the time of data cut-off received an allogeneic transplantation in CR1. Three patients with AML had emergence of a PTPN11 mutation at the time of relapse, at 245, 343 and 2262 days after initial diagnosis. PTPN11 was the only new mutation identified in 2 patients, and emerged in conjunction with an NRAS and KDM6A mutation in 1 patient. One patient received treatment, attaining a CRi after HMA + venetoclax, but relapsed 112 days later. The other 2 patients passed at 10 and 21 days after relapse. Conclusions PTPN11 mutations were relatively rare, occurring in only 22 patients with AML during a 3.5-year time span. Stark differences were noted in patients with de novo AML versus t/s-AML and PTPN11 mutations. De novo AML patients frequently demonstrated NPM1 mutations and a normal karyotype, while t/s-AML patients frequently had RUNX1 and FLT3 mutations, as well as adverse cytogenetics. Although the impact of PTPN11 mutations in AML may be influenced by concurrent NPM1 mutations or cytogenetic characteristics, the context of AML may ultimately have the largest impact.

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Williams, Emma. « P03 Leflunomide treatment for inflammatory bowel disease and intestinal failure caused by TTC7A deficiency ». Archives of Disease in Childhood 108, n^o 5 (19 avril 2023) : 2.1–2. http://dx.doi.org/10.1136/archdischild-2023-nppg.3.

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TTC7A deficiencyUltra-rare autosomal-recessive variants in tetratricopeptide repeat domain 7A gene (TTC7A) have been discovered in patients presenting with severe intestinal disease. Mutations in the TTC7A gene cause intestinal epithelial and immune defects resulting in apoptotic enterocolitis, multiple intestinal atresia, and recurrent intestinal stenosis. Patients face high mortality rates with palliation as the current standard of care.1LeflunomideIn 2020 a high throughput screen identified drugs that increased cell viability in patients with TTC7A; leflunomide reduced caspase 3 and 7 (responsible for cell death) activity in cells by 96%. In zebrafish with disruption of TTC7A, leflunomide restored gut motility, reduced intestinal tract narrowing, and increased intestinal cell survival.1From a literature review, only 3 patients in the world have been prescribed leflunomide for TTC7A deficiency with ‘encouraging results’.2however no case reports have been completed on treatment safety or effectiveness.A common adverse effect of leflunomide is liver toxicity due to production of a toxic intermediate; however, the reaction appears to be idiosyncratic and unpredictable.3Full blood count and liver function tests must be checked before initiation of leflunomide, every two weeks during the first six months of treatment, and every 8 weeks thereafter.4The patientA 7-year-old male on home parenteral nutrition with TTC7A deficiency was admitted to hospital with high ileostomy output and persistent vomiting with a background of mucosal gastrointestinal inflammation and pyloric stenosis. On behalf of the gastroenterology team, the paediatric gastroenterology pharmacist applied for urgent internal funding and clinical governance approval for leflunomide treatment with the aim to ameliorate intestinal disease. Leflunomide 10 mg daily costs £3.11/month. Treatment was approved, the patient and his family were counselled by the pharmacist and the patient began treatment of leflunomide 10 mg via PEG tube daily.Adverse eventAfter two weeks of treatment the patient’s alkaline phosphate (ALP) and Gamma GT (GGT) had doubled and their alanine transaminase (ALT) had increased 10-fold. Advice from the pharmacist was sought. On review of the leflunomide summary of product characteristics4: ‘Rare cases of severe liver injury, including cases with fatal outcome, have been reported during treatment with leflunomide//If ALT elevations of more than 3-fold the upper limit of normal are present, leflunomide must be discontinued and wash-out procedures initiated.’ A decision was made to stop treatment, however a washout procedure with cholestyramine or activated charcoal was not possible as the patient had minimal oral intake due to vomiting. The pharmacist filed a yellow card report.Follow upThe patient’s ALT normalised after 3 weeks and GGT after 2 months of treatment cessation. It took 8 months for the patient’s ALP to normalise.Lessons learntUnfortunately, it was impossible to assess the potential gastrointestinal benefits of leflunomide in this patient due to the rapid onset of significant liver toxicity. Liver toxicity may have been identified sooner if a blood test was taken 1 week after treatment initiation. Monitoring liver function earlier following initiation of leflunomide treatment may be helpful to minimise liver toxicity in patients with TTC7A deficiency.ReferencesJardine S, Anderson S, Babcock S,et al. Drug screen identifies leflunomide for treatment of inflammatory bowel disease caused by TTC7A deficiency.Gastroenterology2020;158:1000–1015.Cerretani J. Going ‘all in’ for Khori: new hope for congenital enteropathy [Internet]. Boston Children’s Hospital, 2020. [accessed May 2022]. Available from: https://answers.childrenshospital.org/khori-congenital-enteropathy/Nuray Aktay A, Gul Karadag S, Cakmak F,et al. Leflunomide in juvenile rheumatoid arthritis.Future Rheumatol2006;1(6):673–682.Electronic Medicines Compendium [Internet]. Leflunomide 10 mg film-coated tablets, 2017 [cited May 2022]. Available from: https://www.medicines.org.uk/emc/product/5395/smpc

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Лазарев, С. Е. « Adaptation mechanisms and life strategies of species of the Robinia L. genus underthe conditions of introduction ». World Ecology Journal, n^o 1() (15 mars 2020) : 48–67. http://dx.doi.org/10.25726/worldjournals.pro/wej.2020.1.3.

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Родовой комплекс Robinia L. представляет большой интерес для мобилизации генетических ресурсов в аридные регионы Европы, Азии и Северной Америки. Различные темпы расширения культигенных ареалов некоторых видов рода RobiniaL. несомненно связаны с особенностями их адаптации и жизненными стратегиями выживания в новых условиях существования. В связи с этим, целью данной работы являлся анализ механизмов адаптации и жизненных стратегий различных видов рода Робиния в условиях интродукции. Объектами исследований стали виды и формы родаRobinia L.: R. neomexicana Gray. (syn. Robinia luxurians (Dieck.) C. K. Shneid.); R. pseudoacacia L.; R. pseudoacacia f. pyramidalis (Pepin) Rehd.; R. pseudoacacia f. umbraculifera (DC) Rehd.; Robinia viscosa Vent. var. hartwegii (Koehne) Ashe, произрастающие в кластерных коллекционных участках ФНЦ агроэкологии РАН, кадастр №34:34:000000:122, 34:34:060061:10. Проведенные исследования позволили выявить у различных представителей родаRobinia L. целый ряд филогенетических адаптаций к воздействию неблагоприятных факторов среды, таких как сильно разветвленная корневая система, ксероморфное строение листьев, ажурность и ветропроницаемость крон, способность переносить продолжительные засухи, симбиотические связи с азотфиксирующими бактериями. Результаты исследований показали, что в процессе интродукции все виды рода Robinia L. используют ряд фенотипических онтогенетических приспособлений. Наиболее важные из них – это смена жизненной формы (дерево - кустарник, одноствольное - многоствольное дерево), а также снижение темпов роста и уменьшение общей высоты растений в зависимости от почвенного плодородия, влагообеспечения и повреждающего воздействия низких зимних температур. Так, на обыкновенных черноземах в условиях Украины в возрасте 20-и лет насаждения из Робинии псевдоакации достигают высоты 14-15 м, тогда как на светло-каштановых почвах в условиях Нижнего Поволжья в этом же возрасте они достигают всего 6 м. Кроме этого, на протяжении последних столетий представители рода Robinia L. выработали ряд генотипических адаптации к новым условиям существования. Данные приспособления являются одними из самых важных, т.к. приводят к появлению качественно новых адаптаций, расширяющих границы экологической пластичности вида. У всех видов сократились сезонные циклы фенологического развития и в настоящее время они укладываются в оптимальные сроки развития древесных интродуцентов в регионах с относительно суровым для них климатом. По показателю фенологической атипичности в условиях Нижнего Поволжья они находятся в нижней половине области нормы (от +1 до 0) по реализации фенологических фаз, что свидетельствует от том, что цикл их развития успешно адаптировался и соответствует вегетационному периоду места интродукции. Как показали наши исследования, все виды рода Robinia L. в процессе акклиматизации, перешагнула температурный порог в - 37°С. Генотипическую природу сформировавшихся адаптаций к низким зимним температурам доказывает сравнительный анализ литературных данных по морозостойкости различных видов рода Robinia L. полученных в начале XX века с данными визуальных и физиологических методов оценки проведенных на протяжении последних десятилетий. Вторым важным доказательством появления адаптаций, закрепленных на генетическом уровне является разница в зимостойкости между формами R. pseudoacacia f. pyramidalis (Pepin) Rehd., R. pseudoacacia f. umbraculifera (DC) Rehd. и типичными представителями R. pseudoacacia L. Отсутствие генетической неоднородности при вегетативном размножении указанных форм остановило процессы микроэволюции, не позволив им адаптироваться в новых условиях существования. Анализ жизненных (экологических) стратегий показал, что в растительных сообществах Робиния псевдоакация может с одинаковым успехом выступать в роли патиента или эксплерента. При этом виалентные свойства у нее выражены намного слабее. Анализ r/K стратегий выживания позволяет отнести ее к r-видам, с высоким генеративным потенциалом, коротким ювенильным и виргинильным этапом развития, способностью к натурализации. Однако, в оптимальных условиях существования в отсутствии конкуренции она, как и многие К-виды может достигать значительного возраста до 400 лет. Все виды рода Robinia L. способны к натурализации в тех или иных регионах вторичного ареала. Однако рекордсменом по этому показателю несомненно является Робиния псевдоакация. По нашему мнению, данный факт объясняется высоким генеративным потенциалом R. pseudoacacia L. по отношению к родственным видам и его высокой хозяйственной значимостью для целей лесозащитного разведения. Представители рода Robinia L. не имеют приспособлений для активного распространения семян на значительные расстояния. Натурализация (вхождение в естественные растительные сообщества) происходит, как правило, в непосредственной близости от искусственных лесозащитных насаждений. Розовоцветковые виды рода Robinia L., ввиду небольшого роста, не представляют особого интереса для целей агролесомелиорации. Данные виды используются обычно в озеленении населенных пунктов как декоративные растения. Искусственная территориальная изоляция от естественных растительных сообществ и относительно низкий генеративный потенциал не позволяют им активно проявлять инвазивные свойства. The Robinia L. genus is of great interest for mobilizing genetic resources in arid regions of Europe, Asia, and North America. The different rates of expansion of cultigen areasof some species of the Robinia L. genus are undoubtedly related to the peculiarities of their adaptation and life strategies for survival in new conditions of existence. In this regard, the purpose of this work was to analyze the mechanisms of adaptation and life strategies of various species of the Robinia genus under the conditions of introduction. The objects of research were species and forms of the Robinia L. genus: R. neomexicana Gray. (syn. Robinia luxurians (Dieck.) C.K. Shneid.); R. pseudoacacia L.; R. pseudoacacia f. pyramidalis (Pepin) Rehd.; R. pseudoacaciaf. umbraculifera (DC) Rehd.; Robinia viscosa Vent. var. hartwegii (Koehne) Ashe, growing in cluster collection sites of the Federal Centerfor Agroecology of the Russian Academy of Sciences, cadastre No. 34:34:000000:122, 34:34:060061:10. Studies have revealed a number of phylogenetic adaptations to adverse environmental factors in various members of the Robinia genus, such as a highly branched root system, xeromorphic structure of leaves, opennessand wind permeability of crowns, the ability to tolerate prolonged droughts, and symbiotic relationships with nitrogen-fixing bacteria. The research results have shown that all species of the Robinia L. genus use a number of phenotypic ontogenetic devices during introduction. Among them, the most important ones are the change of life form (tree – shrub, single-trunk tree – multi-trunk tree), as well as a decrease in growth rates and a decrease in the overall height of plants, depending on soil fertility, moisture supply, and the damaging effects of low winter temperatures. For example, on ordinary chernozems in Ukraine at the age of 20 years, plantings ofRobinia pseudoacacia reach a height of 14-15 m, while on light chestnut soils in the Lower Volga region at the same age, they reach only 6 meters. In addition, over the past centuries, representatives of the Robinia L. genus have developed a number of genotypic adaptations to new conditions of existence. These adaptations are among the most important ones, because they lead to the appearance of qualitatively new adaptations that expand the boundaries of ecological plasticity of the species. All species have reduced their seasonal cycles of phenological development and currently meetthe optimal time frame for the development of introduced trees in regions with a relatively harsh climate for them. According to the indicator of phenological atypicality, they are in the lower half of the normal range (from +1 to 0) in terms ofthe implementation of phenological phases, which indicates that the cycle of their development has successfully adapted and corresponds to the vegetation period of the place of introduction. As shown by the authors’research, all species of the Robinia L. genus in the process of acclimatization crossed the temperature threshold of –37°C. The genotypic nature of the formed adaptations to low winter temperatures is proved by a comparative analysis of the literature data on the frost resistance of various species of the Robinia L. genus obtained at the beginning of the 20thcentury with the data of visual and physiological assessment methods conducted over the past decades. The second important proof of the appearance of adaptations fixed at the genetic level is the difference in winter hardiness between the forms of R. pseudoacacia f. pyramidalis (Pepin) Rehd.; R. pseudoacacia f. umbraculifera (DC) Rehd. and typical representatives of R. pseudoacacia L. The absence of genetic heterogeneity in the vegetative reproduction of these forms of Robinia stopped the processes of microevolution, not allowing them to adapt to the new conditions of existence. Analysis of life (environmental) strategies has shown that in plant communities,Robiniacan equally well act as a patient or an explerent. At the same time, the violent properties of Robinia are much less pronounced. Analysis of r/K survival strategies allows classifying it as an r-species with high generative potential, short juvenile and virginal stages of development, and the ability to naturalize. However, in optimal conditions of existence in the absence of competition, Robinia, like many K-species, can reach a significant age of up to 400 years. All Robinia species are capable of naturalization in certain regions of the secondary range. However, the record holder for this indicator is undoubtedly Robiniapseudoacacia. In the authors’opinion, this fact is explained by the high generative potential of R. pseudoacacia L. in relation to related species and its high economic significance for the purposes of forest protection breeding. Representatives of the Robinia L. genus do not have adaptations for active seed propagation over long distances. Naturalization (entering natural plant communities) usually occurs in the immediate vicinity of artificial forest protection stands at a distance. Pink-flowered species of the RobiniaL.genus,due to their small growth, are not of particular interest for agroforestry purposes. These types are usually used in landscaping settlements as ornamental plants. Artificial territorial isolation from natural plant communities and relatively low generative potential do not allow them to actively exhibit invasive properties.

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Chang, Shun-Chiao, Christian Stefan Eichinger et Polly Field. « The natural history and burden of illness of metachromatic leukodystrophy : a systematic literature review ». European Journal of Medical Research 29, n^o 1 (18 mars 2024). http://dx.doi.org/10.1186/s40001-024-01771-1.

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Abstract Background Metachromatic leukodystrophy (MLD; OMIM 250100 and 249900) is a rare lysosomal storage disease caused by deficient arylsulfatase A activity, leading to accumulation of sulfatides in the nervous system. This systematic literature review aimed to explore the effect of MLD on the lives of patients. Methods The Ovid platform was used to search Embase, MEDLINE, and the Cochrane Library for articles related to the natural history, clinical outcomes, and burden of illness of MLD; congress and hand searches were performed using ‘metachromatic leukodystrophy’ as a keyword. Of the 531 publications identified, 120 were included for data extraction following screening. A subset of findings from studies relating to MLD natural history and burden of illness (n = 108) are presented here. Results The mean age at symptom onset was generally 16–18 months for late-infantile MLD and 6–10 years for juvenile MLD. Age at diagnosis and time to diagnosis varied widely. Typically, patients with late-infantile MLD presented predominantly with motor symptoms and developmental delay; patients with juvenile MLD presented with motor, cognitive, and behavioral symptoms; and patients with adult MLD presented with cognitive symptoms and psychiatric and mood disorders. Patients with late-infantile MLD had more rapid decline of motor function over time and lower survival than patients with juvenile MLD. Commonly reported comorbidities/complications included ataxia, epilepsy, gallbladder abnormalities, incontinence, neuropathy, and seizures. Conclusions Epidemiology of MLD by geographic regions, quantitative cognitive data, data on the differences between early- and late-juvenile MLD, and humanistic or economic outcomes were limited. Further studies on clinical, humanistic (i.e., quality of life), and economic outcomes are needed to help inform healthcare decisions for patients with MLD.

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Quero, Rubí, Manuel Camacho et Yubisay Mundaraín. « Juvenile Ovarian Granulosa Cell Tumor - A Case Report ». Advances in Medicine and Engineering Interdisciplinary Research 1, n^o 2 (29 mai 2023). http://dx.doi.org/10.32629/ameir.v1i2.1216.

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The granulosa cell tumor is an unusual ovarian tumor, even more so in pediatric age. The literature indicates that the prevalence and the incidence is more higher in patients after the fifth decade of the life with a frequency of 3.4 % of all ovarian malignancies, since most of them correspond to the benign pathologies. The tumor produces symptoms derived from the secretion of the estradiol. The granulosa cell tumor has been described as better prognosis, less aggressive than other ovarian neoplasms and the natural history was longer. Surgery is the main diagnostic, staging and therapeutic approach. The adjuvant chemotherapy is recommended only in the early stages with risk factors or in the advanced stages, as it can improve disease-free and relapse-free survival rates, such as radiation therapy. So far, the most commonly used regimens are bleomycin, etoposide, and cisplatin, with a high reaction rate. Whenever possible, surgical rescue is proposed.

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Illing, Björn, Jennifer Sehl et Stefan Reiser. « Turbidity effects on prey consumption and survival of larval European smelt (Osmerus eperlanus) ». Aquatic Sciences 86, n^o 3 (juillet 2024). http://dx.doi.org/10.1007/s00027-024-01103-9.

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AbstractThe anadromous European smelt (Osmerus eperlanus) plays a key role in food webs of many riverine ecosystems in Europe. However, population sizes in several German rivers (e.g. Elbe or Weser rivers) have diminished drastically over the past decade. Turbidity has been considered one of the stressors affecting the successful recruitment of European smelt, as their early life stages may be particularly sensitive to changes in the abiotic environment. In this study, we investigated whether prey consumption and survival of European smelt larvae would be negatively affected by an acute exposure to elevated turbidity. We reared the larvae in the laboratory and exposed them in four separate trials (18 to 26 days post hatch, 9.5 ± 0.8 mm standard length, mean ± SD) to six turbidity levels (0–500 NTU, nephelometric turbidity units). We found that prey uptake increased at low turbidity levels and decreased at high turbidity levels, with an optimum between 100 and 200 NTU. Survival started to decrease at turbidity levels above 300 NTU. In addition, we conducted a systematic literature analysis in which we found that prey consumption of larval and juvenile fishes had been tested across a wide range of turbidity levels, mostly using pelagic (e.g. planktonic) prey items, with more studies focusing on perciform fishes and juvenile rather than larval life stages. Our empirical findings contribute to establishing thresholds for optimal larval European smelt performance under increased turbidity and provide valuable information for developing mechanistic models that assess potential consequences for European smelt recruitment dynamics.

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Piskin, Bulent, Abdullah Durmaz, Cumhur Sipahi, Alper Uyar et Savaş Özarslantürk. « Surgical and Prosthetic Management of a Juvenile Ossifying Fibroma In a 5-Year-Old Child : A Clinical Report ». Kapadokya Üniversitesi, 1 janvier 2024. http://dx.doi.org/10.54995/asc.3.1.5.

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Juvenile ossifying fibroma (JOF) is a rare and benign fibro-osseous lesion of the craniofacial skeleton commonly seen in children under 15 years of age. Despite being classified as benign, JOF lesions are aggressive in nature and reach large dimensions in a short period of time. Thus, their resections may cause large orofacial defects which have serious detrimental effects on the functional and nutritional requirements of pediatric patients being in the pe-riod of growth. Therefore, its early diagnosis and proper surgical and prost-hetic management are of vital importance for the survival of patients. However, no detailed reports in the literature describe the prosthetic mana-gement of large intraoral defects in pediatric patients under 10 years old.

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Chen, Rongxin, Shu Liu, Lijuan Tang, Xinyue Yu, Ziwei Meng, Yu Hu, Jing Li et Xuanwei Liang. « On the knowledge of solitary juvenile xanthogranuloma of the eyelid : a case series and literature review ». Graefe's Archive for Clinical and Experimental Ophthalmology, 27 janvier 2022. http://dx.doi.org/10.1007/s00417-022-05560-6.

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Abstract Purpose Solitary eyelid juvenile xanthogranuloma (JXG) is extremely rare, and there is limited literature on its clinical features and treatment outcomes. Here, we present a case series and comprehensive review of the literature on patients with isolated eyelid JXG. Methods We systematically extracted data from our institution’s records of isolated eyelid JXG cases and conducted a search for additional cases from the literature utilising the PubMed, Wanfang, and Chinese National Knowledge Infrastructure (CNKI) databases. Patients with JXG were analysed with respect to age, sex, clinical presentation, therapy, and outcome. Group comparisons were performed. Results Thirty-two patients (including 13 at our institution and 19 from prior publications) were identified. The median age at first presentation was higher in current patients than in the patients from the published cases (median 9 years, range 1.2 to 47.0 years; median 2 years, range 0.5 months to 46.0 years, respectively, P = 0.014). Of the patients who had known characteristics, no significant differences were observed between the two groups in terms of sex, affected eye, eyelid site, type of cutaneous involvement, or duration of symptoms (each P > 0.05). Seventeen (54.8%) patients were male. The most common lesion location was the upper eyelid (n = 10, 62.5%). Twenty-four (75.0%) cutaneous lesions had full-thickness skin involvement; 8 (25.0%) subcutaneous masses had a chalazion-like appearance. Histologically, the JXG masses were characterised by Touton giant cells with inflammatory cells. Additionally, there was no significant difference in treatment modalities between the two groups (P = 0.072), and 24 (75.0%) patients underwent surgical excision. The overall recurrence-free survival was 3.6 to 52.8 (median 27.0) months in the current patients. For published cases with available follow-up information, there was no recurrence in 10 cases and improvement in 1 case, with a median follow-up of 9.5 months. Conclusion Solitary eyelid JXG is a rare clinical entity and should be included in the differential diagnosis of eyelid mass lesions in patients of all age groups. Surgical excision is often selected for efficient treatment and to obtain an excisional biopsy.

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Lu, ChuChu, Agnieszka Gudowska et Joanna Rutkowska. « What do zebra finches learn besides singing ? Systematic mapping of the literature and presentation of an efficient associative learning test ». Animal Cognition, 10 juin 2023. http://dx.doi.org/10.1007/s10071-023-01795-w.

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AbstractThe process of learning in birds has been extensively studied, with a focus on species such as pigeons, parrots, chickens, and crows. In recent years, the zebra finch has emerged as a model species in avian cognition, particularly in song learning. However, other cognitive domains such as spatial memory and associative learning could also be critical to fitness and survival, particularly during the intensive juvenile period. In this systematic review, we provide an overview of cognitive studies on zebra finches, with a focus on domains other than song learning. Our findings indicate that spatial, associative, and social learning are the most frequently studied domains, while motoric learning and inhibitory control have been examined less frequently over 30 years of research. All of the 60 studies included in this review were conducted on captive birds, limiting the generalizability of the findings to wild populations. Moreover, only two of the studies were conducted on juveniles, highlighting the need for more research on this critical period of learning. To address this research gap, we propose a high-throughput method for testing associative learning performance in a large number of both juvenile and adult zebra finches. Our results demonstrate that learning can occur in both age groups, thus encouraging researchers to also perform cognitive tests on juveniles. We also note the heterogeneity of methodologies, protocols, and subject exclusion criteria applied by different researchers, which makes it difficult to compare results across studies. Therefore, we call for better communication among researchers to develop standardised methodologies for studying each cognitive domain at different life stages and also in their natural conditions.

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Schmiege, Stephanie C., Brendan M. Buckley, Dennis Stevenson, Truong Quang Cuong, Le Canh Nam et Kevin L. Griffin. « Contrasting physiological traits of shade tolerance in Pinus and Podocarpaceae native to a tropical Vietnamese forest : insight from an aberrant flat-leaved pine ». Tree Physiology, 25 septembre 2020. http://dx.doi.org/10.1093/treephys/tpaa123.

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Abstract The absence of pines from tropical forests is a puzzling biogeographical oddity potentially explained by traits of shade intolerance. Pinus krempfii (Lecomte), a flat-leaved pine endemic to the Central Highlands of Vietnam, provides a notable exception as it seems to compete successfully with shade-tolerant tropical species. Here, we test the hypothesis that successful conifer performance at the juvenile stage depends on physiological traits of shade tolerance by comparing the physiological characteristics of P. krempfii to coexisting species from two taxa: the genus Pinus, and a relatively abundant and shade-tolerant conifer family found in pantropical forests, the Podocarpaceae. We examined leaf photosynthetic, respiratory and biochemical traits. Additionally, we compiled attainable maximum photosynthesis, maximum RuBP carboxylation (Vcmax) and maximum electron transport (Jmax) values for Pinus and Podocarpaceae species from the literature. In our literature compilation, P. krempfii was intermediate between Pinus and Podocarpaceae in its maximum photosynthesis and its Vcmax. Pinus exhibited a higher Vcmax than Podocarpaceae, resulting in a less steep slope in the linear relationship between Jmax and Vcmax. These results suggest that Pinus may be more shade intolerant than Podocarpaceae, with P. krempfii falling between the two taxa. However, in contrast, Vietnamese conifers’ leaf mass per areas and biochemical traits did not highlight the same intermediate nature of P. krempfii. Furthermore, regardless of leaf morphology or family assignation, all species demonstrated a common and extremely high carbon gain efficiency. Overall, our findings highlight the importance of shade-tolerant photosynthetic traits for conifer survival in tropical forests. However, they also demonstrate a diversity of shade tolerance strategies, all of which lead to the persistence of Vietnamese juvenile conifers in low-light tropical understories.

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Accolla, Chiara, Amelie Schmolke, Maxime Vaugeois et Nika Galic. « Density‐dependent population regulation in freshwater fishes and small mammals : A literature review and insights for Ecological Risk Assessment ». Integrated Environmental Assessment and Management, 26 septembre 2023. http://dx.doi.org/10.1002/ieam.4845.

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ABSTRACTThe regulation of populations through density dependence (DD) has long been a central tenet of studies of ecological systems. As an important factor in regulating populations, DD is also crucial for understanding risks to populations from stressors, including its incorporation in population models applied for this purpose. However, studying density‐dependent regulation is challenging because it can occur through various mechanisms, and their identification in the field, as well as the quantification of the consequences on individuals and populations, can be difficult. We conducted a targeted literature review specifically focusing on empirical laboratory or field studies addressing negative DD in freshwater fish and small rodent populations, two vertebrate groups considered in pesticide ecological risk assessment (ERA). We found that the most commonly recognized causes of negative DD were food (63% of 19 reviewed fish studies, 40% of 25 mammal studies) or space limitations (32% of mammal studies). In addition, trophic interactions were reported as causes of population regulation, with predation shaping mostly small mammal populations (36% of the mammal studies) and cannibalism impacting freshwater fish (26%). In the case of freshwater fish, 63% of the studies were experimental (i.e., with a length of weeks or months). They generally focused on the individual‐level causes and effects of DD, and had a short duration. Moreover, DD affected mostly juvenile growth and survival of fish (68%). On the other hand, studies on small mammals were mainly based on time series analyzing field population properties over longer timespans (68%). DD primarily affected survival in sub‐adult and adult mammal stages and, to a lesser extent, reproduction (60% vs. 36%). Furthermore, delayed DD was often observed (56%). We conclude by making suggestions on future research paths, providing recommendations for including DD in population models developed for ERA, and making the best use of the available data.

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Şahin Arslan, Necmiye, et Thomas E. Martin. « Comparative reproductive ecology of Old and New World Trogons, an order in decline across the world ». Ecology and Evolution 14, n^o 4 (avril 2024). http://dx.doi.org/10.1002/ece3.11273.

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AbstractMany tropical species show declining populations. The pantropical order Trogoniformes has 76% of its species ranked as declining, reflecting a worldwide problem. Here, we report on the reproductive ecology and life history traits of the declining and near‐threatened old world Whitehead's Trogon (Harpactes whiteheadi), the declining new world Collared Trogon (Trogon collaris), and the stable Masked Trogon (T. personatus). We also reviewed the literature on reproductive ecology and life history traits of trogons to assess possible commonalities that might help explain population declines. We found that the declining Whitehead's and Collared Trogons had reasonable nest success (32% and 25%, respectively), while the stable Masked Trogon had poor reproductive success (9%), all contrary to population trends. However, the limited literature data suggested that poor reproductive success may be common among trogons, which may contribute to population declines. Parents fed young at a low rate and had long on‐bouts for incubation and nestling warming that reduced activity at the nest, as favored by high nest predation risk over evolutionary time. We found that young fledged from the nest with poorly developed wings, as also favored by high nest predation risk. Evolved nestling periods among trogon species suggests that poor wing development is likely common. Wing development has been shown to affect juvenile survival after leaving the nest. The poor wing development may be an important contributor to population declines that deserves more attention. Evolved life history traits are important to recognize as creating population vulnerabilities in a changing world.

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Koenig, Christopher, Felicia Coleman et James V. Locascio. « Estimates of Western Atlantic Goliath Grouper (Epinephelus Itajara) Bycatch Mortality in Commercial Fisheries of the Southeastern United States From 2002 to 2022 ». Qeios, 26 avril 2024. http://dx.doi.org/10.32388/rwef3z.2.

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The Atlantic Goliath Grouper (_Epinephelus itajara_) in the southeastern United States is in decline due to increasingly large and persistent red tides, loss of juvenile habitat, and severe cold-weather events. In this paper, we address another source of mortality -- bycatch, focusing on data from NOAA Fisheries logbooks for commercial fisheries operating between 2002 to 2022. We calculate capture related mortality based on known patterns of depth-related barotrauma drawn from the literature and assume that all fish captured at depths greater than 30 m died if unvented prior to release. The overall result suggests that 75% (14,124 of 18,770) of Goliath Grouper captures died from barotrauma, as they either hemorrhaged or were unable to return to the bottom because of increased buoyancy due to expanded gas trapped in the swim bladder. While fishers currently use self-reporting to document these mortalities, this approach often results in biases toward lower capture and a higher survival rate. Thus, we strongly urge the Florida Fish and Wildlife Conservation Commission and the National Oceanic and Atmospheric Administration to quit using logbook data and instead require that fishers use paid observers and remote electronic monitoring on all commercial and charter vessels to ensure more effective, timely and reliable bycatch data for this and other protected species.

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Pollentier, Christopher D., Scott D. Hull et David M. MacFarland. « Sensitivity of multiple vital rates for Ruffed Grouse in the Upper Great Lakes Region ». Ornithological Applications 123, n^o 1 (1 février 2021). http://dx.doi.org/10.1093/ornithapp/duaa063.

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Abstract Effective management of wildlife requires a full understanding of population dynamics and knowledge of potential drivers that influence population growth. The Ruffed Grouse (Bonasa umbellus) is a popular upland game bird widely distributed across the northern United States and Canada that has experienced population declines within portions of its range in response to forest maturation and habitat loss. Although the species has been extensively studied, few efforts have been made to synthesize demographic data into a sensitivity analysis to guide management actions. We reviewed the literature and compiled Ruffed Grouse vital rates from 14 field studies conducted across four decades (1982−2018) within the Upper Great Lakes region of Michigan, Minnesota, and Wisconsin, USA. We parameterized a deterministic matrix model to evaluate population dynamics and conducted sensitivity analyses to identify vital rates projected to have the greatest influence on the finite rate of population change (λ). Our modeling effort projected a stable but highly variable annual rate of population change (λ = 1.01; 95% CI = 0.88–1.14) for Ruffed Grouse in the Upper Great Lakes region. Stochastic rates of population change derived from spring drumming surveys (λ = 1.01; 95% CI = 0.61–1.45) and Christmas Bird Count surveys (λ = 0.99; 95% CI = 0.62–1.76) of the corresponding regional population provided validation of stable trends over the same time period as our demographic model. Prospective elasticities and variance-scaled sensitivities suggested λ would be greatly influenced by components of reproductive performance: nesting success, chick survival, and post-fledging juvenile survival. Retrospective analysis indicated that much of the overall variability in λ and annual productivity was also attributed to annual variation in nesting success. Management of this species has often focused on fall and overwinter survival, but population projection models provided little evidence that survival was the predominant factor affecting population growth of Ruffed Grouse in this region. A suite of confounding factors and demographic processes that drive population trends can differ significantly across a species’ range. In the Upper Great Lakes region, management efforts aimed at maximizing reproductive success would likely have the greatest potential influence on Ruffed Grouse population growth. Other types of systematic, regional survey data can also be useful for validating population trends derived from demographic modeling studies.

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Armstrong, Nigel, Andrew Olaye, Caro Noake et Francis Pang. « A systematic review of clinical effectiveness and safety for historical and current treatment options for metachromatic leukodystrophy in children, including atidarsagene autotemcel ». Orphanet Journal of Rare Diseases 18, n^o 1 (29 août 2023). http://dx.doi.org/10.1186/s13023-023-02814-2.

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Abstract Objective To understand the benefit-risk profile for historical and current treatments for MLD. Methods A systematic review was conducted on the effectiveness, safety, and costs of MLD treatments: allogeneic haematopoietic stem cell transplantation (HSCT) and atidarsagene autotemcel (arsa-cel) according to best practice. Results A total of 6940 titles and abstracts were retrieved from the literature searches and 26 from other sources. From these, 35 manuscripts reporting on a total of 12 studies were selected for inclusion in the review. There were no controlled multi-armed trials. However, we provide observations comparing two interventional therapies (alloHSCT and arsa-cel) and each of these to standard/supportive care (natural history). There were no benefits for survival, gross motor function and cognitive function for LI patients receiving alloHSCT, as patients experienced disease progression similar to LI natural history. For juvenile patients receiving alloHSCT, no differences in survival were observed versus natural history, however stabilisation of cognitive and motor function were reported for some patients (particularly for pre- or minimally-symptomatic LJ patients), while others experienced disease progression. Furthermore, alloHSCT was associated with severe complications such as treatment-related mortality, graft versus host disease, and re-transplantation in both LI and EJ treated patients. Most LI and EJ patients treated with arsa-cel appeared to have normal development, preservation, or slower progression of gross motor function and cognitive function, in contrast to the rapid decline observed in natural history patients. A survival benefit for arsa-cel versus natural history and versus alloHSCT was observed in LI patients.LI and EJ patients treated with arsa-cel had better gross motor function and cognitive function compared to alloHSCT, which had limited effect on motor and cognitive decline. No data has been reported for arsa-cel treatment of LJ patients. Conclusions Overall, this systematic review indicates that compared to NHx and HSCT, treatment with arsa-cel results in clinically relevant benefits in LI and EJ MLD patients by preserving cognitive function and motor development in most patients, and increased survival for LI patients. Nevertheless, further research is required to confirm these findings, given they are based on results from non-RCT studies.

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Beerepoot, Shanice, Stefan Nierkens, Jaap Jan Boelens, Caroline Lindemans, Marianna Bugiani et Nicole I. Wolf. « Peripheral neuropathy in metachromatic leukodystrophy : current status and future perspective ». Orphanet Journal of Rare Diseases 14, n^o 1 (4 novembre 2019). http://dx.doi.org/10.1186/s13023-019-1220-4.

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Abstract Metachromatic leukodystrophy (MLD) is an autosomal recessively inherited metabolic disease characterized by deficient activity of the lysosomal enzyme arylsulfatase A. Its deficiency results in accumulation of sulfatides in neural and visceral tissues, and causes demyelination of the central and peripheral nervous system. This leads to a broad range of neurological symptoms and eventually premature death. In asymptomatic patients with juvenile and adult MLD, treatment with allogeneic hematopoietic stem cell transplantation (HCT) provides a symptomatic and survival benefit. However, this treatment mainly impacts brain white matter, whereas the peripheral neuropathy shows no or only limited response. Data about the impact of peripheral neuropathy in MLD patients are currently lacking, although in our experience peripheral neuropathy causes significant morbidity due to neuropathic pain, foot deformities and neurogenic bladder disturbances. Besides, the reasons for residual and often progressive peripheral neuropathy after HCT are not fully understood. Preliminary studies suggest that peripheral neuropathy might respond better to gene therapy due to higher enzyme levels achieved than with HCT. However, histopathological and clinical findings also suggest a role of neuroinflammation in the pathology of peripheral neuropathy in MLD. In this literature review, we discuss clinical aspects, pathological findings, distribution of mutations, and treatment approaches in MLD with particular emphasis on peripheral neuropathy. We believe that future therapies need more emphasis on the management of peripheral neuropathy, and additional research is needed to optimize care strategies.

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Articles de revues sur le sujet « Survival – Miscellanea – Juvenile literature »

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