Готові списки джерел за темами / Primary hyperoxaluria type 1

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Добірка наукової літератури з теми "Primary hyperoxaluria type 1"

Автор: Grafiati

Опубліковано: 11 березня 2023

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Статті в журналах з теми "Primary hyperoxaluria type 1"

Cochat, Pierre. "Primary hyperoxaluria type 1." Kidney International 55, no. 6 (June 1999): 2533–47. http://dx.doi.org/10.1046/j.1523-1755.1999.00477.x.

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Ajzensztejn, M. J., N. J. Sebire, R. S. Trompeter, and S. D. Marks. "Primary hyperoxaluria type 1." Archives of Disease in Childhood 92, no. 3 (March 1, 2007): 197. http://dx.doi.org/10.1136/adc.2006.107334.

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Abukhatwah, Mohamed W., Samia H. Almalki, Mohammed S. Althobaiti, Abdulla O. Alharbi, Najla K. Almalki, and Naglaa M. Kamal. "Primary hyperoxaluria Type 1." Medicine 99, no. 25 (June 19, 2020): e20371. http://dx.doi.org/10.1097/md.0000000000020371.

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Kumar, Aman, Prateek Kinra, and A. W. Kashif. "Autopsy Findings in an Infant with Primary Hyperoxaluria (Type-1)." Annals of Pathology and Laboratory Medicine 7, no. 12 (December 30, 2020): C178–182. http://dx.doi.org/10.21276/apalm.2908.

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Histopathological findings in oxalosis patient are limited in the literature, although it has high mortality. Oxalosis, which is defined as deposition of calcium oxalate crystals in tissues, is the final stage of various hyperoxaluric syndromes. It is often missed and is rare. The diagnosis is often delayed, since it requires special laboratory tests for establishing the diagnosis. Kidneys, blood vessel walls, and bones are the major sites for crystal deposition. We present an infant autopsy case of primary hyperoxaluria, type 1. Diagnosis was established with genetic testing. On autopsy, calcium oxalate crystals which were refringent to polarized light were found in both kidneys.

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Cochat, P., A. Deloraine, M. Rotily, F. Olive, I. Liponski, and N. Deries. "Epidemiology of primary hyperoxaluria type 1." Nephrology Dialysis Transplantation 10, supp8 (January 1, 1995): 3–7. http://dx.doi.org/10.1093/ndt/10.supp8.3.

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Ichiyama, Arata, Toshiaki Oda, and Eiko Maeda-Nakai. "Primary Hyperoxaluria Type 1 in Japan." Cell Biochemistry and Biophysics 32, no. 1-3 (2000): 171–76. http://dx.doi.org/10.1385/cbb:32:1-3:171.

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Takayama, Tatsuya, Masao Nagata, Arata Ichiyama, and Seiichiro Ozono. "Primary Hyperoxaluria Type 1 in Japan." American Journal of Nephrology 25, no. 3 (2005): 297–302. http://dx.doi.org/10.1159/000086361.

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Cochat, Pierre, Aurélia Liutkus, Sonia Fargue, Odile Basmaison, Bruno Ranchin, and Marie-Odile Rolland. "Primary hyperoxaluria type 1: still challenging!" Pediatric Nephrology 21, no. 8 (August 2006): 1075–81. http://dx.doi.org/10.1007/s00467-006-0124-4.

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Frishberg, Yaacov, Sofia Feinstein, Choni Rinat, and Alfred Drukker. "Hypothyroidism in primary hyperoxaluria type 1." Journal of Pediatrics 136, no. 2 (February 2000): 255–57. http://dx.doi.org/10.1016/s0022-3476(00)70112-0.

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Frishberg, Yaacov, Georges Deschênes, Jaap W. Groothoff, Sally-Anne Hulton, Daniella Magen, Jérôme Harambat, William G. van’t Hoff, et al. "Phase 1/2 Study of Lumasiran for Treatment of Primary Hyperoxaluria Type 1." Clinical Journal of the American Society of Nephrology 16, no. 7 (May 13, 2021): 1025–36. http://dx.doi.org/10.2215/cjn.14730920.

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Background and objectivesIn the rare disease primary hyperoxaluria type 1, overproduction of oxalate by the liver causes kidney stones, nephrocalcinosis, kidney failure, and systemic oxalosis. Lumasiran, an RNA interference therapeutic, suppresses glycolate oxidase, reducing hepatic oxalate production. The objective of this first-in-human, randomized, placebo-controlled trial was to evaluate the safety, pharmacokinetic, and pharmacodynamic profiles of lumasiran in healthy participants and patients with primary hyperoxaluria type 1.Design, setting, participants, & measurementsThis phase 1/2 study was conducted in two parts. In part A, healthy adults randomized 3:1 received a single subcutaneous dose of lumasiran or placebo in ascending dose groups (0.3–6 mg/kg). In part B, patients with primary hyperoxaluria type 1 randomized 3:1 received up to three doses of lumasiran or placebo in cohorts of 1 or 3 mg/kg monthly or 3 mg/kg quarterly. Patients initially assigned to placebo crossed over to lumasiran on day 85. The primary outcome was incidence of adverse events. Secondary outcomes included pharmacokinetic and pharmacodynamic parameters, including measures of oxalate in patients with primary hyperoxaluria type 1. Data were analyzed using descriptive statistics.ResultsThirty-two healthy participants and 20 adult and pediatric patients with primary hyperoxaluria type 1 were enrolled. Lumasiran had an acceptable safety profile, with no serious adverse events or study discontinuations attributed to treatment. In part A, increases in mean plasma glycolate concentration, a measure of target engagement, were observed in healthy participants. In part B, patients with primary hyperoxaluria type 1 had a mean maximal reduction from baseline of 75% across dosing cohorts in 24-hour urinary oxalate excretion. All patients achieved urinary oxalate levels ≤1.5 times the upper limit of normal.ConclusionsLumasiran had an acceptable safety profile and reduced urinary oxalate excretion in all patients with primary hyperoxaluria type 1 to near-normal levels.Clinical Trial registry name and registration number:Study of Lumasiran in Healthy Adults and Patients with Primary Hyperoxaluria Type 1, NCT02706886

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Дисертації з теми "Primary hyperoxaluria type 1"

Von, Schnakenburg Claus Christian. "Molecular analysis of the AGXT gene and linkage studies in primary hyperoxaluria type 1." Thesis, University College London (University of London), 1998. http://ethos.bl.uk/OrderDetails.do?uin=uk.bl.ethos.299831.

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DEMARTA, JOCELYNE. "L'hyperoxalurie primaire de type i de revelation tardive chez l'adulte : a propos de deux cas." Reims, 1992. http://www.theses.fr/1992REIMM081.

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Newbound, Garret C. "Transcriptional control of human t-cell leukemia virus type-1 in primary lymphocytes /." The Ohio State University, 1997. http://rave.ohiolink.edu/etdc/view?acc_num=osu1487948440826361.

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Hayman, Anna. "The biodiversity of human immunodeficiency virus type 1 : evolution during primary infection and transmission." Thesis, University College London (University of London), 2002. http://ethos.bl.uk/OrderDetails.do?uin=uk.bl.ethos.252357.

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Estève, Julie. "Transfert de gènes dans les cellules souches pluripotentes induites : application à la thérapie génique de l'hyperoxalurie primitive de type 1." Thesis, Bordeaux, 2018. http://www.theses.fr/2018BORD0280/document.

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L’hyperoxalurie primitive de type 1 (ou HP1) est une maladie héréditaire du métabolisme liée à un déficit en enzyme hépatocytaire AGT (alanine:glyoxylate aminotransférase), codée par le gène AGXT. Ce déficit entraîne, chez les patients atteints d’HP1, une excrétion hépatique accrue d’oxalate ; celui-ci est ensuite éliminé dans les urines où il se complexe avec le calcium pour former des néphrolithiases oxalo-calciques massives, pouvant conduire à une insuffisance rénale chronique. Le seul traitement curatif disponible pour cette pathologie est la greffe allogénique combinée hépatorénale, actuellement limitée par la disponibilité des donneurs de greffons, une morbi-mortalité significative et la nécessité d’un traitement immunosuppresseur au long cours. L’objectif du projet de recherche est de développer une thérapie génique de l’HP1 par greffe de cellules hépatiques autologues génétiquement corrigées. La faible disponibilité et la difficulté d’amplification in vitro des hépatocytes adultes nous a conduit à explorer la piste des cellules souches pluripotentes induites (iPSCs) pour produire des cellules hépatiques humaines utilisables en médecine régénérative. Nous avons dérivé et caractérisé des lignées de cellules iPSCs à partir de fibroblastes de patients atteints d’HP1, après expression transitoire des facteurs de reprogrammation par des vecteurs Sendai. Nous avons développé deux stratégies de thérapie génique additive par insertion d’un minigène codant une séquence optimisée de l’ADNc AGXT au moyen (1) d’un vecteur lentiviral à expression hépato-spécifique et (2) d’un processus de recombinaison homologue au locus AAVS1 facilité par le système de clivage ciblé de l’ADN « CRISPR/Cas9 ». Enfin, nous avons mis en évidence l’expression de la cassette thérapeutique après différenciation hépatocytaire des iPSCs génétiquement corrigées. Ces résultats ouvrent de nouvelles perspectives de médecine régénérative pour l’HP1 par transplantation de cellules hépatocytaires autologues génétiquement corrigées dérivées d’iPSCs de patients
Primary hyperoxaluria type 1 (or PH1) is an inherited metabolic disorder related to the deficiency of the hepatic AGT enzyme (alanine:glyoxylate aminotransferase), which is encoded by the AGXT gene. In PH1 patients, this deficiency leads to oxalate overexcretion by liver, followed by urine filtration and complexation with calcium to form massive calcium-oxalate nephrolithiasis potentially leading to chronic renal failure. The only available curative treatment is combined hepatorenal allogeneic engraftment, which is currently limited by the availability of transplant donors, significant morbidity and mortality, and the need for long-term immunosuppressive treatment. The aim of our research project is to develop gene therapy for PH1, consisting in engraftment of genetically corrected autologous liver cells. Considering that adult hepatocytes are hardly available and expandable in vitro, we chose to explore the use of induced pluripotent stem cells (iPSCs) to produce human liver cells for application in regenerative medicine. We derived and characterized iPSC lines from PH1 patient fibroblasts after transient expression of reprogramming factors delivered by Sendai virus vectors. We developed two additive gene therapy strategies by inserting a minigene encoding an optimized AGXT cDNA sequence using (1) a lentiviral vector designed for liver-specific expression and (2) homologous recombination process at the AAVS1 locus favoured by the targeted DNA cutting system “CRISPR/Cas9”. Finally, we highlighted therapeutic cassette expression after hepatic differentiation of genetically corrected iPSCs. These results pave the way for regenerative medicine for PH1 by transplantation of genetically modified autologous hepatocyte-like cells derived from patient-specific iPSCs

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Hildick, Keri. "Mechanisms underlying the trafficking and distribution of cannabinoid receptor type 1 in primary hippocampal neurons." Thesis, University of Bristol, 2013. http://ethos.bl.uk/OrderDetails.do?uin=uk.bl.ethos.654590.

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coupled receptors (GPCRs) in the central nervous system, where it is concentrated at the axonal plasma membrane of specific neurons. Whilst the , therapeutic and psychotropic effects of cannabinoid receptor stimulation have been recognised for over 4000 years, through recreational and medicinal Cannabis use, ,it is only in the last few decades that our scientific understanding of both exogenous and endogenous cannabinoid system regulation has significantly advanced. At the presynaptic terminal, CB1R activation mediates neurotransmission through retrograde messengers, which are released from the post-synaptic terminal in response to activity-dependent signal transduction. Consequently the CB1R can be considered as a master regulator of synaptic transmission. However, compared to other GPCRs, the field of CB1R trafficking is in its infancy and the pathways and mechanisms of CB1R regulation are at best, incomplete. Using N-terminal fluorescent-tagged CB1R constructs, I have investigated the trafficking and diffusional mobility of CB1Rs in cultured hippocampal neurons. My work has revealed a crucial role for the C-terminal tail of the CB1R (ctCB1R) in regulating both its plasma membrane dynamics and surface localization. Specifically, using truncation and deletion mutagenesis I identified a 21 amino acid stretch in the ct-CB1R, which is critical for maintaining its axonal polarized surface distribution in hippocampal neurons. This region corresponds to a putative helical motif, H9, for which no known functional role has previously been identified. Moreover, I have demonstrated that the ct-CB1R . is sufficient to promote an axonal fate, presumably through interactions with specific scaffolding and adaptor proteins. Accordingly, I have performed an unbiased proteomics analysis using affinity purification mass spectrometry and have identified several candidate interactors, including a cluster of proteins associated with clathrin-mediated 'endocytosis, which , provide additional mechanistic insight into the underlying biochemical machinery regulating CB1R distribution in neurons .

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Raker, Verena [Verfasser]. "Herpes simplex virus type 1 (HSV-1) infection alters growth factor signaling in primary cortical brain cells / Verena Raker." Hannover : Bibliothek der Tierärztlichen Hochschule Hannover, 2017. http://d-nb.info/1150340320/34.

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Loro, Emanuele Loro. "Normal myogenesis and increased apoptosis in myotonic dystrophy type-1 muscle cells." Doctoral thesis, Università degli studi di Padova, 2010. http://hdl.handle.net/11577/3423200.

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Myotonic dystrophy type 1 (DM1) is caused by (CTG)n expansion in the 3’-untranslated region of DMPK gene. Mutant transcripts are retained in nuclear RNA foci, which sequester RNA binding proteins thereby misregulating their functions (i.e. splicing regulation). Controversy still surrounds the pathogenesis of the DM1 muscle distress, characterized by myotonia, weakness and wasting with distal muscle atrophy. Eight primary human cell lines from adult-onset (DM1) and congenital (cDM1) patients, (CTG)n range 90-1800, were successfully differentiated into aneural-immature and contracting-innervated-mature myotubes. Morphological, immunohistochemical, RT-PCR and Western blotting analyses of several markers of myogenesis indicated that in vitro differentiation-maturation of DM1 myotubes was comparable to age-matched controls. In all pathological muscle cells, (CTG)n expansions were confirmed by long PCR and RNA fluorescence in-situ hybridization. Moreover, the DM1 myotubes displayed the splicing alteration of insulin receptor and MBNL1 genes associated to the DM1 phenotype. Considerable myotube loss and atrophy of 15-day-differentiated DM1 myotubes indicated activated catabolic pathways, as confirmed by the presence of apoptotic (caspase-3 activation, cytochrome c release, chromatin fragmentation) and autophagic (P62/LC3) markers. Treatment with the pancaspase inhibitor Z-VAD significantly reduced the decrease in myonuclei number and in average width in15-day-differentiated DM1 myotubes. We thus propose that the muscle wasting typical in DM1 is due to impairment of muscle mass maintenance-regeneration, through premature apoptotic-autophagic activation, rather than altered myogenesis.
La distrofia miotonica di tipo 1 (DM1) è causata dall'espansione (CTG)n nella regione trascritta ma non tradotta al 3' del gene DMPK. I trascritti mutati sono trattenuti in foci nucleari, i quali sequestrano diverse proteine leganti RNA spesso alterandone le funzioni (i.e. regolazione dello splicing). A livello del muscolo, i meccanismi patogenetici che portano a miotonia, debolezza e perdita di massa dei muscoli distali, non sono ad oggi chiari. Otto linee di mioblasti primari umani, ottenuti da biopsie di pazienti affetti da DM1 nelle forme adulta e congenita (range di espansione tra 90 e 1800 CTG), sono state differenziate ed innervate con successo, ottenendo miotubi in grado i contrarre. L'analisi morfologica e la quantificazione di diversi marker di miogenesi mediante RT-PCR e Western blotting, hanno indicato che il diferenziamento in vitro dei mioblasti primari DM1 è indistinguibile da quello ottenuto con mioblasti di controllo. In ciascuna linea DM1 è stata confermata l'espansione (CTG)n mediante long-PCR ed ibridizzazione in situ. Inoltre, nei miotubi DM1 è stato rilevata l'alterazione dello splicing del recettore per l'insulina e di MBNL1, caratteristica del fenotipo DM1. A 15 giorni di differenziamento, una considerevole perdita di miotubi DM1 ha suggerito l'attivazione di pathways catabolici, come confermato dalla presenza di marker di apoptosi (taglio proteolitico della caspasi 3, rilascio di citocromo c dai mitocondri, frammentazione della cromatina) e di autofagia (aumento dei livelli di LC3 lipidato e di P62). Il trattamento con l'inibitore delle caspasi Z-VAD si è dimostrato efficace nell'attenuare la riduzione del numero di mionuclei e del calibro medio dei miotubi a 15 giorni di differenziamento. Proponiamo quindi che la compromissione muscolare tipica della DM1 sia dovuta, più che ad un'alterata miogenesi, a problemi nei meccanismi di mantenimento/rigenerazione, che si esplicano attraverso la prematura attivazione di apoptosi e/o autofagia

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Villablanca, Andrea. "Genetic background of familial primary hyperparathyroidism /." Stockholm, 2003. http://diss.kib.ki.se/2003/91-7349-520-4/.

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Gaston, Andrea Michelle Marshall. "Adolescents with type 1 diabetes mellitus : an exploration of patients' and their primary caregivers' illness representations." Thesis, University of Leeds, 2004. http://ethos.bl.uk/OrderDetails.do?uin=uk.bl.ethos.410941.

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Книги з теми "Primary hyperoxaluria type 1"

Franco, Brunella. Oral-facial-digital type 1 syndrome. Edited by Neil Turner. Oxford University Press, 2018. http://dx.doi.org/10.1093/med/9780199592548.003.0319_update_001.

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This chapter discusses oral-facial-digital type 1 syndrome (OFD1), which represents a rare syndromic form of inherited renal cystic disease associated with dysfunction of primary cilia. The disease is transmitted as an X-linked dominant male lethal trait. Embryonic lethality in affected hemizygous males is usually reported in the first and second trimesters of pregnancy. The clinical spectrum for this disease includes malformation of the face, oral cavity, and digits with a high degree of phenotypic variability, even within the same family, possibly due to X-inactivation. Renal cystic disease is present in over 65% of adult cases and is usually observed in the second and third decades of life.

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Meiners, Antonia, Uwe Otto, Herman Melville, Wolfgang Condrus, Helmut Krauss, and Herbert Stass. Moby Dick, 1 Cassette. Patmos, 2002.

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Melville, Herman. Moby Dick: Part 1. Adamant Media Corporation, 2001.

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Dickens, Charles. Oliver Twist: Vol. 1. Independently published, 2020.

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Oliver Twist (centenary Cover 1). Penguin Random House, 2012.

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Melville, Herman. Moby Dick Readalong (Illustrated Classics Collection 1). American Guidance Service, 1994.

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Moriuchi, Hiroyuki. Human T-cell Lymphotropic Virus. Oxford University Press, 2018. http://dx.doi.org/10.1093/med/9780190604813.003.0010.

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Human T-cell lymphotropic virus type 1 (HTLV-1), a human retrovirus that infects an estimated 10–20 million people worldwide, has endemic foci in Japan, West and Central Africa, the Caribbean, Central and South America, and Melanesia. Also, it is the etiological agent of a lymphoproliferative malignancy, adult T-cell leukemia/lymphoma (ATLL), as well as chronic inflammatory diseases such as HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP). HTLV-1 can be transmitted vertically, sexually, or by blood-borne transmission. ATLL occurs in approximately 5% of carriers who are infected during early childhood, and primary prevention is the only strategy likely to reduce this fatal disease. Children born to carrier mothers acquire the virus predominantly from breastfeeding. In endemic areas, mother-to-child transmission (MTCT) can be significantly reduced by screening pregnant women for the HTLV-1 antibody, followed by replacing breastfeeding with exclusive formula feeding. Indications for serological screening and recommendations for prevention of perinatal transmission are reviewed in this chapter.

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Levy, David. Macrovascular complications, hypertension, and lipids. Oxford University Press, 2016. http://dx.doi.org/10.1093/med/9780198766452.003.0008.

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Premature vascular disease is common in Type 1 diabetes, especially in women and those with long duration. Many studies have identified early vascular involvement, using carotid Doppler and coronary artery calcification. Symptoms of coronary heart disease are often absent or muted, and the best methods for identifying occult coronary heart disease in Type 1 patients are not known. The concept of ideal cardiovascular health is valuable in planning preventive lifestyle and medical interventions. ‘Essential’ hypertension in young Type 1 patients is common, and reflects increased arterial stiffness. Hypertension is invariable in patients with any degree of albuminuria or renal impairment. Statin treatment in patients over 40 years old is recommended, but the evidence base is weak. Statins and ezetimibe are the only agents of prognostic value currently available for prevention of vascular events. Primary prevention with aspirin needs individual assessment. Insulin resistance/metabolic syndrome is frequent in Type 1 diabetes.

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Harrison, Mark. Errors. Oxford University Press, 2017. http://dx.doi.org/10.1093/med/9780198765875.003.0066.

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This chapter describes types of errors as applied to Emergency Medicine, and in particular the Primary FRCEM examination. The chapter outlines the key details of type 1 errors and type 2 errors. This chapter is laid out exactly following the RCEM syllabus, to allow easy reference and consolidation of learning.

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Bulterys, Marc, Julia Brotherton, and Ding-Shinn Chen. Prevention of Infection-Related Cancers. Oxford University Press, 2017. http://dx.doi.org/10.1093/oso/9780190238667.003.0066.

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This chapter discusses primary prevention measures that disrupt transmission of oncogenic infections. It begins by discussing vaccination against hepatitis B virus (HBV) and human papillomavirus (HPV), two major causes of cancer for which safe and effective vaccines are currently available. It briefly discusses the importance of treatment and prophylaxis against human immunodeficiency virus type 1 (HIV-1), which potentiates the virulence of other viral infections as well as directly increasing the incidence of non-Hodgkin lymphoma. It does not discuss the treatment of HBV or hepatitis C virus (HCV) infection, since these are considered in Chapters 25 and 33. Also beyond the scope of this chapter are the randomized clinical trials currently underway to assess the efficacy and feasibility of eradication of Helicobacter pylori (Chapters 24, 31), vaccination against Epstein-Barr virus (EBV) (Chapters 24, 26, 39), or the prevention of schistosomiasis and liver flukes (Chapters 24, 33, and 52).

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Частини книг з теми "Primary hyperoxaluria type 1"

Danpure, C. J., P. J. Cooper, P. R. Jennings, P. J. Wise, R. J. Penketh, and C. H. Rodeck. "Enzymatic Prenatal Diagnosis of Primary Hyperoxaluria Type 1: Potential and Limitations." In Studies in Inherited Metabolic Disease, 286–88. Dordrecht: Springer Netherlands, 1989. http://dx.doi.org/10.1007/978-94-009-1069-0_29.

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Danpure, C. J., and P. R. Jennings. "Deficiency of Peroxisomal Alanine: Glyoxylate Aminotransferase in Primary Hyperoxaluria Type 1." In Proceedings in Life Sciences, 374–78. Berlin, Heidelberg: Springer Berlin Heidelberg, 1987. http://dx.doi.org/10.1007/978-3-642-71325-5_40.

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Danpure, C. J., and P. R. Jennings. "Enzymatic Heterogeneity in Primary Hyperoxaluria Type 1 (Hepatic Peroxisomal Alanine: Glyoxylate Aminotransferase Deficiency)." In Studies in Inherited Metabolic Disease, 205–7. Dordrecht: Springer Netherlands, 1988. http://dx.doi.org/10.1007/978-94-009-1259-5_32.

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Raghavan, K. G., and K. V. Inamdar. "Role of Hydroxypyruvate in the Manifestation of Primary Hyperoxaluria L-Glyceric Aciduria Type-II." In Urolithiasis 2, 9–12. Boston, MA: Springer US, 1994. http://dx.doi.org/10.1007/978-1-4615-2556-1_2.

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Marangella, M., M. Petrarulo, C. Vitale, D. Cosseddu, and F. Linari. "Glycolate and Oxalate Plasma Levels and Renal Handling in Patients With Type 1 Primary Hyperoxaluria." In Urolithiasis 2, 79. Boston, MA: Springer US, 1994. http://dx.doi.org/10.1007/978-1-4615-2556-1_16.

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Ishikawa, K., T. Suzuki, T. Funai, K. Nishiyama, C. Uchida, and A. Ichiyama. "A liver enzyme, serine:pyruvate/alanine:glyoxylate aminotransferase and its mutant in a primary hyperoxaluria type 1 case." In Biochemistry of Vitamin B6 and PQQ, 337–41. Basel: Birkhäuser Basel, 1994. http://dx.doi.org/10.1007/978-3-0348-7393-2_53.

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Suzuki, Toshiaki, Kozo Nishiyama, Tsuneyoshi Funai, Keiji Tanaka, Akira Ichihara, and Arata Ichiyama. "Energy-Dependent Degration of a Mutant Serine:Pyruvate/Alanin: Glyoxylate Aminotransferase in a Primary Hyperoxaluria Type 1 C." In Intracellular Protein Catabolism, 137–40. Boston, MA: Springer US, 1996. http://dx.doi.org/10.1007/978-1-4613-0335-0_16.

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Thompson, G. N., P. Purkiss, and C. J. Danpure. "The Subcellular Metabolism of Glyoxylate in Primary Hyperoxaluria Type 1: The Relationship Between Glycine Production and Oxalate Overproduction." In Studies in Inherited Metabolic Disease, 212–14. Dordrecht: Springer Netherlands, 1988. http://dx.doi.org/10.1007/978-94-009-1259-5_34.

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Petrarulo, M., M. Marangella, D. Cosseddu, and F. Linari. "Primary Hyperoxaluria Type 2: Specific and Simple High-Performance Liquid Chromatographic Determination for L-Glyceric Acid in Body Fluids." In Urolithiasis 2, 145. Boston, MA: Springer US, 1994. http://dx.doi.org/10.1007/978-1-4615-2556-1_49.

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Watts, R. W. E., and M. A. Mansell. "Primary Hyperoxaluria." In Oxalate Metabolism in Relation to Urinary Stone, 65–81. London: Springer London, 1988. http://dx.doi.org/10.1007/978-1-4471-1626-4_5.

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Тези доповідей конференцій з теми "Primary hyperoxaluria type 1"

Mbeledogu, Chukwudumebi, Sally-Anne Hulton, Ashish Chikermane, Girish Gupte, Khalid Sharif, Evelyn Ong, Lauren Johansen, Indra Van Mourik, Chayarani Kelgeri, and Jane Hartley. "L6 Morbidity associated with primary hyperoxaluria type 1 (PH1) following liver transplantation: an aid for counselling of families." In Abstracts of the BSPGHAN Annual Meeting, 25–27 April 2022. BMJ Publishing Group Ltd, 2022. http://dx.doi.org/10.1136/flgastro-2022-bspghan.69.

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Yang, Xiaozhou, Yanyun Yang, Qin Cai, Ying Zhao, Ao Fang, Weiwei Hu, and Anshi Xu. "Primary experiments on 2-D and 1-D fiber-type optical phased array." In Asia Pacific Optical Communications, edited by Ken-ichi Kitayama, Pierpaolo C. Ghiggino, Kim Roberts, and Yikai Su. SPIE, 2008. http://dx.doi.org/10.1117/12.803108.

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Leicht, Douglas, and Kirk Olsen. "Comparison of 15-5PH Stainless Steel Type 1 versus Type 2 Fatigue Data for Aircraft Primary Structural Elements." In SAE 2015 AeroTech Congress & Exhibition. 400 Commonwealth Drive, Warrendale, PA, United States: SAE International, 2015. http://dx.doi.org/10.4271/2015-01-2613.

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Kosmider, Beata, Liudmila Vlasenko, Nathaniel Marchetti, Sudhir Bolla, Chenna Mandapati, Nathaniel Xander, Gerard Criner, and Karim Bahmed. "Abstract 500: Impairment of DNA double strand break repair in human primary alveolar type II cells in emphysema." In Proceedings: AACR Annual Meeting 2017; April 1-5, 2017; Washington, DC. American Association for Cancer Research, 2017. http://dx.doi.org/10.1158/1538-7445.am2017-500.

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Huang, Jianguo, Mark Chen, Melodi J. Whitley, Hsuan-Cheng Kuo, Andrea Walens, Yvonne M. Mowery, David V. Mater, et al. "Abstract 2810: Using CRISPR/Cas9 to generate primary soft tissue sarcoma in genetically engineered and wild-type mice." In Proceedings: AACR Annual Meeting 2017; April 1-5, 2017; Washington, DC. American Association for Cancer Research, 2017. http://dx.doi.org/10.1158/1538-7445.am2017-2810.

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Sharma, Anil K., and Rolf D. Hubmayr. "Matrix Stiffness Regulates Epithelial To Mesenchymal Transition In Rat Primary Type 1 Alveolar Epithelial Cells In Vitro." In American Thoracic Society 2012 International Conference, May 18-23, 2012 • San Francisco, California. American Thoracic Society, 2012. http://dx.doi.org/10.1164/ajrccm-conference.2012.185.1_meetingabstracts.a5565.

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Cremolini, Chiara, Rosa Berenato, Federica Morano, Roberto Moretto, Federica Perrone, Elena Tamborini, Daniele Rossini, et al. "Abstract LB-238: Dissecting primary resistance to anti-EGFR monoclonal antibodies (anti-EGFRs) inRASandBRAFwild-type (wt) metastatic colorectal cancer (mCRC)." In Proceedings: AACR Annual Meeting 2017; April 1-5, 2017; Washington, DC. American Association for Cancer Research, 2017. http://dx.doi.org/10.1158/1538-7445.am2017-lb-238.

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Boros, Ildiko´, Attila Aszo´di, and Ga´bor Le´gra´di. "Numerical Investigation of Thermal Stratification in the Primary Circuit of VVER-440 Type Reactors." In 14th International Conference on Nuclear Engineering. ASMEDC, 2006. http://dx.doi.org/10.1115/icone14-89529.

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Thermal stratification in the primary loops and in the connected pipes can limit the lifetime of the piping, or lead to penetrating cracks due to the stresses caused by the temperature differences and the cyclic temperature changes. Therefore it is essential to determine the thermal hydraulic parameters of the stratified flow. The determination of the affected pipes can be based on the international operational experience and on engineering consideration. The most affected pipes in PWRs are the pressurizer surge line, the injection pipe of the emergency core cooling systems and the feedwater injection pipe of the steam generators. CFD codes can provide an appropriate tool for the examination of the development and the breaking up of the stratification and the determination of the temperature distribution. However, the challenge of the uncertainty of the boundary conditions has to be faced because of the unknown flow circumstances. According to an extensive evaluation, performed in 1998 by the VEIKI, in the VVER-440/213 units of Paks NPP the most affected pipe is the pressurizer surge line [1]. To find out the possible thermal stratification in the surge line, a temperature monitoring system was installed on the YP20 leg of the surge line of the Unit 1 of the Paks NPP in 2000. The measurements showed that during the heat-up period there is a thermal stratification almost all time in the surge line [2]. The maximum temperature differences reach 140 K (140 °C). The surge line has been modeled with the CFD code CFX-5.7. The performed transient simulations confirmed the existence of a thermal stratification in the surge line, but showed permanent recirculation of colder coolant in the lower layer, caused by the asymmetric arrangement of the surge line legs and the asymmetric connection of the two legs to the main loop. In this paper, the surge line model and the results of the transient simulations are presented. The CFD model of the injection pipe of the high pressure Emergency Core Cooling System and the performed simulations for the analysis of occurrence of thermal stratification are presented as well.

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Noh, Byeong Jae, Wha Soo Kim, Sun Hong Kwon, and Jang Young Chung. "Comparative Wet Drop Experiments of Mark III and KC-1 for Membrane Type LNG Carriers." In ASME 2009 28th International Conference on Ocean, Offshore and Arctic Engineering. ASMEDC, 2009. http://dx.doi.org/10.1115/omae2009-79289.

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The increasing demand for safe storage and transportation of LNG (Liquefied Natural Gas) in the global energy market initiated a trend for further development of the design technology for LNG tanks. An LNG tank in LNG carriers or FLNGs (LNG-FPSO, LNG-FSRU) of the membrane type is constituted with primary and secondary metal alloy membrane barriers incorporated in insulation panels. One of the key technical issues associated with LNG carriers or FLNGs is the ability of the cargo containment system to withstand sloshing loads induced by ship motion in harsh environment. To assess the safety of membrane LNG tanks with a newly proposed configuration of CCS (Cargo Containment System) against sloshing, usually a comparative approach is adopted through numerical simulations and/or testing. The aim of the comparative approach is to assess the relative sloshing load and structural capacity under the fundamental assumption that the current membrane systems are safe. Over the period 2004-08, a JDP (joint development project) for an LNG CCS was carried out by Korea Gas Corporation (KOGAS) in corporation with major Korean shipyards and Korean universities. The aim of the project was to develop “a new CCS for membrane Type LNG carriers” which could lead to the improved and updated CCS of the structural safety. In this paper, experimental research results for the evaluation of structural performance of GTT MARK III type and KC-1 (Korean Cargo Containment System) insulation system, which is currently under development by KOGAS in corporation with major Korean shipyards, under hydro impact loads are presented. Based on the results of wet drop tests, a comparative evaluation was carried out for the time history of the impact pressure on the primary barrier and damage characteristics and so on. It is expected that the state-of-the art of engineering research described in this work can be directly applicable for the design and the development of high-valued LNG carriers and FLNGs.

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Patel, Sagar S., Ramesh Natarajan, and Rebecca L. Heise. "Mechanotransduction of Primary Cilia in Lung Adenocarcinoma." In ASME 2012 Summer Bioengineering Conference. American Society of Mechanical Engineers, 2012. http://dx.doi.org/10.1115/sbc2012-80435.

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Lung cancer causes more than 1 million deaths worldwide annually [1]. In a recent study by the American Cancer Society in 2011, more than 221,000 new cases of lung cancers were reported [2]. Out of these, the mortality rate was found in roughly 70% of the cases [2]. Lung cancer is divided into two major categories: small cell and non-small cell. In the United States, non-small cell lung cancer accounts for 85% of all lung cancers and is considered the most common type of lung cancer [2]. It is usually resistant to chemotherapy, therefore making it extremely difficult to treat [3]. Furthermore adenocarcinomas, a type of non-small cell lung cancer, occur towards the periphery of the lungs and are the most common type accounting for 40–45% of all lung cancer cases [3]. Epithelial cells in the healthy lungs undergo stresses during inhalation and expiration of normal breathing. In addition to the forces of normal breathing, lung cancer cells may also experience abnormal mechanical forces due to pre-existing lung diseases such as asthma, bronchitis and chronic obstructive pulmonary disease or other tumor associated structural changes. These conditions can significantly alter the structure of the lungs and cell phenotype [4]. The change in the structure of the lungs affects the mechanical environment of the cells. Changes in extracellular (ECM) stiffness, cell stretch, and shear stress influence tumorigenesis and metastasis [5]. One mechanism through which the cells sense and respond to the cellular mechanical environment is through the primary cilia [6–7]. Primary cilia are non-motile, solitary structures formed from the cellular microtubules and protrude out of each cell. They have also been shown to play an important role in facilitating common cancer signaling pathways such as Sonic Hedgehog and Wnt/β-catenin signaling [8–9]. The objective of this study was to test the hypothesis that lung cancer cells respond to mechanical stimuli with the formation of primary cilia that are necessary for 3 hallmarks of tumor progression: proliferation, epithelial mesenchymal-transition, and migration.

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Звіти організацій з теми "Primary hyperoxaluria type 1"

Wu, Xin. The efficacy and safety of anti-CD20 antibody treatments in relapsing multiple sclerosis: a systematic review and network meta-analysis. INPLASY - International Platform of Registered Systematic Review and Meta-analysis Protocols, June 2022. http://dx.doi.org/10.37766/inplasy2022.6.0075.

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Review question / Objective: The objectives of this systematic review were to evaluate the efficacy and safety of the three existing anti-CD20 antibodies for the treatment of relapsing multiple sclerosis and to aid clinicians in choosing medications. Eligibility criteria: We set the inclusion criteria as follows: (1) study type: RCT; (2) language restriction: only available in English; (3) participants: patients ≥18 years of age diagnosed with relapsing MS, whether with a relapsing–remitting course or a secondary progressive course; (4) intervention: anti-CD20 antibody treatments including ocrelizumab, ofatumumab, rituximab, and corresponding control including placebo and active treatments; (5) outcomes: clinical outcomes including annualized rate of relapse (ARR), the number of patients free of relapse, and the number of patients with confirmed disease progression (CDP); magnetic resonance imaging(MRI) outcomes including gadolinium-enhancing lesion change in T1, change in the volume of lesions on T2, the number of patients with no new or newly enlarged lesions in T2 and the brain volume change (BVC); safety outcomes including adverse events (AEs) and serious adverse events (SAEs). Included RCTs were not requested to supply all the outcomes mentioned above. We set the exclusion criteria as follows: (1) study type: retrospective studies, cohort studies, case reviews and case reports; (2) patients diagnosed with primary progressive MS.

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Splitter, Gary A., Menachem Banai, and Jerome S. Harms. Brucella second messenger coordinates stages of infection. United States Department of Agriculture, January 2011. http://dx.doi.org/10.32747/2011.7699864.bard.

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Aim 1: To determine levels of this second messenger in: a) B. melitensiscyclic-dimericguanosinemonophosphate-regulating mutants (BMEI1448, BMEI1453, and BMEI1520), and b) B. melitensis16M (wild type) and mutant infections of macrophages and immune competent mice. (US lab primary) Aim 2: To determine proteomic differences between Brucelladeletion mutants BMEI1453 (high cyclic-dimericguanosinemonophosphate, chronic persistent state) and BMEI1520 (low cyclicdimericguanosinemonophosphate, acute virulent state) compared to wild type B. melitensisto identify the role of this second messenger in establishing the two polar states of brucellosis. (US lab primary with synergistic assistance from the Israel lab Aim 3: Determine the level of Brucellacyclic-dimericguanosinemonophosphate and transcriptional expression from naturally infected placenta. (Israel lab primary with synergistic assistance from the US lab). B. Background Brucellaspecies are Gram-negative, facultative intracellular bacterial pathogens that cause brucellosis, the most prevalent zoonosis worldwide. Brucellosis is characterized by increased abortion, weak offspring, and decreased milk production in animals. Humans are infected with Brucellaby consuming contaminated milk products or via inhalation of aerosolized bacteria from occupational hazards. Chronic human infections can result in complications such as liver damage, orchitis, endocarditis, and arthritis. Brucellaspp. have the ability to infect both professional and non-professional phagocytes. Because of this, Brucellaencounter varied environments both throughout the body and within a cell and must adapt accordingly. To date, few virulence factors have been identified in B. melitensisand even less is known about how these virulence factors are regulated. Subsequently, little is known about how Brucellaadapt to its rapidly changing environments, and how it alternates between acute and chronic virulence. Our studies suggest that decreased concentrations of cyclic dimericguanosinemonophosphate (c-di-GMP) lead to an acute virulent state and increased concentrations of c-di-GMP lead to persistent, chronic state of B. melitensisin a mouse model of infection. We hypothesize that B. melitensisuses c-di-GMP to transition from the chronic state of an infected host to the acute, virulent stage of infection in the placenta where the bacteria prepare to infect a new host. Studies on environmental pathogens such as Vibrio choleraeand Pseudomonas aeruginosasupport a mechanism where changes in c-di-GMP levels cause the bacterium to alternate between virulent and chronic states. Little work exists on understanding the role of c-di-GMP in dangerous intracellular pathogens, like Brucellathat is a frequent pathogen in Israeli domestic animals and U.S. elk and bison. Brucellamust carefully regulate virulence factors during infection of a host to ensure proper expression at appropriate times in response to host cues. Recently, the novel secondary signaling molecule c-di-GMP has been identified as a major component of bacterial regulation and we have identified c-di-GMP as an important signaling factor in B. melitensishost adaptation. C. Major conclusions, solutions, achievements 1. The B. melitensis1453 deletion mutant has increased c-di-GMP, while the 1520 deletion mutant has decreased c-di-GMP. 2. Both mutants grow similarly in in vitro cultures; however, the 1453 mutant has a microcolony phenotype both in vitro and in vivo 3. The 1453 mutant has increased crystal violet staining suggesting biofilm formation. 4. Scanning electron microscopy revealed an abnormal coccus appearance with in increased cell area. 5. Proteomic analysis revealed the 1453 mutant possessed increased production of proteins involved in cell wall processes, cell division, and the Type IV secretion system, and a decrease in proteins involved in amino acid transport/metabolism, carbohydrate metabolism, fatty acid production, and iron acquisition suggesting less preparedness for intracellular survival. 6. RNAseq analysis of bone marrow derived macrophages infected with the mutants revealed the host immune response is greatly reduced with the 1453 mutant infection. These findings support that microlocalization of proteins involved in c-di-GMP homeostasis serve a second messenger to B. melitensisregulating functions of the bacteria during infection of the host.

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Kungwankiattichai, Smith, Ben Ponvilawa, Claudie Roy, Pattaraporn Tunsing, Florian Kuchenbauer, and Weerapat Owattanapanich. Maintenance with Hypomethylating Agents after Allogeneic Stem Cell Transplantation in Acute Myeloid Leukemia and Myelodysplastic Syndrome: A Systematic Review and Meta-Analysis. INPLASY - International Platform of Registered Systematic Review and Meta-analysis Protocols, November 2021. http://dx.doi.org/10.37766/inplasy2021.11.0078.

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Review question / Objective: P: Patients with AML or MDS after allo-SCT; I: Hypomethylating agents after allo-SCT; C: Observation after allo-SCT; O: Overall survival rates. Condition being studied: Hypomethylating agents (HMAs) seem to have a range of properties favorable to post-allogeneic hematopoietic stem cell transplantation (allo-SCT) maintenance in acute myeloid leukemia (AML) patients. This meta-analysis was performed to review all relevant studies to compare the outcomes of patients undergoing allo-SCT for AML or MDS receiving HMA maintenance therapy with observation only. Information sources: The systematic search of the Embase and MEDLINE databases identified 4,416 articles, from which 512 duplicates were removed. This resulted in 3,904 articles available for title and abstract review. Subsequently, 3,875 articles were excluded as the article type and study design did not fulfill the inclusion criteria, or there was no report on a primary outcome of interest. The remaining 29 articles underwent full-length review and 18 of those were excluded for the aforementioned reasons. Ultimately, the eligibility criteria for our meta-analysis were met by 11 studies: 2 RCTs, 1 prospective cohort study, and 8 retrospective cohort studies.

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Boyle, M., and Elizabeth Rico. Terrestrial vegetation monitoring at Fort Matanzas National Monument: 2019 data summary. National Park Service, May 2022. http://dx.doi.org/10.36967/nrds-2293409.

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The Southeast Coast Network (SECN) conducts long-term terrestrial vegetation monitoring as part of the nationwide Inventory and Monitoring Program of the National Park Service (NPS). The vegetation community vital sign is one of the primary-tier resources identified by SECN park managers, and it is currently conducted at 15 network parks (DeVivo et al. 2008). Monitoring plants and their associated communities over time allows for targeted understanding of ecosystems within the SECN geography, which provides managers information about the degree of change within their parks’ natural vegetation. 2019 marks the first year of conducting this monitoring effort at four SECN parks, including Fort Matanzas National Monument (FOMA). Nine vegetation plots, located on Anastasia and Rattlesnake Islands, were established at Fort Matanzas National Monument in June. Data collected in each plot included species richness across multiple spatial scales, species-specific cover and constancy, species-specific woody stem seedling/sapling counts and adult tree (greater than 10 centimeters [3.9 inches {in}]) diameter at breast height (DBH), overall tree health, landform, soil, observed disturbance, and woody biomass (i.e., fuel load) estimates. This report summarizes the baseline (year 1) terrestrial vegetation data collected at Fort Matanzas National Monument in 2019. Data were stratified across two dominant broadly defined habitats within the park (Maritime Upland Forests/Shrublands and Maritime Open Uplands). Noteworthy findings include: Eighty-two vascular plant taxa (species or lower) were observed across nine vegetation plots, including eight species not previously documented within the park. The most frequently encountered species in each broadly defined habitat included: Maritime Upland Forests and Shrublands: saw palmetto (Serenoa repens), yaupon (Ilex vomitoria), southern/eastern red cedar (Juniperus silicicola + virginiana), American beautyberry (Callicarpa americana), and American burnweed (Erectites hieraciifolius). Maritime Open Uplands: sea oats (Uniola paniculata), earleaf greenbriar (Smilax auriculata), and dixie sandmat (Euphorbia bombensis). ne non-native species, Brazilian pepper (Schinus terebinthifolia), categorized as invasive by the Florida Exotic Pest Plant Council (FLEPPC 2019) was encountered in one Maritime Upland Forest and Shrubland plot during this monitoring effort. There were not any rare plants tracked by the Florida Department of Agriculture and Consumer Services (FDACS 2020) found during this monitoring effort. All plants located in these monitoring plots are fairly common throughout Florida, as well as across the Southeast Coast. Three species observed, however, are on the FDACS 2020 list of commercially exploited plants within the state. These include saw palmetto, cinnamon fern (Osmundastrum cinnamomeum), and coontie (Zamia integrifolia var. umbrosa). Southern/eastern red cedar and cabbage palmetto (Sabal palmetto) were the most dominant species within the tree stratum of the Maritime Upland Forest and Shrubland habitat type. Species that dominated the sapling and seedling strata of this type included yaupon and cabbage palmetto. More than 75% of the trees measured in the parks Maritime Upland Forest and Shrubland habitat type were alive and experiencing healthy vigor. Of the 22 trees that were dead, more than 50% of those were southern/eastern red cedar. Most of those individuals that were observed with moderate or severe decline and greater than 50% dieback were southern/eastern red cedars. Although red bay (Persea borbonia) was identified as one of the “principal understory tree” species within Fort Matanzas National Monument’s maritime forests in 2004 (Zomlefer et al. 2004), tree-sized individuals were rarely detected on plots during this monitoring effort. This may be in part due to the detection of laurel wilt disease within St. Johns County in 2006 (USDA 2021). Based on the low detection...

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Boyle, Maxwell, and Elizabeth Rico. Terrestrial vegetation monitoring at Fort Pulaski National Monument: 2019 data summary. National Park Service, December 2021. http://dx.doi.org/10.36967/nrds-2288716.

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The Southeast Coast Network (SECN) conducts long-term terrestrial vegetation monitoring as part of the nationwide Inventory and Monitoring Program of the National Park Service (NPS). The vegetation community vital sign is one of the primary-tier resources identified by SECN park managers, and monitoring is currently conducted at 15 network parks (DeVivo et al. 2008). Monitoring plants and their associated communities over time allows for targeted understanding of ecosystems within the SECN geography, which provides managers information about the degree of change within their parks’ natural vegetation. 2019 marks the first year of conducting this monitoring effort on four SECN parks, including Fort Pulaski National Monument (FOPU). Twelve vegetation plots were established at Fort Pulaski National Monument in August. Data collected in each plot included species richness across multiple spatial scales, species-specific cover and constancy, species-specific woody stem seedling/sapling counts and adult tree (greater than 10 centimeters [3.9 inches {in}]) diameter at breast height (DBH), overall tree health, landform, soil, observed disturbance, and woody biomass (i.e., fuel load) estimates. This report summarizes the baseline (year 1) terrestrial vegetation data collected at Fort Pulaski National Monument in 2019. Data were stratified across two dominant broadly defined habitats within the park (Maritime Tidal Wetlands and Maritime Upland Forests and Shrublands). Noteworthy findings include: Sixty-six vascular plant taxa were observed across 12 vegetation plots, including six taxa not previously known from the park. Plots were located on both Cockspur and McQueen’s Island. The most frequently encountered species in each broadly defined habitat included: Maritime Tidal Wetlands: smooth cordgrass (Spartina alterniflora), perennial saltmarsh aster(Symphyotrichum enuifolium), and groundsel tree (Baccharis halimifolia) Maritime Upland Forests and Shrublands: yaupon (Ilex vomitoria), southern/eastern red cedar (Juniperus silicicola + virginiana), and cabbage palmetto (Sabal palmetto). Four non-native species identified as invasive by the Georgia Exotic Pest Plant Council (GA-EPPC 2018) were found during this monitoring effort. These species (and their overall frequency of occurrence within all plots) included: Japanese honeysuckle (Lonicera japonica; 17%), bahiagrass (Paspalum notatum; 8%), Vasey’s grass (Paspalum urvillei; 8%), and European common reed (Phragmites australis; 8%). Two rare plants tracked by the Georgia Department of Natural Resources (GADNR 2013) were found during this monitoring effort. These include Florida wild privet (Forestiera segregata) and Bosc’s bluet (Oldenlandia boscii). Southern/eastern red cedar and cabbage palmetto were the most dominant species within the tree stratum of the maritime Upland Forest and Shrubland habitat type. Species that dominated the sapling and seedling strata of this type included yaupon, cabbage palmetto, groundsel tree, and Carolina laurel cherry (Prunus caroliniana). The health status of sugarberry (Celtis laevigata)—a typical canopy species in maritime forests of the South Atlantic Coastal Plain--observed on park plots appeared to be in decline, with most stems experiencing elevated levels of dieback and low vigor. Over the past decade, this species has been experiencing unexplained high rates of dieback and mortality throughout its range in the Southeastern United States; current research is focusing on what may be causing these alarming die-off patterns. Duff and litter made up the majority of downed woody biomass (fuel loads) across FOPU vegetation plots.

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Sukenik, Assaf, Paul Roessler, and John Ohlrogge. Biochemical and Physiological Regulation of Lipid Synthesis in Unicellular Algae with Special Emphasis on W-3 Very Long Chain Lipids. United States Department of Agriculture, January 1995. http://dx.doi.org/10.32747/1995.7604932.bard.

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Various unicellular algae produce omega-3 (w3) very-long-chain polyunsaturated fatty acids (VLC-PUFA), which are rarely found in higher plants. In this research and other studies from our laboratories, it has been demonstrated that the marine unicellular alga Nannochloropsis (Eustigmatophyceae) can be used as a reliable and high quality source for the w3 VLC-PUFA eicosapentaenoic acid (EPA). This alga is widely used in mariculture systems as the primary component of the artificial food chain in fish larvae production, mainly due to its high EPA content. Furthermore, w3 fatty acids are essential for humans as dietary supplements and may have therapeutic benefits. The goal of this research proposal was to understand the physiological and biochemical mechanisms which regulate the synthesis and accumulation of glycerolipids enriched with w3 VLC-PUFA in Nannochloropsis. The results of our studies demonstrate various aspects of lipid synthesis and its regulation in the alga: 1. Variations in lipid class composition imposed by various environmental conditions were determined with special emphasis on the relative abundance of the molecular species of triacylglycerol (TAG) and monogalactosyl diacylglycerol (MGDG). 2. The relationships between the cellular content of major glycerolipids (TAG and MGDG) and the enzymes involved in their synthesis were studied. The results suggested the importance of UDP-galactose diacylglycerol galactosyl (UDGT) in regulation of the cellular level of MGDG. In a current effort we have purified UDGT several hundredfold from Nannochloropsis. It is our aim to purify this enzyme to near homogeneity and to produce antibodies against this enzyme in order to provide the tools for elucidation of the biochemical mechanisms that regulate this enzyme and carbon allocation into galactolipids. 3. Our in vitro and in vivo labeling studies indicated the possibility that phosphatidylcholine (PC) and phosphatidylethanolamine (PE) are associated with desaturation of the structural lipids, whereas shorter chain saturated fatty acids are more likely to be incorporated into TAG. 4. Isolation of several putative mutants of Nannochloropsis which appear to have different lipid and fatty acid compositions than the wild type; a mutant of a special importance that is devoid of EPA was fully characterized. In addition, we could demonstrate the feasibility of Nannochloropsis biomass production for aquaculture and human health: 1) We demonstrated in semi-industrial scale the feasibility of mass production of Nannochloropsis biomass in collaboration with the algae plant NBT in Eilat; 2) Nutritional studies verified the importance algal w3 fatty acids for the development of rats and demonstrated that Nannochloropsis biomass fed to pregnant and lactating rats can benefit their offspring.

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Lundgren, Jonathan, Moshe Coll, and James Harwood. Biological control of cereal aphids in wheat: Implications of alternative foods and intraguild predation. United States Department of Agriculture, October 2014. http://dx.doi.org/10.32747/2014.7699858.bard.

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The overall objective of this proposal is to understand how realistic strategies for incorporating alternative foods into wheat fields affect the intraguild (IG) interactions of omnivorous and carnivorous predators and their efficacy as biological control agents. Cereal aphids are a primary pest of wheat throughout much of the world. Naturally occurring predator communities consume large quantities of cereal aphids in wheat, and are partitioned into aphid specialists and omnivores. Within wheat fields, the relative abilities of omnivorous and carnivorous predators to reduce cereal aphids depend heavily on the availability, distribution and type of alternative foods (alternative prey, sugar, and pollen), and on the intensity and direction of IG predation events within this community. A series of eight synergistic experiments, carefully crafted to accomplish objectives while accounting for regional production practices, will be conducted to explore how cover crops (US, where large fields preclude effective use of field margins) and field margins (IS, where cover crops are not feasible) as sources of alternative foods affect the IG interactions of predators and their efficacy as biological control agents. These objectives are: 1. Determine the mechanisms whereby the availability of alternative prey and plant-provided resources affect pest suppression by omnivorous and carnivorous generalist predators; 2. Characterize the intensity of IGP within generalist predator communities of wheat systems and assess the impact of these interactions on cereal aphid predation; and 3. Evaluate how spatial patterns in the availability of non-prey resources and IGP affect predation on cereal aphids by generalist predator communities. To accomplish these goals, novel tools, including molecular and biochemical gut content analysis and geospatial analysis, will be coupled with traditional techniques used to monitor and manipulate insect populations and predator efficacy. Our approach will manipulate key alternative foods and IG prey to determine how these individual interactions contribute to the ability of predators to suppress cereal aphids within systems where cover crop and field margin management strategies are evaluated in production scale plots. Using these strategies, the proposed project will not only provide cost-effective and realistic solutions for pest management issues faced by IS and US producers, but also will provide a better understanding of how spatial dispersion, IG predation, and the availability of alternative foods contribute to biological control by omnivores and carnivores within agroecosystems. By reducing the reliance of wheat producers on insecticides, this proposal will address the BARD priorities of increasing the efficiency of agricultural production and protecting plants against biotic sources of stress in an environmentally friendly and sustainable manner.

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Rine, Kristin, Roger Christopherson, and Jason Ransom. Harlequin duck (Histrionicus histrionicus) occurrence and habitat selection in North Cascades National Park Service Complex, Washington. National Park Service, April 2022. http://dx.doi.org/10.36967/nrr-2293127.

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Harlequin ducks (Histrionicus histrionicus) are sea ducks that migrate inland each spring to nest along fast-flowing mountain streams. They are considered one of the most imperiled duck species in North America and occur in two distinct populations on the Atlantic and Pacific coasts. The Pacific coast population includes Washington State, where harlequin ducks breed in the Olympic, Cascade, and Selkirk Mountains. This species is designated as a Management Priority Species by the National Park Service within North Cascades National Park Service Complex (NOCA). This report summarizes harlequin duck surveys conducted during 15 years across a 27-year period (1990 and 2017) on major streams within NOCA, and incidental observations collected from 1968–2021. The primary objectives of these surveys were to 1) document the distribution and abundance of harlequin duck observations within NOCA boundaries, 2) describe productivity (number of broods and brood size), 3) describe breeding chronology of harlequin ducks, and 4) describe habitat characteristics of breeding streams. Sixty-eight stream surveys over 15 years resulted in observations of 623 individual harlequin ducks comprising various demographics, including single adults, pairs, and broods. In addition, we collected 184 incidental observations of harlequin ducks from visitors and staff between 1968–2021. Harlequin ducks were observed on 22 separate second- to sixth-order streams throughout NOCA across the entire 53-year span of data, both incidentally and during harlequin duck surveys by Park staff. Harlequin ducks were detected on 8 of the 13 streams that were actively surveyed. Excluding recounts, 88.7% (n = 330) of individual harlequin duck observations during surveys occurred in the Stehekin River drainage. Between all surveys and incidental observations, 135 unpaired females without broods were sighted across all NOCA waterways. Thirty-nine broods were recorded between NOCA surveys and incidental observations, with a mean brood size of 3.61 (± 1.44 SD; range = 2–10). Breeding pairs were recorded as early as April 5 and were seen on streams until June 15, a period of less than seven weeks (median: May 2), but most pairs were observed within a 3-week span, between April 26 and May 17. Single females (unpaired with a male, with (an)other female(s), or with a brood) were observed on streams between April 26 and August 25 (median: July 3), though most observations were made within a 5-week period between June 12 and July 19. Habitat data collected at adult harlequin duck observation sites indicate that the birds often used stream reaches with features that are characteristic of high-energy running water. While adults occupied all instream habitat types identified, non-braided rapids and riffles were used most frequently, followed by pools and backwaters. Larger instream substrate sizes (cobbles and boulders) were present at most observation sites. Adult harlequin ducks were more often found at locations that lacked visible drifting or lodged woody debris, but drift debris was a slightly more abundant debris type. The presence of gravel bars and at least one loafing site was common. Adult harlequin ducks were more often observed in association with vegetation that offered some cover over the channel, but not where banks were undercut. The average channel width at adult observation sites was 34.0 m (range: 6-80 m; n = 114) and 27.6 m (± 15.7 m; range: 10-60 m; n = 12) at brood observation sites. Compared to adult harlequin duck sites, broods were observed more frequently in low velocity habitat (pools, backwaters), but rarely in rapids. Cobble and boulder substrates were still the most dominant substrate type. Contrary to adult ducks, broods were observed most often observed in meandering stream channels, a morphology indicative of low gradient, low velocity stream reaches. Most broods were observed in stream reaches with gravel bars, loafing sites, and...

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Schiller, Brandon, Tara Hutchinson, and Kelly Cobeen. Cripple Wall Small-Component Test Program: Dry Specimens (PEER-CEA Project). Pacific Earthquake Engineering Research Center, University of California, Berkeley, CA, November 2020. http://dx.doi.org/10.55461/vsjs5869.

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This report is one of a series of reports documenting the methods and findings of a multi-year, multi-disciplinary project coordinated by the Pacific Earthquake Engineering Research Center (PEER) and funded by the California Earthquake Authority (CEA). The overall project is titled “Quantifying the Performance of Retrofit of Cripple Walls and Sill Anchorage in Single-Family Wood-Frame Buildings,” henceforth referred to as the “PEER–CEA Project.” The overall objective of the PEER–CEA Project is to provide scientifically based information (e.g., testing, analysis, and resulting loss models) that measures and documents seismic performance of wood-frame houses with cripple wall and sill anchorage deficiencies as well as retrofitted conditions that address those deficiencies. Three primary tasks support the earthquake loss-modeling effort. They are: (1) the development of ground motions and loading protocols that accurately represent the diversity of seismic hazard in California; (2) the execution of a suite of quasi-static cyclic experiments to measure and document the performance of cripple wall and sill anchorage deficiencies to develop and populate loss models; and (3) nonlinear response history analysis on cripple wall-supported buildings and their components. This report is a product of Working Group 4: Testing, whose central focus was to experimentally investigate the seismic performance of retrofitted and existing cripple walls. This present report focuses on non-stucco or “dry” exterior finishes. Paralleled by a large-component test program conducted at the University of California, Berkeley (UC Berkeley) [Cobeen et al. 2020], the present report involves two of multiple phases of small-component tests conducted at University of California San Diego (UC San Diego). Details representative of era-specific construction–specifically the most vulnerable pre-1960s construction–are of predominant focus in the present effort. Parameters examined are cripple wall height, finish style, gravity load, boundary conditions, anchorage, and deterioration. This report addresses all eight specimens in the second phase of testing and three of the six specimens in the fourth phase of testing. Although conducted in different testing phases, their results are combined here to co-locate observations regarding the behavior of all dry finished specimens. Experiments involved imposition of combined vertical loading and quasi-static reversed cyclic lateral load onto eleven cripple walls. Each specimen was 12 ft in length and 2-ft or 6-ft in height. All specimens in this report were constructed with the same boundary conditions on the top, bottom, and corners of the walls. Parameters addressed in this report include: dry exterior finish type (shiplap horizontal lumber siding, shiplap horizontal lumber siding over diagonal lumber sheathing, and T1-11 wood structural panels), cripple wall height, vertical load, and the retrofitted condition. Details of the test specimens, testing protocol (including instrumentation), and measured as well as physical observations are summarized. Results from these experiments are intended to support advancement of numerical modeling tools, which ultimately will inform seismic loss models capable of quantifying the reduction of loss achieved by applying state-of-practice retrofit methods as identified in FEMA P-1100 Vulnerability-Base Seismic Assessment and Retrofit of One- and Two-Family Dwellings.

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Ullman, Diane, James Moyer, Benjamin Raccah, Abed Gera, Meir Klein, and Jacob Cohen. Tospoviruses Infecting Bulb Crops: Evolution, Diversity, Vector Specificity and Control. United States Department of Agriculture, September 2002. http://dx.doi.org/10.32747/2002.7695847.bard.

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Objectives. The overall goal of the proposed research was to develop a mechanistic understanding of tospovirus evolution, diversity and vector specificity that could be applied to development of novel methods for limiting virus establishment and spread. Our specific objectives were: 1) To characterize newly intercepted tospoviruses in onion, Hippeastrum and other bulb crops and compare them with the known tomato spotted wilt virus (TSWV) and its isolates; 2) To characterize intra- and interspecific variation in the virus transmission by thrips of the new and distinct tospoviruses. and, 3) To determine the basis of vector specificity using biological, cellular and molecular approaches. Background. New tospoviruses infecting bulb crops were detected in Israel and the US in the mid-90s. Their plant host ranges and relationships with thrips vectors showed they differed from the type member of the Tospovirus genus, tomato spotted wilt virus (TSWV). Outbreaks of these new viruses caused serious crop losses in both countries, and in agricultural and ornamental crops elsewhere. In the realm of plant infecting viruses, the tospoviruses (genus: Tospovirus , family: Bunyaviridae ) are among the most aggressive emerging viruses. Tospoviruses are transmitted by several species of thrips in a persistent, propagative fashion and the relationships between the viruses and their thrips vectors are often specific. With the emergence of new tospoviruses, new thrips vector/tospovirus relationships have also arisen and vector specificities have changed. There is known specificity between thrips vector species and particular tospoviruses, although the cellular and molecular bases for this specificity have been elusive. Major conclusions, solutions and achievements. We demonstrated that a new tospovirus, iris yellow spot virus (IYSV) caused "straw bleaching" in onion (Allium cepa) and lisianthus necrosis in lisianthus (Eustoma russellianum). Characterization of virus isolates revealed genetic diversity among US, Brazilian, Dutch and Israeli isolates. IYSV was not seed transmitted, and in Israel, was not located in bulbs of infected plants. In the US, infected plants were generated from infected bulbs. The relationship between IYSV and Thrips tabaci was shown to be specific. Frankliniella occidentalis, the primary vector of many other tospoviruses, did not transmit IYSV isolates in Israel or the US. Furthermore, 1': tabaci populations varied in their transmission ability. Transmission was correlated to IYSV presence in thrips salivary glands. In Israel, surveys in onion fields revealed that the onion thrips, Thrips tabaci Lindeman was the predominant species and that its incidence was strongly related to that of IYSV infection. In contrast, in the U.S., T. tabaci and F. occidentalis were present in high numbers during the times sampled. In Israel, insecticides reduced onion thrips population and caused a significant yield increase. In the US, a genetic marker system that differentiates non-thrips transmissible isolates from thrips transmissible isolate demonstrated the importance of the M RNA to thrips transmission of tospoviruses. In addition, a symbiotic Erwinia was discovered in thrips and was shown to cause significant artifacts in certain types of virus binding experiments. Implications, scientific and agricultural. Rapid emergence of distinct tospoviruses and new vector relationships is profoundly important to global agriculture. We advanced the understanding of IYSV in bulb crops and its relationships with thrips vector species. The knowledge gained provided growers with new strategies for control and new tools for studying the importance of particular viral proteins in thrips specificity and transmission efficiency.

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