Journal articles on the topic 'Wright family History'

Eleven recently discovered letters in the Royal Library in Stockholm, written by Mamah Bouton Borthwick and Frank Lloyd Wright to Ellen Key, the Swedish social theorist and feminist reformer, between 1911 and 1914, shed new light on Key's influence, not only on the couple's image of themselves as radical reformers, but also on the design and concept of Taliesin, the house that Wright built as a residence, workshop, and retreat for them in 1913. These letters reveal that Borthwick, a client and neighbor of Wright's in Oak Park, discovered Key's writings soon after she and Wright abandoned their families and fled to Europe in 1909; from that point until August 1914, when Borthwick was murdered by a deranged servant at Taliesin, both she and Wright became avid disciples of Key's philosophy, and looked to her for guidance and support. It has long been known that Key's many publications on subjects such as marriage, divorce, birth control, children's education, and individual freedom, were read with interest by Wright and Borthwick, and that Borthwick was named Key's "only authorized translator" in English. The letters, analyzed in the context of close readings of Key's most significant and widely read texts, offer new insights into the meaning of Key's writings for the couple, revealing an explicit connection between Key's ideas and Taliesin, which Borthwick describes as having been "founded on Ellen Key's ideal of love." The texts provide further evidence of the feminist influence on Wright's emerging ideas about individual responsibility, artistic freedom, the family, and household life.

This article examines Frank Lloyd Wright's House on the Mesa project, which, despite its familiarity to most historians of twentieth-century architecture, has never been thoroughly studied within the general context of Wright's expansive oeuvre and the specific circumstances of the Museum of Modern Art's 1932 Modern Architecture: International Exhibition. Numerous drawings for the project survive in the Frank Lloyd Wright Archives at Taliesin West, although only photographic evidence survives of the original model. Scattered references to the project appear in Wright's writings, most notably his correspondence with wealthy Denver businessman George Cranmer, whose family served as a kind of inspirational muse for the architect. Of special importance is a letter from Wright to critic Lewis Mumford recently discovered in the Lewis Mumford Papers at the University of Pennsylvania. Handwritten on the back of a photograph of the project's model, Wright's letter sheds new light on some of the project's technical innovations, which included textile-block walls, cantilevered roofs, and stepped casements. Less a response to the International Style, as is commonly held, the project was Wright's model of individualized, machine-age luxury for a merit-based democracy.

Context: Peak expiratory flow rate (PEFR) is an effort-dependent parameter, emerging from the large airways within about 100-120 msec of the start of forced expiration. It measures the degree of obstruction in the airways. A child from an asthmatic family, having significantly low PEFR values than its height and age matched peers, can be considered under impending asthma category. Aims: The present study was proposed to detect early stages of airway obstruction in school going children. Settings and Design: Observational study conceived in the department of Physiology, AIIMS, Jodhpur, Rajasthan. Methods and Material: Hundred students of age group 7-15 years participated in the study. A family history for presence/absence of asthmatic symptoms was taken from all subjects. After anthropometric examination, PEFR values were recorded in standing position, using the Mini Wright Peak Flow meter after demonstrating them the right procedure. Three measurements were taken and the highest reading was recorded. Statistical Analysis Used: Fisher’s exact test was done to calculate the two tailed ‘P’ value, Odd’s ratio and relative risk. Results: Thirty-one Percent children with family history of airway obstruction showed PEFR values below 80% of the predicted value, while 5.74% who were not having any family history of asthma, also showed values below 80% of the predicted value. With Fisher’s exact test, the two tailed ‘P’ value was 0.0155 (significant). Conclusion: The results of this study support the vital role of PEFR related to changes in airflow, which eventually can result in early identification of children with airway obstruction.

Introduction This study has examined the impact of the World Health Organization's Research into Global Hazards of Travel (WRIGHT) Project's phase 1 report on the information given by airlines to their passengers regarding traveller's thrombosis. Methods Official websites of all airlines flying from Heathrow (UK) and John F Kennedy (USA) were located through links on the websites of these two busy international airports. In June 2007, each site was scrutinized by three independent researchers to identify if traveller's thrombosis and its risk factors were discussed and what methods of prevention were advised. This exercise was repeated a year after the publication of the WRIGHT report. Results One hundred and nineteen international airlines were listed in 2007 (12 were excluded from analysis). A quarter (27/107) of airlines warned of the risk of traveller's thrombosis. A year later, five airlines were no longer operational and there had been no increase in the discussion of traveller's thrombosis (23/102). Additional risk factors discussed in June 2007 versus September 2008: previous venous thromboembolism (16%, 15%); thrombophilia (14%, 15%); family history (11%, 9%); malignancy (12%, 14%); recent surgery (19%, 16%); pregnancy (17%, 16%) and obesity (11%, 12%). Prophylaxis advice given in June 2007 versus September 2008: in-flight exercise (34%, 42%); Hydration (30%, 34%); medical consultation prior to flying (20%, 18%); graduated compression stockings (13%, 12%); aspirin (<1%, <1%) and heparin (5%, 7%). Conclusions The majority of world airlines continue to fail to warn of the risk of traveller's thrombosis or offer appropriate advice. Alerting passengers at risk gives them an opportunity to seek medical advice before flying.

The Larkin Company of Buffalo, New York, was established in the 1870s as a small soap producer and grew to become a large mail-order house. Larkin's success could be attributed to a unique sales strategy created by Elbert Hubbard, called “The Larkin Idea,” which had as its motto, “From Factory-to-Family: Save All Cost Which Adds No Value.” The company sold its products exclusively through the mail to women in cooperative buying clubs. Employing a variety of marketing, advertising, and employee welfare practices, the Larkin Company built a unified corporate family of “Larkinites“—employees, customers, and executives. Larkin executives also hired architect Frank Lloyd Wright to construct a modern office complex, which became the physical representation of Larkin's culture. But changes in marketing, the departure and deaths of key executives, a seemingly anachronistic corporate culture, and poor business decisions combined to undermine the company in the mid-1920s, and by 1940 the company was virtually dead.

When and how did Barack Obama's now well-known “hope” mantra take shape? Carpio's essay explores this question through close readings of key passages from Obama's autobiography. It is nearly three hundred pages into the autobiography before the phrase “the audacity of hope” appears, at the end of the “Chicago” section. Obama has just been accepted to Harvard Law School and has yet to take his first trip to Africa to find his paternal family when he hears the phrase from his infamous ex-pastor, Jeremiah Wright. The essay places this moment from the “Chicago” section in the context of the entire autobiography to illuminate why, for Obama, it takes audacity to hope that we can transcend America's history of racial conflict. In the process, the essay reveals Obama's dark view of race relations in America before he became the symbol of a supposedly post-racial America that he is now.

Background. Brucellosis is one of the most common diseases of humans and animals and its clinical manifestations differ from asymptomatic infection to chronic illness associated with recurrence of symptoms. This study aimed to evaluate the prevalence of brucellosis in asymptomatic children 7 to 12 years old in Kahak, Iran.Methods. In this study, 186 children 7 to 12 years old were evaluated. Demographic data and exposure to the brucellosis agent were recorded and blood samples for the Wright, Coombs, and 2ME tests were collected. All the study subjects were followed up for one year about the appearance of symptoms.Results. The mean age was 10 ± 1.72 years and 51% were boys. Family history was positive for brucellosis in 15% of children. A total of 8 children were brucellosis seropositive and, in subsequent follow-up, 6 of them showed the disease symptoms.Conclusion. This study showed that approximately 4.3% of children in endemic areas can have asymptomatic brucellosis and many of these children may be symptomatic in short term.

Although the existence of Quakers in Virginia is well known, the best recent surveys of Virginia history devote only passing attention to them, mostly in the context of expanding religious freedoms during the revolutionary era. Few discuss the Quakers themselves or the nature of Quaker settlements although notably, Warren Hofstra, Larry Gragg, and others have studied aspects of the Backcountry Quaker experience. Recent Quaker historiography has reinterpreted the origins of the Quaker faith and the role of key individuals in the movement, including the roles of Quaker women. Numerous studies address Quaker women collectively. Few, however, examine individual families or women of different generations within a single family, and Robynne Rogers Healey has argued for “more biographies of less well-known Quaker women”. This essay uses a four-generation genealogical case study of the Quaker Bowater-Wright family to analyze the development of the Quaker faith in the Virginia backcountry and the lower South and its spread into the Old Northwest. In the backcountry environment, with its geographically isolated settlements and widely dispersed population, early Quaker migrants found fertile ground for both their economic and religious activities. The way of life that developed there differed significantly from the hierarchical Anglican structure of the Tidewater region and the more vocal evangelical groups with their independent congregational structure in the southern backcountry. This article argues that Quaker women played a critical role in shaping Quaker migration and institutional growth in eighteenth and nineteenth century America. It also suggests that the Quaker institutional structure reinforced family connections by creating a close bond that united southern Quakers across a great geographical area.

Between 2009 and 2011, Norwegian author Karl Ove Knausgård published a monumental novel project in six parts (over 3,500 pages) in which he described the minutiae of daily life: family troubles, ordinary routines, everyday discourse, drinking, strolling through town and so on. The literary project became a media sensation with translations in many languages, readers all over the Western world, and a lot of interviews and reviews to be found online. Why were the books so successful; what is it in them that engages readers? Drawing on theories of sociologist C. Wright Mills and philosopher Henri Lefebvre, this article argues that this ambitious as well as paradoxical literary project sheds light on the social and cultural position of the late modern subject in a European middle class. Knausgård in his self-narration creates an Everyman, while at the same time fashioning a self as an obsessed artist that is everything but ordinary. In a crucial part of the final book, Knausgård shows us Adolf Hitler as a bitter young man, but also as someone ‘whose youth resembles my own’. Here the self-positioning relates to ongoing European history as well as to the lack of historical perspective in our current age.

Abstract Background The misleading clinical presentation of brucellar sacroiliitis, which is usually confused with involvement of the lumbosacral hinge or the hip, is responsible for diagnostic and therapeutic delay. We aimed to study the epidemiological, clinical and therapeutic features of brucellar sacroiliitis. Methods We conducted a retrospective study including all patients hospitalized in the infectious disease department for brucellar sacroiliitis between 1992 and 2020. The diagnosis of brucellosis was based on positive wright agglutination test and/or positive blood cultures. Results We included 12 patients, among whom 8 were males. The mean age was 35±13 years. Ten patients consumed unpasteurized milk and 9 had a close contact with animals. Three patients were previously treated for brucellosis and 4 patients had a family history of brucellosis. The revealing symptoms were sacroiliac joint pain (7 cases) and low back pain (5 cases), associated with fever and night sweats (9 cases). There were 8 cases localized on the left side of the joint. Spondylodiscitis was associated with sacroiliitis in 3 cases and genitourinary brucellosis in one case. An accelerated erythrocyte sedimentation rate and elevated C-reactive protein levels were noted in 7 cases, anemia in 7 cases and leukopenia in 4 cases. X-ray examination of sacroiliac joints revealed thickening of the sacroiliac joint (3 cases). Bone scintigraphy, which was performed in 8 cases, showed hyperfixation of the sacroiliac joint. Sacroiliac computed tomography and magnetic resonance imaging, performed in 6 cases and 4 cases, respectively, showed signs of sacroiliitis in all cases and soft tissue abscess in 2 cases. Blood cultures were positive to Brucella in 2 cases. All patients received doxycycline and rifampicin, associated with trimethoprim/sulfamethoxazole in 2 cases. The median duration of treatment was 4.5 months [3-9 months]. The disease evolution was favorable in 10 cases. Sequelae represented by sacroiliac joint pain was noted in 4 cases. There were 2 relapsing cases. Conclusion The diagnosis of brucellar sacroiliitis is based mainly on the imaging results and serological testing. Respecting preventive measures is a priority in order to eradicate brucellosis. Disclosures All Authors: No reported disclosures

Wright's article has two themes running through it: a discussion of the meaning of De Manneville and a history of custody in England from medieval times onward set against historians' theories of family development. Comment on her article then is best divided into two parts. I begin with her wide-ranging history, for here she makes an indisputable contribution to women's history that needs only notice and emphasis.

1582 Background: Predictive genetic testing for familial cancer may alert people to highly elevated risk prior to disease onset. Genetic test reporting has been shown to improve uptake of prophylactic screening and procedures, but whether test reporting also promotes increased performance of primary preventive behavior is unknown. Methods: Unaffected adult participants ( N= 124) from high-risk melanoma families, ages 16-69 (mean = 35.24, 52% men) were enrolled. Participants from families that carried a CDKN2A/p16 mutation received a personal genetic test result and counseling about management recommendations whereas control participants from families without a CDKN2A/16 mutation received equivalent counseling and management recommendations based on family history alone. Photoprotection outcomes were compared between CDKN2A/p16 participants (31 carriers, 44 noncarriers) and the no-test control group ( n= 49), allowing the effects of receiving a genetic test result to be distinguished from the effects of counseling alone. Assessments were seasonally timed to capture tanning during the summer months. Melanin Index (MI) scores, measures of skin tanning obtained through reflectance spectroscopy, were assessed at the dorsal wrist and face. Tanning of the dorsal wrist and face were calculated by subtracting baseline MI scores at an unexposed site on the same individual. Results: Multilevel model analyses examined changes in tanning over time while controlling for clinician-rated skin type, age, gender, date of assessment, and group differences in phenotypic factors and family medical history. Participants who received positive test results were significantly less tan at the wrist one year after their previous summer baseline ( b= -.11, p< .001). No-test controls and noncarriers had no change in tanning. The magnitude of decrease in tanning measurements observed among CDKN2A/ p16 carriers approximated one skin type. Facial tanning did not differ from baseline for any group. Conclusions: A positive melanoma genetic test result promotes reduced UVR exposure. Future research should examine why positive genetic test reports were more motivating to patients than equivalent counseling based on family history.

AbstractBackground:This study aimed to assess determinants of anthropometric measures in a nationally representative sample of Iranian children and adolescents.Methods:This nationwide study was conducted among 13,280 students, aged 6–18 years, who were randomly selected from 30 provinces in Iran. Anthropometric measures were determined by calibrated instruments. Demographic and socio-economic (SES) variables, lifestyle behaviors, family history of chronic disease and prenatal factors were studied, as well. A hierarchical Bayesian tri-variate analysis was used to assess the factors associated with obesity measures of the body mass index (BMI), waist-to-height ratio (WHtR) and wrist circumference (WrC).Results:The results showed that the BMI was associated with SES score, family history of obesity, family history of diabetes mellitus, physical inactivity, screen time, duration of sleep, breakfast consumption, birth weight, breastfeeding, junk food and place of residence (urban-rural). All these factors were also significantly associated with WrC except for consumption of junk food. Many of these factors had a partial but significant relationship with WHtR.Conclusions:Various factors contribute to obesity. Preventive and educational programs on manageable factors such as increasing physical activity, eating breakfast and limiting TV or screen time could be helpful in controlling obesity in schoolchildren and reducing associated complications.

Lichen striatus is a benign, self-limited linear dermatosis of unknown etiology seen primarily in children. We report here the unusual simultaneous occurrence of this condition in siblings, a manifestation which may offer insight into the pathogenesis of this condition. CASE REPORTS Patient 1 A 2-year-old Japanese girl had a nonpruritic 0.5 x 5 cm hypopigmented band located on the right wrist and forearm, which had been present for several weeks. The past medical history was unremarkable, but family history was notable for atopic disease: asthma in a cousin and allergic rhinitis in her mother. A clinical diagnosis of lichen striatus was made, and the patient was observed without treatment.

We present a case of carpal tunnel syndrome caused by systemic tumoral calcinosis. A 54-year-old woman experienced hand numbness that persisted for nine months. She had no family history or medical problem. A plain radiograph of her right wrist showed a calcified mass on the volar side of the wrist joint. The patient also experienced pain in her contralateral wrist joint and both right and left shoulders, which had calcification on radiography around the joint. Her condition was diagnosed as carpal tunnel syndrome caused by systemic tumoral calcinosis and a resection biopsy was performed. Histopathological analysis of the biopsied specimen showed basophile deposition inside the fibrous connective tissue. At 14 months after the treatment, she no longer had pain or numbness in her fingers and there was no recurrence of the mass. This patient’s condition is considered as a case of nonfamilial, systemic primary tumoral calcinosis. Since incomplete resection leads to a recurrence of the lesion, a follow-up radiography examination is needed to monitor symptom recurrence.

Background:Reactive arthritis is a form of spondyloarthritis with aseptic joint involvement occurring after a gastrointestinal or urogenital infection. Most commonly associated with Chlamydia trachomatis, Salmonella, Shigella, Campylobacter, and Yersinia. Syphilis is an infection caused by the spirochete Treponema pallidum and is not usually associated with reactive arthritis. Syphilis is a great imitator of other diseases due to its broad presentation including painless chancre, constitutional symptoms, adenopathy, rash, synovitis, neurological and ocular findings.Objectives:To discuss a patient who presented with symptoms of rheumatoid arthritis (RA) but was later diagnosed with syphilis.Methods:31 year old male, former tobacco smoker, referred to Rheumatology for sudden onset joint pains, elevated anti-cyclic citrullinated peptide (anti-CCP), and elevated inflammatory markers. He reported pain in bilateral wrists, fingers, and right elbow for 6 weeks. Associated with 45 minutes of morning stiffness and new onset lower back pain without stiffness. He denied trauma, fever, chills, skin rash, dysuria, or diarrhea. Initiated trial naproxen 500mg twice a day only to have minimal relief. Patient is sexually active with men and was recently diagnosed with oropharyngeal gonorrhea treated with azithromycin 4 months prior. All other STI screening including syphilis, gonorrhea, HIV were negative at that time. Patient is on emtricitabine and tenofovir disoproxil fumarate for HIV pre-exposure prophylaxis. He denied family history of immune mediated conditions. Exam was significant for mild synovitis of both wrists and bilateral 2nd metacarpophalangeal joints. Initial labs revealed weakly positive anti-CCP 21 (normal <20), sedimentation rate 64 (normal ESR 0-15 mm/hr), C-reactive protein 24 (normal CRP 0-10 mg/L), and negative RF, ANA, HLA B27. During a short trial of prednisone taper, there was temporary improvement in symptoms, however synovitis recurred upon completion. Hydroxychloroquine (HCQ) 200mg twice a day was started for possible RA and he was referred to Ophthalmology for baseline retinopathy screening. Incidentally, he developed right sided blurry vision 2 weeks after initiation of HCQ. He was diagnosed with panuveitis of the right eye with inflammation of the optic nerve head and prednisone 40mg daily was initiated for presumed ocular manifestation of RA. However, further workup of panuveitis revealed reactive Treponema pallidum antibody and RPR quantity 1:32. Prednisone was immediately discontinued and he was referred to the emergency department for possible neurosyphilis.Results:Lumbar puncture showed cerebral spinal fluid with 260 red blood cells, 1 white blood cell, 27mg/dL protein, 60mg/dL glucose, non reactive VDRL, reactive pallidum IgG antibody, and negative cultures. Meningitis and encephalitis panels were negative. Patient completed a 14 day course of IV penicillin G with complete remission of joint pain, visual symptoms, and normalization of anti-CCP, ESR, and CRP.Conclusion:This case highlights how syphilis may mimic signs and symptoms of RA including symmetrical small joint pain, morning stiffness, elevated inflammatory markers, and positive anti-CCP. Anti-CCP is >96% specific for RA but was a false positive in this patient. There have only been few reported cases noting positive anti-CCP with reactive arthritis. This is a rare case of reactive arthritis secondary to syphilis with resolution of symptoms upon treating the syphilis.References:[1]Carter JD. Treating reactive arthritis: insights for the clinician. Ther Adv Musculoskelet Dis. 2010 Feb;2(1):45-54.[2]Cohen SE, Klausner JD, Engelman J, Philip S. Syphilis in the modern era: an update for physicians. Infect Dis Clin North Am. 2013 Dec;27(4):705-22.[3]Singh Sangha M, Wright ML, Ciurtin C. Strongly positive anti-CCP antibodies in patients with sacroiliitis or reactive arthritis post-E. coli infection: A mini case-series based review. Int J Rheum Dis. 2018 Jan;21(1):315-321.Disclosure of Interests:None declared.

Abstract Objective To describe the utility of wrist circumference in the identification of cardiometabolic risk in overweight and obese children. Methods A cross-sectional study was conducted in the obesity clinic of a tertiary care referral hospital over a two year period. All children and adolescents aged 5–17 years with nutritional overweight and obesity were recruited. Data pertaining to chronological age, sex, risk factors and family history were collected. Clinical assessment of anthropometry: Weight, height, body mass index (BMI), Tanner’s stage, wrist circumference, blood pressure, waist circumference and triceps skinfold thickness done as per standard criteria. Biochemical assessment of blood glucose, serum insulin, lipid profile, and Homeostatic Model for Insulin Resistance performed after 12 h of fasting. Results We recruited 118 subjects (mean age 10.9 years, 71.1% males, 87.3% obese and 12.7% overweight); 30 (25.4%) had metabolic syndrome. The mean z scores of wrist circumference of children who presented with and without metabolic syndrome was 2.7 ± 0.8 and 2.1 ± 0.7, respectively (p<0.05). We observed a fair positive correlation between wrist circumference z score and BMI z score, (r=0.5; p<0.05). On receiver operating characteristic curve analysis, 97th percentile of wrist circumference predicted metabolic syndrome among overweight and obese children with a sensitivity of 86.7% and specificity of 37.5% (AUC=0.675). Conclusions Wrist circumference with 97th percentile as a cut-off is a useful tool to identify metabolic syndrome amongst overweight and obese children and adolescents.

Abstract Objective: Polycystic ovary syndrome (PCOS) is linked with metabolic syndrome, which includes obesity, insulin resistance, dyslipidemia, and endocrine function in patients with PCOS. The aim of this study was to determine the prevalence of adolescent PCOS and characteristics associated with PCOS in adolescents in Babol, Iran. Materials and methods: A cross-sectional study was conducted on 1549 girl high school students aged 16–20 years who were living in Iran. After overnight fasting, blood samples were collected to determine hormones and lipid levels in PCOS patients between the first and fifth days of their menstrual period or withdrawal bleeding. Results: The overall prevalence of adolescent PCOS was 129 (8.3%) (95% CI; 4.0, 12.0). Irregular menstruation was observed in 24.1% of adolescents. A total of 68% of PCOS adolescents had insulin resistance. Compared with non-PCOS adolescents, the OR of adolescent PCOS for family history of hirsutism was 1.53 (95% CI, 1.06–2.20; p=0.024), family history of irregular menstrual cycle was 2.27 (95% CI, 1.56–3.30; p<0.001), menarche age <13 years was 1.95 (95% CI, 1.36–2.81; p<0.001), hirsutism was 1.14 (CI 95%, 1.10–1.17; p<0.001), acne was 5.10 (CI 95%, 3.52–7.40; p<0.001), and wrist circumference was 1.28 (CI 95%, 1.07–1.54; p=0.007). Conclusion: The presenting findings showed the high prevalence of PCOS among Iranian adolescents. Emphasis should be placed on clinical screening in adolescents with high-risk factors, including irregular menstrual, menarche <13 years, hirsutism, acne, high wrist circumference, and paternal obesity to help reduce the risk of developing metabolic disturbance and to find ways to improve long-term health.

Metaphors of family play a particular part in representing and justifying the public role of elite families and media empires in Mexico and the Philippines, two countries on opposite sides of the Pacific that feature linked histories of Spanish colonial heritage and intimate connections to the cultural and economic history of the modern United States. The media industries of Mexico and the Philippines share some important characteristics: powerful commercial television networks are operated by prominent elite family companies, whose multimedia empires wield political and economic influence nationwide. An industry model of elite family dominance is reflected in the ways that contemporary television programs, hosts, and viewers understand themselves as belonging to sorts of ‘television families’. The nature of Mexican and Philippine television industries as family businesses writ large merits more extensive comparative historical exploration. These parallel cases draw attention to how media may be productively compared and studied across the Pacific regions of Asia and the Americas.

This manuscript presents a report of bullous pemphigoid rash associated with COVID-19 for the first time. The objective of this manuscript is to present a unique dermatological case in the setting of a COVID-19-positive infection to further recognize the virus symptomatology. A 37-year-old female with a past medical history of class III obesity, type II diabetes mellitus, and hypertension presented to the emergency department in September 2020 with inpatient and outpatient follow-up through to November 2020. The patient denied any personal or family history of skin disorders. The patient tested positive for COVID-19 prior to hospitalization and presented to the hospital with severe, persistent, pruritic rash meeting dermatopathological, serologic, and clinical criteria for bullous pemphigoid diagnosis. Histopathology H&E punch biopsy from her left flexor wrist demonstrated epidermal keratinocyte necrosis, subepidermal vesiculation with eosinophils, gossamer stranding of the papillary dermis, and subepidermal edema. Direct immunofluorescence punch biopsy from her left flexor wrist demonstrated strong linear IgG staining at the dermoepidermal junction, with weaker and focal linear C3 staining. Antigen-specific serology was consistent with bullous pemphigoid. There was no previously reported cutaneous association of COVID-19 infection with bullous pemphigoid making this case an important addition to the body of evidence helping to identify bullous pemphigoid in the setting of viral infection.

The hawkmoth Manduca sexta and one of its preferred hosts in the North American Southwest, Datura wrightii, share a model insect–plant relationship based on mutualistic and antagonistic life-history traits. D. wrightii is the innately preferred nectar source and oviposition host for M. sexta. Hence, the hawkmoth is an important pollinator while the M. sexta larvae are specialized herbivores of the plant. Olfactory detection of plant volatiles plays a crucial role in the behavior of the hawkmoth. In vivo, the odorant receptor coreceptor (Orco) is an obligatory component for the function of odorant receptors (ORs), a major receptor family involved in insect olfaction. We used CRISPR-Cas9 targeted mutagenesis to knock out (KO) the MsexOrco gene to test the consequences of a loss of OR-mediated olfaction in an insect–plant relationship. Neurophysiological characterization revealed severely reduced antennal and antennal lobe responses to representative odorants emitted by D. wrightii. In a wind-tunnel setting with a flowering plant, Orco KO hawkmoths showed disrupted flight orientation and an ablated proboscis extension response to the natural stimulus. The Orco KO gravid female displayed reduced attraction toward a nonflowering plant. However, more than half of hawkmoths were able to use characteristic odor-directed flight orientation and oviposit on the host plant. Overall, OR-mediated olfaction is essential for foraging and pollination behaviors, but plant-seeking and oviposition behaviors are sustained through additional OR-independent sensory cues.

Background The field of dietary assessment has a long history, marked by both controversies and advances. Emerging technologies may be a potential solution to address the limitations of self-report dietary assessment methods. The Monitoring and Modeling Family Eating Dynamics (M2FED) study uses wrist-worn smartwatches to automatically detect real-time eating activity in the field. The ecological momentary assessment (EMA) methodology was also used to confirm whether eating occurred (ie, ground truth) and to measure other contextual information, including positive and negative affect, hunger, satiety, mindful eating, and social context. Objective This study aims to report on participant compliance (feasibility) to the 2 distinct EMA protocols of the M2FED study (hourly time-triggered and eating event–triggered assessments) and on the performance (validity) of the smartwatch algorithm in automatically detecting eating events in a family-based study. Methods In all, 20 families (58 participants) participated in the 2-week, observational, M2FED study. All participants wore a smartwatch on their dominant hand and responded to time-triggered and eating event–triggered mobile questionnaires via EMA while at home. Compliance to EMA was calculated overall, for hourly time-triggered mobile questionnaires, and for eating event–triggered mobile questionnaires. The predictors of compliance were determined using a logistic regression model. The number of true and false positive eating events was calculated, as well as the precision of the smartwatch algorithm. The Mann-Whitney U test, Kruskal-Wallis test, and Spearman rank correlation were used to determine whether there were differences in the detection of eating events by participant age, gender, family role, and height. Results The overall compliance rate across the 20 deployments was 89.26% (3723/4171) for all EMAs, 89.7% (3328/3710) for time-triggered EMAs, and 85.7% (395/461) for eating event–triggered EMAs. Time of day (afternoon odds ratio [OR] 0.60, 95% CI 0.42-0.85; evening OR 0.53, 95% CI 0.38-0.74) and whether other family members had also answered an EMA (OR 2.07, 95% CI 1.66-2.58) were significant predictors of compliance to time-triggered EMAs. Weekend status (OR 2.40, 95% CI 1.25-4.91) and deployment day (OR 0.92, 95% CI 0.86-0.97) were significant predictors of compliance to eating event–triggered EMAs. Participants confirmed that 76.5% (302/395) of the detected events were true eating events (ie, true positives), and the precision was 0.77. The proportion of correctly detected eating events did not significantly differ by participant age, gender, family role, or height (P>.05). Conclusions This study demonstrates that EMA is a feasible tool to collect ground-truth eating activity and thus evaluate the performance of wearable sensors in the field. The combination of a wrist-worn smartwatch to automatically detect eating and a mobile device to capture ground-truth eating activity offers key advantages for the user and makes mobile health technologies more accessible to nonengineering behavioral researchers.

Based on a recent Dutch national guideline, we propose a structured stepwise diagnostic approach for children with growth failure (short stature and/or growth faltering), aiming at high sensitivity for pathologic causes at acceptable specificity. The first step is a detailed clinical assessment, aiming at obtaining relevant clinical clues from the medical history (including family history), physical examination (emphasising head circumference, body proportions and dysmorphic features) and assessment of the growth curve. The second step consists of screening: a radiograph of the hand and wrist (for bone age and assessment of anatomical abnormalities suggestive for a skeletal dysplasia) and laboratory tests aiming at detecting disorders that can present as isolated short stature (anaemia, growth hormone deficiency, hypothyroidism, coeliac disease, renal failure, metabolic bone diseases, renal tubular acidosis, inflammatory bowel disease, Turner syndrome [TS]). We advise molecular array analysis rather than conventional karyotyping for short girls because this detects not only TS but also copy number variants and uniparental isodisomy, increasing diagnostic yield at a lower cost. Third, in case of diagnostic clues for primary growth disorders, further specific testing for candidate genes or a hypothesis-free approach is indicated; suspicion of a secondary growth disorder warrants adequate further targeted testing.

Flexor pollicis longus paralysis related to idiopathic anterior interosseous nerve syndrome is well known, but few reports exist on bilateral disease. A 24-year-old man with no personal or family history of neurological disease developed isolated total loss of active flexion of the right thumb’s interphalangeal joint after undergoing a wrist arthroscopy. Surgical exploration 5 weeks after onset showed flexor pollicis longus tendon to be intact; anterior interosseous nerve decompression was done with no abnormalities found. Because of persistent paralysis, electromyography was performed showing findings consistent with anterior interosseous nerve syndrome. After 7 months without recovery, the patient underwent tendon transfer. After 6 years, the patient presented with left-sided isolated flexor pollicis longus paralysis and electromyography indicated anterior interosseous nerve syndrome. Examination 9 months after onset showed persistent complete flexor pollicis longus paralysis but by 15 months spontaneous complete recovery had occurred. Anterior interosseous nerve syndrome can occur bilaterally and is likely to resolve completely without intervention but recovery may take longer than a year.

Family hypercholesterolemia (HSX) is a form of genetically deterministic increase in blood lipid levels associated with a high risk of cardiovascular disease, usually at a young age. HSX is a common genetic disease found in the general population in most countries in 1:500 people. Clinically xantomas are found in achilles tendor and wrist flexors, lipoid arc of the cornea, concentration of total cholesterol and low-density lipoproteins is 4.911.6 mmol/l. Gilberts syndrome is a hereditary benign hyperbilirubinium, associated with a decrease in the functional activity of the liver enzyme uridinfosfat-glucuronosil transferase. Clinically, this syndrome appers in intermittent jaundice, which is provoked by physical activity, consumption of alcoholic beverages, insulation and an increase in the level of indirect bilirubin within 20100 micromol/ml. The article presents a rare clinical case of genetic combination of HSC SSC and Gilbert syndrome a young patient has and discusses the elevated bilirubin levels protective role in the atherosclerosis progression in Gilbert syndrome.

Gout is a microcrystalline arthropathy resulting from the deposition of sodium urate microcrystals in the articular and periarticular tissues, and even in the extra-articular tissues. It affects mainly men and its mechanism is at the origin of a delay in diagnosis, and consequently its severe forms can occur. We report a case of tophaceous gout in a 59 year old adult, with no family history of gout. Gout, whose first attack was at the age of 40, was polyarticular and affected the left wrist, elbows, knees, ankles and the left big toe. The diagnosis was based on clinical signs (tophi at the elbows, ankles, hands, and auricles) and paraclinical signs ( hyperuricemia at 620 ʯmol/l With normal at150-360 ʯmol/l in women and 310-420 ʯmol/l in men, presence of sodium urate cristals in the puncture fluid of the right knee, double- contour image on ultrasonography) by the ACR/Eular classification criteria. The background treatment was based on 120mg of Febuxostat per day with a favorable follow-up marked by the regression of signs.

Background: Osteoporosis is a skeletal disease with a strong genetic background. The study aimed to identify the genetic determinants of early-onset familial osteoporosis and low bone mineral density (BMD) in a two-generation Maltese family. Methods: Fifteen relatives aged between 28–74 years were recruited. Whole genome sequencing was conducted on 12 relatives and shortlisted variants were genotyped in the Malta Osteoporotic Fracture Study (MOFS) for replication. Results: Sequential variant filtering following a dominant inheritance pattern identified rare missense variants within SELP, TGF-β2 and ADAMTS20, all of which were predicted to be likely pathogenic and participate in osteoimmunology. TGF-β2 c.1136C>T was identified in five individuals from the MOFS in heterozygosity, four of whom had osteopenia/osteoporosis at the lumbar spine and hip, and/or had sustained a low-trauma fracture. Heterozygosity for the ADAMTS20 c.4090A>T was accompanied by lower total hip BMD (p = 0.018) and lower total serum calcium levels in MOFS (p < 0.01), recapitulating the findings from the family. Women carrying at least one copy of the alternative allele (TC/CC) for SELP c.2177T>C exhibited a tendency for lower lumbar spine BMD and/or wrist fracture history relative to women with TT genotype. Conclusions: Our findings suggest that the identified variants, alone or in combination, could be causal factors of familial osteoporosis and low BMD, requiring replication in larger collections.

AbstractMulticentric carpotarsal osteolysis syndrome (MCTO) is a rare skeletal disorder caused by heterozygous mutations in the MAFB gene (v-maf musculoaponeurotic fibrosarcoma oncogene ortholog B). This is an autosomal dominant condition with a high frequency of sporadic cases. MCTO is characterized by osteolysis of the carpal, metacarpal, and tarsal bones beginning in early childhood with musculoskeletal rheumatologic symptoms such as pain and disability. Renal involvement can be seen in more than half of the patients; from ages 16 months to 42 years and manifests from proteinuria to end-stage renal failure requiring renal transplantation. The association of MAFB gene mutations with this genetic condition has aided in understanding the pathophysiology of the disease. We report here a 7-year-old Caucasian boy and his 33-year-old mother diagnosed with MCTO, with the boy having concomitant juvenile idiopathic arthritis. He was initially diagnosed with arthritis at age 5 years based on bilateral wrist synovial swelling, morning stiffness, and weakness with family history of his mother being diagnosed with erosive psoriatic arthritis leading to limb deformities. Initial therapy for the boy included methotrexate and infliximab with moderate response. Later, during the course of his disease, he underwent a genetic evaluation at age 7 years for history of learning disabilities and dysmorphic features. Maternal evaluation and radiographic examination led to a clinical diagnosis of MCTO in the mother, and subsequent testing for MAFB gene in the son revealed a mutation at c.206C > T (p.Ser69Leu), the most commonly reported genetic change in MCTO. Nevertheless, further imaging still confirmed ongoing arthritis, and therapy was adjusted based on its progression including abatacept, tocilizumab, and pamidronate. Our report highlights the possibility of concomitant inflammatory arthropathy in MCTO.

Aim: To determine the prevalence of hypocalcemia fits in exclusively breast fed infants. Study design: Prospective study. Place and duration of study: Department of Paediatric Medicine, Abbas Institute of Medical Sciences/Medical College Muzafarabad AJ Kashmir from 01-04-2021 to 31-03-2022. Methodology: One hundred breast fed infants were enrolled. A 0.5cc blood of child was withdrawn for conductance of serum calcium and 25(OH) vitamin D3 test while 2cc of mother blood was also withdrawn for analyzing their calcium levels as well as vitamin D status in duplicate batches. Radiological imaging through x ray image of wrist for detection of rickets was performed in each infant. Infants having complain of fits were specifically observed fit/seizure time duration of the fit, the time when it occurred, eye rotation and cyanosis condition. Any family history regarding epilepsy especially in mothers was also noted. Results: The mean age of the infants was 5.5±2.3 months with a range of 1-12 months. Majority of the infants were male gender with a parentage of 66% while there were 34% female gender infants. Within the total number of infants included 30 children were having clinically defined rickets with 25 such infants who were having vitamin D level below the level of 20ng/ml. Conclusion: There is a low prevalence of hypocalcemia fit among breast fed infants. Keywords: Prevalence, Hypocalcemic fits, Breast fed infants.

Abstract Introduction: Perfluoroalkyl substances (PFAS) like Perflouroctanoate (PFOA) and Perflurooctane sulfonate (PFOS) are ubiquitous environmental contaminants that have been in industrial use for many years. Many known adverse effects include malignancies, reproductive and thyroid dysfunction. However, there is limited literature regarding PFAS causing low bone density. We report a case of a premenopausal woman with a history of exposure to PFAS who was recently diagnosed with osteoporosis. Clinical Case: A 36-year-old lady with a history of hypothyroidism, on levothyroxine, presented to the orthopedics clinic with complaints of sudden onset right foot pain with no trauma. She was found to have a fracture of her right second metatarsal bone. Notably, over a period of five years she had suffered multiple fractures including metatarsal, elbow and a wrist fracture, all with minimal or no trauma. She denied smoking, alcohol, chronic steroid or PPI use, history of malabsorption, celiac disease, kidney stones, malignancy or liver problems. Her menstrual cycles were regular; she was on oral contraceptives in the past for dysmenorrhea. She is on Vitamin D supplementation and consumes adequate dairy products daily. There is no family history of hip fracture or osteoporosis. Labs showed: Calcium 8.9mg/dl, Phosphorus 2.4mg/dl (2.5–4.5mg/dl), intact PTH was 92.7pg/ml (8-97pg/ml), 24-hour urine calcium was undetectable. Vitamin D was 56.6ng/ml (30-100ng/ml). CBC, TSH, FSH, liver & kidney functions were all normal. Anti-endomysial, anti-gliadin and anti-tissue trans glutaminase antibodies were all unremarkable. IgA level was 362mg/dl (8–352 mg/dl). DXA scan revealed the lowest Z-score (-3.1) in the lumbar spine. She reported a history of exposure to PFAS with a blood level of 22.3ng/ml for PFOA and 48.4ng/ml for PFOS in the year 2005. Plan is to initiate bisphosphonate therapy for the treatment of osteoporosis. Discussion: PFAS are known endocrine disruptive agents that have been used widely in making a wide range of consumer products including nonstick and stain-resistant coatings of cookware, food containers. Recent studies suggest that serum PFAS concentrations were associated with lower bone density. There was a higher incidence of lower lumbar spine bone density in patients exposed to PFOS. PFOA is believed to compete with calcitriol at the same binding site on Vitamin D receptor resulting in changes in the osteoblasts thereby decreasing bone mineralization. Conclusion: There needs to be increased awareness about the association of low bone density in patients exposed to PFAS. This is especially important in the evaluation of premenopausal osteoporosis. References: 1. Environ Health Perspect. 2016 Jan;124 (1):81–7, 2. J Clin Endocrinol Metab 2014; 99(6) 2173–2180, 3. Sci Rep 2020 Oct 8;1091):16789

IntroductionGlucocorticoids are widely prescribed for a variety of diseases and are known to cause neuropsychiatric as well as somatic side effects.ObjectiveTo review the incidence, clinical characteristics, course and treatment of neuropsychiatric effects of glucocorticoids.AimWe have described the case of a 86-year-old woman. She had no personal and no psychiatric medical history in her family. She presented wrist arthritis requiring high doses of an oral corticoid treatment (prednisona 20 mg/d). After a week, she started with symptoms characterised by persecutory and surveillance delusions. Organicity was ruled out. The patient got a progressive recovery after starting anti-psychotic medication and progressive reduction of the steroid drugs.MethodsWe have performed a literature review of the neuropsychiatric complications of glucocorticoids using the PubMed database.ResultsNeuropsychiatric effects of glucocorticoids involve affective, behavioural, and cognitive manifestations. The incidence is variable, between 2 and 60% of patients who receive steroids. Although the effects of glucocorticoids are unpredictable, the administered dose is the most significant risk factor for the development of neuropsychiatric symptoms. Dosage reduction typically results in clinical recovery. Although the limited data on this subject, it is a problem that clinicians face on their regular basis. The administration of anti-psychotics or mood stabilizers may be beneficial in the prevention and treatment of this syndrome.ConclusionThe neuropsychiatric effects of glucocorticoids are unpredictable and non-specific. More controlled trials are needed in order to perform evidence-based clinical guidelines for the treatment with glucocorticoids and for the prevention of neuropsychiatric manifestations.Disclosure of interestThe authors have not supplied their declaration of competing interest.

Abstract Background: Werner syndrome (WS; MIM# 277700) is a rare genetic disorder characterized by accelerated aging and predisposition to cancers. The causal gene, WRN, encodes a multifunctional nuclear protein that belongs to the RecQ family of DNA helicases. The earliest sign of WS is short stature due to the lack of pubertal growth spurt. In addition to the aged-appearance, WS patients typically develop functional declines of multiple organs including the endocrine system in their middle ages. Frequent Endocrine disorders include Type 2 Diabetes (T2D), dyslipidemia (DL), hypothyroidism, hypogonadism and osteoporosis. Most common causes of deaths are myocardial infarction and cancers in their 50s. Clinical Case: A 54-year old man was admitted for right wrist septic arthritis. His medical history was pertinent for premature cataract diagnosed in his 2nd decade. He had uncontrolled T2D (peak HbA1C level 10%) since the age of 32 years, treated with oral hypoglycemics including glimepiride, sitagliptin and metformin then shifted to insulin over the past 2 years. He also had DL since the age of 41 years, characterized by hypertriglyceridemia (766 mg/dL) with very low HDL (7 mg/dL). At age 50, he was diagnosed with hypothyroidism and negative anti-TPO antibodies. Moreover, he had refractory bilateral leg ulcers that manifested at the age of 35 years requiring multiple debridments, premature coronary artery disease at the age of 38 years and ischemic cardiomyopathy. On examination, his height was 154 cm and weight 42 kgs. He had prominent eyes, a pinched nose, grey and sparse scalp hair with absence of eyebrows and eyelashes. Hoarse voice was easily noticeable. He had very thin limbs, flat feet, central fat distribution and muscle wasting. Skin was shiny, tight, and atrophic with overlying hypermelanosis. Old bilateral hyperkeratotic deep feet ulcers were noticed with variable necrosis. Blood chemistry testing showed: HbA1C 8.2%, disturbed lipid profile, TG 341 mg/dL, HDL 17 mg/dL, LDL 155 mg/dL, and TSH 21 µU/mL (0.2 - 4.2 µU/mL). MRI of the foot revealed Achilles’ tendon calcification. His parents were 2nd degree cousins and had normal features. Three of his siblings had similar features and premature death. His diabetes was atypical due to his low BMI, high insulin requirements and the relatively early onset. WS was suspected based on the overall features of accelerated aging with characteristic deep leg ulcers and the family history. Genetic sequence analysis revealed a novel pathogenic homozygous nonsense variant mutation c.1111G&gt;T, p.Glu371* of the WRN gene. Conclusion: Werner syndrome is a rare genetic disease, but it carries high morbidity and mortality burden, in addition to impaired quality of life. It should be highly suspected in patients with atypical T2D, DL, premature coronary disease, refractory leg ulcers with features of accelerated aging and short stature.

Abstract Introduction: Familial hypoparathyroidism is a rare cause of hypocalcaemia. We report a case of long-standing hypocalcaemia secondary to hypoparathyroidism caused by a novel GATA3 variant resulting in multiple organ involvement. Case: A 20 year old girl was referred to our bone metabolic clinic for hypocalcaemia. Her past medical history included Bechet’s disease, epilepsy and depression. She had bilateral sensorineural hearing loss and encephalitis as a child. She underwent right nephrectomy for an atrophic non-functioning kidney at the age of 16. Current medication included hydroxychloroquine, diazepam, oral calcium and cholecalciferol. It was noted that the hypocalcaemia dated back to 8 years, she denied any typical symptoms of hypocalcaemia but she did report visual and auditory hallucinations, fatigue and had low seizure threshold. She sustained recurrent fractures of her arm, elbow and wrist. Initial investigations: Corrected calcium 1.88 (2.20-2.60mmol/L), Phosphate 1.54 (0.80–1.50mmol/L), PTH 1.2 (1.6–6.9pmol/), 25-OH vitamin D 37 (50-120nmol/L). Myeloma screen, thyroid, renal and liver functions were all within the normal reference range. Other bone markers: Serum Procollagen Type 1 Amino Terminal Peptide was mildly raised at 82 (19-69ug/L), CTX 0.42 (0.1-0.5ug/L), 1,25 OH Vitamin D 29 (55-139pmol/L), 24,25-dihydroxyvitamin D was normal with normal 25:24,25 Dihyroxyvitamin D ratio at 18 normal. Bone density was in the normal range for her age. MRI of the brain was normal with no evidence of calcification. There was a family history of hypocalcemia in her estranged father. Subsequent genetic analysis showed a novel likely pathogenic GATA3 missense variant (c.961T&gt;C p.(Cys321Arg). She was started on alfacalcidol and achieved near normocalcemia with adjusted calcium levels of 2.18nmol/L. Conclusion: Pathogenic variants in the GATA3 gene are responsible for Hypoparathyroidism-deafness-renal dysplasia (HDR) syndrome. In our patient, a novel missense variant in GATA3, p.(Cys321Arg), has been detected. This variant disrupts one of four conserved cysteine residues within a zinc-finger domain, which is involved in DNA binding and is presumed to have a deleterious effect on protein function. Patients may have longstanding asymptomatic hypocalcaemia with atypical features hence genetic testing is recommended in patient with multi-organ involvement. Alfacalcidol successfully restored calcium homeostasis in this case.

Background:The prevalence of psoriasis (PsO) in children is estimated between 0.5-1% (1), and can be associated with muskuloskeletal involvement, although the prevalence and typology of such involvement is unknown. (2)Over the last years ultrasound associated with Doppler (PDUS) has become an important tool for evaluating joint involvement in children with Juvenile Inflammatory arthritis (JIA). Several studies have shown the high sensitivity of this technique for detecting joint involvement, as well as high acceptability, due to the lack of radiation or of sedation (3).Objectives:To evaluate the prevalence of joints and entheses involvement in children affected by PsO.Methods:Observational cross-sectional study aiming at evaluating 150 consecutive children (≤16 years) affected by skin PsO and addressed by a dermatologist. For each child a standardized clinical and ultrasound evaluation of joint and entheses is performed at the following bilateral sites: a) Entheses: quadriceps tendon, proximal and distal patellar ligament, Achilles tendon, plantar fascia and extensor elbow tendon), b) joints: metacarpophalangeal, proximal and distal interphalangeal, wrist, elbow, knee, ankle, and metatarsophalangeal. PDUS evaluation is performed by an independent examiner, blinded to the clinical assessment of each subject.Results:41 patients were included until now, where 9 patients presented some kind of symptom (painful joint or enthesis), 24 patients have family history of psoriasis and none of them had family history of psoriatic arthritis. Demographic and clinical characteristics are shown in table 1, whilst PDUS findings in Table 2.Table 1.Demographic and clinical characteristics:Total (n=41)Asymptomatic (n=32)Symptomatic (n=9)pMales22 (53.7%)18 (56.3%)4 (44.4%)NSAge9.4 ± 3.98.91 ± 3.511.3 ± 4.6NSPso duration (years)3.5 ± 3.42.9 ± 2.95.6 ± 4.5<0.05PASI5 ± 4.34.8 ± 4.55.7 ± 3.56NSBSA4.3 ± 3.94.47 ± 4.34.1 ± 2.26NSNail involvement25 (61%)19 (59.4%)6 (66.7%)NSPlaques psoriasis20 (48.8%)14 (43.8%)6 (66.7%)NSTJC ≥1, n/tot (%)9 (22%)0 (0)9 (100%)<0.001SJC ≥1, n/tot (%)2 (4.9%)0 (0)2 (22.2%)<0.05Entheseal pain, n/tot (%)6 (14.6%)0 (0)6 (66.7%)<0.001Dactylitis000NAPASI: Psoriasis area severity index. BSA: Body surface area. TJC: Tender Joint Count. SJC: Swollen Joint Count.Table 2PDUS Findings:PatientsTotal (n=41)Asymptomatic (n=32)Symptomatic (n=9)p≥1 ultrasound abnormality, n/tot (%)19 (46.3%)13 (40.6%)6 (66.7%)NS≥1 joint effusion, n/tot (%)11 (26.8%)9 (28.1%)2 (22.2%)NS≥1 synovitis, n/tot (%)3 (7.3%)1 (3.1%)2 (22.2%)NS≥1enthesitis, n/tot (%)7 (17.1%)3 (9.4%)4 (44.4%)<0.05≥1 tenosynovitis, n/tot (%)0 (0)0 (0)0 (0)NA≥1 nail with modified structure, n/tot (%)22 (53.7%)19 (59.4%)3 (33.3%)NSConclusion:This study shows that the presence of ultrasound abnormalities was higher in the symptomatic group and considering the fact that the most frequent inflammatory ultrasound finding was enthesitis, we might consider the enthesis as a possible landmark for developing juvenile psoriatic arthritis.References:[1]Michalek et al. J Eur Acad Dermatol Venereol, 2017[2]Petty et al. J Rheumatol, 2004[3]Buchmann et al. Radiol Clin N Am, 2004Acknowledgments:This research was conducted while Luis Coronel was a PARTNER FellowDisclosure of Interests:None declared

Abstract Abstract 1302 Poster Board I-324 Combined vitamin K-dependent (VKD) coagulation factor deficiency is an autosomal recessive bleeding disorder associated with defects in either the γ-carboxylase (GGCX) which carboxylates VKD proteins to render them active or the vitamin K epoxide reductase (VKORC1) which supplies the reduced vitamin K cofactor required for carboxylation. Such deficiencies are rare, and due to mutations within either gene. Of note some mutations within the GGCX gene have recently been found associated with the pseudoxanthoma elasticum (PXE) syndrome, suggesting a role for GGCX in skin development. We report a new case of combined VKD coagulation factor deficiency resulting from two mutations in the GGCX gene, and the first identified in a French child, who exhibited impaired function in hemostatic VKD factors. The propositus exhibited bleeding at sites of venipuncture at birth, and at the age of 3 months was admitted in emergency for spontaneous multiple hematomas of the chest and thighs; recently, at the age of 1 year, he exhibited a hematoma of the wrist following a casual fall. Coagulation and VKD factors were low [PT (Control/patient): 12.8/>100 sec; II : 3 % ; VII :2% ; X :3%] while the non-VKD factor V was normal (105%). Vitamin K infusion corrected the bleeding tendency as well as coagulation paramenters [PT (Control/patient): 12.8/14.8 sec; II :72% ; VII+X : 62%; factor V remained normal at 102%]. Family analysis revealed that both parents and one brother were unaffected, both clinically and biologically, but that a brother had died of an unexplained abdominal hemorrhage, in the neonatal period. Taken together the clinical family history is consistent with a recessive trait. No sign of PXE was found in the family. DNA sequence analysis of the propositus did not identify any mutations in the VKORC1 gene but revealed two new heterozygous mutations in the carboxylase gene: a G10233T transversion in exon 11 (G1565U in mRNA) that caused an W493C substitution and a C12078T transition in the last exon (15) (C2196U in mRNA) that caused a premature R704stop, presumably deleting the last 55 C-terminal amino acids. W493 is a highly conserved amino acid and its homozygous mutation (for S493) has recently been reported in a case of VKD coagulation factor deficiency associated with PXE. R704 mutation has never been reported previously. Family analysis showed that W493C was transmitted by the father, R704stop by the mother, that the deceased brother carried both mutations, while the unaffected brother carried neither. Mutational analysis was carried out by site-directed mutagenesis of the GGCX cDNA subsequently subcloned into the BacPak8 baculovirus-based vector, and GGCX was expressed in the virally-infected SF21 insect cell line. Protein expression level of the enzyme was assessed by western blotting, and enzymatic activity was evaluated by measuring the incorporation of [14]C-CO2 within the Boc-Glu-Glu-Leu-OMe peptide. No difference in expression level was detected for either mutant protein compared to the wild type protein. However, while no difference in molecular weight was seen between the W493C mutant and wild type on wertern blotting, the R704stop mutation generated a shorter form of carboxylase than wild type, consistent with the predicted shorter reading frame. W493C mutant exhibited a much lower activity than wild type, suggesting a functional role for W493. The activity of the R704stop mutant is currently being assessed, as well as the activity of the coexpressed mutants. Since PXE has been correlated with a W493S mutation, a role for the W493C mutation in future development of PXE in this young patient will be assessed during follow-up. Disclosures No relevant conflicts of interest to declare.

Introduction: Arthrogryposis multiplex congenita is not a disease but a term describing multiple congenital contractures. Etiological factors include neurological and primary myogenic diseases. This rare syndrome is present at birth and is characterized by reduced mobility of many joints. The contractures involve two or more joints with ankylosis. The accompanying musculature is hypoplastic, but multiple pterygia are also present. Arthrogryposis multiplex congenita is a heterogeneous group of disorders with the incidence rate of 6.2/100000 liveborn infants. The true incidence cannot be established, because many cases result in spontaneous miscarriages or stillbirth. More than 90% of cases are associated with birth defects. The cause of this syndrome is unknown. Many forms are not hereditary, though there are hereditary forms as well. Case report. This paper presents a case with arthrogryposis multiplex congenita. The pregnancy was not controlled regularly. During the pregnancy, oligohydramnion was detected. Due to contractures, labor ended is cesarean section. The child was born in the 34th week of gestation. Flexion and extension joint contractures were observed. Active and passive mobility of the afflicted joints was reduced. There was a limited motor function in the shoulder, elbow and wrist joints with a slight internal rotation of the shoulder joint and lower arm joints during pronation. The hips were subluxated; the feet were in equinovarus position and the fingers in ulnar deviation with partial syndactyly of the 4th and 5th fingers on the left hand. The infant had abnormal dermatoglyphics. The neck was short, and the 2nd and 3rd cervical vertebrae were fused. There was also a slight left-sided thoracic scoliosis. Trismus was present due to the existing ankylosis of the temporomandibular joint. The karyotype was normal. The serum creatinine phosphokinase was slightly elevated. The electromyographic picture indicate non-specific signs of myopathy. Discussion and conclusion. This is a case report of a "stiff joint syndrome". Due to the fact that data from the family history were unavailable, we could not establish the type of syndrome. However, heredity, growth and development at later age, as well as IQ, might significantly help in definite differentiation of this syndrome. In pregnancy, oligohydramnion should indicate more detailed ultrasonographic examination, as ankylosed joints can be detected in utero. .

THE AGE OF AI: Artificial Intelligence and the Future of Humanity by Jason Thacker. Grand Rapids, MI: Zondervan Thrive, 2020. 192 pages. Hardcover; $22.99. ISBN: 9780310357643. *There are not yet many books that engage with artificial intelligence theologically. Jason Thacker's The Age of AI: Artificial Intelligence and the Future of Humanity, written for a general audience, provides an important start to much-needed theological discussions about autonomous and intelligent technologies. As an early effort in this complex interdisciplinary dialogue, this book deserves credit for its initial exploratory efforts. Thacker's book also points to the larger and more complex territory requiring further exploration. *Thacker, creative director at the Ethics and Religious Liberty Commission of the Southern Baptist Convention and project lead for their "Artificial Intelligence: An Evangelical Statement of Principles," is eager to draw attention to the pervasive and disruptive presence of artificial intelligence in our lives. While some may be distracted by images of AI that are speculative--the utopian Commander Data or the dystopian Terminator--many have not given much thought to the actual forms of AI that are part of our lives already, such as recommendation systems and digital assistants. "AI is everywhere," Thacker says; "And we aren't prepared." To help the unprepared understand AI, Thacker provides an orientation to current AI developments and explores the wide-ranging impacts of these on self-understanding, medicine, family, work, war, privacy, and the future. Along the way, he recalls biblical wisdom about old moral problems and imperatives, such as what the Ten Commandments prohibit and what Micah 6:8 prescribes (doing justice, loving mercy, and journeying attentively with God). He also offers a number of familiar biblical assurances, such as not being afraid and trusting in God. *All of this is helpful, to an extent. Thacker's major conclusions about AI are that we should not let our creations--our artificial agents--supersede human agency, and that we should not place too much hope in technology, for it alone cannot save us. Both of these are important points, although neither is very controversial nor necessarily theological: transparency is called for in many AI ethical frameworks, and we are well into a period of technological disenchantment. *Thacker starts The Age of AI by asking two significant questions. First, what does it mean to be human? Thacker looks to Genesis 1, which states--three times--that God created humans in the image of God. Clearly, this is an important theological claim; it is also a very complex one. There are various interpretations of what it means to be created in the image of God, and this is only the first chapter of the biblical narrative. Thacker emphasizes a functional interpretation of Genesis 1: We are called to work to glorify God. Elsewhere, however, Thacker shifts to a more essentialist interpretation that emphasizes human dignity. He asserts that our dignity does not come from what we do and that "nothing in this world defines us" (p. 117). But what about the work we are called to do in and for the world? *Another challenge of beginning in Genesis 1 is what happens in Genesis 3--humanity's rebellion against God. Thacker claims that "the image of God in us was not lost" (p. 19), though he does not address the extent to which this image was corrupted. For Christians, what is most important is Jesus's redemption and transformation of that fallen image. What does the image of God in Christ, the new Adam, reveal about the future of humanity? *Questions raised by Thacker's answer to his first question carry over into his answer to his second question, what is technology (including AI)? For Thacker, technology itself is morally neutral: "What's sinful isn't the sword but how people choose to use it" (p. 20). Given Isaiah's eschatological image of swords beaten into plowshares, many would argue that the sword is part of a system of weaponry and warfare that is immoral and must come to an end. Going beyond Isaiah, Jacques Ellul concluded that the biblical city, as an image of the technological society, must ultimately be destroyed: the city is an autonomous, multi-agent system with a diabolical power that exceeds the power of the human agents who created it. (Ellul almost seems to suggest that there is something like a rogue AI in the Bible!) Ellul goes too far with this, missing the good in the city and the transformative power of new creation over sinful systems, but he rightly points to the deformative power of technology. Thacker acknowledges that technology profoundly changes us and our world, positively and negatively, but he seems to suggest that humans can easily remain in control of and essentially unchanged by it. *Thacker's emphasis on Genesis, "where everything began," appears to close off any discussion about evolution and its insights into the role of technology in our emergence as a species. Indeed, the archeological record reveals that the use of simple stone tools shaped ancient human bodies and brains. Technology not only preceded the arrival of Homo sapiens, it shaped our understanding of what a human being is in form and function. Furthermore, throughout human history, technology has continued to change us fundamentally. Consider, for example, Walter Ong's insight that the technology of writing restructured consciousness. From the perspective of evolution and cultural development, technologies have been shaping and changing what we are from the beginning. *Thacker critiques Max Tegmark and Yuval Noah Harari for conflating evolution and cultural development, but that misses their interest in how humans might continue to outrun natural selection through innovation--a path our species has been on for many millennia, at least since the agricultural revolution and the creation of the complex artificial environments we call cities. As controversial as they may be, Tegmark and Harari point to how a deeper historical and philosophical understanding of technology enables us to explore questions about the holistic transformation of humans and human agency. *Thacker's view of technology encourages pursuing "technological innovation to help push back the effects of the fall" (p. 70). He worries that we might be tempted to "transcend our natural limitations," although it is not clear how far we are permitted to push back against the corrupted creation. He also fears "the people of God buying the lie that we are nothing more than machines and that somehow AI will usher in a utopian age" (p. 182). Educating people to resist being reduced to the status of machines (or data or algorithms) should be a learning outcome in any class or discussion about AI. As for ushering in a utopian age, this is one way of describing (in a kingdom-of-God sense) the Christian vocation: participating with God in the new creation. And perhaps AI has a role in this. *Thacker is absolutely right that we need a foundational understanding of who we are and of what technology is, and his answers provoke a number of questions for further exploration. The Bible reflects a rich interplay between human technological and spiritual development, from Edenic agriculture through Babelian urban agencies. And, as a technology itself, the Bible participates in these developments through its origin, nature, and function to mediate divine agency that transforms human agency. The biblical narrative makes it clear that we are not going back to the primordial garden in Genesis; we are moving toward the eschatological city, New Jerusalem, imaged in Revelation--"and what we will be has not yet been revealed" (1 John 3:2). How we understand the relationship between technological transformation and the transformation of all things through the new creation deserves much more attention within Christian theology. *With AI, it is clear that we are facing an even more profound restructuring of our lives and world--and of our selves. Rather than looking back to the imago Dei corrupted in the beginning, Christians might find it more generative to look to the imago Christi. As N. T. Wright powerfully argues in History and Eschatology: Jesus and the Promise of Natural Theology (SPCK, 2019), the new creation inaugurated through the resurrection of Jesus provides a radically new perspective on creation. This includes us and our artificial creations. While Thacker believes "nothing will ever change fundamental aspects of the universe" (p. 168), some of us may imagine AI participating in the new creation. *For someone just beginning to think about AI and Christianity, The Age of AI might be a good place to start. But more needs to be read and written to explore the theological and technological questions this book raises. *Reviewed by Michael J. Paulus Jr., Dean of the Library, Assistant Provost for Educational Technology, and Director and Associate Professor of Information Studies, Seattle Pacific University, Seattle, WA 98119.

This paper concerns the study of the specifics of self-consciousness and self-identity of one of the indige-nous minorities of Siberia — the Teleuts, in different periods of their ethnic history from the mid. 18th century until the present time. Main forms of Teleuts’ identity are considered: national; class; ethnic; ancestral and local. The instances of using various ethnonyms, genonymums and class attributes by Teleuts are analyzed. Identity multi-variance is considered as a mechanism of minority self-preservation and adaptation to the continuous assimilative influence of the nonethnic majority. The paper is based on the archival sources and field materials of the author collected during the expeditions to Teleuts in 1978–2014. The field materials include samples of the folklore, writ-ten folk literature, records of biographies, family chronicles and narratives about other societies recorded by the author. It has been shown how Teleuts ideas about other societies adjusted in the course of their adaptation to the new social and cultural environment within the Russian state. The attention has been drawn to how the ethnic consolidation of the Teleuts in the 19th–20th cc. transformed the local self-consciousness and self-identity. The names associated with small territorial communities often acquired derogatory sense and transferred from endo- to exo-type. It has been deduced how the legal status of the ethnic group within the state influences development of their ethnic culture. Notably, the non-Russians status of the Teleuts brought to them some rights and privileges in terms of the land tenure, taxes and exemption from the compulsory military service. On one hand, this has been helping to strengthen the national identity of the Teleuts, but on the other hand, it facilitates their ethnic self-affirmation. Teleuts have always been proud with their non-Russians status within the Russian state. The ethnic status of the Teleuts in the post-Soviet period is protected by their official recognition in 1989 as a separate ethnic group and subsequent affiliation with the indigenous minorities of the North.

Background:Classification criteria for calcium pyrophosphate deposition disease (CPPD) will facilitate clinical research on this common crystalline arthritis. ACR/EULAR are jointly sponsoring development of CPPD classification criteria using a multi-phase process.Objectives:To report preliminary results from the first two phases of a four-phase process for developing CPPD classification criteria.Methods:CPPD classification criteria development is overseen by a 12-member Steering Committee. Item generation (Phase I) included a scoping literature review of five literature databases and contributions from a 35-member Combined Expert Committee and two Patient Research Partners. Item reduction and refinement (Phase II) involved a Combined Expert Committee meeting, discussions among Clinical, Imaging, and Laboratory Advisory Groups, and an item rating exercise to assess the influence of individual items toward classification. The Steering Committee reviewed the modal rating score for each item (range -3 [strongly pushes away from CPPD] to +3 [strongly pushes toward CPPD]) to determine items to retain for future phases of criteria development.Results:Item generation yielded 420 items (312 from the literature, 108 from experts/patients). The Advisory Groups eliminated items they agreed were unlikely to distinguish between CPPD and other forms of arthritis, yielding 127 items for the item rating exercise. Fifty-six items, most of which had a modal rating of +/- 2 or 3, were retained for future phases (see Table 1). As numerous imaging items were rated +3, the Steering Committee recommended focusing on imaging of the knee, wrist, and one additional affected joint for calcification suggestive of CPP crystal deposition.Conclusion:The ACR/EULAR CPPD classification criteria working group has adopted both data- and expert-driven approaches, leading to 56 candidate items broadly categorized as clinical, imaging, and laboratory features. Remaining steps for criteria development include domain establishment, item weighting through a multi-criteria decision analysis exercise, threshold score determination, and criteria validation.Table 1.Categories of items retained for future phases of classification criteria developmentAge in decade at symptom onsetAcute inflammatory arthritis (e.g. knee, wrist, 1st MTP joint*)Recurrence and pattern of joint involvement (e.g. 1 self-limited episode, >1 self-limited episode)Physical findings (e.g. palpable subcutaneous tophus*, psoriasis*)Co-morbidities and family history (e.g. Gitelman disease, hemochromatosis, familial CPPD)Osteoarthritis location and features (e.g. 2nd or 3rd MCP joint, wrist)Synovial fluid findings (e.g. CPP crystals present, CPP crystals absent on 1 occasion* or 2 occasions*, monosodium urate crystals present*)Laboratory findings (e.g. hypomagnesemia, hyperparathyroidism, rheumatoid factor*, anti-CCP*)Plain radiograph: calcification in regions of fibro- or hyaline cartilage+Plain radiograph: calcification of the synovial membrane/capsule/tendon+Conventional CT: calcification in regions of fibro- or hyaline cartilage+Conventional CT: calcification of the synovial membrane/capsule/tendon+Ultrasound: CPP crystal deposition in fibro- or hyaline cartilage+Ultrasound: CPP crystal deposition in synovial membrane/capsule/tendons+Dual-energy CT: CPP crystal deposition in fibro- or hyaline cartilage+Dual-energy CT: CPP crystal deposition in synovial membrane/capsule/tendon+*Potential negative predictor +Assessed in the knee, wrist, and/or 1 additional affected jointDisclosure of Interests:Sara Tedeschi Consultant of: NGM Biopharmaceuticals, Tristan Pascart: None declared, Augustin Latourte Consultant of: Novartis, Cattleya Godsave: None declared, Burak Kundaki: None declared, Raymond Naden: None declared, William Taylor: None declared, Nicola Dalbeth Speakers bureau: Abbvie and Janssen, Consultant of: AstraZeneca, Dyve, Selecta, Horizon, Arthrosi, and Cello Health, Tuhina Neogi: None declared, Fernando Perez-Ruiz: None declared, Ann Rosenthal: None declared, Fabio Becce Consultant of: Horizon Therapeutics, Grant/research support from: Siemens Healthineers, Eliseo Pascual: None declared, Mariano Andrés: None declared, Thomas Bardin: None declared, Michael Doherty: None declared, Hang Korng Ea: None declared, Georgios Filippou: None declared, John FitzGerald: None declared, Marwin Gutierrez: None declared, Annamaria Iagnocco: None declared, Tim Jansen Speakers bureau: Abbvie, Amgen, BMS, Grunenthal, Olatec, Sanofi Genzyme, Consultant of: Abbvie, Amgen, BMS, Grunenthal, Olatec, Sanofi Genzyme, Minna Kohler Speakers bureau: Lilly, Consultant of: Novartis, Frederic Lioté: None declared, Mark Matza: None declared, Geraldine McCarthy Consultant of: PK Med, Roberta Ramonda: None declared, Anthony Reginato: None declared, Pascal Richette: None declared, Jasvinder Singh Speakers bureau: Simply Speaking, Consultant of: Crealta/Horizon, Medisys, Fidia, UBM LLC, Trio health, Medscape, WebMD, Adept Field Solutions, Clinical Care options, Clearview healthcare partners, Putnam associates, Focus forward, Navigant consulting, Spherix, Practice Point communications, Francisca Sivera: None declared, Alexander So: None declared, Lisa Stamp: None declared, Janeth Yinh: None declared, Chio Yokose: None declared, Robert Terkeltaub Consultant of: Sobi, Horizon Therapeutics, Astra-Zeneca, Selecta, Grant/research support from: Astra-Zeneca, Hyon Choi: None declared, Abhishek Abhishek Consultant of: NGM Biopharmaceuticals.

BackgroundThe prevalence of type 2 diabetes mellitus (T2DM) and of cardiovascular disease (CVD) is believed to be higher among people with intellectual disability (ID) than in the general population. However, research on prevalence and prevention in this population is limited.ObjectivesThe objectives of this programme of work were to establish a programme of research that would significantly enhance the knowledge and understanding of impaired glucose regulation (IGR) and T2DM in people with ID; to test strategies for the early identification of IGR and T2DM in people with ID; and to develop a lifestyle education programme and educator training protocol to promote behaviour change in a population with ID and IGR (or at a high risk of T2DM/CVD).SettingLeicestershire, UK.ParticipantsAdults with ID were recruited from community settings, including residential homes and family homes. Adults with mild to moderate ID who had an elevated body mass index (BMI) of ≥ 25 kg/m2and/or IGR were invited to take part in the education programme.Main outcome measuresThe primary outcome of the screening programme was the prevalence of screen-detected T2DM and IGR. The uptake, feasibility and acceptability of the intervention were assessed.Data sourcesParticipants were recruited from general practices, specialist ID services and clinics, and through direct contact.ResultsA total of 930 people with ID were recruited to the screening programme: 58% were male, 80% were white and 68% were overweight or obese. The mean age of participants was 43.3 years (standard deviation 14.2 years). Bloods were obtained for 675 participants (73%). The prevalence of previously undiagnosed T2DM was 1.3% [95% confidence interval (CI) 0.5% to 2%] and of IGR was 5% (95% CI 4% to 7%). Abnormal IGR was more common in those of non-white ethnicity; those with a first-degree family history of diabetes; those with increasing weight, waist circumference, BMI, diastolic blood pressure or triglycerides; and those with lower high-density lipoprotein cholesterol. We developed a lifestyle educational programme for people with ID, informed by findings from qualitative stakeholder interviews (health-care professionals,n = 14; people with ID,n = 7) and evidence reviews. Subsequently, 11 people with ID (and carers) participated in pilot education sessions (two groups) and five people attended education for the feasibility stage (one group). We found that it was feasible to collect primary outcome measures on physical activity and sedentary behaviour using wrist-worn accelerometers. We found that the programme was relatively costly, meaning that large changes in activity or diet (or a reduction in programme costs) would be necessary for the programme to be cost-effective. We also developed a quality development process for assessing intervention fidelity.LimitationsWe were able to screen only around 30% of the population and involved only a small number in the piloting and feasibility work.ConclusionsThe results from this programme of work have significantly enhanced the existing knowledge and understanding of T2DM and IGR in people with ID. We have developed a lifestyle education programme and educator training protocol to promote behaviour change in this population.Future workFurther work is needed to evaluate the STOP Diabetes intervention to identify cost-effective strategies for its implementation.Trial registrationClinicalTrials.gov NCT02513277.FundingThe National Institute for Health Research Programme Grants for Applied Research programme and will be published in full inHealth Research Programme Grants for Applied Research; Vol. 5, No. 11. See the NIHR Journals Library website for further project information.

The Aulacidae is a small family of Hymenoptera that are parasitic on wood-boring beetles and wasps. They have a worldwide distribution but are generally poorly studied, particularly for the southern hemisphere. This study is part of a series that aims to describe the Australian fauna which is relatively species-rich compared with other regions. We describe 39 new Aulacus species from eastern mainland Australia and Tasmania: A. anici Jennings & Austin, sp. nov., A. aquilus Jennings & Austin, sp. nov., A. bamagensis Jennings & Austin, sp. nov., A. bashfordi Jennings & Austin, sp. nov., A. bicolor Jennings & Austin, sp. nov., A. boonanghiensis Jennings & Parslow, sp. nov., A. brabyi Jennings & Austin, sp. nov., A. broadi Jennings & Austin, sp. nov., A. burnsi Jennings & Austin, sp. nov., A. confusus Jennings & Parslow, sp. nov., A. dandenongensis Jennings & Austin, sp. nov., A. deansi Jennings & Austin, sp. nov., A. doddi Jennings & Austin, sp. nov., A. froggatti Jennings & Austin, sp. nov., A. glorious Jennings & Parslow, sp. nov., A. hackeri Jennings & Austin, sp. nov., A. insularis Jennings & Austin, sp. nov., A. jamberoo Jennings & Parslow, sp. nov., A. kittelae Jennings & Austin, sp. nov., A. kiwarrakensis Jennings & Austin, sp. nov., A. leai Jennings & Austin, sp. nov., A. mareebaensis Jennings & Austin, sp. nov., A. naumanni Jennings & Austin, sp. nov., A. nebo Jennings & Austin, sp. nov., A. neboissi Jennings & Austin, sp. nov., A. obcordellus Jennings & Parslow, sp. nov., A. pallidus Jennings & Austin, sp. nov., A. quickei Jennings & Austin, sp. nov., A. rieki Jennings & Austin, sp. nov., A. scitulus Jennings & Parslow, sp. nov., A. simsoni Jennings & Austin, sp. nov., A. smithi Jennings & Austin, sp. nov., A. tasmanicus Jennings & Austin, sp. nov., A. tiernyi Jennings & Austin, sp. nov., A. umbackae Jennings & Austin, sp. nov., A. walkeri Jennings & Parslow, sp. nov., A. warraensis Jennings & Austin, sp. nov., A. willamsi Jennings & Austin, sp. nov., and A. wrightae Jennings & Austin, sp. nov. We also redescribe 14 species: A. albimanus (Kieffer), A. aroueti (Girault), A. atriceps Kieffer, A. elegans (Kieffer), A. festivus (Kieffer), A. flavicornis (Kieffer), A. flavimanus (Kieffer), A. fuscicornis Cameron, A. longiventris (Kieffer), A. minutus Crosskey, A. pallidicaudis (Cameron), A. planiceps (Szépligeti), A. truncatus (Kieffer), and A. vespiformis (Kieffer). As well, A. nigriventris (Kieffer) is synonymised with A. albimanus (Kieffer) syn nov. This brings to 60 the total number of species known from Australia (71 for the Australian region), although one species, A. biroi (Szépligeti), remains incertae sedis. A key to Australian Aulacus species is presented, along with notes on taxonomic history and host relationships.

Background:Eye symptoms: myopia, prolapse of the upper eyelid, epiblepharon in the upper eyelid are small diagnostic criteria for joint hypermobility syndrome (JHS).There are few publications in the literature on the relationship between JHS and primary open-angle glaucoma (POAG).It is known that in the development of JHS, the distribution of collagen of types I and III with the predominance of collagen of type III is important, the latter is encoded by the COL3A1 gene. When using POAG in the connective tissue of the middle and deep layers of the sclera by the immunohistochemical method, intense focal accumulation of type I and III collagen was previously revealed, and in the layers of the sclera’s own substance, type III collagen, unusual for it.Objectives:To study articular and extraarticular clinical manifestations, instrumental, laboratory signs, as well as to conduct molecular genetic studies on the carriage of the Col3Al gene in patients with a diagnosis of POAG and compare them.Methods:Nine consecutive patients with an established diagnosis of POAG (burdened heredity by glaucoma) with arthralgia were sent for consultation to the V.A. Nasonova Research Institute of Rheumatology from the Moscow Helmholtz Research Institute of Eye Diseases. All patients are women, the average age is 56.7 ± 10.5 years, the average Beighton score - 4.86 ± 1.7, the mean value of the Westergren ESR - 11,8 ± 5.1 mm/h, CRPhs- 4.9 ± 9.4 mg / 1, all of them were seronegative for rheumatoid factor (RE) and ACCP. All patients responded to the JHS diagnosis according to the 1998 Brighton diagnostic criteria. DNA was isolated from the leukocyte fraction of venous blood using the Wizard DNA Purification Kit (Promega) according to the manufacturer’s instructions. The study of gene polymorphisms was performed by the method of minisequencing with subsequent time-of-flight mass spectrometry of a sample on a matrix (MALDI-TOF) in the clinical diagnostic laboratory of NPF L1TEX LLC using a standard protocol (Wise C.A., 2003).Results:9 patients had arthralgia, 8 - vertebralgia, 3 - myalgia. 2 had a history of wrist joint dislocation, 7 had flat feet (3 of them had Hallucis valgus), 5 had spondylosis and spondylolisthesis (protrusions and disc herniation according to MRI of the spine), and 4 had excessive skin and / or striate atrophy of skin. Extraarticular manifestations: mitral valve prolapse was detected in 3 patients (in 1 of them + atrial septal defect) with ultrasound of the heart, in 3 - descent of the internal organs (nephroptosis, uterine prolapse), in 4 - pronounced varicose veins of the lower extremities. All patients had a carrier state of the A allele identified by marker C.2092G> A and C allele c.2244T> C of the COL3A1 gene, and a family history of glaucoma. Identification of compliance with JHS diagnostic criteria and the presence of genetic factors (COL3A1 gene) in patients with POAG is of great scientific importance, since it confirms not only clinical associations, but also the genetic proximity of these two conditions. It is also difficult to overestimate the practical value, since patients with POAG need the help of a doctor in the treatment of their articular and other non-ophthalmological manifestations of JHS, and establishing a diagnosis of JHS will require a more thorough examination of the eyes in terms of detecting POAG, its treatment or prevention.Conclusion:The association of JHS, POAG and COL3A1 gene necessitates further study of the association of JHS and POAG: POAG as a clinical manifestation of JHS, on the one hand, and the role of JHS as a possible risk factor for the development of POAG - on the other hand.Table 1.Demographic, clinical and laboratory characteristics of patients with Schnitzler’s syndromePtsThe age (y)The age at onset (y)Diagnosis delay (y)ESR (<15 mm/h)СRP (<6g/l)M-gradient (g/l)AnakinraCanakinumabThe treatment duration (y)151404311077,1015253282240295,7000336293140447,80134585331001927,6110,556966249965,1110,5Abbreviations: Pts – patients, y- years, ESR – erythrocyte sedimentation rate, CRP – C-reactive proteinDisclosure of Interests:None declared

Introduction: Obesity defined as increased fatty mass is progressively rising in recently, even though its affects begins to all systems in childhood and adolescence periods, the most important morbidity and mortality reason of obesity is its effects on the cardiovascular system. Researches point out endothelial dysfunction and atherosclerosis as the reason of the cardiovascular system disease in obesity. The studies conducted on childhood period related to this subject are highly limited and the results of these are also controversial. Therefore in our study the effects of obesity on endothelial functions in children and adolescents was assessed by flow mediated dilation (FMD) method. In addition to that, effects of epidemiological, biochemical, hormonal and clinical features of cases to FMD were investigated. Material and method: A total number of 104 cases were cover in this study. Obese group (group 1) was consisted of 59 children whose body mass index (BMI) was ≥ 95th percentile and mean age was 12 ± 2.8 years old. The control group (group 2) consisted of 45 children whose body mass index (BMI) was between 25th -84th percentil and mean age was 11.4 ± 2.9 years old. The detailed history, epidemiological data and physical examination were performed. The population classified three groups according to sport activities. 97th percentile and higher values were accepted as morbid obesity. The blood pressure was measured with a mercury sphygmomanometer with utilizing the proper size cuff in compliance with the criterion used by the “National High Blood Pressure Education Program Working Group”. The complete blood count and biochemistry tests (renal and liver function tests, electrolytes, lipids, hsCRP) of the cases were analysed with biochemistry Roche Cobas Integra 800 and hormon assays of the cases (thyroid function tests, diurnal cortisol, ACTH, 17 OHP, prolactin, DHEA-S) were analysed by ECLIA method on Roche Elecsys 2010 device in the laboratory of our hospital. IR-HOMA values > 2.5 in prepuberal and > 4 in pubertal were defined as the insulin resistance. Bone ages of cases were evaluated with left hand wrist X-ray by using Greulich and Pyle Bone Age Atlas. flow mediated dilation (FMD) was used to assess the endothelial functions of all cases. The brachial artery was evaluated with SPG 12 MHz surface probes by using GE voluson ultrasound system in this method. FMD was expresses as percent (%) increase according to the basal vein dimension. 7% mean value was taken as the limit in the comparisons. Results: The ratio of male and female was 20/39 in group 1 and 14/31 in group 2. 32.3% of the cases in group 1 and 47.6% of the cases in group 2 were prepubertal. The waist and hip circumferences ratio of the group 1 (0.86 ± 0.05) was significantly higher than group 2 (0.80 ± 0.07). While there was no difference between groups 1 and 2 in terms of the birth weight, using duration period of vitamin D and beginning time to additional nutrition, breastfeeding duration of group 1 (10.6 ± 7.8 months) was significantly shorter than group 2 (14 ± 7.4 months). BMIs of the mothers in group 1 were statistically higher than the mothers in group 2 (27.5 ± 4.8 kg/m² and 24.3 ± 3.2 kg/m² respectively. The mean of IR-HOMA was 4 ± 2.9 in group 1 and 1.9 ± 0.8 in group 2 and there was the insulin resistance in 51% of the obese cases. The dyslipidemia was diagnosed in 38.5% of the cases in group 1. The systolic and diastolic blood pressures in group 1 (117 ± 12.2 mmHg and 73.7 ± 9.4 mmHg respectively) were significantly higher than in group 2 (107.5 ± 9.1 mmHg and 68.2 ± 7.1 mmHg respectively). Hypertension was determined in 25% of the cases included in group 1. The minimum values of FMD in groups 1 and 2 were 1.01% and 3.1% respectively. The maximum values of FMD in groups 1 and 2 were 9.7% and 15% respectively. The mean values of FMD was %5 ± 2.3 in group 1 and %8.1 ± 3.5 in group 2. Compared with group 2, group 1 demonstrated significantly impaired FMD. There was no association between FMD and the birth weight, breastfeeding duration, physical exercises in two groups. A negative correlation was found between FMD and BMI (p < 0.01, r = -0.402). The correlation was determined between FMD and BMI of the mother (p = 0.017, r = -0.305) and the presence of obese individuals in the family (p = 0.021, r =-0.413). It was found that a significant negative correlation between FMD and waist-hip circumference ratio (p = 0.003, r = -0.421). When each groups were assessed in terms of biochemical and hormonal characteristics, there was low negative correlation between FMD and uric acid level and strong negative correlation between FMD and ALT level were determined in group 1. Conclusion: In our study showed that the obesity begins in the childhood period may cause to the endothelial dysfunction. For this reason, according to our opinion, recognition prior indicators of endothelial dysfunction in early time may be helpful both to take the precautions required and to prevent cardiovascular complications in childhood and influences to the adult period. The rising sizes of the waist and hip circumferences, positive family history for obesity and obesity of the parents were determined as the most important parameters negative affecting FMD. Unlike the literature, the association between endothelial dysfunction and GGT level the indicator of the hepatosteatosis in obese children was also found as well as FMD and ALT have also a close association independent from BMI in this study. Thus, a different point of view was formed since ALT may possibly have a predictor value in the assessment of the endothelial functions and it is also found as a highlighted risk factors for the endothelial dysfunction in this study. Because of this reason, it can be recommended that when the liver function tests carry out in obese children it does not show only hepatosteatosis but also can be used as an early indicator of the cardiovascular complications of obesity. Another important subject to be emphasize that the ALT level in the childhood period may be an early cardiovascular risk indicator in both obese and nonobese children.

1. Introduction This Special Issue (Part 2) expands upon the theme “Building Local Capacity for Long-term Disaster Resilience” presented in Special Issue Part 1 (JDR Volume 5, Number 2, April 2010) by examining the evolving concept of disaster resilience and providing additional reflections upon various aspects of its meaning. Part 1 provided a mixed set of examples of resiliency efforts, ranging from administrative challenges of integrating resilience into recovery to the analysis of hazard mitigation plans directed toward guiding local capability for developing resiliency. Resilience was broadly defined in the opening editorial of Special Issue Part 1 as “the capacity of a community to: 1) survive a major disaster, 2) retain essential structure and functions, and 3) adapt to post-disaster opportunities for transforming community structure and functions to meet new challenges.” In this editorial essay we first explore in Section 2 the history of resilience and then locate it within current academic and policy debates. Section 3 presents summaries of the papers in this issue. 2. Why is Resilience a Contemporary Theme? There is growing scholarly and policy interest in disaster resilience. In recent years, engineers [1], sociologists [2], geographers [3], economists [4], public policy analysts [5, 6], urban planners [7], hazards researchers [8], governments [9], and international organizations [10] have all contributed to the literature about this concept. Some authors view resilience as a mechanism for mitigating disaster impacts, with framework objectives such as resistance, absorption, and restoration [5]. Others, who focus on resiliency indicators, see it as an early warning system to assess community resiliency status [3, 8]. Recently, it has emerged as a component of social risk management that seeks to minimize social welfare loss from catastrophic disasters [6]. Manyena [11] traces scholarly exploration of resilience as an operational concept back at least five decades. Interest in resilience began in the 1940s with studies of children and trauma in the family and in the 1970s in the ecology literature as a useful framework to examine and measure the impact of assault or trauma on a defined eco-system component [12]. This led to modeling resilience measures for a variety of components within a defined ecosystem, leading to the realization that the systems approach to resiliency is attractive as a cross-disciplinary construct. The ecosystem analogy however, has limits when applied to disaster studies in that, historically, all catastrophic events have changed the place in which they occurred and a “return to normalcy” does not occur. This is true for modern urban societies as well as traditional agrarian societies. The adoption of “The Hyogo Framework for Action 2005-2015” (also known as The Hyogo Declaration) provides a global linkage and follows the United Nations 1990s International Decade for Natural Disaster Reduction effort. The 2005 Hyogo Declaration’s definition of resilience is: “The capacity of a system, community or society potentially exposed to hazards to adapt by resisting or changing in order to reach and maintain an acceptable level of functioning and structure.” The proposed measurement of resilience in the Hyogo Declaration is determined by “the degree to which the social system is capable of organizing itself to increase this capacity for learning from past disasters for better future protection and to improve risk reduction measures.” While very broad, this definition contains two key concepts: 1) adaptation, and 2) maintaining acceptable levels of functioning and structure. While adaptation requires certain capacities, maintaining acceptable levels of functioning and structure requires resources, forethought, and normative action. Some of these attributes are now reflected in the 2010 National Disaster Recovery Framework published by the U.S. Federal Emergency Management Agency (FEMA) [13]. With the emergence of this new thinking on resilience related to disasters, it is now a good time to reflect on the concept and assess what has recently been said in the literature. Bruneau et al. [1] offer an engineering sciences definition for community seismic resilience: “The ability of social units (e.g., organizations, communities) to mitigate hazards, contain the effects of disasters when they occur, and carry out recovery activities in ways that minimize social disruption and mitigate the effects of future earthquakes.” Rose [4] writes that resiliency is the ability of a system to recover from a severe shock. He distinguishes two types of resilience: (1) inherent – ability under normal circumstances and (2) adaptive – ability in crisis situations due to ingenuity or extra effort. By opening up resilience to categorization he provides a pathway to establish multi-disciplinary approaches, something that is presently lacking in practice. Rose is most concerned with business disruption which can take extensive periods of time to correct. In order to make resource decisions that lower overall societal costs (economic, social, governmental and physical), Rose calls for the establishment of measurements that function as resource decision allocation guides. This has been done in part through risk transfer tools such as private insurance. However, it has not been well-adopted by governments in deciding how to allocate mitigation resources. We need to ask why the interest in resilience has grown? Manyena [11] argues that the concept of resilience has gained currency without obtaining clarity of understanding, definition, substance, philosophical dimensions, or applicability to disaster management and sustainable development theory and practice. It is evident that the “emergency management model” does not itself provide sufficient guidance for policymakers since it is too command-and-control-oriented and does not adequately address mitigation and recovery. Also, large disasters are increasingly viewed as major disruptions of the economic and social conditions of a country, state/province, or city. Lowering post-disaster costs (human life, property loss, economic advancement and government disruption) is being taken more seriously by government and civil society. The lessening of costs is not something the traditional “preparedness” stage of emergency management has concerned itself with; this is an existing void in meeting the expanding interests of government and civil society. The concept of resilience helps further clarify the relationship between risk and vulnerability. If risk is defined as “the probability of an event or condition occurring [14]#8221; then it can be reduced through physical, social, governmental, or economic means, thereby reducing the likelihood of damage and loss. Nothing can be done to stop an earthquake, volcanic eruption, cyclone, hurricane, or other natural event, but the probability of damage and loss from natural and technological hazards can be addressed through structural and non-structural strategies. Vulnerability is the absence of capacity to resist or absorb a disaster impact. Changes in vulnerability can then be achieved by changes in these capacities. In this regard, Franco and Siembieda describe in this issue how coastal cities in Chile had low resilience and high vulnerability to the tsunami generated by the February 2010 earthquake, whereas modern buildings had high resilience and, therefore, were much less vulnerable to the powerful earthquake. We also see how the framework for policy development can change through differing perspectives. Eisner discusses in this issue how local non-governmental social service agencies are building their resilience capabilities to serve target populations after a disaster occurs, becoming self-renewing social organizations and demonstrating what Leonard and Howett [6] term “social resilience.” All of the contributions to this issue illustrate the lowering of disaster impacts and strengthening of capacity (at the household, community or governmental level) for what Alesch [15] terms “post-event viability” – a term reflecting how well a person, business, community, or government functions after a disaster in addition to what they might do prior to a disaster to lessen its impact. Viability might become the definition of recovery if it can be measured or agreed upon. 3. Contents of This Issue The insights provided by the papers in this issue contribute greater clarity to an understanding of resilience, together with its applicability to disaster management. In these papers we find tools and methods, process strategies, and planning approaches. There are five papers focused on local experiences, three on state (prefecture) experiences, and two on national experiences. The papers in this issue reinforce the concept of resilience as a process, not a product, because it is the sum of many actions. The resiliency outcome is the result of multiple inputs from the level of the individual and, at times, continuing up to the national or international organizational level. Through this exploration we see that the “resiliency” concept accepts that people will come into conflict with natural or anthropogenic hazards. The policy question then becomes how to lower the impact(s) of the conflict through “hard or soft” measures (see the Special Issue Part 1 editorial for a discussion of “hard” vs. “soft” resilience). Local level Go Urakawa and Haruo Hayashi illustrate how post-disaster operations for public utilities can be problematic because many practitioners have no direct experience in such operations, noting that the formats and methods normally used in recovery depend on personal skills and effort. They describe how these problems are addressed by creating manuals on measures for effectively implementing post-disaster operations. They develop a method to extract priority operations using business impact analysis (BIA) and project management based business flow diagrams (BFD). Their article effectively illustrates the practical aspects of strengthening the resiliency of public organizations. Richard Eisner presents the framework used to initiate the development and implementation of a process to create disaster resilience in faith-based and community-based organizations that provide services to vulnerable populations in San Francisco, California. A major project outcome is the Disaster Resilience Standard for Community- and Faith-Based Service Providers. This “standard” has general applicability for use by social service agencies in the public and non-profit sectors. Alejandro Linayo addresses the growing issue of technological risk in cities. He argues for the need to understand an inherent conflict between how we occupy urban space and the technological risks created by hazardous chemicals, radiation, oil and gas, and other hazardous materials storage and movement. The paper points out that information and procedural gaps exist in terms of citizen knowledge (the right to know) and local administrative knowledge (missing expertise). Advances and experience accumulated by the Venezuela Disaster Risk Management Research Center in identifying and integrating technological risk treatment for the city of Merida, Venezuela, are highlighted as a way to move forward. L. Teresa Guevara-Perez presents the case that certain urban zoning requirements in contemporary cities encourage and, in some cases, enforce the use of building configurations that have been long recognized by earthquake engineering as seismically vulnerable. Using Western Europe and the Modernist architectural movement, she develops the historical case for understanding discrepancies between urban zoning regulations and seismic codes that have led to vulnerable modern building configurations, and traces the international dissemination of architectural and urban planning concepts that have generated vulnerability in contemporary cities around the world. Jung Eun Kang, Walter Gillis Peacock, and Rahmawati Husein discuss an assessment protocol for Hazard Mitigation Plans applied to 12 coastal hazard zone plans in the state of Texas in the U.S. The components of these plans are systematically examined in order to highlight their respective strengths and weaknesses. The authors describe an assessment tool, the plan quality score (PQS), composed of seven primary components (vision statement, planning process, fact basis, goals and objectives, inter-organizational coordination, policies & actions, and implementation), as well as a component quality score (CQS). State (Prefecture) level Charles Real presents the Natural Hazard Zonation Policies for Land Use Planning and Development in California in the U.S. California has established state-level policies that utilize knowledge of where natural hazards are more likely to occur to enhance the effectiveness of land use planning as a tool for risk mitigation. Experience in California demonstrates that a combination of education, outreach, and mutually supporting policies that are linked to state-designated natural hazard zones can form an effective framework for enhancing the role of land use planning in reducing future losses from natural disasters. Norio Maki, Keiko Tamura, and Haruo Hayashi present a method for local government stakeholders involved in pre-disaster plan making to describe performance measures through the formulation of desired outcomes. Through a case study approach, Nara and Kyoto Prefectures’ separate experiences demonstrate how to conduct Strategic Earthquake Disaster Reduction Plans and Action Plans that have deep stakeholder buy-in and outcome measurability. Nara’s plan was prepared from 2,015 stakeholder ideas and Kyoto’s plan was prepared from 1,613 stakeholder ideas. Having a quantitative target for individual objectives ensures the measurability of plan progress. Both jurisdictions have undertaken evaluations of plan outcomes. Sandy Meyer, Eugene Henry, Roy E. Wright and Cynthia A. Palmer present the State of Florida in the U.S. and its experience with pre-disaster planning for post-disaster redevelopment. Drawing upon the lessons learned from the impacts of the 2004 and 2005 hurricane seasons, local governments and state leaders in Florida sought to find a way to encourage behavior that would create greater community resiliency in 2006. The paper presents initial efforts to develop a post-disaster redevelopment plan (PDRP), including the experience of a pilot county. National level Bo-Yao Lee provides a national perspective: New Zealand’s approach to emergency management, where all hazard risks are addressed through devolved accountability. This contemporary approach advocates collaboration and coordination, aiming to address all hazard risks through the “4Rs” – reduction, readiness, response, and recovery. Lee presents the impact of the Resource Management Act (1991), the Civil Defence Emergency Management Act (2002), and the Building Act (2004) that comprise the key legislation influencing and promoting integrated management for environment and hazard risk management. Guillermo Franco and William Siembieda provide a field assessment of the February 27, 2010, M8.8 earthquake and tsunami event in Chile. The papers present an initial damage and life-loss review and assessment of seismic building resiliency and the country’s rapid updating of building codes that have undergone continuous improvement over the past 60 years. The country’s land use planning system and its emergency management system are also described. The role of insurance coverage reveals problems in seismic coverage for homeowners. The unique role of the Catholic Church in providing temporary shelter and the central government’s five-point housing recovery plan are presented. A weakness in the government’s emergency management system’s early tsunami response system is noted. Acknowledgements The Editorial Committee extends its sincere appreciation to both the contributors and the JDR staff for their patience and determination in making Part 2 of this special issue possible. Thanks also to the reviewers for their insightful analytic comments and suggestions. Finally, the Committee wishes to again thank Bayete Henderson for his keen and thorough editorial assistance and copy editing support.

El autor es un estudioso de los derechos de la antigüedad anteriores a Roma. Entre otros trabajos se encuentran «Una pequeña aportación a la Historia universal del derecho a través del comentario al Código de Hattusas», «Notas para una Historia general del Arbitraje» (Sobre procedimientos extrajudiciales en el Egipto faraónico), una tesis doctoral sobre las «Instituciones familiares en el Egipto faraónico», «El régimen matrimonial en el derecho paleobabilónico» y «El matrimonio y sus crisis en el derecho de la antigüedad». En el presente trabajo el autor nos presenta un estudio sobre el matrimonio hitita, los modos matrimoniales, el rapto (restringido) y la compra (lo común), así como su evolución a través de los diversos institutos jurídicos existentes: La kusata, la iwaru o el assu, y la problemática crítica que se podía plantear en el matrimonio y sus soluciones, a la luz del conocimiento que nos aportan las tabletas A y B, más conocidas con el nombre de «código» hitita. Plantea también el autor la posibilidad de ceremonias religiosas y fiestas familiares, apoyándose en las figuras que aparecen en la fotografía, del fragmento de vaso, que aporta del museo de Ankara.The author is studious of the antique wrights before Rome. Among his different works are «A small study to ttie Universal History of wrights», through the comment of Hattusas, «Notes for a General History of reference » (about extrajudicial proceedings in the pharaonic Egipt) a doctoral thesis about the «family institutions in the pharaonic Egipt», «The marriage rules in the paleobabilonic wright», and «The marriage and its crissis in the oíd wright». At present, the author is working on the hitita marriage, the forms of marriage, the rapture (restricted) and the buying (the usual), so its evolution through different juridical institutions: The kusata, the iwaru or the assu and the problems could be expossed in the marriage and its solutions through the knowledges on tables A and B, better known by the ñame of hitita code. The author also introduces the possibility of religious ceremonys and family parties, based on the figures showed on photographies of the piece of ceramies from the Ankara Museum.

Joseph Wright of Derby (1734-1797) painted two portraits of Dr Erasmus Darwin, ca . 1770 and 1792-3, and painted a second version of the later one, in which the right arm and hand is in a different pose from the first version. Two copies of the first portrait and seven of the second portrait, of which James Rawlinson of Derby (1769-1848) made at least three, have been traced. Rawlinson also independently painted two portraits of Dr Darwin, of which he made a second version of the earlier one. Two busts of Erasmus Darwin, one in alabaster and one in plaster, that have been attributed to William Coffee of Derby ( fl . 1790-1846), are also discussed. The number of versions and copies of portraits of Erasmus Darwin reflect the wish of members of his family and his friends to possess a portrait of him, as well as his growing fame. Erasmus Darwin was married twice with many children, including two illegitimate daughters born between the marriages. To help elucidate the family relationships, a biographical note precedes the discussion of the portraits and there is a family tree in Appendix 2.

Background: It is important to assess how well patients respond to their medical treatments by observing the results that appear during the clinical treatments. As such, the clinical treatments and results must obtain information on how effective recommended treatments were for patients with diabetes. Objective: This study examines how patients with diabetes mellitus responded towards their clinical treatments, where the probability distribution of patients and the types of treatment received were derived from the Rasch probabilistic model. Methods: This is a retrospective study wherein data were collected from patients’ medical records at a local public hospital in Selangor, Malaysia. Clinical and demographic information such as fasting blood glucose, hemoglobin A1c (HbA1c), family history, type of diabetes (type 1 or type 2), types of medication (oral or insulin), compliance with treatments, gender, race and age were chosen as the agents of measurement. Methods: This is a retrospective study wherein data were collected from patients’ medical records at a local public hospital in Selangor, Malaysia. Clinical and demographic information such as fasting blood glucose, hemoglobin A1c (HbA1c), family history, type of diabetes (type 1 or type 2), types of medication (oral or insulin), compliance with treatments, gender, race and age were chosen as the agents of measurement. Results: The use of Rasch analysis in the present study helped to compare the patients’ responses towards the DM treatments and identify the types of treatment they received. Results from the Wright map show that a majority of the Diabetes Mellitus patients who were diagnosed with Type 2 diabetes have no controlled readings of HbA1c during their first and second visits to the medical center. However, patients with a family history of Diabetes Mellitus who took oral medication have controlled readings of fasting blood glucose based on the probabilistic outcomes of the treatment received by the patients. Conclusion:: Controlled readings were found only in the readings of fasting blood glucose during the first and second visits, followed by family history, types of medication received, and compliance with the treatment. This study has recommended that type 2 patients with diabetes without a family history of diabetes mellitus need to exercise more control over the readings of HbA1c.