DIFFUSE JUVENILE SYSTEMIC SCLEROSIS PATIENTS SHOW DISTINCT ORGAN INVOLVEMENT, ANTIBODY PATTERN AND HAVE SIGNIFICANTLY MORE SEVERE DISEASE IN THE LARGEST JSSC COHORT OF THE WORLD. RESULTS FROM THE JUVENILE SCLERODERMA INCEPTION COHORT

Peer reviewe

Standardized Definitions for Bioprosthetic Valve Dysfunction Following Aortic or Mitral Valve Replacement: JACC State-of-the-Art Review.

peer reviewedBioprosthetic valve dysfunction (BVD) and bioprosthetic valve failure (BVF) may be caused by structural or nonstructural valve dysfunction. Both surgical and transcatheter bioprosthetic valves have limited durability because of structural valve deterioration. The main objective of this summary of experts participating in a virtual workshop was to propose standardized definitions for nonstructural and structural BVD and BVF following aortic or mitral biological valve replacement with the goal of facilitating research reporting and implementation of these terms in clinical practice. Definitions of structural BVF, based on valve reintervention or death, underestimate the true incidence of BVF. However, definitions solely based on the presence of high transprosthetic gradient at a given echocardiogram during follow-up overestimate the incidence of structural BVD and BVF. Definitions of aortic or mitral structural BVD must therefore include the confirmation by imaging of permanent structural changes to the leaflets alongside evidence of deterioration in valve hemodynamic function at echocardiography follow-up

Proceedings of the 24th Paediatric Rheumatology European Society Congress: Part three

From Springer Nature via Jisc Publications Router.Publication status: PublishedHistory: collection 2017-09, epub 2017-09-0

Identification of musculoskeletal problems using pGALS examination in school going boys in Chennai, India

Background and Aim: pGALS (pediatric Gait Arms Legs Spine) is a useful screening tool in identifying musculoskeletal problems in children. Although musculoskeletal problems are common in children, only a small fraction of them have an underlying serious pathology. There is limited data on this subject from north India but none from south India. Our aim was to identify the prevalence of musculoskeletal problems in school going boys in the local community. Methods: We conducted a prospective cross-sectional study among school boys 7–17 years of age from September 2017 to July 2019, in Chennai. The parents or the boys were asked pGALS screening questions and this was followed by the extended pGALS examination including Beighton's scoring for hypermobility. If abnormalities in the screening tool were found, the subjects were then referred to a pediatric rheumatologist for further evaluation. Results: A total of 1543 boys were included in this study. Of these 37% of the study group were found to be hypermobile. According to our study, the prevalence of growing pains was 4.6%. Of 2 boys with suspected pathology 1 boy had enthesitis-related arthritis and another had slipped upper femoral epiphysis (SUFE). The estimated prevalence of juvenile idiopathic arthritis (JIA) was 65/100000. According to our study 12.3% of the children were obese. Conclusion: pGALS is a useful tool to screen for musculoskeletal problems in school going boys. The estimated prevalence of JIA was 65/100000. Of them, 37% of the children had hypermobility. Obese children were more likely to complain of musculoskeletal pain (P value 0.00)

Familial Blau syndrome:First molecularly confirmed report from India

Blau syndrome (BS) is a rare autoinflammatory disorder characterized by the clinical triad of arthritis, uveitis, and dermatitis due to heterozygous gain-of-function mutations in the NOD2 gene. BS can mimic juvenile idiopathic arthritis (JIA)-associated uveitis, rheumatoid arthritis, and ocular tuberculosis. We report a family comprising a mother and her two children, all presenting with uveitis and arthritis. A NOD2 mutation was confirmed in all the three patients – the first such molecularly proven case report of familial BS from India

Therapeutic plasma exchange for pediatric nonrenal disease indications and outcomes: A single-center experience

Introduction: Outcome data in pediatric plasma exchange, especially in nonrenal indications are scarce. We aimed to evaluate its role and outcome in our patients. Subjects and Methods: A retrospective study of children admitted in the year 2016 to the Pediatric Intensive Care Unit requiring plasma exchange for nonrenal indications was undertaken. Plasma exchange was given as adjunctive therapy along with primary treatment for the disease concerned. Demographic and clinical data were studied, and descriptive statistical analysis was carried out. Results: Ten children underwent plasma exchange during this 1-year period with a male: female ratio of 3:2 and a mean age of 10 years (range 3–16 years). The indications were acute disseminated encephalomyelitis (n = 2), acute neuromyelitis optica (n = 1), catastrophic antiphospholipid antibody syndrome secondary to systemic lupus erythematosus (SLE) (n = 1), severe SLE with cerebritis/hemophagocytic lymphohistiocytosis (HLH) (n = 2), severe dengue sepsis with HLH/multi-organ dysfunction syndrome (n = 2), and thrombotic microangiopathy secondary to snake bite envenomation (n = 2). All received either 1.5 or 2 times plasma volume exchange (mean sessions – 4, range = 1–6). The mean duration of stay in hospital was 17.2 days (range = 3–40 days), and follow-up was 78 days (range = 3–180 days), with the majority of children (8/10, 80%) survived from the catastrophic illness at the time of discharge. Two children (2/10, 20%) succumbed due to the disease per se in severe dengue sepsis in one and enterobacteriaceae sepsis (hospital-acquired pneumonia) in another. Conclusion: Plasma exchange was found to be beneficial as complementary therapy in a critical care setting, especially for nonrenal indications