An amiss complement pathway can cause atypical hemolytic uremic syndrome (aHUS) with microangiopathic hemolytic anemia, thrombocytopenia and acute kidney injury. An observational study to understand complement abnormalities and outcome among pediatric aHUS in absence of targeted therapy was done. We enrolled 35 children from July 2017 to December 2018. Besides recording clinical details, hematological and renal parameters were assessed. Complement analysis included a one-time evaluation of C3, C4, anti-factor H antibody (VIDITEST human ELISA kit), factor H, I, B (Sinogeneclon ltd.) and CD46/membrane co-factor protein (MCP) (flow cytometry). SPSS version 23 (Chicago, IL) was used for analysis. Median age was 49 months (sex ratio of 1.7:1). Hypertension was noted in 74.2%(26) and central nervous system involvement in 34.3%(12). At admission, mean hemoglobin was 6.7±1.8 g/dL, median platelet count was 78x109 cells/L(38,101) and median eGFR was 11.91ml/min/1.73m2(6.5, 21.3). C3 was low in 57% (20), while 25.7% (9) had low C4 levels. Anti-factor H antibody was positive in 44% (15). Low expression of MCP in leucocytes was seen in 26.7% (8). Further, 3 had low factor H and high factor B respectively, while 2 had low factor I levels. Plasma-therapy was initiated in 65.7%. Remission was noted in 48.5% (17), 31.4% (11) died and others discontinued treatment. Though anti-factor H antibody is the most common followed closely by low MCP expression, abnormal levels of different complement proteins were observed. Outcome was dismal without eculizumab. We recommend comprehensive complement analysis in pediatric aHUS. Development and availability of targeted therapy remains unquestionable.

Antiglomerular basement membrane disease is a small vessel vasculitis which affects the lungs and kidneys, causing diffuse alveolar haemorrhage and rapidly progressive glomerulonephritis respectively. It is extremely uncommon in children. Diagnosis requires the presence of specific anti-GBM autoantibodies. Presentation with renal involvement is much more common than pulmonary involvement. However, a small proportion of cases might have isolated pulmonary involvement. Minimal changes on renal biopsy could still be present in these patients. Treatment of antiglomerular basement membrane disease includes plasmapheresis and immunosuppression. We present a case where a 7 year old boy presented with a long history suggestive of pulmonary haemorrhage but no renal manifestations and was diagnosed on the basis of anti-GBM autoantibodies and renal biopsy. He responded well to immunosuppressive therapy alone.

Myriad faces of X-linked agammaglobulinemia in a young boyAuthors: Murugan Sudhakar1, Manpreet Arora1, Lesa Dawman1, Dharmagat Bhattarai1, Pratap Kumar Patra1, Ankur Kumar Jindal1, Ritambhra Nada2, Amit Rawat1, Karalanglin Tiewsoh1