2-year-old boy was diagnosed as suffering from Hemophilia A(Factor VIII<1%) at 3 months of age following an episode of left kneehemarthrosis. He was started on continuous prophylaxis at 12 monthsfollowing multiple episodes of hemarthroses and bleeding from oral cavityfollowing accidental injuries. At 15 months of age he underwent lymph-node excision biopsy following persistent fever and lymphadenopathyunder GA with factor support. At 18 months of age, he was diagnosed to beinhibitor positive (128 BU) following an episode of unresponsive kneehemarthrosis. He received high dose factor VIII for one episode of oralbleeding. Bypassing agentswereemployed for subsequentbleeds. Immunetolerance was not attempted due to logistic constraints. At the age of 22months of age, he presented with puffiness of eyelids and legs and reducedurine output. Hypoalbuminemia, hypercholesterolemia and nephroticrangeproteinuriasupportedthediagnosisofnephroticsyndrome.HIV,HCVand HBV were negative. He was started on 2mg/kg/day of prednisolone.Difficult venous access, persistent bruising, need for bypassing agents forinfected paronychia were challenges faced while management. He startedimproving after 7 days and subsequentlyachieved remission by day 14 andcontinues to remain so following tapering of steroids.
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机译：一名2岁男孩在左膝关节血栓形成发作后3个月被诊断患有A型血友病（因子VIII <1％）。在多次发作性玫瑰花病和因意外伤害导致口腔出血之后的12个月，他开始进行连续预防。在15个月大时，他在GA的支持下持续发烧和淋巴结肿大后接受了淋巴结切除活检。在18个月大时，他被诊断为无反应性膝关节关节炎后的抑制剂阳性（128 BU）。他接受了高剂量的VIII因子治疗，导致一次流血。旁路代理被用于后续出血。由于后勤方面的限制，未尝试免疫耐受。在22个月大时，他出现眼睑和腿浮肿和尿量减少。低白蛋白血症，高胆固醇血症和肾病范围蛋白尿支持肾病综合征的诊断。HIV，HCV和HBV阴性。他开始以泼尼松龙2mg / kg /天的剂量开始治疗。在治疗过程中，困难的静脉通路，持续的瘀伤，需要用于感染甲沟炎的绕过剂的需求。他在7天后开始改善，随后在第14天获得缓解，并且在类固醇逐渐减少后一直保持这种状态。
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