Constantinescu A R, Weiss L S, Saidi P, Eisele J, Ettinger L J
Department of Pediatrics, UMDNJ-Robert Wood Johnson Medical School, New Brunswick 08901, USA.
J Pediatr Hematol Oncol. 1997 Jul-Aug;19(4):345-7. doi: 10.1097/00043426-199707000-00014.
The case of a 4-year-old boy with hemophilia B with inhibitor who developed nephrotic syndrome is described. The possible association between factor IX therapy and nephrotic syndrome in patients with hemophilia B is discussed.
A chart review of a 4-year-old boy with hemophilia B and an inhibitor who developed nephrotic syndrome with transient hypocomplementemia was performed. In addition, a literature search was undertaken to determine the prevalence of this association and possible etiologic factors.
Although the nephrotic syndrome was resistant to steroid therapy and Bebulin (Osterreichisches Institut für Haemoderivate Ges.M.B.H., Subsidiary of Immuno AG, Vienna, Austria) infusions were continued, the edema resolved and proteinuria decreased. Seven month later, proteinuria, accompanied by transient hypocomplementemia, increased again. A rise in factor IX inhibitor level was observed. The patient received no immunosuppressive therapy, and exhibited a continuous decrease in urinary protein excretion over the following months.
A discussion about possible differential diagnoses and a review of the literature are presented.
描述一名患B型血友病且产生抑制物的4岁男孩罹患肾病综合征的病例。探讨B型血友病患者中IX因子治疗与肾病综合征之间可能存在的关联。
对一名患B型血友病且产生抑制物并罹患伴有短暂低补体血症的肾病综合征的4岁男孩进行病历回顾。此外，进行文献检索以确定这种关联的发生率及可能的病因。
尽管肾病综合征对类固醇治疗耐药且继续输注贝布灵（奥地利血液制品研究所，免疫股份公司子公司，奥地利维也纳），水肿消退且蛋白尿减少。7个月后，伴有短暂低补体血症的蛋白尿再次增加。观察到IX因子抑制物水平升高。该患者未接受免疫抑制治疗，在随后几个月尿蛋白排泄持续减少。
给出了关于可能的鉴别诊断的讨论及文献综述。
