IgA腎症を合併した先天性第Ⅶ因子欠乏症の1症例

Abstract

A 32-year-old man associated with factor Ⅶ deficiency and IgA nephropathy is reported. According to his family history, the patient's parents were cousins in marriage and his younger brother was also affected by factor Ⅶ deficiency. Twenty-five years ago, moderate proteinuria (1-3 g/day) was first discoverd. A renal biopsy specimen showed mild to moderate mesangial proliferative glomerulonephritis with IgA deposits. Hemostatic tests demonstrated typical factor Ⅶ deficiency : marked prolongation of coagulation time, partial thromboplastin time and absence of factor Ⅶ. In his past clinical course he had had a transient cerebrovascular attack presenting with left hemiparesis 15 years ago. At last observation, his renal function remained normal although moderate proteinuria persisted. To the author's knowledge, this is the first reported case associated with these two diseases.