Gutiérrez Millet V, Navas Palacios J J, Ortega Ruano R, Barrientos Guzmán A, Usera Sarraga G, Prieto Carles C, Montalbán M A, Rodicio J L
Med Clin (Barc). 1981 Jan 10;76(1):1-7.
Idiopathic mesangial glomerulonephritis with IgA deposits was observed in two relatives, father and son, in a family of 5 members. In the father the disease started at age 43 with relapsing macroscopic hematuria, proteinuria, renal failure and hypertension, with a progressive course in the ensuing four years. The affected son, the oldest of three brothers, developed relapsing macroscopic hematuria at age 16; two years later renal function was normal and there was no hypertension, but microhematuria persisted without proteinuria. The mother and the other two brothers had no clinical or biological signs of renal disease. Serum immunoglobulins (IgG, IgA, and IgM) and complement (C3, C4, C3 proactivator) were normal in the patients and their relatives. Histocompatibility typing demonstrated the presence of HLA-Bw35 in the father and the two unaffected sons, being negative in the mother and the affected son. The analysis of HLA-Bw35 in 23 patients with IgA mesangial glomerulonephritis gave positive results in 30% of them, while the control group had a positivity of 15% (p non significant with the X2 test). The present observations suggest that IgA mesangial glomerulonephritis is a potentially familial and hereditary renal disease. HLA-Bw35 antigen appears not to be a genetic marker of the disease in our geographical area.
在一个五口之家的父子两名亲属中观察到伴有IgA沉积的特发性系膜增生性肾小球肾炎。父亲43岁起病,表现为复发性肉眼血尿、蛋白尿、肾衰竭和高血压,在随后四年中病情呈进行性发展。患病的儿子是三兄弟中的老大,16岁时出现复发性肉眼血尿;两年后肾功能正常,无高血压,但镜下血尿持续存在,无蛋白尿。母亲和另外两个兄弟无肾脏疾病的临床或生物学体征。患者及其亲属的血清免疫球蛋白(IgG、IgA和IgM)和补体(C3、C4、C3前活化剂)均正常。组织相容性分型显示父亲和两个未患病的儿子存在HLA - Bw35,母亲和患病儿子为阴性。对23例IgA系膜增生性肾小球肾炎患者的HLA - Bw35分析显示,其中30%呈阳性,而对照组的阳性率为15%(经卡方检验,P无显著性差异)。目前的观察结果提示,IgA系膜增生性肾小球肾炎是一种潜在的家族性和遗传性肾脏疾病。在我们所在地区,HLA - Bw35抗原似乎不是该疾病的遗传标记。