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文章报道了一家中2个同胞兄弟患先天性肾病。例2经尸体剖解,病理证实。2例均于生后第四天出现典型的临床症状。例1于生后55天死亡,例2因并发副大肠杆菌败血症于生后11天死亡。讨论 1.先天性肾病的分型,认为分原发型及继发型(继发于各种感染)。原发型根据病理、临床、遗传模式又分为:(1)婴儿微囊性肾病又称芬兰型,为常染色体隐性遗传,是先天性肾病中最多见的一型,除具典型临床症状外,肾组织光镜检查常可见近曲小管
The article reported that one of two siblings suffering from congenital kidney disease. Case 2 through the body section, pathology confirmed. Two cases were typical symptoms of the fourth day after birth. Case 1 died 55 days after birth, case 2 died of eleven days after birth due to concurrent E. coli septicemia. Discussion 1. The classification of congenital nephropathy, that primary and secondary sub-type (secondary to a variety of infections). Primary type according to pathology, clinical, genetic model is divided into: (1) infant microcapsule nephropathy, also known as Finnish type, autosomal recessive inheritance, congenital nephropathy is the most common type, with typical clinical symptoms In addition, microscopic examination of renal tissue often visible proximal convoluted tubules