Dittrich S, Haas N A, Bührer C, Müller C, Dähnert I, Lange P E
Department of Congenital Heart Disease/Pediatric Cardiology, German Heart Center, Berlin.
Acta Paediatr. 1998 Sep;87(9):949-54. doi: 10.1080/080352598750031608.
Nephropathy is known to occur in patients with long-standing cyanotic congenital heart disease (CCHD). In order to assess the incidence, nature and degree of the problem among such patients, discriminating urine analyses were performed in 26 patients with CCHD, with a mean age of 22 (10-42) y. Ten patients showed reduced glomerular function, six of whom also had advanced glomerulopathy. Glomerular filtration rates were below normal in half of the patients and occurred with glomerular-type proteinuria in five, with tubular-type proteinuria in one and without pathological proteinuria in four. An elevated haematocrit and duration of cyanosis were identified as the main risks factors for the development of glomerulopathy. The risk of developing glomerular lesions rose sharply during the second decade of life. Nephropathy in CCHD is common and the dominant feature is glomerular damage, which is related to the duration of cyanosis and the extent to which the haematocrit is elevated.
已知肾病发生于患有长期青紫型先天性心脏病（CCHD）的患者中。为了评估此类患者中该问题的发生率、性质和程度，对26例平均年龄为22（10 - 42）岁的CCHD患者进行了鉴别性尿液分析。10例患者出现肾小球功能减退，其中6例还患有晚期肾小球病。一半患者的肾小球滤过率低于正常，5例伴有肾小球型蛋白尿，1例伴有肾小管型蛋白尿，4例无病理性蛋白尿。血细胞比容升高和发绀持续时间被确定为发生肾小球病的主要危险因素。在生命的第二个十年期间，发生肾小球病变的风险急剧上升。CCHD患者中的肾病很常见，主要特征是肾小球损伤，这与发绀持续时间和血细胞比容升高程度有关。
