Krull F, Ehrich J H, Wurster U, Toel U, Rothgänger S, Luhmer I
Department of Paediatric Nephrology, Medizinische Hochschule, Hannover, Germany.
Acta Paediatr Scand. 1991 Dec;80(12):1214-9. doi: 10.1111/j.1651-2227.1991.tb11811.x.
Patients with congenital cyanotic heart disease may develop a glomerulopathy with proteinuria and impaired renal function. In order to investigate this problem we conducted a study on 27 patients with uncorrected cyanotic heart disease who were between 1 day and 25 years old. As a consequence of hypoxaemia haematocrit was elevated to 57%. Proteinuria was above 150 mg/day/1.73 m2 body surface in 12 patients. Only one of 9 children under 10 years of age had pathological proteinuria presenting as isolated albuminuria. Seven out of 10 patients between 11 and 20 years had an elevated proteinuria with a glomerular pattern. Creatinine clearance was normal in these patients. All four patients above 20 years of age had a considerable glomerular proteinuria with a mean excretion of 5.7 g/24 h/1.73 m2 body surface. These patients suffered additionally from chronic cardiac failure and creatinine clearance was below the normal range. There was a clear relationship between pathological proteinuria and age of the patients and thus duration of hypoxaemia. Patients with pathological proteinuria had a significant higher erythrocyte count (7.3 +/- 1.3 vs 5.6 +/- 1.4 10(12)/l p less than 0.01) and a lower mean corpuscular haemoglobin. In summary, children with persistent congenital cyanotic heart disease have substantial risk of developing a glomerulopathy if the cyanosis remains unchanged for more than ten years.
患有先天性青紫型心脏病的患者可能会出现伴有蛋白尿和肾功能受损的肾小球病。为了研究这个问题，我们对27例年龄在1天至25岁之间、未矫正的青紫型心脏病患者进行了一项研究。由于低氧血症，血细胞比容升高至57%。12例患者的蛋白尿超过150mg/天/1.73m²体表面积。10岁以下的9名儿童中只有1名患有病理性蛋白尿，表现为单纯性白蛋白尿。11至20岁的10例患者中有7例蛋白尿升高，呈肾小球型。这些患者的肌酐清除率正常。所有4例20岁以上的患者都有大量的肾小球蛋白尿，平均排泄量为5.7g/24小时/1.73m²体表面积。这些患者还患有慢性心力衰竭，肌酐清除率低于正常范围。病理性蛋白尿与患者年龄以及低氧血症持续时间之间存在明显的关系。患有病理性蛋白尿的患者红细胞计数显著更高（7.3±1.3对5.6±1.4×10¹²/L，p<0.01），平均红细胞血红蛋白更低。总之，如果青紫持续超过十年，患有持续性先天性青紫型心脏病的儿童有患肾小球病的重大风险。
