Dittrich S, Kurschat K, Lange P E
Department of Congenital Heart Disease, German Heart Center Berlin.
Scand J Urol Nephrol. 2001 Oct;35(5):411-5. doi: 10.1080/003655901753224486.
There are few data on non-immune nephropathy in cyanotic congenital heart disease reported in the literature. Rheologic changes have been suspected to be involved in the pathogenesis. This study was performed to describe the characteristics of renal functional and hemorheological abnormalities in a large group of patients with cyanotic congenital heart disease.
Thirty-five cyanotic patients with median oxygen saturation of 82% (range 38-92%), age 18 years (range 5-63 years), and 13 acyanotic controls with atrial septal defect, age 37 years (range 20-66 years) were included. Red cell indices, blood and plasma viscosity were analyzed. Renal function was evaluated with measurements of albumin, alpha1-microglobulin, transferrin, immunoglobuline, and N-acetyl-beta-D-glucosaminidase in the urine.
Fifteen cyanotic patients and 1 control patient had pathologic albuminuria (p < 0.05). In cyanotic patients blood and plasma viscosity, erythrocyte count, hemoglobin, and hematocrit were elevated while mean corpuscular volume and mean corpuscular hemoglobin were decreased (p < 0.05). The possible impact of blood hyperviscosity on proteinuria is indicated by multiple regression analysis (odds ratio = 0.5, confidence intervals 22-130, p < 0.05).
Our study shows a coincidence of elevated blood viscosity and proteinuria in patients with cyanotic congenital heart disease. This observation supports the hypothesis that impaired peritubular capillary blood flow with increased intraglomerular blood pressure may add to chronic glomerular dysfunction.
文献中报道的关于青紫型先天性心脏病非免疫性肾病的数据较少。流变学变化被怀疑参与了发病机制。本研究旨在描述一大组青紫型先天性心脏病患者的肾功能和血液流变学异常特征。
纳入35例青紫型患者,中位氧饱和度为82%(范围38%-92%),年龄18岁(范围5-63岁),以及13例房间隔缺损的非青紫型对照患者,年龄37岁(范围20-66岁)。分析红细胞指数、血液和血浆粘度。通过检测尿中白蛋白、α1-微球蛋白、转铁蛋白、免疫球蛋白和N-乙酰-β-D-氨基葡萄糖苷酶来评估肾功能。
15例青紫型患者和1例对照患者有病理性蛋白尿(p<0.05)。青紫型患者的血液和血浆粘度、红细胞计数、血红蛋白和血细胞比容升高,而平均红细胞体积和平均红细胞血红蛋白降低(p<0.05)。多元回归分析表明血液高粘度对蛋白尿可能有影响(比值比=0.5,置信区间22-130,p<0.05)。
我们的研究表明青紫型先天性心脏病患者存在血液粘度升高和蛋白尿并存的情况。这一观察结果支持了以下假设,即肾小管周围毛细血管血流受损伴肾小球内血压升高可能会加重慢性肾小球功能障碍。