Awad Hesham, el-Safty Ibrahim, Abdel-Gawad Moustafa, el-Said Salwa
Departments of Pediatrics, Faculty of Medicine, Ain-Shams University, Cairo, Egypt.
Am J Med Sci. 2003 Mar;325(3):110-4. doi: 10.1097/00000441-200303000-00002.
Nephropathy has long been recognized as a potential complication of congenital cyanotic heart disease (CCHD). The present study was undertaken to investigate some aspects of glomerular function by measuring urinary total protein, microalbumin, and tubular function by assessing urinary alpha-1-microglobulin. The structural integrity of the renal proximal tubules was also studied by measuring urinary activities of the brush-border enzyme leucine-aminopeptidase and the lysosomal enzyme -acetyl-beta-d-glucosaminidase. The levels of hematocrit (Hct) and oxygen saturation were also investigated as predisposing factors for renal impairment in CCHD.
These investigations were done by recruiting 86 children who were grouped as follows: the control group (G1 ) consisted of 14 children (aged 4-12 years); the other 72 children with CCHD were divided according to age (ie, duration of cyanosis) into 4 equal groups, each containing 18 patients: G2 (age <1 year), G3 (age > or = 1 year and <5 years), G4 (age > or = 5 years and <10 years), and G5 (age > or = 10 years). In addition, 10 of the 72 patients underwent a palliative surgery and were included as G6 (regardless of age: 2 from G3, 4 from G4, and 4 from G5 ) to study the effect of the palliative surgery on the above-mentioned parameters.
Results of the present work showed that with increasing duration of cyanosis (ie, on going from G2 to G5 ) among the studied children with CCHD, there was a significant elevation in the urinary excretion of the investigated functional and structural parameters of the glomeruli and proximal tubules compared with the control children. The data also showed a significant increase in Hct, whereas oxygen saturation was significantly decreased. Results of G6 after the palliative surgery demonstrated a significant decrease in the urinary excretion of the investigated parameters of the kidney, with a significant decrease in Hct and increase in oxygen saturation levels, compared with the results of the patients of this group before the palliative surgery.
These results suggest impairment of both glomerular and tubular functions as well as structure of the proximal tubules among children with CCHD and that the palliative surgery has significantly improved this impairment.
肾病长期以来一直被认为是先天性青紫型心脏病（CCHD）的潜在并发症。本研究旨在通过测量尿总蛋白、微量白蛋白来研究肾小球功能的某些方面，并通过评估尿α-1-微球蛋白来研究肾小管功能。还通过测量刷状缘酶亮氨酸氨肽酶和溶酶体酶β-乙酰氨基葡萄糖苷酶的尿活性来研究肾近端小管的结构完整性。此外，还研究了血细胞比容（Hct）水平和氧饱和度作为CCHD患者肾功能损害的易感因素。
通过招募86名儿童进行这些调查，分组如下：对照组（G1）由14名儿童（4 - 12岁）组成；其他72名CCHD儿童根据年龄（即青紫持续时间）分为4个相等的组，每组18例患者：G2（年龄<1岁），G3（年龄≥1岁且<5岁），G4（年龄≥5岁且<10岁），G5（年龄≥10岁）。此外，72例患者中的10例接受了姑息性手术，并作为G6组（不分年龄：G3组2例，G4组4例，G5组4例）纳入研究，以研究姑息性手术对上述参数的影响。
本研究结果表明，在研究的CCHD儿童中，随着青紫持续时间的增加（即从G2组到G5组），与对照儿童相比，肾小球和近端小管的功能性和结构性参数的尿排泄量显著升高。数据还显示Hct显著增加，而氧饱和度显著降低。姑息性手术后G6组的结果表明，与该组患者姑息性手术前的结果相比，肾脏相关参数的尿排泄量显著降低，Hct显著降低，氧饱和度水平升高。
这些结果表明CCHD儿童的肾小球和肾小管功能以及近端小管结构均受损，且姑息性手术显著改善了这种损害。
