Department of Internal Medicine, School of Medicine, Keio University, 35 Shinanomachi, Shinjuku-ku, Tokyo, 160-8582, Japan.
Department of Pathology, School of Medicine, Keio University, Tokyo, Japan.
CEN Case Rep. 2021 Aug;10(3):354-358. doi: 10.1007/s13730-021-00573-2. Epub 2021 Jan 21.
Cyanotic congenital heart disease is occasionally associated with kidney dysfunction, which is known as cyanotic nephropathy or cyanotic glomerulopathy. The clinical presentation of cyanotic nephropathy includes proteinuria, decreased estimated glomerular filtration rate, hyperuricemia, thrombocytopenia, or polycythemia. Although advances in surgical procedures have improved the prognosis of cyanotic congenital heart diseases, adult cases of cyanotic nephropathy are still rare, and there are few reports of kidney biopsy in adults with cyanotic nephropathy. Here, we present the case of a 41-year-old patient with Fontan palliation who developed nephrotic range proteinuria and had a kidney biopsy, which showed glomerular hypertrophy with segmental glomerulosclerosis around vascular poles, suggesting adaptive focal segmental glomerulosclerosis. This case provides further understanding of kidney dysfunction due to cyanotic congenital heart disease and shows the need for attention in the management for prevention of progression to end-stage renal disease and in the selection of renal replacement therapy.
紫绀型先天性心脏病偶尔与肾功能障碍相关，即紫绀性肾病或紫绀性肾小球病。紫绀性肾病的临床表现包括蛋白尿、估计肾小球滤过率降低、高尿酸血症、血小板减少或红细胞增多症。尽管手术方法的进步改善了紫绀型先天性心脏病的预后，但成人紫绀性肾病仍然罕见，且成人紫绀性肾病的肾脏活检报道很少。在这里，我们报告了 1 例 Fontan 姑息治疗的 41 岁患者，该患者发生了肾病范围的蛋白尿，并进行了肾脏活检，显示血管极周围有局灶节段肾小球硬化的肾小球肥大，提示适应性局灶节段肾小球硬化。该病例进一步了解了紫绀型先天性心脏病引起的肾功能障碍，并表明需要在管理中注意预防进展为终末期肾病，以及在选择肾脏替代治疗时注意。
