PROGNOSIS OF THE NEPHROTIC SYNDROME IN SICKLE GLOMERULOPATHY - A RETROSPECTIVE STUDY
PROGNOSIS OF THE NEPHROTIC SYNDROME IN SICKLE GLOMERULOPATHY - A RETROSPECTIVE STUDY
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DOI：
10.1159/000167444
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发表时间：
1987-03-01
影响因子：
4.2
通讯作者：
DUNEA, G
中科院分区：
文献类型：
--
作者：
BAKIR, AA;HATHIWALA, SC;DUNEA, G
在240名患有镰状细胞性贫血超过11年的成年人中，12人患有肾病综合征。9例（75%）肾小球病变，镰状肾小球病，包括系膜扩张和基底膜复制。6例患者出现IV型肾小管性酸中毒。9例患者中有4例在24个月内死亡（17 . ±. 5;平均值±。SD），而5例存活36个月或更长时间（80 . ±. 49）;在年龄、入院时血肌酐、C3水平、尿蛋白排泄量、肾小管性酸中毒发生率等方面，前者与后者无显著性差异。慢性氮质血症3例，急性肾功能衰竭2例。在22例镰状肾小球病患者中（我们的9例增加到文献中的13例），11例在2年内死亡。其中10例（91%）发生肾功能衰竭，而11例中只有5例（45%）存活超过2年（p < 0.05）。镰状细胞贫血一般人群的5年死亡率为3.75%，75%的15岁或以上患者存活18年或以上。肾病综合征最常由镰状肾小球病引起，发生在4%的镰状细胞贫血患者中，导致三分之二的肾功能衰竭，半数患者在2年内死亡。慢性氮质血症的发展与早期死亡率密切相关。其预后比一般镰状细胞性贫血患者差得多。
Of 240 adults with sickle cell anemia seen over 11 years, 12 had the nephrotic syndrome. In 9 (75%) the glomerular lesion, sickle glomerulopathy, consisted of mesangial expansion and basement membrane duplication. Six patients had type IV renal tubular acidosis. Four of the 9 Patients died within 24 months (17 .+-. 5; mean .+-. SD), while 5 survived 36 months or longer (80 .+-. 49); no significant differences were seen between the former and the latter in age, admission serum creatinine and C3 levels, urinary protein excretion, or the frequency of renal tubular acidosis. Chronic azotemia developed in 3 and acute renal shutdown in another 2. Of 22 patients with sickle glomerulopathy (our 9 added to 13 from the literature) 11 died within 2 years. Ten of these (91%) had developed renal failure, compared to only 5 of the 11 (45%) who survived longer than 2 years (p < 0.05). The 5-year mortality in the general population of sickle cell anemia is 3.75%, and 75% of patients aged 15 years or older survive 18 years or longer. The nephrotic syndrome, most often caused by sickle glomerulopathy, occurs in 4% of patients with sickle cell anemia, leading to renal failure in two-thirds and death in 2 years in half the patients. The development of chronic azotemia correlates strongly with early mortality. The prognosis is much worse than that in the general population of sickle cell anemia.