Hodgin Jeffrey B, Rasoulpour Majid, Markowitz Glen S, D'Agati Vivette D
Department of Pathology, Columbia University, College of Physicians & Surgeons, New York, NY 10032, USA.
Clin J Am Soc Nephrol. 2009 Jan;4(1):71-6. doi: 10.2215/CJN.01700408. Epub 2008 Nov 19.
Low birth weight (LBW), resulting from intrauterine growth retardation (IUGR) or prematurity, is a risk factor for adult hypertension and chronic kidney disease. LBW is associated with reduced nephron endowment and increased glomerular volume; however, the development of secondary focal segmental glomerulosclerosis (FSGS) has not been reported previously.
DESIGN, SETTING, PARTICIPANTS & MEASUREMENTS: The authors describe six patients with clinical and pathologic findings suggesting a secondary form of FSGS, in whom a history of prematurity and very LBW was obtained. No other known causes of secondary FSGS were identified.
The cohort consisted of two women and four men with a mean age of 32 yr. Patients were born at 22 to 30 wk gestation with mean birth weight of 1054 g (range 450 to 1420 g). Mean 24-h urine protein was 3.3 g/d (range 1.3 to 6.0 g/d), mean creatinine clearance 89 cc/min (range 71 to 132 cc/min), mean creatinine 1.2 mg/dl (range 0.9 to 1.5 mg/dl), and mean serum albumin 4.1 g/dl (range 3.4 to 4.8 g/dl). No patient had full nephrotic syndrome. Renal biopsy revealed FSGS involving a minority (mean 8.8%) of glomeruli, with a predominance of perihilar lesions of sclerosis (five of six patients), glomerulomegaly (all six patients), and only mild foot process effacement (mean 32%), all features typical of postadaptive FSGS.
Our findings support that very LBW and prematurity promote the development of secondary FSGS. Because birth history is often not obtained by adult nephrologists, this risk factor is likely to be underrecognized.
低出生体重(LBW)由宫内生长迟缓(IUGR)或早产导致,是成人高血压和慢性肾病的一个风险因素。低出生体重与肾单位数量减少和肾小球体积增大有关;然而,此前尚未有继发性局灶节段性肾小球硬化(FSGS)发生的报道。
设计、地点、参与者及测量指标:作者描述了6例临床和病理表现提示为继发性FSGS的患者,这些患者均有早产和极低出生体重史。未发现其他已知的继发性FSGS病因。
该队列包括2名女性和4名男性,平均年龄32岁。患者出生时孕周为22至30周,平均出生体重为1054克(范围450至1420克)。24小时尿蛋白平均为3.3克/天(范围1.3至6.0克/天),肌酐清除率平均为89毫升/分钟(范围71至132毫升/分钟),肌酐平均为1.2毫克/分升(范围0.9至1.5毫克/分升),血清白蛋白平均为4.1克/分升(范围3.4至4.8克/分升)。无患者患有完全性肾病综合征。肾活检显示FSGS累及少数肾小球(平均8.8%),以肾门周围硬化性病变为主(6例患者中有5例),肾小球肿大(所有6例患者),且仅伴有轻度足突融合(平均32%),所有这些特征均为适应性后FSGS的典型表现。
我们的研究结果支持极低出生体重和早产会促使继发性FSGS的发生。由于成年肾病学家常常未了解患者的出生史,这一风险因素可能未得到充分认识。