Praga M, Hernández E, Morales E, Campos A P, Valero M A, Martínez M A, León M
Department of Nephrology, Hospital Universitario 12 de Octubre, Carretera de Andalucia, Km 5, 400, E-28041 Madrid, Spain.
Nephrol Dial Transplant. 2001 Sep;16(9):1790-8. doi: 10.1093/ndt/16.9.1790.
Several cases of obesity-associated focal segmental glomerulosclerosis (OB-FSG) have been reported but little is known about the clinico-pathological features of this entity and its long-term outcomes.
We studied 15 obese patients (BMI 35+/-5.2 kg/m(2)) with biopsy-proven FSG. They were compared with a control group of 15 non-obese patients with idiopathic FSG (I-FSG).
Mean proteinuria at the time of renal biopsy was 3.1+/-2 g/24 h in OB-FSG; it reached the nephrotic range (> or =3.5 g/24 h) during follow-up in 12 patients (80%), but none of them had oedema, hypoproteinaemia, or hypoalbuminaemia. Proteinuria was more marked amongst I-FSG (6.5+/-4.2 g/24 h) and most of them developed oedema and biochemical nephrotic syndrome. Glomerulomegaly was observed in all renal biopsies from OB-FSG patients (mean glomerular diameter 256+/-24 microm in OB-FSG vs 199+/-26 microm in I-FSG, P<0.001). Twelve OB-FSG patients (80%) were treated with ACE inhibitors (ACEI) and proteinuria significantly decreased within the first 6 months of treatment but showed a later increase. None of the obese patients achieved a sustained weight loss. Seven (46%) patients with OB-FSG experienced a progressive renal insufficiency and five of them started intermittent dialysis. Kaplan-Meier estimated probabilities of renal survival after 5 and 10 years were 77 and 51%, respectively, in OB-FSG patients, and 52 and 30% in I-FSG (P<0.05). The risk of developing progressive renal failure among OB-FSG patients was statistically correlated with serum creatinine and creatinine clearance at presentation.
OB-FSG indicates a poor prognosis with almost one-half of patients developing advanced renal failure. Knowledge of the clinico-pathological features of this entity (obesity, FSG lesions with glomerulomegaly, absence of nephrotic syndrome despite nephrotic-range proteinuria) should be helpful in establishing an accurate and early diagnosis.
已有几例肥胖相关的局灶节段性肾小球硬化(OB - FSG)的病例报道,但对于该疾病实体的临床病理特征及其长期预后知之甚少。
我们研究了15例经活检证实为FSG的肥胖患者(BMI 35±5.2 kg/m²)。将他们与15例非肥胖的特发性FSG(I - FSG)患者组成的对照组进行比较。
OB - FSG患者肾活检时的平均蛋白尿为3.1±2 g/24小时;随访期间12例患者(80%)蛋白尿达到肾病范围(≥3.5 g/24小时),但他们均无水肿、低蛋白血症或低白蛋白血症。I - FSG患者的蛋白尿更为明显(6.5±4.2 g/24小时),且大多数患者出现水肿和生化性肾病综合征。在所有OB - FSG患者的肾活检中均观察到肾小球肿大(OB - FSG患者的平均肾小球直径为256±24微米,而I - FSG患者为199±26微米,P<0.001)。12例OB - FSG患者(80%)接受了血管紧张素转换酶抑制剂(ACEI)治疗,治疗的前6个月蛋白尿显著下降,但随后又有所上升。肥胖患者均未实现持续体重减轻。7例(46%)OB - FSG患者出现进行性肾功能不全,其中5例开始间歇性透析。Kaplan - Meier估计OB - FSG患者5年和10年后的肾脏存活概率分别为77%和51%,I - FSG患者分别为52%和30%(P<0.05)。OB - FSG患者发生进行性肾衰竭的风险与就诊时的血清肌酐和肌酐清除率具有统计学相关性。
OB - FSG提示预后不良,近一半患者会发展为晚期肾衰竭。了解该疾病实体的临床病理特征(肥胖、伴有肾小球肿大的FSG病变、尽管蛋白尿达到肾病范围但无肾病综合征)有助于准确早期诊断。