García-de la Puente Silvestre, Orozco-Loza Iraida Luz, Zaltzman-Girshevich Samuel, de Leon Bojorge Beatriz
Department of Nephrology, National Institute of Pediatrics, Ministry of Health, Mexico City, Mexico.
Pediatr Nephrol. 2008 Jun;23(6):929-35. doi: 10.1007/s00467-008-0754-9.
The clinical outcome of patients with membranoproliferative glomerulonephritis (MPGN) varies, with some patients progressing to end-stage renal disease. The aim of this retrospective study was to analyze the initial clinical signs and laboratory test results associated with an MPGN prognosis. The study cohort consisted of 47 patients with idiopathic MPGN Type I treated at the National Institute of Pediatrics, Mexico City, between 1971 and 2001. The median follow-up was 3 years. The three different outcomes of interest were death, renal failure, and nephrotic syndrome. The patients' ages ranged between 4 and 16 years. All patients had different degrees of proteinuria, hyperlipidemia, and microscopic/macroscopic hematuria, and 85.1% of them showed hypocomplementemia. Clinical outcomes varied, however, the most common was nephrotic syndrome, either alone or combined with other syndromes, which accounted for 74.5% of all cases. Fifteen patients died. Treatment with methylprednisolone improved the patient's condition, while the use of chloroquine or cyclophosphamide worsened it. Twenty-two patients had some degree of renal failure; glomerular filtration rate (GFR) levels and albumin values were negatively associated to renal failure, while treatment with methylprednisolone decreased the probability of renal failure. Nephrotic syndrome persisted in 18 patients; hemolytic complement and hemoglobin values were negatively associated with nephrotic syndrome, while macroscopic hematuria was positively associated with it. Signs that suggested a poor prognosis during diagnosis were low GFR, low albumin, low hemolytic complement, and macroscopic hematuria. Treatment with methylprednisolone seemed to improve prognosis, however, this needs to be confirmed with randomized studies.
膜增生性肾小球肾炎（MPGN）患者的临床结局各不相同，部分患者会进展至终末期肾病。这项回顾性研究的目的是分析与MPGN预后相关的初始临床体征和实验室检查结果。研究队列包括1971年至2001年在墨西哥城国家儿科研究所接受治疗的47例特发性I型MPGN患者。中位随访时间为3年。关注的三种不同结局是死亡、肾衰竭和肾病综合征。患者年龄在4至16岁之间。所有患者均有不同程度的蛋白尿、高脂血症以及镜下/肉眼血尿，其中85.1%的患者出现低补体血症。然而，临床结局各不相同，最常见的是肾病综合征，单独出现或与其他综合征合并出现，占所有病例的74.5%。15例患者死亡。甲基泼尼松龙治疗改善了患者状况，而使用氯喹或环磷酰胺则使其病情恶化。22例患者出现一定程度的肾衰竭；肾小球滤过率（GFR）水平和白蛋白值与肾衰竭呈负相关，而甲基泼尼松龙治疗降低了肾衰竭的可能性。18例患者肾病综合征持续存在；溶血补体和血红蛋白值与肾病综合征呈负相关，而肉眼血尿与之呈正相关。诊断期间提示预后不良的体征包括低GFR、低白蛋白、低溶血补体和肉眼血尿。甲基泼尼松龙治疗似乎改善了预后，然而，这需要通过随机研究来证实。
