马拉维伊丽莎白女王中央医院儿童蛋白尿性肾病
Proteinuric kidney disease in children at Queen Elizabeth Central Hospital, Malawi.
作者信息
Mwanza Zondiwe Victor, McCulloch Mignon, Drayson Mark, Plant Timothy, Milford David V, Dreyer Gavin
机构信息
Department of Paediatrics and Child health, Queen Elizabeth Central Hospital, Blantyre, Malawi.
Department of Paediatrics and Child health, University of Cape Town, Cape Town, South Africa.
出版信息
BMC Nephrol. 2018 Jan 31;19(1):21. doi: 10.1186/s12882-018-0832-6.
BACKGROUND
There is a paucity of data on paediatric kidney disease in developing countries such as Malawi. Descriptive research on kidney disease is essential to improving patient outcomes.
METHODS
We conducted a cross-sectional study at a tertiary hospital in Malawi from 2012 to 2013. Children under 14 years with proteinuric kidney disease were enrolled from paediatric wards and outpatient clinics at Queen Elizabeth Central Hospital (QECH). Demographic, clinical and laboratory data were collected from patients at enrolment and at 3 months review at which point clinical status and disease outcome were ascertained.
RESULTS
Thirty-four (22 male) patients were studied, mean age 8.54 (SD = 3.62 years). Glomerular disease (n = 25, 68%) was the most common presumed renal lesion at presentation. Nephritic syndrome (10) was characterised by a lower baseline complement C3 than nephrotic syndrome (p = 0.0027). Seven (47%) cases of nephrotic syndrome achieved complete remission. Eight (80%) cases of nephritic syndrome improved with supportive therapy. Nineteen (56%) patients presented with clinically significant renal damage with eGFR< 60 ml/min/1.73m. Six patients presented in chronic kidney disease (CKD) stage 5 of unclear aetiology, five (83%) died. Three (9%) patients had impaired kidney function and obstructive uropathy demonstrated on ultrasound, two recovered after surgery and one died. Eight (24%) patients had acute kidney injury (AKI) due to primary kidney disease, three of these patients progressed to CKD stage G3a. Seven (21%) patients were lost to follow up.
CONCLUSION
Kidney disease is a significant cause of mortality and morbidity in children at QECH. Less than half of Nephrotic syndrome cases achieved complete remission. Mortality is highest in children with CKD of unclear cause. Some patients with AKI secondary to primary renal disease progressed to CKD. Understanding the aetiology of paediatric kidney disease and improving patient outcomes by developing enhanced diagnostic and clinical services are priorities at QECH and within Malawi.
背景
在马拉维等发展中国家，关于儿科肾脏疾病的数据匮乏。对肾脏疾病进行描述性研究对于改善患者预后至关重要。
方法
我们于2012年至2013年在马拉维的一家三级医院开展了一项横断面研究。从伊丽莎白女王中央医院（QECH）的儿科病房和门诊招募14岁以下患有蛋白尿性肾脏疾病的儿童。在入组时以及3个月复查时收集患者的人口统计学、临床和实验室数据，并确定此时的临床状况和疾病转归。
结果
共研究了34例患者（22例男性），平均年龄8.54岁（标准差=3.62岁）。肾小球疾病（n=25，68%）是最常见的初诊时推测的肾脏病变。肾炎综合征（10例）的基线补体C3低于肾病综合征（p=0.0027）。7例（47%）肾病综合征病例实现完全缓解。8例（80%）肾炎综合征病例经支持治疗后病情改善。19例（56%）患者出现具有临床意义的肾损害，估算肾小球滤过率（eGFR）<60ml/min/1.73m²。6例病因不明的慢性肾脏病（CKD）5期患者中，5例（83%）死亡。3例（9%）患者肾功能受损且超声显示存在梗阻性尿路病，2例术后恢复，1例死亡。8例（24%）患者因原发性肾脏疾病发生急性肾损伤（AKI），其中3例进展为CKD 3a期。7例（21%）患者失访。
结论
在QECH，肾脏疾病是儿童死亡和发病的重要原因。不到一半的肾病综合征病例实现完全缓解。病因不明的CKD患儿死亡率最高。一些原发性肾脏疾病继发AKI的患者进展为CKD。了解儿科肾脏疾病的病因并通过发展强化诊断和临床服务改善患者预后是QECH以及马拉维的工作重点。
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