Anigilaje Emmanuel Ademola, Fashie Andrew Patrick, Ochi Clement
Nephrology Unit, Department of Paediatrics, University of Abuja Teaching Hospital, Abuja, Nigeria.
Sudan J Paediatr. 2019;19(2):126-139. doi: 10.24911/SJP.106-1547399573.
The response to steroid in childhood nephrotic syndrome (CNS) varies across geographical regions, depending on aetiology, genetics, and the underlying pathology. Recently, there is an increasing steroid responsiveness among Nigerian children with nephrotic syndrome (NS). This is the first report of CNS at the University of Abuja Teaching Hospital, Gwagwalada, Abuja, Nigeria, between 15th January 2016 and 30th June 2018. Prednisolone was administered to all the children with NS according to the regimen of the International Study of Kidney Disease in Children. There were 46 children aged 17 months to 18 years, including 37 males and 9 females. The peak age was 6-10 years with a mean age of 8.2 ± 4.4 years. Forty-one (89.1%) had idiopathic NS (INS). Secondary NS occurred in five (10.9%) children with hepatitis B infection, sickle cell anaemia, haemolytic-uraemic syndrome, and post-infectious glomerulonephritis (two cases). was not seen. Overall, steroid-sensitive NS (SSNS) was seen in 34 (73.9%) and in 32 (78%) with INS. Five (16.7%) of the 30 with SSNS relapsed on follow-up. Twelve (26.1%) were resistant to steroid (steroid-resistant NS, SRNS). Renal biopsies in five SRNS revealed focal segmental glomerulosclerosis in three, minimal change lesion in one, and severe interstitial fibrosis/glomerulosclerosis in another one. Four (8.7%) children who had SRNS died. A child with SRNS is surviving on renal transplant from a living-unrelated donor. The study supports the notion that steroid responsiveness is increasing among ethnic black Nigerian children. Pre-treatment renal biopsy may be unwarranted.
儿童肾病综合征（CNS）对类固醇的反应因地理区域而异，这取决于病因、遗传学和潜在病理。最近，尼日利亚肾病综合征（NS）患儿对类固醇的反应性有所增加。这是2016年1月15日至2018年6月30日期间尼日利亚阿布贾瓜瓜尔达市阿布贾大学教学医院关于CNS的首份报告。所有NS患儿均按照儿童肾脏病国际研究方案给予泼尼松龙治疗。共有46名年龄在17个月至18岁之间的儿童，其中37名男性，9名女性。高峰年龄为6 - 10岁，平均年龄为8.2±4.4岁。41名（89.1%）患有特发性NS（INS）。继发性NS发生在5名（10.9%）儿童中，病因包括乙型肝炎感染、镰状细胞贫血、溶血尿毒综合征和感染后肾小球肾炎（2例）。未观察到[此处原文缺失内容]。总体而言，34名（73.9%）患儿出现类固醇敏感型NS（SSNS），INS患儿中32名（78%）出现SSNS。30名SSNS患儿中有5名（16.7%）在随访中复发。12名（26.1%）对类固醇耐药（类固醇耐药型NS，SRNS）。5名SRNS患儿的肾活检显示，3名有局灶节段性肾小球硬化，1名有微小病变，另1名有严重间质纤维化/肾小球硬化。4名（8.7%）SRNS患儿死亡。1名SRNS患儿通过来自非亲属活体供体的肾移植存活。该研究支持了尼日利亚黑人儿童对类固醇的反应性正在增加这一观点。治疗前肾活检可能没有必要。
