Department of Paediatrics and Child Health, College of Health Sciences, Nelson R Mandela School of Medicine, University of KwaZulu- Natal, Durban, South Africa.
S Afr Med J. 2020 Jul 7;110(7):678-685. doi: 10.7196/SAMJ.2020.v110i7.14319.
The outcome and response of idiopathic nephrotic syndrome (NS) to steroids have been linked to race.
To determine the age of presentation, sex, race, histopathology, kidney function and disease status at the last hospital visit and correlate these with steroid response in Indian and black African children with idiopathic NS.
This is a retrospective review of 231 children aged 1 - 14 years, who were seen at Inkosi Albert Luthuli Central Hospital, Durban, South Africa (SA) from 2003 to 2018.
The mean (standard deviation (SD)) age of presentation was 6.2 (3.4) years, with the majority of children (n=107; 46.3%) presenting at an early age (1 - 3 years) with a mean (SD) follow-up of 3.0 (2.4) years. One-hundred and twenty-one (52.4%) were males and 110 (47.6%) were females, with a male/female ratio of 1.1:1. There were 166 (71.9%) black African and 65 (28.1%) Indian children. The latter presented at a younger age than black African children (p<0.001). Seventy-six (32.9%) children were steroid sensitive (SS) and 155 (67.1%) were steroid resistant (SR). Black African children were more likely to be SR (odds ratio (OR) 2.0; p=0.02; 95% confidence interval (CI) 1.1 - 3.7). A kidney biopsy was performed in 209 (90.5%) children. Minimal change disease (MCD) was observed in 32 (13.9%) children and 162 (70.1%) had focal segmental glomerulosclerosis (FSGS). Black African children were slightly more likely to have FSGS; this, however, did not reach statistical significance (122/166 (73.5%) v. 40/65 (61.5%); OR 1.73; p=0.08; 95% CI 0.94 - 3.18). On comparing disease status at last hospital visit by race, 49/65 (75.4%) Indian and 94/166 (56.6%) black African children were in remission. At last hospital visit, black African children were less likely to be in remission than Indian children (OR 0.47; p=0.02; 95% CI 0.2 - 0.9), while 15/65 (23.1%) Indian and 47/166 (28.3%) black African children had relapsed, with no significant difference between the two groups. One (1.5%) Indian child and 25 (15.1%) black African children had end-stage kidney disease (ESKD) (OR 9.27; p=0.03; 95% CI 1.2 - 70.4) ‒ the majority had FSGS. Sixteen (61.5%) received renal replacement therapy.
Our study shows a rising incidence of FSGS, with the majority of patients having SRNS, particularly black African children. This highlights the need for alternative efficacious therapy in the management of this disease. Also, a higher percentage of black African children with both MCD and FSGS were SS on histopathological examination, which was in keeping with reports from other regions in SA. There are still major challenges for the inclusion of all children into a chronic dialysis and transplant programme.
特发性肾病综合征（NS）的结局和对类固醇的反应与种族有关。
确定印度和非洲黑人儿童特发性 NS 的发病年龄、性别、种族、组织病理学、肾功能和最后一次就诊时的疾病状态，并将这些与类固醇反应相关联。
这是对 2003 年至 2018 年在南非德班英科西·阿尔贝·卢图利中央医院就诊的 231 名年龄在 1 至 14 岁的儿童进行的回顾性研究。
平均（标准偏差（SD））发病年龄为 6.2（3.4）岁，大多数儿童（n=107；46.3%）在早期（1-3 岁）就诊，平均（SD）随访时间为 3.0（2.4）年。121 名（52.4%）为男性，110 名（47.6%）为女性，男女比例为 1.1:1。有 166 名（71.9%）是非洲黑人，65 名（28.1%）是印度儿童。后者比非洲黑人儿童更早发病（p<0.001）。76 名（32.9%）儿童对类固醇敏感（SS），155 名（67.1%）对类固醇耐药（SR）。非洲黑人儿童更有可能是 SR（优势比（OR）2.0；p=0.02；95%置信区间（CI）1.1-3.7）。对 209 名（90.5%）儿童进行了肾脏活检。32 名（13.9%）儿童观察到微小病变性肾病（MCD），162 名（70.1%）患有局灶节段性肾小球硬化症（FSGS）。非洲黑人儿童更有可能患有 FSGS；然而，这并没有达到统计学意义（122/166（73.5%）比 40/65（61.5%）；OR 1.73；p=0.08；95%CI 0.94-3.18）。按种族比较最后一次就诊时的疾病状态，49/65（75.4%）印度儿童和 94/166（56.6%）非洲黑人儿童处于缓解状态。在最后一次就诊时，非洲黑人儿童缓解的可能性低于印度儿童（OR 0.47；p=0.02；95%CI 0.2-0.9），而 15/65（23.1%）印度儿童和 47/166（28.3%）非洲黑人儿童复发，两组之间无显著差异。一名（1.5%）印度儿童和 25 名（15.1%）非洲黑人儿童患有终末期肾病（ESKD）（OR 9.27；p=0.03；95%CI 1.2-70.4）——大多数儿童患有 FSGS。16 名（61.5%）接受了肾脏替代治疗。
我们的研究显示 FSGS 的发病率上升，大多数患者患有 SRNS，尤其是非洲黑人儿童。这突显了在该疾病的管理中需要替代有效治疗方法的必要性。此外，在组织病理学检查中，大多数患有 MCD 和 FSGS 的非洲黑人儿童为 SS，这与南非其他地区的报告一致。仍然存在将所有儿童纳入慢性透析和移植计划的主要挑战。
