A 10 year review of idiopathic nephrotic syndrome in children: a single-centre experience, Johannesburg, South Africa

Bakhiet, Yassir Mahgoub
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Background: Idiopathic nephrotic syndrome (INS) is the commonest type of nephropathy seen in children. The histopathological types and steroid response patterns of INS have been changing over the years and this has been attributed to differences in ethnicity and geographical location. The aim of this study was to determine the steroid response pattern, renal histopathology and complications in a cohort of the children treated for INS by the Division of Paediatric Nephrology, CMJAH, Johannesburg, South Africa between 2004 and 2013. Method: A retrospective study was carried out to review the records of 163 children between the ages of 2 and 16 years managed for INS over a 10 year period. Results: The majority (111) of the 163 children were of the black racial group. There were 97 boys and 66 girls. The mean age of onset was 5.3 years ± 2.8, with the highest rate of INS seen in the 2-6 year age group (71.2%). Only 132/163 had a renal biopsy performed (MCD 52.3%, FSGS 43.2%, MesPGN 4.5%). The black race had a similar rate of MCD (38.8%) and FSGS (37.8%), while the white race had a higher rate of MCD (64.3%) when compared to FSGS (14.3%). Ninety four (57.7%) patients were steroid sensitive (SSNS) while 69 patients (42.3%) were steroid resistant (SRNS). Minimal change disease was the most common histopathological type seen in SSNS (60%), while FSGS was the most common observed in patients who had SRNS (65.2%). There was a statistically significant association between the various steroid response patterns and the different histopathological types. The highest rate of resistance to all treatment after a mean follow up of 60 months was seen among children of the mixed race and black racial groups (50.0% and 40.5% respectively). Stunted growth (52.1%), hypertension (47.2%) and reduced eGFR (25.8%) were the most common complications observed. Conclusions: There appears to be an increase in the rate of FSGS in all the racial groups, and an increase in the rate of MCD in the black race group, when compared to previous South African studies. Furthermore, steroid response was also observed to have increased significantly among the black racial group when compared with previous studies. Although hypertension was the most common complication observed in our cohort, a very high rate of stunted growth was also observed. This may be due to the significant number of patients with reduced eGFR, SRNS and FSGS in our cohort. The use of long term steroid therapy may have contributed to this high rate.
Dissertation submitted to the Faculty of Health Sciences, University of Witwatersrand, Johannesburg, in fulfillment of the requirements for the degree of Master of Science in Medicine Johannesburg, 2016