3. Electronic Theses and Dissertations (ETDs) - All submissions
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Item A 10 year review of idiopathic nephrotic syndrome in children: a single-centre experience, Johannesburg, South Africa(2016-10-12) Bakhiet, Yassir MahgoubBackground: 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.Item A profile of patients with minimal change nephropathy between 2001 and 2010 at the Witwatersrand Academic Complex(2015-09-07) Mkandawire, Mercy JulietAvailable literature on primary minimal change nephropathy (MCN) predominantly reflects Western and Asian populations, with little data describing the disease in black patients. We therefore studied the demographic and clinical profile of patients presenting with MCN at the Witwatersrand Academic Complex. The results of 1,618 renal biopsies performed at our centre between 2001 and 2010 were reviewed; 47 patients with MCN were identified (prevalence of 2.9%).The patients were predominantly of black race (83%), the male : female ratio was 1.04:1 and the mean age was 31.8± 12.1 years. The majority of patients (90%) fitted the criteria for the nephrotic syndrome. 18% of patients had elevated serum creatinine levels and 6.4% had associated hypertension. An association was found between gender and age; with a predominance of males amongst younger patients (less than 30 years) compared to a predominance of females amongst the older patients. Records of treatment and outcomes were available for 28 patients, all of whom received initial corticosteroid therapy (average dose of prednisone 0.8mg /kg/day). The average duration of therapy was 29 weeks. 57.1% achieved remission with no further relapse. No clinical or demographic parameters were identified that predicted response to corticosteroid therapy. 39.2% of patients had probable steroid dependance/resistance. Of these patients, 58.3% had a single relapse and 41.7% had double relapses. The mean time to relapse was 27.8±19.4 months with 83% of patients relapsing within 48 months; the mean time to relapse was longer in males (39.3±17.5 months) as compared to females who relapsed in 18±16.9 months, which was significant at the 10% level (P = 0.09). MCN is rare amongst Black Africans but should be considered in the differential diagnosis of nephrotic syndrome. The disorder in these patients may be less responsive to corticosteroids and a longer course of therapy may be required to induce remission. Males may be more likely to remain in remission for a longer time period.