An audit of biopsy proven minimal change nephrotic syndrome in children at Chris Hani Baragwanath academic hospital

Autor: Parbhoo, Kaajal
Jazyk: angličtina
Rok vydání: 2016
Druh dokumentu: Diplomová práce
Popis: A Research Report submitted to the Faculty of Health Sciences, University of the Witwatersrand, in partial fulfillment of the requirements for the degree Of Master of Medicine Johannesburg, 2016
Objective: To evaluate the clinicopathological features, response to treatment and outcomes in children presenting to Chris Hani Baragwanath Academic Hospital with biopsy proven minimal change nephrotic syndrome. Methods: A retrospective record review was conducted. Available records of children, between the ages of 1 and 14 years, who had nephrotic syndrome clinically and who were proven to be minimal change nephrotic syndrome on renal biopsy, were studied. Children who presented from January 1996 to December 2010 were included. Their demographics, clinical features on presentation, biopsy results, management and outcomes were studied. Results: In the 15 year period there were 129 (29% of all NS) children with minimal change nephrotic syndrome. Seventeen patients were excluded because 4 were not biopsied and 13 patients’ records could not be traced. The remaining 112 patients were included in the study. Ages ranged from 1 year to 13.6 years with a median age of 3.8 years (IQR 2.6-5.9). There was a male predominance, with 72 males and 40 females (1.8:1). The majority of the children studied were Black African (89.3%). On presentation 68.8% had microscopic haematuria. Although 59.8% had a blood pressure at presentation which was above the 95th centile for gender, height and age, only 33.9% had sustained hypertension. On initial biopsy, 34% were found to have the mesangial hypercellular variant of minimal change disease and 6% had the IgM variant of minimal change disease. Two patients went into spontaneous remission. The remainder, were treated with oral corticosteroids. Of those treated, 58.9% were steroid responsive, 19.6% were steroid resistant and 8.9% were initially responsive but subsequently became steroid resistant. Of the sample, 22.3% were steroid dependent and 16.1% were frequent relapsers. Second line immunosuppressive therapy was needed in 38 (33.9%) patients. The three second line immunosuppressant agents used were intravenous pulsed cyclophosphamide (28.5%), intravenous pulsed methyl prednisolone (9.8%) and mycophenolate mofetil (MMF) (7.1%). Repeat biopsies were performed on 22 children (19.6%). Four of the 22 repeat biopsies showed focal segmental glomerular sclerosis (FSGS).The average length of follow up was 4.86 years (median 3.58). At the last visit, 75.9% of the study group was in remission. During the course of follow up, 41.1% were admitted to hospital for a suspected bacterial infection. A high proportion of patients were lost to follow up (62%). The mortality rate was 1.8%. Conclusion: At Chris Hani Baragwanath Academic Hospital, all children with nephrotic syndrome are biopsied prior to initiating steroid therapy due to the high prevalence of tuberculosis infection and poor compliance in our population. This practice has highlighted differences between the children in our population with minimal change disease compared to that reported by the International Study of Kidney Disease in Children (ISKDC). In our study there were a higher proportion of children with initial hypertension and haematuria, and fewer children that responded to steroid therapy. This differs from the ISKDC findings in 1978. Their study had predominantly Caucasian children, and our study had predominantly Black African children. These differences in ethnicity may account for the differences.
MT2017
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