Nephrotic syndrome in South African children: changing perspectives over 20 years

Abstract

We review our 20-year experience of 636 children with nephrotic syndrome (NS) in Durban, South Africa; 306 (48.2%) were blacks, 307 (48.2%) Indians and 23 (3.6%) were a mixed group (coloured); 91 (14.3%) could not be categorised and were excluded from the analysis. In Indian children, 134 of 286 (46.8%) had biopsy-proven minimal change NS (MCNS) and 94.8% of these were steroid sensitive (SS); 60 (21%) had SSNS but without renal biopsy; 59 (20.6%) had focal segmental glomerulosclerosis (FSGS), with only 4.4% of these being SS. In blacks, membranous nephropathy accounted for 40% of cases; 86.2% were associated with hepatitis B virus antigens. Typical SSNS continues to be uncommon among blacks. Only 14.4% had either biopsy-proven SS-MCNS or SSNS; 32 had MCNS lesions on biopsy, but 18 were steroid resistant (SR); 67 of 236 (28.4%) had FSGS, all of whom were SR. Among coloured patients, 5 of 23 (21.7%) had biopsy-proven SS-MCNS and or unbiopsied SSNS; 10 (43.5%) had FSGS and 6 of 23 (26.1%) had membranous nephropathy. Proliferative lesions were present in only 2 of 23 (8.6%) coloured patients and was uncommon in all population groups. Overall mortality was 3.1%. In brief, this is the largest reported series of NS among children in Africa and shows a typical pattern in Indians, an unusual pattern of histological types in blacks and an intermediate picture in coloureds.

Childhood kidney disease in developing countries: Is it a forgotten disease?

“…[6] Schistosoma haematobium and Schistosoma mansoni, together with Salmonella typhi, have been reported as the most highly prevalent forms of GN and nephrotic syndrome in Egypt, while a post-infectious type of proliferative nephropathy is seen in Tunisia (northern Africa). [6,7] In the malarial-infested regions of Kenya, Nigeria, Uganda, and parts of Ghana, Plasmodium malariae is the most frequent cause of GN. In Zimbabwe (southern Africa) a post-infectious, diffuse proliferative, exudative form of GN is most often seen.…”

“…In Zimbabwe (southern Africa) a post-infectious, diffuse proliferative, exudative form of GN is most often seen. [7] Hepatitis B-related nephropathy contributed to a fair percentage as a cause of nephrotic syndrome in black children prior to 1994 in SA. In KwaZulu-Natal (KZN) Province, the rate was 34.3%, [7] while it was 12% in Johannesburg (Gauteng Province), [8] but following the introduction of hepatitis B immunisations in 1994, the disease has almost been eradicated with only two sporadic cases seen in the last decade in KZN.…”

“…[7] Hepatitis B-related nephropathy contributed to a fair percentage as a cause of nephrotic syndrome in black children prior to 1994 in SA. In KwaZulu-Natal (KZN) Province, the rate was 34.3%, [7] while it was 12% in Johannesburg (Gauteng Province), [8] but following the introduction of hepatitis B immunisations in 1994, the disease has almost been eradicated with only two sporadic cases seen in the last decade in KZN. [9] With better screening and treatment for syphilis, congenital syphilis-associated membranous nephropathy has also almost disappeared.…”

Steroid Sensitive Nephrotic Syndrome in Children

“…The majority of the remaining patients have focal segmental glomerulosclerosis and mesangioproliferative glomerulonephritis. Mesangiocapillary or membranoproliferative glomerulonephritis and idiopathic membranous nephropathy are uncommon in childhood and almost all are steroid resistant [5,9,10].…”

“…Whilst in developed countries steroid sensitive minimal change disease predominates, the NS amongst black children in Africa does not conform to the model established in other continents [5]. Black children have a paucity of minimal change disease and an increasing frequency of focal segmental glomerulosclerosis (FSGS), a high incidence of steroid resistant disease, a less satisfactory outcome and an identifiable causative agent in many [5]. risk of developing end-stage kidney disease with a need for renal replacement therapy.…”