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Background: Indications for peritoneal dialysis in children with post-dysenteric hemolytic uremic syndrome (D+ HUS) have not been thoroughly evaluated. Although early institution of dialysis may reduce mortality, the procedure has attendant complications., Aim: To determine whether the use of more stringent criteria for instituting dialysis had a better outcome to that of using conventional criteria., Method: Following an outbreak of Shigella dysenteriae type 1 D+ HUS in KwaZulu/Natal during June 1994 to October 1995, we compared the renal outcome and mortality between two periods: before May 1995 (69 children) when conventional criteria for dialysis were employed and after May 1995 (70 children) when more stringent criteria for dialysis were applied., Results: The mean age of presentation was 35 months, 79 (56.8%) were males. Both groups were comparable except for gut perforation, which was more frequent before May 1995, and hypertension and severe disease, which were more frequent after May 1995. Seventy patients underwent dialysis, 36 (52.2%) before May 1995. There were no significant differences in renal outcome or death following discharge from hospital in both groups. Overall mortality was 20.1%, 15 (53.6%) of the 28 children that demised presented before May 1995., Outcome: Accordingly, although children with more severe disease and a higher frequency of hypertension presented after May 1995, there were no significant differences in morbidity or mortality in those using stringent criteria for dialysis, compared to those in whom conventional criteria were used., Conclusion: We showed that several children, who would previously have been dialyzed, may be managed conservatively, without an increase in mortality or morbidity.

The biosocial background in which the hepatitis B virus (HBV) carrier state with membranous nephropathy (MN) develops was studied by evaluating HBV carriage and proteinuria among 195 family members and household contacts of 31 index HBV carrier children with MN. Unrelated individuals from the communities of these index cases who were negative for HBV served as controls (n = 123). HBV was determined by using third-generation enzyme-linked immunosorbent assay, slot-blot hybridization, and nested polymerase chain reaction. Patterns of proteinuria were determined by using sodium dodecyl sulfate-polyacrylamide gel electrophoresis; immunoglobulin G and haptoglobulin were suggestive of MN. Seventy-two members (36.9%) of the study group (n = 195) were HBV carriers; 21 of these carriers (29.2%) had proteinuria. Twenty-eight members (41.2%) of the study group who were HBV negative (n = 68) and 26.8% of the controls showed proteinuria. This lack of association between HBV carriage and proteinuria remained when controlled for sex and family relationship. HBV was not protective against the development of proteinuria. Proteinuria suggestive of MN was strongly associated with an abnormal protein-creatinine ratio (P: = 0.001), but was not significantly different between subjects and controls (8.7% versus 6.5%; P: = 0.5). Genetic influences or environmental exposures in these subjects may be responsible for the proteinuria, suggesting underlying glomerular basement membrane damage. Discordance between the HBV carrier state and patterns of proteinuria in the study group suggest that HBV and MN may not be causally related or may reflect exceptional interaction between specifically vulnerable individuals and HBV.

Aim: The aim of this paper was to describe the clinical course, histological grading, response to therapies and complications of nephrotic children with focal segmental glomerulosclerosis (FSGS) in Durban, South Africa., Methods: The demographic data, response to drug therapy, complications, renal function and disease status at the last hospital visit were recorded in children diagnosed as FSGS on histology. Biopsies were graded according to the degree of glomerulosclerosis and interstitial involvement., Results: Seventy-five children, 32 Indian and 43 Black were studied. FSGS as a proportion of all children seen with nephrotic syndrome, has risen between 1970 to 1995 from 1.8% to 20% in Indian children and 5% to 28% in Black children. Of those children followed for two years or more, the two racial groups were similar with respect to age at presentation, gender, histological grading, hypertension, renal outcome and response to therapies except for cushingoid features (p < 0.7), alopecia (p < 0.003) and respiratory tract infections (p < 0.001) which occurred more frequently in Indian children. Forty-three children were followed for between 2-16 years: 14 (10 Indian and 4 Black (p < 0.5)) (29%) had grade 1 changes, 24(13 Indian, 11 Black (p < 0.98)) (56%) had grade 2 changes, 3 (Indian (p < 0.26)) (7%) had grade 3 changes and 2 (Black (p < 0.17)) grade 4 changes. Over this period 27 (63%) had normal renal function, 6 (14%) died, 4 (9%) developed impaired renal function, 6 (14%) chronic renal insufficiency and 6 (14%) end-stage renal disease. Three of the latter were transplanted. At the last hospital visit, Indian children had remitted more often (p < 0.6) and Black children suffered relapse more frequently (p < 0.03). Two children had spontaneous disease remission, and 5 had sustained remission following a course of prednisolone and/or cyclophosphamide., Conclusion: This, one of the largest studies of FSGS in children, shows a marked rise in this condition and some differences between Indian and Black children. The outcome in Black children was not as poor as anticipated.

Background: Hepatitis B virus (HBV)-associated membranous nephropathy (HBVMN) is an important cause of childhood nephrotic syndrome in regions endemic for the virus, but little is understood of the biosocial context in which the disease develops. We evaluated HBV status and proteinuria in family members and household contacts of index children with HBVMN to test the hypothesis that HBV carriage and asymptomatic proteinuria are closely linked and may be causally associated., Methods: Thirty-one black children with biopsy-proven HBVMN were the index cases. One hundred and fifty-two family members and 43 black household contacts were the subjects of the study. We assessed HBV carrier status by testing for HBV antigens and antibodies using enzyme-linked immunosorbent assays (ELISA) and for HBV DNA by using slot-blot hybridization and the polymerase chain reaction. Sequencing of the precore region of HBV was done in a subset of both index cases and subjects. Proteinuria was assessed by measuring the urinary protein/creatinine ratio., Results: Seventy-two (37%) of the 195 family members and household contacts were HBV carriers, and 53 (27%) had a protein/creatinine ratio greater than the physiological limit. The frequency of abnormal proteinuria was not significantly different in those with [22 out of 72 (30.5%)] or without [33 out of 104 (32%)] HBV carriage. This lack of association remained when carriers were classified into those who were HBsAg positive only and those with active viral replication (HBsAg and/or HBeAg and/or HBV DNA; P = 0.01). Family members were more predisposed to HBV carriage than household contacts, but abnormal proteinuria was present with equal frequency (P = 0.48). Age had a significant impact on proteinuria, with children less than five years being more likely to have abnormal proteinuria (P = 0.008). The prevalence of abnormal proteinuria in family members and household contacts of the index cases was more than that in community-based controls. The 10 index HBVMN cases and the 14 family members and household contacts who were tested all had HBV of genotype A., Conclusion: These results suggest that the family members and household contacts of children with HBVMN are at very high risk of HBV carriage; they also have asymptomatic proteinuria at a significantly higher rate than community-based controls. The HBV carrier status was not associated with proteinuria, a finding supported by peak prevalences of proteinuria in those under five years but no corresponding peak for HBV carriage. Proteinuria may indicate glomerular basement membrane dysfunction. Environmental and social factors may underpin development of these two covert disorders, but are insufficient to account for the index cases of HBVMN. The emergence of children with HBVMN from such households additionally depends on unidentified and possibly genetic factors.

Hepatitis B virus (HBV)-related membranous nephropathy (MN) is prominent in secondary nephrotic syndromes (NS) in Africa, but the features of this disease and the spectrum of associated glomerulopathies have not been adequately documented in black children. HBV was detected in 93 children with NS included in this study. In 70 patients the histological type was membranous; 46 were followed for a mean of 3.4 years (range 1-11 years). Spontaneous elimination of both HBsAg and HBeAg occurred in 10 (21.7%) patients; 16 (34.8%) cleared HBeAg alone. Co-existing liver disease occurred in 18 (25.7%) and hypocomplementemia (C3,C4) in 47.1% and 11.4% of children, respectively. Sixty-five (92.9%) patients had normal renal function, 1 (1.4%) impaired renal function, 3 (4.3%) chronic renal insufficiency, and 1 (1.4%) end-stage renal disease at last hospital visit. Twelve patients were in remission, all having cleared HBeAg. HBVMN was clinically indistinguishable from 24 children with idiopathic MN, although biochemical characteristics were different. This report delineates the natural history of HBV infection in black South African children with NS, the majority of whom have MN. Disease remission in HBVMN parallels elimination of HBV antigens, particularly HBeAg. Comparison of HBVMN with idiopathic MN revealed clinically indistinguishable characteristics but unexplained biochemical differences.

We report 81 of 107 cases of hemolytic uremic syndrome (HUS), admitted between July 1994 and February 1996, following an outbreak of Shigella dysenteriae type 1 dysentery in Kwazulu/Natal. All patients, excluding 1, were black with a mean age of 38 months (range 1-121); 50 (61.7%) were males. The mean duration of dysentery was 11.3 days (range 1-41) and HUS 15 days (range 1-91). Most patients had acute oliguric renal failure (90.1%), 42 (51.6%) required peritoneal dialysis. Complications included encephalopathy 30 (37.0%), convulsions 12 (14.8%) and hemiplegia 2 (2.3%), gastrointestinal perforation 8 (9.9%), protein losing enteropathy 26 (32.1%), toxic megacolon 4 (4.9%), rectal prolapse 5 (6.2%), hepatitis 11 (13.6%), myocarditis 5 (6.2%), congestive cardiac failure 3 (3.7%), cardiomyopathy 3 (3.7%), infective endocarditis 1 (1.2%), septicemia 15 (18.5%), disseminated intravascular coagulation 17 (21%). Leukemoid reactions were found in 74 (91.3%) patients, hyponatremia in 56 (69.1%), and hypoalbuminemia in 67 (82.7%). Stool culture for Shigella dysenteriae type I was positive in only 7 (8.6%) patients; Shiga toxin assays were not performed. Outcome was as follows: recovery 32 (39.5%), impaired renal function 8 (9.9%), chronic renal failure 26 (32.1%), end-stage renal disease 1 (1.2%), and death 14 (17.3%) patients.

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.

Seven children with steroid-resistant focal segmental glomerulosclerosis (SR-FGS) were placed on a therapeutic protocol of methylprednisolone (MP), oral prednisone (pred) and oral cyclophosphamide (CYC) given over 16 months (regimen A). Another 5 children with SR-FGS were treated with a shorter course of intravenous CYC (monthly doses over 6 months), intravenous MP (3 consecutive daily doses) and oral pred 2 mg/kg (alternate days) (regimen B). With regimen A, 1 child had a short remission, and in the others, oedema subsided, the urine protein/ creatinine ratio decreased, haematuria disappeared and the estimated glomerular filtration rate (GFR) increased. The observation period was 21-42 months and the drugs were well tolerated. With regimen B, 2 patients went into complete remission, 1 had partial remission, 1 failed to respond and another died because of severe concurrent infections. In the responding children, oedema cleared, the urine protein/ creatinine ratio decreased, haematuria disappeared and the GFR rose. The follow-up was between 3 and 34 months. Minor side effects were alopecia and transient hypertension. Both regimens improved the quality of life of most children. Compared with regimen A, regimen B is six times less costly with a quarter of the number of hospital visits. These observations may be of value in designing appropriate multicentre controlled trials, which have been advocated recently, for the rational and optimum management of SR-FGS.

Evidence of virus-induced systemic lupus erythematosus (SLE) is illustrated by documentation of a 10-year old girl who developed SLE (satisfying ARA criteria) soon after infection with Epstein-Barr virus (EBV) infection. She showed complete remission 24 months later following an aggressive course of immunosuppressive therapy. The appearance and disappearance of serological evidence of EBV infection, followed by the onset and complete clinical and serological remission of SLE, which in this case had unusually mild complications, suggest a causal relationship.

Objective: To determine the clinical spectrum of rickets among black children admitted to King Edward VIII Hospital, Durban., Design: Prospective study of black children with rickets beyond infancy., Setting: Hospital-based population; King Edward VIII Hospital, Durban., Participants: A total of 37 patients, aged 1-12 years, were recruited over a 3-year period. None had been on vitamin D or calcium supplementation prior to investigation., Outcome Measures: Rickets was diagnosed clinically, radiologically and biochemically (by a raised alkaline phosphatase value of > 350 IU). Gastro-intestinal, hepatic and renal glomerular causes were excluded in all patients using standard clinical and laboratory criteria., Results: Twenty-three patients were diagnosed as having privational rickets. Nine had 25-hydroxyvitamin D (25-OHD) levels of < 10 ng/ml while 14 had levels within the normal range and were suspected of having dietary calcium deficiency. Ten had a phosphopenic variety of rickets; the remaining 4 had healing or healed rickets on the basis of radiological assessment and normal biochemical values. Pain together with difficulty in walking and bowing of the lower limbs were the main reasons for presentation. The main clinical findings were thickened wrists and ankles and rickety rosary (100%), stunting (85%), anterior bowing of lower limbs (70%) and genu valgum (65%). The calcium and vitamin D deficiency group showed a much better clinical, biochemical and radiological response to therapy than the phosphopenic group on follow-up (18 patients)., Conclusion: This is the first substantial report on rickets in the older child in Natal, which extends the findings from Transvaal, thereby establishing a recognisable pattern of rickets beyond infancy in South Africa. It draws attention to the common clinical presentations which may alert health professionals to the presence of this problem. This report demonstrates that the two commonest types are privational rickets (due to calcium and/or vitamin D deficiency) and phosphopenic rickets.