Hepatitis B virus-associated glomerulonephritis: electron microscopic studies in 98 children.

Ninety-eight children with glomerulonephritis concomitant with hepatitis B surface HBs antigenemia were studied, the antigenemia being first documented at the clinical onset of glomerulopathy. Initial diagnoses, based on examination of the paraffin sections, varied, membrano-proliferative, mesangial, and membranous glomerulonephritis being most frequently considered. However, electron microscopic examination showed that 77 children had a uniform type of glomerulopathy, irrespective of the light microscopic appearance. This type was diagnosed as secondary membranous glomerulonephritis. The clinical course of this nephropathy was relatively indolent and short. Moreover, in many children, elimination of some hepatitis B virus (HBV) antigens from the circulation was also associated with clinical remission of glomerulopathy. The remaining 21 children with HBs antigenemia had various morphological forms of glomerulonephritis, these being similar to their idiopathic counterparts in both morphology and clinical course. The distinct clinical and morphological picture of secondary membranous glomerulonephritis with HBs antigenemia occurring in 77 of 98 children supports the hypothesis that HBsV-associated glomerulonephritis is of the secondary membranous type. Thus, we conclude that in children HBV antigenemia associated with glomerulonephritis other than secondary membranous is coincidental.

Prognostic factors in the hemolytic-uremic syndrome.

During 1972-1986, 142 children with the hemolytic-uremic syndrome were treated. Most of them were infants (73%). The total mortality rate reached 25.4%. Computer analysis revealed the following risk factors of a fatal outcome: severe gastrointestinal symptoms during the prodromal period, coma, convulsions, malignant hypertension, persistence of prodromal symptoms over 7 days, hyperkalemia over 7 mmol/l, acidosis with bicarbonate level less than 15 mmol/l, a delay of over 5 days in starting dialysis, and transport to dialysis unit of over 100 km. The greatest risk of death existed during the first 3 weeks from onset. Among 142 children, 106 survived the acute phase. They were followed up from 2 to 16 years. Nine were lost to follow-up. Twelve children developed chronic renal failure.

Henoch-Schönlein nephritis in children--a clinicopathological study.

36 children with Henoch-Schönlein nephritis had their renal biopsy specimens studied by light and electron microscopic and immunofluorescence antibody techniques. Though no pathognomic changes were found the histological picture was characteristic. The severity of histological changes correlated well with the clinical manifestation and disease persistence. IMF studies showed characteristic mesangial IgA staining. The most prominent ultrastructural feature was segmental mesangial and subendothelial deposits with basement membrane changes. The amount of deposits was a good exponent of disease activity-children with few deposits recovering shortly. In spite of long duration, the outcome after an average 4 year follow-up was good, the majority of children having improved and renal insufficiency developing rarely. Renal biopsy is essential for an estimation of severity of renal disease and enables prognosis of disease persistence and long term outlook.

[Gastrointestinal complications in children with hemolytic-uremic syndrome]. / Gastrointestinale Komplikationen bei Kindern mit hämolytisch-urämischem Syndrom.

Eight children out of 106 with haemolytic-uraemic syndrome (HUS) had marked gastrointestinal complications requiring close cooperation between the paediatrician and paediatric surgeon. These children showed signs of ileus and peritonitis. Six children hat these complications in the prodromal stage, whereas the other two children showed them in the anuria stage. The complications were closely connected with multiple erosions of the mucosa and ulcerations of the intestinal wall which resulted in perforation of the intestinal wall in two cases. In two further cases, intestinal vagination was seen which was limited toward the small intestine. All operated children died. The authors are of the opinion that prognosis in HUS with severe gastrointestinal complications is very infaust.

Mesangial proliferative glomerulonephritis in children.

Over a ten year period 105 children with a histological diagnosis of a mesangial proliferative glomerulonephritis were diagnosed. Patients were divided into two groups according to their clinical presentation at the time of diagnosis. Ninety two children presented with nephrotic syndrome (NS) of whom 82 received steroid therapy. No response was observed in 26 children and in 56 remissions were short in duration and subsequent relapses were frequent. Eighty nine children with the nephrotic syndrome were treated with cyclophosphamide (CP) of whom 26 had a steroid resistant NS, 53 were steroid dependent and 10 were previously untreated. Eighty four entered remission with a mean duration of 46 months. Only 5 children did not respond to treatment with CP. No correlation could be found between the results of therapy and the degree of morphological changes on examination of renal biopsy. The second group consisted of 13 children presenting with a persistent nephritic syndrome and or proteinuria. These children were untreated and no progression of renal disease was observed after several years follow up.