Renée Habib

Berger's disease in children: Natural history and outcome.

The clinical course and outcome of 91 children less than 15 years of age at onset and followed for at least 1 year have been retrospectively analyzed. The course has been characterized by recurrent macroscopic hematuria in 74 patients, by proteinuria-microscopic hematuria and a single episode of macroscopic hematuria occurring either at onset or a few months later in 8, by proteinuria-microscopic hematuria in 7, and by proteinuria only in 1. Lastly, one patient showed rapidly progressive renal failure. Four groups were identified by light microscopy: minimal glomerular changes (26), focal and segmental glomerulonephritis (41), pure mesangial proliferation (3) and proliferative glomerulonephritis with crescents (21). A good correlation was found between the glomerular lesions observed by light microscopy and the outcome. In this series we have not observed a dramatic clinical deterioration suggesting a transformation from one histologic type to another, as reported by others. None of the 70 patients belonging to the first three groups has impaired renal function but two with focal and segmental glomerulonephritis have developed hypertension. Although the clinical course is benign, many patients have, at the last observation, an abnormal urinalysis characterized by microscopic hematuria and/or mild proteinuria; the proteinuria is over 1 g/24 h in six patients with focal and segmental glomerulonephritis. Ten patients remained in clinical remission for several years, but mesangial IgA deposits were still present in the only patient who had a repeat biopsy while in remission. In contrast, none of the patients with proliferative glomerulonephritis with crescents has had a prolonged remission. Six patients developed terminal renal failure 0.7, 0.11, 2, 4, 8 and 10 years after onset. Two additional patients are in moderate chronic renal failure with hypertension 10 and 12 years after onset. Most children show a persistent nephropathy, (in five proteinuria is over 1 g/24 h), and two of them have developed hypertension. Therapeutic trials using drugs with side-effects should, therefore, be used only in this group of patients.

Extramembranous glomerulonephritis inchildren: Report of 50 cases

Pathologic and clinical data concerning 50 children with extramembranous glomerulonephritis (EMGN) are reported. This type of glomerular lesion is characterized by thickening of the capillary walls, due to subepithelial deposits without endocapillary proliferation. EMGN occurred in all age groups including infancy. It was often latent and discovered by routine urinalysis. Nephrotic syndrome and hematuria were frequent but not constant. In no instance could a specific etiology be demonstrated. Corticosteroids as well as immunosuppressive drugs seemed ineffective. Remissions often occurred while patients received no treatment. Patients were followed for one to ten years. Twenty-six children had complete remissions, 17 of them without subsequent relapse. Twenty-four patients had persistent proteinuria. The only five patients who developed chronic renal failure presented with persistent biochemical changes of the nephrotic syndrome. EMGN is not an uncommon nephropathy in children, but seems to have a more benign course in children than in the older age groups hitherto reported.

Glomerular Basement Membrane Changes in Hereditary Glomerular Diseases

Ultrastructural glomerular basement membrane changes are present in most hereditary glomerular diseases: thick and thin basement membrane with splitting of the lamina densa in Alports syndrome, thin basement membrane in familial benign essential hematuria, and thick basement membrane with the presence of collagen-like fibrils in the nail-patella syndrome. They are useful markers for diagnosis. Moreover, their knowledge has set the problem of the primary biochemical defect in basement membrane metabolism accounting for morphological abnormalities and clinical disturbances. Further biochemical and immunochemical investigations are still required for a better understanding of hereditary glomerular diseases.

Coexistence of antenatal, infantile, and juvenile nephrotic syndrome in a single family

1. Bunn HF, Haney DN, Kamin S, et al: The biosynthesis of human hemoglobin A~c: Slow glycosylation of hemoglobin in vivo, J Clin Invest 57:1652, 1976. 2. Koeulg RJ, Peterson CM, Jones RL, et al: Correlation of glucose regulation and hemoglobin AI0 in diabetes mellitus, N Engl J Med 295:417, 1976. 3. Widness JA, Rogler-Brown TL, McCormick KL, et al: Rapid fluctuations in glycohemoglobin (hemoglobin A~) related to acute changes in glucose, J Lab Clin Med 95:386, 1980. 4. McDonald M J, Shapiro R, Bleichman M, et al: Glycosylated minor components of human adult hemoglobin, J Biol Chem 253:2327, 1978. 5. Haney DN, and Bunn HF; Glycosylation of hemoglobin in vitro: Affinity labeling of hemoglobin by glucose-6-phosphate, Proc Natl Acad Sci 73:3534, 1976. 6. Stevens VJ, Vlassara H, Abati A, and Cerami A: Nonenzymatic glycosylation of hemoglobin, J Biol Chem 252:2998, 1977. 7. Garrick LM, McDonald M J, Shapiro R, et al: Structural analysis of the minor human hemoglobin components: Hb A~I Hb AI~ 2 and Hb A~b, Eur J Biochem 106:353, 1980.