Recent investigations have shown that the nephrotic syndrome in very young infants cannot be considered a homogeneous entity as is suggested by Finnish authors (congenital nephrotic syndrome), since in this disease distinct histologic and clinical patterns have been described. In 3 patients recently investigated 2 morphologic pictures were documented by the light, electron microscopy and immunofluorescence studies. In 2, the histologic diagnosis was consistent with 'diffuse mesangial sclerosis' (DMS), while proliferative lesions of unclear type were observed in the third patient. A rapid progression towards death characterized the clinical course of the infants with DMS, even though infectious complications rather than true renal failure were the leading causes of death. The presence of immunoglobulins and complement deposits in 2 patients with DMS, as well as in the infant with proliferative lesions, gives rise to pathogenetic problems in the nephrotic syndrome developing during the first year of life.
|Translated title of the contribution||Nephrotic syndrome in very young infants: optical, electron microscope and immunofluorescence studies in three patients|
|Number of pages||10|
|Publication status||Published - 1976|
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