[Nephrotic syndrome in infancy - a heterogenous disease. A case of sporadic connatal nephrotic syndrome (author's transl)].
The course of illness in a patient with connatal nephrotic syndrome is reported. It started already during the first week, renal failure was absent. The picture differs, however, from the congenital Finnish nephrotic syndrome in the following points: No family history, infection during pregnancy, unremarkable birthweight (3075 g) and weight of placenta (690 g), early micro-hematuria, and rise of IgM, arterial hypertension and partial sensitivity to steroids with incomplete remissions and finally duration of life of 1 year and 8 months. The difference from the Finnish type is confirmed with light- and electron-microscopy and immuno-fluorescence investigations. Morphological changes make it appear that it belongs into group 3 of nephrotic syndrome after Habib (16), with its minimal and focal glomerular changes. An attempt is made to devise a classification of the nephrotic syndrome during the first year of Life according to its cause. The importance of an absolute indication for renal biopsy is stressed.
D001724:Birth Weight; D005260:Female; D006417:Hematuria / Q000150:complications; D006801:Humans; D006973:Hypertension / Q000150:complications; D007075:Immunoglobulin M / Q000032:analysis; D007223:Infant; D008297:Male; D008854:Microscopy, Electron; D008856:Microscopy, Fluorescence; D009929:Organ Size; D010920:Placenta / Q000033:anatomy & histology; D011247:Pregnancy; D011251:Pregnancy Complications, Infectious / Q000150:complications; D012075:Remission, Spontaneous
D007075:Immunoglobulin M
Die Säuglingsnephrose - eine heterogene Erkrankung. Darstellung an Hand eines Falles von sporadischem konnatalem nephrotischen Syndrom