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Nephrotic syndrome in the 1st year of life.

https://arctichealth.org/en/permalink/ahliterature220554
Source
Pediatr Nephrol. 1993 Aug;7(4):347-53
Publication Type
Article
Date
Aug-1993
Author
R. Habib
Author Affiliation
Inserm U. 192, Hôpital Necker-Enfants Malades, Paris, France.
Source
Pediatr Nephrol. 1993 Aug;7(4):347-53
Date
Aug-1993
Language
English
Publication Type
Article
Keywords
Disorders of Sex Development - pathology
Female
Finland - epidemiology
Glomerular Mesangium - pathology
Glomerulosclerosis, Focal Segmental - pathology
Humans
Infant
Infant, Newborn
Male
Nephrosis - pathology
Nephrotic Syndrome - congenital - epidemiology - pathology
Wilms Tumor - pathology
Abstract
Among the various primary conditions which may be associated with a nephrotic syndrome at birth or within the 1st year of life, the best known is the congenital nephrotic syndrome of finnish type (CNF) characterized by irregular pseudocystic dilatation of proximal tubules. This disease, very frequent in Finland, is often familial with an autosomal recessive mode of inheritance. Patients are steroid resistant, but the cause of death is usually not uraemia but infection or severe diarrhoea with electrolyte imbalance. The second condition is idiopathic nephrosis including minimal change disease, diffuse mesangial proliferation and focal segmental glomerular sclerosis. As opposed to CNF, infants with "early onset nephrosis" may respond to steroid therapy as older children do and may even recover. However, there are no histopathological criteria which allow the certain differentiation of idiopathic nephrosis from CNF. The third condition is diffuse mesangial sclerosis (DMS), a clinicopathological entity which can occur as an isolated finding or be associated with male pseudohermaphroditism and/or Wilms' tumour (Drash syndrome). From a morphological point of view, DMS is easy to differentiate from CNF because of the characteristic pattern of involvement of the glomeruli. From a clinical point of view, the nephropathy, almost always characterized by a nephrotic syndrome, has two distinct features: it is most often diagnosed in the first 2 years of life and it progresses rapidly to end-stage renal failure, which usually occurs before the age of 3 years. The clinical findings in 36 patients with DMS are presented. The nephropathy was isolated in 22 infants and associated with male pseudohermaphroditism and/or Wilms' tumour in 14.(ABSTRACT TRUNCATED AT 250 WORDS)
PubMed ID
8398639 View in PubMed
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