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PRIMARY FAMILIAL ADDISON'S DISEASE
Author(s) -
WILLIAMS H. E.,
FREEMAN MAVIS
Publication year - 1965
Publication title -
journal of paediatrics and child health
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.631
H-Index - 76
eISSN - 1440-1754
pISSN - 1034-4810
DOI - 10.1111/j.1440-1754.1965.tb02588.x
Subject(s) - medicine , aplasia , disease , adrenal cortex , addison's disease , pediatrics , hypoplasia , rare disease , adrenal insufficiency , pathology
SYNOPSIS Primary familial Addison's Disease is a rare disorder in children and may be manifested by either total adrenal failure with salt losing features, or by partial adrenal failure without salt loss. Knowledge of the pathology is limited and so far aplasia, hypoplasia or degeneration of the adrenal cortex has been described. This communication concerns a remarkable family of 5 children, 3 of whom have distinctive clinical and biochemical findings of partial adrenal failure without salt loss.