[Auto-immune juvenile polyendocrinopathy (author's transl)].
The observation on a young girl of a chronic mucocutaneous candidiasis associated with a pernicious anemia and a primary defect of parathyroid, adrenal and gonadal functions has brought us to a general review on non tumoral, multiple endocrinopathies in children. The analysis of the 47 observations of literature including the symptomatic triad, moniliasis, hypoparathyroidism and adrenal insufficiency, correlated with 107 other cases including only an incomplete triad but other well systematized associations, brings us to enlarge the fame of Whitaker's syndrom, to the more generalized one of auto immune juvenile poly-endocrinopathies. It seems to us that this new entity can be individualized: --by clinical features, linked to the age of occurence, to the predilection for the female sex, the specific chronology of the symptoms (moniliasis, hypoparathyroidism, primary hypothyroidism, adrenal and gonadal insufficiencies, Hashimoto thyroiditis, chronic diarrhoea, alopecia, pernicious anemia, "idiopathic" liver disease). --by genetic findings : compatible with an autosomic recessive transmission. --by the antaomo pathological aspect of the destroyed endocrine glandes on which the lymphocyte infiltration rises. --and by the etiopathogenic mechanism which is certainly linked to a defect of cell mediated immunity. Moreover, these different features seem to differentiate this juvenile syndrom from the polyendocrinopathies of the adult.
D000293:Adolescent; D000309:Adrenal Insufficiency / Q000150:complications; D000328:Adult; D000505:Alopecia / Q000150:complications; D000740:Anemia / Q000150:complications; D001327:Autoimmune Diseases / Q000175:diagnosis / Q000235:genetics; D002177:Candidiasis / Q000150:complications; D002648:Child; D002675:Child, Preschool; D002908:Chronic Disease; D003967:Diarrhea / Q000150:complications; D004700:Endocrine System Diseases / Q000175:diagnosis / Q000235:genetics / Q000276:immunology; D005260:Female; D006801:Humans; D007006:Hypogonadism / Q000150:complications; D007011:Hypoparathyroidism / Q000150:complications; D008107:Liver Diseases / Q000150:complications; D008297:Male; D013959:Thyroid Diseases / Q000150:complications
Les polyendocrinopathies juvéniles auto-immunes.