Myocardiopathy and isolated glucocorticoid deficit with ACTH resistance: a fortuitous association?

Hereditary syndrome of unresponsiveness to ACTH is a rare autosomal recessive disorder characterized by an isolated glucocorticoid deficiency which is exceptionally associated to regressive cardiomyopathy. A male newborn had iterative episodes of hypoglycemia since the first hours of life. Acute bro...

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Bibliographic Details
Published in:	Archives de pédiatrie : organe officiel de la Société française de pédiatrie Vol. 6; no. 3; pp. 283 - 285
Main Authors:	Vigneron, P, Cabrol, S, Tillous-Borde, I, Tahraoui, L, Bégué, P, Costil, J, Begeot, M, Baudon, J J
Format:	Journal Article
Language:	French
Published:	France 01-03-1999
Subjects:	Adrenal Insufficiency - congenital Adrenal Insufficiency - genetics Cardiomyopathy, Dilated - congenital Cardiomyopathy, Dilated - genetics Genes, Recessive - genetics Genetic Carrier Screening Glucocorticoids - deficiency Humans Hypoglycemia - congenital Hypoglycemia - genetics Infant, Newborn Male Mutation - genetics Receptors, Corticotropin - genetics
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Summary:	Hereditary syndrome of unresponsiveness to ACTH is a rare autosomal recessive disorder characterized by an isolated glucocorticoid deficiency which is exceptionally associated to regressive cardiomyopathy. A male newborn had iterative episodes of hypoglycemia since the first hours of life. Acute bronchiolitis at the age of 14 days was associated with transitory dilated cardiomyopathy. Hypoglycemia was due to glucocorticoid deficiency secondary to ACTH insensitivity. Molecular biology showed a composite heterozygotism for the ACTH receptor gene. Any congenital glucocorticoid deficiency should lead to search for cardiomyopathy.
Bibliography:	ObjectType-Case Study-2 SourceType-Scholarly Journals-1 ObjectType-Feature-4 content type line 23 ObjectType-Report-1 ObjectType-Article-3
ISSN:	0929-693X