Is There A Relation Between Cardiomyopathy And Isolated Glucocorticoid Deficiency With Acth Insensitivity?

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.Case report. - 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.Conclusion. - Any congenital glucocorticoid deficiency should lead to search for cardiomyopathy. (C) 1999 Elsevier, Paris.