title: Autoimmune polyglandular syndrome type 1 with reversible dilated cardiomyopathy: complete recovery after correction of hypocalcemia and hypocortisolemia.
abstract: Autoimmune polyglandular syndrome type 1 (APS1) - characterized by the triad of mucocutaneous candidiasis, hypoparathyroidism, and primary adrenal insufficiency - is an uncommon entity. In this case report we describe the case of a young girl who presented with classic features of APS1 and dilated cardiomyopathy, which were missed during her presentation to the children's hospital. Her condition improved only when appropriate replacement therapy in the form of calcium, calcitriol and hydrocortisone was instituted. Hypocalcemia and hypocortisol state are curable causes of myocardial dysfunction and subsequent congestive cardiac failure, and should always be considered in the differential diagnosis of resistant congestive cardiac failure, especially in children. To the best of our knowledge, cardiomyopathy in the setting of APS1 has never been described previously and can remain unrecognized for a long time and present with life threatening complications.
authors: Department of Endocrinology, Sher I Kashmir Institute of Medical Sciences, Soura, Srinagar, Jammu and Kashmir, India.
authors: Arshad Iqbal
authors: AI
authors: Wani
authors: Khalid Jamal
authors: KJ
authors: Farooqui
authors: Mir Iftikhar
authors: MI
authors: Bashir
authors: Shahnaz Ahmad
authors: SA
authors: Mir
authors: Ajaz Ahmad
authors: AA
authors: Lone
authors: Shariq Rashid
authors: SR
authors: Masoodi
mesh_terms: D002118:Calcium / Q000627:therapeutic use; D002311:Cardiomyopathy, Dilated / Q000150:complications* / Q000188:drug therapy; D002648:Child; D005260:Female; D006801:Humans; D006854:Hydrocortisone / Q000172:deficiency* / Q000627:therapeutic use; D006996:Hypocalcemia / Q000188:drug therapy / Q000209:etiology*; D008279:Magnetic Resonance Imaging; D016884:Polyendocrinopathies, Autoimmune / Q000150:complications* / Q000188:drug therapy / Q000473:pathology; D012074:Remission Induction; D016896:Treatment Outcome
chemical_list: D002118:Calcium; D006854:Hydrocortisone