title: A rare cause of heart failure in iron-overload thalassaemic patients-primary hypoparathyroidism.
abstract: Hypocalcemia due to hypoparathyroidism (HPT) is a late complication of iron-overloaded patients with b-thalassaemia major (TM). The majority of patients have mild disease with parasthesias, while in the more severe form tetany, seizures or cardiac failure may occur. In the last 20 years we observed heart failure in 2 out of 38 (5.2%) TM patients (aged 18 and 22 years) with hypocalcemia secondary to HPT associated to iron overload. Calcium supplementation and vitamin D induced correction of hypocalcemia and resulted in an improvement of cardiac function. Calcium plays a key role in the maintenance and regulation of normal cardiac function. Extra-cellular calcium is indispensable for the contractile process since the sarcoplasmatic reticulum is unable to maintain a sufficient amount of calcium to trigger myocardial contraction. In conclusion, our observations stress the importance of a regular iron chelation therapy, adherence to treatment of endocrine complication and regular follow-up of TM patients with hypocalcemia.
authors.affiliation: Pediatric and Adolescent Unit, St Anna Hospital, Ferrara, Italy.
authors.forename: V
authors.initials: V
authors.lastname: De Sanctis
authors.forename: G
authors.initials: G
authors.lastname: Borsari
authors.forename: S
authors.initials: S
authors.lastname: Brachi
authors.forename: E
authors.initials: E
authors.lastname: Gubellini
authors.forename: M R
authors.initials: MR
authors.lastname: Gamberini
authors.forename: G
authors.initials: G
authors.lastname: Carandina
mesh_terms: D000293:Adolescent; D003937:Diagnosis, Differential; D006333:Heart Failure / Q000175:diagnosis / Q000453:epidemiology*; D006801:Humans; D008297:Male; D017086:beta-Thalassemia / Q000453:epidemiology*