title: Endocrinopathies in Turkish children with Beta thalassemia major: results from a single center study.
abstract: The endocrinological complications in β-thalassemia major patients do affect the life quality to a large extend. In this study, the endocrinological complications of 47 β-thalassemia patients, who have been followed-up at our hospital's pediatric hematology department, were evaluated. Out of β-thalassemia major cases included to this study, the 55.3% was male and 44.7% was female. The patients' mean levels of ferritin, whose mean age was 10.0 ± 4.5 years (2-20 years), were 2497 ± 1469 ng/mL (472-8558 ng/mL). At least one endocrinological pathology in 27 out of 47 (57.4%) and more than one endocrinological pathology in 14 out of 47 (29.7%) thalassemia patients were observed. The most frequently observed complication in followed-up cases was vitamin D insufficiency and deficiency (78.2%). The other complications in decreasing order were pubertal failure (41.6%), growth retardation (25.5%), decreased bone-mineral density (22.2%), secondary hyperparathyroidism (11.5%), overt hypothyroidism (4.25%), subclinical hypothyroidism (2.12%), and impaired glucose tolerance (2.12%). There was no statistically significant difference between serum mean ferritin level and endocrin complications (P > .05). Four patients (8.5%) had decreased signal intensity in pituitary magnetic resonance imaging (MRI) but this finding was not associated with ferritin levels (P = .87). MRI parameters were similar between patients with and without gonadal dysfunction. Mean height of the pituitary gland was 4.98 ± 1.1 mm (3-9 mm) and this was similar to those normal values in the literature. Ferritin levels were not correlated with pituitary height (P > .05). Beta thalassemia major, having the potential of leading to multisystemic complications, is a chronic disease that should be treated and followed-up by a multidisciplinary approach. Due to frequently encountered endocrinological complications, beta thalassemic patients should be followed-up regularly by hematology and endocrinology departments in coordination.
authors.affiliation: 1Department of Pediatric Hematology, Ankara Children's Hematology and Oncology Hospital, Ankara, Turkey.
authors.forename: Pamir
authors.initials: P
authors.lastname: Isik
authors.forename: Nese
authors.initials: N
authors.lastname: Yarali
authors.forename: Betül
authors.initials: B
authors.lastname: Tavil
authors.forename: Fatma
authors.initials: F
authors.lastname: Demirel
authors.forename: Gülşah Bayram
authors.initials: GB
authors.lastname: Karacam
authors.forename: Rukiye Unsal
authors.initials: RU
authors.lastname: Sac
authors.forename: Ali
authors.initials: A
authors.lastname: Fettah
authors.forename: Serdar
authors.initials: S
authors.lastname: Ozkasap
authors.forename: Abdurrahman
authors.initials: A
authors.lastname: Kara
authors.forename: Bahattin
authors.initials: B
authors.lastname: Tunc
mesh_terms: D000293:Adolescent; D015519:Bone Density; D002648:Child; D002675:Child, Preschool; D004700:Endocrine System Diseases / Q000209:etiology*; D005260:Female; D005293:Ferritins / Q000097:blood; D006801:Humans; D008297:Male; D014808:Vitamin D Deficiency / Q000209:etiology; D055815:Young Adult; D017086:beta-Thalassemia / Q000150:complications*
chemical_list: D005293:Ferritins
keywords: endocrinology; pediatrics; radiology; thalassemia