title: Differences in the prevalence of growth, endocrine and vitamin D abnormalities among the various thalassaemia syndromes in North America.
abstract: This study aimed to determine differences in the rates of growth, endocrine- and calcium-related abnormalities in the various thalassemia syndromes in North America treated with current therapies. Medical history, physical examinations and blood and urine collections were obtained from patients with all thalassemia syndromes age 6 years and older in the Thalassemia Clinical Research Network. 361 subjects, 49% male, mean age 23.2 years (range 6.1-75 years) were studied. Approximately 25% of children and adults, regardless of the thalassemia syndrome, had short stature. Overall growth in children was mildly affected. Final height was close to midparental height (z = -0.73 +/- 1.24). Patients with beta thalassemia major (TM) had higher rates of hypogonadism, multiple endocrinopathies, worse hyperglycaemia, subclinical hypoparathyroidism and hypercalciuria. Hypogonadism remained the most frequent endocrinopathy and was frequently under-treated. 12.8% of the subjects had 25 vitamin D concentrations less than 27 nmol/l and 82% less than 75 nmol/l, regardless of the thalassemia syndrome. Adolescents had lower 25 vitamin D levels than children and adults. Compared to patients with other thalassemia syndromes, those with beta TM suffered from higher rates of multiple endocrinopathies, abnormal calcium metabolism and hypercalciuria. Vitamin D abnormalities were high among adolescents.
authors: Department of Pediatrics, Pediatric Endocrinology, New York Presbyterian Hospital/Weill Medical College of Cornell, New York, NY 10065, USA. mvogiatz@med.cornell.edu
authors: Maria G
authors: MG
authors: Vogiatzi
authors: Eric A
authors: EA
authors: Macklin
authors: Felicia L
authors: FL
authors: Trachtenberg
authors: Ellen B
authors: EB
authors: Fung
authors: Angela M
authors: AM
authors: Cheung
authors: Elliott
authors: E
authors: Vichinsky
authors: Nancy
authors: N
authors: Olivieri
authors: Melody
authors: M
authors: Kirby
authors: Janet L
authors: JL
authors: Kwiatkowski
authors: Melody
authors: M
authors: Cunningham
authors: Ingrid A
authors: IA
authors: Holm
authors: Martin
authors: M
authors: Fleisher
authors: Robert W
authors: RW
authors: Grady
authors: Charles M
authors: CM
authors: Peterson
authors: Patricia J
authors: PJ
authors: Giardina
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D015415:Biomarkers / Q000097:blood; D002118:Calcium / Q000097:blood; D002648:Child; D003430:Cross-Sectional Studies; D005260:Female; D005293:Ferritins / Q000097:blood; D006130:Growth Disorders / Q000097:blood / Q000150:complications* / Q000503:physiopathology; D013006:Growth Hormone / Q000172:deficiency; D006801:Humans; D007006:Hypogonadism / Q000097:blood / Q000150:complications / Q000503:physiopathology; D016014:Linear Models; D016015:Logistic Models; D008297:Male; D008875:Middle Aged; D010281:Parathyroid Hormone / Q000097:blood; D015995:Prevalence; D013002:Somatomedins / Q000032:analysis; D013789:Thalassemia / Q000150:complications* / Q000453:epidemiology* / Q000378:metabolism / Q000503:physiopathology; D014481:United States / Q000453:epidemiology; D014808:Vitamin D Deficiency / Q000097:blood / Q000150:complications* / Q000503:physiopathology; D055815:Young Adult
chemical_list: D015415:Biomarkers; D010281:Parathyroid Hormone; D013002:Somatomedins; D013006:Growth Hormone; D005293:Ferritins; D002118:Calcium