Differences in the prevalence of growth, endocrine and vitamin D abnormalities among the various thalassaemia syndromes in North America Journal Article
| Authors: | Vogiatzi, M. G.; Macklin, E. A.; Trachtenberg, F. L.; Fung, E. B.; Cheung, A. M.; Vichinsky, E.; Olivieri, N.; Kirby, M.; Kwiatkowski, J. L.; Cunningham, M.; Holm, I. A.; Fleisher, M.; Grady, R. W.; Peterson, C. M.; Giardina, P. J. |
| Article 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. © 2009 Blackwell Publishing Ltd. |
| Keywords: | adolescent; adult; child; controlled study; preschool child; school child; aged; middle aged; young adult; major clinical study; hepatitis c; liver cirrhosis; united states; biological markers; linear models; disease association; heart disease; logistic models; prevalence; calcium; growth hormone; growth hormone deficiency; short stature; hyperglycemia; blood sampling; growth disorders; vitamin d; diabetes mellitus; thalassemia; beta thalassemia; blood transfusion; insulin; oral antidiabetic agent; cross-sectional studies; vitamin d deficiency; bone mass; physical examination; somatomedin c; testosterone blood level; hypogonadism; urinalysis; hypothyroidism; sex hormone; parathyroid hormone; parathyroid hormone blood level; testosterone; deferoxamine; north america; somatomedin binding protein 3; growth disorder; hormone substitution; medroxyprogesterone acetate; chorionic gonadotropin; growth rate; hypoparathyroidism; endocrinopathies; hypercalciuria; conjugated estrogen; calcium intake; calcium metabolism; ferritin blood level; iron chelation; polyendocrinopathy; sickle cell beta thalassemia; thalassemia major; vitamin blood level; ferritins; somatomedins |
| Journal Title: | British Journal of Haematology |
| Volume: | 146 |
| Issue: | 5 |
| ISSN: | 0007-1048 |
| Publisher: | John Wiley & Sons |
| Date Published: | 2009-09-01 |
| Start Page: | 546 |
| End Page: | 556 |
| Language: | English |
| DOI: | 10.1111/j.1365-2141.2009.07793.x |
| PUBMED: | 19604241 |
| PROVIDER: | scopus |
| PMCID: | PMC2798591 |
| DOI/URL: | |
| Notes: | --- - "Cited By (since 1996): 6" - "Export Date: 30 November 2010" - "CODEN: BJHEA" - "Source: Scopus" |
