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. 2009 Sep;146(5):546-56.
doi: 10.1111/j.1365-2141.2009.07793.x. Epub 2009 Jul 13.

Differences in the prevalence of growth, endocrine and vitamin D abnormalities among the various thalassaemia syndromes in North America

Maria G Vogiatzi  1 , Eric A Macklin, Felicia L Trachtenberg, Ellen B Fung, Angela M Cheung, Elliott Vichinsky, Nancy Olivieri, Melody Kirby, Janet L Kwiatkowski, Melody Cunningham, Ingrid A Holm, Martin Fleisher, Robert W Grady, Charles M Peterson, Patricia J Giardina; Thalassemia Clinical Research Network
Collaborators, Affiliations

Differences in the prevalence of growth, endocrine and vitamin D abnormalities among the various thalassaemia syndromes in North America

Maria G Vogiatzi et al. Br J Haematol. 2009 Sep.

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.

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Figures

Figure 1
Figure 1
Plots of height, bone age and weight of study participants. A. Stature of male participants vs. calendar age (●) and bone age (■). B: Stature of female participants vs. calendar age (●) and bone age (■). Individuals are connected by dashed lines. C: Weight of male participants vs. calendar age. D: Weight of female participants vs. calendar age.
Figure 2
Figure 2
IGF1 and IGFBP3 vs. age stratified by gender and hypogonadal status. +: hypogonadal, o: not hypogonadal. IGF1 concentrations below normal range were found in 57.7% of children 6-11y, 56.8% of those 12-19y and 83.3% of subjects older than 20y. All children 6-11y had normal IGFBP3 levels. IGFBP3 concentrations below normal range were found in 12.5% of children 12-19y and 58.3% of subjects older than 20y.
Figure 3
Figure 3
Parathyroid hormone and total Calcium intake by vitamin D sufficiency and diagnosis. Parathyroid hormone is significantly lower in beta TM compared to beta TI (p=0.02), and decreases with vitamin D (p<0.001). Total Calcium intakes does not significantly vary by diagnosis (p=0.76) or vitamin D sufficiency (p=0.88).
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