The Indian Journal of Pediatrics

, Volume 72, Issue 2, pp 159–164 | Cite as

Growth of children with β-thalassemia major

  • Louis C. K. Low
Symposium on Growth and Its Disorders

Abstract

Hypertransfusion and regular chelation therapy have allowed improved survival in patients with thalassemia major (TM). Despite medical advances, growth failure and hypogonadism remain significant clinical problems in these patients in adolescence. Disproportionate truncal shortening which is common especially among adolescents with thalassemia, is due to platyspondyly resulting from a combination of factors like hemosiderosis, desferrioxamine toxicity or deficiency of trace elements. Although growth hormone (GH) deficiency and GH neurosecretory dysfunction have been described in TM patients, most short TM patients have normal GH reserve. The low serum IGF-1 and IGFBP-3 concentrations in TM patients despite having normal GH reserve and serum GH binding protein levels suggest that a state of secondary GH insensitivity exists. The pubertal growth spurt may be impaired in TM patients going through spontaneous or induced puberty and may have a negative effect on final adult height. GH therapy in dosages ranging from 0.5–1.0 IU/kg/wk has resulted in a significant improvement in growth velocity in short TM children without any adverse effects on skeletal maturation, blood pressure, glucose tolerance and serum lipids. There is limited evidence that GH treatment can result in an improved final adult height in short TM children. Careful and regular clinical and biochemical monitoring should be preformed on these patients while they are treated with GH.

Key words

Thalassaemia major Truncal shortening GH insensitivity 

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Copyright information

© Dr. K C Chaudhuri Foundation 2005

Authors and Affiliations

  • Louis C. K. Low
    • 1
  1. 1.Department of Pediatrics and Adolescent MedicineThe University of Hong Kong, Queen Marg HospitalHong Kong SAR, PR China

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