Growth hormone (GH) deficiency in patients with β-thalassemia major and the efficacy of recombinant GH treatment
- 240 Downloads
Patients with β-thalassemia major still suffer growth retardation. After excluding patients with cortisol deficiency, hypothyroidism, hypogonadism, delayed puberty, malnutrition, severe congestive heart failure, and severely impaired liver function, 29 patients were enrolled in this study. Fifteen (52%) patients exhibited growth retardation and underwent two growth hormone (GH) provocation tests. Eight (53%) of the 15 patients had GH deficiency and were subsequently treated with subcutaneous recombinant human GH (Genotropin, Pharmacia Corporation, Sweden). Growth velocity increased from the pretreatment rate of 3.1±0.4 cm/year to 7.1±1.6 cm/yr (p<0.001) after 1 year and to 6.8±1.3 cm/year (p<0.001) after 2 years. Patients with growth retardation had lower insulin like growth factor-1 (p=0.001) and insulin like growth factor binding protein-3 (p=0.003) levels than those without growth retardation. In patients with β-thalassemia major, growth retardation is a common complication and GH deficiency plays an important role. Thalassemic patients with GH deficiency can safely increase their growth velocity with recombinant human GH for2 years; however, the effect on final height still needs to be determined.
Keywordsβ-thalassemia major Growth hormone (GH) deficiency Recombinant human GH
We would like to thank Miss Tsai-Chung Li, a teacher at the institute of Chinese Medicine at the China Medical College in Taichung, for her statistical assistance and interpretation.
- 6.Jain M, Sinha Rs, Chellani H, Anad NK (1995) Assessment of thyroid function and its role in body growth in thalassemia major. Indian-Pediatr 32:312–319Google Scholar
- 7.Roth C, Pekrun A, Bartz M, Jarry H, Eber S, Lakomek M, Schroter W (1997) Short stature and failure of pubertal development in thalassaemia major: evidence for hypothalamic neurosecretory dysfunction of growth hormone secretion and defective pituitary gonadotropin secretion. Eur J Pediatr 156:777–783PubMedGoogle Scholar
- 20.Walter Chen, Jasson Chiang, Po Chao Huang (1999) Revised growth charts, Taiwan, 1997. M Taiwan J Med 4:256–263Google Scholar
- 21.Greulich WW, Pyle SI (1970) Radiographic Atlas of Skeletal Development of the Hand and Wrist. Stanford University, New YorkGoogle Scholar