ABSTRACT
Objective: Evaluation of growth hormone in poly- transfused thalassemic patients with short stature and effect of L-carnitine therapy in patients with growth hormone deficiency
Method: The study included. 30 beta-thalassemic patients with mean age 13.8 +/-1.7 yr and 30 children with constitutional short stature as a control. Anthropometric measurements, thyroid profile, insulin like growth factor -1 [IGF-1] and growth hormone [GH] provocation by 2 tests were done at the beginning of the study. Anthropometric measurements and pubertal assessment were done for all patients after 6 months. Eight patients with inadequate GH response to both clonidine and ITT were given L-Carnitine treatment at a dose of 50 mg/kg/day divided into three doses for 6 months. They were reevaluated after 6 months of therapy
Results: Twelve [40%] patients had sub-clinical hypothyroidism [diagnosed by normal free thyroxin level and elevated thyroid stimulating hormone], 10 [33.3%] patients had growth hormone deficiency. Peak GH and growth velocity /{cm and Standard Deviation Score [SDS]] were significantly lower while weight [SDS] and weight / height SDS were significantly higher than patients with constitutional short stature [P < 0.05]. A significant positive correlation was found between height and target height [cm]. Eight patients with growth hormone deficiency were given L-carnitine for 6 months [50 mg/Kg/day]. After L-carnitine treatment hemoglobin level, peak GH, IGF-1 and growth velocity [cm and SDS] were significantly increase and the number of blood transfusions was significantly decrease [p<0.05]. Delta changes were higher in height [cm and SDS], estimated mature height, sitting height and lower in target height - height [SDS and cm] six months after L-carnitine treatment in beta-thalassemic patients with growth hormone deficiency [p <0.05]
Conclusion: Growth hormone[GH] deficiency is an etiologic factor in beta-thalassemia patients with short stature. L-Carnitine can promote GH secretion and growth