Screening of growth hormone deficiency in short thalassaemic patients and effect of L-carnitine treatment

Abstract

Introduction: Evaluation of growth hormone (GH) in short thalassaemic patients and effect of L-carnitine therapy in those with hormone deficiency.

Material and methods: The study included 30 β-thalassaemic patients aged 13.8 ±1.7 years and 30 children with constitutional short stature as controls. Anthropometric measurements (basal and after 6 months), thyroid profile, insulin-like growth factor-1 (IGF-1) and GH provocation by 2 tests were carried out. Eight patients with inadequate GH response to both clonidine and ITT were given L-carnitine treatment for 6 months. They were re-evaluated (clinically, anthropometrically and in the laboratory by doing GH stimulation test) after 6 months of therapy.

Results: Twelve (40%) patients had sub-clinical hypothyroidism and 10 (33.3%) had growth hormone deficiency (GHD). 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 in patients with constitutional short stature (p < 0.05). A significant positive correlation was found between height and target height (cm). Haemoglobin levels, peak GH, IGF-1 and growth velocity (cm & SDS) were significantly higher and the number of blood transfusions was significantly lower in GH deficiency patients after L-carnitine treatment (p < 0.05). Delta changes were higher in height (cm & SDS), estimated mature height and sitting height and lower in target height - height (SDS and cm) six months after L-carnitine treatment in β-thalassaemic patients with GHD (p < 0.05).

Conclusions: Growth hormone deficiency is an aetiological factor in thalassaemic patients with short stature. L-carnitine can promote GH secretion and growth.

Keywords: L-carnitine treatment; growth hormone; thalassaemia.

Figure 1

Correlation between height and target height of thalassemic patients