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

Abstract

IntroductionEvaluation of growth hormone (GH) in short thalassaemic patients and effect of L-carnitine therapy in those with hormone deficiency.Material and methodsThe 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.ResultsTwelve (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).ConclusionsGrowth hormone deficiency is an aetiological factor in thalassaemic patients with short stature. L-carnitine can promote GH secretion and growth.

l‐carnitine downregulate the muscle wasting effect of glucocorticoids in pemphigus patient: A randomized double‐blind placebo‐controlled study

“…In the present study, inter and intra‐comparison showed a significant rise in level of IGF‐1. The present study confirms recent studies in humans, pigs, chickens, and rats which revealed that supplementation of carnitine increases plasma IGF‐1 concentrations (Beshlawy et al, 2010 ; Brown et al, 2008 ; Di Marzio et al, 1999 ; Doberenz et al, 2006 ; Heo et al, 2001 ; Keller et al, 2011 ; Keller et al, 2013 ; Kita et al, 2002 ). Instead, the result of the study in patients with liver cirrhosis showed that carnitine supplementation did not affect the IGF1 levels (Ohara et al, 2018 ) also Circulating IGF‐I was not affected by LC treatment on porcine fetal (Waylan et al, 2005 ).…”

A Systematic Review and Meta-Analysis of Stature Growth Complications in β-thalassemia Major Patients

“…After that, 433 records were excluded, and 213 records entered full-text assessment for eligibility criteria. Finally, 74 studies included in the meta-analysis ( Figure 1 ) [ 6 7 11 18 19 20 21 22 23 24 25 26 27 28 29 30 31 32 33 34 35 36 37 38 39 40 41 42 43 44 45 46 47 48 49 50 51 52 53 54 55 56 57 58 59 60 61 62 63 64 65 66 67 68 69 70 71 72 73 74 75 76 77 78 79 80 81 82 83 84 85 86 87 88 ].…”