Circulating Growth Hormone (GH), Insulin-like Growth Factor-I (IGF-I) and Free Thyroxine, GH Response to Clonidine Provocation and CT Scanning of the Hypothalamic-pituitary Area in Children with Sickle Cell Disease

Abstract

Serum growth hormone (GH), cortisol, free thyroxine (FT4), thyroid-stimulating hormone (TSH), and insulin like growth factor I (IGF-I) concentrations were measured in 15 children with sickle cell disease (SCD) together with their heights < 5th percentile for age and gender, and in 15 healthy age-matched children who had normal variant short stature (NVSS). GH response to an oral dose of clonidine (0.15 mg/m2) and cortisol response to ACTH stimulation were determined in the two groups. Children with SCD had significantly lower serum concentrations of IGF-I and decreased GH response to stimulation. Eight out of the 15 children with SCD did not mount an appropriate GH response to clonidine provocation (> 10 micrograms/l). CT scanning of the hypothalamic-pituitary area in those eight children with SCD revealed a partial or complete empty sella in all of them. It appears that defective GH release, and consequently low IGF-I production and slow growth velocity in children with SCD might be secondary to hypoxic-vascular insults to their hypothalamic-pituitary axis during one or more of the sickling episodes.

Growth and nutritional status of children with homozygous sickle cell disease

“…88 Interest in growth hormone dysfunction has motivated a series of studies by Soliman and co-workers who demonstrated abnormalities in the growth hormone (GH)/insulinlike growth factor-I (IGF-I) axis. 54,[89][90][91][92] In a study of 21 pre-pubertal SS children with poor growth (height ,10th centile), defective GH secretion and low insulin-like IGF-1 and IGF binding-protein-3 were demonstrated in 43%, with a reduced response of IGF-1 production to GH injection. The disease severity score was significantly higher in the group with defective GH secretion than in the group with normal GH secretion.…”

Growth Hormone Deficiency in Patients with Sickle Cell Disease and Growth Failure

“…The authors concluded that vascular insult to the hypothalamicpituitary axis appears to be the cause of growth failure in these patients 6 . b measured serum GH and IGF-I, using Clonidine provocation, in 15 patients with SCD with short stature (heights -2 SD or more below the mean) and compared them to healthy age-matched children with normal variant short stature.…”

“…Brought to you by | University of California -San Francisco Authenticated Download Date | 2/20/15 2:50 PM deficiency was associated with growth failure in one study, and pituitary infarction has also been proposed as a cause of GH deficiency in patients with sickle cell disease with short stature 6 .…”

Does blood transfusion affect pituitary gonadal axis and sperm parameters in young males with sickle cell disease?

“…In support of this effect, testicular and pituitary gland volumes of patients with SCD are reported to be smaller with many infarcts in the testicles and pituitary gland. [222340]…”

“…Therefore, in the transfusion-dependent type of SCD, iron toxicity may also add to different organ damage including the pituitary gland and gonads. [212223]…”