Endocrine Abstracts

Introduction: Growth retardation in sickle cell children is common and multifactorial. Recent evidence suggests damage to the somatotropic axis. We report the case of a patient followed for homozygous sickle cell anemia and in whom we discover an associated GH deficiency.

Observation: It is a 13 year old child followed for homozygous sickle cell anemia, splenectomized for 2 years, not polytransfused. We were sent to the hematology department before a delay in weight gain with a delay in bone maturation (bone age = 9 years). On examination, he was 139 cm <-2DS in height and 30 kg <3rd percentile in weight. The initial assessment showed a low IGF1 level, a correct nutritional balance and a negative celiac disease serology. Endocrine exploration was in favor of growth hormone deficiency (the GH peak on catapressan test was 6.51 ng/ml and the GH peak on insulin hypoglycemia test was 3.91 ng/ml). An associated corticotropic insufficiency was eliminated by a cortisol peak at 632.8 nmol/l (during the insulin hypoglycemia test) as well as a thyrotropic insufficiency (normal thyroid balance). The pituitary hypothalamic MRI was normal.

Discussion and conclusions

Systematic growth assessment is necessary in children with sickle cell disease as stunting (CR) is common and complex in this condition. Several factors are responsible for this: hematological state (anemia), endocrine function (GH deficiency, hypothyroidism), nutritional status (iron deficiency, vitamin B12 and folates) and bone state (necrosis of epiphyses bone) and hemochromatosis (if polytransfused child). Exploring CR in this population involves evaluating all of these parameters.