Introduction: Growth failure is the most frequent endocrine abnormality observed in patients with sickle-cell disease SCD. Decreased synthesis of IGF1 might be secondary to a disturbed GHIGF1 axis and defective GH secretion has been reported in some patients. Infarction, atrophy, and hemorrhage may occur in the pituitary gland in SCD during or following the vaso-occlusive crisis.
Objective: To define the possible abnormalities of pituitary gland in SCD we measured the circulating concentrations of IGF1 and studied the magnetic resonance imaging (MRI) of the pituitary gland in seven adolescents and young adults with SCD with short stature (HtSDS <−2) and history of recurrent painful crisis.
Methods: Seven patients with SCD (age: 24.2±4.5 years) and short stature (HtSDS=2.5±0.4) and history of severe and recurrent vaso-occlusive crisis (at least three in the past 3 years) were studied. All were transfusion-dependent, with full pubertal development (Tanners stage 5) (euogonadal). They were regularly transfused since early childhood and underwent chelation therapy using desferrioxamine which was replaced by deferasirox for the last 45 years.
Results: In the seven patients with SCD circulating IGF1 were decreased (IGF1 SDS=−2.1±0.5) compared to adults standards. Pituitary MRI showed abnormalities in 4/7 of these patients in the form of heterogeneous appearance of the anterior pituitary, presence of single or multiple hypointense foci due to hemosiderin deposition in the pituitary (4/7) and significantly decreased (2/7) or increased volume (1/7). These lesions can be explained by hemosiderosis of the gland and/or ischemia during the vaso-occlusive crisis.
Conclusions: Pituitary MRI showed significant abnormalities of the anterior pituitary gland in SCD patients with short stature and significant history of vaso-occlusive crisis. This study demonstrated the value of MRI of the pituitary to support investigating of the GHIGF1 axis in these patients.
03 - 07 May 2014
European Society of Endocrinology