Endocrine Abstracts

Introduction: Abnormalities of midline (AML) can be isolated or to be associated to secretory pituitary abnormalities that reflect a developmental defect of the hypothalamic pituitary (HP) region.

Aim: Find AML in GH deficiency (GHD) and see their relationship with the appearance of the HP region and the severity of hypopituitarism (I P).

Population and methodology: 160 children (141 ♂, ♀ 19) (IGH) underwent a clinical examination, a hypophysiogramme (test glucagon/propranolol/GH test insulin/GH/cortisol, IGF1, FT₄, TSH, ACTH, Prolactin, urinary density±test fluid restriction (gonadotropin axis has not been evaluated because prepubertal bone age) and magnetic resonance imaging HH (MRI). The exploration was complemented by a complementary assessment in the presence of visceral malformations.

Results: A ML are found in 60% of cases. Facial malformations are predominant (85%). They are represented mainly by ocular signs (congenital strabismus: 72%) cerebral MRI revealed intracranial abnormalities of midline in 25% of children.

Other abnormalities not within the ALM are also associated with IGH in 16% of cases. The AM L are more frequent when there are abnormalities HH region (P=0.04).

The number of combined pituitary deficits is more important when the AM L are present (P<10⁻³).

The AML presence is independent of the number of abnormal HH region (P=0.76).

Discussion and conclusion: ALM are clinical markers of hypopituitarism. They have to consider a systematic neuroendocrine and visceral exploration. Their presence has a high diagnostic and etiological value because it directs toward to the congenital hypothalamic disorders and permanent déficit. Regular monitoring are necessary.