Pituitary morphovolumetric changes in Alstrom syndrome.

ABSTRACT:

Purpose

Alström syndrome (AS) is a rare monogenic ciliopathy characterized by cone-code dystrophy, leading to early blindness, and obesity. Early endocrinological dysfunctions, especially growth hormone deficiency and hypogonadism, are detected in about half of AS patients. This MRI study investigates the presence of pituitary gland abnormalities in a large cohort of AS patients.

Methods

Pituitary morphological changes (gland flattening with partial or total empty sella) were evaluated on midsagittal high-resolution T1-weighted images of 32 AS patients (mean-age 23.2±9.4 years; range: 6-45, 15 females) and 21 unrelated healthy subjects (mean age 23.2±11.2 years; range: 6-43; 10 females).

Results

Among AS patients, 11/32 (34%) had total empty sella and 6/32 (19%) parti

SUBMITTER: Citton V 

PROVIDER: S-EPMC5510590 | biostudies-literature | 2016 Jun

REPOSITORIES: biostudies-literature