Impaired hypothalamic endocrine function in neuroacanthocytosis.
Two sisters aged 28 and 26, suffering from neuroacanthocytosis (NA), had hypothyroidism and showed a flat growth hormone (GH) response to the insulin tolerance test. GH-releasing hormone (GHRH) administration resulted in a normal GH response in the older sister and a partial GH response in the younger sister. A pathological process--attributable to NA--can be suggested, leading to impaired hypothalamic function, but sparing the pituitary.
Kontoleon
P E
PE
Second Endocrine Department, Alexandra General Hospital, 8 Ofiountos Street, Athens, Greece. pangr@hol.gr
Ilias
I
I
Matsouka
A
A
Gatzonis
S
S
Spiropoulou
C
C
Papapetrou
P D
PD
D000328:Adult; D004700:Endocrine System Diseases / Q000209:etiology / Q000503:physiopathology*; D005260:Female; D013006:Growth Hormone / Q000172:deficiency / Q000378:metabolism; D013007:Growth Hormone-Releasing Hormone / Q000494:pharmacology / Q000627:therapeutic use; D006801:Humans; D007027:Hypothalamic Diseases / Q000209:etiology / Q000503:physiopathology*; D007030:Hypothalamo-Hypophyseal System / Q000503:physiopathology*; D007037:Hypothyroidism / Q000209:etiology / Q000503:physiopathology; D054546:Neuroacanthocytosis / Q000150:complications / Q000503:physiopathology*; D009490:Neurosecretory Systems / Q000503:physiopathology*; D016896:Treatment Outcome
D013006:Growth Hormone; D013007:Growth Hormone-Releasing Hormone