Síndrome de Prader-Willi: tratamiento con hormona de crecimiento en dos casos / Use of growth hormone in Prader-Willi syndrome: report of 2 cases
Rev. méd. Chile
; 129(10): 1186-1190, oct. 2001. tab
Article
in Es
| LILACS
| ID: lil-301912
Responsible library:
CL1.1
ABSTRACT
Prader-Willi syndrome (PWS) is a neurogenetic disorder caused by the absence or abnormal inactivation of a critical region of the paternal chromosome 15. Clinical manifestations include marked hypotonia at birth, progressive obesity that starts during the second year of life, stunting, hypogonadism and some dysmorphic features. Some of the symptoms and signs can be explained by growth hormone (GH) deficiency. We report two females aged 12 and 13 years old with PWS. Both were very short and obese, showed blunted GH responses to provocative stimuli and had low plasma levels of Insulin Growth Factor-1 (IGF-1). They have been on GH treatment for more than two years, demonstrating a marked growth acceleration, reduction in their fat mass, improvement of their muscular strength and an increase in their IGF-1 levels
Full text:
1
Index:
LILACS
Main subject:
Prader-Willi Syndrome
/
Human Growth Hormone
Limits:
Adolescent
/
Female
/
Humans
Language:
Es
Journal:
Rev. méd. Chile
Journal subject:
MEDICINA
Year:
2001
Type:
Article