Growth hormone secretion in Prader-Willi syndrome.
Prader-Willi Syndrome (PWS) is a multisystem defect characterized by obesity, hypogenitalism and short stature for genetic background. Low GH serum levels have been found in patients with PWS and were related to a hypothalamic-pituitary dysfunction. We studied spontaneous nocturnal GH secretion and GH-response to provocative tests in five patients affected by PWS. We observed in three of them (Group A) abnormally low GH and IGF-1 serum levels. In the other two patients (Group B) GH secretion and IGF-1 serum levels were normal. In all patients no thyroid dysfunction was observed. These data might suggest the presence of two different subgroups of patients affected by PWS, from an endocrinological point of view. An abnormally low GH secretion would be evident only in a subgroup of patients, which appears to be normal in the remaining patients. This casistic is small in number, but if our data will be confirmed by more extensive studies it may be possible to identify a specific population of PWS patients who could benefit from recombinant GH-therapy.
Istituto di Clinica Pediatrica, University of Siena, Italy.
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Berardi
D000293:Adolescent; D002648:Child; D005260:Female; D019382:Human Growth Hormone / Q000097:blood* / Q000172:deficiency; D006801:Humans; D008297:Male; D011218:Prader-Willi Syndrome / Q000097:blood*; D011863:Radioimmunoassay
D019382:Human Growth Hormone