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Titolo:
Evidence for decreased growth hormone in patients with hypothalamic hamartoma due to Pallister-Hall syndrome
Autore:
Feuillan, P; Peters, KF; Cutler, GB; Biesecker, LG;
Indirizzi:
NICHHD, Dev Endocrinol Branch, NIH, Bethesda, MD 20892 USA NICHHD Bethesda MD USA 20892 docrinol Branch, NIH, Bethesda, MD 20892 USA NHGRI, NIH, Bethesda, MD 20892 USA NHGRI Bethesda MD USA 20892NHGRI, NIH, Bethesda, MD 20892 USA
Titolo Testata:
JOURNAL OF PEDIATRIC ENDOCRINOLOGY & METABOLISM
fascicolo: 2, volume: 14, anno: 2001,
pagine: 141 - 149
SICI:
0334-018X(200102)14:2<141:EFDGHI>2.0.ZU;2-H
Fonte:
ISI
Lingua:
ENG
Soggetto:
PRECOCIOUS PUBERTY; NORMAL GIRLS; SECRETION; HYPOPITUITARISM; BOYS;
Keywords:
growth hormone; somatotropin; hypothalamic hamartoma; Pallister-Hall syndrome;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Life Sciences
Citazioni:
26
Recensione:
Indirizzi per estratti:
Indirizzo: Feuillan, P NICHHD, Dev Endocrinol Branch, NIH, Bldg 10,Rm 10N262, Bethesda, MD 20892 USA NICHHD Bldg 10,Rm 10N262 Bethesda MD USA 20892 a, MD 20892 USA
Citazione:
P. Feuillan et al., "Evidence for decreased growth hormone in patients with hypothalamic hamartoma due to Pallister-Hall syndrome", J PED END M, 14(2), 2001, pp. 141-149

Abstract

Pallister-Hall syndrome (PHS) is characterized by hypothalamic hamartoma, bifid epiglottis, and central or postaxial polydactyly, Familial transmission is autosomal dominant; isolated cases also occur. To screen for hypothalamic-pituitary dysfunction in PHS, we studied a 12 year-old boy (patient #1), and 14 additional patients (patients #2-14: 7M, 7F; ages 4-72 yr), We performed serial sampling of GH, LH/FSH, TSH, and cortisol from 20.00-08 00 h, At 08.00 h, we measured IGF-I, peak responses of LH and FSH after GnRH, and cortisol after ACTH, We found that 6/7 children, including patient #1, and 6/8 adults had low or absent spontaneous GH secretion and/or low levels of IGF-I, Patient #1 also had accelerated pubertal development, but no other patient had abnormalities of the pituitary-gonadal axis, and none of the 14 patients had an abnormal thyroid or adrenal axis. We conclude that decreased pituitary GH secretion is common in PHS, and may exist in the absence of other forms of endocrine dysfunction.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 15/07/20 alle ore 02:56:32