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Hyperghrelinemia is a common feature of Prader-Willi syndrome and pituitary stalk interruption: a pathophysiological hypothesis.
Horm Res 2004; 62(1):49-54HR

Abstract

BACKGROUND

Elevated plasma ghrelin levels have recently been reported in adults and children with Prader-Willi syndrome (PWS). The aim of the study is to investigate the relationship between obesity, growth hormone (GH) deficiency (GHD) and ghrelinemia in PWS and to examine whether hyperghrelinemia is specific to PWS.

METHODS

We measured fasting ghrelinemia in children with PWS, idiopathic GHD (iGHD), obese children, controls and in 6 children presenting another congenital syndrome associated with GHD: pituitary stalk interruption (PSI).

RESULTS

Children with PWS exhibited significantly higher ghrelin levels (995 pg/ml (801/1,099, median 1st/3rd quartile)) than iGHD (517 pg/ml (392/775)), obese (396 pg/ml (145/610)) and control (605 ng/ml (413/753)) children. Similar to PWS hyperghrelinemia was found in PSI children (1,029 pg/ml (705/1,151)), and was not modified by GH treatment.

CONCLUSION

We conclude that hyperghrelinemia in PWS and PSI is not related to GH secretion. We hypothesize that a major site of ghrelin action is at the hypothalamic level and that a 'ghrelin resistance' syndrome may be present in these patients, primarily due to a hypothalamic defect. Combined alterations such as impaired serotonin receptor regulation associated with abnormal ghrelin responsiveness are probably responsible for obesity in PWS.

Authors+Show Affiliations

Division of Endocrinology, Bone Diseases, Genetics and Gynecology, Children's Hospital, INSERM U563 (CPTP), IFR 30, Hôpital Purpan, Toulouse, France. crc@chu-toulouse.frNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article

Language

eng

PubMed ID

15192277

Citation

Tauber, Maithé, et al. "Hyperghrelinemia Is a Common Feature of Prader-Willi Syndrome and Pituitary Stalk Interruption: a Pathophysiological Hypothesis." Hormone Research, vol. 62, no. 1, 2004, pp. 49-54.
Tauber M, Conte Auriol F, Moulin P, et al. Hyperghrelinemia is a common feature of Prader-Willi syndrome and pituitary stalk interruption: a pathophysiological hypothesis. Horm Res. 2004;62(1):49-54.
Tauber, M., Conte Auriol, F., Moulin, P., Molinas, C., Delagnes, V., & Salles, J. P. (2004). Hyperghrelinemia is a common feature of Prader-Willi syndrome and pituitary stalk interruption: a pathophysiological hypothesis. Hormone Research, 62(1), pp. 49-54.
Tauber M, et al. Hyperghrelinemia Is a Common Feature of Prader-Willi Syndrome and Pituitary Stalk Interruption: a Pathophysiological Hypothesis. Horm Res. 2004;62(1):49-54. PubMed PMID: 15192277.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Hyperghrelinemia is a common feature of Prader-Willi syndrome and pituitary stalk interruption: a pathophysiological hypothesis. AU - Tauber,Maithé, AU - Conte Auriol,Françoise, AU - Moulin,Pierre, AU - Molinas,Catherine, AU - Delagnes,Véronique, AU - Salles,Jean Pierre, Y1 - 2004/06/08/ PY - 2003/10/10/received PY - 2004/03/23/accepted PY - 2004/6/12/pubmed PY - 2004/12/22/medline PY - 2004/6/12/entrez SP - 49 EP - 54 JF - Hormone research JO - Horm. Res. VL - 62 IS - 1 N2 - BACKGROUND: Elevated plasma ghrelin levels have recently been reported in adults and children with Prader-Willi syndrome (PWS). The aim of the study is to investigate the relationship between obesity, growth hormone (GH) deficiency (GHD) and ghrelinemia in PWS and to examine whether hyperghrelinemia is specific to PWS. METHODS: We measured fasting ghrelinemia in children with PWS, idiopathic GHD (iGHD), obese children, controls and in 6 children presenting another congenital syndrome associated with GHD: pituitary stalk interruption (PSI). RESULTS: Children with PWS exhibited significantly higher ghrelin levels (995 pg/ml (801/1,099, median 1st/3rd quartile)) than iGHD (517 pg/ml (392/775)), obese (396 pg/ml (145/610)) and control (605 ng/ml (413/753)) children. Similar to PWS hyperghrelinemia was found in PSI children (1,029 pg/ml (705/1,151)), and was not modified by GH treatment. CONCLUSION: We conclude that hyperghrelinemia in PWS and PSI is not related to GH secretion. We hypothesize that a major site of ghrelin action is at the hypothalamic level and that a 'ghrelin resistance' syndrome may be present in these patients, primarily due to a hypothalamic defect. Combined alterations such as impaired serotonin receptor regulation associated with abnormal ghrelin responsiveness are probably responsible for obesity in PWS. SN - 0301-0163 UR - https://www.unboundmedicine.com/medline/citation/15192277/Hyperghrelinemia_is_a_common_feature_of_Prader_Willi_syndrome_and_pituitary_stalk_interruption:_a_pathophysiological_hypothesis_ L2 - https://www.karger.com?DOI=10.1159/000078862 DB - PRIME DP - Unbound Medicine ER -