期刊
HORMONE RESEARCH IN PAEDIATRICS
卷 73, 期 5, 页码 312-319出版社
KARGER
DOI: 10.1159/000308162
关键词
Kisspeptin; KISS1R; GPR54; Reproduction; LHRH; Hypothalamic hamartoma
资金
- US Eunice Kennedy Shriver National Institute of Child Health and Human Development [U54 HD 028138, T32 HD 07369, F32 HD 056759]
- Barrow Neurological Foundation
- Novo Nordisk Lawson Wilkins Pediatric Endocrine Society
- EUNICE KENNEDY SHRIVER NATIONAL INSTITUTE OF CHILD HEALTH & HUMAN DEVELOPMENT [R01HD043341, U54HD028138] Funding Source: NIH RePORTER
- EUNICE KENNEDY SHRIVER NATIONAL INSTITUTE OF CHILD HEALTH &HUMAN DEVELOPMENT [F32HD056759] Funding Source: NIH RePORTER
Background/Aims: Hypothalamic hamartomas are the most common identifiable cause of central precocious puberty (CPP). Hamartoma characteristics proposed to be associated with CPP include specific anatomic features and expression of molecules such as gonadotropin-releasing hormone (GnRH), transforming growth factor alpha (TGF alpha), and GRM1A, which encodes the type 1 metabotropic glutamate receptor alpha isoform. We sought to determine whether hamartomas that cause CPP could be distinguished by anatomic features, expression of these molecules, or expression of KISS1, whose products signal through the receptor GPR54 to stimulate GnRH release. Methods: Clinical records and radiologic images were reviewed for 18 patients who underwent hamartoma resection for intractable seizures; 7 had precocious puberty. Resected tissue was examined for expression of GnRH, GnRH receptor (GnRHR), TGF alpha, KISS1, GPR54, and GRM1A Results: Hypothalamic hamartomas associated with CPP were more likely to contact the infundibulum or tuber cinereum and were larger than hamartomas not associated with CPP. GnRH, TGF alpha, and GnRHR were expressed by all hamartomas studied. Expression of KISS1, GPR54, and GRM1A did not differ significantly between hamartomas associated and not associated with CPP. Conclusion: Anatomic features rather than expression patterns of candidate molecules distinguish hypothalamic hamartomas that are associated with CPP from those that are not. Copyright (C) 2010 S. Karger AG, Basel
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