دورية أكاديمية
Clinical and genetic characterization of pituitary gigantism: an international collaborative study in 208 patients.
| العنوان: | Clinical and genetic characterization of pituitary gigantism: an international collaborative study in 208 patients. |
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| المؤلفون: | Rostomyan, Liliya, Daly, Adrian, PETROSSIANS, Patrick, Natchev, Emil, Lila, Anurag Ranjan, Lecoq, Anne-Lise, Lecumberri Santamaria, Beatriz, Trivellin, Giampaolo, Salvatori, Roberto, Moraitis, Adreas, Holdaway, Ian, Kranenburg-Van Klaveren, Dianne, Zatelli, Maria Chiara, Palacios, Nuria, Nozieres, Cecile, Zacharin, Margaret, Ebeling, Tapani Marcus Lauri, Ojaniemi, Marja, Rozhinskaya, Liudmila, Verrua, Elisa, Jaffrain Rea, Marie-Lise, Filipponi, Silvia, Guskova, Daria, Pronin, Vyacheslav, Bertherat, Jerome, Belaya, Zhanna, Ilovaiskaya, Irena, Sahnoun Fathallah, Mouna, Sievers, Caroline, Stalla, Gunter K., CASTERMANS, Emilie, CABERG, Jean-Hubert, Sorkina, Ekatarina, Auriemma, Renata, Mittal, Sachin, Kareva, Maria, Lysy, Philippe, Emy, Philippe, de Menis, Ernesto, Choong, Catherine, Mantovani, Giovanna, Bours, Vincent, de Herder, Wouter W., Brue, Thierry, Barlier, Anne, Neggers, Sebastian, Zacharieva, Sabina, Chanson, Philippe, Shah, Nalini S., Stratakis, Constantine A., Naves, Luciana Ansaneli, Beckers, Albert |
| المصدر: | Endocrine-Related Cancer (2015) |
| بيانات النشر: | Society for Endocrinology |
| سنة النشر: | 2015 |
| المجموعة: | University of Liège: ORBi (Open Repository and Bibliography) |
| مصطلحات موضوعية: | Human health sciences, Endocrinology, metabolism & nutrition, Sciences de la santé humaine, Endocrinologie, métabolisme & nutrition |
| الوصف: | peer reviewed ; Despite being a classical growth disorder, pituitary gigantism has not been studied previously in a standardized way. We performed a retrospective, multicenter, international study to characterize a large series of pituitary gigantism patients. We included 208 patients (163 males; 78.4%) with growth hormone excess and current/previous abnormal growth velocity for age or final height >2SD above country normal means. The median onset of rapid growth was 13.0 years and occurred significantly earlier in females than in males; pituitary adenomas were diagnosed earlier in females than males (15.8 vs. 21.5 years, respectively). Adenomas were >/=10 mm (i.e. macroadenomas) in 84%, of which extrasellar extension occurred in 77% and invasion in 54%. GH/IGF-1 control was achieved in 39% during long-term follow-up. Final height was greater in those with younger age of onset, with larger tumors and higher GH levels. Later disease control was associated with a greater difference from mid-parental height (r=0.23, P=0.02). AIP mutations occurred in 29%; microduplication at Xq26.3 -X-linked acro-gigantism (X-LAG)- occurred in two familial isolated pituitary adenoma (FIPA) kindreds and in ten sporadic patients. Tumor size was not different in X-LAG, AIP mutated and genetically-negative patient groups. AIP-mutated and X-LAG patients had significantly younger age at onset and diagnosis, but disease control was worse in genetically-negative cases. Pituitary gigantism patients are characterized by male predominance and large tumors that are difficult to control. Treatment delay increases final height and symptom burden. AIP mutations and X-LAG explain many cases, but no genetic etiology is seen in >50% of cases. |
| نوع الوثيقة: | article in journal/newspaper |
| اللغة: | English |
| تدمد: | 1351-0088 1479-6821 |
| العلاقة: | urn:issn:1351-0088; urn:issn:1479-6821; https://orbi.uliege.be/handle/2268/184329Test; info:hdl:2268/184329; scopus-id:2-s2.0-84942106611; info:pmid:26187128 |
| DOI: | 10.1530/ERC-15-0320 |
| الإتاحة: | https://doi.org/10.1530/ERC-15-0320Test https://orbi.uliege.be/handle/2268/184329Test |
| حقوق: | restricted access ; http://purl.org/coar/access_right/c_16ecTest ; info:eu-repo/semantics/restrictedAccess |
| رقم الانضمام: | edsbas.9D6FBDCB |
| قاعدة البيانات: | BASE |
Full Text Finder | تدمد: | 13510088 14796821 |
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| DOI: | 10.1530/ERC-15-0320 |