The Etiology and Clinical Features of Non-CAH Gonadotropin-Independent Precocious Puberty: A Multicenter Study

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dc.authoridAycan, Zehra/0000-0003-4584-2976
dc.authoridÇetinkaya, Semra Çağlar/0000-0003-3974-2872
dc.authoridSağlam, Halil/0000-0003-0710-5422
dc.authoridKara, Cengiz/0000-0002-8989-560X
dc.authoridTuran, Serap/0000-0002-5172-5402
dc.authoridsiklar, zeynep/0000-0003-0921-2694
dc.authoridbereket, abdullah/0000-0002-6584-9043
dc.authorwosidAKIN, Leyla/JNS-3643-2023
dc.authorwosidAycan, Zehra/AAQ-9537-2020
dc.authorwosidÇetinkaya, Semra Çağlar/AAA-1747-2022
dc.authorwosidSağlam, Halil/C-7392-2019
dc.authorwosidKara, Cengiz/AAI-3335-2021
dc.authorwosidTuran, Serap/U-7195-2018
dc.authorwosidsiklar, zeynep/AAN-3819-2020
dc.contributor.authorAtay, Zeynep
dc.contributor.authorYesilkaya, Ediz
dc.contributor.authorErdeve, Senay Savas
dc.contributor.authorTuran, Serap
dc.contributor.authorAkin, Leyla
dc.contributor.authorEren, Erdal
dc.contributor.authorDoger, Esra
dc.date.accessioned2024-08-04T20:41:46Z
dc.date.available2024-08-04T20:41:46Z
dc.date.issued2016
dc.departmentİnönü Üniversitesien_US
dc.description.abstractAim: The causes of gonadotropin-independent precocious puberty are diverse, and often have overlapping clinical and biochemical features. With the exception of congenital adrenal hyperplasia (CAH), disorders that cause gonadotropin-independent precocious puberty (GIPP) are uncommon. The literature is devoid of any large-scale studies on the etiologic distribution of GIPP. The aim of this study was to determine the frequency of each etiology in a cohort of patients with GIPP (excluding those with CAH), and to evaluate the clinical and laboratory features of these patients. Materials and Methods: This multicenter, nationwide web-based study collected data on patients who presented with non-CAH GIPP in Turkey. Results: Data were collected for 129 patients (102 girls and 27 boys) from 29 centers. Based on the data collected, the estimated prevalence of non-CAH GIPP in the studied population was 14 in 1 000 000 children. Functional ovarian cyst was the most common etiology, accounting for 37% of all cases, followed by McCune-Albright syndrome (MAS) (26%). Among the patients with MAS, 11.7% had fibrous dysplasia, 32.3% had caf-au-lait spots, and 52.9% had both. Human chorionic gonadotrophin-secreting tumors included choriocarcinoma of the liver, hepatoblastoma, and germ cell tumors of the sellar-suprasellar region and mediastinum. Patients with adrenocortical tumors presented at an earlier age than those with other etiologies. Ovarian tumors included mature cystic teratoma, dysgerminoma, juvenile granulosa tumor, and steroid cell tumor. Despite overlapping features, it was possible to identify some unique clinical and laboratory features associated with each etiology. Conclusion: This largest cohort of patients with non-CAH GIPP to date yielded an estimation of the frequency of non-CAH GIPP in the general pediatric population and showed that girls were affected at a rate 4-fold greater than that of boys owing to functional ovarian cysts and MAS, which were the two most common etiologies. The data collected also provided some unique characteristics associated with each etiology.en_US
dc.description.sponsorshipTurkish Pediatric Endocrinology and Diabetes Society [052014]en_US
dc.description.sponsorshipThis work was supported by the Turkish Pediatric Endocrinology and Diabetes Society (No: 052014).en_US
dc.identifier.doi10.1210/jc.2015-3500
dc.identifier.endpage1988en_US
dc.identifier.issn0021-972X
dc.identifier.issn1945-7197
dc.identifier.issue5en_US
dc.identifier.pmid26964727en_US
dc.identifier.scopus2-s2.0-84969759473en_US
dc.identifier.scopusqualityQ1en_US
dc.identifier.startpage1980en_US
dc.identifier.urihttps://doi.org/10.1210/jc.2015-3500
dc.identifier.urihttps://hdl.handle.net/11616/97334
dc.identifier.volume101en_US
dc.identifier.wosWOS:000378819700011en_US
dc.identifier.wosqualityQ1en_US
dc.indekslendigikaynakWeb of Scienceen_US
dc.indekslendigikaynakScopusen_US
dc.indekslendigikaynakPubMeden_US
dc.language.isoenen_US
dc.publisherEndocrine Socen_US
dc.relation.ispartofJournal of Clinical Endocrinology & Metabolismen_US
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanıen_US
dc.rightsinfo:eu-repo/semantics/openAccessen_US
dc.subjectMccune-Albright-Syndromeen_US
dc.subjectJuvenile Hypothyroidismen_US
dc.subjectAdrenocortical Tumorsen_US
dc.subjectExperienceen_US
dc.subjectMutationsen_US
dc.titleThe Etiology and Clinical Features of Non-CAH Gonadotropin-Independent Precocious Puberty: A Multicenter Studyen_US
dc.typeArticleen_US

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