The etiology and clinical features of non-CAH gonadotropin-independent precocious puberty: A multicenter study

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dc.contributor.buuauthorEren, Erdal
dc.contributor.buuauthorSağlam, Halil
dc.contributor.departmentUludağ Üniversitesi/Tıp Fakültesi/Çocuk Endokrinolojisi Anabilim Dalı.tr_TR
dc.contributor.orcid0000-0002-1684-1053tr_TR
dc.contributor.orcid0000-0003-0710-5422tr_TR
dc.contributor.researcheridAAM-1734-2020tr_TR
dc.contributor.researcheridC-7392-2019tr_TR
dc.contributor.researcheridAAH-1155-2021tr_TR
dc.contributor.scopusid36113153400tr_TR
dc.contributor.scopusid35612700100tr_TR
dc.date.accessioned2022-11-30T12:31:52Z
dc.date.available2022-11-30T12:31:52Z
dc.date.issued2016-03-03
dc.descriptionÇalışmada 24 yazar bulunmaktadır. Bu yazarlardan sadece Bursa Uludağ Üniversitesi mensuplarının girişleri yapılmıştır.tr_TR
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.sponsorshipTürk Pediatrik Endokrinoloji ve Diyabet Derneği - 052014tr_TR
dc.identifier.citationAtay, Z. vd. (2016). "The etiology and clinical features of non-CAH gonadotropin-independent precocious puberty: A multicenter study". Journal of Clinical Endocrinology and Metabolism, 101(5), 1980-1988.en_US
dc.identifier.endpage1988tr_TR
dc.identifier.issn0021-972X
dc.identifier.issn1945-7197
dc.identifier.issue5tr_TR
dc.identifier.pubmed26964727tr_TR
dc.identifier.scopus2-s2.0-84969759473tr_TR
dc.identifier.startpage1980tr_TR
dc.identifier.urihttps://doi.org/10.1210/jc.2015-3500
dc.identifier.urihttps://academic.oup.com/jcem/article/101/5/1980/2804758?login=true
dc.identifier.urihttp://hdl.handle.net/11452/29639
dc.identifier.volume101tr_TR
dc.identifier.wos000378819700011tr_TR
dc.indexed.pubmedPubMeden_US
dc.indexed.scopusScopusen_US
dc.indexed.wosSCIEen_US
dc.language.isoenen_US
dc.publisherEndocrine Socen_US
dc.relation.collaborationYurt içitr_TR
dc.relation.collaborationSanayitr_TR
dc.relation.journalJournal of Clinical Endocrinology and Metabolismen_US
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergitr_TR
dc.rightsinfo:eu-repo/semantics/openAccessen_US
dc.subjectEndocrinology & metabolismen_US
dc.subjectMccune-albright-syndromeen_US
dc.subjectJuvenile hypothyroidismen_US
dc.subjectAdrenocortical tumorsen_US
dc.subjectExperienceen_US
dc.subjectMutationsen_US
dc.subject.emtreeChorionic gonadotropinen_US
dc.subject.emtreeTestosteroneen_US
dc.subject.emtreeAdrenal cortex tumoren_US
dc.subject.emtreeAlbright syndromeen_US
dc.subject.emtreeArticleen_US
dc.subject.emtreeBlood levelen_US
dc.subject.emtreeChilden_US
dc.subject.emtreeChoriocarcinomaen_US
dc.subject.emtreeClinical featureen_US
dc.subject.emtreeCohort analysisen_US
dc.subject.emtreeCongenital adrenal hyperplasiaen_US
dc.subject.emtreeControlled studyen_US
dc.subject.emtreeDemographyen_US
dc.subject.emtreeDisease activityen_US
dc.subject.emtreeDisease associationen_US
dc.subject.emtreeDysgerminomaen_US
dc.subject.emtreeFemaleen_US
dc.subject.emtreeFunctional ovarian cysten_US
dc.subject.emtreeGerm cell tumoren_US
dc.subject.emtreeGranulosa cell tumoren_US
dc.subject.emtreeHepatoblastomaen_US
dc.subject.emtreeHumanen_US
dc.subject.emtreeLiver choriocarcinomaen_US
dc.subject.emtreeMajor clinical studyen_US
dc.subject.emtreeMaleen_US
dc.subject.emtreeMolecular pathologyen_US
dc.subject.emtreeMulticenter studyen_US
dc.subject.emtreeNeoplasmen_US
dc.subject.emtreeNon congenital adrenal hyperplasia gonadotropin independent precocious pubertyen_US
dc.subject.emtreeOvary cysten_US
dc.subject.emtreeOvary teratomaen_US
dc.subject.emtreePrecocious pubertyen_US
dc.subject.emtreePreschool childen_US
dc.subject.emtreePrevalenceen_US
dc.subject.emtreePriority journalen_US
dc.subject.emtreeSchool childen_US
dc.subject.emtreeSteroid cell tumoren_US
dc.subject.emtreeTesticular enlargementen_US
dc.subject.emtreeTurkey (republic)en_US
dc.subject.emtreeCafe au lait spoten_US
dc.subject.emtreeComplicationen_US
dc.subject.emtreeFibrous dysplasiaen_US
dc.subject.emtreePuberty, precociousen_US
dc.subject.emtreeSymptom assessmenten_US
dc.subject.emtreeTurkeyen_US
dc.subject.meshCafe-au-lait spotsen_US
dc.subject.meshChilden_US
dc.subject.meshChild, preschoolen_US
dc.subject.meshFemaleen_US
dc.subject.meshFibrous dysplasia of boneen_US
dc.subject.meshFibrous dysplasia, polyostoticen_US
dc.subject.meshHumansen_US
dc.subject.meshMaleen_US
dc.subject.meshOvarian cystsen_US
dc.subject.meshPuberty, precociousen_US
dc.subject.meshSymptom assessmenten_US
dc.subject.meshTurkeyen_US
dc.subject.scopusGonadorelin Derivative; Synthetic Hormones; Gnrh Agonisten_US
dc.subject.wosEndocrinology & metabolismen_US
dc.titleThe etiology and clinical features of non-CAH gonadotropin-independent precocious puberty: A multicenter studyen_US
dc.typeArticle
dc.wos.quartileQ1en_US

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