The Etiology and Clinical Features of Non-CAH Gonadotropin-Independent Precocious Puberty: A Multicenter Study
| Autor: | Erkan Sari, Tulay Guran, Aysşehan Akinci, Cengiz Kara, Selim Kurtoglu, Samim Özen, Gülay Karagüzel, Zehra Yavas Abali, Erdal Eren, Zeynep Atay, Filiz Tutunculer, Meltem Tayfun, Esra Döğer, Abdullah Bereket, Aysun Bideci, Semra Çetinkaya, Zehra Aycan, Gulcan Seymen Karabulut, Halil Saglam, Zeynep Şıklar, Serap Turan, Ediz Yeşilkaya, Leyla Akin, Şenay Savaş Erdeve |
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| Přispěvatelé: | Atay, Zeynep, Yesilkaya, Ediz, Erdeve, Senay Savas, Turan, Serap, Akin, Leyla, Eren, Erdal, Doger, Esra, Aycan, Zehra, Abali, Zehra Yavas, Akinci, Aysehan, Siklar, Zeynep, Ozen, Samim, Kara, Cengiz, Tayfun, Meltem, Sari, Erkan, Tutunculer, Filiz, Karabulut, Gulcan Seymen, Karaguzel, Gulay, Cetinkaya, Semra, Saglam, Halil, Bideci, Aysun, Kurtoglu, Selim, Guran, Tulay, Bereket, Abdullah, Uludağ Üniversitesi/Tıp Fakültesi/Çocuk Endokrinolojisi Anabilim Dalı., Sağlam, Halil, AAM-1734-2020, C-7392-2019, AAH-1155-2021, Ege Üniversitesi, OMÜ |
| Jazyk: | angličtina |
| Rok vydání: | 2016 |
| Předmět: |
Hepatoblastoma
Male Pediatrics Turkey Endocrinology Diabetes and Metabolism Clinical Biochemistry Granulosa cell tumor Puberty Precocious ADRENOCORTICAL TUMORS Biochemistry Fibrous dysplasia Turkey (republic) 0302 clinical medicine Endocrinology Precocious puberty Non congenital adrenal hyperplasia gonadotropin independent precocious puberty Germ cell tumor Prevalence Medicine Testosterone 030212 general & internal medicine Choriocarcinoma Disease activity Polyostotic fibrous dysplasia Child Endocrinology & metabolism Priority journal MCCUNE-ALBRIGHT-SYNDROME education.field_of_study Steroid cell tumor Cafe-au-Lait Spots Ovary cyst Multicenter study Chorionic gonadotropin Ovary teratoma ComputingMilieux_MANAGEMENTOFCOMPUTINGANDINFORMATIONSYSTEMS Ovarian Cysts Cafe au lait spot Child Preschool Cohort Female InformationSystems_MISCELLANEOUS Cohort analysis Symptom Assessment Human medicine.medical_specialty Population 030209 endocrinology & metabolism Gonadorelin Derivative Synthetic Hormones Gnrh Agonist Dysgerminoma Major clinical study Fibrous Dysplasia Polyostotic Gonadotropin-Independent Precocious Puberty Article 03 medical and health sciences Disease association Internal medicine Liver choriocarcinoma Humans Congenital adrenal hyperplasia Testicular enlargement education Adrenal cortex tumor Demography Molecular pathology business.industry MUTATIONS ComputerSystemsOrganization_COMPUTER-COMMUNICATIONNETWORKS Biochemistry (medical) Fibrous Dysplasia of Bone medicine.disease ComputingMethodologies_PATTERNRECOGNITION Clinical feature Preschool child JUVENILE HYPOTHYROIDISM Albright syndrome Etiology Blood level Functional Ovarian Cyst Neoplasm EXPERIENCE School child business Controlled study Complication Functional ovarian cyst |
| Popis: | WOS: 000378819700011 PubMed ID: 26964727 Aim: 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. Turkish Pediatric Endocrinology and Diabetes Society [052014] This work was supported by the Turkish Pediatric Endocrinology and Diabetes Society (No: 052014). |
| Databáze: | OpenAIRE |
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