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Management of Central Precocious Puberty in Children with Hypothalamic Hamartoma

 Junghwan Suh  ;  Youngha Choi  ;  Jun Suk Oh  ;  Kyungchul Song  ;  Han Saem Choi  ;  Ahreum Kwon  ;  Hyun Wook Chae  ;  Ho-Seong Kim 
 CHILDREN-BASEL, Vol.18(8) : 711, 2021-08 
Journal Title
Issue Date
central precocious puberty ; gelastic seizure ; hypothalamic hamartoma
Hypothalamic hamartoma (HH) is a rare, congenital, and benign lesion of the tuber cinereum, typically presenting with central precocious puberty (CPP), gelastic seizure, and developmental delay. This study aimed to investigate CPP in HH patients and compare clinical features between before and after gonadotropin-releasing hormone (GnRH) agonist treatment. A total of 30 HH patients under 18 years of age who visited Severance Children's Hospital between January 2005 and May 2020 were retrospectively reviewed. Fourteen patients were male (46.7%) and sixteen (53.3%) were female, with a mean age at diagnosis was4.2 ± 2.9 years. During follow-up, 24 patients (80.0%) were diagnosed with CPP, 15 patients (50.0%) had gelastic seizure, and 13 patients (43.3%) had developmental delay. The gelastic seizure was significantly associated with sessile type HH rather than pedunculated type HH (85.7% vs. 18.8%, p = 0.001). After GnRH agonist treatment, discrepancies between bone age and chronological age decreased (3.3 ± 1.3 years to 2.0 ± 1.7 years, p = 0.002). Additionally, height standard deviation score for bone age was increased, and predicted adult height increased significantly in females, while males showed an increasing trend. Clinical symptoms of HH were closely associated with the location of HH, and GnRH agonist treatment was safe and effective in the management of CPP caused by HH.
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1. College of Medicine (의과대학) > Dept. of Pediatrics (소아과학교실) > 1. Journal Papers
Yonsei Authors
Kwon, Ah Reum(권아름) ORCID logo https://orcid.org/0000-0002-9692-2135
Kim, Ho Seong(김호성) ORCID logo https://orcid.org/0000-0003-1135-099X
Suh, Junghwan(서정환) ORCID logo https://orcid.org/0000-0002-2092-2585
Song, Kyungchul(송경철) ORCID logo https://orcid.org/0000-0002-8497-5934
Chae, Hyun Wook(채현욱) ORCID logo https://orcid.org/0000-0001-5016-8539
Choi, Han Saem(최한샘) ORCID logo https://orcid.org/0000-0003-4140-0345
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