hrp0089p2-p253 | Growth & Syndromes P2 | ESPE2018

Influence of Puberty on Adult Height of SGA Children Treated with GH

Lopez-Siguero Juan P. , Munoz-Martinez Pablo , Borras-Perez Ma Victoria , Alvarez-Casano Maria , Sanchez-Moreno Sonia , Martinez-Aedo Ma Jose

Introduction: Published studies on pubertal growth of SGA patients on GH therapy are scarce. An earlier and shorter duration of puberty has been described. Treatment optimization may be necessary and also know their influence on adult height.Objetives: Analyse the evolution of height durig puberty in SGA patients treated with GH. Asses the age of onset of puberty and its relationship to adult and target height.Methods: Retrospectiv...

hrp0095p1-516 | Growth and Syndromes | ESPE2022

Ovarian hilus cell hyperplasia: a rare cause of progressive virilization in a girl with Turner syndrome in the absence of Y chromosomal material

van der ZwanY.G. , Spath M.A. , van Setten P.A. , van der Velden J.

Introduction: Turner syndrome is a relatively common chromosomal abnormality affecting 1:2000 girls. Virilization can occur in girls with Turner syndrome which is in most cases associated with the presence of an Y chromosome. Mosaicism is found in 25% of the karyotypes, in 6% of those the Y chromosome is present. This could lead to development of androgen producing gonadoblastoma/dysgerminoma. Active screening to search for Y chromosomal material is included i...

hrp0095p1-564 | Pituitary, Neuroendocrinology and Puberty | ESPE2022

Adenohypophyseal Hyperfuction in Childeren and Adolescents with McCune-Albright Syndrome

Xie Liu-lu , Li Yan-hong , Ma Hua-mei , Du Min-lian

Background: McCune-Albright Syndrome(MAS)is a rare disease caused by somatic gain-of-function mutations of the GNAS gene, presenting with fibrous dysplasia(FD), cafe-au-lait spots and gonadotropin-independent precocious puberty(PP). Adenohypophyseal hyperfunction syndromes is reported uncommon but potentially severe complication of MAS.Method: A prospective study was performed to evaluate the prevalence of the pit...

hrp0095p1-178 | Sex Differentiation, Gonads and Gynaecology, and Sex Endocrinology | ESPE2022

Human chorionic gonadotropin (hCG)–secreting hepatoblastoma in an male infant with peripheral precocious puberty and 11-year follow-up

MA Hua-mei , LI Yan-hong , Chen Qiu-li , DU Min-lian

Background: Hepatoblastoma is the most common malignant pediatric hepatic tumor virtually confined to infants and young children. Accompanied by raised levels of alpha fetoprotein (AFP),rarely, the beta human chorionic gonadotropin (β hCG) levels may also be elevated, which can cause male peripheral precocious puberty (PPP).Objective: To report a case of HB presented with PPP with elevated serum AFP, β h...

hrp0095p1-585 | Sex Differentiation, Gonads and Gynaecology, and Sex Endocrinology | ESPE2022

Long Term Follow-up of A Boy Who Had Suprasella Tumor Recurrence 4 Years After the First Diagnosis of Extracranial GCTs

Shi Xian-jun , Li Yanh-hong , Ma Hua-mei , Du Min-lian

Background: In children with extracranial germ cell tumors (GCTs), brain metastases are rare and are classified as “poor prognosis”. The longest interval to brain metastases was 36 months after diagnosis in children, however, it is still less understood.Methods: We report a boy who had suprasella tumor recurrence 4 years after the first diagnosis of extracranial GCTs (thymus).Re...

hrp0095p1-394 | Thyroid | ESPE2022

Analysis on the influencing factors of neonatal serum FT3, FT4 and TSH

Zhang Cong , Zheng Rongxiu , Ma Shifeng , Wu Chushan , Liu Xiaoxiao , Cheng Siqi , Zhao Yue , Jiang Haiyan

Background: With the increasing survival rate of extremely premature infants, neonatal thyroid dysfunction is more frequently observed clinically. Despite the guidelines, the timing of screening for neonatal thyroid function remains controversial, especially for premature infants. At present, there is no unified standard for serum FT3, FT4 and TSH levels of newborns of different gestational ages and postnatal days.Objective:</str...

hrp0095p2-10 | Adrenals and HPA Axis | ESPE2022

Long-term Outcomes for Ninety-four Boys with Central Precocious Puberty or Early and Fast Puberty in Chinese Boys

Chen Zhixin , Chen Qiuli , Li Yanhong , Ma Huamei , Zhang Jun , Guo Song

Objective: To assess the efficacy and influential factors of GnRHa treatment for Chinese CPP/EFP boys.Methods: From April, 1994 to June, 2020, 94 boys diagnosed with CPP or EFP who had reached the final adult height were retrospectively included. Among these patients, 41 patients received no treatment, 43 patients received GnRHa treatment alone and 10 patients received GnRHa + GH treatment. The final adult height and the...

hrp0095p2-23 | Adrenals and HPA Axis | ESPE2022

Primary pigmented nodular adrenocortical disease in a young boy associated with a rare somatic mutation of the PRKACA: case report and literature review

Xu Yu-ying , Li Yan-hong , Chen Qiu-li , Ma Hua-mei , Zhang Jun , Guo Song

Introduction: Cushing's syndrome(CS) is rare in the pediatric population, and the cause vary with age. Primary pigmented nodular adrenocortical disease (PPNAD) is one of the causes of CS, though it is a rare disorder. It has been proposed that in children with CS, PPNAD should be suspected. Here we report a young boy with CS due to PPNAD associated with a somatic mutation of the PRKACA.Case Presentation: The child w...

hrp0095p2-216 | Pituitary, Neuroendocrinology and Puberty | ESPE2022

β-hcg secreting intracranial germinoma in a girl with isosexual precocious puberty : A case report and review of literature

Chen Zhixin , Chen Qiuli , Ma Huamei , Li Yanhong , Zhang Jun , Guo Song

Objective: To analyze the clinical characteristics and treatment of non-germinomatous germ cell tumor (NGGCT) in one girl with the initial onset of homosexual peripheral precocious puberty (PPP) and central diabetes insipidus (CDI) and eventually progressed into central precocious puberty (CPP).Methods: The clinical data of a girl who was diagnosed as NGGCT with the onset of PPP and CDI and eventually progressed into CPP...

hrp0092p2-114 | Fat, Metabolism and Obesity | ESPE2019

Five Years' Follow-up of the Effect of Sex Steroid Hormone on Lipid and Glucose Metabolism in Girls with Turner Syndrome

Guo Song , Li Yanhong , Zhang Jun , Chen Qiuli , Ma Huamei , Chen Hongshan , Du Minlian

Objective: We sought to evaluate the role of long-term HRT on the lipid profile and glucose metabolism in girls with TS.Design: A pre-test/post-test observational study.Seting: Pediatric TS clinic in The First Affiated Hospital, Sun Yat-sen University.Patients: 56 girls with TS had accurate maintenance HRT data....