hrp0084p3-716 | Diabetes | ESPE2015

Hyperglycaemia During Chemotherapy for Acute Lymphoblastic Leukaemia Among Taiwanese Children

Tsai Meng-Che , Chen Jainn-Shiu

Background: Hyperglycaemia is a common occurrence during the treatment for paediatric acute lymphoblastic leukaemia (ALL). Emergence of new evidence exhibits conflicting results. The incidence of hyperglycaemia during chemotherapy has not been well described in the Asian population.Objective and hypotheses: The aim of study is to delineate the characteristics of paediatric patients at risk for hyperglycaemia during chemotherapy.Met...

hrp0082p1-d3-47 | Bone (1) | ESPE2014

Relation Between CNP Signaling Pathway and the Effect of Combined Treatment with GnRHa and rhGH on the Linear Growth in Mid/Late Pubertal Girls at Great Bone Ages with CPP or EFP

M A Hua-mei , S U Zhe , L I Yin-ya

Objectives: To evaluate the effect of combined treatment with GnRH analogue (GnRHa) and recombinant human GH (rhGH) on the linear growth in mid/late pubertal girls at great bone ages with central precocious puberty (CPP) or early and fast puberty (EFP). To investigate the relation between C-type natriuretic peptide (CNP) signaling pathway and GH’s effect on linear growth in these girls. Methods 22 girls were diagnosed as CPP or EFP, whose bone ages were older than 11.5 ye...

hrp0082p2-d1-457 | Growth | ESPE2014

Relation Between CNP and the Effect of Combined Treatment With GnRHa and GH on the Linear Growth in Mid/Late Pubertal Girls with Central Precocious Puberty or Early and Fast Puberty at Great Bone Ages

Hua-mei M A , Zhe S U , Yin-ya L I

Background: It’s well known that GnRH analogue (GnRHa) could not improve the final height of mid/late pubertal girls with central precocious puberty (CPP) or early and fast puberty (EFP) for their low growth potential.Objectives: To evaluate the effect of combined treatment with GnRHa and GH on the linear growth in mid/late pubertal girls with CPP/EFP at great bone ages. To investigate the relation between C-type natriuretic peptide (CNP) and GH&#14...

hrp0082p3-d1-762 | Fat Metabolism & Obesity | ESPE2014

A Rare Case of Sea-Blue Histiocytosis Associated with Niemann–Pick Disease Type B in a 8-year and 9-month Old Boy with Hypertension

Ma Hua-mei , Su Zhe , Li Yan-hong

Background: Sea-blue histiocytosis is a morphological finding that can be associated both with acquired conditions of increased cellular turnover and inborn errors of lipid metabolism.Objective and hypotheses: To present a Chinese boy of hypertension and sea-blue histiocytosis secondary to Niemann–Pick disease type B.Methods: Diagnosis was confirmed by the bone marrow aspiration and the specific enzyme assay of leukocytes (def...

hrp0084p3-590 | Adrenals | ESPE2015

Characterisation of Ovarian Adrenal Rest Tumours in Children and Adolescent Females with Congenital Adrenal Hyperplasia due to 21-Hydroxylase Deficiency

Su Zhe , Li Yanhong , Ma Huamei , Du Minlian

Background: Ovarian adrenal rest tumours (OARTs) are rare in contrast to testicular adrenal rest tumours (TARTs).Objective and hypotheses: To summarise the characterization of OART in children and adolescent females with congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency (21-OHD).Method: We have diagnosed four cases of CAH 21-OHD with OART in the recent 5 years and summarised the characterisations.<p class="a...

hrp0084p3-793 | DSD | ESPE2015

A Novel Mutation of the AR Gene Causes Androgen Insensitivity Syndrome: A Case Report

longjiang Zhang , xionghui Xian , shufeng Tian , xia Liu , zhe Su

Background: A 8-year-old Chinese girl was referred by the endocrinologist of our hospital because of ambiguous external genitalia.Physical examination revealed breast and axillary hair and pubic hair at Tanner stage 1, There had two mass located within two side of inguinal regions,labia fusion, the size of clitoris was 2.0×1.0 cm, there was a vaginal opening. US images revealed a solid nodile suggestive of testicular tissue located in both inguinal regions. The MRI exam c...

hrp0089p3-p359 | Sex Differentiation, Gonads and Gynaecology or Sex Endocrinology P3 | ESPE2018

Long-Term Follow-up in a Chinese Child with Lipoid Congenital Adrenal Hyperplasiadue to STARmutation

Zhao Xiu , Liu Xia , Wang Li , Pan Lili , Zhang Longjiang , Su Zhe

Congenital lipoid adrenal hyperplasia (CLAH) is the most severe and extremely rare form of congenital adrenal hyperplasia (CAH). The typical features include 46, XY disorder of sex development (DSD), early-onset adrenal crisis and enlarged adrenal with fatty accumulation. We reported a case of congenital lipoid adrenal hyperplasia (CLAH) caused by steroidogenic acute regulatory protein (STAR) gene mutation. The patient had typical early-onset adrenal crisis at the age of 2 mon...

hrp0089p3-p360 | Sex Differentiation, Gonads and Gynaecology or Sex Endocrinology P3 | ESPE2018

46,XY Gonadal Dysgenesis Accompanied by Neuropathy Caused by a DHH Mutation

Su Zhe , Pan Lili , Wang Li , Chen Weiyan , Song Jianming , Li Shoulin

Objectives: To summarize the clinical manifestations of a patient of 46,XY partial gonadal dysgenesis (PGD) accompanied by neuropathy. This is the first reported case of DHH mutation from China.Methods: We retrospectively reviewed the case and summarized the clinical history, physical examinations, laboratory and genetics tests, electromyography, ultrasound, surgical exploration and histopathology results.Results: The 14.3-year-old...

hrp0082p3-d2-639 | Adrenals &amp; HP Axis (1) | ESPE2014

Four Cases of Ovarian Adrenal Rest Tumors in Chinese Girls with Congenital Adrenal Hyperplasia due to 21-Hydroxylase Deficiency

Zhe Su , Jun Zhang , Huamei Ma , Qiuli Chen , Minlian Du , Yanhong Li , Hongshan Chen

Background: Ovarian adrenal rest tumors (OART) is a rare complication of congenital adrenal hyperplasia (CAH) and is not very well characterized yet.Objective and hypotheses: We report four cases of OART in Chinese girls with CAH due to 21-hydroxylase deficiency (21 OHD).Method: We describe the clinical, imaging, and surgical findings of the patients.Results: The four cases of CAH with OART included three sal...

hrp0082p3-d3-803 | Gonads and Gynaecology | ESPE2014

Menstrual Regularity Among Early Menarche Girls and CPP or EFP Girls Treated with GnRHa

Qiuli Chen , Yanhong Li , Zhe Su , Huamei Ma , Hongshan Chen , Jun Zhang , Minlian Du

Objective: We assessed in a retrospective unicenter study the effect on menstrual regularity of early menarche and treatment with GnRH analogs (GnRHa) in central precocious puberty (CPP) or early and fast puberty (EFP) girls.Methods: Six hundred and ten healthy girls were interviewed and their menarche age and menstrual interval were recorded. One hundred and sixty-nine CPP or EFP girls who were treated with GnRHa were fellowed up, and their menarche age...