The main functions of glucagon-like peptide-1(GLP-1) includes diminishing food intake and enhancing glucose-stimulated insulin secretion. Evidence from basic research suggests that reproduction system is also a target of GLP-1. GLP-1 receptor agonists are now widely used in managing patients with obesity and type 2 diabetes, thus, there is a need to explore the effects of GLP-1 on the human reproductive system. This paper is a Chinese translation of "Effects of Glucagon-like Peptide-1 on the Reproductive Axis in Healthy Men" , published on J Clin Endocrinol Metab[Izzi-Engbeaya C, Jones S, Crustna Y, et al. J Clin Endocrinol Metab, 2020, 105(4). DOI: 10.1210/clinem/dgaa072], with the permission from the original journal. This research studied the effects of acute GLP-1 infusion on sex hormones in healthy men through a randomized, single-blind, placebo-controlled study. The results showed that during GLP-1 infusion, the mean levels of serum luteinizing hormone(LH), follicle stimulating hormone(FSH), and testosterone, as well as the pulsatility of LH and testosterone were similar with vehicle infusion. The data from this study indicates that acute GLP-1 administration has no impact on reproductive hormone secretion in healthy men.

Autoimmune hypophysitis is an inflammation of the pituitary gland resulting from autoimmune response, which can be classified as primary hypophysitis and secondary hypophysitis according to the etiology.The clinical presentation includes hypopituitarism and a pituitary mass.The diagnosis is based on clinical findings and radiology, while the pathology serves as the golden standard.The treatment is aimed at reducing the size of the pituitary mass, replacing the defective hormones and decreasing hyperprolactinemia.This review is about the epidemiology, pathogenesis, diagnosis, and treatment of lymphocytic hypophysitis, hypophysitis secondary to cytotoxic T-4 blockade, and IgG4-related hypophysitis.

Craniopharyngioma is a kind of benign tumor of the relict squamous epithelial cells originated from the pituitary gland of cranial cheek pouch in the process of embryonic development. With an overall incidence of 0.13 cases per 100 000 person-years, they may represent up to 5%-10% of intracranial tumors in children. There is always debate regarding the appropriate treatment for craniopharyngiomas which often present symptomatically given their proximity to critical brain structures, and pose significant surgical challenges. And the complications caused by the treatment such as diabetes insipidus, hypothalamic obesity, and hypopituitarism, that throw out a challenge to the postoperative management and the quality of the patients. Craniopharyngioma should be recognized as a chronic disease requiring constant monitoring along with the clinical and multidisciplinary treatment in order to provide optimal care of surviving patients.

Idiopathic hypogonadotropic hypogonadism (IHH) is characterized by delayed or absent puberty and lowered sexual function as a result of impaired pulsatile gonadotropin-realeasing hormone (GnRH) secretion.Identification of TAC3/TACR3 mutations as the culprits of IHH revealed that neurokinin B (NKB) signaling pathway was involved in the regulation of pulsatile GnRH secretion.This review focuses on the involvement of NKB signaling in pulsatile GnRH release,the discovery of TAC3/TACR3 mutations and the phenotypes,and treatment of patients who carry TAC3 or/and TACR3 mutations.

[Summary] Patients with infertility and non-obese polycystic ovary syndrome ( PCOS) were treated with continuous subcutaneous pulse infusion of GnRH. After the treatment, a 32-year old female had regular menstrual cramps. Dominant follicle occurred after 2 months of treatment. The patient was pregnant and now has a healthy baby boy. It shows that the pulse infusion of GnRH could induce spontaneous ovulation and natural fertilization of the patients with non-obese PCOS.

Objective To analyze the clinical characteristics of two patients with 3β-hydroxysteroid dehydrogenase deficiency and to explore their molecular genetic defects.Methods The clinical features and laboratory data of two patients were collected.The exons of HSD3B2 gene were amplified by PCR and sequenced by Sanger sequencing.Results Patient 1, aged 5 yrs old, was raised as a girl with 46, XY karyotype, presented with hyperpigmentation, female infant vulva, clitoral hypertrophy, and bilateral cryptorchidism;Patient 2, aged 11 yrs old, was raised as a girl at birth but as a boy after 1 yr old for known 46, XY karyotype, presented with hyperpigmentation, micropenis and severe hypospadias.Both patients had markedly elevated adrenocorticotropin and decreased cortisol.Two homozygous missense mutations in HSD3B2 gene were identified:conversions of codon Pro155 toLeu(p.P155L)inpatient1,andcodonAla82toThr(p.A82T)inpatient2,bothofwhichwerereportedforthe first time in China.Conclusion The patients with 3β-hydroxysteroid dehydrogenase deficiency in 46,XY karyotype mainly present with male pseudohermaphroditism and adrenocortical deficiency, and the diagnosis should rely on the steroids detection and HSD3B2 gene screening.

Objective To explore more suitable calculation method of the insulin dosage in insulin hypoglycemia-growth hormone stimulation test(insulin tolerance test, ITT). Methods Fifty-six subjects suspected of growth hormone deficiency were divided into primary and secondary onset groups. All the patients took oral glucose tolerance test and ITT. Homeostasis model of assessment for insulin resistance index ( HOMA-IR) and insulin sensitivity index ( ISI), area under insulin curve ( AUCINS ) and the area under glucose curve ( AUCPG ) were calculated. The insulin dosages during ITT between two groups were compared and the main factors influencing the insulin dosage were analyzed. Results There was no difference in the insulin dosage during ITT between primary and secondary groups. The actual dosage of insulin in this cohort study revealed a significant difference from the initial insulin dosage recommended by the guideline. Multiple linear regression analysis found that AUCINS and body mass index were the independent factors affecting the insulin dosage. Then the optimized coefficient of ITT ( γ) were found. Conclusion The insulin dosage used in our study was inconsistent with the guidelines-recommended ones. In order to make ITT more efficient and safer, a more optimized calculation method to improve the successful rate of insulin-induced hypoglycemia in ITT is proposed.

Objective To explore the molecular defects of CYP17A1 gene in a family with 17?-hydroxylase deficiency. Methods Clinical features and laboratory data were collected from the pedigree with 17?-hydroxylase derficiency and the proband was hospitalized in Shanghai Clinical Center for Endocrine and Metabolic Diseases. PCR and subclone sequencing were performed to screen the mutations of CYP17A1 gene. Results The patient was diagnosed as 17?-hydroxylase deficiency according to the clinical presentations, laboratory examination and blood level of steroid hormones. A new type of mutation was identified as a base deletion and a base transversion (TAC/AA) at codon 329 in the patient. It produced a missense mutation of Tyr→Lys at codon 329 and the open reading frame shift following this codon. The patient was homozygous mutation and her parents were heterozygote carrying TAC329AA mutation. Conclusion 17?-hydroxylase deficiency in this family was caused by CYP17A1 mutation (TAC329AA) which was first identified as a complex defects of missense mutation and the open reading frame shift at codon 329.

Objective To explore the relationship between serum leptin level and the hypothalamuspituitary-gonad ( HPG) axis in female adolescents with anorexia nervosa ( AN). Methods Sixteen newly diagnosed female adolescents with AN in Shanghai and Zhejiang province were investigated, and their serum leptin, FSH, LH and body composition were measured before and after 18 weeks treatment, and their menorrhea cycles were observed. The rhythm of 24 h serum leptin secretion was studied in 4 patients with AN. Twenty-six, healthy females with normal weight and without contraceptive medication were studied as controls. Results (1) All of 16 patients had amenorrhea, 3 primary amenorrhea and 13 secondary amenorrhea. At admission LH in 14 patients and FSH in 12 patients were below the low limit of the normal reference range (1.5 U/L and 4. 6 U/L respectively), and among them 5 patiend had both 15% weight gain and significantly increased serum leptin level (P

Objective To investigate the clinical and genetic characteristics in a male patient with 21hydroxylase deficiency combined with adrenal and testicular tumors.Methods Clinical features and laboratory data were collected from the patient.Testicular biopsy was performed.The CYP21 gene was sequenced for mutations.Results The patient presented left adrenal and testicular enlargements.The laboratory examinations showed that plasma ACTH,androstenedione,testosterone,progesterone,and 17-hydroxyprogesterone were markedly elevated.CT scan revealed that the right adrenal gland being resected and the left adrenal with nodular enlargement.Furthermore,testicular biopsy showed a prominent peritubular fibrosis with increased number of peritubular fibroblasts,tubular hyalinisation,and calcification.Sequencing analysis showed a A>G homozygous mutation at intron 2.Conclusion Patients with untreated 21-hydroxylage deficiency may.have adrenal adenomas and(or)testicular adrenal rest tumor simultaneously.