Uterine artery embolization (UAE) is the purpose of treatment-related diseases by embolizing the vascular network of uterine lesions. Because of the small damage, the recovery time of patients is short, and it is recognized by the majority of patients. However, it is still important to pay attention to the poor effect and complications of 10% of the patients. Three-dimensional reconstruction of the lesion before surgery, could calculate the optimal angle of inclination of the tube to guide uterine artery embolization, reduce the X-ray. Radiation dose, exposure time and rational distribution of embolic agents to achieve personalized guidance for UAE. Now, we elaborate the research progress of three-dimensional reconstruction in the application of uterine artery embolization in gynecological diseases.

Chloride Channel-3 (ClC-3) is a member of the Cl2 channel superfamily,which is encoded by CLCN3 gene.It involves regulating electrical activity,cell volume,proliferation,migration,invasion,apoptosis and intracellular pH,which have become a hot spot of current research.In recent years,the research of ClC-3 in gynecological diseases is increasing day by day.This article will review the research progress of ClC-3 and benign and malignant gynecological diseases,especially the relationship between ClC-3 and the occurrence and development of endometriosis.

Objective To compare the results of GnRHa stimulation test and GnRH stimulation test in girls with Idiopathic central precocious puberty.Methods The girls aged 6-10 who were diagnosed with early breast development (＜ 8 years old) from January 2016 to June 2018 were randomly divided into GnRHa stimulation test group and GnRH stimulation test group according to clinical manifestations,auxiliary examinations and follow-up.The difference in the results of the stimulation test between the two groups was compared.Results A total of 108 girls were enrolled in this study,and 40 (37％) of the GnRHa stimulation tests were performed.Among them,25 (63％) patients with idiopathic central precocious puberty had the confirmed diagnosis,and the GnRH stimulation test was performed.Of the 68 (63％) patients,30 (44％) had idiopathic central precocious puberty.The peaks of LH in the GnRHa challenge test group and the GnRH challenge test groupwere 11.33 (6.81,15.79) and 7.89 (5.35,14.21),and the FSH peaks were 15.68 (10.18,20.06) and 17.26 (13.34,21.42),showing no significant differences (U =1078.50,P =0.07;U =1617.50,P=0.10).Thepeak values of LH/FSH were0.86 (0.37,1.17)and0.52 (0.31,0.83),respectively,and there was a statistical difference (U =953.00,P =0.01).GnRH challenge test showed the ICPP sensitivity of 90.91％ (81.10％-100.72％),specificity of 94.29％ (86.60％-101.98％),total coincidence rate of 92.65％ (86.44 ％-98.85％),Yoden index of 0.85 (0.72％-0.98％).Sensitivity of the GnRHa challenge test showed the ICPP diagnosis rate of 95.24％ (86.13％-104.35％),specificity of 73.68％ (53.88％-93.48％),total coincidence rate of 85.00％ (73.93％-96.07％),Yoden index of 0.69 (0.47-0.91).Conclusion For the diagnosis of idiopathic central precocious puberty in girls,the GnRHa challenge test is more sensitive than the GnRH challenge test,and the specificity and the Yoden index are both low.Therefore,routine use of GnRHa stimulation test as the replacement of GnRH stimulation test is not recommended.

OBJECTIVE: To summarize clinical manifestations, inheritance pattern and mutations of NR0B1 gene in 7 children with X-linked adrenal dysplasia congenita (XL-AHC). METHODS: Clinical data of the 7 children was collected. Next-generation sequencing was carried out to detect potential mutations in the coding regions of adrenal gland-related genes. Suspected mutations were verified with Sanger sequencing. RESULTS: In all of the children, the initial symptom was adrenocortical insufficiency. Five cases had neonatal onset, while the remaining two developed it at the age of 2. Three cases (42.9%) had a short stature and 1 showed growth retardation (14.3%). Of the 7 cases, 6 (85.7%) had mutations occurring in exon 1, and 1 (14.3%) had it occurring in exon 2. Four cases (57.1%) were frameshift mutations, 2 cases (28.6%) were nonsense mutations and 1 case (14.3%) was missense mutation. Two mutations were known to be pathogenic, and 5 had not been reported previously. Maternal inheritance was found in 6 cases. Three children had a maternal uncle died of unexplained causes. The mothers of 2 children had a history of spontaneous abortions. One child had a brother died of unexplained reason. CONCLUSION Male children with primary adrenal insufficiency should be routinely checked for NR0B1 mutations, especially those with a family history. mutations of NR0B1 gene occur mostly in exon 1, with frameshift mutations being the most common type. The development of all patients with XL-AHC should be closely monitored during follow-up.
Adrenal Insufficiency ; Child ; DAX-1 Orphan Nuclear Receptor ; DNA Mutational Analysis ; Genes, X-Linked ; Humans ; Hypoadrenocorticism, Familial ; Male ; Mutation

OBJECTIVETo analyze clinical characteristics, genetic mutation and therapeutic effect of seven patients diagnosed with congenital hyperinsulinism(CHI).

METHODSClinical data for the patients was retrospectively analyzed.

RESULTSAll patients presented with hyperinsulinism(serum insulin:2.0-58.4 mU/L),even after hypoglycemia (blood glucose: 0.7-2.39 mmol/L) has developed. Mutations were identified in 4 patients (57.1%), which included a heterozygous c.262C to T(p.R88C) mutation in exon 4 of the UCP2 gene, a heterozygous c.1495C to A(p.G499C) mutation in exon 12 of the GLUD1 gene, a heterozygous c.1493C to T(p.S498L) mutation in exon 1 of the GLUD1 gene, and a heterozygous c.4432G to A(p.G1478R) mutation in exon 37 of the ABCC8 gene. The patient carrying a maternally inherited ABCC8 mutation was treated with cornstarch and had his blood glucose kept normal. All other patients responded well to diazoxide.

CONCLUSIONA genetic diagnosis was attained for 51.7% of patients in this study. Mild CHI patients can have their blood glucose controlled by giving cornstarch. Diazoxide is safe and effective for most CHI patients.

The concept of enhanced recovery after surgery has been widely used in various disci-plines. Because of the need of anaesthesia and metabolism after surgery and the particularity of gynecologi-cal pelvic surgery, most surgical operations require indwelling catheter. However, indwelling catheter has many complications, such as urinary tract infection, difficult intubation, leakage, bladder function damage and difficult extubation. This article mainly discussed the progress of rehabilitation surgery and the applica-tion of early removal of ureter, and discussed the complications of indwelling catheter, in order to shorten the time of indwelling catheter after surgery, so as to reduce the symptoms of urinary tract irritation, the rate of urinary tract infection, and the nervous and anxious mood of patients, as well as accelerate the recovery of patients after surgery.