UCH-L1(ubiquitin carboxy-terminal hydrolases L1)is a member of the carboxyl-terminal ubiquitin hydrolase family,naturally,that there is hydrolase activity,however,and that there is ligase acitivity,which is different from the other members of UCHs.UCH-L1 hydrolase activity could keep the pool of free ubiquitin and compromise the ubiquitin-dependent degradation pathway,while UCH-L1 dimerizational-dependent,ubiquityl ligase activity could produce undegradable,K63-linked polyubiquitin chains that could inhibit proteasomal activity.Therefore,UCH-L1 is involved in the more diverse physiological activities,including neuron formation,gonadal development and fertilization.Mutation of UCH-L1 is linked to the neurodegenerative diseases,such as Parkinson's disease.Moreover,abnormal expression of UCH-L1 is response to carcinogenesis in many tissue such as thyroid lung.

AIM: To discuss the effects of phytoestrogenic-genistein on cathepsin K (CK) expression stimulated by interleukin-1α (IL-1α) in osteoclast-like cells (OCLs) . METHODS: The OCLs were isolated from tissue of human giant cell tumor of bone (GCT) . The cells treated with reagents were divided into 7 groups including control (treated with phenol red-free-DMEM), vehicle (treated with 1.2 nmol· L-1 IL- 1α), 10-10-10-6genistein, genistein+ ICI 182.780, and 17[β-estrodiol (17β-E2) group. The cells were treated with 1.2 nmol· L-1IL-1α after pre-treated with genistein or 17β-E2 for 48 h (excluded the control group) . Expression of CK wasdetermined by reverse transcriptase-polymerase chain reaction (RT-PCR) and Western blot in OCLs stimulated by IL-1α in the presence of genistein or 17[β-E2. RESULTS: The obvious increase of expression of CK by IL1α in vehicle group was noted in comparing with control group (P ＜ 0.01 ) . Genistein down-regulated CK gene expression stimulated by IL-1α at the transcription level in a dose-dependent manner (r = 0.68, P ＜ 0.01 ) .Genistein down-regulated CK protein expression stimulated by IL-1α also in a dose-dependent manner (r = 0.61,P ＜ 0.01 ). The effects of genistein were abrogated partly after treatment with the estrogen receptor antagonist ICI 182.780. CONCLUSION: Genistein inhibits CK expression stimulated by IL-1α partly through estrogen receptor in OCLs.

Objective:To evaluate high resolution esophageal manometry (HREM) in peroral endoscopic myotomy (POEM) for pediatric achalasia of cardia (AC).Methods:Data of 30 AC children who received POEM in Xi′an Children′s Hospital from January 2013 to September 2019 were reviewed. HREM was performed before and 6 months after POEM. Preoperative and postoperative lower esophageal sphincter pressure (LESP), 4-second integrated relaxation pressure (4sIRP), Eckardt scores and nutritional status were compared.Results:Children with AC aged between 4-14 years. Postoperative LESP was 5.50±1.13 mmHg (1 mmHg=0.133 kPa), which was significantly lower than that before operation (26.23±4.47 mmHg) with significant difference ( t=-24.623, P<0.001). Postoperative median 4sIRP was 5 mmHg, which was 20 mmHg lower than that before operation (25 mmHg) with significant difference ( Z=-4.786, P<0.001). Postoperative median Eckardt symptom score decreased significantly compared with that before (1 VS 8, Z=-4.796, P<0.001). Severe malnutrition of the AC children improved evidently to normal( Z=-5.166, P<0.001). Conclusion:POEM can significantly improve the characteristics of esophageal dynamics in children with AC, and HREM can be an important indicator for follow-up evaluation of POEM.

OBJECTIVE:To establish a method for simultaneous determination of α-pinene,β-pinene and linalool in volatile oil of Hedychium flavum. METHODS:The volatile oil was extracted from H. flavum according to steam distillation stated in Chinese Pharmacopoeia(2015 edition volume Ⅳ). GC method was adopted to determine the content. The determination was performed on HP-5 capillary chromatographic column(30 m×0.032 mm×0.25 μm)with hydrogen flame ion detector(FID),nitrogen gas as carrier gas,with injector temperature of 200 ℃,and detector temperature of 250 ℃,column flow rate of 0.8 mL/min, samples size of 1 μL and split ratio of 40:1 by programmed temperature. RESULTS:The linear range of α-pinene,β-pinene and linalool were 0.090 5-2.413 3 mg/mL(r=0.999 9),0.098 3-2.620 0 mg/mL(r=0.999 9),0.169 1-4.510 0 mg/mL(r=0.999 8), respectively. RSDs of precision,stability(12 h),reproducibility tests were no more than 2.0%(n=6). The average recoveries were 99.84%(RSD=0.49%,n=6),100.24%(RSD=1.38%,n=6),99.41%(RSD=1.67%,n=6),respectively.The contents of α-pinene,β-pinene and linalool ranged 0.214 4-1.325 0,0.766 2-3.172 1,0.357 4-1.518 7 mg/g in volatile oil from 23 batches of H. flavum produced in Guizhou province. CONCLUSIONS:The method established in this experiment is rapid,simple and accurate.It can be used for the content determination of α-pinene,β-pinene and linalool in volatile oil from H.flavum.

Objective:To explore the efficacy and safety of endoscopic radial incision (ERI) for congenital membranous duodenal stenosis (MDS).Methods:The clinical data of 13 children with MDS receiving ERI in the Department of Gastroenterology of Xi'an Children's Hospital from May 2017 to December 2021 were reviewed and analyzed. The perioperative management, surgical procedures, postoperative complications and follow-up were summarized.Results:There were 5 boys and 8 girls with a median disease duration of 8 (2-20) months, and the median age of diagnosis was 13 months (5-30 months). The septum of 10 cases (10/13) was located in the descending part of the duodenum, and that of 3 cases (3/13) in the horizontal part. The papilla of 1 case (1/13) opened on the septum, that of 3 cases (3/13) within 5 cm of the mouth side of the septum, and that of 9 cases (9/13) within 5 cm of the anal side of the septum. The median diameter of the septal aperture was 3 mm (2-6 mm). All 13 children successfully underwent ERI with a median operation time of 20 min (15-32 min). The average surgical incision was 3 strokes (2-4 strokes), and the endoscope with outer diameter 9.9 mm could pass stenosis after ERI. The median incision diameter was 10 mm (10-12 mm). All patients achieved relief of clinical symptoms after ERI. One patient (1/13) suffered from the postoperative delayed bleeding, which was stopped by endoscopic titanium clamping. No intestinal perforation or duodenal papilla injury occurred, and median postoperative hospital stay was 6 days (5-10 days). The upper gastrointestinal angiogram and gastroscopy were repeated 3 months after ERI, and the median diameter of stenosis was 12 mm (10-15 mm), which was significantly dilated compared with before. The mean body weight increase at 1 month after ERI was 1.20 kg (0.50-1.80 kg), and the mean body weight increase at 3 months was 3.50 kg (2.50-4.00 kg), which reached the normal body weight of the same age.Conclusion:ERI is safe and effective for the treatment of MDS in children, and shows good clinical application and promotion value.

Objective:To investigate the diagnostic and therapeutic value of one-time biliopancreatic-duct-imaging-system (eyeMax)-assisted endoscopic retrograde appendicitis therapy (ERAT) for children.Methods:A total of 11 children who were diagnosed as having uncomplicated acute appendicitis by imaging in Xi′an Children′s Hospital from August to November 2022 were enrolled. All patients received subscope-assisted ERAT. Subscope was intubated into the appendix cavity to observe the mucosa directly. Appendicitis was treated through cleansing, fecalith extraction, stent drainage. The clinical manifestations under subscope were recorded, as well as the treatment success rate, intubation success rate, the effective rate, complication incidence during and after the operation.Results:The age of 11 children was 7.93±2.67 years old. Appendix intubation was successful in all patients. Congestion and swelling of the mucosa in appendiceal orifice and appendix cavity were seen under the subscope in 11 children. There were 6 cases with appendiceal fecaliths, 8 cases with pus and 6 cases with luminal distortion or stenosis. Perforation was observed in 1 case during the operation and no other complication occurred. All patients were treated under subscope, including flushing appendiceal cavity (11 cases), fecalith extraction with extraction basket (3 cases), and appendiceal drainage with stenting (2 cases). The symptoms and signs were relieved after the operation, and the effective rate within 48 hours was 10/11. There were no procedure-related complications or recurrence during postoperative follow-up for 1 week to 4 months.Conclusion:Acute appendicitis could be diagnosed by observing the appendix cavity directly under one-time biliopancreatic-duct-imaging-system-assisted ERAT, and also could be treated with the system, where appendix could be preserved and radiation damage could be avoided with safety and effectiveness.

Folliculogenesis is essential for production of female gametes in vertebrates. However, the molecular mechanisms underlying follicle development, particularly apoptosis regulation in ovary, remain elusive. Here, we generated sox3 knockout zebrafish lines using CRISPR/Cas9. sox3 knockout led to follicle development retardation and a reduced fecundity in females. Comparative analysis of transcriptome between sox3 and wild-type ovaries revealed that Sox3 was involved in pathways of ovarian steroidogenesis and apoptosis. Knockout of sox3 promoted follicle apoptosis and obvious apoptosis signals were detected in somatic cells of stages III and IV follicles of sox3 ovaries. Moreover, Sox3 can bind to and activate the promoter of cyp19a1a. Up-regulation of Cyp19a1a expression promoted 17β-estradiol synthesis, which inhibited apoptosis in follicle development. Thus, Sox3 functions as a regulator of Cyp19a1a expression, via 17β-E2 linking apoptosis suppression, which is implicated in improving female fecundity.