In April 2025， the European Association for the Study of the Liver （EASL） released an updated edition of clinical practice guidelines on Wilson’s disease， and compared with the 2012 edition， the updated guidelines perform detailed elaboration and updates on clinical manifestations， diagnostic algorithms， treatment strategies， and monitoring protocols， emphasize the role of serum exchangeable copper in the diagnosis and monitoring of Wilson’s disease， and provide the reference ranges for clinical application. The updated guidelines also recommend and summarize the drugs for symptomatic treatment of neuropsychiatric symptoms， add a dedicated section on transitioning pediatric patients to adult care， and discuss the optimal timing， multidisciplinary team composition， and implementation frameworks of the transition plan. These updates fully reflect the latest evidence and the development of clinical needs in the diagnosis and treatment of Wilson’s disease. This article gives an excerpt of the recommendations in the guidelines.

Objective:To evaluate the feasibility of using cortical bone trajectory (CBT) screws in the osteoporotic thoracolumbar fixation by comparing the bone CT values at the bone-screw interface between traditional trajectory (TT) screws and CBT screws in patients with different bone densities.Methods:The high-resolution CT imaging data of thoracolumbar segments following thoracic or lumbar spine fractures from April 2020 to October 2022 were collected at The Second Hospital Affiliated to Wenzhou Medical University for retrospective analysis. They were divided into 3 groups: a normal bone mass group, an osteopenia group and an osteoporosis group. From each group 30 cases were chosen (90 cases in total, 36 males and 54 females). All the data were imported into Mimics 18.0 for three-dimensional bone reconstruction in which placement of TT and CBT screws was simulated on the vertebrae from T10 to L2 (non-fractured vertebrae). Regions of interest (ROI) where each simulated screw intersected the bone were segmented to measure their CT bone values. For each vertebra in each group, the relative difference percentage in average CT value of ROI between TT and CBT screws was calculated. The CT values of ROI were compared in the same group between TT and CBT screws from T10 to L2; the CT values of ROI were compared in the same screws among the 3 groups from T10 to L2; the CT values of ROI were compared between the CBT screws in the osteopenia and osteoporosis groups and the TT screws in the normal bone mass group; the relative difference percentages in average CT value of ROI between CBT and TT screws were compared between the 3 groups from T10 to L2.Results:The average CT value of ROI for CBT screws was significantly higher than that for TT screws from T10 to L2 in every group ( P< 0.001); as for the CT values of ROI for CBT and TT screws from T10 to L2, the osteoporosis group0.05). At T10, T12, and L1, the relative difference percentage in average CT value of ROI between CBT and TT screws was significantly higher in the osteopenia and osteoporosis groups than that in the normal bone mass group ( P<0.05), but there was no such a difference between the osteopenia and the osteoporosis groups ( P>0.05). At T11 and L2, there was no significant difference between the 3 groups in the relative difference percentage in average CT value of ROI between CBT and TT screws ( P>0.05). Conclusions:As bone mass decreases, both CBT and TT screws lead to a significant decrease in the bone density at the bone-screw interface. In patients with osteoporosis, CBT screws can still lead to a higher bone density at the bone-screw interface than TT screws, thus providing a higher strength at the bone-screw interface.

Acute liver failure (ALF) is a rare and extremely severe clinical form of Wilson's disease (WD), characterized by progressive aggravation of jaundice and significant coagulation disorder with acute intravascular hemolysis. There is a high risk of severe complications such as hepatic encephalopathy and acute renal failure, and the disease progresses rapidly after onset and has a high mortality rate. At present, it is difficult to diagnose WD presenting as ALF in the early stage due to a lack of unified indicators for rapid diagnosis. Liver transplantation was considered the only effective treatment method for this disease in the past; however, recent studies have shown that medical treatment without liver transplantation can achieve autologous liver relief and recovery in some patients with WD-ALF.

Objective To investigate the factors affecting the vaginal birth after cesarean (VBAC). Methods Totaly 298 women who underwent trial of labor after cesarean section (TOLAC) from Jan 2015 to Dec 2015 were recruited from Beijing Obstetrics and Gynecology Hospital, FuXing Hospital, Tongzhou Maternal and Child Health Hospital of Beijing, the Second Affiliated Hospital of Chongqing Medical University and the People′s Hospital of Chengyang District of Qingdao. The maternal age, the interval from the last cesarean section, the body mass index (BMI) before pregnancy, the weight gain during pregnancy, the way into labor, the Bishop score before labor, the gestational age and the birth weight of the neonate were recorded in a self-made form. The factors affecting VBAC were analyzed by univariate analysis and multivariable logistic regression. Results (1)The incidence of VBAC, uterine rupture, postpartum hemorrhage and neonatal asphyxia were 70.5%(210/298), 2.7%(8/298), 9.4% (28/298) and 1.3% (4/298), respectively. No maternal death and perinatal death occurred. (2)The univariate analysis suggested that the maternal age, the BMI before pregnancy, the Bishop score before labor, the labor induction, the gestational age at delivery and the neonatal weight were factors affecting VBAC. The maternal age and the Bishop score before labor were significantly higher in the VBAC group than in the unsuccessful TOLAC group(P<0.05). While the BMI before pregnancy, the induction rate, the gestational weeks at delivery and the birth weight of the neonate were significantly lower in the VBAC group than in the unsuccessful TOLAC group (P<0.05). Multivariable logistic regression analysis showed that successful VBAC was affected by the maternal age, the BMI before pregnancy, the Bishop score before labor and the birth weight of the neonates(P<0.05). Conclusion The maternal age, the BMI before pregnancy, the Bishop score before labor and the birth weight of neonate are the main factors affecting VBAC.

The study is aimed to research the relationship between the seedling grade of Codonopsis pilosula and yield and quality of medicinal materials, so as to provide basis for establishing seedling standard. Thirty seedlings of C. pilosula were collected from the main production areas in Gansu province, such as Weiyuan, Minxian, Zhangxian, Dangchang and Longxi, root length and diameter and weight of all the samples were measured. According to the clustering results, seedlings were divided into 3 levels, and field experiments were conducted with three levels seedling, yield and quality were tested in laboratory. Results have showed that emergence of grades 1 was faster than that of grades 2 and 3. Yield of grades 1 was significantly higher than that of grades 2 and 3 (P<0.05). Propargyl glycoside content of grades 1 was the highest, and significantly higher than that of grades 3. Polysaccharide content of grades 3 was the highest and significantly higher than that of grades 1 and 2 (P<0.05). So considering yield, quality and investment cost of C. pilosula, planting seedlings of C. pilosula should select that root length>15.6 cm, root diameter>2.7 mm, root weight>0.56 g.

Objective To compare the effects of application of clavicular hook plate combined with wire anchors anatomical coracoclavicular ligament reconstruction and application of clavicular hook plate in the treatment of NeerII distal clavicle fracture and Tossy Ⅲtype~V acromioclavicular joint dislocation. Methods A retrospective analysis of the clinical data from June 2006 to June 2013. Total 73 cases patients suffered with Neer Ⅱtype distal clavicle fractures and Tossy Ⅲtype~V acromioclavicular joint dislocation. Of which , 41 cases were subjected to treatment with clavicular hook plate , 32 patients subjected to treatment of using clavicular hook plate combined with anchors .The incision length, operative time, postoperative complications, length of hospital stay and postoperative 1 month, 6 month shoulder VAS score of two groups were analyzed; the shoulder function of both groups after 1 month, 6 months were assessed by using Constant shoulder function assessment method. Results Surgical incision length and operational time between the two groups were statistically significant (P<0.05), while the amount of bleeding was not statistically significant. All patients were followed up . The two groups did not occur any complications such as loosening, decoupling, acromioclavicular joint dislocation and wound infections. Hospitalization time was 5~14 d (averaged 10d), no significant difference between two groups. 4 the shoulder Constant score and VAS scores showed no significant difference 1 months postoperation; 6 months after hook plate removed, VAS score and Constant shoulder score improved significantly in anchors hook plate group (P<0.05). Conclusion Anatomical coracoclavicular ligament reconstruction by application of hook plate combined with anchors is a good biomechanical model characterized with simple surgery , less trauma and good clinical outcomes , worthy of clinical application.

Objective To evaluate the maternal and fetal outcomes of planned delay in treatment for cervical microinvasive squamous cancer during pregnancy.Methods A prospective study of pregnant women was done from August 1,2007 to May 31,2010.Pregnant women who had not been carried out cervical cytological screening within one year were got thin-prep cytology test (TCT) screening at their initial prenatal visit.Patients with abnormal cytological results were performed colposcopic examination and directed biopsy.Women with cervical microinvasive cancer were followed up every 8 to 12 weeks.If lesion progression were suspected,compared with previous image,repeated biopsy directed by colposcopy should be performed.Once worsening invasive cancer was confirmed,the pregnancy should be terminated timely.All patients should be reevaluated 6 to 12 weeks postpartum with repeated colposcopic examination and biopsy.All mothers were performed cold knife conization (CKC) at 6 to 12 weeks postpartum.Results We totally diagnosed 17 cases cervical microinvasive squamous carcinoma during pregnancy.The positive rate is 6.2/10 000 (17/27 230).After informed consent,15 pregnant women decided to delay treatment until fetal maturation.The mean gestational age of initial diagnosis was (19.3 ± 5.9) weeks.The women were followed up 2 to 4 times during pregnancy.Only 1 patient was verified lesion progression by directed biopsy at 34 weeks and delivered by cesarean section.The progression rate during pregnancy was 1/15.The mean delivered time was (37.1 ± 1.8) weeks (ranged from 34 to 40 weeks).The mean diagnosis-to-delivery interval was (18.4 ± 5.2) weeks.All patients were delivered by cesarean section and all newborns had good outcomes.Finally we confirmed 1 case with cervical cancer stage Ⅰ a2,11 cases with stage Ⅰ al,3 cases with cervical intraepithelial neoplasia (CIN) Ⅲ by pathological diagnosis after CKC during 6 to 12 weeks postpartum.All cases were disease free after follow-up ranged from 22 to 48 months.Conclusions It is necessary to perform TCT screening for pregnant women who have not been carried out cervical cytology screening within 1 year.If cervical microinvasive squamous cancer were suspected during pregnancy,in order to achieve fetal maturity it is acceptable for the women who desired pregnancy to delay treatment under closely monitoring until postpartum.

Objective To investigate the relationship between cervical intraepithelial neoplasia (CIN) and high-risk (HR)HPV infection among late pregnant women.Methods From Aug.2007 to Feb.2010,168 women at 13 to 32 gestational weeks undergoing prenatal examination in Beijing Obstetrics and Gynecology Hospital went through three stage cervical disease screening,including 21 women with cervicitis and 147 women with C1N (42 women with CIN Ⅲ,37 women with CIN Ⅱ and 68 women with CIN Ⅰ ).Hybrid capture assay version Ⅱ ( HC- Ⅱ ) test was used to measure HR-HPV DNA load,and the logarithmic transtormation (log10) was performed.All 168 women were followed up to postpartum 3 -6 months.HR-HPV infections rates of cervicitis and different CIN,the rate of HR-HPV infection turned naturally negative at postpartum of 3 to 6 months,and HR-HPV load at pregnancy and 3 -6 months postpartum were observed.Results ( 1 ) HR-HPV infection rate:CIN Ⅲ,Ⅱ,Ⅰ and cervicitis pregnant women's HR-HPV positive infection rates were 98％ (41/42),86％ ( 32/37 ),76％ ( 52/68 ) and 62％( 13/21 ) respectively,which reached statistical difference (P =0.002).(2) HR-HPV naturally negative:the rate of pregnant women with different levels of CIN who turned HR-HPV naturally negative within 3 -6 months of postpartum were CIN Ⅲ 5％ (2/41),CIN Ⅱ 47％ (15/32),CIN Ⅰ 52 ％ (27/52) and cervicitis 10/13,which also reached statistical difference among those four groups (P =0.000).(3) HR-HPV load:pregnant women with different grade of CIN and cervicitis HR-HPV DNA load were CIN Ⅲ 2.02 ng/L(1.53,2.67 ng/L),CIN Ⅱ 1.94 ng/L ( 0.75,2.75 ng/L),CIN Ⅰ 2.04 ng/L (0.08,2.95 ng/L) and cervicitis 1.98 ng/L( -0.07,2.47 ng/L).There was no significantly different HPV load in women with cervicitis and different CIN (P =0.719).At 3 -6 months postpartum,HR-HPV load was CIN Ⅲ1.55 ng/L(0.90,2.10 ng/L),which was significantly higher than the amount of CIN Ⅱ 0.09 ng/L(-0.69,1.74 ng/L),CIN Ⅰ 0.48 ng/L( -0.56,2.2 ng/L) and cervicitis -0.46 ng/L ( -0.78,1.40 ng/L,P =0.036).Conclusions With the increasing of CIN grade,the rate of HR-HPV infection in pregnant women was increased,however,the rate of HR-HPV turning negative naturally at 3 -6 months postpartum decreased.With different CIN grade during pregnancy,HR-HPV DNA load did not change significantly,but HR-HPV DNA load increased at 3 -6 months of postpartum.HR-HPV DNA loads with the same grade of CIN and cervicitis during pregnancy higher than that of postpartum among pregnant women.