Biliary fibrosis (BF) is the result of pathological repair of bile tract injury, characterized by thickening and sclerosis of the bile duct wall and progressive stricture of the lumen, which may ultimately lead to serious adverse outcomes such as biliary obstruction, biliary cirrhosis, liver failure, and hepatobiliary malignancies. Current research describes BF as a pathological feature of certain bile tract diseases, lacking a systematic summary of its etiology, pathophysiology, molecular mechanisms, and treatment. BF is a common but easily neglected disease state in biliary system, which may promote the development and progression of hepatobiliary diseases through abnormal repair mechanism after pathological biliary tract injury. Based on the latest research progress from both domestic and international perspectives, the authors review the concept, clinical manifestation, etiology, pathogenesis, and therapeutic strategies of BF to provide a reference for clinical physicians.

Humans ; Melanoma/therapy* ; Immunotherapy ; Syndrome ; Skin Neoplasms

  Objective  Quantitative imaging evaluation was performed on the liver and spleen system lesions of patients with Gaucher disease after treatment. in order to deepen the understanding of Gaucher disease.  Methods  From August 1999 to August 2018, we registered, examined and treated children with Gaucher disease, and conducted quantitative imaging research on 40 children with Gaucher disease who were intensively followed up in Beijing Children's hospital, Capital Medical University until August 2018. At the same time, 34 normal volunteers were matched. All subjects were scanned with magnetic resonance imaging(MRI). The fat fraction(FF), iron content(R2^*), standard apparent diffusion coefficient(sADC), slow apparent diffusion coefficient(D), fast apparent diffusion coefficient(D^*) and perfusion fraction(f)of the liver and spleen were measured. The quantitative parameter values measured by patients with Gaucher disease and normal subjects were statistically analyzed by independent sample t-tests.  Results  The results showed that there was no significant difference in FF, R2^*, sADC, D, D^*, f of the liver and spleen, and liver elasticity was also within the normal range. However, the volume of liver and spleen in patients was significantly different from that in normal subjects.  Conclusions  After treatment, the volume of the liver and spleen in patients with Gaucher disease is greater than that of normal people, but other quantitative parameters are within the normal range, indicating that long-term enzyme replacement therapy can delay the progress of liver and spleen diseases to a certain extent. Quantitative imaging has a certain value in the evaluation of Gaucher disease.

A fast,reliable,and cost-effective liquid chromatography-tandem mass spectrometry method was established to determine the effects of the traditional Chinese medicine employed to treat coronavirus disease 2019,namely,Lianhua Qingwen granules,Huoxiang Zhengqi capsules,Jinhua Qinggan granules,Shufeng Jiedu capsules,and Angong Niuhuang pills,on the pharmacokinetics of lopinavir/ritonavir in rats.Blood samples were prepared using the protein precipitation method and atazanavir was selected as the internal standard(IS).Separation was performed on an Agilent ZORBAX eclipse plus C18(2.1 mm x 50 mm,1.8 μm)column using acetonitrile and water containing 0.1％formic acid as the mobile phase for gradient elution.The flow rate was 0.4 mL/min and the injection volume was 2 μL Agilent Jet Stream electrospray ionization was used for mass spectrometry detection under positive ion multiple reaction monitoring mode at a transition of m/z 629.3→447.3 for lopinavir,m/z 721.3→296.1 for rito-navir,and m/z 705.4→168.1 for the IS.The method showed good linearity in the concentration range of 25-2500 ng/mL(r=0.9981)for lopinavir and 5-500 ng/mL(r=0.9984)for ritonavir.The intra-day and inter-day precision and accuracy were both within±15％.Items,such as dilution reliability and residual effect,were also within the acceptable limits.The method was used to determine the effects of five types of traditional Chinese medicines on the pharmacokinetics of lopinavir/ritonavir in rats.The pharmaco-kinetic results showed that the half-life of ritonavir in the groups administered Lianhua Qingwen granules and Huoxiang Zhengqi capsules combined with lopinavir/ritonavir was prolonged by approx-imately 1.5-to 2-fold relative to that in the control group.Similarly,the pharmacokinetic parameters of lopinavir were altered.Overall,the results of this study offer important theoretical parameters for the effective clinical use of five types of traditional Chinese medicines combined with lopinavir/ritonavir to reduce the occurrence of clinical adverse reactions.

The aim of this study was to investigate the preventive effect of pancreatic duct stent on acute pancreatitis after endoscopic retrograde cholangiopancreatography. A retrospective analysis of the case data of patients who first underwent endoscopic retrograde cholangiopancreatography for choledocholithiasis in the Beijing Friendship Hospital from January 2015 to December 2019 for 5 years. According to whether the pancreatic duct stent was indwelled during the operation, they were divided into pancreatic duct stent group (147 cases) and non-indwelling pancreatic duct stent group (192 cases). The incidence of acute pancreatitis after endoscopic retrograde cholangiopancreatography was compared between the two groups according to COTTON criteria. Independent sample t test, Pearson Chi-square test (χ 2) and Fisher′s exact test were used to compare groups′ differences. There were 2 cases of acute pancreatitis in the pancreatic duct stent group, all of which improved after 48 hours. There were 22 cases of acute pancreatitis in the non-indwelling pancreatic duct stent group, of which 20 cases improved within 48 hours, and the other 2 cases had severe pancreatitis, which improved and discharged after 30 days of treatment. There was significant difference in the incidence of acute pancreatitis between the pancreatic duct stenting group (1.4%) and the group without placement of pancreatic duct stents (11.5%) (χ2=12.905, P<0.001). In conclusion, Pancreatic duct stent may be an effective method to prevent PEP.

The aim of this study was to investigate the preventive effect of pancreatic duct stent on acute pancreatitis after endoscopic retrograde cholangiopancreatography. A retrospective analysis of the case data of patients who first underwent endoscopic retrograde cholangiopancreatography for choledocholithiasis in the Beijing Friendship Hospital from January 2015 to December 2019 for 5 years. According to whether the pancreatic duct stent was indwelled during the operation, they were divided into pancreatic duct stent group (147 cases) and non-indwelling pancreatic duct stent group (192 cases). The incidence of acute pancreatitis after endoscopic retrograde cholangiopancreatography was compared between the two groups according to COTTON criteria. Independent sample t test, Pearson Chi-square test (χ 2) and Fisher′s exact test were used to compare groups′ differences. There were 2 cases of acute pancreatitis in the pancreatic duct stent group, all of which improved after 48 hours. There were 22 cases of acute pancreatitis in the non-indwelling pancreatic duct stent group, of which 20 cases improved within 48 hours, and the other 2 cases had severe pancreatitis, which improved and discharged after 30 days of treatment. There was significant difference in the incidence of acute pancreatitis between the pancreatic duct stenting group (1.4%) and the group without placement of pancreatic duct stents (11.5%) (χ2=12.905, P<0.001). In conclusion, Pancreatic duct stent may be an effective method to prevent PEP.

Objective: To explore the imaging manifestations of thoracic CT in patients with Gaucher disease (GD) in order to improve the diagnostic ability. Methods: Forty-three patients with GD were collected from May 2003 to October 2018 in Beijing Children′s Hospital, including 25 males and 18 females, aged from 10 to 34 years, with an average age of (21±6) years. All the patients underwent routine chest CT examinations, and analysis and description of pulmonary interstitial and parenchyma imaging manifestations were performed. Results: Among the 43 GD patients, 20 patients presented with abnormal chest CT findings: 10 showed diffuse interlobular septa thickening, mainly distributed in the lower lobes of both lungs; 5 showed ground glass opacities in a single or multiple lobes of the lung. There were 2 cases with small nodules, which showed round-like nodules of different sizes. One case had pulmonary fibrosis, especially in the left upper lobe. Other manifestations included bullae in 3 cases,localized pleural thickening in 2 cases, pneumothorax in 1 case; pulmonary hypertension in 1 case and thymus enlargement in 12 cases. Most of the GD patients had pulmonary lesions between 10 and 14 years old. The signs of interlobular septa thickening and thymus enlargement were common, with 5 cases in each age group. Conclusions GD involves the lungs in half of the patients. The manifestations of the lungs are diverse, and most of them are diffuse interstitial lesions. The main signs are interlobular septal thickening and ground glass opacity, which are consistent with the pathology of Gaucher cell infiltration.But the signs are not specific, the diagnosis should be made in combination with the clinical information, and attention should be paid to the differentiation of lung infiltration caused by other diseases.

Objective To explore the significance of the cut point of peak level of luteinizing hormone (LH) in gonadotropin-releasing hormone (GnRH) agonist test in the diagnosis of precocious puberty in obese girls.Methods According to the diagnostic criteria of children's central precocious puberty diagnosis consensus (2015),796 cases of precocious puberty (peak level of LH ≥3.3 IU/L) in Children's Hospital of Zhejiang University School of Medicine from January 2014 to December 2015 were divided into normal weight group (573 cases),overweight group (170 cases) and obesity group (53 cases).The 3 groups were compared in terms of basic level of LH,basic level of follicle stimulating hormone (FSH),peak level of LH and FSH,ratio of LH (peak)/FSH (peak),sex hormone binding protein (SHBG),and children with 3.3 IU/L ≤peak level of LH ＜5.0 IU/L were followed up.The accuracy of the diagnosis of central precocious puberty using peak level of LH was analyzed by using receiver operating characteristic (ROC) curve.Results The median of LH (peak) in the obese group was 6.92 IU/L,the median of SHBG was 46.52 nmol/L,the median of LH (peak) in normal weight group was 8.92 IU/L,and the median of SHBG was 87.28 nmol/L.There were significant differences between the 2 groups (P ＜ 0.05,0.001).A total of 89 cases in normal weight group and 65 cases of obesity/overweight group (3.3 IU/L ≤peak level of LH ＜5.0 IU/L) were followed up for 1 year,and 18 cases in normal weight group and 33 cases in obesity/overweight group developed to central precocious puberty,while the number of cases in the 2 groups was significantly different (P ＜0.001).The ROC curve analysis showed that the cut point of peak level of LH was 4.16 IU/L,the sensitivity was 0.606,the specificity was 0.656,and the Jordan index was the largest (0.344).Conclusions The peak level of LH in GnRH agonist test is important for the diagnosis of central precocious puberty in girls,but the effect of body mass index on its cut point needs to be considered.

Objective To analyze the CT and MR imaging characteristics of the subcutaneous and organ lesions of uvenile xanthogranuloma (JXG) in children.Methods The CT and MR images of the head,chest,abdomen and subcutaneous soft tissue of 11 patients (6 females and 5 males,aged 2 months-12 years old,median age 8 months old) with JXG from 2006 to 2018 were collected and retrospectively analyzed.Eight patients underwent CT scan and/or contrast-enhanced CT scan,and 7 patients underwent MRI and/or MRI and contrast-enhancement MRI.Results In 11 cases of JXG,5 cases were classified as simple skin type,involving the skin and deep soft tissue in the right posterior neck,the right frontal and parietal parts of the head,the nose root,and the fat layer and the deep muscle in the extremities.The lesions were the subcutaneous soft tissue nodules or mass showing isodensity on CT or high signal on T2WI.And the other 6 cases were classified as systemic type whose lesions involved one or more than one part except the skin.In the 6 cases,3 cases involved the central nervous system,including the pituitary gland,cerebral hemisphere,cerebellum,brain stem,basal ganglia,thalamus,optic chiasma and spinal cord.The lesions of the pituitary gland were manifested as pituitary enlargement,thickening of the pituitary stalk,and disappearance of high signal on T1WI in the neurohypophysis.The brain parenchyma,the spinal cord and the optic chiasma showed multiple flake or nodular masses,which were iso/low signal on T1WI,low / high signal on T2WI,diffusion restricted,and enhanced markedly.Two cases involved lung tissue,and their lesions showed diffuse or scattered multiple cloudy shadow,reticular opacity,strip and small nodule shadow on chest CT.There was pleural effusion in one case.Two cases involved the livers and 2 cases involved the kidneys,showing multiple nodules with low signal on T2,WI and slight enhancement.One case involving the pancreas showed a low density in the head of the pancreas and slight enhancement on CT.One case involving the greater omentum,chest wall and peritoneum showed thickening of the corresponding tissues on CT.Conclusions Subcutaneous and systemic lesions of JXG show some characteristics on CT and MRI images.The main manifestations are single or multiple nodulars,which are diffusely distributed and vary in size in subcutaneous soft tissue and various organs.The combination of imaging appearances,clinical manifestations and biopsy has important clinical significance for the correct diagnosis and evaluation of JXG.

Objective To identify the risk factors for postoperative spinal cord injury in Stanford type A aortic dissection patients.Methods 210 Stanford type A aortic dissection(TAAD) patients underwent Sun's procedure in Beijing Aortic Disease Center during July 2014 to March 2015.14 patients had spinal cord injury after surgery.Clinical data and computed tomography angiography(CTA) imaging of aorta were retrospectively analyzed and multi-logistic regression analysis was performed to identify risk factors for spinal cord injury post operation.Results 14 out of 210(6.7％) patients had transient or permanent spinal cord injury after surgery.Univariate analysis showed only false lumen derived intercostal arteries at eighth thoracic vertebral level (T8) to first lumbar vertebral level (L1) was significantly associated with post-surgery spinal cord injury (P =0.000).Multi-logistic regression analysis showed that false lumen derived intercostal arteries (P =0.000) and age (P =0.016) were significantly associated with postoperative spinal cord injury.Conclusion Major intercostal arteries derived from false lumen and rapid thrombogenesis in false lumen are the major risk factors for postoperative spinal cord injury in Stanford type A aortic dissection patients.