Objective To realise the clinical features and to reduce the diagnostic errors of hepatolenticular degeneration patients with liver injure. Methods Clinical manifestations, results of las tests and admitting diagnosis of 23 hepatolenticular degeneration cases were reviewed. Results All cases showed the elevation of serum transami-nase and/or total bilirubin. 17 cases(73.9% ) had manifestation of liver malfunction while nervous system symptoms were merely found in 2 cases. The decreased ceruloplasmin and elevated 24 hour urinary cuprum were detected in 23 and 21 cases respectively. Positive K-F rings were observed in 17 of 23 patients. Misdiagnosis rate of admitting diagno-sis exceeded 43.5%. 2 cases died after treating with penicillamine. Conclusion Liver injure that is one of the com-mon clinical features results in the diagnostic errors, but the combination detection will improve the accuracy of hepa-tolenticular degeneration.

Objective To investigate the diagnostic value of capsule endoscopy (CE), CT enterography (CTE), ileocolonoscopy and small bowel follow through (SBFT) for small bowel Crohn's disease (CD). Methods Fifty-seven consecutive patients with CD underwent ileocolonoscopy, CTE, CE, and SBFT. It included the presence of the following symptoms and signs: abdominal pain, weight loss,diarrhea, fever and positive fecal occult blood test. The location and the characteristics of intestinal and extra-intestinal lesions detected by four techniquks were compared. The proportions of patients with positive findings using each examination were compared. Results Of the 57 patients, 50 underwent ileocolonoscopy, terminal ileum lesion was found in 33 patients (66. 00% ), the remaining 17 (34.0%)were normal; among 34 patients who had CTE, 29 of small bowel lesion were found (85. 29% ); CE were performed in 27 patients, due to prolonged gastric transit one time, the capsule did not reach the cecum in one patient during battery lifetime. CE showed small bowel lesion in 26 patients (96.30% ); SBF was performed in 39 patients and 26 of small bowel lesion were detected (66. 67% ). CE had the highest diagnostic yield for CD and ileocolonoscopy had the lowest, and there were statistically significant difference among the 4 examinations (P = 0. 006 ). The combinded positive rates of two methods were: CE + CTE 92. 86% (13/14), SBFT + CTE 90. 91% (20/22), CE + ileocolonoscopy 95. 65% (22/23), CE + SBFT100% (17/17), ileocolonoscopy + CTE 89. 66% ( 26/29 ), ileocolonoscopy + SBFT 77.78% ( 28/36 ), but there were no significant differences between each two examinations. Conclusion CE, CTE have a higher yield in depicting mild to moderate finding of CD than SBFT. CE is better for assessing early mucosal disease,whereas CTE is better for detecting transmural and extraluminal abnormalities. Most important, CE plus CTE may depict nonobstructive CD of the small bowel when conventional techniques such as ileocolonoscopy or SBFF have negative or inconclusive finding. CE provides us explanations for the symptoms of patients, decision to follow up or therapy.

Objective To study the effects of Interferon(IFN)in chronic hepatitis B patients genotype B and C.Methods 20 cases were genotype B ,23 cases were genotype C,all the patients were treated with 5 million units of IFN-a-lb,im,qod,for 12 months,viral markers,liver function and adverse drug reactions were observed.Results There was no statistically significant difference between B,C genotype in the negative conversion rate of HBV-DNA (60.0% ,39.1%),the negative conversion rate of HBeAg(42.9% ,30.8%),anti-HBe seroconversion rate(35.7% ,23.1 %)and the rate ALT normalization(85.0% ,73.9%)(t = 1.86,0.69,0.68 ,0.79,P ＞ 0.05).Conclusion The results suggested that therapeutic efficacy of IFN-α-lb was not significantly different between B,C genotypes.

ObjectiveTo investigate the variation of peripheral blood CD34+ cells during the hematopoietic stem cell mobilization,and its influence on the collecting timing and results.Methods Twenty-seven cases of peripheral blood hematopoietic stem cell mobilization and collection from April 2010 to December 2011 were analyzed,including 13 autologous cases mobilized with chemotherapy combined with granulocyte colony-stimulating factor (G-CSF,10 μg· kg-1 · d-1) and 14 cases of healthy donors mobilized with only G-SCF (7.5 μg · kg- 1 · d- 1 ).The number of peripheral blood CD34+ cells was counted,and its correlation with the yield of mononuclear cells (MNCs) and CD34+cells was analyzed.ResultsMNCs (5.84 ± 1.48) × 108/kg and CD34+ cells (3.93 ± 2.16) × 106/kg were obtained in healthy donors,and (6.58 ± 3.72) × 108/kg MNCs and (3.98 ± 3.06) × 106/kg CD34+ cells were obtained in autologous cases,respectively.There was only 1 failure in autologous cases.The peak of peripheral blood CD34+ cells in autologous cases appeared at day 4 after the treatment of G-CSF,and in healthy donors the number of peripheral blood CD34+ cells at day 5 was still in ascendant phase.The CD34+ cells/kg in the collection products were positively correlated with the percentage and absolute value of peripheral blood CD34+ cells.The cases ratio of CD34+ cells≥2× 106/kg in the products of single collection was up to 76.2％ (16/21) in the cases with peripheral blood CD34+ cells absolute value greater than 20/μl.ConclusionThe number of peripheral blood CD34+ cells was an important monitoring indicator in hematopoietic stem cell mobilization and collection,CD34+ cell absolute value ≥20/μl could be used as collection threshold.

Objective:To investigate the changes of different cytokines in systemic juvenile idiopathic arthritis（SJIA）and their role in early diagnosis.Methods:A retrospective study was conducted to select pediatric patients with fever accompanied by elevated levels of CRP，erythrocyte sedimentation rate（ESR），and ferritin who visited the Department of Renal Immunology，Children's Hospital of Soochow University from November 2019 to January 2023.Compare the differences in CRP，ESR，ferritin，and 34 plasma cytokines levels between children with SJIA and other autoimmune diseases.Cytokine risk was analyzed by regression；Correlation analysis was performed to determine whether cytokines were associated with lymphocyte subsets and disease activity.Results:During the study period，118 children with fever accompanied by elevated CRP，ESR and ferritin were eligible，among whom 20 children with SJIA were diagnosed，and 98 children with other autoimmune diseases were diagnosed.There was no statistically significant difference in CRP and ESR between SJIA patients and children with other autoimmune diseases（Z values were 0.721 and 0.345，all P>0.05）；There were significant differences in ferritin，IL-27，IL-17A，IL-31，IFN-γ，IL-18 between children with SJIA and those with other autoimmune diseases（Z values were 2.628，-2.052，-2.763，-2.135，4.067，4.419，all P<0.05）.Univariate Logistic regression analysis showed that IL-18（ OR=1.003，95% CI：1.002~1.004， P<0.001），IFN-γ（ OR=1.004，95% CI：1.001~1.007， P=0.004），IL-27（ OR=0.846，95% CI：0.716~0.999， P=0.049），IL-31（ OR=0.657，95% CI：0.451~0.959， P=0.028）were closely related to the occurrence of SJIA，and stepwise Logistic regression analysis indicated that the increase of IL-18（ OR=1.005，95% CI：1.003~1.009， P=0.004）increased the risk of SJIA，and the increase of IL-5（ OR=0.619，95% CI：0.402~0.953， P=0.029）decreased the risk.IL-18（ r=0.673， P=0.020），IL-27（ r=0.486， P=0.041）and TNF-α（ r=0.560， P=0.016）were positively correlated with the activity of SJIA. Conclusion:IL-18 presents a characteristic cytokine positively correlated with the risk of SJIA，while IL-5 presents a protective cytokine against SJIA.Both cytokines have independent predictive power for the risk.

Objective To explore proprioceptive changes in patients with knee osteoarthritis(KOA)before and after total knee arthroplasty(TKA).Methods Thirty-four KOA patients were selected as the experimental group and divided into posterior-cruciate-retaining TKA(CR-TKA)and posterior-stabilized TKA(PS-TKA)groups according to the surgical method and followed up for three months after the surgery.Twenty healthy individuals were included as the control group.The proprioception(position sense,kinesthesia,and force sense)of healthy individuals and KOA patients before and after surgery was assessed using the Biodex system III isokinetic training system,self-designed force sense test equipment,and surface electromyography test system,and the data were processed and analyzed.Results Compared with healthy individuals,KOA patients had significantly worse position sense at 30°,45°,and 60°,kinesthesia,and semitendinosus force sense in the affected and unaffected knees(P<0.05).Three months after surgery,there were significant differences in the force sense of the affected biceps femoris and contralateral semitendinosus forces in the CR-TKA group compared with healthy individuals(P<0.05).There were no statistically significant differences in deviation for preoperative and 3-month preoperative position sense,kinesthetic sense,and force sense on the affected and contralateral knee joints between the CR-TKA and PS-TKA groups(P>0.05).Conclusions Knee proprioception in KOA patients was significantly impaired compared with that in healthy individuals.No significant improvement in proprioception was found three months after TKA in the CR-TKA and PS-TKA groups.There was no difference in proprioception among the different surgical methods.The results can provide data support for clinical diagnosis and treatment,as well as determine a direction for subsequent rehabilitation programs.

Objective: To evaluate efficacy of the BiRd regimen, a combination of clarithromycin, lenalidomide, and dexamethasone, in the treatment of patients with relapsed/refractory multiple myeloma (RRMM) . Methods: Patients with RRMM treated with BiRd between September 11, 2013 and August 1, 2016 at six centers were included to evaluate overall survival rate (ORR) , clinical benefit rate (CBR) , progression-free survival (PFS) , overall survival (OS) , as well as adverse events. Results: Of 30 patients with RRMM, 27 patients were evaluable, and ORR and CBR were 51.9% (14/27) and 66.7% (18/27) respectively, including 1 sCR (3.7%) , 3 CR (11.1%) , 3 VGPR (11.1%) , and 7 PR (25.6%) . In 13 patients with prior Rd, ORR and CBR were 38.5% (5/13) and 61.5% (8/13) respectively, of which 5 patients with ≥MR carried high-risk cytogenetic[ (e.g.17p- or t (4;14) ] together with at least one of other adverse-prognostic cytogenetic (e.g.13q- and/or 1q21+) . In 24 patients with prior bortezomib-based therapy, ORR and CBR were 45.8 and 62.5%, respectively. With a median follow-up time of 14.9 (range 1.0-33.8) months, the median PFS and OS were 12.0 (95%CI 11.6-12.4) and 27.6 (95%CI 15.1-40.1) months, respectively. The BiRd regimen was well tolerated. Conclusion The BiRd regimen is an effective and safety protocol for RRMM, including those carrying high-risk cytogenetic markers.

Objective:To investigate the risk factors of dysplasia in patients with ulcerative colitis (UC) in China.Methods:From March 1st, 2012 to December 30th, 2013, a total of 154 UC patients were prospectively enrolled from the following 11 hospitals, Xijing Hospital of Digestive Diseases, Ruijin Hospital affiliated to Shanghai Jiao Tong University School of Medicine, Peking Union Medical College Hospital, Renji Hospital affiliated to Shanghai Jiao Tong University School of Medicine, Nanfang Hospital affiliated to Southern Medical University, China-Japan Friendship Hospital, The Second Hospital of Hebei Medical University, West China Hospital affiliated to Sichuan University, The Seventh Medical Center of PLA General Hospital, Zhongshan Hospital affiliated to Xiamen University, and the First Affiliated Hospital of Anhui Medical University. The patients were followed up till December 1st, 2017. All the UC patients underwent colon endoscopy and histopathological evaluation. T test and Chi-square test were used for statistical analysis. Cox proportional risk model was used for identifying the risk factors of dysplasia in UC patients. Results:Finally, 133 UC patients were enrolled, the age was (50.0±11.9) years, the diagnosis age was (35.5±11.6) years, the course of disease was (14.5±6.7) years, and the number of endoscopic examinations was (3.4±1.6) times. A total of 21 patients were detected with dysplasia. No patients were detected with colorectal cancer. The results of univariate analysis revealed that the diagnosis age (hazard ratio ( HR)=1.05, 95% confidence interval ( CI) 1.01 to 1.10, P=0.009) and extensive colitis ( HR=2.92, 95% CI 0.97 to 8.79, P=0.057) were factors with statistically significant difference. The results of multivariate analysis revealed that the old age at diagnosis ( HR=1.06, 95% CI 1.02 to 1.11, P=0.003) and extensive colitis ( HR=3.68, 95% CI 1.21 to 11.19, P=0.022) were independent risk factors of dysplasia in UC patients. The cumulative incidence of dysplasia of UC patients with extensive colitis was higher than that of patients with left-sided colitis (24.3%, 17/70 vs. 6.3%, 4/63), and the difference was statistically significant ( χ2=8.023, P=0.005). Conclusions:Extensive colitis and older age at diagnosis are two independent risk factors of dysplasia in UC patients of our country. The cancer monitoring should be strengthened in UC patients with long course of disease and extensive colitis.

Objective:To investigate the radiation dosimetry and biodistribution of 68Ga-FAPI-04 PET/CT in patients with hepatobiliary tumor. Methods:A total of six patients with hepatic lesions who underwent PET/CT examination in Peking Union Medical College Hospital were enrolled. After intravenous injection of radiotracer 68Ga-FAPI-04 at (170.57 ± 14.43) MBq, whole-body imaging were performed at the time points of 3, 10, 15, 20, 30 and 60 min, respectively. Biodistribution pattern was observed. Regions of interest were manually delineated. Radiation dosimetry of all target organs were calculated by Olinda/EXM software. Results:The radioactive uptake dissipated gradually in liver whereas it was relatively stable in tumor lesions. The average SUV max of tumor lesions reached the maximum value (13.87± 2.55) at 20 min after injection. The target-to-background ratio increased with time, reaching the maximum value (10.09 ± 8.17) at 30 min after injection. The average effective dose in total body was (0.020 ± 0.002) mSv/MBq and organ with the highest effective dose was bladder wall at (0.146 ± 0.035) mSv/MBq. Conclusions:The effective dose in total body of 68Ga-FAPI-04 was similar to that of 18F-FDG. 68Ga-FAPI-04 is expected to be a PET/CT radiotracer for hepatobiliary tumors in consideration of rapid tumor uptake, low accumulation of liver background, and no influence of blood sugar levels.

Objective To explore the proprioception characteristics of knee joints for knee osteoarthritis (KOA) patients before unicondylar knee arthroplasty (UKA) and total knee arthroplasty (TKA). Methods Twenty-nine single-compartment KOA patients were selected. Fifteen patients were treated with UKA (UKA group) and fourteen patients were treated with TKA (TKA group). The test was performed 1 to 3 days before the operation.The keen society scores (KSS) of KOA patients in UKA group and TKA group were compared, and their joint position sense and kinesthesia were compared with control group. Results Significant differences in KSS were found in TKA group and UKA group, and the knee joint position sense and kinesthesia showed no significant differences between TKA group and UKA group.There were no significant differences in the knee joint position sense and kinesthesia between the operated leg and unoperated leg in TKA group and UKA group, and between the left leg and right leg in control group. Compared with control group, there were significant differences between UKA group and TKA group in 60° position sense of the operated leg and unoperated leg.The kinesthesia of the operated leg and unoperated leg in UKA group and TKA group were also significantly different from that in control group. Conclusions Compared with control group, the proprioception of the knee joint in KOA patients was significantly reduced before the surgery, but the characteristics of proprioception in UKA group and TKA group were similar.