Castleman disease (CD) is a rare lymphoproliferative disease that is clinically classified into unicentric Castleman disease (UCD) and multicentric Castleman disease (MCD). According to the status of human herpes virus-8 (HHV-8) infection, MCD is further classified into HHV-8-positive MCD, and HHV-8-negative MCD, which is also called idiopathic multicentric Castleman disease (iMCD). There are standard treatment options for both UCD and HHV-8-positive MCD, but there has been no uniform standard of diagnosis and treatment for iMCD, which mainly relied on the experience of clinicians. In recent years, Castleman Disease Collaborative Network (CDCN) has newly defined the concept and diagnostic criteria of iMCD, and a comprehensive guidance on the treatment of iMCD was established in November 2018. In this paper, we try to review the current treatment options and advances of iMCD, which might help Chinese clinicians to diagnose and treat this rare disease.

Objective To summarize the clinical experience of successful liver transplantation from infant donation after cardiac death (DCD) for infant with biliary astresia (BA).Methods The donor was a 16-months-old girl with a body weight of 10 kg,who died of irreversible anoxic cerebral damage after sudden asphyxiation.The recipient was a 24-months-old girl with a body weight of 12 kg,who suffered from icteric concurrent late biliary cirrhosis after the Porta-jejunum anastomosis because of congenital BA.The DCD liver was classically orthotopically transplanted into the infants recipient.The warm ischemia time was 7 min,the cold ischemia time was 360 min,and the graft volume to the standard liver volume (GV/SLV) was 1.02.After operation,the vital signs and transplanted liver function of the recipient were monitored,and the recipient was given treatments of anti-infection,anticoagulation,and improving the microcirculation.The recipient was treated with the triple immunosuppression protocol of tacrolimus,mycophenolate and prednisone to prevent rejection.Results The operating time of the recipient was 480 min,the non-liver stage was 65 min,and the blood loss was 230 mL.The endotracheal intubation was removed from the recipient at 12 h,and the recipient started to eat at 48 h aftcr operation.The recipient had a hepatic artery thrombus on the 3rd and 15th day after operation,and the hepatic artery had re-blood-supply after the hepatic artery catheterization and continuous perfusion with urokinase.The recipient was discharged on the 42nd day,and the recipient was in satisfactory condition to present.Conclusion The infant DCD liver is a better graft for infant liver transplantation for BA.The surgical complications can be reduced with matched volume of donor-recipient liver; and it can guarantee a successful operation with perfect operative technique and careful perioperative management.

Objective:To determine the minimum clinically-important difference (MCID) in the rehabilitation effect among children with haemophilic knee joint contracture.Methods:The data describing 28 children with an average age of 13.89±3.00 years and haemophilic knee joint contracture who received no less than 10 sessions of physiotherapy in the Department of Rehabilitation Medicine at the Peking Union Medical College Hospital were analyzed. The therapeutic effect of the treatement was quantified in terms of Haemophilia Joint Health Scores (HJHSs) for their knees. The MCID after the therapy was evaluated using the mean change method, multivariate linear regression, receiver operating characteristics, and the distribution-based method.Results:The MCID for the improvement of knee HJHS was 5.13 by the mean change method, 4.31 by multivariate linear regression, 3.50 according to the ROC curve and 1.64 by the distribution-based method. Taking all of them into consideration, 4.31 was found to be an appropriate value.Conclusions:The MCID after physical therapy for the improvement in knee HJHS for a child with haemophilic knee contracture is 4.31. Improvements greater than 4.31 can be considered clinically significant.

Objective:To explore the chain mediating effect of social support and coping style between stigma and self-management behavior in diabetic foot patients.Methods:A total of 224 patients with diabetic foot who visited the Outpatient Department of Xinxiang Central Hospital were selected as participants by convenience sampling. Type 2 Diabetes Stigma Assessment Scale, Social Support Rating Scale, Medical Coping Modes Questionnaire and Diabetes Foot Self-Care Behavior Scale were used for survey. Model 6 in the SPSS PROCESS program was used to test for chain mediating effects.Results:Univariate analysis showed that there were statistically significant differences in self-management behavior scores among patients of different ages, educational levels, work status, average monthly family income, Wagner grading, and whether they received foot health education ( P<0.05). Correlation analysis showed that there was a significant correlation between stigma, social support, coping style, and self-management behavior, with statistical differences ( P<0.05). Mediating effect analysis showed that, in addition to the direct effect of stigma on the self-management behavior of diabetic foot patients, it also had an impact on self-management behavior through the separate mediating effect of social support and coping style (facing and resignation) and the chain mediating role of the two. Conclusions:Stigma can not only directly affect the self-management behavior of diabetic foot patients, but also indirectly affect them through the mediating role of social support and coping style (facing and resignation). The self-management of patients with diabetic foot can be improved by reducing their stigma, increasing social support and encouraging patients to be brave in coping with the disease.

Objective:To explore the level of physical activity of Chinese adults with haemophilia and its related factors so as to provide a reference for their comprehensive management.Methods:The daily physical activity of adults with haemophilia was surveyed using the International Physical Activity Questionnaire (IPAQ). A total of 110 questionnaires were eligible for analysis at last. The results were compared with published data from other societies using independent sample t-tests and chi squared tests. Spearman correlation analysis and multivariate logistic regression analysis were applied to explore factors related to the subjects′ physical activity levels.Results:Fully 30.9% of the subjects claimed to maintain a high level of physical activity. This was significantly different from the distribution of physical activity observed in Japan (12.4%), Ireland (50.9%) and the United Kingdom (43.1%). According to the Spearman correlation analysis, the number of joints affected, anxiety, and depression are all significantly related to the level of physical activity among such adults. Multivariate logistic regression analysis showed that depression was an independent factor affecting the patients′ level of physical activities.Conclusion:Chinese and Japanese adults with hemophilia have relatively low levels of physical activity, possibly influenced by depression.

Objective To compare the short-term clinical effects of unilateral dual-channel endoscopic discectomy(UBED)and percutaneous endoscopic intervertebral discectomy(PEID)in the treatment of L5S,Lumbar disc herniation,LDH.Methods From January 2019 to January 2021,a total of 57 cases of L5S,LDH treated by UBED or PEID were analyzed retrospectively,including 30 cases in UBED group and 27 cases in PEID group.The operation time,intraoperative fluoroscopy times,postoperative hospitalization days and surgical complications were compared between the two groups.The visual analogue scale(VAS)and oswestry disability index(ODI)were used to compare the postoperative quality of life of the two groups,and the modified MacNab criteria was used to evaluate the clinical efficacy in the last follow-up.Results The operation time of UBED group and PEID group was(75.30±8.44)minutes and(68.37±4.63)minutes,respectively,and the difference between the two groups was statistically significant(P＜0.05).VAS and ODI of 1 week,3 months,1 year and 1.5 years after surgery in 2 groups were significantly decreased compared with those before surgery,with statistical significance(P＜0.05).The VAS score of low back pain in UBED group was higher than that in PEID group[(3.87±1.14)points vs(2.70±0.67)points]at 1 week after surgery(P＜0.05),and there was no significant difference in VAS and ODI at the other time points(P＞0.05).There was no statistical difference in the results of modified MacNab criteria in the last follow-up(P＞0.05).There were 2 cases of dural tear in PEID group,1 case of dural tear in UBED group and 1 case of temporary nerve root injury in PEID group after operation,all of which were cured after symptomatic treatment.Conclusion Compared with PEID,UBED has a longer operation time,more trauma and more obvious low back pain in the short term after operation.The short-term curative effect of the two operations on L5S1 LDH is similar,the incidence of complications is low,and the times of fluoroscopy are few.Both operations are safe and effective.

Objective: To improve the understanding of rare anti-myelin-associated glycoprotein (MAG) positive IgM monoclonal gammopathy related peripheral neuropathy (IgM-PN) . Methods: Eleven cases of IgM paraproteinemia and anti-MAG antibody positive neuropathy diagnosed since 2014 in Peking Medical Union College Hospital were summarized. The medical records including clinical manifestation, lab results, treatment and prognosis were analyzed. Results: Among the 11 patients (8 male and 3 female) , the median onset age is 63 years old (range from 52 to 77 years old) . The peripheral neuropathy of 9 patients were characterized by distal onset of numbness, 6 patients suffered from muscle weakness. The nerve conduction velocity study indicated that all 11 patients had demyelinating peripheral nerve damage, which was sensory predominant and more severe in lower limbs, 6 of them had secondary axonal damage. Monoclonal IgM gammopathy was identified in all 11 patients, among which 6 were IgM κ, 2 IgG κ and IgM κ bi-clonal, 3 IgM λ. Three patients were diagnosed with Waldenström’s macroglobulinaemia. The anti-MAG-IgM antibody was positive in all 11 cases. After diagnosis, 9 patients received combination chemotherapy including rituximab or rituximab treatment alone. The monoclonal IgM level declined significantly in 7 patients. The neuropathy was stable or improved. Conclusions Anti-MAG antibody positive IgM-PN is a rare M protein related disease. In peripheral neuropathy with undetermined etiology, we suggest to screen M protein and anti-MAG antibody. Chemotherapy including rituximab or rituximab alone is recommended as first-line therapy.