Objective To investigate the correlation between the appendicular skeletal mass index(ASMI)and os-teoporosis(OP)in postmenopausal patients with type 2 diabetes(T2DM).Methods 164 hospitalized postmeno-pausal elderly T2DM patients were selected and their bone density(BMD)and appendicular skeletal mass were measured using dual energy X-ray absorption method(DXA).ASMI=appendicular skeletal mass/height2(kg/m2),and they were divided into OP group and non OP group based on T value.The general clinical data,blood biochemical indicators,and ASMI between the two groups were compared,and Logistic regression analysis,ROC curve were further used to analyze the correlation and diagnostic power.Results Compared with non OP group,OP group had a higher incidence of sarcopenia(SAC)(P＜0.05);the differences in age,ASMI,body mass index(BMI),and estradiol(E2)had significant differences between the two groups(P＜0.05);Logistic regression analysis showed that the reduction of ASMI[OR=0.133,95％CI(0.029-0.611)],BMI[OR=0.785,95％CI(0.625-0.985)],and E2[OR=0.967,95％CI(0.942-0.993)]were protective factors for OP;receiver operating curve(ROC)suggested that the AUC of ASMI for OP prediction was 0.752[95％CI(0.632-0.872),P＜0.001]with sensitivity 87.5％,specificity 47.8％,and the best diagnostic value was 5.52 kg/m2.Conclusion The reduction of ASMI,BMI,and E2 is positively correlated with the occurrence of osteoporosis.ASMI is an important protective factor for osteoporosis.Early OP screening and risk factor assessment should be conducted for elderly postmenopausal T2DM patients,and early intervention measures should be taken to reduce the risk of falls and fractures.

Objective:To evaluate the efficacy and safety of ustekinumab (UST) for Crohn′s disease (CD) .Methods:A retrospective cohort study was conducted to collect clinical data of CD patients with active lesions in colonoscopy before the treatment of UST in Renji Hospital of Shanghai Jiaotong University School of Medicine from May 2020 to September 2021. Primary endpoint was the endoscopic response and remission rates at the 24th/32th week after the treatment of UST.Results:A total of 36 CD patients who were endoscopically active at baseline [25 men, 11 women; mean age, 29.8±8.7 years; disease duration, 38.0 (15.5, 66.1) months] were included. According to Montreal classification, 4 patients (11.1%) were L1 type (terminal ileum) , 4 (11.1%) were L2 type (colon) , 28 (77.8%) belonged to L3 type (ileocolon) , and upper digestive tract involvement occurred in 4 (11.1%) . As for disease behavior, 28 patients (77.8%) had non-structuring and non-penetrating lesions; 5 (13.9%) had structuring lesions and 3 (8.3%) had penetrating lesions. (1) Endoscopic activity: At the 24th/32th week, the endoscopic remission rate and response rate were 33.3% (12/36) and 63.9% (23/36) , respectively. There was no statistically significant difference in endoscopic remission rate and response rate between first-line and second-line usages of UST (all P>0.05) . (2) Clinical activity: Among the 36 patients, 16 were in the clinical active phase, and 20 patients were in the clinical remission phase at baseline. The clinical remission rate and clinical response rate of 16 clinical active patients at the 24th/32th week were 81.2% and 93.8% respectively. (3) Radiological activity: Twenty-seven patients completed the radiological evaluation at the 24th/32th week. In 3 patients with L1 lesions, 2 achieved response or partial response and no response in 1. In 24 patients with L3 lesions, radiological response occurred in 5 patients (20.8%) , partial response in 19 (79.2%) , and no response in 5 (20.8%) . In 19 patients with active perianal fistula at baseline, 6 achieved healing fistule at the 24th/32th week, 2 had partial response, 6 remained stable, while progress were seen in the other 5. (4) Serological and nutritive index: Compared with baseline values, the body mass index, hemoglobin and serum albumin levels of patients were significantly improved at the 24th/32th week (all P<0.05) , but the level of C-reactive protein and erythrocyte sedimentation rate at the 24th/32th week showed no significant difference (all P>0.05) . (5) Safety: No serious adverse events and infusion reactions were observed, and adverse events occurred in 2 patients. Conclusion:UST can effectively improve the endoscopic manifestations, clinical symptoms, imaging and nutritive index of CD patients with good safety.

Objective:To evaluate the efficacy and safety of ustekinumab (UST) for Crohn′s disease (CD) .Methods:A retrospective cohort study was conducted to collect clinical data of CD patients with active lesions in colonoscopy before the treatment of UST in Renji Hospital of Shanghai Jiaotong University School of Medicine from May 2020 to September 2021. Primary endpoint was the endoscopic response and remission rates at the 24th/32th week after the treatment of UST.Results:A total of 36 CD patients who were endoscopically active at baseline [25 men, 11 women; mean age, 29.8±8.7 years; disease duration, 38.0 (15.5, 66.1) months] were included. According to Montreal classification, 4 patients (11.1%) were L1 type (terminal ileum) , 4 (11.1%) were L2 type (colon) , 28 (77.8%) belonged to L3 type (ileocolon) , and upper digestive tract involvement occurred in 4 (11.1%) . As for disease behavior, 28 patients (77.8%) had non-structuring and non-penetrating lesions; 5 (13.9%) had structuring lesions and 3 (8.3%) had penetrating lesions. (1) Endoscopic activity: At the 24th/32th week, the endoscopic remission rate and response rate were 33.3% (12/36) and 63.9% (23/36) , respectively. There was no statistically significant difference in endoscopic remission rate and response rate between first-line and second-line usages of UST (all P>0.05) . (2) Clinical activity: Among the 36 patients, 16 were in the clinical active phase, and 20 patients were in the clinical remission phase at baseline. The clinical remission rate and clinical response rate of 16 clinical active patients at the 24th/32th week were 81.2% and 93.8% respectively. (3) Radiological activity: Twenty-seven patients completed the radiological evaluation at the 24th/32th week. In 3 patients with L1 lesions, 2 achieved response or partial response and no response in 1. In 24 patients with L3 lesions, radiological response occurred in 5 patients (20.8%) , partial response in 19 (79.2%) , and no response in 5 (20.8%) . In 19 patients with active perianal fistula at baseline, 6 achieved healing fistule at the 24th/32th week, 2 had partial response, 6 remained stable, while progress were seen in the other 5. (4) Serological and nutritive index: Compared with baseline values, the body mass index, hemoglobin and serum albumin levels of patients were significantly improved at the 24th/32th week (all P<0.05) , but the level of C-reactive protein and erythrocyte sedimentation rate at the 24th/32th week showed no significant difference (all P>0.05) . (5) Safety: No serious adverse events and infusion reactions were observed, and adverse events occurred in 2 patients. Conclusion:UST can effectively improve the endoscopic manifestations, clinical symptoms, imaging and nutritive index of CD patients with good safety.

Objective:To assess the short-term efficacy and safety of tacrolimus in patients with refractory Crohn′s disease (CD) , and analyze the influencing factors of clinical response.Methods:A single center restrospective cohort study was conducted. The clinical data of patients with refractory CD in Renji Hospital of Shanghai Jiaotong University School of Medicine from March 2014 to June 2019 were analyzed retrospectively. The patients received tacrolimus treatment for at least 3 months. Clinical response, clinical remission and relapse after tacrolimus treatment were evaluated by Crohn′s disease activity index (CDAI) . According to the existence of clinical response after 3 months of tacrolimus treatment, the patients were divided into clinical response group and non-clinical response group. The differences in clinical data between the 2 groups were assessed by univariate analysis. The variables with P<0.1 in univariate analysis and having clinical significance were further analyzed by multivariate Logistic regression to determine the independent risk factors of clinical response. The receiver operating characteristic (ROC) curve analysis was performed to evaluate the value of risk factors in predicting the clinical response of tacrolimus for the treatment of refractory CD. Results:A total of 45 patients with refractory CD were included, including 31 males and 14 females with the age of 32 (27, 39) years old. The disease duration was 61.0 (28.0, 97.5) months. The CDAI was 203 (175, 229) points before the treatment of tacrolimus while it decreased to 137 (117, 175) points after the treatment of tacrolimus for 3 months, and the difference was significant ( Z = -5.512, P<0.01) . After the treatment of tacrolimus for 3 months, 13 patients (28.9%) with clinical response were set as clinical response group and 32 (71.1%) without clinical response were set as non-clinical response group. Univariate analysis showed that the differences in gender, CDAI before the treatment of tacrolimus and neutrophil-to-lymphocyte ratio (NLR) between the clinical response group and non-clinical response group were statistically significant (all P<0.05) . Gender, CDAI before the treatment of tacrolimus and NLR were included for the multivariate Logistic regression analysis. The results showed that CDAI ( OR = 1.026, 95% CI: 1.006-1.046, P = 0.012) and NLR ( OR = 2.605, 95% CI: 1.290-5.258, P = 0.008) were the independent risk factors for predicting clinical response. The areas under ROC curve of CDAI, NLR and NLR combined with CDAI in predicting clinical response of tacrolimus in patients with refractory CD were 0.786 (95% CI : 0.648-0.924) , 0.764 (95% CI: 0.595-0.934) and 0.861 (95% CI : 0.729-0.992) , the optimal cut-off values were 189.15, 2.82 and 0.31, sensitivities were 100%, 84.6% and 84.6%, and specificities were 53.1%, 71.9% and 84.4%, respectively. Twenty-six patients continued to receive tacrolimus for 12 months, and the clinical remission rate was 73.1% (19/26) and the recurrence rate was 26.9% (7/26) . Forty-five patients were followed up for 1 year, adverse effects occurred in 6 and there was no severe adverse effects during the treatment of tacrolimus. Conclusions:Tacrolimus can be used as an immunosuppressant to induce the remission and maintenance in refractory CD patients. CDAI and NLR can be used as independent indicators to predict the clinical response of tacrolimus in the treatment of patients with refractory CD, and the combination of CDAI and NLR has higher prediction efficiency.

Objective:To investigate the familial inheritances, clinical features, treatments and outcomes of familial Waldenstrom macroglobulinemia (WM) patients.Methods:The clinical manifestations, laboratory examinations, diagnosis and treatments, and follow-up data of 6 familial WM patients who were admitted to Yancheng No.1 People's Hospital from June 2002 to July 2019 were retrospectively analyzed, and the literature was reviewed.Results:Among 6 WM patients, 4 patients had dizziness and fatigue at the onset, 1 patient had recurrent low-grade fever and abnormal sweating as the first manifestations, 1 patient was hospitalized due to pulmonary infection, and WM was found later. Two brothers of the patients were diagnosed with WM, another 2 brothers of the patients had IgM-type monoclonal gammopathy of undetermined significance (MGUS) during the physical examination. All the 6 patients were middle-aged/elderly men, with a median age of 63 years old (51-70 years old). The median follow-up time were 71.5 months (4-217 months), and by the end of the follow-up (June 2020), 2 cases died of pulmonary infection, and 1 of them developed acute myeloid leukemia; the other 4 cases were in regular chemotherapy. Two IgM-MGUS patients were followed up without symptoms.Conclusions:WM patients have familial aggregation, and their clinical manifestations are highly heterogeneous. Patients with family history may have poor prognosis. It is necessary to strengthen the awareness of WM and family history screening.

Objective:To assess the short-term efficacy and safety of tacrolimus in patients with refractory Crohn′s disease (CD) , and analyze the influencing factors of clinical response.Methods:A single center restrospective cohort study was conducted. The clinical data of patients with refractory CD in Renji Hospital of Shanghai Jiaotong University School of Medicine from March 2014 to June 2019 were analyzed retrospectively. The patients received tacrolimus treatment for at least 3 months. Clinical response, clinical remission and relapse after tacrolimus treatment were evaluated by Crohn′s disease activity index (CDAI) . According to the existence of clinical response after 3 months of tacrolimus treatment, the patients were divided into clinical response group and non-clinical response group. The differences in clinical data between the 2 groups were assessed by univariate analysis. The variables with P<0.1 in univariate analysis and having clinical significance were further analyzed by multivariate Logistic regression to determine the independent risk factors of clinical response. The receiver operating characteristic (ROC) curve analysis was performed to evaluate the value of risk factors in predicting the clinical response of tacrolimus for the treatment of refractory CD. Results:A total of 45 patients with refractory CD were included, including 31 males and 14 females with the age of 32 (27, 39) years old. The disease duration was 61.0 (28.0, 97.5) months. The CDAI was 203 (175, 229) points before the treatment of tacrolimus while it decreased to 137 (117, 175) points after the treatment of tacrolimus for 3 months, and the difference was significant ( Z = -5.512, P<0.01) . After the treatment of tacrolimus for 3 months, 13 patients (28.9%) with clinical response were set as clinical response group and 32 (71.1%) without clinical response were set as non-clinical response group. Univariate analysis showed that the differences in gender, CDAI before the treatment of tacrolimus and neutrophil-to-lymphocyte ratio (NLR) between the clinical response group and non-clinical response group were statistically significant (all P<0.05) . Gender, CDAI before the treatment of tacrolimus and NLR were included for the multivariate Logistic regression analysis. The results showed that CDAI ( OR = 1.026, 95% CI: 1.006-1.046, P = 0.012) and NLR ( OR = 2.605, 95% CI: 1.290-5.258, P = 0.008) were the independent risk factors for predicting clinical response. The areas under ROC curve of CDAI, NLR and NLR combined with CDAI in predicting clinical response of tacrolimus in patients with refractory CD were 0.786 (95% CI : 0.648-0.924) , 0.764 (95% CI: 0.595-0.934) and 0.861 (95% CI : 0.729-0.992) , the optimal cut-off values were 189.15, 2.82 and 0.31, sensitivities were 100%, 84.6% and 84.6%, and specificities were 53.1%, 71.9% and 84.4%, respectively. Twenty-six patients continued to receive tacrolimus for 12 months, and the clinical remission rate was 73.1% (19/26) and the recurrence rate was 26.9% (7/26) . Forty-five patients were followed up for 1 year, adverse effects occurred in 6 and there was no severe adverse effects during the treatment of tacrolimus. Conclusions:Tacrolimus can be used as an immunosuppressant to induce the remission and maintenance in refractory CD patients. CDAI and NLR can be used as independent indicators to predict the clinical response of tacrolimus in the treatment of patients with refractory CD, and the combination of CDAI and NLR has higher prediction efficiency.

Background: Some of the active perianal fistulizing Crohn's disease (CD) patients achieving remission with infliximab (IFX) therapy would develop relapse of perianal fistula within weeks to years after discontinuation of IFX therapy. Aims: To assess the outcomes of patients with perianal fistulizing CD after discontinuation of IFX therapy and the risk factors for relapse of perianal fistula. Methods: The clinical data of patients with perianal fistulizing CD who received IFX therapy at Shanghai Renji Hospital between June 2013 and May 2019 and stopped IFX therapy after achieving complete or partial radiological remission were collected retrospectively and analyzed. Demographic data, clinical and imaging characteristics, as well as data of IFX treatment and relapse of perianal fistula were extracted. Kaplan-Meier analysis was performed to calculate the cumulative probabilities of perianal and luminal relapse, while Cox proportional hazards model was applied to identify the risk factors for relapse. Results: A total of 56 perianal fistulizing CD patients who had been treated with IFX and stopped IFX therapy were included. Of them 26 achieved complete radiological remission and 30 achieved partial radiological remission. The median follow-up time was 20.5 months. Twenty-one patients (37.5%) had relapse of perianal fistula. The cumulative probabilities of perianal relapse were 29.0%, 33.7% and 42.8% at 12, 24 and 60 months after IFX discontinuation, respectively; and the cumulative probabilities of luminal relapse were 21.7%, 31.2% and 56.4% at 12, 24 and 60 months after IFX discontinuation, respectively. Multivariate analysis showed that non-stricturing and non-penetrating type (HR=9.711, 95% CI: 1.210-77.939, P=0.032) and involvement of rectum (HR=3.034, 95% CI: 1.119-8.231, P=0.029) were independent risk factors for relapse of perianal fistula, while the frequency of using of IFX therapy was a protective factor (HR=0.885, 95% CI: 0.792-0.990, P=0.032). Conclusions: There is a high risk of relapse of perianal fistulizing CD after discontinuation of IFX therapy. Non-stricturing and non-penetrating type and rectal involvement are risk factors for relapse of perianal fistula, and increasing the frequencies of using IFX therapy is crucial for the maintenance of remission.

We present a case of 32-year-old male with profound mental retardation and autism spectrum disorder who had presented with seizures, rigidity and elevated creatine kinase and was initially diagnosed as neuroleptic malignant syndrome (NMS). The patient subsequently had a complicated clinical course, developing refractory status epilepticus, which lead to the eventual diagnosis of progressive encephalomyelitis with rigidity and myoclonus (PERM). We discuss the clinical similarities and differences between NMS and PERM, and highlight the need to consider alternative diagnoses when the clinical picture of NMS is atypical, particularly in this patient group where the history and clinical examination may be challenging.
Adult ; Autism Spectrum Disorder ; Autistic Disorder ; Creatine Kinase ; Diagnosis ; Encephalomyelitis* ; Humans ; Intellectual Disability ; Male ; Myoclonus* ; Neuroleptic Malignant Syndrome* ; Seizures ; Status Epilepticus

Objective:To evaluate the relationship between the phthalate ester exposure and the population obesity with Meta-analysis, and to provide a new idea for prevention and control of obesity. Methods:A comprehensive search was performed in English databases (Pubmed, Web of Science, The Cochrane Library, Elsevier Science Direct and OVID) and Chinese databases (Sinomed database, CNKI database, VIP database, Wanfang database).The studies about the relationship between phthalate ester exposure and the population obesity were retriveded.The Chinese and English studies were selected according to the inclusion criteria and exclusion criteria.Meta-analysis was performed using RevMan 5.3 software.Results:Six studies were finally obtained, involving 1259 samples.The Meta-analysis results showed that the monobutyl phthalate (MBP) level in urine of the obesity population was increased 4.1 times compared with the normal population (95%CI:1.43-6.76);while the combined effect values of dibutyl phthalate (DBP), di-2-ethylhexyl phthalate (DEHP) and diethyl phthalate (DEP) level in serum of the population in two groups were 1.17 (95%CI:0.64-1.69), 0.80 (95%CI:0.13-1.48), and 0.72 (95%CI:-0.19-1.63);the combined effect values of monoethylhexyl phthalate (MEHP) and monoethyl phthalate (MEP) levels in urine were 1.75 (95%CI:-0.45-3.96) and 2.75 (95%CI: 0.36-5.15);there were no significant differences (P>0.05).Conclusion:The elevated MBP levels in the urine may be a risk factor for obesity in the population, suggesting that MBP may contribute to obesity.