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Juvenile dermatomyositis (JDM) is a rare autoimmune disorder with multi-system involvement, often presenting with a heliotrope rash, Gottron's papules, and proximal muscle weakness. JDM patients with anti-nuclear matrix protein 2 (anti-NXP2) positivity tend to have more severe manifestations, including a higher risk of gastrointestinal (GI) complications such as dysphagia, intestinal motility changes, edema, malabsorption, ulcers, and perforations. These complications are associated with poor outcomes and high mortality rates, particularly in patients with anti-NXP2 positivity. A case is presented of a 12-year-old girl with JDM who developed multiple GI perforations after being treated with high-dose methylprednisolone. Despite multiple surgical attempts, the patient experienced continued leakage and new perforations. The treatment approach was shifted to include jejunostomy, plasma exchanges, fresh frozen plasma support, and tofacitinib, leading to gradual improvement in muscle strength and reduction in inflammation. GI involvement in JDM is a significant concern due to its association with poor prognosis and high mortality. The use of high-dose glucocorticoids must be carefully considered in JDM patients with GI involvement, as they may contribute to the development of perforations and complicate treatment. A combination of plasma exchange, fresh frozen plasma support, low-dose glucocorticoids, and Janus kinase inhibitors may offer a safer treatment strategy for managing refractory JDM with GI complications. The case highlights the importance of a multidisciplinary approach to treatment and the need for further research to determine the necessity of high-dose glucocorticoid therapy following GI involvement in JDM.
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Chronic obstructive pulmonary disease (COPD) is characterized by irreversible airflow obstruction and pathologically proven chronic inflammation of airway,pulmonary parenchyma and pulmonary blood vessels.It is divided into stable COPD and acute exacerbation of COPD (AECOPD).As a comprehensive intervention,pulmonary rehabilitation has remarkable therapeutic effect on stable COPD.At present,the effect of pulmonary rehabilitation of COPD has been widely confirmed.We will briefly review the research progress on pulmonary rehabilitation of AECOPD in recent years.
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Ventilator-associated pneumonia (VAP) is the most common hospital acquired infection,which increases the burden of patients significantly.Early diagnosis and effective prevention are important for VAP management.The guidelines for diaguosis,prevention and treatment of ventilator-associated pneumonia was issued by Chinese Society of Critical Care Medicine Chinese Medical Association in 2013.This article reviews the new progress of the diagnosis,treatment and prevention of VAP after the guideline was published.
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Objective To investigate the multi -slice CT angiography(MSCTA)findings and explore the clinical value of cavernous transformation of the portal vein(CTPV).Methods CT and clinical materials of 29 cases CTPV were retrospectively analyzed.Results Portal vein obstruction and surrounding fine dialated portoportal collat-erals were found in all 29 cases,Gastroesophageal varices and abnormal hepatic perfusion signs were detected in 25 patients and 8 cases respectively.Pericholedochal venous plexus and cystic vein were dilated and varicose in 9 cases. Conclusion Multi -slice CT has an important clinical role in CTPV diagnosis and treatment,and it can be used to evaluate the the portal vein obstructed status,the collateral vessels,and the accompanied complications of CTPV.
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Objective To explore the effect of transcatheter hepatic artery chemoembolization(TACE)between different types of liver cancer patients with portal vein tumor thrombus(PVTT).Methods 413 liver cancer patients with PVTT were divided into two groups,TACE(332 cases)group and control group(81 cases);To compare the survival rate and the curative effect of two group.Results Survival rate in the TACE group of 3 months,6 months,12 months,24 months were 93.4%,71.7%,44.9%,24.4%,in the control group 3 months,6 months,12 months,24 months survival rate were 24.7%,2.5%,0,0.The difference has statistical significance(P＜0.01).In the TACE group,type Ⅰ,Ⅱ,Ⅲ,Ⅳ of PVTT patients treatmented with TACE also has different survival rate and different curative effect(P＜0.01).Conclusion TACE treatment is particularly effective for type Ⅰ,type Ⅱ and type Ⅲ patients of PVTT.
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Objective To study the expression of toll rexeptor-4 in patients with systemic infanunatory response syndrome(SIRS)and multiple organ dysfunction syndrome (MODS) induced by acute cerebral vascular disease(ACVD).Method Totally 153 ACVD patients,admitted to Departments of Neurology Medicine and ICU of the First,the Second and the Forth Hospitals of Jilin University from October 2005 to March 2006,were enrolled in this study.The patients were admitted to the hospitals within 3 days after onset,and were confirmed by CT or MRI.The 153 patients in the prospective study was divided into three groupa:ACVD group(n=60).SIRS group induced by ACVD(n=45),MODS group induced by ACVD(n=48).The diagnosis criteria accorded with the criterion set by the Forth Conference of Cerebrovuscular Disease in China.The exclusion criterion inchided:①patients with infeetion or inflammatory disease two weeks before the onset of AVCD;②patients with cardiac disease,including acute myocarditis,acute myocardial infarction,angina episodes,degenerative calcific valvular disease,and so on;③patients with use of hormone recently;④malignant tumor,autoimmunity system disease,liver disease,kidney disease and hematologic disease.The expression of TLR-4 mRNA gene of the nucleated cell in the peripheral blood in the ACVD patient was measured by using RT-PCR.Statistics methods of t test,q test and simplex factor analysis of variance have been used Statistical analysis was carried out by using T-test,one-way analysis of variances,and q-test.Results The expression of TLR-4mRNA obviously increased on the third day after onset of AVCD.Compared with ACVD group,the expression of TLR-4mRNA in SIRS group was significantly higher (0.61±0.13 vs.0.98±0.15,P＜0.01).Compared with SIRS group,the expression of TLR-4mRNA in MODS group was significantly higher(0.98±0.15 vs.1.32±0.38,P＜0.01).The expression of TLR-4mRNA had a increasing tendency with the severity of AVCD.The expression of TLR-4mRNA was higher in patients with higher MODS score(P＜0.01).In the MODS group,the expression of TLR-4 mRNA was higher in the fatal patients than that in the survivors(1.56±0.16 vs.1.32±0.26,P＜0.01).Conclusions The mechanism of ACVD.which causes SIRS and MODS,may be associated with excessive immunologic respun of the organism.
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Objective:To clone human anti digoxin ScFv from a human semi synthetic phage antibody library.Methods:①Digoxin BSA conjugate(Dig BSA) was preparaed by a modified periodate oxidation method.②A semi synthetic phage antibody library was panned against immobilized Dig BSA.Collected clones were analysed by ELISA,inhibition ELISA and DNA Sequencing.Results:①During the four rounds panning against Dig BSA,the enrichment of the eluted phage particles was observed;②Analysis of the eluted clones identified one clone that could bind Dig as well as other digitalis.③Sequencing analysis showed that the variable genes of the positive clone belonged to VH5 and V?1 subgroup respectively.Conclusion:Human Dig specific ScFv that could bind Dig and other digitalis has been cloned from a semi synthetic phage antibody library which may provide a potential reagent for the diagnosis and therapy of Dig toxication.