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Wilson disease(WD)is an inherited disorder caused by mutations in the ATP7B gene.It is characterized by pathological accumulation of copper in the organs throughout the body,especially the liver and brain. This article reviews the latest progress of existing and emerging therapies. At present,the most commonly used treatment methods are oral chelators of copper and zinc agents. Liver transplantation can be used as a treatment for end-stage patients. However,these treatment methods have some shortcomings:many side effects of drugs,poor treatment effect,lifelong treatment,poor patient compliance and so on. At present,the choice of drugs is still in the exploratory stage,and there are no guidelines to specify which patients should receive which treatment. In addition,there are many emerging therapies in different experimental stages,such as new chelators of copper,targeted molecular therapy,gene therapy and cell transplantation. The old methods have many disadvantages and lack large controlled clinical trials,and emerging research is still in its infancy,which are urgent questions.
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Coagulopathy in patients with liver disease is considered to be a fragile "rebalancing" state.Conventional coagulation test commonly used in clinic can not reflect the whole process of coagulation, so its application is limited.In recent years, thromboelastography has developed rapidly as a rapid and real-time detection method to evaluate the coagulation status of whole blood, and has unique advantages in the application of liver disease.The article reviews the application in transfusion guidance of thromboelastography and its association with severity of liver disease, bleeding, thrombosis, mortality and conventional coagulation testing.Although thromboelastography has some shortcomings, it can better reflect the real coagulation state of patients with liver disease and help reduce the use of blood products.Pediatric data are scarce, but the use of thromboelastography alone or in combination with conventional coagulation test will have broad prospects.
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Matrix metalloproteinases(MMPs)are a large family of zinc-dependent endopeptidases, which are mainly synthesized by connective tissues, it can degrade the extracellular matrix(ECM)and basement membrane, affect the regeneration and reconstruction of normal tissues, and participate in the pathological process of malignant tumors.Matrix metalloproteinase-7(MMP-7)is the smallest member of the metalloproteinase family.It is expressed in many tissues of the body, such as thyroid, breast, lung, digestive tract, reproductive system and hepatobiliary system.In recent years, the expression of MMP-7 in hepatobiliary diseases has attracted more and more attention.MMP-7 is not only involved in the growth, metastasis and invasion process of hepatobiliary malignant tumors, but also highly expressed in liver fibrosis, biliary atresia and other diseases.This paper reviews the expression of MMP-7 in the above diseases.
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Inflammatory bowel disease(IBD) is a complex multifactorial disease characterized by chronic recurrent intestinal inflammation.The etiology of IBD has not yet been determined, and relevant studies have focused on the genetic susceptibility, immune response, intestinal microbiome and environmental factors of the host.Studies have found that interleukin-10 receptor A(IL-10RA)plays important roles in the pathogenesis of IBD.IL-10RA can not only affect the intestinal barrier, but also affect the intestinal immune system.Furthermore, the mutation of IL-10RA itself is highly correlated with very early-onset IBD.
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Tumor-related diarrhea is a rare cause of chronic diarrhea in children, which is usually one of the clinical manifestations of specific functional syndrome of neuroendocrine neoplasms.Diarrhea may appear as the initial symptom or accompany symptom of disease progression, and it is generally relieved or cured after surgical resection.The present review mainly introduces several common neuroendocrine neoplasms which cause chronic diarrhea in children, and summarizes their different diarrhea features and mechanisms, as well as key points in diagnosis and treatment, in order to improve the rate of early diagnosis and prognosis of children.
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Inflammatory bowel disease is a chronic and nonspecific intestinal disease that is mainly treated with drugs.At present, Infliximab (IFX) is the only biological agent applied for children with inflammatory bowel disease in China.It is essentially a biosynthetic chimeric monoclonal antibody, and can be combined with tumor necrosis factor-alpha(TNF-α) on T cells inhibition of inflammatory response through cytotoxicity and induction of lymphocyte apoptosis.IFX can induce remission in the short term and maintain remission in the long term.It can also promote mucosal repair, improve the quality of life and reduce complications.Clinical studies revealed that there were a proportion of patients who respond poorly or ineffectively to IFX treatment.Furthermore, due to the high cost of biological agents, bio generic drugs are gradually starting to be developed and applied.This article provided an overview of the application of IFX on inflammatory bowel disease in children.
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Progressive familial intrahepatic cholestasis (PFIC) is a rare heterogenous groups of autosomal recessive monogenic disease, resulting in abnormal bile acid formation and secretion.According to the type of gene mutation, PFIC was classified as PFIC 1-6 and more new mutated genes have been identified, such as ABCC12, VPS33B, et al, making diagnosis and treatment more accurate.PFIC is characterized by progressive jaundice and pruritus, growth and development disorders, and fat-soluble vitamin deficiency.Except for PFIC3, the level of γ-glutamyl transpeptidase in serum of PFIC is normal, which is an important clue for clinical diagnosis.However, each type of PFIC has its own characteristics such as the age of onset, disease severity, extrahepatic symptoms, and prognosis.With the progress of the disease, it eventually develops into fibrosis, cirrhosis and liver failure.Treatment includes nutrition, medication, partial external/internal biliary diversion, and liver transplantation.Recent studies on transporters and gene therapy have brought new hope to patients with PFIC.
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Acute pancreatitis in children is a common acute and critical disease in clinical practice,and its incidence is increasing.The motality of moderate severe acute pancreatitis or severe acute pancreatitis is up to 10%-15%.Proper nutrition support can reduce the infection rate and mortality and improve the disease outcome under the condition of high metabolism and negative nitrogen balance,especially early enteral nutrition can benefit the patients significantly.Proper nutrition support methods and approaches,proper enteral nutrition initiation timing,optimized enteral nutrition formulations and quantity are all the hot spots at present.
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Objective To investigate the effect of miR-7 on the migration ability of intestinal epithelial cells and its related mechanisms. Methods LS174T cells were transfected with miR-7 mimic and miR-7 inhib-itor respectively. The effect of miR-7 on cell migration ability was detected by transwell chamber. The changes of PI3K, p-Akt and Akt protein in each group were detected by Western Blot. Results Compared with the control group, miRNA mimic negative control group and miRNA inhibitor negative control group, the number of cells in the miR-7 mimic group through the tranwell chamber decreased, the cell migration rate decreased, and the expression of PI3K and p-Akt protein decreased at the same time, the difference was statistically signifi-cant (P<0. 05). In the miRNA inhibitor group, the number of cells in the tranwell chamber increased, the cell migration rate increased, and the expression of PI3K and p-Akt protein was increased at the same time, the difference was statistically significant (P<0. 05). Conclusion miR-7 can inhibit the migration of LS174T cells, which may be through the regulation of the PI3K/Akt signaling pathway.
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Objective To investigate the regulation of miR-7 on intestinal trefoil factor (TFF3) and its effect on the proliferation and migration of intestinal epithelial cells. Methods miR-7 mimics and miR-7 inhibitor were transfected into LS174T cells respectively. The experiment was divided into 5 groups,including blank control group,miR-7 mimic negative control group,miR-7 mimic group,miR-7 inhibitor negative con-trol group,miR-7 inhibitor group. MTT assay was used to detect cell proliferation. Cell scratch assay was used to detect the effect of miR-7 on cell migration. Western blot was used to detect the change of TFF3 protein in each group. Results Compared with the blank control group,the miR-7 mimic negative control group and the miR-7 inhibitor negative control group,the OD value of the miR-7 mimic group decreased significantly, the difference was statistically significant(P<0. 05); the cell scratch interval increased,the cell migration rate decreased,the difference was statistically significant( P<0. 05); and the TFF3 protein expression was accompanied(P<0. 05). The OD value of the miR-7 inhibitor group significantly increased,the difference was statistically significant(P<0. 05); the cell scratch gap decreased,the cell migration rate was enhanced, the difference was statistically significant( P <0. 05); and the expression of TFF3 protein increased ( P <0. 05). Conclusion miR-7 can regulate the expression of TFF3 and further inhibit the proliferation and migration of LS174T cells.
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0bjective To construct the program of diet for adults with crohn′s disease. Methods Based on evidence, two rounds of consultation with experts using Delphi method were conducted to construct the program of diet for adults with crohn′s disease. Results The diet program consisted of 5 first-level entries,12 second-1evel entries and 33 third-level entries.The response rate of both rounds' questionnaire were 100%;the authority coefficients were 0.808 and 0.811,respectively;the coordination coefficients ranged from 0.149 to 0.212. Conclusions The Evidence-based diet program for adult with Crohn′s disease was reliable, the experts who participated were representative, had consistent opinions on items of each level for the program of diet for adults with crohn′s disease. Experts also had high levels of enthusiasm and authority, and could provide some degree guidance for diet nursing.
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Inflammatory bowel disease ( IBD) is a group of chronic non-specific inflammatory bow-el diseases that requires lifelong treatment and long-term follow-up. In recent years,the incidence of IBD in children is increasing year by year, which seriously affects children's quality of life, growth and mental health. The diagnosis of IBD requires a comprehensive analysis of patients'history,lab biochemistry,radiolo-gy,endoscopy and histopathology examinations and so on. There are no reliable non-invasive tests and bio-markers for follow-up. Trefoil factor family 3 ( TFF3 ) , identified essencial in the repairment of intestinal inflammation and promoting mucosal regeneration, playsan important rolein the pathogenesis of IBD. We review the relationship between intestinal trefoil factor and IBD,and further to discuss the possibility whether intestinal trefoil factor can act as a serum marker to assess the activity of IBD.
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Objective To choose a reasonable non-drug treatment program for women with postpartum breast pain. Methods Based on an adequate assessment of the patients′ condition, the clinical questions were proposed and the references were searched in a series of databases, such as Cochrane Library, PubMed, Ovid, CINAHL, CNKI, Wanfang, Weipu, CBM. Results A preliminary search of 484 articles on cabbage therapy for postpartum breast pain was carried. Through rigorous preliminary screening and screening, 11 articles were finally included, including 2 systematic reviews, 2 randomized controlled trials and 7 quasi-experiment. Through the analysis of the inclusion literature, the data was extracted, and the evidence and summary evidence were strictly evaluated.According to the results of evidence, based on the patients′ condition and the wishes of the family, the cold and hot cabbage leaves were alternately applied to the breast of 10 postpartum women with breast engorgement, the breast distended pain were improved. Conclusions The method of evidence-based nursing can provide safe and effective treatment for postpartum women with breast engorgement.
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Objective Intestinal epithelial barrier damage is closely related to a variety of gastrointestinal disease,how to maintain its function effectively is the key to treat all these diseases.This research attempts to explore the protective effect and its mechanism of toll-like receptor 2 (Toll-like receptor,TLR2)on permeability of intestinal epithelial barrier by experiments in vitro,to lay a foundation for new treatment methods.Methods We cultured non-transfected Caco-2 cells,TLR2-deficiency Caco-2 cells,TLR2-overexpressed Caco-2 cells in normal control group until the 21 st d,then tested transepithelial electrical resistance(TEER) which reacts the permeability of epithelial barrier.We cultured 3 types of cells in inflammation group until the 19th d treated with 10 ng/ml IL-1 beta for 48 h,then tested TEER values at the 21st d.We treated 3 types of cells in inhibition group with PI3K/Akt pathway inhibitor for 1h befor IL-1 beta,then tested TEER values at the 21st d.Results TEER value of TLR2-deficiency Caco-2 cell monolayer significantly reduced (P < 0.01),whereas TEER value of TLR2-overexpressed Caco-2 monolayer raised,but without statistically significant.TLR2 can prevent IL-1 beta caused TEER decreasing (P < 0.01),but the effect disappeared after given PI3K/Akt pathway inhibitor.Conclusion TLR2 can regulate the permeability of intestinal epithelial barrier.In addition,TLR2 can protect permeability increasing caused by inflammation,this effect mediated by PI3 K/Akt pathway.
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Objective Inflammatory bowel disease is an important chronic gastrointestinal disease of childhood and adolescence.Intestinal mucosa barrier damage plays an important role in its pathogenesis.This study attempts to use IL-1β stimulating Caco-2 cell monolayer simulates inflammatory intestinal epithelial barrier in vitro,provides the basis for studying the pathogenesis and treatment of IBD.Methods Caco-2 cells were cultivated in vitro until the 21st day to simulate intestinal epithelial cell monolayer barrier.The cells of inflammation group one were disposed with IL-1β for 2 h since the 5th day and detected TEER every other day until the 21st day.The cells of inflammation group two were disposed with IL-1 β at the 18th day for 0,12,24,48,72h, and detected TEER respectively.Normal control group cells were cultured with common medium and detected TEER at the corresponding time point.Results The TEER of Caco-2 cells gradually increased from the 5th to 15th days,reached 600Ω·cm2 in the 15th day of a plateau until to the 21st day.Since the 5th day,the TEER of inflammation group one were all lower than normal group,and still to the 21st day < 500Ω·cm2.Inflammation group two shows the time dependence TEER gradually reduce,peaked at 48 hours,then a slight increase in 72 hours.Conclusion The Caco-2 cells cultured for 2 ~ 3 weeks can form intestinal epithelial monolayer barrier with polarity,then treated with IL-1 β can manufacture inflammatory intestinal epithelial barrier model in vitro.
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Objective To evaluate the efficacy and safety of bicyclol tablet on hepatic lesions caused by Epstein-Barr virus( EBV) infection in children.Methods A single-center controlled retrospective study was conducted in 121 children with hepatic lesions caused by EBV infection for evaluating safety,tolerability, and efficacy of treatment with bicyclol tablets or Glycyrrhizin capsules.Childer n in bicyclol group ( n=63 ) were treatedw ith bicyclol at blets and cotn rol group ( n =58 ) were treated with Glycyrrhizin capsules.The course of the treatment were both 8 weeeks for two groups.The level of the EBV load pretreatment and plas-ma aminotransferase,blood routine,urine routine pretreatment and 1 week,4 weeks and 8 weeks after treat-mentw ere analyzed er trospectively.Rse ults (1) The pal smaA LT level sigin ficantly decreased in theb icy-clol group compared with that in the contro l group(P<0.01), se pecai lly the levle after 8 weeks treatm ent. (2) Bicycol l was more effce tive in the bicyclol group than Glycyrrhizin capslu se in the control group( P<0.01).(3) Both grousp had no significantlya dvesr e events.Conclusion Bicyclol tablet can derc ease plas-ma aminotransferase level,espce ially ALT,inc hildren caused by EBV infection with better efficiency and safety.
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Objective To establish and evaluate intestinal epithelial barrier model using Caco-2 cell so as to play a foundation for next study of barrier permeability.Methods Caco-2 cells were cultured in vitro then seeded into Transwell cell culture inserts.The permeability of the intestinal epithelial barrier was detected by transepithelial electrical resistance(TEER)and lucifer yellow flux,and verified by transmission electron micro-scope.Different concentrations of PAF(0,50,100,and 200 nmol /L)were exposed for 24 hours to Caco-2 mono-layer when cultured 21 days.The tight junction was observed under transmission electron microscope.Assess-ment of ZO-1 protein localization and expression were detected by immunofluorescence and Western blot analy-sis.Results Cultured Caco-2 cell confluencd as monolayer with time passed.From 5th day,TEER increased, then reached 600Ω?cm2 at 15th day and lasted to 21 st day,there was little flux of lucifer yellow,transmission e-lectron microscopy also found cells differentiated better,had well-arranged villi and polarity alined as monolayer, forming completed tight junction which was the marker of intestinal epithelial barrier model in vitro.TEER de-creased and lucifer yellow flux increased in cells exposed to PAF.The permeability reached the peak when ex-posed to 100 nmol /L PAF(P <0.01 ),tight junction disrupted,ZO-1 protein expression downregulated,abnor-mal localization and distribution was assessed by immunofluorescence staining.Conclusion Cultured Caco-2 cells for 2-3w can be used to study intestinal epithelial barrier as a model in vitro.PAF increased intestinal epi-thelial permeability,which would correlate to the decreased protein expression and abnormal distribution of ZO-1.
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ObjectiveTo investigate the significances of clinical diagnostic approaches to biliary atresia (BA) and intrahepatic cholestasis (IHC) in children, and to improve clinicians′ understanding of BA and reduce the rates of missed diagnosis and false diagnosis. MethodsA total of 133 children diagnosed with cholestasis with persistent jaundice admitted to our department from July 2011 to June 2014 were divided into IHC group with 111 patients and BA group with 22 patients. The general clinical trial data were reviewed and analysed and the significances of clinical manifestations, laboratory examination, and imaging features for differential diagnosis of BA and IHC were evaluated. Comparison of continuous data between the two groups was made by t test and comparison of categorical data between the two groups was made by chi-square test. When the sample characteristics for chi-square test were not suitable, the comparison was made by Fisher′s test. ResultsSignificant differences in clinical manifestations of kaolin stools and enlarged and hardened liver and spleen were observed between the two groups (P<0.01). Total bilirubin (TB), direct bilirubin (DB), gamma GGT (γ-GT), alanine aminotransferase (ALT), and aspartate aminotransferase (AST) levels in BA group were significantly higher than those in IHC group (P<0.01). The specificity, positive predictive value, and positive likelihood ratio of γ-GT were the highest among them, while TB had the highest sensitivity, the highest negative predictive value, and the lowest negative likelihood ratio. The sensitivity and negative predictive value of TB, DB, and γ-GT in the parallel experiment reached 100%. The specificity and positive predictive value of them in the serial experiment were 98% and 88.9%, respectively. There were significant differences in the hepatic portal fibrous mass and gallbladder hypokinesia detected with ultrasonography of the liver, gallbladder, and spleen and in magnetic resonance cholangiopancreatography (MRCP) features between the two groups (P<001). The specificity and positive predictive values of them in serial experiment reached 100%. ConclusionKaolin stools, enlarged and hardened liver and spleen, TB, DB, γ-GT, ultrasonography, and MRCP of the liver and gallbladder are important indices to distinguish between BA and IHC and a combined analysis of them can improve the diagnostic accuracy.
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Objective To evaluate the protection of combined clostridium butyricum and bifidobac-terium living powders for antibiotic-associated diarrhea ( AAD ) with all kinds of infections in hospitalized children,and to compare the therapeutic effect with saccharomyces boulardii. Methods This study was a prospective,randomized case-control clinical trial which collected the data of the hospitalized children with all kinds of infections in Pediactric Department of Shengjing Hospital of China Medical University between May 2011 to May 2012. A total of 552 cases were enrolled and 480 cases completed the study. A total of 240 chil-dren were in experimental group,80 cases received combined clostridium butyricum and bifidobacterium liv-ing powders 840 mg per time,twice a day and the other 160 cases received saccharomyces boulardii 250 mg per time,twice a day,for one week; the control group took none of probiltics. Two groups received routine antibiotic therapy. Everyday′s defecate frequency was recorded, the traits of excrement according to bristol stool assessment scale were evaluated,the incidence of diarrhea and drug related adverse reactions were coun-ted. Results During the studied 7 days,the AAD incidence was 4. 2%(10/240) in experimental group and 20. 4%(49/240) in control group,there was significant difference between two groups. The risk of AAD in experimental group decreased 58. 5%. Compared to saccharomyces boulardii,combined clostridium butyricum and bifidobacterium living powders decreased 38. 2% (RR=0. 728, 95%CI 0. 257~0. 784, P=0. 009). Compared to control group,the average defecate frequency decreased in experimental group,diarrhea duration contracted,there was statistic difference between two groups ( P<0. 01 ) . No drug related adverse reactions happened during the trial. Conclusion Both combined clostridium butyricum and bifidobacterium living powders and saccharomyces boulardii could effectively reduce the risk of AAD in hospitalized children with bacterial infection,relieve diarrhea symptoms,short the duration of diarrhea,and did not find the adverse reac-tions. Combined clostridium butyricum and bifidobacterium living powders and saccharomyces boulardii had the same protective effect for AAD of northern China children.
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Objective To introduce the nursing care of severe asthma patients treated by sedatives and muscle relaxants combined with mechanical ventilation. Methods Nurses with more than 3 years of working experiences in respiratory intensive care unit participated in the nursing care,such as ventilation monitoring,sedative effect assessment and artificial airway management. Results All the 18 patients were treated successfully,and the duration of mechanical ventilation ranged from 6 to 192 hours. The indicators of circulation and arterial gas after weaning were improved significantly (P<0.01). Conclusion Proper nursing care and correct sedative effect assessment is an important point to ensure treatment efficacy and reduce complications for severe asthma patients treated by sedatives and muscle relaxants combined with mechanical ventilation.