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Prevalence and risk factors for impaired renal function among Asian patients with nonalcoholic fatty liver disease

Hepatobiliary & Pancreatic Diseases International 2024年第3期23卷 241-248页

作者： Chao Sun George Boon-Bee Goh Wan-Cheng Chow Wah-Kheong Chan Grace Lai-Hung Wong Wai-Kay Seto Yi-Hsiang Huang Han-Chieh Lin I-Cheng Lee Hye Won Lee Seung Up Kim Vincent Wai-Sun Wong Jian-Gao Fan Center for Fatty Liver Disease Department of GastroenterologyXinhua Hospital Affiliated to Shanghai Jiao Tong University School of MedicineShanghai Key Lab of Pediatric Gastroenterology and NutritionShanghai 200092China Department of Gastroenterology&hepatology Singapore General HospitalSingaporeSingapore Gastroenterology and hepatology Unit Department of MedicineFaculty of MedicineUniversity of MalayaKuala LumpurMalaysia Department of Medicine and Therapeutics and State Key Laboratory of Digestive Disease The Chinese University of Hong KongHong KongChina Department of Medicine The University of Hong KongHong KongChina Institute of Clinical Medicine National Yang Ming Chiao Tung University School of MedicineTaipeiTaiwanChina Division of Gastroenterology and hepatology Taipei Veterans General HospitalTaipeiTaiwanChina Faculty of Medicine National Yang Ming Chiao Tung University School of MedicineTaipeiTaiwanChina Department of Internal Medicine Yonsei University College of MedicineSeoulKorea

Background:Nonalcoholic fatty liver disease(NAFLD)is associated with impaired renal function,and both diseases often occur alongside other metabolic ***,the prevalence and risk factors for impaired renal function in patients with NAFLD remain *** objective of this study was to identify the prevalence and risk factors for renal impairment in NAFLD ***:All adults aged 18-70 years with ultrasound-diagnosed NAFLD and transient elastography examination from eight Asian centers were enrolled in this prospective *** fibrosis and cirrhosis were assessed by FibroScan-aspartate aminotransferase(FAST),Agile 3+and Agile 4 *** renal function and chronic kidney disease(CKD)were defined by an estimated glomerular filtration rate(eGFR)with value of<90 mL/min/1.73 m^(2) and<60 mL/min/1.73 m^(2),respectively,as estimated by the CKD-Epidemiology Collaboration(CKD-EPI)***:Among 529 included NAFLD patients,the prevalence rates of impaired renal function and CKD were 37.4%and 4.9%,*** multivariate analysis,a moderate-high risk of advanced liver fibrosis and cirrhosis according to Agile 3+and Agile 4 scores were independent risk factors for CKD(P<0.05).Furthermore,increased fasting plasma glucose(FPG)and blood pressure were significantly associated with impaired renal function after controlling for the other components of metabolic syndrome(P<0.05).Compared with patients with normoglycemia,those with prediabetes[FPG≥5.6 mmol/L or hemoglobin A1c(HbA1c)≥5.7%]were more likely to have impaired renal function(P<0.05).Conclusions:Agile 3+and Agile 4 are reliable for identifying NAFLD patients with high risk of *** glycemic control in the prediabetic stage might have a potential renoprotective role in these patients.

关键词： Nonalcoholic fatty liver disease Impaired renal function Agile 3+ Agile 4 Metabolic syndrome

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Vulvar Swelling in a 12-Year-Old Girl: An Early Manifestation of Crohn’s Disease

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Case Reports in Clinical Medicine 2024年第7期13卷 219-226页

作者： Stefania Rendina Dolores Ferrara Rosanna Mamone Sara Isoldi Massimo Zeccolini Francesco Esposito Department of Radiology Universit degli studi di Napoli Luigi Vanvitelli Naples Italy Department of Radiology A.O.R.N. Santobono-Pausilipon Children Hospital Naples Italy pediatric Gastroenterology and hepatology Unit Santobono-Pausilipon Childrens Hospital Naples Italy

Vulvar Crohn’s disease (VCD) is a rare complication of Crohn’s disease, especially in pediatric population. An early diagnosis can result difficult if the complication does not present in conjunction with the classic gastrointestinal symptoms that characterize this disease. In this study we present the case of a 12-year-old girl whose initial symptom of Crohn’s disease was a symptomatic vulvar swelling promoted by a rectovaginal fistula. We also provide an overview of Crohn’s disease and the vulvar changes found in the course of this disease.

关键词： Recto-Vaginal Fistula Vulvar Swelling Metastatic Crohn’s Disease

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Diseases of bile duct in children

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World Journal of Gastroenterology 2024年第9期30卷 1043-1072页

作者： Sutha Eiamkulbutr Chomchanat Tubjareon Anapat Sanpavat Teerasak Phewplung Nimmita Srisan Palittiya Sintusek Department of pediatrics King Chulalongkorn Memorial HospitalBangkok 10330Thailand Department of pediatrics Faculty of MedicineChulalongkorn University and King Chulalongkorn Memorial HospitalBangkok 10330Thailand Department of Pathology Chulalongkorn UniversityBangkok 10330Thailand Department of Radiology Chulalongkorn UniversityBangkok 10330Thailand Department of Surgery Faculty of MedicineChulalongkorn University and King Chulalongkorn Memorial HospitalBangkok 10330Thailand Center of Excellence in Thai pediatric Gastroenterology Hepatology and ImmunologyDivision of GastroenterologyDepartment of PediatricsKing Chulalongkorn Memorial HospitalChulalongkorn UniversityBangkok 10330Thailand

Several diseases originate from bile duct *** studies on these diseases,certain etiologies of some of them still cannot be *** most common disease of the bile duct in newborns is biliary atresia,whose prognosis varies according to the age of surgical *** diseases such as Alagille syndrome,inspissated bile duct syndrome,and choledochal cysts are also time-sensitive because they can cause severe liver damage due to *** majority of these diseases present with cholestatic jaundice in the newborn or infant period,which is quite difficult to differentiate regarding clinical acumen and initial *** cholangiography is potentially necessary to make an accurate diagnosis,and further treatment will be performed synchronously or planned as findings *** article provides a concise review of bile duct diseases,with interesting cases.

关键词： Bile duct Cholestasis Biliary atresia Biliary hypoplasia Biliary imaging Inspissated bile syndrome Choledochal cyst

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Calcium/calcimimetic via calcium-sensing receptor ameliorates cholera toxin-induced secretory diarrhea in mice

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World Journal of Gastroenterology 2024年第3期30卷 268-279页

作者： Lie-Qi Tang Johnathan Fraebel Shi Jin Steven P Winesett Jane Harrell Wen-Han Chang Sam Xianjun Cheng Department of pediatrics University of FloridaGainesvilleFL 32610United States College of Medicine University of FloridaGainesvilleFL 32610United States Applied Physiology and Kinesiology University of FloridaGainesvilleFL 32610United States Brain Rehabilitation Research Center Malcom Randall VA Medical CenterGainesvilleFL 32610United States Department of Medicine Endocrine Research UnitVeterans Affairs Medical CenterUniversity of CaliforniaSan FranciscoSan FranciscoCA 94121United States Department of pediatric Gastroenterology Hepatologyand NutritionUniversity of Florida Shands Children’s HospitalGainesvilleFL 32608United States

BACKGROUND Enterotoxins produce diarrhea through direct epithelial action and indirectly by activating the enteric nervous ***-sensing receptor(CaSR)inhibits both *** latter has been well documented in vitro but not in *** hypothesis to be tested was that activating CaSR inhibits diarrhea in *** To determine whether CaSR agonists ameliorate secretory diarrhea evoked by cholera toxin(CTX)in *** CTX was given orally to C57BL/6 mice to induce *** and calci-mimetic R568 were used to activate *** maximize their local intestinal actions,calcium was administered luminally via oral rehydration solution(ORS),whereas R568 was applied serosally using an intraperitoneal *** verify that their actions resulted from the intestine,effects were also examined on Cre-lox intestine-specific CaSR *** outcome was measured biochemically by monitoring changes in fecal Cl-or clinically by assessing stool consistency and weight *** CTX induced secretory diarrhea,as evidenced by increases in fecal Cl-,stool consistency,and weight loss following CTX exposure,but did not alter CaSR,neither in content nor in ***,calcium and R568 were each able to ameliorate diarrhea when applied to diseased *** CaSR involvement is suggested by gene knockout experiments where the anti-diarrheal actions of R568 were lost in intestinal epithelial CaSR knockouts(villinCre/Casrflox/flox)and neuronal CaSR knockouts(nestinCre/Casrflox/flox).CONCLUSION Treatment of acute secretory diarrheas remains a global *** advances in diarrhea research,few have been made in the realm of diarrhea *** therapy has remained the standard of care,although it does not halt the losses of intestinal fluid and ions caused by *** is no cost-effective therapeutic for *** and other studies suggest that adding calcium to ORS or using calcimimetics to activate intestinal CaSR might

关键词： Cholera Enteric nervous system Secretory diarrhea Oral rehydration solution Calcium-sensing receptor Gene knockout

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Metabolic dysfunction-associated steatotic liver disease:A silent pandemic

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World Journal of hepatology 2024年第4期16卷 511-516页

作者： Arghya Samanta Moinak Sen Sarma Department of pediatric Gastroenterology Sanjay Gandhi Postgraduate Institute of Medical SciencesLucknow 226014India

The worldwide epidemiology of non-alcoholic fatty liver disease(NAFLD)is showing an upward trend,parallel to the rising trend of metabolic syndrome,owing to lifestyle *** pathogenesis of NAFLD has not been fully understood ***,NAFLD has emerged as a public health concern in the field of hepatology and metabolisms *** changes in the nomenclature from NAFLD to metabolic dysfunction-associated steatotic liver disease have brought a positive outlook changes in the understanding of the disease process and doctor-patient *** changes are the main treatment ***,clinical trial using drugs that target‘insulin resistance’which is the driving force behind NAFLD,have shown promising *** translational research is needed to better understand the underlying pathophysiological mechanism of NAFLD which may open newer avenues of therapeutic *** role of gut dysbiosis in etiopathogenesis and use of fecal microbiota modification in the treatment should be studied *** of this silent epidemic by spreading awareness and early intervention should be our priority.

关键词： Metabolic dysfunction Fatty liver Obesity Insulin resistance

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Fecal microbiota transplantation in the treatment of hepatic encephalopathy:A perspective

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World Journal of hepatology 2024年第5期16卷 678-683页

作者： Arghya Samanta Moinak Sen Sarma Department of pediatric Gastroenterology Sanjay Gandhi Postgraduate Institute of Medical SciencesLucknow 226014India

Due to its complex pathogenesis,treatment of hepatic encephalopathy(HE)continues to be a therapeutic *** late,gut microbiome has garnered much attention for its role in the pathogenesis of various gastrointestinal and liver diseases and its potential therapeutic *** evidence suggests that gut micro-biota plays a significant role in cerebral *** in the gut microbiota has been documented in patients with HE in a number of clinical and experimental *** on gut dysbiosis in patients with HE has opened newer therapeutic avenues in the form of probiotics,prebiotics and the latest fecal microbiota transplantation(FMT).Recent studies have shown that FMT is safe and could be effective in improving outcomes in advanced liver disease patients presenting with ***,questions over the appropriate dose,duration and route of administration for best treatment outcome remains unsettled.

关键词： Fecal microbiota Dysbiosis Hepatic encephalopathy Cirrhosis

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Hepatic pseudotumor:A diagnostic challenge

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World Journal of hepatology 2024年第5期16卷 667-670页

作者： Arghya Samanta Moinak Sen Sarma Department of pediatric Gastroenterology Sanjay Gandhi Postgraduate Institute of Medical SciencesLucknow 226014India

Hepatic pseudotumors are rare lesions of unknown origin,characterized by the proliferation of fibrous connective tissue and inflammatory cell *** mimic malignant lesions clinically,and radiologically,given their non-specific clinical and imaging *** pathophysiology of hepatic pseudotumor is incompletely understood and there are no standardized criteria for *** have been reported to develop in various organs in the body with the lung and liver being the most common *** pseudotumors develop in patients with underlying triggers of liver inflammation and injury,including infections,autoimmune liver diseases,bile duct injury,or *** pse-udotumors respond well to conservative treatment with antibiotics,and steroids and some may regress spontaneously,thus avoiding unnecessary *** condition is rewarding to *** is important to recognize pseudotumor as a distinct clinical entity and include it in the differential of liver masses with atypical imaging features.

关键词： Hepatic pseudotumor Infection Stroglyloides Hepatic resection

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Recent updates on progressive familial intrahepatic cholestasis types 1, 2 and 3: Outcome and therapeutic strategies

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World Journal of hepatology 2022年第1期14卷 98-118页

作者： Seema Alam Bikrant Bihari Lal Department of pediatric hepatology Institute of Liver and Biliary SciencesNew Delhi 110070India

Recent evidence points towards the role of genotype to understand the phenotype,predict the natural course and long term outcome of patients with progressive familial intrahepatic cholestasis(PFIC).Expanded role of the heterozygous transporter defects presenting late needs to be suspected and *** of pruritus,nutritional rehabilitation,prevention of fibrosis progression and liver transplantation(LT)in those with end stage liver disease form the crux of the *** in PFIC has its own unique issues like high rates of intractable diarrhoea,growth failure;steatohepatitis and graft failure in PFIC1 and antibody-mediated bile salt export pump deficiency in *** inhibiting apical sodium-dependent bile transporter and adenovirus-associated vector mediated gene therapy hold promise for future.

关键词： Genotype Biliary diversion Gene therapy Liver transplantation

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Glycogen storage diseases:An update

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World Journal of Gastroenterology 2023年第25期29卷 3932-3963页

作者： Ersin Gümüs Hasan Ozen Department of pediatric Gastroenterology Hepatology and NutritionHacettepe University Faculty of MedicineIhsan Dogramaci Children’s HospitalAnkara 06230Turkey

Glycogen storage diseases(GSDs),also referred to as glycogenoses,are inherited metabolic disorders of glycogen metabolism caused by deficiency of enzymes or transporters involved in the synthesis or degradation of glycogen leading to aberrant storage and/or *** overall estimated GSD incidence is 1 case per 20000-43000 live *** are over 20 types of GSD including the *** heterogeneous group of rare diseases represents inborn errors of carbohydrate metabolism and are classified based on the deficient enzyme and affected *** primarily affect liver or muscle or both as glycogen is particularly abundant in these ***,besides liver and skeletal muscle,depending on the affected enzyme and its expression in various tissues,multiorgan involvement including heart,kidney and/or brain may be *** GSDs share similar clinical features to some extent,there is a wide spectrum of clinical ***,the goal of treatment is to maintain glucose homeostasis by dietary management and the use of uncooked *** addition to nutritional interventions,pharmacological treatment,physical and supportive therapies,enzyme replacement therapy(ERT)and organ transplantation are other treatment approaches for both disease manifestations and longterm *** lack of a specific therapy for GSDs has prompted efforts to develop new treatment strategies like gene *** early diagnosis and aggressive treatment are related to better prognosis,physicians should be aware of these conditions and include GSDs in the differential diagnosis of patients with relevant manifestations including fasting hypoglycemia,hepatomegaly,hypertransaminasemia,hyperlipidemia,exercise intolerance,muscle cramps/pain,rhabdomyolysis,and muscle ***,we aim to provide a comprehensive review of *** review provides general characteristics of all types of GSDs with a focus on those with liver involvement.

关键词： Glycogen storage disease Liver Muscle Hypoglycemia

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Primary liver transplantation vs transplant after Kasai portoenterostomy in children with biliary atresia: A retrospective Brazilian single-center cohort

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World Journal of Transplantation 2024年第1期14卷 112-119页

作者： Melina Utz Melere Valberto Sanha Marco Farina Carolina Soares da Silva Luiza Nader Cristine Trein Angelica Maria Lucchese Cristina Ferreira Antonio Nocchi Kalil Flavia Heinz Feier Department of hepatology and Liver Transplantation Santa Casa de Porto AlegrePorto Alegre 90050170Brazil Department of Hepato-biliary-pancreatic Surgery and Liver Transplantation Irmandade Santa Casa de Misericórdia de Porto AlegrePorto Alegre 90020-090Brazil Department of Surgical Oncology Santa Rita Hospital/Santa Casa de Misericórdia de Porto AlegrePorto Alegre 90050-170Rio Grande do SulBrazil

BACKGROUND Biliary atresia(BA)is the most common indication for pediatric liver transplantation,although portoenterostomy is usually performed ***,due to the high failure rate of portoenterostomy,liver transplantation has been advocated as the primary procedure for patients with *** is still unclear if a previous portoenterostomy has a negative impact on liver transplantation *** To investigate the effect of prior portoenterostomy in infants un-dergoing liver transplantation for *** This was a retrospective cohort study of 42 pediatric patients with BA who underwent primary liver transplantation from 2013 to 2023 at a single tertiary center in *** with BA were divided into two groups:Those undergoing primary liver transplantation without portoenterostomy and those undergoing liver transplantation with prior *** variables were compared using the Student’s t-test or the Kruskal-Wallis test,and categorical variables were compared using theχ2 or Fisher’s exact test,as *** Cox regression analysis was performed to determine risk factors for portal vein *** and graft survival analyses were conducted with the Kaplan–Meier product-limit estimator,and patient subgroups were compared using the two-sided log-rank *** Forty-two patients were included in the study(25[60%]girls),23 undergoing liver transplantation without prior portoenterostomy,and 19 undergoing liver transplantation with prior *** with prior portoenterostomy were older(12 vs 8 months;P=0.02)at the time of liver transplantation and had lower pediatric End-Stage Liver Disease scores(13.2 vs 21.4;P=0.01).The majority of the patients(35/42,83%)underwent livingdonor liver *** group of patients without prior portoenterostomy appeared to have a higher incidence of portal vein thrombosis(39 vs 11%),but this result did not reach statistical *** portoenterostomy

关键词： Hepatic portoenterostomy Biliary atresia Liver transplantation Patient outcome assessment Portal vein Survival

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