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| 1 | Liver transplantation for nonalcoholic fatty liver disease:New challenges and new opportunities显示文摘Nonalcoholic fatty liver disease(NAFLD)is becoming rapidly one of the most common indications for orthotopic liver transplantation in the world.Development of graft steatosis is a significant problem during the posttransplant course,which may happen as a recurrence of pre-existing disease or de novo NAFLD.There are different risk factors that might play a role in development of graft steatosis including post-transplant metabolic syndrome,immune-suppressive medications,genetics and others.There are few studies that assessed the effects of NAFLD on graft and patient survival;most of them were limited by the duration of follow up or by the number of patients.With this review article we will try to shed light on post-liver transplantation NAFLD,significance of the disease,how it develops,risk factors,clinical course and treatment options. | Mina Shaker Adam Tabbaa Mazen Albeldawi Naim Alkhouri | 2014 | World Journal of Gastroenterology2014，20，18： | 15 |
| 2 | Johanson-Blizzard syndrome with mild phenotypic features confirmed by UBR1 gene testing显示文摘Johanson-Blizzard syndrome (JBS) is a rare autosomal recessive condition associated with exocrine pancreatic insufficiency,and is characterized by hypoplastic nasal alae,mental retardation,sensorineural hearing loss,short stature,scalp defects,dental abnormalities and abnormal hair patterns. Growth hormone deficiency,hypopituitarism,and impaired glucagon secretion response to insulin-induced hypoglycemia have been reported. Congenital heart defects have also been described in this condition. Mental retardation is typically moderate to severe in patients with JBS; however,normal intelligence can occur. In the pancreas,there is a selective defect of acinar tissue,whereas the islets of Langerhans and ducts are preserved. Diabetes has been reported in older children,suggesting the progressive nature of pancreatic disease. The molecular basis of JBS has recently been mapped to chromosome 15q15-q21 with identified mutations in the UBR1 gene. We report the case of a 7-year-old female with pancreatic insufficiency and mild phenotypic features,in whom the diagnosis of JBS was established using recently described molecular testing for the UBR1 gene. | Naim Alkhouri Barbara Kaplan Marsha Kay Amy Shealy Carol Crowe Susanne Bauhuber Martin Zenker | 2008 | World Journal of Gastroenterology2008，14，44： | 4 |
| 3 | Alpha-1 antitrypsin deficiency and the risk of hepatocellular carcinoma in end-stage liver disease显示文摘AIM:To evaluate the association between alpha-1 antitrypsin deficiency(A1ATD) and hepatocellular carcinoma(HCC) in patients with end-stage liver disease(ESLD).METHODS:Patients with cirrhosis and ESLD referred to the Cleveland Clinic Foundation for liver transplantation between 2003 and 2014 were included in the study(N = 675). ESLD was defined as having histological features of cirrhosis and/or radiological evidence of cirrhosis in the context of portal hypertension(ascites,variceal bleeding,thrombocytopenia,or hepatic encephalopathy). A1 ATD was diagnosed using phenotype characterization(MZ or ZZ),liver biopsy detection of PAS-positive diastaseresistant(PAS+) globules,or both. Patients with other causes of liver diseases such as hepatitis C virus(HCV),alcoholic liver disease and non-alcoholic steatohepatitis(NASH) or NASH were also included in the study. HCC was diagnosed by using imaging modalities,biopsy findings,or explanted liver inspection. Follow-up time was defined as the number of years from the diagnosis of cirrhosis to the diagnosis of hepatocellular carcinoma,or from the diagnosis of cirrhosis to the last follow up visit. The rate of HCC was assessed using time-tointerval analysis for interval censored data.RESULTS:This study included 675 patients. 7% of subjects had A1ATD(n = 47). Out of all subjects who did not have A1 ATD,46% had HCV,17% had alcoholic liver disease,19% had NASH and 18% had another primary diagnosis. Of the 47 subjects with A1 ATD,15 had a primary diagnosis of A1ATD(PI*ZZ phenotype and PAS+ globules),8 had a PI*MZ phenotype alone,14 had PAS+ alone,and 10 had both the PI*MZ phenotype and PAS+. Median follow-up time was 3.4(25th,75 th percentiles:1,5.2) years. The overall rate of hepatocellular carcinoma in all subjects was 29%(n = 199). In the A1 ATD group,the incidence rate of HCC was 8.5% compared to 31% in the group of patients with other causes of cirrhosis(P = 0.001). Patients with ESLD due to A1 ATD had the lowest yearly cumulative rate of hepatocellular carcinoma at 0.88% per year compared to 2.7% for those with HCV cirrhosis,1.5% in patients with NASH and 0.9% in alcohol-induced liver disease(P < 0.001).CONCLUSION:Within this group of patients with ESLD,there was no significant association between A1 ATD and increased risk of HCC. | Clara Antoury Rocio Lopez Nizar Zein James K Stoller Naim Alkhouri | 2015 | World Journal of Hepatology2015，7，10： | 3 |
| 4 | Ultrasonographic Quantitative Estimation of Hepatic Steatosis in Children With NAFLD显示文摘 | Angela Shannon Naim Alkhouri Christine Carter-Kent Lidia Monti Rita Devito Rocio Lopez Ariel E. Feldstein Valerio Nobili | 2011 | Journal of Pediatric Gastroenterology and Nutrition2011，，2： | 3 |
| 5 | Ultrasonographic Quantitative Estimation of Hepatic Steatosis in Children With NAFLD显示文摘 | Angela Shannon Naim Alkhouri Christine Carter-Kent Lidia Monti Rita Devito Rocio Lopez Ariel E. Feldstein Valerio Nobili | 2011 | Journal of Pediatric Gastroenterology and Nutrition2011，，2： | 2 |
| 6 | The Inflamed Liver and Atherosclerosis: A Link Between Histologic Severity of Nonalcoholic Fatty Liver Disease and Increased Cardiovascular Risk显示文摘 | Naim Alkhouri Tarek Abu-Rajab Tamimi Lisa Yerian Rocio Lopez Nizar N. Zein Ariel E. Feldstein | 2010 | Digestive Diseases and Sciences2010，，9： | 2 |
| 7 | Ombitasvir/paritaprevir/ritonavir + dasabuvir +/-ribavirin in real world hepatitis C patients显示文摘BACKGROUND The hepatitis C virus(HCV) NS5A inhibitor ABT-267(ombitasvir, OBV), the HCV NS4/4A protease inhibitor ABT-450(paritaprevir, PTV), the CYP3A inhibitor ritonavir(r) and the non-nucleoside NS5B polymerase inhibitor ABT-333(dasabuvir, DSV)(OBV/PTV/r + DSV) with or without ribavirin(RBV) is a direct-acting antiviral regimen approved in the United States and other major countries for the treatment of HCV in genotype 1(GT1) infected patients. Patients with HCV who are considered 'hard-to-cure' have generally been excluded from registration trials due to rigorous study inclusion criteria, presence of comorbidities and previous treatment failures.AIM To investigate the efficacy of this regimen in HCV G1-infected patients historically excluded from clinical trials.METHODS Patients were ≥ 18 years old and chronically infected with HCV GT1(GT1a, GT1b or GT1a/1b). Patients were treatment-na?ve or previously failed a regimen including pegylated interferon/RBV +/-telaprevir, boceprevir, or simeprevir.One hundred patients were treated with the study drug regimen, which was administered for 12 or 24 wk +/-RBV according to GT1 subtype and presence/absence of cirrhosis. Patients were evaluated every 4 wk from treatment day 1 and at 4 and 12 wk after end-of-treatment.RESULTS Many of the patients studied had comorbidities(44.2% hypertensive, 33.7%obese, 20.2% cirrhotic) and 16% previously failed HCV treatment. Ninety-six patients completed study follow-up and 99% achieved 12-wk sustained virologic response. The majority(88.4%) of patients had undetectable HCV RNA by week 4. The most common adverse events were fatigue(12%), headache(10%),insomnia(9%) and diarrhea(8%); none led to treatment discontinuation. Physical and mental patient reported outcomes scores significantly improved after treatment. Almost all(98%) patients were treatment compliant.CONCLUSION In an all-comers HCV GT1 population, 12 or 24-wk of OBV/PTV/r + DSV +/-RBV is highly effective and tolerable and results in better mental and physical health following treatment. | Nicole Loo Eric Lawitz Naim Alkhouri Jennifer Wells Carmen Landaverde Angie Coste Rossalynn Salcido Michael Scott Fred Poordad | 2019 | World Journal of Gastroenterology2019，25，18： | 2 |
| 8 | Analysis of breath volatile organic compounds as a noninvasive tool to diagnose nonalcoholic fatty liver disease in children显示文摘 | Naim Alkhouri Frank Cikach Katharine Eng Jonathan Moses Nishaben Patel Chen Yan Ibrahim Hanouneh David Grove Rocio Lopez Raed Dweik | 2014 | European Journal of Gastroenterology & Hepatology2014，，1： | 1 |
| 9 | Characterization of patients with both alcoholic and nonalcoholic fatty liver disease in a large United States cohort显示文摘BACKGROUND Nonalcoholic fatty liver disease(NAFLD)is the hepatic manifestation of the metabolic syndrome(MetS)and is characterized by steatosis in the absence of significant alcohol consumption.However,MetS and significant alcohol intake coexist in certain individuals which may lead to the development of BAFLD.AIM To assess the clinical characteristics of patients with both alcoholic and NAFLD(BAFLD)in a large cohort in the United States.METHODS Adults from the National Health and Nutrition Examination Survey between 2003-2014 were included.NAFLD was diagnosed based on elevated alanine aminotransferase(ALT)and being overweight or obese in the absence of other liver diseases.BAFLD patients met the criteria for NAFLD but also had either MetS or type 2 diabetes and consumed excessive amounts of alcohol.Univariable and multivariable analysis were performed to assess differences between NAFLD and BAFLD and to compare severity based on a validated fibrosis score(FIB4 index).RESULTS The prevalence of NAFLD was at 25.9%(95%CI;25.1-26.8)and that of BAFLD was 0.84%(0.67,1.02)which corresponds to an estimated 1.24 million Americans affected by BAFLD.Compared to NAFLD,patients with BAFLD were more likely to be male,smokers,have higher ALT,aspartate aminotransferase,triglycerides,and lower platelets;P<0.01 for all.More importantly,after adjusting for MetS components,BAFLD patients were significantly more likely to have advanced fibrosis[adjusted OR(95%CI)based on FIB4 index>2.67 was 3.2(1.4,7.0),P=0.004].CONCLUSION A significant percentage of the American general population is afflicted by BAFLD and these patients tend to have more advanced liver fibrosis. | George Khoudari Amandeep Singh Mazen Noureddin Danielle Fritze Rocio Lopez Imad Asaad Eric Lawitz Fred Poordad Kris V Kowdley Naim Alkhouri | 2019 | World Journal of Hepatology2019，11，10： | 1 |
| 10 | A Combination of the Pediatric NAFLD Fibrosis Index and Enhanced Liver Fibrosis Test Identifies Children With Fibrosis显示文摘 | Naim Alkhouri Christine Carter–Kent Rocio Lopez William M. Rosenberg Massimo Pinzani Giorgio Bedogni Ariel E. Feldstein Valerio Nobili | 2011 | Clinical Gastroenterology and Hepatology2011，，2： | 1 |
| 11 | Novel therapeutic targets for nonalcoholic fatty liver disease显示文摘 | Akiko Eguchi Davide Povero Naim Alkhouri Ariel E Feldstein | 2013 | Expert Opinion on Therapeutic Targets2013，，7： | 1 |
| 12 | The Breathprints in Patients With Liver Disease Identify Novel Breath Biomarkers in Alcoholic Hepatitis显示文摘 | Ibrahim A. Hanouneh Nizar N. Zein Frank Cikach Luma Dababneh David Grove Naim Alkhouri Rocio Lopez Raed A. Dweik | 2014 | Clinical Gastroenterology and Hepatology2014，，3： | 1 |
| 13 | Hepatitis C treatment with triple therapy in a patient with hemophilia A显示文摘We report a case of successful treatment of chronic hepatitis C infection with telaprevir-based triple therapy in a patient with hemophilia A complicated by factor Ⅷ inhibitor. A twenty-two years old male with hereditary hemophilia A and high-titer factor Ⅷ inhibitor was taking maintenance doses of recombinant factor Ⅷ. He visited our clinic for treatment of his chronic hepatitis C with the newly instituted protease inhibitor based therapy. He was diagnosed with hepatitis C genotype 1a at one year of age. He was initiated on telaprevir, ribavirin and peg-interferon for treatment of hepatitis C and qualified for response-guided therapy. He completed treatment at 24 wk with minimal adverse effects. Notably, after 4 wk of hepatitis C treatment, his factor Ⅷ inhibitor screen was negative and the dose for recombinant factor Ⅷ decreased by half of the initial dosing before he was treated for hepatitis C. We suspect that suppressing hepatitis C may help decrease factor Ⅷ inhibitor level and the need for recombinant factor Ⅷ. | Gurshawn Singh Reuben Sass Rayan Alamiry Nizar Zein Naim Alkhouri | 2013 | World Journal of Clinical Cases2013，1，3： | 0 |
| 14 | Simple diagnostic algorithm identifying at-risk nonalcoholic fatty liver disease patients needing specialty referral within the United States显示文摘BACKGROUND There is an urgent need to risk stratify patients with suspected nonalcoholic fatty liver disease(NAFLD)and identify those with fibrotic nonalcoholic steatohepatitis.This study aims to apply a simple diagnostic algorithm to identify subjects with at-risk NAFLD in the general population.AIM To apply a simple diagnostic algorithm to identify subjects with at-risk NAFLD in the general population.METHODS Adult subjects were included from the National Health and Nutrition Examination Survey database(2017-2018)if they had elevated alanine aminotransferase(ALT)and excluded if they had evidence of viral hepatitis or significant alcohol consumption.A fibrosis-4(FIB4)cutoff of 1.3 differentiated patients with low risk vs high risk disease.If patients had FIB4>1.3,a FAST score<0.35 ruled out advanced fibrosis.Patients with FAST>0.35 were referred to a specialist.The same algorithm was applied to subjects with type 2 diabetes mellitus(T2DM).RESULTS Three thousand six hundred and sixty-nine patients were identified who met all inclusion and exclusion criteria.From this cohort,911(28.6%)patients had elevated ALT of which 236(22.9%)patients had elevated FIB4 scores≥1.3.Among patients with elevated FIB4 score,75(24.4%)had elevated FAST scores,ruling in advanced fibrosis.This accounts for 2.0%of the overall study population.Applying this algorithm to 737 patients with T2DM,213(35.4%)patients had elevated ALT,85(37.9%)had elevated FIB4,and 42(46.1%)had elevated FAST scores.This accounts for 5.7%of the population with T2DM.CONCLUSION The application of this algorithm to identify at-risk NAFLD patients in need for specialty care is feasible and demonstrates that the vast majority of patients do not need subspecialty referral for NAFLD. | Naim Alkhouri Pankaj Aggarwal Phuc Le Julia Payne Celine Sakkal Prido Polanco Stephen Harrison Mazen Noureddin | 2022 | World Journal of Hepatology2022，14，8： | 0 |
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