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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 | Characterization of gut microbiomes in nonalcoholic steatohepatitis (NASH) patients: A connection between endogenous alcohol and NASH显示文摘 | Lixin Zhu Susan S. Baker Chelsea Gill Wensheng Liu Razan Alkhouri Robert D. Baker Steven R. Gill | 2013 | Hepatology2013，，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： | 3 |
| 6 | 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 |
| 7 | 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 |
| 8 | 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 |
| 9 | Neutrophil to lym- phocyte ratio: a new marker for predicting steatohepatitis and fi- brosis in patients with nonalcoholic fatty liver disease 显示文摘 | Alkhouri N Morris-Stiff G Campbell C | 2012 | Liver Int2012，32，2： | 1 |
| 10 | Neutrophil to lym- phocyte ratio: a new marker for predicting steatohepatitis and fibrosis in patients with nonalcoholic fatty liver disease 显示文摘 | Alkhouri N Morris-Stiff G Campbell C | 2012 | Liver Intemation- al2012，32，2： | 1 |
| 11 | Retinol-binding protein 4: A promising circulat- ing marker of liver damage in pediatric nonalco- holic fatty liver disease显示文摘 | NOBILI V ALKHOURI N ALISI A | 2009 | Clin Gastroenterol Hepatol2009，7，5： | 1 |
| 12 | lnlracranial pressure monitoring in children with severe traumatic brain injury:national trau- ma data hank-based review of outcomes 显示文摘 | Alkhoury F Kyriakides TC | 2014 | JAMA Surg2014，149，6： | 1 |
| 13 | Ultrasono- graphic quantitative estimation of hepatic steatosis in children with NAFLD显示文摘 | Shann on A Alkhouri N Carte r-Kent C | 2011 | J Pediatr Gastroenterol Nutr2011，53，2： | 1 |
| 14 | Chronic ho?mocysteine exposure upregulates endothelial adhesion molecules and mediates leukocyte: endothelial cell interactions under flow conditions显示文摘 | Alkhoury K Parkin SM Homer- V anniasinkam S | 2011 | EurJ Vase Endovasc Surg2011，41，3： | 1 |
| 15 | ‘Normal’ vitalsigns belie occult hypoperfusion in geriatric traumapatients显示文摘 | Martin JT Alkhoury F O’Connor JA | 2010 | Am Surg2010，76，1： | 1 |
| 16 | 1RF- 1 and miRNA126 modulate VCAM- 1 expression in response to a high fat meal显示文摘 | SUN C ALKHOURY K WANG YI | 2012 | Circ Res2012，111，8： | 1 |
| 17 | Short bowel syndrome in children:current and potential therapies显示文摘 | Uko V Radhakrishnan K Alkhouri N | | 0，，03： | 1 |
| 18 | Lipotoxieity in nonalcoholic fat- ty liver disease: not all lipids are created equal显示文摘 | Alkhouri N Dixon LJ Feldstein AE | 2009 | Expert Rev Gas- troenterol Hepatol2009，3，4： | 1 |
| 19 | A prospective study of safety and satisfaction with same-day discharge after laparoscopic ap- pendectomy for acute appendicitis 显示文摘 | Alkhoury F Burnweit C Malvezzi L | 2012 | J Pediatr Surg2012，47，2： | 1 |
| 20 | Familial adenomatous poly-posis in children and adolescents显示文摘 | Alkhouri N Franciosi JP Mamula P | 2010 | J Pediatr Gastroenterol Nutr2010，51，6： | 1 |
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