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      Spleen and Liver Volumetrics as Surrogate Markers of Hepatic Venous Pressure Gradient in Patients With Noncirrhotic Portal Hypertension

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          Abstract

          Noncirrhotic portal hypertension (NCPH) is a rare disease that may lead to serious clinical consequences. Currently, noninvasive tools for the assessment of NCPH are absent. We investigated the utility of spleen and liver volumetrics as a marker of the presence and severity of portal hypertension in this population. A cohort of NCPH patients evaluated between 2003 and 2015 was retrospectively studied. The association of spleen and liver volumes with the hepatic venous pressure gradient (HVPG) level was evaluated using locally weighted scatterplot smoothing curves. A cohort of patients with viral hepatitis‐related liver disease was used as controls. Of the 86 patients with NCPH evaluated during the study period, 75 (mean age, 35 ± 17; 73% males) were included in the final analysis. Patients with portal hypertension had significantly higher spleen and liver to body mass index (BMI) ratios compared to patients with HVPG <5 mm Hg (39.5 ± 27.9 versus 22.8 ± 10.6 cm 3/kg/m 2, P = 0.003; 91.1 ± 40.1 versus 71.4 ± 16.7 cm 3/kg/m 2, P = 0.014, for spleen/BMI and liver/BMI, respectively). In contrast to the patients with viral hepatitis, a positive linear correlation was observed in the NCPH cohort between spleen/BMI and liver/BMI (above a cutoff of 25 and 80 cm 3/kg/m 2, respectively) and HVPG level. Additionally, only in the NCPH cohort was an increase in spleen/BMI range quartile predictive of a higher prevalence of portal hypertension and clinically significant portal hypertension (trend, P = 0.014 and 0.031, respectively). Conclusion: Spleen and liver volumetrics may have utility in the assessment of NCPH as a noninvasive biomarker that can be performed using routine radiologic examinations. Further studies are needed to validate these findings. ( Hepatology Communications 2018; 00:000‐000)

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          Most cited references20

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          Histological grading and staging of chronic hepatitis.

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            Beta-blockers to prevent gastroesophageal varices in patients with cirrhosis.

            Nonselective beta-adrenergic blockers decrease portal pressure and prevent variceal hemorrhage. Their effectiveness in preventing varices is unknown. We randomly assigned 213 patients with cirrhosis and portal hypertension (minimal hepatic venous pressure gradient [HVPG] of 6 mm Hg) to receive timolol, a nonselective beta-blocker (108 patients), or placebo (105 patients). The primary end point was the development of gastroesophageal varices or variceal hemorrhage. Endoscopy and HVPG measurements were repeated yearly. During a median follow-up of 54.9 months, the rate of the primary end point did not differ significantly between the timolol group and the placebo group (39 percent and 40 percent, respectively; P=0.89), nor were there significant differences in the rates of ascites, encephalopathy, liver transplantation, or death. Serious adverse events were more common among patients in the timolol group than among those in the placebo group (18 percent vs. 6 percent, P=0.006). Varices developed less frequently among patients with a baseline HVPG of less than 10 mm Hg and among those in whom the HVPG decreased by more than 10 percent at one year and more frequently among those in whom the HVPG increased by more than 10 percent at one year. Nonselective beta-blockers are ineffective in preventing varices in unselected patients with cirrhosis and portal hypertension and are associated with an increased number of adverse events. (ClinicalTrials.gov number, NCT00006398.) Copyright 2005 Massachusetts Medical Society.
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              Non-cirrhotic portal hypertension - diagnosis and management.

              NCPH is a heterogeneous group of liver disorders of vascular origin, leading to PHT with near normal HVPG. NCPF/IPH is a disorder of young adults or middle aged women, whereas EHPVO is a disorder of childhood. Early age acute or recurrent infections in an individual with thrombotic predisposition constitute the likely pathogenesis. Both disorders present with clinically significant PHT with preserved liver functions. Diagnosis is easy and can often be made clinically with support from imaging modalities. Management centers on control and prophylaxis of variceal bleeding. In EHPVO, there are additional concerns of growth faltering, portal biliopathy, MHE and parenchymal dysfunction. Surgical shunts are indicated in patients with failure of endotherapy, bleeding from sites not amenable to endotherapy, symptomatic hypersplenism or symptomatic biliopathy. Persistent growth failure, symptomatic and recurrent hepatic encephalopathy, impaired quality of life or massive splenomegaly that interferes with daily activities are other surgical indications. Rex-shunt or MLPVB is the recommended shunt for EHPVO, but needs proper pre-operative radiological assessment and surgical expertise. Both disorders have otherwise a fairly good prognosis, but need regular and careful surveillance. Hepatic schistosomiasis, CHF and NRH have similar presentation and comparable prognosis.
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                Author and article information

                Contributors
                ohad.etzion@nih.gov
                Christopher.Koh@nih.gov
                Journal
                Hepatol Commun
                Hepatol Commun
                10.1002/(ISSN)2471-254X
                HEP4
                Hepatology Communications
                John Wiley and Sons Inc. (Hoboken )
                2471-254X
                16 July 2018
                August 2018
                : 2
                : 8 ( doiID: 10.1002/hep4.v2.8 )
                : 919-928
                Affiliations
                [ 1 ] Liver Diseases Branch, National Institute of Diabetes and Digestive and Kidney Diseases National Institutes of Health Bethesda MD
                [ 2 ] Clinical Research Center Soroka University Medical Center Beer‐Shiva Israel
                [ 3 ] Biomedical and Metabolic Imaging Branch, National Institute of Diabetes and Digestive and Kidney Diseases National Institutes of Health Bethesda MD
                [ 4 ] Laboratory of Pathology, National Cancer Institute National Institutes of Health Bethesda MD
                [ 5 ] Department of Laboratory Medicine National Institutes of Health Clinical Center Bethesda MD
                [ 6 ] Medical Genetics Branch National Human Genome Research Institute Bethesda MD
                [ 7 ] Laboratory of Clinical Infectious Diseases, National Institute of Allergy and Infectious Diseases National Institutes of Health Bethesda MD
                [ 8 ] Mucosal Immunity Section, National Institute of Allergy and Infectious Diseases National Institutes of Health Bethesda MD
                [ 9 ] National Heart, Lung, and Blood Institute, Hematology Branch National Institutes of Health Bethesda MD
                [ 10 ] Center for Interventional Oncology National Institutes of Health Bethesda MD
                Author notes
                [*] [* ] ADDRESS CORRESPONDENCE AND REPRINT REQUESTS TO:

                Ohad Etzion, M.D.

                Liver Diseases Branch, National Institute of Diabetes and Digestive and Kidney Diseases National Institutes of Health

                10 Center Drive, Building 10, Room 9B16, MSC 1800

                Bethesda, MD 20892

                E‐mail: ohad.etzion@ 123456nih.gov

                Tel: +1‐301‐496‐1721

                Or

                Christopher Koh, M.D., M.H.Sc.

                Liver Diseases Branch, National Institute of Diabetes and Digestive and Kidney Diseases National Institutes of Health

                10 Center Drive, Building 10, Room 9B16, MSC 1800

                Bethesda, MD 20892

                E‐mail: Christopher.Koh@ 123456nih.gov

                Tel: +1‐301‐496‐1721

                Article
                HEP41198
                10.1002/hep4.1198
                6078215
                30094403
                f5aeaa07-3d2b-4a0f-80fa-b31296e2401e
                © 2018 The Authors. Hepatology Communications published by Wiley Periodicals, Inc., on behalf of the American Association for the Study of Liver Diseases.

                This is an open access article under the terms of the http://creativecommons.org/licenses/by-nc-nd/4.0/ License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non‐commercial and no modifications or adaptations are made.

                History
                : 07 November 2017
                : 12 March 2018
                : 18 April 2018
                Page count
                Figures: 4, Tables: 3, Pages: 10, Words: 5885
                Funding
                Funded by: Intramural Research Program of the National Institutes of Diabetes and Digestive and Kidney Diseases
                Funded by: National Cancer Institute
                Funded by: Clinical Center of the National Institutes of Health
                Funded by: National Institutes of Health
                Award ID: K99‐999999
                Categories
                Original Article
                Original Articles
                Custom metadata
                2.0
                hep41198
                August 2018
                Converter:WILEY_ML3GV2_TO_NLMPMC version:version=5.4.4 mode:remove_FC converted:06.08.2018

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