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      Case Report: Hepatopulmonary syndrome as the first clinical manifestation of cirrhosis in a patient with underlying chronic lung disease

      case-report
      1 , a , 1
      F1000Research
      F1000 Research Limited
      Hepatopulmonary Syndrome, COPD, MAI Infection, Bronchiectasis

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          Abstract

          An 86 year old woman with multiple chronic lung diseases (including chronic obstructive pulmonary disease, bronchiectasis, and untreated mycobacterium avium-intracellulare) presented with two weeks of increased shortness of breath, notably worse when seated as compared to when lying down. After treatments focused on her known conditions did not resolve her dyspnea, the differential diagnosis was broadened and she was found to have evidence of cirrhosis on imaging. As a result of this new diagnosis, transthoracic echocardiography and arterial blood gas analysis were performed and together yielded the diagnosis of hepatopulmonary syndrome. We describe a rare presentation of hepatopulmonary syndrome manifesting as a patient’s first clinical evidence of suspected cirrhosis, a diagnosis made difficult by this patient’s numerous other lung diseases which muddied the picture.

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          Most cited references24

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          An official ATS/IDSA statement: diagnosis, treatment, and prevention of nontuberculous mycobacterial diseases.

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            Portopulmonary hypertension and hepatopulmonary syndrome.

            The clinically and pathophysiologically distinct entities of portopulmonary hypertension and hepatopulmonary syndrome occur in a substantial proportion of patients who have advanced liver disease of different causes. These disorders are notoriously underdiagnosed, but they have a substantial impact on survival and require focused treatment. Abnormal intrapulmonary vascular dilatation, the hallmark of hepatopulmonary syndrome, can cause profound hypoxaemia that can be very difficult to treat. By contrast, portopulmonary hypertension results from excessive pulmonary vasoconstriction and vascular remodelling that eventually leads to right-heart failure. Insights into the pathogeneses of these syndromes have led to novel therapeutic approaches. However, in severely affected patients, effective treatment remains a difficult task. In selected patients, liver transplantation represents the only treatment option, but the decision to do isolated liver transplantation is particularly challenging in patients who have severe pulmonary disease involvement. Data from several centres have contributed to provide criteria that allow improved prediction of which patients may, or may not, benefit from liver transplantation alone.
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              Mycobacterium avium complex pulmonary disease presenting as an isolated lingular or middle lobe pattern. The Lady Windermere syndrome.

              Pulmonary disease due to Mycobacterium avium complex (MAC-PD) radiographically resembles that due to tuberculosis; it preferentially affects elderly white men with predisposing pulmonary disorders (PDPD). Twenty-nine patients with MAC-PD were identified from a community-based population, and the medical records and chest roentgenograms (CRs) of six with a previously undescribed pattern of MAC-PD were reviewed. The distinctive clinical and demographic features of these six patients were identified and summarized. All were women who tended to be elderly. None had clinically evident PDPD. The dependent portion of the lingula or its counterpart, the middle lobe, was initially affected. Hilar adenopathy, volume loss, and cavitary disease were uniformly absent. To account for the distinctive features of this syndrome, we offer the hypothesis that habitual voluntary suppression of cough may have led to the development of nonspecific inflammatory processes in these poorly draining lung regions, upon which MAC-PD engrafted. We offer the term, Lady Windermere's syndrome, to describe this pattern among elderly women and to suggest that their fastidiousness may be its root cause.
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                Author and article information

                Contributors
                Role: ConceptualizationRole: InvestigationRole: Writing – Original Draft PreparationRole: Writing – Review & Editing
                Role: ConceptualizationRole: SupervisionRole: Writing – Review & Editing
                Journal
                F1000Res
                F1000Res
                F1000Research
                F1000Research
                F1000 Research Limited (London, UK )
                2046-1402
                16 May 2019
                2018
                : 7
                : 1175
                Affiliations
                [1 ]Internal Medicine, Montefiore Medical Center, Bronx, Bronx, NY, 10029, USA
                [1 ]Department of Gastroenterology, Medical School, University of Crete, Heraklion, Greece
                [1 ]Department of Pulmonary and Critical Care Medicine, Mercy Hospital, Joplin, MO, USA
                [1 ]Pneumology Service, Thorax Institute, Hospital Clinic-The August Pi i Sunyer Biomedical Research Institute (IDIBAPS)-Networked Centre for Biomedical Research on Respiratory Diseases (CIBERES) (Hospital Clínic-IDIBAPS-CIBERES), University of Barcelona, Barcelona, Spain
                Author notes

                No competing interests were disclosed.

                Competing interests: No competing interests were disclosed.

                Competing interests: No competing interests were disclosed.

                Competing interests: No competing interests were disclosed.

                Author information
                https://orcid.org/0000-0002-7983-5160
                Article
                10.12688/f1000research.15434.2
                6635985
                31354935
                cdfb6b5f-2ea8-4af5-bc8d-7497dd1cad91
                Copyright: © 2019 Murphy C and Arad D

                This is an open access article distributed under the terms of the Creative Commons Attribution Licence, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 8 May 2019
                Funding
                The author(s) declared that no grants were involved in supporting this work.
                Categories
                Case Report
                Articles

                hepatopulmonary syndrome,copd,mai infection,bronchiectasis

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