Hepatic Medicine : Evidence and Research最新文献

{"title":"Secondary Sclerosing Cholangitis After SARS-CoV2: ICU Ketamine Use or Virus-Specific Biliary Tropism and Injury in the Context of Biliary Ischemia in Critically Ill Patients?","authors":"Alessandra Bartoli,&nbsp;Carmela Cursaro,&nbsp;Hajrie Seferi,&nbsp;Pietro Andreone","doi":"10.2147/HMER.S384220","DOIUrl":"https://doi.org/10.2147/HMER.S384220","url":null,"abstract":"<p><strong>Purpose: </strong>From the beginning of the Severe Acute Respiratory Syndrome CoronaVirus-2 (SARS-CoV2) pandemic, different cases of a cholangiopathy with features of secondary sclerosing cholangitis in critically ill patients (SSC-CIP) have been reported. Patients developing it are generally recovering from severe Coronavirus disease 19 (COVID-19) and required intensive care unit (ICU) admission and mechanical ventilation. Many of them have been administered with ketamine during their ICU stay. The pathogenesis of this novel disease is still debated, and, since prognosis is poor, efforts are needed in order to better understand it.</p><p><strong>Patients and methods: </strong>In this review, we focused our attention on COVID-19 SSC clinical, imaging, and histology findings in order to clarify the different pathogenetic options, particularly in regard of the ischemic-direct viral damage and ketamine-related theories, beginning with a recapitulation of SSC-CIP and ketamine-induced cholangiopathy in abusers. The research has been conducted using PubMed and Google Scholar databases. Key-words were \"Secondary Sclerosing Cholangiopathy\", \"SSC-CIP\", \"Secondary Sclerosing Cholangiopathy in critically ill patients\", \"Ketamine and cholangiopathy\", \"Ketamine abusers and liver disease\", \"Ketamine-related cholangiopathy\", \"SARS-CoV2 infection and liver disease\", \"post Covid-19 secondary sclerosing cholangitis\", \"Covid-19 cholangiopathy\".</p><p><strong>Results: </strong>Many authors, based on the clinical, histological, imaging, and prognostic features of the disease, have pointed out the similarities between post COVID-19 SSC and SSC-CIP; however, peculiar features in the former were not previously observed. Therefore, a direct viral cytopathic action and SARS-CoV2-related coagulopathy are considered the most likely causes. On the other hand, ketamine, with the available data, cannot be surely linked as the main determinant cause of cholangiopathy. Moreover, ketamine-induced cholangitis (KIC) presentation is different from post COVID-19 SSC. Its role as a cofactor precipitating the disease cannot be ruled out.</p><p><strong>Conclusion: </strong>Post COVID-19 SSC is a rare clinical entity following severe COVID-19 disease. The most accepted theory is that a sum of different insults determines the disease: biliary ischemia, direct viral damage, toxic bile, possibly worsened by ketamine and hyperinflammation due to the cytokine storm. Given the severe prognosis of the disease, with persistent cholangiopathy, organ failure, and orthotopic liver transplantation (OLT), further study on this novel clinical entity is needed.</p>","PeriodicalId":12917,"journal":{"name":"Hepatic Medicine : Evidence and Research","volume":"15 ","pages":"93-112"},"PeriodicalIF":2.1,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/ef/9f/hmer-15-93.PMC10404108.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9955815","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Thrombocytopenia (TCP), MELD Score, and Fibrosis Index (FI) Among Hospitalized Patients with Chronic Liver Disease (CLD) in Ma'abar City, Dhamar Governorate, Yemen: A Cross-Sectional Study.","authors":"Mohammed Haidar Hazaa Al-Dholae,&nbsp;Mohammed Kassim Salah,&nbsp;Omar Yahya Al-Ashmali,&nbsp;Abdul Salam Mohamed Al Mokdad,&nbsp;Mohammed Ali Al-Madwami","doi":"10.2147/HMER.S392011","DOIUrl":"https://doi.org/10.2147/HMER.S392011","url":null,"abstract":"<p><strong>Purpose: </strong>This study sought to assess the prevalence of thrombocytopenia (TCP), underlying aetiologies of chronic liver disease, and the grading and prognostic systems for chronic liver disease (CLD) using non-invasive biomarkers: the Fibrosis index and the Model for End-Stage Liver Disease-Na (MELD-Na) Score, respectively.</p><p><strong>Patients and methods: </strong>This was a 15-month multi-centric cross-sectional study of 105 patients with chronic liver disease (CLD). The study was conducted using Sept 2019 to Nov 2020 admission records of CLD patients from Ma'abar City in Dhamar Governorate, Yemen.</p><p><strong>Results: </strong>A total of 63 (60%) and 42 (40%) patients were identified as thrombocytopenic and non-thrombocytopenic, respectively. The means ± SD of the MELD score and FI were 19 ± 7.302 and 4.1 ± 1.06. TCP prevalence among leukopenic and non-leukopenic patients was 89.5% and 53.5%, respectively (P = 0.004). Likewise, the prevalence of traditional-ultrasonography-diagnosed cirrhotic patients needing liver transplantation (LT) was 82.3% versus 61.3% among corresponding non-cirrhotic patients (P = 0.000).</p><p><strong>Conclusion: </strong>The prevalence of TCP among the participants of this study was similar to the global rate. However, the prevalence of decompensation was much higher among CLD patients than that found elsewhere, highlighting a need to improve methods for the early diagnosis of CLD in Yemen. This study also identified problems with the diagnostic work-up for non-infectious aetiologies of CLD. The findings suggest the need to improve clinician awareness about effective diagnostic strategies for these aetiologies.</p>","PeriodicalId":12917,"journal":{"name":"Hepatic Medicine : Evidence and Research","volume":"15 ","pages":"43-50"},"PeriodicalIF":2.1,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/4a/a5/hmer-15-43.PMC10153436.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9767269","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The Accuracy of Ultrasound Controlled Attenuation Parameter in Diagnosing Hepatic Fat Content.","authors":"Sebastiana Atzori,&nbsp;Yasmin Pasha,&nbsp;James B Maurice,&nbsp;Simon D Taylor-Robinson,&nbsp;Louise Campbell,&nbsp;Adrian K P Lim","doi":"10.2147/HMER.S411619","DOIUrl":"https://doi.org/10.2147/HMER.S411619","url":null,"abstract":"<p><strong>Purpose: </strong>The Controlled Attenuation Parameter (CAP score) is based on ultrasonic properties of retropropagated radiofrequency signals acquired by Fibroscan^TM (Echosens, Paris, France). Since ultrasound propagation is influenced by the presence of fat, CAP score was developed to quantify steatosis. The aim of this study was to delineate the accuracy of CAP in diagnosing hepatic steatosis, compared to the gold standard of liver biopsy.</p><p><strong>Patients and methods: </strong>A total of 150 patients underwent same-day liver biopsy and measurement of hepatic steatosis with Fibroscan. Only examinations with 10 satisfactory measurements, and an inter-quartile range of less than 30% of the median liver stiffness values were included for data analysis. Histological staging was then correlated with median values and Spearman correlation calculated. P values of <0.05 were considered statistically significant.</p><p><strong>Results: </strong>For diagnosis of hepatic steatosis (HS), CAP could predict the steatosis S2 with AUROC 0.815 (95% CI 0.741-0.889), sensitivity (0.81) and specificity (0.73) when the optimal cut-off value was set at 288 dB/m. CAP detected histological grade S3 with AUROC 0.735 (95% CI 0.618-0.851), sensitivity (0.71) and specificity (0.74), with a cut-off value of 330 dB/m. The AUROC for steatosis grade S1 was 0.741 (95% CI 0.650-0.824), with a cut-off value of 263 dB/m with sensitivity 0.75 and specificity 0.70. Univariate analysis showed a correlation between CAP and diabetes (p 0.048).</p><p><strong>Conclusion: </strong>The performance of CAP to diagnose steatosis severity decreases as steatosis progresses. CAP is associated with diabetes but not other clinical factors and parameters of the metabolic syndrome.</p>","PeriodicalId":12917,"journal":{"name":"Hepatic Medicine : Evidence and Research","volume":"15 ","pages":"51-61"},"PeriodicalIF":2.1,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10263157/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9648134","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Mucinous Cystic Neoplasms of the Liver: Epidemiology, Diagnosis, and Management.","authors":"Jeffrey A Hutchens,&nbsp;Kevin J Lopez,&nbsp;Eugene P Ceppa","doi":"10.2147/HMER.S284842","DOIUrl":"https://doi.org/10.2147/HMER.S284842","url":null,"abstract":"<p><p>Mucinous cystic neoplasms (MCNs) are rare tumors of the liver, occasionally seen in the biliary tree. Epidemiologic data are limited by their indolence and recent changes to diagnostic criteria. They are considered premalignant lesions capable of invasive behavior. While their etiology remains unknown, their female predominance, age of onset, and hormonally responsive ovarian-type stroma suggest ectopic organogenesis during embryologic development. MCNs can typically be recognized on imaging; yet, invasiveness is often indeterminate, and percutaneous tissue biopsy has shown limited value. Therefore, complete excision is recommended for all lesions as focal malignant transformation and metastatic disease has been reported.</p>","PeriodicalId":12917,"journal":{"name":"Hepatic Medicine : Evidence and Research","volume":"15 ","pages":"33-41"},"PeriodicalIF":2.1,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/98/b0/hmer-15-33.PMC10066895.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9258282","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Factors Associated with in-Hospital Mortality in Malagasy Patients with Acute Decompensation of Liver Cirrhosis: A Retrospective Cohort.","authors":"Chantelli Iamblaudiot Razafindrazoto,&nbsp;Nitah Harivony Randriamifidy,&nbsp;Behoavy Mahafaly Ralaizanaka,&nbsp;Jean Tsitamita Andrianoelison,&nbsp;Haga Tsilavo Ravelomanantsoa,&nbsp;Mialitiana Rakotomaharo,&nbsp;Domoina Harivonjy Hasina Laingonirina,&nbsp;Sonny Maherison,&nbsp;Jolivet Auguste Rakotomalala,&nbsp;Anjaramalala Sitraka Rasolonjatovo,&nbsp;Andry Lalaina Rinà Rakotozafindrabe,&nbsp;Tovo Harimanana Rabenjanahary,&nbsp;Soloniaina Hélio Razafimahefa,&nbsp;Rado Manitrala Ramanampamonjy","doi":"10.2147/HMER.S401628","DOIUrl":"https://doi.org/10.2147/HMER.S401628","url":null,"abstract":"<p><strong>Background: </strong>Cirrhosis is a pathology responsible for a significant hospital morbidity and mortality. The objective of this study was to determine the factors associated with hospital mortality in a sample of Malagasy cirrhotics.</p><p><strong>Patients and methods: </strong>This was a retrospective cohort study from January 2018 to August 2020 conducted in the Hepato-Gastroenterology Unity, University Hospital Joseph Raseta Befelatanana, Antananarivo, Madagascar.</p><p><strong>Results: </strong>One hundred and eight patients were included. The mean age was 51.13±13.50 years with a sex ratio of 2.37. The etiology of cirrhosis was dominated by alcohol (44.44%), hepatitis B virus (24.07%) and hepatitis C virus (13.89%). Twenty-eight patients (25.93%) had died. Factors associated with in-hospital mortality were hepatic encephalopathy (OR: 14.16; 95% CI: 5.08-39.4; p: 0.000), renal failure (OR: 8.55; 95% CI: 2.03-39.9; p: 0.0034), gastrointestinal bleeding (OR: 3.25; 95% CI: 1.32-7.92; p: 0.0099), hyponatraemia <130mmol/L (OR: 3.34; 95% CI: 1.04-10.6; p=0.046), Child-Pugh C classification (OR: 0.19; 95% CI: 0.12-0.21; p: 0.000), and MELD-Na score >32 (OR: 27.5; 95% CI: 4.32-174.8; p: 0.004).</p><p><strong>Conclusion: </strong>The in-hospital mortality rate during acute decompensation of cirrhosis remains high in Madagascar. Hepatic encephalopathy, renal failure, GI bleeding and hyponatraemia are the main clinico-biological factors affecting in-hospital mortality. Early intervention on these modifiable factors is an important step to improve hospital outcomes. The natraemia, MELD score and MELD-Na score should be used in routine practice in Madagascar to identify patients with acute decompensation of cirrhosis at high risk of death.</p>","PeriodicalId":12917,"journal":{"name":"Hepatic Medicine : Evidence and Research","volume":"15 ","pages":"21-26"},"PeriodicalIF":2.1,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/9f/85/hmer-15-21.PMC10019520.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9153504","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Primary Biliary Cholangitis: Promising Emerging Innovative Therapies and Their Impact on GLOBE Scores.","authors":"Aalam Sohal,&nbsp;Kris V Kowdley","doi":"10.2147/HMER.S361077","DOIUrl":"https://doi.org/10.2147/HMER.S361077","url":null,"abstract":"<p><p>Primary biliary cholangitis (PBC), previously referred to as primary biliary cirrhosis, is an autoimmune disorder leading to the destruction of intra-hepatic bile ducts. If untreated, progressive bile duct damage and cholestasis can lead to ductopenia and result in cirrhosis. Ursodiol, the first drug approved for PBC, has changed the natural history of this disease and improved patient outcomes. Subsequently, several new prediction models incorporating a response to ursodiol were developed. These include the GLOBE score, which was shown to predict long-term outcomes in patients with PBC. In 2016, obeticholic acid (OCA) became the second drug to be approved by the FDA, predominantly based on improvement in alkaline phosphatase (ALP) levels. This trial has subsequently influenced the design of clinical trials. Several drugs are currently being evaluated as therapeutic options for PBC, with improvement in ALP being a main endpoint. In this review, we will discuss the impact of new therapies on GLOBE scores in patients with PBC.</p>","PeriodicalId":12917,"journal":{"name":"Hepatic Medicine : Evidence and Research","volume":"15 ","pages":"63-77"},"PeriodicalIF":2.1,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/13/b7/hmer-15-63.PMC10259525.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9686189","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Risk Factors for Hepatitis B Virus Infection in North Ethiopia: A Case-Control Study.","authors":"Desalegn Weldebrhan,&nbsp;Hailemariam Berhe,&nbsp;Yohannes Tesfay","doi":"10.2147/HMER.S407069","DOIUrl":"https://doi.org/10.2147/HMER.S407069","url":null,"abstract":"<p><strong>Background: </strong>Hepatitis B virus infection (HBV) is an important clinical and public health problem that contributes to liver-related public health morbidity and mortality. Although childhood vaccination was introduced in 1980, hospital admissions, morbidity and mortality rates from HBV infection increased in Ethiopia. Risk factors for HBV infection and associated complications generally vary from case to case. No epidemiological studies have identified the risk factors for HBV infection in northern Ethiopia. Therefore, this study aimed to identify risk factors for HBV infection in specialist and teaching hospitals in Ayder.</p><p><strong>Methods: </strong>From March 2019 to May 2019, an unmatched hospital-based case-control study has been carried out on a total of 213 patients [71 cases and 142 controls] in northern Ethiopia. Cases were selected sequentially and two consecutive controls were selected for each case by a simple random method. The data were collected using pretested questionnaires structured by the interviewer as part of a face-to-face interview. Data were entered in Epi Data version 3.1, exported and analyzed with SPSS version 22. Binary and multivariable logistic regression analyses were used. Statistical significance was given as P <0.05.</p><p><strong>Results: </strong>Multivariate logistic regression analysis revealed that patients with familial exposure to hepatitis (AOR 3.7, 95% CI: 1.5-9.01), prior traditional medical procedure (AOR 1.2, 95% CI: 1.08-3.4), any history of dental procedures (AOR 3.8, 95% CI: 1.8-9.01) were associated risk factors to hepatitis B virus infection, and awareness of sexually transmitted hepatitis B virus infection (AOR 0.084, 95% CI: 0.01-0.6) is less likely to be infected with hepatitis B virus infection.</p><p><strong>Conclusion: </strong>This study (findings) demonstrated that contact with a case of hepatitis in the family, history of dentist visits, prior traditional medical procedure, and lack of awareness of its transmission through sexual contact have been identified as independent risk factors for the development of hepatitis B virus infection.</p>","PeriodicalId":12917,"journal":{"name":"Hepatic Medicine : Evidence and Research","volume":"15 ","pages":"79-91"},"PeriodicalIF":2.1,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/07/72/hmer-15-79.PMC10363344.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9872795","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"COVID-19-Associated Liver Injury.","authors":"Daniel T Gildea,&nbsp;Stephanie M Woo,&nbsp;Corinne E O'Connor,&nbsp;Amol S Rangnekar","doi":"10.2147/HMER.S384108","DOIUrl":"https://doi.org/10.2147/HMER.S384108","url":null,"abstract":"<p><p>This review analyzes data regarding liver injury associated with COVID-19 infection. We discuss reported effects on the liver from both COVID-19 and COVID-19 treatment as well as pathophysiology, review the potential role of drug-induced liver injury as an etiology of COVID-19-associated liver injury, and touch on other reports of significant outcomes including COVID-19 cholangiopathy and autoimmune hepatitis. Finally, we review the implications of COVID-19 infection in liver transplant recipients.</p>","PeriodicalId":12917,"journal":{"name":"Hepatic Medicine : Evidence and Research","volume":"15 ","pages":"1-9"},"PeriodicalIF":2.1,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/15/3c/hmer-15-1.PMC9960793.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10802534","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"COVID-Associated Cast-Forming Cholangiopathy: A Commentary on Disease Mechanism, Treatment, and Prognosis.","authors":"Yara Sarkis,&nbsp;Nasir Saleem,&nbsp;Raj Vuppalanchi,&nbsp;Mark Gromski","doi":"10.2147/HMER.S384176","DOIUrl":"https://doi.org/10.2147/HMER.S384176","url":null,"abstract":"<p><p>The complete impact of COVID-19 infection continues to develop since the onset of the COVID-19 pandemic. COVID-19 cholangiopathy has been recently described in a subset of patients who recovered from severe COVID-19 infection. The most common phenotype of patients suffering from COVID-19 cholangiopathy had severe infection requiring a stay in the intensive care unit, mechanical ventilation and vasopressor medications. Patients with COVID-cholangiopathy present with severe and prolonged cholestatic liver injury. In cases where biliary cast formation is identified, we defined the entity as \"COVID-19 cast-forming cholangiopathy\". This subset of COVID-19 cholangiopathy is not well understood and there are no standardized diagnosis or management to this date. The reported clinical outcomes are variable, from resolution of symptoms and liver test abnormalities to liver transplant and death. In this commentary, we discuss the proposed pathophysiology, diagnosis, management, and prognosis of this disease.</p>","PeriodicalId":12917,"journal":{"name":"Hepatic Medicine : Evidence and Research","volume":"15 ","pages":"27-32"},"PeriodicalIF":2.1,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/0a/93/hmer-15-27.PMC10066716.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9247375","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Polycystic Liver Disease: Pathophysiology, Diagnosis and Treatment.","authors":"Luiz Fernando Norcia,&nbsp;Erika Mayumi Watanabe,&nbsp;Pedro Tadao Hamamoto Filho,&nbsp;Claudia Nishida Hasimoto,&nbsp;Leonardo Pelafsky,&nbsp;Walmar Kerche de Oliveira,&nbsp;Ligia Yukie Sassaki","doi":"10.2147/HMER.S377530","DOIUrl":"https://doi.org/10.2147/HMER.S377530","url":null,"abstract":"<p><p>Polycystic liver disease (PLD) is a clinical condition characterized by the presence of more than 10 cysts in the liver. It is a rare disease Of genetic etiology that presents as an isolated disease or associated with polycystic kidney disease. Ductal plate malformation, ciliary dysfunction, and changes in cell signaling are the main factors involved in its pathogenesis. Most patients with PLD are asymptomatic, but in 2-5% of cases the disease has disabling symptoms and a significant reduction in quality of life. The diagnosis is based on family history of hepatic and/or renal polycystic disease, clinical manifestations, patient age, and polycystic liver phenotype shown on imaging examinations. PLD treatment has evolved considerably in the last decades. Somatostatin analogues hold promise in controlling disease progression, but liver transplantation remains a unique curative treatment modality.</p>","PeriodicalId":12917,"journal":{"name":"Hepatic Medicine : Evidence and Research","volume":"14 ","pages":"135-161"},"PeriodicalIF":2.1,"publicationDate":"2022-09-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/bc/12/hmer-14-135.PMC9528914.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"33490332","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}