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https://www.readbyqxmd.com/read/28504497/liver-disease-and-dyslipidemia-as-a-manifestation-of-lysosomal-acid-lipase-deficiency-lal-d-clinical-and-diagnostic-aspects-and-a-new-treatment-an-update
#1
Luisa Bay, Cristina Canero Velasco, Mirta Ciocca, Andrea Cotti, Miriam Cuarterolo, Alejandro Fainboim, Eduardo Fassio, Marcela Galoppo, Federico Pinero, Paula Rozenfeld
Lysosomal acid lipase deficiency (LAL-D) is still a little recognized genetic disease with significant morbidity and mortality in children and adults. This document provides guidance on when to suspect LAL-D and how to diagnose it. It is recommended to add lysosomal acid lipase deficiency to the List of differential diagnoses of sepsis, oncological diseases, storage diseases, persistent diarrhea, chronic malnutrition, and hemophagocytic lymphohistiocytosis. It should also be considered in young patients with dyslipidemia and atherosclerosis as well as diseases associated with fatty liver and/or hepatomegaly...
June 1, 2017: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/28502381/linking-lysosomal-acid-lipase-insufficiency-to-the-development-of-cryptogenic-cirrhosis
#2
EDITORIAL
Robert C Block, Babak Razani
No abstract text is available yet for this article.
April 25, 2017: Atherosclerosis
https://www.readbyqxmd.com/read/28489830/-clinical-and-laboratory-characteristics-of-patients-with-pulmonary-hypertension-and-pulmonary-vascular-complications-hospitalized-at-the-instituto-nacional-de-salud-del-ni%C3%A3-o
#3
Alexis Jose Ormeño Julca, Carlos Melchor Alvarez Murillo, Pedro Miguel Amoretti Alvino, Angel Aladino Florian Florian, Rosa Aurora Castro Johanson, Maria Danisa Celi Perez, Olga Rocío Huamán Prado
The hepatopulmonary syndrome (HPS) and portopulmonary hypertension (PPHN) are distinct pulmonary vascular complications of portal hypertension (PHT) and are associated with increased morbidity and mortality. OBJECTIVES: To describe the clinical and laboratory characteristics of patients with pulmonary hypertension and pulmonary vascular complications hospitalized at the Instituto Nacional de Salud del Niño. MATERIALS AND METHODS: We included patients with HTP hospitalized from January 2012 to June 2013 and that during its evolution progressed with SHP or HTPP...
January 2017: Revista de Gastroenterología del Perú: órgano Oficial de la Sociedad de Gastroenterología del Perú
https://www.readbyqxmd.com/read/28433112/wilson-disease-liver-pathology
#4
Maciej Pronicki
The liver in Wilson disease may demonstrate a wide range of damage patterns. Some patients may present almost no detectable microscopic pathology, while others display lesions consistent with fulminant hepatitis or acute liver failure. Most liver biopsy specimens show moderate to severe steatosis, variable degree of portal and/or lobular inflammation, and fibrosis eventually progressing to cirrhosis. Additional findings include liver cell degeneration and ballooning, Mallory hyaline bodies, liver cell necrosis, and glycogenation of periportal hepatocytic nuclei...
2017: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/28416360/primary-hepatic-angiosarcoma-and-liver-transplantation-radiological-surgical-histological-findings-and-clinical-outcome
#5
M Tran Minh, A Mazzola, F Perdigao, F Charlotte, G Rousseau, F Conti
Angiosarcoma is a rare type of soft tissue sarcoma that accounts for less than 1% of all sarcomas and only 2% of all primary hepatic tumours. Thorotrast, arsenic, and vinyl chloride monomer are frequently listed as occupational exposure risks. The estimated latency is long (10-40 years) in occupational cases and very long (60 years or more) in non-occupational cases. The symptoms and CT-scan appearance of hepatic angiosarcoma (HAS) are non-specific. We present a case of a 65-year-old Caucasian male with history of cryptogenic cirrhosis, low alpha-foetoprotein levels and a single, 4-cm nodule of potential atypical hepatocellular carcinoma (no washout at MRI and CT-scan) in segment VIII...
April 14, 2017: Clinics and Research in Hepatology and Gastroenterology
https://www.readbyqxmd.com/read/28405148/effect-of-liver-cirrhosis-on-long-term-outcomes-after-acute-respiratory-failure-a-population-based-study
#6
Chih-Cheng Lai, Chung-Han Ho, Kuo-Chen Cheng, Chien-Ming Chao, Chin-Ming Chen, Willy Chou
AIM: To assessed the effect of liver cirrhosis (LC) on the poorly understood long-term mortality risk after first-ever mechanical ventilation (1-MV) for acute respiratory failure. METHODS: All patients in Taiwan given a 1-MV between 1997 and 2013 were identified in Taiwan's Longitudinal Health Insurance Database 2000. Each patient with LC was individually matched, using a propensity-score method, to two patients without LC. The primary outcome was death after a 1-MV...
March 28, 2017: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/28396038/severe-reduction-of-blood-lysosomal-acid-lipase-activity-in-cryptogenic-cirrhosis-a-nationwide-multicentre-cohort-study
#7
Francesco Angelico, Stefano Ginanni Corradini, Daniele Pastori, Silvia Fargion, Anna Ludovica Fracanzani, Mario Angelico, Luigi Bolondi, Giulia Tozzi, Pietro Luigi Pujatti, Giancarlo Labbadia, Gino Roberto Corazza, Maurizio Averna, Francesco Perticone, Giuseppe Croce, Marcello Persico, Tommaso Bucci, Francesco Baratta, Licia Polimeni, Maria Del Ben, Francesco Violi
BACKGROUND AND AIMS: Blood lysosomal acid lipase (LAL) is reduced in non-alcoholic steatohepatitis, which is the major cause of cryptogenic cirrhosis (CC); few data on LAL activity in CC do exist. We investigated LAL activity in a cohort of patients with liver cirrhosis. METHODS: This is a multicentre cohort study including 274 patients with liver cirrhosis of different aetiology from 19 centres of Internal Medicine, Gastroenterology and Hepatology distributed throughout Italy...
March 31, 2017: Atherosclerosis
https://www.readbyqxmd.com/read/28393690/hepatic-amp-kinase-as-a-potential-target-for-treating-nonalcoholic-fatty-liver-disease-evidence-from-studies-of-natural-products
#8
Gang Xu, Kaixun Huang, Jun Zhou
BACKGROUND: Nonalcoholic fatty liver disease (NAFLD), the most common chronic liver disease, is the leading cause of cryptogenic cirrhosis and has consistently been implicated in related metabolic disorders, such as dyslipidemia and type 2 diabetes (T2D). However, the pathogenesis of NAFLD remains to be elucidated, and no established therapeutic regimens for treating NAFLD exist. Adenosine monophosphate (AMP)-activated protein kinase (AMPK), the main cellular energy sensor, has been implicated as a key regulator of hepatic lipid and glucose metabolism...
April 4, 2017: Current Medicinal Chemistry
https://www.readbyqxmd.com/read/28318950/cholesteryl-ester-storage-disease-an-underdiagnosed-cause-of-cirrhosis-in-adults
#9
REVIEW
Mamta Pant, Kiyoko Oshima
Cholesteryl Ester Storage Disease (CESD), is a rare multisystem autosomal recessive disorder and belongs to the broad family of lysosomal storage disorders. It can present anytime from infancy and childhood to even adulthood. The clinical manifestations are generally severe in infants and with milder forms in adults. One of the prominent sites of involvement is liver. Due to low awareness of this condition among physicians including surgical pathologists, majority of the liver biopsies, especially from the adults are often misdiagnosed as non-alcoholic fatty liver disease/non-alcoholic steatohepatitis or cryptogenic cirrhosis...
February 9, 2017: Annals of Diagnostic Pathology
https://www.readbyqxmd.com/read/28293093/clinical-features-and-natural-history-of-cryptogenic-cirrhosis-compared-to-hepatitis-c-virus-related-cirrhosis
#10
Luca Rinaldi, Fabio Nascimbeni, Mauro Giordano, Chiara Masetti, Barbara Guerrera, Annalisa Amelia, Maria Chiara Fascione, Stefano Ballestri, Dante Romagnoli, Rosa Zampino, Riccardo Nevola, Enrica Baldelli, Natalina Iuliano, Valerio Rosato, Amedeo Lonardo, Luigi Elio Adinolfi
AIM: To characterize natural history of cryptogenic cirrhosis (CC) and compare its clinical features and outcomes to those of hepatitis C virus (HCV)-related cirrhosis. METHODS: A prospective cohort of 102 consecutive patients at their first diagnosis of CC were enrolled in this study. The clinical data and outcomes were compared to an age- and Child-Pugh class-matched cohort of 110 patients with HCV-related cirrhosis. Diagnosis of cirrhosis was based on compatible clinical and laboratory parameters, ultrasound/endoscopic parameters and, whenever possible, on histological grounds and transient elastography...
February 28, 2017: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/28280701/renoportal-anastomosis-in-living-donor-liver-transplantation-with-prior-proximal-splenorenal-shunt
#11
Fatih Ozdemir, Koray Kutluturk, Bora Barut, Perviz Abbasov, Ramazan Kutlu, Cuneyt Kayaalp, Sezai Yılmaz
For transplant surgeons, end-stage liver disease with portal venous thrombosis and a previous splenorenal shunt (SRS) is a significant challenge during liver transplantation. Thrombosis of the portal vein can be corrected by surgical interventions, such as portal venous thrombectomy or surgical removal of the thrombosed portal vein. Even also placement of a graft between the mesenteric vein and the graft portal vein can be performed. If these maneuvers fail, a renoportal anastomosis (RPA) can be performed to achieve adequate graft inflow...
February 24, 2017: World Journal of Transplantation
https://www.readbyqxmd.com/read/28260429/low-utility-of-fluorodeoxyglucose-positron-emission-tomography-computed-tomography-for-detecting-hepatocellular-carcinoma-in-patients-before-liver-transplantation
#12
Faisal Alotaibi, Monther Kabbani, Faisal Abaalkhail, Alicia Chorley, Hany Elbeshbeshy, Waleed Al-Hamoudi, Saleh Alabbad, Markus U Boehnert, Mohammad Alsofayan, Wael Al-Kattan, Baderaldeen Ahmed, Dieter Broering, Mohamed Al Sebayel, Hussien Elsiesy
OBJECTIVES: Our program routinely used fluorodeoxyglucose-positron emission tomography/computed tomography as part of the liver transplant evaluation of patients with hepatocellular carcinoma. The aim of this study was to evaluate the role of this imaging modality in the pretransplant work-up. MATERIALS AND METHODS: This was a retrospective chart review of our liver transplant database from January 2011 to December 2014 for all patients with hepatocellular carcinoma who underwent a liver transplant...
February 2017: Experimental and Clinical Transplantation
https://www.readbyqxmd.com/read/28223722/to-screen-or-not-to-screen-celiac-antibodies-in-liver-diseases
#13
REVIEW
Janaína Luz Narciso-Schiavon, Leonardo Lucca Schiavon
Celiac disease (CD) is a systemic immune-mediated disorder triggered by dietary gluten in genetically predisposed individuals. The typical symptoms are anemia, diarrhea, fatigue, weight loss, and abdominal pain. CD has been reported in patients with primary sclerosing cholangitis, primary biliary cholangitis, autoimmune hepatitis, aminotransferase elevations, nonalcoholic fatty liver disease, hepatitis B, hepatitis C, portal hypertension and liver cirrhosis. We evaluate recommendations for active screening for CD in patients with liver diseases, and the effect of a gluten-free diet in these different settings...
February 7, 2017: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/28216976/correlation-of-transient-elastography-with-hepatic-venous-pressure-gradient-in-patients-with-cirrhotic-portal-hypertension-a-study-of-326-patients-from-india
#14
Ashish Kumar, Noor Muhammad Khan, Shrihari Anil Anikhindi, Praveen Sharma, Naresh Bansal, Vikas Singla, Anil Arora
AIM: To study the diagnostic accuracy of transient elastography (TE) for detecting clinically significant portal hypertension (CSPH) in Indian patients with cirrhotic portal hypertension. METHODS: This retrospective study was conducted at the Institute of Liver, Gastroenterology, and Pancreatico-Biliary Sciences, Sir Ganga Ram Hospital, New Delhi, on consecutive patients with cirrhosis greater than 15 years of age who underwent hepatic venous pressure gradient (HVPG) and TE from July 2011 to May 2016...
January 28, 2017: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/28211259/liver-transplantation-from-a-deceased-donor-with-%C3%AE-thalassemia-intermedia-is-not-contraindicated-a-case-report
#15
Ersin Gumus, Osman Abbasoglu, Cahit Tanyel, Fatma Gumruk, Hasan Ozen, Aysel Yuce
The use of extended criteria donors who might have previously been deemed unsuitable is an option to increase the organ supply for transplantation. This report presents a pediatric case of a successful liver transplantation from a donor with β-thalassemia intermedia. A patient, 6-year-old female, with a diagnosis of cryptogenic liver cirrhosis underwent deceased donor liver transplantation from a thalassemic donor. Extreme hyperferritinemia was detected shortly after transplantation. The most probable cause of hyperferritinemia was iron overload secondary to transplantation of a hemosiderotic liver...
May 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/28149148/pediatric-liver-transplantation-our-experiences
#16
Ahmet Basturk, Aygen Yılmaz, Ersin Sayar, Ayhan Dinçhan, İbrahim Aliosmanoğlu, Halil Erbiş, Bülent Aydınlı, Reha Artan
OBJECTIVE: The aim of our study was to evaluate our liver transplant pediatric patients and to report our experience in the complications and the long-term follow-up results. MATERIALS AND METHODS: Patients between the ages of 0 and 18 years, who had liver transplantation in the organ transplantation center of our university hospital between 1997 and 2016, were included in the study. The age, sex, indications for the liver transplantation, complications after the transplantation, and long-term follow-up findings were retrospectively evaluated...
October 2016: Eurasian Journal of Medicine
https://www.readbyqxmd.com/read/28144999/risk-of-hepatocellular-carcinoma-among-individuals-with-different-aetiologies-of-cirrhosis-a-population-based-cohort-study
#17
J West, T R Card, G P Aithal, K M Fleming
BACKGROUND: Among patients with cirrhosis, only those determined to be at risk for hepatocellular carcinoma (HCC) should undergo surveillance. However, little is known about how different aetiologies of cirrhosis affect risk for HCC. AIM: To quantify the cumulative incidence of HCC among a representative population of people with cirrhosis of the liver of varying aetiology. METHODS: We identified subjects with hepatic cirrhosis from the UK's General Practice Research Database (1987-2006)...
February 1, 2017: Alimentary Pharmacology & Therapeutics
https://www.readbyqxmd.com/read/28101016/cellulitis-in-a-liver-transplant-patient-as-an-initial-manifestation-of-disseminated-cryptococcal-disease
#18
Marcos Davi Gomes de Sousa, Fred Bernardes Filho, Luís Eduardo Barros Costa Fernandes, Cássia Regina Guedes Leal, Cristiane Rocha Magalhães, Marcos Antonio Custódio Neto da Silva, Halime Silva Barcaui
A 50-year-old male underwent liver transplantation due to cryptogenic cirrhosis and was admitted with severe pain in the left leg as well as phlogosis. Amoxicillin/clavulanic acid was prescribed, assuming bullous erysipelas. Among the tests performed, the latex agglutination test for the Cryptococcus sp. antigen was positive, and in both the blood culture and blister culture Cryptococcus sp. was isolated. Daily fluconazole was started. Even though liposomal amphotericin B has been started on the fifth day of hospitalization, the patient progressed to death...
September 2016: Case Reports in Dermatology
https://www.readbyqxmd.com/read/28029021/bile-acid-nuclear-receptor-farnesoid-x-receptor-therapeutic-target-for-nonalcoholic-fatty-liver-disease
#19
REVIEW
Sun Gi Kim, Byung Kwon Kim, Kyumin Kim, Sungsoon Fang
Nonalcoholic fatty liver disease (NAFLD) is one of the causes of fatty liver, occurring when fat is accumulated in the liver without alcohol consumption. NAFLD is the most common liver disorder in advanced countries. NAFLD is a spectrum of pathology involving hepatic steatosis with/without inflammation and nonalcoholic steatohepatitis with accumulation of hepatocyte damage and hepatic fibrosis. Recent studies have revealed that NAFLD results in the progression of cryptogenic cirrhosis that leads to hepatocarcinoma and cardiovascular diseases such as heart failure...
December 2016: Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28006764/telomere-dysfunction-in-nonalcoholic-fatty-liver-disease-and-cryptogenic-cirrhosis
#20
Ido Laish, Batya Mannasse-Green, Ruth Hadary, Tal Biron-Shental, Fred M Konikoff, Aliza Amiel, Yona Kitay-Cohen
Nonalcoholic fatty liver disease (NAFLD) and cryptogenic cirrhosis (CC) are considered preneoplastic conditions that might progress to hepatocellular carcinoma. We evaluated parameters of telomere dysfunction in these patient groups to study the correlation between telomere length and the progression of NAFLD. We analyzed peripheral lymphocytes from 22 patients with NAFLD, 20 patients with CC, and 20 healthy, age-matched controls. Telomere length was analyzed using quantitative fluorescence in situ hybridization, and cellular senescence was evaluated by the percentage of cells with senescence-associated heterochromatin foci...
2016: Cytogenetic and Genome Research
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