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cryptogenic cirrhosis

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https://www.readbyqxmd.com/read/28318950/cholesteryl-ester-storage-disease-an-underdiagnosed-cause-of-cirrhosis-in-adults
#1
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
#2
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
#3
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
#4
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
#5
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
#6
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
#7
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...
February 17, 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/28149148/pediatric-liver-transplantation-our-experiences
#8
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
#9
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
#10
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
#11
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
#12
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
https://www.readbyqxmd.com/read/27959847/-pulmonary-embolism-after-endoscopic-injection-with-n-butyl-2-cyanoacrylate-for-gastric-varices
#13
Gabriela Robaina, Ricardo Albertini, Martín Carranza, Pablo Herrena Najum
Gastric varices occur in one-third of patients with portal hypertension. Bleeding from gastric varices remains a significant cause of death. Currently the first-line of treatment for gastric varices is endoscopic obliteration with N-butyl-2-cyanoacrylate. Though relatively safe, this option has several well-known complications. We report the case of a 61-year-old male patient with cryptogenic cirrhosis, who presented with fever, tachycardia and hypoxemia after endoscopic obliteration with N-butyl-2-cyanoacrylate...
2016: Medicina
https://www.readbyqxmd.com/read/27922486/the-post-transplant-course-of-patients-undergoing-liver-transplantation-for-nonalcoholic-steatohepatitis-versus-cryptogenic-cirrhosis-a-retrospective-case-control-study
#14
Lukas W Unger, Merima Herac, Katharina Staufer, Andreas Salat, Gerd Silberhumer, Michael Hofmann, Michael Trauner, Susanne Rasoul-Rockenschaub, Thomas Soliman, Thomas Reiberger, Gabriela A Berlakovich
BACKGROUND: Nonalcoholic fatty liver disease (NAFLD) can be considered the hepatic manifestation of the metabolic syndrome with nonalcoholic steatohepatitis (NASH) as its progressive form. With increasing prevalence of the metabolic syndrome, NASH cirrhosis is becoming a leading cause for liver transplantation. Some cases of orthotopic liver transplantation (OLT) due to cryptogenic cirrhosis (CC) might show typical features of NASH cirrhosis. Therefore, our aim was to assess recurrence of liver fibrosis in patients transplanted for NASH versus CC after OLT...
March 2017: European Journal of Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/27872411/osteoporosis-in-pediatric-liver-transplantation
#15
Walid Faraj, Ghina El Nounou, Abdallah Abou Al Naaj, Nancy Nakhoul, Ali Haydar, Mohammad Khalife
Liver transplantation provides an important, often life-saving treatment for end-stage liver disease. Osteoporosis post-liver transplantation has been described in adults; however, this has not been described in the pediatric population to date. We present a case of a 13-year-old female patient who underwent an orthotopic liver transplant for cryptogenic liver cirrhosis. Her immunosuppressants were tacrolimus and prednisone. Four months posttransplant, she started complaining of bilateral lower limb pain and limping while walking, progressing to a point where she was almost immobile...
December 2016: Progress in Transplantation
https://www.readbyqxmd.com/read/27822261/the-cause-and-effect-relationship-between-type-2-diabetes-mellitus-and-clinically-overt-cryptogenic-cirrhosis-a-matter-that-must-be-seriously-revised
#16
Abbas Tavakolian Arjmand, Nasrin Razavianzadeh
No abstract text is available yet for this article.
September 2016: Hepatitis Monthly
https://www.readbyqxmd.com/read/27818588/update-on-occult-hepatitis-b-virus-infection
#17
REVIEW
Manoochehr Makvandi
The event of mutations in the surface antigen gene of hepatitis B virus (HBV) results in undetectable hepatitis B surface antigen with positive/negative anti-hepatitis B core (anti-HBc) antibody status in serum and this phenomenon is named occult hepatitis B infection (OBI). The presence of anti-HBc antibody in serum is an important key for OBI tracking, although about 20% of OBI cases are negative for anti-HBc antibody. The diagnosis of OBI is mainly based on polymerase chain reaction (PCR) and real-time PCR assays...
October 21, 2016: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/27805526/histologic-changes-in-bone-marrow-biopsies-from-liver-transplant-patients
#18
Pelin Börcek, Binnaz Handan Özdemir, Çiğdem Sercan, Eda Yılmaz Akçay, Sema Karakuş, Mehmet Haberal
OBJECTIVES: Liver transplant may complicated by various hematologic conditions, resulting in indication for bone marrow biopsy. Immunosuppressive therapies, specific infections, and secondary neoplasms affect bone marrow. In the present study, we evaluated the histologic spectrum of bone marrow findings in liver allograft recipients. MATERIALS AND METHODS: Of 338 patients who received liver transplants and were followed at the Başkent University, Faculty of Medicine, 44 patients underwent bone marrow biopsy...
2016: Experimental and Clinical Transplantation
https://www.readbyqxmd.com/read/27621523/ayurvedic-management-of-cirrhotic-ascites
#19
G Aswathy, Prasanth Dharmarajan, Ananth Ram Sharma, V K Sasikumar, M R Vasudevan Nampoothiri
Cirrhosis is the final stage of most of the chronic liver diseases and is most invariably complicated by portal hypertension resulting in ascites. A case of chronic liver disease with portal hypertension (cryptogenic cirrhosis), managed at Amrita School of Ayurveda is discussed in this paper. The clinical picture was that of an uncomplicated cirrhotic ascites. Snehapāna (therapeutic oral administration of lipids) followed by virecana (purgation) was done after an initial course of nityavirecana (daily purgation)...
April 2016: Ancient Science of Life
https://www.readbyqxmd.com/read/27555800/idiopathic-noncirrhotic-portal-hypertension-current-perspectives
#20
REVIEW
Oliviero Riggio, Stefania Gioia, Ilaria Pentassuglio, Valeria Nicoletti, Michele Valente, Giulia d'Amati
The term idiopathic noncirrhotic portal hypertension (INCPH) has been recently proposed to replace terms, such as hepatoportal sclerosis, idiopathic portal hypertension, incomplete septal cirrhosis, and nodular regenerative hyperplasia, used to describe patients with a hepatic presinusoidal cause of portal hypertension of unknown etiology, characterized by features of portal hypertension (esophageal varices, nonmalignant ascites, porto-venous collaterals), splenomegaly, patent portal, and hepatic veins and no clinical and histological signs of cirrhosis...
2016: Hepatic Medicine: Evidence and Research
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