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cryptogenic cirrhosis

Baek Gyu Jun, Woong Cheul Lee, Jae Young Jang, Soung Won Jeong, Young Don Kim, Gab Jin Cheon, Yun Nah Lee, Sang Gyune Kim, Young Seok Kim, Young Sin Cho, Sae Hwan Lee, Hong Soo Kim
BACKGROUND: Spontaneous bacterial peritonitis (SBP) is one of the severe complications of liver cirrhosis. Early detection of high-risk patients is essential for prognostic improvement. The aim of this study is to investigate the predictive factors related to in-hospital mortality in patients with SBP. METHODS: This was a retrospective study of 233 SBP patients (181 males, 52 females) who were admitted to four tertiary referral hospitals between August 2002 and February 2013...
March 19, 2018: Journal of Korean Medical Science
Avesh J Thuluvath, Po-Hung Chen, Paul J Thuluvath, Sergey Kantsevoy, Yulia Savva
BACKGROUND: Nonalcoholic steatohepatitis (NASH) cirrhosis is a common indication for liver transplantation (LT) in the U.S. There is a paucity of data on retransplantation (re-LT) in those who were initially transplanted for NASH. METHODS: We queried the UNOS data sets from 2002-2016 to analyze the outcomes of adults with NASH (n=128) and compared them to groups that received re-LT for cryptogenic cirrhosis (CC, n=189), alcoholic cirrhosis (AC, n=300) or autoimmune hepatitis cirrhosis (AIH-C, n=118) after excluding multiple-organ re-LT and individuals with HCC...
February 20, 2018: Transplantation
Oana Anisa Nutu, Iago Justo Alonso, Alberto Alejandro Marcacuzco Quinto, Jorge Calvo Pulido, Luis Carlos Jiménez Romero
Refractory ascites is an uncommon complication that may develop postoperatively after liver transplantation. The diagnosis and treatment of this condition is a real challenge. We report two cases of patients who underwent a transplant due to cryptogenic cirrhosis and developed refractory ascites during the immediate postoperative period. This is a serious complication associated with decreased survival by up to one year and a reduced quality of life. After ruling out the main causes of ascites, a portal hyperflow was a potential etiology...
February 7, 2018: Revista Española de Enfermedades Digestivas
G Losurdo, A Castellaneta, M Rendina, S Carparelli, G Leandro, A Di Leo
BACKGROUND: De novo non-alcoholic fatty liver disease (NAFLD) in liver-transplanted patients for cirrhosis not due to non-alcoholic steatohepatitis (NASH) is becoming a growing phenomenon. AIMS: We performed a systematic review and evaluated the prevalence of this event and possible associated factors. METHODS: A literature search in medical databases (PubMed, MEDLINE/OVIDSP, Science Direct and EMBASE) was performed in March 2017. Relevant publications were identified in most important databases...
January 22, 2018: Alimentary Pharmacology & Therapeutics
Timothy M Reynolds, Clare Mewies, John Hamilton, Anthony S Wierzbicki
AIMS: Lysosomal acid lipase deficiency (LALD) is an autosomal recessive disorder of cholesterol ester storage associated with hepatic disease, cirrhosis and accelerated atherosclerosis. Its prevalence in the general population, patients with dyslipidaemia and raised transaminases is unclear. This study attempted to identify the prevalence of LALD from patients with abnormal results in laboratory databases. METHODS: Electronic laboratory databases were interrogated to identify from clinical biochemistry records patients with a phenotype of low high-density lipoprotein-cholesterol (≤0...
January 22, 2018: Journal of Clinical Pathology
Takahiro Kamio, Eiji Hiraoka, Kotaro Obunai, Hiroyuki Watanabe
Constrictive pericarditis (CP) is defined as impedance to diastolic filling caused by a fibrotic pericardium. The diagnosis of CP is a clinical challenge and requires a high index of clinical suspicion. The signs and symptoms of CP include fatigue, edema, ascites, and liver dysfunction. These can be mistakenly diagnosed as primary liver disease. We present the case of a 69-year-old woman with a 7-year history of leg edema and a 2-year history of ascites who was initially diagnosed with cryptogenic liver cirrhosis and was finally diagnosed with CP...
January 11, 2018: Internal Medicine
Stephen Caldwell, Giulio Marchesini
No abstract text is available yet for this article.
December 13, 2017: Journal of Hepatology
Ulla B Berg, Antal Németh
OBJECTIVES: Based on studies with hepatorenal syndrome, it is widely regarded that renal function is impacted in chronic liver disease. Therefore we investigated renal function in our children with chronic liver disease. METHODS: In a retrospective study of 277 children with chronic liver disease, renal function was investigated as glomerular filtration rate (GFR) and effective renal plasma flow (ERPF), measured as clearance of inulin and para-amino hippuric acid or clearance of iohexol...
December 12, 2017: Journal of Pediatric Gastroenterology and Nutrition
S Synoracki, S Kathemann, K W Schmid, H Jastrow, H A Baba
BACKGROUND AND CLINICAL SETTING: Lysosomal acid lipase deficiency is an autosomal recessive storage disease caused by mutations in the LIPA gene. The accumulation of cholesteryl esters and triglycerides in hepatocytes lead to hepatomegaly with progressive fibrosis and liver cirrhosis. Characteristically, patients have a hepatomegaly combined with high serum levels of cholesterol, LDL-cholesterol and in some cases triglyceride, whereas HDL-cholesterol is decreased. Histologically, hepatocytes show a microvesicular steatosis with typically ballooned Kupffer cells...
December 12, 2017: Der Pathologe
Narendra S Choudhary, Sanjay Saran Baijal, Sanjiv Saigal, Amit Agarwal, Neeraj Saraf, Rohit Khandelwal, Vaibhav Jain, Anubhav Harish Khandelwal, Abhay Kapoor, Deepak Jain, Smurti R Misra, Rajesh Puri, Randhir Sud, Arvinder S Soin
Background: Large portosystemic shunts (PSSs) may lead to recurrent encephalopathy in patients with cirrhosis and embolization of these shunts may improve encephalopathy. Material and methods: Five patients underwent balloon-occluded retrograde transvenous obliteration (BRTO) or plug-assisted retrograde transvenous obliteration (PARTO) of a large PSS at our center in last 2 years for recurrent hepatic encephalopathy (HE) at a tertiary care center at north India...
December 2017: Journal of Clinical and Experimental Hepatology
Mohamed Darwish Ahmed Abd Alla, Saleh Ahmed Elibiary, George Y Wu, Mostafa Kamel El-Awady
Background and Aims: Occult HCV infections (OCIs) include IgG antibody seronegative cryptogenic (COCIs), as well as seropositive secondary naïve (SNOCIs) and experienced (SEOCIs) cases. We used peripheral-blood-mononuclear-cell (PBMC)-PCR to evaluate COCIs and SNOCIs prevalence, serum HCV spontaneous disappearance (SCSD) in naïve cirrhotics and non-cirrhotics, intra-PBMC HCV-RNA strands in relation to cirrhosis density in naïve non-viremia cases, and HCV-RNA seroconversion after 1 year of solitary naïve intra-PBMC infection...
December 28, 2017: Journal of Clinical and Translational Hepatology
Paul Joseph Thuluvath, Steven Hanish, Yulia Savva
BACKGROUND: The outcomes of liver transplantation (LT) in patients with cryptogenic cirrhosis (CC) have not been adequately examined except for small case series. We believe that patients currently listed as CC have truly cryptogenic liver disease and may have different post-LT outcomes compared to nonalcoholic steatohepatitis (NASH). METHODS: We compared the post-LT outcomes of adults with CC (n=3241) and compared them with cirrhosis from NASH (n=4089), alcohol (AC, n=7837) and autoimmune hepatitis (AIH, n=1435) using the UNOS database from 2002-16...
December 5, 2017: Transplantation
K Munawar, N Rajagopalan, A Grigorian, D Dennis, M Guglin
Organ donor criteria continue to be extended in an attempt to meet growing demands. Patients with continuous-flow left ventricular assist devices are one group of potential donors being considered. One concern with this group is the effect of continuous flow for a prolonged duration, as opposed to normal pulsatile flow, on end-organ function. We report the 1st case of a liver transplantation from a donor who had a continuous-flow left ventricular assist device for 9 months. The recipient was a 69-year-old woman with a history of cryptogenic cirrhosis and hepatocellular carcinoma...
December 2017: Transplantation Proceedings
Viniyendra Pamecha, Deeplaxmi Purushottam Borle, Senthil Kumar, Kishore Gurumoorthy Subramanya Bharathy, Piyush Kumar Sinha, Shridhar Vasantrao Sasturkar, Vibuti Sharma, Chandra Kant Pandey, Shiv Kumar Sarin
BACKGROUND: Deceased donor liver transplant (DDLT) is an uncommon procedure in India. We present our experience of DDLT from a public sector teaching hospital. METHODS: A retrospective analysis of all DDLT was performed from April 2012 till September 2016. Demographics, intraoperative, donor factors, morbidity, and outcome were analyzed. RESULTS: During the study period, 305 liver transplants were performed, of which 36 were DDLT (adult 32, pediatric 4; 35 grafts; 1 split)...
November 29, 2017: Indian Journal of Gastroenterology: Official Journal of the Indian Society of Gastroenterology
Paul J Thuluvath, Sergey Kantsevoy, Avesh J Thuluvath, Yulia Savva
BACKGROUND & AIMS: We hypothesized that patients currently diagnosed with cryptogenic cirrhosis (CC) have truly 'cryptogenic' liver disease, which is unlikely to have evolved from NASH. The aim of this study is to characterize patients with CC, and compare their characteristics to patients with cirrhosis of other etiologies. METHODS: To investigate this, we compared the clinical characteristics of adults with CC (n = 7,999) to those with cirrhosis caused by non-alcoholic steatohepatitis (NASH) (n = 11,302), alcohol (n = 21,714) and autoimmune hepatitis (n = 3,447), using the UNOS database from 2002-16...
November 21, 2017: Journal of Hepatology
Newton Key Hokama, Marcelo Padovani de Toledo Moraes, Paula de Oliveira Montandon Hokama, Fernando Gomes Romeiro
Most patients with alpha1 antitrypsin deficiency do not receive this diagnosis until developing severe complications, in particular when respiratory symptoms are absent. This is a reason for making alpha1 antitrypsin deficiency a possible diagnosis among patients with cryptogenic cirrhosis or other conditions of liver disease without a clear etiology. In this report, a case of cryptogenic cirrhosis is presented, showing the role of serum protein electrophoresis in the diagnosis, which was made before liver biopsy...
2017: Case Reports in Hepatology
Ismail Cem Sormaz, Gülçin Yegen, Filiz Akyuz, Fatih Tunca, Yasemin Giles Şenyürek
Hepatocellular carcinoma (HCC) is the most common primary malignancy of the liver, and extrahepatic metastases are typically found during disease progression. The incidence of adrenal metastasis (AM) from HCC in autopsy series ranges from 4.6 to 12.5%, and it is the second most common site of metastasis after the lungs. To date, there have been few reports of patients who underwent adrenalectomy for isolated AM from HCC after liver transplantation (LT). A woman aged 55 years was referred to our clinic for the evaluation of a right adrenal mass that was detected by abdominal ultrasonography at another center...
October 2017: Indian Journal of Surgery
Fateh Bazerbachi, Erin E Conboy, Taofic Mounajjed, Kymberly D Watt, Dusica Babovic-Vuksanovic, Shailendra B Patel, Patrick S Kamath
Sitosterolemia is an autosomal recessive metabolic disease caused by mutations in ABCG5 or ABCG8 genes which encode for the (ATP)-binding cassette (ABC) transporters that are responsible for the trafficking of xenosterols. Liver involvement is not a recognized manifestation of this disease, and cirrhosis has been reported only once in the medical literature. We describe a fatal case of a 21-year old South Asian male who presented with decompensated cirrhosis, and biochemical abnormalities consistent with sitosterolemia...
October 16, 2017: Annals of Hepatology
Ji Soo Song, Hee Chul Yu, Woo Sung Moon
No abstract text is available yet for this article.
October 19, 2017: Korean Journal of Internal Medicine
Tomi W Jun, Ming-Lun Yeh, Ju Dong Yang, Vincent L Chen, Pauline Nguyen, Nasra H Giama, Chung-Feng Huang, Ann W Hsing, Chia-Yen Dai, Jee-Fu Huang, Wan-Long Chuang, Lewis R Roberts, Ming-Lung Yu, Mindie H Nguyen
BACKGROUND & AIMS: Although hepatitis B virus (HBV) and hepatitis C virus (HCV) infections remain major risk factors for hepatocellular carcinoma (HCC), non-viral causes of HCC, particularly non-alcoholic fatty liver disease (NAFLD), are becoming increasingly prevalent. The aim of this study was to compare the clinical characteristics and survival of cryptogenic and viral HCC. METHODS: We conducted a retrospective cohort study involving 3878 consecutive HCC patients seen at two tertiary centres in the United States and one in Taiwan from 2004 to 2014...
October 16, 2017: Liver International: Official Journal of the International Association for the Study of the Liver
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