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      Unexplained Findings of Kayser-Fleischer-Like Rings in a Patient with Cryptogenic Cirrhosis

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          Abstract

          Cryptogenic cirrhosis (CC) is defined as cirrhosis occurring in an individual without an identifiable cause of liver disease, such as excessive alcohol consumption, viral hepatitis infection, hemochromatosis, autoimmune hepatitis, primary biliary cirrhosis, primary sclerosing cholangitis, chronic intake of medications that could induce cirrhosis, alpha-1-antitrypsin deficiency, Wilson's disease, or any other rare cause of cirrhosis according to the clinical context. Cryptogenic cirrhosis is a common cause of liver-related morbidity and mortality in the United States. Nonalcoholic fatty liver disease is now recognized as the most common cause of cryptogenic cirrhosis. A biopsy specimen is also important for detecting histological advanced disease, which may be clinically silent and undetected by liver-related tests or diagnostic imaging. We are presenting an unusual case of a patient with cryptogenic cirrhosis found to have Kayser-Fleischer-like rings without evidence of Wilson's disease.

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          Nonalcoholic fatty liver disease: an underrecognized cause of cryptogenic cirrhosis.

          Cryptogenic cirrhosis is a common cause of liver-related morbidity and mortality in the United States. Nonalcoholic fatty liver disease (NAFLD) is now recognized as the most common cause of cryptogenic cirrhosis. However, the diagnosis of cirrhosis in patients with NAFLD appears to be delayed compared with those with other chronic liver diseases and thus carries a higher mortality rate. This delay in diagnosis is illustrated in our case of a 53-year-old man who presented with hepatic hydrothorax and ascites, whose workup revealed cirrhosis due to NAFLD. Although a diagnosis of presumed NAFLD can be made noninvasively, a definitive diagnosis requires a liver biopsy specimen. A biopsy specimen is also important for detecting histologically advanced disease, which may be clinically silent and undetected by aminotransferases or diagnostic imaging. Although there are no proven treatments, recommendations for patients with NAFLD include avoidance of hepatotoxins and aggressive management of associated conditions, such as hypertriglyceridemia and type 2 diabetes mellitus.
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            Prevalence of obesity and diabetes in patients with cryptogenic cirrhosis: a case-control study.

            It has recently been suggested that nonalcoholic steatohepatitis (NASH) is an under-recognized cause of cryptogenic cirrhosis (CC) on the basis of higher prevalence of obesity and type II diabetes among these patients. To test this hypothesis, we studied 65 consecutive patients with advanced cirrhosis (Child-Pugh Score >/= 7) of undetermined etiology (CC) from our active waiting list for liver transplantation in January 1993, 1996, and 1999. For each patient, we selected 2 age- and sex-matched controls from the corresponding lists. The prevalence of obesity (defined as body mass index [BMI] >/= 30) and diabetes were compared between the groups. Sixteen patients (and their 32 controls) with CC were excluded as further review of records suggested other possible etiologies. Thus, the final analysis included 49 patients and 98 controls. The etiology of cirrhosis in the control group was alcohol in 16.3%, chronic viral hepatitis in 30.6%, autoimmune hepatitis in 8.2%, and primary biliary cirrhosis (PBC) or primary sclerosing cholangitis in 35.7%. The prevalence of obesity (55% vs. 24%) and type II diabetes (47% vs. 22%) was significantly higher in patients with CC compared with controls. Twenty-three percent of patients with CC had both obesity and diabetes compared with 5% among controls (P =.002). There was no difference in the prevalence of hypercholesterolemia (serum cholesterol > 200 mg/dL) between the groups. In conclusion, patients with advanced CC are more likely to be obese and diabetic compared with age- and sex-matched patients with advanced cirrhosis. This supports the hypothesis that NASH may be an etiological factor in some of the patients with CC.
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              Survival, liver failure, and hepatocellular carcinoma in obesity-related cryptogenic cirrhosis.

              Despite the rising incidence of obesity and diabetes, there is little emphasis on morbidity and mortality from obesity-related cirrhosis, usually considered a rare and asymptomatic condition. Our aim was to assess survival and the occurrence of hepatocellular carcinoma and complications of hepatic insufficiency in obesity-related cryptogenic cirrhosis compared with cirrhosis of other origins. We analyzed retrospectively 27 overweight patients with cryptogenic cirrhosis (CC-O), 10 lean patients with cryptogenic cirrhosis (CC-L) and 391 patients with hepatitis C virus-related cirrhosis (C-HCV). In CC-O patients, cirrhosis was detected later in life than in C-HCV and CC-L patients. Severe liver disease was as frequent in CC-O as in C-HCV patients as indicated by the proportion of Child B or C or of episodes of hepatic decompensation. Survival of CC-O patients was lower than that of untreated, age- and sex-matched C-HCV controls (P <.02 at 30 months), with a higher mortality of Child B or C patients. Hepatocellular carcinoma was detected in 8 of 27 (27%) CC-O patients versus 21% of matched C-HCV controls with a similar age cumulated incidence, suggesting a comparable carcinogenic potential. In conclusion, obesity-related cirrhosis should now be recognized as a distinct entity that can cause severe liver disease and death. Increased awareness of and better diagnostic strategies for nonalcoholic steatohepatitis in overweight patients are urgently needed.
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                Author and article information

                Journal
                Case Rep Gastrointest Med
                Case Rep Gastrointest Med
                CRIM.GM
                Case Reports in Gastrointestinal Medicine
                Hindawi Publishing Corporation
                2090-6528
                2090-6536
                2012
                15 March 2012
                : 2012
                : 438525
                Affiliations
                Department of Medicine, Nassau University Medical Center, East Meadow, NY 11554, USA
                Author notes
                *Ghulamullah Shahzad: shahzag@ 123456gmail.com

                Academic Editors: O. I. Giouleme and A. J. Lembo

                Article
                10.1155/2012/438525
                3350065
                22606437
                791c1e62-f7a7-4425-850c-8c52b002bc4c
                Copyright © 2012 Mahreema Jawairia et al.

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

                History
                : 30 November 2011
                : 4 January 2012
                Categories
                Case Report

                Gastroenterology & Hepatology
                Gastroenterology & Hepatology

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