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Tuesday, 3 December 2013

Cirrhosis - Diseases associated with Cirrhosis

Cirrhosis is defined as a condition of irreversible scarring liver as a result of liver tissue by fibrosis due to final phase of chronic liver diseases of that can lead to poor function of the liver and liver failure. According to the statistics, Number of discharges with chronic liver disease or cirrhosis as the first-listed diagnosis: 101,000 in 2009 and Deaths per 100,000 population: 10.3 in 2010(a). Hepatitis B infection cause of the disease is very prevalent in South-East Asia.
Diseases associated Cirrhosis
1. Autoimmune diseases
In the study to assess the prevalence of concurrent extrahepatic autoimmune diseases in patients with autoimmune hepatitis (AIH)/primary biliary cirrhosis (PBC) overlap syndrome and applicability of the 'mosaic of autoimmunity' in these patients, in a population, 31 (43.6%) patients had extrahepatic autoimmune diseases, including autoimmune thyroid diseases (13 patients, 18.3%), Sjögren syndrome (six patients, 8.4%), celiac disease (three patients, 4.2%), psoriasis (three patients, 4.2%), rheumatoid arthritis (three patients, 4.2%), vitiligo (two patients, 2.8%), and systemic lupus erythematosus (two patients, 2.8%).conducted by the Numune Research and Education Hospital, showed that a large number of autoimmune diseases were associated with AIH/PBC overlap patients. Therefore, extended screening for existing autoimmune diseases during the routine assessment of these patients is recommended. The study also sugested that the concept of 'mosaic of autoimmunity' is a valid clinical entity that is applicable to patients with AIH/PBC overlap syndrome(34).

2. Cardiovascular disease
Impaired glucose tolerance or diabetes are frequently observed in cirrhosis. Cirrhotic patients, even in the presence of overt diabetes, are at low risk of cardiovascular disease. The low prevalence may be related to shorter duration of diabetic disease, also in relation to reduced life expectancy, as well as to liver disease-induced abnormalities protecting the cardiovascular system from atherosclerosis(35).

3. Hepatic angiomyolipoma
Hepatic angiomyolipomas are increasingly recognized as incidental findings during surveillance for cirrhosis or investigations for unrelated conditions. Awareness of their diverse morphological spectrum in liver biopsy is necessary to avoid misdiagnosis as hepatocellular carcinoma, metastatic melanoma or other malignant neoplasms(36).

4. Obesity
Obesity is associated with an aggressive course in chronic viral hepatitis. In a randomized trial of beta-blockers in the prevention of varices in whom data on body mass index (BMI) was available, consisted of 161 patients with compensated cirrhosis, found that obesity has a deleterious effect on the natural history of compensated cirrhosis of all etiologies, independent of portal pressure and liver function. Weight reduction may be a valuable therapeutic measure in this patient population(37).

5. Steatohepatitis
According to the study by University of Sydney at Westmead Hospital, age, activity of steatohepatitis, and established fibrosis predispose to cirrhosis, which has a 7- to 10-year liver-related mortality of 12% to 25%. Many cases of cryptogenic cirrhosis are likely endstage NASH. While endstage NAFLD currently accounts for 4% to 10% of liver transplants, this may soon rise(38).

6. Glycogen storage disease
Glycogen storage disease Ib is an inborn error of carbohydrate metabolism leading to impaired glycogenolysis and gluconeogenesis. There is a report of a case of on the development of liver cirrhosis in a nine-year-old boy in the course of glycogen storage disease Ib and discuss possible underlying pathomechanisms(39).
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Sources
(a) http://www.cdc.gov/nchs/fastats/liverdis.htm
(34) http://www.ncbi.nlm.nih.gov/pubmed/22465972
(35) http://www.ncbi.nlm.nih.gov/pubmed/10086647
(36) http://www.ncbi.nlm.nih.gov/pubmed/22949933
(37) http://www.ncbi.nlm.nih.gov/pubmed/21567436
(38) http://www.ncbi.nlm.nih.gov/pubmed/16447287
(39) http://www.ncbi.nlm.nih.gov/pubmed/23357201