Idiopathic non-cirrhotic intrahepatic portal hypertension: common cause of cryptogenic intrahepatic portal hypertension in a Southern Indian tertiary hospital.
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Date
2009-05
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Abstract
Background and aim Patients with intrahepatic portal hypertension and negative etiological work-up for liver disease are often labeled as having cryptogenic cirrhosis. The aim of
this study was to evaluate causes of liver disease in patients
with unexplained intrahepatic portal hypertension.
Methods We retrospectively analyzed cause of liver disease
in all patients with cryptogenic intrahepatic portal hypertension
who underwent liver biopsies between June 2005 to
June 2007 in our center.
Results Five hundred and seventeen patients underwent
liver biopsies of whom 227 had portal hypertension. Of these,
the cause of liver disease could not be detected prior to liver
biopsy in 62 patients. Causes of liver disease identified after
liver biopsy in these 62 patients were: idiopathic non-cirrhotic
intrahepatic portal hypertension (NCIPH) (30 patients,
48 ), cirrhosis (14), fatty liver disease (7) and other causes
(11). Initial presentations in idiopathic NCIPH patients were
splenomegaly and anemia (18 patients), variceal bleed (9) and
ascites (3). Median age (range) of patients at first presentation
was 32 (15-57) years, and 19 were male. Majority (90 )
were in Child’s class A. Hepatic vein pressure gradient was
<5 mmHg in 2 of 7 NCIPH patients tested.
Conclusions We identified 30 patients with idiopathic
NCIPH at our center over the 2 year study period. The clinical
presentation and investigations of NCIPH closely mimic cryptogenic
cirrhosis. Idiopathic NCIPH should be considered as a differential diagnosis of cryptogenic cirrhosis in India.
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Keywords
Cryptogenic cirrhosis, hepatic vein pressure gradient, liver biopsy
Citation
Madhu Kadiyala, Avinash Balekuduru, Ramakrishna Banumathi, Eapen C E, Shyamkumar N K, Zachariah Uday, Chandy George, Kurian George. Idiopathic non-cirrhotic intrahepatic portal hypertension: common cause of cryptogenic intrahepatic portal hypertension in a Southern Indian tertiary hospital. Indian Journal of Gastroenterology. 2009 May-Jun; 28(3): 83-87.