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Idiopathic non-cirrhotic intrahepatic portal hypertension: common cause of cryptogenic intrahepatic portal hypertension in a Southern Indian tertiary hospital

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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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Authors and Affiliations

  1. Department of Gastrointestinal Sciences, Christian Medical College, Vellore, 632 004, India

    Kadiyala Madhu, Balekuduru Avinash, C. E. Eapen, Uday Zachariah, George Chandy & George Kurian

  2. Department of Pathology, Christian Medical College, Vellore, 632 004, India

    Banumathi Ramakrishna

  3. Department of Radiology, Christian Medical College, Vellore, 632 004, India

    N. K. Shyamkumar

Authors
  1. Kadiyala Madhu
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  2. Balekuduru Avinash
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  3. Banumathi Ramakrishna
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  4. C. E. Eapen
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  5. N. K. Shyamkumar
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  6. Uday Zachariah
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  7. George Chandy
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  8. George Kurian
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Corresponding author

Correspondence to C. E. Eapen.

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Madhu, K., Avinash, B., Ramakrishna, B. et al. Idiopathic non-cirrhotic intrahepatic portal hypertension: common cause of cryptogenic intrahepatic portal hypertension in a Southern Indian tertiary hospital. Indian J Gastroenterol 28, 83–87 (2009). https://doi.org/10.1007/s12664-009-0030-3

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  • DOI: https://doi.org/10.1007/s12664-009-0030-3

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