The authors are grateful for funds received from the Fluid Research Fund at Christian Medical College (Vellore, India) for this study. Potential conflict of interest: Nothing to report.
Article first published online: 30 NOV 2011
Copyright © 2011 American Association for the Study of Liver Diseases
Volume 54, Issue 6, pages 2275–2276, December 2011
How to Cite
Goel, A., Ramakrishna, B., Madhu, K., Zachariah, U., Ramachandran, J., Keshava, S. N., Elias, E. and Eapen, C. E. (2011), Idiopathic noncirrhotic intrahepatic portal hypertension is an ongoing problem in India. Hepatology, 54: 2275–2276. doi: 10.1002/hep.24750
Potential conflict of interest: Nothing to report.
- Issue published online: 30 NOV 2011
- Article first published online: 30 NOV 2011
- Accepted manuscript online: 26 OCT 2011 12:12PM EST
- Manuscript Revised: 22 SEP 2011
- Manuscript Accepted: 22 SEP 2011
- Manuscript Received: 13 SEP 2011
To the Editor:
Schouten et al. describe the need for histological confirmation of idiopathic noncirrhotic portal hypertension and its contrasting incidence between the West and India.1 We prefer the term idiopathic noncirrhotic intrahepatic portal hypertension (NCIPH) to distinguish it from extrahepatic portal vein thrombosis—the most common cause of pediatric portal hypertension at our center.2
After the report from Chandigarh in 2002,3 there is scarce literature on the incidence of biopsy-proven NCIPH in India. We herein report on our recent experience with NCIPH.
From 2005 to 2007, retrospective analysis of 227 portal hypertensive patients who underwent liver biopsy at our center showed that of 62 patients labeled as having “cryptogenic cirrhosis,” 30 (48%) were diagnosed as having NCIPH after liver biopsy.4
We prospectively studied the prevalence of NCIPH among all new portal hypertensive patients in our unit from July 2009 to July 2010 (after institutional ethics committee approval). NCIPH was diagnosed as per the previously described criteria.4 The need for liver biopsy in each patient was decided on a case-by-case basis, based on the clinical scenario.
Of 610 consecutive new portal hypertensive patients studied, cryptogenic cirrhosis (210 patients) was the most common cause of portal hypertension identified after noninvasive tests. Of 44 cryptogenic cirrhosis patients who underwent liver biopsy, 17 (39%) had NCIPH and 8 had “true cryptogenic cirrhosis.” NCIPH and true cryptogenic cirrhosis patients were 27 (range, 14-59) and 42 (range, 25-67) years old, respectively; 10 and 4 patients, respectively, were males. Hepatic venous pressure gradient measured in 15 NCIPH and 4 true cryptogenic cirrhosis patients was 7 (range, 1-21) and 18 (range, 10-27) mmHg, respectively (P = 0.012).
Liver biopsies were performed percutaneously in 4 NCIPH patients and transjugularly in 13. Number of cores in percutaneous biopsies was 3 per patient and 3 (range, 1-6) in transjugular biopsies; length of the largest core was 13 (range,12-15) in percutaneous and 12 mm (range, 6-16) in transjugular biopsies. The number of portal tracts in liver biopsies was 10 (range, 5-20). Liver biopsies showed no significant fibrosis (6 patients), mild portal/periportal fibrosis (10), moderate fibrosis (1), mild perisinusoidal fibrosis (1), abnormal portal venous ectasia (6), and mild diffuse sinusoidal dilatation (8); no patient had cirrhosis or severe fibrosis.
In summary, in 2009-2010 and 2005-2007,4 39%-48% of patients with clinical diagnosis of cryptogenic cirrhosis who underwent liver biopsy at our center had NCIPH.
- 1Garcia-Pagan JC, Valla DC, Janssen HL. Idiopathic noncirrhotic portal hypertension. HEPATOLOGY 2011; 54: 1071-1081.,
- 2Aetiology of paediatric portal hypertension—experience of a tertiary care centre in South India. Trop Doct 2009; 39: 42-44., , , , , , et al.
- 3Non-cirrhotic portal fibrosis (idiopathic portal hypertension): experience with 151 patients and a review of the literature. J Gastroenterol Hepatol 2002; 17: 6-16., , , , , , et al.
- 4Idiopathic non-cirrhotic intrahepatic portal hypertension: common cause of cryptogenic intrahepatic portal hypertension in a Southern Indian tertiary hospital. Indian J Gastroenterol 2009; 28: 83-87., , , , , , et al.
Ashish Goel*, Banumathi Ramakrishna, Kadiyala Madhu*, Uday Zachariah*, Jeyamani Ramachandran*, Shyamkumar N. Keshava, Elwyn Elias§, Chundamannil E. Eapen*, * Departments of Hepatology, Christian Medical College, Vellore, India, Pathology, Christian Medical College, Vellore, India, Radiology, Christian Medical College, Vellore, India, § Liver Unit, University Hospital Birmingham , Birmingham, United Kingdom.