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We would like to thank Goel et al. for their reply to our review of idiopathic noncirrhotic portal hypertension (INCPH).1, 2

The authors studied the prevalence of INCPH among all new portal hypertension patients from July 2009 until July 2010. Eventually, 17 (39%) patients were diagnosed with INCPH. The authors described the morphological features observed in liver specimens from these 17 INCPH patients. Interestingly, neither the presence of nodular regenerative hyperplasia nor obliteration of portal venules (phlebosclerosis)—both the most frequently observed morphological features in liver specimens of Western INCPH patients—were found.3, 4 The apparent absence of these features in Indian INCPH patients could imply differences in pathophysiological mechanisms between patients from different continents.

As reported by Goel et al., liver biopsy is mandatory in the diagnosis of INCPH. Its main role is the exclusion of liver cirrhosis, which may be very difficult to discern by radiological examinations only. In addition, the diagnosis of INCPH can be suggested or supported by the presence of morphological features such as nodular regenerative hyperplasia, phlebosclerosis, increased number of portal vascular channels, paraportal shunt vessels, and sinusoidal dilatation. However, one must take into account that these features can also be observed in patients without clinical signs of portal hypertension.

References

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  • 1
    Schouten JN, Garcia-Pagan JC, Valla DC, Janssen HL. Idiopathic noncirrhotic portal hypertension. HEPATOLOGY 2011; 54: 1071-1081.
  • 2
    Hillaire S, Bonte E, Denninger MH, Casadevall N, Cadranel JF, Lebrec D, et al. Idiopathic non-cirrhotic intrahepatic portal hypertension in the West: a re-evaluation in 28 patients. Gut 2002; 51: 275-280.
  • 3
    Cazals-Hatem D, Hillaire S, Rudler M, Plessier A, Paradis V, Condat B, et al. Obliterative portal venopathy: portal hypertension is not always present at diagnosis. J Hepatol 2011; 54: 455-461.
  • 4
    Hillaire S, Bonte E, Denninger MH, Casadevall N, Cadranel JF, Lebrec D, et al. Idiopathic non-cirrhotic intrahepatic portal hypertension in the West: a re-evaluation in 28 patients. Gut 2002; 51: 275-280.

J.N.L. Schouten M.D.*, H.L.A. Janssen Ph.D.*, * Department of Gastroenterology Hepatology, University Hospital Rotterdam, Rotterdam, The Netherlands.