Skip to main content
SpringerLink
Log in

Idiopathic Portal Hypertension

  • Chapter
  • First Online:
Clinical Investigation of Portal Hypertension

  • 1017 Accesses

Abstract

Idiopathic portal hypertension (IPH) is a disease presenting as non-cirrhotic portal hypertension due to a presinusoidal portal blood flow block and increased portal blood flow with an enlarged spleen, although the etiologic factors have not been identified. IPH is most common in middle-aged females and is predominant in females versus males in the overall population in Japan. The major signs and symptoms are pancytopenia with splenomegaly and gastroesophageal varices. The clinical manifestations are relatively mild and insidious in most of the patients, except in the case of variceal hemorrhage. A high incidence of portal vein thrombosis is also characteristic. Diagnosis is made by the differentiating IPH from the diseases of portal hypertension with identified causes because there are no specific clinical findings for the diagnosis of IPH. Portal and hepatic venography or liver histology is helpful for the diagnosis. The treatment of portal hypertension-related problems is the same as for problems related to liver cirrhosis. The clinical course and overall survival are well maintained in patients for whom gastroesophageal varices are effectively controlled. However, complications such as refractory ascites or intestinal ectopic varices may appear in the late stages of the disease.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 169.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Hardcover Book
USD 219.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Okuda K. Noncirrhotic portal hypertension. In: Okuda K, Mitchell DG, Itai Y, Ariyama J, editors. Hepatobiliary diseases pathophysiology and imaging. London: Blackwell Science; 2001. p. 287–314.

    Google Scholar 

  2. Banti G. Del l’anemia splenica. Arch di Scuola d’Anat Patol (Firenze). 1983;2:53–122.

    Google Scholar 

  3. Banti G. Ueber Morbus Banti Folia Haematol. 1910;10:33–74.

    Google Scholar 

  4. Hsia DYY, Gellis SS. Portal hypertension in infants and children. Am J Dig Child. 1955;90:290–8.

    CAS  Google Scholar 

  5. Polish E, Christie J, Cohen A, et al. Idiopathic portal hypertension (Banti’s syndrome). Ann Intern Med. 1962;56:624–7.

    Article  CAS  Google Scholar 

  6. Williams P, Parsonson A, Somers K. Portal hypertension in idiopathic tropical splenomegaly. Lancet I. 1966;287:329–33.

    Article  Google Scholar 

  7. Ramalingaswarmi B, Wig HL, Sarma SK. Cirrhosis of the liver in northern India: pathological study. Arch Intern Med. 1962;110:350–8.

    Article  Google Scholar 

  8. Mikkelsen WP, Edmondson HA, Peters RL, et al. Extra- and intra-hepatic portal hypertension without cirrhosis (hepatoportal sclerosis). Ann Surg. 1965;162:602–20.

    Article  CAS  Google Scholar 

  9. Boyer JL, Sengupta KP, Biswas SK, et al. Idiopathic portal hypertension: comparison with portal hypertension of cirrhosis and extrahepatic portal vein obstruction. Ann Intern Med. 1967;66:41–68.

    Article  CAS  Google Scholar 

  10. Schouten JNL, Garcia-Pagan JC, Valla DC, Janssen HLA. Idiopathic noncirrhotic portal hypertension. Hepatology. 2011;54:1071–81.

    Article  Google Scholar 

  11. Kotani K, Kawabe J, Morikawa H, et al. Comprehensive screening of gene function and networks by DNA microarray analysis in Japanese patients with idiopathic portal hypertension. Mediat Inflamm. 2015;2015:349215. https://doi.org/10.1155/2015/349215. Epub 2015 Oct 4.

    Article  CAS  Google Scholar 

  12. Tsuneyama K, Harada K, Katayanagi K, et al. Overlap of idiopathic portal hypertension and scleroderma: report of two autopsy cases and a reviewer of literature. J Gastroenterol Hepatol. 2002;17:217–23.

    Article  Google Scholar 

  13. Okabayashi A. Experimental production of noncirrhotic portal hypertension and splenomegaly with prolonged antigenic stimulation. In: Okuda K, Omata M, editors. Idiopathic portal hypertension. Tokyo: University of Tokyo press; 1983. p. 197–217.

    Google Scholar 

  14. Kono K, Ohnishi K, Omata M, et al. Experimental portal fibrosis produced by intraportal injection of killed nonpathogenic E. coli in rabbits. Gastroenterology. 1988;94:787–96.

    Article  CAS  Google Scholar 

  15. Akahoshi T, Hashizume M, Tanoue K, et al. Role of the spleen in liver fibrosis in rat may be mediated by growth factor beta-1. J Gastroenterol Hepatol. 2002;17:59–65.

    Article  CAS  Google Scholar 

  16. Sato Y, Nakanuma Y. Role of endothelial-mesenchymal transition in idiopathic portal hypertension. Histol Histopathol. 2013;28:145–54.

    PubMed  Google Scholar 

  17. Morikawa H, Tamori A, Nishiguchi S, et al. Expression of connective tissue growth factor in the human liver with idiopathic portal hypertension. Mol Med. 2007;13:240–5.

    Article  CAS  Google Scholar 

  18. Hirota Y, Annual Report of Research Committee on Portal Hemodynamics Abnormalities, The Ministry of Health Labor and Welfare of Japan;2006:82–92 (Japanese).

    Google Scholar 

  19. Okudaira M, Ohbu M, Okuda K. Idiopathic portal hypertension and its pathology. Semin Liver Dis. 2002;22:59–71.

    Article  Google Scholar 

  20. Futagawa S, Fukazawa M, Horisawa M, et al. Portographic liver changes in idiopathic noncirrhotic portal hypertension. AJR. 1980;134:917–23.

    Article  CAS  Google Scholar 

  21. Okuda K, Kono K, Ohnishi K, et al. Clinical study of eighty-six cases of idiopathic portal hypertension and comparison with cirrhosis with splenomegaly. Gastroenterology. 1984;86:600–10.

    CAS  PubMed  Google Scholar 

  22. Futagawa S, Fukazawa M, Musha H, et al. Hepatic venography in noncirrhotic idiopathic portal hypertension. Radiology. 1981;141:303–8.

    Article  CAS  Google Scholar 

  23. Hillaire S, Bonte E, Denninger MH, et al. Idiopathic non-cirrhotic intrahepatic portal hypertension in the West: a re-evaluation in 28 patients. Gut. 2002;51:275–80.

    Article  CAS  Google Scholar 

  24. Matsutani S, Maruyama H, Akiike T, et al. Study of portal vein thrombosis in patients with idiopathic portal hypertension in Japan. Liver Int. 2005;25:978–83.

    Article  Google Scholar 

  25. Matsutani S. Blood coagulation inhibitors and portal vein thrombosis in patients with portal hypertension. Annual Report of Research Committee on Portal Hemodynamics Abnormalities, The Ministry of Health Labor and Welfare of Japan;2009:31–5 (Japanese).

    Google Scholar 

  26. Ohnishi K, Saito M, Sato S, et al. Portal hemodynamics in idiopathic portal hypertension (Banti’s syndrome) comparison with chronic persistent hepatitis and normal subjects. Gastroenterology. 1987;92:751–8.

    Article  CAS  Google Scholar 

  27. Furuichi Y, Moriyasu F, Taira J, et al. Noninvasive diagnostic method for idiopathic portal hypertension based on measurements of liver and spleen stiffness by ARFI elastography. J Gastroenterol. 2013;48:1061–8.

    Article  Google Scholar 

  28. Qureshi K, Patel S, Meillier A. The use of thrombopoietin receptor agonist for correction of thrombocytopenia prior to elective procedures in chronic liver diseases: review of current evidence. Int J Hepatol. 2016;2016:1802932. Epub 2016 Oct 9.

    Article  Google Scholar 

  29. Ichimura S, Sasaki R, Takemura Y, et al. The prognosis of idiopathic portal hypertension in Japan. Intern Med. 1993;32:441–4.

    Article  CAS  Google Scholar 

  30. Murai Y, Ohfuji S, Fukushima W, et al. Prognostic factors in patients with idiopathic portal hypertension: two Japanese nationwide epidemiological surveys in 1999 and 2005. Hepatol Res. 2012;42:1211–20.

    Article  Google Scholar 

  31. Sawada S, Sato Y, Aoyama H, et al. Pathological study of idiopathic portal hypertension with an emphasis on cause of death based on records of annuals of pathological autopsy cases in Japan. J Gastroenterol Hepatol. 2006;22:204–9.

    Article  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Gastroenterology, Funabashi Municipal Medical Center, Funabashi, Chiba, Japan

    Shoichi Matsutani

Authors
  1. Shoichi Matsutani
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Shoichi Matsutani .

Editor information

Editors and Affiliations

  1. Department of Advanced Gastrointestinal Endoscopy, Fukushima Medical University, Fukushima, Japan

    Katsutoshi Obara

Rights and permissions

Reprints and permissions

Copyright information

© 2019 Springer Nature Singapore Pte Ltd.

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Matsutani, S. (2019). Idiopathic Portal Hypertension. In: Obara, K. (eds) Clinical Investigation of Portal Hypertension. Springer, Singapore. https://doi.org/10.1007/978-981-10-7425-7_57

Download citation

  • DOI: https://doi.org/10.1007/978-981-10-7425-7_57

  • Published:

  • Publisher Name: Springer, Singapore

  • Print ISBN: 978-981-10-7424-0

  • Online ISBN: 978-981-10-7425-7

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation