| H01686 | |
| H番号 | H01686 |
| 名称 | 特発性門脈圧亢進症; 非硬変症性門脈線維症; バンチ症候群 |
| 概要 | Idiopathic portal hypertension (IPH) is a disorder generally classified as a noncirrhotic portal hypertension of unknown etiology, and is clinically characterized by portal hypertension, splenomegaly, and pancytopenia. The principal pathologic changes of IPH are devastation of the intrahepatic terminal portal radicles with considerable portal fibrosis and secondary atrophy of the liver parenchyma that follows portal thrombosis. The incidence varies considerably with the country. In contrast to its high prevalence in India, IPH is comparatively a rare disorder in Western countries. Middle-aged women are more prone to IPH in Japan. IPH usually suggests a benign prognosis, but sometimes is complicated with severe hemorrhage due to ruptured esophageal varices, or massive splenomegaly. It is mainly managed by supportive treatment such as endoscopic, radiological and/or surgical management for esophageal varices and/or splenomegaly. The definite etiology of IPH is still uncertain, but there are several theories on the potential pathogenesis of IPH. These theories include immunological disorders, infections, and genetic variants. |
| カテゴリ | 消化器系疾患 |
| ネットワーク | - |
| 病因遺伝子 | - |
| 病原体 | - |
| 環境要因 | - |
| 発癌物質 | - |
| 治療薬 | - |
| コメント | Japanese study groups usually use the term of "IPH", whereas Indian researchers prefer to use the term non-cirrhotic portal fibrosis for the name of this disorder. Groups in Western countries have proposed terms such as nodular regenerative hyperplasia (NRH), hepatoportal sclerosis, or Idiopathic noncirrhotic portal hypertension. |
| リンク | ICD-11: DB98.70 ICD-10: K76.6 MeSH: C537903 |
| 文献 | PMID:25343110 著者 Tanaka T, Sugawara Y, Kokudo N タイトル The current clinical aspects of idiopathic portal hypertension. 雑誌 Intractable Rare Dis Res 2:94-7 (2013) DOI:10.5582/irdr.2013.v2.3.94 PMID:11928079 著者 Okudaira M, Ohbu M, Okuda K タイトル Idiopathic portal hypertension and its pathology. 雑誌 Semin Liver Dis 22:59-72 (2002) DOI:10.1055/s-2002-23207 |