Idiopathic portal hypertension in a patient with mixed connective tissue disease and protein C deficiency

Yoshiro Horai, Tomoya Miyamura, Akie Hirata, Masataka Nakamura, Soichiro Takahama, Hitoshi Ando, Rumi Minami, Masahiro Yamamoto, Eiichi Suematsu

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We report a 29-year-old woman with a 2.5 year history of mixed connective tissue disease (MCTD) who developed idiopathic portal hypertension (IPH) and thrombocytopenia as a result of hypersplenism. She had recurrent esophagogastric variceal rupture. Hematological examination also revealed low levels of protein C activity. The liver biopsy specimen showed non-specific mild inflammation and no thrombi. However, portal vein thrombosis developed after splenectomy. This was a rare case of severe complications of IPH accompanying MCTD and protein C deficiency.

Original languageEnglish
Pages (from-to)2013-2016
Number of pages4
JournalInternal Medicine
Issue number18
Publication statusPublished - Oct 21 2010
Externally publishedYes


All Science Journal Classification (ASJC) codes

  • Internal Medicine

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