• Internal medicine · Jan 2010

    Review Case Reports

    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, and Eiichi Suematsu.
    • Department of Internal Medicine and Rheumatology, Clinical Research Center, National Hospital Organization Kyushu Medical Center, Fukuoka. yoshirohorai0518@qmed.hosp.go.jp
    • Intern. Med. 2010 Jan 1; 49 (18): 201320162013-6.

    AbstractWe 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.

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