title: Idiopathic portal hypertension in a patient with mixed connective tissue disease and protein C deficiency.
abstract: 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.
authors.affiliation: Department of Internal Medicine and Rheumatology, Clinical Research Center, National Hospital Organization Kyushu Medical Center, Fukuoka. yoshirohorai0518@qmed.hosp.go.jp
authors.forename: Yoshiro
authors.initials: Y
authors.lastname: Horai
authors.forename: Tomoya
authors.initials: T
authors.lastname: Miyamura
authors.forename: Akie
authors.initials: A
authors.lastname: Hirata
authors.forename: Masataka
authors.initials: M
authors.lastname: Nakamura
authors.forename: Soichiro
authors.initials: S
authors.lastname: Takahama
authors.forename: Hitoshi
authors.initials: H
authors.lastname: Ando
authors.forename: Rumi
authors.initials: R
authors.lastname: Minami
authors.forename: Masahiro
authors.initials: M
authors.lastname: Yamamoto
authors.forename: Eiichi
authors.initials: E
authors.lastname: Suematsu
mesh_terms: D000328:Adult; D005260:Female; D006801:Humans; D006975:Hypertension, Portal / Q000150:complications / Q000175:diagnosis; D008103:Liver Cirrhosis / Q000150:complications / Q000175:diagnosis; D008947:Mixed Connective Tissue Disease / Q000150:complications* / Q000175:diagnosis*; D010198:Pancytopenia / Q000150:complications / Q000175:diagnosis; D020151:Protein C Deficiency / Q000150:complications* / Q000175:diagnosis*; D013163:Splenomegaly / Q000150:complications / Q000175:diagnosis; D000094724:Idiopathic Noncirrhotic Portal Hypertension