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

doi:10.2169/internalmedicine.49.3834

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

Research output: Contribution to journal › Article › peer-review

3 Citations (Scopus)

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.

Original language	English
Pages (from-to)	2013-2016
Number of pages	4
Journal	Internal Medicine
Volume	49
Issue number	18
DOIs	https://doi.org/10.2169/internalmedicine.49.3834
Publication status	Published - 2010
Externally published	Yes

All Science Journal Classification (ASJC) codes

Internal Medicine

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10.2169/internalmedicine.49.3834

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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.",

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AU - Horai, Yoshiro

AU - Miyamura, Tomoya

AU - Hirata, Akie

AU - Nakamura, Masataka

AU - Takahama, Soichiro

AU - Ando, Hitoshi

AU - Minami, Rumi

AU - Yamamoto, Masahiro

AU - Suematsu, Eiichi

PY - 2010

Y1 - 2010

N2 - 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.

AB - 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.

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JO - Internal Medicine

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ER -

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

Abstract

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