Idiopathic Portal Hypertension in a Patient with Mixed Connective Tissue Disease and Protein C Deficiency

被引：4

作者：

Horai, Yoshiro ^{[1
]}

Miyamura, Tomoya ^{[1
]}

Hirata, Akie ^{[1
]}

Nakamura, Masataka ^{[1
]}

Takahama, Soichiro ^{[1
]}

Ando, Hitoshi ^{[1
]}

Minami, Rumi ^{[1
]}

Yamamoto, Masahiro ^{[1
]}

Suematsu, Eiichi ^{[1
]}

机构：

[1] Natl Hosp Org Kyushu Med Ctr, Clin Res Ctr, Dept Internal Med & Rheumatol, Fukuoka, Japan

来源：

INTERNAL MEDICINE | 2010年 / 49卷 / 18期

关键词：

idiopathic portal hypertension; mixed connective tissue disease; protein C deficiency;

D O I：

10.2169/internalmedicine.49.3834

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

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.

引用

页码：2013 / 2016

页数：4

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