Idiopathic Portal Hypertension in a Patient with Mixed Connective Tissue Disease and Protein C Deficiency
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...
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| Veröffentlicht in: | Internal Medicine 2010, Vol.49(18), pp.2013-2016 |
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| container_end_page | 2016 |
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| container_issue | 18 |
| container_start_page | 2013 |
| container_title | Internal Medicine |
| container_volume | 49 |
| creator | Horai, Yoshiro Miyamura, Tomoya Hirata, Akie Nakamura, Masataka Takahama, Soichiro Ando, Hitoshi Minami, Rumi Yamamoto, Masahiro Suematsu, Eiichi |
| description | 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. |
| doi_str_mv | 10.2169/internalmedicine.49.3834 |
| format | Article |
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Med.</addtitle><description>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. 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| subjects | Adult Female Humans Hypertension, Portal - complications Hypertension, Portal - diagnosis idiopathic portal hypertension Liver Cirrhosis - complications Liver Cirrhosis - diagnosis mixed connective tissue disease Mixed Connective Tissue Disease - complications Mixed Connective Tissue Disease - diagnosis Pancytopenia - complications Pancytopenia - diagnosis protein C deficiency Protein C Deficiency - complications Protein C Deficiency - diagnosis Splenomegaly - complications Splenomegaly - diagnosis |
| title | Idiopathic Portal Hypertension in a Patient with Mixed Connective Tissue Disease and Protein C Deficiency |
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