Hemodialysis initiation for a case of end-stage renal disease with hemorrhagic-acquired F13 deficiency

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  • 後天性血友病XIIIを伴う末期腎不全患者に対し透析療法を導入した経験
  • 症例報告 後天性血友病ⅩⅢを伴う末期腎不全患者に対し透析療法を導入した経験
  • ショウレイ ホウコク コウテンセイ ケツユウビョウ Ⅹ Ⅲ オ トモナウ マッキ ジンフゼン カンジャ ニ タイシ トウセキ リョウホウ オ ドウニュウ シタ ケイケン

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Abstract

For hemodialysis therapy in an 84-year-old woman with end-stage renal disease, arteriovenous shunt formation was performed in the left wrist. Because of massive postoperative bleeding with an unknown cause, she was admitted to our hospital and the arteriovenous fistula was closed. Blood platelet count, prothrombin time and activated-partial thromboplastin time were within the normal ranges at the time of hospitalization. An arteriovenous fistula was then created in the right wrist; however, massive bleeding from the surgical site occurred suddenly 1 day after surgery. We closed the arteriovenous fistula and suspected factor XIII (F13) deficiency. Her plasma F13 activity level was 17% (normal range: 70-140%), and she was diagnosed with hemorrhagic-acquired F13 deficiency. A continuous ambulatory peritoneal dialysis catheter was placed safely in the abdominal cavity along with a prophylactic infusion of factor XIII concentrate. A few weeks later, she developed pleuroperitoneal communication. Continuous ambulatory peritoneal dialysis was discontinued. Finally, in this patient, a permanent replacement catheter was inserted into the right subclavian vein along with prophylactic infusion of factor XIII concentrate. No critical problems were found, such as fatal bleeding or delay in wound healing at the insertion site. She has been receiving maintenance dialysis therapy without any significant problems.

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