A Case of Congenital Factor XIII Deficiency With Umbilical Hemorrhage in a Neonate

• Akiyoshi Junko

  Departments of Pediatric Surgery

• Suita Sachiyo

  Departments of Pediatric Surgery

• Taguchi Tomoaki

  Departments of Pediatric Surgery

• Yamanouchi Takeshi

  Departments of Pediatric Surgery

• Suga Naohiro

  Departments of Pediatrics, Graduate School of Medical Sciences, Kyushu University

• Nomura Akihiko

  Departments of Pediatrics, Graduate School of Medical Sciences, Kyushu University

• Ohga Shoichi

  Departments of Pediatrics, Graduate School of Medical Sciences, Kyushu University

• Hara Toshiro

  Departments of Pediatrics, Graduate School of Medical Sciences, Kyushu University

Bibliographic Information

Other Title

• 臍出血に対し外科的処置を繰り返した先天性XIII因子欠損症の1新生児例
• 臍出血に対し外科的処置を繰り返した先天性13因子欠損症の1新生児例
• サイシュッケツ ニ タイシ ゲカテキ ショチ オ クリカエシタ センテンセイ 13 インシ ケッソンショウ ノ 1 シンセイジレイ

Abstract

A seven-day-old girl manifested umbilical hemorrhage after cord-drop. She was treated by coagulation therapy using AgNO_3 several times ; however, it failed. She was admitted to our institute on the eighth day after birth. Routine screening tests for blood coagulation, such as platelet count, bleeding time, PT and APTT were within normal limits. She underwent surgical treatment, including ligations of umbilical vessels, two times on day eight and day 10 after birth. Once hemostasis was obtained, however, hemorrhage started again. Examination of factor XIII activity showed 0%. Umbilical hemorrhage was well controlled by replacement of factor XIII concentrates. The roles of factor XIII (fibrin-stabilizing factor) are stabilizing clotting and cross-linking of the fibrin polymer. The most common symptom of factor XIII deficiency is delayed umbilical hemorrhage, while there are some reports of patients who died of intracranial hemorrhage. Although factor XIII deficiency shows no abnormalities in the routine screening tests for blood coagulation, delayed umbilical bleeding should be recognized as an important sign for early diagnosis.

Journal

• Journal of the Japanese Society of Pediatric Surgeons

  Journal of the Japanese Society of Pediatric Surgeons 41 (1), 18-22, 2005

  The Japanese Society of Pediatric Surgeons

Keywords

• Factor XIII
• Deficiency
• Umbilical hemorrhage
• Intracranial hemorrhage
• Neonate

Details

• CRID: 1390001204822897152
• NII Article ID: 110002120453
• NII Book ID: AN00192281
• DOI: 10.11164/jjsps.41.1_18
• ISSN: 21874247
• ISSN: 0288609X
• NDL BIB ID: 7260683
• Web Site: https://ndlsearch.ndl.go.jp/books/R000000004-I7260683
• Web Site: https://search.jamas.or.jp/link/ui/2005188878
• Text Lang: ja
• Data Source:
  • JaLC
  • NDL
  • CiNii Articles
  • KAKEN
• Abstract License Flag: Disallowed