| Item type |
[ELS]学術雑誌論文 / Journal Article(1) |
| 公開日 |
2017-05-24 |
| タイトル |
|
|
タイトル |
新規血友病A 患者に対する第VIII因子製剤を用いた緊急対応 : 2 乳児例報告 |
| タイトル |
|
|
タイトル |
Emergency Medical Treatment with Recombinant Factor VIII for Hemostatic Control of Newly Diagnosed Hemophilia A:Two Infantile Case Reports |
|
言語 |
en |
| 言語 |
|
|
言語 |
jpn |
| キーワード |
|
|
主題 |
重症型血友病A |
| キーワード |
|
|
主題 |
凝固第VIII因子製剤 |
| キーワード |
|
|
主題 |
活性化部分トロンボプラスチン時間 |
| 資源タイプ |
|
|
資源タイプ識別子 |
http://purl.org/coar/resource_type/c_6501 |
|
資源タイプ |
journal article |
| 雑誌書誌ID |
|
|
収録物識別子 |
AA00629581 |
| 論文名よみ |
|
|
タイトル |
シンキ ケツユウビョウ A カンジャ ニ タイ スル ダイ 8 インシ セイザイ オ モチイ タ キンキュウ タイオウ : 2 ニュウジ レイ ホウコク |
| 著者 |
市川, 純子
佐藤, 雄也
加納, 優治
奥谷, 真由子
今高, 城治
福島, 啓太郎
黒澤, 秀光
有阪, 治
Ichikawa, Junko
Sato, Yuya
Kano, Yuji
Okuya, Mayuko
Imataka, George
Fukusima, Keitaro
Kurosawa, Hidemitsu
Arisaka, Osamu
|
| 著者所属(日) |
|
| 著者所属(日) |
|
| 著者所属(日) |
|
| 著者所属(日) |
|
| 著者所属(日) |
|
| 著者所属(日) |
|
| 著者所属(日) |
|
| 著者所属(日) |
|
| 著者所属(英) |
|
|
|
en |
|
|
Department Of Pediatrics, Dokkyo Medical University School Of Medicine |
| 著者所属(英) |
|
|
|
en |
|
|
Department Of Pediatrics, Dokkyo Medical University School Of Medicine |
| 著者所属(英) |
|
|
|
en |
|
|
Department Of Pediatrics, Dokkyo Medical University School Of Medicine |
| 著者所属(英) |
|
|
|
en |
|
|
Department Of Pediatrics, Dokkyo Medical University School Of Medicine |
| 著者所属(英) |
|
|
|
en |
|
|
Department Of Pediatrics, Dokkyo Medical University School Of Medicine |
| 著者所属(英) |
|
|
|
en |
|
|
Department Of Pediatrics, Dokkyo Medical University School Of Medicine |
| 著者所属(英) |
|
|
|
en |
|
|
Department Of Pediatrics, Dokkyo Medical University School Of Medicine |
| 著者所属(英) |
|
|
|
en |
|
|
Department Of Pediatrics, Dokkyo Medical University School Of Medicine |
| 記事種別(日) |
|
|
内容記述タイプ |
Other |
|
内容記述 |
症例報告 |
| 抄録(日) |
|
|
内容記述タイプ |
Other |
|
内容記述 |
確定診断前に凝固第VIII因子(F VIII)製剤の緊急投与を要した重症型血友病A の2 乳児例を報告する.症例1 は10 か月男児.反復する嘔吐を主訴に入院した.左上肢に腫張を伴う筋肉内出血と硬膜外血腫を認めた.症例2 は10 か月男児.止血困難な口唇裂傷後の出血を主訴に来院し,重度の貧血を認めた.2 例ともプロトロンビン時間(PT)正常,活性化プロトロンビン時間(APTT)の著明な延長より,血友病を強く疑った.いずれも重篤な出血を認めたため,凝固因子活性値による確定診断を待たず,緊急でF VIII製剤の投与を試みた.投与後2 例ともAPTT は改善し,出血症状も改善した.後日2 例ともにF VIII活性が1%未満と判明し,重症型血友病A と確定診断された. |
| 抄録(英) |
|
|
内容記述タイプ |
Other |
|
内容記述 |
The cause of hemophilia A and B involves loss of factor VIII( FVIII) and factor IX( FIX), respectively. The hereditary form of this hemorrhagic disease is X-linked recessive. It is well established that the critical region for hemophilia A is localized on Xq28 and for hemophilia B on Xq27.1- 27.2. The initial symptom is bleeding in the mucous membranes often accompanied by intramuscular and intraarticular hemorrhage. The hemorrhages in the joints cause joint contracture as a sequela. The diagnosis of hemophilia is based on a normal bleeding and prothrombin time(PT)and prolonged activated partial thromboplastin time (APTT). We report here two infants of the severe typical from of hemophilia A who were treated with sufficient needed dosage of recombinant factor VIII before a final diagnosis was made. Case 1 was a 10-month-old boy. He was hospitalized for recurrent vomiting. He had intramuscular bleeding with swelling of the left shoulder and upper extremity. A head CT showed multiple epidural hematomas. Case 2 was a 10-month-old boy. He was sent to our hospital because of a lip laceration that did not stop bleeding, and he had severe anemia. We made the diagnosis of hemophilia A based on both their normal laboratory finding of PT and on the finding of extended APTT. Before confirming the decision diagnosis of hemophilia, we intravenously injected recombinant FVIII immediately, because of the severe hemorrhagic symptoms. After the therapy, both the APTT and hemorrhagic symptoms improved. These two cases were later confirmed as a severe infantile form of hemophilia A with less than 1 % factor VIII activity. |
| 書誌情報 |
Dokkyo journal of medical sciences
巻 42,
号 2,
p. 83-87,
発行日 2015-07-25
|
| ISSN |
|
|
収録物識別子タイプ |
ISSN |
|
収録物識別子 |
03855023 |
KJ00009895942.pdf (306.4 kB)
