1.A Case Peport of Hemphilia B (Christmas Disease).
Bae Sik LIM ; Jae Kyu LEE ; Dong Choon SHIN
Journal of the Korean Pediatric Society 1977;20(6):467-471
We have reported on a case of hemophilia B (Christmas disease) of 6 month old Korean who was have admitted to our hospital in duration of 7 days on Aug. 1974. Clinical study review of literature for a case were made briefly.
Hemophilia B
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Humans
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Infant
2.The first case report of a patient with coexisting hemophilia B and Down syndrome.
Pulkit RASTOGI ; Narender KUMAR ; Jasmina AHLUWALIA ; Reena DAS ; Inusha PANIGRAHI
Blood Research 2017;52(1):75-76
No abstract available.
Down Syndrome*
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Hemophilia A*
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Hemophilia B*
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Humans
3.Urgent craniectomy for subdural hematoma in the 8-month-old infant with unrecognized hemophilia B.
Jong Bun KIM ; Hyun Ju JUNG ; Kyong Shil IM ; Sang Woo HAN ; Sang Hun LEE
Korean Journal of Anesthesiology 2013;64(1):82-83
No abstract available.
Hematoma, Subdural
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Hemophilia A
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Hemophilia B
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Humans
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Infant
4.How I treat hemophilia with inhibitors.
Chinese Journal of Hematology 2019;40(10):801-803
7.Gene therapy for hemophilia B.
Zhang-biao LONG ; Guo-wei ZHANG ; Xiao-dong XI
Chinese Journal of Hematology 2013;34(9):809-811
9.Clinical characteristics of 520 children with hemophilia in a single medical center.
Min XUAN ; Rong-feng FU ; Feng XUE ; Yan-hui YANG ; Lei ZHANG ; Ren-chi YANG
Chinese Journal of Hematology 2013;34(11):913-917
<b>OBJECTIVEb>To analyze the clinical characteristics, diagnosis and treatment of pediatric hemophilia in single center over the decade.
<b>METHODSb>A retrospective study was conducted with 520 hemophilic children hospitalized in our medical center between January 2002 and December 2012.
<b>RESULTSb>All the patients were male including 438 hemophilia A (HA) and 82 hemophilia B (HB). There were significant differences in APTT between severe and mild- to moderate hemophilia (P<0.05). In pediatric HA and HB, delay time of diagnosis were 1.42 and 1.17 year, respectively. Children of 7-12 years were the largest population of visiting a doctor, and the spontaneous bleeding episode was the main cause. The most common hemorrhage site was soft tissue in early childhood, but joint was increasingly affected with age as children growth. All bleeding sites and frequencies were not associated with plasma factor level of patient (P>0.05). Knee and anKle were mainly involved in early child, while elbow and shoulder were involved increasingly in later childhood. Additionally, in HA and HB, inhibitor occurrence were 8.9%(19/214) and 12.8%(5/39), inducing 78.9%(15/19) and 40.0%(2/5) of high titer inhalator, and antiHCV-positive rate were 2.8%(11/397) and 2.5%(2/79), respectively.
<b>CONCLUSIONb>Our data highlights that delay in diagnosis and blood-borne infections were significantly reduced over the decade, but the development of inhibitor still remains a major challenge with wide-scale usage of factor in replacement therapy.
Adolescent ; Child, Preschool ; Hemophilia A ; Hemophilia B ; Humans ; Infant ; Male ; Retrospective Studies
10.A Case of Hemophilia B Diagnosed after Rhinosinus Surgery in 37 Year-Old Patient.
Korean Journal of Otolaryngology - Head and Neck Surgery 2018;61(6):308-311
Hemophilia is an X-linked recessive disorder, which is classified into hemophilia A, defined by factor VIII deficiency and hemophilia B, defined by factor IX deficiency. The usual clinical presentation is spontaneous bleeding and prolonged activated partial thromboplastin time in a person without history of a coagulation disorder. The severity of hemophilia describes how serious a problem is and has been defined by a traditional classification into three forms: severe, moderate, mild. Hemophilia has never been reported after a rhinosinus surgery in otorhinolaryngology in Korea, but we encountered a 37-year-old man with hemophilia B who had undergone a rhinosinus surgery. He had no bleeding tendency in the past nor a family history for bleeding. But the patient presented with continuous nasal bleeding for a few days after surgery. We report this case of hemophilia B diagnosed after rhinosinus surgery that was cured with Factor IX replacement therapy with a review of the relevant literature.
Adult
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Classification
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Endoscopy
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Epistaxis
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Factor IX
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Hemophilia A*
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Hemophilia B*
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Hemorrhage
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Humans
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Korea
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Otolaryngology
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Partial Thromboplastin Time