[Clinical analysis of hereditary coagulation factor â
¤ deficiency].
Zhonghua Yi Xue Za Zhi
; 98(36): 2920-2924, 2018 Sep 25.
Article
em Zh
| MEDLINE
| ID: mdl-30293350
Objective: To analyze the clinical characteristics of hereditary coagulation factor â
¤ deficiency (Fâ
¤D) and to improve the diagnosis and treatment ability of hereditary Fâ
¤D. Methods: A total of 17 patients with hereditary Fâ
¤D admitted to the Department of Hematology, Peking University People's Hospital from February 2013 to January 2018 were selected, and their clinical characteristics, laboratory examination, treatment and prognosis were retrospectively analyzed. Results: There were 9 males and 8 females patients with Fâ
¤D, the median age was 36 (1-72 ) years. The median age of men was 39 (1-72)years, and the median age of women was 33 (8-56)years. There was no significant difference between them (P=0.793). The median prothrombin time(PT) and activated partial thromboplastin time(APTT) values were 21.0(13.0-39.6) s and 54.6(38.2-121.2) s, and the median level of plasma Fâ
¤ was 8.2% (0.9%-39%). Thirteen cases (13/17) were mild, 3 cases (3/17) were moderate, and only 1 case (1/17) was severe according to the Fâ
¤ level. Five cases (5/17) had bleeding, including 3 cases with skin ecchymosis, 1 case with vaginal bleeding, and 1 case with gastrointestinal bleeding. According to the severity of bleeding, 1 case (1/17) had severe bleeding, and the other 4 cases (4/17) were mild bleeding. Conclusions: Patients with hereditary Fâ
¤D either have or do not have bleeding symptoms, with prolonged PT and APTT and decreased plasma of Fâ
¤ activity. There is no need for treatment for those without bleeding symptoms. Fresh frozen plasma is the first choice for patients who have bleeding, and the overall prognosis is good.
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Texto completo:
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Base de dados:
MEDLINE
Assunto principal:
Tempo de Tromboplastina Parcial
/
Transtornos da Coagulação Sanguínea
Tipo de estudo:
Observational_studies
/
Prognostic_studies
Limite:
Adolescent
/
Adult
/
Aged
/
Animals
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Child
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Child, preschool
/
Female
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Humans
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Infant
/
Male
Idioma:
Zh
Ano de publicação:
2018
Tipo de documento:
Article