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 ; Humans ; Infant

No abstract available.
Hematoma, Subdural ; Hemophilia A ; Hemophilia B ; Humans ; Infant

No abstract available.
Down Syndrome* ; Hemophilia A* ; Hemophilia B* ; Humans

Factor VIII ; Hemophilia A ; Hemophilia B ; Humans

China ; epidemiology ; Hemophilia A ; epidemiology ; Hemophilia B ; epidemiology ; Humans ; Prevalence

China ; Hemophilia A ; therapy ; Hemophilia B ; therapy ; Humans ; Quality of Life

Genetic Therapy ; Hemophilia B ; therapy ; Humans

Anticoagulants ; pharmacokinetics ; Child ; Hemophilia A ; drug therapy ; Hemophilia B ; drug therapy ; Humans ; Precision Medicine

<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.

OBJECTIVE: To investigate the joint health status of patients with hemophilia treated on-demand and to analyze the incidence trend of hemophilic arthritis, so as to probide the scientific clinical data for furture study. METHODS: The clinical data of patients with hemophilia admitlted in Tianjin municipal first central hospital form March 2016 to October 2017 were collected, the basic information of patients was recorded; the joint health status was evaluated by using the hemophillia Joint Health Score (HJHS) 2.1; the life guality of patients was analyzed by using the MOS item short form-36 health survey, SF-36; the correlation of joint function with life guatity was analyzed by pearson correlation test. RESULTS: 196 ont of 210 patients with hemophila were treated on demand. The average age of patients was 27.81(2-73) years old, Among 196 patients, 189 was hemophilia A (96.43%) and 9 was hemophilia B (3.57). The patients without joint involvement, patients with 1 joint and 32 jionts involvement accounted for 3.57%, 11.72% and 84.71% respectively. The incidonce of involvement in elbow, knae and ankle joints was 71.93%, 80.61% and 82.91% respectively. The joint invlvement rate in patients with mild, intermediate and severe hemphilia accounted for 61.66%, 72.40% and 80.73% respectively. The mean HJHS in intermediate and severe henophilia patients was 23.59±17.02 scores and 26.69±17.68 scores respectively, there was no statistical difference (P>0.05). The joint fanction in hemophilia patients negatively correlated with life gnality of patients (r = 0.076). CONCLUSION The incidence of arthritis in hemophilia patients is high, and at least 1 joint has been involved in patients aged over 10 years old, moreover the multiple joint involvement exrsts in most patients. The impairment of joint function affects the routine behavious and activities.
Adolescent ; Adult ; Aged ; Child ; Child, Preschool ; Health Status ; Hemophilia A ; Hemophilia B ; Humans ; Middle Aged ; Young Adult