A Young Hemophilia Patient with Advanced Hemophilic Arthritis and HIV Infection / 罕见病研究

A young male diagnosed with severe hemophilia A since childhood, was presented with recurrent joint and urinary bleeding. Annualized bleed rates dropped below five with low dose prophylactic medication.Bleeding in the right knee joint recently aggravated. Due to coexisting HIV infection and advanced hemophilic arthritis, the patient was managed by a multi-disciplinary team(MDT).Total knee arthroplasty was performed by an experienced surgeon using modern prosthesis design and intraoperative navigation technologies.Physical and rehabilitation therapy was provided during the postoperative period, and joint function improved. The MDT managed the young patient with HIV infection and advanced hemophilic arthritis. The patient was diagnosed with osteoporosis thought to have been caused by hemophilia, HIV infection and antiviral drugs; and he received treatment. The treatment of this patient reflects the importance of multidisciplinary cooperation in the management of difficult and rare diseases.

Factors influencing the joint health of haemophilic children / 中华物理医学与康复杂志

Objective To explore the joint health of children with haemophilia and its influencing factors.Methods Sixty boys with haemophilia (aged 4-18 years) were recruited from Peking Union Medical College Hospital.They were divided by age into a five to seven year-old group (group A,n=16),an eight to twelve year-old group (group B,n =18) and a thirteen to eighteen year-old group (group C,n =26).The haemophilia joint health score (HJHS) (version 2.1) was used to evaluate the condition of their elbows,knees and ankles.Spearman's method was applied to analyze the influencing factors.Results The average HJHSs of the knees,elbows and ankles of all of subjects were 8.65±6.41,5.30±5.30,and 5.40±4.25 respectively.The average for the knees was significantly higher than for the elbows and ankles.The average HJHS for the elbows of group A was significantly lower than that of their knees.The average HJHS for their knees was not significantly different from that of their ankles.For those aged 13-18 years,the average HJHS for their knees was significantly higher than that for their elbows and ankles.There was no significant difference between the HJHSs for the elbows,knees and ankles of the 8-12 year-olds.The HJHSs were positively correlated with age but negatively correlated with average therapeutic dose and exercise.Conclusion The joint health of haemophilic boys,especially that of the knees,decreases with age.Their joint health can be improved by medication and by taking reasonable physical exercise.