الفهرس 
HEMOPHILIC ARTHROPATHYOnly 14 pages are availabe for public view
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Abstract
Hemophilic arthropathy is the most debilitating complication of hemophilia resulting in chronic joint pain and diminished range of motion that requires multidisciplinary approach of management.
Non selective non steroidal anti-inflammatory drugs (ns-NSAID) are analgesic, anti-inflammatory drugs used for different types of arthropathies with limited usage in hemophilia for its high risk of GIT toxicities.
Selective COX-2 inhibitor celecoxib (Celebrex ®) is an analgesic, anti-inflammatory and anti-angiogenic agent with low GIT toxicity.
This cross sectional study was conducted on 50 patients with hemophilia A and B followed at pediatric hematology clinic, Ain Shams University. Patients were randomly divided into 2 equal groups. group A: patients who received factor replacement therapy and oral celecoxib on 3 mg/kg/day for 30 days and group B patients who did not receive Celebrex with their factor replacement therapy.
We aimed to determine the efficacy of celecoxib in hemophilic pain control which was judged by pain score, collected data using Haemo-QOL, joint health by HJHS and assessing joint structures through US and the conventional Petterson score as well as celecoxib safety through blood pressure, heart rate monitoring, ECG, bleeding time and occult blood in stool done at study entry and at day 30.
Our study showed no significant difference between study groups A and B of hemophilics as regards demographic data and (BMI). Also; heart rate and blood pressure showed no difference at day 1 and day 30.
Occurrence of target joint did not differ between both groups with hemophilia. Highly significant improvement in the ABR among groups on prophylaxis therapy and on demand therapy group who received Celebrex with improved school absenteeism among both study groups with hemophilia receiving prophylaxis therapy and those on on demand therapy who received Celebrex.
HJHS showed no significant difference between group A and B at study entry and 6 months later. However, significant difference as regards subjective feeling of pain (but not degree of joint deformity) was found among patients of group A on prophylactic therapy (before and after adding Celebrex) (p=0.005), improved HJHS not reaching statistical significance among group A receiving on demand therapy (p=0.088), significant improvement among group B patients on prophylactic therapy (p=0.026) while worsening of HJHS among group B patients over on demand therapy (p=0.058).
Subjective pain score assessment showed no significant difference between hemophilia patients in groups A and B at study entry (day 1), however, highly significant lowering was observed in the pain score among group A patients at day 3 with median (IQR) 1(1-2), highly significant lowering was observed in the study group A at day 7 and 30 with median (IQR) 1(1-2) in group A and p value <0.001. Among group A patients; 60.0% (n=15) showed complete response at day 30 of Celebrex intake with range of pain score at study entry day 1 (1-2) with 4 cases with target joint, 24 % (n=6) showed partial response with pain score at study entry (3-4) and 5 cases with target joint and 16% (n=4) showed no response with pain score at study entry (4-6) and 4 cases with target joint.
Haemo-Qol showed highly significant difference among the study groups A and B with hemophilia at time of study entry with patients on prophylaxis therapy with the lowest scores. However, highly significant difference was found 12 months later with mean difference of -7.82±0.84 in group A patients on prophylactic factor replacement therapy, -3.38 ± 1.44 in group B patients on prophylactic therapy, -1.50 ± 0.86 in group A patients on demand therapy as participants recall improved pain and physical health along the one month period and 2 week later of celecoxib intake however other domains where improved only in groups of prophylactic therapy.
In our study, US joint score showed no significant difference among study groups A and B with hemophilia at study entry, and no change was reported 6 months later.
Pettersson score by X-ray showed no significant difference among study groups A and B at time of study entry and 6 months later.
Vasoactive Endothelial Growth Factor (VEGF) level did not show significant difference among study groups A and B with hemophilia at time of study entry, with highly significant owering at day 30 after intake of Celebrex in patients of group A (p value <0.001).
Our study showed no difference in Electrocardiogram (ECG) at study entry, day 30 and 6 months later, bleeding time, occult blood in stool and presence of symptoms of gastritis at study entry and at day 30.
Conclusion
Oral celecoxib can be considered an effective and safe, analgesic, anti-inflammatory and anti-angiogenic adjuvant therapy in hemophilic arthropathy that leads to improvement of joint pain, arthropathy, hence quality of life, when combined with regular continuous prophylactic factor replacement. However, it cannot reverse preexisting radiological joint structural changes.