الفهرس 
HEMOPHILIAOnly 14 pages are availabe for public view
 |  | from | 32 |  |  |
| | | from | 32 | | |
Abstract
H
emophilia is a hereditary bleeding disorder and There are two types of hemophilia, hemophilia A and hemophilia B. Hemophilia is traditionally classified as ‘mild’, ‘moderate’, or ‘severe’, depending on the degree of clotting factor deficit (Canadian Hemophilia Society, 2009).
Hemarthrosis accounting for more than 90% of all serious bleeding events in patients with severe hemophilia (Pergantou et al., 2006).
Over time, complications from recurrent hemarthrosis, and soft-tissue hematomas can result in severe arthropathy (Gringeri et al., 2011).
Arthropathy as a consequence of hemophilia represents the single largest cause of morbidity in patients with hemophilia (Manco et al., 2007).
Low bone mineral content or bone mineral density is defined as a BMC or areal BMD Z-score that is less than or equal to -2.0, adjusted for age, gender and body size, as appropriate (The International Society for Clinical Densitometry, 2007).
Prolonged immobilization and the reduction in weight bearing activities play an important role in the pathogenesis of osteopenia in these patients (Nair et al., 2007).
Our study included twenty patients were suffering from hemophilia include male patients with age ranging from 4-18 years.19 patients with hemophilia A and 1 patient with hemophilia B.
All patients were assessed for BMD using DXA scan interpretation done by Z -score, clinical assessment was done using Hemophilia joint health score (HJHS) and functional assessment was done by functional independence score of hemophilia (FISH).
BMD showed (-4.0 to 0) however BMD of -2 or less is considered low bone mineral density in children, there was highly significant negative correlation with HJHS (p=0.0001) and highly significant positive correlation with functional score (p=0.002). There was significant negative correlation between number of attacks& DXA (p=0.035). This indicates better BMD with less severe disease and less joint affection and better functional performance.
The HJHS total score ranges between (11- 73) however highest score (124).
There was +ve significant correlation between each of age (p=0.030) degree of factor deficiency (p=0.025), and number of attacks (p=0.016) with HJHS score, while a negative significant correlation was detected total function score and HJHS (p=0.0001). This indicates increase in age, and severity of hemophilia causes more severe joints affection.
Total FISH score ranged between (8-32) while the normal value is 32. There was highly significant negative correlation between number of attacks (p=0.0001) & degree of deficiency (p=0.002) and total functional score, also there is highly significant correlation between type of treatment and total functional score (p=0.017). This indicates that severity of hemophilia affects the functional state of hemophiliacs.
from the previous results we conclude that, results of DXA showed significant positive correlation with functional score and a significant negative correlation with total HJHS and number of attacks of bleeding. In patients with severe hemophilia number of attacks of joint bleeding is the main contributor to arthropathy which is main cause for reduced bone mass in the hemophiliacs, most probably the hemophilic arthropathy which is typically associated with chronic pain and loss of joint function often already from an early age subsequently leading to inactivity so prophylactic treatment is an important protector of joints as it decreases the bleeding frequency and hence protect against osteoporosis in children also supplementation of calcium and vitamin D in hemophilic children also help in protecting those children from osteoporosis.