Comparison of quality of life between hemophilic children receiving prophylaxis and on demand therapy in Tehran

Improving quality of life in a chronic disease such as hemophilia is a major goal of treatment. Hemophilic patients can be treated either prophylactically or on demand. Since haemophilia quality of life may be affected by the disease and treatment outcomes, comparison of these regimens in respect to quality of life outcomes provides useful information. The purpose of this study is to compare the quality of life between hemophilic children receiving prophylactic and on demand therapy in Tehran, Iran. A descriptive -comparative design was used. The sample of the study consisted of 60 hemophilic children which were recruited from haemophilia treatment centres in Tehran. Of these children 30 were receiving prophylactic therapy and 30 of them were treated on-demand .Data was collected by haemophilia-specific QoL questionnaire [HAEMO-QoL]. The questionnaire consists of 8 dimensions: physical health, feeling, view of yourself, family, friends, others, sport and dealing with haemophilia.The questionnaire consists of 18 items using a5-point scale.The Higher scores represent a poorer quality of life and lower scores represent better quality of life. Data were analyzed by SPSS 15 statistical software. The mean age of children was 5.3 +/- 1.2 and 6 +/- 1 in prophylactic and on demand therapy respectively. The mean total score of quality of life was 40 +/- 9.8 and 58 +/- 10.9 for children in prophylactic therapy and on-demand treatment respectively. There was a significant difference between the quality of life scores in two groups. Analysis of subscale scores showed a lower quality of life score for family domain in prophylactic therapy group, while the scores for family and physical health subscsles in on-demand group showed a poor quality of life. Results of the study suggest that quality-of-life may be better for haemophilia children treated prophylactically

Social consequences of infected haemophilia cases in the Islamic Republic of Iran

The unintentional contamination of haemophilia patients with HIV in the early 1980s raised serious questions about the safety of blood product supplies worldwide. The events initiated a cascade of consequences for both infected patients and the national health systems of many countries, including the Islamic Republic of Iran. Lawsuits have been filed in the courts mostly in developed countries, leading to the establishment of some kind of reimbursement programme for haemophilia patients who acquired viral infections. In the late 1990s the courts ordered the Iranian Ministry of Health, in addition to providing free care with the latest treatments, to pay compensation to the haemophilia patients. The adverse consequences of these events on the equitable distribution of resources in the Iranian health care system are discussed in this paper

[Evaluasion and comparison of washed RBCs by closed and open systems]

Leukocyte filters are effective for WBC reduction but they cannot inhibit passing plasma proteins and as a result repeated protein entry may produce allergic transfusion reactions. To deal with this problem, washed RBC method is used. The traditional wash method is an open system through which waste products are carried away in sewers with the risk of environmental pollution. Newly introduced approach for washed RBCs consists of a closed system whereby waste products enter into a bag. In this study, the two methods were compared. The Two open and close wash methods were compared in terms of health system, leukoreduction, risk of transmission of infection and quality control. In each method, 100 bags were washed, coded and then transmitted to different units of blood culture, flowcytometry as well as quality control. The data were collected and analyzed by SPSS14. 200 bags [100 for each method] were studied. Microbiologically, there were no positive results for any of the methods. In quality control also there was not any significant difference in the two methods. In flowcytometry, we didn.t observe any significant correlation in leukocyte count in the two methods before washing [p=0.072], however.there was correlation between them after washing [p<0.0001], demonstrating that the new method was better for leukoreduction. The new washing system method was a superior way because it involves a close system where waste products are discharged into a side bag and disposed as hospital waste. Meanwhile, this approach is more convenient for leukoreduction. In our country, since we still need a washing system for some transfusions, this method is deemed to be a decent and practical one because it impedes environmental pollution

Evaluation and comparison of washed RBCs by closed and open systems

Leukocyte filters are effective for WBC reduction but they cannot inhibit passing plasma proteins and as a result repeated protein entry may produce allergic transfusion reactions. To deal with this problem, washed RBC method is used. The traditional wash method is an open system through which waste products are carried away in sewers with the risk of environmental pollution. Newly introduced approach for washed RBCs consists of a closed system whereby waste products enter into a bag. In this study, the two methods were compared. The two open and closed wash methods were compared in terms of health system, leukoreduction, risk of transmission of infection and quality control. In each method, 100 bags were washed, coded and then transmitted to different units of blood culture, flowcytometry as well as quality control. The data were collected and analyzed by SPSS 14. 200 bags [100 for each method] were studied. Microbiologically, there were no positive results for any of the methods. In quality control also there was not any significant difference in the two methods. In flowcytometry, we didn't observe any significant correlation in leukocyte count in the two methods before washing [p=0.072], however. The correlation between them after washing [p<0.0001], demonstrating that the new method was better for leukoreduction. The new washing system method was a superior way because it involves a closed system where waste products are discharged into a side bag and disposed as hospital waste. Meanwhile, this approach is more convenient for leukoreduction. In our country, since we still need a washing system for some transfusions, this method is deemed to be a decent and practical one because it impedes environmental pollution

Efficacy and safety of Iranian made deferasirox [Osveral]in Iranian major thalassemic patients with transfusional iron overload: a one year prospective multicentric open-label non-comparative study

To determine the efficacy, adverse effects and safety of a new Iranian generic product of deferasirox [Osveral] in Iranian transfusion dependent major thalassemic [TD-MT] patients. In 9 main thalassemia treatment centers, all of TD-MT patients [aged >/= 2 yrs] with serum ferritin [SF] levels >/= 1000 ng/ml, or >100 ml/kg of RBC transfusion, who could not tolerate parental iron chelating were recruited regardless of their previous iron chelation therapy. Periodical clinical and laboratory evaluations were conducted for adverse effects [AEs]. Primary efficacy end point was Mean of Relative Change of Serum Ferritin [MRC-SF] from the baseline level during one year. Analysis of variance [ANOVA], t test, chi-square or Fisher exact test were used for statistic analysis appropriately [P values <0.05 were considered as statistical significant]. In 407 cases the male/female ratio was 0.98. Mean age was 11.5 +/- 7.4 [2-58] years. The mean of initiating dose of Osveral and mean usage dose during the study was 23.5 +/- 4.9 mg/kg and 24.9 +/- 4.9 mg/kg respectively. MRC-SF was -11.44% +/- 38.92 and it showed significant decline in SF [P value<0.001] one hundred and forty eight patients out of 407 patients experienced at least one. AE, the most common of them were transient increase in serum creatinin [97;24.1%] and > 5 time increase in transaminases [24;5.89%].The causes of discontinuation of treatment were non-satisfactory treatment [24; 5.8%], poor or non-compliance of patients [21;5.1%], and adverse effects [13; 3.1%]. A detailed comparison with similar studies on deferasirox [Exjade] shows a promising efficacy and safety for its Iranian generic product [Osveral]

Efficiency of BclI restriction fragment length polymorphism for detection of hemophilia a carriers in Sistan and Baluchestan Province, Southeast of Iran

Indirect genetic diagnosis using polymorphic DNA markers can detect carriers of hemophilia A. This technique is preferable in developing countries because of its simplicity and cost effectiveness compared to direct mutation analysis. In the present study, we examined usefulness of intragenic marker BclI restriction fragment length polymorphism [RFLP] at intron 18, for carrier detection. How this marker is informative was tested in 102 members of 16 hemophiliac families from Sistan and Baluchestan province, Southeast of Iran. Blood samples were obtained from 29 hemophilia A patients and 73 of their relatives, after taking informed consents. DNA was extracted using proteinase K digestion followed by DNA precipitation. Factor VIII gene polymorphism was identified by the polymerase chain reaction/RFLP which is both sensitive and economical. Our results showed that almost 69.8% of Xchromosomes had restriction site for BclI enzyme. The heterozygosity rate for BclI polymorphism in tested women was 61.4%, signifying the usefulness of this marker in carrier detection. The informative rate respecting this polymorphism was 43.7% meaning that a remarkable percent of families from the target population could be diagnosed using this marker alone. In Sistan and Baluchestan province where there is limited access to sophisticated facilities of molecular diagnosis, use of PCR-based analysis of DNA polymorphism in the BclI locus can be used to identify a remarkable percentage of the carriers and even for prenatal diagnosis. Meanwhile, it is necessary to evaluate the effectiveness of other polymorphic DNA markers to enhance the informative rate

Ocular side effects of desferal in patients with beta thalassemia

To evaluate the ocular side effects of desferal in beta-thalassemia major patients. This cross-sectional study was conducted on 100 beta-thalassemia major subjects. All patients underwent complete ophthalmic examination, color vision test, and visual field evaluation. Electroretinography [ERG] was performed for 50 patients. Mean patient age was 14.4 years. Only 9 patients complained from ocular problems including nyctalopia [8 subjects] and permanent visual loss [one case], but eye examination revealed abnormalities in 74 subjects including cataract in 43, fundus abnormality in 9; abnormal visual field in 71 with the most common abnormality being paracentral scotoma [64%], and abnormal color vision in 35 with the most common abnormality being tritanopia [81%]. All patients had abnormal ERG with abnormal amplitude of "a" and "b" waves in scotopic phase. There was no significant correlation between age, duration of desferal usage or serum ferritin with ocular findings. All patients who received more than 40 mg/kg/day of desferal had ocular abnormalities. The main risk factor for ocular finding in desferal users is the dosage- ERG and perimetry are useful for early detection of ocular problems in these patients

[Classification arthropathies with severe hemophilia type A referred to Ali Asghar pediatric in Zahedan in 2003]

Hemarthrosis is the most common sign of severe hemophilia type A in all ages which leads to irreversible complications in joints. These Complications cause morbidity and high expenses that could be decreased by a good organized therapeutic and prophylactic management. There is not any standard quantitative scoring system to evaluate the complications of joints, used in our country systematically and we have not any published data about it. In this study, Petterson and Gilbert's scoring system has been used to evaluate joints status in a cross sectional descriptive analytic manner. Twenty out 32 cases of the severe hemophilia type A patients who came to Ali Asghar hospital in 1381, were included on our study. Based on Petterson and Gilbert's scoring system, knees, ankles and elbows were selected joints. Physical examination was first done by a pediatrician and confirmed by an orthopedic surgeon PA and lateral X-ray of selected joints were reported by a radiologist .All of the above physicians were fix during the study. Finally we reported our results in tables and charts and analyzed with SPSS [One-way Analysis of Variance, Wilcoxon Matched-Pairs, Kruskal-Wallis]. In this study, the most common radiological pathologic finding was osteoporosis [32.5%] and the rarest one was erosion at joint margin [3.3%]. In physical examination, decreasing in range of motion [35%] and instability [0%] was the most common and the rarest pathologies. The most involved joints in radiology and physical exam were right knee [28.7%] and left knee [19.3%] and the rarest one's were left ankle [13.7%] and left elbow [10.6%]. The severity of complications [both physically and radiologically] has been related with the titer of inhibitor, but there was not any correlation with age [one-way analysis of variance]. There was not any statistically significant difference between radiologic and physical scoring in every joint. Ourfindings showed that the majority of our patients had at least one joint involvement [85% physically and 75% radiologically].On the other hand, 45% of 120 evaluated joints had radiological problems and 55% of them were physically insult. This high prevalence of morbidity explained the cause of high expenses for treatment of patients in this province. To increase in titer of inhibitor is the main factor for increasing of morbidity. Considering high morbidity in our patients, the statistical similarity between joints complications in different age groups, showed the importance of prophylaxis protocols for them obviously. It seems there is no relation between radiological scoring and physical score, except for left elbow joint [Wilcoxon Matched-pairs, P=0.454]. Pain is a good alarm, but it had no effect on scores. Prevalence of involved joints can be dependent on kind of culture