Clinical audit of inherited bleeding disorders in a developing country

Sajid, Muhammad Raihan; Khalid, Safoorah; Mazari, Nazish; Azhar, Waleed Bin; Khurshid, Mohammad

doi:10.4103/0377-4929.59183

Cited by 20 publications

(24 citation statements)

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“…In other words this may be a sporadic finding. These findings are contrary to previous studies [11][12][13][14][15][16][17][18][19][20]. Probably further studies are needed to clarify this issue.…”

Section: Discussioncontrasting

confidence: 85%

Bleeding symptoms in heterozygous carriers of inherited coagulation disorders in southern Iran

Mahmoodi

Peyvandi

Afrasiabi

et al. 2011

Blood Coagulation & Fibrinolysis

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The objective of the present study was to investigate the prevalence of bleeding symptoms in individuals who are heterozygous for recessively inherited coagulation disorders (RICDs) and to determine the association of these bleeding symptoms with type of RICDs. This was a retrospective cross-sectional study being performed in Shiraz Hemophilia Society (Shiraz, Southern Iran). In this study, bleeding symptoms of the parents (heterozygous) of the patients (homozygous) who were registered and had definite diagnosis as autosomal recessive coagulation disorder were evaluated. These inherited disorders include factor I, V, VII, X, XI, XIII deficiency, combined factor VII and X deficiency, combined factor V and VIII deficiency, all platelet disorders and von Willebrand disease (VWD) type III. 50.3% individuals underwent genotype and mutation study to confirm their heterozygosity. We included 350 heterozygote individuals for inherited coagulation disorders among whom there were 175 (50%) men and 175 (50%) women. Those who were heterozygous for factor VII deficiency had significantly higher prevalence of subcutaneous hematoma (P=0.011). In the same way heterozygous patients for Bernard-Soulier syndrome had higher prevalence of hypermenorrhea (P=0.012) and obstetric (normal vaginal delivery or cesarean delivery) bleeding (P=0.012). Heterozygosity for factor X and XIII deficiency was associated with prolonged or massive bleeding during operations (P=0.001) and after minor traumas (P=0.019), respectively. Heterozygosity for RICDs is associated with some bleeding symptoms. Thus bleeding tendency and homeostasis disturbance should be kept in mind in those who are heterozygous for RICDs and more preoperative care and correction of coagulation indices is highly recommended.

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Section: Discussioncontrasting

confidence: 85%

Bleeding symptoms in heterozygous carriers of inherited coagulation disorders in southern Iran

Mahmoodi

Peyvandi

Afrasiabi

et al. 2011

Blood Coagulation & Fibrinolysis

View full text Add to dashboard Cite

show abstract

“…However, in developing countries, because of the lack of safe blood products, treatment strategies, and registration centers for arranging necessary equipment, and possibly because of costrelated problems, patients are at the risk of acquiring a disability. In addition, according to an estimation in a study, only 25% of all hemophilic patients receive adequate treatment, and unfortunately most of them die before they reach the age of 20 years (42). Nevertheless, some developing countries in EMRO, like Iran, are making efforts to ensure better care for patients (63).…”

Section: Discussionmentioning

confidence: 99%

Lack of Knowledge About Hepatitis C Infection Rates Among Patients With Inherited Coagulation Disorders in Countries Under the Eastern Mediterranean Region Office of WHO (EMRO): A Meta-Analysis

Alavian

Aalaei‐Andabili²

2012

Hepat Mon

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Context: Hepatitis C virus (HCV) infection is a public health problem. HCV alone is responsible for 90% cases of acute hepatitis among multiply transfused patients who are at risk of cirrhosis and hepatocellular carcinoma (HCC). Objectives: To provide a clear picture of available data, we performed a systematic review of the epidemiological characteristics of HCV infection among patients with inherited coagulation disorders in the countries under the Eastern Mediterranean Region Office (EMRO). Evidence Acquisition: Meta-analysis was carried out on the basis of results of electronic and manual search. This analysis included studies in English, French, and Persian that met with the following criteria: (1) appropriate study design: cross-sectional, case-control, and cohort; (2) studies reporting HCV prevalence according to enzyme immune assay; (3) studies in which the sample population was enrolled from EMRO countries. According to the results of the heterogeneity tests, we used fixed-effect/random-effect model for our meta-analysis, with the "Metan" command. Results:We included 30 studies, most of which were from Iran. The mean age of the subjects ranged from 13 to 27.1 years. The majority of the patients were male (range, 81% to 100%). The pooled estimate of HCV infection among patients with inherited coagulation disorders was 48.07% (95% confidence interval [CI], range: 27.39-55.68) in Iran, 36.03% (95% CI, range: 4.466-67.598) in Pakistan, and 48.27% (95% CI,) in all the EMRO countries taken together. Conclusions: In Iran and other EMRO countries, the HCV infection rate among patients with inherited coagulation disorders is high. Our study shows that there is a lack of knowledge about infections in such patients in most of the EMRO countries. It is the responsibility of health-policy makers to address this knowledge gap and provide safe and adequate treatment for patients in high-risk groups.

show abstract

“…However, in developing countries, because of the lack of safe blood products, treatment strategies, and registration centers for arranging necessary equipment, and possibly because of cost-related problems, patients are at the risk of acquiring a disability. In addition, according to an estimation in a study, only 25% of all hemophilic patients receive adequate treatment, and unfortunately most of them die before they reach the age of 20 years [42]. Nevertheless, some developing countries in EMRO, like Iran, are making efforts to ensure better care for patients [63].…”

Section: Discussionmentioning

confidence: 99%

Lack of Knowledge About Hepatitis C Infection Rates Among Patients With Inherited Coagulation Disorders in Countries Under the Eastern Mediterranean Region Office of WHO (EMRO): A Meta-Analysis

Alavian

Aalaei‐Andabili²

2012

Hepat Mon

View full text Add to dashboard Cite

ContextHepatitis C virus (HCV) infection is a public health problem. HCV alone is responsible for 90% cases of acute hepatitis among multiply transfused patients who are at risk of cirrhosis and hepatocellular carcinoma (HCC).ObjectivesTo provide a clear picture of available data, we performed a systematic review of the epidemiological characteristics of HCV infection among patients with inherited coagulation disorders in the countries under the Eastern Mediterranean Region Office (EMRO).Evidence AcquisitionMeta-analysis was carried out on the basis of results of electronic and manual search. This analysis included studies in English, French, and Persian that met with the following criteria: (1) appropriate study design: cross-sectional, case-control, and cohort; (2) studies reporting HCV prevalence according to enzyme immune assay; (3) studies in which the sample population was enrolled from EMRO countries. According to the results of the heterogeneity tests, we used fixed-effect/random-effect model for our meta-analysis, with the “Metan” command.ResultsWe included 30 studies, most of which were from Iran. The mean age of the subjects ranged from 13 to 27.1 years. The majority of the patients were male (range, 81% to 100%). The pooled estimate of HCV infection among patients with inherited coagulation disorders was 48.07% (95% confidence interval [CI], range: 27.39–55.68) in Iran, 36.03% (95% CI, range: 4.466–67.598) in Pakistan, and 48.27% (95% CI, range: 36.12–60.43) in all the EMRO countries taken together.ConclusionsIn Iran and other EMRO countries, the HCV infection rate among patients with inherited coagulation disorders is high. Our study shows that there is a lack of knowledge about infections in such patients in most of the EMRO countries. It is the responsibility of health-policy makers to address this knowledge gap and provide safe and adequate treatment for patients in high-risk groups.

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Clinical audit of inherited bleeding disorders in a developing country

Cited by 20 publications

References 0 publications

Bleeding symptoms in heterozygous carriers of inherited coagulation disorders in southern Iran

Bleeding symptoms in heterozygous carriers of inherited coagulation disorders in southern Iran

Lack of Knowledge About Hepatitis C Infection Rates Among Patients With Inherited Coagulation Disorders in Countries Under the Eastern Mediterranean Region Office of WHO (EMRO): A Meta-Analysis

Lack of Knowledge About Hepatitis C Infection Rates Among Patients With Inherited Coagulation Disorders in Countries Under the Eastern Mediterranean Region Office of WHO (EMRO): A Meta-Analysis

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