Hereditary Bleeding Disorders in Riyadh, Saudi Arabia

Al-Fawaz, I.; Gader, A. M. A.; Bahakim, Hassan M.; Almohareb, Fahad; Al-Momen, Abdulkarim K.; Harakati, Mohamed

doi:10.5144/0256-4947.1996.257

Cited by 26 publications

(27 citation statements)

References 15 publications

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“…This result reflects that VWD with hemophilia is the most frequently encountered IBD and points to increased incidence of platelet dysfunction disorders. Few published studies in Saudi Arabia and Jordan describe the distribution of IBD in the population [15,16]. They obtained the distribution of IBD resembling what has already been established by western countries.…”

Section: Discussionmentioning

confidence: 60%

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The spectrum of inherited bleeding disorders in pediatrics

El-Bostany

Omer²,

Salama³

et al. 2008

Blood Coagulation &Amp; Fibrinolysis

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Inherited bleeding disorders (IBDs) are caused by quantitative and qualitative alterations of either platelets or plasma proteins involved in coagulation and fibrinolysis. Hemophilias are the most frequent IBDs; however, accumulated data from various studies reported that von Willebrand disease (VWD) is the most common cause of IBD, with an increased incidence of platelet function defects, mostly due to the increased rate of consanguinity in some communities. VWD is an inherited disorder of homeostasis due to quantitative or qualitative defect of von Willebrand factor. Data on its epidemiology and impact in developing countries are limited. The objective of this study was to assess the local prevalence of some IBD and establish the clinical and historical variables that are predictive for those bleeding disorders in pediatrics. The study involved 43 children with various bleeding manifestations and 15 age- and sex-matched controls, recruited from the Pediatrics Hematology Clinic at the National Research Centre, Sausan Mubarek children's hospital in Cairo, Egypt and the King Abdulaziz University Hospital, Jeddah, Kingdom of Saudi Arabia. Hematological profile included platelet counts and function, prothrombin time, partial thromboplastin time, factor VIII antigen and its activity, factor IX antigen and its activity, von Willebrand factor and its activity assayed with multimeric analysis. A total of 12 (27.9%) children had VWD, 11 (25.5%) had hemophilia A, three (7%) had hemophilia B, seven (16.3%) had platelet dysfunction and 10 (23.3%) had bleeding with undiagnosed cause. Two of the VWD cases had type I, three had type II, four had type III and one case appeared to have type IIM and another to have IIB VWD. Bruising and epistaxis were the main symptoms in all children with VWD The majority of platelet dysfunction disorders were diagnosed as Glanzmann's thrombasthenia. VWD and Glanzmann's thrombasthenia should be considered not uncommon causes of IBDs in children in Egypt and Kingdom of Saudi Arabia. Routine hematological screening should be mandatory in children with positive family history of bruising and bleeding as a predictor for IBD.

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Section: Discussionmentioning

confidence: 60%

“…Glanzmann's thrombasthenia is a rare disorder caused by a defect in the glycoprotein IIb/IIIa complex receptors. Its incidence is increased in geographic regions where consanguinity is common [15,20,21]. Recurrent epistaxis was the main symptom in children (71.4%) with platelet dysfunction disorders.…”

Section: Discussionmentioning

confidence: 98%

The spectrum of inherited bleeding disorders in pediatrics

El-Bostany

Omer²,

Salama³

et al. 2008

Blood Coagulation &Amp; Fibrinolysis

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“…Few published studies in Saudi Arabia, Jordan and Egypt describe the distribution of IBD in the population 6,7,8. They obtained the distribution of IBD resembling what has already been established by western countries.…”

Section: Discussionmentioning

confidence: 94%

Descriptive Epidemiology of Hemophilia and Other Coagulation Disorders in Mansoura , Egypt

Tonbary

El-Ashry

Zaki

2010

Mediterr J Hematol Infect Dis

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Hemophilia represent the most severe inherited bleeding disorder (INB), it’s thought to affect inviduals from all geographical areas in equal frequency. In Egypt which has a population of approximately (80million) consanguineous marriage are frequent, therefore autosomal recessive coagulation disorders reach a higher prevalence than in many other countries.The primary aim of this study was to describe the epidemiological situation of hemophilia in Mansoura, Egypt, as based on retrospective analysis of clinical records Mansoura University Children Hospital between years 2000 and 2008. The second aim was to assess the orthopedic complications and occurrence of hepatitis C in those patients and relate this status to the type of replacement therapy received prior to the study.The study included 72 children with hematological disorders registered from 2000 to 2008 in MUCH. The hemophilic patient was defined as a person with physician-diagnosed hemophilia A or B and a measured factor VIII or IX activity level of 30% or less. Persons with acquired inhibitors of FVIII or FIX excluded. Severity level was categorized as mild if the factor activity level was 6–30%, moderate if 1–5% and severe if <1% of normal.The severe presentation represents the majority in 76.7% followed by moderate severity in 17.2%.The commonest IBDs was hemophilia A affecting 44 patients, followed by Hemophilia B affecting 15 patients. The rare types were Factor XI deficiency, Factor V deficiency, Factor VII deficiency and combined FVIII, FIX and FX deficiency. The commonest orthopedic manifestation needing therapy was found among hemophilia A representing 8.3%. Hepatitis C viremia detected by PCR was found in 11.1% of patients. The bleeding complications as hematoma or hemarthrosis were the common complications. Nevertheless, 44.4% of patients had no complications, From this study we can conclude that the most common IBDs in our locality is hemophilia A followed by hemophilia B. The common presenting symptom was bleeding following male circumcision. Hepatitis C infection and arthropathy represented the main complications. The discovery of IBDs in young age children with proper supportive therapy could prevent arthropathy. Proper screening of blood and blood products reduce the risk of viral hepatitis and HIV acquisition.

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“…There was no review of vWD in the developing world. A few articles described regional epidemiology, [13][14][15][23][24][25] some referred to the existence of patients with vWD among those evaluated for bleeding disorders [26][27][28][29][30] or described the profile of small numbers of these patients, 31,32 and others mentioned the response to therapy with DDAVP. 33,34 There were few case reports.…”

Section: Vwd In Developing Countriesmentioning

confidence: 99%

“…33,34 There were few case reports. [26][27][28][29][30] In a review of the epidemiology of coagulation [35][36][37][38][39] disorders in the world, 40 most of the data from developing countries were from a publication of the World Federation of Haemophilia (WFH), Status and Atlas of Hemophilia Worldwide, 1984. 41 Therefore, an international questionnaire survey was conducted in 1998 to collect more recent data regarding vWD from as many developing countries as possible.…”

Section: Vwd In Developing Countriesmentioning

confidence: 99%

Epidemiology of von Willebrand Disease in Developing Countries

Srivastava

Rodeghiero²

2005

Semin Thromb Hemost

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There is limited information on von Willebrand disease (vWD) in developing countries. A questionnaire survey in 1998 showed that overall, less than one third of the expected number of patients with vWD in the population had been detected. Among those registered, the proportion with the severe form of the disease was relatively higher, forming up to 50% of the total, particularly in those countries with high prevalence of consanguineous marriages. Diagnosis was based on von Willebrand factor antigen (vWF:Ag) assays, vWF ristocetin cofactor (vWF:Rco) activity, and ristocetin-induced platelet aggregation (RIPA) at the reporting centers, but vWF multimer analysis was not widely available. Responsiveness to desmopressin acetate (DDAVP), which could be very significant clinically, was not often assessed. An attempt was made to assess accuracy of reporting of the prevalence of vWD by conducting a second survey aimed at normalizing the reported numbers with that of severe hemophilia A in a defined population on the presumption that the latter is less likely to be missed. These data showed that in most countries, the ratio of patients with vWD to severe hemophilia varied between 0.1 to 0.6 with a mean of approximately 0.4, as opposed to an expected ratio of approximately 1 (based on population-based data from Italy), confirming the underdiagnosis of vWD even within the same health-care system. A system for collecting more extensive and detailed data on vWD from developing countries (where 80% of the world's population lives) is required. Efforts are needed to develop national registries and make at least basic services for diagnosis and treatment widely available.

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Hereditary Bleeding Disorders in Riyadh, Saudi Arabia

Cited by 26 publications

References 15 publications

The spectrum of inherited bleeding disorders in pediatrics

The spectrum of inherited bleeding disorders in pediatrics

Descriptive Epidemiology of Hemophilia and Other Coagulation Disorders in Mansoura , Egypt

Epidemiology of von Willebrand Disease in Developing Countries

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