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Increased incidence of Glanzmann's thrombasthenia in Jordan as compared with Scandinavia
Scandinavian Journal of Haematology
|March 1, 1983
Insights
Glanzmann's thrombasthenia, a rare inherited bleeding disorder, affects 12 Jordanian children from 9 families, often linked to consanguinous marriages. This study highlights its prevalence and clinical features in Jordan.
Area of Science:
- Hematology
- Genetics
- Pediatrics
Background:
- Glanzmann's thrombasthenia is a rare inherited hemorrhagic disorder.
- Consanguinity is a significant factor in the inheritance of genetic disorders.
Purpose of the Study:
- To describe clinical and laboratory findings of Glanzmann's thrombasthenia in Jordanian patients.
- To emphasize the role of consanguinity in the occurrence of this disorder.
Main Methods:
- Case series description of 12 patients from 9 families.
- Clinical and laboratory data collection.
Main Results:
- All 12 patients were children with varying degrees of mucosal bleeding.
- All patients were offspring of consanguinous marriages.
- Glanzmann's thrombasthenia is the second most common inherited hemorrhagic disorder in Jordan.
Conclusions:
- Consanguinity plays a crucial role in the high prevalence of Glanzmann's thrombasthenia in Jordan.
- Early diagnosis and management are important for affected children.
Abstract:
12 Jordanian patients from 9 families with Glanzmann's thrombasthenia are described. All of them are products of consanguinous marriages. All cases are children with varying severity of mucosal bleeding. The clinical and laboratory findings are described. The importance of consanguinity is discussed and emphasized. Glanzmann's thrombasthenia is the second most common inherited haemorrhagic disorder in Jordan.