Study Results
The study team has not published outcome measurements, participant flow, or safety data for this trial yet. Check back later for updates.
Basic Information
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UNKNOWN
201 participants
OBSERVATIONAL
2020-08-01
2020-12-01
Brief Summary
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Beta- thalassemia is a group of recessively inherited disorders of hemoglobin synthesis characterized by reduced synthesis of the ß-globin chain caused by a mutation. The homozygous state results in severe anemia which needs regular blood transfusion.
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Detailed Description
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The combination of transfusion and chelation therapy has dramatically extended the life expectancy of these patients, thus transforming thalassemia from a rapidly fatal disease of childhood to a chronic illness compatible with a prolonged life. On the other hand, frequent blood transfusions leading to iron overload and the chronic nature of the disease have contributed to a whole new spectrum of complications in adolescents and young adults suffering from thalassemia major.
Complications associated with beta thalassemia, aside from the aforementioned anemia, are as follows
* Extramedullary hematopoiesis
* Asplenia secondary to splenectomy
* Medical complications from long-term transfusional therapy - Iron overload and transfusion-associated infections (eg, hepatitis) Increased risk for infections resulting from asplenia (eg, encapsulated organisms such as pneumococcus) or from iron overload (eg, Yersinia species))
* Cholelithiasis (eg, bilirubin stones) Study of the prevelance and risk fators responsible for thalassemia complication can help in adopting appropriating strategies for management of these complication.
Conditions
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Study Design
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OTHER
CROSS_SECTIONAL
Study Groups
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Cases
Demographic, physical examination, data will becollected .
Demographic data collected includ clinical diagnosis, sex, race, genotype, and phenotype.
Historical data included information regarding immunizations, infection exposures, surgical procedures, organ dysfunction(Organ dysfunction was defined as a history of requiring medical treatment for heart disease, diabetes (Type 1 or 2), hypoparathyroidism, hypothyroidism, growth hormone deficiency, or gonadal failure) age at transfusion initiation, chelation therapy, type of RBC product, transfusion reactions, and antigen matching. Data will be collected from chart review, patient interviews, blood banking records, and laboratory
Interventions
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Demographic, physical examination, data will becollected .
Demographic data collected includ clinical diagnosis, sex, race, genotype, and phenotype.
Historical data included information regarding immunizations, infection exposures, surgical procedures, organ dysfunction(Organ dysfunction was defined as a history of requiring medical treatment for heart disease, diabetes (Type 1 or 2), hypoparathyroidism, hypothyroidism, growth hormone deficiency, or gonadal failure) age at transfusion initiation, chelation therapy, type of RBC product, transfusion reactions, and antigen matching. Data will be collected from chart review, patient interviews, blood banking records, and laboratory
Eligibility Criteria
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Inclusion Criteria
Exclusion Criteria
* Children who diagnosed as other types of thalassemia except Beta thalassemia major
1 Year
18 Years
ALL
No
Sponsors
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Assiut University
OTHER
Responsible Party
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Tahany Naeem Saied Matta
Principal Investigator
Central Contacts
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References
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Modell B, Darlison M. Global epidemiology of haemoglobin disorders and derived service indicators. Bull World Health Organ. 2008 Jun;86(6):480-7. doi: 10.2471/blt.06.036673.
Weatherall DJ. The inherited diseases of hemoglobin are an emerging global health burden. Blood. 2010 Jun 3;115(22):4331-6. doi: 10.1182/blood-2010-01-251348. Epub 2010 Mar 16.
Other Identifiers
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Prev TM
Identifier Type: -
Identifier Source: org_study_id
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