الفهرس 
ThalassemiaOnly 14 pages are availabe for public view
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Abstract
Pulmonary hypertension is a serious disease with various complications. It is underestimated by clinicians in their routine examination. Patients with either thalassemia or sickle cell anemia are at high risk for pulmonary hypertension because of the nature of their disease, complication of the treatment and compliance of the patients.
The aim of this study is to compare between β-thalassemia (major and intermedia) and sickle cell anemia as regard chelation-transfusion compliance and its effect upon cardiopulmonary function with special emphasis on left ventricular function, pulmonary artery pressure and ventilation perfusion scan of the lung.
The present cross sectional study was carried out on twenty two patients aged 10 years and older (range of age = 11-27) with either beta thalassemia (major or intermedia) or sickle cell anemia, following up in the Pediatric Hematology Clinic, Children Hospital, Ain Shams University.
All patients were subjected to detailed history taking, full clinical examination, laboratory investigation (Hb level, Hb level per year, Serum ferritin level and lactate dehydrogenase (LDH) assessment), electrocardiography, echocardiogragrphy and ventilation perfusion lung scan.
Patients were divided into 3 groups according to the type of the disease: 7 thalassemia major patients, 7 thalassemia intermedia patients and 8 sickle cell anemia patients.
This study revealed the following results:
• All patients with thalassemia intermedia had high serum ferritin level and some of thalassemia major patients had very high serum ferritin level.
• Bad compliance is the attitude of all patients with thalassemia intermedia on desferal therapy causing hemosiderosis and affection of lung perfusion as is evident in our case presentation.
• Quantitative results of the V/Q scan were normal in patient with no hemosiderosis, good chelation transfusion compliance or who didn’t need any chelation transfusion protocol.
• Hemosiderosis, anemia, tachycardia and pulmonary hypertension might affect the lung perfusion.
• Repeated vasoocclusive crisis among sickle cell anemia patients might cause pulmonary hypertension and affection of lung perfusion.
• V/Q scan may give a clue for pulmonary hypertension earlier than echocardiography through the discrepancy of the zonal lung perfusion in association with hemosiderosis.
• Though pulmonary embolism usually presenting by mismatched segmental defects, old emboli may give ‘non specific’ matched defects on the V/Q scan as encountered in one of our cases