الفهرس 
THALASSEMIA
Airway hyperresponsivenessOnly 14 pages are availabe for public view
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Abstract
P
ulmonary dysfunction is one of the least understood complications in patients with chronic hemolytic anemias, although it is not uncommon, having been reported in up to 80% of patients with the disease ( Eidani et al.,2009).
Atopic manifestations are also reported in patients with chronic hemolytic anemias, affecting their clinical conditions and share in pulmonary dysfunction as regard respiratory manifestations and abnormal pulmonary function tests.
The aim of this study was to assess the pulmonary functions and atopic manifestations in multi-transfused chronic hemolytic anemia patients.
We also aimed at finding out the relation between serum IgE level and pulmonary function tests in chronic hemolytic anemia patients.
This study included sixty patients with transfusion dependent chronic hemolytic anemia, 40 patients with thalassemia, 20 patients with sickle cell disease (require frequent transfusion at least once per month), aged from 7 to 12 years from both sexes and 60 apparently healthy children age and sex matched was included as a control group.
All patients were subjected to the following:
Full history taking including: age, sex, duration of disease, frequency of blood transfusion, chelation therapy, chronic hemolytic anemia related complications especially liver complications, history of splenectomy, time elapsed since splenectomy and symptoms suggestive of atopy (allergic rhinitis, atopic dermatitis and asthma).
- Clinical chest examination.
- Laboratory investigations included:
- Mean serum hemoglobin in the last year.
- Mean serum ferritin in the last year.
- Hepatitis markers.
For both chronic hemolytic anemia patients and control group.
- Pulmonary function test for assessment of spirometric values (FEV1, FVC, PEF&MMEF), using VIASYS Healthcare Gmbh, libniz strasse.
- Measuring the level on IgE in peripheral blood by ELISA using TECAN® instrument.
This study revealed the following results:
- The results of Pulmonary function tests revealed that 28 out of 60 patients had abnormal pulmonary function tests representing 46.67%.
- Restrictive lung disease represent 30%, obstructive lung disease 10% and mixed lung disease 6.67%.
- Thalassemia Patients with abnormal pulmonary function tests represent 42.50% of the thalassemic patients. Restrictive lung disease represents 30.00%, obstructive lung disease 7.50% and mixed lung disease 5.00%.
- Sickle cell disease Patients (SCD) with abnormal pulmonary function tests represent 55% of the SCD patients. Restrictive lung disease represents 30.00%, obstructive lung disease 15% and mixed lung disease 10%.
- FEV1, FVC, PEF and MMEF were inversely related to the age and duration of illness of the patients especially in sickle cell disease patients.
- Pulmonary function tests were negatively correlated with time elapsed since splenectomy especially in sickle cell disease patients.
- Pulmonary function tests in thalassemia and sickle cell disease patients with respiratory manifestations and/or atopic manifestations were significantly reduced.
- S.IgE level was higher in chronic hemolytic anemia patients with respiratory and/ or atopic manifestations, especially in splenectomized sickle cell disease patients.
- There was no correlation between frequency of blood transfusion, serum ferritin level and all parameters of pulmonary function tests in both groups of chronic hemolytic anemia.