الفهرس 
Hemolytic AnemiaOnly 14 pages are availabe for public view
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Abstract
Pulmonary hypertension (PHT) is a common complication of hereditary hemolytic anemias including sickle cell disease, thalassemia, hereditary spherocytosis, and paroxysmal nocturnal hemoglobinuria.
The pathogenesis of PHT complicating hemolytic anemias is probably heterogeneous, including hemolysis and its effect on nitric oxide bioavailability, asplenia, thrombosis, chronic lung diseases, and iron overload.
The aim of this study was to detect prospectively elevated pulmonary artery pressure and risk factors among patients with chronic hemolytic anemias (β thalassemia and SCD) with stress on correlation between PHT,D-Dimer test and ABG analysis.
This study included twenty patients previously diagnosed as β thalassemia and SCD regularly attending for follow up in the Hematology Outpatient Clinic in Elmaady Military Hospital; they had neither symptoms nor signs suggesting pulmonary and cardiac abnormalities.All patients were in a steady state and were subjected to full history taking, clinical and laboratory examination in addition to Imaging that included chest X-ray and doppler echcocardiography.
The mean age of the patients was 20.5±6.5 years ranging from 13 to 31ys, 55% of the patients were males and 45% were females. 50% of the patients had beta thalathemia and 50% had sickle cell anemia. 100% of the patients were on transfusion therapy, age of onset of blood transfusion in the patients ranged from 1 to 5 yrs.
The prevalence of pulmonary hypertension (PASP≥30 mmHg) was 30% and they were of mild severity (<40mmHg), the mean pulmonary artery systolic pressure was 27±7.49 mmHg ranging from 17-40 mmHg.
Regarding the clinical and laboratory risk factors associated with pulmonary hypertension; patients who develop PHT are characterized by history of pallor, increasing hemolytic markers as high reticulocytic count, total bilirubin, AST and low hemoglobin level, also higher creatinine level was associated with PHT patients.
No significant differences as regards age, gender, genotype, family history, ALT, direct bilirubin, LDH, platelet count, WBC and history of splenectomy between PHT +ve and PHT -ve patients.
There was significant positive correlation between systolic blood pressure and PHT being higher among patients with PHT.
D-Dimer level was found to correlate proportionally with estimated systolic pulmonary artery pressure (ESPAP) measured.
Lower oxygen saturation was common in patients with PHT.
Therefore, in the light of this study, results and discussions, it was concluded that high pulmonary artery pressure may occur in adults suffering from hemolytic anemias. Screening by echocardiography, D-Dimer test and ABG analysis may contribute to early detection and therapeutic treatment that may potentially reverse this disease process.