Pulmonary hypertension in sickle cell hemoglobinopathy: A clinicopathologic study of 20 cases

Haque, Abida K.; Gokhale, Sumita; Rampy, Bill A.; Adegboyega, Patrick A.; Duarte, Alexander G.; Saldana, Mario J.

doi:10.1053/hupa.2002.128059

Cited by 190 publications

(144 citation statements)

References 25 publications

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“…One retrospective study reported a median survival of 25.6 months for SCD patients with PAH confirmed by right heart catheterization [5]. Autopsy studies revealed that up to 75% of SCD patients have histological evidence of PAH [6]. In recent years, it has become of growing importance to have precise early and convenient method to diagnose PAH in SCD [7].…”

Section: Introductionmentioning

confidence: 99%

Uric Acid as a Potential Biomarker of Pulmonary Arterial Hypertension in Patients with Sickle Cell Disease

Joshi

Anjum

Gowda

et al. 2011

Indian J Hematol Blood Transfus

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Serum uric acid (UA) is emerging as a strong and independent marker for pulmonary arterial hypertension (PAH). PAH is well recognized as a life threatening complication of sickle cell disease (SCD). However, the association between UA and PAH in SCD is unknown. We reviewed electronic medical records (EMR) of 559 consecutive adult SCD patients from Kings County Hospital Center (KCHC) between January 2005 and February 2010. Patients (n = 96) with measurement of UA in close temporal proximity to the transthoracic echocardiography (TTE) were identified. PAH was defined as pulmonary artery systolic pressure (PASP) C30 mm Hg. Patients (n = 16) with other risk factors which may cause PAH and chronic renal insufficiency were excluded. In 18 patients, TTE could not measure PASP. Finally, 62 patients were selected. Statistical analysis was performed using Student t tests, Pearson correlation coefficient and multivariate regression analysis. Out of 62 patients, 30 had PAH. Patients with PAH had a higher UA level (8.67 ± 4.8 vs. 5.35 ± 2.1, P = 0.001). We found strong positive correlation between the UA level and PASP (r = 0.71; P \ 0.0001). This correlation was independent of diuretic use. UA could be a potential marker for PAH in SCD. However, its' prognostic and pathophysiologic role in SCD patients with PAH needs to be further investigated.

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Section: Introductionmentioning

confidence: 99%

Uric Acid as a Potential Biomarker of Pulmonary Arterial Hypertension in Patients with Sickle Cell Disease

Joshi

Anjum

Gowda

et al. 2011

Indian J Hematol Blood Transfus

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“…33,41,44,45 Recent autopsy studies suggest that up to 75% of sickle cell patients have histological evidence of pulmonary arterial hypertension at the time of death. 46 Similarly, retrospective studies have demonstrated that 40%-50% of patients with thalassemia intermedia, 43 and 10%-75% of patients with thalassemia major, have echocardiographic evidence of pulmonary hypertension. 47,48…”

Section: Pulmonary Arterial Hypertension In Sickle Cell Disease Prevamentioning

confidence: 99%

Cardiopulmonary Complications of Sickle Cell Disease: Role of Nitric Oxide and Hemolytic Anemia

Gladwin

Kato²

2005

Hematology

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Medical advances in the management of patients with sickle cell disease, thalassemia, and other hemolytic anemias have led to significant increases in life expectancy. Improved public health, neonatal screening, parental and patient education, advances in red cell transfusion medicine, iron chelation therapy, penicillin prophylaxis for children, pneumococcal immunization, and hydroxyurea therapy have all likely contributed to this effect on longevity. 1,2 Importantly, as a generation of patients with sickle cell disease and thalassemia ages, new chronic complications of these hemoglobinopathies develop. In this context, pulmonary hypertension is emerging as one of the leading causes of morbidity and mortality in adult sickle cell and thalassemia patients, and likely in patients with other hemolytic anemias. A common feature of both sickle cell disease and thalassemia is intravascular hemolysis and chronic anemia. Recent data suggest that chronic intravascular hemolysis is associated with a state of endothelial dysfunction characterized by reduced nitric oxide (NO) bioavailability, pro-oxidant and pro-inflammatory stress and coagulopathy, leading to vasomotor instability and ultimately producing a proliferative vasculopathy, a hallmark of which is the development of pulmonary hypertension in adulthood. 3-5 In conclusion, pulmonary hypertension is common in patients with hereditary hemolytic anemias and is associated with a high risk of death in patients with sickle cell disease. New therapies targeting this vasculopathy and aimed at normalizing the vasodilator:vasoconstrictor balance are discussed.

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“…In fact, degenerative elastic tissue lesions have been encountered in pulmonary autopsies in patients with hemoglobinopathies such as sickle cell disease. 46 Furthermore, endothelial dysfunction promotes hypercoagulability and in situ thrombus formation within the pulmonary vascular bed. In β-thalassemia in particular, the oxidative stress resulting from chronic hemolysis is enhanced by the presence of iron overload and free-radical formation and the expected effect seems to be more pronounced.…”

Section: 5mentioning

confidence: 99%

Heart disease in thalassemia intermedia: a review of the underlying pathophysiology

Aessopos

Kati²,

Farmakis³

2007

Haematologica

110

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Pulmonary hypertension in sickle cell hemoglobinopathy: A clinicopathologic study of 20 cases

Cited by 190 publications

References 25 publications

Uric Acid as a Potential Biomarker of Pulmonary Arterial Hypertension in Patients with Sickle Cell Disease

Uric Acid as a Potential Biomarker of Pulmonary Arterial Hypertension in Patients with Sickle Cell Disease

Cardiopulmonary Complications of Sickle Cell Disease: Role of Nitric Oxide and Hemolytic Anemia

Heart disease in thalassemia intermedia: a review of the underlying pathophysiology

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