Mean pulse pressure distribution by gender and age, adjusted for hemoglobin. The introduction of treatments that induce protective fetal hemoglobin and reduce infectious complications has greatly prolonged survival. Common systemic manifestations: episodes of arm, leg, back, and stomach pain Common ophthalmic manifestations: hemorrhagic arteriolar occlusions in peripheral retina Ophthalmic manifestations common in Sickle-Hemoglobin C Disease and Sickle-Thalassemia Disease, less common in Sickle Cell Disease, and rare in Sickle Cell Trait
doi: 10.1016/S0140-6736(16)00647-4. Public Library of Science Treat Respir Med. Pegelow CH(1), Colangelo L, Steinberg M, Wright EC, Smith J, Phillips G, Vichinsky E. Author information: (1)Department of Pediatrics, University of Miami School of Medicine, Florida 33101, USA.

These processes culminate in the development of pulmonary hypertension, left ventricular diastolic heart disease, dysrhythmia, and sudden death. Improvements in health infrastructure, preventive care, and clinical treatments have reduced the morbidity and mortality of sickle-cell disease in developed countries. Public Library of Science Cardiopulmonary organ dysfunction and chronic kidney injury have a large effect on morbidity and premature mortality, and typically accelerate in the second decade of life.

Sickle cell disease is characterized by recurrent episodes of ischemia-reperfusion injury to multiple vital organ systems and a chronic hemolytic anemia, both contributing to progressive organ dysfunction.


2020 May-Jun;72(3):205-208. doi: 10.1016/j.ihj.2020.05.007. 2020 Feb 20;8:38. doi: 10.3389/fped.2020.00038. doi: 10.7759/cureus.7105.Fiocchi J, Urits I, Orhurhu V, Orhurhu MS, Giacomazzi S, Hoyt B, Kaye AD, Kaye RJ, Viswanath O.Curr Pain Headache Rep. 2020 Mar 21;24(5):17. doi: 10.1007/s11916-020-00854-y.Ochocinski D, Dalal M, Black LV, Carr S, Lew J, Sullivan K, Kissoon N.Front Pediatr. Gordeuk VR, Sachdev V, Taylor JG, Gladwin MT, Kato G, Castro OL.Am J Hematol. Epub 2020 May 28.Blood. 2006; 5:31–45.

Elsevier Science Pulse pressure, a predictor of mortality, was higher in males of all ages. Natural history of blood pressure in sickle cell disease: risks for stroke and death associated with relative hypertension in sickle cell anemia. 2013 Oct-Dec;83(4):289-94. doi: 10.1016/j.acmx.2013.05.003. 2020 Jun 12;115(4):43. doi: 10.1007/s00395-020-0799-x.Cureus. 2013 Oct 30;14:237. doi: 10.1186/1471-2369-14-237.Makubi A, Mmbando BP, Novelli EM, Lwakatare J, Soka D, Marik H, Tibarazwa K, Ngaeje M, Newton CR, Gladwin MT, Makani J.Br J Haematol. The median (IQR) for pulse pressure was 45 mmHg (39–55) and in linear regression analysis pulse pressure was associated with total hemoglobin (beta = -0.4 per mg/dl, P = 0.031), age (beta = 0.3 per year, P < 0.001) and male gender (beta = 4.4, P < 0.001) (Fig 2).

Unable to load your collection due to an error This site needs JavaScript to work properly. These associated risk factors for pulmonary hypertension suggest that pulmonary hypertension represents one element of the systemic vasculopathy seen in some patients with sickle cell disease (systemic hypertension, renal failure and priapism) and is mechanistically linked to hemolytic rate, iron overload and cholestatic hepatic dysfunction. doi: 10.1016/S0140-6736(16)00647-4. Please enable it to take advantage of the complete set of features! In sickle-cell disease, a point mutation in the β-globin chain causes haemoglobin to polymerise within erythrocytes during deoxygenation, altering red blood cell rheology and causing haemolysis. In this Series paper, we review the mechanisms, clinical features, and epidemiology of major cardiovascular complications in patients with sickle-cell disease and discuss how screening and intervention could reduce their incidence.

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