Sickle cell disease acute chest syndrome

Hammerman SI, Kourembanas S, Con-ca TJ, Tucci M, Brauer M, Farber H.W. Endothelin-1 production during the acute chest syndrome in sickle cell disease. Am J Respir Crit Care Med 1997 156 280-285. 

Transplant and allergy also intersect in indirect ways. Acute chest syndrome is a frequent complication in patients with sickle cell disease and has been recently reported to be exacerbated or precipitated by asthma and respiratory allergies. Since SCT can halt this pulmonary destruction process, ongoing investigation at our and other institutions is focused on determining genetic and cytokine pathways and modulators for asthma in sickle cell disease. 

Vichinsky EP, Neumayr LD, Earles AN, et al. Causes and outcomes of the acute chest syndrome in sickle cell disease. National Acute Chest Syndrome Study Group. N Engl J Med 342 1855-1865,2000. 

The cost to society of sickle cell disease is high in terms of human suffering and in the financial burden of providing treatment to alleviate the pain and symptoms of the afflicted individnals and the loss of income to the individnals and their families and communities. Health problems due to sickle cell disease include chronic anemia, vaso-occlnsive crises, splenic sequestration, acute chest syndrome, stroke, splenic and renal dysfunction, and snsceptibility to bacterial infections (2,5,12). In Africa, treatments for sickle cell disease are limited... 

Some of the clinical consequences in SS disease include megaloblastic erythropoiesis, aplastic crisis, stroke, bone pain crisis, proneness to infection particularly by Pneumococcus, Salmonella, and Haemophilus due to hypos-plenism and acute chest syndrome. Prophylactic use of penicillin and antipneumococcal and Haemophilus vaccines has aided in the management of life-threatening infectious complications of SS disease. Neonatal screening has been used in the identification of infants with sickle cell disease so that risk of infection can be modulated by appropriate immunizations and penicillin prophylaxis. The acute chest syndrome characterized by chest pain is due to clogged pulmonary capillaries in a small number of studies, patients have been treated with inhaled nitric oxide, which dilates blood vessels with clinical improvement. 

FIGURE 101-7. Hydroxyurea use in sickle cell disease. ACS, acute chest syndrome ALT, alanine aminotransferase ANC, absolute neutrophil count CBC, complete blood cell count HbF, fetal hemoglobin Hgb, hemoglobin HbSS, homozygous sickle hemoglobin MCV, mean corpuscular volume. (From Dover etaP and Sickle Cell Disease Care Consortiums )... 

Dean D, Neumayr L, Kelly DM, et al. Chlamydia pneumoniae and acute chest syndrome in patients with sickle cell disease. J Pediatr Hematol Oncol 2003 25 46-55. 

Knight-Madden J, Hambleton I. Inhaled bronchodilators for acute chest syndrome in people with sickle cell disease. Cochrane Database Syst Rev 2003 3 CD003733. 

Sullivan KJ, Goodwin SR, Evangelist J, et al. Nitric oxide successfully used to treat acute chest syndrome of sickle cell disease in a young adolescent. Crit Care Med 1999 27 2563-2568. 

HU reduces vaso-occlusive events in patients with sickle cell disease. It does so via several mechanisms that ultimately increase synthesis of HbF, which promotes solubility of hemoglobin within red cells. It also reduces adhesion of red cells to vascular endothelium, and by suppressing the production and adhesiveness of neutrophils, decreases their contribution to vascular occlusion. HU reduces the frequency of painful events, acute chest syndrome, and secondary strokes in sickle cell patients. 

Susceptibility factors Sickle cell disease Of 11 patients with sickle cell disease who were given G-CSF, seven had severe adverse reactions, including vaso-occlusive episodes, acute chest syndrome, multiorgan system failure, and death [8 ]. This suggests that G-CSF should not be used in individuals with sickle cell disease except in the... 

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