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REVIEW ARTICLE
Year : 2014  |  Volume : 1  |  Issue : 3  |  Page : 111-118

Acute chest syndrome


1 Department of Medicine, Clinical Haemato-Oncology Unit, Ahmadu Bello University Teaching Hospital, Zaria, Nigeria
2 Pulmonology Unit, Ahmadu Bello University Teaching Hospital, Zaria, Nigeria

Correspondence Address:
Bello Jamoh Yusuf
Clinical Haemato-Oncology Unit, Department of Medicine, Ahmadu Bello University Teaching Hospital, Zaria
Nigeria
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DOI: 10.4103/2384-5147.138930

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Sickle cell anaemia - the disease that combines molecular biology, clinical features, biochemistry, pathology, natural selection, population genetics, gene expression and genomics - is the world's most common life threatening monogenic disorder. Acute chest syndrome is a common complication of SCA and it has been identified as the most common cause of mortality in adult patients with SCA. In addition to elaboration of pro-inflammatory cytokines and up-regulation of cellular adhesion molecules, interplay among red cell sequestration, fat embolism and pulmonary infection, which are the pertinent pathophysiological phenomena that operate in a vicious cycle, lead to the clinical features of ACS. Chest infection, usually caused by atypical organisms, is a more common trigger in children, while fat embolism is considered as a more common trigger in adults. More common clinical features are cough, fever and chest pain, although the pattern of these symptoms varies between children and adults cohorts. The operational definition of ACS appears to be a bit loose, making it difficult to categorically distinguish from other differential diagnoses like pneumonia, especially in resource-poor areas. However, when ACS is diagnosed, treatment should be aggressive, addressing analgesia, hydration, the use of broad-spectrum antibiotics, inhaled bronchodilators, anticoagulation and blood transfusion if required. Randomized trials on efficacy of novel agents like statins, glycoprotein IIa/IIIb inhibitors and phospholipase inhibitors are still on-going.


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