Acute chest syndrome in sickle cell disease: clinical presentation and pharmacological management

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AUGUST, 2016
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Abstract
Acute chest syndrome (ACS) is a common cause of hospitalization and mortality in sickle cell patients across the globe. There appears to be paucity of data concerning ACS and its pharmacological management in Ghana and Africa as a whole. This study was aimed at assessing the prevalence and clinical presentation of ACS, and its pharmacotherapy in children with Sickle Cell Disease (SCD) at Komfo Anokye Teaching Hospital (KATH) in Kumasi, Ghana. Method: The designs of the study was retrospective, in which medical records of the children 14 years and below with SCD and at least an episode of ACS were retrieved and assessed at the Sickle Cell Clinic of KATH. One hundred and seven cases among 1336 children with sickle cell disease met the criteria for inclusion in the study. The ages of the patients ranged from 8 months to 14 years with a mean of 5.7 ± 3.5 years. The prevalence of ACS was 8%, with a 2.2 episodes/ month rate of occurrence. Forty percent of the patients were between the ages of 5 to 9 years. Seventy five percent (n=80) presented with fever and 65% (n=70) presented with cough. Other symptoms like rhinorrhoea and irritability were common in younger patients (< 5 years), p = 0.012 and 0.001 respectively. Abdominal pain and chest pain occurred mostly in the older patients (≥ 5 years), p = 0.024 and < 0.001 respectively. Bacteria isolates were found in 12 of 79 cases (15.2%) that had blood culture information. Eighty four patients (80.7%) received cefuroxime and gentamicin dual therapy for empiric treatment of infection in ACS. The dose range of gentamicin used in the hospital was 3 to 9 mg/kg body weight as opposed to the recommended 5 to 7 mg/kg, as a single daily dose. The empiric antibiotic therapy for infections in ACS patients did not cover for atypical bacteria as recommended in standard guidelines. Forty seven patients received paracetamol alone for pain management and 47 received paracetamol and ibuprofen. Morphine was the preferred opioid prescribed, which is in line with recommendation by WHO guidelines for pain management in SCD patients. The prevalence of ACS was 8%. The majority of patients were between the ages 2 to 9 years. Fever and cough were among the common clinical symptoms presented. Other clinical features like rhinorrhoea, irritability, chest pain and abdominal pain varied with age. Treatment regimen for ACS in the hospital included pharmacotherapy for infections, pain management, hydration with IV fluids and blood transfusion in patients with severe anaemia. All of these were in conformity with standard guidelines and literature.
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A thesis submitted in partial fulfillment of the requirements for the award of a master of philosophy degree in clinical pharmacology to the Deparment of Pharmacology Faculty of Pharmarcy and Parmaceutical Sciences, College of Health Sciences,
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