Abstract

Acute chest syndrome (ACS) is a leading cause of death from sickle cell disease worldwide accounting for about 25% of all deaths. The aim of this study was to determine the prevalence, clinical features and outcome in Port Harcourt, Nigeria. Materials and Methods: A retrospective cohort study during a five year period. Records of all patients with sickle cell anaemia (SCA) admitted into the Wards were examined. Those enrolled for the study satisfied two criteria: 1) lower respiratory tract symptoms and 2) new pulmonary infiltrates on the chest radiograph. Sociodemographics, genotype, clinical and laboratory features, treatment given and outcome were obtained. Data were analysed by descriptive statistics. Variables were compared by students’ t-test. P value ≤ 0.05 was regarded as significant. Results: A total of 345 children with sickle cell anaemia were admitted during the 5 year period. Twelve of them had acute chest syndrome (3.5%). Majority 7 (58.3%) of them were under 5 years. There were more males 8 (66.7%) than female 4 (33.3%). The most common clinical features were fever 12 (100%), cough 10 (83.3%), chest pain 5 (41.7%), pulmonary consolidation 12 (100%), and respiratory distress 12 (100%). The admitting diagnosis were bronchopneumonia 6 (50%), severe malaria 3 (25%) and vaso-occlusive crises 3 (25%). There were very high levels of leukocyte. Received ceftriaxone or ampicillin + gentamicin ± oral erythromycin), paracetamol 12 (100%), ibuprofen 8 (66.7%), tramadol 3 (25.0%), pentazocine 8 (66.7%) and blood transfusion 9 (75%). The average length of stay was 7 days (range 4 - 14 days). One patient died (8.3%). Conclusion: ACS is not uncommon in children with SCA in Port Harcourt. Education of parents on the need to recognize early symptoms of the disease is essential. Clinicians must be trained to correctly diagnose and manage it promptly and efficiently to avoid its related disastrous consequences.

Highlights

  • Sickle cell anemia is one of the most prevalent genetic diseases worldwide [1] [2]

  • The records of all patients with sickle cell anaemia who were admitted to the Children Emergency and Paediatric Wards of the University of Port Harcourt Teaching Hospital (UPTH), Port Harcourt, Nigeria, during the period stated by the study were carefully examined

  • Those admitted to the study satisfied two criteria: 1) lower respiratory tract symptoms and 2) new pulmonary infiltrates on the chest radiograph

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Summary

Introduction

Sickle cell anemia is one of the most prevalent genetic diseases worldwide [1] [2]. It frequently poses a task to health care providers for whom the disease can be considered one of the most significant haemoglobinopathies. Amid a diverse range of complications of varying complexity, none can become as rapidly disastrous as acute chest syndrome (ACS) [2]. It is the leading cause of death from sickle cell disease (SCD) worldwide accounting for about 25% of all deaths. Acute chest syndrome (ACS) is a leading cause of death from sickle cell disease worldwide accounting for about 25% of all deaths. Records of all patients with sickle cell anaemia (SCA) admitted into the Wards were examined Those enrolled for the study satisfied two criteria: 1) lower respiratory tract symptoms and 2) new pulmonary infiltrates on the chest radiograph. Clinicians must be trained to correctly diagnose and manage it promptly and efficiently to avoid its related disastrous consequences

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