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Associated morbidities in children with sickle-cell anaemia presenting with severe anaemia in a malarious area
J P Ambe1, J O Fatunde, O O Sodeinde
1Department of Paediatrics, College of Medical Sciences, University of Maiduguri, Borno State, Nigeria.
Insights
Sickle-cell anaemia (SCA) patients in crisis often face malaria and bacterial infections, increasing risks. Prompt evaluation and treatment of these infections are crucial for better outcomes in SCA patients.
Area of Science:
- Hematology
- Pediatrics
- Infectious Diseases
Background:
- Sickle-cell anaemia (SCA) is a severe genetic blood disorder.
- Patients with SCA frequently experience painful crises, often associated with severe anaemia.
Purpose of the Study:
- To investigate the common complications and outcomes of severe anaemia crises in SCA patients.
- To identify factors associated with increased morbidity and mortality in SCA crises.
Main Methods:
- Prospective study of 104 SCA patients with severe anaemia (PCV ≤ 15%).
- Classification of crises based on physical findings, bilirubin, and reticulocyte counts.
- Investigations included malaria testing, blood cultures, and radiological studies.
Main Results:
- Malaria (66%) and bacterial infections (17.3%) were the most frequent complications.
- Acute chest syndrome was more common in hyperhaemolytic and splenic sequestration crises.
- Conjugated hyperbilirubinaemia was significantly higher in hyperhaemolytic crises (P=0.001).
- Overall case fatality was 86.5/1,000 SCAs; no fatalities in aplastic crisis.
Conclusions:
- Co-existing bacterial infections and conjugated hyperbilirubinaemia increase morbidity and mortality in SCA crises.
- Early evaluation and prompt treatment of infections are vital for SCA patients in crisis.
Abstract:
A prospective study of 104 consecutive cases of patients with sickle-cell anaemia (SCA) presenting with severe anaemia (packed cell volume < or = 15%) was carried out in the Children's Emergency Ward of the University College Hospital, Ibadan, in 1991. The patients were classified according to the type of anaemic crisis, by physical findings, serum bilirubin and reticulocyte counts. Other investigations included a blood film for malaria parasites, blood culture, radiological investigation and lumbar puncture when necessary. The most common problems associated with SCA patients in anaemic crisis were malaria and bacterial infections--68 (66%) and 18 (17.3%) of cases, respectively. Acute chest syndrome was significantly more frequent in patients with hyperhaemolytic and acute splenic sequestration crisis compared with aplastic crisis (P < 0.05). Conjugated hyperbilirubinaemia was also significantly more frequent among patients with hyperhaemolytic crisis compared with all other anaemic crises (chi2 = 13.18, P = 0.001). The overall case fatality was 86.5/1,000 SCAs, with no fatalities in those with aplastic crisis. There were complications in six of the nine mortalities. Co-existing bacterial infections and conjugated hyperbilirubinaemia were associated with increased morbidity and mortality in patients with anaemic crisis. Patients with SCA crisis should have early evaluation and prompt treatment for associated infections.
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