Transactions of the Royal Society of Tropical Medicine and Hygiene (2008) 102, 526—531
available at www.sciencedirect.com
journal homepage: www.elsevierhealth.com/journals/trst
REVIEW
Managing acute chest syndrome of sickle
cell disease in an African setting
A.E. Fawibe
*
Department of Internal Medicine, Federal Medical Center, Bida, Niger State, Nigeria
Received 2 December 2007; received in revised form 17 March 2008; accepted 19 March 2008
KEYWORDS
Acute chest
syndrome;
Sickle cell anaemia;
Hydroxyurea;
Analgesics;
Nigeria;
Africa
Summary Despite the fact that acute chest syndrome contributes immensely to morbidity
and mortality in patients with sickle cell anaemia, its exact aetiopathogenesis is very complex
and not yet well understood. Therefore, a high index of suspicion is needed in its diagnosis,
and appropriate treatment should be commenced as soon as possible to prevent lethal com-
plications of this condition, especially in Nigeria where appropriate diagnostic and therapeutic
facilities may not be readily available. This is very important, as it may even develop on hos-
pital admission. There is a need to further investigate preventive measures such as the use
of hydroxyurea and the newly introduced Nicosan, especially in those people with recurrent
disease, in order to reduce both short- and long-term complications of this syndrome among
sickle cell patients in Nigeria.
© 2008 Royal Society of Tropical Medicine and Hygiene. Published by Elsevier Ltd. All rights
reserved.
1. Introduction
Pulmonary complications are major contributors to morbid-
ity, and indeed mortality, in patients with sickle cell anaemia
(SCA), and in the long run lung involvement constitutes a
paramount determinant of survival. Clinical lung involve-
ment in patients with SCA commonly takes two forms: acute
chest syndrome (ACS) and chronic lung disease (Weil et al.,
1993). This article aims at reviewing ACS, with a view to
improving its management in African settings.
Charache et al. (1979) used the term ACS to describe
episodes of fever, chest pain and pulmonary infiltrates in
∗
Present address: P.O. Box 4923, GPO Ilorin, Kwara State, 240001,
Nigeria. Tel.: +234 8033948165, +234 8075269697.
E-mail address: drdemola@yahoo.com
adult sickle cell patients, many of whom seemed to have
pulmonary infarction. Currently, the syndrome is defined
by the appearance of a new pulmonary infiltrate on chest
radiography, fever, cough, chest pain and dyspnoea with
hypoxaemia, and leucocytosis (Vichinsky et al., 1997).
2. Incidence and risk factors
Although anecdotal series on ACS are available, there are
no firm data on its incidence in Nigeria. It was present in
6% of 102 adolescents and adults in a monocentric study
conducted in Lagos, Nigeria (Ibidapo and Akinyanju, 2000).
However, studies done elsewhere have shown that it is a
common complication that occurs in about 50% of patients
with SCA and is recurrent in about 80% of them (Golden et
al., 1998; Leong and Stark, 1998). In half of the patients,
it is preceded by vaso-occlusive crises affecting the long
0035-9203/$ — see front matter © 2008 Royal Society of Tropical Medicine and Hygiene. Published by Elsevier Ltd. All rights reserved.
doi:10.1016/j.trstmh.2008.03.018