BOF: General (Haemoglobinopathy)

Which ONE of the following organisms is the COMMONEST cause of bacterial septic death in infants with sickle cell disease?

a) Escherichia coli

b) Salmonella sp.

c) Staphylococcus aureus

d) Streptococcus pneumonia

e) Yersinia enterocolitica

Answer:

d) Streptococcus pneumonia

Explanation:

Sepsis due to functional hyposplenism makes death from encapsulated organisms such as streptococci or haemophilus a significant risk particularly in infants with sickle cell disease. Osteomyelitis is most commonly caused by salmonella species (Staphylococcus in non-sickle patients). Streptococcus pneumonia and other gram negative rods are also commonly found. The importance of a pneumococcal vaccination regime and prophylactic antibiotics particularly in children cannot be overemphasied and should form part of the comprehensive management and patient and parent empowerment and education strategy of any centre encountering this disease.

References:

Cooperative Study of Sickle Cell Disease. AU Gill FM; Sleeper LA; Weiner SJ; Brown AK; Bellevue R; Grover R; Pegelow CH; Vichinsky E SO. Clinical events in the first decade in a cohort of infants with sickle cell disease. Blood 1995 Jul 15;86(2):776-83.

Chamber JB, Forsythe DA, Bertrand SL et al. Retrospective review of osteoarticular infections in a pediatric sickle cell age group. Journal of Pediatric Orthopedics 2000; 20(5): 682-5.