50 Years Ago in The Journal of Pediatrics:
Sickle cell disease with salmonella osteomyelitis
Article Outline
Roberts AR, Hilburg LE. J Pediatr 1958;53:170-5
The following scenario is familiar to all clinicians who provide care for children with sickle cell disease: a toddler with HbSS presents with fever and pain, either dactylitis or other localized bone tenderness. Is this just another vaso-occlusive pain crisis or could this be osteomyelitis?
Although Salmonella osteomyelitis in sickle cell disease was first reported over 70 years ago,1 the article by Roberts and Hilburg 50 years ago2 was among the first to recognize the “more than coincidental” propensity of Salmonella species to cause osteomyelitis in this patient population. The authors described four African-American children with sickle cell disease who developed Salmonella osteomyelitis: three cases in the phalanges and one in the humeral head, including the first report of an affected child with HbSC disease. After appropriate drainage, all four recovered with antibiotic therapy; numerous Salmonella species were identified. The authors noted that Salmonella osteomyelitis in sickle cell disease was unusual because it developed without trauma, involved no predominant species, and could be multifocal. Furthermore, they noted that early radiographic changes did not distinguish osteomyelitis from uncomplicated sickle cell disease. Finally, they speculated that transient Salmonella bacteremia might find a reservoir in dead bone marrow often found in patients with sickle cell disease, thereby explaining this unusual association. Collectively, these observations were remarkably prescient; our current understanding of the pathophysiology of Salmonella osteomyelitis is essentially unchanged from this original discussion. The authors also noted the difficulty in differentiating a sickle cell vaso-occlusive crisis with bone involvement from true bone infection, which remains a clinical challenge even today.
In the current era of new pathogens and changing patterns of antibiotic resistance, it is noteworthy that many years after this important early contribution of Roberts and Hilburg, Salmonella osteomyelitis remains a serious but treatable pathogen for children with sickle cell disease.3 In 1997, Wright et al4 described 32 additional episodes of Salmonella osteomyelitis, noting a frequent association with preceding avascular necrosis of bone. Today’s clinicians, just like those 50 years ago, must be ever vigilant when providing care for children with sickle cell disease who present with fever and pain—it just might be osteomyelitis.
References
- . The bone changes in sickle cell anemia. South Med J. 1937;30:249
- . Sickle cell disease with Salmonella osteomyelitis. J Pediatr. 1958;53:170–175
- . Salmonella infection in sickle cell disease: A clear and present danger. J Pediatr. 1997;130:350–351
- . Septicemia caused by salmonella infection: an overlooked complication of sickle cell disease. J Pediatr. 1997;130:394–399
PII: S0022-3476(07)00796-2
doi:10.1016/j.jpeds.2007.08.030
© 2008 Mosby, Inc. All rights reserved.
