A review of the hospital records of all patients coded with a discharge diagnosis of hereditary hemoglobin abnormality and acute or chronic osteomyelitis at the Johns Hopkins Hospital during the period 1958 to 1968 identified ten children with hemoglobinopathies and fully documented bone infections caused by gram-negative organisms. The ages of these patients ranged from eleven months to nine years. Seven had SS hemoglobin; two, S-thalassemia; and one, SC hemoglobin. The infections were caused by Salmonella typhimurium in three patients, by Salmonellaheidelberg in two, and by Salmonella derby, Escherichia coli, and Haemophilus influenzae in one each. One patient died of a fatal septicemia. The others survived and recovered completely from their bone infection. There were no joint infections.

The notable findings in this study were as follows:

The unique susceptibility of patients with sickle-cell disease to bone infection, especially Salmonella paratyphosa infection, was confirmed and the possible causative factors were reviewed and discussed.

The characteristic and inevitable roentgenographic osseous changes were found not to be related to the type of infecting organism, its virulence, or the success or failure of therapy, but rather to the combined effects of the abnormality in hemoglobin and the infection.

The problems and pitfalls in diagnosis and treatment were illustrated and discussed.

The effectiveness of chloramphenicol in eradicating salmonella infection despite the presence of pronounced roentgenographic changes in the affected bone or bones was demoustrated. The complications of chloransphenicol therapy and their prevention were also described.