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May 1979

Isolated Congenital Asplenia: An Occult Case of Overwhelming Sepsis

Author Affiliations

School of Medicine of the Health Sciences Center State University of New York Stony Brook, NY; Department of Pediatrics Long Island Jewish-Hillside Medical Center New Hyde Park, NY 11040

Am J Dis Child. 1979;133(5):552-553. doi:10.1001/archpedi.1979.02130050096021

Overwhelming bacterial infections occur more frequently in children in whom a splenectomy was performed, in children with a condition of autosplenectomy who have sickle cell disease, and in children who have the asplenia syndrome (Ivermark's syndrome: congenital absence of the spleen, cardiovascular anomalies, and other malformations). It is more difficult to recognize children with isolated congenital asplenia who are otherwise normal but who have increased susceptibility to overwhelming bacterial sepsis. The purpose of this report is to draw attention to the latter entity and to stress the use of appropriate prophylaxis aimed at preventing recurrent sepsis in these patients.

Report of a Case.—An 8-week-old girl was admitted to Long Island Jewish-Hillside Medical Center, New Hyde Park, NY, with a one-week history of poor feeding and irritability. On the day of admission, focal right-sided seizures progressing to generalized seizures developed. Her temperature was 39.4 °C, and she was stuporous, responded

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