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Isolated Congenital Asplenia: An Occult Case of Overwhelming Sepsis

Isolated Congenital Asplenia: An Occult Case of Overwhelming Sepsis Abstract 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 References 1. Waldman JD, Rosenthal A, Smith AL, et al.: Sepsis and congenital asplenia . J Pediatr 90:555-559, 1977.Crossref 2. Freedom RM: The asplenic syndrome: A review of significant extracardiac structural abnormalities in 29 necropsied patients . J Pediatr 81:1130-1133, 1972.Crossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png American Journal of Diseases of Children American Medical Association

Isolated Congenital Asplenia: An Occult Case of Overwhelming Sepsis

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References (2)

Publisher
American Medical Association
Copyright
Copyright © 1979 American Medical Association. All Rights Reserved.
ISSN
0002-922X
DOI
10.1001/archpedi.1979.02130050096021
Publisher site
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Abstract

Abstract 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 References 1. Waldman JD, Rosenthal A, Smith AL, et al.: Sepsis and congenital asplenia . J Pediatr 90:555-559, 1977.Crossref 2. Freedom RM: The asplenic syndrome: A review of significant extracardiac structural abnormalities in 29 necropsied patients . J Pediatr 81:1130-1133, 1972.Crossref

Journal

American Journal of Diseases of Children – American Medical Association

Published: May 1, 1979

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