| Asplenia as a cause of sudden unexpected death in childhood. | |
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MedLine Citation:
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PMID: 10208339 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Sudden unexpected death in childhood is rare. The commonest causes of such deaths are a result of fulminating infections of the respiratory or nervous systems. Other causes include unsuspected congenital abnormalities of the heart, acute metabolic disorders, and rarities such as internal hemorrhages and pulmonary thrombosis. Recognition of children with congenital asplenia who are otherwise normal but have an increased susceptibility to overwhelming sepsis is extremely difficult. We reviewed 1763 autopsy files from our institution over 5 years (1990-1995), of which 293 were classified as pediatric cases. The vast majority of the cases were stillbirths and deaths within the first year of life as a result of complex congenital anomalies. Four cases of asplenia were identified in our entire series, 3 of which were of the congenital syndromal variety and 1 of which was a case of isolated sporadic congenital asplenia. All 4 cases of asplenia were analyzed in detail with respect to autopsy findings and cause of death. Severe complex cardiac malformations were present in the congenital syndromal asplenia patients; these other malformations contributed significantly to their death. In this report, we discuss in detail the autopsy findings in a previously healthy 4-year-old girl who presented with a brief 8-hour history of being unwell and died within 4 hours of admission into the hospital. She had sporadic, isolated congenital asplenia complicated by high-grade type 6B pneumococcemia and acute bilateral adrenal hemorrhage (Waterhouse-Friderichsen syndrome). Previously healthy children who clinically deteriorate very rapidly should have a blood smear done as part of their clinical workup. The detection of Howell-Jolly bodies on a peripheral blood smear can be an indicator of asplenia, and this diagnosis can be confirmed by medical imaging of the abdomen. Such steps may aid in the aggressive management of isolated congenital asplenia and thereby avert untimely death. |
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Authors:
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R Kanthan; T Moyana; J Nyssen |
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Publication Detail:
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Type: Case Reports; Journal Article |
Journal Detail:
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Title: The American journal of forensic medicine and pathology Volume: 20 ISSN: 0195-7910 ISO Abbreviation: Am J Forensic Med Pathol Publication Date: 1999 Mar |
Date Detail:
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Created Date: 1999-06-07 Completed Date: 1999-06-07 Revised Date: 2011-02-02 |
Medline Journal Info:
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Nlm Unique ID: 8108948 Medline TA: Am J Forensic Med Pathol Country: UNITED STATES |
Other Details:
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Languages: eng Pagination: 57-9 Citation Subset: IM |
Affiliation:
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Department of Pathology, College of Medicine, Royal University Hospital, Saskatoon, Saskatchewan, Canada. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Abnormalities, Multiple* Adolescent Bacteremia / complications Child Child, Preschool Death, Sudden / etiology* Fatal Outcome Female Forensic Medicine Humans Infant Infant, Newborn Pneumonia, Pneumococcal / complications Spleen / abnormalities* Splenic Diseases / complications Waterhouse-Friderichsen Syndrome / complications |
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