The absent and vanishing spleen: congenital asplenia and hyposplenism--two case reports. Acta paediatrica (Oslo, Norway : 1992) [Acta Paediatr] Journal article | | Title | The absent and vanishing spleen: congenital asplenia and hyposplenism--two case reports. | | Author(s) | Halbertsma FJ, Neeleman C, Weemaes CM, van Deuren M | | Institution | Department of Paediatric Intensive Care, University Medical Centre St. Radboud, Nijmegen, The Netherlands. f.halbertsma@ic.umcn.nl | | Source | Acta Paediatr 2005 Mar; 94(3):369-71. | | MeSH | Child, Preschool
Erythrocyte Inclusions
Fatal Outcome
Female
Humans
Regional Blood Flow
Spleen
| | Abstract | Two unrelated patients are reported: one with isolated familial asplenia diagnosed postmortem, the other with isolated hyposplenism diagnosed after recurring invasive bacterial infections. Because both children died of fulminant septic shock, the importance of early diagnosis of splenic dysfunction is evident. Clues for an early diagnosis of congenital asplenia are recurrent invasive bacterial infections, Howell-Jolly bodies in the blood smear or a relative with congenital isolated asplenia. Although the guidelines for infection prevention in asplenism--patient education, antibiotic prophylaxis and vaccination--are well defined, controversy remains as to how to differentiate hyposplenism from functional asplenism.
Conclusion: Based on the present observations, we define a patient as functionally asplenic--and therefore at risk for life-threatening infections-when Howell-Jolly bodies are present in the blood smear, a very small spleen is found by ultrasound, or splenic blood flow is compromised. | | Language | eng | | Pub Type(s) | Case Reports
Journal Article
| | PubMed ID | 16028659 |
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