Congenital asplenia study: clinical and laboratory characterisation of adults with congenital asplenia.
Congenital asplenia
Congenital polysplenia
Howell-Jolly bodies
Spleen
Journal
Annals of hematology
ISSN: 1432-0584
Titre abrégé: Ann Hematol
Pays: Germany
ID NLM: 9107334
Informations de publication
Date de publication:
Jul 2022
Jul 2022
Historique:
received:
13
09
2021
accepted:
11
01
2022
pubmed:
23
4
2022
medline:
22
6
2022
entrez:
22
4
2022
Statut:
ppublish
Résumé
Congenital asplenia is a rare disorder commonly associated with other visceral and cardiac congenital anomalies. Isolated congenital asplenia is even less common than syndromic forms. The risk of severe bacterial infections associated with asplenia is the most concerning clinical implication and carries a significant mortality risk. Prophylactic measures against the clinical syndrome known as overwhelming postsplenectomy infections (OPSI) include vaccination, prophylactic and emergency antibiotics and health education including fever management and travel advice. This case series describes fourteen adults with congenital asplenia and polysplenia syndrome, most of whom were diagnosed incidentally as adults, and outlines the nature of their diagnosis, clinical phenotype, family history and key pathology findings.
Identifiants
pubmed: 35451619
doi: 10.1007/s00277-022-04765-3
pii: 10.1007/s00277-022-04765-3
doi:
Substances chimiques
Anti-Bacterial Agents
0
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
1421-1434Informations de copyright
© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.
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