Congenital asplenia study: clinical and laboratory characterisation of adults with congenital asplenia.

Autor: Butel-Simoes GI; Monash Infectious Diseases, Monash Health, Victoria, Australia. gbutelsimoes@gmail.com., Jones P; Spleen Australia, Alfred Health, Victoria, Australia.; Department of Infectious Diseases, Alfred Health, Victoria, Australia., Wood EM; School of Public Health and Preventive Medicine, Monash University, Victoria, Australia.; Department of Haematology, Monash Health, Victoria, Australia., Spelman D; Spleen Australia, Alfred Health, Victoria, Australia.; Department of Infectious Diseases, Alfred Health, Victoria, Australia., Woolley IJ; Monash Infectious Diseases, Monash Health, Victoria, Australia.; Spleen Australia, Alfred Health, Victoria, Australia.; Department of Infectious Diseases, Alfred Health, Victoria, Australia.; Centre for Inflammatory Diseases, School of Clinical Sciences, Monash University, Victoria, Australia., Ojaimi S; Monash Infectious Diseases, Monash Health, Victoria, Australia.; Centre for Inflammatory Diseases, School of Clinical Sciences, Monash University, Victoria, Australia.; Immunology Laboratory, Monash Pathology, Monash Health, Victoria, Australia.
Jazyk: angličtina
Zdroj: Annals of hematology [Ann Hematol] 2022 Jul; Vol. 101 (7), pp. 1421-1434. Date of Electronic Publication: 2022 Apr 22.
DOI: 10.1007/s00277-022-04765-3
Abstrakt: 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.
(© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)
Databáze: MEDLINE
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