An overview of how on‐call consultant paediatricians can recognise and manage severe primary immunodeficiencies

Severe primary paediatric immunodeficiency syndromes are rare and potentially fatal unless suspected, diagnosed and treated early. We provide clinical guidance and support for on‐call consultant paediatricians working in secondary level hospitals on how to recognise and manage children with these co...

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Bibliographic Details
Published in:Acta Paediatrica 2019-12, Vol.108 (12), p.2175-2185
Main Authors: Wekell, Per, Hertting, Olof, Holmgren, Daniel, Fasth, Anders
Format: Article
Language:English
Subjects:
Child
Chronic granulomatous disease
Clinical Medicine
Congenital Bone Marrow Failure Syndromes - diagnosis
Diagnosis, Differential
Granulomatous Disease, Chronic - diagnosis
haemophagocytic lymphohistiocytosis
Humans
Klinisk medicin
Lymphohistiocytosis, Hemophagocytic - diagnosis
Medicin och hälsovetenskap
Neutropenia - congenital
Neutropenia - diagnosis
Pediatrics
primary immune deficiencies
Primary immunodeficiencies
Referral and Consultation
Severe combined immunodeficiency
Severe Combined Immunodeficiency - diagnosis
severe congenital neutropaenia
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Summary:Severe primary paediatric immunodeficiency syndromes are rare and potentially fatal unless suspected, diagnosed and treated early. We provide clinical guidance and support for on‐call consultant paediatricians working in secondary level hospitals on how to recognise and manage children with these conditions. Our paper addresses four conditions that risk the most severe outcomes if they are not adequately cared for during on‐call periods, such as weekends: severe combined immunodeficiency, haemophagocytic lymphohistiocytosis, severe congenital neutropaenia and chronic granulomatous disease. Conclusion On‐call paediatricians are provided with advice on handling the most severe primary immunodeficiencies.
ISSN:0803-5253
1651-2227
1651-2227
DOI:10.1111/apa.14930