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. 2019 Dec;108(12):2175-2185.
doi: 10.1111/apa.14930. Epub 2019 Aug 23.

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

Per Wekell #  1   2 , Olof Hertting #  3   4 , Daniel Holmgren  2   5 , Anders Fasth  2   6
Affiliations

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

Per Wekell et al. Acta Paediatr. 2019 Dec.

Abstract

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.

Keywords: chronic granulomatous disease; haemophagocytic lymphohistiocytosis; primary immune deficiencies; severe combined immunodeficiency; severe congenital neutropaenia.

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