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. 2018 Nov 12;19:219. doi: 10.1186/s12931-018-0923-8

Table 3.

Most common extrapulmonary manifestations of PID (adapted from JC Aldave, JFM) [27]

MOST COMMON EXTRAPULMONARY MANIFESTATIONS OF PID N (Composition of panel) Votes in agreement (%) Degree of agreement
 Recurrent or complicated sinusitis 43 (AI, AP, PI, PP) 100.0 Unanimity
 Recurrent or complicated otitis 43 (AI, AP, PI, PP) 93.0 Consensus
 Other extrapulmonary infections requiring admission 43 (AI, AP, PI, PP) 88.4 Consensus
 Ectodermal dysplasia 43 (AI, AP, PI, PP) 72.1 Majority
 Chronic diarrhea or malabsorption 43 (AI, AP, PI, PP) 93.0 Consensus
 Difficult-to-treat giardiasis 43 (AI, AP, PI, PP) 93.0 Consensus
 Autoimmune cytopenias or other autoimmunity 43 (AI, AP, PI, PP) 93.0 Consensus
 Lymphadenopathies and hepatosplenomegaly 43 (AI, AP, PI, PP) 97.7 Consensus
 Related cancers, especially associated with viruses 43 (AI, AP, PI, PP) 83.7 Consensus
 Family history of ID or consistent manifestations 43 (AI, AP, PI, PP) 88.4 Consensus
 Post-vaccinal infections 43 (AI, AP, PI, PP) 79.1 Majority

AI: adult immunologists; AP: adult pulmonologists; ID: immunodeficiency; PI: pediatric immunologists; PID: primary immunodeficiency disease; PP: pediatric pulmonologists