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Recessive inborn er...
Recessive inborn errors of type I IFN immunity in children with COVID-19 pneumonia
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Zhang, Q (author)
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Matuozzo, D (author)
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Le Pen, J (author)
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show more...
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Lee, D (author)
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Moens, L (author)
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Asano, T (author)
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Bohlen, J (author)
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Liu, ZY (author)
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Moncada-Velez, M (author)
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Kendir-Demirkol, Y (author)
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Jing, HE (author)
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Bizien, L (author)
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Marchal, A (author)
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- Abolhassani, H (author)
- Karolinska Institutet
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Delafontaine, S (author)
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Bucciol, G (author)
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Bayhan, GI (author)
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Keles, S (author)
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Kiykim, A (author)
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Hancerli, S (author)
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Haerynck, F (author)
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Florkin, B (author)
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Hatipoglu, N (author)
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Ozcelik, T (author)
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Morelle, G (author)
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Zatz, M (author)
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Ng, LFP (author)
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Lye, DC (author)
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Young, BE (author)
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Leo, YS (author)
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Dalgard, CL (author)
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Lifton, RP (author)
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Renia, L (author)
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Meyts, I (author)
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Jouanguy, E (author)
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- Hammarstrom, L (author)
- Karolinska Institutet
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- Pan-Hammarstrom, Q (author)
- Karolinska Institutet
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Boisson, B (author)
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Bastard, P (author)
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Su, HC (author)
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Boisson-Dupuis, S (author)
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Abel, L (author)
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Rice, CM (author)
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Zhang, SY (author)
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Cobat, A (author)
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Casanova, JL (author)
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(creator_code:org_t)
- 2022-06-16
- 2022
- English.
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In: The Journal of experimental medicine. - : Rockefeller University Press. - 1540-9538 .- 0022-1007. ; 219:8
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https://doi.org/10.1...
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Abstract
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- Recessive or dominant inborn errors of type I interferon (IFN) immunity can underlie critical COVID-19 pneumonia in unvaccinated adults. The risk of COVID-19 pneumonia in unvaccinated children, which is much lower than in unvaccinated adults, remains unexplained. In an international cohort of 112 children (<16 yr old) hospitalized for COVID-19 pneumonia, we report 12 children (10.7%) aged 1.5–13 yr with critical (7 children), severe (3), and moderate (2) pneumonia and 4 of the 15 known clinically recessive and biochemically complete inborn errors of type I IFN immunity: X-linked recessive TLR7 deficiency (7 children) and autosomal recessive IFNAR1 (1), STAT2 (1), or TYK2 (3) deficiencies. Fibroblasts deficient for IFNAR1, STAT2, or TYK2 are highly vulnerable to SARS-CoV-2. These 15 deficiencies were not found in 1,224 children and adults with benign SARS-CoV-2 infection without pneumonia (P = 1.2 × 10−11) and with overlapping age, sex, consanguinity, and ethnicity characteristics. Recessive complete deficiencies of type I IFN immunity may underlie ∼10% of hospitalizations for COVID-19 pneumonia in children.
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- ref (subject category)
- art (subject category)
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- By the author/editor
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Zhang, Q
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Matuozzo, D
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Le Pen, J
-
Lee, D
-
Moens, L
-
Asano, T
-
show more...
-
Bohlen, J
-
Liu, ZY
-
Moncada-Velez, M
-
Kendir-Demirkol, ...
-
Jing, HE
-
Bizien, L
-
Marchal, A
-
Abolhassani, H
-
Delafontaine, S
-
Bucciol, G
-
Bayhan, GI
-
Keles, S
-
Kiykim, A
-
Hancerli, S
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Haerynck, F
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Florkin, B
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Hatipoglu, N
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Ozcelik, T
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Morelle, G
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Zatz, M
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Ng, LFP
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Lye, DC
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Young, BE
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Leo, YS
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Dalgard, CL
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Lifton, RP
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Renia, L
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Meyts, I
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Jouanguy, E
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Hammarstrom, L
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Pan-Hammarstrom, ...
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Boisson, B
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Bastard, P
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Su, HC
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Boisson-Dupuis, ...
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Abel, L
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Rice, CM
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Zhang, SY
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Cobat, A
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Casanova, JL
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show less...
- Articles in the publication
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The Journal of e ...
- By the university
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Karolinska Institutet