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Deprecated: Implicit conversion from float 233.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 J+Exp+Med 2022 ; 219 (8): ä Nephropedia Template TP
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Recessive inborn errors of type I IFN immunity in children with COVID-19 pneumonia #MMPMID35708626
Zhang Q; Matuozzo D; Le Pen J; Lee D; Moens L; Asano T; Bohlen J; Liu Z; Moncada-Velez M; Kendir-Demirkol Y; Jing H; Bizien L; Marchal A; Abolhassani H; Delafontaine S; Bucciol G; Bayhan GI; Keles S; Kiykim A; Hancerli S; Haerynck F; Florkin B; Hatipoglu N; Ozcelik T; Morelle G; Zatz M; Ng LFP; Lye DC; Young BE; Leo YS; Dalgard CL; Lifton RP; Renia L; Meyts I; Jouanguy E; Hammarstrom L; Pan-Hammarstrom Q; Boisson B; Bastard P; Su HC; Boisson-Dupuis S; Abel L; Rice CM; Zhang SY; Cobat A; Casanova JL
J Exp Med 2022[Aug]; 219 (8): ä PMID35708626show ga
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 x 10-11) and with overlapping age, sex, consanguinity, and ethnicity characteristics. Recessive complete deficiencies of type I IFN immunity may underlie approximately 10% of hospitalizations for COVID-19 pneumonia in children.