Infections in Infants with SCID: Isolation, Infection Screening, and Prophylaxis in PIDTC Centers
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ORIGINAL ARTICLE
Infections in Infants with SCID: Isolation, Infection Screening, and Prophylaxis in PIDTC Centers Morna J. Dorsey 1 & Nicola A. M. Wright 2 & Natalia S. Chaimowitz 3 & Blachy J. Dávila Saldaña 4,5 & Holly Miller 6 & Michael D. Keller 7 & Monica S. Thakar 8 & Ami J. Shah 9 & Rolla Abu-Arja 10 & Jeffrey Andolina 11 & Victor Aquino 12 & J. L. Barnum 13 & Jeffrey J. Bednarski 14 & Monica Bhatia 15 & Francisco A. Bonilla 16 & Manish J. Butte 17 & Nancy J. Bunin 18 & Sharat Chandra 19,20 & Sonali Chaudhury 21 & Karin Chen 22 & Hey Chong 13 & Geoffrey D. E. Cuvelier 23 & Jignesh Dalal 24 & Magee L. DeFelice 25 & Kenneth B. DeSantes 26 & Lisa R. Forbes 27 & Alfred Gillio 28 & Fred Goldman 29 & Avni Y. Joshi 30 & Neena Kapoor 31 & Alan P. Knutsen 32 & Lisa Kobrynski 33 & Jay A. Lieberman 34 & Jennifer W. Leiding 35,36 & Benjamin Oshrine 36 & Kiran P. Patel 37 & Susan Prockop 38 & Troy C. Quigg 39 & Ralph Quinones 40 & Kirk R. Schultz 41 & Christine Seroogy 42 & David Shyr 43,44 & Subhadra Siegel 45 & Angela R. Smith 46 & Troy R. Torgerson 8 & Mark T. Vander Lugt 47 & Lolie C. Yu 48 & Morton J. Cowan 1 & Rebecca H. Buckley 49 & Christopher C. Dvorak 1 & Linda M. Griffith 50 & Elie Haddad 51 & Donald B. Kohn 52 & Brent Logan 53 & Luigi D. Notarangelo 54 & Sung-Yun Pai 55,56 & Jennifer Puck 1 & Michael A. Pulsipher 31 & Jennifer Heimall 57 Received: 22 May 2020 / Accepted: 7 September 2020 # Springer Science+Business Media, LLC, part of Springer Nature 2020
Abstract Purpose The Primary Immune Deficiency Treatment Consortium (PIDTC) enrolled children with severe combined immunodeficiency (SCID) in a prospective natural history study of hematopoietic stem cell transplant (HSCT) outcomes over the last decade. Despite newborn screening (NBS) for SCID, infections occurred prior to HSCT. This study’s objectives were to define the types and timing of infection prior to HSCT in patients diagnosed via NBS or by family history (FH) and to understand the breadth of strategies employed at PIDTC centers for infection prevention. Methods We analyzed retrospective data on infections and pre-transplant management in patients with SCID diagnosed by NBS and/or FH and treated with HSCT between 2010 and 2014. PIDTC centers were surveyed in 2018 to understand their practices and protocols for pre-HSCT management. Results Infections were more common in patients diagnosed via NBS (55%) versus those diagnosed via FH (19%) (p = 0.012). Outpatient versus inpatient management did not impact infections (47% vs 35%, respectively; p = 0.423). There was no consensus among PIDTC survey respondents as to the best setting (inpatient vs outpatient) for pre-HSCT management. While isolation practices varied, immunoglobulin replacement and antimicrobial prophylaxis were more uniformly implemented. Conclusion Infants with SCID diagnosed due to FH had lower rates of infection and proceeded to HSCT more quickly than did those diagnosed via NBS. Pre-HSCT management practices were highly variable between centers, although uses of prophylaxis and immu
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