Evidence map›Paper›PMID 33006109›Full record

ArticleJournal of clinical immunology2021

Infections in Infants with SCID: Isolation, Infection Screening, and Prophylaxis in PIDTC Centers.

Morna J Dorsey, Nicola A M Wright, Natalia S Chaimowitz, Blachy J Dávila Saldaña, Holly Miller, Michael D Keller, Monica S Thakar, Ami J Shah, Rolla Abu-Arja, Jeffrey Andolina and 49 more

Erratum issued Registry-linked trialOpen access · greenAbstract read
In one paragraph

Article in Journal of clinical immunology, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. An erratum has been issued. It is linked to trial NCT01186913 (A Prospective Natural History Study of Diagnosis, Treatment and Outcomes of Children With SCID Disorders), which is not on this map. Cited by 27 papers.

0numbers the graph read from it
0cells of the map it votes in
27citing papers in PubMed
3.1field-weighted citation impact, top 7% of its field
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

NCT01186913 enrolling by invitationnot on this map

A Prospective Natural History Study of Diagnosis, Treatment and Outcomes of Children With SCID Disorders (RDCRN PIDTC-6901)

TypeobservationalSponsorNational Institute of Allergy and Infectious Diseases (NIAID)Ran2010 to 2028Enrolled690ConditionsSevere Combined Immunodeficiency (SCID), Leaky SCID, Omenn Syndrome, Reticular Dysgenesis
3 · Its place in the literature

Who cites it

27 citing papers in PubMed, 55 citations in OpenAlex.

  1. Article
  2. Review
  3. Review
  4. Article
  5. Article
  6. Informed clinical decisions by outfoxing human FOXN1 variants.The Journal of allergy and clinical immunology · 2025
    Review
  7. First pilot study for newborn screening of severe T and B lymphopenias in ColombiaBiomedica : revista del Instituto Nacional de Salud · 2024
    Article
  8. Article
  9. Article
  10. Impact of newborn screening for SCID on the management of congenital athymia.The Journal of allergy and clinical immunology · 2024
    Article
  11. Article
  12. Article
  13. Article
  14. Article
  15. Review
  16. Article
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4 · The record

Corrections and comments

5 · Who and what money

Authors and funding

59 authors at 20 institutions in 2 countries.

Morna J DorseyDivision of Pediatric Allergy, Immunology, & Bone Marrow Transplant, Benioff Children's Hospital, University of California San Francisco, San Francisco, CA, USA.
Nicola A M WrightDivision of Hematology/Immunology, Department of Pediatrics, Alberta Children's Hospital, University of Calgary, Calgary, AB, Canada.
Natalia S ChaimowitzSection of Immunology, Allergy and Retrovirology, Department of Pediatrics, William T. Shearer Center for Human Immunobiology, Texas Children's Hospital, Baylor College of Medicine, Houston, TX, USA.
Blachy J Dávila SaldañaDivision of Blood and Marrow Transplantation, Children's National Medical Center, Washington, DC, USA.
Holly MillerCenter for Cancer and Blood Disorders, Phoenix Children's Hospital, Phoenix, AZ, USA.
Michael D KellerDivision of Allergy & Immunology, Children's National Health System, and Division of Pediatrics, George Washington University, Washington, DC, USA.
Monica S ThakarFred Hutchinson Cancer Research Center, University of Washington, Seattle, WA, USA.
Ami J ShahDivision of Stem Cell Transplantation and Regenerative Medicine, Lucille Packard Children's Hospital, Stanford School of Medicine, Stanford, CA, USA.
Rolla Abu-ArjaNationwide Children's Hospital, Columbus, OH, USA.
Jeffrey AndolinaDepartment of Pediatrics, Golisano Children's Hospital, University of Rochester Medical Center, Rochester, NY, USA.
Victor AquinoUniversity of Texas, Southwestern, Dallas, TX, USA.
J L BarnumUPMC Children's Hospital of Pittsburgh, Pittsburgh, PA, USA.
Jeffrey J BednarskiDepartment of Pediatrics, Washington University in St. Louis School of Medicine, St. Louis, MO, USA.
Monica BhatiaPediatric Stem Cell Transplant Columbia, University Irving Medical Center, New York, NY, USA.
Francisco A BonillaNortheast Allergy, Asthma & Immunology (private practice), Leominster, MA, USA.
Manish J ButteDivision of Immunology, Allergy, and Rheumatology, Department of Pediatrics, University of California Los Angeles, Los Angeles, CA, USA.
Nancy J BuninCellular Therapy and Transplant Section, Division of Oncology, Children's Hospital of Philadelphia, Philadelphia, PA, USA.
Sharat ChandraDivision of Bone Marrow Transplantation and Immune Deficiency, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.
Sonali ChaudhuryDivision of Pediatric Hematology, Oncology, Stem Cell Transplantation, Ann & Robert H Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, IL, USA.
Karin ChenDivision of Allergy and Immunology, Department of Pediatrics, University of Utah School of Medicine, Salt Lake City, UT, USA.
Hey ChongUPMC Children's Hospital of Pittsburgh, Pittsburgh, PA, USA.
Geoffrey D E CuvelierPediatric Blood and Marrow Transplant Program, CancerCare Manitoba, Department of Pediatrics and Child Health, Rady Faculty of Health Sciences, University of Manitoba, Winnipeg, MB, Canada.
Jignesh DalalPediatric Bone Marrow Transplant, Rainbow Babies and Children's Hospital, Cleveland, OH, USA.
Magee L DeFeliceDivision of Allergy and Immunology, Nemours/AI duPont Hospital for Children, Wilmington, DE, USA.
Kenneth B DeSantesDivision of Hematology, Oncology and Bone Marrow Transplant, Department of Pediatrics, University of Wisconsin School of Medicine, Madison, WI, USA.
Lisa R ForbesWilliam T Shearer Center for Human Immunobiology, Texas Children's Hospital, Baylor College of Medicine, Houston, TX, USA.
Alfred GillioJoseph M Sanzari's Childrens Hospital, Hackensack University Medical Center, Hackensack, NJ, USA.
Fred GoldmanDepartment of Pediatrics, Division of Hematology Oncology, University of Alabama at Birmingham, Birmingham, AL, USA.
Avni Y JoshiPediatric and Adult Allergy/Immunology, Mayo Clinic, Rochester, MN, USA.
Neena KapoorSection of Transplantation and Cellular Therapy, Children's Hospital Los Angeles Cancer and Blood Diseases Institute, USC Keck School of Medicine, Los Angeles, CA, USA.
Alan P KnutsenPediatric Allergy and Immunology, Cardinal Glennon Children's Hospital, St. Louis, MO, USA.
Lisa KobrynskiChildren's Healthcare of Atlanta, Emory University Department of Pediatrics, Allergy and Immunology, Atlanta, GA, USA.
Jay A LiebermanDepartment of Pediatrics, The University of Tennessee Health Science Center, Memphis, TN, USA.
Jennifer W LeidingDivision of Allergy and Immunology, Department of Pediatrics, University of South Florida, St. Petersburg, FL, USA.
Benjamin OshrineJohns Hopkins All Children's Hospital, Cancer and Blood Disorders Institute, St. Petersburg, FL, USA.
Kiran P PatelChildren's Healthcare of Atlanta, Atlanta, GA, USA.
Susan ProckopDepartment of Pediatrics, Bone Marrow Transplant Service, Memorial Sloan Kettering Cancer Center, New York, NY, USA.
Troy C QuiggPediatric Blood and Marrow Transplantation Program, Methodist Children's Hospital, San Antonio, TX, USA.
Ralph QuinonesPediatric Hematology, Oncology and Bone Marrow Transplant, Children's Hospital Colorado, Aurora, CO, USA.
Kirk R SchultzMichael Cuccione Childhood Cancer Research Program, BC Children's Hospital and Research Institute, Vancouver, British Columbia, Canada.
Christine SeroogyUniversity of Wisconsin School of Medicine and Public Health, Madison, WI, USA.
David ShyrDivision of Pediatric Hematology/Oncology, Department of Pediatrics, University of Utah School of Medicine, Primary Children's Hospital, Salt Lake City, UT, USA.
Subhadra SiegelDivision of Pediatric Pulmonology, Allergy and Immunology and Sleep Medicine, Westchester Medical Center, Valhalla, NY, USA.
Angela R SmithPediatric Blood and Marrow Transplantation, University of Minnesota, Minneapolis, MN, USA.
Troy R TorgersonFred Hutchinson Cancer Research Center, University of Washington, Seattle, WA, USA.
Mark T Vander LugtBlood and Marrow Transplant Program, University of Michigan, Ann Arbor, MI, USA.
Lolie C YuDivision of Heme-Onc/HSCT, Children's Hospital/LSUHSC, New Orleans, LA, USA.
Morton J CowanDivision of Pediatric Allergy, Immunology, & Bone Marrow Transplant, Benioff Children's Hospital, University of California San Francisco, San Francisco, CA, USA.
Rebecca H BuckleyDivision of Allergy and Immunology, Department of Pediatrics and Department of Immunology, Duke University School of Medicine, Durham, NC, USA.
Christopher C DvorakDivision of Pediatric Allergy, Immunology, & Bone Marrow Transplant, Benioff Children's Hospital, University of California San Francisco, San Francisco, CA, USA.
Linda M GriffithDivision of Allergy, Immunology and Transplantation, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, USA.
Elie HaddadPediatric Immunology and Rheumatology Division, CHU Sainte-Justine, Department of Pediatrics, Department of Microbiology, Immunology and Infectious Disease, University of Montreal, Montreal, QC, Canada.
Donald B KohnDepartment of Pediatrics, David Geffen School of Medicine, University of California, Los Angeles, Los Angeles, CA, USA.
Brent LoganDivision of Biostatistics, Medical College of Wisconsin, Milwaukee, WI, USA.
Luigi D NotarangeloLaboratory of Clinical Immunology and Microbiology, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, USA.
Sung-Yun PaiDivision of Hematology-Oncology, Boston Children's Hospital, Boston, MA, USA.
Jennifer PuckDivision of Pediatric Allergy, Immunology, & Bone Marrow Transplant, Benioff Children's Hospital, University of California San Francisco, San Francisco, CA, USA.
Michael A PulsipherSection of Transplantation and Cellular Therapy, Children's Hospital Los Angeles Cancer and Blood Diseases Institute, USC Keck School of Medicine, Los Angeles, CA, USA.
Jennifer HeimallDivision of Allergy and Immunology, Children's Hospital of Philadelphia, Wood 3301, 3401 Civic Center Blvd, Philadelphia, PA, 19104, USA. heimallj@email.chop.edu.ORCID 0000-0001-7939-5102
University of California, San Francisco · USBaylor College of Medicine · USChildren's Hospital of Los Angeles · USChildren's Hospital of Philadelphia · USChildren's Hospital of Pittsburgh · USChildren's National · USJohns Hopkins University · USNational Institutes of Health · USUniversity of California, Los Angeles · USUniversity of Washington · USUniversity of Wisconsin–Madison · USAsthma and Allergy Specialists · USBC Children's Hospital · CABoston Children's Hospital · USCancerCare Manitoba · CACardinal Glennon Children’s Medical Center · USCenter for Cancer and Blood Disorders · USCentre Hospitalier Universitaire Sainte-Justine · CAChildren's Healthcare of Atlanta · USChildren's Hospital Colorado · US

Funding

X-RAY CRYSTALLOGRAPHYP30CA008748 · NCI · SLOAN-KETTERING INSTITUTE FOR CANCER RES · PI Nicholas D Socci · 1985 to 2026
$347.4M
Data Resource for Analyzing Blood &Marrow TransplantsU24CA076518 · NCI · MEDICAL COLLEGE OF WISCONSIN · PI Amy M Moskop, Bronwen Shaw · 1998 to 2026
$105.2M
PB vs. BMU01HL069294 · NHLBI · MEDICAL COLLEGE OF WISCONSIN · PI HOROWITZ, MARY MARESCA · 2001 to 2010
$63.3M
Rare Diseases Data Management and Coordinating CenterU01TR001263 · NCATS · UNIVERSITY OF SOUTH FLORIDA · PI KRISCHER, JEFFREY P · 2014 to 2018
$28.1M
Training of Clinical Investigators in Primary Immune DeficienciesU54AI082973 · NIAID · UNIVERSITY OF CALIFORNIA, SAN FRANCISCO · PI PAI, SUNG-YUN · 2009 to 2023
$23.3M
Data Management and Coordinating Center (DMCC)U54NS064808 · NINDS · UNIVERSITY OF SOUTH FLORIDA · PI KRISCHER, JEFFREY P · 2009 to 2013
$16.5M
Characterization of the cellular and molecular bases of inborn errors of immunityZIAAI001222 · NIAID · NATIONAL INSTITUTE OF ALLERGY AND INFECTIOUS DISEASES · PI NOTARANGELO, LUIGI · 2017 to 2025
$15.2M
Blood and Marrow Transplant Clinical Trials NetworkU10HL069254 · NHLBI · UNIVERSITY OF UTAH · PI PULSIPHER, MICHAEL A · 2011 to 2016
$839k
Primary Immune Deficiency Treatment Consortium Annual Scientific MeetingR13AI094943 · NIAID · UNIVERSITY OF CALIFORNIA, SAN FRANCISCO · PI PUCK, JENNIFER M. · 2011 to 2025
$239k
NCATS NIH HHS U01 TR001263NCI NIH HHS P30 CA008748NCI NIH HHS U24 CA076518NHLBI NIH HHS U01 HL069294NHLBI NIH HHS U10 HL069254NIAID NIH HHS R13 AI094943NIAID NIH HHS U54 AI082973NINDS NIH HHS U54 NS064808
6 · The paper itself

Abstract

purposeThe 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.

methodsWe 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.

resultsInfections 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.

conclusionInfants 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 immunoglobulin support were more consistent. This study demonstrates a critical need for development of evidence-based guidelines for the pre-HSCT management of infants with SCID following an abnormal NBS.

trial registrationNCT01186913.

Indexed as

Infection ControlAge of OnsetAntibiotic ProphylaxisClinical Decision-MakingDisease ManagementDisease SusceptibilityFemaleHematopoietic Stem Cell TransplantationHumansInfantInfant, NewbornInfectionsMaleNeonatal ScreeningPrognosisPublic Health Surveillancehematopoietic stem cell transplantInfectionsnewborn screeningprimary immunodeficiencyprophylaxissevere combined immunodeficiency

Identifiers

PMID33006109
PMCPMC8388237
OpenAlexW3091060236

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Registered trials

Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.