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Journal of clinical immunology · 2020

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

СтатьяПоддержка NIH: внешняя программаResearch Support, N.I.H., IntramuralПоддержка исследования: вне правительства СШАПоддержка правительства СШАПоддержка службы здравоохранения СШАНазвание и аннотация: Перевод пока не подготовлен
Обработка статьи · этапы, ошибки и управление
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Dorsey Morna J, Wright Nicola A M, Chaimowitz Natalia S, Dávila Saldaña Blachy J, Miller Holly, Keller Michael D, Thakar Monica S, Shah Ami J, Abu-Arja Rolla, Andolina Jeffrey, Aquino Victor, Barnum J L, Bednarski Jeffrey J, Bhatia Monica, Bonilla Francisco A, Butte Manish J, Bunin Nancy J, Chandra Sharat, Chaudhury Sonali, Chen Karin, Chong Hey, Cuvelier Geoffrey D E, Dalal Jignesh, DeFelice Magee L, DeSantes Kenneth B, Forbes Lisa R, Gillio Alfred, Goldman Fred, Joshi Avni Y, Kapoor Neena, Knutsen Alan P, Kobrynski Lisa, Lieberman Jay A, Leiding Jennifer W, Oshrine Benjamin, Patel Kiran P, Prockop Susan, Quigg Troy C, Quinones Ralph, Schultz Kirk R, Seroogy Christine, Shyr David, Siegel Subhadra, Smith Angela R, Torgerson Troy R, Vander Lugt Mark T, Yu Lolie C, Cowan Morton J, Buckley Rebecca H, Dvorak Christopher C, Griffith Linda M, Haddad Elie, Kohn Donald B, Logan Brent, Notarangelo Luigi D, Pai Sung-Yun, Puck Jennifer, Pulsipher Michael A, Heimall Jennifer

Аннотация

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 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 REGISTRATION: NCT01186913.

Предметные рубрики MeSH

ИП Урсолов Владлен Сергеевич

ИНН 500118482352 · ОГРНИП 314500117700049

Реквизиты и контакты