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Your search keyword '"Heimall, Jennifer R."' showing total 4 results
Start Over Author "Heimall, Jennifer R." Journal journal of allergy and clinical immunology
4 results on '"Heimall, Jennifer R."'

1. Intestinal microbiome and metabolome signatures in patients with chronic granulomatous disease

Author

Chandrasekaran, Prabha, primary, Han, Yu, additional, Zerbe, Christa S., additional, Heller, Theo, additional, DeRavin, Suk See, additional, Kreuzberg, Samantha A., additional, Marciano, Beatriz E., additional, Siu, Yik, additional, Jones, Drew R., additional, Abraham, Roshini S., additional, Stephens, Michael C., additional, Tsou, Amy M., additional, Snapper, Scott, additional, Conlan, Sean, additional, Subramanian, Poorani, additional, Quinones, Mariam, additional, Grou, Caroline, additional, Calderon, Virginie, additional, Deming, Clayton, additional, Leiding, Jennifer W., additional, Arnold, Danielle E., additional, Logan, Brent R., additional, Griffith, Linda M., additional, Petrovic, Aleksandra, additional, Mousallem, Talal I., additional, Kapoor, Neena, additional, Heimall, Jennifer R., additional, Barnum, Jessie L., additional, Kapadia, Malika, additional, Wright, Nicola, additional, Rayes, Ahmad, additional, Chandra, Sharat, additional, Broglie, Larisa A., additional, Chellapandian, Deepak, additional, Deal, Christin L., additional, Grunebaum, Eyal, additional, Lim, Stephanie Si, additional, Mallhi, Kanwaldeep, additional, Marsh, Rebecca A., additional, Murguia-Favela, Luis, additional, Parikh, Suhag, additional, Touzot, Fabien, additional, Cowan, Morton J., additional, Dvorak, Christopher C., additional, Haddad, Elie, additional, Kohn, Donald B., additional, Notarangelo, Luigi D., additional, Pai, Sung-Yun, additional, Puck, Jennifer M., additional, Pulsipher, Michael A., additional, Torgerson, Troy R., additional, Kang, Elizabeth M., additional, Malech, Harry L., additional, Segre, Julia A., additional, Bryant, Clare E., additional, Holland, Steven M., additional, and Falcone, Emilia Liana, additional

Published
2023
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4. Allogeneic hematopoietic cell transplantation is effective for p47phox chronic granulomatous disease: A  Primary Immune Deficiency Treatment Consortium study.

Author

Grunebaum E, Arnold DE, Logan B, Parikh S, Marsh RA, Griffith LM, Mallhi K, Chellapandian D, Lim SS, Deal CL, Kapoor N, Murguía-Favela L, Falcone EL, Prasad VK, Touzot F, Bleesing JJ, Chandrakasan S, Heimall JR, Bednarski JJ, Broglie LA, Chong HJ, Kapadia M, Prockop S, Dávila Saldaña BJ, Schaefer E, Bauchat AL, Teira P, Chandra S, Parta M, Cowan MJ, Dvorak CC, Haddad E, Kohn DB, Notarangelo LD, Pai SY, Puck JM, Pulsipher MA, Torgerson TR, Malech HL, Kang EM, and Leiding JW

Subjects
Humans, Male, Female, Child, Child, Preschool, Adolescent, Infant, Young Adult, Transplantation, Homologous, Transplantation Conditioning methods, Graft vs Host Disease, Adult, Treatment Outcome, Granulomatous Disease, Chronic therapy, Granulomatous Disease, Chronic genetics, Hematopoietic Stem Cell Transplantation, NADPH Oxidases genetics
Abstract

Background: P47phox (neutrophil cytosolic factor-1) deficiency is the most common cause of autosomal recessive chronic granulomatous disease (CGD) and is considered to be associated with a milder clinical phenotype. Allogeneic hematopoietic cell transplantation (HCT) for p47phox CGD is not well-described., Objectives: We sought to study HCT for p47phox CGD in North America., Methods: Thirty patients with p47phox CGD who received allogeneic HCT at Primary Immune Deficiency Treatment Consortium centers since 1995 were included., Results: Residual oxidative activity was present in 66.7% of patients. In the year before HCT, there were 0.38 CGD-related infections per person-years. Inflammatory diseases, predominantly of the lungs and bowel, occurred in 36.7% of the patients. The median age at HCT was 9.1 years (range 1.5-23.6 years). Most HCTs (90%) were performed after using reduced intensity/toxicity conditioning. HCT sources were HLA-matched (40%) and -mismatched (10%) related donors or HLA-matched (36.7%) and -mismatched (13.3%) unrelated donors. CGD-related infections after HCT decreased significantly to 0.06 per person-years (P = .038). The frequency of inflammatory bowel disease and the use of steroids also decreased. The cumulative incidence of graft failure and second HCT was 17.9%. The 2-year overall and event-free survival were 92.3% and 82.1%, respectively, while at 5 years they were 85.7% and 77.0%, respectively. In the surviving patients evaluated, ≥95% donor myeloid chimerism at 1 and 2 years after HCT was 93.8% and 87.5%, respectively., Conclusions: Patients with p47phox CGD suffer from a significant disease burden that can be effectively alleviated by HCT. Similar to other forms of CGD, HCT should be considered for patients with p47phox CGD., (Copyright © 2024 American Academy of Allergy, Asthma & Immunology. All rights reserved.)

Published
2024
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