Abstract | BACKGROUND: OBJECTIVES: This study aimed to compare clinical outcomes (mortality and morbidity) in children managed conservatively and curatively. METHODS: Children were identified from specialist centers and advertising through special interest groups. Clinical data were collected from medical records regarding infections, inflammatory complications and growth, other admissions, and curative treatment. Comparisons were made for patients not undergoing HSCT and patients after HSCT. RESULTS: Seventy-three living children were identified, 59 (80%) of whom were recruited. Five deceased children were also identified. Clinical information was available for 62 children (4 deceased). Thirty (48%) children had undergone HSCT. Children who did not undergo transplantation had 0.71 episodes of infection/admission/surgery per CGD life year (95% CI, 0.69-0.75 events per year). Post-HSCT children had 0.15 episodes of infection/admission/surgery per transplant year (95% CI, 0.09-0.21 events per year). The mean z score for height and body mass index (BMI) for age was significantly better in post-HSCT children. Survival in the non-HSCT group was 90% at age 15 years. Survival in the post-HSCT group was 90%. CONCLUSIONS: Children with CGD not undergoing transplantation have more serious infections, episodes of surgery, and admissions compared with post-HSCT children. Children undergoing transplantation have better height for age. Survival is good at the end of the pediatric age range and also after HSCT.
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Authors | Theresa Cole, Mark S Pearce, Andrew J Cant, Catherine M Cale, David Goldblatt, Andrew R Gennery |
Journal | The Journal of allergy and clinical immunology
(J Allergy Clin Immunol)
Vol. 132
Issue 5
Pg. 1150-5
(Nov 2013)
ISSN: 1097-6825 [Electronic] United States |
PMID | 23870668
(Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
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Copyright | Copyright © 2013 American Academy of Allergy, Asthma & Immunology. Published by Mosby, Inc. All rights reserved. |
Topics |
- Adolescent
- Child
- Child, Preschool
- Female
- Granulomatous Disease, Chronic
(epidemiology, therapy)
- Hematopoietic Stem Cell Transplantation
- Humans
- Infant
- Infant, Newborn
- Infections
(etiology)
- Ireland
- Male
- Morbidity
- Mortality
- Treatment Outcome
- United Kingdom
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