Clinical course and outcome predictors of critically ill infants with complete DiGeorge anomaly following thymus transplantation.

Abstract

OBJECTIVES:

To identify risk factors for PICU admission and mortality of infants with complete DiGeorge anomaly treated with thymus transplantation. We hypothesized that age at transplantation and the presence of congenital heart disease would be risk factors for emergent PICU admission, and these factors plus development of septicemia would increase morbidity and mortality.

DESIGN:

Retrospective review.

SETTING:

Academic medical-surgical PICU.

PATIENTS:

All infants with complete DiGeorge anomaly treated with thymus transplantation between January 1, 1993, and July 1, 2010.

INTERVENTIONS:

None.

MEASUREMENTS AND MAIN RESULTS:

Consent was obtained from 71 infants with complete DiGeorge anomaly for thymus transplantation, and 59 infants were transplanted. Median age at transplantation was 5.0 months (range, 1.1-22.1 mo). After transplantation, 12 of 59 infants (20%) required 25 emergent PICU admissions. Seven of 12 infants (58%) survived to PICU discharge with six surviving 6 months posttransplantation. Forty-two of 59 infants (71%) transplanted had congenital heart disease, and 9 of 12 (75%) who were admitted to the PICU had congenital heart disease. In 15 of 25 admissions (60%), intubation and mechanical ventilation were necessary. There was no difference between median ventilation-free days between infants with and without congenital heart disease (33 d vs 23 d, p = 0.544). There was also no correlation between ventilation-free days and age of transplantation (R, 0.17; p = 0.423). Age at transplantation and the presence of congenital heart disease were not associated with risk for PICU admission (odds ratio, 0.95; 95% CI, 0.78-1.15 and odds ratio, 1.27; 95% CI, 0.30-5.49, respectively) or PICU mortality (odds ratio, 0.98; 95% CI, 0.73-1.31 and odds ratio, 0.40; 95% CI, 0.15-1.07, respectively).

CONCLUSIONS:

Most transplanted infants did not require emergent PICU admission. Age at transplantation and the presence of congenital heart disease were not associated with PICU admission or mortality.

• Authors: Jan Hau Lee, M Louise Markert, Christoph P Hornik, Elizabeth A McCarthy, Stephanie E Gupton, Ira M Cheifetz, David A Turner
• Journal: Pediatric critical care medicine : a journal of the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies (Pediatr Crit Care Med) Vol. 15 Issue 7 Pg. e321-6 (Sep 2014) ISSN: 1529-7535 [Print] United States
• PMID: 25068252 (Publication Type: Journal Article, Research Support, N.I.H., Extramural, Research Support, Non-U.S. Gov't)

Topics

• Age Factors
• Critical Care
• DiGeorge Syndrome (complications, mortality, surgery)
• Female
• Heart Defects, Congenital (complications)
• Hospitalization
• Humans
• Infant
• Male
• Retrospective Studies
• Risk Factors
• Sepsis (complications)
• Thymus Gland (transplantation)
• Treatment Outcome