Clinical Course and Outcome Predictors of Critically Ill... : Pediatric Critical Care Medicine (original) (raw)

Online Clinical Investigations

Clinical Course and Outcome Predictors of Critically Ill Infants With Complete DiGeorge Anomaly Following Thymus Transplantation

Lee, Jan Hau MBBS, MRCPCH1,2,3; Markert, M. Louise MD, PhD4,5; Hornik, Christoph P. MD, MPH1,2; McCarthy, Elizabeth A. RN, MSN4; Gupton, Stephanie E. RN, MSN, CPNP4; Cheifetz, Ira M. MD, FCCM, FAARC1; Turner, David A. MD1

1Division of Pediatric Critical Care, Department of Pediatrics, Duke Children’s Hospital, Durham, NC.

2Duke Clinical Research Institute, Duke University Medical Center, Durham, NC.

3Children’s Intensive Care Unit, Department of Paediatric Subspecialties, KK Women’s and Children’s Hospital, Singapore.

4Division of Pediatric Allergy and Immunology, Department of Pediatrics, Duke Children’s Hospital, Durham, NC.

5Department of Immunology, Duke University Medical Center, Durham, NC.

Current address for Dr. McCarthy: Department of Surgery, Duke University Medical Center, Durham, NC.

Presented, in part, in an abstract format at the Society of Critical Care Medicine’s 42nd Critical Care Congress, San Juan, Puerto Rico, January 2013.

Dr. Hornik receives salary support for research from the National Center for Advancing Translational Sciences of the National Institutes of Health (UL1TR001117). Dr. Markert is employed by Duke University, lectured for various entities (various seminars and grand rounds), has stock options with various entities, and received support for travel from various entities (various seminars and grand rounds). Her institution received grant support from the National Institutes of Health (NIH) and the Hartwell Foundation. Dr. McCarthy is employed by Duke University. Her institution received grant support from the NIH. Dr. Cheifetz consulted for Philips, Hill-Rom, and Teleflex (Medical Advisory Board) and provided expert testimony for various entities. His institution received grant support from Covidien, Teleflex, CareFusion, and Philips. The remaining authors have disclosed that they do not have any potential conflicts of interest.

For information regarding this article, E-mail: [email protected]

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.