title: Clinical course and outcome predictors of critically ill infants with complete DiGeorge anomaly following thymus transplantation.
abstract: OBJECTIVE
  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.
  
  
  METHODS
  Retrospective review.
  
  
  METHODS
  Academic medical-surgical PICU.
  
  
  METHODS
  All infants with complete DiGeorge anomaly treated with thymus transplantation between January 1, 1993, and July 1, 2010.
  
  
  METHODS
  None.
  
  
  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: 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.
authors: Jan Hau
authors: JH
authors: Lee
authors: M Louise
authors: ML
authors: Markert
authors: Christoph P
authors: CP
authors: Hornik
authors: Elizabeth A
authors: EA
authors: McCarthy
authors: Stephanie E
authors: SE
authors: Gupton
authors: Ira M
authors: IM
authors: Cheifetz
authors: David A
authors: DA
authors: Turner
mesh_terms: D000367:Age Factors; D003422:Critical Care; D004062:DiGeorge Syndrome / Q000150:complications / Q000401:mortality / Q000601:surgery*; D005260:Female; D006330:Heart Defects, Congenital / Q000150:complications; D006760:Hospitalization; D006801:Humans; D007223:Infant; D008297:Male; D012189:Retrospective Studies; D012307:Risk Factors; D018805:Sepsis / Q000150:complications; D013950:Thymus Gland / Q000637:transplantation*; D016896:Treatment Outcome