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2014 ; 15
(7
): e321-6
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Clinical course and outcome predictors of critically ill infants with complete
DiGeorge anomaly following thymus transplantation
#MMPMID25068252
Lee JH
; Markert ML
; Hornik CP
; McCarthy EA
; Gupton SE
; Cheifetz IM
; Turner DA
Pediatr Crit Care Med
2014[Sep]; 15
(7
): e321-6
PMID25068252
show ga
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.