Jan Hau Lee1, M Louise Markert, Christoph P Hornik, Elizabeth A McCarthy, Stephanie E Gupton, Ira M Cheifetz, David A Turner. 1. 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.
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.
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: Ales Janda; Petr Sedlacek; Manfred Hönig; Wilhelm Friedrich; Martin Champagne; Tadashi Matsumoto; Alain Fischer; Benedicte Neven; Audrey Contet; Danielle Bensoussan; Pierre Bordigoni; David Loeb; William Savage; Nada Jabado; Francisco A Bonilla; Mary A Slatter; E Graham Davies; Andrew R Gennery Journal: Blood Date: 2010-06-07 Impact factor: 22.113
Authors: M L Markert; D S Hummell; H M Rosenblatt; S E Schiff; T O Harville; L W Williams; R I Schiff; R H Buckley Journal: J Pediatr Date: 1998-01 Impact factor: 4.406
Authors: T S Cole; I C Johnstone; M S Pearce; B Fulton; A J Cant; A R Gennery; M A Slatter Journal: Bone Marrow Transplant Date: 2011-02-28 Impact factor: 5.483
Authors: M Louise Markert; Marilyn J Alexieff; Jie Li; Marcella Sarzotti; Daniel A Ozaki; Blythe H Devlin; Debra A Sedlak; Gregory D Sempowski; Laura P Hale; Henry E Rice; Samuel M Mahaffey; Michael A Skinner Journal: Blood Date: 2004-04-20 Impact factor: 22.113
Authors: Josephus P J van Gestel; Casper W Bollen; Marc B Bierings; Jaap Jan Boelens; Nico M Wulffraat; Adrianus J van Vught Journal: Biol Blood Marrow Transplant Date: 2008-12 Impact factor: 5.742
Authors: Robert F Tamburro; Raymond C Barfield; Michele L Shaffer; Surender Rajasekaran; Paul Woodard; R Ray Morrison; Scott C Howard; Richard T Fiser; Jeffrey E Schmidt; Elaine M Sillos Journal: Pediatr Crit Care Med Date: 2008-05 Impact factor: 3.624
Authors: A Fischer; P Landais; W Friedrich; B Gerritsen; A Fasth; F Porta; A Vellodi; M Benkerrou; J P Jais; M Cavazzana-Calvo Journal: Blood Date: 1994-02-15 Impact factor: 22.113
Authors: George S Amatuni; Robert J Currier; Joseph A Church; Tracey Bishop; Elena Grimbacher; Alan Anh-Chuong Nguyen; Rajni Agarwal-Hashmi; Constantino P Aznar; Manish J Butte; Morton J Cowan; Morna J Dorsey; Christopher C Dvorak; Neena Kapoor; Donald B Kohn; M Louise Markert; Theodore B Moore; Stanley J Naides; Stanley Sciortino; Lisa Feuchtbaum; Rasoul A Koupaei; Jennifer M Puck Journal: Pediatrics Date: 2019-02 Impact factor: 7.124
Authors: Mohamed Bikhet; Mahmoud Morsi; Hidetaka Hara; Leslie A Rhodes; Waldemar F Carlo; David Cleveland; David K C Cooper; Hayato Iwase Journal: Pediatr Transplant Date: 2020-08-26