title: Patients with primary immunodeficiencies in pediatric intensive care unit: outcomes and mortality-related risk factors.
abstract: OBJECTIVE
  The aims of this study were to review the frequency, characteristics, and the clinical course of primary immunodeficiency (PID) patients admitted to pediatric intensive care unit (PICU) and attempt to identify factors related with mortality that might predict a poor outcome.
  
  
  METHODS
  We performed a retrospective review of children with PID aged 1 month to 18 years and admitted to PICU from January 2002 to January 2012 in our tertiary teaching children's hospital.
  
  
  RESULTS
  There were a total of 51 patients accounting for 71 admissions to the PICU. The most common diagnosis was severe combined immunodeficiency. Respiratory problems were the leading cause for admission. A total of 20 patients received hematopoietic stem cell transplantation. Immune reconstitution was achieved in 9 (45 %) patients and eight of them did survive. In all 56 % of all admission episodes resulted in survival. Risk factors for mortality included requirement of mechanical ventilation (P < .001), number of organ system failure (P = .013), need for renal replacement therapy (P < .001), use of inotropes (P < .001), higher Pediatric Logistic Organ Dysfunction (PELOD) score (P = .005), and length of PICU stay (P < .001).
  
  
  CONCLUSIONS
  This is the first study regarding the outcome and mortality-related risk factors for PID patients requiring PICU admission. We suggest that PICU management is as important as early diagnosis and treatment for these patients. Prediction of those at risk for poorer outcome might be beneficial for accurate intensive care management and survival.
authors: Department of Pediatrics, Division of Pediatric Critical Care, Ankara University Faculty of Medicine, Ankara, Turkey, odek@ankara.edu.tr.
authors: Cağlar
authors: C
authors: Odek
authors: Tanil
authors: T
authors: Kendirli
authors: Figen
authors: F
authors: Doğu
authors: Ayhan
authors: A
authors: Yaman
authors: Göksel
authors: G
authors: Vatansever
authors: Funda
authors: F
authors: Cipe
authors: Sule
authors: S
authors: Haskoloğlu
authors: Can
authors: C
authors: Ateş
authors: Erdal
authors: E
authors: Ince
authors: Aydan
authors: A
authors: Ikincioğullari
mesh_terms: D000293:Adolescent; D002423:Cause of Death; D002648:Child; D002675:Child, Preschool; D005260:Female; D018380:Hematopoietic Stem Cell Transplantation; D017052:Hospital Mortality; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000175:diagnosis / Q000453:epidemiology* / Q000628:therapy; D007223:Infant; D007231:Infant, Newborn; D015278:Intensive Care Units, Pediatric; D008297:Male; D012189:Retrospective Studies; D012307:Risk Factors; D016896:Treatment Outcome