Introduction Primary immunodeficiency disorders (PID) are characterized by poor or absent function in one or more components of the immune system. Early diagnosis and treatment is crucial for preventing morbidity and mortality. Despite adequate treatment, most of these patients require intensive care because of organ dysfunctions related to infections and HSCT complications. We reviewed our PID patients admitted to pediatric intensive care unit (PICU) over a 10-year period.
Patients and methods: PID patients, who were admitted to PICU between 1 January 2002 and 1 January 2012, were included. Data were collected from patient medical records.
Results A total of 51 patients (27 males) were admitted to PICU. There were a total of 71 admission episodes. The median age was 12 months. Age and sex were not significantly associated with mortality. The most common diagnosis was SCID. A total of 20 patients underwent HSCT. In all, 52 (73.2%) of all admission episodes were for respiratory problems, 10 (14%) for proven infections, 8 (11.4%) for neurological problems and 1 (1.4%) for surgical problems. Of the 71 episodes, 51 (71.8%) required mechanical ventilation, 11 (15.4%) required renal replacement therapy, 32 (45%) required inotropes. In all, 40/71 (56.3%) of the episodes resulted in survival. Requirement for ventilation, inotropes and renal replacement therapy were related with poor outcome. Multi-organ failure, PELOD score, duration of PICU admission were associated with mortality.
Conclusion Patients with PID had a high rate of PICU admission. Multi-organ failure was associated with mortality. Despite adequte support mortality rates were around 50%.