Asthma Allergy Immunology

Asthma Allergy Immunology

2009, Vol 7, Num, 3     (Pages: 180-188)

Fatal acute respiratory distress syndrome in children with combined immunodeficiencies

Tanıl KENDİRLİ 1, Figen DOĞU 2, Aydan İKİNCİOĞULLARI 2, Caner AYTEKİN 2, Alişan YILDIRAN 2, Mutlu YÜKSEK 2, Erdal İNCE 3,

1 Division of Pediatric Intensive Care Unit, Department of Pediatrics, Faculty of Medicine, Ankara University, Ankara, Turkey
2 Division of Pediatric Immunology and Allergy, Department of Pediatrics, Faculty of Medicine, Ankara University, Ankara, Turkey
3 Division of Pediatric Infectious Diseases, Department of Pediatrics, Faculty of Medicine, Ankara University, Ankara, Turkey

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Acute respiratory distress syndrome (ARDS) is an acute form of severe alveolar-capillary disease that evolves after a direct or indirect lung injury. It is characterized by impaired oxygenation (PaO2/FiO2 < 200), bilateral pulmonary densities detectable by radiography and a pulmonary wedge occlusion pressure of less than 18 mmHg. Mortality remains high among children with ARDS, particularly in the presence of serious underlying conditions such as sepsis and multiple organ dysfunction syndrome. Here, we report five children with ARDS who were also diagnosed as either severe or combined immunodeficiency. Surfactant was given to four patients and mechanical ventilation was performed in all children. Although one responded temporarily, all patients died due to ARDS and multiple organ dysfunction syndrome. In conclusion, if pneumonia and ARDS develop in a patient with combined immunodeficiency, it usually ends with multiple organ dysfunction syndrome leading to mortality.

Keywords : Respiratory distress syndrome, immunodeficiency syndrome, cytomegalovirus, child, therapy