Fatal Acute Respiratory Distress Syndrome in Children with Combined Immunodeficiencies

Tanıl Kendirli
Figen Doğu
Aydan İkincioğulları
Caner Aytekin
Alişan Yıldıran
Mutlu Yüksek
Erdal İnce

Abstract

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
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