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Acute lung injury (ALI) and its more severe form, acute respiratory distress syndrome (ARDS), are devastating disorders of overwhelming pulmonary inflammation and hypoxemia, resulting in high morbidity and mortality.To provide the clinician with a summary of the literature on the epidemiology, diagnosis, and an evidence-base for management of ALI/ARDS in children.PubMed search for clinical trials, selected literature review of other relevant studies on epidemiology and diagnosis.Lower mortality combined with a relatively lower frequency of ALI/ARDS in children makes performance of clinical trials challenging. Based on expert opinion, the following are recommended: 1) avoid tidal volumes ≥10 mL/kg body weight; 2) keep plateau pressure ≤30 cm H2O, arterial pH at 7.30 to 7.45, and Pao2 60 to 80 torr (8 to 10.7 kPa) (Spo2 ≥90%); 3) provide sedation, analgesia, and stress ulcer prophylaxis; and 4) use a 10 g/dL hemoglobin threshold for packed red blood cell transfusion in unstable patients (shock or profound hypoxia). Evidence supports dropping the hemoglobin transfusion threshold to 7 g/dL once profound hypoxia and shock have resolved. Promising therapies for pediatric ALI/ARDS based on pediatric studies include endotracheal surfactant, high-frequency oscillatory ventilation, noninvasive ventilation, and use of extracorporeal membrane oxygenation as a rescue therapy. Promising therapies based on adult trials include use of corticosteroids for lung inflammation and fibrosis, use of 4 to 6 mL/kg tidal volumes and restrictive fluid management. Prone positioning, bronchodilators, inhaled nitric oxide, tight glucose control, and high-flow nasal cannula (HFNC) oxygen are therapies that require further study before they can be recommended for children with ALI/ARDS.