title: Acute hemorrhagic respiratory failure caused by Wegener's granulomatosis successfully treated by bronchoalveolar lavage with diluted surfactant.
abstract: Wegener's granulomatosis (WG) is an idiopathic inflammatory systemic disease that can occasionally cause an acute respiratory distress syndrome. We report on a 17-year-old girl with Wegener's granulomatosis and acute hemorrhagic respiratory failure successfully treated using bronchoalveolar lavage with diluted porcine surfactant (Curosurf; 4.8 mg/mL) followed by a low-dose bolus of surfactant. The cumulative dose of surfactant was 40 mg/kg BW. The lavage with diluted surfactant and the administration of the bolus were performed with a flexible bronchoscope. The patient was ventilated during the whole procedure, stayed hemodynamically stable and showed only a very short phase of desaturation. The PaO2/FiO2 ratio increased from 54.8 to 62.4 after one hour, to 106 after 17 hours and finally to 280 after four days. The patient was extubated five days after lavage treatment, and almost normal lung function was restored after eight weeks. Bronchoalveolar lavage with diluted surfactant by flexible bronchoscopy allows selective and direct drug administration and removes airway and alveolar debris. The technique reduces the amount of surfactant needed to overcome inhibition and thereby reduces therapy costs. We conclude that this early therapeutic intervention with surfactant might help to avoid an invasive rescue therapy such as extra corporeal membrane oxygenation, thus improving outcome in terms of faster recovery of lung function.
authors.affiliation: Division of Neonatology and Paediatric Intensive Care, University Hospital, University of Vienna, Vienna, Austria. Michael.Hermon@akh-wien.ac.at
authors.forename: Michael M
authors.initials: MM
authors.lastname: Hermon
authors.forename: Johann
authors.initials: J
authors.lastname: Golej
authors.forename: Wolfgang
authors.initials: W
authors.lastname: Emminger
authors.forename: Stefan
authors.initials: S
authors.lastname: Puig
authors.forename: Zsolt
authors.initials: Z
authors.lastname: Szepfalusi
authors.forename: Gerhard
authors.initials: G
authors.lastname: Trittenwein
mesh_terms: D000208:Acute Disease; D000293:Adolescent; D001688:Biological Products / Q000008:administration & dosage / Q000627:therapeutic use*; D001992:Bronchoalveolar Lavage Fluid; D005260:Female; D005500:Follow-Up Studies; D014890:Granulomatosis with Polyangiitis / Q000150:complications* / Q000175:diagnosis / Q000000981:diagnostic imaging; D006470:Hemorrhage / Q000150:complications / Q000209:etiology; D006801:Humans; D008171:Lung Diseases / Q000150:complications / Q000209:etiology; D010743:Phospholipids / Q000008:administration & dosage / Q000627:therapeutic use*; D011650:Pulmonary Alveoli; D011663:Pulmonary Surfactants / Q000008:administration & dosage / Q000627:therapeutic use*; D013902:Radiography, Thoracic; D012131:Respiratory Insufficiency / Q000000981:diagnostic imaging / Q000188:drug therapy* / Q000209:etiology*; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D001688:Biological Products; D010743:Phospholipids; D011663:Pulmonary Surfactants; C068291:poractant alfa