Faculty Bibliography

Aims & Objectives: High frequency percussive ventilation (HFPV) rapidly delivers subphysiologic tidal volumes using a volume-diffusive respirator and provides similar or improved oxygenation and ventilation at lower peak, mean, and end-expiratory pressures when compared with conventional ventilation (CV). We present the case of a child with acute respiratory distress syndrome (ARDS) from pulmonary hemorrhage treated with HFPV. Methods Case Report Results A ventilator-dependent 5-year-old male with pyruvate dehydrogenase deficiency presented with acute hypoxemic respiratory failure. Chest radiography showed bilateral pulmonary infiltrates and tracheal aspirate revealed hemosiderin laden macrophages. Methylprednisolone and empiric antibiotics were administered for pulmonary hemorrhage and severe ARDS. The patient was placed on high-frequency oscillatory ventilation (HFOV) then HFPV (Figure 1). Subsequent dislodgement of a blood clot was followed by improvement in OI and chest radiography (Figure 2). The patient was discharged on home ventilator settings on HD#11. (Figure prsented). Conclusions HFPV has been shown to decrease morbidity and mortality by improving mucociliary clearance. To our knowledge this is the first reported case of HFPV use in a pediatric patient with ARDS from pulmonary hemorrhage. Early implementation of HFPV may be a safe and effective ECMO-sparing strategy to improve oxygenation in patients with ARDS due to pulmonary hemorrhage

Aims & Objectives: Patients with Ehlers-Danlos syndrome (EDS) have abnormal collagen production or secretion leading to hyperextensibility of the skin, hypermobility of the joints, and increased tissue fragility. Although uncommon, respiratory manifestations of EDS have been described. The typical causes of death in patients with EDS are viscus rupture and arterial hemorrhage. Some deaths have been attributed to pulmonary hemorrhage. We report a case of acute pulmonary hemorrhage as the presenting finding of granulatomatosis with polyangiitis in a child with EDS. Methods Case Report Results A 14-year-old female with EDS presented with a 2-month history of fever, fatigue, unintentional weight loss, arthralgias, myalgias, and progressive dyspnea. Chest radiography revealed diffuse alveolar opacities and laboratory evaluation revealed anemia and thrombocytopenia. Chest computed tomography (CT) showed pulmonary hemorrhage. Anti-neutrophil cytoplasmic antibodies targeting proteinase 3 were positive for granulomatosis with polyangiitis. She was admitted on highflow nasal cannula oxygen therapy and given pulse-dose intravenous methylprednisolone. Subsequent improvement in respiratory status was observed; she was treated with rituximab and weaned off oxygen. Patient was discharged home in stable condition on hospital day #5 and remained on oral prednisone, with a 4-week course of rituximab, and was started oral azathioprine. (Figure prsented). Conclusions To our knowledge this is the first reported case of polyangiitis with granulomatosis presenting with pulmonary hemorrhage in a child with EDS. An increased index of suspicion may improve outcomes