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Am. J. Respir. Crit. Care Med., Volume 165, Number 1, January 2002, 128-131

Successful Treatment of Endogenous Lipoid Pneumonia due to Niemann-Pick Type B Disease with Whole-Lung Lavage

ANDREW G. NICHOLSON, ATHOL U. WELLS, JAMES HOOPER, DAVID M. HANSELL, ANDREA KELLEHER, and CLIFF MORGAN

Departments of Histopathology, Respiratory Medicine, Biochemistry, Imaging, and Anaesthesia, Royal Brompton Hospital, London, United Kingdom

In Type B Niemann-Pick disease, progressive pulmonary infiltration is a major cause of morbidity and mortality, although the disease is usually diagnosed before adulthood in other organ systems. To date, no successful treatment of pulmonary involvement by Niemann-Pick disease has been documented. We describe the case of a patient with Niemann-Pick Type B disease who presented with extensive endogenous lipoid pneumonia and life-threatening hypoxia following bypass grafting for severe coronary artery disease. A surgical lung biopsy at the time of grafting revealed characteristic histology and ultrastructural features of Niemann-Pick disease, with confirmatory findings in biochemical studies. Because of the severity of the patient's symptoms, bilateral whole-lung lavage was undertaken, leading to symptomatic improvement, lessening of parenchymal opacification on high-resolution computed tomographic scanning, and a marked improvement in resting arterial oxygen tension while breathing air to 10.3 kPa from 8.4 kPa. Whole-lung lavage may be a potentially useful modality of treatment for patients with pulmonary involvement by Niemann-Pick Type B disease.




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