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There were significant amounts of PAS positive proteinacous material. There were large amounts of neutral lipid and the lipid could be seen in the lining of the alveoli on oil and red staining. A fair amount of inflammation is evident in keeping with pneumonitis and interstitial disease. Cholesterol granulomas were seen. The conclusion was of PAP with some features of lipoid pneumonitis.

He was managed with methlyprednisone and seemed to improve in his respiratory status enough to be extubated. He did not tolerate extubation and developed a pneumothorax. Unfortunately, with no further therapies he died peacefully a week later.

Summary

He was a 2 and 1/2 year old boy with Lysinuric protein intolerance complicated by pulmonary alveolar proteinosis. His imaging showed the characteristic features of "crazy paving" though his bronchoalveolar lavage was less typical. He did not respond to whole lung lavage as might be expected with PAP and he had recurrent respiratory problems and fever. His lung biopsy confirmed PAP with the features of lipoid pneumonitis.

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