McDonald J W, Roggli V L, Bradford W D
Department of Pathology, Duke University Medical Center, Durham, North Carolina 27710.
Pediatr Pathol. 1994 May-Jun;14(3):505-11. doi: 10.3109/15513819409024280.
We report a unique case of coexisting exogenous lipoid pneumonia, endogenous lipoid pneumonia (ELP), and pulmonary alveolar proteinosis (PAP) in a 5-year-old patient with severe neurodevelopmental disease. The patient presented with gastroesophageal reflux and presumed chronic lung disease resulting from recurrent aspiration pneumonias and succumbed to respiratory failure. The autopsy showed lipid-laden macrophages and periodic acid-Schiff-positive granular material in alveolar spaces and multilamellated structures within both alveolar macrophages and extracellular debris. These findings were similar to those in previous reports of coexisting ELP and PAP in the setting of gastroesophageal reflux. However, the present case differed by the presence of scattered large osmiophilic extracellular lipid vacuoles. Besides strengthening the association between ELP and PAP and their relationship to gastroesophageal reflux, this case suggests that they may arise together with exogenous lipoid pneumonia, through related mechanisms, in the setting of neurodevelopmental disease.
我们报告了一例独特的病例，一名患有严重神经发育疾病的5岁患者同时存在外源性类脂性肺炎、内源性类脂性肺炎（ELP）和肺泡蛋白沉积症（PAP）。该患者因胃食管反流以及反复吸入性肺炎导致的慢性肺病而就诊，最终死于呼吸衰竭。尸检显示肺泡腔内有充满脂质的巨噬细胞和过碘酸-希夫染色阳性的颗粒物质，肺泡巨噬细胞和细胞外碎片内有多层结构。这些发现与之前关于在胃食管反流情况下ELP和PAP并存的报道相似。然而，本病例的不同之处在于存在散在的大嗜锇性细胞外脂质空泡。除了加强ELP和PAP之间的关联以及它们与胃食管反流的关系外，本病例表明，在神经发育疾病背景下，它们可能通过相关机制与外源性类脂性肺炎一起出现。
