Porzionato Andrea, Macchi Veronica, Da Broi Ugo, Giraudo Chiara, Miotto Diego, Rodriguez Daniele, Moreschi Carlo, De Caro Raffaele
Department of Molecular Medicine, University of Padova, Padova, Italy.
Pathol Int. 2015 Jan;65(1):27-32. doi: 10.1111/pin.12228. Epub 2014 Nov 18.
Diffuse pulmonary ossification (DPO) is a rare condition characterized by diffuse metaplastic bone formation in the lungs. Two patterns have been described: dendriform, with a coral-like network of bone spiculae along the alveolar septa; and nodular, with lobulated fragments in the alveolar spaces. Dendriform DPO is frequently associated with chronic pulmonary pathologies. We present here the first case of DPO associated with a long-lasting vegetative state. Micro-computed tomography (MicroCT) was applied to analyze the distribution of pulmonary ossification in volumes of lung samples. It showed a mean volume percentage of ossification of 0.79% and 3-D reconstructions permitted to reveal the branching pattern and internal cavities of some ossifications. The occurrence of DPO in a persistent vegetative state (PVS) may be favored by recurrent pulmonary infections, due to aspiration and immunological defects, and respiratory instability, due to brain damage and the fact of being bedridden. Fibrotic reactions probably represent a preliminary step in bone formation. Further studies could examine the incidence and clinical significance of DPO in subjects in PVS or patients who are bedridden for other reasons. MicroCT may facilitate analysis of more case histories, with greater sensitivity with respect to classic microscopic analyses.
弥漫性肺骨化（DPO）是一种罕见疾病，其特征为肺部出现弥漫性化生骨形成。已描述了两种模式：树状，沿肺泡间隔有珊瑚状骨小梁网络；以及结节状，肺泡腔内有分叶状碎片。树状DPO常与慢性肺部疾病相关。我们在此报告首例与长期植物状态相关的DPO病例。应用微计算机断层扫描（MicroCT）分析肺样本体积中肺骨化的分布。结果显示骨化的平均体积百分比为0.79%，三维重建能够揭示一些骨化的分支模式和内部腔隙。由于误吸和免疫缺陷导致的反复肺部感染，以及因脑损伤和卧床状态导致的呼吸不稳定，可能有利于持续性植物状态（PVS）中DPO的发生。纤维化反应可能是骨形成的初步步骤。进一步的研究可以考察PVS患者或因其他原因卧床的患者中DPO的发生率及临床意义。与传统显微镜分析相比，MicroCT可能有助于分析更多病例，且具有更高的敏感性。
