FIG 2 Lung histology of 9mo with ACD/MPV. (a) Representation of a hypertrophic arterial wall (Ar) in hematoxylin and eosin and (b) Verhoeff stained. (c) Representation of the misaligned pulmonary veins (V) adjacent to the lymphatic vessel (L) and (d) alveolar dysplasia of the lung parenchyma (Alv) in Verhoeff stained. (e) Hematoxylin-eosin illustration of the misaligned pulmonary veins (V) and (g) alveolar dysplasia (Alv) adjacent to the thickened pulmonary arteriole (Ar) in (f) Verhoeff stained. (g, h) Hematoxylin-eosin and immunostaining for CD31 (brown color) highlighting a reduced number of alveolar capillary endothelial cells located away from the inner side of the alveoli (arrows) in ACD/MPV (i) Hematoxylin and eosin illustration of the alveolar dysplasia at high magnification.(j) Transmission electron microscopy showing abnormal lamellar bodies (Lb) in alveolar epithelial cells of alveolar dysplasia. The 1.5 cm straight line corresponds to magnifications of (a,e) 500; (f,g) 200; (b,d,h) 100; (i) 50 and (j) x 15.000.
References
  1. Slot E., Edel G., Cutz E., van Heijst A., Post M., Schnater M., Wijnen R., Tibboel D., Rottier R., de Klein A. (2018). Alveolar capillary dysplasia with misalignment of the pulmonary veins: clinical, histological, and genetic aspects. Pulmonary circulation8 (3), 2045894018795143. https://doi.org/10.1177/2045894018795143
  2. Ito Y., Akimoto T., Cho K., Yamada M., Tanino M., Dobata T., Kitaichi M., Kumaki S., Kinugawa Y. (2015). A late presenter and long-term survivor of alveolar capillary dysplasia with misalignment of the pulmonary veins. European journal of pediatrics174 (8), 1123–1126. https://doi.org/10.1007/s00431-015-2543-3
  3. Petetta C., Tattoli L., Botta G., Di Vella G. (2020). Two autopsy cases of siblings with alveolar capillary dysplasia: clinical and post-mortem issues. Forensic science, medicine, and pathology16 (1), 180–183. https://doi.org/10.1007/s12024-019-00153-y
  4. Alturkustani M., Li D., Byers JT., Szymanski L., Parham, DM., Shi W., Wang LL. (2021). Histopathologic features of alveolar capillary dysplasia with misalignment of pulmonary veins with atypical clinical presentation. Cardiovascular pathology : the official journal of the Society for Cardiovascular Pathology50 , 107289. https://doi.org/10.1016/j.carpath.2020.107289
  5. Sen P., Yang Y., Navarro C., Silva I., Szafranski P., Kolodziejska K. E., Dharmadhikari AV., Mostafa H., Kozakewich H., Kearney D., et al (2013). Novel FOXF1 mutations in sporadic and familial cases of alveolar capillary dysplasia with misaligned pulmonary veins imply a role for its DNA binding domain. Human mutation34 (6), 801–811. https://doi.org/10.1002/humu.22313