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Paraseptal emphysema

MedGen UID:
538541
Concept ID:
C0264394
Disease or Syndrome
Synonyms: Distal acinar emphysema; Peripheral lobular emphysema; Subpleural emphysema
SNOMED CT: Paraseptal emphysema (31898008); Peripheral lobular emphysema (31898008); Subpleural emphysema (31898008)
 
HPO: HP:0033649

Definition

Paraseptal emphysema is characterized by predominant involvement of the distal alveoli and their ducts and sacs. It is characteristically bounded by any pleural surface and the interlobular septa. This emphysema is characterized by subpleural and peribronchovascular regions of low attenuation separated by intact interlobular septa, sometimes associated with bullae. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVParaseptal emphysema

Conditions with this feature

Interstitial lung disease due to ABCA3 deficiency
MedGen UID:
410074
Concept ID:
C1970456
Disease or Syndrome
For a general phenotypic description and a discussion of genetic heterogeneity of pulmonary surfactant metabolism dysfunction, see SMDP1 (265120).

Professional guidelines

PubMed

Hochhegger B, Patel PP, Zanon M, Müller E, Ferreira Correa L, Verma N, Mohammed TL, Quinto Dos Reis Hochhegger D, Irion K, Marchiori E
Lung 2022 Dec;200(6):817-820. Epub 2022 Oct 22 doi: 10.1007/s00408-022-00576-5. PMID: 36271930
Slebos DJ, Ten Hacken NH, Hetzel M, Herth FJF, Shah PL
Respiration 2018;96(1):1-11. Epub 2018 Jul 10 doi: 10.1159/000490193. PMID: 29991060Free PMC Article

Recent clinical studies

Etiology

Wei Y, Yang L, Wang Q
BMC Pulm Med 2024 Jun 8;24(1):274. doi: 10.1186/s12890-024-03088-5. PMID: 38851701Free PMC Article
Marciano BE, Olivier KN, Folio LR, Zerbe CS, Hsu AP, Freeman AF, Filie AC, Spinner MA, Sanchez LA, Lovell JP, Parta M, Cuellar-Rodriguez JM, Hickstein DD, Holland SM
Chest 2021 Oct;160(4):1350-1359. Epub 2021 Jun 3 doi: 10.1016/j.chest.2021.05.046. PMID: 34089740Free PMC Article
González J, Henschke CI, Yankelevitz DF, Seijo LM, Reeves AP, Yip R, Xie Y, Chung M, Sánchez-Salcedo P, Alcaide AB, Campo A, Bertó J, Del Mar Ocón M, Pueyo J, Bastarrika G, de-Torres JP, Zulueta JJ
PLoS One 2019;14(7):e0219187. Epub 2019 Jul 25 doi: 10.1371/journal.pone.0219187. PMID: 31344121Free PMC Article
Janssen R, Piscaer I, Franssen FME, Wouters EFM
Expert Rev Respir Med 2019 Apr;13(4):381-397. Epub 2019 Feb 22 doi: 10.1080/17476348.2019.1580575. PMID: 30761929
Copeland CR, Nath H, Terry NLJ, Wilson CG, Kim YI, Lynch DA, Bodduluri S, Wells JM, Dransfield MT, Díaz AA, Washko GR, Foreman MG, Bhatt SP; Genetic Epidemiology of COPD (COPDGene) Investigators
Ann Am Thorac Soc 2018 Apr;15(4):479-484. doi: 10.1513/AnnalsATS.201709-713OC. PMID: 29298081Free PMC Article

Diagnosis

Chae KJ, Hwang HJ, Duarte Achcar R, Cooley JC, Humphries SM, Kligerman S, Lynch DA
Radiographics 2024 Jun;44(6):e230165. doi: 10.1148/rg.230165. PMID: 38752767
Marciano BE, Olivier KN, Folio LR, Zerbe CS, Hsu AP, Freeman AF, Filie AC, Spinner MA, Sanchez LA, Lovell JP, Parta M, Cuellar-Rodriguez JM, Hickstein DD, Holland SM
Chest 2021 Oct;160(4):1350-1359. Epub 2021 Jun 3 doi: 10.1016/j.chest.2021.05.046. PMID: 34089740Free PMC Article
Janssen R, Piscaer I, Franssen FME, Wouters EFM
Expert Rev Respir Med 2019 Apr;13(4):381-397. Epub 2019 Feb 22 doi: 10.1080/17476348.2019.1580575. PMID: 30761929
Raoof S, Bondalapati P, Vydyula R, Ryu JH, Gupta N, Raoof S, Galvin J, Rosen MJ, Lynch D, Travis W, Mehta S, Lazzaro R, Naidich D
Chest 2016 Oct;150(4):945-965. Epub 2016 May 13 doi: 10.1016/j.chest.2016.04.026. PMID: 27180915Free PMC Article
Edge J, Simon G, Reid L
Br J Dis Chest 1966 Jan;60(1):10-8. doi: 10.1016/s0007-0971(66)80016-5. PMID: 5920503

Therapy

Lee HJ, Jin KN, Lee HW, Lee JK, Park TY, Heo EY, Kim DK
Int J Chron Obstruct Pulmon Dis 2022;17:2301-2315. Epub 2022 Sep 18 doi: 10.2147/COPD.S372735. PMID: 36159655Free PMC Article
Janssen R, Piscaer I, Franssen FME, Wouters EFM
Expert Rev Respir Med 2019 Apr;13(4):381-397. Epub 2019 Feb 22 doi: 10.1080/17476348.2019.1580575. PMID: 30761929
Mouronte-Roibás C, Fernández-Villar A, Ruano-Raviña A, Ramos-Hernández C, Tilve-Gómez A, Rodríguez-Fernández P, Díaz ACC, Vázquez-Noguerol MG, Fernández-García S, Leiro-Fernández V
Int J Chron Obstruct Pulmon Dis 2018;13:3563-3570. Epub 2018 Oct 29 doi: 10.2147/COPD.S178109. PMID: 30464438Free PMC Article
Ruppert AM, Perrin J, Khalil A, Vieira T, Abou-Chedid D, Masmoudi H, Crequit P, Giol M, Cadranel J, Assouad J, Gounant V
Diagn Interv Imaging 2018 Jul-Aug;99(7-8):465-471. Epub 2018 Mar 20 doi: 10.1016/j.diii.2018.01.017. PMID: 29571700
Copeland CR, Nath H, Terry NLJ, Wilson CG, Kim YI, Lynch DA, Bodduluri S, Wells JM, Dransfield MT, Díaz AA, Washko GR, Foreman MG, Bhatt SP; Genetic Epidemiology of COPD (COPDGene) Investigators
Ann Am Thorac Soc 2018 Apr;15(4):479-484. doi: 10.1513/AnnalsATS.201709-713OC. PMID: 29298081Free PMC Article

Prognosis

Wei Y, Yang L, Wang Q
BMC Pulm Med 2024 Jun 8;24(1):274. doi: 10.1186/s12890-024-03088-5. PMID: 38851701Free PMC Article
Xie W, Jacobs C, Charbonnier JP, Slebos DJ, van Ginneken B
Sci Rep 2023 Aug 29;13(1):14147. doi: 10.1038/s41598-023-40116-6. PMID: 37644032Free PMC Article
Maetani T, Tanabe N, Shiraishi Y, Shimada T, Terada S, Shima H, Mochizuki F, Sakamoto R, Kaji S, Oguma T, Sato S, Iijima H, Masuda I, Hirai T
Respiration 2023;102(3):194-202. Epub 2023 Jan 23 doi: 10.1159/000529031. PMID: 36689922Free PMC Article
Klont F, Horvatovich P, Bowler RP, van Rikxoort E, Charbonnier JP, Kwiatkowski M, Lynch DA, Humphries S, Bischoff R, Ten Hacken NHT, Pouwels SD
Respir Res 2022 Jan 24;23(1):15. doi: 10.1186/s12931-022-01934-w. PMID: 35073932Free PMC Article
González J, Henschke CI, Yankelevitz DF, Seijo LM, Reeves AP, Yip R, Xie Y, Chung M, Sánchez-Salcedo P, Alcaide AB, Campo A, Bertó J, Del Mar Ocón M, Pueyo J, Bastarrika G, de-Torres JP, Zulueta JJ
PLoS One 2019;14(7):e0219187. Epub 2019 Jul 25 doi: 10.1371/journal.pone.0219187. PMID: 31344121Free PMC Article

Clinical prediction guides

Shiraishi Y, Tanabe N, Shimizu K, Oguma A, Shima H, Sakamoto R, Yamazaki H, Oguma T, Sato A, Suzuki M, Makita H, Muro S, Nishimura M, Sato S, Konno S, Hirai T
Chest 2023 Aug;164(2):327-338. Epub 2023 Jan 31 doi: 10.1016/j.chest.2023.01.034. PMID: 36736486
Maetani T, Tanabe N, Shiraishi Y, Shimada T, Terada S, Shima H, Mochizuki F, Sakamoto R, Kaji S, Oguma T, Sato S, Iijima H, Masuda I, Hirai T
Respiration 2023;102(3):194-202. Epub 2023 Jan 23 doi: 10.1159/000529031. PMID: 36689922Free PMC Article
Marciano BE, Olivier KN, Folio LR, Zerbe CS, Hsu AP, Freeman AF, Filie AC, Spinner MA, Sanchez LA, Lovell JP, Parta M, Cuellar-Rodriguez JM, Hickstein DD, Holland SM
Chest 2021 Oct;160(4):1350-1359. Epub 2021 Jun 3 doi: 10.1016/j.chest.2021.05.046. PMID: 34089740Free PMC Article
González J, Henschke CI, Yankelevitz DF, Seijo LM, Reeves AP, Yip R, Xie Y, Chung M, Sánchez-Salcedo P, Alcaide AB, Campo A, Bertó J, Del Mar Ocón M, Pueyo J, Bastarrika G, de-Torres JP, Zulueta JJ
PLoS One 2019;14(7):e0219187. Epub 2019 Jul 25 doi: 10.1371/journal.pone.0219187. PMID: 31344121Free PMC Article
Araki T, Nishino M, Zazueta OE, Gao W, Dupuis J, Okajima Y, Latourelle JC, Rosas IO, Murakami T, O'Connor GT, Washko GR, Hunninghake GM, Hatabu H
Eur J Radiol 2015 Jul;84(7):1413-8. Epub 2015 Mar 18 doi: 10.1016/j.ejrad.2015.03.010. PMID: 25868675Free PMC Article

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