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Pulmonary bulla

MedGen UID:
537120
Concept ID:
C0241982
Disease or Syndrome
Synonym: Bullae
SNOMED CT: Bulla of lung (278976004)
 
HPO: HP:0032446

Definition

Pulmonary bullae are rounded focal regions of emphysema with a thin wall which measure more than 1 cm in diameter. They are often subpleural in location and are typically larger in the apices. In some cases, bullae can be very large and result in compression of adjacent lung tissue. A giant bulla is arbitrarily defined as one that occupies at least one third of the volume of a hemithorax. When large, bullae can simulate pneumothorax. The most common cause is paraseptal emphysema but bullae may also be seen in association with centrilobular emphysema. [from HPO]

Term Hierarchy

Conditions with this feature

Ehlers-Danlos syndrome, type 4
MedGen UID:
82790
Concept ID:
C0268338
Disease or Syndrome
Vascular Ehlers-Danlos syndrome (vEDS) is characterized by arterial, intestinal, and/or uterine fragility; thin, translucent skin; easy bruising; characteristic facial appearance (thin vermilion of the lips, micrognathia, narrow nose, prominent eyes); and an aged appearance to the extremities, particularly the hands. Vascular dissection or rupture, gastrointestinal perforation, or organ rupture are the presenting signs in most adults with vEDS. Arterial rupture may be preceded by aneurysm, arteriovenous fistulae, or dissection but also may occur spontaneously. The majority (60%) of individuals with vEDS who are diagnosed before age 18 years are identified because of a positive family history. Neonates may present with clubfoot, hip dislocation, limb deficiency, and/or amniotic bands. Approximately half of children tested for vEDS in the absence of a positive family history present with a major complication at an average age of 11 years. Four minor diagnostic features – distal joint hypermobility, easy bruising, thin skin, and clubfeet – are most often present in those children ascertained without a major complication.
Immunodeficiency 89 and autoimmunity
MedGen UID:
1794237
Concept ID:
C5562027
Disease or Syndrome
Immunodeficiency-89 and autoimmunity (IMD89) is an autosomal recessive immune disorder characterized by adult onset of recurrent infections, allergies, microcytic anemia, and Crohn disease (see 266600) (Yang et al., 2020).

Professional guidelines

PubMed

Chávez KV, Pimienta-Ibarra AS, Máruquez-González H, Flores-Garcés JC, Peña-Mirabal ES, Bolaños-Morales FV
Gen Thorac Cardiovasc Surg 2022 Jun;70(6):566-574. Epub 2022 Jan 18 doi: 10.1007/s11748-022-01771-0. PMID: 35041128Free PMC Article
Plojoux J, Froudarakis M, Janssens JP, Soccal PM, Tschopp JM
Clin Respir J 2019 Apr;13(4):195-201. Epub 2019 Mar 18 doi: 10.1111/crj.12990. PMID: 30615303
Tschopp JM, Bintcliffe O, Astoul P, Canalis E, Driesen P, Janssen J, Krasnik M, Maskell N, Van Schil P, Tonia T, Waller DA, Marquette CH, Cardillo G
Eur Respir J 2015 Aug;46(2):321-35. Epub 2015 Jun 25 doi: 10.1183/09031936.00219214. PMID: 26113675

Recent clinical studies

Etiology

Mei LY, Feng YG, Tao SL, Jiang B, Dai FQ, Zhou JH, Shen C, Guo W, Tan QY, Deng B
J Cardiothorac Surg 2022 Dec 12;17(1):306. doi: 10.1186/s13019-022-02064-6. PMID: 36510224Free PMC Article
Li C, Liu S, Sui X, Wang J, Song W, Xu W, Xu KF, Tian X, Zhang W
PLoS One 2018;13(12):e0206858. Epub 2018 Dec 5 doi: 10.1371/journal.pone.0206858. PMID: 30517110Free PMC Article
Zhou Q, Xiao W, An E, Zhou H, Yan M
Eur J Anaesthesiol 2012 May;29(5):223-8. doi: 10.1097/EJA.0b013e32834f23a3. PMID: 22228239
Kaneda M, Tarukawa T, Watanabe F, Adachi K, Sakai T, Nakabayashi H
Interact Cardiovasc Thorac Surg 2010 Jun;10(6):940-4. Epub 2010 Mar 18 doi: 10.1510/icvts.2010.233551. PMID: 20299444
Ogawa D, Shiota Y, Marukawa M, Hiyama J, Mashiba H, Yunoki K, Imai S, Motohiro K, Sasaki N, Taniyama K
Respiration 1999 Nov-Dec;66(6):555-8. doi: 10.1159/000029436. PMID: 10575345

Diagnosis

Ye Y, Zhan Y
Am J Emerg Med 2024 Sep;83:162.e1-162.e3. Epub 2024 Jul 9 doi: 10.1016/j.ajem.2024.07.008. PMID: 39004543
Ma DJ, Liu HS, Li SQ, Zhou XY, Cui YS, Wu HW, Zhou WX
Medicine (Baltimore) 2017 Sep;96(35):e7733. doi: 10.1097/MD.0000000000007733. PMID: 28858088Free PMC Article
Byrd RP Jr, Roy TM
Tenn Med 2013 Jan;106(1):39-42. PMID: 23477242
Ng CS, Sihoe AD, Wan S, Lee TW, Arifi AA, Yim AP
Can Respir J 2001 Sep-Oct;8(5):369-71. doi: 10.1155/2001/927067. PMID: 11694917
Ogawa D, Shiota Y, Marukawa M, Hiyama J, Mashiba H, Yunoki K, Imai S, Motohiro K, Sasaki N, Taniyama K
Respiration 1999 Nov-Dec;66(6):555-8. doi: 10.1159/000029436. PMID: 10575345

Therapy

Li C, Liu S, Sui X, Wang J, Song W, Xu W, Xu KF, Tian X, Zhang W
PLoS One 2018;13(12):e0206858. Epub 2018 Dec 5 doi: 10.1371/journal.pone.0206858. PMID: 30517110Free PMC Article
Li WL, Li YH, Yang YB, Lv LH
Can Respir J 2018;2018:5806834. Epub 2018 Oct 23 doi: 10.1155/2018/5806834. PMID: 30425754Free PMC Article
Koratala A, Bhatti V
BMJ Case Rep 2017 May 4;2017 doi: 10.1136/bcr-2017-220058. PMID: 28473433Free PMC Article
Byrd RP Jr, Roy TM
Tenn Med 2013 Jan;106(1):39-42. PMID: 23477242
Zhou Q, Xiao W, An E, Zhou H, Yan M
Eur J Anaesthesiol 2012 May;29(5):223-8. doi: 10.1097/EJA.0b013e32834f23a3. PMID: 22228239

Prognosis

Li C, Liu S, Sui X, Wang J, Song W, Xu W, Xu KF, Tian X, Zhang W
PLoS One 2018;13(12):e0206858. Epub 2018 Dec 5 doi: 10.1371/journal.pone.0206858. PMID: 30517110Free PMC Article
Nagorni-Obradović L, Pešut D, Marić D, Stević R
Vojnosanit Pregl 2016 Dec;73(12):1160-3. doi: 10.2298/VSP150422077N. PMID: 29341574
Kaneda M, Tarukawa T, Watanabe F, Adachi K, Sakai T, Nakabayashi H
Interact Cardiovasc Thorac Surg 2010 Jun;10(6):940-4. Epub 2010 Mar 18 doi: 10.1510/icvts.2010.233551. PMID: 20299444
Ogawa D, Shiota Y, Marukawa M, Hiyama J, Mashiba H, Yunoki K, Imai S, Motohiro K, Sasaki N, Taniyama K
Respiration 1999 Nov-Dec;66(6):555-8. doi: 10.1159/000029436. PMID: 10575345
Kiyooka K, Maniwa Y, Okada M
Ann Thorac Cardiovasc Surg 1999 Oct;5(5):293-9. PMID: 10550714

Clinical prediction guides

Li C, Liu S, Sui X, Wang J, Song W, Xu W, Xu KF, Tian X, Zhang W
PLoS One 2018;13(12):e0206858. Epub 2018 Dec 5 doi: 10.1371/journal.pone.0206858. PMID: 30517110Free PMC Article
Gökçe M, Saydam O, Altin R, Kart L
Tuberk Toraks 2009;57(4):435-8. PMID: 20037862
Bradshaw DA, Murray KM, Amundson DE
Thorax 1996 May;51(5):549-50. doi: 10.1136/thx.51.5.549. PMID: 8711689Free PMC Article

Recent systematic reviews

Ma DJ, Liu HS, Li SQ, Zhou XY, Cui YS, Wu HW, Zhou WX
Medicine (Baltimore) 2017 Sep;96(35):e7733. doi: 10.1097/MD.0000000000007733. PMID: 28858088Free PMC Article

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