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Pulmonary bleb

MedGen UID:
341774
Concept ID:
C1857434
Anatomical Abnormality
Synonym: Pulmonary blebs
 
HPO: HP:0032447

Definition

A bleb is a small gas-containing space within the visceral pleura or in the subpleural lung, not larger than 1 cm in diameter. CT findings show a bleb as a thin-walled cystic air space contiguous with the pleura. [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.

Professional guidelines

PubMed

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
Anderson PD
J Pharm Pract 2012 Feb;25(1):61-8. Epub 2011 Nov 11 doi: 10.1177/0897190011420677. PMID: 22080590
Yazkan R, Han S
Tuberk Toraks 2010;58(3):334-43. PMID: 21038147

Recent clinical studies

Diagnosis

Gelabert C, Nelson M
West J Emerg Med 2015 May;16(3):447-9. Epub 2015 Apr 9 doi: 10.5811/westjem.2015.3.24809. PMID: 25987927Free PMC Article
Toklu AS, Korpinar S, Erelel M, Uzun G, Yildiz S
Respir Med 2008 Aug;102(8):1145-7. Epub 2008 Jun 20 doi: 10.1016/j.rmed.2008.03.012. PMID: 18571913
Simon BC, Paolinetti L
J Emerg Med 2005 Aug;29(2):201-5. doi: 10.1016/j.jemermed.2005.04.009. PMID: 16029833

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