MCID: UNL013
MIFTS: 18

Unilateral Absence of a Pulmonary Artery malady

Category: Rare diseases

Aliases & Classifications for Unilateral Absence of a Pulmonary Artery

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Aliases & Descriptions for Unilateral Absence of a Pulmonary Artery:

Name: Unilateral Absence of a Pulmonary Artery 48
Isolated Unilateral Absence of a Pulmonary Artery 48
Pulmonary Artery, Isolated Unilateral Absence of 48
 
Congenital Absence of the Pulmonary Artery 48
Isolated Uapa 48

Classifications:



Summaries for Unilateral Absence of a Pulmonary Artery

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NIH Rare Diseases:48 Unilateral absence of the pulmonary artery (UAPA) is a heart defect that is present from birth. The pulmonary artery takes blood from the heart to the lungs. In the absence of a pulmonary artery, other blood vessels compensate by supplying blood to the lungs. Pressure can build inside these vessels and lead to heart and lung complications. UAPA can be left sided or right sided. It most often occurs alone, but can occur with other heart anomalies and birth defects. Signs and symptoms include repeat lung infections, lower exercise tolerance, shortness of breath, chest pain, and fluid in the lungs. Risk for these symptoms increase with age. Complications of UAPA include, hemoptysis (coughing up blood), respiratory hemorrhage, and high blood pressure in the arteries to the lung (pulmonary hypertension). Serious complications may be triggered by stress on the body, such as pregnancy and altitude sickness. Last updated: 2/26/2014

MalaCards based summary: Unilateral Absence of a Pulmonary Artery, also known as isolated unilateral absence of a pulmonary artery, is related to mullerian aplasia and macrocephaly mesodermal hamartoma spectrum. An important gene associated with Unilateral Absence of a Pulmonary Artery is THBD (Thrombomodulin), and among its related pathways are Formation of Fibrin Clot (Clotting Cascade) and Complement and coagulation cascades. Affiliated tissues include testes and lung.

Related Diseases for Unilateral Absence of a Pulmonary Artery

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Graphical network of the top 20 diseases related to Unilateral Absence of a Pulmonary Artery:



Diseases related to unilateral absence of a pulmonary artery

Symptoms & Phenotypes for Unilateral Absence of a Pulmonary Artery

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Drugs & Therapeutics for Unilateral Absence of a Pulmonary Artery

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Interventional clinical trials:

Search ClinicalTrials, NIH Clinical Center for Unilateral Absence of a Pulmonary Artery

Genetic Tests for Unilateral Absence of a Pulmonary Artery

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Anatomical Context for Unilateral Absence of a Pulmonary Artery

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MalaCards organs/tissues related to Unilateral Absence of a Pulmonary Artery:

36
Testes, Lung

Publications for Unilateral Absence of a Pulmonary Artery

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Articles related to Unilateral Absence of a Pulmonary Artery:

(show all 21)
idTitleAuthorsYear
1
Anesthesia for cesarean section in a patient with isolated unilateral absence of a pulmonary artery. (28017175)
2017
2
Bosentan in pulmonary hypertension secondary to unilateral absence of a pulmonary artery. (25965593)
2015
3
Selective Embolization of Systemic Collaterals for the Treatment of Recurrent Hemoptysis Secondary to the Unilateral Absence of a Pulmonary Artery in a Child. (25145922)
2015
4
A hidden cause of recurrent hemoptysis: unilateral absence of a pulmonary artery. (23786954)
2013
5
Unilateral absence of a pulmonary artery: a rare disorder with variable presentation. (22481838)
2012
6
A 36-year-old woman with a history of dextrocardia and dyspnea. Diagnosis: unilateral absence of a pulmonary artery (UAPA) with right hypoplastic lung. (19648732)
2010
7
Age-related clinical characteristics of isolated congenital unilateral absence of a pulmonary artery. (20814784)
2010
8
Case of the Month #134. Unilateral absence of a pulmonary artery. (18697722)
2008
9
Unilateral absence of a pulmonary artery in absent pulmonary valve syndrome: a case report and review of literature. (17095983)
2006
10
Pregnancy with congenital unilateral absence of a pulmonary artery. (16752272)
2006
11
Isolated unilateral absence of a pulmonary artery treated by pneumonectomy in an adult: report of a case. (16715422)
2006
12
Isolated unilateral absence of a pulmonary artery: a case report and review of the literature. (12377882)
2002
13
Isolated unilateral absence of a pulmonary artery: influence of systemic circulation on alveolar capillary vessels. (11564222)
2001
14
Unilateral absence of a pulmonary artery. (9880979)
1998
15
Unilateral absence of a pulmonary artery. Data from cardiopulmonary exercise testing. (8417905)
1993
16
Unilateral absence of a pulmonary artery: congenital disease or embolic occlusion? (1804679)
1991
17
Congenital unilateral absence of a pulmonary artery: a report of two adult cases. (2310105)
1990
18
Unilateral absence of a pulmonary artery. (3843667)
1985
19
Unilateral absence of a pulmonary artery. (6641308)
1983
20
Truncus arteriosus with unilateral absence of a pulmonary artery. Criteria for operability and surgical results. (837510)
1977
21
Surgical treatment of tetralogy of Fallot with unilateral absence of a pulmonary artery. (5079344)
1972

Variations for Unilateral Absence of a Pulmonary Artery

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Expression for genes affiliated with Unilateral Absence of a Pulmonary Artery

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Search GEO for disease gene expression data for Unilateral Absence of a Pulmonary Artery.

Pathways for genes affiliated with Unilateral Absence of a Pulmonary Artery

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GO Terms for genes affiliated with Unilateral Absence of a Pulmonary Artery

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Biological processes related to Unilateral Absence of a Pulmonary Artery according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1blood coagulationGO:00075969.1THBD, VWF

Sources for Unilateral Absence of a Pulmonary Artery

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2CDC
6CNVD
10DGIdb
15ExPASy
16FDA
17FMA
27GTR
28HGMD
29HMDB
30ICD10
31ICD10 via Orphanet
32ICD9CM
33IUPHAR
34KEGG
37MedGen
39MeSH
40MESH via Orphanet
41MGI
44NCI
45NCIt
46NDF-RT
49NINDS
50Novoseek
52OMIM
53OMIM via Orphanet
57PubMed
58QIAGEN
63SNOMED-CT via Orphanet
67Tumor Gene Family of Databases
68UMLS
69UMLS via Orphanet