MCID: UNL013
MIFTS: 21

Unilateral Absence of a Pulmonary Artery malady

Categories: Rare diseases

Aliases & Classifications for Unilateral Absence of a Pulmonary Artery

Aliases & Descriptions for Unilateral Absence of a Pulmonary Artery:

Name: Unilateral Absence of a Pulmonary Artery 50
Isolated Unilateral Absence of a Pulmonary Artery 50
Pulmonary Artery, Isolated Unilateral Absence of 50
Congenital Absence of the Pulmonary Artery 50
Isolated Uapa 50

Classifications:



Summaries for Unilateral Absence of a Pulmonary Artery

NIH Rare Diseases : 50 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 urogenital adysplasia and immune-mediated encephalomyelitis. An important gene associated with Unilateral Absence of a Pulmonary Artery is THBD (Thrombomodulin), and among its related pathways/superpathways are Formation of Fibrin Clot (Clotting Cascade) and Complement and coagulation cascades. Affiliated tissues include lung, heart and testes.

Related Diseases for Unilateral Absence of a Pulmonary Artery

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

Drugs & Therapeutics for Unilateral Absence of a Pulmonary Artery

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

Genetic Tests for Unilateral Absence of a Pulmonary Artery

Anatomical Context for Unilateral Absence of a Pulmonary Artery

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

39
Lung, Heart, Testes

Publications for Unilateral Absence of a Pulmonary Artery

Articles related to Unilateral Absence of a Pulmonary Artery:

(show all 21)
id Title Authors Year
1
Anesthesia for cesarean section in a patient with isolated unilateral absence of a pulmonary artery. ( 28017175 )
2017
2
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
3
Bosentan in pulmonary hypertension secondary to unilateral absence of a pulmonary artery. ( 25965593 )
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
Pregnancy with congenital unilateral absence of a pulmonary artery. ( 16752272 )
2006
10
Unilateral absence of a pulmonary artery in absent pulmonary valve syndrome: a case report and review of literature. ( 17095983 )
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

Expression for Unilateral Absence of a Pulmonary Artery

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

Pathways for Unilateral Absence of a Pulmonary Artery

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

id Super pathways Score Top Affiliating Genes
1
Show member pathways
11.16 THBD VWF
2 10.77 THBD VWF

GO Terms for Unilateral Absence of a Pulmonary Artery

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

id Name GO ID Score Top Affiliating Genes
1 blood coagulation GO:0007596 8.96 THBD VWF
2 hemostasis GO:0007599 8.62 THBD VWF

Sources for Unilateral Absence of a Pulmonary Artery

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60 QIAGEN
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70 UMLS via Orphanet
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