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Peripheral pulmonary vessel aplasia

MedGen UID:
376462
•Concept ID:
C1848877
•
Finding
HPO: HP:0005316

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPeripheral pulmonary vessel aplasia

Conditions with this feature

Tetraamelia syndrome 1
MedGen UID:
860705
•Concept ID:
C4012268
•
Disease or Syndrome
Tetraamelia syndrome-1 (TETAMS1) is characterized by complete limb agenesis without defects of scapulae or clavicles. Other features include bilateral cleft lip/palate, diaphragmatic defect with bilobar right lung, renal and adrenal agenesis, pelvic hypoplasia, and urogenital defects (Niemann et al., 2004). Genetic Heterogeneity of tetraamelia syndrome Tetraamelia syndrome-2 (TETAMS2; 618021) is caused by mutation in the RSPO2 gene (610575) on chromosome 8q23.
See: Condition Record
Tetraamelia syndrome 1

Professional guidelines

PubMed

Monitoring the microcirculation in the diagnosis and follow-up of systemic sclerosis patients: Focus on pulmonary and peripheral vascular manifestations.
Ruaro B, Nallino MG, Casabella A, Salton F, Confalonieri P, De Tanti A, Bruni C
Microcirculation 2020 Nov;27(8):e12647. Epub 2020 Jul 26 doi: 10.1111/micc.12647. PMID: 32603500
Hyperleukocytosis: emergency management.
Jain R, Bansal D, Marwaha RK
Indian J Pediatr 2013 Feb;80(2):144-8. Epub 2012 Nov 24 doi: 10.1007/s12098-012-0917-3. PMID: 23180411
Endothelin receptor blockade in the management of pulmonary arterial hypertension: selective and dual antagonism.
Trow TK, Taichman DB
Respir Med 2009 Jul;103(7):951-62. Epub 2009 Mar 21 doi: 10.1016/j.rmed.2009.02.016. PMID: 19304472

Recent clinical studies

Etiology

Peripheral Pulmonary Artery Stenosis Is Not a Contraindication to a Single-ventricle Pathway.
Al-Khaldi A, Alsahari A, Alotay A, Momenah T, Alfonso JJ
Ann Thorac Surg 2022 May;113(5):1563-1568. Epub 2021 Apr 1 doi: 10.1016/j.athoracsur.2021.03.059. PMID: 33811885
Mosaic Neurocutaneous Disorders and Their Causes.
Ruggieri M, Praticò AD
Semin Pediatr Neurol 2015 Dec;22(4):207-33. Epub 2015 Nov 12 doi: 10.1016/j.spen.2015.11.001. PMID: 26706010
Patent arterial duct.
Forsey JT, Elmasry OA, Martin RP
Orphanet J Rare Dis 2009 Jul 10;4:17. doi: 10.1186/1750-1172-4-17. PMID: 19591690Free PMC Article
Serotonin and the heart.
Frishman WH, Grewall P
Ann Med 2000 Apr;32(3):195-209. doi: 10.3109/07853890008998827. PMID: 10821327
Mechanisms of hypercoagulability.
Dahl OE
Thromb Haemost 1999 Aug;82(2):902-6. PMID: 10605801

Diagnosis

BMPR2 Mutation and Metabolic Reprogramming in Pulmonary Arterial Hypertension.
Cuthbertson I, Morrell NW, Caruso P
Circ Res 2023 Jan 6;132(1):109-126. Epub 2023 Jan 5 doi: 10.1161/CIRCRESAHA.122.321554. PMID: 36603064
Wide-ranging clinical spectrum of paradoxical embolism.
Jolobe O
Postgrad Med J 2022 Dec;98(1166):958-966. Epub 2021 May 18 doi: 10.1136/postgradmedj-2020-139691. PMID: 34006630
Basic thoracic ultrasound for the respiratory physician.
Jimborean G, Ianoşi ES, Nemeş RM, Toma TP
Pneumologia 2015 Jul-Sep;64(3):12-8. PMID: 26738366
PEComa: morphology and genetics of a complex tumor family.
Thway K, Fisher C
Ann Diagn Pathol 2015 Oct;19(5):359-68. Epub 2015 Jun 9 doi: 10.1016/j.anndiagpath.2015.06.003. PMID: 26144278
Patent arterial duct.
Forsey JT, Elmasry OA, Martin RP
Orphanet J Rare Dis 2009 Jul 10;4:17. doi: 10.1186/1750-1172-4-17. PMID: 19591690Free PMC Article

Therapy

Spectrum of Neurological Manifestations in Covid-19: A Review.
Garg RK
Neurol India 2020 May-Jun;68(3):560-572. doi: 10.4103/0028-3886.289000. PMID: 32643664
Vascular Behçet's syndrome: an update.
Emmi G, Bettiol A, Silvestri E, Di Scala G, Becatti M, Fiorillo C, Prisco D
Intern Emerg Med 2019 Aug;14(5):645-652. Epub 2018 Nov 29 doi: 10.1007/s11739-018-1991-y. PMID: 30499073
Systemic microvascular dysfunction in microvascular and vasospastic angina.
Ford TJ, Rocchiccioli P, Good R, McEntegart M, Eteiba H, Watkins S, Shaukat A, Lindsay M, Robertson K, Hood S, Yii E, Sidik N, Harvey A, Montezano AC, Beattie E, Haddow L, Oldroyd KG, Touyz RM, Berry C
Eur Heart J 2018 Dec 7;39(46):4086-4097. doi: 10.1093/eurheartj/ehy529. PMID: 30165438Free PMC Article
Surgical Techniques for Repair of Peripheral Pulmonary Artery Stenosis.
Mainwaring RD, Hanley FL
Semin Thorac Cardiovasc Surg 2016 Summer;28(2):418-424. Epub 2016 Jul 18 doi: 10.1053/j.semtcvs.2016.07.003. PMID: 28043454
Endothelin receptor blockade in the management of pulmonary arterial hypertension: selective and dual antagonism.
Trow TK, Taichman DB
Respir Med 2009 Jul;103(7):951-62. Epub 2009 Mar 21 doi: 10.1016/j.rmed.2009.02.016. PMID: 19304472

Prognosis

BMPR2 Mutation and Metabolic Reprogramming in Pulmonary Arterial Hypertension.
Cuthbertson I, Morrell NW, Caruso P
Circ Res 2023 Jan 6;132(1):109-126. Epub 2023 Jan 5 doi: 10.1161/CIRCRESAHA.122.321554. PMID: 36603064
Monitoring the microcirculation in the diagnosis and follow-up of systemic sclerosis patients: Focus on pulmonary and peripheral vascular manifestations.
Ruaro B, Nallino MG, Casabella A, Salton F, Confalonieri P, De Tanti A, Bruni C
Microcirculation 2020 Nov;27(8):e12647. Epub 2020 Jul 26 doi: 10.1111/micc.12647. PMID: 32603500
Basic thoracic ultrasound for the respiratory physician.
Jimborean G, Ianoşi ES, Nemeş RM, Toma TP
Pneumologia 2015 Jul-Sep;64(3):12-8. PMID: 26738366
Patent arterial duct.
Forsey JT, Elmasry OA, Martin RP
Orphanet J Rare Dis 2009 Jul 10;4:17. doi: 10.1186/1750-1172-4-17. PMID: 19591690Free PMC Article
Isolated peripheral pulmonary artery stenoses in the adult.
Kreutzer J, Landzberg MJ, Preminger TJ, Mandell VS, Treves ST, Reid LM, Lock JE
Circulation 1996 Apr 1;93(7):1417-23. doi: 10.1161/01.cir.93.7.1417. PMID: 8641032

Clinical prediction guides

BMPR2 Mutation and Metabolic Reprogramming in Pulmonary Arterial Hypertension.
Cuthbertson I, Morrell NW, Caruso P
Circ Res 2023 Jan 6;132(1):109-126. Epub 2023 Jan 5 doi: 10.1161/CIRCRESAHA.122.321554. PMID: 36603064
Hemodynamic and Clinical Implications of Impaired Pulmonary Vascular Reserve in the Fontan Circulation.
Egbe AC, Miranda WR, Anderson JH, Borlaug BA
J Am Coll Cardiol 2020 Dec 8;76(23):2755-2763. doi: 10.1016/j.jacc.2020.10.003. PMID: 33272370Free PMC Article
Spectrum of Neurological Manifestations in Covid-19: A Review.
Garg RK
Neurol India 2020 May-Jun;68(3):560-572. doi: 10.4103/0028-3886.289000. PMID: 32643664
Basic thoracic ultrasound for the respiratory physician.
Jimborean G, Ianoşi ES, Nemeş RM, Toma TP
Pneumologia 2015 Jul-Sep;64(3):12-8. PMID: 26738366
Rho kinase and hypertension.
Wirth A
Biochim Biophys Acta 2010 Dec;1802(12):1276-84. Epub 2010 May 9 doi: 10.1016/j.bbadis.2010.05.002. PMID: 20460153

Recent systematic reviews

Prognostic factors for overall survival of stage III non-small cell lung cancer patients on computed tomography: A systematic review and meta-analysis.
van Laar M, van Amsterdam WAC, van Lindert ASR, de Jong PA, Verhoeff JJC
Radiother Oncol 2020 Oct;151:152-175. Epub 2020 Jul 22 doi: 10.1016/j.radonc.2020.07.030. PMID: 32710990

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