Abstract:
:To shed light on the efficacy of oxygen therapy in pulmonary arterial hypertension (PAH) when hypoxemia is not present, we report seven years of observational data for a female patient recorded between February 2012 and February 2019 when she was aged 7.8-14.8 years. The patient was born with an atrial septal defect (closed spontaneously at 2.3 years) and ventricular septal defect (surgically repaired at 8.3 years) and then diagnosed with PAH at 8.9 years . The patient was prescribed bosentan soon after diagnosis and for the next 4.8 years, during which a first phase of oxygen therapy (nocturnal) was trialed for 2.8 years. Mean pulmonary arterial pressure (mPAP) and systolic PAP (sPAP) remained stable and at mild levels when oxygen was administered, but then increased progressively to severe levels over two years without oxygen. This coincided with worsening right ventricular pathology during the later part of this period without oxygen. Re-initiation of more intensive oxygen therapy while the patient was still on bosentan and before pharmocotherapy was changed coincided with a large and rapid fall in sPAP, confirmed by right heart catheterization measurements of mPAP. During this entire observation period, the patient remained normoxemic. These observations challenge the notion that oxygen therapy should be restricted to patients with hypoxemia and strengthen calls for further study of oxygen therapy in PAH.
journal_name
Pulm Circjournal_title
Pulmonary circulationauthors
Green S,Stuart Ddoi
10.1177/2045894019845610subject
Has Abstractpub_date
2019-04-01 00:00:00pages
11772045894019845610issue
2eissn
2045-8932issn
2045-8940journal_volume
9pub_type
杂志文章abstract::Endothelial nitric oxide synthase (eNOS) plays a major role in maintaining pulmonary vascular homeostasis. Tetrahydrobiopterin (BH4), an essential cofactor that stabilizes the dimerization of eNOS and balances nitric oxide (NO) and superoxide production, may have therapeutic potential in pulmonary hypertension. In the...
journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1086/677361
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journal_title:Pulmonary circulation
pub_type: 杂志文章
doi:10.1086/686491
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journal_title:Pulmonary circulation
pub_type: 杂志文章
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pub_type: 杂志文章
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pub_type: 杂志文章
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pub_type: 杂志文章,评审
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doi:10.1177/2045894018773049
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journal_title:Pulmonary circulation
pub_type: 杂志文章,评审
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doi:10.4103/2045-8932.87309
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journal_title:Pulmonary circulation
pub_type:
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更新日期:2015-09-01 00:00:00
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pub_type:
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abstract::Pulmonary arterial hypertension (PAH) is a major complication of sickle cell disease (SCD). Low levels of apolipoprotein A1 (Apo-A1) have been implicated in the development of PAH in SCD. We speculate that lower levels of Apo-A1 are related to dysregulation of the ubiquitin-proteasome pathway (UPP). Of 36 recruited pa...
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pub_type: 杂志文章
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abstract:: ...
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abstract::[This corrects the article DOI: 10.1086/688060.]. ...
journal_title:Pulmonary circulation
pub_type: 已发布勘误
doi:10.1086/689215
更新日期:2016-12-01 00:00:00