Primary pulmonary hypertension: an overview of epidemiology and pathogenesis.

Ghamra, Ziad; Dweik, Raed A.

doi:10.3949/ccjm.70.suppl_1.s2

Cited by 48 publications

(32 citation statements)

References 15 publications

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“…It is an interesting finding that although drug use is common in the elderly (25,26), we found a lower OLDU rate for this age group in our study. On the other hand, the overbalance of offlabel use in young adults and children and the female predominance in the applications could be associated with the nature of the most common diagnoses found in this study, such as SLE, MS, and other PHD (27)(28)(29). UHs are tertiary healthcare centers that have the healthcare service capacity to treat more serious illnesses and embody specific subspecialties.…”

Section: Discussionmentioning

confidence: 99%

A nationwide evaluation of off-label drug utilization in Turkey

Özdamar¹,

Akıcı²,

Alkan³

et al. 2017

Turk J Med Sci

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Background/aim: Off-label drug use (OLDU) is under the control of the Turkish Medicines and Medical Devices Agency (TMMDA) in Turkey. It was aimed to investigate demographic and medical features of patients with OLDU applications in Turkey. Materials and methods:A total of 4426 electronic OLDU application records of the TMMDA were evaluated retrospectively. Information regarding patients' demographic characteristics, diagnoses, requested drugs, institutions, and specialties of the physicians were evaluated.Results: OLDU applications were mostly made by rheumatologists (21.5%) and 95.2% of them were approved by the TMMDA. The mean age of the patients was 35 years and 54.4% of them were female. Off-label drugs were mostly prescribed for patients aged 18-64 years (62.1%) and were most frequently prescribed by physicians from university medical centers (81.0%). Systemic lupus erythematosus (10.1%) was the most common diagnosis. Mycophenolate (16.1%) and rituximab (10.1%) were the most frequently prescribed off-label drugs. There were differences regarding some characteristics of patients and their physicians among most frequently prescribed off-label drugs (P < 0.05). Conclusion:It is noteworthy that OLDU applications showed demographical and institutional differences. It is expected that this study will provide important contributions to physicians working in the relevant area with respect to treatment alternatives of diseases with treatment challenges.

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Section: Discussionmentioning

confidence: 99%

A nationwide evaluation of off-label drug utilization in Turkey

Özdamar¹,

Akıcı²,

Alkan³

et al. 2017

Turk J Med Sci

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“…Recent investigations between endothelial cells and SMCs revealed that endothelial dysfunction contributes to vascular remodeling through the proliferative effects of some vasoconstrictors (14)(15). In addition, the loss of vasodilators can promote cell proliferation (14).…”

Section: Endothelial Dysfunction and Pahmentioning

confidence: 99%

Molecular Mechanisms of Pulmonary Arterial Remodeling

Crosswhite

Sun

2014

Mol Med

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Pulmonary arterial hypertension (PAH) is characterized by a persistent elevation of pulmonary arterial pressure and pulmonary arterial remodeling with unknown etiology. Current therapeutics available for PAH are primarily directed at reducing the pulmonary blood pressure through their effects on the endothelium. It is well accepted that pulmonary arterial remodeling is primarily due to excessive pulmonary arterial smooth muscle cell (PASMC) proliferation that leads to narrowing or occlusion of the pulmonary vessels. Future effective therapeutics will be successful in reversing the vascular remodeling in the pulmonary arteries and arterioles. The purpose of this review is to provide updated information on molecular mechanisms involved in pulmonary arterial remodeling with a focus on growth factors, transcription factors, and epigenetic pathways in PASMC proliferation. In addition, this review will highlight novel therapeutic strategies for potentially reversing PASMC proliferation.

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“…PAH is characterized by high pulmonary artery pressure and increased pulmonary vascular resistance, which often result in right ventricular (RV) failure and premature death. [7][8][9][10] The disease etiology is complicated and can be associated with other pulmonary, cardiac, or systemic diseases or other factors, including drug use and toxin stimuli. The idiopathic form of PAH (IPAH) has no identifiable etiology, affects mostly young women, and if treated has an average survival of 2-3 years from the time of diagnosis.…”

Section: Introductionmentioning

confidence: 99%

Novel Methods in Pulmonary Hypertension Phenotyping in the Age of Precision Medicine (2015 Grover Conference Series)

et al. 2016

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Among pulmonary vascular diseases, pulmonary hypertension (PH) is the best studied and has been the focus of our work. The current classification of PH is based on a relatively simple combination of patient characteristics and hemodynamics. This leads to inherent limitations, including the inability to customize treatment and the lack of clarity from a more granular identification based on individual patient phenotypes. Accurate phenotyping of PH can be used in the clinic to select therapies and determine prognosis and in research to increase the homogeneity of study cohorts. Rapid advances in the mechanistic understanding of the disease, improved imaging methods, and innovative biomarkers now provide an opportunity to define novel PH phenotypes. We have recently shown that altered metabolism may affect nitric oxide levels and protein glycosylation, the peripheral circulation (which may provide insights into the response to therapy), and exhaled-breath analysis (which may be useful in disease evaluation). This review is based on a talk presented during the 2015 Grover Conference and highlights the relevant literature describing novel methods to phenotype pulmonary arterial hypertension patients by using approaches that involve the pulmonary and systemic (peripheral) vasculature. In particular, abnormalities in metabolism, the pulmonary and peripheral circulation, and exhaled breath in PH may help identify phenotypes that can be the basis for a precision-medicine approach to PH management. These approaches may also have a broader scope and may contribute to a better understanding of other diseases, such as asthma, diabetes, and cancer.

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Primary pulmonary hypertension: an overview of epidemiology and pathogenesis.

Cited by 48 publications

References 15 publications

A nationwide evaluation of off-label drug utilization in Turkey

A nationwide evaluation of off-label drug utilization in Turkey

Molecular Mechanisms of Pulmonary Arterial Remodeling

Novel Methods in Pulmonary Hypertension Phenotyping in the Age of Precision Medicine (2015 Grover Conference Series)

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