Results 91 to 100 of about 1,381,406 (265)

Systematic Cardiac Phenotyping of Patients With Copy Number Variants in the 15q11.2 Breakpoint 1 to Breakpoint 2 Region: A Retrospective Cohort Study From Nine Pediatric Cardiac Centers

American Journal of Medical Genetics Part A, EarlyView.
ABSTRACT Microdeletions impacting 15q11.2 breakpoint (BP) 1 to BP2, adjacent to the Prader–Willi critical region, have previously described neuropsychiatric associations, with potential low penetrance presentations of congenital heart disease (CHD) also identified.
Morgan B. Wright, Maria B. Padua, Lindsey R. Helvaty, Kim L. McBride, Gabrielle Geddes, Vidu Garg, Seema R. Lalani, Stephanie Burns Wechsler, Jennelle C. Hodge, Benjamin J. Landis, Stephanie M. Ware   +10 more
wiley   +1 more source

The Swiss registry for pulmonary arterial hypertension: the paediatric experience.

, 2007
Pulmonary arterial hypertension is a rare disease with a poor prognosis. Epidemiological data are scarce, particularly in the paediatric population. A registry was recently developed in order to collect epidemiological data on patients with pulmonary ...
Tolsa, J.F., Fasnacht, M.S., Beghetti, M.   +2 more
core   +1 more source

Repeated instillations of bleomycin in male mice induce a perivascular remodeling at distance of fibrotic areas in the context of progressive pulmonary fibrosis

Animal Models and Experimental Medicine, EarlyView.
We established a model of progressive pulmonary fibrosis via repeated intratracheal instillation (ITI) of leomycin (BLM) (A). Perivascular remodeling was observed in areas distant from the fibrotic areas and lesions induced by repeated ITI of BLM simulate those observed in IPF patients (B). Thus, this model has the capacity to track and investigate the
Céline‐Hivda Yegen, Zakaria Maakoul, Dominique Marchant, Patrice Callard, Julia Bourse, Jean‐François Bernaudin, Hilario Nunes, Carole Planès, Emilie Boncoeur, Nicolas Voituron   +9 more
wiley   +1 more source

Diaphragm weakness in pulmonary arterial hypertension: role of sarcomeric dysfunction

, 2012
Item does not contain fulltextWe previously demonstrated that diaphragm muscle weakness is present in experimental pulmonary arterial hypertension (PH). However, the nature of this diaphragm weakness is still unknown. Therefore, the aim of this study was
Stienen, G.J.M., van Hees, H.W.H., Vonk-Noordegraaf, A., Hees, H.W.H. van, Vonk Noordegraaf, A., Manders, E., de Man, F.S., Ottenheijm, C.A.C., Handoko, M.L., Westerhof, N., Stienen, G.J.   +10 more
core   +1 more source

Genome‐wide network analysis identifies the lncRNA‐92467/miR‐205‐5p/PTPRM/CAMs axis in a rat model of hypoxic pulmonary hypertension

Animal Models and Experimental Medicine, EarlyView.
The pathogenesis of hypoxic pulmonary hypertension (HPH) remains unclear. In this study, we explored its key regulatory mechanisms using animal models, RNA sequencing, and cellular assays. We found that lncRNA‐92467 functions as a ceRNA, binding miR‐205‐5p, and thereby upregulating PTPRM, inhibiting abnormal proliferation and migration of endothelial ...
Yan‐Ying Shen, Yi‐Bing Chen, Xin Li, Xiang Xu, Ge Guo, Han‐Lu Li, Yong‐Ming Yao, Chun‐Lei Liu   +7 more
wiley   +1 more source

Transcriptional profiling of lung cell populations in idiopathic pulmonary arterial hypertension

Pulmonary Circulation, 2020
Despite recent improvements in management of idiopathic pulmonary arterial hypertension, mortality remains high. Understanding the alterations in the transcriptome–phenotype of the key lung cells involved could provide insight into the drivers of ...
Didem Saygin, Tracy Tabib, Humberto E.T. Bittar, Eleanor Valenzi, John Sembrat, Stephen Y. Chan, Mauricio Rojas, Robert Lafyatis   +7 more
doaj   +1 more source

Acute necrotising pulmonary vasculitis and pulmonary hypertension in a juvenile dog [PDF]

, 2008
A five-month-old female Jack Russell terrier was presented for investigation of acute lethargy, anorexia, coughing, respiratory distress and weakness. Examination findings included cyanosis, a grade 3 of 6 systolic heart murmur and prolonged capillary ...
Russell, N.J., Nicholls, P.K., Olivry, T., Irwin, P.J., Hopper, B.J.   +4 more
core  

New models of pulmonary hypertension based on VEGF receptor blockade-induced endothelial cell apoptosis

, 2012
In spite of treatment, severe angioproliferative pulmonary arterial hypertension (PAH) remains a disease characterized by great morbidity and shortened survival.
Drake, Jennnifer I., Gomez-Arroyo, Jose G., Al Husseini, Aysar, Bogaard, Herman Jan, Mizuno, Shiro, Nicolls, Mark R., Taraseviciene-Stewart, Laima, Voelkel, Norbert F., Farkas, Laszlo   +8 more
core   +1 more source

Aortic valve disease: Novel animal models for advancing our understanding of the underlying mechanisms

Animal Models and Experimental Medicine, EarlyView.
Due to the complex pathophysiology of aortic valve disease (AVD) and the resulting lack of effective pharmacotherapy, existing animal models are valuable research tools, but each has its own strengths and limitations. This review systematically surveys the major AVD models, compares their induction methods, pathological features, advantages and ...
Liya Hou, Xue Liu, Wangxing Hu, Xianbao Liu, Wei Zhu   +4 more
wiley   +1 more source

Gut microbiota modification suppresses the development of pulmonary arterial hypertension in an SU5416/hypoxia rat model

, 2020
The pathogenesis of pulmonary arterial hypertension is closely associated with dysregulated inflammation. Recently, abnormal alterations in gut microbiome composition and function were reported in a pulmonary arterial hypertension experimental animal ...
Sakao, Seiichiro, Sanada, Takayuki J., Hosomi, Koji, Miwa, Hideki, Kobayashi, Takayuki, Suda, Rika, Yanagisawa, Asako, Tanabe, Nobuhiro, Shoji, Hiroki, Naito, Akira, Park, Jonguk, Tatsumi, Koichiro, Ikubo, Yumiko, Kunisawa, Jun, Mizuguchi, Kenji   +14 more
core   +1 more source