Results 81 to 90 of about 29,066 (192)

Human iPSC‐Derived Vascularised Lung Organoids for Modelling COPD and Pulmonary Hypertension

Cell Proliferation, EarlyView.
Vascularised lung organoids (vLOs) that faithfully mimic human lung tissue architecture and disease pathology are critical for advancing pulmonary research but remain challenging to generate. Here, we developed a robust self‐organisation protocol to produce vLOs with cellular heterogeneity and functional vasculature. The engineered blood vessels within
Simin Jiang, Liangliang Tian, Jingge Qu, Yi Hong, Dian Chen, Zihang Pan, Huanyu Long, Lanhe Chu, Weijing Kong, Qiyang Yao, Xiaojing Ma, Yun Zhao, Baihui Ma, Kai Wang, Yahong Chen   +14 more
wiley   +1 more source

Expert Consensus on the Diagnosis and Monitoring of Paroxysmal Nocturnal Hemoglobinuria (PNH): An Algorithmic Approach in an Era of New Treatments

European Journal of Haematology, EarlyView.
ABSTRACT Paroxysmal nocturnal hemoglobinuria (PNH) is a rare hematologic disorder caused by a defect of glycosylphosphatidyl‐anchored proteins, leading to an uncontrolled complement‐mediated hemolysis. The advent of complement inhibitors in clinical practice radically changed patients' outcomes and survival.
Bruno Fattizzo, Monia Marchetti, Elisa Cannizzo, Arianna Gatti, Massimo Geuna, Anna Paola Iori, Maddalena Raia, Simona Sica, Francesco Buccisano   +8 more
wiley   +1 more source

Cisterna Chyli Dilation Is a Risk Factor for Liver Nodules and Hepatocellular Carcinoma in Fontan‐Associated Liver Disease: Pathophysiological Role of Lymphatic Dysfunction

Hepatology Research, EarlyView.
ABSTRACT Aim In Fontan‐associated liver disease (FALD), chronic congestion often confounds conventional fibrosis markers, complicating surveillance for hepatocellular carcinoma (HCC). Although lymphatic dysfunction is fundamental to Fontan physiology, its contribution to hepatocarcinogenesis remains unclear.
Koji Imoto, Masatake Tanaka, Takeshi Goya, Yuuki Azuma, Tomonobu Hioki, Yusuke Morita, Keisuke Sakaguchi, Nobuhiro Fujita, Takamori Kakino, Ayako Ishikita, Kenichiro Yamamura, Ichiro Sakamoto, Kousei Ishigami, Kohtaro Abe, Yoshihiro Ogawa   +14 more
wiley   +1 more source

Coexistence of pulmonary arterial hypertension and straight back syndrome in a patient with a novel BMPR2 variant affecting cytoplasmic tail domain

European Journal of Medical Research
Background Pathologic variants in the bone morphogenetic protein receptor-2 (BMPR2) gene cause a pulmonary arterial hypertension phenotype in an autosomal-dominant pattern with incomplete penetrance.
Mi Tang, Jun Luo, Qingqing Liu, Jie Song
doaj   +1 more source

Hot topics in the mechanisms of pulmonary arterial hypertension disease: cancer-like pathobiology, the role of the adventitia, systemic involvement, and right ventricular failure

Pulmonary Circulation, 2019
In order to intervene appropriately and develop disease-modifying therapeutics for pulmonary arterial hypertension, it is crucial to understand the mechanisms of disease pathogenesis and progression.
Edda Spiekerkoetter, Elena A. Goncharova, Christophe Guignabert, Kurt Stenmark, Grazyna Kwapiszewska, Marlene Rabinovitch, Norbert Voelkel, Harm J. Bogaard, Brian Graham, Soni S. Pullamsetti, Wolfgang M. Kuebler   +10 more
doaj   +1 more source

ЛЕГОЧНАЯ АРТЕРИАЛЬНАЯ ГИПЕРТЕНЗИЯ КАК ПРОЯВЛЕНИЕ ПАРАНЕОПЛАСТИЧЕСКОГО СИНДРОМА ПРИ ГИПЕРНЕФРОМЕ

Архивъ внутренней медицины, 2012
We give a case report of idiopathic pulmonary arterial hypertension (PAH) in a male patient with cancer of kidney. The hypothesis of pathogenetic link between these diseases was made.
А. В. Ягода, И. Б. Павлова, Н. Н. Гладких, Ф. С. Семенова, Ю. В. Громова   +4 more
doaj   +1 more source

Risk of Hypertension and Mortality Among People With Narcolepsy or Idiopathic Hypersomnia: A Retrospective Cohort Analysis of the Danish National Patient Registry

Journal of Sleep Research, EarlyView.
This retrospective cohort study using data from the Danish National Patient Registry shows that individuals with narcolepsy or idiopathic hypersomnia have greater prevalence and risk of incident hypertension compared with matched controls, highlighting the elevated cardiovascular risk associated with these conditions.
Poul Jørgen Jennum, Michael Ibsen, Sam Mettam, Sarah C. Markt, Douglas S. Fuller, Jessica K. Alexander, Rikke Ibsen, Jakob Kjellberg, Caroleen Drachenberg   +8 more
wiley   +1 more source

Cardiac sympathetic dysfunction in pulmonary arterial hypertension: lesson from left-sided heart failure

Pulmonary Circulation, 2019
Sympathetic nervous system hyperactivity has a well-recognized role in the pathophysiology of heart failure with reduced left ventricular ejection fraction.
Valentina Mercurio, Teresa Pellegrino, Giorgio Bosso, Giacomo Campi, Paolo Parrella, Valentina Piscopo, Carlo G. Tocchetti, Paul M. Hassoun, Mario Petretta, Alberto Cuocolo, Domenico Bonaduce   +10 more
doaj   +1 more source

Validation of a Novel, Low‐Cost, Portable MRI‐Compatible Exercise Device in Healthy Volunteers and Patients With Pulmonary Hypertension

NMR in Biomedicine, Volume 39, Issue 11, November 2026.
We developed a simple, low‐cost MR‐compatible exercise device as an alternative to commercial ergometers. In this proof‐of‐concept study, it evoked a robust stress response during MR‐augmented cardiopulmonary exercise testing and distinguished pulmonary arterial hypertension patients from healthy controls.
Ruta Virsinskaite, James T. Brown, Tushar Kotecha, Darren Bower, Jennifer A. Steeden, Javier Montalt‐Tordera, Olivier Jaubert, Marianna Fontana, J. Gerry Coghlan, Daniel S. Knight, Vivek Muthurangu   +10 more
wiley   +1 more source

Surviving Males With PORCN Variants: Expanding the Clinical, Molecular, and Mechanistic Spectrum

Clinical Genetics, Volume 110, Issue 5, Page 605-615, November 2026.
Pathogenic PORCN variants are compatible with male survival in both mosaic and non‐mosaic states, expanding the FDH/PONGOS spectrum and improving diagnosis and genetic counseling. ABSTRACT Pathogenic variants in PORCN cause focal dermal hypoplasia (FDH/Goltz syndrome), an X‐linked dominant disorder historically considered lethal in males, with milder ...
Lucía Miranda‐Alcaraz, Simone Carbonera, Mónica Mora‐Gómez, Natalia Gallego‐Zazo, Inmaculada Guillén, Elena Padilla, Gaia Visani, Alessia Asaro, Valeria Vásquez‐Amell, Mario Cazalla, Manuel Rodríguez‐Canó, Cristina Silván, Pedro Arias, Juan Andrés Jiménez‐Estrada, Tomás Valle, Alejandro Cruz‐Utrilla, Pilar Escribano‐Subías, Nuria Ochoa Parra, María Jesús del Cerro Marín, Julián Nevado, Spanish PAH Consortium, Pablo Lapunzina, Fabio Sirchia, Jair Tenorio‐Castano   +23 more
wiley   +1 more source