Results 41 to 50 of about 4,727 (145)
European Journal of Haematology, EarlyView.ABSTRACT Objectives Genetic rare diseases (GRDs), including chronic granulomatous disease, familial hemophagocytic lymphohistiocytosis, and congenital neutropenia, often require hematopoietic stem cell transplantation (HSCT) as the only curative option.
Bo Kyung Kim +6 more
wiley +1 more source
Pulmonary Hypertension Related to Left-Sided Cardiac Pathology
Pulmonary Medicine, 2011 Pulmonary hypertension (PH) is the end result of a variety of diverse pathologic processes. The chronic elevation in pulmonary artery pressure often leads to right ventricular pressure overload and subsequent right ventricular failure. In patients with
Todd L. Kiefer, Thomas M. Bashore
doaj +1 more source
GCN2 in proteostasis: structural logic, signalling networks and disease
The FEBS Journal, EarlyView.Threats to protein synthesis activate the kinase GCN2, initiating the integrated stress response (ISR). GCN2 is triggered by stalled ribosomes and uncharged tRNAs, which accumulate when amino acids are scarce. The ISR adjusts cellular physiology by promoting redox balance, protein quality control, and mitochondrial optimisation.
JiaYi Zhu, Stefan J. Marciniak
wiley +1 more source
Pulmonary Circulation, 2020 Mutations in the gene encoding bone morphogenetic protein receptor type II ( BMPR2 ) have been associated with heritable pulmonary arterial hypertension (HPAH), whereas mutations in the gene encoding eukaryotic translation initiation factor 2 alpha ...
Ifeoma Oriaku +5 more
doaj +1 more source
Translating cardiovascular ion channel and Ca2+ signalling mechanisms into therapeutic insights
The Journal of Physiology, EarlyView.Abstract figure legend This white paper integrates mechanistic discoveries across ion channel biology, Ca2+ signalling and multiscale cardiovascular physiology to highlight new opportunities for accelerating research and guiding next‐generation therapies. Printed with permission from ®Anita Impagliazzo Medical Illustration. [Correction added on 2 March
Silvia Marchianò +18 more
wiley +1 more source
Clinical Pharmacology &Therapeutics, Volume 120, Issue 3, Page 702-712, September 2026.Busulfan's narrow therapeutic index and high pharmacokinetic (PK) variability warrant investigation of its exposure–toxicity relationship. We retrospectively analyzed 334 pediatric and young adult patients who underwent allogeneic hematopoietic stem cell transplantation at Seoul National University Children's Hospital between 2009 and 2020 and received
Sungyeun Bae +7 more
wiley +1 more source
Histopathology, Volume 89, Issue 3, Page 403-425, September 2026.This review highlights changes relevant to pathologists in the updated multidisciplinary classification of interstitial pneumonias. Changes include expansion beyond idiopathic disease, subclassification as interstitial (fibrotic vs non‐fibrotic) and alveolar filling disorders, expansion to include additional patterns (e.g.
Andrew G Nicholson +7 more
wiley +1 more source
The Role of Hematopoietic Cell Transplantation in Ataxia‐Telangiectasia
Pediatric Blood &Cancer, Volume 73, Issue 8, August 2026.ABSTRACT Background Ataxia‐telangiectasia (A‐T) is a DNA repair disorder characterized by neurodegeneration, immunodeficiency, and cancer predisposition. Hematopoietic cell transplantation (HCT) is an established therapy in related disorders such as Fanconi anemia (FA) and Nijmegen breakage syndrome (NBS), but its role in A‐T is unclear.
Laila Alkhouli +3 more
wiley +1 more source
Comprehensive Physiology, Volume 16, Issue 4, August 2026.Compared to traditional cardiometabolic comorbidities, COPD and atrial fibrillation are uniquely linked to pulmonary vascular remodeling in HFpEF. The impact of these two comorbidities on pulmonary vascular‐left atrial axis is highlighted by the shared biventricular morphology among HFpEF subgroups.
Mariana Garcia‐Arango +20 more
wiley +1 more source
Pulmonary veno-occlusive disease
Revue des maladies respiratoires, 2018 Pulmonary veno-occlusive disease (PVOD) is a rare form of pulmonary hypertension (PH) characterized by preferential remodelling of pulmonary venules and angioproliferation. PVOD term includes idiopathic, heritable (biallelic mutations of EIF2AK4 gene), drugs and toxins induced (alkylating agents, organic solvents) and connectivite-associated forms ...
A, Boucly +11 more
openaire +3 more sources