Results 61 to 70 of about 1,712,061 (282)

Lipid Metabolic Dysregulation Driven by OSMR Mutations Underlies Amyloidogenesis in Primary Localized Cutaneous Amyloidosis

Advanced Science, EarlyView.
OSMR mutations disrupt STAT5 signaling and AKR1B10‐mediated lipid metabolism, leading to lipid accumulation and protein aggregation in keratinocytes. This metabolic reprogramming drives amyloid deposition in primary localized cutaneous amyloidosis, revealing a mechanistic link between lipid dysregulation and amyloidogenesis.
Huiting Liu, Wen Zheng, Chao Yang, Tao Yang, Qiuyu Chen, Ping Lu, Huan Yang, Jun Liu, Bin Yang   +8 more
wiley   +1 more source

Aquagenic Pruritus Questionnaire: Predicting Myeloproliferative Neoplasms in Patients with Aquagenic Pruritus

Dermatology and Therapy
Introduction Aquagenic pruritus (AP) is an underrecognized condition in which patients perceive itch following contact with water on clinically non-lesional skin.
Felix Witte, Elke Weisshaar, Martin Metz, Steffen Koschmieder, Susanne Isfort, Andreas E. Kremer, Martin Griesshammer, Svenja Royeck, Sonja Ständer, Claudia Zeidler   +9 more
doaj   +1 more source

Genotype–Phenotype Correlations of Monoallelic PFIC Variants in Pediatric Liver Disease: A Multicenter Retrospective Cohort Study

American Journal of Medical Genetics Part A, EarlyView.
ABSTRACT Progressive familial intrahepatic cholestasis (PFIC) is classically caused by biallelic pathogenic variants, yet monoallelic variants of uncertain significance (VUS) in PFIC‐associated genes are increasingly identified in children with cholestasis, creating diagnostic uncertainty.
Brett J. Hoskins, Tiziano Pramparo, Chaowapong Jarasvaraparn, Michael J. Wilsey, Voytek Slowik, Lakshmi Kunam, Janis M. Stoll, Ruben E. Quiros‐Tejeira, Simon Lam, Wikrom Karnsakul   +9 more
wiley   +1 more source

Increased Risk of Intrahepatic Cholestasis of Pregnancy in Women With Systemic Lupus Erythematosus Exposed to Azathioprine

Arthritis &Rheumatology, EarlyView.
Objective To evaluate the risk of intrahepatic cholestasis of pregnancy (ICP) in azathioprine (AZA)–exposed versus unexposed systemic lupus erythematosus (SLE) pregnancies within the multicenter prospective Lupus in Pregnancy (LEGACY) cohort. Methods LEGACY is conducted at Systemic Lupus International Collaborating Clinics in Canada, South Korea, Peru,
Reem Farhat, Maria del Carmen Zamora‐Medina, Sang‐Cheol Bae, Megan R. W. Barber, Ann E. Clarke, Paul R. Fortin, Zahi Touma, Carl A. Laskin, Isabelle Malhamé, Giada Sebastiani, Christine Peschken, Manuel F. Ugarte‐Gil, Alexandra Legge, Sasha Bernatsky, Évelyne Vinet   +14 more
wiley   +1 more source

Prevalence and Burden of Somatic Symptom Disorder in Atopic Dermatitis and Chronic Pruritus—The Persistent Somatic Symptoms Across Diseases: Pruritus (SOMA.PRU) Study

JEADV Clinical Practice
Background Somatic symptom disorder (SSD) is a relatively “novel” psychiatric diagnosis defined as excessive thoughts, feelings, or behaviors related to persistent somatic symptoms such as chronic pruritus (CP) and could be important in this population ...
Stefan M. Kahnert, Ansgar Koechel, Gina L. Frank, Svenja Royeck, Claudia Zeidler, Felix Witte, Christian Mess, Konstantin Agelopoulos, Volker Huck, Bernd Löwe, Rupert Conrad, Gudrun Schneider, Stefan W. Schneider, Sonja Ständer   +13 more
doaj   +1 more source

Integrated Clinical and Proteomic Profiling of CD19 Chimeric Antigen Receptor T Cell Therapy in Progressive Systemic Sclerosis

Arthritis &Rheumatology, EarlyView.
Objective To characterize the clinical, immunologic, and proteomic changes associated with CD19 chimeric antigen receptor T cell therapy in patients with progressive systemic sclerosis (SSc). Methods Patients with progressive SSc received CD19 chimeric antigen receptor (CAR)‐T cell therapy and were observed longitudinally for safety, clinical efficacy,
Chenhan Jia, Wanyi Lin, Zhangyi Zhao, Fenglin Wu, Chaoyu Gu, Yuankai Sun, Zhe Ding, Chang Wei, Xinyu Li, Cuiwei Xie, Peng Xia, Zhen Xia, Xuesong Liu, Shaoying Yang, Chunyan Zhang, Hanlin Yin, Liangjing Lu   +16 more
wiley   +1 more source

Selective Nerve Fibre Activation in Patients with Generalized Chronic Pruritus: Hint for Central Sensitization?

Acta Dermato-Venereologica, 2019
Central sensitization induces pain augmentation in chronic pain states. An analogous mechanism is speculated for chronic pruritus. This study compared patients with chronic pruritus (n = 79) of different origins (atopic dermatitis, chronic pruritus on ...
Manuel P. Pereira, Konstantin Agelopoulos, Johannes Köllner, Gitta Neufang, Martin Schmelz, Sonja Ständer   +5 more
doaj   +1 more source

Expert Perspective: Toward Treat‐to‐Target Management in Adult Idiopathic Inflammatory Myopathies

Arthritis &Rheumatology, Accepted Article.
Treat‐to‐target (T2T) has transformed the management of several rheumatic diseases through predefined therapeutic goals, structured disease assessment, and timely treatment adjustment. Despite major advances in the understanding and treatment of idiopathic inflammatory myopathies (IIM), a formal T2T framework for adult IIM has not yet been established.
Shiri Keret, Nantakarn Pongtarakulpanit, Rohit Aggarwal   +2 more
wiley   +1 more source

Efficacy and safety of empagliflozin for treating neutropenia and neutrophil dysfunction in paediatric patients with glycogen storage disease type Ib: A systematic review and meta‐analysis

British Journal of Clinical Pharmacology, EarlyView.
Aims Glycogen storage disease type Ib (GSD‐Ib) is a rare genetic disorder causing neutropenia and neutrophil dysfunction in children. G‐CSF has been the primary treatment, but emerging data support the potential of empagliflozin, an SGLT2 inhibitor, as a promising investigational option.
Elizabeth Iwasyk, Ryan Jin, Fabio Tuzzolino, Giusy Ranucci, Donatella Madonia, Alessio Provenzani   +5 more
wiley   +1 more source

Repeated intradermal lipopolysaccharide challenge responses in healthy volunteers: Implications for clinical pharmacology studies

British Journal of Clinical Pharmacology, EarlyView.
Aim Intradermal application of lipopolysaccharide (LPS), a Toll‐like receptor 4 agonist, induces a local inflammatory response and is used as a human challenge model to evaluate the pharmacodynamics of investigational medicinal products. While currently applied in a single, parallel‐group setting, alternative within‐subject designs involving repeated ...
Alexandra A. J. Sillé, Juliette A. van den Noort, Iva Lelios, Diana R. Pereira, Marzia A. Scelsi, Erica S. Klaassen, Philipp Schoenenberger, Naomi B. Klarenbeek, Pierre‐Eric Juif, Pascal Espie, Manon A. A. Jansen, Matthijs Moerland, Richard W. D. Welford   +12 more
wiley   +1 more source