Results 61 to 70 of about 70,611 (273)

Therapy for Myhre Syndrome: Goals, Misconceptions, and Current Agents

American Journal of Medical Genetics Part C: Seminars in Medical Genetics, EarlyView.
ABSTRACT Myhre Syndrome (MYHRS, MIM #139210) is a rare, multisystem connective tissue disorder caused by recurrent heterozygous gain‐of‐function pathogenic variants in the SMAD4 gene, a key player in TGF‐β signaling and a regulator of extracellular matrix homeostasis.
Alessandro De Falco, Alfonso Manuel D'Alessio, Nicola Brunetti‐Pierri   +2 more
wiley   +1 more source

Delayed Diagnosis of Constrictive Pericarditis Resulting in Recurrent Heart Failure: A Case Report

Clinical Medicine Insights: Case Reports
Constrictive pericarditis can lead to compromised diastolic ventricular filling due to pericardial inflammation and fibrosis. A diagnosis of constrictive pericarditis was established by identifying structural and hemodynamic features through ...
Chunliang Wang, Yuzhu Fan, Guiting Liang, Yu Chen, Tian Tu, Juan Du   +5 more
doaj   +1 more source

The Effects of Angiotensin Converting Enzyme Inhibitors (ACE-I) on Human N-Acetyl-Seryl-Aspartyl-Lysyl-Proline (Ac-SDKP) Levels: A Systematic Review and Meta-Analysis [PDF]

, 2015
BACKGROUND: Tuberculous pericardial effusion is a pro-fibrotic condition that is complicated by constrictive pericarditis in 4% to 8% of cases. N-acetyl-seryl-aspartyl-lysyl-proline (Ac-SDKP) is a ubiquitous tetrapeptide with anti-fibrotic properties ...
Borkum, Megan S, Engel, Mark E, Mayosi, Bongani M, Mnguni, Ayanda Trevor   +3 more
core   +2 more sources

Prediction of Relapse and Glucocorticoid Dependence in Eosinophilic Granulomatosis With Polyangiitis: Findings From a Large European Cohort

Arthritis &Rheumatology, EarlyView.
Objective Eosinophilic granulomatosis with polyangiitis (EGPA) is a small vessel vasculitis characterized by eosinophilia, asthma, and ear, nose, and throat (ENT) involvement. Although glucocorticoids (GCs) are effective in controlling symptoms, relapses and GC dependence are common. The aim of this study was to develop predictive models for vasculitis
Matthias Papo, Pauline Martinot, Renato A. Sinico, Vítor Silvestre‐Teixeira, Nils Venhoff, Maria‐Letizia Urban, Michele Iudici, Juliane Mahrhold, Francesco Locatelli, Giulia Cassone, Franco Schiavon, Benjamin Seeliger, Thomas Neumann, Claudia Feder, Matthieu Groh, Chiara Marvisi, Maxime Samson, Thomas Barba, David Jayne, Arianna Troilo, Jens Thiel, Bernhard Hellmich, Sara Monti, Carlomaurizio Montecucco, Carlo Salvarani, Jean‐Emmanuel Kahn, Bernard Bonnotte, Cécile‐Audrey Durel, Xavier Puéchal, Luc Mouthon, Loïc Guillevin, Giacomo Emmi, Augusto Vaglio, Raphaël Porcher, Benjamin Terrier, on behalf of the French Vasculitis Study Group and the Eosinophilic Granulomatosis with Polyangiitis European Study Group   +35 more
wiley   +1 more source

Malignant pleural mesothelioma with constrictive pericarditis as the first manifestation: A case report

Clinical Case Reports, 2023
Pleural mesothelioma (PM) with pericardial involvement is extremely rare. We now report a rare case of malignant PM with constrictive pericarditis as the first presentation.
Cheng‐Peng He, Di‐Wei Tu, Ting‐Wei Zhang, Qian Zhang, Qiang Zhang, Di Kang, Ying‐Ying Wang, Ying‐Ying Li, Bin Zhang, Sha‐Sha Han, Hong‐Bo Li   +10 more
doaj   +1 more source

Constrictive Pericarditis: A Medical or Surgical Disease?

Journal of Cardiovascular Imaging, 2019
Constrictive pericarditis is a disease of the pericardium resulting from chronic inflammation and/or scar responsible for a clinical feature of left and right ventricular failure.
Sung‐A Chang, J. Oh
semanticscholar   +1 more source

A Rare RIPK3 Variant Enhances Necroptosis and Promotes Inflammation in a Still's Disease‐like Autoinflammatory Syndrome

Arthritis &Rheumatology, Accepted Article.
Objectives Still's Disease represents a prototypical polygenic systemic autoinflammatory diseases (SAIDs), characterized by recurrent systemic inflammation and dysregulation of innate immunity. Despite extensive clinical characterization, familial clustering Still's disease remains unreported.
Longfang Chen, Qihua Dai, Yu Xiao, Jialin Dai, Mengyan Wang, Yuning Ma, Yujie Shen, Zhimo Xu, Jianfen Meng, Xia Chen, Da Yi, Yue Sun, Hui Shi, Honglei Liu, Xiaobing Cheng, Yutong Su, Junna Ye, Huihui Chi, Zhuochao Zhou, Tingting Liu, Jialin Teng, Chengde Yang, Jinchao Jia, Qiongyi Hu   +23 more
wiley   +1 more source

A failing right heart in an eggshell: A case report of idiopathic constrictive pericarditis

Clinical Case Reports
key Clinical Message Constrictive Pericarditis is a rare fibrotic conversion of the pericardium that results in non‐specific clinical symptoms such as hepatomegaly, ascites, pleural effusions, and lower extremity edema.
Nivedha Balaji, Sheriff N. Dodoo, Alex Adams, Priyadarshini Dixit, Giancarlo Acosta, Jaime Burkle   +5 more
doaj   +1 more source

Pericardial mesothelioma presenting as chronic constrictive pericarditis. A series of three cases from a single institution

Indian Journal of Pathology and Microbiology, 2018
Pericardial mesotheliomas are rare tumors which often present with features of constrictive pericarditis. We present clinical, imaging, histological, and immunohistochemical findings of three cases presenting with chronic constrictive pericarditis.
Monalisa Hui, K R Harshavardhana, Shantveer G Uppin   +2 more
doaj   +1 more source

Chronic constrictive pericarditis: a rare cardiac involvement in primary Sjögren’s syndrome

BMC Cardiovascular Disorders, 2023
Background Constrictive pericarditis represents a chronic condition and systemic inflammatory diseases are a known, yet uncommon, cause. Pericardial involvement is seldom reported in primary Sjögren’s syndrome, usually occurring in association with ...
Fabiana Duarte, Luís Oliveira, Tomás Fontes, Sância Ramos, Raquel Dourado, Dinis Martins   +5 more
doaj   +1 more source