Results 31 to 40 of about 1,687 (159)

Severe ADEM‐Like Neuroinflammatory Disease and Cerebrovascular Fragility With Recurrent Pseudoaneurysms and Moyamoya in a Familial Germline CBL Mutation: Expanding the Clinical Phenotype

American Journal of Medical Genetics Part A, EarlyView.
ABSTRACT Heterozygous germline variants in CBL disrupt its function as a negative regulator of the Ras/MAPK pathway, classically predisposing to Juvenile myelomonocytic leukemia (JMML) and moyamoya. We describe two affected siblings carrying a paternally inherited CBL variant (c.1210 T> C, p.
Michal Bar‐Hakim, Anat Abramovich, Sara Via‐Dorembus, Gali Heimer, Bruria Ben‐Zeev, Etai Adam, Assaf A. Barg, Jonathan Roth, Basheer Sheick‐Yousif, Rony Beeri Berkowiz, Michal Feldon, Haim Bassan, Moran Hausman‐Kedem   +12 more
wiley   +1 more source

Treatment with Corticosteroid and/or Immunosuppressive Agents before Surgery can Effectively Improve the Surgical Outcome in Patients with Takayasu's Arteritis

Journal of Investigative Surgery, 2019
Objective: To analyze the clinical outcome of corticosteroid and/or immunosuppressive treatment preoperatively in patients with Takayasu's arteritis. Patients and Methods: Forty-six patients with Takayasu's arteritis who received cardiovascular surgery ...
Tie Zheng, Shuai Zhu, Jia-Fu Ou, Wei-Gang Fang, Zhi-Yu Qiao, Rui-Dong Qi, Li Chen, Lei Chen, Cheng-Nan Li, Li-Li Pan, Qing Zhu, Dong Chen, Xue-Jun Sun, Jun-Ming Zhu   +13 more
doaj   +1 more source

Anaesthetic Management of Patients with Takayasu’s Arteritis for Open Cholecystectomy: a Report of Two Cases

Journal of Nepal Medical Association, 2015
Takayasu’s arteritis is a well known yet rare form of large vessel vasculitis. This inflammatory disease often affects the ascending aorta and aortic arch, causing obstruction of the aorta and its major arteries. Anaesthetic management for these patients
Gentle Sunder Shrestha, Ninadini Shrestha, Battu Kumar Shrestha, Pramesh Sunder Shrestha   +3 more
doaj   +3 more sources

A Multifaceted Interplay Among Hemophagocytosis, Interleukin‐18, and Type I Interferon Distinguishes Still Disease From Other Autoinflammatory Diseases

Arthritis &Rheumatology, EarlyView.
Objective The unknown pathophysiology and the lack of specific features for systemic juvenile idiopathic arthritis and adult‐onset Still disease (collectively known as Still disease; SD) delay diagnosis and appropriate treatment. The goal of this study was to identify features and mechanisms that distinguish SD from other systemic autoinflammatory ...
Yvonne M. Mueller, Charlotte Girard, Harmen J. G. van de Werken, Stefan J. Erkeland, Dwin G. B. Grashof, Laurie Vaillant, Renske J. H. den Dekker, Iris van Zuijen, Sharon Veenbergen, Fanny Coffin, Eric Bindels, Yvan Jamilloux, Katerina Laskari, Vassili Soumelis, Bruno Fautrel, Cem Gabay, Peter D. Katsikis   +16 more
wiley   +1 more source

Effective Performance of the 2022 American College of Rheumatology/EULAR Classification Criteria for Antineutrophil Cytoplasmic Antibody–Associated Vasculitis in Pediatric Patients: An ARChiVe Study

Arthritis &Rheumatology, EarlyView.
Objective To assess the 2022 American College of Rheumatology (ACR)/EULAR classification criteria for antineutrophil cytoplasmic antibody–associated vasculitis (AAV) in children with chronic small‐to‐medium vessel vasculitis. Methods A cohort of 574 patients, identified by physician's diagnosis (MD‐diagnosis) in A Registry of Childhood Vasculitis, was ...
David A. Cabral, Else S. Bosman, Nick McPhate, Simranpreet K. Mann, Kirandeep K. Toor, Kimberly A. Morishita, Raashid Luqmani, Michael W. Beresford, James Bistolarides, Sarah Campillo, Sirirat Charuvanij, Kathryn Cook, Paul Dancey, Marietta de Guzman, Samundeeswari Deepak, Barbara Eberhard, Melissa Elder, Samuel Gagne, Katie Harrison, Adam Huber, Archana Khan, Susan Kim, Marisa S. Klein‐Gitelman, Tzielan C. Lee, Suzanne C. Li, Neil Martin, Flora McErlane, L. Nandini Moorthy, Alvaro H. Orjuela, Jonathan Park, Phil Riley, Alan M. Rosenberg, Susan Shenoi, Vidya Sivaraman, Sodality Sutnga, Tamara Tanner, Stacey E. Tarvin, Marinka Twilt, Linda Wagner‐Weiner, Rae S. M. Yeung, Kelly L. Brown, for the ARChiVe Investigators Network within the PedVas Initiative   +41 more
wiley   +1 more source

Age‐Associated B Cells Contribute to Inflammation Via Antibody‐Independent Mechanisms in Takayasu Arteritis

Arthritis &Rheumatology, EarlyView.
Objective Although Takayasu arteritis (TAK) is not a prototypical autoantibody‐mediated disease, accumulating evidence suggests that B cells are involved. This study aimed to investigate the pathway of B cell activation and its contributions to TAK pathogenesis. Methods Histologic analysis of paravascular lymph nodes and affected arteries was conducted
Chenglong Fang, Xiaoxi Yang, Xiaochuan Sun, Shangyi Jin, Lihong Du, Jinwei Gao, Jing Li, Zhen Chen, Yuexin Chen, Mengtao Li, Xinping Tian   +10 more
wiley   +1 more source

Rare vasculitis types and obstetric and neonatal outcomes – A population‐based study

International Journal of Gynecology &Obstetrics, EarlyView.
Abstract Objective Vasculitis is an infrequent pathology among reproductive‐aged women. While data exists regarding pregnancy outcomes in the more common vasculitis subtypes, data is limited regarding these outcomes in rare vasculitis subtypes. We aimed to compare pregnancy and perinatal outcomes between women who suffered from rare types of vasculitis
Uri Amikam, Ahmad Badeghiesh, Haitham Baghlaf, Richard Brown, Michael H. Dahan   +4 more
wiley   +1 more source

Takayasu’s Arteritis [PDF]

Circulation, 2006
A 24-year-old woman presented with sudden paresis of the right hemisoma. Systolic blood pressure in the left arm was 80 mm Hg and in the right arm was 110 mm Hg. The laboratory data showed systemic inflammation (erythrocyte sedimentation rate: 47 mm/h; C-reactive protein, 3.57 mg/dL). Echo-color Doppler …
Meini S., De Franco V., Auteri A., Pieragalli D.   +3 more
openaire   +2 more sources

Transcranial Color‐Coded Duplex Sonography in Large‐Vessel Vasculitis Compatible With Takayasu Arteritis Presenting Intracranial Involvement and Vertebrobasilar Dolichoectasia: A Case Report

Journal of Clinical Ultrasound, EarlyView.
This case illustrates sequential intracranial occlusions in large‐vessel vasculitis compatible with Takayasu arteritis preserving perfusion through robust collateral pathways. Transcranial color‐coded duplex sonography identified characteristic blunted flow patterns and focal turbulence, underscoring its value in detecting hemodynamic compromise beyond
Maria Júnia Lira e Silva, Jordana Gaudie Gurian, Isabella Mesquita Venâncio, Eva Carolina Rocha, Gisele Sampaio Silva   +4 more
wiley   +1 more source

Takayasu’s Arteritis Presenting as Ischemic Stroke in Young Patient

Journal of Rawalpindi Medical College, 2019
Takayasu’s arteritis is a chronic inflammatory vasculopathy. It commonly affects large blood vessels like aorta and its branches. Disease prevalence is more in women in second to third decade of life.
Sehrish Iqbal, Awais Abbasi, Noor Fatima, Muhammad Hamza, Najia Mahmood, Muhammad Khurram   +5 more
doaj