keyword
https://read.qxmd.com/read/38523185/the-role-of-cardiac-imaging-in-assessing-the-cardiac-involvement-of-type-1-gaucher-disease-a-case-report-with-review-of-literature
#21
JOURNAL ARTICLE
Ahmed Youssouf Addou, Wafa El Mire, Nawal Doghmi, Aatif Benyass
BACKGROUND: Gaucher disease (GD) is a lysosomal storage disease that leads to the accumulation of glucocerebroside within reticuloendothelial cells, haematological, neurological, skeletal and abdominal organs. These clinical manifestations are common to all types of GD, but categorization depends on the absence of neurological involvement (type I) or its presence (type II and III). Cardiac involvement is rare and only reported in few cases, where valvular and aortic calcifications were associated with type IIIc...
March 24, 2024: Egyptian Heart Journal: EHJ
https://read.qxmd.com/read/38523167/identification-of-non-coding-rna-signatures-in-idiopathic-pulmonary-fibrosis
#22
JOURNAL ARTICLE
Alperen Elek, Esra Bozgeyik, Halil Caska, Zekihan Gocer, Ibrahim Bozgeyik
BACKGROUND: Idiopathic pulmonary fibrosis (IPF) is a deadly, chronic, progressive, irreversible interstitial lung disease characterized by the formation of scar tissue resulting in permanent lung damage. The average survival time following diagnosis is only 3-5 years, with a 5-year survival rate shorter than that of many cancers. Alveolar epithelial cell injury followed by irregular repair is the primary pathological process observed in patients with IPF. An evident characteristic of IPF is the development of fibroblastic foci representing active fibrotic areas...
March 25, 2024: Irish Journal of Medical Science
https://read.qxmd.com/read/38522973/a-new-proposal-for-phenotypic-classification-and-outcome-assessment-of-dermatomyositis-based-on-clinical-manifestations-and-serological-testing
#23
JOURNAL ARTICLE
Ting Huang, Ting Ding, Liqing Ding, Shasha Xie, Xiaojing Li, Qiming Meng, Xiaomeng Wu, Hui Luo, Hongjun Zhao
BACKGROUND: Dermatomyositis (DM) is an infrequent disease subgroup of idiopathic inflammatory myopathies characterized by distinct skin lesions. However, high heterogeneity makes clinical diagnosis and treatment of DM very challenging. OBJECTIVES: Unsupervised classification in DM patients and analysis of key factors related to clinical outcomes. METHODS: This retrospective study was conducted between 2017 and 2022 at the Department of Rheumatology, Xiangya Hospital, Central South University...
March 23, 2024: Anais Brasileiros de Dermatologia
https://read.qxmd.com/read/38522908/secondary-pulmonary-alveolar-proteinosis-development-during-the-treatment-for-anti-aminoacyl-trna-synthetase-antibody-positive-interstitial-lung-disease
#24
JOURNAL ARTICLE
Suzuka Matsuoka, Keiichi Fujiwara, Yuki Takigawa, Saki Ito, Sho Mitsumune, Keisuke Shiraha, Mayu Goda, Tomoyoshi Inoue, Miho Fujiwara, Eri Nakamura, Hiromi Watanabe, Kenichiro Kudo, Akiko Sato, Ken Sato, Takuo Shibayama
A 70-year-old woman with anti-aminoacyl-tRNA synthetase (ARS) antibody-positive interstitial lung disease (ARS-ILD) received daily medications and regular cyclophosphamide cycles for recurring exacerbations. Approximately four years after immunosuppression initiation, the patient was admitted for progressive dyspnea on exertion. Chest computed tomography (CT) findings were suggestive of acute exacerbation. Despite intensified immunosuppressive treatment, the radiographic findings worsened, and serum Krebs von den Lungen-6 (KL-6) levels increased...
March 25, 2024: Internal Medicine
https://read.qxmd.com/read/38522361/endobronchial-silicone-spigot-in-prolonged-air-leaks-nationwide-study-on-outcomes-and-risk-factors-for-treatment-failure
#25
JOURNAL ARTICLE
Jumpei Taniguchi, Shotaro Aso, Jo Taisuke, Hiroki Matsui, Kiyohide Fushimi, Hideo Yasunaga
BACKGROUND: The endobronchial silicone spigot, also known as the endobronchial Watanabe spigot, is used in bronchoscopic interventions to manage prolonged pulmonary air leakage. However, the outcomes of this procedure have not been thoroughly investigated. METHODS: Using a Japanese national inpatient database from April 2014 to March 2022, we assessed the clinical characteristics and outcomes of all eligible patients who received the endobronchial spigot. We also investigated risk factors associated with treatment failure...
March 23, 2024: Respiratory Investigation
https://read.qxmd.com/read/38522142/a-cluster-of-type-i-interferon-regulated-genes-associates-with-disease-activity-and-prognosis-in-patients-with-iga-nephropathy
#26
JOURNAL ARTICLE
Shu Qu, Ting Gan, Yan-Na Wang, Yuan-Yuan Qi, Yue-Miao Zhang, Celine C Berthier, Li-Jun Liu, Su-Fang Shi, Ji-Cheng Lv, Hong Zhang, Xu-Jie Zhou
The exact pathogenesis of IgA nephropathy (IgAN) is complex and so far, not well defined. Since it has been shown that microbial infections could induce high levels of type I interferon (IFN-I) and there is an evident link between mucosal infection and gross hematuria in IgAN, we hypothesized that IFN-I may play a role in the pathogenic process. In this study, we investigated the type I interferon status in IgAN based on the expression of 17 IFN-regulated genes (IRGs) in whole blood from 59 IgAN patients in a cross-sectional study, of which 34 patients followed longitudinally...
March 23, 2024: International Immunopharmacology
https://read.qxmd.com/read/38522139/single-cell-transcriptome-profiling-of-infrapatellar-fat-pad-highlights-the-role-of-interstitial-inflammatory-fibroblasts-in-osteoarthritis
#27
JOURNAL ARTICLE
Hongxu Pu, Chenghao Gao, Yi Zou, Liming Zhao, Guanghao Li, Changyu Liu, Libo Zhao, Meng Zheng, Gaohong Sheng, Xuying Sun, Xingjie Hao, Chaolong Wang, Ximiao He, Jun Xiao
OBJECTIVES: Osteoarthritis (OA) is a whole-joint disease in which the role of the infrapatellar fat pad (IFP) in its pathogenesis is unclear. Our study explored the cellular heterogeneity of IFP to understand OA and identify therapeutic targets. METHODS: Single-cell and single-nuclei RNA sequencing were used to analyze 10 IFP samples, comprising 5 from OA patients and 5 from healthy controls. Analyses included differential gene expression, enrichment, pseudotime trajectory, and cellular communication, along with comparative studies with visceral and subcutaneous fats...
March 23, 2024: International Immunopharmacology
https://read.qxmd.com/read/38521926/the-role-of-lung-biopsy-for-diagnosis-and-prognosis-of-interstitial-lung-disease-in-systemic-sclerosis-a-systematic-literature-review
#28
REVIEW
A Damiani, M Orlandi, C Bruni, G Bandini, G Lepri, C Scaletti, C Ravaglia, F Frassanito, S Guiducci, A Moggi-Pignone, M Matucci-Cerinic, V Poletti, L Tofani, T V Colby, S Bellando Randone, Sara Tomassetti
BACKGROUND: The prognostic and theragnostic role of histopathological subsets in systemic sclerosis interstitial lung disease (SSc-ILD) have been largely neglected due to the paucity of treatment options and the risks associated with surgical lung biopsy. The novel drugs for the treatment of ILDs and the availability of transbronchial cryobiopsy provide a new clinical scenario making lung biopsy more feasible and a pivotal guide for treatment. The aim of our study was to investigate the usefulness of lung biopsy in SSc ILD with a systematic literature review (SLR)...
March 23, 2024: Respiratory Research
https://read.qxmd.com/read/38521840/post-marketing-surveillance-of-the-safety-and-effectiveness-of-nivolumab-for-classic-hodgkin-lymphoma-in-japan
#29
JOURNAL ARTICLE
Akira Kawasaki, Kiyohiko Hatake, Itaru Matsumura, Koji Izutsu, Tomohiro Hoshino, Ayumi Akamatsu, Akito Kakuuchi, Kensei Tobinai
Nivolumab was approved for relapsed/refractory classic Hodgkin lymphoma (cHL) in Japan in 2016. After its approval, a prospective, non-interventional, observational post-marketing surveillance was initiated to evaluate the safety and effectiveness of nivolumab treatment for up to 12 months in patients with relapsed/refractory cHL. Of 304 registered patients, 288 were included in safety analyses and 282 in effectiveness analyses. There were 191 (66.3%) male patients, median age was 64.0 years, and 54 patients (18...
March 23, 2024: International Journal of Hematology
https://read.qxmd.com/read/38521611/kidney-manifestations-of-sarcoidosis
#30
JOURNAL ARTICLE
Francesco Bonella, Adriane Dm Vorselaars, Benjamin Wilde
Renal involvement is a clinically relevant organ manifestation of sarcoidosis, leading to increased morbidity and complications. Although the exact incidence remains unknown, renal disease is likely to occur in up to one third of all sarcoidosis patients. Every patient with newly diagnosed sarcoidosis should receive a renal work-up and screening for disrupted calcium metabolism. Amid various forms of glomerulonephritis, granulomatous interstitial nephritis is the most common one, but it rarely leads to renal impairment...
March 22, 2024: Journal of Autoimmunity
https://read.qxmd.com/read/38519827/a-case-of-lomentospora-prolificans-endophthalmitis-treated-with-the-novel-antifungal-agent-olorofim
#31
JOURNAL ARTICLE
Michael Dong, Fiona Pearce, Nandini Singh, Ming-Lee Lin
PURPOSE: To report a case of endogenous Lomentospora prolificans endophthalmitis treated with the novel antifungal agent Olorofim. CASE REPORT: A 57-year-old man developed disseminated Lomentospora prolificans with right endophthalmitis on the background of immunosuppression following lung transplantation for interstitial lung disease. He was treated with early vitrectomy, intravitreal voriconazole, and systemic Olorofim, voriconazole and terbinafine. His symptoms improved and remained stable in the right eye...
March 22, 2024: Journal of Ophthalmic Inflammation and Infection
https://read.qxmd.com/read/38519114/inhaled-treprostinil-in-patients-with-pulmonary-hypertension-associated-with-interstitial-lung-disease-with-less-severe-haemodynamics-a-post-hoc-analysis-of-the-increase-study
#32
JOURNAL ARTICLE
Jason Weatherald, Steven D Nathan, Karim El-Kersh, Rahul G Argula, Hilary M DuBrock, Franz P Rischard, Steven J Cassady, James Tarver, Deborah J Levine, Victor F Tapson, Chunqin Deng, Eric Shen, Manisit Das, Aaron B Waxman
BACKGROUND: Inhaled treprostinil (iTre) is the only treatment approved for pulmonary hypertension due to interstitial lung disease (PH-ILD) to improve exercise capacity. This post hoc analysis evaluated clinical worsening and PH-ILD exacerbations from the 16-week INCREASE study and change in 6-minute walking distance (6MWD) in the INCREASE open-label extension (OLE) in patients with less severe haemodynamics. METHODS: Patients were stratified by baseline pulmonary vascular resistance (PVR) of <4 Wood units (WU) versus ≥4 WU and <5 WU versus ≥5 WU...
March 22, 2024: BMJ Open Respiratory Research
https://read.qxmd.com/read/38519110/prediction-of-progressive-fibrosing-interstitial-lung-disease-in-patients-with-systemic-sclerosis-insight-from-the-crdc-cohort-study
#33
JOURNAL ARTICLE
Min Hui, Xinwang Duan, Jiaxin Zhou, Mengtao Li, Qian Wang, Jiuliang Zhao, Yong Hou, Dong Xu, Xiaofeng Zeng
BACKGROUND: This study aims to establish a reliable prediction model of progressive fibrosing interstitial lung disease (PF-ILD) in patients with systemic sclerosis (SSc)-ILD, to achieve early risk stratification and to help better in preventing disease progression. METHODS: 304 SSc-ILD patients with no less than three pulmonary function tests within 6-24 months were included. We collected data at baseline and compared differences between SSc patients with and without PF-ILD...
March 22, 2024: RMD Open
https://read.qxmd.com/read/38519013/extracranial-transport-of-brain-lymphatics-via-cranial-nerve-in-human
#34
JOURNAL ARTICLE
Safiye Çavdar, Damlasu Altınöz, Tevriz Dilan Demir, İlke Ali Gürses, Gülnihal Özcan
Extracranial waste transport from the brain interstitial fluid to the deep cervical lymph node (dCLN) is not extensively understood. The present study aims to show the cranial nerves that have a role in the transport of brain lymphatics vessels (LVs), their localization, diameter, and number using podoplanin (PDPN) and CD31 immunohistochemistry (IHC) and Western blotting. Cranial nerve samples from 6 human cases (3 cadavers, and 3 autopsies) were evaluated for IHC and 3 autopsies for Western blotting. The IHC staining showed LVs along the optic, olfactory, oculomotor, trigeminal, facial, glossopharyngeal, accessory, and vagus nerves...
March 20, 2024: Neuroscience Letters
https://read.qxmd.com/read/38519008/age-related-changes-in-meningeal-lymphatic-function-are-closely-associated-with-vascular-endothelial-growth-factor-c-expression
#35
JOURNAL ARTICLE
Qi Liu, Cheng Wu, Qian Ding, Xiang-Yu Liu, Ni Zhang, Jun-Hui Shen, Zi-Tong Ou, Tuo Lin, Hong-Xiang Zhu, Yue Lan, Guang-Qing Xu
Meningeal lymphatic vessels (MLVs) have crucial roles in removing metabolic waste and toxic proteins from the brain and transporting them to the periphery. Aged mice show impaired meningeal lymphatic function. Nevertheless, as the disease progresses, and significant pathological changes manifest in the brain, treating the condition becomes increasingly challenging. Therefore, investigating the alterations in the structure and function of MLVs in the early stages of aging is critical for preventing age-related central nervous system degenerative diseases...
March 20, 2024: Brain Research
https://read.qxmd.com/read/38518719/novel-air-liquid-interface-culture-model-to-investigate-stiffness-dependent-behaviors-of-alveolar-epithelial-cells
#36
JOURNAL ARTICLE
Yuto Takahashi, Satoru Ito, Jungfeng Wang, Jeonghyun Kim, Takeo Matsumoto, Eijiro Maeda
Pulmonary alveoli are functional units in gas exchange in the lung, and their dysfunctions in lung diseases such as interstitial pneumonia are accompanied by fibrotic changes in structure, elevating the stiffness of extracellular matrix components. The present study aimed to test the hypothesis that such changes in alveoli stiffness induce functional alteration of epithelial cell functions, exacerbating lung diseases. For this, we have developed a novel method of culturing alveolar epithelial cells on polyacrylamide gel with different elastic modulus at an air-liquid interface...
March 15, 2024: Biochemical and Biophysical Research Communications
https://read.qxmd.com/read/38518217/vulvar-lichenoid-dermatoses-with-emphasis-on-the-distinction-between-lichen-sclerosus-and-lichen-planus-a-10-year-study
#37
JOURNAL ARTICLE
Sueallen Lorna D'Souza, Gayatri Ravikumar, Meryl Antony, Rajalakshmi Tirumale
OBJECTIVES: Lichen planus (LP) and lichen sclerosus (LS) are the most common vulvar lichenoid dermatoses. The diagnostic challenges are due to site-specific variation in microscopic appearance and small-sized biopsies. Authentication of diagnostic criteria to distinguish LS and LP to uncover any resemblance or divergence in presentation of these conditions is attempted. METHODS: Cases of vulvar LP and LS diagnosed between January 2012 to December 2022 were included...
April 1, 2024: Journal of Lower Genital Tract Disease
https://read.qxmd.com/read/38517050/neutrophil-extracellular-traps-induce-pyroptosis-of-pulmonary-microvascular-endothelial-cells-by-activating-the-nlrp3-inflammasome
#38
JOURNAL ARTICLE
Peipei Zhao, Jiarui Zhu, Ling Bai, Wenlan Ma, Feifei Li, Cen Zhang, Liangtao Zhao, Liuyang Wang, Sigong Zhang
Excessive formation of neutrophil extracellular traps (NETs) may lead to myositis-related interstitial lung disease (ILD). There is evidence that NETs can directly injure vascular endothelial cells and play a pathogenic role in the inflammatory exudation of ILD. However, the specific mechanism is unclear. This study aimed to investigate the specific mechanism underlying NET-induced injury to human pulmonary microvascular endothelial cells (HPMECs). HPMECs were stimulated with NETs (200 ng/ml) in vitro...
March 22, 2024: Clinical and Experimental Immunology
https://read.qxmd.com/read/38516502/whipple-s-disease-presenting-with-a-chief-complaint-of-dyspnea-and-cough-from-pulmonary-invasion-without-evidence-of-gastrointestinal-involvement
#39
Michael Ladna, John George, Christopher E Forsmark
A patient with immune thrombocytopenia, systemic lupus erythematosus on chronic corticosteroids, and interstitial lung disease was referred to the pulmonology clinic due to progressively worsening dyspnea. A bronchoscopy was done and a thorough workup was negative for any infectious pathology or malignancy. A lung biopsy with MicroGenDX test (MicroGen Diagnostics, Lubbock, TX) revealed Tropheryma whipplei , consistent with a Whipple disease diagnosis. Histopathology of biopsy specimens from an esophagogastroduodenoscopy showed moderate chronic active Helicobacter gastritis and unremarkable duodenal specimens without evidence of Tropheryma whipplei ...
February 2024: Curēus
https://read.qxmd.com/read/38516472/nephrological-detrimental-impacts-resulting-from-novel-immunotherapy-drugs-used-in-the-treatment-of-cancer-a-systematic-review
#40
REVIEW
Amisha Jaiswal, Kainaat Shergill, Kusalik Boppana, Naiela E Almansouri, Saloni Bakkannavar, Youmna Faheem, Tuheen Sankar Nath
The most recent advancements in cancer therapy center on efficiently and conveniently enhancing a patient's natural immune system. Immune checkpoint inhibitors (ICIs) are antibodies that target cytotoxic thymus (T) lymphocyte antigen-4 (CTLA-4) and its receptor. They function by stimulating T-cell activity against malignancies. Immune-related adverse events (irAEs) are a distinct class of inflammatory side effects that are specific to a given organ. Antineoplastic medications can impact any part of the kidney, leading to the development of proteinuria, hypertension, electrolyte abnormalities, glomerulonephritis, and both acute and chronic interstitial nephritis...
February 2024: Curēus
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