Remain idiopathic
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Whole-exome sequencing (WES) has become an efficient diagnostic test for patients with likely monogenic conditions such as rare idiopathic diseases or sudden unexplained death. Yet, many cases remain undiagnosed. Here, we report the added diagnostic yield achieved for 101 WES cases re-analyzed 1 to 7 years after initial analysis.
8p vibransone 28-03-2024 3 2 Download
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Remitting seronegative symmetrical synovitis with pitting edema is a rare rheumatic condition of the elderly population that is well described but whose mechanisms remain little studied. This syndrome is characterized by symmetrical swelling located mainly on the dorsal part of the hands and the feet.
5p vigamora 23-05-2023 3 2 Download
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Patients with Down syndrome are at a higher risk of developing autoimmune disorders such as thyroiditis, diabetes, and celiac disease compared with the general population. Although some diseases are well known to be associated with Down syndrome, others such as idiopathic pulmonary hemosiderosis and ischemic stroke due to protein C deficiency remain rare.
5p videadpool 05-05-2023 3 2 Download
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Myeloperoxidase antineutrophil cytoplasmic antibody (MPO-ANCA) is often positive in patients with interstitial lung disease (ILD), which is also often present in patients with microscopic polyangiitis (MPA). A possible association between MPO-ANCA, MPA, and idiopathic ILD remains unclear.
10p vimackenziebezos 29-11-2021 10 1 Download
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Pirfenidone is an anti-fibrotic agent shown to slow the progression of idiopathic pulmonary fibrosis (IPF). However, its effectiveness in association with serological autoimmune features in IPF remains unclear.
7p vijichoo2711 31-05-2021 8 1 Download
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Nintedanib is effective for treating idiopathic pulmonary fibrosis (IPF), but some patients may exhibit a suboptimal response and develop on-treatment acute exacerbation (AE-IPF), hepatic injury, or mortality. It remains unclear which patients are at risk for these adverse outcomes.
13p vijichoo2711 31-05-2021 10 1 Download
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Adolescent idiopathic scoliosis (AIS) is a common spinal deformity. Posterior spinal fusion remains an important surgical treatment for AIS. This study aims to determine the predictive factors for intraoperative blood loss in AIS surgery.
7p vianttinic2711 20-04-2021 18 2 Download
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Idiopathic pulmonary fibrosis (IPF) is a devastating condition characterized by progressive lung function decline and early mortality. While early accurate diagnosis is essential for IPF treatment, data evaluating the impact of hospital academic status on IPF-related mortality remains limited.
11p vimichigan2711 26-03-2021 16 3 Download
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Usual interstitial pneumonia can present with a probable pattern on high-resolution computed tomography (HRCT), but the probability of identifying usual interstitial pneumonia by surgical lung biopsy in such cases remains controversial.
8p vimichigan2711 26-03-2021 14 2 Download
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The intensity and frequency of cough remain unclear in interstitial lung disease (ILD). The aim of this study was to evaluate the intensity and frequency of cough in idiopathic interstitial pneumonias (IIPs), connective tissue disease-associated interstitial lung disease (CTD-ILD), and chronic hypersensitivity pneumonia (CHP), and examine their associations with clinical indices.
10p vimichigan2711 26-03-2021 13 2 Download
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Patients with interstitial lung disease (ILD) are occasionally positive for anti-neutrophil cytoplasmic antibodies (ANCAs). Diferences between ILDs secondary to microscopic polyangiitis (MPA) and isolated ANCA-positive idiopathic interstitial pneumonia (IIP) remain unclear.
10p vimichigan2711 26-03-2021 8 1 Download
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Aging is a known risk factor of idiopathic pulmonary fibrosis (IPF). However, the pathogenic mechanisms underlying the effects of advanced aging remain largely unknown. Telomeric repeat-containing RNA (TERRA) represents a type of long noncoding RNA.
15p vimaine2711 26-03-2021 7 2 Download
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Innate T helper type 2 (Th2) immune responses mediated by interleukin (IL)-33, thymic stromal lymphopoietin (TSLP), and IL-25 have been shown to play an important role in pulmonary fibrosis of animal models; however, their clinical implications remain poorly understood.
9p vimaine2711 26-03-2021 5 2 Download
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Eosinophilic pleural effusion (EPE) is attributed to several well-recognised causes. However, some patients remain idiopathic, even after thorough clinical work-up. The present study aimed to better characterize idiopathic EPE (IEPE) and to outline the diagnostic procedure for this disease.
7p vimaine2711 26-03-2021 10 2 Download
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Baicalin has been reported to have anti-fibrosis effect; however, its mechanism still remains to be elucidated. Adenosine A2a receptor (A2aR) is a novel inflammation regulator, and transforming growth factor-β1 (TGF-β1)-induced extracellular signal regulated kinase1/2 (ERK1/2) signaling pathway plays an important role in idiopathic pulmonary fibrosis (IPF).
11p vimaine2711 26-03-2021 5 1 Download
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Recent morphological analyses of vertebrae in patients with scoliosis have revealed three-dimensional (3D) deformities in the vertebral bodies. However, it remains controversial whether these deformities are secondary changes caused by asymmetrical vertebral loading or primary changes caused by aberrant asymmetrical vertebral growth.
8p vitennessee2711 02-02-2021 8 2 Download
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Although the pathogenesis of adolescent idiopathic scoliosis (AIS) remains unclear, there are little evidences of the pathogenesis in patients with thoracolumbar/lumbar AIS. The purpose of this study was to identify proteins or proteomes that may be causally related to the pathogenesis of AIS with structured thoracolumbar/ lumbar curvature using two-dimensional fluorescence difference gel electrophoresis (2D-DIGE).
8p vivermont2711 28-01-2021 11 2 Download
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Postoperative neck tilt (PNT) is a phenomenon in adolescent idiopathic scoliosis (AIS) patients which is distinct form shoulder imbalance. There were scarce studies performed to explore the risk factors for PNT in Lenke 1 and 2 AIS patients, and whether it can be predicted after surgery remains unknown.
9p vivermont2711 28-01-2021 8 2 Download
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The optimal anchor density in adolescent idiopathic scoliosis (AIS) surgery to achieve good curve correction remains unclear. The purpose of the study is to analyze the correlations between three-dimensional curve correction and anchor density in the pedicle screw-based posterior fusion of AIS.
10p vivermont2711 28-01-2021 10 2 Download
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Classification of the spinal deformity in adolescent idiopathic scoliosis (AIS) remains two-dimensional (2D) as the spinal radiographs remain the mainstay in clinical evaluation of the disease. 3D classification systems are proposed, however are time consuming.
9p vimariana2711 22-12-2020 7 0 Download