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Clinical Rheumatology - This study aimed to assess treatment patterns and frequency of inadequate response associated with advanced therapy initiation among patients with ankylosing spondylitis...  相似文献   
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Shen  Pei  Zhang  Dahe  Abdelrehem  Ahmed  Jin  Shan  Luo  Yi  Yang  Chi 《Clinical oral investigations》2022,26(12):7071-7081
Clinical Oral Investigations - To investigate the association between sexual maturation and anterior disc displacement (ADD) of temporomandibular joint (TMJ). Adolescents aged...  相似文献   
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Clinical Oral Investigations - To analyze differences in variation of orthodontic diagnostic measurements on lateral cephalograms reconstructed from ultra low dose-low dose (ULD-LD) cone beam...  相似文献   
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PurposeThis study aimed to describe the spectrum of cancers observed in Bloom Syndrome and the observed survival and age of first cancer diagnosis in Bloom syndrome as these are not well-defined.MethodsData from the Bloom Syndrome Registry (BSR) was used for this study. Cancer history, ages of first cancer diagnosis, and ages of death were compiled from the BSR and analyzed.ResultsAmong the 290 individuals in the BSR, 155 (53%) participants developed 251 malignant neoplasms; 100 (65%) were diagnosed with 1 malignancy, whereas the remaining 55 (35%) developed multiple malignancies. Of the 251 neoplasms, 83 (33%) were hematologic and 168 (67%) were solid tumors. Hematologic malignancies (leukemia and lymphoma) were more common than any of the solid tumors. The most commonly observed solid tumors were colorectal, breast, and oropharyngeal. The cumulative incidence of any malignancy by age 40 was 83%. The median survival for all participants in the BSR was 36.2 years. There were no significant differences in time to first cancer diagnosis or survival by genotype among the study participants.ConclusionWe describe the spectrum of cancers observed in Bloom syndrome and the observed survival and age of first cancer diagnosis in Bloom syndrome. We also highlight the significant differences in survival and age of diagnosis seen among different tumor types and genotypes.  相似文献   
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目的探讨产前超声联合MRI对孤立性血管环的诊断及预后情况。方法选取我院经产前超声心动图检查诊断为孤立性血管环的胎儿82例作为观察对象,其中13例进行胎儿心脏MRI检查,观察走行于血管环内的气管有无受压,并以Kappa法对超声检出气管受压及MRI检出气管受压间的一致性进行评定,并随访出生后情况。结果82例孤立性血管环包括右位主动脉弓伴左锁骨下动脉迷走(RAA+ALSA组)63例(76.83%,63/82),双主动脉弓(DAA组)16例(19.51%,16/82),肺动脉吊带(PAS组)3例(3.66%,3/82)。产前超声诊断气管受压结果和MRI诊断结果一致性好(Kappa=0.83)。随访DAA组生后10例(62.50%,10/16)患儿出现呼吸道症状;RAA+ALSA组生后1例(1.67%,1/60)患儿出现呼吸道症状;PAS组胎儿生后均出现呼吸道症状。血管环胎儿染色体检查检出异常3例(5.56%,3/54)。结论产前超声心动图检查可明确诊断血管环并进行分型,MRI对于评估血管环气管通畅性有很好的补充作用。不合并染色体异常的孤立性血管环预后良好。  相似文献   
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Whether the underlying mutations are homozygous, heterozygous, or co-inherited with other hemoglobinopathies, sickle cell disease is known to afflict the kidneys, leading to the clinical entity known as sickle cell nephropathy (SCN). Although common, SCN remains diagnostically elusive. Conventional studies performed in the context of renal disorders often fail to detect early stage SCN. This makes the quest for early diagnosis and treatment more challenging, and it increases the burden of chronic kidney disease-related morbidity among patients. Novel diagnostic tools have been employed to overcome this limitation. In this study, we discuss various biomarkers of SCN, including those employed in clinical practice and others recently identified in experimental settings, such as markers of vascular injury, endothelial dysfunction, tubulo-glomerular damage, and oxidative stress. These include kidney injury molecule-1, monocyte chemoattractant protein-1, N-acetyl-B-D-glucosaminidase, ceruloplasmin, orosomucoid, nephrin, and cation channels, among others. Furthermore, we explore the potential of novel biomarkers for refining diagnostic and therapeutic approaches and describe some obstacles that still need to be overcome. We highlight the importance of a collaborative approach to standardize the use of promising new biomarkers. Finally, we outline the limitations of conventional markers of renal damage as extensions of the pathogenic process occurring at the level of the organ and its functional subunits, with a discussion of the expected pattern of clinical and biochemical progression among patients with SCN.  相似文献   
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目的:探讨以口腔颌面部症状为首发的颌骨转移性腺癌(metastatic adenocarcinoma of the jaw,MAJ)临床及CT影像学特征.方法:收集2006~2020年符合纳入标准的MAJ患者,回顾性分析其临床及CT影像学特点.结果:14例分别源于肺(6例)、肝(4例)、肾(2例)、前列腺和贲门(各1例).骨质改变分为5型:溶骨型占5/14,边缘呈浸润型改变;混合型占4/14,边缘大多呈虫蚀状改变;成骨型占1/14,边缘呈硬化型改变;类囊型及牙槽骨吸收型分别占3/14、1/14,边缘均呈地图型改变.结论:MAJ多见于中老年男性的下颌骨后部.首次将其在传统分类基础上,新增类囊型及牙槽骨吸收型.MAJ以进展迅速的溶骨型骨质破坏伴骨膜反应及局限性软组织肿块,且颌骨无明显膨隆及颏部麻木综合征为特征,为临床诊断提供重要依据.  相似文献   
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