Metabolic bone diseases

  • 文章类型: Journal Article
    膝关节骨关节炎(OA膝关节)是一种常见的老年病,需要进行全膝关节置换术。假体周围骨折是常见的并发症之一,这与代谢性骨病密切相关。
    我们在2020年11月至2021年4月期间对291名OA膝关节门诊患者进行了一项前瞻性研究。基线特征,双能X线骨密度仪和血液检测结果采用logistic回归分析,以比值比表示,95%置信区间和P值。
    在82.91%的患者中发现了总体代谢性骨疾病。维生素D不足(53.38%)是最普遍的,其次是骨量减少(44.38%),肾功能受损(26.69%),和骨质疏松症(17.45%)。维生素D不足的危险因素为年龄≥71岁(OR0.33,P=0.003)和双侧患侧(OR1.99,P=0.007)。对于骨质减少和骨质疏松症,危险因素是年龄,身体质量指数,患侧,慢性肾脏病(P<0.05)。
    在维生素D不足方面,年龄>70岁和双侧OA膝关节在许多方面显着相关,如减少皮肤合成和日常暴露于阳光和各种疾病,包括CKD和GI吸收不良。骨质减少和骨质疏松症的危险因素可以在多种机制中解释。例如,激素和细胞因子代谢异常会导致脂肪细胞分化和脂肪积累的增加。这种情况可能导致成骨细胞分化减少和破骨细胞活性增加,这可能导致对骨强度的负面影响。
    代谢性骨病在大多数OA膝关节患者中很常见,和维生素D(血清25-羟维生素D)的量不足,骨质疏松,主要确定了骨量减少的条件。这些可预防的疾病具有大多可以纠正的风险因素,这些可预防的疾病具有大多可以纠正的风险因素,例如,获得更多的户外活动,服用维生素D补充剂或开始骨质疏松症治疗计划。需要进一步分析以建立可靠的证据来比较OA和非OA组之间的危险因素。
    UNASSIGNED: Osteoarthritis of the knee (OA knee) is a common geriatric disease that require total knee arthroplasty. Periprosthetic fracture is one of the common complications, which is strongly related to metabolic bone disease.
    UNASSIGNED: We conducted a prospective study on 291 outpatients with OA knee between November 2020 and April 2021. Baseline characteristics, dual-energy X-ray absorptiometry and blood test results were analysed using logistic regression analysis and expressed as odds ratio, 95% confidence intervals and P-values.
    UNASSIGNED: Overall metabolic bone diseases were found in 82.91% of patients. Vitamin D insufficiency (53.38%) was the most prevalent, followed by osteopenia (44.38%), impaired renal function (26.69%), and osteoporosis (17.45%). Risk factors of vitamin D insufficiency are age ≥71 years (OR 0.33, P = 0.003) and bilateral affected side (OR 1.99, P = 0.007). For osteopenia and osteoporosis, risk factors were age, body mass index, affected side, and chronic kidney disease (P < 0.05).
    UNASSIGNED: In the aspect of Vit D insufficiency, age>70 and bilateral OA knee were significantly related in many ways, such as reduction in cutaneous synthesis and daily exposure to sunlight and various diseases, including CKD and GI malabsorption. The risk factors of osteopenia and osteoporosis can be explained throughout several mechanisms. For instance, abnormality in hormone and cytokines metabolism will lead to the increase in adipocyte differentiation and fat accumulation. And that situation may lead to the decrease in osteoblast differentiation and increased osteoclast activity which could lead to negative impact on bone strength.
    UNASSIGNED: Metabolic bone diseases were common in most patients with OA knee, and the insufficiency in amount of vitamin D(serum 25-hydroxyvitamin D), osteoporosis, and osteopenia conditions were mainly identified. These preventable conditions have risk factors that are mostly correctable These preventable conditions have risk factors that are mostly correctable, for example gain more outdoor activities, consume vitamin D supplement or start osteoporosis treatment program. Further analysis is necessary to establish solid evidence in the comparison of risk factors between the OA and non-OA groups.
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  • 文章类型: Journal Article
    牙齿萌出是口腔颌面系统发育的重要过程。一些遗传性和获得性疾病可能会影响这个严格调控的过程,导致过早,延迟,甚至是失败的牙齿萌出。本文的目的是回顾影响牙齿萌出的代谢性骨疾病的文献和临床参数。它检查了牙齿萌出的生理方面以及代谢性骨骼疾病引起的病理生理变化,包括骨代谢的变化,密度,和结构。这篇评论的搜索策略包括在PubMed中进行电子搜索,谷歌学者,Medline,Scopus,和Cochrane图书馆使用以下关键字:“代谢性骨骼疾病”,“牙齿萌出”,“延迟的牙齿萌出”,每种报告的疾病都与“牙齿萌出障碍”相结合,涵盖截至2024年3月的出版物,仅限于英语来源。了解代谢性骨骼疾病对牙齿萌出的影响对于管理与这些疾病相关的牙齿和骨骼表现至关重要。这篇综述表明,多学科治疗方法可以显着改善患有此类疾病的患者的口腔结局。临床医生应了解可能出现的特定牙齿异常,并考虑全面评估和个性化治疗计划。这些发现强调需要进一步研究解决这些异常的靶向疗法。
    Tooth eruption is an essential process for the development of the oral and maxillofacial system. Several inherited and acquired diseases might affect this tightly regulated process, resulting in premature, delayed, or even failed tooth eruption. The purpose of this article is to review the literature and the clinical parameters of metabolic bone diseases that affect tooth eruption. It examines the physiological aspects of tooth eruption and the pathophysiological changes induced by metabolic bone diseases, including changes in bone metabolism, density, and structure. The search strategy for this review included an electronic search in PubMed, Google Scholar, Medline, Scopus, and the Cochrane Library using the following keywords: \"metabolic bone diseases\", \"tooth eruption\", \"delayed tooth eruption\", and each reported disease in combination with \"tooth eruption disorders\", covering publications up to March 2024 and limited to English-language sources. Understanding the influence of metabolic bone diseases on tooth eruption is crucial for managing both dental and skeletal manifestations associated with these disorders. This review suggests that a multidisciplinary approach to treatment may significantly improve oral outcomes for patients suffering from such conditions. Clinicians should be aware of the specific dental abnormalities that may arise and consider comprehensive evaluations and individualized treatment plans. These findings underscore the need for further research into targeted therapies that address these abnormalities.
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  • 文章类型: Journal Article
    背景:全髋关节置换术(THA)后的大多数假体周围骨折是脆性骨折,使患者有资格诊断骨质疏松症。然而,目前尚不清楚有多少患者在受伤前被诊断出患有骨质疏松症或接受了适当的评估,诊断,受伤后的治疗。
    方法:我们确定了从2000年至2018年在一个机构使用模块化凹槽锥形柄治疗的171个温哥华B2(109)和B3(62)股骨假体周围骨折。患者平均年龄为75岁(范围,35至94),50%是女性,平均BMI为29(范围,17至60)。我们确定了患有骨质疏松症或骨质减少症的患者,骨折风险评估工具(FRAX),骨矿物质密度(BMD)测试,内分泌学会诊,和骨质疏松症药物。适合年龄的BMD测试定义为不迟于推荐年龄65(女性)或70岁(男性)后一年。平均随访时间为11年(范围,4至21)。
    结果:从站立高度坠落导致94%的骨折,根据定义,符合骨质疏松症定义事件。骨质疏松诊断的患病率从假体周围骨折前的20%增加到后的39%(P<0.001)。骨量减少的患病率从骨折前的13%上升到骨折后的24%(P<0.001)。任一诊断的患病率从骨折前的24%增加到骨折后的44%(P<0.001)。没有患者在骨折前记录FRAX评分,之后只有2%的人得分。骨折前BMD检测的患病率为21%,骨折后为22%(P=0.88)。在最后的后续行动结束时,只有16%的人接受了适合年龄的BMD检测.内分泌学咨询的患者比例从骨折前的6%增加到骨折后的25%(P<0.001)。双膦酸盐治疗的比例在骨折前为19%,在骨折后为25%(P=0.08)。
    结论:尽管THA后的大多数假体周围骨折是脆性骨折,使患者有资格进行骨质疏松症诊断,在诊断方面仍然存在重大差距,筛选,内分泌学随访,和治疗。像非关节成形术脆性骨折一样,假体周围骨折后需要一个系统的方法。
    BACKGROUND: Most periprosthetic fractures following total hip arthroplasty (THA) are fragility fractures that qualify patients for osteoporosis diagnoses. However, it remains unknown how many patients were diagnosed who had osteoporosis before injury or received the proper evaluation, diagnosis, and treatment after injury.
    METHODS: We identified 171 Vancouver B2 (109) and B3 (62) periprosthetic femur fractures treated with a modular fluted tapered stem from 2000 to 2018 at 1 institution. The mean patient age was 75 years (range, 35 to 94), 50% were women, and the mean body mass index was 29 (range, 17 to 60). We identified patients who had osteoporosis or osteopenia diagnoses, a fracture risk assessment tool (FRAX), bone mineral density (BMD) testing, an endocrinology consult, and osteoporosis medications. Age-appropriate BMD testing was defined as no later than 1 year after the recommended ages of 65 (women) or 70 years (men). The mean follow-up was 11 years (range, 4 to 21).
    RESULTS: Falls from standing height caused 94% of fractures and thus, by definition, qualified as osteoporosis-defining events. The prevalence of osteoporosis diagnosis increased from 20% before periprosthetic fracture to 39% after (P < .001). The prevalence of osteopenia diagnosis increased from 13% before the fracture to 24% after (P < .001). The prevalence of either diagnosis increased from 24% before fracture to 44% after (P < .001). No patients had documented FRAX scores before fracture, and only 2% had scores after. The prevalence of BMD testing was 21% before fracture and 22% after (P = .88). By the end of the final follow-up, only 16% had received age-appropriate BMD testing. The proportion of patients who had endocrinology consults increased from 6% before the fracture to 25% after (P < .001). The proportion on bisphosphonate therapy was 19% before fracture and 25% after (P = .08).
    CONCLUSIONS: Although most periprosthetic fractures following THA are fragility fractures that qualify patients for osteoporosis diagnoses, there remain major gaps in diagnosis, screening, endocrinology follow-up, and treatment. Like nonarthroplasty fragility fractures, a systematic approach is needed after periprosthetic fractures.
    METHODS: Level III, retrospective cohort study.
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  • 文章类型: Journal Article
    维生素D的合成和吸收在许多身体机能中起着至关重要的作用。然而,由于阳光照射不足和饮食不足等因素,缺陷仍然存在。研究强调了饮食等生活方式元素的重要性,阳光照射,和保持最佳维生素D水平的体力活动。旨在解决缺陷的策略强调在调整生活方式的同时进行补充,特别是在中东和北非(MENA)等阳光充足的地区。尽管阿拉伯世界充满阳光,维生素D缺乏仍然是一个普遍的问题。这个问题是由各种因素引起的,包括文化习俗,如覆盖大部分皮肤区域的传统服装,限制阳光照射,和环境因素,如空气污染,减少紫外线渗透。饮食习惯和生活方式的选择也有助于这种缺陷。应对持续的大流行需要集中力量提高认识。虽然有些人可能认识到维生素D缺乏引起的常见疾病,比如软骨病和软骨病,许多人仍然不知道与这种情况相关的更广泛的健康风险,包括非骨骼表现。此外,人们对这种激素的许多隐藏益处缺乏了解。因此,优先考虑深入研究这些方面的教育举措对于有效应对当前的健康危机至关重要。这篇文献综述旨在报道维生素缺乏症的骨骼和骨骼外后果,并简要讨论MENA等阳光充足地区矛盾的维生素D缺乏的原因。这是通过审查2000年1月至2024年1月之间发表的相关文章来完成的,这些文章来自PubMed等数据库,UpToDate,Scopus,和CINAHL,专注于英语语言文学和使用关键词,如“维生素D缺乏”和“外表现。\"
    The synthesis and absorption of Vitamin D play crucial roles in numerous bodily functions, yet deficiencies persist due to factors like insufficient sunlight exposure and dietary inadequacy. Research underscores the significance of lifestyle elements such as diet, sun exposure, and physical activity in maintaining optimal Vitamin D levels. Strategies aimed at tackling deficiencies emphasize supplementation alongside lifestyle adjustments, especially in regions with abundant sunlight like the Middle East and North Africa (MENA). Despite the abundance of sunshine in the Arab world, there remains a prevalent issue of Vitamin D deficiency. This problem arises from various factors, including cultural practices such as traditional clothing covering most skin areas, which limit sun exposure, and environmental factors like air pollution that reduce UV penetration. Dietary habits and lifestyle choices also contribute to this deficiency. Dealing with the ongoing pandemic requires a focused effort to enhance awareness. While some individuals may recognize common diseases caused by Vitamin D deficiency, such as rickets and osteomalacia, many remain unaware of the broader health risks associated with the condition, including non-skeletal manifestations. Additionally, there is a lack of understanding regarding the numerous hidden benefits of this hormone. Therefore, prioritizing educational initiatives that delve into these aspects is essential to effectively combat the current health crisis. This literature review aims to report both skeletal and extraskeletal consequences of hypovitaminosis and briefly discuss the cause of paradoxical vitamin D deficiency in sunny regions like the MENA. This was done by reviewing pertinent articles published between January 2000 and January 2024, sourced from databases such as PubMed, UpToDate, Scopus, and CINAHL, focusing exclusively on English language literature and using keywords such as \"Vitamin D deficiency\" and \"Extraskeletal manifestations.\"
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  • 文章类型: Journal Article
    肝细胞学探讨了肝功能与内分泌系统之间的复杂关系。慢性肝病如肝硬化可由于毒素积累和蛋白质合成中断而引起内分泌紊乱。尽管它很重要,评估肝硬化患者的内分泌问题经常被忽视。本文对流行病学进行了全面的回顾,病理生理学,诊断,和治疗肝硬化的内分泌紊乱。审查是使用PubMed/Medline进行的,EMBASE,和Scielo数据库,涵盖172篇文章。肝硬化与内分泌紊乱有关,包括糖尿病,低血糖,少肌症,甲状腺功能异常,低促性腺激素性性腺功能减退,骨病,肾上腺功能不全,生长激素功能障碍,和继发性醛固酮增多症。诊断糖尿病和检测低血糖的最佳工具是口服葡萄糖耐量试验和连续血糖监测系统,分别。肌肉减少症可以通过影像学和功能测试来评估,而其他内分泌疾病则使用激素测定和影像学研究进行评估。治疗方案包括二甲双胍,胰高血糖素样肽-1类似物,钠-葡萄糖协同转运蛋白-2抑制剂,和胰岛素,对糖尿病控制是有效和安全的。遵循既定的低血糖管理标准,激素替代疗法对于其他内分泌功能障碍通常是必要的。肝移植可以解决其中的一些问题。
    Hepatocrinology explores the intricate relationship between liver function and the endocrine system. Chronic liver diseases such as liver cirrhosis can cause endocrine disorders due to toxin accumulation and protein synthesis disruption. Despite its importance, assessing endocrine issues in cirrhotic patients is frequently neglected. This article provides a comprehensive review of the epidemiology, pathophysiology, diagnosis, and treatment of endocrine disturbances in liver cirrhosis. The review was conducted using the PubMed/Medline, EMBASE, and Scielo databases, encompassing 172 articles. Liver cirrhosis is associated with endocrine disturbances, including diabetes, hypoglycemia, sarcopenia, thyroid dysfunction, hypogonadotropic hypogonadism, bone disease, adrenal insufficiency, growth hormone dysfunction, and secondary hyperaldosteronism. The optimal tools for diagnosing diabetes and detecting hypoglycemia are the oral glucose tolerance test and continuous glucose monitoring system, respectively. Sarcopenia can be assessed through imaging and functional tests, while other endocrine disorders are evaluated using hormonal assays and imaging studies. Treatment options include metformin, glucagon-like peptide-1 analogs, sodium-glucose co-transporter-2 inhibitors, and insulin, which are effective and safe for diabetes control. Established standards are followed for managing hypoglycemia, and hormone replacement therapy is often necessary for other endocrine dysfunctions. Liver transplantation can address some of these problems.
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  • 文章类型: Journal Article
    背景:Paget骨病是一种引起骨畸形和骨质量损害的局灶性骨骼疾病。尽管无症状病例的患病率正在增加,疾病的进展可能导致无效的并发症,从而影响生活质量。在临床和治疗管理方面存在疑问,虽然抗吸收药物的有益作用,特别是双膦酸盐是已知的。然而,关于预防疾病并发症的随机对照试验提供的信息有限,因此来自代谢性骨病主要科学学会的专家小组之间关于治疗适应症的立场存在一定的差异。这个特遣部队,由意大利骨质疏松学会任命的专家代表组成,矿物质代谢和骨骼疾病以及意大利佩吉特骨病协会的成员,认为有必要为早期诊断和临床管理提供更具体和最新的适应症。
    方法:通过选定的关键问题,我们为该疾病的诊断和治疗提出了循证建议.在缺乏良好证据支持明确建议的情况下,从文献中获得的信息以及专家小组的专家意见被用来为临床实践提供建议。
    结论:对每个选定的关键问题提供了证据质量的描述和对陈述强度的支持。PDB的诊断应主要基于症状和典型的生化和放射学特征。虽然诊断时所有有症状的病例都必须接受治疗,在无症状的患者以及在存在生化复发的以前接受过治疗的患者中,关于治疗适应症的证据较少。然而,鉴于有效的静脉注射双膦酸盐如唑来膦酸盐的安全性和长期疗效,建议在诊断时治疗大多数病例,即使不是所有病例。
    BACKGROUND: Paget\'s disease of bone is a focal skeletal disorder causing bone deformities and impairing bone quality. Despite the prevalence of asymptomatic cases is increasing, the progression of the disease can lead to invalidating complications that compromise the quality of life. Doubts on clinical and therapeutic management aspects exist, although beneficial effects of antiresorptive drugs, particularly bisphosphonates are known. However, limited information is available from randomized controlled trials on the prevention of disease complications so that somewhat contrasting positions about treatment indications between expert panels from the main scientific societies of metabolic bone diseases exist. This task force, composed by expert representatives appointed by the Italian Society of Osteoporosis, Mineral Metabolism and Skeletal Diseases and members of the Italian Association of Paget\'s disease of bone, felt the necessity for more specific and up to date indications for an early diagnosis and clinical management.
    METHODS: Through selected key questions, we propose evidence-based recommendations for the diagnosis and treatment of the disease. In the lack of good evidence to support clear recommendations, available information from the literature together with expert opinion of the panel was used to provide suggestions for the clinical practice.
    CONCLUSIONS: Description of the evidence quality and support of the strength of the statements was provided on each of the selected key questions. The diagnosis of PDB should be mainly based on symptoms and the typical biochemical and radiological features. While treatment is mandatory to all the symptomatic cases at diagnosis, less evidence is available on treatment indications in asymptomatic as well as in previously treated patients in the presence of biochemical recurrence. However, given the safety and long-term efficacy of potent intravenous bisphosphonates such as zoledronate, a suggestion to treat most if not all cases at the time of diagnosis was released.
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  • 文章类型: Case Reports
    低磷血症通常发生在炎症性肠病(IBD)患者中,并可引起相当大的发病率。IBD的鉴别诊断包括营养原因和一些静脉内输注铁制剂引起的低磷酸盐血症。
    我们介绍了一名37岁男子患有活动性克罗恩病的病例,表现为行走困难,椎骨和跟骨骨折。他长期患有低磷酸盐血症。由于存在慢性腹泻和维生素D缺乏,首先考虑了低磷酸盐血症的营养原因;然而,对适当补充口服磷和维生素D的反应最小。然后考虑了铁输注引起的低磷酸盐血症,但是磷酸盐水平的最低点先于任何铁输注。因此,工作是针对不太常见的原因进行的。他最终被诊断出患有肿瘤诱导的骨软化症,由膝关节周围磷性间充质肿瘤分泌过多的成纤维细胞生长因子23(FGF23)引起。成功切除肿瘤后,他的症状和生化异常完全缓解。
    本案例说明了IBD患者低磷酸盐血症的研究方法。如果营养或铁输注引起的低磷酸盐血症的时程和对磷酸盐补充的反应不如预期,应该考虑不太常见的原因。
    UNASSIGNED: Hypophosphatemia occurs commonly in inflammatory bowel disease (IBD) patients and can cause considerable morbidity. The differential diagnoses in IBD include nutritional causes and hypophosphatemia induced by some formulations of intravenous iron infusions.
    UNASSIGNED: We present the case of a 37-year-old man with active Crohn\'s disease, presenting with difficulty walking and fractures of the vertebrae and calcaneus. He had long-standing hypophosphatemia. Nutritional causes for hypophosphatemia were considered in the first instance given the presence of chronic diarrhea and vitamin D deficiency; however, there was minimal response to appropriate supplementation with oral phosphorous and vitamin D. Iron infusion-induced hypophosphatemia was then considered, but the nadir phosphate level preceded any iron infusion. Therefore, work-up was undertaken for less common causes. He was ultimately diagnosed with tumor-induced osteomalacia, caused by excess fibroblast growth factor 23 (FGF23) secretion from a phosphaturic mesenchymal tumor about the knee. He had complete resolution of symptoms and biochemical abnormalities following successful resection of the tumor.
    UNASSIGNED: This case illustrates the approach to investigation of hypophosphatemia in IBD patients. If the time course and response to phosphate supplementation are not as expected for nutritional or iron infusion-induced hypophosphatemia, less common causes should be considered.
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  • 文章类型: Journal Article
    代谢性骨骼疾病涵盖了广泛的疾病,这些疾病共同改变了骨骼代谢,导致骨骼缺陷。这与发病率增加有关,残疾,和死亡率。代谢性骨病的病因与遗传因素有着密切的联系,TP53是与其相关的基因之一。TP53的单核苷酸多态性(SNP)Arg72Pro是与几种病理相关的遗传因素,包括癌症,中风,和骨质疏松症。这里,我们旨在分析TP53Arg72ProSNP对人源化Tp53Arg72Pro敲入小鼠骨量的影响。这项工作报告了TP53Arg72Pro多态性在骨骼微结构中的影响,OPG表达式,和凋亡骨状态。结果表明,TP53Arg72Pro多态性(Pro72-p53)的脯氨酸变体与骨组织恶化有关,较低的OPG/RANK比率,降低骨组织凋亡。总之,TP53Arg72Pro多态性可调节骨微结构,可能是一种遗传生物标志物,可用于鉴定代谢性骨改变风险增加的个体.
    Metabolic bone diseases cover a broad spectrum of disorders that share alterations in bone metabolism that lead to a defective skeleton, which is associated with increasing morbidity, disability, and mortality. There is a close connection between the etiology of metabolic bone diseases and genetic factors, with TP53 being one of the genes associated therewith. The single nucleotide polymorphism (SNP) Arg72Pro of TP53 is a genetic factor associated with several pathologies, including cancer, stroke, and osteoporosis. Here, we aim to analyze the influence of the TP53 Arg72Pro SNP on bone mass in humanized Tp53 Arg72Pro knock-in mice. This work reports on the influence of the TP53 Arg72Pro polymorphism in bone microarchitecture, OPG expression, and apoptosis bone status. The results show that the proline variant of the TP53 Arg72Pro polymorphism (Pro72-p53) is associated with deteriorated bone tissue, lower OPG/RANK ratio, and lower apoptosis in bone tissue. In conclusion, the TP53 Arg72Pro polymorphism modulates bone microarchitecture and may be a genetic biomarker that can be used to identify individuals with an increased risk of suffering metabolic bone alterations.
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  • 文章类型: Journal Article
    青少年型系统性红斑狼疮(JSLE)患者进入成年期的风险很高,与疾病相关的发病率,例如骨量减少和骨质疏松症。本研究旨在评估JSLE的临床特征,并分析这些患者的低骨密度(BMD)相关因素。
    包括在韩国一家医院诊断为JSLE的儿童和青少年。人口统计,临床,收集了实验室数据和糖皮质激素和改善疾病的抗风湿药物的使用情况。使用双能X线骨密度仪测量腰椎BMDZ评分,收集腰椎影像学数据。
    本研究共纳入29例JSLE患者。在这些病人中,7例腰椎Z评分为-2.0或更低,被指定为低BMD组.比较低BMD组和非低BMD组之间临床参数和治疗变量的差异。较高的累积糖皮质激素剂量,糖皮质激素暴露时间较长,和较高的累积羟氯喹剂量与低骨密度有关;主要因素是暴露时间。BMD和临床资料之间没有显著的相关性,SLE疾病活动,或骨代谢标志物。
    糖皮质激素暴露的持续时间,累积糖皮质激素剂量,和累积羟氯喹剂量是JSLE患者低骨密度的危险因素,主要因素是糖皮质激素暴露的持续时间。因此,应常规监测JSLE患者的低BMD和潜在的骨折风险,应考虑保留糖皮质激素的治疗方案。
    OBJECTIVE: Patients with juvenile-onset systemic lupus erythematosus (JSLE) are at a high risk of entering adulthood with disease-related morbidities like reduced bone mass and osteoporosis. This study aimed to evaluate the clinical characteristics of JSLE and to analyze the factors associated with low bone mineral density (BMD) in these patients.
    METHODS: Children and adolescents diagnosed with JSLE at a single institution in Korea were included. Demographic, clinical, and laboratory data as well as details about the use of glucocorticoids (GCs) and disease-modifying antirheumatic drugs were collected. The lumbar spine (LS) BMD z-score was measured using dual energy x-ray absorptiometry, and lateral thoracolumbar spine radiographs were collected.
    RESULTS: A total of 29 patients with JSLE were included in this study. Of these patients, 7 had a BMD z-score of -2.0 or lower and were designated as the low BMD group. The differences in the clinical parameters and treatment variables between the low BMD and non-low BMD groups were compared. Higher cumulative GC dose, longer GC exposure, and higher cumulative hydroxychloroquine (HCQ) dose were all associated with low BMD; among them, the main factor was the duration of GC exposure. There was no significant correlation between BMD and clinical profile, disease activity, or bone-metabolism markers.
    CONCLUSIONS: The duration of GC exposure, cumulative GC dose, and cumulative HCQ dose were risk factors for low BMD in patients with JSLE, with the main factor being the duration of GC exposure. Thus, patients with JSLE should be routinely monitored for low BMD and potential fracture risks, and GC-sparing treatment regimens should be considered.
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  • 文章类型: Case Reports
    非创伤性骨折(ATF)是一种脆性骨折亚型,偶尔会出现法医学问题。ATF被定义为裂缝,因为低能量机制通常被认为不能产生裂缝。“他们是一种被低估的疾病,流行病学变化。以前报道的ATF现象不仅发生在老年人中,而且在儿童中,年轻人,老年人,和动物。这项研究是一个简短的回顾性病例系列,探讨了三级护理大学医院的无创伤骨折。在两年的时间里,共发现7例ATF病例。然而,只有5人符合纳入标准。病理性骨折的局部原因(例如,转移)和老年人虐待或忽视被排除。病例临床概况的比较,断裂剖面,管理完成了。所有5例病例均为体弱的女性,因药物和多种疾病而产生严重的骨毒性负担。ATF的介绍包括典型(如疼痛)和非典型(如无痛,响亮的裂缝,和突然的赠品)症状学。一名ATF同时出现了原因不明的无菌热。3例骨折不止1例(骨折级联),确认并进行X光检查。除1例进行髋关节置换术外,所有病例均采用保守治疗。护理计划包括管理骨毒性多发病负担,专注于整个身体,不仅仅是骨折或骨头。该研究提供了有关ATF表现挑战的见解(如骨折急性期反应:成骨无菌热)。风险因素通常被认为是骨质疏松症,但它通常是系统性和多因素的。骨折警告标志的高风险可以帮助减少ATF的发生或骨折级联。检测到四个ATF类别,以帮助医疗保健系统识别高风险患者并提高医务人员的意识,家庭,和照顾者。未来需要对高危人群进行研究,以了解ATF知识差距,挑战,最好的治疗方法。
    Atraumatic fractures (ATFs) are a fragility fracture subtype with occasional medicolegal issues. ATFs are defined as fractures because of a \"low-energy mechanism that is usually considered incapable of producing a fracture.\" They are an underreported disorder, with epidemiological variations. ATF phenomena were previously reported not only in older adults, but also in children, young adults, older adults, and animals. This study is a short retrospective case series exploring atraumatic fractures in a tertiary care university hospital. Over a period of two years, a total of seven ATF cases were identified. However, only five fulfilled the inclusion criteria. Local causes of pathologic fractures (e.g., metastasis) and elder abuse or neglect were excluded. Comparison of the cases\' clinical profile, fracture profile, and management was done. All five cases were frail females with significant osteotoxic burdens from medications and multi-morbidities. ATF presentations included typical (as pain) and atypical (as painless, loud crack, and sudden giveaway) symptomatology. One ATF had a coincident unexplained aseptic fever. Three cases had more than one fracture (fracture cascade), confirmed and followed up by x-rays. All the cases were managed conservatively except for one case that underwent hip hemiarthroplasty. Plans of care included managing the osteotoxic multi-morbidities burden, focusing on the whole body, not only on the fracture or bone. The study provided insights about challenges in presentations of ATF (as the bone fracture acute phase reaction: osteogenic aseptic fever). Risk factors are classically assumed to be osteoporosis, but it is usually systemic and multifactorial. A high risk of fracture warning sign could help decrease ATF occurrence or fracture cascades. Four ATF categories were detected to help healthcare systems identify high-risk patients and raise awareness among medical staff, families, and caregivers. Future studies of the at-risk groups are needed to understand ATF knowledge gaps, challenges, and the best treatments.
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