Calciphylaxis

钙化
  • 文章类型: Journal Article
    角质病定义为钙盐在皮肤中的沉积。营养不良形式是最常见的,通常发生在与胶原病相关的慢性炎症过程中。治疗选择包括手术切除以及一些药物治疗。总的来说,已知治疗性干预措施的证据非常有限,缺乏有效的建议.静脉注射硫代硫酸钠已成功用于治疗钙化。在我们的案例系列中,5例营养不良性钙质沉着症患者每月连续五天接受至少6个周期的硫代硫酸钠静脉注射,单次剂量为12.5g和25.0g,分别。钙化病变的减少无法确定,但病情稳定。静脉注射硫代硫酸钠可以抵消皮肤钙质沉着的进展。成功使用皮下应用硫代硫酸钠,正如文献中所描述的,表明可以实现更高的皮肤生物利用度,以对钙质角质层产生溶解作用。据报道,高剂量硫代硫酸钠在治疗钙化中的功效进一步支持了这一点。
    Calcinosis cutis is defined as the deposition of calcium salts in the skin. The dystrophic form is the most common and usually occurs in chronic inflammatory processes associated with collagenoses. Therapeutic options include surgical excision as well as a few pharmacological treatments. Overall, the evidence for the known therapeutic interventions is very limited and there is a lack of valid recommendations. Intravenous sodium thiosulfate has been used successfully in the treatment of calciphylaxis. In our case series, five patients with dystrophic calcinosis cutis received intravenous sodium thiosulfate for at least six cycles on five consecutive days per month, with single doses of 12.5 g and 25.0 g, respectively. A reduction in the calcified lesions could not be proven with certainty, but stable disease conditions were achieved. Intravenous sodium thiosulfate may counteract the progression of calcinosis cutis. The successful use of epicutaneously applied sodium thiosulfate, as described in the literature, suggests that a higher cutaneous bioavailability can be achieved to exert a lytic effect on calcinosis cutis. This is further supported by the reported efficacy of high-dose sodium thiosulfate in the treatment of calciphylaxis.
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  • 文章类型: Case Reports
    角质病是皮肤的常见疾病,钙盐在皮下区域的积累,尽管有来自马哈拉施特拉邦农村地区的罕见病例报告。这种情况通常是无症状的,可能表现为单一生长或几种不同大小的生长。观察到其临床表现为结节或斑块,而不会对下层组织造成伤害。据报道,这种情况是创伤的继发表现,恶性肿瘤,和结缔组织疾病,并具有多因素的潜在病因。对包括医疗和外科手术的治疗的建议取决于病症的表现和严重程度。最终决定可以基于从诸如细针抽吸和放射成像的诊断模态获得的结果。这是一名35岁的男性,在过去两年中左脚肿胀,没有任何相关病史。患者通过手术切口管理,恢复良好。
    Calcinosis cutis is a common ailment of the skin, caused by the accumulation of calcium salts in the subcutaneous regions, though there are rare case reports from rural Maharashtra region. This condition is usually asymptomatic and might manifest as a single growth or several different-sized growths. Its clinical presentation is observed as nodules or plaques without causing injury to underlying tissues. The condition is reported to be a secondary presentation to trauma, malignancies, and connective tissue diseases and has multifactorial underlying etiologies. Recommendation for treatments including both medical and surgical procedures is contingent upon the manifestation and severity of the condition. The final decision can be based on the results obtained from diagnostic modalities like fine needle aspiration and radiological imaging. This is the case of a 35-year-old male with a swelling on his left foot for the past two years without any associated medical history. The patient was managed by surgical incision and had a good recovery.
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  • 文章类型: Journal Article
    背景:从历史上看,钙中毒患者经历过诊断挑战和高发病率,然而,随着时间的推移,有限的数据可用于检查这些特征。
    目的:主要目标是a)调查与钙化延迟诊断相关的因素和b)评估发病率结果。次要目标是提供最新的死亡率。
    方法:对2006年1月1日至2022年12月31日诊断为钙化的302例成年患者进行回顾性分析。进行单变量和多变量统计分析。
    结果:非肾源性钙化(p=0.0004)和手指受累(p=0.0001)与诊断延迟增加显著相关,而手臂(p=0.01)和生殖器(p=0.022)的受累导致诊断天数减少。几乎所有生殖器患者,手指,或脚趾受累有肾源性疾病。每位患者的并发症数量随着时间的推移而减少,尤其是伤口感染(p=0.028),病变数量增加(p=0.012),和反复住院(p=0.020)。更新后的1年死亡率分别为36.70%和30.77%的肾性和非肾性钙化,分别。
    结论:限制包括回顾性性质和来自单一机构的数据。
    结论:诊断延迟,特别是在非肾源性钙化中,每位患者的并发症随着时间的推移而减少,强调持续意识对加快诊断的重要性。近年来死亡率持续改善。
    BACKGROUND: Calciphylaxis patients historically have experienced diagnostic challenges and high morbidity; however limited data is available examining these characteristics over time.
    OBJECTIVE: The primary goals were to a) investigate factors associated with diagnostic delay of calciphylaxis and b) assess morbidity outcomes. The secondary goal was to provide updated mortality rates.
    METHODS: A retrospective review of 302 adult patients diagnosed with calciphylaxis between January 1, 2006 and December 31, 2022 was conducted. Univariate and multivariate statistical analyses were performed.
    RESULTS: Nonnephrogenic calciphylaxis (P = .0004) and involvement of the fingers (P = .0001) were significantly associated with an increased diagnostic delay, whereas involvement of the arms (P = .01) and genitalia (P = .022) resulted in fewer days to diagnosis. Almost all patients with genitalia, finger, or toe involvement had nephrogenic disease. The number of complications per patient decreased with time, especially for wound infections (P = .028), increase in lesion number (P = .012), and recurrent hospitalizations (P = .020). Updated 1-year mortality rates were 36.70% and 30.77% for nephrogenic and nonnephrogenic calciphylaxis, respectively.
    CONCLUSIONS: Limitations include the retrospective nature and data from a single institution.
    CONCLUSIONS: Diagnostic delay, particularly in nonnephrogenic calciphylaxis, and complications per patient decreased with time, highlighting the importance of continued awareness to expedite diagnosis. Mortality rates have continued to improve in recent years.
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  • 文章类型: Case Reports
    钙化预防是一种引起缺血性皮肤坏死的疾病,通常与终末期肾病或接受透析的患者有关。在没有终末期肾病的患者中很少发生,和治疗选择是有限的。该病例报告描述了无终末期肾脏疾病或透析史的钙化患者。用硫代硫酸钠处理,第一行选项,由于代谢紊乱不得不停止,限制愈合过程。这种罕见疾病的诊断和治疗对于防止可能导致的进一步并发症很重要。
    Calciphylaxis is a disorder causing ischemic skin necrosis, typically associated with end-stage renal disease or those receiving dialysis. Occurrence is rare in those without end-stage renal disease, and treatment options are limited. This case report describes a patient with calciphylaxis without end-stage renal disease or history of dialysis. Treatment with sodium thiosulfate, a first line option, had to be stopped due to metabolic derangements, limiting the healing process. Diagnosis and treatment of this rare disorder are important to prevent further complications that may result.
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  • 文章类型: Journal Article
    钙敏感症是一种罕见的与显著的发病率和死亡率相关的疾病。数据登记处是罕见疾病的宝贵信息来源。我们回顾了澳大利亚和新西兰透析和移植登记处(ANZDATA)中记录的钙化预防病例,并评估了这种情况的关联和结果。
    获得了2019年至2022年期间澳大利亚和新西兰接受肾脏替代疗法(KRT)的所有钙化病例的数据。将该队列与2019年至2022年接受KRT但无钙化发作的所有患者进行比较。Cox比例风险回归包括钙化发作的时变协变量,用于死亡率,模型仅限于透析患者。
    从2019年到2022年,有333例患者出现钙化事件。透析患者的总发生率为每1000例透析患者年4.5(4.1-5.1)次。中位年龄为63岁(四分位数范围[IQR]:55-73岁),54%是女性,66%有糖尿病,59%的人肥胖(体重指数[BMI]≥30kg/m2),77%的人正在接受血液透析(HD)治疗。与没有钙化的患者相比(n=46,526),有钙化倾向的患者更有可能年龄较大,女性,患有糖尿病,更大的BMI,冠状动脉,和外周血管疾病。KRT开始后,钙化的中位时间为3.2年(IQR:0.9-6.7)。一半的钙化患者在诊断后12个月死亡。钙离子透析<1年和发作后1至4年的患者死亡率的调整后风险比(HR)为5.8(4.9-6.9)和1.5(1.0-2.1),分别与没有钙化的透析患者进行比较。
    钙化激素病是一种罕见但危及生命的疾病,在KRT患者诊断后12个月内死亡负担最大。
    UNASSIGNED: Calciphylaxis is a rare disorder associated with significant morbidity and mortality. Data registries are an invaluable source of information for rare diseases. We reviewed cases of calciphylaxis recorded in the Australia and New Zealand Dialysis and Transplant Registry (ANZDATA) and evaluated associations and outcomes of this condition.
    UNASSIGNED: Data was obtained on all cases of calciphylaxis reported between 2019 and 2022 in Australian and New Zealand patients on kidney replacement therapy (KRT). This cohort was compared to all patients in the registry who received KRT from 2019 to 2022 without an episode of calciphylaxis. Cox proportional hazards regression including a time-varying covariate for calciphylaxis episode was conducted for mortality with models restricted to patients on dialysis only.
    UNASSIGNED: From 2019 to 2022, 333 patients had calciphylaxis episodes reported. Overall incidence rate for patients on dialysis was 4.5 (4.1-5.1) episodes per 1000 patient-years on dialysis. Median age was 63 (interquartile range [IQR]: 55-73) years, 54% were female, 66% had diabetes, 59% were obese (body mass index [BMI] ≥ 30 kg/m2) and 77% were receiving hemodialysis (HD) treatment. Compared to patients without calciphylaxis (n = 46,526), patients with calciphylaxis were more likely to be older, female, and have diabetes, greater BMI, coronary artery, and peripheral vascular disease. The median time to calciphylaxis was 3.2 (IQR: 0.9-6.7) years after KRT commencement. Half of the patients with calciphylaxis died by 12 months from diagnosis. Adjusted hazard ratio (HR) of mortality for patients on dialysis with calciphylaxis <1 year and 1 to 4 years after an episode was 5.8 (4.9-6.9) and 1.5 (1.0-2.1), respectively compared to patients on dialysis without calciphylaxis.
    UNASSIGNED: Calciphylaxis is a rare but life-threatening condition in people on KRT with the greatest mortality burden within 12 months of diagnosis.
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  • 文章类型: Case Reports
    钙化是一种独特的医学病症,其特征是患者皮肤中真皮和皮下脂肪组织水平的小动脉和软组织的中间层钙化。钙化的进展速度很快,从血流量减少开始,导致皮肤缺血性变化,可表现为疼痛的皮肤红斑结节或斑块,后来表现为皮肤溃疡。大多数受钙化影响的患者有易感的合并症,如终末期肾病,长期血液透析和钙电解质异常,磷酸盐,甲状旁腺激素水平.本报告介绍了一名72岁的血液透析女性患者发生钙化的情况。早期预后的方法(早期诊断的方法),包括临床表现,危险因素,成像技术,和实验室调查,正在讨论。鉴于在开始血液透析的仅仅三个月内就出现了钙化,因此该病例尤其值得注意。在大多数患者中,时间线明显短于通常观察到的时间段。(本报告中详细介绍的病例概述了仅接受血液透析三个月的患者中钙化的快速发作。)这位患有早发性钙化病的患者强调了钙化病的不可预测性质,即使在血液透析的初始阶段,也需要提高临床警惕性。
    Calciphylaxis is a unique medical condition characterized by calcification of the medial layer of arterioles and soft tissues in a patient\'s skin at the level of the dermis and subcutaneous adipose tissue. The rate of progression of calciphylaxis is rapid, starting with a reduction of blood flow that leads to ischemic changes in the skin that can manifest as painful cutaneous erythematous nodules or plaques and later as skin ulceration. The majority of patients affected by calciphylaxis have predisposing comorbidities such as end-stage renal disease with a long history of hemodialysis and electrolyte abnormalities in calcium, phosphate, and parathyroid hormone levels. This report presents the case of a 72-year-old female patient on hemodialysis who developed calciphylaxis. The methods for early prognosis (the methods of early diagnosis), including clinical presentation, risk factors, imaging techniques, and laboratory investigations, are discussed. The presented case is particularly noteworthy given the onset of calciphylaxis within a mere three months of initiating hemodialysis, a timeline significantly shorter than the typically observed period in most patients. (The case detailed in this report outlines the rapid onset of calciphylaxis in a patient who was receiving hemodialysis for only three months.) This patient with early-onset calciphylaxis highlights the unpredictable nature of calciphylaxis and the need for increased clinical vigilance even in the initial stages of hemodialysis.
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  • DOI:
    文章类型: English Abstract
    BACKGROUND: Calciphylaxis is a serious vascular disorder characterized by calcification of tunica media, intimal hyperplasia, thrombosis, and skin necrosis. It was described in patients with renal failure (UC), although it can occur in its absence (NUC). Its risk factors are under study and its diagnosis can be complex. Over a decade ago, its mortality was estimated at 60-80%. Recent studies indicate that it has decreased (40%).
    METHODS: A retrospective study was carried out in the period between January 1, 2011 and December 31, 2019. The past medical record, clinical characteristics, laboratory and histopathological findings, and evolution of all patients with calciphylaxis evaluated at the Hospital Italiano de Buenos Aires were reviewed.
    RESULTS: Thirty-nine patients were included. Sixtyone percent were men and 39% were NUC cases. Eightytwo percent had arterial hypertension, 66% obesity and 46% diabetes. Of those, 49% received coumarin anticoagulants. All patients with NUC and 75% with UC presented ulcers with necrosis, located more frequently on the legs. In 72% of the cases the histological diagnosis was made with one biopsy. In all the treatment was multimodal and mortality at one year was 42%.
    CONCLUSIONS: We observed a high proportion of patients with NUC, in relation to what is reported in the literature, and that half received vitamin K antagonists. The histological diagnosis was made with one biopsy in most of the cases, as the surgical technique for taking the sample, the Von Kossa staining and the evaluation by an expert pathologist were the key of it.
    Introducción: La calcifilaxis es un trastorno vascular grave caracterizado por depósito de calcio en túnica media arteriolar, trombosis y necrosis cutánea. Se describió en pacientes con insuficiencia renal (CU), aunque puede producirse en su ausencia (CNU). Sus factores de riesgo están en estudio y su diagnóstico puede ser complejo. Su mortalidad se estimaba en 60-80%, aunque trabajos recientes indican que ha disminuido (40%). Métodos: Estudio retrospectivo entre el 1/1/2011 y el 31/12/2019. Se revisaron los antecedentes, las características clínicas, los hallazgos de laboratorio e histopatológicos, y la evolución de todos los pacientes con diagnóstico de calcifilaxis evaluados en el Hospital Italiano de Buenos Aires. Resultados: Se incluyeron 39 pacientes. El 61.5% (24) eran hombres y 38.5% (15) fueron casos de CNU. De éstos, 82% presentaba hipertensión arterial, 66% obesidad y 46% diabetes. El 49% recibía anticoagulantes dicumarínicos. Todos los pacientes con CNU y 75% con CU presentaron úlceras de fondo necrótico, localizadas con mayor frecuencia en las piernas. En 72% de los casos el diagnóstico histológico se efectuó con una toma de biopsia. En todos, el tratamiento fue multimodal y la mortalidad al año fue de 42%. Conclusión: Observamos una elevada proporción de pacientes con CNU, en relación con lo comunicado en la literatura, y la mitad recibía anticoagulantes dicumarínicos. El diagnóstico histológico se efectuó por biopsia en la mayor parte de los casos, para lo cual la toma quirúrgica de la muestra, la tinción con Von Kossa y la evaluación por un patólogo experto fueron claves.
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  • 文章类型: Letter
    暂无摘要。
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  • 文章类型: Journal Article
    缺乏对钙化患者进行肾脏替代治疗的指南,通常导致难以最佳治疗这些患者。一名接受血液透析的60岁男性退伍军人表现为左下肢钙化和顽固性疼痛。他的病情因慢性背痛而变得复杂,长期阿片类药物治疗,和心理创伤史。他最终被转移到钙化治疗中心,但由于败血症和血流动力学不稳定而无法耐受进一步的治疗。他被转为采取安慰措施,并在医院死亡。即使在资源充足的三级医疗中心,解决复杂的疼痛生理和复杂的创伤也具有挑战性。尽管有钙化预防疗法和创伤知情护理,在这一患者人群中,痛苦和死亡率仍然很高。在这个群体中有很多工作要做,特别是在考虑过去的创伤经历对医疗保健参与和疼痛管理的影响时。
    Guidelines are lacking for patients with calciphylaxis on renal replacement therapy, often leading to difficulty optimally treating these patients. A 60-year-old male veteran receiving hemodialysis presented with calciphylaxis of the left lower extremity and intractable pain. His condition was complicated by chronic back pain, long-term opioid therapy, and psychological trauma history. He was ultimately transferred to a calciphylaxis treatment center but was unable to tolerate further treatments due to sepsis and hemodynamic instability. He was transitioned to comfort measures and died in the hospital. Addressing complicated pain physiologies and complex trauma is challenging even in well-resourced tertiary medical centers. Despite the availability of calciphylaxis therapies and trauma-informed care, there remains a high rate of suffering and mortality in this patient population. There is much work to be done in this cohort, particularly when considering the implications of past traumatic experiences on health care engagement and pain management.
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  • 文章类型: Journal Article
    非骨性异常通常在骨扫描中看到,并且可能与血管中的各种病理有关,感染,和炎症病因。骨扫描上的弥漫性软组织放射性示踪剂摄取通常归因于肾脏或代谢紊乱。钙化是钙在小血管中的沉积,皮肤,和其他器官导致血管阻塞和皮肤坏死。这是一种病理生理学未知的罕见疾病。钙化的诊断具有挑战性,需要跨学科的方法,包括临床发现,实验室结果,医学成像,还有皮肤活检.早期诊断很重要,因为该疾病与高发病率和死亡率相关。这篇综述文章的目的是强调骨闪烁显像在评估钙化中的作用,并将其与其他成像方式和组织病理学联系起来。
    Nonosseous abnormalities are often seen on bone scans and can be related to a wide variety of pathology ranging across vascular, infection, and inflammatory etiology. Diffuse soft tissue radiotracer uptake on bone scans is typically attributed to renal or metabolic derangements. Calciphylaxis is the deposition of calcium in small blood vessels, skin, and other organs leading to vascular obstruction and skin necrosis. It is a rare disorder with unknown pathophysiology. Diagnosis of calciphylaxis is challenging and requires an interdisciplinary approach including clinical findings, laboratory results, medical imaging, and skin biopsy. An early diagnosis is important as the disease is associated with high morbidity and mortality. The purpose of this review article is to highlight the role of bone scintigraphy in the evaluation of calciphylaxis and to correlate the findings with other imaging modalities and histopathology.
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