renal osteodystrophy

肾性骨营养不良
  • 文章类型: Case Reports
    背景:该病例报告强调了一例因终末期肾病(ESRD)而进行透析的肾性骨营养不良和继发性甲状旁腺功能亢进(SHPTH)患者的被忽视的股骨颈骨折的成功治疗,从而为医学文献做出了贡献。它强调了甲状旁腺切除术(PTX)在恢复骨密度(BMD)和促进骨折愈合方面的功效,解决ESRD患者的严重并发症。
    方法:一名36岁女性,患有肾性骨病,因ESRD进行透析,有左髌腱断裂病史,右跟腱撕脱骨折.持续的右髋部疼痛导致发现被忽视的右股骨颈骨折,由于患者的复杂病史,最初被忽略。跟腱修复后两个月,患者接受PTX治疗难治性SHPTH.术后过程包括康复和负重锻炼。值得注意的是,接骨术2年后,射线照相评估表明假体周围骨折的牢固结合和BMD的显着改善,展示治疗方法的疗效。
    结论:PTX,结合适当的康复,对于改善ESRD伴SHPTH患者的BMD和骨折愈合至关重要。
    BACKGROUND: This case report contributes to the medical literature by highlighting the successful management of a neglected femoral neck fracture in a patient with renal osteodystrophy and secondary hyperparathyroidism (SHPTH) who was on dialysis due to end-stage renal disease (ESRD). It underscores the efficacy of parathyroidectomy (PTX) in restoring bone mineral density (BMD) and promoting fracture healing, addressing a significant complication in ESRD patients.
    METHODS: A 36-year-old female with renal osteodystrophy and on dialysis due to ESRD presented with a history of left patellar tendon rupture and later, a right achilles tendon avulsion fracture. Persistent right hip pain led to the discovery of a neglected right femoral neck fracture, which was initially overlooked due to the patient\'s complex medical history. Two months post-achilles tendon repair, the patient underwent PTX to manage the refractory SHPTH. The postoperative course included rehabilitation and weight-bearing exercises. Remarkably, 2 years after osteosynthesis, radiographic assessments indicated a solid union of the periprothesis fracture and significant improvement in BMD, showcasing the efficacy of the treatment approach.
    CONCLUSIONS: PTX, combined with appropriate rehabilitation, is crucial for improving BMD and fracture healing in ESRD patients with SHPTH.
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  • 文章类型: Case Reports
    Sagliker综合征(SS)是一种罕见但独特的肾性骨营养不良形式,与慢性肾脏疾病(CKD)患者管理不善的继发性甲状旁腺功能亢进(SHPT)相关。我们介绍了一个28岁的男性,患有终末期CKD的血液透析10年,表现出进行性面部畸形和颌面部骨痛。体格检查显示上颌骨和下颌骨的双侧扩张以及面部不对称。放射学发现包括颌面部骨弥漫性骨增厚和多房性囊肿,虽然实验室检查显示钙水平下降和甲状旁腺激素升高,确认SHPT。尽管涉及肾病学的多学科管理,内分泌学,和颌面外科,患者病情恶化,表现为社区获得性肺炎,导致心肺骤停和死亡。该病例强调了治疗CKD严重HPT的挑战,并强调了早期评估和综合多学科护理对预防不可逆并发症的重要性。
    Sagliker syndrome (SS) is a rare but distinctive form of renal osteodystrophy associated with poorly managed secondary hyperparathyroidism (SHPT) in patients with chronic kidney disease (CKD). We present a case of a 28-year-old male with end-stage CKD on hemodialysis for 10 years, who exhibited progressive facial deformities and maxillofacial bone pain. Physical examination revealed bilateral expansion of the maxillary and mandibular bones and facial asymmetry. Radiological findings included diffuse bone thickening and multilocular cysts in the maxillofacial bones, while laboratory tests showed decreased levels of calcium and elevated parathyroid hormone, confirming SHPT. Despite multidisciplinary management involving nephrology, endocrinology, and maxillofacial surgery, the patient\'s condition deteriorated and he manifested community-acquired pneumonia leading to cardiopulmonary arrest and death. This case underscores the challenges in managing severe HPT in CKD and emphasizes the importance of early assessment and comprehensive multidisciplinary care to prevent irreversible complications.
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  • 文章类型: Journal Article
    慢性肾脏病(CKD)已成为全球10%以上人口的主要非传染性疾病之一。骨和矿物质疾病是CKD患者的常见并发症,导致生活质量差。高骨折风险,发病率和心血管死亡率增加。根据肾脏疾病:改善全球结果,肾性骨营养不良是指骨活检中发现的骨形态的变化,而CKD-矿物质和骨骼疾病(CKD-MBD)定义了包括生化和激素改变在内的复杂紊乱,骨和矿物质代谢紊乱和骨外钙化。因此,CKD-MBD的管理应侧重于上述参数,包括高磷酸盐血症的治疗,低钙血症,PTH和维生素D水平异常。关于骨脆性骨折,骨质疏松症和肾性骨营养不良,构成CKD-MBD的骨成分,抗骨质疏松药物是治疗的主要手段。然而,彻底阐明CKD-MBD发病机制对于理想的个性化治疗方法至关重要.在本文中,我们根据现有文献对CKD-MBD的病理学和治疗进行综述,并特别关注最新进展.
    Chronic kidney disease (CKD) has emerged as one of the leading noncommunicable diseases affecting >10% of the population worldwide. Bone and mineral disorders are a common complication among patients with CKD resulting in a poor life quality, high fracture risk, increased morbidity and cardiovascular mortality. According to Kidney Disease: Improving Global Outcomes, renal osteodystrophy refers to changes in bone morphology found in bone biopsy, whereas CKD-mineral and bone disorder (CKD-MBD) defines a complex of disturbances including biochemical and hormonal alterations, disorders of bone and mineral metabolism and extraskeletal calcification. As a result, the management of CKD-MBD should focus on the aforementioned parameters, including the treatment of hyperphosphatemia, hypocalcemia, abnormal PTH and vitamin D levels. Regarding the bone fragility fractures, osteoporosis and renal osteodystrophy, which constitute the bone component of CKD-MBD, anti-osteoporotic agents constitute the mainstay of treatment. However, a thorough elucidation of the CKD-MBD pathogenesis is crucial for the ideal personalized treatment approach. In this paper, we review the pathology and management of CKD-MBD based on the current literature with special attention to recent advances.
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  • 文章类型: Journal Article
    骨化三醇和拟钙剂用于治疗慢性肾病(CKD)继发的甲状旁腺功能亢进。骨化三醇给药和随后的血清钙浓度增加降低甲状旁腺激素(PTH)水平,这应该会减少骨骼重塑。我们以前报道过,当保持给定的PTH浓度时,拟钙剂的加入与骨细胞活性增加有关。应在肾性骨营养不良的动物模型中评估骨化三醇给药是否在PTH保持恒定的同时影响骨细胞活性。本研究的目的是比较CKDPTH钳制大鼠骨化三醇和拟钙剂给药的骨作用。结果表明,以诱导PTH分泌相似减少的剂量施用骨化三醇和拟钙剂对患有继发性甲状旁腺功能亢进的5/6肾切除(Nx)大鼠和患有钳夹PTH的Nx大鼠的成骨细胞活性产生了不同的影响。值得注意的是,在两种大鼠模型中,骨化三醇的给药降低了成骨细胞的活性,而拟钙剂增加骨细胞活性。体外,骨化三醇补充抑制β-连环蛋白的核易位和减少增殖,成骨,以及分化为成骨细胞的间充质干细胞的矿化。总之,除了骨化三醇和拟钙剂在甲状旁腺水平的作用,这些治疗对骨细胞有特定的影响,而与PTH水平无关.
    Calcitriol and calcimimetics are used to treat hyperparathyroidism secondary to chronic kidney disease (CKD). Calcitriol administration and the subsequent increase in serum calcium concentration decrease parathyroid hormone (PTH) levels, which should reduce bone remodeling. We have previously reported that, when maintaining a given concentration of PTH, the addition of calcimimetics is associated with an increased bone cell activity. Whether calcitriol administration affects bone cell activity while PTH is maintained constant should be evaluated in an animal model of renal osteodystrophy. The aim of the present study was to compare in CKD PTH-clamped rats the bone effects of calcitriol and calcimimetic administration. The results show that the administration of calcitriol and calcimimetic at doses that induced a similar reduction in PTH secretion produced dissimilar effects on osteoblast activity in 5/6 nephrectomized (Nx) rats with secondary hyperparathyroidism and in Nx rats with clamped PTH. Remarkably, in both rat models, the administration of calcitriol decreased osteoblastic activity, whereas calcimimetic increased bone cell activity. In vitro, calcitriol supplementation inhibited nuclear translocation of β-catenin and reduced proliferation, osteogenesis, and mineralization in mesenchymal stem cells differentiated into osteoblasts. In conclusion, besides the action of calcitriol and calcimimetics at parathyroid level, these treatments have specific effects on bone cells that are independent of the PTH level.
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  • 文章类型: Journal Article
    这项研究调查了在接受血液透析的慢性肾脏病(CKD)患者中,动态18F-NaFPET/CT的扫描时间缩短后得出的Ki-Patlak是否可以提供与更长扫描相当的预测准确性。27例慢性血液透析患者,在2021年12月至2023年8月期间共纳入42次扫描.动态18F-NaFPET/CT扫描,持续60-90分钟,在注射后立即获得,覆盖12岁中期胸椎至骨盆区域。对腰椎(L1-L4)和两个前髂峰在15、30、45、60和90分钟的骨时间-活动曲线进行了Ki-Patlak分析。使用Spearman的等级相关性(rs)和类间相关系数来评估Ki-Patlak在缩短扫描时间和标准扫描时间之间的相关性和一致性。测试了骨特异性碱性磷酸酶(BsAP)和抗酒石酸酸性磷酸酶同工型5b(TRAP5b)与个体Ki-Patlak的相关性。在腰椎(rs=0.858,p<0.001)和髂前区域(rs=0.850,p<0.001)的缩短的30分钟扫描和更长的60-90分钟扫描的Ki-Patlak值之间观察到了很强的相关性和良好的一致性。BsAP和Ki-Patlak之间的相关性较弱,统计学上无统计学意义。这一发现表明,提议的缩短的动态18F-NaFPET/CT扫描可有效评估接受血液透析的CKD患者的骨代谢通量。为骨转换预测提供了一种非侵入性的替代方法。
    This study investigated whether Ki-Patlak derived from a shortened scan time for dynamic 18F-NaF PET/CT in chronic kidney disease (CKD) patients undergoing hemodialysis can provide predictive accuracy comparable to that obtained from a longer scan. Twenty-seven patients on chronic hemodialysis, involving a total of 42 scans between December 2021 and August 2023 were recruited. Dynamic 18F-NaF PET/CT scans, lasting 60-90 min, were immediately acquired post-injection, covering the mid-twelfth thoracic vertebra to the pelvis region. Ki-Patlak analysis was performed on bone time-activity curves at 15, 30, 45, 60, and 90 min in the lumbar spine (L1-L4) and both anterior iliac crests. Spearman\'s rank correlation (rs) and interclass correlation coefficient were used to assess the correlation and agreement of Ki-Patlak between shortened and standard scan times. Bone-specific alkaline phosphatase (BsAP) and tartrate-resistant acid phosphatase isoform 5b (TRAP5b) were tested for their correlation with individual Ki-Patlak. Strong correlations and good agreement were observed between Ki-Patlak values from shortened 30-min scans and longer 60-90-min scans in both lumbar spine (rs = 0.858, p < 0.001) and anterior iliac crest regions (rs = 0.850, p < 0.001). The correlation between BsAP and Ki-Patlak in the anterior iliac crests was weak and statistically insignificant. This finding suggests that a proposed shortened dynamic 18F-NaF PET/CT scan is effective in assessing bone metabolic flux in CKD patients undergoing hemodialysis, offering a non-invasive alternative approach for bone turnover prediction.
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  • 文章类型: Journal Article
    慢性肾脏病(CKD)患者的骨折风险增加了几倍。尽管发病率很高,相关的发病率过高和过早死亡,CKD的骨脆性,或CKD相关的骨质疏松症,仍然是一个盲点在肾脏病与巨大的治疗差距。定义骨表型是在患者水平上适当治疗CKD相关骨质疏松症的先决条件。在本次审查中,我们建议10个实用的“提示和技巧”来评估CKD患者的骨骼健康。我们描述了临床,生物化学,和骨骼健康的放射学评估,以及可用诊断的优点和局限性。骨活检,诊断肾骨疾病的黄金标准,是侵入性的,不能广泛使用;虽然在复杂的情况下很有用,我们不认为它是CKD相关骨质疏松患者骨评估的重要组成部分.此外,我们主张在当地部署多学科专家团队,国家,和潜在的国际水平。最后,我们解决了诊断中的知识差距,特别是早期检测,适当的“实时”监测这一高度脆弱人群的骨骼健康,和新兴的诊断工具,目前主要用于研究,这可能是在临床实践的地平线上。
    Patients with chronic kidney disease (CKD) experience a several-fold increased risk of fracture. Despite the high incidence and the associated excess morbidity and premature mortality, bone fragility in CKD, or CKD-associated osteoporosis, remains a blind spot in nephrology with an immense treatment gap. Defining the bone phenotype is a prerequisite for the appropriate therapy of CKD-associated osteoporosis at the patient level. In the present review, we suggest 10 practical \'tips and tricks\' for the assessment of bone health in patients with CKD. We describe the clinical, biochemical, and radiological evaluation of bone health, alongside the benefits and limitations of the available diagnostics. A bone biopsy, the gold standard for diagnosing renal bone disease, is invasive and not widely available; although useful in complex cases, we do not consider it an essential component of bone assessment in patients with CKD-associated osteoporosis. Furthermore, we advocate for the deployment of multidisciplinary expert teams at local, national, and potentially international level. Finally, we address the knowledge gaps in the diagnosis, particularly early detection, appropriate \"real-time\" monitoring of bone health in this highly vulnerable population, and emerging diagnostic tools, currently primarily used in research, that may be on the horizon of clinical practice.
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  • 文章类型: Case Reports
    自发性股骨颈骨折是罕见的,尤其是当它们在双边发生时。肾性骨营养不良是这些骨折的原因之一,应牢记。我们报告了一例年轻女性,该女性患有双侧髋部疼痛,并因肾性骨营养不良而发现双侧股骨颈骨折。这是首次出现未确诊的终末期肾病。此病例报告旨在强调调查年轻患者中这些罕见骨折的原因并讨论可用的手术选择的重要性。
    一名19岁女性主诉双侧髋部疼痛。在体检时,两个大腿触诊时都有压痛。她的检查对贫血很重要,高水平的肌酐,低钙血症,碱性磷酸酶升高,和甲状旁腺激素.骨盆X光片显示双侧股骨颈骨折。考虑到她很小的年龄,她的代谢紊乱,为了避免让她接受大手术,我们用两侧三颗空心螺钉固定治疗她的骨折。我们的目的是报告这种情况,因为这是一个非常年轻的患者中以前未发现的5期慢性肾脏疾病(CKD)的不寻常表现。
    CKD引起的肾性骨营养不良可表现为自发性双侧股骨颈骨折。医生应该高度怀疑这种情况,不要错过具有多种后遗症的慢性疾病。此外,这些骨折有很高的并发症和死亡风险,所以应该及时解决。
    UNASSIGNED: Spontaneous femur neck fracture is rare, especially when they occur bilaterally. Renal osteodystrophy is among the causes of these fractures that should be kept in mind. We report a case of a young female who presented with bilateral hip pain and was found to have bilateral femur neck fracture due to renal osteodystrophy. This was the first presentation of an undiagnosed end-stage kidney disease. This case report aims to highlight the importance of investigating the cause of these rare fractures in young patients and discuss available surgical options.
    UNASSIGNED: A 19-year-old female presented complaining of bilateral hip pain. On physical examination, there was tenderness on palpation of both thighs. Her workup was significant for anemia, a high level of creatinine, hypocalcemia, elevated alkaline phosphatase, and parathyroid hormone. A pelvis radiograph showed bilateral femur neck fracture. Considering her very young age, the metabolic derangements she had and to avoid exposing her to a major surgery, we treated her fractures by fixation using three cannulated screws on each side. We aimed to report this case as it is an unusual presentation of a previously undetected stage 5 chronic kidney disease (CKD) in a very young patient.
    UNASSIGNED: Renal osteodystrophy due to CKD can present with spontaneous bilateral femur neck fracture. Physicians should have a high index of suspicion for this condition not to miss a chronic disease with multiple sequelae. Furthermore, these fractures carry a high risk of complications and mortality, so they should be addressed promptly.
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  • 文章类型: Journal Article
    胸锁关节(SCJ)的化脓性关节炎是一种罕见的疾病,占所有关节感染的不到1%。我们报告了一例患有腹膜透析的终末期肾病患者的SCJ严重的双侧化脓性关节炎。一名44岁的女性在从Tenckhoff导管出口部位感染中恢复后一个月出现右SCJ感染。她完成了为期六周的抗生素治疗,但进展为双侧SCJ化脓性关节炎伴骨髓炎,需要对双侧锁骨头进行多次手术清创和切除。进一步的成像显示肾性骨营养不良和退行性关节变化的迹象,类似于焦磷酸钙沉积。终末期肾病患者有多种危险因素,包括免疫系统功能障碍,肾性骨营养不良和透析进入部位增加了对菌血症和播种的易感性。在这样的病人中,及时评估是必要的,以确保迅速诊断和治疗这种潜在的衰弱状况。涉及各种专业的多学科团队对于此类患者的整体护理和降低复发风险至关重要。
    Septic arthritis of the sternoclavicular joint (SCJ) is a rare condition that comprises <1% of all joint infections. We report a case of severe bilateral septic arthritis of the SCJ in a patient with end-stage renal disease on peritoneal dialysis. A 44-year-old female presented with right SCJ infection 1 month after recovering from a tenckhoff catheter exit-site infection. She completed 6 weeks of antibiotics however this progressed to bilateral SCJ septic arthritis with osteomyelitis necessitating multiple surgical debridement and excision of bilateral clavicular heads. Further imaging showed signs of renal osteodystrophy and degenerative joint changes resembling calcium pyrophosphate deposition. Patients with end-stage renal disease have multiple risk factors including immune system dysfunction, renal osteodystrophy and dialysis access sites that increase susceptibility to bacteraemia and seeding. Therefore in such patients, prompt assessment is necessary to ensure expeditious diagnosis and treatment of this potentially debilitating condition. A multidisciplinary team involving various specialties is crucial for the holistic care for such patients and to reduce the risk of recurrence.
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  • 文章类型: Case Reports
    一名63岁的女性,继发于肾小球肾炎的终末期CKD,关于血液透析治疗,出现脊柱侧弯,背痛,以及计划进行矫正手术的身体功能逐渐丧失。外科医生要求优化骨骼健康,因为DXA扫描显示脊柱骨质疏松,臀部,和前臂。由于先前的甲状旁腺次全切除术和正常的甲状旁腺激素和骨特异性碱性磷酸酶水平,怀疑存在低骨转换状态。进行了骨活检,发现骨转换较低,矿化缺陷,和严重的骨质疏松症。患者接受了钙和强化维生素D补充治疗,随后是为期2年的特立帕肽疗程。骨转换标志物的监测表明骨合成代谢反应对治疗,重复DXA显示脊柱和髋部BMD增加。治疗结束时的重复活检显示正常的骨转换和矿化。该病例证明了晚期CKD患者的骨骼健康状况。由于没有针对CKD患者骨折预见性的随机试验,护理必须是个性化的,并且通常基于专家的意见。骨活检的使用在指导治疗中是安全和有益的。
    A 63-yr-old woman with end-stage CKD secondary to glomerulonephritis, on hemodialysis therapy, presented with scoliosis, back pain, and progressive loss of physical function for which corrective surgery was planned. Optimization of bone health was requested by the surgeon as a DXA scan had revealed osteoporosis at spine, hip, and forearm. Due to previous subtotal parathyroidectomy and normal parathyroid hormone and bone-specific alkaline phosphatase levels, a low bone turnover state was suspected. An iliac bone biopsy was performed and revealed low bone turnover, a mineralization defect, and severe osteoporosis. The patient was treated with calcium and intensified vitamin D supplementation, followed by a 2-yr course of teriparatide. Monitoring of bone turnover markers indicated a bone anabolic response to therapy, and a repeat DXA showed increases in BMD at spine and hip. A repeat biopsy at end of treatment showed normal bone turnover and mineralization. This case demonstrates the complicated bone health of patients with advanced CKD. As there are no randomized trials for fracture pretention in patients with CKD, care must be individualized and is often based on expert opinion. The use of bone biopsy is safe and informative in guiding therapy.
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  • 文章类型: Journal Article
    慢性肾脏病(CKD)是一个全球性的健康问题,患病率不断上升。影响全球6.975亿人。它带来了巨大的负担,2017年造成3580万残疾调整寿命年(DALYs)和120万死亡。CKD的死亡率在1990年至2017年间增加了41.5%,将其定位为全球死亡的重要原因。CKD与多种健康并发症有关,影响心血管,神经学,营养,和内分泌方面。一个突出的并发症是CKD-矿物质和骨骼疾病(MBD),涉及骨转换失调的复杂状况,矿化,和力量,伴有软组织和血管钙化。矿物质代谢的改变,包括钙,磷酸盐,甲状旁腺激素(PTH),维生素D,成纤维细胞生长因子-23(FGF-23),还有Klotho,在CKD-MBD中起关键作用。这些骚乱,在CKD早期观察到,有助于骨疾病和肾性骨营养不良(ROD)的进展。血管钙化(VC)是CKD-MBD的重要组成部分,CKD加速。病理生理学涉及血管平滑肌细胞的复杂过程和钙蛋白颗粒(CPP)的形成。VC与心血管事件和死亡率密切相关,强调其预后意义。各种血清标志物和成像技术,包括侧线X线,KauppilaScore,Adragao分数,和脉搏波速度,协助VC检测。此外,pQCT提供了关于动脉钙化的有价值的信息,提供优于传统评分系统的优势。CKD构成了巨大的全球健康负担,和它的并发症,包括CKD-MBD和VC,显着导致发病率和死亡率。了解矿物质代谢之间的复杂关系,骨骼疾病,血管钙化对有效诊断和治疗干预至关重要。
    Chronic kidney disease (CKD) is a global health issue with a rising prevalence, affecting 697.5 million people worldwide. It imposes a substantial burden, contributing to 35.8 million disability-adjusted life years (DALYs) and 1.2 million deaths in 2017. The mortality rate for CKD has increased by 41.5% between 1990 and 2017, positioning it as a significant cause of global mortality. CKD is associated with diverse health complications, impacting cardiovascular, neurological, nutritional, and endocrine aspects. One prominent complication is CKD-mineral and bone disorder (MBD), a complex condition involving dysregulation of bone turnover, mineralization, and strength, accompanied by soft tissue and vascular calcification. Alterations in mineral metabolism, including calcium, phosphate, parathyroid hormone (PTH), vitamin D, fibroblast growth factor-23 (FGF-23), and Klotho, play pivotal roles in CKD-MBD. These disturbances, observed early in CKD, contribute to the progression of bone disorders and renal osteodystrophy (ROD). Vascular calcification (VC) is a key component of CKD-MBD, accelerated by CKD. The pathophysiology involves complex processes in vascular smooth muscle cells and the formation of calciprotein particles (CPP). VC is closely linked to cardiovascular events and mortality, emphasizing its prognostic significance. Various serum markers and imaging techniques, including lateral plain X-ray, Kauppila Score, Adragao Score, and pulse wave velocity, aid in VC detection. Additionally, pQCT provides valuable information on arterial calcifications, offering an advantage over traditional scoring systems. CKD poses a substantial global health burden, and its complications, including CKD-MBD and VC, significantly contribute to morbidity and mortality. Understanding the intricate relationships between mineral metabolism, bone disorders, and vascular calcification is crucial for effective diagnosis and therapeutic interventions.
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