PROGRESS IN PEDIATRIC CARDIOLOGY最新文献

英文中文

Infantile scimitar syndrome with severe pulmonary hypertension with novel 3p26 microdeletion/12q23–24 microduplication: Case report and literature review

IF 0.6 Q4 PEDIATRICS

PROGRESS IN PEDIATRIC CARDIOLOGY

Pub Date : 2024-11-17 DOI: 10.1016/j.ppedcard.2024.101768

Junpei Kawamura , Yoshihiro Takahashi , Koji Nakae , Kentaro Ueno , Yukiko Tazaki , Ikeda Toshiro , Yasuhiro Okamoto

Infantile scimitar syndrome is a rare type of partial pulmonary venous return that is sometimes complicated by severe pulmonary hypertension, which has a poor prognosis. As the genetic background of Scimitar syndrome remains unclear, we report a case of an infant with scimitar syndrome that was analyzed using a chromosomal microarray. Fetal echocardiography revealed an abnormal return of the entire right pulmonary vein to the inferior vena cava. The patient presented with abnormal physical features and a history of atrial septal defects, multiple muscular ventricular septal defects, chronic pleural chylothorax, and hypertrophic pyloric stenosis. The patient died at the age of 3 months despite receiving multidisciplinary treatment with nitric oxide and a pulmonary vasodilator for severe pulmonary hypertension. Chromosomal microarray analysis revealed a copy number loss of 3p.26.1–26.3 (6.5 Mb), a copy number gain of 12q23.2–24.3 (30.8 Mb), and a determined karyotype of 46, XY, der (3) t (3;12) (p26.1;q23.2), arr [grch37] 3p26.1p26.3(62,199_6,541,934) x1,12q23.2q24.3(102,869,918_133,747,247) x3. We report a case of multiple malformations, including Scimitar syndrome and severe pulmonary hypertension with a novel unbalanced translocation involving a 3p26.1–26.3 microdeletion and a 12q23.2–24.3 microduplication. The relationship between unbalanced translocations and the phenotype and prognosis of scimitar syndrome requires further investigation.

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引用次数: 0

A novel approach to thrombectomy and catheter directed tissue-type plasminogen activator in a toddler post-fontan 为一名脑瘫后幼儿实施血栓切除术和导管引导组织型血浆蛋白酶原激活剂的新方法

IF 0.6 Q4 PEDIATRICS

PROGRESS IN PEDIATRIC CARDIOLOGY

Pub Date : 2024-11-09 DOI: 10.1016/j.ppedcard.2024.101767

Vaishnavi Divya Nagarajan , Laura A. Miller-Smith , Yoshi O. Otaki , Ashok Muralidaran , Grant H. Burch , Laurie B. Armsby , Becky J. Riggs

16-month-old with single ventricle Glenn physiology underwent early placement of an extra-cardiac non-fenestrated Fontan. Post-operative course complicated by a nearly occlusive inferior vena cava thrombus and an intra-Fontan thrombus causing multiorgan system failure. Both thrombi were emergently removed with fluoroscopy, ultrasound, and computer guided clot-aspirator in the pediatric catheterization lab.

16个月大的单心室格伦患者，早期接受了心外无瘘丰坦手术。术后因几乎闭塞的下腔静脉血栓和导致多器官系统衰竭的丰坦内血栓而并发症。在儿科导管室通过透视、超声和计算机辅助血栓抽吸器紧急清除了这两个血栓。

引用次数: 0

Left ventricular pseudoaneurysm following balloon aortic valvuloplasty in a preterm neonate: Case report 早产新生儿球囊主动脉瓣成形术后的左心室假性动脉瘤：病例报告

IF 0.6 Q4 PEDIATRICS

PROGRESS IN PEDIATRIC CARDIOLOGY

Pub Date : 2024-10-24 DOI: 10.1016/j.ppedcard.2024.101763

Theodore J. Millette , James J. Gangemi , Shelby C. White , Michael J. Shorofsky

Left ventricular aneurysms and pseudoaneurysms are rare in the pediatric population. We report a case of a preterm neonate with critical aortic valve stenosis who developed a left ventricular pseudoaneurysm and perforation following transcatheter balloon aortic valvuloplasty on day four of life. The possible etiologies of myocardial injury and the unique surgical repair technique are discussed.

左心室动脉瘤和假性动脉瘤在小儿中非常罕见。我们报告了一例患有重度主动脉瓣狭窄的早产新生儿，在出生后第四天接受经导管球囊主动脉瓣成形术后出现左心室假性动脉瘤和穿孔。本文讨论了心肌损伤的可能病因和独特的手术修复技术。

引用次数: 0

Fetal supraventricular tachycardia with giant cardiac rhabdomyoma: Role of post natal sirolimus in a developing country 胎儿室上性心动过速伴巨大心脏横纹肌瘤：产后西罗莫司在发展中国家的作用

IF 0.6 Q4 PEDIATRICS

PROGRESS IN PEDIATRIC CARDIOLOGY

Pub Date : 2024-10-24 DOI: 10.1016/j.ppedcard.2024.101762

Anis Munirah Mohd Kori , Nursyahirah Anum Mohd Radzi , Ammar Mohamad Ziyadi , Intan Juliana Abd Hamid , Nor Rosidah Ibrahim , Noraida Ramli , Mohd Rizal Mohd Zain

Primary cardiac tumors in fetuses are rare and mainly represent rhabdomyoma. The tumors can be clinically silent or cause hemodynamically significant obstructions. We present a case of giant neonatal cardiac rhabdomyoma with right inflow obstruction complicated by fetal supraventricular tachycardia. Fetal echocardiography revealed the presence of supraventricular tachycardia with multiple cardiac masses, one of which caused an obstruction of the right inflow of the heart. As the baby developed hemodynamic instability due to the mass effect, oral sirolimus was initiated postnatally. Despite the limitation of evidence on the usage of sirolimus in neonatal population, this case report revealed the benefits of postnatal sirolimus in a decrease in the size of the mass without any adverse medication effects. Sirolimus has demonstrated efficacy in neonatal cardiac rhabdomyoma, however, a large prospective study is needed to demonstrate the efficacy.

胎儿的原发性心脏肿瘤非常罕见，主要是横纹肌瘤。这些肿瘤可能在临床上无症状，也可能导致血流动力学上的明显梗阻。我们报告了一例新生儿巨大心脏横纹肌瘤并发右心室流入道梗阻和胎儿室上性心动过速的病例。胎儿超声心动图显示，胎儿存在室上性心动过速和多个心脏肿块，其中一个肿块导致右心流入道梗阻。由于肿块效应导致婴儿血流动力学不稳定，产后开始口服西罗莫司。尽管在新生儿群体中使用西罗莫司的证据有限，但该病例报告显示了产后使用西罗莫司的益处，即在减少肿块大小的同时不会产生任何药物不良反应。西罗莫司对新生儿心脏横纹肌瘤有一定疗效，但还需要大型前瞻性研究来证明其疗效。

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引用次数: 0

It is a fine line with flecainide: A case of flecainide toxicity in a pediatric patient 非卡尼是一种微妙的药物：一例儿科非卡尼中毒病例

IF 0.6 Q4 PEDIATRICS

PROGRESS IN PEDIATRIC CARDIOLOGY

Pub Date : 2024-10-18 DOI: 10.1016/j.ppedcard.2024.101761

Aura Daniella Santi-Turchi , Keerthana Banala , Michelle Jadotte , Sherrie Joy Baysa , Steven Fishberger

Flecainide is a class IC antiarrhythmic used in the treatment of supraventricular and ventricular tachyarrhythmias in the pediatric population. It has a narrow therapeutic index that requires careful dosing and monitoring of levels, as well as education on preparation and administration. Many different factors alter the bioavailability of this medication, and special consideration must be taken among pediatric patients. Clinical signs of toxicity include lethargy, bradycardia, altered mental status, and ventricular arrhythmias. Management of flecainide toxicity involves administering sodium bicarbonate as well as lipid emulsion therapy. We present the case of a child found to have flecainide toxicity and discuss management strategies.

氟卡尼是一种 IC 类抗心律失常药物，用于治疗儿童室上性和室性快速性心律失常。它的治疗指数较窄，需要谨慎给药和监测药物浓度，并进行配药和用药方面的教育。许多不同的因素会改变这种药物的生物利用度，因此必须特别考虑到儿科患者。中毒的临床表现包括嗜睡、心动过缓、精神状态改变和室性心律失常。处理非卡尼中毒的方法包括使用碳酸氢钠和脂质乳剂治疗。我们介绍了一例发现福卡尼中毒的患儿，并讨论了处理策略。

引用次数: 0

Migration of a pacemaker into the sigmoid colon in a three-year-old child: A case report 一名 3 岁儿童的心脏起搏器移入乙状结肠：病例报告

IF 0.6 Q4 PEDIATRICS

PROGRESS IN PEDIATRIC CARDIOLOGY

Pub Date : 2024-10-16 DOI: 10.1016/j.ppedcard.2024.101760

I.A. Soynov, A.N. Arkhipov, S.N. Manukian, D.A. Elesin, T.S. Khapaev, A.B. Romanov

Implantation of an epicardial pacemaker in young children is carried out in a pocket above the rectus muscle. In extremely rare cases, pediatric patients may experience migration of the pacemaker into the abdominal cavity. Symptoms can range from mild abdominal discomfort, diarrhea, vomiting to potentially dangerous intestinal obstruction due to perforation of the large intestine. We present a case of a 3-year-old child with migration of the pacemaker into the sigmoid colon with the formation of a colonic fistula. Early diagnosis helps to avoid serious complications in the case of pacemaker migration. Any change in the position of the pacemaker will indicate the need for pacemaker reimplantation to prevent life-threatening symptoms.

为幼儿植入心外膜起搏器是在直肌上方的口袋中进行的。在极少数情况下，小儿患者可能会出现起搏器移位到腹腔的情况。症状可能包括轻微的腹部不适、腹泻、呕吐，也可能因大肠穿孔而导致危险的肠梗阻。我们介绍了一例起搏器移位到乙状结肠并形成结肠瘘的 3 岁儿童病例。早期诊断有助于避免起搏器移位引起的严重并发症。起搏器位置的任何变化都表明需要重新植入起搏器，以防止出现危及生命的症状。

引用次数: 0

Clinical insights: Resolving the specter of cardiac sequelae in multisystem inflammatory syndrome in children (MIS-C) – A 24-month follow-up case series 临床见解：消除儿童多系统炎症综合征（MIS-C）心脏后遗症的阴影--24 个月的随访病例系列

IF 0.6 Q4 PEDIATRICS

PROGRESS IN PEDIATRIC CARDIOLOGY

Pub Date : 2024-10-10 DOI: 10.1016/j.ppedcard.2024.101759

Carolina A.B. Capuruco , Maicon Rodrigues Albuquerque , Daniela Valadão Rosa , Fernanda da Silva Barbosa , Caio Augusto Domingos , Cleonice Carvalho de Coelho Mota , Débora Marques de Miranda

Background

During the COVID-19 pandemic, multisystem inflammatory syndrome (MIS-C) emerged as a novel and severe complication.

Objectives

The present prospective study followed patients in Brazil from February 2020 to December 2022, and evaluated children discharged from reference centers with an MIS-C diagnosis. The aim of the study is to describe long-term cardiac findings.

Methods

Following a clinical and imaging procedure, children with MIS-C were followed up.

Results

Thirty-six children during hospitalization coursed with fever; 61.1 % presented with gastrointestinal symptoms, 77.7 % with cardiocirculatory manifestations, and 28 % with respiratory issues. Shock was observed in 37 % of patients, and the average hospital stay was 9 ± 5 days, with 54 % exhibiting severe symptoms requiring intensive care. Elevated inflammatory and cardiac markers were common; 42 % of patients presented elevated troponin and D-dimer levels. During intensive care, echocardiographic abnormalities were found in 56 % of the patients, including coronary dilation and pericardial effusion. All patients were hospitalized and treated with intravenous immunoglobulin, oral steroids, and acetylsalicylic acid, resulting in 86 % recovery. However, 14 % of the patients had persistent (mild cardiac) alterations at discharge. Sex and previous chronic conditions did not affect the persistence of cardiac findings, whereas marginal age differences indicated that older children tend to have more severe symptoms. BMI was identified as a risk factor but must be considered carefully. Six months post-discharge, all patients successfully recovered from cardiac alterations.

Conclusion

Only a few case series have reported persistent MIS-C findings, and this case series elicits a positive post-discharge prognosis for MIS-C over time.

背景在COVID-19大流行期间，多系统炎症综合征（MIS-C）作为一种新的严重并发症出现。结果36名患儿在住院期间出现发热，61.1%的患儿伴有胃肠道症状，77.7%的患儿伴有心血管表现，28%的患儿伴有呼吸道问题。37% 的患者出现休克，平均住院时间为 9 ± 5 天，54% 的患者症状严重，需要重症监护。炎症和心脏标志物升高很常见；42%的患者肌钙蛋白和D-二聚体水平升高。在重症监护期间，56%的患者发现超声心动图异常，包括冠状动脉扩张和心包积液。所有患者均住院治疗，并接受静脉注射免疫球蛋白、口服类固醇和乙酰水杨酸治疗，结果 86% 的患者康复。然而，14%的患者在出院时仍有持续性（轻微的心脏）改变。性别和既往的慢性疾病并不影响心脏症状的持续性，而年龄上的微小差异则表明，年龄较大的儿童症状往往更为严重。体重指数被认为是一个风险因素，但必须谨慎考虑。出院后六个月，所有患者均成功摆脱了心脏改变。

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引用次数: 0

Are you asleep? Comparing sleep metrics in pediatric cardiac patients with objective and subjective indicators

IF 0.6 Q4 PEDIATRICS

PROGRESS IN PEDIATRIC CARDIOLOGY

Pub Date : 2024-10-06 DOI: 10.1016/j.ppedcard.2024.101758

Nichelle L. Duncan , Rebecca Harrell , Scarlett Anthony , Cierra Buckman , Lauren Sarno , Samuel F. Sears

Background

Examination of sleep in pediatric cardiology patients has typically relied on self-reports with unknown reliability to objective assessments. Wearable technologies provide an objective data source for evaluating sleep. Improved understanding and treatment of sleep in pediatric heart patients may be an untapped aspect that could improve health-related quality of life.

Objectives

The purpose of this study was to identify patterns of duration and quality of sleep in pediatric cardiology patients using both subjective and objective assessment.

Methods

Patients were recruited in a pediatric cardiology clinic and completed a set of sleep and quality of life questionnaires, including the Pittsburg Sleep Quality Index (PSQI), the Pediatric Cardiac Quality of Life Inventory (PCQLI), and the Cardiac Anxiety Questionnaire (CAQ). Patients also agreed to wear an actigraphy watch for 7 days, and caregivers were also recruited as proxy raters.

Results

The sample included 31 patients with a mean age of 15.58 (SD = 1.89). Patients self-reported an average sleep duration of 7.61 h (SD = 1.67, range = 3–11). In contrast, objective sleep data indicated that patients obtained an average of 5.98 h of sleep nightly (SD = 1.01, range = 3.23–7.46) with a sleep efficiency score of 73.2 (SD = 6.23) and a sleep latency period of 21.04 min (SD = 19.16, range = 1–63). Parent-reported sleep duration on the PSQI had a mean of 8.10 h (SD = 1.34, range = 6–11). Thirty four percent of patients met PSQI cut off score for poor sleep based on self-report and 30 % based on parental-report.

Conclusions

Pediatric cardiac patients report better sleep duration than objective assessments. These data suggest that sleep complaints from pediatric cardiac patients may benefit from objective assessment and may need enhanced clinical attention since complaints may overestimate the duration of sleep.

{"title":"Are you asleep? Comparing sleep metrics in pediatric cardiac patients with objective and subjective indicators","authors":"Nichelle L. Duncan , Rebecca Harrell , Scarlett Anthony , Cierra Buckman , Lauren Sarno , Samuel F. Sears","doi":"10.1016/j.ppedcard.2024.101758","DOIUrl":"10.1016/j.ppedcard.2024.101758","url":null,"abstract":"<div><h3>Background</h3><div>Examination of sleep in pediatric cardiology patients has typically relied on self-reports with unknown reliability to objective assessments. Wearable technologies provide an objective data source for evaluating sleep. Improved understanding and treatment of sleep in pediatric heart patients may be an untapped aspect that could improve health-related quality of life.</div></div><div><h3>Objectives</h3><div>The purpose of this study was to identify patterns of duration and quality of sleep in pediatric cardiology patients using both subjective and objective assessment.</div></div><div><h3>Methods</h3><div>Patients were recruited in a pediatric cardiology clinic and completed a set of sleep and quality of life questionnaires, including the Pittsburg Sleep Quality Index (PSQI), the Pediatric Cardiac Quality of Life Inventory (PCQLI), and the Cardiac Anxiety Questionnaire (CAQ). Patients also agreed to wear an actigraphy watch for 7 days, and caregivers were also recruited as proxy raters.</div></div><div><h3>Results</h3><div>The sample included 31 patients with a mean age of 15.58 (SD = 1.89). Patients self-reported an average sleep duration of 7.61 h (SD = 1.67, range = 3–11). In contrast, objective sleep data indicated that patients obtained an average of 5.98 h of sleep nightly (SD = 1.01, range = 3.23–7.46) with a sleep efficiency score of 73.2 (SD = 6.23) and a sleep latency period of 21.04 min (SD = 19.16, range = 1–63). Parent-reported sleep duration on the PSQI had a mean of 8.10 h (SD = 1.34, range = 6–11). Thirty four percent of patients met PSQI cut off score for poor sleep based on self-report and 30 % based on parental-report.</div></div><div><h3>Conclusions</h3><div>Pediatric cardiac patients report better sleep duration than objective assessments. These data suggest that sleep complaints from pediatric cardiac patients may benefit from objective assessment and may need enhanced clinical attention since complaints may overestimate the duration of sleep.</div></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":"77 ","pages":"Article 101758"},"PeriodicalIF":0.6,"publicationDate":"2024-10-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143519231","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Guideline-directed medical therapy for dilated cardiomyopathy in the era of pediatric ventricular assist devices: Improving transplant-free survival 小儿心室辅助装置时代的扩张型心肌病指导性药物治疗：提高无移植生存率

IF 0.6 Q4 PEDIATRICS

PROGRESS IN PEDIATRIC CARDIOLOGY

Pub Date : 2024-09-29 DOI: 10.1016/j.ppedcard.2024.101755

Benjamin A. Olsen , Melanie D. Everitt

Background

Cardiomyopathy remains the leading cause of transplantation in children greater than one year of age, with low five-year transplant free survival. Despite these poor outcomes, the potential for myocardial recovery in pediatric dilated cardiomyopathy (DCM) has long been reported. In the current era of guideline directed medical therapy (GDMT), however, achievement of myocardial recovery has not been rigorously studied. Ventricular assist devices (VADs) can also be employed as a bridge to recovery, but VAD explants for recovery are rare.

Aims of review

A focus of the Sixth International Conference on Childhood Cardiomyopathy was achievement of myocardial recovery in the era of GDMT and VADs. Key questions included: (1) what is the potential for myocardial recovery; (2) what role does GDMT play in achieving myocardial recovery; (3) can durable left ventricular assist devices (LVADs) be a reasonable means to achieving recovery; (4) how do rates of recovery compare between GDMT and LVADs; and (5) what are the next steps in improving rates of myocardial recovery? This review aims to answer these questions by providing a summary of the existing literature.

Key scientific concepts of review

The future directions for pediatric DCM are to use GDMT and VADs as complementary therapies to improve not only survival to transplant, but to assess for recovery parameters so that more can be learned about the potential for transplant-free survival in patients supported by VAD and GDMT.

背景心肌病仍然是一岁以上儿童接受移植的主要原因，其五年无移植存活率很低。尽管治疗效果不佳，但小儿扩张型心肌病（DCM）的心肌恢复潜力却早有报道。然而，在目前指导性医疗疗法（GDMT）的时代，心肌恢复的实现还没有得到严格的研究。第六届儿童心肌病国际会议的重点是在 GDMT 和 VAD 时代实现心肌恢复。关键问题包括(1) 心肌恢复的潜力有多大；(2) GDMT 在实现心肌恢复中扮演什么角色；(3) 耐用的左心室辅助装置 (LVAD) 能否成为实现恢复的合理手段；(4) GDMT 和 LVAD 的恢复率如何比较；以及 (5) 提高心肌恢复率的下一步是什么？本综述旨在通过对现有文献进行总结来回答这些问题。综述的主要科学概念小儿 DCM 的未来发展方向是将 GDMT 和 VAD 作为辅助疗法，不仅提高移植存活率，而且评估恢复参数，以便更多地了解 VAD 和 GDMT 支持下的患者无移植存活的潜力。

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引用次数: 0

Decreased ventricular repolarization variability in one-year-olds of gestational diabetes mothers 妊娠糖尿病母亲一岁孩子心室再极化变异性降低

IF 0.6 Q4 PEDIATRICS

PROGRESS IN PEDIATRIC CARDIOLOGY

Pub Date : 2024-09-29 DOI: 10.1016/j.ppedcard.2024.101756

Martin Schmidt , Alexander Hammer , Hagen Malberg , Silvia M. Lobmaier , Peter Ewert , Renate Oberhoffer-Fritz , Annette Wacker-Gussmann

Background

Gestational diabetes mellitus (GDM) is currently the most common medical complication in pregnancy, affecting approximately 13 % of all pregnant women. Although long-term effects on the offspring are still unclear, previous studies indicate ventricular myocardial changes.

Objectives

The aim of our study was to investigate whether early biomarkers such as QT interval variability (QTV) are sensitive enough to predict these structural changes in children of GDM mothers.

Methods

We focused on children of GDM mothers. Healthy children of mothers without GDM served as controls. All of them were examined at the German Heart Center, Munich, Germany. Heart rate variability (HRV) and QTV measures were extracted from 10 to 15 min Einthoven II electrocardiograms, split into 5 min windows, to characterize the effects of possible autonomic nervous system alterations and cellular ventricular mutations.

Results

75 children were included in this prospective observational case-control study; 42 of them were children of GDM mothers. The median age at the examination was 12 months (11–13 months). We found decreased QTV as a measure of ventricular repolarization variability in one-year-olds of GDM mothers compared to healthy controls (p < 0.05).

Conclusion

We have found increased very low frequency HRV in females and decreased QTV in male children of GDM mothers, which suggests diverse responses and could reflect increased sympathetic tone and altered ventricular myocardium at a cellular level, respectively. Further work is required to understand the long-term significance of these findings in terms of providing an easy-to-use and cost-effective technology for early diagnosis of myocardial damage.

背景妊娠期糖尿病（GDM）是目前最常见的妊娠并发症，约占所有孕妇的 13%。我们的研究旨在探讨 QT 间期变异性（QTV）等早期生物标志物是否足以敏感地预测 GDM 母亲所生子女的结构变化。我们的研究重点是 GDM 母亲的子女，没有 GDM 母亲的健康子女作为对照组。他们都在德国慕尼黑的德国心脏中心接受了检查。从 10 至 15 分钟的 Einthoven II 心电图中提取心率变异性（HRV）和 QTV 测量值，分成 5 分钟窗口，以描述可能的自律神经系统改变和细胞心室变异的影响。检查时的中位年龄为 12 个月（11-13 个月）。我们发现，与健康对照组相比，GDM 母亲的一岁子女的心室再极化变异性指标 QTV 降低了（p < 0.05）。结论我们发现，GDM 母亲的子女中，女性的极低频心率变异性增加，男性的 QTV 降低，这表明了不同的反应，可能分别反映了交感神经张力的增加和心室心肌细胞水平的改变。要了解这些发现的长期意义，还需要进一步的工作，以便为早期诊断心肌损伤提供一种易于使用且经济有效的技术。

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