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A Rare Variant of Mazabraud’s Syndrome Overlapping with McCune-Albright Syndrome with a Clinical Review: A Case Report 一罕见的马扎布罗氏综合征与麦库恩-奥尔布赖特综合征重叠的变异与临床回顾:一个病例报告
Pub Date : 2022-06-30 DOI: 10.15746/sms.22.008
D. Lee, S. Kwak, S. Jang, Hwanjun Choi, Jun Hyuk Kim
Mazabraud syndrome (MS) is a rare and sporadic disorder. It is mainly characterized by fibrous dysplasia (FD) of single or multiple bones and intramuscular myxomas (IM). Data on the prevalence since it was first reported, clinical features, and prognosis are extremely scarce. We report a case of a 59-year-old woman with IM and polyostotic FD. She also had multiple cafe’-au-lait spots suggestive of McCune-Albright syndrome (MAS). On magnetic resonance imaging, there are masses with well-defined heterogeneous enhancement, accompanied by an inner cyst in the vastus lateralis muscle and femur. These radiological results are identical to those of FD. After surgical intervention with excision of intramuscular soft-tissue mass, a diagnosis of IM of MS was confirmed. Given that cafe’-au-lait spots also appeared, the patient was diagnosed with a variant of MS with some of the clinical characteristics of MAS.
马扎布罗综合征(MS)是一种罕见的散发性疾病。主要表现为单骨或多骨纤维发育不良(FD)和肌内黏液瘤(IM)。自首次报道以来,有关该病的患病率、临床特征和预后的数据极为缺乏。我们报告一例59岁女性IM和多囊性FD。她身上还有多处咖啡斑,提示她患有麦丘内-奥尔布赖特综合征(MAS)。在磁共振成像上,有明确的不均匀强化肿块,并伴有股外侧肌和股骨内囊肿。这些放射学结果与FD相同。手术切除肌内软组织肿块后,确诊为多发性硬化症。考虑到还出现了咖啡斑,该患者被诊断为多发性硬化症的变体,具有多发性硬化症的一些临床特征。
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引用次数: 0
Effect of Remifentanil Concentration on Propofol Demand during Total Intravenous Anesthesia 瑞芬太尼浓度对全静脉麻醉中异丙酚需求的影响
Pub Date : 2022-06-30 DOI: 10.15746/sms.22.006
Young Jun Kim, J. Song
Objective: The purpose of this study was to investigate the effects of remifentanil concentration during total intravenous anesthesia changes on propofol requirements, vital signs, wake-up time, and postoperative pain.Methods: After induction of anesthesia, the concentration of remifentanil was divided into 2 ng/mL (R2 group) and 6 ng/mL (R6 group). In each group, the propofol concentration for maintaining a bispectral index (BIS) of 40–50, systolic blood pressure, diastolic blood pressure, mean arterial pressure, heart rate, time from drug discontinuation to eye opening, and pain score in the recovery room were all measured using the Student t-test. Differences between the two groups were verified for statistical significance.Results: The average BIS scores maintained during the time of measurement were 45.0±3.8 and 45.5±3.9 in the R2 and R6 groups, respectively. The effective concentrations of propofol to maintain these corresponding BIS scores were 3.0±0.6 mcg/mL and 2.4±0.7 mcg/mL in the R2 and R6 groups, respectively.Conclusion: On using propofol and remifentanil during total intravenous anesthesia, the R6 group showed a reduction in the amount of propofol required to maintain a constant BIS, as well as reduced intraoperative blood pressure and pulse compared to those in the R2 group. No significant effects were observed in the time taken for the patient to wake up postoperatively and in postoperative pain experienced by the patient.
目的:探讨静脉全麻醉时瑞芬太尼浓度变化对异丙酚需氧量、生命体征、苏醒时间及术后疼痛的影响。方法:麻醉诱导后,瑞芬太尼浓度分为2 ng/mL (R2组)和6 ng/mL (R6组)。各组患者维持双谱指数(BIS) 40-50的异丙酚浓度、收缩压、舒张压、平均动脉压、心率、停药至睁眼时间、康复室疼痛评分均采用学生t检验。两组间的差异经统计学意义验证。结果:R2组和R6组在测量期间的BIS平均评分分别为45.0±3.8分和45.5±3.9分。在R2组和R6组,维持相应BIS评分的异丙酚有效浓度分别为3.0±0.6 mcg/mL和2.4±0.7 mcg/mL。结论:与R2组相比,R6组在全静脉麻醉中使用异丙酚和瑞芬太尼时,维持恒定BIS所需的异丙酚量减少,术中血压和脉搏也降低。在患者术后醒来所需的时间和患者术后经历的疼痛方面没有观察到明显的影响。
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引用次数: 0
Chondrolipoma of the Shoulder: A Case Report 肩部软骨脂肪瘤1例报告
Pub Date : 2022-06-30 DOI: 10.15746/sms.22.010
S. Kang
Chondrolipoma is rare and is mainly encountered in large-sized, long-standing lipomas. Chondrolipoma can be found at almost any site of the body, particularly in the connecting tissue of the skeletal system, breast, pharynx, and nasopharynx. A small number of chondrolipomas have been reported in the literature with rare magnetic resonance examples available. Here, we report a rare case of chondrolipoma in the shoulder and review its radiographic and pathologic correlation. This paper aims to report plain radiograph, computed tomography, and magnetic resonance examples of chondrolipoma
软骨脂肪瘤是罕见的,主要发生在大的,长期存在的脂肪瘤。软骨脂肪瘤几乎可以在身体的任何部位发现,特别是在骨骼系统、乳房、咽和鼻咽的连接组织中。少量的软骨脂肪瘤已报道在文献中罕见的磁共振例子可用。在此,我们报告一例罕见的肩关节软骨脂肪瘤,并回顾其影像学和病理学的相关性。本文的目的是报告平片,计算机断层扫描和磁共振的例子软骨脂肪瘤
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引用次数: 0
A Case of Transient Neonatal Diabetes Mellitus Attributable to a Nonspecific Mutation in the ABCC8 Gene ABCC8基因非特异性突变引起的新生儿短暂性糖尿病1例
Pub Date : 2021-12-30 DOI: 10.15746/sms.21.030
Won Seob Shin, H. Jeong, J. Koh
Neonatal diabetes mellitus (NDM) is defined as hyperglycemia that persists for more than 2 weeks and requires insulin therapy. NDM principally occurs before 6 months of age. Transient NDM (TNDM) is a clinical form of NDM that persists for a median of 12 weeks and resolves completely by 18 months. However, it may relapse as type 2 DM during early adulthood. The major causes of TNDM are mutations in chromosome 6q24 or the KCNJ11 or ABCC8 genes; the latter encode the two subunits of the pancreatic adenosine triphosphate (ATP)-sensitive potassium channel (KATP-channel). This condition responds well to oral sulfonylurea therapy. Herein, we report a neonate who was small for gestational age and exhibited TNDM symptoms. Genetic analysis revealed a nonspecific mutation in ABCC8; he was successfully treated with oral sulfonylurea.
新生儿糖尿病(NDM)被定义为持续2周以上的高血糖,需要胰岛素治疗。NDM主要发生在6个月前。短暂性NDM (TNDM)是一种临床形式的NDM,持续时间中位数为12周,18个月完全消退。然而,它可能在成年早期复发为2型糖尿病。TNDM的主要原因是染色体6q24或KCNJ11或ABCC8基因突变;后者编码胰腺三磷酸腺苷(ATP)敏感钾通道(katp通道)的两个亚基。这种情况对口服磺脲治疗反应良好。在此,我们报告一个新生儿谁是小于胎龄和表现出TNDM症状。遗传分析显示ABCC8存在非特异性突变;口服磺脲治疗成功。
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引用次数: 0
A Case of Migration of Pipeline Embolization Device Causing Rupture during Treatment of an Unruptured Vertebral Artery Dissecting Aneurysm 未破裂椎动脉夹层动脉瘤治疗中管道栓塞装置移位致破裂1例
Pub Date : 2021-12-30 DOI: 10.15746/sms.21.027
S. Kim, Dong-Kyu Yeo, Gwang Soo Lee
Endoluminal reconstruction of an intracranial aneurysm using flow-diverting devices, such as the pipeline embolization device (PED), is a new treatment modality with good clinical outcomes. The device was originally indicated for challenging cases, such as wide-necked large or giant aneurysms, and is gaining popularity as a reliable treatment for nearly all intracranial aneurysms. The overall complication rate of flow-diverting devices use is 17.0%, including occlusion of side-branching or perforating arteries, rerupture of the aneurysm, in-stent thrombosis, and, rarely, stent migration. We report a rare complication of the PED: delayed migration of the PED after successful stent implantation during treatment of an unruptured vertebral artery dissecting aneurysm, which resulted in rupture of the aneurysm. Further, we discuss technical steps that can be taken to prevent this potential complication.
利用管道栓塞装置(PED)等分流装置进行颅内动脉瘤腔内重建是一种新的治疗方式,具有良好的临床效果。该设备最初用于具有挑战性的病例,如宽颈大动脉瘤或巨型动脉瘤,并且作为几乎所有颅内动脉瘤的可靠治疗方法而越来越受欢迎。使用分流装置的总并发症发生率为17.0%,包括侧分支或穿孔动脉闭塞、动脉瘤再破裂、支架内血栓形成,以及很少发生的支架迁移。我们报告一个罕见的PED并发症:在治疗未破裂的椎动脉夹层动脉瘤期间,成功植入支架后PED延迟迁移,导致动脉瘤破裂。此外,我们讨论了可以采取的技术步骤,以防止这种潜在的并发症。
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引用次数: 0
Cardiac Arrest due to Carbon Dioxide Embolism During Laparoscopic Gynecologic Surgery of a Patient with Previous Abdominal Surgery: A Case Report 有腹部手术史的腹腔镜妇科手术中二氧化碳栓塞导致心脏骤停1例
Pub Date : 2021-12-30 DOI: 10.15746/sms.21.025
N. Kim
Catastrophic carbon dioxide (CO2) embolism is a rare, but potentially life-threatening, the complication of laparoscopic gynecologic surgery. We report the case of a healthy 53-year-old woman who developed CO2 embolism and cardiac arrest during laparoscopic surgery. She had a history of two cesarean sections and had extensive peritoneal adhesions. After placement of the trocar and insufflation of CO2, end-tidal CO2 dropped from 35 to 15 mm Hg, and the patient had a cardiovascular collapse. In this patient, CO2 embolism was diagnosed on the basis of a sudden decrease in end-tidal CO2, hypotension, and hypoxemia. The patient was managed quickly and aggressively. The patient recovered completely following the treatment for CO2 embolism, with no cardiopulmonary or neurological sequelae. There is an increased risk of catastrophic CO2 embolism during laparoscopic gynecologic surgery in patients with previous abdominal surgery. Therefore, the surgeon and anesthesiologist should remain vigilant to promote early detection of CO2 embolism.
灾难性的二氧化碳(CO2)栓塞是一种罕见的,但可能危及生命的腹腔镜妇科手术并发症。我们报告的情况下,一个健康的53岁妇女谁发展CO2栓塞和心脏骤停在腹腔镜手术。她有两次剖宫产史,并有广泛的腹膜粘连。置入套管针并注入CO2后,潮末CO2从35 mm Hg降至15 mm Hg,患者发生心血管衰竭。在这个病人中,CO2栓塞的诊断是基于潮末CO2突然下降、低血压和低氧血症。病人得到了迅速而积极的治疗。患者在CO2栓塞治疗后完全恢复,无心肺或神经系统后遗症。有腹部手术史的患者在腹腔镜妇科手术中发生灾难性CO2栓塞的风险增加。因此,外科医生和麻醉师应保持警惕,促进CO2栓塞的早期发现。
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引用次数: 0
Procedural Sedation of Preterm Neonate with Frequent Apnea during Magnetic Resonance Imaging: A Case Report 磁共振成像过程中频繁呼吸暂停的早产儿镇静1例
Pub Date : 2021-12-30 DOI: 10.15746/sms.21.021
Jaewoong Jung, Ju-Hun Park, Y. Chung, W. Chae
Magnetic resonance imaging (MRI) is a useful and safe imaging modality for examining preterm infants. However, MRI examination requires careful precautions, and infants and children are likely to require deep sedation or anesthesia to keep them still during the examination. Sedation has various risks and the greatest concern of sedation is cardiorespiratory events. In addition, delicate titration is also necessary for preterm infants because propofol pharmacokinetics is different from those in older children. We successfully completed sedation of a preterm neonate (gestational age, 32+1 weeks; birth weight, 1,970 g) with a history of frequent apnea through careful assessment and continuous monitoring. We want to suggest alternative options for airway management of the high risk of respiratory complications.
磁共振成像(MRI)是一种有效且安全的检查早产儿的成像方式。然而,MRI检查需要仔细的预防措施,婴儿和儿童在检查期间可能需要深度镇静或麻醉以保持静止。镇静有多种风险,镇静最令人担忧的是心肺事件。此外,由于异丙酚的药代动力学与年龄较大的儿童不同,早产儿也需要精细的滴定。我们成功地完成了1例早产新生儿(胎龄32+1周;出生体重1,970 g),通过仔细评估和持续监测有频繁呼吸暂停史。我们想建议的替代方案,气道管理的高风险呼吸系统并发症。
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引用次数: 0
Anesthetic Management for Spine Surgery in a Patient with Left Internal Carotid Artery Occlusion and Penetrating Aortic Ulcer: A Case Report 左颈内动脉闭塞伴穿透性主动脉溃疡的脊柱外科麻醉处理1例报告
Pub Date : 2021-12-30 DOI: 10.15746/sms.21.026
Misoon Lee, Dasom Choi, B. Koo, S. Cho
A key challenge of anesthesia is to provide patients with safe and optimized anesthetic management to improve prognosis and minimize mortality and morbidity. To this end, the anesthesiologist should comprehensively understand the patient’s physical status through pre-anesthetic assessment and carefully monitor the patient during surgery. Several types of novel patient-monitoring devices may be useful to achieve this purpose. We report a case of anesthetic management in a patient with left internal carotid artery occlusion and penetrating aortic ulcer.
麻醉的关键挑战是为患者提供安全和优化的麻醉管理,以改善预后,减少死亡率和发病率。为此,麻醉师应通过麻醉前评估全面了解患者的身体状况,并在术中仔细监测患者。几种新型的病人监护设备可能有助于实现这一目的。我们报告一例麻醉管理的病人左颈内动脉闭塞和穿透性主动脉溃疡。
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引用次数: 0
Biomedical Ethics for Jehovah’s Witnesses and Patient Blood Management 耶和华见证人和病人血液管理的生物医学伦理
Pub Date : 2021-12-30 DOI: 10.15746/sms.21.013
S. Cho
Jehovah’s Witnesses refuse blood transfusions according to religious beliefs, and for this reason, most hospitals and doctors have refused their treatment. There are more than 100,000 religious people in Korea, but there are few bloodless centers that can receive their treatment. So, the number of Jehovah’s Witnesses patients visiting bloodless centers in Soonchunhyang University Bucheon Hospital has been increasing every year. Despite this situation, no legal or medical countermeasure has yet been proposed against them. Therefore, I would like to take a bioethical approach based on “principles of biomedical ethics” and introduce “patient blood management” which is currently spreading in advanced medical countries.
耶和华见证人根据宗教信仰拒绝输血,因此,大多数医院和医生拒绝为他们治疗。韩国有10万多名信教人士,但能接受他们治疗的“不流血的中心”却很少。因此,前往顺天乡大学富川医院无血中心的“耶和华见证会”患者逐年增加。尽管如此,还没有针对他们提出法律或医疗对策。因此,我想以“生物医学伦理原则”为基础,采用生物伦理学的方法,介绍目前在医学先进国家流行的“患者血液管理”。
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引用次数: 0
Infected Epidermoid Cyst Including Multiple Nails at the Amputated Thumb Stump: A Case Report 感染的表皮样囊肿包括多个指甲在拇指残端:1例报告
Pub Date : 2021-12-30 DOI: 10.15746/sms.21.024
Dae-Geun Kim, B. H. Kwack
Fingertip amputation is a common injury among trauma occurring in the upper extremity. After amputation of the fingertip, there are several treatment options according to the degree of damage. Also, the skin flap is sometimes performed when skin defects are accompanied. Among the complications associated with fingertip injury, cyst formation at the amputation stump is rare but some cases have been reported based on the pathologic findings. There was a case of an infected epidermal inclusion cyst containing multiple nail plates at the amputation stump of the thumb, so we would like to report it with a review of the literature.
指尖截肢是上肢外伤中常见的一种损伤。指尖截肢后,根据损伤程度有几种治疗选择。此外,有时在伴有皮肤缺损的情况下进行皮瓣修复。在与指尖损伤相关的并发症中,在断指残端形成囊肿是罕见的,但一些病例根据病理结果被报道。有一例感染的表皮包涵囊肿包含多个指甲板在拇指截肢残端,所以我们想报告它并复习文献。
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引用次数: 0
期刊
Soonchunhyang Medical Science
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