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  • 文章类型: Journal Article
    一名60多岁患有高淀粉酶血症的男子接受了对比增强计算机断层扫描,这显示了他右侧和左侧腋窝的盆腔肿块。因此,进行了详细的检查。在进行Sonazoid®(全氟丁烷)对比增强超声检查时,发现右侧盆腔肿块从早期就表现出向心对比增强。随后,造影剂从中心消失,并在血管后阶段被洗掉。怀疑肿块是由血管畸形引起的。切除右侧盆腔肿块,经过组织病理学检查,它被检测到是由毛细血管畸形组成的。因此,研究发现,Sonazoid®超声造影检查有助于诊断腹膜后肿块.
    A man in his 60s with hyperamylasemia underwent contrast-enhanced computed tomography, which revealed masses in his pelvic cavity on the right side and in the left axilla. Hence, a detailed examination was performed. Upon performing Sonazoid® (perfluorobutane) contrast-enhanced ultrasound, it was discovered that the right-sided pelvic cavity mass exhibited centripetal contrast-enhancement right from the early stage. Subsequently, the contrast material disappeared from the center and was washed out in the postvascular phase. The mass was suspected to be caused by vascular malformations. The right-sided pelvic cavity mass was excised, and upon histopathological examination, it was detected to be composed of capillary malformations. Thus, it was found that Sonazoid® contrast-enhanced ultrasound examination could aid in diagnosing retroperitoneal masses.
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  • 文章类型: Journal Article
    经过持续42年的长期植入,研究了AISI304不锈钢螺钉与颅骨之间的界面。使用最先进的分析技术分析包含界面区域的样品,包括二次离子质量,傅里叶变换红外,拉曼,和X射线光电子能谱。使用聚焦离子束技术从界面区域切割用于扫描透射电子显微镜的局部样品。以涵盖微米和纳米级分辨率的长度尺度记录了整个界面的化学成分,并且在植入物周围和远端颅骨之间发现了相关差异,表明种植体周围区域通常较年轻的骨组织。此外,能量色散光谱显示出80nm厚的富含氧的钢表面层,表明AISI304材料经历了腐蚀攻击。攻击与金属离子的运输有关,即,亚铁和三价铁,进入邻近植入物的骨层。结果符合释放的铁离子和破骨细胞增殖之间的预期相互作用。相互作用产生自催化过程,其中铁离子刺激破骨细胞活性,而新鲜骨吸收位点的形成通过酸性破骨细胞细胞外区室与植入物表面之间的相互作用促进腐蚀过程。因此,自催化过程可导致种植体周围骨的加速周转。
    Interfaces between AISI 304 stainless steel screws and cranial bone were investigated after long-term implantation lasting for 42 years. Samples containing the interface regions were analyzed using state-of-the-art analytical techniques including secondary ion mass, Fourier-transform infrared, Raman, and X-ray photoelectron spectroscopies. Local samples for scanning transmission electron microscopy were cut from the interface regions using the focused ion beam technique. A chemical composition across the interface was recorded in length scales covering micrometric and nanometric resolutions and relevant differences were found between peri-implant and the distant cranial bone, indicating generally younger bone tissue in the peri-implant area. Furthermore, the energy dispersive spectroscopy revealed an 80 nm thick steel surface layer enriched by oxygen suggesting that the AISI 304 material undergoes a corrosion attack. The attack is associated with transport of metallic ions, namely, ferrous and ferric iron, into the bone layer adjacent to the implant. The results comply with an anticipated interplay between released iron ions and osteoclast proliferation. The interplay gives rise to an autocatalytic process in which the iron ions stimulate the osteoclast activity while a formation of fresh bone resorption sites boosts the corrosion process through interactions between acidic osteoclast extracellular compartments and the implant surface. The autocatalytic process thus may account for an accelerated turnover of the peri-implant bone.
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  • 文章类型: Case Reports
    急性铁中毒是非常罕见的,主要是由于成年人的故意摄入。它可以导致多器官毒性,在严重的情况下,可能演变成急性肝功能衰竭和心血管衰竭,这是死亡的主要原因。临床结果在很大程度上取决于元素铁的摄入量和治疗的准备,其中包括支持,早期肠道净化和去铁胺。尽管及时干预,急性肝功能衰竭可能危及生命,肝移植是唯一可能挽救生命的措施。在这个案例报告中,我们描述了一例由于故意摄入导致暴发性肝功能衰竭的严重急性铁中毒,通过肝移植成功管理。
    Acute iron poisoning is an exceedingly rare occurrence, mainly when resulting from intentional ingestion in adults. It can lead to multi-organ toxicity and, in severe cases, may evolve into acute liver failure and cardiovascular collapse, which are the main causes of death. The clinical outcome is largely dependent on the amount of elemental iron ingested and the readiness of treatment, which includes support, early intestinal decontamination and deferoxamine. Despite timely intervention, acute liver failure can be life-threatening, with liver transplantation being the only potentially life-saving measure. In this case report, we describe a case of severe acute iron poisoning due to intentional ingestion that led to fulminant liver failure, which was successfully managed with liver transplantation.
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  • 文章类型: Journal Article
    目的:本研究旨在评估中国孕妇母体铁状态和胎盘铁转运蛋白表达与先兆子痫(PE)风险的关系。
    方法:共纳入94名PE受试者和112名健康孕妇。收集空腹血样以检测母体铁状态。分娩时收集胎盘样品以检测二价金属转运蛋白1(DMT1)和铁转运蛋白-1(FPN1)的mRNA和蛋白质表达。采用Logistic分析探讨产妇铁状态与PE风险的关系。探讨了胎盘铁转运蛋白与母体铁状态的关系。
    结果:调整协变量后,膳食总铁,非血红素铁摄入量和血清铁调素与PE呈负相关,调整后的OR(95CIs)为0.40(0.17,0.91),0.42(0.18,0.94)和0.02(0.002,0.13),分别。对于最高的三分位数和最低的三分位数,血清铁(4.08(1.58,10.57))和铁蛋白(5.61(2.36,13.31))与PE呈正相关。PE组胎盘组织中DMT1和FPN1的mRNA表达和蛋白水平均上调(p<0.05)。胎盘组织中DMT1和FPN1mRNA的表达与血清铁调素呈负相关(r=-0.71,p<0.001;r=-0.49,p<0.05)。
    结论:结论:产妇铁状态与PE风险密切相关,胎盘DMT1和FPN1在PE中上调,这可能是预防PE的有希望的目标。
    OBJECTIVE: This study aimed to assess the associations of maternal iron status and placental iron transport proteins expression with the risk of pre-eclampsia (PE) in Chinese pregnant women.
    METHODS: A total of 94 subjects with PE and 112 healthy pregnant women were enrolled. Fasting blood samples were collected to detect maternal iron status. The placenta samples were collected at delivery to detect the mRNA and protein expression of divalent metal transporter 1 (DMT1) and ferroportin-1 (FPN1). Logistic analysis was used to explore the associations of maternal iron status with PE risk. The associations of placental iron transport proteins with maternal iron status were explored.
    RESULTS: After adjusting for covariates, dietary total iron, non-heme iron intake and serum hepcidin were negatively associated with PE, with adjusted ORs (95%CIs) were 0.40 (0.17, 0.91), 0.42 (0.18, 0.94) and 0.02 (0.002, 0.13) for the highest versus lowest tertile, respectively. For the highest tertile versus lowest tertile, serum iron (4.08 (1.58, 10.57)) and ferritin (5.61 (2.36, 13.31)) were positively associated with PE. The mRNA expressions and protein levels of DMT1 and FPN1 in placenta were up-regulated in the PE group (p < 0.05). The mRNA expressions of DMT1 and FPN1 in placenta showed a negative correlation with the serum hepcidin (r = -0.71, p < 0.001; r = -0.49, p < 0.05).
    CONCLUSIONS: In conclusion, the maternal iron status were closely associated with PE risk, placental DMT1 and FPN1 were upregulated in PE which may be a promising target for the prevention of PE.
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  • 文章类型: Journal Article
    纳米零价铁(nZVI)是全球范围内用于污染场地修复的主要纳米材料。本研究旨在评估在污染场地的纳米修复中使用nZVI的可持续性,并确定影响在修复中使用nZVI的可持续性的因素。选择了五个试点使用nZVI的案例研究。生命周期分析工具用于评估环境,经济,社会影响,和可持续性。生命周期分析的功能单位为1.00m3的修复土壤和地下水。巴西的案例研究是最不可持续的,而美国的案例研究是最可持续的。只有功能单元的修改导致可持续性指数的变化。不同因素影响NZVI在修复中的可持续性,主要因素是过程中使用的nZVI的量。最后,这项工作大大有助于nZVI在修复中的最新可持续利用。这是对在补救中使用nZVI的可持续性进行详细和全面评估的开创性研究。通过案例分析,有可能确定影响nZVI补救生命周期可持续性的主要因素。
    Nanoscale zero valent iron (nZVI) is globally the main nanomaterial used in contaminated site remediation. This study aims to evaluate the sustainability of using nZVI in the nanoremediation of contaminated sites and to determine the factors that affect the sustainability of the use of nZVI in remediation. Five case studies of nZVI use on a pilot scale were selected. Life cycle analysis tools were used to evaluate environmental, economic, social impacts, and sustainability. The functional unit of the life cycle analyses was 1.00 m3 of remediated soil and groundwater. Case study of Brazil was the least sustainable, while case study of United States was the most sustainable. Only the modification of the functional unit results in variations in the sustainability index. Different factors influence the sustainability of nZVI in remediation, the main factor being the amount of nZVI used in the processes. Finally, this work contributes significantly to the state-of-the-art sustainable use of nZVI in remediation. This is a pioneering study in the detailed and comprehensive assessment of the sustainability of the use of nZVI in remediation. Through the analysis of case studies, it is possible to determine the main factors that influence the sustainability of the nZVI remediation life cycle.
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  • 文章类型: Journal Article
    目的:不安腿综合征(RLS)是维持性血液透析(MHD)患者普遍存在的神经系统并发症。然而,MHD-RLS患者的脑血流量(CBF)改变仍然未知.通过利用动脉自旋标记(ASL)技术,我们评估了不同脑区CBF的波动,并结合临床分析了MHD患者发生RLS的危险因素.
    方法:31例MHD合并RLS患者(MHD-RLS组)和31例非RLS患者根据年龄匹配,性别,以及认知功能(MHD-nRLS组)。通过图像预处理和数据分析,获得了不同大脑区域CBF值的变化,两组间差异较大的脑区CBF值与RLS评分相关。此外,比较了基线数据的差异,通过利用多因素逻辑回归,研究了发生RLS的独立危险因素.
    结果:与MHD-nRLS组相比,MHD-RLS组右颞上回CBF升高,右侧海马的CBF减少,左额中回,直角三角形额下回,左眶中额回,左中前回,离开了普雷苏斯.仅左中央前回CBF与透析时间校正后的RLS评分呈负相关(r=-0.436,P=0.016)。因此,通过逐步方法的多因素回归分析得出,左中前回CBF值(OR:0.968,95CI:0.944-0.993,P=0.012)仍然是MHD患者RLS的独立危险因素。此外,结果表明,血液透析时间(OR:1.055,95CI:1.014-1.098,P=0.008)和血清铁水平(OR:0.685,95CI:0.551-0.852,P=0.001)也是发生RLS的危险因素。
    结论:患有MHD-RLS的患者在多个脑区表现出CBF的改变。值得注意的是,左中央前回可能是影响MHD患者RLS发病的关键区域.此外,血液透析持续时间延长和血清铁水平相对不足是MHD患者发生RLS的危险因素.
    OBJECTIVE: Restless legs syndrome (RLS) stands as a prevalent neurological complication within maintenance hemodialysis (MHD) patients. However, the alterations in cerebral blood flow (CBF) among MHD-RLS patients remain uncharted. Through the utilization of the arterial spin labeling (ASL) technique, we evaluated the fluctuations in CBF within distinct brain regions and analyzed the risk factors for the development of RLS in MHD patients in the context of the clinic.
    METHODS: Thirty-one MHD patients with concomitant RLS (MHD-RLS group) and thirty-one non-RLS patients matched based on age, gender, as well as cognitive function (MHD-nRLS group) were included. Through image preprocessing and data analysis, the changes in CBF values in distinct brain regions were obtained, and the CBF values of brain regions with substantial differences between the two groups were correlated with the RLS scores. Furthermore, the differences in baseline data were compared, and through the utilization of multifactorial logistic regression, the independent risk factors for the development of RLS were examined.
    RESULTS: Compared with the MHD-nRLS group, the MHD-RLS group had increased CBF in the right superior temporal gyrus, reduced CBF in the right hippocampus, left middle frontal gyrus, inferior frontal gyrus of right triangle, middle frontal gyrus of left orbit, left precentral gyrus, and left precuneus. Only left precentral gyrus CBF were negatively correlated with RLS scores after correction for dialysis duration(r = -0.436, P = 0.016). Accordingly, multifactorial regression analysis by stepwise method yielded that the left precentral gyrus CBF values(OR: 0.968, 95%CI: 0.944-0.993, P = 0.012) remained an independent risk factor for RLS in MHD patients. In addition, the results showed that hemodialysis duration (OR: 1.055, 95%CI: 1.014-1.098, P = 0.008) and serum iron levels (OR: 0.685, 95%CI: 0.551-0.852, P = 0.001) were also risk factors for the development of RLS.
    CONCLUSIONS: Patients afflicted with MHD-RLS exhibit alterations in CBF across several brain regions. Notably, the left precentral gyrus might serve as a pivotal region influencing the onset of RLS among MHD patients. Furthermore, extended hemodialysis duration and a relative insufficiency in serum iron levels independently contribute as risk factors for RLS development within the MHD patient population.
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  • 文章类型: Journal Article
    先兆子痫是一种原发性胎盘疾病,胎盘血管化受损导致子宫胎盘灌注不足。我们旨在调查先兆子痫妇女胎盘与健康对照组之间金属和类金属含量的差异。这是63名先兆子痫妇女和113名健康妇女的病例对照研究。从病历中获得临床数据。电感耦合等离子体质谱法(ICP-MS)用于测量胎盘金属和类金属含量。与健康对照组相比,先兆子痫与显著较低浓度的必需元素(镁,钙,铁,铜,锌,和硒)在胎盘组织中。经过多变量调整后,硒浓度的四分位数间距(IQR)增加与子痫前期风险降低相关,OR为0.50(95%CI:0.33~0.77).多种选择的金属和类金属的联合作用与先兆子痫的风险降低有关。胎盘镁含量较低,铬,铁,锌,和硒浓度的先兆子痫病例表明其发病机制的潜在联系。它还为揭示先兆子痫的潜在机制和潜在干预策略的未来研究提供了一个有趣的途径。
    Preeclampsia is a primary placental disorder, with impaired placental vascularization leading to uteroplacental hypoperfusion. We aimed to investigate differences in metal and metalloid content between the placentas of women with preeclampsia and healthy controls. This was a case-control study in 63 women with preeclampsia and 113 healthy women. Clinical data were obtained from medical records. Inductively coupled plasma mass spectrometry (ICP-MS) was used to measure the placental metals and metalloids content. Compared with healthy control subjects, preeclampsia was associated with a significantly lower concentration of essential elements (magnesium, calcium, iron, copper, zinc, and selenium) in the placental tissue. After multivariable adjustment, an interquartile range (IQR) increase in selenium concentration was associated with a reduced risk of preeclampsia with an OR of 0.50 (95% CI: 0.33-0.77). The joint effects of multiple selected metals and metalloids were associated with a reduced risk of preeclampsia. The lower placental magnesium, chromium, iron, zinc, and selenium concentrations of preeclampsia cases indicate a potential link to its pathogenesis. It also provides an intriguing avenue for future research in revealing the underlying mechanisms and potential intervention strategies for preeclampsia.
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  • 文章类型: Journal Article
    目的:微量营养素缺乏是典型的“晚期诊断”乳糜泻(CeD)的特征。这项研究旨在确定“早期诊断”筛查确定的CeD儿童中微量营养素缺乏的患病率,以确定常规检测对这些患者缺乏的临床价值。
    方法:对在大规模筛查研究中诊断出的筛查确定的CeD患者进行了病例对照研究(84例患者,平均年龄11.3±2.6岁)。控件(443个孩子,平均年龄10.8±2.5岁)乳糜泻血清学筛查阴性。血红蛋白,血清铁水平,铁蛋白,叶酸,维生素B12,维生素A,维生素E,25-OH维生素D,锌,和硒被测量。
    结果:平均血清血红蛋白水平,铁,铁蛋白,维生素D,锌,铜,CeD患者的硒含量显着低于健康对照组(血红蛋白12.56vs.13.02g/dL[p=0.04];铁10.61vs.17.6μmol/L[p<0.001],铁蛋白25.7vs.48.3µg/L[p<0.001],维生素D29.1vs.37.5nmol/L,锌11.9vs.21.7μmol/L,铜18.9vs.32.5μmol/L,硒1.04vs.1.36µmol/L;p<0.001)。乳糜泻和严重肠损伤患者(MarshIIIb和IIIc)的血清铁蛋白和维生素A水平明显低于轻度肠损伤患者(MarshII和IIIa)(铁蛋白15vs.22µg/L,p<0.025;维生素A0.85vs.1.35μmol/L,p=0.007)。
    结论:在“早期诊断”筛查确定的CeD病例中仍可检测到微量营养素缺乏,临床相关结果强烈支持CeD筛查和早期诊断的努力。
    OBJECTIVE: Micronutrient deficiencies characterize classical \"late-diagnosed\" celiac disease (CeD). This study aimed to identify the prevalence of micronutrient deficiencies among children with \"early-diagnosed\" screening-identified CeD to determine the clinical value of routine testing for deficiencies in those patients.
    METHODS: A case-control study was conducted on screening-identified CeD patients diagnosed during a mass screening study (84 patients, mean age 11.3 ± 2.6 years). The controls (443 children, mean age 10.8 ± 2.5 years) were negative for celiac disease serological screening. Hemoglobin, serum levels of iron, ferritin, folate, vitamin B12, vitamin A, vitamin E, 25-OH vitamin D, zinc, and selenium were measured.
    RESULTS: The mean serum levels of hemoglobin, iron, ferritin, vitamin D, zinc, copper, and selenium were significantly lower in CeD patients than in healthy controls (hemoglobin 12.56 vs. 13.02 g/dL [p = 0.04]; iron 10.61 vs. 17.6 µmol/L [p < 0.001], ferritin 25.7 vs. 48.3 µg/L [p < 0.001], vitamin D 29.1 vs. 37.5 nmol/L, zinc 11.9 vs. 21.7 µmol/L, copper 18.9 vs. 32.5 µmol/L, selenium 1.04 vs. 1.36 µmol/L; p < 0.001). Patients with celiac and severe intestinal damage (Marsh IIIb and IIIc) had significantly lower serum ferritin and vitamin A levels than patients with mild intestinal damage (Marsh II and IIIa) (ferritin 15 vs. 22 µg/L, p < 0.025; vitamin A 0.85 vs. 1.35 µmol/L, p = 0.007).
    CONCLUSIONS: Micronutrient deficiencies are still detectable in \"early-diagnosed\" screening-identified CeD cases, a clinically relevant result that strongly supports efforts for screening and early diagnosis of CeD.
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  • 文章类型: Case Reports
    背景:由于其不同的临床表现和没有常规筛查,用于缺失性β-地中海贫血的复合杂合子可能难以诊断。这可能导致疾病进展和治疗延迟。
    方法:我们报道了一个罕见β-地中海贫血家族的家系分析和遗传研究。
    方法:家系分析和基因研究证明该患者是β-地中海贫血CD17/东南亚遗传性持续性胎儿血红蛋白缺失的复合杂合子,继承自父母。磁共振成像T2*检查显示肝脏中严重的铁沉积。超声心动图提示心内膜垫缺损。
    方法:患者在接受最终分子遗传学诊断后接受地拉罗司治疗。Deferasirox的初始每日一次剂量为20mg/kg/d。
    结果:患者在首次就诊后三个月停止用药。两年后,患者就诊于肝胆胰疾病科。建议他在先天性心脏病手术修复后接受脾切除术。然而,患者因经济负担而拒绝手术治疗。
    结论:我们报告说,胎儿血红蛋白是筛查β-珠蛋白基因大缺失的敏感指标,这可以通过多重连接依赖性探针扩增试验得到有效证实。在非输血依赖性地中海贫血患者中,应定期进行铁状态评估,铁螯合治疗应及早开始。该病例将为罕见基因型β-地中海贫血的诊断提供见解,并对遗传咨询具有重要意义。
    BACKGROUND: Compound heterozygotes for deletional β-thalassemia can be difficult to diagnose due to its diverse clinical presentations and no routine screenings. This can lead to disease progression and delay in treatment.
    METHODS: We reported pedigree analysis and genetic research in a family with rare β-thalassemia.
    METHODS: Pedigree analysis and genetic research demonstrated that the patient was a compound heterozygote for β-thalassemia CD17/Southeast Asian hereditary persistence of fetal hemoglobin deletion, inherited from the parents. Magnetic resonance imaging T2* examination revealed severe iron deposition in the liver. Echocardiography revealed endocardial cushion defect.
    METHODS: The patient was treated with Deferasirox after receiving the final molecular genetic diagnosis. The initial once-daily dose of Deferasirox was 20 mg/kg/d.
    RESULTS: The patient discontinued the medication three months after the first visit. Two years later, the patient visited the Department of Hepatobiliary and Pancreatic Diseases. He was recommended to undergo splenectomy after surgical repair of the congenital heart disease. However, the patient refused surgical treatment because of the economic burden.
    CONCLUSIONS: We report that fetal hemoglobin is a sensitive indicator for screening large deletions of the β-globin gene, which can be effectively confirmed by the multiplex ligation-dependent probe amplification assay. In non-transfusion-dependent thalassemia patients, iron status assessment should be regularly performed, and iron chelation treatment should be initiated early. This case will provide insights for the diagnosis of rare genotypes of β-thalassemia and has important implications for genetic counseling.
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  • 文章类型: Case Reports
    背景:本病例报道1例恶性嗜铬细胞瘤,表现为视力改变并伴有肺转移和复发。
    方法:一名10岁的汉族女孩出现视力变化,最终通过对比增强计算机断层扫描诊断为嗜铬细胞瘤,尿香草扁桃酸.在治疗高血压和手术后,临床症状消失。使用肾上腺刻度评分系统的嗜铬细胞瘤在组织学上证实了具有肺转移的恶性嗜铬细胞瘤,并在遗传上具有琥珀酸脱氢酶复合物铁硫亚基B突变,三个月后,由于高风险和复发迹象,我们进行了非计划手术.在撰写本病例报告时,她没有症状。我们病人的病例突出了考虑诊断恶性嗜铬细胞瘤的重要性,以及可能复发的长期随访。
    结论:尽管有公认的与嗜铬细胞瘤相关的经典临床表现,非典型介绍,比如儿童的视力变化,应该考虑。此外,由于复发风险较高,肾上腺嗜铬细胞瘤评分高,琥珀酸脱氢酶复合物铁硫亚基B突变的恶性嗜铬细胞瘤儿童需要长期随访,甚至非计划手术.
    BACKGROUND: This case report documents a case of malignant pheochromocytoma manifested as vision changes with lung metastasis and recurrence.
    METHODS: A 10-year-old Han Chinese girl presented with vision changes and was eventually diagnosed with pheochromocytoma by contrast-enhanced computed tomography, urine vanillylmandelic acid. After medication for hypertension and surgery, clinical symptoms disappeared. Malignant pheochromocytoma with lung metastasis was confirmed histologically using the Pheochromocytoma of the Adrenal Gland Scaled Score scoring system and genetically with succinate dehydrogenase complex iron sulfur subunit B mutation, and 3 months later, unplanned surgery was performed because of the high risks and signs of recurrence. She is asymptomatic as of the writing of this case report. Our patient\'s case highlights the importance of considering a diagnosis of malignant pheochromocytoma, and long-term follow-up for possible recurrence.
    CONCLUSIONS: Although there are well-recognized classic clinical manifestations associated with pheochromocytoma, atypical presentation, such as vision changes in children, should be considered. In addition, malignant pheochromocytoma children with a high Pheochromocytoma of the Adrenal Gland Scaled Score and succinate dehydrogenase complex iron sulfur subunit B mutation require a long-term follow-up or even unplanned surgery because of the higher risk of recurrence.
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