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Occurrence of granulocytic sarcoma after allogenic hematopoietic stem cell transplantation 同种异体造血干细胞移植后粒细胞肉瘤的发生
Pub Date : 2018-03-30 DOI: 10.33425/2639-8478.1005
Merve Pamukçuoğlu, M. S. Pepeler, Burcu Ülküden, G. Pu, N. Akyürek, Ş. Akı, G. Sucak
Myeloid sarcoma (MS), chloroma and granulositic sarcoma (GS) are all used to describe tumours which proliferate as a result of blasts in the extramedullary parts of the body [1]. Chloroma took its’name from the colour of the tumour [1,2]. It is usually green [1] Granulositic sarcoma occurs in Myelodysplastic syndrome (MDS), Chronic Myelocyter Leukemia (CML) and in 2-8% of AML patients, at a younger age [3,4]. İt usually seen at 1-81 years of age and the size of GS can be from 2 to 20 cm [1]. Compression symptoms of pain and bleeding were as a result of the mass effect of GS. We determined the localizing of mass by Positron emission tomography with fluoroD-glucose integrated with computed tomography (FDG-PET CT) [1,5]. Mass is of containing immature granulocytic series cells and also pathological examination is very difficult [6]. There are three pathologic groups. Group-1 predominantly contains myeloblasts which are poorly differentiated. Group -2 contains moderately differentiated myeloblasts and promyelosites. Promyelosites are dominant. Group-3 contains an equal value of well matured promyelosites and myelosites [4]. Mitotic activity is very different in GS. Kİ-67/MIB1 score was always high (50%95%) [1,7]. When we take a biopsy from a different part of the tumor mitotic activity can change [4]. Single body macrophage demonstrates faster tumor cell turnover [4], but GS does not always have uniform structure nor uniform chromosomal anomalies [4,7].
髓样肉瘤(MS)、淋巴瘤和颗粒肉瘤(GS)都被用来描述由于身体髓外部分的成纤维细胞而增殖的肿瘤[1]。Chloroma的名字来源于肿瘤的颜色[1,2]。它通常是绿色的[1]粒细胞肉瘤发生在骨髓增生异常综合征(MDS)、慢性粒细胞白血病(CML)和2-8%的AML患者中,年龄较小[3,4]。伊特通常在1-81岁时出现,GS的大小可以从2到20厘米[1]。疼痛和出血的压迫症状是GS质量效应的结果。我们通过正电子发射断层扫描结合氟代葡萄糖和计算机断层扫描(FDG-PET CT)确定了质量的定位[1,5]。含有未成熟粒细胞系细胞的肿块,病理检查也很困难[6]。有三个病理组。第1组主要包含分化较差的成髓细胞。第2组包含中等分化的成髓细胞和早幼粒细胞。Promyelocites占主导地位。第3组含有同等价值的成熟早幼粒细胞和骨髓粒细胞[4]。GS的有丝分裂活性差异很大。Kï-67/MIB1评分一直很高(50%95%)[1,7]。当我们从肿瘤的不同部位进行活检时,有丝分裂活性可能会发生变化[4]。单体巨噬细胞表现出更快的肿瘤细胞周转[4],但GS并不总是具有均匀的结构或均匀的染色体异常[4,7]。
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引用次数: 1
A pilot study of narrative approach for terminally ill cancer patient at home hospice 晚期癌症病人在家安宁疗护的叙事方法初步研究
Pub Date : 2018-01-01 DOI: 10.15761/CCRR.1000383
M. Ando, Mayumi Yamamoto, Yasuyoshi Ninosaka, K. Okamura
In these days in Japan, the number of elders is increasing rapidly and many will be die in near future. Home hospice is one of the facilities where they spend their final stage. In Japan the Ministry of Health, Labour and Welfare has recommended the expansion of equality for accessibility of care [1]. However, since home hospice has just begun rapidly in Japan, there are few a formal psychological or spiritual care.
在这些日子里,日本的老年人数量正在迅速增加,许多人将在不久的将来死去。家庭临终关怀是他们度过最后阶段的设施之一。在日本,厚生劳动省建议扩大保健服务可及性方面的平等。然而,由于家庭临终关怀在日本刚刚起步,很少有正式的心理或精神护理。
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引用次数: 1
Hypertension and Hypertensive Heart Disease in Children and Adolescents 儿童和青少年高血压和高血压心脏病
Pub Date : 2018-01-01 DOI: 10.15761/ccrr.1000384
M. F. Elshamaa
The epidemic of overweight and obesity in youth is increasing the prevalence of prehypertension and hypertension among children and adolescents. The younger the child is at presentation and the more severe the blood pressure abnormality, the more likely a secondary cause of hypertension to be present. Measurement of blood pressure (BP) in children requires adaptation to the age and size of the children. Interpretation must be related to normative values specific to age, gender and height. Evaluation is primarily aimed at identifying secondary causes of hypertension, associated comorbidities, additional risk factors, and evidence of target organ damage. Ambulatory blood pressure monitoring is emerging as a useful tool for evaluation of some patients, particularly for those with suspected white coat hypertension. Uncontrolled and prolonged elevation of blood pressure can lead to a variety of changes in the myocardial structure, coronary vasculature, and conduction system of the heart. These changes in turn can lead to the development of left ventricular hypertrophy, coronary artery disease, various conduction system diseases, and systolic and diastolic dysfunction of the myocardium, cardiac arrhythmias (especially atrial fibrillation), and congestive heart failure. Although these diseases generally develop in response to chronically elevated BP, marked and acute elevation of BP can lead to accentuation of an underlying predisposition to any of the symptoms traditionally associated with chronic hypertension. Management of prehypertension and hypertension is directed at the underlying cause, exacerbating factors, and the magnitude of the blood pressure abnormality. Healthy behavioral changes are a primary management tool for treating hypertension, and more particularly prehypertension and for addressing other cardiovascular risk factors, such as obesity. Pharmacological management is reserved for patients with hypertension who do not respond to behavioral changes, have additional cardiovascular risk factors or diabetes, are symptomatic or have developed target organ damage. Correspondence to: Manal Fuad Elshamaa, Pediatrics Department &Echocardiography Clinic, National Research Centre, Cairo, Egypt, E-mail: manal_elshmaa@hotmail.com
青少年超重和肥胖的流行增加了儿童和青少年高血压前期和高血压的患病率。患儿发病年龄越小,血压异常越严重,继发高血压的可能性越大。测量儿童血压(BP)需要适应儿童的年龄和体型。口译必须与具体到年龄、性别和身高的规范性价值有关。评估的主要目的是确定高血压的继发原因、相关合并症、其他危险因素和靶器官损伤的证据。动态血压监测正在成为评估一些患者的有用工具,特别是对那些怀疑有白大衣高血压的患者。不受控制和长期的血压升高可导致心肌结构、冠状动脉血管和心脏传导系统的各种变化。这些变化反过来可导致左室肥厚、冠状动脉疾病、各种传导系统疾病、心肌收缩和舒张功能障碍、心律失常(尤其是心房颤动)和充血性心力衰竭的发展。虽然这些疾病通常是长期血压升高的反应,但明显的急性血压升高可导致任何传统上与慢性高血压相关的症状的潜在易感性加剧。高血压前期和高血压的管理是针对潜在的原因,加剧因素和血压异常的程度。健康的行为改变是治疗高血压,尤其是高血压前期和解决其他心血管危险因素(如肥胖)的主要管理工具。对于行为改变无反应、有心血管危险因素或糖尿病、有症状或靶器官损害的高血压患者,药理学管理是保留的。通讯:Manal Fuad Elshamaa,儿科和超声心动图诊所,国家研究中心,开罗,埃及,E-mail: manal_elshmaa@hotmail.com
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引用次数: 1
Taking tobacco control for a lap and lunch: Lessons learned from tobacco control initiatives and the impact on the uptake of obesity management strategies 将烟草控制进行一圈和午餐:从烟草控制行动中吸取的教训和对采用肥胖管理战略的影响
Pub Date : 2018-01-01 DOI: 10.15761/CCRR.1000386
Taniya S. Nagpal, M. Fagan, H. Prapavessis
Smoking prevalence has been declining in recent years due to utilizing a multi-faceted approach including; policy implementation, ongoing education and behavioural interventions. It has been suggested that the tobacco control approach can be applied to obesity management but the uptake has been minimal. This article examines plausible reasons as to why the suggested strategies from tobacco control have not yet taken effect for obesity management specifically aiming to increase physical activity and nutrition. Utilizing tobacco control strategies to increase physical activity and healthy dietary habits to prevent obesity is not a novel idea, however unlike tobacco prevalence we have not seen a decline in rates of obesity. The purpose of this short communication is to highlight potential reasons as to why the lessons learned from tobacco control have not seen the same success on obesity prevalence specifically in North America.
近年来,由于采取了多方面的措施,吸烟率一直在下降,包括:政策执行、持续教育和行为干预。有人建议,烟草控制方法可以应用于肥胖管理,但收效甚微。这篇文章探讨了为什么烟草控制建议的策略尚未对肥胖管理产生影响的合理原因,特别是旨在增加身体活动和营养。利用烟草控制策略来增加体育活动和健康的饮食习惯来预防肥胖并不是一个新颖的想法,然而,与烟草流行不同,我们没有看到肥胖率的下降。这篇简短交流的目的是强调从烟草控制中吸取的经验教训没有在肥胖流行方面取得同样成功的潜在原因,特别是在北美。
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引用次数: 0
Cerebral venous thrombosis associated with Migraine 脑静脉血栓与偏头痛相关
Pub Date : 2018-01-01 DOI: 10.15761/CCRR.1000399
R. Verma, Sagarika Mahapatro, A. More
Migraine is one of the commonest neurological disorder encountered by physicians. It affects around 20% of the females and 12% males of the normal population. It is a genetically determined, neurovascular disorder having complex pathophysiology [1]. Cerebral venous thrombosis is a rare type of stroke, which affects 0.5-1% of all types of strokes .Cerebral venous thrombosis presents with varied neurological manifestations, which include headache, seizures, altered sensorium, focal neurological deficits and intracranial hypertension. There are various infective and non infective conditions associated with cerebral venous thrombosis [2].
偏头痛是医生遇到的最常见的神经系统疾病之一。正常人群中约有20%的女性和12%的男性患有此病。这是一种遗传决定的神经血管疾病,具有复杂的病理生理。脑静脉血栓形成是一种罕见的中风类型,约占所有中风类型的0.5-1%。脑静脉血栓形成表现为多种神经系统症状,包括头痛、癫痫发作、感觉改变、局灶性神经功能缺损和颅内高压。有多种与脑静脉血栓形成相关的感染性和非感染性疾病。
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引用次数: 0
Molybdenum Cofactor Deficiency 缺钼辅助因子
Pub Date : 2018-01-01 DOI: 10.15761/CCRR.1000404
W. Nyhan
Molybdenum cofactor deficiency is a rare condition characterized by brain dysfunction (encephalopathy) that worsens over time. Babies with this condition appear normal at birth, but within a week they have difficulty feeding and develop seizures that do not improve with treatment (intractable seizures). Brain abnormalities, including deterioration (atrophy) of brain tissue, lead to severe developmental delay; affected individuals usually do not learn to sit unassisted or to speak. A small percentage of affected individuals have an exaggerated startle reaction (hyperekplexia) to unexpected stimuli such as loud noises. Other features of molybdenum cofactor deficiency can include a small head size (microcephaly) and facial features that are described as "coarse."
钼辅助因子缺乏症是一种罕见的以脑功能障碍(脑病)为特征的疾病,随着时间的推移而恶化。患有这种疾病的婴儿在出生时看起来很正常,但在一周内,他们就会出现进食困难,并出现癫痫发作,这种症状得不到治疗(顽固性癫痫发作)。大脑异常,包括脑组织恶化(萎缩),导致严重的发育迟缓;受影响的人通常无法学会独自坐着或说话。一小部分受影响的人对意外的刺激(如大声的噪音)有夸张的惊吓反应(过度兴奋症)。钼辅助因子缺乏的其他特征包括头小(小头畸形)和面部特征被描述为“粗糙”。
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引用次数: 0
Metastatic Clear Cell Renal Cell Carcinoma with an Unknown Primary in a Living Donor Kidney Transplant Recipient 肾移植活体供者中原发不明的透明细胞转移性肾细胞癌
Pub Date : 2018-01-01 DOI: 10.15761/CCRR.1000415
Ahmed Elfadaly, Nelish S Ardeshna, O. Ekwenna, J. Ortiz
A 68-year-old male presented to the emergency department with severe epigastric pain six months post living donor renal transplant from his daughter-in-law. The patient had an uncomplicated transplant course and was in his usual state of health until the onset of fatigue and constant abdominal pain one week prior. He also reported weight loss, decreased appetite, and night sweats. Past medical history included hypertension, diabetes type II, basal cell carcinoma, and coronary artery bypass surgery. The physical exam was unremarkable except for jaundice and pallor. An abdominal CT scan, colonoscopy, and esophagogastroduodenoscopy were performed several days earlier at a different facility and did not show acute pathology. He was admitted for further workup due to the severity of the abdominal pain. Abstract
一名68岁男性在接受儿媳活体肾移植6个月后,因严重的上腹部疼痛而就诊于急诊科。患者的移植过程简单,健康状况正常,直到一周前出现疲劳和持续腹痛。他还报告体重减轻、食欲减退和盗汗。既往病史包括高血压、II型糖尿病、基底细胞癌和冠状动脉搭桥手术。体检除了黄疸和脸色苍白外,没有什么特别之处。腹部CT扫描、结肠镜检查和食管胃十二指肠镜检查于几天前在另一家医院进行,未显示急性病理。由于腹痛严重,他入院接受进一步检查。摘要
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引用次数: 2
Rupture of umbilical cord at birth: an unusual case of acute neonatal haemorrhage 出生时脐带破裂:一个罕见的急性新生儿出血病例
Pub Date : 2018-01-01 DOI: 10.15761/ccrr.1000433
L. Petris, M. Sabatini, E. Ruffini
Onset of labor was spontaneous as well the membrane rupture with appearance of large amount of clear amniotic fluid. Fetal heart tones remained strong and regular until the delivery that occurred after two hours from the onset of labor and no signs of fetal distress were recorded. The fetal head engaged in the left occiput transverse position and afterwards it rotated easily to right occiput anterior position until the cervix was fully dilatated. One umbilical cord knot was around the fetal neck, however the fetus was delivered with no traction. During the expulsion phase of the labor a complete spontaneous severance of all the three umbilical vessels occurred at fetus side while umbilical cord was regularly attached to the placenta side.
产程是自发的,胎膜破裂,出现大量清澈的羊水。胎心音一直保持强烈和规律,直到分娩后两小时发生的分娩,没有记录胎儿窘迫的迹象。胎头位于左枕横位,之后容易旋转至右枕前位,直至宫颈完全扩张。胎儿颈部有一个脐带结,但胎儿在没有牵引的情况下分娩。在产程排出期,胎儿侧的三根脐带血管完全自发断裂,而脐带有规律地附着在胎盘侧。
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引用次数: 1
Systemic lupus erythematosus presenting as generalized chorea: case report and review of literature 表现为全身性舞蹈病的系统性红斑狼疮:病例报告及文献回顾
Pub Date : 2018-01-01 DOI: 10.15761/CCRR.1000391
M. Abdalla, Ahmad O Ahmad, Husam Altahan, Ibrahim A AlOraini, Abdulrahman Altahan
Neuropsychiatric systemic lupus erythematosis (NPSLE) is one of the main diagnostic criteria of systemic lupus erythematosis (SLE), occurring in 25% 75% of affected patients [1,2]. Chorea is recognized as one of the clinical manifestations of NPSLE especially in children, however it is very rare to occur as the first presenting feature of SLE [3]. In this report we describe a teenage girl who presented acutely with generalized chorea and was found later on to have SLE. Her case will be described in detail and the topic of NPSLE will be reviewed.
神经精神性系统性红斑狼疮(NPSLE)是系统性红斑狼疮(SLE)的主要诊断标准之一,发生率为25% ~ 75%的患者[1,2]。舞蹈病被认为是NPSLE的临床表现之一,尤其是在儿童中,但很少作为SLE b[3]的首发表现。在这个报告中,我们描述了一个十几岁的女孩,她表现出急性全身性舞蹈病,后来被发现有SLE。她的案例将被详细描述,NPSLE的主题将被回顾。
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引用次数: 0
Intravascular papillary endothelial hyperplasia with pseudoepitheliomatous hyperpasia 血管内乳头状内皮增生伴假上皮瘤性增生
Pub Date : 2018-01-01 DOI: 10.15761/CCRR.1000424
I. Ponniah
Received: June 18, 2018; Accepted: June 24, 2018; Published: June 30, 2018 A 42-year old apparently healthy male presented with the complaints of a swelling and bleeding. History revealed no significant findings except that he was a smoker for 10-years. On examination, a small mucosal elevation of 1 x 1 cm in size with small pin point openings was observed in the right side of the palate close to the midline raphe. Under the clinical impression of necrotizing sialometaplasia, the lesion was excised for histopathological evaluation. The microscopy showed keratinized surface epithelium with pseudoepitheliomatous hyperplasia and dilated endothelial cell lined vascular spaces with adjacent mucous minor salivary glands. High power examination of the vascular spaces revealed projection of the vessel wall into the lumen and covered by endothelial cells with admixed fibrin mesh. The vascular pathology also involved the adjacent minor salivary gland lobules (Figure 1). The differential diagnosis includes hemangioma and necrotizing sialometaplasia. The capillary size dilated blood vessel would indicate hemangioma but the tufted papillary projections are not typical of it. The presence of pseudoepitheliomatous hyperplasia may be confused with the more common necrotizing sialometaplasia, but the latter also show acinar degeneration with preservation of the lobular architecture, squamous metaplasia and inflammation. The present microscopic features are consistent with intravascular papillary endothelial hyperplasia.
收稿日期:2018年6月18日;录用日期:2018年6月24日;一名42岁的健康男性出现肿胀和出血的主诉。病史显示,除了他吸烟10年之外,没有其他重大发现。检查时,在上颚右侧靠近中线处可见1 × 1 cm大小的小粘膜隆起,并有小针状开口。在临床表现为坏死性唾液化生的情况下,切除病变进行组织病理学评估。镜检显示角化表面上皮伴假上皮瘤增生,内皮细胞扩张,血管间隙与相邻的粘液小唾液腺排列。高倍镜血管间隙显示血管壁投射到管腔内,并被混合纤维蛋白网的内皮细胞覆盖。血管病变还包括邻近的小唾液腺小叶(图1)。鉴别诊断包括血管瘤和坏死性唾液化生。血管扩张的毛细血管大小提示血管瘤,但簇状乳头状突起不是典型的血管瘤。假性上皮瘤性增生可能与更常见的坏死性唾液化生混淆,但后者也表现为保留小叶结构的腺泡变性、鳞状化生和炎症。目前的显微镜特征与血管内乳头状内皮增生一致。
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引用次数: 0
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Clinical case reports and reviews
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