Systemic amyloidosis presents a huge amyloidoma in the nasopharynx： A case report

A tumor-like presentation of systemic amyloidosis in the nasopharynx is exceedingly rare. A male of 52-yearold with a recurrent mass in the nasopharynx underwent surgical resection 2 years ago. Immunohistochemical examination revealed M protein band and kappa immunoglobulin light chain. The sections of the biopsy of the mass in the nasopharynx and anterior abdominal wall were stained with Congo red and examined with microscopy. Amyloid deposits were found. It was diagnosed as systemic amyloidoma. Colchicine, melphalan and prednisone were administered. The mass decreased its size in the 8 months of follow-up. This case suggests that a solitary tumor in the nasopharynx might be an early sign of systemic amyloidosis because of the slow progression of the disease. The existence of a solitary amyloidoma in any location of the body should lead the doctor to look for other symptoms and signs of systemic amyloidosis.     

以头痛为首发症状的嗜铬细胞瘤1例报告并文献复习

目的:探讨1例以头痛为首发症状的嗜铬细胞瘤患者的临床表现和诊断方法。方法:记录患者临床症状,利用放射免疫法动态检测患者活性肾素;化学发光法检测患者血中醛固酮水平;高效液相色谱法检测患者血中肾上腺素和去甲肾上腺素水平;CT检查患者肾上腺影像;手术取肾上腺肿瘤行病理学活检和HE染色检测。结果:该嗜铬细胞瘤患者剧烈头痛发作时常伴血压、活性肾素和醛固酮水平明显升高;肾上腺CT扫描可见左肾上腺1个类圆形低密度影,肾上腺CT增强扫描可见该肿块动脉期呈不均匀强化;病理活检HE染色检测,透明和嗜酸性细胞呈巢状及束状排列,出血、坏死明显,部分细胞有异型。结论:嗜铬细胞瘤是一种分泌儿茶酚胺的内分泌肿瘤,应主要结合临床症状、生化、影像和病理确诊。     

罗库溴铵致过敏性休克1例

全身麻醉诱导期间需短时间内应用多种药物,药物起效的同时可能会发生过敏性反应。过敏性反应的临床表现多样,包括皮疹、颜面水肿、呼吸道痉挛、血压急剧下降、心率增快或减慢等,过敏性休克罕见却极其危险,抢救不及时可威胁生命。麻醉状态下,尤其是全身麻醉,患者不能及时提出不适主诉,亦有手术无菌单等覆盖身体,这些因素都会造成麻醉师对过敏反应发现的不及时,从而可能延误治疗。因此,在麻醉过程中应对患者皮肤状态以及监测指标给予足够的重视。 肌松药是全身麻醉用药的重要组成部分,其化学结构中含有的季铵基团是导致麻醉过程中发生过敏反应的常见原因。肌松药相关性过敏反应占全部麻醉相关性过敏反应的60%［1］。本文报告1例麻醉诱导期间发生过敏性休克的病例,并回顾了麻醉期间过敏反应的诊断、处理等相关资料。     

神经纤维瘤病1型相关嗜铬细胞瘤并小肠间质瘤1例报告并文献复习

目的 提高对1型神经纤维瘤病(neurofibromatosis type 1,NF1)相关腹部肿瘤的认识及诊治水平.方法 分析1例嗜铬细胞瘤(pheochromocytoma,PHEO)并有小肠间质瘤(gastrointestinal stromal tumour,GIST)的NF1的临床资料,结合文献复习,检索相似病例,对临床资料、治疗及预后进行分析.结果 男,37岁,CT示右肾上腺占位.因小肠穿孔急诊行剖腹探查,切除部分小肠,病理示小肠GIST.药物准备4周后行腹腔镜右肾上腺肿瘤切除术,病理示组合性PHEO.文献检索到18例,共19例病例,男9例,女10例,平均年龄54岁;肾上腺PHEO9例(9/19)为双侧,5例(5/19)为组合性PHEO;GIST累及胃4例(4/19),小肠16例(16/19),结肠2例(2/19),且大多为多发性病变(13/17),7例(7/7)检测均无KIT和PDGFRA基因突变.手术治疗后多无复发.结论 NF1可同时合并PHEO和GIST,GIST多为多发性病变,患有GIST的NF1接受手术前应排查有无PHEO,PHEO可导致围手术期心血管并发症的高风险.此类疾病手术治疗预后良好.     

Multiple myeloma associated with light-chain amyloidosis manifesting as gastric retention: a case report and review of the literature

We report a case of multiple myeloma associated with light-chain amyloidosis in a 62-year old woman. The patient came to hospital with the main complaint of epigastric pain and gastroscopy showed gastric retention. The patient had been diagnosed with multiple myeloma associated with light-chain amyloidosis after biopsy of the gastric mucosa and bone marrow aspirate. A review of the literature was also performed.     

Childhood bruxism: a clinical review and case report

The present case report refers to a patient who reported to the department with the complaint of teeth grinding (Bruxism). A brief review of the literature is reported concerning the aetiology, clinical diagnosis and the therapeutic approach of the disease.     

欧乃派克静脉注射致过敏性休克1例

1　病例报告　男 ,38岁 ,因怀疑肝脏肿瘤于 2 0 0 1- 12 - 10行CT增强扫描检查 .既往无任何药物过敏史 ,检查前也未做过敏试验 .用 30 0 g· L- 1 欧乃派克 75 m L (挪威奈科明造影剂公司生产 )快速静脉注射 ,2～ 6 m L· s- 1 ,注射后 3min,患者即感胸闷、心慌、气短、大汗淋漓、烦燥不安、呼吸困难、脉搏细弱 ,渐陷入昏睡状态 .查体 :神志不清 ,双侧瞳孔等大等圆 ,对光反射迟钝 ,BP8/5 .3k Pa,P12 0· min- 1 ,R36· min- 1 .立即平卧吸氧 ,保持呼吸道通畅 ,肾上腺素 0 .5 mg,sc;非那根 2 5mg,im;迅速建立两条静脉通道 ,应用血管活性…     

Mitochondrial encephalomyopathy presenting with clinical Leigh's disease: report of a case.

Leigh's disease is one of the mitochondrial encephalomyopathies. This article presents a 7-month-old baby boy who had been well-being since birth until 6 months of age when episodic downward gaze of both eyes with limitation of horizontal eye movement were noted. This episode of cranial nerve palsies lasted about 4-5 days and subsided spontaneously. The second attack was noted one month later, to be associated with hypotonia and truncal ataxia. Episodic hyperventilation with resultant gasping and myoclonus was noted at the third attack but spontaneous respiration resumed soon with persistent ophthalmoplegia and truncal ataxia. Lumbar puncture, brain MRI, amino acid assay and cardiac echo all showed negative finding. The oral glucose lactate stimulation test revealed an elevation of lactic acid, brain stem evoked potential indicated bilateral obscure 4th and 5th waves, and muscle biopsy showed ragged red fibres with aggregation of structurally abnormal mitochondria noted under electron microscope. Coenzyme Q, thiamine and carnitine had been given before biochemical study; however, the neurological symptoms did not show any improvement. Biochemical study finally revealed normal respiratory chain enzymes including NADH-coenzyme Q reductase, succinate coenzyme Q reductase and cytochrome c oxidase while other enzymes were technically unavailable for study. Unfortunately the patient died at 18-month-old due to respiratory failure.     

Multifocal epithelioid hemangioendothelioma: case report of a clinical chamaeleon

Epithelioid hemangioendothelioma is an extremely rare vascular bone tumor with a slow growth and poor prognosis. The term was designed to describe neoplasms that had an appearance in between hemangiomas and sarcomas. Various synonyms for epithelioid hemangioendothelioma are used clinically: low grade anaplastic angiosarcoma, cellular hemangioma, histiocytoid hemangioma and angioendothelioma. However, it represents 1% of all vascular neoplasms and is locally aggressive. We report the course of disease of a 47-year-old man who presented to our clinic with unspecific abdominal and back pain. Radiological findings revealed multiple lesions in the spine as well as liver and spleen involvement. Tumor histology of the bone and liver biopsies confirmed the diagnosis of epithelioid hemangioendothelioma. Although treatment was initiated with thalidomide, the patient developed multiple organ dysfunction syndrome (MODS) and succumbed to his disease. This case report may contribute to the data on clinical findings and natural history of this rare tumor.     

以肝内胆管扩张为首发临床表现的华支睾吸虫病1例报告

华支睾吸虫病因其起病隐匿、发展缓慢、临床表现轻微而不典型,往往得不到早期诊治,在病程晚期则可导致严重并发症.我科收治1例以肝内胆管扩张为首发临床表现的华支睾吸虫病,结合相关文献浅谈早期诊治该病的体会.     

妊娠合并巨乳症1例报告

妊娠期合并巨乳症临床上罕见,多为双侧性,常为永久性病变,体积增大迅速,不能自行消退。常发生于第二次妊娠,下次妊娠时复发,本疾病由于乳房组织受正常激素刺激的异常反应所致,继发坏死、出血和感染者很多见,病死率颇高。巨乳症的确切病因尚不明确,一般认为可能与乳腺组织对内分泌的刺激过分敏感有关,而性激素却在正常范围。