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新英格兰:口内黑素瘤

一60岁女性口腔钝挫伤后上牙龈出现黑色变2月。患者既往体健,无吸烟史和饮酒史。体检示上颌前牙唇侧(图A)和腭侧(图B)可及弥漫性的黑素色素沉着,无牙齿松动,未及淋巴结肿大。X线未见骨质破坏,PET-CT示上颌前牙区可及高代谢信号,未见淋巴结转移。切除活检组织病理学检查示恶性黑素瘤。口腔内的原发性恶黑不常见。不幸的是口腔恶黑早期无明显症状,因此很可能延误诊断。该患者病变切除后进行化疗。诊断后随访26月病情复发且有远处转移。 Intraoral Malignant Melanoma A 60-year-old woman presented with a 2-month history of black discoloration on her upper gums after blunt trauma to the mouth. She had previously been well and was a lifelong nonsmoker and nondrinker. On examination, diffuse black pigmentation was seen on both the labial and palatal sides of the anterior maxilla (Panels A and B, respectively), without tooth mobility or palpable lymphadenopathy. No bone destruction was detected on a standard dental radiograph. A hypermetabolic signal was seen in the anterior maxillary area on positron-emission tomography–computed tomography, with no metastatic spread to the lymph nodes. Incisional biopsy and histologic analysis revealed malignant melanoma. Primary malignant melanoma is uncommon in the oral cavity. ...

新英格兰:肺萎陷

一位终末期肝病的46岁女性因复发性肝性胸水需多次胸腔穿刺,现出现呼吸困难而就诊。胸部检查右肺未及呼吸音,X线检查示右肺液气胸(图A中箭头所示气胸,星号示胸水)。猪尾导管排尽积水后仍持续性气胸(图B箭头所示)。胸部CT检查示肺萎陷(阻塞性肺不张后气胸),胸膜增厚,未见支气管阻塞(图C)。肺萎陷因慢性炎症引起的胸膜增厚阻止了肺复张引起。胸膜壁层不分离,其间充盈液体,从而引起液气胸。排净胸水后引起阻塞性肺不张后气胸的原因是肺不能再扩张到充满胸腔。阻塞性肺不张后气胸的其它原因排除了支气管阻塞。肺萎陷的治疗为手术治疗,切除纤维化的胸膜脏层以利于肺部扩张。该患者行胸腔镜手术切除了胸膜和胸膜固定术后右肺完全复张。 Trapped Lung A 46-year-old woman with end-stage liver disease that was complicated by recurrent hepatic hydrothorax requiring multiple thoracenteses presented with breathlessness. Chest examination found no breath sounds on the right side. Radiography of the chest revealed a hydropneumothorax (Panel A; the arrows indicate pneumothorax, and the asterisk hydrothorax). The hydrothorax was drained with the use of a pigtail catheter, with persistent pneumothorax (Panel B, arrows). A computed tomographic scan of the chest showed trapped lung (pneumothorax ex vacuo) with thickened visceral pleura and no evidence of endobronchial obstruction (Panel C). Trapped lung is due to fibrous visceral ple...

新英格兰:眼结膜黑素瘤

一位78岁女性既往有青光眼史和糖尿病史。患者常规进行白内障检查,发现其右眼出现一红色斑和褐色斑丘疹病变。患者自诉该病变出现已1年,期间病变大小出现变化,偶有出血,但无疼痛、瘙痒或视力改变。行巩膜角膜切除术,并辅以冷冻治疗和外酒精外用。组织病理学检查示恶性结膜黑素瘤,伴原发性获得性不典型黑变病,厚1mm宽4mm。结膜黑素瘤可源于原发性获得性黑变病,常有局部转移,治疗后易复发。该患者辅助治疗有化疗和局部外用丝裂霉素。淋巴检查、骨扫描和头颈、胸腹CT检查未凶转移,治疗8月后未见复发。 Conjunctival Melanoma A 78-year-old woman with a medical history of glaucoma, diabetes, and cataracts presented for a routine ophthalmologic visit, during which she was found to have an erythematous and brown maculopapular lesion in her right eye. She reported that the lesion had been present for approximately 1 year, during which time it had fluctuated in size and bled occasionally, but that it had not been associated with pain, pruritus, or visual changes. An excisional scleral keratectomy was performed, followed by cryotherapy and alcohol application. Histopathological analysis revealed malignant conjunctival melanoma associated with primary acquired melanosis with atypia, measuring 1.0 mm thick and 4.0 mm in diameter. Conjunctival melanoma can ...

新英格兰NEJM:副肿瘤性神经性肌强直

一位70岁男性患有冠心病,慢性阻塞性肺疾病,40年吸烟史,2月来出现肌肉震颤和痉挛,汗多,并且体重减轻了10kg。体检发现肌纤维震颤(非自觉性肌肉颤搐,肌肉痉挛)和肌束震颤(可在此 观看视频 )。肌纤维震颤在睡觉时加重并持续存在。压缩肌肉后未见无力,强直,萎缩或者弛等现象。肌电力示特异性的神经性肌强直和肌纤维震颤放电特征。血清学检查示电压门控钾通道复杂抗体阳性(396pmol/l,正常值<85pmol/l)。根据临床表现,肌电力和血清学检查,诊断为神经性肌强直。这种强直是由于外周神经过度兴奋导致了肌纤维的激活。开始使用卡马西平治疗,但是症状控制不理想。可能是因为神经性肌强直可能是副肿瘤性的,患者有吸烟史和明显的体重减轻史,因此应该排除癌症的可能。锁骨上淋巴结活检显示小细胞肺癌。患者于3月后因化疗并发症而死亡。 Paraneoplastic Neuromyotonia A 70-year-old man with coronary artery disease, chronic obstructive pulmonary disease, and a 40-pack-year smoking history presented to a neurologist with a 2-month history of muscle twitching and spasms, excessive sweating, and a 10-kg weight loss. The physical examination revealed myokymia (involuntary, undulating muscle twitching) and fasciculations (see video ). Myokymia worsened with exercise and persisted during sleep. There was no muscle weakness, rigidity, atrophy, or delayed muscle relaxation after contraction. Needle electromyography showed characteristic neuromyotonic and myokymic discharges...

新英格兰NEJM:慢性肾病引的甲改变

一位59岁女性16年前左肾因多囊肾而进行了肾移植,3年来指甲出现了改变。该患者一直接受强的松,霉酚酸酯和他克莫司的免疫抑制治疗。该患者处于慢性肾闰4期,一年来出现血肌酐水平升高(1.7-2.2mg/dl「150-194umol/l」),血尿毒瘾氮升高(26-28mg/dl 「9.3-13.6mmol/l」)。查体发现患者的所有指甲远端50%的部分甲床呈粉红色,近端出现白色带。Lindsay甲,也叫对半甲,最早见于1967年报道,指慢性肾病患者20-60%的甲床出现红色,粉红色或者褐色。氮质血症水平和甲床之间并无相关性。近端出现白色带可能与慢性贫血有关,褐色带可能与β-黑素细胞刺激激素增多而引起的黑色素沉积增多有关。本病无特殊治疗方法,以治疗原有疾病为主,控制慢性肾病是第一位的。 Lindsay’s Nails in Chronic Kidney Disease A 59-year-old woman who had undergone transplantation of the left kidney 16 years earlier for the treatment of polycystic kidney disease presented with an incidental finding of nail changes that had been present for at least 3 years. She was undergoing treatment with prednisone, mycophenolate mofetil, and tacrolimus for immunosuppression. She had stage 4 chronic kidney disease, with elevated levels of creatinine (1.7 to 2.2 mg per deciliter [150 to 194 μmol per liter]) and blood urea nitrogen (26 to 38 mg per deciliter [9.3 to 13.6 mmol per liter]) during the past year. On examination, all the patient’s fin...

新英格兰NEJM:二尖瓣环状钙化

一位81岁女性因口腔癌术后出现心衰。诊断表明患者围手术期体内水分过多并出现肺栓塞,这导致了患者术后出现心衰。胸片显示心脏部分出现一个大的密集钙化区,与二尖瓣钙化一致。二尖瓣钙化呈半圆形(术前正后位如图A和图B箭头所示,侧位如图C箭头所示)。胸部CT横切面示二尖瓣后外侧出现环形钙化(轴切面如图D箭头所示,短轴切面如图E箭头所示)。胸部超声检查示二尖瓣狭窄和中度返流,左心室舒张功能障碍,左室射心数为69%。老年人的二尖瓣钙化通常通过胸部X线检测到,一般认为是退行性改变,一般无明显临床症状。当二尖瓣钙化较重时,可以引起心脏瓣膜功能障碍(如本例患者),常导致完全性房室传导阻滞,二尖瓣返流,有时也会引起二尖瓣狭窄。二尖瓣钙化可能与糖尿病,高血压,高血脂血症,以及继发于肾衰甲状旁腺功能亢进有关。该患者住院期间通过口服药物进行保守治疗,未进行手术治疗,现已失访。 Mitral Annular Calcification An 81-year-old woman was transferred to the medical service with cardiac failure after surgery for oral cancer. A diagnostic workup indicated perioperative overhydration and pulmonary embolism as causative factors for her symptoms. Chest radiography revealed a large, dense area of calcification overlying the heart, consistent with mitral annular calcification. Calcification was semicircular in the location of the mitral valve, as seen on the preoperative posterior–anterior view (Panel A, arrowhead; Panel B, arrow, shows an enlarged view) and a lateral view (Panel C, arrowhead). Comput...

新英格兰NEJM:胺碘酮诱发的轮生状角膜病变

一位50岁的的女性自述双眼可看到光晕数周。患者既往无眼外伤史和角膜屈光手术史。2年前患者口服胺磺酮治疗房颤。体格检查发现双眼矫正视力为20/20,并有轻度散光。眼裂隙灯检查示角膜上皮呈轮生状,即胺碘酮诱发轮生状角膜病的典型表现。眼的其它部分正常。轮生状角膜病是胺碘酮常见的副作用。角膜的改变极少引起视力障碍,但是有些患者可能会看到光晕和色环。胺碘酮其他眼部副作用还有白内障和视神经病变。建议患者不用担心,可以配载太阳镜以减轻光晕症状,另外坚持每年门诊随访。最后一次随访是就诊的12个月后,眼科仅有角膜沉积物,其他一切正常。 Amiodarone-Induced Vortex Keratopathy A 50-year-old woman was referred by her general practitioner after she reported seeing halos around lights through both eyes for a few weeks. She had no history of ocular trauma or previous refractive corneal surgery. She had begun taking amiodarone for atrial fibrillation 2 years previously. On physical examination, her best corrected visual acuity was 20/20 bilaterally. Refraction showed minimal astigmatism. Slit-lamp examination of the anterior segment showed a whorl-like pattern of corneal epithelial deposits bilaterally, characterizing amiodarone-induced vortex keratopathy (also called cornea verticillata). Other ocular structures were normal. Vortex keratopathy is a common side effect ...

新英格兰NEJM:成人会厌炎

一位65岁既往体健女性因咽喉痛,声音低沉和自我感觉发热7天而就诊于急诊科。体格检查示:患者体温正常,血压140/86mmHg,血氧饱和度正常(98%)。患者前颈部轻度肿胀并伴有压痛,颈部淋巴结未触及肿大,患者无流涎。X线示颈外侧软组织「拇指片」影(图A箭头所示),表明患者有会厌肿胀,提示会厌炎。CT示实体肿胀以及会厌部(图B箭头所示),舌根和舌扁桃体水肿。间接喉镜检查示会厌和舌根出现红斑和炎症,从而出现舌扁桃体的肥大,喉部再现痰。因可能出现气道阻塞的可能,给予患者头孢曲松钠和地塞米松治疗,并留观了48小时。患者血培养一直为阴性。第3天患者症状消失,患者可以进食。患者出院后给予头孢呋辛治疗1周。 Epiglottitis in an Adult A previously healthy 65-year-old woman presented to the emergency department with a 7-day history of throat pain, difficulty swallowing, muffled voice, and subjective fevers. On presentation, she was afebrile and hemodynamically stable with normal oxygen saturation (blood pressure, 140/86 mm Hg; oxygen saturation, 98% while the patient was breathing ambient air). Physical examination revealed mild tenderness and swelling in the anterior neck, without cervical lymphadenopathy or drooling. Lateral soft-tissue radiography of the neck showed the “thumb sign” (Panel A, arrow), indicating a swollen epiglottis, suggestive of epiglottitis. Computed tomography of the neck re...

新英格兰NEJM:指纹消失

一65岁女性乳腺癌Ⅳ期患者在银行交易时不能授权,因为系统无法辨认的指纹(图A)。3月来该患者因三阴性乳腺癌(即雌激素受体,孕激素受体和人表皮生长因子受体2缺失的一种乳腺癌)接受卡培他滨和贝伐单抗治疗。在第一个化疗期间,出现1级手-足综合症(掌跖红肿),但是通过局部治疗症状后消失。手-足综合症是某些化疗药物的副作用,以手掌和足部的红肿疼痛为特征。经过第三个化疗疗程,手-足综合症症状加重,并且出现自我护理活动受限。PET-CT示乳腺癌的肺转移减少了50%,患者终止了卡培他滨的治疗,之后又维持在小剂量的治疗水平。患者没有出现更严重的毒副作用,但拇指指纹(图B)和手指指纹(图C)消失了。我们给他提供了一封关于指纹消失的说明信,指出乳腺癌的化疗是造成指纹消失的原因。 Loss of Fingerprints A 65-year-old woman with stage IV breast cancer presented after being denied authorization to perform a banking transaction because her fingerprints were unrecognizable (Panel A). For 3 months, she had undergone treatment for triple-negative breast cancer (a tumor characterized by the lack of expression of estrogen receptor, progesterone receptor, and human epidermal growth factor receptor type 2 [HER2]) with capecitabine and bevacizumab. During the first cycle, grade 1 of the hand–foot syndrome (palmar–plantar erythrodysesthesia) developed and was successfully treated with topical agents. The hand–foot syndrome is a side effect of certai...

新英格兰NEJM:眼前房出血

一33岁男性猪饲养员因双眼出现突发性双眼视模糊、疼痛、发红和畏光而就诊。患者有10天的持续高热史(39.4℃),全身不适和肌肉疼痛史。眼科检查示双侧虹膜睫状体炎(前葡萄膜炎)。泼尼松龙滴眼液和软膏等局部治疗后疼痛和畏光减轻,但第二天左眼前房出现和血(图A 3点示位置有一光反射区,图B 9点位置示前房部分有一明亮区)。实验室检查示γ-谷氨酰转移酶为129U/L (正常值<55U/L),丙氨酸氨基转移酶为49U/L (正常值<45U/L),白细胞计数为11700/ml (正常值范围为4400/ml-11300/ml)。临床和实验室检查结果提示诊断为钩端螺旋体病。血清学检查钩端螺旋体 interrogans serovar serjoe 的IgM和IgG抗体阳性。眼前房出血常因眼外伤引起,但是在极少数病例可引感染引起。经过持续的局部和系统阿莫西林治疗,患者的症状逐渐消退,2周内实验室检查和视力也恢复正常,前房出血和眼部发红也在1月内消失。 Hyphema A 33-year-old man who was a pig farmer presented with sudden blurred vision, pain, redness, and photophobia in both eyes. He had a 10-day history of persistent fever (temperature, 39.4 C°), malaise, and myalgia. Ophthalmologic examination revealed bilateral iridocyclitis (anterior uveitis). Local treatment (prednisolone eyedrops and ointment and cyclopentolate eyedrops) alleviated the pain and photophobia overnight, but the next morning he had hyphema in his left eye (Panel A shows a view with the light-source reflection at the 3 o’clock position, and Pane...

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