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神经纤维瘤(neurofibromatosis,NF)是一种渐进性、可侵害全身各系统的常染色体显性遗传性疾病[1]。NF分为4种类型,Ⅰ型神经纤维瘤(NF1)最多见,也被称作Yon Reek1inghausen病,约占NF的90%,临床上主要表现为皮肤牛奶咖啡色斑和多发神经纤维瘤[2]。NF1并发血管病变发生率约为 相似文献
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神经纤维瘤是一种少见的常染色体显性遗传性疾病(又称冯雷克林霍增氏病),是由畸变显性基因引起的神经外胚层和中胚层组织发育异常所致,常见NFI型,发病率为1/3500.其病变特点为全身大面积皮肤牛奶咖啡斑和神经纤维瘤样皮肤肿瘤及神经纤维瘤[1,2].常可累及神经、肌肉、骨骼、内脏、皮肤等部位,因其发病部位不同,故临床症状差异较大[3].尽管手术不能阻止其再生长、复发,但考虑到影响患者生理功能和容貌特征及病情发展较快,目前仍以外科手术治疗为主.我院于2010年3月收治1例全身多发性神经纤维瘤患者,经手术后精心的治疗和护理,患者己痊愈出院.现报道如下. 相似文献
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神经纤维瘤病(neurofibromatosis,NF)是一种常染色体显性遗传性疾病,是由染色体基因突变所造成的。神经纤维瘤起源于神经外膜、神经束膜或神经内膜,可发生于体表的任何部位,大多分布于真皮或皮下等浅表组织,严重者可侵及内脏及骨骼等深部组织。生长于躯干及四肢的瘤体常随年龄增长而缓慢生长。常表现为生长缓慢的无痛性结节或肿块,当肿块增大到一定程度,往往导致局部和邻近器官的下坠移位。我科收治1例背部巨大神经纤维瘤并脊髓损伤的病人,予以行背部巨大恶变神经纤维瘤切除术,术后伤口愈合后出院。现介绍如下。 相似文献
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神经纤维瘤(neurofibromatosis,NF)又称芮克林病,为全身显性遗传病,是一种不多见的病症。NF是由神经鞘细胞及成纤维细胞组成的良性肿瘤。手术是治疗NF的唯一有效方法。2006年12月收治1例头颈部神经纤维瘤患儿,现将其护理报告如下。 相似文献
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[目的]总结Ⅰ型巨大丛状神经纤维瘤病人的围术期护理。[方法]对17例Ⅰ型巨大丛状神经纤维瘤病人根据不同部位采取瘤体切除+不同的皮瓣修复术,加强术前心理护理、完善术前相关准备,术后严密观察病情、做好术后大出血护理、创面管理、营养支持、防治相关并发症等。[结果]病人手术均成功,皮瓣全部成活。[结论]加强Ⅰ型巨大丛状神经纤维瘤病人的围术期护理是手术成功的保证。 相似文献
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1例马凡氏综合征Bentall术的护理 总被引:1,自引:3,他引:1
马凡氏综合征是一种少见的染色体显性遗传性的全身结缔组织性疾病。临床主要特征为眼睛晶状体异位或近视,骨骼系统改变为肢体过长、蜘蛛状指等,心血管的病变主要为主动脉瘤及夹层动脉瘤,可伴有主动脉瓣关闭不全等,约30%~60%的病例可侵犯心血管系统。此病主要的死亡原因为扩张的主动脉瘤夹层破裂及主动脉环扩大致主动脉瓣严重反流而造成的难以控制的心力衰竭。手术是治疗该病的唯一手段,而bentall手术(升主动脉、主动脉瓣替换、冠状动脉移植术)是外科治疗主动脉根部动脉瘤的首选方式。2003年12月28日我院收治一例马凡氏综合征患者,经Bentall手术后,患者生活质量明显提高,报道如下。 相似文献
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肺大泡(pulmonary bulla)是各种原因导致的肺泡内压力升高,肺泡壁破裂互相融合,最后形成巨大的囊泡状改变.肺大泡可单发或多发,其差距较大,大者可占据整个肺叶.多个小的肺大泡有融合成一个或更大的肺大泡趋势.肺大泡可占据一个肺叶甚至整个胸腔,压迫周围肺组织,造成余肺膨胀不全,影响肺功能.往往因剧烈咳嗽或运动,肺内压骤然升高,肺大泡突然破裂形成自发性气胸[1].慢性阻塞性肺疾病(COPD)伴有肺大泡病人常因正压通气致多发性肺大泡破裂,导致气胸,从而列为机械通气的相对禁忌证,因此护理此类疾病有一定难度,我科2016年3月28日收入1例肝癌术后突发肺大泡破裂的病人,在重症监护室(ICU)治疗及护理后于4月12日脱机拔管好转转出ICU.现将护理总结如下. 相似文献
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Dohi O Hatori M Ichinohasama R Hosaka M Hashimoto S Kokubun S 《The Tohoku journal of experimental medicine》2006,208(2):169-176
We experienced two unusual cases of diffuse large B-cell lymphoma (DLBCL), which occurred in a patient with neurofibromatosis type I and a patient with neurofibromatosis type II. B-cell lymphoma is one of the most common phenotypic subgroups of malignant lymphoma. Neurofibromatosis I is characterized by café-au-lait spots and neurofibromas. Neurofibromatosis II typically consists of bilateral schwannomas of the acoustic nerve. Malignant lymphomas rarely coexist with neurofibromatosis I, and no coexistence with neurofibromatosis II has been reported. The patient with neurofibromatosis I was a 50-year-old Japanese woman, clinically manifesting von Recklinghausen's disease since infancy, who noticed an egg-sized tumor in her shoulder. The patient with neurofibromatosis II was a 39-year-old Japanese man who noticed multiple soft tissue tumors in his neck, buttock, and elbow. Biopsied materials from both cases were examined by multiparameter methods, including flow cytometry. Flow cytometry revealed large-scale cells to be tumor cells, and they were positive for CD19, CD20, and CD22. Both patients received chemotherapy, and the tumors disappeared. The patient with neurofibromatosis I was alive without recurrence at 4 years after treatment, while the patient with neurofibromatosis II died of recurrence. To the best of our knowledge, this is the first case of malignant lymphoma arising in a neurofibromatosis II patient. As for neurofibromatosis I, there were some reports about occurrence of malignant lymphoma. It is important to be aware of possibility of association of malignant tumors not only of the nervous system but also of unrelated to the nervous system when tumors appear in neurofibromatosis patients. 相似文献
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Background
Spontaneous arterial bleeding in patients with type 1 neurofibromatosis is uncommon. It can be fatal if not recognized and treated in a timely manner.Objectives
Diagnose and treat an uncommon complication in type 1 neurofibromatosis.Case Report
We report a patient with type 1 neurofibromatosis who bled spontaneously from a pseudoaneurysm arising from a branch of the left thyrocervical artery. The patient was successfully treated with endovascular therapy.Conclusion
Due to the high fragility of involved vessels in patients with type 1 neurofibromatosis, emergent angiography and endovascular therapy should be undertaken without delay, regardless of the presence or absence of contrast extravasation on computed tomography scan. 相似文献13.
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Hiroko Naganuma Masato Funaoka Shusei Fujimori Hiroshi Yoshioka Hiroko Hirano Hideaki Ishida Tomoya Komatsuda Mamiko Yamada Yuji Nishikawa 《Journal of Medical Ultrasonics》2007,34(2):107-111
We report a case of neurofibromatosis type 1 (NF1) complicated by a malignant triton tumor (MTT), with an emphasis on B-mode
sonographic (US) and contrast-enhanced US (CEUS) findings. To the best of our knowledge, this is the first report describing
CEUS findings of MTT. The mass was poorly demarcated and composed of an internal echogenic area and an outer hypoechoic zone.
CEUS findings showed the outer zone to be strongly enhanced, and the internal area was very poor in blood flow because of
necrotic tissues. 相似文献
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脊柱骨折最严重的并发症是脊髓损伤,而颈椎骨折合并颈髓损伤因其损伤平面高,并发症多,所以致残率高,死亡率也高.手术是颈椎骨折的主要治疗手段.各种并发症防治和护理是降低此类病人死亡率的关键,也是改善病人预后和提高其生存质量的有效手段.
1临床资料
我院2008年1月-2011年6月共收治8例颈椎骨折合并脊髓损伤病人,均为男性,年龄37岁~59岁,平均45.25岁;住院天数15d~73d,平均36.52d;其中5例完全损伤,3例不完全损伤.病人均采用气管插管全身麻醉,有5例行颈椎前路手术,3例行颈椎后路手术,术后均戴颈围托固定4周~8周. 相似文献