儿童髓母细胞瘤的综合治疗

目的 探讨儿童髓母细胞瘤（MB）的综合治疗方法及预后。方法 59例MB患儿均行头部CT或MRI检查后行手术切除肿瘤，病理证实。结果 本组52例肿瘤全切除，6例次全切除，1例部分切除。术后症状明显改善56例，3例死亡。结论 对小儿脑髓母细胞瘤尽量应做到手术全切除，解除脑脊液循环梗阻。术后辅以放疗等可明显减少或延缓肿瘤复发，改善患儿生活质量，延长生存期。     

21例儿童髓母细胞瘤的术后护理体会

髓母细胞瘤绝大多数见于儿童，发病高峰在10岁之前。肿瘤多发于小脑蚓部，由于肿瘤生长速度快，常在短期内出现颅内压增高症状及小脑和脑干功能受损症状。若能早期手术并术后精心护理，对于减少并发症及改善生活质量具有重要意义。2004年1月～2005年1月，我院手术治疗21例儿童髓母细胞瘤。现总结术后护理体会如下。     

以关节炎起病的神经母细胞瘤一例

儿童恶性肿瘤可以表现为关节痛或关节炎,神经母细胞瘤是儿童常见的恶性肿瘤之一,主要见于幼儿。由于其临床表现复杂,病变部位隐匿,早期无症状,原发肿瘤及肿瘤转移的临床表现各异,容易误诊。本文报告一例以关节炎为突出表现的少年神经母细胞瘤。患者男,15岁,因“左膝关节肿痛伴发热2个月”入院。     

科学家深挖表观遗传组鉴别出引发髓母细胞瘤的关键原因

正近日,来自圣犹大儿童研究医院的研究人员通过研究鉴别出了引发脑部肿瘤亚型4组髓母细胞瘤的细胞,该研究或为开发新型靶向疗法抵御常见脑部肿瘤提供一定帮助和希望。研究人员利用分析工具Chip-Seq,基于表观遗传调节子的活性鉴别并且追踪了不同亚型髓母细胞瘤的差异性,这些表观遗传调节子包括一些已知的主要调节转录因子,其可以结合名为增强子及超级增强子的DNA序列,这种主要的调     

RFC在MTX治疗急性白血病中的作用及研究进展

甲氨蝶呤(MTX)是经典的抗叶酸代谢药物，主要用于肿瘤及风湿性疾病的治疗。近年来，大剂量MTX在防治儿童急性白血病髓外浸润和全身巩固治疗方面取得了极大的成功，但部分淋巴系白血病及髓系白血病却存在对MTX的原发及获得性耐药。研究证实，还原叶酸载体(RFC)表达缺失及功能障碍是导致MTX耐药、白血病复发的机制之一。现就RFC在MTX治疗急性白血病中的作用及研究进展综述如下。     

儿童结核性淋巴结支气管瘘23例临床分析

目的探讨儿童结核性淋巴结支气管瘘的发生原因、早期诊断、治疗方法及影响预后的相关因素。方法对23例患儿确诊有淋巴支气管瘘的临床资料进行回顾性分析。结果痊愈16例,并发症6例,死亡1例。早期诊治疗效较好。纤支镜是诊断和治疗的重要手段。结论原发肺结核肺门淋巴结肿大液化坏死穿透气管壁,形成淋巴结支气管瘘,坏死物经瘘口沿气道播散发生支气管内膜结核,易致管腔狭窄或闭塞并发肺不张。预后与早期诊断、合理治疗等因素有关。     

成人肝母细胞瘤的诊治及研究进展

成人肝母细胞瘤在临床上十分罕见,其发病机制尚不明确,目前对该疾病的总体认识不足,临床上极易误诊。介绍了肝母细胞瘤的发病机制、临床表现、影像学及病理学特点等内容,重点对成人肝母细胞瘤的治疗方式选择及最新的生物治疗研究进展作出了初步的阐述。通过对各种治疗方式的比较,认为目前治疗该疾病最佳的方案是以外科根治性切除手术为基础的综合治疗。但是由于该疾病恶性程度高,治疗经验匮乏,总体疗效仍不满意。最新的生物治疗靶点的相继报道可能为其治疗带来新的突破,但其临床疗效尚需检验,且成人肝母细胞瘤的发病机制是否与儿童一致也未有研究证实,指出还需进一步的临床研究加以证实和完善。     

白瞳症的鉴别诊断(附2例误诊病例)

白瞳症是由于晶状体、玻璃体、视网膜的白色混浊或肿物遮闭了正常眼底反光而出现的一种临床现象。常见于:①先天性白内障;②视网膜母细胞瘤;③Coat's病;④玻璃体脓肿或眼内炎;⑤晶状体后纤维增生症;⑥永存性增生性原发玻璃体。后几种在鉴别诊断上有一定困难,尤其是病史和体征不典型的视网膜母细胞瘤与Coat's病,往往在眼球摘除术后行病理检查才能确诊。由于视网膜母细胞瘤与Coat's病的治疗手段及预后截然不同,所     

儿童与老年血行播散性肺结核临床比较

血行播散性肺结核是一种病情十分严重的结核病,及时诊断、治疗是关键,为了解儿童与老年血行播散性肺结核在起病方式、病变类型、合并肺外结核病情况,本文就两组临床资料予以分析.     

髓母细胞瘤的临床特征与手术入路选择

目的 探讨儿童髓母细胞瘤(MDB)的临床特征和手术入路的选择.方法 回顾性分析自2011年1月至2019年12月采用经小脑延髓裂(CMF)入路治疗的32例MDB患儿的临床资料,其中经单侧CMF入路28例,采用双侧CMF入路4例.分析患儿的临床特征及预后.结果 发生于<10岁的儿童28例(87.5％),其中<3岁的婴幼儿...     

艾滋病合并结核性脑膜炎脑脊液置换联合鞘内注射的短期疗效观察

目的评价艾滋病合并结核性脑膜炎患者脑脊液置换联合鞘内注射的短期疗效。方法将患者随机分为常规治疗组和联合治疗组,各30例。常规治疗组予以异烟肼、利福平、吡嗪酰胺、乙胺丁醇等抗结核药物治疗。联合治疗组在常规抗结核药物治疗的基础上加用脑脊液置换联合鞘内注入异烟肼及地塞米松。分别记录2组患者临床症状及脑脊液变化情况。治疗2个月后,比较2组疗效差异。结果联合治疗组总有效率明显优于常规治疗组(P0.05)。结论艾滋病合并结核性脑膜炎患者脑脊液置换联合鞘内注射安全性良好,治疗效果明显。     

Rare case of pancreatic cancer with leptomeningeal carcinomatosis

Leptomeningeal carcinomatosis occurs very rarely in patients with pancreatic cancer.Leptomeningealcarcinomatosis is characterized by multifocal seeding of the leptomeninges by malignant cells that originate from a solid tumor.To the best of our knowledge,brain metastasis from pancreatic cancer is extremely rare.Leptomeningeal carcinomatosis is estimated to occur in 3% to 8% of cases of solid tumors.The clinical manifestation usually involves neurological symptoms,including dizziness,headache,vomiting,nausea,and hemiparesis,symptoms similar to those of meningitis or brain tumors.Diagnostic methods for leptomeningeal carcinomatosis include brain magnetic resonance imaging and cerebrospinal fluid examination.Here,we describe a case of leptomeningeal carcinomatosis in which the primary tumor was later determined to be pancreatic cancer.Brain magnetic resonance imaging findings showed mild enhancement of the leptomeninges,and cerebrospinal fluid cytology was negative at first.However,after repeated spinal taps,atypical cells were observed on cerebrospinal fluid analysis and levels of tumor markers such as carbohydrate antigen 19-9 in cerebrospinal fluid were elevated.Abdominal computed tomography,performed to determine the presence of extracerebral tumors,revealed pancreatic cancer.Pancreatic cancer was confirmed histopathologically on examination of an endoscopic ultrasound-guided fine needle aspiration specimen.     

Thiobarbituric acid reactive substances and vitamin E in serum and cerebrospinal fluid from patients with cerebral apoplexy

Thiobarbituric acid (TBA) reactive substances in serum and cerebrospinal fluid from patients with cerebral apoplexy were determined and their relationship to this disease was studied. TBA reactants in serum were elevated in patients with cerebral apoplexy, but there was no difference between patients with brain tumor or other neurological disease and healthy persons. TBA reactants in cerebrospinal fluid in patients with cerebral aneurysm, cerebral arterial and venous anomalies or intracerebral hematoma, and its levels in patients with cerebral infarction or brain tumor were also higher than in healthy persons. Serum α-tocopherol levels were elevated in patients with cerebral infarction, but were relatively low in patients with cerebral aneurysm, cerebral arterial and venous anomalies or intracerebral hematoma. The reverse was the case with cerebrospinal fluid levels of α-tocopherol. That is, the sequence of decreasing level of α-tocopherol in cerebrospinal fluid was intracerebral hematoma, cerebral aneurysm, cerebral arterial and venous anomalies, and cerebral infarction. The levels of TBA reactive substances in patients with cerebral apoplexy showed a positive correlation between serum and cerebrospinal fluid, and the levels of α-tocopherol showed a similar correlation.     

A life-threatening central nervous system-tuberculosis inflammatory reaction nonresponsive to corticosteroids and successfully controlled by infliximab in a young patient with a variant of juvenile idiopathic arthritis

We report a young patient with a variant of juvenile idiopathic arthritis, who, after 4 years of infliximab treatment, developed miliary tuberculosis (TB) with central nervous system involvement (meningitis and multiple tuberculomas). After anti-TB treatment, clinical and radiologic responses were observed, but severe cerebrospinal fluid and brain inflammatory reaction, nonresponsive to corticosteroids, persisted. It was considered a life-threatening paradoxical reaction based on initial cerebrospinal fluid isolation of Mycobacterium tuberculosis fully sensitive to primary anti-TB drugs. After 4 months in the hospital, infliximab was administered considering that infliximab is a potent tumor necrosis factor α inhibiting agent that participates in the formation and preservation of granulomas and may help to modulate the exaggerated cell-mediated immune response against mycobacterial antigens. Clinical complications associated to brain inflammation resolved, and after 3 years of follow-up, the patient remains self-sufficient without neurologic sequels.     

Intracranial dissemination of an ACTH secreting pituitary neoplasm--a case report and review of the literature.

A 52-year-old woman developed recurrent hypercortisolism 3 yr after successful surgical treatment of Cushing's disease. At postmortem, eosinophilic pituitary tissue showing positive ACTH immunohistochemical staining was present in the frontal lobe and cerebellum but there was no tumor in the sella. In the absence of a pituitary tumor, extrasellar ACTH producing tissue could arise from seeding of the cerebrospinal fluid with tumor cells at the time of operation or from an atypical pituitary carcinoma. In this report we review the mechanisms of intracranial dissemination of pituitary tissue and ACTH-secreting pituitary carcinomas, including parasellar invasion, meningeal seeding, and cerebrospinal and hematogenous spread.     

Fluconazole levels in serum and cerebrospinal fluid according to daily dosage in patients with cryptococcosis and other fungal infections

Fluconazole is extensively used for the treatment of candidiasis and cryptococcosis. Among other factors, successful treatment is related to appropriate fluconazole levels in blood and cerebrospinal fluid. In the present study, fluconazole levels were determined in 15 patients, 14 of whom had AIDS and 13 had neurocryptococcosis. The only selection criterion was treatment with fluconazole, which was performed with a generic or similar form of the drug. Fluconazole level was determined by high performance liquid chromatography and the susceptibility profile of Cryptococcus spp. isolated from the patients was assessed by broth microdilution. Blood and cerebrospinal fluid fluconazole levels were found to be related to the fluconazole daily dose, and exceeded the minimum inhibitory concentration of this antifungal for the Cryptococcus spp. isolates. A good correlation was observed between serum and cerebrospinal fluid drug concentration. In conclusion, treatment with non-original fluconazole under usual medical practice conditions results in appropriate blood and cerebrospinal fluid levels of the drug for inhibiting Cryptococcus spp. susceptible to this antifungal drug. The relatively common failures of neurocryptococcosis treatment appear not to be due to insufficient fluconazole levels in the cerebrospinal fluid, especially with the use of daily doses of 400–800 mg.     

Carcinoid carcinomatous meningitis

A 67-year-old woman with metastatic carcinoid tumor developed neurologic signs and symptoms of left facial paralysis and progressive lower extremity weakness. Results of cytologic evaluation of cerebrospinal fluid were normal. However, biochemical analysis of the cerebrospinal fluid showed extreme elevations of 5-hydroxyindoleacetic acid and serotonin, consistent with carcinomatous meningitis caused by carcinoid tumor. Subsequent contrast myelography confirmed the presence of meningeal metastases.     

脑脊液免疫球蛋白作为神经梅毒诊断和愈后判断指标的研究

目的探讨脑脊液免疫球蛋白对神经梅毒的诊断和愈合判断的价值。方法回顾36例多次住院神经梅毒临床和实验室检查等资料,采用SPSS 17.0进行统计分析。结果36例神经梅毒患者中无症状神经梅毒13例,麻痹性痴呆11例,脑膜血管梅毒5例,脊髓痨5例,脑膜神经梅毒2例。初次检查脑脊液免疫球蛋白(Ig)IgG、白细胞、IgM、IgA和蛋白升高分别为35例(97.2%)、30例(83.3%)、29例(80.6%)、26例(72.2%)、25例(69.4%)。血清甲苯胺红不加热试验(TRUST),脑脊液TRUST、IgA、IgG、IgM、白细胞、蛋白皆较治疗前降低,差异有统计学意义(P<0.05)。结论脑脊液中的免疫球蛋白可以作为神经梅毒的诊断和愈后判断的新指标。     

Cerebrospinal fluid flow abnormalities in patients with neoplastic meningitis. An evaluation using 111indium-DTPA ventriculography

Cerebrospinal fluid flow dynamics were evaluated by 111Indium-diethylenetriamine pentaacetic acid (111In-DTPA) ventriculography in 27 patients with neoplastic meningitis. Nineteen patients (70 percent) had evidence of cerebrospinal fluid flow disturbances. These occurred as ventricular outlet obstructions, abnormalities of flow in the spinal canal, or flow distrubances over the cortical convexities. Tumor histology, physical examination, cerebrospinal fluid analysis, myelograms, and computerized axial tomographic scans were not sufficient to predict cerebrospinal fluid flow patterns. These data indicate that cerebrospinal fluid flow abnormalities are common in patients with neoplastic meningitis and that 111In-DTPA cerebrospinal fluid flow imaging is useful in characterizing these abnormalities. This technique provides insight into the distribution of intraventricularly administered chemotherapy and may provide explanations for treatment failure and drug-induced neurotoxicity in patients with neoplastic meningitis.