Acute disseminated encephalomyelitis: the importance of early magnetic resonance imaging

The diagnosis of acute disseminated encephalomyelitis (ADEM) is frequently missed or delayed with consequent delay in instituting therapy in the crucial phase of the illness. The role of MRI in the diagnosis of ADEM is well established, however, the value of its early utilization of treatment on the outcome of patients has not been adequately stressed. Three patients with ADEM are described. Delay in the diagnosis of the first was associated with severe sequelae, while in the other two early diagnosis and institution of corticosteroid therapy which was facilitated by MRI, was associated with a better outcome. MRI should be carried out early once the diagosis of ADEM is entertained.     

尖吻蝮蛇毒引起类DIC病因的研究

本文借助 DEAE-sephadex A-50和 Sephadex G-75柱层析从尖吻蝮蛇毒(AAV)内分离并纯化出一种类凝血酶(TLE).这种 TLE 以1.5U/Kg 体重注入麻醉狗的静脉后,观察到血凝筛选试验 Fgn、BPC、PT、TT 和血清 FDP 全部异常;但凝血因子Ⅱ(Quick 二期法)、AT-Ⅲ以及纤维结合蚤白(Fn)含量仍在正常范围内.这提示实验狗血循环内未有凝血酶形成的证据,从而说明 AAV-TLE是 AAV 中毒患者并发类 DIC 综合征的主要病因.     

Disseminated histoplasmosis with oral manifestation

Histoplasmosis is a fungal disease that affects humans and is caused by Histoplasma capsulatum. The presentation of the infection may be acute, chronic, or disseminated. The disseminated form has extrapulmonary manifestations which may include oral manifestation a. A patient with AIDS sought treatment and he had disseminated histoplasmosis with oral manifestations. The purpose of this case report and literature review is to emphasize the role the dental team has in the diagnosis of disseminated disease when a patient presents with oral manifestations associated with the disease. This case report is clinically relevant because it is not uncommon for oral manifestations to be one of the first signs of systemic disease.     

Acute severe combined demyelination

We present a second case in which Guillain-Barré syndrome (GBS) and acute disseminated encephalomyelitis (ADEM) appeared simultaneously, both in acute and fulminant form. The patient, a 10-year-old girl, presented with acute onset of coma and flaccid, areflexic quadriparesis. The elevated CSF protein levels and delayed F waves fulfilled the criteria of GBS and an MRI study revealed extensive multifocal demyelination compatible with a diagnosis of ADEM. Prompt clinical response followed by complete recovery was achieved by treatment with corticosteroids. It is suggested that acute severe combined demyelination might constitute a separate entity in which the demyelinating process, involving simultaneously the central and the peripheral nervous systems, indicates immune response against a component of the myelin of one system carrying cross-antigenicity with the other.     

Gadolinium ring enhancement and mass effect in acute disseminated encephalomyelitis

A 9-year-old boy presented with a subacute history of optic neuritis followed by brainstem involvement, with fever and a lymphocytic pleocytosis in the cerebrospinal fluid. Gadolinium-enhancing ring lesions were demonstrate in the white matter of the cerebrum, brainstem and cerebellum on day 17 of the illness, all appearing simultaneously as part of a monophasic illness. A parietal lesion exerted mass effect. Needling and biopsy yielded no evidence of a pyogenic lesion, tumour or tuberculosis and showed vasculitis. There was insufficient material for myelin staining. Dexamethasone therapy lead to rapid improvement of the radiological lesions: MRI and CT on day 34 of the illness showed complete clearing of the lesions except for residual abnormality at the biopsy site.     

全反式维甲酸治疗急性早幼粒细胞白血病20例临床疗效观察

单用全反式维甲酸(RA)诱导治疗急性早幼粒细胞白血病(APL)20例,完全缓解率达90%,诱导天数短(中位数为32d)。治疗过程中未发生DIC,亦无因强烈化疗引起骨髓抑制后造成的感染和出血的危险。该药的副反应轻,患者均能耐受。该组的中位生存期已达630d,可望长期存活。     

Detection of bloodstream pathogens in a bacille Calmette-Guérin (BCG)-vaccinated pediatric population in Malawi: a pilot study

Children in Malawi receive bacille Calmette-Guérin (BCG) vaccination within the first 3 days of life. Thus, we hypothesized that Malawian children infected with the human immunodeficiency type 1 virus (HIV-1) might be particularly vulnerable to dissemination of the BCG Mycobacterium bovis strain with which they were vaccinated. Following informed consent by parents, we studied children admitted to a Malawi general hospital during the 1998 wet and dry seasons. Blood from cohorts of acutely ill children was cultured for bacteria, including mycobacteria, and fungi, and tested for anti-HIV-1 antibodies. It was shown that non- typhi Salmonella and Escherichia coli were the predominant bloodstream pathogens during the wet and dry seasons, and that bloodstream dissemination of the BCG M. bovis strain is uncommon in HIV-1-infected children who receive the BCG vaccine.     

新生儿硬肿症播散性血管内凝血和肺出血的研究及其临床意义

目的 :探讨不同病情硬肿症播散性血管内凝血 (DIC)的出凝血功能改变 ,为合理治疗提供理论依据。方法 :比较观察组 10 0例和对照组 5 0例 5项出凝血指标检测结果。结果 :观察组不同病情之间比较 :凝血酶原时间 ,3P试验 ,血小板计数和外周血红细胞形态无显著性差异 (P均 >0 0 5 ) ;试管法凝血时间比较 ,有显著性差异 (P <0 0 5 )。观察组与对照组 5项指标比较有非常显著性差异 (P <0 0 1)。结论 :新生儿硬肿症存在播散性血管内凝血的出凝血功能改变 ,病情越重改变越明显 ,越易合并DIC、肺出血 ,病死率也越高 ,硬肿症早期血液多呈现高凝状态 ,DIC的晚期多呈现低凝状态。早期肝素治疗 ,不仅可改变血液的出凝血状态 (高凝状态 ) ,而且是防治DIC ,肺出血 ,提高治愈率的有效途径。治疗硬肿症加强综合支持疗法 ,尤其是维持内环境稳定纠正酸中毒切不可忽视 ,否则肝素在酸性环境中灭活失效