Haemorrhagic shock-encephalopathy syndrome: plasmapheresis as a therapeutic approach

We present the case of a 4.5-week-old boy with acute encephalopathy, shock, intestinal bleeding and disseminated intravascular coagulation. The clinical course and typical laboratory parameters were compatible with a diagnosis of haemorrhagic shock-encephalopathy syndrome (HSE). Immediate shock treatment, repeated haemodialysis and plasmapheresis did not prevent a fatal outcome 4 days after the onset of clinical symptoms.Abbreviations HSE haemorrhagic shock-encephalopathy syndrome - HUS haemolytic uraemic syndrome     

Haemorrhagic shock encephalopathy

Three children suffered from acute onset of convulsions and progressive coma associated with hyperpyrexia, profound shock and generalized bleeding tendency. No causative agent could be identified. Despite aggressive resuscitation they all died. Post-mortem examination revealed cerebral oedema, petechial haemorrhages of the gut, lungs and kidneys, and a generalized depletion of lymphocytes in the lymphoid organs. Features were compatible with haemorrhagic shock encephalopathy, which is a highly fatal disease. The possible role of hyperpyrexia in its pathophysiology is discussed. More careful case identification and research in various possible aetiological factors may help elucidate its pathogenesis.     

Haemorrhagic shock encephalopathy syndrome presenting with myoglobinuria

An infant with haemorrhagic shock encephalopathy syndrome (HSES) who in addition presented with hyperpyrexia and myoglobinuria is reported. As rhabdomyolysis is a feature of heat stroke and malignant hyperthermia, the association of HSES with myoglobinuria supports the hypothesis that HSES may be a form of hypermetabolic state triggered by hyperthermia.     

Haemorrhagic shock encephalopathy syndrome presenting with myoglobinuria.

An infant with haemorrhagic shock encephalopathy syndrome (HSES) who in addition presented with hyperpyrexia and myoglobinuria is reported. As rhabdomyolysis is a feature of heat stroke and malignant hyperthermia, the association of HSES with myoglobinuria supports the hypothesis that HSES may be a form of hypermetabolic state triggered by hyperthermia.     

Haemorrhagic shock encephalopathy syndrome in the British Isles.

The aetiopathogenesis of haemorrhagic shock encephalopathy syndrome (HSES) remains unclear and after concern that a novel environmental agent was the cause, the British Paediatric Association and the Public Health Laboratory Service Communicable Disease Surveillance Centre in 1983 initiated surveillance of this condition in the British Isles. After 1986 cases were ascertained via the British Paediatric Surveillance Unit 'active' reporting scheme; this report presents the findings for 1985-8. Sixty five patients were reported, of whom 52 satisfied the criteria for inclusion. Of those whose outcome was known, 24 (46%) died, 18 had severe neurological damage, and six survived apparently intact. Epidemiological features of note were: the median age of 15 weeks (range 3-140); statistically significant clustering of admission times suggesting a peak onset period at night; lack of geographic clusters, of secular trends and, except for a slight excess in winter months, of seasonality. Clinical and pathological features followed a highly consistent pattern, suggesting that HSES is an individual clinical entity distinguishable from conditions with similar presentations, such as septicaemia and Reye's syndrome. There was no microbiological or epidemiological evidence to support the emergence of a novel environmental agent. Many of the features of HSES were, however, the same as those described in heat stroke and we suggest that the two conditions are the same even though there is usually no history of overt overheating.     

Ascites and encephalopathy in chronic liver disease

Ascites and particularly encephalopathy in the setting of chronic liver disease are traditionally thought to be poor prognostic markers of end stage liver disease. Diagnostic and therapeutic approaches to these complications are based on experience with adult patients, however, differences in the spectrum of aetiologies causing end stage liver disease together with the relative resistance of the pediatric patient to encephalopathy impact on the management of such patients. The following is a review of current thinking in the aetiology and management of ascites and encephalopathy in the setting of chronic liver disease in children.     

脓毒症相关性脑病治疗进展

脓毒症相关性脑病(SAE)是脓毒症进程中常见的并发症,并SAE患者病死率较高,远期认知功能障碍显著影响幸存者的生活质量。其发病机制仍未阐明,缺乏特异性治疗方法,目前仍以脓毒症综合救治为主,多种针对脑病的干预措施尚处于研究阶段。未来研究需明确减少脓毒症大脑炎性反应、保护血脑屏障、抑制神经递质传递异常等病理生理损伤关键点的方法,从而防治SAE患者脑功能永久性损害。     

Cat-scratch disease with reversible encephalopathy

A case of cat-scratch disease (CSD) complicated by reversible encephalopathy is presented. Neurological complications of CSD are uncommon. Laboratory and radiological examinations were negative. There was complete recovery.     

病毒感染相关急性脑病

病毒感染相关急性脑病临床并非少见,严重病例病死率和致残率高,严重影响儿童健康。本文重点介绍常见类型病毒感染相关急性脑病的病因、病理机制、临床特点、危险因素及治疗。     

氨基胍、地塞米松和氨力农对内毒素休克兔肾功能保护作用的比较

目的比较地塞米松(Dex)、氨基胍(AG)和氨力农(Amr)三种作用机制不同的药物对内毒素休克兔肾功能的保护作用。方法40只兔随机均分成5组:手术对照、内毒素(LPS)、Dex、Amr和AG治疗组。分别于术后30min(T0)、注射内毒素并达到休克诊断标准时(T)、用药治疗后1～6h(T1～T6)等各时间点记录血压、心输出量。于T0～T6分别取血、留尿,测血一氧化氮(NO)、血尿素氮(BUN)、肌酐(Scr)和尿视黄醇结合蛋白(RBP)水平。结果AG组尿量自T2始显著高于LPS组,BUN、Scr自T2始显著低于LPS组。Dex组尿量始终与LPS组差异无显著性,而BUN于T6、Scr自T2始显著低于LPS组。Amr组尿量与LPS组差异无显著性,BUN、Scr于T6显著低于LPS组。各治疗组RBP均显著低于LPS组,且AG组RBP亦明显低于Dex和Amr组。实验结束时仅AG组NO含量与LPS组差异有显著性。结论三种药物中AG对肾保护作用最强,Dex次之,Amr最弱。     

脓毒症相关性脑病的生物学标志物研究进展

脓毒症相关性脑病(SAE)是导致脓毒症患者病死率增高的常见并发症,但其发病机制仍不清楚,无公认的诊断标准,缺乏有效的干预措施。随着对SAE病理生理机制研究的不断深入,探索疾病特异的相关生物标志物对寻找有效的诊疗手段有一定意义。     

促红细胞生成素对缺氧缺血性脑病的神经保护作用

研究表明,促红细胞生成素(EPO)对新生儿缺氧缺血性脑病具有神经保护作用,EPO能明显抑制脑缺血诱导的神经细胞凋亡,对缺氧缺血导致损伤的神经元有一定的保护作用.其机制可能包括抑制NO过度合成、抗谷氨酸兴奋毒性等.     

Hashimoto thyroiditis,distal renal tubular acidosis,pernicious anaemia and encephalopathy: A rare combination of auto-immune disorders in a 12-year-old girl

A case of a 12-year-old girl with a multiple auto-immune disorder is reported. She showed Hashimoto thyroiditis which subsequently developed to hashitoxicosis and distal renal tubular acidosis at 5 years of age, pernicious anaemia at the age of 9 and severe encephalopathy at the age of 12. Laboratory studies revealed very high titres of anti-microsomal and anti-thyroglobulin antibodies and positive gastric parietal cell antibody. As to the encephalopathy, positive oligoclonal IgG bands and high values of IgG index and IgG synthesis ratio in CSF were observed with aggravation of her neurological symptoms. High-dose steroid therapy was effective toward the encephalopathy. Paediatricians should pay careful attention to patients with Hashimoto thyroiditis for association with other auto-immune disorders.     

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目的探讨铅中毒脑病的临床特点及预后。方法回顾分析衡阳市中心医院儿科2002～2003年26例铅中毒脑病患儿的临床资料及随访结果。结果26例铅中毒脑病患儿临床均表现为频繁的抽搐伴意识障碍,中度以上贫血。伴呕吐23例、腹泻21例、发热11例、肝大5例。26例患儿治疗前血铅平均为8.486μmol/L,尿铅平均为3.598μmol/L,Hb均低于90g/L,最低55g/L。血钙均低于1.86mmol/L,最低1.03mmol/L。脑脊液蛋白均增高,22例心肌酶谱增高,提示心肌损害明显。头颅CT/MRI仅1例脑室系统轻度扩张,全部病例经驱铅治疗后无一例死亡,1年后随访,继发性癫疒间4例,脑瘫4例。结论铅中毒脑病是铅中毒的严重类型,预后不良。对不明原因的无热惊厥者应注意及时行血铅检查,以便早期诊断,防止漏诊。     

早产儿脑病研究进展

早产儿脑病是复杂的原发性脑损伤和继发性脑发育异常疾病.过去几年对该病有了较为深刻的认识,该文就最新的有关早产儿脑病概念演变,脑损伤后的细胞分子机制和损伤后发育障碍,尤其是活化小胶质细胞介导的少突胶质细胞损伤机制、神经/髓鞘受损和丘脑、板层下神经元及大脑皮层的受损机制或发育成熟障碍进行综述,以便对该病有更深刻的认识.     

小儿脓毒症相关性脑病的临床诊断与治疗探讨

目的 探讨脓毒症相关性脑病患儿的早期临床诊断与治疗策略.方法 对9例脓毒症相关性脑病患儿,根据其临床表现,按个体化原则采用早期综合加强策略控制脓毒症外,尽早大剂量血浆、丙种球蛋白(IVIG)调整免疫平衡,并加用小剂量甘露醇、鲁米那等保护脑功能并积极控制抽搐状态.结果 9例脓毒症患儿存活6例,死亡3例.结论 针对脓毒症复杂的病理生理和免疫失衡,运用早期综合加强策略控制脓毒症,防止多器官功能不全加重,早期诊断和治疗脓毒症相关性脑病,可显著提高存活率,降低病死率.