Intracranial hypertension in Africans with cerebral malaria

The causes of death and neurological sequelae in African children with cerebral malaria are obscure. Intracranial pressure (ICP) was monitored and cerebral perfusion pressure (CPP) calculated in 23 Kenyan children with cerebral malaria. Four children had severe intracranial hypertension (ICP > 40 mm Hg, CPP < 40 mm Hg): two died, one with an ICP of 158 mm Hg and signs of transtentorial herniation, the other one with an ICP of 42 mm Hg and cardiorespiratory arrest. The other two survived with severe neurological sequelae. Nine had intermediate intracranial hypertension (ICP > 20 mm Hg, CPP < 50 mm Hg) and 10 had mild intracranial hypertension (maximum ICP 10-20 mm Hg); all survived without severe sequelae. Mannitol controlled the ICP in children with intermediate intracranial hypertension, but it did not prevent the development of intractable intracranial hypertension in children with severe intracranial hypertension. Intracranial hypertension is a feature of Kenyan children with cerebral malaria and severe intracranial hypertension is associated with a poor outcome.     

Leiomyosarcoma of the oesophagus: A case report and literature review of leiomyosarcoma

Leiomyosarcomas are uncommon tumours. Oesophageal leiomyosarcomas are even rarer. A case is presented of this rare tumour which on review of the literature seems to be the first patient surviving 22 years from the original treatment. We include a literature review of the clinical behaviour and management of leiomyosarcoma.     

Costs and cost-effectiveness of the meeting centres support programme for people living with dementia and carers in Italy,Poland and the UK: The MEETINGDEM study

We examined the costs and cost-effectiveness of the Meeting Centre Support Programme (MCSP) implemented and piloted in the UK, Poland and Italy, replicating the Dutch Meeting Centre model. Dutch Meeting Centres combine day services for people with dementia with carer support. Data were collected over 2015–2016 from MCSP and usual care (UC) participants (people with dementia-carer dyads) at baseline and 6 months. We examined participants’ health and social care (HSC), and societal costs, including Meeting Centre (MC) attendances. Costs and outcomes in MCSP and UC groups were compared. Primary outcomes: Persons with dementia: quality-adjusted life years (EQ-5D-5L-derived); QOL-AD. DQoL was examined as a secondary outcome. Carers: Short Sense of Competence Questionnaire (SSCQ). Incremental cost-effectiveness ratios (ICER) and cost-effectiveness acceptability curves were obtained by bootstrapping outcome and cost regression estimates. Eighty-three MCSP and 69 UC dyads were analysed. The 6-month cost of providing MCSP was €4,703; participants with dementia attended MC a mean of 45 times and carers 15 times. Including intervention costs, adjusted 6-month HSC costs were €5,941higher in MCSP than in UC. From the HSC perspective: in terms of QALY, the probability of cost-effectiveness was zero over willingness-to-pay (WTP) ranging from €0 to €350,000. On QOL-AD, the probability of cost-effectiveness of MCSP was 50% at WTP of €5,000 for a one-point increase. A one-point gain in the DQoL positive affect subscale had a probability of cost-effectiveness of 99% at WTP over €8,000. On SSCQ, no significant difference was found between MCSP and UC. Evidence for cost-effectiveness of MCSP was mixed but suggests that it may be cost-effective in relation to gains in dementia-specific quality of life measures. MCs offer effective tailored post-diagnostic support services to both people with dementia and carers in a context where few evidence-based alternatives to formal home-based social services may be available.     

原发性醛固酮增多症33例

1　临床资料1.1　一般情况　我院近 2 0 a经手术及病理证实的 33例原发性醛固酮增多症 (原醛 )患者 ,男 11例 ,女 2 2例 .年龄 11～ 5 5(平均 32± 9)岁 .病程 1mo～ 18a,平均 (6 .4± 5 .6 ) a. 33例均有血压增高 ,为 (2 2～ 32 / 13.3～ 2 4) k Pa,平均 (2 5 .7± 2 .8/16 .5± 2 .6 ) k Pa.肌无力 16例 ,典型周期性麻痹 14例 .高血压和肌无力及周期性麻痹初发时间均有记录的 2 4例中 ,后两者在高血压前出现者 2例 ,与高血压同时出现者 8例 ,在高血压后 (1～ 11a)出现者 14例 .多尿和夜尿增多 2 2例 .手足搐搦 9例 .1.2　实验室检查　测…     

Severe malformations in males from families with osteopathia striata with cranial sclerosis

Osteopathia striata with cranial sclerosis (OS-CS) is a bone dysplasia characterized by longitudinal striations of the long bones and sclerosis of the craniofacial bones. Affected patients show macrocephaly, ocular hypertelorism, frontal bossing, broad nasal bridge and abnormalities of the palate. Anomalies such as hearing loss, congenital heart defect, vertebral anomalies and mental impairment have also been reported. Pedigree analysis has suggested an autosomal dominant inheritance, but a recent report of a family with significantly more affected males than females suggested the possibility of X-linked inheritance. Here we describe a new family with OS-CS (the twelfth in the literature) with four affected individuals (two males and two females) spanning three generations. The affected male in the third generation was stillborn with multiple congenital anomalies, whereas the other three affected individuals had mild features. This family may represent another example of X-linked OS-CS where the mutated gene(s) is more severe in males.     

三维超声心动图测定正常人及二尖瓣病变伴或不伴房颤患者左心耳容量及其收缩功能

目的:探讨三维超声心动图评价左心耳功能的价值.方法:采用三维超声心动图对52例正常人及二尖瓣病变伴或不伴心房纤颤患者测定左心耳容量及其收缩功能,并与二维超声心动图和多普勒超声心动图比较.结果:根据左心耳容量测定计算出的左心耳射血分数(EFv)与左心耳峰值血流排空速度(Pev)相关良好(r＝0.818,P＜0.01).根据左心耳面积测定计算出的左心耳射血分数(EFa)与Pev的相关性低于前者(r＝0.7444,P＜0.01).将所有患者的EFv与EFa进行比较,虽然两者间未见明显差异(P＞0.05),但在重复性检验中EFa的范围和两测量数值之差的均数加减标准差都大于EFv的相应数值,左心耳容量在正常人明显小于有二尖瓣病变的患者,而收缩功能则前者明显高于后者(P值均＜0.01).在相同二尖瓣病变患者之间进行比较,合并房颤者左心耳容量大于窦性心率者,而左心耳收缩功能则前者低于后者(P值均＜0.05).另外,二尖瓣病变合并房颤者的左心耳自发性"超声造影"发生率高于窦性心率者.结论:用三维超声心动图测量左心耳容量和评价其收缩功能是可行的,评价左心耳功能时较二维超声心动图更准确,二尖瓣病变患者的左心耳容量增加,收缩功能减低,常伴左心耳自发性"超声造影",上述改变在合并房颤者更明显.     

Low levels of erythroid and myeloid progenitors in thrombopoietin-and c- mpl-deficient mice

Thrombopoietin (TPO), the ligand for the c-mpl receptor, has been shown to be the major regulator of platelet production. Mice deficient in either c-mpl or TPO generated by homologous recombination show a dramatic decrease in platelet counts, but other blood cell counts are normal. Because TPO treatment of myelosuppressed mice not only enhances the recovery of platelets but also accelerates erythroid recovery, we investigated the levels of myeloid and erythroid progenitor cells in TPO-or c-mpl-deficient mice. Our results show that the number of megakaryocyte, granulocyte-macrophage, erythroid, and multilineage progenitors are significantly reduced in the bone marrow, spleen, and peripheral blood of either TPO-or c-mpl-deficient mice. Administration of recombinant murine TPO to TPO-deficient mice and control littermate mice significantly increased the absolute number of myeloid, erythroid, and mixed progenitors in bone marrow and spleen. This increase was especially apparent in TPO-deficient mice where numbers were increased to a level greater than in diluent-treated control mice and approached or equaled that in the TPO-treated control mice. Moreover, TPO- administration greatly increased the number of circulating progenitors as well as platelets in both TPO-deficient and control mice. Furthermore, the megakaryocytopoietic activity of other cytokines in the absence of a functional TPO or c-mpl gene was shown both in vitro and in vivo.     

肺树突状细胞成熟分子的表达与慢性阻塞性肺疾病急性加重相关

背景和目的:树突状细胞(DCs)在初始T细胞和效应T细胞接触抗原后的活化和诱导分化方面发挥着重要作用.然而DCs在COPD中的作用尚不清楚,为数不多的研究所得结论不同,且未涉及DCs的成熟状态与COPD严重程度之间的关系.本研究旨在明确DCs成熟分子的表达与COPD分期,以及与肺CD4+T细胞急性活化标记CD69表达之间的关系.