Subacute posttraumatic ascending myelopathy (SPAM): A potential complication of subarachnoid shunt for syringomyelia?

Context: Treatment of primary spinal syringomyelia is still controversial. Among others, shunting syrinx fluid to the subarachnoid, peritoneal or pleural space has been utilized with varying success. Shunt obstruction, migration, and infection represent the most common complications of these procedures.

Findings: The authors present the case of an 81-year-old woman who developed an unusual neurological deterioration resembling a subacute posttraumatic ascending myelopathy (SPAM) after the insertion of a syringosubarachnoid shunt for the treatment of slow-growing D10 syringomyelia.

Conclusion/Clinical Relevance: To date, no cases of SPAM secondary to the insertion of a syringosubarachnoid shunt for the treatment of syringomyelia have been reported. The potential pathogenesis related to this phenomenon is discussed.     

Early posterior vitreous detachment is associated with LAMA5 dominant mutation

Background: Extracellular matrix molecular components, previously linked to multisystem syndromes include collagens, fibrillins and laminins. Recently, we described a novel multisystem syndrome caused by the c.9418G>A p.(V3140M) mutation in the laminin alpha-5 (LAMA5) gene, which affects connective tissues of all organs and apparatus in a three generation family. In the same family, we have also reported a myopic trait, which, however, was linked to the Prolyl 4-hydroxylase subunit alpha-2 (P4HA2) gene. Results of investigation on vitreous changes and their pathogenesis are reported in the present study.Materials and Methods: Nineteen family individuals underwent complete ophthalmic examination including best-corrected visual acuity (BCVA), fundus examination, fundus photography, intraocular pressure measurement, axial length measurement using ocular biometry, Goldmann visual field examination, standard electroretinogram, SD-OCT. Segregation analysis of LAMA5 and P4HA2 mutations was performed in enrolled members.Results: The vitreous alterations fully segregated with LAMA5 mutation in both young and adult family members. Slight reduction of retinal thickness and peripheral retinal degeneration in only two patients were reported.Conclusions: In this work we showed that PVD is a common trait of LAMA5 multisystem syndrome, therefore occurring as an age-unrelated trait. We hypothesize that the p.(V3140M) mutation results in a reduction of retinal inner limiting membrane (ILM) stability, leading to a derangement in the macromolecular structure of the vitreous gel, and PVD. Further investigations will be necessary to elucidate the role of wild type and mutated LAMA5 in the pathogenesis of PVD.     

A Tool to Assess Patient and Surrogate Knowledge About the POLST (Physician Orders for Life-Sustaining Treatment) Program

ContextIt is especially important that patients are well informed when making high-stakes, preference-sensitive decisions like those on the Physician Orders for Life-Sustaining Treatment (POLST) form. However, there is currently no way to easily evaluate whether patients understand key concepts when making these important decisions.ObjectivesTo develop a POLST knowledge survey.MethodsExpert (n = 62) ratings of key POLST facts were used to select items for a POLST knowledge survey. The survey was administered to nursing facility residents (n = 97) and surrogate decision-makers (n = 112). A subset (n = 135) were re-administered the survey after a standardized advance care planning discussion to assess the scale's responsiveness to change.ResultsThe 19-item survey demonstrated adequate reliability (α = 0.72.). Residents' scores (x = 11.4, standard deviation 3.3) were significantly lower than surrogate scores (x = 14.7, standard deviation 2.5) (P < 0.001). Scores for both groups increased significantly after administration of a standardized advance care planning discussion (P < 0.001). Although being a surrogate, age, race, education, cognitive functioning, and health literacy were significantly associated with higher POLST Knowledge Survey scores in univariate analyses, only being a surrogate (P < 0.001) and being white (P = 0.028) remained significantly associated with higher scores in multivariate analyses.ConclusionThe 19-item POLST Knowledge Survey demonstrated adequate reliability and responsiveness to change. Findings suggest the survey could be used to identify knowledge deficits and provide targeted education to ensure adequate understanding of key clinical decisions when completing POLST.     

血清胱抑素C和尿微量白蛋白联合检测在人类免疫缺陷病毒感染者肾功能损伤检测中的应用价值

目的评估血肌酐和尿常规的常规检测基础上联合血清胱抑素C和尿微量白蛋白检测在人类免疫缺陷病毒（HIV）感染者肾功能损伤检测中的应用价值。 方法以2019年2～5月于首都医科大学附属北京地坛医院感染一科住院的169例HIV感染者为研究对象，完善其尿常规、尿微量白蛋白、血肌酐、血清胱抑素C检测；分析尿蛋白及尿微量白蛋白的阳性检出率及其差异，血肌酐升高及血清胱抑素C升高的比例及其差异。计算应用替诺福韦酯（TDF）及合并丙型肝炎、高血压、糖尿病、肿瘤的肾功能损伤的相对危险度。 结果169例HIV感染者中尿常规示尿蛋白阳性者5例（3.0%），尿微量白蛋白升高者17例（10.1%），两者阳性检出率差异具有统计学意义（χ² = 5.9、P = 0.007）。血肌酐升高者10例（5.9%），血清胱抑素C升高者20例（11.8%），两者阳性检出率差异具有统计学意义（χ² = 3.0、P = 0.042）。在尿常规和血肌酐检测的基础上联合检测尿微量白蛋白和血清胱抑素C的总体阳性检出率为49例（30.0%）。CD4⁺ T淋巴细胞计数< 200个/μl与≥ 200个/μl组患者血清胱抑素C水平分别为0.94（0.83，1.05）mg/L、0.85（0.77，0.95）mg/L，差异具有统计学意义（Z =-3.02、P = 0.003）。应用TDF及合并丙型肝炎、高血压、糖尿病、肿瘤的肾功能损伤的相对危险度分别为1.1、1.5、1.9、2.2和1.4。 结论HIV感染者中，单纯以尿常规为依据评估有无蛋白尿，以血肌酐水平为依据评估肾小球滤过功能会低估肾功能损伤的患病率。在常规检测血肌酐和尿常规的基础上联合检测血清胱抑素C和尿微量白蛋白在提高HIV感染者肾功能损伤检出率方面具有重要的应用价值。低CD4⁺ T淋巴细胞计数、应用TDF及合并丙型肝炎、高血压、糖尿病、肿瘤均为肾功能损伤的危险因素。     

Alveolar crest contour changes after guided bone regeneration using different biomaterials: an experimental in vivo investigation

To evaluate the changes in alveolar contour after guided bone regeneration (GBR) with two different combinations of biomaterials in dehiscence defects arou     

从GAMP的理念谈制药行业自动化工程建设与验证的有关问题

根据国际药物工程协会(ISPE)所提GAMP的理念,谈有关制药行业自动化工程建设与验证方面的问题,其中包括GAMP的基本思想与概念、制药行业自动化工程建设与验证关系问题以及我国制药行业自动化工程建设与验证所存在的问题。