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Are ATXN2 variants modifying our understanding about neural pathogenesis,phenotypes,and diagnostic?

Neural Regeneration Research 2022年第11期17卷 2445-2447页

作者： Jose Miguel Laffita-Mesa Martin Paucar Per Svenningsson Department of Clinical Neuroscience(CNS) Karolinska Universitetssjukhuset

ATXN2 one gene with multiple phenotype effects:ATXN2 gene encodes a cytosolic protein(ataxin-2)with pleiotropic functions(see below).This gene contains a number of exonic Cytosine-Adenine-Guanine(CAG)-repeats which encodes a polyglutamine tract(polyQ)in the N-terminal intrinsically disordered region(IDR)of the ***2CAG repeats are interrupted by CAA codons which is relevant only for DNA and RNA but not for protein since CAA also encodes glutamine(Q).

关键词： pathogenesis neural interrupted

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Sugammadex比新斯的明更快逆转维库溴铵所致神经肌肉阻滞的多中心随机对照研究

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麻醉与镇痛 2011年第5期7卷 52-62页

作者： Karin S. Khuenl-Brady, MD Magnus Wattwil, MD Bernard F. Vanacker, MD Jose I. Lora-Tamayo, MD Henk Rietbergen, MSc Jose A. alvarez-Gomez, MD 安博（译） Medical University Innsbruck Innsbruck Austria universitetssjukhuset orebro Orebro Sweden Universitair Ziekenhuis Leuven Gasthuisberg Leuven Belgium Hospital Universitario Puerta de Hierro Madrid Spain Schering-Plough Oss The Netherlands Hospital Universitario Cartagena Spain. 不详

背景Sugammadex是一种具有特异结构的γ-环糊精，能选择性地与肌松药结合快速逆转罗库溴铵所致的神经肌肉阻滞，并较小程度地逆转维库溴铵所致的神经肌肉阻滞。该研究中，我们对比了行择期手术的患者中sugammadex和新斯的明对逆转维库... 详细信息

背景Sugammadex是一种具有特异结构的γ-环糊精，能选择性地与肌松药结合快速逆转罗库溴铵所致的神经肌肉阻滞，并较小程度地逆转维库溴铵所致的神经肌肉阻滞。该研究中，我们对比了行择期手术的患者中sugammadex和新斯的明对逆转维库溴铵所致神经肌肉阻滞的作用。方法年龄≥18岁，ASA分级I-Ⅲ级拟行择期手术的患者，在七氟醚／阿片类镇痛药麻醉下给予插管剂量维库溴铵（0．1mg／kg），如有必要，在4个成串刺激（train—of-four，TOF）中第二个颤搐波（T2）恢复时给予的0．02-0．03mg／kg的维持剂量。用加速肌动监测仪（TOF-Watch@SX监测仪，先灵-葆雅，爱尔兰都柏林）监测神经肌肉阻滞。术毕，在最后一次给予维库溴铵后咒恢复时，给患者随机静脉注射sugammadex（2mg／kg）或新斯的明（50μg／kg）加格隆溴铵（10μg/kg）。从注药开始至TOF比值恢复至0．9的时间作为主要有效终点。结果Sugammadex组TOF比值恢复至0．9的时间明显快于新斯的明组（分别为2．7分钟[95％置信区间，CI：2．2—2．3分钟]和17．9分钟[95％CI：13．1—24．3分钟]，P〈0．0001）。Sugammadex组TOF恢复至0．8和0．7的平均恢复时间也明显缩短。使用两种药物后均未出现严重不良事件或不可预料的副作用。结论Sugammadex逆转维库溴铵所致神经肌肉阻滞的作用明显快于新斯的明。

关键词：多中心随机对照研究神经肌肉阻滞维库溴铵新斯的明快速逆转阿片类镇痛药择期手术 γ-环糊精

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Amino Acids during Perioperative Period

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Open Journal of Anesthesiology 2017年第9期7卷 287-295页

作者： Takeshi Yokoyama Ippei Yamaoka Takashi Hitosugi Eva Selldén Department of Dental Anesthesiology Faculty of Dental Science Kyushu University Fukuoka Japan Medical Foods Research Institute OS-1 Division Otsuka Pharmaceutical Factory Inc. Naruto Japan Anestesi- Operations-och Intensivv&aring rdskliniken Karolinska Universitetssjukhuset Solna Stockholm Sweden

During anesthesia, thermoregulation is impaired and hypothermia will frequently occur in most patients. Hypothermia affects immunologic activity, bleeding tendency and the recovery from anesthesia. Therefore, it may prolong hospital stay, and increase morbidity, e.g. surgical site infections, cardiac events and multiple organ dysfunctions in trauma. External warming is often used to prevent hypothermia. However, infusion of amino acids is also valuable to prevent hypothermia due to their enhanced thermogenic action under anesthesia. During surgery, amino acids administration would maintain the body homeostasis, and counteract the disadvantageous fasting metabolism. Postoperatively, amino acids may be advantageous for the healing of the surgical wound. Thus, appropriate nutritional management, including glucose, during the perioperative period would prevent catabolism, frequently occurring after surgery. Protocols like ERAS (Enhanced Recovery After Surgery) are proposed for quick recovery after surgery. ERAS protocol recommends preoperative carbohydrate and early enteral nutrition, but does not include infusion of amino acids during the perioperative period. During prolonged surgery, patients clearly need good nutritional support. In this article, we intend to describe the problems of hypothermia briefly, and explain the mechanism of amino acids in hypothermia prevention. In addition, we address some evidences of nutritional management during the perioperative period.

关键词： Anesthesia Hypothermia Amino Acids Nutrition

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Symptomatic Val122del mutated hereditary transthyretin amyloidosis: Need for early diagnosis and prioritization for heart and liver transplantation

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Hepatobiliary & Pancreatic Diseases International 2021年第4期20卷 323-329页

作者： Adriano-Valerio Schettini Laura Llado JulieK Heimbach JoseGonzalez Costello Marie Tranäng Olivier Van Caenegem Richard C Daly Peter Van den Bergh Carlos Casasnovas Joan Fabregat John J Poterucha Maxime Foguenne Bo Göran Ericzon Jan Lerut Institute for Experimental and Clinical Research(IREC) Catholic University of Louvain(UCL)Avenue Hippocrate 55Brussels 1200Belgium Liver Transplant Unit Department of SurgeryHospital Universitari de BellvitgeCalle de la Feixa LlargaBarcelona 08907Spain Division of Transplantation Surgery William J.von Liebig Transplant CenterMayo Clinic2001st St SWRochesterMN 55905USA Advanced Heart Failure and Transplant Unit Cardiology DepartmentHospital Universitari de BellvitgeCalle de la Feixa LlargaBarcelona 08907Spain Familial Amyloidotic Polyneuropathy World Transplant Registry(FAPWTR) Division of Transplantation SurgeryKarolinska Universitetssjukhuset HuddingeF82Karolinska InstitutetStockholm 14186Sweden Cardiothoracic Intensive Care Unit Department of Cardiovascular DiseasesUniversity Hospitals Saint-Luc-UCLAvenue Hippocrate 10Brussels 1200Belgium Cardiovascular Surgery Department Mayo Clinic2001st St SWRochesterMN 55905USA Neuromuscular Reference Centre University Hospital Saint-Luc-UCLAvenue Hippocrate 10Brussels 1200Belgium Neuromuscular Unit Neurology DepartmentHospital Universitari de BellvitgeCalle de la Feixa LlargaBarcelona 08907Spain Division of Gastroenterology and Hepatology Mayo Clinic2001st St SWRochester MN 08907USA Division of Transplantation Surgery CLINTECKarolinska Universitetssjukhuset HuddingeF82Karolinska InstitutetStockholm 14186Sweden

Background: Hereditary transthyretin(ATTRv) amyloidosis is an autosomal dominant disease linked to transthyretin gene mutations which cause instability of the transthyretin tetramer. After dissociation and misfolding they reassemble as insoluble fibrils(i.e. amyloid). Apart from the common Val30 Met mutation there is a very heterogeneous group of non-Val30 Met mutations. In some cases, the clinical picture is dominated by a rapidly evolving restrictive and hypertrophic cardiomyopathy. Methods: A case series of four liver recipients with the highly clinically relevant, rare and particularly aggressive Val122 del mutation is presented. Medical and surgical therapeutic options, waiting list policy for ATTRv-amyloidosis, including the need for heart transplantation, and status of heart-liver transplantation are discussed. Results: Three patients needed a staged(1 patient) or simultaneous(2 patients) heart-liver transplant due to rapidly progressing cardiac failure and/or neurologic disability. Domino liver transplantation was impossible in two due to fibrotic hepatic transformation caused by cardiomyopathy. After a follow-up ranging from 3.5 to 9.5 years, cardiac(allograft) function was maintained in all patients, but neuropathy progressed in three patients, one of whom died after 80 months. Conclusions: This is the first report in(liver) transplant literature about the rare Val122 del ATTRv mutation. Due to its aggressiveness, symptomatic patients should be prioritized on the liver and, in cases with cardiomyopathy, heart waiting lists in order to avoid the irreversible neurological and cardiac damage that leads to a rapid lethal outcome.

关键词： Hereditary transthyretin amyloidosis Heart transplantation Liver transplantation Non-Val30Met mutation Val122del mutation Domino liver transplantation

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