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Malignant Hyperthermia

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https://www.readbyqxmd.com/read/28719515/anaesthetic-management-of-patients-with-myopathies
#1
Mark Schieren, Jerome Defosse, Andreas Böhmer, Frank Wappler, Mark U Gerbershagen
: The anaesthetic management of patients with myopathies is challenging. Considering the low incidence and heterogeneity of these disorders, most anaesthetists are unfamiliar with key symptoms, associated co-morbidities and implications for anaesthesia. The pre-anaesthetic assessment aims at the detection of potentially undiagnosed myopathic patients and, in case of known or suspected muscular disease, on the quantification of disease progression. Ancillary testing (e.g. echocardiography, ECG, lung function testing etc...
July 15, 2017: European Journal of Anaesthesiology
https://www.readbyqxmd.com/read/28706901/intraoperative-intracavitary-hyperthermic-chemotherapy-for-malignant-pleural-mesothelioma
#2
REVIEW
Kazunori Okabe
Malignant pleural mesothelioma (MPM) is a dreadful disease with a poor prognosis. Multimodality therapy including surgical macroscopic complete resection is performed to treat operable MPM. Intraoperative intracavitary hyperthermic chemotherapy for MPM was reviewed. Appropriate papers published between 2006 and present were extracted by the PubMed advanced search by MPM (Title/Abstract), chemotherapy (Title/Abstract), and hyperthermia (All fields). Among the selected papers, those written in English, and treated more than ten MPM patients were reviewed...
June 2017: Annals of Translational Medicine
https://www.readbyqxmd.com/read/28697029/malignant-hyperthermia-in-a-morbidly-obese-patient-depletes-community-dantrolene-resources-a-case-report
#3
Fabio Magistris, Jonathan Gamble
During resection of a duodenal carcinoid tumor, a 28-year-old morbidly obese woman developed suspected malignant hyperthermia. This hypermetabolic state posed a diagnostic challenge given the similar intraoperative presentation of carcinoid crisis and malignant hyperthermia. The patient's weight posed therapeutic challenges as massive doses and prolonged administration of dantrolene were required that quickly depleted the available supply. Current dantrolene dosing recommendations are based on actual body weight despite a paucity of literature in obese patients...
July 10, 2017: A & A Case Reports
https://www.readbyqxmd.com/read/28687594/reduced-threshold-for-store-overload-induced-ca-2-release-is-a-common-defect-of-ryr1-mutations-associated-with-malignant-hyperthermia-and-central-core-disease
#4
Wenqian Chen, Andrea Koop, Yingjie Liu, Wenting Guo, Jinhong Wei, Ruiwu Wang, David H MacLennan, Robert T Dirksen, S R Wayne Chen
Mutations in the skeletal muscle ryanodine receptor (RyR1) cause malignant hyperthermia (MH) and central core disease (CCD), while mutations in the cardiac ryanodine receptor (RyR2) lead to catecholaminergic polymorphic ventricular tachycardia (CPVT). Most disease-associated RyR1 and RyR2 mutations are located in the N-terminal, central, and C-terminal regions of the corresponding RyR isoform. An increasing body of evidence demonstrates that CPVT-associated RyR2 mutations enhance the propensity for spontaneous Ca(2+) release during store Ca(2+) overload, a process known as store-overload induced Ca(2+) release (SOICR)...
July 7, 2017: Biochemical Journal
https://www.readbyqxmd.com/read/28682948/skeletal-muscle-metabolic-dysfunction-in-patients-with-malignant-hyperthermia-susceptibility
#5
Sara J Thompson, Sheila Riazi, Natalia Kraeva, Michael D Noseworthy, Tammy E Rayner, Jane E Schneiderman, Barbara Cifra, Greg D Wells
BACKGROUND: Malignant hyperthermia (MH), a pharmacogenetic disorder of skeletal muscle, presents with a potentially lethal hypermetabolic reaction to certain anesthetics. However, some MH-susceptible patients experience muscle weakness, fatigue, and exercise intolerance in the absence of anesthetic triggers. The objective of this exploratory study was to elucidate the pathophysiology of exercise intolerance in patients tested positive for MH with the caffeine-halothane contracture test...
August 2017: Anesthesia and Analgesia
https://www.readbyqxmd.com/read/28665830/undiagnosed-pheochromocytoma-simulating-malignant-hyperthermia
#6
Nisha S Ramani, Robert Stoppacher, Ajaykumar C Morani, Charles Catanese
INTRODUCTION: Pheochromocytomas are rare catecholamine-producing neuroendocrine tumors. They are surgically curable but can be lethal if remain undiagnosed. We report a patient earlier diagnosed with malignant hyperthermia but later found to have pheochromocytoma on autopsy. CASE REPORT: After a preprocedural pain block for elective right shoulder arthroscopy, a 53-year-old hypertensive white man developed chest pain. In the operating room, he had increased blood pressure...
June 30, 2017: American Journal of Forensic Medicine and Pathology
https://www.readbyqxmd.com/read/28664843/management-of-soft-tissue-tumors-of-the-upper-extremity-a-review
#7
Kevin T Jubbal, Gehaan D'Souza, Reid A Abrams, Anna A Kulidjian
INTRODUCTION: Management of malignant tumors of the hand and wrist is challenging and is generally approached by limb salvage or amputation. With advances in care, amputation has been superseded by limb salvage as the treatment of choice. METHODS: A narrative literature review was performed to identify articles on the topic of management of soft tissue tumors of the upper extremity, including surgical management, adjuvant radiation therapy, and chemotherapy. RESULTS: A total of 29 articles were selected...
2017: SICOT-J
https://www.readbyqxmd.com/read/28660414/-stocks-of-dantrolene-in-anesthesia-and-intensive-care-units-in-germany-nationwide-online-survey-with-1673-participants
#8
E Pfenninger, S Heiderich, W Klingler
BACKGROUND: A malignant hyperthermia (MH) crisis is a potentially fatal complication in anesthesia and intensive care units (ICU). Rapid administration and adequate dosage of dantrolene is the only known effective pharmacological and causal treatment of an MH crisis. International anesthesiology societies recommend an initial dose of 2.0-2.5 mg/kg body weight (BW). The necessary total dosage should be titrated up to 10 mg/kg BW depending on the effectiveness. OBJECTIVE: The goal of this study was an analysis of the stocking situation of dantrolene in Germany...
June 28, 2017: Der Anaesthesist
https://www.readbyqxmd.com/read/28625829/combinatorial-immunotherapy-and-nanoparticle-mediated-hyperthermia
#9
REVIEW
Austin J Moy, James W Tunnell
Immune checkpoint therapy has become the first widely adopted immunotherapy for patients with late stage malignant melanoma, with potential for a wide range of cancers. While some patients can experience long term disease remission, this is limited only to a subset of patients and tumor types. The path forward to expand this therapy to more patients and tumor types is currently thought to be combinatorial treatments, the combination of immunotherapy with other treatments. In this review, the combinatorial approach of immune checkpoint therapy combined with nanoparticle-assisted localized hyperthermia is discussed, starting with an overview of the different nanoparticle hyperthermia approaches in development, an overview of the state of immune checkpoint therapy, recent reports of immune checkpoint therapy and nanoparticle-assisted hyperthermia in a combinatorial approach, and finally a discussion of future research topics and areas to be explored in this new combinatorial approach to cancer treatment...
June 15, 2017: Advanced Drug Delivery Reviews
https://www.readbyqxmd.com/read/28610455/non-fourier-thermal-transport-induced-structural-hierarchy-and-damage-to-collagen-ultrastructure-subjected-to-laser-irradiation
#10
Nilamani Sahoo, Arunn Narasimhan, Purbarun Dhar, Sarit Kumar Das
Comprehending the mechanism of thermal transport through biological tissues is an important factor for optimal ablation of cancerous tissues and minimising collateral tissue damage. The present study reports detailed mapping of the rise in internal temperature within the tissue mimics due to NIR (1064 nm) laser irradiation, both for bare mimics and with gold nanostructures infused. Gold nanostructures such as mesoflowers and nano spheres have been synthesized and used as photothermal converters to enhance the temperature rise, resulting in achieving the desired degradation of malignant tissue in targeted region...
June 13, 2017: International Journal of Hyperthermia
https://www.readbyqxmd.com/read/28605946/design-and-characterization-of-a-phased-antenna-array-for-intact-breast-hyperthermia
#11
Sergio Curto, Aleix Garcia-Miquel, Minyoung Suh, Neus Vidal, Jose M Lopez-Villegas, Punit Prakash
PURPOSE: Currently available hyperthermia technology is not well suited to treating cancer malignancies in the intact breast. This study investigates a microwave applicator incorporating multiple patch antennas, with the goal of facilitating controllable power deposition profiles for treating lesions at diverse locations within the intact breast. MATERIALS AND METHODS: A 3D-computational model was implemented to assess power deposition profiles with 915 MHz applicators incorporating a hemispheric groundplane and configurations of 2, 4, 8, 12, 16 and 20 antennas...
June 12, 2017: International Journal of Hyperthermia
https://www.readbyqxmd.com/read/28604462/anesthetic-management-of-2-pediatric-patients-with-concurrent-diagnoses-of-mitochondrial-disease-and-malignant-hyperthermia-susceptibility-a-case-report
#12
Jonathon H Nelson, Richard F Kaplan
We report the case of 2 pediatric patients with coexisting diagnoses of malignant hyperthermia susceptibility and mitochondrial disease in 2 different surgical settings. Due to the rare occurrence of each disorder, and even more so together, we reviewed evidence-based anesthetic concerns and described our perioperative management, with the goal of aiding future practitioners in safely caring for these patients. Consent was obtained for both patients, as well as IRB approval before publication.
June 8, 2017: A & A Case Reports
https://www.readbyqxmd.com/read/28584051/ion-pulling-simulations-provide-insights-into-the-mechanisms-of-channel-opening-of-the-skeletal-muscle-ryanodine-receptor
#13
David D Mowrey, Le Xu, Yingwu Mei, Daniel A Pasek, Gerhard Meissner, Nikolay V Dokholyan
The type 1 ryanodine receptor (RyR1) mediates Ca(2+) release from the sarcoplasmic reticulum to initiate skeletal muscle contraction and is associated with muscle diseases, malignant hyperthermia, and central core disease. To better understand RyR1 channel function, we investigated the molecular mechanisms of channel gating and ion permeation. An adequate model of channel gating requires accurate, high-resolution models of both open and closed states of the channel. To this end, we generated an open-channel RyR1 model using molecular simulations to pull Ca(2+) through the pore constriction site of a closed-channel RyR1 structure determined at 3...
June 5, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/28580087/successful-early-application-of-extracorporeal-membrane-oxygenation-to-support-cardiopulmonary-resuscitation-for-a-patient-suffering-from-severe-malignant-hyperthermia-and-cardiac-arrest-a-case-report
#14
Hyub Huh, Jae Seung Jung, Sang Jae Park, Min Kyung Park, Choon Hak Lim, Seung Zhoo Yoon
Malignant hyperthermia (MH) may lead to metabolic crisis of skeletal muscle in susceptible individuals following exposure to triggering agents such as volatile anesthetics or depolarizing muscle relaxants. MH is a rare and a potentially lethal disease, which can lead to cardiac arrest. We report a case of severe MH, in which the rapidly evolving signs of hypermetabolism eventually resulted in cardiac arrest. Despite conventional treatments following cardiopulmonary resuscitation, the patient's vital signs did not improve...
June 2017: Korean Journal of Anesthesiology
https://www.readbyqxmd.com/read/28571205/postoperative-malignant-hyperthermia-a-medical-emergency-a-case-report-and-review-of-literature
#15
Amit Kumar Sinha, Poonam Kumari, Maheshkumar Manilal Vaghela, Chandni Sinha, Bindey Kumar
Malignant hyperthermia is a rare life threatening pharmacological disorder. Preoperative clinical diagnosis is not possible. Due to its rarity and severity, it must be taken as an important differential diagnosis for postoperative hyperthermia. We hereby report a case of postoperative malignant hyperthermia in a four-year-old female child, who was operated for type 1 choledochal cyst and had difficult postoperative sequel. We lost that child due to unavailability of Dantrolene sodium in emergency drug cart.
April 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28558105/confusing-terminology-neuroleptic-malignant-syndrome-vs-malignant-hyperthermia-reply
#16
Philippe Damier, Jasem Al-Hashel
No abstract text is available yet for this article.
May 30, 2017: JAMA Neurology
https://www.readbyqxmd.com/read/28558090/confusing-terminology-neuroleptic-malignant-syndrome-vs-malignant-hyperthermia
#17
Ronald S Litman
No abstract text is available yet for this article.
May 30, 2017: JAMA Neurology
https://www.readbyqxmd.com/read/28547000/whole-exome-sequencing-of-a-patient-with-suspected-mitochondrial-myopathy-reveals-novel-compound-heterozygous-variants-in-ryr1
#18
Patrick R Blackburn, Duygu Selcen, Jennifer M Gass, Jessica L Jackson, Sarah Macklin, Margot A Cousin, Nicole J Boczek, Eric W Klee, Elliot L Dimberg, Kathleen D Kennelly, Paldeep S Atwal
BACKGROUND: Pathogenic variants in ryanodine receptor 1 (RYR1, MIM# 180901) are the cause of congenital myopathy with fiber-type disproportion, malignant hyperthermia susceptibility type 1, central core disease of muscle, multiminicore disease and other congenital myopathies. METHODS: We present a patient with global developmental delay, hypotonia, myopathy, joint hypermobility, and multiple other systemic complaints that were noted early in life. Later she was found to have multiple bone deformities involving her spine, with severe scoliosis that was corrected surgically...
May 2017: Molecular Genetics & Genomic Medicine
https://www.readbyqxmd.com/read/28538150/lanthanides-report-calcium-sensor-in-the-vestibule-of-ryanodine-receptor
#19
Sándor Sárközi, István Komáromi, István Jóna, János Almássy
Ca(2+) regulates ryanodine receptor's (RyR) activity through an activating and an inhibiting Ca(2+)-binding site located on the cytoplasmic side of the RyR channel. Their altered sensitivity plays an important role in the pathology of malignant hyperthermia and heart failure. We used lanthanide ions (Ln(3+)) as probes to investigate the Ca(2+) sensors of RyR, because they specifically bind to Ca(2+)-binding proteins and they are impermeable to the channel. Eu(3+)'s and Sm(3+)'s action was tested on single RyR1 channels reconstituted into planar lipid bilayers...
May 23, 2017: Biophysical Journal
https://www.readbyqxmd.com/read/28529748/local-modulated-electro-hyperthermia-in-combination-with-traditional-chinese-medicine-vs-intraperitoneal-chemoinfusion-for-the-treatment-of-peritoneal-carcinomatosis-with-malignant-ascites-a-phase-ii-randomized-trial
#20
Clifford L K Pang, Xinting Zhang, Zhen Wang, Junwen Ou, Yimin Lu, Pengfei Chen, Changlin Zhao, Xiaopu Wang, Hongyu Zhang, Sergey V Roussakow
The purpose of this study was to develop a safe and non-toxic alternative to the conventional conservative treatment of peritoneal carcinomatosis with malignant ascites (PCMA) by investigating the efficacy and safety of local modulated electro-hyperthermia (mEHT) combined with the traditional Chinese medicine (TCM) 'Shi Pi' herbal decoction, compared with standard intraperitoneal chemoinfusion (IPCI). A randomized, controlled, single-center, open-label clinical trial (phase II) with two parallel groups (allocation ratio, 1:1) was conducted to investigate the efficacy and safety of mEHT+TCM (study group, SG) vs...
May 2017: Molecular and Clinical Oncology
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