Malignant Hyperthermia Abstract A patient, waiting to undergo his cardiac surgery, is lying on a surgical platform. An anesthesiologist enters the surgical room and injects some general anesthetics into the patient’s bloodstream. The patient appears normal until after a few minutes, the patient suddenly experiences increasing body temperatures leading to a high fever, muscle rigidity, and increased heart rate. The anesthesiologist is perturbed, runs out of the surgery room, and alerts the surgical staff of the patient’s alarming symptoms. The surgical staff identifies the symptoms as Malignant Hyperthermia. What exactly is Malignant Hyperthermia and how is it caused? Malignant Hyperthermia, a rare skeletal muscular disease found in humans, pigs, horses, and many other animals, is a channelopathy caused by abnormal calcium channels. This paper serves to investigate the problems within the specific ion channels known to cause the disease. The goal of the paper is to understand more about Malignant Hyperthermia and about how particular ion channels associated with the disease operate. Learning about the chemistry behind the disease is crucial in order to prevent future occurrences of Malignant Hyperthermia and to design new treatments. Introduction Malignant hyperthermia is a fatal, inherited disorder that affects less than 200,000 people in the United States. [1] As mentioned in the abstract, Malignant Hyperthermia is channelopathy, or a disease caused by mutations in channel protein genes. Malignant Hyperthermia is triggered by anesthetics, which includes common inhalants, and by medication containing succinylcholine, a substance often used as a muscle relaxant in emergency medicines. [2] After the initial ... ... middle of paper ... ...anodine receptor-Ca2+ release channels in malignant hyperthermia." Biophys J. 73(4) Oct 1997 29. Jul 2008 . [8] Jiang, Dawei, Wenqian Chen1, Jianmin Xiao, Ruiwu Wang, Huihui Kong, Peter P. Jones, Lin Zhang, Bradley Fruen, and S. R. Wayne Chen. "Reduced Threshold for Luminal Ca2+ Activation of RyR1 Underlies a Causal Mechanism of Porcine Malignant Hyperthermia." J. Biol. Chem. Vol. 283, Issue 3025 July 2008. 29 Jul 2008 . [9] Korf, Bruce R. "Korf Genetics." Human Genetics and Genomics . Blackwell Publishing. 29 Jul 2008 . [10] Millar, 2007. Cerebrospinal Fluid Research. 29 Jul 2008 .
The normal core temperature in adults ranges between 36.5°C and 37.5°C and hypothermia can be defined as core body temperature less than 36°C.(ref 1)
The patient is alert, oriented, no acute distress, she is sitting upright in her chair, however.
Maintaining normal core body temperature (normothermia) in patients within perioperative environments is both a challenging and important aspect to ensure patient safety, comfort and positive surgical outcomes (Tanner, 2011; Wu, 2013; Lynch, Dixon & Leary, 2010). Normorthermia is defined as temperatures from 36C to 38C, and is maintained through thermoregulation which is the balance between heat loss and heat gain (Paulikas, 2008). When normothermia is not maintained within the perioperative environments, and the patient’s core body temperature drops below 36C, they are at risk of developing various adverse consequences due to perioperative hypothermia (Wagner, 2010). Perioperative hypothermia is classified into three
Rothrock, J. C. (2007). Alexander's Care of the Patient in Surgery. St Louis, MO: Mosby Elsevier.
The group of scientists hoped to determine the structure of the channel-forming domains in CFTR. The key experiment, called substituted-cysteine-accessibility method or S.C.A.M, consisted of mutating and substituting 9 consecutive residues in the M1 membrane spanning segment with cysteine in Xenopus oocytes, or eggs. If the mutated channels with cysteine still function, then they assumed that the structures of the mutated and normal channels were similar. Next, they determined the accessibility of the cysteine residue by adding the reagents MTSEA and MTSES, which are highly specific reagents that form a mixed disulfide with a free sulfhydryl covalently linking the reagent to the cysteine. In other words, if the MTSEA and MTSES bond with the cysteine residue and alter the conduction, they can assume the accessibility of the residue and then infer that the side chain of the corresponding wild type residue, or the residue before substitution, lines the channel. This process had been used to determining the structures of ion ch...
What? The patient is 65-year-old man Mr. John Douglas who is suffering from dysphagia and have been admitted to the surgical ward for insertion of a percutaneous endoscopic gastrostomy (PEG). Apart from that, he is a Type 1 diabetes patient and has weakness in his right leg and arm because of right-sided hemiplegia. He is thin in appearance and has stage 1 pressure sore on his right heel.
There is progressive vasoconstriction of arterioles until the BP exceeds the upper limit of auto regulation, followed by breakthrough vasodilation, increase in cerebral blood flow, blood-brain barrier dysfunction, and cerebral oedema(Rodriguez-Yanez et al., 2006). Cerebral ischemia results in severely ischemic tissue with failure of electrical activity and ionic pumps (Rodriguez-Yanez et al., 2006) There is increase in the release of the excitatory amino acid glutamate due to electrical failure. (Rodriguez-Yanez et al., 2006) . Glutamate receptors are activated as a result and cause the opening of ion channels that allow potassium ions to leave the cell and sodium and calcium ions to enter. This has a number of physiological effects.
A few minutes prior to first operation at 7:30 AM, the surgeon assigned to patient administer local anesthesia.
Anesthesiologists take care of patients’ body signs while they are in surgery and also with their pain management during surgery (“Physicians” para. 10). During the surgery they give the patient anesthetics or drugs. These drugs help relieve pain the patient feels during their surgery or any other procedure (“Physicians” para. 10). They will also position the patient onto the operating table to make them feel comfortable and surgically accessible for surgery (“Anesthesiologist” What’s para. 1). Most use local, intravenous, spinal, or caudal methods to inject the anesthetic for sedation; the anesthesiologist prepares the patient for surgery (“Anesthesiologist” What’s para. 1). They record the types and amounts of amnesia used(“Anesthesiologist” What’s para. 1). They also watch over the patient’s body temperature, blood pressure, heart rate, and breathing rate (“Physicians” para. 10). After the procedure, they decide when the patient has recovered from the anesthetic and is able to go home (“Anesthesiologist” What’s para. 1). Anesthesiologists can ...
When the patient arrived in the room, the nurse identified the patient, and made sure she knew what procedure was ready to take place. He then helped the CRNA place the patient on the bed.
As we can see, due to triage or even shortage of sickbed, some patients were lying in front of the register counter.
I seized an opportunity to quietly speak with her and she explained that she had not received an adequate amount of rest the night before and the journey down had been exhausting. She also expressed concerns about being fearful about going into the operating room. I overheard a nurse earlier ask the group as a whole if anyone wanted an ativan to ease anxiety and the group consensus was no. I felt that because it was unanimous, she may have been embarrassed if it was only her that requested it. My concern for this patient was for her to remain comfortable and provide any healing initiatives that would reassure her that she was safe.
Wilson disease is caused by a mutation in the ATP7B gene. The ATP7B gene is responsible for the metabolism of coper in our bodies. ATP7B gives instructions for making copper-transporting ATPase proteins. These proteins help with the transport of metals into and out of cells. (U.S. National Library of Medicine, 2016).
The patient was transferred into my care via the Emergency Assessment Unit for Surgical Patients (EAUS). I was given handover by the charge nurse who has already pre-a...
Most diseases are caused by a type of genetic component. Many of the diseases that have been caused by gene mutations are undiagnosed. These remain undiagnosed because the disease is so rare that the doctor does not know how to diagnose the patient. Many sy...