scholarly journals Anesthetic Management of a Child with Mitochondrial Neurogastrointestinal Encephalopathy

2015 ◽  
Vol 2015 ◽  
pp. 1-3 ◽  
Author(s):  
Vianey Q. Casarez ◽  
Acsa M. Zavala ◽  
Pascal Owusu-Agyemang ◽  
Katherine Hagan
Keyword(s):  
Case Report ◽  
Anesthetic Management ◽  
Volatile Anesthetic ◽  
Autosomal Recessive Disorder ◽  
Hearing Impairments ◽  
Hematopoietic Stem ◽  
Gastrointestinal Dysmotility ◽  
Anesthetic Agents ◽  
Medication Selection ◽  
Repeated Use

Mitochondrial neurogastrointestinal encephalomyopathy (MNGIE) is an autosomal recessive disorder associated with deficiency of thymidine phosphorylase (TP). Associated manifestations include visual and hearing impairments, peripheral neuropathies, leukoencephalopathy, and malnutrition from concomitant gastrointestinal dysmotility and pseudoobstruction. Given the altered metabolic state in these patients, specific consideration of medication selection is advised. This case report will describe the anesthetic management used in a 10-year-old girl with MNGIE. She had multiple anesthetics while undergoing allogeneic hematopoietic stem cell transplantation. This case report will discuss the successful repeated use of the same anesthetic in this pediatric patient, with the avoidance of volatile anesthetic agents, propofol, and muscle relaxant.

scholarly journals Mitochondrial Neurogastrointestinal Encephalomyopathy (MNGIE-MTDPS1)

2018 ◽  
Vol 7 (11) ◽  
pp. 389 ◽  
Author(s):  
Massimiliano Filosto ◽  
Stefano Cotti Piccinelli ◽  
Filomena Caria ◽  
Serena Gallo Cassarino ◽  
Enrico Baldelli ◽  
...  
Keyword(s):  
Early Diagnosis ◽  
Thymidine Phosphorylase ◽  
Early Adulthood ◽  
Autosomal Recessive Disorder ◽  
Diagnostic Procedures ◽  
Loss Of Function ◽  
Hematopoietic Stem ◽  
Mitochondrial Neurogastrointestinal Encephalomyopathy ◽  
Gastrointestinal Dysmotility ◽  
Near Future

Mitochondrial neurogastrointestinal encephalomyopathy (MNGIE-MTDPS1) is a devastating autosomal recessive disorder due to mutations in TYMP, which cause a loss of function of thymidine phosphorylase (TP), nucleoside accumulation in plasma and tissues, and mitochondrial dysfunction. The clinical picture includes progressive gastrointestinal dysmotility, cachexia, ptosis and ophthalmoparesis, peripheral neuropathy, and diffuse leukoencephalopathy, which usually lead to death in early adulthood. Other two MNGIE-type phenotypes have been described so far, which are linked to mutations in POLG and RRM2B genes. Therapeutic options are currently available in clinical practice (allogeneic hematopoietic stem cell transplantation and carrier erythrocyte entrapped thymidine phosphorylase therapy) and newer, promising therapies are expected in the near future. Since successful treatment is strictly related to early diagnosis, it is essential that clinicians be warned about the clinical features and diagnostic procedures useful to suspect diagnosis of MNGIE-MTDPS1. The aim of this review is to promote the knowledge of the disease as well as the involved mechanisms and the diagnostic processes in order to reach an early diagnosis.

Anesthesia for rabbits submitted to experimental surgeries: Report of a series of eight anesthesias

2020 ◽  
pp. 151-158
Author(s):  
Rafael Antonio Caldart Bedin ◽  
Maisa Schultz ◽  
Antonio Bedin
Keyword(s):  
Muscle Relaxation ◽  
Anesthetic Management ◽  
Laboratory Animals ◽  
Average Weight ◽  
Anesthetic Induction ◽  
Anesthetic Agents ◽  
Reasonable Degree ◽  
Bioethical Debate ◽  
Precordial Stethoscope

Anesthesia for laboratory animals is a matter of biomedical concern and one of the most present dilemmas in the current bioethical debate. The use of anesthetic agents in experimental surgery aims at analgesia and restraining the animal, in order to achieve a reasonable degree of muscle relaxation and to produce sufficient analgesia. This practice requires the use of protocols for the administration of safe and efficient doses. Eight New Zealand rabbits were submitted to laparotomies demonstrating the surgical technique discipline of the local medical course. For pre-anesthetic medication, acepromazine 1 mg.kg-1 associated with ketamine 15 mg.kg-1 was used subcutaneously. Anesthesia was maintained with isoflurane and oxygen under a laryngeal mask in a Mapleson D anesthesia system and under spontaneous breathing. Hydration was performed with 10 ml.kg-1 saline every hour. A thermal mattress was used. Precordial stethoscope, pulse oximetry and clinical parameters were used for monitoring. For euthanasia, ketamine 10 mg.kg-1 associated with potassium chloride 19.1% 1 ml.kg-1 was used intravenously. The average weight of the rabbits was 2721.25 ± 275.01 grams and the duration of the anesthetic procedure was 120 ± 87 minutes. Discussion. In long-term anesthesia, such as laparotomies, the use of pre-anesthetic medication and then anesthetic induction by the combination of agents is recommended. However, anesthetic management requires monitoring to prevent insufficient or excessive doses from occurring.

scholarly journals General anesthesia with remimazolam in a patient with mitochondrial encephalomyopathy: a case report

2021 ◽  
Vol 7 (1) ◽  
Author(s):  
Yuji Suzuki ◽  
Matsuyuki Doi ◽  
Yoshiki Nakajima
Keyword(s):  
Lactic Acidosis ◽  
General Anesthesia ◽  
Mitochondrial Disease ◽  
Muscle Relaxation ◽  
Anesthetic Management ◽  
Perioperative Period ◽  
Mitochondrial Encephalomyopathy ◽  
Anesthesia Induction ◽  
General Anesthetic ◽  
Anesthetic Agents

Abstract Background Systemic anesthetic management of patients with mitochondrial disease requires careful preoperative preparation to administer adequate anesthesia and address potential disease-related complications. The appropriate general anesthetic agents to use in these patients remain controversial. Case presentation A 54-year-old woman (height, 145 cm; weight, 43 kg) diagnosed with mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes underwent elective cochlear implantation. Infusions of intravenous remimazolam and remifentanil guided by patient state index monitoring were used for anesthesia induction and maintenance. Neither lactic acidosis nor prolonged muscle relaxation occurred in the perioperative period. At the end of surgery, flumazenil was administered to antagonize sedation, which rapidly resulted in consciousness. Conclusions Remimazolam administration and reversal with flumazenil were successfully used for general anesthesia in a patient with mitochondrial disease.

scholarly journals Total Intravenous Anesthesia for Myocardial Protection and Preconditioning

2021 ◽  
Author(s):  
Minati Choudhury
Keyword(s):  
Protective Effect ◽  
Surgical Procedure ◽  
Myocardial Protection ◽  
Myocardial Injury ◽  
Volatile Anesthetic ◽  
Organ Protection ◽  
Intravenous Anesthesia ◽  
Intravenous Anesthetic ◽  
Anesthetic Agents ◽  
Anesthetic Preconditioning

AbstractPerioperative myocardial injury is common after any major surgical procedure even with best possible anesthesia and surgical management. Organ preservation during surgical procedure prevents morbidity and mortality. The effect of ischemic preconditioning on myocardial as well as other organ protection is well known. A variety of other agents also shown to have preconditioning thus protective effect on myocardium during anesthesia and surgery. The beneficial effect of volatile anesthetic preconditioning is well studied. However, the effect of intravenous anesthetic agents on this context is still way to go. This review is an attempt to look into the latest available research regarding the preconditioning and myocardial protective effect of intravenous anesthetic agents.

scholarly journals Newer Volatile Anesthetic Agents in Cardiac Anesthesia: Review of Literature

2021 ◽  
Author(s):  
Alka Mandke ◽  
Manjula Sarkar ◽  
Charulata Deshpande ◽  
Arun Maheshwari ◽  
Bhupesh Kumar ◽  
...  
Keyword(s):  
Volatile Anesthetic ◽  
Cochrane Library ◽  
Cochrane Central Register ◽  
Cardiac Anesthesia ◽  
Medical Subject Headings ◽  
Holistic View ◽  
Anesthetic Agents ◽  
Cardiac Anaesthesia ◽  
Mesh Terms ◽  
Inhalational Anaesthesia

AbstractMyocardial protection with volatile anesthetic agents have been suggested by multiple studies. These studies, however, are scattered and are often limited to a particular aspect of cardiac anesthesia. Older inhalational agents like halothane is known to cause significant hepatic damage in patients undergoing long duration surgeries while isoflurane is known to have marked vasodilating properties that also affects the coronary arteries leading to coronary “steal” phenomenon. Additionally, newer agents, like sevoflurane and desflurane, have shown more prominent cardioprotective effects than older agents. We searched ScholarOne, Medline, Embase, and the Cochrane Central Register of Controlled Trials (CENTRAL) in the Cochrane Library. The medical subject headings (MeSH) terms “anaesthesia, inhalational,” “anaesthesia, intravenous, or TIVA,” and “Cardiac anaesthesia or Cardiac Surgery” were used. Additional studies were identified by review of the reference sections of all eligible studies. The aim of this review article is to bring together the evidences with newer inhalational agents and provide a holistic view of their benefits and shortcomings in cardiac anesthesia.

scholarly journals Anesthetic management for cesarean delivery in a woman with congenital atlantoaxial dislocation and Chiari type I anomaly: a case report and literature review

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Yuyan Nie ◽  
Weimin Zhou ◽  
Shaoqiang Huang
Keyword(s):  
Case Report ◽  
Cesarean Delivery ◽  
Anesthetic Management ◽  
Neurological Complications ◽  
Atlantoaxial Dislocation ◽  
Epidural Needle ◽  
Team Approach ◽  
Anesthetic Techniques ◽  
Type I ◽  
Combined Spinal Epidural Anesthesia

Abstract Background The preferable choice of anesthesia for the patients with congenital atlantoaxial dislocation (CAAD) and type I Arnold Chiari malformations (ACM-I) has been a very confusing issue in clinical practice. We describe the successful administration of combined spinal-epidural anesthesia for a woman with CAAD and ACM-1 accompanied by syringomyelia. Case presentation Our case report presents the successful management of a challenging obstetric patient with CAAD and ACM-1 accompanied by syringomyelia. She had high risks of difficult airway and aspiration. The injection of bolus drugs through the spinal or epidural needle may worsen the previous neurological complications. The patient was well evaluated with a multidisciplinary technique before surgery and the anesthesia was provided by a skilled anesthesiologist with slow spinal injection. Conclusions An interdisciplinary team approach is needed to weigh risks and benefits for patients with CAAD and ACM-1 undergoing cesarean delivery. Therefore, an individual anesthetic plan should be made basing on the available anesthetic equipments and physicians’ clinical experience on anesthetic techniques.

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