Small Doses Muscle Relaxant in General Anesthesia
Muscle RelaxantThis study will be held to assess the effect of minimal dose muscle relaxant on postoperative pulmonary function. The time to extubation in minutes will be the primary outcome as a clinical indicator of the return of muscle power. The extubation time will be defined as the time from injecting the muscle relaxant reversing agent given when regain the train of four (TOF) ratio to 0.9 till removal of endotracheal tube. Intraoperative surgical conditions will be assessed in the form of surgeon satisfaction and the need for more muscle relaxant boluses. Postoperative complications as desaturation (peripheral oxygen saturation (Spo2) less than 90%), the need for re-intubation or ventilation support will be recorded. Patient lung will be assessed using ultrasound-based lung aeration score. Also, the diaphragmatic and intercostal muscle function will be assessed in the early postoperative period. Immediate postoperative pulmonary function tests will be evaluated using simple spirometer. Patients will be followed up for 28 days for detection of pulmonary complications.
Withdrawal of Tiratricol Treatment in Males With Monocarboxylate Transporter 8 Deficiency (MCT8...
Monocarboxylate Transporter 8 DeficiencyAllan-Herndon-Dudley SyndromeThis is a double-blind, randomized phase 3 multicenter placebo-controlled study in at least 16 evaluable male participants diagnosed with MCT8 deficiency. Male participants, from 4 years of age (at randomization) and having demonstrated stable maintenance treatment with tiratricol, will be randomized to receive placebo or tiratricol for 30 days or until reaching rescue criterion (serum total triiodothyronine [T3] > upper limit of normal [ULN] of the participant's normal range, for a sample collected during the 30-day Randomized Treatment Period). The research hypothesis to be tested is that, for participants in the placebo group, removal of tiratricol will lead to an increase of serum total T3 concentration, measured by liquid chromatography with tandem mass spectrometry (LC/MS/MS), above the ULN and requirement of rescue treatment with tiratricol, compared to those who continue to receive tiratricol.
Self-Supporting Nasopharyngeal Airway (ssNPA) Treating Upper Airway Obstruction in Hypotonia
Obstructive Sleep ApneaHypertonia5 moreThe researchers are investigating if the Self-Supporting Nasopharyngeal Airway (ssNPA) device can be used in the treatment of obstructive sleep apnea in children with Hypotonic Upper Airway Obstruction (HUAO).
Osteopathic Treatment on Motor Development of Hypotonic Infants.
HypotoniaInterventional study involving the human at risk and minimal constraint (RIPH2), randomized single-blind comparing the impact of osteopathic treatment on the axial tone of the hypotonic infant versus simulation
Muscle Relaxants and Laryngeal Local Anesthetics for Laryngeal Mask Airway Insertion Decreasing...
AnesthesiaGeneralLaryngeal mask airway (LMA) is currently the most widely used supraglottic airway device with advantages of simple and fast placement, reduced anesthesia drug use, more stable hemodynamics, and less throat discomfort after anesthesia compared to endotracheal intubation. Some studies claimed the use of muscle relaxants or local anesthetics (sprays or lubricants containing local anesthetics) for the throat before LMA placement can reduce the dosage of induction agents and postoperative throat complications. In modern society, more and more elderly people undergo elective surgery with the need of general anesthesia. However, they are the more vulnerable population with tendency of greater hemodynamic changes with more induction agent usage. Propofol is one of the most used induction agents which may lead to a drop in blood pressure. The objective of this study is to observe whether the dose of propofol and the changes in hemodynamics can be reduced by using muscle relaxants or laryngeal local anesthetics in elderly who receiving general anesthesia with LMA insertion.
Myotonometer Intra- and Inter Examiner Reliability on Spinal Muscular Atrophy
Muscle Tone PoorSpinal Muscular AtrophyThe primary aim of the study was to measure the intra-rater and inter-rater reliability of MyotonPRO in measuring postural muscle tone and mechanical properties in individuals with spinal muscular atrophy (SMA). The secondary aim is to question the existence of a relationship between the functional levels of individuals with SMA and their muscle tone and biomechanical properties. It is assumed that the outputs to be obtained from this research will form the norm data for moyotonometer evaluation in children with SMA.
MCT8 Deficiency Caregiver Study
Monocarboxylate Transporter 8 (MCT8) DeficiencyAllan-Herndon-Dudley SyndromeCaregivers face many responsibilities outside of their role as a friend or parent, which can lead to emotional, financial, social, and professional challenges. To better understand the impact of MCT8 deficiency on caregivers, Egetis Therapeutics are conducting an online survey for adult caregivers of persons living with the MCT8 deficiency.
A Natural History Study of hnRNP-related Disorders
Neurodevelopmental DisordersIntellectual Disability6 moreThe purpose of this study is to analyze patterns in individuals with hnRNP (and other) genetic variants, including their neurological comorbidities, other medical problems and any treatment. The investigators will maintain an ongoing database of medical data that is otherwise being collected for routine medical care. The investigators will also collect data prospectively in the form of questionnaires, neuropsychological assessments, motor assessments, and electroencephalography to examine the landscape of deleterious variants in these genes.
Effect of Ulinastatin on the Action of NDMRs (Rocuronium / Cisatracurium)
Nondepolarising Muscle RelaxantsThe aim of this research was to determine the influence of ulinastatin on nondepolarising muscle relaxants Rocuronium and Cisatracurium.
Remifentanil Without Muscle Relaxant for Thoracotomy
Elective Open ThoracotomyAlthough the administration of muscle relaxation is essential standard of care for thoracic procedures, it could cause long-reversal times and postoperative residual curarization (PORC) increasing length of post-anesthesia care unit (PACU) stay and hospital costs. Sugammadex offers new perspectives to reduce the incidence of PORC. Unfortunately it is not available in many countries because of its significantly high cost. We hypothesized that the use of target-controlled remifentanil infusion (TCI) with the non-muscle relaxant (NMR) would be associated with comparable surgical conditions and reduced total costs compared with the use of neuromuscular blockers during thoracotomy. After ethical approval, 66 patients scheduled for elective thoracotomy under sevoflurane anesthesia with TCI remifentanil will be included in this prospective, randomized, single-blind, controlled study. Patients will be randomly assigned to receive cisatracurium or saline (n = 33 for each group) throughout the procedure. Laryngoscopy and intubating conditions, intraoperative modified thoracic surgery rating scale (Table below), incidence of light anesthesia, and use of vasopressors and anesthetics, clinical recovery, incidence of PORC, PACU and hospital stays, and total costs will be recorded.