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Total 115 results found since Jan 2013.

Anesthetic considerations in a parturient with Freeman-Sheldon syndrome
Freeman-Sheldon Syndrome (FSS) also known as cranio-carpal-tarsal dysplasia, Windmill-Vane-Hand syndrome, distal arthrogryposis type 2 and whistling face syndrome, is a rare congenital myopathy disorder.1 First reported in 1938, it is described by facial and skeletal abnormalities, the most common being microstomia with pouting lips, campodactyly, ulnar deviation of the fingers and talipes equinovarus.2 Facial characteristics include micrognathia, microglossia, high arched palate, vertical skin folds in the jaw and ‘mask-like’ facies.
Source: International Journal of Obstetric Anesthesia - February 11, 2016 Category: Anesthesiology Authors: K. Fisher, F. Qasem, P. Armstrong, I. McConachie Tags: Case report Source Type: research

Anesthetic considerations in a parturient with Freeman–Sheldon syndrome
Freeman–Sheldon syndrome (FSS) also known as cranio-carpal-tarsal dysplasia, Windmill-Vane-Hand syndrome, distal arthrogryposis type 2 and whistling face syndrome, is a rare congenital myopathy disorder.1 First reported in 1938, it is described by facial and skeletal abnormalities, the most common being microstomia with pouting lips, camptodactyly, ulnar deviation of the fingers and talipes equinovarus.2 Facial characteristics include micrognathia, microglossia, high arched palate, vertical skin folds in the jaw and ‘mask-like’ facies.
Source: International Journal of Obstetric Anesthesia - February 10, 2016 Category: Anesthesiology Authors: K. Fisher, F. Qasem, P. Armstrong, I. McConachie Tags: Case report Source Type: research

Anesthetic considerations in a parturient with Freeman –Sheldon syndrome
Freeman –Sheldon syndrome (FSS) also known as cranio-carpal-tarsal dysplasia, Windmill-Vane-Hand syndrome, distal arthrogryposis type 2 and whistling face syndrome, is a rare congenital myopathy disorder.1 First reported in 1938, it is described by facial and skeletal abnormalities, the most common being microstomia with pouting lips, camptodactyly, ulnar deviation of the fingers and talipes equinovarus.2 Facial characteristics include micrognathia, microglossia, high arched palate, vertical skin folds in the jaw and ‘mask-like’ facies.
Source: International Journal of Obstetric Anesthesia - February 10, 2016 Category: Anesthesiology Authors: K. Fisher, F. Qasem, P. Armstrong, I. McConachie Tags: Case report Source Type: research

Role of noninvasive ventilation in perioperative patients with neuromuscular disease: a clinical case
Publication date: January–February 2016 Source:Brazilian Journal of Anesthesiology (English Edition), Volume 66, Issue 1 Author(s): Ana Marinho, Mario José Guimarães, Neusa Cristina Ribeiro Lages, Carlos Correia The inclusion body myositis is an inflammatory myopathy that leads to chronic muscle inflammation associated with muscle weakness. It is characterized by a restrictive ventilatory syndrome requiring ventilatory support under non-invasive ventilation. The authors describe a clinical case and the anaesthetic management of a patient with inclusion body myopathy candidate for vertebroplasty, which highlights ...
Source: Brazilian Journal of Anesthesiology - January 11, 2016 Category: Anesthesiology Source Type: research

Neuromuscular blocking effects of cisatracurium and its antagonism with neostigmine in a canine model of autosomal-recessive centronuclear myopathy
Conclusions The spontaneous return of T2 was not different between groups. However, neostigmine-facilitated recovery was significantly slower in dogs with CNM. Canine autosomal-recessive CNM does not preclude the use of cisatracurium or its antagonism with neostigmine.
Source: British Journal of Anaesthesia - November 18, 2015 Category: Anesthesiology Authors: Martin-Flores, M., Pare, M. D., Campoy, L., Gleed, R. D. Tags: Translational Research Source Type: research

Telephone enquiries on the topic of malignant hyperthermia : Evaluation of the content and subsequent diagnostic results at the MH Center Leipzig.
CONCLUSION: Private individuals and families affected by a MH disposition often showed good compliance with respect to counseling and diagnostics for MH and contacted the MH center more often than medical doctors. A more comprehensive cooperation with the medical profession is preferable and necessary to obtain a systematic and broad synopsis of characteristic and uncharacteristic signs and symptoms of MH. The telephone conversations analyzed as well as the diagnostic results (IVCT and genetic testing) underline that MH disposition is still a current and relevant topic. PMID: 26481388 [PubMed - as supplied by publisher]
Source: Der Anaesthesist - October 19, 2015 Category: Anesthesiology Authors: Petersen B, Busch T, Meinecke CD, Börge B, Kluba K, Kaisers UX, Rüffert H Tags: Anaesthesist Source Type: research

Dystrophinopathies
Semin Neurol 2015; 35: 369-384DOI: 10.1055/s-0035-1558982The dystrophinopathies fall along a spectrum of muscular dystrophy phenotypes, with variable involvement of skeletal and cardiac muscle. The diagnosis of dystrophinopathy should be suspected in any patient with a highly elevated creatine kinase level beyond the context of rhabdomyolysis secondary to toxic or metabolic myopathy. Genetic testing for dystrophinopathy is highly sensitive and specific, and identifying a proband will often lead to implications for several relatives at risk for cardiomyopathy, weakness, or anesthetic reactions. Management of the dystrophino...
Source: Seminars in Neurology - October 6, 2015 Category: Neurology Authors: Brandsema, John F.Darras, Basil T. Source Type: research

The kalaemic and neuromuscular effects of succinylcholine in centronuclear myopathy: A pilot investigation in a canine model
We examined the kalaemic and neuromuscular effects of succinylcholine in dogs with and without autosomal-recessive CNM. DESIGN: A prospective, experimental study. SETTING: Anaesthesiology laboratory, College of Veterinary Medicine, Cornell University, New York, USA. PATIENTS: Six dogs with autosomal-recessive CNM and six control dogs. INTERVENTIONS: Dogs received succinylcholine 0.3 mg kg−1 during isoflurane anaesthesia. MAIN OUTCOME MEASURES: Whole blood potassium concentration was measured 5 min before and after succinylcholine administration. Neuromuscular function was measured with acceleromyography and single ...
Source: European Journal of Anaesthesiology - September 2, 2015 Category: Anesthesiology Tags: Neuromuscular blocking agents Source Type: research

Characteristics of anesthesia in patients with MELAS syndrome : Case report of anesthesia in video-assisted thoracoscopy.
Abstract The mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes (MELAS) syndrome is a disease triggered by a disorder in energy production within mitochondria. The cause of this syndrome is a mutation in the mitochondrial DNA where in 80 % of cases an A-to-G mutation is present at nucleotide 3243 and with a prevalence of 18.4/100,000 in the population. Predominantly affected are organ systems with a high energy metabolism, such as the heart, brain and musculature. During the premedication visit a thorough patient history and examination with respect to neurological impairments must ...
Source: Der Anaesthesist - August 28, 2015 Category: Anesthesiology Authors: Haas A, Wappler F Tags: Anaesthesist Source Type: research

Cardiopulmonary Resuscitation Interface Adapted for Postextubation Continuous Noninvasive Ventilatory Support
ABSTRACT: The authors report that a new oral interface designed for cardiopulmonary resuscitation and use during anesthesia permitted the successful extubation of an “unweanable” 27-yr-old woman with nemaline rod myopathy to continuous noninvasive ventilatory support. She had failed two previous extubation attempts. Tracheotomy and institutional care were avoided as a result.
Source: American Journal of Physical Medicine and Rehabilitation - August 22, 2015 Category: Rehabilitation Tags: Case Report Source Type: research

Advances in the Care of Adults With Congenital Heart Disease
The significant decline in mortality among children and adolescents with congenital heart disease (CHD) is associated with an increasing prevalence of CHD in adults, particularly those with moderate to severe defects. As a significant percentage of adolescents and young adults are lost to follow-up in the transition from pediatric to adult care, they may present for elective procedures with substantial CHD-associated morbidity. In addition to the specific cardiac defect, the procedures performed, and the current pathophysiological status, several factors should be considered when managing the adult with CHD. These include ...
Source: Seminars in Cardiothoracic and Vascular Anesthesia - August 18, 2015 Category: Anesthesiology Authors: Nasr, V. G., Kussman, B. D. Tags: Reviews Source Type: research

Twenty years later: a single‐centre, repeat retrospective analysis of equine perioperative mortality and investigation of recovery quality
Conclusion and clinical relevanceAnaesthetic/recovery‐associated mortality was comparable to previously reported figures except intra‐operative deaths were not reported. Fractures remained responsible for the largest proportion of recovery‐associated deaths. Improvements to the recovery process that can reduce fracture occurrence are still required.
Source: Veterinary Anaesthesia and Analgesia - June 17, 2015 Category: Veterinary Research Authors: Alexandra HA Dugdale, Jessica Obhrai, Peter J Cripps Tags: Research Paper Source Type: research

RYR1‐related myopathies: a wide spectrum of phenotypes throughout life
ConclusionsThis broad range of RYR1‐related disorders often presents to the general paediatric and adult neurologist. Its recognition is essential for genetic counselling and improving patients' safety during anaesthesia. Future research should focus on in vitro testing by the in vitro contracture test and functional characterization of the large number of RYR1 variants whose precise effects currently remain uncertain.
Source: European Journal of Neurology - May 11, 2015 Category: Neurology Authors: M. Snoeck, B. G. M. Engelen, B. Küsters, M. Lammens, R. Meijer, J. P. F. Molenaar, J. Raaphorst, C. C. Verschuuren‐Bemelmans, C. S. M. Straathof, L. T. L. Sie, I. F. Coo, W. L. Pol, M. Visser, H. Scheffer, S. Treves, H. Jungbluth, N. C. Voermans, E.‐ Tags: Original Article Source Type: research

Sugammadex usage in a patient with dermatomyositis
We present a patient with DM who needed urgent abdomen surgery.
Source: Journal of Clinical Anesthesia - April 21, 2015 Category: Anesthesiology Authors: Pınar Kendigelen, Ayse Cigdem Tutuncu, Gulruh Ashyralyyeva, Elif Aybike Hamamcioglu, Guner Kaya Tags: Correspondence Source Type: research