Anesthesia recommendations in patients suffering from Arthrogryposis multiplex congenita
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چکیده
Disease name: Arthrogryposis multiplex congenita ICD 10: Q74.32 Synonyms: Arthrogryposis, Amyoplasia Congenita, Congenital Arthromyodysplasia, GuérinStern syndrome, Myodystrophia Fetalis Deformans AMC is the clinical description of a congenital syndrome with an estimated incidence of 1:3000-10,000 [25,30,38]. Already within the uterus, the movement frequency of the foetus is reduced due to multiple – mostly symmetrical – joint contractures. Primarily, the great joints of extremities, the spine [12] but also the otolaryngologic region [10,11] are affected with varying severity. Furthermore, a reduced muscle mass and a merely developed subcutaneous tissue are notable. During growth, numerous deformities are generated. Other organ systems can be involved (CNS, cardiovascular system, lungs, gastrointestinal tract, urogenital tract, abdominal wall). In most cases, patients develop normal intelligence.
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Characterization of a group unrelated patients with arthrogryposis multiplex congenita.
OBJECTIVE Arthrogryposis multiplex congenita is a relatively rare neuromuscular syndrome, with a prevalence of 1:3000-5000 newborns. In this study, the authors describe the clinical features of a group of 50 unrelated Mexican patients with arthrogryposis multiplex congenita. METHODS Patients were diagnosed by physical and radiographic examination and the family history was evaluated. RESULT...
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We have presented a case of a 22-year-old patient having a rare variety of arthrogryposis multiplex congenita arthrogryposis with epileptic seizures and defect in neural migration. We have described the patient’s disease history, the clinical, and laboratory data by giving prominence to the lack of mental retardation and the late onset of the generalized tonic-clonic seizures, despite the prese...
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Arthrogryposis multiplex congenita (AMC) is a symptom complex of congenital joint contractures associated with neurogenic and myopathic disorders. It is diagnosed at birth and often progresses to a state of significant disability. Pregnancy in a woman with AMC is at high risk due to diminished pulmonary reserve, increased risk of thromboembolism and anesthesia. Successful pregnancy in patients ...
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Corresponding author: Burn Young Heo, M.D., Ph.D., Department of Anesthesiology and Pain Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, 81, Irwon-ro, Gangnam-gu, Seoul 135-710, Korea. Tel: 82-2-3410-0736, Fax: 82-2-3410-6626, E-mail: [email protected] This is an open-access article distributed under the terms of the Creative Commons Attribution Non-Commerc...
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Arthrogryposis Multiplex Congenita is a condition characterized by multiple contractures of the joints. Involvement of the temporomandibular joint is a common complication that limits mandibular opening. A case of Arthrogryposis Multiplex Congenita with anterior open bite and limited maximal incisal opening is presented. Orthognathic surgery, consisting of segmental LeFort I osteotomy and bilat...
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