Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0026850 (muscular dystrophy)
 5,870 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Duchenne muscular dystrophy, an X-linked disorder characterized by progressive muscle weakness, is the most common muscular dystrophy among children leading to death before the end of third decade. Anesthesia in such patients pose a great challenge due to various complications associated with it. The dreaded metabolic and clinical complications occur due to various inhalational anesthetics and succinylcholine in this subset of patients. We are reporting a child with diagnosed Duchenne muscular dystrophy who underwent excision of dentigerous cyst in oral cavity under procedural sedation with combination of dexmedetomidine and fentanyl and thus administration of general anesthesia was avoided.
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PMID:Dexmedetomidine and fentanyl combination for procedural sedation in a case of Duchenne muscular dystrophy. 2588 95

Walker-Warburg syndrome is a rare congenital disorder. Several features, including muscular dystrophy, hydrocephalus, and oropharyngeal abnormalities, have important implications in the perioperative setting. We present a case of general anesthesia in an infant and discuss perioperative considerations to guide clinicians faced with the management of patients with this syndrome.
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PMID:Perioperative considerations in Walker-Warburg syndrome. 2640 Dec 79

Calcinosis cutis is a rare clinical condition wherein calcium is deposited in the layers of the skin. Various types of calcinosis cutis have been described based on histology and location. Airway may be involved only if calcinosis cutis is associated with overlapping syndrome of connective tissue disorders and muscular dystrophy. I report a rare case of dystrophic calcinosis cutis without any connective tissue disorders with the unanticipated difficult airway. A young lady with dystrophic calcinosis cutis of the gluteal region was posted for surgical excision and flap cover procedure under general anesthesia. Unanticipated difficult airway situation arised and was effectively managed. Patient had an uneventful recovery.
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PMID:Calcinosis cutis: A rare cause for difficult airway. 2671 83

Duchenne's muscular dystrophy (DMD) is a neuromuscular disease with a progressive course. It is the most common and most severe muscular dystrophic disorder for which the application of anesthesia is critical, due to muscle weakness, and cardiac and pulmonary involvement. Successful application of spinal anesthesia in a 2-year-old boy with DMD undergoing bilateral inguinal hernia repair is described in the present report. It is proposed that spinal anesthesia is an effective alternative to general anesthesia in certain pediatric patients, including those with DMD, for whom general anesthesia poses increased risk.
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PMID:[Application of spinal anesthesia in a pediatric patient with Duchenne's muscular dystrophy]. 2722 42

Merosin-deficient congenital muscular dystrophy (MD-CMD) is the most common and severe form of congenital muscular dystrophy and is characterized by progressive severe hypotonia due to the absence of the merosin chain around muscle fibers. The main anesthetic concerns include a possible association with malignant hyperthermia, the risk of anesthesia-induced rhabdomyolysis, a difficult airway and postoperative respiratory failure. We report the case of an uneventful general anesthesia (GA) in a two-year-old boy with MD-CMD for the placement of an implantable venous access system. The goal of our anesthetic management was to reduce the risk of respiratory depression. We considered the possibility of loss of spontaneous ventilation against the known, but rare, risk of rhabdomyolysis and we choose for a balanced GA with sevoflurane, short acting opioids and a pressure support ventilation mode instead of a trigger-free anesthesia. Our anesthetic management and the perioperative concerns for this particular syndrome are described.
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PMID:Pressure-support ventilation in a child with merosin-deficient congenital muscular dystrophy under sevoflurane anesthesia. 2987 69


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