Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0011633 (dermatomyositis)
 4,181 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 19-year-old girl suffering from active dermatomyositis was given suxamethonium 60 mg during anaesthesia for termination of pregnancy. A prolonged suxamethonium action occurred which was explained by the finding of homozygous atypical plasmacholinesterase in her blood. Although no fasciculations were seen immediately after injection of the drug, a period of fasciculations progressing from the extremities to the head and trunk occurred during recovery of muscle tone. No hyperpyrexia or elevation of serum creatine phosphokinase occurred. This was ascribed to the steroid therapy she received. Plasma from four other patients suffering from dermatomyositis was also investigated and one young woman, also pregnant, was found to be heterozygous for the atypical enzyme.
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PMID:Dermatomyositis, suxamethonium action and atypical plasmacholinesterase. 62 8

The significant features of neuromuscular blockade with vecuronium in a patient with dermatomyositis are described: vecuronium 0.08 mg/kg resulted in 90%, 0.12 mg/kg in 100% neuromuscular blockade. In contrast to claims made in some previous publications, dermatomyositis did not produce increased sensitivity to vecuronium. Onset time and duration of action were also within normal limits in our patient. Time of spontaneous recovery until antagonism with neostigmine was markedly prolonged, but the dermatomyositis was only one of various possible explanations. Although there are potential hazards in the use of neostigmine in patients with dermatomyositis, antagonism of the neuromuscular block with 2 mg neostigmine was without problems in our patient. Our data support recent suggestions to reconsider the implications of dermatomyositis for anesthesia.
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PMID:[Neuromuscular blockade using vecuronium in dermatomyositis]. 257 54

A 58 year old woman suffering from dermatomyositis underwent elective surgery for spinal caries. Concerning the anesthetic management of patient suffering from dermatomyositis, there is little information on the appropriate use of muscle relaxants. It is generally suspected that the patient is sensitive to nondepolarizing muscle relaxants. Anesthesia was with oxygen-nitrous oxide and fentanyl. Pancuronium 6 mg was given intravenously after awake intubation and an additional dose of 2 mg was given after 7.3 hours. During anesthesia neuromuscular function was monitored by neuromuscular transmission monitor (Datex Relaxograph). Duration of neuromuscular block was defined as the time for the twitch height to recover from total paralysis to 25% of the control value. Duration in this patient was 3.1 hours and this was longer as compared with the values of 1.1-1.8 hours obtained in 7 control patients. It is suggested that a usual dose of muscle relaxants results in a relatively higher effect in the patients with dermatomyositis because of their diminished muscle mass. The anesthetist should be careful in using muscle relaxants. The muscle relaxants should be given to such a patient with monitoring closely the neuromuscular function using a neuromuscular transmission monitor.
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PMID:[Anesthetic management of a patient with dermatomyositis--clinical observation of the effect of muscle relaxants]. 258 19

The anaesthetic management of two patients with severe muscle weakness--one suffering from acute dermatomyositis, the other from acute polymyositis--is described. Both patients presented for surgery for malignancy. Anaesthesia was induced with etomidate in one, thiopentone in the other. Alfentanil was used for analgesia and atracurium for muscle paralysis in both. Neuromuscular blockade was monitored using a peripheral nerve stimulator and no problems were experienced. Recovery of neuromuscular transmission and ventilatory function after operation were normal.
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PMID:Anaesthesia and acute dermatomyositis/polymyositis. 313 34

We report four cases of difficult airway managed with a laryngeal mask airway (LMA) under propofol anaesthesia; this method was chosen to minimize psychological and physical trauma. Case 1 was a boy with dermatomyositis; Case 2, a girl wearing a base ring of a stereotactic frame; Case 3, a boy with Treacher-Collins syndrome; Case 4, a boy with Goldenhar syndrome. They were anaesthesized with propofol and though spontaneous ventilation became shallower, or apnoea occurred, the stimulus of inserting an LMA awakened the patient and caused more active spontaneous ventilation while a patent airway was established by the LMA. All tracheal intubations were performed smoothly and without delay through the LMA, and we believe that psychological and physical trauma was thus minimized.
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PMID:Management of difficult airways with a laryngeal mask airway under propofol anaesthesia. 1018 60

A 72-year-old woman diagnosed with critical descending aortic stenosis was scheduled for endovascular treatment by angioplasty and implantation of an aortic stent. Her medical history included arterial hypertension, lipid metabolic disorder, obesity, Takayasu disease, dermatopolymyositis, and alleged allergy to iodine contrast and local anesthetics. After the allergies were ruled out, it was decided to use a regional anesthetic technique to avoid the postoperative complications of general anesthesia and achieve better hemodynamic control during surgery. Surgery was carried out under epidural anesthesia and intravenous sedation. After angioplasty and during self-expansion of the stent, the patient's hemodynamics deteriorated rapidly; she lost consciousness and required orotracheal intubation and immediate resuscitation measures. The literature describes in detail the management of patients with thoracic aortic lesions, including the most appropriate way to provide anesthesia. General anesthesia seems to be preferred, although care is taken to individualize the decision. We analyze this case of a patient with severe thoracic aortic stenosis undergoing endovascular treatment under epidural anesthesia.
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PMID:[Severe hemodynamic deterioration during epidural anesthesia for endovascular treatment of thoracic aortic stenosis]. 1628 44

A 66-year-old female with dermatomyositis and severe respiratory failure from collagen lungs, dependent on domiciliary oxygen therapy, was scheduled for a mastectomy. Anesthesia was induced with dexmedetomidine (DEX) 6 microg x kg(-1) x h(-1) for 10 minutes and maintained at 0.7 microg x kg(-1) x h(-1), along with a target controlled infusion of propofol combined with epidural anesthesia. No narcotic or muscle relaxant was used. The airway was secured using a laryngeal mask airway and spontaneous breathing was preserved. The perioperative course was uneventful without any pulmonary complications. DEX has a certain analgesic property and a mild respiratory depressant effect. Therefore, it is considered useful as a concomitant anesthetic agent for perioperative management of patients with respiratory failure.
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PMID:[Validity of intraoperative administration of dexmedetomidine in a patient with dermatomyositis-derived respiratory failure]. 1705 85

Our patient showed major abdominal cutaneous necrosis. Detersion removed the entire thickness of half of the right-hand wall of the abdomen. We are going to explain how, by combining well known procedures, we conducted this closure. This deals with a patient aged 53, with a long case history of dermatomyositis and highly debilitating sub-cutaneous calcinosis. This patient has been treated with Imurel and high doses of corticoids since 1997. In the face of the much debilitated terrain of the patient, it was not certain that a local flap or even a pediculated flap could be made to cover this loss of substance with a minimum of risk. A cutaneous extension was then envisaged using a system of Wisebands fillets. To protect the parietal plate, accelerate its growth and reduce the skin tension, we used in combination a system of foam dressing with negative pressure therapy (NPT). The optimized NPT was used for 2 weeks. The Wisebands were installed for 1 month. The treatment lasted for 50 days and required five short sessions of general anaesthesia. The histopathological interpretation revealed an EBV lymphoma. The assessment of the extension and the therapeutic treatment of the lymphoma contributed to the duration of hospitalisation and the number of general anaesthesia sessions. The synergy effect of these two associated procedures have allowed a faster skin closure; 18 months later, no complications have occurred. The wound has closed totally and the abdominal wall is solid in spite of not having resorted to a flap or separation.
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PMID:[Optimized negative pressure therapy. Case report]. 1919 81

A 59-year old woman had been suffering from myalgia, eruption and dyspnea on effort for a month. She was referred to our hospital because her symptoms were not improved by antibiotic therapy at a clinic. At first presentation she showed cutaneous manifestations including heliotrope eyelids and mechanic's hands, slightly elevated serum creatine kinase (CK), elevated serum C-reactive protein level, and interstitial pneumonia (IP), which led to a diagnosis as dermatomyositis. After admission to our hospital, her hypoxia due to IP progressed despite disappeared myalgia and normalized serum CK level. Intravenous steroid pulse therapy followed by oral cyclosporine and intravenous cyclophosphamide pulse therapy was not effective for the IP for more than a month. Gastroscopy revealed superficial depressed (0-IIc) type early gastric cancer. However, it was out of indication for endoscopic mucosal resection because of the histopathologic type (signet ring cell carcinoma) and possible submucosal invasion. In addition to those immunosuppressive therapies, proximal gastrectomy with total intravenous anesthesia was performed. One month after the operation, improvement of IP as well as cutaneous manifestations was confirmed by respiratory function test, pulmonary computed tomography, and discontinuance of oxygen inhalation. We conclude that this was a case of clinically amyopathic dermatomyositis with rapidly progressive IP and gastric cancer, which was ameliorated by a combination of medication (steroid, cyclosporine, and cyclophosphamide) and surgery (gastrectomy for early gastric cancer).
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PMID:[Case of immunosuppressant-resistant amyopathic dermatomyositis with rapidly progressive interstitial pneumonia ameliorated after resection of gastric cancer]. 2279 May 79

Dermatomyositis (DM) is one of the idiopathic inflammatory myopathies caused by complement-mediated vasculopathy or vasculitis in the muscle. Although the gastrointestinal (GI) mucosa has been reported to be involved as a result of vasculitis or vasculopathy, ulceration in the pharynx is a rare manifestation of DM. A 54-year-old woman complaining of muscle weakness in the extremities, low-grade fever, and dysphagia was diagnosed as having DM. Despite medical treatment with corticosteroids and immunosuppressive agents, her DM progressed rapidly, leading to exacerbation of the dysphagia. About 3 weeks after undergoing tracheostomy as a preventive measure against aspiration, the patient developed intractable respiratory tract hemorrhage. Repeated laryngoendoscopy revealed a bleeding ulceration in the pharynx that required hemostasis with electric cautery under general anesthesia. No bleeding recurred thereafter. Histopathologically, the pharynx exhibited nonspecific inflammatory cell infiltration in the muscle tissue. This rare manifestation may be considered in cases of DM with unexplainable airway bleeding.
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PMID:Adult dermatomyositis with bleeding ulcer in the pharynx. 2532 39


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