Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0026827 (hypotonia)
 5,860 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Muscle relaxant drugs are the most frequent cause of anaphylactic and anaphylactoid reactions during anaesthesia. We report a case of a life-threatening anaphylactic reaction during induction of anaesthesia with severe bronchospasm as the first clinical symptom. Mechanical ventilation was nearly impossible. The patient required a multimodal antiallergic therapy and a high-dose catecholamine therapy for stabilization. Rocuronium was identified as the allergic agent using intradermal testing.
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PMID:[Severe anaphylaxis from rocuronium]. 1692 17

Central core disease (CCD) is an inherited neuromuscular disorder characterised by central cores on muscle biopsy and clinical features of a congenital myopathy. Prevalence is unknown but the condition is probably more common than other congenital myopathies. CCD typically presents in infancy with hypotonia and motor developmental delay and is characterized by predominantly proximal weakness pronounced in the hip girdle; orthopaedic complications are common and malignant hyperthermia susceptibility (MHS) is a frequent complication. CCD and MHS are allelic conditions both due to (predominantly dominant) mutations in the skeletal muscle ryanodine receptor (RYR1) gene, encoding the principal skeletal muscle sarcoplasmic reticulum calcium release channel (RyR1). Altered excitability and/or changes in calcium homeostasis within muscle cells due to mutation-induced conformational changes of the RyR protein are considered the main pathogenetic mechanism(s). The diagnosis of CCD is based on the presence of suggestive clinical features and central cores on muscle biopsy; muscle MRI may show a characteristic pattern of selective muscle involvement and aid the diagnosis in cases with equivocal histopathological findings. Mutational analysis of the RYR1 gene may provide genetic confirmation of the diagnosis. Management is mainly supportive and has to anticipate susceptibility to potentially life-threatening reactions to general anaesthesia. Further evaluation of the underlying molecular mechanisms may provide the basis for future rational pharmacological treatment. In the majority of patients, weakness is static or only slowly progressive, with a favourable long-term outcome.
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PMID:Central core disease. 1750 18

After collapsing, a 70-year-old patient with certain comorbidities developed an extensive hematoma of the lower leg because of anticoagulation-therapy. For operative intervention the patient received general anesthesia, because of the risk of bleeding. As a consequence a severe hypotonia occurred with renal failure. For revision, the patient received peripheral regional anesthesia despite an elevated bleeding risk. Risk-benefit analysis in case of an elevated bleeding risk while performing peripheral regional anesthesia will be discussed.
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PMID:[Case report: bleeding complications and regional anesthesia--risks and benefits]. 1766 Dec 61

We report the successful management of anesthesia in a patient with stiff-person syndrome (SPS) undergoing a thymectomy using a volatile anesthetic combined with epidural anesthesia. The anesthetic concern in patients with SPS is the possibility of postoperative hypotonia due to the presence of excessive gamma-aminobutyric acid (GABA) resulting from an interaction between the anesthetic agents and preoperatively taken therapeutic drugs. Epidural anesthesia has the advantages of decreasing the required amount of anesthetics with GABAergic action, and relieving the postoperative pain that causes the symptoms of SPS. Epidural anesthesia could be a useful technique in SPS patients.
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PMID:Considerations for general anesthesia combined with epidural anesthesia in a patient with stiff-person syndrome. 1800 16

Percutaneous endoscopic gastrostomy (PEG) is a common technique for gastrostomy placement. However, certain children may not be candidates for PEG, such as those with craniofacial or foregut anomalies and prior surgery. Laparoscopic gastrostomy has also gained popularity, but this requires 2 or 3 trocar sites. The use of a larger single operating laparoscope or multiple-port laparoscopic techniques may not be practical in small children and infants. We describe a simple technique for gastrostomy tube placement in infants using a 4-mm operative bronchoscope. A 1.4-kg infant with a cleft palate and hypotonia underwent general anesthesia. A 5-mm laparoscopic port was placed in the left upper quadrant at the site of the intended gastrostomy. Following pneumoperitoneum, a 4-mm bronchoscopic optical grasper was inserted into the abdomen via the single port. The stomach was grasped and pulled out through the port site. The extracorporeal portion of stomach was matured as a gastrostomy. A low-profile gastrostomy button was placed. Proper position of the gastrostomy device was verified intraoperatively using dye. At 2 months follow-up, the child and gastrostomy are without complication. This technique is minimally invasive and provides direct visualization through one 5-mm abdominal port without the requirement of endoscopy and blind percutaneous entrance into the abdominal cavity. This single-site laparoscopic gastrostomy may be a practical alternative for infants who may not be candidates for PEG or larger single-port operating systems.
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PMID:Single site laparoscopic gastrostomy with a 4-mm bronchoscopic optical grasper. 2015 77

Pudendal nerve block may be indicated during instrumental delivery in situations where peridural anesthesia is unavailable. We report three cases of neonatal lidocaine intoxication following maternal pudendal block during delivery. Clinical features were hypotonia, pupillary mydriasis fixed to light, apnea, cyanosis and seizures. Two neonates required mechanical ventilation. Lidocaine was found in the serum of two babies. In all three cases, recovery was complete. The pharmacokinetics of lidocaine in a highly vascularized perineum during labor increase the risk of neonatal intoxication. A possible intoxication by local anesthetics should be considered in neonates presenting an acute distress in the delivery room.
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PMID:[Lidocaine intoxication in newborn following maternal pudendal anesthesia: report of three cases]. 1840 71

Stiff-person syndrome is a rare disease characterized by muscle rigidity and painful spasms in the axial and limb muscles. The authors reported here a case of an axilally lymphadenectomy in a 46-year-old woman with stiff-person syndrome. With train of four ratio (TOFR) monitoring at the ulnar nerve, general anesthesia was induced and maintained with fentanyl, vecuronium and propofol with target controlled infusion. A TOFR, BIS monitor and invasive arterial pressure monitoring were employed. During the operation, there was no muscle rigidity and spasm. Ten minutes after the operation, she was fully awake and train of four ratio recovered to 95%, and extubated uneventfully. We chose propofol, because of previous reports about prolonged hypotonicity by interaction of baclofen and isoflurane. Preoperative good symptom control, choice of total intravenous anesthesia (TIVA), and application of the electrical nerve stimulator prevented postoperative hypotonia and resulted in safe anesthetic management.
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PMID:[Anesthesia for a patient with stiff-person syndrome]. 1841 1

Stuve Wiedemann syndrome (SWS) is an autosomal recessively inherited syndrome which is characterized by bowing of the long bones, camptodactyly, facial dysmorphism, hypotonia, feeding and swallowing difficulties, and respiratory distress. In most cases episodes of unexplained hyperthermia are present. Patients with SWS can develop hyperthermia in conjunction with anesthesia and surgery, and a relationship has been suggested between the syndrome and malignant hyperthermia. We describe a 3-year-old child diagnosed with SWS to whom we administered general anesthesia during the removal of a corneal ulcer and dilatation of the lacrimal duct. Our patient had received, uncomplicated, inhalational anesthesia five times previously for different operations. There were no anesthesia-related complications in the present or previous perioperative periods. On one occasion the patient developed mild postoperative hyperthermia. We believe that this hyperthermia is different from the specific disorder of malignant hyperthermia and that sevoflurane can be safely used in patients with SWS. We also describe symptomatically related syndromes and their theoretical risks for anesthesia.
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PMID:Stuve Wiedemann syndrome and related syndromes: case report and possible anesthetic complications. 1920 41

Patients with upper airway obstruction during sleep are at constant risk of hypoxic and hypercarbic episodes and are especially vulnerable during anaesthesia and sedation as the abnormal anatomy is compounded by drug-related respiratory depression. Elective procedures in patients with the obstructive sleep apnoea (OSA) should be usually delayed, allowing for the preoperative home treatment (diet, alcohol abstinence, nasal CPAP/BiPAP during night). Respiratory supportive techniques, started at home, should be continued in the hospital, both in preoperative and postoperative periods. Patients with OSA should be also thoroughly examined for possible anatomic abnormalities of the upper airway that may complicate laryngoscopy and/or intubation. Heavy premedication should be avoided; in special cases of very nervous patients oral clonidine may be used. Careful preoxygenation is mandatory, opioids should be used sparingly. Muscle relaxant should be calculated for an ideal body weight. Isoflurane should be avoided. The OPS and obese patients are usually extubated in the sitting or lateral positions to avoid limitation of FRC by elevated diaphragm. In selected cases, prolonged intubation and/or ventilation are recommended. Regional anaesthesia are usually safe in these patients, however, opioids should be used carefully. When sedation is required, ketamine or dexmedetomidine may be used.
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PMID:[Perioperative risk in patients with sleep apnoea]. 1946 21

Joubert syndrome is a rare autosomal recessive disorder, which is characterized by absence or underdevelopment of the cerebellar vermis and severe developmental delay. The other common features include ataxia, an abnormal breathing pattern, abnormal eye movements and hypotonia. We report the anesthetic management in a 13-year-old girl with Joubert syndrome, scheduled for cauterization of nasal mucosa under general anesthesia. She had episodes of tachypnea and apnea. Oral midazolam 10 mg and famotidine 20 mg were administered 30 min before surgery. Anesthesia was induced and maintained with sevoflurane and nitrous oxide in oxygen. Vecuronium 2 mg was used to facilitate tracheal intubation. Mechanical ventilation was performed with a low ventilation setting of respiratory rate 5 beats x min(-1) and peak inspiratory pressure 9 cm H2O to maintain normal end-tidal CO2. Flurbiprofen axetil 30 mg was administered intravenously for analgesia, because opioids are not recommended. After reversal of muscle relaxation by atropin 0.5 mg and neostigmine 1.5 mg, her trachea was extubated. She did not develop postoperative apnea. In this patient with Joubert syndrome, midazolam, sevoflurane, nitrous oxide and flurbiprofen axetil were used without any complications.
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PMID:[General anesthesia for a girl with Joubert syndrome]. 2022 61


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