Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0025362 (mental retardation)
 15,878 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 36-year-old woman weighing 31.7 kg with mental retardation was scheduled for dental treatment under general anesthesia. She had undergone anticonvulsant therapy (phenytoin, clonazepam, zonisamide) for years. Standard monitors and bispectral index (BIS) monitor were applied except for an accelomyography. Anesthesia was induced with propofol and vecuronium, and maintained with nitrous oxide in oxygen, with 1.5-2.0% end-tidal concentration of sevoflurane. Forty minutes after induction of anesthesia, spontaneous respiration (SR) started suddenly despite adequate depth of anesthesia (BIS value 35-40). Vecuronium 1 mg was administered and SR stopped immediately. After the event, however, SR started repeatedly and then additional vecuronium was administered every 30-40 minutes to stop SR until the end of the treatment. During the treatment, no factors (hypercapnia, hypoxemia, hyperthermia and so on) to shorten the muscle relaxation were observed. The treatment finished uneventfully She became awake rapidly and extubated. Post-extubation period was also uneventful. In this case, chronic phenytoin therapy induced resistance to vecuronium was highly suspected; however, since clonazepam and zonisamide have elevation effects on blood concentration of phenytoin, they might be also cofactors in resistance to vecuronium. Therefore, patients undergoing chronic anticonvulsant therapy should be paid more attention because they have resistance to neuromuscular blocking drugs.
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PMID:[Resistance to vecuronium induced muscle relaxation in a patient with mental retardation receiving phenytoin for chronic anticonvulsant therapy]. 2134 54

A deletion 8p syndrome is a relatively uncommon congenital disease characterized by mental retardation associated with multiple malformation that make anesthetic management a challenge. Anesthetic management of a patient with deletion 8p syndrome may pose a serious problem mainly from difficult tracheal intubation, aspiration complication and cardiac malformation. We experienced a case of 10 year-old boy with a deletion 8p syndrome who underwent appendectomy under the general anesthesia. Intubation was performed by video glidescope after unsuccessful attempt with Macintosh laryngoscope. A high arched palate, short neck, poor patient cooperation due to mental retardation and occasional autistic behaviour made airway management difficult. This case should alert anesthesiologists to the greater difficulties of managing patients with deletion 8p syndrome.
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PMID:The general anesthesia experience of deletion 8p syndrome patient -A case report-. 2211 Aug 88

Williams syndrome is characterized by the triad of supravalvular aortic stenosis (SAS), mental retardation and elfin facies. Generally, difficult airway is expected in patients with Williams syndrome by characteristic face. A 26-year-old female with Williams syndrome was scheduled for abdominal myomectomy under general anesthesia. Difficult mask ventilation and tracheal intubation were anticipated because of micrognathia, mandibular retrusion, and a Mallanpati class III airway. Before induction of anesthesia the patient breathed 100% oxygen for 3 minutes. Anesthesia was induced and maintained with propofol, remifentanil and rocuronium bromide. Mask ventilation was easily performed. The direct laryngoscopic view was Cormack grade I and there was no difficulty in the tracheal intubation. After induction of anesthesia, anesthetic course was uneventful. According to the most previous clinical reports in patients with Williams syndrome in Japan, mask ventilation and tracheal intubation were performed easily contrary to preoperative airway assessment. In view of SAS, mental retardation, airway deformity and airway assessment in previous clinical reports, we should select the optimal strategy for airway management in patients with Williams syndrome.
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PMID:[Airway management in a patient with Williams syndrome]. 2211 59

Rett syndrome is a neurological disease that occurs only in females and it manifests with mental retardation, seizures, movement disorders, autistic behavior and abnormal breathing. A 19-year-old female with Rett syndrome underwent ophthalmologic surgery under general anesthesia at our institution. Airway control was difficult due to her limited mouth opening. We recommend that anesthesiologists should have proper knowledge about this disease and the patients to avoid the complications and problems that can be encountered during the perioperative period.
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PMID:Anesthetic management of an adult patient with Rett syndrome and limited mouth opening -A case report-. 2214 93

Crouzon's syndrome is an autosomal dominant disorder with complete penetrance and variable expressivity. Described by a French neurosurgeon in 1912, it is a rare genetic disorder. Crouzon's syndrome is caused by mutation in the fibroblast growth factor receptor 2 (FGFR2) gene. Normally, the sutures in the human skull fuse after the complete growth of the brain, but if any of these sutures close early then it may interfere with the growth of the brain. The disease is characterized by premature synostosis of coronal and sagittal sutures which begins in the first year of life. Case report of a 7 year old boy is presented with characteristic features of Crouzon's syndrome with mental retardation. The clinical, radiographic features along with the complete oral rehabilitation done under general anesthesia and preventive procedures done are described.
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PMID:Crouzon's syndrome: A review of literature and case report. 2221 36

Rett syndrome (RTT) is a congenital neurological disorder associated with mutations in the gene encoding MECP2 on the X chromosome. An 18-year-old woman (150 cm in height and 29 kg in weight) had been diagnosed with RTT and showed myotonic trismus, frequent attacks of apnea, mental retardation, spastic paraplegia, scoliosis, and microcephalus with micrognathia. She was scheduled to undergo laparoscopic fundoplication and gastrostomy under general anesthesia. Nasal bronchofiberscopic intubation (BFI) was planned because difficult airway due to trismus and micrognathia was expected. Referring to the bispectral index (BIS), anesthesia was induced with intermittent intravenous thiopental (total 125 mg), resulting in successful opening of the mouth by 1.5 of a finger width and establishment of manual ventilation. Following intravenous administration of rocuronium (20 mg), oral BFI was easily accomplished despite Cormack grade III. Anesthesia was satisfactorily maintained with inhalation of sevoflurane (1.0-1.5%) and continuous infusion of remifentanil (0.1-0.2 microg x kg(-1) x min(-1)) with the BIS value ranging from 30 to 50. She recovered smoothly from anesthesia using sugammadex (50 mg). However, she immediately demonstrated trismus and an attack of apnea with shivering, which were successfully resolved by warming the body and intravenous fentanyl (50 microg bolus and subsequent infusion at a rate of 10 microg x hr(-1)). The postoperative course was uneventful. Characteristically, RTT shows an extremely wide range of neurological symptoms. Therefore, it is of great importance to respond to each of those symptoms during the perioperative management of patients with RTT.
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PMID:[Anesthetic management of a patient with Rett syndrome associated with trismus and apnea attacks]. 2233 70

Fetal hydantoin syndrome is a rare disorder that is believed to be caused by exposure of a fetus to the anticonvulsant drug phenytoin. The classic features of fetal hydantoin syndrome include craniofacial anomalies, prenatal and postnatal growth deficiencies, underdeveloped nails of the fingers and toes, and mental retardation. Less frequently observed anomalies include cleft lip and palate, microcephaly, ocular defects, cardiovascular anomalies, hypospadias, umbilical and inguinal hernias, and significant developmental delays. Anaesthesia for incidental surgery in such a patient poses unique challenges for the anesthesiologist. We report the successful management of a 4-year-old male child with fetal hydantoin syndrome, cleft palate, spina bifida, atrial septal defect, and dextrocardia for tibialis anterior lengthening under subarachnoid block.
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PMID:Fetal hydantoin syndrome and its anaesthetic implications: a case report. 2308 54

Dental treatment for patients with physical or mental disability is often performed under general anesthesia due to level of cooperation with treatment, type and location of treatment, time required, or number of times patient is required to attend hospital. University hospitals are receiving an increasing number of requests from local private dentists and dental clinics to provide dental care for patients with physical or mental disability which can only be performed under general anesthesia. We carried out a retrospective survey of the routes of referral and types of dental treatment carried out in such patients under general anesthesia at Tokyo Dental College Suidobashi Hospital. The survey covered a 5-year, 9-month period from April 2006 to December 2011, during which 163 patients, comprising 106 men and 57 women, were treated. Their age ranged widely, from 2 to 53 years, with a high proportion (118 patients, 72.4%) being minors aged under 20. Among the total number of patients, 69 (42.3%) had disorders associated with mental retardation. One hundred and two patients (62.6%) had been referred from other medical institutions, with a particularly high number coming from public dental clinics for patients specializing in such patients. Conservative restorative procedures were performed in most cases (59.4%), with composite resin restorations being particularly frequent. After treatment, many patients returned to their referring medical institutions, which were responsible for subsequent management, but information on the posttreatment status was not available for some patients. Tokyo Dental College Suidobashi Hospital frequently collaborates with local medical institutions, and the present results suggest the importance of improving such collaboration.
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PMID:Dental treatment for patients with physical or mental disability under general anesthesia at Tokyo Dental College Suidobashi Hospital. 2331 23

Sanjad-Sakati syndrome (SSS) is a rare genetic disorder with autosomal recessive pattern of inheritance characterized by hypoparathyroidism, sever growth failure, mental retardation, susceptibility to chest infection, and dentofacial anomalies. A child with SSS was referred to the dental departmentseeking dental help for sever dental caries which was attributed to his dietary habits and quality of dental tissues. Full restorative rehabilitation was done under general anesthesia. Two years later, the child presented with recurrent caries affecting uncrowned teeth. High carries recurrence rate was blamed for the nutritional habits endorsed by the parents. Only steel crowned teeth survived such hostile oral environment which suggested shifting of treatment strategy towards full coverage restorations instead of classical cavity preparations and fillings during a second attempt for dental treatment under general anesthesia and for the dental treatment of two cousins of the same child. The author recommends effective health education for parents including the nature of their child's genetic disorder, nutritional needs, and dental health education to improve the life style of such children.
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PMID:Sanjad-sakati syndrome dental management: a case report. 2353 22

We experienced management of general anesthesia in a patients with Coffin-Siris syndrome (CS syndrome) which is an autosomal dominant disorder characterized by mental retardation, growth failure, hypoplasia of the fifth finger's distal phalanx and limb, and syndrome-specific facial appearance. Anesthesia was induced by sevoflurane by mask. After obtaining muscle relaxation by rocuronium, laryngoscopy by Machintosh #2 failed to reveal the vocal cord. However, the vocal cord was revealed by AirwayScope (AWS) for the pediatrics and then tracheal intubation was successful. Surgical procedures and anes-thetic management were performed uneventfully. This case demonstrates usefulness of AWS in pediatric patients with difficult intubation.
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PMID:[Case report : usefulness of the airwayscope for difficult intubations in a pediatric patients with Coffin-Siris syndrome]. 2377 34


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