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)

Sanjad-Sakati syndrome (SSS) is a rare genetic disorder characterized by congenital hypoparathyroidism, hypocalcemia and hyperphosphatemia, seizures, severe intrauterine and postnatal growth failure, dwarfism, mental retardation, dysmorphic features including retromicrognathia and abnormal dentition and increased susceptibility to infection. It is mainly confined to children in the Middle-East countries. We report the anesthesia management of a 12-year-old boy with SSS for dental treatment, and discuss the anesthesia implications of this disorder.
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PMID:Anesthesia management for the child with Sanjad-Sakati syndrome. 1704 Mar 10

We report successful anesthetic management of a morbidly obese patient with mental retardation employing inhalation induction with sevoflurane in sitting position and epidural catheterization using ultrasound sonography. Inhalation induction with sevoflurane keeps spontaneous respiration and induction in sitting position may provide a greater margin of safety for airway control. Therefore, this method of induction is useful for morbidly obese patient. Regional anesthesia in an obese patient can be technically challenging because of difficulties in identifying the useful body landmarks. We successfully used ultrasound sonography to identify spinous process and could insert an epidural catheter at the right place.
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PMID:[Anesthetic management of a morbidly obese patient with mental retardation]. 1705 80

Opioids are used in clinical practice for sedation, anesthesia, and analgesia. Their effects depend on their pharmacokinetic and pharmacodynamic characteristics. The liver is the major site for the biotransformation of most opioids. The major metabolic pathway is oxidation. Metabolism influences distribution, clearance, onset, and offset of opioid drugs. Action also depends on the coupling of opioids with the class of receptors involved and on localization of specific receptors. Three major types of opioid receptors, designated as mu, delta, and kappa, present in the central nervous system, are coupled to G proteins and inhibit adenylyl cyclase. Down's syndrome is a congenital condition characterized by mental retardation and particular physical features. Neurotransmission alterations are important. Alteration in the concentration of opioids in the cortex of these patients has been demonstrated. Neurobiological abnormalities and, in some, abnormalities in the neurotransmission systems, anxiety, and, in particular, nociception all suggest that structural and functional alterations of opioid receptors may be present. A clear knowledge of these multiple abnormalities is essential for skillful management of the perioperative period and for a good outcome for patients with Down's syndrome.
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PMID:Opioids and Down's syndrome. 1731 25

During preoperative evaluation for anesthesia in the Down patient, it is important to focus attention on the functional conditions of the patient and systems that frequently show anomalies. One of the challenges of evaluating pre-operative conditions and potential risks in the Down patient is the lack of a gold-standard evaluation score; cervical spine abnormalities, reduced dimensions and malformations of the airways, neurological changes, respiratory and cardiac disease, as well as endocrinological and metabolic alterations. We suggest, as a possible method of evaluation for patients with mental retardation and possible malformations, a new scale which takes the functional and mental conditions into account: the Sensorial, Psychological, Anatomical, Biological, Operational and Surgical (SPABOS) Compliance Score.
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PMID:Preanesthetic evaluation and assessment of children with Down's syndrome. 1733 15

In 1980s, the authors experienced 5 patients with "a peculiar form of acute encephalitis/encephalopathy "which is characterized by three features: 1. Complex partial seizures with secondary generalization recur incessantly or continue persistently without regaining consciousness for many days in spite of intensive diazepam (DZP) therapy; usually general anesthesia at ICU setting for 2-3 weeks becomes mandatory. 2. After weaning from long-run anesthesia, seizures of the same type still persist, though much less frequent, during the convalescent and chronic phases of the disease throughout. There is no seizure-free interval between the acute and chronic stages. 3. Etiology is totally unknown;extensive laboratory examinations mostly remain within normal ranges, though clinical features such as acute onset, frequent accompaniment of fever, etc., mimic those of acute encephalitis/encephalopathy. The synopsis of our 5 patients were; age ranged from 2 to 5 years old, no antecedent history of seizures, fever of moderate degree preceded seizures a few days and persisted in the acute stage; complex partial seizures with secondary generalization recurred several times every hour. Circulatory/respiratory compromise necessitated a drastic intervention with barbiturate coma at ICU. Etiology was unknown. Follow-up for 19 years in average revealed mental retardation and chronic epilepsies in all patients. Since our first report in 1987, 49 similar cases with ours have been sporadically reported. The majority of the reported cases had been submitted to longstanding general anesthesia and placed in barbiturate coma for weeks to 2 months. The death occurred in 7 patients. In all reported cases, the first line drugs such as DZP iv, phenytoin iv, etc did not work, and it was highly recommended to place the patient under barbiturate coma as early as possible.
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PMID:[Acute non-herpetic encephalitis with severe refractory status epilepticus --its overwhelming ictogenicity, epileptogenicity, long-term prognosis and review of the literature]. 1737 Jun 36

A 28-month-old boy (height, 76 cm; weight, 9.4 kg) diagnosed as having Williams syndrome presented for dental care. We report a case of postoperatively suspected malignant hyperthermia after the administration of general anesthesia for dental treatment in this patient with severe supravalvular aortic stenosis and pulmonary artery hypoplasia. Anesthesia was maintained through the inhalation of nitrous oxide and sevoflurane with oxygen. The patient was hemodynamically stable and no other abnormalities were observed. After the completion of the dental treatment, he was transferred to the pediatric ward. On arrival at the ward, the patient's core temperature increased to 39.5 degrees C and tachypnea (RR, 30 breaths/min) was observed. The SPO2 during inhalation was slightly low (92%-93%). Serum biochemistry revealed an elevated CK level (1345 U/L) but no other abnormal findings. Twelve hours after the dental treatment, the patient's core temperature fell to 37.4 degrees C. After hospitalization for 4 days, the patient was discharged in good condition. In the present case, general anesthesia was employed for dental treatment despite severe supravalvular aortic stenosis and peripheral pulmonary artery hypoplasia, because conventional dental therapy was very difficult as a result of the patient's mental retardation and hyperkinesia. The present case suggests that the use of volatile agents that could trigger malignant hyperthermia should be avoided wherever possible.
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PMID:General anesthesia for dental treatment in a Williams syndrome patient with severe aortic and pulmonary valve stenosis: suspected episode of postoperatively malignant hyperthermia. 1768 62

We retrospectively reviewed 33 patients (35 cases) who underwent foreign body removal at our institution from 1995 through 2003. Male-female ratio was 21 : 12 and the most frequent age was one year. The most common foreign bodies (FBs) were nuts (n = 14) and plastics (n = 7). A repeater (3 cases) had mental retardation. Patients were referred to our institution with an average interval of 90 hours, and after 1.3 hospitals. All the patients were managed with general anesthesia. Direct laryngoscopy was performed to extract FBs in 11 cases suspected of having pharyngeal or laryngeal FBs. In 24 cases suspected of having tracheobronchial FBs, the trachea was intubated and a flexible fiberoptic bronchoscopy was performed to locate the FBs. FBs were found in the trachea in 2 cases and in the bronchus in 18 patients and were successfully extracted by rigid bronchoscopy in 10 cases. All the patients were admitted for fear of laryngotracheal edema. There were no significant postoperative complications.
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PMID:[Perioperative management of airway foreign bodies in 35 pediatric patients]. 1787 48

Coffin-Siris syndrome is a rare genetic disease characterized by coarse facial features, sparse scalp hair, hirsutism, hypoplasia of the distal phalanges, hypoplastic nail in the fifth digit, and mental retardation and delayed growth evident in both weight and height. Most cases are sporadic, but the possibility of recessive or dominant autosomal inheritance has been suggested. Facial abnormalities that make intubation difficult and mental retardation that interferes with cooperation are aspects of this disease that can affect the choice of type of anesthesia. We report the case of a parturient with Coffin-Siris syndrome who refused epidural analgesia for labor pain and for whom the obstetrician later decided that an emergency cesarean was necessary due to fetal distress.
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PMID:[Emergency cesarean in a patient with Coffin-Siris syndrome]. 1808 10

The management of paediatric dentistry treatment is essentially based on behaviour management but some behaviour troubles or mental retardation may hinder this kind of treatment at the dental office without any premedication. This often leads the dentist to change his treatment planning even if this may compromise the quality of treatment . Conscious sedation techniques enable stress and pain control during the active treatment phase and represent a useful alternative to general anaesthesia which cannot be used on a routine based level. Conscious sedation by the inhalation of nitrous oxide and oxygen (MEOPA) represents a good choice, as well as by its harmlessness as by its fast reversibility. MEOPA is a precious help in our practice, provided that its administration is totally under central and all contra-indication are respected. However sedation by inhalation should in no case be systematized and its goal must remain the progressive rehabilitation of the patient in a circuit of traditional ambulatory care.
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PMID:[Sedation with 50 % nitrous oxide/oxygen in paediatric dentistry]. 1894 73

Uncontrolled head motion during CT/MRI cerebral blood flow (CBF) imaging has been estimated between 3 and 15% of the cases. We present a pharmacological approach which permitted us to maintain the incident at 0.06% with few side effects. The protocol involves the systematic use of general anesthesia (sevoflurane) in children below five years and those with mental retardation. In anxious, claustrophobic or agitated adults, mild sedation with propofol, midazolam or hydroxyzine was used with mild effects on CBF. We strongly recommend the availability of basic cardiorespiratory resuscitation equipment and a recovery room before any sedation or general anesthesia is undertaken.
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PMID:Pharmacological control of head motion during cerebral blood flow imaging with CT or MRI. 1906 94


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