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

Taking cognizance of the embryology and newer diagnostic techniques, the transpalatal route is strongly recommended as the optimal surgical procedure for the management of choanal atresia. It should be performed as soon as general anesthesia can be safely administered. In selected instances, but particularly when a patient is at increased surgical risk, necessitating surgical brevity, the endonasal puncture technique should be considered as the initial procedure. Parents must be appraised of the potential for subsequent growth deficiencies, mental retardation, and crossbite.
 ...
PMID:Choanal atresia. Clinical considerations for management. 651 91

Continued investigation in genetic biology has helped the medical profession to prevent complications in patients with this defect. Moreover, early diagnosis and treatment are recommended, in order to prevent mental retardation and visual deterioration. Refinement of techniques concerning preparation of the patient for general anesthesia and postoperative care merit further investigation. Simple measures undertaken in the two patients described made the completion of dental treatment possible without serious complications.
 ...
PMID:Comprehensive dental treatment under general anesthesia for patients with homocystinuria. 693 38

A probably new syndrome of craniosynostosis, ataxia, trigeminal anaesthesia, and parietal alopecia area associated with pons-vermis fusion anomaly (atresia of the fourth ventricle), in two unrelated Mexican girls, is described. The cerebellar anomaly was proven by CT scan only and it correlated with ataxia. Other abnormalities seen in both patients were midfacial hypoplasia, bilateral corneal opacities, low-set ears, mental retardation and short stature. This disorder could be a new neurocutaneous syndrome.
 ...
PMID:Craniosynostosis, ataxia, trigeminal anaesthesia and parietal alopecia with pons-vermis fusion anomaly (atresia of the fourth ventricle). Report of two cases. 713 29

In summary, their are a great many causes of mental retardation, each of which must be investigated carefully prior to induction of anesthesia. There are a few basic points to be remembered, no matter what the disease: (1) establish how much the patient understands and whether or not the patient can communicate, (2) establish whatever rapport is possible and use that both preoperatively and postoperatively in place of medication if possible, (3) avoid early extubation, and (4) be prepared for fairly long-term ventilation if the child cannot cooperate. The choice of anesthetic agents and preoperative and postoperative medications, their route of administration, and amount will be dictated by the disease entity, its severity, and the scope of the contemplated surgery.
 ...
PMID:Anesthesia for the mentally retarded patient. 720 92

The case of a 23 years old woman, affected by the Cohen syndrome, who underwent general anesthesia for extensive dental surgery, is reported. The Cohen syndrome is an autosomal recessive syndrome that causes mental retardation, obesity, short stature as well as oral, ocular, and limb anomalies. The problems the anesthesiologist could deal with include the capacity of the patient to cooperate; difficult intubation because of maxillary hypoplasia, micrognathia, narrow and high-arched palate, and prominent maxillary central incisors; generalized muscular hypotonia; moderate leukopenia, that could theoretically increase the risk of infection: and, finally, possible associated mitral valve prolapse or hiatus hernia. In the case reported the presence of mitral valve prolapse or hiatus hernia was ruled out echographically. The patient was premedicated with diazepam and atropine i.m.; general anesthesia was carried out by propofol-fentanyl association and myorelaxation was obtained with atracurium. Nasotracheal intubation was performed easily in spite of oral anomalies so that the usefulness of thyromental distance, which was 7 cm long, as a clinical test to evaluate a potentially difficult intubation was confirmed. Noteworthy, the thyromental distance was the only test which was suitable for the uncooperative patient. At the end of surgery muscular tone recovered promptly and the endotracheal tube could be regularly removed. No complication was registered postoperatively.
 ...
PMID:[General anesthesia in Cohen syndrome. Report of a clinical case]. 767 74

The anaesthetic management of a 5-month-old Chinese infant with Rubinstein-Taybi syndrome requiring bilateral inguinal hernia repairs is described. This is a rare congenital syndrome characterised by mental retardation, broad thumbs and first toes, craniofacial abnormalities, recurrent respiratory infections and, in one third of cases, congenital heart disease. General anaesthesia combined with caudal block was successfully used. The main complications encountered were minor difficulties with intubation and easily precipitated episodes of oxygen desaturation.
Anaesthesia 1995 Jan
PMID:Anaesthesia in an infant with Rubinstein-Taybi syndrome. 770 43

A 4-year-old female with Cockayne syndrome presented for cataract extraction under general anesthesia. She was thin and frail; her neck, epiglottis and larynx were stiff; she was deaf and blind; and she could not speak, sit unaided, or perspire. At the time of her admission, she weighed 5.5 kg. Cockayne syndrome is a disease of childhood characterized by mental retardation and premature aging. Although the underlying abnormality appears to be autosomal recessive inheritance or metabolic (possibly thymic) dysfunction, there is no consensus on etiology. The multiple organ involvement carries significant implications for the anesthetist. Intubation can be technically difficult, and care of the skin can be problematic. Essential hypertension, hepatic deficiencies, osteoporosis, deafness, blindness, and other effects of premature aging may be encountered making perioperative management a challenge.
 ...
PMID:Cockayne syndrome: a case report. 781 Feb 87

Watson's syndrome is an uncommon genetic disorder whose features include mental retardation and pulmonary valvular stenosis. The purpose of this report is to describe one management strategy used successfully to anaesthetize a woman with this disorder who presented for Caesarean section. Epidural anaesthesia using 0.5% bupivacaine without epinephrine in 2-3 ml boluses to a total dose of 15 ml was administered over 30 min. Invasive haemodynamic monitoring in the form of arterial and central venous catheters were used to guide therapy and help ensure maternal and fetal well-being. Intravenous ketamine in doses of 10-20 mg every five minutes to a total dose of 245 mg (4.5 mg.kg-1) was used to overcome the patient's uncooperative nature and facilitate invasive procedures. Postoperative analgesia was provided using 3 mg epidural morphine. The patient was observed in the intensive care unit for the first postoperative day and experienced an uncomplicated intra- and postoperative course. We conclude that this technique represents a safe and effective method for anaesthetizing patients with this complicated problem for Caesarean section.
 ...
PMID:Anaesthesia for caesarean section in a patient with Watson's syndrome. 782 60

Menkes disease is a rare, sex-linked recessive disorder characterized by kinky hair, convulsion, mental retardation, bone and connective tissue lesions, and hypothermia. These symptoms have been attributed to suppression of copper-dependent enzymes resulting from copper deficiency. We report a case of a 7-month-old infant with Menkes disease who underwent repair of inguinal hernia. Anesthesia was maintained with sevoflurane-N2O-O2, and the operation was carried out uneventfully. Although the patient had been medicated with anticonvulsants preoperatively, transient seizure occurred in the recovery room. We also discuss pathophysiology and anesthetic management of a patient with Menkes disease.
 ...
PMID:[Anesthetic management of an infant with Menkes disease]. 823 Jul 25

From 1985 to 1989, analysis of the umbilical cord blood gas in 546 cases of cesarean section delivery under spinal anesthesia was carried out to evaluate its relationship with neonatal asphyxia, and the following results were obtained. (1) The UApH of a normal newborn with an Apgar score > 7 was 7.28 +/- 0.04. Acidosis occurred in 33.3% of the infants born with an Apgar score < 7, but the incidence was only 3.9% in those with an Apgar score > 7. Sixteen% of the acidosis was metabolic and 84% belonged to the mixed type. The UApH and Apgar scores were most significantly related. (2) The abnormal UVpH was 4.9% (< M - 2SD). (3) There was no significant difference in UApH between cesarean section performed before and after the onset of labor. (4) The short term morbidity included: one death, 2 cases of convulsions, 2 cases of cerebral edema, and one case of periventricular leukomalacia. (5) The long term morbidity in the 29 cases referred to NICU included: 18 cases of normal growth, 2 cases of severe cerebral palsy, 3 cases of mild cerebral palsy, and 6 cases of mental retardation. (6) In view of the long term morbidity of the newborn, as seen in the correlation between the Apgar score and UApH, it was considered that, the critical points in neonatal asphyxia were Apgar score < or = 3 and UApH < 7.10. My results suggested that the umbilical cord blood gas analysis was helpful for the diagnosis of neonatal asphyxia, and the prognosis of the newborn.
 ...
PMID:[Evaluation of routine gas determinations in umbilical cord blood at cesarean section]. 842 48


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>