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)

Rad-87 and RRa are new acoustic monitoring devices which can monitor the respiratory rate. To our knowledge, no studies have reported the RRa sensor used in pediatric patients after surgery. We succeeded in measuring the respiratory rate with the RRa sensor in the Pediatric Intensive Care Unit(PICU). A 10-year-old boy, 14.5 kg in weight and 119.6 cm in height, with cerebral palsy, mental retardation, epilepsy, and obstructive sleep apnea due to adenoidal and tonsillar hypertrophy, was scheduled for adenotonsillectomy under general anesthesia. Anesthesia was maintained with oxygen, air, sevoflurane (1.5-2.0%), remifentanil (0.1 to 0.5 microg . kg-1. min-1), and fentanyl (4 microg . kg-1). The operating time was 55 minutes, and the duration of anesthesia was 133 minutes. After finishing the surgery, we attached the RRa sensor to his anterior neck and monitored his respiratory rate. Furthermore, RRa could count his respiratory rate, during transfer from the operating room to PICU. The patient was sedated with dexmedetomidine (0.28 microg . kg-1 . min-1) at PICU, and his respiratory rate was accurately measured with the RRa sensor. We hope that Rad-87 and RRa sensors will become useful for measuring the respiratory rate in pediatric patients in the future.
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PMID:[Use of RRa sensor in a pediatric patient with post-adenotonsillectomy]. 2390 11

Adrenoleukodystrophy (ALD) is a genetic disorder with demyelination of the central nervous system and adrenal insufficiency. A 24-year-old man with ALD was scheduled for dental treatment under general anesthesia. He was diagnosted as having ALD at the age of 5. Past medical history included recurrent cervical cellulitis, adrenal insufficiency, mental retardation, muscle weakness and seizure disorder. General anesthesia was induced using betamethasone as a steroid cover, sevoflurane and nitrous oxide-oxygen and maintained with sevoflurane and nitrous oxide-oxygen. Nasal intubation was performed without using a muscle relaxant. Patients with ALD cannot metabolize very long chain fatty acid, so we did not use propofol containing long chain fatty acid. Operation and anesthesia were uneventful. There were no complications during and after anesthesia.
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PMID:[Anesthetic management of a patient with adrenoleukodystrophy]. 2455 40

A 12-year-old male patient with Coffin-Lowry syndrome was scheduled for posterior cervical decompression and fusion for cervical spinal injuries. The patient had features of Coffin-Lowry syndrome including mental retardation, prominent forehead, a short nose with a wide tip, a wide mouth with full lips, short stature, microcephaly, and kyphoscoliosis. We anticipated major troubles related to anesthesia such as difficult ventilation and intubation, communication difficulty during induction and extubation, and difficulty in using a naso-pharyngeal airway. In addition, we had to stabilize neck alignments during intubation because cervical vertebrae were unstable and spinal cord has already been injured. Therefore, we scheduled slow induction with sevoflurane maintaining spontaneous respiration. As we found the full mouth opening of the patient after the induction, we inserted an intubating laryngeal mask, through which ventilation was successfully maintained. A tracheal tube was inserted through the intubating laryngeal mask. When the surgery was completed, we extubated using a tube introducer in the trachea. As we found that the patient's airway was open, we removed the introducer. In conclusion, with a thorough planning of the anesthetic management, we successfully managed anesthesia for cervical spinal surgery in a patient with Coffin-Lowry syndrome.
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PMID:[Boy with Coffin-Lowry syndrome associated with spinal cord injuries]. 2460 Nov 20

Cohen syndrome is a very rare disease. Complication by spinal deformity has been reported, but management and surgery for spinal deformity in Cohen syndrome has not been previously described. The objective of this study was to examine the outcome of surgical treatment for kyphoscoliosis of Cohen syndrome with a literature review. The patient was a 14-year-old male with the characteristics of Cohen syndrome: truncal obesity, mental retardation, arachnodactyly, microcephalia, and a facial malformation. Scoliosis was conservatively treated with a brace at 13 years of age, but the spinal deformity rapidly progressed within a year. Plain radiographs before surgery showed scoliosis of 47 degrees (T5-T11) and 79 degrees (T11-L3), and kyphosis of 86 degrees (T7-L1). One-stage anteroposterior corrective fusion of T4-L3 was scheduled after 2-week Halo traction. Postoperative respiratory management was carefully performed because of Cohen syndrome-associated facial malformation, obesity, and reduced muscle tonus. Respiration was managed with intubation until the following day and no respiratory problems occurred. After surgery, thoracolumbar scoliosis was 28 degrees (correction rate: 65%). Kyphosis was markedly improved from 86 degrees to 20 degrees, achieving a favorable balance of the trunk. The outcome is favorable at 6.5 years after surgery. In conclusion, Cohen syndrome is often complicated by spinal deformity, particularly kyphosis, that is likely to progress even in adulthood. In our patient, spinal deformity progressed within a short period, even with brace treatment. Surgery should be required before progression to the severe spinal deformity with careful attention to general anesthesia.
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PMID:Surgical treatment for kyphoscoliosis in Cohen syndrome. 2464 Jan 85

We present a 9-year-old boy with X-linked cerebral adrenoleukodystrophy (X-linked ALD) and previous umbilical cord transplant who required general anaesthesia. An anaesthetic plan for each individual should be tailored to ensure the best possible anaesthetic care for these patients. The anaesthetic considerations include mental retardation, seizure disorder, hypotonia, liver function abnormalities, gastro-oesophageal reflux, impaired adrenocortical function and immunosuppression. Pre-operative sedation should be avoided because of hypotonia of the pharyngeal muscles. Anti-convulsants are continued, and potentially epileptogenic anaesthetic agents are avoided. The patient was intubated using a modified rapid sequence induction with a head up position of 30 degrees. Four other cases have been reported in literature. Nevertheless, there is still no established anaesthetic management for these patients, and total intravenous anaesthesia can be considered as a safe and alternative method of anaesthesia. To the best of our knowledge, this is the first reported use of total intravenous anaesthesia with propofol and remifentanil in a case of cerebral adrenoleukodystrophy, and with a favourable outcome.
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PMID:Anaesthesia for a child with adrenoleukodystrophy: A case report and review of the literature. 2470 Sep 3

We administered general anesthesia for a patient with 8 trisomy mosaic and cerebral palsy. Constitutional 8 trisomy mosaic has been associated with syndromic dysmorphology, corneal opacities, leukemia and trophoblastic disease. In Japan only 4 reports of general anesthesia related with 8 trisomy were found. This patient was a 24-year-old woman (140 cm, 35 kg), with mental retardation, poor body development and severe scoliosis. Since she suffered from repeated serious asthma and pneumonia since childhood, tracheotomy was performed at the age of 9. Epileptic seizures were also seen and antiepileptics were prescribed. This time, general anesthesia was scheduled for the extraction of a maxillary cyst. Anesthesia was induced slowly with sevoflurane from the tracheotomy, followed by rocuronium 25 mg i.v., and maintained with sevoflurane 1.5-2 % and remifentanil 0.05-0.2 microg x kg(-1) x min(-1) Throughout the operation, BIS score fluctuated between 40-60, and stable anesthesia was maintained. We reversed the rocuronium with sugammadex 140 mg promptly. The 8 trisomy mosaic patient is known to have various complications related to circulation and respiration. Careful management is necessary in anesthesia for an 8 trisomy patient.
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PMID:[Anesthetic management of a patient with 8 trisomy mosaic combined with cerebral palsy]. 2478 18

A 32-year old man with Sturge-Weber syndrome, a rare congenital disease of multiple angiomatous lesions including cervical cortex, face, oral soft tissues, larynx and trachea, underwent the excision of a back lipoma. His hemangioma which extended into the region of the right ophthalmic nerve and superior maxillary nerve is extremely large. He also had mental retardation and epilepsy. No apparent hemangioma was found in his oral cavity, pharynxes, larynx and trachea by preoperative exam. His hemangioma made it difficult to cover his mouth and nose by usual face mask. Though we first considered awake intubation, he was difficult to obey our command. So we searched for the face mask covering his nose and mouth without pressing his facial angioma. Finally, we discovered the full-face mask for NIPPV. After induction with propofol, we confirmed the perfect mask fit, and ventilation by two-person method was effective. Then we administered rocuronium and fentanyl, and intubated without difficulty. The patient was maintained by sevoflurane. He was hemodynamically stable. He was extubated without bleeding and respiratory problems. We experienced general anesthesia of a patient with Sturge-Weber syndrome who had a giant facial hemangioma. With full-face NIPPV mask we safely induced general anesthesia.
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PMID:[Anesthetic management of a patient with Sturge-Weber syndrome associated with a giant facial hemangioma]. 2497 67

We present a 17-year-old man who underwent brain magnetic resonance imaging and laboratory exams for uncontrolled seizure. Patients with an autistic disorder require deep sedation or, occasionally, general anesthesia even for radiologic exams or simple procedures. The anesthetic management of an obese, violent patient with a severe autistic disorder and mental retardation can be challenging to anesthesiologists and requires a more careful approach in selecting adequate anesthetics and doses. This case emphasizes the importance of having a detailed plan to ensure the smooth process of premedication, anesthetic induction, maintenance, emergence and safe discharge of incorporated patients in the event of unexpected situations. A 5% lidocaine patch to relieve the pain from the intramuscular injection and intravenous cannulation, intramuscular midazolam as premedication, and propofol for the maintenance of sedation can be a good sedation protocol in incorporated patients.
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PMID:Sedation using 5% lidocaine patches, midazolam and propofol in a combative, obese adolescent with severe autistic disorder undergoing brain magnetic resonance imaging: a case report. 2555 44

Panthothenate kinase-associated neurodegeneration (PKAN) (Hallervorden-Spatz disease) is a rare autosomal recessive chromosomal disorder characterised by progressive neuroaxonal dystrophy. The characteristic features include involuntary movements, rigidity, mental retardation, seizures, emaciation. The anaesthetic concerns include difficult airway, aspiration pneumonia, dehydration, and post-operative respiratory, and renal insufficiency. We report successful anaesthetic management of a 9-year-old intellectually disabled male child with PKAN, scheduled for ophthalmic surgery under general anaesthesia.
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PMID:Anaesthetic management of a child with panthothenate kinase-associated neurodegeneration. 2568 13

Job's syndrome also known as hyper-IgE syndrome is a rare disorder associated with immunodeficiency. It includes recurrent abscesses of staphylococcal origin affecting skin and musculoskeletal system, recurring pneumonia and pneumatoceles, eosinophilia, craniofacial, and bone growth changes. This case report describes the disease and anesthetic management. Ten-year-old male child weighing 18 kg was diagnosed recently as Job's syndrome and was posted for abscess drainage over chest, back, upper, and lower limb. He had associated severe mental retardation. The choice of anesthetic technique was based on multiple surgical sites and associated mental retardation. Due to associated mental retardation child was sedated under our vigilance with syrup midazolam 9 mg orally and EMLA cream applied with occlusive dressing 30 min prior to shifting to OT. Upon arrival to the OT, pulse oximetry, noninvasive blood pressure, Electrocardiogram, temperature probe, cardioscope were attached. Inhalation induction done with sevoflurane and IV line secured with 22G cannula. Glycopyrolate 0.1 mg + fentanyl 30 mcg + Propofol 30 mg given intravenously. General anesthesia was maintained with bag and mask ventilation with oxygen + nitrous oxide + sevoflurane with spontaneous ventilation. Postoperative period was uneventful. This case is presented in view of rarity of its occurrence and frequency of repeated surgical intervention and anesthesia. Associated mental retardation in this case could be an isolated incident or could be an occurrence related to underlying disease process itself.
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PMID:Anesthetic management in a child with Job's syndrome. 2588 24


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