Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0036572 (seizures)
 80,221 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A review has been conducted of ongoing clinical and laboratory studies of hypothermic circulatory arrest (HCA) and low-flow cardiopulmonary bypass (LFB) at a children's hospital in Boston. A prospective randomized clinical trial of HCA versus LFB has shown a higher incidence of perioperative seizures in patients randomized to HCA. At 1 year of age, neurologic and developmental studies have shown an association between seizures and worse outcome. Longer duration of HCA is associated with a worse score on the Bayley scale assessment of gross and fine motor function in particular, as well as a higher probability of neurologic abnormality. A retrospective review of development after HCA for Senning procedure has shown a correlation between more alkaline pH (alpha-stat strategy) during cooling before HCA and lower developmental score relative to a more acidotic strategy (pH stat). The institutional change to alpha-stat was accompanied by several cases of choreoathetosis after HCA. Currently, patients are being randomized between alpha-stat and pH-stat. Laboratory studies have used a piglet model with assessment of cerebral blood flow and metabolism as well as high-energy phosphates and cerebral pH determined by magnetic resonance spectroscopy. High-energy phosphates are maintained by a flow rate of 50 mL/kg/min but are undetectable after approximately 35 minutes of HCS. A pH-stat is associated with more rapid recovery of high-energy phosphates after HCA than alpha-stat. Recent studies have examined the role of nitric oxide in the causation of brain injury after HCA as well as the potential utility of cerebroplegia in increasing the safe duration of circulatory arrest.
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PMID:Hypothermia, circulatory arrest, and the pediatric brain. 863 89

Metabolic diseases of the nervous system vary considerably in their clinical and pathological aspects. In neurological presentations of these disorders dominate mental retardation and epileptic syndrome. We have studied 27 patients of age from 3 months to 3 years: PKU -- 15 cases; homocystinuria -- 4; hyper-prolinemia -- 1; methylmalonic acidemia -- 5 and combined disorders -- 2. Epileptic syndrome was revealed in 21 patients, mental retardation in 1, spasticity in 5 and ataxia in 1 patient. Epileptic syndrome was presented with generalized seizures (grand mal -- 6 cases, myoclonic absences -- 13 cases) and partial seizures (simple motor -- 2 cases). Investigations did not found reliable correlations between certain forms of enzymophaties and EEG patterns. Patients were treated by pathogenic (dietary management with protein-modified diet and vitamin therapy) and symptomatic (anticonvulsants) treatment. We have achieved the positive therapeutic effect by pathogenic and anticonvulsive treatment in 11 patients. All these patients were from the first group (1-3 year). The best outcome was observed in the cases of the early diagnosed PKU. The most severe mental retardation and resistant epilepsy were revealed in patients with combined disorders of metabolism and vitamin-non-responsive forms of MMA and HCS.
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PMID:[Peculiarities of epileptic syndrome in children with metabolic disorders of nervous system]. 1636 68