Drugs: actions and reactions. Debriding enzymes.
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Biomedical subjects
Publications and source records attributed to C Young.
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A study was undertaken in twenty-eight patients to compare the effects of the supplementation of nitrous oxide and oxygen anaesthesia with either 0.5--1.0% halothane or 50 microgram fentanyl/kg on the metabolic and hormonal response to gynaecological surgery. The results showed that the increases in blood glucose, plasma cortisol and growth hormone concentrations observed in the halothane group of patients were abolished in those who received fentanyl. It is postulated that the ability of large doses of fentanyl to inhibit the cortisol and growth hormone response to surgery may be of value in ameliorating the catabolic response to trauma.
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Hemimegalencephaly is a rare congenital anomaly of the brain characterized by unilateral hypertrophy, usually with an abnormal gyri pattern and derangement of the cortical architecture. We report 2 patients with hemimegalencephaly who presented with early-onset seizures, hemiparesis and developmental delay. An electroencephalogram showed hemispheric continuous spikes and spike-and-waves in one patient and repetitive spike-and-waves in the other. Magnetic resonance imaging showed left hemimegalencephaly in both cases and in case 1 pachygyria and heterotopia. A functional hemispherectomy was done on case 1 at the age of 33 months and on case 2 at 7 months due to difficulty in seizure control with antiepileptic drugs. The frequency of seizure dramatically decreased and there was an obvious improvement in neurologic development after surgical intervention. Pathology revealed disorganized lamination of the cortical layers with increased neuron size and bizarre-shaped neurons in both cases. Heterotopia of neurons and glia in the subarachnoid space was noted in case 1. Hemispherectomy should be performed as soon as possible when medical treatment fails to control seizures.
Four female children with clinically-definite diagnoses of multiple sclerosis (MS) were studied with multimodal evoked potentials (EPs), electroencephalogram (EEG), cerebrospinal fluid (CSF) analysis, computed tomography (CT) and magnetic resonance imaging (MRI). Correlations among the clinical features, neuroimaging and neurophysiological studies were also evaluated. Their ages of the onset ranged from 5 to 11 years. The clinical evolution in the all four MS cases was relapsing-remitting form. MRI studies in three cases showed abnormal demyelinating plaques in the brainstem, cerebellum and white matter of the cerebrum. CT examination, performed in the other MS case, did not reveal hypodensic lesion. Despite that MRI failed to document optical nerve lesions, three cases had abnormal visual evoked potentials (VEPs). Somatosensory evoked potentials (SSEPs) were abnormal in three cases. Brainstem auditory evoked potentials (BAEPs) were abnormal in two cases presenting with signs of brainstem dysfunction. The conclusion was that paraclinical evidences obtained from MRI and multimodal evoked potential studies are of value as an aid in localizing involved areas and detecting silent lesions in children with MS.
Classification and electroencephalographic studies were based on 215 children with chronic headache ranged in age from 3 to 16 years (9.53 +/- 2.86 years old) who were referred to the Pediatric Neurology Clinics of National Taiwan University Hospital due to chronic or severe headache from December 1989 to December 1992. The most common type of headache was seizure headache which constituted 27.0% of all these cases. Migraine headache accounted for 22.3%. The abnormal and borderline electroencephalogram (EEG) rate in seizure headache patients were 75.9% and 24.1% respectively. Comparing the results of migraine patients were 8.3% and 8.3% respectively. Seven cases have been diagnosed as orthostatic dysregulation clinically. All of them presented normal EEG. The response of seizure headache to anticonvulsants was good in the patients with abnormal (87.9%) and borderline EEG (55.6%). We conclude that seizure headache is not uncommon in children and it responses good to anticonvulsants.
Seven patients were endoscopically diagnosed as having a fungal esophagitis with mycologic or histologic support for the diagnosis from 1979 to 1991 in the Department of Pediatrics, National Taiwan University Hospital. The major causative agent was Candida albicans. Other fungi isolated were Candida Krusei, Trichosporon cutaneum, Trichosporon beigelii, and Rhodotorula rubra, but they all resembled one another under endoscopic examination. The most common presenting symptom was hematemesis, and the lower part of the esophagus was more often involved. Only one patient was documented to have oral thrush. Most of the children did not present typical symptoms of esophagitis such as dysphagia or odynophagia, and they tended to be in more advanced stages of the disease when the diagnosis was made.
From May 1984 to April 1995, a total of 16 patients (12 females, 4 males) with cerebral venous thrombosis, diagnosed by computed tomography (CT), conventional cerebral angiography, magnetic resonance imaging (MRI) or magnetic resonance angiography (MRA), were reviewed retrospectively. The age ranged from 1 month to 16 years of age (average: 2.5 years) with 8 below 1 year of age. The presenting symptoms for infants were mental change (75%) and seizure (100%), mainly generalized (63%) in character. Associated illness was mainly closed head injury, diarrhea or dehydration. All infants had mild to severe motor handicap in a 1 to 10 year follow-up. In contrast, older children frequently presented with headache (37%) or consciousness change (50%), and were more frequently associated with sepsis or local infections. Four (50%) of them recovered completely, but two died and two were finally in a vegetative state. For the four patients with poor prognosis, all had severe initial insults and widespread sinus thrombosis. MRI and MRA are better than CT for the diagnosis of cerebral venous thrombosis. It was concluded that prognosis for venous thrombosis cases in infants is worse than in older children, but this also depends on the severity of initial insults. For infants who present with intractable seizures, cerebral venous thrombosis should be taken into account when the seizures are difficult to control.
Nine patients below 20 years of age (4 males and 5 females), who were diagnosed to have acute disseminated encephalomyelitis (ADEM) by clinical findings and magnetic resonance imaging (MRI), were reviewed retrospectively. They ranged from 4 months to 20 years of age with an average of 8.6 years. Seven patients (78%) received neurophysiological studies, which included electroencephalography, multimodality evoked potentials (EPs), nerve conduction velocity and/or F-wave measurement. The presentation symptoms were mainly headache, vomiting, consciousness change and motor deficits. Seven (78%) of nine patients had symptoms preceded by fever or upper respiratory tract infections; one (11%) was preceded by trivalent mumps, measles, rubella vaccination and no definite predisposing factor was found in another. Computed tomography (CT) scans were abnormal in five (71 %) of seven children, while MRI showed multiple lesions in seven (78%) of nine children. The lesions in MRI were mainly in the brainstem (n = 6), basal ganglion (n = 5), thalamus (n = 4), periventricular white matter (n = 4) and cerebellum (n = 4). EPs disclosed spinal cord involvement in all patients who received the examination. Peripheral neuropathy was disclosed in one patient. It was concluded that associated radiculoneuropathy is possible in patients with ADEM. Both MRI and neurophysiologic studies are complementary for diagnosis of ADEM.
A 4-year-10-month-old boy with late infantile type neuronal ceroid lipofuscinosis was reported. He presented with progressive dementia, loss of visual acuity, gradual regression of speech and motor functions, and myoclonic jerks. A hyperactive deep tendon reflex was noted, but there was neither muscle weakness nor hepatomegaly. Serum lactate, pyruvate and ammonia levels were within normal limits. The funduscopic examination showed diffuse mottling of the retinal pigmented epithelium. The electroencephalogram showed irregular bilateral spike-and-waves or polyspike-and-waves and isolated focal spikes from the bilateral parieto-occipital regions. The wave forms of visual evoked potentials were flat. The electroretinogram was unrecordable. The somatosensory evoked potentials showed prolonged central conduction times from bilateral median nerves. The brainstem auditory evoked potentials were within normal limits. Diffuse cerebral and cerebellar atrophy were noted on magnetic resonance imaging. The diagnosis was confirmed by the electron-dense cytoplasmic inclusion bodies within the conjunctival squamous epithelial cells. No specific treatment was available. Regular anticonvulsants were not given during follow-up because seizures attacked him only on occasion. His visual acuity was progressively impaired. In addition to nearly total absence of speech, an inability to communicate and walk independently was also noted. The clinical features according to the subtypes, radiology, pathology, managements and prenatal diagnosis for this case are discussed.
Since 1987, we have diagnosed 10 patients, 4 males and 6 females, aged 2-11 years at the last evaluation, who all met the following criteria of severe myoclonic epilepsy in infancy (SMEI): generalized or unilateral long-lasting febrile clonic seizures in the first year of life; the subsequent appearance of myoclonic seizures and other types of seizure (partial seizures, atypical absences and convulsive status epilepticus); and neuropsychological deterioration for a certain period. Family histories of epilepsy and febrile seizures could be traced in 1 and 3 cases, respectively. None of them had previous personal history of brain insult. Electroencephalographic (EEGic) recordings in febrile seizure stage were normal; and continuous prophylaxis with phenobarbital failed to prevent the recurrence of febrile seizures. EEG studies in myoclonic stage showed generalized spike-and-waves, polyspike-and-waves, focal abnormalities and/or photosensitivity. The seizures were highly resistant to antiepileptic drugs. Our experiences suggested that comedication of valproic acid, clonazepam and carbamazepine may be most effective in treatment of the diverse seizures including myoclonic seizures, myoclonic-tonic-clonic seizures, atypical absences and partial seizures. Myoclonic seizures and atypical absences diminished in parallel to a clear-cut decrease in generalized abnormalities on EEG in 4 cases aged more than 7 years. However, the partial seizures, secondarily generalized seizures and status epilepticus were still present. Further investigations should aim to identify the underlying etiology and to search more effective treatment.
The purpose of this study was to assess the osseous response to implanted particles of an anorganic xenograft bone mineral. Bio-Oss, in six patients with severely resorbed alveolar ridges. The observation period varied between 9 and 44 months; in five of six patients, long-term bone regeneration around the implants was observed. Histologic examination of biopsy material obtained from the graft area showed Bio-Oss particles still present in all patients after the varying observation periods. This raises the question whether Bio-Oss may be regarded as a resorbable material, as previously reported.