[Diagnostic problems in neoplasms of the cervical spinal cord in infants].
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Biomedical subjects
Publications and source records attributed to T Kmieć.
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Treatment of patients with fulminant liver failure is a challenge of contemporary medicine. Liver transplantation, in this group, is presently the only reasonable alternative, but in many patients the disastrous condition of the patient results in serious life-threatening complications, including neurological sequelae, which may influence the quality of life after transplantation, and in some cases even cause death. From 1990 to 2004, we performed 241 liver transplantations in children, including 20 transplanted due to fulminant liver failure (8.2%). Serious neurological complications followed liver transplantation in five cases (20%), three of which were fatal. The analysis revealed that the duration of pretransplant coma (grade III or IV) strongly correlated with the incidence of neurological complications (P < .05). Also a suboptimal quality of the donor liver and poor early graft function may contribute to these posttransplant complications.
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Out of over 100 children with the Lennox-Gastaut syndrome observed in the Outpatient Clinic and Department of Paediatric Neurology, Children's Health Center the authors present 6 cases in which the course of the disease was progressive and devastating. The main signs were astatic-myoclonic and generalized seizures, regression of psychomotor development, followed by mental regression, pareses of extremities, decortication symptoms and somatic cachexia. The authors suggest that the clinical course, the character of epileptic seizures very poor prognosis in certain children with the Lennox-Gastaut syndrome make the syndrome similar in its clinical aspects to subacute sclerosing panencephalitis and require careful differential diagnosis for excluding the latter disease.
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In the years 1988-1990, 100 children aged from 2 to 18 years were examined, who were hospitalized for bronchial asthma of moderately severe and severe clinical course. In all children the examinations were performed twice at intervals of several weeks or several months during administration of the full set of drugs, including euphylline. The results were estimated according to the number and character of electric discharges. Out of the total number of 100 children in 52 cases abnormal EEG record was found. Generalized paroxysmal discharges were found in 16 children mainly with severe and moderately severe course of asthma. Generalized changes were recorded in 12 children mainly with moderately severe course of the disease. Milder focal changes were found in 23 children. Most evident changes occurred in children with over five years of disease duration and with history of unconsciousness episodes. The studies carried out indicate a relatively frequent, that is in half the cases, damage to the central nervous system following repeated episodes of hypoxia.
West syndrome is a from of epileptic attacks of infants. Diagnosis of West syndrome includes: presence of the sudden violent flexion of the trunk and limbs, psychomotoric development retardation, especially after the onset of attacks, abnormal EEG records, and therapeutical problems. Clinical course and results of therapy were analysed in 66 children with West syndrome (39 boys and 27 girls). Children were divided into four groups, depending on etiology of the disease. Group 1 included 39 children with lesions to CNS during pregnancy; group 2-8 children with developmental CNS disorders, group 3-6 children with a history of encephalitis or meningitis, and group 4-13 children in whom etiology of West syndrome was unclear. Patients were treated with Synacthen-Depot in a daily dose of 0.03 mg/kg combined with other anti-epileptic agents. The most difficult to treat were those children in whom West syndrome occurred below 6 months of life, were psychomotor retarded before the onset of symptoms, hormonal treatment was introduced with delay, there were additional seizures of different etiology, and there were frequently recurrent infections.
Clinical course and results of therapy were analysed in the group of 92 children, aged between 3 and 9 years, with diagnosed Lennox-Gastaut syndrome. The obtained results of an analysis have shown that Lennox-Gastaut syndrome origin is not clear--causative factor can not be established in 1/3 of patients whereas in 1/2 of them abnormal course of pregnancy and perinatal period is noted. Together with seizures of various origin, other focal neurological symptoms, mental retardation and abnormalities in CT scans of the brain are frequently seen in patients with Lennox-Gastaut syndrome. Clinical course, prognosis and results of therapy are largely dependent on the degree of mental development before the onset of epileptic seizures, course of pregnancy and perinatal period, and the time of therapy. Children with Lennox-Gastaut syndrome require relative polytherapy in which valproic acid derivatives are predominating together with benzodiazepines, and temporary corticosteroids. An improvement was achieved in about 30% of the treated children. Prognosis in the remaining 70% of children is rather poor. Irregular administration of drugs, frequent changes of anti-epileptic agents, too low doses and abnormal environmental effects (abnormal parental attitudes) affect the results of therapy. An emphasis is on the poor prognosis in Lennox-Gastaut syndrome proceeded with West syndrome.