[Various cases of infantile hypotonia].
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Biomedical subjects
Publications and source records attributed to G Porro.
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Forty children with hypotonia and non progressive cerebral impairment were observed. In all cases muscle morpho-histometric and ultrastructural studies were performed, in 13 cases muscular acetylcholinesterase study was carried out. The Authors pointed out the high frequency (92% of cases) of muscle abnormalities: histochemical alteration of fibre type distribution (type 1 or type 2 fibres prevalence, type 2C persistence), diameter change (hypertrophy or hypotrophy of the fibres). In 37.5% of the cases, randomly distributed, were also present myofibrillar degeneration, Z band streaming, desalignment or marked destructuration of the sarcomeres. The muscular acetylcholinesterase study showed the same anomalous pattern of molecular forms (11 out of 13 cases), with increase of light (6S, 4S) and disappearance of heavy (16S) and medium forms (13S, 10S), without significant change of enzymatic activity. The possible alterated influence of CNS on muscle fibre differentiation and growth because of abnormal neural control is discussed. This hypothesis even if could be related with abnormal fibre typing and diameter, do not seem to explain the ultrastructural and biochemical abnormalities observed.
The effect of age on the steady state levels of phenobarbitone, alone and when given with phenytoin, sodium valproate and ethosuccimide has been measured in 1 361 children whose ages ranged from one month to 12 years. The relationship between dose and plasma level differed in those under 2 years from those aged between 2 years and 12 years. The addition of the other drugs altered the pharmacokinetics of phenobarbitone.
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