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Biomedical subjects

J Bautista

Publications and source records attributed to J Bautista.

7 recordsLinked to original sources

Galactose metabolism in isolated perfused suckling-rat liver.

The metabolic conversion of 1, 2, or 4 mM galactose to glucose was studied in isolated livers of suckling rats. Whereas galactose uptake during perfusion with 1 and 2 mM galactose was linear throughout the 90-min experiment, uptake was delayed for 35 min when 4 mM galactose was perfused. Studies with radioactive galactose revealed a parallel disappearance of galactose and the appearance of [14C]glucose; about 80% of the galactose taken up was converted to glucose. Galactose perfusion appeared to reduce the basal amount of glucose derived from substrates other than galactose. The specific activities in the galactose-perfused livers of the three major galactose metabolizing enzymes, galactokinase, galactose-1-phosphate uridylyltransferase, and uridine diphosphogalactose-4-epimerase, revealed that the transferase was significantly lower, whereas that of galactokinase and epimerase were significantly higher than in livers perfused without galactose. No meaningful changes were observed in the levels of either phosphorylated or uridylated hexoses in these studies.

Animal Population Groups

Hypokalemic periodic paralysis in primary hyperaldosteronism. Subclinical myopathy with atrophy of the type 2A muscle fibers.

A case of a patient suffering from primary hyperaldosteronism is reported. In this case the disease is manifested clinically by periodic paralysis and hypopotasemia without permanent myopathy. The morphological study of the muscle demonstrates selective atrophy of the type 2A fibers as the most pronounced alteration. These findings suggest a chronic myopathic process.

Aged

Hereditary distal myopathy with onset in early infancy. Observation of a family.

The study of a family affected with hereditary distal myopathy with onset in early infancy is presented. Complete neurological examination was necessary in several members of the two last generations to discover the existence of the abnormalities of which they were unaware. The propositus was the most affected member of the family iwth distal paresis of the upper and lower extremities and selective paresis of the deltoid muscles. In addition he had kyphoscoliosis, talipes valgus and limitation of mobility of several joints. The onset of the disease was estimated as before the age of 2 when the child started walking. There was no progression of the disease. Clinical examination suggested a myopathic origin of the condition. A sural nerve biopsy was normal. Light-microscopy histochemical studies disclosed a predominance of type I fibres which were at the same time hypotrophic. Subsarcolemmal deposits of mitochondria were present although they were scanty and of normal ultrastructural appearance. In view of the morphological presentation it is postulated that this disease should be classified within the groups of myopathies accompanied by disproportion of fibres and selective atrophy of type I fibres.

Adult

Myotonia in centronuclear myopathy.

Centronuclear myopathy, which is unusual because of clinical myotonia, is described in two sisters. The diagnosis was established in adult life, but the first symptoms were noticed in infancy. The outstanding points of the clinical picture were mild amyotrophy, paresis, and clinical myotonia.

Adenosine Triphosphatases