Pediatric research
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Twelve heartworm-free mongrel dogs (10 males, 2 females) mean weight 22.2 kg (range 17.7--28.2 kg) were sedated and placed in a supine position with the neck extended. A double tracheotomy was performed under sterile conditions. The first tracheotomy tube was inserted 2 cm above the sternum in the direction of the carina using a shortened Silastic American tracheotomy tube (id 10 mm); the second was inserted two cartilage rings proximal to the first and approximately 5 cm distal to the larynx. ⋯ Results indicate no statistically significant change in resistance from control for ultrasonic mist or warm moist treatment regimens. Warm dry, cold dry, and cold moist treatments, however, all produced significant reductions in translaryngeal resistance (74%, 60%, and 54%, respectively), there being no difference between cold dry and cold moist and warm dry and cold dry. Significant differences between warm dry and cold moist treatments were obtained, the former decreasing translaryngeal resistance the greatest amount...
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A male child died at 7 months of age with progressive neurologic deterioration and persistent metabolic acidosis. Investigations during life showed this child to have elevated blood pyruvate, lactate, and alpha-ketoglutarate as well as elevation of branched chain amino acids and occasional hypoglycemia. Cofactor therapy using either thiamine-HCl (2 g/kg/24 hr) or thiamine tetrahydrofurfuryl disulfide had no measurable effect on the clinical or biochemical status of the patient. ⋯ Examination of the individual activities of pyruvate dehydrogenase complex showed pyruvate decarboxylase (E1) to be normal in liver and other tissues. Dihydrolipoyl dehydrogenase (E3), on the other hand, was deficient in all tissues tested. alpha-Ketoglutarate dehydrogenase complex, which depends of E3 for its total activity, was also deficient in all tissues tested. The absence of this enzyme id discussed in relation to the clinical and biochemical status of the patient.
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Comparative Study
The rate of cerebral utilization of glucose, ketone bodies, and oxygen: a comparative in vivo study of infant and adult rats.
Cerebral blood flow (CBF) was measured by means of Celabeled microspheres in infant (20-day-old) and adult (3-month-old) rats, anesthetised with Na-5-ethyl-5-(1-methylpropyl)2-thiobarbituric acid. Cerebral arteriovenous differences of acetoacetate, D-beta-hydroxybutyrate, glucose, lactate, and oxygen and brain DNA content were determined in other groups of similarly treated infant and adult animals fed or starved for 48 or 72 hr. The mean CBF values of 0.48+/-0.04 and 0.62+/-0.07 ml/(g X min), +/- SEM, in infant and adult animals, respectively, were not significantly different. ⋯ Calculated cerebral metabolic rate for oxygen (CMRO2), assuming complete oxidation of glucose and ketone bodies and expressed as micromoles per (mg DNA X min), was similar in fed and starved rats of both age groups (Table 3), indicating that ketone bodies serve as an alternative substrate for glucose during starvation. Calculated CMRO2 for glucose plus ketone bodies was similar to the measured CMRO2 in adult rats both in the fed and the starved groups. For infant rats, calculated CMRO2 for glucose plus ketone bodies was higher than measured CMRO2, indicating that in this age group a portion of substrate was used for synthesis or storage rather than for complete oxidation.
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Enzyme replacement therapy was studied in a chimeric mannosidosis calf which had received a natural transplacental transplant of normal lymphocytes from its co-twin. There was considerable reduction in the pathology of certain organs and in the amount of storage oligosaccharides, but the clinical course of this neurologic disease was not significantly altered. It was postulated that if this disease had been purely visceral the transplant would have been relatively effective.