Alterations of serum triiodothyronine levels during reduced and compensatory growth of steers.
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Biomedical subjects
Publications and source records attributed to H Bickel.
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Three patients with maple syrup urine disease were treated during the acute neonatal stage. Multiple exchange transfusions proved to be a satisfactory means of achieving rapid clinical and biochemical improvement during this phase. On the other hand, evidence is provided suggesting that in addition to exchange transfusions, a high calorie intake above 150 Cal/kg body weight/day is necessary to lower the plasma concentration of the branched chain amino acids to near-normal levels. As long as this calorie intake was not provided, further exchange transfusions failed to lower the plasma leucine concentration to below 17 mg/100 ml in one patient. It is assumed that this high calorie intake is necessary to prevent the breakdown of endogenous protein. Treatment of acute episodes in maple syrup urine disease should therefore not only eliminate the elevated alpha-keto acids and branched chain amino acids quickly (i.e. by multiple exchange transfusions or peritoneal dialysis), but in addition should provide a high calorie intake.
Forty-seven patients at the Hospital for Sick Children, London, who had phenylketonuria and were on a low-phenylalanine diet (21 early-treated--that is, treatment started before the age of 4 months--and 26 late-treated) were placed on a normal diet between the ages of 5 and 15 years. They showed significant falls in mean IQ of about six points after the diet was withdrawn. Twenty-two similar patients (five early-treated and 17 late-treated) at the Universitäts-Kinderklinik, Heidelberg, who were placed on a relaxed low-phenylalanine rather than a normal diet, showed smaller and non-significant falls in mean IQ. During the period of strict diet none of the patients in London or Heidelberg showed any consistent falls in IQ. These results suggest that complete withdrawal of the low-phenylalanine diet during childhood leads to a fall in intellectual progress in many patients.
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Corafusin, an infusion solution containing lidocaine, electrolytes and sorbitol, was tested during 4-day continuous infusion of patients with myocardial infarction. The serum sodium level remained constant within the normal range whereas initially reduced serum potassium and magnesium concentration increased to normal within 24 hours. Both sodium and water balance were generally equilibrated during the entire test period. Testing of lactate and non-esterified fatty acids revealed a significant decrease within the first 24 hours. On the basis of these findings it can be concluded that the infusion solution tested is suited for lidocaine infusion therapy of patients with myocardial infarction.
Blood acid-base status, serum electrolytes, and urine pH were examined in 64 infants and children with phenylketonuria (PKU) treated with three different low phenylalanine protein hydrolyzates (Aponti, Cymogran, AlbumaidXP) and two synthetic amino acid mixtures (Aminogran, PAM). The formulas caused significant differences in acid-base status, serum potassium, and chloride, and in urine pH. In acid-base balance studies in two patients with PKU, Aponti, PAM, and two modifications of PAM (P2 + P3) were given. We observed a change from mild alkalosis to increasing metabolic acidosis from Aponti (serum bicarbonate 25,8 mval/liter) to P3 (24,0Y, P2 (19, 3) and PAM (17,0). Urine pH decreased and renal net acid excretion increased. In the formulas PAM, P2 and P3 differences in renal net acid excretion correlated with differences in chloride and sulfur contents of the diets and of the urines. New modifications of AlbumaidXP and of PAM, prepared according to our recommendations, showed normal renal net acid excretion (1 mEq/kg/24 hr) in a balance study performed in one patient with PKU and normal acid base status in 20 further patients.
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If glucose, fructose, and xylitol are infused in the immediate post-operative period at rates necessary for parenteral nutrition, all three substrates produce specific undersired effects. These effects can be reduced significantly when the three substrates are infused in the form of a mixture. The composition and the rate of intake of such a mixed solution are of importance.
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The concentration of hydroxybutyrate and pyruvate during infusion of sorbitol was significantly depressed in 12 healthy subjects. The dosage ranged between 0.25 and 0.50 g/kg BW/h. Considering the blood parameter the hunger ketosis was depressed by the infusion rate of 0.25 g/kg/h. Cholesterol concentration, too, was significantly lowered by sorbitol.