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Biomedical subjects

H K Berry

Publications and source records attributed to H K Berry.

At least 55 records · Page 3Linked to original sources
PubMed1977

Screening for metabolic disorders among high risk infants and children.

In a screening program in Cincinnati urine specimens from over 20,000 infants and children were tested for inherited metabolic disorders involving amino acids, carbohydrates, phenolic acids, organic acids, keto acids, mucopolysaccharides, and imidazoles. The subjects were selected on the basis of symptoms such as vomiting, diarrhea, acidosis, seizures, failure to thrive, delayed development, mental retardation, and others. The tests were based primarily on paper chromatographic techniques. Patients with 21 different metabolic disorders were found. The patterns of abnormal excretion of amino acids and other metabolites are often useful in making a diagnosis.

Amino Acid Metabolism, Inborn Errors↗
PubMed1976

Treatment of children with phenylketonuria using a phenylalanine-free protein hydrolysate (Albumaid XP).

Albumaid XP, a phenylalanine-free protein hydrolysate, was used for treatment of five phenylketonuric infants born between January, 1970, and September, 1972. The results were compared with those obtained from the five infants most recently treated with Lofenalac in our clinic prior to 1970. Treatment was begun by 2 months of age in all instances. Satisfactory physical growth and mental development were achieved using either Albumaid XP or Lofenalac, and there were no major differences in the outcomes with either treatment. The two protein sources may be used interchangeably for treatment of phenylketonuria.

Amino Acids↗
PubMed1976

The effects of Z plasty on hypertrophic scars.

Hypertrophic scar tissue has been observed to soften and thin following Z plasty without removal of any of the scar tissue. Histochemical studies of tissue before and after Z plasty showed that abnormally sulfated mucopolysaccharides were replaced by normal acid mucopolysaccahrides within 14 days. Collagen in hypertrophic scar appeared in nodules and fibers perpendicular to the surface of the skin rather than as long straight fibers parallel to the skin surface as normally seen. Elastic fibers were reduced and scar was relatively avascular. Following Z plasty the amount of collagen decreased and fibers were oriented in bundles at right angles to each other, parallel to the surface. When tissue was examined by electron microscopy collagen fibers showed marked variation in diameter. Microfibrils were increased and there were aggregates of fibrils with periodicity of 1500 to 1600 A. Following Z plasty, areas which previously contained only microfibrils now had unit collagen fibers with normal periodicity. Urinary excretions of hydroxyproline and hydroxylysine, amino acids found only in collagen, were increased following Z plasty, coinciding with the softening of the scar. Excretion of the disaccharide, glucosylgalactosylhydroxylysine, predominantly found in skin, increased following Z plasty, while excretion of the monosaccharide, galactosyl-hydroxylysine, predominant in bone collagen, remained constant. Amino acid composition of collagen, remained constant. Amino acid composition of collagen isolated from hypertrophic scars was similar to that present in skin and tendon, and unlike that in cartilage or infant dermis. Hydroxyproline content was slightly decreased and hydroxylysine content slightly increased compared to skin or tendon. Histochemical, ultrastructural and biochemical studies of the same specimens suggested that collagen with disordered fibril formation was present in the hypertrophic scar and was degraded following Z plasty. Changes in the molecule which occurred after synthesis of the protein core might be responsible both for the failure of normal maturation of the scar and for its sensitivity to degradative enzymes following Z plasty.

Adolescent↗
PubMed1975

Dietary supplement and nutrition in children with cystic fibrosis.

Assessment of nutritional status of patients with cystic fibrosis of the pancreas (CFP) showed that poor growth was associated with low concentrations of albumin, urea nitrogen, and cholesterol in serum and with elevated white blood cell (WBC) counts. Patients with CFP maintained weight approximately 1 standard deviation below the mean until 8 years, after which there was a progressive decline in growth rate compared to normal. A complete dietary supplement consisting of a beef serum hydrolysate, a glucose polymer, and medium-chain triglycerides was given to 15 patients for a year. Patients who received the diet showed significant gains in weight, significant increase in clinical score, significant increase in serum albumin level, and a significant drop in the WBC count compared to control patients who did not receive the supplement.

Adolescent↗
PubMed1975

Biochemical effects of induced phenylketonuria in rats.

Phenylketonuria (PKU) was induced in rats by the combined feeding of 3 per cent excess phenylalanine and 0.12 per cent of p-chlorophenylalanine, an inhibitor of phenylalanine and tryptophan hydroxylases. Increased concentrations of phenylalanine and increased ratio of phenylalanine to tyrosine were demonstrated in blood from pregnant rats fed the experimental PKU diet from day 10 to 20 of pregnancy, in fetal blood and amniotic fluid of fetal animals from mothers fed the PKU diet, and in blood of rats fed the PKU diet for 28-30 days beginning at 20-21 days of age. Both phenylpyruvic acid and orthohydroxyphenylacetic acid were excreted by rats fed the PKU diet, but neither were detected in urine in animals fed either excess phenylalanine or excess inhibitor alone. Reduced serotonin concentrations were found in brains of rats fed p-chlorophenylalanine, either alone or in combination with excess phenylalanine in the PKU diet. These biochemical changes in rats with induced PKU and the behavioral changes described earlier are similar to those of the human condition. The animal model should prove useful in searching for the mechanism of the disease.

Amino Acids↗
PubMed1975

Serum amino acid concentrations during pregnancy of women heterozygous for phenylketonuria.

Increased concentrations of phenylalanine were found in blood specimens from pregnant women who were heterozygous for phenylketonuria, but the concentration differences between normal controls, nonpregnant heterozygous, and pregnant heterozygous, and pregnant heterozygous women were small. Phenylalanine concentrations in cord blood were in the same range as in maternal blood at time of delivery. Phenylalanie concentrations in the carrier do not rise sufficiently during pregnancy to harm the infant in utero.

Amino Acids↗