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Biomedical subjects

I Krieger

Publications and source records attributed to I Krieger.

At least 37 records · Page 2Linked to original sources
PubMed1980

Acrodermatitis enteropathica without hypozincemia: therapeutic effect of a pancreatic enzyme preparation due to a zinc-binding ligand.

The clinical course and intestinal absorption studies of a female infant who developed diarrhea after cessation of breast feeding, mood changes, and intermittently had mild perioral and perianal rashes are described. She showed a partial response to a pancreatic enzyme preparation which was attributed to its content of a zinc-binding ligand, picolinic acid. Complete recovery occurred on pharmacologic doses of zinc. Exacerbation occurred twice upon withdrawal of the oral zinc medication. The zinc concentrations of plasma and intestinal mucosa were normal.

Acrodermatitis↗
PubMed1980

Studies of glycine metabolism and transport in fibroblasts from patients with nonketotic hyperglycinemia.

Glycine transport in both normal and nonketotic hyperglycinemia fibroblasts was shown to occur by a sodium-dependent system. No significant difference could be detected in either the Km's (1.4 to 2.0 mM) or the Vmax's (6.2 to 16 nmole per mg protein per min) of the three control and three patient cell lines. Valine was a weak competitive inhibitor of glycine uptake. Ki's from both groups fell into the 5.6 to 5.8 mM range. Plasma levels of valine of one patient reached a maximum of 0.6 mM following a valine load. Glycine cleavage activity could not be detected in either control or nonketotic hyperglycinemia fibroblast lines. Serine utilization was the same in both nonketotic hyperglycinemia and control lines.

Biological Transport↗
PubMed1979

Atypical clinical course of ornithine transcarbamylase deficiency due to a new mutant (comparison with Reye's disease).

A male infant with ornithine transcarbamylase (OTC) deficiency is described who was relatively symptom free for 4 months, gradually developed severe spasticity due to cerebral atrophy, and died at 13 months of age. Liver OTC activity was 1.5% of the normal mean. The mutant OTC showed an increased apparent Km for ornithine and an increased pH optimum. These kinetic findings fail to explain the atypical clinical course. The clinical picture of patients with genetic OTC deficiency who present during acute exacerbations together with the elevation of serum glutamic oxaloacetic transaminase and microvesicular fat accumulation in liver, as seen in this case, may suggest Reye's syndrome; however, electronmicroscopic examination of this patient suggested that the normal appearance of mitochondria helps to distinguish the two.

Adult↗
PubMed1978

Relation of specific dynamic action of food (SDA) to growth in rats.

The relation between food induced thermogenesis and anabolic processes was investigated in normal and malnourished rats. The metabolic rate was measured 5 and 17 hr after food removal. The difference between the two measurements was 3.9% during growth arrest of malnourished rats gaining 0.6 g/day, but 20.0 and 28.7% during growth recovery when rats were gaining 4.1 and 5.3 g/day, respectively. The 5 hr postprandial metabolic rates rose in normal rats from 15.6 kcal/day at 4 weeks to 45.3 kcal/day at 15 weeks, and then declined to 38.5 kcal/day at 36 weeks. The difference between 5 and 17 hr postprandial metabolic rates was 28.8% in 5 to 15-week-old rats gaining 5.2 g/day, and 1.1% in 24 to 32-week-old rats who were no longer gaining weight.

Aging↗
PubMed1977

Cerebrospinal fluid glycine in nonketotic hyperglycinemic: effect of treatment with sodium benzoate and a ventricular shunt.

In three infants with nonketotic hyperglycinemia, glycine was increased three-to fourfold in plasma, 13- to 28-fold in lumbar spinal fluid, and was higher yet in ventricular fluid. Oral sodium benzoate lowered cerebrospinal fluid (CSF) glycine by greater than 40%, but did not change the abnormal plasma: CSF ratio. An adult control, made hyperglycinemic with oral glycine, had a normal plasma: CSF ratio. Treatment of one patient with sodium benzoate from birth did not prevent mental retardation; the degree of brain stem depression was a function of CSF glycine in another patient. The persistance of glycine elevation in CSF, although therapy maintained normal concentration in plasma, appears to be caused by overproduction in brain and limitation of the high-capacity lumbar spinal reabsorptive mechanism. Treatment through lowering of CNS glycine by use of a ventricular shunt was explored.

Amino Acid Metabolism, Inborn Errors↗
PubMed1977

Metabolic rate and body composition in rats nutritionally deprived before or after weaning.

In order to manipulate cell size and cell number, rats were subjected to nutrient restriction either prenatally and until weaning at 3 weeks (group I), or from 3 until 10 weeks (group II). Body weights of group I rats were 30% of normal at the height of deprivation at 3 weeks. By 16 weeks of age maximum weights were reached, which remained subnormal in the majority; by 26 weeks only one of six had attained a normal weight. Body weights of group II were 27% of normal at the height of deprivation at 10 weeks; subsequently, only one of six did not recover. The weight, protein, and DNA content of liver, kidney, and heart were significantly decreased. The combined weight and protein content of the three organs was 30% of normal in group I and 25% of normal in group II; the DNA content of the three organs was 57% and 38% of normal in group I and group II, respectively. The greater deficit in weight than DNA content, and the mean protein/DNA ratios reflect a decrease in cell size (or increase in the cell density) of the three organs, which was greater in group I (60%) than group II (71%). Muscle cell density was increased in group I, but not in group II. At 16 weeks of age all parameters were similar in the two experimental groups (ranging from 68% to 82% or normal). Organ weight, protein, and DNA content remained deficient only in group I; these values were 74%, 70%, and 77% of normal, respectively, at 32 weeks. The deficit was proportionate to the deficit in body weight. The basal metabolic rate was measured 17-20 hr after food removal (BMR) on the day the animals were killed. In group I and II correlations between the BMR and four parameters of body composition were linear during a 4-period at the height of deprivation. Comparison of means from the regressions showed no difference between group I and II, or between these groups and normal controls with regard to the BMR per body weight, organ weight, and organ protein. The BMR per mg DNA was lower in group I and II than in normal control rats (P less than 0.05). The BMR of normal rats age 3-32 weeks showed a curvilinear correlation with body weight (BWt), BMR = 1.24 BWt0.583; organ weight (OWt), BMR = 4.30 OWt0.766; and organ protein (OPr), BMR = 4.30 OPr0.604. By contrast, the regression on DNA was linear (BMR = 7.97 + 0.449), although marked changes in body composition occurred between 3 and 32 weeks.

Animals↗
PubMed1976

Nitrogen balance and calorie efficiency in small-for-date dwarfism.

Accelerated weight gain was induced in eight infants, including two patients with dwarfing syndromes, who were small for their dates of birth and continued to be small after birth. The calorie cost of weight gain was higher than in control infants with linear growth failure due to undernutrition, but the degree of inefficiency did not seem to be of practical significance. Nitrogen retentions were appropriate for weight gain or better. Nitrogen retentions in excess of those expected on the basis of weight gain were seen in infants with small-for-date dwarfism and in controls when calorie intakes were low. The ability to induce good nitrogen retentions is thus no reflection of linear growth potential.

Abnormalities, Multiple↗
PubMed1976

Therapeutic effects of glycine in isovaleric acidemia.

The effect of glycine administration on acute leucine loading (125 mg/kg) was tested in a patient with isovaleric acidemia. Serum isovaleric acid at 1-3/4 hr after the leucine loading alone was elevated to 5.60 mg/100 ml and urinary isovaleryglycine excretion was 9.90 mg/mg creatine/24 hr. Whe the same amount of leucine was given with glycine (250 mg/kg) serum isovaleric acid was only 0.93 mg/200 ml. Unfortunately, urine was collected for only 12 hr after the leucine-glycine loading. However, the amount of urinary isovaleryglycine was 26.2 mg/mg creatine in this period. In the following experiments in which a meal containing 80 mg leucine/kg was given, serum isovaleric acid was elevated to 1.14 and 1.01 mg/100 ml at 3 hr and 6 hr after the loading, respectively. How-ever, serum isovaleric acid was only 0.53 and 0.79 mg/100 ml at 3 and 6 hr, respectively, when the identical mean was given with 2 g glycine. The effect of long term glycine administration (250 mg/kg/24 hr) was also tested. It did not prevent two ketotic episodes which were caused by infections. However, the duration of clinical symptoms such as vomiting and a large anion gap in the acute episodes were much shorter with rectal glycine administration. The patient's linear growth and weight gain durin glycine administration was much better than that in the pretreatment period.

Acidosis↗
PubMed1976

Propionic acidemia and hyperlysinemia in a case with ornithine transcarbamylase (OTC) deficiency.

A female infant with episodic hyperammonemia due to a disorder of the urea cycle and who had hyperlysinemia and an unusual elevation of short chain fatty acids, mainly propionate, is described. Both occurred apparently only during attacks of hyperammonemia. Propionic acidemia was ruled out by enzyme studies. OTC deficiency was diagnosed on the basis of: 1) decreased enzyme activity in leukocytes;2) hyperammonemia in response to protein intakes in excess of 2.0 g/kg/day; 3) orotic aciduria in the patient and her asymptomatic mother; 4) suggestive evidence of x-linked dominant inheritance; and 5) exclusion of citrullinemia, argininosuccinic aciduria, argininemia, and disorders of lysine metabolism that are associated with hyperammonemia. Homocitrullinuria, presence of epsilon-N-acetyl-l-lysine in urine, and absence of saccharopine indicate deficiency of the saccharopine pathway of lysine degradation. However, alpha-ketoglutarate reductase was normal in fibroblasts. Since these metabolites were observed only in conjunction with hyperammonemia but not after a lysine load, we suggest that there was competition between ammonia and lysine for alpha-ketoglutarate. The link between disorders of the urea cycle and short chain fatty acid metabolism remains unexplained..

Acid-Base Imbalance↗
PubMed1975

Free serum thyroxine level and basal metabolic rate. Aids to diagnosis in malnutrition and small-for-gestational-age dwarfism.

Measurement of the basal metabolic rate (BMR) and the total and free serum thyroxine values in response to feeding are aids in the diagnosis of growth failure. Infants with small-for-gestational-age dwarfism gained weight poorly in the hospital, had a low BMR before and after spontaneous or induced weight gain, and a normal serum thyroxine value. Infants with linear growth failure due to chronic malnutrition had a normal BMR but a low serum thyroxine value that rose to normal with weight gain; infants with clinical signs of recent weight loss had a low BMR and a low serum thyroxine value, both of which rose to normal with weight gain. Increases of the BMR were sharp and very rapid; they preceded the rise of the serum thyroxine value in some cases.

Basal Metabolism↗