Familial Hypoglycemia Precipitated by Amino Acids

PEDIATRICS ◽  
1956 ◽  
Vol 18 (2) ◽  
pp. 192-192

This paper reports three cases of hypoglycemia in one family and an unrelated case in which profound hypoglycemia and convulsions were produced by the administration of proteins or amino acids. The patients described are similar to the group which have been reported in the literature as infantile idiopathic hypoglycemosis. Casein, leucine, and isovaleric acid fed to the patients caused a marked fall in the fasting concentration of true blood sugar. High protein feeding led to more convulsions and lower concentrations of sugar in the blood than did a low protein diet. Leucine had the most dramatic effect. Casein and leucine did not produce a fall in the sugar in the blood in normal individuals. The literature on metabolic interrelations between amino acids and carbohydrates and the concentration of sugar in the blood is reviewed. The mode of action of amino acids in depressing the concentration of sugar in the blood in infants with spontaneous hypoglycemia is considered and several hypotheses proposed. This paper is of unusual interest and of great importance for the treatment of infants with spontaneous idiopathic hypoglycemia. It is suggested that in this particular type of hypoglycemia high protein diets should be avoided. Small amounts of carbohydrate may be given 30 to 40 minutes after the ingestion of a meal containing protein to offset the effect of protein in lowering the concentration of sugar in the blood.

1993 ◽  
Vol 264 (6) ◽  
pp. G1057-G1065 ◽  
Author(s):  
C. Moundras ◽  
C. Remesy ◽  
C. Demigne

The aim of the present study was to evaluate the effect of changes in dietary protein level on overall availability of amino acids for tissues. For this purpose, rats were adapted to diets containing various concentrations of casein (7.5, 15, 30, and 60%) and were sampled either during the postprandial or postabsorptive period. In rats fed the protein-deficient diet, glucogenic amino acids (except threonine) tended to accumulate in plasma, liver, and muscles. In rats fed high-protein diets, the hepatic balance of glucogenic amino acids was markedly enhanced and their liver concentrations were consistently depressed. This response was the result of a marked induction of amino acid catabolism (a 45-fold increase of liver threonine-serine dehydratase activity was observed with the 60% casein diet). The muscle concentrations of threonine, serine, and glycine underwent changes parallel to plasma and liver concentrations, and a significant reduction of glutamine was observed. During the postabsorptive period, adaptation to high-protein diets resulted in a sustained catabolism of most glucogenic amino acids, which accentuated the drop in their concentrations (especially threonine) in all the compartments studied. The time course of metabolic adaptation from a 60 to a 15% casein diet has also been investigated. Adaptation of alanine and glutamine metabolism was rapid, whereas that of threonine, serine, and glycine was delayed and required 7-11 days. This was paralleled by a relatively slow decay of liver threonine-serine dehydratase (T-SDH) activity in contrast to the rapid adaptation of pyruvate kinase activity after refeeding a high-carbohydrate diet.(ABSTRACT TRUNCATED AT 250 WORDS)


Reproduction ◽  
2011 ◽  
Vol 141 (5) ◽  
pp. 697-706 ◽  
Author(s):  
Gina C Micke ◽  
Tracy M Sullivan ◽  
I Caroline McMillen ◽  
Sheridan Gentili ◽  
Vivienne E A Perry

Changes in maternal nutrient intake during gestation alterIGFreceptor abundance and leptin (LEP) mRNA expression in fetal adipose tissue. It is not known whether such changes persist into adult life and whether they are associated with an effect on phenotype. We investigated the effect of high (240%) and low (70%) levels of recommended daily crude protein intake for beef heifers during the first and second trimesters of gestation on singleton progeny (n=68): subcutaneous (SC) adipose tissue depth at rump (P8) and rib (RF) sites from 65 until 657 days of age; plasma leptin concentrations from birth until 657 days and expression ofIGF1andIGF2, their receptors (IGF1RandIGF2R) andLEPmRNA in perirenal (PR), omental (OM) and SC adipose tissue at 680 days of age. High-protein diets during the first trimester increasedLEPandIGF1mRNA in PR of males and females, respectively, compared with low-protein diets, and decreasedIGF1RmRNA in SC of all progeny but increased RF depth of males between 552 and 657 days. High-protein diets compared with low-protein diets during the second trimester increasedIGF1RmRNA in PR and OM of all progeny;LEPmRNA in PR of males; andIGF2andIGF2RmRNA in OM of all progeny. Conversely,LEPmRNA in OM andIGF2mRNA in PR of all progeny were decreased following exposure to high- compared with low-protein diets during the second trimester. Heifer diet during gestation has permanent sex- and depot-specific effects on the expression of adipogenic and adipocytokine genes and offspring adiposity.


PEDIATRICS ◽  
1962 ◽  
Vol 29 (1) ◽  
pp. 90-96
Author(s):  
Oliver W. Vaughan ◽  
L. J. Filer ◽  
Helen Churella

One-day-old piglets were fed diets of either 50% or 14% protein for 8 weeks; they were given intravenous injections with S35-methionine-labeled plasma protein and were given a nonprotein diet. The plasma protein turnover and the nitrogen excreted were estimated by measuring the S35 activity and the nitrogen in aliquots of blood, urine and feces. During a 102-day period of protein privation, the animals that had received the high-protein diet lost little weight, while pigs previously fed a low level of protein lost 4.4 kg. However the high-protein group had a considerably faster rate of plasma protein turnover, catabolized a much large quantity of protein, and excreted more S35 and nitrogen than did the low-protein group. It is concluded that high-protein diets may make pigs less well able to cope with the stress of sudden protein deprivation.


2002 ◽  
Vol 35 (6) ◽  
pp. 601-607 ◽  
Author(s):  
Janira Lúcia Assumpção Couto ◽  
Haroldo da Silva Ferreira ◽  
Dinalva Bezerra da Rocha ◽  
Maria Eugênia Leite Duarte ◽  
Monica Lopes Assunção ◽  
...  

The effects of high and low-protein diets on the structure of the jejunal mucosa were studied in Schistosoma mansoni infected mice (morphology and histomorphometry). Weaning male albino mice were infected with 80 cercariae, fed with high (20%) or low-protein (5%) diets and compared to uninfected controls under the same conditions. Mice were sacrificed 12 weeks after infection. Animals submitted to a low-protein diet showed lower weight curves, mainly when infected. In the jejunal mucosa, finger-like villi were the predominant pattern among uninfected high-protein fed animals, while the infected ones showed leaf-shaped and flattened villi in most cases. Undernourished infected mice had 65.7% leaf-shaped villi. A significant increase in the number of goblet cells was seen in infected mice. A decrease in the number of absorptive cells was detected in undernourished mice, particularly in infected ones.


1941 ◽  
Vol 74 (6) ◽  
pp. 591-600 ◽  
Author(s):  
H. Philipsborn ◽  
L. N. Katz ◽  
S. Rodbard

The effect of high and low protein diets were studied on fourteen dogs in twenty-four different experiments. In only two of these animals, both with moderate renal excretory failure, was a reversible rise in blood pressure elicited by a high protein diet. The possible mechanisms involved in meeting an increased excretory load are discussed.


1992 ◽  
Vol 67 (2) ◽  
pp. 223-233 ◽  
Author(s):  
J. G. H. Sterck ◽  
J. Ritskes-Hoitinga ◽  
A. C. Beynen

Increased intakes of protein have been shown to reduce kidney calcification (nephrocalcinosis) in female rats. Two questions were addressed in the present study. First, can protein-induced inhibition of nephrocalcinosis be demonstrated when the diets used are balanced for calcium, magnesium and phosphorus in the added protein? Second, can the protein effect be explained by the frequently observed magnesiuria after giving high-protein diets? Nephrocalcinosis was induced in female rats by giving purified diets containing 151 g casein/kg and either an increased concentration of P (6 v. 2 g/kg) or a decreased concentration of Mg (0·1 v. 0·4 g/kg). To these diets 151 g ovalbumin/kg was added at the expense of glucose, and the diets were balanced for Ca, Mg and P in ovalbumin. The diets were given for 29 d. In rats fed on the diet containing 151 g protein/kg, an increased intake of P or a decreased intake of Mg caused nephrocalcinosis as measured chemically by analysis of kidney Ca as well as histologically by scoring kidney sections stained according to Von Kossa's method. The addition of ovalbumin to the diet prevented the induction of nephrocalcinosis. High P intake and low Mg intake with the low-protein diets induced enhanced loss of albumin in urine, suggesting that nephrocalcinosis caused kidney damage. Increased protein intake with a non-calcinogenic diet also caused increased albumin excretion in urine. Irrespective of the composition of the background diet, increased protein intake caused increased urinary excretion of Mg. When all dietary groups were considered, differences in nephrocalcinosis and urinary Mg output were not proportionally related.Nephrocalcinosis: Phosphorus: Magnesium: Protein: Rat


Author(s):  
Biji Xavier ◽  
K. K. Jain ◽  
N. P. Sahu ◽  
A. K. Pal ◽  
G. Maheswarudu

Eighty four days feeding trial (3 feeding cycle ; each cycle of 28 days comprising 21 days with low protein and 7 days with normal or high protein diets) was conducted with Labeo rohita fingerlings to investigate the growth performance. Four diets; D1 (10% CP); D2 (30% CP); D3 (35% CP) and D4 (40% CP) were prepared. A total of 225 fingerlings were distributed randomly into five treatments in triplicates. T1 and T2 groups were fed with D1 and D2 diets respectively throughout the experimental period. The feeding cycle of 28 days consisted of 21 days feeding with D1 and 7 days with D2 (T3); D3 (T4) or D4 (T5). Higher growth rate in terms of specific growth rate, feed conversion ratio and apparent net protein utilisation were found in T2 and T3 group compared to T4 and T5. However, protein efficiency ratio of T3 group was significantly higher than the T2 group (p<0.05). Highest and lowest content of protein and lipid was observed in T2 group, whereas, the ash content was highest in T1 group. Feeding cost wasconsiderably reduced with T3 group of low protein intake (43.63%) registering 20.17% saving in production cost. The results indicate that fish fed with cyclic feeding schedule of 21 days with D1 (10% CP) followed by 7 days with a normal diet, D2 (30% CP) could reduce the production cost with considerable savings in feed cost.


1982 ◽  
Vol 206 (1) ◽  
pp. 13-18 ◽  
Author(s):  
P Fafournoux ◽  
C Rémésy ◽  
C Demigné

After adaptation of rats to a 90%-casein diet, hepatic uptake of alanine is strikingly increased in vivo, with concomitant appearance of a concentration of favourable for uptake. With a high-protein diet, uptake of 2-aminoisobutyrate by isolated hepatocytes in the presence of various concentrations of substrates suggested induction of the A system (high-affinity system), whose emergence has been reported during starvation or after glucagon treatment. The other system (ASC, L) were characterized: induction processes only affected the A system. Dibutyryl cyclic AMP addition resulted in an increase in 2-aminoisobutyrate transport at low substrate concentration, the response being greater after adaptation to a high-protein diet. Evidence is presented suggesting that the increased uptake of amino acids by the liver of rats fed on high-protein diets is obtained by developing favourable gradients and enhancing transport capacities. These adaptations allow sufficient amounts of amino acids to enter the liver, where accelerated metabolism plays a decisive role.


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