GLYCOGEN DISEASE OF THE LIVER (VON GIERKE'S DISEASE) WITH HEPATOMATA

PEDIATRICS ◽  
1955 ◽  
Vol 16 (6) ◽  
pp. 785-800
Author(s):  
Howard H. Mason ◽  
Dorothy H. Andersen
Keyword(s):  
Lactic Acid ◽  
Blood Sugar ◽  
Terminal Illness ◽  
Serum Lipids ◽  
Right Heart Failure ◽  
Total Serum ◽  
Epinephrine Injection ◽  
Chemical Studies ◽  
Severity Of The Disease ◽  
Sugar Levels

A case of glycogen disease of the liver (von Gierke's disease), followed from the age of 6 months to death at 10½ years, is reported. The child was dwarfed and mentally retarded. The diagnosis is supported by blood sugar concentrations following the administration of monosaccharides and following epinephrine injection as well as by post-mortem anatomical and chemical studies. Hypoglycemia was controlled by frequent feedings which contained glucose or starch. Levulose and galactose were restricted because low tolerance tests suggested that these substances were rapidly removed from the blood by the liver; after phosphorylation and conversion in the liver their release as glucose was evidently blocked by the same mechanism that blocked the release of glucose from glycogen. Blood lactic acid was consistently elevated, the degree of elevation bearing an inverse relationship to blood sugar levels. It is suggested that excess blood lactic acid contributes to the acidosis and so therapy of the acidosis with lactates would be illogical. The concentration of total serum lipids was always high but fluctuated widely, reaching a peak of 7.45 gm./100 ml., higher during periods of prolonged hypoglycemia and probably reflecting the mobilization of body fat during these periods. The terminal illness was marked by 2 unexplained complications: multiple hepatomata and congestive right heart failure. The severity of the disease varies from patient to patient, in the present case appearing to decrease with advancing age.

SERUM-LIPIDS AND BLOOD-SUGAR LEVELS IN CHILDHOOD DIABETES

The Lancet ◽  
1958 ◽  
Vol 271 (7023) ◽  
pp. 707-710 ◽  
Author(s):  
O.H. Wolff ◽  
H.B. Salt
Keyword(s):  
Blood Sugar ◽  
Serum Lipids ◽  
Childhood Diabetes ◽  
Sugar Levels

The Effects of Isocaloric Exchange of Dietary Starch and Sucrose on Glucose Tolerance, Plasma Insulin and Serum Lipids in Man

1970 ◽  
Vol 38 (1) ◽  
pp. 1-9 ◽  
Author(s):  
M. G. Dunnigan ◽  
T. Fyfe ◽  
M. T. McKiddie ◽  
S. M. Crosbie
Keyword(s):  
Glucose Tolerance ◽  
Blood Sugar ◽  
Serum Lipids ◽  
Plasma Insulin ◽  
Serum Triglyceride ◽  
Dietary Carbohydrate ◽  
Plasma Insulin Levels ◽  
Aged Subjects ◽  
Sugar Levels ◽  
Dietary Starch

1. The effects of isocalorically exchanging dietary starch and sucrose on glucose tolerance, plasma insulin and serum lipids were examined in nine middle-aged subjects. A ‘sucrose’ period in which 70% of dietary carbohydrate was supplied as sucrose was alternated with a ‘sucrose free’ period in which dietary carbohydrate was supplied mainly as starch. Each period lasted 4 weeks; eleven balances were completed. 2. Changes in body weight during the balances were small and statistically insignificant. 3. Fasting blood sugar levels were significantly elevated during the ‘sucrose’ period. During 50 g glucose tolerance tests, blood sugar levels were slightly higher during the ‘sucrose’ period but this difference was not statistically significant. 4. Plasma insulin levels were similar during the dietary periods, both in the fasting state and after 50 g of glucose. 5. Mean levels of serum cholesterol, serum triglyceride and plasma NEFA showed no significant differences between the two dietary periods. 6. It is concluded that glucose tolerance, plasma insulin and serum lipids are not significantly altered by the substitution of sucrose for starch at levels of sucrose intake comparable to those in the Western diet.

scholarly journals Blood Sugar Levels and Serum Lipids in Cases with Glucose Intolerance

1986 ◽  
Vol 13 (6) ◽  
pp. 1389-1396
Author(s):  
Noboru SAITO ◽  
Fusae OHTSUJI ◽  
Kimiko MATSUSHITA ◽  
Tohru KITA
Keyword(s):  
Blood Sugar ◽  
Glucose Intolerance ◽  
Serum Lipids ◽  
Sugar Levels

scholarly journals The Relationships between Blood Sugar Levels and Serum Lipids in Cases with Glucose Intolerance

1987 ◽  
Vol 15 (3) ◽  
pp. 619-625
Author(s):  
Noboru SAITO ◽  
Kimiko MATSUSHITA ◽  
Fusae OHTSUJI ◽  
Tatehiko OKADA
Keyword(s):  
Blood Sugar ◽  
Glucose Intolerance ◽  
Serum Lipids ◽  
Sugar Levels

Plasminogen Activation in Diabetes Mellitus: Normalization of Blood Sugar Levels Improves Impaired Enzyme Kinetics In Vitro

1985 ◽  
Vol 54 (02) ◽  
pp. 413-414 ◽  
Author(s):  
Margarethe Geiger ◽  
Bernd R Binder
Keyword(s):  
Plasminogen Activator ◽  
Blood Sugar ◽  
Metabolic Disorders ◽  
Normal Values ◽  
Diabetic Patients ◽  
Type I ◽  
Plasminogen Activation ◽  
Lag Period ◽  
Sugar Levels

SummaryWe have demonstrated previously that fibrin enhanced plasmin formation by the vascular plasminogen activator was significantly impaired, when components isolated from the plasma of three uncontrolled diabetic patients (type I) were used to study plasminogen activation in vitro. In the present study it can be demonstrated that functional properties of the vascular plasminogen activators as well as of the plasminogens from the same three diabetic patients are significantly improved after normalization of blood sugar levels and improvement of HbAlc values. Most pronounced the Km of diabetic vascular plasminogen activator in the presence of fibrin returned to normal values, and for diabetic plasminogen the prolonged lag period until maximal plasmin formation occurred was shortened to almost control values. From these data we conclude that the observed abnormalities of in vitro fibrinolysis are not primarily associated with the diabetic disease, but might be secondary to metabolic disorders caused by diabetes.

Effects of Rapid Infusion of Ethanol on some Factors Controlling Blood Sugar Levels in Man

1972 ◽  
Vol 33 (3) ◽  
pp. 722-733 ◽  
Author(s):  
John W. Dundee ◽  
Martin Isaac ◽  
Elizabeth A. Davis ◽  
Brian Sheridan
Keyword(s):  
Blood Sugar ◽  
Rapid Infusion ◽  
Sugar Levels

BESTIMMUNG DER INSULINAKTIVITÄT IM MENSCHLICHEM SERUM NACH GLUKOSEBELASTUNG ALS PRÜFUNG DER FUNKTIONSFÄHIGKEIT DES PANKREAS

1963 ◽  
Vol 42 (3) ◽  
pp. 437-452 ◽  
Author(s):  
H. Daweke
Keyword(s):  
Blood Sugar ◽  
Pancreatic Insufficiency ◽  
Normal Subjects ◽  
Insulin Binding ◽  
Epididymal Adipose Tissue ◽  
Moderate Increase ◽  
Glucose Loading ◽  
Before And After ◽  
Oral Hypoglycaemic Drugs ◽  
Severity Of The Disease

Using the method of glucose-1-14C oxydation to 14CO2 on the rat epididymal adipose tissue, the insulin-like activities (ILA) in the serum have been compared before and after oral loading with glucose in normal subjects, in maturity-onset diabetics and in insulin-requiring diabetics. In maturity-onset diabetics mean fasting values were found to be 30% below normal while in insulin-requiring diabetics they were 85% above normal. In normal subjects there was observed, 30 minutes after glucose loading, a moderate increase in blood sugar together with an increase of ILA of 222% above the starting value; in maturity-onset diabetics the increase in ILA was only 106% while the blood sugar was markedly increased. After glucose loading in maturity-onset diabetics, the total amount of insulin detected during the period of the experiment was, on the average, only 45% of that found in normal subjects. In insulin requiring diabetics there was no increase but, on the contrary, a steady decrease of the ILA values, while the blood sugar excessively increased. In general ILA values were higher than those in maturity-onset diabetics. No difference in response was found between maturity-onset diabetics treated with diet alone and those treated with diet and oral hypoglycaemic drugs. In contrast to the absolute ILA values, the index of insulin reserve, is of value in assessing the functional capacity of the pancreas. This index decreases progressively with the severity of the disease and reaches a maximum of 54% of the normal in maturity-onset diabetics, which can satisfactorily be explained by pancreas insufficiency. Only in some cases of insulin-requiring diabetics was an insulin reserve still detectable. The biological inactivity of the insulin circulating in the blood can be deduced from the increased ILA-values, as compared with those found in maturity-onset diabetics. Obviously some of this insulin can be released by the addition of glucose. It is likely that, in addition to pancreatic insufficiency, insulin-binding or insulin-inactivating antibodies play a part in the pathogenesis of insulin-requiring diabetes.

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