Blood Galactose Research Articles

A patient with hereditary galactosemia studied with a screening method for galactose in urine.

SummaryA case of hereditary galactosemia in a newborn infant is reported. Dietary treatment was started on the 9th day of life, which caused a dramatical improvement in the patient's condition. Diagnosis was confirmed by assay of galactose in urine and blood, and finally by demonstration of the enzyme deficiency in the red blood cells of the patient. Both parents had low values for galactose‐1‐phosphate uridyl transferase activity in red blood cells, in agreement with the fact that they are heterozygotes for hereditary galactosemia.A recently described test paper for galactose in urine was used both in the diagnosis of the disease and for the control of the suitability of the patient's diet.In addition to galactose, the urine also contained large anounts of galactitol. On the introduction of galactose‐free diet, galactitol dis‐appeared from the urine more slowly than did galactose.There was an increased concentration of amino acids in the blood and in the urine, as a result of liver damage and also decreased tubular reabsorption. Also the aminoaciduria and the increased blood amino acid concentration disappeared on galactose‐free diet.

Automated blood galactose analysis as a screening method for galactosaemia in milk-fed newborns.

The method of using automated analysis of galactose with galactose oxidase in blood absorbed on discs of filter paper is proposed. A galactose concentrations of 33–100 mg/100 ml the recovery was about 80 per cent the day, 60 per cent after 2 weeks storage at room temperature, and nearly 100 per cent after storage at −80° C for various lengths of time. Blood samples from 202 healthy infants five days old gave zero values for galactose. As yet, no infant with hereditary galactosaemia has been investigated.

Enzymatic determinations of blood galactose with galactose oxidase and galactose dehydrogenase

Enzymatic determinations of blood galactose with galactose oxidase and galactose dehydrogenase

Sugars in blood and urine of milk-fed normal young infants.

Tengström, B. & Wranne, L. Sugars in Blood and Urine of Milk-Fed Normal Young Infants. Scand. J. clin. Lab. Invest. 22, 137-141, 1968. Sixteen normal infants, aged 4-6 days, were given 50 ml of human milk. In blood drawn at regular intervals during the following four hours no galactose was found. In ten infants 4-6 days old the urine collected during four hours after the feeding of 50 ml of human milk contained 0-11 mg of galactose, 0-23 mg of lactose, and 0-3 mg of glucose. The highest concentrations of these sugars were 126, 252, and 14 mg/100 ml, respectively.The 24-hour excretion of sugars in 18 infants aged 1 week-4 months was also studied. 0-90 mg of galactose, 70-249 mg of lactose, and 0-42 mg of glucose were excreted.Routine screening of newborn infants for the detection of galactosaemia can probably be made by testing for galactose in blood.

A Simple Chromatographic Screening Test for the Detection of Galactosemia in Newborn Infants

A simple chromatographic screening procedure for the detection of galactosemia in newborn infants is described. The test uses blood collected and dried on filter paper cards and depends upon the demonstration of blood galactose levels greater than 10 mg/100 ml, levels which are not found in normal newborn infants. The test is economical in materials and time.

A methodological study of the enzymatic determination of galactose in human whole blood, plasma and erythrocytes with galactose oxidase

A method for routine determinations of galactose in whole blood, plasma and erythrocytes with galactose oxidase, peroxidase and o-dianisidine is described. A simple procedure is used to eliminate the disturbing effect of substances such as ascorbic acid, glutathione and uric acid in the method. The quotient galactose in erythrocytes/galactose-in-saline after suspending erythrocytes in galactose-containing saline was the same when determined with the galactose oxidase method as the quotient galactose-in-erythrocytes/galactose-in-plasma when determined with [ 14C]-galactose. This has been taken as an evidence of the accuracy of the galactose oxidase method.

Studies of Blood Enzymes In Mongolism

<h3>To the Editor:—</h3> In previous communications (<i>Lancet</i><b>1</b>:1191,1965;<i>New Eng J Med</i><b>273</b>:1356, 1965) we reported diminished levels of whole blood serotonin and significantly increased levels of leukocyte, erythrocyte, and whole blood galactose¹-phosphate-uridylyltransferase, leukocyte acid and alkaline phosphatases, erythrocyte glucose-6-phosphate dehydrogenase and leukocyte-5-nucleotidase in patients with trisomic mongolism. Normal values were found in the translocation type of mongolism (Down's syndrome). Further studies of the same group of 12 trisomic and 12 translocation mongols matched by age and sex with 12 healthy controls are the subject of the present report. The selection of patients, materials, and chemical methods used have been previously described. Plasma hydroxyproline was measured by the method of LeRoy et al¹and the following five plasma enzymes were assayed according to Sigma²: leucine aminopeptidase, fructose aldolase, isocitric dehydrogenase, glutamic pyruvic transaminase, and creatine phosphokinase. <h3>Results:—</h3> The results are summarized in the Table. Plasma leucine

Quantitative analysis of plasma galactose and glucose by gas-liquid chromatography

A rapid, specific, and reproducible method is described for the determination of galactose and glucose in plasma or blood by means of gas-liquid chromatography. The use of trimethylsilyl ethers facilitates the separation and identification of the respective anomers of galactose and glucose on a Carbowax 20 M column. Several experiments are presented describing the effect of diet and injections of galactose on the blood levels of galactose and glucose in the adult female rat.

THE HANDLING OF SOYA ALPHA-GALACTOSIDES BY A NORMAL AND A GALACTOSEMIC CHILD

1. No alpha-galactosidase activity in homogenates of human small intestinal mucosa was demonstrated either with stachyose, raffinose, or melibiose. 2. In a healthy child, oral raffinose and melibiose loads failed to produce changes in blood glucose, blood galactose, or red cell galactose-1-phosphate. Trace amounts of either saccharide were excreted in the urine. Following the raffinose load, diarrhea occurred and raffinose and melibiose as well as their hydrolysis products were found in the feces. 3. In a galactosemic patient, red cell galactose-1-phosphate rose as expected after ingestion of 2 gm of galactose. No such rise was seen after administration of equivalent and of double equivalent amounts of stachyose. Prolonged dietary supplementation with raffinose did not significantly alter non-fasting erythrocyte galactose-1-phosphate. 4. It is concluded that soybean formulas are generally safe for galactosemic infants; however, caution is advised for patients suffering from diarrhea.

An Automatic Spectrophotometric Method for the Specific Enzymatic Determination of Galactose in Whole Blood and Plasma.

An Automatic Spectrophotometric Method for the Specific Enzymatic Determination of Galactose in Whole Blood and Plasma.

Determination of Exogenous Galactose in Blood using "Specially Purified" Glucose Oxidase

Abstract A method is described for the determination of exogenous galactose in blood by a modification of the Nelson-Somogyi hexose procedure in which glucose oxidase is used to eliminate interfering glucose. The use of "specially purified" glucose oxidase (SPGO) achieves low, regular blanks and satisfactory replicates, and enhances accuracy, in contrast to the results obtained with crude enzyme preparations. The ready availability of SPGO makes the method satisfactory and practical for the clinical laboratory.

THE ESTIMATION OF BLOOD GALACTOSE USING A GLUCOSE OXIDASE-CATALASE REAGENT.

The removal of the blood glucose by yeast fermentation in a conventional blood galactose method is replaced by an enzymic reaction using a glucose oxidase-catalase reagent. The method can be readily modified to suit a variety of blood sugar estimation methods.

A new method for microanalysis of blood galactose

A new method for microanalysis of blood galactose

Determination of Blood Galactose

AbstractA new method of determining blood galactose is reported. The aldohexoses remaining after removal of the glucose by fermentation were determined by the o-toluidine method.

Determination of Galactose in Blood

Abstract A modification of the orcinol procedure of Brückner has been developed to permit rapid analysis of galactose in blood. The method is sensitive to increased amounts of galactose in blood filtrates, but accurate estimations of low concentrations are difficult because of interfering substances. Calculation of results has been simplified by the use of constants. Their derivations are given.

Possible relations of hypoglycaemia induced by mono-amine oxidase inhibition and angina pectoris.

The initially favorable results of the value of the mono-amine oxidase inhibitors in treatment of angina pectoris have latterly been tempered by further experiences of their use(l), and the response to therapy is probably, at best, unpredictable. However, it is of interest that Weiss et al.(2), using one of these drugs, iproniazid, in treatment of depressive patients, showed that those patients whose appetite, weight and sense of well being improved, demonstrated a definite hypoglycaemic response to the drug. It occurred to us that a similar correlation with hypoglycaemia may also exist in patients who showed improvement in the symptoms of angina pectoris as a result of such treatment. In considering the nature of this hypoglycaemia, the ganglion blocking action of iproniazid (3) may have resulted in a type of hypoglycaemic insensitivity due to inhibition of sympathetic discharge to the adrenal medulla and liver, such as follows hexamethonium therapy(4): or secondly, the slight galactose intolerance noted by Weiss et al. may have been a factor in production of hypoglycaemia, since it is known that an increased blood galactose may have some hypoglycaemic action in the normal individual(5). To try to elucidate these points it was decided to compare the response of total blood sugar and blood glucose levels to a mixed oral dose of glucose and galactose under the influence of ganglion blockade from mecamylamine, and mono-amine oxidase inhibition from iproniazid and nialamide respectively. The latter, unlike iproniazid, produces only minimal postural hypotension (6). Methods. The following carbohydrate tolerance test was performed in all subjects. A fasting venous blood sample was collected. then a mixture of 25 g glucose and 25 g galactose in 250 ml water was given by mouth. Further venous samples were withdrawn 10, 20, 30, and 45 minutes later.

Enhancement by hypophysectomy of insulin effect on extrahepatic galactose transfer.

Fasted, normal, and hypophysectomized rats were eviscerated and nephrectomized under light pentobarbital anesthesia. Glucose was infused intravenously at rates that maintained a nearly constant blood glucose concentration. Single injections of galactose were given that produced an equilibrium concentration at 60 min of 247 ± 30 mg%. The galactose level was not significantly different at 90 min. Thus insulin could be administered at 60 min and the 60–90 min blood galactose change used as an index of insulin action. The extrahepatic uptake of galactose in hypophysectomized rats was demonstrated to be hypersensitive to insulin by two criteria: a) significant decreases in blood galactose concentration of hypophysectomized rats were produced by 0.01 unit of insulin/kg, a dose that had no effect on normal rats; b) after 0.02 unit of insulin/kg, changes in blood levels of galactose were greater in hypophysectomized rats than in their normal controls. Hypophysectomy did not alter galactose distribution volume. In neither normal nor hypophysectomized rats did prior administration of growth hormone alter either volume distribution of galactose or insulin hypersensitivity.

The Blood Galactose Disappearance Curve as a Test of Liver Function

The Blood Galactose Disappearance Curve as a Test of Liver Function

Micro-Method for Determination of Blood Galactose by Means of Glucose Oxidase (Notatin) and Anthrone

(1958). Micro-Method for Determination of Blood Galactose by Means of Glucose Oxidase (Notatin) and Anthrone. Scandinavian Journal of Clinical and Laboratory Investigation: Vol. 10, No. 2, pp. 203-210.

Micro-Method for Determination of Blood Galactose by Means of Glucose Oxidase (Notatin) and Anthrone

Micro-Method for Determination of Blood Galactose by Means of Glucose Oxidase (Notatin) and Anthrone