摘要:

Abstract.The ability of a galactokinase deficient patient to metabolize galactose, galactitol and galactonate was quantitated. In galactokinase deficiency, conversion of galactose to CO2is minimal. Apparently the defect is extensive, involving all tissues. Galactitol and galactonate, injected intravenously, were not metabolized. The administration of C‐1 and C‐2 labelled galactose resulted in14CO2excretory patterns similar to that observed in uridyltransferase deficient mutants. The different fates of C‐1 and C‐2 observed in this galactokinase deficient patient give support to the existence of a direct oxidative pathway for galactose. Galactonate, although present in urine during the period of observation following injection of radioactive galactose failed to become l

ISSN:0014-2972    

DOI:10.1111/j.1365-2362.1974.tb02318.x

出版商:Blackwell Publishing Ltd    

年代:1974

数据来源: WILEY

摘要:

Abstract.Biologically active bovine parathyroid hormone (b PTH) 1–34 fragment infused over one hour in normal subjects produced an immediate and sharp increase in the excreted fractions of filtered bicarbonate, sodium and potassium, followed by the return to pre‐infusion levels as soon as the administration of b PTH was stopped. There was a gradual but steady increase in the excreted fraction of filtered phosphate but a decrease in the excreted fraction of filtered calcium and magnesium. The excreted fractions of these ions were still abnormal 150 minutes after the completion of PTH infusion. The urinary excretion of 3′5′‐cyolic AMP increased immediately about one hundred‐fold but returned rapidly to pre‐infusion levels. Urinary clearance of cyclic AMP approximated glomerular filtration rate in control periods and was twenty to thirty times greater during b PTH infusion. In subjects overloaded with bicarbonate, b PTH brought about a decrease in bicarbonateTmand the same effects on the urinary excretion of other electrolytes. 3′5′‐cyclic AMP excretion was clearly higher in control periods and reached higher levels during b PTH infusion when compared to subjects without an alkaline load. 3′5′‐cyclic AMP excretion and fractional clearance were also clearly higher in subjects not given b PTH when control periods were compared to periods with bicarbonate infusion or after acetazolamide administration. During distal blockade obtained by simultaneous administration of chlorothiazide and ethacrynic acid, there was a delay in the rise of 3′5′‐cyclic AMP excretion after b PTH administration. It can be concluded from these studies that the pattern of excretion of 3′5′‐cyclic AMP is similar to that of bicarbonate, sodium and potassium. The increase of 3′5′‐cyclic AMP excretion when urinary pH is above 7 suggests a diffusion trapping mechanism for the secretion into the lumen of this nucleotide. Distal diuretics used in distal blockade did not inhibit 3′5′‐cyclic AM

ISSN:0014-2972    

DOI:10.1111/j.1365-2362.1974.tb02319.x

出版商:Blackwell Publishing Ltd    

年代:1974

数据来源: WILEY

摘要:

Abstract.A variety of metabolic parameters were evaluated in 23 healthy male volunteers, after ethanol administration (1.5 g/kg) and correlated to the symptoms of hangover. The degree of intoxication and hangover were estimated using a simple test program according to which 11 subjects had severe and 12 mild hangover. Sequential determinations of blood ethanol, acetaldehyde, glucose, lactate, free fatty acids (FFA), triglycerides (TG) and ketone bodies were made during the 21 hours after administration. Each subject served as his own control by taking part in an identical experimental session but receiving no ethanol.The maximal hangover occurred 12 to 14 hours after the initiation of drinking. At this time almost all of the ethanol and acetaldehyde had been eliminated from the blood. The intensity of the hangover did not correlate with the peak concentrations of ethanol or acetaldehyde in the blood. Marked hypoglycaemia regularly followed the administration of ethanol, but the nadir blood glucose values had no correlation with the intensity of hangover. Ethanol induced a significant rise in blood lactate concentration, the extent of which was similar in subjects with mild or severe hangover.During the most intensive hangover plasma FFA level increased when compared to the values observed during the control period. These concentrations were significantly higher in subjects with severe hangover than in those whose hangover was mild. An increase in plasma TG concentration regularly followed ethanol administration but the extent of the rise did not correlate with the degree of intoxication or hangover. Ethanol had a marked antiketogenic effect during the first 8 hours of the experiment after which the concentration of ketone bodies rose rapidly above the control level. Again the changes showed no correlation with the intensity of hangover.The results indicate that the intensity of hangover bears little relation to the changes in the metabolite concentrations measured. This suggests that the symptoms of hangover can not be accounted for by the ethanol‐induced alterations of the major blood metabolite concentration

ISSN:0014-2972    

DOI:10.1111/j.1365-2362.1974.tb02320.x

出版商:Blackwell Publishing Ltd    

年代:1974

数据来源: WILEY

摘要:

Abstract.A sixty‐one year‐old man with Gaucher's disease is described. The disease was manifested by splenomegaly, hypersplenism and elevations of serum acid phosphatase and immunoglobulin but no bone lesions were roentgenologically demonstrable and bone marrow biopsy failed to reveal Gaucher cells. The diagnosis was established prior to splenectomy by the finding of decreased β‐D‐glucoside glucohydrolase (β‐D‐GGH) activity in his peripheral leukocytes. Examination of the surgically removed spleen revealed Gaucher cells, a large excess of glucocerebroside and decreased β‐D‐GGH activity. Splenectomy was followed by normalization of haemoglobin, white cell and platelet counts and of the serum acid phosphatase and immunoglobulin levels. The patient's leukocyte β‐D‐GGH activity showed an optimum pH similar to that of normal leukocyte enzyme, about 5.4, using both glucocerebroside (Glc‐Cer) and 4‐methylumbelliferyl‐β‐D‐glucopyranoside (MU‐Glc) as substrate. The patient's spleen β‐D‐GGH activity showed a pH optimum of about 4.5, which differed slightly from that of normal spleen enzyme. The Km values of β‐D‐GGH in the patient's leukocytes for Glc‐Cer and MU‐Glc were 1.35 × 10−4M and 3.6 × 10−3M, respectively, i.e. in the normal range. The patient's splenic tissue Km values were for Glc‐Cer and MU‐Glc 0.52 × 10−4M and 5.8 × 10−3M, respectivel

ISSN:0014-2972    

DOI:10.1111/j.1365-2362.1974.tb02321.x

出版商:Blackwell Publishing Ltd    

年代:1974

数据来源: WILEY

摘要:

Abstract.The validity of the intravenous fat tolerance test (IVFTT) as a tracer for the fractional turnover rate of endogenous plasma triglycerides (TG) has been studied in 32 fasting men with either normal or elevated plasma TG concentrations. The endogenous plasma TG turnover was determined by sampling arterial and hepatic vein blood, determination of splanchnic net secretion of plasma TG and calculation of fractional TG turnover rate. Later the fractional elimination rate of exogenous TG was determined following a single IV injection of Intralipid® (IVFTT). The TG fractional removal rate constants derived from these two tests were significantly correlated (r= 0.6‐0.7) but IVFTT gave higher absolute values. A statistical evaluation showed that the error for the determination of the fractional turnover rate of endogenous TG and the IVFTT were about 30–50% and 10% respectively. It is probable that the correlation would have been still better if the error for the estimation of endogenous plasma TG turnover had been lower. A significant negative correlation was found between very low density lipoprotein‐TG concentration and fractional removal rate of both endogenous and exogenous plasma TG (r= −

ISSN:0014-2972    

DOI:10.1111/j.1365-2362.1974.tb02322.x

出版商:Blackwell Publishing Ltd    

年代:1974

数据来源: WILEY

摘要:

Abstract.There is marked alteration in endocrine activity during acute myocardial infarction (AMI). Many of the hormones involved act by changing target cell concentrations of adenosine 3′:5′ monophosphate (cyclic AMP). Since cyclic AMP can escape from cells, changes in intracellular concentrations of the nucleotide may be reflected in changes in plasma levels. The purpose of this study was to determine whether plasma cyclic AMP concentrations alter during the development of AMI.—Elevation of plasma cyclic AMP concentrations was noted during the first few hours following the onset of symptoms of AMI. The highest concentrations, recorded in a group of four patients with shock, showed a range of 54–193 pmol/ml which was significantly elevated compared to the normal range of 9–22 pmol/ml. This increase in plasma cyclic AMP correlated significantly and positively with plasma concentrations of total catecholamines. Patients with the worst prognosis had the highest plasma concentrations of cyclic AMP and total catec

ISSN:0014-2972    

DOI:10.1111/j.1365-2362.1974.tb02323.x

出版商:Blackwell Publishing Ltd    

年代:1974

数据来源: WILEY

摘要:

Abstract.Several years ago we demonstrated in man that increasing plasma very low density lipoprotein (VLDL) triglyceride (TG) concentrations were associated with increasing VLDL‐TG production rates, and that this relationship described a rectangular hyperbola which could be characterized in terms of enzyme kinetics. Utilizing this approach Nikkilä and Kekki confirmed these observations in a large group of normal subjects. Moreover, after analyzing the Km and Vmax values of their subjects they noted a striking and important sex difference: females were found to have significantly lower Km values than men, whereas the Vmax values for the two groups were the same. This indicates that for a given VLDL‐TG production rate, females will have lower plasma TG concentrations. We have now studied 53 subjects with widely varying plasma TG concentrations, and have been able to detect a similar sex difference in subjects with hypertriglyceridaemia. Furthermore, we have noted a highly significant direct relationship between TG concentration and production in both sexes, and have found no relationship between an individual's Km value and his coexisting plasma TG level. These results allow us to conclude that: 1) the sex difference in TG kinetics which Nikkilä and Kekki originally note in normals can now be extended to include hypertriglyceridaemic subjects, and 2) in general, overproduction, and not underutilization, is the initiating event in most cases of hypertriglycerid

ISSN:0014-2972    

DOI:10.1111/j.1365-2362.1974.tb02324.x

出版商:Blackwell Publishing Ltd    

年代:1974

数据来源: WILEY

摘要:

Abstract.The purpose of the present study was to assess the influence of liver disease on the hepatic drug‐metabolizing enzyme systems. The clearance of antipyrine was found to be significantly decreased in 13 patients with liver disease compared with 9 control patients (18.5 and 58.6 ml/min. respectively). Among the patients with liver disease, those with a severely reduced capacity for work had significantly lower clearance of antipyrine than non incapacitated patients (12.5 and 25.4 ml/min. respectively). The clearance of antipyrine was significantly correlated with the galactose elimination capacity, the serum albumin and the prothrombin values. The results indicate that the drug‐metabolizing capacity of the liver changes in parallel with the other metabolic functions, and the use of the clearance of antipyrine as a quantitative test of liver function is sugges

ISSN:0014-2972    

DOI:10.1111/j.1365-2362.1974.tb02325.x

出版商:Blackwell Publishing Ltd    

年代:1974

数据来源: WILEY

摘要:

Abstract.Several reports have demonstrated that the lungs are the most important site of conversion of angiotensin I to angiotensin II. The purpose of the present study was to assess the extent of extra‐pulmonary conversion in man, during cardiopulmonary bypass. Plasma concentrations of angiotensin I and immunoreactive angiotensin II, and plasma renin activity were simultaneously determined, using specific radioimmunoassays, during extra‐corporeal circulation in 13 patients undergoing major cardiac surgery. Generally the renin‐angiotensin system was stimulated during cardiopulmonary bypass with maximum values occurring at different time. A highly significant correlation was found between plasma renin activity and angiotensin I and II concentrations respectively, as well as between these two peptides. Positive correlations were also obtained between arterial and venous samples for plasma renin activity and angiotensin I and II. Thus the presence of angiotensin II in plasma in the absence of pulmonary circulation and its parallel variations with plasma renin activity indicate that converting activity by extra‐pulmonary sources is not neg

ISSN:0014-2972    

DOI:10.1111/j.1365-2362.1974.tb02326.x

出版商:Blackwell Publishing Ltd    

年代:1974

数据来源: WILEY

摘要:

Abstract.During a follow‐up examination of patients after radioiodine therapy for thyrotoxicosis, 128 patients without recurrent hyperthyroidism were investigated for the clarification of different degrees of hypothyroidism. The clinical diagnosis and the conventional tests for circulating thyroid hormones were compared to the estimation of serum thyroid stimulating hormone (TSH), and to the thyrotropin‐releasing hormone (TRH) test, which was performed with 500 μg synthetic TRH I.V., and referred to age and sex specific normal ranges. Moreover, serum triiodothyronine (T3) was estimated by radioimmunoassay. Results: 1. A gradual classification of biochemical group‐differences gave more significant discrimination than a division into groups based on clinical impression. Different grades of severity of hypothyroidism could be demonstrated by highly significant differences of free thyroxine index (FT4‐Index) between the 1st group of patients with low FT4‐Index and a 2nd group with raised basal TSH (and normal FT4‐Index), between the 2nd group and a 3rd group with an elevated value of Δ TSHmax(and normal FT4‐Index and basal TSH), and between the 3rd group and a 4th group of biochemically normal reacting patients and controls. There was a less significant difference with very considerable overlap between clinically established groups. 2. T3‐uptake, total T4and FT4‐Index are not sufficient for detecting hypothyroidism in individual patients, although their group‐differences are significant in biochemical classification. 3. Neither between clinical nor between biochemical groups was there any significant correlation with serum cholesterol. 4. Triiodothyronine can be normal or elevated in a situation with low T4and raised TSH concentrations. Conclusions: Evidence could be given that hypothyroidism is a graded phenomenon. Its classification by biochemical data is more reasonable than a clinical division. Advancing severity of hypothyroidism after radiation therapy is compensated during a certain period by supplementary production of triiodothyronine. As thyroid hormone concentration in patients with pathological serum TSH or TRH‐test is significantly lower than in euthyroid patients or in controls, replacement therapy in early stages of hypothyroidis

ISSN:0014-2972    

DOI:10.1111/j.1365-2362.1974.tb02327.x

出版商:Blackwell Publishing Ltd    

年代:1974

数据来源: WILEY