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Biomedical subjects

O Stokke

Publications and source records attributed to O Stokke.

At least 73 records · Page 4Linked to original sources

Deficiency of fumarylacetoacetase without hereditary tyrosinemia.

A variant of the enzyme fumarylacetoacetase (FAH) (E.C.3.7.1.2) in healthy individuals, determined by the enzyme activity, is reported. Analysis of family members of probands with low FAH activity suggests that the enzyme variant causing low activity could be the product of a pseudodeficiency gene. Assumed homozygotes for this gene have only slightly higher enzyme activity than patients with the metabolic disorder hereditary tyrosinemia I (hepatorenal type). No clinical abnormalities have been found in association with the postulated gene.

Adult↗

Dextran 70 versus donor plasma as colloid in open-heart surgery under extreme haemodilution.

Dextran 70 and donor plasma were compared as colloid in the priming solution during heart-lung perfusion in open-heart surgery. The patients underwent surgery for coronary artery or aortic valve disease. One group of nine patients had plasma in the priming solution, while ten patients received dextran 70. Prior to perfusion the patients in the dextran group received monovalent hapten to prevent anaphylactic reactions towards dextran. During the perfusion the same level of haemodilution was established in both groups; the haematocrit values fell from about 39 vol. per cent before perfusion to about 19 vol. per cent after 45 min of perfusion. Eighteen hours postoperatively, the haematocrit had increased to about 35 vol. per cent. The mean concentrations of total serum protein in preoperative samples from the plasma and dextran group were 66.0 g/l and 66.3 g/l, respectively, and fell to 42.6 g/l and 28.3 g/l in samples taken after 45 min of perfusion. The serum-protein concentration in the dextran group remained significantly lower than that of the plasma group throughout the postoperative period. A similar pattern was seen for the albumin concentration. Preoperatively the mean values of the colloid osmotic pressure of plasma were 24.2 mmHg and 25.5 mmHg in the plasma and dextran group, respectively. Corresponding figures 45 min after start of perfusion were 13.9 mmHg and 16.9 mmHg, respectively, significantly higher in the dextran group than in the plasma group. The colloid osmotic pressure exerted by dextran was about 9 mmHg during heart-lung perfusion and about 4 mmHg 18 h later.(ABSTRACT TRUNCATED AT 250 WORDS)

Aortic Valve Insufficiency↗

Effect of exercise training in patients with essential hypertension.

The aim of the present study was to examine the effect of regular exercise training in moderate essential hypertension. Twenty subjects (one woman and 19 men) trained for 45 minutes three times a week for 12 weeks, to 60% of their maximal heart rate. Blood pressure was measured at rest and during a maximal exercise test on bicycle ergometer. All subjects achieved training effect measured as a significant reduction in heart rate at comparable submaximal work level and a significant increase in duration of exercise. Diastolic blood pressure at rest decreased from 107 +/- 7 to 101 +/- 5 mmHg (P < 0.01), and at corresponding submaximal exercise level 116 +/- 11 to 106 +/- 11 mmHg (P < 0.01). No significant reduction in systolic blood pressure was found. Exercise training might in some patients with moderate essential hypertension be an alternative to pharmacological treatment.

Adult↗

Dental features in congenital persistent renal tubular acidosis of proximal type.

The dental features in a hitherto unknown type of renal tubular acidosis (capillary blood pH 7.07-7.15) of proximal type are reported. The patient presented agenesis of three second premolars, delayed development and eruption of permanent teeth, delayed shedding of the primary dentition and severe enamel hypoplasia of the permanent teeth. Apart from exceptionally thin enamel, histologic, fluorescent and polarization microscopic and microradiographic investigation of three primary teeth did not reveal unusual findings. The changes are most probably due to a generalized, acidosis-induced defect in several highly differentiated ectodermal tissues.

Acidosis, Renal Tubular↗

Total creatine kinase activity in cerebrospinal fluid as an indicator of brain damage during open-heart surgery.

In a selected series of 33 patients undergoing open-heart surgery, the creatine kinase (CK) activity in cerebrospinal fluid (CSF) was compared with the clinical results in regard to the central nervous system. The level of CK activity in CSF showed correlation with the degree of irreversible neuronal damage. Determination of CK in CSF may thus be of prognostic value in these patients. Caution is essential, however, in the setting of strict limits above which return to normal or acceptable quality of life is not to be expected.

Brain Damage, Chronic↗

Clinical chemical changes in physical activity.

Physical activity intervenes in metabolism in a number of areas. The changes depend on both the degree and duration of the effort. This article describes some of the biochemical changes known to us, and which on the whole are reflected in the blood and urine. Diagnostically important enzymes, especially CK, ASAT and LD, remain significantly increased for several days after heavy exercise. Renal function is temporarily depressed. The serum levels of urea, creatinine and uric acid rise and may stay high for several days. Electrolyte disturbances and acid-base shifts are short-lived and disappear in the course of hours. Fluid balance can take two or three days to return to normal. The leucocyte and thrombocyte counts in the blood rise and the concentration of several coagulation factors increases significantly. There are in addition changes in plasma proteins. Results of a series of common clinical chemical analyses may be affected by physical activity. It is important to recognise these possibilities in order to avoid clinical misinterpretations.

Acid-Base Equilibrium↗

Accumulation of pyroglutamic acid (5-oxoproline) in homocystinuria.

A 12-year-old boy with homocystinuria due to a cystathionine synthetase defect was shown to accumulate large amounts of pyroglutamic acid (pyroglu). In four different urine samples the concentration ranged between 4.1 and 9.7 mol/mol of creatinine. The pyroglu concentration in plasma was 1.6 mmol/l, and in erythrocytes 750 nmol/g cells. Normally only trace amounts are present. The pyroglu levels normalized during treatment with pyridoxine and folic acid. Accumulation of pyroglu has not previously been described in homocystinuria. Homocysteine can replace cysteine as a substrate for gamma-glutamylcysteine synthetase, an enzyme involved in the biosynthesis of glutathione, giving gamma-glutamylhomocysteine. This dipeptide is efficiently split by gamma-glutamylcyclotransferase to pyroglu and homocysteine. This reaction sequence most likely explains the accumulation of pyroglu in our patient. Ten other homocystinuria patients, who all had lower plasma levels of homocysteine, did not accumulate pyroglu. Our patient might either be a new variant, or more likely the pyroglu formation occurs only high levels of homocysteine.

Amino Acids↗

Assay of fumarylacetoacetate fumarylhydrolase in human liver-deficient activity in a case of hereditary tyrosinemia.

The activity of human liver fumarylacetoacetate fumarylhydrolase (EC 3.7.1.2) has been determined with fumarylacetoacetate as substrate. The Km was found to be 1.3 mu mol/l. Subcellular fractionation showed localization of the enzyme in the particle-free supernatant (cytosol). ZnCl2, CuCl2 and p-chloromercuribenzoic acid had a marked inhibitory effect on the enzyme activity, but no inhibition was observed with a number of anions and substrate analogs. Fumarylacetoacetate fumarylhydorlase activity in liver tissue from a patient with hereditary tyrosinemia was found to be less 2% of the controls. The assay is applicable to 3 mg of liver tissue which may be obtained by needle biopsy.

Acetoacetates↗

Intra-ocular pressure before, during and after extracorporeal circulation in pigs.

Variations in intra-ocular pressure (IOP) were demonstrated before, during and after extracorporeal circulation in pigs. Most probably both the observed increase and reduction in IOP may be explained by corresponding changes in blood osmolality, blood pressure and body temperature. In contrast to clinical findings, no increase in IOP was observed during the period of extracorporeal circulation.

Animals↗

The effect of haemodilution on arterial blood pressure, central venous pressure, intracranial and intra-ocular pressures in pigs.

During the open-heart surgery, haemodilution is performed before extracorporeal circulation is started by the sampling of autologous blood and substituting an electrolyte solution. Following extracorporeal circulation, first the machine-blood and then the autologous blood is reinfused. The haemodilution and reinfusion procedures lead to alterations in blood osmolality and tissue fluid distribution, initiating changes in arterial blood pressure, central venous pressure, intracranial and intraocular pressures. These changes were studied in pigs. It is believed that these potentially hazardous pressure alterations can be minimized through proper handling of the procedure. This study presents some of the mechanisms involved.

Animals↗

Moderate and extreme haemodilution in open-heart surgery: electrolytes, urea, creatinine and osmolality.

Two different programs of treatment, called moderate and extreme haemodilution, respectively, have been compared in patients undergoing aortic valve replacement. Serum electrolytes, urea, creatinine and osmolatity were satisfactory in both groups during and after surgery. A substantial retention of sodium took place in the moderately diluted patients. The extreme group lost more potassium in the urine than the moderate one. A small post-operative rise in creatinine indicates a slight kidney damage in the moderate group. Increased serum urea following moderate dilution might partly be due to increased protein catabolism.

Aortic Valve↗