PubMed Health⌕ Search

Biomedical subjects

W Tillmann

Publications and source records attributed to W Tillmann.

At least 19 recordsLinked to original sources
PubMed1989

Influence of the red blood cell Ca2+-ion concentration on the erythrocyte aggregation in stasis.

Ca2+ ions were transported into the cell by incubation of the erythrocyte suspension with ionophore A23187, a lipophil electric neutral ion complexing substance. Erythrocyte aggregation could be increased twice, when doubling the intracellular Ca2+-ion concentration. Our measurements lead to the suggestion that an increase of the cytoplasmatic Ca2+ ion changes the physical and/or biochemical properties of the aggregation receptors on the membrane surface, i.e., cell-protein interactions are regulated by alteration of the intracellular Ca2+-ion concentration.

Adolescent↗
PubMed1986

[Continuous subcutaneous deferoxamine infusions in thalassemia major. Improvement in glucose tolerance].

Exocrine and endocrine pancreatic functions were studied in 30 patients with homozygotic beta-thalassaemia. All were treated with continuous subcutaneous deferoxamine infusions for a mean period of 30 months. Three patients (aged 18-22 years) had insulin-dependent diabetes, two before and one shortly after the onset of deferoxamine administration. There was no improvement during the treatment. An abnormal glucose tolerance test was demonstrated in 14 patients (47%) before and in seven (23%) during deferoxamine infusion. Enzyme activity of alpha-amylase and lipase as an expression of exocrine pancreatic function was normal in all during the observation period. Improvement in endocrine pancreatic function was apparently age-dependent: the younger the patient at the onset of treatment the more likely is normalization of the oral glucose tolerance test.

Adolescent↗
PubMed1986

[Longitudinal growth of patients with homozygote beta-thalassemia during continuous subcutaneous infusion of deferoxamine].

Subcutaneous continuous infusions of deferoxamine were administered to 28 children with thalassaemia major. Longitudinal growth beyond the eleventh year was favourably influenced. The normal growth spurt during puberty was not demonstrable in six patients (three boys and three girls) during their puberty. Taking into account standard height allowing for parents' height, prospective final height and ethnic origin, the treatment favourably influenced longitudinal growth in 26 of the 28 children.

Age Determination by Skeleton↗
PubMed1986

[Reduced deformability of erythrocytes as a common denominator of hemolytic anemias].

Impairment of red cell deformability (and decrease of survival time) is the common trait of congenital haemolytic anemias with the exception of the enzyme defect glucose-6-phosphate-dehydrogenase (GPDH) deficiency. Causes for increased red cell rigidity may be: spherocytosis (familial hemolytic anemia), instable less fluid Hb (instable hemoglobinopathy), abnormal HbS-formation (sickle cell disease), genetic synthesis defect of Hb chains (homozygous beta-thalassemia), enzyme defects (autosomal recessive pyruvate-kinase deficiency). With GPDH red cell deformability remains unchanged. Splenectomy may be beneficial in anemias with erythrocyte rigidification if clinical condition so requires (repeated transfusions, aplastic and hemolytic crises). Assessment of red cell deformability in vitro saves laborous and exposure to radiation involving testing of cell survival time (e.g. radiochrome test).

Anemia, Hemolytic, Congenital↗
PubMed1985

[Continuous subcutaneous deferoxamine treatment in thalassemia major. Decrease of hemosiderosis and improvement of liver function].

Liver function during continuous subcutaneous deferoxamine therapy was investigated in 29 patients with homozygotic beta-thalassaemia. Average duration of treatment was 26 months (range 8-51 months). A decrease in haemosiderosis and an improvement in liver function was observed in 27 patients: Mean liver density, determined by computed tomography, decreased from 98 to 84 HU, mean serum ferritin concentration fell from 8028 to 3661 ng/ml, mean serum GOT activity from 44 to 13 U/l and GPT from 51 to 16 U/l. Mean cholinesterase activity, reflecting the improved synthetic activity of the liver, increased from 4063 to 4530 U/l.

Adolescent↗
PubMed1985

[Frequent occurrence of Yersinia infection in hemosiderosis].

Antibodies against Yersinia were found in 12 of 50 patients with hemosiderosis, in 11 of 47 patients with thalassemia major and in one of three patients with Blackfan-Diamond anemia. All patients were treated with subcutaneous continuous deferoxamin-infusions. A systemic yersiniosis occurred in seven patients, all with homozygous beta-thalassemia, in five during and in two before treatment with deferoxamin. Hemosiderosis and infusions with deferoxamin seem to increase the risk of yersinia septicemia.

Adolescent↗
PubMed1984

Aggregate formation of erythrocytes and diabetic retinopathy in children, adolescents, and adults with diabetes mellitus (type I).

In vitro measurements were carried out to study the aggregation of erythrocytes in 33 children and young adolescents, three older adolescents, and 38 adults with type I diabetes. The aggregate formation of erythrocytes in stasis was increased in adult patients with both "good" and "poor" metabolic control when compared to control values. The aggregation of red cells in all children and the younger adolescents, both those under good and poor metabolic control, did not statistically differ from those of controls. Each of the three older adolescents showed an increased aggregate formation of their erythrocytes compared to controls. All adult patients under poor metabolic control exhibited various stages of diabetic retinopathy as shown by fluorescence angiography. Only one child under poor metabolic control showed an early stage of retinopathy (stage I according to Malone). The three older adolescents showed an increased aggregate formation of their erythrocytes. These patients exhibited also stage I of retinopathy. We suggest that the increased aggregation of erythrocytes of both adult and older adolescent patients may be one of the reasons for the development of diabetic retinopathy. Moreover, the normal erythrocyte aggregation of the diabetic children and younger adolescents could help to explain the rare occurrence of microangiopathies in childhood diabetes.

Adolescent↗
PubMed1984

[Deferoxamine in hemosiderosis. Fecal iron excretion during continuous subcutaneous infusion].

Faecal iron excretion during continuous subcutaneous deferoxamine infusion was measured by flameless atom-absorption spectral photometry in 21 patients with homozygous ss-thalassaemia and one patient with Blackfan-Diamond anaemia. Taking into account faecal and urinary iron excretion, more iron was eliminated than was taken up during transfusion and by intestinal absorption. Urine contained 37.9%, faeces 62.1% of the amount of iron eliminated.

Adolescent↗
PubMed1984

Erythrocyte pyruvate kinase deficiency: characterization of a new variant (PK "Aarau").

A new PK variant with moderate hemolytic anemia is described. The enzymes of the nonanemic parents show sigmoidal reaction kinetics, with normal kinetic parameters, but differ with respect to nucleotide specificity, thermostability, and the concentrations of the glycolytic intermediates in the erythrocytes. The most characteristic features of the patient's (daughter) enzyme are a 30% activity, hyperbolic reaction kinetics and only two bands in the SDS-gel electrophoresis instead of three bands observed with the parental enzymes. Moreover, the pH-optimum is shifted to the acidic range, the affinity for PEP and ADP is decreased, ATP inhibition is negligible and FDP-activation is roughly ten times smaller than with controls. The concentrations of 2,3-DPG, 2-PG and PEP in the erythrocyte are increased, but ATP decreased. As there is no consanguinity in the parents and their enzymes are different this PK mutant can be considered to be compound-heterozygous for two different mutant PK alleles.

Adenosine Triphosphate↗
PubMed1984

In-vitro wall shear measurements at aortic valve prostheses.

Wall shear distributions during the cardiac cycle at the valve rings of Starr-Edwards, Björk-Shiley and Lillehei-Kaster aortic valves are measured and compared with thresholds reported for shear-induced trauma of blood components. Further, for the disk valves, the influence of pulse rate on wall shear stresses is evaluated. Hot film anemometry with flush-mounted wall shear probes is used as measurement technique in a pulsatile flow mock circuit. The experimental systolic data support the better hemodynamic characteristics of the disk valves over the ball valve also with respect to the threshold shear stresses of flow induced blood trauma. These results are confirmed by postoperative clinical studies, where lower LDH-values are found with the disk than with the ball valves. During diastole, however, high shear stresses are measured and calculated at the valve ring of the Björk-Shiley prosthesis, which can be referred to the non-overlapping closing mechanism. This result is discussed with respect to the often observed thrombus formation at the disk downstream of the smaller orifice of the Björk-Shiley valve.

Aortic Valve↗
PubMed1983

On the temperature- and salt-dependent conformation change in human erythrocyte pyruvate kinase.

The influence of temperature, K+, Mg2+ and fructose 1,6-bisphosphate on human red cell pyruvate kinase was investigated. Kinetic measurements between 4 degrees C and 43 degrees C revealed a remarkable influence of the temperature on the allosteric behaviour of the enzyme. Below a transition region between 15 degrees C and 20 degrees C (as obtained from an Arrhenius plot) the enzyme shows non-cooperative behaviour, as can be deduced from Michaelis-Menten, Hill and Scatchard plots. At temperatures above 20 degrees C cooperativity increases with rising temperature. This effect becomes even more pronounced at higher temperatures upon addition of increasing amounts of K+ and Mg2+ accompanied by a slight decrease of the reaction velocity. Fructose 1,6-bisphosphate, however, abolishes cooperativity at every temperature and salt concentration measured. Difficulties which arise in evaluating the correct values of V, Km and the Hill coefficient nH with cooperative systems are met by using a computer program of Wieker, Johannes and Hess, especially designed for the determination of kinetic parameters obtained from sigmoidal steady-state kinetics.

Erythrocytes↗
PubMed1983

[Puberty-specific gonadotropin rhythm in girls with homozygous beta-thalassemia during continuous subcutaneous desferrioxamine infusions and a simultaneous blood transfusion regimen].

The aim of the study was to find endocrinological parameters for the effectiveness of both, s.c. desferrioxamine therapy and a blood transfusion regimen, in 5 girls with homozygous beta-thalassemia treated in this way over 12-18 months. Physiologically, a sleep-dependent rhythm of follicle-stimulating hormone (FSH) and luteinizing hormone (LH) secretion is detectable during early to mid puberty. Girls with homozygous beta-thalassemia develop persistant hypogonadotropic hypogonadism in the course of untreated hypothalamo-pituitary hemosiderosis and do not show this rhythm. All our patients developed a sleep-dependent gonadotropin rhythm, increased estradiol concentrations as well as secondary sex characteristics, including menarche in one girl. The occurence of sleep-dependent gonadotropin rhythms in previously hypogonadotropic girls emphasizes the effectiveness of s.c. desferrioxamine therapy of hypothalamo-pituitary hemosiderosis in combination with a blood transfusion regimen.

Adolescent↗
PubMed1983

[Infection prevention following splenectomy: protective effect of a new 17-valent pneumococcal vaccine].

The antibody response to a new, 17-valent pneumococcal vaccine (Moniarix; Smith Kline-RIT) was tested in 43 splenectomised or functionally asplenic patients. The vaccine contained the following types of capsular antigens: 1, 2, 3, 4, 6A, 7F, 8, 9N, 11A, 12F, 14, 15F, 17F, 18C, 19F, 23F, 25 (Danish nomenclature). The vaccination was well tolerated. Local, reversible reddening and swelling was seen in 22 patients. The only systemic side effect was a rise of temperature in 13 patients. Serum titers of more than 300 ng/ml of specific antibodies were considered protective. A complete immunization of all patients was reached only with the capsular antigens 11A and 19F. The other antigens only induced protective serum concentrations in some of the patients. Considering the frequency of infections with the different types of antigens in Western Europe, percentage of asplenic patients at risk for infections with all pneumococcal types has been reduced by the vaccination to about 34%. It is proposed that the combination of pneumococcal vaccine and penicillin-prophylaxis provide effective protection for splenectomised patients against bacterial infections. The duration of penicillin-prophylaxis depends on the age of the patient at the time of splenectomy and the basic disease.

Adolescent↗
PubMed1983

Erythrocyte pyruvate kinase deficiency. A kinetic study of the membrane-localised and cytoplasmatic enzyme from six patients.

Investigations on the activity and the kinetic behavior of red cell pyruvate kinase in the erythrocyte membrane and hemolysate of 6 patients with pyruvate kinase (PK) deficiency are presented and compared with those of normal individuals. Hemolysate: The PK in the hemolysate of normal individuals showed the well-known allosteric properties, reflected by sigmoidal reaction kinetics (Hill-coefficient greater than 1). In contrast to these findings the enzymes of 4 of the patients which had not been splenectomised and which were all in satisfactory condition showed between 15 and 35% of the PK activity of normal individuals in the hemolysates, whereas in the case of the 2 splenectomised patients only 5 and 15% of this activity could be measured. All of the patients' enzymes showed non allosteric (hyperbolic) reaction kinetics (Hill-coefficient approximately 1.0). Red cell membranes: Our studies confirmed earlier reports that not only the hemolysate of the patients, but also their red cell membranes ('ghosts') exhibit PK activity. In normal individuals the activity of the membrane-localised PK is about 5% of the cytoplasmatic one. In case of the membrane-localised enzyme of all patients--irrespective if they were splenectomised or not--the activity is decreased to 30-50%, compared to the activity of ghosts of the control persons. Reaction kinetic studies on the PK isolated from red cell membranes from normal individuals as well as from all 6 patients exhibited hyperbolic Michaelis-Menten kinetics with Hill coefficients around 1.0.

Adolescent↗