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Biomedical subjects

W Tillmann

Publications and source records attributed to W Tillmann.

At least 55 records · Page 3Linked to original sources
PubMed1977

Congenital nonspherocytic hemolytic anemia associated with glucosephosphate isomerase deficiency: variant Paderborn.

The deficient red cell enzyme glucosephosphate isomerase (GPI) was characterized in a patient of German origin who had already been described, with congenital nonspherocytic hemolytic anemia, and in his heterozygous parents. The variant enzyme differs from the known GPI variant enzyme differs from the known GPI variants by the electrophoretic mobility, the thermal stability, and the leukocyte activity. No differences are found between normal GPI and the variant regarding the affinity to fructose-6-phosphate, the pH optimum and the thermal optimum. Since the electrophoretic pattern and the properties of the parenteral GPI are identical the propositus seems to be homozygous for an abnormal allele and not double-heterozygous as some other cases with GPI deficiency are. Recently, immunological studies have shown that the variant differs from other similar variants. According to the birthplace of the patient the variant is called "Paderborn".

Anemia, Hemolytic, Congenital Nonspherocytic↗
PubMed1977

[Decreased flexibility of newborn infant erythrocytes].

The rheological properties of erythrocytes of 10 full-term infants were studied. A method of filtration and measurements of viscosity of erythrocyte suspensions with a hematocrit of 80% were used. Erythrocytes of newborn infants were less flexible than erythrocytes of healthy adults. Rigidity of erthrocytes was due to an increased hemoglobin content of the erythrocyte membranes which was measured in isolated ghosts. After incubation of the cells with acetylphenylhydrazin Heinz-bodies were formed in the erthrocytes of adults. The flexiblity of these cells decreased markedly. More Heinz-bodies were found in the erythrocytes on the newborn infants, when their cells were incubated under identical conditions. These erythrocytes became more rigid than the erythrocytes of adults. From these results we conclude that the more rigid erythrocytes of the newborn infants would not pass the splenic sinus, especially when they contain Heinz-bodies. The rheological properties of the erythrocytes of newborn infants explain the shortened life-span of red cells of newborn infants.

Cell Survival↗
PubMed1977

Decreased deformability of erythrocytes in haemolytic anaemia associated with glucosephosphate isomerase deficiency.

Deformability of erythrocytes from four patients with different types of glucosephosphate isomerase (D-glucose-6-phosphate ketoisomerase, GPI) deficiency has been determined by cell filtration. Young as well as whole erythrocyte populations had a markedly increased rigidity and an abnormally strong attachment of haemoglobin to the inner surface of isolated membranes. Acidic environment may enhance membran rigidity in vitro and also during passage of the erythrocytes through the spleen. The decrease of deformability at a pH of 6.8 was most pronounced in the splenectomized patients, and likewise in erythrocytes from the splenic artery, which were obtained from one patient during splenectomy. It is suggested that the metabolic environment of the spleen, with its low pH, impairs the deformability of GPI-deficient erythrocytes and predisposes them to splenic sequestration. The clinical improvement of all patients following splenectomy which is accompanied by an increase of the erythrocyte survival time and by unchanged reticulocyte counts, is in accordance with this view.

Adult↗
PubMed1977

Membrane deformability of erythrocytes with glucose-6-phosphate dehydrogenase Hamburg.

Deformability of erythrocytes of a patient with chronic nonspherocytic hemolytic anemia caused by a G-6-PD variant (G-6-PD Hamburg) in red cells was studied. The flow rate of erythrocytes from this patient through 5 mum polycarbonate sieves was higher than that of red cells from healthy adults. Even under oxidative stress in vitro, the deformability of erythrocytes of the patient was only slightly decreased. The residual hemoglobin content of red cell membranes (ghosts) from this patient was lower than that of ghosts from healthy adults and of ghosts prepared from comparable reticulocyte-rich blood withoug G-6-PD deficiency. In contrast to the low enzymatic activity of G-6-PD Hamburg in the hemolysate, a high activity of this enzyme could be demonstrated in the ghosts from the patient. In view of the flexibility of this patient's erythrocytes splenectomy is not to be recommended.

Adult↗
PubMed1977

[Indomethacin induced corneal deposits (author's transl)].

After a summary of corneal changes, caused by drugs (gold, Chloroquine, Phenothiazines, Amiodaron), a personal case of indomethacin induced deposits is described. The chief characteristic was was a ring-shaped formation of the grey-whitish lines in the epithelium. A retinopathy was not present.

Aged↗
PubMed1976

Severe anemia due to transient pure red cell aplasia in early childhood. Arrest at the level of the committed stem cells?

Five patients, 11 months to 3 4/12 years old with severe normochromic, normocytis anemia and reticulocytopenia are reported. At the height of erythropoietic arrest when erythroid precursor cells were completely absent, undifferentiated stem cells (transitional cells), accumulated in the bone marrow. They disappeared again upon spontaneous resumption of erythropoiesis. We suggest that the erythropoietic arrest had occurred at the level of the hematopoietic stem cell. All patients recovered within 1 week of diagnosis. No steroid therapy was given. Failure to recognize this clinical entity leads to unnecessary diagnostic and therapeutic procedures including the transfusion of blood.

Anemia, Aplastic↗
PubMed1976

Rheological properties of red cells in haemoglobin Köln disease.

The rheological properties of erythrocytes of four patients with haemoglobin Köln (Hb Köln) disease were studied. Filtration of erythrocytes through polycarbonate sieves with a pore diameter of 5 mum and viscosity measurements of erythrocytes suspensions with a PCV of 80% were carried out. The rheological properties of erythrocytes of two of the patients were severely altered. In both patients an increased haemoglobin attachment to erythrocyte membranes resulting in a decreased filtration rate of erythrocytes was found. One of these cases had been splenectomized some years before. About half of the erythrocytes of this patient contained large Heinz bodies. Erythrocytes of this patient showed a decreased filtration rate and an increased viscosity. Moreover, erythrocytes of healthy adults containing Heinz bodies after incubation with acetylphenylhydrazine showed a decreased filtration rate as well as an increased viscosity. Membranes of erythrocytes of the other patients contained haemoglobin in an amount within the normal range of haemoglobin content of membranes of healthy adults. Erythrocytes of these patients showed normal rheological properties. The haemolytic process in these patients was only mild. We suggest that the amount of total haemoglobin attached to the erythrocyte membrane corresponds to the percentage of the unstable Hb Köln present in the erythrocytes of the particular patient.

Adolescent↗
PubMed1976

[Luxation and evulsion of the eyeball by car accidents (author's transl)].

The following unusual lesions have been seen, caused by splintered motor-car windscreens: 1. complete evulsion of the globe in combination with a visual field defect of the upper temporal quadrant of the other eye, 2. luxation of the globe, where an alleged lost big portion of the upper lid was dislocated deep into the orbit by glass splinters.

Accidents, Traffic↗
PubMed1976

[Complete or peripheral iridectomy in acute glaucoma? (author's transl)].

The re-examinations of 28 complete sector and 27 peripheral iridectomies, performed because of acute glaucoma, showed an equally good regulation of IOP from both methods, even in longstanding cases (stadium III of Leydhecker) and when an operation during an attack is necessary. The postoperative reduction in vision mainly caused by cataract, was seen more frequently after sector iridectomy. In acute glaucoma the peripheral iridectomy as the less extensive and less severe procedure is to be preferred to the sector iridectomy with the exception, that an optical iridectomy is indicated.

Adult↗
PubMed1976

[Senile retinoschisis and cryocoagulation (author's transl)].

This is a report of experiences with the cryotherapy of senile retinoschisis. In spite of the prophylaxis retinal detachment occurred in 2 out of 30 eyes. The critical question, whether the prophylactic treatment is worthwhile or not, can be positively answered, when compared to the results of non treated retinoschisis (Shea et al., Okun and Cibis). A macular puckering was not seen.

Adult↗
PubMed1976

[Mycosis fungoides affecting the eye and internal organs (author's transl)].

In two patients with mycosis fungoides the following were found in the region of the eye: Infiltration of the eyelid (sometimes board-like), infiltrations and swellings of the connective tissue, orbital infiltrations (hard as a board) with secondary glaucoma, atrophy of the optic nerve, amaurosis, Descemet spots, cells in the aqueous humour, preretinal opacities of the vitreous body), intussusception of retinal vessels, grey infiltration of the retina.

Adult↗
PubMed1976

[Unusial oraparallel changes in the retinal periphery (author's transl)].

6 patients (8 eyes) showed peripheral oraparallel changes, that differ ophthalmoscopically from lattice degeneration: Smaller and mostly depigmented, they have a longer circular extension without palisades. The lower temporal quadrant was mainly concerned. Probably the unusual retinal changes are a variation of the pigmentary epithelium without a tendency to form a retinal detachment.

Adolescent↗
PubMed1975

Membrane-localized pyruvate kinase of red blood cells in hemolytic anemia associated with pyruvate kinase deficiency.

Pyruvate kinase activity of red blood cell membranes, which is normally masked, has been determined after mechanical disruption of the membranes in normal individuals and in three homozygous patients with pyruvate kinase deficiency. Although patients 1 and 2, who were siblings, had relatively high enzyme activities in their hemolysates, they had the severest form of the disorder. The activities of their membrane fragments were decreased to seven per cent of fragments of normal membranes. Patient 3 had a mild form of hemolytic anemia despite a low enzyme activity of his hemolysates. The membrane fragments of this patient contained 28 per cent of the pyruvate kinase activity of normal fragments. The data suggest a relationship between the amount of membrane-localized pyruvate kinase and the severity of the clinical disorder. The reduced production of ATP by the enzyme portion localized within the membrane may cause an impairment of membrane functions in pyruvate kinase deficiency.

Aged↗