Search PubMed⌕ Search

Biomedical subjects

M Gahr

Publications and source records attributed to M Gahr.

137 records · Page 8Linked to original sources
PubMed1977

Imbalance of globin chain synthesis in newborn infants with hemolytic disease after exchange transfusion.

The relative rates of beta-, gamma-, and alpha-globin chain formation in reticulocytes of six newborn infants ,ith hemolytic disease were determined before and in weekly intervals after exchange transfusion. Directly after birth (before exchange transfusion) the beta/alpha, gamma/alpha, and (beta + gamma)/alpha ratios in newborn infants with hemolytic disease (0.35 +/- 0.20, 0.67 +/- 0.12, and 1.06 +/- 0.29) were similar to those of normal newborn infants (0.33 +/- 0.07, 0.65 +/- 0.06, and 0.98 +/- 0.06). The gradual increase in the beta/alpha ratio seen during the postnatal period of eight normal newborn infants was also observed in newborn infants with hemolytic disease after exchange transfusion. In contrast, the gamma/alpha ratio was found to be increased (1.89 +/- 0.25) in all infants with hemolytic disease in the second week after exchange transfusion. The corresponding value for normal newborn infants was 0.48 +/- 0.25. After exchange transfusion the number of hemoglobin F-containing erythrocytes increased. Possible reasohs for this imbalance of globin chain formation after exchange transfusion may be the decreased erythropoiesis and/or the abnormal high hemoglobin A concentration after exchange transfusion or the introduction of an unknown, gamma chain formation-stimulating agent by exchange transfusion. Speculation In newborn infants with hemolytic disease the repression of gamma-globin chain formation normally seen during the postnatal period is replaced by a stimulated gamma-globin chain production after exchange transfusion.

Erythroblastosis, Fetal↗
PubMed1976

Haemolytic anaemia due to glucose-6-phosphate dehydrogenase (G6PD) deficiency: demonstration of two new biochemical variants, G6PD Hamm and G6PD Tarsus.

Two new variants of erythrocytic glucose-6-phosphate dehydrogenase have been found in one German patient and in another patient of Turkish origin. The enzymes were partially purified 165-fold and 111-fold respectively. Both revealed reduced activity, increased thermolability and a pH-optimum in the alkaline region (8.5 and 9.0). One variant (G6PD Hamm) had a low Km-value for glucose-6-phosphate, the other (G6PD Tarsus) exhibited an increased affinity for glucose-6-phosphate and a reduced affinity for NADP+. This enzyme showed an increased inhibitor constant for NADPH with respect to NADP+. Electrophoretic mobility was normal in both cases. 2-Desoxy glucose-6-phosphate was utilized to an increased rate by both variant enzymes (46% and 33%). Also galactose-6-phosphate (29% and 25%) and deamino-NADP+ (230% and 261%) gave increased utilization rates. The mothers of both patients could be identified as heterozygous for this enzyme deficiency.

Adolescent↗
PubMed1976

Fetal erythropoiesis and dyserythropoiesis in juvenile chronic myeloid leukaemia.

Haemoglobin fractions and 16 enzymatic activities of red cells of a patient with juvenile chronic myeloid leukaemia are compared to normal, to comparably reticulocyte-rich, non-neonatal and to fetal red cells. The activities of hexokinase, triosephosphate isomerase, glyceraldehyde-3-phosphate dehydrogenase, monophosphoglyceromutase, enolase and glucose-6-phosphate dehydrogenase are significantly increased in fetal red cells beyond the activities of cell populations with comparable reticulocytosis. The activities of these enzymes are also increased in the patient's erythrocytes. Together with a haemoglobin F concentration of 54% and a concentration of haemoglobin Bart's of 1% these variations reflect the fetal nature of the red cells. Simultaneously, signs of dyserythropoiesis are found in the red cells of the patient: a very high activity of hexokinase and a low pyruvate kinase activity.

Blood Cell Count↗
PubMed1976

Glucose-6-phosphate dehydrogenase (G-6-PD) deficiency in Switzerland. Demonstration of a new variant (G-6-PD Aarau) with chronic nonsphaerocytic haemolytic anaemia.

A new variant of erythrocytic glucose-6-phosphate dehydrogenase has been found in a family of Swiss origin. It is associated with chronic nonsphaerocytic haemolytic anaemia. The enzyme from the erythrocytes of a young boy of this family was partially purified 110-fold and characterized. It revealed reduced catalytic activity, increased thermolability and two maxima of the pH activity curve at pH 7.0 and 8.5. The Km value for glucose-6-phosphate was reduced, that for NADP was normal. The enzyme showed an increased inhibitor constant for NADPH with respect to NADP. Electrophoretic mobility was normal (B+). 2-Desoxyglucose-6-phosphate and galactose-6-phosphate were utilized at normal rates, whereas the analogue deamino-NADP gave an increased utilization rate. The mother of the propositus could be identified as heterozygous for this enzyme deficiency. Chronic haemolysis is possibly due to the increased thermolability of the variant enzyme.

Anemia, Hemolytic, Congenital Nonspherocytic↗
PubMedDate not supplied

[Initial experiences in the treatment of children with metastatic and recurrent neuroblastoma using meta-iodobenzylguanidine].

At four involved hospitals 16 children were treated with Metaiodobenzylguanidine (131J-MIBG). These children had a relapse of neuroblastoma stage III or IV or did not respond sufficiently to chemotherapy. In 10 children extreme bone pain disappeared and they became free of fever during the MIBG-treatment. 10 children responded to the therapy demonstrated either by decrease of the solid tumor part or by decrease of catecholamines in urine or plasma or by decrease of bone-marrow involvement. One patient reached a complete remission continuing up to present time (longer than 180 days). 9 patients died meanwhile of tumor progression. 131J-MIBG is an effective instrument, to get a decrease of the tumor up to a complete remission in extensively pretreated patients with tumor relapse, which are refractory to chemotherapy.

3-Iodobenzylguanidine↗
PubMedDate not supplied

[Chronic benign neutropenia].

In four patients we found a benign chronic neutrocytopenia. They suffered from recurrent infections of the mouth and pharynx region and the upper airway. Furthermore pyogenic skin infections and a lymphadenitis were seen. With the exception of a maturity arrest of the myelopoiesis in the bone marrow there were no hematologic abnormalities. Antibiotic therapy was necessary only during periods of acute infections. As long as there is no consistent terminology we propose to subsume this syndrome under the name benign chronic neutrocytopenia.

Adolescent↗
PubMedDate not supplied

Neonatal septicemia and meningitis in Göttingen, West Germany.

Data were analyzed from 181 infants treated for neonatal septicemia and/or meningitis between 1962 and 1974 (n = 88) and between 1975 and 1982 (n = 93). In addition to an increase in the incidence of septicemia (1962 to 1974, 0.88 case/1000 live births (LB)/year; 1975 to 1982, 2.0 cases/1000 LB/year), there was also a change in the pattern of infection. Group B streptococcal infections were first observed in 1975. Infections with Escherichia coli increased (1962 to 1974, 0.25 case/1000 LB; 1975 to 1982, 0.75 case/1000 LB), while those with Pseudomonas aeruginosa decreased (1962 to 1974, 0.15 case/1000 LB; 1975 to 1982, 0.1 case/1000 LB). Although the incidence of meningitis was similar in both time periods (0.5 case/1000 LB), the relative number of cases declined (1962 to 1974, 51 of 88 patients; 1975 to 1982, 23 of 93 patients). Case fatality also decreased during the second time period (1962 to 1974, 53%; 1975 to 1982, 29%). The most common predisposing factors for septicemia were prolonged rupture of membranes (21 of 93 cases) and prematurity (41 of 93). Blood analysis showed that the immature/total neutrophil ratio was more consistently abnormal (60% of the cases) than were total counts of white blood cells, neutrophils or immature neutrophils.

Enterobacteriaceae Infections↗