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Biomedical subjects

R Seger

Publications and source records attributed to R Seger.

At least 145 records · Page 8Linked to original sources

[Septic granulomatosis in adults].

Chronic granulomatous disease is a usually X-linked disorder of granulocyte bacterial killing. At least 8 variants can be distinguished biochemically. Two male patients aged 20 and 24 years with a relatively benign clinical course are presented. A partial defect of the membrane-bound cytochrome b-245 with a residual bactericidal capacity was detected in both. Due to continuous antibacterial prophylaxis (trimethoprim/sulfamethoxazol) and treatment of infections with intracellularly accumulating agents (e.g. rifampicin, fosfomycin, clindamycin), even patients with complete defects of bacterial killing can reach adulthood today. Physicians and surgeons should be aware of this disorder, which is no longer a "fatal granulomatous disease of childhood".

Adult↗

[Bacteremia and sepsis as sequelae of suction biopsy of the rectal mucosa].

A neonate developing E. coli septicaemia following a rectal suction biopsy is presented. To determine the incidence of transient bacteraemia following this procedure, a prospective study was performed. In nine children aerobic and anaerobic blood cultures were taken before and 5, 10, 15 and 20 minutes after the beginning of the investigation. Transient bacteraemia was demonstrated in two patients by growth of E. coli, Strept. faecalis and Bact. fragilis. In two others growth of Staph. aureus was thought to be due to contamination. In five patients all cultures were sterile. None of the children developed symptoms of septicaemia. Antibiotic prophylaxis is discussed and recommended.

Biopsy, Needle↗

Short communication. Glycogenosis Ib: neutrophil microbicidal defects due to impaired hexose monophosphate shunt.

We studied neutrophil microbicidal function and oxidative metabolic activity in a patient with glycogenosis Ib. The intracellular killing defect and the respiratory burst abnormality in gycogenosis Ib neutrophils were confirmed. The impaired oxygen-dependent microbicidal activity was shown to result from impaired hexose monophosphate shunt activity (impaired endogenous NADPH synthesis) and could be corrected by homogenization of the cells, followed by the addition of exogenous NADPH. Our data are thus consistent with a possible role for glucose-6-phosphate transport in neutrophil microbicidal function. We recommend a continuous prophylaxis with co-trimoxazole in patients with glycogen storage disease Ib.

Adolescent↗

[Neonatal Streptococcus group B sepsis: problems of early diagnosis, therapy and prevention].

38 cases of neonatal group B streptococcal (GBS) sepsis (31 with early onset and 7 with late onset) were observed during the years 1976-1982. Early onset disease showed the following clinical characteristics: 1. frequent lack of risk factors for infection in obstetric history, 2. very early onset of symptoms of respiratory distress, 3. rapid development of shock after only minor or missing clinical signs of infection, and 4. unspecific findings on chest radiography. Neutropenia or marked leukocyte left shift as well as the presence of gram-positive cocci in gastric or tracheal aspirate proved to be useful diagnostic clues. The latex agglutination test for the detection of GBS-antigen in urine yielded in our hands many false positive results and unspecific reactions. Given the big difficulties of early diagnosis of early onset GBS-sepsis, the relatively liberal use of antibiotics in newborns with respiratory distress is probably unavoidable. Hereby prior culturing of blood and early termination of antibiotic therapy with negative cultures (of blood, CSF, tracheal aspirate) seems essential to us. The clinical significance of newer therapeutic (granulocyte transfusions, exchange transfusions, immunoglobulins) and prophylactic (intra-partum antibiotics, vaccination) modalities is according to the current literature still not clear in many respects.

Antigens, Bacterial↗

Intermittent ketoconazole therapy of chronic mucocutaneous candidiasis in childhood.

We report the clinical and laboratory findings in two children with chronic mucocutaneous candidiasis (CMC) treated successfully with intermittent long-term ketoconazole therapy. Both had chronic infection of the nails, skin and mucous membranes with positive cultures for candida. Both were resistant to multiple local and systemic antifungal agents. After institution of ketoconazole therapy there was a dramatic improvement with clearing of the oral (one week), skin (two months) and nail lesions (5 months). After 8 months the drug was stopped and clinical remission persisted for 10 and 7 months respectively. Relapse of oral candidiasis was treated with a short course of ketoconazole (4-16 weeks) leading to complete healing of the lesions. Clinical improvement was not related to an amelioration in lymphocyte transformation. There was no change in the progressive deterioration of the lymphocyte responses to candida antigen which was probably due to persisting candida cell wall components (e.g. mannan).

Administration, Oral↗

[Neuraminidase-producing pneumococci in the pathogenesis of hemolytic-uremic syndrome].

Hemolytic-uremic syndrome (HUS) accompanied by pneumococcal infections forms a characteristic subgroup of HUS. Pneumococcal neuraminidase splits off neuraminic acid from the glycoproteins present on the surface of red cells, thrombocytes and endothelial cells, and thus exposes the hidden Thomsen cryptantigen (T-Ag). The T-Ag can then react with a complement-fixing antibody of the IgM class which is present in all human plasmas after the age of 6 months. Early diagnosis of T-transformation should be attempted. Highly suggestive hints are: pneumonia, hemolytic anemia, reticulocytopenia, difficulties in ABO typing, a positive direct Coombs test and a positive minor cross-match. The definite diagnosis of T-transformation is established with the aid of anti-T agglutinins from Arachis hypogaea, the common peanut. Two children aged 19 and 22 months with pneumonia, Coombs-positive hemolytic anemia, HUS and exposure of the T-Ag on the red cell membrane are described. In one of them, circulating neuraminidase and circulating pneumococcal antigen of serotype 3 were found. In both children exchange transfusions resulted in elimination of circulating neuraminidase and of T-transformed red cells prone to hemolysis.

Female↗

Granulocyte dysfunction in transcobalamin II deficiency responding to leucovorin or hydroxocobalamin-plasma transfusion.

Granulocytes from a 6-year-old boy with congenital transcobalamin II (TC II) deficiency were found to have abnormally low antibacterial activity against Staphylococcus aureus and very low intracellular levels of the cobalamin coenzymes. Transfusion of hydroxocobalamin (OH-Cbl) bound to normal plasma temporarily restored granulocyte bactericidal activity and increased cellular levels of the cobalamin coenzymes. Granulocyte function was also temporarily restored by oral Leucovorin. The defect appeared to be causally related to the patient's TC II deficiency and indirectly to a deficiency of cobalamin and folate coenzymes.

Blood Proteins↗

Necrotising enterocolitis and neuraminidase-producing bacteria.

In 9 out of 26 newborns with necrotising enterocolitis (NEC) exposure of the Thomsen-cryptantigen (T-antigen), probably due to the action of circulating bacterial neuraminidase, was demonstrated on red blood cells. The serological titres seemed to correlate with the clinical course of the disease. Neuraminidase-producing clostridia were isolated in two of the patients. Reaction between the exposed T-antigen and anti-T-agglutinins, normally present in human blood, may lead to difficulties during blood transfusion. This potential transfusion hazard is best avoided by routine T-antigen-tests and by transfusion of packed or washed red blood cells to T-antigen-positive patients.

Antibody Specificity↗

Hemolytic-uremic syndrome associated with neuraminidase-producing microorganisms: treatment by exchange transfusion.

In two infants with pneumonia, Coombs test positive hemolytic anemia and hemolytic uremic syndrome (HUS), exposure of the Thomsen cryptantigen, probably due to the action of circulating neuraminidase, was demonstrated. Reaction between the exposed T-antigen and anti-T agglutinin, normally present in human blood, can lead to difficulties in serological testing and during blood transfusion. The place of exchange transfusions using washed RBC or heparinized whole blood in the management of this subgroup of HUS is discussed.

Antigens↗

[Granulocyte dysfunction in transcobalamin II deficiency].

Granulocytes from a boy with congenital transcobalamin II (TC II) deficiency were found to have abnormally low antibacterial activity against Staphylococcus aureus. Transfusion of normal plasma supplemented with hydroxocobalamin temporarily restored granulocyte bactericidal activity to normal. Granulocyte function was also temporarily restored by oral leucovorin. The defect appears to be causally related to the patient's TC II deficiency and indirectly to an intracellular deficiency of cobalamin and folate coenzymes [1].

Blood Bactericidal Activity↗

Phagocyte dysfunction in common variable immune deficiency.

The history of a 13-year old boy is reported who suffered from frequent bacterial, enteroviral, and protozoal infections since late infancy. A decrease in the serum levels of IgG2, IgG3, IgA, a neutrophil dysfunction, and a partial cellular immune deficiency could be demonstrated. A deficiency of folic acid produced a pancytopenia which enhanced the patient's susceptibility to infections. The combined substitution of gammaglobulins and folic acid only was able to break this vicious cycle.

Adolescent↗