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Biomedical subjects

W H Hitzig

Publications and source records attributed to W H Hitzig.

At least 55 records · Page 3Linked to original sources

[Counting of thrombocytes in a medium-sized clinical routine laboratory. Comparative study of a new electronic counting procedure].

Three platelet counting methods, i.e. the phase contrast microscopic method, the Thrombocounter (Coulter) and Thrombocell 1000 (Contraves), have been analyzed and compared. Critical evaluation of the results showed marked superiority of the electronic systems over microscopic counting regarding reproducibility and time requirement. The simplicity of sample preparation, which requires no hematocrit correction, and the availability of a capillary blood method renders the Thrombocell 1000 superior to the Coulter Thrombocounter.

Adolescent↗

Chronic granulomatous disease: fatal septicemia caused by an unnamed gram-negative bacterium.

A 2-year-old boy with proven X-linked chronic granulomatous disease was placed under continuous co-trimoxazole prophylaxis. He remained free of infection for 4 years. At age 6.25 years, he suddenly developed a fever with no localizing signs and died 16 days later in septic shock. A gram-negative, catalase-positive, halophilic, aerobic bacterium was cultured from blood, bone marrow, and ascitic fluid. This organism could not be identified in microbiological laboratories in Europe and the United States. Its biochemical features indicate that it may belong to a species which has not previously been described.

Bacteria↗

Genetics of immunodeficiency diseases.

Immunodeficiency syndromes in childhood are partly congenital and due to metabolic errors. A majority are inherited, and from this group examples are presented of autosomal-recessive, X-linked, and dominant transmission. Reliable carrier identification and prenatal diagnosis are possible in a minority of these diseases, but clinical immunologists hope that new techniques will soon offer these possibilities to all families with hereditary forms of IDS.

Child↗

[Prognostic value of T-cell markers in juvenile acute lymphatic leukemia].

Of 58 children with acute lymphatic leukemia who had undergone marker studies of blasts, only 6 (10%) had greater than 30 E+ blasts. Of these, 5 had a high WBC and organomegaly, but only 2 suffered a fatal course. Two more have been in CCR for over 5 years despite other risk factors. Patients with less than 30% E+ cells did not differ from common ALL patients clinically or in their response to treatment. However, 3 older children with Hp-positive but E negative blasts had a very high WBC and died without having remission. Only standardized marker studies, treatment and follow-up of a large series of ALL patients will show the importance to be attached to these and other T-cell markers compared with the better-known clinical and hematologic risk factors.

Acid Phosphatase↗

[Preventive immunizations-status in 1980/81].

This ia a brief report on a symposium held during the 16th International Congress of Pediatrics (September 1980) and an account on the consequences to be drawn for the present situation in Switzerland: The vaccination (immunization against small pox) can be suppressed worldwide. Immunizations with attenuated viruses should be encouraged in a greater extent: all children should receive measles vaccine at the age of 18 months; no other than financial reasons can be brought forward against simultaneous application of rubella and mumps vaccine. Pertussis vaccination would be continued, since its suppression in different regions was followed by great epidemic outbreaks of whooping cough. In the new edition of the Swiss "Guidelines on Vaccination" all these considerations are taken into account.

Child↗

Transitory and persistent IgA deficiency. Reevaluation of 19 pediatric patients once found to be deficient in serum IGA.

In 21 out of 5181 serum samples tested between 1968 and 1977 IgA was not detectable. In this subsequent study, 19 individuals could be reinvestigated by clinical and laboratory methods. At reevaluation 26% showed high, 16% normal and 58% subnormal serum IgA. Late maturation of the IgA system therefore occurs frequently. Lack of secretory IgA was proven in 16% and the material used were tears: even in the group with subnormal IgA clinical manifestations are minimal. Development, compensatory mechanisms and criteria for a new definition of selective IgA deficiency are discussed.

Child↗

[Acute myelocytic leukemia in childhood: results of treatment from 1964 to 1979].

From 1964 to 1979, 57 children with acute myelocytic leukemia have been treated at the University Children's Hospital, Zürich. The overall remission rate was 0.60, with a median remission duration of 2 months and survival of 6.4 months. Patients with FAB type M 1 responded best, but longtime survivors were observed from all FAB types. Whereas in the 1960s only 4/19 achieved (short) remission, now, since the introduction of cytosine arabinoside, remissions are observed on 79% but median survival (10 months) is still short compared with acute lymphocytic leukemia. However, 11/38 children treated since 1971 survived for more than 2 years. Four of these relapsed (after less than 23 months of complete remission), while of the remaining seven, 5 have been off treatment for 5 to 77 months. This group includes children of both sexes, all ages, low and high WBS's, with organ infiltrates (excluding CNS and skin), and in whom the time taken to achieve M 1 marrow played no role. These results show that, with combination chemotherapy including cytosin arabinoside, remission can almost always be attained, but prolonged remission can be maintained only in about 1/5 of these children. The problems of maintenance treatment and CNS-prophylaxis need more attention in the future.

Adolescent↗

[Elevation of unsaturated transcobalamin II in autoimmune diseases: study of the process in lupus erythematosis and dermatomyositis].

In an earlier study concerning patients with autoimmune diseases, a more reliable correlation of clinical disease activity was found with raised concentrations of the serum protein transcobalamin II than with several tests now in common use (complement factor C3, antinuclear antibody titer and antinative DNA binding capacity). These findings were subsequently confirmed in long-term observations over 1 to 3 years: the present paper discusses 5 of 7 investigated cases (2 with SLE, 2 with dermatomyositis of changing activity, and 1 with an erroneous diagnosis of SLE) in detail.

Adolescent↗

[New possibilities for immunoglobulin substitution in antibody deficiency syndrome].

The effect of an unmodified immunoglobulin for intravenous substitution in patients with antibody deficiency syndrome (ADS) has been studied. Over 100 doses of Sandoglobulin were administered to ADS patients without any sign of anaphylactoid reaction. There is no upper limit on the quantity infused. Peak values and basal values can be calculated and therapy can be adjusted accordingly. The halflife of the preparation equals that of native IgG in a healthy person. Comparing days with temperature above 38 degrees C (100.4 degrees F), days under antibiotics, and absence from work during equal periods with and without Sandoglobulin, the beneficial effect of this preparation is obvious. Sandoglobulin can be recommended for longterm therapy in ADS.

Antibodies↗

[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↗

[Prognostic changes in juvenile abdominal non-Hodgkin's lympomas (NHL)].

From 1964-1978 primary non-Hodgkin's lymphoma was diagnosed and treated in 15 children (13 boys and 2 girls). In the 10 patients treated up to 1974, median survival was only 4 months (the longest being 16 months). Since 1974 all such patients have been put on high-dose combined chemotherapy as early as possible. Of 5 children thus treated, 3 have survived for 60, 26 and 11 months respectively and are still alive disease-free. The other two died after 4 1/2 and 5 1/2 months respectively: both had very widespread disease at diagnosis and chemotherapy was delayed by postoperative complications. The 4 most recent tumors have been classified immunologically: 3 were of the B-cell type and exhibited the classic "Burkitt-type" morphology. Two of these patients are alive and well. Although so small a patient material allows no firm conclusions, the authors feel that early intensive chemotherapy may be curative even in this poor-prognosis group of intraabdominal non-Hodgkin's lymphoma.

Adolescent↗

[Therapeutic use of immunoglobulins in children (author's transl)].

The indications for immunoglobulin therapy in childhood have been well established for many years: prophylaxis is effective with single small doses, a full substitution needs multiple large applications. All other recommendations are ill-defined and result from wishful thinking rather than from solid clinical data. New is the possibility of safe i. v. Ig-application which could be realized through appropriate modification of gammaglobulin preparations. Some properties of the commercially available products are summarized. Our own experience with one of them is encouraging: as a matter of fact, fully effective gammaglobulin substitution is now possible in patients with antibody deficiency syndrome.

Child↗

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↗

Bone marrow participates in the biosynthesis of human transcobalamin II.

Studies concerning the site of synthesis of the vitamin B12 binding serum protein, transcobalamin II, have been done in various mammalian animals. The actual site of biosynthesis in man has not yet been defined. The finding that bone marrow derived cells release apo-transcobalamin II in the mouse led us to examine the genetic patterns of transcobalamin II in man, both before and after marrow transplantation. A gradual but incomplete transformation of the recipient's transcobalamin II type into donor's type, corresponding to 75% or less of the total activity, was registered in 4 cases. Surprisingly, persistent host-type TC II, in spite of different donor type, was observed in 4 further marrow recipients. We conclude that hematopoietic cells transferred with the transplanted marrow participate in the biosynthesis of human transcobalamin II.

Anemia, Aplastic↗