Biomedical subjects
Publications and source records attributed to E Kleihauer.
Population genetic studies in Saudi Arabia are performed for EsD, GPT, AcP, ADA, AK, 6-PGD, PGM, C3, Tf, Hp, Gc, Pi, Bf, Hb, ABO-blood groups and Rh-factor, level of the third component of complement and immunoglobulins. The data are compared with reported frequencies in European and African populations.
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A patient with an inborn cellular immunodeficiency syndrome developed a graft-versus-host reaction after a transfusion with packed red cells. This diagnosis was confirmed by skin biopsy and finally proved by tissue typing.
In 35 children with long-standing diabetes mellitus, a significant correlation was found between the hemoglobin A1c (HbA1c)--and the 24-hour urinary glucose excretion. By contrast, 11 newly diagnosed diabetic children had grossly elevated HbA1c-concentrations, but no correlations could be established between the levels of HbA1c and the duration of symptoms, blood glucose, glycosuria, ketonuria and the acid--base status. However, HbA1c and C-peptide were significantly correlated. The elevated HbA1c-concentrations decreased towards normal in all of these 11 children after 2--3 months following adequate therapy. The results suggest that the determination of HbA1c may serve as a valuable metabolic control index in children with long-standing diabetes mellitus, but adds little information in newly diagnosed patients. For the individual diabetic child during the early treatment period, HbA1c may be the index of choice for adequacy of metabolic control.
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19 months after the clinical manifestation of acute lymphoblastic leukaemia, Hodgkin's disease, stage Ia, of the cervical lymphnodes developed in a 10 year old girl during continuous complete remission of leukaemia under chemotherapy. After a regional irradiation and after completing the antileukamic therapy the patient is at present off therapy, healthy and without signs of relapse of both malignant systemic diseases. The coincidence of acute lymphoblastic leukaemia in children with other malignant neoplasias is rare. The expected frequency of second malignancies and the theories concerning oncogenesis are shortly reviewed.
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Thirty patients with severe drug-induced agranulocytosis were admitted to the Ulm University Hospital between January, 1968 and October, 1976. All of 13 already infected patients treated with antimicrobial decontamination in a plastic isolation bed system survived. But nine of 17 patients treated in the open ward died. The cases were not randomised and there was a difference in age between the two groups, but nevertheless the results suggest that antimicrobial decontamination and isolation is superior to treatment in an open ward.
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A new method of membrane protein analysis was used to demonstrate an erythrocyte membrane protein anomaly. Our approach employed electrofocusing linked to sodiumdodecylsulfate polyacrylamide gel electrophoresis. We observed an aberrant protein, isoelectric at approximately pH 4.5 with an apparent molecular weight of approximately 25,000 in membrane preparations from a patient with congenital stomatocytosis. This protein has not been observed in any healthy donor examined to date.
Histologic, immunohistologic and electron microscopic findings in three children with primary immunodeficiencies are reported. Classical X-linked infantile agammaglobulinemia Bruton was present in case 1 (male, aged 16 years), selective cellular immunodeficiency with thrombopenia in case 2 (male, aged 2 1/2 years) and non-lymphopenic severe combined immunodeficiency in case 3 (male, aged 1 3/4 years). At autopsy, all three cases exhibited unusual types of pneumonia. In case 2 a generalized cytomegalovirus infection was present. Case 3 disclosed panmyelopathia and chronic liver lesions due to severe GvH-reaction subsequent to bone marrow transplantation. A detailed morphologic study of the immune system revealed distinct alterations in the thymus, spleen, and lymph nodes and the lymphatic tissues of the gastrointestinal tract characteristic of an immunodeficiency state, either humoral (case 1), cellular (case 2) or combined (case 3).
A patient is presented who manifested the typical clinical and pathological features of congenital immunodeficiency and agranulocytosis (reticular dysgenesia). Treatment under gnotobiotic conditions enabled the measurement of immunogical parameters up to the 17th week of life with the following results: negative skin test, low response to phytohaemagglutinin, weak response in the mixed leukocyte culture and very few E rosettes. Peripheral lymphocytes and lymphocytes in the lymphatic tissues were markedly decreased. Humoral immunoglobulins and plasma cells in the organs were decreased. The in vitro culture of hemopoietic cells showed a diminished content of myelopoietic progenitor cells ("committed stem cells"). It is concluded that the disease may be primarily a defect of stem cells with regard to differentiation in myelopoiesis or lymphopoiesis.
An electrophoretically HbA-like hemoglobin component is produced in increasing amounts during storage in hemolysate preparations from macerated tissue (liver, kidney, spleen) of fetuses. Within twenty four hours after hemolysate preparation the "fast moving" fraction increases up to 40 per cent of total hemoglobin, while the concentration of HbA remains constant (5 - 7%) in hemolysates obtained from peripheral blood of the same donor individuals. By structural studies (fingerprint and aminoacid analysis) the HbA-like component was identified as an artefact of HbF, characterized by the absence of the C-terminal arginine of the alpha chains. From experimental data it is concluded, that the break down product results from a digestion of HbF by carboxypeptidase B, the enzyme being released from the macerated tissues. Analogous to a modification of HbA, i.e. Hb Koelliker (alpha2 minus 141 Arg beta2), the structure of the degradation product of HbF is alpha2 minus 141 Arg gamma2 (HbF Koelliker).
The second occurrence of Hb M Milwaukee is reported in two members of a German family who had cyanosis since early childhood. Contrary to earlier reports, Hb M Milwaukee exhibits a distinct heat instability. It is suggested, that in this family the variant resulted from a new mutation.