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Biomedical subjects

R Dopfer

Publications and source records attributed to R Dopfer.

At least 91 records · Page 5Linked to original sources
PubMed1985

[Serum lysozyme level in children with acute leukemia and malignant diseases].

Serum lysozyme activity was measured in samples from 65 children with acute lymphatic and myelogenous leukemia, solid tumors and malignant lymphoma in comparison with 45 healthy children. All children with acute lymphatic leukemia (ALL) had significantly reduced levels of lysozyme before starting therapy compared with a control group (p less than 0,01). Children with ALL in complete remission had lysozyme levels comparable to normal children, while children with ALL in relapse showed pathological low levels again. Children with acute myelogenous leukemia (AML), solid tumors and malignant lymphomas had higher lysozyme concentration before therapy than healthy children. Determination of lysozyme activity in children with acute leukemia and malignant tumors is of value for diagnosis and to control the effect of therapy.

Child↗
PubMed1984

Immotile cilia syndrome: reduced chemotaxis and reduced number of intramembranous particles in granulocytes.

The neutrophil granulocytes of four patients with immotile cilia syndrome were investigated by means of freeze-fracture technique. Whereas most granulocytic functions (adherence, phagocytosis, killing of micro-organisms, reduction of NBT, and chemoluminescence) were in the normal range, chemotaxis of the neutrophils was clearly reduced; their plasma membrane revealed a profound reduction in the density of intramembrane particles. An interrelationship between reduced particle density and defective chemotaxis in neutrophils is assumed, but not yet proven.

Adult↗
PubMed1984

IgG therapy in systemic lupus erythematosus--two case reports.

Two paediatric patients with systemic lupus erythematosus were treated with immunoglobulin G (IgG). In the first case treatment resulted in regression of the most acute symptoms and a long remission was achieved. In the second patient, who was treated during the chronic stage of the disease, there was no significant effect on the course of the SLE.

Azathioprine↗
PubMed1984

In vivo effects of interleukin 2 on lymphocyte subpopulations in a patient with a combined immunodeficiency.

This report describes a clinical trial with Interleukin 2 (IL-2) on a 17-month old male child with combined immunodeficiency (Nezelof's syndrome). IL-2 was prepared from conditioned media of phytohemagglutinin-stimulated leukocytes from buffy coats. The purification of IL-2 involved chromatography on Matrex Blue A sepharose and gel filtration chromatography. The preparation was free of macrophage cytotoxicity factor, macrophage migration inhibition factor and colony-stimulating factor. It contained negligible activity of interferon-gamma. IL-2 activity was adjusted to 1600 U/ml, which corresponds to about 0.8 micrograms homogeneous IL-2/ml. The patient was treated over a 50-day period with a total dose of 20,000 U IL-2, which was injected subcutaneously. IL-2 was well tolerated. Within 3 weeks, the treatment led to a normalization of a lymphocytosis which had prevailed for the previous 3 months. A pronounced eosinophilia also improved but did not reach normal levels. The most striking effect was a normalization of the OKT4+/OKT8+ ratio with a concomitant relative increase in OKT3+ cells in the peripheral blood. No effects were seen on E rosette formation, B cell counts or serum Ig levels. Also NK or ADCC activity remained high, as before the treatment. Infectious episodes and requirement for antibiotic treatment were less frequent during IL-2 therapy. Some effects of IL-2 were transient, e.g., the counts of OKT4+ and OKT3+ cells which returned to pathological values a few weeks after the treatment was discontinued.

Humans↗
PubMed1984

Clinical aspects, diagnosis, and treatment of the Kaufman syndrome (hydrocolpos, hypospadias, polydactyly).

Three girls with the Kaufmann syndrome are reported. In these children the hydrocolpos was not caused by a vaginal atresia but by a stenosis of the vaginal introitus combined with female hypospadias. These children suffered from additional micrognathia. The parents of two of the children suffered from additional micrognathia. The parents of two of the children were related to each other. No such family history could be discovered in the third child. It is possible that this syndrome is autosomal recessive and sexlinked. As the associated malformations may endanger life and therefore necessitate immediate operation, the hydrocolpos should be treated at first conservatively. Later on, corrective operations should be performed.

Abnormalities, Multiple↗
PubMed1983

NK cell function in severe combined immunodeficiency (SCID): evidence of a common T and NK cell defect in some but not all SCID patients.

The immunologic work-up of eight infants with the clinical diagnosis of severe combined immunodeficiency (SCID) was performed with special emphasis on natural killer (NK) cell function and ontogeny. Contrary to previous reports, our study shows that not all SCID patients lack NK activity; some may even express very high NK- and antibody-dependent cellular cytotoxicity (ADCC). The present group of eight SCID infants was homogeneous with respect to normal levels of the purine metabolism enzymes adenosine deaminase (ADA) and purine nucleoside phosphorylase (PNP). They all had low serum Ig levels and were defective for specific antibody formation against BSA and diphtheria toxin (DiT). None of the infants' peripheral blood mononuclear cells (PBMC) proliferated significantly upon in vitro stimulation with PHA, concanavalin A (Con A), pokeweed mitogen (PWM), and irradiated allogeneic lymphocytes. Seven of eight patients, however, responded significantly to mitogenic factors present in a lectin-free interleukin 2 (IL 2) preparation, and two exhibited a positive costimulation as well with simultaneous exposure to IL 2 + Con A. The lymphocyte marker analysis revealed high percentages of OKT10+ cells in seven of eight infants, whereas peripheral T cells (OKT3+) with suppressor/killer (OKT8+) or helper/inducer (OKT4+) phenotypes were abnormally low in all infants with one exception. The PBMC of two patients formed low to normal percentages of E rosettes but expressed no B cell markers (B-/SCID). The six other infants had high percentages of B cells (B+/SCID) but lacked E rosette-forming cells. High NK and ADCC activity was found in the two B-/SCID patients. The B+/SCID infants either totally lacked NK and ADCC function (four of six) or expressed low to normal NK activity together with some T cell markers as revealed by monoclonal antibody staining but not by E rosette formation (two of six). From the data presented, an ontogenic model is proposed that assumes the status of an independent cell lineage in between T cells and monocytes for human NK cells, or that places these cells in close proximity to early differentiation steps of the T cell lineage. In any case, NK cell function clearly constitutes an additional parameter of heterogeneity in the immunologic analysis of SCID.

Adenosine Deaminase↗
PubMed1983

[Combination of a Shwachman syndrome and a complex granulocyte function disorder in a girl].

We report the case of a 1-year-old girl with the typical symptoms of Shwachman syndrome: neutropenia, insufficiency of the exocrine pancreas, and metaphyseal dysostosis. The clinical course is complicated by recurrent infections caused by the neutropenia and an additional defect of granulocyte function. We demonstrate a severe defect of chemotactic activity as well as a disturbance of the intracellular oxidative metabolism leading to defective killing of Staphylococcus aureus and Candida albicans.

Bone Marrow Diseases↗
PubMed1982

Evaluation of infants with acidosis using a perinatal "negative factor" score.

Using Prechtl's concept of optimal conditions as modified by Michaelis et al. [7] we analysed the case records of 50 acidotic newborn infants (umbilical artery pH of 7,250 or less) and of 34 controls. Acidotic infants had a significantly higher negative (or adverse) factor score. There was a correlation between the degree of acidosis and the negative factor score. There were, however, no relations between scores in isolated subgroups of negative factors and fetal acidosis though the same was not true for some combinations of subgroups (such as pre-eclampsia, operative delivery, fetal bradycardia in the second stage of labour).

Acidosis↗
PubMed1982

[Fatal granulocyte function defect in a male infant. Results of in vitro and in vivo stimulation with ascorbic acid as well as in vitro stimulation with levamisol and lithium chloride].

We report about a boy who suffered from repeated bacterial infections starting at the age of 4 weeks. A severe defect of chemotactic activity of the neutrophils, and an additional deficient phagocytosis were discovered. The child died at the age of 3 months from septicemia. Chemotactic activity could be stimulated in vitro by ascorbic acid and levamisole but not by lithium chloride. In vivo, however, the effect of ascorbic acid was minimal and treatment with this vitamin could not prevent the lethal end.

Ascorbic Acid↗
PubMed1981

Hydrometrocolpos, postaxial polydactyly, congenital heart disease, and anomalies of the gastrointestinal and genitourinary tracts: a rare autosomal recessive syndrome.

Nine cases with the hydrometrocolpospolydactyly syndrome (4 males, 5 females) from four unrelated families are presented. Leading symptoms of this rare disorder were hydrocolpos and postaxial polydactyly. Three affected girls had urinary hydrocolpos without vaginal septum or imperforate hymen, one had partial vaginal atresia, and one had no hydrometrocolpos. Glandular hypospadias and prominent scrotal raphe are added to the spectrum of malformations in this disorder in males. The literature is reviewed and problems in genetic counseling in this autosomal recessive disorder are discussed.

Abnormalities, Multiple↗
PubMed1981

[Pulmonary blastoma in a 4 year old girl (author's transl)].

A case of a pulmonary blastoma is presented. The tumor's histological demarcation is difficult and contested. The diagnosis was delivered by two independent pathologists. Because of the malignancy of the tumor we decided on a combined surgical-cytostatic-radiological therapy, which has not yet been done in this way. The patient was symptom-free for 6 months, she then died however, from a brain metastasis.

Carcinoma, Squamous Cell↗
PubMed1980

[Obstetrical and postnatal complications in children of chronically alcoholic mothers (author's transl)].

The histories of mothers addicted to chronic abuse of alcohol always present severe complications during pregnancy and for most of their children during the peri- and postnatal period as well. The question arises to what extent peri- and postnatal complications may influence the clinical aspect of the alcoholembryopathy (AE) in these children. In 35 children with AE all details of their histories could be traced. By using Prechtl's concept of optimal conditions two different populations could be found amongst the children with AE, one with and the other without severe peri- and postnatal complications (exclusively peri- and postnatal asphyxia). No correlation could be found between the severity of AE and perinatal asphyxia. The result suggests reservation in rating peri- and posnatal asphyxia as an always potent factor causing brain damage.

Asphyxia Neonatorum↗