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Biomedical subjects

W Jost

Publications and source records attributed to W Jost.

At least 19 recordsLinked to original sources
PubMed1997

Autosomal recessive lateralization and midline defects: blastogenesis recessive 1.

In this report, we present 2 sibships in which midline and lateralization anomalies are demonstrated. Because midline and lateralization processes are early embryological events, we suggest calling this sequence Blastogenesis Recessive 1 (BGR1). Since connexin 43 gene mutations were demonstrated in some polyasplenia patients and according to connexin 43 temporospatial tissue expression, we hypothesize that this gene could bear mutations responsible for the anomalies reported in these two sibships.

Abnormalities, Multiple↗
PubMed1996

Arrested cerebral adrenoleukodystrophy: a clinical and proton magnetic resonance spectroscopy study in three patients.

We report three unrelated boys with X-linked adrenoleukodystrophy with onset of typical neurological symptoms of cerebral adrenoleukodystrophy between the age of 7 and 11 years. In contrast to the expected rapid progression, these patients showed an apparent arrest of initial neurological deterioration for subsequent periods of 5-12 years. Repeated neuroimaging revealed no progression of demyelination. Despite regional variability of demyelination, proton magnetic resonance spectroscopy revealed a specific metabolic pattern in all patients, with only moderate reduction of N-acetylaspartate, normal or reduced choline-containing compounds, normal or enhanced myo-inositol and no detectable lactate, which differs from findings in progressive cerebral adrenoleukodystrophy which usually exhibits a severe reduction of N-acetylaspartate and marked increases of choline-containing compounds, myo-inositol, and lactate. The ability to identify this newly described subgroup of patients with cerebral adrenoleukodystrophy is important for medical advice and planning of therapy.

Aspartic Acid↗
PubMed1996

[Age-dependent MR imaging of the tibia in children up to 2 years old. The findings in children without bone marrow-relevant diseases or therapies].

PURPOSE: Since the beginning of bone marrow conversion presents with substantial differences as shown by anatomical or magnetic resonance studies, the purpose of this study was to demonstrate via MRI an age-dependent bone marrow conversion of the tibia in children of up to two years of age. METHODS: We studied the bone marrows of the tibia in 24 children ranging from one month to two years by means of MRI. T1-weighted SE-sequences were used. Children who suffered from diseases affecting the bone marrow were excluded. A retrospective analysis of the MR images was performed. RESULT: A gradual increase of signal intensity could be demonstrated in the epiphyses and the diaphysis of the tibia beginning shortly after birth. During the first two years of life a further increase of signal intensity could be observed in these regions, progressing through metaphyses up to the growth plates. CONCLUSION: First signs of bone marrow conversion can be detected in the tibia shortly after birth. Bone marrow infiltration in the tibia is expected to be recognised by MRI from this time onward.

Aging↗
PubMed1994

[The clinic as profit center and management with reference figures].

Financial responsibility is one of the important aspects of hospital management. At the hospital of Uster a trial with a profit center concept was started in October 1991. This concept included development of management tools such as reference figures to check costs, returns and performance. Thus it was possible to obtain an equilibrium between personnel costs and performance within 6 months. In the first half of 1993 the budgeted performance figures for 1993 have so far been reached. Although a complete implementation of a profit center concept in a public hospital does not seem possible, a profit center-like structure helps to obtain financial control and enables the hospital management to readily adapt to changing conditions.

Budgets↗
PubMed1992

[Neurocysticercosis in an 8-year-old girl].

We report about an 8-year-old girl with signs of elevated intracranial pressure. X-ray, CT and MRT scan showed an inner hydrocephalus as well as an intracranial calcification. By western blot analysis a Taenia solium cysticercosis could be diagnosed. In spite of spontaneous remission of the symptoms we treated with Praziquantel. Resection of the cyst was not necessary. Diagnostic and therapeutic problems of cysticercosis are discussed.

Animals↗
PubMed1991

Amino acid sequence of an extracellular, phosphate-starvation-induced ribonuclease from cultured tomato (Lycopersicon esculentum) cells.

The primary structure of an extracellular ribonuclease (RNase LE) from Pi-depleted media of cultured cells of Lycopersicon esculentum L. cv. Lukullus has been determined. This was carried out by analysis of peptides isolated after enzymatic and chemical cleavage of the reduced and S-ethylpyridylated protein. RNase LE consists of 205 amino acid residues and has a molecular mass of 22,666 Da and an isoelectric point of 4.24. The enzyme contains 10 half-cystines. There are no potential N-glycosylation sites in the sequence. The sequence of RNase LE is homologous with those of self-incompatibility proteins of several higher plant species and with those of a number of fungal RNases. The sequence similarity with the family of self-incompatibility proteins is greater than with the fungal RNases, suggesting that the self-incompatibility proteins arose from ancestral RNase by gene duplication after the divergence of higher plants and fungi. Two pentapeptide sequences, i.e. HGLWP and KHGTC (or KHGSC), are present at identical positions in all the aligned proteins, suggesting that they contribute to the active site.

Amino Acid Sequence↗
PubMed1991

[Polyvalent immunoglobulins in prevention of viral infections in children with neoplastic diseases treated with immunosuppressive therapy].

31 children with leukemias, lymphomas and solid tumors treated by polychemotherapy received prophylactic i.v. polyvalent immunoglobulins during 48 hours after exposion with varicella-zoster-virus, rubella-virus or mumps-virus. None of the 23 children with immunity against the exposed virus developed the infection. 8 children had no antibodies against the exposed virus. The prophylaxis with the polyvalent immunoglobulin protected them against chickenpox (6 children) and rubella (2 children). All children showed an elevation of the IgG level in plasma and the antibody titers against measles-, mumps-, rubella- and varicella-zoster-virus after application of the immunoglobulin. A study with a larger number of patients is necessary to confirm our results, that polyvalent immunoglobulins with high titers against exposed viruses are effective in prevention of this diseases in immunocompromised children.

Adolescent↗
PubMed1991

[Subjectively experienced anxiety as an aspect of coping with illness in children with malignant diseases].

The present paper focuses the perceived anxiety of pediatric oncologic patients (N = 30) at the time of medical treatment. Comparison with a control group of not chronically ill patients (N = 20) results: Children and adolescents with oncologic diseases are reporting significant less anxiety; this is relatively independent of the asked objects and situations. The invasiveness of treatment (applied chemotherapy or not) does not show any influence on the amount of perceived anxiety of oncological patients. Based on current empirical and conceptual findings of coping with serious illnesses the self reported diminished anxiety of pediatric oncologic patients is termed intraphysical "denial" coping mode. The issue of adaptivity is discussed. Finally, initial consequences for psychosocial care of pediatric oncologic patients are described.

Adaptation, Psychological↗
PubMed1990

[Intraspinal, extradural hemorrhage in a 7-year-old boy with hemophilia B].

A 7-year-old boy with severe hemophilia B suffered an intraspinal extradural hemorrhage without preceding trauma. Since the age of 3 years the patient is HIV-1 positive. The actual hemorrhage caused a strong pain in the thoracolumbar area without signs of spinal cord compression. It was diagnosed by CT-scan. By early high dose factor-IX substitution the hemorrhage was stopped and no neurological complications occurred. The further substitution regime is described.

Child↗
PubMed1990

[Status of plasmapheresis and cyclosporin A in the treatment of systemic lupus erythematosus].

We report the history of 2 teenagers suffering from systemic lupus erythematosus for more than 5 years. Both of them were treated with total plasma exchange (TPE) and Ciclosporin A. They responded well to this therapy and achieved remissions. Especially the 2nd patient showed a dramatic clinical improvement after a relapse caused by sun exposure. Both treatment modalities are discussed and the following conclusions are drawn: TPE is an important therapeutic element in treating patients with SLE, whereas Ciclosporin A is not recommended for therapy of first choice. Further prospective and controlled studies have to show, if there is a benefit of this therapy, especially in childhood.

Adolescent↗
PubMed1983

A modified high-performance thin-layer plate for the separation of purines and pyrimidines.

Several ways of using the recently developed high-performance thin-layer chromatography (HPTLC) precoated plate NH2 F 254s to separate purines and pyrimidines are described. This precoated plate is coated with silica gel 60 which has been chemically modified with alkylamino groups. In view of the chemical properties of the functional groups bonded to the silica gel matrix, the HPTLC precoated plate NH2 F 254s can be considered to be a weak basic ion-exchange plate. In aqueous eluants the substances are separated principally according to charge differences. The HPTLC precoated plate NH2 F 254s can, however, also be used to separate uncharged, polar compounds with organic solvents. Examples of separations and chromatograms for its use in both aqueous and organic eluants are given.

Chromatography, Thin Layer↗