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Biomedical subjects

M Beck

Publications and source records attributed to M Beck.

At least 289 records · Page 16Linked to original sources
PubMed1987

Morquio's disease type B (beta-galactosidase deficiency) in three siblings.

The clinical and biochemical findings in 3 siblings with Morquio's disease type B (mucopolysaccharidosis (MPS) IV B) are presented. Their phenotype is characterised by short trunk dwarfism with kyphoscoliosis and thoracic deformity. Radiographic findings include general platyspondyly, dysplasia of the pelvis and epiphyseal abnormalities. The patients are of normal intelligence. In the urine of all 3 affected children abnormal oligosaccharide excretion was found by thin-layer chromatography and in 1 of them keratosulphaturia was detected. The clinical diagnosis was confirmed biochemically by demonstration of a profound deficiency of beta-galactosidase activity in cultured fibroblasts. The clinical picture is compared with that of other cases in the literature and the possible molecular basis of the different phenotypes of beta-galactosidase deficiency (variants of monosialo-ganglioside-1 (GM1)-gangliosidosis, Morquio's disease type B) is discussed.

Adolescent↗
PubMed1987

Central cholecystokinin octapeptide reduces glucose utilization in subcortical but not cortical rat brain.

Cholecystokinin octapeptide was administered intraventricularly to laboratory rats in a behaviorally active dose. Quantitative regional glucose utilization was assessed as a measure of neuronal activity using [14C]2-deoxyglucose. CCK-8 reduced glucose utilization in selected subcortical nuclei, but left most areas of cortex unaffected. The subcortical nuclei were chiefly extrapyramidal systems. The diminutive pharmacologic action on cortical neuronal activity implies CCK-8 may act here as a neuronal modulator, in concert with other central transmitters, rather than having prominent singular actions.

Animals↗
PubMed1987

Reactivation of herpes simplex virus is associated with production of a low molecular weight factor that inhibits lymphokine activity in vitro.

Peripheral blood mononuclear cells obtained during recrudescent Herpes simplex virus (HSV) infection and stimulated with UV-inactivated viral antigen (UV-HSV) for 24 hr produced a low molecular weight (dialyzable) factor that inhibited lymphokine activity. This factor prevented the expression of leukocyte inhibitory factor (LIF) activity, but not its production. It was not made in UV-HSV-stimulated cultures grown in presence of 2 X 10(-6) M indomethacin nor in cultures of peripheral blood mononuclear cells obtained during convalescence or quiescence (greater than 4 days from onset of clinical symptoms) or from seropositive controls without a history of recurrent HSV disease. Dialyzable inhibitory factor production required OKM1+, OKT8+, and OKIa+ cells as determined by complement-mediated lysis with monoclonal antibody. Dialyzable inhibitory factor activity was associated with a trypsin-sensitive 8.2 K fraction as determined by Sephadex chromatography followed by sodium dodecyl sulfate-acrylamide gel electrophoresis.

Antigens, Viral↗
PubMed1987

Stress-related immune suppression: health implications.

This study used a year-long prospective design to assess linkages among distress, immunity, and illness. Serial blood samples were collected from 40 first-year medical students at the first, third, and fifth examination periods, as well as 1 month before each. There were significant decrements in the production of gamma-interferon by concanavalin A-stimulated lymphocytes obtained at the time of examinations. Antibody titers to Epstein-Barr virus (EBV) increased during examination periods, suggesting reactivation of latent EBV and therefore poorer cellular immune control of latent virus. We obtained data that suggest that T-cell killing by memory T lymphocytes of EBV transformed autologous B lymphocytes also declined during examination periods. The activity of a lymphokine, leukocyte migration inhibition factor, normally suppressed during recrudescence of herpes simplex virus type 2 infections, was altered during examination periods and an increase in both plasma and intracellular levels of cyclic AMP associated with examination stress was observed. An increase in the incidence of self-reported symptoms of infectious illness was also associated with examination periods. The data support the linkage between stress-related immunosuppression and health.

Adult↗
PubMed1987

[Not Available].

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Congresses as Topic↗
PubMed1986

A prospective study of intrauterine insemination of processed sperm from men with oligoasthenospermia in superovulated women.

The effectiveness of intrauterine insemination (IUI) was compared with that of intracervical insemination (ICI) in 49 infertile couples, in whom the major cause for infertility was oligoasthenospermia. All women had ovulation stimulated with either a clomiphene citrate (CC)-human gonadotropin combination or human gonadotropins alone. The ovulatory dose of human chorionic gonadotropin (hCG) was given after adequate estradiol levels were reached. The timing of inseminations was standardized--IUI was 28 hours after hCG and ICI was immediately after hCG administration. Only one insemination per month was performed with either IUI or ICI. The first treatment cycle was assigned randomly to be either IUI or ICI, and subsequent inseminations were alternated. A total of 182 cycles were completed, with 96 IUIs and 86 ICIs. Pregnancy occurred in eight patients, seven with IUI (14.3%) and one with ICI (2.0%); the difference is significant at P less than 0.05. The pregnancy rate per treatment cycle was 7.3% versus 1.2% (P less than 0.001). This study supports the use of IUI with processed sperm in the treatment of infertility due to oligoasthenospermia.

Adult↗
PubMed1986

Heterogeneity of Morquio disease.

Further clinical heterogeneity of Morquio disease, mucopolysaccharidosis IV (MPS IV), is delineated by the observation of a 30-year-old man with unusually mild clinical manifestations. He is 156 cm tall, has comparatively mild skeletal abnormalities and fine corneal deposits. Keratosulfaturia is absent. N-Acetylgalactosamine-6-sulfate (GalNAc-6-S) sulfatase (E.C. 3.1.6.-) was markedly reduced in his fibroblasts. The residual enzyme activity exhibited a pH profile comparable to that of patients with the "classical" form of the disorder. From our observation and a review of the literature it is concluded that Morquio disease can be divided in several subgroups: besides the severe ("classical") type A there exist an intermediate and a mild form that are also caused by a GalNAc-6-S sulfatase deficiency. A late-onset variant of Morquio disease, which is due to a deficiency of beta-galactosidase, has been classified as type B. In addition, patients with mild manifestation of the disease and normal activities in fibroblasts of GalNAc-6-S sulfatase and beta-galactosidase have been observed (type C). The genetic nature of the broad clinical variability of Morquio disease is incompletely understood: it is partially caused by different enzyme defects. Other factors thought to influence the clinical expression include the pH profile of the residual enzyme activity and an additional neuraminidase defect.

Adult↗
PubMed1986

Treatment of nickel dermatitis with Trientine.

23 nickel-sensitive patients with hand eczema were treated with Trientine 300 mg daily and a placebo in a double-blind, crossover trial. No significant improvement occurred in the hand eczema. A surprising finding was that there was no detectable increase in urinary nickel excretion, in contrast to animal studies.

Adult↗
PubMed1986

Immune response to Edmonston-Zagreb measles virus strain in monovalent and combined MMR vaccine.

The live Edmonston-Zagreb measles vaccine is currently produced either on chick-embryo fibroblasts (CEF) or on human diploid cells (HDC). Its stability meets the WHO requirements. Since the vaccine licensure in 1967 the Edmonston-Zagreb measles virus strain has been administered to over 20 million vaccinees either as a monovalent vaccine or as a component of the combined MR, MMR and MM vaccines. Immunogenicity studies have shown the persistence of the elicited HI antibody for a minimum of 16 years. In children aged 13-17 months a greater than 95% seroconversion was recorded after subcutaneous administration both CEF and HDC vaccines. The vaccine has also been shown to be highly immunogenic when administered intranasally. In 6-12 month old infants the Edmonston-Zagreb monovalent vaccine elicited a 100% HI antibody response after both s.c. and i.n. administration. The GMT of antibody 42 days after vaccination was significantly higher in those given HDC vaccine, regardless of the age of the vaccinees or the route of immunization.

Administration, Intranasal↗
PubMed1985

Intralysosomal formation and metabolic fate of N-acetylglucosamine 6-sulfate from keratan sulfate.

The physiological relevance of the ability of beta-N-acetylhexosaminidase A to liberate N-acetylglucosamine 6-sulfate from polymeric keratan sulfate was investigated. Upon intravenous injection into rats of [35S]sulfate-labeled proteokeratan sulfate up to 25% of the radioactivity excreted with the urine were identified as N-acetyl-glucosamine 6-sulfate. Within 24 h, however, excretion of inorganic sulfate rose at the expense of the sulfated monosaccharide. Upon incubation in vitro of liver lysosomes from rats treated with proteokeratan sulfate, inorganic sulfate and minor amounts of sulfated monosaccharide were found in the incubation fluid. Cultured rat peritoneal macrophages ingested proteokeratan sulfate with a clearance rate of 6-9 micrograms X h-1 X mg cell protein-1 and degraded it rapidly. Inorganic sulfate but not N-acetylglucosamine 6-sulfate was delivered to the culture medium. During a chase period the amount of intracellular N-acetylglucosamine 6-sulfate fell, and a corresponding amount of sulfate could be found extracellularly. Significant amount of N-acetylglucosamine 6-sulfate were only found in the culture medium when the cells were challenged with zymosan. These results suggest that N-acetylglucosamine 6-sulfate is a physiological intermediate during the degradation of keratan sulfate, but is usually hydrolyzed intralysosomally by N-acetylglucosamine-6-sulfate sulfatase. Genetic deficiency of the sulfatase in humans therefore results in excessive excretion of the sulfated amino sugar but not of keratan sulfate.

Acetylglucosamine↗