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Biomedical subjects

J Manson

Publications and source records attributed to J Manson.

49 records · Page 3Linked to original sources
PubMed1990

Modulation of interleukin 1 beta gene expression using antisense phosphorothioate oligonucleotides.

The production of interleukin 1 beta (IL1 beta) in lipopolysaccharide (LPS) stimulated monocytes was inhibited by 98% using antisense phosphorothioate oligonucleotides complementary to the 5' untranslated and exon 6 regions of IL1 beta mRNA. A sense phosphorothioate oligonucleotide failed to inhibit IL1 beta production. The inhibition of IL1 beta synthesis was not due to reduced cell viability and [35S]methionine incorporation showed that it could not be accounted for by an overall inhibition in protein synthesis. Tumour necrosis factor (TNF) and IL1 alpha production was also inhibited but to a lesser extent than IL1 beta. The use of antisense phosphorothioate oligonucleotides to inhibit IL1 production should enable the complex pathways of IL1 regulation to be elucidated and provide information on the biological role of these cytokines.

Base Sequence↗
PubMed1988

Treatment of infantile phytanic acid storage disease: clinical, biochemical and ultrastructural findings in two children treated for 2 years.

Two patients with infantile phytanic acid storage disease (infantile Refsum disease), one of whom showed the presence of morphologically normal peroxisomes in a liver biopsy, were treated with a low phytanic acid diet for more than 2 years and the effects of treatment on certain clinical, biochemical and ultrastructural parameters were examined. Both patients showed evidence of either an improvement or stabilisation in their clinical condition. Plasma phytanic acid levels decreased to near normal values in approximately 6 weeks after the introduction of the diet; plasma pipecolic acid also declined markedly but the decrease was not so rapid and its level remained abnormal. C26:C22 fatty acid ratios decreased very slowly and even after 2 years the values remained grossly abnormal. Despite the marked reduction of phytanic acid in the liver, there was an increase in the C26:C22 fatty acid ratios and this appeared to be paralleled by an increase in inclusion bodies. Our data suggest that some patients with the infantile form of Refsum disease may show some clinical benefit from dietary management and this is reflected biochemically by decreases in the plasma levels of phytanic acid and pipecolic acid.

Eicosanoic Acids↗
PubMed1988

Congenital blindness, porencephaly, and neonatal thrombocytopenia: a report of four cases.

Four unrelated infants with neonatal thrombocytopenia associated with congenital blindness and porencephaly have been seen over an 18-year period. The association of congenital blindness with neonatal thrombocytopenia has not previously been reported. All children had clinical purpura in the neonatal period; in three cases, thrombocytopenia was confirmed, while in one case, the diagnosis of thrombocytopenia was presumptive; in two cases, there was evidence of circulating maternal serum platelet isoantibodies. Extensive investigation for intrauterine infection was negative in the three cases with confirmed thrombocytopenia. The thrombocytopenia resolved spontaneously after the neonatal period. It is postulated that the porencephalies were the consequence of prenatal cerebrovascular episodes. The etiology of the optic atrophy is unclear. Serial cranial ultrasound investigation is recommended for all neonates with thrombocytopenia, even if neurologically asymptomatic in the neonatal period, and serial prenatal cranial ultrasound investigation is recommended for infants of mothers with a history of having previously had infants with neonatal isoimmune thrombocytopenia.

Adolescent↗
PubMed1986

Intracranial histiocytosis X: a case report.

A case of intracranial histiocytosis X that presented radiologically with thickening of the pituitary stalk is presented. The radiologic findings, including a blush seen on angiography, are described.

Adult↗
PubMed1982

Quantitative immunoassays for diagnosis and carrier detection in cystic fibrosis.

Quantitative immunoprecipitation and immunoradiometric assays have been developed for a protein present in the serum of cystic fibrosis homozygotes, and to a lesser extent in the serum of heterozygotes. When tested on a panel of sera from 14 cystic fibrosis patients, 29 heterozygotes and 23 controls, the immunoprecipitation assay allowed correct assignments to be made on 94% of occasions with one batch of antiserum and 95% with another. With the same panel of sera, the immunoradiometric assay allowed 94% correct assignments. It is suggested that such accuracy is the maximum that can be expected in the present state of knowledge of cystic fibrosis.

Blood Proteins↗
PubMed1978

Serum alphafetoprotein in cystic fibrosis of the pancreas.

Serum alphafetoprotein concentrations were measured by three different types of radioimmunoassay in 30 patients with cystic fibrosis of the pancreas and in 55 controls. The highest value obtained in cystic patient was 10.2 ng/ml and in a control 10.8 ng/ml. These are within published normal limits. Previously reported large increases in serum AFP in patients with cystic fibrosis and in heterozygote carriers have not been confirmed.

Cystic Fibrosis↗
PubMed1978

Giving and getting surgery in Utah: an urban-rural comparison.

Using the Blue Cross/Blue Shield and Medicare records for 1 year in Utah, we examined the pattern of surgical performance for 15 selected procedures. Only 60% of those identifying themselves as general surgeons had their specialty boards. General practitioners performed 24% of the procedures. Urban general practitioners performed proportionately more surgery than did their rural counterparts. There was no clear pattern of difference in fees or length of stay by specialty or board certification status. For most procedures studied, at least a third of the rural patients had their operations in urban hospitals. In only 3% of the cases did an urban physician operate in a rural hospital. The pattern of surgery for rural and urban residents was strikingly similar.

Certification↗
PubMed1977

The effects of thalidomide and two analogues on the regenerating forelimb of the newt.

Oral administration (3 mg/day) of thalidomide during the dedifferentiation and early limb-bud stages of newt forelimb regeneration produced a variety of specific limb deformities. Proximal and preaxial skeletal elements were the most severely malformed, e.g. preaxial hemimelia, severe proximal deformities, and preaxial polydactyly. Likewise, oral, daily doses (3 mg) of the teratogenic analogue, EM12, on days 7 and 8 following bilateral amputation caused the same incidence and type of forelimb abnormalities as did thalidomide. Conversely, the non-teratogenic analogue, EM87, when orally administered (3 mg/day) on days 7 and 8 post-amputation resulted in a low rate of limb deformities, similar in type to those seen in control regenerates. The type of limb deformities observed in the regenerating newt forelimb following thalidomide treatment nearly mimic those seen in the human and monkey syndromes. Therefore, the newt represents a possible model for investigating some of the problems associated with thalidomide teratogenesis.

Amputation, Surgical↗
PubMedDate not supplied

The cellular prion protein binds copper in vivo.

The normal cellular form of prion protein (PrPC) is a precursor to the pathogenic protease-resistant forms (PrPSc) believed to cause scrapie, bovine spongiform encephalopathy (BSE) and Creutzfeldt-Jakob disease. Its amino terminus contains the octapeptide PHGGGWGQ, which is repeated four times and is among the best-preserved regions of mammalian PrPC. Here we show that the amino-terminal domain of PrPC exhibits five to six sites that bind copper (Cu(II)) presented as a glycine chelate. At neutral pH, binding occurs with positive cooperativity, with binding affinity compatible with estimates for extracellular, labile copper. Two lines of independently derived PrPC gene-ablated (Prnp0/0) mice exhibit severe reductions in the copper content of membrane-enriched brain extracts and similar reductions in synaptosomal and endosome-enriched subcellular fractions. Prnp0/0 mice also have altered cellular phenotypes, including a reduction in the activity of copper/zinc superoxide dismutase and altered electrophysiological responses in the presence of excess copper. These findings indicate that PrPC can exist in a Cu-metalloprotein form in vivo.

Animals↗