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Biomedical subjects

R Will

Publications and source records attributed to R Will.

At least 37 records · Page 2Linked to original sources

Urinary excretion of the pyridinium cross-links of collagen in systemic lupus erythematosus.

The aim of this study was to measure the urinary excretion of the pyridinium cross-links of collagen and to determine their usefulness as markers of reduced bone mineral density in systemic lupus erythematosus (SLE). All female SLE patients managed at a single centre were invited to participate in a cross-sectional study of urinary pyridinium cross-links excretion (HPLC), bone mineral density (DXA), and SLE-related variables. Ninety-one women with SLE were studied, 35 of whom were postmenopausal. Pyridinoline/Creatinine (Pyd/Cr) and deoxypyridinoline/Cr (Dpd/Cr) levels in postmenopausal women were significantly increased compared with premenopausal values (p = 0.010 and p = 0.004, respectively). Univariate linear regression analysis revealed a significant association of Dpd/Cr with reduced femoral neck and lumbar spine BMD (p = 0.001, p<0.001), and of Pyd/Cr with reduced femoral neck BMD (p = 0.020). In addition, the association of Pyd/Cr with reduced lumbar spine BMD approached significance (p = 0.055). Stepwise multiple linear regression analysis adjusting for other variables confirmed a significant association of Dpd/Cr with reduced lumbar spine BMD (p = 0.006), and a significant association of both Pyd/Cr and Dpd/Cr with postmenopausal status (p = 0.003, p<0.001). It was concluded that in this SLE population, the urinary excretion of Dpd/Cr was a useful marker of reduced BMD at the lumbar spine. Menopausal status was a major predictor of cross-links excretion in SLE.

Absorptiometry, Photon↗

Chemotherapy with praziquantel has the potential to reduce the prevalence of Echinococcus multilocularis in wild foxes (Vulpes vulpes).

In order to evaluate the applicability of anthelminthic treatment of wild foxes (Vulpes vulpes) to limit their infection with Echinococcus multilocularis, bait pellets, each containing 50 mg praziquantel, were repeatedly distributed in an area of 566 km2 where many foxes are infected, in southern Germany. After six baiting campaigns (15-20 baits/km2) over a period of 14 months, the prevalence of the cestode in foxes, initially 32%, had fallen to 4%. The effect was most pronounced in the central part of the treated area, where no positive fox was found in the 2 months before the end of the trial. The study was controlled for other factors that could influence the parasite's prevalence, such as the availability of intermediate hosts. While the potential of this baiting method to remove E. multilocularis from wild hosts has been demonstrated, the question of its long-term efficacy and other unresolved problems have to be addressed by consecutive studies before routine application can be recommended.

Animals↗

Creutzfeldt-Jakob disease in the elderly.

Creutzfeldt-Jakob disease (CJD) is typically described as a pre-senile dementia. However, cases do occur in the elderly and a case of sporadic CJD in an 86-year-old patient is described. The database of the UK national surveillance unit has been studied, and the age-specific incidences for various age groups over the period 1980-93 calculated. Cases of CJD in those over 80 years old have been identified and their clinical characteristics examined. There is no evidence that CJD presents atypically in the elderly, or that large numbers of cases are being missed in the elderly due to poor ascertainment.

Adult↗

Interstitial lung disease in recent onset rheumatoid arthritis.

Interstitial lung disease (ILD) is associated with rheumatoid arthritis (RA); however, the prevalence and natural history are undefined. Our aim was to determine the prevalence of ILD associated with RA using a number of sensitive techniques in patients with joint disease of less than 2-yr duration. Patients who met ARA criteria for RA were recruited from community-based and hospital rheumatologists and assessed using the following measures: clinical, lung physiology, radiology (chest X-ray, high resolution CT [HRCT]), bronchoalveolar lavage (BAL) and 99mTc-DTPA nuclear scan. Thirty-six patients (25 female and 11 male) of joint disease duration of (mean +/- SD) 13.2 +/- 8.6 mo were studied. Abnormalities consistent with ILD were found in one or more investigations in 21 of 36 (58%), which were in lung physiology in 22%, CXR in 6%, HRCT in 33%, BAL in 52%, and 99mTc-DTPA nuclear scan in 15%. Based on the results, they were categorized as having clinically significant ILD (Group 1), abnormalities compatible with ILD, but no clinically significant ILD (Group 2) and no abnormalities compatible with ILD (Group 3). Five of 36 (14%) were in Group 1, 16 of 36 (44%) in Group 2, and 15 of 36 (42%) in Group 3. The only risk factor for the presence of abnormalities compatible with ILD was male gender (p < 0.04, Student's t test). In conclusion, changes consistent with ILD in early RA are frequent. The significance of these changes is being determined in a longitudinal study.

Adult↗

Genetic basis of Creutzfeldt-Jakob disease in the United Kingdom: a systematic analysis of predisposing mutations and allelic variation in the PRNP gene.

Creutzfeldt-Jakob disease (CJD) is a transmissible neurodegenerative disorder characterized by the accumulation of aggregates of a cellular protein, PrP, in the brain. In both human and animals, genetic alterations to the gene encoding PrP (PRNP in human) modulate susceptibility to CJD. The recent epidemic of bovine spongi-form encephalopathy in the UK has raised the possibility of transmission from animal produce to humans. To provide a baseline against which to assess possible risk factors, we have determined the frequencies of predisposing mutations and allelic variants in PRNP and their relative contributions to disease. Systematic PRNP genotype analysis was performed on suspected CJD cases referred to the National Surveillance Unit in the UK over the period 1990-1993. Inspection of 120 candidate cases revealed 67 patients with definite and probable CJD, based on clinical and neuropathological criteria. No PRNP mutations were detected in any of the remaining 53 patients assessed as "non-CJD". A disease-associated mutation in the PRNP gene was identified in nine (13.4%) definite and probable cases of CJD, a reliable estimate of the incidence of PRNP-related inherited CJD based on a prospective epidemiological series. Within the group of sporadic CJD patients (lacking PRNP mutations), we confirmed that the genotype distribution with respect to the common methionine/valine (Met/Val) polymorphism at codon 129 within PRNP was significantly different from the normal Caucasian population. The incidence of Met homozygosity at this site was more than doubled and correlated with increased susceptibility to the development of sporadic CJD. Unlike other recent studies, Val homozygosity was also confirmed to be a significant risk factor in sporadic CJD, with the relative risks for the three genotypes Met/Met: Val/Val:Met/Val being 11:4:1.

Alleles↗

Intracerebral distribution of infectious amyloid protein in spongiform encephalopathy.

We studied the regional distribution of infectious amyloid protein by western immunoblots of brain tissue extracts from 37 patients with different forms of spongiform encephalopathy, i.e., 16 sporadic cases, 18 familial cases with a variety of mutations, and 3 iatrogenic cases. In sporadic and familial Creutzfeldt-Jakob disease, amyloid protein concentrations were usually highest in the frontotemporal regions of the cerebral cortex, whereas iatrogenic Creutzfeldt-Jakob disease and Gerstmann-Sträussler-Scheinker syndrome had as high or higher concentrations in the deep cerebral nuclei and cerebellum. As a group, familial cases had lower amyloid protein concentrations than either sporadic or iatrogenic cases, and fatal familial insomnia patients had the lowest concentrations found in any form of disease. This hierarchy of amyloid protein concentrations corresponds to the experimental transmission rates observed for each form of disease and is consistent with the concept that the protein molecule is an integral component of the infectious agent. Regional amyloid protein pattern analysis of brain and spinal cord may help to distinguish sporadic from environmentally acquired infections, as for example, cases of human disease suspected to have arisen from exposure to sheep or cows infected with scrapie or bovine spongiform encephalopathy.

Aged↗

Neuropathological phenotype and 'prion protein' genotype correlation in sporadic Creutzfeldt-Jakob disease.

A systematic study of 'prion protein' genotype in cases of sporadic Creutzfeldt-Jakob disease showing amyloid plaques staining with anti-prion protein antibody has been performed. This revealed a relative excess of cases with valine at position 129 of the gene's open reading frame. The observation emphasises the importance of this site of common polymorphism in influencing the neuropathological phenotype in human spongiform encephalopathy.

Aged↗

[The occurrence of Babesia divergens in the Freiburg i. Br. district and piroplasmosis prevention trials in cattle].

The occurrence of Babesia divergens in the Freiburg i. Br. district is reported. By interviewing farmers and by serological investigations, three valleys where located in which bovine piroplasmosis occurs since many years. Main objective of this study was to strategically control the vector ticks, Ixodes ricinus with the synthetic pyrethroid flumethrin, in order to prevent clinical piroplasmosis after turnout to pastures in spring, without interfering with protective immunity against Babesia divergens. A total of 200 cattle were used for trials in the period 1990-92. Three pour-on treatments of fortnightly intervals provided best results. Only 4.3% of 163 treated cattle contracted piroplasmosis, in contrast to 19% of 37 untreated cattle. All cattle were sero-negative at the time of turnout. At the end of pasture periods 1990 and 1991 82.4 and 79.6% respectively of treated cattle were sero-positive.

Animals↗

Familial versus sporadic ankylosing spondylitis. Two different diseases?

OBJECTIVE: To define potential differences and the possible contribution of susceptibility or severity genes in familial versus sporadic ankylosing spondylitis (AS). METHODS: Three hundred twenty patients with AS were studied: 160 who had first-degree relatives with AS (familial) and 160 age- and sex-matched controls who had no first-degree relative with the disease (sporadic). Disease expression in the two groups was evaluated using an index of physical, psychological, and social functioning (the Arthritis Impact Measurement Scales [AIMS]) and an assessment of spinal mobility. RESULTS: Familial disease was significantly milder than sporadic disease as assessed by all measures, e.g., spinal mobility score (mean 4.08 versus 4.65, P < 0.038), AIMS overall impact score (mean 2.63 versus 3.59, P = 0.002), AIMS physical activity score (4.19 versus 5.10 [P = 0.004]), AIMS social function score (4.02 versus 4.60, P = 0.023), and AIMS pain score (4.15 versus 5.33, P = 0.002). CONCLUSION: The greater prevalence of AS in at-risk families may be explained by the occurrence of more AS "susceptibility" genes in those families, whereas the more severe disease, seen in patients with sporadic AS, is conferred by the presence of more "severity" genes than "susceptibility" genes.

Adolescent↗

Family theatre: an interdisciplinary strategy for teaching family assessment.

RN students in a 2-year post-diploma programme undertake the integration of the concept of family as client, and the relationship between chronic illness and the work of families. The authors present a collaborative, interdisciplinary strategy to teach family assessment, which combines the resources and talents of nurses and drama students.

Education, Nursing, Baccalaureate↗

Sex ratio in the spondyloarthropathies and its relationship to phenotypic expression, mode of inheritance and age at onset.

OBJECTIVE: To investigate the interrelated effect of phenotypic expression [i.e., primary ankylosing spondylitis (1 degree AS) or disease secondary to psoriasis (Ps) AS or inflammatory bowel disease (IBD)AS], age at onset, sex and inheritance of responsible genes in AS. METHODS: Three studies were performed to evaluate 1949 subjects with AS. Subgroups of the patients were formed for each study depending on disease type (1 degree AS = 1695; Ps AS = 173; IBD AS = 81), nature of inheritance or age at onset of AS symptoms. These groups were further subdivided to assess the effect of sex. RESULTS: The sex ratio of the entire group was 2.6:1 in favor of men. However, IBD AS had an equal sex distribution as does IBD alone. By contrast, Ps, which has an equal sex ratio as a lone event or in association with arthritis, resulted in a male dominance of 4.1:1 when it occurred as Ps AS. Women with IBD AS had a significantly younger onset compared to women with 1 degree AS [mean onset 21.7 years (SD 6.65) vs mean onset 24.4 years (SD 9.79), respectively; p = 0.019]. A younger age at onset was found in women with familial disease [mean 22.2 years (SD 7.55)] compared with the mean onset of sporadic disease in women [24.5 years (SD 10.0); p = 0.0059]. There was a progressive fall in the sex ratio as the age at onset increased (p = 0.053). For example: M:F ratio of < 20 years old was 3:1 compared to 1.8:1 for those with an onset of > 40 years. CONCLUSION: Sex ratio and age at onset are influenced both by each other and such factors as disease type and familial versus sporadic occurrence. These data help provide a predictable pattern of disease in spondyloarthropathy.

Adolescent↗

Comparison of two yttrium-90 regimens in inflammatory and osteoarthropathies.

Two yttrium-90 (90Y) radiosynovectomy procedures were compared. One procedure, performed at the Royal Perth Rehabilitation Hospital (RPRH) required a shorter immobilisation time than that performed at the Sir Charles Gardiner Hospital (SCGH). There were no significant differences in outcome between the two procedures for the groups with inflammatory and osteoarthropathy. Thirty two patients (45 joints) with inflammatory arthropathy were treated (25 with rheumatoid arthritis, three with psoriatic arthritis, two with ankylosing spondylitis, and two with unspecified inflammatory arthropathy) and 40 patients (58 joints) with osteoarthropathy. A separate assessment of local lymph node spread in patients treated by the RPRH showed a minor spread of 90Y in one of 37 joints assessed. A marked improvement in the patient evaluation scores in the inflammatory arthropathy group at three months persisted at 12 months. Good lasting responses were more common in patients with inflammatory arthropathy with a normal joint or early radiological disease. A marked improvement in the pain and evaluation scores occurred at three months in the group with osteoarthropathy but had disappeared by six months after treatment.

Arthritis, Psoriatic↗

Increasing age at presentation for patients with ankylosing spondylitis.

An analysis of the age at first presentation was undertaken in patients with ankylosing spondylitis and mechanical back pain seen at the London Hospital department of rheumatology between 1952 and 1983. There was a significant positive correlation with the calendar year of presentation in the patients with ankylosing spondylitis but a negative correlation in those with mechanical back pain. An increasing age at presentation in ankylosing spondylitis is likely to be due to an increasing age at disease onset--all anticipated biases would act in the opposite direction. This observation in a prospective study supports the findings of other studies using different epidemiological techniques.

Adult↗