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Biomedical subjects

G S Fell

Publications and source records attributed to G S Fell.

At least 19 recordsLinked to original sources
PubMed1992

DNA-based presymptomatic diagnosis of Wilson disease.

Investigation using DNA markers in a family with Wilson disease revealed that an apparently normal child of 10 years of age with non-diagnostic copper biochemistry had the disease. The procedure used linked restriction fragment length polymorphic markers. Demonstration of increased liver copper concentration from a liver biopsy confirmed the diagnosis and the child was started on chelation therapy. Two other asymptomatic siblings were shown, using the same techniques, not to have the disease. Similar analysis was carried out on another family with just one index case.

Chromosomes, Human, Pair 13↗
PubMed1992

Autonomous hyperparathyroidism not associated with increased aluminum absorption.

A single 6-g dose of aluminum hydroxide was given to eight patients on regular hemodialysis before and 3 days after parathyroidectomy (PTX). Serum aluminum levels were measured before and after 5 h after ingestion of aluminum hydroxide to examine indirectly the effects on absorption of aluminum from the gastrointestinal tract of both high and low parathyroid hormone (PTH). Plasma PTH levels fell significantly from 2,724 +/- 1,830 ng/l (mean +/- 1 SD) before PTX to 352 +/- 63 ng/l after PTX. However, no significant change was detected in serum aluminum levels before or after PTX (1.45 +/- 0.96 to 1.92 +/- 0.88 mumol/l before PTX; 2.02 +/- 1.41 to 2.89 +/- 1.19 mol/l after). These findings indicate that in patients with chronic renal failure, high plasma levels of PTH are not associated with significant increases in serum aluminum concentrations after ingestion of a large quantity of aluminum hydroxide. Parathyroid hormone may not enhance intestinal absorption of aluminum in humans.

Adult↗
PubMed1991

Ranitidine suppresses aluminium absorption in man.

1. Intragastric pH monitoring was performed before and after the single-blind administration of ranitidine or placebo (saline) in eight healthy subjects and four patients with end-stage renal disease who were on regular haemodialysis. 2. The subjects were studied on two occasions and were given aluminum hydroxide (1185 mg) orally 90 min after the administration of ranitidine or saline. 3. Plasma aluminum concentrations and, in normal men, urinary excretion of aluminum were monitored before and after the oral aluminum load. 4. Intragastric pH increased significantly with ranitidine but not with placebo (P less than 0.001). Urinary aluminum excretion increased significantly after the administration of aluminum hydroxide during the placebo phase (P less than 0.001) but not during the ranitidine phase. Plasma aluminium concentrations were higher in the patients with renal failure than in the normal subjects (P less than 0.05), but were unchanged in both groups after the oral aluminium load. 5. This study shows that gastric pH is an important factor in the modulation of aluminum absorption in man, and indicates that reducing gastric acid secretion with ranitidine may reduce the toxicity of orally administered aluminium compounds.

Adult↗
PubMed1991

Aluminium uptake by intestinal cells: effect of iron status and precomplexation.

The investigation of different aluminium-binding agents and metabolic conditions that might act as critical factors in modulating aluminium absorption could yield valuable information in the understanding of aluminium gastrointestinal absorption. We evaluated the effect of iron depletion in aluminium uptake and the role of transferrin and citrate in aluminium incorporation by the intestinal epithelial cell line RIE1. Both complex, aluminium-citrate and aluminium-transferrin were prepared in a molar ratio of 2:1; both facilitated the aluminium uptake although a greater aluminium incorporation was found when aluminium was administered as aluminium-transferrin. This difference became even greater in the iron-depleted cells (normal cells vs iron-depleted cells): 7.7 +/- 1.4 versus 30 +/- 5.6 ng Al/micrograms DNA (P less than 0.05). From these and previous data it is possible to speculate that iron status could modulate the intestinal uptake of aluminium and that both transferrin and citrate would act as effective carriers in the incorporation of aluminium into mucosal cells.

Aluminum↗
PubMed1991

Wilson's disease in Scotland.

The prevalence and clinical features of Wilson's disease in Scotland were investigated. Thirty three cases were identified but adequate information was available on only 28. In 1989, the prevalence rate was 4 per million. Ten patients with a mean (SEM) age of 18 (1.9) years presented with neurological symptoms, 12 patients aged 14 (1.7) years presented with hepatic symptoms, and six patients aged 12 (0.9) years were asymptomatic siblings of patients with Wilson's disease. Nine (56%) of the 16 patients who underwent liver biopsy on presentation were found to have cirrhosis. Penicillamine treatment was stopped in nine patients because of: abnormal peripheral blood count (6), rash (2), and patient's own choice (1). Nineteen patients were alive in 1989 -12 were well, one had chronic liver failure, four chronic neurological disabilities, and two had both chronic liver failure and neurological disabilities. Twelve patients died from: complications of chronic liver failure (2), acute liver failure (4), pneumonia associated with immobility (4), and other causes (2). Several patients who died had received incomplete medical supervision.

Adolescent↗
PubMed1991

Comparison of inorganic elements from autopsy tissue of young and elderly subjects.

Concentrations of nine inorganic elements in autopsy samples of kidney, liver, heart and skeletal muscle from 33 aged subjects, mean age 80 years, were compared with concentrations in similar samples from 12 young healthy accident victims, mean age 29 years. Tissue samples were removed within 48 hours of death and dry weight was used as a frame of reference. Potassium and magnesium were significantly lower in muscle (P less than 0.001) and heart tissue (P less than 0.001) of elderly subjects. There was evidence of accumulation of zinc in liver tissue of the elderly (P less than 0.01). Copper was significantly lower in the heart tissue of elderly subjects (P less than 0.001) whereas manganese was lower in both the heart (P less than 0.01) and the kidney tissue (P less than 0.001) of the elderly. Selenium concentrations were lower in the kidney (P less than 0.001) of the elderly. Elderly heart tissue appeared to accumulate chromium (P = 0.007) and aluminium (P less than 0.001). No significant differences were found for iron concentrations between these two populations. These major differences in tissue element concentrations between young and aged subjects merit further attention.

Age Factors↗
PubMed1990

The effects of intraoperative hypothermia and cardiopulmonary bypass on trace metals and their protein binding ratios.

The ability of intraoperative hypothermia to modify the metabolic response to cardiopulmonary bypass (CPB) was assessed by serial alterations in iron, zinc and copper, and in their molar binding ratios to their respective transport proteins, in 20 male patients under-going elective coronary artery surgery and randomised to an operative blood temperature of 28 degrees C or 20 degrees C. Decreases in serum iron and zinc concentrations, typical of the acute phase response, were preceded by early rises. Significant alterations in the metal: protein molar binding ratios preceded significant changes in the serum concentrations of the metals and occurred earliest in the zinc: albumin binding ratio, which was apparent by the time of skin incision. An intraoperative temperature of 20 degrees C modified iron and zinc concentrations and their protein binding ratios during surgery but not in the post-operative period. These early changes in trace metals and their protein binding ratios are a simple and inexpensive method of quantitating the response to surgical injury and may be useful in assessing new interventions in cardiopulmonary bypass. An awareness of the trace element response to surgical injury is essential to avoid misdiagnoses of iron deficiency anaemia or zinc deficiency.

Adult↗
PubMed1990

Wilson's disease presenting as acute fulminant hepatic failure.

A fatal case of fulminant hepatic failure in an adolescent is described. Post-mortem examination revealed the cause to be Wilson's Disease. This rare presentation is characterised by apparently atypical changes in copper metabolism. Wilson's Disease should be a differential diagnosis of any adolescent presenting in liver failure.

Adolescent↗
PubMed1989

Plasma albumin and haemodilution: the problem of interpretation in sequential studies.

The ability of four formulae (3 based on packed cell volume and one on alpha-2 macroglobulin) to correct for postoperative dilutional changes in plasma albumin was assessed in 4 patients undergoing coronary artery surgery. Cardiopulmonary bypass is a stringent test of the efficacy of the formulae to correct for haemodilution as a large volume of fluid is added to the circulation in a relatively short period of time. Plasma albumin was measured 8 times in the perioperative period in the 4 patients and the clinical situation was mimicked in vitro by adding various volumes of diluent to whole blood. None of the formulae consistently corrected for predicted changes in plasma albumin concentration in vivo; two of the formulae corrected for in vitro changes in plasma albumin but the same formulae produced considerable over-correction when applied in vivo. The problem of correcting for perioperative dilutional changes in plasma albumin is discussed.

Coronary Artery Bypass↗
PubMed1989

Determination of nine inorganic elements in human autopsy tissue.

A survey of nine elements of accepted clinical relevance has been performed on tissue sets comprising of kidney, liver, heart and muscle taken at autopsy from healthy individuals killed as the result of an accident. The concentrations are reported using tissue wet weight, dry weight and nitrogen content as frames of reference. This has allowed direct comparisons to be made with other studies without resort to the use of conversions, using average factors, between one frame of reference and another. Such conversions are shown to be unreliable and may generate spurious differences. The results obtained are largely consistent with those reported from studies in other parts of the world.

Adolescent↗
PubMed1989

Changes in iron, zinc, and copper concentrations in serum and in their binding to transport proteins after cholecystectomy and cardiac surgery.

Concentrations of iron, zinc, and copper and of their respective transport proteins transferrin, albumin, and ceruloplasmin were measured in serum after elective cholecystectomy and cardiac surgery. The pattern of changes in the concentrations of iron, zinc, and copper was reproducible, with an early increase in serum iron and zinc, then a decrease in the concentrations as these metals are dissociated from their serum transport proteins. Zinc and iron concentrations change before the increase in C-reactive protein, which begins 8 h after incision, whereas the copper concentration in the serum remains constant in the early postoperative period. Quantitative and kinetic differences were observed in both the trace metal and protein changes after cardiac surgery and cholecystectomy. These studies indicate the complexity of interpreting changes in trace elements in serum after surgery.

Adult↗
PubMed1988

Factors influencing lead concentrations in shed deciduous teeth.

Data collected for the Edinburgh Lead Study have been used to investigate lead concentrations in children's naturally shed deciduous teeth. A within-child multiple-regression analysis has shown that the upper jaw has a higher concentration of lead than the lower, and that there is a gradient of decreasing concentration from the front to the back of the mouth. Even after the effects of jaw and tooth type have been allowed for, the concentration is still found to be negatively correlated with the weight of the tooth and with the age at which the tooth was shed. No statistically significant effects could be attributed to caries, fillings, or the incomplete resorption of roots. A single-valued index of tooth lead has been derived for each child, taking into account the fact that children gave different types of teeth.

Age Factors↗
PubMed1987

Aluminium bone disease in patients receiving plasma exchange with contaminated albumin.

Aluminium balance studies were carried out on eight patients with various immunological disorders who were receiving plasma exchange with albumin solutions known to be contaminated with aluminium. Four patients with impaired renal function (creatinine clearance less than 50 ml/min) retained between 60% and 74% of the aluminium infused during a single plasma exchange. Transiliac bone biopsy specimens from three patients in this group had a high content of aluminium and showed histological evidence of current or previous bone disease related to aluminium. Two of these patients suffered intermittent bone pain. The main route of excretion of injected aluminium was in urine, only a small proportion of the total input being removed in the "plasma bag" during plasma exchange. The extent of aluminium retention and bone deposition was not reflected by the plasma aluminium concentration before or after plasma exchange. Treatment of five patients with intravenous desferrioxamine increased the plasma aluminium concentration and urinary output of aluminium in those with evidence of aluminium retention. These studies show that patients with poor renal function receiving treatment with albumin contaminated with aluminium retain the metal and deposit it in bone, where it may eventually cause aluminium bone disease. Plasma exchange should be used with caution in patients with renal impairment.

Adult↗
PubMed1987

Cadmium concentrations in human kidneys from the UK.

The concentration of cadmium in the cortex and medulla of nearly 1000 kidneys obtained at autopsy was determined by atomic absorption spectrophotometry. Particular care was taken over the analytical procedures to ensure the accuracy and precision of the results; intrakidney variability was examined and found to be small. The frequency distributions of cadmium concentrations were approximately lognormal. There was much variability in cadmium concentrations between individuals, with geometric coefficients of variation for both cortex and medulla of around 100%. Cadmium concentrations varied with age and smoking habits, but not sex. Values were successively higher than those in the previous age-group up to 50-59 years (geometric mean 19 micrograms/g), after which they were successively lower; on average, heavy smokers had cadmium concentrations some 15-20% higher than those in light smokers who in turn had values some 15-20% higher than those in non-smokers. No definite conclusions could be reached on the variation of cadmium concentrations with either cause of death or location, or over time. The present study, the first of its kind in the UK, has provided benchmark data; its results are broadly in line with those from previous but smaller studies in other countries.

Adult↗