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R Paisey

Publications and source records attributed to R Paisey.

18 recordsLinked to original sources
PubMed2007

Is arterial stiffening in Alström syndrome linked to the development of cardiomyopathy?

BACKGROUND: Alström syndrome (AS) is a rare autosomal recessive condition characterized by retinal degeneration, childhood obesity, and severe insulin resistance. Dilated cardiomyopathy of unknown aetiology is a well-recognized and potentially lethal complication. The aim of this study was to investigate the relationship between vascular function, hyperinsulinaemia and cardiac performance in AS. MATERIALS AND METHODS: Fifteen subjects with AS (mean age 21 years, range 10-35) were studied and compared with age-, sex-, and blood pressure-matched healthy controls. Large artery stiffness and wave reflections were assessed in both groups by measuring aortic and brachial pulse wave velocity (PWV) (carotid-femoral and carotid-radial) and augmentation index (AIX) (Sphygmocor). In AS subjects, left ventricular function was assessed by echocardiography and metabolic parameters including fasting insulin, glucose, lipids and brain natriuretic peptide were also measured. RESULTS: Comparing AS subjects vs. controls (mean +/- SD), AIX was elevated in AS subjects (18 +/- 9% vs. 3 +/- 11%, P < 0.0001). No significant changes in brachial PWV (8.1 +/- 1.3 m s(-1) vs. 7.3 +/- 1.1 m s(-1), P = 0.14) or aortic PWV (6.5 +/- 1.1 m s(-1) vs. 6.0 +/- 1.0 m s(-1), P = 0.26) were observed. AS subjects were hyperinsulinaemic and had disturbances in lipid profiles relative to controls. No correlations were observed between vascular, metabolic and echocardiographic parameters. CONCLUSIONS: In AS there are alterations in the shape of the central arterial pressure waveform associated with augmented aortic systolic pressure and indicative of increased wave reflection. Unfavourable central arterial haemodynamics in AS may contribute to the development of cardiomyopathy but other aetiological factors are probably involved.

Adolescent↗
PubMed2006

Syndromic obesity and diabetes: changes in body composition with age and mutation analysis of ALMS1 in 12 United Kingdom kindreds with Alstrom syndrome.

CONTEXT: Alström syndrome (AS) is a monogenic form of infancy-onset obesity and insulin resistance, caused by ALMS1 mutations. The natural history of the insulin resistance is unknown, in particular how this relates to changes in body composition. It is also unclear how ALMS1 mutations relate to the characteristic phenotype. OBJECTIVES: Our objectives were to characterize body composition and metabolic parameters, to establish ALMS1 mutation spectrum of United Kingdom AS patients, and to determine whether a genotype-phenotype correlation exists. DESIGN AND PATIENTS: We conducted a cross-sectional cohort study of 12 unrelated subjects with AS. Age-standardized body composition was assessed by anthropometry and dual-energy x-ray absorptiometry and insulin sensitivity by homeostasis model assessment. The exons and intron-exon boundaries of ALMS1 were directly sequenced. SETTING: The study was performed during the annual Alström Syndrome UK multidisciplinary screening clinic. RESULTS: AS patients have early-onset obesity, but body mass index, waist circumference, and body fat from dual-energy x-ray absorptiometry were negatively correlated with age (r = -0.37, P = 0.2; r = -0.84, P = 0.002; and r = -0.6, P = 0.05). Despite this, insulin resistance increased, demonstrated by raised fasting insulin and fall in homeostasis model assessment insulin sensitivity with age (r = -0.64, P = 0.02). ALMS1 mutations were identified in 10 of 12 patients, with a potential founder mutation in exon 16 present in five [np 10775del (C); Del3592fs/ter3597]. No genotype-phenotype correlation was observed. CONCLUSIONS: We identified mutations in ALMS1 in more than 80% of patients with no genotype-phenotype correlation. In AS, severe childhood obesity, waist circumference, and body fat decrease with age, whereas insulin resistance increases. The abdominal obesity, insulin resistance, diabetes, hypertriglyceridemia, and hypertension suggest that AS could represent a monogenic model for the metabolic syndrome.

Absorptiometry, Photon↗
PubMed2006

Short-term effects of severe dietary carbohydrate-restriction advice in Type 2 diabetes--a randomized controlled trial.

OBJECTIVE: This study sought to examine the effects of a 3-month programme of dietary advice to restrict carbohydrate intake compared with reduced-portion, low-fat advice in obese subjects with poorly controlled Type 2 diabetes. RESEARCH DESIGN AND METHODS: One hundred and two patients with Type 2 diabetes were recruited across three centres and randomly allocated to receive group education and individual dietary advice. Weight, glycaemic control, lipids and blood pressure were assessed at baseline and 3 months. Dietary quality was assessed at the end of study. RESULTS: Weight loss was greater in the low-carbohydrate (LC) group (-3.55 +/- 0.63, mean +/- sem) vs. -0.92 +/- 0.40 kg, P = 0.001) and cholesterol : high-density lipoprotein (HDL) ratio improved (-0.48 +/- 0.11 vs. -0.10 +/- 0.10, P = 0.01). However, relative saturated fat intake was greater (13.9 +/- 0.71 vs. 11.0 +/- 0.47% of dietary intake, P < 0.001), although absolute intakes were moderate. CONCLUSIONS: Carbohydrate restriction was an effective method of achieving short-term weight loss compared with standard advice, but this was at the expense of an increase in relative saturated fat intake.

Cholesterol↗
PubMed1994

Diabetes and alternative medicine: cause for concern.

'Alternative' medicines are becoming increasingly popular, and in this paper we describe our experience with alternative approaches to orthodox diabetes management. Four patients with insulin-dependent diabetes reduced or stopped their insulin in favour of therapeutic approaches including prayer, faith healing, unusual diets, and supplements of vitamins and trace elements. This resulted in ketoacidosis in three, in one case life-threatening; and weight loss and hyperglycaemia in the other. One patient developed serious retinopathy. Additionally, eight other types of alternative diabetic treatment are described, not as far as we know associated with such serious complications. These include homeopathy, reflexology, meditation, herbal treatment, 'cellular nutrition', 'subconscious healing', 'pearl therapy' (drinking milk in which pearls have been boiled) and 'astrotherapy' (typing pieces of coral around the arm). Diabetes is a chronic incurable disease, for which modern treatments remain somewhat unsatisfactory. It is therefore perhaps not surprising that some patients seek alternative treatments with more attractive claims. Diabetes health professionals need to be aware of the potential dangers associated with some of these treatments.

Adult↗
PubMed1984

Correlation between skin glycosylation and glycaemic control in human diabetes.

Glycosylation of human skin has been measured by an adaptation of the thiobarbituric acid reaction on samples obtained mainly at autopsy from normal and diabetic subjects. The mean (+/- 1 SD) glycosylation of skin from 33 normal subjects was 0.072 +/- 0.012 mumol fructosamine/100 mg wet weight tissue compared with 0.127 +/- 0.034 mumol (P less than 0.01) in the 10 diabetic subjects. There was a correlation between mean blood glucose levels in the 6 months before death and skin glycosylation in the diabetics, (r = 0.71, P = 0.025), but no correlation between age, sex, or the presence or absence of diabetic complications and skin glycosylation. Skin glycosylation in samples from four non diabetics was increased from 0.075 +/- 0.015 to 0.126 +/- 0.01 mumol fructosamine/100 mg wet weight tissue (P less than 0.01) after incubation with 50 mmol/l glucose solution for 7 d at 37 degrees C.

Adolescent↗
PubMed1984

Is soft tissue radiology useful in acromegaly?

Twenty-six acromegalics had measurements of serum growth hormone (GH) levels during a glucose tolerance test, X rays of the heel for heel pad thickness, and of the hand for bone/soft tissue ratio. These measurements were performed before and between 2 and 7 years after treatment, which was by transethmoidal hypophysectomy, external irradiation or a combination of the two. Neither the heel pad thickness nor bone/soft tissue ratio correlated with the mean serum GH nor with each other before and after treatment. The nine patients who were assessed as showing a good clinical response to treatment showed a significantly greater fall in mean serum GH than the 17 patients who were assessed as unchanged or partial responders (40.1 +/- 20 mmu/l compared with 9.9 +/- 14.0 mmu/l, p = 0.02). However, there were no significant differences in changes of heel pad thickness nor bone/soft tissue ratio between these groups. It is concluded that these radiological measurements of soft tissues are unhelpful in the follow-up of acromegalic patients.

Acromegaly↗
PubMed1983

A collaborative study of the measurement of glycosylated haemoglobin by several methods in seven laboratories in the United Kingdom.

A collaborative study compared methods for measuring glycosylated haemoglobin in seven laboratories in the United Kingdom. No satisfactory standard for general use was found. Satisfactory internal quality control systems were in use for each assay which allowed the maintenance of a normal range in each participating laboratory. No satisfactory quality control system suitable for general use could be identified. Costs and convenience of the assays are reported. The technical problems with each type of assay and precautions for their use were identified, such as the need for standardization in incubation times, the practicability of automation of colorimetric assays, and the precision of pH needed for buffers in column separation methods. The relevance of the technical problems to interpretation of measurements is also considered. It is concluded that laboratories measuring glycosylated haemoglobin should maintain a normal range, use 'in-house' quality controls to monitor assay performance and keep clinical colleagues informed of the findings and of any changes in methodology that might affect the interpretation of results.

Autoanalysis↗
PubMed1978

The effects of chlorpropamide and insulin on serum lipids, lipoproteins and fractional triglyceride removal.

The effects of chlorpropamide on serum lipids, lipoproteins and fractional triglyceride removal have been studied over 12 months on 10 maturity onset diabetics not controlled on diet alone. Similar studies were carried out in 6 maturity onset diabetics who had failed to respond to sulphonylureas and 6 new insulin requiring diabetics. In the chlorpropamide treated patients there was an initial fall in serum and VLDL triglyceride but this effect was lost at 12 months. There was no change in fractional triglyceride removal. At 12 months there was a fall in LDL and a rise in HDL choelsterol. An initial improvement in glucose tolerance and insulin secretion was maintained at 12 months. In the insulin treated group the initial fall in serum and VLDL triglyceride was maintained at 12 months and was accompanied by an increase in fractional triglyceride removal. There was also a fall in LDL and a rise in HDL cholesterol at 12 months. The failure of chlorpropamide to maintain the reduction in serum and VLDL triglyceride could be of importance in the genesis of coronary heart disease in maturity onset diabetics. The fall in LDL and rise in HDL cholesterol found both with chlorpropamide and insulin might be beneficial.

Adult↗
PubMed1978

Plasma cyclic AMP response to glucagon as an index of tissue responsiveness to thyroid hormone.

The rise in plasma cyclic AMP after glucagon has been studied in hyper- and hypothyroid patients before and after treatment. In the hyperthyroid group, each individual patient showed a decrease in cyclic AMP response to glucagon on becoming euthyroid, but there was no change after treatment with propranolol. In the hypothyroid group, each individual patient showed an increase in response on becoming euthyroid on thyroxine. We suggest that peak plasma cyclic AMP response to glucagon could be used to monitor tissue responisveness to thyroid hormone during treatment of hypo- and hyperthyroidism, though change in this response is a more sensitive indicator of change from the hypothyroid to the euthyroid state than from the hyperthyroid to the euthyroid state.

Adult↗
PubMedDate not supplied

Glycosylated haemoglobin in uraemia.

Glycosylated haemoglobin (GHb) was measured in 71 patients with stable chronic renal failure by the thiobarbituric acid (TBA) reaction and by agar gel electrophoresis. Nineteen patients were diabetic. Of the non-diabetics, 22 were treated conservatively (including 8 children), 15 by maintenance haemodialysis, and 15 by continuous ambulatory peritoneal dialysis. GHb measured by both methods correlated with postprandial blood glucose levels. There was a significant discrepancy between the two methods only in patients with serum urea concentrations greater than 30 mmol/l, mean +/- SD, (6.8 +/- 2.6% vs 8.2 +/- 2.5% for TBA and electrophoresis, respectively). This difference, delta GHb, correlated with serum urea, serum creatinine, and serum bicarbonate, but after logistic regression of results from all 71 patients only serum urea was associated with delta GHb. Lower haemoglobin and GHb and high fetal haemoglobin concentrations in the haemodialysis group suggested increased haemolysis in these patients. Measurement of GHb by the TBA method and by agar gel electrophoresis remain useful indicators of hyperglycaemia in patients with mild, stable chronic renal failure.

Adolescent↗