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C R Paterson

Publications and source records attributed to C R Paterson.

At least 73 records · Page 4Linked to original sources
PubMed1988

Hypothesis: Shwachman's syndrome of exocrine pancreatic insufficiency may be caused by neonatal copper deficiency.

Shwachman's syndrome is, after cystic fibrosis, the most common cause of exocrine pancreatic insufficiency in childhood. The cause of the disorder is not known but we were struck by the fact that the histological appearances of pancreatic atrophy in this condition resemble those seen in experimental copper deficiency, in which the pancreatic acinar damage persists long after the copper deficiency is relieved. Other features of Shwachman's syndrome include neutropenia, anaemia and abnormalities of the ribs and of the metaphyses of long bones. All these findings have also been reported in children with copper deficiency during the 1st year of life. We suggest that some or all cases of Shwachman's syndrome are caused by a period of copper deficiency in early infancy.

Anemia↗
PubMed1987

Clinical and radiological features of osteogenesis imperfecta type IVA.

This paper reports a survey of 78 patients with osteogenesis imperfecta (OI) type IVA, the variant that causes the greatest difficulties in differential diagnosis. A subgroup of children aged between five and eleven were compared with a control group from local schools. Among the OI children there were, apart from the high number of fractures, also significantly increased incidences of bruising, nosebleeds, excessive sweating and hypermobility of joints. Although none of the children with OI type IV had the dark blue or grey sclerae of the type I disease, an appreciable number had pale blue sclerae in early childhood. Radiologically normal bone texture was seen at the time of the first fracture in 10 out of 17 patients. Hypertrophic callus was seen in five patients and metaphyseal fractures in four. We hope that the information on this large group of patients will assist in the prevention of diagnostic difficulties.

Age Factors↗
PubMed1986

Measurement of blood levels of 7-dehydrocholesterol in man by high-performance liquid chromatography.

A high-performance liquid chromatography procedure was established to measure 7-dehydrocholesterol (7DHC) in the blood. Interference studies were performed using 20 similar compounds likely to give analytical errors. 7DHC was extracted with a methanol chloroform mixture. Extraction recoveries were reproducible and acceptable, with low methodological variations from batch-to-batch. Results show that 7DHC is not detectable in the blood, contrary to the previous reports. Possible explanations for its absence in the blood are: (1) it is confined to the skin and is not present in the blood in this form; (2) because the molecule is labile, it is destroyed by the methods applied; (3) previous reports on 7DHC levels were based on non-specific methods.

Cholestadienols↗
PubMed1986

The value of serum 25-hydroxyvitamin D measurements in hypoparathyroid and pseudohypoparathyroid patients treated with calciferol.

In 23 patients with hypoparathyroidism or pseudohypoparathyroidism treated with vitamin D, and in whom the dosage was adjusted downward or upward in response to hypercalcemia or hypocalcemia respectively, assays of serum 25-hydroxyvitamin D (25-OHD) were carried out in addition to the usual serum calcium assays. In 120 assays there was a significant correlation between serum 25-OHD levels and serum calcium levels (corrected for serum albumin). There was, however, no clear distinction between the 25-OHD levels of patients who were hypocalcemic, normocalcemic or hypercalcemic. The highest serum 25-OHD level found in a hypocalcemic patient was 1193 nmol/L and the lowest serum 25-OHD level found in a hypercalcemic patient was 605 nmol/L. It was not possible to predict subsequent episodes of hypocalcemia or hypercalcemia from the serum 25-OHD levels. The 25-OHD assay was found to be useful only in checking compliance. We conclude that the assay of serum 25-OHD is of no more value than serum calcium alone in the management of compliant patients.

25-Hydroxyvitamin D 2↗
PubMed1986

Risk of dominant mutation in older fathers: evidence from osteogenesis imperfecta.

The mean paternal age at birth of 80 presumed mutant cases of dominant osteogenesis imperfecta (OI) was significantly higher than that of population controls and remained so after adjusting for maternal age. There was also an increase in mean maternal age (not significant) which disappeared after adjusting for paternal age. No significant increase in maternal or paternal age was found in cases having OI either of a dominant type with an affected parent or of a type (Sillence type III) usually regarded as recessive. We conclude that, as in certain other dominant conditions, the risk of mutant OI increases with paternal age. However, the rate of increase of risk with paternal age appears to be considerably lower than, for example, in achondroplasia. The overall risk of fresh dominant mutation in older fathers may therefore be lower than has previously been suggested.

Adult↗
PubMed1985

HLA antigens and familial benign hypercalcaemia.

We have determined the blood groups and HLA haplotypes in 15 members in four generations of a large kindred with familial benign hypercalcaemia (familial hypocalciuric hypercalcaemia). No linkage was seen between the disorder and ABO or Rh blood groups or the HLA antigens.

Blood Group Antigens↗
PubMed1985

Calcium-ATPase activity in erythrocyte ghosts from patients with familial benign hypercalcaemia.

It has been suggested that the parathyroid glands and the kidneys are insensitive to the high extracellular calcium levels found in familial benign hypercalcaemia (FBH) (familial hypocalciuric hypercalcaemia) and that there may be a general disorder of the plasma membrane 'calcium pump'. We have found that the activity of the calcium-stimulated, magnesium-dependent ATPase of erythrocyte ghost membranes from patients with FBH was significantly higher (p less than 0.01) than that from normal subjects. Values in FBH, as a group, were higher than those in primary hyperparathyroidism, but the difference was not significant. We suggest that the membrane abnormality in FBH could be a disorder of the regulation of the calcium pump.

Adult↗
PubMed1984

Long term follow up of untreated primary hyperparathyroidism.

Fourteen patients with primary hyperparathyroidism and whose initial serum calcium concentrations were 2.75 mmol/l (11.0 mg/100 ml) or more were followed up for five to 23 years without operative treatment. One had osteitis fibrosa when seen and died with a fibrosarcoma 22 years later. The remaining 13 patients, who were followed up for a mean of 10 years, came to little obvious harm from not being operated on. Their serum calcium concentrations did not rise and there was no evidence of progressive renal impairment. In four patients who presented originally with renal calculi there were three further episodes of renal colic in 54 patient years of follow up. Conservative management of primary hyperparathyroidism is not an unreasonable option, and patients who do not have symptoms need not necessarily be pressed to accept surgery.

Aged↗
PubMed1984

Correlation between serum ionised calcium and serum albumin concentrations in two hospital populations.

One quarter of 172 patients from two hospitals with no obvious disturbances of calcium homeostasis and with total serum calcium concentrations that were normal after adjustment for albumin concentration had low serum ionised calcium concentrations. The low values were not due to changes in pH but were associated with hypoalbuminaemia. Significant positive regressions of ionised calcium on albumin concentration were observed in patients from both hospitals and also in 48 healthy laboratory staff. Because the regressions did not differ between patients and healthy subjects the low ionised calcium values associated with hypoalbuminaemia are unlikely to have been of pathological importance. These findings indicate that interpreting serum ionised calcium concentrations in patients with a reduced serum albumin concentration on the basis of a reference range determined in subjects with a normal serum albumin concentration may be clinically misleading.

Adult↗
PubMed1984

Osteogenesis imperfecta after the menopause.

We studied the fracture rate as a function of age in 45 women and 20 men with osteogenesis imperfecta. In each variant of the disorder, the fracture rate in women peaked in childhood, declined in adolescence, and rose again after the menopause. In contrast, the fracture rate in men remained low after adolescence. After the menopause women were vulnerable to crush fractures of the spine as well as fractures of the long bones. We conclude that the increased fracture rate after the menopause in women with osteogenesis imperfecta reflects the superimposition of the effects of age-related bone loss on those of the defective collagen structure of osteogenesis imperfecta, and that hormone-replacement therapy may be specifically indicated in this group of patients from the time of the menopause. We also suggest that osteogenesis imperfecta should be included in the differential diagnosis of women presenting with crush fractures of the spine.

Age Factors↗
PubMed1984

California psychological inventory profiles of peer-nominated assertives, unassertives, and aggressives.

Examined the California Psychological Inventory (CPI) profiles of peer-nominated assertives , unassertives , and aggressives . Eighty males from two social fraternities peer-nominated assertives , unassertives and aggressives . The nomination procedure yielded 12 nominees per group. A profile analysis of the CPI indicated that assertives and aggressives were significantly more similar than were assertives and unassertives , and unassertives and aggressives . Results of a subscale analysis indicated that assertives and aggressives were significantly higher on Dominance, Capacity for Status, Sociability, and Social Presence than Unassertives , but were not significantly different from each other on these subscales. Also, assertives were significantly higher on Socialization, Self-Control, and Achievement via Conformance than were aggressives . Finally, assertives were significantly higher on Achievement via Conformance than were unassertives and aggressives . Implications are discussed.

Achievement↗