Biomedical subjects
Publications and source records attributed to R J Walton.
For the year 1974, in Manitoba, 905,586 x-ray examinations were performed. The services were provided in 154 facilities, using 332 x-ray units, conducted by 566.6 full-time equivalent individuals, at a cost of $11,117,183. The examinations per capita have increased from 0.52 to 0.86 in the past decade while the cost per examination rose only from $11.00 to $12.63.
The clinical and biochemical features of four adults with Engelmann's disease (Camurati-Engelmann disease; progressive diaphyseal dysplasia) are presented. One young patient, with a particularly severe form of the disease, is discussed in detail. Biochemical abnormalities were found in three of the patients. In two of them, one with localized hyperostosis and one with generalized bone disease, the only changes were an increase in the plasma alkaline phosphatase and urinary total hydroxyproline excretion. The most severely affected patients, who had had progressive and generalized bone disease from age two and a half years, also had persistent hypocalcaemia and hyperphosphataemia, a positive calcium balance, and a very low urine calcium excretion. It is suggested that some patients with Engelmann's disease may have a previously unrecognized metabolic disorder associated with increased retention of calcium and excessive bone formation. The possible role of abnormal phosphate metabolism in this increased formation of bone, and the relationship of Engelmann's disease to other hyperostoses, are discussed.
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The activity of proline imino-peptidase (EC 3.4.1.4) may provide a measure of collagen degradation, and a method for its estimation in human serum is described. The mean value of this enzyme activity in 48 normal adults of 4.7 mU/l (S.D. 0.9) did not change with sex, age or dietary collagen. In 8 patients with liver disease associated with elevated levels of plasma alkaline phosphatase activity the mean value was normal (4.5 mU/l, S.D. 1.5). However, in 25 patients with untreated Paget's disease of bone, values were significantly increased (mean 7.4 mU/l; S.D. 2.8; p less than 0.001) and were positively correlated with plasma alkaline phosphatase activity (r = 0.75; p less than 0.001) and with urine total hydroxyproline excretion (r = 0.68; p less than 0.001). In patients given disodium ethane-1-hydroxy-1,1-diphosphonate for 3--6 months, serum proline imino-peptidase activity decreased to normal values.
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Adult-onset osteomalacia with multiple renal tubular defects and generalized aminoaciduria is uncommon, and where familial it is characteristically an autosomal recessive disorder. This paper describes a kindred in which the syndrome has appeared in four successive generations, apparently inherited in a dominant manner, and possibly associated with diabetes mellitus. The proposita had hypophosphataemia, renal glycosuria, proteinuria and generalized aminoaciduria, and at the age of 22 developed symptoms of osteomalacia which responded to treatment with oral phosphate. Her father had been similarly affected: renal glycosuria was first noted when he was 24, and 12 years later he developed diabetes mellitus from which he died. One sister, aged 31, has renal glycosuria, aminoaciduria and hypophosphataemia without bone disease. In the three preceding generations at least seven other individuals had crippling bone disease and profound muscle weakness of early adult onset; in four, preterminal polydipsia was recorded, and others had renal glycosuria or diabetes mellitus. Three of the five children in the latest generation have slight proteinuria but not other detectable abnormality. The possible association between these renal tubular defects and diabetes mellitus is discussed.
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A through-knee amputation for suspected osteogenic sarcoma was performed on a patient with Paget's disease of the tibia. Plasma alkaline phosphatase (AP) activity fell to normal values, apparently as a single exponential function of time, with a half-life of 1.7 days. The daily turnover of bone-derived plasma AP was estimated to be approximately 200 I.U. from the Pagetic tibia and 20 I.U. from the rest of the skeleton.
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1. 1,25-Dihydroxycholecalciferol (1,25-(OH)2-D3) was given at a dose of 5-0 nmol (2-1 mug) daily by mouth for 4-12 days to three patients with hypophosphataemic (type I), vitamin D-resistant rickets. 2. 1,25-(OH)2-D3 increased intestinal absorption and urinary excretion of calcium without significant effect on the renal handling of phosphate or its plasma concentration. 3. It is concluded that in this type of vitamin D-resistant rickets the renal phosphate abnormality is unlikely to be due to diminished endogenous production of 1,25-(OH)2-D3. 4. The difference between this condition and other hypophosphataemic states is discussed.