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Biomedical subjects

R Steendijk

Publications and source records attributed to R Steendijk.

At least 19 recordsLinked to original sources

The pattern of growth and growth retardation of patients with hypophosphataemic vitamin D-resistant rickets: a longitudinal study.

Growth in height of 16 patients (5 boys and 11 girls) with hypophosphataemic rickets (HR) was studied in a longitudinal survey. The data shortly before and during puberty were analysed on the basis of Preece Baines curves, fitted to the original data; for the analysis at the age of 5 years, the original data were used. It appeared that the overall shape of the individual and average growth pattern could be adequately described by the Preece Baines method. The results further showed that from the age of 5 years onwards, average height was approximately two standard deviations below the normal mean for Dutch children. The patients showed a normal pubertal growth spurt which was, in general, insufficient to restore the growth retardation already established before adolescence. The four children who did show catch-up growth between the age of 5 years and adulthood had minimal rachitic lesions. The greater impact of the disease on growth in early childhood than on adolescent growth could be explained by the fact that HR mainly affects the growth of the legs, the major contributor to body size in early childhood. Finally, it was found that the difference between bone age, as determined by the Tanner Whitehouse (TW2)-method, and chronological age was not significant and the adult height in all patients except two could be adequately predicted from bone age and height.

Adolescent

Remarkable catch-up growth in a boy with steroid-responsive nephrotic syndrome.

A boy is described who was followed from 4 to 21.5 years of age, during which time he was treated with high doses of corticosteroids for a steroid-responsive nephrotic syndrome. For the first 10 years therapy was mainly continuous and height decreased from -0.5 to -4.5 SDS. Later discontinuous treatment without a reduction in the total weekly dose was accompanied by rapid catch-up growth. Final height was -1.0 SDS. During most of the time skeletal age was severely retarded and a discrepancy between the development of the short bones of the hand and the carpal bones was noted.

Adrenal Cortex Hormones

Metacarpal measurements in X-linked hypophosphataemic rickets.

In 15 girls and 7 boys with hypophosphataemic vitamin D-resistant rickets, midshaft diameter, combined cortical thickness, cortical area and metacarpal length were determined in the second metacarpal on radiographs of the left hand. The values obtained were compared with age-matched and height-matched controls. In a longitudinal study the same bone measurements were taken in these 22 patients and in additional 3 boys. The metacarpal diameter in the patients was increased whereas the combined cortical thickness was decreased. The former feature appeared to have its onset in early childhood. Cortical area was normal for age and increased for height, which was taken to indicate a normal or increased bone mass in these children. Metacarpal length was normal for age and height in the boys and increased for height but still within the normal range in girls. This is in agreement with the normal arm-span that has been found in these children.

Adolescent

The relation between attained adult height and the metaphyseal lesions in hypophosphataemic vitamin-D resistant rickets.

In 13 young adult patients (5 men and 8 women) adult height was correlated with the average rachitic activity on radiographs of the wrist, taken at intervals during childhood. To this end the rachitic activity was classified into 4 different, clearly distinguishable stages according to its severity. The coefficient of correlation between adult height (expressed as standard deviation score) and the average rachitic activity or score was -0.796 (p less than 0.01), indicating that adult height varied inversely with the severity of the disease. The regression equation between adult height (y) and the rachitic score (x) was: y = -1.53x + 0.90; the SEy was 0.76. Although the coefficient of correlation was highly significant, this value for SEy indicated that much of the observed variation was due to other factors, apart from the rachitic process.

Adult

Distribution of the perilacunar hypomineralized areas in cortical bone from patients with familial hypophosphatemic (vitamin D-resistant) rickets.

The perilacunar areas of low mineral density in microradiographs from cortical bone of patients with hypophosphatemic (vitamin D-resistant) rickets are not evenly distributed throughout the bone tissue. Their frequency and distribution were determined in bone from 9 patients with this disease. It was found that the lesion was more frequent in haversian bone than in interstitial bone, and along the inner circumference of growing haversian systems as compared with outer circumference. These findings indicate that the lesion is the result of retarded mineralization, and that mineralization slowly proceeds in these areas as the bone becomes older. A relatively high frequency of the lesion was also found in osteons with an elliptical cross section along the long axis of the ellipse. The cause of the abundance of the lesion at these sites is not clear, but it is possible to explain the uneven distribution in elliptical osteons by assuming an unequal rate of bone formation in these structures.

Adult

The effect of a low-calcium diet in lactating rats; observations on the rapid development and repair of osteoporosis.

Female rats were given a low-calcium diet (0.05%) during the last three weeks of the lactating period, followed by a normal diet (1.03% calcium) during the first three weeks after lactation. The resulting bone loss and its recovery were studied by means of microradiography, tetracycline-uptake, quantitative estimation of the cortical area of cross-sections from the femoral midshaft, and estimation of total body calcium. The cortical area in the femoral midshaft fell to 46% of its original value during depletion, and then rose to 78% during the first three weeks after weaning. Total body calcium fell from 1.12% to 0.60% of body weight and then increased to 0.89%. Removal of bone occurred mainly in the spongiosa and on the endosteal side of the cortex. Subsequently, new bone was laid down on the endosteal side, but also to some extent on the periosteal side of the cortex. The mineral density of this new bone was low. During the recovery phase resorption cavities within the cortex were filled in a concentric manner as in Haversian remodeling. Neither this feature nor the low mineral density of bone are normally present in the rat.

Animals