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Biomedical subjects

R Steendijk

Publications and source records attributed to R Steendijk.

At least 19 recordsLinked to original sources
PubMed1993

Neurological and adrenal dysfunction in the adrenal insufficiency/alacrima/achalasia (3A) syndrome.

Review of 20 patients with glucocorticoid deficiency (three cases also with salt loss) associated with absent tear secretion (19 cases) and achalasia of the cardia (15 cases) revealed neurological abnormalities in 17 including hyper-reflexia, muscle weakness, dysarthria, and ataxia together with impaired intelligence and abnormal autonomic function, particularly postural hypotension. These findings indicate that significant neurological problems are common in this multisystem disorder.

Addison Disease↗
PubMed1992

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↗
PubMed1986

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↗
PubMed1986

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↗
PubMed1985

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↗
PubMed1984

Height, sitting height and leg length in patients with hypophosphataemic rickets.

Height, sitting height (SH) and subischeal leg length (SLL) were determined in 5 boys and 11 girls with hypophosphataemic (vitamin D-resistant) rickets, aged 4-14 years. Their average height was -2.05 +/- 1.22 SDS, and SLL (average value: -2.59 +/- 1.18 SDS) was more reduced than SH (average value: -0.91 +/- 1.37 SDS). SLL and SH were abnormally low in 11 and 3 of the patients respectively. The difference between SLL and SH was abnormally low in only 4 of the children, indicating a mild degree of disproportion. None of the patients had relatively long legs. There was no relation between height and the degree of disproportion, a finding which tallied with the relatively mild degree of disproportion. This indicated that the normal interindividual differences in proportion were more important than a preferential effect of rickets on leg length.

Adolescent↗
PubMed1982

Growth and development in a girl with pseudohypoparathyroidism and hypothyroidism. A longitudinal study.

Statural growth, physical and skeletal development of a girl with pseudohypoparathyroidism and primary hypothyroidism were analysed in a longitudinal study, which lasted for 12 years from the age of 0.8 years. Growth in height, which slowed down when thyroid therapy was withheld, was within the normal range. Still, as a result of early puberty, the girl became a small adult. Skeletal age was advanced over chronological years by an average of 2.7 years. This rapid skeletal development was more pronounced in the tubular bones than in the round bones of the hand. Two of the five metacarpals of the left hand grew more slowly than the others. This became clinically apparent at the age of 5.6 years. In all five metacarpals growth ceased at the same time, indicating that the abnormally short size of the two metacarpals did not result from early epiphyseal closure.

Age Determination by Skeleton↗
PubMed1982

Catch-up growth following long-term administration of essential fatty acids in a girl with growth failure and essential fatty acid deficiency.

A 10-year-old girl with benign recurrent intrahepatic cholestasis, malabsorption of fat, growth failure (growth rate 1.2 cm/year) and deficiency of essential fatty acids (EFA) is described. Long-term administration of EFA, mainly by cutaneous application of sunflower seed oil, was followed by a remarkable catch-up growth (23.8 cm in 3.5 years) while the serum values for EFA improved. Since no other changes in the therapeutic regimen occurred and other causes of growth failure and subsequent acceleration of growth could be ruled out, it is highly probable that the observed increase in growth rate was the result of the administration of EFA.

Body Height↗
PubMed1982

Growth and maturation in pseudo-hypoparathyroidism: a longitudinal study in 5 patients.

In 4 girls and 1 boy with pseudo-hypoparathyroidism growth and physical maturation were followed longitudinally for 7-13 years until adult height had been reached. As a result of early puberty and cessation of growth all patients were relatively shorter as adults than in their childhood years. The difference between average height at the age of 8.0 years and average adult height was 2.25 SD. This observation offers an explanation for the finding in the literature that short stature is more common in adults with this disease than in children. Skeletal age was advanced in all cases and the development of the tubular bones of the hand was more advanced than the development of the round bones. It is possible that this difference resulted from inappropriately early closure of the epiphyseal discs of disproportionally short metacarpals and phalanges. On the other hand it may be an aspecific phenomenon of advanced skeletal maturation.

Adolescent↗