[Migration in Quebec: synthesis and a bibliography].
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Biomedical subjects
Publications and source records attributed to J W Gregory.
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McCune-Albright syndrome is a rare disorder caused by an activating mutation of the alpha subunit of the Gs protein and is characterized by multiple hormonal abnormalities, fibrous dysplasia of bone, and cutaneous pigmentation. We report a 10-week-old infant with McCune-Albright syndrome. He presented with failure to thrive, cardiorespiratory distress, and pigmented macules on his face, trunk, and limbs. Following the clinical diagnosis of McCune-Albright syndrome, he was subsequently shown to have hypercortisolism and hyperthyroidism.
OBJECTIVE: The study examined the degree to which male and female survivors of acute lymphoblastic leukaemia (ALL) perceive effort at low and moderate intensity exercise in association with related physiological variables. MATERIALS AND METHODS: Participants were 67 children. Thirty-five (14 boys and 21 girls) were long-time survivors of ALL and 32 (18 boys and 14 girls) were control subjects. The Children's Effort Rating Table (CERT) was used to measure whole-body perceived exertion at low and moderate intensity exercise. Peak oxygen uptake was measured using a motorized treadmill. CERT and physiological data were analysed using 2 x 2 mixed analyses of variance, appropriate t-tests and coefficients of correlation. RESULTS: In absolute terms, boys treated for ALL found perception of effort to be more strenuous at both low (3.9 vs. 3.5 units) and moderate (6.1 vs. 5.3 units) intensity exercise than control subjects, although differences were not significant (p > 0.05); girls treated for ALL found perception of effort to be the same as controls at low intensity exercise (3.1 vs. 3.1 units) but slightly higher than controls at moderate intensity exercise (5.6 vs. 5.2 units); neither of these differences were significant (p > 0.05). When CERT values were adjusted for (.-)VO(2) peak (%) and heart rate (HR) peak (%) differences remained non-significant. There were no significant interactions (Intensity x Group) in males, but the interaction for (.-)VO(2) peak (%) was significant in females (p < 0.05). The main effect for Intensity (low and moderate) was significant for all variables in boys and girls (p < 0.0001). The main effect for Group (ALL and controls) identified significantly greater absolute (b.p.m.) and relative (%) HR values in ALL boys at low and moderate intensity exercise. In female ALL and control subjects the interaction (Intensity x Group) distinguished between (.-)VO(2) peak (%) at moderate intensity exercise and HR peak (%) at low and moderate intensity exercise. Coefficients of correlation between perceived effort and (.-)VO(2) peak (%) in boys and girls were low to high (0.28-0.76), and between absolute and relative HR were also low to high (0.33-0.73). There were low correlations between time 'off therapy' and perceived effort, (.-)VO(2) peak (%) and HR peak (%) (-0.003 to -0.49). CONCLUSION: It was concluded that perception of effort in survivors of ALL at low and moderate intensity exercise was the same as that of control subjects. Correlations between perceived effort and physiological variables at moderate exercise were low to high, while those between perceived effort and time from treatment were generally weak.
Recently published standards for body mass index (BMI) based on population studies of height and weight in healthy British children allow an easy but indirect assessment of adiposity in healthy children. However, assessment of adiposity based on standards derived from reference populations may not be appropriate for use in subjects with disease states associated with abnormalities of growth and body composition. This hypothesis was tested by comparison between BMI standard deviation scores (SDS) and more direct measures of body fat derived from dual-energy X-ray absorptiometry (DEXA) and skinfold thickness in groups of children, receiving growth hormone, with inflammatory bowel disease, previously treated for malignancy, and healthy controls. Excess adiposity was defined as a body fat greater than the 85th percentile and was compared to a BMI SDS of +1.0. Overall the sensitivity and specificity for a BMI SDS of +1.0 to correctly identify individuals as having excess adiposity was 66% and 94%, respectively, when body fat was measured by DEXA, and 50% and 100% when estimated from skinfold measurements, respectively. There were no significant differences in these statistics whether applied to the individual disease groups or to healthy controls. These findings suggest that BMI under-predicts the prevalence of excess adiposity in children with disease states but surprisingly to no greater degree than that seen in healthy subjects.
In the majority of cases, short stature is due to normal physiological variations in growth and maturation. However, there are a significant number of other causes, some of which are amenable to treatment. This article reviews the clinical assessment and investigation of the short child in order to identify those children who might benefit from treatment.
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