A patient with nucleoside phosphorylase deficiency, selective t-cell deficiency, and autoimmune hemolytic anemia.
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Biomedical subjects
Publications and source records attributed to C Imperato.
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The relationship between growth and pulmonary disease has been studied mainly in asthma and cystic fibrosis. In asthma, the most frequent chronic pulmonary disease in childhood, results were conflicting, until the degree of severity of the disease and growth phases were taken into account. Research on 683 children has shown that the percentage of underweight was higher in chronic than intermittent asthma. Moreover, the distribution of underweight patients by ages is different in the two types of asthma: uniform in intermittent asthma; two peaks below the age of 2 and above the age of 12 respectively in chronic asthma. Further data of 65 children treated with slow-releasing theophylline for approximately two years corroborates that puberty is a particularly vulnerable period. Indeed, in the most severe asthmatic males, theophylline is able to completely normalize the growth pattern in childhood but not in puberty. In cystic fibrosis malabsorption makes the study of the relationship between growth and pulmonary disease more complex. The pattern of growth in patients with cystic fibrosis is moving away from the normal pattern with ageing, hence the worsening of pulmonary disease is responsible for the worsening in the growth pattern. The growth pattern today is far better than that of 20 years ago. However, puberty, especially in female patients, is a critical period. Often the puberal spurt is delayed for a few years or is even completely absent. In a group of patients with chronic pulmonary disease due to different causes, weight is more implicated than height and the same pattern was observed in cystic fibrosis. Moreover, as in asthma, weight and height are more implicated in females than males. The entity of alteration observed is midway between the minimal in asthma and the maximal in cystic fibrosis.
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Several anthropometric parameters (weight, height, thickness skinfolds, and body circumferences) together with the bioelectric impedance (BIA) were measured in a group of 407 healthy prepuberal children (205 males, mean age +/- SD: 6.8 +/- 1.6 years; 202 females, mean age +/- SD: 6.6 +/- 1.6 years) who did not practice any competitive sport. The group was divided into six weight classes (underweight to obese 3). The average values of the anthropometric data, BIA, FM (which is derived from the thickness skinfolds and BIA), TBW (derived from BIA), AFA and AMA (both derived from the forearm circumference and triceps skinfold) were all evaluated in relation to sex and weight classes. The average values of all the measured thickness skinfolds, FM and AFA were higher in the females. When considering the body circumferences, only the wrist circumference showed a statistically significant difference between sexes, being higher in males. In contrast both the rates waist/hips circumferences and waist/thigh circumferences were higher in males. As far as the BIA and FM average values is concerned, these were higher in females. The average value of FM derived from measuring the thickness skinfolds (males = 17.65 +/- 4.89%: females = 23.81 +/- 4.72% of body weight) proved higher than that one derived from BIA (males = 17 +/- 9.02%; females = 19.27 +/- 9.18%). The analysis of anthropometric and BIA data in relation to the weight classes showed significant variations of the mean values, except the rates waist/hips circumferences and waist/thigh circumferences. Therefore our data seem to exclude the presence of any difference of the fat distribution between sexes in paediatric age.(ABSTRACT TRUNCATED AT 250 WORDS)