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Mortality rate at the age of 20 years in children with the lethal PiZZ genotype of alpha 1-antitrypsin deficiency is in the order of 20-40%, but is significantly lower than in previously studied adults, partly as a result of successful neonatal care. We therefore studied the growth of PiZZ children and their parents to assess the role of modern neonatal care in this regard. We studied the growth of 31 PiZZ children and their parents prospectively from birth to the age of 1 year. We found that daily weight gain during the neonatal period was positively correlated with mid-parental height, whereas in PiZZ children and in their parents, bone age at presentation was positively correlated with the cumulative years of height gain from birth to the end of the growth period. No such correlations were found for weight gain. The timing of growth spurt in the children (mean 1.7 years), the start of pubertal growth spurt in boys (mean 11.2 years), and the difference between height of the child and mid-parental height (mean 4 cm) did not differ from previous observations in PiSZ children and also differed from the findings in unaffected siblings. The present study provides no indications that modern neonatal management has positive effects on growth outcome, but this needs to be confirmed in a randomised trial.Chronic acid-base disorders and liver dysfunction in anesthetized dogs: effect of plasma volume expansion.
In 7 anesthetized mongrel dogs, we measured ventilation and the concentration of arterial blood gases and plasma electrolytes over 5 hr in the basal state and during acidosis, alkalosis, and large-volume plasma volume expansion. In acidosis, plasma b