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  • 1
    ISSN: 1432-1076
    Keywords: Liver glycogenosis ; Phosphorylase kinase ; Phosphorylase ; Longitudinal study
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We report a longitudinal study of 41 patients with liver glycogenosis due to phosphorylase kinase deficiency. In their youth, patients displayed hepatomegaly (92%), growth retardation (68%), delayed motor development (52%), hypercholesterolaemia (76%), hypertriglyceridaemia (70%), elevation of glutamate pyruvate transaminase (56%) and fasting hyperketosis (44%). With age, these clinical and biochemical abnormalities gradually disappeared and most adult patients were asymptomatic.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 155 (1996), S. 870-876 
    ISSN: 1432-1076
    Keywords: Body composition ; Fat-free mass ; Mid-upperarm circumference ; Muscle area ; Skinfold
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Knowledge of the body composition in terms of fat and fatfree mass is used in paediatrics to estimate a child's nutritional status. To obtain the fat content or protein content sophisticated techniques exist. These techniques are often difficult to apply in daily practice. An anthropometric approach is therefore relevant because of its simplicity. In this study skinfold thicknesses and midupperarm circumference are presented as reference values and used to derive the fat-free mass fraction of the body and the arm muscle area.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 155 (1996), S. 870-876 
    ISSN: 1432-1076
    Keywords: Key words Body composition ; Fat-free mass ; Mid-upperarm ; circumference ; Muscle area ; Skinfold
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Knowledge of the body composition in terms of fat and fat-free mass is used in paediatrics to estimate a child’s nutritional status. To obtain the fat content or protein content sophisticated techniques exist. These techniques are often difficult to apply in daily practice. An anthropometric approach is therefore relevant because of its simplicity. In this study skinfold thicknesses and mid-upperarm circumference are presented as reference values and used to derive the fat-free mass fraction of the body and the arm muscle area. Conclusion Despite the shortcomings of an anthropometric approach in estimating the fraction of fat-free mass or protein content, its simplicity makes the method of calculating these fractions on skinfold thicknesses and mid-upperarm circumference valuable to apply in daily paediatric practice.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 149 (1990), S. 768-770 
    ISSN: 1432-1076
    Keywords: Head circumference ; Growth ; Newborn
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We have developed and tested a new instrument to measure the occipitofrontal head circumference of newborn infants more accurately. The intra- and interobserver variability was determined. The results indicate that the head circumference of newborn infants can be measured with an accuracy of 1 mm.
    Type of Medium: Electronic Resource
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  • 5
    ISSN: 1432-1076
    Keywords: Beckwith-Wiedemann Syndrome ; Hypoglycaemia ; Hyperinsulinism ; Hypoketonaemia ; Somatostatin
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Hypoglycaemia is a frequent finding during the neonatal period and may be due to insulin overproduction. Patients with Beckwith-Wiedemann syndrome have reduced numbers of somatostatin-producing cells and decreased extractable somatostatin. In this study the effect of long-acting somatostatin (SMS 201–995) on the glucose and insulin levels in an infant with Beckwith-Wiedemann syndrome and hyperinsulinaemic non-ketotic hypoglycaemia is described. SMS 201–995 lowered basal insulin levels while maintaining normal glucose and insulin homeostasis. During fasting however, both glucose levels declined rapidly whereas insulin levels did not. The absence of both ketosis and elevated levels of free fatty acids and lactate during hypoglycaemia, as observed in our patient, are important diagnostic clues since the insulin levels themselves may sometimes be only slightly elevated
    Type of Medium: Electronic Resource
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