Nitrogen deposition in malnourished children with cystic fibrosis

Article Abstract:

Children who are malnourished in terms of protein and total energy (calorie) intake often exhibit stunted growth and wasting. Catch-up growth follows nutritional rehabilitation. However, improved height and weight do not accurately reflect the status of body composition, especially with diseases where fluid and electrolyte (salt) balance is impaired, such as liver cirrhosis, cystic fibrosis (CF), or kidney disease. Thus, it is important to assess aspects of body composition, such as lean body mass (LBM), separately from height and weight. Gamma-neutron-activation analysis has been shown to provide accurate measurements of total body nitrogen (TBN), an indicator of body protein. This technique was used to assess protein nutritional status in 21 malnourished children (9 female) with CF who were undergoing nutritional rehabilitation, and in 21 healthy children. Values of all variables, including height, weight, LBM, TBN, and ratios of TBN to height, weight, or LBM, were all significantly lower in CF patients. After matching for sex, height, and bone age (a marker of pubertal status), the ratios of TBN to height and TBN/LBM remained significantly lower in CF patients, while TBN/weight was similar, probably due to increased water content and decreased body fat mass. Long-term studies of the CF patients given supplemental feedings indicated that resulting weight gain correlated significantly with nitrogen deposition. However, the association between growth in height and nitrogen deposition was variable. Some children with CF were able to maintain normal linear growth in spite of poor weight gain and low nitrogen deposition. This supports other studies that suggest that skeletal growth may be maintained at the expense of protein depletion from muscle and organs. The study shows the significance of evaluating TBN in children who have been malnourished. (Consumer Summary produced by Reliance Medical Information, Inc.)

Health aspects, Measurement, Growth disorders, Nitrogen in the body, Nitrogen (Nutrient)

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Pancreatic function in infants identified as having cystic fibrosis in a neonatal screening program

Article Abstract:

Cystic fibrosis is a genetic disease which causes the secretions of the body to be thick and consequently difficult to eliminate. As a result of this, the lungs are often severely affected. Cystic fibrosis occurs when an infant receives a defective gene from both parents; the condition is classified as a Mendelian dominant genetic disorder. The exact gene which causes cystic fibrosis was recently located on the long-arm of chromosome 7. The ability to test for such irregularities of the chromosomes of infants is relatively difficult and expensive. A system which uses an immunologic test of dried blood (an immunoreactive test) taken from newborns is commonly used to screen babies for the presence of cystic fibrosis. The test assesses the level of blood pancreatic isoamylase (an enzyme produced by the pancreas and required to metabolize starch) as an index of pancreatic enzyme production. Recent concerns about this method have been raised in the case of cystic fibrosis babies who have relatively normal pancreatic function. The present study associates the level of pancreatic functioning with the results of blood screening for cystic fibrosis. The diagnosis of cystic fibrosis is confirmed by a standard test called the sweat test. Ninety-eight infants who were identified by the blood test as having cystic fibrosis were referred to the study and 78 ultimately participated. Of these children, 37 percent had nearly normal pancreatic measures which were assessed by various physiologic means other than blood enzymes. This study demonstrates that the immunoreactive test is able to identify cystic fibrosis children who maintain pancreatic functioning (approximately 37 percent of children). At present it is not possible to identify the rate of false negative results of patients who have adequate pancreatic functioning. (Consumer Summary produced by Reliance Medical Information, Inc.)

Research, Physiological aspects, Comparative analysis, Medical screening, Health screening, Lung diseases, Genetic disorders, Pancreatic diseases

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Screening newborns for inborn errors of metabolism by tandem mass spectrometry

Article Abstract:

Tandem mass spectrometry can be used to identify newborn babies who may have a metabolic disease. This technique measures the amount of amino acids and other substances to detect any unusual amounts that could be caused by a metabolic disease. However, many of these babies would have no symptoms and it is not clear whether they would ever develop symptoms.

Analytical Laboratory Instrument Manufacturing, Analytical instruments, Ion Mass Spectroscopy, Mass spectrometry, Metabolism, Inborn errors of, Inborn errors of metabolism

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