Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: EC:3.2.1.23 (beta-galactosidase)
 14,648 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

To assess the advisability of using lactose-containing formulas in the rehabilitation of severely malnourished children, indices of clinical recovery, growth and restoration of body proteins and gastrointestinal function were measured longitudinally during the initial 45 days of hospitalization in 20 male, preschool children with kwashiorkor and marasmic-kwashiorkor. All patients received a diet based on cows' milk, but half were allocated to a formula pretreated with beta-galactosidase to hydrolyze the lactose, while the others received the untreated, intact milk. The groups were identical with respect to clinical criteria on admission. For the final 37 days of the protocol, the subjects received 4 g of protein and 150 kcal of energy per kg per day. More diarrhea was experienced by the intact lactose group during early hospitalization. Overall, recovery was satisfactory in both cohorts, and there were no differences in rates of growth, body protein repletion, restoration of energy reserves nor intestinal functions. In conclusion, the routine reduction of lactose content from a milk-based diet for severe protein-energy malnutrition offers no advantages.
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PMID:The effect of dietary lactose on the early recovery from protein-energy malnutrition. I. Clinical and anthropometric indices. 643 94

Protein energy malnutrition (PEM) is common in underprivileged populations in many parts of the world and results from diets deficient in protein (kwashiorkor) or protein and calories (marasmus). The literature documents renal tubular abnormalities in children with PEM. In PEM the reabsorption of amino acids and phosphate is defective. In many kidney disorders in which renal tubular function is impaired (e.g., diabetes, preeclampsia, nephrotic syndrome, sickle cell anemia), lysosomal enzymuria ensues. We compared the urinary excretion of the following five lysosomal enzymes in 31 Nigerian children with marasmus, kwashiorkor, or marasmic-kwashiorkor: beta-hexosaminidase, alpha-galactosidase, beta-galactosidase, beta-glucuronidase, and alpha-mannosidase. All of the protein energy malnourished children and the 18 age- and gender-matched controls were from the city of Jos, located in central Nigeria. In the severely malnourished children, the urine levels of all five lysosomal enzymes (expressed as units of enzyme activity per mg creatinine) were markedly increased. The greatest increases were seen with beta-hexosaminidase (16-fold) and beta-glucuronidase (14-fold). Routine clinical analyses also revealed that, relative to the control population, the sera of the 14 most severely malnourished patients contained 2- to 5-fold more vitamin B12 and markedly reduced levels (15%, p < 0.00001) of calcium. These data are significant in that they document lysosomal enzymuria in Nigerian children with severe PEM and point to the potential diagnostic utility of the urinary beta-galactosidase determination for assessing renal function in children with this disorder.
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PMID:Lysosomal enzymuria in protein energy malnutrition. 948 33