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Biomedical subjects

H A Lunde

Publications and source records attributed to H A Lunde.

6 recordsLinked to original sources
PubMed1990

Inborn errors of carnosine and homocarnosine metabolism.

Serum carnosinase deficiency with carnosinuria has been reported in 23 children with neurological signs and/or mental retardation. In adults four cases in one family had serum carnosinase deficiency, carnosinuria, and in addition elevated homocarnosine in CSF and in the brain. The mother was one of these cases but had no clinical symptoms; however her three children have spastic paraparesis, retinitis pigmentosa and mental retardation. Serum carnosinase deficiency alone is not the cause of the neurological symptoms. When two of the affected children consumed carnosine, anserine or homocarnosine, they metabolized these compounds much less rapidly than did two normal control individuals.

Adult↗
PubMed1988

Fasting plasma amino acid concentrations in PKU children on two different levels of protein intake.

Two groups of children with phenylketonuria were treated with different amounts of protein. One group (RDA group) received protein as recommended by the U.S. Food and Nutrition Board, the other (FAO group) the amount recommended by the FAO/WHO 1973 ad hoc Expert Committee. Two of the children in the FAO group showed a decrease in length/growth velocity which raised the question of the adequacy of the protein content in the diet. Since deviations from normal plasma amino acids have been observed in protein deficient, energy-adequate diets, we examined these parameters in the PKU children. In the two children with retardation in length/growth, very high glycine values as well as very high alanine values were observed at the end of the study. These observations strengthened our suspicion that the FAO/WHO 1973 recommendations are marginal.

Amino Acids↗
PubMed1986

Homocarnosinosis: influence of dietary restriction of histidine.

Homocarnosinosis, an inherited disorder, is characterized by an elevated level of the dipeptide homocarnosine (Hca) in the CSF and the brain and, in addition, by carnosinuria and serum carnosinase deficiency. In three children with homocarnosinosis the biochemical aberration co-exists with paraplegia, retinitis pigmentosa, and a progressive mental deficiency. In the mother, however, only the biochemical aberration was present without clinical symptoms. In order to study whether this elevated level of Hca and increased excretion of carnosine (Car) could be reduced towards normal, a dietary regiment with restriction of histidine (His) was maintained for nearly 2 1/2 years for two of the patients, 33 and 39 years old, with homocarnosinosis associated with neurological symptoms. His was reduced by about 90% in the CSF, in the plasma and in the urine. Within 5-6 months CSF Hca was reduced by about 70%, and urinary Car by 22 and 42%. The clinical neurological symptoms, however, did not alter significantly together with these biochemical changes.

Adult↗