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Biomedical subjects

S O Lie

Publications and source records attributed to S O Lie.

At least 91 records · Page 5Linked to original sources
PubMed1985

Net protein utilization determined by rat bioassay of a protein hydrolysate and a diet for children with phenylketonuria.

In a previous study (Kindt et al. 1983, 1984) it was assumed that a protein hydrolysate, devoid of phenylalanine, together with intact protein as given to children with phenylketonuria (PKU), was equivalent to egg or milk protein. One group of children was given this 'PKU protein' in amounts corresponding to the Joint FAO/WHO ad hoc Expert Committee (1973) recommendations. The results indicated that the Joint FAO/WHO ad hoc Expert Committee (1973) recommended levels of protein intake were marginal. The purpose of the present study was to evaluate whether the quality of the protein hydrolysate, together with intact protein ('PKU protein'), is equivalent to egg or milk protein. This was done using a rat bioassay. Four protein sources were used: (1) egg protein, (2) protein hydrolysate, (3) protein hydrolysate diluted with non-essential amino acids, (4) protein hydrolysate mixed with food proteins ('PKU protein'), comparable with the diet previously used (Kindt et al. 1983, 1984). The results indicated that the 'PKU protein' was of very high quality: net protein utilization (NPU) greater than 90. The protein hydrolysate alone and the protein hydrolysate diluted with non-essential amino acids gave a NPU greater than 80. The conclusion drawn from the present study is that the 'PKU protein', as used in the treatment of children with PKU, is equivalent to egg or milk protein. This supported the view that the Joint FAO/WHO ad hoc Expert Committee (1973) recommended levels of intake were marginal.

Animals↗
PubMed1985

Inhibitory activity in mouse lung-conditioned medium studied in the agar assay for bone marrow colony-forming cells: removal by vitamin C.

The growth of granulocyte/macrophage colony-forming cells (CFC) from bone marrow, with mouse lung-conditioned medium (LCM) as source of colony-stimulating factor, was tested in five strains of mice. Maximum stimulation of growth was usually obtained at a LCM dilution of 1:5 (final concentration, 1.8% vol/vol). Higher concentrations nearly always caused inhibition. The inhibitory activity was resistant to heating (56 degrees C, 30 min) and was not removed by dialysis, but could be extracted with chloroform, suggesting a lipophilic inhibitor of high molecular weight. On gel filtration (Sephadex G50) the stimulatory activity eluted in the first fraction, whereas the inhibitory activity could not be recovered. Ascorbic acid reduced or prevented the inhibition (optimum concentration, 0.45 mmol/l), and provides a convenient means of stabilizing the CFC agar assay system. In physiological conditions the vitamin might influence the responsiveness of CFC to growth-regulating factors.

Animals↗
PubMed1985

Deficiency of fumarylacetoacetase without hereditary tyrosinemia.

A variant of the enzyme fumarylacetoacetase (FAH) (E.C.3.7.1.2) in healthy individuals, determined by the enzyme activity, is reported. Analysis of family members of probands with low FAH activity suggests that the enzyme variant causing low activity could be the product of a pseudodeficiency gene. Assumed homozygotes for this gene have only slightly higher enzyme activity than patients with the metabolic disorder hereditary tyrosinemia I (hepatorenal type). No clinical abnormalities have been found in association with the postulated gene.

Adult↗
PubMed1985

Natural killer cell activity of peripheral blood and bone marrow mononuclear cells from patients with childhood acute lymphoblastic leukemia.

Patients with untreated acute lymphoblastic leukemia had highly significantly reduced natural killer cell activity in peripheral blood (p less than 0.01) and bone marrow (p less than 0.001) mononuclear cells compared with that in peripheral blood mononuclear cells in normal healthy controls. Patients in remission had normal killer cell activity (p greater than 0.5) both in peripheral blood and bone marrow mononuclear cells. Thus, the natural killer cell activity correlated well with the disease activity in the patients.

Adolescent↗
PubMed1985

Vaccination of children with malignant disease against varicella.

Nineteen seronegative children and one young adult with malignant disease in remission and on maintenance chemotherapy were immunized with the Oka-strain live attenuated varicella vaccine (Varilrix). Side effects were moderate and a rash was seen in 50% of the patients after vaccination. Humoral immune response to the vaccine was tested by the fluorescent antibody to membrane antigen (FAMA) test, a simpler indirect immunofluorescence test (IFT), and an enzyme-linked immunosorbent assay (ELISA). The seroconversion rate after immunization varied according to the method used. Seven out of 8 responded by FAMA, 15 out of 20 by IFT, and 12 out of 20 by ELISA. A decline in post-vaccination varicella-zoster virus (VZV) antibodies was seen in some responders. In 2 children, detectable levels of passively transferred VZV antibodies at the time of vaccination, due to varicella-zoster immune globulin, may have interfered with or modified the response to the vaccine. Cross-reacting antibodies to herpes simplex virus may have possibly interfered with vaccine response in a third child. Six out of 8 children receiving a second vaccine dose showed a good serological response. Specific cell-mediated immune response to the vaccine, measured by lymphocyte proliferation tests, corresponded well with the humoral response in the initial study of 8 patients. Two children who had responded to the vaccine were exposed to varicella in their families without contracting the clinical disease.

Adolescent↗
PubMed1984

Is phenylalanine requirement in infants and children related to protein intake?

Two groups of children with phenylketonuria (PKU) received protein at two different levels. The protein source was a protein hydrolysate, devoid of phenylalanine, and intact protein from milk, vegetables and fruit. One group (RDA group) was given protein at a level based on the recommendations of the (US) Food and Nutrition Board (1974, 1980). The other group (FAO group) was given protein at the level of intake corresponding to the Joint FAO/WHO ad hoc Expert Committee (1973) safe levels of intake of egg or milk protein. The children were monitored very closely for several years. From an earlier study evaluating the protein intake of the two groups it was suspected that the Joint FAO/WHO ad hoc Expert Committee (1973) recommendations were marginal. In the present study the phenylalanine intake of the two groups required to maintain the plasma phenylalanine concentration at the required level was established. The results showed that the RDA group required more phenylalanine than the FAO group. This difference was statistically significant from the age of 5-15 months. We have interpreted the greater requirement for phenylalanine in the RDA group as a result of a greater nitrogen intake and thus a more rapid chemical maturation of N (increase in protein concentration of the body with age). It is known that up to the age of 6 months the chemical maturation of N is related to the N intake. In the present study we have found that this difference in chemical maturation lasted up to the age of 15 months.(ABSTRACT TRUNCATED AT 250 WORDS)

Body Weight↗
PubMed1984

Immunoblastic lymphadenopathy with early onset in two boys: immunohistochemical study and indication of decreased proportion of circulating T-helper cells.

Two boys, 5 and 18 years old, had had frequent infections from their second month of life and had later suffered from generalized lymphadenopathy, intermittent maculopapular exanthema, anaemia, and weight loss. Histopathological features of lymph nodes were consistent with immunoblastic lymphadenopathy. Immunohistochemistry showed hyperimmune state with numerous T cells and macrophages along with selective polyclonal IgM-producing cell response in one patient, and IgG- and IgM-producing cells in the other. Both had severe deficiency of serum IgA. Classification of T-cell subsets (OKT4+ and OKT8+) in peripheral blood indicated an imbalance with a relative excess of putative suppressor cells (OKT4+/OKT8+ less than 1; normal, 1.7). It is suggested that decreased T-helper cell activity may explain the observed maturational defects in the B-cell system which probably underlie a hyper-reactive state induced by some sort of triggering, e.g. chronic infection. During steroid treatment the general condition improved remarkably and the lymphadenopathy and hepatosplenomegaly decreased, but the T-cell subset proportions and the serum immunoglobulin levels remained unchanged.

Child, Preschool↗
PubMed1984

The great majority of childhood lymphoblastic leukaemias are identified by monoclonal antibodies as neoplasias of the B-cell progenitor compartment.

36 acute leukaemias in children, 24 lymphoblastic and 14 myelogenous, have been examined with a set of 10 monoclonal antibodies by indirect fluorescent staining. In the lymphoblastic group the leukaemic cells of 4 children were found to have T-cell phenotype, while 19 of the other 20 T-cell phenotype negative cases were found to be positive for the c-ALL antigen. All 20 were negative for surface immunoglobulin as well as cytoplasmic mu-heavy chains. However, 17 (85%) reacted positively with the monoclonal antibody AB-1 which we have developed against a B-cell lymphoma, thus revealing B-lineage specificity. Another B-lineage-associated antibody (AB-2) reacted with 8/20 (40%) of the cases with distribution similar to B-1. These findings suggest that the great majority of non-T-non-B acute lymphoblastic leukaemias are neoplasms derived from the B-cell progenitor compartment. Moreover, monoclonal antibody testing allows further sub-categorization in this group. Similarly the acute-myelogenous leukaemia group could be subdivided into phenotypic subsets. The importance of using panels of monoclonal antibodies in the diagnosis of acute leukaemias is discussed.

Adolescent↗
PubMed1984

Vitamin A and/or high-dose Ara-C in the maintenance of remission in acute myelogenous leukaemia in children?

8 children with acute myelogenous leukaemia were brought into remission with a induction regimen containing cytosin arabinoside (Ara-C), 6-thioguanine and adriamycin given as the DNA complex. After remissions were obtained, the children were consolidated with high-dose Ara-C (2 g/m2 every 12 h for 6 doses) and were given 50 000 IU of vitamin A daily as an inducer of differentiation. All children are in continuous relapse-free survival for periods of 5 to 29 months (mean 14 months).

Child↗