Children with cystic fibrosis (CF) often have a poor vitamin A status. We found in the present work a reduced level of serum retinol in older children with this disease. As retinol enters intestinal lymph as retinyl esters, the enzyme acyl-CoA:retinol acyltransferase (ARAT) may be of importance for retinol absorption. We have assayed ARAT activity in duodenal mucosal homogenate from children with CF. There was a large variation within the group. However, mean ARAT activity was not significantly decreased, as compared with controls. Thus, the lack of pancreatic enzymes probably is the main reason for reduced vitamin A absorption in CF. In celiac disease and in lactose intolerance--both mucosal disorders--a significant reduction of mean ARAT activity was found.

Acyltransferases↗

PubMedDate not supplied

Central nervous system disease in childhood acute lymphoblastic leukemia: prognostic factors and results of treatment.

In the five Nordic countries, 808 children 1 to 15 years of age (428 boys, 380 girls) were diagnosed with non-B acute lymphoblastic leukemia (ALL) from July 1981 through June 1986. Complete remission was achieved in 770 children (95%). Central nervous system (CNS) involvement at diagnosis was noticed in 34 children, of whom 26 achieved remission. Of these 26 patients 11 subsequently relapsed, 5 in the central nervous system. An interim analysis in January 1990 (observation time 3 1/2 to 8 1/2 years) revealed that isolated CNS relapse had occurred in 70 children (9.0%). Of these 70 patients, 12 out of 142 children (8.5%) had initially received irradiation and 58 out of 628 children (9.2%) only chemotherapy as CNS-prophylaxis. There was a significant higher risk for boys (12%) than for girls (6%) to relapse in the CNS compartment. Unfavorable prognostic factors for survival after isolated CNS relapse were short duration of first remission and male sex. In high-risk patients after an isolated CNS relapse, there was no difference in prognosis related to treatment with or without irradiation as initial CNS prophylaxis.

Antineoplastic Combined Chemotherapy Protocols↗

PubMedDate not supplied

Long-term cerebral effects of CNS chemotherapy in children with acute lymphoblastic leukemia.

Fifteen 7-16-year-old patients, treated in 1981-1984 for acute lymphoblastic leukemia (ALL) in first complete remission, were studied. As a central nervous system prophylaxis, all the children were treated with repeated methotrexate (MTX) instillations, but none were irradiated. The study protocol included magnetic resonance (MR) and a battery of neuropsychological tests. Small, punctate white-matter lesions were found by MR in eight children, probably minor vascular lesions. All the children were within normal intelligence range with a mean total WISC-R IQ of 109. Minor neuropsychologic problems were found in two patients, while one child showed a more extensive specific learning disorder in school.

Adolescent↗

PubMedDate not supplied

Evaluation of retinyl palmitate and cytarabine treatment in a slowly growing rat leukemia model.

The slowly effect of retinyl palmitate in prolonging survival time in a slowly growing rat promyelocytic leukemia model (BNML) was tested. Retinyl palmitate was given in addition to cytarabine, which has previously been shown to prolong survival time in these rats. The effect of cytarabine on liver store and plasma level of retinol was also tested. The results show that retinyl palmitate did not prolong survival time in the leukemic rats. Similarly, cytarabine did not reduce the level of retinol in liver or plasma.

Animals↗