No abstract

In a follow-up matched control study the 93 (70.5%) survivors of 132 children treated with a national protocol for acute lymphoblastic leukemia (ALL) and 5 survivors of the other 21 cases of ALL in childhood diagnosed in the same period were evaluated. Thus it was also a population-based study. The national treatment protocol was used in the period 1975-1980. Methotrexate (MTX) infusions combined with intrathecal MTX were used as prophylaxis against neuroleukemia instead of irradiation. Neither doxorubicin (Adriamycin) nor cyclophosphamide was used in the protocol. A questionnaire covering demographic data, number of offspring, learning problems, level of athletic performance, education, and work status as well as medical information was used. Forms were received from 94 (96%) of the 98 adult surviving cases and corresponding controls in the family. Interviews were performed in the remaining four cases (4%). There were no statistical differences between the two groups with respect to physical and mental health and quality of life. Hospital records of all patients were also checked for possible late effects. There was no definite case of secondary malignant neoplasm; however, there was one case of prolactinoma and only one case of serious sequelae (hemiparesis during therapy), probably due to intrathecal and intravenous MTX.

N-3 fatty acid deficiency is described in a 7-year-old girl who had been fed solely by gastric tube from the age of 3 years. She had received the same nutrient during the last 22 months, supplying 16.2% of calories from linoleate and 0.07% from alpha-linolenate. She weighed 9.5 kg at the age of 3, and 10.3 kg at the start of the study, the weight being constant the last 15 months. She was now supplemented daily with 1.2 ml of a linseed- and cod liver-oil mixture (5:1, v/v), thereby increasing her alpha-linolenate intake to 0.71% and total N-3 fatty acids to 0.74% of total energy. Two months later, her rate of weight gain was 0.43 kg/month. After 5 months, the fatty acid supplement was changed to 7.5 ml of cod liver oil daily, thereby changing alpha-linolenate and total N-3 acid intake to 0.10% and 1.3% of total energy, respectively. Her rate of weight gain now increased to 0.64 kg/month, and her length increased from 117 to 122 cm in 5 months. The results indicate that the optimal dietary requirement for N-3 fatty acids in this child is higher than 0.74%, and extrapolation estimated the optimal requirement to be 1.1%-1.2% of total calories. The results suggest that N-3 fatty acids are required for normal growth in man.

Abstract. A family with two patients, a brother and a sister, afflicted with familial lecithin:cholesterol acyltransferase deficiency is described. The patients had corneal infiltration, normochromic anemia, and proteinuria. No lecithin:cholesterol acyltransferase activity could be demonstrated in their plasma. They had high plasma concentration of unesterified cholesterol, lecithin and triglyceride, and low concentration of cholesteryl ester, which acyl groups were mainly saturated and mono‐unsaturated. Paper electrophoresis of plasma lipoproteins showed absence or highly reduced levels of α‐ and pre‐β‐lipoproteins. The erythrocytes contained increased amounts of cholesterol and lecithin, and decreased amounts of sphingomyelin and phosphatidylethanolamine. Post‐heparin lipolytic activity, tested in one of the patients, showed values within the normal range. No relevant abnormalities were found in the parents of the patients or in their only sib, a healthy female.

This study included all 690 children in Norway diagnosed as having acute lymphocytic leukemia (ALL) from July 1975 till the end of 1997. Relapses and deaths were monitored until the end of 2000. Neuroleukemia prophylaxis was intravenous methotrexate (MTX) infusions as intermediate-dose methotrexate (IDM) or high-dose methotrexate (HDM) combined with intrathecal MTX. From 1992, systemic therapy was considerably intensified, and, in addition, patients in a subgroup of the high-risk and very high-risk groups were given prophylactic cranial irradiation. The overall findings showed that MTX significantly reduced central nervous system (CNS)-related relapses, and, in general, reinforced systemic therapy reduced significantly non-CNS relapses and deaths. The overall crude survival was 75%. During the study period, the crude survival improved for patients on standard protocols from initially 65 to 90%. Forty patients (6%) developed isolated CNS relapse, 27 (4%) had combined CNS relapse, whereas 180 (26%) had non-CNS relapse. When IDM and HDM were compared, the cumulative risk for isolated CNS relapse was significantly lower with HDM, 12 and 5%, respectively. For any relapses that involved the CNS, the risk remained significantly lower for HDM, 8 versus 18%. Of the 40 patients with isolated CNS relapse, 23 survived (58%).