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Biomedical subjects

J K Lloyd

Publications and source records attributed to J K Lloyd.

At least 37 records · Page 2Linked to original sources

Oral vitamin E supplements can prevent the retinopathy of abetalipoproteinaemia.

Six patients with abetalipoproteinaemia are described who received large doses of oral vitamin E for between 12 and 18 years in addition to a low fat diet and supplements of the other fat soluble vitamins. The progressive retinopathy observed in untreated abetalipoproteinaemia was substantially modified and most probably prevented by this therapy. Angioid streaks were noted in one patient. Treatment with vitamin A alone did not prevent or arrest the progression of the retinal lesion.

Abetalipoproteinemia↗

The role of vitamin E in the treatment of the neurological features of abetalipoproteinaemia and other disorders of fat absorption.

Studies in patients with abetalipoproteinaemia and other chronic and severe fat malabsorptive states, and neuropathological studies in the vitamin E-deficient human, monkey and rat indicate that vitamin E is important for normal neurological function. Appropriate vitamin E supplementation is, therefore, advisable for all patients with chronic fat malabsorption who have low serum vitamin E concentrations.

Abetalipoproteinemia↗

Double blind trial of bezafibrate in familial hypercholesterolaemia.

A six month, double blind, crossover controlled trial of bezafibrate was conducted in 14 children with familial hypercholesterolaemia all of whom had a strong family history of early coronary heart disease. The bezafibrate was given twice daily in a dose of 10 to 20 mg/kg/day. The mean plasma total cholesterol concentration on bezafibrate was 22% lower than during the period on placebo and there was a moderate rise in high density lipoprotein cholesterol. Bezafibrate may be a useful adjunct to treatment in these children.

Adolescent↗

Vitamin E and neurological function.

Three lines of evidence indicate that vitamin E is important for normal neurological function in man. First, in abetalipoproteinaemia early therapy with vitamin E delays, and may prevent, the development of neurological complications, and in patients with established lesions treatment can arrest or reverse the neuropathy. Secondly, in other chronic disorders of fat absorption with severe vitamin E deficiency, neurological manifestations can be improved by vitamin E. Thirdly, the neuropathological changes observed in vitamin-E-deficient states in man (such as abetalipoproteinaemia, chronic liver disease, and cystic fibrosis) are similar to those reported in vitamin-E-deficient rats and monkeys.

Abetalipoproteinemia↗

Vitamin E and neurological function: abetalipoproteinaemia and other disorders of fat absorption.

Evidence that vitamin E is important for normal neurological function in humans is presented. First, in abetalipoproteinaemia early therapy with vitamin E delays and may prevent the development of the neurological complications, and in patients with established lesions treatment can arrest or reverse the neuropathy. Second, in other chronic disorders of fat absorption with severe vitamin E deficiency, neurological manifestations which are very similar to those described in untreated abetalipoproteinaemia can be improved by vitamin E. Vitamin E supplementation is therefore advisable for all patients with chronic fat malabsorption who have low serum vitamin E concentration. Serum vitamin E concentrations should also be measured in patients with spinocerebellar disorders, whatever the aetiology.

Abetalipoproteinemia↗

Long-term follow-up of children with familial hypercholesterolaemia treated with cholestyramine.

35 children with the heterozygous form of familial hypercholesterolaemia (FH) who were followed for up to 8 years after starting on cholestyramine treatment showed a progressive decrease in compliance with therapy with time, so that only 55% remained on treatment after 6 years and only 48% after 8 years. Long-term compliance was significantly better in those starting treatment before age 10. Plasma-cholesterol was lowered in all children taking cholestyramine: for the group the mean reduction in plasma-cholesterol ranged from 26 to 44% during the years of the study, on a mean cholestyramine dose of around 0.4 g/kg/day. Cholestyramine thus effectively lowers plasma-cholesterol in FH, but for long-term use compliance is a problem in many children.

Adolescent↗

Disturbance in parent-child relationship following preterm delivery.

Increasing concern is expressed that the psychosocial development of preterm infants may be hindered by a disturbance of parental attitudes following the initial period of specialised care. Attitudes of parents of 17 preterm infants were compared with those of parents of 17 full-term infants at a single semi-structured interview six to 20 months after the birth. The groups of parents were matched for parity and did not differ in their social, ethnic and educational backgrounds. There was evidence of some disturbance in parent-child relationships in the preterm group, consisting of delays in maternal attachment to the child, negative maternal perception of the child compared with expectation of an 'average' baby, and persistent parental anxiety about leaving the child with a baby-sitter. In addition, two preterm children had been abused or neglected. Parental reaction to a preterm birth is discussed and the need for adequate support to be given to parents in the early period following the birth is stressed.

Anxiety↗

Surface binding and catabolism of low-density lipoprotein by circulating lymphocytes from patients with abetalipoproteinaemia, with observations on sterol synthesis in lymphocytes from one patient.

Surface binding of low-density lipoprotein (LDL), degradation of LDL protein and sterol synthesis were investigated in freshly isolated lymphocytes from normal and abetalipoproteinamic human subjects. LDL binding as a function of LDL concentration showed no evidence of the presence of high-affinity binding sites in fresh lymphocytes from either group of subjects. The rate of degradation of LDL protein by lymphocytes from the patients was no greater than that from the normal subjects and, in the fresh lymphocytes of the one patient studied, sterol synthesis was not increased. We conclude that the formation of LDL receptors and the synthesis of sterol in circulating lymphocytes are largely suppressed and that in normal subjects this may be due to the presence of some plasma constituent other than LDL, possibly the apoE protein. This conclusion is discussed in relation to the possible contribution of LDL receptors to the degradation of LDL protein in vivo.

Abetalipoproteinemia↗

Sterol balance in a patient with abetalipoproteinaemia.

Total-body cholesterol synthesis was measured in a woman with abetalipoproteinaemia and in a normal woman of similar age. The rate of synthesis of cholesterol was 15.4 +/- 4.1 mg/kg/day in the patient and 14.3 +/- 2.6 mg/kg/day in the control subject, indicating that cholesterol synthesis in the whole body is not increased in the complete absence of plasma low density lipoprotein.

Abetalipoproteinemia↗