Diet of young children and cardiovascular disease.
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Biomedical subjects
Publications and source records attributed to L S Taitz.
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Fifty per cent of the offspring of adults with the adult (dominant) form of polycystic kidney disease are carriers of the abnormal gene. Clinical symptoms and signs before adolescence are rare, but renal ultrasonography may detect evidence of cyst formation. Twenty two children, all offspring of parents with known adult polycystic kidney disease, have undergone renal ultrasonography. In six cases evidence of disease was detected without clinical manifestations at the ages of 1, 2, 5, 8, 13, and 14 years. There were no renal masses, hypertension, haematuria, or evidence of renal insufficiency. In four children from three sibships, whose families had no previous history of renal disease, bilateral renal masses were noted to be present at birth. In each case one parent was subsequently found to have adult polycystic kidney disease. At the ages of 1, 4, 6, and 20 years, while renal masses were still palpable, there was no evidence of renal insufficiency or hypertension in the younger children, while the oldest had mild renal failure. An analysis of the reported cases in childhood is suggestive of a bimodal distribution of enlarged kidneys, with a number of cases diagnosed at birth or soon after, followed by an increasing incidence during later childhood. Adult polycystic kidney disease presenting at birth may be qualitatively different from the disease detected by screening programmes of children at risk.
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Does manipulation of a child's diet in the first five years of life result in a reduced prevalence of coronary artery disease in later life? The findings of a recently published study (Bogalusa) are compared with British recommendations. Suitable modifications of the under fives' diet are suggested.
A child of 10 months with proven homocystinuria was found to be selenium depleted and we report the serial monitoring of replacement therapy. Selenium, as an enriched yeast preparation, equivalent to 50 mug on alternate days was required for maintenance. Indices of selenium status returned to normal in varying times. The child initially demonstrated an abnormality of an in vitro challenge by hydrogen peroxide to her erythrocytes, that returned to within normal limits with selenium therapy alone, the serum vitamin E concentration being normal.
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Simple, incentive based behaviour modification, with or without a modest programme of psychotherapy involving outpatient visits every four to six weeks, seems to be associated with a useful cure rate in children with lower bowel function disorders. Appreciable social disadvantage seems to be the most important factor mitigating against a successful outcome, associated with non-compliance with treatment. Failure to respond to treatment was associated with important psychological problems. These were more common in the socially disadvantaged groups. Children from satisfactory social backgrounds who have lower bowl disturbances can be effectively treated by fairly simple programmes. More elaborate and expensive strategies should be reserved for those whose psychosocial circumstances make it possible to predict a less satisfactory outcome.
The biochemical, dermatological and neurological motor disorders of biotinidase deficiency (multiple carboxylase deficiency) show a dramatic response to pharmacological doses of biotin. This condition is characterised by the accumulation of biocytin and depletion of biotin. Neuromuscular function returns to normal with the reversal of the characteristic organic acidaemia. It would appear that the optic and auditory nerves or their related neurological structures may suffer damage from the excess biocytin and deficient biotin. Despite reversal of the dermatological and psychomotor abnormalities children are likely to be left with auditory and/or visual handicaps if diagnosis and treatment is delayed beyond the first year of life. Treatment with biotin was commenced 6, 18, and 13 months after onset of symptoms. Two children subsequently were found to have visual impairment (acquired retinal dysplasia) and two had sensori-neural deafness. In one patient both defects were present.
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Poor growth in association with child abuse is well recognised, but the eventual outcome with respect to growth has not been clearly defined. In a study of 95 children who had suffered child abuse standard deviation (SD) scores for height and weight were significantly below the mean at presentation and improved at follow up. Sixty four children who remained at home showed significant increase in height SD scores only (p less than 0.01). Twenty children were either taken into long term fostercare or adopted and showed significant increases in height and weight SD scores (p less than 0.001 and p less than 0.01, respectively). The remaining 11 children, who were fostered for short periods only, showed little change in either index. Catch up growth for height defined as a change in SD scores of one or more occurred in seven (11%) of the children at home compared with 11 (55%) of those taken into long term fostercare (p less than 0.001). Catch up growth for weight occurred in 14 (22%) of those at home and 10 (50%) of those in long term fostercare (p less than 0.01). Children suffering child abuse show greater catch up growth when taken into long term fostercare. Growth patterns should be used to decided where these children are placed.
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A case of haemolytic uraemic syndrome in a three year old boy is described. The clinical course was complicated by hyperglycaemia, and biochemical assay of plasma showed an almost total lack of pancreatic insulin. Extensive necrosis of the exocrine and endocrine pancreas was found at necropsy. The possible pathological physiology of this complication and its prognostic importance are discussed.
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Two cases of hypocalcaemia following removal of the stomach due to complications of surgery for tracheo-oesophageal anomalies are reported. In both instances grand mal seizures were the presenting features with radiological and biochemical evidence of rickets. Both children had elevated blood parathyroid hormone levels. The bone changes, hypocalcemia and symptoms responded to parenteral vitamin D therapy. Sub total gastrectomy is a rare event in infancy and consequently no previous cases of vitamin D lack has been recorded as a complication of gastric surgery in this age group.
Anorectal manometry and suction biopsy were carried out on 47 children with constipation or soiling, or both. Patients were divided into two groups. Group 1 (37 patients): functional faecal retention, group 2 (10 patients): functional faecal soiling without retention. Ganglion cells or normal acetylcholinesterase staining, or both, was demonstrated in all cases. Normal inhibition of internal sphincter could be achieved by rectal distension in all except 2 children with severe constipation. Resting sphincteric pressures, pressure responses, and conscious rectal sensitivity thresholds were similar in groups 1 and 2, but were increased compared with controls. In group 1 alone, the critical volume increased parallel with conscious rectal sensitivity threshold. Since the complete relaxation of internal sphincter occurs before conscious rectal sensation arises in children with soiling without retention, this may be an important factor, at least in some of the soilers.
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