Nutritional status of some micronutrients and trace elements in patients with phenylketonuria

Children with special needs; those having inborn errors of metabolism are prone to certain micronutrient deficiencies either due to rigid therapeutic dietary restriction, aversion to certain food stuffs or due to recurrent episodes of vomiting and diarrhea. This will eventually ensue if no parallel specific elemental replacement measures are carried out. Micronutrient deficiencies often occur concurrently, and there are many interactions between micronutrients. Children with phenylketonuria [PKU] are treated with semi-synthetic formula containing low phenylalanine [Phe] content. It provides the majority of protein and energy in the diet while the rest of phenylalanine are met by low protein natural foods. Because of the restricted intake of high biological value protein, children with PKU are often expected to have lower than normal plasma concentrations of certain micronutrients. The aim of the study is to assess the effects of phenylalanine restricted diet on the growth of our PKU patients after one year of dietary management. We also aimed to investigate the nutritional status of the following trace elements and micro-nutrients; zinc, copper, vitamins C, E, A, and B-carotene among 17 PKU patients following dietary coupled with multivitamin supplementation. Data were compared to those of their matched normal controls. PKU patients were found to have marginal vitamin A deficiency as the mean plasma level was 25.2 +/- 6.62ug/dl versus 48.5 +/- 10.1ug/dl of their control. The difference was very highly significant [p<0.0001]. Mean plasma levels of B-carotene was also less than that of controls with a highly significant difference [50.65 +/- 15.37ug/dl Vs.-75.80 +/- 19.60ug/dl; p<0.001]. Mean plasma levels of zinc, copper, vitamins C and E were comparable to those of controls without statistical significance [p>0.05]. Physical growth parameters were not significantly different between the two groups. A basic nutritional care plan for dietary intervention managing PKU children should involve periodic assessment of protein and calorie nutritional status in addition to regular careful evaluation of micronutrient nutritional status

therapeutic role of vitamin E and C on non syndromic sensorineural hearing loss

Antioxidants help in preventing further degeneration or possibly even reverse degenerative processes after they have been activated. Free radical scavengers, such as vitamin E and vitamin C, protect inner ear from oxidative damage. To evaluate the efficacy of two classes of agents found effective in preventing sensorineural hearing loss [antioxidants and low fat diet] and determine if it can improve the degree of hearing loss by preventing oxidative damage. fifty four patients were divided into two groups, twenty four patients were given vitamin E and C for two months and thirty patients were given low fat diet [without animal fats] for two months. Auditory function was monitored along with plasma concentrations of Beta carotene, vitamins A, E and C and lipid peroxidation. Also, zinc, copper, calcium, phosphorus and magnesium together with immunoglobulin G, M and A levels were estimated before and after treatment. Significant increase in antioxidants and decrease in lipid peroxidation was observed in the group which was given vitamin E and C. The addition of antioxidants significantly enhanced the evoked responsiveness over that observed with the low fat diet group indicating a putative role of antioxidants in the pathogenesis of sensorineural hearing loss. The findings showed that supplemental dietary antioxidant may have a beneficial role in the prevention of deafness. It also proved a relationship between low fat diet and improvement of hearing loss

Oxidative stress in nonsyndromic sensorineural hearing loss

Ototoxicity seems to result from the inhibition of cochlear antioxidant defenses, causing an increase in the amount of reactive oxygen species. These findings suggest a causal relationship between the formation of reactive oxygen species, oxidative stress and functional/morphological ear damage. auditory function was monitored along with plasma concentrations of copper, zinc, calcium, phosphorus, magnesium and iron. Further more, lipid peroxidation, beta carotene, vitamin A, E and C activities and immunoglobulin G, M and A levels were estimated. A significant decrease in calcium, phosphorus, beta carotene, and vitamin E activities as well as low levels of immunoglobulin G and M were found. The observed increase in lipid peroxidation is indicative for oxidative stress which suggests a putative role of antioxidants in the pathogenesis of sensorineural hearing loss. Conclusion: The results support the hypothesis that dietary and immune factors influence individual susceptibility to hearing loss. Further studies are needed to verify whether antioxidants, correction of deficient nutrients and/or immune modulation would improve sensorineural hearing loss

effect of diet on antioxidant status in patients with galactosemia

Galactosemia is an autosomal recessively inherited disorder of galactose metabolism. It has good prognosis, if detected in neonatal period or early infancy. Treatment consists of life long dietary restriction of galactose. Present study included eight patients with galactosemia on dietary treatment, five of them had galactose-1-phosphate uridyltransferase deficiency known as classical galactosemia and three had uridine-diphosphate galactose-4' epimerase deficiency. Clinical evaluation of patients under galactose restricted diet and assessment of the antioxidant status in response to dietary therapy was done. Delayed milestones were present in all patients, jaundice at birth was present in 4 and low birth weight was present in 3 patients. Craniofacial dysmorphism was present in 5 patients. Hepatomegaly was present in 6 patients. MRI of the brain showed brain atrophy in 3 patients and demyelination in 2 patients. There was cataract in 7 patients. The levels of zinc, copper, iron, calcium, phosphate, magnesium, selenium, manganese, beta-carotene and vitamin A were evaluated in the blood of galactosemic patients on galactose restricted diet and a comparison between trace elements, beta-carotene and vitamin A in studied patients with galactosemia and controls was done. Copper, calcium, phosphate, manganese and beta-carotene levels in blood were significantly decreased in our patients [p<0.001] than in controls. These findings suggest that patients on galactose restricted diet are at risk of oxidative stress. The data emphasize the importance of dietary supplementation with an antioxidant containing beta-carotene, calcium, copper, selenium and manganese to inhibit oxidative stress in these patients. Consequently this will minimize the neurological deficits improve bone mineralization, reduce the development of retinopathy and damage to liver cells in patients with galactosemia

effect of diet on antioxidant status in patients with galactosemia

Galactosemia is an autosomal recessively inherited disorder of galactose metabolism. It has a good prognosis, if detected in neonatal period or early infancy. Treatment consists of life long dietary restriction of galactose. Our study included eight patients with galactosemia on dietary treatment, five of them had galactose-1-phosphate unidyltransferase deficiency known as classical galactosemia and three had uridine-diphosphate galactose-4' epimerase deficiency. Delayed milestones were present in all patients, jaundice at birth was present in 4 and low birth weight was present in 3 patients. Craniofacial dysmorphism was present in 5 patients. Hepatomegaly was present in 6 patients. MRI of the brain showed brain atrophy in 3 patients and demyelination in 2 patients. There was cataract in 7 patients. The aim of this study was to asses the antioxidant status in response to dietary-therapy. The levels of zinc, copper and Iron, calcium, phosphate, magnesium, selenium, manganese, beta-carotene and vitamin A were evaluated in the blood of galactosemic patients on galactose restricted diet. Also, a comparison between trace elements, beta-carotene and vitamin A in studied patients with galactosemia and controls was done. Copper, calcium, phosphate, manganese and beta-carotene levels in blood were significantly decreased in our patients [p<0.001] than in controls. These findings suggest that treated galactosemic patients are at risk of oxidative stress and abnormal bone mineralization. Therefore, therapeutic intervention in these cases should be more appropriately targeted. The data emphasise the importance of antioxidants and trace elements in minimizing the neurological deficits in galactosaemic patients

Effect of passive smoking and familial essential hypertension on vitamins and lipid profiles in children

In this study we examined the effect of passive exposure of children with a family history of essential hypertension [CFHEH] to tobacco, on the lipid profite, vitamin E [Vit. E] and vitamin C [Vit. C]. This study was carried out on 39 passive smoker children of fathers with essential hypertension of both sexes [18 females and 21 mates], their ages ranged from 8-13 years. A group of 15 healthy children [7 females and 8 males] with the same age range and with no history of any disease was included as control group. Serum total lipids, triglycerides, cholesterol, high density Iipoproteins [HDL], low density lipoproteins [LDL], Vit, E and Vit, C were estimated in serum. A highly significant decreased in the levels of serum Vit. C and Vit. E [0.2 micro g/dt +/- 0.3 11 and 2.45 mg/dl +/- 0.152], respectively as compared to the control children [0.291 pg/dl +/- 0.113 and 3.491 mg/dl +/- 0.141], a significant increase in serum level of triglycerides and cholesterol in passive smoking children of fathers with family history of essential hypertension [250.34 mg/dl +/- 2.345 and 180,5 mg/dl +/- 3.45], respectively, as compared to the control children [152.75 mg/dl +/- 4.61 and 80.794 mg/dl +/- 4.9], respectively. No significant changes in the level of total lipids and HDL-cholesterol in passive smoking children of fathers with familial hypertension [589.833 mg/dl +/- 12.5 and 69.941 mg/dl +/- 5, 34], respectively, as compared to the control children [558.47 mg/dl +/- 13.8 and 63.333 mg/dl +/- 4.5], respectively. fidso, a significant increase in LDL-cholesterol in passive smokers of children of fathers with familial hypertension [52,323 mg/dl +/- 3.42] as compared to control children [31.666 mg/dt +/- 2.5]. It was concluded that passive smoking and a positive family history of essential hypertension are important risk factors for cardiovascular diseases