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

Percentiles of blood pressure of Egyptian children and adolescents [3-17 years]

Blood pressure [BP] percentiles for Egyptian children and adolescents provide representative Egyptian BP levels and their use may be essential. The present study aimed to determine and describe levels of systolic blood pressure [SBP] and diastolic blood pressure [DBP] across age and gender of 22,072 healthy children and adolescents to drive reference percentiles for BP of Egyptian children and adolescents. It also aimed to assess the association between age, sex, height and BMI with BP in healthy Egyptian children and adolescents. The study is a cross-sectional one, including 22,072 healthy Egyptian children and adolescents [10997 boys and 11075 girls] at age range of 3 to 17 years from nurseries and private schools in Cairo and Giza Governorates. A questionnaire was designed including: Full medical and family history and full clinical examination and Height, weight and BP measurements for all subjects. The study results revealed that both systolic BPand Diastolic BP were significantly higher in Egyptian children compared to other ethnic groups and that BP is influenced by gender, age and height and body mass index [BMI]. The study recommends development of national strategy to reduce overweight and obesity during childhood

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

cytogenetice and immunologic studies in recurrent fetal losses.

The aim of this study was to evaluate the cytogenetic and immunologic role in human reproduction. The study included 158 women with history of repeated fetal losses, their ages ranged between 18-45 years [mean = 31 years] and the number of previous losses ranged between 2-8 [mean = 4]. The clinical evaluation, pedigree construction, pelvic ultrasound examination was done to all cases. Chromosomal studies were done to 66 couples with history of early fetal losses [during 1st trimester] using GTG-banding technique. A total number of 120 pregnant women with history of fetal losses and 30 normal pregnant women used as controls was subjected to a further immunologic investigation. Anticardiolipin antibodies were measured using ELISA and lupus anticoagulant [LA] by activated partial thromboplastin time [APTT]. The study concluded that both genetic and immunologic factors have been identified as causes of repeated fetal losses. However, chromosomal aberrations play an important role in the etiology of early fetal losses