Bone disease in upper Egyptian transfusion dependent pediatric B-thalassemia major

Thalassemia and the blood transfusion complications associated with it predispose children to poor bone health. This study was conducted to determine the prevalence of bone-related abnormalities within this population. Forty two transfusion-dependent beta thalassemic subjects 5-19 years old in Assiut, Egypt, participated in this cross-sectional study. Medical history by interview and review of medical records, physical examinations including anthropometric measures and puberty assessment, and bone densitometry using DEXA and height adjustment were performed. The incidence of underweight and short stature was 73.8% and 69%, respectively, which were indicators of malnutrition among thalassemic subjects in this study. Low bone density [BMD Z-scores < -2] was detected in the lumbar spine and femoral region in 54.7% and 95.2% of subjects, respectively. But after height adjustment they became 11.9% and 64.3%, respectively. Forty-seven percent of patients had a history of fractures and the cumulative risk for which increased almost with age. Seventy-eight percent of patients reported bone pain, whilst back/hip pain was present in 69% of thalassemic children and adolescents. Genu valgus deformity and scoliosis were seen in 40.5% and 11.9% of patients. High incidence of low bone density and deficit in other aspects of bone health among thalassemia patients makes routine bone health assessment necessary for this vulnerable group. Considering influencing factors, dietary counseling and preventive supplementation therapy for this high risk group of children and adolescents may be necessary, although this should be assessed by intervention studies

Protein C, protein S and antithrombin III in children with complicated congenital heart diseases

This study was carried out on 40 patients with congenital heart disease [CHD] aged three months to seven years and divided into two groups: The first included 28 patients with complicated CHD and the second group included 12 patients with uncomplicated CHD. Ten healthy children were chosen as controls. A coagulation profile consisting of protein C, protein S, antithrombin III, factor V and factor VIII were evaluated in all patients and controls. The mean values of protein C, protein S, antithrombin III, factor V and factor VIII were significantly decreased in patients with complicated CHD compared with both uncomplicated cases and controls. Eighteen out of twenty-two patients with complicated CHD had low protein C levels [2 standard deviations below the normal mean value of the controls]. Of these children, three developed thrombotic complications and eight had evidences suggestive of consumption coagulopathy [decreased factors V and VIII]. It was concluded that decreased levels of coagulation inhibitors protein C, protein S and antithrombin III were observed in ill children with complicated CHD. With reduced levels of protein C, children with complicated CHD might have a tendency toward thrombotic complications

Oxidant stress in children with iron deficiency anemia

The susceptibility to oxidant stress was evaluated in forty-one children with iron deficiency anemia [IDA] as well as ten healthy children as a control group. The levels of thiobarbituric acid reactive substances [TBARS] as a measure of lipid peroxides, the antioxidant enzymes catalase and glutathione peroxidase [GPX] in red blood cells [RBCs] and superoxide dismutase [SOD] in both RBCs and plasma together with the non-enzymatic antioxidants glutathione in both RBCs and plasma, vitamin C and beta-carotene in plasma were measured in all patients and controls. The study revealed significant decrease in the levels of catalase [7.6 +/- 2.3], GPX [14.4 +/- 2.0], glutathione in both RBCs and plasma [435.4 +/- 56.5, 368.8 +/- 123.7], vitamin C [0.24 +/- 0.15] and beta-carotene [78.1 +/- 25.2] in children with IDA compared with the healthy control group [11.34 +/- 3.0, 19.9 +/- 2.7, 636.4 +/- 54.3, 640 +/- 94.9, 1.4 +/- 0.3, 118.5 +/- 22.9, respectively]. Consistent with the reduced levels of enzymatic and non- enzymatic antioxidants is the significant increased levels of the lipid peroxidation marker [TBARS]in both plasma and RBCs in children with IDA compared with the control group. The study revealed increased vulnerability to oxidant stress in children with IDA

Urinary cortisol and minerals excretion in high risk newborns

Twenty four hours urine specimens have been analysed with regard to cortisol, sodium, potassium, calcium and phosphate in 48 Full-term and premature newborns. The mean values of 24 hours urinary cortisol in 10 uncomplicated premature newborns [12.45 +/- 5.8 ug/kg], in 16 complicated premature newborns [24.35 +/- 15.55 ug/kg] and in 12 complicated full-term [14.9 +/- 10.91 ug/kg] were found to be significantly higher than that in 10 uncomplicated full-tern newborns [6.68 +/- 2.76 ug/kg]. Significant negative correlations between cortisol excretion and gestational age were found in the group of normal full-term [r = -0.73, P < 0.02] and in the group of uncomplicated premature newborns [r = 0.81, P < 0.01]. Significantly higher levels of 24 hours sodium, potassium, calcium and phosphate urinary excretion were observed in uncomplicated preterm [2.43 +/- 1.14 m Eq/kg, 1.31 +/- 0.31, 1.65 +/- 0.23 mg/kg and 28.97 +/- 5.4 mg/kg respectively] compared to normal full-term [0.88 +/- 0.17 mEq/kg P < 0.01, 1.01 +/- 0.19 mEq/kg P 0.05, 0.64 +/- 0.1 mg/kg P < 0.01 and 7.27 +/- 1.07 mg/kg P < 0.01 respectively]. Complicated premature newborns excrete significantly higher level of sodium, calcium and phosphate [2.99 +/- 1.30 mEq/kg, 3.02 +/- 1.62 mg/kg and 36.70 +/- 19.15 mg/kg respectively] than the group of normal full-term newborns P < 0.01 for each. Complicated full-term newborns excrete significantly more sodium [2.11 +/- 1.60 mEq/kg] and phosphate [36.65 +/- 7.88 mg/kg] than normal full-term neonates P < 0.05 and P < 0.01 respectively. Significant negative correlations were found between either sodium or phosphate excretion and gestational age in the group of uncomplicated premature newborns [r = 0.78 P < 0.01 and r = -0.80 P < 0.01 respectively]. The results were discussed in the light of the available data