Nagla El-Melegy

Renal functions in pediatric patients with beta-thalassemia major: relation to chelation therapy: original prospective study.

BackgroundIn β-thalassemia, profound anemia and severe hemosiderosis cause functional and physiological abnormalities in various organ systems. In recent years, there have been few published studies mainly in adult demonstrating renal involvement in β-thalassemia. This prospective study was aimed to investigate renal involvement in pediatric patients with transfusion dependant beta-thalassemia major (TD-βTM), using both conventional and early markers of glomerular and tubular dysfunctions, and to correlate findings to oxidative stress and iron chelation therapy.MethodsSixty-nine TD-βTM patients (aged 1-16 years) and 15 healthy controls (aged 3-14 years) were enrolled in this study. Based on receiving chelation therapy (deferoxamine, DFO), patients were divided into two groups: group [I] with chelation (n = 34) and group [II] without chelation (n = 35). Levels of creatinine (Cr), calcium (Ca), inorganic phosphorus (PO4), uric acid (UA) and albumin were measured by spectrophotometer. Serum (S) levels of cystatin-C (SCysC) and total antioxidant capacity (STAC) and urinary (U) levels of β2-microglobulin (Uβ2MG) were measured by immunosorbent assay (ELISA). Urinary N-acetyl-beta-D-glucosaminidase (UNAG) activity and malondialdehyde (UMDA) were measured by chemical methods. Estimated glomerular filtration rate (eGFR) was determined from serum creatinine.ResultsIn patient with and without chelation, glomerular [elevated SCysC, SCr, Ualbumin/Cr and diminished eGFR]; and tubular dysfunctions [elevated SUA, SPO4, UNAG/Cr, Uβ2MG/Cr] and oxidative stress marker disturbances [diminished STAC and elevated UMDA/Cr] were reported than controls. In patients with chelation, SCysC was significantly higher while, STAC was significantly lower than those without chelation. In all patients, SCysC showed significant positive correlation with SCr and negative correlation with eGFR; STAC showed significant positive correlation with eGFR and negative correlation with SCysC, SCr, UNAG/Cr; UMDA/Cr showed significant positive correlation with Ualbumin/Cr, Uβ2MG/Cr, UNAG/Cr.ConclusionsOur data confirm high frequency of glomerular and tubular dysfunctions in TD-βTM pediatric patients which could be attributed to oxidative stress and DFO therapy.

Oxidative Modification of Low-Density Lipoprotein in Relation to Dyslipidemia and Oxidant Status in Children With Steroid Sensitive Nephrotic Syndrome

It has been proposed that nephrotic syndrome is a consequence of an imbalance between oxidant/antioxidant statuses. The present study aimed to assess oxidant and antioxidant status in relation to dyslipidemia in children during remission and relapse phases of steroid sensitive nephrotic syndrome (SSNS). The study dealt with 40 children diagnosed as SSNS. They were categorized into two subgroups. The first subgroup included 25 children during remission stage. The second subgroup included 15 children during relapse. Control group consisted of age and gender-matched 15 healthy children. Significantly higher serum levels of malondialdehyde, oxidized LDL, total cholesterol, LDL cholesterol, triglycerides, apolipoprotein A-I, and apolipoprotein-B were observed in patients with SSNS especially in the relapsers. The serum levels of albumin, glutathione peroxidase activity, vitamin C, A, and E, and HDL cholesterol were significantly lower in patients especially among relapsers. In conclusion, a strong relationship between the oxidant/antioxidant status and dyslipidemia is documented in patients with SSNS, especially among relapsers. No normalization of the biochemical indices was observed despite the use of glucocorticoids. Therefore, the combined use of steroid, antioxidant therapy, and lipid lowering therapy can be recommended in such children.

Angiogenic biomarkers in children with congenital heart disease: possible implications.

BackgroundVascular endothelial growth factor (VEGF), platelet derived endothelial cell growth factor/thymidine phosphorylase (PD-ECGF/TP) and leptin are known as potent angiogenic factors The objective of the study was to evaluate these angiogenic factors VEGF, PD-ECGF/TP and leptin in children with congenital heart disease (CHD) and the factors that lead to angiogenesis in such cases.MethodsSixty CHD children were studied and divided into two groups (n = 30); cyanotic-CHD (C-CHD) and acyanotic-CHD (A-CHD). Twenty five healthy children were included as controls.ResultsSignificantly higher serum levels of VEGF, PD-ECGF/TP activity and leptin were detected in patients with CHD, particularly in patients with C-CHD. CHD patients with SpO2 <90%, pulmonary hypertension (PH), severe pulmonary stenosis (PS), detectable collaterals, cardiomegaly and/or heart failure showed significantly higher levels of these factors than those with higher SpO2 or those without these findings.ConclusionHypoxia, PH and PS are important factors that lead to harmful angiogenesis. However, angiogenesis could be essential in some cases of CHD as coarctation of aorta to enhance renal perfusion. This may provide new ways for therapeutic strategies aiming at reducing or promoting angiogenesis in CHD to improve patients outcome.

Carnitine and Liver Functions Assessment in ChildrenTreated with Anticonvulsant Drugs@@@تقييم حالة الكارنيتين ووظائف الكبد في الأطفال الذين يعالجون بأدوية الصرع

This study was conducted to evaluate the impact of antiepileptic drugs on the liver function and L-carnitine levels in children suffering from ep- ilepsy. Sixty epileptic children aged from 1 to 15 years, together with 20 healthy controls were studied. Among them twenty-five children were treated with phenytoin (group I), 25 were treated with carbamazepine (group II) and 10 patients were treated with phenobarbital (group III). In each of the three groups, serum free carnitine levels were significantly decreased after one month of therapy compared with pretherapy levels (p<0.01, p<0.01, p<0.01 re- spectively). Meanwhile, the levels of L-carnitine in treated children collective- ly were significantly decreased in comparison with healthy children (p<0.01). Hypocarnitinemia was detected in 28% of group I, 56% of group II , and 30% of group III. Serum SGOT was significantly increased in patients treated with phenytoin and phenobarbital (p<0.01, p<0.05 respectively) compared with pre- therapy levels. Whereas serum lactate and total bilirubin were significantly in- creased after therapy in patients treated with carbamazepine in comparison to that before significantly increased (p<0.05 for each) in comparison with con- trols. Moreover, a significant negative correlation was found between values of carnitine and both lactate (p<0.01) and lactate/pyruvate ratio (p<0.05) after therapy in carbamazepine treated group. It should be taken into account that carnitine rich food in the form of milk and milk products must be taken as a supplement to the antiepileptic drugs especially carbamazepine.