Evaluation of oxidative stress in children with congenital heart defects.

BACKGROUND: A significant cause of death and chronic illness in childhood is caused by cardiovascular diseases, including congenital heart disease (CHD). This study aims to investigate the oxidative stress status and to establish its association with CHD in children. METHODS: The study involves measurements of malondialdehyde (MDA), protein carbonyl (PCO), total anti-oxidant capacity, high-sensitive C-reactive protein (hs-CRP), fibrinogen and cytokine (interleukin [IL-6] and tumor necrosis factor-α) levels in 43 children with CHD and 30 healthy age-matched children. RESULTS: MDA, PCO, hs-CRP, fibrinogen, IL-6 and tumor necrosis factor-α were significantly elevated while total anti-oxidant capacity was significantly declined in patients compared with the controls. MDA was positively correlated with PCO, hs-CRP, Qp/Qs and systolic pulmonary artery pressure. PCO was positively correlated with hs-CRP, fibrinogen, IL-6 and systolic pulmonary artery pressure. CONCLUSION: Oxidative stress and its association with other markers in children with CHD was established. To the best of our knowledge, this is the first time that PCO has been used as a biomarker in CHD and it may be employed as a new diagnostic biomarker in CHD and in the assessment of its severity.

Intracranial hemorrhage: clinical and demographic features of patients with late hemorrhagic disease.

BACKGROUND: This retrospective study presents clinical, demographical features and radiological findings as well as outcomes of 31 infants with intracranial hemorrhage (ICH) due to vitamin K deficiency and hence evaluates the risk factors involved. METHODS: Thirty-one cases (17 males and 14 females) having a mean age of 52.52 ± 20.80 days with intracranial hemorrhage due to late hemorrhagic disease of the newborn (LHDN), hospitalized in our clinics were included in the study. Cranial computerized tomography (CT) was performed in all patients for the diagnosis and evaluation of ICH. RESULTS: It was found that the most frequent presenting symptoms were pallor (77.4%), seizures (58%), altered consciousness (58%), vomiting (44%) and poor feeding (35%). Pulsatile fontanel was found in 61% and bulging in 26%. Seven (22.5%) patients had prior history of antibiotic usage. All patients (93.5%) except two were breast fed. Sixteen (51.6%) were delivered at home. Eighteen (58%) had a history of single-dose vitamin K prophylaxis on the first day of delivery. Parenchymal (44%), subdural (39%) or subarachnoidal (22.5%) bleeding was observed. Seven (22.6%) were exitus. During the follow-up period (ranging from 3 months to 18 months) neurological examination findings were recorded. CONCLUSION: Our results indicate that it may be questionable whether single-dose vitamin K prophylaxis at birth is adequate for the prevention of LHDN and if a different timing of this prophylaxis should be made for the exclusively breast fed infants.

Levels of proinflammatory cytokines and hs-CRP in patients with homozygous familial hypercholesterolaemia.

OBJECTIVE: Homozygous familial hypercholesterolemia (FH) is an extremely rare (1/1,000,000) condition characterized by markedly increased LDL cholesterol levels and a significantly increased risk of premature coronary heart disease (CHD). We aimed to evaluate the levels of high-sensitivity C-reactive protein (hs-CRP) and proinflammatory cytokines, which are known to be associated with atherogenesis, in patients with this condition. METHOD AND RESULTS: A total of 10 patients with homozygous FH (5 women and 5 men, mean age 17.0 +/- 6.9 years, body mass index (BMI) (18.8 +/- 1.9 kg/m2) and 16 healthy controls were included. hs-CRP levels, proinflammatory cytokine levels and lipid parameters were measured and compared between patients and control subjects. Homozygous FH patients had significantly higher total cholesterol, LDL-cholesterol and Lp(a) levels and significantly lower triglyceride and HDL cholesterol levels, compared to controls (P = 0.0001, for all). Serum hs-CRP (3.7 +/- 1.3 mg/L vs. 0.6 +/- 0.6 mg/L) and IL-1beta, IL-2R, IL-6, IL-8, IL-10, TNF-alpha levels were all significantly higher in the homozygous FH group, compared to controls (P = 0.0001, for all). CONCLUSIONS: Homozygous FH patients have significantly higher levels of hs-CRP and circulating proinflammatory cytokines, which may explain their increased risk of atherosclerotic disease. hs-CRP is an important biomarker that may be helpful in the identification of asymptomatic CHD in FH patients.

Cranial magnetic resonance imaging findings of nutritional vitamin B12 deficiency in 15 hypotonic infants.

BACKGROUND: Nutritional vitamin B(12) deficieny is common among infants in the developing and underdeveloped countries. There is limited information concerning neuroimaging findings in infants with vitamin B(12) deficiency in the literature. AIMS: The aim of this study is to evaluate the cranial magnetic resonance imaging (MRI) changes and clinical characteristics of hypotonic infants due to vitamin B(12) deficiency. MATERIALS AND METHODS: A total of 15 infants with neuroradiologic investigations were diagnosed with nutritional B(12) vitamin deficiency. Cranial MRI was performed on all infants. RESULTS: Five infants were female (33%) and the mean age of infants was 12.3 ± 5.5 months. Hypotonia and neurodevelopmental retardation were present in all patients. MRI demonstrated thinning of the corpus callosum in 6 (40%), cortical atrophy in 5 (33.3%), large sylvian fissures in 5 (33.3%), ventricular dilatation in 3 (20%), asymetric large lateral ventricle in 2 (13.3%) and delayed in myelination in 2 (13.3%) patients. Four infants had normal MRI findings. CONCLUSION: Because of the importance of vitamin B(12) in the development of the brain, MRI findings may be detected and useful in infants with vitamin B(12) deficiency.

Bone mineral density in children with beta-thalassemia major in Diyarbakir.

Bone mineral status has extensively been investigated in adult thalassemics but less in thalassemic children. This study involves measurements of the bone mineral density (BMD), various demographic and biochemical parameters in 47 thalassemic children and 50 healthy controls with comparable age, sex, socioeconomic and regional distribution. Patients have significantly higher aspartate aminotransferase, alanine aminotransferase, phosphorous, osteocalcin, serum carboxy terminal teleopeptide fragment of type I collagen, intact parathyroid hormone (iPTH) and ferritin levels while they have significantly lower 25-hydroxy vitamin D (25OH-D), alkaline phosphatase and z-scores both at lumbar and femur compared to controls. Patients with high iPTH (30%) had significantly lower z-scores and 25OH-D while larger osteocalcin. We conclude that a significantly lower BMD in beta-thalassemic children compared with their healthy counterparts is a complex process and may partially attributed to their slower physical development, caused by iron overload and chelation therapy which may influence the liver as well as the endocrine tissues.

Assessment of thyroid function in children aged 1-13 years with Beta-thalassemia major.

OBJECTIVE: Hypothyroidism usually appears in the second decade of life and is thought to be associated with iron overload in patients with thalassemia major. This study aimed to evaluate thyroid dysfunctions in patients with beta-thalassemia major and to see if they appear in the earlier period of life. METHODS: Thyroid function and iron load status were evaluated in 90 children with a mean age of 7.17±3.78 years with beta-thalassemia major by measuring serum free thyroxin (FT4), serum free triiodothyronine (FT3), total thyroxin (T3), serum total triiodothyronine (T4), thyroid-stimulating hormone (TSH) and ferritin levels from serum of patients admitted to the Pediatric Department, Faculty of Medicine University of Dicle between March 2005 and July 2009. A control group formed from an age-sex matched healthy children with a mean age of 6.98±3.66 years was also included. A standard thyrotropin releasing hormone test was applied to 3 patients who had high TSH levels and were classified as subclinical primer hypothyroidism. The study was designed according to the Declaration of Helsinki and informed consent was obtained from the parents of all participants. FINDINGS: All thyroid parameters in patients were in the normal ranges compared with the controls except three of them which had high TSH levels. Serum ferritin level (2703±1649 ng/mL) in patients was significantly higher than in controls (81.5±15.5 ng/mL). CONCLUSION: The work implies that hypothyroidism could be even seen in the first decade of life in patients with beta-thalassemia major in spite of improved hematological cares.

Malondialdehyde (MDA) and protein carbonyl (PCO) levels as biomarkers of oxidative stress in subjects with familial hypercholesterolemia.

OBJECTIVE: Familial hypercholesterolemia (FH) is clinically characterized by elevated total and low-density lipoprotein (LDL) cholesterol levels in plasma, which has high risk for developing atherosclerosis. Increased oxidative stress (OS) and FH have been related to atherosclerosis. The study aims to evaluate oxidative stress in patients with hypercholesterolemia by measuring lipid peroxidation and protein carbonyl (PCO) levels in these patients. PCO in these patients may provide a new diagnostic biomarker for oxidative damage in atherosclerosis. DESIGN AND METHOD: Total cholesterol (Tc), low-density lipoprotein-cholesterol (LDL-c), triglyceride (TG), high-density lipoprotein-cholesterol (HDL-c), lipoprotein(a) (Lp-a) levels and carotid intima-media thickness were measured to evaluate characteristics of patients (11 homozygous and 25 heterozygous) with FH. 25 age-gender-BMI matched healthy control subjects were included in the study for comparison. RESULTS: MDA and PCO levels were significantly higher in homozygous patients compared with those of heterozygous and controls and it was found that they are positively correlated with LDL-c, Tc, Lp-a and IMT while negatively correlated with HDL-c. The heterozygous group also had significantly higher MDA and PCO levels compared with controls. CONCLUSION: The data obtained could be important for understanding the alterations presented by FH and could be related to their increased risk of developing atherosclerosis. To our knowledge, measurements of PCO in patients with FH are not recorded before and this may be used as a biomarker for protein oxidation, which may play a role in the increased cardiovascular risk of patients with FH.