Clinical and Demographic Evaluation According to MEFV Genes in Patients with Familial Mediterranean Fever.

The present study examined the relationship between clinical findings and mutation analyses in children with Familial Mediterranean Fever (FMF) in the inner Black Sea region of Turkey. This retrospective, cross-sectional study included patients with FMF who were evaluated between 2007 and 2015. FMF was diagnosed according to the Tel Hashomer criteria. FMF mutations were analyzed using a Real-time PCR System (Roche Diagnostics, Mannheim, Germany), and patients were classified into three groups according to allele status. The most common symptom was abdominal pain (99%, n = 197). The most frequent mutations were M694V and R202Q. Chest pain was reported more often in patients homozygous for M694V (61.4%). Although fever, abdominal pain, and arthritis were more commonly observed with the M694V mutation, chest pain was the most common symptom in R202Q carriers (n = 10, 32.3%). Proteinuria was observed in 42 (21.2%) patients, frequently accompanied by the M694V mutation (28.6%). The most common mutations in children with FMF in Turkey were M694V and R202Q. Recurrent abdominal pain and arthritis/arthralgia were commonly observed in patients with M694V and R202Q mutations. Moreover, chest pain was commonly seen with the R202Q mutation. Thus, R202Q might be a disease-causing mutation in FMF patients.

Negative correlation among vitamin B12 levels, obesity severity and metabolic syndrome in obese children: A case control study.

OBJECTIVE: To determine the relationship among vitamin B12 status, obesity severity, and metabolic syndrome and its components in obese children.. METHODS: This case-control study was conducted at the School of Medicine, Gaziosmanpasa University, Tokat, Turkey, from January 2012 and October 2014, and comprised cases of obese and healthy children. The obese children were divided into three groups according to body mass index-standard deviation score quartiles. Group 1 included the first quartile, group 2 included the second and third quartiles, and group 3 included the fourth quartile. Patients with a body mass index of >95th percentile, according to reference curves for Turkish children and adolescents, were considered obese.Patients with a body mass index between15th and 85th percentile were considered to have normal weight. The World Health Organisation's modified metabolic syndrome criteria for children were used to diagnose metabolic syndrome.SPSS 19 was used for data analysis. RESULTS: Of the 256 participants, 153(59.8%) were obese and 103(40.2%) were healthy controls. The mean age of the obese children was 12.69±2.29 years and that of healthy controls was 13.05±2.48 years. Mean vitamin B12 levels were significantly lower among obese children than healthy volunteers (p<0.001). Age and body mass index-standard deviation score were significantly associated with vitamin B12 status (r= -0.175, p=0.030; r= -0.210, p=0.09, respectively). CONCLUSIONS: Increase in body mass index-standard deviation score was associated with a decrease in vitamin B12 levels.

Is the IL-6 -174G/C Gene Polymorphism Related to the Disease Severity Score in Turkish Children with Familial Mediterranean Fever?

Familial Mediterranean fever (FMF) is an autosomal recessively inherited disease characterized by recurrent self-limited attacks of fever accompanied by aseptic inflammation of serosal spaces, joints and skin, peritonitis, pleuritis, and arthritis. Clinical features differ according to genetics variants. The aim of this study was to identify relationship between IL-6 -174G/C gene polymorphisms and clinical features, disease severity score (DSS) and proteinuria in children diagnosed with FMF. In this study, 99 children who were followed-up in Gaziosmanpasa University Medical Faculty Department of Pediatrics and diagnosed with Familial Mediterranean fever according to Tel-Hashomer criteria were included. One hundred and fifty seven children who admitted to the hospital with any complain and found healthy included in control group. Genotyping was done for polymorphism in a promoter region of IL-6 gene (G/C at -174). The IL-6 -174G/C gene polymorphism and the clinical features of FMF, proteinuria, the DSS, and the healthy control group were investigated. Data for the clinical features were obtained retrospectively from the electronic records of patients. All of the genotyping of blood samples were done in Medical Genetic laboratory of Gaziosmanpasa University School of Medicine. The results revealed that the distribution of the genotypes and allele frequencies of the IL-6 -174G/C polymorphism were not significantly different between the FMF patients and the healthy controls. The IL-6 -174G/C polymorphisms did not affect proteinuria, the DSS, and the clinical features of FMF patients.

[Hypovitaminosis D in Children with Hashimoto’s Thyroiditis]. / Hipovitaminosis D en niños con tiroiditis de Hashimoto.

BACKGROUND: Vitamin D deficiency or insufficiency may play a role in the pathogenesis of certain autoimmune diseases. AIM: To measure vitamin D levels in children with Hashimoto’s thyroiditis (HT) (either with subclinical or marked hypothyroidism) and in healthy controls. MATERIAL AND METHODS: We included 68 children with HT aged 12 ± 4 years (39 females) from a pediatric outpatient clinic and 68 healthy children aged 10 ± 4 years (37 females). Calcium metabolism parameters, thyroid function tests and anti-thyroid peroxidase (anti-TPO), anti-thyroglobulin (anti-TG) and 25 hydroxy vitamin D (25OHD) levels were measured. RESULTS: Patients were older than controls but well matched by gender distribution. Mean 25OHD levels were significantly lower in HT patients than controls (16.8 ± 9.3 and 24.1 ± 9.4 ng/mL respectively, P < 0.01). Frequency of vitamin D deficiency was 76 and 35% in HT patients and controls, respectively (P < 0.001). CONCLUSIONS: Vitamin D deficiency is more common in children with HT than healthy controls.

Simple markers for subclinical inflammation in patients with Familial Mediterranean Fever.

BACKGROUND: In this study we investigated the potential of neutrophil/lymphocyte ratio (NLR), platelet/lymphocyte ratio (PLR), mean platelet volume (MPV), and red cell width distribution (RDW) as new inflammatory markers to identify chronic inflammations during symptom-free periods in children diagnosed with Familial Mediterranean Fever (FMF). MATERIAL/METHODS: The study included 153 children diagnosed with FMF based on the Tel-Hashomer Criteria, and 90 healthy volunteers. Hospital records were obtained to collect NLR, PLR, MPV, RDW, and FMF scores and the FMF mutation analyses of the patients enrolled in the study. Data on proteinuria were also collected and defined as a protein/creatinine ratio>0.2. RESULTS: NLR, PLR, MPV, and RDW were significantly higher in symptom-free FMF patients than in the control group. C-reactive protein values also weakly correlated with NLR, PLR, MPV, and RDW, but the correlation was not statistically significant. NLR had the strongest correlation with CRP. The NLR cut-off point to indicate subclinical inflammation in symptom-free FMF patients was calculated to be 1.65. CONCLUSIONS: NLR, PLR, MPV, and RDW are potential subclinical inflammation markers in patients with FMF. NLR, PLR, MPV, and RDW values are higher in patients with FMF during symptom-free periods. NLR was found to be the most reliable marker for subclinical inflammation when compared to PLR, MPV, and RDW. We also found that these markers are not significantly higher in proteinuric patients when compared with levels in non-proteinuric patients.

Hypovitaminosis D in children with familial Mediterranean fever.

PURPOSE: Vitamin D deficiency or insufficiency plays a role in the initiation and perpetuation of certain autoimmune diseases. The purpose of this study was to measure the vitamin D status of children with Familial Mediterranean Fever (FMF) and compare it to their healthy peers. METHODS: A total of 50 FMF patients and 49 healthy children were enrolled in this prospective study. Vitamin D levels were measured via HPLC. Demographic data, FMF symptom severity scores and the levels of other disease activity markers were retrieved from our hospital database. RESULTS: The mean age and gender balance of patients and controls were similar, being 8.4 ± 3.8 years and 19 male patients, and 9.1 ± 3.0 years and 25 male controls, respectively. The mean 25(-OH) vitamin D3 levels were 15.94 ± 9.66 µg/L in FMF patients and 41.22 ± 21.31 µg/L in controls. Vitamin D levels were normal in 12% of FMF patients, insufficient in 62% and deficient in 26%. No vitamin D deficiency was evident in any control subject; 30% had insufficient and 70% had normal vitamin D levels. Plasma vitamin D3 levels were similar in all patients despite varying FMF symptom severity scores. CONCLUSIONS: Vitamin D deficiency is frequent in children with FMF but is not associated with disease severity score.

Ursodeoxycholic acid protects neonatal rats from necrotizing enterocolitis: a biochemical, histopathological, and immunohistochemical study.

BACKGROUND: The pathophysiology of necrotizing enterocolitis (NEC) includes the massive production of endogenous cytokines with exaggerated activation of inflammatory pathways. Ursodeoxycholic acid (UDCA) has been used as an anti-inflammatory, antioxidant, and anti-apoptotic agent. We investigated the possible protective effects of UDCA in a neonatal rat pup model of NEC. METHODS: We randomly divided rat pups into three groups: a control group, a non-treated NEC group, and a UDCA-treated NEC group. We induced NEC by feeding formula enterally and via hypoxia/reoxygenation. Intestinal samples were collected for histopathological and immunohistochemical evaluation. Blood samples were taken for biochemical analyses. RESULTS: UDCA significantly reduced the extents of terminal ileal and jejunal injuries compared to the NEC group (p < .01), reduced Bax and caspase-3 immunoreactivities (both p < .01), and lowered serum levels of platelet-activating factor and intestinal fatty acid-binding protein (p < .01, p = .023, respectively). CONCLUSIONS: In a rat model of NEC, UDCA protects against adverse intestinal histological, immunohistochemical, and biochemical changes. UDCA significantly reduces the effects of NEC on the rat pup intestine.

Neonatal Thrombocytopenia and the Role of the Platelet Mass Index in Platelet Transfusion in the Neonatal Intensive Care Unit

Background: Neonatal thrombocytopenia is a common hematological abnormality that occurs in 20­35% of all newborns in the neonatal intensive care unit. Platelet transfusion is the only known treatment; however, it is the critical point to identify neonates who are really at risk of bleeding and benefit from platelet transfusion as it also has various potential harmful effects. Aims: To investigate the prevalence and risk factors of neonatal thrombocytopenia and its relationship to intraventricular hemorrhage in the neonatal intensive care unit and to determine whether the use of platelet mass index-based criteria could reduce the rate of platelet transfusion. Study Design: Retrospective cohort study. Methods: This study was conducted in the neonatal intensive care unit of a tertiary university hospital. The medical records of neonates in the neonatal intensive care unit with platelet counts <150×109/L between January 2013 and July 2016 were analyzed. Results: During the study period, 2,667 patients were admitted to the neonatal intensive care unit, and 395 (14%) had thrombocytopenia during hospitalization. The rate of intraventricular hemorrhage was 7.3%. Multiple logistic regression analysis showed that although lower platelet counts were associated with a higher intraventricular hemorrhage rate, the effects of respiratory distress syndrome, sepsis, and patent ductus arteriosus were more prominent than the degree of thrombocytopenia. Thirty patients (7%) received platelet transfusion, and these patients showed a significantly higher mortality rate than their non-platelet transfusion counterparts (p<0.001). In addition, it was found that the use of platelet mass index-based criteria for platelet transfusion in our patients would reduce the rate of platelet transfusion by 9.5% (2/21). Conclusion: Neonatal thrombocytopenia is usually mild and often resolves without treatment. As platelet transfusion is associated with an increased mortality rate, its risks and benefits should be weighed carefully. The use of platelet mass index-based criteria may reduce platelet transfusion rates in the neonatal intensive care unit, but additional data from prospective studies are required.

Evaluation of executive functions in children and adolescents with familial Mediterranean fever.

The aim of the present study was to investigate neuropsychological test performance in children and adolescents with familial Mediterranean fever (FMF). A total of 88 children and adolescents aged 8 to 17 years were included, 52 with FMF and 36 healthy controls. After the participants were administered the Children Depression Inventory (CDI) and the Screen for Child Anxiety Related Emotional Disorders (SCARED), they completed the battery tests of the Central Nervous System Vital Signs (CNSVS), a neurocognitive test battery, via computer. The battery calculates seven domain scores (Memory, Psychomotor Speed, Processing Speed, Reaction Time, Complex Attention, Executive Function, and Cognitive Flexibility) and a summary score (Neurocognition Index [NCI]). A statistically significant difference between the FMF and control groups was found in six out of seven domains, where the scores of the participants with FMF were found to be significantly lower than those of the control participants (p < .05). Although the mean Reaction Time score of the participants with FMF was found to be lower than that of the control participants, the finding was not statistically significant (p > .05). The mean CDI and SCARED scores of the participants with FMF were found to be significantly higher than those of the control participants (p < .05). Low scores in the Processing Speed and Psychomotor Speed domains of the CNSVS were significantly correlated with higher SCARED scores (r = -.37, p = .01). Impaired cognitive functions should be taken into consideration in children and adolescents with FMF when assessing and managing this population.

Role of the Polymorphisms of Uncoupling Protein Genes in Childhood Obesity and Their Association with Obesity-Related Disturbances.

BACKGROUND: Obesity, one of the most common disorders observed in clinical practice, has been associated with energy metabolism-related protein genes such as uncoupling proteins (UCPs). Herein, we evaluated UCPs as candidate genes for obesity and its morbidities. METHODS: A total of 268 obese and 185 nonobese children and adolescents were enrolled in this study. To determine dyslipidemia, hypertension, and insulin resistance, laboratory tests were derived from fasting blood samples. UCP1-3826 A/G, UCP2 exon 8 deletion/insertion (del/ins), and UCP3-55C/T variants were also genotyped, and the relationships among the polymorphisms of these UCPs and obesity morbidities were investigated. RESULTS: The mean ages of the obese and control groups were 11.61 ± 2.83 and 10.74 ± 3.36 years, respectively. The respective genotypic frequencies of the AA, AG, and GG genotypes of UCP1 were 46.3%, 33.2%, and 20.5% in obese subjects and 46.5%, 42.2%, and 11.4% in the controls (p = 0.020). G alleles were more frequent in obese subjects with hypertriglyceridemia (42.9%; p = 0.048) than in those without, and the GG genotype presented an odds ratio for obesity of 2.02 (1.17-3.47; p = 0.010). The polymorphisms of UCP2 exon 8 del/ins and UCP3-55C/T did not influence obesity risk (p > 0.05). The I (ins) allele was associated with low HDL cholesterolemia (p = 0.023). CONCLUSION: The GG genotype of the UCP1-3826 A/G polymorphism appears to contribute to the onset of childhood obesity in Turkish children. The GG genotype of UCP1, together with the del/del genotype of the UCP2 polymorphism, may increase the risk of obesity with synergistic effects. The ins allele of the UCP2 exon 8 del/ins polymorphism may contribute to low HDL cholesterolemia.

Assessment of Neurocognitive Functions in Children and Adolescents with Obesity.

Obesity is linked to adverse neurocognitive outcomes including reduced cognitive functioning. We aimed to investigate the differences in neuropsychological test performance of Turkish children and adolescents with obesity and healthy peers. Study includes 147 children and adolescents ranging in age from 8 to 16 years: 92 with obesity and 55 with healthy controls. After the participants were administered the Children's Depression Inventory (CDI) and the Screen for Child Anxiety Related Disorders (SCARED), they completed the battery tests of the Central Nervous System Vital Signs (CNSVS), a neurocognitive test battery, via computer. The battery calculates seven domain scores (Memory, Psychomotor speed, Processing speed, Reaction time, Complex attention, Executive Function, Cognitive flexibility), and a summary score (Neurocognition Index [NCI]). There was a statistically significant difference between the obesity and control groups on all cognitive domains. The mean NCI score of the obesity group was 81.3 ± 10.24 compared to 97.29 ± 4.97 for the control group. The mean NCI score in the obesity group was significantly lower than that of the control group (p < 0.001). The mean scores of other domains of obese patients were also found to be significantly lower than those of the control participants (p < 0.001). The mean SCARED scores of the participants with obesity were found to be significantly higher than those of the control participants (p < 0.05). However, no statistically significant relationship was found between the SCARED and the CNSVS scores. Cognitive dysfunction in children and adolescents with obesity should be taken into consideration when assessing and managing this population.

Association between vitamin D levels and cardiovascular risk factors in obese children and adolescents.

BACKGROUND AND AIM: Childhood obesity is associated with an increased risk of chronic disease. We aimed to determine the association between vitamin D deficiency and cardiovascular risks in obese children. METHOD: The studied children were selected from obese children who were followed up at obesity clinic, aged 6-17 years. Basic demographic information and laboratory data were collected retrospectively from hospital records. RESULTS: A total of 310 students (178 [57.4%] girls) were evaluated for 25-hydroxyvitamin D (25[OH] D) levels in late winter/spring. The prevalence rates of vitamin D deficiency, insufficiency, and sufficiency were 62.3%, 34.5%, and 3.2%, respectively. Insulin resistance was observed in 146 (47.1%) children; the frequencies of dyslipidemia and hypertension were 31% and 19.4%, respectively. The mean atherogenic dyslipidemia ratio was higher in the deficient group (p = 0.049). Inverse correlations of 25(OH) D levels were observed with homeostasis model assessment of insulin resistance values (r = -0.146, p = 0.010). The mean values of 25(OH) D (ng/mL) were lower in girls (12.15 ± 6.60) than in boys (16.48 ± 8.69) (p < 0.05) and in children with hypertension (11.92 ± 5.48) than in those without (14.50 ± 8.24) (p < 0.05). CONCLUSIONS: Vitamin D deficiency is observed more frequently than expected in obese children and adolescents. Our findings indicate that low 25(OH) D levels are associated with insulin resistance. Vitamin D deficiency could contribute to the morbidities associated with childhood obesity, such as insulin resistance or diabetes mellitus, increased cardiovascular/cardiometabolic risks, atherogenic dyslipidemia, and hypertension.

Peripapillary Retinal Nerve Fiber Layer and Ganglion Cell-Inner Plexiform Layer Thickness in Children with Familial Mediterranean Fever.

PURPOSE: To evaluate the thickness of the peripapillary retinal nerve fiber layer (RNFL) and retinal ganglion cell-inner plexiform layer (GCIPL) in children with familial Mediterranean fever (FMF). METHODS: The study included 39 FMF patients and 36 healthy controls. After detailed ocular examination, the thickness of the peripapillary RNFL and GCIPL were measured by spectral domain optic coherence tomography (SD-OCT). All measurements were taken from the right eye of the patients and controls. According to their disease severity score (DSS), the patients were divided into two groups: patients with DSS ≤5 and those with DSS >5. RESULTS: There were no statistically significant differences in peripapillary RNFL and retinal GCIPL thickness between patients with FMF and controls. CONCLUSION: It appears that FMF does not affect the RNFL and GCIPL thickness.

Prevalence of hypercalciuria and urinary calcium excretion in school-aged children in the province of Tokat.

AIM: Hypercalciuria is an important cause of urinary tract symptoms, and also frequently results in urolithiasis. Urinary calcium excretion varies for geographic areas. We aimed to assess percentiles of urinary calcium excretion and prevalence of hypercalciuria for school-aged children in Tokat (city located in inner northern region of Turkey). MATERIAL AND METHODS: One thousand three hundred seventy-five children aged 6 to 18 years were enrolled in the study. Urine samples were obtained randomly. The children's variables as sex, age, length, and weight were recorded. Urinary calcium and creatinine determined from the urine samples and urinary calcium/creatinine ratios (mg/mg) were calculated. Percentiles of urinary calcium/creatinine ratios were also assessed for each age of the children. RESULTS: Six hundred eighty-three of the 1 375 children were girls and 692 were boys. The mean age of the children was 11.68±3.43 years. Some 23.9% of the children were living in rural regions and 76.1% were were living in urban regions. The mean urinary calcium/creatinine ratio was 0.080±0.24 and the 95th percentile value of the urinary calcium/creatinine ratio was 0.278. The hypercalciuria prevalence for school-aged children was 4.7% when the urinary calcium/creatinine ratio value for hypercalciuria was accepted as ≥0.21. The prevalence of hypercalciuria in rural and urban regions was 7.60% and 3.82%, respectively (p<0.05). Hypercalciuria was present in 7 of 141 patients who were obese (4.96%) and 58 of 1 234 patients who were not obese (4.70%) (p>0.05). CONCLUSION: The prevalence of hypercalciuria and urinary calcium excretion vary for different geographic areas, not only for countries. The percentiles of urinary calcium excretion should be assesed for every geographic region and the prevalance of hypercalciuria should be determined with these values. There is controversy as to whether obesity is a risk factor for hypercalciuria.

Merosin-negative congenital muscular dystrophy: magnetic resonance spectroscopy findings.

Congenital muscular dystrophies (CMD) are heterogeneous group of muscle disorders with autosomal recessive inheritance. Merosin deficiency has been identified in some patients with CMD all of whom also had white matter abnormalities on MRI. In postmortem studies, the brain showed extensive myelin pallor with a spongy appearance of white matter and moderate astrocytosis or demyelination. Direct assessment of neuropathologic aspects of MN-CMD such as demyelination is possible with MR spectroscopy (MRS). Although previous reports have described several neuro-imaging findings of this disease, MRS findings have not been reported in literature. In this case, we report MRS features of a 4-year old girl with MN-CMD. MRS of brain demonstrated that N-acetylaspartate (NAA)/Creatine (Cr) ratio was normal. Increased Choline (Cho)/Cr and Myo-inositol (MI)/Cr ratios were obtained. These findings were interpreted as demyelination and gliosis of white matter.

RELATIONSHIPS AMONG THYROID HORMONES AND OBESITY SEVERITY, METABOLIC SYNDROME AND ITS COMPONENTS IN TURKISH CHILDREN WITH OBESITY.

BACKGROUND: we investigated the relationships between thyroid function and obesity severity, metabolic syndrome (MS) and MS components in 260 obese children and adolescents 10-17 years of age. OBJECTIVES: we aimed to determine the association of thyroid functions with obesity severity and the components of metabolic syndrome (MS) in pediatric obese patients. METHODS: only obese children and adolescents were included, and divided the obese children into three groups according to body mass index (BMI)-SDS quartiles. The first quartile was group 1, the second and third quartiles were group 2, and the fourth quartile was group 3. Group 3 indicated severe obesity. The modified WHO criteria adapted for children were used to diagnose MS. We assessed anthropometric data and serum biochemical parameters, including the lipid profile and fasting glucose (FG), insulin, thyroid-stimulating hormone (TSH), free thyroxine (fT4), and free triiodothyronine (fT3) levels. Blood pressure (BP) was measured with a standard digital sphygmomanometer. The homeostasis model assessment of insulin resistance was calculated to determine insulin resistance (IR). RESULTS: TSH level was significantly higher in obese children with MS than that in the others (p = 0.045). Mean TSH level was not different among the BMI-SDS groups (p = 0.590). TSH levels and the fT3/fT4 ratio were not different in children with dyslipidemia, IR or hypertension (p = 0.515, 0.805, 0.973, 0.750, 0.515, and 0.805, respectively). DISCUSSION: obesity severity does not affect TSH level or the fT3/fT4 ratio in obese children and adolescents. IR is in close relationship with TSH level. Elevated TSH level is a risk factor for MS.

Antecedentes: hemos investigado las relaciones entre la función tiroidea y la severidad de la obesidad, el síndrome metabólico (MS) y los componentes del MS en 260 niños y adolescentes obesos de entre 10 y 17 años de edad. Objetivos: pretendemos determinar la asociación de las funciones tiroideas con la severidad de la obesidad y los componentes del síndrome metabólico (MS) en pacientes pediátricos obesos. Métodos: solo se incluyeron niños y adolescentes obesos, y se dividió a los niños obesos en tres grupos según los cuartiles de índice de masa corporal (BM). El primer cuartil fue el grupo 1, el segundo y tercer cuartil fueron el grupo 2, y el cuarto cuartil fue el grupo 3. El grupo 3 indicó obesidad severa. Los criterios WHO modificados adaptados para los niños se utilizaron para diagnosticar MS. Evaluamos los datos antropométricos y los parámetros del suero bioquímico, incluyendo el perfil lípido y los niveles de glucosa en ayunas (FG), insulina, hormona estimulante del tiroides (TSH), tiroxina libre (fT4) y triyodotironina libre (fT3). Se midió la presión sanguínea (BP) con un esfigmomanómetro digital estándar. La evaluación del modelo de homeostasis de la resistencia de la insulina se calculó para determinar la resistencia a la insulina (IR). Resultados: el nivel de TSH fue significativamente mayor en niños obesos con MS que en los demás (p = 0,045). El nivel medio de TSH no fue diferente entre los grupos BMI (p = 0,590). Los niveles de TSH y la proporción fT3/ fT4 no fueron diferentes en niños con dislipidemia, IR o hipertensión (p = 0,515; 0,805; 0,973; 0,750; 0,515 y 0,805, respectivamente). Discusión: la severidad de la obesidad no afecta al nivel de TSH ni a la proporción fT3/fT4 en niños y adolescentes obesos. La IR está en relación directa con el nivel de TSH. El nivel elevado de TSH es un factor de riesgo para el MS.

Effects of valproate and carbamazepine on serum levels of homocysteine, vitamin B12, and folic acid.

Homocysteine (HMC) is a sulfur containing amino acid, which plays a role in methionine metabolism. Folic acid (FA) and vitamin B12 (B12) are essential for remethylization of HMC to methionine. HMC level increases in the deficiency of these vitamins. Hyperhomocysteinemia causes vascular endothelial damage, which causes atherosclerosis. The aim of this study is to investigate the effect of valproate (VA) and carbamazepine (CBZ) on the serum levels of HMC, B12, and FA.Thirty-six children receiving CBZ and 30 children receiving VA for epilepsy for the last 1-year period and 29 healthy children as control were the population of this study. After 6 h of fasting serum HMC, B12, and FA levels were measured and results were compared statistically. Mean values of HMC, FA, and B12 levels in control group were 9.2+/-2.7 micromol/l, 9.0+/-2.0 ng/ml, and 342+/-162 pg/ml, in VA group 14.0+/-6.8 micromol/l, 7.3+/-2.9 ng/ml, and 368+/-159 pg/ml, in CBZ group 16.0+/-13.1 micromol/l, 7.5+/-3.3 ng/ml, and 285+/-158 pg/ml, respectively. Serum HMC levels were higher in VA and CBZ groups than control group (P<0.01 and P<0.05, respectively). Serum FA levels were lower in VA and CBZ groups compared to control group (P<0.05). Serum levels of B12 were not different between VA and control groups (P>0.05). In CBZ group serum B12 levels were lower than control group (P<0.05).FA may be added to the treatment protocol (if the patients take only CBZ, then B12 should also be added) for patients taking these antiepileptic drugs to decrease the degenerative effect of VA and CBZ on vascular endothelium.

Syndrome of inappropriate antidiuretic hormone associated with Crimean-Congo hemorrhagic fever.

Crimean-Congo hemorrhagic fever (CCHF) is a potentially fatal systemic disease in children caused by a tick- borne virus. Many different clinical and laboratory findings are seen in CCHF. We report here an atypical presentation of CCHF with hyponatremia. CCHF with electrolyte imbalance is not reported before. A 4-year-old girl presented with fever, fatigue and unconsciousness with hyponatremia. Based on the clinical and epidemiological findings, virus infection was suspected. Hyponatremia is has never been reported in Crimean-Congo hemorrhagic fever (CCHF), as was observed in this case. The diagnosis was confirmed by detection of IgM antibody to CCHF virus and positive Real-Time PCR. We report the first case of imported CCHF presenting as hyponatremia. This electrolyte imbalance has never been reported before in CCHF in children, and the clinician should consider this entity in complications to explain unconsciousness.

Higher hdl levels are a preventive factor for metabolic syndrome in obese Turkish children.

AIM: The definition of childhood metabolic syndrome has not been described clearly. Childhood obesity is increasing gradually, and the incidence of childhood metabolic syndrome is also rising. We aimed to show metabolic syndrome components and preventive factors for metabolic syndrome in obese children Methods: In the present study, 187 obese children and adolescents 5-18 years old were investigated retrospectively. Demographic data, anthropometric measurements, body mass index, blood pressure values, insulin levels, oral glucose tolerance test results, total cholesterol, high density lipoprotein, and triglyceride levels were obtained from hospital records. A body mass index > 95th percentile was considered obese. Insulin resistance was calculated according to the oral glucose tolerance test with 1.75 g/kg glucose maximum 75 g glucose. The insulin sensitivity index and homeostatic model assessment-insulin resistance (HOMA IR) were calculated and compared. Metabolic syndrome was diagnosed according to the modified WHO criteria adapted for metabolic syndrome in children. RESULTS: Abnormal glucose homeostasis was detected in 53% of subjects. Dyslipidaemia was present in 45.7% and hypertension in 16.6% of the patients. Metabolic syndrome was identified in 24.6% of obese children and adolescents. High HOMA-IR values and fasting glucose levels, elevated triglycerides and lower HDL levels were an indication of metabolic syndrome. CONCLUSION: Obesity and insulin resistance are significant factors for the development of metabolic syndrome in children and adolescents. In obese children higher HDL levels are preventive factor for metabolic syndrome. Preventing obesity and insulin resistance may decrease the prevalence of metabolic syndrome.

Objetivo: El síndrome metabólico infantil no ha sido definido aún con claridad. La obesidad infantil se está incrementando progresivamente al igual que la incidencia del síndrome metabólico infantil. Nuestro objetivo ha sido mostrar los componentes del síndrome metabólico y sus factores preventivos en los niños obesos. Metodología: Este estudio analizó de forma retrospectiva a 187 niños y adolescentes obesos de entre 5 y 18 años. Los datos demográficos, las medidas antropomórficas, los índices de masa corporal, los valores de presión sanguínea, los niveles de insulina, los resultados de test de tolerancia a la glucosa oral, el total de colesterol, las lipoproteínas de gran densidad y los niveles de triglicéridos fueron obtenidos de registros hospitalarios. Una masa corporal con un índice superior a 95 percentiles fue considerada como obesidad. La resistencia a la insulina se calculó de acuerdo con el test de tolerancia a la glucosa oral con 1,75 g/kg de glucosa y un máximo de 75 gramos de glucosa. Se calculó y comparó el índice de sensibilidad a la insulina y la evaluación del modelos homeostático- resistencia a la insulina (HOMA IR). El síndrome metabólico fue diagnosticado de acuerdo con los nuevos criterios de la OMS adaptados a los síndromes metabólicos infantiles. Resultados: Se observó una homeostasis de glucosa anormal en el 53% de los casos. La dislipidemia estaba presente en el 45,7% de los pacientes y la hipertensión en un 16,6%. El síndrome metabólico fue identificado en un 24,6% de los niños y adolescentes obesos. Altos valores de HOMA-IR y de glucosa, triglicéridos elevados y niveles bajos de HDL eran indicadores de síndrome metabólico. Conclusión: La obesidad y la resistencia a la insulina son factores significativos para el desarrollo del síndrome metabólico en niños y adolescentes. En niños obesos altos niveles de HDL son un factor preventivo del síndrome metabólico. Prevenir la obesidad y la resistencia a la insulina puede reducir el predominio del síndrome metabólico.

Association between vitamin D levels and cardiovascular risk factors in obese children and adolescents / Asociación entre los niveles de vitamina D y factores de riesgo cardiovascular en niños y adolescentes obesos

Background and aim: Childhood obesity is associated with an increased risk of chronic disease. We aimed to determine the association between vitamin D deficiency and cardiovascular risks in obese children. Method: The studied children were selected from obese children who were followed up at obesity clinic, aged 6-17 years. Basic demographic information and laboratory data were collected retrospectively from hospital records. Results: A total of 310 students (178 [57.4%] girls) were evaluated for 25-hydroxyvitamin D (25[OH] D) levels in late winter/spring. The prevalence rates of vitamin D deficiency, insufficiency, and sufficiency were 62.3%, 34.5%, and 3.2%, respectively. Insulin resistance was observed in 146 (47.1%) children; the frequencies of dyslipidemia and hypertension were 31% and 19.4%, respectively. The mean atherogenic dyslipidemia ratio was higher in the defi cient group (p = 0.049). Inverse correlations of 25(OH) D levels were observed with homeostasis model assessment of insulin resistance values (r = -0.146, p = 0.010). The mean values of 25(OH) D (ng/mL) were lower in girls (12.15 ± 6.60) than in boys (16.48 ± 8.69) (p < 0.05) and in children with hypertension (11.92 ± 5.48) than in those without (14.50 ± 8.24) (p < 0.05). Conclusions: Vitamin D deficiency is observed more frequently than expected in obese children and adolescents. Our findings indicate that low 25(OH) D levels are associated with insulin resistance. Vitamin D deficiency could contribute to the morbidities associated with childhood obesity, such as insulin resistance or diabetes mellitus, increased cardiovascular/cardiometabolic risks, atherogenic dyslipidemia, and hypertension (AU)

Introducción y objetivo: la obesidad infantil se asocia a un riesgo aumentado de enfermedades crónicas. El objetivo de este estudio es determinar la relación entre la deficiencia en vitamina D y el riesgo cardiovascular en niños obesos. Método: se seleccionaron niños tratados en la clínica de obesidad, con edades entre 6 y 17 años. Los datos de laboratorio y la información demográfica básica se recogieron de forma retrospectiva a partir de las historias clínicas. Resultados: se evaluaron 310 estudiantes (178, 57,4% mujeres) midiendo los niveles de vitamina D a finales de invierno y en primavera. La prevalencia de deficiencia en vitamina D, insuficiencia y suficiencia fueron 62,3%, 34,5% y 3,2% respectivamente. Se encontró resistencia insulínica en 146 niños (47,1%); mientras que la frecuencia de dislipemia e hipertensión fue de 31% y 19,4%, respectivamente. La razón de aterogenicidad debida a dislipemia fue mayor en el grupo deficiente (p = 0,049). Se encontró una correlación inversa entre los niveles de 25-OH-D y los valores de HOMA (r = -0,146; p = 0,01). Los valores medios de vitamina D (ng/Ml) fueron inferiores en niñas (12,15 ± 6,60) que en niños (16,48 ± 8,69) (p < 0,05) y en niños con hipertensión (11,92 ± 5,48 vs. 14,50 ± 8,24 en normotensos) (p < 0,05). Conclusiones: se encontró una prevalencia de deficiencia en vitamina D en niños y adolescentes obesos superior a lo esperado. Nuestros hallazgos indican que los niveles bajos de vitamina D se asocian con resistencia insulínica. La deficiencia en vitamina D podría contribuir a las morbilidades que se asocian a la obesidad infantil, como la resistencia insulínica o la diabetes mellitus, el aumento del riesgo cardiovascular, la dislipemia y la hipertensión (AU)