Ergün Sönmezgöz

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.

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

ABSTRACT 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.

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.

Assessment of Neurocognitive Functions in Children and Adolescents with Obesity

ABSTRACT 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 Childrens 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.

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.

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.

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

ABSTRACT 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.

Response to Letter from Dr. Salih Uzun et al. Regarding “Peripapillary Retinal Nerve Fiber Layer and Ganglion Cell-Inner Plexiform Layer Thickness in Children with Familial Mediterranean Fever”

First of all, we thank Uzun et al. for their interest in our study. In our exclusion criteria, which we did mention in the article, were patients with cycloplegic refractive errors of more than ±1.00 diopters (D). Cegarra et al. found that there is an anatomic relationship between ocular axial length and refractive errors. Axial length changes according to refractive errors and cycloplegic refractive errors of less than ±1.00 D may not significantly affect axial length. Twenty of our patients had a M694V gene mutation and 19 of them had other gene mutations. We divided the patients into two groups: patients with M694V gene mutation and the patients with other gene mutations— and we performed the statistics again. There were no statistically significant differences in all measured parameters between the patients with M694V gene mutation and the patients with other mutations (p>0.01). All of our patients were in remission with colchicine. It would be more appropriate to compare patients who used and did not use colchicine, but we did not have such patients. Therefore we performed statistics among the patients who had already used colchicine. We divided the patients into two groups: those who used colchicine for 0–24 months and those who used it for 24–96 months—we performed the statistics again. There were no statistically significant differences between the two groups (p>0.01). Finally, it seems that oral colchicine does not affect the retinal nerve fiber layer (RNFL) and ganglion cell-inner plexiform layer (GCIPL) thickness in human children.

Internet use patterns and Internet addiction in children and adolescents with obesity

There are no data regarding the Internet addiction (IA) rates and patterns in youth with obesity.

A Rare Seen Hemorrhage Disorder: Factor XI Deficiency Hemophilia C

75 Meandros Med Dent J 2018;19:75-8 Öz Faktör XI eksikliği kadınları ve erkekleri etkileyen otozomal resesif geçişli aynı zamanda otozomal dominant geçiş de gösterebilen bir kanama bozukluğudur. Yahudi olmayan genel popülasyonda Faktör XI eksikliği yaygın değildir ve yaklaşık görülme sıklığı 1/1 milyondur. Klinik fenotipi heterojendir ve kanama riski bireyin hemostatik değişikliklere verdiği cevaba göre değişmektedir. Faktör XI’in plazma düzeyi ile kanama eğilimi arasında zayıf bir ilişki vardır. Faktör XI eksikliğinde hasta hemofili A ve B’ye benzemeyen şekilde yaygın olmamakla birlikte spontan kanama ve cerrahi ya da travmayı takip eden kanamalar için risk altındadır. Hemartroz ve kas içi kanama hemofili A ve B’de yaygındır ancak Faktör XI eksikliği olanlarda tipik olarak görülmez. Yaygın görülen semptomlar kolay morarma ve burun kanamasıdır. Sıklıkla öyküde kanamanın zor durduğuna yönelik bulgular görülmektedir. Bu olguda mandibula üzerinde kitle nedeni ile operasyon öncesi incelemelerinde aktive parsiyel tromboplastin zamanı uzunluğu tespit edilerek nadir görülen bir kanama bozukluğu nedeni olan Faktör XI eksikliği hemofili C tanısı alan olgunun kanama diyatezi açısından yönetimi sunulmuştur. Factor XI deficiency is a hemorrhage disorder which can be either autosomal recessive or/and autosomal codominant and effects both women and men. Factor XI deficiency is uncommon in non-Jewish general population and average frequency of disorder is 1/1 million. Clinical phenotype is nonhomogeneous and bleeding risk depends on individual’s reaction against hemostatic variances. There is a poor relation between the Factor XI plasma level and bleeding tendency. In case of Factor XI deficiency, uncommon, unlike hemophilia A and B, spontaneous hemorrhage, surgical or trauma sequent are at risk. Hemarthrosis and intramuscular bleeding are common in hemophilia A and B but not typically seen in Factor XI deficiency disorders. Common symptoms are easy bruising and nosebleed. History of the disorder mostly indicates the findings about the difficulty in staunching. In this case, after detecting the activated partial thromboplastin time length before the operation due to build upon mandibula, management of patients, having Factor XI deficiency hemophilia C which is a cause of rare seen hemorrhage disorder, are presented in terms of bleeding