Association of Macrophage Migration Inhibitory Factor Gene -173 G/C Polymorphism (rs755622) with Familial Mediterranean Fever in Children.

Objectives The aim of this study was to identify the genotypic analysis and allele frequencies of the -173 G/C polymorphism in the macrophage migration inhibitory factor ( MIF ) gene in children diagnosed with familial Mediterranean fever (FMF). Methods The study included 98 children who were diagnosed with FMF according to the Tel Hashomer criteria and one hundred and 57 healthy children as the control group. Genotyping was done for a polymorphism in a promoter region of the MIF gene (G/C at position -173). Results The relationship of FMF prevalence and -173 G/C genotype of the MIF gene was statistically significant. Individuals with the CC genotype seem to be predisposed to FMF. Conclusion The C/C polymorphism at position -173 of the MIF gene could be associated with excessive inflammation and immune response and can lead to susceptibility to FMF.

[Evaluation of Efficacy of Ribavirin on Laboratory Test and Severity Score in Crimean-Congo Hemorrhagic Fever in Children]. / Çocuklarda Kirim-Kongo Kanamali Atesinde Ribavirinin Laboratuvar Testi ve Siddet Skoru Üzerindeki Etkinliginin Degerlendirilmesi.

Crimean-Congo hemorrhagic fever (CCHF) is endemic in Tokat province. It is observed in all age groups. The case fatality rate varies 20-30% in hospitalized patients and 0-5% among children. Ribavirin use and supportive measures are the main treatment modalities, but the effects of ribavirin in CCHF are controversial. Most of the studies investigating the effectiveness of ribavirin are done by comparing the initial and the final laboratory values. The purpose of this study was to evaluate the effects of oral ribavirin use in children with CCHF by using all the clinical and laboratory findings and to investigate the presence of unnoticed results. In the study, the data of 67 patients under 19 years old who were hospitalized and followed up with a diagnosis of CCHF during 2012-2020 epidemic period were retrospectively analyzed. Epidemiological and demographic characteristics, clinical and laboratory data were retrieved from the patient files. We implemented a linear mixed-effects model to assess the effects of the ribavirin, taking into account the repeated measures of the data. Ribavirin was given to 33 (49.2%) of the patients. 54 (80.6%) of the patients were male, 13 (19.4%) were female, 52 (77.6%) of the patients were living in rural areas and 15 (22.4%) were living in urban areas. The mean age was 15.1 (median= 15.5, range= 8-18) years in patients who did not receive ribavirin, and 15.2 (median= 15, range= 11-18) years in ribavirin administered patients. At the time of admission 63 (94%) of the patients had fever, 60 (89.6%) had fatigue, 38 (56.7%) had rash, 48 (71.6%) had myalgia, 30 (44.8%) had headache, 25 (37.3%) had abdominal pain, 30 (44.8%) had vomiting, 21 (31.8%) had diarrhea, 29 (43.3%) had drowsiness, 29 (43.3%) had bleeding in the mucosa and 27 (40.3%) had petechiae. Drowsiness and mucosal bleeding rates were significantly higher in patients who received ribavirin (p<0.05). The time from tick bite to hospital admission was 4.85 (median= 4.5, range= 2-9) days in patients who did not receive ribavirin, and 4.64 (median= 5, range= 2-9) days in ribavirin administered patients. The mean length of the hospital stay in patients who did not receive ribavirin was 6.68 (median= 7, range= 4-10) days, while it was 7.45 (median= 8, range= 3-13) days in those treated with ribavirin. Linear-mixed effect test results showed that ribavirin decreased hemoglobin (Hb) (95% GA= -0.12/-0.02), suppressed white blood cell count (WBC) (95% GA= -0.15/-0.02) and absolute lymphocyte count (ALC) (95% GA= -0.12/0.05), and decreased alanine aminotransferase (ALT) (95% GA= -9.32/-1.49), lactate dehydrogenase (LDH) (95% GA= -25.06/-2.36) and severity score index (95% GA= -0.28/-0.004). As a result, ribavirin suppressed Hb, WBC and ALC levels in children with CCHF, but decreased liver enzymes and severity score much faster in the elapsed time.

Hemophagocytosis in the Acute Phase of Fatal Kawasaki Disease in a 4 Month-Old Girl.

BACKGROUND: Kawasaki disease is a systemic vasculitis predominately affecting coronary arteries. Hemophagocytic lymphohistiocytosis can complicate the course of Kawasaki disease. Rare cases of secondary hemophagocytic lymphohistiocytosis occurring during the acute phase of Kawasaki disease have been reported. CASE REPORT: We report here a 4 month-old girl with diffuse coronary ectasia and secondary hemophagocytic lymphohistiocytosis occurring during the acute phase of incomplete Kawasaki disease. CONCLUSION: Due to the large overlap in clinical symptoms, the presence of atypical findings for Kawasaki disease should suggest the possible diagnosis of hemophagocytic lymphohistiocytosis in these patients.

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.

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.

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.

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.

How does parents' visual perception of their child's weight status affect their feeding style?

INTRODUCTION: Eating style is one of the prominente factors that determine energy intake. One of the influencing factors that determine parental feeding style is parental perception of the weight status of the child. AIM: The aim of this study is to evaluate the relationship between maternal visual perception of their children's weight status and their feeding style. METHOD: A cross-sectional survey was completed with only mother's of 380 preschool children with age of 5 to 7 (6.14 years). Visual perception scores were measured with a sketch and maternal feeding style was measured with validated "Parental Feeding Style Questionnaire". RESULTS: The parental feeding dimensions "emotional feeding" and "encouragement to eat" subscale scores were low in overweight children according to visual perception classification. "Emotional feeding" and "permissive control" subscale scores were statistically different in children classified as correctly perceived and incorrectly low perceived group due to maternal misperception. CONCLUSION: Various feeding styles were related to maternal visual perception. The best approach to preventing obesity and underweight may be to focus on achieving correct parental perception of the weight status of their children, thus improving parental skills and leading them to implement proper feeding styles.

Introducción: El estilo de alimentación es uno de los factores prominentes que determina la ingesta de energía. Uno de los factores que influyen en el estilo de alimentación paterna es la percepción de los padres del estado de peso del niño. Objetivo: El propósito de este estudio fue evaluar la relación entre la percepción visual de la madre del estado de peso de su hijo y su estilo de alimentación. Método: Se realizó un estudio transversal con madres de 380 niños preescolares de 5 a 7 (6,14 años). Las puntuaciones de la percepción visual se midieron mediante unos dibujos y el estilo de alimentación materna se medió con el cuestionario validado "Parental Feeding Style Questionnaire". Resultados: Las puntuaciones de las subescalas de las dimensiones de alimentación parental "alimentación emocional" y "animar a comer" eran bajas en niños con sobrepeso de acuerdo con la clasificación de la percepción visual. Las puntuaciones de las subescalas "alimentación emocional" y "control permisivo" eran estadísticamente distintas en los niños clasificados como correctamente percibidos e incorrectamente percibidos bajos por una mala percepción materna. Conclusión: Diversos estilos de alimentación se relacionaban con la percepción visual materna. El mejor abordaje para evitar la obesidad y el peso bajo podría estar en centrarse en conseguir una correcta percepción parental del estado de peso de sus hijos, mejorando así las habilidades paternas y conllevando la implantación de unos estilos de alimentación adecuados.