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Background During perimenopause, the rise in serum follicle-stimulating hormone (FSH) is associated with increased adiposity, insulin resistance (IR), and metabolic syndrome (MetS). However, data for the pubertal period, which is characterized by increasing FSH levels and changing body composition, are limited. Objectives To investigate the relationships between FSH and anthropometric changes, IR markers, and development of MetS in the peripubertal period. Methods Uppsala Longitudinal Study of Childhood Obesity (ULSCO) is an ongoing study that aims to understand the factors contributing to childhood obesity and the development of obesity-related diseases. We analysed the subset of participants who were prepubertal at the first visit (n = 95, 77 with obesity). Mean follow-up time was 3.0 +/- 1.4 years. Results Higher serum FSH levels at the first visit were associated with an increased likelihood of elevation in body mass index (BMI SDS) (p = 0.025, OR = 16.10) and having MetS (p = 0.044, OR = 4.67) at the follow-up. We observed nonlinear relationships between varying serum FSH levels and markers of adiposity and IR, especially in girls. At the first visit, when girls were prepubertal, FSH was negatively associated with BMI (beta = -0.491, p = 0.005) and positively associated with sex hormone-binding globulin (SHBG) (beta = 0.625, p = 0.002). With the progression of puberty, negative associations between BMI and SHBG disappeared while FSH became positively associated with HOMA-IR (beta = 0.678, p = 0.025) and fasting insulin (beta = 0.668, p = 0.027). Conclusions Higher serum FSH levels in prepubertal children were associated with an increased risk of MetS development during pubertal transition. Along with nonlinear associations between varying serum FSH levels and IR markers, our results might imply a relationship between FSH and IR of puberty.

Context: Limited data are available on the exact incidence of disorders of sex development (DSD) with genital ambiguity at birth. Objective: To determine frequency of ambiguous genitalia in newborns. Design: Prospective multicenter study. Setting: Three tertiary care hospitals. Patients or Other Participants: All 14,177 babies born during the study period were included. Main Outcome Measures: All newborns were examined at birth; data on weeks of gestation, birth weight, and length were collected. A structured questionnaire was used for data collection. Quigley and Prader scales were used for phenotypic grading. Clinical and genetic investigations were performed. Results: Eighteen babies with ambiguous genitalia were found among 14,177 newborns (1.3/1000). Fifteen newborns had 46, XY DSD, one had 46, XX congenital adrenal hyperplasia, and one had 45, X/46, XY mixed gonadal dysgenesis. Karyotype analysis was not done in one baby who died in the neonatal period. The ratio of prematurity was higher in the DSD group (44% vs 11%; P < 0.001) and the ratio of small for gestational age was also higher in the DSD group (22% vs 5%; P = 0.007). Eight babies with DSD had mothers who had additional medical conditions, such as preeclampsia, depression, insulin resistance, and gestational diabetes mellitus. Conclusion: The frequency of ambiguous genitalia was higher than in previous studies, but, as with any experiment, the finding should be met with caution because this study was conducted in tertiary care hospitals. In addition, lower birth weight in the DSD group supports the hypothesis that early placental dysfunction might be important in the etiology of male genital anomalies. Copyright (C) 2019 Endocrine Society

Objectives We aimed to evaluate total serum calcium (TSC) and ionized serum calcium (ISC) levels and their effects on clinical outcomes in neonates underwent exchange transfusion (ET). Method In this study, the data of newborn infants who underwent ET due to hyperbilirubinemia in a third level neonatal intensive care unit (NICU) were retrospectively analyzed. The patients were monitored by electrocardiogram during ET. Cardiac and respiratory rates, peripheral oxygen saturation, blood pressure values ​​and clinical findings as convulsion, tremor, hypertonia, laryngospasm, cyanosis and apnea were recorded in ET observation forms. The infants with no symptoms of hypocalcemia during the procedure were not routinely given IV calcium gluconate. TSC and ISC measured at the beginning, at the end and 24 h after the end of ET were evaluated retrospectively. Results Data of 36 newborn patients were evaluated. Median gestational age was 39 (35−40) weeks, mean birthweight was 2840 ± 841 (mean ± SD) grams. During the ET, desaturation was observed in five patients(13.9 %), sinus bradycardia in six(16.7 %), tachypnea in two(5.5 %), sinus tachycardia in one(2.8 %), and rare ventricular extrasystoles in one(2.8 %). Hypocalcaemia was not detected in any of the patients at the beginning of ET. Hypocalcemia was observed in two cases (5.5 %) at the end of ET. There was no statistically significant difference between the TSC and ISC levels at the beginning of ET, at the end and at the end of 24 h. Conclusion As a result, routine intravenous (IV) calcium administration seems to be unnecessary provided that vital signs and neurological status are closely monitored during ET.

Low levels of SHBG have become a marker for insulin resistance and diabetes. Babies born to mothers who are obese, have diabetes, or smoke during pregnancy are at greater risk of developing obesity and diabetes later in life.To examine the impact of maternal obesity, diabetes and smoking on SHBG levels in newborns.This cross-sectional study is part of an ongoing multicenter, longitudinal study.98 healthy newborns and their parents, including 16 mothers with diabetes and 31 mothers with a smoking history.Cord blood and second day venipuncture samples were collected for measurement of SHBG and insulin.Babies born to mothers with diabetes had lower SHBG levels in cord blood [14.0 (8.9-20.4) vs. 19.6 (14.9-25.1) nmol/L; p=0.011] and on day 2 [18.8 (12.6-21.2) vs. 22.9 (17.1-29.1) nmol/L; p=0.015] than controls. Maternal diabetes remained negatively associated with SHBG levels in cord blood (p=0.02) and on day 2 (p=0.04) when adjusted for mothers' age, smoking status, pre-pregnancy weight and weight gain during pregnancy. SHBG levels in cord blood and day 2 samples were similar in babies born to mothers who were overweight-obese but not diabetic vs. normal weight, or were smokers when compared to non-smokers.SHBG levels are lower in newborns born to mothers with diabetes than without diabetes, and may be a marker for babies' life-long risk for abnormal metabolic health. On the other hand, the adverse effects of tobacco smoke on the fetus do not appear to directly influence SHBG levels.Low levels of SHBG have become a marker for insulin resistance and diabetes. Babies born to mothers who are obese, have diabetes, or smoke during pregnancy are at greater risk of developing obesity and diabetes later in life.To examine the impact of maternal obesity, diabetes and smoking on SHBG levels in newborns.This cross-sectional study is part of an ongoing multicenter, longitudinal study.98 healthy newborns and their parents, including 16 mothers with diabetes and 31 mothers with a smoking history.Cord blood and second day venipuncture samples were collected for measurement of SHBG and insulin.Babies born to mothers with diabetes had lower SHBG levels in cord blood [14.0 (8.9–20.4) vs. 19.6 (14.9–25.1) nmol/L; p=0.011] and on day 2 [18.8 (12.6–21.2) vs. 22.9 (17.1–29.1) nmol/L; p=0.015] than controls. Maternal diabetes remained negatively associated with SHBG levels in cord blood (p=0.02) and on day 2 (p=0.04) when adjusted for mothers’ age, smoking status, pre-pregnancy weight and weight gain during pregnancy. SHBG levels in cord blood and day 2 samples were similar in babies born to mothers who were overweight-obese but not diabetic vs. normal weight, or were smokers when compared to non-smokers.SHBG levels are lower in newborns born to mothers with diabetes than without diabetes, and may be a marker for babies’ life-long risk for abnormal metabolic health. On the other hand, the adverse effects of tobacco smoke on the fetus do not appear to directly influence SHBG levels.

Introduction Despite much advancement in medicine, endocrine and metabolic diseases remain an important cause of morbidity and even mortality in children. Objective The present study was planned to investigate the evaluation of hearing that also includes high frequencies, and the presence and degree of vertigo and tinnitus symptoms in pediatric patients diagnosed with endocrine diseases such as type 1 diabetes mellitus (DM), growth hormone deficiency (GHD), obesity, idiopathic short stature, and precocious puberty Methods The present study included a patient group of 207 children patients diagnosed with endocrine disease (95 males, 112 females; mean age 9.71 years old [range 6–16 years old]) and a control group including 55 healthy children who do not have any kind of chronic disease (26 males, 29 females; mean age 9.33 years old [range 6–16 years old]). The subjects underwent a hearing test with frequencies between 250 and 20,000 Hz. The vestibular and tinnitus symptoms were evaluated with the Pediatric Vestibular Symptom Questionnaire. Results Out of 207 patients in the patient group, 5 (2.4%) had hearing loss in pure tones, 10 (4.8%) had it in high frequencies, 40 (19.3%) had tinnitus symptoms, and 18 (8.7%) had vertigo symptoms. A total of 4 out of 207 patients in the study group (1.9%), 2 out of 59 with type 1 DM patients (3.4%), 1 out of 46 with GHD (2.2%), and 1 out of 43 obesity patients (2.3%) had hearing loss, vertigo, and tinnitus symptoms. Conclusions Our results suggest that some childhood endocrine diseases can cause some changes in the inner ear, although the exact cause is unknown. Perhaps, a detailed hearing and balance examination should be a routine in a child diagnosed with an endocrine disease. We think it is necessary to work on more comprehensive patient groups and tests in the future.

Background: SHBG levels are low in obesity, and low SHBG levels are a biomarker for the development of T2DM and the metabolic syndrome. We sought to determine whether low SHBG in newborns will predict childhood obesity. Methods: We studied 94 healthy, singleton, full-term newborns, and measured their length, weight (BW), waist circumference, and skinfold thicknesses. We collected cord blood as well as day 2 venous blood samples for the measurement of SHBG and insulin (ALPCO, Salem NH). Maternal age, pre-pregnancy weight, pregnancy weight gain, and glucose screening test results were obtained from obstetrical records. Mothers with chronic diseases were excluded from the study. When babies were 2 years old, we administered a questionnaire to collect information about their eating, sleeping, screen viewing habits, and anthropometric measurements at ages 6, 12, and 24 months (n=47). Overweight was defined as a BMI SDS of ≥1 and

Factors associated with aortic dilation and dissection in patients with Turner syndrome (TS) remain unclear. We assessed magnetic resonance imaging-based aortic diameters at nine predefined anatomic positions and examined associations of increased aortic diameters with B-type natriuretic peptide (BNP), A-type NP (ANP), growth hormone treatment, insulin-like growth factor 1 (IGF1), and estrogen status. Forty-seven patients with TS aged 7.3–21 years and 34 healthy peers were enrolled in this study. Aortic diameters were higher in patients with TS at three positions than in controls (p

Background: As KATP channel mutations are the most common cause of neonatal diabetes mellitus (NDM) and patients with these mutations can be treated with oral sulfonylureas, empiric therapy is a common practice for NDM patients. Case presentation: A non-syndromic, small for gestational age baby born to first-degree consanguineous parents was diagnosed with NDM. Because of hypo- and hyperglycemic episodes and variability in insulin requirement, we initiated a trial of glibenclamide, with a presumptive diagnosis of NDM caused by a KATP channel mutation. However, this empiric sulfonylurea trial did not improve the patient’s glycemic control and resulted in resistance to exogenous insulin. Genetic testing identified a previously reported homozygous INS promoter mutation (c.-331C>G), which was not responsive to sulfonylurea therapy. Conclusions: In light of our results, we recommend to confirm the genetic diagnosis as soon as possible and decide on sulfonylurea treatment after a genetic diagnosis is confirmed.

Objective To investigate the relationship between brain masculinization and retinal thickness in children with congenital adrenal hyperplasia (CAH). Methods Forty-five patients with CAH aged between 4 and 18 years and 30 age-matched healthy controls were included in this prospective study. Macular area was examined with optical coherence tomography (OCT); central subfield thickness (CST), cube volume (CV) and macular retinal thickness (MT) were measured in each subject. A gender identity questionnaire (GIQ) was used for the evaluation of gender happiness index. Results Girls with CAH had a higher CV (p = 0.002) and MT (p = 0.003) than healthy girls. No significant difference was found between boys with CAH and healthy boys regarding the retinal thickness measurements. Mean CST, CV and MT were significantly higher in boys than in girls in the control group (p = 0.013, p Conclusions As retina is part of the brain, our finding appears to be a morphological evidence of the excess androgen exposure on brain structures in girls with CAH. In addition, we suggest using retinal thickness measurements as a marker of prenatal excess androgen exposure in future studies.

Background: Thiamine responsive megaloblastic anemia syndrome (TRMA) is an autosomal recessive disorder characterized by non type 1 diabetes mellitus (DM), sensorineural hearing loss and megaloblastic anemia and caused by mutations in SLC19A2 gene, encoding a thiamine transporter protein. Case: A 3-month-old male infant presented with megaloblastic anemia, DM, patent ductus arteriosus and hypertriglyceridemia. His autoimmune markers for DM were positive but with the additional finding of sensorineural deafness he was diagnosed with TRMA and thiamine therapy was started. His anemia was improved and insulin needs decreased and his genetic studies revealed a homozygous frameshift mutation, c.641del within coding region of SLC19A2 gene. Conclusion: Clinical presentation of TRMA could be highly variable and some co-existence could perplex physicians, but this diagnosis should be considered in all patients with DM and anemia and further assessment should be done.

Background Although studied widely in adulthood, little is known about endocrinological disorders during critical illnesses in childhood. The aims of this study were to define the endocrinological changes in patients admitted to pediatric intensive care unit (PICU) and to identify their effects on prognosis. Methods Forty patients with a mean age of 5.1 years admitted to PICU were enrolled in the study. Blood samples were taken at admission and at 24 and 48 h to measure cortisol, adrenocorticotropic hormone (ACTH), prolactin, growth hormone (GH), GH binding protein (GHBP), insulin-like growth factor-binding protein-3 (IGFBP-3) and interleukin-6 (IL-6). The severity of the patient's condition was assessed using pediatric risk of mortality (PRISM) and pediatric logistic organ dysfunction (PELOD) scores. Results PRISM and PELOD scores were significantly higher in non-survivors. Cortisol, ACTH, prolactin, GH, GHBP, IGFBP-3 and IL-6 were not significantly different between the survivors and non-survivors. There was a negative correlation between baseline IGFBP-3 and PRISM scores. A positive correlation was seen between cortisol level at 24 h and PRISM score. On multivariate linear regression analysis, PRISM score was best explained by ACTH and cortisol at 24 h. A positive weak correlation was detected between IL-6 at 24 h and PELOD scores. Conclusions Although there was no difference between survivors and non-survivors regarding the studied endocrine parameters, there were associations between cortisol, ACTH, IL-6 and IGFBP-3 and risk assessment scores, and, given that these scores correlated with mortality, these parameters might be useful as prognostic factors.

siklar, zeynep/0000-0003-0921-2694; berberoglu, merih/0000-0003-3102-0242; Peltek Kendirci, Havva Nur/0000-0001-7398-765X WOS: 000329913500012 PubMed: 24013997 Objective: To evaluate the adherence to growth hormone (GH) therapy and identify the influencing factors and outcomes in children. Methods: A total of 217 GH-naive patients in 6 pediatric endocrinology clinics were enrolled in the study. Structured questionnaires were filled out and patients were evaluated at the initiation and 3rd, 6th, and 12th months of therapy. Patients were categorized into 4 adherence segments based on percentage of doses omitted at each evaluation period, classified as excellent if 0%, good if 5%, fair if 5 to 10%, and poor if > 10%. Results: There was a decrement in adherence to GH therapy during the study period (P = .006). Patients who showed excellent and good adherence to therapy had better growth velocity and growth velocity standard deviation scores (SDSs) (P = .014 and P = .015, respectively). A negative correlation between growth velocity SDS and number of missed injections was also observed (r = -.412; P = .007). A positive correlation between delta insulin-like growth factor-1 (IGF-1) SDS and growth velocity was demonstrated (r = .239; P = .042). IGF-1 levels were significantly higher in patients who showed excellent and good adherence to therapy (P = .01). Adherence was better in boys than in girls (P = .035), but adherence rates were not associated with age, cause of GH treatment, socioeconomic status, person who administered the injections, type of injection device, or GH product. Conclusion: Poor adherence to GH therapy was common in our group of patients and was one of the factors underlying suboptimal growth during therapy. Before considering other problems that can affect growth, clinicians should confirm good adherence to therapy.

Objective: The aim of this study was to evaluate the girls referred to the pediatric outpatient clinic with a presumptive diagnosis of early puberty due to early onset of breast development or pubarche. Methods: Within the study period, we evaluated 289 subjects referred for concerns about early onset of puberty. History, anthropometric data, bone age (BA), hormones including luteinizing hormone (LH), follicle-stimulating hormone (FSH), estradiol, and dehydroepiandrosterone sulfate, as well as pelvic ultrasonography (USG)-derived ovarian and uterine volumes were analyzed. Results: Of the 289 girls referred for early onset of pubertal development, 64 (22.1%) had false alarms for puberty. Of the remaining 225 girls, 41 (18.2%) were diagnosed as premature pubarche, 56 (24.9%) as premature thelarche (PT), and 128 (56.9%) as precocious puberty (PP). Girls with early-onset puberty had more advanced BA, greater uterine and ovarian volumes, and also higher LH values than subjects with PP and PT. Nearly half of these girls were 7-8 years of age. Body mass index (BMI) standard deviation score was significantly higher in the PP cases. Conclusions: There is a need for primary care physicians to be more knowledgeable on puberty and on puberty problems. There seems to be a preponderance of PP in 7-8-year-old children . Increased BMI may have a role in the trend towards earlier onset of puberty Conflict of interest:None declared.

BackgroundIn this study, we examined the hypothesis that weight gain and linear growth during the first years of life influence the onset of puberty both in girls and in boys.MethodsA cohort of 157 healthy children, aged 6-9 years, was evaluated and their growth patterns were analyzed retrospectively. Repeated measures mixed model was used to examine the longitudinal anthropometric data.ResultsGirls with pubertal signs were heavier than their peers starting at 9 months of age (P=0.02), and the difference became more evident over time (P

Study Objective To investigate the characteristics of children with ovarian cysts and evaluate treatment strategies. Design Retrospective study. Setting Eight pediatric endocrinology clinics, Turkey. Participants A total of 100 children and adolescents with ovarian cysts. Interventions Patient data collected via retrospective chart review. Patients were stratified according to age into 4 groups (newborns, 1-12 months, 1-8 years, and 8-18 years). Main Outcome Measures Special emphasis was given to torsion and tumor cases, concomitant diseases, treatment modalities, and problems during follow-up. Results Most newborns and infants were asymptomatic with the cysts being discovered incidentally; in girls ages 1-8, symptoms were common, including breast budding (47.1%, 16 of 34) and vaginal bleeding (29.4%, 10 of 34). Girls older than 8 years mostly presented with abdominal pain (31.6%, 12 of 38) and menstrual irregularity (21.1%, 8 of 38). Most of our patients were diagnosed with a simple ovarian cyst, but 9 patients were found to have ovarian tumors. Ovarian torsion was detected in 7 patients; 5 with large and 2 with small cysts (

Permanent neonatal diabetes mellitus (PNDM) is a rare condition presenting before six months of age. Mutations in the genes encoding the ATP-sensitive potassium (KATP) channel are the most common causes. Sulfonylurea (SU) therapy leads to dramatic improvement in diabetes control and quality of life in most patients who carry these mutations. Here, we report the long-term follow-up results of two siblings with PNDM who were treated with insulin until ABCC8 gene mutation was identified, and were successfully transferred to oral SU therapy. After 3.5 years of follow-up on SU, one patient had a very good response, while the other one had a poor response. Bad compliance to diet was thought to be the most probable reason for poor glycemic control in this patient. In conclusion, molecular genetic diagnosis in all patients with PNDM is recommended. Compliance to treatment should be an important aspect of the follow-up of these patients. Conflict of interest:None declared.

Background/Aim: The exon 3-deleted/full-length (d3/fl) growth hormone (GH) receptor (GHR) polymorphism has been associated with responsiveness to GH therapy in some diagnostic groups. However, there are still controversies on this issue. To evaluate the effect of the GHR exon 3 polymorphism on growth after 1 and 2 years of GH therapy in Turkish patients with GH deficiency (GHD) and Turner’s syndrome (TS) and the distribution of GHR exon 3 isoforms. Materials and Methods: 218 patients with GHD (125 males/93 females) and 43 patients with TS were included in the study. The control group included 477 healthy adults aged from 18 to 57 years (54 females/423 males). Anthropometric parameters and insulin-like growth factor (IGF)-1 and IGF binding protein (IGFBP)-3 were evaluated annually. GHR isoforms were studied using simple multiplex PCR. Height and body mass index were expressed as standard deviation score (SDS). Results: There were no differences among TS, GHD and healthy adults regarding the distribution of GHR exon 3 isoforms (fl/fl, fl/d3 and d3/d3). There was a significant increase in height SDS in both diagnostic groups on GH therapy; however, there were neither differences in height SDS and Δheight velocity between fl/fl, fl/d3 and d3/d3 groups nor a correlation between the distribution of GHR exon 3 isoforms and change in IGF-1 SDS and IGFBP-3 SDS levels on GH therapy in either of the diagnostic groups. There was also no gender difference in GHR isoforms in healthy adults. Conclusion: The results suggest that responsiveness to GH therapy does not depend on the exon 3 GHR genotypes in GHD and TS patients.

The aim of this study was to evaluate the thyroid function alterations in a group of epileptic children taking antiepileptic drugs (AEDs). Patients demographic data and the free throxine (fT4) and thyroid-stimulating hormone (TSH) levels at the beginning of the treatment and at the third, sixth and ninth months of AED treatment were recorded retrospectively. A total of 106 children, 59 males and 47 females, were enrolled in the study. Mean patient age was 3.7 years, ranging between 3 months and 14 years. In total, 54% of patients were on valproic acid (VPA), 16% phenobarbital (PB), 14% were on carbamazepine (CBZ), 6% were on oxcarbazepine (OXC), 5% were on levetiracetam, and 5% were on topiramate therapy. There were no significant differences in average fT4 values between the drug groups. But the mean fT4 levels of the patients on VPA therapy showed a clear decrease within the observation period. No significant difference in average TSH values between the groups was detected in the beginning and in the third and sixth month. However, in the ninth month, a significant increase in TSH values was found in the VPA group (p = 0.007). In the patients taking VPA, average TSH values rose progressively while staying within normal limits. During follow-up, thyroid dysfunction were found in 21 patients (19.6%). A statistically significant relationship was found between severe electroencephalography (EEG) findings and thyroid dysfunction (p = 0.041). It was concluded that epileptic children with severe EEG findings and using VPA could have thyroid dysfunction. These patients should be followed up closely by thyroid function tests during treatment.

ABALI, SAYGIN/0000-0001-6552-2801; Gurbuz, Fatih/0000-0003-2160-9838; Hatun, Sukru/0000-0003-1633-9570; Abaci, Ayhan/0000-0002-1812-0321; Turan, Serap/0000-0002-5172-5402; Buyukinan, Muammer/0000-0002-2937-823X; yuksel, bilgin/0000-0003-4378-3255; Ozgen, Ilker Tolga/0000-0001-6592-9652; binay, cigdem/0000-0002-7749-8818; ozkan, Behzat/0000-0002-9153-8409; Demir, Korcan/0000-0002-8334-2422; Eren, Erdal/0000-0002-1684-1053 WOS: 000373099300016 PubMed: 26788866 To evaluate the anthropometric features of girls with Turner syndrome (TS) at birth and presentation and the effect of karyotype on these parameters. Data were collected from 842 patients with TS from 35 different centers, who were followed-up between 1984 and 2014 and whose diagnosis age ranged from birth to 18 years. Of the 842 patients, 122 girls who received growth hormone, estrogen or oxandrolone were excluded, and 720 girls were included in the study. In this cohort, the frequency of small for gestational age (SGA) birth was 33%. The frequency of SGA birth was 4.2% (2/48) in preterm and 36% (174/483) in term neonates (P

Sex hormone-binding globulin (SHBG) is a circulating glycoprotein that transports testosterone and other steroids in the blood. Interest in SHBG has escalated in recent years because of its inverse association with obesity and insulin resistance, and because many studies have linked lower circulating levels of SHBG to metabolic syndrome, type 2 diabetes, nonalcoholic fatty liver disease, polycystic ovary syndrome, and early puberty. The purpose of this review is to summarize molecular, clinical, endocrine, and epidemiological findings to illustrate how measurement of plasma SHBG may be useful in clinical medicine in children.

Even early life in utero cannot provide a safe place for newborns. The first acquaintance with chemicals takes place in utero and/or with mother's milk after delivery. Besides legislations and bans to persistent organic pollutants (POPs), these chemicals are still affecting the general population especially the children as they are one of the populations most susceptible to chemicals, and also the health problems may arise in the future. Our objective is to collect the first data in newborns in Turkey to determine baseline levels of POPs in the general population and estimate the potential cancer risk related to exposure. Twenty-nine organochlorine pesticides (OCPs) and 18 polychlorinated biphenyl (PCB) congeners in blood samples of newborn boys (0-1 month old) who were born in Istanbul, Turkey, in 2010-2012 were evaluated with high-resolution gas chromatography-high-resolution mass spectrometry (HRGC/HRMS). Results for analyzed chlorinated compounds are as follows: hexachlorocyclohexane (Sigma HCH) 1828 +/- 3650 pg/g lipid, dichlorodiphenyltrichloroethane (Sigma DDT) 10,000 +/- 15,398 pg/g lipid, and Sigma PCB 1068 +/- 1823 pg/g lipid. 4,4'-DDT, 4,4'-dichlorodiphenyldichloroethylene (4,4'-DDE), hexachlorobenzene, and PCB 138 and 153 are the major contaminants. New POPs as lindane 61 +/- 268 pg/g lipid, pentachlorobenzene 13 +/- 74 pg/g lipid, and endosulfan 29 +/- 106 pg/g lipid are also detected in blood. Estimated total risk for lifetime PCB exposure is less than 1 x 10(-5), an acceptable risk. Blood concentration levels will be important base data in the assessment of health concerns of newborns as well as for studies about how endocrine disruptors affect humans.

WOS: 000360842500004, PubMed: 26831551, Objective: Children with Turner syndrome (TS) have a specific growth pattern that is quite different from that of healthy children. Many countries have population-specific growth charts for TS. Considering national and ethnic differences, we undertook this multicenter collaborative study to construct growth charts and reference values for height, weight and body mass index (BMI) from 3 years of age to adulthood for spontaneous growth of Turkish girls with TS. Methods: Cross-sectional height and weight data of 842 patients with TS, younger than 18 years of age and before starting any therapy, were evaluated. Results: The data were processed to calculate the 3rd, 10th, 25th, 50th, 75th, 90th and 97th percentile values for defined ages and to construct growth curves for height-for-age, weight-for-age and BMI-for-age of girls with TS. The growth pattern of TS girls in this series resembled the growth pattern of TS girls in other reports, but there were differences in height between our series and the others. Conclusion: This study provides disease-specific growth charts for Turkish girls with TS. These disease-specific national growth charts will serve to improve the evaluation of growth and its management with growth-promoting therapeutic agents in TS patients., Turkish Pediatric Endocrinology and Diabetes Society [012013], This work was supported by the Turkish Pediatric Endocrinology and Diabetes Society (Grand number: 012013).

The human body is not a chemically uncontaminated system. Every simple action that humans undertake, such as drinking water, eating, nursing, and even breathing air, puts the system under environmental xenobiotic exposure stress. Environmental chemicals have been shown to produce unwanted effects on health and remove the right to healthy living, starting from the first encounter in utero to geriatrics, throughout the lifespan. Organochlorine pesticides (OCPs) and polychlorinated biphenyl (PCB) levels, important members of the persistent organic pollutants (POPs), have been detected before in human breast milk and also in the adipose tissue of women from different regions of Turkey; however, there was no information about the blood levels of these chemicals. This study generated the first information that evaluates OCP and PCB contamination levels in the blood of the women living in Turkey. The current study measured the blood concentrations of OCPs and PCBs in 58 healthy women (age 20-41 years; mean age 28 years) who were living in Istanbul, Turkey, in the years 2010-2012. Samples were analyzed for 29OCPs and 18 PCB congeners using high-resolution gas chromatography/high-resolution mass spectrometry (HRGC/HRMS). PCB 153 was the predominant congener (643.2 pg/g lipid), followed by PCB 138 and PCB 180. 4,4'-DDE (24872.8 pg/g lipid) was the most common organochlorinated pesticide contaminant in studied blood samples. Results for analyzed chlorinated compounds were as follows: Sigma PCB 2682 +/- 3300 pg/g lipid; Sigma DDT 25,938+28,644 pg/g lipid; and Sigma HCH 2930 +/- 2222 pg/g lipid, respectively. The mean concentration of Sigma WHOPCB-TEQ was 0.037 pg/g on a lipid basis. This information will be important base data during the assessment of the general health concerns of women, as well as for studies about how endocrine disruptors affect humans for forthcoming studies.

WOS: 000218770800002 PubMed ID: 27617131 Congenital hypothyroidism (CH) is the most common neonatal endocrine disease, and germ-line mutations in the TPO gene cause the inherited form of the disease. Our aim in this study was to determine the genetic basis of congenital hypothyroidism in three affected children coming from a consanguineous Turkish family. Because CH is usually inherited in autosomal recessive manner in consanguineous/multicase families, we adopted a two-stage strategy of genetic linkage studies and targeted sequencing of the candidate genes. First, we investigated the potential genetic linkage of the family to any known CH locus, using microsatellite markers, and then screened for mutations in linked-gene by conventional sequencing. The family showed potential linkage to the TPO gene and we detected a homozygous duplication (c. 1184_1187dup4) in all cases. The mutation segregated with disease status in the family. This study confirms the pathogenicity of the c. 1184_1187dup4 mutation in the TPO gene and helps establish a genotype/phenotype correlation associated with this mutation. It also highlights the importance of molecular genetic studies in the definitive diagnosis and accurate classification of CH.

WOS: 000351307200005, PubMed ID: 25800473, Objective: Turner syndrome (TS) is a chromosomal disorder caused by complete or partial X chromosome monosomy that manifests various clinical features depending on the karyotype and on the genetic background of affected girls. This study aimed to systematically investigate the key clinical features of TS in relationship to karyotype in a large pediatric Turkish patient population. Methods: Our retrospective study included 842 karyotype-proven TS patients aged 0-18 years who were evaluated in 35 different centers in Turkey in the years 2013-2014. Results: The most common karyotype was 45, X (50.7%), followed by 45, X/46, XX (10.8%), 46, X, i(Xq) (10.1%) and 45, X/46, X, i(Xq) (9.5%). Mean age at diagnosis was 10.2 +/- 4.4 years. The most common presenting complaints were short stature and delayed puberty. Among patients diagnosed before age one year, the ratio of karyotype 45, X was significantly higher than that of other karyotype groups. Cardiac defects (bicuspid aortic valve, coarctation of the aorta and aortic stenosis) were the most common congenital anomalies, occurring in 25% of the TS cases. This was followed by urinary system anomalies (horseshoe kidney, double collector duct system and renal rotation) detected in 16.3%. Hashimoto's thyroiditis was found in 11.1% of patients, gastrointestinal abnormalities in 8.9%, ear nose and throat problems in 22.6%, dermatologic problems in 21.8% and osteoporosis in 15.3%. Learning difficulties and/or psychosocial problems were encountered in 39.1%. Insulin resistance and impaired fasting glucose were detected in 3.4% and 2.2%, respectively. Dyslipidemia prevalence was 11.4%. Conclusion: This comprehensive study systematically evaluated the largest group of karyotype-proven TS girls to date. The karyotype distribution, congenital anomaly and comorbidity profile closely parallel that from other countries and support the need for close medical surveillance of these complex patients throughout their lifespan.

PubMedID: 25500790 To investigate the specific mutations in PROP1, POU1F1, LHX3, and HESX1 genes in patients with combined pituitary hormone deficiency (CPHD) in Turkey. Seventy-six patients with CPHD were included in this study. Based on clinical, hormonal, and neuro-radiological data, relevant transcription factor genes were evaluated by Sanger sequencing and multiplex ligation-dependent probe amplification. Total frequency of mutations was 30.9 % in patients with CPHD. Frequency was significantly higher in familial patients (p = 0.001). Three different types of mutations in PROP1 gene (complete gene deletion, c.301-302delAG, a novel mutation; IVS1+2T>G) were found in 12 unrelated patients (21.8 %). Mutations in PROP1 gene were markedly higher in familial than in sporadic cases (58.8 vs. 5.3 %, p

Herbs have been used for centuries to prevent and control many diseases. The biggest challenge and problem is lack of information about the effect of herbs and its side effects. Thyme (thymus vulgaris) is a small shrubby plant with a strong, spicy taste, and odor. Thyme has carminative, diaphoretic, expectorant, sedative, antibacterial and antifungal properties. It also has antispasmodic effects; tea made by infusing the herb in water or thyme juice ready-to-use is traditionally frequently used for infantile colic in our country. A fourteen-day-old male newborn was admitted to the emergency department with severe respiratory distress. There was a history of 50 mL of thyme juice (added table sugar) ingestion given for his infantile colic two hours before admission. He had hypoglycemia, hyperuricemia, and lactic acidosis. Further investigation confirmed fructose 1-6 diphosphatase deficiency in the patient. We thought that lactic acidosis may have been triggered by fructose added to the thyme water to sweeten its taste. However, phenolic compounds of thyme juice may also cause acidosis.

Objective In this study, we aimed to investigate the genetic background of thyroid dyshormonogenesis (TDH). Context Thyroid dyshormonogenesis comprises 10-15% of all cases of congenital hypothyroidism (CH), which is the most common neonatal endocrine disorder, and might result from disruptions at any stage of thyroid hormone biosynthesis. Currently seven genes (NIS, TPO, PDS, TG, IYD, DUOX2 and DUOXA2) have been implicated in the aetiology of the disease. Design As TDH is mostly inherited in an autosomal recessive manner, we planned to conduct the study in consanguineous/multi-case families. Patients One hundred and four patients with congenital TDH all coming from consanguineous and/or multi-case families. Measurements Initially, we performed potential linkage analysis of cases to all seven causative-TDH loci as well as direct sequencing of the TPO gene in cases we could not exclude linkage to this locus. In addition, in silico analyses of novel missense mutations were carried out. Results TPO had the highest potential for linkage and we identified 21 TPO mutations in 28 TDH cases showing potential linkage to this locus. Four of 10 distinct TPO mutations detected in this study were novel (A5T, Y55X, E596X, D633N). Conclusions This study underlines the importance of molecular genetic studies in diagnosis, classification and prognosis of CH and proposes a comprehensive mutation screening by new sequencing technology in all newly diagnosed primary CH cases. WellChild Wellcome Trust European Commission European Commission QEHB Foundation Trust UK Research & Innovation (UKRI) Medical Research Council UK (MRC) (G0601811) UK Research & Innovation (UKRI) Medical Research Council UK (MRC) European Commission (G0601811) National Institute for Health Research (NIHR) (NF-SI-0508-10198)

Background There are still controversies whether insulin resistance (IR) develops in preterm born children during early childhood. Objective To investigate the role of leptin, soluble leptin receptor (sOB-R), adiponectin, visfatin and insulin sensitivity in the pathogenesis of possible IR in preterm born children during early childhood. Patients and metods Twenty-nine preterm small for gestational age (SGA) born children (Group 1) and 25 preterm appropriate for gestational age (AGA) born children (Group 2), matched for gestational age and sex were included in the study. Mean chronological age at investigation was 3.3 ± 0.7 years and not different between the groups. Blood samples for fasting blood glucose, insulin, proinsulin, adiponectin, leptin, sOB-R and visfatin were obtained. Results Mean height and weight standard deviation scores (SDS) at investigation were significantly lower in Group 1 than in Group 2, but there was no significant difference in body mass index (BMI) SDS between the groups. Catch-up growth (CUG) was higher in Group 1 than in Group 2. There was no difference regarding homeostasis model assessment for IR (HOMA-IR), leptin, sOB-R, adiponectin, proinsulin and visfatin values between the groups. In the whole group, log visfatin showed a negative correlation with Δweight SDS. There was a positive correlation between HOMA-IR and BMI SDS. Adiponectin levels showed a positive correlation with log visfatin levels in all groups. Conclusion Preterm born children whether AGA or SGA do not show IR in early childhood if BMI is normal. Significant differences between the preterm SGA and preterm AGA groups regarding the adipocytokine levels were not detected.

Idiopathic short stature (ISS) refers to pathophysiologically wide and heterogeneous range of disorders, which are considered to involve defects in growth hormone (GH) insulin like growth factor-1 (IGF-1) axis. This study was designed to evaluate GH- IGF-1 axis and investigate IGF-1 gene polymorphisms in ISS. 108 patients with a mean age of 11.7±3.6 years constituted the study group, while 108 age and gender matched children with normal stature constituted the control group. Serum IGF-1 and insulin-like growth factor binding protein-3 (IGFBP-3) levels and two polymorphisms in IGF-1 gene (rs35767, rs17032362) were investigated. While mean IGF-1 SDS value was lower in study group (p=0.002), no difference was detected between mean IGFBP-3 SDS values. The IGF-1 gene rs35767 polymorphism genotype distribution did not exhibit a statistical difference between study (7.1% wild type, 29.6% heterozygous, 63.3% homozygous) and control groups (3.8% wild type, 39.6% heterozygous, 56.6% homozygous). IGF-1 gene rs17032362 polymorphism genotype distribution was not significantly different either between study (94.8% wild type, 5.2% heterozygous, 0% homozygous) and control groups (97.2% wild type, 2.8% heterozygous, 0% homozygous). Comparing the cases with wild type, homozygous and heterozygous carriers for both polymorphisms with respect to height, weight, BMI, IGF-1 and IGFBP-3 SDS values, no significant difference was detected. IGF-1 SDS levels of patients with ISS were significantly lower compared to control group. There was no difference between IGFBP-3 SDS levels. No effect of IGF-1 gene rs35767 and rs17032362 polymorphisms on stature, IGF-1 and IGFBP-3 levels could be demonstrated.

Congenital hypothyroidism (CH) is the most common neonatal endocrine disorder and results in mental retardation if untreated. Eighty-five percent of CH cases are due to disruptions in thyroid organogenesis and are mostly sporadic, but about 2% of thyroid dysgenesis is familial, indicating the involvement of genetic factors in the aetiology of the disease. In this study, we aimed to investigate the Mendelian (single-gene) causes of non-syndromic and non-goitrous congenital hypothyroidism (CHNG) in consanguineous or multi-case families. Here we report the results of the second part (n=105) of our large cohort (n=244), representing the largest such cohort in the literature, and interpret the overall results of the whole cohort. Additionally, 50 sporadic cases with thyroid dysgenesis and 400 unaffected control subjects were included in the study. In familial cases, first, we performed potential linkage analysis of four known genes causing CHNG (TSHR, PAX8, TSHB, and NKX2-5) using microsatellite markers and then examined the presence of mutations in these genes by direct sequencing. In addition, in silico analyses of the predicted structural effects of TSHR mutations were performed and related to the mutation specific disease phenotype. We detected eight new TSHR mutations and a PAX8 mutation but no mutations in TSHB and NKX2-5. None of the biallelic TSHR mutations detected in familial cases were present in the cohort of 50 sporadic cases. Genotype/phenotype relationships were established between TSHR mutations and resulting clinical presentations. Here we conclude that TSHR mutations are the main detectable cause of autosomal recessively inherited thyroid dysgenesis. We also outline a new genetic testing strategy for the investigation of suspected autosomal recessive non-goitrous CH.

Obesity is associated with a number of risk factors, such as hyperlipidemia, hyperinsulinemia, hypertension, and early atherosclerosis. Evidence indicates that atherosclerosis begins in childhood and progresses over decades. In this work, we examined the relationship between cardiovascular risk factors and ultrasonographic signs of subclinical atherosclerosis in 77 obese children and adolescents compared to 40 non-obese healthy peers. Carotis intima media thickness (cIMT), carotid artery compliance (CAC), brachial artery flow-mediated dilatation (FMD), and established cardiovascular risk factors were studied. In the obese patients, cIMT was significantly increased (0.57 mm vs 0.45 mm, p < 0.001) whereas CAC (1.84% vs 3.29%, p < 0.001) and FMD (9.67 % vs 14.81%, p < 0.001) were significantly decreased. In multiple linear regression analysis, a relation was observed between cIMT, CAC, brachial FMD on one hand, and body mass index (BMI) on the other. Among the lipid anomalies, only hypertriglyceridemia was found to be positively correlated with cIMT. Additionally, we found a significant association between waist circumference (WC) and FMD. These findings indicate that obesity in children is associated with arterial wall alterations and endothelial dysfunction. In hyperlipidemic situations, only hypertriglyceridemia was found to be positively correlated with cIMT. This finding has consistently indicated TG to be a risk factor for the development of atherosclerosis. To our knowledge this is the first study to determine the relation between FMD and WC, which is used as a parameter of obesity in childhood.

Non-clinical safety evaluation using juvenile animals is becoming more important in development of medicinal products for paediatric use. It is considered that many physiological changes occur in juvenile animals during their development and this could affect toxicokinetics (TK) and toxicodynamics (TD) of some drugs. However, the changes in TK caused by physiological development have not been fully investigated. Our previous study showed many differences between juvenile and adult rats, in concentration of serum proteins, mRNA expression of CYP enzymes and transporters, and so on. In this study, TK analysis of single dose study in juvenile rats treated with quinidine, which binds mainly to a1-acid glycoprotein (AGP), was conducted to assess the impact on TK. Quinidine at a dose of 30mg/kg was administered intravenously to 14-, 21and 49-day-old rats (n=4/sex/age), and its concentration in plasma was analyzed. As a result, the AUCall of quinidine decreased with sex maturation between 21and 49-day-old rats from 10,500 to 6900ngh/mL (males), and from 8200 to 5710ngh/mL (females). This decrease is considered to be caused mainly by faster elimination of quinidine in 49-day-old rats because z, the elimination rate constant, increased with age between 21and 49-day-old rats from 0.290 to 0.660 (1/h) in males, 0.306 to 0.842 (1/h) in females. In this presentation, the mechanism of these differences and its impact on TK will be discussed.

Background and Aims The perfusion index (PI); is an easy, non-invasive technique for the assessment of peripheral perfusion. The aim of this study was to determine the peripheral PI reference values and PI variability of clinically and hemodynamically stable newborns during the first five days. Method Pre- (right hand) and postductal (foot) PI values were recorded on the sixth hours, first, second, third and fifth day of 241 newborns life with the new generation pulse oximeter [MASIMO Rad 7 Oximeter, USA]. Results A total of 241 newborns (196 term, 45 preterm) were included in the study. The average gestation age of all newborns was 38.4±2.0 weeks and birth weight was 3204±566 grams. According to the analysis of repeated measurements of term and preterm groups within the first 5 days, PI values of right hand and foot did not vary. However, right hand PI values were significantly higher than foot PI values (p Conclusion The peripheral perfusion of stable newborns did not vary significantly during the first five days. It was considered, higher PI value of term newborns compared to preterm newborns, is the result of early adaptation in the microvascular blood flow. PI values obtained from stable newborns may be guiding for further studies planned on various diseases associated with impaired perfusion.

Background and Aim Researches to identify markers with high sensitivity and specificity in the diagnosis of neonatal sepsis are being held in parallel to recent advances in neonatology. In this study, we aimed to determine predictive values of MPV in the diagnosis of neonatal sepsis. Methods All infants diagnosed with clinical sepsis according to clinical and laboratory findings were included in this prospective study. Blood samples for hemoglobin, hematocrit, number of leukocyte, absolute neutrophyl count (ANC), number of platelet, MPV, CRP, blood culture were obtained from each patient within the first 24 hours of hospitalization. Patients who have positive culture results were accepted as proven sepsis. Patients were separated into three groups as proven sepsis (n=82) (Group-1), clinical sepsis (n=64) (Group-2) and control group (n=142) (Group-3). Results Group-1 CRP levels were higher (p=0.001) and number of platelets were lower (p=0.001) compared with other two groups. Leukocyte, ANC and MPV values were significantly high in Group-1 and Group-2 compared with Group 3 and there was no difference between Group-1 and Group-2 (p=0.001). Negative correlation was observed between MPV and platelet levels (r= –0.24, p=0.001) whereas positive correlation was observed between MPV and CRP levels (r=0.26, p=0.001). It was noted that for CRP- Specificity 82%, sensitivity 92%, negative predictive value (NPV) 83%, positive predictive value (PPV) 91%, and for MPV- Specificity 54%, sensitivity 82%, NPV 63%, PPV 76%. Conclusion High serum MPV levels in addition to CRP levels may be helpful in the diagnosis of newborns suspected to have sepsis.

Background and Aim Levels of serum N-terminal pro-brain natriuretic peptide (NT-proBNP), cystatin-C ve urinary β2 microglobulin in newborns with hypoxic ischemic encephalopathy (HIE) were examined in this study. Methods In this study, 25 infants diagnosed with HIE were evaluated prospectively. The diagnosis was made according to criterias of American Gynaecelogy and Obstetric Academy (ACOG, 2003). Serum creatinine, NT-proBNP, cystatin C and urinary β2 microglobulin in all patients were measured on the 1st and 5th days of hospitalization. Results The mean gestational age was 38.7 weeks and the birth weight was 3255 grams. Patients were classified as stage-1 (n=5), stage-2 (n=15) and stage-3 (n=5) HIE according to Sarnat classification. Therapeutic hypothermia was established in 6 patients. Acute renal failure (ARF) developed in 3 cases with stage 3 HIE. Peritoneal dialysis was performed for 2 of them. First day serum creatinine levels were higher than the 5th day levels (p=0.01). NT-proBNP and cystatin-C levels was significantly lower on the fifth day (p=0.01). Although not statistically significant, urinary β2 microglobulin (mg/g cre) levels on the 1st day were higher than the 5th day (p=0.40). On the first day of hospitalization, a statistically significant correlation between NT-proBNP and creatinine (p=0.02), cystatin-C (p=0.01) and urinary β2 microglobulin levels (p=0.01) were determined. NT-proBNP and cystatin-C levels were significantly high on the first day in infants developing ARF. Conclusion It may be beneficial to evaluate serum N-terminal proBNP ve cystatin-C with creatinin levels in HIE patients for the diagnosis, severity and follow-up of ARF.

Background & aim Congenital heart malformation (CHM) is one of the most frequent and important abnormalities in newborns. In this study we retrospectively analyzed the frequency and distribution of the congenital heart diseases in our NICU. Method Newborns hospitalized in NICU between 2005 and 2011 were retrospectively analyzed. Gestational age, birth weight, consanguinity, type of congenital heart disease extracted from the computerized database. CHMswere classified as follows; left-to-right shunt, obstructive, cyanotic with decreased pulmonary flow, cyanotic with increased pulmonary flow and others. Results A total of 706 newborns were diagnosed as congenital heart disease during 7-year study period among the 7450 admission (9.5%). Consanguinity rate was 22.3% and 30.4% of these were first degree relatives. 42.7%, 17.3%, 13%, 11.6% were left-to-right shunt, obstructive, cyanotic with decreased pulmonary flow, cyanotic with increased pulmonary flow and others, respectively. Most frequent heart malformations were ASD (25.5%), VSD (12.6%), Aortic coarctation (10.8%), PDA (9.5%), TGA (8.8%), pulmonary atresia (8.2%), AVSD (4.1%), hypoplastic left heart (3.8%), pulmonary stenosis (3.1%), TOF (2.5%). 37 % of the newborns had at least one congenital malformation in other organ systems. Conclusion ASD, VSD and aortic coarctation were most common congenital heart disease followed in our NICU.

Background and aim Retinopathy of prematurity (ROP) is one of the major morbidity among preterm infants. Although, local anesthetics reduce pain to some extent, eye examination still remains as a painful procedure. We aimed to evaluate the effect of oral sucrose combined with local anesthetics for pain relief during ophthalmological examination. Method A total of forty patients under 32 weeks of gestational age were included in the study. Infants were randomly assigned to receive either oral sucrose solution (Group-1; n=21) or sterile water (Group-2; n=19) combined with topical proparacaine hydrochloride two minutes before examination. Pacifier was used in all patients as non-nutritive sucking during the study. Pain score was evaluated by premature infant pain profile (PIPP) scale. Each infant was videorecorded during and after the procedure. Results Both groups were similar in terms of gestational age, birth weight, postnatal age and actual weight. There was no significant difference between groups in behavioral state, heart rate and oxygen saturation before the examination. At speculum insertion, heart rate variability was similar in both groups whereas oxygen desaturation was apparent in Group-2 (Group-1: 1.7±0.8 and Group-2: 2.5±0.6, p=0.001) and PIPP scores were also lower in Group-1 (Group-1: 14.5±1.8 and Group-2: 17.2±1.7, p=0.001). Total time of crying was significantly shorter in Group-1 (Group-1: 58.8±12.1 and Group-2: 96.3±24, p=0.001). Conclusion Procedural pain is known to have acute and even long term negative. behavioral and developmental effects in neonates. In our study, use of sucrose in addition to local anesthetics during ophthalmological examination is shown to attenuate pain.

Background and Aim In cyanotic congenital heart diseases (CCHD), with the changes in intestinal flora, necrotizing enterocolitis (NEC) and sepsis frequency increases. In this study, the effect of synbiotic use on morbidity and mortality in newborns with CCHD is evaluated. Methods Fifty-seven newborns with CCHD were randomly assigned to receive either synbiotic [probiotic: Bifidobacterium lactis, 5x10 colony forming unit, 30mg+prebiotic: Chicory inulin, 900 mg (1 sachet/day Maflor®, Cidex), n=29] (Group-1) or placebo (n=28) (Group-2). Synbiotic or placebo was started with the first enteral feeding after hospitalization in NICU and continued until the infants were discharged. Results There was no difference regarding the demographic and clinical features between groups. No difference was stated considering the first enteral feeding age and the time interval to reach full enteral feeding between groups. It was also observed that the duration of mechanical ventilation in Group-1 was shorter (p=0.007). Early and late clinical sepsis rate and late culture proven sepsis were lower in Group-1 (p=0.001). NEC (stage≥2) was significantly higher in Grup-2 (p=0.01). The frequency of increased gastric residuals during enteral feding was less in Group-1 (p=0.001). The mortality rate was significantly lower in Group-1 (p=0.02). In multiple regression analysis it was observed that synbiotic use reduces mortality independently from birth weight, gender and surgical attempt (p=0.01). Conclusion Introduction of synbiotics with the first enteral feeding in newborns with CCHD after hospitalization in NICU shows a positive effect on morbidity and mortality.

Background and Aim Cardiac catheterization is an important diagnostic tool. In this study, the frequency and the factors affecting the development of thrombosis were prospectively evaluated in neonates who were subjected to diagnostic and interventional cardiac catheterization. Method 29 patients were enrolled in this study. Blood samples were taken for complete blood count, prothrombin, activated partial thromboplastin time, INR ratio, mutation of factor V Leiden, prothrombin 20210 A, MTHFR C667 and A1298 before the procedure. 50 U/kg bolus of heparin was infused during catheterization and 20 U/kg/hour infusion of heparin was given to patients with clinically suspected thrombosis. Doppler was performed in all patients within 6 hours after catheterization. Results Arterial catheterization in 16 cases, venous catheterization in 7 cases and both were applied in 6 cases. Arterial thrombosis in 7 patients and venous thrombosis in two patients was detected. It’s observed that infusion of 20U/kg/h heparin had no effect on the development of arterial thrombosis. On the development of arterial and venous thrombosis, patient age, gender, diagnosis, treatments received prior to catheterization, hemoglobin and platelet count, PT, aPTT and INR values and Factor V Leiden, prothrombin 20210 A, MTHFR C667, A1298 mutations were found as not impacting. Conclusion There are no exact protocols for the prevention of thrombosis during and after catheterization. In this study, 50 unit/kg heparine bolus during catheterization and 20 unit/kg/hour heparine infusion after catheterization did not prevent the development of thrombosis. Extensive studies are needed to determine the appropriate drugs and/or doses of preventive treatments.