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Item Incidence of Type 1 Diabetes in Children Aged Below 18 Years during 2013-2015 in Northwest Turkey(GALENOS YAYINCILIK, 2018-01-01) Poyrazoglu, Sukran; Bundak, Ruveyde; Abali, Zehra Yavas; Onal, Hasan; Sarikaya, Sevil; Akgun, Abdurrahman; Bas, Serpil; Abali, Saygin; Bereket, Abdullah; Eren, Erdal; Tarim, Omer; Guven, Ayla; Yildiz, Metin; Aksakal, Derya Karaman; Yuksel, Aysegul; Karabulut, Gulcan Seymen; Hatun, Sukru; Ozgen, Tolga; Cesur, Yasar; Azizoglu, Mehmet; Dilek, Emine; Tutunculer, Filiz; Cakir, Esra Papatya; Ozcabi, Bahar; Evliyaoglu, Olcay; Karadeniz, Songul; Dursun, Fatma; Bolu, Semih; Arslanoglu, Ilknur; Mutlu, Gul Yesiltepe; Kirmizibekmez, Heves; Isguven, Pinar; Ustyol, Ala; Adal, Erdal; Ucar, Ahmet; Cebeci, Nurcan; Bezen, Didem; Binay, Cigdem; Semiz, Serap; Korkmaz, Huseyin Anil; Memioglu, Nihal; Sagsak, Elif; Peltek, Havva Nur; Yildiz, Melek; Akcay, Teoman; Turan, Serap; Guran, Tulay; Atay, Zeynep; Akcan, Nese; Cizmecioglu, Filiz; Ercan, Oya; Dagdeviren, Aydilek; Bas, Firdevs; Issever, Halim; Darendeliler, FeyzaObjective: To assess the incidence of type I diabetes mellitus (T1DM) in children under 18 years of age in the northwest region of Turkey during 2013-2015. Methods: All newly diagnosed T1DM cases were recorded prospectively during 2013-2015. Total, as well as gender and age group specific (0-4, 5-9. 10-14 and 15-17 age) mean incidences per 100,000 per year were calculated. Results: There were 1,773 patients diagnosed during 2013-2015 (588 cases in 2013, 592 cases in 2014, 593 cases in 2015). Of these, 862 (48.6 \%) were girls and 911 (51.4\%)were boys. The mean age at diagnosis was 9.2 +/- 4.2 years and it was not significantly different between girls (9.0 +/- 4.1 years) and boys (9.4 +/- 4.4 years) (p = 0.052). The crude mean incidence was 8.99/100.000 confidence interval (CI) (95\% CI: 8.58-9.42). Although mean incidence was similar between boys {[}8.98/100.000 (CI: 8.40 to 9.58)] and girls {[}9.01/100.000 (CI: 8.42 to 9.63)], there was male predominance in all groups except for 5-9 year age group. The standardized mean incidence was 9.02/100.000 according to the World Health Organization standard population. The mean incidence for the 0-4, 5-9, 10-14 and 15-17 age groups was 6.13, 11.68, 11.7 and 5.04/1 00.000 respectively. The incidence of T1DM was similar over the course of three years (p = 0.95). A significant increase in the proportion of cases diagnosed was observed in the autumn-winter seasons. Conclusion: The northwest region of Turkey experienced an intermediate incidence of T1DM over the period of the study.Item Cranial MRI Abnormalities and Long-term Follow-up of the Lesions in 770 Girls With Central Precocious Puberty(ENDOCRINE SOC, 2021-01-01) Helvacioglu, Didem; Turan, Serap Demircioglu; Guran, Tulay; Atay, Zeynep; Dagcinar, Adnan; Bezen, Digdem; Ozturan, Esin Karakilic; Darendeliler, Feyza; Yuksel, Aysegul; Dursun, Fatma; Kilinc, Suna; Semiz, Serap; Abali, Saygin; Yildiz, Metin; Onder, Asan; Bereket, AbdullahContext: Central precocious puberty (CPP) may arise from central nervous system (CNS) lesions in a few affected girls. Recently, the incidence of girls with CPP has increased mostly in 6-8 year olds, in whom the necessity of magnetic resonance imaging (MRI) is debated. Objective: To investigate the frequency, long-term outcome and potential predictors of CNS lesions in a large cohort of girls with CPP. Methods: A multicenter cohort of 770 Turkish girls with CPP who had systematic cranial MRI between 2005 and 2017. Age at puberty onset was <6 years in 116 and 6-8 years in 654. CNS lesions were followed until final decision(6.2 +/- 3.1 years). Potential predictors of CNS lesions were evaluated by univariate analyses. Results: A total of 104/770 (13.5\%) girls had abnormal brain MRI. Of these, 2.8\% were previously known CNS lesions, 3.8\% had newly detected and causally related CNS lesions, 3.1 \% were possibly, related and 3.8\% were incidental. Only 2 (0.25\%) neoplastic lesions (1 low grade glioma and 1 meningioma) were identifiedItem Seizures and diagnostic difficulties in hyperinsulinism-hyperammonemia syndrome(TURKISH J PEDIATRICS, 2016-01-01) Aka, Sibel; Alanay, Yasemin; Boodhansingh, Kara E.; Stanley, Charles A.; Semiz, SerapHyperinsulinism/hyperammonemia (HI/HA) syndrome is a rare disorder presented with recurrent hypoglycemia and elevated serum ammonia, which may lead to development delays, permanent neurologic damages, if it remains underdiagnosed. It is caused by activating mutations in the GLUD1 gene which encodes the intra-mitochondrial enzyme glutamate dehydrogenase (GDH). HI/HA syndrome is considered the second most common form of hyperinsulinism (HI), and usually associated with epileptic seizures, mental retardation and generalized dystonia. We reported a patient who was diagnosed as HI/HA with multiple episodes of seizuresItem Neonatal Problems and Infancy Growth of Term SGA Infants: Does ``SGA'' Definition Need to Be Re-evaluated?(FRONTIERS MEDIA SA, 2021-01-01) Abali, Saygin; Beken, Serdar; Albayrak, Eda; Inamlik, Aysegul; Bulum, Burcu; Bulbul, Ezgi; Eksi, Gulten Zeynep; Ay, Zeynep Alize; Karabay, Melis; Kaya, Didem; Halici, Muge; Semiz, Serap; Korkmaz, AyseIntroduction: The exact definition of small-for-gestational-age (SGA) infant is still controversial among clinicians. In this study, we aimed to understand which definition is better in terms of establishing both early postnatal problems and growth. In this way, we compared early neonatal problems and infancy growth of term infants with birth weight (BW) < -2 SDS and with BW between 10th percentile (-1.28 SDS) and -2 SDS.Methods: A single center retrospective cohort study was conducted. Preterm infants, multiple gestations and newborns with any congenital anomalies were excluded from the study. Study group was defined as Group 1 (n = 37), infants BW < -2.00 SDSItem An Uncommon Cause of Hypernatremia in Very Low Birth Weight Premature Infants: Idiopathic Central Diabetes Insipidus(AVES, 2021-01-01) Aktas, Selma; Kazanci, Ebru; Semiz, Serap; Korkmaz, AyseCentral diabetes insipidus (CDI) is a water homeostasis disorder characterized by an inability to concentrate urine because of insufficient production of antidiuretic hormone. Dehydration with hypernatremia can occur during the neonatal period in preterm neonates in association with insensible water loss, high urine output, and reduced sodium excretion. A high index of suspicion is required to diagnose CDI in preterm neonates. We report two cases, who presented persistent hypernatremia with polyuria despite increased fluid supply and low sodium intake. CDI diagnosis was confirmed by the therapeutic test with oral vasopressin analog. Investigations were all normal