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Clinical and laboratory evaluation of children with congenital hyperinsulinism: A single center experience
(Walter de Gruyter GmbH, 2023) Gündoğdu, Semra; Çiftci, Mustafa; Atay, Enver; Ayaz, Akif; Ceran, Ömer; Atay, Zeynep
Objectives: To evaluate and present the data regarding clinical, laboratory, radiological and the results of molecular genetic analysis of patients with hyperinsulinemic hypoglycemia in our clinics. Methods: A total of 9 patients with CHI followed at Istanbul Medipol University. Data related to gender, age at presentation, birth weight, gestational age, consanguinity, glucose and insulin levels at diagnosis, treatment modalities, response to treatment, the results of genetic analysis and radiological evaluation were gathered from the files. Results: The oldest age at presentation was 6 months. K-ATP channel mutation was detected in 55% (n: 5). Diazoxide unresponsiveness was seen in 55% (n: 5). Octreotide was effective in 3 of them. F-18-DOPA PET performed in 4 diazoxide unresponsive patients revealed focal lesion in 3 of them. Spontaneous remission rate was 66% (n:6). All the patients with normal genetic result achieved spontaneous remission. Spontaneous remission was even noted in diazoxide unresponsive patients and in patients with focal lesion on F-18-DOPA PET. Conclusions: Clinical presentation of patients with congenital hypereinsulinism is heterogeneous. Spontaneous remission rate is quite high even in patients with severe clinical presentation. It is important to develop methods that can predict which patients will have spontaneous remission. Reporting the clinical and laboratory data of each patient is important and will help to guide the management of patients with hyperinsulinemic hypoglycemia.
Çoğul gebelikten doğan yenidoğanlarda tiroid fonksiyon testleri ve etki eden faktörler
(İstanbul Medipol Üniversitesi Tıp Fakültesi, 2019) Keleşoğlu, Emre; Atay, Enver
Tiroid hormonları organ sistemlerinin neredeyse hepsinin fonksiyonunu etkileyen, bebeklerde özellikle beyin ve somatik gelişim, erişkinde metabolik aktiviteler açısından oldukça önemli hormonlardır. Bu çalışmanın amacı, çoğul gebelikten doğan yenidoğanlarda tiroid fonksiyon testi durumu ve bunu etkileyen faktörlerin incelenmesidir. Retrospektif tipteki araştırmamızda 2017 yılında Medipol Mega Hastaneler Kompleksi ve Koşuyolu Hastanesinde doğumu gerçekleşen 349 erkek ve 340 kız olmak üzere toplam 689 bebek üzerinde yürütülmüştür. Katılımcıların maternal bilgileri (anne yaşı, gravida, parite vb.), doğum özellikleri (doğum şekli, doğum kilosu, doğum anındaki boyu, doğum haftası, gebelik sıralaması, yardımcı üreme tekniği kullanımı vb.), maternal tiroid problemleri, maternal komorbiditeler (Diyabet, HT vb.), bebeğe ait US ve EKO görüntüleme sonuçları ve tiroid fonksiyon test sonuçları hastane bilgi yönetim sistemi ve hasta dosyalarından temin edilmiştir. Doğumlardan 322'si ikiz (%95,55) 15'i üçüzdü (%4,45). Yardımcı üreme tekniği kullanma oranı %57,86'di. 34 annede (%10,27) tiroid hastalığı mevcuttu (27 hipotiroidi(gebelik öncesi) (%8,1), 3 gebelikte ortaya çıkan hipotiroidi (%0,9),2 hipertiroidi (%0,6), 2 guatr (%0,6)). 28 anne (%8,33) tiroid tedavisi almıştı. 100 bebek (%14,53) SGA ve 13 bebek (%1,89) LGA'dır. 46 annede (%13,65) diyabet vardı. Hipotiroidi tanısı alan 7 bebek (%1,01) vardı(Ortalama TSH düzeyi 69,65 mıu/l, fT4 1,04 ng/dl). Annesi hipotiroidi tedavisi alan 57 bebekten 3'ünde (%5,26) hipotiroidi vardı. Dopamin alan bebeklerde doğum haftası ve ilk Ft4 ortalaması almayanlara göre anlamlı olarak daha düşüktür(hepsi için p<0,05). Bebeklerin doğum sırasına göre, ilk serbest T4 değeri ve ilk TSH değeri karşılaştırıldığında aralarında anlamlı ilişki bulunamamıştır(her ikisi için de p>0,05). Sonuç olarak, çoklu gebeliklerde hipotiroidi oldukça sıktır. TFT üzerine etki eden prematüre doğum, maternal tiroid hastalığı ve yenidoğan döneminde dopamin kullanımı gibi pek çok faktör mevcuttur. Çoğul gebeliklerden doğan bebeklerin TFT sonuçları arasında anlamlı fark saptanmamıştır.
Congenital depressed fracture of the skull in a neonate
(2012) Atay, Enver; Tokmak, Mehmet; Can, E.; Ovalı, Hüsnü Fahri
Objective: Congenital depression of the skull is a rare event and the cause is not always clear. It may be complicated by brain injury, hematoma and epilepsy. This case is presented to draw attention to this rare congenital disorder which may raise suspicions in the family and discuss treatment options. Description: This baby boy was born at term by cesarean section and the depressed fracture of the right parietal bone, 5 cm × 4 cm, with a depth of 7.7 mm was noted at the first examination. Conventional and 3-dimentional computed tomography of the skull confirmed the diagnosis. The neurological examination was unremarkable. The depressed portion was elevated by surgery; the baby was growing well in the first month. Comments: Skull fracture is frequently assumed to have resulted from trauma, but it may occur prenatally.
Elevated 1-h post-load plasma glucose levels in normal glucose tolerance children with obesity is associated with early carotid atherosclerosis
(Elsevier, 2020) Kılınç, Suna; Demirtaş, Tuna; Atay, Enver; Ceran, Ömer; Atay, Zeynep
Context: Evidence suggests thatthe 1-h post-load plasma glucose (1-h PG) ?155 mg/dL during an oral glucose tolerance test(OGTT) predicts development oftype 2 diabetes (T2DM) and associated complications, among adults with normal glucose tolerance (NGT), but relevant data on children is scarce. Objectives: To investigate whether NGT children with obesity whose 1-h PG is ?155 mg/dL have an increased carotid intima-media thickness (IMT) and exhibit non-alcoholic fatty liver disease (NAFLD) diagnosed by ultrasonography, as compared with NGT subjects with 1-h PG <155 mg/dL and impaired glucose tolerance (IGT). Methods: Cardio-metabolic profile, OGTT, measurements of carotid IMT and liver ultrasonography were analyzed in 171 non-diabetic children with obesity. Subjects were divided into 3 groups: NGT subjects with a 1-h PG <155 mg/dL, NGT subjects with a 1-h PG ?155 mg/dL, and IGT subjects. Results: As compared with NGT individuals with a 1-h PG <155 mg/dL, NGT individuals with a 1-h PG ?155 mg/dL exhibited higher carotid IMT (0.75 ± 0.15 mm vs. 0.68 ± 0.15 mm; p < 0.05). No significant differences were observed in carotid IMT between IGT and NGT subjects with a 1-h PG ?155 mg/dL (0.75 ± 0.18 mm vs 0.75 ± 0.15 mm; p > 0.05). Of the three glycemic parameters, 1-h and 2-h PG, but not fasting glucose, were significantly correlated with carotid IMT. There were no significant differences for increased risk of having NAFLD between the three groups. Conclusions: These data suggest that a value of 1-h PG ?155 mg/dL in children and adolescents with obesity is as important as IGT with respect to cardiovascular risks.
Evaluation of the patients with congenital rickets
(Haydarpaşa Numune Training and Research Hospital, 2020) Atay, Enver
Introduction: The present study aims to evaluate the clinical findings and biochemical properties of the patients who were followed up in the neonatal intensive care unit due to congenital rickets. Methods: The data of patients who were followed up in our neonatal intensive care unit between March 2015 and March 2020 due to congenital rickets were analyzed retrospectively from the patient’s files and registration forms. The findings of physical examination and biochemical tests of the patients were recorded at the time of admission. Results: Six patients were included in this study. Four of the patients were girls. The mean age of application was 19±6 days. All patients were admitted with convulsion. The average calcium at the first application was 5.5±0.7 mg/dL; ionized calcium 0.7±0.02 mmol/L; phosphorus 6.9±1.2 mg/dL; alkaline phosphatase 747±148 U/L; parathyroid hormone 265±40 pg/mL and mean 25-OH vitamin D 3.4±0.4 ng/mL. All cases were hospitalized and treated and diagnosed with congenital rickets. Discussion and Conclusion: Despite vitamin D prophylaxis, rickets can still be seen in our country. With this study, we aimed to draw attention to the significance of vitamin D prophylaxis.
Evaluation of Vitamin D status and its correlation with gonadal function in children at mini-puberty
(Karger, 2018) Kılınç, Suna; Atay, Enver
Objective: Most recent evidence from conducted in human and animal studies suggests that vitamin D has a potential role in the physiology of reproductive function in both genders. We investigate the role of vitamin D in male and female gonadal function at mini-puberty period with particular emphasis on production of sex steroids and gonadal peptide hormones. Additionaly, this study evaluated serum levels of luteinizing hormone (LH), folliclestimulating hormone (FSH), estradiol (E2), total testosteron (TT), anti-müllerian hormone (AMH), and inhibin B in a large prospective cohort of infant at mini-puberty period.
Evaluation of vitamin D status and its correlation with gonadal function in children at mini-puberty
(Wiley, 2019) Kılınç, Suna; Atay, Enver; Ceran, Ömer; Atay, Zeynep
Context The effects of Vitamin D on reproductive function in adults have gained interest. Studies have demonstrated some associations. Hypothalamic-pituitary-gonadal axis is activated during the first 6 months of life, called as mini-puberty. This HPG activation is important for future gonadal function. There are no data regarding the association of gonadal hormones and 25(OH)D levels at mini-puberty. Demonstration of any association would form the basis for studies that will search for the effects of 25(OH)D on gonadal hormones at mini-puberty. Objective To characterize the associations between 25(OH)D levels and gonadal hormones at mini-puberty. Design Cross-sectional cohort analysis. Patient(s) or other participant(s) A total of 180 (94 boys and 86 girls) healthy appropriate-for-gestational-age neonates were included. Main outcome measure(s) 25(OH)D, LH, FSH, total testosterone, oestradiol, AMH and inhibin B levels were measured at postnatal 30-45 days. All infants were divided into three groups including vitamin D deficiency (<10 ng/mL), vitamin D insufficiency (10-20 ng/mL) and vitamin D sufficiency (>20 ng/mL). Correlations between vitamin D status and reproductive hormones were analysed. Result(s) Total testosterone level was higher (mean: 0.52 +/- 0.32 vs 0.26 +/- 0.2 ng/mL; P: 0.008) and inhibin B was lower in 25(OH)D deficient than sufficient girls (mean: 21.2 +/- 15.71 vs 53.25 +/- 47.25 pg/mL; P: 0.021). Conclusion(s) A modest effect of 25(OH)D was identified on total testosterone and inhibin B in girls at mini-puberty. The 25(OH)D may have an effect on gonadal function during early life. Randomized controlled trials could clarify the importance of vitamin D on gonadal hormones at mini-puberty.
Frequency of hematological findings associated with severe plasma vitamin B12 deficiency in infants and adolescents
(Clinical Laboratory Publication, 2014) Atay, Enver; Akın, Mehmet; Özhan, Bayram; Öztekin, Osman; Karakuş, Yasin Tuğrul; Erdoğan, Fırat
Background: The aim of this study was to determine the hematological status of severe vitamin B12 deficiency in infants and adolescents. Methods: This study involved 95 infants and 117 adolescents with severe plasma vitamin B12 deficiency (< 120 pg/mL) and normal plasma folate and ferritin. Infants were aged between one and 24 months. Adolescents were aged between 11 and 17 years. Results: Macrocytic anemia was associated with nine (9.5%) out of 95 infants with severe vitamin B12 deficiency. Neutropenia was found in 16 (16.8%) out of 95 infants with severe vitamin B12 deficiency. Thrombocytopenia was not found in 95 infants with severe vitamin B12 deficiency. Macrocytic anemia was found in two (1.7%) out of 117 adolescents with a severe vitamin B12 deficiency. Neutropenia was associated in one (0.8%) out of 117 adolescents with severe vitamin B12 deficiency. Thrombocytopenia was not found in 117 adolescents with severe vitamin B12 deficiency. Conclusions: Low clinical or hematological findings for B12 deficiency in infants and adolescents living in regions at risk, such as those with low consumption of meat and other animal products warrant the measurement of vitamin B12 level.
Immature event-related alpha dynamics in children compared with the young adults during inhibition shown by day-night stroop task
(Frontiers Media SA, 2023) Güntekin, Bahar; Alptekin, Simay; Yıldırım, Ebru; Aktürk, Tuba; Uzunlar, Hakan; Çalışoğlu, Pervin; Eroğlu Ada, Figen; Atay, Enver; Ceran, Ömer
Introduction: Inhibitory control develops gradually from infancy to childhood and improves further during adolescence as the brain matures. Related previous studies showed the indispensable role of task-related alpha power during inhibition both in children and young adults. Nonetheless, none of the studies have been able to investigate the direct differences in brain responses between children and young adults when confronted with a stimulus that should be inhibited. Because, unlike event-related designs, task-related designs involve continuous tasks over a certain period, which precludes the possibility of making such a comparison. Accordingly, by employing event-related design, the present study first time in the literature, aimed to analyze the event-related alpha phase locking and event-related alpha synchronization/ desynchronization to differentiate the inhibitory processes in children compared to young adults. Methods: Twenty children between the ages of 6 to 7 years and 20 healthy young adult subjects between the ages of 18 to 30 years were included in the study. Day-night Stroop task was applied to all subjects during 18-channel EEG recordings. Event-related time-frequency analysis was performed with the complex Morlet Wavelet Transform for the alpha frequency band (8–13 Hz). Event related spectral perturbation (ERSP) in three different time windows (0–200 ms, 200–400 ms, 400–600 ms) and Event-related phase locking in the early time window (0–400 ms) was calculated. Results: The children had increased alpha power in early and late time windows but decreased alpha phase locking in the early time windows compared to young adults. There were also topological differences between groups; while young adults had increased alpha phase-locking in frontal and parietal electrode sites, children had increased occipital alpha power and phase locking. Discussion: The shift in event-related alpha power observed from posterior to anterior regions with age may suggest a progressive maturation of the frontal areas involved in inhibitory processes from childhood to adulthood. The results of the present study showed that children and young adults had different EEG oscillatory dynamics during inhibitory processes at alpha frequency range.
Responsiveness to parenteral iron therapy in children with oral iron-refractory iron-deficiency anemia
(Taylor & Francis, 2014) Akın, Mehmet; Atay, Enver; Öztekin, Osman; Karadeniz, Cem; Karakus, Yasin Tuğrul; Yılmaz, Bilal; Erdoğan, Fırat
Intravenous (IV) ferric iron (Fe)-carbohydrate complexes are used for treating Fe deficiency in children with iron-refractory iron-deficiency anemia (IRIDA). An optimal treatment has yet to be determined. There are relatively little publications on the responsiveness to IV iron therapy in children with IRIDA. Patients and Method: This study analyzed responses to IV iron sucrose therapy given to 11 children, ranging in age from 2 to 13 years (mean 4.8 years), with iron-deficiency anemia who were unresponsive to oral iron therapy. Results: The hemoglobin and ferritin values (mean) of the 11 children with IRIDA were 7.7 g/dL and 4.8 ng/mL at diagnosis. Both hemoglobin and ferritin levels increased to 9.5 g/dL, and 24 ng/mL, respectively, at 6 weeks after the first therapy. Although the level of hemoglobin was steady at 6 months after the first, and 6 weeks after the second therapy, the ferritin levels continued to increase up to 30 ng/mL and 47 ng/mL at 6 months after the first and 6 weeks after the second therapy, respectively. Conclusion: We recommend that IRIDA should be considered in patients presenting with iron-deficiency anemia of unknown cause that is unresponsive to oral iron therapy. Our results suggest that IV iron therapy should be administered only once in cases of IRIDA. Continued administration of IV iron would be of no benefit to increase hemoglobin levels. On the contrary, ferritin levels may continue to increase resulting in untoward effects of hyperferritinemia.
The effects of anti-HBs antibodies passed through transplacental route on immunization induced by HBV vaccine and natural course of passively transmitted HBs antibodies
(Haydarpaşa Numune Training and Research Hospital, 2020) Atay, Enver; Say, Aysu
Introduction: Hepatitis B infection is an important infection that concerns public health. In this study, we aimed to evaluate whether anti-Hbs antibodies born from mothers who are immune to hepatitis B virus with natural or vaccination and passed from mother to baby transplacentally affect the natural course of these passive anti-HBs antibody titers. Methods: In this study, 68 healthy term newborn babies were included. The immune status of the mothers of these newborn babies was evaluated. Babies were divided into three groups. Group 1, babies whose mothers were positive for AntiHBs antibody, anti-HBS titers were evaluated consecutively at 0, 1, 3, 6 and 8 months without HBV vaccine. In Group 2, babies whose mothers were positive for anti-HBs antibody were vaccinated with HBV vaccine at 0.1 and 6 months, and these babies were examined for anti-HBS titers at 0.1, 3, 6 and 8 months. In Group 3, babies whose mothers were negative of anti-HBs antibody were vaccinated with HBV vaccine at 0, 1 and 6 months, and babies were examined at 0.1, 3, 6 and 8 months. Results: Group 1 consisted of 22 babies, Group 2 consisted of 24 babies and Group 3 consisted of 22 babies. Percentages of anti-HBs titers falling below <10 mIU/mL at 1, 3, 6 and 8 months were found to be 40.9%, 50%, 59.09%, and 100% of nonvaccinated babies whose mothers were positive for anti-HBs antibody. Discussion and Conclusion: We found that the antibody response to HBV vaccine administered to infants with passive antibodies was similar to the antibody response in mothers vaccinated with their mothers HBsAg (-), anti-HBs (-), anti-HBcIgM (-) and the same vaccine calendar. Thus, it was found that anti-HBs antibodies that started this transplacentalntal pathway did not affect the antibody response produced by the HBV vaccine administered at 0, 1 and 6 months
The role of makorin ring finger protein-3, kisspeptin, and neurokinin B in the physiology of minipuberty
(Walter de Gruyter GmbH, 2021) Atay, Enver; Kılınç, Suna; Ülfer, Gözde; Yiğitbaşı, Türkan; Çakıcı, Çağrı; Turan, Murat; Ceran, Ömer; Atay, Zeynep
Background: There is no data regarding the interrelationships of circulating Makorin Ring Finger Protein-3 (MKRN3), Kisspeptin (KISS1), and Neurokinin B (NKB) concentrations during minipuberty in humans.Objective: To determine temporal changes in circulating concentrations of MKRN3, KISS1, NKB, and gonadotropins and investigate interrelationships between them in healthy full-term (FT) and preterm (PT) infants during minipuberty period.Methods: A prospective study of 6-month follow-up performed. Eighty-seven healthy newborns, 48 FT (19 boys/29 girls), and 39 PT (21 boys/18 girls) (gestational age 31-37 weeks), were included. Blood samples were taken at 7 days (D7), 2 months (M2), and 6 months (M6) of age. Serum MKRN3, KISS1, NKB, LH, FSH, total testosterone (TT), and estradiol (E2) concentrations were measured.Results: Seventy infants completed the study. MKRN3, KISS1, and NKB concentrations were similar in FT girls and boys. PT boys and girls also had similar concentrations of MKRN3, KISS1, and NKB. FT babies had significantly higher NKB concentrations than PT babies at D7, M2, and M6. MKRN3 and KISS1 concentrations do not differ between FT and PT babies. A strong positive correlation was found between MKRN3 and KISS1 at each time point and in all groups. FSH, LH, TT/E2 concentrations decrease while those of MKRN3 and KISS1 have a trend to increase toward the end of minipuberty. No correlation was detected between gonadotropins and MKRN3, KISS1, NKB concentrations.Conclusion: Strong positive correlation demonstrated between KISS1 and MKRN3 suggests that interrelationship between molecules controlling minipuberty is not similar to those at puberty.
Theta and alpha oscillatory responses differentiate between six-to seven-year-old children and adults during successful visual and auditory memory encoding
(Elsevier, 2020) Güntekin, Bahar; Uzunlar, Hakan; Çalışoğlu, Pervin; Eroğlu-Ada, Figen; Yıldırım, Ebru; Aktürk, Tuba; Atay, Enver; Ceran, Ömer
The healthy maturation of the brain is one of the intriguing topics that need to be investigated to understand human brain and child development. The present study aimed to investigate the development of memory processes both for auditory and visual memory using electroencephalography (EEG)-Brain Dynamics methodologies.Sixteen healthy children between the ages of 6 and 7 years and eighteen healthy young adults (age: 21.32 +/- 3.28 years) were included in the study. EEG was recorded from 18 channels during the visual and auditory memory paradigms. Two different subtests of the WISC-IV IQ test were applied to all children. Eventrelated theta (4-7 Hz), alpha (8-13 Hz) power and phase-locking were analyzed.The young adults had higher memory performance than the children for both auditory and visual paradigms. The children had increased theta phase-locking and left alpha power in response to the remembered objects in comparison to the forgotten objects. The young adults had higher theta and alpha phase-locking than the children over the frontal and central locations (p < 0.05), and the children had higher parietal-occipital alpha phase-locking than the young adults. There was an increase in alpha power in children, whereas young adults had decreased post-stimulus alpha power in response to memory paradigms.The present study showed that frontocentral theta and alpha phase-locking had an essential role in brain maturation and successful memory performance. Event-related theta and alpha responses could be one of the important indicators of the mature and healthy brain, and these responses could change depending on the maturation state and age.
Thyroid function tests and affecting factors in twins and triplets
(Karger, 2018) Keleşoğlu, Emre; Atay, Zeynep; Abalı, Saygın; Atay, Enver; Turan, Murat; Gündoğdu, Semra; Ceran, Ömer
[Abstract Not Available]
Transient gonadal activation and infant growth velocity
(Karger, 2019) Kılınç, Suna; Hamikçıkan, Şahin; Atay, Enver; Atay, Zeynep
Background: Hypothalamic-pituitary-gonadal axis is activated during the first 6 months of life, called as mini-puberty in which reproductive hormone levels may reach to adult levels. Although, our understanding of the pathophysiology of sex steroids interaction with growth in puberty is increasing, very little is known about the relationship between sex steroids and growth at this period of life. Material and Methods: 142 (67 girls, 75 boys) healthy appropriate-for-gestational age neonates were included. Insulin-like growth factor 1(IGF-1), insulin-like growth factor 3 (IGFBP-3), luteinizing hormone (LH), follicle stimulating hormone (FSH), total testosterone (TT) and estradiol (E2) levels were measured at postnatal 2nd month of life. In all cases height and weight were measured at the 1st, 2nd, 4th, 6th, 9th and 12th months of age. GV was monitored and compared with sex steroids and growth factors. Results: The mean LH and TT levels were significantly higher in boys than girls (P = 0.001). In girls, the mean FSH level was significantly higher than in boys (P = 0.001). There was no statistically significant difference between the mean E2, IGF-1 and IGFBP-3 levels in boys and girls (p> 0.05). The GV was significantly faster from birth to 6 months of age in boys than in girls (P <0.05). The highest GV was observed at 1 and 2 months of age, simultaneously with the peak of postnatal gonadal activation. There was a positive correlation between GV and TT in both sexes (P <0.05). Conclusion: These results may provide a new perspective on the effect of transient gonadal activation on infant growth velocity.

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