Search

Objectives The aim of this study is to determine the plasma free carnitine and acyl-carnitine levels at the time of diabetic ketoacidosis (DKA) diagnosis, and at the end of DKA treatment and to investigate their association with the duration of DKA treatment in children with DKA. Methods A total of 40 children with DKA who were treated consecutively in a tertiary health center for DKA were included in the study. The median age of the children was 11.3 years (1.1–17.5) and 25 of them (62.5%) were girls. In addition to routine blood tests, plasma free carnitine and acyl-carnitine levels were measured just before the start of intravenous insulin therapy and at the time of discontinuation of intravenous insulin therapy when DKA therapy was completed. Results There was no difference in plasma free carnitine and acyl-carnitine levels before and after DKA treatment (p=0.776 and p=0.743 respectively). However, while the frequency of low plasma free carnitine was 30% at the beginning of the treatment, it was observed that this frequency was 20% at the end of the DKA treatment. There was no correlation between duration of DKA treatment and plasma free carnitine or acyl-carnitine levels at admission (p=0.497, r=−0.111 and p=0.474, r=0.116 respectively). Conclusions There is no a relationship between duration of DKA treatment and plasma free carnitine or acyl-carnitine level at admission in children with DKA.

Objective: In this study, we aimed to investigate the clinical features of children who received growth hormone (GH) treatment with a diagnosis of GH deficiency (GHD). Material and Methods: The clinical features of children treated with GH treatment between 01.06.2013-31.12.2018 in a tertiary healthcare service were retrospectively analyzed. Children with isolated GHD and children with panhipopituitarism were compared. Results: In total, 70 boys 118 children were included in the study. 78 (66.3%) of patients were treated with GH due to isoleted GHD, 14 (11.9%) due to bio-inactive GH syndrome, 9 (7.6%) due to panhypopituitarism, 6 (5.1%) due to GH neurosecretory dysfunction, 6 (5.1%) due to Turner syndrome and 5 (4.2%) due to off-label reasons.There were no significant diffrence between groups of isoleted GHD and panhipopituitarism regarding gender, age of diagnosis and oxological features at diagnosis. Serum IGF-1 SDS, IGFBP-3 SDS, TSH and pik GH response to klonidine test were lower in panhipopituitarism group (p=0.026, p=0.002, p=0.009 ve p=0.005, respectively). Significantly higher growth rates in the first 2 years in the patient with panhypopituitarism were determined (p

Aim: Autoimmune polyendocrinopathy syndrome type 3 (APS3) is defined as the presence of autoimmune thyroid disease along with another autoimmune disorder other than Addison’s disease. If an autoimmune gastrointestinal disease is a component of the syndrome, it is defined as APS3b. In this study, we aimed to determine the frequency of APS3b in patients diagnosed with type 1 diabetes (T1D) in childhood. Material and Methods: A patient group of 446 children (232 males) with T1D whose follow-ups were conducted between 01.06.2013 and 31.08.2019 was included in the study. Hashimoto’s thyroiditis (HT) was defined with positive anti-TPO and/or anti-Tg test results whereas autoimmune enteropathy was defined with histologic findings which consisted with celiac disease (CD) in small bowel biopsy following a positive anti tissue transglutaminase IgA. Results: In total, 61 (13.7%) patients had HT and 34 (7.6%) patients had CD. While 87 (19.5%) of the patients had HT or CD, the occurrence of both HT and CD (APS3b) in the same patient was observed in only 4 patients (3 females) (0.9%). Patients with APS3b had low median age of diagnosis compared with patients with HT or CD alone, and patients with none of these two diseases, but the difference is not statistically significant (p=0.087). Conclusion: While HT or CD has been detected in approximately one-fifth of children with T1D, APS3b is rarely observed in this patient group.

Corona virus disease 2019 (COVID-19) includes a wide range of diseases with varying pathophysiology in children and adults. Although the disease mainly affects the respiratory tract, neurological involvement is also reported in the literature. The most common neurological complaints due to COVID-19 are headache, dizziness and anosmia. Acute necrotizing myelitis, acute demyelinating encephalomyelitis (ADEM), acute axonal neuropathy, acute transverse myelitis, and Guillian-Barre syndrome have been reported as neurological dysfunctions associated with COVID-19.A ten-year-old male patient presented with complaints of fever, headache and generalized muscle pain. The patient developed inability to walk and significant muscle weakness during the disease course, and he was diagnosed with ADEM and transverse myelitis on magnetic resonance imaging (MRI). As the etiological agent, COVID-19 was detected in both the respiratory panel sample and the cerebrospinal fluid (CSF) sample by the polymerase chain reaction (PCR) technique. Pulse steroid, IVIG, and plasmapheresis treatment were administered. He started to stand with support during follow-up.We presented a case of COVID-19 related ADEM and transverse myelitis who responded to pulse steroid, IVIG, and plasmapheresis.

Introduction: Ultrasonography and fine needle aspiration biopsy are reliable diagnostic methods used to predict malignancy in thyroid nodules. They are useful in identifying patients for therapy or preventing unnecessary surgery. Materials and Methods: The aim of this study was to compare clinical and ultrasonographic findings with fine needle aspiration biopsy and histopathological examination results in children with thyroid nodules. Retrospective study. This multicentre study included pediatric patients with thyroid nodules, followed up between July 2002 and November 2019 in nine Turkish Pediatric Endocrinology Units. Clinical, ultrasonographic, cytological and histopathological findings (malignant or benign) were analyzed retrospectively. Fine needle aspiration biopsy findings were classified according to American Thyroid Association criteria. Results: A total of 203 children were included, of whom 82.3% (n=167) were female. Mean±standard deviation age was 14.06±2.26 (range 3.7-19) years. Cytological diagnoses were: non–diagnostic or unsatisfactory 1.9%; benign 59.6%; atypia or follicular lesion of undetermined significance, suspicious for follicular neoplasm in 2.4%; suspicious for malignancy 12.8%; and malignant in 1.9%. Surgery was performed in 59 (29.1%) patients. In total 33 (16.3%) patients were diagnosed with malignancy. In patients with benign fine needle aspiration biopsy results, malignancy was detected in 17.6% when they underwent surgery. Malignancy was present in 44.4% of the patients with cytologically determined atypia or follicular lesion of undetermined significance. The diagnostic accuracy of fine needle aspiration biopsy was 79%. Conclusion: This study is first multicentre study investigating thyroid nodules in children and adolescents in Turkey. In this population fine needle aspiration biopsy has an important role in diagnosis, but ultrasonographic features of nodule should also be taken into consideration. Atypia or follicular lesion of undetermined significance may be of greater clinical significance than has previously been reported. Giriş: Ultrasonografi ve ince iğne aspirasyon biyopsisi, tiroid nodüllerinde maligniteyi öngörmede kullanılan güvenilir tanı yöntemleridir. Doğru hastayı bulmada ve gereksiz ameliyatları önlemede faydalıdırlar. Bu çalışmanın amacı tiroid nodülü bulunan çocuklarda ince iğne aspirasyon biyopsisi ile klinik, ultrasonografik bulgular ve histopatolojik inceleme sonuçlarını karşılaştırmaktır. Gereç ve Yöntem: Bu çok merkezli çalışmada, Türkiye’den 9 farklı Pediatrik Endokrinoloji Ünitesinde Temmuz 2002-Kasım 2019 tarihleri arasında takip edilen tiroid nodüllü hastaların verileri incelendi. Tiroid nodüllü çocuk hastaların klinik, ultrasonografik, sitolojik ve histopatolojik bulguları (malign veya benign) retrospektif olarak incelendi. İnce iğne aspirasyon biyopsisi ile takip edilen hastaların bulguları Amerikan Tiroid Birliği’ne göre sınıflandırıldı. Bulgular: Çalışmaya toplam 203 çocuk dahil edildi, hastaların %82,3’ü (n=167) kadındı. Hastaların ortalama yaşı 14,06±2,26 yıl (dağılım 3,7-19 yıl) idi. Hastaların sitolojik tanıları şu şekildeydi: %1,9’u tanı dışı veya yetersiz, %59,6’sı benign, önemi belirsiz atipi veya foliküler lezyon, %2,4’ü foliküler neoplazm şüpheli, %12,8’i malignite şüphesi, %1,9’u malign idi. 59 hastaya cerrahi uygulandı. Otuz üç hastaya malignite tanısı konuldu. Benign ince iğne aspirasyon biyopsisi sonucu olan hastaların %17,6’sında ameliyat olduklarında malignite saptandı. Önemi belirlenemeyen sitolojiye sahip atipi veya foliküler lezyonu olan hastaların %44,4’ünde malignite mevcuttu. İnce iğne aspirasyon biyopsisinin tanısal doğruluğu %79 idi. Sonuç: Bu çalışma, Türkiye’de çocuk ve ergenlerde tiroid nodüllerini araştıran ilk çok merkezli çalışmadır. Tiroid nodülü olan çocuklarda ince iğne aspirasyon biyopsisi tanıda önemli bir yere sahiptir ancak nodülün ultrasonografik özellikleri de dikkate alınmalıdır. Atipi veya önemi belirsiz foliküler lezyon bilinenden daha önemli olabilir.

BACKGROUND The present study investigated the relationship between detection of organic pathologies with magnetic resonance imaging of the pituitary gland, clinical and laboratory findings, and treatment response. MATERIAL AND METHODS The study included a total of 183 patients who had isolated growth hormone deficiency, received at least 1 year of treatment, returned regularly for follow-ups, and whose pituitary magnetic resonance images were available. The patients were divided into 2 groups: those with and without pathological evidence with magnetic resonance imaging. Clinical and laboratory features and treatment responses were compared between patients with and without pathological evidence with magnetic resonance imaging. RESULTS Of the 183 patients, 105 were females and 78 were males, and 114 patients (62.2%) were prepubertal and 69 patients (37.8%) were pubertal. Their mean age was 10.01±3.25 years (1-17.6 years). Pituitary images of 153 (83.6%) patients were normal. Of the patients with detected pathologies (16.4%), 19 (10,4%) had pituitary hypoplasia, 5 (2.7%) had partial empty sella, 3 (1.7%) had ectopic neurohypophysis and 3 (1.7%) had empty sella, pineal, and arachnoid cyst. A statistically significant increase was observed in the height increase rate after treatment compared to before treatment in both groups (p

BACKGROUND This study aimed to determine the frequency and duration of remission in children and adolescents newly diagnosed with type 1 diabetes and to investigate factors associated with these parameters. MATERIAL AND METHODS Fifty patients newly diagnosed with T1DM were followed for 1 year. Daily insulin requirement of less than 0.5 U/kg/day dose when the HbA1c value is less than 8% was regarded as partial remission. Patients were grouped according to their remission duration. Clinical and laboratory characteristics of the remission groups and non-remission groups were compared to find factors influencing remission and to investigate their contribution to the duration of remission. RESULTS Remission was observed in 24 (48%) out of 50 patients included in the study. Remission frequency was found to be associated with age, sex, and puberty. Longer duration of remission was more frequent in the younger age group, in pre-pubertal stage, and in male patients. Daily insulin dose and basal insulin requirement of those who went into remission was found to be significantly lower than in the other patients at discharge. CONCLUSIONS Decreased daily total and basal insulin requirement at discharge are valuable in predicting remission. The remission process in type 1 diabetes still has many characteristics that need to be clarified. Therefore, more extensive studies are needed.

OBJECTIVE To assess the incidence of T1D in children aged

Çalışmada 24 yazar bulunmaktadır. Bu yazarlardan sadece Bursa Uludağ Üniversitesi mensuplarının girişleri yapılmıştır. Objective: Hypophosphatemic rickets (HR) is a rare renal phosphate-wasting disorder, which is usually X-linked and is commonly caused by PHEX mutations. The treatment and follow-up of HR is challenging due to imperfect treatment options. Methods: Here we present nationwide initial and follow-up data on HR. Results: From 24 centers, 166 patients were included in the study. Genetic analysis (n = 75) showed PHEX mutation in 80% of patients. The mean follow-up period was 6.7 +/- 2.4 years. During the first 3-years of treatment (n = 91), mild increase in phosphate, decrease in alkaline phosphatase and elevation in parathyroid hormone (PTH) levels were detected. The height standard deviation scores were -2.38, -2.77, -2.72, -2.47 at initial, 1st, 2nd and 3rd year of treatment, respectively (p > 0.05). On follow-up 36% of the patients showed complete or significant improvement in leg deformities and these patients had similar phosphate levels at presentation with better levels in 1st and 2nd years of treatment; even the treatment doses of phosphate were similar. Furthermore, 27 patients developed nephrocalcinosis (NC), the patients showed no difference in biochemical differences at presentation and follow-up, but 3rd year Pill was higher. However, higher treatment doses of phosphate and calcitriol were Found in the NC group. Conclusion: HR treatment and follow-up is challenging and our results showed higher treatment doses were associated with NC without any change in serum phosphate levels, suggesting that giving higher doses led to increased phosphaturia, probably through stimulation of fibroblast growth factor 23. However, higher calcitriol doses could improve bone deformities. Safer and more efficacious therapies are needed.

Background: Idiopathic intracranial hypertension (IIH) is one of the most important and rare complications of obesity. Prevalence of IIH in childhood obesity is not known. The purpose of this study was to determine the prevalence of IIH in obese children and adolescents and to investigate the relevant factors. Methods: In this study, 1058 obese children and adolescents between 2 and 18 years of age were included. Funduscopic examination was conducted for all subjects. In cases with papilledema, increase in intracranial pressure was clarified by measuring CSF pressure with a lumbar puncture. Other causes of IIH were ruled out with clinical, laboratory tests and imaging techniques. Results: Idiopathic intracranial hypertension was diagnosed in 14 subjects (1.32%). Rates of headache and systemic hypertension in subjects with IIH was determined to be 78.6% and 28.6%, respectively. Morning cortisol, fasting insulin and HOMA-IR values were found to be significantly higher in this cases (p Conclusions: In childhood obesity, IIH should be kept in mind as a serious complication. Funduscopic examination is an easy method that suggests IIH. In particular, obese children with systemic hypertension and refractory headache should be considered for IIH.

Objective: Clinicians should show an awareness on the menstrual characteristics of adolescent girls which may differ from adults in some aspects. To define menstrual cycle features among high school girls residing in a city center in southeastern Turkey. Methods: A cross-sectional survey was conducted on 1256 girls attending a high school located in the city center of Elazig, Turkey. Data from 879 girls (median age, 16.2 years; range, 13.6-19.2 years) who agreed to participate in the study and had started to menstruate were evaluated. Results: Mean age at menarche was 12.7±1.3 years (range, 8.2-17.3 years). The mean cycle duration was 28.7±4.4 days, and the mean menstrual flow lasted 5.9±1.3 days. Severe, moderate, and mild dysmenorrhea was reported in 29%, 43%, and 28% of the girls, respectively, and 52% used analgesics for dysmenorrhea. A total of 34% of the girls defined their menstrual cycle as irregular, and 32% reported school absenteeism due to menstruation-associated complaints (pain and/or heavy bleeding). Menstrual bleeding affected attendance to classes and other school activities, daily work, social, family, and friend relationships, as well as sports/exercise activities in 43%, 49%, 58%, 48%, 44%, and 60% of the participants, respectively. In total, 30% of the responders had a problem with menstruation, and 12% and 17% of these stated that they consulted a primary care physician or specialist, respectively. Conclusion: Dysmenorrhea was found to be common in adolescent Turkish girls and to affect daily life in approximately half of the girls.

Salgilanan haberci molekuller olan sitokinler hedeflerindeki hucreleri belirli membran reseptorlerini baglayarak etkiler. Pek cok hucre islevi sitokin reseptoru aracili hucre ici sinyal mekanizmalarinin uyeleri tarafindan duzenlenir. Sitokin reseptorlerinin siniflandirmasi hucre disi sitokin baglayici alanlar ve ortak hucre ici sinyal mekanizmalarinin yapisal homolojilerine dayanir. Sitokin reseptorlerinin temel bir siniflandirmasi tip 1 sitokin reseptorlerini, tip 2 sitokin reseptorlerini, tumor nekroz faktor reseptoru ailesini, interlokin 1 reseptoru ailesini ve yedi transmembran G proteinkenetli reseptorleri icerir. Bu yazida tip 1 sitokin reseptorlerinin yapisina ve iliskili sinyal yollarina odaklanildi. Anahtar sozcukler: Sitokinler; reseptorler; tip 1 sitokin reseptorleri. ABSTRACT

Background: Although the relationship between celiac disease (CD) and type 1 diabetes mellitus (DM) is well recognized, there are no studies of this association in Southeast Region of our country. The aim of this study was to identify the prevalence of CD in a group of children with type 1 DM undergoing treatment in the Pediatric Endocrinology Department of our hospital. Methods: The patients followed up with diagnosis of type 1 DM in the Outpatient Clinics of Pediatric Endocrinology in our hospital were enrolled in the study. Anti-tissue transglutaminase (anti-tTG) IgA was measured in blood collected from these patients. Patients with anti-tTG IgA positive then had duodenal biopsy. Biopsy samples were obtained through an endoscopy procedure. Biopsy specimens were evaluated and classified according to Marsh classification. Results: The study participants consisted of 218 patients (girls, n = 117: 53.7%; boys, n = 101: 46.3%) with type 1 DM with a mean age of 12.9 ± 4.5 years (range 2 - 18 years). Sixteen patients had anti-tTG IgA positive. All of the cases with positive serology underwent duodenal biopsy. On histopathological evaluation, 11 patients had mucosal alterations compatible with celiac disease; five patients had non-specific histologic changes or normal biopsies. The prevalence of CD was 5% among 218 patients. Five cases with normal biopsy results were evaluated as having latent CD. Conclusions: These findings indicate that the prevalence of CD in children with type 1 DM was higher than that in general population. Periodic screening of diabetic children and adolescents in terms of CD is indicated to ensure early diagnosis and treatment. Int J Clin Pediatr. 2016;5(2):32-35 doi: http://dx.doi.org/10.14740/ijcp239w

Aim The negative effect of antiepileptic drugs on bone health has been previously documented. However, which antiepileptic drug is safer in regard to bone health is still questionable. Our aims were to investigate the bone mineral density alterations in pediatric patients who receive antiepileptic medication for a minimum of two years and to compare the results of these drugs. Materials and methods Fifty-nine patients (32 males, 27 females; mean age: 8.6 ± 4.6 years) and a control group (13 males, 7 females; mean age: 7.6 ± 3.3 years) were included in the study. The patients were receiving necessarily the same antiepileptic drugs (AEDs) for at least two years, and none of the patients had mental retardation or cerebral palsy. The patients were divided into three groups: group 1 (patients receiving levetiracetam (LEV), n = 20), group 2 (patients receiving carbamazepine (CBZ), n = 11), and group 3 (patients receiving valproic acid (VPA), n = 28). Plasma calcium (Ca), phosphorus (P), parathyroid hormone (PTH), alkaline phosphatase (ALP), vitamin D levels, and bone mineral density (BMD) values of femur and vertebras (L1-4) and z-scores (comparative results of BMD values of the patients with the age- and gender-matched controls in device database) of the groups were compared. Results The differences between P, PTH, ALP and age, Ca and BMD results, and vitamin D levels of the patients in all four groups was not statistically significant according to Kruskal–Wallis test (p > 0.05). The z-score levels of all the patient and control groups were also not statistically significantly different compared with each other. Conclusion In contrast to previous reports in pediatric patients, our study has documented that there is not a considerable bone loss in patients receiving long-term AED medication. Although levetiracetam has been proposed as bone-protecting medication, we did not observe any difference between AEDs regarding bone mineral density after two years of treatment.

WOS: 000351307200006, PubMed ID: 25800474, Objective: Approaches to diagnosis and treatment of growth hormone deficiency (GHD) in children vary among countries and even among centers in the same country. This survey, aiming to facilitate the process of preparing the new consensus on GHD by the Turkish Pediatric Endocrinology and Diabetes Society, was designed to evaluate the current practices in diagnosis and treatment of GHD in different centers in Turkey. Methods: A questionnaire covering relevant items for diagnosis and treatment of GHD was sent out to all pediatric endocrinology centers. Results: Twenty-four centers returned the questionnaire. The most frequently used GH stimulation test was L-dopa, followed by clonidine. Eighteen centers used a GH cut-off value of 10 ng/mL for the diagnosis of GHD; this value was 7 ng/mL in 4 centers and 5 ng/mL in 2 centers. The most frequently used assay was immunochemiluminescence for determination of GH, insulin-like growth factor-1 and insulin-like growth factor binding protein-3 concentrations. Sex steroid priming in both sexes was used by 19 centers. The most frequently used starting dose of recombinant human GH (rhGH) in prepubertal children was 0.025-0.030 mg/kg/day and 0.030-0.035 mg/kg/day in pubertal children. Growth velocity was used in the evaluation for growth response to rhGH therapy in all centers. Anthropometric measurements of patients every 3-6 months, fasting blood glucose, bone age and thyroid panel evaluation were used by all centers at follow-up. Main indications for cessation of therapy were decreased height velocity and advanced bone age. Fourteen centers used combined treatment (rhGH and gonadotropin-releasing analogues) to increase final height. Conclusion: Although conformity was found among centers in Turkey in current practice, it is very important to update guideline statements and to modify, if needed, the approach to GHD over time in accordance with new evidence-based clinical studies.

A 3-month-old female infant presented with swelling of the external genitalia. She had been born prematurely, at 27 weeks of gestation. After discharge, her mother noted the gradual development of genital swelling. Ultrasonography of the abdomen revealed two cysts on the right ovary.