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Congenital adrenal hyperplasia (CAH) is classified as classical CAH and non-classical CAH (NCCAH). In the classical type, the most severe form comprises both salt-wasting and simple virilizing forms. In the non-classical form, diagnosis can be more confusing because the patient may remain asymptomatic or the condition may be associated with signs of androgen excess in the postnatal period or in the later stages of life. This review paper will include information on clinical findings, symptoms, diagnostic approaches, and treatment modules of NCCAH.

Yeşilbaş O, Şevketoğlu E, Kıhtır HS, Hatipoğlu N, Yıldırım HM, Akyol MB, Aktay-Ayaz N, Gökçe İ. Leptospirosis in a child with acute respiratory distress syndrome. Turk J Pediatr 2017; 59: 688-692. Leptospirosis is an infectious vasculitis, which can occur with different clinical features. While it is generally a subclinical and self-limited infection; kidney and liver dysfunction, pulmonary hemorrhage, thrombocytopenia, and cardiovascular collapse may occurr. A six-year-old boy presented with acute respiratory distress syndrome, shock, thrombocytopenia-associated multiple organ failure, and persistent high fever secondary to leptospirosis. Persistent high fever was resistant to intravenous immunoglobulin and pulse steroid therapy. He was successfully treated with plasmapheresis and hemofiltration with endotoxin-cytokine cleaning filter. In conclusion; leptospirosis may cause thrombocytopenia-associated multiple organ failure, persistent high fever, and acute respiratory distress syndrome. Plasmapheresis and hemofiltration should be considered in cases of severe leptospirosis with multiorgan failure.

Contiguous gene deletions of chromosome Xp21 can lead to glycerol kinase deficiency and severe adrenocortical insufficiency (AI) in a male newborn among other problems. We describe our experience with two such patients who presented with dysmorphic facies, AI, and pseudo-hypertriglyceridemia. Both infants had normal serum 17-hidroxyprogesterone levels, and adrenal glands could not be observed with ultrasonography. Creatine kinase and triglyceride levels were measured to elucidate the etiology of adrenal hypoplasia and were above normal limits in both cases. Both patients required steroid and salt supplementation. They were both found to have Xp21.2 deletions (DMD, NR0B1, GK, IL1RAPL1). We conclude that AI in the context of other genetic abnormalities should prompt chromosomal investigations in the absence of another unifying explanation., Competing Interests: Financial Disclosure: The authors declared that this study received no financial support.

This is an observational epidemiological study to describe causes of bacterial meningitis among persons between 1 month and 18 y of age who are hospitalized with suspected bacterial meningitis in 7 Turkish regions. covering 32% of the entire population of Turkey. We present here the results from 2013 and 2014. A clinical case with meningitis was defined according to followings: any sign of meningitis including fever, vomiting, headache, and meningeal irritation in children above one year of age and fever without any documented source, impaired consciousness, prostration and seizures in those < 1 y of age. Single tube multiplex PCR assay was performed for the simultaneous identification of bacterial agents. The specific gene targets were ctrA, bex, and ply for N. meningitidis, Hib, and S. pneumoniae, respectively. PCR positive samples were recorded as laboratory-confirmed acute bacterial meningitis. A total of 665 children were hospitalized for suspected acute meningitis. The annual incidences of acute laboratory-confirmed bacterial meningitis were 0.3 cases / 100,000 population in 2013 and 0.9 cases/100,000 in 2014. Of the 94 diagnosed cases of bacterial meningitis by PCR, 85 (90.4%) were meningococcal and 9 (9.6%) were pneumococcal. Hib was not detected in any of the patients. Among meningococcal meningitis, cases of serogroup Y, A, B and W-135 were 2.4% (n = 2), 3.5% (n = 3), 32.9% (n = 28), and 42.4% (n = 36). No serogroup C was detected among meningococcal cases. Successful vaccination policies for protection from bacterial meningitis are dependent on accurate determination of the etiology of bacterial meningitis. Additionally, the epidemiology of meningococcal disease is dynamic and close monitoring of serogroup distribution is comprehensively needed to assess the benefit of adding meningococcal vaccines to the routine immunization program.

Human metapneumovirus (hMPV), formerly classified in Paramyxoviridae family is now moved into Pneumoviridae, which was described as a novel family. It causes upper and lower respiratory tract infections (LRTIs) usually in children younger than five years old. The recent epidemiological studies indicated that hMPV is the second most frequently detected virus in LRTIs of young children, following the respiratory syncytial virus (RSV). Bronchiolitis obliterans (BO) is a chronic obstructive lung disease characterized by fibrosis of the distal respiratory airways. It is usually a result of an inflammatory process triggered by a LRTI related to adenovirus, RSV, Mycoplasma pneumoniae, measles virus, Legionella pneumophila, influenza virus or Bordetella pertussis as a causative agent. In this report, a case of hMPV bronchiolitis complicated with BO has been reported to point out the complications and severity of the clinical progress belongs to this virus. A three-month-old female patient has admitted to our pediatric intensive care unit with the diagnosis of acute bronchiolitis and respiratory failure. She was born at term, weighing 2950 gram and had been hospitalized in newborn intensive care unit for 11 days with the diagnosis of transient tachypnea of the newborn and neonatal sepsis. On auscultation, there were bilateral crepitant rales, wheezing and prolonged expirium. Her oxygen saturation was 97-98% while respiratory support was given with a non-rebreathing reservoir mask. Complete blood count, procalcitonin and C-reactive protein levels were in normal ranges. The chest radiography yielded right middle lobe atalectasia, left paracardiac infiltration and bilateral air trapping. A nasopharyngeal swab sample was analyzed by a commercial multiplex real-time reverse transcriptase-polymerase chain reaction (Thermo Fisher Scientific®, USA) developed for the detection of 15 respiratory viruses. Her sample yielded positive result for only hMPV. On the 4th day of hospitalization, the patient was intubated because of respiratory failure and carbon dioxide retention. She was extubated on the 19th day but could not tolerate. In the thorax computed tomography (CT), bilateral hyperinflation, patchy infiltration, mosaic perfusion and atelectasis especially bilateral posterior areas were detected. Bronchoscopy was normal except mild bronchomalacia in right middle lobe bronchus. The patient was diagnosed as BO secondary to hMPV bronchiolitis, according to the clinical, virological, bronchoscopic and thorax CT results. On the 76th day of admission, she was discharged with respiratory support with home ventilation via a tracheostomy cannula and medical treatments of oral metilprednisolone, nebulized salbutamol and budesonide. In conclusion, hMPV should not be undervalued especially in infants with severe LRTI that can be complicated with BO.

Objective: Noonan syndrome (NS) is a multisystem disorder, and short stature is its most striking manifestation. Optimal growth hormone (GH) treatment for NS is still controversial. In this study, using a nationwide registration system, we aimed to evaluate the growth characteristics and the clinical features of NS patients in Turkey and their growth response to GH treatment., Methods: Children and adolescents with a diagnosis of NS were included inthe study. Laboratory assessment including standard GH stimulation test results were evaluated. Height increment of patients with or without GH treatment were analyzed after three years of therapy., Results: A total of 124 NS patients from different centers were entered in the web-based system. Short stature and typical face appearance were the most frequently encountered diagnostic features of our patients. Of the 84 patients who were followed long-term, 47 hadreceived recombinant human GH (rhGH). In this group of 47 patients, height standard deviation score (HSDS) increased from -3.62±1.14 to -2.85±0.96 after three years of therapy, indicating significant differences from the patients who did not receive GH treatment. PTPN11 gene was analyzed in 61 patients, and 64% of these patients were found to have a mutation. HSDS at admission was similar in patients with or without PTPN11 gene mutation., Conclusion: A diagnosis of NS should be kept in mind in all patients with short stature showing systemic clinical findings. GH therapy is effective for improvement of short stature especially in the first two years of treatment. Further studies are needed for optimisation of GH therapy and evaluation of final height data in NS patients.

Background: Leptospirosis is a zoonotic infectious disease caused by pathogenic spirochetes of the genus Leptospira. Although it is usually asymptomatic and self-limited, severe potentially fatal illness accompanied by multi-organ failure may occur., Case Report: Here we report an unusual case of severe leptospirosis successfully treated with continuous venovenous hemofiltration (CVVHF) and therapeutic plasma exchange (TPE). The patient presented with pericardial tamponade, renal failure and macrophage activation syndrome, and later suffered prolonged jaundice and sclerosing cholangitis during hospitalization in the pediatric intensive care unit (PICU). To the best of our knowledge, sclerosing cholangitis due to leptospirosis has not been reported in the literature., Conclusion: Leptospirosis should be kept in mind in the differential diagnosis of sepsis and septic shock with fever, thrombocytopenia, jaundice and renal failure. TPE and CVVHF should start early after the diagnosis of leptospirosis with multiorgan failure., Competing Interests: No conflict of interest was declared by the authors.

Gonadotropin-releasing hormone analogues are common treatment option in central precocious puberty in childhood as well as in endometriosis, infertility, and prostate cancer in adults. Pseudotumor cerebri is a rare side effect observed in adults. We present the case of a girl with precocious puberty treated with triptorelin acetate who developed pseudotumor cerebri after the 4th dose. She had headaches, and her blood pressure was detected to be above the 99 percentile. There were no causes underlying of hypertension such as cardiac, renal, or endocrine. Neurological examination was normal except bilateral papilledema. Cranial magnetic resonance imaging was normal. Cerebrospinal fluid (CSF) opening pressure was elevated. Triptorelin therapy was ceased and acetazolamide was applied; CSF pressure returned to normal. We observed pseudotumor cerebri after precocious puberty treatment, a finding for the first time ever seen in childhood.

Objective: The aim of this study was to establish the association between anthropometric parameters and non-alcoholic fatty liver disease (NAFLD) and to determine the most reliable measurement as a parameter in predicting NAFLD., Methods: Two-hundred fifty-three obese children of ages 10 to 18 years were enrolled in this study. Anthropometric data and metabolic parameters such as fasting blood glucose, insulin and lipid levels, were measured. Liver function tests were assessed. NAFLD was determined by ultrasound., Results: Most metabolic parameters and anthropometric indices were significantly higher in children with NAFLD. A univariate logistic regression analysis was performed, taking NAFLD status as the dependent variable and anthropometric parameters as the independent variables. NAFLD was affected significantly by the anthropometric values. The multiple logistic regression analysis showed that neck circumference (NC) was the only parameter which determined the risk in both genders. Each 1 cm increase in the NC increased the risk of NAFLD 1.544-fold (p<0.001, 95% confidence interval (CI): 1.357-2.214) in the boys and 1.733-fold (p=0.001, 95% CI: 1.185-2.012) in the girls. Receiver operating characteristic analysis was performed to compare the reliability of anthropometric measurements. NC was observed to be a better indicator., Conclusion: Measurement of the NC was shown to be associated with NAFLD in children. We suggest the use of NC as a novel, simple, practical, and reliable anthropometric index in predicting children at risk for NAFLD.

Objective: The burdens imposed on a child and his/her parents by a diagnosis of type 1 diabetes mellitus (T1DM) adversely affect their health-related quality of life (HRQoL). HRQoL is important for prognosis and is related to metabolic control. To evaluate the HRQoL of Turkish children and adolescents with T1DM and to assess the correlation of HRQoL subscales (including physical and psychosocial health) with metabolic control, and particularly with hypo- and hyperglycaemic episodes., Methods: This cross-sectional study included 70 participants with T1DM aged between 8 and 18 years (study group) and 72 healthy controls who were matched to the study group in terms of age, gender, and sociodemographic characteristics (control group), and their parents. HRQoL was determined by the Pediatric Quality of Life Inventory. As an indicator of metabolic control, the most recent hemoglobin A1c (HbA1c) levels were obtained and the number of hypo- and hyperglycaemic episodes over the past one month were checked., Results: The study group had similar HRQoL scores for children's self-reports and parents' proxy-reports to the control group apart from a decreasing psychosocial health score for parents' proxy-reports in the study group. Although HbA1c level was not related to HRQoL scores, lower number of hypo- and hyperglycaemic episodes were associated with an increase in psychosocial health scores and physical health scores as well as an increase in the total score for parents' proxy-reports., Conclusion: Although there was no correlation between metabolic control and HRQoL in children's self-reports, the improving HRQoL levels in parents' proxy-reports were associated with good metabolic control.

Human bocavirus (HBoV), that was first identified in 2005 and classified in Parvoviridae family, is a small, non-enveloped, single-stranded DNA virus, responsible for upper and lower respiratory tract infections, especially in young children. Although HBoV generally causes self-limited influenza-like illness, it may also lead to pneumonia, bronchiolitis, croup and asthma attacks. In this report, a case of acute bronchiolitis complicated with pneumomediastinum and bilateral pneumothorax caused by HBoV has been presented. A three-year-old boy was referred to our pediatric intensive care unit with a two day history of fever, tachypnea, hypoxia and respiratory failure. On auscultation, there were widespread expiratory wheezing and inspiratory crackles. The chest radiography yielded paracardiac infiltration and air trapping on the right lung and infiltration on the left lung. The patient had leukocytosis and elevated C-reactive protein level. On the second day of admission, respiratory distress worsened and chest radiography revealed right pneumothorax and subcutaneous emphysema in bilateral cervical region and left chest wall. He was intubated because of respiratory failure. In the thorax computed tomography, pneumomediastinum and bilateral pneumothorax were detected and right chest tube was inserted. Repetitive blood and tracheal aspirate cultures were negative. A nasopharyngeal swab sample was analyzed by multiplex real-time polymerase chain reaction method with the use of viral respiratory panel (FTD(®) Respiratory Pathogens 21 Kit, Fast-Track Diagnostics), and positive result was detected for only HBoV. On the ninth day of admission, pneumomediastinum and bilateral pneumothorax improved completely and he was discharged with cure. In conclusion, HBoV bronchiolitis may progress rare but severe complications, it should be kept in mind as an etiological agent of the respiratory tract infections especially children younger than five years old.

Pediatric sarcoidosis comprises a spectrum of childhood granulomatous inflammatory conditions. Pathological hallmark of the disease is granuloma formation that is seen in the affected tissues and almost any organ or system can be involved. There are two forms of pediatric sarcoidosis. One is seen in older children and the clinical picture is very similar to that of adult sarcoidosis and the other one is seen in early childhood. Sarcoidosis in early childhood can be divided as Blau syndrome (familial form) and early onset sarcoidosis (sporadic form). In both of the diseases there is a defect in the NOD2/CARD15 gene. The typical triad of early onset sarcoidosis is polyarthritis, dermatitis and uveitis. Interferon-γ receptor 1 deficiency is caused by defects in the IFNγR1 gene and non-tuberculosis mycobacterial pathogens are the leading causes of infections that start in early childhood. Herein we report a patient who presented with the symptoms of early onset sarcoidosis and also had partial interferon-γ receptor 1 deficiency that presented with BCG-osis. In addition to anti-mycobacterial treatment, methotrexate and prednisolone were used in therapy.

Gonadotropin-releasing hormone analogues are used in the treatment of prostate cancer, breast cancer, endometriosis, and uterine leiomyomas in adults and often in the treatment of precocious puberty in children. Many adverse effects have been reported for gonadotropin-releasing hormone analogues, but anaphylaxis is rarely reported as an adverse effect. Frequent cross-reactions, particularly during childhood, and diversity of the time of onset of anaphylactic manifestations complicate the diagnosis. A patient who exhibited anaphylactic allergic reactions to two different agents used in the treatment of central precocious puberty presented here because the case has an atypical course and is the first in the literature.

Objective: To investigate the efficacy and safety of n-3 polyunsaturated fatty acids (PUFA) treatment in obese children with nonalcoholic fatty liver disease (NAFLD)., Methods: One hundred and eight obese (body mass index (BMI) >95th percentile for age and sex) adolescents with NAFLD were included in the study. Mean age of the subjects was 13.8 ± 3.9 years (9-17 yrs). The diagnosis of NAFLD was based on the presence of liver steatosis with high transaminases. The subjects were randomly divided into two groups. Group 1 (PUFA group, n=52) received a 1000 mg dose of PUFA once daily for 12 months and lifestyle intervention. Group 2 (placebo group, n=56) received a recommended diet plus placebo and lifestyle intervention for 12 months. Insulin resistance was evaluated by homeostasis model assessment of insulin resistance (HOMA-IR) from fasting samples., Results: BMI, fasting insulin levels and HOMA-IR values in both groups decreased significantly at the end of the study. In group 1, 67.8% of the patients had a decrease from baseline in the prevalence of steatosis (p<0.001). Frequency of elevated alanine aminotransferase (ALT) levels (39.2% to 14.2%; p<0.01) and elevated aspartate aminotransferase (AST) levels (25% to 17.8%; p=0.01) decreased significantly in the PUFA group. Following a 12-month diet plus placebo and lifestyle intervention treatment, 40.3% (21) of the patients in the placebo group also showed a decrease in frequency of steatosis (p=0.04) and slight decreases in frequency of elevated ALT levels (38.4% to 28.8%; p=0.01) and AST levels (30.7% to 28.8%; p>0.05)., Conclusion: Our results indicated that n-3 PUFA treatment is safe and efficacious in obese children with NAFLD and can improve ultrasonographic findings and the elevated transaminase levels.

Infectious diseases are the leading cause of FUO. A case of prolonged fever with hepatic and pulmonary tuberculosis as a final diagnosis is herein presented. A 4-year-old, otherwise healthy boy presented with an axillary temperature of up to 39.5ºC for the previous 3 weeks. His medical history revealed an occasional increase in body temperature up to 38.5ºC for the last 6 months. Physical examination revealed coarse breath sounds on the basal lung area. Chest X-ray showed mediastinal lymphadenomegaly and computed tomography revealed paratracheal conglomerated lymph nodes and a groundglass appearance on the right lung. There were multiple contrast-enhanced, hypoechoic nodules with central necrosis in the liver parenchyma on abdominal magnetic resonance imaging. Open liver biopsy yielded chronic granulomatous inflammation compatible with pathological findings of tuberculosis infection. The culture specimen was positive for Mycobacterium tuberculosis. The patient improved rapidly after antituberculous therapy was initiated. Tuberculosis, especially in its disseminated form, poses a distinct diagnostic challenge in cases of prolonged fever with unproven etiology, and thus persistence should be exercised in disclosing the cause of such fevers.

Aim: To investigate metabolic syndrome (MetS) and MetS criteria, and to establish whether metabolic syndrome criteria were associated with non-alcoholic fatty liver disease (NAFLD) in obese children., Methods: A total of 451 pubertal obese children (8-18 years old) were enrolled in the study. Patients were divided into three groups according to the degree of steatosis. Antropometric and laboratory measurements of the participants were recorded., Results: Of 451 obese children, 217 (48.1%) were diagnosed as having NAFLD and 96 (21.3%) as having MetS. The frequency of abdominal obesity, hypertension, impaired fasting glucose, hyperinsulinemia, dyslipidemia and type 2 diabetes mellitus (T2DM) were 61.8% (279), 25.7% (116), 4.4% (20), 54.3% (245), 41% (185) and 2.2% (10), respectively. The prevalence of NAFLD among patients with MetS [73% (70/96)], was significantly higher than the frequency of hypertension [55% (53/96)] and abnormalities of glucose metabolism [23% (22/96)], but almost equal to the frequency of dyslipidemia [78% (75/96)]. The prevalence of MetS criteria were higher in patients with NAFLD than those without NAFLD. Except impaired fasting glucose, blood pressure and T2DM significant difference was found between groups for all. It was observed that the number of MetS criteria increased in parallel with the severity of steatosis., Conclusion: NAFLD in obese children is strongly associated with multiple MetS criteria. In addition to NAFLD is not only a liver disease, but also early mediator that reflects metabolic disorder, and liver ultrasound can be a useful tool for MetS screening., (Copyright © 2013 Asian Oceanian Association for the Study of Obesity. Published by Elsevier Ltd. All rights reserved.)

Background/aim: There are close interactions among the developing oral cavity, pituitary gland, and central nervous system (CNS) in early embryonic life. In this study we aimed to screen endocrine abnormalities in patients with orofacial clefts in the neonatal period., Materials and Methods: Thirty-one patients with isolated orofacial median clefts wereincluded in the study. Pituitary, thyroid, and adrenal hormones were measured at the first week and remeasured in the third or fourth weeks. Imaging studies were done for detection of CNS anomalies in all patients., Results: Endocrine abnormality was detected in 22 (70.9%) patients. The number of patients with single and multiple endocrine abnormalities were 13 (41.9%) and 9 (29%), respectively. Thyroid hormone-related disorders were detected in 10 (32.3%) patients. Growth hormone deficiency was detected in 4 (12.9%) patients. Adrenocorticotrophic hormone and/or glucocorticoid deficiency was detected in 5 (16.1%) patients. Neonatal hypoglycemia due to endocrinological abnormalities was detected in 6 (19.4%) patients. Defected mini-puberty was seen in 2 (15.4%) patients. There was no relationship between the types of orofacial cleft and endocrine abnormalities., Conclusion: Endocrinological evaluation of the patients with orofacial clefts in the neonatal period is a worthwhile endeavor to detect hormone deficiencies regardless of the type of the cleft.

The purpose of the present study is to compare male patients with the fibromyalgia syndrome (FMS) with healthy individuals in terms of the sexual function. For the purposes of this study, 37 sexually active male FMS patients and 30 healthy controls were enrolled. The demographic data of the patients were recorded, and the widespread pain observed in FMS was graded with the help of the visual analogue scale (VAS 0-100 mm). Sexual function was assessed according to the international index of erectile function (IIEF) scoring system. The disease-related quality of life was measured with the help of the Short Form-36 quality of life questionnaire (SF-36 QoL). Levels of anxiety and depression observed in the patients were graded through the Hospital Anxiety and Depression Scale (HADS). Patients with FMS had significantly lower scores in each of the five domains of the IIEF in comparison with the healthy control group (p < 0.001). Patients' age and widespread pain were negatively correlated with the IIEF scores (p < 0.05). The SF-36 scores (physical functioning, role limitations due to physical problems, pain and general health perception) were observed to be positively correlated with the IIEF scores (p < 0.05). No significant relationship has been observed between the scores obtained from the domains of IIEF and the psychological status (p > 0.05). FMS leads to an impairment in the sexual function in male patients, which is especially strongly associated with the age, widespread pain and the quality of life.

Micropenis is a medical diagnosis based on correct measurement of length. If stretched penile length is below the value corresponding to - 2.5 standard deviation of the mean in a patient with normal internal and external male genitalia, a diagnosis of micropenis is considered. Micropenis can be caused by a variety of factors including structural or hormonal defects of the hypothalamic-pituitary-gonadal axis. It can also be a component of a number of congenital syndromes. For the etiological evaluation, endocrinologic tests are important. This article reviews the etiology, diagnosis, treatment and management of micropenis.

Context: An association between low birth weight, insulin resistance (IR), dyslipidemia, and atherogenesis has been shown in girls with precocious adrenarche (PA)., Objective: To evaluate whether girls with PA born appropriate for gestational age (AGA) have increased risk for metabolic complications at initial evaluation., Design/methods: We conducted a cross-sectional study on 69 AGA born girls with PA (mean (±s.d.) age 7.1±1 years) and 45 body mass index (BMI)- and waist circumference (WC)-matched prepubertal peers born AGA (mean (±s.d.) 7.5±1.9 years). A standard 2-h oral glucose tolerance test with insulin sampling was performed. Fasting plasma lipids and high-sensitivity C-reactive protein were analyzed, and blood pressure was recorded. Insulin sensitivity (IS) index (ISIcomp), homeostasis model assessment of IR, and atherogenic index (AI) (triglycerides/high-density lipoprotein cholesterol) were calculated., Setting: The study was performed at University Hospital., Results: AI was significantly lower in girls with PA than in controls (P<0.001), and it was correlated with BMI SDS (r=0.44, P=0.001) and WC (r=0.39, P=0.001). The significant correlation of AI with ISIcomp (r=-0.38, P=0.001) disappeared after correcting for BMI (r=-0.185, P=0.16). Multivariate regression analysis revealed that DHEAS was the only significant parameter influencing AI in girls with born AGA (R (2)=0.475 β=-0.018, P=0.0001)., Conclusions: Metabolic screening in prepubertal AGA born girls with PA may yield favorable lipid profiles. AI in girls with PA is increased in relation to decreasing IS and increasing BMI and WC. DHEAS seems to have the most significant effect on AI.

Objective: Intrauterine growth references are primarily useful indicators in the assessment of the general health status of newborn infants. Although Lubchenco's references are still used in many neonatal care units, we believe that there is a need for up-to-date intrauterine growth references specific for different populations. To develop gestational age-and gender-specific national references for birth weight, birth length and head circumference., Methods: Data were collected from neonatal records of perinatology services of eleven hospitals from January to December 2009. The anthropometry of a total of 4750 singleton live births born between 28 and 41 weeks of gestation were recorded. Means and standard deviations were calculated, and percentiles for each gender and gestational week were produced using the LMS program. The results were compared with US infants and also with local data., Results: Gestational age- and gender-specific 3rd, 5th, 10th, 15th, 25th, 50th, 75th, 85th, 90th, 95th and 97th percentile values were produced. Comparison of the 10th, 50th and 90th percentile values showed that the boys were heavier and longer than the girls. Head circumference values were also higher in the boys. Proportions of small for gestational age (SGA), appropriate for gestational age (AGA) and large for gestational age (LGA) infants in the sample were 10.1%, 79.1% and 10.8%, respectively., Conclusion: These gender- and gestational age-specific references will be of use in clinical practice and also for research purposes until more comprehensive, reliable and accessible national data pertaining to the intrauterine growth of Turkish infants are produced.

The efficacy of Quantiferon-TB gold test (QFT-GIT) remains to be documented in pediatric population. Tuberculin skin test (TST) is a conventional test available for the diagnosis of latent tuberculosis infection (LTBI). We aimed to investigate the concordance between QFT-GIT and TST in children with and without tuberculosis infection. Ninety-seven patients, aged 3 months-14 years, admitted to pediatric outpatient clinics of Dr. Sadi Konuk Training Hospital Bakırköy, Turkey between March 2008 and April 2009 were recruited. Demographic features, TST results, history of exposure to active tuberculosis (TB), chest X-ray findings, clinical history, presence of Bacillus Calmette Guerin (BCG) vaccination scar were recorded. Patients were categorized into four groups namely, active TB, LTBI, no TB and healthy. It was found that BCG scar positivity did not influence QFT-GIT results. There was a statistically significant agreement between QFT-GIT and TST results (κ = 0.486; p < 0.01). In patients ≥ 5 years of age, TST positivity and QFT positivity had a significant relationship (p < 0.01). In all patient groups, sensitivity and specificity was 65.85 % and 82.14 %, respectively. In active TB group, TST and QFT-GIT results demonstrated significant agreement ratio of 40.8 % (κ = 0.364; p < 0.01). Sensitivity and specificity was 100 % and 30 %, respectively. Utilization of QFT-GIT in the diagnosis of LTBI reduces false-positive results and prevents unnecessary treatment with INH and its adverse effects.

This study was performed to investigate the viral etiological agents, age distribution and clinical manifestations of lower respiratory tract infection (LRTI) in hospitalized children. The viral etiology and clinical findings in 147 children (1 month to 5 years of age) hospitalized with acute LRTI were evaluated. Cell culture was used for isolation of influenza viruses and direct fluorescent antibody assay for parainfluenza viruses (PIVs), respiratory syncytial virus (RSV) and adenoviruses (ADVs). Reverse-transcriptase polymerase chain reaction was employed for human metapneumovirus (hMPV). One hundred and six of all patients (72.1%) were male, and 116 children (79.8%) were < or = 2 years. A viral etiology was detected in 54 patients (36.7%). RSV was the most frequently isolated (30 patients, 55.6%), and PIV (27.8%), hMPV (13%), influenza-A (9.3%), and ADV (5.6%) were also shown. Dual infection was detected in six patients. There were no statistically significant differences between the two groups (with isolated virus or no known viral etiology) with respect to symptoms, clinical findings, laboratory work-up, or radiological data. Length of hospital stay was also not different. Determination of the etiology of acute LRTI in children less than 5 years of age seems impossible without performing virological work-up, whether viral or nonviral in origin.

Background: Growth hormone (GH), insulinlike growth factor-1 (IGF-1), and IGF-binding protein-3 (IGFBP-3) are used in the neonatal period for evaluation of diseases such as GH deficiency, intrauterine growth restriction, and hypoglycemia. However, GH level has been reported as a single value during the neonatal period. In this study, we attempted to determine the changes of GH, IGF-1, and IGFBP-3 in the neonatal period for each week., Methods: One hundred and two appropriate-for-gestational age (AGA) term neonates who did not have any diseases that would interfere with GH-IGF axis were included in this cross-sectional study. Blood samples were collected and serum GH, IGF-1 and IGFBP-3 levels were measured by immunoradiometric analysis (IRMA) and weekly values were obtained for the first postnatal four weeks., Results: We showed that GH level [median (1st-3rd quartile)] decreases from the first to the fourth postnatal week [25.1 (18.5-37.4); 17.2 (13.2-22.8); 17.6 (12.2-20.2); 14.4 (6.60-19.8) ng/mL, respectively)], IGF-1 and IGFBP-3 levels [median (lst-3rd quartile)] increase [41.7 (18.0-70.0); 55.9 (39.0-103); 53.0 (40.0-97.7); 71.7 (44.3-137) and 1852 (1597-2451); 2430 (1645-2838); 2841 (2280-3675); 3018 (2151-4189) ng/mL, respectively)]., Conclusions: GH, IGF-1 and IGFBP-3 values can be evaluated for each week separately instead of for the entire neonatal period.

The involvement of the central nervous system (CNS) in brucellosis is rare and has a broad range of presentations. Subacute and chronic meningoencephalitis are described as the most common neurologic manifestations. We report a six-year-old boy with culture-proven neurobrucellosis who presented with an acute picture of meningoencephalitis. Cerebrospinal fluid (CSF) analysis revealed pleocytosis with slight elevation of protein. The agglutination test titer was elevated in serum and Brucella spp. were isolated from both blood and CSF. He was treated with trimethoprim-sulfamethoxazole plus rifampin and streptomycin. His clinical and laboratory features improved with specific antibiotic therapy and no sequela was observed in the short-term follow-up. Due to protean clinical features, unfamiliarity with the disease can delay the diagnosis in children who are not occupationally exposed. In endemic areas, neurobrucellosis should be considered in the evaluation of patients with unexplained neurologic symptoms.

Objective: Childhood obesity is associated with an increased risk for insulin resistance. The underlying mechanism for the physiological increase in insulin levels in puberty is not clearly understood. The aim of the present study was to determine the cut-off values for homeostasis model assessment for insulin resistance (HOMA-IR) in obese children and adolescents according to gender and pubertal status., Methods: Two hundred and eight obese children and adolescents (141 girls, 127 boys) aged between 5 and 18 years were included in the study. The children were divided into prepubertal and pubertal groups. A standard oral glucose tolerance test (OGTT) was carried out in all children. A total insulin level exceeding 300 μU/mL in the blood samples, collected during the test period, was taken as the insulin resistance criterion. Cut-off values for HOMA-IR were calculated by receiver operating characteristic (ROC) analysis., Results: In the prepubertal period, the rate of insulin resistance was found to be 37% in boys and 27.8% in girls,while in the pubertal period, this rate was 61.7% in boys and 66.7% in girls. HOMA-IR cut-off values for insulin resistance in the prepubertal period were calculated to be 2.67 (sensitivity 88.2%, specificity 65.5%) in boys and 2.22 (sensitivity 100%, specificity 42.3%) in girls, and in the pubertal period, they were 5.22 (sensitivity 56%, specificity 93.3%) in boys and 3.82 (sensitivity 77.1%, specificity 71.4%) in girls., Conclusions: Since gender, obesity and pubertal status are factors affecting insulin resistance, cut-off values which depend on gender and pubertal status, should be used in evaluation of insulin resistance.

Objective: The purpose of this study was to determine the cut-off values for waist circumference (WC) and mid-upper arm circumference (MUAC) and to assess their use in screening for obesity in children., Methods: Anthropometric measurements of a total of 2621 boys and 2737 girls aged 6-17 years were analyzed. WC and MUAC values were compared with ROC analysis using body mass index (BMI) cut-off values of the International Obesity Task Force (IOTF) and using WC≥ 90th percentile.for MUAC., Results: In both genders, except for boys and girls in the 6-year age group and post-pubertal boys, the differences between area under curve (AUC) values for WC and MUAC were not significant, indicating that both indices performed equally well in predicting obesity. Sensitivity was suboptimal through age groups 6-9 years in the boys and sensitivity was suboptimal at 6, 7,14 and 17 years both in boys and girls., Conclusions: We conclude that MUAC can be a useful parameter in screening obesity and body fat distribution in children and, can be applied in epidemiological studies and in clinical practice.

Background: One of the mostly used and preferred method in following the growth of children is to plot weight and height values of the children on standard percentile charts. It is essential for each country to use its own populations' updated percentile curves. However, data on the growth of children living in different regions are also needed for comparison with the national standards., Methods: This study was conducted in Kayseri with a trained team in order to obtain anthropometrical measurements in children and adolescents. Weight and height measurements from 5727 (2785 boys, 2942 girls) healthy school children (aged between 6 to 18 years) from all socioeconomic levels were randomly selected. Smoothed percentile curves were produced by LMS method., Results: Smoothed percentile curves including the percentile values for 3rd, 5th, 10th, 25th, 50th, 75th, 85th, 90th, 95th and 97th and standard deviation scores were calculated for boys and girls. The 3rd, 50th and 97th centiles of weight and height of these children were compared with the respective values of the established national standards obtained from Istanbul children., Conclusions: This study presents data and smoothed percentile curves for weight and height measurement of healthy central Anatolia children aged 6 to 18 years. Nationwide studies are needed to bring out the regional differences in our country.

Ectrodactyly-ectodermal dysplasia-clefting (EEC) syndrome is characterized by ectodermal dysplasia, ectrodactyly and facial clefting with multiple congenital anomalies such as urinary tract anomaly, lacrimal duct obstruction, and hearing loss. This syndrome is a rare disease transmitted by autosomal dominant inheritance with variable penetrance. Clinical expression is variable. In EEC syndrome with midline defect hypothalamo-pituitary endocrinopathy is expected, however hormonal disorders in EEC syndrome have rarely been reported. We present two patients with EEC syndrome associated with hypothalamo-pituitary insufficiency.

Background: Zinc (Zn) plays a central role in the activation of numerous enzyme systems that synthesize and degrade bioactive peptides. Some of these bioactive peptides, also called neuropeptides, are involved in the regulation of food intake., Objective: In this study we aimed to demonstrate the relationship between serum ghrelin and hair Zn concentrations in children., Methods: Prepubertal children brought to our outpatient clinics by their parents because of signs and symptoms of pica, poor appetite, poor growth, and other complaints were included in the study. The children were divided into two groups according to Zn hair concentrations. Group 1 consisted of children with low (< 70 μg/g) hair Zn levels, and group 2 of children with normal ( ≥ 70 μg/g) hair Zn levels. Hair Zn concentrations, serum ghrelin, insulin-like growth factor I (IGF-I) and IGF-binding protein-3 (IGFBP-3) levels were measured in all children., Results: There were 10 children with low hair Zn levels (group 1) and 15 with normal levels (group 2). Serum IGF-I, IGFBP-3 and ghrelin concentrations of group 1 (103.1 ± 71.8 ng/mL, 1412.8 ± 615.7 ng/mL and 0.96 ± 0.22 ng/mL, respectively) were lower than in group 2(164.9 ± 40.5 ng/mL, 2398.5 ± 295.5 ng/mL and 1.21 ± 0.23 ng/mL, respectively). In univariate analysis, Zn hair concentration was positively associated with serum IGF-I (r=0.424, p=0.035) and IGFBP-3 (r=0.671, p < 0.001) concentrations. The correlation between ghrelin and hair Zn concentrations was not significant (r=0.202, p=0.333)., Conclusion: Serum ghrelin concentrations might be affected by low hair Zn concentrations in children.

Chickenpox is a common infectious disease of the pediatric age group with rare complications such as hemorrhagic varicella and arterial thrombotic purpura. Medical support is the mainstay of treatment in such cases but for the rescue of necrotic tissues, hyperbaric oxygen (HBO) therapy should be applied in addition to anticoagulant intervention. We report an infant with acute arterial thrombotic purpura which developed after varicella eruption and who made full recovery with the help of HBO as an adjunctive treatment modality. Fresh frozen plasma and low molecular weight heparin were given for prolonged prothrombin time and thromboemboli on the 2nd-4th digits of his right foot. Protein C, protein S and factor V levels were found to be normal in our patient. Necrotic lesions on the toes regressed with repeated HBO treatment and amputation was not needed.