The relationship between serum phenylalanine levels, genotype, and developmental assessment test results in non-phenylketonuria mild hyperphenylalaninemia patients.

Unlabelled: Phenylalanine (PA) levels below 360 µmol/L do not require treatment; however, cognitive deficits have been observed in patients with elevated PA levels, necessitating a safe upper limit for treatment and therapeutic objectives. The main purpose of this study is to evaluate the correlation between developmental assessments (Denver Developmental Screening Test-II [DDST-II] and Ankara Developmental Screening Inventory [ADSI]) and electroencephalogram (EEG) findings with blood PA levels and genotypic data in non-phenylketonuria mild Hyperphenylalaninemia (HPA) patients, to re-evaluate their treatment status based on potential adverse outcomes. This study encompassed 40 patients aged 1-5 years diagnosed with HPA and not on treatment, identified through initial blood PA levels, and monitored for a minimum of 1 year on an unrestricted diet.

Corrosion Behavior of Nickel-Titanium Arch Wires Following the Use of Different Mouthwashes: An Study.

Objective: The aim of this double-blind study was to compare the extent of corrosion on the surface of nickel-titanium (NiTi) wires in various mouthwashes.

Methods: A total of 80 patients who received orthodontic treatment with as-received 0.016x0.

Proinflammatory indicators and the relevance of echocardiography in children with cystic fibrosis.

As the life expectancy improves in cystic fibrosis, cardiac dysfunction is becoming an important risk factor for morbidity and mortality. Here, the association of cardiac dysfunction with proinflammatory markers and neurohormones between cystic fibrosis patients and healthy children was investigated. Echocardiographic measurements of right and left ventricular morphology and functions together with levels of proinflammatory markers and neurohormones (renin, angiotensin-II, and aldosterone) were obtained and analysed in a study group of 21 cystic fibrosis children aged 5-18 years and compared with age- and gender-matched healthy children.

Evaluation of ketogenic diet therapy in children diagnosed with drug-resistant epilepsy: a single-center experience.

Background: We evaluate here the effect of the ketogenic diet (KD) on children with drug-resistant epilepsy (DRE) in terms of clinical effectiveness, anthropometric measurements, and some electroencephalogram (EEG) and biochemical findings.

Methods: Included in the study were 18 children (median age 70 months, 61.1% female) who received the classical KD and modified Atkins diet (MAD) for at least one year due to DRE.

Evaluation of micronutrient levels in children with cerebral palsy.

Background: Many studies evaluating the nutritional status of children with cerebral palsy (CP) have focused on energy requirements and protein intake. The present work aimed to assess nutritional status and micronutrient levels of children with (CP).

Methods: This multicenter, cross-sectional and observational study was conducted in 10 different cities in Turkey.

Assessment of respiratory tract viruses in febrile neutropenic etiology in children and comparison with healthy children with upper/lower respiratory tract infection.

Objective: This study aims to compare the frequency of respiratory viruses using real-time and multiplex polymerase chain reaction technology and nasopharyngeal swabs taken during exacerbation of patients aged 0-18 years followed for febrile neutropenia (FN) with non-FN children.

Methods: This prospective study included a total of 40 patients with FN and malignancies followed at Eskisehir Osmangazi University, Department of Pediatric Hematology and Oncology. The control group (n=76) consisted of age-matched patients with upper respiratory tract infections (URTIs) or lower respiratory tract infections (LRTIs) who were admitted to the emergency service due to fever.

Alkaptonuria in Turkey: Clinical and molecular characteristics of 66 patients.

Alkaptonuria (AKU) is an inborn error of metabolism caused by the deficiency of homogentisate 1,2-dioxygenase (HGD) as a result of a defect in the HGD gene. HGD enzyme deficiency results in accumulation of homogentisic acid (HGA) in the body, which in turn leads to multisystemic clinical symptoms. The present study aimed to investigate the presenting symptoms, age at diagnosis, and clinical and genetic characteristics of AKU patients followed-up in different centers in Turkey.

Patients with cerebrotendinous xanthomatosis diagnosed with diverse multisystem involvement.

Cerebrotendinous xanthomatosis (CTX) is a lipid storage disease caused by deficiency of sterol 27-hydroxylase enzyme encoded by CYP27A1 gene. This multicenter, cross-sectional descriptive study aimed to document clinical characteristics of CTX patients of different ages, clinical presentations of early-diagnosed patients, and responses to short-term chenodeoxycholic acid (CDCA) treatment. Seven of 11 CTX patients were diagnosed in childhood.

Social Jetlag Is Associated with the Frequency of Consumption of Sugar-Sweetened Beverages and a High BMI Percentile in Adolescents: Results of the Cross-Sectional Family Life, Activity, Sun, Health, and Eating (FLASHE) Study.

Background: Social jetlag (SJL) occurs in adolescents due to misalignment of the biological and social clocks, so that most teens wake up earlier than their biological clocks on weekdays and delay bedtime and wake up time on weekends. This shift in sleep timing among adolescents is associated with an adverse endocrine and behavioral risk profile, in addition to increased food consumption and increased body mass index (BMI).

Objective: The aim of this study was to determine the association between SJL, and the frequency of consumption of multiple food and beverage groups and BMI percentile in adolescents.

What is new in peripartum hysterectomy? A seventeen year experience in a tertiary hospital.

Objective: To identify changing trends in peripartum hysterectomy (PH), both elective. cesarean hysterectomy and emergency cesarean hysterectomy, at a single training and research hospital over the last 17 years in Istanbul, Turkey.

Materials And Methods: A retrospective cohort study was performed between January 2001 and September 2017.

[Fabry disease in cardiology: Diagnosis and therapeutic approaches].

Fabry disease is a rare, progressive, X-linked inherited storage disorder due to absent or deficient of lysosomal alfa galactosidase A activity. Deficient activity of alfa-galactosidase A results in progressive accumulation of globotriaosylceramide in a variety of tissues and organs including myocardium, kidney and nerve system. This disorder predominantly affects males; however, female heterozygotes may also be affected with a less severe clinical picture.

The association of lipid metabolism and non-alcoholic fatty liver disease in children with obesity.

Aim: Obesity, insulin resistance, and hyperlipidemia have been shown as risk factors for non-alcoholic fatty liver disease. In this study, the association between lipid and lipoprotein metabolism abnormalities and the presence of non-alcoholic fatty liver disease was investigated in patients with obesity.

Material And Methods: In this study, the clinical, laboratory and imaging findings of 357 children and adolescent patients (199 girls and 158 boys) aged 2-18 years who were diagnosed as having obesity between 2013 and 2018 were retrospectively analyzed.

Sudden cardiac arrest during induction of anaesthesia in paediatric patient with glutaric aciduria type II.

Glutaric aciduria type II (GA2) is an autosomal recessive metabolic disorder of amino acid and lipid metabolism, which is serious and rare. The most serious form is seen in early infancy and is associated with very high mortality rates. Here, we present an 8-month-old male patient with GA2 who had electrocardiographic ST ST-segment depression and sudden cardiac arrest at 10th minute of emergency operation (central venous catheter placement).

Patients eligible for modulator drugs: Data from cystic fibrosis registry of Turkey.

Background: A better understanding of cystic fibrosis transmembrane conductance regulator biology has led to the development of modulator drugs such as ivacaftor, lumacaftor-ivacaftor, tezacaftor-ivacaftor, and elexacaftor-tezacaftor-ivacaftor. This cross-sectional study evaluated cystic fibrosis (CF) patients eligible for modulator drugs.

Methods: Data for age and genetic mutations from the Cystic Fibrosis Registry of Turkey collected in 2018 were used to find out the number of patients who are eligible for modulator therapy.

Evaluation of FGF-19 and β-klotho as biomarkers in patients with intrahepatic cholestasis of pregnancy.

Introduction: Fibroblast growth factor-19 (FGF-19) and its co-receptor, beta-klotho, regulate bile acid synthesis in the liver as an enterohepatic feedback mechanism. In this study, our aim was to investigate the circulating FGF-19 and β-klotho levels in intrahepatic cholestasis of pregnancy (ICP) cases.

Material And Methods: A cross-sectional study including 40 women whose pregnancies were complicated with ICP were recruited for the study group.

Characteristics of total laparoscopic hysterectomy among women with or without previous cesarean section: retrospective analysis.

Background: The number of hysterectomized patients with previous cesarean sections (CSs) has increased due to increasing CS rates. A previous history of CS has been demonstrated to be an important risk factor for major complications in total laparoscopic hysterectomy. The aim here was to evaluate the major complications and safety of TLH in patients with previous CS.

Human mature milk zearalenone and deoxynivalenol levels in Turkey.

Introduction: Zearalenone (ZEA) and deoxynivalenol (DON) are toxic fungal secondary metabolites, found mainly in contaminated food, that are associated with serious health problems. It is important to identify undesirable toxins and metabolites that may be present in human milk. The aim of this study was to evaluate human milk ZEA and DON levels, total daily intake of ZEA and DON; and their possible relationship with maternal dietary habits.

The role of ADAMTS-2, collagen type-1, TIMP-3 and papilin levels of uterosacral and cardinal ligaments in the etiopathogenesis of pelvic organ prolapse among women without stress urinary incontinence.

Objective(s): To investigate the potential role of 'a disintegrin-like and metalloproteinase with thrombospondin type motifs-2 (ADAMTS-2), collagen type-1, tissue inhibitor of metalloproteinases-3 (TIMP-3) and papilin' levels in the uterosacral ligament (USL) and cardinal ligament (CL) of the uterus on the etiopathogenesis of pelvic organ prolapse (POP) among postmenopausal women without stress urinary incontinence (SUI).

Study Design: A total of 45 postmenopausal women, 22 diagnosed as POP stage III-IV and 23 age- and body mass index (BMI)-matched controls referred for hysterectomy due to POP or benign gynecological disease, respectively, were recruited prospectively for our study. The biopsies of the USL and CL were obtained during hysterectomy.

Serum RDW and MPV in Ankylosing Spondylitis: Can they show the disease activity?

Objective: To determine RDW and MPV levels in Ankylosing Spondylitis (AS) and to investigate their relations with disease activity.

Materials And Methods: 133 patients with AS (male: 80, female: 53) and age-sex matched 133 controls (male: 79, female: 54) were enrolled. Demographic data, disease activity scores, Complete Blood Count (CBC), Erythrocyte Sedimentation Rate (ESR) and C-Reactive Protein (CRP) levels were recorded.

Evaluation of subfoveal choroidal thickness in pregnant women using enhanced depth imaging optical coherence tomography.

Purpose: To evaluate the subfoveal choroidal thickness (SFCT) measured by enhanced depth imaging optical coherence tomography (EDI-OCT) in pregnant women.

Methods: In this prospective and cross-sectional study, 100 pregnant women and 100 age-matched nonpregnant women were enrolled. The SFCT was measured by EDI-OCT.

Effect of body posture on intraocular pressure and ocular perfusion pressure in nonglaucomatous pregnant women.

Purpose: To investigate the effect of different positions on the intraocular pressure (IOP) and ocular perfusion pressure (OPP) in nonglaucomatous pregnant.

Material And Methods: Thirty-one women in their third trimester of pregnancy were enrolled in this prospective and observational study. IOP in both eyes was measured with a Tono-pen® in the sitting position (Si-P), in the supine position (Su-P), in the right lateral decubitus position (R-LDP), and left lateral decubitus position (L-LDP).

Osseous metaplasia of the cervix and endometrium: a case of secondary infertility.

Objective: To report a case of osseous metaplasia of the cervix and endometrium as a cause of secondary infertility.

Design: Case report.

Setting: Istanbul Bakirkoy Women and Children Teaching and Research Hospital.

Persistent subumbilical discharge associated with actinomycosis caused by intrauterine contraceptive device: a case report.

Intrauterine devices are a very effective form of contraception used worldwide for more than 2 centuries. One of the serious, but rare, complications of intrauterine contraception is perforation through the uterine wall into the pelvic or abdominal cavity. In this report we describe, persistent periumbilical discharge associated actinomycosis caused by migration of a copper intrauterine contraceptive device.

Is lower socio-economic status a risk factor for Helicobacter pylori infection in pregnant women with hyperemesis gravidarum?

Background/aims: Lower socio-economic status was demonstrated to be a major risk factor for infection with H. pylori infection in the general population. The aim of the present study was to investigate any possible association between infection with H.