Implementation and Outcome of a Protocol-Based Treatment for Diabetic Ketoacidosis in a Tertiary Care Pediatric Emergency Department.

Background: The standardization of clinical practice in emergency treatment of diabetic ketoacidosis (DKA) is a prerequisite for improving patient care. For this purpose, a standardized DKA protocol incorporating a two-bag system has been implemented since January 2020 in our center.

Objectives: To assess the impact of the development and utilization of the standard treatment pathway for DKA on patient outcomes.

Lack of NAD(P)+ transhydrogenase activity in patients with primary adrenal insufficiency due to NNT variants.

Background: Pathogenic variants in the nicotinamide nucleotide transhydrogenase gene (NNT) are a rare cause of primary adrenal insufficiency (PAI), as well as functional impairment of the gonads.

Objective: Despite the description of different homozygous and compound heterozygous NNT variants in PAI patients, the extent to which the function and expression of the mature protein are compromised remains to be clarified.

Design: The activity and expression of mitochondrial NAD(P)+ transhydrogenase (NNT) were analyzed in blood samples obtained from patients diagnosed with PAI due to genetically confirmed variants of the NNT gene (n = 5), heterozygous carriers as their parents (n = 8), and healthy controls (n = 26).

Immunogenicity, safety and clinical outcomes of the SARS-CoV-2 BNT162b2 vaccine in adolescents with type 1 diabetes.

Introduction: The mRNA-based BNT162b2 (Pfizer-BioNTech) vaccine has been shown to elicit robust systemic immune response and confer substantial protection against the severe coronavirus disease (COVID-19), with a favorable safety profile in adolescents. However, no data exist regarding immunogenicity, reactogenicity and clinical outcomes of COVID-19 vaccines in adolescents with type 1 diabetes (T1D). In this prospective observational cohort study, we examined the humoral immune responses and side effects induced by the BNT162b2 vaccine, as well as, the rate and symptomatology of laboratory-confirmed COVID-19 vaccine breakthrough infections after completion of dual-dose BNT162b2 vaccination in adolescents with T1D and compared their data with those of healthy control adolescents.

Genotype of congenital adrenal hyperplasia patients with testicular adrenal rest tumor.

Unlabelled: Testicular adrenal rest tumor (TART) is one of the important complications that can cause infertility in male patients with congenital adrenal hyperplasia (CAH) and should therefore be diagnosed and treated at an early age. The factors that result in TART in CAH have not been completely understood. The aim of this study is to evaluate the genotype-phenotype correlation in CAH patients with TART.

Steroid Hormone Profiles and Molecular Diagnostic Tools in Pediatric Patients With non-CAH Primary Adrenal Insufficiency.

Context: There is a significant challenge of attributing specific diagnoses to patients with primary adrenal insufficiency of unknown etiology other than congenital adrenal hyperplasia (non-CAH PAI). Specific diagnoses per se may guide personalized treatment or may illuminate pathophysiology.

Objective: This work aimed to investigate the efficacy of steroid hormone profiles and high-throughput sequencing methods in establishing the etiology in non-CAH PAI of unknown origin.

Cranial MRI Abnormalities and Long-term Follow-up of the Lesions in 770 Girls With Central Precocious Puberty.

Context: Central precocious puberty (CPP) may arise from central nervous system (CNS) lesions in a few affected girls. Recently, the incidence of girls with CPP has increased mostly in 6-8 year olds, in whom the necessity of magnetic resonance imaging (MRI) is debated.

Objective: To investigate the frequency, long-term outcome and potential predictors of CNS lesions in a large cohort of girls with CPP.

Clinical features of the diabetes eating problem survey-revised Turkish version in children and adolescent with type 1 diabetes.

Objectives We aim to delineate clinical characteristics that place individuals with type 1 diabetes (T1DM) at risk of developing eating problems by using Turkish version of diabetes eating problem survey-revised (DEPS-R). Methods The patients aged 9-18 years with T1DM who came to the pediatric endocrine outpatient clinic for control between February and December 2019 completed Turkish version of DEPS-R. Clinical and laboratory findings were obtained from patient files.

Management of Thyrotoxicosis in Children and Adolescents: A Turkish Multi-center Experience.

Objective: To determine the demographic and biochemical features of childhood and juvenile thyrotoxicosis and treatment outcome.

Methods: We reviewed the records of children from 22 centers in Turkey who were diagnosed with thyrotoxicosis between 2007 to 2017.

Results: A total of 503 children had been diagnosed with thyrotoxicosis at the centers during the study period.

Cardiac MRI and 3D contrast-enhanced MR angiography in pediatric and young adult patients with Turner syndrome.

Background/aim: This study aimed to describe the spectrum and frequency of cardiovascular abnormalities in pediatric and young adult patients with Turner syndrome (TS) using cardiac MRI and MR angiography.

Materials And Methods: This prospective study consisted of 47 female patients of pediatric age and young adults with a karyotypically confirmed diagnosis of TS. All patients underwent cardiac MRI and contrast-enhanced MR angiography.

Maturity onset diabetes of youth (MODY) in Turkish children: sequence analysis of 11 causative genes by next generation sequencing.

Background: Maturity-onset diabetes of the youth (MODY), is a genetically and clinically heterogeneous group of diseasesand is often misdiagnosed as type 1 or type 2 diabetes. The aim of this study is to investigate both novel and proven mutations of 11 MODY genes in Turkish children by using targeted next generation sequencing.

Methods: A panel of 11 MODY genes were screened in 43 children with MODY diagnosed by clinical criterias.

Evaluating the Efficacy of Treatment with a GnRH Analogue in Patients with Central Precocious Puberty.

Objective. GnRH analogues (GnRHa) are used in the treatment of central precocious puberty (CPP). The purpose of this study was to evaluate the efficacy of treatment with a GnRHa (leuprolide acetate) in patients with CPP.

Effects of GnRH analogue treatment on anterior pituitary hormones in children with central precocious puberty.

Introduction And Purpose: This study aims to investigate the effect of Gonadotropin-releasing hormone analogues (GnRHa) treatment on anterior pituitary hormones in female children with central precocious puberty (CPP).

Subjects And Method: There were 62 female children who had been diagnosed with CPP and received GnRHa (Leuprolide acetate, 3.75 mg intramuscular/subcutaneous/28 days) included in the study.

Approach to thyroid nodules in children and adolescents.

Thyroid nodule prevalence is about 1.8% in healthy children; however, malignancy frequency is higher than in adults. Approximately 26.

An uncommon cause of hypoglycemia: insulin autoimmune syndrome.

Background: Insulin autoimmune syndrome (IAS) is a condition characterized by hypoglycemia associated with the presence of autoantibodies to insulin in patients who have not been injected with insulin.

Case Report: A female patient (aged 16 years and 3 months) presented with the complaint of being overweight. Physical examination revealed a body weight of 78.

Evaluation of asymmetric dimethylarginine (ADMA) levels in children with growth hormone deficiency.

Objective: To investigate serum asymmetric dimethylarginine (ADMA) levels in children with isolated growth hormone deficiency (GHD) and to determine the effect of GH replacement therapy on these levels.

Methods: 31 patients diagnosed with isolated GHD and 29 age-and sex-matched healthy children were enrolled in the study. Height, weight and waist circumference were measured in all subjects.

A girl with steroid cell ovarian tumor misdiagnosed as non-classical congenital adrenal hyperplasia.

Ovarian steroid cell tumors are rarely encountered in prepubertal girls. The majority of these tumors produce hormones, testosterone being the leading one. These tumors may either coexist with or imitate congenital adrenal hyperplasia (CAH).

Increased central corneal thickness in patients with Turner syndrome.

Purpose: To evaluate central corneal thickness (CCT) values in patients with Turner syndrome (TS) and compare these values with healthy subjects.

Methods: A total of 31 subjects with TS and 67 age- and sex-matched healthy subjects made up the study and control group, respectively. The CCT values were measured by an ultrasound pachymeter in this cross-sectional prospective study.

Evaluation of bone mineral density in children with type 1 diabetes mellitus.

Background: Factors such as vascular/neurological mechanisms, poor glycemic control, abnormalities in vitamin D/calcium, secondary hyperparathyroidism, and hypercalciuria have been blamed for the unfavorable effects of type 1 diabetes mellitus (type 1 DM) on bone health. In this present study, we aimed to evaluate the frequency of low bone mineral density (BMD) in children with type 1 DM.

Method: Among 100 type 1 DM patients, a 25-hydroxy vitamin D level of <10 ng/mL was accepted as vitamin D deficiency and the level of 10-20 ng/dL was accepted as vitamin D insufficiency.

Long-term follow-up of Cushing's disease:a case report.

Cushing's disease is a condition in which hypercortisolism develops due to excessive hypophyseal adrenocorticotropic hormone production. It is rare in childhood. In this paper, we report the case of a 10-year-old male patient with hypophyseal microadenoma-related Cushing's disease who presented with obesity and was found to show poor height growth at follow-up.

Hyperinsulinemic hypoglycemia: experience in a series of 17 cases.

Objective: Hyperinsulinemic hypoglycemia (HIH) is a genetically heterogeneous disorder with both familial and sporadic variants. Patients with HIH may present during the neonatal period, infancy, or childhood and may show transient, prolonged, and persistent features. In this study, we aimed to discuss our experience with HIH patients, based on a series of 17 patients.

Diabetes mellitus with Laron syndrome: case report.

There are different opinions concerning changes in glucose metabolism in patients with Laron syndrome. In this paper we discuss the treatment results of our patient with Laron syndrome who developed diabetes during late adolescence. A 19-year-old boy with Laron syndrome was referred to our clinic for follow-up.

A rare combination: congenital adrenal hyperplasia due to 21 hydroxylase deficiency and Turner syndrome.

A combination of Turner syndrome (TS) and classical congenital adrenal hyperplasia (CAH) is rare. A one-day-old newborn was referred to our hospital with ambiguous genitalia. The parents were third-degree relatives.

Assessment of the 21-hydroxylase deficiency and the adrenal functions in young females with Turner syndrome.

There are few reports of an association between Turner syndrome (TS) and 21-hydroxylase deficiency. However, this association is more frequent in some populations. The aim of this study was to evaluate the incidence of 21-hydroxylase deficiency in patients with TS in our population.

Assessment of the knowledge of diabetes mellitus among school teachers within the scope of the managing diabetes at school program.

Objective: Training teachers and education professionals on diabetes is crucial for full-time monitoring of diabetic children in schools. The objective of this study was to assess the knowledge on diabetes in a group of school teachers in Turkey.

Methods: Between November 2010 and November 2011, 1054 teachers from three regions of Ankara were given a questionnaire to assess their knowledge on diabetes.

A pediatric Conn syndrome case.

Conn syndrome, which is rarely encountered in children, is characterized by increased aldosterone, low renin level, and arterial hypertension. Severe complications, such as impaired vascular smooth muscle function secondary to increased aldosterone, endothelial dysfunction, deterioration of left ventricular functions, acute effects on the cardiovascular system, and proteinuria, may be observed. We present a case of primary aldosteronism in a patient who has been followed up for approximately 2 years.