Evaluation of Cross-Reactivity Between Penicillins and Cephalosporins in Children with a History of Cephalosporin Allergy.

Objectives: The cross-reactivity problem between cephalosporins and penicillins has mainly been evaluated in the context of patients allergic to penicillins. However, we have little data regarding the opposite aspect of the problem, i.e.

The Role of Adenovirus in Hepatitis of Unknown Etiology Among Children in Turkey, July 2022 to January 2023.

Background: In the first half of 2022, the increase in cases of severe acute hepatitis in children with no epidemiological link and unknown cause has aroused concern worldwide. In this study, we aimed to determine the frequency of adenovirus in children with hepatitis of unknown origin in Turkey.

Methods: In this study, which was conducted with the participation of 16 centers from Turkey, between July 2022 and January 2023, 36 pediatric patients under the age of 18 who met the definition of acute hepatitis were included.

Growth, tolerance and safety outcomes with use of an extensively hydrolyzed casein-based formula in infants with cow's milk protein allergy.

Objective: To evaluate growth, tolerance and safety outcomes with use of an extensively hydrolyzed casein-based formula (eHCF) in infants with cow's milk protein allergy (CMPA).

Methods: A total of 226 infants (mean ± SD age: 106.5 ± 39.

Long-Term Follow-Up and Outcome of Pediatric Acute Pancreatitis: A Multicenter Study.

Objective: Over the past decades, the incidence of acute pancreatitis is increasing, but the progression of acute recurrent pancreatitis and chronic pancreatitis is still not well documented in children. The aim of this multicenter study is to delineate the changes that occur in a certain time period in the course of childhood pancreatitis.

Materials And Methods: The data of consecutive patients hospitalized with acute pancreatitis between 2010 and 2017 in 4 different pediatric gastroenterology units were reviewed.

Clinical and Genetic Spectrum of Myotonia Congenita in Turkish Children.

Background: Myotonia congenita is the most common form of nondystrophic myotonia and is caused by Mendelian inherited mutations in the CLCN1 gene encoding the voltage-gated chloride channel of skeletal muscle.

Objective: The study aimed to describe the clinical and genetic spectrum of Myotonia congenita in a large pediatric cohort.

Methods: Demographic, genetic, and clinical data of the patients aged under 18 years at time of first clinical attendance from 11 centers in different geographical regions of Türkiye were retrospectively investigated.

Nutritional characteristic of children with inflammatory bowel disease in the nationwide inflammatory bowel disease registry from the Mediterranean region.

Background/objectives: We analyzed the nationwide pediatric inflammatory bowel disease (PIBD) registry (1998-2016), to evaluate the nutritional status at the time of diagnosis.

Subjects/methods: Nine types of nutritional status by the combination of weight-for-length (<2 years)/body mass index (>2 years) and length/height-for-age with three categories (<-2, -2 to 2, and >2 SD) were described. Malnutrition was defined by WHO criteria.

Clinical and genetic spectrum of a large cohort of patients with δ-sarcoglycan muscular dystrophy.

Sarcoglycanopathies include four subtypes of autosomal recessive limb-girdle muscular dystrophies (LGMDR3, LGMDR4, LGMDR5 and LGMDR6) that are caused, respectively, by mutations in the SGCA, SGCB, SGCG and SGCD genes. Delta-sarcoglycanopathy (LGMDR6) is the least frequent and is considered an ultra-rare disease. Our aim was to characterize the clinical and genetic spectrum of a large international cohort of LGMDR6 patients and to investigate whether or not genetic or protein expression data could predict a disease's severity.

The frequency of and factors affecting functional gastrointestinal disorders in infants that presented to tertiary care hospitals.

This study aimed to determine the prevalence of infantile functional gastrointestinal disorders (FGIDs) based on Rome IV diagnostic criteria, and to determine the associated patient demographic and nutritional characteristics. A total of 2383 infants aged 1-12 months which were evaluated by 28 general pediatricians and pediatric gastroenterologists on the same day at nine tertiary care hospitals around Istanbul, Turkey, between November 2017 and March 2018, were included in the study. Patients included consulted the pediatric outpatient clinics because of any complaints, but not for vaccines and/or routine well child follow-ups as this is not part of the activities in the tertiary care hospitals.

A Rare Cause of Autism Spectrum Disorder: Megaconial Muscular Dystrophy.

Megaconial congenital muscular dystrophy (OMIM 602541) is defined by early-onset hypotonia, mildly elevated serum creatine kinase (CK) levels, muscle wasting, cardiomyopathy, psychomotor developmental delay and intellectual disability. The disease is caused by loss-of-function mutations in Choline kinase beta gene (CHKB) and has specific muscle biopsy findings. Here we investigate two patients with weakness of proximal muscles and generalized muscle atrophy, skin changes, agressiveness, social communication and empathy difficulties.

Broadly effective metabolic and immune recovery with C5 inhibition in CHAPLE disease.

Complement hyperactivation, angiopathic thrombosis and protein-losing enteropathy (CHAPLE disease) is a lethal disease caused by genetic loss of the complement regulatory protein CD55, leading to overactivation of complement and innate immunity together with immunodeficiency due to immunoglobulin wasting in the intestine. We report in vivo human data accumulated using the complement C5 inhibitor eculizumab for the medical treatment of patients with CHAPLE disease. We observed cessation of gastrointestinal pathology together with restoration of normal immunity and metabolism.

Assessment of Hearing Function in Children with Inflammatory Bowel Disease.

Objectives: Inflammatory bowel disease (IBD), a chronic pathology that affects many organ systems, appears after dysregulated immune response in genetically predisposed patients. Inner organ involvement has been shown in various autoimmune diseases because of its immunosensitivity. In this study, we aimed at showing sensorineural hearing loss (SNHL) as a result of possible subclinical inflammation in patients with IBD during the remission period.

Sublingual Atropine Sulfate Use for Sialorrhea in Pediatric Patients.

Sialorrhea is a frequent problem and may lead to aspiration in patients with swallowing dysfunction. We aimed to assess the effectiveness and safety of sublingual atropine sulfate treatment in pediatric patients with sialorrhea. The medical records of patients who had received sublingual atropine sulfate between January 2015 and January 2016 were reviewed retrospectively.

Effects of Adrenocorticotropic Hormone Treatment on Heart Muscle in Patients with Infantile Spasm.

Background and aim Infantile spasm (IS) is a common epileptic syndrome of childhood epilepsies. The most effective treatment for IS is adrenocorticotropic hormone (ACTH). We hypothesized that ACTH treatment might change myocardial systolic and diastolic performance and cause cardiovascular side effects.

Helicobacter pylori infection in children with celiac disease: Multi-center, cross-sectional study.

Background: An inverse association has been suggested between celiac disease (CD) and Helicobacter pylori (Hp) infection in children; however, there are inconsistent data. The purpose of this multi-center study is to evaluate the association between Hp and CD in childhood.

Methods: Children who underwent endoscopy between July 2016 and November 2017 in four pediatric gastroenterology centers were included in the study.

Evaluation of autonomic nervous system functions in frame of heart rate variability in children with inflammatory bowel disease in remission.

Aghdasi-Bornaun H, Kutluk G, Keskindemirci G, Öztarhan K, Dedeoğlu R, Yılmaz N, Tosun Ö. Evaluation of autonomic nervous system functions in frame of heart rate variability in children with inflammatory bowel disease in remission. Turk J Pediatr 2018; 60: 407-414.

HAX-1 deficiency: Characteristics of five cases including an asymptomatic patient.

Background: Mutations in the HAX-1 gene cause an autosomal recessive form of severe congenital neutropenia (SCN), which particularly manifests with recurrent skin, lung and deep tissue infections from the first few months of life.

Objective: We retrospectively evaluated the clinical and laboratory findings of the patients diagnosed with SCN carrying HAX1 gene mutations.

Methods: A total of five patients with SCN, carrying a HAX1 gene mutation, were evaluated in terms of clinical and laboratory findings.

Sequential therapy versus standard triple therapy for Helicobacter pylori eradication in children: any advantage in clarithromycin-resistant strains?

Objective: There has been a marked decrease in the eradication rates of Helicobacter pylori infection with standard triple therapy worldwide. Hence, sequential therapy has gained attention as a promising treatment during the last few years. This study was carried out to compare the efficacy of sequential versus standard triple therapy in the context of clarithromycin (CLA) resistance.

Current diagnosis and management of simple hepatic cysts detected prenatally and postnatally.

Purpose: The optimal management of simple hepatic cysts (SHCs) remains controversial. A retrospective review was carried out to evaluate the clinical presentation and surgical consideration of children with SHCs and outcome of management at our institution.

Methods: We performed a retrospective review of all patients diagnosed with SHCs between January 1993 and May 2013.

What is the diagnostic utility of endoscopic scoring systems in children?

Background/aims: The aim of this study was to evaluate the consistency of the Savary-Miller, the Hetzel-Dent and the Los Angeles endoscopic classification systems and to compare them with the esophageal histopathology in children.

Material And Methods: Children between the ages of 5-17 years who underwent esophagogastroduodenoscopy were included in the study. The endoscopic reports and the still images of the esophagus were reclassified by the same gastroenterologist according to the Savary-Miller, Hetzel- Dent and Los Angeles scoring systems.

Niemann - Pick disease associated with hemophagocytic syndrome.

Hemophagocytic lymphohistiocytosis (HLH) is a disease characterized by phagocytosis of blood cells by macrophages within the lymphoreticular tissue. It can develop secondary to some diseases or be familial as a result of genetic mutations. Niemann-Pick disease (NPD) is a very rare lipid storage disease.

Penicillin sensitivity among hospital nurses without a history of penicillin allergy.

Aim: This paper reports a study to document the prevalence of penicillin sensitivity among nurses who had no previous history of sensitivity and worked in an inner city hospital in Turkey.

Background: Frequent and prolonged exposure to any drug including penicillin may increase the risk of hypersensitivity to these drugs. Local administration of drugs increases the likelihood of sensitization.

Nutritional vitamin B12 deficiency in hospitalized young children.

The authors sought to determine prevalence, social, economic, and dietary patterns of young children (n = 20) identified as having vitamin B12 deficiency anemia after admission to their hospital in the last 3 years. The diagnosis of vitamin B12 deficiency was based on symptoms and clinical findings, findings on peripheral blood films and bone marrow aspirates, and serum levels of vitamin B12. The children had been exclusively breast-fed without any animal food supplementation.