Isolation, identification, and in vitro probiotic characterization of forty novel Bifidobacterium strains from neonatal feces in Erzurum province, Türkiye.

Background: Neonatal feces are one of the most important sources for probiotic isolation. The purpose of this study was the isolation and identification of Bifidobacterium spp. from neonatal feces and the evaluation of in vitro probiotic properties of strains including safety tests.

Seronegative autoimmune hepatitis in childhood.

Comprehensive guidelines on seropositive autoimmune hepatitis have been published for both adults and children, although these guidelines comprise only limited knowledge about seronegative autoimmune hepatitis. Autoimmune hepatitis presents as an acute or chronic progressive disease and poor outcomes are inevitable if left untreated. The absence of autoantibody positivity, hypergammaglobulinemia and lack of comprehensive algorithms makes seronegative autoimmune hepatitis a mysterious disease.

Biliary atresia and congenital disorders of the extrahepatic bile ducts.

Biliary atresia (BA) and choledochal cysts are diseases of the intrahepatic and extrahepatic biliary tree. While their exact etiopathogeneses are not known, they should be treated promptly due to the potential for irreversible parenchymal liver disease. A diagnosis of BA may be easy or complicated, but should not be delayed.

Clinical Features of Children with COVID-19 Infection: Single-Center Experience.

Objective: Compared to adult studies, there are few epidemiological and clinical reports on coronavirus disease 2019 in children. We aimed to present the demographic, epidemiological, and clinical findings of hospitalized pediatric coronavirus disease 2019 patients.

Materials And Methods: Patients aged 0-18 years who were hospitalized between March and July 2020 due to severe acute respiratory syndrome coronavirus 2 infection were evaluated retrospectively.

Acute-on-chronic liver failure in children.

Although various complex definitions of acute-on-chronic liver failure (ACLF) have been suggested in relation to adult patients, there is currently no universal definition of the syndrome in pediatric patients. In simplified terms, ACLF is characterized by the acute deterioration of the liver functions due to the effects of a precipitating factor on the basis of a chronic liver disease. Acute events and underlying liver diseases are very different in children from those seen in adults.

Acute-on-Chronic Liver Failure in Children: A Single-Center Experience.

Objectives: Acute-on-chronic liver failure and its outcomes have not yet been evaluated in detail in children. We aimed to evaluate the etiology, acute events, and prognostic factors of acute-on-chronic liver failure in children.

Materials And Methods: Pediatric patients (age 2-18 years) diagnosed with acute-on-chronic liver failure between April 2014 and April 2020 were evaluated retrospectively.

A Novel Mutation of Gene in a Case of Wilson Disease.

Wilson disease (WD) (OMIM# 277900) is an autosomal recessive inherited disorder characterized by excess copper (Cu) storage in different human tissues, such as the brain, liver, and the corneas of the eyes. It is a rare disorder that occurs in approximately 1 in 30,000 individuals. The clinical presentations of WD are highly varied, primarily consisting of hepatic and neurological conditions.

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.

Altered von Willebrand Factor and ADAMTS13 Levels in Children With Cirrhosis and Extrahepatic Portal Hypertension.

Background/aim: This study was concerned with whether vWF (von Willebrand factor) and a disintegrin and metalloprotease with a thrombospondin type 1 motif, member 13 (ADAMTS13) has altered in patients with cirrhosis and extrahepatic portal hypertension (EPH). We aimed to investigate changes to vWF and ADAMTS13 in children with cirrhosis and EPH.

Patients And Methods: This study was conducted between January and October 2019 with both cirrhosis and EPH patients and with healthy volunteers.

Liver Involvement in Children with Alpha-1 Antitrypsin Deficiency: A Multicenter Study.

Purpose: Alpha-1 antitrypsin deficiency (A1ATD) in one of the most common genetic causes of liver disease in children. We aimed to analyze the clinical characteristics and outcomes of patients with A1ATD.

Methods: This study included patients with A1ATD from five pediatric hepatology units.

Analysis of correlation between liver fat fraction and AST and ALT levels in overweight and obese children by using new magnetic resonance imaging technique.

Background/aims: Proton density fat fraction (PDFF) magnetic resonance (MR) imaging can be a useful technique for volumetric measurements of liver fat. The purpose of our study was to evaluate the correlation between liver fat fraction (LFF) and aspartate aminotransferase (AST) and alanine aminotransferase (ALT) levels in children who are overweight and obese.

Materials And Methods: Overall, 25 children, aged 9-17 years, were included.

The Frequency of Lysosomal Acid Lipase Deficiency in Children With Unexplained Liver Disease.

Objectives: Evidence suggests that lysosomal acid lipase deficiency (LAL-D) is often underdiagnosed because symptoms may be nonspecific. We aimed to investigate the prevalence of LAL-D in children with unexplained liver disease and to identify demographic and clinical features with a prospective, multicenter, cross-sectional study.

Methods: Patients (aged 3 months-18 years) who had unexplained transaminase elevation, unexplained hepatomegaly or hepatosplenomegaly, obesity-unrelated liver steatosis, biopsy-proven cryptogenic fibrosis and cirrhosis, or liver transplantation for cryptogenic cirrhosis were enrolled.

The Liver Fat Fraction and Abdominal Subcutaneous and Visceral Fat Volume Distribution in Normal-Weight, Overweight, and Obese Children Using a New Magnetic Resonance Imaging Technique.

Objectives: The purpose of this study was to evaluate liver fat fraction and subcutaneous and visceral fat volumes using new magnetic resonance imaging in normal-weight, overweight, and obese children.

Methods: Patients at below the 85th percentile of body mass index (BMI) z score (5/25 patients) were assigned to the normal-weight group; patients between 85th and 95th percentile of BMI z score (9/25 patients) were assigned to the overweight group, and patients above the 95th percentile of BMI z score (11/25 patients) were assigned to the obese group. Liver fat fraction and subcutaneous and visceral fat volumes were measured on 3-dimensional volume measurement workstation.

Bone mineral density and vitamin K status in children with celiac disease: Is there a relation?

Background/aims: To investigate bone mineral density (BMD) in children with celiac disease (CD) and to evaluate the association between vitamin K levels and osteoporosis.

Materials And Methods: Children with CD and age- and sex-matched healthy control subjects were prospectively included in the study. BMD was measured, and serum anti-tissue transglutaminase IgA, ferritin, folate, vitamin B12, 25-hydroxy vitamin D and K2, calcium, phosphate, alkaline phosphatase, and parathormone were assayed in all subjects.

Childhood chronic gastritis and duodenitis: Role of altered sensory neuromediators.

Aim: To investigate the roles of the neuropeptides vasoactive intestinal peptide (VIP), substance P (SP), and calcitonin gene-related peptide (CGRP) in chronic gastritis and duodenitis in children.

Methods: Biopsy samples from the gastric and duodenal mucosa of 52 patients and 30 control subjects were obtained. Samples were taken for pathological examination, immunohistochemical staining, enzyme activity measurements and quantitative measurements of tissue peptide levels.

Total oxidant status, total antioxidant capacity and ischemia modified albumin levels in children with celiac disease.

In our study, we aimed to investigate ischemia modified albumin (IMA) as an oxidative stress marker, as well as other oxidant and antioxidant markers that have not been evaluated in children with celiac disease. A total of 37 pediatric patients who were diagnosed with celiac disease (CD) and 29 healthy children were enrolled in this prospective study. We evaluated the IMA, total oxidant status, total antioxidant capacity, sulfhydryl, and advanced oxidation protein products in all of the subjects.

A very rare cause of acute pancreatitis: Berardinelli-Seip congenital lipodystrophy.

Pancreatitis is among rare diseases in pediatrics clinics. It is usually presented with a sign of underlying systemic disease. Berardinelli-Seip congenital lipodystrophy (BSCL) is a very rare disease characterized by near absence of adipose tissue resulting in apparent muscle hypertrophy from birth or early infancy associated with severe insulin resistance.

The role of Bifidobacterium lactis B94 plus inulin in the treatment of acute infectious diarrhea in children.

Background/aims: In contrast to many other studies of probiotic species, the number of publications evaluating Bifidobacterium lactis and its combinations with prebiotics as treatments for acute infectious diarrhea is limited. We investigated the synbiotic effects of B. lactis B94 plus inulin on acute infectious diarrhea.

Microvillus inclusion disease associated with necrotizing enterocolitis in a premature infant.

Microvillus inclusion disease is one of the congenital diarrheal disorders characterized by the appearance of inclusion bodies on the intestinal epithelium. To date there are a few cases and also a few other associated finding reports related to this life-threatening disease in literature. In this report, we present a premature infant with microvillus inclusion disease that was associated with necrotizing enterocolitis.

Reliable and powerful laboratory markers of cobalamin deficiency in the newborn: plasma and urinary methylmalonic acid.

Background: Diagnosing cobalamin deficiency is critical, given the high prevalence of cobalamin deficiency particularly in developing countries. Measuring serum cobalamin levels is of limited diagnostic sensitivity, in other words its specificity and sensitivity are low. The present study investigated the changes in the levels of metabolic markers - plasma homocysteine, plasma methylmalonic acid (MMA) and urinary MMA - of cobalamin metabolism.

A rare outcome of iron deficiency and pica: Rapunzel syndrome in a 5-year-old child iron deficiency and pica.

Bezoar is defined as the accumulation of organic or nonbiological substances inside the gastrointestinal system. Trichobezoars are the most frequently detected ones and are mostly present in patients with neuropsychiatric disorders. The continuance of the trichobezoar tail-shaped extension over the duodenum and jejunum is described in Rapunzel syndrome.