Factors associated with eosinophilic esophagitis in an urban, tertiary care pediatric aerodigestive population undergoing triple endoscopy.

Background: Children with aerodigestive dysfunction often undergo triple endoscopy (flexible bronchoscopy, rigid direct laryngoscopy and bronchoscopy, and esophagogastroduodenoscopy) for diagnostic evaluation as well as screening prior to airway reconstruction. Prevalence and risk factors for eosinophilic esophagitis (EoE) in this population are poorly understood.

Methods: A retrospective chart review was performed for pediatric patients, aged 0-21 years, who received a triple endoscopy with biopsy from January 1, 2015, to December 31, 2019, at the Children's Hospital at Montefiore (CHAM).

Diagnosis and management of children with Blue Rubber Bleb Nevus Syndrome: A multi-center case series.

Background: Blue Rubber Bleb Nevus Syndrome (BRBNS) is a rare, severe, sporadically occurring disorder characterized by multiple venous malformations.

Aims: To present and analyze a case series of pediatric patients with BRBNS and to describe diagnostic approaches and management options applied.

Patients And Methods: Multicenter, retrospective study, evaluating the diagnosis and management of children with BRBNS.

Interleukin 1β Mediates Intestinal Inflammation in Mice and Patients With Interleukin 10 Receptor Deficiency.

Interleukin 10 receptor (IL10R)-deficient mice develop spontaneous colitis and, similarly, patients with loss-of-function mutations in IL10R develop severe infant-onset inflammatory bowel disease. Loss of IL10R signaling in mouse and human macrophages is associated with increased production of interleukin 1β. We demonstrated that innate immune production of IL1β mediates colitis in IL10R-deficient mice.

Disseminated langerhans cell histiocytosis presenting as cholestatic jaundice.

Langerhans cell histiocytosis (LCH) is a disorder associated with proliferation of Langerhans cells in various organs. LCH secondary to multisystem involvement can present in a variety of ways. Because of its infiltrative nature, LCH can involve the skin, lymph nodes, the lung or the liver.

Single-center experience with 1-step low-profile percutaneous endoscopic gastrostomy in children.

Objectives: The 1-step low-profile percutaneous endoscopic gastrostomy (1-step PEG) uses a single procedure that allows immediate use of a low-profile device. The aim of the present study was to provide our experience with this device and to analyze complications and outcomes after the initial placement.

Methods: We performed a retrospective chart review of pediatric patients with 1-step PEG placement done by our pediatric gastroenterologists between 2006 and June 2011.

Developmental expression of Eph and ephrin family genes in mammalian small intestine.

Background: Eph receptor tyrosine kinases EphB2 and EphB3, and ephrin-B1 ligand play a critical role in regulating small intestinal epithelial cell migration. Although well studied in developing brain, the expression pattern of Ephs/ephrins has not been delineated in the developing small intestine.

Aims: To examine the gene expression of all known members of Ephs/ephrins during development of mouse small intestine.

Hereditary hemorrhagic telangiectasia and juvenile polyposis: an overlap of syndromes.

Hereditary hemorrhagic telangiectasia (HHT) (Osler-Weber-Rendu syndrome) is a syndrome characterized by multiorgan telangiectases and arteriovenous malformations. A subset of patients with a mutation in the MADH4 gene on chromosome 18 exhibits an overlapping syndrome of HHT and juvenile polyposis (JPS). We present one such family.

Comparative phenotypic and CARD15 mutational analysis among African American, Hispanic, and White children with Crohn's disease.

Background: Despite a large body of literature on the subject of Crohn's disease (CD), very little information is available on racial/ethnic differences related to disease presentation, clinical course, and genetics. The first identified CD susceptibility gene, CARD15, seems to be present in up to 40% of white children with CD. However, the frequency of this gene among patients with CD of other racial/ethnic groups in the United States is not known.