Whole Foods Introduction Associated With Symptomatic Anastomotic Ulceration in Children With Short Bowel Syndrome.

Objectives: Anastomotic ulceration (AU) is a rare but life-threatening complication of pediatric short bowel syndrome (SBS). AUs may be challenging to detect and refractory to treatment. This study aimed to identify features associated with symptomatic bleeding AUs in children with SBS and factors that may impact resolution of bleeding.

Dietary fiber-based regulation of bile salt hydrolase activity in the gut microbiota and its relevance to human disease.

Complications of short bowel syndrome (SBS) include malabsorption and bacterial overgrowth, requiring prolonged dependence on parenteral nutrition (PN). We hypothesized that the intolerance of whole food in some SBS patients might be due to the effect of dietary fiber on the gut microbiome. Shotgun metagenomic sequencing and targeted metabolomics were performed using biospecimens collected from 55 children with SBS and a murine dietary fiber model.

Impact of Pancreatic Enzymes on Enteral Fat and Nitrogen Absorption in Short Bowel Syndrome.

Objectives: Patients with short bowel syndrome (SBS) can have a high morbidity rate. To minimize morbidity, enteral autonomy is the primary goal in clinical management of patients with SBS. This is often difficult to achieve because of significant malabsorption.

Pathologic lower extremity fractures in children with Alagille syndrome.

Objectives: : In this retrospective study, we aimed to determine the incidence and distribution of fractures in patients with Alagille syndrome, 1 of the leading inherited causes of pediatric cholestatic liver disease.

Materials And Methods: : Surveys regarding growth, nutrition, and organ involvement were distributed to patient families in the Alagille Syndrome Alliance of the Children's Hospital of Philadelphia research database. Patients with a history of fracture were identified by their response to 1 question, and details characterizing each patient's medical, growth, and fracture history were obtained through chart review and telephone contact.

Lateral sinus thrombosis as a complication of otitis media: 10-year experience at the children's hospital of Philadelphia.

Objectives: Lateral sinus thrombosis is a rare intracranial complication of otitis media that is traditionally described in countries with poor access to medical care. Our goal was to describe the clinical presentation, management, and outcome of patients diagnosed with lateral sinus thrombosis in a US tertiary care center and to highlight the clinically relevant differences in presentation between these patients and those described in previous reports.

Patients And Methods: The medical charts of 13 patients diagnosed with otogenic lateral sinus thrombosis were reviewed.

Bile duct proliferation in Jag1/fringe heterozygous mice identifies candidate modifiers of the Alagille syndrome hepatic phenotype.

Unlabelled: Alagille syndrome (AGS) is a heterogeneous developmental disorder associated with bile duct paucity and various organ anomalies. The syndrome is caused by mutations in JAG1, which encodes a ligand in the Notch signaling pathway, in the majority of cases and mutations in the NOTCH2 receptor gene in less than 1% of patients. Although a wide array of JAG1 mutations have been identified in the AGS population, these mutational variants have not accounted for the wide phenotypic variability observed in patients with this syndrome.

Transseptal suture to secure middle meatal spacers.

Middle meatal spacers are used by many sinus surgeons to aid postoperative care. Aspiration of a spacer is a concern. We demonstrate a novel method of securing spacers with a transseptal suture to prevent aspiration.

Craniofacial resection of advanced juvenile nasopharyngeal angiofibroma.

Objective: To describe the results of a craniofacial approach to resection of stage IIIB juvenile nasopharyngeal angiofibroma, performed by an integrated skull base surgical team.

Design: A retrospective case-series review was conducted with postoperative follow-up ranging from 28 to 63 months.

Setting: Operations were performed at a tertiary medical center.