Publications by authors named "Joseph A Picoraro"

Surgical intervention is often indicated in pediatric inflammatory bowel disease (IBD) for medically refractory disease or complications of severe disease. Specifically, surgical intervention via ileal diversion allows for fecal flow to be redirected away from diseased distal bowel and through an ileostomy. It is utilized in patients who have medically refractory colitis, severe perianal disease, or irreversible bowel damage.

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Background: Restorative proctocolectomy and IPAA have become the surgical procedure of choice in pediatric patients with medically refractory colitis or familial adenomatous polyposis.

Objective: This systematic review aims to assess the diagnosis and treatment of pouch disorders in pediatric patients who undergo IPAA.

Data Sources: A literature search was performed using MEDLINE, Google Scholar, and Embase for all publications describing outcomes of pediatric IPAA.

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Children who undergo ileal pouch anal anastomosis (IPAA) surgery for refractory ulcerative colitis (UC) may ultimately develop a Crohn's disease (CD) phenotype. This de novo CD is open to broad interpretation and misattribution, and its manifestation in children is poorly understood. The surgically altered environment of the ileal pouch is at risk of a spectrum of ileal pouch disorders, which have limited description in children.

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Endoscopic characterization of pediatric inflammatory bowel disease (IBD) has developed in accordance with advances in treatment and improved understanding of disease progression and complications. Reliable and consistent endoscopic reporting practices and tools continue to evolve. The roles of endoscopic ultrasonography, capsule endoscopy, and deep enteroscopy in the care of children and adolescents with IBD are beginning to be clarified.

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Fistula in inflammatory bowel disease (IBD) is a well-known yet poorly understood phenotype. Pathophysiology is largely based on the activation of the epithelial-mesenchymal transition (EMT); however, interactions with the microbiome, genetics, mechanical stress and the presence of stricturing disease, and surgical complications play a role. Perianal penetrating disease represents a more severe phenotype in IBD.

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Food additives in general, and emulsifiers in particular, are considered to be important dietary components with a potential to harm the intestine, in part by promoting intestinal inflammation. There is inadequate objective information about the specific nature and the magnitude of the problem.The Food and Drug Administration (FDA) has recognized approximately 450 items added to our foods as being generally regarded as safe and has placed them on a generally regarded as safe (GRAS) list.

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Background: Endoscopic mucosal healing is the gold standard for evaluating Crohn's disease (CD) treatment efficacy. Standard endoscopic indices are not routinely used in clinical practice, limiting the quality of retrospective research. A method for retrospectively quantifying mucosal activity from documentation is needed.

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Pouchitis, Crohn's disease of the pouch, cuffitis, polyps, and extraintestinal manifestations of inflammatory bowel disease are common inflammatory disorders of the ileal pouch. Acute pouchitis is treated with oral antibiotics and chronic pouchitis often requires anti-inflammatory therapy, including the use of biologics. Aetiological factors for secondary pouchitis should be evaluated and managed accordingly.

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Restorative proctocolectomy with ileal pouch-anal anastomosis is an option for most patients with ulcerative colitis or familial adenomatous polyposis who require colectomy. Although the construction of an ileal pouch substantially improves patients' health-related quality of life, the surgery is, directly or indirectly, associated with various structural, inflammatory, and functional adverse sequelae. Furthermore, the surgical procedure does not completely abolish the risk for neoplasia.

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Article Synopsis
  • Many people with Crohn's disease or ulcerative colitis might need surgery even with new treatments available.
  • Common surgeries include removing parts of the intestines or creating new ways for waste to leave the body.
  • Doctors use special tools called endoscopes to closely check and treat problems in the intestines, but it can be tricky when the intestines have been changed by surgery.
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Objectives: Endoscopic mucosal improvement is the gold standard for assessing treatment efficacy in clinical trials of Crohn's disease. Current endoscopic indices are not routinely used in clinical practice. The lack of endoscopic information in large clinical registries limits their use for research.

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The COVID-19 pandemic triggered an unprecedented expansion of telemedicine, leading to development of new workflows. We conducted a survey of telemedicine practice among pediatric gastroenterology practitioners on March 26, 2020. Responses were coded and analyzed.

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The various malformations of the aerodigestive tract collectively known as esophageal atresia/tracheoesophageal fistula (EA/TEF) constitute a rare group of birth defects of largely unknown etiology. Previous studies have identified a small number of rare genetic variants causing syndromes associated with EA/TEF. We performed a pilot exome sequencing study of 45 unrelated simplex trios (probands and parents) with EA/TEF.

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Uses of nutritional therapies for inflammatory bowel disease (IBD) are of tremendous interest to the lay and professional communities. This interest currently outweighs the scientific basis for deciding on a particular therapy for any given patient. Some nutritional therapies have credible reports, in peer-review journals, validating their use for some patients.

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The Crohn's & Colitis Foundation has facilitated transformational research in pediatric inflammatory bowel disease (IBD), through the RISK and PROTECT studies, that has laid the groundwork for a comprehensive understanding of molecular mechanisms of disease and predictors of therapeutic response in children. Despite these advances, children have lacked timely and informed access to the latest therapeutic advancements in IBD. The Crohn's & Colitis Foundation convened a Pediatric Resource Organization for Kids with Inflammatory Intestinal Diseases (PRO-KIIDS) Clinical Innovations Meeting at the inaugural Crohn's and Colitis Congress in January 2018 to devise how to advance the care of children with IBD.

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Recent advances in high-throughput laboratory technologies and bioinformatics tools are redefining how we view inflammatory bowel disease (IBD). Instead of 2 diseases, we now see a diverse set of molecular subtypes. Large-scale investigation of the genome, exome, transcriptome, proteome, metabolome, microbiome, and epigenome are providing transformative insights into the pathophysiology of IBD, with the promise of accurately predicting prognosis and targeting therapy.

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Purpose Of Review: Treatment options for inflammatory bowel disease (IBD) have rapidly expanded as the treatment paradigm has shifted from controlling symptoms to reducing lifetime inflammatory burden. Families are confronted with the actual and perceived risks of this ever-expanding array of choices. We aim to review the shared decision-making process in pediatric IBD to ensure an optimal therapeutic plan for the child and their family.

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Acyl-CoA:diacylglycerol acyltransferase (DGAT)1 and DGAT2 catalyze triglyceride (TG) biosynthesis in humans. Biallelic loss-of-function mutations in human result in severe congenital diarrhea and protein-losing enteropathy. Additionally, pharmacologic inhibition of DGAT1 led to dose-related diarrhea in human clinical trials.

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Severe congenital neutropenia type IV (SCN IV) is a syndrome of severe neutropenia, cardiac and urogenital defects, prominent superficial veins, facial dysmorphism, failure to thrive (FTT), and intermittent thrombocytopenia, caused by a glucose-6-phosphatase catalytic subunit 3 (G6PC3) gene mutation. SCN IV has been linked to glycogen storage disease type 1b as both disorders involve disruption of the glucose-6-phosphatase/glucose-6-phosphate transporter complex, leading to arrested neutrophil maturation. Emerging evidence suggests that neutrophil function plays an important role in intestinal integrity, evidenced by inflammatory bowel disease in certain neutropenic patients.

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Background And Aims: Gastrojejunal feeding tubes (GJTs) are typically converted from gastrostomy feeding tubes by interventional radiology in many pediatric centers to provide both postpyloric feeding and gastric decompression. Endoscopic transgastric GJT placement via an established gastric stoma can be performed without sedation and with minimal fluoroscopy but is relatively new in pediatrics with limited description. This study analyzed the success rate, adverse events, and technical issues associated with endoscopic GJT placement via a transgastric approach in pediatric patients at a large children's hospital.

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Background: L-serine plays an essential role in neuronal development and function. Although a non-essential amino acid, L-serine must be synthesised within the brain because of its poor permeability by the blood-brain barrier. Within the brain, its synthesis is confined to astrocytes, and its shuttle to neuronal cells is performed by a dedicated neutral amino acid transporter, ASCT1.

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