JPGN Reports最新文献

D-lactic acidosis (D-LA) is an uncommon complication of short bowel syndrome characterized by elevated plasma D-lactate and encephalopathy. Treatments include rehydration, dietary carbohydrate restriction, and antibiotics to alter the gut microbiota. Fecal microbiota transplantation (FMT) has recently been used in children to successfully treat D-LA. We compared the clinical course and then utilized metagenomic shotgun sequencing to describe changes in the composition and function of the intestinal microbiome following FMT in 2 patients with recurrent D-LA. FMT altered the composition of the fecal microbiota in these 2 patients with recurrent D-LA, though not necessarily in a consistent manner. Importantly, microbial metabolic pathways were also impacted by FMT, which may be critical for achieving desired clinical outcomes. While sample size limits the generalizability of our results, these findings set the stage for further understanding of the role of microbes in the pathogenesis of recurrent D-LA.

The incidence of foreign body ingestion has increased during the coronavirus disease 2019 pandemic. As face masks became increasingly available, we report a case of accidental ingestion of the metal strip insert of a surgical mask. After initially progressing, its progress halted after 24 hours. This case highlights the challenges of timing the endoscopic removal of long objects, especially considering the reduced endoscopic availability during the pandemic. Despite only causing local trauma, the strip was impacted at the duodenojejunal flexure with the potential to cause obstruction. Limiting morbidity relies on urgent removal and prevention of similar ingestions by emphasizing the safe use and storage of masks.

Multidisciplinary care is recommended for optimal pediatric inflammatory bowel disease (IBD) care, including psychosocial providers (eg, psychologists). However, health care professionals' (HCPs) perceptions of and engagement with psychosocial providers in pediatric IBD is lacking.

Methods: Cross-sectional REDCap surveys were completed by HCPs (eg, gastroenterologists) across American ImproveCareNow (ICN) centers. Demographics and self-reported perceptions of and engagement with psychosocial providers were collected. Data were analyzed at participant and site levels through descriptives, frequencies, an independent t test, and exploratory analyses of variance.

Results: A total of 101 participants from 52% of ICN sites participated. Participants were 88% gastrointestinal physicians, 49% identifying as female, 94% non-Hispanic, and 76% Caucasian. Of ICN sites, 75% and 94% of sites reported outpatient and inpatient psychosocial care, respectively. Participants referred for various clinical reasons to psychosocial providers (eg, illness adjustment). At the participant level, 92% of HCPs reported psychosocial care was very important and 64% reported their clinical thresholds shifted to engage psychosocial providers earlier in care. Barriers to psychosocial care included limited psychosocial providers (92%), psychosocial providers availability (87%), and IBD patients' lack of openness to psychosocial care (85%). One-way analyses of variance by HCP length of experiences were not statistically significant on perceived understanding of psychosocial providers or perceived changes in clinical threshold over time.

Conclusion: HCPs overall reported positive perceptions of and frequent engagement with psychosocial providers in pediatric IBD. Limited psychosocial providers and other notable barriers are discussed. Future work should continue interprofessional education of HCPs and trainees and efforts to improve access to psychosocial care in pediatric IBD.

A male pediatric patient with elevated liver enzyme and bile acid levels, bile duct hypoplasia, mild liver fibrosis, and pruritus was initially diagnosed with progressive familial intrahepatic cholestasis. The patient did not respond to treatments of ursodeoxycholic acid and naltrexone. Subsequent treatment with odevixibat resulted in improvements in serum bile acid levels and pruritus within a few weeks of initiation. During the course of odevixibat treatment, genetic testing results and additional clinical findings indicated a diagnosis of Alagille syndrome, a condition that shares some clinical features with progressive familial intrahepatic cholestasis. Odevixibat treatment was continued off label, during which time the patient's serum bile acid levels dropped to within the normal limit and pruritus was completely ameliorated. This report suggests odevixibat may be an effective treatment option for Alagille syndrome.

Recessive dystrophic epidermolysis bullosa (EB) is a rare disease characterized by painful blistering and erosion of the skin, sometimes referred to as "butterfly skin disease" because patients' skin becomes as fragile as butterfly wings. In addition to severe dermatologic manifestations, EB patients also experience complications affecting epithelial surfaces including the gastrointestinal tract. While gastrointestinal complications such as oral mucosal ulceration, esophageal strictures, constipation, and gastroesophageal reflux are common in EB patients, reports of colitis are rare. Here we describe a patient with recessive dystrophic EB who developed EB-associated colitis. This case highlights the diagnostic challenges as well as the gaps in our current understanding of the prevalence, pathogenesis, and treatment of EB-associated colitis.

Childhood obesity is associated with many comorbidities. Bariatric surgery is known to be efficient for reducing weight in adolescents.

Objectives: The primary outcome was to identify somatic or psychosocial factors associated with success at 24 months after a laparoscopic adjustable gastric banding (LAGB) procedure in our cohort of adolescents with severe obesity. Secondary endpoints were to describe weight loss outcomes, comorbidity resolution, and complications.

Methods: We have retrospectively reviewed medical records of patients who had LAGB placed between 2007 and 2017. Factors associated with success at 24 months after LAGB were researched, with success being defined as positive percentage of excess weight loss (%EWL) at 24 months.

Results: Forty-two adolescents underwent a LAGB procedure, the mean %EWL was 34.1% at 24 months, with improvement in most comorbidities and without major complications. Having lost weight before surgery was associated with success, whereas a high body mass index at surgery was associated with a higher risk of failure. No other factor was found to be associated with success.

Conclusion: Comorbidities mostly improved 24 months after LAGB and no major complication occurred. Having lost weight before surgery was associated with a successful surgery, whereas a high body mass index at surgery increases the risk of failure.

Anti-TNF antibodies have become a first-line therapy in moderate-to-severe inflammatory bowel diseases. However, there may be some rare paradoxical events and those affecting joints causing severe symptoms need a scrupulous differential diagnosis. When these events occur, it may be necessary to discontinue treatment and shift to another drug class. Herein, we report the case of a 15-year-old boy affected by Crohn's disease, who developed a paradoxical reaction after the second dose of infliximab. Clinical remission was achieved shifting to budesonide and azathioprine and continuing maintenance therapy with azathioprine alone. To date, no other paradoxical events have occurred.

Screening children with obesity for nonalcoholic fatty liver disease leads to identification of elevated alanine aminotransferase (ALT) and is a common cause for referral to pediatric gastroenterology. Guidelines recommend that children with positive screening ALT be evaluated for causes of ALT elevation beyond nonalcoholic fatty liver disease. One clinical challenge is that autoantibodies can be present in patients with obesity and thus may or may not represent autoimmune hepatitis. This case series highlights the importance of a comprehensive evaluation to reach an accurate diagnosis.

We describe a 15-year-old female diagnosed with necrotizing pancreatitis in the setting of coronavirus disease 2019 with severe complications including splenic vein and portal vein thromboses, pleural effusion requiring chest tube, acute hypoxic respiratory failure requiring noninvasive positive-pressure ventilation, and new-onset insulin-dependent diabetes mellitus, requiring over a month-long hospitalization. Following discharge, the patient experienced a prolonged loss of appetite, nausea, and extreme weight loss., During her prolonged hospitalization, she was diagnosed with necrotizing pancreatitis with walled-off collection which was ultimately treated with transgastric endoscopic ultrasound-guided drainage, multiple endoscopic necrosectomies, lumen-apposing metal stents, and double-pigtail plastic stent. Nine months after her initial presentation, patient's clinical symptoms improved, and her weight stabilized. This case highlights the importance of recognizing acute and necrotizing pancreatitis and its morbidities as complications associated with coronavirus disease 2019.