JPGN reports最新文献

Asparaginase is a critical component of modern therapy for acute lymphoblastic leukemia (ALL). Its use has improved cure rates for both children and adults with this disease. However, asparaginase therapy can be complicated by significant toxicities, including acute pancreatitis (AP). Such pancreatitis can be severe and result in life-threatening or fatal complications. Here, we describe five cases of severe asparaginase-induced pancreatitis in children receiving therapy for ALL. Despite maximal supportive care, these patients experienced severe symptoms and were unable to tolerate enteral nutrition (EN) initially after presentation. This case series highlights clinical features associated with intolerance to EN in this population. Providers should be mindful of these features when considering EN trials in patients with severe asparaginase-associated AP. Some patients may require total parenteral nutrition to adequately support nutrition and recovery in severe asparaginase-associated AP.

Allergies and other chronic immune mediated conditions are becoming increasingly common. Here we utilized a prospective birth cohort called the Celiac Disease Genomic Environmental Microbiome and Metabolomic (CDGEMM) study to examine the frequency of parent reported allergic conditions and their association with celiac disease (CeD). We examined 271 children at-risk of CeD from the United States and found a high frequency of allergic conditions. In our overall cohort, 19.8% reported food protein-induced allergic proctocolitis (FPIAP), 12.5% reported IgE-mediated food allergy, and 14.7% reported atopic dermatitis. Among the 23 children with CeD, 21.74% had FPIAP, 8.7% had an IgE-mediated food allergy, and 21.74% had atopic dermatitis. No significant association between allergic conditions and CeD was found (p > 0.35 for all). These results highlight the widespread occurrence of parent-reported allergic conditions in children but do not suggest an association between allergic conditions and CeD development.

In 2013, a survey of NASPGHAN members on their involvement in managing pediatric obesity revealed that most pediatric gastroenterologists viewed their roles as primarily supportive, focusing on the management of obesity-related gastrointestinal comorbidities. This 10-year follow-up survey, which targeted pediatric gastroenterology programs and practices, found similar perceptions, along with an increased interest in learning about obesity pharmacotherapy. Additionally, most programs and practices reported limited access to dedicated dietitians, a lack of funding, and a desire for greater institutional support for multidisciplinary programs addressing obesity. Notably, none of the funded programs cited philanthropy as a source of funding. These findings underscore the need for greater advocacy to increase dietitian support and educational opportunities in obesity pharmacotherapy, the development of multidisciplinary teams, and the exploration of philanthropic support for innovative programs in the management of children with obesity.

Objectives: Patients with cerebral palsy (CP) often have gastrointestinal dysmotility. An inciting event, such as infection, may lead to progressive decline in bowel motility and episodes of acute pediatric intestinal pseudo-obstruction (PIPO). Surgery can be implemented when medical therapy fails, but it is unclear if it can improve or lengthen the quality of life. Here, we explore this question with a case series.

Methods: We performed a retrospective chart review and identified five patients with CP who were hospitalized between January 2017 to January 2024, secondary to a triggering event. They all developed subsequent decline in bowel function.

Results: We present five gastrostomy tube-dependent patients with CP who had prolonged hospitalizations after a triggering event and an associated decline in intestinal motility. Case 1 is a 7-year-old female with feeding intolerance after a viral infection and a hospital stay of 30 days. She received anal sphincter botulinum toxin injection and returned to baseline. Case 2 is 21-year-old male with aspiration pneumonia who became dependent on total parenteral nutrition (TPN). He died after complications associated with midgut volvulus. Case 3 is an 18-year-old male with feeding intolerance following COVID and required several procedures, a hospital stay of 205 days, and gradual return to baseline. Case 4 is a 15-year-old male with a small bowel obstruction and recurrent volvulus, prolonged hospitalization, and death. Case 5 is a 4-year-old female with frequent PIPO triggered by urinary tract infections.

Conclusion: Patients with CP are susceptible to a decline in bowel function. A balance between prolonging life and improving quality of life should always be considered. TPN in place of surgical interventions might help decrease hospitalizations and surgical morbidity. Surgery is reasonable for mechanical obstruction, but invasive procedures should not be assumed to improve quality of life.

Rumination syndrome (RS) is a common pediatric disorder of gut-brain interaction. However, a dearth of research on efficacious treatment to help guide clinical decision-making remains. Thus, relying on well-established treatment recommendations for other conditions with similar mechanisms or symptom profiles may be helpful; for example, conceptualizing RS treatment targets to address it as a "tic disorder of the gut" could be beneficial. To demonstrate the clinical utility of this conceptualization, we presented two cases that provide broad implications for treating pediatric RS. In both cases, akin to pediatric tic and related disorders, cognitive distress and physical discomfort were alleviated temporarily by rumination episodes. Thus, we recommend a targeted diagnostic assessment, including a functional analysis incorporating a transdiagnostic evaluation of symptoms. Close comanagement with specialty physicians and psychologists is also highly recommended. The patients represent complex yet standard cases seen in pediatric psychology outpatient clinics, suggesting the clinical utility of implications for psychologists and allied health professionals.

Objectives: Strict gluten-free diet (GFD) is the mainstay of therapy in celiac disease (CD), however, data on factors determining long-term adherence is limited. We aimed to compare serology, dietician detailed interview (DDI) and various questionnaires (Biagi, modified Leffler) to assess GFD adherence, to identify determinants of adherence and to assess the impact of counseling.

Methods: In this prospective study, consecutive children (<18 years) with CD on GFD were enrolled between November 2022 and May 2024. GFD adherence was assessed by anti-tissue transglutaminase (tTG), DDI and two questionnaires at baseline and after 6 months of follow-up.

Results: A total of 103 children (64 boys, median age 8 years [interquartile range [IQR]: 4-12.5]) on GFD (median duration 35 months [IQR: 11-52]) were enrolled. Adherence as assessed by serology, DDI, Biagi score and modified Leffler score were 73.8%, 48.5%, 66.2%, and 60.3%, respectively. Upon regression analysis, having a sibling with CD, and higher parental knowledge about CD influenced positively to adherence to GFD, while age >10 years and longer duration of follow-up influenced negatively to adherence. After dietary counseling, GFD-adherence improved on follow-up (p < 0.05 in all).

Conclusions: Almost a third of children have adherence issue. Having a sibling with CD, and higher parental awareness about CD positively and older age and longer follow-up negatively influenced GFD-adherence. Adherence improved significantly with dietary counseling on follow-up.

Objectives: To evaluate the long-term follow-up status of patients with a history of feeding tube dependence who completed a multidisciplinary, day treatment program.

Method: We conducted a 17-item Qualtrics survey of patients who participated in treatment to improve the volume and variety of solid food intake and replace enteral feeding due to chronic food refusal an average of 6 years following intervention.

Results: Of the 81 patients included in the original outcome study, 75 met eligibility criteria; 36 caregivers (48%) completed the survey. Survey respondents and non-respondents were similar in terms of patient demographics and status at discharge. Most patients (80%) who achieved full wean from feeding tube dependence at program discharge maintained their wean at the time of the survey. Most caregivers (89%) described their child's relationship with food as "good" or "neutral".

Discussion: Intensive multidisciplinary intervention holds benefits for children with complex feeding problems, including advancing the volume and variety of food consumed during meals and reducing the need for enteral feeding. The durability of these benefits, however, is not well understood. The current follow-up survey of patients who completed an intensive feeding program suggests that treatment gains, such as the reduction of tube dependency, endure for most patients.

Conclusion: Our findings provide provisional evidence of the long-term effectiveness of our intensive multidisciplinary model in promoting oral intake and reducing tube dependence among young children with chronic food refusal. Future research should seek to better understand who benefits most from this model of care.

A 16-year-old female presenting with acute left-sided abdominal pain, diarrhea, nausea, and vomiting associated with a 20-lb weight loss. She was found to have positive chlamydia polymerase chain reaction upon admission. She underwent endoscopy and colonoscopy, with pathology revealing marked inflammation, complete villous blunting with absence of goblet and Paneth cells, with increased crypt apoptosis of the proximal duodenum through terminal ileum, concerning for autoimmune enteropathy. Anti-enterocyte autoantibodies were positive. She was treated with 14 days of doxycycline for her chlamydia infection and had symptomatic improvement without immunomodulatory therapies. Repeat endoscopy 6 months after completion of antimicrobials showed histologic improvement, with the presence of some goblet and Paneth cells, and only mild villous blunting. Together, her histology and clinical response suggest that she exhibited an autoimmune-like enteropathy, though the resolution of her symptoms without steroids suggests this was not a true autoimmune process.

Pediatric patients with active and quiescent inflammatory bowel disease (IBD) have known fatigue, but there is limited understanding of how fatigue is clinically assessed. This study explored perspectives of pediatric IBD patients and healthcare professionals (HCPs) regarding fatigue assessments. A multidisciplinary team, including two patient advocates, recruited pediatric IBD patients and HCPs from the ImproveCareNow network to complete cross-sectional, REDCap surveys. Forty-three pediatric patients with IBD (M age 14.2 years old, 54% female) completed the patient survey; 56% had clinically elevated fatigue on the PROMIS Fatigue Short Form and >67% endorsed their HCPs had never or not often asked about fatigue. Sixty-six HCPs (70% physicians or advanced practice nurses, 61% female) completed the HCP survey, with 61% reporting routinely asking about fatigue in clinical visits. Study findings demonstrate discrepancies in patient and HCP perspectives regarding fatigue assessments and the need to improve assessment and care for fatigue in pediatric IBD.

[This corrects the article DOI: 10.1002/jpr3.12148.].