JPGN reports最新文献

Objectives: The use of biologic therapy is increasing in pediatric patients with inflammatory bowel disease (IBD). However, efficacy may be compromised by increased drug clearance and anti-drug antibodies (ADAs). Historically, concomitant immunomodulator therapy (CIT) has been used to prevent ADA formation. Pediatric studies evaluating CIT have focused largely on white, non-Hispanic patients and have demonstrated variable benefits. This study evaluated the utility of CIT in preventing ADAs in a pediatric IBD population with high Hispanic representation.

Methods: We reviewed the charts of patients undergoing biologic induction at the Miller Children's Pediatric IBD Center between 2013 and 2023. Patients with missing therapeutic drug monitoring data or incomplete follow-up were excluded. Categorical variables were compared using chi-square or Fisher's exact tests, and continuous variables were analyzed using t tests. Multivariate logistic regression identified independent predictors of ADA formation.

Results: Of 163 pediatric patients, 75 had Crohn's disease (CD) and 80 had ulcerative colitis (UC). High-dose infliximab (IFX) was protective against ADA formation (p < 0.001), as were corticosteroids (p = 0.029). UC patients were more likely to receive corticosteroids at induction (CD: 27/75, UC: 45/80; p = 0.011). Among UC patients, CIT reduced ADA formation (odds ratio: 0.18; 95% confidence interval: 0.04-0.73; p = 0.031). Hispanic patients were more likely to have UC (CD: 18/73, UC: 46/80; p < 0.001). Hispanic UC patients had shorter times to biologic initiation (p = 0.025) and were more likely to receive both high-dose IFX and corticosteroids at the time of biologic induction (p = 0.044).

Conclusions: High-dose IFX may obviate the need for CIT in ADA prevention in pediatric IBD. UC patients may benefit more from CIT than CD patients. Disparities in treatment timing and medication use among Hispanic UC patients highlight the need for further investigation.

This is a comprehensive review of pediatric foreign body (FB) ingestions, emphasizing the global burden, epidemiology, and management strategies. Predominantly occurring in children under 6, with a peak between 6 months and 3 years, these incidents pose significant health risks with substantial regional variations in ingested objects-ranging from household items to caustic substances. The pathophysiological effects of ingestions are outlined, highlighting specific dangers associated with button batteries, sharp objects, and caustic agents, which can lead to severe tissue damage and long-term complications. A survey of (70) pediatric gastroenterologists from FISPGHAN societies revealed varied regional practices in the frequency of different FB ingestions with food impaction highest in North America and Ocenaia and caustic ingestion more common is Asia. Button batterers were serious concerns across all regions. Management approaches, specifically intubation practice varied across regions. A review of management protocols for different types of ingestions are provided, emphasizing the urgency of endoscopic removal and follow-up care. Prevention strategies are critical, with a focus on public health interventions, legislation, and education to mitigate the risks associated with FB and caustic ingestions. This report underscores the need for enhanced preventive measures and uniform management guidelines to reduce the incidence and improve outcomes of pediatric ingestions worldwide.

We present a case of a young female patient with persistent and severe fat-soluble vitamin deficiency since infancy. Despite extensive investigations during childhood, the underlying cause remained elusive. The patient was generally asymptomatic while receiving continuous vitamin subsidy. Exome sequencing performed at age 18 revealed a homozygous missense variant (Pro65Leu) in the gene SLC10A2, which encodes a bile acid transporter in the ileum. A bile retention scan showed severe bile acid malabsorption, with only 1.6% of radioactively labeled bile acids retained 7 days after intake. Treatment with a bile acid sequestrant was attempted but discontinued due to side effects and no significant effect on intestinal vitamin uptake. This case highlights the importance of early genetic testing in patients with unexplained fat-soluble vitamin deficiency. It also emphasizes the need for further research to elucidate the clinical spectrum and management of primary bile acid malabsorption due to SLC10A2 deficiency.

Gastrointestinal (GI) bleeding can be a common symptom in the pediatric population. Vascular malformations, which cause symptoms based on their location and effect on surrounding structures, are an uncommon cause of GI bleeding. We present the case of a 6-year-old male with a 1-year history of hematochezia, constipation, and microcytic anemia. Physical exam demonstrated a firm, mobile, non-circumferential anal mass measuring 4 cm in diameter, and located predominantly at the right-anterior bowel wall. Initial imaging, including abdominal radiograph and abdominal ultrasound, was unremarkable. Colonoscopy was visually and histologically negative. Magnetic resonance imaging of the pelvis showed an extensive pelvic venous-lymphatic malformation in the left hemipelvis, with extension to the right hemipelvis and left lower extremity, displacement of the rectum and colon, and protrusion into the anal canal. He was started on Sirolimus with symptom improvement. This case discusses a rare presentation of a pediatric vascular malformation presenting with a protruding anal mass.

Objectives: This study aimed to assess the nutritional status of children aged 1 month to 14 years during admission and discharge at Tikur Anbesa Specialized Hospital, Addis Ababa, Ethiopia.

Methods: A prospective descriptive study design was employed, utilizing structured and nonstructured questionnaires to collect data. A total of 400 children, aged 1 month to 14 years, were included in the study. Anthropometric measurements were conducted to determine their nutritional status. Parents were interviewed to gather information on socio-demographic characteristics, clinical history, and feeding patterns of the children. Descriptive analysis was performed using SPSS software version 23.0. Chi-square tests were utilized to identify differences, while odds ratios (ORs) were employed to assess and quantify associations between categorical outcomes. ORs were reported with 95% confidence intervals (CIs). A p-value less than 0.05 was considered statistically significant.

Results: The study included 400 children, consisting of 245 (61.3%) males and 155 (38.8%) females. During admission, the assessment of nutritional status revealed that 301 (75.3%) of the children were classified as normal, 99 (24.7%) undernourished. On discharge 228 (57.0%) normal, 172 (43.0%) undernourished. Gender was found to be significantly associated with undernutrition during admission. Females were 2.376 times more likely to be exposed to undernutrition compared to males, with a 95% CI for the OR ranging from 1.497 to 3.773.

Conclusion: The prevalence of undernutrition was significantly higher on discharge than during admission. Therefore, providing high-quality nursing care is crucial for children admitted to the hospital.

Severe acute respiratory syndrome coronavirus 2 (SARS‑CoV‑2) has known liver tropism. Multiple reports and studies demonstrated liver injury early in the pandemic. This retrospective cross-sectional comparison evaluates predictors of transaminase elevation during acute SARS-CoV2 infection, with particular interest in evaluating whether vaccination is associated with transaminase elevations. We extracted electronic medical record data for pediatric SARS-CoV2 patients presenting at safety net hospitals in Brooklyn, NY, between March 2020 and March 2022 with a coincident comprehensive metabolic panel, without multisystem inflammatory syndrome in children, prior liver disease and sickle cell disease (n = 133): 79.2% Black and 87% non-Hispanic. Transaminase elevation was more common among younger patients and patients requiring hospitalization or intensive care unit care. Vaccination was associated with lower quantitative levels of transaminase elevation but not the binary indicator for elevated transaminases. In aggregate, our results suggest transaminase elevation is a function of SARS-CoV2 disease severity.

Inflammatory bowel disease (IBD), including Crohn's Disease (CD) and ulcerative colitis, is a chronic inflammatory condition affecting the gastrointestinal tract. Treatment for IBD depends on disease severity and can include medical and surgical management. Advances in treatment and the availability of biologics have significantly reduced the need for surgical interventions. Eosinophilic mesenteric vasculitis (EMV) is a rare form of intestinal vasculitis that can mimic IBD. Diagnosis of EMV is challenging as it requires full-thickness biopsies. It can be mistaken for CD due to its response to steroids, which are a first-line therapy for EMV; however, EMV typically does not respond to other IBD-specific therapies. We present the case of a 15-year-old girl with a history of autoimmune hemolytic anemia who initially appeared to have CD but was diagnosed with EMV following a lack of clinical remission and persistence of the colonic stricture despite biologic therapy, which ultimately led to bowel obstruction symptoms requiring surgical resection.

Kirberg et al. described in 2016 a one-step gastrostomy tube placement, the 'pull-introducer' technique, designed for small-for-gestational-age neonates, combining two endoscopic techniques. Nine infants (weight 3.4-8.1 kg) in our centre required a gastrostomy placement (two infants) or a combined gastrostomy placement and Nissen fundoplication (seven infants). A single-stage balloon gastrostomy was preferred in all cases because of a significant anaesthetic risk. The pull-introducer method was chosen because the commercially available one-step balloon gastrostomy gastropexy devices were considered too large. There were no immediate or long-term complications (except local infection). All nine infants were discharged from the hospital as planned. After 6 months, the balloon gastrostomy tube was replaced with a balloon button or balloon gastrostomy tube.

Genital edema is a rare presentation of Crohn's disease (CD), also known as metastatic CD (MCD). This may precede, co-occur with, or follow gastrointestinal symptoms and present a diagnostic challenge. We aimed to characterize the features, clinical courses, pathogenesis, and outcomes of patients with MCD to increase understanding and promote timely management. A retrospective review of four patients diagnosed with MCD was conducted at Phoenix Children's and Children's Hospital Colorado. Patients presented with painful and painless penoscrotal swelling with and without erythema. Scrotal histopathology revealed granulomatous inflammation, and genetic testing identified pathogenic variants in NOD2, COL7A1, and Chek2, as well as additional variants of uncertain significance. Treatments included antibiotics, steroids, biologics, and methotrexate with mixed responses. Further research and clinical trials are needed to better understand the pathogenesis and develop best practices.