Celiac disease in North America: What is the current practice of pediatric gastroenterology providers?

Objectives: While guidelines exist for the diagnosis and management of pediatric celiac disease (CeD), current practices in North America are not well-described. This study aimed to explore current practice patterns to identify gaps and direct future clinical, training and research initiatives. Methods: A 23-item survey designed by the Celiac Disease Special Interest Group was distributed electronically to its members. Questions explored four themes: (1) screening and diagnosis pre and post the coronavirus disease (COVID)-19 pandemic, (2) treatment and monitoring, (3) family screening and transition of care, and (4) CeD focused training. Results: The survey response rate was 10.8% (278/2552). Most respondents were from the United States (89.9%, n = 250) and Canada (8.6%, n = 24). While endoscopy remained the gold standard, serology-based diagnosis was accepted by 47.5% (132/278). In response to the COVID-19 pandemic, 37.4% of providers changed their diagnostic practice. Barriers to care included: lack of insurance coverage for dietitians, wait times, and lack of CeD focused training. During fellowship 69.1% (192/278) reported no focused CeD training. Conclusion: Survey results revealed practice variation regarding the diagnosis and management of CeD in North America including a substantial proportion accepting non-biopsy, serology-based diagnosis, which increased during the COVID-19 pandemic. Variations in screening, diagnosis, interval surveillance, and family screening were also identified. Dedicated CeD education in pediatric gastroenterology fellowship may be an opportunity for standardizing practice and advancing research. Future North American guidelines should take current care patterns into consideration and develop new initiatives to improve care of children with CeD.

Clinical presentation and factors associated with gluten exposure in children with celiac disease.

OBJECTIVES: The prevalence of celiac disease (CeD) is increasing, yet it is still underdiagnosed, in part because of its heterogeneous presentation. Diagnostic criteria are evolving and management with strict adherence to a gluten-free diet is challenging for many. We aimed to characterize the clinical presentation of CeD among a large multicenter cohort of pediatric patients and to identify factors associated with gluten-free diet adherence. METHODS: Patients with CeD aged 0-18 years were recruited from 11 United States health centers. Parents completed surveys about gluten-free diet adherence and patient electronic health records were reviewed. Logistic regression analyses were performed to identify risk factors associated with gluten exposure. RESULTS: Charts were reviewed for 460 children with a median age of 6.4 years. Abdominal pain was reported in 57% of the cohort, but diverse symptoms were identified. Parent surveys were completed for 455 participants. Sixty-five (14%) participants were at high risk for gluten exposure based on parental reports of weekly or daily gluten exposure or eating gluten by choice in the past year. Participants under the age of 5 years had a lower risk of gluten exposure, while participants without repeat serology testing 18 months after initial diagnosis were at higher risk of gluten exposure. CONCLUSIONS: In a large, multicenter cohort of pediatric CeD patients, clinical presentation is highly variable, necessitating a high index of suspicion to make a diagnosis. Parent surveys indicate that 14% of patients are at high risk of gluten exposure, with patient age and lack of close follow-up associated with gluten-free diet adherence.

Twelve-year outcomes of intestinal failure-associated liver disease in children with short-bowel syndrome: 97% transplant-free survival and 81% enteral autonomy.

Our aim was to analyze the outcomes in children with short-bowel syndrome (SBS), parenteral nutrition dependence (PND), and intestinal failure-associated liver disease (IFALD) treated in our Intestinal Rehabilitation Program (IRP) during 2007-2018. We retrospectively reviewed charts of 135 patients with SBS-PND at the time of enrollment in IRP; of these, 89 (66%) had IFALD, defined as conjugated bilirubin (CB) of ≥2 mg/dl at enrollment and/or abnormal liver biopsy showing stage 2-4 fibrosis. Outcomes included resolution of CB, enteral autonomy, laboratory parameters (platelets, aspartate aminotransferase to platelet ratio index), growth trends, transplant rates, and mortality. Of the 89 patients, 74 had elevated CB at enrollment; the other 15 had normalized CB but had fibrosis on liver biopsy. Thirty-eight patients had liver biopsies: 36 (95%) had fibrosis, including 21/36 with bridging fibrosis/cirrhosis. The median proportion of residual small bowel was 23% (interquartile range, 13%-38%) of the expected length for age and median, daily energy requirement by PN was 100%. Two received a transplant, three died (one posttransplant), and the remaining 85 survived; 69 (81%) achieved enteral autonomy. Seventy-three (99%) of the 74 patients with hyperbilirubinemia normalized their CB with medical treatment. In a subset of eight of 89 patients with initial platelet count of <100,000/µl(median 50,500/µl) and median CB of 21 mg/dl, seven achieved CB normalization and had improved platelet count. Overall survival was 97% (censored 96.3%). We demonstrate high transplant-free survival and enteral autonomy rates among children with SBS-IFALD relying on low-dose soybean lipid emulsion.

Advances in Diagnostic Imaging in Pediatric Gastroenterology.

PURPOSE OF REVIEW: The purpose is to provide a review of cross-sectional imaging updates in the assessment of gastrointestinal diseases, relevant to clinical practice and research. RECENT FINDINGS: New magnetic resonance imaging contrast agents (Eovist) are taken up by hepatocytes and excreted via the biliary tree. As such, a lesion will retain contrast only if hepatocytes are present, which aids in refining the differential diagnosis. Magnetic resonance enterography is a method for non-invasively diagnosing and following various GI conditions, predominantly inflammatory bowel disease. Contrast-enhanced ultrasound uses gas-filled microbubbles providing superb temporal resolution most notably in the arterial phase, which aids in differentiating lesions. Elastography is a new technique which assesses stiffness of liver for evaluating fibrosis. These new techniques provide more accurate diagnoses and information, often limiting ionizing radiation exposure from other modalities. While ultrasound will still remain the initial imaging modality, familiarity with these other options is valuable for appropriate pathology workup.

Draining ears and tympanostomy tubes: a survey of pediatric otolaryngologists and pediatric emergency medicine physicians.

INTRODUCTION: Posttympanostomy tube otorrhea also known as acute otitis media with tympanostomy tubes (AOMT) occurs in 15% to 80% of children with tympanostomy tubes. Its management is fairly standardized among pediatric ear, nose, and throat (ENT) physicians owing to recommendations published by the American Academy of Otolaryngology-Head and Neck Surgery. Pediatric emergency medicine (EM) physicians have no such guidelines. OBJECTIVE: This study aimed to compare management of AOMT by pediatric ENT and EM physicians. METHODS: A 27-question online survey was disseminated via SurveyMonkey.com using e-mail addresses of ENT and EM physicians via organization directories and professional listserves. RESULTS: A total of 175 and 174 responses were received from EM and ENT physicians, respectively. Higher proportion of EM physicians used oral antibiotics to treat AOMT (54% [n = 94] vs 9% [n = 16], P < 0.001). Virtually all ENT physicians used topical antibiotics, compared with 87% of EM physicians. Only 6% (n = 10) of EM physicians used suction to clean ear canals (aural toilet) before instilling topical antibiotics, compared with 81% (n = 138) of ENT physicians. Most ENT physicians (80% [n = 138]) instructed patients to keep the treated ear up for 10 to 60 seconds after instilling the drops and to use the tragal pump technique to direct the medication down the ear canal and through the tube (92% [n = 157]). Only 56% (n = 98) and 24% (n = 41) of EM physicians did the same. CONCLUSIONS: There are large differences between ENT and EM physicians with respect to: the use of systemic antibiotics, techniques of using ototopical antibiotics, methods of aural toilet in treating AOMT, and directions given to patients.

Congenital paraurethral cysts in two newborn girls: differential diagnosis, management strategies, and spontaneous resolution.

BACKGROUND: At least five types of interlabial masses of different etiologies may present in a female neonate. The more serious type of interlabial mass must be differentiated from the benign and self-resolving paraurethral or hymenal cyst. Clues include appearance and color of the mass and the location of the mass in relation to the urethral meatus and the vaginal opening. Clinicians should be able to distinguish lesions that require aggressive intervention, i.e. surgery, from those that self-resolve and merely require observation. CASE: Two unrelated newborn girls each had a protruding faint-yellow-colored spherical interlabial cyst. The cyst was located anterior to the vaginal orifice and partially obscured the urethral meatus. Neither girl had any voiding problems. No other congenital anomalies were detected. Both cysts resolved rapidly and completely without surgical intervention. SUMMARY AND CONCLUSION: Paraurethral cysts of the newborn and hymenal cysts rarely cause urinary obstruction or spotting, and are self-resolving. When positively identified, no evaluation of upper urinary tract is required and neither aspiration of cyst contents or marsupialization procedure is necessary.