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Functional luminal imaging probe (FLIP) measures pressure–geometry relationships of digestive luminal space. When used in esophageal disorders, it provides several luminal parameters that help better understand the pathophysiology. Data about the potential utility of FLIP in pediatrics are scarce and there is no standardized use in children. We aim to describe the use of FLIP in our center, its safety, feasibility, and clinical impact in esophageal disorders in children. Consecutive FLIP recordings performed at the Centre Hospitalier Universitaire-Sainte-Justine, Montréal, Canada between February 2018 and January 2021 were extracted. A chart review was conducted for demographics and medical history. Symptomatology after the procedure was evaluated with validated dysphagia scores. Nineteen patients were included (11 girls, median age 16 years, range 3.2–19.6) with achalasia (n = 5), post-Heller's myotomy dysphagia (n = 3), esophagogastric junction outflow obstruction (n = 3), congenital esophageal stenosis (n = 2); post-esophageal atresia repair stricture (n = 3), and post-fundoplication dysphagia (n = 3). There was no significant correlation between integrated relaxation pressure measured with high resolution manometry and distensibility index (DI). The use of FLIP made it possible to differentiate between dysphagia related to an esophageal obstruction (DI < 2.8 mm2/mmHg) and dysphagia without major motility disorder (DI > 2.8 mm2/mmHg) that guided the indication for dilation. FLIP led to a change in management in 47% of the patients. Forty-seven percent of the patients were symptom free at the time of the evaluation. FLIP provides key esophageal luminal values and therefore can play an important role in pediatric esophageal disorders management.

To develop models to accurately determine the outcomes of diagnostic endoscopies performed in children and adolescents without known gastrointestinal disease. Retrospective chart review of all endoscopies performed in children 2 to 18 years of age without known gastrointestinal disease from January 1 to December 31, 2000. The association between age, presenting symptoms, physical examination findings, laboratory investigations, and endoscopy outcomes was assessed. Predictive models for positive outcomes on endoscopy were estimated for upper and lower endoscopies separately by use of multiple logistic regression. Receiver operating curves were constructed to evaluate the performance of the models. A model with a sensitivity of 95% and specificity of 40% was considered clinically significant. Positive findings on endoscopy were found in 191 (55%) of 346 and in 120 (59%) of 204 upper and lower endoscopies, respectively. Age above 13 years, vomiting, and hypoalbuminemia were significant predictors of positive upper endoscopies. Rectal bleeding, hypoalbuminemia, and elevated erythrocyte sedimentation rate were significant predictors of positive lower endoscopies. Extrapolating from the receiver operating curves, a sensitivity of 95% corresponded to a specificity of 10% for the upper endoscopy model and 30% for the lower endoscopy model. In our population of children and adolescents, several clinical characteristics were predictive of positive upper and lower endoscopy outcomes. Predictive models composed of these clinical variables were statistically, but not clinically, significant. The inclusion of additional clinical characteristics that could be assessed in prospective studies will likely improve the clinical significance of endoscopy outcome prediction.

To develop models to accurately determine the outcomes of diagnostic endoscopies performed in children and adolescents without known gastrointestinal disease.

To review the authors’ experience with eosinophilic esophagitis. Between 1993 and 2001, the authors identified 12 patients with eosinophilic esophagitis defined on histologic criteria (≥20 eosinophils per high‐power field in the distal esophageal epithelium). The authors reviewed medical records for details of clinical presentation; laboratory data; radiologic, endoscopic, and histologic findings; and the results of continuous esophageal pH probe monitoring. Seventy‐five percent of the patients were male. The median age at presentation was 10.8 years (range, 1–17 years). Commonly reported symptoms were dysphagia with solid food (66%), epigastric pain (42%), food impaction (50%), and vomiting (8%). Food allergy was reported in 50% and asthma in 33%. Peripheral eosinophilia (>700/mm3) was found in 42%. Upper gastrointestinal series performed in eight patients, showed esophageal luminal narrowing in three. Computed tomography, performed in two patients, revealed thickening of the esophageal wall. Esophageal pH probe monitoring, performed in nine patients, revealed no abnormal acid reflux. All of the monitored patients had episodic alkalinization of the esophagus. Upper endoscopic analysis revealed white specks on the esophageal mucosa in 42%, esophageal narrowing in 33%, esophageal rings in 25%, and esophageal furrowing in 8%. The mean eosinophils per high‐power field was 65 (range, 20–200). Histologic characteristics included juxtaluminar (33%) and peripapillary clusters of eosinophils (33%), increased papillary height (50%), and basal cell hyperplasia (34%). Solid food dysphagia was the most common feature of eosinophilic esophagitis in our patients. Alkalinization of the esophagus was found in all nine pH probe recordings of eosinophilic esophagitis patients and may represent a previously unreported characteristic of the condition.

To review the authors’ experience with eosinophilic esophagitis.

North American Indian childhood cirrhosis (NAIC) is a distinct, rapidly evolving form of familial cholestasis found in aboriginal children from northwestern Quebec. This is a retrospective review of the 30 patients treated in Quebec since the discovery of NAIC in 1970. The clinical records and histologic samples from 30 patients were reviewed. Extensive metabolic, biochemical, viral, genetic, and radiologic studies were performed in most patients. Genetic analysis suggests autosomal recessive inheritance and a carrier frequency of 10% in this population. Gene mapping studies showed that the NAIC gene is located on chromosome 16q22. Typically, patients have neonatal cholestatic jaundice (70%) or hepatosplenomegaly (20%) with resolution of clinical jaundice by age 1 year but persistent direct hyperbilirubinemia. Portal hypertension was documented in 29 patients (91%). Variceal bleeding (15 patients, 50%) occurred as early as age 10 months. Surgical portosystemic shunting was performed in 13 of these 15 patients (87%); 4 (31%) rebled after 1 to 5 years. Fourteen patients died (47%). In 10 (71%), liver disease was the cause. Four children died of liver failure before liver transplantation became available. In transplanted livers, no recurrence of NAIC was observed after 1 to 10 years. Recognized infectious, metabolic, toxic, autoimmune, and obstructive causes of cirrhosis have been eliminated. The histologic features of NAIC show early bile duct proliferation and rapid development of portal fibrosis and biliary cirrhosis, suggesting a cholangiopathic phenomenon. Together with gene mapping studies showing that the NAIC gene is different from those of other familial cholestases, these observations suggest that NAIC is a distinct entity that could be classified as “progressive familial cholangiopathy.”

North American Indian childhood cirrhosis (NAIC) is a distinct, rapidly evolving form of familial cholestasis found in aboriginal children from northwestern Quebec. This is a retrospective review of the 30 patients treated in Quebec since the discovery of NAIC in 1970.