Filling in the "GAPPS": an unusual presentation of a child with gastric adenocarcinoma and proximal polyposis of the stomach
Gastric Adenocarcinoma and Proximal Polyposis of the Stomach (GAPPS) is a very rare gastric polyposis syndrome characterized by numerous polyps of the gastric fundus and body. We present the unusual case of a 10-year-old Polish-American male with history of eosinophilic esophagitis, who was found to have multiple fundic gland polyps (FGP) with low grade dysplasia on esophagogastroduodenoscopy. Subsequent evaluation including genetic testing confirmed the diagnosis of GAPPS, and after exhaustive multidisciplinary consultation the decision was made to proceed with prophylactic total gastrectomy given the markedly increased risk of gastric adenocarcinoma in GAPPS patients. To our knowledge, this represents the youngest patient diagnosed with GAPPS and the youngest patient who has undergone prophylactic gastrectomy for this disease at age 8 and 10Â years, respectively. The pathophysiology, presentation, and treatment of GAPPS in a pediatric patient are discussed.
Concurrent celiac disease and eosinophilic esophagitis in a pediatric cohort: More than a coincidence [Meeting Abstract]
Introduction: Celiac Disease (CeD) and Eosinophilic Esophagitis (EoE) represent two distinct histopathologic diseases, yet sometimes are diagnosed concomitantly in the same patient. The literature conflicts as to whether there is an increased risk of EoE in the CeD population compared to the general population. Further, it is unclear if the concurrence of both diseases represents a distinct pathophysiology. The purpose of our study is to characterize pediatric patients with both CeD and EoE. Specifically, we compared demographics, personal/family history, and lab values in patients with both CeD and EoE compared to patients with CeD only.
Method(s): We performed a retrospective single-center case-control study. All patients with CeD, confirmed by Marsh 2 or 3 on duodenal biopsies, seen in our pediatric CeD clinic over the past 5 years were included. Patients that did not meet histologic criteria for CeD diagnosis, without esophageal biopsies, or without available pathology results were excluded. EoE patients were then identified by histological findings of >15 eosinophils per high-power field on esophageal biopsy. Through chart review, we identified the patients' sex, body mass index percentile at diagnosis, age at presenting symptoms and diagnosis, personal history and family history of atopy or autoimmune disease, and albumin, hemoglobin, absolute eosinophil count (AEC), and tissue transglutaminase antibody IgA at time of diagnosis. We used unpaired t-test to compare values and Fisher's exact test to compare presentation and symptoms in the patients with both CeD and EoE and those with only CeD.
Result(s): There were 225 CeD patients identified. We excluded 18 patients that did not meet the CeD definition, 24 patients that did not follow up, 34 patients without pathology records, and 1 patient without esophageal biopsy. We included the remaining 148 patients in our study. Of these patients, 137 had CeD only (92.6%) and 11 had both CeD and EoE (7.4%). Patients with both CeD and EoE had higher AEC prior to diagnosis with a mean of 454.1/muL +/-122.7/muL compared to 231.9/muL +/-19.5/muL for patients with only CeD (P=0.003). We found no difference in other variables evaluated (Table).
Conclusion(s): We found a higher proportion of EoE in CeD patients than expected in the general population, suggesting a possible connection between the two diseases. In addition, we demonstrated that patients with both EoE and CeD have a higher AEC than patients with only CeD. This may be helpful in determining which patients may additionally have EoE when performing endoscopy on suspected CeD patients. Next steps include comparing our cohort of both CeD and EoE to EoE alone and further evaluating our CeD/EoE cohort. We believe that by investigating patients with both CeD and EoE, we will be able to better understand these individual diseases and optimize care for our patients
Proton pump inhibitor administration in neonates and infants. Lack of consensus - An ASPO survey
OBJECTIVE:Laryngopharyngeal and Gastroesophageal reflux (LPR and GER) are distinct clinical entities that present with a range of non-specific symptoms. The exact prevalence in the pediatric population is unknown. While there has been an increase in the use of PPIs, lack of clear guidelines, conflicting evidence regarding efficacy and safety concerns with long-term use require physicians to use their own anecdotal experience and clinical judgement when treating patients. The goal of this study was to evaluate practice patterns among pediatric otolaryngologists regarding the use of proton-pump inhibitors for reflux-related conditions. METHODS:A survey was submitted to American Society of Pediatric Otolaryngology (ASPO) members to determine practice patterns regarding use of PPIs for reflux-related conditions in the newborn and infant population. Statistical analysis using Fisher's exact test was performed. RESULTS:37% of respondents would not prescribe PO PPIs in neonates, with 50% not prescribing IV PPIs. 60% would prescribe a PPI as second or third-line treatment for infants (10 weeks to 1-year). Only 10% would prescribe as first-line in this age group. 48% would prescribe PPIs once daily and 19% as BID. No significant practice differences exist based on years of experience, number of relevant patients seen, and setting of practice. CONCLUSION/CONCLUSIONS:There was no agreement regarding dosage, frequency and duration of PPI treatment for reflux disease in neonates and infants. There was also no correlation with experience or practice setting. This emphasizes the need for a multidisciplinary approach and consensus statement to guide management of GER and LPR in this population.
Size and Prevalence of Pediatric Aerodigestive Programs in 2017
OBJECTIVE:Pediatric aerodigestive programs appear to be rapidly proliferating and provide multidisciplinary, coordinated care to complex, medically fragile children. Pediatric subspecialists are considered essential to these programs. This study evaluated the state of these programs in 2017 by surveying their size, composition, prevalence, and the number of patients that they serve. METHODS:The North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition Aerodigestive Special Interest Group leadership distributed an 11-question survey to the Pediatric Gastroenterology International Listserv. The mean time of the programs' existence, number of half-day clinics, number of procedure days, number of patients evaluated, and the lead primary specialty were evaluated. RESULTS:Thirty-four programs responded. Twenty-five were based in academic centers. Thirty-one programs were located across the United States. The average time of program existence was 5.3 years (standard deviation [SD] = 4.3; range 1-17 years). Approximately 64.7% were started in the past 5 years. Twelve programs were based in the division of gastroenterology. The average number of gastroenterologists serving aerodigestive programs was 2 (SD = 1.1). The mean number of half-day clinic sessions and procedure days were 2.8 (SD = 2.9) and 2.6 (SD = 2), respectively. New and follow-up visits per year in each program averaged 184 (SD = 168; range 10-750). CONCLUSIONS:Pediatric aerodigestive programs are prevalent, proliferating, and serve a large number of complex patients across North America and the world. This survey demonstrated that programs are predominantly based in academic settings. The number of patients cared for by aerodigestive centers varies widely depending on size and age of program.
Airway and esophageal eosinophils in children with severe uncontrolled asthma
AIM/OBJECTIVE:Children with severe uncontrolled asthma (SUA) have a high burden of symptoms and increased frequency of asthma exacerbations. Reflux esophagitis and eosinophilic esophagitis are important co-morbid factors for SUA. Both are associated with the presence of eosinophils in esophageal mucosa. We hypothesized that esophageal eosinophils are frequently present and correlate with the presence of airway eosinophils in children with SUA. METHOD/METHODS:We performed a retrospective analysis of a prospective database of children who underwent "triple endoscopy" (sleep laryngoscopy, bronchoscopy with bronchoalveolar lavage [BAL] and endobronchial biopsy [EBB], and esophagogastroduodenoscopy with esophageal biopsy [EsB]) at our Aerodigestive Center for evaluation of SUA. Children with known cystic fibrosis, primary ciliary dyskinesia, and aspiration-related lung disease were excluded. RESULT/RESULTS:Twenty-four children (21 males) ages 2-16 years were studied. Elevated BAL eosinophils were found in 10 (42%) patients, endobronchial eosinophils in 16 (67%); 7 (29%) had endobronchial eosinophils without elevated BAL eosinophils. Esophageal eosinophils were found in 11 (46%) patients. There was a correlation between the amount of eosinophils in BAL and EBB (Râ€‰=â€‰0.43, Pâ€‰=â€‰0.05) airway eosinophils, defined as elevated BAL and/or EBB eosinophils, correlated with esophageal eosinophils (Râ€‰=â€‰0.41, Pâ€‰=â€‰0.047). CONCLUSION/CONCLUSIONS:We concluded that airway and esophageal eosinophils are frequently present in children with SUA.
Association of airway esophageal eosinophils in children with refractory asthma and chronic cough [Meeting Abstract]
PURPOSE: Reflux esophagitis (ReE) and eosinophilic esophagitis (EoE) are associated with the presence of eosinophils in esophageal mucosa and are considered to be important co-morbid factors for chronic cough and asthma in adults. We hypothesize that esophageal eosinophils related to ReE and EoE are present in children with refractory asthma and chronic cough and correlate with airway eosinophilia. METHODS: We performed a retrospective analysis of medical records of children who underwent "triple endoscopy" (sleep laryngoscopy, bronchoscopy with bronchoalveolar lavage (BAL) and endobronchial biopsy (EBB), and esophagogastroduodenoscopy with esophageal biopsy (EsB)) at our Aerodigestive Center for evaluation of refractory asthma and cough. Inclusion criteriawere cough for 8 weeks or more with no response to trial of antibiotics and systemic/inhaled corticosteroids (ICS), poor control of asthma symptoms, and/or airflowlimitations and air trapping despite use ICS or ICS/long-acting beta-agonist combination. Children with known cystic fibrosis, primary ciliary dyskinesia and aspiration into airway were excluded. RESULTS: Thirty-two children (22 males) met inclusion criteria. Nineteen had refractory asthma and 13 had chronic cough. There were no significant complications recorded after procedures including EBB. Eosinophils (>1%) were present in BAL of 8 (25%) children. EBB showed eosinophils in 17 (53%) children. There were a total of 19 children with eosinophils isolated from the airway (either BAL or EBB), 4 (21%) had them in BAL alone, 8 (42%) in EBB only, and 7 (37%) in both BAL and EBB. EoE was diagnosed in 6 children (19%) and ReE in 13 (41%). EsB revealed esophageal eosinophils in 47% of children. Presence of eosinophils in EsB was related to presence of eosinophils in EBB chi2 (1, N = 32), p = 0.026, but not BAL (p=0.89). CONCLUSIONS: ReE and EoE with esophageal eosinophils was present in 47% of children with refractory asthma and chronic cough. There is a significant relationship between airway and esophageal eosinophils, which becomes evident only when EBB is performed for detection of airway eosinophils. Further research is required for understanding the association of airway and esophageal eosinophilia in the development and management of refractory asthma and cough
Pediatric patients with chronic cough and recurrent croup: The case for a multidisciplinary approach
OBJECTIVE: To evaluate the results of our multidisciplinary approach to recurrent croup and chronic cough. METHODS: Retrospective chart review of all patients with recurrent croup and chronic cough managed at a tertiary care children's hospital by our Comprehensive Airway, Respiratory, and Esophageal (CARE) Team. Charts were reviewed for all patients who carried a diagnosis of recurrent croup or chronic cough. Patients were excluded if they did not receive a full workup, including micro-direct laryngoscopy, flexible and/or rigid bronchoscopy, bronchioalveolar lavage (BAL), and upper endoscopy with biopsies. We reviewed the records for the presence of gastrointestinal complaints, abdominal pain and failure to thrive (FTT) and compared the children with documented esophagitis to the remaining children. RESULTS: Forty patients met inclusion criteria. 53% had airway abnormalities; the most common was tracheomalacia, followed by enlarged adenoids. 38% had esophagitis (group 1) while 62% had normal esophageal biopsies (group 2). Among the children in group 1, 27% met criteria for eosinophilic esophagitis (>15 eosinophils per high powered field). There was no significant difference between groups 1 and 2 based on the presence of gastrointestinal complaints, abdominal pain and/or FTT (p>0.05). There was no significant difference between the groups based on the location or presence of an airway abnormality (p>0.05). CONCLUSIONS: Children with recurrent croup and chronic cough may benefit from a multidisciplinary approach to management. Our CARE Team approach led to a specific diagnosis in almost 95% of patients.
Impedance and extraesophageal manifestations of reflux in pediatrics
OBJECTIVES/HYPOTHESIS: Extraesophageal manifestations of gastroesophageal reflux (GER) include such signs and symptoms as cough, asthma, respiratory symptoms, hoarseness, and laryngoscopic findings. We reviewed the role of MII-pH monitoring in the evaluation of these findings in children to determine whether there is an association with pathological acid or nonacid reflux. STUDY DESIGN: Retrospective chart review. METHODS: We retrospectively reviewed charts from patients who underwent MII-pH. Inclusion criteria were ages 0 to 21 years with extraesophageal signs or symptom. Data were analyzed using dedicated software and manually reviewed. Reflux composite score was calculated based on DeMeester criteria. Impedance scores were calculated based on adult criteria. Symptom indexes were calculated. RESULTS: A total of 119 MII-pH studies were performed. Of those, 63 studies met inclusion criteria. There were 39 males and 24 females with mean age 7.32 +/- 4.1 years. The most common indication was cough. Six children had pathological GER based on DeMeester score. Using impedance criteria, only 10 of 63 patients had an abnormal evaluation (mean reflux episodes 107). Seven patients (15.2%) were found to have an association between symptom and reflux event. CONCLUSIONS: No association was demonstrated between the extraesophageal signs and symptoms and pathological GER based on DeMeester score or the number of reflux events based on impedance testing.
Small Intestinal Transit Time in Children With Crohn's Disease [Meeting Abstract]
Multichannel Intraluminal Impedance(MII) and Extraesophageal Manifestations of Gastroesophageal Reflux (GER) in Pediatrics [Meeting Abstract]