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T1126 Prospective Analysis of Clinical Symptoms in Pediatric Eosinophilic Esophagitis (EoE)
endoscopies had picture to visualize the EP. 29 patients with EA-TEF had VACTERL syndrome (an association of at least three of the following malformations: Vertebral, Anorectal, Cardiac, Tracheal, Esophageal, Renal and Limb), and among them 5 (17%) had an EP. There were no differences between the group with EA-TEF with or without EP regarding sex, prematurity, type of EA-TEF, presence or not of VACTERL syndrome or of any other congenital abnormalities. Our study demonstrates for the first time that the presence of a gastric EP is clearly associated with EA-TEF irrespective of the presence or not of other malformations. EA-TEF etiology and pathophysiology is currently unknown. Whether this association can be related to the same developmental mechanisms remains to be further studied and could lead to new pathophysiological hypothesis.
T1127
Background: The diagnosis of eosinophilic esophagitis (EoE) is based on the histologic finding of ≥ 20 eosinophils (eos)/hpf in esophageal biopsies (bx) collected at upper endoscopy (EGD). Grade C evidence suggests “multiple bx specimens” from different esophageal locations are required to diagnose EoE. Our objective was to determine if two bx from both the proximal and distal esophagus were adequate to make the histologic diagnosis of EoE. Methods: Prospective, multi-center, nested case-control study of outpatients (ages 1 to 17 years) undergoing EGD to evaluate vomiting, failure to thrive, feeding aversion, abdominal pain, or dysphagia. Subjects whose guardians gave written informed consent had an EGD with two esophageal bx obtained from the distal and 2 bx obtained from the proximal esophagus. If eos were present on bx, the densest area provided the eos count. Intracellular (IC) and extracellular (EX) eos derived neurotoxin (EDN) levels (scale of 0 to 6) were assessed by immunofluorescence stain of the bx. Subjects with ≥ 20 eos/hpf in either proximal or distal bx are cases; those with zero eos/hpf at both levels are normal controls, and those with at most 1 to 19 eos/hpf at either level are intermediates (intrm). Results: 146 patients participated. 20 cases, 96 normal controls, and 30 intrm were identified. Cases had a distal mean eos count of 40 (range 20 to 100) and proximal mean eos count of 36 (range 20 to 100). 17 of 20 cases showed concordance with ≥ 20 eos/hpf in both proximal and distal bx. 3 cases had distal eos counts ≥ 20 and proximal eos counts of 3, 10, and 10 eos/hpf, respectively. Intrm cases had a range of 0 to 10 eos/hpf on distal bx and 0 to 15 eos/hpf on proximal bx. There were 5 intrm with 0 eos on proximal bx and < 15 eos on distal bx, and 9 intrm cases with 0 eos on distal bx and < 15 eos on proximal bx. Conclusion: All cases had the histologic diagnosis of EoE confirmed with 2 distal esophageal bx. The addition of a proximal bx did not increase the diagnosis of EoE. Intrm cases with 0 eos/hpf at one level did not have significant eos/hpf at the opposite level to make the histologic diagnosis of EoE. There are greater levels of IC and EX EDN in cases versus controls. Further study of esophageal bx in the intrm range with IC and EX EDN is needed to evaluate if this staining pattern might help differentiate patients with EoE. IC and EX EDN Results
* Type III = EA with distal TEF T1126 Prospective Analysis of Clinical Symptoms in Pediatric Eosinophilic Esophagitis (EoE) Rayna Grothe, Yvonne Romero, Deborah K. Freese, Jeanne Tung, Mounif El-Youssef Background: EoE requires an upper endoscopy (EGD) with esophageal biopsies (bx) for diagnosis. Our objective was to determine which clinical symptoms in children correlate with number of eosinophils(eos)/hpf in esophageal bx. Methods: Prospective, multi-center, nested case-control study of outpatients (ages 1 to 17 years) scheduled to undergo EGD to evaluate vomiting, failure to thrive, feeding aversion, abdominal pain or dysphagia. A clinical history was obtained. Subjects whose guardians gave written informed consent underwent EGD with bx. Subjects with ≥ 20 eos/hpf are deemed cases and were compared with two groups; subjects with zero eos/hpf are normals, subjects with 1-19 eos/hpf are intermediate. Results: 146 consecutive patients participated. 20 cases, 30 intermediates and 96 normals were identified. Conclusions: Cases, intermediates and normals similarly presented with failure to thrive, vomiting, abdominal pain, heartburn, acid regurgitation, and environmental allergies. In both cases and normals, there is a wide age range in presenting symptoms. The presence of dysphagia and food impaction, while more common in cases, does not distinguish cases from normals. With regard to the frequency of other diseases, cases were more likely than normals and intermediates to have food allergies. Asthma was equally present in both cases and normals.
T1128 Measurement of Macrolipase Fraction in Children with Elevated Lipase Using Polyethylene Glycol Precipitation Prateek D. Wali, Zhaoping He, Amy S. Gutowski, Karoly Horvath Background: Macroenzymes are formed in the serum by self-polymerization or binding to proteins, mainly immunoglobulin. Their high molecular mass can lead to delayed renal elimination and accumulation in the serum, while maintaining their enzyme activity. Elevated levels of macrolipase (ML) may lead to a false diagnosis of pancreatic disease. Polyethylene glycol precipitation (PEG) is a method used to measure macroenzymes and hormones. It has not been reported for detection of macrolipase. Aims: To assess the value of PEG precipitation method in macrolipase measurement. Establish the cut-off values for macrolipasemia. Determine the prevalence of macrolipasemia in children with prolonged elevation in serum lipase and analyze associations with underlying diseases and other laboratory results. Methods: Remnant serum samples with normal and elevated serum lipase activities were collected from the clinical laboratory. The ML fraction was determined with the PEG precipitation method. The lipase activities of untreated and PEG-precipitated specimens were measured by an automated VITROS LIPA Slides System. The difference between the total and PEG-treated activity was considered ML activity. This was expressed as a percentage of the total lipase activity. Results: The control group consisted of 47 children with normal total serum lipase activity. The study group was 14 children with prolonged hyperlipasemia. Their lipase elevation persisted between 9 and 67 days (27 ± 16). In the control group, 10 of the 47 (21.3%) had a measurable ML fraction. These 10 patients had a ML ratio with a mean and SD of 16±8 % and were used to set cut-off values for further analysis. In the study group, 12 (85.7%) of the 14 patients had a ML ratio with a mean and SD of 33±11% (p<0.001). Two of the 10 controls (20%) and 9 of 14 study patients (64.2%) had ML ratio over 24% (>1SD). Two study patients had a ML ratio between 24-31%, while 7 had a higher elevation above 32% (>2SD). In our study an elevation in ML was frequently found in children with scoliosis after spinal fusion repair, malignancy, and autoimmune, renal or liver diseases. Conclusions: Our preliminary study revealed significantly higher ML fractions in children with hyperlipasemia as compared to controls. The PEG precipitation method is a promising test for ML measurement. Further studies to validate these findings are necessary. Measurement of ML activity is clinically important to avoid unnecessary testing, and treatment. T1129 Clinical Course of Pediatric Patients with Primary Sclerosing Cholangitis Folashade A. Jose, Melvin B. Heyman, Linda Ferrell, Ann L. Clark, Philip Rosenthal Background: Primary sclerosing cholangitis (PSC) is a chronic, idiopathic cholestatic disorder of the intra- and extra-hepatic biliary system and is often associated with inflammatory bowel disease (IBD). Few data are available on the natural history of pediatric patients with PSC with or without IBD. Methods: Medical records including liver biopsy, endoscopy,
A-505
AGA Abstracts
AGA Abstracts
Number and Location of Eosinophils and Eosinophil Derived Neurotoxin Staining in Pediatric Eosinophilc Esophagitis (EoE) Rayna Grothe, Brock J. Doubledee, Hirohito Kita, Yvonne Romero, Thomas C. Smyrk, Gail Kephart, Gary Neidich, Felicity Enders
T1127
Background: The diagnosis of eosinophilic esophagitis (EoE) is based on the histologic finding of ≥ 20 eosinophils (eos)/hpf in esophageal biopsies (bx) collected at upper endoscopy (EGD). Grade C evidence suggests “multiple bx specimens” from different esophageal locations are required to diagnose EoE. Our objective was to determine if two bx from both the proximal and distal esophagus were adequate to make the histologic diagnosis of EoE. Methods: Prospective, multi-center, nested case-control study of outpatients (ages 1 to 17 years) undergoing EGD to evaluate vomiting, failure to thrive, feeding aversion, abdominal pain, or dysphagia. Subjects whose guardians gave written informed consent had an EGD with two esophageal bx obtained from the distal and 2 bx obtained from the proximal esophagus. If eos were present on bx, the densest area provided the eos count. Intracellular (IC) and extracellular (EX) eos derived neurotoxin (EDN) levels (scale of 0 to 6) were assessed by immunofluorescence stain of the bx. Subjects with ≥ 20 eos/hpf in either proximal or distal bx are cases; those with zero eos/hpf at both levels are normal controls, and those with at most 1 to 19 eos/hpf at either level are intermediates (intrm). Results: 146 patients participated. 20 cases, 96 normal controls, and 30 intrm were identified. Cases had a distal mean eos count of 40 (range 20 to 100) and proximal mean eos count of 36 (range 20 to 100). 17 of 20 cases showed concordance with ≥ 20 eos/hpf in both proximal and distal bx. 3 cases had distal eos counts ≥ 20 and proximal eos counts of 3, 10, and 10 eos/hpf, respectively. Intrm cases had a range of 0 to 10 eos/hpf on distal bx and 0 to 15 eos/hpf on proximal bx. There were 5 intrm with 0 eos on proximal bx and < 15 eos on distal bx, and 9 intrm cases with 0 eos on distal bx and < 15 eos on proximal bx. Conclusion: All cases had the histologic diagnosis of EoE confirmed with 2 distal esophageal bx. The addition of a proximal bx did not increase the diagnosis of EoE. Intrm cases with 0 eos/hpf at one level did not have significant eos/hpf at the opposite level to make the histologic diagnosis of EoE. There are greater levels of IC and EX EDN in cases versus controls. Further study of esophageal bx in the intrm range with IC and EX EDN is needed to evaluate if this staining pattern might help differentiate patients with EoE. IC and EX EDN Results
* Type III = EA with distal TEF T1126 Prospective Analysis of Clinical Symptoms in Pediatric Eosinophilic Esophagitis (EoE) Rayna Grothe, Yvonne Romero, Deborah K. Freese, Jeanne Tung, Mounif El-Youssef Background: EoE requires an upper endoscopy (EGD) with esophageal biopsies (bx) for diagnosis. Our objective was to determine which clinical symptoms in children correlate with number of eosinophils(eos)/hpf in esophageal bx. Methods: Prospective, multi-center, nested case-control study of outpatients (ages 1 to 17 years) scheduled to undergo EGD to evaluate vomiting, failure to thrive, feeding aversion, abdominal pain or dysphagia. A clinical history was obtained. Subjects whose guardians gave written informed consent underwent EGD with bx. Subjects with ≥ 20 eos/hpf are deemed cases and were compared with two groups; subjects with zero eos/hpf are normals, subjects with 1-19 eos/hpf are intermediate. Results: 146 consecutive patients participated. 20 cases, 30 intermediates and 96 normals were identified. Conclusions: Cases, intermediates and normals similarly presented with failure to thrive, vomiting, abdominal pain, heartburn, acid regurgitation, and environmental allergies. In both cases and normals, there is a wide age range in presenting symptoms. The presence of dysphagia and food impaction, while more common in cases, does not distinguish cases from normals. With regard to the frequency of other diseases, cases were more likely than normals and intermediates to have food allergies. Asthma was equally present in both cases and normals.
T1128 Measurement of Macrolipase Fraction in Children with Elevated Lipase Using Polyethylene Glycol Precipitation Prateek D. Wali, Zhaoping He, Amy S. Gutowski, Karoly Horvath Background: Macroenzymes are formed in the serum by self-polymerization or binding to proteins, mainly immunoglobulin. Their high molecular mass can lead to delayed renal elimination and accumulation in the serum, while maintaining their enzyme activity. Elevated levels of macrolipase (ML) may lead to a false diagnosis of pancreatic disease. Polyethylene glycol precipitation (PEG) is a method used to measure macroenzymes and hormones. It has not been reported for detection of macrolipase. Aims: To assess the value of PEG precipitation method in macrolipase measurement. Establish the cut-off values for macrolipasemia. Determine the prevalence of macrolipasemia in children with prolonged elevation in serum lipase and analyze associations with underlying diseases and other laboratory results. Methods: Remnant serum samples with normal and elevated serum lipase activities were collected from the clinical laboratory. The ML fraction was determined with the PEG precipitation method. The lipase activities of untreated and PEG-precipitated specimens were measured by an automated VITROS LIPA Slides System. The difference between the total and PEG-treated activity was considered ML activity. This was expressed as a percentage of the total lipase activity. Results: The control group consisted of 47 children with normal total serum lipase activity. The study group was 14 children with prolonged hyperlipasemia. Their lipase elevation persisted between 9 and 67 days (27 ± 16). In the control group, 10 of the 47 (21.3%) had a measurable ML fraction. These 10 patients had a ML ratio with a mean and SD of 16±8 % and were used to set cut-off values for further analysis. In the study group, 12 (85.7%) of the 14 patients had a ML ratio with a mean and SD of 33±11% (p<0.001). Two of the 10 controls (20%) and 9 of 14 study patients (64.2%) had ML ratio over 24% (>1SD). Two study patients had a ML ratio between 24-31%, while 7 had a higher elevation above 32% (>2SD). In our study an elevation in ML was frequently found in children with scoliosis after spinal fusion repair, malignancy, and autoimmune, renal or liver diseases. Conclusions: Our preliminary study revealed significantly higher ML fractions in children with hyperlipasemia as compared to controls. The PEG precipitation method is a promising test for ML measurement. Further studies to validate these findings are necessary. Measurement of ML activity is clinically important to avoid unnecessary testing, and treatment. T1129 Clinical Course of Pediatric Patients with Primary Sclerosing Cholangitis Folashade A. Jose, Melvin B. Heyman, Linda Ferrell, Ann L. Clark, Philip Rosenthal Background: Primary sclerosing cholangitis (PSC) is a chronic, idiopathic cholestatic disorder of the intra- and extra-hepatic biliary system and is often associated with inflammatory bowel disease (IBD). Few data are available on the natural history of pediatric patients with PSC with or without IBD. Methods: Medical records including liver biopsy, endoscopy,
A-505
AGA Abstracts
AGA Abstracts
Number and Location of Eosinophils and Eosinophil Derived Neurotoxin Staining in Pediatric Eosinophilc Esophagitis (EoE) Rayna Grothe, Brock J. Doubledee, Hirohito Kita, Yvonne Romero, Thomas C. Smyrk, Gail Kephart, Gary Neidich, Felicity Enders