Comparison of PPIs and H_2-receptor antagonists plus prokinetics for dysmotility-like dyspepsia

AIM： To compare efficacy of proton pump inhibitors （PPIs） with H2-receptor antagonists （H2RA$） plus pro- kinetics （Proks） for dysmotility-like symptoms in func- tional dyspepsia （FD）. METHODS： Subjects were randomized to receive openlabel treatment with either rabeprazole 10 mg od （n = 57） or famotidine 10 mg bid plus mosapride 5 mg tid （n = 57） for 4 wk. The primary efficacy endpoint was change （%） from baseline in total dysmotility-like dyspepsia symptom score. The secondary efficacy endpoint was patient satisfaction with treatment. RESULTS： The improvement in dysmotility-like dyspep- sia symptom score on day 28 was significantly greater in the rabeprazole group （22.5% ± 29.2% of baseline） than the famotidine ＋ mosapride group （53.2%±58.6% of baseline, P 〈 0.0001）. The superior benefit of rabeprazole treatment after 28 d was consistent regardless of Helicobacter pylori status. Significantly more subjects in the rabeprazole group were satisfied or very satisfied with treatment on day 28 than in the famotidine ＋ mosapride group （87.7% vs 59.6%, P = 0.0012）. Rabeprazole therapy was the only significant predictor of treatment response （P 〈 0.0001）, defined as a total symptom score improvement ≥ 50%. CONCLUSION： PPI monotherapy improves dysmotil- ity-like symptoms significantly better than H2RAs plus Proks, and should be the treatment of first choice for Japanese FD.

High-resolution colonic manometry and its clinical application in patients with colonic dysmotility: A review

The detailed process and mechanism of colonic motility are still unclear, and colonic motility disorders are associated with numerous clinical diseases. Colonic manometry is considered to the most direct means of evaluating colonic peristalsis. Colonic manometry has been studied for more than 30 years;however, the long duration of the examination, high risk of catheterization, huge amount of real-time data, strict catheter sterilization, and high cost of disposable equipment restrict its wide application in clinical practice. Recently, highresolution colonic manometry (HRCM) has rapidly developed into a major technique for obtaining more effective information involved in the physiology and/or pathophysiology of colonic contractile activity in colonic dysmotility patients. This review focuses on colonic motility, manometry, operation, and motor patterns, and the clinical application of HRCM. Furthermore, the limitations, future directions, and potential usefulness of HRCM in the evaluation of clinical treatment effects are also discussed.

EFFECT OF ERYTHROMYCIN ON GASTRIC DYSMOTILITY AND NEUROENDOCRINE PEPTIDES IN RATS WITH DIABETES MELLITUS

Objective To investigate the effect of erythromycin on the contractive activity of the isolated gastric antrum smooth muscle and somatostatin （SS）, vasoactive intestinal peptide （VIP）, motilin （MTL）, and substance P （SP） in plasma and isolated gastric antrum tissue of diabetes mellitus （DM） rat models. Methods Thirty male Sprague-Dawley rats were divided into three groups： control group （n = 10）, DM group （n = 10）, and erythromycin group （DM models with erythromycin treatment, n = 10）. A single dose of streptozotocin （100 mg/kg, dissolved in 0. I mol/L citric acid buffer, pH4.5） was injected intraperitoneally. After 48 to 72 hours, rats with blood glucose above 16.7 mmol/L and urine glucose level to be （＋＋＋ ） to （＋＋＋＋） over one week were considered successful DM models. The resting tension, mean contractile amplitude and fi＇equency of spontaneous change in isolated longitudinal and circular gastric antrum smooth muscle strips were measured. SS, VIP, MTL, and SP levels in plasma and gastric antrum tissue were measured using radioimmunoassay. Results （1） In the isolated gastric antrum smooth muscle strips, the gastric motility parameters were lower in DM group than those in control group except circular smooth muscle contractile amplitude and longitudinal smooth muscle contractile fi＇equency. The gastric motility parameters were significantly strengthened in erythromycin group, compared with DM group except longitudinal smooth muscle resting tension （P 〈 0.01 ）. （2） Plasma SS, VIP, and MTL concentrations in DM group were higher than those in control （P 〈 0.05）, while the SP level decreased （P 〈 0.05）. In the gastric antrum, SS of DM group was significantly higher than that of control group （P 〈 0.01 ）, while SP and MTL levels were lower than those of control group （P 〈 0.05 and P 〈 0.01, respectively）. However, the level of VIP in gastric antrum tissue did not change among three groups. The plasma level of SS in erythromycin group was higher than that of DM group （P 〈 0.05）. （3） The blood glucose was lower in erythromycin group than DM group （P 〈 0.01 ）. Conclusions Erythromycin has direct effects on contractive activity of gastric smooth muscle in diabetic rats, but there are few effects on neuroendocrine peptides. Gastric-motility disorders in diabetic rats have a correlation with the changes of neuroendocrine peptide levels in plasma and gastric antrum tissue.

Japanese apricot improves symptoms of gastrointestinal dysmotility associated with gastroesophageal reflux disease

AIM: To investigate the effects of Japanese apricot(JA) consumption on gastroesophageal reflux disease(GERD)-related symptoms.METHODS: Participants included individuals living in Minabe-cho,a well-known JA-growing region,who received specific medical check-ups by the local community health service in 2010.GERD-related symptoms were examined in 1303 Japanese individuals using a validated questionnaire,the Frequency Scale for Symptoms of GERD(FSSG),which consists of 7 questions associated with acid reflux symptoms and 5 questions asking about gastrointestinal dysmotility symptoms.Each question was answered using a 4-point scale,with higher scores indicating more severe GERDrelated symptoms.Subjects were divided into two groups according to their intake of dried and pickled JA: daily intake(≥ 1 JA daily)(392 subjects) and none oroccasional intake(< 1 JA daily)(911 subjects).FSSG scores were compared between subjects who consumed JA daily and those who did not.Next,subjects were stratified by age,gender and Helicobacter pylori(H.pylori) status for subanalyses.RESULTS: Those who ate JA daily were significantly older than those who did not(60.6 ± 10.5 years vs 56.0 ± 11.0 years,P < 0.001).Total FSSG scores were significantly lower in subjects with daily JA intake than in those with none or only occasional intake(2.13 ± 3.14 vs 2.70 ± 3.82,P = 0.005).In particular,subjects who consumed JA daily showed significantly improved FSSG dysmotility scores compared with subjects who did not(1.05 ± 1.58 vs 1.46 ± 2.11,P < 0.001).In contrast,the FSSG reflux score did not differ between subjects with and without daily intake of JA(1.08 ± 1.90 vs 1.24 ± 2.11,P = 0.177).Subanalysis indicated that improvement in dysmotility by JA intake was specifically observed in non-elderly(1.24 ± 1.68 vs 1.62 ± 2.22,P = 0.005) and H.pylori-negative subjects(0.99 ± 1.58 vs 1.57 ± 2.06,P < 0.001).GERD patients(total FSSG score ≥ 8) were less frequently observed among subjects with daily intake of JA as compared to those without daily intake of JA(6.1% vs 9.7%,P = 0.040).CONCLUSION: Daily JA intake may improve digestive dysmotility symptoms,resulting in relief of GERD symptoms.The effect is more obvious in non-elderly and H.pylori-negative subjects.

Is there a pathologic basis for gastrointestinal dysmotility?

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Water-jet vs traditional triangular tip knife in peroral endoscopic myotomy for esophageal dysmotility:A systemic review and metaanalysis

BACKGROUND Peroral endoscopic myotomy is an increasingly used less invasive modality to treat esophageal dysmotility.Recently,triangular tip knife with integrated water jet function has been introduced to mitigate multiple instrument exchanges.AIM To compare traditional triangular tip knife and water jet knife in terms of procedural success,duration,instrument exchanges,coagulation forceps use,and adverse events.METHODS We conducted a systemic review and meta-analysis with two authors independently in electronic databases(PubMed,Embase,and Cochrane Library)from inception through May 2021.In addition,we conducted a relevant search by Reference Citation Analysis(RCA)(https://www.referencecitationanalysis.com).A fixed-effects model was used to calculate weighted mean,odds ratio(OR),and confidence intervals(CI).RESULTS We included 7 studies involving 558 patients.Triangular knife and water jet knife were similar in odds of procedural success with ratio of 4.78(95%CI=0.22-102.47)and odds of clinical success with ratio of 0.93(95%CI=0.29-2.97),respectively.Water jet knife had fewer instrument exchanges compared to triangular knife(2.21,95%CI=1.98-2.45 vs 11.9,95%CI=11.15-12.70)and usage of coagulation forceps(1.75,95%CI=1.52-1.97 vs 2.63,95%CI=2.37-2.89).Adverse events were higher in triangular knife group(OR:2.30,95%CI=1.35-3.95).CONCLUSION Peroral endoscopic myotomy using water jet knife is comparable in terms of pro cedural success to triangular tip knife.Water jet knife also required shorter procedural duration,less instrument exchanges,coagulation devices,and overall adverse events.

Hirschsprung's disease associated enterocolitis:A comprehensive review

Hirschsprung’s disease(HSCR)is a congenital disorder characterized by failure of the neural crest cells to migrate and populate the distal bowel during gestation affecting different lengths of intestine leading to a distal functional obstruction.Surgical treatment is needed to correct HSCR once the diagnosis is confirmed by demonstrating the absence of ganglion cells or aganglionosis of the affected bowel segment.Hirschsprung’s disease associated enterocolitis(HAEC)is an inflammatory complication associated with HSCR that can present either in the pre-or postoperative period and associated with increased morbidity and mortality.The pathogenesis of HAEC remains poorly understood,but intestinal dysmotility,dysbiosis and impaired mucosal defense and intestinal barrier function appear to play a significant role.There is no clear definition for HAEC,but the diagnosis is primarily clinical,and treatment is guided based on severity.Here,we aim to provide a comprehensive review of the clinical presentation,etiology,pathophysiology,and current therapeutic options for HAEC.

Gastrointestinal and nutritional care in pediatric neuromuscular disorders

Neuromuscular diseases(NMDs)affect the development and growth of the neuromuscular system in children.The pathology can occur anywhere along the neuromuscular pathway,from the brain to the nerves to the muscle fibers.These diseases have a profound impact on the quality of life not only of children but also of their families.The predominant manifestation in NMDs is hypotonia,which leads to muscle weakness and fatigue,reduced mobility,and decreased physical performance.However,multiple organ systems can be affected,with resulting orthopedic,cardiac,infectious,respiratory,and nutritional problems.Children with NMD present an increased risk for several dietary and feeding difficulties because of their neuromuscular diagnosis,presentation,and severity.These problems include chronic gastrointestinal issues(constipation,dysphagia,gastroesophageal reflux,and diarrhea),dysphagia,malnutrition,and body composition alterations.As a result,compared to the overall pediatric population,infants and children with NMD are more likely to be malnourished,ranging from failure to thrive to overweight or obesity.Disease-specific guidelines vary in level of detail and recommendations for dietary management.Overall,nutritional data available are sparse,with the exception of Duchenne muscular dystrophy,spinal muscular atrophy,and congenital muscular dystrophy.The purpose of this review is to describe the spectrum of nutritional challenges in children with NMD and to summarize the main dietary and gastrointestinal recommendations for each neuromuscular disorder to provide guidance for daily clinical practice.

Is irritable bowel syndrome an organic disorder?

Irritable bowel syndrome(IBS) is a common gastrointestinal disorder that is generally considered to be functional because there appears to be no associated anatomical defect. Stress and psychological factors are thought to play an important role in IBS. The gut neuroendocrine system(NES),which regulates all functions of the gastrointestinal tract,consists of endocrine cells that are scattered among the epithelial cells of the mucosa,and the enteric nervous system. Although it is capable of operating independently from the central nervous system(CNS),the gut NES is connected to and modulated by the CNS. This review presents evidence for the presence of an anatomical defect in IBS patients,namely in the gastrointestinal endocrine cells. These cells have specialized microvilli that project into the lumen and function as sensors for the luminal content and respond to luminal stimuli by releasing hormones into the lamina propria,which starts a chain reaction that progresses throughout the entire NES. The changes in the gastrointestinal endocrine cells observed in IBS patients are highly consistent with the other abnormalities reported in IBS patients,such as visceral hypersensitivity,dysmotility,and abnormal secretion.

Pathophysiology of constipation in the older adult

This review provides information on the definition of constipation, normal continence and defecation and a description of the pathophysiologic mechanisms of constipation. In addition, changes in the anatomy and physiology of the lower gastrointestinal tract associated with aging that may contribute to constipation are described. MEDLINE (1966-2007) and CINAHL (1980-2007) were searched. The following MeSH terms were used: constipation/etiology OR constipation/ physiology OR constipation/physiopathology) AND (age factors OR aged OR older OR 80 and over OR middle age). Constipation is not well defined in the literature. While self-reported constipation increases with age, findings from a limited number of clinical studies that utilized objective measures do not support this association. Dysmotility and pelvic floor dysfunction are important mechanisms associated with constipation. Changes in GI function associated with aging appear to be relatively subtle based on a limited amount of conflicting data. Additional research is warranted on the effects of aging on GI function, as well as on the timing of these changes.

Assessment of the cardiovascular and gastrointestinal autonomic complications of diabetes

The global prevalence of diabetes mellitus is increasing; arguably as a consequence of changes in diet, lifestyle and the trend towards urbanization. Unsurprisingly, the incidence of both micro and macrovascular complications of diabetes mirrors this increasing prevalence. Amongst the complications with the highest symptom burden, yet frequently under-diagnosed and sub-optimally treated, is diabetic autonomic neuropathy, itself potentially resulting in cardiovascular autonomic neuropathy and gastrointestinal(GI) tract dysmotility. The aims of this review are fourfold. Firstly to provide an overview of the pathophysiological processes that cause diabetic autonomic neuropathy. Secondly, to discuss both the established and emerging cardiometric methods for evaluating autonomic nervous system function in vivo. Thirdly, to examine the tools for assessing pan-GI and segmental motility and finally, we will provide the reader with a summary of putative non-invasive biomarkers that provide a pathophysiological link between lowgrade neuro inflammation and diabetes, which may allow earlier diagnosis and intervention, which in future may improve patient outcomes.

Critically ill patients and gut motility: Are we addressing it?

Gastrointestinal(GI) dysmotility is a common problemin the critically ill population. It can be a reflection and an early sign of patient deterioration or it can be an independent cause of morbidity and mortality. GI dysmotility can be divided for clinical purposes on upper GI dysmotility and lower GI dysmotility. Upper GI dysmotility manifests by nausea, feeding intolerance and vomiting; its implications include aspiration into the airway of abdominal contents and underfeeding. Several strategies to prevent and treat this condition can be tried and they include prokinetics and post-pyloric feeds. It is important to note that upper GI dysmotility should be treated only when there are clinical signs of intolerance(nausea, vomiting) and not based on measurement of gastric residual volumes. Lower GI dysmotility manifests throughout the spectrum of ileus and diarrhea. Ileus can present in the small bowel and the large bowel as well. In both scenarios the initial treatment is correction of electrolyte abnormalities, avoiding drugs that can decrease motility and patient mobilization. When this fails, in the case of small bowel ileus, lactulose and polyethylene glycol solutions can be useful. In the case of colonic pseudo obstruction, neostigmine, endoscopic decompression and cecostomy can be tried when the situation reaches the risk of rupture. Diarrhea is also a common manifestation of GI dysmotility and the most important step is to differentiate between infectious sources and non-infectious sources.

Esophageal manifestation in patients with scleroderma

The esophagus is the most commonly affected part of the gastrointestinal system in patients with systemic sclerosis(SSc).Esophageal involvement may lead to a significant reduction in patient quality of life.The exact pathophysiology is complex and not yet fully elucidated.Ultimately,esophageal smooth muscle becomes atrophied and replaced by fibrous tissue leading to severe motility disturbance of the distal esophagus.Symptoms are mainly attributed to gastroesophageal reflux disease and to esophageal dysmotility.Compelling evidence has correlated esophageal involvement to the severity of pulmonary disease.No formed guidelines exist about the diagnostic modalities used to assess esophageal disease in patients with SSc,though upper gastrointestinal endoscopy is the first and most important modality used as it can reveal alterations commonly observed in patients with SSc.Further exploration can be made by high resolution manometry and pH-impedance study.Proton pump inhibitors remain the mainstay of treatment,while prokinetic agents are commonly used as add-on therapy in patients with symptoms attributed to gastroesophageal reflux disease not responding to standard therapy as well as to motility disturbances.Gastroesophageal reflux disease symptoms in patients with SSc are frequently difficult to manage,and new therapeutic modalities are emerging.The role of surgical treatment is restricted and should only be preserved for resistant cases.

Dysphagia lusoria: A late onset presentation

Dysphagia lusoria is a term used to describe dysphagia secondary to vascular compression of the oesophagus. The various embryologic anomalies of the arterial brachial arch system often remain unrecognised and asymptomatic, but in 30%-40% of cases can result in tracheo-oesophageal symptoms, which in the majority of cases manifest as dysphagia. Diagnosis of dysphagia lusoria is via barium swallow and chest Computed tomography scan. Manometric abnormalities are variable, but age-related manometric changes may contribute to clinically relevant dysphagia lusoria in patients who present later in life. Our report describes a case of lateonset dysphagia secondary to a right aortic arch with an aberrant left subclavian artery, which represents a rare variant of dysphagia lusoria. The patient had proven additional oesophageal dysmotility with solid bolus only and a clinical response to dietary modification.

Motility index measured by magnetic resonance enterography is associated with sex and mural thickness

BACKGROUND Recently,a technique has been developed to use magnetic resonance enterography(MRE)for the evaluation of small bowel motility.The hypothesis was that assessment of the motility index(MI)should reflect differences in motility between clinical conditions.AIM To aim of the present observational,cross-sectional study was to evaluate the use of the MI in daily clinical practice.METHODS All consecutive patients aged 18-70 years who were referred for MRE at the Department of Radiology during a 2-year period were asked to participate.Healthy volunteers were included as controls.MRE was prepared and conducted in accordance with clinical routines.On the day of examination,all the participants had to complete the visual analog scale for irritable bowel syndrome(IBS)and IBS-symptom severity scale.Maps of MI were calculated from dynamic MR images.ANOVA was used to evaluate differences in MI between groups,classified as healthy,Crohn’s disease,ulcerative colitis,IBS,other assorted disorders and dysmotility.Logistic and linear regression were applied to the MI values.All medical records were scrutinized for medical history.RESULTS In all,224 examinations were included(inclusion prevalence 76.3%),with 22 controls and 202 patients.There was a significant difference in the MI of the jejunum(P=0.021)and terminal ileum(P=0.007)between the different groups.The MI was inversely associated with the mural thickness of the terminal ileum in men(P<0.001)and women(P=0.063)after adjustments,and tended to be lower in men than in women(P=0.056).Subjectively observed reduction of motility on MRI was accomplished by reduced MI of terminal ileum in men(P<0.001)and women(P=0.030).In women,diarrhea was inversely associated with the MI of the jejunum(P=0.029),and constipation was positively associated with the MI of the terminal ileum(P=0.039).CONCLUSION Although MIs differ across diseases,a lower MI of the terminal ileum is mainly associated with male sex and an increased mural thickness.Symptoms are weakly associated with the MI.

Lymphocytic esophagitis:Report of three cases and review of the literature

Lymphocytic esophagitis(LyE) is a rare condition characterised histologically by high numbers of esophageal intraepithelial lymphocytes without significant granulocytes infiltration, in addition to intercellular edema("spongiosis"). The clinical significance and natural history of LyE is poorly defined although dysphagia is reportedly the most common symptom. Endoscopic features range from normal appearing esophageal mucosa to features similar to those seen in eosinophilic esophagitis, including esophageal rings, linear furrows, whitish exudates, and esophageal strictures/stenosis. Symptomatic gastroesophageal reflux disease is an inconsistent association. LyE has been associated in paediatric Crohn's disease, and recently in primary esophageal dysmotility disorder in adults. There are no studies assessing effective treatment strategies for LyE; empirical therapies have included use of proton pump inhibitor and corticosteroids. Esophageal dilatation have been used to manage esophageal strictures. Ly E has been reported to run a benign course; however there has been a case of esophageal perforation associated with Ly E. Here, we describe the clinical, endoscopic and histopathological features of three patients with lymphocytic esophagitis along with a review of the current literature.

Spectrum of esophageal motility disorders in patients with liver cirrhosis

Disorders of esophageal motility have been described in patients with cirrhosis in a small number of studies.In this review,we aim to provide an overview of the available evidence on esophageal motility disorders in cirrhosis and their clinical implications.This review delves into the following concepts:(1)Gastroesophageal reflux disease is common in liver cirrhosis due to many mechanisms;however,when symptomatic it is usually nocturnal and has an atypical presentation;(2)Endoscopic band ligation is better than sclerotherapy in terms of its effect on esophageal motility and seems to correct dysmotilities resulting from the mechanical effect of esophageal varices;(3)Chronic alcoholism has no major effects on esophageal motility activity other than lower esophageal sphincter hypertension among those with alcoholic autonomic neuropathy;(4)An association between primary biliary cholangitis and scleroderma can be present and esophageal hypomotility is not uncommon in this scenario;and(5)Cyclosporin-based immunosuppression in liver transplant patients can have a neurotoxic effect on the esophageal myenteric plexus leading to reversible achalasia-like manifestations.

Gastrointestinal amyloidosis: A focused review

Amyloidosis,a heterogenous group of disorders,is characterized by the extracellular deposition of autologous,insoluble,fibrillar misfolded proteins.These extracellular proteins deposit in tissues aggregated inß-pleated sheets arranged in an antiparallel fashion and cause distortion to the tissue architecture and function.In the current literature,about 60 heterogeneous amyloidogenic proteins have been identified,out of which 27 have been associated with human disease.Classified as a rare disease,amyloidosis is known to have a wide range of possible etiologies and clinical manifestations.The exact incidence and prevalence of the disease is currently unknown.In both systemic and localized amyloidosis,there is infiltration of the abnormal proteins in the layers of the gastrointestinal(GI)tract or the liver parenchyma.The gold standard test for establishing a diagnosis is tissue biopsy followed by Congo Red staining and apple-green birefringence of the Congo Red-stained deposits under polarized light.However,not all patients may have a positive tissue confirmation of the disease.In these cases additional workup and referral to a gastroenterologist may be warranted.Along with symptomatic management,the treatment for GI amyloidosis consists of observation or localized surgical excision in patients with localized disease,and treatment of the underlying pathology in cases of systemic amyloidosis.In this review of the literature,we describe the subtypes of amyloidosis,with a primary focus on the epidemiology,pathogenesis,clinical features,diagnosis and treatment strategies available for GI amyloidosis.

Constipation,deficit in colon contractions and alpha-synuclein inclusions within the colon precede motor abnormalities and neurodegeneration in the central nervous system in a mouse model of alphasynucleinopathy

Background:Gastrointestinal dysfunction can affect Parkinson’s disease(PD)patients long before the onset of motor symptoms.However,little is known about the relationship between gastrointestinal abnormalities and the development of PD.Contrary to other animal models,the human A53T alpha-synuclein(αS)transgenic mice,Line G2–3,developsαS-driven neurological and motor impairments after 9 months of age,displaying a long presymptomatic phase free of central nervous system(CNS)dysfunction.Methods:To determine whether this line can be suitable to study constipation as it occurs in prodromal PD,gastrointestinal functionality was assessed in young mice through a multidisciplinary approach,based on behavioral and biochemical analysis combined with electrophysiological recordings of mouse intestinal preparations.Results:We found that the A53TαS mice display remarkable signs of gastrointestinal dysfunction that precede motor abnormalities andαS pathology in the CNS by at least 6 months.YoungαS mice show a drastic delay in food transit along the gastrointestinal tract,of almost 2 h in 3 months old mice that increased to more than 3 h at 6 months.Such impairment was associated with abnormal formation of stools that resulted in less abundant but longer pellets excreted,suggesting a deficit in the intestinal peristalsis.In agreement with this,electrically evoked contractions of the colon,but not of the ileum,showed a reduced motor response in both longitudinal and circular muscle layers inαS mice already at 3 months of age,that was mainly due to an impaired cholinergic transmission of the underlying enteric nervous system.Interestingly,the presence of insoluble and aggregatedαS was found in enteric neurons in both myenteric and submucosal plexi only in the colon of 3 months oldαS mice,but not in the small intestine,and exacerbated with age,mimicking the increase in transit delay and the contraction deficit showed by behavioral and electrical recordings data.Conclusions:Gastrointestinal dysfunction in A53TαS mice represents an early sign ofαS-driven pathology without concomitant CNS involvement.We believe that this model can be very useful to study disease-modifying strategies that could extend the prodromal phase of PD and haltαS pathology from reaching the brain.

Long-term esophageal and respiratory outcomes in children with esophageal atresia and tracheoesophageal fistula

Objectives:Few studies have evaluated the long-term complications and outcomes of esophageal atresia with or without tracheoesophageal fistula(EA/TEF)beyond childhood.The aim of our study was to characterize the esophageal and respiratory morbidity of EA/TEF through evaluation of clinical symptoms,diagnostic testing and therapeutic intervention at a tertiary care center.Methods:Patients with congenital EA/TEF evaluated from 2011 to 2014 were included.Demographic characteristics,type and mode of repair of EA/TEF,clinical symptoms,radiographic,endoscopic,bronchoscopic and medication use data were obtained.Results:A total of 43 patients were identified.The median age of this predominantly Caucasian population was 8 years(interquartile range:3,20).Twenty(62.5%)had type C(EA with distal TEF)abnormality.Twenty-one(48.8%)patients had heartburn,19(44.1%)had acid regurgitation,and 31(72.1%)had dysphagia to solids.Barium swallow in 26 patients revealed strictures in 17(65.4%),dysmotility in 20(76.9%)and recurrent fistulas in four patients(15.4%).Thirty patients underwent upper endoscopy,of which 21(70.0%)had a stricture,and six(20.0%)had recurrent fistula requiring surgical intervention.Eight(18.6%)patients underwent fundoplication.Pulmonary evaluation showed cough and choking in 31(72.1%)patients and dyspnea and wheezing in 32(53.4%)patients.Recurrent respiratory infections were reported in 19(44.2%).patients.Other findings included tracheomalacia in 86.7% and restrictive lung disease in 54.5%of patients.Conclusion:There is a high burden of residual esophageal and pulmonary pathology in patients with EA/TEF.Ongoing follow-up is required to monitor both the clinical symptoms and treatment responses.