Objectives: Colonic manometry is a test used in the evaluation of children with defecation disorders unresponsive to conventional treatment. The most commonly reported protocol in pediatrics consists of a study that lasts approximately 4 hours. Given the wide physiological variations in colonic motility throughout the day, longer observation may detect clinically relevant information. The aim of the present study was to compare prolonged colonic manometry studies in children referred for colonic manometry with the more traditional short water-perfused technology.Methods: Colonic manometry studies of 19 children (8 boys, mean age 9.4 +/- 0.9, range 3.9-16.3) with severe defecation disorders were analyzed. First, a "standard test" was performed with at least 1-hour fasting, 1-hour postprandial, and 1-hour postbisacodyl provocation recording. Afterwards, recordings continued until the next day.Results: In 2 of the 19 children, prolonged recording gave us extra information. In 1 patient with functional nonretentive fecal incontinence who demonstrated no abnormalities in the short recording, 2 long clusters of high-amplitude contractions were noted in the prolonged study, possibly contributing to the fecal incontinence. In another patient evaluated after failing use of antegrade enemas through a cecostomy, short recordings showed colonic activity only in the most proximal part of the colon, whereas the prolonged study showed normal motility over a larger portion of the colon.Conclusions: Prolonged colonic measurement provides more information regarding colonic motor function and allows detection of motor events missed by the standard shorter manometry study.

BACKGROUND:Solid-state (SS) manometry catheters with portable data loggers offer many potential advantages over traditional water-perfused (WP) systems, such as prolonged recordings in a more physiologic ambulatory setting and the lack of risk for water overload. The use of SS catheters has not been evaluated in comparison with perfused catheters in children. This study aims to compare data provided by SS and WP catheters in children undergoing colonic manometry studies. METHODS:A SS catheter and a WP catheter were taped together such that their corresponding sensors were at the same location. Simultaneous recordings were obtained using the SS and WP catheters (both 8 channels, 10 cm apart) in 15 children with severe defecation disorders referred for colonic manometry. Signals were recorded for a minimum of 1 h during fasting, 1 h after ingestion of a meal, and 1 h after the administration of bisacodyl. Solid-state signals from the data logger were analyzed against the perfused signals. All high-amplitude propagated contractions (HAPCs), the most recognizable and interpreted colonic motor event, were evaluated for spatial and temporal features including their durations, amplitudes, and propagation velocities. KEY RESULTS:A total of 107 HAPCs were detected with SS and 91 with WP catheters. All WP-HAPC were also observed with SS. Linear regression analysis showed that SS catheters tended to give higher readings in the presence of amplitudes <102 mmHg and lower reading with amplitudes >102 mmHg. An opposite trend was found for the duration of contractions. No significant difference was found for HAPC velocity. CONCLUSIONS & INFERENCES:SS catheters are more sensitive in recording HAPCs in children with defecation disorders compared with the more traditional WP assembly. There is a difference in measurements of amplitude between the two systems. Solid-state catheters offer potential advantages over WP catheters in children, being portable, safer to use, and may provide data over a more prolonged period.

Objectives: Quality of life (QOL) is reportedly poor in children with Crohn disease (CD) but improves with increasing disease duration. This article aims to detail QOL in a cohort of Australian children with CD in relation to disease duration, disease activity, and treatment.Materials and Methods: QOL, assessed using the IMPACT-III questionnaire, and disease activity measures, assessed using the Pediatric Crohn's Disease Activity Index (PCDAI), were available in 41 children with CD. For this cohort, a total of 186 measurements of both parameters were available.Results: QOL was found to be significantly lower, and disease activity significantly higher (F = 31.1, P = 0.00), in patients within 6 months of their diagnosis compared with those up to 2.5 years, up to 5 years, and beyond 5 years since diagnosis. Higher disease activity was associated with poorer QOL (r = -0.51, P 0.00). Total QOL was highest in children on nil medications and lowest in children on enteral nutrition. The PCDAI (t = -6.0, P = 0.00) was a significant predictor of QOL, with the clinical history (t = -6.9, P = 0.00) and examination (t = -2.9, P = 0.01) sections of the PCDAI significantly predicting QOL. Disease duration, age, or sex was neither related to nor significant predictors of QOL, but height z score and type of treatment approached significance.Conclusions: Children with CD within 6 months of their diagnosis have impaired QOL compared with those diagnosed beyond 6 months. These patients, along with those with growth impairment, ongoing elevated disease activity with abdominal pain, diarrhoea and/ or perirectal and extraintestinal complications, may benefit from regular assessments of QOL as part of their clinical treatment.

OBJECTIVE:There are no objective ambulatory studies on the temporal relationship between reflux and cough in children. Commercial pHmetry loggers have slow capture rates (0.25 Hz) that limit objective quantification of reflux and cough. The authors aimed to evaluate if there is a temporal association between cough and acid pH in ambulatory children with chronic cough. DESIGN, SETTING AND PATIENTS:The authors studied children (aged <14 years) with chronic cough, suspected of acid reflux and considered for pHmetry using a specifically built ambulatory pHmetry-cough logger that enabled the simultaneous ambulatory recording of cough and pH with a fast (10 Hz) capture rate. MAIN OUTCOME MEASURES:Coughs within (before and after) 10, 30, 60 and 120 s of a reflux episode (pH<4 for >0.5 s). RESULTS:Analysis of 5628 coughs in 20 children. Most coughs (83.9%) were independent of a reflux event. Cough-reflux (median 19, IQR 3-45) and reflux-cough (24.5, 13-51) sequences were equally likely to occur within 120 s. Within the 10 and 30 s time frame, reflux-cough (10 s=median 2.5, IQR 0-7.25; 30 s=6.5, 1.25-22.25) sequences were significantly less frequent than reflux-no cough (10 s=27, IQR 15-65; 30 s=24.5, 14.5-55.5) sequences, (p=0.0001 and p=0.001, respectively). No differences were found for 60 and 120 s time frame. Cough-reflux sequence (median 1.0, IQR 0-8) within 10 s was significantly less (p=0.0001) than no cough-reflux sequences (median 29.5, 15-67), within 30 s (p=0.006) and 60 s (p=0.048) but not within 120 s (p=0.47). CONCLUSIONS:In children with chronic cough and suspected of having gastro-oesophageal reflux disease, the temporal relationship between acid reflux and cough is unlikely causal.

The spectrum of motility disorders ranges from relatively benign conditions such as gastroesophageal reflux and functional dyspepsia to life-threatening illnesses such as chronic intestinal pseudo-obstruction (CIPO). Motility disorders account for up to 15% of pediatric patients with intestinal failure.1Guarino A. De Marco G. Italian National Network for Pediatric Intestinal FailureNatural history of intestinal failure, investigated through a national network-based approach.J Pediatr Gastroenterol Nutr. 2003; 37: 136-141Crossref PubMed Scopus (68) Google Scholar In other more common causes of intestinal failure, such as short gut syndrome, abnormal motility also plays an important role in determining whether patients will be able to be weaned from parenteral nutrition. Pseudo-obstruction represents the most severe form of motility disorder and may be considered an insufficiency of the intestinal pump, very much like heart failure is caused by an insufficiency of the cardiac pump. Although this review focuses mostly on CIPO, much of the information about pathophysiology and diagnostic and therapeutic approaches is applicable to other less severe forms of motility disorders. Pseudo-obstruction may be congenital or acquired, primary or secondary (Table 1).2Rudolph C.D. Hyman P.E. Altschuler S.M. Christensen J. Colletti R.B. Cucchiara S. Di Lorenzo C. Flores A.F. Hillemeier A.C. McCallum R.W. Vanderhoof J.A. Diagnosis and treatment of chronic intestinal pseudo-obstruction in children report of consensus workshop.J Pediatr Gastroenterol Nutr. 1997; 24: 102-112Crossref PubMed Scopus (161) Google Scholar In most pediatric cases, symptoms are present from birth or early infancy.3Heneyke S. Smith V.V. Spitz L. Milla P.J. Chronic intestinal pseudo-obstruction treatment and long term follow up of 44 patients.Arch Dis Child. 1999; 81: 21-27Crossref PubMed Scopus (133) Google Scholar Regardless of the underlying cause, 2 main groups can be identified based on histopathology and patterns of motility abnormalities: visceral myopathy and visceral neuropathy. Neuropathic disorders are more common and may be primary (sometimes familial) or secondary (eg, in utero insults such as fetal alcohol syndrome or postnatal injuries such as ischemic events or viral infections). Mitochondrial disorders such as mitochondrial neurogastroencephalopathy also may be complicated by neuropathic pseudo-obstruction,4Teitelbaum J.E. Berde C.B. Nurko S. Buonomo C. Perez-Atayde A.R. Fox V.L. Diagnosis and management of MNGIE syndrome in children case report and review of the literature.J Pediatr Gastroenterol Nutr. 2002; 35: 377-383Crossref PubMed Scopus (55) Google Scholar with the gastrointestinal symptoms often preceding the neurologic dysfunction. Recently, abnormalities of the gastrointestinal pacemaker cells, the interstitial cells of Cajal, have been described in patients with motility disorders.5Streutker C.J. Huizinga J.D. Campbell F. Ho J. Riddell R.H. Loss of CD117 (c-kit)- and CD34-positive ICC and associated CD34-positive fibroblasts defines a subpopulation of chronic intestinal pseudo-obstruction.Am J Surg Pathol. 2003; 27: 228-235Crossref PubMed Scopus (83) Google Scholar, 6Jain D. Moussa K. Tandon M. Culpepper-Morgan J. Proctor D.D. Role of interstitial cells of Cajal in motility disorders of the bowel.Am J Gastroenterol. 2003; 98: 618-624Crossref PubMed Scopus (112) Google Scholar, 7Isozaki K. Hirota S. Miyagawa J. Taniguchi M. Shinomura Y. Matsuzawa Y. Deficiency of c-kit+ cells in patients with a myopathic form of chronic idiopathic intestinal pseudo-obstruction.Am J Gastroenterol. 1997; 92: 332-334PubMed Google Scholar, 8Kenny S.E. Vanderwinden J.M. Rintala R.J. Connell M.G. Lloyd D.A. Vanderhaegen J.J. De Laet M.H. Delayed maturation of the interstitial cells of Cajal: a new diagnosis for transient neonatal pseudo-obstruction. Report of two cases.J Pediatr Surg. 1998; 33: 94-98Abstract Full Text PDF PubMed Scopus (123) Google Scholar Functionally, these patients may have features of both myopathy and neuropathy. Motility problems also may complicate structural gastrointestinal abnormalities. Patients with a history of malrotation, atresia, enterocolitis, or gastroschisis repair frequently have abnormal motility in the remaining gut, limiting tolerance of enteral feeds even when the length of residual small bowel apparently is sufficient.3Heneyke S. Smith V.V. Spitz L. Milla P.J. Chronic intestinal pseudo-obstruction treatment and long term follow up of 44 patients.Arch Dis Child. 1999; 81: 21-27Crossref PubMed Scopus (133) Google Scholar Animal studies have shown that ischemic insults and exposure to amniotic fluid have additive deleterious effects on gastrointestinal motility, with the potential to cause long-term dysfunction.9Srinathan S.K. Langer J.C. Blennerhassett M.G. Harrison M.R. Pelletier G.J. Lagunoff D. Etiology of intestinal damage in gastroschisis. III: morphometric analysis of the smooth muscle and submucosa.J Pediatr Surg. 1995; 30: 379-383Abstract Full Text PDF PubMed Scopus (59) Google Scholar, 10Langer J.C. Bell J.G. Castillo R.O. Crombleholme T.M. Longaker M.T. Duncan B.W. Bradley S.M. Finkbeiner W.E. Verrier E.D. Harrison M.R. Etiology of intestinal damage in gastroschisis, II. Timing and reversibility of histological changes, mucosal function, and contractility.J Pediatr Surg. 1990; 25: 1122-1126Abstract Full Text PDF PubMed Scopus (116) Google Scholar, 11Langer J.C. Longaker M.T. Crombleholme T.M. Bond S.J. Finkbeiner W.E. Rudolph C.A. Verrier E.D. Harrison M.R. Etiology of intestinal damage in gastroschisis. I: Effects of amniotic fluid exposure and bowel constriction in a fetal lamb model.J Pediatr Surg. 1989; 24: 992-997Abstract Full Text PDF PubMed Scopus (191) Google Scholar, 12Oyachi N. Lakshmanan J. Ross M.G. Atkinson J.B. Fetal gastrointestinal motility in a rabbit model of gastroschisis.J Pediatr Surg. 2004; 39: 366-370Abstract Full Text Full Text PDF PubMed Scopus (23) Google Scholar Damage is progressive throughout gestation.10Langer J.C. Bell J.G. Castillo R.O. Crombleholme T.M. Longaker M.T. Duncan B.W. Bradley S.M. Finkbeiner W.E. Verrier E.D. Harrison M.R. Etiology of intestinal damage in gastroschisis, II. Timing and reversibility of histological changes, mucosal function, and contractility.J Pediatr Surg. 1990; 25: 1122-1126Abstract Full Text PDF PubMed Scopus (116) Google Scholar However, in human beings, deliberate premature delivery of fetuses affected by gastroschisis has not been found to be beneficial.13Simmons M. Georgeson K.E. The effect of gestational age at birth on morbidity in patients with gastroschisis.J Pediatr Surg. 1996; 31: 1060-1062Abstract Full Text PDF PubMed Scopus (37) Google ScholarTable 1Causes of Chronic Intestinal Pseudo-obstructionMuscle Disorders Myotonic dystrophy Duchenne muscular dystrophyPostoperative Small bowel ileus Colonic pseudo-obstructionAutoimmune Generalized SLE Scleroderma Dermatomyositis Polymyositis Celiac disease Gastrointestinal Autoimmune myositis Autoimmune ganglionitisOncology/haematology Chemotherapy and/or bone marrow/stem cell transplant Phaeochromocytoma Ganglioneuroblastoma (paraneoplastic syndrome) Small cell carcinoma (paraneoplastic syndrome) Multiple myeloma Sickle cell diseaseInfectious/post-infectious Chagas disease Cytomegalovirus Herpes zoster Epstein Barr virus Kawasaki diseaseEndocrine Diabetes mellitus Hypoparathyroidism HypothyroidismMetabolic Mitochondrial cytopathiesToxins Fetal alcohol syndrome Jellyfish envenomationDrugs Diltiazem and nifedipine Cyclopentolate/phenylephrine eye drops (neonates)Developmental Delayed maturation of interstitial cells of CajalMiscellaneous Ehlers Danlos syndrome Eosinophilic gastroenteritis Angioedema Crohn's disease Radiation injury Open table in a new tab Treatable causes of CIPO are rare but should be considered in every case because of the potential value of specific therapy. In some cases of delayed maturation of the enteric nervous system or of the interstitial cells of Cajal, resolution may be spontaneous.8Kenny S.E. Vanderwinden J.M. Rintala R.J. Connell M.G. Lloyd D.A. Vanderhaegen J.J. De Laet M.H. Delayed maturation of the interstitial cells of Cajal: a new diagnosis for transient neonatal pseudo-obstruction. Report of two cases.J Pediatr Surg. 1998; 33: 94-98Abstract Full Text PDF PubMed Scopus (123) Google Scholar In other conditions such as celiac disease,14Dawson D. Sciberras C. Whitwell J. Coeliac disease presenting with intestinal pseudo-obstruction.Gut. 1984; 25: 1003-1008Crossref PubMed Scopus (29) Google Scholar hypothyroidism,15Bassotti G. Pagliacci M.C. Nicoletti I. Pelli M.A. Morelli A. Intestinal pseudoobstruction secondary to hypothyroidism. Importance of small bowel manometry.J Clin Gastroenterol. 1992; 14: 56-58Crossref PubMed Scopus (33) Google Scholar, 16Abbasi A.A. Douglass R.C. Bissell G.W. Chen Y. Myxedema ileus. A form of intestinal pseudo-obstruction.JAMA. 1975; 234: 181-183Crossref PubMed Scopus (39) Google Scholar Kawasaki disease,17Akikusa J.D. Laxer R.M. Friedman J.N. Intestinal pseudoobstruction in Kawasaki disease.Pediatrics. 2004; 113: e504-e506Crossref PubMed Scopus (28) Google Scholar and cystic fibrosis with meconium ileus or distal intestinal obstruction syndrome, treatment of the underlying disease may improve or reverse severe dysmotility. Pseudo-obstruction has been described in association with DNA viruses such as Herpes simplex, Epstein-Barr virus, and cytomegalovirus.18Sonsino E. Mouy R. Foucaud P. Cezard J.P. Aigrain Y. Bocquet L. Navarro J. Intestinal pseudoobstruction related to cytomegalovirus infection of myenteric plexus.N Engl J Med. 1984; 311: 196-197PubMed Google Scholar, 19Ategbo S. Turck D. Gottrand F. Bonnevalle M. Wattre P. Lecomte-Houcke M. Farriaux J.P. Chronic intestinal pseudo-obstruction associated with cytomegalovirus infection in an infant.J Pediatr Gastroenterol Nutr. 1996; 23: 457-460Crossref PubMed Scopus (16) Google Scholar, 20Debinski H.S. Kamm M.A. Talbot I.C. Khan G. Kangro H.O. Jeffries D.J. DNA viruses in the pathogenesis of sporadic chronic idiopathic intestinal pseudo-obstruction.Gut. 1997; 41: 100-106Crossref PubMed Scopus (90) Google Scholar Such cases of dysmotility after specific viral infections theoretically may respond to antiviral therapy, although clinical data are lacking. Rare cases of autoimmune myositis also have been reported with symptoms of pseudo-obstruction improving with corticosteroid therapy.21Ginies J.L. Francois H. Joseph M.G. Champion G. Coupris L. Limal J.M. A curable cause of chronic idiopathic intestinal pseudo-obstruction in children idiopathic myositis of the small intestine.J Pediatr Gastroenterol Nutr. 1996; 23: 426-429Crossref PubMed Scopus (30) Google Scholar Similarly, some cases of CIPO in children and young adults appear to be caused by autoimmune lymphocytic destruction of myenteric ganglia.22Smith V.V. Gregson N. Foggensteiner L. Neale G. Milla P.J. Acquired intestinal aganglionosis and circulating autoantibodies without neoplasia or other neural involvement.Gastroenterology. 1997; 112: 1366-1371Abstract Full Text PDF PubMed Scopus (151) Google Scholar, 23De Giorgio R. Barbara G. Stanghellini V. De Ponti F. Salvioli B. Tonini M. Velio P. Bassotti G. Corinaldesi R. Clinical and morphofunctional features of idiopathic myenteric ganglionitis underlying severe intestinal motor dysfunction a study of three cases.Am J Gastroenterol. 2002; 97: 2454-2459Crossref PubMed Scopus (97) Google Scholar In these cases, anti-Hu (antineuronal nuclear, ANNA-1) antibodies appear to be a useful serologic marker.22Smith V.V. Gregson N. Foggensteiner L. Neale G. Milla P.J. Acquired intestinal aganglionosis and circulating autoantibodies without neoplasia or other neural involvement.Gastroenterology. 1997; 112: 1366-1371Abstract Full Text PDF PubMed Scopus (151) Google Scholar, 23De Giorgio R. Barbara G. Stanghellini V. De Ponti F. Salvioli B. Tonini M. Velio P. Bassotti G. Corinaldesi R. Clinical and morphofunctional features of idiopathic myenteric ganglionitis underlying severe intestinal motor dysfunction a study of three cases.Am J Gastroenterol. 2002; 97: 2454-2459Crossref PubMed Scopus (97) Google Scholar Cases of eosinophilic myenteric ganglionitis also have been described.24Schappi M.G. Smith V.V. Milla P.J. Lindley K.J. Eosinophilic myenteric ganglionitis is associated with functional intestinal obstruction.Gut. 2003; 52: 752-755Crossref PubMed Scopus (91) Google Scholar Although uncommon, these inflammatory and autoimmune cases are important to identify because they may respond to anti-inflammatory medications.22Smith V.V. Gregson N. Foggensteiner L. Neale G. Milla P.J. Acquired intestinal aganglionosis and circulating autoantibodies without neoplasia or other neural involvement.Gastroenterology. 1997; 112: 1366-1371Abstract Full Text PDF PubMed Scopus (151) Google Scholar, 23De Giorgio R. Barbara G. Stanghellini V. De Ponti F. Salvioli B. Tonini M. Velio P. Bassotti G. Corinaldesi R. Clinical and morphofunctional features of idiopathic myenteric ganglionitis underlying severe intestinal motor dysfunction a study of three cases.Am J Gastroenterol. 2002; 97: 2454-2459Crossref PubMed Scopus (97) Google Scholar, 24Schappi M.G. Smith V.V. Milla P.J. Lindley K.J. Eosinophilic myenteric ganglionitis is associated with functional intestinal obstruction.Gut. 2003; 52: 752-755Crossref PubMed Scopus (91) Google Scholar In CIPO, abdominal radiographs may show dilated loops of small bowel and air-fluid levels, except in patients who are not being fed and have venting enterostomies. Contrast radiology should be performed using water-soluble material to avoid the formation of barium concretions in the colon. Upper gastrointestinal series with small-bowel follow-through studies show dilated loops of bowel with very slow transit through a featureless intestine (Figure 1). Because contrast material often becomes diluted in fluid-filled bowel loops, recognition of mucosal details and detection of partial bowel obstruction may be arduous. Antroduodenal manometry is used to determine the pathophysiology of symptoms in CIPO.25Hyman P.E. McDiarmid S.V. Napolitano J. Abrams C.E. Tomomasa T. Antroduodenal motility in children with chronic intestinal pseudo-obstruction.J Pediatr. 1988; 112: 899-905Abstract Full Text PDF PubMed Scopus (76) Google Scholar Antroduodenal manometry assesses contraction amplitude and spatial and temporal organization of phasic contractions. The presence of normal patterns, such as the migrating motor complex in fasting (Figure 2), and a change to postprandial motility pattern with a test meal indicate intact enteric neuromuscular function. Manometry is useful to distinguish myopathy, in which contraction amplitude is reduced but spatial and temporal organization is preserved (Figure 3), from neuropathy, in which contractions have normal amplitude but are uncoordinated and lack normal physiologic patterns. Intrinsic or visceral neuropathy is characterized by abnormal (Figure 4) or even absent phase III of the migrating motor complex, the most recognizable motor pattern during fasting. A normal motor response to food is dependent on the integrity of both intrinsic and extrinsic neural control systems, and the gastrointestinal smooth muscle. In the presence of normal amplitude contractions, an impaired or even absent motor response to food may occur in both visceral neuropathy and extrinsic autonomic neuropathy.26Wingate D. Small bowel manometry.Am J Gastroenterol. 1995; 90: 536-539PubMed Google Scholar In the presence of severe, long-standing disease with intestinal dilatation, antroduodenal manometry recordings may be nonspecific, with negligible contractile activity detected.Figure 3Manometric tracing from a child with hollow visceral myopathy. Amplitude of contractions is less than 50 mm Hg in the antrum and less than 20 mm Hg in the duodenum.View Large Image Figure ViewerDownload (PPT)Figure 4Manometric tracing from a child with visceral neuropathy. There is evidence of abnormal configuration of a phase III motor migrating complex with some retrograde migration (third recording site from above) and lack of propagation (most distal recording site).View Large Image Figure ViewerDownload (PPT) Antroduodenal manometry also may be used to suggest prognosis27Fell J.M. Smith V.V. Milla P.J. Infantile chronic idiopathic intestinal pseudo-obstruction the role of small intestinal manometry as a diagnostic tool and prognostic indicator.Gut. 1996; 39: 306-311Crossref PubMed Scopus (67) Google Scholar and likely response to treatment.28Hyman P.E. Di Lorenzo C. McAdams L. Flores A.F. Tomomasa T. Garvey 3rd, T.Q. Predicting the clinical response to cisapride in children with chronic intestinal pseudo-obstruction.Am J Gastroenterol. 1993; 88: 832-836PubMed Google Scholar If the migrating motor complex is present, patients are likely to tolerate enteral feeding.29Di Lorenzo C. Flores A.F. Buie T. Hyman P.E. Intestinal motility and jejunal feeding in children with chronic intestinal pseudo-obstruction.Gastroenterology. 1995; 108: 1379-1385Abstract Full Text PDF PubMed Scopus (105) Google Scholar Similarly, the presence of phase III of the migrating motor complex is associated with a favorable response to prokinetic therapy with cisapride.28Hyman P.E. Di Lorenzo C. McAdams L. Flores A.F. Tomomasa T. Garvey 3rd, T.Q. Predicting the clinical response to cisapride in children with chronic intestinal pseudo-obstruction.Am J Gastroenterol. 1993; 88: 832-836PubMed Google Scholar In patients showing symptoms of intestinal pseudo-obstruction, the presence of normal manometry studies should lead to the consideration of emotional or factitious disorders.30Hyman P.E. Bursch B. Beck D. DiLorenzo C. Zeltzer L.K. Discriminating pediatric condition falsification from chronic intestinal pseudo-obstruction in toddlers.Child Maltreatment. 2002; 7: 132-137Crossref PubMed Scopus (43) Google Scholar, 31Hyman P.E. Chronic intestinal pseudo-obstruction.in: Hyman P.E. Pediatric gastrointestinal motility disorders. Volume 1. Academy Professional Information Services, Inc, New York1994: 115-128Google Scholar Frequently, the severity of motility dysfunction varies throughout the gastrointestinal tract. Motility studies may identify areas with preserved motility and different feeding strategies then may be devised, bypassing the affected segments. For example, if the stomach is more affected than the small bowel, gastrojejunal or jejunostomy feedings may be better tolerated.29Di Lorenzo C. Flores A.F. Buie T. Hyman P.E. Intestinal motility and jejunal feeding in children with chronic intestinal pseudo-obstruction.Gastroenterology. 1995; 108: 1379-1385Abstract Full Text PDF PubMed Scopus (105) Google Scholar Motility studies of the rest of the gut, including colonic manometry and/or radiopaque marker studies, may show distal motility impairment. In such cases, a defunctioning ileostomy may decompress the gut, increasing the ability to receive enteral feeds. When both the upper and lower gastrointestinal tract are diseased, a colectomy is less likely to be beneficial,32Glia A. Akerlund J.E. Lindberg G. Outcome of colectomy for slow-transit constipation in relation to presence of small-bowel dysmotility.Dis Colon Rectum. 2004; 47: 96-102Crossref PubMed Scopus (70) Google Scholar although we believe that abnormal foregut motility is not an absolute contraindication to placement of an ileostomy in CIPO. Esophageal manometry may be abnormal both in children and adults with CIPO.33Boige N. Faure C. Cargill G. Mashako L. Cordeiro-Ferreira G. Viarme F. Cezard J.P. Navarro J. Manometrical evaluation in visceral neuropathies in children.J Pediatr Gastroenterol Nutr. 1994; 19: 71-77Crossref PubMed Scopus (31) Google Scholar, 34Schuffler M.D. Pope 2nd, C.E. Esophageal motor dysfunction in idiopathic intestinal pseudoobstruction.Gastroenterology. 1976; 70: 677-682PubMed Scopus (56) Google Scholar Transit studies document prolonged whole-gut transit times in CIPO.3Heneyke S. Smith V.V. Spitz L. Milla P.J. Chronic intestinal pseudo-obstruction treatment and long term follow up of 44 patients.Arch Dis Child. 1999; 81: 21-27Crossref PubMed Scopus (133) Google Scholar Radio-opaque marker studies may be useful to identify the site of functional obstruction in CIPO. In a child with pseudo-obstruction related to neuronal intestinal dysplasia, marker studies indicated a hold-up in the ascending colon. The child did well after defunctioning ileostomy.35Hase T. Kodama M. Kishida A. Naka N. Shimadera S. Egawa T. Ohno M. Shimada M. The application of radio-opaque markers prior to ileostomy in an infant with chronic intestinal pseudo-obstruction report of a case.Surg Today. 1998; 28: 83-86Crossref PubMed Scopus (7) Google Scholar Scintigraphic studies have been used in adults to measure gastric, small-bowel, and colonic transit. Patterns of ileocolonic transfer of solid chyme may suggest the underlying pathophysiology. It was noted that bolus filling of the colon was less frequent in patients with myopathic CIPO whereas it was preserved in patients with neuropathic CIPO.36Greydanus M.P. Camilleri M. Colemont L.J. Phillips S.F. Brown M.L. Thomforde G.M. Ileocolonic transfer of solid chyme in small intestinal neuropathies and myopathies.Gastroenterology. 1990; 99: 158-164Abstract PubMed Google Scholar Scintigraphic evaluation of small-bowel transit also has been used to characterize subgroups of children with functional dyspepsia.37Chitkara D.K. Delgado-Aros S. Bredenoord A.J. Cremonini F. El-Youssef M. Freese D. Camilleri M. Functional dyspepsia, upper gastrointestinal symptoms, and transit in children.J Pediatr. 2003; 143: 609-613Abstract Full Text Full Text PDF PubMed Scopus (46) Google Scholar Electrogastrography was used previously as a screening test for motility abnormalities. However, results are nonspecific, correlate poorly with symptoms, and there is considerable overlap between children with motility abnormalities and controls.38Di Lorenzo C. Reddy S.N. Flores A.F. Hyman P.E. Is electrogastrography a substitute for manometric studies in children with functional gastrointestinal disorders?.Dig Dis Sci. 1997; 42: 2310-2316Crossref PubMed Scopus (37) Google Scholar Therefore, this technique has lost some of its appeal and manometry is now considered a more definitive investigation for pseudo-obstruction.39Cucchiara S. Borrelli O. Salvia G. Iula V.D. Fecarotta S. Gaudiello G. Boccia G. Annese V. A normal gastrointestinal motility excludes chronic intestinal pseudoobstruction in children.Dig Dis Sci. 2000; 45: 258-264Crossref PubMed Scopus (58) Google Scholar Increasingly, full-thickness biopsy specimens are obtained to seek a specific pathologic diagnosis. As the range of therapies for CIPO continues to expand, more informative classifications may be necessary to guide management. Biopsy samples should be analyzed in referral laboratories for a wide range of known abnormalities. The biopsy sample should be divided to send some tissue for routine light microscopy (in formalin), some for electron microscopy (in glutaraldehyde), and samples for immunohistochemistry and enzyme histochemistry (snap-frozen).3Heneyke S. Smith V.V. Spitz L. Milla P.J. Chronic intestinal pseudo-obstruction treatment and long term follow up of 44 patients.Arch Dis Child. 1999; 81: 21-27Crossref PubMed Scopus (133) Google Scholar Immunoreactivity for c-Kit is a marker for interstitial cells of Cajal and it has been used to show abnormalities in interstitial cells of Cajal distribution in children with CIPO.40Feldstein A.E. Miller S.M. El-Youssef M. Rodeberg D. Lindor N.M. Burgart L.J. Szurszewski J.H. Farrugia G. Chronic intestinal pseudoobstruction associated with altered interstitial cells of Cajal networks.J Pediatr Gastroenterol Nutr. 2003; 36: 492-497Crossref PubMed Scopus (101) Google Scholar Mitochondrial abnormalities may be suggested by megamitochondria in myenteric ganglion cells from rectal suction or full-thickness biopsy specimens4Teitelbaum J.E. Berde C.B. Nurko S. Buonomo C. Perez-Atayde A.R. Fox V.L. Diagnosis and management of MNGIE syndrome in children case report and review of the literature.J Pediatr Gastroenterol Nutr. 2002; 35: 377-383Crossref PubMed Scopus (55) Google Scholar and increased serum lactate, pyruvate, and thymidine levels. Mitochondrial DNA from skeletal muscle confirms the presence of mutations.4Teitelbaum J.E. Berde C.B. Nurko S. Buonomo C. Perez-Atayde A.R. Fox V.L. Diagnosis and management of MNGIE syndrome in children case report and review of the literature.J Pediatr Gastroenterol Nutr. 2002; 35: 377-383Crossref PubMed Scopus (55) Google Scholar If available through research programs, investigations such as in situ hybridization for specific abnormalities may be diagnostic. For example, in situ hybridization on biopsy specimen tissue from patients with megacystis microcolon hypoperistalsis syndrome may show abnormalities of the nicotinic acetylcholine receptor.41Richardson C.E. Morgan J.M. Jasani B. Green J.T. Rhodes J. Williams G.T. Lindstrom J. Wonnacott S. Thomas G.A. Smith V. 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BACKGROUNDGastroesophageal reflux disease (GORD) is said to be the causative factor in up to 41% of adults with chronic cough. Treatment for GORD includes conservative measures (diet manipulation), pharmaceutical therapy (motility or prokinetic agents, H(2)-antagonist and proton pump inhibitors (PPI)) and fundoplication.OBJECTIVESTo evaluate the efficacy of GORD treatment on chronic cough in children and adults with GORD and prolonged cough that is not related to an underlying respiratory disease, i.e. non-specific chronic cough.SEARCH STRATEGYWe searched the Cochrane Airways Group Specialised Register, the Cochrane Register of Controlled Trials (CENTRAL), MEDLINE, EMBASE, review articles and reference lists of relevant articles. The date of last search was 8 April 2010.SELECTION CRITERIAAll randomised controlled trials (RCTs) on GORD treatment for cough in children and adults without primary lung disease.DATA COLLECTION AND ANALYSISTwo review authors independently assessed trial quality and extracted data. We contacted study authors for further information.MAIN RESULTSWe included 19 studies (six paediatric, 13 adults). None of the paediatric studies could be combined for meta-analysis. A single RCT in infants found that PPI (compared to placebo) was not efficacious for cough outcomes (favouring placebo OR 1.61; 95% CI 0.57 to 4.55) but those on PPI had significantly increased adverse events (OR 5.56; 95% CI 1.18 to 26.25) (number needed to treat for harm in four weeks was 11 (95% CI 3 to 232)). In adults, analysis of H(2) antagonist, motility agents and conservative treatment for GORD was not possible (lack of data) and there were no controlled studies of fundoplication. We analysed nine adult studies comparing PPI (two to three months) to placebo for various outcomes in the meta-analysis. Using intention-to-treat, pooled data from studies resulted in no significant difference between treatment and placebo in total resolution of cough (OR 0.46; 95% CI 0.19 to 1.15). Pooled data revealed no overall significant improvement in cough outcomes (end of trial or change in cough scores). We only found significant differences in sensitivity analyses. We found a significant improvement in change of cough scores at end of intervention (two to three months) in those receiving PPI (standardised mean difference -0.41; 95% CI -0.75 to -0.07) using generic inverse variance analysis on cross-over trials. Two studies reported improvement in cough after five days to two weeks of treatment.AUTHORS' CONCLUSIONSPPI is not efficacious for cough associated with GORD symptoms in very young children (including infants) and should not be used for cough outcomes. There is insufficient data in older children to draw any valid conclusions. In adults, there is insufficient evidence to conclude definitely that GORD treatment with PPI is universally beneficial for cough associated with GORD. Clinicians should be cognisant of the period (natural resolution with time) and placebo effect in studies that utilise cough as an outcome measure. Future paediatric and adult studies should be double-blind, randomised controlled and parallel-design, using treatments for at least two months, with validated subjective and objective cough outcomes and include ascertainment of time to respond as well as assessment of acid and/or non-acid reflux.

Journal of Pediatric Gastroenterology and NutritionVolume 39, Issue S1 p. S149-S149 ABSTRACTS: Poster Session Abstracts P0238 ANTITHROMBIN SUPPLEMENTATION AFTER LIVER TRANSPLANTATION F. L. Connor, F. L. Connor Gastroenterology Department, Royal Children’s Hospital, Brisbane, AustraliaSearch for more papers by this authorJ. A. McEneiry, J. A. McEneiry Intensive Care Unit, Brisbane, AustraliaSearch for more papers by this authorK. Beale, K. Beale Liver Transplantation Department, Royal Children’s Hospital, Brisbane, AustraliaSearch for more papers by this authorH. Smith, H. Smith Haematology Department, Royal Brisbane Hospital, Brisbane, AustraliaSearch for more papers by this authorS. Lynch, S. Lynch Liver Transplantation Department, Princess Alexandra Hospital, Brisbane, AustraliaSearch for more papers by this authorJ. Fawcett, J. Fawcett Liver Transplantation Department, Princess Alexandra Hospital, Brisbane, AustraliaSearch for more papers by this author F. L. Connor, F. L. Connor Gastroenterology Department, Royal Children’s Hospital, Brisbane, AustraliaSearch for more papers by this authorJ. A. McEneiry, J. A. McEneiry Intensive Care Unit, Brisbane, AustraliaSearch for more papers by this authorK. Beale, K. Beale Liver Transplantation Department, Royal Children’s Hospital, Brisbane, AustraliaSearch for more papers by this authorH. Smith, H. Smith Haematology Department, Royal Brisbane Hospital, Brisbane, AustraliaSearch for more papers by this authorS. Lynch, S. Lynch Liver Transplantation Department, Princess Alexandra Hospital, Brisbane, AustraliaSearch for more papers by this authorJ. Fawcett, J. Fawcett Liver Transplantation Department, Princess Alexandra Hospital, Brisbane, AustraliaSearch for more papers by this author First published: 01 June 2004 https://doi.org/10.1002/j.1536-4801.2004.tb12668.x Submitted by: [email protected] Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onEmailFacebookTwitterLinkedInRedditWechat No abstract is available for this article. Volume39, IssueS1June 2004Pages S149-S149 RelatedInformation

Journal of Pediatric Gastroenterology and NutritionVolume 39, Issue S1 p. S109-S110 ABSTRACTS: Poster Session Abstracts P0136 PP THE CLINICAL UTILITY OF ANTRODUODENAL MANOMETRY F. L. Connor, F. L. Connor Division of Gastroenterology, Children’s Hospital of Pittsburgh, Pittsburgh, United StatesSearch for more papers by this authorC. Di Lorenzo, C. Di Lorenzo Division of Gastroenterology, Children’s Hospital of Pittsburgh, Pittsburgh, United StatesSearch for more papers by this author F. L. Connor, F. L. Connor Division of Gastroenterology, Children’s Hospital of Pittsburgh, Pittsburgh, United StatesSearch for more papers by this authorC. Di Lorenzo, C. Di Lorenzo Division of Gastroenterology, Children’s Hospital of Pittsburgh, Pittsburgh, United StatesSearch for more papers by this author First published: 01 June 2004 https://doi.org/10.1002/j.1536-4801.2004.tb12566.x Submitted by: [email protected] Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onEmailFacebookTwitterLinkedInRedditWechat No abstract is available for this article. Volume39, IssueS1June 2004Pages S109-S110 RelatedInformation

Interobserver variability affects investigations involving assessment of complex visual data, such as histopathology, radiology and motility. This study assessed interobserver variation for interpretation of antroduodenal manometry (ADM), as this has not been previously investigated. Thirty-five ADM recordings from children aged 0.3-18 years were independently evaluated by five experienced paediatric gastroenterologists who were blinded to cases' clinical histories. Intra-class correlation (ICC) was analysed for detection and measurement of phase three of the migrating motor complex (MMC) and Cohen's kappa statistic was calculated between observer pairs for detection of specific motility features and final diagnosis. Observers were unanimous on the differentiation of normal and abnormal motility in 63% of cases. There was excellent interobserver agreement for the number of phase three of the MMC in fasting (ICC = 0.82, P < 0.0001) and for measurements of phase three of the MMC (ICC = 0.9999, P < 0.0001). Detection of other normal and abnormal motility patterns varied more. Objective findings such as the presence of phase three of the MMC correlated more closely than findings that involved the integration of several variables, such as final diagnosis. However, these data overall indicate that agreement between expert observers for the distinction of normal and abnormal antroduodenal motility compares favourably with other standard medical assessments.