The role of motility in functional abdominal disorders in children.
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Biomedical subjects
Publications and source records attributed to C Di Lorenzo.
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OBJECTIVES: To evaluate the clinical presentation and to assess the usefulness of antroduodenal manometry (ADM) and the results of multidisciplinary team management in 12 neurologically normal adolescents (9 girls) with rumination. STUDY DESIGN: All patients had extensive investigations that ruled out other causes of their chronic symptoms. We performed ADM in all patients. A multidisciplinary approach was used for the nutritional and behavioral rehabilitation of these patients. RESULTS: The median age at presentation was 14 years (range, 9-19 years), and the average duration of symptoms was 17 months. All patients complained of postprandial, effortless regurgitation, and the majority had weight loss and abdominal pain. Results of fasting ADM were normal in all. The postprandial ADM showed brief, simultaneous pressure increases at all recording sites, associated with regurgitation in 8 patients. No emesis was observed in the other 4 children during the study. Treatment included nutritional support in combination with antidepressants and anxiolytics (n = 6), cognitive therapy with biofeedback or relaxation techniques (n = 7), and pain management (n = 2). Resolution or improvement of symptoms was seen in 10 of the 12 patients, and successful transition to oral feedings was achieved in all during the follow-up period, which ranged from 5 to 36 months. CONCLUSIONS: Rumination is a distinct functional gastrointestinal disorder of otherwise healthy children and adolescents, which can be diagnosed on the basis of clinical features. The ADM shows a characteristic pattern and rules out motility disorders that are often confused with rumination. A multidisciplinary team approach is associated with satisfactory recovery in most patients.
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We wished to investigate the urodynamic characteristics and colonic motility in a group of children with severe chronic constipation and lower urinary tract symptoms. We performed colonic manometry using an endoscopically placed catheter. The urodynamic studies consisted of cystometry, electromyography of the external urethral sphincter, measurement of urinary flow rate, and urethral pressure profile. We found abnormal colonic motility in all patients. Findings included: absent gastrocolonic response (N = 8), absent high-amplitude propagated contractions (HAPCs) (N = 4), and abnormal propagation of HAPCs (N = 7). Urodynamic features were abnormal in 10 children. Findings included: uninhibited bladder contractions (N = 6), hypertonic bladder (N = 2), sphincter dyssynergy (N = 2), small capacity bladder (N = 1). In all children constipation improved, in three after a partial colectomy. Urinary symptoms persisted. We conclude that some children with severe constipation may have a neuropathy affecting both the colonic and lower urinary tracts systems. In this group of patients treatment of constipation does not result in resolution of urinary symptoms.
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OBJECTIVE: The goal of this retrospective study was to assess whether 99mTc-white blood cell (WBC) scintigraphy and upper gastrointestinal small bowel follow-through (UGI-SBFT) could exclude inflammation in children suspected of having inflammatory bowel disease (IBD). METHODS: Of a population of 313 children who had a 99mTc-WBC scan, 130 children were studied exclusively to rule out IBD. Sixty-nine colonoscopies with biopsies were done within a short time interval of the 99mTc-WBC scans. There were also 51 controls studied with 99mTc-WBC scintigraphy. RESULTS: Of the 130 children studied to exclude IBD, the final diagnosis was Crohn's disease in 27, ulcerative colitis in nine, miscellaneous colitis in 13, probably normal in 42, and normal in 39. The 99mTc-WBC scans were positive in all but three newly diagnosed Crohn's disease, ulcerative colitis, or miscellaneous colitis children. The false-negative 99mTc-WBC studies were seen in children with mild inflammation on biopsies and normal UGI-SBFT studies. In the 46 children with a true-positive 99mTc-WBC scan, 81% (17/21) of UGI-SBFT studies were normal. In five children with equivocal UGI-SBFT studies, the 99mTc-WBC scan correctly predicted if inflammation was present in the terminal ileum. CONCLUSIONS: Our results suggest that 99mTc-WBC is useful as an initial screening modality to exclude IBD, and is more sensitive than UGI-SBFT studies.
OBJECTIVES: Eosinophilic esophagitis, previously confused with esophageal inflammation due to gastroesophageal reflux, has recently begun to be distinguished from it. We undertook this analysis of our large series of children with the condition to clarify its spectrum: its presenting symptoms; its relation to allergy, respiratory disease, and reflux; its endoscopic and histological findings; and its diagnosis and therapy. METHODS: We analyzed the details of our clinical series of 30 children with eosinophilic esophagitis, defining it as > or =5 eosinophils per high power field in the distal esophageal epithelium. Retrospective chart review was supplemented by prospective, blinded, duplicate quantitative evaluation of histology specimens, and by telephone contact with some families to clarify subsequent course. Presentation and analysis of the series as a whole is preceded by a case illustrating a typical presentation with dysphagia and recurrent esophageal food impactions. RESULTS: Presenting symptoms encompass vomiting, pain, and dysphagia (some with impactions or strictures). Allergy, particularly food allergy, is an associated finding in most patients, and many have concomitant asthma or other chronic respiratory disease. A subtle granularity with furrows or rings is newly identified as the endoscopic herald of histological eosinophilic esophagitis. Histological characteristics include peripapillary or juxtaluminal eosinophil clustering in certain cases. Association with eosinophilic gastroenteritis occurs, but is not common. Differentiation from gastroesophageal reflux disease is approached by analyzing eosinophil density and response to therapeutic trials. Therapy encompasses dietary elimination and anti-inflammatory pharmacotherapy. CONCLUSION: Awareness of the spectrum of eosinophilic esophagitis should promote optimal diagnosis and treatment of this elusive entity, both in children and in adults.
OBJECTIVE: Years after surgery for Hirschsprung's disease, many children continue to suffer from fecal incontinence or constipation. The purpose of the present investigation was to define the physiology underlying the persistent symptoms in children after surgery for Hirschsprung's disease, and to determine the outcome of interventions based on the results of the motility testing. METHODS: We studied 46 symptomatic patients (5.5+/-3.3 yr old, 35 male) >10 months after surgery for Hirschsprung's disease. We performed a colonic manometry with a catheter placed with the tip in the proximal colon. We used a structured questionnaire and phone interview to follow up the patients an average of 34 months after the manometry. RESULTS: We identified four motility patterns: 1) high-amplitude propagating contractions (HAPCs) migrating through the neorectum to the anal sphincter, associated with fecal soiling (n = 18); 2) normal colonic manometry associated with fear of defecation and retentive posturing (n = 9); 3) absence of HAPCs or persistent simultaneous contractions over two or more recording sites (n = 15), associated with constipation (n = 13); and 4) normal colonic motility and a hypertensive internal anal sphincter (>80 mm Hg) (n = 4). We based treatment on results of the motility studies. There was improvement in global health (mean score, 3.9+/-1.1 vs 2.8+/-1.3 at the time of initial evaluation, p < 0.001) and emotional health (3.8+/-1.1 vs 2.6+/-1.1, p < 0.0001). Improvement in the number of bowel movements occurred in 72% of children. Resolution or decreased abdominal pain was reported in 80%. CONCLUSIONS: Colonic manometry clarifies the pathophysiology and directs treatment in symptomatic children after surgery for Hirschsprung's disease.
We reviewed 312 (99m)Tc leukocyte (99m)Tc-WBC studies to evaluate the sensitivity and specificity of (99m)Tc-WBC for identifying small-bowel and gastric inflammation in children with Crohn's disease. The (99m)Tc-WBC scans were interpreted blindly and compared to the results of colonoscopy with biopsies and surgical specimens. In 46 children, total colonoscopy was done within a few days of a (99m)Tc- WBC scan and in 13 others surgical specimens were available. Of the 37 children in whom the terminal ileum was visualized endoscopically (24) or surgically (13), the WBC-Tc(99m) scan showed a similar degree of inflammation in 33. The sensitivity of (99m)Tc-WBC scanning was 91% and the specificity 96%. The scans allowed evaluation of inflammation in the terminal ileum in 22 children in whom the endoscopist did not cannulate it; the scans were abnormal in 10 of these 22 children. In the children with no ileoscopy the results of the (99m)Tc-WBC scan were consistent with the laboratory values, the gastroenterologist's clinical assessment, and long-term clinical follow-up. Finally, the scans of 46 controls did not reveal any false-positive findings in the terminal ileum. Five children with gastritis documented by gastroscopy had normal (99m)Tc-WBC scans and upper gastrointestinal barium studies. This study demonstrates in a large number of normal and abnormal children with different pre-test probabilities of having inflammatory bowel disease that scintigraphy is reliable in identifying small-bowel inflammation.
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Antibodies to Neospora caninum were measured in bovine foetuses, dairy cows and beef cows in Argentina using the IFAT, the N. caninum agglutination test, and the recombinant NCDG1 and NCDG2 ELISA. Serum antibodies (IFAT titre 1:80) were found in 20 of 82 (24.4%) dairy cow foetuses and one of 22 (4.5%) beef cow foetuses. Microscopic lesions suggestive of neosporosis were seen in brains of seven of eight foetuses with IFAT titres of 1:80. Antibodies (IFAT) were found in 122 of 189 (64.5%) dairy cows that aborted. Serum antibody titres (IFAT) of 189 dairy cows that aborted were: < 1:25 (67 cows), 1:25 (four cows), 1:50 (16 cows), 1:200 (seven cows), 1:> or = 800 (95 cows). Of the 87 sera with IFAT titres of < or = 1:50, 57 had no antibodies in 1:40 dilution and 30 had titres of 1:40 in the N. caninum agglutination test. Thus, sera from at least 56 dairy cows which had aborted were seronegative both in the N. caninum agglutination test and the IFAT. The distribution of positive and negative sera was similar when measured by ELISA, except that, depending on cut-off titre, the ELISA indicated a greater number of seropositive cows that were negative by the IFAT and N. caninum agglutination test. These results suggest that transplacental transmission of N. caninum in dairy cows in Argentina is frequent.
PURPOSE: The aim of this study was to analyze factors impacting on the survival of pediatric patients with intestinal failure referred for intestinal transplantation (ITx). METHODS: Two hundred fifty-seven children (mean age, 3.4+/-0.26 years) with intestinal failure were evaluated for ITx between 1990 and 1998. All patients were dependent on total parenteral nutrition (TPN) for a mean of 31+/-2.7 months. The mean follow-up time from the date of evaluation was 9.2+/-0.9 months. RESULTS: Eighty-two (32%) children underwent ITx with a mean waiting time of 10.1+/-1.3 months (simultaneous liver-intestinal allograft in 68% instances). Of the 175 patients who did not undergo transplantation, 120 died, 23 were lost to follow-up, and 32 are alive. Younger patients (< or =1 year) had poorer survival rates than patients older than 1 year (P<.0001). The patients with the worse prognosis were those with necrotizing enterocolitis, and those with the best prognosis were those with Hirschsprung's disease. Patients with "surgical" causes had poorer survival rates than those with "nonsurgical" causes (P<.04). Patients with bridging fibrosis or established cirrhosis had an earlier mortality than patients with portal fibrosis (P<.003). The worst survival rate was found for patients with bilirubin levels of greater than 3 mg/dL (P<.0001), plateletcounts less than 100.000/mL (P<.0001), prothrombin time greater than 15 seconds (P = .03) or partial thromboplastin time greater than 40 seconds (P<.04). Children who at the time of evaluation needed only an isolated intestinal allograft had a better prognosis than those who required a combined liver-intestine allograft (P<.00001). With multivariate analysis independent prognosis risk factors of poor outcome were hyperbilirubinemia and severity of histopathologic damage. CONCLUSIONS: Early referral for ITx should occur before the development of liver dysfunction, taking into consideration the aforementioned risk factors that would facilitate the development and ominous evolution to liver failure.
Following surgical correction of imperforate anus, voluntary bowel control is frequently poor because of abnormal anorectal function. Using colonic manometry we investigated the role of colonic motility in the pathogenesis of fecal soiling in children following imperforate anus repair. Thirteen children with repaired imperforate anus and fecal soiling underwent motility testing 2-12 years after anoplasty. All had fecal incontinence unresponsive to conventional medical treatment. Colonic manometry was performed using water-perfused catheters. Anorectal manometry was undertaken in 10 patients. Motility study results, treatment and outcomes were compared. All patients had high-amplitude propagating contractions (HAPCs) with an average of 80% propagation into the neorectum. There was no correlation between HAPC number or morphology and any variable. Internal anal sphincter resting pressure was low in 6/10 patients. Relaxation of the internal anal sphincter was present in 6/10 children. Only 1 of 5 patients able to cooperate was capable of generating a normal maximal squeeze pressure. Therapeutic regimens were changed in 11 patients with clinical improvement in five. Fecal soiling in patients with repaired imperforate anus is a multifactorial problem including propagation of excessive numbers of HAPCs into the neorectum as well as internal anal sphincter dysfunction. Colonic manometry in conjunction with anorectal manometry aids in the understanding of the pathophysiology of fecal soiling and guides clinical management in children with repaired imperforate anus.
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BACKGROUND: In earlier studies, erythromycin stimulated but octreotide inhibited gastric antral contractions, as each drug induced phase 3-like episodes. METHODS: To assess the effect of erythromycin pretreatment on octreotide-induced changes in antroduodenal motility, 16 patients were studied (mean age, 8.7 +/- 1.5 years, 8 male): 6 with severe gastroesophageal reflux, 4 with cyclic vomiting, 3 with gastroparesis, 2 with chronic intestinal pseudo-obstruction, and 1 with Crohn's disease and unexplained nausea and vomiting. After recording fasting antroduodenal motility for 3 hours, 1 mg/kg intravenous erythromycin was administered over 30 minutes. Sixty minutes after the erythromycin infusion, 0.5 microg/kg subcutaneous octreotide was administered, followed 1 hour later by a meal. RESULTS: Phase 3 occurred spontaneously in 10 patients and after erythromycin in 12 patients. When administered after erythromycin, octreotide immediately induced phase 3s contractions in 15 patients, beginning in the antrum. In 7 children, some of the octreotide-induced phase 3s did not propagate. After the meal, antral contractions continued in all patients. The fed pattern was replaced in 14 patients by alternating phase 3 and phase 1 activities. CONCLUSIONS: Pretreatment with erythromycin prevented octreotide-induced inhibition of antral contractions. Inhibition of antral contractions by octreotide may be mediated through either a direct or indirect suppression of motilin release, because antral contractions persist after pretreatment with the motilin receptor agonist erythromycin.
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BACKGROUND: Constipation, defined as a delay or difficulty in defecation, present for 2 or more weeks, is a common pediatric problem encountered by both primary and specialty medical providers. METHODS: The Constipation Subcommittee of the Clinical Guidelines Committee of the North American Society for Pediatric Gastroenterology and Nutrition has formulated clinical practice guidelines for the management of pediatric constipation. The Constipation Subcommittee, consisting of two primary care pediatricians, a clinical epidemiologist, and pediatric gastroenterologists, based its recommendations on an integration of a comprehensive and systematic review of the medical literature combined with expert opinion. Consensus was achieved through Nominal Group Technique, a structured quantitative method. RESULTS: The Subcommittee developed two algorithms to assist with medical management, one for older infants and children and the second for infants less than 1 year of age. The guidelines provide recommendations for management by the primary care provider, including evaluation, initial treatment, follow-up management, and indications for consultation by a specialist. The Constipation Subcommittee also provided recommendations for management by the pediatric gastroenterologist. CONCLUSIONS: This report, which has been endorsed by the Executive Council of the North American Society for Pediatric Gastroenterology and Nutrition, has been prepared as a general guideline to assist providers of medical care in the evaluation and treatment of constipation in children. It is not intended as a substitute for clinical judgment or as a protocol for the management of all patients with this problem.
BACKGROUND: Children with chronic intestinal pseudo-obstruction (CIPO) often require total parenteral nutrition (TPN) which puts them at risk of liver failure and recurrent line infections. Intestinal transplantation has become a therapeutic option for TPN dependent children with intestinal failure who are failing management with TPN. AIMS: To investigate the outcome of children with CIPO referred for intestinal transplantation. METHODS: A retrospective review was carried out of records and diagnostic studies from 27 patients with CIPO referred for intestinal transplantation. RESULTS: Five children were not listed for transplantation: two because of parental decision, two because of suspicion of Munchausen syndrome by proxy, and one because he tolerated enteral nutrition. Six are still TPN dependent and awaiting transplantation. Eight children died awaiting transplantation. Eight children underwent transplantation. Three died (two months, seven months, and four years after transplant). Five children are alive with a median follow up of 2.6 years (range two months to six years). All transplanted children were able to tolerate full enteral feedings. The postoperative course was complicated by dumping syndrome, Munchausen syndrome by proxy, narcotic withdrawal, and uncovering of achalasia. Conclusion-Intestinal transplantation may be a life saving procedure in children with CIPO. Early referral and thorough pretransplant evaluation are keys to successful transplantation.