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OBJECTIVES:Esophageal high-resolution manometry (EHRM) has evolved rapidly from a research tool to a routine investigation in adult clinical practice. This study proposes and evaluates a standardized EHRM protocol for use in pediatric clinical practice.METHODS:Thirty pediatric patients underwent unsedated EHRM. Indications for EHRM were dysphagia, feeding difficulty, or pre-fundoplication assessment. Two 20-channel customized water-perfused silicone catheters, with an outside diameter of 3.8 mm (MuiScientific, Ontario, CA), were used. The catheters had one distal gastric channel, five channels 0.5 cm apart for the e-sleeve, and 14 proximal channels either 1 cm (for children <5 years) or 2 cm apart (for children >5 years). Single wet swallows, multiple rapid swallows (MRS), and solid swallows were systematically studied.RESULTS:The median age was 10 years (range 6 months–15 years). The esophageal motor findings were normal peristalsis (n=15), peristaltic dysfunction (n=12), achalasia (n=3), and spasm on consumption of solid food (n=2). The distal contractile integral adjusted for esophageal length (DCIa) of patients with peristaltic dysfunction was significantly lower than that of patients without peristaltic dysfunction (P<0.001). On MRS, aperistalsis with lack of esophagogastric junction (EGJ) relaxation was observed in patients with achalasia, and aperistalsis with complete EGJ relaxation was observed in patients with severe peristaltic dysfunction. On consumption of solid food, esophageal spasm associated with bolus impaction was observed in two patients.CONCLUSIONS:This study provides objective information with regard to topography pressure parameters in esophageal motility disorders of childhood while using a standardized EHRM protocol. The new DCIa variable may be useful for the assessment of patients with peristaltic dysfunction. [ABSTRACT FROM AUTHOR]

OBJECTIVES:The use of tumor necrosis factor-alpha (TNF-α) antagonists has changed the therapeutic strategy for Crohn's disease (CD). Adalimumab (ADA), a fully human anti-TNF-α monoclonal antibody, is an effective therapy for patients with CD, both naive patients and those intolerant or refractory to Infliximab (IFX), a chimeric anti-TNF-α agent. However, the use of ADA is rarely reported in pediatric CD. We performed an open prospective evaluation of short- and long-term efficacy and safety of ADA in children with moderate-to-severe CD.METHODS:A total of 23 pediatric CD patients (9 naive and 14 intolerant or unresponsive to IFX) received ADA subcutaneously as a loading schedule at weeks 0 and 2, and at every other week (eow) during a 48-week maintenance phase. Loading and maintenance doses were 160/80 and 80 mg eow in 13 cases, 120/80 and 80 mg eow in 2, and 80/40 and 40 mg eow in 8 cases. The primary efficacy outcomes were clinical remission and response at different scheduled visits along the maintenance phase. At baseline, 13 patients also received immunomodulators (IMs).RESULTS:At weeks 2, 4, 12, 24, and 48, remission rates were 36.3, 60.8, 30.5, 50, and 65.2%, respectively, whereas response rates were 87, 88, 70, 86, and 91%, respectively. Four patients at week 24 and 2 at week 48 received IMs; the mean daily corticosteroid dose, disease activity index, C-reactive protein level, and erythrocyte sedimentation rate decreased significantly throughout the trial. No serious adverse events were recorded.CONCLUSIONS:ADA can be an effective and safe biological agent for inducing and maintaining remission in children with moderate-to-severe CD, even in those with previous IFX therapy. [ABSTRACT FROM AUTHOR]

OBJECTIVES:Food allergy is thought to trigger functional constipation in children but the underlying mechanisms are still unknown. Mast cells (MCs) and their relationship with nerve fibers (NFs) in the rectal mucosa, as well as anorectal motility, were studied in children with refractory chronic constipation before and after an elimination diet for cow's milk, egg, and soy proteins.METHODS:Thirty-three children (range: 1–10.8 years) underwent anorectal manometry and suction rectal biopsy before and after 8 weeks of oligoantigenic diet. MCs and NFs were identified immunohistochemically. Quantification of MCs (%MC/area) and MCs within 10 μm of NFs (%MC-NF/area) was performed by computer-assisted analysis.RESULTS:Eighteen children responded to the diet (R-group) and fifteen did not (the NR-group). At baseline there was a significant difference in anal resting pressure (ARP; mm Hg), percentage of relaxation (%R), and residual pressure (RP; mm Hg) of anal canal during rectal distension between the R-group (66±4.1, 84.3±2.8, 10.4±2.3, respectively) and the NR-group (49±5, 92.2±1.7, 4.8±1.7, respectively; P<0.05). After the diet, significant changes in ARP, RP, and %R were observed only in the R-group (44±3.7, 93.7±1.5, 3.8±1.2, respectively; P<0.05). At baseline, the R-group showed an increase in %MC/area (8.3±0.7) and %MC-NF/area (5.2±2.6) with respect to the NR-group (5.1±0.5 and 2.3±0.4, respectively; P<0.05). After the diet, only the R-group showed a significant reduction of %MC/area and %MC-NF/area (4.4±0.5 and 2.2±0.4, respectively; P<0.001). Both ARP and RP significantly correlated with %MC/area and %MC-NF/area; %R showed a significant inverse correlation with both %MC/area and %MC-NF/area.CONCLUSIONS:In children with food allergy-related chronic constipation, an increase in both rectal MC density and spatial interactions between MCs and NFs correlates with anal motor abnormalities. These variables are significantly affected by the diet.Am J Gastroenterol 2009; 104:454–463; doi:10.1038/ajg.2008.109; published online 20 January 2009 [ABSTRACT FROM AUTHOR]

OBJECTIVES: Although muscular dystrophy (MD) affects primarily striated muscles, smooth muscle cells of the gastrointestinal tract may also be involved. We recorded gastric electrical activity and gastric emptying time (GET) in children with MD at initial presentation and at 3-yr follow-up in order to detect gastric motor abnormalities and study their evolution along the clinical course.METHODS: Twenty children with MD (median age: 4.6 yr; range age: 3–7 yr) were investigated by means of ultrasonography, for measuring GET, and by electrogastrography (EGG); 70 children served as controls.RESULTS: Ten patients had Duchenne muscular dystrophy (DMD) and 10 Becker muscular dystrophy (BMD). GET was significantly more delayed in MD patients (DMD, median: 195 min; range 150–260 min; BMD, median: 197 min; range: 150–250 min) than in controls (median: 150 min; 110–180 min;p<0.05); it markedly worsened at the follow-up in DMD (median: 270 min; range 170–310 min;p<0.001vscontrols) but not in BMD patients (median: 205 min; 155–275 min;p<0.05vsDMD). Baseline EGG showed a significantly lower prevalence of normal rhythm and significantly higher prevalence of dysrhythmias in both groups of patients as compared to controls (% of normal rhythm: DMD 66.7± 8.2, BMB 67.2± 11.5, controls 85.3± 7.2,p<0.001; % of tachygastria: DMD 28.4± 8.0, BMB 29.8± 12.3, controls 10.6± 5.1,p<0.001; % of dominant frequency instability coefficient: DMD 36.1± 6.0, BMB 33.2± 2.9, controls 17.9± 7.1,p<0.001); furthermore, no difference in fed-to-fasting ratio of the dominant EGG power was found between the two groups and controls (DMD 2.84± 1.27, BMB 2.82± 0.98, controls 3.04± 0.85, ns). However, at the follow-up no significant change in the prevalence of normal rhythm and dysrhythmias occurred in both groups (nsvsbaseline values), whereas only DMD patients showed a marked reduction in fed-to-fasting power ratio (0.78± 0.59;p<0.001vscontrols and BMD;p<0.05vsbaseline), which correlated with the progressive neuromuscular weakness occurring in DMD subjects (r, 0.75;p<0.001).CONCLUSIONS: In children with MD, there is an early abnormality in gastric motility that is due to deranged regulatory mechanisms, whereas contractile activity of smooth muscle cells seems to be preserved. At the follow-up, DMD patients exhibited a progressive failure in neuromuscular function, which was accompanied by a gastric motility derangement with worsening in GET and in EGG features suggesting an altered function of gastric smooth muscle cells.(Am J Gastroenterol 2005;100:695–702) [ABSTRACT FROM AUTHOR]