Clinical significance of detection of internal anal sphincter in children with functional constipation / 中华胃肠外科杂志

<p><b>OBJECTIVE</b>To investigate the structural and functional changes of internal anal sphincter (IAS) in children with functional constipation (FC), and to evaluate the association between the thickness of IAS and the severity of clinical symptoms.</p><p><b>METHODS</b>A total of 35 children with FC(constipation group,17 with incontinence) between June 2008 and December 2008 at the Shengjing Hospital of China Medical University were evaluated using anal manometry and endosonography. These patients were compared to 23 hospitalized children who were excluded for digestive and endocrinal diseases(control group). A validated symptom score(SS) was used to assess the severity of symptoms. The sum of SS ranged between 0 and 65.</p><p><b>RESULTS</b>Anorectal manometry showed reflex relaxation of IAS in response to distension of rectal balloon in all patients. Rectal perceptional threshold in FC group was significantly higher than that in the controls[(42.4 ± 19.5) ml vs.(29.1 ± 15.6) ml, P<0.05]. The lowest volume for inducing reflex relaxation of IAS was significantly higher than that in the controls [(55.6 ± 31.6) ml vs.(30.5 ±13.8) ml, P<0.05]. The thickening of IAS was noted in all the patients[(3.8 ± 1.7) mm vs.(2.5 ± 1.0) mm, P<0.05]. However, there was no significant difference between FC and control in median resting anal sphincter pressure[(170.8 ± 62.3) mm Hg vs. (161.3 ± 51.1) mm Hg, P>0.05]. The median symptom score was 9.3 ± 4.3 in the FC group. The thickness of IAS correlated significantly with total symptom severity score(r=0.407, P<0.05). There was no correlation between thickness of IAS and age, sex, or duration of disease(P>0.05).</p><p><b>CONCLUSIONS</b>Structural and functional changes of internal anal sphincter exist in children with functional constipation. The thickness of internal anal sphincter correlates significantly with symptom severity.</p>

Expression and significance of Notch-1 and Jagged-2 in patients with Hirschsprung disease / 中华胃肠外科杂志

<p><b>OBJECTIVE</b>To investigate the expression of Notch-1 and Jagged-2 in the normal and spastic segments of colon in patients with Hirschsprung disease(HD), and to explore the correlation of Notch-1 and Jagged-2 with pathogenesis of HD.</p><p><b>METHODS</b>From 2005 to 2010, resected colon specimens of 30 cases with HD were selected for this study. Normal colonic segments were served as control group, while the transitional and spastic segments as experimental group. Immunohistochemical staining, Western blotting, and RT-PCR were applied to detect the expression of Notch-1 and Jagged-2.</p><p><b>RESULTS</b>A large number of Notch-1 and Jagged-2 positive gangliocytes were observed in the control group, while none was observed in spastic segments. Significantly less Notch-1 and Jagged-2 positive gangliocytes were found in the transitional segments. Western blotting revealed that Notch-1 and Jagged-2 protein levels in spastic segments (0.19±0.02 and 0.13±0.04) were less than that in transitional segments and normal segments (0.58±0.05 and 0.52±0.04, 0.72±0.04 and 0.69±0.04, respectively)(P<0.05). RT-PCR revealed that Notch-1 and Jagged-2 mRNA levels were consistent with protein expression.</p><p><b>CONCLUSION</b>Notch-1 and Jagged-2 are not expressed in spastic colon segments, which may be associated with the pathogenesis of HD.</p>

Expression of Wnt5a in the terminal rectum of children with anorectal malformation / 中国当代儿科杂志

<p><b>OBJECTIVE</b>To study the expression of Wnt5a protein in the terminal rectum of children with anorectal malformation (ARM) and the possible association between Wnt5a and ARM.</p><p><b>METHODS</b>Specimens were obtained from 20 children with ARM, 7 children with acquired rectovestibular fistula and 6 children with non-gastrointestinal tract disease (control group). The expression of Wnt5a protein in the terminal rectum was determined by immunohistochemistry and Western blot.</p><p><b>RESULTS</b>Wnt5a was mainly expressed in the rectum of the myenteric nerve plexus, mucosal layer and submucosa in the control group. Compared with the control group, Wnt5a expression in the terminal rectum decreased significantly in the ARM group, and decreased more significantly in children with high ARM. The results of Western blot showed the expression of Wnt5a protein in the high, intermediate and low ARM groups were significantly lower than that in the acquired rectovestibular fistula and the control groups (P<0.01). The expression of Wnt5a protein in the high and the intermediate ARM groups were also lower than that in the low ARM group (P<0.01). There was no significant difference in the Wnt5a protein expression between the acquired rectovestibular fistula and the control groups.</p><p><b>CONCLUSIONS</b>The expression of Wnt5a in the termina1 rectum decreases in children with ARM, suggesting Wnt5a may play an important role in the development of ARM.</p>

Cell apoptosis during the cloacal embryonic development in rats with anorectal malformations / 中国当代儿科杂志

<p><b>OBJECTIVE</b>In the normal embryonic development of anorectum, apoptosis plays an important role. To explore the role of apoptosis in anorectal malformations (ARM), this study investigated cell apoptosis during the cloacal embryonic development in ARM embryos.</p><p><b>METHODS</b>ARM embryos were induced by intragastric administration of ethylenethiourea (125 mg/kg) for pregnant rats on embryonic day 10 (E10). The distribution of apoptotic cells in the cloaca was ascertained by hematoxylin and eosin and TUNEL staining in the normal control embryos (n=102) and ARM embryos (n=147) on E13, E13.5, E14, E15 and E16.</p><p><b>RESULTS</b>On E13, apoptotic cells were detected in the urorectal septum of rat embryos in the control group. With the development of embryos, the number of apoptotic cells in the mesenchyme of urorectal septum gradually increased and a large number of apoptotic cells were seen in the dorsal rectal mesenchyme. On E14, apoptotic cells appeared at the terminal rectum and the dorsal cloacal membrane. On E15, the urorectal septum fused with the cloacal membrane and apoptotic cells in the urorectal septum mesenchyme continuously extended down to the fusion region. Compared with the control group, apoptotic cells in the urorectal septum, the dorsal rectal mesenchyme and the cloacal membrane of the ARM rat embryos were significantly reduced during the embryonic development. The development of the urorectal septum was delayed and it did not fuse with the cloacal membrane in ARM embryos.</p><p><b>CONCLUSIONS</b>During the embryonic development of cloaca, abnormal apoptosis in the urorectal septum, the dorsal rectal mesenchyme and the cloacal membrane may be one of the reasons for anorectal malformations. The proper regulation of cell apoptosis may be one of the key mechanisms for normal development of anorectum in the embryonic stage.</p>

Evaluation of anorectal function after transanal one-stage endorectal pull through operation in children with Hirschsprung's disease / 中国当代儿科杂志

<p><b>OBJECTIVE</b>The short-term efficacy of the transanal one-stage endorectal pull through operation for Hirschsprung's disease is satisfactory. However the long-term outcome of anorectal function has not been fully understood. The aim of this study was to evaluate the stooling pattern, colonic motility and anal sphincter performance after transanal one-stage pull through operation in children with Hirschsprung's disease.</p><p><b>METHODS</b>Fifty-eight children who underwent transanal one-stage pull through operation for Hirschsprung's disease were followed up. The mean follow-up duration was 15.8 months (range, 6-24 months). The stooling patterns of the patients were investigated by the informed questionnaire. Barium enema, defecography, total and segmental colonic transit time and the anorectal vector manometry were performed. Thirty- three healthy children were used as controls.</p><p><b>RESULTS</b>Most of patients had normal stool consistency and frequency. Postoperative enterocolitis occurred in 3 patients, and constipation was found in five patients. Postoperative soiling was observed in 9 patients. None of the 58 patients had incontinence, cuff infection, anastomotic leak and mortality. The barium enema showed that the configuration of the colon recovered well in most of patients. Postopertive defecography showed the anorectal angle of all the patients was open, fixed and bigger than that of preoperation and the healthy controls (P < 0.01). Postoperatively, the mean total gastrointestinal transit time (TGITT), the left colonic transit time (LCTT) and rectosigmoid colonic transit time (RSTT) in the 58 patients were significantly shorter than preoperatively (P < 0.01) and were similar to those of the control group. The rectoanal inhibitory reflex was regained in 5 patients. The anal maximal pressure of the patients with constipation in resting and squeezing condition were significantly higher than those of the asymptomatic patients and controls (P < 0.05). The vector volume (VV) and vector symmetric index (VSI) in patients with soiling were significantly lower than those in preoperation and the controls (P < 0.05). The VSI in the patients with constipation was significantly higher compared with the controls (P < 0.05).</p><p><b>CONCLUSIONS</b>The stooling function, colonic motility and anal sphincter performance manifest well in most of the patients after the transanal endorectal pull through operation for Hirschsprung's disease. Stooling disorders in few cases are probably related to decrease or disappearance of the sigmoid loops, dysfunction of the "neorectosigmoid", an open and fixed anorectal angle and achalasia of the internal anal sphincter.</p>

Determination of total and segmental colonic transit time in constipated children / 中华儿科杂志

<p><b>OBJECTIVE</b>To determine the total and segmental colonic transit time of normal Chinese children and to explore its value in constipation in children.</p><p><b>METHODS</b>The subjects involved in this study were divided into 2 groups. One group was control, which had 33 healthy children (21 males and 12 females) aged 2 - 13 years (mean 5 years). The other was constipation group, which had 25 patients (15 males and 10 females) aged 3 - 14 years (mean 7 years) with constipation according to Benninga's criteria. Written informed consent was obtained from the parents of each subject. In this study the simplified method of radio opaque markers was used to determine the total gastrointestinal transit time and segmental colonic transit time of the normal and constipated children, and in part of these patients X-ray defecography was also used.</p><p><b>RESULTS</b>The total gastrointestinal transit time (TGITT), right colonic transit time (RCTT), left colonic transit time (LCTT) and rectosigmoid colonic transit time (RSTT) of the normal children were 28.7 +/- 7.7 h, 7.5 +/- 3.2 h, 6.5 +/- 3.8 h and 13.4 +/- 5.6 h, respectively. In the constipated children, the TGITT, LCTT and RSTT were significantly longer than those in controls (92.2 +/- 55.5 h vs 28.7 +/- 7.7 h, P < 0.001; 16.9 +/- 12.6 h vs 6.5 +/- 3.8 h, P < 0.01; 61.5 +/- 29.0 h vs 13.4 +/- 5.6 h, P < 0.001), while the RCTT had no significant difference. X-ray defecography demonstrated one rectocele, one perineal descent syndrome and one puborectal muscle syndrome, respectively.</p><p><b>CONCLUSION</b>The TGITT, RCTT, LCTT and RSTT of the normal children were 28.7 +/- 7.7 h, 7.5 +/- 3.2 h, 6.5 +/- 3.8 h and 13.4 +/- 5.6 h, respectively. With the segmental colonic transit time, constipation can be divided into four types: slow-transit constipation, outlet obstruction, mixed type and normal transit constipation. X-ray defecography can demonstrate the anatomical or dynamic abnormalities within the anorectal area, with which constipation can be further divided into different subtypes, and combined use of the gastrointestinal transit time and X-ray defecography is of clinical importance in exploration of etiology of constipation.</p>