Development and validation of a childhood self-efficacy for functional constipation questionnaire.

BACKGROUND: Children with functional constipation fear painful bowel movements leading to stool withholding behavior. Self-efficacy is the belief that an individual can accomplish a given goal. If children with constipation avoid defecation because they think that they are unable defecate comfortably, this low self-efficacy may prevent treatment success. The aim of the current study was to develop and validate a constipation specific self-efficacy scale. METHODS: The self-efficacy for functional constipation questionnaire (SEFCQ) was developed by the authors and evaluated by 10 children and seven experts. Ninety-nine healthy children and 122 children with functional constipation completed the SEFCQ and three other questionnaires measuring related constructs. KEY RESULTS: Minor changes were made in wording based on feedback from experts and children. Factor analysis showed two scales, a 7 item Action scale (Cronbach's α = 0.88) and a 7 item Emotion scale (Cronbach α = 0.86). The SEFCQ total scale correlated positively with general self-efficacy (r = .32, P < .001) and quality of life (r = .20; P < .01) and negatively with anxiety (r = -.15; P < .05). Scores on the SEFCQ were higher in children without functional constipation compared to those with functional constipation (53.33 + 3.38 vs 39.34 + 7.19, P < .001). CONCLUSIONS & INFERENCES: We developed a constipation specific self-efficacy questionnaire with good initial internal reliability, excellent face validity and adequate content validity. A low self-efficacy for defecation, may make the child resist their physical urge to defecate and hence, the need for further studies to assess its effect on treatment outcomes.

Interobserver variability in antroduodenal manometry.

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.

Cyclic vomiting syndrome in adults.

Cyclic vomiting syndrome (CVS) was initially described in children but can occur in all age groups. Cyclic vomiting syndrome is increasingly recognized in adults. However, the lack of awareness of CVS in adults has led to small numbers of diagnosed patients and a paucity of published data on the causes, diagnosis and management of CVS in adults. This article is a state-of-knowledge overview on CVS in adults and is intended to provide a framework for management and further investigations into CVS in adults.

Ileal manometry in children following ileostomies and pull-through operations.

Our aim was to analyse the patterns of ileal contractions in children. We reviewed the charts of 23 children who had ileal manometry studies (16 males), mean age 7 years (range 2 months to 17 years). We positioned the manometry catheters with 4-8 recording sites, 5 or 15 cm apart, through ileostomies fashioned for clinically indicated reasons. We studied six additional children with persistent faecal soiling following endorectal pull through for Hirschsprung's disease; the catheters were positioned through the anus and colon into the ileum. We recorded phasic and tonic intermittent contractions in all the subjects, clustered contractions (rate 5-9 min-1, duration 20-120 s) in 19 subjects with ileostomies and four with endorectal pull throughs. In 13 children there were prolonged propagated contractions, > 60 mmHg in amplitude, > 15 s in duration, propagating at rates of 2-6 cm s-1 over at least 20 cm. The migrating motor complex was rare; in 55 h of fasting recording there were two phase III sequences. There are four distinctive features of ileal manometry recordings in children: random intermittent contractions, clustered contractions, prolonged propagated contractions and tonic contractions. The features of ileal motility differ from motility in the proximal small bowel.

Colonic diversion for intractable constipation in children: colonic manometry helps guide clinical decisions.

BACKGROUND: Colonic manometry helps discriminate functional and behavioral causes for childhood constipation from colonic neuromuscular disease. METHODS: Of 375 colonic manometries performed for clinical indications, 12 could not be interpreted because of chronic colonic dilation. Based on colonic manometries that showed either no contractions or an absence of the gastrocolonic response or an absence of high-amplitude propagating contractions, the authors recommended diverting colostomies or ileostomies in 12 chronically constipated children (mean age, 4 years; range, 2-14 years, 5 boys). Before study, medical treatment was ineffective in all children. These children had persistently dilated colons with pathologic diagnoses of intestinal neuronal dysplasia (n = 4), hypoganglionosis (n = 2), hollow visceral myopathy (n = 1), and normal (n = 5). RESULTS: Six to 30 months after diversion, the authors restudied all the children. Eleven of 12 diverted colons were no longer dilated. In two patients, abnormal motility involving the entire colon was unchanged from the initial study, small bowel motility was abnormal, and we recommended no further surgery. In two cases, the colon remained abnormal but small bowel motility was normal, and we recommended subtotal colectomy and ileoproctostomy. In four cases, the left colon remained abnormal, but the right colon was normal, and we recommended reanastomosis after left hemicolectomy. In four cases, motility in the diverted colons was normal, including a gastrocolonic response and high-amplitude propagating contractions, and the authors recommended reanastomosis. Defecation problems resolved in 10 of 12 when followed up 5 to 30 months after treatment. CONCLUSION: These data suggest that in some cases of intractable childhood constipation associated with colonic distention, temporary diversion improved colonic motility. Colonic manometry may be used to predict which patients will benefit from resection or reanastomosis.

Rapid maturation of gastric relaxation in newborn infants.

We studied gastric volume, wall compliance, sensory perception, and receptive relaxation during the first postnatal 80 h in 17 healthy term infants, using a computer-driven air pump and simultaneously measuring pressure and volume within a latex balloon placed through the oropharynx into the stomach. To evaluate gastric compliance, we measured pressures while we infused air into the intragastric balloon at different rates (10, 20, and 60 mL/min) in random sequence. In all infants, there was a linear relationship between intragastric pressure and volume to the maximum pressure tested, 30 mm Hg. Gastric compliance ranged from 0.2 mL/mm Hg to 3.8 mL/mm Hg. Different infusion rates had no effect on compliance. We calculated gastric receptive relaxation by measuring the volume needed to maintain a constant pressure of 10 mm Hg within the balloon for 5 min. Gastric receptive relaxation ranged from 0.5 mL/min to 54 mL/min. Gastric compliance and receptive relaxation increased with postnatal age (r = 0.70, p < 0.005; r = 0.79, p < 0.001, respectively) and with number of feedings (r = 0.80, r = 0.88, respectively, both p < 0.001). There was no correlation between weight or type of feeding (breast versus formula) and either gastric compliance or relaxation. In conclusion, these results may explain the small feedings that neonates ingest in the first days of life. During the first 3 postnatal d, the newborn stomach becomes more compliant and develops more receptive relaxation, associated with a larger volume capacity.

Causal explanations, concern and optimism regarding self-injurious behaviour displayed by individuals with Cornelia de Lange syndrome: the parents' perspective.

Research into behaviours associated with specific syndromes, such as Cornelia de Lange syndrome (CdLS), has neglected to explore the parents' perspective, particularly the potential impact of the notion of behavioural phenotypes on parents' causal explanations. Given the research focus on self-injurious behaviour (SIB) in CdLS, the present study examined parental concern across four topographies of challenging behaviour, causal explanations for these behaviours and optimism for change. As part of a larger study, a questionnaire survey of 86 parents of children and adults with CdLS was conducted. Quantitative data on parental concern and optimism with regard to behaviour problems were collected. Causal explanations for behaviour problems were examined by subjecting open-ended responses to a content analysis. Parents were as concerned about physical aggression and disruptive behaviours as they were about SIB. The majority of parents had deconstructed how CdLS might be associated with SIB in terms of other factors associated with CdLS, such as degree of intellectual disability. Parents did not believe that CdLS influenced SIB more than other challenging behaviours and their beliefs did not effect optimism regarding future change in the behaviour. Despite the focus of research on SIB in CdLS, parents of children and adults with CdLS are also concerned about other challenging behaviours. There was no evidence that a deterministic perspective had been adopted by parents and causal explanations were unrelated to optimism for future change.

A coordinated change in chemokine responsiveness guides plasma cell movements.

Antibody-secreting plasma cells are nonrecirculatory and lodge in splenic red pulp, lymph node medullary cords, and bone marrow. The factors that regulate plasma cell localization are poorly defined. Here we demonstrate that, compared with their B cell precursors, plasma cells exhibit increased chemotactic sensitivity to the CXCR4 ligand CXCL12. At the same time, they downregulate CXCR5 and CCR7 and have reduced responsiveness to the B and T zone chemokines CXCL13, CCL19, and CCL21. We demonstrate that CXCL12 is expressed within splenic red pulp and lymph node medullary cords as well as in bone marrow. In chimeric mice reconstituted with CXCR4-deficient fetal liver cells, plasma cells are mislocalized in the spleen, found in elevated numbers in blood, and fail to accumulate normally in the bone marrow. Our findings indicate that as B cells differentiate into plasma cells they undergo a coordinated change in chemokine responsiveness that regulates their movements in secondary lymphoid organs and promotes lodgment within the bone marrow.

Lesbians and economic/social change.

SUMMARY This article discusses the globalisation juggernaut and its impacts on women in general and lesbians in particular. It outlines the inadequacies of the pink dollar g/luppie image of lesbians, and considers what lesbian economics involve. This leads to outlining some political implications for our own communities of globalisation and its individualistic world, internally, in coalitions, and in our dealings with governments. It concludes that economics is an important influence on our communities, for good and ill, and that values and politics should be critical to how we handle economic issues. Continuing the creation of lesbian economy and value systems and influencing those of the straight world are ongoing, difficult tasks.

Coexpression of the chemokines ELC and SLC by T zone stromal cells and deletion of the ELC gene in the plt/plt mouse.

The spontaneous mutant mouse strain, plt/plt, lacks the secondary lymphoid organ chemokine (SLC)-ser gene and has disrupted trafficking of T cells and dendritic cells (DCs) to lymphoid tissues. We demonstrate here that the gene for the related chemokine, Epstein-Barr virus-induced molecule-1 ligand chemokine (ELC), is also deleted in this immunodeficient mouse strain. Using a combination of approaches, including bone marrow reconstitution and double in situ hybridization, we show in wild-type mice that ELC is expressed by T zone stromal cells that also make SLC. Smaller amounts of ELC are made by DCs, predominantly of the CD8(+) phenotype. We propose that ELC- and SLC-expressing T zone stromal cells play a central role in bringing naive T cells and DCs together for the initiation of immune responses.

Follicular stromal cells and lymphocyte homing to follicles.

Follicular dendritic cells (FDCs), the best defined stromal cell subset within lymphoid follicles, play a critical role in presenting intact antigen to B lymphocytes. The discovery that many follicular stromal cells make B-lymphocyte chemoattractant (BLC), a CXC chemokine that attracts CXCR5+ cells, provides a basis for understanding how motile B cells come into contact with stationary FDCs. Here we review our work on BLC and discuss properties of BLC-expressing follicular stromal cells. We also review the properties of primary follicle and germinal center FDCs and suggest a model of FDC development that incorporates information about BLC expression. Finally, we consider how antigen recognition causes T and B lymphocytes to undergo changes in chemokine responsiveness that may help direct their movements into, or out of, lymphoid follicles.

Function of hollow viscera in children with constipation and voiding difficulties.

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.

Colonic motility after surgery for Hirschsprung's disease.

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.

A chemokine-driven positive feedback loop organizes lymphoid follicles.

Lymphoid follicles are B-cell-rich compartments of lymphoid organs that function as sites of B-cell antigen encounter and differentiation. CXC chemokine receptor-5 (CXCR5) is required for B-cell migration to splenic follicles, but the requirements for homing to B-cell areas in lymph nodes remain to be defined. Here we show that lymph nodes contain two types of B-cell-rich compartment: follicles containing follicular dendritic cells, and areas lacking such cells. Using gene-targeted mice, we establish that B-lymphocyte chemoattractant (BLC/BCA1) and its receptor, CXCR5, are needed for B-cell homing to follicles in lymph nodes as well as in spleen. We also find that BLC is required for the development of most lymph nodes and Peyer's patches. In addition to mediating chemoattraction, BLC induces B cells to up-regulate membrane lymphotoxin alpha1beta2, a cytokine that promotes follicular dendritic cell development and BLC expression, establishing a positive feedback loop that is likely to be important in follicle development and homeostasis. In germinal centres the feedback loop is overridden, with B-cell lymphotoxin alpha1beta2 expression being induced by a mechanism independent of BLC.

Rumination syndrome in adolescents.

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.

Colonic motility in children with repaired imperforate anus.

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.