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Distinct facial appearance with nasal hypoplasia, constipation, severe mental retardation and hypotonia in two unrelated young males.

In this report we describe two unrelated young males with severe mental retardation, persisting hypotonia, and constipation. A maternal uncle of one of these two boys died at the age of 18 months and presented the same clinical symptoms. The triad mental retardation, hypotonia, constipation is a characteristic finding in the FG syndrome, an X-linked mental retardation syndrome. At the present time, there is increasing evidence that the FG syndrome-phenotype may be present in different XLMR conditions, e.g. the fragile X syndrome. In addition to the triad severe mental retardation, hypotonia, constipation, the present two male index patients had a characteristic facial appearance with nasal hypoplasia, relative microcephaly and pre- and postnatal overgrowth. The question is raised whether the present two males are examples of a specific entity within the FG-syndrome-like phenotype.

Abnormalities, Multiple↗

A comparative study: the efficacy of liquid paraffin and lactulose in management of chronic functional constipation.

OBJECTIVES: To determine and compare efficacy, safety and optimal dose of two laxatives, liquid paraffin and lactulose, in 40 children with chronic functional constipation. METHODS: A total of 20 children were treated with liquid paraffin and 20 with lactulose for 8 weeks and at an initial dose of 1 mL/kg per day for both drugs. The dose was adjusted every 3 days as required and a diary was kept to monitor dose, side-effects, stool frequency and consistency, and other symptoms. RESULTS: During first 4 weeks, improvement in stool consistency and frequency was significantly higher in liquid the paraffin group (P < 0.01 and P < 0.05, respectively). Improvement in the number of stools per week was also significantly higher in the liquid paraffin group during the last 4 weeks of therapy (P < 0.05). Compliance rates averaged 95% in the liquid paraffin group and 90% in the lactulose group during the first 4 weeks of therapy and 90% in the liquid paraffin group and 60% in the lactulose group during the last 4 weeks of therapy (chi(2) = 4.8, SD = 1, P = 0.02). During the first 4 weeks of therapy and during the last 4 weeks of therapy, mostly side-effects and poor symptom control, respectively, influenced the compliance in the liquid paraffin group. CONCLUSIONS: Liquid paraffin is more effective in the treatment of children with constipation. Patients treated with liquid paraffin responded more rapidly than patients responding to lactulose and showed fewer side-effect. It is considered that late response and side-effects in conjunction with poor symptom control influence the patient compliance and the successful treatment of childhood constipation.

Cathartics↗

Clinical significance of functional constipation categorized by colonic transit time and pelvic floor electromyography.

OBJECTIVE: Functional constipation (FC) is a common gastrointestinal disorder, the incidence of which is increasing each year with the changes in life style and diet, and the age of patients is becoming younger. Correct categorization will guide the treatment of FC and in the present study colonic transit time (CTT) and pelvic floor electromyography (PFE) were evaluated for their clinical significance in categorizing FC. METHODS: Thirty-two cases of FC diagnosed by Rome II criteria with a duration of 6 months to 30 years were enrolled. Radio-opaque markers were used to detect CTT, the transit index (TI) and colonic evacuation rate; PFE was used to observe whether there was any paradoxical movement between the pelvic floor and abdominal muscles. RESULTS: Colonic transit time was recorded in 32 patients: 20 had outlet obstruction constipation and 12 had slow transit type. In the outlet obstruction type, PFE showed varying degrees of paradoxical movement between the muscles, whereas in the slow transit type, only 4 cases showed it. CONCLUSION: Functional constipation can be categorized by CTT and PFE, which are simple, painless and feasible to perform.

Adult↗

Surgery for constipation.

Over a period of 30 years a total of 27 patients have been subjected to partial or complete colectomy and anastomosis for constipation. Patients fall into four groups: (i) functional constipation; (ii) adult megacolon; (iii) megasigmoid and (iv) persistent Hirschsprung's disease. The first two groups comprised 17 patients with resistant constipation, with or without megacolon or dolichocolon. Seven (41%) of these patients subsequently required operation for acute small-bowel obstruction due to adhesions. In two patients a permanent ileostomy was necessary for persistent rectal inertia after colectomy. The functional results in these first two groups were good. The third and fourth groups had similar presenting features; five had megasigmoid, and in these resection of the sigmoid colon gave good results. The remaining five patients with proven Hirschsprung's disease responded well to a pull-through resection (4) and to colectomy and anastomosis (1).

Adolescent↗

Severe constipation as the presenting complaint of phaeochromocytoma.

Phaeochromocytoma is a rare tumour which may present with gastrointestinal features. We report two patients who presented with severe constipation and were found to have a malignant phaeochromocytoma. Both patients deteriorated following barium contrast radiology. In the first patient, constipation improved following pharmacological manipulation with alpha and beta adrenoreceptor antagonists. The diagnosis of phaeochromocytoma must be considered in a constipated patient with the features of catecholamine excess.

Adrenal Gland Neoplasms↗

Long-term results of the antegrade continent enema procedure for constipation in adults.

OBJECTIVE: The aim of this study was to evaluate the long-term results of the Antegrade Continent Enema (ACE) procedure for treating severe constipation in adults. METHODS: Over 10 years 37 ACE conduits were created in 32 patients (median age 35 years, 26 women) with constipation caused by slow transit, obstructed defaecation or both. Conduits were created from the appendix (n = 20, 54%), ileum (n = 10, 27%), neoappendix caecostomy (n = 5, 14%) or colon (n = 2, 5%). Clinical records were retrospectively reviewed to determine outcome. RESULTS: After a median follow up of 36 (range 13-140) months, 28 (88%) required at least one further procedure on a primary conduit, including reversal in 19 (59%). Five patients had a second conduit fashioned, two successfully. Conduit type and constipation cause did not significantly influence the rates of ACE reversal or major revision. Ileal conduits were associated with fewer minor revision procedures for stenosis (1 in 7 patients) than appendix conduits (21 in 20 patients). There was one (3%) serious complication. Satisfactory ACE function was ultimately achieved in 47% of patients, at last follow up. After ACE reversal, 9 (28%) patients underwent formation of an end stoma and 3 patients had a colectomy. CONCLUSIONS: Revision procedures are common, but approximately half of patients can expect satisfactory long-term ACE function. ACE conduit reversal does not preclude subsequent alternative surgical strategies to treat this difficult condition.

Adolescent↗

Scintigraphic assessment of slow transit constipation with special reference to right- or left-sided colonic delay.

OBJECTIVE: Subtotal colectomy and ileorectal anastomosis for slow transit constipation has several side-effects. The motor abnormality in some patients may be segmental which could motivate a limited resection of the colon. Therefore a diagnostic tool to identify a segmental colonic motor dysfunction is needed. The aim of this study was to evaluate a scintigraphic method to assess colonic transit with special reference to right- or left-sided delay. METHODS: Twenty-three constipated patients (19 women, mean age 50 years) with slow colonic transit on radio-opaque marker studies and 13 healthy individuals (11 women, mean age 46 years) were studied. All subjects were examined with oral (111)Indium-DTPA scintigraphy. The scintigraphic results for patients and controls were presented as geometric centre of radioactivity and percent activity over time in the right, the left and the recto-sigmoid colon. The inter-observer variation in the interpretation of the scans was also evaluated. RESULTS: There was no difference in transit time between the groups of patients and controls in the right colon whereas the patients had a significant delay in the left colon (P < 0.05). Two patients had a marked delay in the right colon followed by relatively rapid transit in the left colon. The inter-observer correlation was good comparing the right, the left and the recto-sigmoid colon (r = 0.58-0.98, P < 0.01-0.001). CONCLUSION: The results indicate that colonic scintigraphy with oral (111)Indium-DTPA may help to select patients for a left or, in a few cases, a right hemicolectomy for slow transit constipation.

Adult↗

Incidence of urinary incontinence and constipation during pregnancy and postpartum: survey of current findings at the Rotunda Lying-In Hospital.

OBJECTIVE: To assess the impact of pregnancy upon continence and constipation. DESIGN: A questionnaire survey. SETTING: Maternity wards in the Rotunda Lying In Hospital, Dublin, Republic of Ireland. POPULATION: 7771 women who were delivered of liveborn infants. METHODS: Questionnaires were delivered and collected by physiotherapy staff as part of routine postnatal care. RESULTS: Analysis of data using chi2 tests showed significant differences between three parity groups [primigravidae, multigravidae (2-4) and multigravidae (5+)] for symptoms of both urinary incontinence (chi2 = 119.54, df = 2, P = 0.000) and constipation (chi2 = 12.53, df = 3, P = 0.002); the incidence of both constipation and urinary incontinence increased with parity. CONCLUSION: The results of this survey have emphasised the relation between parity and postpartum incontinence which stresses the importance of early diagnosis and intervention.

Constipation↗

Fruit and fibre (Pajala porridge) in the prevention of constipation.

BACKGROUND AND AIMS: Constipation is a common problem in geriatric wards and in the elderly population. Although high-fibre diets can help relieve constipation non-pharmacologically in many patients, traditional laxatives still remain the standard treatment. A fibre supplement in the form of raw bran is not always well tolerated. We wanted to study the effects of a daily consumption of a fruit- and fibre-rich porridge on stool frequency, perceived well-being and the costs for laxatives, when compared with traditional treatment with laxatives, in geriatric patients. METHODS: Twenty patients in secondary geriatric wards (hospital rehabilitation wards) were randomized into an intervention group (porridge group) and a control group (standard diet without porridge) for a 1-week run-in and 2-week study, with registration of clinical data, e.g. medical treatment, laxative consumption, stool frequency and perceived well-being. RESULTS: The patients in the porridge group had a daily defaecation without laxatives on average 76% of the time (10.7/14 days) compared with 23% of the time (3.3/14 days) in the non-porridge group (p = 0.003). The discomfort was less in the porridge group (2.5 vs. 6.5 on a 10-degree visual analogue scale, p = 0.008) when compared with the control group. The cost for laxatives was 93% lower in the intervention group (2.5 vs. 37.5) for the 2-week study. CONCLUSIONS: A fibre-rich porridge was effective, well liked and tolerated and reduced the need for laxatives in geriatric patients. We conclude that a daily fibre-rich meal ought to be included in the treatment strategies of constipation in hospital wards.

Aged↗

Treatment of intractable constipation with orlistat: a report of three cases.

Intractable constipation is a frequent side effect of opioid treatment of chronic pain. Orlistat, an FDA approved pill for weight loss, has gastrointestinal side effects including fecal incontinence, oily spotting, fecal urgency, and increased defecation. The author postulated that capitalizing on the known side effects of orlistat and prescribing orlistat to obese, chronic pain patients with opioid induced constipation would provide relief. The following report describes three positive cases of the use of orlistat for the treatment of opioid related constipation.

Adult↗

Toward evidence-based prescribing at end of life: a comparative analysis of sustained-release morphine, oxycodone, and transdermal fentanyl, with pain, constipation, and caregiver interaction outcomes in hospice patients.

OBJECTIVE: The primary goal of this investigation was to examine selected outcomes in hospice patients who are prescribed one of three sustained-release opioid preparations. The outcomes examined include: pain score, constipation severity, and ability of the patient to communicate with caregivers. PATIENTS AND SETTINGS: This study included 12,000 terminally ill patients consecutively admitted to hospices and receiving pharmaceutical care services between the period of July 1 and December 31, 2002. DESIGN: We retrospectively examined prescribing patterns of sustained-release morphine, oxycodone, and transdermal fentanyl. We compared individual opioids on the aforementioned outcome markers, as well as patient gender, terminal diagnosis, and median length of stay. RESULTS: Patients prescribed a sustained-release opioid had similar average ratings of pain and constipation severity, regardless of the agent chosen. Patients prescribed transdermal fentanyl were reported to have more difficulty communicating with friends and family when compared with patients prescribed either morphine or oxycodone. On average, patients prescribed transdermal fentanyl had a shorter length of stay on hospice as compared with those receiving morphine or oxycodone. CONCLUSION: There was no difference in observed pain or constipation severity among patients prescribed sustained-release opioid preparations. Patients receiving fentanyl were likely to have been prescribed the medication due to advanced illness and associated dysphagia. Diminished ability to communicate with caregivers and a shorter hospice course would be consistent with this profile. Further investigation is warranted to examine the correlation between a patient's ability to interact with caregivers and pain control achieved.

Aged↗

Circulating gastrointestinal hormone abnormalities in patients with severe idiopathic constipation.

OBJECTIVE: This study aimed to determine if there is an abnormality of circulating gastrointestinal hormones in patients with severe idiopathic constipation. METHODS: Twelve patients, all female (median age 34 yr) and 12 healthy controls (eight female, median age 32 yr) were studied. A radioisotope-labeled solid/liquid meal was ingested, and the serum hormone response, as well as the relationship between serum hormones and rates of gastric emptying and small intestinal transit, were studied for 180 min postprandially. RESULTS: Somatostatin levels were higher in patients with constipation (basal level, controls vs patients, 31 vs 57 pmol/L, p < 0.05, median values; peak level, 48 vs 60, p < 0.05). Patients showed a significantly lower somatostatin integrated incremental meal response (2182 vs 104, p < 0.05). No correlation was found between the somatostatin levels and rates of upper gastrointestinal transit in patients. Pancreatic glucagon was significantly decreased (p=0.04). Enteroglucagon levels were significantly lower (p > 0.05) in patients between 30 and 60 min after the meal. The peak found after the meal in normal subjects was absent. Basal levels of pancreatic glucagon correlated with small bowel transit by two different measures: head of meal (r=0.69, p=0.03) and cecal filling at the time of 50% gastric emptying (r=0.84, p=0.002). No significant differences between the two groups could be found for basal and peak levels at different times and integrated incremental response to the meal for insulin, gastric inhibitory polypeptide (GIP), glucagon-like peptide-1 (GLP-1), cholecystokinin (CCK), gastrin, pancreatic polypeptide (PP), motilin, neurotensin, and peptide tyrosine tyrosine (PYY). CONCLUSION: Patients with severe idiopathic constipation have specific abnormalities of circulating gut hormones that most likely play a role in gastrointestinal motility and that may be of pathophysiological significance.

Acute Disease↗

Psychological state and quality of life in patients having behavioral treatment (biofeedback) for intractable constipation.

OBJECTIVE: Behavioral techniques, including biofeedback, improve symptoms in a majority of patients with intractable idiopathic constipation. However, it is unknown whether there is also improvement in patients' psychological well-being and quality of life (QOL). It is also unknown whether psychological or QOL factors can be used to predict the response to treatment. We aimed to assess these factors prospectively. METHODS: Thirty-one consecutive patients (median age 36 yr) receiving biofeedback retraining for idiopathic constipation were studied. A bowel symptom record, the General Health Questionnaire-28 (GHQ-28) (n = 31) and Hospital Anxiety and Depression Scale (n = 20), both psychological measures, and the Short Form-36 (SF-36) (n = 22), a generic QOL measure, were recorded before and after treatment. RESULTS: Twenty-two of the patients felt subjectively symptomatically improved. When considering all patients, treatment resulted in decreased depression (p < 0.05), anxiety (p < 0.05), and somatic symptoms (p < 0.01) (psychological measures: GHQ-28), and improved general health (p < 0.05) and vitality (p < 0.05) (QOL measures: SF-36). Patients who improved symptomatically showed significantly higher scores on the GHQ-28 and SF-36, in contrast to the unchanged scores in patients who did not symptomatically improve. Pretreatment GHQ-28 did not predict who would benefit from treatment, but patients in whom pain, emotional problems, or low vitality interfered with daily living (SF-36 pain subscales) were significantly (p < 0.05) less likely to respond to treatment. CONCLUSIONS: The symptomatic improvement produced by biofeedback in constipated patients is associated with improved psychological state and QOL. Some of the psychological morbidity in these patients is reversible. QOL parameters may be useful for predicting a likely response to treatment.

Adult↗

Efficacy and safety of traditional medical therapies for chronic constipation: systematic review.

OBJECTIVES: Constipation is common, and its treatment is unsatisfactory. Although many agents have been tried, there are limited data to support their use. Our aim was to undertake a systematic review of the efficacy and safety of traditional medical therapies for chronic constipation and to make evidence-based recommendations. METHODS: We searched the English literature for drug trials evaluating treatment of constipation by using MEDLINE and PUBMED databases from 1966 to 2003. Only studies that were randomized, conducted on adult subjects, and published as full manuscripts were included. Studies were assigned a quality score based on published methodology. Standard forms were used to abstract data regarding study design, duration, outcome measures, and adverse events. By using the cumulative evidence of published data for each agent, recommendations were made regarding their use following the United States Preventive Services Task Force guidelines. RESULTS: Good evidence (Grade A) was found to support the use of polyethylene glycol (PEG) and tegaserod. Moderate evidence (Grade B) was found to support the use of psyllium, and lactulose. There was a paucity of quality data regarding many commonly used agents including milk of magnesia, senna, bisacodyl, and stool softeners. CONCLUSIONS: There is good evidence to support the use of PEG, tegaserod, lactulose, and psyllium. Surprisingly, there is a paucity of trials for many commonly used agents. These aspects should be considered when designing trials comparing new agents with traditional therapies because their use may not be well validated.

Adult↗

Chronic constipation: assessment and management in the elderly.

This manuscript presents a comprehensive look at the assessment and management of chronic constipation in the elderly. Chronic constipation is a common problem among the elderly. Pharmacologic and nonpharmacologic treatment measures can be used to manage this problem. This information can serve the practitioner as a practice guideline in the diagnosis and treatment of chronic constipation in the elderly.

Age Factors↗

Parent satisfaction in a nurse led clinic compared with a paediatric gastroenterology clinic for the management of intractable, functional constipation.

AIMS: To assess parent satisfaction with a nurse led clinic for children with intractable, functional constipation compared with a paediatric gastroenterology outpatient clinic. METHODS: A validated questionnaire covering six separate domains in 48 statements (provision of information, empathy with the patient, technical quality and competence, attitude towards the patient, access to and continuity with the caregiver, and overall satisfaction) was employed. For all outcomes, the data were analysed on an intention-to-treat basis. RESULTS: 90/107 (84%) questionnaires were returned: 40/51 (78%) from the paediatric gastroenterology clinic and 50/56 (89%) from the nurse led clinic. Results indicated a high "total" satisfaction with the clinical care, but these were statistically significantly higher in the nurse led clinic compared with the paediatric gastroenterology clinic (median score 8.7 and 7.3 respectively, out of a maximum score of 10). This difference was consistent across all domains when the scores from those attending the nurse led clinic were compared with those attending the paediatric gastroenterology clinic (information median score 8.7 v 7.5; empathy 9.0 v 7.3; competence 9.1 v 8.0; attitude 8.7 v 7.3; access 8.2 v 6.7). All comparisons were highly statistically significant. CONCLUSION: These results provide firm evidence that parents of children with intractable constipation are satisfied with the care they receive in both the paediatric and nursing clinic setting. Parent satisfaction, however, was significantly higher in those attending the nurse led clinic. This study adds further support to the development of a nurse led service to manage intractable, functional constipation in children.

Adolescent↗

Constipation and reversible urinary tract abnormalities.

Urinary tract anomalies were prospectively investigated with ultrasound in 29 children with functional constipation. These children were compared before and after treatment with 451 age matched healthy controls without constipation. The bladder residue and upper renal tract dilatation after micturition were significantly increased in the group with constipation and improved after treatment.

Adolescent↗

Megarectum in constipation.

BACKGROUND: Faecal impaction is frequently observed in children with chronic constipation. The term megarectum is often used to describe this finding. AIM: To evaluate rectal functioning and rectal measures in constipated children with a filled rectum, in order to define the terms faecal impaction, enlarged rectum, and megarectum. METHODS: All children underwent radiological investigation, colonic transit time study, anorectal manometry, and rectal volume and rectal wall compliance measurements. Patients with faecal impaction were compared with controls, who had an empty rectum on digital rectal examination. RESULTS: A total of 31 patients and six controls were included in the study. The mean duration of complaints was 4.2 years and all had faecal incontinence. The colonic transit times in the patients showed a distinct delay in the rectosigmoid segment. Anorectal manometry was not significantly different between patients and controls. The rectal width in patients was 0.68 and in controls 0.52 with an upper limit of 0.61. The pressure-volume curve in patients showed significant less relaxation at a distension of 50 ml. The slope of the curve (corresponding with rectal wall compliance) was comparable for patients and controls. CONCLUSIONS: We suggest that faecal impaction is a filled rectum found on digital rectal examination; an enlarged rectum is defined by a rectopelvic ratio greater than 0.61; and megarectum is defined in those with significant abnormalities found with anorectal manometry, pressure-volume curves, or rectal compliance investigation. A diminished relaxation of the rectum on rectal distension could be the first sign of megarectum in children with chronic constipation.

Adolescent↗