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Cureus Aug 2022is a gram-positive, anaerobic, spore-forming bacterium that produces toxins A and B, disrupting the intestinal brush border and resulting in severe diarrhea. The most... (Review)
Review
is a gram-positive, anaerobic, spore-forming bacterium that produces toxins A and B, disrupting the intestinal brush border and resulting in severe diarrhea. The most common causes of infection include prolonged antibiotic use, proton pump inhibitors (PPIs), and long-term hospitalization resulting in complications such as pseudomembranous colitis and toxic megacolon. This systematic review aims to consider fecal microbiota transplantation (FMT) as an early treatment modality in infection to prevent complications and reduce related morbidity and mortality. We systematically screened three databases using regular keywords such as "fecal microbiota transplantation," "," "pseudomembranous colitis," and "toxic megacolon" and Medical Subject Headings (MeSH) terms. We applied the inclusion and exclusion criteria and performed a thorough quality appraisal using standardized checklists. We were finally left with 10 articles, including seven case reports, one case series, and two observational studies. Questions remain as to the route of administration of FMT, timing, safety, availability, and the number of sittings required. More randomized controlled trials are needed to address all these questions and to assess the safety of FMT. We believe the role of FMT is very important as it can prevent related complications and would be an ideal treatment option in a population group that is often unfit for surgical management.
PubMed: 36168377
DOI: 10.7759/cureus.28402 -
Archives of Disease in Childhood Dec 2017The objective of this study was to develop a Hirschsprung's disease (HD) core outcome set (COS).
OBJECTIVE
The objective of this study was to develop a Hirschsprung's disease (HD) core outcome set (COS).
METHODS
Candidate outcomes were identified from a systematic review and stakeholder nomination. A three-phase Delphi process and consensus meeting were used to prioritise candidate outcomes based on scores assigned by stakeholder participants using a nine-point scale. In phases two and three, participants were shown graphical representations of their panel's scores and all panels' scores respectively for each outcome from the previous phase. After the third phase, outcomes prioritised by two or three panels were taken forward to the consensus meeting. The COS was formed from the 10 highest scoring outcomes meeting the threshold for inclusion (≥70% 7-9 and <15% 1-3).
RESULTS
Eighty-nine stakeholders (82%) completed all three phases of the Delphi process. Seventy-four outcomes were assessed in phase one of the Delphi process, the following 10 of which met criteria for inclusion in the COS: (1) death with cause specified, (2) long-term faecal incontinence, (3) long-term voluntary bowel movements without need for enemas, or rectal or colonic irrigation, (4) long-term psychological stress for the individual with Hirschsprung's disease, (5) long-term urinary incontinence, (6) objective score of quality of life, (7) objective score of bowel function, (8) unplanned reoperation, (9) >need for a permanent stoma, (10) enterocolitis.
CONCLUSIONS
This HD COS is formed of 10 outcomes deemed important by key stakeholders. Use of this COS in research will reduce outcome reporting heterogeneity and increase our ability to identify gold standard treatments for HD.
Topics: Adolescent; Child; Child, Preschool; Delphi Technique; Developed Countries; Hirschsprung Disease; Humans; Infant; Infant, Newborn; Patient Reported Outcome Measures; Severity of Illness Index; Stakeholder Participation; Treatment Outcome
PubMed: 28784616
DOI: 10.1136/archdischild-2017-312901 -
Pediatric Surgery International Feb 2018Hirschsprung-associated enterocolitis (HAEC) is a life-threatening complication of Hirschsprung's disease. HAEC is reported to occur in 6-50% of patients preoperatively... (Meta-Analysis)
Meta-Analysis Review
BACKGROUND/PURPOSE
Hirschsprung-associated enterocolitis (HAEC) is a life-threatening complication of Hirschsprung's disease. HAEC is reported to occur in 6-50% of patients preoperatively and in 2-35% postoperatively. The exact cause of HAEC is not fully understood, but disturbances of intestinal microbiota have recently been reported in patients with HAEC. In recent years, the administration of probiotics has been proposed to reduce the incidence of HAEC. We conducted a systematic review and meta-analysis to determine the effect of probiotics on postoperative HAEC.
METHODS
A systematic literature search for relevant articles was performed in four databases using the combinations of following terms "probiotics", "microbiota", "enterocolitis", "Lactobacillus", "Bifidobacterium", "Saccharomyces", "Streptococcus", and "Hirschsprung disease/Hirschsprung's disease" for studies published between 2002 and 2017. The relevant cohorts of the effect of probiotics in postoperative patients were systematically searched for clinical outcomes. Odds ratio (OR) or standard mean difference (SMD) with 95% confidence intervals (CI) were calculated using standardized statistical methodology.
RESULTS
The search strategy identified 1274 reports. Overall, five studies met defined inclusion criteria, reporting a total of 198 patients. Two studies were prospective multicenter randomized control trials. Lactobacillus, Bifidobacterium, Streptococcus, and Enterococcus were used as probiotics. The incidence of HAEC with/without probiotics was 22.6 and 30.5%, respectively, but this was not statistically different (OR 0.72; 95% CI 0.37-1.39; P = 0.33).
CONCLUSION
This study shows that the administration of probiotics was not associated with a significant reduction in the risk of HAEC. Additional studies are required to understand more fully the role of microbiota and complex interactions that cause HAEC. With increasing knowledge of the role of microbiota in HAEC, we are likely to understand better the potential benefits of probiotics in this disease.
Topics: Enterocolitis; Gastrointestinal Microbiome; Hirschsprung Disease; Humans; Probiotics
PubMed: 28983778
DOI: 10.1007/s00383-017-4188-y -
Pediatric Surgery International Aug 2015Hirschsprung's disease (HSCR) is cited as a classical component in the constellation of features found in children with Mowat-Wilson syndrome (MWS), which is caused by a... (Review)
Review
BACKGROUND
Hirschsprung's disease (HSCR) is cited as a classical component in the constellation of features found in children with Mowat-Wilson syndrome (MWS), which is caused by a mutation of the ZEB2 gene. The prevalence and phenotype of HSCR in those with MWS has yet to be determined. Similarly, it is not known if children with MWS who undergo a curative pull-through operation experience similar functional outcomes. We aimed to delineate the clinical features of those with MWS and HSCR and to determine if these patients experience unfavourable outcomes following pull-through surgery.
METHODS
A systematic review of the literature using the key search term "Mowat Wilson" was performed using three online databases. Clinical data were collected on all patients with a diagnosis of MWS confirmed by ZEB2 analysis. Data regarding bowel function in children with biopsy-proven HSCR were recorded where available. Statistical analysis was performed using SPSS (v. 20.0).
RESULTS
Fifty-two articles were reviewed in the final analysis, incorporating data on 256 patients with a diagnosis of MWS. HSCR was diagnosed in 111 patients (43.4%). Males with HSCR had a slightly increased risk of genital tract anomalies (e.g. hypospadias) compared to those without HSCR (RR 1.79, p = 0.05). Data pertaining to disease phenotype and functional outcome were only available on 42 and 13 patients, respectively. Rectosigmoid aganglionosis was the most common sub-type of HSCR, being described 26 patients (66.7%), albeit accounting for a lower proportion than would normally be expected in an HSCR population. Only two patients (15.4%) were described as having normal bowel function at follow-up with the remainder having terminal stomas, or experiencing troublesome persistent bowel symptoms and recurrent enterocolitis.
CONCLUSION
Hirschsprung's disease is present in approximately 45% of patients diagnosed with MWS. Although there is a relative lack of data available on the clinical phenotype of HSCR in this group and their functional outcome following pull-through operation, our data suggest an increased prevalence of long-segment aganglionosis and an increased risk of clinically significant persistent bowel symptoms following pull-through surgery, in many cases necessitating terminal stoma formation.
Topics: Child; Facies; Hirschsprung Disease; Humans; Intellectual Disability; Microcephaly
PubMed: 26156877
DOI: 10.1007/s00383-015-3732-x -
Pediatric Surgery International Aug 2017Hirschsprung's disease (HSCR) is known to occur in families. The reported overall incidence of familial cases is 7.6%, with a higher incidence of 15-21% in total colonic... (Meta-Analysis)
Meta-Analysis Review
AIM OF THE STUDY
Hirschsprung's disease (HSCR) is known to occur in families. The reported overall incidence of familial cases is 7.6%, with a higher incidence of 15-21% in total colonic aganglionosis and 50% in the rare total intestinal aganglionosis. HSCR is extremely rare in twins. The aim of this study was to systematically analyse the patterns of HSCR in twins published in the literature.
METHODS
Electronic databases Pubmed and Medline were screened for relevant articles using the keywords "Hirschsprung's disease", "aganglionosis", "twins", "monozygotic", and "dizygotic". Examining reference lists identified further relevant papers.
MAIN RESULTS
Twelve studies with a total of 18 twin pairs were included in this analysis. 67% twins were discordant. HSCR was found in 24 out of 36 twin subjects (67%), of which 83% affected were male. Rectosigmoid type was reported in 71% of patients, long-segment disease in 21, and 8% presented with a total aganglionosis. Three twin pairs had at least one family member affected with HSCR.
CONCLUSION
HSCR was found in two-thirds of twin subjects with a male predominance. Rectosigmoid aganglionosis was the most common variant. Disease discordance was identified, where environmental insults were postulated to be predisposing factors to disease expression. Future studies investigating the disease-associated mutations in the already identified HSCR genes should provide insights into the genetic basis of HSCR in twins.
Topics: Diseases in Twins; Female; Hirschsprung Disease; Humans; Incidence; Male; Twins
PubMed: 28601901
DOI: 10.1007/s00383-017-4110-7 -
Balkan Medical Journal Jan 2021Hirschsprung's disease and sigmoid volvulus can sometimes be seen in the same patient.
BACKGROUND
Hirschsprung's disease and sigmoid volvulus can sometimes be seen in the same patient.
AIMS
To investigate the presence of Hirschsprung's disease in patients with sigmoid volvulus and to discuss the diagnosis and treatment methods.
STUDY DESIGN
Systematic review.
METHODS
This systematic review has been reported in line with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses and the methodological quality of systematic reviews guidelines. The PubMed and Scopus databases were scanned using the keywords "Hirschsprung* volvulus*" and "congenital aganglionic megacolon volvulus*". The reference list of the selected studies was reviewed for cross-checking. Two reviewers independently screened the available literature. Only the Hirschsprung's disease cases involving sigmoid volvulus were included, and cases of patients with volvulus in other sites was excluded. There was no restriction with respect to the publication language and type of writing. The primary outcome was morbidity and mortality.
RESULTS
A total of 31 cases were analyzed in 22 articles; 97% of the patients were under the age of 40, 90% were male. There was a statistically significant difference in the necessity for relaparotomy between patients who were scheduled for sigmoid volvulus therapy with the suspicion of Hirschsprung's disease and patients who were treated without suspicion of Hirschsprung's disease (0% vs 37.5%, p=0.02). While there was no postoperative death in cases with suspected Hirschsprung disease, this mortality rate was 25% in cases without suspicion (p = 0.08).
CONCLUSION
Hirschsprung's disease should be excluded with rectal biopsy if a patient with sigmoid volvulus is under 40 years of age and has complaints of constipation from childhood.
Topics: Adult; Aged, 80 and over; Child; Child, Preschool; Female; Hirschsprung Disease; Humans; Infant; Infant, Newborn; Intestinal Volvulus; Male
PubMed: 32856883
DOI: 10.4274/balkanmedj.galenos.2020.2020.4.131 -
Journal of Pediatric Urology Dec 2016There is a paucity of recent evidence regarding long-term urological and sexual outcomes following surgery for Hirschsprung disease (HD). We aimed to undertake a... (Review)
Review
PURPOSE
There is a paucity of recent evidence regarding long-term urological and sexual outcomes following surgery for Hirschsprung disease (HD). We aimed to undertake a systematic review of all HD literature to define these outcomes.
MATERIALS AND METHODS
A systematic literature search was conducted on studies from 1966 to 2014. Relevant articles were assessed for urological/sexual operative complications and functional sequelae. Studies were analysed in qualitative (Rangel score) and quantitative syntheses.
RESULTS
Initially 257 reports were assessed, with 24 studies were eligible for inclusion (1972-2014). Mean study quality was 16.5 ± SD 4.8 (range 6-23), indicating overall fair/poor quality. Ten studies (1021 patients) reported operative complications, with ureteric/urethral/vaginal injury occurring in seven (0.7%) patients. In three studies, the primary outcome was urological functional assessment. From 17 studies, 52/2546 patients (2.0%) had reported urinary incontinence. In infants, absent spontaneous erections post-operatively was reported in 3/203 patients (1.5%, 5 studies); of these 3, parents did not note spontaneous erections pre-operatively either. In older patients, erectile dysfunction occurred in 6/498 (1.2%) males. Other sexual outcomes were reported in 10 studies, with 5/10 studies (416 patients) reporting no erectile dysfunction. In the other studies reports ranged from non-specified sexual dysfunction in one study to diverse sexual related problems in nine (7.8%) of their patients in another.
CONCLUSIONS
Urological/sexual outcomes are rarely reported after HD surgery (24 studies over 42 years). Study quality is usually poor and a large proportion of the studies are more than 30 years old. In the majority of series it is unclear whether urological and sexual function impairments were not present or if they were not assessed. Prospective reporting of urological/sexual outcome is required, in particular in the era of new surgical techniques/approaches to HD.
Topics: Adult; Child; Hirschsprung Disease; Humans; Male; Postoperative Complications; Sexual Dysfunction, Physiological; Treatment Outcome; Urologic Diseases
PubMed: 27733240
DOI: 10.1016/j.jpurol.2016.07.019 -
Diseases of the Colon and Rectum Dec 2022In patients with ulcerative colitis or familial adenomatous polyposis who develop neoplasia or fail medical therapy and require colectomy, restorative proctocolectomy...
BACKGROUND
In patients with ulcerative colitis or familial adenomatous polyposis who develop neoplasia or fail medical therapy and require colectomy, restorative proctocolectomy with IPAA is often indicated. Although often well tolerated, IPAA can be complicated by cuffitis or inflammation of the remaining rectal cuff. Although much has been published on this subject, there is no clear and comprehensive synthesis of the literature regarding cuffitis.
METHODS
Our systematic literature review analyzes 34 articles to assess the frequency, cause, pathogenesis, diagnosis, classification, complications, and treatment of cuffitis.
RESULTS
Cuffitis occurs in an estimated 10.2% to 30.1% of pouch patients. Purported risk factors include rectal cuff length >2 cm, pouch-rectal anastomosis, stapled anastomosis, J-pouch configuration, 2- or 3-stage IPAA, preoperative Clostridium difficile infection, toxic megacolon, fulminant colitis, preoperative biologic use, medically refractory disease, immunomodulator/steroids use within 3 months of surgery, extraintestinal manifestations of IBD, and BMI <18.5 kg/m2 at the time of colectomy. Adverse consequences associated with cuffitis include decreased quality-of-life scores, increased risk for pouchitis, pouch failure, pouch excision, and pouch neoplasia.
CONCLUSIONS
Given the similarities between pouchitis and cuffitis, diagnosis and treatment of cuffitis should proceed according to the International Ileal Pouch Consortium guidelines. This review found that the majority of the current literature fails to distinguish between classic cuffitis (a form of reminant ulcerative proctitis) and nonclassic cuffitis (resulting from other causes). Further work is needed to distinguish the unique risk factors and endoscopic characteristics associated with each subtype, and further randomized clinical trials should be conducted to strengthen the evidence for treatment options.
Topics: Humans; Pouchitis; Proctocolectomy, Restorative; Colonic Pouches; Colitis, Ulcerative; Adenomatous Polyposis Coli
PubMed: 36399769
DOI: 10.1097/DCR.0000000000002593 -
Pediatric Surgery International Jan 2022Hirschsprung's associated enterocolitis (HAEC) is a complication of Hirschsprung's Disease (HD) with considerable morbidity and mortality. The variability in...
PURPOSE
Hirschsprung's associated enterocolitis (HAEC) is a complication of Hirschsprung's Disease (HD) with considerable morbidity and mortality. The variability in presentation leads to a wide variety of the reported prevalence pre-and postoperatively. This systematic review aimed to clarify the prevalence of HAEC in short-(S-HD), long (L-HD), TCA and the type of operation used.
METHODS
A systematic literature-based search for relevant cohorts was performed using Pubmed/Medline, Cochrane Library from its inception to May 2021. Studies reporting on pre-and postoperative enterocolitis, segment length, and surgical procedure (Soave, Swenson, Duhamel) were included. Pooled prevalence and subgroup analysis have been calculated for pre-and postoperative HAEC.
RESULTS
4738 articles were identified from the literature search, among which 57 studies, including 9744 preoperative and 8568 postoperative patients, were included. The groups were sorted by length of the aganglionic segment for further analysis. The pooled prevalence for preoperative HAEC was 18.3% for all types, 15.2% for S-HD and 26.1% for TCA. The pooled prevalence for postoperative HAEC was in total 18.2% for all segment lengths and used techniques. Subgroup analysis showed no significant difference in the occurrence of postoperative enterocolitis between the three techniques.
CONCLUSION
The prevalence of preoperative HAEC increases with segment length. However, pooled data suggest that the postoperative risk for developing HAEC, independently of the employed method and segment length, is comparable to the preoperative risk.
Topics: Enterocolitis; Hirschsprung Disease; Humans; Infant; Morbidity; Postoperative Complications; Postoperative Period; Prevalence
PubMed: 34595554
DOI: 10.1007/s00383-021-05020-y -
World Journal of Gastroenterology Apr 2022Patients with Hirschsprung disease (HD) are at risk of persistent constipation, fecal incontinence or recurrent enterocolitis after surgical treatment, which in turn may... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Patients with Hirschsprung disease (HD) are at risk of persistent constipation, fecal incontinence or recurrent enterocolitis after surgical treatment, which in turn may impact physical and psychosocial functioning. Generic health-related quality of life (HRQoL) and disease-specific health-related quality of life are relevant outcome measures to assess the impact of HD on the QoL of these patients.
AIM
To summarize all available evidence on HRQoL of patients with HD after surgery and the impact of possible moderating factors.
METHODS
Pubmed, Web of Sciences, PsycInfo and Embase were searched with search terms related to 'Hirschsprung disease', 'Pediatrics' and 'Quality of life'. Mean and standard deviation of generic HRQoL overall and domain scores were extracted from each study, as well as data describing potential factors associated with QoL. Random effect models were used for meta-analytic aggregation of generic HRQoL scores. Meta-regression was used to assess the relationship between patient and clinical characteristics and generic HRQoL. Disease-specific HRQoL outcomes of patients with HD were systematically reviewed.
RESULTS
Seventeen articles were included in the systematic review ( = 1137 patients) and 15 in the quantitative meta-analysis ( = 1024 patients). Four studies reported disease-specific HRQoL. Patient's age ranged between 0 and 21 years. Meta-analytic aggregation showed a non-significantly impaired generic HRQoL ( = -0.168 [95%CI: -0.481; 0.145], = 0.293, = 94.9) in patients with HD compared to healthy controls. Physical ( = -0.042 [95%CI: -0.419; 0.335], = 0.829, = 95.1), psychosocial ( = -0.159 [95%CI: -0.458; 0.141], = 0.299, = 93.6) and social HRQoL ( = -0.092 [95%CI: -0.642; 0.457], = 0.742, = 92.3) were also not significantly lower compared to healthy controls. There was no relation between health-related outcomes and the sex of the patients and whether generic HRQoL was measured by parental proxy or self-report. Disease-specific complaints of patients with HD impaired physical HRQoL, but not psychosocial and social HRQoL.
CONCLUSION
In this systematic review and meta-analysis, no evidence was found for impaired generic HRQoL in patients with HD compared to healthy controls, neither for moderating effects of sex, parental proxy or self-report.
Topics: Adolescent; Adult; Child; Child, Preschool; Constipation; Fecal Incontinence; Hirschsprung Disease; Humans; Infant; Infant, Newborn; Quality of Life; Self Report; Young Adult
PubMed: 35645538
DOI: 10.3748/wjg.v28.i13.1362