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Cureus Apr 2024infection (CDI) is a clinical and laboratory diagnosis. Populations at higher risk of developing disease require a high clinical index of suspicion for laboratory... (Review)
Review
INTRODUCTION
infection (CDI) is a clinical and laboratory diagnosis. Populations at higher risk of developing disease require a high clinical index of suspicion for laboratory testing to avoid incorrect assumptions of colonization. Common risk factors include recent antibiotic use, elderly (>65 years old), and immunocompromised patients. ssays should be ordered in an algorithm approach to diagnose an infection rather than colonization. Screening tests are widely available in hospital systems, but novel molecular testing may aid in diagnosis in patients with inconclusive or discordant antigen and toxin test results. Methods: Data was extracted from PubMed, Scopus, and Cumulative Index of Nursing and Allied Health Literature (CINAHL) databases based on the keywords "", "toxin assay", and "toxic megacolon". The data extracted is based on the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) 2020 guidelines. A total of 27 reports were included in this systematic review.
RESULTS
Testing patients with a significant gastrointestinal surgical history, hypogammaglobulinemia, inflammatory bowel disease, intensive care unit, and immunocompromised patients for CDI is highly recommended. Diarrhea in these subsets of patients requires correlation of clinical context and an understanding of assay results to avoid over- and under-treating.
CONCLUSION
CDI should be considered in all patients with traditional risk factors. Heightened clinical suspicion of CDI is required in patients with hypogammaglobulinemia, transplant recipients, patients with gastrointestinal surgical history, and inflammatory bowel disease. Testing should be limited to patients with clinical manifestations of CDI to ensure a high pretest probability for test interpretation. Healthcare workers should adhere to testing algorithms to optimize yield in the appropriate clinical context. Diagnostic assays should follow a sequential, stepwise approach to categorize the toxin expression status of the bacteria accurately.
PubMed: 38800338
DOI: 10.7759/cureus.59016 -
Pediatric Surgery International Jan 2020Hirschsprung's disease (HSCR) and anorectal malformation (ARM) are often associated with other congenital malformations, but the association of each other is rare. Some...
BACKGROUND/PURPOSE
Hirschsprung's disease (HSCR) and anorectal malformation (ARM) are often associated with other congenital malformations, but the association of each other is rare. Some studies have reported the incidence of HSCR associated with ARM ranging from 2.0 to 3.4%. The purpose of this study was to update the current epidemiological and therapeutic features of this rare congenital association.
METHODS
A systematic literature search for relevant articles was performed in four databases using a combination of the following terms "association of Hirschsprung's disease and anorectal malformation", "aganglionosis and anorectal malformation" and "congenital megacolon and anorectal malformation" for studies published between 1952 and 2019. Reference lists were screened for additional cases.
RESULTS
Forty-three studies met the defined inclusion criteria, reporting a total of 126 patients who were diagnosed with HSCR with ARM. Thirty articles reported 42 single case reports of this association. Twelve articles reported 66 cases of HSCR in case series of 3309 ARM patients, resulting in an incidence of 2% of this association. Associated syndrome was found in 25 cases (20%): Currarino syndrome in 11, Down syndrome in 8, Cat eye syndrome in 4 and Pallister-Hall syndrome in 2 patients. Extent of aganglionosis was reported in 62 cases: short or rectosigmoid aganglionosis was reported in 44, long segment aganglionosis in 8, total colonic aganglionosis in 9 and total intestinal aganglionosis in 1 case.
CONCLUSION
Although the association of ARM and HSCR is rare, the incidence of HSCR among ARM cases seems to be higher than in the general pediatric population. There was a high incidence of coexistence of ARM and HSCR with severe associated syndromes.
Topics: Anorectal Malformations; Child; Hirschsprung Disease; Humans
PubMed: 31552492
DOI: 10.1007/s00383-019-04580-4 -
Clinical Toxicology (Philadelphia, Pa.) Jun 2022Enemas containing phosphate are widely prescribed and may cause important adverse effects. A systemic review published in 2007 reported the literature on the adverse...
INTRODUCTION
Enemas containing phosphate are widely prescribed and may cause important adverse effects. A systemic review published in 2007 reported the literature on the adverse effects of phosphate enemas from January 1957 to March 2007 and identified 12 deaths. These were thought due to electrolyte disturbances, heart failure and kidney injury. These data raised concerns about the use of phosphate enemas in routine practice. Newer osmotic-based enema alternatives are now available that do not contain absorbable ions. We sought to review the literature since this review and evaluate the latest data on the toxicity of phosphate-containing enemas. To gain a fuller picture we included case series and larger studies as well as case reports.
OBJECTIVES
To review the toxicity of phosphate enemas, particularly with respect to acute metabolic consequences and their associated clinical features. To identify risk factors for metabolic toxicity and consider whether phosphate enemas should be relatively contra-indicated in specific patient groups.
METHODS
A systematic literature review was conducted in PubMed, Google Scholar, and Cochrane Reviews (2005-2021) using the search terms 'phosphate enema or sodium phosphate enema' or 'phosphate-based enema' or (phosphate AND enema) or (Fleet AND enema) or 'sodium phosphate laxatives' or 'sodium phosphate catharsis' or 'sodium phosphate cathartic'. Relevant papers were read, and data were extracted.
RESULTS
The searches identified 489 papers of which 25 were relevant: seven papers were case reports or small case series of metabolic abnormalities from the use of phosphate enemas in nine children, six were case reports on 16 adults. Nine papers were large case series or clinical studies that included data on systemic metabolic effects, of varying size from 24 healthy volunteers to a cohort of 70,499 patients. Case reports identified seven adult deaths but none in children. Children most often presented with decreased consciousness (6/9), and tetany (4/9). In adults overall only five cases had clinical features reported, hypotension was seen in four and QT prolongation in two. Treatment was generally symptomatic, with intravenous fluid and calcium salts for electrolyte changes and hypocalcaemia, and vasopressors for severe hypotension. Haemodialysis was used in three children and peritoneal dialysis in one, all of whom survived. In adults, haemodialysis did not prevent death in two of four cases in whom it was used. Common factors underlying toxicity were inappropriately high phosphate dose, or enema retention, both resulting in greater absorption of phosphate. Associated pre-disposing conditions included Hirschsprung disease in children and co-morbidity and renal impairment (2/5) in older adults. Absolute reported changes in serum phosphate or calcium were not accurate indicators of outcome. Larger case series and clinical trials confirm an acute effect of phosphate enemas on serum phosphate, which was related to both dose and retention time. These effects were not seen with non-phosphate preparations. In these cases series, adverse events were rarely reported.
CONCLUSION
Phosphate enemas are potentially toxic, particularly in young children with Hirschsprung disease and in the elderly with co-morbidity. Raised awareness of the risk of phosphate enemas is still required. Other less toxic enema preparations are available and should be considered in patients at extremes of age. If phosphate enemas are the only clinical option careful monitoring of biochemical sequelae should be undertaken.
Topics: Aged; Calcium; Child; Child, Preschool; Enema; Hirschsprung Disease; Humans; Hypotension; Laxatives; Phosphates
PubMed: 35510830
DOI: 10.1080/15563650.2022.2054424 -
Journal of Pediatric Surgery Sep 2021Long-Segment Hirschsprung Disease (LSHD) differs clinically from short-segment disease. This review article critically appraises current literature on the definition,... (Review)
Review
OBJECTIVE
Long-Segment Hirschsprung Disease (LSHD) differs clinically from short-segment disease. This review article critically appraises current literature on the definition, management, outcomes, and novel therapies for patients with LSHD.
METHODS
Four questions regarding the definition, management, and outcomes of patients with LSHD were generated. English-language articles published between 1990 and 2018 were compiled by searching PubMed, Scopus, Cochrane Central Register of Controlled Trials, Web of Science, and Google Scholar. A qualitative synthesis was performed.
RESULTS
66 manuscripts were included in this systematic review. Standardized nomenclature and preoperative evaluation for LSHD are recommended. Insufficient evidence exists to recommend a single method for the surgical repair of LSHD. Patients with LSHD may have increased long-term gastrointestinal symptoms, including Hirschsprung-associated enterocolitis (HAEC), but have a quality of life similar to matched controls. There are few surgical technical innovations focused on this disorder.
CONCLUSIONS
A standardized definition of LSHD is recommended that emphasizes the precise anatomic location of aganglionosis. Prospective studies comparing operative options and long-term outcomes are needed. Translational approaches, such as stem cell therapy, may be promising in the future for the treatment of long-segment Hirschsprung disease.
Topics: Enterocolitis; Evidence-Based Practice; Hirschsprung Disease; Humans; Prospective Studies; Quality of Life
PubMed: 33993978
DOI: 10.1016/j.jpedsurg.2021.03.046 -
Journal of Pediatric Surgery Mar 2022Little is known about psychosocial and behavioral factors that impact the quality of life of patient's with anorectal malformations (ARM) and Hirschsprung disease... (Review)
Review
INTRODUCTION
Little is known about psychosocial and behavioral factors that impact the quality of life of patient's with anorectal malformations (ARM) and Hirschsprung disease (HSCR). We aimed to highlight the psychosocial, emotional, and behavioral themes that affect these patients.
METHODS
A qualitative literature review of articles published between 1980 and 2019 was performed. Articles that reported quality of life (QoL) measures not directly related to bowel function and incorporated data on patients aged 0-21 years old were included. Data were separated based on distinct developmental time points.
RESULTS
In the neonatal period, parents relayed uncertainty about the future and feeling overwhelmed by lack of social support. Difficulties with anxiety, peer rejection, and behavioral problems were noted in primary grades, while adolescents experienced low self-confidence, poor body image, and depression. Young adults expressed hesitancy to engage in romantic relationships or sexual activity. Lack of long-term follow-up, an incomplete transition to adult healthcare, and lack of psychology services leave young adults without guidance to manage a chronic condition.
CONCLUSION
Multiple psychosocial stressors are present in the lives of ARM and HSCR patients. Provision of developmentally matched medical, psychological, and community-based supports for ARM and HSCR patients and their families can lead to improved QoL.
Topics: Adolescent; Adult; Anorectal Malformations; Child; Child, Preschool; Hirschsprung Disease; Humans; Infant; Infant, Newborn; Parents; Quality of Life; Social Support; Young Adult
PubMed: 34127258
DOI: 10.1016/j.jpedsurg.2021.05.004 -
BMC Gastroenterology Mar 2020Advances in surgical techniques and perioperative care have improved the short- and mid-term postoperative outcomes of patients with Hirschsprung disease (HD). However,... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Advances in surgical techniques and perioperative care have improved the short- and mid-term postoperative outcomes of patients with Hirschsprung disease (HD). However, the long-term outcomes of these patients (older than 10 years) have not been fully investigated. The aim of this systematic review is to clarify the prevalence of long-term outcomes and the quality of life of these patients.
METHODS
PubMed, AMED, Cochrane Library, CINAHL and PsycINFO databases were searched from inception to October 2018, following the Meta-analysis of Observational Studies in Epidemiology (MOOSE) guideline. Original studies reporting the outcomes of patients older than ten years with HD were selected and reviewed. The overall prevalence of fecal incontinence, constipation, bowel function score, bladder dysfunction symptoms, and patients' quality of life were extracted from the included studies and pooled through the random-effects meta-analysis model. The heterogeneity and variation in the pooled estimations were evaluated by Cochrane's Q test and the I test. The sensitivity analysis was conducted by the sequential omission of individual studies. Publication bias was evaluated by Egger's linear regression test. The whole procedure was conducted with Stata (version 14).
RESULTS
In total, 3406 articles were identified from the literature search, among which twelve studies, including 625 patients, were included for analysis. The pooled prevalences of fecal incontinence, constipation, and bladder dysfunction symptoms and good to excellent bowel function scores were 0.20 (95% CI 0.13-0.28), 0.14 (95% CI 0.06-0.25), 0.07 (95% CI 0.04-0.12), and 0.95 (95% CI: 0.91-0.97), respectively; the pooled mean score of gastrointestinal-related quality of life was 118 (95% CI: 112.56-123.44).
CONCLUSIONS
HD patients older than ten years old have an overall high prevalence of fecal incontinence and a low quality of life. Targeted and evidence-based follow-up procedures and transitional care are essential to meet these patients' long-term care needs. Prospective and multicenter research that focuses on the attributes and predictors of the long-term prognosis of patients with HD are necessary.
Topics: Adolescent; Adult; Constipation; Fecal Incontinence; Follow-Up Studies; Hirschsprung Disease; Humans; Intestines; Middle Aged; Quality of Life; Treatment Outcome; Urinary Bladder; Young Adult
PubMed: 32164539
DOI: 10.1186/s12876-020-01208-z -
Journal of Crohn's & Colitis May 2020Gastrointestinal ultrasound is useful in the assessment of patients with Crohn's disease, but its application in ulcerative colitis [UC] is less well established. Here...
BACKGROUND AND AIMS
Gastrointestinal ultrasound is useful in the assessment of patients with Crohn's disease, but its application in ulcerative colitis [UC] is less well established. Here we systematically review the role of gastrointestinal ultrasound in patients with UC.
METHODS
Searches of the PUBMED and EMBASE databases were performed with the following search strategy: [ultrasound OR sonography] AND [intestinal OR bowel] AND [ulcerative colitis OR inflammatory bowel disease]. The final search was performed in August 2019.
RESULTS
Of 6769 studies identified in the search with a further two studies found from other sources, 50 studies met the inclusion criteria. Increased bowel wall thickness and detection of increased blood flow by colour Doppler were the most often applied criteria for defining disease activity and distribution. When compared with other reference investigations, gastrointestinal ultrasound accurately determined disease extent, severity and response to medical therapy. While further information can be obtained from haemodynamic measurements of the abdominal vessels and contrast-enhanced ultrasound, their clinical value was uncertain. Likewise, hydrocolonic sonography has few advantages over standard gastrointestinal ultrasound examination. Of several scoring systems proposed, there is disparity between the measures and a general lack of validation. There has been limited application of gastrointestinal ultrasound in acute severe ulcerative colitis with toxic megacolon, and, while performing well in children, normal limits differ from those in adults.
CONCLUSION
Current evidence indicates that gastrointestinal ultrasound has utility in the non-invasive assessment of patients with UC. Continued advances in technology with better image resolution, validation of scoring systems and application at the point of care by gastroenterologists are likely to contribute to increased use of gastrointestinal ultrasound in routine clinical practice.
Topics: Colitis, Ulcerative; Humans; Patient Care Management; Procedures and Techniques Utilization; Ultrasonography
PubMed: 31562739
DOI: 10.1093/ecco-jcc/jjz163 -
Pediatric Surgery International Jan 2023Hesitations concerning the long-term results of transanal endorectal pull-through (TEPT) due to prolonged anal stretching and resultant stricture and continence problems... (Meta-Analysis)
Meta-Analysis Review
AIM
Hesitations concerning the long-term results of transanal endorectal pull-through (TEPT) due to prolonged anal stretching and resultant stricture and continence problems has been started to be questioned. This meta-analysis intended to compare long-term results between TEPT and transabdominal (TAB) pull-through techniques in the surgical management of Hirschsprung's disease.
METHODS
All publications between the years 1998-2021 in the PubMed, Medline, Google Scholar, Cochrane databases were reviewed. Retrospective and prospective comparative studies for TEPT, TAB as well as Laparoscopic-assisted TEPT (LTEPT) were included. Data included age at operation, postoperative constipation, enterocolitis, incontinence, stricture, and soiling rates.
RESULTS
Eighteen publications met the inclusion criteria for TAB and TEPT, and six for TEPT and LTEPT. Patients who underwent TEPT had significantly younger operation age than patients with TAB (SMD - 1.02, 95%Cl - 1.85 to - 0.18, p: 0.0168). Postoperative constipation (OR 0.39, 95% Cl 0.25-0.61 p < 0.0001) and enterocolitis (OR 0.65, 95% Cl 0.46-0.90, p: 0.0108) rates were significantly lower in TEPT groups. Postoperative incontinence (OR 1.06, 95% Cl 0.56-2.01, p: 0.8468), stricture (OR 1.97, 95% Cl 0.81-4.80, p: 0.1352) and soiling rates were similar between the two groups. Furthermore, when TEPT and LTEPT results were compared, incidence of incontinence (OR 7.01, 95% Cl 0.75-65.33, p: 0.0871), constipation (OR 1.95, 95% Cl 0.70-5.37, p: 0.199), enterocolitis (OR 3.16, 95% Cl 0.34-29.55 p: 0.3137), stricture (OR 1.33, 95% Cl 0.29-6.15, p: 0.7188) and soiling (OR 1.57, 95% Cl 0.57-4.31, p: 0.3778) were similar for both techniques.
DISCUSSION
TEPT is superior to TAB in terms of constipation and enterocolitis. Contrary to concerns, postoperative incontinence rates are not statistically different. However, further publications about long-term LTEPT results are necessary for more reliable conclusions.
Topics: Humans; Infant; Hirschsprung Disease; Digestive System Surgical Procedures; Treatment Outcome; Retrospective Studies; Prospective Studies; Constriction, Pathologic; Constipation; Enterocolitis; Postoperative Complications
PubMed: 36692536
DOI: 10.1007/s00383-023-05378-1 -
Journal of Pediatric Surgery Jun 2024Despite surgical advances for complex congenital colorectal conditions, such as anorectal malformation (ARM) and Hirschsprung disease (HD), many adolescents require... (Review)
Review
BACKGROUND
Despite surgical advances for complex congenital colorectal conditions, such as anorectal malformation (ARM) and Hirschsprung disease (HD), many adolescents require transfer from specialist pediatric to adult providers for ongoing care.
METHODOLOGY
A systematic review of PubMed, MEDLINE and Embase was conducted to identify what is known about the transitional care of patients with ARM and HD (PROSPERO # CRD42022281558). The Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) framework guided our reporting of studies that focused on the transition care of 10-30-year-olds with ARM and HD.
RESULTS
Eight studies were identified that included patient and parent (n = 188), and/or clinician perspectives (n = 334). Patients and clinicians agreed that transitional care should commence early in adolescence to support transfer to adult care when a suitable level of maturation is reached. There was little evidence from patients that transfer happened in a timely or coordinated manner. Patients felt that clinicians did not always understand the significance of transfer to adult services. No models of transition care were identified. Surgeons ranked ARM and HD as the most common conditions to experience delayed transfer to adult care. Beyond pediatric surgeons, patients also highlighted the importance of general practitioners, transitional care coordinators and peer support groups for successful transition.
CONCLUSIONS
There is little research focused on transitional care for patients with ARM and HD. Given evidence of delayed transfer and poor experiences, the development of models of transitional care appears essential.
Topics: Humans; Transition to Adult Care; Adolescent; Anorectal Malformations; Adult; Hirschsprung Disease; Child; Young Adult
PubMed: 38493027
DOI: 10.1016/j.jpedsurg.2024.02.012 -
Journal of Pediatric Surgery Jun 2024The literature on transitional care in anorectal malformation (ARM) and Hirschsprung's disease (HD) is diverse and heterogeneous. There is a lack of standards and...
BACKGROUND
The literature on transitional care in anorectal malformation (ARM) and Hirschsprung's disease (HD) is diverse and heterogeneous. There is a lack of standards and guidelines specific to transitional care in these conditions. We aim to establish and systematically categorize challenges and solutions related to colorectal transition care.
METHODS
Systematic review of qualitative studies from MEDLINE, EMBASE, PubMed and Scopus databases (2008-2022) was conducted to identify the challenges and solutions of healthcare transition specific to ARM and HD. Thematic analyses are reported with reference to patient, healthcare provider and healthcare system.
RESULTS
Sixteen studies from 234 unique articles were included. Fourteen themes related to challenges and solutions, each, are identified. Most challenges identified are patient related. The key challenges pertain to: (1) patient's lack of understanding of their disorder, resulting in over-reliance on the pediatric surgical team and reluctance towards transitioning to adult services; (2) a lack of education and awareness among adult colorectal surgeons in caring for pediatric colorectal conditions and inadequate communication between pediatric and adult teams; and (3) a lack of structured transition program and joint-clinic to meet the needs of the transitioning patients. The key solutions are: (1) fostering young adult patient's autonomy and independence; (2) conducting joint pediatric-adult transition clinics; and (3) ensuring a structured and coordinated transition program is available using a standardized guideline.
CONCLUSION
A comprehensive framework related to barriers and solutions for pediatric colorectal transition is established to help benchmark care quality of transitional care services.
LEVEL OF EVIDENCE
IV.
TYPE OF STUDY
Systematic review without meta-analysis.
Topics: Humans; Hirschsprung Disease; Anorectal Malformations; Transition to Adult Care
PubMed: 37996349
DOI: 10.1016/j.jpedsurg.2023.10.066