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Pediatrics in Review Dec 2021
Topics: Hirschsprung Disease; Humans
PubMed: 36017952
DOI: 10.1542/pir.2020-004912 -
Medizinische Klinik, Intensivmedizin... Oct 2015Toxic megacolon constitutes a feared, life-threatening complication of severe intestinal inflammation and is a challenge for interdisciplinary medical care. (Review)
Review
BACKGROUND
Toxic megacolon constitutes a feared, life-threatening complication of severe intestinal inflammation and is a challenge for interdisciplinary medical care.
OBJECTIVES
Specific aspects of conservative treatment based on current scientific evidence derived from guidelines, qualified reviews, and scientific studies are presented, which provide a rational approach and maximize therapeutic success.
MATERIALS AND METHODS
This work is based on a selective literature review and the authors' experience of many years in gastroenterology and intensive care.
RESULTS
Toxic megacolon requires a rapid interdisciplinary assessment. Depending on the underlying etiology, an individual treatment concept needs to be developed. If an infectious or inflammatory cause is probable, a conservative approach can reduce perioperative morbidity and mortality. A step-wise approach with controlled reevaluations of the response to therapy after 72 h and 7 days avoids uncontrolled delay of surgical options further ensuring patient safety.
CONCLUSION
Despite a decreasing incidence of toxic megacolon, it remains an interdisciplinary therapeutic challenge.
Topics: Colitis, Ulcerative; Critical Care; Critical Illness; Cytomegalovirus Infections; Enterocolitis, Pseudomembranous; Evidence-Based Medicine; Guideline Adherence; Humans; Interdisciplinary Communication; Intersectoral Collaboration; Megacolon, Toxic; Prognosis
PubMed: 26369529
DOI: 10.1007/s00063-015-0076-1 -
Best Practice & Research. Clinical... 2022Hirschsprung disease (HSCR) and Paediatric Intestinal Pseudo-obstruction (PIPO) comprise two of the most recognized and severe disorders of gastrointestinal (GI)... (Review)
Review
Hirschsprung disease (HSCR) and Paediatric Intestinal Pseudo-obstruction (PIPO) comprise two of the most recognized and severe disorders of gastrointestinal (GI) motility. HSCR is a developmental disorder of the enteric nervous system invariably affecting the large intestine, whereas the majority of PIPO conditions represent congenital disorders of one or more components of the neuromusculature and more diffusely affect the GI tract. Histopathology is deemed the gold standard for the diagnosis of HSCR and, arguably, of PIPO, but, other diagnostic modalities such as manometric and genetic studies have seen recent advances that may increase their utility. Especially for PIPO, management is multidisciplinary and best performed in specialist referral centres. Surgery remains the only viable treatment for HSCR and appears essential to optimize and sustain feeding and viability of intestinal function in PIPO patients. Novel therapies such as neural stem cell transplants show promise for the future.
Topics: Child; Enteric Nervous System; Gastrointestinal Motility; Hirschsprung Disease; Humans; Intestinal Pseudo-Obstruction
PubMed: 35331399
DOI: 10.1016/j.bpg.2021.101765 -
Seminars in Pediatric Surgery Dec 2022Bowel has its own elegant nervous system - the enteric nervous system (ENS) which is a complex network of neurons and glial clones. Derived from neural crest cells... (Review)
Review
Bowel has its own elegant nervous system - the enteric nervous system (ENS) which is a complex network of neurons and glial clones. Derived from neural crest cells (NCCs), this little brain controls muscle contraction, motility, and bowel activities in response to stimuli. Failure of developing enteric ganglia at the distal bowel results in intestinal obstruction and Hirschsprung disease (HSCR). This Review summarises the important embryological development of the ENS including proliferation, migration, and differentiation of NCCs. We address the signalling pathways which determine NCC cell fate and discuss how they are altered in the context of HSCR. Finally, we outline the anatomical defects and the mechanisms underlying gut motility in HSCR.
Topics: Humans; Hirschsprung Disease; Enteric Nervous System; Neural Crest
PubMed: 36417785
DOI: 10.1016/j.sempedsurg.2022.151227 -
Seminars in Pediatric Surgery Apr 2022Total Colonic Hirschsprung Disease (HD) can be challenging from a diagnostic and management standpoint and occurs in around 8% of cases of HD. Long term outcomes are... (Review)
Review
Total Colonic Hirschsprung Disease (HD) can be challenging from a diagnostic and management standpoint and occurs in around 8% of cases of HD. Long term outcomes are difficult to compare due to variation in length of aganglionosis, chosen surgical techniques, and terminology utilized in the literature. In this review we highlight some of the management controversies and clinical challenges and emphasize future areas of suggested collaboration and research.
Topics: Hirschsprung Disease; Humans
PubMed: 35690465
DOI: 10.1016/j.sempedsurg.2022.151165 -
Gastroenterology Nov 2022
Topics: Humans; Megacolon; Diverticulitis, Colonic
PubMed: 35667409
DOI: 10.1053/j.gastro.2022.06.004 -
Critical Care Nursing Clinics of North... Mar 2018Gastrointestinal (GI) motility problems are common complications in critical care patients. GI problems contribute to an increased risk of morbidity and mortality. Toxic... (Review)
Review
Gastrointestinal (GI) motility problems are common complications in critical care patients. GI problems contribute to an increased risk of morbidity and mortality. Toxic megacolon (TM) is a type of acquired megacolon categorized as a medical emergency and includes severe inflammation affecting all layers of the colon wall. The high incidence of GI complications in critically ill patients requires the critical care nurse to provide close monitoring of patients at risk and an acute awareness of the causation, signs and symptoms, and treatment of various GI motility disorders, including gastroparesis, ileus, and TM.
Topics: Critical Care; Critical Care Nursing; Critical Illness; Gastrointestinal Motility; Gastroparesis; Humans; Ileus; Megacolon, Toxic
PubMed: 29413206
DOI: 10.1016/j.cnc.2017.10.010 -
International Journal of Colorectal... Aug 2020Large bowel obstruction and megacolon formation secondary to complicated diverticulitis is rare.
PURPOSE
Large bowel obstruction and megacolon formation secondary to complicated diverticulitis is rare.
METHODS
We present a case of an 84-year-old woman surviving large bowel obstruction and mega-megacolon formation secondary to complicated diverticulitis, with an impressive presentation of abdominal distention.
RESULTS
The patient's symptoms, laboratory test results, and imaging were consistent with large bowel obstruction. The patient underwent urgent exploratory laparotomy. Upon entry in the abdomen, it was unexpected that the extreme colonic wall thickening had prevented perforation, indicating the longtime course of illness. The biopsy of the specimen from the site of the obstruction demonstrated an inflammatory obstructing mass.
CONCLUSION
This report aims to point out the atypical and in-extremes presentation of an otherwise common disease.
Topics: Aged, 80 and over; Diverticulitis; Female; Humans; Intestinal Obstruction; Laparotomy; Megacolon
PubMed: 32447480
DOI: 10.1007/s00384-020-03642-7 -
International Journal of Molecular... Jun 2019Hirschsprung disease (HSCR, OMIM 142623) is due to a failure of enteric precursor cells derived from neural crest (EPCs) to proliferate, migrate, survive or... (Review)
Review
Hirschsprung disease (HSCR, OMIM 142623) is due to a failure of enteric precursor cells derived from neural crest (EPCs) to proliferate, migrate, survive or differentiate during Enteric Nervous System (ENS) formation. This is a complex process which requires a strict regulation that results in an ENS specific gene expression pattern. Alterations at this level lead to the onset of neurocristopathies such as HSCR. Gene expression is regulated by different mechanisms, such as DNA modifications (at the epigenetic level), transcriptional mechanisms (transcription factors, silencers, enhancers and repressors), postranscriptional mechanisms (3'UTR and ncRNA) and regulation of translation. All these mechanisms are finally implicated in cell signaling to determine the migration, proliferation, differentiation and survival processes for correct ENS development. In this review, we have performed an overview on the role of epigenetic mechanisms at transcriptional and posttranscriptional levels on these cellular events in neural crest cells (NCCs), ENS development, as well as in HSCR.
Topics: Adenosine Triphosphate; Animals; Biomarkers; Chromatin Assembly and Disassembly; DNA Methylation; Epigenesis, Genetic; Gene Expression Regulation; Genetic Association Studies; Genetic Predisposition to Disease; Hirschsprung Disease; Histones; Humans; Polycomb-Group Proteins; RNA, Untranslated
PubMed: 31247956
DOI: 10.3390/ijms20133123 -
Journal of Gastrointestinal Surgery :... May 2023
Topics: Adult; Humans; Hirschsprung Disease
PubMed: 36717467
DOI: 10.1007/s11605-022-05560-1