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Stem Cell Research Sep 2023Visceral myopathies are debilitating conditions characterized by dysfunction of smooth muscle in visceral organs (bowel, bladder, and uterus). Individuals affected by...
Visceral myopathies are debilitating conditions characterized by dysfunction of smooth muscle in visceral organs (bowel, bladder, and uterus). Individuals affected by visceral myopathy experience feeding difficulties, growth failure, life-threatening abdominal distension, and may depend on intravenous nutrition for survival. Unfortunately, our limited understanding of the pathophysiology of visceral myopathies means that current therapies remain supportive, with no mechanism-based treatments. We developed a patient-derived iPSC line with a c.769C > T p.R257C/+ mutation, the most common genetic cause of visceral myopathy. This cell line will facilitate studies of how the ACTG2 R257C heterozygous variant affects smooth muscle development and function.
Topics: Female; Humans; Induced Pluripotent Stem Cells; Actins; Intestinal Pseudo-Obstruction; Intestines; Mutation
PubMed: 37572398
DOI: 10.1016/j.scr.2023.103176 -
Archives of Iranian Medicine Dec 2022
Topics: Humans; Muscular Dystrophy, Oculopharyngeal; Intestinal Pseudo-Obstruction; Ophthalmoplegia; Brain Diseases
PubMed: 37543914
DOI: 10.34172/aim.2022.132 -
The American Surgeon Nov 2023Eosinophilic myenteric ganglionitis (EMG) is a rare pathologic finding within the Auerbach myenteric plexus characterized by eosinophilic infiltration on light...
Eosinophilic myenteric ganglionitis (EMG) is a rare pathologic finding within the Auerbach myenteric plexus characterized by eosinophilic infiltration on light microscopy. The plexus's ultimate obliteration results in chronic intestinal pseudo-obstruction (CIPO). EMG is almost exclusively seen in the pediatric population. The diagnosis of EMG is made through full-thickness rectal biopsy and EMG is not detectable through routine screening measures such as imaging or colonoscopy. The current treatment modality for this disorder is not standardized, and has often been treated with systemic steroids given its eosinophilic involvement. This case presents a 73-year-old male with chronic constipation presenting with new obstipation in the setting of recent orthopedic intervention requiring outpatient opioids. Admission radiographs were consistent with sigmoid volvulus. Following endoscopic detorsion, exploratory laparotomy revealed diffuse colonic dilation and distal ischemia requiring a Hartmann's procedure. Surgical pathology revealed EMG, increasing the complexity of subsequent surgical decision-making after his urgent operation.
Topics: Male; Humans; Child; Aged; Intestinal Volvulus; Colon; Intestinal Pseudo-Obstruction; Myenteric Plexus; Colonoscopy; Sigmoid Diseases
PubMed: 37501639
DOI: 10.1177/00031348231191198 -
Surgical Endoscopy Oct 2023Percutaneous endoscopic cecostomy (PEC) is a viable treatment option for patients with persistent or recurrent acute colonic pseudo-obstruction (ACPO; Ogilvie's... (Review)
Review
INTRODUCTION
Percutaneous endoscopic cecostomy (PEC) is a viable treatment option for patients with persistent or recurrent acute colonic pseudo-obstruction (ACPO; Ogilvie's syndrome). It should be generally considered in patients that are refractory to pharmacologic and endoscopic decompression, especially those not amenable to surgical intervention due to an increased perioperative risk. Physicians are rather unfamiliar with this approach given the limited number of reports in the literature and paucity of guideline resources, although guidelines concerning ACPO and covering the role of endoscopy were recently published by three major expert societies, all within the last 2 years.
PATIENTS AND METHODS
We retrospectively identified three consecutive patients who underwent PEC placement at a Czech tertiary referral center between May 2018 and December 2021: all for recurrent ACPO. In addition, we summarized the current guidelines in order to present the latest knowledge related both to the procedure and management approach in patients with ACPO.
RESULTS
The placement of PEC was successful and resulted in clinical improvement in all cases without any adverse events.
CONCLUSION
The results of our experience are in line with previous reports and suggest that PEC may become a very useful tool in the armamentarium of modalities utilized to treat ACPO. Furthermore, the availability of guideline resources now offers comprehensive guidance for informed decision-making and the procedural aspects.
Topics: Humans; Cecostomy; Colonic Pseudo-Obstruction; Decompression, Surgical; Endoscopy, Gastrointestinal; Lumbar Vertebrae; Retrospective Studies
PubMed: 37500922
DOI: 10.1007/s00464-023-10281-w -
Boletin Medico Del Hospital Infantil de... 2023Bowel obstruction due to accidental ingestion of foreign objects occurs rarely in children because 80 to 90% of the objects can pass freely through the gastrointestinal...
BACKGROUND
Bowel obstruction due to accidental ingestion of foreign objects occurs rarely in children because 80 to 90% of the objects can pass freely through the gastrointestinal tract.
CASE REPORT
We report a case of a 14-month-old infant who presented bowel obstruction caused by the ingestion of hydrogel beads (sodium polyacrylate). Hydrogel beads are used as sensory and didactic toys that can increase their initial size 200 to 400 times by liquid absorption. An abdominal X-ray was perfomed in anteroposterior supine projection, where a round filling defect at the loop of the right flank was detected; this came to our attention because hydrogel beads are usually radiolucent. The diagnosis was established by abdominal ultrasound where free intraperitoneal fluid was reported with data of small bowel pseudo-obstruction by foreign objects. Conservative treatment was prescribed, finding persistence of increased abdominal perimeter, so an enterotomy was performed for their removal; finding impacted hydrogel beads 30 centimeters from the ileocecal valve.
CONCLUSIONS
Hydrogel beads are dangerous for the pediatric population. The evolution of the patient was favorable thanks to the knowledge of the foreign objects ingested. The expectant behavior that had to be executed, stands out because we had no knowledge as to the maximum size of the hydrogel in the gastrointestinal tract.
Topics: Infant; Humans; Child; Hydrogels; Intestine, Small; Intestinal Obstruction; Foreign Bodies
PubMed: 37490685
DOI: 10.24875/BMHIM.22000137 -
JPMA. the Journal of the Pakistan... Jul 2023Duodenal ulcer perforation, a frequent surgical emergency, needs simple closure with indirect Graham's Omentopexy which is effective with excellent results in majority...
Duodenal ulcer perforation, a frequent surgical emergency, needs simple closure with indirect Graham's Omentopexy which is effective with excellent results in majority of cases despite patients' late presentation. The objective of the study was to determine the frequency of postoperative complications of perforated duodenal ulcer, conducted in the Surgery Department, Jinnah Postgraduate Medical Centre, Karachi, from March 20, 2018 to September 20, 2018. The study was a descriptive case series of 108 patients of both genders with perforated duodenal ulcer > 1 week old with ASA score I & II. Patients with trauma and comorbidities were excluded. The patients underwent laparotomy and peritoneal toilet, and after noting the site of perforation indirect Graham's Omentopexy was performed. Complications like duodenal fistula, peritonitis, and paralytic ileus, and patient's death within 10 days of surgery were noted. Age ranged from 18 to 50 years with mean age of 35.027±5.13 years, mean weight 71.120±12.77 kg, mean height 1.541 ±0.09 metres, mean BMI 29.975±4.99 kg/m2, and the mean duration of complaint was 4.194±1.30 weeks. Male predominance in 75 (69.4%) patients. Duodenal fistula was seen in 10 (9.3%) patients, peritonitis 12 (11.1%), paralytic ileus 14 (13%) and mortality was in 11 (10.2%) patients.
Topics: Humans; Male; Female; Adult; Infant; Duodenal Ulcer; Risk Factors; Peptic Ulcer Perforation; Peritonitis; Fistula
PubMed: 37469068
DOI: 10.47391/JPMA.4768 -
Neuroscience Letters Aug 2023Enteric glial cells are emerging as critical players in the regulation of intestinal motility, secretion, epithelial barrier function, and gut homeostasis in health and...
Enteric glial cells are emerging as critical players in the regulation of intestinal motility, secretion, epithelial barrier function, and gut homeostasis in health and disease. Enteric glia react to intestinal inflammation by converting to a 'reactive glial phenotype' and enteric gliosis, contributing to neuroinflammation, enteric neuropathy, bowel motor dysfunction and dysmotility, diarrhea or constipation, 'leaky gut', and visceral pain. The focus of the minireview is on the impact of inflammation on enteric glia reactivity in response to diverse insults such as intestinal surgery, ischemia, infections (C. difficile infection, HIV-Tat-induced diarrhea, endotoxemia and paralytic ileus), GI diseases (inflammatory bowel diseases, diverticular disease, necrotizing enterocolitis, colorectal cancer) and functional GI disorders (postoperative ileus, chronic intestinal pseudo-obstruction, constipation, irritable bowel syndrome). Significant progress has been made in recent years on molecular pathogenic mechanisms of glial reactivity and enteric gliosis, resulting in enteric neuropathy, disruption of motility, diarrhea, visceral hypersensitivity and abdominal pain. There is a growing number of glial molecular targets with therapeutic implications that includes receptors for interleukin-1 (IL-1R), purines (P2X2R, A2BR), PPARα, lysophosphatidic acid (LPAR1), Toll-like receptor 4 (TLR4R), estrogen-β receptor (ERβ) adrenergic α- (α-R) and endothelin B (ETBR), connexin-43 / Colony-stimulating factor 1 signaling (Cx43/CSF1) and the S100β/RAGE signaling pathway. These exciting new developments are the subject of the minireview. Some of the findings in pre-clinical models may be translatable to humans, raising the possibility of designing future clinical trials to test therapeutic application(s). Overall, research on enteric glia has resulted in significant advances in our understanding of GI pathophysiology.
Topics: Humans; Infant, Newborn; Gliosis; Clostridioides difficile; Enteric Nervous System; Gastrointestinal Diseases; Neuroglia; Inflammation; Abdominal Pain; Gastrointestinal Motility; Diarrhea; Constipation; Intestinal Pseudo-Obstruction
PubMed: 37451357
DOI: 10.1016/j.neulet.2023.137395 -
Revista Espanola de Enfermedades... Jul 2023Immune checkpoint inhibition therapy using targeted monoclonal antibodies is a relatively new therapeutic approach for patients with several cancer types including...
Immune checkpoint inhibition therapy using targeted monoclonal antibodies is a relatively new therapeutic approach for patients with several cancer types including non-small cell lung cancer1. Targeted monoclonal antibodies based drugs can activate anti-tumor immunity by blocking immune checkpoint receptors (CTLA-4, PD-1 receptor and its ligand PD-L1), in order to restore T-cell effector function2,3. However, the use of immune checkpoint inhibitors can lead to a unique side effect profile termed as immune-related adverse events (irAEs). Loss of T-cell inhibition results in an enhanced immune response driven by T-cell activation and is capable of inducing an autoimmune-related inflammation in normal tissue as a consequence of impaired self tolerance4. These irAEs can potentially involve every organ system including gastrointestinal, dermatologic, hepatic, and endocrine toxicities. For example, Fernandez-Gordon Sanchez et al reported a patient with immune-mediated colitis and nonimmune-mediated cholestasic injury induced by pembrolizumab that was successfully treated with Ustekinumab5. We report a steroid-refractory case of lung adenocarcinoma patient with an unusual irAE (intestinal pseudo-obstruction) during the treatment with immune checkpoint inhibitors that could be successfully managed by the administration of infliximab.
PubMed: 37449519
DOI: 10.17235/reed.2023.9796/2023 -
Journal of Medical Case Reports Jul 2023Sclerosing encapsulating peritonitis is a rare condition with a typical macroscopic appearance, with fibrocollagenous membrane enclosing loops of the small intestine,...
BACKGROUND
Sclerosing encapsulating peritonitis is a rare condition with a typical macroscopic appearance, with fibrocollagenous membrane enclosing loops of the small intestine, causing intestinal obstruction. Unexplained recurrent abdominal pain, obstruction, and a large array of other possible clinical signs and symptoms make sclerosing encapsulating peritonitis a diagnostic challenge.
CASE PRESENTATION
A 48-year-old man of Persian ethnicity was admitted multiple times to the emergency surgery department due to recurrent sudden abdominal pain and chronic obstruction without significant findings in medical history or clinical evaluation. Computed tomography was positive for proximal jejunal dilatation and duodenojejunal flexure stenosis due to internal mesenteric hernia. Exploratory laparoscopy, followed by laparotomy, confirmed thick membrane-like fibrous tissue with complete small intestinal loop envelopment. Extensive membrane excision and adhesiolysis was performed, but no mesenteric herniation was found. Early postoperative paralytic ileus with introduction of low-dose steroid therapy, based on histopathological and immunological results, confirming type III sclerosing encapsulating peritonitis, was completely resolved.
CONCLUSION
Sclerosing encapsulating peritonitis is a rare and difficult-to-diagnose condition, further divided into primary and secondary sclerosing encapsulating peritonitis, on the basis of underlying etiology, dictating treatment modality and prognosis. Intraoperative diagnosis and surgical treatment are mandatory, besides a wide variety of abdominal computed tomography scans, inconclusive results, and clinical presentations. There are so far no known specific markers for the diagnosis of sclerosing encapsulating peritonitis.
Topics: Humans; Middle Aged; Peritonitis; Intestinal Obstruction; Prognosis; Laparotomy; Abdominal Pain
PubMed: 37443141
DOI: 10.1186/s13256-023-04020-x -
Annals of Medicine and Surgery (2012) Jul 2023Appendectomy is a surgical procedure performed on a patient with acute appendicitis and is often performed by surgery and performed in an emergency. The authors have...
UNLABELLED
Appendectomy is a surgical procedure performed on a patient with acute appendicitis and is often performed by surgery and performed in an emergency. The authors have carried out this study, which aims to describe the surgical characteristics of appendectomies.
METHODS
This retrospective, descriptive, and documentary cross-sectional study was conducted from October 2021 to October 2022. Within this time ~591 acute abdominal surgical procedures were performed, including 196 appendectomies performed in the general surgery department.
RESULTS
This study focused on 196 appendectomies performed out of a total of 591 surgeries performed, with an incidence of 34.2%. 51 cases (26%) for the age group between 15 and 20 years, 129 cases (65.8%) of women participated in the appendectomy. 133 (67.8%) acute appendicitis, 48 (24.5%) appendicular abscess and 15 (7.7%) appendicular peritonitis were indications for appendectomies. For the American Society of Anesthesiologists (ASA) score: for the ASA I, 112 (57.1%) of the patients had no conditions other than those requiring surgery (appendectomy). For the Altemeier classification, the authors recorded 133 (67.9%) own surgeries. 56 (28.6%) surgical site infections followed by 39 (19.8%) inflammation (swelling and redness), 37 (18.8%) pain, 24 (12.4%) purulent peritonitis, 21 (10, 7%) postoperative hemorrhage and 19 (9.7%) paralytic ileus and 157 (80.1%) patients benefited from medical treatment.
CONCLUSION
The low incidence of complications related to laparotomy appendectomy has been reduced to a minimum thanks to the respect of sanitary measures and the quality of the technique.
PubMed: 37427177
DOI: 10.1097/MS9.0000000000000966