"Intestinal pseudo-obstruction"

Anti-PD1-/PDL1-induced chronic intestinal pseudo-obstruction: three cases treated with vedolizumab after corticosteroid failure with mixed results. Immune checkpoint inhibitors (ICI), i.e., anti-PD1/PDL1 and anti-CTLA-4, have reshaped the prognosis of many cancers. Increased use of ICI has led to the onset of new adverse events. Neurological immune-related adverse events are rare, heterogenous , and potentially life-threatening. Chronic intestinal pseudo-obstruction (CIPO) is an immune-related autonomic plexus neuropathy that may be caused by infiltration of the myenteric plexus by CD8 + T cells. It is a rare and potentially fatal side effect that can be difficult to diagnose early because of initial nonspecific clinical presentation including vomiting, nausea, diarrhea, and constipation. Some rare

Efficacy and safety of rifaximin in patients with chronic intestinal pseudo-obstruction: a randomized, double-blind, placebo-controlled, phase II-a exploratory trial. Chronic intestinal pseudo-obstruction (CIPO) is a rare intractable disease with limited treatment options. Small intestinal bacterial overgrowth (SIBO) often co-occurs with several diseases, including CIPO. While rifaximin (RFX

Glutamine supplementation as a novel metabolic therapeutic strategy for LIG3-dependent chronic intestinal pseudo-obstruction. We recently identified a recessive syndrome due to LIG3 mutations in patients with chronic intestinal pseudo-obstruction, leukoencephalopathy and neurogenic bladder. LIG3 mutations affect mitochondrial DNA (mtDNA) maintenance, leading to defective energy production. We

Novel KIF26A variants associated with pediatric intestinal pseudo-obstruction (PIPO) and brain developmental defects. Pediatric intestinal pseudo-obstruction (PIPO) is a rare congenital disorder of the enteric nervous system with distal colon aganglionosis potentially leading to intestinal obstruction. Recently, biallelic variants in KIF26A, encoding a crucial motor protein for the migration

Incidence, diagnostics, therapeutic management and outcomes of paediatric intestinal pseudo-obstruction in the Netherlands: A 20-year retrospective cohort study. To describe incidence, clinical course, diagnostic and therapeutic management and long-term follow-up of paediatric intestinal pseudo-obstruction (PIPO) in the Netherlands between 2000 and 2020. Multicenter, national, retrospective

Small-bowel transit scintigraphy in children with paediatric intestinal pseudo-obstruction. evidence of small intestinal dysmotility is a key criterion for the diagnosis of paediatric intestinal pseudo-obstruction (PIPO). Small bowel scintigraphy (SBS) allows for objective measurement of small bowel transit (SBT), but limited data is available in children. We aimed to evaluate the utility of SBS

Acute intestinal pseudo-obstruction secondary to Sjogren's syndrome in pregnancy: a case report and literature review. Intestinal pseudo-obstruction (IPO) is a rare disease, and its clinical manifestations can resemble mechanical intestinal obstruction leading to unnecessary and potentially harmful surgery. Certain autoimmune diseases have been associated with IPO, however, cases secondary

"Clinical utility of ileal motility in children with defecation disorders and children with chronic intestinal pseudo-obstruction". Little is known about ileal motility patterns and their utility in children. Here, we present our experience with children undergoing ileal manometry (IM). A retrospective review of children with ileostomy comparing IM between two groups: A (chronic intestinal pseudo-obstruction [CIPO]) and B (feasibility of ileostomy closure in children with defecation disorders). We also compared the IM findings with those from antroduodenal manometry (ADM), and evaluated the joint effect of age, sex, and study indication group on IM results. A total of 27 children (median age 5.8 years old, range 0.5 to 16.74 years, 16 were female) were included (12 in group A and 15

Pediatric intestinal pseudo-obstruction: An international survey on diagnostic and management strategies in the European Reference Network for rare Inherited and Congenital Anomalies intestinal failure teams. Pediatric intestinal pseudo-obstruction (PIPO) management is based on nutritional, medical and surgical care while available evidence is scarce. The aim of this study was to outline

Paediatric Intestinal Pseudo obstruction Downloadedfrom https://journals.lww.com/jpgnby BhDMf5ePHKav1zEoum1tQfN4a+kJLhEZgbsIHo4XMi0hCywCX1AWnYQp/IlQrHD3wxNooCNzZviFlOANdCwt9BPIdrLH54MCUJj+Dx1UaCD/r6fk/cSIcA==on 05/29/2018Downloadedfrom https://journals.lww.com/jpgnby BhDMf5ePHKav1zEoum1tQfN4a+kJLhEZgbsIHo4XMi0hCywCX1AWnYQp/IlQrHD3wxNooCNzZviFlOANdCwt9BPIdrLH54MCUJj+Dx1UaCD/r6fk/cSIcA==on 05/29 /2018 Copyright © ESPGHAN and NASPGHAN. All rights reserved.Paediatric Intestinal Pseudo-obstruction: Evidenceand Consensus-based Recommendations Froman ESPGHAN-Led Expert GroupyNikhil Thapar,Efstratios Saliakellis,zMarc A. Benninga,Osvaldo Borrelli,§Joe Curry,jjChristophe Faure,ôRoberto De Giorgio,#Girish Gupte,Charles H. Knowles,yyAnnamaria Staiano,zzYvan Vandenplas, and§§Carlo Di

LPAR1 regulates enteric nervous system function through glial signaling and contributes to chronic intestinal pseudo-obstruction. Gastrointestinal motility disorders involve alterations to the structure and/or function of the enteric nervous system (ENS) but the causal mechanisms remain unresolved in most cases. Homeostasis and disease in the ENS are processes that are regulated by enteric glia with chronic intestinal pseudo-obstruction (CIPO), a severe motility disorder, showed reduced glial LPAR1 expression in the colon and ileum. These data suggest that enteric glial LPAR1 signaling regulates gastrointestinal motility through enteric glia and could contribute to severe motility disorders in humans such as CIPO.

Twenty Years of Gut Transplantation for Chronic Intestinal Pseudo-obstruction: Technical Innovation, Long-term Outcome, Quality of Life, and Disease Recurrence. To define long-term outcome, predictors of survival, and risk of disease recurrence after gut transplantation (GT) in patients with chronic intestinal pseudo-obstruction (CIPO). GT has been increasingly used to rescue patients with CIPO

Pediatric Intestinal Pseudo-Obstruction: Impact of Neonatal and Later Onset on Clinical and Nutritional Outcomes. The aim of the study was to evaluate long-term nutritional outcomes and clinical characteristics in a cohort of children with pediatric intestinal pseudo-obstruction (PIPO) at neonatal-onset (NO-PIPO) and at later-onset (LO-PIPO). All children fulfilling new PIPO criteria over a 30

Chronic intestinal pseudo-obstruction with pneumatosis cystoides intestinalis in a patient with systemic sclerosis: A case report. Chronic intestinal pseudo-obstruction (CIPO) and pneumatosis cystoides intestinalis (PCI) are rare abdominal diseases and the pathological mechanisms have not been fully elucidated. Systemic sclerosis (SSc), which is characterized by the progressive sclerotic changes

Lupus intestinal pseudo-obstruction and hydronephrosis: Case report. Intestinal pseudo-obstruction (IPO) is a rare and life-threatening complication of lupus. A patient with long-standing lupus developed recurrent abdominal pain and distension as well as nausea and emesis. Imaging showed dilated small bowel loops with air-fluid levels and bowel wall thickening. She also had bilateral hydronephrosis. She was given high-doses of intravenous steroids and cyclophosphamide. Her symptoms resolved within a week of starting immunosuppression. She was eventually transitioned to mycophenolate mofetil. She remained in remission and immunosuppression was successfully stopped after 1 year. Intestinal pseudo-obstruction is a rare complication of lupus that is often seen in association

The Use of Pyridostigmine in a Child With Chronic Intestinal Pseudo-Obstruction. Chronic intestinal pseudo-obstruction is a rare disorder that affects the motility of the gastrointestinal tract. It results in acute or subacute intestinal obstruction symptoms in the absence of mechanical lesion. It can lead to intestinal failure in children with significant strain on nutrition, growth , and development. There is no universally agreed protocol for management of chronic intestinal pseudo-obstruction in children, and there is wide variation in clinical practice.

Intestinal pseudo-obstruction as the initial manifestation of systemic lupus erythematosus. Small bowel obstruction is common in emergency departments. However, the exact cause of intestinal pseudo-obstruction (IPO) is often misdiagnosed. IPO is considered a severe manifestation of systemic lupus erythematosus (SLE). However, IPO is rare as the initial manifestation of SLE. This paper reports

The chronic intestinal pseudo-obstruction subtype has prognostic significance in patients with severe gastrointestinal dysmotility related intestinal failure. Severe gastrointestinal dysmotility (GID) is a significant cause of chronic intestinal failure (CIF) with unclear benefits of sub-classifying into Chronic Intestinal Pseudo-obstruction (CIPO) and non-CIPO sub-types. We compared outcomes

Causes and prognosis of chronic intestinal pseudo-obstruction in 48 subjects: A 10-year retrospective case series. The aim of the study was to evaluate the prognosis and survival of pediatric subjects with chronic intestinal pseudo-obstruction (CIPO) and investigate the independent risk factors affecting their prognosis.This was a retrospective case series of all pediatric subjects suffering from

Deletion of IP₃R1 by Pdgfrb-Cre in mice results in intestinal pseudo-obstruction and lethality. Inositol 1,4,5-trisphosphate receptors (IPRs) are a family of intracellular Ca release channels located on the membrane of endoplasmic reticulum, which have been shown to play critical roles in various cellular and physiological functions. However, their function in regulating