Gastrointestinal dysmotility complicating Behçet's syndrome: description of a newly recognised clinical phenotype.

OBJECTIVES: Gastrointestinal dysmotility may arise in a variety of auto-immune and auto-inflammatory diseases and hitherto has not been described in Behçet's syndrome (BS). METHODS: We present data on a cohort of seven patients under our care who presented with symptoms of and investigations compatible with an immune associated disorder of gastrointestinal motility, or enteric neuropathy. RESULTS: We describe the clinical features and investigation results. We undertook a trial of a novel treatment in the disease, apheresis, and noted a response not only to the enteric neuropathy but also to the systemic features of the disease, despite previous maximal immunosuppressive therapy in most cases. CONCLUSIONS: Gastrointestinal dysmotility may arise in BS and is effectively treated by apheresis. The mechanism by which this response is made immunologically requires to be elucidated in future studies.

The efficacy and results of medical treatment in postoperative ileus.

Background: Postoperative paralytic ileus refers to the disruption of the normal coordinated propulsive motor activity of the gastrointestinal system following surgery. Surgery causes inflammation in the muscle walls of organs with an intestinal lumen that, in turn, leads to a decrease in intestinal motility. Aim: The aim of this study was to investigate the efficacy of gastrografin, neostigmine, and their combined administration in patients diagnosed with paralytic ileus in the postoperative period. Patients and Methods: One-hundred twelve patients were included from January 2017 and November 2019. The retrospective study is involving prolonged postoperative ileus cases following colorectal surgery. The effect of gastrografin, neostigmine, and gastrografin neostigmine combination was compared retrospectively in the treatment of prolonged ileus after surgery. Results: The study covered 112 patients. Gastrografin was administered to 63 patients; neostigmine was administered to 29, while 20 patients received the combination of the two. Data pertaining to the comparison of the two groups revealed that patients in the gastrografin group were discharged earlier than those in the neostigmine group. Further, patients in the combined group had earlier gas and/or stool discharge and were also discharged from the hospital earlier than those in the neostigmine group. Conclusion: Gastrografin and combined use of gastrografin and neostigmine are effective and viable methods for postoperative ileus cases. Gastrografin can safely be used in patients with anastomoses.

Vitamin B12 deficiency presenting as intestinal pseudo-obstruction in short bowel syndrome: A case report.

Bowel obstruction and nutrient deficiencies are commonly encountered complications seen during follow-up of patients with short bowel syndrome. It is rare to see vitamin B12 deficiency among micronutrient deficits. The onset of B12 deficiency can be insidious, with autonomic features preceding other symptoms. In this study, we report a case in which vitamin B12 deficiency presented with intestinal dysmotility mimicking mechanical bowel obstruction.

Mitochondrial neurogastrointestinal encephalomyopathy in a Pakistani female: a case report.

BACKGROUND: Mitochondrial neurogastrointestinal encephalopathy is a rare multisystem autosomal recessive disease caused by mutations in the TYMP gene, that encodes for thymidine phosphorylase. Mitochondrial neurogastrointestinal encephalopathy is a progressive degenerative disease characterized by a distinctive tetrad of gastrointestinal dysmotility, peripheral neuropathy, ophthalmoplegia with ptosis, and asymptomatic leukoencephalopathy. It provides a diagnostic dilemma to physicians in regions like Pakistan because of a lack of genetic study availability and associated financial constraints of the population. However, with careful examination and a few basic investigations, mitochondrial neurogastrointestinal encephalopathy can be diagnosed by ruling out most of the close differentials. CASE PRESENTATION: We report the case of a 23-year-old Asian female whose chief complaints were epigastric pain, bilious emesis, weight loss for 3 months, and bilateral lower limb weakness for 20 days. All clinical signs and symptoms along with relevant investigations including nerve conduction studies, electromyography, and magnetic resonance imaging of the brain were highly suggestive of mitochondrial neurogastrointestinal encephalopathy syndrome. Because of financial constraints, genetic studies could not be performed. The patient was managed with a multidisciplinary approach involving gastroenterology, physiotherapy, and nutrition departments. Currently, therapeutic options for the disease include allogeneic hematopoietic stem cell transplant and carrier erythrocyte entrapped thymidine phosphorylase; however, these could not be provided to the patient owing to certain limitations. CONCLUSIONS: As misdiagnosis and delayed diagnosis are quite common in this disease, the prime objective of this case report is to increase the basic understanding of this disease, especially its signs and symptoms, and address the limitations regarding the diagnostic investigations and management of patients with mitochondrial neurogastrointestinal encephalopathy.

Intestinal pseudo-obstruction in systemic lupus erythematosus: an analysis of nationwide inpatient sample.

Intestinal pseudo-obstruction (IPO) is a rare gastrointestinal complication of systemic lupus erythematosus (SLE). We aimed to determine the prevalence and in-hospital outcomes of SLE-related intestinal pseudo-obstruction (SLE-IPO) using a large national database. This is a retrospective study using Nationwide Inpatient Sample (NIS) from 2016 to 2018. We included adult patients with SLE using International Classification of Diseases 10th Revision (ICD-10) code M32 in combination with codes for IPO, and we determined the in-hospital mortality, occurrence of sepsis, length of hospitalization, use of total parenteral nutrition (TPN), and frequency of surgical gastrointestinal procedures. The prevalence of IPO among hospitalized adult SLE patients was 1.7%, and the in-hospital mortality of SLE-IPO was 5.04%. Compared with SLE patients without IPO (group 2), SLE-IPO patients had higher in-hospital mortality (OR 2.07, 95%CI 1.60-2.64), higher risk of sepsis (OR 1.63, 95%CI 1.42-1.87), longer in-hospital stay (ß 3.09, 95%CI 2.76-3.42), and higher frequency of TPN (OR 16.20, 95%CI 11.62-22.21) and gastrointestinal procedures (OR 5.47, 95%CI 4.62-6.43). SLE-IPO was associated with increased risk of sepsis (OR 1.29, 95%CI 1.12-1.49), compared with idiopathic IPO patients (group 3), and a possible increase in the frequency of gastrointestinal procedures (OR 1.14, 95%CI 0.96-1.34) compared with IPO secondary to diabetes mellitus (group 4), although not statistically significant. SLE-IPO patients were at twofold risk of in-hospital mortality compared with SLE patients without IPO. SLE-IPO was also associated with longer in-hospital stay and increased frequency of TPN and gastrointestinal procedures. Key Points • The prevalence of IPO in adult SLE patients was 1.7% in this US-based database. • In-hospital mortality rate of SLE-IPO was 4.95%. SLE-IPO patients were associated with twofold risk of in-hospital mortality compared with those without IPO. • SLE-IPO patients were also at increased risk of sepsis, and they had higher rate of TPN and gastrointestinal procedures.

[A case of mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) complicated by chronic intestinal pseudo-obstruction].

A 42-year-old woman presented at our hospital with acute paraphasia and word finding difficulty. She was not paralyzed or ataxic. Mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) was diagnosed based on brain MRI finding of edematous lesions in bilateral temporal lobe cortexes that did not match the vascular territory, elevated lactate and pyruvate levels in blood and cerebrospinal fluid, and the presence of a mtDNA 3243A>G mutation. From six months before her visit, she had persistent anorexia, bloating, nausea and vomiting, and weight loss to 25 kg. We diagnosed her condition as chronic intestinal pseudo-obstruction (CIPO) associated with MELAS, because a gastroenterologist had previously diagnosed her with megacolon associated with colonic dysfunction. Usually, CIPO is often associated with the chronic phase of MELAS. However, since CIPO complication from the early stage of the disease is occasionally encountered, it is necessary to include mitochondrial disease in differential diagnosis of CIPO of unknown cause.

Impact of post-operative paralytic ileus on post-operative outcomes after surgery for colorectal cancer: a single-institution, retrospective study.

PURPOSE: Post-operative paralytic ileus (POI) occurs after surgery because of gastrointestinal dysfunction caused by surgical invasion. We therefore investigated the frequency of POI after laparoscopic colorectal surgery in patients with colorectal cancer using a strictly defined POI diagnosis and identified associated risk factors. METHODS: Patients who underwent initial laparoscopic surgery for colorectal cancer between January 2014 and December 2018 were included. The primary end point was the incidence of POI. A multivariate logistic regression analysis revealed the contributing risk factors for POI. RESULTS: Of the 436 patients, 94 (21.6%) had POI. Compared with the non-POI group, the POI group had significantly higher frequencies of infectious complications (p < 0.001), pneumonia (p < 0.001), intra-abdominal abscess (p = 0.012), anastomotic leakage (p = 0.016), and post-operative bleeding (p = 0.001). In the multivariate analysis, the right colon (odds ratio [OR] 2.180, p = 0.005), pre-operative chemotherapy (OR 2.530, p = 0.047), pre-operative antithrombotic drug (OR 2.210, p = 0.032), and post-operative complications of CD grade ≥ 3 (OR 12.90, p < 0.001) were independent risk factors for POI. CONCLUSION: Post-operative management considering the risk of post-operative bowel palsy may be necessary for patients with right colon, pre-operative chemotherapy, pre-operative antithrombotic drug or severe post-operative complications.

Chronic Intestinal Pseudo-obstruction with Mitochondrial Diseases.

Chronic intestinal pseudo-obstruction (CIPO) is a rare disorder of intestinal dysmotility characterized by chronic symptoms, including vomiting and abdominal pain, associated with bowel obstruction without any mechanical obstructive causes. We herein report a case of mitochondrial diseases with recurrent duodenal obstruction that was initially diagnosed as superior mesenteric artery syndrome (SMAS) for a few years but was later diagnosed as CIPO. Since CIPO is known to be associated with mitochondrial diseases, it should be considered in the differential diagnosis of patients with mitochondrial diseases presenting with recurrent intestinal obstruction.

Etiology and Management of Pediatric Intestinal Failure: Focus on the Non-Digestive Causes.

BACKGROUND: Intestinal failure (IF) is defined as reduction in functioning gut mass below the minimal amount necessary for adequate digestion and absorption. In most cases, IF results from intrinsic diseases of the gastrointestinal tract (digestive IF) (DIF); few cases arise from digestive vascular components, gut annexed (liver and pancreas) and extra-digestive organs or from systemic diseases (non-digestive IF) (NDIF). The present review revised etiology and treatments of DIF and NDIF, with special focus on the pathophysiological mechanisms, whereby NDIF develops. METHODS: We performed a comprehensive search of published literature from January 2010 to the present by selecting the following search strings: "intestinal failure" OR "home parenteral nutrition" OR "short bowel syndrome" OR "chronic pseudo-obstruction" OR "chronic intestinal pseudo-obstruction" OR "autoimmune enteropathy" OR "long-term parenteral nutrition". RESULTS: We collected overall 1656 patients with well-documented etiology of IF: 1419 with DIF (86%) and 237 with NDIF (14%), 55% males and 45% females. Among DIF cases, 66% had SBS and among NDIF cases 90% had malabsorption/maldigestion. CONCLUSIONS: The improved availability of diagnostic and therapeutic tools has increased prevalence and life expectancy of rare and severe diseases responsible for IF. The present review greatly expands the spectrum of knowledge on the pathophysiological mechanisms through which the diseases not strictly affecting the intestine can cause IF. In view of the rarity of the majority of pediatric IF diseases, the development of IF Registries is strongly required; in fact, through information flow within the network, the Registries could improve IF knowledge and management.

Cannabinoids Improve Gastrointestinal Symptoms in a Parenteral Nutrition-Dependent Patient With Chronic Intestinal Pseudo-Obstruction.

Chronic intestinal pseudo-obstruction (CIPO) is a rare and challenging cause of pediatric intestinal failure, requiring long-term parenteral nutrition in most cases. Despite optimal management, some patients experience chronic abdominal pain and recurrent obstructive episodes with a major impact on their quality of life. Cannabinoids have been successfully used in some conditions. However, their use in CIPO has never been reported in the literature. We report a case of successful use of medicinal cannabinoids in a patient with CIPO, resulting in a significant reduction of abdominal pain, vomiting, and subocclusive episodes and increased appetite and weight, without major adverse events. Although further observations are required to consolidate these findings, this case may be helpful for other patients suffering from the same condition.

Gastroesophageal Reflux Disease and Foregut Dysmotility in Children with Intestinal Failure.

Gastrointestinal dysmotility is a common problem in a subgroup of children with intestinal failure (IF), including short bowel syndrome (SBS) and pediatric intestinal pseudo-obstruction (PIPO). It contributes significantly to the increased morbidity and decreased quality of life in this patient population. Impaired gastrointestinal (GI) motility in IF arises from either loss of GI function due to the primary disorder (e.g., neuropathic or myopathic disorder in the PIPO syndrome) and/or a critical reduction in gut mass. Abnormalities of the anatomy, enteric hormone secretion and neural supply in IF can result in rapid transit, ineffective antegrade peristalsis, delayed gastric emptying or gastroesophageal reflux. Understanding the underlying pathophysiologic mechanism(s) of the enteric dysmotility in IF helps us to plan an appropriate diagnostic workup and apply individually tailored nutritional and pharmacological management, which might ultimately lead to an overall improvement in the quality of life and increase in enteral tolerance. In this review, we have focused on the pathogenesis of GI dysmotility in children with IF, as well as the management and treatment options.

Use of pediatric donor en bloc kidneys along with bladder segment in pediatric liver-kidney and multivisceral-kidney transplantation.

The combination of pediatric multivisceral and kidney transplantation leads to additional recipient risks due to the number of anastomoses and to the small sizes of donor structures. The inclusion of donor kidneys, ureters, and a bladder patch en bloc with multivisceral organs decreases the number and complexity of anastomoses and has not yet been reported. Four patients were transplanted in this fashion; three underwent multivisceral-kidney and one underwent liver-kidney transplantation. The first patient was a 3-year-old male with polycystic kidney disease and congenital hepatic fibrosis. The second was a 7-year-old female with complications from necrotizing enterocolitis. The third was a 12-month-old male with megacystis microcolon intestinal hypoperistalsis syndrome and secondary hydronephrosis, and the fourth was a 3-year-old male with multiple intestinal resections secondary to incarcerated hernia. The third patient developed a right ureteral stenosis with an intact bladder patch. The fourth child expired from maintained abdominal sepsis. The first 3 patients maintained normal graft function. There were no cases of thrombosis, arterial stenosis, or urinary leakages. These reported cases demonstrate that small pediatric en bloc transplantation of the multivisceral organs and dual kidneys with a bladder patch anastomosis is a feasible and less complex alternative to the standard procedure.

Paralytic ileus as the presenting symptom for Guillain-Barré syndrome: a case report.

Guillain-Barré syndrome (GBS) is an acute neuroimmunological disorder characterized by rapidly ascending symmetrical limb weakness, areflexia, and sensory deficits. Approximately 65% of patients with GBS present with autonomic dysfunction, which commonly occurs in advanced stages. However, paralytic ileus, a sign of gastrointestinal dysautonomia, is rare as the presenting feature in GBS before motor weakness becomes evident. We report the case of a 54-year-old man admitted to the Emergency Department with paralytic ileus as the prodromal feature in early-stage GBS. Total parenteral feeding and prokinetic use were initiated, but no clinical improvement was observed. The patient showed rapid progression to quadriplegia, which was ultimately determined to be respiratory muscle failure requiring mechanical ventilation and intensive care unit admission. He underwent 5 days of intravenous immunoglobulin therapy and muscle strength was partially improved thereafter. However, the patient's enteral nutritional support was undesirable because of persistent poor gastric emptying complicated by fungemia and profound sepsis throughout the hospital course. Finally, he died 1 month after admission. Ignorance of this unusual prodrome to GBS could result in delayed treatment, along with potential progression to life-threatening events. Early recognition of GBS and prompt immunotherapy are critical for reducing morbidity and mortality.

[Fatal cachexia caused by mitochondrial neuro-gastro-intestinal encephalomyopathy].

In this case report, a 23-year-old normal-functioning young man was repeatedly admitted to the hospital with mal-nutrition and pseudo-obstruction. External ophthalmoplegia, global muscular atrophy and demyelinating sensory-motor-autonomic neuropathy became evident. An MRI showed symmetrical white matter lesions and muscle biopsy atrophic muscle fibres. A TYMP mutation confirmed the diagnosis, and the patient had a rapidly fatal disease course. Mitochondrial neuro-gastro-intestinal encephalo-myopathy is rare and often overlooked. In less advanced disease, stem cell transplantation can correct thymidine phosphorylase deficiency.

Neurological autoimmune disorders with prominent gastrointestinal manifestations: A review of presentation, evaluation, and treatment.

BACKGROUND: The identification of autoantibodies directed against neuronal antigens has led to the recognition of a wide spectrum of neurological autoimmune disorders (NAD). With timely recognition and treatment, many patients with NAD see rapid improvement. Symptoms associated with NAD can be diverse and are determined by the regions of the nervous system affected. In addition to neurological symptoms, a number of these disorders present with prominent gastrointestinal (GI) manifestations such as nausea, diarrhea, weight loss, and gastroparesis prompting an initial evaluation by gastroenterologists. PURPOSE: This review provides a general overview of autoantibodies within the nervous system, focusing on three scenarios in which nervous system autoimmunity may initially present with gut symptoms. A general approach to evaluation and treatment, including antibody testing, will be reviewed.