-
British Medical Journal (Clinical... May 1986
Topics: Aged; Colonic Diseases; Humans; Intestinal Obstruction; Intestinal Pseudo-Obstruction; Male
PubMed: 3087479
DOI: 10.1136/bmj.292.6533.1463 -
Nutrients Nov 2020Gastrointestinal dysmotility is a common problem in a subgroup of children with intestinal failure (IF), including short bowel syndrome (SBS) and pediatric intestinal... (Review)
Review
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.
Topics: Child; Gastroesophageal Reflux; Gastrointestinal Motility; Gastrointestinal Tract; Humans; Intestinal Pseudo-Obstruction; Short Bowel Syndrome
PubMed: 33217928
DOI: 10.3390/nu12113536 -
Ultrasound in Obstetrics & Gynecology :... Jun 2019Fetal megacystis presents a challenge in terms of counseling and management because of its varied etiology and evolution. The aim of this study was to present a...
OBJECTIVE
Fetal megacystis presents a challenge in terms of counseling and management because of its varied etiology and evolution. The aim of this study was to present a comprehensive overview of the underlying etiologies and structural anomalies associated with fetal megacystis.
METHODS
This was a retrospective multicenter study of cases referred to the fetal medicine unit of one of the eight academic hospitals in The Netherlands with a diagnosis of fetal megacystis. For each case, data on and measurements of fetal urinary tract and associated structural anomalies were collected. All available postmortem examinations and postnatal investigations were reviewed in order to establish the final diagnosis. In the first trimester, fetal megacystis was defined as longitudinal bladder diameter (LBD) ≥ 7 mm, and in the second and third trimesters as an enlarged bladder failing to empty during an extended ultrasound examination lasting at least 40 min.
RESULTS
Of the 541 pregnancies with fetal megacystis, it was isolated (or solely accompanied by other signs of lower urinary tract obstruction (LUTO)) in 360 (67%) cases and associated with other abnormal ultrasound findings in 181 (33%) cases. The most common associated ultrasound anomaly was an increased nuchal translucency thickness (22%), followed by single umbilical artery (10%) and cardiac defect (10%). A final diagnosis was established in 418 cases, including 222 (53%) cases with isolated LUTO and 60 (14%) infants with normal micturition or minor isolated urological anomalies. In the remaining 136 (33%) cases, concomitant developmental or chromosomal abnormality or genetic syndrome was diagnosed. Overall, 40 chromosomal abnormalities were diagnosed, including trisomy 18 (n = 24), trisomy 21 (n = 5), Turner syndrome (n = 5), trisomy 13 (n = 3) and 22q11 deletion (n = 3). Thirty-two cases presented with anorectal malformations involving the anus, rectum and urogenital tract. In cases with confirmed urethral and anal atresia, megacystis occurred early in pregnancy and the bladder appeared severely distended (the LBD (in mm) was equal to or greater than twice the gestational age (in weeks)). Fetal macrosomia was detected in six cases and an overgrowth syndrome was detected in four cases, comprising two infants with Beckwith-Wiedemann syndrome and two with Sotos syndrome. Megacystis-microcolon-intestinal hypoperistalsis syndrome was diagnosed in five (1%) cases and prenatally suspected only in one case.
CONCLUSIONS
Although the main cause of fetal megacystis is LUTO, an enlarged fetal bladder can also be present as a concomitant finding of miscellaneous genetic syndromes, developmental disturbances and chromosomal abnormalities. We provide an overview of the structural anomalies and congenital disorders associated with fetal megacystis and propose a practical guide for the differential diagnosis of genetic syndromes and chromosomal and developmental abnormalities in pregnancies presenting with fetal megacystis, focusing on the morphological examination of the fetus. © 2018 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of the International Society of Ultrasound in Obstetrics and Gynecology.
Topics: Abnormalities, Multiple; Colon; Female; Humans; Intestinal Pseudo-Obstruction; Netherlands; Pregnancy; Pregnancy Outcome; Retrospective Studies; Ultrasonography, Prenatal; Urinary Bladder
PubMed: 30043466
DOI: 10.1002/uog.19182 -
EMBO Molecular Medicine Oct 2015Mitochondrial disorders are a group of highly invalidating human conditions for which effective treatment is currently unavailable and characterized by faulty energy... (Review)
Review
Mitochondrial disorders are a group of highly invalidating human conditions for which effective treatment is currently unavailable and characterized by faulty energy supply due to defective oxidative phosphorylation (OXPHOS). Given the complexity of mitochondrial genetics and biochemistry, mitochondrial inherited diseases may present with a vast range of symptoms, organ involvement, severity, age of onset, and outcome. Despite the wide spectrum of clinical signs and biochemical underpinnings of this group of dis-orders, some common traits can be identified, based on both pathogenic mechanisms and potential therapeutic approaches. Here, we will review two peculiar mitochondrial disorders, ethylmalonic encephalopathy (EE) and mitochondrial neurogastrointestinal encephalomyopathy (MNGIE), caused by mutations in the ETHE1 and TYMP nuclear genes, respectively. ETHE1 encodes for a mitochondrial enzyme involved in sulfide detoxification and TYMP for a cytosolic enzyme involved in the thymidine/deoxyuridine catabolic pathway. We will discuss these two clinical entities as a paradigm of mitochondrial diseases caused by the accumulation of compounds normally present in traces, which exerts a toxic and inhibitory effect on the OXPHOS system.
Topics: Brain Diseases, Metabolic, Inborn; Humans; Intestinal Pseudo-Obstruction; Mitochondrial Diseases; Mitochondrial Encephalomyopathies; Mitochondrial Proteins; Muscular Dystrophy, Oculopharyngeal; Mutation; Nucleocytoplasmic Transport Proteins; Ophthalmoplegia; Purpura; Thymidine Phosphorylase
PubMed: 26194912
DOI: 10.15252/emmm.201505040 -
Minerva Gastroenterologica E Dietologica Dec 2004Thyroid diseases may be related to gastrointestinal motility symptoms. Such symptoms can vary in degree and, sometimes, are the only clue of a thyroid disease or, at... (Review)
Review
Thyroid diseases may be related to gastrointestinal motility symptoms. Such symptoms can vary in degree and, sometimes, are the only clue of a thyroid disease or, at least, the first. The mechanism by which the thyroid hormones can influence gastrointestinal motility, even if not still completely elucidated, can be found in a synergism between a direct effect of the thyronins and an indirect effect mediated by cathecolamines on the muscle cell receptors. Neck discomfort and dysphagia are common findings in patients with thyroid diseases. Hyper- and hypothyroidism can impair esophageal motility, modifying pharyngo-esophageal structure and/or muscular function and interacting with the neuro-humoral regulation of the esophageal peristalsis. Oesophageal motility alterations, observed in patients affected by small non-toxic goiter, are less understandable. At the gastro-duodenal level, basic and postprandial electric rhythm alterations have been observed in hyperthyroid patients, often associated with delayed gastric emptying, too. In such patients, the autonomous nervous system dysfunction may even modify the neuro-hormonal mutual regulation (vagal influence decrease) of the gastro-duodenal myoelectric activity. Hypothyroidism may cause a delay of the gastric emptying too, but such pattern may also be related to an associated autoimmune disease or to an independent chronic modification of the gastric mucosa. Diarrhoea and malabsorption are common findings together with hyperthyroidism, whereas constipation is frequently observed in hypothyroidism. The clinically most demanding situation is certainly the secondary chronic intestinal pseudo-obstruction syndrome, which involves the bowel in most cases, but may also show up by means of a mega-small bowel or a mega-duodenum, or even all of the above. In conclusion it may be stated that: 1) thyroid diseases may be related to symptoms due to digestive motility dysfunction. 2) Any segment of the gastrointestinal trait may be involved. 3) The typical clinical manifestations of the thyroid illnesses may be borderline, missing or concealed by other intercurrent illnesses, especially in the elderly patients. 4) Motility-related digestive symptoms may conceal an underlying, easily misdetected, thyroid disease and must be therefore carefully analyzed.
Topics: Esophageal Motility Disorders; Gastrointestinal Motility; Humans; Hypothyroidism; Intestinal Diseases; Intestinal Pseudo-Obstruction; Thyroid Diseases
PubMed: 15788986
DOI: No ID Found -
Journal of Clinical Oncology : Official... Mar 2013Many cancer centers offer acupuncture services. To date, a comprehensive systematic review of acupuncture in cancer care has not been conducted. The purpose of this... (Review)
Review
PURPOSE
Many cancer centers offer acupuncture services. To date, a comprehensive systematic review of acupuncture in cancer care has not been conducted. The purpose of this review was to evaluate the efficacy of acupuncture for symptom management in patients with cancer.
METHODS
Medline, Embase, CINAHL, Cochrane (all databases), Scopus, and PubMed were searched from inception through December 2011 for prospective randomized clinical trials (RCT) evaluating acupuncture for symptom management in cancer care. Only studies involving needle insertion into acupuncture points were included. No language limitations were applied. Studies were assessed for risk of bias (ROB) according to Cochrane criteria. Outcomes by symptom were designated as positive, negative, or unclear.
RESULTS
A total of 2,151 publications were screened. Of those, 41 RCTs involving eight symptoms (pain, nausea, hot flashes, fatigue, radiation-induced xerostomia, prolonged postoperative ileus, anxiety/mood disorders, and sleep disturbance) met all inclusion criteria. One positive trial of acupuncture for chemotherapy-induced nausea and vomiting had low ROB. Of the remaining studies, eight had unclear ROB (four positive, three negative, and one with unclear outcomes). Thirty-three studies had high ROB (19 positive, 11 negative, and three with both positive and negative outcomes depending on the symptom).
CONCLUSION
Acupuncture is an appropriate adjunctive treatment for chemotherapy-induced nausea/vomiting, but additional studies are needed. For other symptoms, efficacy remains undetermined owing to high ROB among studies. Future research should focus on standardizing comparison groups and treatment methods, be at least single-blinded, assess biologic mechanisms, have adequate statistical power, and involve multiple acupuncturists.
Topics: Acupuncture Therapy; Antineoplastic Agents; Anxiety; Evidence-Based Medicine; Fatigue; Hot Flashes; Humans; Intestinal Pseudo-Obstruction; Mood Disorders; Nausea; Neoplasms; Observer Variation; Pain; Pain Management; Radiotherapy; Randomized Controlled Trials as Topic; Sleep Wake Disorders; Treatment Outcome; Xerostomia
PubMed: 23341529
DOI: 10.1200/JCO.2012.43.5818 -
Revista Espanola de Enfermedades... Feb 2007Chronic intestinal pseudo-obstruction (CIPO) is a syndrome characterized by the presence of recurrent episodes of clinical intestinal obstruction in the absence of... (Comparative Study)
Comparative Study Review
Chronic intestinal pseudo-obstruction (CIPO) is a syndrome characterized by the presence of recurrent episodes of clinical intestinal obstruction in the absence of obstructive lesions. Although this syndrome is rare, it causes a high morbidity. It is caused by a disturbance of the intestinal motility, that results in a failure of the progression of the intestinal content. Basically, the failure of the intestinal motility is a consequence of muscular disorder, neurological disorder or both. Usually, CIPO is secondary to other systemic disease; however, in the last years, many cases of primary CIPO have been described. The use of new manometric tecniques and specific histological procedures have allowed to clarify the pathogenesis of some of these entities including mitochondrial diseases and paraneoplasic syndromes. Clinical manifestations of CIPO are diverse, depending on the location and extension of the motility disorder. As the diagnosis of this disease is usually not an easy task, patients frecuently undergo unnecesary surgical interventions, are diagnosed of psyquiatric disorders, or the correct diagnosis is delayed several years after the first symptoms arise. The aims of the treatment are to maintain the nutritional condition and to improve symptoms using nutritional measures, drugs or, eventually, endoscopical or surgical procedures.
Topics: Chronic Disease; Cisapride; Dietary Supplements; Endoscopy; Gastrointestinal Agents; Humans; Intestinal Pseudo-Obstruction; Nutritional Status; Octreotide; Palliative Care; Prognosis; Radiography, Abdominal; Tomography, X-Ray Computed
PubMed: 17417923
DOI: 10.4321/s1130-01082007000200008 -
Rheumatology (Oxford, England) Sep 2023SSc is an autoimmune disease characterized by excessive fibrosis in multiple organs, including the gastrointestinal (GI) tract. GI symptoms of SSc such as intestinal...
OBJECTIVES
SSc is an autoimmune disease characterized by excessive fibrosis in multiple organs, including the gastrointestinal (GI) tract. GI symptoms of SSc such as intestinal pseudo-obstruction (IPO) are often refractory to conventional intervention and can result in longer in-hospital stay or even increased mortality. We aimed to summarize the insights to date regarding the efficacy of IVIG against GI symptoms of SSc to unveil what we should focus on in future studies.
METHODS
Herein we report the response of GI symptoms in three cases with SSc-myositis overlap who received IVIG administration. We also conducted a systematic literature review to summarize previous reports regarding the efficacy of IVIG upon the GI manifestations of SSc, according to the PRISMA 2020 guideline.
RESULTS
The case series demonstrated remarkable and rapid improvement of GI symptoms, including IPO, after IVIG administration. The literature review revealed that previous reports also support the efficacy and safety of IVIG against GI manifestations of SSc. However, they were all retrospective studies and lacking description of the short-term outcome after IVIG administration with objective and quantitative metrics.
CONCLUSION
IVIG seems to be a promising therapeutic option for the management of GI symptoms in SSc, including IPO. Investigators should focus more on short-term outcomes to properly assess the therapeutic benefit of IVIG, ideally using reliable quantitative measures in a multicentre randomized placebo-controlled setting.
Topics: Humans; Immunoglobulins, Intravenous; Retrospective Studies; Scleroderma, Systemic; Gastrointestinal Diseases; Intestinal Pseudo-Obstruction
PubMed: 36825818
DOI: 10.1093/rheumatology/kead093 -
Polish Archives of Internal Medicine Aug 2020
Topics: Animals; Humans; Intestinal Pseudo-Obstruction; Liver; Strongyloides stercoralis
PubMed: 32388970
DOI: 10.20452/pamw.15352 -
Developmental Biology Sep 2016Over the last 20 years, there has been increasing focus on the development of novel stem cell based therapies for the treatment of disorders and diseases affecting the... (Review)
Review
Over the last 20 years, there has been increasing focus on the development of novel stem cell based therapies for the treatment of disorders and diseases affecting the enteric nervous system (ENS) of the gastrointestinal tract (so-called enteric neuropathies). Here, the idea is that ENS progenitor/stem cells could be transplanted into the gut wall to replace the damaged or absent neurons and glia of the ENS. This White Paper sets out experts' views on the commonly used methods and approaches to identify, isolate, purify, expand and optimize ENS stem cells, transplant them into the bowel, and assess transplant success, including restoration of gut function. We also highlight obstacles that must be overcome in order to progress from successful preclinical studies in animal models to ENS stem cell therapies in the clinic.
Topics: Animals; Cell- and Tissue-Based Therapy; Disease Models, Animal; Enteric Nervous System; Gastrointestinal Tract; Guidelines as Topic; Hirschsprung Disease; Humans; Intestinal Pseudo-Obstruction; Neural Stem Cells; Stem Cell Transplantation
PubMed: 27059883
DOI: 10.1016/j.ydbio.2016.04.001