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      Urinary glutamine/glutamate ratio as a potential biomarker of pediatric chronic intestinal pseudo-obstruction

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          Abstract

          Chronic intestinal pseudo-obstruction (CIPO) is a rare intestinal motility disorder with significant morbidity and mortality in pediatric patients. The diagnosis of CIPO is difficult, because it is clinically based on the symptoms and signs of bowel obstruction which are similar to the clinical manifestations of other gastrointestinal diseases like short bowel syndrome (SBS). Therefore, it is desirable to identify and establish new laboratory diagnostic markers for CIPO that are reliable and easily accessible. In our study we have identified the ratio of the urinary glutamine and glutamic acid as a promising biomarker for distinguishing suspected CIPO cases and simple SBS cases. The area under ROC curve was 0.83, at cutoff value = 7.04 with sensitivity of 65% and specificity of 92%.

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          The online version of this article (doi:10.1186/s13023-017-0615-3) contains supplementary material, which is available to authorized users.

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          Chronic intestinal pseudo-obstruction.

          Chronic intestinal pseudo-obstruction (CIP) is a rare and serious disorder of the gastrointestinal (GI) tract characterized as a motility disorder with the primary defect of impaired peristalsis; symptoms are consistent with a bowel obstruction, although mechanical obstruction cannot be identified. CIP is classified as a neuropathy, myopathy, or mesenchymopathy; it is a neuropathic process in the majority of patients. The natural history of CIP is generally that of a progressive disorder, although occasional patients with secondary CIP note significant symptomatic improvement when the underlying disorder is identified and treated. Symptoms vary from patient to patient depending on the location of the luminal GI tract involved and the degree of involvement; however, the small intestine is nearly always involved. Common symptoms include dysphagia, gastroesophageal reflux, abdominal pain, nausea, vomiting, bloating, abdominal distension, constipation or diarrhea, and involuntary weight loss. Unfortunately, these symptoms are nonspecific, which can contribute to misdiagnosis or a delay in diagnosis and treatment. Since many of the symptoms and signs suggest a mechanical bowel obstruction, diagnostic tests typically focus on uncovering a mechanical obstruction, although routine tests do not identify an obstructive process. Nutrition supplementation is required for many patients with CIP due to symptoms of dysphagia, nausea, vomiting, and weight loss. This review discusses the epidemiology, etiology, pathogenesis, diagnosis, and treatment of patients with CIP, with an emphasis on nutrition assessment and treatment options for patients with nutrition compromise.
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            Diagnosis and treatment of chronic intestinal pseudo-obstruction in children: report of consensus workshop.

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              Gastrointestinal motility disorders in children.

              The most common and challenging gastrointestinal motility disorders in children include gastroesophageal reflux disease (GERD), esophageal achalasia, gastroparesis, chronic intestinal pseudo-obstruction, and constipation. GERD is the most common gastrointestinal motility disorder affecting children and is diagnosed clinically and treated primarily with acid secretion blockade. Esophageal achalasia, a less common disorder in the pediatric patient population, is characterized by dysphagia and treated with pneumatic balloon dilation and/or esophagomyotomy. Gastroparesis and chronic intestinal pseudo-obstruction are poorly characterized in children and are associated with significant morbidity. Constipation is among the most common complaints in children and is associated with significant morbidity as well as poor quality of life. Data on epidemiology and outcomes, clinical trials, and evaluation of new diagnostic techniques are needed to better diagnose and treat gastrointestinal motility disorders in children. We present a review of the conditions and challenges related to these common gastrointestinal motility disorders in children.
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                Author and article information

                Contributors
                yanjunkai2015@sjtu.edu.cn
                zhoukejun1984@hotmail.com
                hjh19911101@qq.com
                852509291@qq.com
                997856659@qq.com
                abelardccwang@gmail.com
                caiw204@sina.com
                Journal
                Orphanet J Rare Dis
                Orphanet J Rare Dis
                Orphanet Journal of Rare Diseases
                BioMed Central (London )
                1750-1172
                28 March 2017
                28 March 2017
                2017
                : 12
                Affiliations
                [1 ]ISNI 0000 0004 0630 1330, GRID grid.412987.1, Department of Pediatric Surgery, , Xin Hua Hospital Affiliated to Shanghai Jiao Tong University School of Medicine, ; 1665 Kongjiang Rd, Shanghai, 200092 China
                [2 ]ISNI 0000 0004 0368 8293, GRID grid.16821.3c, , Shanghai Institute for Pediatric Research, ; Shanghai, China
                [3 ]Shanghai Key Laboratory of Pediatric Gastroenterology and Nutrition, Shanghai, China
                [4 ]ISNI 0000 0001 0727 1557, GRID grid.411234.1, Department of Public Health, , Aichi Medical University, ; Aichi, Japan
                Article
                615
                10.1186/s13023-017-0615-3
                5371254
                © The Author(s). 2017

                Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License ( http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver ( http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

                Funding
                Funded by: Shanghai Key Laboratory of Pediatric Gastroenterology and Nutrition
                Award ID: 14DZ2272400
                Award Recipient :
                Funded by: Shanghai Municipal Commission of Health and Family Planning
                Award ID: 2013ZYJB0017
                Award Recipient :
                Funded by: FundRef http://dx.doi.org/10.13039/501100003399, Science and Technology Commission of Shanghai Municipality;
                Award ID: 14411950400/ 14411950401
                Award Recipient :
                Categories
                Letter to the Editor
                Custom metadata
                © The Author(s) 2017

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