chronic intestinal pseudo-obstruction

慢性假性肠梗阻
  • 文章类型: Case Reports
    治疗精神分裂症性紧张症和慢性肠道假性梗阻(CIPO)具有挑战性。紧张症的病理尚不清楚。关于这个问题的报道或研究很少。在这种情况下,我们介绍了一名诊断为精神分裂症的中年女性,其具有紧张性特征和合并症CIPO。在治疗过程中,改良电惊厥疗法(mECT)部分改善了她的昏迷和CIPO。劳拉西泮完全减轻了她的昏迷和CIPO。这是第一份描述劳拉西m在患有精神分裂症合并症和CIPO的患者中完全缓解的报告,这可能有助于探索精神分裂症伴紧张症和CIPO合并症的病理生理学和治疗。
    It is challenging to manage schizophrenic catatonia and comorbid chronic intestinal pseudo-obstruction (CIPO). The pathology of catatonia is unclear. There are few reports or research on this issue. In this case, we present a middle-aged woman diagnosed with schizophrenia with catatonic features and comorbid CIPO. In the treatment process, modified electroconvulsive therapy (mECT) improved her stupor and CIPO partially. Lorazepam alleviated her stupor and CIPO completely. It is the first report describing complete remission with lorazepam in patient suffering from comorbid schizophrenic catatonia and CIPO, which may benefit the exploration of pathophysiology and treatment of comorbidity of schizophrenia with catatonia and CIPO.
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  • 文章类型: Case Reports
    Visceral myopathy is a rare genetic disorder that commonly affects the digestive and renal systems. Manifestations include a clinical spectrum covering chronic intestinal pseudo-obstruction (CIPO) and megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS). The smooth muscle actin γ-2 gene (ACTG2) is one of the most common disease-causing genes. Here, we present a case of pediatric intestinal pseudo-obstruction associated with a novel missense ACTG2 mutation, c.588G>C/p.E196D. His parents had no this mutation, which suggested the possibility of spontaneous mutation. Amino acid conservation analysis of γ-2 actin showed replacement of glutamate at position 196 by aspartate. The patient suffered from recurrent episodes of abdominal bloating, undergone repeated gastrointestinal surgery, had feeding difficulties, and required long-term parenteral nutrition support. The patient had no other specific symptoms or underlying diseases. X-ray of the abdomen showed dilation of the intestine as well as an air-fluid pattern. The manifestations of biopsy were various. All biochemical tests were normal, and the possibility of secondary intestinal pseudo-obstruction was excluded. The mutation site of ACTG2 in the present study has not been previously described in patients with visceral myopathy, and thus, our study reveals a novel mutation of ACTG2-associated visceral myopathy in a patient with CIPO. This report can serve as a reference for future research and further expands the map of genetic variation for visceral myopathy.
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  • 文章类型: Journal Article
    Chronic intestinal pseudo-obstruction (CIPO) is a type of intestinal dysfunction presenting as symptoms of intestinal obstruction but without actual mechanical obstruction. An extremely low incidence, non-specific clinical symptoms, strong heterogeneity, and no definitive cause in some patients make CIPO very difficult to diagnose correctly. Imaging and gastrointestinal manometry are commonly used. Most patients have progressive worsening of their symptoms and require intervention, and nutritional assessment and treatment are very important to determine the prognosis. With improvements in surgical techniques, small bowel transplantation is a feasible treatment option for patients with advanced CIPO; however, the long-term prognosis for CIPO patients remains unsatisfactory. Generally, the disease is rare and difficult to diagnose, which leads to clinicians\' lack of understanding of the disease and results in a high rate of misdiagnosis. This review describes the characteristics of CIPO and the latest developments in diagnosis and treatment, in detail. The goal of our review is to improve clinicians\' understanding of CIPO so that the disease is identified quickly and accurately, and treated as early as possible to improve patients\' quality of life.
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  • 文章类型: Case Reports
    患有抗CV2/塌陷素反应介质蛋白(CRMP)5抗体的患者出现更频繁的舞蹈病,小脑共济失调,uveo/视网膜症状,以及Lambert-Eaton肌无力综合征或重症肌无力.慢性假性肠梗阻(CIPO)是一种肠运动功能障碍疾病,是由神经肌肉疾病引起的,在没有机械性梗阻的情况下具有复发性或持续性肠梗阻。我们报告了与自主神经功能障碍相关的CRMP5抗体阳性副肿瘤神经综合征(PNS)患者的病例(最明显的表现为CIPO)。ClPO是PNS的最稀有形式之一。一些抗CV2/CRMP5抗体阳性的PNS患者可能有致命的并发症,例如CIPO。为了检测PNS患者是否有CIPO的风险,需要及时诊断和适当治疗。
    Patients with anti-CV2/collapsin response mediator protein (CRMP)5 antibodies present with more frequent chorea, cerebellar ataxia, uveo/retinal symptoms, and Lambert-Eaton myasthenic syndrome or myasthenia gravis. Chronic intestinal pseudo-obstruction (CIPO) is an intestinal motility dysfunction disease dysmotility that is caused by a neuromuscular disease with recurrent or persistent intestinal obstruction in the absence of mechanical obstruction. We report the case of a patient with CRMP5 antibody-positive paraneoplastic neurological syndrome (PNS) that is associated with autonomic dysfunction (presenting most remarkably as CIPO). CIPO is one of the rarest forms of PNS. Some PNS patients who are positive for anti-CV2/CRMP5 antibodies may have fatal complications such as CIPO. To detect if PNS patients are at risk for CIPO, a timely diagnosis and appropriate treatment are required.
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  • 文章类型: Letter
    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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