Chronic intestinal pseudo-obstruction (CIPO) is a se- vere digestive syndrome characterized by derangement of gut propulsive motility which resembles mechanical obstruction, in the absence of any obstructive process. ...Chronic intestinal pseudo-obstruction (CIPO) is a se- vere digestive syndrome characterized by derangement of gut propulsive motility which resembles mechanical obstruction, in the absence of any obstructive process. Although uncommon in clinical practice, this syndrome represents one of the main causes of intestinal failure and is characterized by high morbidity and mortality. It may be idiopathic or secondary to a variety of diseases. Most cases are sporadic, even though familial forms with either dominant or recessive autosomal inheritance have been described. Based on histological features in- testinal pseudo-obstruction can be classified into three main categories:neuropathies, mesenchymopathies, and myopathies, according on the predominant involvement of enteric neurones, interstitial cells of Cajal or smooth muscle cells, respectively. Treatment of intestinal pseu- do-obstruction involves nutritional, pharmacological and surgical therapies, but it is often unsatisfactory and the long-term outcome is generally poor in the majority of cases.展开更多
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 clinica...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.展开更多
Visceral myopathy is one of the causes of chronic intestinal pseudo-obstruction. Most cases pathologically reveal degenerative changes of myocytes or muscularis propia atrophy and fibrosis. Abnormal layering of muscul...Visceral myopathy is one of the causes of chronic intestinal pseudo-obstruction. Most cases pathologically reveal degenerative changes of myocytes or muscularis propia atrophy and fibrosis. Abnormal layering of muscularis propria is extremely rare. We report a case of a 9-mo-old Thai male baby who presented with chronic intestinal pseudo-obstruction. Histologic findings showed abnormal layering of small intestinal muscularis propria with an additional oblique layer and aberrant muscularization in serosa. The patient also had a short small bowel without malrotation, brachydactyly,and absence of the 2nd to 4th middle phalanges of both hands. The patient was treated with cisapride and combined parenteral and enteral nutritional support.He had gradual clinical improvement and gained body weight. Subsequently, the parenteral nutrition was discontinued. The previously reported cases are reviewed and discussed.展开更多
Chronic intestinal pseudo-obstruction(CIPO) is a rare disease due to a severe gastrointestinal motility disorder which may mimic,on both clinical and radiological grounds,mechanical obstruction.We report a case of a 2...Chronic intestinal pseudo-obstruction(CIPO) is a rare disease due to a severe gastrointestinal motility disorder which may mimic,on both clinical and radiological grounds,mechanical obstruction.We report a case of a 26-year-old woman who presented to our institution for plain abdominal radiography for referred long-lasting constipation with recurrent episodes of abdominal pain and distension.At X-ray,performed both in the upright and supine position,an isolated air-fluid level was depicted in the left flank,together with a number of radiological signs suggestive of pneumoperitoneum.First,subphrenic radiolucency could be observed in the upright film.Second,the intestinal wall of some jejunal loops appeared to be outlined in the right flank.Third,the inferior cardiac border was clearly depicted in the upright film.The patient however had no evidence ofperitoneal signs but only hypoactive bowel movements.Unenhanced multi-detector computed tomography(MDCT) of the abdomen and pelvis was therefore performed.MDCT revealed abnormal air-driven distension of the small and large bowel,without evidence of extraluminal air.All radiological signs of pneumoperitoneum turned out to be false-positive results.The patient was submitted to pan-colonoscopy and to anorectal manometry to rule out Hirshprung's disease,and was finally discharged with a diagnosis of CIPO.展开更多
Chronic intestinal pseudo-obstruction (CIP) is an infre-quent complication of an active systemic lupus erythema-tosus (SLE). We illustrate a case of SLE inactive-related CIP. A 51-year old female with inactive SLE (EC...Chronic intestinal pseudo-obstruction (CIP) is an infre-quent complication of an active systemic lupus erythema-tosus (SLE). We illustrate a case of SLE inactive-related CIP. A 51-year old female with inactive SLE (ECLAM score 2) was hospitalized with postprandial fullness, vomiting, abdominal bloating and abdominal pain. She had had no bowel movements for five days. Plain abdominal X-ray revealed multiple fluid levels and dilated small and large bowel loops with air-fluid levels. Intestinal contrast radiology detected dilated loops. CIP was diagnosed. The patient was treated with prokinetics, octreotide, claritromycin, rifaximin, azathioprine and tegaserod without any clinical improvement. Then methylprednisolone (500 mg iv daily) was started. After the first administration, the patient showed peristaltic movements. A bowel movement was reported after the second administration. A plain abdominal X-ray revealed no air-fluid levels. Steroid therapy was slowly reduced with complete resolution of the symptoms. The patient is still in a good clinical condition. SLE-related CIP is generally reported as a complication of an active disease. In our case, CIP was the only clinical demonstration of the SLE.展开更多
AIM: To reveal the frequency, characteristics and prognosis of chronic intestinal pseudo-obstruction (CIP) in mitochondrial disease patients. METHODS: Between January 2000 and December 2010, 31 patients (13 males and ...AIM: To reveal the frequency, characteristics and prognosis of chronic intestinal pseudo-obstruction (CIP) in mitochondrial disease patients. METHODS: Between January 2000 and December 2010, 31 patients (13 males and 18 females) were di-agnosed with mitochondrial diseases at our hospital. We conducted a retrospective review of the patients' sex, subclass of mitochondrial disease, age at onset of mitochondrial disease, frequency of CIP and the age at its onset, and the duration of survival. The age at onset or at the first diagnosis of the disorder that led to the clinical suspicion of mitochondrial disease was also examined. RESULTS: Twenty patients were sub-classified with mitochondrial encephalopathy with lactic acidosis and stroke-like episodes (MELAS), 8 with chronic progressive external ophthalmoplegia (CPEO), and 3 with myoclonus epilepsy associated with ragged-red fibers (MERRF). Nine patients were diagnosed with CIP, 8 of the 20 (40.0%) patients with MELAS, 0 of the 8 (0.0%) patients with CPEO, and 1 of the 3 (33.3%) patients with MERRF. The median age (range) at the diagnosis and the median age at onset of mitochondrial disease were 40 (17-69) and 25 (12-63) years in patients with CIP, and 49 (17-81) and 40 (11-71) years in patients without CIP. During the survey period, 5 patients (4 patients with MELAS and 1 with CPEO) died. The cause of death was cardiomyopathy in 2 patients with MELAS, cerebral infarction in 1 patient with MELAS, epilepsy and aspiration pneumonia in 1 patient with MELAS, and multiple metastases from gastric cancer and aspiration pneumonia in 1 patient with CPEO. CONCLUSION: Patients with CIP tend to have disorders that are suspected to be related to mitochondrial diseases at younger ages than are patients without CIP.展开更多
Inflammatory bowel diseases(IBD),Crohns disease and ulcerative colitis,are chronic conditions associated with high morbidity and healthcare costs.The natural history of IBD is variable and marked by alternating period...Inflammatory bowel diseases(IBD),Crohns disease and ulcerative colitis,are chronic conditions associated with high morbidity and healthcare costs.The natural history of IBD is variable and marked by alternating periods of flare and remission.Even though the use of newer therapeutic targets has been associated with higher rates of mucosal healing,a great proportion of IBD patients remain symptomatic despite effective control of inflammation.These symptoms may include but not limited to abdominal pain,dyspepsia,diarrhea,urgency,fecal incontinence,constipation or bloating.In this setting,commonly there is an overlap with gastrointestinal(GI)motility and absorptive disorders.Early recognition of these conditions greatly improves patient care and may decrease the risk of mistreatment.Therefore,in this review we describe the prevalence,diagnosis and treatment of GI motility and absorptive disorders that commonly affect patients with IBD.展开更多
Experimental evidence indicates that chronic mechanical sub-occlusion of the intestine may damage the enteric nervous system （ENS）, although data in humans are lacking. We here describe the first case of enteric deg...Experimental evidence indicates that chronic mechanical sub-occlusion of the intestine may damage the enteric nervous system （ENS）, although data in humans are lacking. We here describe the first case of enteric degenerative neuropathy related to a congenital obstruction of the gut. A 3-year and 9-mo old girl began to complain of vomiting, abdominal distension, constipation with air-fluid levels at plane abdominal radiology. Her subsequent medical history was characterized by 3 operations： the first showed dilated duodeno-jejunal loops in the absence of occlusive lesions; the second （2 years later） was performed to obtain full-thickness biopsies of the dilated intestinal loops and revealed hyperganglionosis at histopathology; the third （9 years after the hyperganglionosis was identified） disclosed a Ladd＇s band which was removed and the associated gut malrotation was corrected. Repeated intraoperative full-thickness biopsies showed enteric degenerative neuropathy along with reduced interstitial cells of Cajal network in dilated loops above the obstruction and a normal neuromuscular layer below the Ladd＇s band. One year after the latest surgery the patient tolerated oral feeding and did well, suggesting that congenital （partial） mechanical obstruction of the small bowel in humans can evoke progressive adaptive changes of the ENS which are similar to those found in animal models of intestinal mechanical occlusion. Such ENS changes mimic neuronal abnormalities observed in intestinal pseudoobstruction.展开更多
Digestion and intestinal absorption allow the body to sustain itself and are the emblematic functions of the bowel.On the flip side,functions also arise from its role as an interface with the environment.Indeed,the gu...Digestion and intestinal absorption allow the body to sustain itself and are the emblematic functions of the bowel.On the flip side,functions also arise from its role as an interface with the environment.Indeed,the gut houses microorganisms,collectively known as the gut microbiota,which interact with the host,and is the site of complex immune activities.Its role in human pathology is complex and scientific evidence is progressively elucidating the functions of the gut,especially regarding the pathogenesis of chronic intestinal diseases and inflammatory conditions affecting various organs and systems.This editorial aims to highlight and relate the factors involved in the pathogenesis of intestinal and systemic inflammation.展开更多
文摘Chronic intestinal pseudo-obstruction (CIPO) is a se- vere digestive syndrome characterized by derangement of gut propulsive motility which resembles mechanical obstruction, in the absence of any obstructive process. Although uncommon in clinical practice, this syndrome represents one of the main causes of intestinal failure and is characterized by high morbidity and mortality. It may be idiopathic or secondary to a variety of diseases. Most cases are sporadic, even though familial forms with either dominant or recessive autosomal inheritance have been described. Based on histological features in- testinal pseudo-obstruction can be classified into three main categories:neuropathies, mesenchymopathies, and myopathies, according on the predominant involvement of enteric neurones, interstitial cells of Cajal or smooth muscle cells, respectively. Treatment of intestinal pseu- do-obstruction involves nutritional, pharmacological and surgical therapies, but it is often unsatisfactory and the long-term outcome is generally poor in the majority of cases.
基金Supported by the Initial Scientific Research Fund of Young of Beijing Tsinghua Changgung Hospital,No.12020C1003.
文摘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.
基金Supported by Faculty of Medicine,Ramathibodi Hospital,Mahidol University,Thailand
文摘Visceral myopathy is one of the causes of chronic intestinal pseudo-obstruction. Most cases pathologically reveal degenerative changes of myocytes or muscularis propia atrophy and fibrosis. Abnormal layering of muscularis propria is extremely rare. We report a case of a 9-mo-old Thai male baby who presented with chronic intestinal pseudo-obstruction. Histologic findings showed abnormal layering of small intestinal muscularis propria with an additional oblique layer and aberrant muscularization in serosa. The patient also had a short small bowel without malrotation, brachydactyly,and absence of the 2nd to 4th middle phalanges of both hands. The patient was treated with cisapride and combined parenteral and enteral nutritional support.He had gradual clinical improvement and gained body weight. Subsequently, the parenteral nutrition was discontinued. The previously reported cases are reviewed and discussed.
文摘Chronic intestinal pseudo-obstruction(CIPO) is a rare disease due to a severe gastrointestinal motility disorder which may mimic,on both clinical and radiological grounds,mechanical obstruction.We report a case of a 26-year-old woman who presented to our institution for plain abdominal radiography for referred long-lasting constipation with recurrent episodes of abdominal pain and distension.At X-ray,performed both in the upright and supine position,an isolated air-fluid level was depicted in the left flank,together with a number of radiological signs suggestive of pneumoperitoneum.First,subphrenic radiolucency could be observed in the upright film.Second,the intestinal wall of some jejunal loops appeared to be outlined in the right flank.Third,the inferior cardiac border was clearly depicted in the upright film.The patient however had no evidence ofperitoneal signs but only hypoactive bowel movements.Unenhanced multi-detector computed tomography(MDCT) of the abdomen and pelvis was therefore performed.MDCT revealed abnormal air-driven distension of the small and large bowel,without evidence of extraluminal air.All radiological signs of pneumoperitoneum turned out to be false-positive results.The patient was submitted to pan-colonoscopy and to anorectal manometry to rule out Hirshprung's disease,and was finally discharged with a diagnosis of CIPO.
文摘Chronic intestinal pseudo-obstruction (CIP) is an infre-quent complication of an active systemic lupus erythema-tosus (SLE). We illustrate a case of SLE inactive-related CIP. A 51-year old female with inactive SLE (ECLAM score 2) was hospitalized with postprandial fullness, vomiting, abdominal bloating and abdominal pain. She had had no bowel movements for five days. Plain abdominal X-ray revealed multiple fluid levels and dilated small and large bowel loops with air-fluid levels. Intestinal contrast radiology detected dilated loops. CIP was diagnosed. The patient was treated with prokinetics, octreotide, claritromycin, rifaximin, azathioprine and tegaserod without any clinical improvement. Then methylprednisolone (500 mg iv daily) was started. After the first administration, the patient showed peristaltic movements. A bowel movement was reported after the second administration. A plain abdominal X-ray revealed no air-fluid levels. Steroid therapy was slowly reduced with complete resolution of the symptoms. The patient is still in a good clinical condition. SLE-related CIP is generally reported as a complication of an active disease. In our case, CIP was the only clinical demonstration of the SLE.
基金Health and Labour Sciences Research Grants for Research on Intractable Diseases, awarded to Nakajima A, from the Ministry of Health, Labour and Welfare of Japan
文摘AIM: To reveal the frequency, characteristics and prognosis of chronic intestinal pseudo-obstruction (CIP) in mitochondrial disease patients. METHODS: Between January 2000 and December 2010, 31 patients (13 males and 18 females) were di-agnosed with mitochondrial diseases at our hospital. We conducted a retrospective review of the patients' sex, subclass of mitochondrial disease, age at onset of mitochondrial disease, frequency of CIP and the age at its onset, and the duration of survival. The age at onset or at the first diagnosis of the disorder that led to the clinical suspicion of mitochondrial disease was also examined. RESULTS: Twenty patients were sub-classified with mitochondrial encephalopathy with lactic acidosis and stroke-like episodes (MELAS), 8 with chronic progressive external ophthalmoplegia (CPEO), and 3 with myoclonus epilepsy associated with ragged-red fibers (MERRF). Nine patients were diagnosed with CIP, 8 of the 20 (40.0%) patients with MELAS, 0 of the 8 (0.0%) patients with CPEO, and 1 of the 3 (33.3%) patients with MERRF. The median age (range) at the diagnosis and the median age at onset of mitochondrial disease were 40 (17-69) and 25 (12-63) years in patients with CIP, and 49 (17-81) and 40 (11-71) years in patients without CIP. During the survey period, 5 patients (4 patients with MELAS and 1 with CPEO) died. The cause of death was cardiomyopathy in 2 patients with MELAS, cerebral infarction in 1 patient with MELAS, epilepsy and aspiration pneumonia in 1 patient with MELAS, and multiple metastases from gastric cancer and aspiration pneumonia in 1 patient with CPEO. CONCLUSION: Patients with CIP tend to have disorders that are suspected to be related to mitochondrial diseases at younger ages than are patients without CIP.
文摘Inflammatory bowel diseases(IBD),Crohns disease and ulcerative colitis,are chronic conditions associated with high morbidity and healthcare costs.The natural history of IBD is variable and marked by alternating periods of flare and remission.Even though the use of newer therapeutic targets has been associated with higher rates of mucosal healing,a great proportion of IBD patients remain symptomatic despite effective control of inflammation.These symptoms may include but not limited to abdominal pain,dyspepsia,diarrhea,urgency,fecal incontinence,constipation or bloating.In this setting,commonly there is an overlap with gastrointestinal(GI)motility and absorptive disorders.Early recognition of these conditions greatly improves patient care and may decrease the risk of mistreatment.Therefore,in this review we describe the prevalence,diagnosis and treatment of GI motility and absorptive disorders that commonly affect patients with IBD.
基金Supported by the Italian Ministry of Education, University and Research (CCOFIN Project No. 2004062155 to RDeG,2004055120 to GB and 2003064378 to RDeG, GB and VS)
文摘Experimental evidence indicates that chronic mechanical sub-occlusion of the intestine may damage the enteric nervous system （ENS）, although data in humans are lacking. We here describe the first case of enteric degenerative neuropathy related to a congenital obstruction of the gut. A 3-year and 9-mo old girl began to complain of vomiting, abdominal distension, constipation with air-fluid levels at plane abdominal radiology. Her subsequent medical history was characterized by 3 operations： the first showed dilated duodeno-jejunal loops in the absence of occlusive lesions; the second （2 years later） was performed to obtain full-thickness biopsies of the dilated intestinal loops and revealed hyperganglionosis at histopathology; the third （9 years after the hyperganglionosis was identified） disclosed a Ladd＇s band which was removed and the associated gut malrotation was corrected. Repeated intraoperative full-thickness biopsies showed enteric degenerative neuropathy along with reduced interstitial cells of Cajal network in dilated loops above the obstruction and a normal neuromuscular layer below the Ladd＇s band. One year after the latest surgery the patient tolerated oral feeding and did well, suggesting that congenital （partial） mechanical obstruction of the small bowel in humans can evoke progressive adaptive changes of the ENS which are similar to those found in animal models of intestinal mechanical occlusion. Such ENS changes mimic neuronal abnormalities observed in intestinal pseudoobstruction.
文摘Digestion and intestinal absorption allow the body to sustain itself and are the emblematic functions of the bowel.On the flip side,functions also arise from its role as an interface with the environment.Indeed,the gut houses microorganisms,collectively known as the gut microbiota,which interact with the host,and is the site of complex immune activities.Its role in human pathology is complex and scientific evidence is progressively elucidating the functions of the gut,especially regarding the pathogenesis of chronic intestinal diseases and inflammatory conditions affecting various organs and systems.This editorial aims to highlight and relate the factors involved in the pathogenesis of intestinal and systemic inflammation.
