Idiopathic Intestinal Pseudo-obstruction Research Articles

僧帽弁逸脱症候群を伴った慢性特発性偽性腸閉塞症の１例

僧帽弁逸脱症候群を伴った慢性特発性偽性腸閉塞症の１例

Efficacy of antibody NF2F11 staining in the investigation of severe long-standing constipation

Abstract A retrospective study of 7 adult patients (6 with severe, long-standing constipation and 1 with chronic idiopathic intestinal pseudoobstruction) was carried out to test the diagnostic potential of the antineurofilament monoclonal antibody NF2F11. In 4 of the cases of constipation and in the 1 case of pseudoobstruction, paraffin sections of resected colon revealed an anomaly in that the axon bundles of both plexus systems remained unstained. This picture differed from that found in the control population. The results are discussed in relation to previous studies of congenital neurogenic abnormalities of the digestive tract in children.

Efficacy of antibody NF2F11 staining in the investigation of severe long-standing constipation: A preliminary report

A retrospective study of 7 adult patients (6 with severe, long-standing constipation and 1 with chronic idiopathic intestinal pseudoobstruction) was carried out to test the diagnostic potential of the antineurofilament monoclonal antibody NF2F11. In 4 of the cases of constipation and in the 1 case of pseudoobstruction, paraffin sections of resected colon revealed an anomaly in that the axon bundles of both plexus systems remained unstained. This picture differed from that found in the control population. The results are discussed in relation to previous studies of congenital neurogenic abnormalities of the digestive tract in children.

Chronic idiopathic intestinal pseudo-obstruction: Clinical and intestinal manometric findings: V. Stanghellini, M. Camilleri, and J.R. Malagelada. Gut 28:5–12, (January), 1987

Chronic idiopathic intestinal pseudo-obstruction: Clinical and intestinal manometric findings: V. Stanghellini, M. Camilleri, and J.R. Malagelada. Gut 28:5–12, (January), 1987

Chronic idiopathic intestinal pseudo-obstruction: clinical and intestinal manometric findings.

We report the clinical and intestinal manometric findings in a group of 42 patients with chronic idiopathic intestinal pseudo-obstruction evaluated at the Mayo Clinic. The main clinical manifestations in these patients were nausea and vomiting (83%), abdominal pain (74%), distension (57%), constipation (36%), diarrhoea (29%), and urinary symptoms (17%). These symptoms preceded surgery in all patients. Air fluid levels or distended bowel loops occurred in 57% and a dilated bladder or urinary excretory pathway in 17%. All patients showed intestinal manometric abnormalities none of which are seen in healthy individuals: aberrant configuration or propagation of interdigestive motor complexes in 25 patients; bursts (greater than 2 min duration) of non-propagated phasic pressure activity in fasting and/or fed state in 30 patients; sustained incoordinated fasting pressure activity in 15 patients; and inability of an ingested meal to convert fasting into fed pattern in 28 patients. We conclude that qualitative analysis of intestinal manometry provides evidence of gut dysmotility in patients with the clinical syndrome of chronic intestinal pseudo-obstruction. These abnormalities of motility can help to establish the correct diagnosis.

Long-term home parenteral nutrition in pediatrics: ten years of experience in 102 patients.

One hundred two pediatric patients received all or part of their nutritional needs parenterally at home during the past decade. All received total parenteral nutrition (TPN) at night during an 8- to 12-h infusion. Patients with short bowel syndrome (33%), inflammatory bowel disease (23%), chronic intractable diarrhea (15%), chronic idiopathic intestinal pseudo-obstruction syndrome (10%), and malignancy (10%) made up the largest groups. The mean duration of parenteral support was 735 days (range, 90-3650 days); the mean number of catheters per patient was 2.1 (range, 1-8). Twenty-one patients continue to receive full or partial home TPN: four for more than 10 years and seven for more than 5 years. Fifty-one no longer require it and have had healing of mucosa or bowel adaptation. Complications related to administration of fluid and electrolytes were quite rare. Biotin deficiency was recognized once. Thirty-one have died, but only 13 deaths were related to TPN. Sepsis in nine and liver failure in two were the most common causes of death in the TPN-related group. Three of 21 still on home TPN have graduated either from high school or college. All but one of the school age children attend regular school; one attends a school for the medically disabled, another attends a school for the mentally gifted.

748 INDICATIONS FOR AND OUTCOME OF PEDIATRIC PATIENTS ON LONG TERM HOME PARENTERAL NUTRITION (HTPN)

The records of pts on HTPN during the past decade(63,490 pt-therapy-days) were reviewed to determine frequency of diagnoses, duration of therapy, morbidity and mortality related to the technique. Diagnostic categories were: Short Bowel Syndrome in 28 pts(31%); Inflammatory Bowel Disease(20%); Malignancies(10%); Intractable Diarrhea of Infancy(10%); Idiopathic Intestinal Pseudo-obstruction(9%) and Miscellaneous Diagnoses(20%). 42% were under 1 year of age; 62% under 3 and 30% above 12 years when TPN was started. The youngest pt at time of discharge was 3 1/2 months and the lowest weight at discharge was 3.1 Kg. The mean duration of therapy was 737 days(r=23-2850). 14 pts have been on HTPN for over 4 years and 7 for over 6 years. 29 pts died during the course of HTPN; however, only 10% (9/90) of these were TPN related. Catheter related sepsis was the cause of death in 6.18 pts are still on full or partial HTPN support, with 50% at or above the 50th percentile for weight for age, and above the 25th percentile for height. Those of school age(7/8) attend regular schools. 41 no longer require TPN. They are doing well on regular diets or with oral supplements, and 75% are at or above the 25th percentile for weight and height for age. HTPN is a safe and lifesaving technique for children who cannot solely satisfy their nutritional needs via the enteral route. Although sepsis is the greatest source of mortality, long term therapy can be done safely for years, free of infection, while the patient's intestine adapts or heals, remission is obtained or until other therapies are developed for the diseases which make HTPN necessary.

Esophageal disorders in the etiology and pathophysiology of dyspepsia.

Dyspepsia may be caused by reflux esophagitis. We evaluated the symptoms of 45 patients aged 52 +/- 14 years who had a follow-up of 1 to 5 years. Endoscopy and histology demonstrated microscopic inflammation in 14, isolated mucosal defects in 12 and severe inflammation in 19 of the 45 patients. Belching was the leading symptom in patients with microscopic and severe esophagitis, heartburn in mild esophagitis. Upper abdominal pain, nausea and vomiting were present in 31%, 24% and 22% of the patients, respectively. Thus, reflux esophagitis is frequently accompanied by symptoms of dyspepsia which resemble those of other causes of dyspepsia. In contrast, disorders of gastric and intestinal motility may be associated with esophageal motor disturbances, particularly in gastric dysrhythmia, diabetic gastroenteropathy, irritable bowel syndrome, and idiopathic intestinal pseudo-obstruction. How much the esophagus contributes to the clinical symptomatology of dyspepsia awaits further elucidation.

Failure of Glucose to Reverse the Jejunal Secretory State in Chronic Idiopathic Intestinal Pseudo-Obstruction (IIP)

Failure of Glucose to Reverse the Jejunal Secretory State in Chronic Idiopathic Intestinal Pseudo-Obstruction (IIP)

Diagnosis and Treatment of Chronic Idiopathic Intestinal Pseudo-Obstruction Syndromes

Diagnosis and Treatment of Chronic Idiopathic Intestinal Pseudo-Obstruction Syndromes

Bullous acrodermatitis due to zinc deficiency during total parenteral nutrition: an ultrastructural study of the epidermal changes

A 5 1/2-year-old girl with idiopathic intestinal pseudo-obstruction became severely depleted of zinc during total parenteral nutrition and developed a vesico-bullous rash on face, hands and feet such as is seen in acrodermatitis enteropathica. Light and electron microscopy of a bullous lesion on one foot revealed a pronounced extracellular edema with cyst and cleft formation in the deep part of the epidermis. A few acantholytic cells were seen. In the electron microscope degenerate keratinocytes showed multiple vacuoles in the dark cytoplasm and slender, finger-like protrusions. Desmosomes were few. The basal lamina was well-preserved forming deep invaginations, which may serve to distinguish the condition from other bullous diseases of the skin.

Pathologic features of familial visceral myopathy

Familial visceral myopathy is the most common cause of chronic primary (idiopathic) intestinal pseudo-obstruction. We studied four family groups with this disease and found that it has a characteristic morphologic appearance. Grossly, there is segmental dilatation of the alimentary tract, often involving multiple sites and most commonly producing a megaduodenum. Microscopically, the involved areas show a characteristic change consisting of degenerating muscle cells and fibrosis, which may involve the full thickness of the muscularis propria but is often more prominent in or limited to the external layer. Degenerating muscle cells appear pale, poorly defined, and fragmented. As residual thread-like remnants become surrounded by collagen or as muscle cells are destroyed, leaving apparent spaces surrounded by collagen, the longitudinal and circular muscles take on a vacuolated appearance easily recognized at low magnifications. Recognition of this change is greatly facilitated by use of a trichrome stain, and mild lesions may be recognized only with such stains. The nondilated segments of intestine show similar changes but of a less severe degree. Neural and vascular structures are apparently normal. Although the lesion most closely resembles progressive systemic sclerosis, the degenerating muscle cells and vacuolated appearance of the muscle serve to distinguish familial visceral myopathy from the latter entity.

Selenium Deficiency and Fatal Cardiomyopathy in a Patient on Home Parenteral Nutrition

An adult patient with chronic idiopathic intestinal pseudo-obstruction maintained on home parenteral nutrition for 6 consecutive years died from cardiomyopathy and ventricular fibrillation. Postmortem examination of the heart revealed widespread myocytolysis and replacement fibrosis similar to that seen in the selenium deficient cardiomyopathy in China (Keshan disease) and animal models. Selenium deficiency in this patient was documented with extremely low concentrations of selenium and decreased activity of the selenoprotein, glutathione peroxidase, in blood, heart, liver, and skeletal muscle. Reports of selenium deficient diets causing myocardial damage in humans and animals and the findings in our patient strongly suggest that his fatal cardiomyopathy was caused by selenium deficiency.

Bacteriological findings in a case of chronic idiopathic intestinal pseudo-obstruction

Summary Clostridium butyricum was isolated in large numbers from clear fluid drained from around the intestines after bowel resection, in a patient with chronic idiopathic intestinal pseudo-obstruction (CIIPO). This organism produced hydrogen gas which was also found in large amounts in the gas distending the bowel. This finding further implicates Clostridium species in conditions in which there is gaseous distension of the bowel. It is possible that there is an infective component in the pathogenesis of CIIPO.

Chronic Idiopathic Intestinal Pseudo-Obstruction Caused by a Degenerative Disorder of the Myenteric Plexus: The Use of Smith's Method to Define the Neuropathology

In this paper we report the pathologic basis of chronic idiopathic intestinal pseudo-obstruction in a patient who had a subtotal colectomy and ileorectal anastomosis for severe obstipation. Conventional light microscopy of the resected intestine showed an increased thickness of the longitudinal muscle, minimal amounts of smooth muscle fibrosis, and normal smooth muscle cells. The morphology of the myenteric plexus was difficult to interpret with this technique, but quantification of colonic neurons revealed a significantly decreased number compared with controls. Silver stains of the myenteric plexus by Smith's method showed: (a) patchy loss of nerve tracts with replacement by Schwann cells, (b) degeneration and decreased numbers of both argryophilic and argyrophobic neurons, (c) fragmentation and dropout of many axons, and (d) increased thickness and disorganized spatial arrangement of other axons. The pathology of this intestinal neuropathy could be missed by conventional light microscopy and may be apparent only when a silver technique is used to visualize the myenteric plexus.

Extrinsic compression of the third part of the duodenum

The clinical findings in 12 patients who exhibited marked to-and-fro peristalsis in the duodenum with extrinsic compression of the third part and proximal dilatation at barium meal examination are described. In nine cases the compression was thought to be due to the superior mesenteric artery and its mesentery. In two of these cases there was associated duodenal ulceration and in another patient there was evidence of chronic idiopathic intestinal pseudo-obstruction. In the remaining three cases, the duodenal compression was caused either by tumour or malignant glands in the root of the mesentery. It is emphasised that vigorous to-and-fro peristalsis in the proximal duodenum seen at fluoroscopy is an abnormal radiological feature which is associated in most cases with significant pathology.

550 THE EXTENT OF MICROBIAL DEGRADATION OF BILE ACIDS: AN IMPORTANT FACTOR MODULATING THEIR ABSORPTION

Experimental work has shown that less polar bile acids, although better absorbed passively, have a reduced affinity for the ileal transport system and are more extensively adsorbed to residues and microbes. This study examines the fecal excretion of bile acids and the % adsorbed in relationship with the qualitative pattern in 2 categories of patients with an intact ileum. The contaminated small bowel syndrome was diagnosed in 4 cases of chronic idiopathic intestinal pseudoobstruction with fat (19.3 ± 6.8g/24h) and bile acid (693 85mg/m2/24h) malabsorption. Primary bile acids were essentially absent. The sum of deoxycholic, lithocholic, 3-ketocholanoic and 3-betacholanoic acid accounted for 80.3±6.2% of the total. Only a small % (16.7±5.4) of bile acids could be recovered in the aqueous phase. A marked reduction (P < .001) of the anaerobic flora in a fresh stool obtained from 6 CF children on triple I.V. therapy was associated with a reduction in the concentration of secondary forms (P<.005) and with a 5-fold increase (10.2 vs 52.7) in the % of bile acids in the aqueous phase when compared to 7 studied off antibiotics. In 4 CF patients studied both on oral cloxacillin and during I.V. therapy, there was no change in steatorrhea (15.9±5.7 vs 18.8±7.8g/24h) but a large decrease in bile acid loss (751±182 vs 366±93). This study suggests that the extent of microbial transformations of bile acids in the GI tract plays a role on their absorption.

Disorganised electrical activity in a child with idiopathic intestinal pseudo-obstruction.

This report presents the findings of investigation of a child with idiopathic intestinal pseudo-obstruction (IIP). Functional abnormalities of the smooth muscle of the gastrointestinal tract were disclosed by electrical recordings from the gut obtained after laparotomy. In vitro analysis of tissue and ultrastructure were undertaken and a possible aetiology of the disorder in this patient based on these findings is presented.

Chronic idiopathic intestinal pseudo-obstruction. A surgical approach.

Chronic idiopathic intestinal pseudo-obstruction is an increasingly recognized syndrome in which patients usually present with an acute or chronic history suggestive of intestinal obstruction, although no obstructing lesion is found at surgery. The diagnosis can be suspected in most cases from the clinical presentation. A diagnostic evaluation should be undertaken and exploratory laparotomy avoided if the diagnosis is confirmed on the basis of the radiographic and manometric data. If, in the acute presentation, exploratory laparotomy proves unavoidable, and dilated, nonmechanically obstructed bowel is found, a full-thickness biopsy specimen should usually be taken and the abdomen closed. A carefully chosen, palliative procedure should be reserved for patients who have well defined clinicoanatomic patterns of involvement, and who are incapacitated by their symptoms despite medical management.

Abnormal Gastrocolic Response in Patients With Intestinal Pseudo-obstruction

Gastrointestinal motility disturbances after endogenous stimuli have been demonstrated only in the esophagus and small intestine in patients with chronic idiopathic pseudo-obstruction. The 1,000-calorie meal stimulated a significant increase in spike and contractile activity in normal subjects. No increase in colonic spike or contractile activity occurred in patients with chronic idiopathic pseudo-obstruction even though colonic motility increased normally following the administration of neostigmine methylsulfate. These findings suggest that the normal response to eating is absent in patients with chronic idiopathic intestinal pseudo-obstruction even though their smooth muscle has been shown to be responsive to cholinergic stimulation. This loss of a gastrocolic response is further evidence for a disorder of the neurohumoral control mechanisms in patients with chronic idiopathic intestinal pseudo-obstruction.