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1/6. pregnancy in a patient with chronic intestinal pseudo-obstruction on long-term parenteral nutrition.

    parenteral nutrition support is provided in most instances for short intervals during pregnancy in conditions where oral/enteral intake is severely compromised. Few reports describe the use of parenteral nutrition from conception to delivery. We report the case of a 30-year-old woman suffering from a severe form of chronic intestinal pseudo-obstruction on long-term parenteral nutrition because of malabsorption and malnutrition. pregnancy and delivery developed uneventfully. The fetus grew normally throughout pregnancy. Our patient needed only slight modifications in her parenteral nutrition regimen during lactation. There were no metabolic complications during pregnancy. We conclude that female patients even with severe forms of gastrointestinal diseases, such as chronic intestinal pseudo-obstruction requiring long-term home parenteral nutrition, can conceive and carry successfully a pregnancy to term.
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2/6. Chronic radiation enteritis after ovarian cancer: from home parenteral nutrition to oral diet.

    INTRODUCTION: External beam radiation of abdominal and pelvic cavities is a current therapy for gynaecological cancer that often produces radiation-induced bowel injury and malnutrition. CASE REPORT: A 72-year old patient underwent surgery and external beam radiation therapy for an ovarian carcinoma. Two years later she was found to have intestinal pseudoobstruction related to chronic radiation enteritis and protein-energy malnutrition. Home parenteral nutrition was prescribed due to poor oral intake, but it was discontinued after 6 catheter-related sepsis and upper cava vein thrombosis. parenteral nutrition could be reintroduced after an angioplasty of that vein, and the patient was operated on with the finding of an incarcerated ileum eventration. Nowadays she maintains a normal nutritional status with oral diet. DISCUSSION: radiation enteritis can lead to perforation, fistulae or strictures of the bowel. Malnutrition is common and parenteral nutrition may be necessary. Surgery can solve these complications, achieves good survival rates and can allow stopping parenteral nutrition.
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3/6. Pneumatosis and pneumoperitoneum in chronic idiopathic intestinal pseudoobstruction.

    Chronic idiopathic intestinal pseudoobstruction is a diagnosis of exclusion for the rare patient with severe small bowel atony without any demonstrable organic cause. The very poor prognosis associated with this disease has been somewhat improved with the advent of parenteral nutrition; nevertheless, these patients follow a stormy course characterized by malnutrition, recurrent obstruction, infection, hemorrhage, and perforation. We describe a 16-year-old boy with this disease who presented to us with vague abdominal pain and pneumoperitoneum. laparotomy showed pneumatosis cystoides intestinalis without intestinal perforation. The presence of free air in the abdominal cavity is an almost pathognomonic sign of intestinal perforation. pneumatosis cystoides intestinalis is one of the very few nonsurgical causes of pneumoperitoneum. Beyond the neonatal period, in which it is the hallmark of necrotizing enterocolitis, it has been described in patients with severe obstructive lung disease, in collagenous disorders, and in the short-bowel syndrome. In the present case, it may have resulted from chronic intestinal distension, allowing air under pressure to dissect through the bowel wall. The challenge of such a unique association of conditions lies in the avoidance of unnecessary surgery in a chronic, essentially nonsurgical disease while avoiding unnecessary delay in a possibly acute surgical event.
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4/6. Chronic intestinal pseudo-obstruction. A report of 27 cases and review of the literature.

    Twenty-seven cases of chronic intestinal pseudo-obstruction are reported. The causes of pseudo-obstruction were progressive systemic sclerosis in 14, hollow visceral myopathy in 4, visceral neuropathy in 2, sclerosing mesenteritis in 1, and jejunal diverticulosis in 1. No identifiable cause was found in five. Chronic pseudo-obstruction is a long-term illness characterized by vomiting, abdominal distention, abdominal pain and weight loss. Involvement is often present throughout the intestine so that patients may present with a variety of symptoms deriving from the esophagus, stomach, small intestine, and colon. Hollow visceral myopathy and visceral neuropathy are usually familial and urologic involvement is sometimes present in the former. Abnormalities of smooth muscle function can be discerned by radiography and esophageal manometry. The pattern and distribution of the abnormalities are helpful in differentiating pseudo-obstruction from true mechanical obstruction. They may also be helpful in differentiating one form of pseudo-obstruction from another. The majority of cases have identifiable pathology within either the smooth muscle or myenteric plexus of the bowel wall. The natural history of pseudo-obstruction is variable. Remissions and exacerbations occur and may be unrelated to anything that is done therapeutically. The illness is unresponsive to any drug known to have an effect on intestinal motility. Antibiotic treatment of small intestinal bacterial overgrowth and selected surgical procedures may occasionally be palliative. Many patients develop malnutrition and require home parenteral nutrition in order to survive.
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5/6. Brown bowel syndrome: report of two cases.

    Brown bowel syndrome is a rare intestinal disorder associated with the deposition of lipofuscin pigment in the smooth muscle cells. We report two such cases presenting with intestinal pseudo-obstruction, abdominal pain, and body weight loss. Both cases had malabsorption and fatty liver. Exploratory laparotomy revealed brownish discoloration of the small bowel wall and enlargement of mesenteric lymph nodes. light microscopy, autofluorescence and ultrastructure studies confirmed the deposition of lipofuscin pigments in the intestinal muscle cells and reticuloendothelial cells of mesenteric lymph nodes. In addition, the calf muscle biopsy of case 1 displayed myopathy and fatty replacement. Skeletal muscle strength of both patients was partially restored after parenteral and oral vitamin E supplement and other conservative treatment, but gastrointestinal symptoms of both patients continued to deteriorate. Thus, brown bowel syndrome associated with prolonged and severe malnutrition and possibly vitamin e deficiency appears only partially responsive to vitamin E supplementation.
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6/6. Familial visceral neuropathy as part of a diffuse neuronal syndrome: four fatal cases in one sibship.

    Familial visceral neuropathy is a rare cause of chronic intestinal pseudo-obstruction. It is characterized by progressive destruction of the gastrointestinal myenteric plexus resulting in dysmotility and associated early satiety, post-prandial bloating, recurrent nausea and vomiting, abdominal distension, chronic diarrhea, weight loss, and malnutrition. In its varying forms, there may be neuronal destruction in other parts of the peripheral and central nervous system. We report on four siblings who presented in their third or fourth decades with initial clinical features of chronic intestinal pseudo-obstruction and eventual progressive diffuse neuronal disease, characterized by leukoencephalopathy and peripheral neuropathy. Within 5 yr of presentation, all four patients died from inanition and sepsis, despite aggressive nutritional support. Their clinical and pathological features are characteristic of familial visceral neuropathy of the autosomal recessive form. This presentation may represent a unique syndrome characterized by a tetrad of polyneuropathy, ophthalmoplegia, leukoencephalopathy, and intestinal pseudo-obstruction.
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