1/35. intestinal pseudo-obstruction and acute pandysautonomia associated with Epstein-Barr virus infection.We report the association of neurological and intestinal disorders with the reactivation of Epstein-Barr virus (EBV) in a child. This previously healthy 13-yr-old boy presented with pharyngitis and acute abdominal ileus. laparotomy excluded a mechanical obstruction. Postoperatively, he suffered from prolonged intestinal obstruction, pandysautonomia, and encephalomyelitis. Histological examination of the appendix and a rectal biopsy taken 3 months after the onset showed an absence of ganglion cells (appendix) and hypoganglionosis (rectum), with a mononucleate inflammatory infiltrate in close contact with the myenteric neural plexuses. EBV-PCR was positive in the blood and cerebrospinal fluid, and in situ hybridization with the Epstein-Barr virus encoded rna probe showed positive cells throughout the appendix wall including the myenteric area, in a mesenteric lymph node, and in the gastric biopsies. EBV spontaneous lymphocytic proliferation was noted in the blood. The serology for EBV showed previous infection but anti-early antigen antibodies were present. No immunodeficiency was found. Neurological and GI recovery occurred after 6 months of parenteral nutrition and bethanechol. The omnipresence of EBV associated with the neurointestinal symptoms suggest that the virus was the causal agent. This is the first documented case of acquired hypoganglionnosis due to EBV reactivation.- - - - - - - - - - ranking = 1keywords = nutrition (Clic here for more details about this article) |
2/35. The striking effect of hyperbaric oxygenation therapy in the management of chronic idiopathic intestinal pseudo-obstruction.Chronic idiopathic intestinal pseudo-obstruction is one of the disorders that is most refractory to medical and surgical treatment. Even when patients are given nutritional support, including total parenteral nutrition, obstructive symptoms seldom disappear. We report a case of chronic idiopathic intestinal pseudo-obstruction, due to myopathy, in which hyperbaric oxygenation therapy was strikingly effective. The presence of myopathy was histologically confirmed on the surgically resected jejunal specimen. hyperbaric oxygenation resulted not only in relief of the patient's obstructive symptoms but also in a rapid decrease of abnormally accumulated intestinal gas. At last, he could resume oral intake without any critical adverse effects. These observations strongly suggest that hyperbaric oxygenation can be an effective therapy in the management of chronic idiopathic intestinal pseudo-obstruction.- - - - - - - - - - ranking = 2keywords = nutrition (Clic here for more details about this article) |
3/35. Chronic idiopathic intestinal pseudo-obstruction.Chronic idiopathic intestinal pseudo-obstruction (CIIP) is a rare heterogeneous clinical syndrome characterized by recurrent episodes of symptoms and signs of intestinal obstruction in the absence of a mechanically obstructing lesion. dilatation of other viscera, such as the renal pelvis, ureter or urinary bladder, is identified in a minority of patients. We report the cases of two patients with CIIP presenting with abdominal fullness and constipation. Radiologic examination of the first patient revealed dilatation of the esophagus, stomach, duodenum and bowel loops up to the ascending colon. The nerve conduction velocity study of the right extremities revealed polyneuropathy and urinary bladder manometry revealed poor sensation. The patient had been admitted to our hospital three times for symptomatic relief within the prior six months. During the last admission, his symptoms persisted without response to medical treatment. Soon after discharge, the patient underwent surgery at another hospital and died of nutritional problems. The second patient was transferred to our hospital after an exploratory laparotomy was performed one month earlier. A radiographic examination revealed distention of the stomach, duodenum, small intestine and ascending colon, as well as bilateral hydronephrosis. Rheumatologic examination revealed no evidence of autoimmune disorder. The patient also had heavy proteinuria due to minimal change disease that was proven by renal biopsy. After receiving prokinetic, cathartic and corticosteroid medication for kidney disease, symptoms improved, but hydronephrosis persisted.- - - - - - - - - - ranking = 1keywords = nutrition (Clic here for more details about this article) |
4/35. 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.- - - - - - - - - - ranking = 10keywords = nutrition (Clic here for more details about this article) |
5/35. Treatment of chronic hypertension with intravenous enalaprilat and transdermal clonidine.We report an 11-year-old boy with hypertension and chronic intestinal pseudo-obstruction, which renders him totally dependent on parenteral nutrition and prevents the use of oral medications. Here we report the feasibility of utilizing chronic i.v. enalaprilat and transdermal clonidine on a chronic basis to control hypertension. Over the last 10 months, the patient's hypertension has been well controlled by 1.25 mg i.v. enalaprilat every 8 h and a 0.2-mg clonidine patch every 6 days, with no apparent side-effects. There are no reports of i.v. enalaprilat usage exceeding 3 weeks' duration. Therefore we believe that it is possible to effect reasonable management of chronic hypertension with the use of chronic i.v. enalaprilat and transdermal clonidine therapy.- - - - - - - - - - ranking = 1keywords = nutrition (Clic here for more details about this article) |
6/35. Submucosal hypoganglionosis causing chronic idiopathic intestinal pseudo-obstruction.A 39-year-old woman presented with recurrent symptoms suggestive of intestinal obstruction. She was put on total parenteral nutrition (TPN) and consequently developed sepsis and endocarditis. TPN was stopped and a venting enterostomy was performed. Biopsies of mucosa and submucosa were taken at surgery; immunohistochemistry for neuronal proteins, protein gene product 9.5 (PGP 9.5) and the glial S-100-protein was done. Many enlarged nerve fiber strands were found in the submucosa. Few small ganglia containing a small number of nerve cells could be observed, suggesting hypoganglionosis. This patient with chronic idiopathic intestinal pseudoobstruction of neurogenic type had a defect in the submucous plexus, whereas visceral neuropathies are usually characterized by defects of the myenteric plexus with normal submucous plexus.- - - - - - - - - - ranking = 1keywords = nutrition (Clic here for more details about this article) |
7/35. Surgical treatment of chronic intestinal pseudo-obstruction: report of three cases.We report the cases of three patients with chronic intestinal pseudo-obstruction (CIP) whose quality of life (QOL) was improved by palliative surgery in combination with home parenteral nutrition (HPN). Loop enterostomy and shortening of the gastrointestinal tract was performed with good results in all three patients to relieve abdominal distension that was progressive despite treatment with prokinetic drugs. Oral intake was also improved after surgical treatment in one patient who underwent massive resection of the jejunoileum. HPN maintained a satisfactory nutritional state in all patients. These three case reports serve to demonstrate that surgical intervention to improve QOL should be considered for patients with CIP if medication is not effective and symptoms are progressive.- - - - - - - - - - ranking = 2keywords = nutrition (Clic here for more details about this article) |
8/35. Treatment of intestinal pseudo obstruction by segmental resection.intestinal pseudo-obstruction refractory to medical therapy is a debilitating problem for specialists dealing with gastrointestinal disorders. We report the case of a newborn who developed severe, recurrent symptoms of intestinal obstruction, due to visceral myopathy. The case was persistently intractable to medical management, leading to repeated laparotomies. Gastrointestinal lesions showed marked dilatation of the entire digestive tract, with enlarging to enormously distended segments at two areas. Resection of these segments improved bowel function, facilitating enteral nutrition. Long-term hyperalimentation and repetitive hospitalizations were also avoided with this procedure. These results suggest that segmental resections can save unnecessary intestinal resections in cases with extensive gastrointestinal involvement.- - - - - - - - - - ranking = 1keywords = nutrition (Clic here for more details about this article) |
9/35. Mitochondrial myopathy (complex I deficiency) associated with chronic intestinal pseudo-obstruction.We report a patient presenting with severe muscular impairment and chronic intestinal pseudo-obstruction (CIP) at the age of eight months. Due to the aggravated symptoms, assisted ventilation, an ileostomy and total parenteral nutrition were required. Later on, the patient developed a locked-in syndrome (Leigh's subacute necrotising encephalomyelopathy) and finally died due to recurrent pneumonia and chronic renal failure. The assessment of muscle biopsies revealed a moderate single-fibre type II atrophy, a variation of muscle fibre calibre with focal fatty degeneration and a decreased reactivity of cytochrome-c oxidase. Although ragged red fibres had not been found, mitochondrial enzyme activities were markedly decreased with the lowest residual activity detected for NADH:Q1 oxidoreductase and NADH:O2 oxidoreductase (complex I deficiency), thereby confirming the diagnosis of mitochondrial myopathy. A molecular genetic analysis could not identify known mutations of mitochondrial dna. Gastrointestinal full-thickness biopsies revealed myenteric hypoganglionosis of the colon and stomach and hyperplasia of the submucosal plexus of the ileum. Some of the intestinal smooth muscle cells displayed bulbous protrusions filled with lateralised mitochondria. mitochondrial myopathies are known to be associated with a variety of clinical syndromes including CIP. However, in contrast to previous reports in which CIP has been attributed to visceral intestinal myopathies, the present case is characterised by neuronal intestinal malformations. Therefore, a mitochondrial myopathy associated with CIP requires a subtle assessment of both the intestinal smooth muscle and the enteric nervous system to identify the underlying pathology.- - - - - - - - - - ranking = 1keywords = nutrition (Clic here for more details about this article) |
10/35. 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.- - - - - - - - - - ranking = 12keywords = nutrition (Clic here for more details about this article) |
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