1/14. Protein-losing enteropathy associated with hypocomplementemia and anti-nuclear antibodies.We report a case of protein-losing enteropathy associated with an autoimmune disorder, presumably systemic lupus erythematosus. Although typical manifestations of systemic lupus erythematosus were lacking, a high serum cholesterol level, a low serum complement level, positivity for anti-nuclear antibody, and positivity for anti-single-strand dna antibody suggested an autoimmune mechanism as the cause of the condition. Although immunohistological examination of duodenal and ileal biopsy specimens failed to reveal deposits of immune complex or complement in the vessels, capillary hyperpermeability was suspected as the mechanism of the condition.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/14. Computed tomography after lymphangiography in the diagnosis of intestinal lymphangiectasia with protein-losing enteropathy in Noonan's syndrome.Noonan's syndrome is a rare congenital disorder that may be associated with abnormalities in the lymphatic drainage. In this case of a 21-year-old man CT after bipedal lymphangiography confirmed the diagnosis of intestinal lymphangiectasy causing protein-losing enteropathy in Noonan's syndrome by showing contrast-enhanced abnormal lymphatic vessels in the mesentery and the intestinal wall. Because of the benefit of diet in case of intestinal involvement, we recommend a thorough documentation of the lymphatic drainage with lymphangiography followed by CT, if clinical signs of lymphatic dysplasia, such as pleural effusions, lymphedema, or hypoproteinemia are present.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
3/14. A case of protein-losing enteropathy caused by intestinal lymphangiectasia in a preterm infant.Intestinal lymphangiectasia is characterized by obstruction of lymph drainage from the small intestine and lacteal dilation that distorts the villus architecture. Lymphatic vessel obstruction and elevated intestinal lymphatic pressure in turn cause lymphatic leakage into the intestinal lumen, thus resulting in malabsorption and protein-losing enteropathy. Intestinal lymphangiectasia can be congenital or secondary to a disease that blocks intestinal lymph drainage. We describe the first case of intestinal lymphangiectasia in a premature infant. The infant presented with peripheral edema and low serum albumin; high fecal concentration of alpha(1)-antitrypsin documented intestinal protein loss. endoscopy showed white opaque spots on the duodenal mucosa, which indicates dilated lacteal vessels. histology confirmed dilated lacteals and also showed villus blunting. A formula containing a high concentration of medium chain triglycerides resulted in a rapid clinical improvement and normalization of biochemical variables. These features should alert neonatologists to the possibility of intestinal lymphangiectasia in newborns with hypoalbuminemia and peripheral edema. The intestinal tract should be examined for enteric protein losses if other causes (ie, malnutrition and protein loss from other sites) are excluded. The diagnosis rests on jejunal biopsy demonstrating dilated lymphatic lacteal vessels.- - - - - - - - - - ranking = 3keywords = vessel (Clic here for more details about this article) |
4/14. Intraabdominal lymphangiomyoma in an infant with protein-losing enteropathy and hemihypertrophy.lymphangiomyoma is an extremely rare tumor occurring exclusively in women of reproductive age. The tumor is characterized by proliferation of immature smooth muscle along the lymphatic vessels of the abdomen, thorax and lung. Although lymphangiomyoma has been reported in a young girl and a girl infant, none has been reported in boys. We report herein a case of lymphangiomyoma in a two-year-old boy. The unusual presentation in this patient was that the tumor arose from the small bowel mesentery without any evidence of lung involvement. The tumor was extirpated and lymphangiomyomatosis was confirmed pathologically.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
5/14. Acquired intestinal lymphangiectasia successfully treated with a low-fat and medium-chain triacylglycerol-enriched diet in a patient with liver transplantation.Intestinal lymphangiectasia is defined as a dilatation of small bowel lymphatic capillaries and a loss of lymph into the bowel lumen. Clinically it is characterized by hypoproteinaemia and oedema. We present here a case of protein-losing enteropathy due to intestinal lymphangiectasia after liver transplantation in a 57-year-old man who was transplanted for hepatitis c virus. Four years after liver transplantation, the patient developed hypoalbuminaemia and ascites associated with recurrence of cirrhosis. The sudden fall in serum albumin led us to look for a cause of reduction other than or in addition to cirrhosis. Duodenal biopsies showed tall villi with dilated lymphatic vessels and widening of the villi caused by oedema, demonstrating intestinal lymphangiectasia. In this case a low-fat diet supplemented with medium-chain triacylglycerols achieved an early clinical improvement with increased serum albumin levels and ascites disappearance. Intestinal lymphangiectasia should be suspected in liver-transplanted patients developing hypoproteinaemia and hypoalbuminaemia after the recurrence of cirrhosis.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
6/14. Angiotropic large cell lymphoma (angioendotheliomatosis) presenting with protein-losing enteropathy.A 73 year old female presented with progressive hypoproteinaemic oedema which was resistant to treatment. The cause was not established until post-mortem, when it was found that she had angiotropic large cell lymphoma involving the vessels of the large intestine with mucosal ulceration. To our knowledge, this is the first report of angiotropic large cell lymphoma, which usually presents with neurological and skin involvement, presenting with protein-losing enteropathy.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
7/14. Endoscopic diagnosis of primary intestinal lymphangiectasia using a high-fat meal.The case of a 17-year-old woman is described, in whom a suspected diagnosis of primary intestinal lymphangiectasia was confirmed endoscopically and histologically only after a high-fat meal had been given the night before the procedure. A characteristic endoscopic finding was the appearance of tiny white dots scattered in the duodenum and jejunum, which on histological examination proved to be dilated lymph vessels. Treatment with low-fat diet and MCT-supplementation was successful.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
8/14. Primary intestinal lymphangiectasia. Case report with radiological and ultrastructural study.A 16-year-old boy with primary intestinal lymphangiectasia presented with peripheral edema of 6 weeks duration. Laboratory and radiological studies included absolute lymphopenia, hypoalbuminemia, steatorrhea, abnormal stool 51Cr-albumin value, edema of small intestinal folds, dilated mesenteric lymphatics, as well as bilateral filling defects in external and common iliac lymph nodes. Abdominal CT scan revealed a possible periaortic mass. Small intestinal biopsies demonstrated normal villous architecture with dilated lacteals, and electron microscopy revealed enterocytes with normal as well as blunted microville, enlarged, dilated lacteals and intercellular vacuoles. An exploratory laparatomy was performed and revealed no masses but dilated serosal lymphatic vessels. Medical treatment, including marked restriction of long chain triglyceride intake, resulted in loss of peripheral edema, weight stabilization, and normal activity.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
9/14. Clinicopathologic consideration of protein-losing enteropathy due to lymphangiectasia of the intestine.The pathogenesis of intestinal lymphangiectasia and mechanism of enteric loss of serum protein has been discussed from the pathologic and roentgenologic viewpoints of the two patients with this disease. Enteric loss of serum protein was attributed to exudation of lymph from ruptured lymphatic vessels, resulting from non-specific multiple ulcers of the small intestine in one patient and malrotation of the gut in another.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
10/14. Bypass enteropathy: an inflammatory process in the excluded segment with systemic complications.Evidence is presented that many of the enteric and systemic manifestations after jejunoileal bypass can be related to an inflammatory process within the bypassed small bowel rather than to the surgically induced sequelae of a short bowel syndrome with malabsorption. Invasion of the excluded segment by fecal flora was associated with a histologically demonstrable inflammatory response of the mucosa. The disorder was of variable severity and duration and occurred in the majority of 28 bypass patients. Progression to a clinical syndrome resembling an acute abdomen occurred in about 15% of the patients. Small bowel ileus and, in some patients, obstruction of the colon were suggested by physical signs and x-ray findings. Surgical exploration in such instances demonstrated an inflammaotry process of the excluded small bowel loops with severe distention of this segment and of the colon, but not organic obstruction. pneumatosis cystoides intestinalis was a sequal in two patients. Exudative protein loss was documented in the severe cases. Most of the systemic sequelae are comparable to those seen with inflammatory diseases of the bowel such as Crohn's disease. fever, excessive weight and lean tissue loss, and the involvement of skin, blood vessels, joints and possibly, the liver suggest an immune response as a common factor in the pathogenesis. The clinical improvement with antibiotics such as metronidazole or with restitution of normal bowel continuity indicates that the bacterial flora in the excluded small bowel segment or its byproducts are causally related to the systemic complications. hyperoxaluria may be primarily the sequela of steatorrhea and not of the inflammatory process.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
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