1/6. 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 = 1keywords = malnutrition (Clic here for more details about this article) |
2/6. Primary protein-losing enteropathy in anti-double-stranded dna disease: the initial and sole clinical manifestation of occult systemic lupus erythematosus?hypoalbuminemia and generalized edema is a common clinical problem and the etiology is usually clear: cirrhosis, nephrotic syndrome, primary gastrointestinal disorders, malnutrition, etc. We present a 23-year-old previously healthy woman of Korean background who presented with generalized edema and a serum albumin of 9 g/L (normal, 35-45 g/L). Intensive investigations failed to reveal liver, renal, or inflammatory gastrointestinal mucosal disease. The antinuclear antibody was positive at a titer of 1:80, and extractable nuclear antigens were positive for SSA/anti-Ro. Anti-double-stranded dna was markedly elevated at 4.6 kU/L (normal, 0-2.0 kU/L). A technetium 99M-labeled albumin study revealed a protein-losing enteropathy, despite normal histologic full-thickness jejunal biopsies. A diagnosis of occult systemic lupus erythematosus resulting in increased intestinal vascular permeability was made. The hypoalbuminemia remained in long-term remission after the initiation of induction and maintenance immunosuppression.- - - - - - - - - - ranking = 1keywords = malnutrition (Clic here for more details about this article) |
3/6. giardiasis with protein-losing enteropathy: diagnosis by fecal alpha 1-antitrypsin determination.giardia lamblia infection was documented by jejunal biopsy in a previously healthy 2-year-old boy with acute onset of hypoproteinemia due to protein-losing enteropathy. All symptoms and abnormal laboratory findings resolved with anti-Giardia therapy. This is only the second case report of giardiasis with documented protein-losing enteropathy. Further application of the fecal alpha 1-antitrypsin assay may help to clarify the relationship between Giardia infection and protein-losing enteropathy and its role in development of malnutrition.- - - - - - - - - - ranking = 1keywords = malnutrition (Clic here for more details about this article) |
4/6. Synthesis rates of albumin and fibrinogen in patients with protein-losing enteropathy and in a patient recovering from protein malnutrition.Using the 14C carbonate method of McFarlane, the synthesis rates of albumin and fibrinogen were determined in four patients with protein-losing enteropathy, in one patient during the recovery phase after severe protein malabsorption and in nine control patients. The following results were obtained: (1) The synthesis rates of albumin and fibrinogen were significantly increased in all patients studied. (2) All patients had low serum albumin levels. The increase in albumin synthesis rate was remarkably uniform in all patients, the average being 645 mg/kg/day or 2.7 times the mean control value. (3) plasma fibrinogen levels were normal in all patients. The fibrinogen synthesis rate varied over a wide range from 42.1 to 199.3 mg/kg/day, i.e. 2-8.5 times the control value.- - - - - - - - - - ranking = 4keywords = malnutrition (Clic here for more details about this article) |
5/6. Congenital enterocyte heparan sulphate deficiency with massive albumin loss, secretory diarrhoea, and malnutrition.BACKGROUND: The molecular basis of protein-losing enteropathy is unknown. However it has been shown that sulphated glycosaminoglycans may be important in regulating vascular and renal albumin loss. methods: We describe three baby boys who presented within the first weeks of life with massive enteric protein loss, secretory diarrhoea, and intolerance of enteral feeds. All required total parenteral nutrition and repeated albumin infusions. No cause could be found in any case despite extensive investigations, including small intestinal biopsy sampling, which were repeatedly normal. FINDINGS: By specific histochemistry, we detected gross abnormality in the distribution of small intestinal glycosaminoglycans in all three infants, with complete absence of enterocyte heparan sulphate. The distribution of vascular and lamina propria glycosaminoglycans was, however, normal. INTERPRETATION: The presentation of these infants suggests that enterocyte heparan sulphate is important in normal small intestinal function.- - - - - - - - - - ranking = 4keywords = malnutrition (Clic here for more details about this article) |
6/6. Surgical treatment of Cronkhite-canada syndrome associated with protein-losing enteropathy: report of a case.PURPOSE: The case of a patient with Cronkhite-canada syndrome, who developed a protein-losing enteropathy, is reported. methods: After localization of the protein-losing region, a right colectomy was performed. RESULTS: hypoproteinemia and ectodermal changes improved postoperatively. CONCLUSIONS: Surgery is an effective treatment for protein-losing enteropathy in Cronkhite-canada syndrome. Ectodermal changes improve after correcting malnutrition.- - - - - - - - - - ranking = 1keywords = malnutrition (Clic here for more details about this article) |