A number of disorders have been described to cause protein losing enteropathy (PLE) in children. Primary intestinal lymphangiectasia (PIL) is one mechanism leading to PLE. Few syndromes are associated with PIL; Hennek...A number of disorders have been described to cause protein losing enteropathy (PLE) in children. Primary intestinal lymphangiectasia (PIL) is one mechanism leading to PLE. Few syndromes are associated with PIL; Hennekam syndrome (HS) is one of them. The principal treatment for PIL is a high protein, low fat diet with medium chain triglycerides supplementation. Supportive therapy includes albumin infusion. Few publications have supported the use of octreotide to diminish protein loss and minimize hypoalbuminemia seen in PIL. There are no publications on the treatment of PIL with octreotide in patients with HS. We report two children with HS and PLE in which we used octreotide to decrease intestinal protein loss. In one patient, octreotide increased serum albumin to an acceptable level without further need for albumin infusions. The other patient responded more dramatically with near normal serum albumin levels and cessation of albumin infusions. In achieving a good response to octreotide in both patients, we add to the publications supporting the use of octreotide in PIL and suggest that octreotide should be tried in patients with PIL secondary to HS. To the best of our knowledge, this is the first case report on the use of octreotide in HS-associated PIL.展开更多
Besides their possible effects on the development of inflammatory bowel disease(IBD),some environmental factors can modulate the clinical course of both ulcerative colitis(UC) and Crohn's disease(CD).This review i...Besides their possible effects on the development of inflammatory bowel disease(IBD),some environmental factors can modulate the clinical course of both ulcerative colitis(UC) and Crohn's disease(CD).This review is mainly devoted to describing the current knowledge of the impact of some of these factors on the outcome of IBD,with special emphasis on smoking and diet.Although the impact of smoking on the susceptibility to develop CD and UC is firmly established,its influence on the clinical course of both diseases is still debatable.In CD,active smoking is a risk factor for postoperative recurrence.Beyond this clinical setting,smoking cessation seems to be advantageous in those CD patients who were smokers at disease diagnosis,while smoking resumption may be of benefit in ex-smokers with resistant UC.The role of dietary habits on the development of IBD is far from being well established.Also,food intolerances are very frequent,but usually inconsistent among IBD patients,and therefore no general dietary recommendations can be made in these patients.In general,IBD patients should eat a diet as varied as possible.Regarding the possible therapeutic role of some dietary components in IBD,lessons should be drawn from the investigation of the primary therapeutic effect of enteral nutrition in CD.Low-fat diets seem to be particularly useful.Also,some lipid sources,such as olive oil,medium-chain triglycerides,and perhaps omega-3 fatty acids,might have a therapeutic effect.Fermentable fiber may have a role in preventing relapses in inactive UC.展开更多
文摘A number of disorders have been described to cause protein losing enteropathy (PLE) in children. Primary intestinal lymphangiectasia (PIL) is one mechanism leading to PLE. Few syndromes are associated with PIL; Hennekam syndrome (HS) is one of them. The principal treatment for PIL is a high protein, low fat diet with medium chain triglycerides supplementation. Supportive therapy includes albumin infusion. Few publications have supported the use of octreotide to diminish protein loss and minimize hypoalbuminemia seen in PIL. There are no publications on the treatment of PIL with octreotide in patients with HS. We report two children with HS and PLE in which we used octreotide to decrease intestinal protein loss. In one patient, octreotide increased serum albumin to an acceptable level without further need for albumin infusions. The other patient responded more dramatically with near normal serum albumin levels and cessation of albumin infusions. In achieving a good response to octreotide in both patients, we add to the publications supporting the use of octreotide in PIL and suggest that octreotide should be tried in patients with PIL secondary to HS. To the best of our knowledge, this is the first case report on the use of octreotide in HS-associated PIL.
文摘Besides their possible effects on the development of inflammatory bowel disease(IBD),some environmental factors can modulate the clinical course of both ulcerative colitis(UC) and Crohn's disease(CD).This review is mainly devoted to describing the current knowledge of the impact of some of these factors on the outcome of IBD,with special emphasis on smoking and diet.Although the impact of smoking on the susceptibility to develop CD and UC is firmly established,its influence on the clinical course of both diseases is still debatable.In CD,active smoking is a risk factor for postoperative recurrence.Beyond this clinical setting,smoking cessation seems to be advantageous in those CD patients who were smokers at disease diagnosis,while smoking resumption may be of benefit in ex-smokers with resistant UC.The role of dietary habits on the development of IBD is far from being well established.Also,food intolerances are very frequent,but usually inconsistent among IBD patients,and therefore no general dietary recommendations can be made in these patients.In general,IBD patients should eat a diet as varied as possible.Regarding the possible therapeutic role of some dietary components in IBD,lessons should be drawn from the investigation of the primary therapeutic effect of enteral nutrition in CD.Low-fat diets seem to be particularly useful.Also,some lipid sources,such as olive oil,medium-chain triglycerides,and perhaps omega-3 fatty acids,might have a therapeutic effect.Fermentable fiber may have a role in preventing relapses in inactive UC.
