Octreotide in Hennekam syndrome-associated intestinal lymphangiectasia

摘要: A number of disorders have been described to cause protein losing enteropathy (PLE) in children. Primary intestinal lymphangiectasia (PIL) is one mechanism leading PLE. Few syndromes are associated with PIL; Hennekam syndrome (HS) them. The principal treatment for PIL a high protein, low fat diet medium chain triglycerides supplementation. Supportive therapy includes albumin infusion. publications supported the use octreotide diminish loss and minimize hypoalbuminemia seen PIL. There no on patients HS. We report two children HS PLE which we used decrease loss. In patient, increased serum an acceptable level without further need infusions. other patient responded more dramatically near normal levels cessation achieving good response both patients, add supporting suggest that should be tried secondary To best our knowledge, this first case HS-associated