title: Octreotide in Hennekam syndrome-associated intestinal lymphangiectasia.
abstract: 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.
authors.affiliation: Child Health Department, Sultan Qaboos University Hospital, Muscat 123, Sultanate of Oman. siham_ss@hotmail.com
authors.forename: Siham
authors.initials: S
authors.lastname: Al Sinani
authors.forename: Yusria Al
authors.initials: YA
authors.lastname: Rawahi
authors.forename: Hamed
authors.initials: H
authors.lastname: Abdoon
mesh_terms: D019465:Craniofacial Abnormalities / Q000175:diagnosis / Q000188:drug therapy* / Q000235:genetics; D020022:Genetic Predisposition to Disease; D005832:Genital Diseases, Male / Q000175:diagnosis / Q000188:drug therapy* / Q000235:genetics; D040941:Heredity; D006801:Humans; D034141:Hypoalbuminemia / Q000209:etiology / Q000517:prevention & control; D007231:Infant, Newborn; D008201:Lymphangiectasis, Intestinal / Q000175:diagnosis / Q000188:drug therapy* / Q000235:genetics; D008209:Lymphedema / Q000175:diagnosis / Q000188:drug therapy* / Q000235:genetics; D008297:Male; D015282:Octreotide / Q000627:therapeutic use*; D010375:Pedigree; D010641:Phenotype; D011504:Protein-Losing Enteropathies / Q000209:etiology / Q000517:prevention & control; D016896:Treatment Outcome
chemical_list: D015282:Octreotide