TY - JOUR
T1 - Octreotide in Hennekam syndrome-associated intestinal lymphangiectasia
AU - Al Sinani, Siham
AU - Al Rawahi, Yusria
AU - Abdoon, Hamed
PY - 2012
Y1 - 2012
N2 - 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 HSassociated PIL.
AB - 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 HSassociated PIL.
KW - Hennekam syndrome
KW - Lymphangiectasia
KW - Octreotide
KW - Protein losing enteropathy
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UR - http://www.scopus.com/inward/citedby.url?scp=84873915757&partnerID=8YFLogxK
U2 - 10.3748/wjg.v18.i43.6333
DO - 10.3748/wjg.v18.i43.6333
M3 - Article
C2 - 23180957
AN - SCOPUS:84873915757
SN - 1007-9327
VL - 18
SP - 6333
EP - 6337
JO - World Journal of Gastroenterology
JF - World Journal of Gastroenterology
IS - 43
ER -