Published in:
01-07-2018 | Scientific Letter
Protein Losing Enteropathy in Hennekam Syndrome
Authors:
Phani Priya Mandula, Rohan Malik, Gaurav Khanna
Published in:
Indian Journal of Pediatrics
|
Issue 7/2018
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Excerpt
To the Editor: Primary intestinal lymphangiectasia (PIL) is a malformation of the intestinal lymphatics. Few genetic syndromes are associated with intestinal lymphangiectasia: Von Recklinghausen, Turner, Noonan, Klippel-Trenaunay and Hennekam syndrome [
1]. A 6-y-old girl presented to us with watery diarrhea and anasarca for two months. She had globally delayed developmental milestones and intellectual disability with history of carpo-pedal spasms and recurrent episodes of erysipelas. On examination there was hypertelorism, flat nasal bridge, gingival hyperplasia, small ears and mouth. Anasarca with erysipelas involving both lower limbs was also observed. …
