A 43-year old @entity1 came to our clinic with chronic @entity294 . Determination of the faecal @entity6927 -antitrypsin-clearance led to the diagnosis of exsudative @entity7017 . Blood counts showed pronounced @entity2905 . Histopathological findings from intestinal and colorectal biopsies showed @entity38336 . Following exclusion of secondary types, our diagnosis was primary @entity9676 . Additional distinctive morphological and anamnestic features strongly suggested presence of @entity12697 . Characteristic manifestations of @entity12697 include changes in the lymphatic vessels in accordance with @entity63276 . Frequently, these changes are localized in the lungs. To date, only rare cases of @entity9676 in @entity12697 have been reported. Treatment consisted of a protein-rich diet, with reduced fat content enriched by middle-chain @entity1718 , as well as twice-daily injections of 200 micrograms octreotide. With this therapy, the symptoms improved.

[ @entity294 in a 43-year-old XXXX ].

@entity1 :: ('9606', 'Species') :: ['patient'] @entity1718 :: ('MESH:D005227', 'Chemical') :: ['fatty acids'] @entity2905 :: ('MESH:D008231', 'Disease') :: ['lymphocytopenia'] @entity294 :: ('MESH:D003967', 'Disease') :: ['diarrhea'] @entity63276 :: ('MESH:C536567', 'Disease') :: ['primary lymphangiectasis'] @entity38336 :: ('MESH:D008200', 'Disease') :: ['diffuse lymphangiectasis'] @entity7017 :: ('MESH:C538273', 'Disease') :: ['enteropathy'] @entity9676 :: ('MESH:D008201', 'Disease') :: ['intestinal lymphangiectasia', 'intestinal lymphangiectasis'] @entity12697 :: ('MESH:D009634', 'Disease') :: ["Noonan's syndrome"] @entity6927 :: ('146', 'Gene') :: ['alpha 1']
@entity1 :: (9606,Species) :: ['patient']
