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A rare case of primary intestinal lymphangiectasia refractory to treatment

 Department of Medical Education, Dubai Health Authority, Dubai, UAE

Correspondence Address:
Khadeeja Mohammed,
Medical Ducation Department, Dubai Health Authority, Rashid Hospital, Dubai
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/HMJ.HMJ_23_20

Primary intestinal lymphangiectasia (PIL), also known as Waldmann's disease, is a rare disorder, characterised by dilated lymphatics supplying the small intestinal wall resulting in lymph leakage into the small-bowel lumen. This protein-losing enteropathy leads to lymphopenia, hypoalbuminaemia and hypogammaglobinaemia. PIL is generally first diagnosed before 3 years of age, rarely in adulthood. Here, we present the case of a 13-year-old Emirati boy with protein-losing enteropathy secondary to intestinal lymphangiectasia refractory to the standard treatment. He was diagnosed initially at 18 months of age and presents with recurrent episodes of lower limb and scrotal swellings and diarrhoea. He requires 4–5 albumin and immunoglobulin infusions per year to control his symptoms. Most of the standard therapies tried so far failed to keep him from remissions. Moreover, he has a persistently positive helicobacter gastritis not controlled by aggressive eradication therapy. He is currently on low-fat diet and sirolimus drug therapy.

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