The morphology of the intestinal mucosa was reviewed using light microscopy and transmission electron microscopy from a two year-old male patient with protein losing primary intestinal lyphangiectasis. Diagnostic studies ruled out recognized forms of secondary intestinal lymphangiectasia (e.g cardiac, hepatic, renal diseases or lymphoreticular cancer). Furthermore, certain features distinguish this patient from the secondary type: decreased immunoglobulin and albumin levels, lymphocytopenia, chylous ascitis and delated lymphatics on small-bowel biopsy.
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