Enteropatia protido-disperdente: la linfangectasia intestinale

Protein-losing enteropathy is a rare condition characterised by protein loss through the intestines, which leads to hypoproteinemia. It may result from various causes, including primary intestinal lymphangiectasia, amyloidosis, rheumatic diseases, parasitic infections, or tumours. Primary intestinal lymphangiectasia is a congenital disease where lymph leaks into the intestines due to dilated lymphatic vessels. It usually appears within the first three years of life; however, even though it is rare and mostly sporadic, some genetic and syndrome associations were studied. Main symptoms include oedema, weight loss, intermittent diarrhoea, nausea and vomiting. Diagnosis is based on radiological, endoscopic and histological findings. Treatment involves nutritional support, medications (immunosuppressants, diuretics, antifibrinolytics) and, in some cases, surgery. This report presents a case of primary intestinal lymphangiectasia in a seven-year-old child.