Choroba Waldmanna jako możliwa przyczyna obrzęków u dzieci - opis przypadku

Waldmann's disease (primary intestinal lymphangiectasia) is a rare disorder of the lymphatic system, in which there are dilated lymphatic vessels, leading to leakage of lymph and protein leakage into the lumen of the smali intestine. It is characterized by lymphopenia, hypogammaglobulinaemia hypoalbuminemia. The main symptom is the swelling of the Iower limbs, but there may be generalized edema, pleural effusion, peritonitis, pericarditis. Protein-losing enteropathy usually leads to malabsorption. Herę we describe a case of 13-year-old boy with asymmetrical swelling of the extremities sińce birth, with no other symptoms, in whom the disease was suspected on the base of typical symptoms of Waldmann disease, additional work-up and a positive farmily history for intestinal lymphangiectasia, despite the lack of histopathological verification.