@article{lymph 4270, author = {H Heimpel, JR Bierich, JM Herrmann, H Meister, J Vollmar}, title = {DYSPLASIA OF THE LYMPHATICS WITH LYMPHOEDEMA, GENERALIZED LYMPHANGIECTASIS, CHYLOTHORAX AND "PSEUDO-STORAGE-DISEASE"}, volume = {12}, year = {1979}, url = {http://journals.librarypublishing.arizona.edu/lymph/article/id/4270/}, issue = {4}, abstract = {<p>A patient howing an unusual association of various abnormalities of the lymphatic vascular system is reported. These abnormalities became first evident in<br />early childhood and consisted of lymphoedema of the left leg, lymphangiectasis in various organs and occlusion of the thoracic duct at its entrance into the venous<br />angle. Chylous effusion and subcutaneous ehyloedema appeared in adolescence. Diagnostic biopsies of spleen, liver and bone-marrow revealed the presence<br />of multiple foamcells, suggesting the diagnoses of lipid storage disease. The patient died from severe honeycomb-lungs at the age of 20. This case cannot be attributed to one of the wellknown disease entities of the lymphatic system. The " pseudo"-storage disease I regarded as secondary to the backflow of chylus into the tissues.</p>}, month = {9}, pages = {228-240}, issn = {2522-7963}, publisher={International Society of Lymphology and the University of Arizona Libraries}, journal = {Lymphology} }