Computed tomographic scan in the diagnosis of bilateral renal lymphangiectasia.

Computed tomographic scan in the diagnosis of bilateral renal lymphangiectasia.
Saudi J Kidney Dis Transpl. 2008 Nov

Rastogi R, Rastogi V.
Yash Diagnostic Center, Yash Hospital and Research Center, Civil Lines, Kanth Road, Moradabad (UP), India. eesharastogi@gmail.com.

Renal lymphangiectasia is a rarely reported disorder of lymphatic malformation. Although benign, it may lead to hypertension and renal failure in undetected or undiagnosed cases. Adult polycystic kidney disease is a close mimic. We herewith present a rare case of bilateral renal lymphangiectasia that was strongly suspected on Computed tomographic (CT) scan and confirmed by aspiration cytology. Awareness about this condition will result in early diagnosis, early treatment and reduced morbidity.

PMID: 18974588 [PubMed - in process]

Embryonic development and malformation of lymphatic vessels.

Embryonic development and malformation of lymphatic vessels.

Novartis Found Symp. 2007

Wilting J, Buttler K, Rössler J, Norgall S, Schweigerer L, Weich HA, Papoutsi M.
Department of Pediatrics 1, Georg-August-University, Goettingen, Germany.

In the human, malformations of lymphatic vessels can be observed as lymphangiectasia, lymphangioma and lymphangiomatosis, with a prevalence of 1.2-2.8 per thousand. Their aetiology is unknown and a causal therapy does not exist. We investigated the origin of lymphatic endothelial cells (LECs) in avian and murine embryos, and compared the molecular profile of LECs from normal and malformed lymphatics of children. In avian embryos, Prox1+ lymphangioblasts are located in the confluence of the cranial and caudal cardinal veins, where the jugular lymph sac (JLS) forms. Cell lineage studies show that the JLS is of venous origin. In contrast, the lymphatics of the dermis are derived from mesenchymal lymphangioblasts located in the dermatomes, suggesting a dual origin of LECs in avian embryos. The same may hold true for murine embryos, where Lyve1+ LEC precursors are found in the cardinal veins, and in the mesenchyme. The mesenchymal cells express the pan-leukocyte marker CD45, indicating a cell type with lymphendothelial and leukocyte characteristics. In the human, such cells might give rise to Kaposi's sarcoma. Microarray analyses of LECs from lymphangiomas of children show a large number of regulated genes, such as VEGFR3. Our studies show that lymphvasculogenesis and lymphangiogenesis occur simultaneously in the embryo, and suggest a function for VEGFR3 in lymphangiomas.

PMID: 18300425 [PubMed - in process]