Cisterna chyli in autosomal dominant polycystic kidney disease

Abstract

Enlarged cisterna chyli is highly prevalent in ADPKD patients but not in age and gender-matched controls.

Evaluation of cisterna chyli diameter with MRI in patients with chronic kidney disease

“…Ito et al found the transverse CC diameter was 4.97 ± 1.87 mm in the group that had portal hypertension and 3.37 ± 1.25 mm in the group that did not have portal hypertension with MRI . In their series involving patients with and without autosomal dominant polycystic kidney disease (including 70 patients in each group), Thimmappa et al found median CC diameter value was 6.1 mm in the patient group and 3.4 mm in the control group with MRI . In our study, while CC diameter measured in the control group was similar to the values reported in previous studies.…”

“…A prior study has shown increased CC diameter in autosomal dominant polycystic kidney disease, using T 2 W MRI sections . Although the study group was not comprised of patients with CKD in that study, they found a negative correlation between GFR value and CC diameter, as in our study.…”

“…There are several studies that investigated the relationship of an enlarged CC with various pathologies like portal hypertension, uncompensated cirrhosis, and autosomal dominant polycystic kidney disease . The aim of this study was to detect the possible increase in CC diameter in patients with CKD compared to the normal population; examine the relationship between glomerular filtration rate (GFR) and CC diameter; and determine whether increased CC diameter can be used as a diagnostic marker in MRI.…”

MRI in autosomal dominant polycystic kidney disease

“…Cisterna chyli was larger in 70 ADPKD patients compared with age-and gender-matched controls without ADPKD (6.1 mm vs. 3.4 mm P < 0.0001) as measured by abdominal MRI. 72 Cisterna chyli diameter directly correlated with TKV, raising the possibility that increased lymphatic drainage of transudate or exudate from renal and extrarenal tissues caused engorgement and, consequently, larger T 2 visualization of cisterna chyli ( Fig. 9).…”

Endothelial-epithelial communication in polycystic kidney disease: Role of vascular endothelial growth factor signalling

“…An attractive prospect is that non-renal vascular abnormalities observed in patients with PKD may result from polycystin loss in the endothelium. Examples of this include impaired flowmediated dilatation in peripheral arteries of children and young adults or normotensive patients with ADPKD compared with healthy controls [76,77], the association between the mutation position in PKD1 and cerebrovascular aneurysms [78] and lymphatic malformations reported in both ADPKD and ARPKD patients [79,80]. Targeted deletion in animal models in vivo, complemented by organ-on a chip technologies in vitro, may help to tease out whether these clinical observations occur as a consequence of mutations of genes such as PKD1 in the endothelium, indirectly through PKD1 loss in vascular smooth muscle [81] or as yet unidentified non-cell-autonomous mechanisms.…”