title: Does Alport syndrome affect the basement membrane of peritoneal vessels?
abstract: Alport syndrome and encapsulating peritoneal sclerosis (EPS) are both rare diseases. Their joint occurrence is highly unlikely. Two patients at our center with Alport syndrome developed EPS. We therefore hypothesized that Alport syndrome might predispose to the development of EPS and that this predisposition might be reflected in a fast peritoneal transport rate at baseline. We compared the mass transfer area coefficient (MTAC) of creatinine and the clearances of albumin, immunoglobulin G, and alpha2-macroglobulin at baseline and for all subsequent available measurements in four patient groups: EPS patients with Alport syndrome, EPS patients without Alport syndrome, Alport patients without EPS, and long-term peritoneal dialysis (PD) patients without EPS. The transport characteristics were obtained during a standard peritoneal permeability analysis. Between July 1995 and December 2008, 5 of 417 PD patients treated at our center had Alport syndrome as their primary kidney disease, and 13 of the 417 developed EPS. Of those 13 EPS patients, 2 had Alport syndrome. We observed no differences in the baseline transport characteristics of the four groups under consideration. Taking all measures of transport characteristics into account, only the MTAC of creatinine was higher in the two EPS groups than in the other two groups (p = 0.01). We could not confirm our hypothesis that Alport syndrome affects peritoneal solute clearances.
authors.affiliation: Department of Medicine, Division of Nephrology, Academic Medical Center, University of Amsterdam, Netherlands. d.e.sampimon@amc.uva.nl
authors.forename: Denise E
authors.initials: DE
authors.lastname: Sampimon
authors.forename: Anniek
authors.initials: A
authors.lastname: Vlijm
authors.forename: Dirk G
authors.initials: DG
authors.lastname: Struijk
authors.forename: Raymond T
authors.initials: RT
authors.lastname: Krediet
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000418:Albumins / Q000378:metabolism; D001485:Basement Membrane / Q000378:metabolism; D001692:Biological Transport; D002199:Capillary Permeability; D002648:Child; D003404:Creatinine / Q000378:metabolism*; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000378:metabolism; D008297:Male; D008875:Middle Aged; D009394:Nephritis, Hereditary / Q000150:complications / Q000378:metabolism* / Q000628:therapy; D010530:Peritoneal Dialysis; D056627:Peritoneal Fibrosis / Q000209:etiology; D010537:Peritoneum / Q000098:blood supply* / Q000378:metabolism; D055815:Young Adult; D000511:alpha-Macroglobulins / Q000378:metabolism
chemical_list: D000418:Albumins; D007074:Immunoglobulin G; D000511:alpha-Macroglobulins; D003404:Creatinine