Open AccessRenal Transplantation in Familial Dysautonomia
Author(s) -
Yelena Rekhtman,
Andrew S. Bomback,
Martin A. Nash,
Scott Cohen,
Albert Matalon,
Dominique M. Jan,
Horacio Kaufmann,
Felicia B. Axelrod,
Jai Radhakrishnan,
Gerald B. Appel
Publication year - 2010
Publication title -
clinical journal of the american society of nephrology
Language(s) - Uncategorized
Resource type - Journals
SCImago Journal Rank - 2.755
H-Index - 151
eISSN - 1555-905X
pISSN - 1555-9041
DOI - 10.2215/cjn.01750210
Subject(s) - medicine , familial dysautonomia , transplantation , supine position , hemodialysis , dialysis , dysautonomia , kidney transplantation , orthostatic vital signs , kidney disease , end stage renal disease , disease , surgery , intensive care medicine , blood pressure
Chronic kidney disease (CKD) is an increasingly recognized complication of familial dysautonomia (FD), a neurodevelopmental disorder with protean systemic manifestations that are the result of sensory and autonomic dysfunction. Progressive renal dysfunction occurs due to chronic volume depletion and cardiovascular lability with supine hypertension and orthostatic hypotension. By age 25, nearly one-half of all patients with FD will have reached stage 3 CKD. Furthermore, dialysis for ESRD in FD patients is associated with multiple complications and poor outcomes.