Prevalence and severity of renal disease in familial dysautonomia

Lior Elkayam, Albert Matalon, Chi-Hong Tseng, Felicia Axelrod
American Journal of Kidney Diseases 2006, 48 (5): 780-6

BACKGROUND: One of the less well-defined complications of familial dysautonomia (FD) is chronic kidney disease (CKD). The goal of this report is to better define the prevalence and severity of kidney disease in this population and identify associated risk factors.

METHODS: We conducted a retrospective analysis of the database of the Dysautonomia Treatment and Evaluation Center at New York University School of Medicine for patients with FD who were seen at ages 15, 20, 25, 30, 35, and 40 years. Estimated glomerular filtration rate (GFR) was compared with that of the general population. Changes in mean blood pressure from supine to erect at ages 15 and 20 years were analyzed for patients who eventually required dialysis therapy and compared with those of the other patients with FD. Percentage of patients requiring dialysis and duration of treatment also were analyzed.

RESULTS: Mean estimated GFR of each predefined age group was considerably less than that of the general population starting at age 15 years (P < 0.001). Patients with FD were more likely to develop stage 3, 4, or 5 CKD than the general population. Of patients who remained alive at age 25 years, 19% eventually required dialysis. Those who required dialysis therapy were less likely to have had a feeding gastrostomy tube placed (P < 0.001) and had much more pronounced postural changes in blood pressure (P < 0.0001) by age 15 years. For those requiring dialysis therapy, average duration of treatment was 9 months.

CONCLUSION: Patients with FD are far more likely than the general population to develop CKD. Patients with FD who eventually required dialysis showed a greater degree of orthostatic hypotension and were significantly less likely to have had a feeding gastrostomy tube placed for hydration before the age of 15 years. Dialysis therapy is not well tolerated in this population.

Full Text Links

Find Full Text Links for this Article


You are not logged in. Sign Up or Log In to join the discussion.

Related Papers

Remove bar
Read by QxMD icon Read

Save your favorite articles in one place with a free QxMD account.


Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"

We want to hear from doctors like you!

Take a second to answer a survey question.