FD patients on dialysis have greater postural BP changes when young than those who do not


Chronic kidney disease (CKD) is more likely to develop in patients with familial dysautonomia (FD) than the general population, data suggest.

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Albert Matalon, MD, and his colleagues at the Dysautonomia Treatment and EvaluationCenter at New York University School of Medicine, studied 596 patients with FD, examining data collected at various patient ages. Stage 3, 4, or 5 CKD developed in 39% of FD patients by age 20, researchers reported in the American Journal of Kidney Diseases (2006;48:780-786).


This rose to 50% by age 25 and 76% by age 35. Of 106 patients who were alive at age 25, 20 (19%) eventually required dialysis. The national average for dialysis treatment in the United States is approximately 0.1%.


The mean estimated glomerular filtration rate (GFR) decreased to significantly less than would be expected for each age group. For example, among 15-year-olds, the eGFR was 110 mL/min in the FD group, which is below the expected 131 mL/min.


At age 40, eGFR was 39 mL/min in the FD group compared with the expected 105 mL/min. FD patients who eventually required dialysis had much more pronounced postural BP changes at age 15 than FD patients who did not require dialysis. Additionally, the FD patients who required dialysis were less likely to have had a feeding gastrostomy tube placed by age 15.