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AUTONOMIC DYSFUNCTION - A MECHANISM FOR RENAL FAILURE?

Familial dysautonomia, a rare autosomal recessive disorder, is characterized by a complex clinical symptomatology related to widespread autonomic and sensory dysfunction. We have previously reported renal failure due to intra-renal vascular and glomerular sclerosis in one dysautonomic; in all, 9 patients whom we have followed up to 18 years of age or more have become uremic. In the present study, renal hemodynamcis were found to be consistently reduced in a group of dysautonomics who were studied at random.Among the 13 patients, aged 7 to 38 years, all had either persistent or episodic hypertension typical of dysautonomia. Glomerular filtration rates (CIN) ranged from 107 down to 20 ml/min/1.73m2 ([xmacr ] = 70). Renal plasma flows (CPAH) were reduced to levels ranging from 560 to 72 ml/min/1.73m2 ([xmacr ] = 350). Filtration fractions averaged 21%. Reductions in GFR and RPF increased with age. Decreased levels of PRA and aldosterone were found in 8 of 12 patients.We have documented depressed renal hemodynamics and progression to uremia in familial dysautonomia. Intermittent hypertension and failure of autoregulation resulting from renal denervation could expose the kidney to vascular and glomerular damage due to hydraulic stress. Dysautonomia may represent a model in man which depicts the key of hemodynamic factors in the causation of glomerular injury.