A DIALYSIS-ENCEPHALOPATHY(DE)-LIKE SYNDROME IN CHILDHOOD: AN INTERNATIONAL SURVEY

Abstract

Recent epidemiologic data suggest that DE may affect as many as 5.3% of adults receiving maintenance dialysis. To determine the magnitude and geographic distribution of this problem in childhood a questionnaire survey of 96 pediatric end-stage renal disease (ESRD)centers was conducted. 14/61 responding centers (23.0%) caring for 728 ESRD patients responded affirmatively to the questionnaire. 24 patients(3. 3%) were reported to have developed unexplained encephalopathy: the geographic distribution of cases was worldwide. 73.9% of patients had congenital renal disease. Personality changes, dementia and regression of developmental milestones were noted in patients from all 14 centers. Seizures were noted in patients from 9/14 centers and an abnormal EEG in those from 10/13. Speech disturbances were seen in cases from 11/14 centers. 13/13 reported patients were receiving aluminum-containing. compounds prior to or at the time of appearance of symptoms. Secondary hyperparathyroidism affected patients from 12/13 centers. Children from 9/13 centers had been dialyzed prior to appearance of neurologic symptoms. Symptoms occurred in 9 children who had not been dialyzed. A syndrome of unexplained progressive neurologic deterioration occurs in children with ESRD and appears to be clinically indistinguishable from that of adult DE. The implications of the above findings are discussed.

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