Improved neurological outcome in children with chronic renal disease from infancy

Abstract

Progressive encephalopathy, developmental delay, microcephaly, electroencephalogram (EEG) and computed tomographic (CT) scan abnormalities have been reported in 80% of children with chronic renal failure (CRF) in infancy. Malnutrition, aluminium intoxication and psychosocial deprivation are proposed as causes. In 15 children with CRF from infancy we evaluated the effect of no aluminium salts and early vigorous nutritional and psychosocial support, in addition to the standard therapy, on neurological development. Six patients underwent dialysis (2 at birth) and 3 received transplants. None of our patients were given aluminium therapy. The nutritional status of the patients in the first 2 years of life was assessed with the waterlow classification. At the end of the follow-up period (mean 50 months range 14-148 months), patients underwent neurodevelopmental assessment, head CT scan, EEG, nerve conduction velocity (NCV) and auditory brain stem evoked response (ABER). None of our patients developed progressive encephalopathy or recurrent seizures. All have a normal neurological examination apart from hypotonia. Microcephaly was present in 5 patients. There was a good correlation between malnutrition in the first 2 years of life and microcephaly. Developmental delay was present in 3 patients; all 3 were microcephalic. There was evidence of brain atrophy on CT scan in only 3 patients. EEG was abnormal in 6 patients, but only severe in 1 patient. Only 1 patient had diminished NCV; all patients had a normal ABER. We conclude that a policy of no oral aluminium therapy and early nutritional support leads to better neurological outcome in children with CRF from infancy.

Outcome after renal transplantation. Part I: Intellectual and motor performance

“…General muscular hypotonia has also been observed in nine, four, and two children with chronic kidney disease, on dialysis, and after RTPL, respectively [6]. Motor performance assessed by the Bayley Scales of Infant Development was normal in 76% of children transplanted at an age < 5 years, and similar to our own findings, neurological comorbidity was the most negative predictor [9].…”

“…patient and graft survival, has steadily improved over the last decades [23,24]. However, information on comprehensive and detailed neurodevelopmental outcome after RTPL is still limited [1][2][3][4][5][6][7][8][9]. This cross-sectional study presents detailed measures of IP and MP in children after RTPL.…”

“…intellectual performance (IP) and motor performance (MP), in children with chronic kidney disease [1][2][3][4][5][6] and in particular after renal transplantation (RTPL) [2,[7][8][9][10] are still limited. Published studies have focussed either on IP [1][2][3][4][5]7] or on MP [6,8]. and only a few studies have assessed both [9,10].…”

Intellectual and motor performance, quality of life and psychosocial adjustment in children with cystinosis

“…Few other studies have examined motor performance in this population. General muscular hypotonia has been a consistent finding in many children with chronic kidney diseases of various origins [40]. Muscular hypotonia can be associated with poorer motor perfor- mance, in particular with static balance tasks.…”

Systematic Review of Structural and Functional Neuroimaging Findings in Children and Adults with CKD

“…Compared with their age-matched controls, the renal patients had significantly enlarged ventricles, with the greatest enlargement noted in the HD group. Using a small sample of pediatric patients who developed CKD at birth, Elzouki and colleagues (47) found that approximately 23% showed cerebral atrophy. Enlarged ventricles, a correlate of brain atrophy, have also been reported by investigators studying primarily pediatric samples; estimates were significantly higher and ranged from 59% (44) to 67% (43).…”