ESPN 51th Annual Meeting

ESPN 2018


 
Analysis of nutritional blood concentrations in children with nephropathic cystinosis
TRIONA JOYCE 1 FRANCES COURT BROWN 1 PERNILLE RASMUSSEN 1 CAROLINE BOOTH 1 JOANNA CLOTHIER 1

1- EVELINA LONDON CHILDRENS HOSPITAL, GUYS AND ST THOMAS NHS FOUNDATION TRUST
 
Introduction:

 Several medications used to treat nephropathic cystinosis have gastrointestinal side effects which could potentially impact upon food and nutrient intake and absorption.  There is little information on micronutrient status and supplementation needs in this patient group.  This study investigated the proportion of our paediatric cystinosis patients achieving normal blood concentrations for copper, zinc, selenium, folate and vitamins A, D, E and B12.  

Material and methods:

 Retrospective review of nutritional blood results between January 2016 and 2018.  

Results:

8 children, 6 white and 2 Asian ethnicity, 5 boys, average eGFR 31-90ml/min/1.73 m2, with median (IQ1, IQ3) age 8.79 (4.99, 11.76) years. One child was receiving an enteral feed and vitamin D, two receiving an oral nutritional supplement, two children receiving either a multivitamin (n=1) or vitamin D (n=1) when nutritional bloods were taken.   Mean folate, zinc and selenium blood concentrations were all within the normal ranges.  There was no evidence of vitamin B12 deficiency with the majority (n=7/8) above the normal range.  Vitamin E levels were either high (n=3/8) or normal.  Mean vitamin A concentrations were variable with low (n=1), normal (n=6) and high vitamin A (n=1) concentrations.  Deficiencies were seen for copper (n=1) and vitamin D (n=2).  Vitamin D concentrations were sufficient in 75%.  One child with both vitamin A and D deficiencies was not receiving any micronutrient supplements or enteral feeds.

Conclusions:

 A very small number of children had deficiencies for copper and vitamins A and D.  Unless a deficiency is suspected, annual monitoring of selenium, folate and vitamins B12 and E does not appear to be indicated.  As several children were receiving a supplementary source of micronutrients, further research is required to determine if micronutrient supplementation is required in the population.