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G401(P) Low vitamin d levels in children with ataxia-telangiectasia
  1. AP Prayle1,2,
  2. A Bush4,5,
  3. M Suri3,
  4. J Bhatt1
  1. Nottingham Children’s Hospital, Nottingham University Hospitals NHS Trust, Nottingham, UK
  2. Child Health, The University of Nottingham, Nottingham, UK
  3. Department of Clinical Genetics, Nottingham University Hospitals NHS Trust, Nottingham, UK
  4. National Heart and Lung Institute, Imperial College London, London, UK
  5. Department of Paediatric Respiratory Medicine, Royal Brompton Hospital, London, UK

Abstract

Aims Ataxia telangiectasia (A-T; birth incidence 1/300,000) is characterised by progressive neurological features, increased sensitivity to radiation and malignancy risk, and progressive respiratory failure. Most children are wheelchair users in the teenage years. Low Vitamin D is important in many immune pathways, is a key factor in osteomalacia, and is implicated in the pathogenesis of multiple respiratory disorders.

Vitamin D deficiency occurs due to low intake or poor sunlight exposure. Children with A-T show high rates of malnutrition despite nutritional support, and may avoid sunlight due to malignancy risk. We assessed the vitamin D levels of a cohort of children with A-T.

Methods 25-Hydroxy-Vitamin D (25OH-VitD) has been measured routinely since May 2015 in our clinic. We use the RCPCH position statement definitions of deficient 25OH-VitD as less than 25 nmol/L, sub-optimal as 25 to less than 50 nmol/L and normal as 50 nmol/L and over. To examine the impact of nutritional status on 25OH-VitD, we calculated the weight Z score for each of these categories, and we compared the proportion wasted (Z score < 2), and a 3-sample test for equality of proportions (prop.test in R 3.3).

Results Measurements were made in 46 children (see table). We restricted our analysis to the first measurement for each patient. 28 were female. 12 (26%) measurements of 25OH-VitD were in the deficient range, 22 (48%) were suboptimal and 12 (26%) were normal. There was no clear impact of wasting (weight Z score < 2) on vitamin D (p=0.4).

Abstract G401(P) Table 1

Conclusion Vitamin D deficiency was extremely common in this cohort of patients. The lack of a clear association of low vitamin D with weight Z score suggests that factors other than malnutrition for example lack of sun exposure may be a factor. We have instituted a programme of screening and supplementation of vitamin D. Together with immobility, Vitamin D deficiency increases the risk of osteoporosis in A-T.

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