Watch Vitamin Levels in Shwachman-Diamond Syndrome

According to a recent small retrospective study in Pancreas (May 2015 – Volume 44 – Issue 4 – p 590–595) with 21 children, there were high rates of vitamin deficiencies (particularly vitamin A) and selenium deficiency.

Nutritional Status in Children with Schwachman-Diamond Syndrome

From abstract:

Results: Twenty patients (95%) had pancreatic insufficiency receiving PERT, 10 (47%) had a combined vitamin and trace element deficiency, 6 (29%) had an isolated vitamin deficiency, and 4 (19%) had an isolated trace element deficiency. Vitamins A and E deficiency occurred in 16 (76%) and 4 (19%) of 21, respectively. Low serum selenium was found in 10 (47%), zinc deficiency in 7 (33%), and copper deficiency in 5 (24%). Eleven patients (52%) were on multivitamin supplementation, and 2 (10%) on zinc and selenium supplements. No statistical differences were found between repeated measurements for all micronutrients.

Conclusions: More than 50% of the children had vitamin A and selenium deficiencies despite adequate supplementation of PERT and supplements. Micronutrients should be routinely measured in SDS patients to prevent significant complications.

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