Nutritional Status in Children With Shwachman-Diamond Syndrome. Issue 4 (May 2015)

• Record Type: Journal Article
• Authors: Pichler, Judith; Meyer, Rosan; Köglmeier, Jutta; Ancliff, Phil; Shah, Neil
• Is Part Of: Pancreas. Volume 44:Issue 4(2015)
• Publication Date: 2015-05
• Subjects: Shwachman-Diamond syndrome -- trace element and vitamin deficiencies -- children -- pancreatic insufficiency -- SDS - Shwachman-Diamond syndrome -- CF - cystic fibrosis -- PERT - pancreatic enzyme replacement therapy; Pancreas -- Diseases -- Periodicals; Pancreas -- Periodicals; Neuroendocrine tumors -- Periodicals; 616.37005
• DOI: 10.1097/MPA.0000000000000291
• Languages: English
• ISSNs: 0885-3177

Abstract

Abstract : Objective: In Shwachman-Diamond syndrome (SDS), pancreatic insufficiency can lead to malabsorption of fat-soluble vitamins and trace elements. The aim of this study was to assess the serum concentrations of vitamins A and E, zinc, copper, and selenium and their deficiencies. Methods: This retrospective review was performed in 21 children (12 were male; median age, 7.8 years) with genetically confirmed SDS at a tertiary pediatric hospital. Pancreatic enzyme replacement therapy (PERT) and vitamin or trace elements supplements were documented. 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.