Nutritional status in children with Shwachman-diamond syndrome.
Pancreas
; 44(4): 590-5, 2015 May.
Article
em En
| MEDLINE
| ID: mdl-25742431
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.
Texto completo:
1
Base de dados:
MEDLINE
Assunto principal:
Insuficiência Pancreática Exócrina
/
Doenças da Medula Óssea
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Estado Nutricional
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Micronutrientes
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Lipomatose
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Síndromes de Malabsorção
Tipo de estudo:
Diagnostic_studies
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Etiology_studies
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Observational_studies
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Risk_factors_studies
Idioma:
En
Ano de publicação:
2015
Tipo de documento:
Article