The Association of Therapy Adherence and Thyroid Function in Adult Patients with Phenylketonuria.
Adult
Case-Control Studies
Diet, Protein-Restricted
Female
Humans
Hungary
Iodine
/ deficiency
Male
Patient Compliance
Phenylalanine
/ blood
Phenylketonurias
/ diet therapy
Prospective Studies
Selenium
/ urine
Thyroglobulin
/ blood
Thyroid Gland
/ diagnostic imaging
Thyrotropin
/ blood
Thyroxine
/ blood
Young Adult
Dietary adherence
Iodine deficiency
Phenylketonuria
Thyroglobulin
Thyroid function
Journal
Annals of nutrition & metabolism
ISSN: 1421-9697
Titre abrégé: Ann Nutr Metab
Pays: Switzerland
ID NLM: 8105511
Informations de publication
Date de publication:
2019
2019
Historique:
received:
05
02
2019
accepted:
04
06
2019
pubmed:
27
6
2019
medline:
6
6
2020
entrez:
27
6
2019
Statut:
ppublish
Résumé
The standard, lifelong therapy of phenylketonuria (PKU) is a natural protein-restricted diet complemented with phenylalanine (Phe)-free L-amino acid mixtures that provide the daily necessary micronutrients. To assess thyroid function and structure and the iodine status of early-treated adult PKU (ETPKU) patients in Hungary. Sixty-nine PKU patients (aged 18-41 years) and 50 healthy controls were enrolled in the study. Thyroid hormones, serum thyroglobulin, thyroid antibodies, urinary iodine, and selenium concentrations were measured, and thyroid ultrasound was performed. The incidence of thyroid dysfunction was infrequent (n = 2). Blood Phe was negatively correlated with thyroid-stimulating hormone (TSH), and PKU patients had higher free thyroxine and lower TSH levels than healthy controls. Although optimal iodine status was found in the entire PKU population, by dividing the patients according to their therapy compliance, we observed that lower therapy adherence was associated with mild iodine deficiency and lower urinary selenium levels. The results of this study suggest that iodine status is strongly influenced by the adherence to therapy in ETPKU patients. No or not enough medical food consumption combined with a low-Phe diet can lead to subclinical iodine deficiency.
Sections du résumé
BACKGROUND
The standard, lifelong therapy of phenylketonuria (PKU) is a natural protein-restricted diet complemented with phenylalanine (Phe)-free L-amino acid mixtures that provide the daily necessary micronutrients.
OBJECTIVE
To assess thyroid function and structure and the iodine status of early-treated adult PKU (ETPKU) patients in Hungary.
METHODS
Sixty-nine PKU patients (aged 18-41 years) and 50 healthy controls were enrolled in the study. Thyroid hormones, serum thyroglobulin, thyroid antibodies, urinary iodine, and selenium concentrations were measured, and thyroid ultrasound was performed.
RESULTS
The incidence of thyroid dysfunction was infrequent (n = 2). Blood Phe was negatively correlated with thyroid-stimulating hormone (TSH), and PKU patients had higher free thyroxine and lower TSH levels than healthy controls. Although optimal iodine status was found in the entire PKU population, by dividing the patients according to their therapy compliance, we observed that lower therapy adherence was associated with mild iodine deficiency and lower urinary selenium levels.
CONCLUSIONS
The results of this study suggest that iodine status is strongly influenced by the adherence to therapy in ETPKU patients. No or not enough medical food consumption combined with a low-Phe diet can lead to subclinical iodine deficiency.
Identifiants
pubmed: 31242484
pii: 000501301
doi: 10.1159/000501301
doi:
Substances chimiques
Phenylalanine
47E5O17Y3R
Thyrotropin
9002-71-5
Thyroglobulin
9010-34-8
Iodine
9679TC07X4
Selenium
H6241UJ22B
Thyroxine
Q51BO43MG4
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
16-23Informations de copyright
© 2019 S. Karger AG, Basel.