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TK Comert
G Koksal

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TK Comert
G Koksal

International Research Journal of Public and Environmental Health
Vol.4 (3),pp. 48-54, March, 2017
ISSN 2360-8803
Available online at https://www.journalissues.org/IRJPEH/
DOI:https://doi.org/10.15739/irjpeh.17.007
Article 17/ID/JPRH011/ 07 pages
Author(s) agree that this article remain permanently open access under the terms of the Creative Commons Attribution License 4.0 International License.



Original Research Article

Daily practices of phenylketonuria in various centres in Turkey “a retrospective, cross-sectional study”

Tugba Kucukkasap Comert*1 and Gulden Koksal2

1Yuksek Ihtisas University, Nutrition and Dietetics Department,Turkey.
2Hasan Kalyoncu University, Nutrition and Dietetics Department, Turkey.

*Corresponding Author Email:  tugbacomert(at)yiu.edu.tr



date Received: January 13, 2017     date Accepted: February 20, 2017     date Published: April 14, 2017


 Abstract

There is an large diversity in treatment aims and procedures in phenylketonuria (PKU)  through the pediatric nutrition and metabolic disease clinics in different regions of Turkey. A survey among professionals was done to determine goals and daily practice.A questionnaire was sent to professionals of the pediatric nutrition and metabolic disease clinics of different regions of Turkey, addressing diagnostic and treatment procedures, numbers of patients necessary for a PKU centre, quidelines followed, numbers of patients treated and professionals involved in care, target phenylalanine (Phe) concentrations, amount of protein prescribed, frequency of monitoring and clinical visits, need for follow-up of various clinical and biochemical data. The dates of diagnosis, blood Phe levels (last 2-3 years), last anthropometric measurements (body weight, height) were ascertained from medical dossiers and records.Eight clinics of six countries answered the questions of daily practices in PKU. Among the various clinics, differences in daily practice were observed with regards to blood Phe values (mg/dL) accepted for initiation of medical nutrition treatment, target blood Phe concentrations (mg/dL), the recommended protein intake and the dosages of protein substitutes, allocating daily Phe allowance, definition of foods that could be eaten without restriction (“free foods”).  Responses show that PKU care varies widely between centres, so it was confirmed that it is necessary to develop a guideline specific for Turkey that covers all practices beginning from the screening of newborns to follow-up.

 

Abbrevations

PKU: phenlketonuria
HPA: hyperphenylalaninemia
Phe: phenylalanine


Key words: Phenylketonuria, quidelines, daily practice, phenylalanine


Comert and Koksal