Olfactory and gustatory perception in Brazilian PKU patients: A cross‐sectional study

Author:

Tonon Tássia1,Martinez Chenia1,Nalin Tatiele2,Poloni Soraia3,Piltcher Otávio Bejzman4,Maillot François5,Franceschetto Bianca Fasolo1,Schwartz Ida Vanessa D.167ORCID

Affiliation:

1. Graduate Program in Medicine: Medical Sciences Universidade Federal do Rio Grande do Sul Porto Alegre Brazil

2. Ultragenyx Brasil Farmacêutica Ltda São Paulo Brazil

3. Nutrition and Dietetics Service Hospital de Clínicas de Porto Alegre Porto Alegre Brazil

4. Department of Ophthalmology and Otolaryngology Universidade Federal do Rio Grande do Sul Porto Alegre Brazil

5. Internal Medicine Department, Reference Center for Inherited Disease University of Tours Tours France

6. Medical Genetics Service Hospital de Clínicas de Porto Alegre Porto Alegre Brazil

7. Department of Genetics Universidade Federal do Rio Grande do Sul Porto Alegre Brazil

Abstract

AbstractPatients with phenylketonuria (PKU) have a highly restrictive diet, which involves restriction of phenylalanine (Phe) intake and daily use of Phe‐free metabolic formula. However, little is known about the potential impact of this diet on chemical senses. The present study aimed to evaluate the olfactory and gustatory perceptions of patients with PKU. A cross‐sectional controlled study which included patients with PKU on dietary treatment and healthy controls was performed. Olfactory perception was assessed using the 12‐item Sniffin’ Sticks test, and taste perception using the Taste Strips test. Twenty‐five patients (mean age 19.3 ± 4.7 years; 13 females) and 25 controls (mean age 19.9 ± 4.9 years, p = 0.676; 13 females) were included. The mean age at treatment onset was 52.8 ± 29.7 days. The mean scores for olfactory and gustatory perceptions, and for bitter and salty flavors, were lower in patients than in controls (p = 0.039, p = 0.004, p = 0.008, and p = 0.020, respectively). Among patients, Phe levels at diagnosis correlated negatively with bitter taste (r = −0.493, p = 0.006). The lower olfaction and gustation scores found in patients may be related to understimulation caused by the highly restrictive PKU diet and the deprivation of flavors from breast milk.

Funder

Hospital de Clínicas de Porto Alegre

Publisher

Wiley

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