Examination of the quality of life and the effect of COVID-19 pandemic on metabolic control in Hungarian paediatric PKU population
Becsei Dóra
Clinical Medicine
Dr. Reusz György
Gyermekgyógyászati Klinika, Bókay utcai részleg, Tanterem
2025-02-20 14:00:00
Prevention of Chronic Diseases in Childhood
Dr. Szabó Attila
Dr. Reusz György, Dr. Zsidegh Petra
Dr. Szücs Nikolette
Dr. Rácz Gábor
Dr. Purebl György
Dr. Serfőző Csilla
Dr. Kósa Magdolna
Assessing the quality of life of patients is receiving an increasing attention. We were the
first to assess the quality of life of Hungarian children with PKU and, to our knowledge,
we were the first in the world to use the MAPI PKU-specific questionnaire to compare
the quality of life of children with good and poor adherence.
We establish that our patients enjoyed a good quality of life, as 80% of the domains
assessed had little or no or impact on their quality of life. The most important factor in
the of quality-of-life domains examined was consumption of diet food. Moderate effects
were achieved by emotional impact and food enjoyment. Patients with classic PKU had
a poorer quality of life than those with HPA, and poor metabolic compliance was
associated with a poorer QoL. The most commonly reported symptom was irritability.
Based on the recognised effects, special attention should be paid to the emotional life of
people with PKU.
We were also among the first to examine the impact of restrictions caused by the
COVID-19 pandemic on the metabolic status of PKU patients.
We observed that the pandemic caused by COVID-19 did not delay the diagnosis and
initiation of treatment of babies born with PKU during this period in Hungary.
Our study showed that the median Phe levels of children and adolescents with PKU
increased during the first year of the COVID-19 pandemic, but overall remained within
the reference range. In the year of the pandemic, eight per cent more dried blood drop
samples were sent compared to the previous year. During the period of the pandemic,
Phe levels in children and adolescents under 12 years of age shifted in different
directions: children had the highest levels during the restrictive period, whereas
adolescents had the highest levels during the summer period without restrictions.
Factors influencing metabolic status (e.g. financial situation, exercise, change in eating
habits, snacking, eating out of boredom, changes in daily rhythm) may have played a
different role in the two age groups.
We observed a significant negative relationship between the number of samples sent
and the median Phe level, supporting the knowledge that a decreasing number of
samples should be given special attention.
