Journal of Pediatrics Review
Journal of Pediatrics Review
J. Pediatr. Rev
Medical Sciences
http://jpr.mazums.ac.ir
1
admin
2322-4398
2322-4401
10.32598
en
jalali
1401
9
1
gregorian
2022
12
1
10
4
online
1
fulltext
en
Assessing Phenylalanine Blood Level in Children With Phenylketonuria in Southern Khorasan Province and Determining the Affecting Social and Demographic Factors
Endocrinology
Original Article
Original Article
<strong>Background</strong>: Phenylketonuria is a metabolic disorder resulting from a defect in phenylalanine metabolism with a global prevalence of 1 in 10000. Delayed initiation of dietary modification leads to brain injury and cognitive and behavioral problems. The main objective of this study was to assess the demographic and social factors affecting the metabolic control of patients having phenylketonuria in Southern Khorasan Province, Iran. <br>
<strong>Methods</strong>: In this cross-sectional descriptive-analytic study which was performed during the summer of 2019, a total of 32 out of 37 known children and adolescents having phenylketonuria in Southern Khorasan Province were assessed. The age and gender of patients, parents’ marital status, parents’ occupational status, parents’ educational level, the distance between home and phenylketonuria clinic, and the number of affected siblings having phenylketonuria were documented. We were not able to contact five patients having phenylketonuria in Southern Khorasan Province. Data were analyzed by SPSS software, version 16 using the Mann-Whitney U test and the Kruskal-Wallis test. The significance level was considered P<0.05.<br>
<strong>Results</strong>: Totally, 32 patients with a Mean±SD age of 6.6±4.7 years enrolled in this study among whom 23 were male (71.9%) and 9 patients were female (28.1%). The Mean±SD phenylalanine level in this study group was 8.1±5.2 mg/dL. The disease was optimally controlled in 14 patients (43.3%) and poorly controlled in 18 patients (56.3%). There was not any statistically significant relation between the metabolic control of the disease and any of the assessed social and demographic factors.<br>
<strong>Conclusion</strong>: The disease was properly controlled in 43.3% of the assessed population, and 56.3% had poor metabolic control. There was not any statistically significant relation between the metabolic control of patients having phenylketonuria in Southern Khorasan Province and the assessed demographic and social variables. As the number of known cases in South Khorasan province is limited, the small sample size could be one of the main limitations of our study.<br>
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Phenylketonuria, Phenylalanine level, Disease control, Demographic factors, Social factors
349
358
http://jpr.mazums.ac.ir/browse.php?a_code=A-10-1005-1&slc_lang=en&sid=1
Tayebeh
Chahkandi
na_chahkandi@yahoo.com
10031947532846009941
10031947532846009941
No
Department of Pediatrics, Medical Toxicology & Drug Abuse Research Center, School of Medicine, Birjand University of Medical Sciences, Birjand, Iran.
Bita
Bijari
bita.bijari@yahoo.com
10031947532846009942
10031947532846009942
No
Department of Community Medicine, Cardiovascular Diseases Research Center, School of Medicine, Birjand University of Medical Sciences, Birjand, Iran.
Niloofar
Hamidi Laeen
niloofarhamidi4@yahoo.com
10031947532846009943
10031947532846009943
Yes
School of Medicine, Birjand University of Medical Sciences, Birjand, Iran.
Sayeh
Shaban
sayeh.shaban@yahoo.com
10031947532846009944
10031947532846009944
No
School of Medicine, Birjand University of Medical Sciences, Birjand, Iran.
Emad
Asgari Jafarabadi
dr.askary@hotmail.com
10031947532846009945
10031947532846009945
No
Department of Pediatrics, School of Medicine, Birjand University of Medical Sciences, Birjand, Iran.