Evaluating the Influence of Social Determinants of Health on Blood Phenylalanine Levels in Phenylketonuria Patients

被引:0
作者
Afseth, Cassandra [1 ]
Knutsen, Josh [2 ]
Lamborn, Thomas [2 ]
Vucko, Erika [1 ,2 ]
Havens, Kirsten [2 ]
Shim, Soo [2 ]
Baker, Joshua [1 ,2 ]
机构
[1] Northwestern Univ, Feinberg Sch Med, Chicago, IL 60611 USA
[2] Ann & Robert H Lurie Childrens Hosp Chicago, Div Genet, Chicago, IL 60611 USA
关键词
healthcare disparities; phenylketonuria; social determinants of health; social vulnerability index; VULNERABILITY; OUTCOMES; CHILDREN; IMPACT;
D O I
10.1002/ajmg.a.63885
中图分类号
Q3 [遗传学];
学科分类号
071007 ; 090102 ;
摘要
Phenylketonuria (PKU) is a genetic metabolic disorder that causes the accumulation of phenylalanine (Phe) in tissues, leading to intellectual disability, seizures, and socioemotional challenges. The role of social determinants of health (SDOH) in PKU management has not been formally studied, and this investigation evaluates the association between in-home and in-office factors on blood Phe levels in PKU patients. We conducted a retrospective chart review on over 200 patients attending the well-resourced PKU Clinic at Lurie Children's Hospital of Chicago. Data included patients' average Phe level, various demographic information, and CDC/ATSDR social vulnerability index (SVI) score. The analysis revealed no significant association between social vulnerability status and average Phe level. However, a significant correlation was found between sapropterin dihydrochloride use and average Phe level. Age interacted separately with sex assigned at birth, pegvaliase use, total Phe samples submitted, and the presence of genetic testing to significantly influence the average Phe level. This study highlights the multifactorial influences on PKU management and underscores the importance of social resources, such as clinic social workers and state-provided formula, in modulating the effects of SDOH on PKU control. Further research in different healthcare settings is needed to understand the social determinants affecting PKU patients comprehensively, which will strengthen advocacy efforts for this population.
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相关论文
共 23 条
[1]   Measuring Historic and Longitudinal Social Vulnerability in Disaster-Prone Communities: A Modification to the Centers for Disease Control and Prevention Social Vulnerability Index (CDC-SVI) [J].
Adepoju, Omolola E. ;
Kiaghadi, Amin .
DISASTER MEDICINE AND PUBLIC HEALTH PREPAREDNESS, 2023, 17
[2]  
[Anonymous], Social Determinants of Health-Healthy People 2030 | health.gov
[3]  
[Anonymous], 2023, SAS/STAT 15.3 User's Guide
[4]  
BioMarin Pharmaceutical Inc, PALYNZIQ PACKAGE INS
[5]  
CDC/ATSDR Social Vulnerability Index 2020 Database U.S, CDCATSDR SVI DATA DO
[6]   Addressing Social Determinants to Improve Patient Care and Promote Health Equity: An American College of Physicians Position Paper [J].
Daniel, Hilary ;
Bornstein, Sue S. ;
Kane, Gregory C. .
ANNALS OF INTERNAL MEDICINE, 2018, 168 (08) :577-+
[7]   The Impact of Social Determinants of Health on Children with Asthma [J].
Federico, Monica J. ;
McFarlane, Arthur E. ;
Szefler, Stanley J. ;
Abrams, Elissa M. .
JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY-IN PRACTICE, 2020, 8 (06) :1808-1814
[8]   Protein status of people with phenylketonuria: a scoping review protocol [J].
Firman, Sarah ;
Ramachandran, Radha ;
Whelan, Kevin ;
Witard, Oliver C. ;
O'Keeffe, Majella .
BMJ OPEN, 2021, 11 (09)
[9]   Living with Phenylketonuria: Lessons from the PKU community [J].
Ford, Suzanne ;
O'Driscoll, Mike ;
MacDonald, Anita .
MOLECULAR GENETICS AND METABOLISM REPORTS, 2018, 17 :57-63
[10]  
Freese KE, 2021, J HEALTH CARE POOR U, V32, P245, DOI 10.1353/hpu.2021.0022