QUALITY MANAGEMENT OF MEDICAL CARE FOR CHILDREN WITH PHENYLKETONURIA
https://doi.org/10.21508/1027-4065-2018-63-5-217-221
Abstract
The article discusses modern data on medical care for children with phenylketonuria. There are the directives and standards for neonatal screening, primary and specialized medical care for patients with phenylketonuria. The authors consider medical and social model of providing specialized nutrition and the indicators of medical care quality for children with phenylketonuria.
About the Authors
G. R. Kletenkova
Children’s Republican Clinical Hospital of Tatarstan Republic.
Russian Federation
Russian Federation
S. Ya. Volgina
Kazan State Medical University.
Russian Federation
Russian Federation
S. Sh. Yafarova
City Children’s Hospital No. 1.
Russian Federation
Russian Federation
References
1. Williams R.А., Mamotte D.S., Burnett J.R. Phenylketonuria: An Inborn Error of Phenylalanine Metabolism. Clin Biochem Rev 2008; 29(1): 31–41.
2. Hanley W.B. Phenylketonuria ( PKU) – What Next? Mini-Review. J Genet Disor Genet Rep 2013; 2(2): 2–6. DOI:10.4172/2327-5790.1000108
3. Medical laboratorydiagnostics. A. Karpischenko ( ed.). Moscow: GEHOTАRMedia 2014. http://www.studentlibrary.ru/book/ISBN9785970429587.html. (in Russ)
4. Diane B.P. Appendix 5. The History of newborn phenylketonuria screening in the US. https://biotech.law.lsu.edu/research/fed/tfgt/appendix5.htm.
5. van Wegberg A.M.J., MacDonald A., Ahring K., Bélanger- Quintana A., Blau N., Bosch A.M., Burlina A. et al. The complete European guidelines on phenylketonuria: diagnosis and treatment. Orphanet J Rare Dis 2017; 12(1): 162. DOI: 10.1186/s13023-017-0685-2
6. Bushueva T.V., Vinyarskaya I.V., Chernikov V.V. Evaluation of the life quality of children with phenylketonuria. Vestnik Rossiyskoy akademii meditsinskikh nauk 2014; 11/12: 39–45. (in Russ)
7. Netrebenko O.K. Postnatal programming: protein intake in infant alimentation. Pediatria 2015; 1: 113–120. (in Russ)
8. Cochrane B.K. Low protein staple foods, nutritional status and disease management in children and adults with phenylketonuria. Glasgow Theses Service, 2014; 174. http://theses.gla.ac.uk/5724/
9. Bushueva T.V., Borovik T.E., Ladodo K.S. Anthropometric development evaluation of children with classic phenylketonuria. Vopr pitaniya 2015; 84(2): 34–43. (in Russ)
10. Coakley K.E., Felner E.I., Tangpricha V., Wilson P.W.F., Singh R.H. Impact of dietary intake on bone turnover in patients with phenylalanine hydroxylase deficiency. JIMD Rep 2017; 36: 67–77. DOI: 10.1007/8904_2016_39
11. van Spronsen F.J., Bélanger-Quintana A., Blau N., Bosch A.M. Key European guidelines for the diagnosis and management of patients with phenylketonuria. Lancet Diabetes Endocrinol 2017; 1: 1–14. DOI: 10.1016/S2213-8587(16)30320-5
12. Bureninina S.M. Coping behavior of mothers raising children with phenylketonuria. Nauchnye trudy Moskovskogo gumanitarnogo universiteta 2017; 1: 46–52. (in Russ)
Review
For citations:
Kletenkova G.R., Volgina S.Ya., Yafarova S.Sh. QUALITY MANAGEMENT OF MEDICAL CARE FOR CHILDREN WITH PHENYLKETONURIA. Rossiyskiy Vestnik Perinatologii i Pediatrii (Russian Bulletin of Perinatology and Pediatrics). 2018;63(5):217-221. (In Russ.) https://doi.org/10.21508/1027-4065-2018-63-5-217-221
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