Literature DB >> 8828623

Towards self-monitoring and self-treatment in phenylketonuria--a way to better diet compliance.

U Wendel1, U Langenbeck.   

Abstract

It has been a long established principle in the treatment of diabetes that the patient or his/her family is responsible for day-to-day monitoring of metabolic control. It is believed that this concept should also now be applied in phenylketonuria. At present, self-monitoring of blood phenylalanine still requires assaying the phenylalanine concentration in capillary blood obtained by finger-stick sampling at home, via mailing to a nearby laboratory. Frequently and rapidly obtained data can guide the patient to adjust dietary phenylalanine intake, provided he and his family have been informed in detail about the disease and trained in practical diet competence. Teaching programmes for patients are to be promoted. A home-monitoring device for blood phenylalanine is at the development stage.

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Year:  1996        PMID: 8828623     DOI: 10.1007/pl00014224

Source DB:  PubMed          Journal:  Eur J Pediatr        ISSN: 0340-6199            Impact factor:   3.183


  8 in total

1.  Development and validation of a robust specific enzyme mediated assay for phenylalanine in serum.

Authors:  R S Campbell; G M Brearley; H Varsani; H C Morris; T P Milligan; S K Hall; P M Hammond; C P Price
Journal:  Clin Chim Acta       Date:  1992-09-30       Impact factor: 3.786

2.  Phenylalanine: application of a simple HPLC technique to its measurement in dried blood spots.

Authors:  S J Standing; R P Taylor
Journal:  Ann Clin Biochem       Date:  1992-11       Impact factor: 2.057

3.  Monitoring of phenylketonuria: a colorimetric method for the determination of plasma phenylalanine using L-phenylalanine dehydrogenase.

Authors:  U Wendel; W Hummel; U Langenbeck
Journal:  Anal Biochem       Date:  1989-07       Impact factor: 3.365

4.  Rapid automated enzymatic measurement of phenylalanine in plasma and blood spots.

Authors:  R P Taylor; I C Smith; S J Standing
Journal:  Clin Chim Acta       Date:  1993-09-30       Impact factor: 3.786

5.  Measurement of gonadotropins in dried blood spots.

Authors:  C M Worthman; J F Stallings
Journal:  Clin Chem       Date:  1994-03       Impact factor: 8.327

6.  Phenylalanine levels in PKU following minor surgery.

Authors:  A E Fiedler; M J Miller; H Bickel; S D Cederbaum
Journal:  Am J Med Genet       Date:  1982-04

7.  Psychological and social findings in adolescents with phenylketonuria.

Authors:  J Weglage; B Fünders; B Wilken; D Schubert; E Schmidt; P Burgard; K Ullrich
Journal:  Eur J Pediatr       Date:  1992-07       Impact factor: 3.183

Review 8.  Hyperphenylalaninemia: diagnosis and classification of the various types of phenylalanine hydroxylase deficiency in childhood.

Authors:  F Güttler
Journal:  Acta Paediatr Scand Suppl       Date:  1980
  8 in total
  10 in total

1.  Living with phenylketonuria: perspectives of patients and their families.

Authors:  C Bilginsoy; N Waitzman; C O Leonard; S L Ernst
Journal:  J Inherit Metab Dis       Date:  2005       Impact factor: 4.982

2.  Development of Metabolic Phenotype in Phenylketonuria: Evaluation of the Blaskovics Protein Loading Test at 5 Years of Age.

Authors:  P Burgard; E Mönch; J Zschocke; U Wendel; U Langenbeck
Journal:  JIMD Rep       Date:  2015-12-19

3.  Predicting the phenylalanine blood concentration from urine analyses. An approach to noninvasive monitoring of patients with phenylketonuria.

Authors:  U Langenbeck; F Baum; A Mench-Hoinowski; H Luthe; A W Behbehani
Journal:  J Inherit Metab Dis       Date:  2005       Impact factor: 4.982

4.  Elevated plasma phenylalanine concentrations may adversely affect bone status of phenylketonuric mice.

Authors:  S Yannicelli; D M Medeiros
Journal:  J Inherit Metab Dis       Date:  2002-09       Impact factor: 4.982

5.  Modelling the phenylalanine blood level response during treatment of phenylketonuria.

Authors:  U Langenbeck; J Zschocke; U Wendel; V Hönig
Journal:  J Inherit Metab Dis       Date:  2001-12       Impact factor: 4.982

6.  Phenylalanine Monitoring via Aptamer-Field-Effect Transistor Sensors.

Authors:  Kevin M Cheung; Kyung-Ae Yang; Nako Nakatsuka; Chuanzhen Zhao; Mao Ye; Michael E Jung; Hongyan Yang; Paul S Weiss; Milan N Stojanović; Anne M Andrews
Journal:  ACS Sens       Date:  2019-11-01       Impact factor: 7.711

7.  Dry storage of multiple reagent types within a paper microfluidic device for phenylalanine monitoring.

Authors:  Lael Wentland; Rachel Polaski; Elain Fu
Journal:  Anal Methods       Date:  2021-01-19       Impact factor: 2.896

8.  "MY PKU": increasing self-management in patients with phenylketonuria. A randomized controlled trial.

Authors:  Amber E Ten Hoedt; Carla Em Hollak; Carolien Ca Boelen; N Ada P van der Herberg-van de Wetering; Nienke M Ter Horst; Cora F Jonkers; Frits A Wijburg; Annet M Bosch
Journal:  Orphanet J Rare Dis       Date:  2011-06-27       Impact factor: 4.123

9.  Development of a Whole Blood Paper-Based Device for Phenylalanine Detection in the Context of PKU Therapy Monitoring.

Authors:  Robert Robinson; Liam Wong; Raymond J Monnat; Elain Fu
Journal:  Micromachines (Basel)       Date:  2016-02-15       Impact factor: 2.891

10.  Requirements for a minimum standard of care for phenylketonuria: the patients' perspective.

Authors:  Tobias S Hagedorn; Paul van Berkel; Gregor Hammerschmidt; Markéta Lhotáková; Rosalia Pasqual Saludes
Journal:  Orphanet J Rare Dis       Date:  2013-12-17       Impact factor: 4.123

  10 in total

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