Phenylketonuria: Current dietary treatment practices in the United States and Canada

Robert O. Fisch, Reuben Matalon, Sanford Weisberg, Kimberlee Michals

Research output: Contribution to journalArticle

28 Citations (Scopus)

Abstract

Objective: A survey of treatment centers for phenylketonuria (PKU) in the United States and Canada was undertaken regarding current practices of dietary treatment of PKU. Methods: A total of 111 centers, who follow more than 6,950 patients with PKU responded to the survey. Results: The majority of the centers, 87%, favor life long dietary control of phenylalanine intake. The survey found lack of uniformity regarding acceptable range of blood phenylalanine levels. The frequency of clinic visits varied and became less frequent as patients got older. Although most of the clinics recommend diet for life, only one third of the clinics follow patients beyond the age of 18 years, therefore, it is unclear who manages these patients beyond that age. The survey also showed a high number of families with children who were reported for medical neglect (3.0% compared to <0.06% in the general population). Because of dietary noncompliance, 1% of the children were removed from the home. Discussion: The survey points to the common treatment goal of diet for life for patients with PKU and underscores the need for uniform guidelines for achieving this goal.

Original languageEnglish (US)
Pages (from-to)147-151
Number of pages5
JournalJournal of the American College of Nutrition
Volume16
Issue number2
StatePublished - Apr 1997
Externally publishedYes

Fingerprint

phenylketonuria
Phenylketonurias
Canada
Phenylalanine
phenylalanine
Diet
Therapeutics
Ambulatory Care
diet
Surveys and Questionnaires
Guidelines
blood
Population

Keywords

  • diet treatment
  • maternal phenylketonuria
  • phenylalanine
  • phenylketonuria (PKU)

ASJC Scopus subject areas

  • Medicine (miscellaneous)
  • Food Science

Cite this

Phenylketonuria : Current dietary treatment practices in the United States and Canada. / Fisch, Robert O.; Matalon, Reuben; Weisberg, Sanford; Michals, Kimberlee.

In: Journal of the American College of Nutrition, Vol. 16, No. 2, 04.1997, p. 147-151.

Research output: Contribution to journalArticle

Fisch, Robert O. ; Matalon, Reuben ; Weisberg, Sanford ; Michals, Kimberlee. / Phenylketonuria : Current dietary treatment practices in the United States and Canada. In: Journal of the American College of Nutrition. 1997 ; Vol. 16, No. 2. pp. 147-151.
@article{2bcd5ecfd6674aea9ed3e68a7a5a08fd,
title = "Phenylketonuria: Current dietary treatment practices in the United States and Canada",
abstract = "Objective: A survey of treatment centers for phenylketonuria (PKU) in the United States and Canada was undertaken regarding current practices of dietary treatment of PKU. Methods: A total of 111 centers, who follow more than 6,950 patients with PKU responded to the survey. Results: The majority of the centers, 87{\%}, favor life long dietary control of phenylalanine intake. The survey found lack of uniformity regarding acceptable range of blood phenylalanine levels. The frequency of clinic visits varied and became less frequent as patients got older. Although most of the clinics recommend diet for life, only one third of the clinics follow patients beyond the age of 18 years, therefore, it is unclear who manages these patients beyond that age. The survey also showed a high number of families with children who were reported for medical neglect (3.0{\%} compared to <0.06{\%} in the general population). Because of dietary noncompliance, 1{\%} of the children were removed from the home. Discussion: The survey points to the common treatment goal of diet for life for patients with PKU and underscores the need for uniform guidelines for achieving this goal.",
keywords = "diet treatment, maternal phenylketonuria, phenylalanine, phenylketonuria (PKU)",
author = "Fisch, {Robert O.} and Reuben Matalon and Sanford Weisberg and Kimberlee Michals",
year = "1997",
month = "4",
language = "English (US)",
volume = "16",
pages = "147--151",
journal = "Journal of the American College of Nutrition",
issn = "0731-5724",
publisher = "American College Of Nutrition",
number = "2",

}

TY - JOUR

T1 - Phenylketonuria

T2 - Current dietary treatment practices in the United States and Canada

AU - Fisch, Robert O.

AU - Matalon, Reuben

AU - Weisberg, Sanford

AU - Michals, Kimberlee

PY - 1997/4

Y1 - 1997/4

N2 - Objective: A survey of treatment centers for phenylketonuria (PKU) in the United States and Canada was undertaken regarding current practices of dietary treatment of PKU. Methods: A total of 111 centers, who follow more than 6,950 patients with PKU responded to the survey. Results: The majority of the centers, 87%, favor life long dietary control of phenylalanine intake. The survey found lack of uniformity regarding acceptable range of blood phenylalanine levels. The frequency of clinic visits varied and became less frequent as patients got older. Although most of the clinics recommend diet for life, only one third of the clinics follow patients beyond the age of 18 years, therefore, it is unclear who manages these patients beyond that age. The survey also showed a high number of families with children who were reported for medical neglect (3.0% compared to <0.06% in the general population). Because of dietary noncompliance, 1% of the children were removed from the home. Discussion: The survey points to the common treatment goal of diet for life for patients with PKU and underscores the need for uniform guidelines for achieving this goal.

AB - Objective: A survey of treatment centers for phenylketonuria (PKU) in the United States and Canada was undertaken regarding current practices of dietary treatment of PKU. Methods: A total of 111 centers, who follow more than 6,950 patients with PKU responded to the survey. Results: The majority of the centers, 87%, favor life long dietary control of phenylalanine intake. The survey found lack of uniformity regarding acceptable range of blood phenylalanine levels. The frequency of clinic visits varied and became less frequent as patients got older. Although most of the clinics recommend diet for life, only one third of the clinics follow patients beyond the age of 18 years, therefore, it is unclear who manages these patients beyond that age. The survey also showed a high number of families with children who were reported for medical neglect (3.0% compared to <0.06% in the general population). Because of dietary noncompliance, 1% of the children were removed from the home. Discussion: The survey points to the common treatment goal of diet for life for patients with PKU and underscores the need for uniform guidelines for achieving this goal.

KW - diet treatment

KW - maternal phenylketonuria

KW - phenylalanine

KW - phenylketonuria (PKU)

UR - http://www.scopus.com/inward/record.url?scp=0030955068&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0030955068&partnerID=8YFLogxK

M3 - Article

C2 - 9100215

AN - SCOPUS:0030955068

VL - 16

SP - 147

EP - 151

JO - Journal of the American College of Nutrition

JF - Journal of the American College of Nutrition

SN - 0731-5724

IS - 2

ER -