Phenylketonuria: Treatment in adolescence and adult life

被引：12

作者：

Brenton, DP ^{[1
]}

Tarn, AC ^{[1
]}

CabreraAbreu, JC ^{[1
]}

Lilburn, M ^{[1
]}

机构：

[1] UCL HOSP, DEPT CHEM PATHOL, LONDON, ENGLAND

来源：

EUROPEAN JOURNAL OF PEDIATRICS | 1996年 / 155卷

关键词：

phenylketonuria; adult dietary treatment;

D O I：

10.1007/PL00014261

中图分类号：

R72 [儿科学];

学科分类号：

100202 ;

摘要：

In our clinic the decision on whether to continue with dietary treatment of phenylketonuria or not is left to each adolescent and adult patient after the advantages and disadvantages, as discussed in this paper, of continuing diet have been presented to them. As a result 61 of 132 patients have stopped diet or declined to restart and only 4 of them have phenylalanine values below 1000 mu mol/l. Seventy-one patients have remained on diet or started again with phenylalanine values below 1000 mu mol/l in 58 of them. This series of 132 excludes women who returned to diet to conceive.

引用

收藏

页码：S93 / S96

页数：4

相关论文

共 23 条

[1] NEUROLOGICAL DETERIORATION IN YOUNG-ADULTS WITH PHENYLKETONURIA [J].

ALLEN, RJ .

LANCET, 1990, 336 (8720) :949-949

[2] INTELLECTUAL-DEVELOPMENT IN 12-YEAR-OLD CHILDREN TREATED FOR PHENYLKETONURIA [J].

AZEN, CG ;

KOCH, R ;

FRIEDMAN, EG ;

BERLOW, S ;

COLDWELL, J ;

KRAUSE, W ;

MATALON, R ;

MCCABE, E ;

OFLYNN, M ;

PETERSON, R ;

ROUSE, B ;

SCOTT, CR ;

SIGMAN, B ;

VALLE, D ;

WARNER, R .

AMERICAN JOURNAL OF DISEASES OF CHILDREN, 1991, 145 (01) :35-39

[3]

BEASLEY MG, 1994, Q J MED, V87, P155

[4] VALINE, ISOLEUCINE, AND LEUCINE - A NEW TREATMENT FOR PHENYLKETONURIA [J].

BERRY, HK ;

BRUNNER, RL ;

HUNT, MM ;

WHITE, PP .

AMERICAN JOURNAL OF DISEASES OF CHILDREN, 1990, 144 (05) :539-543

[5] WHITE-MATTER ABNORMALITIES IN PATIENTS WITH TREATED HYPERPHENYLALANINEMIA - MAGNETIC-RESONANCE RELAXOMETRY AND PROTON SPECTROSCOPY FINDINGS [J].

BICK, U ;

ULLRICH, K ;

STOBER, U ;

MOLLER, H ;

SCHUIERER, G ;

LUDOLPH, AC ;

OBERWITTLER, C ;

WEGLAGE, J ;

WENDEL, U .

EUROPEAN JOURNAL OF PEDIATRICS, 1993, 152 (12) :1012-1020

[6] MAGNETIC-RESONANCE-IMAGING OF THE BRAIN IN PHENYLKETONURIA [J].

CLEARY, MA ;

WALTER, JH ;

WRAITH, JE ;

JENKINS, JPR ;

ALANI, SM ;

TYLER, K ;

WHITTLE, D .

LANCET, 1994, 344 (8915) :87-90

[7] DIETARY-PROBLEMS OF PHENYLKETONURIA - EFFECT ON CNS TRANSMITTERS AND THEIR POSSIBLE ROLE IN BEHAVIOR AND NEUROPSYCHOLOGICAL FUNCTION [J].

GUTTLER, F ;

LOU, H .

JOURNAL OF INHERITED METABOLIC DISEASE, 1986, 9 :169-177

[8] VITAMIN-B(12) DEFICIENCY IN ADOLESCENTS AND YOUNG-ADULTS WITH PHENYLKETONURIA [J].

HANLEY, WB ;

FEIGENBAUM, A ;

CLARKE, JTR ;

SCHOONHEYT, W ;

AUSTIN, V .

LANCET, 1993, 342 (8877) :997-997

[9] PRELIMINARY-REPORT ON THE EFFECTS OF DIET DISCONTINUATION IN PKU [J].

KOCH, R ;

AZEN, CG ;

FRIEDMAN, EG ;

WILLIAMSON, ML .

JOURNAL OF PEDIATRICS, 1982, 100 (06) :870-875

[10] BIOCHEMICAL AND NEUROPSYCHOLOGICAL EFFECTS OF ELEVATED PLASMA PHENYLALANINE IN PATIENTS WITH TREATED PHENYLKETONURIA - A MODEL FOR THE STUDY OF PHENYLALANINE AND BRAIN-FUNCTION IN MAN [J].

KRAUSE, W ;

HALMINSKI, M ;

MCDONALD, L ;

DEMBURE, P ;

SALVO, R ;

FREIDES, D ;

ELSAS, L .

JOURNAL OF CLINICAL INVESTIGATION, 1985, 75 (01) :40-48