نتایج جستجو برای: early treated phenylketonuria etpku

تعداد نتایج: 1110023  

Journal: :British medical journal 1967
J B Stephenson M S McBean

Most local health authorities in Great Britain have used the Phenistix test on wet napkins or diapers (napkin-Phenistix test) to screen all young infants for phenylketonuria (Centerwall et al., 1963). This test is a sensitive and reliable method of detecting phenylpyruvic aciduria (Gibbs and Woolf, 1959; Woolf, 1963), which is an early sign of true phenylketonuria (Allen et al., 1964; Berry and...

Journal: :Przeglad lekarski 2011
Mirosław Bik-Multanowski Jacek J Pietrzyk

Phenylketonuria is the most common inborn error of metabolism. Adult patients often discontinue dietary treatment and can subsequently develop serious brain dysfunction. Some of them, however, do not present any symptoms, despite long-term exposition to high blood phenylalanine concentration. As the extent of brain toxicity of hyperphenylalaninemia is not clear in adults, new diagnostic methods...

Journal: :Egyptian Journal of Health Care 2023

Phenylketonuria (PKU) is an autosomal recessive inborn error of phenylalanine metabolism resulting from deficiency hydroxylase (PAH).

Journal: :Journal of abnormal psychology 1968
C J Friedman Sibinga MSMS I M Steisel H M Sinnamon

2 groups of children with phenylketonuria, those with a history of early sensory restriction or isolation and those without, were compared on measures of (a) interpersonal behavior, (b) language comprehension, (c) intelligence, and (d) incidence and severity of personality disturbances. The findings, discussed in the context of sensory-deprivation and sensory-restriction research, supported the...

Journal: :iranian journal of child neurology 0
s. salehpour md,assistant professor, pediatric endocrinologist, mofid children’s hospital, shahid beheshti medical university d. babaie md, resident, department ofpediatrics, mofid children’s hospital, shahid beheshti medical university

objective too much restriction of dietary proteins can cause severe protein malnutrition,which can occur in adjusting the diet for some kinds of aminoacidopathies, urea cycle disorder and organic academia. this report presents the case of a 1.5-year-old boy with history of phenylketonuria with a three weeks history of erythematous scaly plaques and edema of his extremities; he had a history of ...

Journal: :Diabetes 2014
Deimante Simaite Julia Kofent Maolian Gong Franz Rüschendorf Shiqi Jia Pamela Arn Kristi Bentler Carolyn Ellaway Peter Kühnen Georg F Hoffmann Nenad Blau Francesca M Spagnoli Norbert Hübner Klemens Raile

Mutations in several genes cause nonautoimmune diabetes, but numerous patients still have unclear genetic defects, hampering our understanding of the development of the disease and preventing pathogenesis-oriented treatment. We used whole-genome sequencing with linkage analysis to study a consanguineous family with early-onset antibody-negative diabetes and identified a novel deletion in PCBD1 ...

Journal: :Journal of Rafsanjan University of Medical Sciences 2023

Investigating the Factors Related to Psychological Well-Being of Parents with Phenylketonuria Children: A Systematic Review

Journal: :Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease 2000

Journal: :Neuropsychology 2012
Alicia L Janos Dorothy K Grange Robert D Steiner Desirée A White

OBJECTIVE Phenylketonuria (PKU) is a hereditary metabolic disorder that often results in neuropsychological impairment, even in individuals treated early and continuously. This study was conducted to examine processing speed, variability in processing speed, and the relationship between processing speed variables and executive abilities in children with early and continuously treated PKU. MET...

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