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  • Evaluation of Plasma Selenium Level, Glutathione Peroxidase Activity and Thyroid Functions in Children with Phenylketonuria Evaluation of Plasma Selenium Level, Glutathione Peroxidase Activity and Thyroid Functions in Children with Phenylketonuria *Rabah M. Shawky, *Eman A. Zaki, **Amr S. Moustafa Pediatric* and Biochemistry** Departments, Ain Shams Medical School, Cairo, Egypt *Rabah M. Shawky, *Eman A. Zaki, **Amr S. Moustafa Pediatric* and Biochemistry** Departments, Ain Shams Medical School, Cairo, Egypt
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  • Phenylketonuria Autosomal Recessive Unless Treated, Irreversible MR Newborn Screening Autosomal Recessive Unless Treated, Irreversible MR Newborn Screening
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  • Reactive Oxygen Species Singlet Oxygen ( 1 O 2 ) Superoxide (O 2 ) Hydroxyl (OH ) Peroxyl (ROO ) Alkoxyl (RO ) Hydrogen Peroxide (H 2 O 2 ) Free Radical Non Free Radical
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  • Natural Antioxidants Enzymes Superoxide Dismutase Catalase Glutathione Peroxidase Vitamins and Trace-elements Water-Soluble: Vitamin C Fat-Soluble: Vitamin A and E Selenium
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  • Oxidative Stress The Imbalance between ROS Natural Antioxidants The Imbalance between ROS Natural Antioxidants
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  • Glutathione Peroxidase (GPX) GPX Gene on Chromosome 3 GPX is a Selenoprotein GSSG GSHH2O2H2O2 H2OH2O GPX NADPH+H + NADP + PPP
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  • Selenium (Se) Essential Micronutrient RDA and RNI Selenoproteins Functions Antioxidant Formation of Active T3 Immune SystemBlood Clotting GPX, Iodothyronine Deiodinase,
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  • Aim of the Work Evaluation of the Changes in Plasma Se Level, GPX Activity and Thyroid Function Tests in PKU Patients This Would Allow Better Follow-up and Careful Adjusting of Dietary Management Protocols for PKU Patients
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  • Work Plan Measurement of Plasma Se Level by Atomic Absorption Measurement of Whole Blood GPX Activity by Spectrophotometry T3, T4, and TSH Levels by Electrochemiluminesnce Immunoassay phe Level by HPLC
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  • Age Distribution
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  • Sex Distribution
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  • Consanguinity among PKU
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  • Family History among PKU
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  • GPX (U/L) of PKU Vs Controls * * Significant
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  • Se (g/dl) of PKU Vs Controls ** ** Highly Significant
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  • Mental Retardation among PKU
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  • Age at the time of Diagnosis of PKU years
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  • The phe (mg/dl) Vs IQ among PKU
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  • GPX (U/L) Vs IQ among PKU
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  • Conclusions Decreased Se level among PKU may be due to dietary restriction PKU is a state of oxidative stress that may be associated with the depletion of natural antioxidants Instead, Se deficiency may be a part of the pathology of PKU
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  • Follow-up of PKU with thyroid function tests is recommended with severe Se deficiency Dietary supplementation with Se is recommended for PKU to allow for The proper functions of selenoproteins Conclusions (Contd)
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  • Thank You Ramadan Kareeeem Amr S. Moustafa