evaluation of plasma selenium level, glutathione peroxidase activity and thyroid functions in...

Click here to load reader

Post on 23-Dec-2015

216 views

Category:

Documents

2 download

Embed Size (px)

TRANSCRIPT

  • Slide 1
  • Slide 2
  • Slide 3
  • 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
  • Slide 4
  • Phenylketonuria Autosomal Recessive Unless Treated, Irreversible MR Newborn Screening Autosomal Recessive Unless Treated, Irreversible MR Newborn Screening
  • Slide 5
  • 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
  • Slide 6
  • Natural Antioxidants Enzymes Superoxide Dismutase Catalase Glutathione Peroxidase Vitamins and Trace-elements Water-Soluble: Vitamin C Fat-Soluble: Vitamin A and E Selenium
  • Slide 7
  • Oxidative Stress The Imbalance between ROS Natural Antioxidants The Imbalance between ROS Natural Antioxidants
  • Slide 8
  • Glutathione Peroxidase (GPX) GPX Gene on Chromosome 3 GPX is a Selenoprotein GSSG GSHH2O2H2O2 H2OH2O GPX NADPH+H + NADP + PPP
  • Slide 9
  • Selenium (Se) Essential Micronutrient RDA and RNI Selenoproteins Functions Antioxidant Formation of Active T3 Immune SystemBlood Clotting GPX, Iodothyronine Deiodinase,
  • Slide 10
  • 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
  • Slide 11
  • 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
  • Slide 12
  • Age Distribution
  • Slide 13
  • Sex Distribution
  • Slide 14
  • Consanguinity among PKU
  • Slide 15
  • Family History among PKU
  • Slide 16
  • GPX (U/L) of PKU Vs Controls * * Significant
  • Slide 17
  • Se (g/dl) of PKU Vs Controls ** ** Highly Significant
  • Slide 18
  • Mental Retardation among PKU
  • Slide 19
  • Age at the time of Diagnosis of PKU years
  • Slide 20
  • The phe (mg/dl) Vs IQ among PKU
  • Slide 21
  • GPX (U/L) Vs IQ among PKU
  • Slide 22
  • 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
  • Slide 23
  • 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)
  • Slide 24
  • Thank You Ramadan Kareeeem Amr S. Moustafa