720697
Descripción
Fichas por gina_evans0312, actualizado hace más de 1 año
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Creado por gina_evans0312
hace más de 10 años
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| Pregunta | Respuesta |
| Deficient Enzyme | Glucose-6-Phosphatase (Converts Glucose-6-Phosphate to Glucose + Phosphate) |
| Issue | G-6-Pase is the terminal step of gluconeogenesis & glycogenlysis, so blood glucose cannot be maintained between meals |
| Location of Issue | Liver, kidneys and SI (liver and kidneys is where gluconeogeneis occurs) |
| Main Result of Defect | Non-glucagon responding Hypoglucaemia |
| Symptoms | Enlarged liver and kidneys, convulsions, distress between meals |
| Problem- Excessive Glycolysis (Excess G-6-P uses this pathway) | Leads to lactoacidosis and acidosis of the blood |
| Problem- Body Compensates for Hypoglycaemia | Increased glucagon/adrenaline to mobilize FAs & TAGs, FAs converted to TAGs & VLDLs, which are deposited in the liver, cheeks and buttocks |
| Treatment (Babies) | Glucose through a tube |
| Treatment (Older Children and Adults) | Glucose drinks at 2-3hr intervals during the night |
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