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DIT proteins/fats

lactulose sugary syrup broken down in gut to create an acidic env which creates NH4 and traps it and other ammonium for gut ion excression
histamine precursor histidine
porphyrin, heme precursor glycine
NO precursor arginine
GABA precursor glutamate
SAM precursor methionine
creatine precursor arginine
RLE for urea cycle CPS I
RLE for hexose monophosphate pathway G6PD
RLE for fatty acid synthesis Acetyl CoA carboxylase
RLE for beta oxidation of fatty acids CAT1
ketone body synthesis HMG CoA synthase
cholesterol synthesis HMG CoA reductiase
bile acid synthesis 7 alpha hydroxylase
Heme synthesis aminolevulinate synthase
What deficiency causes familial hypercholesterolemia LDL receptors
dietary modifications in a pt with PKU low phenylalanine, increased tyrosine, replace THF
full term neonate becomes MR and hyperactive and has a musty odor PKU
2 yo girl has increased abd girth, FTT and skin/hair depigmentation kwashiorkor
a mddle aged man has dark spots on his sclera and his urine turns black upon sitting alkaptonuria
genetic disease where tx includes protein restrictino to prevent MR, ketoacidosis and death maple syrup urine disease
18yo female has moderate generalized abd pain, normal WBC and no fever. she has paresthesias i her lower extremities. acute intermittant porphyria
45 yo male alcoholic with blistering lesions in sun exposed areas especially the dorsum of the hands. he has face hypertrichosis porphyria cutanea tarda
tx for homocystinuria decreased methionine, increased cystine, increased B6, B12, and folate
Created by: kayjames