Diet as Therapy Flashcards

1
Q

examples of diet as the specific treatment for rare metabolic disorders

A

*phenylketonuria = decreased phenylalanine
*maple syrup urine disease = decreased branch chain amino acids
*glycogen storage disease = (+) nocturnal starch
*hereditary fructose intolerance = decreased sucrose and fructose
*galactosemia = (-) milk/dairy products
*sitosterolemia = decreased plant sterols
*adrenoleukodystrophy = decreased VLCT, increased C18:1+C22:1
*abetalipoproteinemia = decreased fat, increased MCT, increased vit A and E

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2
Q

abetalipoproteinemia - clinical features

A

*rare autosomal recessive disorder
*neonatal diarrhea, steatorrhea
*failure to thrive & poor growth
*hemolytic anemia
*retinal degeneration
*progressive neurologic dysfunction and early death in teenage years

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3
Q

abetalipoproteinemia - biochemical features

A

*very low plasma triglyceride and cholesterol
*absence of chylomicrons, VLDL, LDL, and apo B
*no post-prandial rise in triglycerides
*pre-chylomicrons absent from enterocyte golgi
*slice mutation in the MTP gene

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4
Q

abetalipoproteinemia - treatment

A

*very low-fat, high carb, high protein diet
*supplementation with MCT (medium-chain triglycerides)
*pharmacologic doses of surfactant solubilized vitamin A and vitamin E
*ALL CLINICAL FEATURES CAN BE PREVENTED

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5
Q

examples of diet as an adjunct to treat common diseases

A

*obesity = lower total caloric index
*diabetes = change percent of energy distribution
*HTN = decrease sodium
*hypercholesterolemia = decrease saturated fat and increase unsaturated fat
*osteoporosis = increase calcium and vit D

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6
Q

post-gastrectomy patients - clinical features

A

*early satiety
*dumping syndrome: pain, diarrhea, vomiting
*iron malabsorption -> iron deficiency anemia
*vitamin B12 malabsorption -> macrocytic anemia + peripheral neuropathy

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7
Q

iron malabsorption post-gastrectomy

A

*normally, stomach acid helps reduce Fe3+ to Fe2+, and Fe2+ can be absorbed by duodenal iron transporters
*post-gastrectomy, non-heme iron (iron from diet) remains in Fe3+ form, leading to poor absorption in duodenum
*therefore, we must either feed heme iron (absorption of heme iron remains intact) or give a supplement of Fe2+ iron

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8
Q

vitamin B12 absorption - overview

A
  1. haptocorrin (made in salivary glands) binds to vit B12 to help get it through the stomach
  2. pancreatic proteases digest haptocorrin away
  3. intrinsic factor (made by parietal cells; resistant to digestion) binds to the B12
  4. cubam (ileal B12 transporter) recognizes B12 bound to intrinsic factor and transports it

note - the cubam transporter is ONLY found in the terminal ileum, so B12 can only be absorbed here

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9
Q

vitamin B12 malabsorption post-gastrectomy

A
  1. haptocorrin (made in salivary glands) binds to vit B12
  2. pancreatic proteases digest haptocorrin away
  3. ABSENT INTRINSIC FACTOR leads to unbound vit B12
  4. cubam transporter cannot recognize unbound B12, leading to failure of absorption
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10
Q

treatment - post-gastrectomy

A

*multiple small, frequent meals
*moderate dietary fat restriction
*oral iron supplementation (Fe2+)
*PARENTERAL vitamin B12

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11
Q

examples of diet as an adjunct for diseases

A

*hypertriglyceridemia = increased omega-3 fatty acids
*seizure disorders = ketogenic diet
*Parkinson’s disease = time-shift protein intake (at night)
*lactase deficiency = decreased lactose

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12
Q

enteric hyperoxaluria (kidney stones due to increased calcium oxalate stones) - clinical & biochemical features

A

*terminal ileum disease or resection leads to fat and bile acid malabsorption
*fat and bile acid malabsorption leads to calcium oxalate kidney stones

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13
Q

enteric hyperoxaluria - treatment

A

*supplement calcium
*high fluid intake
*low oxalate diet (no root veggies, tea, cocoa, peanuts, bran, rhubarb)
*moderate fat restriction

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14
Q

examples of diet for “disease prevention”

A

*Mediterranean diet prevents ASCVD, diabetes, and osteoporosis
*DASH diet prevents HTN and diabetes
*MIND diet prevents cognitive decline & alzheimer’s

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