Metabolic disorders Flashcards
(21 cards)
How do you treat paget’s disease?
Asymptomatic = clinical surveillance
Skull, long bones, or vertebrae involvement = bisphosphonates
Zoledronic acid IV
Calcium and vitamin D supplements
→ correct Vitamin D deficiency prior to treatment
How do you treat hemochromatosis?
Avoid intake - red meat, supplemental iron, etc
ETOH, Vitamin C, raw shellfish
Depletion of iron stores by phlebotomy - 2-3y of weekly phlebotomy
Done for symptomatic patients
OR men-serum ferritin OR high fasting iron saturation
Consider PPIs to lower maintenance phlebotomy need
Chelation w/ deferoxamine if pt has anemia w/ iron overload from Thalassemia and intolerant of phlebotomy
How do you treat wilson disease
Restriction of dietary copper (shellfish, organ, nuts, mushrooms, chocolate)
Oral D-penicillamine - 1 hour before or 2 hours after food
Oral pyridoxine to refresh B6 stores
SE: GI intolerance, hypersensitivity, autoimmune reactions, nephrotoxicity, bone marrow toxicity
Trientine hydrochloride (less SE, but $) or trientine tetrahydrochloride
2nd line = oral zinc acetate or zinc gluconate for first line asymptomatic or pregnant patients, maintenance
non-pharm obesity treatment
diet, physical activity, behavior modifications
→ unprocessed foods, limiting high calorie foods, emphasizing mediterranean diet, low-glycemic index diet, meal replacement
→ meal planning, self-monitoring, food log, recognize eating cues
→ aerobic exercise, higher intensity
Bariatric surgery in those with BMI
40+ or 35+ with complications (sleeve gastrectomy - irreversible, Roux-en-Y gastric bypass - reversible)
– CI: poor cardiac reserve, COPD or respiratory dysfunction, severe psych disorders, nonadherence to medical treatment
how do you treat b1 deficiency
Large parenteral doses of thiamine (only effective if magnesium is repleted)
– should receive simultaneous therapeutic doses of other water-soluble vitamins
Dry beriberi → neuro
Wet beriberi → cardio
how do you treat b2 deficiency
Treat empirically
Foods: meat, fish, dairy products
Oral preparations
IV preparations
how do you treat vitamin b3
Oral niacin as nicotinamide
how do you treat vitamin c deficiency
ascorbic acid
how do you treat vitamin a deficiency
Vitamin A daily for 1 week
how do you treat vitamin A toxicity
Withdrawal of vitamin A from diet
how do you treat vitamin d deficiency
D3 (ergocalciferol)
– increased dose if patient is obese, has malabsorption syndrome, or is taking meds affecting metabolism
Calcitriol orally or IV with chronic renal failure, nephrotic syndrome, or severe other syndromes, severe liver disease, impaired hepatic metabolism
UV light
Monitoring: 10-12 weeks after starting replacement therapy, consider monitoring for toxicities
Prevent = dietary intake (fatty fish, cod liver oil, egg yolks, sun-exposed mushrooms, fortified foods), sun exposure
how do you treat vitamin e deficiency
large doses PO
how do you treat vitamin K deficiency
Vitamin K1 IV or PO
Oral dose
IV administration = faster normalized PT (low dose and slowly due to risk of anaphylaxis)
how do you treat refeeding syndrome
Start feeds at low rate (~50%), with empiric thiamine and electrolyte administration
Risk is greatest in first 72 hours – 2x/day electrolyte checks and generous electrolyte replenishment if low levels are identified
how do you treat whipple disease
Antibiotics for a year – ceftriaxone IV for 14 days
Bactrim PO x 12 months
how do you treat SIBO
Cipro, augmentin, bactrim x 7-10 days
how do you treat short gut/bowel syndrome
Supplemental IV B12, modified diet, loperamide
Teduglutide
Parenteral nutrition → mortality
how do you treat lactase deficiency
Lactose free diet (counsel on dietary calcium), lactase supplements
how do you treat celiac disease
Gluten free diet with no cross-contamination
– avoid dairy/take lactase until intestinal symptoms have improved
Rechallenge w/ gluten may trigger severe diarrhea with dehydration and electrolyte imbalance and may require TPN and IV/oral steroids
Annual monitoring: CBC, CMP, gastric bypass lab panel
DEXA
→ high risk of osteoporosis or osteopenia
→ high association of other AI illness, autoimmune hepatitis
→ Can develop to intestinal T-cell lymphoma
how to treat an allergic reaction
Avoidance of food including cross-contamination with guaranteed access to epinephrine injector at all times
Refer to immunologist for immunotherapy
