Goljan/Pathoma/UWorld Miscellaneous Flashcards
(164 cards)
Caisson disease aka decompression sickness
atmospheric pressure increases –> N2 gas becomes dissolved in tissue
quick assent - bubbles emerge from solution, can occlude blood vessels
- quadriplegia - nerves of SC susceptible are highly susceptible
- loss of bladder control
- death
treat with hyperbaric oxygen chamber
anorexia nervosa
bulimia nervosa
anorexia nervosa:
distorted body image
refeeding syndrome - HYPOphosphatemia (P becomes trapped in intracellular space)
osteoporosis! - after you lose 15-20% body weight, GnRH release stops
- how do you treat this?
1) encourage weight gain - in general, for boards, think cheap
2) later consider birth control pills
death - due to heart failure
little evidence to support pharmacotherapy as primary treatment in anorexia
bulimia nervosa:
no distorted body image
vomiting - metabolic alkalosis with respiratory compensation
- hypoxemia - not good for heart –> premature ventricular contractions –> v. fib –> death
obesity
kg/(height m^2)
main complication is HTN (mechanism is not fully understood)
marasmus
kwashikor
marasmus:
- TOTAL calorie deprivation, nutrients still present
treat by feeding
- NO edema, there is muscle wasting
kwashikor:
missing protein = small child with swollen abdomen
-anemia
-cell immunity issues - cant fight against Candida, mumps
-low albumin, ascites, fatty livers (due to decreased apolipoprotein synthesis)
- skin lesions - hyperkeratosis and hyperpigmentation
-have to force feed - more likely to die (compared to marasmus)
there are two diseases where you want to restrict protein
- renal disease - protein means more urea
- cirrhosis of liver - defective urea cycle, cant metabolize ammonia
- most ammonia comes from bacteria - convert urea to ammonia –> ammonia is reabsorbed –> urea cycle in liver
- hepatic encephalopathy
vitamins ADEK
random note - water soluble vitamins are cofactors for biochemical reactions
- B complex def = dermatitis, glossitis, diarrhea
fat soluble vitamins - mineral oil intake can cause deficiency
A - growth (muscle, bone), rhodopsin, prevents squamous metaplasia (keratomalacia –> cornea goes soft –> blindness)
- essential for specialized epithelium - pancreatic tissue, goblet cells
- most common causes of blindness - trachoma (WW), DM (US), vitamin A deficiency
- follicular hyperkeratosis ~ goosebumps
- excess - hunter that eats bear liver –> cerebral edema, headache. Liver tox.
- acute tox - N&V, vertigo, blurred vision - teratogenic - cleft palate, cardiac abnormalities
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vitamin D - most importantly source is sunlight
-reabsorbed in jejunum
-PTH puts 1a-hydroxylase in the proximal tubule
increased bone mineralization at low levels, increased bone resorption at high levels
vitamin D binds to receptor on osteoBLAST- causes release of alk phos
-remember when calcitonin binds to osteoclast receptor - it inhibits the osteoclast, only hormone that binds to osteoclasts
PTH binds to osteoblast –> releases IL1 aka osteoclast-activating factor
- estrogen/testosterone keeps a check on IL1
- ….osteoporosis in women
deficiency - most commonly due to renal disease (DM is the most common cause of chronic renal disease in the US)
- all CRF pts are put on 1,25-vitamin D - NOT the formulation you get in the store
- over the counter vitamin D - has to pass through liver and kidneys
osteomalacia - soft bones, pathologic fractures
kids (rickets) - craniotabes (recoil when you press on the skull of a KID), rachitic rosary (because that is where osteoid IS, but it cant be mineralized)
- type 1 - missing 1a-hydroxylase
- type 2 (XD) - missing vitamin D receptor
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vit E- maintains cell membranes, prevents lipid peroxidation of cell membranes (prevents breakdown by phospholipase A)
- can also neutralize oxidized LDL - oxidized LDL is MORE atherogenic (LDL that macrophages phagocytose) = cardioprotective
deficiency is very uncommon - seen in kids with CF
- these kids have pancreas problems!!!
- can get hemolytic anemia - RBC membranes get damage
- myelin issues - posterior column disease, spinal/cerebellar-type disease
tox - prevents synthesis of vitamin K-dep coagulation factors = anti-coagulated
- can OD by taking an extra capsule
- OR if you are taking vitamin E with warfarin
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vit K - phy-something
made by ANaerobes in the gut
-bacteria makes K2 - has to converted to the active form K1 by epoxide reductase
K1, gamma-carboxylase - glutamic acid residues carboxylated
- these coagulation factors are bound to a clot by Ca
- carboxylated glutamic acid residues BIND the Ca –> clot formation
scenario - rat poison = warfarin!, kid eats rat poison….
-treat with IM vitamin K
kid lives with elderly grandparents –> hemorrhage, why?
-kid ate grandparents warfarin
CYP 450 inducers
CYP450 system is in sER
inducers - phenytoin, alcohol, rifampin, barbiturates
- -> sER hyperplasia - items made in the liver are metabolized faster
- this includes drugs and 25-OH-vitamin D
scenarios
- pts can get hypocalcemia (vit D deficiency)
- lady on OCP + phenytoin - gets pregnant
GGT - enzyme of sER, that is why you have elevated GGT in alcoholics
vitamin C
hydroxylates proline and lysine - in the Golgi apparatus
deficiency - tea and toast diet
- hemarthrosis (and hemophilia A) - blood vessels are unstable and rupture
- perifollicular hemorrhages - ring (of blood) around the follicles
- glossitis
- hemorrhagic diaphysis
excess - GI upset, increase in uric acid stones, calcium oxalate stones, can increase Fe tox
- increases absorption of non-heme Fe - reduces Fe to Fe2+
vitamin C - ancillary treatment for methemoglobinemia, it is a scavenger for free radicals
cofactor for making catecholamines - makes NE from DOPA, epi from NE
thiamine B1
think ATP - lose the production of about 30 ATP (6 from pyruvate to acetyl coA, 24 from TCA)
pyruvate dehydrogenase, transketolase, a-ketoglutarate dehydrogenase (and branched-chain ketoacid dehydrogenase)
dry beri beri - need a lot of ATP to make myelin
- peripheral neuropathy (foot drop due to common peroneal palsy, wrist drop due to radial palsy, ulnar palsy), symmetrical muscle wasting
- W-K psychosis - Wernicke is confusion, ataxia, nystamus, ophthalmoplegia
- damage to medial dorsal nucleus of thalamus..
wet beri beri - (neuropathy +) heart failure
deficiency ddx by increased RBC transketolase following B1 administration
pt comes into ER comatose
give them
- IV glucose
- IV thiamine
- naloxone - in case of OD
niacin
nicotinic acid - lipid lowering agent that lowers VLDLs and increases HLD, used to treat familial combined HLD (elevated cholesterol and TGs)
- side effect flushing - take aspirin beforehand to avoid
deficiency can be caused by Hartnup dz (AR, tryptophan is a neutral amino acid), malignant carcinoid syndrome, izoniazid
pellagra, peripheral neuropathy
riboflavin B2
cofactor for glutathione reductase
deficiency - cheilosis, corneal vascularization, normocytic anemia
pyridoxine B6
converted to PLP (pyridoxal P) - ALT, AST, decarboxylation reactions, glycogen phosphorylase
synthesis of cystathionine, niacin, histamine, etc.
heme synthesis:
- succinyl coA + glycine, ALA synthase, B6 cofactor
- cytochrome system is also the heme system
cofactor for transaminase reaction
- alanine is the most important substrate for making glucose in the fasting state (alanine - NH2 –> pyruvate), a-ketoglutarate takes this NH2
- aspartate - NH2 –> oxaloacetate
(- glutamate -NH2 forms a-ketoglutarate)
synthesis of NTs
- neonates deficient in B6 will get seizures
most common cause of deficiency - isoniazid
def - peripheral neuropathy (isoniazid, OCPs)…
pantothenic acid
involved in fatty acid synthase - important in making palmitic acid and coA
def –> adrenal insufficiency
biotin B7
pyruvate carboxylase, biotin –> oxaloacetate
- pyruvate will build up and it will be forced to become lactic acid
cofactor for other carboxylation enzymes (acetyl –> malonyl, propionyl –> methylmalonyl)
deficient if you eat 7 raw eggs a day or with antibiotic use
- rash and go bald
trace elements
chromium:
glucose tolerance factor - helps insulin do its job
fl:
to prevent dental caries
too much Fl - white chalky teeth
- and calcification of ligaments where they insert into bone
selenium:
PPP and forming glutathione
- cofactor for glutathione peroxidase = selenium is an antioxidant (vitamin E capsules w/ selenium)
Cu
lysyl oxidase - cross links between collagen fibrils AND elastic tissue
dissecting aortic aneurysm
Zn
older person with dysgeusia = abnormal taste and anosmia = abnormal smell
rash on face and acrodermatitis enteropathica (desquamating rash on butt)
collagenase (metalloenzyme) contains Zn - breaks down type 3 collagen so you can make type 1 collagen
- poor wound healing
hypogonadism and decreased adult hair
diabetics are all Zn deficient
fiber
soluble fiber can lower cholesterol
insoluble fiber - hold water and toxins (lipocholic acid - carcinogenic bile acid) in the colon
- main method of recycling estrogen is through bile
- fiber will grab estrogen too - decreased risk of breast cancer
most common benign tumors
women: located in uterus - leiomyoma (aka fibroid), do NOT become leiomyosarcomas
men: lipoma
tubular adenoma
aka strawberry on a stick
precursor lesion to colon cancer
carcinoma
malignant cancer of epithelial cells
- squamous cells
- glands - adenocarcinoma
- urothelium - transitional cell carcinoma
melanoma
malignancy of melanocytes, nevus is the benign lesion
- S100 positive
- neural crest origin
step 1) excision
scenario- 2 yo with nodules to skin
- nodules are S100 positive
- answer - adrenoblastoma with mets to the skin
APUD tumor - a group of apparently unrelated cells
- neuroendocrine tumor
embryonal rhabdomyosarcoma
most common sarcoma of childhood
- grape-like mass off vagina or penis - desmin positive, striations on histology
mixed tumor
mixed tumors have 2 different tissues
parotid gland tumor - mobile mass at angle of the jaw
-usu malignant
v. s. teratoma - tumor of all 3 cell layers, germ-cell tumor
- 16 yo girl presents wiht RLQ pain, calcifications seen on imaging
- RLQ diff - ectopic pregnancy, appy, Crohns
- teratomas stay midline - pineal gland, ovary