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56 Cards in this Set
- Front
- Back
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classic triad of Multiple sclerosis symptoms
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SIN
-scanning speech -intention tremor, incontinence, internuclear opthalmoplegia -nystagmus |
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with what disorders do MS patients present with?
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-optic neuritis
-MLF syndrome -hemiparesis -hemisensory symptoms -bladder/bowel incontinence |
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gene defect of alport syndrome
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IV - under the floor (4)
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child presents with nephritis, deafness, and ocular dysfunction what are you thinking they might have
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alport syndrome
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what type of collagen organizes into bone, skin, tendons?
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one
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what type of collagen organizes into cartilage?
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two
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type 3 collagen organizes into?
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reticulin: skin, blood vessels, uterus, fetal tissue, granulation tissue
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type 4 collagen organizes into?
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basement membrane or basil lamina
-important in basement membrane of kidney, eyes, and ears |
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leonine facies is caused by what organism?
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mycobacterium leprae (acid-fast bacillus that likes cool temps)
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where does mycobacterium leprae inhabit
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armadillos in the US
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2 types of leprosy
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1) lepromatous: diffusely over skin and is communicable; low cell-mediated immunity with humoral Th2 response
2. tuberculoid: limited to few hypoesthetic skin plaques; high cell-mediated immunity with largely Th1-type immunity response |
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antidote for digoxin
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digoxin immune Fab
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causes of nutmeg liver
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RHF, bud-chiari
can lead to centrilobular congestion and necrosis and eventually cardiac cirrhosis |
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abdominal pain gets worse with eating
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gastric ulcer
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abdominal pain gets better with eating
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duodenal ulcer
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most common causes of gastric ulcers
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h. pylori, NSAIDS, decreased protection
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ankylosing spondylitis other symptoms
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uveitis, aortic regurgitation
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endocrine functions of kidney
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-EPO
-1,25 - vitamin D -renin -prostoglandins |
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clinical finding:
peripheral neuropathy of hands and feet, angiokeratomas lysosomal storage disease? |
fabry's disease
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deficient enzyme of fabry's disease
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alpha-galactosidase
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accumulated substrate of fabry's disease (inheritance)
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ceramide trihexoside (XR)
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clinical findings:
corneal clouding + mental retardation, gargoylism, airway obstruction, hepatosplenomegaly lysosomal storage disease? |
Hurler's syndrome
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deficient enzyme of hurler's syndrome (inheritance)
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alpha-L-iduronidase (AR)
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accumulated substrate of hurler's syndrome
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heparan sulfate, dermatan sulfate
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clinical findings:
no corneal clouding + mental retardation, aggressive behavior lysosomal storage disease? |
Hunter's syndrome
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deficient syndrome of Hunter's
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iduronase sulfatase
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accumulated substrate of Hunter's
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heparan sulfate, dermatan sulfate
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clinical findings:
demyelinating disease affects peripheral nerves (and central, dementia) lysosomal storage disease? |
metachromatic leukodystrophy
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deficient enzyme of metachromatic leukodystrophy
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arylsulfatase A
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accumulated substrate in metachromatic leukodystrophy
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cerebroside sulfate
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inheritance of hunter's
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XR
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inheritance of metachromatic leukodystrophy
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AR
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inheritance of niemann-pick disease
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AR
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inheritance of krabbe's disease
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AR
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clinical findings:
sphingomyelin build up, progressive neurodegeneration, cherry red spot on macula, foam cells lysosomal storage disease? |
niemann-pick disease
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deficient enzyme in neimann-pick disease
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sphingomyelinase
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accumulated substrate in neimann-pick disease
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sphingomyelin
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clinical findings:
accumulation of galactocerebroside in brain, peripheral neuropathy, developmental delay, optic atrophy, globoid cells lysosomal storage disease? |
krabbe's disease
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deficient enzyme in krabbe's disease
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beta-galactocerebrosidease
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accumulated substrate of krabbe's
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galactocerebroside
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clinical findings:
hepatosplenomegaly, aseptic necrosis of femur, bone crises, macrophages that look like crumpled tissue paper lysosomal storage disease? |
gaucher's
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clinical findings:
progressive neurodegenertaion, developmental delay, cherry-red spot on macula, lysosomes with onion skin, no hepatosplenomegaly lysosomal storage disease? |
tay sachs
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deficient enzyme of tay-sachs
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hexosaminidase A
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deficient enzyme of gaucher's
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glucocerebrosidase
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accumulated substrate in tay-sachs
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GM2 ganglioside
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accumulated substrate in gaucher's
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glucocerebroside
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inheritance of tay-sachs
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AR
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inheritance of gaucher's
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AR
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neomycin class
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aminoglycocides
(Mean" GNATS : gentamicin, neomycin, amikacin, tobramycin, streptomycin) |
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neomycin mechanism of action
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bacteroicidal: inhibit formation of initiation complex and cause misreading of mRNA
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neomycin toxicities
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Nephrotoxicity, ototoxicity, teratogen
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beta-blockers and asthmatics
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exacerbates asthma (bronchoconstriction)
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Beta-1 selective blockers
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A BEAM of Beta 1 blockers:
Acebutolol, Betaxolol, Esmolol, Atenolol, Metoprolol |
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primary cortical adrenal insufficiency
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Addisons: A!!
Adrenal atrophy, Absence of hormonal production, All three cortical divisions (spared medulla) |
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primary vs secondary cortical adrenal insufficiency
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1: hypotension (hyponatremia volume contraction), hyperkalemia, acidosis, skin hyperpigmentation
2:decrease in ACTH production which has NO hyperpigmentation and no hyperkalemia |
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non-hodgkin's lymphoma characteristics
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-HIV, immunosuppression
-multiple, peripheral nodes, B cells -fewer signs and symptoms -20-40 year olds |
