Term
What cell types/tissues arise from neural crest cells? |
|
Definition
ANS, Cranial Nerves, dorsal root ganglion, Celiac Ganglia, Schwann Cells, Pia, Arachnoid, Bones of the skull, Anterior chamber of eye structures, Chromaffin cels of adrenal medulla,
thyroid and laryngeal cartilage, parafollicular cells, odontoblasts, bones of the inner ear, aorticopulmonary septum,
enteric nervous system, entrochromaffin cells, melanocytes |
|
|
Term
Which Amino acids are modified in the GOLGI appparatus? |
|
Definition
Serine
Threonine
Asparagine
"Momma Golgi is Serious with her Threat, eat your Asparagus" |
|
|
Term
|
Definition
I cell disease is a lysosomal storage disorder: failure of addition of a monnose-6-phosphate to lysosome enzymes: enzymes secreted out of the cell instead of being used in lysosome to breakdown stuff:
results in accumulation of stuff in lysosomes (inclusion bodies) clinically: enlarged liver, spleen, coarse faces, mental retardation, motor retardation due to skeletal abnormalities and joint restriction: death before 10 yo.[image]
|
|
|
Term
What is the maternal component of the placenta? |
|
Definition
|
|
Term
What are the stages of the embryo between conception and an inner cell mass? |
|
Definition
zygote-->morula-->blastocyst-->inner cell mass & trophoblast
[image] |
|
|
Term
What placental structure secretes hCG? |
|
Definition
|
|
Term
What drugs are contraindicated in preganancy? |
|
Definition
A3,C3,
D,
F2,
G,L,P,S,
T2,
V,W
ACE inhibitors: renal damge
Alkylating agents: absence of digits, mulitple anomalies
Aminoglycosides: CN 8 toxicity
Carbamazepine: neural tube defect, craniofacial, fingernail hypoplasia, developmental delay, IUGR
Clarithromycin
Chloramphenicol
Diethylstilbestrol: DES: vaginal clear cell adenocarcinoma: mullerian anomalies
Flourouquinolones
Folate Antagonists (methotrexate and pyrimethamine): neural tube defects
Griseofulvin
Lithium: Ebstein's anomaly (atrialized tight ventricle)
Phenytoin: many problems
Sulfonamides
Tetracycline: discolored teeth
Thalidomide: limb defects
Valproate:inhibition of folate absorption: neural tube defects
Warfarin: bone deformities, fetal hemorrhage, abortion, opthalmologic abnorm.
|
|
|
Term
Which vitamin should not be supplemented in large amounts during preganancy? |
|
Definition
|
|
Term
What molecules provide the structural framework for DNA and the nuclear envelope? |
|
Definition
|
|
Term
What must be present on a protein in order for that protein to gain entry into the nucleus? |
|
Definition
nuclear localization signal |
|
|
Term
Which Cyclin-CDK complexes assist in the progression from G1 phase to S phase? |
|
Definition
Cyclin D-CDK4
&
Cyclin E-CDK2 |
|
|
Term
Which proteins prevent the progression of the cell into S phase? |
|
Definition
Tumor suppressor proteins Rb and p53 |
|
|
Term
Which Cyclin CDK complexes assist in progression from G2 to M phase? |
|
Definition
Cyclin A-CDK2
&
Cyclin B-CDK1 |
|
|
Term
What molecule targets proteins in the endoplasmic reticulum for lysosomes? |
|
Definition
|
|
Term
Which cell types are rich in SMOOTH ER? |
|
Definition
Hepatocytes
&
Hormone producing cells of the adrenal cortex |
|
|
Term
What transcription factor is bound to Rb, and when unbound, the cell can prepare to move from G1 into S phase? |
|
Definition
|
|
Term
What drugs act on microtubules? |
|
Definition
Mebendazole/Thiabendazole (helminth)
Griseofulvin (fungal)
Vincristine/Vinblastine (cancer)
Paclitaxel(breast cancer)
Colchicine (gout) |
|
|
Term
Which organelle is responsible for the breakdown of very long chain fatty acids (VDL)? |
|
Definition
Peroxisomes via beta oxidation |
|
|
Term
What are the intermediate filaments for CT, Muscle, Epithelium, and axons? |
|
Definition
CT: Vimentin
Muscle: Desmin
Epethilium:Cytokeratin
Axon: Neural filaments
*can be used as tumor marker to determine the origin of the tumor* |
|
|
Term
Which amino acids are found in Nuclear Localization Signals? |
|
Definition
Proline
Arginine
Lysine
Remember: you have to be "PAL" to get into the nucleus |
|
|
Term
What is Kartagener Syndrome? |
|
Definition
dyenein arm defect:
immotile cilia
bronchiectasis
infertility
situs inversus[image] |
|
|
Term
What drugs act on the arachidonic acid pathway? |
|
Definition
Corticosteroids: PLA2 inhibitor
Zileuton: LOX inhibitor
Aspirin, NSAIDs, acetaminophen, COX2 inhibitors: COX inhibitor
Zafirluklast, Monteklast: LTC4 and LTD4 receptors |
|
|
Term
What are the two most abundant substances in the plasma membrane? |
|
Definition
Cholesterol and Phospholipids |
|
|
Term
What protein is involved in transport of the endocytosed vesicle from the plasma membrane to the endosome? |
|
Definition
|
|
Term
What is digoxin? WHat does it treat and how? |
|
Definition
A cardiac glycoside: which directly inhibits the Na-K ATPase causing an increase in intracellular CALCIUM and an increase in cardiact CONTRACTILITY
used to treat A-fib and CHF |
|
|
Term
What causes scurvy and why? |
|
Definition
deficiency of Vitamin C due to an inability to hydroxylate proline and lysine residues during collagen synthesis |
|
|
Term
What amino acids are found in large concentrations in collagen? |
|
Definition
hydroxylated and glycosylated proline and lysine
(proline and lysine are found in elastin too, but not modified) |
|
|
Term
Name the different types of collagen and where they are found. Name associated disorders |
|
Definition
Type I:Strong (bone, wound repair) Osteogenisis imperfecta
Type II:Slippery (cartilage, nucleus pulposis,vitreous body)
Type III:Bloody (BVs, skin, granulation tissue, uterus, fetal tissue) Ehler's Danlos
Type IV:BM (basement membrane) Alports syndrome |
|
|
Term
Briefly describe process of leukocyte extravasation and associated molecules. |
|
Definition
Rolling : selectins
Tight binding: integrins
Diapedisis: PECAM-1
Migration: IL-8, LTB4, Kallikrein,C5a |
|
|
Term
What cytokine is particularly important in the formation of granulomas? |
|
Definition
|
|
Term
|
Definition
a TNF-alpha blocker which breaks down granulomas. used for chronic inflammation like RA or Crohn's
*must do PPD before treatment bc if used on someone with latent TB, it can reactivate TB by breaking down the granulomas* |
|
|
Term
What cell type plays a role in inflammation by generation fibrinogen and CRP? |
|
Definition
|
|
Term
What cell is most repsonsible for the ACUTE phase of inflammation? |
|
Definition
|
|
Term
What findings are associated with Ehlers Danlos? |
|
Definition
Hyperelastic skin
Hypermobile joints
easy bruising
easy bleeding
berry aneurysms
(Type 3 collagen defect)
cousin nadia: 3 SISTERS |
|
|
Term
Metalloproteases need____ to do their function in wound healing which is___ |
|
Definition
Metaliproteases use Zinc to streamline and remodel the extracellular matrix |
|
|
Term
What is Chediak-Higashi Syndrome? |
|
Definition
rare autosomal recessive disorder caused by a mutation of a lysosomal trafficking regulator protein
-decrease in phagocytosis.
-recurrent pyogenic infections,
-partial albinism
-peripheral neuropathy.[image] |
|
|
Term
What area of the colon is most susceptible to ischemic damage? |
|
Definition
The watershed aread of the splenic flexure[image] |
|
|
Term
What is a lipofuscin granule? |
|
Definition
Wear and tear pigment: sign of aging: yelow-brown pigment containing residues of lysosomal digestions. found in the liver, kidney, heart muscle, retina, adrenals, nerve cells, and ganglion cells
[image] |
|
|
Term
What does the telencephalon become? |
|
Definition
Cerebral hemispheres, basal ganglia, hippocampus and amygdala
[image] |
|
|
Term
What does the diencephalon become? |
|
Definition
Thalamus, hypothalamus and the optic nerves and tracts
[image] |
|
|
Term
What does the Mesencephlon become? |
|
Definition
|
|
Term
What does the Metencephalon become? |
|
Definition
the Pons and the Cerebellum |
|
|
Term
What does the Myelencephalon become? |
|
Definition
|
|
Term
What is a neural tube defect and what is it associated with? |
|
Definition
Failure of the neuropores to fuse in the 4th week resulting in a persistent connection between amniotic cavity and spinal canal
associated with Low Folic acid intake before conception and during pregnancy
elevated alpha-fetoprotein in amniotic fluid and maternal serum along with increase AChesterase
[image] |
|
|
Term
How do you check for neural tube defects in a pregnancy? |
|
Definition
elevation of AFP in maternal serum AND amniotic fluid
and elevation of Acetylcholinesterase |
|
|
Term
How do you check for down's syndrome in pregnancy? |
|
Definition
|
|
Term
If you see a tuft of hair/dimple upon newborn exam in the lumbo-sacral region what should you be concerned about?
[image] |
|
Definition
spina bifida occulta: failure of bony spinal canal to close, but no structural herniation
[image] |
|
|
Term
What is the difference between a menigocele and a myelomeningocele? |
|
Definition
A meningocele is herniation of the meninges only through a spinal canal defect.
A myelomeningocele is herniation of the meninges and the spinal cord through a spinal canal defect. |
|
|
Term
What defect is associated wit holoprosencelphaly? |
|
Definition
Sonic hedgehog protein defect
failure of L and R hemispheres to septate
moderate: cleft lip/palate
severe:cyclopia
also associated with Patau syndrome (trisomy 13) and Fetal Alcohol Syndrome
[image] |
|
|
Term
What is anencephaly and its characterististic?
|
|
Definition
anterior neural tube defect:
no forebrain, open calvarium
elevated AFP, polyhydramnios (no fetal swalling center)
associated with maternal type I diabetes and low folic acid[image]
|
|
|
Term
What are Chiari malformations? |
|
Definition
Posterior fossa malformations
-cerebellar tonsillar and vermian herniation through foramen magnum with acqueductal stenosis and hydrocephalus
I:mild, subclinical
II-IV:clinical symptoms: thoacolumbar myelomeningocele and
Syringomelia
[image] |
|
|
Term
What is Dandy-Walker syndrome? |
|
Definition
A posterior fossa malformation like Arnold-Chiari
-agenisis of cerebellar vermis with cystic enlargement of 4th ventricle
associated with spina bifida and hydrocephalus
[image] |
|
|
Term
What are the classic presenting symptoms of syringomyelia? |
|
Definition
A cystic enlargement of the central canal of the spinal cord: crossing fibers of the spinothalamic tract are typically damaged first.
assoc with chiari malformation I
cape like bilateral loss of pain and temperature sensation in upper extremeties
(fine touch is preserved)
usually C8-T11
[image][image] |
|
|
Term
|
Definition
RER staining in the cell bodies and dendrites
(NO RER IN AXONS)[image] |
|
|
Term
What are the supportive cells of the nervous system? |
|
Definition
Astrocytes:physical support, repair K metabolism, removal of excess NT, maintains BBB, reactive gliosis in injury, marker is GFAP for astrocyte tumors
Microglia:Phagocytes, make multinucleated giant cells in response to HIV
Oligodendroglia:destroyed in MS, look like fried eggs on H&E
Ependymal cells: arises from neuroectoderm, line ventricles and make CSF Schwann cells:myelinates one axon in PNS, promotes axonal regeneration, destroyed in Guilan Barre Syndrome, associated with acoustic neuroma (schwannoma) NFtype II
[image] |
|
|
Term
Describe the layers of the BBB |
|
Definition
ENDOTHELUM
(nonfenestrated capillary endothelial cells)
BASEMENT MEMBRANE
ASTROCYTE FOOT PROCESSES |
|
|
Term
What is excessive Norepinephrine associated with?
low levels? |
|
Definition
excessive: anxiety and mania
low: depression |
|
|
Term
Where is NE made in the brain? |
|
Definition
Locus ceruleus
reticular formation
solitary tract |
|
|
Term
What conditions are associated with an increase in dopamine? |
|
Definition
Schizophrenia and depression |
|
|
Term
What are the 4 major dopaminergic pathways and what is the result of blocking each? |
|
Definition
MesoCor-MesoLim-NigroStri-TuberoIn
Mesocortical Pathway: ventral tegmental of midbrain to cortex
blocking: increase in negative symptoms of psychosis
Mesolimbic Pathway: ventral tegmental of midbrain to limbic system. primary target for anti-schizo drugs
blocking: relief of psychosis
Nigrostriatal Pathway: substantia nigra(pars compacta) to the striatum (caudate and putamen)
blocking: parkinsonian symptoms
Tuberoinfundibular pathway: arcuate nucleus of hypothalamus to pituitary
blocking: increase in release of prolactin from pituitary
gynecomastia, galactorrhea and ameonrhhea
[image] |
|
|
Term
Chlamydia species
(sketchy) |
|
Definition
|
|
Term
What are the effects of decreased 5-HT in the brain? |
|
Definition
(serotonin)
depression, anxiety and decreased alertness
originates in the Raphe nucleus
"You are missing Sera so you will be sad, anxious and just wanna sleep until she comes back" |
|
|
Term
What major diseases are associated with a decrease in Acetylcholine in the brain? |
|
Definition
Alzheimer's and Huntington's disease
(increased in parkinsons) |
|
|
Term
What amino acid is GABA synthesized from?
what cofactor does it require?
What happens if there is a deficiency?
Where is it synthesized? |
|
Definition
Glutamate-->GABA
needs Vitamin B6 (PYRIDOXINE)
main inhibitory NT of CNS
deficiency of B6 can result in convulsions bc less inhibition
decreased in HD: chorea
synthesized in nucleus accumbens
[image] |
|
|
Term
What is the main inhibitory neurotransmitter of the spinal cord? |
|
Definition
|
|
Term
What is the main excitatory neurotransmitter of the CNS? |
|
Definition
|
|
Term
What is the reticular activating system? |
|
Definition
Mediates consciousness, attentiveness, and alertness Consists of the reticular formation, locus ceruleus, and raphe nuclei
lesions cause coma
[image]
|
|
|
Term
What is Sjorgen's Disease? |
|
Definition
Connective Tissue Disease
Sicca complex: Dry eyes, dry mouth
Rheumatoid Arthritis
Antibodies to SS-A(Ro) or Anti-SS-B(La), increased IgG, leukopenia, thrombocytopenia
[image] |
|
|
Term
What are the differences between Rheumatoid Arthritis and OsteoArthiritis? |
|
Definition
RA:inflammatory
erosions
onset 35-45
begins over months
symmetric
systemic
MCP,PIP
OA:noninflammatory
spur formation
onset after 40
begins over years
asymmetric
involves ONLY joints
DIP, PIP, LS spine
[image] |
|
|
Term
|
Definition
a connective-tissue disease related to polymyositis that is characterized by inflammation of the muscles and the skin. characterized by symmetrical proximal muscle weakness, plaque like lesions over MCP, DIP, and PIP: Gottron's papules and heliptrope rash over eyelids (young children can get calcinotic lesions in BVs of legs)
must measure muscle enzymes (CPK, Aldolase, LDH, Neopterin and vWF antigen) along with RF, ANA
[image][image] |
|
|
Term
What is Raynaud's phenomenon? |
|
Definition
symptom of various connective tissue diseases like scleroderma, dermatomyositis, polymyositis, Sjorgrens, SLE, and others..
caused by vasospasms resulting in excessively reduced blood flow in response to cold or emotional stress, causing discoloration of the fingers, toes, and occasionally other areas.
[image] |
|
|
Term
|
Definition
An autoimmune connective tissue disease characterized by hardening of the skin. previously called CREST syndrome for the symptoms: Calcinosis, Raynaud's, esophageal dysmotility, sclerodactyly, and telangiectasia
check for Ant-centromere (limited) and Ant-SCL70(diffuse)
localized can cause morphea: hardened lesions
[image]
28 year old woman: notice skin tightening around lips
[image] |
|
|
Term
Name two Medium sized vessel vasculitis |
|
Definition
Polyarteritis Nodosa
Kawasaki's Disease |
|
|
Term
What is polyarteritis nodosa? |
|
Definition
A medium vessel vasculitis that causing multisystem necrotizing vasculitis of small and medium vessels occuring at the bifurcations of arteries resulting in intmal proliferation, wall degeneration, and fibrinoid necrosis leading to occlusion and ischemia to end organs.
splinter hemorrhages, painful nodules, livedo and purpura, testicular pain, mononeuritis multiplex
sausage link look from dilatation and narrowing
associated with Hep B (10-30%)
NO PULMONARY INVOLVEMENT
[image]
[image]
sausage link
[image]
[image] |
|
|
Term
What is Kawasaki's disease? |
|
Definition
Acute systemic vasculitis, leading cause of acquired heart disease in children in developed countries
-self limited usually involving primarily the coronary arteries
child under 5 yo.
sx: high fever>5days
bilateral nonexudative conjunctivitis
strawberry tongue
cracked lips
oropharyngeal erythema
rash , edema
cervical LAD
very high platelet count and size increase
increase liver enzymes
Tx: important to prevent CAD: high dose aspirin and IVIG
[image][image]
|
|
|
Term
What are two large vessel vasculitis?
|
|
Definition
Giant cell arteritis
Takayasu's arteritis |
|
|
Term
What is Giant cell arteritis? |
|
Definition
aka cranial or temporal arteritis
inflammation of the medium and large arteries branching from the carotid, particulary the temporal. with headache, scalp pain, tender artery, fever malaise, fatigue, anorexia, weight loss, sweats, jaw caludication EMERGENCY: BECAUSE CAN LEAD TO OPTIC NEUROPATHY
Tx: high dose steroids
>50yo, more in women, rare in blacks
segmental involvement: with giant cells
assoc. with polymyalgia rheumatica (stiffness, aching and pain in the neck, shoulders, low back, hips and thighs
elevated ESR
[image][image]
[image] |
|
|
Term
What is Takayasu's Arteritis? |
|
Definition
large and medium vessel arteritis usually inolving te aortic arch and its branches
mostly affects girls and Asian women
histology is the same as Giant cell arteritis
check pulses and perform angiography
Tx prednisone perhaps comb with methotrexate/mycophenolate
[image]
[image] |
|
|
Term
Name three ANCA associated small vessel vascultis |
|
Definition
Granulomatosis with polyangiitis
Churg Strauss Syndrome
Microscopic polyangiitis |
|
|
Term
What is Granulomatosis with polyangiitis (GPA)? |
|
Definition
aka Wegener's
small vessel granulomatous vasculitis of:
upper and lower respiratory tracts
glomerulonephritis
can involve any organ system
more commly in middle aged whites
eye involvement
skin lesions
nasal septal perforation->saddle nose
eustachian tube blockage->otits media
tracheal stenosis-->airway obstruction
cardiac involvement
CNS
pulmonary:cough, hemoptysis, dyspnea, chest discomfort
Renal:high mortality if untreated
C-ANCA
ANCA- PR3
Tx: glucocorticoid, prednisone, cyclophosphamide, rituximab
[image][image] |
|
|
Term
What syndrome is characterized by severe asthma, peripheral tissue eosinophilia, extravascular granuloma formation and small vessel vasculitis of multiple organ systems? |
|
Definition
Churg Strauss
aka allergic granulomatosis and angiitis
often see nasal polyps
p-ANCA |
|
|
Term
What is Microscopic polyangiitis? |
|
Definition
small vessel vasculitis that tends to involve the kidneys more than the lungs
histologically similar to PAN
75% ANCA: anti-myeloperoxidase
[image] |
|
|
Term
What is a non-ANCA small vessel vasculitis most commonly seen in children with palpable purpura and a seasonal variation? And what Immunoglobulin is associated with it? |
|
Definition
Henoch-Schonlein Purpura
IgA
usually see peak in the spring |
|
|
Term
What would you first think if you saw his in a child with abdominal pain and diarrhea?[image] |
|
Definition
|
|
Term
Essential Mixed Cryoglobulinemia is often secondary to what infection? |
|
Definition
Hep C
These disease are also linked to Hep C:
Multiple myeloma, lymphoproliferative disorders, connective tissue disease, liver disease |
|
|
Term
What is Bechet's Syndrome?
|
|
Definition
leukocytoclastic venulitis with fibrinoid necrosis
most commin in mediterranean, middle east and far east ppl
recurrent apthous ulcers, painful, shallow or deep with necrosis (can involve genitals)
bilateral eye involvment
may have GI ulcerations
[image] |
|
|
Term
Polyarteritis nodosa is associated with what infection? |
|
Definition
|
|
Term
Anti-dsDNA and C3 and C4 are often markers of this immune complex disease |
|
Definition
|
|
Term
What is the preferred management for SLE? |
|
Definition
hydroxychloroquine with further meds chosen based on organ involvement |
|
|
Term
What drug is first line for Fibromyalgia? |
|
Definition
Amitriptyline (small dose at bedtime) |
|
|
Term
|
Definition
dry, thickened, scaly or flaky skin
commonly genetic
[image] |
|
|
Term
|
Definition
rare AR disease of thick ridged cracked skin. Usually fatal
[image] |
|
|
Term
What is a Colloidion baby? |
|
Definition
thick taut membran
e that cracks with respiration and may shed leaving normal or abnormal skin[image] |
|
|
Term
|
Definition
Cystic Hygroma: benign tumor of lymphatics |
|
|
Term
|
Definition
Strawberry hemangioma (aka nevus): benign endothelial cell tumor most common tumor in infants, children |
|
|
Term
|
Definition
AR disorder due to lack of the enzyme tyrosinase which is needed in melanocytes to make melanin |
|
|
Term
What is this picture describing?[image] |
|
Definition
Piebaldism: AD localized albinism, lack of melanin in patch of skin or hair: white forelock due to KIT gene encoding a tyrosine receptor |
|
|
Term
What is causing this?[image] |
|
Definition
Arthrogryposis Multiplex Congenita
widespread joint contractures due to failure of muscle development, decreased fetal movement
|
|
|
Term
What is congenital Torticollis? |
|
Definition
Wry neck: stiffness/fibrosis of sternocleidomastoid[image] |
|
|
Term
What is this baby's condition called?
[image] |
|
Definition
Amelia: complete absence of limb. suppression of limb bud development in week 4
Merolemila: partial absence of limb. arrest/disturbance od diferentiation/growth in week 5
(a form is ectrodactyly)
|
|
|
Term
What condition does this family have?
[image] |
|
Definition
Ectrodactyly: a form of meromelia resulting in hand/foot, lobster claw deformity. absent central digit, with developmental failure of one or more digital ray.
|
|
|
Term
What form of meromelia is characterized by the absence of a thumb specifically?
[image] |
|
Definition
congenital absence of radius: failure of radial mesenchyme primordium formation in wk 5
|
|
|
Term
What are the effects of oligohydramnios? |
|
Definition
|
|
Term
What disorder does he have?
[image] |
|
Definition
Achondroplasia: mutation in FGFR3causing failure of long bone growth
Autosomal dominant: homozygous can be fatal |
|
|
Term
What is the most common lethal dwarfism? |
|
Definition
Thanatrophoric Dysplasia
FGFR3 defect
femurs are refered to as french telephone receiver
most die in newborn period
[image] |
|
|
Term
2 year old has history of multiple bone fractures with minor trauma, hepatosplenomegaly, palsies involving CN II, VII and VIII. He is treated with bone marrow stem cell transplantation. What cell in his bones was most likely disfunctional? |
|
Definition
Osteoclast: this child has osteopetrosis |
|
|
Term
What disorder is characterized by disorganized cartilage at growth plates and articular surfaces due to the inability of chondrocyte to catabolize GAG? |
|
Definition
Mucopolysaccharidoses[image] |
|
|
Term
|
Definition
AR lysosomal storage disease caused by deficiency in Glucocerebrosidase causing glucocerebroside to accumulate in macrophages
Gaucher cells: crumpled paper cytoplasm
Type I:nonneuropathic, milder
Sx:hepatosplenomegaly, bone erosion, fracture, pain
Tx:enzyme replacement
[image][image] |
|
|
Term
What is happening in this bone?
[image] |
|
Definition
Osteomyelitis
Tx: antibiotics, surgical drainage
Primariy caused by pyogenous bacteria-S.aureus (80%)
metaphysis usually affected in children
epiphysis and subchondral areas in adults |
|
|
Term
Who is most at risk for osteomyelitis caused by salmonella? |
|
Definition
Sickle cell patients
more prone to infections by encapsulated bacteria [image] |
|
|
Term
What are the significant bony changes that can occur in the setting of osteomyelitis? |
|
Definition
Sequestrum and Involucrum
Sequestrum: necrotic bone in acute inflammation
Involucrum: new bone forms a sleeve around dead infected bone [image] |
|
|
Term
This is a picture of? And what gene is it associated with?
[image] |
|
Definition
Osteochondroma
- inactivation of EXT1 gene in sporadic tumors
- encodes proteins for proteoglycan biosynthesis and reduced expression causes ineffective endochondral ossification
|
|
|
Term
What bone dysfunction is most often in young male, causing nocturnal pain and relieved by aspirin/NSAIDs ususally in the proximal femur?
What is causing the pain? |
|
Definition
Osteoid Osteoma
most common site is proximal femur
pain caused by prostaglandin E2 made by tumor
tumor nidus is radiolucent, well defined and circumscribed zone surrounded by sclerotic bone
[image] [image]
dont confuse with osteoblastoma which is mostly in spine and long bones with dull achy pain |
|
|
Term
What bone condition is characterized by woven bone in the middle of benign looking fibroblasts?
|
|
Definition
fibrous dysplasia
70%:monostic
polyostic can involve the carniofacial, pelvic, shoulder girdle regions and be more severe
[image] [image] [image] |
|
|
Term
What is this? What gene is associated?
[image] |
|
Definition
Aneurysmal Bone Cysts
benign, but rapidly growing
translocation 17p13 upregulating
USP6 gene which encodes a protease that upregulates NFkB which increases metalloproteases |
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Term
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Definition
Malignant osteoblasts making bone matrix
most common primary malignant bone tumor
bones of the knee most commonly
young patients
Tx:chemo followed by surgery
poor prognosis if metastasis to lungs
Rb gene mutation
p53 mutations
INK4a mutations and p14
[image] |
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Term
What is the most common primary malignant bone tumor? |
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Definition
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Term
Which bone pathologies share a translocation11/22 and over expression of c-myc? |
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Definition
Ewing sarcoma and Primitive neuralectoderm tumor
EWS and PNET
small blue cell tumors
affects adolescents and rarely AA
present w/ tender masses, fever, leukocytosis mimicking acute osteomyelitis
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Term
What does this child have? What other sx would you observe?
[image] |
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Definition
Hand-Schuller Chrisitan Disease
a type of Langerhans Cell Histiocytosis[image]
rare: lytic skull lesions, diabetes insipidus, exopthalmus
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Term
What are Bierback granules associated with? |
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Definition
Langerhans Cell Histiocytosis
[image] |
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Term
Sarcomas express which immunohistochemical stain element? |
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Definition
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Term
What is Paget Disease of bone? |
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Definition
osteoclast dysfunction: gain in bone mass
common in caucasians
Can be polyostotic or monostotic
increased risk of osteosarcoma
[image] |
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Term
What causes osteonecrosis? |
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Definition
aka avascular necrosis: ischemia from vessel damage, obstruction, embolism, injury caused by trauma, steroids, infection, XRT, connective tissue disease, alcohol, SS, idiopathic
usually causes damage to femoral head [image] |
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Term
Enchondromas usually occur where? |
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Definition
hands: increased risk for osteosarcoma
overgrowth of cartilage, benign adult bone tumor
[image] |
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Term
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Definition
Chondrosarcoma:painful enlarging mass, buly tumor, most de novo but can arise in endochondroma or osteosarcoma
adult bone tumor
common in axial skeleton,
rare in distal extremities
dedifferentiated: worse prognosis
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Term
What is a giant cell tumor? |
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Definition
Adult bone tumor: osteoclastoma
benign, locally aggressive, cystic degeneration
collection of mononuclear tumot cells expressing RANKL which promotes proliferation of osteoclasts
knee is common site
[image] |
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Term
What are the most common metastatic bone tumors in adults? |
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Definition
Carcinoma-lung, kidney, breast, prostate |
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Term
An elevated alkaline phosphate level in older adult should raise suspicion of what? |
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Definition
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Term
A positive Schober's Test is used to diagnose what? |
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Definition
Ankylosing Spondylitis
(remember assoc. with HLAB27) |
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Term
Joint aspiration reveals birefringent needle shaped crystals. Pt. has joint swelling and pain in right knee. What is the most likely diagnosis? |
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Definition
Acute gout
(remember parallel :yellow)
[image] |
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Term
What is the most common complication of Giant Cell arteritis? |
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Definition
ischemic optic neuropathy |
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Term
What drug should be inititated immediately if you suspect giant cell arteritis? |
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Definition
systemic corticosteroids to decrease risk of ischemic optic neuritis |
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Term
What neurotransmitters have altered levels in Anxiety? |
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Definition
decreased GABA
increased NE |
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Term
What arachadonic acid product opposes that of prostacyclin? |
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Definition
Thromboxane: platelet aggregate, increases vasclar tone, increases bronchial tone |
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Term
What part of the brain inhibits the limbic system so that we are not merely acting on instinct? |
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Definition
Preforntal cortex: decision making and allows for delayed gratification to exist[image] |
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Term
What part of the brain houses our instincts, considered the "animal brain" or the "id" by freud? |
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Definition
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Term
What region of the brain governs the circadian rhythm? |
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Definition
the suprachiasmatic nucleus of the hypothalamus
"You need sleep to be charismatic"
MELATONIN
[image] |
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Term
A lesion of this brain region would result in hyperthermia |
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Definition
the Anterior hypothalamus (also called preoptic:GnRH release)
Anterior/Cooling: A/C Please
Coolin and Parasympathetic
also releases GnRH
[image] |
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Term
A lesion of this area of the brain results in hypothermia |
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Definition
Posterior Hypothalamus
responsible for heating and sympathetic
[image] |
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Term
A lesion of this region of the brain results in neurogenic diabetes insipidus |
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Definition
Supraoptic nucleus of the hypothalamus
produces ADH and thus regulates water balance
[image] |
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Term
A lesion of this region of the brain would result in hyperphagia and obesity |
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Definition
Ventromedial nucleus of the hypothalamus stimulates satiety
stimulated by leptin
"If you injure your VM, you grow ventrally and medially"
[image] |
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Term
A lesion of this region of the brain leads to anorexia and failure to thrive |
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Definition
Lateral nucleus of the hypothalamus stimulates hunger and is thus inhibted by leptin
[image] |
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Term
What region of the brain makes oxytocin? |
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Definition
The paraventricular and supraoptic nuclei of the hypothalamus
[image]
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Term
Which area of the brain responds to emetics? |
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Definition
The area postrema which is not protected by the blood brain barrier
[image] |
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Term
Name as many nuclei of the hypothalamus and their function |
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Definition
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Term
What is the arcuate nucleus repsonsible for? |
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Definition
releases many hormones (CRT, GHRH, TRH, somatostatin, dopamine) to the anterior pituutary and regulates hunger and satiety
[image] |
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Term
Where does the neurohypophysis originate from embryologically? And where does it recieve its projections from? |
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Definition
neuroectoderm
recieves projections from supraoptic (ADH/vasopressin) and paraventricular(oxytocin) nuclei |
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Term
Which region of the brain regulate the Parasympathetic Nervous System? |
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Definition
The Anterior and the Preoptic Nuclei |
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Term
Which nucleus receives input from the retina? |
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Definition
The suprachiasmatic nucleus to regulate circadian rhythm (melatonin) |
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Term
Which region of the brain regulates the Sympathetic Nervous System? |
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Definition
the Posterior and Lateral Nuclei of the hypothalamus |
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Term
List the stages of Sleep and the associated EEG waveforms for each:
Awake(eyes open)
Awake(eyes closed)
Stage 1
Stage 2
Stage 3
REM |
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Definition
Awake (open):Beta
Awake (closed): Alpha
Stage 1 (light sleep): Theta
Stage 2 (45% of sleep here:deeper/bruxism): Sleep spindles and K complexes:
Stage 3 and 4(deepest nonREM-slow wave: sleep walking, night terrors, and bedwetting): Delta
REM: Beta (brain uses a lot of oxygen here;looks like u are awake)
"at nights, BATS Drink Blood |
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Term
What is the prinicipal neurotransmitter in REM sleep? |
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Definition
Acetylcholine
Think about REST and DIGEST
[image] |
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Term
How does depression affect sleep stages? |
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Definition
decreases slow wave sleep (stage 3 and 4)
Decreases REM latency (increases total REM)
increases REM early in sleep cycle
repreated nighttime awakenings
early morning awakening (screeening question)
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Term
The elderly spend more time in which sleep stage? |
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Definition
Stage 2
they have a hard time getting to REM sleep |
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Term
How often does REM sleep occur? |
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Definition
every 90 minutes
duration of REM increases through night
time to get to REM sleep decreases during the night
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Term
What predominance in the Raphe nucleus is essential to initiating sleep? |
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Definition
serotonergic predominance |
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Term
What neurotransmitter is essential for initiating sleep?
What neurotransmitter is essential for REM sleep? |
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Definition
Serotonin for initiation
Acetylcholine for REM |
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Term
What mediates horizontal eye movements (during REM and, Fast phase nastagmus, saccades)? |
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Definition
PPRF
Paramedian Pontine Reticular Formation
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Term
What do EtOH, barbituates and benzos do to sleep stages? |
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Definition
they decrease
REM sleep
and Delta sleep (slow wave: stage 3and 4)
so you sleep more like an old person and you lose high quality sleep |
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Term
How do you treat night terrors? |
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Definition
Benzos can be useful because they decrease Stage 3 and 4 Sleep when night terrors and sleep walking occur. |
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Term
What medications are used for insomnia? |
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Definition
melatonin: not very effective, causes vivid dreams, safe for less than 3 months
valerian and chamomile: OTC herbals
Antihistamines:poor sleep quality, not for long term, avoid in elderly
Trazadone: Antidepressent: increases REM sleep, small risk of priapism
TCAs: Antidepressant, small risk of arrhythmias, avoid in elderly
Long acting benzos: addictive, short term (less than 35 days)
Zolpidem: long acting benzo like
Lunesta: may be used long term
Ramelteon: non addictive bc acts on melatonin receptors instead of GABA/benzo receptors, not for hepatic pts |
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Term
What is the pathway by which retinal stimulation induces the release of melatonin? |
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Definition
Darkness causes suprachiasmatic nucleus to release NE
which stimulates the pineal gland to release melatonin |
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Term
What is this picture showing. It is an embryological anomaly that is lateral and does not move with swallowing. Why does it occur?
[image] |
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Definition
Branchial Cleft cyst caused by persistence of the cervical sinuses derived from he second to fourth branchial/pharyngeal clefts that should have been obliterated
[image]
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Term
The branchial/pharyngeal puches are derived from what embryologic tissue? |
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Definition
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Term
What does the third pharyngeal/branchial pouches become?
the fourth pouch? |
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Definition
Third: inferior parathyroid and the thymus
Fourth: superior parathyroid
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Term
Aberrant development of the third and fourth pharyngeal poouches can lead to what syndrome? |
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Definition
DiGeorge Syndrome
CATCH22
Cardiac defects
abnormal facies
thymic aplasia-->low T cells-->recurrent viral/fungal and other infections
Cleft palate
hypoCalcemia-->tetany
immature T cells
22q11 deletion |
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Term
What does Branchial/Pharyngeal Arch 1 give rise to? |
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Definition
M and T structures
mandible
meckel's cartilage
malleus
mandibular ligament
muscles of mastication
masseter
medial and lateral pterygoid
myohyoid
temporalis
antertior two thirds of the tongue
tensor tympani
tensor vali palatini
nerve innervation: mandibular and maxiallary branches of Trigeminal
[image]
[image]
[image]
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Term
Failure of the first pharyngeal arch neural crest cells to migrate causes what? |
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Definition
Treacher-Collins Syndrome (another T word)
facial abnormalities with usually little to no mental dysfunction
[image] |
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Term
What does the Branchial/Pharyngeal Arch 2 give rise to? |
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Definition
S structures
stapes
styloid process
styloid-hyoid ligament
lesser horn of hyoid
stapedius
innervation: cranial nerve Seven (7)
-muscles of facial expression
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Term
What does the branchial/Pharyngeal Arch 3 give rise to? |
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Definition
Pharyngeal:
stylo-pharyngeus muscle
innervation: glossopharyngeal nerve
congenital abnormality:pharyngo-cutaneous fistula
[image]
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Term
What do the Branchial/Pharyngeal Arches 4 and 6 give rise to? |
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Definition
CricoThyroid and Larynx
innervation: laryngeal branches of vagus nerve
innervate muscles of the larynx (except cricoid)
superior laryngeal: swallowing
recurrent laryngeal :speaking
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Term
Label the 12 cranial nerves
[image] |
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Definition
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Term
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Definition
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Term
Which cranial nerve is responsible for:
eyelid opening:
taste from anterior 2/3 of tongue:
head turning:
tongue movement: |
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Definition
eyelid opeing: CNIII (oculomotor)
taste from anterior 2/3 of tongue: CN VII (facial)
head turning: CN XI (accessory)
tongue movement: CN XII (hypoglossal) |
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Term
Which cranial nerve is responsible for:
muscles of mastication:
balance:
monitoring the carotid body and sinus chemo and baroreceptors: |
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Definition
muscles of mastication: CN V (Trigeminal)
balance:CN VIII (vestibulocochlear)
monitoring the carotid body and sinus chemo and baroreceptors: CN IX (glossopharyngeal) |
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Term
What would cause this abnormality?
[image] |
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Definition
lesion of CN VI (Abducens)
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Term
Name the lesion.
Eye looks down and out with ptosis, pupilary dilation and loss of accomodation |
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Definition
CN III
(uncal herniation) |
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Term
Name the lesion.
Eye moves upward, partcularly with contralateral gaze and ipsilateral head tilt. difficulty looking down causing diplopia (problems going down stairs) |
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Definition
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Term
Name the lesion.
medially directed eye that can't abduct. |
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Definition
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Term
How do you test for Inferior Oblique muscle of the eye functioning? |
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Definition
Have patient look UP
(IOU) |
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Term
How do you test for CN IV functioning? |
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Definition
Have the patient look down from adducted position |
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Term
|
Definition
KNOW IT!
OTOMAI
Occulomotor, Trochlear, Opthalmic, Maxillary, Abducens, Internal carotid |
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Term
What structures run through the cavernous sinus?
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Definition
Internal carotid artery, pituitary gland, extraocular muscle nerves (oculomotor, trochlear and abducens), V1 and V2 of trigeminal nerve (opthalmic and maxillary), postganglionic sympathetic fibers en route to orbit
[image] |
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Term
What are the 5 branches of the Facial Nerve? |
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Definition
To Zanzibar By Motor Car
Temporal
Zygomatic
Buccal
Mandibular
Cervical
[image] |
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Term
What muscles cause the jaw to close?
And what is their innervation? |
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Definition
Masseter
Temporalis
Medial pterygoid
(all innervated by mandibular division of trigeminal nerve)
[image] |
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Term
What muscle causes the jaw to open? And what is the innervation? |
|
Definition
Lateral pterygoid
mandibular division of trigeminal nerve
[image]
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Term
What structure does the facial nerve go through? |
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Definition
The parotid gland
[image] |
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Term
What innervates the parotid gland? |
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Definition
glossopharyngeal nerve
CN IX |
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Term
Having the patient say:
Mi mi mi
la la la
khu khu khu
tests what nerves? |
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Definition
mimimi: facial (muscles of mastication)
lalala: hypoglossal (tongue)
khukhukhu: vagus (elevation of palate) |
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Term
Which CN lesions would cause vertigo, nystagmus and nausea and vomiting? |
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Definition
CN VIII
VESTIBULOCOCHLEAR |
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Term
Where are the CN nuclei located in brainstem?
|
|
Definition
CN III & IV: midbrain
CN V, VI, VII, VIII: pons
CN IX, X, XI, XII: medulla
[image]
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Term
Name the three vagal nuclei and their function |
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Definition
nucleus SolitArius: taste, baroreceptors, gut distension
Nucleus Ambiguus: motor to pharynx, larynx and upper esophagus
Dorsal Motor nucleus:: misnomer-autonomic information to heart, lungs, upper GI
NASA Motor
Nsolitarius: think about eating alone
Nambiguus: think A for Action
Dorsal motor: think misnomer
[image]
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Term
A lesion to the Nucleus Ambiguus would cause what symptoms? |
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Definition
Hoarseness
difficulty swallowing
loss of gag reflex |
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Term
What are the three main regulators of the Parasympathetic Nervous System? |
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Definition
Preoptic area of hypothalamus
Dorsal Motor nucleus
Vagus Nerve |
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Term
What are the main structures of the Sympathetic Nervous System? |
|
Definition
Posterior and Lateral Hypothalamus
Sympathetic Ganglion T1-L3 |
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|
Term
What is Horner's Syndrome? |
|
Definition
injury to the superior cervical ganglion causing ptosis, miosis, anhydrosis
[image]
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Term
What segment of the renal tubule reabsorbs 67% of the fluid and electrolytes filtered by the glomerulus? |
|
Definition
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|
Term
Which segment of the nephron is responsible for concentrating the urine |
|
Definition
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Term
Which part of the nephron is the site of secretion of organic anions and cations? |
|
Definition
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Term
Which part of the nephron is always impermeable to water? |
|
Definition
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Term
Which part of the nephron is permeable to water only in the presence of ADH? |
|
Definition
Late distal tubule and collecting duct |
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Term
Which part of the nephron is the site of the Na/2Cl/K co-transporter? |
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Definition
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Term
Which part of the nephron is responsible for diluting urine? |
|
Definition
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|
Term
Where is urine concentrated in the nephron? |
|
Definition
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|
Term
Where is the site of water reabsorption in the loop of henle? |
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Definition
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Term
What effect does aldosterone have on the intercalated cells and principal cells of the collecting duct? |
|
Definition
intercalated cells: aldosterone stimulates acid secretion
prinicipal cells: aldosterone increases Na reabsorption and K secretion
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Term
What class of drugs inhibit the Na/Cl/K symtransporter in the thick ascending limb? |
|
Definition
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|
Term
Which diuretic is good for altitude sickness? |
|
Definition
Acetazolamide
respiratory alkalosis from hyperventialtion so acetazolamide is a
carbonic anhydrase inhibitor to help you excrete bicarb and alkalanize the urine
also treats glaucoma
SULFA DRUG
can cause metablic acidosis |
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Term
Which diuretic can cause gynecomastia? |
|
Definition
Spirinolactone
K sparing, aldosterone antagonist but binds to androgen receptors; therefore, can be used to treat acne and PCOS |
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Term
Which diuretics are known to cause contraction alkalosis? |
|
Definition
LOOP diuretics and Thiazides |
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|
Term
What class of drug is ethacrynic acid? |
|
Definition
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Term
Which diuretic would you use for CHF, pulmonary edema and edema associated with nephrotic syndrome? |
|
Definition
LOOP diuretics
(furosemide..etc) |
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|
Term
Which diuretic would you use to treat mild to moderate HTN? |
|
Definition
Thiazide diuretic
acts at the Distal tubule |
|
|
Term
Name the lesion
scapular winging |
|
Definition
|
|
Term
name the lesion
loss of forearm pronation |
|
Definition
|
|
Term
name the lesion
cannot abduct or adduct fingers |
|
Definition
interosseus muscles innervated by the ulnar nerve |
|
|
Term
name the nerve damaged
weak lateral rotation of th arm |
|
Definition
suprascapular nerve, axillary nerve |
|
|
Term
name the damaged nerve
unable to abduct arm beyond 10 degrees |
|
Definition
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|
Term
|
Definition
|
|
Term
nephropathy and AV nodal block |
|
Definition
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|
Term
port wine stain in the opthalmic division of the trigeminal nerve |
|
Definition
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|
Term
hematuria+ fever+ rash+eosiniphilia |
|
Definition
acute interstitial nephritis
can be drug induced and treated with corticosteroids |
|
|
Term
Thyroidization of kidneys |
|
Definition
|
|
Term
Cancer associated with schistosoma haematobium |
|
Definition
|
|
Term
What neoplasm is associated with hashimotos thyroiditis? |
|
Definition
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|
Term
What neoplasm is associated with down's syndrome
|
|
Definition
|
|
Term
What neoplasm is associated with tuberous sclerosis? |
|
Definition
cardiac rhabdomyoma
astrocytoma
angiomyolipoma |
|
|
Term
What neoplasm is ataia telangiectasia associate with? |
|
Definition
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|
Term
What neoplasm is Paget's disease of the bone associated with? |
|
Definition
Osteosarcoma and Fibrosarcoma |
|
|
Term
Which vitamin deficiency can be caused by isoniazid use? |
|
Definition
|
|
Term
gram negative oxidase positive diplococci |
|
Definition
|
|
Term
gram negative bacteria that only stains on silver |
|
Definition
Legionella
test urine if suspect the cause of pneumonia |
|
|
Term
which gram negative bug causes corneal infections in contact lens wearers |
|
Definition
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|
Term
Which drug binds to the 23s rRNA of the 50s subunit and inhibits the bacterial integration complex? |
|
Definition
Linezolid (oral)
used for MRSA and VRE
Macrolides to doo |
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|
Term
What can you NOT take tetracyclines with? |
|
Definition
Milk (calcium)
magnesium
iron-containing preparations
|
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|
Term
What are the sude effects of the aminoglycosides? |
|
Definition
Nephrotoxic
Ototoxic
Teratogenic
"NOT" |
|
|
Term
What drugs have photosensitivity reactions? |
|
Definition
"SAT"
Sulfonamides
Amiodarone
Tetracyclines |
|
|
Term
What causes grey baby syndrome? |
|
Definition
grey baby: Chloramphenicol
Grey MAN: Amiodarone
RED MAN: Vancomycin |
|
|
Term
What kind of antibiotic is Gentamycin? |
|
Definition
|
|
Term
What kind of antibiotic is Clindamycin? |
|
Definition
|
|
Term
What are the clinical uses of Macrolides? |
|
Definition
URIs
Pneumonia
STDs
gram positive infections in Penicillin-allergic pts
Atypical pneumonia |
|
|
Term
Which virus is associated with erythema infectiosum? |
|
Definition
|
|
Term
|
Definition
|
|
Term
Only DNA virus that is not double stranded
|
|
Definition
|
|
Term
Heterophil negative mononucleosis |
|
Definition
|
|
Term
What antibiotics are safe during pregnancy? |
|
Definition
Penicillins
Piperacillin
Cephalosporins
Macrolides
Metronidazole AFTER 1ST trimester
Nitrifurantoin (esp for UTIs) |
|
|
Term
|
Definition
|
|
Term
What antiviral inhibits CMV DNA polymerase? |
|
Definition
|
|
Term
Which antivirals can be used as prophylaxis for influenza A? |
|
Definition
|
|