Shared Flashcard Set

Details

Step 1-last test for derm and DIT A-B
derm, cell cycle, inflammation, intro neuro and embryo
255
Medical
Graduate
02/25/2015

Additional Medical Flashcards

 


 

Cards

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
What is I-cell disease?
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
Decidua basalis
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
syncytiotrophoblast
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
VITAMIN A
Term
What molecules provide the structural framework for DNA and the nuclear envelope?
Definition
nuclear lamins
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
MANNOSE-6-POSPHATE
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
Transcription factor E2F
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
Clathrin
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
TNF-alpha
Term
What is IMFLIXIMAB?
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
hepatocytes
Term
What cell is most repsonsible for the ACUTE phase of inflammation?
Definition
NEUTROPHILS
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
the midbrain
Term
What does the Metencephalon become?
Definition
the Pons and the Cerebellum
Term
What does the Myelencephalon become?
Definition
the medulla
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
decrease in AFP
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
What is Nissl substance?
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
[image]
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
Glycine
Term
What is the main excitatory neurotransmitter of the CNS?
Definition
Glutamine
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
What is dermatomyositis?
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
What is scleroderma?
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
Henoch-Schonlein Purpura
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
Hep B
Term
Anti-dsDNA and C3 and C4 are often markers of this immune complex disease
Definition
SLE
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
What is Icthyosis?
Definition

dry, thickened, scaly or flaky skin

commonly genetic

[image]

Term
What is harlequin fetus?
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

What is this ?

[image]

Definition
Cystic Hygroma: benign tumor of lymphatics
Term

What is this?

[image]

Definition
Strawberry hemangioma (aka nevus): benign endothelial cell tumor most common tumor in infants, children
Term
What is Albinism?
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
[image]
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
What is Gaucher Disease?
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

Term
What is an osteosarcoma?
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]

Term
What is the most common primary malignant bone tumor?
Definition
osteosarcoma
Term
Which bone pathologies share a translocation11/22 and over expression of c-myc?
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

 

Term

 

 

What does this child have? What other sx would you observe?

[image]

Definition

Hand-Schuller Chrisitan Disease

a type of Langerhans Cell Histiocytosis[image]

rare: lytic skull lesions, diabetes insipidus, exopthalmus

 

Term
What are Bierback granules associated with?
Definition

Langerhans Cell Histiocytosis

[image]

Term
Sarcomas express which immunohistochemical stain element?
Definition
vimentin
Term
What is Paget Disease of bone?
Definition

osteoclast dysfunction: gain in bone mass

common in caucasians

Can be polyostotic or monostotic

increased risk of osteosarcoma

[image]

Term
What causes osteonecrosis?
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]

Term
Enchondromas usually occur where?
Definition

hands: increased risk for osteosarcoma

overgrowth of cartilage, benign adult bone tumor

[image]

Term
What is this? [image]
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

 

Term
What is a giant cell tumor?
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]

Term
What are the most common metastatic bone tumors in adults?
Definition
Carcinoma-lung, kidney, breast, prostate
Term
An elevated alkaline phosphate level in older adult should raise suspicion of what?
Definition
bone metastases
Term
A positive Schober's Test is used to diagnose what?
Definition

Ankylosing Spondylitis

(remember assoc. with HLAB27)

Term
Joint aspiration reveals birefringent needle shaped crystals. Pt. has joint swelling and pain in right knee. What is the most likely diagnosis?
Definition

Acute gout

(remember parallel :yellow)

[image]

Term
What is the most common complication of Giant Cell arteritis?
Definition
ischemic optic neuropathy
Term
What drug should be inititated immediately if you suspect giant cell arteritis?
Definition
systemic corticosteroids to decrease risk of ischemic optic neuritis
Term
What neurotransmitters have altered levels in Anxiety?
Definition

decreased GABA

increased NE

Term
What arachadonic acid product opposes that of prostacyclin?
Definition
Thromboxane: platelet aggregate, increases vasclar tone, increases bronchial tone
Term
What part of the brain inhibits the limbic system so that we are not merely acting on instinct?
Definition
Preforntal cortex: decision making and allows for delayed gratification to exist[image]
Term
What part of the brain houses our instincts, considered the "animal brain" or the "id" by freud?
Definition

Limbic system

[image]

Term
What region of the brain governs the circadian rhythm?
Definition

the suprachiasmatic nucleus of the hypothalamus

"You need sleep to be charismatic"

MELATONIN

[image]

Term
A lesion of this brain region would result in hyperthermia
Definition

the Anterior hypothalamus (also called preoptic:GnRH release)

Anterior/Cooling: A/C Please

Coolin and Parasympathetic

 also releases GnRH

[image]

Term
A lesion of this area of the brain results in hypothermia
Definition

Posterior Hypothalamus

responsible for heating and sympathetic

[image]

Term
A lesion of this region of the brain results in neurogenic diabetes insipidus
Definition

Supraoptic nucleus of the hypothalamus

produces ADH and thus regulates water balance

[image]

Term
A lesion of this region of the brain would result in hyperphagia and obesity
Definition

Ventromedial nucleus of the hypothalamus stimulates satiety

stimulated by leptin

"If you injure your VM, you grow ventrally and medially"

[image]

Term
A lesion of this region of the brain leads to anorexia and failure to thrive
Definition

Lateral nucleus of the hypothalamus stimulates hunger and is thus inhibted by leptin

[image]

Term
What region of the brain makes oxytocin?
Definition

The paraventricular and supraoptic nuclei of the hypothalamus

[image]



Term
Which area of the brain responds to emetics?
Definition

The area postrema which is not protected by the blood brain barrier

[image]

Term
Name as many nuclei of the hypothalamus and their function
Definition
[image]
Term
What is the arcuate nucleus repsonsible for?
Definition

releases many hormones (CRT, GHRH, TRH, somatostatin, dopamine) to the anterior pituutary and regulates hunger and satiety

[image]

Term
Where does the neurohypophysis originate from embryologically? And where does it recieve its projections from?
Definition

neuroectoderm

recieves projections from supraoptic (ADH/vasopressin) and paraventricular(oxytocin) nuclei

Term
Which region of the brain regulate the Parasympathetic Nervous System?
Definition
The Anterior and the Preoptic Nuclei
Term
Which nucleus receives input from the retina?
Definition
The suprachiasmatic nucleus to regulate circadian rhythm (melatonin)
Term
Which region of the brain regulates the Sympathetic Nervous System?
Definition
the Posterior and Lateral Nuclei of the hypothalamus
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

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

Term
What is the prinicipal neurotransmitter in REM sleep?
Definition

Acetylcholine

 

Think about REST and DIGEST

 

[image]

Term
How does depression affect sleep stages?
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)

 

Term
The elderly spend more time in which sleep stage?
Definition

Stage 2

they have a hard time getting to REM sleep

Term
How often does REM sleep occur?
Definition

every 90 minutes

duration of REM increases through night

time to get to REM sleep decreases during the night

 

Term
What predominance in the Raphe nucleus is essential to initiating sleep?
Definition
serotonergic predominance
Term

What neurotransmitter is essential for initiating sleep?

What neurotransmitter is essential for REM sleep?

Definition

Serotonin for initiation

Acetylcholine for REM

Term
What mediates horizontal eye movements (during REM and, Fast phase nastagmus, saccades)?
Definition

PPRF

Paramedian Pontine Reticular Formation

 

 

Term
What do EtOH, barbituates and benzos do to sleep stages?
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

Term
How do you treat night terrors?
Definition
Benzos can be useful because they decrease Stage 3 and 4 Sleep when night terrors and sleep walking occur.
Term
What medications are used for insomnia?
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

Term
What is the pathway by which retinal stimulation induces the release of melatonin?
Definition

Darkness causes suprachiasmatic nucleus to release NE

which stimulates the pineal gland to release melatonin

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]

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]

 

Term
The branchial/pharyngeal puches are derived from what embryologic tissue?
Definition
endoderm
Term

What does the third pharyngeal/branchial pouches become?

the fourth pouch?

Definition

Third: inferior parathyroid and the thymus

Fourth: superior parathyroid

 

 

Term
Aberrant development of the third and fourth pharyngeal poouches can lead to what syndrome?
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

Term
What does Branchial/Pharyngeal Arch 1 give rise to?
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]

 

 

Term
Failure of the first pharyngeal arch neural crest cells to migrate causes what?
Definition

Treacher-Collins Syndrome (another T word)

facial abnormalities with usually little to no mental dysfunction

[image]

Term
What does the Branchial/Pharyngeal Arch 2 give rise to?
Definition

S structures

stapes

styloid process

styloid-hyoid ligament

lesser horn of hyoid

stapedius

innervation: cranial nerve Seven (7)

-muscles of facial expression

 

 

Term
What does the branchial/Pharyngeal Arch 3 give rise to?
Definition

Pharyngeal:

stylo-pharyngeus muscle

innervation: glossopharyngeal nerve

congenital abnormality:pharyngo-cutaneous fistula

[image]

 

Term
What do the Branchial/Pharyngeal Arches 4 and 6 give rise to?
Definition

CricoThyroid and Larynx

innervation: laryngeal branches of vagus nerve

innervate muscles of the larynx (except cricoid)

superior laryngeal: swallowing

recurrent laryngeal :speaking

 

 

 

Term

 

 

Label the 12 cranial nerves

[image]

Definition
[image]
Term

 

know this

[image]

 

 

Definition
KNOW IT
Term

Which cranial nerve is responsible for:

eyelid opening:

taste from anterior 2/3 of tongue:

head turning:

tongue movement:

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)

Term

Which cranial nerve is responsible for:

muscles of mastication:

balance:

monitoring the carotid body and sinus chemo and baroreceptors:

Definition

muscles of mastication: CN V (Trigeminal)

balance:CN VIII (vestibulocochlear)

monitoring the carotid body and sinus chemo and baroreceptors: CN IX (glossopharyngeal)

Term

What would cause this abnormality?

[image]

Definition

lesion of CN VI (Abducens)

 

Term

Name the lesion.

Eye looks down and out with ptosis, pupilary dilation and loss of accomodation

Definition

CN III

(uncal herniation)

Term

Name the lesion.

Eye moves upward, partcularly with contralateral gaze and ipsilateral head tilt. difficulty looking down causing diplopia (problems going down stairs)

Definition

 

 

CN IV

[image]

Term

Name the lesion.

medially directed eye that can't abduct.

Definition
CN VI
Term
How do you test for Inferior Oblique muscle of the eye functioning?
Definition

Have patient look UP

 

(IOU)

Term
How do you test for CN IV functioning?
Definition
Have the patient look down from adducted position
Term
Know this [image]
Definition

KNOW IT!

 

OTOMAI

 

Occulomotor, Trochlear, Opthalmic, Maxillary, Abducens, Internal carotid

Term

What structures run through the cavernous sinus?

 

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]

Term
What are the 5 branches of the Facial Nerve?
Definition

To Zanzibar By Motor Car

Temporal

Zygomatic

Buccal

Mandibular

Cervical

[image]

Term

What muscles cause the jaw to close?

And what is their innervation?

Definition

Masseter

Temporalis

Medial pterygoid

(all innervated by mandibular division of trigeminal nerve)

[image]

Term
What muscle causes the jaw to open? And what is the innervation?
Definition

Lateral pterygoid

mandibular division of trigeminal nerve

[image]

 

Term
What structure does the facial nerve go through?
Definition

 The parotid gland

[image]

Term
What innervates the parotid gland?
Definition

glossopharyngeal nerve

CN IX

Term

Having the patient say:

Mi mi mi

la la la

khu khu khu

 

tests what nerves?

Definition

mimimi: facial (muscles of mastication)

lalala: hypoglossal (tongue)

khukhukhu: vagus (elevation of palate)

Term
Which CN lesions would cause vertigo, nystagmus and nausea and vomiting?
Definition

CN VIII

VESTIBULOCOCHLEAR

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]

 

 

 

 

Term
Name the three vagal nuclei and their function
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]

 

 

Term
A lesion to the Nucleus Ambiguus would cause what symptoms?
Definition

Hoarseness

difficulty swallowing

loss of gag reflex

Term
What are the three main regulators of the Parasympathetic Nervous System?
Definition

Preoptic area of hypothalamus

Dorsal Motor nucleus

Vagus Nerve

Term
What are the main structures of the Sympathetic Nervous System?
Definition

Posterior and Lateral Hypothalamus

Sympathetic Ganglion T1-L3

Term
What is Horner's Syndrome?
Definition

injury to the superior cervical ganglion causing ptosis, miosis, anhydrosis

[image]

 

Term
What segment of the renal tubule reabsorbs 67% of the fluid and electrolytes filtered by the glomerulus?
Definition
Proximal tubule
Term
Which segment of the nephron is responsible for concentrating the urine
Definition
collecting duct
Term
Which part of the nephron is the site of secretion of organic anions and cations?
Definition
Proximal tubule
Term
Which part of the nephron is always impermeable to water?
Definition
thick ascending limb
Term
Which part of the nephron is permeable to water only in the presence of ADH?
Definition
Late distal tubule and collecting duct
Term
Which part of the nephron is the site of the Na/2Cl/K co-transporter?
Definition
Thick ascending limb
Term
Which part of the nephron is responsible for diluting urine?
Definition
thick ascending limb
Term
Where is urine concentrated in the nephron?
Definition
collecting duct
Term
Where is the site of water reabsorption in the loop of henle?
Definition
thin descending limb
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

 

Term
What class of drugs inhibit the Na/Cl/K symtransporter in the thick ascending limb?
Definition
LOOP diuretics
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

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

Term
Which diuretics are known to cause contraction alkalosis?
Definition
LOOP diuretics and Thiazides
Term
What class of drug is ethacrynic acid?
Definition
non sulfa loop diuretic
Term
Which diuretic would you use for CHF, pulmonary edema and edema associated with nephrotic syndrome?
Definition

LOOP diuretics

(furosemide..etc)

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
long thoracic nerve
Term

name the lesion

 

loss of forearm pronation

Definition
median nerve
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
axillary nerve
Term
waxy casts in urine
Definition
end stage renal disease
Term
nephropathy and AV nodal block
Definition
late lyme disease
Term
port wine stain in the opthalmic division of the trigeminal nerve
Definition
Sturge Weber syndrome
Term
hematuria+ fever+ rash+eosiniphilia
Definition

acute interstitial nephritis

 

can be drug induced and treated with corticosteroids

Term
Thyroidization of kidneys
Definition
chronic pyelonephritis
Term
Cancer associated with schistosoma haematobium
Definition
SCC of bladder
Term
What neoplasm is associated with hashimotos thyroiditis?
Definition
Lymphomas
Term

What neoplasm is associated with down's syndrome

 

Definition
ALL and AML
Term
What neoplasm is associated with tuberous sclerosis?
Definition

cardiac rhabdomyoma

astrocytoma

angiomyolipoma

Term
What neoplasm is ataia telangiectasia associate with?
Definition
Leukemias and lymphomas
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
B6 pyridoxine
Term
gram negative oxidase positive diplococci
Definition
Neisseria
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
Pseudomonas
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

Term
What can you NOT take tetracyclines with?
Definition

Milk (calcium)

magnesium

iron-containing preparations

 

 

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
Aminoglycoside
Term
What kind of antibiotic is Clindamycin?
Definition
Lincosamide
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
Parvovirus B19
Term
Downey cells
Definition
EBV
Term

Only DNA virus that is not double stranded

 

Definition
Parvovirus
Term
Heterophil negative mononucleosis
Definition
CMV
Term
What antibiotics are safe during pregnancy?
Definition

Penicillins

Piperacillin

Cephalosporins

Macrolides

Metronidazole AFTER 1ST trimester

Nitrifurantoin (esp for UTIs)

Term
break bone fever
Definition
dengue
Term
What antiviral inhibits CMV DNA polymerase?
Definition

Foscarnet

Ganciclovir

Term
Which antivirals can be used as prophylaxis for influenza A?
Definition

Zanamivir

Oseltamivir

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