Step Review: Biochem, Cardio, Endocrine, Neuro Flashcards
1
Q
What two amino acids are histones rich in?
A
Lysine and Arginine (Positive charge)
2
Q
Which strand of DNA is methylated in DNA replication?
A
Template strand (helps with mismatch repair)
3
Q
What 3 amino acids are required for purine synthesis?
A
GAG- Glycine, Aspartate, Glutamine
4
Q
What enzyme is impaired in orotic acicduria?
A
UMP Synthase
5
Q
What is the MOA of mycophenolate and ribavirin?
A
Inhibit inosine monophosphate dehydrogenase (final step in purine synthesis
6
Q
What enzyme is the rate limiting step in the production of IMP from Ribose-5 phosphate?
A
PRPP synthetase
7
Q
In the Lac operon, where does the repressor bind?
A
Operator
8
Q
List the steps of the cell cycle in order. When does nucleotide excision repair occur? Mismatch Repair?
A
M-G1-S-G2-repeat
G1- NER
G2- MMR
9
Q
Mutations in non-homologous end joining can result in what?
A
Fanconi anemia, Ataxia Telangiectasia
10
Q
Describe RNA poly I, II and III. Which RNA is the most numerous? Largest? Smallest?
A
Poly I: makes rRNA (nucleolus- most numerous)
Poly II: Makes mRNA (largest)
Poly III: makes tRNA (smallest)
11
Q
What is the mechanism of action of alpha-amanitin toxin?
A
Inhibits RNA polymerase II
12
Q
Where do the processes of adding the 5’ cap, polyA tail and splicing happen? Not all necessarily the same place?
A
Nucleus (hnRNA–>mRNA)
13
Q
What are anti-Smith antibodies actually reactive to? What is associated with anti-U1 RNP antibodies?
A
snRNPs, mixed connective tissue disease
14
Q
Which end and with what sequence do tRNAs bind amino acids?
A
3’ end with CCA
15
Q
Does tRNA loading require energy? What about initiation of protein synthesis?
A
Yes, 1 ATP for loading, 1 GTP for initiation
16
Q
What is occurring when Rb is hypophosphorylated? What phosphorylates it?
A
Cell cycle inhibition as Rb binds and inactivates transcription factor E2F. If a CDK phosphorylates RB–> cell cycle can progress through G1-S checkpoint. (Note: cyclins activate CDKs)
17
Q
Where are N-linked oligosaccharides added? O-linked?
A
N-RER
O-Golgi
18
Q
What is the defect in I-cell disease?
A
Failure of golgi to phosphorylate mannose residues due to defect in N-acetylglucosaminyl-1-phosphotransferase. This means proteins are exported rather than sent to lysosomes
19
Q
Coarse facial features, clouded corneas, restricted joint movement, and high plasma levels of lysosomal enzymes
A
I-cell disease (often fatal in childhood)
20
Q
What traffics proteins from:
1) Ribosome to RER?
2) Golgi to ER (retrograde)
3) ER to golgi (anterograde)
4) Trans golgi to lysosomes and plasma membrane to endosomes
A
1) Signal recognition particle (SRP)
2) COP I
3) COP II
4) Clathrin
21
Q
Are microvilli a form of microtubule?
A
No!!
22
Q
What has the 9+2 arrangement of microtubules?
A
Cilia (use dynein cross links to move)
23
Q
Where is the collagen triple helix formed(involves hydroxylation)? Where is collagen cross linked? What cofactors are needed for each?
A
In RER (vitamin C), extracellularly (copper for lysyl oxidase)
24
Q
Hyperextensible skin, tendency to bleed and hyper mobile joints? May also be associated with what vascular problem?
A
Ehlers-Danlos, berry and aortic aneurysms (joint and skin due to type V collagen, vascular problems with type III)
25
What gives elastin its elastic properties?
Cross-linking (this occurs extracellularly)
26
What technique is used to detect since nucleotide polymorphisms and copy number variations?
Microarrays
27
What is RNAi?
RNA interference involves dsRNA that is complementary to the mRNA sequence of interest. When transfected to human cells, dsRNA separates and promotes degradation of the target mRNA
28
Unilateral cafe-au-lair spots, polyostotic fibrous dysplasia, precocious puberty? What is the defect? What allows survival?
McCune-Albright Syndrome. Defect in G-protein signaling. Can only survive if patient has mosaicism (lethal if affects all cells)
29
Heterodisomy indicates an error in what stage of replication?
Meiosis I
30
Inheritance pattern of hypophosphatemic rickets (Vitamin D resistant rickets)?
X-linked dominant (increased phosphate wasting at proximal tubule)
31
Inheritance of Alport syndrome?
X-linked dominant
32
Mitochondrial encephalopathy, lactic acidosis and strokes? Inheritance?
MELAS, mitochondrial (ragged red fibers) Failure of oxidative phosphorylation causes it
33
Inheritance of hereditary spherocytosis
AD
34
What is the mutation in Li-Fraumeni syndrome?
p53 mutation (multiple malignancies)
35
Disease with numerous hamartomas?
Tuberous sclerosis
36
Chromosome for CF?
Chromosome 7 deletion of Phe508
37
What do you suspect if you see increased immunoreactive trypsinogen on a newborn screen?
Cystic Fibrosis
38
Difference in mutations in Duchenne vs Becker?
Duchenne is a deletion in dystrophin, Becker is a non-frameshift insertion
39
Common cause of death in Duchenne MD?
Dilated cardiomyopathy
40
What occurs in FMR1 gene in Fragile X syndrome to decrease expression?
Methylation
41
What is the inheritance pattern for myotonic dystrophy?
AD
42
Elfin face, intellectual disability, hypercalcemia, extreme friendliness, CV problems
Williams syndrome (C7)
43
Vitamin that can cause alopecia, hepatic toxicity, and pseudo tumor cerebri?
Vitamin A
44
Administration of what vitamin will result in increased transketolase activity if it is deficient?
Vitamin B1 (thiamine)
45
Vitamin deficiency with cheilosis and corneal vascularization?
B2
46
What is required for niacin synthesis?
Vitamins B2 and B6 plus tryptophan
47
Diarrhea, Dementia and Dermatitis?
Vitamin B3 deficiency
48
What is hartnup disease?
Deficiency of neutral acid transporters in PCT (e.g. tryptophan)
49
What vitamin is vital to transamination?
Vitamin B6
50
What type of reaction does the vitamin that can be depleted by raw egg whites partake in?
Carboxylation
51
What vitamin is needed for dopamine beta hydroxylase? What does this enzyme do?
Vitamin C, dopamine to NE
| Vitamin C also helps absorb iron (promotes Fe3+-->Fe2+)
52
What if you see a neurologic presentation similar to vitamin B12 deficiency with no megaloblastic anemia or hyper segmented neutrophils? May even see acanthocytosis and muscle weakness
Vitamin E deficiency (antioxidant)
53
Deficiency if you cannot smell and have poor wound healing?
Zinc
54
What is fomepizole?
Inhibitor of alcohol dehydrogenase (antidote for methanol or ethylene glycol poisoning)
55
What three processes occur in both mitochondria and cytoplasm?
Heme synthesis, urea cycle, gluconeogenesis
56
Major regulator of urea cycle?
N-acetylglutamate
57
Major rate limiting enzymes in purine and pyrimidine synthesis?
Purine: Glutamine phosphoribosylpyrophosphate (PRPP) amidotransferase
Pyrimidine: Carbamoyl phosphate synthetase II
58
What are the roles of HMG-CoA synthase and reductase?
Synthase: Ketogenesis
Reductase: Cholesterol synthesis
59
Which shuttle produces 32 ATP in heart/liver and which produces 30 in muscle?
32- malate-aspartate
| 30: glycerol-3-phosphate
60
What mutation leads to maturity-onset diabetes of the young?
Glucokinase
61
Name the 5 cofactors required for pyruvate dehydrogenase. Which does arsenic inhibit? What other enzyme is virtually the same?
Thiamine, lipoic acid, CoA(B5), FAD, NAD
Arsenic inhibits lipoid acid
alpha-ketoglutarate dehydrogenase
62
What builds up with a pyruvate dehydrogenase complex deficiency?
Lactic acid and alanine
63
What enzyme converts pyruvate to oxaloacetate for gluconeogenesis?
Pyruvate carboxylase (B7)
64
Which enzyme in the TCA cycle is embedded in the inner mitochondrial membrane and used in Ox Phos?
Succinate dehydrogenase
65
At what complex to cyanide and carbon monoxide work?
Complex IV
66
What is a direct inhibitor of ATP synthase?
Oligomycin
67
Which enzyme in gluconeogenesis is in the mitochondria? Required cofactors?
pyruvate carboxylase, B7, ATP
68
Which gluconeogenesis enzyme utilizes GTP? What TCA step can produce this?
PEP carboxykinase (succinyl CoA synthetase)
69
What part of FA can enter gluconeogenesis?
Only odd chain via propionyl-CoA entering as succinyl CoA via malonyl CoA (Even chain cannot enter)
70
Failure to track objects or develop a social smile in an infant?
Galactokinase deficiency
71
What amino acid contributes one amine group in urea?
Aspartate
72
What does tyrosinase do?
Tyrosine-->DOPA-->melanin
73
What is homogentisic acid in the degradation process of?
Tyrosine to fumarate
74
What amino acids are not reabsorbed in cystinuria? Where?
COLA- Cystine, Ornithine, Lysine, Arginine in PCT and intestinal amino acid transporters
75
What does a positive urinary cyanide-nitroprusside test indicate?
Cystinuria
76
What form of glucose is needed for glycogen synthesis by glycogen synthase?
UDP-glucose
77
Increased glycogen in liver, increased lactate, increased triglycerides, increased uric acid, and hepatomegaly? Enzyme?
Von Gierke- Glucose 6 phosphatase
78
Why in mcardle disease do you see a second-wind phenomenon?
Increased muscular blood flow. Muscle glycogen phosphorylase
79
Episodic peripheral neuropathy, angiokeratomas, hypohidrosis? Enzyme? What builds up? What kills them?
Fabry disease, alpha-galactosidase A, ceramide trihexoside, renal failure or CV disease late
80
pancytopenia, HSM, bone problems/pain. Enzyme?
Gaucher (glucocerebrosidase)
81
What builds up in Tay-Sachs?
GM2 gangliosidase
82
Gargoylism, corneal clouding, HSM. Enzyme?
Hurler (alpha-L-iduronidase)
83
Two causes of hypoketoic hypoglycemia?
Carnitine deficiency or acyl-CoA dehydrogenase deficiency
84
In alcoholism, where is oxaloacetate shunted? Why?
Malate, regenerates NAD+
85
How long in starvation does glycogen last?
1 day
86
What molecule inhibits the carnitine shuttle? Where does it come from?
If fatty acid synthesis is occurring, malonyl-CoA is being used and inhibits the carnitine shuttle and thus stops futile creation of FA
87
What apolipoprotein is a LPL cofactor? What disease is this implicated in?
C-II, type I familial dyslipidemia (hyperchylomicronemia)
88
What apolipoprotein is associated with chylomicron secretion? Remnant uptake? LDL receptor binding?
B-48
E
B-100
89
Which two lipoproteins carry the most cholesterol?
HDL and LDL (in opposite directions)
90
How is LDL taken up in target tissues?
Receptor mediated endocytosis
91
Which two apolipoproteins are "stored" on HDL?
E and C
92
What does alcohol do to HDL synthesis?
Increases it
93
What is the problem in type IIa familial hypercholesterolemia? Inheritance?
Absent/defective LDL receptors, AD
94
What is type IV dyslipidemia? What can result?
HyperTGemia, overproduction of VLDL by liver. High TGs can cause acute pancreatitis
95
What is the prosencephalon?
Forebrain-->Telencephalon and Diencephalon(thalamus and hypothalamus)
96
Which portion of the brain forms the fourth ventricle?
Rhombencephalon-->Metencephalon (Pons, Cerebellum), Myelencephalon
97
Which portion of the brain forms the cerebral aqueduct?
Mesencephalon (midbrain)
98
What does increased AChE on an amniotic fluid tap suggest?
Neural tube defect
99
What is anencephaly associated with?
maternal type 1 diabetes
100
Signaling pathway for holoprosencephaly? Associations?
sonic-hedgehog, patau and FAS
101
What spinal tracts are associated with syringomyelia? Normal Level? Association?
Spinothalamic (anterior white commisure), C8-T1, chiari I malformation (headaches, Drake Hardy's sis)
102
What tongue muscle is innervated by CN X? What does it do?
Palatoglossus (elevates posterior tongue during swallowing)
103
What neural cells do HIV infect?
Microglia
104
What neural cells are responsible for reactive gliosis to neural injury?
Astrocytes
105
Where is the inflammatory infiltrate in GBS? CSF finding?
Endoneurium (single nerve fiber), isolated increase in protein
106
What nerve covering must be rejoined in microsurgery? Function?
Perineurium, Permeability barrier
107
Where is GABA produced? ACh? NE? Serotonin?
GABA- Nucleus accumbens
ACh- Basal nucleus of Meynert
NE- Locus ceruleus
Serotonin- Raphe nucleus
108
What type of edema do you get with destruction of the BBB?
vasogenic
109
What molecule makes you feel full? Where?
Leptin, ventromedial hypothalamus
110
What causes extra ocular movements during REM sleep?What NT is increased during this stage? Memory or No?
Paramedian pontine reticular formation (PPRF, conjugate gaze center). ACh, Yes
111
Preferred tx for sleep enuresis?
Desmopressin (oral) is preferred over imipramine
112
What is the final visual destination past the lateral geniculate nucleus?
Calcarine sulcus
113
What is the dopaminergic pathway that results in positive symptoms? Extrapyramidal symptoms?
Mesolimbic, Nigrostriatal
114
Which cerebellar peduncle is the output? Which receives input from ipsilateral spinal cord? Contralateral cortex?
Superior, Inferior, Middle
115
If you injure the left cerebellar laterally, which direction do you fall?
Left
116
What does dopamine input do to the direct and indirect basal ganglia pathways?
Stimulates direct (D1), inhibits indirect (D2), both facilitate movement
117
What type of tremor is worsened with movement or anxiety? Treatment?
Essential tremor. Often patients self medicate with alcohol but we prefer propranolol or primidone
118
Hemiballisimus is associated with what nucleus?
Contralateral subthalamic
119
What are Lewy bodies composed of? What are they associated with? Intracellular or extracellular?
alpha-synuclein, Parkinson, intracellular
120
What causes neuronal death in huntington's?
NMDA-R binding and glutamate excitotoxicity
121
Where is the wernicke area of the brain?
Superior temporal gyrus
122
What is always intact with transcortical aphasia of any type? (Name tells you the rest)
Repetition (watershed areas without actual areas)
123
What disease is associated with Kluver-Bucy Syndrome?
HSV-1 encephalitis
124
Which parietal cortex injury results in hemineglect?
Non-dominant (usually right)
125
Agraphia, acalculia, finger agnosia, L-R disorientation
Gerstmann syndrome, dominant parietal cortex
126
What brain lesion causes the eyes to look away from side of lesion? Toward?
Away- Paramedian pontine reticular formation (PPRF)
| Toward- Frontal eye field (look For lesion)
127
Cerebral perfusion relies on what gradient? What usually modulates it?
MAP-ICP gradient, generally driven by Pco2 unless severe hypoxia (Po2
128
Contralateral paralysis and/or sensory loss with the absence of cortical signs suggests a stroke where?
Lenticulostriate artery (internal capsule), usually due to hypertension (lacunar infarct)
129
What would you suspect with a chronic hypertension patient with thalamus or basal ganglia stroke? (internal structures)
Charcot-Bouchard microaneurysm
130
Aneurysm where can compress optic chiasm? CN III?
ACA, PCA
131
Where is the lesion in a stroke patient who has allodynia weeks to months after stroke? What is this called?
Thalamic lesion, Central Post-Stroke Pain Syndrome
132
Skull fracture can cause what type of hematoma? What do patients look like?
Epidural (talk then die)
133
What is xanthochromia and what should you be worried about?
Bloody or yellow spinal tap, subarachnoid hemorrhage
134
What do you suspect in a pt. who had a subarachnoid hemorrhage and 4-10 days later has symptoms of an ischemic infarct (e.g. neural sx). Treatment/prevention?
Vasospasm, nimodipine
135
When do you see red neurons?
12-48 hours post ischemia
136
Most likely cause of brain embolism?
Atrial fibrillation (potentially could be a DVT with a patent foramen ovale)
137
Where do dural venous sinuses empty?
Internal jugular vein
138
What sinus does the ophthalmic vein drain into?
Cavernous sinus
139
What is the foramen of monro?
Connects lateral ventricles to 3rd ventricle
140
Risk factors for idiopathic increase in ICP (pseudo tumor cerebri)
Vitamin A excess, danazol, tetracycline
| CN VI palsy without change in mental status
141
Where does spinal cord end? Where does subarachnoid space end?
L1-L2 border, S2 (lumbar puncture is at L3-L5)
142
Which type of motor neuron lesion gives you fasciculations?
LMN (twitching due to under stimulation)
143
What is the difference in poliomyelitis and werdnig hoffman disease?
Polio is asymmetric, WH is symmetric (both are floppy baby)
144
What enzyme can cause ALS?
Defect in superoxide dismutase 1 (SOD1)
145
Where does poliovirus replicate before entering CNS?
oropharynx and SI (fecal-oral transmission)
146
What is the chromosome for Friedreich ataxia? What is the gene? What does the gene do, what dysfunctions as a result?
c9, frataxin (iron-binding protein that leads to mitochondrial dysfunction)
147
How does Friedreich ataxia present? Cause of death?
kyphoscoliosis in childhood. Also has diabetes and hypertrophic cardiomyopathy which kills them
148
What are the functions of the superior and inferior colliculi?
Superior- conjugate vertical gaze center
Inferior- hearing
"eyes above ears"
149
Paralysis of conjugate vertical gaze causes and name?
Parinaud syndrome: pinealoma, stroke, hydrocephalus
150
Where does the middle meningeal artery exit? CN VII? CN X?
MMA- foramen spinosum
CN VII- internal auditory meatus
CN X- jugular foramen (with CN IX and XI)
151
What cranial nerve does not have a thalamic relay to cortex?
CN I
152
What muscle does CN IX innervate?
stylopharyngeus (elevation of pharynx and larynx)
153
What does "everything wet in the head?" Exception?
Facial nerve with the exception of the parotid gland which is innervated by CN IX
154
What cranial nerve nucleus does taste?
Nucleus solitarius
155
Where does the vagus nerve have motor activity? PSNS?
Motor- nucleus aMbiguus- pharynx, larynx, esophagus
| PSNS- Dorsal Motor Nucleus- Heart, lungs, upper GI
156
What reflex is all one nerve? Branches?
Jaw jerk V3 both ways (muscle spindle)
157
What muscle opens the mouth?
Lateral pterygoid
158
Does UMN or LMN lesion of facial nerve spare the forehead?
UMN. Nucleus for forehead is innervated by UMN from both sides
159
Treatment for Bell palsy?
Corticosteroids, acyclovir
160
Nerve most susceptible to injury in cavernous sinus?
CN VI
161
What is a cholesteatoma? Due to fat and cholesterol?
Overgrowth of desquamated keratin debris within middle ear space that can erode ossicles and cause conductive hearing loss. Looks like a nasty thing behind eardrum. NOOOOO!
162
What are the layers on the back of the eye from inner to outer?
Retina, choroid, sclera
163
What muscarinic fibers are in the eye? What do they do?
M3. Increase outflow via canal of schlemm and constrict the pupil (iris)
164
What types of drugs decrease the synthesis of aqueous humor? Where is it produced?
Ciliary body, beta blockers, alpha-2 agonists, carbonic anhydrase inhibitors
165
Hyperopia vs Myopia. Describe relative to where the light focuses.
The light focuses in behind the retina of the hyperopic eye and in front of the myopic eye.
166
What is presbyopia, what causes it?
Age-related impaired accommodation due to decreased lens elasticity
167
Progressive peripheral field visual loss? Painful? Painless?
Glaucoma. Open-angle is painless, closed/narrow angle is painful (true emergency if acute)
168
What race is more predisposed to open-angle glaucoma?
African americans
169
What drug can you not give with acute closure glaucoma?
Anything that does mydriasis (alpha 1)
170
What causes loss of central vision?
Age-related macular degeneration
171
What is ranibizumab? What does it treat?
Anti-VEGF antibody that can treat eye conditions associated with neovascularization causing loss of vision (wet macular degeneration, proliferative diabetic retinopathy)
172
Flashes and floaters leading to curtain drawn down on vision? What causes it?
Retinal detachment. Separation of neurosensory layer of retina (photoreceptors) from outermost pigmented epithelium
173
Painless progressive vision loss beginning with night blindness?
Retinitis pigmentosa (inherited retinal degeneration)
174
What nerve causes pupil constriction?
Edinger-Westphal nucleus-->ciliary ganglion (CNIII)--> short ciliary nerves
175
What is the relation of CN II to the edinger westphal nucleus?
CN II--> pretectal nuclei--> bilateral EW nuclei (cause of consensual reflex)
176
What nerve mediates mydriasis?
Long ciliary nerve (sympathetics via ciliospinal center of budge to cervical ganglion)
177
Light in one eye to the other results in down bilateral constriction. What is the name and what is the cause?
Marcus-Gunn pupil, damage to optic nerve or severe retinal injury
178
What is the Meyer loop? Parietal or Temporal?
Temporal optic radiation
179
Which two nerves does the MLF connect? What nerve fires first? What is a defect called? Which eye gets nystagmus?
CN VI to CN III (CN VI first). A defect is called internuclear ophthalmoplegia (often in MS) in which the abducting eye gets nystagmus
180
Describe senile plaques and neurofibrillary tangles in alzheimers. Intracellular/extra, composition?
Plaques: extracellular beta amyloid
Tangles: intracellular hyperphosphorylated tau protein
181
What correlates with the degree of dementia in alzheimers? What is this functionally?
Number of neurofibrillary tangles, cytoskeletal elements
182
What is the composition of the round inclusions in frontotemporal dementia? What are they called?
hyperphosphorylated tau, Pick bodies
183
Dementia and hallucinations followed by parkinsonian features? What inclusions do you see (composition, location)
Lewy body dementia
| Intracellular Lewy Bodies- alpha synuclein primarily in cortex
184
Startle myoclonus is a symptom of what dementia disease?
CJD
185
What is the triad with MS? What is the cause? Important findings for diagnosis (2)?
Charcot triad: scanning speech, intention tremor and nystagmus. Autoimmune etiology. Periventricular plaques and oligoclonal bands.
186
What might you give for spasticity in MS? Acute MS flares?
Baclofen
| Steroids
187
What is acute inflammatory demyelinating polyradiculopathy? What is the CSF finding? What drug do you not use?
```
Common subtype of GBS
Albuminocytologic dissociation (isolated inc. in protein)
No role for steroids
```
188
Foot deformities, lower extremity weakness and sensory deficits? Cause?
Charcot-Marie-Tooth: autosomal dominant disorder related to defective production of proteins involved in structure and function of peripheral nerves or myelin sheath.
189
What builds up in Krabbe? Metachromatic leukodystrophy? Difference?
Krabbe: galactocerbroside, peripheral neuropathy, optic atrophy
ML: sulfatides, central and peripheral demyelination
190
What two antibodies increase risk of reactivating latent JC virus infection? What is this called?
PML- rituximab, natalizumab
191
Difference in simple and complex partial seizures?
Both affect single brain area, simple has consciousness intact while complex does not
192
What is the classic EEG association with absence seizures?
3Hz wave.
193
What is the difference when positional testing produces delayed nystagmus vs. immediate? Which may change directions?
Immediate: brainstem or cerebellar lesion (may change)
Delayed: inner ear etiology (more common)
194
Is sturge-weber syndrome inherited? What gene?
No, it is a somatic activating mutation of GNAQ gene
195
From what cell line are neurofibromas derived?
Neural crest cells (schwann cells)
196
What 3 tumors are associated with VHL?
Hemangioblastomas, renal carcinoma, pheochromocytoma
197
What can hemangioblastomas produce? Where are they located?
Often cerebellar and can produce Epo
198
What brain tumor is S100+? What does this mean?
Schwannoma, this is a neural crest marker (e.g. melanocytes in melanoma)
199
What cancer is associated with eosinophilic, corkscrew shaped inclusions? What are these called?
Pilocytic (low grade) astrocytoma is associated with rosenthal fibers
200
Perivascular rosettes in brain tumor
Ependymoma
201
What brain tumor can produce precocious puberty in males? How?
Pinealomas produce beta-hCG and thus can produce precocious puberty in males
202
What drug do you use for glaucoma emergencies?
Pilocarpine
203
How do opiods work?
Open K+ channels, close Ca2+ channels, and thus decrease synaptic transmission
204
How does tramadol work? What are some adverse effects?
Very weak opiod agonist, inhibits 5-HT and NE reuptake
| AEs: Serotonin syndrome, decreases seizure threshold
205
What drug must be titrated slowly to watch for SJS?
Lamotrigine
206
Major concern with valproate?
Hepatotoxic
207
What seizure drug might cause mental dulling and kidney stones?
Topiramate
208
What is a contraindication for barbiturates? Do they induce or inhibit p450?
Porphyria, induce
209
What do lipid and blood solubility have to do with anesthetic effects?
high lipid solubility: high potency
| High blood solubility: slow onset
210
What drugs cause malignant hyperthermia?
Inhaled anesthetics or succinycholine
211
What is the order of loss with nerve block based on size/myelination and the senses
1) small myelinated>small un>large mye> large un
| Pain, temp, touch, pressure
212
Which local anesthetic causes: severe CV toxicity? methemoglobinemia?
Bupivacaine
| benzocaine
213
What drugs are dopamine agonists?
Bromocriptine (ergot), pramipexole, ropinirole
214
What do carbidopa, tolcapone, and selegiline block?
carbidopa: peripheral DOPA decarboxylase
- capone: COMT (tolcapone central and peripheral)
selegiline: MAO-B
215
CNS uses of amantadine vs. memantine
Amantadine: increase dopamine release and decrease uptake (parkinson)
Memantine: NMDA antagonist that helps prevent excitotoxicity (alzheimers)
216
What do tetrabenazine and reserpine do and what are they used for?
Inhibit VMAT-->decreased dopamine packaging into vesicles (Huntington's)
217
What is the most common cause of hydrocephalus in newborns?
Cerebral aqueduct stenosis
218
In a lumbar puncture, what layer are you trying to reach?
Subarachnoid space. DO NOT PIERCE PIA
219
What do you suspect with multiple infarcts in watershed areas of the brain all at once?
Moderate global ischemia
220
What effect can an insulinoma have on the brain?
It can cause global cerebral ischemia via repeated episodes of hypoglycemia
221
How long must symptoms persist to be classified as a stroke rather than a TIA?
24 hours
222
What is the cause of a hemorrhagic infarct in the brain?
Embolus (when the embolus lyses, the infarction becomes hemorrhagic)
223
What causes lacunar strokes?
Hyaline arteriosclerosis (diabetes or chronic HTN)
224
What layer do berry aneurysms lack?
Media
225
Subfalcine herniation compresses what? Uncal herniation?
subfalcine- ACA
| Uncal- PCA, CN III
226
Where does metachromatic leukodystrophy product accumulate? Result? What about Krabbe?
ML- sulfatides in lysosomes of oligodendrocytes
| Krabbe- Macrophages
227
What is the cause of adrenoleukodystrophy? Inheritance?
Impaired addition of CoA to very long chain fatty acids, obviously hurts adrenals and brain (x-linked)
228
What is destroyed by the autoimmune attack in MS?
myelin and oligodendrocytes
229
What are two random associations with MS?
HLA-DR2, further distance from equator
230
What is the difference in delirium from dementia?
Delirium can go away and involves CHANGING LEVELS OF CONSCIOUSNESS
231
Which apolipoprotein is associated with alzheimers? Specifically which increases and decreases risk?
ApoE4 increases risk, ApoE2 decreases risk
232
What is Tau?
A microtubule associated protein
233
MPTP exposure was related to development of what?
Parkinson disease
234
Why is anticipation seen with Huntington disease?
Further expansion of repeats in spermatogenesis
235
What is stretched in NPH that causes sx? What improves sx?
Corona radiata, lumbar puncture
236
Spike wave complexes on EEG?
CJD
237
Necrotic areas with pseudopalisading around it brain tumor?
GBM
238
What cells are forming a meningioma?
Arachnoid cells
239
Calcified tumor in white matter? Maybe with seizures?
Oligodendroglioma
240
What does drop metastasis refer to? What tumor?
Metastasis of medulloblastoma to the cauda equina (spreads via CSF)
241
What embryonic structure forms the coronary sinus? The SVC?
CS- left horn of sinus venosus
| SVC- right common cardinal vein and right anterior cardinal vein
242
Which portion of the intraventricular septum is usually responsible for a VSD? What embryonic structure is this derived from?
Membranous portion, endocardial cushion
243
What type of cells are responsible for outflow tract anomalies such as TOF and TOGV?
Neural crest cells are needed form truncal and bulbar ridges to spiral. No neural crest migration--> anomalies
244
What is the ductus venosus? How many umbilical veins and arteries are there in the cord?
Ductus venosus is what allows the IVC blood to bypass the hepatic circulation. 1 vein (from mom) and two arteries branching off of the internal iliac arteries
245
Where does most of the highly oxygenated blood that reaches the fetal heart come from and go? What about the deoxygenated blood?
IVC- oxygenated blood that mostly goes through foramen ovale
| SVC- deoxygenated blood that goes through heart and is shunted through the PDA
246
What forms the median and medial umbilical ligaments?
Median- allantois
| Medial- umbilical arteries
247
Arrange the pericardium layers from outer to inner. Where is the pericardial cavity.
Fibrous pericardium--> parietal serous pericardium--> visceral serous (pericardial cavity is between parietal and visceral layers)
248
Pulse pressure is inversely proportional to what?
Arterial compliance
249
In early exercise, CO is maintained by what? What about late exercise?
Early: increase in SV and HR
Later: SV plateaus, only inc. HR
250
Which phase of the cardiac cycle is preferentially shortened with an increased heart rate?
Diastole
251
Catecholamines cause an increase in contractility of the heart by inhibiting what?
Phospholamban
252
What do ventricular diameter and wall thickness due to myocardial oxygen demand?
Increased diameter requires more oxygen (increased wall tension), increased wall thickness decreases the demand (this is why it thickens)
253
What accounts for most of TPR?
Arterioles
254
Which phase of the cardiac cycle is the period of highest oxygen consumption?
Isovolumetric contraction
255
What are S3 and S4 associated with? Which can be normal?
S3- high filling pressure (normal in young adults and children)
S4- stiff ventricle wall (left lateral decubitus)
256
What wave is absent in the jugular venous pulse curve with cardiac tamponade?
Y descent
257
Normal splitting is affected how by inspiration? What does paradoxical splitting indicate? Fixed?
Normal is increased by inspiration (inc. venous return, P2 after A2)
Paradoxical: aortic stenosis LBBB
Fixed: ASD
258
What increases the intensity of right heart sounds?
Inspiration (up venous return)
259
Where is aortic regurgitation heard?
Left sternal border
260
Late systolic crescendo murmur with mid systolic click?
Mitral valve prolapse
261
What tells you the severity of mitral stenosis?
Decreased interval between S2 and OS correlates with increased severity
262
What phase in the cardiac action potential is the upstroke?
Phase 0
263
Does cardiac muscle require extracellular calcium to contract?
yes, skeletal muscle does not
264
What phases are in the pacemaker action potential? What stimulates the upstroke?
Phase 0, 3 and 4 (four is a slow depolarization due to funny currents If), calcium channels cause depolarization
265
Why are there no Na+ channels in the pacemakers?
There are, they are permanently inactivated because of the less negative resting potential of these cells (-70 vs -85)
266
What is the treatment for torsades?
MgSO4
267
What are the two congenital long QT syndromes? Which is associated with deafness? Inheritance?
Romano-Ward: AD, no deafness
Jervell and Lange-Nielsen Syndrome, AR, sensorineural deafness
Both usually due to ion channel defects
268
What syndrome is associated with ECG pattern of pseudo-right bundle branch block and ST elevations in V1-V3? Cause of death? Inheritance/typical pt?
Brugada syndrome, AD in Asian males
| SCD can be prevented by ICD
269
What do you associate with delta waves?
WPW syndrome
270
What is a first degree AV block?
PR interval is prolonged >200msec
271
Mobitz 1 vs Mobitz II vs 3rd degree?
Mobitz 1: progressive lengthening then drop
Mobitz 2: random drop
3rd degree: atria and ventricles independent of each other
272
What can cause 3rd degree heart block?
Lyme disease
273
What is the mechanism of ANP and what are the effects?
Works via cGMP and causes vasodilation and down Na+ reabsorption at the renal collecting tubule. Also dilates afferent and constricts efferent arteriole
274
What substance in blood can be used to diagnose HF?
BNP
275
What nucleus in the brainstem does baroreceptors?
Solitary
276
What happens in baroreceptors when the pressure drops?
Down stretch leads to down firing which disinhibits sympathetics and leads to increase in pressure.
277
What is the cushing reaction and how does it work?
Triad of hypertension, bradycardia and respiratory depression. It happens with increased ICP that causes cerebral arterial constriction and ischemia. Increased pCO2 causes a central reflex to increase perfusion pressure which via baroreceptors causes bradycardia.
278
Do central chemoreceptors respond to O2?
No, not directly
279
What determines auto regulation of skeletal muscle blood flow at rest vs during exercise?
At rest it is sympathetic tone while it is local metabolites during exercise (adenosine, lactate, K+, etc.)
280
What type of congenital heart shunt causes early cyanosis? Mnemonic?
Right to left (all have Ts)
281
What is caused by anterosuperior displacement of the infundibular septum? What is the most important to determine prognosis of it?
Tetralogy of Fallot, pulmonary stenosis
282
What is usually responsible for an ASD? Where is the defect?
Generally ostium secundum, but ostium primum defects occur with other cardiac anomalies (down syndrome)
283
What cardiac defect is associated with a diabetic mother?
Transposition of the great vessels
284
What is the heart defect associated with William's syndrome?
supravalvular aortic stenosis
285
What cardiac defects are associated with DiGeorge?
TOF, Truncus arteriosus
286
What causes hyperplastic arteriolosclerosis? What cells proliferate?
Severe hypertension, proliferation of SM cells
287
After fatty streaks form, what cells migrate in to form an atheroma?
Smooth muscle cells (involves PDGF and FGF, often supplied by platelets)
288
What cells deposit the ECM in atherosclerotic plaques?
Smooth muscle cells
289
What layer of the blood vessel degenerates to form an aneurysm?
Media
290
What causes an aortic dissection? Biggest risk factor?
Longitudinal intimal tear. Hypertension
291
What are the two types of aortic dissection and their treatment?
Stanford A involves Ascending aorta: surgery
| Type B: No ascending aorta involvement, tx is beta blockers then vasodilators
292
What is the principle behind pharmacologic stress tests?
Coronary steal syndrome: vessels distal to a block are already maximally dilated due to ischemia, so if you give a vasodilator (e.g. dipyridamole) other vessels that are well perfused dilate and steal blood flow.
293
In a non-STEMI, what area of the heart has infarcted?
Subendocardium, this is an area particularly vulnerable to ischemia
294
When do you see post infarction fibrinous pericarditis?
1-3 days after, neutrophil stage
295
When do troponin and CK-MB rise and fall to normal?
Troponin: rise in 4 hours, fall in 7-10 days
| CK-MB: rise in 6 hours, fall in 48 hours
296
What ECG changes clue you in to LCX, RCA, PDA strokes?
LCX: Lateral (leads I, aVL)
RCA: inferior (leads II, III, aVF)
PDA: ST depression in V1-V3 with tall R waves
297
Which papillary muscle is most likely to rupture? What is the result?
Posteromedial papillary muscle resulting in severe mitral regurgitation
298
How does outflow obstruction happen in HOCM?
Asymmetric septal hypertrophy and systolic anterior motion of the mitral valve cause obstruction of outflow.
299
What is endomyocardial fibrosis with a predominant eosinophilic infiltrate?
Loffler syndrome
300
When do you not use beta blockers with heart failure?
acute decompensation
301
What happens to preload and after load in cardiogenic shock? Obstructive?
Increased preload (blood can't get through), and after load (blood pressure is dropping so resistance tries to counter it)
302
Type of shock with a decrease in both preload and afterload?
Distributive
303
Round white spots on retina surrounded by hemorrhage?
Roth spots, endocarditis (bacterial)
304
What do you suspect if you see a granuloma with giant cells in the heart?
Rheumatic fever (this is an aschoff body)
305
What causes equilibration of diastolic pressure in all four heart chambers?
Cardiac tamponade
306
When do you see Kussmaul sign? What is it?
increase in JVP with inspiration (usually should be down). See it in constrictive pericarditis or restrictive cardiomyopathies
307
How does polyarteritis nodosa happen? What should you probably associate?
Immune complexes lead to transmural inflammation of the arterial wall with fibrinoid necrosis. Hepatitis B
308
What do you suspect if you see innumerable renal micro aneurysms?
PAN
309
What kills kids with Kawasaki Disease?
Coronary artery aneurysms
310
What is the other name for Wegener vasculitis?
Granulomatosis with polyangiitis
311
What is the triad of Henoch-Schonlein Purpura? What causes it?
Skin, Arthralgias, and GI problems secondary to IgA immune complex deposition
312
What drugs are used for HTN in pregnancy?
Hydralazine, labetalol, methyldopa, nifedipine
313
What blood pressure medicine can causes hyperprolactinemia?
Non-dihydropyridine CCBs
314
What toxicity might you see with nitroprusside?
Cyanide toxicity
315
What is fenoldopam?
D1 agonist that causes coronary, peripheral, renal and splanchnic vasodilation
316
Which anti-lipid drug can result in gout?
Niacin
317
To what patients do you not want to give cardiac glycosides?
Patients with renal failure
318
Describe class I antiarrhytmics in relation to length of action potential
Ia: prolonged (increased QT)
Ib: decreased
Ic: No change
319
Which class of antiarrhytmics is best post-MI? Which is contraindicated?
Class Ib is best, Class Ic is contraindicated
320
What phase of the cardiac action potential do B-blockers affect?
Phase four of the nodal action potential (sympathetics use this phase to increase heart rate)
321
What labs need to be checked with amiodarone?
PFTs, LFTs, TFTs
322
What drug can cause blue/gray skin deposits and corneal deposits?
Amiodarone
323
How does adenosine work as an antiarrhytmic?
Allows K+ out of the cell causing hyperpolarization, effect is blunted by caffeine and theophylline
324
What anti arrhythmic can cause flushing, hypotension and chest pain?
Adenosine
325
What arteries are involved with takayasu arteritis?
Aortic arch at branch points
326
What type of vasculitis can lead to a string of pearls appearance on imaging?
PAN
327
Treatment for PAN, Wegener, and Microscopic polyangiitis is all the same, what is it?
Cyclophosphamide and steroids
328
What kind of problem do you suspect with hematuria rolling an URI?
IgA nephropathy (Henoch-Schonlein Purpura perhaps)
329
What leads to HTN with increased rain and unilateral kidney atrophy? What might be a cause of this in young females?
Renal artery stenosis (or a clip). Fibromuscular dysplasia
330
Where is the plaque in atherosclerosis? What layer?
Intima
331
What is the first step in the pathogenesis of atherosclerosis?
Endothelial damage
332
What percent stenosis is required for symptoms to arise?
70%
333
What causes hyaline arteriolosclerosis? What is the pathogenesis?
Long-standing hypertension or DM. Proteins leaking into the vessel wall causing thickening
334
What is the difference in the renal failure associated with hyaline and hyper plastic arteriolosclerosis?
Hyaline produces glomerular scarring leading to slow onset chronic renal failure while hyper plastic causes fibrinoid necrosis of the vessel wall leading to acute renal failure with "flea bitten" appearance
335
What is the pathogenesis of aortic dissection with its most common risk factor?
Hypertension--> hyaline arteriolosclerosis--> intimal thickening--> decreased blood flow of vasa vasorum--> medial atrophy--> intimal tear with dissection through the media
336
Where is an AAA classically found?
Below renal arteries but above aortic bifurcation (L2-L4)
337
What is the major cause of AAA?
Atherosclerosis decreasing diffusion of O2 to the media leading to atrophy and weakness of the vessel wall
338
What cancer is associated with PVC, arsenic and thrortrast?
Liver angiosarcoma
339
What can you give in prinzmetal angina besides nitroglycerin?
CCBs
340
What causes contraction band necrosis related to a MI?
Reperfusion of irreversibly damaged cells results in calcium influx and hypercontraction of the myofibrils
341
What is the cause of paroxysmal nocturnal dyspnea?
Increased venous return when lying flat
342
What congenital cardiac defect is most associated with FAS?
VSD
343
Lower extremity cyanosis without pulse differential?
PDA
344
In infantile coarctation of the aorta, where is the coarctation in relation to the PDA?
Coarctation is proximal
345
What is associated with the adult form of coarctation of the aorta?
Bicuspid aortic valve
346
What is the valvular consequence of acute rheumatic fever vs. chronic rheumatic heart disease?
Acute is regurgitation plus JONES, chronic is stenosis
347
What appearance of the aortic valve is a heavy hitting clue for chronic rheumatic heart disease?
Fusion of the commissures
348
Concomitant aortic stenosis with fusion of the valve comissures and mitral stenosis?
rheumatic valve disease
349
What causes mitral valve prolapse (as in what has happened to the valve)?
Myxoid degeneration (accumulation of ground substance)
350
What bacteria causes most endocarditis in damaged valves? Healthy valves?
Damaged- S. viridans
| Healthy- S. aureus
351
What is the pathogenesis of subacute endocarditis?
Damaged endocardial surface develops thrombotic vegetations (platelets and fibrin) and bacteremia leads to trapping of bacteria in these vegetations
352
What is the difference in acute and subacute endocarditis?
Acute is large vegetations on a previously healthy valve that destroy it, subacute is small vegetations on a previously damaged valve that do not destroy it
353
What organisms do you suspect in endocarditis with negative blood cultures?
HACEK (haemophilus, actinobacillus, cardiobacterium, eikenella, kingella)
354
What causes janeway lesions, osier nodes, splinter hemorrhages and roth spots in endocarditis?
Emolization of septic vegetations
355
What type of heart problem might you see with pregnancy?
Dilated cardiomyopathy (late pregnancy or soon after birth)
356
Low voltage EKG with diminished QRS amplitude suggests what?
Restrictive cardiomyopathy
357
Anterior midline neck mass that moves with swallowing or protrusion of the tongue?
Thyroglossal duct cyst
358
What germ cell layer is thyroid tissue derived from? Parafollicular cells (C cells) of the thyroid?
Endoderm, neural crest
359
What hormones come from the anterior pituitary? Which are acidophils and basophils?
Acidophils: GH, Prolactin
Basophils: ACTH, TSH, FSH, LH
360
What four hormones share a subunit? Which subunit is shared and which one determines specificity?
TSH, LH, FSH and B-hCG all share the alpha subunit but the beta subunits confer specificity
361
What germ cell layers are anterior and posterior pituitary derived?
Anterior: surface ectoderm (Rathke's)
Posterior: neuroectoderm (hypothalamus)
362
Where would you find B-cells of the pancreas? (both the general and specific)
In the central region of the islets of langerhans (INsulin is INside)
363
Where is preproinsulin synthesized? What form is in secretory granules?
RER, in secretory granules, proinsulin is cleaved to insulin and C-peptide (both excreted for this reason)
364
What type of receptor is the insulin receptor?
Tyrosine kinase
365
Does insulin cross the placenta? Glucose?
Insulin no, glucose yes
366
Where do you find insulin dependent glucose transporters?
Striated muscle and adipose tissue
367
What type of transporters for sugar are on spermatocytes?
Glut-5 (fructose)
368
How is insulin released?
Glucose--> increased ATP--> closes K+ channels--> depolarization--> ca2+ open and influx--> exocytosis
369
Which pathways of insulin receptor activation stimulate cell growth/DNA synthesis vs. biomolecule synthesis and receptor transport to membrane?
Cell growth/DNA synth: RAS/MAP kinase path
| Rest: PI3-K pathway
370
How does insulin influence glucagon secretion?
Increased insulin decreases glucagon
371
What all does CRH increase?
ACTH, MSH, B-endorphin (POMC)
372
What suppresses GnRH?
Prolactin (basis of lactating "birth control" and amenorrhea in prolactinoma)
373
What hormone causes an increase in prolactin release? Decrease?
Increased by TRH, decreased by dopamine
374
How could renal failure play a role in prolactin secretion?
Decreased prolactin elimination would give higher levels of prolactin
375
What does estrogen do to prolactin secretion?
Increases it (OCPs, Pregnancy)
376
Through what intermediate does growth hormone work? (both names) Where does this come from
IGF-1 or somatomedin C comes from the liver
377
What causes orexigenesis?
Ghrelin (stimulates hunger)
378
Where is leptin produced?
Adipose tissue
379
What does the V1 receptor do?
Modulate blood pressure (vasoconstriction and increased pressure)
380
What main enzyme does ketoconazole block in relation to the adrenals?
Cholesterol desmolase
381
Name two hormones that cause an increase in insulin resistance
Cortisol and GH (consider that they both might need an increase in glucose to do their jobs)
382
What causes striae?
Cortisol downregulates fibroblast activity
383
How can exogenous corticosteroids cause reactivation of TB and candidiasis?
Blocks IL-2 production
384
How would you describe the relationship of cortisol to many other hormones (e.g. ADH)?
Permissive
385
Describe the relationship between serum pH and calcium concentrations
In an increased pH, more calcium is bound (less cations are around to bind negative proteins since no H+)--> hypocalcemia effectively
386
Which hormone causes an increase in GI absorption of Ca2+ and PO4?
Vitamin D
387
Where is PTH produced?
Chief cells of parathyroid
388
Where in the nephron does PTH promote calcium reabsorption?
Distal tubule (like thiazides)
389
What do you se an increase of in the urine with PTH besides PO4?
cAMP (PTH hits G proteins)
390
How does PTH affect alkaline phosphatase?
It increases it because it increases osteoblast function in order to activate osteoclasts via RANK (this is why intermittent PTH can stimulate bone formation)
391
What is the relation of magnesium to PTH secretion?
Slight down in Mg leads to an increase in PTH where a severe drop in Mg causes down secretion
392
Where is most T3 formed?
Target tissues
393
What is the relation of thyroid hormones and beta-adrenergic effects?
Thyroid hormone up regulates B1 receptors.
394
In what cases does TBG increase? Decrease?
Increase: Pregnancy and OCP use (estrogen)
Decrease: Corticosteroid use, liver failure
395
What enzyme is responsible for oxidation, organification and coupling of thyroid hormone? What drugs block this? Which one also stops peripheral conversion?
Thyroid peroxidase
| Methimazole and propylthiouracil block this but PTU also blocks peripheral 5'-deiodinase
396
What hormones work via intracellular receptors (7)?
Progesterone, Estrogen, Testosterone, Cortisol, Aldosterone, T3/T4 and vitamin D
397
What receptors do acidophils target (pituitary)?
Nonreceptor tyrosine kinases
398
What is Cushing disease?
ACTH secreting pituitary adenoma
399
What is metyrapone and what can it be used for?
Blocks last step of cortisol synthesis and should cause a decrease in cortisol and increase in ACTH. Helps determine if adrenal insufficiency is primary or secondary/tertiary.
400
Do you see skin and mucosal hyper pigmentation in primary or secondary adrenal insufficiency?
Primary because ACTH would be increased and thus MSH would too (POMC derivatives)
401
What is addison disease? Most common causes?
Chronic primary adrenal insufficiency, most often caused by autoimmune destruction in western world and TB in developing world.
402
What is Conn syndrome?
Primary hyperaldosteronism due to an adrenal adenoma
403
Which tumor can cross the midline in the childhood abdomen? What cell type is it derived from? Odd paraneoplastic syndrome?
Neuroblastoma, neural crest, opsoclonus-myoclonus syndrome
404
What tumors are associated with Homer-Wright rosettes?
Neuroblastoma (adrenal) and medulloblastoma (cerebellum)
405
What is the relationship of cholesterol levels with hyper and hypothyroidism? Why?
Hypothyroid- increased cholesterol
Hyperthyroid- decreased
Related to LDL receptor expression
406
Can you see increased CK with hypo or hyperthyroid myopathy?
Hypothyroid
407
HLA associated with hashimoto? What cancer does it predispose for?
HLA-DR5, non-Hodgkin lymphoma
408
Hurthle cells, lymphoid aggregates with germinal centers in enlarged thyroid?
Hashimoto
409
What thyroid problem follows a viral infection often? Presentation? Histology?
```
de Quervain (subacute granulomatous thyroiditis)
PAINFUL thyroid, granulomatous inflammation
```
410
Fixed, rock hard thyroid with inflammatory infiltrate? What antibody is it related to?
Riedel thyroiditis, IgG4-related systemic disease
411
Tall, crowded follicular epithelial cells with scalloped colloid.
Graves disease
412
What are two findings that are characteristic of Graves disease but not of other hyperthyroid causes? How do they occur?
Pretibial myxedema due to the antibodies stimulating TSH receptors on dermal fibroblasts and exophthalmos due to increased fibroblast secretion of hydrophilic GAGs which leads to swelling.
413
Follicular histology without capsular invasion in thyroid? With invasion?
Thyroid adenoma vs. follicular carcinoma
414
How does papillary thyroid carcinoma spread? Follicular?
Papillary is a normal carcinoma that spreads via lymphatics, follicular spreads via blood
415
What thyroid cancer is associated with childhood radiation? Name two characteristic histologic findings.
Papillary thyroid carcinoma
| Orphan annie eyes and psammoma bodies
416
What thyroid cancer is associated with the MEN syndromes? Mutation? What cells in the thyroid?
Medullary carcinoma, RET mutation, C-Cells (parafollicular)
417
What do you suspect in a person with short 4th/5th digits, short stature and hypocalcemic signs? Heritability? Cause? Which parent must it be inherited from?
AD, (Albright hereditary osteodystrophy)pseudohypoparathyroidism (low calcium despite adequate levels of PTH). Due to defective Gs protein alpha subunit causing end organ resistance to PTH. Must be inherited from mother to have resistance. From father results in pseudo pseudo...
418
Mild hypercalcemia and hypocalciuria with normal to elevated PTH levels?
Familial hypocalciuric hypercalemia (defective calcium sensing receptor needs higher than normal Ca levels to suppress PTH)
419
Cystic subperiosteal bone spaces filled with brown fibrous tissue? Cause?
Osteitis fibrosa cystic which is caused by primary hyperparathyyroidism
420
What is renal osteodystrophy?
Renal disease causes secondary hyperparathyroidism which leads to bone lesions
421
What is Nelson syndrome?
Enlargement of existing ACTH-secreting pituitary adenoma after bilateral adrenalectomy for the refractory cushing disease
422
What normally happens to GH following oral glucose tolerance test? What should you suspect if not?
Usually it decreases but it doesn't in acromegaly (pituitary mass often)
423
Pegvisomant
GH receptor antagonist
424
What type of DI is caused by lithium?
Nephrogenic
425
What is the volume status with SIADH? Sodium levels?
euvolemic hyponatremia with continued urinary Na+ excretion
426
What are the major complications associated with type I and II DM?
Type I: DKA, Type II: Hyperosmolar coma
427
Most common cause of death in DM patient?
Myocardial infarction
428
What is the cause of cataracts and neuropathy in DM?
Osmotic damage due to sorbitol accumulation in organs with aldose reductase and low or absent sorbitol dehydrogenase
429
Antibodies with type I DM?
Glutamic acid decarboxylase antibodies
430
Which type of DM is more genetic? HLA types?
Type I: HLA DR3 and 4
| Type II: More genetic predisposition
431
Which type of DM has amyloid near the islet cells?
Type II
432
Describe the histology of type I and II DM?
Type I: Islet leukocytic infiltrate (autoimmune)
| Type II: Islet amyloid polypeptide (IAPP) deposits
433
Which ketone is favored in DKA?
beta-hydroxybutyrate>acetoacetate
434
What electrolyte must be given in DKA treatment? Why might this be overlooked in real life?
potassium even though lab value will say patient is hyperkalemic
435
What causes hyperosmolar hyperglycemia nonketotic syndrome (HHNS)?
Increased glucose causes increased osmotic diuresis. This results in dehydration that can cause HHNS if fluids are not adequately replaced
436
What might you suspect if you see a depressed patient with DVTs, hyperglycemia and skin dermatitis?
Glucagonoma
437
What vitamin deficiency might you see with carcinoid syndrome?
Niacin (the tryptophan is utilized to make 5HT)
438
Inheritance pattern of MEN syndromes?
Autosomal dominant
439
What tumors are with MEN 1, 2A and 2B?
1: Pituitary, pancreas, parathyroids
2A: parathyroids, pheochromocytoma, medullary thyroid
2B: pheo, medullary thyroid, mucosal neuromas
440
Name three syndromes associated with a marfanoid habitus.
Marfans, MEN 2B, homocystinuria
441
Which, between glargine and glulisine is rapid acting and long acting?
Glargine is long acting "glarge", glulisine is rapid
442
Which diabetes drugs can cause disulfarim-like effects?
First generation sulfonylureas (mides)
443
What are two results of increased ppar-gamma activation?
Increased adiponectin and insulin sensitivity in the periphery
444
Which drug for hyperthyroidism is used in pregnancy?
Propylthiouracil
445
What are conivaptan and tolvaptan? Name another drug with the same function.
ADH antagonists (V2 specifically), demeclocycline
446
What is fludrocortisone?
Synthetic analog of aldosterone with little glucocorticoid activity
447
What is cincacalet? What is it used for?
It is a drug that sensitizes Ca-sensing receptor in parathyroid gland to circulation calcium. Used in hyperparathyroidism (primary or secondary)
448
What is the most common cause of death in acromegaly?
Cardiac failure- GH causes growth of visceral organs
449
Etiology of sheehan syndrome?
Pituitary doubles in size during pregnancy but the blood supply doesn't increase significantly. Blood loss at partition leads to infarction.
450
What symptoms might you see in a patient with SIADH? Why?
Mental status changes and seizures because hyponatremia leads to neuronal swelling and cerebral edema
451
How does glucose level change in hyperthyroidism?
Hyperglycemic due to increased gluconeogenesis and glycogenolysis
452
Are the exophthalmos and pretrial myxedema in graves disease due to the thyroid?
No, the antibody- fibroblasts behind orbit and on shin express the TSH receptor
453
What is the difference in myxedema and edema?
Edema is due to water while myxedema is due to a myxoid substance like GAGs
454
Arrhythmia, hyperthermia and vomiting with hypovolemic shock
Thyroid storm (usually presents in stress)
455
What is dyshormonogenetic goiter?
Congenital defect in thyroid hormone production. It most commonly involves thyroid peroxidase
456
What causes the deepening of voice and large tongue in hypothyroidism?
Myxedema: accumulation of GAGs in the skin and soft tissue
457
Antimicrosomal antibodies are also known as what?
anti-thyroid peroxidase
458
What should you suspect in a hypothyroid patient with an enlarging thyroid gland late in the disease course?
B-cell (marginal zone) lymphoma
459
Suspicion in patient with fibrotic thyroid and less than 40? What if it is an old patient?
Young: riedel fibrosing thyroiditis, old: anaplastic carcinoma
460
Can you distinguish between follicular adenoma and carcinoma by FNA?
No, the capsule is the only difference
461
Name the four carcinomas that spread hematogenously.
Follicular carcinoma of the thyroid, renal carcinoma, choriocarcinoma, hepatocellular carcinoma
462
What is a classic thing that hypercalcemia can cause?
Acute pancreatitis, thus you must link this with hyperparathyroidism
463
What can thyrotoxicosis do to bone?
Increased resorption (possible up ALP)
464
What type of hypersensitivity reaction is type I DM?
Type IV (T lymphocyte mediated)
465
What causes DKA?
Generally stress-->Epi--> glucagon-->lipolysis and ketogenesis
466
How does obesity predispose to type II DM?
Obesity leads to decreased numbers of insulin receptors
467
Which renal arterioles are preferentially involved in diabetes? What does this cause?
Efferent arterioles, causes hyperfiltration injury with microalbuminuria
468
What do you suspect with watery diarrhea, hypokalemia and achlorhydria?
VIPoma
469
What do you suspect with achlorhydria and cholelitiasis with steatorrhea?
Somatostatinoma
470
HTN, hypokalemia and metabolic alkalosis with low aldosterone and low renin? Tx?
Liddle syndrome: constitutive action of ENaC channels in collecting tubules. Tramterine or amiloride
471
How does cushing syndrome cause hypertension with hypokalemia and metabolic alkalosis?
HTN: high cortisol increases sensitivity of peripheral vessels to catecholamines (specifically alpha 1)
HypoK and met. alk: at very high levels cortisol cross reacts with mineralocorticoid receptors
472
What cancer can result in adrenal insufficiency? (not adrenal)
Lung cancer: it loves to metastasize to the adrenals
473
What cell type in the adrenal medulla makes catecholamines?
Chromaffin cells
474
What do you suspect in a patient who gets weary, hypertensive and rapid heart rate while urinating?
Pheochromocytoma on the bladder wall (10% acre outside of the adrenal- rule of 10s)
475
What is the rule of 10s?
Pheochromocytoma: 10% bilateral, familial, malignant, outside adrenal medulla
476
What four syndromes are pheochromocytomas associated with?
MEN 2A and 2B, VHL, NF type 1
