Notes Flashcards
0
Q
What do you give a cancer patient on Methotrexate to ensure that he or she doesn’t suffer from pancytopenia and apthous ulcers?
A
Give Folinic Acid (or leucovorin), which is a 5-formyl-derivative of THF, which doesn’t require dihydrofolate reductase (DHF reductase) to be converted to THF. THF is a methyl donor used in amino acid synthesis, thymidilic acid synthesis, and purine synthesis. MTX acts on DHF reductase to inhibit downstream DNA synthesis of all cells, including cancer cells. Side effect is to suppress bone marrow and GI tract mucosa.
1
Q
What are the normal values for aPTT and PT? What do each of these test?
A
PT: 11-14 seconds (Extrinsic: FVII)
aPTT: 25-35 seconds (Intrinsic: FXII, FXI, FIX, FVIII)
2
Q
What is odansetron?
A
5-HT3 inhibitor of GI tract/area postrema/solitary nucleus & tract, used for nausea prevention (especially after chemotherapy)
3
Q
What traverses through the Cribiform plate?
A
CN I Olfactory bundles
4
Q
What traverses through the Optic Canal, in the middle cranial fossa?
A
CN II, opthalmic artery, central retinal vein
5
Q
What traverses through the Superior orbital fissure?
A
CN III, IV, V1, VI, opthalmic vein, and sympathetic fibers
6
Q
What traverses through the foramen rotundum?
A
CN V2 (Maxillary)
7
Q
What traverses through the foramen ovale?
A
CN V3 (Mandibular)
8
Q
What traverses through the foramen spinosum?
A
MMA (middle meningeal artery) and vein
9
Q
What traverses through the internal acoustic meatus?
A
CN VII & VIII
10
Q
What traverses through the Jugular foramen?
A
CN IX, X, XI, jugular vein
11
Q
What traverses through the hypoglossal canal?
A
CN XII
12
Q
What traverses through the foramen magnum?
A
Spinal roots of CNXI (spinal accessory), brain stem, and vertebral arteries
13
Q
What is Vernet Syndrome?
A
Lesions of the jugular foramen cause CN IX, X, XI dysfunction:
- CN IX - loss of taste from posterior 1/3 of tongue, reduced parotid gland secretion
- CN IX/X - loss of gag reflex, dysphagia
- CN X - dysphonia and hoarseness, soft palate drop with deviation toward normal side
- CN XI - SCM and trapezius muscle pareses
14
Q
Where does negative selection in T-cell maturation occur?
A
Medulla of the Thymus, with thymic medullary epithelia and dendrites. Serves to eliminate T cells that are overly autoreactive. (Positive selection occurs prior to negative selection in the thymic cortex.)
15
Q
In carbon monoxide poisoning, how will PaO2 and % carboxyhemoglobin be affected?
A
CO poisoning does not affect the partial pressure of oxygen in the plasma; it only affects the amount that is able to bind to hemoglobin. CO has a much higher affinity than O2. Carboxyhemoglobin will increase to >2%. Note: CO poisoning has nothing to do with methemoglobinemia.
16
Q
How do you calculate Attributable Risk Percent? (The % of an outcome that can be attributed to a risk factor)
A
ARP is the excess risk in the exposed population that can be attributed to the risk factor. Can use the RR to get the ARP:
ARP = (RR-1)/RR or (risk in exposed - unexposed)/risk in exposed
17
Q
What is the mainstay of DVT treatment in a pregnant woman?
A
Heparin, to increase ATIII activity which inhibits FIIa and FXa
(Coumadin, the normal drug of choice for DVT, is teratogenic!)
18
Q
What chromosome carries the Bcl gene?
A
Chromosome 18, protooncogene for B cell lymphomas
19
Q
What chromosome carries the C-myc gene?
A
Chromosome 8, oncogene, Burkitt lymphoma
20
Q
What chromosome carries the immunoglobulin heavy chain enhancer element?
A
Chromosome 14
translocations can cause cancer:
- t(14;18) is Follicular Lymphoma with Bcl
- t(8;14) is Burkitt’s with c-myc
- t(11;14) is Mantle cell lymphoma with cyclin D (G1-S transition)
21
Q
What drug has potent anti-inflammatory effects but without the side effects of bleeding and GI ulceration?
A
Selective COX-2 Inhibitors (i.e. celecoxib) do not impair platelet function because platelets predominantly express COX-1.
22
Q
How does the Parvovirus attach to RBCs?
A
P antigen
23
Q
How does HIV attach to T cells?
A
gp120 on viral envelope to CD4 on T cells, in addition to the coreceptor CXCR4 and CCR5.
24
How does CMV attach to cells?
GAGs on host cell surface
25
How does EBV bind?
EBV gp350 with CD21 on B cells
26
What is Warfarin's mechanism of action?
It inhibits vit. K dependent gamma-carboxylation of glutamic acid residues of clotting factors 2, 7, 9, 10.
27
What is used to treat Heparin-induced Thrombocytopenia?
HIT paradoxically causes thrombosis. Use argatroban, which is a direct thrombin inhibitor.
28
What is the major regulator of iron metabolism in the body?
Hepcidin, made by liver as an acute phase reactant, causes the internalization of ferroportin in enterocytes and macrophages. (Stops transport in the GI tract and stops Fe release by macrophages).
29
How do CYP enzymes affect Warfarin therapy?
CYP 450 2C9 inhibitors INCREASE activity.
CYP 450 2C9 enhancers DECREASE activity.
Rifampicin, phenobarbital, and phenytoin are universal CYP450 enhancers and will decrease the activity of Warfarin to below therapeutic levels. (Inhibitors include cimetidine, amiodarone, bactrim, and flagyl)
30
What kind of mutation is involved in HbC and HbS disease?
Missense mutation (single base substitution cause coding for a different amino acid)
HbS (glutamate -> valine) - neg to nonpolar
HbC (glutamate -> lysine) - neg to positive
The speed of Hb during gel electrophoresis: HbA>HbS>HbC
31
What organism is diagnosed by performing a germ tube test?
Candida albicans.
32
Vinca Alkaloids are used to treat what?
Cancer treatment that halts the cell cycle in Mitosis, by inhibiting spindle-microtubule formation and separation of daughter cells.
Can be neurotoxic -> peripheral neuropathy.
33
What is necessary for diagnosis of Acute Intermittent Porphyria?
Elevated ALA and porphobilinogen during acute attacks.
Usually comes with abdominal pain and neurological distress, and often due to increased activity of ALAS after decrease in heme synthesis (due to alcohol, barbiturates, and hypoxia).
34
Auer rods stain positively for what enzyme? What do these findings indicate?
peroxidase, AML
35
What is adding during the sickling test?
sodium metabisulfite
36
What cancers are related to mediastinal mass?
Thymoma, Hodgkin's lymphoma, T-ALL, DLBCL with primary mediastinal involvement.
37
What the stop codons?
UAA, UAG, UGA
Mutation to a stop codon = "nonsense mutation"
38
What are the 8 types of AML?
```
M0 - Minimally differentiated AML
M1 - AML without differentiation
M2 - AML with maturation
M3 - APL -- associated with t(15;17)
M4 - Acute myelomonocytic leukemia
M5 - Acyte monocytic leukemia
M6 - Acute erythroleukemia
M7 - Acute megakaryocytic leukemia
```
39
How do you calculate the number needed to harm?
NNH = 1 / Attributable Risk
where Attributable Risk = Event rate of intervention - Event rate of placebo
This calculation is similar to number needed to treat (NNT)
40
What disease is associated with diffuse sheets of large lymphocytes with nuclei >5x the size of small lymphocytes found on lymph node biopsy?
Large B-Cell Lymphoma
41
After transfusion, will stored red blood cells lose or gain electrolytes?
They can gradually lose intracellular potassium to the surrounding solution, potentially causing hyperkalemia.
42
What antiviral role does Raltegravir play in HIV therapy?
Raltegravir is an integrase inhibitor that ultimately halts mRNA transcription.
After viral entry and the reverse transcriptase-dependent formation of a double-stranded DNA intermediate, integrase inserts the viral dsDNA into host cell chromosomes to become a provirus. Inhibition of integrase prevents the virus from using human RNA pol II to transcribe its message.
43
Name 2 protease inhibitors used in HIV therapy.
Saquinavir, Ritonavir
44
Name a fusion inhibitor of HIV.
Enfuvirtide, which binds to HIV's gp41 on the envelope and thereby prevents it from undergoing a conformational change necessary for fusion.
45
Name a reverse transcriptase inhibitor used in HIV therapy.
NNRTIs: nevirapine, efavirenz, delavirdine
NRTIs: zidovudine, zalcitabine, tenofovir, lamivudine
46
What is maraviroc's mechanism of action?
HIV antiviral -- CCR5 antagonist that blocks HIV attachment and entry of other CCR5-tropic viruses into the the CD4+ T cell.
47
Should you increase or decrease doses of 6-mercaptopurine and 6-thioguanine when taken with allopurinol?
You should decrease the dose!
6-MP and 6-TG are activated by HGPRT, but inactivated by xanthine oxidase. If XO is inhibited by agents like allopurinol, metabolism of 6-MP and 6-TG is inhibited, and toxic levels can be achieved.
48
What do azathioprine, 6-MP and 6-thioguanine do?
Anti-neoplastic purine analogs that inhibit purine synthesis.
Used in organ rejection prevention, RA, SLE (aza)
leukemia, IBD (6MP and 6TG)
49
What enzyme can be deficient in severe combined immune deficiency (SCID) and is also over-expressed in some malignancies?
Adenosine deaminase, which converts adenosine to inosine in purine metabolism.
Pentostatin is a cytotoxic purine analog/irreversible inhibitor of ADA.
Cladribine is a cytotoxic purine analog/resistant to ADA degradation.
50
What is the major adverse effect of Zidovudine?
Bone marrow suppression
It is a DNA polymerase inhibitor.
51
What is a major adverse effect of Foscarnet?
Associated with numerous electrolyte abnormalities.
52
What two drugs are commonly associated with the formation of SJS or TEN?
Nevirapine and efavirenz, two antivirals.
53
Carbon tetrachloride causes cell injury through what mechanism?
CCl4 is oxidized by the P450 system, which forms free radical CCl3. This radical interacts with lipids in membranes, degrading them and forming H2O2, in a process called lipid peroxidation.
54
What DNA mechanism is messed up in Lynch/NPCC1 syndrome?
DNA Mismatch Repair
55
What disease puts AIDS patients at higher risk of Non-Hodgkins Lymphoma?
EBV-induced B-lymphocyte proliferation
56
H. pylori infection is implicated the in the pathogenesis of what kind of cancers?
Gastric adenocarcinoma and MALToma.
57
BK Virus primarily causes what two diseases in the immunocompromised?
Nephropathy or hemorrhagic cystitis.
58
How does nitrite exposure affect oxygenation?
Nitrites cause poisoning by oxidizing heme iron to the ferric state. (Normally, iron is in the ferrous (II) state. Methemoglobin is formed from Iron, and it is unable to bind oxygen. Thus, a state of functional anemia is induced.
Fe2+ --> Fe3+ (methemoglobin)
PaO2 will be normal because amount of oxygen dissolved in the plasma is the same and is unrelated to Hg function. All measures of bound oxygen and carrying capacity will decrease.
59
What causes a right shift in the oxygen dissociation curve?
Increased H
Increased 2,3 DPG
Increased Temp
Rightward shift = less affinity for O2, unloading is easier.
60
What causes a leftward shift?
Decreased H
Decreased 2,3 DPG
Decreased Temp
Leftward shift = increased affinity to O2
61
What kind of antiretrovirals do not require activation via intracellular phosphorylation?
NNRTIs, like nevirapine, efavirenz, delavirdine.
62
How do T-cells look on a blood smear in a patient with infectious mononucleosis?
CD8+ T cells will clonally expand in response to the EBV virus, to kill infected B cells. They will classically appear as much larger cells with abundant cytoplasm, an eccentrically-placed nucleus, and a cell membrane that appears to conform to the borders of other neighboring cells.
63
What do matrix metalloproteinases degrade?
Zinc-containing enzymes that degrade ECM and BM, composed primarily of laminin and collagens 4 and 6. Normal part of tissue remodeling and embryogenesis. Role in invasive metastases of cancer through BM.
64
What is hemoglobin Gower and when is it found in the blood?
Earliest hemoglobin in fetus, synthesized by the yolk sac and is replaced by Hb Portland, then Hb Gower 2, before HbF production begins at 10-12 weeks.
65
When do infants start switching to HbA from HbF?
First 6 months of life
66
What DVT treatment would prolong aPTT and PT, but not TT (thrombin time)?
Direct factor Xa inhibitors have the most specific activity against factor Xa, without inhibiting thrombin.
67
What kind of leukemia is associated with Down's Syndrome?
ALL and AML
ALL > 5 yrs in children with Down's
AML < 5 yrs in children with Down's
68
In a normal distribution, 95% of observations will lie between what two parameters?
+/- 2 standard deviations of the mean.
Only 68% lie within 1 SD of the mean.
99.7% lie within 3 SD of the mean.
69
What are Gardos channel blockers used for?
In Sicklers, Gardos channel blockers hinder efflux of K+ and water from the cell (via a Ca2+ dependent channel), preventing dehydration of the erythrocytes. Dehydration and deoxygenation of Hb causes HbS to polymerize. Thus, Gardos channel blockers reduce HbS polymerization.
70
What are risk factors for ovarian cancer?
Infertility, nulliparity, miscarriages, ovarian dysfunction, and use of clomiphene citrate to induce ovulation -- probably due to irregularities of reparative processes of ovarian surfaces.
71
What can decrease the risk of ovarian cancer in women?
- Use of OCPs because limits the number of ovulations a woman will have --> less ovulations --> less surface repair
- Multiparity
- Breastfeeding
72
Why do sicklers experience acute chest syndrome, abdominal pain and bone pain?
Due to vaso-occlusive events in the lungs, spleen, and bone.
73
What is one of the most common ways to get hemolytic uremic syndrome?
Eating undercooked, contaminated ground beef that has EHEC O157:H7.
74
What is the drug of choice for malaria?
Usually, in areas known to have chloroquine sensitivity, chloroquine is the treatment of choice. In Africa, there is some resistance, so you can treat with mefloquine, a quinine analog. Primaquine is required to kill vivax and ovale schizonts.
75
What is abciximab and when is it used?
Abciximab is a chimeric mouse-human monoclonal Ab against GpIIb/IIIa receptor on platelets. It is used to block the final step in platelet aggregation, and during angioplasty procedures in patients with acute coronary syndrome.
76
How is IL-2 used as a chemotherapy?
IL-2 acts to regulate the activation and differentiation of T cells to aid in tumor destruction.
77
What is Infliximab?
A monoclonal IgG1 Ab against TNF alpha, used in RA, ankylosing spondylitis, and Crohn's disease.
78
What is Bruton's agammaglobulinemia?
X-linked immunodeficiency that results in a deficiency of all forms of Ab and low B cell counts due to a defect in B cell maturation. May lack germinal centers and primary lymphoid follicles in lymph nodes.
Predisposed to recurrent pneumonias
79
What is the folate trap?
Vitamin B12 (cobalamin) is required for the conversion of homocystine + N-5-methyl-THF --> methionine + THF. A methyl group on methylated THF is transferred to homocystine.
80
Vitamin B6 is required for the synthesis of what?
Vitamin B6 is required for both steps in the conversion of homocystine to cystathionine to cysteine (with cytathionine synthase and cystathionase as enzymes, respectively.)
81
B12 is used in the process of converting _______ to succinyl coa.
Methylmalonyl CoA
Increased in B12 deficiency, but NOT in folate deficiency.
82
Inhibition or deficiency of Protein C can predispose patients to _______
Warfarin-induced skin necrosis. Warfarin decreases carboxylation and function of anticoagulant proteins C and S. Once started, it can reduce protein C activity by nearly 50%, resulting in a transient hypercoagulable state.
83
What are some natural anticoagulants found in the blood?
ATIII, Protein C, Protein S
84
What kind of receptor is JAK2 (mutated in some myeloproliferative diseases)?
Non-receptor tyrosine kinase.
In polycythemia vera, can be associated with epo-receptor.
85
What is mutated in Li-Fraumeni syndrome?
p53 tumor suppressor.
AD, predisposition to variety of cancers
86
Some cells have alternate glycolytic pathway that produces no net ATP. What kind of cells do this?
Erythrocytes.
87
What respiratory structure is covered by stratified squamous epithelium? What type of microbe has an affinity for this structure?
True vocal chords. Stratified squamous epithelia is mostly found in the oropharynx, laryngopharynx, anterior epiglottis, upper part of the posterior epiglottis and the true vocal chords.
Most of the other upper respiratory structures are lined by ciliated, pseudostratified, columnar, mucus-secreting epithelia.
A small DNA virus that has an affinity for this epithelia is a small DNA virus like HPV.
88
Why do you check the phospholipid content of amniotic fluid?
To assess fetal lung maturity, because PL is a major component of pulmonary surfactant (ie Lecithin:sphingomyelin ratio >2 is mature).
89
When you suspect fetal neural tube defects, what should you test?
Elevated AFP in amniotic fluid, which suggests closure defects like spina bifida.
90
What do you test if you want to detect a fetal adrenal disorder?
Amniocentesis, to measure 17-hydroxyprogesterone levels;
| Gene linkage analysis involving HLA region or chromosome 6; or detection of abnormal genes via molecular probes.
91
What accumulates within cell lysosomes in Tay-Sachs disease?
GM2-ganglioside accumulates in TS disease because of a deficiency in beta-hexoaminidase A.
92
What kind of inheritance pattern is observed in Tay-Sachs?
Autosomal recessive neurodegenerative disease.
93
What are telltale signs of Tay Sachs?
At 2-6 months, babies develop progressive weakness, hypotonia, loss of motor skills, abnormal startle reflex with acoustic stimuli, cherry red macula.
94
How do you distinguish Niemann-Pick from Tay Sachs?
TS has no hepatosplenomegaly.
95
What activates trypsinogen to trypsin?
duodenal enterokinase
96
Where is the duodenal bulb?
Begins at the pylorus, is posterior to the gallbladder and liver, ends at the neck of the gallbladder. Ulcers here can cause hemorrhage (as opposed to anterior bulb ulcers which can cause perforation).
97
What is the only IV agent that improves renal perfusion while also lowering BP?
Fenoldopam, a Dopamine-1 selective receptor agonist. Used for short-term management of severe hypertension and can be safely used in hypertensive crises. Causes arteriolar dilation, natriuresis.
98
What are some digoxin toxicities? Why do you see these more as the patient ages?
Visual changes and GI disturbances. Because of decline of renal clearance relative to age. Digoxin is renally cleared.
99
In congenital adrenal hyperplasia, what is the most common type?
21-hydroxylase deficiency = decreased glucocorticoids and mineralocorticoids, increased androgens.
100
What does a 17alpha-hydroxylase deficiency impair?
Less common form of CAH, which impairs androgen, estrogen and cortisol synthesis by the adrenal gland, but has normal-high mineralocorticoid production. Will see low androgen, low estrogen, boys with hypogonadism, hypertension and hypokalemia.
101
A treatment for angle-closure glaucoma also can affect which part of the nephron in the kidney?
Acetazolamide is a carbonic anhydrase inhibitor. Bicarbonate is released into aqueous humor, so a CAI will cause a decrease in intraocular pressure -> decreased secretion.
CA exists in the proximal tubule of the kidney and CAIs can block reabsorption of HCO3- in the PCT, causing an increased urine pH.
102
Large levels of serum antibody against polyribitol ribose phosphate (PRP) gives protection against what?
H influenza type B epiglottitis.
103
What are the main features of myasthenia gravis?
Waxing/waning progressive weakness of voluntary muscles (especially those innervated by brainstem motor nuclei), weakness during continued activity, rapid restoration with rest, improvement with cholinesterase inhibitors.
104
What is Myasthenia Gravis?
Auto-immune disorder with antibodies against the postsynaptic Ach-receptor, causing internalization and complement fixation. Ultimately decreases end plate potential.
105
What is a cholinesterase inhibitor that can be used for Myasthenia Gravis treatment?
Pyridostigmine can be used, but may cause GI side effects like cramping, nausea, and diarrhea. Can use Scopolamine, a selective muscarinic cholinergic receptor antagonist, to treat side effects.
106
What is fluoxetine?
Serotonin-substrate reuptake inhibitor (SSRI) used for depression.
107
What information do you need to do a PCR?
The DNA sequence in the regions FLANKING the target DNA to be amplified.
108
What kind of DNA pol do you need to use in PCR and why?
Taq polymerase because it is heat stable and will survive the denaturation phase of PCR.
109
What are the 3 major features of ataxia-telangiectasia disorder?
- Ataxia, due to cerebellar atrophy
- Severe immunodeficiency with repeated sinopulmonary infections
- Increased risk of cancer due to inefficient DNA repair and hypersensitivity to ionizing radiation
110
Loss of caudate nucleus is seen in what disease?
Huntington
111
Loss of neurons in the substantia nigrans and presence of Lewy bodies occurs in what disease ?
Parkinsons
112
What is Ataxia-Telangiectasia?
AR disease with defect in DNA repair genes.
113
How is schizoaffective disorder different from bipolar with manic episodes and psychotic features?
Schizoaffective disorder is characterized by mood symptoms with concurrent symptoms of schizophrenia. If a patient with schizophrenic history comes in with NEW mood symptoms, think schizoaffective.
114
How do you calculate an Odds Ratio?
Based on a 2x2 table, ad/bc
As opposed to a RR = [a/(a+b)] / [c/(c+d)]
115
What nerves does the lower trunk of the brachial plexus carry?
T1 and C8 spinal levels, which ultimately contribute to the median and ulnar nerves (innervate the intrinsic muscles of the hand).
116
What does injury to the Long Thoracic Nerve cause?
Winging of the scapula due to serratius anterior muscle paralysis
117
What are the clinical features of Legionella pneumonia?
- High fever with bradycardia
- HA, confusion
- Watery diarrhea
- Hyponatremia
- Gram Stain with many PMNs, but no organisms
118
Think Legionella when...
Systemic infection (ie with lung, neuro, GI), when patients come from cruise trip or hotel stay.
Unilobar infiltrate on lung CXR to lobar consolidation.
119
What organism causes cold agglutinins and pneumonia?
Mycoplasma pneumoniae --> tracheobronchitis usually in young adutls though.
120
What is Losartan?
Angiotensin Receptor Blocker (ARB), that competively binds to AT-1 receptors.
- Stops action of AT II
- Stops negative feedback on renin system, causing increases in renin, AT I, and AT II
- NO EFFECT ON BRADYKININ or ACE ACTIVITY!
121
What do rodenticides affect?
Most contain brodifacoum, which depletes vitamin K-dependent clotting factors. This causes an acquired coagulopathy. Treat immediately with fresh frozen plasma and vitamin K.
122
What does cryoprecipate contain?
F8, F13, vWF, fibrinogen
123
Almost all aminotransferases use ________ as the amino group acceptor.
alpha-ketoglutarate
For example, alanine's amino group is added to AKG to make pyruvate (for glucose production) and glutamate (which is further metabolized by glutamate dehydrogenase to ammonia, which goes into the urea cycle, and AKG)
124
_____________ is a major AA responsible for nitrogen transfer to the liver for disposal.
Alanine
125
How can prolactinomas lead to osteoporosis?
High levels of prolactin suppress GnRH from HTH, which leads to low estrogen in females --> accelerated bone loss.
126
Multiple Endocrine Neoplasia Type I (MEN I) consists of the triad:
Three P's
1. hyperParathyroid
2. Peptic ulcer due to gastrin-secreting tumor of Pancreas
3. Pituitary adenoma
127
What kind of E. coli does not classically ferment sorbitol?
O157:H7 Shiga-like toxin strains do not ferment sorbitol, unlike more other strains. (Also does not produce glucuronidase.)
128
What bacteria produce toxins that can inactivate EF-2?
Corynebacterium diptheriae and Pseudomonas
129
What bacteria can release toxins that activate guanylate cyclase and lead to watery diarrhea and electrolyte loss?
ETEC, Yersinia.
130
What is the presentation of minimal change (MCD) nephrotic syndrome?
- increased permeability of glomerular cap wall -> proteinuria
- proteinuria -> low albumin -> drop in colloid osmotic pressure -> edema
- low intravascular volume -> RAA and ADH triggered --> aldosterone -> sodium & water retention
- liver increases synthesis of proteins (ie lipoproteins -> lipiduria)
131
In the case of a thromboembolic event in the brain, what microscopic changes will you see 12-48 hours after time of injury?
Red neurons
At 24-72 h, you'll see necrosis and PMNs. At 3-5 d, macrophages. At 1-2 w, reactive gliosis and vascular proliferation with liquefactive necrosis seen macroscopically. At >2 w, glial scar.
132
What are the muscles attached to the rotator cuff?
SITS muscles: Supraspinatus, Infraspinatus, Teres Minor, and Subscapularis.
133
What muscle is responsible for the first 10-15 degrees of arm abduction? For the >30 degrees of arm abduction?
Supraspinatus
134
What are the hallmarks of catatonic schizophrenia?
Significant disturbance of motor function -- abnormal voluntary movements, excessive motor activity, echolalia (imitating other people's speech), echopraxia (imitating their movements), extreme negativism.
135
Name the type of bone for each wall of the orbit.
Superior: thick plate of frontal bone
Lateral: thick bone of zygoma and greater/lesser sphenoid wings
Inferior: very thin separating orbit from maxillary sinus
Medial: thin ethmoid and lacrimal bones, separating from ethmoid air cells.
136
What does the power of a study indicate?
Probability of seeing a difference when one truly exists. Power is reciprocally related to type II error (beta), which states there is no difference between groups where one truly exists!
Power = 1 - beta
137
What is Type I error?
Describes the probability of seeing a difference when there is NO difference in reality.
138
What is Type II error?
Probability of stating there is no difference between groups where one truly exists!
139
What kind of epithelia exists on the ovaries and Fallopian tube?
Simple columnar
140
What kind of epithelia exists on the ectocervix vs. endocervix?
```
Ecto = Stratified Squamous (ecto = "outside" like skin or vagina)
Endo = Simple columnar
```
141
What are the 3 D's of Pellagra (Niacin deficiency)?
Dementia, Dermatitis, Diarrhea
142
Vitamin A toxicity can cause _____
intracranial HTN, skin changes, and hepatosplenomegaly.
143
What kind of drugs can curb the development of tolerance to opioids?
NMDA receptor antagonists (ie ketamine) that block glutamate actions on the receptor. It is believed that activated NMDA receptors cause increasesd phosphorylation of opioid receptors and increased NO levels.
144
What chromosome is affected in Cystic Fibrosis?
Chromosome 7 - Autosomal Recessive
145
What are the chances that an unaffected individual (with unaffected parents) and one affected sibling with an autosomal recessive condition will be a carrier?
2/3. Because he or she is not homozygous, there are only 3 options.
146
What do the following findings indicate?
| - JVD, tachycardia, hypotension, pulsus paradoxus
Cardiac tamponade
147
Which type of BPH treatment improves prostate volume?
5-alpha reducases like finasteride or dutasteride help reduce prostate volume, while alpha adrenergic antagonists only help to relax smooth muscle cells of the bladder neck. Alpha adrenergic antagonists and antimuscarinics do not reduce prostate volume
148
What is pancoast syndrome?
Caused by tumor in the lung apex, which can metastasize to superior sulcus and is characterized by:
- shoulder pain radiating to axilla and hand paresis due to lower brachial plexus involvement (C8, T1, T2)
- ipsilateral Horner's syndrome
- Subclavian vessel compression -> upper ext edema
- Spinal cord compression if extensive enough
149
How would a Medium Chain acyl-CoA Dehydrogenase deficiency affect glucose and ketones?
Decrease both, especially after a period of starvation lasting 16-24 hours when peripheral tissues start relying on lipid-derived fuels. Deficiency leads to impaired beta-oxidation of fatty acids. (Saves glucose for organs that need it specifically). Induces severe hypoglycemia.
150
What is buspirone?
Buspirone is a 5HT1a agonist, and acts as tx for generalized anxiety disorder. It is safe and effective, but had weeks-long delay in onset of response. Does not cause dependence.
151
What should you be worried about if you see bilious vomiting in a few days old infant?
Sign of intestinal obstruction below duodenum, usually due to vascular accidents in utero. Can be major vessel occlusion (ie the SMA) that causes narrowing and obliteration of the lumen -> blind ending jejunum.
152
What must be true for a naked RNA to be able to induce viral protein synthesis when introduced to a host cell?
The viral RNA must be single-stranded and positive sense in order to use the host's intracellular machinery for translation. Only SSRNA+ are infective.
153
What two microbes cause PID?
Neisseria gonorrhoeae and Chlamydia trachomatis.
154
Severe PID is known to increase the risk of ________ by 50%.
ectopic pregnancy
155
What is the chloride shift?
In RBC in venous blood, CO2 is taken up from the tissues and is coverted to Carbonic acid by carbonic anhydrase. It spontaneously converts to H+ and bicarbonate, which diffuses freely into plasma. To maintain electrical neutrality, Chloride enters RBC and causes the chloride shift in the venous blood.
156
What is spectrin?
Gives RBCs cell flexibility
157
What is the mechanism of action of benzodiazepines?
GABAa receptor-chloride channel binder that increases the frequency of opening of Cl- channels, which hyperpolarizes/stabilizes the membrane (rendering it less excitable).
Sedative hypnotic with anxiolytic, muscle relaxant, and anticonvulant actions.
158
Where is the complement binding region located on IgG and IgM?
Fc receptor on heavy Ig chain region, closer to the hinge point.
IgM circulates in pentameric form, and is thus a much better activator of complement.
159
What is the drug used in medical abortion?
Mifepristone, which is a progesterone antagonist. Used with a prostaglandin-E1 analog, Misoprostol.
160
In addition to ceftriaxone, what should you give to an infant with suspected meningitis?
Ampicillin to cover Listeria.
CTX covers Neisseria meningitidis, Strep Pneumo, Strep B, H Flu B.
161
Where is epinephrine chiefly producted?
Adrenal glands, by conversion of NE to E, utilizing the Phenylethanolamin-N-Methyltransferase enzyme. (Cortisol increases the synthesis of this enzyme.)
162
Prostacyclins ___________ platelet aggregation.
Inhibits.
Produces in vascular endothelia. It vasodilates, increases vascular permeability, inhibits platelet aggregation. Oppose TXA2.
163
What is a 95% confidence interval?
It is the range of values in which one can be 95% confident that the true mean of the underlying population falls.
Calculate by Mean +/- [1.96 x SD / (n^1/2)]
164
What gene is affected in Huntington Disease?
Chromosome 4, with increased # of CAG repeats
165
What is tetrabiopterin (BH4) used for?
Cofactor used by hydroxylases in the synthesis of tyrosine, dopa, serotonin, and NO. 5HT is made from tryptophan, and its initial step is catalyzed by an enzyme that uses BH4 as a cofactor. Deficiency in dihydrobiopterin reductase uncommonly causes PKU.
166
How do you detect a neural tube defect in utero?
Amniocentesis and check for Alpha-fetoprotein and ACE in the amniotic fluid,
167
What is Hirschprung's Disease?
Failure of neural crest migration into the intestinal wall --> absence of myenteric and submucosal plexi in colon.
168
What is the most common presentation of cerebral amyloid angiopathy?
Recurrent hemorrhagic stroke, usually in lobar and in cerebral hemispheres and involving smaller areas of brain parenchyma (vs. HTN hemorrhagic strokes are larger and involve basal ganglia.
169
Name genetic diseases that arise from partial deletions.
Cri-du-chat (5p deletion), Prader-Willi (15q deletion), Angelman (15q deletion) syndromes
170
What is reaction formation?
Behavior pattern in which a person does the opposite of what he feels or desires. Usually a short-term immature response.
171
What is identification?
Refers to modeling one's behavior after someone who is perceived to be more powerful.
172
What is the difference between repression and suppression?
Repression is a relatively thorough removal of disturbing psychological material from conscious awareness. Suppression is voluntary, conscious, and typically more temporary.
173
What kind of immune deficiency is seen in Ataxia-Telangiectasia?
IgA deficiency, primarily. Predisposes to infections of upper and lower airways.
Due to impaired DNA break repair (ATM gene)
174
What is Lesch-Nyhan syndrome?
X-linked recessive, defective gene for hypoxanthine-guanine phosphoribosyltransferase (HGPRT), which leads to severe hyperuricemia and gout. See patients exhibiting self-mutilating behavior, mental retardation, choreoathetoid movements, spasticity.
175
What disease causes defective sphingolipid degradation?
Niemann Pick disease, AR defect in sphingomyelinase --> accumulation of sphingomyelin within monocytes. Death in early childhood.
Hepatosplenomegaly, anema, motor neuropathy, cherry red spot on macula.
176
What is the major function of the ventromedial nucleus of the HTH?
Mediates satiety, stimulated by leptin.
177
What is the major function of the posterior nucleus of the HTH?
Mediates heat conservation, sympathetics
178
What is the major function of the anterior nucleus of the HTH?
Heat dissipation; pArasympathetics.
179
What is the major function of the lateral nucleus of the HTH?
Mediates hunger, inhibited by leptin.
180
What is the major function of the arcuate nucleus of the HTH?
Dopamine secretion, GHRH, and gonadotropins.
181
What is the major function of the paraventricular nucleus of the HTH?
ADH, CRH, Oxytocin, Thyrotropin-releasing hormone secretion
182
What is the major function of the supraoptic nucleus of the HTH?
ADH, oxytocin secretion
183
What is the major function of the suprachiasmatic nucleus of the HTH?
Circadian rhythms regulation and pineal gland function.
184
What do topoisomerases do?
Relieve unwinding tension caused by helicases in DNA replication.
185
What removes RNA primers in DNA replication of bacteria?
DNA pol I is the only bacterial DNA pol with 5' to 3' exonuclease activity (works in both excision repair and RNA primer removal).
186
What toxin can cause a defect in the release of GABA or Glycine?
tetanus toxin - resulting in muscle spasms, spastic paralysis, and hyperreflexia
187
What does polymixin cover?
Gram negatives
188
What does the Thayer-Martin selective medium grow, and what is in it?
Isolates Neisseria species. Contains vancomycin, polymixin (colistin), nystatin (fungi, yeast), and trimethoprim (gram negatives other than neisseria, ie proteus)
189
PCP is most likely to induce _____________, leading to mortality.
violent behavior/trauma
190
What CN is affected in vertical diplopia? (AKA, what is the most common cause of vertical diplopia?)
Trochlear nerve (CN IV)
Sometimes oculomotor nerve too.
191
A vascular lesion of the ___ and ___ nuclei of the thalamus can lead to complete contralateral sensory loss.
VPL (extremities) and VPM (face)
192
What would a lesion in the internal capsule cause?
Contralateral motor deficit.
193
What is the major cause of lacunar infarcts?
Occlusion of small penetrating arteries supplying deep brain structures, usually caused by lipohyalinosis and microatheromas, as a result of uncontrolled HTN or DM.
194
What is sublimation?
Mature defense mechanisms involving channeling an unacceptable impulse into an acceptable form of behavior. (ie patient with sexually explicit thoughts becomes a sex therapist)
195
Irritation of the ______ nerve can cause dyspnea and hiccups.
Phrenic (C3-C5) nerve, which supplies the ipsi hemidiaphragm.
196
SVC syndrome usually is associated with ___________ .
SCC of the lung (centrally located)
197
What is a Wilms tumor?
nephroblastoma in 2-4yr olds, presents with palpable flank mass.
198
_______________ can cause opsoclonus-myoclonus.
Neuroblastoma -- increased N-myc genes, most common extracranial neoplasm in children. Has associated paraneoplastic syndromes like opsoclonus-myoclonus.
199
The parietal cortex is important in integrating ________ information.
Sensory
200
Destruction of the subthalamic nucleus can lead to ___________.
Hemiballism (involuntary flinging movements of extremities, contralateral to lesion).
201
How does Pick's disease present?
Atrophy of frontal lobe -- manifests as dementia, behavioral disinhibition, speech difficulties
202
What is Fragile X syndrome?
Increased number of trinucleotide repeats *CGG* in the familiar mental retardation gene, located on the long arm of the X-chromosome (appears as a gap since the repeat doesn't stain well). 2nd most common cause of mental retardation.
203
How does Fragile X present?
Mental retardation, macrosomia, large jaw/ears/forehead, long/thin face, macroorchidism.
204
Short stature, broad chest, webbed neck, and low hair line are signs of ___________.
Turner's syndrome (45 XO)
205
Klinefelter syndrome has the karyotype:
47 XXY, affects only males.
Tall, gynecomastia, small firm testes, infertility (due to low testosterone)
206
What kind of hypersensitivity reaction is involved in Erythroblastosis fetalis?
Type II -- Maternal Ab IgG formed against fetal erythrocytes.
207
What are 3 possible causes of intestinal B12 malabsorption?
pancreatic insufficiency, intestinal bacterial overgrowth, ileal disease (if not dietary nor IF deficient) -- Can tell through Schilling test with radiolabelled B12/test urine output. Then, add IF with RA B12 to see if it's due to an IF deficiency.
208
Cladribine is a _______________, resistant to adenosine deaminase and is used to treat __________________.
Purine analog, hairy cell leukemia.
209
What is the purpose of thehexose monophosphate (HMP) shunt?
1. To produce NADPH as a reducing equivalent, to maintain glutathione in a reduced state so that it protects erythrocytes from oxidative damage. Only process that makes NADPH in the erythrocyte.
2. The synthesis of ribose 5-phosphate for nucleotide synthesis.
210
What drug has oxidant properties and can precipitate hemolysis in a patient with G6PD disease?
Bactrim for UTIs.
211
What is the inheritance pattern of G6PD disease?
X-linked recessive.
212
What is paroxysmal nocturnal hemoglobinuria?
Mutation in GPI anchor of DAF (CD55) and CD59, which are cell-surface proteins that inactivate complement and protect the cell.
- Hemolytic anemia
- Hypercoag state (thrombosis is common cause of death)
- Decreased blood counts
213
CD55 and CD59 protein deficiency is diagnostic of ________.
paroxysmal nocturnal hemoglobinuria.
214
The major clinical manifestation of factor V Leiden is ______.
DVT, cerebral vein thrombosis, and recurrent pregnancy loss.
215
What is Methotrexate?
A folic acid analog that functions by binding and inactivating DHR, which reduces folate to THF. Inhibit thymidylate formation. Can be rescued by leucovorin, but 5FU cannot be rescued.
216
What is gemcitabine and cytarabine?
Pyrimidine analog antimetabolites
217
DNA damage from exposure to UV radiation leads to formation of ____________.
pyrimidine dimers (thymine dimers)
218
Vinca alkaloids are _______ phase-specific agents.
M-phase, because they prevent MT formation during mitotic spindle formation. Cannot align and segregate daughter chromosomes during mitosis.
219
Mercaptopurine is _______ phase specific
S-phase. Purine analog, associated with cholestatis and hepatitis.
220
What are androgen's effect on RBC production?
Enhances - that's why males have higher Hematocrit.
221
.The most common type of childhood neoplasm is __________
leukemia, (second is brain tumors like pilocytic astrocytomas)
222
What are medulloblastomas?
High grade, malignant brain tumor of childhood, usually in vermis, solid, sheets of small blue cells with hyperchromatic nuclei and scant cytosol. Causes severe HA and cerebellar dysfunction.
223
What is heteroplasmy?
Mixture of two type of genetic material, and is responsible for the clinical variability of mitochondrial diseases, like MELAS. (Daughter cells split mitochondria randomly during mitosis)
224
What syndrome causes immunodeficiency, albinism, and neurological defects like horizontal nystagmus?
Chediak-Higashi Syndrome, an AR disease, causes neuropathies, immunodeficiency from a defect in phagosome-lysosome fusion, and abnormal melanin storage.
225
What is DiGeorge's Syndrome?
Failure of formation of 3rd and 4th branchial arches due to mutation on Ch22, causing immunodeficiency - thymic and parathyroid hypoplasia. (recurrent infections).
226
What is the Wiskott-Aldrich syndrome?
X-linked, immunodeficiency (B and T cell), eczema, and thrombocytopenia.
227
What is the metanephric diverticulum?
Ureteric bud -- sprouts off the caudal portion of mesonephric duct; gives rise to collecting tubules and ducts, major and minor calyces, renal pelvis, and ureters.
Induces the formation of the metanephric mesoderm (gives rise to glomeruli, Bowmans space, PCT, loop of Henle, and DCT) with reciprocal exchange of inductive signals.
228
A decrease in _______ will cause achlorhydria.
gastrin
229
What kind of organisms have N-acetylmuramic acid and N-acetylglucosamine in the cell well?
Gram+ and Gram- organisms
230
What is alpha-1-antitrypsin?
Serum protein that inhibits several different proteolytic enzymes, especially neutrophil elastase and reduces inflammation.
Deficiency can cause panacinar emphysema or cirrhosis. Think about this in patients with early onset of chronic bronchitis, emphysema, as well as nonsmokers with chronic obstructive pulmonary disease.
231
Anthracycline chemotherapy is cardiotoxic and can cause ___________.
Dilated cardiomyopathy.
232
Hemochromatosis, amyloidosis, and sarcoidosis can cause what kind of cardiomyopathy?
Restrictive.
233
K+ depletion stimulates what kind of cells to reabsorb extra K?
Alpha-intercalated cells in the kidney's late distal and cortical collecting tubules. Principal cells secrete K under conditions of normal or increased K load.
234
The Posterior Cruciate Ligament attaches to what?
Attaches posteriorly to the intercondylar area of the tibia and the anterior LATERAL surface of the medial epicondyle of the femur. (ACL attaches medially to the posterior of the lateral femoral condyle.)
235
The deep peroneal nerve innervates what parts of the foot?
The extensors and the great dorsiflexors.
Prolonged compression can cause foot drop. Comes from the common peroneal, which is easy to injure because it lies at the lateral neck of the fibula.
236
Damage to what nerve will cause loss of ability to plantarflex?
Tibial nerve.
237
Damage to what nerve causes wrist drop?
Radial nerve.
238
What is a demyelinating syndrome of peripheral nerves, characterized by ascending muscle weakness and paralysis?
Guillain-Barre Syndrome.
Can be attributable to Campy infection in 10-30% of cases.
239
Immunity to reinfection for influenza type A relies on what kind of antibodies?
Adaptive immunity: specific anti-hemagglutinin IgA (nasopharynx) and IgG (serum) antibodies.
240
What kind of picornavirus is acid-labile?
Rhinovirus
| The enteroviruses are acid-stable. ie Coxsackie A, Echovirus, Poliovirus, Hep A
241
What drug can be used to treat essential tremor?
Propranolol, a beta-adrenergic antagonist.
242
What is the pattern of inheritance for essential tremor?
For the familial kind, AD.
243
What is the filtration fraction?
The ratio of GFR to Renal Plasma Flow = GFR/RPF
Normally, 1/5 of the plasma that passes through the glomerular capillaries is filtered into the bowman's capsule.
244
Where does synthesis of steroid hormones and drug detoxification occur?
SER
245
What do eplenerone and spironolactone treat?
These are aldosterone antagonists, used for Conn's syndrome treatment (aldosterone-secreting adenoma) or for hypertension.
246
What is the site of action for aminoglycosides?
30S ribosomal subunit.
247
Unlike loop diuretics, thiazides can cause hyper________.
Hypercalcemia.
248
All protease inhibitors, as part of HAART therapy, can cause these adverse effects:
Lipodystrophy (buffalo hump), hyperglycemia (increased insulin resistance), P450 inhibition.
249
Indinavir is a protease inhibitor that can cause specific side effects such as ______________.
Nephrotoxicity and nephrolithiasis.
250
What is Pemphigus Vulgaris?
Autoimmune disorder with Ab against Desmogleins 1 and 3 (desmosomal), causing painful, flaccis bullae and skin erosions that spread laterally upon pressure (asboe-hansen sign) and form new bullae with gentle traction (Nikolsky sign).
251
What disease is characterized by autoantibodies against hemidesmosomal proteins?
Bullous pemphigoid
252
What are the three cardinal findings in Wiskott-Aldrich syndrome?
1. Eczema, 2. Recurrent Infections of encapsulated organisms (B and T cell disorder), 3. Thrombocytopenia.
X-linked.
253
What drug should you use to mitigate the effects of cisplatin therapy?
Amifostine is a cytoprotective free-radical scavenging agent used to decrease the cumulative nephrotoxicity associated with platinum-containing agents, which form reactive oxygen species that can form DNA crosslinks.
254
What is the name of a G-CSF analog used to stimulate the proliferation and differentiation of granulocytes?
Filgrastim.
255
What is fomepizole?
Antidote in rubbing alcohol or antifreeze (ethylene glycol) poisoning, through competitively antagonizing alcohol dehydrogenase. Prevents conversion into toxic metabolites.
256
What can you use to mitigate the effects of doxorubicin or other anthracyclines?
Dexrazoxane is an iron chelating agent that can help prevent cardiotoxicity and CHF.
257
Unlike LMWH, unfractionated heparin can bind to both _______ and __________.
Antithrombin and thrombin
258
Dysphagia in a patient with iron-deficiency anemia and spoon nails is suspect for __________.
Plummer-Vinson syndrome and esophageal webs.
259
Kozak consensus sequence occurs on mRNA with ___ or ___, three bases before a start codon like AUG.
Adenine and Guanine
Mutation in this position is associated with thalassemia intermedia.
260
Basophilic stippling can occur in what condition?
Lead poisoning -- stippling on a background of hypochromic microcytic anemia, on peripheral blood smear.
261
How are wet and dry age-related macular degeneration (AMD) different?
Wet: abnormal blood vessels with subretinal hemorrhage/fluid, gray subretinal membrane. Treat with Anti-VEGF.
Dry: subretinal drusen deposits or pigment changes. Dry progresses to wet usually.
262
EGFR inhibitors like erlotinib and gefitinib are used for what kind of cancers?
non-small lung cell cancer.
263
What is the structure of the Parvovirus?
Parvoviruses, like B19, are single-stranded DNA viruses with no envelope.
264
What is the major cause of aplastic crisis in sicklers?
Parvovirus B19
265
Why are sicklers prone to megaloblastic anemia?
Sicklers get folate deficiency from rapid turnover of erythrocytes. Folate amounts cannot keep up with DNA synthesis and impair its process, so cells divide slowly and end up larger than normal.
266
How do pufferfish cause poisoning?
Release tetrodotoxin, a neurotoxin that binds to voltage gated sodium channels in nerve and cardiac tissue, preventing sodium influx and depolarization.
267
When symptoms of schizophrenia are present for a period of 1-6 months, ____________ disorder is the diagnosis
schizophreniform. >6 mos: schizophrenia
30-50% of schizophreniform patients will NOT develop schizophrenia
268
What is acting out?
Immature defense mechanism, in which one avoids unacceptable feelings by behaving badly. ie throwing a tantrum
269
What is displacement?
Immature defense mechanism -- transferring feelings to a more acceptable object,
270
What is projection?
Immature defense mechanism, in which one attributes one's own feelings to others.
271
What are craniopharyngiomas?
Calcified cystic tumors arising in the remnants of the Rathke's pouch, causing HA, growth failure, and bitemporal hemianopia.
272
What do blotchy red muscle fibers on gomori trichrome stain mean?
This finding indicates that there is a mitochondrial myopathy, inherited from a mother.
273
What virus can have dsDNA dormant in the sacral dorsal root ganglia?
HSV-1 and HSV-2 and VZV
274
What is the most common cause of aseptic meningitis?
Enterovirus - ssRNA (Coxsackie, echo, polio), fecal-oral transmission.
Poliovirus can cause LMN injury.
275
What organs does Von Hippel-Lindau disease affect?
Rare, AD disease. Causes capillary hemangioblastomas in retina and cerebellum; causes congenital cysts in the kidney, liver, and pancreas. Increased risk for renal cell carcinoma.
276
What is Sturge Weber syndrome?
Rare, congenital neurocutaneous disorder -- facial angiomas, leptomeningeal angiomas. Skin involvement overlying V1 and V2 distributions. Seizures, mental retardation, hemiplegia, skull radioopacities.
277
What is the inheritance pattern of myotonic dystrophy?
Autosomal dominant, due to abnormal trinucleotide repeat expansion *CTG*.
See cataracts, frontal balding, gonadal atrophy, type 1 muscle atrophy
278
What does kinesin do?
ATP-dependent motor protein that causes anterograde transport
279
What are the four major characteristics of Horner's syndrome?
Partial Ptosis (Muller muscle, symp control)
Miosis (dilator pupillae muscle, symp control)
Anhydrosis (sweating, symp control)
Enopthalmos (deeper in orbit)
280
How does juvenile pilocytic astrocytoma present?
Well differentiated tumor in the cerebellum, brainstem, hypothalamic region, or optic pathways, with spindle cells with hair-like glial processes, mixed with ROSENTHAL FIBERS and granular eosinophilic bodies.
281
What histological finding is key in Ependymomas?
Pseudorosettes with glial fibrillary acidic protein (GFAP)
282
Autosomal dominant polycystic kidney disease can cause subarachnoid hemorrhage (berry aneurysm burst). If a patient has a negative CT scan, what else must you do to rule out a SAH?
Lumbar puncture to measure presence of xanthochromia (blood) in CSF. Most sensitive test for SAH.
283
What is the most common cause of sporadic encephalitis?
HSV-1, at the temporal lobe
284
Where does HSV-1 encephalitis usually occur?
Predilection to the temporal lobe.
285
Where are the nitrogen atoms in urea derived from?
NH3 and aspartate in the urea cycle.
286
What is the enzyme that catalyzes the rate-limiting step in the urea cycle?
Carmaboyl Phosphate Synthetase I (CPS1) (activated by N-acetylglutamate)
287
What is the most common finding with posterior cerebral artery stroke?
Contralateral homonymous hemianopia with macular sparing.
288
Tetracyclines should be avoided during pregnancy, because they can cause ______________ and discoloration of _____________.
fetal bone growth retardation and discoloration of deciduous teeth because they localize to dentin and enamel of developing teeth.
289
What disease causes "streak ovaries," infertility and amenorrhea and may also involve short stature, webbed neck, and coarctation of the aorta?
Turner's syndrome (45 XO)
290
What does pretibial myxedema or exopthalmos indicate?
Late manifestation of Grave's disease, which is an autoimmune condition caused by the production of thyrotropin receptor-stimulating antibodies --> hyperthyroidism.
291
Why will girls, in the few years following menarche, experience prolonged, heavy menstrual bleeding and marked variability in cycles?
Anovulatory cycles cause endometrial lining to remain in a proliferative phase until ovulation occurs regularly.
292
Polyprotein product is part of the viral life cycle of what kind of viruses?
Echoviruses
cleaved by viral protease to generate a complete set of functional, individual proteins from one mRNA.
293
What muscle opens the jaw?
Lateral pterygoid, innervated by V3
294
To be statistically significant, the confidence interval must not contain _________.
The null value of 1.0, when referring to relative risk. When this is met, this equates to finding that the p value is less that 0.05.
295
What is the treatment for hypertriglyceridemia?
Lifestyle -> Fibrates (activate peroxisome proliferator-activated receptor alpha, PPAR-alpha) -> Niacin (decreases synthesis of hepatic TGs and VLDL) and fish oils (decrease hepatic VLDL and TGs by 20-30%).
296
What is the treatment for hypercholesterolemia?
Lifestyle -> statin -> Ezetimibe (inhibits intestinal absorption of cholesterol)
297
What is the tx for Low HDL?
Lifestyle -> Niacin
298
_________ are the cause of pharyngoconjunctival fever that classically occurs in small outbreaks in small living quarters (summer camp, military recruits).
Adenoviruses
299
Direct inguinal hernias occur where in relation to the inferior epigastric vessels and due to what?
Medial to the inferior epigastric vessels and due to weakness of the transversalis fascia.
300
Indirect inguinal hernias are located where and due to what?
Located lateral to inferior epigastric vessels and due to persistent processus vaginalis and failur of the internal inguinal ring to close. Usually in male infants! Covered by three spermatic fascial layers.
301
During Acetaminophen toxicity, NAPQI (toxic) is generated and the liver's ability to do sulfation and glucuronide conjucation is saturated. What do you treat patients with?
N-acetyl cysteine (NAC), which acts as a glutathione substitute and binds to the toxic metabolite. Provides sulfhydryl groups to enhance the nontoxic sulfation elimination of tylenol.
302
SERMS like ______________ can have tissue-specific behavior, like imitating or antagonizing the activity of estrogen.
Raloxifene.
Agonist in bone, Antagonist in breast tissue and uterus
303
What is the inheritance pattern for the familial version of pulmonary artery hypertension?
Thought to be autosomal dominant, but follows a two-hit hypothesis.
A mutation in the BMPR2 gene (first insult, predisposes to pulmonary vasculature disease) followed by a second insult like infection, drugs and ion channel defects that cause vascular proliferation and elevated pulmonary pressures.
304
What is Hemachromatosis?
HFE gene on short arm of Ch6 codes for an HLA type 1-like molecule that affects iron absorption from the GI tract.
305
When can cardiac free wall rupture occur (usually)?
Consequence of an acute MI, usually 3-7 days after the onset of total ischemia, when coag necrosis, PMN infiltration and enzymatic lysis of CT have weakened the myocardium.
306
The Mesonephric ducts develop into what structures?
epididymis, ductus deferens, and seminal vesicles.
307
What do the paramesonephric ducts give rise to?
Uterine tubes, uterus, cervix, and upper vagina (under the influence of estrogen)
308
What gives rise to the glans penis (males) and glans clitorus (females)
Genital tubercle.
309
What does the urogenital sinus become?
In males: bladder, urethra, prostate, and bulbourethral glands
In females: bladder, urethra, lower vagina, and bartholin glands
310
What virus has partially double stranded circular DNA and reverse transcriptase?
HBV, part of the hepadnavirus family of DNA viruses
311
Where does Phencyclidine (PCP) work?
NMDA receptor antagonist, hallucinogen
characterized by aggressive, violent behavior and nystagmus.
312
At peak exercise, a normal person would have the following parameters = in the peripheral and pulmonary circulations?
Blood flow per minute
313
What antituberculous agent is notorious for causing visual changes?
Ethambutol, affects bacterial carbohydrate polymerization in cell wall by inhibiting arabinosyl transferase.
314
Isoniazid does what?
Antituberculous agent, inhibits mycolic acid synthesis
liver toxicity and peripheral neuropathy
315
What drugs are associated with Achilles' tendon rupture?
Fluoroquinolones like ciprofloxacin
316
Dopamin inhibits prolactin secretion from the pituitary gland via what pathway?
The tuberoinfundibular pathway that connects HTH and the anterior pituitary.
317
What does the mesolimbic-mesocortical system do?
Regulates behavior, associated with schizophrenia
318
What does the nigrostriatal pathway do?
Regulates the coordination of voluntary movements, associated with parkinsonism
319
What type of diarrhea is seen in Carcinoid or Crohn's?
Secretory
320
Insulin binds to what kind of receptor and indirectly activates what second messenger?
Tyrosine kinase receptor, activates intracellular protein phosphatase (dephosphorylates glycogen synthase to activate it and dephosphorylates fructose 1,6-bisphosphatase to inactive gluconeogenesis).
321
Can women with Turner's syndrome get pregnant?
Due to premature ovarian failure, usually cannot without an oocyte donation, but <5% do get pregnant without medical help.
322
What kind of infections necessitate a working cell-mediated immunity?
Intracellular pathogens like Legionella, Neisseria gonorrhea, Listeria, viruses, protozoans. Escape humoral immunity.
323
In a positively skewed distribution, what is the shift sequence in the positive direction?
mean>median>mode, where mean is most positively shifted.
324
Bupropion carries risk of __________, especially in patients with anorexia.
Seizures.
Antidepressant, anti-tobacco dependence, hypoactive sexual disorders -- inhibits presynaptic uptake of dopamine and NE.
325
Medications with risk of seizures are:
Bupropion, Isoniazid (without pyridoxine), clozapine, ciprofloxacin, and imipenems
326
What is the mechanism of action of Sildenafil?
Inhibits cGMP phosphodiesterase in target cells, thus increasing cGMP intracellularly. Effect is similar to NO or ANP second messenger system.
327
What are the adult structures developed from the 1st branchial arch?
Mandible, maxilla, malleus, incus, zygoma, vomer, palate, temporal bone. Associated with trigeminal nerve.
Muscles: muscles of mastication, anterior belly of the digastric, mylohyoid, tensor tympani, and tensor veli palatini.
328
What are the structures related to the second branchial arch?
Associated with facial nerve.
Bones: Styloid process, lesser horn of the hyoid, and the stapes (NC derived)
Muscles: muscles of facial expression, stylohyoid, stapedius, and posterior belly of the digastric.
329
What is the mechanism of action of oseltamivir?
Antiviral, sialic acid analog inhibitor of neuraminidase (Inf A and B virus), does not require intracellular activation.
330
Foscarnet has many actions. Name them.
- inhibits DNA pol in herpesviruses
- inhibits reverse transcriptases in HIV
- inhibits RNA pol
- does not require intracellular activation
331
What is deposited in the glomerulus to create the crescent finding characteristic of rapidly progressive glomerulonephritis (RPGN)?
Fibrin deposits, glomerular parietal cells, monocytes, and macrophagaes.
332
What are the 4 stages of iron poisoning?
1. nausea, diarrhea, abdominal pain
2. GI symptoms resolve
3. metabolic acidosis, hepatic dysfunction, and hypoglycemia
4. scarring of GI tract
333
What are the signs of lead poisoning?
Colicky abdominal pain, blue line at gum tooth line, wrist/foot drop, microcytic/hypochromic anemia with basophilic stippling.
334
Polyarteritis Nodosa is associated with what infection in 10-30% of cases?
HBV infection.
Transmural, segmental, necrotizing inflammation of medium to small sized arteries.
Causes fever, weight loss, malaise, abdominal pain, melena
335
What constitutes 95% of the lung epithelial lining?
Type I pneumocytes
336
Echinocandin antifungals do what?
block synthesis of glucans in cell wall.
ie caspofungin (most active against candida and aspergillus) and micafungin
337
What can predispose you to bladder cancer?
Smoking, Occupational exposures (rubber, etc)
338
Use of sildenafil with what other drug is absolutely contraindicated?
Nitrates -- can cause high cGMP and severe hypotension.
339
What branchial arch gives rise to the Common carotid artery and the proximal internal carotid artery?
3rd pharyngeal and aortic arch
340
What pharyngeal arch gives rise to a portion of the Maxillary artery?
1st pharyngeal/aortic arch.
341
What pharyngeal arch gives rise to the subclavian arteries and the true aortic arch?
4th pharyngeal arch
342
What pharyngeal arch gives rise to the pulmonary artery and the ductus arteriosus?
6th arch
343
What is paroxysmal supraventricular tachycardia?
Common dysrhythmia with a re-entrant circuit in the AV node. Treat with adenosine or with vagal maneuvers like carotid sinus massage or valsalva.
344
What does daptomycin treat and how?
MRSA and gram positives, by causing depolarization of the bacterial cell membrane and inhibition of DNA, RNA, and protein synthesis.
Causes myopathy and CPK elevation.
345
How do you calculate false negatives, given the number of people who actually have the disease?
False negatives = (1 - Sensitivity)*number of people with the disease
346
What cardiac anomaly is associated with Turner's syndrome?
Coarctation of the aorta.
347
Down Syndrome is associated with what cardiac defect?
ASD (ostum primum)
348
What cause pulsus parvus et tardus?
Aortic stenosis
349
What does a third heart sound indicate?
Low-frequency sounds just after S2, commonly associated with increased left ventricular end-systolic volume, which occurs in the setting of left ventricular systolic failure.
350
Of the calcium channel blockers, which drug is the most effective on the heart and which is most selective for the peripheral vasculature?
Verapamil - heart
Diltiazem - between
Nifedipine - peripheral vasculature
351
What does the QRS complex correspond to?
Corresponds to ventricular depolarization and phase 0 on the myocyte action potential graph. (Class I anti-arrhythmics modulate inward Na movement).
352
Norepinephrine stimulates _______ adrenoreceptors, which utilize cAMP pathway.
beta-1, to increase contractility of heart.
353
In what leads would a transmural septum infarct cause an ST elevation?
Leads V1 and V2
354
In what leads would a transmural anterior left ventricular infarct cause an ST elevation?
Leads V3 and V4
355
In what leads would a transmural lateral wall infarct cause an ST elevation?
Leads V5-V6
356
Homocysteine is converted to ____________ using methylcobalamin and methyl-THF.
Methionine.
357
The result of insufficient ___________ is patchy atelectasis of alveoli due to increased surface tension.
Surfactant.
358
By infusing saline into a patient with severe blood loss, you are increasing ventricular preload, thereby extending the end-diastolic _______________ length.
ventricular sarcomere
359
Only _______ viruses can do reassortment
segmented
nonsegmented, like adenovirus, cannot reassort
360
Aldolases are responsible for metabolizing fructose 1,6-bisphosphonate to G3P and DHAP. A deficiency in this enzyme is life-threatening. What sugars must be avoided in the diet?
Sucrose --> fructose and glucose.
361
Thiazide diuretics cause HYPER____ and HYPO____.
Hyper: uricemia, calcemia, glycemia, lipidemia
Hypo: kalemia, tension
362
What would increase Alk Phos?
Biliary disease, pregnancy, bone disease, malignancy, or total parenteral nutrition.
363
What are kupffer cells?
Macrophage-derived phagocytic cells in the liver sinusoids in the RESystem
364
What kind of hypersensitivity is involved in acute post-strep glomerulonephritis?
Type III, immune complex
365
______ and ______ are released by the walls of the atria and the ventricles and counteract endothelin, sympathetic effects, and angiotension II.
ANP and BNP, work through guanylate cyclase to increase cGMP to cause vasodilation and diuresis/natriuresis. BNP is Elevated in CHF patients, released when ventricles are stretched often as in CHF.
366
What hormone produced by the kidney constricts veins and dilates arteries to increase kidney perfusion?
Bradykinin, metabolized by ACE.
Patients on ACEIs can get angioedema due to metabolism of bradykinin.
367
In patients with COPD, O2 supplementation is bad because it can cause _____
Brain centers use hypoxia to drive respiratory function. Can lead to resp suppression and coma. (In normal adults, brain uses hypercapnia and pCO2 to mediate resp drive. pCO2 is the most potent cerebrovasodilator).
368
Lesions to what nerve can cause quadriceps weakness, loss of patellar reflex, and loss of sensation over the anterior/medial thigh and medial leg?
Femoral nerve, which runs through the psoas major muscle in the retroperitoneum, emerges laterally between the psoas and the iliacus and then runs beneath the inguinal ligament of the thigh. May also have pain in the inguinal area.
369
In 11beta-hydroxylase deficiency (CAH), you may see hypertensiona and hypokalemia. Why?
Buildup of a weak mineralocorticoid, 11-deoxycorticosterone, which can lead to development of low-renin HTN and hypokalemia, even in the setting of low aldosterone synthesis.
370
UV Radiation can damage DNA and form _________ that must be repaired by UV-endonucleases and replaced by polymerases/ligases.
Thymine dimers.
371
What is the action of desmopressin (DDAVP)?
Synthetic analog of vasopressin (ADH) that increases vWF release from endothelial cells, and can be used in mild vWF disease.
372
What are the nonpolar, hydrophobic AAs?
valine, alanine, isoleucine, methionine, and phenylalanine
Often are integral membrane proteins in the transmembrane domains (composed of alpha helices)
373
What bug is an intracellular Gram+ rod, with a narrow zone of beta hemolysis, and shows tumbling motility at low temperatures? What part of the immune system is necessary to deal with this infection?
Listeria monocytogenes, cell-mediated immunity since it is intracellular.
374
What drug used in inferior MI treatment of bradycardia is contraindicated in patients with angle closure glaucoma?
Atropine decreases vagal influences on the SA and AV nodes, causing an increase in HR. However, it also increases intraocular pressure and can precipitate angle-closure glaucoma in susceptible people.
375
What should you worry about in a patient with fever, strawberry tongue, and a rash?
Kawasaki disease and complication of coronary artery aneurysm. Distinguish this clinical picture from Scarlet fever, which will present usually with URI symptoms and pharyngitis.
376
What lung findings may you see in a patient with septic shock?
ARDS, with pulmonary interstitial and intra-alveolar edema, inflammation, and alveolar hyaline membranes.
377
What can you treat psychotic patients who are on medications that cause extrapyramidal symptoms?
Benztropine and dephenhydramine.
378
Blockade of __________ receptors causes Orthostatic hypotension.
alpha 1 adrenergic
379
What is enoxaparan?
LMWHeparin, binds and activates AT III, anticoagulant, effective in reducing death and recurrent when used in acute MI setting.
380
What are thionamides, methimazole and propylthiouracil?
antithyroid medications that decrease the formation of TH via inhibition of thyroid peroxidase, which stops formation and coupling of iodine products.
PTU also inhibits peripheral conversion of T4 to T3
381
What is the treatment for moderate 21-hydroxylase deficiency CAH?
ACTH-suppression by administering low doses of corticosteroids. (Low endogenous corticosteroids leads to increased ACTH, which cycles back to increasing androgen production.)
382
What disease predisposes patients to nosebleeding, severe menorrhagia, and bleeding of other mucosal sites?
vWF deficiency/disease
383
Enzyme deficiencies in the early steps in porphyrin synthesis cause ___________ manifestations while derangement in later steps lead to _________.
neuropsychiatric; photosensitivity.
384
What does cyanide bind to?
Cytochrome a-a3 complex, which is critical for electron transport during oxphos. Treat with amyl nitrite (oxidizing agent) to create methemoglobin which binds cyanide, to save mitochondria from CN.
385
____________ is a deficiency in ____________, which leads to subperiosteal hematomas, hemarthrosis, hemorrhages, gingival swelling, anemaia, impaired wound healing and weakened immunity.
Scurvy, due to Vitamin C deficiency.
386
What is tumor lysis syndrome, and what is its effect on the kidney?
Oncologic emergency due to tx of cancers with rapid cell turnover, in which large amounts of intracellular ions and nucleic acids are released into the serum. These nucleic acids are catabolized into uric acid and can cause obstructive uropathy and acute renal failure. In addition, electrolytes like K+ can cause arrhythmias. Use allopurinol or rasburicase (converts urate to allantoin which is much more soluble).
387
What is the cause of most cases of homocystinuria, and what is the Tx?
Cystathionine synthetase deficiency, which causes Marfanoid features, ectopia lentis, developmental delay, with high risk of developing thromboembolism. 50% of affected patients respond to B6 pyridoxine supplementation, which is the cofactor for Cystathionine synthetase.
388
TNF alpha inactivates insulin receptors by what mechanism?
Serine residue phosphorylation, which inhibits the ability of insulin to cause tyrosine phosphorylation, and hinders downstream signaling -> insulin resistance.
389
What is amitryptiline and what are its major side effects?
Amitryptiline is a TCA, used for depression or peripheral neuropathy. It is an anti-cholinergic drug, and will have a wide range of SE related to anti-cholinergic effects: Tremor, insomnia, conduction defects, arrhythmias, hypotension, hyperthermia, flushing, ileus, urinary retention, sinus tachycardia, peripheral vasodilation, sedation
390
What enzymes does lead bind to?
ALAD and ferrochelatase are enzymes most sensitive to lead inhibition. (part of the heme synthesis pathway)
391
What are the most effective agents for chronic preventative treatment of acute asthma exacerbations?
Inhaled glucocorticoids like fluticazone. (not for acute episodes)
392
What is theophylline?
bronchodilator via phosphodiesterase inhibition. Can cause seizures, tachyarrhythmias, vomiting, abd pain, diarrhea.
393
Why do PMN counts increase after steroid therapy?
As a result of demargination of leukocytes previously attached to the vessel wall. Most other WBC counts will decrease.
394
What gives the femoral head its blood supply? Which is most susceptible to injury?
Superior and inferior gluteal arteries PLUS the medal and lateral femoral circumflex arteries (trochanteric anastomoses). The medial femoral circumflex is the largest contributor to the femoral blood supply and is most vulnerable to damage due to its close association with the posterior aspect of the femoral neck. (Can lead to avascular necrosis).
395
During which phase of acute tubular necrosis do patients become dehydrated and develop severe hypokalemia due to high volume, hypotonic urine?
The recovery phase.
396
What kind of murmur would a patent ductus arteriosus cause?
continuous murmur heard best at the left infraclavicular region with maximal intensity at S2.
397
What happens to C peptide after it is cleaved from insulin?
Secreted in islet cell granules in equimolar concentrations with insulin.
398
What is the mechanism of action of anastrozole in the tx of breast cancer metastasis?
It is an aromatase inhibitor, that blocks the peripheral conversion of androgens to estrogen, which is the main hormone responsible for the growth and development of malignant breast tumors.
Other specific, potent third generation aromatase inhibitors are letrozole, exemestane.
399
What is the treatment for HER2/neu positive breast cancers?
Trastuzumab, a monoclonal antibody against HER2/neu receptors (which normally leads to activation of a tyrosine kinase pathway).
400
What fungus can colonize the bronchial mucosa and complicate asthma or cystic fibrosis?
Aspergillus fumigatus can do so via a hypersensitivity reaction, resulting in Allergic Bronchopulmonary Aspergillosis and eosinophilia, high serum IgE and IgG against Aspergillus. May see eventual proximal bronchiestasis.
401
What is positive predictive value?
Represents a test's ability to correctly identify those with the disease from all those who had positive results. Depends on the prevalence of the disease.
402
How does IgG act as an opsonin?
By binding Ag at its Fab site and subsequently binding a phagocyte at its Fc site of the heavy Ig chain, near the carboxy terminal.
403
What is the treatment for PID?
Need to use a 3rd generation cephalosporin to treat the Neisseria gonorrhea, but you also need to use azithromycin or doxycycline to treat chlamydia (which is not sensitive to beta lactams).
404
What is the first-line HTN treatment for pregnant folk?
Methyldopa.
405
How do corticosteroids exert their anti-inflammatory effects?
- Inhibits PLA2 activity, decreasing prostaglandin and leukotriene synthesis.
- Depress immune response by reducing macrophage phagocytosis and IL-1 secretion.
406
In patients with chronic AS and concentric LV hypertrophy, what contributes significantly to left ventricular filling?
Atrial contraction and atrial kick. Afib can cause loss of this contraction and reduce LV preload/CO sufficiently to result in dangerous hypotension.
407
What are the 3 main manifestations of Sicklers?
Hemolysis, vasoocclusive symptoms (ie dactylitis/hand foot syndrome, small bone infarctions), Infections.
408
What are the 3 F's for risk factors for gallbladder disease?
Forty, Fat, Female.
409
What stimulates the release of CCK?
CCK is released by I cells when fat-protein-rich chyme enters the duodenum. It functions to increase pancreatic enzyme secretion by acinar cells and gallbladder contraction and to decrease gastric emptying. Biliary colicky pain can occur when an inflamed gallbladder contracts.
410
In healthy individuals, what is the major stimulator of respiratory drive? What about in patients with lung disease?
```
Healthy: PaCO2, detected by central chemoreceptors
Prolonged hypercapnia (COPD): Hypoxia, detected by carotid/aortic bodies
```
411
What is the most aggressive type of lung CA and has no indication for surgical resection?
Small cell lung cancer, which already usually has mets at time of presentation.
412
Where is the Posterior Cruciate Ligament and what is its function?
Originates from the anterolateral surface of the medial femoral condyle and inserts into the posterior intercondylar area of the tibia. It prevents the posterior displacement of the tibia relative to the femur.
413
What is tachyphylaxis and how does it relate to rhinitis and nasal congestion treatment?
Rapidly declining effect after few days' use. Occurs because of decresaed production of endogenous norepinephrine from the nerve terminals due to a negative feedback mechanism, resulting in relative vasodilation and congestion.
414
What passes through the Inferior Orbital Fissure?
Maxillary Nerve (CN V2), Infraorbital vessels, and branches of the sphenopalatine ganglion. (DO NOT ENTER THE ORBIT)
415
What do you use to treat a norepinephrine leak, causing local tissue necrosis at the site of infusion?
NE Leak causes intense Alpha-1 adrenoreceptor mediated vasoconstriction that can lead to local tissue necrosis. Give phentolamine or other alpha receptor blockers to cause vasodilation.
416
Of the nitrates, which is the most bioavailable?
Isosorbide-5-mononitrate is an active metabolite of isosorbide dinitrate (dinitrate undergoes major first pass metabolism), with a BA close to 100%
417
What kind of tissue does a 99mmTc-pertechnetate scan detect?
presence of gastric mucosa (ie Meckel diverticulum with ectopic gastric mucosa, which is due to a failure of obliteration of the omphalomesenteric duct.)
418
Rank the following in order of least susceptible to organ ischemia due to thromboembolic infarction: kidney, spleen, heart, CNS, liver
Liver < spleen < kidney < heart < CNS
419
What are some telltale signs of opioid withdrawal?
dilated pupils, piloerection, hyperactive bowel sounds, lacrimating/yawning.
420
Endocardial cushion defects are commonly associated with what congenital disease?
Down Syndrome.
421
What is DiGeorge Syndrome?
Thymic aplasia and failure of parathyroid formation --> defective development of 3rd and 4th pharyngeal pouches. Ch 22q11.2 deletion is found in 90% of cases.
- T cell deficiency
- Tetralogy of Fallot, aortic arch abnormalities
422
Turner's syndrome is associated with what heart defect?
Coarctation of the aorta.
423
Friedrich's ataxia is associated with what heart defect?
Hypertrophic cardiomyopathy
Frataxin mutation, gait ataxia, AR
424
Is secretion of conjugated bilirubin into the biliary system through passive or active transport?
Active transport.
MRP2 protein (ATP-binding), causing backup of conjugated bili (leaks out passively out of hepatocytes' BL surface and reaches high serum levels that then are excreted in the urine).
425
What jaundice mechanisms are caused by increased unconjugated bilirubin?
Excessive production of bilirubin
Reduced hepatocyte uptake (passive)
Impaired conjugation
426
What jaundice mechanisms are caused by increased conjugated bilirubin?
Decreased hepatocellular excretion
| Impaired bile flow (intra and extrahepatic)
427
What disease is indicated by distended macrophages in the intestinal lamina propria?
Whipple disease
428
What is Wilson's Disease?
AR disorder characterized by toxic accumulation of Cu within organ tissues, esp. liver, brain, and eye. In the liver, Cu is incorporated into alpha2 globulin to form ceruloplasmin. It is secreted into bile and excreted in stool normally.
429
What is ticlopidine used for and why isn't it used so often?
Used in patients who are allergic to aspirin or clopidogrel (another ADP-antagonist, antiplatelet drug) in the Tx of MI prevention. It is not used that often because of severe SE, like neutropenia, fever, mouth ulcers.
430
First-time use of ACE inhibitors must be carefully monitored because of what early side effect? What other drugs should you be worried about using concurrently?
ACEIs can cause first-dose hypotension (perhaps due to vagal stimulation) and can be potentiated by concurrent use of loop diuretics that already cause hypovolemia/hyponatremia.
431
In Duchenne muscular dystrophy, what is mutated?
Dystrophin, a structural muscle protein.
X-linked condition.
432
Carpal tunnel syndrome can be associated with other medical states?
High volume states (Renal failure, dialysis, pregnancy), DM, acromegaly, RA.
433
What is another chemical name for lactose?
Galactosyl-beta-1,4-glucose -- TRICKY!
434
How many types of histones are there, and which one is not part of the histone core?
5 - H1, H2A, H2B, H3, H4
H1 is the outside core, involved in DNA packaging of nucleosomes into more compact structures.
435
What is the polyol pathway and what morbidity is it associated with?
In hyperglycemic states, glucose enters cells of tissues like the lens and the seminal vesicles (which use fructose for energy), and is converted into Sorbitol by aldose reductase. Then, sorbitol is converted to fructose by sorbitol dehydrogenase, which limits the amount of accumulated sorbitol. However, in hyperglycemic states, this dehydrogenase is overwhelmed. Sorbitol still accumulates and leads to osmotic pressure. Water influxes into lens cells --> hydropic lens fibers degenerate --> cataract formation.
436
When a healthy adult fasts, when will glycogen stores run out?
12-18 hours.
437
What 4 enzymes are used in gluconeogenesis to bypass unidirectional glycolytic enzymes (pyruvate kinase, hexokinase, and phosphofructokinase)?
```
Phosphoenolpyruvate carboxylase (PEPCK) - bypasses pyruvate kinase.
Fructose 1,6-bisphosphonate - bypasses PFK
Glucose-6-phosphatase - bypasses hexokinase
Pyruvate carboxylase, bypasses pyruvate kinase along with PEPCK
```
438
What disease is indicated by the presence of short outer dextrin-like structures in the cytosol of hepatocytes?
Cori Disease - debranching enzyme deficiency so alpha-1,6-glucosidic branch points cannot be degraded, so small chain dextrin like material accumulates. This disease causes hypoglycemima, hypertriglyceridemia, ketoacidosis, and hepatomegaly.
439
How does Pompe Disease present?
Deficiency of lysosomal enzyme acid maltase, which breaks down small amts of glycogen. Causes cardiomegaly, hypotonia, and histo shows abnormal glycogen accumulation in lysosomes.
440
Hartnup disease causes a deficiency in what vitamin?
Niacin, B3, because it is synthesized from tryptophan, which cannot be intestinally or renally absorbed in patients with Hartnup disease.
441
What is Ehlers-Danlos syndrome?
Group of rare heredity disorders involving CT found in skin, tendons, ligaments, muscles, and vasculature. EDS results in hypermobile joints, fragile and hyperelastic skin, and easy bruising due to decreased tissue strength. It is caused by mutations affecting the collagen genes or collagen synthesis enzymes like lysyl-hydroxylase or procollagen peptidase.
442
What do telomerases add to the 3' end of DNA strands at the terminal end of chromosomes?
Telomere region has TTAGGG repeats. Stem cells have the longest telomere. Terminally differentiated cells have the shortest. Bloom syndrome is a premature aging disease with shortened telomeres.
443
The best indicator for severe MR with left ventricular volume overload is the presence of ____________.
an S3 gallop, which reflects an increased rate of LV filling due to large volume of regurgitant flow.
444
What are the firstline treatments for isolated systolic HTN?
Thiazide diuretics or dihydropyridine calcium antagonists in nondiabetics. ACEI or ARB for diabetics.
445
What is amlodipine?
Ca2+ channel blocker, used in HTN that can lead to flushing and peripheral edema.
446
What heart meds have a negative chronotropic effect, causing bradycardia and hypotension?
Beta blockers, Non-dihydropyridine ca2+ channel blockers, Cardiac glycosides, amiodarone and sotalol, cholinergic agonists.
447
What is the adaptation to pressure overload in the LV?
Concentric hypertrophy.
448
What is the adaptation to volume overload in the LV?
eccentric hypertrophy (chamber dilation)
449
The most common site of injury in motor vehicle accidents is the ______________.
Aortic isthmus, connection between the ascending and descending aorta distal to the branch point of the left subclavian artery.
450
What are the major SE of statins?
Hepatitis, myositis, and myalgias.
451
Niacin can be used in therapy for dyslipidemia. What are its effects and what is a common SE?
Niacin improves HL by decreasing hepatic synthesis of TGs and VLDL. It also increases HDL by 25-30%. Major toxicities include: gout, cutaneous vasodilation, hyperglycemia and acanthosis nigricans, hepatitis.
452
What dyslipidemia drugs should be used with caution in patients with preexisting gallbladder disease?
Fibrates (gemfibrozil) and bile acid-binding resins (cholestyramine, colestipol) because they both increase cholesterol excretion by the liver. Reduction of serum LDL occurs and an increased risk of gallstone formation.
453
What disease is associated with Libman-Sacks endocarditis?
SLE, in which there are small cardiac valvular vegetations, consisting of sterile, finely granular fibrinous eosinophilic material, that may result from IC deposition. Can result in regurgitation or stenosis of the valve.
454
What anti-arrhythmic drug class affects QRS complex duration?
Class 1C antiarrhythmics like Flecainide are potent Na channel blockers that exhibit use-dependence by prolonging the QRS duration especially at higher heart rates.
455
What structure in the brain most frequently undergoes necrosis in the setting of thiamine deficiency (Wernicke encephalopathy)?
Mammillary body --> involved in the Papez circuit, a neural pathway of the limbic system which controls emotion and memory.
456
Lesions of the splenium of the corpus callosum will lead to ________ without __________.
alexia, without agraphia
457
The vast majority of Down syndrome cases arise due to nondisjunction during _________
Maternal meiosis 1
458
Proton pump inhibitors like ___________ block the final common pathway of gastric acid secretion from parietal cells, which is stimulated by Ach, histamine, and gastrin.
Lansoprazole.
459
NADPH is created in the __________ pathway and is used in anabolic reactions, as an electron donor (including fatty acid and cholesterol synthesis).
HMP Shunt
460
What are the histologic findings of primary biliary cirrhosis?
destruction of intralobular bile ducts by granulomatous inflammation and heavy portal tract infiltrate of macrophages, lymphocytes, plasma cells, and eosinophils.
461
What drug can you use in a female patient experiencing idiopathic hirsutism (otherwise healthy)?
Treat hirsutism caused by androgen excess with spironolactone, because it blocks androgen receptors at the hair follicles, and decreases testosterone production. (Also is a K-sparing diuretic, and may cause gynecomastia in men.) Can also use anti-androgens, flutamide (androgen receptor antagonist) and finasteride (5alphareductase inhibitor)
462
What is Chronic Granulomatous Disease?
Deficient intracellular killing --> X-linked immunodeficiency resulting frmo the inability of phagocytes to synthesize NADPH oxidase, which is essential to the lysosomal oxidative burst. Will suffer from recurrent infections of catalase positive organisms like staph.
463
Somatic and parasympathetic fibers of the oculomotor nerve have separate blood supplies. Which does diabetic neuropathy affect?
Diabetic CN III neuropathy only causes ischemia to the somatic fibers, causing ptosis and a down and out gaze. It spare the PS fibers, so pupillary light reflex is intact.
464
What are the AAs with 3 titratable protons?
His, Arg, Lys, Asp, Glutamate, cysteine, and tyrosine
465
Noise-induced hearing loss results from trauma to the _________________.
Stereociliated hair cells of the organ of Corti. (Usually due to prolonged exposures to noises great than 85 dB, which can cause distortion or fracture of the stereocilia due to shearing forces against the tectorial membrane.
466
What causes rupture of the tympanic membrane and what kind of hearing loss ensues?
Due to infection, trauma, pressure changes when the Eustachian tube is blocked or sudden, very loud noises. Rupture causes conductive hearing loss.
467
Name the steps of the transduction of mechanical auditory forces in the Organ of Corti.
1. Sound reaches the middle ear by vibrating the tympanic membrane.
2. Vibration is transferred to oval window by the ossicles.
3. Vibration of the oval window causes vibration of the basilar membrane, which in turn causes bend of the hair cell cilia against the tectorial membrane.
4. Hair cell bending causes oscillating hyperpolarization and depolarization of the auditory nerve, thus creating nerve impulses from sound.
468
Both Chlamydia trachomatis and U. urealyticum cause nongonococcal urethritis, but do not have ________________ within the cell wall, meaning that Abx targeting the cell wall will not be effective.
peptidoglycans.
Cannot use CTX to treat, must use Abx like azithromycin.
469
During fetal development, what structures do the paramesonephric ducts fuse to form?
uterine tubes, uterus, cervix, and superior 1/3 of the vagina.
470
What does actinic keratosis look like on histology? What cancer does it predispose patients to?
Actinic keratosis - hyperkeratosis in the stratum corneum and parakeratosis (abnormal retention of cellular nuclei in the stratum corneum).
A small % of patients with AK will transform into invasive SCC.
471
What are the histological findings in psoriasis?
clubbed rete ridges, hyperkeratosis, PMNs in the stratum corneum and perivascular lymphocytic infiltrates.
472
What is clozapine used for? What should you monitor?
Used for the treatment of both negative and positive symptoms of schizophrenia and psychosis. Acts on the D4 receptors (unique!). Monitor blood cell counts because it may cause granulocytosis. It can also cause seizures.
473
The presence of fluid in the pulmonary interstitium decreases lung ___________.
Compliance.
Can happen in LV heart failure.
474
What do spike and dome appearance of the basement membrane of the glomerulus indicate?
Membranous glomerulopathy.
Lack of hypercellularity, uniform thickening of capillary wall
475
What should you administer if you have warfarin overdose?
Give fresh frozen plasma. Vitamin K can help over time as well, but not as acutely.
476
What should you administer in the event of heparin overdose or the need for heparin reversal?
Protamine, which binds and chemically inactivates heparin
477
At high doses, ___________ is a drug that can increase factor 8 activity in patients with Hemophilia A and vWF disease.
Desmopressin, usually used for diabetes insipidus because it is an ADH analog.
478
Aminocaproic acid is a ____________ agent. What is its mechanism of action?
Anti-fibrinolytic. Inhibits plasminogen activators and antiplasmin activity. Helps achieve hemostasis when fibrinolysis is the cause of bleeding.
479
What is lead-time bias?
An apparent increase in survival when patients are diagnosed earlier, but the overall prognosis is actually unchanged.
480
Virally-infected respiratory epithelia will secrete what kind of interferons in response to viruses?
IFN alpha and beta, which are type I interferons that act on neighboring cells via paracrine signaling to stimulate them to synthesize antiviral proteins (like RNase L and protein kinase R, an eIF-2 inhibitor) that degrade intracellular mRNA or halts protein synthesis.
481
Interferon gamma is secreted by what types of immune cells and what is its function?
Secreted by NK cells and T cells, to promote Th1 differentiation, MHC Class II expression, and activate macrophages, especially in response to antigens and mitogens.
482
What kind of reaction occurs in hyperacute rejection?
Ab-mediated rxn cause by preformed Ab within the recipient that are directed against donor Ag. Type II HS rxn.
Will see organ become cyanotic and mottled upon initial perfusion of organ, due to fibrinoid necrosis of the small vessels.
483
What drug is appropriate to use in an alcoholic patient with pneumonia/abcess formation in the lung?
Worry about S pneumo for general population, but for alcoholics, also worry about polymicrobial infections by oral anaerobes like Bacteroides, Prevotella, Fusobacterium, and Peptostreptococcus, admixed with aerobes. CLINDAMYCIN can cover strep pneumo AND oral anaerobes.
Similar to a macrolide, binds 50S ribosomal unit.
484
In Multiple Sclerosis patients, you may see a loss of what type of CNS cell?
Oligodendrocytes. Demyelinating disease.
485
In Multiple Sclerosis patients, you may detect what in the CSF?
Oligoclonal bands of IgG in the CSF.
486
Absence of an anal opening is most often associated with what kind of fistulas?
Imperforate anus is associated with urorectal, urovesical, or urovaginal fistulas and other urinary tract defects like hypospadias, epispadias, and bladder extrophy.
487
What are the VACTERL congenital anomalies?
Vertebral, Anal atresia, Cardiac anomalies, Tracheoesophageal fistula, Esophageal atresia, Renal, Limb.
Rare.
488
The upper anal canal is formed from the ________, and the lower part is derived from the invagination of the ____________. Junction between these canals is closed in embryonic life by an anal membrane located at the pectinate line.
Hindgut, surface ectoderm.
489
What catalyzes the conversion of cholesterol to pregnenolone?
Desmolase, which is a side chain cleavage enzyme. Absence leads to absence of steroid hormones, incompatible with life.
490
What are the symptoms associated with Friedreich ataxia?
- Progressive ataxia of all four limbs and degeneration of dorsal columns = loss of position and vibration sense.
- Hypertrophic cardiomyopathy (most common cause of death, in addition to bulbar dysfunction/inability to protect airway)
- Skeletal abnormalities
- Diabetes mellitus in 10%
491
What is the cause of Friedreich ataxia?
AR condition, mutates gene on Ch9 with increased trinucleotide repeats. (GAA)
492
In a midshaft injury of the humerus, what artery and nerve are you most worried about injuring?
Deep brachial artery and radial nerve, which innervates the forearm extensors, wrist extensors, digits' extrinsic extensors, and brachioradialis and supinator muscles. It also provides cutaneous sensory innervation to the dorsal hand, forearm and upperarm.
493
In a fracture to surgical neck of the humerus, what artery and nerve are you most worried about injuring?
Anterior circumflex artery and axillary nerve.
494
What kind of injuries to the upper extremity will cause injury to the brachial artery?
Supracondylar fractures.
495
What is Crigler-Najjar syndrome?
AR disease, with total genetic lack of UGT enzyme that conjugates bilirubin to its soluble and excretable form.
Causes deposits in various tissues, and can lead to kernicterus with jaundice and neuro impairment.
496
What is Dubin-Johnson syndrome?
AR disorder with the absence of biliary transport protein, MRP2, used in the hepatocellular excretion of bilirubin glucuronides into the bile canaliculi. Leads to darkly pigmented urine.
497
What are the organs of the foregut?
Esophagus to the second part of the duodenum, liver, gallbladder, and pancreas.
498
What is the main blood supply of the foregut?
Celiac trunk coming off the abdominal aorta.
499
What organs are part of the midgut? Blood supply?
Distal duodenum to first 2/3 of the transverse colon, supplied by SMA.
500
What organs are part of the hindgut? What is its main blood supply?
Last 1/3 of the transverse colon to upper part of the anal canal. Inferior mesenteric Artery.
501
Amatoxins from ingested mushrooms inhibit what type of synthesis?
mRNA synthesis. Binds to DNA-dep RNA pol II to halt mRNA synthesis.
502
1 gram of protein yields how many calories?
1 g protein or carb = 4 cal of energy
| 1 g fat = 9 cal of energy
503
What are the viruses that cause bronchitis and bronchiolitis?
Inf A, RSV, and coronavirus, which are all enveloped, ssRNA viruses.
504
What is the average total body water amount in L?
41 L on average.
Extracellular = 14 L (1/3 of total body water, with 3L plasma and rest in Interstitial Fluid)
505
Chlorhexidine destabilizes cell membrane walls and coagulates intracellular constituents. It is used in hospitals often for sterilization, except in the case of ______________.
Not for neurologic, otologic and ophthalmologic procedures due to neurotoxicity.
506
Lithium has been linked to _____________ in infants exposed in utero.
Ebstein's anomaly, in which there is apical displacement of the tricuspid valve leaflets, decreased RV volume, and atrialization of the RV.
507
Gestational diabetes is associated with what in the infant?
fetal macrosomia, caudal regression syndrome, hypoglycemia, hypocalcemia, and hypertrophic cardiomyopathy.
508
Stimulation of ______-adrenergic receptors inhibit insulin release, while stimulation of ______-adrenergic receptors stimulates insulin release.
alpha inhibits insulin release
| beta stimulates insulin release.
509
What kind of drug is pheenoxybenzamine?
Alpha blocker.
510
___________ are the earliest lesion of atherosclerosis, and are present in all individuals over the age of 10 years.
Fatty streaks, which contain intimal, lipid-filled foam cells and SMC with engulfed lipoproteins.
511
What is the primary somatosensory cortex called?
Brodmann's areas 3,1 & 2
512
What is the auditory cortex called and what lobe is it in?
Brodmann's areas 41, 42 in the temporal lobe.
513
Which Hepatitis Virus infection usually does not have a chronic state?
HAV, which is usually clinically silent (anicteric)
514
What are some organisms that can cause an infection with a small infectious dose?
Shigella, Campy, E histolyticia (as few as 1 organism!), and giardia (as few as 1!)
515
What is the believed mechanism of ACEI-induced angioedema?
ACE is normally supposed to break down bradykinin. ACEis block this process and cause an accumulation of Kinins, which are potent vasodilators that ultimately increase the vascular permeability --> angioedema.
516
Tubular obstruction with urate crystals occurs during ____________, which can present as acute renal failure after therapy for __________.
Tumor lysis syndrom, malignancy.
517
What is dexrazoxane?
Iron-chelating agent that can help prevent anthracyclin (ie doxorubicin) cardiotoxicity.
518
What can you use to prevent nephrotoxicity in patients taking platinum-containing chemotherapy?
Amifostine. Free radical scavenger.
519
What can you use to prevent tumor lysis syndrome?
Allopurinol
520
What will you find on EM in a patient with Post-Strep Glomerulonephritis?
Humps of IC deposits on epithelial side of the BM. On IF, you may see coarse granular deposits of IgG and C3, with characteristic starry sky appearance. On histology, will see hypercellularity. Labs will show hematuria, proteinuria, and urine RBC casts with hypercellular glomeruli.
521
Prader Willi and Angelman syndromes both carry deletions in the same gene (Ch 15q), but have very different phe because of genomic imprinting. What is the pattern of inheritance of each?
Prader-Willi: If the deletion is from the father; phe: insatiable hunger/thirst and emotional instability.
Angelman: If the deletion is from the mother; phe: happy puppet = jerky movements, and pathologically happy disposition.
522
What part of the aorta's blood vessel wall is first torn in an aortic dissection?
Intima.
523
What is (adult) wet beriberi vs. dry beriberi?
Caused by thiamine B1 deficiency.
Dry = Symmetrical peripheral neuropathy with sensory and motor impairments, especially distally.
Wet = Same neuropathies as Dry but including cardiac involvement (cardiomegaly, myopathy, CHF, peripheral edema, and tachycardia)
524
Pyridoxine deficiency is characterized by what symptoms? What drug is chemically similar and can cause synthesis of defective end products?
Vitamin B6 deficiency is characterized by cheilosis, glossitis, dermatitis, and peripheral neuropathy. Isoniazid is structurally similar to B6 and can compete with it in the synthesis of NTs (like GABA) and can increase the renal excretion of Vitamin B6.
525
In Lesch-Nyhan syndrome, what enzyme will have increased activity?
Phosphoribosyl pyrophosphate amidotransferase. PRPP is a substrate of the deficient HGPRT enzyme, which now accumulates and is shunted into de novo purine synthesis pathways.
526
When patients receive phenytoin, a known SE may be gingival hyperplasia. What is the mechanism of formation?
PDGF is overexpressed and stimulate gingival macrophages, which in turn stimulate the proliferation of gingival cells and alveolar bone.
527
At Functional Residual Capacity, the airway pressure = _______ cm H2O, while the intrapleural pressure normally = _________ cm H2O.
Airway P = 0 cm H2O
| IPP = -5 cm H2O
528
What drug should you give to pregnant women who are HIV+?
Give a viral enzyme inhibitor, like zidovudine, which is a retroviral reverse transcriptase inhibitor. Should initiate at 14 weeks gestation and continue oral administration in the infant for 6 weeks postpartum. Can reduce the risk of perinatal transmission by about 2/3 in HIV+ women who were not previously treated with ARVs.
529
___________ infection should be considered in unvaccinated patients with membranous pharyngitis or obstructive laryngotracheitis who have recently emigrated.
Corynebacterium diptheria.
Its exotoxin inhibits protein synthesis through ADP-ribosylation of elongation factor 2 --> cell death and necrosis.
530
How do you calculate the Absolute Risk Reduction?
ARR = Event Rate (control) - Event Rate (treatment group)
531
What is the difference between minute and alveolar ventilation?
Alveolar ventilation does not include dead space volume while the minute ventilation does.
Alveolar ventilation = (Tidal volume - Dead space)*breaths/minute
Minute ventilation = (Tidal Volume)*breaths/minute
532
If serum FTA-ABS tests are positive, and the patient has an aortic regurgiation murmur and mediastinal widening, what is your diagnosis?
Tertiary syphilis, which starts with vasa vasorum endarteritis and obliteration, resulting in inflammation, ischemia, and weakening of the adventitia. Aneurysmal dilatation results.
533
The risk of statin-induced myopathies is increased when statins are taken concurrently with _____________.
Fibrates, which increase the concentration of most statins.
i.e. Gemfibrozil and fenofibrate
534
What disease has the following findings? Gowers sign and calf enlargement in a male child (3-6 yrs)
Duchenne muscular dystrophy, X-linked recessive deletion of gene for dystrophin protein (a structural component of skeletal muscle fibers).
535
Adenosine and dypyridamole are selective _____________ of the coronary vessels.
Vasodilators.
Do not give in cases of myocardial ischemia because it will vasodilate arterioles in nonischemic regions of the heart, thereby decreasing perfusion to the already ischemic regions. This is called the "coronary steal."
536
What is pentazocrine and why should you not use it in patients who are on morphine for pain?
It is a partial mu agonist and antagonist that can precipitate withdrawal in patients who are taking and are dependent on morphine.
537
Name some selective mu-agonists.
Morphine, methadone, codeine, meperidine, fentanyl, levorphanol, oxymorphone.
538
How do cholinomimetic help treat glaucoma?
They promote ciliary muscle contraction, causing the lens to be more convex.
539
How do cholinergic agonists like pilocarpine and carbachol help treat glaucoma?
Cause miosis by promiting contraction of the iris sphincter, which causes the anterior chamber to become wider --> makes the trabecular meshwork more accessible to outflow of aqueous humor.
540
How do beta blockers help treat glaucoma? What other drugs have a similar effect?
Diminish the secretion of aqueous humor by ciliary epithelium. Acetazolamide are Carbonic anhydrase inhibitors that have a similar ultimate effect of decreasing humor secretion.
541
What does the glossopharyngeal nerve innervate?
Somatic motor: stylopharyngeus
PS motor: Parotid gland secretion (Inferior salivatory nucleus -> CN IX -> otic ganglion -> travels CN V -> parotid gland)
General Sensory: inner surface of the tympanic membrane, Eustachian tube, posterior 1/3 tongue, tonsillar region, upper pharynx (afferent gag reflex), carotid body and carotid sinus.
542
What are atropine's effects?
CNS effects (may progress to coma or resp failure), dry/flushed skin, hyperthermia, mydriasis, cycloplegia, bronchodilation, tachycardia, constipation, and urinary retention.
543
What should you administer in the case of an atropine overdose?
Physostigmine, an anticholinesterase inhibitor (crosses BBB into CNS too), and increases Ach availability.
Neostigmine and Edrophonium are also anticholinesterase inhibitors but do not cross the BBB and so is not as effective. .
544
Without T1 helper cells, ________ synthesis required for activation of macrophages does not occur (necessary for delayed hypersensitivity rxns and cytotoxicity against intracellular organisms).
Interferon gamma.
545
A deficiency in ______ receptors would make helper T cells unable to differentiate into the Th1 sub population.
IL-12 into Th1
| IL-4 to differentiate into Th2
546
What type of helper T cells are involved in cell-mediated immunity? In humoral immunity?
```
Cell-mediated = Th1
Humoral = Th2
```
547
Adding carbidopa to a Parkinsons patient's medication will alleviate what side effects?
Reduces most of the peripheral side effects of levo-dopa. Arrhythmias, postural hypertension, nausea and vomiting.
However, it doesn't help with behavioral changes.
548
What causes a systolic ejection murmur (SEM) that is accentuated by standing?
Hypertrophic obstructive cardiomyopathy. When standing, venous return is decreased -> LV EDV and stroke volume decreased -> accentuates the murmur.
(Aortic stenosis is the more common cause of SEM.)
549
When you have LAD occlusion (alone), what is the preferred vessel for bypass grafting? When you have occlusions in multiple coronary arteries, what is the preferred vessel for grafting?
LAD alone: left internal mammary artery
| Multiple: Saphenous vein grafts.
550
Nitrates are used for stable angina because they have anti-ischemic properties. However, they can cause a paradoxical increase in myocardial oxygen demand. Explain this phenomenon.
Nitrates are vasodilators that can cause a decrease in blood pressure. This perceived hypotension is sensed and catecholamines are released in effect, causing a reflex tachycardia that increasese oxygen demand. Use with beta blockers to slow tachycardia.
551
Extreme myofiber disarray and interstitial fibrosis on cardiac histo strongly suggests ______________.
Hypertrophic cardiomyopathy, which is usually caused by mutations in genes for cardiac sarcomere proteins (beta-myosin heavy chain.)
552
________________ are found in histology of patients with rabies infection.
Negri bodies: round, eosinophilic inclusions seen in the cytosol of pyramidal neurons of the hippocampus and cerebellar purkinje fibers.
553
The ____________ nodes drain nearly all cutaneous lymph from the umbilicus to the feet, including the external genitalia and anus (up to the dentate line).
Superficial inguinal lymph nodes (which drain into the deep inguinal, then into common iliac and the para aortic nodes)
554
Lymphatic drainage of the testis go to the ________ nodes.
Para-aortic nodes because of the testes' intra-abdominal origin.
555
The foregut's blood supply is what?
Celiac trunk
| esophagus, stomach, liver, gallbladder, pancreas, and upper duodenum
556
What is the midgut's blood supply?
Superior Mesenteric Artery
Lower duodenum, small intestine, ascending colon, and proximal 2/3 of the transverse colon.
557
What is the hindgut's blood supply?
Inferior Mesenteric Artery
Distal third of the transverse colon, descending and sigmoid colon.
558
Valproate increases the risk of fetal ___________ defects, because it inhibits intestinal folate absorption.
neural tube
559
What kind of immune cells are responsible for destroying cells with decreased or absent MHC class I proteins?
NK cells. Usually these changes occur in virus-infected cells and tumor cells.
560
What cytokines activated NK cells? What CD-markers are expressed on NK cells?
IFN-gamma, and IL-12.
Express CD16, CD56
561
In breast milk, all vitamins are present in adequate amounts except for two vitamins. Please name them.
Vitamin D and Vitamin K.
Usually sunlight is enough supplementation for a light-skinned infant, but you should also tell parents to give Vitamin D supplements for dark-skinned babies who are exclusively breast fed.
562
Patients undergoing total gastrectomy should receive lifelong __________ supplementation.
Vitamin B12 supplements because the parietal cells of the stomach secrete Intrinsic Factor, which is necessary for B12 absorption.
563
The __________ nerve is commonly blocked during childbirth (especially if the woman has progressed too far in labor to receive an epidural). This nerve is derived from nerve roots _____-____, and provide sensory innervation to the perineum and the genitals.
Pudendal nerve, S2-S4.
564
The bell of the stethoscope is best for hearing ________-frequency heart sounds, like _______.
low, like S3
A maneuver to make S3 louder is to have the patient lie in the left lateral decubitus position and exhale all air out. This decreases volume of lungs and brings the heart closer to the chest wall.
565
The diaphragm of the stethoscope is best for hearing ________-frequency heart sounds, like _______.
high, like S1 or S2.
566
Of the five GLUT transporters, only _______ is responsive to insulin action.
Glut-4, which is on muscle cells and adipocytes.
567
What does an S4 heart sound indicate?
Extra low frequency heart sound right before the S1 (at the end of diastole). It is often heard in adults due to age-related decrease in LV compliance, or in pathologically stiff LVs (i.e. Restrictive cardiomyopathy or LV hypertrophy). A loud one is always pathologic in the young. Thought to be atrial contraction and resultant onrush of blood striking the stiff LV.
568
If you see a patient with a long hx of "painful crises," with signs of osteomyelitis, think ___________ instead of _____________.
Think salmonella, rather than staph aureus as your first choice in diff dx for osteomyelitis etiology in patients with Sickle Cell disease.
569
What is Cheyne-Stokes respiration?
Cyclic breathing in which apnea (suspension of external breathing) is followed by gradually increasing tidal volumes, and then gradually decreasing tidal volumes.
Seen in advanced heart disease (CHF) or neurological disease (stroke, brain tumors, TBI),
570
In men, FSH is responsible for what two actions?
1. Spermatogenesis
| 2. Inhibin B production (which induces negative feedback on FSH and LH).
571
Vasospasm can occur secondary to a subarachnoid hemorrhage (if the patient survives). What should you administer to prevent vasospasms of CNS arteries?
Nimodipine, a selective Ca channel blocker.
572
____________ is an antipsychotic that can cause retinal deposits that resemble retinitis pigmentosa, while _____________ is one that can cause corneal deposits.
Thioridazine - retinal
| Chlorpromazine - corneal
573
What is phenytoin's MOA for its use in seizure prophylaxis?
Inhibits neuronal high-frequency firing by reducing the ability of Na channels to recover from inactivation.
574
What enzymes are mutated in Orotic Aciduria? What supplements can you give patients to help with symptoms?
Disorder of pyrimidine metabolism, with defective orotate phosphoribosyl transferase and OMP decarboxylase (both depend on one single peptide for activity). Give uridine supplementation, which is converted to UMP (an inhibitor of the initial step of pyrimidine synthesis, CPS-II enzyme, and stops orotic acid buildup.)
575
What are niacin's main side effects?
Cutaneous mostly -- flushing, heat, itching -- possibly mediated by prostaglandins because pretreatment with aspirin helps diminish these effects.
Also, may serum uric acid, increase insulin insensitivity (acanthosis nigricans, increase diabetic drugs when taking niacin too), and vasodilator (be careful using with anti-hypertensive therapy because hypotension may occur).
576
What protein is released in Vancomycin-induced Red Man Syndrome?
Histamine
577
What is the pathogenesis of migraine headaches?
Pain due to activation of trigeminal afferents that innervate the meninges. This causes a release of vasoactive neuropeptides like substance P and CGRP. Nociceptive pathways also become more sensitive to both painful and nonpainful stimuli.
578
What do you give to abort migraine HA?
Triptans like sumatriptan are 5HT agonists that counter migraines by inhibiting release of vasoactive peptides and blocking pain pathways in the brain stem.
579
_________ is a polypeptide precursor that goes on to produce beta-endorphins, ACTH, and MSH.
POMC
580
What is cauda equina syndrome?
Results from a massive rupture of an IV disk or a space-occupying lesion in the lower vertebrae that can cause compression of 2 or more of the 18 spinal nerve roots of the cauda equina.
Symptoms: Low back pain, saddle anesthesia, loss of anocutaneous reflex, bowel/bladder dysfunction, loss of ankle-jerk reflex with plantarflexion weakness in feet.
581
What kind of HS rxn is involved in anaphylaxis?
Type I HS rxn, involving IgE -- requires cross-linking of two IgE molecules on the surface of the cell by one molecule of allergen to cause degranulation of substances that lead to hemodynamic and respiratory instability of anaphylaxis.
582
In restrictive lung diseases, high expiratory flow rates are achieved in spite of low lung volumes due to _____________ and _____________.
Increased elastic recoil pressure and increased radial traction on the conducting airways by the fibrotic pulmonary interstitium.
583
Restrictive lung disease cause increased or decreased lung compliance?
Decreased
584
Squatting improves cyanosis in Tetralogy of Fallot patients because the increase in systemic pressure reduces the amount of _____________ through the VSD.
reduces right to left shunting, aka reduces the amount of blood that bypasses the lungs.
585
What chromosome is deleted in Von Hippel Lindau disease?
Autosomal dominant Ch 3p deletion, which causes cerebellar hemangioblastoma, clear cell renal carcinoma, and pheochromocytomas.
586
Where is the Rb anti-oncogene located?
Ch 13.
587
What is Zileuton and what is it used for?
Selective inhibitor of lipoxygenase pathway that leads to decreased formation of leukotrienes. Used for treatment of asthma.
588
What causes bronchiolitis obliterans syndrome?
Chronic rejection of a lung transplant, which can occur months to years after transplant. The immune rxn affects the small airways, causing dyspnea and wheezing.
(Hyperacute, due to preformed Ab against blood Ag)
(Acute, due to cell-mediate response causing vascular damage)
589
In Cystic Fibrosis patients, CFTR mutations impair the secretion of Chloride in most exocrine glands except for ______ glands.
Sweat glands, in which CFTR mutation impairs ABSORPTION of NaCl.
590
In Cystic Fibrosis, CFTR protein abnormalities can be detected in patients who do not have a positive sweat test by what other test?
Transepithelial potential difference. CFTR abnormalities reduce luminal chloride secretion AND increases Na and net water absorption. This results in dehydrated mucus and a negative transepithelial potential difference. This occurs in most exocrine glands, except sweat glands.
591
Berry aneurysms are particularly prone to rupture when associated with congenital cardiac anomaly like _________________.
Coarctation of the aorta. (causes HTN in branches of the aortic arch feeding the brain blood supply.)
592
The most important mediator of sepsis is _____.
TNF-alpha, an acute phase cytokine produced by activated macrophages.
IL1 and IL6 are imp too.
593
____________ is a cofactor and CO2 carrier on the surface of carboxylase enzymes that is necessary for numerous conversions, including pyruvate to oxaloacetate.
Biotin.
Can get deficiency if you have a poor diet or you have excessive ingestion of avidin (egg whites) which binds to biotin.
594
Name the series of gene mutations that are involved in the "adenoma-to-carcinoma sequence" of colon cancer.
1. Progression of small polyp -- APC tumor suppressor gene out.
2. Increasing polyp size -- K-ras protooncogene facilitates.
3. Malignant transformation -- p53 and DCC tumor suppressor genes out.
595
Duodenal ulcer pain is relieved by _________ of food.
Ingestion
596
What is a rare congenital vascular disorder characterized by facial port-wine stain and leptomeningeal capillary-venous malformations?
Sturge-Weber syndrome
597
What autosomal dominant condition is associated with renal angiomyolipomas?
Tuberous sclerosis
598
What is Oseltamivir used for?
Treatment of Inf A or B infections. It is a neuraminidase inhibitor that impairs the release of newly formed virions from infected host cells and impairs the viral penetration of mucous secretions that overlie the respiratory epithelium.
599
What do you give to patients with atropine overdose?
Atropine overdose causes a blockade of visceral muscarinic receptors. Give Physostigmine, a cholinesterase inhibitor that will suppress Ach degradation.
600
What structures are compressed due to an uncal herniation?
Herniation of the medial temporal lobe through the gap between the crus cerebri and the tentorium =
1. Ipsi CN III compression
2. Ipsi Post Cerebral Artery compression
3. Compression of contralateral cerebral peduncle
4. Brainstem hemorrhage due to stretching and rupture of basilar artery.
601
Individuals with albinism have melanocytes that do not produce melanin because of absent or defective _______ enzyme.
Tyrosinase
602
All forms of contact dermatitis result from this type of hypersensitivity rxn:
Type IV delayed-type HS rxn.
603
Damage to what nerve results in wrist drop, or the inability to extend the wrist and fingers.
Radial Nerve, C5-C8,T1
604
Damage to the _________ nerve will limit hand flexion and opposition of the thumb.
Median
605
Damage to the recurrent branch of the median nerve of the deep branch of the ulnar nerve would limit ___________.
Finger abduction.
606
Severe intellectual disability, seizure hx, and abnormal pallor of catecholaminergic brain nuclei (locus ceruleus, substantia nigra, vagal nucleus) indicate what condition in a child?
PKU, phenylalanine hydroxylase deficiency.
607
What drug can be used for nicotine cessation and reduces cravings/withdrawal while also decreasing pleasurable effects of cigarettes?
Varenicline - partial Alpha4-beta2 nicotinic Ach receptor agonist.
608
What is Reye Syndrome?
Microvesicular fatty change of the liver in children after salicylate treatment.
609
_____________ is the primary histo finding in patients with eczematous dermatitis.
Spongiosis.
610
What organism can cause salmon-colored rose spots on abdomen, hepatosplenomegaly, and lead to hemorrhagic diarrhea/sepsis??
Salmonella typhi.
611
What are the neonatal complications of gestational diabetes?
```
Premature delivery
Fetal macrosomia
Neural tube defects
Hypoglycemia
Hypocalcemia
Polycythemia
Resp distress
Transient hypertrophic cardiomyopathy
```
612
What antiviral is more effective than acyclovir in the treatment of CMV retinitis?
Gancyclovir, a guanine nucleoside analogue.
613
What histo findings are characteristic of acute viral hepatitis?
1. Hepatocyte swelling
2. mononuclear cell infiltrates
3. Eosinophilic apoptotic hepatocytes (Councilman bodies).
614
___________ necrosis is a histo pattern of injury related to vasculitides and seen in the walls of vessels affected by immune complex deposition.
Fibrinoid
615
What are the afferent and efferent limbs of the gag reflex?
Afferent: Glossopharyngeal (IX)
Efferent: Vagus (X)
616
_____________ (Vitamin ____) is an essential cofactor for the transamination and decarboxylation of amino acids, for gluconeogenesis and for other processes.
Pyridoxine, B6
617
What is a paraneoplastic syndrome?
Paraneoplastic syndromes are often associated with systemic cancer and are usually caused by substances produced against the tumor cells. These substances cross-react with the body's own healthy organs and tissues.
```
ie Anti-Yo (ovary and breast), anti-P/Q (lung), anti-Hu (lung) which cross react with neurons and cause cerebellar degeneration.
ie ACTH secretion (causing Cushing syndrome)
ie PTHrP (Hypercalcemia)
```
618
What are the CYP inducers?
```
Carbamazepine
Phenobarbital
Phenytoin
Rifampin
Griseofulvin
```
619
What are the CYP 450 Inhibitors?
```
Cimetidine
Ciprofloxacin
Erythromycin
Azole antifungals
Grapefruit juice
Isoniazid
Ritonavir (protease inhibitors
```
620
Mutations of the fibrillin-1 gene predisposes to ___________, a frequent histo finding associated with aortic aneurysms and dissections.
myxomatous changes, cystic medial degeneration.
621
What lung condition causes exertional dyspnea and produces eggshell calcifications of hilar nodes and birefringent particles surrounded by fibrous tissue?
Silicosis.
622
What virus causes croup? (URI, hoarse cough, stridor)
Parainfluenza virus, a paramyxovirus.
623
What is primary biliary cirrhosis?
Autoimmune destruction of intrahepatic bile ducts and cholestasis.
Pruritus, fatiue, hepatosplenomegaly, xanthomas. Elevated alk phos and cholesterol. Can progress to jaundice, steatorrhea, portal HTN, and osteopenia. Associated with other autoimmune disease.
624
Lithium is handled primarily by the kidneys similar to what other substance?
Na, with similar filtration and reabsorption % in the PCT.
Giving thiazides, ACEis and NSAIDs (renal injury) can impair lithium clearance.
625
Cyproheptadine is used in the treatment of ______ overdose with symptoms of ______ syndrome.
MAO inhibitors + SSRI, serotonin syndrome.
626
___________ is often prescribed to prevent recurrent calcium stones in adults when diet is unsuccessful
Potassium citrate, which binds to free ionized Ca.
627
What heart sound or murmur is best heard when the patient sits up and leans forward?
Aortic regurgitation
628
What drugs do you use to treat the ocular issues caused by Graves' disease?
High dose steroids to decrease the severity of inflammation and decrease extraocular volume. Conventional anti-Graves treatments like PTU or methimazole are not effective against exopthalmos.
629
In general, most enzyme deficiency conditions follow and ______ inheritance pattern, while diseases due to defective noncatalytic proteins tend to follow a _______ pattern.
Enzyme deficiency = AR
| Defective, noncatalytic proteins = AD
630
What are 3 different drugs that target dihydrofolate reductase in the folate acid synthesis pathway?
Trimethoprim, MTX, and pyrimethamine (antimalarial/antitoxoplasmosis)
631
What is Gilbert Syndrome?
Familiar disorder of bilirubin glucuronidation -> reduced UDP-glucuronyl transferase production. Normal LFTs but abnormal unconj bilirubin.
632
What is Dubin-Johnson syndrome?
Rare syndrome, with conjugated chronic hyperbilirubinemia, not associated with hemolysis. Direct bili is at least 50% with an otherwise normal liver function profile.
633
What enzyme is defective in Maple Syrup Urine Disease?
Alpha-keto acid dehydrogenase, which degrades branched chain AAs (leucine, isoleucine, valine) beyond their deaminated alpha keto acid state.
634
Which brainstem nuclei contains serotonin-releasing neurons?
The Raphe nuclei
635
What kind of neurons does the nucleus cerulous house?
NE-secreting neurons which activate fight or flight response
Located in the dorsal pons.
636
What kind of neurons does the nucleus basalis of Meynert house?
Cell bodies of cholinergic neurons. (In Alzheimers, these secrete inadequate amounts of Ach)
637
What % of patients will fall outside of 1 SD of the mean in a normal curve? Of 2 SD of the mean? Of 3 SD?
In a normal curve, 68% lie within 1 SD, thus 32% will be outside -- with 16% above and 16% below 1 SD of the mean.
95% are within 2 SD, with 5% outside -- 2.5% above, and 2.5% below 2 SDs.
99.7% are within 3 SD, with 0.3% outside
638
What are the symptoms associated with carcinoid symptoms and where do these lesions usually occur?
If the carcinoid tumor has metastasized to an extra-intestinal region, then it can release 5-HT to the systemic circulation and cause wheezing, diarrhea, syncope, and facial flushing. If it is only in the GI tract, the tumor will not have symptoms. It usually occurs in the APPENDIX.
639
What is octreotide used for?
Synthetic analog of somatostatin, acts on receptors to inhibit secretion of many hormones --> treats Serotonin syndrome
Also used to inhibit secretory diarrhea in VIPomas.
640
What is the classic tried of Ménière's disease?
Tinnitus, vertigo, and sensorineural hearing loss (Rinne test shows normal hearing conduction and Weber test reveals lateralizations to the healthy side).
Ménière's disease is characterized by increased volume of endolymph, which causes damage to the vestibular and cochlear components of inner ear.
641
What is etanercept?
TNF-alpha inhibitor, soluble receptor decoy protein that links soluble TNF-alpha receptors to the Fc of human IgG1 to reduce the biological activity of TNF-alpha.
Used in the tx of Rheum Arthritis
642
Previously well-nourished individuals can take up to several years to develop a complete dietary deprivation of this vitamin:
Cobalamin, B12
643
What are the classic symptoms of patients with multiple myeloma?
Easy fatigability, constipation, back pain, and azotemia.
On histo, see large eosinophilic casts composed of Bence-Jones proteins in tubular lumens.
644
What are the different cancers that cause psammoma bodies?
Thyroid - papillary carcinoma
Pleura - mesothelioma
Brain - meningioma
Endometrium - papillary serous carcinoma
645
What is Henoch-Schonlein purpura?
Most common small vessel vasculitis in children. Thought that prior URI stimulates IgA production -> IgA-containing immune complexes deposit on vessels.
IgA-mediated leukocytoclastic hypersensitivity vasculitis.
646
What is the triad of findinds in patients with Granulomatosis with polyangiitis (Wegeners)?
1. Pulmonary - cough/hemoptysis
2. URI - sinusitis or mucosal ulcerations
3. Renal - RPGN (crescentic).
647
Why does squatting help diminish the murmurs in MVP and HCM?
Squatting causes increased venous return (preload) and increased peripheral vascular resistance (afterload). Under these conditions, the LV volume is enlarged and brings valve leaflets into a normal arrangement, facilitating proper closure.
648
What is dobutamine?
beta-adrenergic agonist, with more activity as follows: beta1>beta2>alpha1
Used for acute heart failure with decreased contractility.
649
Mitral valve prolapse is the most common cardiac abnormality that predisposes 15- to 60- year olds to _______________.
native valve bacterial endocarditis (NVBE)
650
What is supine hypotension syndrome?
Pregnant women >20 weeks can get compression of the IVC by the uterus in the supine position, which can reduce venous return and CO of the heart.
651
What are the symptoms of Kawasaki disease and what is the major complication?
Vasculitis of medium-sized vessels in young children. Most feared complication is coronary artery aneurysm.
1. Persistent fever
2. Bilateral conjunctivitis
3. Lymphadenopathy
4. Cutaneous involvement (strawberry tongue, edema/erythema of palms and soles, desquamation of fingertips, generalized rash)
652
Normal levels of this vitamin and its metabolite are needed to maintain differentiation of specialized epithelia, including mucus-secreting columnar epithelium.
Vitamin A, retinoic acid
Avitaminosis A defiency can lead to squamous metaplasia of epithelial cells.
653
Killed vaccines amplify what immune response to the pathogen?
Humoral immunity, which produces neutralizing Abs that impair viral attachment to host cells.
Killed vaccines do not infect host cells and therefore do not enter the MHC class 1 Ag-processing pathway, which is required for a cell-mediated immune response.
654
DNA pol ____ has 5' to 3' exonuclease activity, while DNA pol _____ has 3' to 5' exonuclease activity.
- DNA pol I has 5' to 3' exonuclease activity, to cut RNA primer and remove damaged DNA. Also has 5' to 3' polymerase activity and 3' to 5' exonuclease activities.
- DNA pol III has 3' to 5' exonuclease activity, which is for proofreading.
655
What sleep medication should you give patients who work in mission-critical positions, and want minimized side effects of the sleep medication?
Short-acting Benzodiazepines like triazolam or alprazolam are best for this type of patient.
Don't use because will be associated with excessive daytime fatigue:
Intermediate - lorazepam
Long-acting - Diazepam, flurazepam, chlordiazepoxide (better for withdrawal)
656
What are the symptoms of early hydrocephalus?
- Macrocephaly (>2 SD above mean for gender/age)
- irritability
- poor feeding
- muscle hypertonicity and hyperreflexia due to UMN damage from stretching of the periventricular pyramidal tracts
657
What viruses are the most common cause of aseptic meningitis in children?
Enteroviruses, like coxsackie, echovirus, poliovirus, and enterovirus.
658
What viruses are the most common causes of the common cold?
Corona, adeno, and rhino
659
In infertility treatment, patients are given several doses of menotropin followed by a single injection of hCG. What is the mechanism of each step?
Menotropin acts like FSH and leads to the formation of the dominant ovarian follicle.
Large doses of hCG can then induce ovulation by simulating the LH surge.
660
What is affected in Malignant Hyperthermia? What is the cause of it?
RyR receptors on the sarcoplasmic reticulum release a ton of Ca after exposure to inhalation anesthetics and succinylcholine (muscle relaxant). This causes muscle rigidity, increased ATP utilization -> heat, rhabdomyolysis.
Treat with dantrolene, which blocks RyR.
661
What tests are involved in the triple test and when is it done?
Triple tests are done to determine alpha-fetoprotein, hCG and estriol levels in pregnant women at 16-18 weeks gestation.
High AFP - often dating error, but can indicate neural tube defects, anterior abdomen defects, and multiple gestation.
Low AFP - Down syndrome
Low Estriol - placental insufficiency
High hCG - multiple gestation, hydatidiform mole, and choriocarcinoma.
662
What is the preferred treatment for Giardia Lambia infection?
Metronidazole.
663
What is the presentation of Klinefelter syndrome?
Infertility, small firm testes, long limbs, bilateral gynecomastia.
47XXY, nondisjunction in meiosis.
- Destroys & hyalinizes seminiferous tubules -> low serum inhibin and small firm testes
- Destroyed Leydig cells -> low testosterone -> erectile dysfunction
- Low testosterone & inhibin -> increased LH and FSH via loss of feedback inhibition
- Hypogonadism -> delayed closure of epiphyseal plates -> long limbs.
664
What is the most useful initial test for serotonin syndrome caused by metastatic carcinoid?
Serum 5HT metabolite, 5-HIAA in a 24-hour urine sample
665
Chronic granulomatous disease is most frequently an X-linked disorder, resulting from a deficiency in the enzyme, __________.
NADPH oxidase, which is necessary for forming the oxidative burst to kill organisms in phagolysosomes. Catalase-positive organisms are ineffectively killed.
666
What is a comedocarcinoma?
A type of ductal carcinoma in situ, of the breast
| Identified as solid sheets of pleomorphic, high-grade cells with central necrosis
667
What is an acute hemolytic transfusion rxn?
Patients after blood transfusion may experience chills, SOB, fever, hypotension, DIC, renal failure, and dark urine. Due to ABO incompatibility between donor and recipient. Will produce a type II hypersensitivity rxn, in which complement is activated (MAC complex -> RBC lysis, Anaphylotoxins -> vasodilation)
668
What are the clinical findings associated with leukocyte adhesion deficiency?
late separation fo the umbilical cord, poor wound healing, recurrent skin infections without pus formation, gingivitis.
669
Female patient (middle-aged) with musculoskeletal pain, insomnia and emotional disturbances probably has what?
Fibromyalgia
670
What causes shaken baby syndrome?
Shaking leads to movement of immature brain in relation to the skull, tearing of the bridging veing, resulting in subdural hematoma. Can cause retinal hemorrhages due to rupture of congested retinal veins.
671
What should you suspect if you see spoon nails?
Iron-deficiency anemia
672
What is the Psoas sign?
Inflammation of the psoas muscle causes pain when the hip is extended. Can be caused by abcess, as a result of hematogenous spread (ie insulin shots)
673
What nerve is normally injured during anterior shoulder dislocations?
Axillary nerve
674
What nerve would be injured during a spiral humeral midshaft fracture?
Radial nerve
675
What is Aldosterone escape?
Aldosterone escape is when increased Na and Cl absorption due to excessive mineralocorticoid secretion leads to intravascular hypervolemia. This hypervolemia causes ANP release, which leads to diuresis and eventual Na loss. You don't see hypernatremia. You only see hypokalemia, metabolic alkalosis, and very mild edema (if any at all).
676
What is Waterhouse-Friderichsen syndrome?
Shock in combination with hyponatremia, hypoglycemia, and hyperkalemia suggests adrenal crisis. Paired with meningitis, you should think about Neisseria and meningococcal meningitis-induced adrenal hemorrhage, which is called Waterhouse-Friderichsen syndrome.
677
How is Churg-Strauss syndrome defined, becides by anti-MPO/p-ANCA?
Combination of adult-onset asthma, eosinophilia, history of allergy, mono or polyneuropathy, migratory pulmonary infiltrates, and paranasal sinus abnormalities.
