Boards Flashcards

0
Q

Bacteria with unusual cell walls

A

Mycoplasma - Sterols & no cell wall

Mycobacteria - Mycolic acid & high lipid content

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1
Q

Polypeptide capsule

A

B. anthracis

All other are polysaccharide

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2
Q

What are the spirochetes?

A

Borrelia
Leptospira
Treponema

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3
Q

Giemsa stained bugs

A

“Certain Bugs Really Try my Patience”

Chlamydia
Borrelia
Rickettsiae
Trypanosomes
Plasmodium
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4
Q

PAS Stain bugs

A

Stains glycogen

Tropheryma whipplei (whipple’s disease)

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5
Q

Zeihl-Neelsen bugs

A

Acid-fast organisms

Nocardia
Mycobacterium

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6
Q

India ink bugs

A

Cryptococcus neoformans

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7
Q

Silver stain bugs

A

Fungi
Legionella
H. pylori

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8
Q

H. flu culture requirements

A

Chocolate agar (Factor V & X)

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9
Q

Neisseria (both) culture requirements

A

Thayer-Martin (VPN: Vanc, Polymixin, Nystatin) media

To inhibit all others

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10
Q

Bordetella pertussis culture requirements

A

Bordet-Gengou (potato) agar

Bordet for Bordetella

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11
Q

Corynebacterium diphtheriae culture requirements

A

Tellurite Plate, Loffler’s media

Teller she’s right, laugh at her after

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12
Q

MTB culture requirements

A

Lowenstein-Jensen agar

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13
Q

Mycoplasma pneumoniae culture requirements

A

Eaton’s agar

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14
Q

Lactose+ enterics culture requirements

A

MacConkey’s agar

shows pink colonies

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15
Q

Legionella culture requirements

A

Charcoal yeast extract with cysteine & iron

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16
Q

Fungi culture requirements

A

Sabouraud’s Agar

“Sab’s a fun-guy”

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17
Q

Obligate aerobes

A

“Nagging Pests Must Breathe”

Nocardia
Pseudomonas
MTB
Bacillus

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18
Q

Obligate anaerobes

A

Clostridium
Bacteroides

Generally foul-smelling, produce gas in tissue, and resistant to amin(O2)glycosides

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19
Q

Obligate intracellular bacteria

A

Stay inside when it’s Really Cold:
Rickettsia
Chlamydia

They can’t make their own ATP.

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20
Q

Facultative intracellular bacteria

A

“Some Nasty Bugs May Live FacultativeLY”

Salmonella
Neisseria
Brucella
Mycobacterium
Listeria
Francisella
Legionella
Yersinia pestis
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21
Q

Test for encapsulated bacteria

A

Quellung reaction (anti-capsule antisera)

If serum is added –> qapsular swellung (quellung)

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22
Q

Encapsulated bacteria

A

SHiNE SKiS

Strep pneumo
H. flu
Neisseria meningitidis
E. coli
Salmonella
Klebsiella
Strep B (GBS)
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23
Q

Catalase positive organisms

A

“You need PLACESS for your cats”

Pseudomonas
Listeria
Aspergillus
Candida
E. coli
Staph aureus
Serratia
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24
Protein-conjugated vaccines
Prevnar (pneumococcal) H. flu type B Meningococcal
25
Urease positive organisms
K PUNCHES ``` Klebsiella Proteus Ureaplasma Nocardia Cryptococcus (fungi) H. pylori Epidermidis Saprophyticus ```
26
Pigment producing bacteria: Red Green/Blue Yellow
``` Red = Serratia Green/Blue = Pseudomonas Yellow = either Actinomyces israelii or Staph aureus ```
27
IgA protease containing bacteria
SHiN Strep pneumo H. influenza (Type B) Neisseria They can all transform DNA and all have an IgA protease (to colonize respiratory epithelium)
28
Transforming bacteria
SHiN Strep pneumo H. influenza (Type B) Neisseria They also have IgA protease
29
Group A Strep virulence factor
M protein Prevents phagocytosis
30
Staph aureus virulence factor
Protein A Binds Fc region of Ig to prevent opsonization & phagocytosis
31
C. diphtheriae toxin
Diphtheria toxin - inactivates elongation factor (EF-2)
32
Pseudomonas toxin
Exotoxin A - inactivates elongation factor (EF-2)
33
Shigella toxin
Shiga toxin: | inactivates 60S ribosome by removing adenine from rRNA
34
EHEC toxin
*Includes O157:H7 Shiga-like toxin: inactivates 60S ribosome by removing adenine from rRNA
35
ETEC toxin
Two toxins: Heat-labile - ^ adenylate cyclase activity --> Cl- excretion Heat-stable - ^ guanylate cyclase activity --> decreased NaCl absorption "Labile in the Air, Stable on the Ground"
36
Anthrax toxin
Edema factor - mimics adenylate cyclase activity
37
Vibrio toxin
Cholera toxin: Permanently activates Gs subunit --> ^ adenylate cyclase activity --> ^ Cl- secretion in gut
38
Pertussis toxin
Pertussis toxin: Disables Gi --> ^ adenylate cyclase activity --> impaired phagocytosis
39
Clostridium tetani toxin
Tetanospasmin - cleaves GABA and Glycine SNARE proteins
40
Clostridium botulinum toxin
Botulinum toxin - Cleaves Ach SNARE proteins
41
Clostridium perfringens toxin
Alpha toxin/Phospholipase C/Lecithinase: Phospholipase that degrades tissue and membranes. Causes gas gangrene and hemolysis.
42
Group A Strep toxin
Streptolysin O: Degrades cell membranes --> hemolysis
43
What do TLR-2 and TLR-4 bind?
TLR-2 = gram+ peptidoglycan TLR-4 = gram- LPS
44
Staph aureus superantigenic toxin
Toxic shock syndrome toxin 1 (TSST-1): Connect MHCII and TCR --> activate all T cells --> ^ IFNg & IL-2
45
Group A Strep superantigenic toxin
Exotoxin A: Connect MHCII and TCR --> activate all T cells --> ^ IFNg & IL-2
46
Bacterial toxins that are encoded in lysogenic phage
ABCDE ``` shigA-like toxin (EHEC) Botulinum toxin Cholera toxin Diphtheria toxin Erythrogenic toxin (GAS) ```
47
Streptococcal spp. hemolysis patterns
Alpha (partial): Strep pneumo Viridans strep Beta (complete): GAS GBS Gamma (absent): Enterococcus (Group D) Strep bovis
48
Novobiocin resistant/sensitive
Staph: Saprophyticus is resistant Epidermidis is sensitive
49
Streptococcus: Optochin sensitive/resistant Bactitracin sensitive/resistant
Optochin: Strep pneumo is afraid of-the-chin, viridans are not Bacitracin BRAS --> B Resistant, A sensitive
50
Gram+ rods
``` Clostridium Corynebacterium Listeria Bacillus Mycobacterium ```
51
What bacterial vaccines are live-attenuated?
Only Bacille Calmette-Guerin (BCG for Tuberculosis) & F. tularensis.
52
Lactose fermenting enterics
test with maconCKEE'S agar ``` Citrobacter Klebsiella E. coli Enterobacter Serratia ```
53
Gram- rod lactose nonfermenters Oxidase+? Oxidase-?
Oxidase+: Pseudomonas H. pylori Oxidase-: Salmonella Shigella Proteus
54
EMB agar
Eosin-Methylene blue agar Lactose fermenters grow as purple/black colonies. E. coli grows as a purple colony with a green shine.
55
Neisseria spp. Glucose? Maltose?
``` Meningococci = Maltose & glucose Gonococci = just Glucose ```
56
VDRL false positives
Viruses (mono, hepatitis) Drugs Rheumatic fever Lupus & Leprosy
57
Rash on palms & soles DDx
Syphilis (secondary & congenital) Rocky Mountain Spotted Fever Coxsackie A (hand, foot, & mouth dz) Kawasaki dz
58
Intestinal nematode routes of infection
Ingested - EAT Enterobius Ascaris Trichinella Cutaneous - SANd Strongyloides Ancylostoma Necator
59
Treatment for intestinal nematodes
Bendazoles "Roundworms are bendy"
60
Treatment for Cestodes & Trematodes
Cestodes (tapeworms) & Trematodes (flukes) = Praziquantel ``` The exceptions are those that have a cyst form: Taenia solium (cysticercosis --> bendazoles) Echinococcus granulosus (liver cysts --> bendazoles) ```
61
Which viral vaccines are live-attenuated?
``` Smallpox Yellow fever Chicken Pox Polio (Sabin) MMR Influenza (intranasal one) ```
62
Which viral vaccines are killed?
"RIP, eh" Rabies Influenza (IM) Polio (Salk) HAV
63
Which viral vaccines are recombinant?
HBV (HBsAg) & HPV (6, 11, 16, 18)
64
What are the DNA viruses?
HHAPPPPy ``` Hepadena Herpes Adeno Pox Parvo Papilloma Polyoma ```
65
What are the exceptions to the rule that viruses are either dsDNA or ssRNA?
ssDNA = Parvovirus (Par 1) dsRNA = Reovirus
66
What viruses are associated with intussusception of the small bowl in children?
Adenovirus & Rotavirus
67
Are viruses haploid or diploid?
All are haploid except retroviruses (2 identical ssRNA copies)
68
Which viruses replicate in the nucleus? | The cytoplasm?
All DNA replicate in nucleus (except poxvirus) All RNA replicate in cytoplasm (except influenza & retroviruses)
69
Which viruses are naked/enveloped?
"Naked CPR & PAPP Calcivirus Picornavirus Reovirus Papillomavirus Adenovirus Parvovirus Polyomavirus All others are enveloped
70
Which viruses are negative stranded?
"Always Bring Polymerase Or Fail Replication" ``` Arenavirus Bunyavirus Paramyxovirus Orthomyxovirus Filovirus Rhabdovirus ```
71
Which viruses can undergo reassortment?
Segmented genomes = BOAR Bunyavirus Orthomyxovirus Arenavirus Reovirus
72
C. difficile toxins
Toxin A - enterotoxin; PMN chemoattractant & secretory diarrhea Toxin B - cytotoxin; actin depolymerization & mucosal necrosis
73
``` HIV genes, what do they encode? gag pol env rev nef ```
``` gag = p24, p7 (capsid and matrix proteins, respectively) pol = Reverse transcriptase, integrase, protease env = gp120, gp41 (gp160 is cleaved into them) rev = protein that transports viral transcripts out of nucleus nef = proteins to downregulate CD4 and MHCI ```
74
What receptor does HIV bind?
Macrophage tropic = CCR5 & CD4 T-cell tropic = CXCR4 & CD4 Early disease is macrophage tropic & late disease is T-cell tropic.
75
``` What do these indicate in the serum? HBsAg HBeAg Anti-HBs Anti-HBc Anti-HBe ```
``` HBsAg = hepatitis B infection HBeAg = active viral replication (high transmissibility) Anti-HBs = immunity to HBV Anti-HBc = window period Anti-HBe = low transmissibility ```
76
What is the order of serum markers in HBV infection?
SECES ``` HBsAg HBeAg Anti-HBc Anti-HBe Anti-HBs ```
77
What cohort of people are immune to HIV?
Homozygous delta32 mutation. These patients have mutant CCR5. Heterozygotes have a slower progression of HIV disease.
78
What dermatologic diseases can infect HIV patients?
``` Candida infection (thrush) Bartonella henselae (bacillary angiomatosis) ```
79
AIDS patient, GI symptoms
Cryptosporidium
80
What neuro diseases can infect HIV patients?
``` Progressive multifocal leukoencephalopathy (JC virus) Toxoplasma abscesses Cryptococcal meningitis CMV retinitis HIV dementia ```
81
What oncologic conditions are found in HIV patients?
``` Kaposi's Sarcoma (HHV-8) Hairy Leukoplakia (EBV) Non-Hodgkin's Lymphoma (Waldeyer's Ring) SCC (cervix or anus if MSM) CNS Lymphoma ```
82
What respiratory infections are seen in HIV patients?
Pneumocystis jirovecii CMV pneumonia Invasive aspergillosis Mycobacterium avium-intracellulare (TB-like illness)
83
What bugs are associated with food poisoning?
``` Vibrio parahaemolyticus & vulnificus Bacillus cereus S. aureus Clostridium perfringens Clostridium botulinum E. coli O157:H7 Salmonella ```
84
What bugs are associated with dysentary?
``` Campylobacter Salmonella Shigella EHEC EIEC Yersinia enterocolitica Entamoeba histolytica ```
85
What bugs are associated with watery diarrhea?
``` ETEC Vibrio cholerae C. difficile (can also cause dysentery) C. perfringens Cryptosporidium (AIDS) Rotavirus (children) Norovirus (adults) ```
86
What are the common causes of pneumonia in neonates?
GBS | E. coli
87
What are the common causes of pneumonia in children?
"Runts May Cough Chunky Sputum" ``` RSV Mycoplasma Chlamydia trachomatis Chlamydia pneumoniae Strep pneumo ```
88
What are the common causes of pneumonia in young adults (18-40)?
Strep pneumo Mycoplasma Chlamydia pneumoniae
89
What are the common causes of pneumonia in older adults (40-65)?
``` Strep pneumo H. influenza Mycoplasma Anaerobes Viruses ```
90
What are the common causes of pneumonia in the elderly (>65)?
``` Step pneumo Influenza H. flu Anaerobes Gram negative rods ```
91
What are the common causes of pneumonia in nosocomial infections?
Staph | Enteric gram negative rods
92
What are the common causes of pneumonia in aspiration?
Anaerobes
93
What are the common causes of pneumonia in Alcoholics/IVDA?
Strep pneumo Klebsiella Staph
94
What are the common causes of pneumonia in CF patients?
Pseudomonas Staph aureus Strep pneumo Burkholderia cepacia
95
What are the common causes of atypical pneumonia?
Mycoplasma Legionella Chlamydia
96
What are the common causes of meningitis in newborns (0-6 mo.)?
GBS E. coli Listeria
97
What are the common causes of meningitis in children (6m-6y)?
Strep pneumo Neisseria meningitidis H. flu (Type B) Enteroviruses
98
What are the common causes of meningitis in most people (6-60y)?
Strep pneumo Neisseria meningitidis (#1 in teens) Enteroviruses HSV
99
What are the common causes of meningitis in the elderly?
Strep pneumo Gram negative rods Listeria
100
What is the empiric therapy for bacterial meningitis?
Cefriaxone + Vancomycin + Ampicillin if < 6m or > 60y (Listeria)
101
``` Osteomyelitis bugs: Diabetics IVDA Sickle Cell Prosthetic replacement Vertebral osteomyelitis Cat/Dog bite Everyone else ```
``` Diabetics - Pseudomonas IVDA - Pseudomonas Sickle Cell - Salmonella Prosthetic replacement - S. epidermidis Vertebral osteomyelitis - MTB Cat/Dog bite - Pasteurella multocida Everyone else - S. aureus ```
102
What does a positive nitrite test indicate? | Positive leukocyte esterase test?
Nitrite = Gram negative bacteria UTI | Leukocyte esterase = Bacterial UTI
103
What are the most common causes of UTI?
1) E. coli 2) Staph saprophyticus 3) Klebsiella Others: Proteus Serratia (nosocomial) Pseudomonas (nosocomial) Enterobacter (nosocomial)
104
Congenital Toxoplasma presentation
Triad: Chorioretinitis Hydrocephalus Intracranial calcifications
105
Congenital Rubella presentation
``` Triad: PDA Cataracts Deafness +/- rash ```
106
Congenital CMV presentation
Convulsions (seizures) Mute (deafness) Violaceous rash (petechiae)
107
Congenital HIV presentation
Recurrent infections | Chronic diarrhea
108
Congenital HSV-2 presentation
Encephalitis | Herpes lesions
109
Congenital syphilis presentation
Stillbirth/hydrops fetalis Syphilis facies: notched teeth, saddle nose Saber shins CNVIII deafness
110
Red rashes of childhood
Rubella (descending) Measles (descending) VZV (begins on trunk) Roseola (HHV-6; follows several days high fever) Parvovirus (slapped cheek rash) Streptococcus pyogenes (sandpaper-like rash) Coxsackie A (vesicular on palms, soles, oral mucosa)
111
What organism causes a painful genital ulcer?
Haemophilus ducreyi "It's so painful that you do-cry"
112
What is the main cause of bacterial vaginosis? What are its characteristics? Treatment?
Gardnerella vaginalis "I don't have a Clue why I smell Fish in the Vagina Garden" Clue cells Fishy smell Gardnerella vaginalis Treatment = Metronidazole
113
What is the most common bacterial STI in the United States?
Chlamydia trachomatis
114
What pathogen should you think of for nosocomial: Mechanical ventilation? Urinary catheterization? Open wounds?
Mechanical ventilation = Pseudomonas Urinary catheterization = E. coli, Proteus Open wounds = S. aureus
115
What microbes are more common in asplenic patients?
Encapsulated organisms, especially SHiN
116
What is the mechanism of beta-Lactams?
Bind to PBP's (transpeptidases) to block cross-linking of peptidoglycan.
117
What is the use of nafcillin?
Naf for staph Cannot use for MRSA.
118
What is the spectrum of Ampicillin/Amoxicillin?
Aminopenicillins = "HELPS kill Enterococci" ``` H. flu E. coli Listeria Proteus Salmonella/Shigella Enterococci ```
119
What is the spectrum of Piperacillin?
Gram- rods Group & viridans strep Anaerobes
120
What are the beta-lactamase inhibitors?
Clavulonic acid Sulbactam Tazobactam
121
What is the mechanism of cephalosporins?
They are beta-lactams that inhibit cell wall synthesis
122
What is the spectrum of 1st generation cephalosporins?
PEcK: gram+ cocci Proteus E. coli Klebsiella
123
What is the spectrum of 2nd generation cephalosporins?
HEN PEcKS: ``` gram+ cocci H. flu Enterobacter Neisseria spp. Proteus E. coli Klebsiella Serratia ```
124
What are 3rd generation cephalosporins used for?
Ceftriaxone: Meningitis empiric therapy Gonorrhea Ceftazidime/Cefoperazone: Pseudomonas
125
What is the spectrum of 4th generation cephalosporins?
Everything except LAME: Listeria Atypicals MRSA Enterococci
126
What is the spectrum of Aztreonam?
Gram- rods only (including pseudomonas)
127
What is the spectrum of the Carbapenems?
Cover pretty much anything. Limited to life-threatening infections due to side effects. "We have a positive ID on a negative MD" Imipenem, Doripenem = gram+ Meropenem, Doripenem = gram-
128
What is the mechanism of Vancomycin?
Inhibits peptidoglycan formation by binding up D-ala D-ala portion of cell wall precursors.
129
What is the spectrum of Vancomycin?
Gram+ only Saved for use in serious multidrug resistant organisms: MRSA Enterococci C. diff
130
Where do each of the protein synthesis inhibitor abx work?
"Buy AT 30, CCEL at 50" 30S inhibitors: Aminoglycosides Tetracyclines ``` 50S inhibitors: Chloramphenicol Clindamycin Erythromycin Linezolid ```
131
What are the common aminoglycosides?
``` Gentamycin Neomycin Amikacin Tobramycin Streptomycin ```
132
What is the spectrum of aminoglycosides?
Gram- rod infections
133
What are the toxicities of aminoglycosides?
AminO2glycosides do NNOT kill anaerobes Nephrotoxic Neuromuscular blockade Ototoxic Teratogenic
134
What toxicities are associated with macrolides?
MACRO: ``` Motility issues Arrhythmia (prolonged QT) Cholestatic hepatitis Rash eOsinophilia ```
135
What two drugs are classically used for anaerobes?
``` Clindamycin = anaerobes above the diaphragm Metronidazole = anaerobes below the diaphragm ```
136
What is the mechanism of Bactrim?
Bactrim = Trimethoprim-Sulfamethoxazole (TMP-SMX) Trimethoprim - inhibits Dihydrofolate reductase Sulfamethoxazole - inhibits Dihydropteroate synthase
137
What is the mechanism of Fluoroquinolones?
Inhibitis DNA Topoisomerase II (gyrase) & Topoisomerase IV -oxacin
138
What is the spectrum of Fluoroquinolones?
Gram- rods of urinary and GI tracts | Neisseria
139
What are the clinical uses of metronidazole?
"GET GAP on the metro" ``` Giardia Entamoeba histolytica Trichomonas Garderella vaginalis Anaerobes Pylori (H. pylori; requires triple therapy PPI+MAC) ```
140
What is the mechanism of Metronidazole?
Forms free radicals in the bacterial cell --> cell damage & death
141
What distinguishes Ertapenem?
It has a longer half life (4h) but does not cover Pseudomonas.
142
What toxicities are associated with doxycyline?
Discoloration of teeth in children under 8y (contraindicated in pregnancy & children) Photosensitivity
143
What is used for prophylaxis for MTB? | MAC?
``` MTB = Isoniazid MAC = Azithromycin ```
144
What is the treatment for MTB?
RIPE Rifampin Isoniazid Pyrazinamide Ethambutol
145
Which anti-MTB drugs are hepatotoxic?
RIP
146
What toxicities are found with Isoniazid?
Neurotoxic Hepatotoxic Drug-induced lupus INH = Injures Neurons & Hepatocytes
147
What toxicities are found with Rifampin?
Hepatotoxic Many drug interactions (^CYP450 activity) Orange body fluids ``` 4 R's: RNA polymerase inhibitor Revs up CYP450 Red/orange body fluids Rapid resistance if used alone ```
148
What toxicities are found with Ethambutol?
Optic side effects: Retrobulbar neuritis Central scotoma Red/green color blindness
149
What is used as prophylaxis for gonococcal or chlamydial conjunctivitis in newborns?
Erythromycin drops
150
What is used as prophylaxis for pregnant women carrying GBS?
Ampicillin
151
What is used as prophylaxis for surgical infections?
Cefazolin (to prevent S. aureus infection)
152
What is used as prophylaxis for recurrent UTI's?
TMP-SMX
153
What are the guidelines for prophylaxis in AIDS patients?
< 200 = TMP-SMX (PCP) < 100 = TMP-SMX (PCP & Toxoplasmosis) < 50 = Azithromycin (MAC)
154
What treatment is recommended for MRSA? | VRE?
``` MRSA = Vancomycin; Daptomycin; Linezolid; Tigecycline; Ceftaroline VRE = Linezolid + Streptogramins ```
155
What infections can H. influenzae cause?
haEMOPhilus Epiglottitis Meningitis Otitis media Pneumonia Incidence has gone way down due to vaccination against Type B. Other nontypeable strains only cause mucosal infections (otitis media, conjunctivitis, bronchitis).
156
What do Th1 cells secrete?
gamma-Interferon | IL-2
157
What are the major cytokines secreted by Th2 cells?
IL-4 IL-5 IL-10 IL-13
158
What cytokines stimulate class switching to IgE?
IL-4 --> IgG & IgE | IL-4 + IL-13 --> IgE
159
What does IL-5 do?
Th2 cells Promotes class switching to IgA and proliferation of eosinophils.
160
Which streptococci can be cultured in bile? | In 6.5% NaCl and bile?
Bile = both Enterococci & other Group D Strep (S. bovis) | 6.5% NaCl = Just enterococci
161
What is the mechanism of Oseltamivir?
Oseltmivir = NA inhibitor (inhibits virion release from cells)
162
What are the most common pathogens in an intra-abdominal infection?
Bacteroides fragilis > E. coli > everything else (polymicrobial)
163
What are the clinical uses for Amphotericin B?
``` Pregnancy (the only antifungal) Serious systemic mycoses Cryptococcus Blasto/Histo/Coccidio Mucormycoses Invasive candida Fungal meningitis (intrathecal) ```
164
What are the adverse effects of Amphotericin B?
Fever/Chills ("Ampho-terrible") Hypotension Anemia Thrombophlebitis
165
What is the treatment for systemic mycoses?
Histo/Blasto/Coccidio Itraconazole/Fluconazole for local infection Amphotericin B for systemic infection
166
Cryptococcal meningitis treatment
Amphotericin B | +/- Flucytosine
167
What is the typical treatment for candida infection?
Fluconazole If invasive --> Echinocandin (Caspofungin, Micafungin)
168
What is the mechanism of Ribavirin?
Inhibits IMP dehydrogenase --> no guanine synthesis
169
When is Ribavirin indicated?
RSV | Chronic Hepatitis C
170
When is Acyclovir indicated?
HSV and VZV infections Does NOT work against CMV (Ganciclovir)
171
What are the oral forms of Acyclovir and Gancyclovir?
Valacyclovir & Valganciclovir
172
When is Foscarnet indicated?
CMV retinitis when Ganciclovir has failed | Acyclovir-resistant HSV
173
When is Cidofovir indicated?
CMV retinitis | Acyclovir-resistant HSV
174
What are possible regimens for HAART?
2 NRTI's + either: 1 NNRTI 1 PI 1 Integrase inhibitor
175
What drug boosts other protease inhibitors in HAART?
Ritonavir **All PI's end in -navir
176
What antiretroviral is used during pregnancy?
Zidovudine (ZDV/AZT) To reduce risk of fetal transmission.
177
What are the adverse effects of Protease inhibitors?
Metabolic syndrome | GI intolerance
178
What NRTI's can cause lactic acidosis?
Didanosine > Stavudine > Zidovudine
179
What antiretroviral should be avoided in pregnancy?
Efavirenz
180
When is IFN-alpha indicated?
Chronic Hepatitis B & C | Kaposi's Sarcoma
181
When is IFN-beta indicated?
Multiple Sclerosis
182
When is IFN-gamma indicated?
NADPH oxidase deficiency
183
Which anti-infectious agents should be avoided in pregnancy?
"SAFe Children Take Really Good Care" ``` Sulfonamides (Kernicterus) Aminoglycosides (Ototoxicity) Fluoroquinolones (Cartilage damage) Clarithromycin (Embryotoxic) Tetracyclines (Discolored teeth) Ribavirin (Teratogen) Griseofulvin (Teratogen) Chloramphenicol (Gray baby syndrome) ```
184
What is used to treat MAC infection?
ARES protocol: Azithromycin Rifampin Ethambutol Streptomycin
185
What is used to treat Hansen's disease?
M. leprae Long term tx Tuberculoid form = Dapsone & Rifampin Lepromatous form = Dapsone, Rifampin, Clofazimine
186
What are the respiratory fluoroquinolones?
Gemifloxacin Levofloxacin Moxifloxacin
187
What are the anti-pseudomonal fluoroquinolones?
Ciprofloxacin | Levofloxacin
188
Rusty colored sputum
Strep pneumo pneumonia
189
What are the two possible outcomes of Bacillus cereus food poisoning?
Heat stable toxin --> Emetic form (1-5h after ingestion) | Heat labile toxin --> diarrheal form (15-20h after ingestion)
190
What types of cells are found in a lymph node? | Where are they found?
``` Cortex = Follicles of B cells Paracortex = T cells (location of high endothelial venules) Medulla = Medullary cords & sinuses contain macrophages ```
191
Lymph drainage: | Upper limb
Axillary
192
Lymph drainage: | Breast
Axillary
193
Lymph drainage: | Stomach
Celiac
194
Lymph drainage: | Duodenum & Jejunum
Superior mesenteric
195
Lymph drainage: | Sigmoid colon
Colic --> inferior mesenteric
196
Lymph drainage: | Rectum & anus (above pectinate line)
Internal iliac
197
``` Lymph drainage: Anal canal (below pectinate line) ```
Superficial inguinal
198
Lymph drainage: | Testes
Superficial & deep plexuses --> para-aortic
199
Lymph drainage: | Scrotum
Superficial inguinal
200
Lymph drainage: | Superficial thigh
Superficial inguinal
201
Lymph drainage: | Dorsolateral foot
Popliteal
202
What are the two main lymphatic ducts and what do they drain? Where do they empty into the circulation?
Right lymphatic duct = Right arm, Right chest, Right 1/2 of head Thoracic duct = everything else They both empty into their respective subclavian veins.
203
What infections are seen in asplenic patients?
Encapsulated organisms ``` Strep pneumo H. flu i Neisseria meningitidis E. coli Salmonella Klebsiella i Strep agalactiae (GBS) ```
204
What hematologic changes are seen in asplenic patients?
Howell-Jolly bodies (nuclear remnants) Target cells Thrombocytosis
205
Which way do T cells move as they mature in the thymus?
From cortex (positive selection) to medulla (negative selection).
206
What are the findings in Down Syndrome?
``` Mental retardation Flat facies Prominent epicanthal folds Oblique palpebral fissures Simian crease Gap between first 2 toes Duodenal atresia Ostium primum-type ASD (cushion defect) ```
207
What are Down syndrome patients at increased risk of?
ALL | Alzheimers (young onset)
208
What are the possible etiologies of Down Syndrome?
1) Meiotic nondisjunction (95% of cases; advanced maternal age) 2) Robertsonian translocation (4% of cases) 3) Mosaicism (1% of cases)
209
What organisms are capable of producing biofilms?
``` Staph epidermidis Strep mutans & sanguinis Pseudomonas Viridans strep Nontypable H. flu ```
210
What are the uses of Rifampin?
MTB & Leprosy = combination therapy | H. flu & N. meningitidis = monotherapy prophylaxis
211
What is Sucrose composed of? Lactose? Maltose?
``` Maltose = Glucose + Glucose Lactose = Glucose + Galactose Sucrose = Glucose + Fructose ```
212
What possible effects does uncontrolled maternal diabetes have on a fetus?
``` Prematurity Fetal macrosomia Neural tube defects Hypoglycemia Hypocalcemia Polycythemia NRDS Transient HOCM ```
213
What is produced through cleavage of POMC?
beta-endorphins ACTH MSH
214
What are the commonest complications of prematurity?
``` NRDS PDA Bronchopulmonary dysplasia Intraventricular hemorrhage (germinal matrix) Necrotizing enterocolitis Retinopathy of prematurity ```
215
What passes through the optic canal?
CNII Ophthalmic artery Central retinal vein
216
What passes through the superior orbital fissure?
CNIII, CNIV, CNV1, CNVI Ophthalmic vein Sympathetic fibers
217
What foramina do the divisions of the trigeminal nerve pass through?
Standing Room Only ``` V1 = Superior orbital fissure V2 = Foramen rotundum V3 = Foramen ovale ```
218
What passes through the foramen spinosum?
Middle meningeal artery & vein
219
What passes through the internal acoustic meatus?
CNVII, CNVIII
220
What passes through the jugular foramen?
CNIX, X, XI Jugular vein "Nine, ten, eleven, jugular foramen"
221
What are "Mongoloid features" associated with?
Down Syndrome | Beta-thalassemia
222
What HLA's are associated with each MHC type?
``` MHCI = HLA-A,B,C MHCII = HLA-DR,DP,DQ ```
223
What do each MHC class bind?
MHCI --> TCR & CD8 (CTL activation) | MHCII --> TCR & CD4 (Th activation)
224
Where are MHCI and MHCII expressed?
``` MHCI = all cells except RBC's MHCII = only on APC's ```
225
What disease is this HLA subtype associated with? | A3
Hemochromatosis
226
What disease is this HLA subtype associated with? | B27
PAIR Psoriasis Ankylosing spondylitis IBD Reiter's syndrome
227
What disease is this HLA subtype associated with? | DQ2/DQ8
"DQ2, Celiac sprue" | Also DQ8
228
What disease is this HLA subtype associated with? | DR2
Multiple sclerosis Hay fever SLE Goodpastures
229
What disease is this HLA subtype associated with? | DR3
T1DM | Graves' Disease
230
What disease is this HLA subtype associated with? | DR4
Rheumatoid arthritis | T1DM
231
What disease is this HLA subtype associated with? | DR5
Pernicious anemia | Hashimoto's thyroiditis
232
What HLA's are associated with T1DM?
DR3 & DR4
233
What do NK cells respond to?
Activity increased by IL-2, IL-12, IFN-alpha, IFN-beta | Activated to kill by absence of MHCI on target cell surface or nonspecific activation signals on target cell surface.
234
What is positive and negative selection of T cells?
Positive selection = TCR cell can bind self Negative selection = TCR does not bind self with high affinity These occur in the thymus.
235
What interactions are required for T cell activation?
CD4: TCR/CD4-MHCII CD28-B7 (dendritic cell) CD8: TCR/CD8-MHCI CD28-B7 (dendritic cell)
236
What interactions are required for B cell activation?
1) BCR-mediated endocytosis (B cell) MHCII-TCR (CD4 T cell) (B cell) CD40-CD40L (T cell) This causes activation of B cell & release of cytokines from T cell --> class switching, affinity maturation, Ab production
237
What cytokines are secreted by Th1 cells?
IFN-gamma
238
What cytokine induces a T cell to go the Th1 route?
IL-12
239
What cytokines do Th2 cells release?
IL-4, IL-5, IL-10, IL-13
240
What do CTL's use to kill cells?
Perforin - holes in membrane Granzyme - serine protease --> apoptosis inside target cell Granulysin - induces apoptosis
241
What is the main purpose of a Th1 & a Th2 response?
Th1: Activates macrophages (inflammation) & intracellular immunity Th2: Recruits eosinophils (parasites) & promotes Ig production (extracellular immunity)
242
What cell surface markers are found on T-regs?
CD3 CD4 CD25
243
What do T-regs secrete?
IL-10 | TGF-beta
244
What are the 3 ways that Ab's combat infection?
1) Opsonization (promotes phagocytosis) 2) Neutralization (prevents binding) 3) Complement activation (MAC)
245
What portion of an antibody activates complement?
CH2 portion (Fc portion close to the neck) This only occurs with IgM & IgG
246
What enzyme adds additional nucleotides during V(D)J recombination?
TdT = Terminal deoxynucleotidyl Transferase
247
What type of Ig's are expressed on mature B cells?
IgM & IgD
248
Most produced Ig subtype? Highest serum titer? Lowest serum titer?
Most produced = IgA Highest titer = IgG Lowest titer = IgE
249
What does IgE do?
Binds to and sensitizes mast cells & basophils Activates eosinophils against worms Type I hypersensitivity
250
What is a Thymus Independent Antigen?
Activation of B cells is T-cell independent, either by: 1) Crosslinking of BCR's due to repeating epitopes 2) TLR activation in an innate cell --> signaling This type of response occurs in response to non-peptide antigens, such as a polysaccharide capsule. It generates NO IMMUNOLOGIC MEMORY. This is why we have to conjugate Strep pneumo, H. flu, Neisseria vaccines to a peptide. Then there can be an immunologic memory and a thymus-dependent response.
251
What is the function of the membrane attack complex?
Lysis GRAM- cells by punching holes in it
252
What are the specific functions of the complement proteins?
C3b - opsonization C3a, C5a - anaphylaxis C5a - PMN chemotaxis C5b-C9 - MAC
253
What are the inhibitors of complement?
Decay Accelerating Factor (DAF) C1 Esterase Inhibitor They help to prevent activation on self
254
What initiates the Classical pathway? Alternative pathway? Lectin pathway?
``` Classical = Ab bound to microbial surface Alternative = C3 spontaneously hydrolyzed to C3b Lectin = Mannose-Binding Lectin (MLB) binds mannose, glucose, etc. (polysaccharide) ```
255
How does a C1 esterase inhibitor deficiency manifest?
Hereditary angioedema, especially periorbital. ACE inhibitors contraindicated
256
How does a C3 deficiency manifest?
Recurrent pyogenic sinus & respiratory tract infections Also ^ susceptibility to Type III (immune complex) hypersensitivity because C3 is involved in clearing of immune complexes.
257
How do C5-C9 deficiencies manifest?
Recurrent Neisseria infections
258
How does DAF deficiency manifest?
Decay Accelerating Factor (GPI anchored enzyme; normally inhibits complement) Complement mediated hemolysis & PNH
259
What amino acid(s) are pyrimidines synthesized from?
Glutamate + Aspartate
260
What amino acid(s) are purines synthesized from?
Glutamate + Aspartate + Glycine
261
What amino acid(s) is Niacin synthesized from?
Trpytophan
262
What amino acid(s) is Thyroxine synthesized from?
Tyrosine
263
What are the intraluminal stimuli for vasodilation?
``` Ach Bradykinin Serotonin Substance P Shear forces NO ```
264
What is the mechanism of Daptomycin?
Depolarization of the microbial cell membrane
265
What toxicity is seen with Daptomycin?
Myopathy & increased CPK
266
What bacteria are most commonly involved in pneumonia that is secondary to a viral infection?
In order: 1) Strep pneumo 2) Staph aureus 3) H. flu
267
How is mesothelioma diagnosed?
Definitive diagnosis is with EM. Mesothelioma = long slender microvilli Adenocarcinoma = short, plump microvilli
268
What is the effect of sympathetic stimulation on insulin secretion by pancreatic beta cells?
It depends: Alpha receptor stimulation --> decreased insulin release Beta receptor stimulation --> increased insulin release As a whole, sympathetic stimulation generally decreases insulin release while parasympathetic stimulation increases it.
269
How is hemophilia inherited?
Both are usually X-linked Hemophilia A is spontaneous in 30% of cases
270
How is Lesch-Nyhan Syndrome inherited?
X-linked recessive
271
What cancers are typically osteolytic in nature?
BLT w/ Ketchup & Mustard ``` Breast Lung Thyroid Kidney Multiple myeloma ```
272
What do IL-1 through IL-5 do?
Hot T-Bone stEAk ``` IL-1 = pyrogen IL-2 = stimulates T cells IL-3 = stimulates bone marrow IL-4 = class switch to IgE IL-5 = class switch to IgA ```
273
What does IL-6 do?
Fever & acute-phase reactants
274
What does IL-8 do?
The major chemoattractant for neutrophils "Clean up on aisle 8"
275
What amino acid(s) is NO synthesized from?
Arginine
277
What cytokines are secreted by Th2 cells?
IL-4,5,10
278
What does IL-10 do?
Inhibits inflammation and Th1 Secreted by Th2 cells & T-regs
279
What occurs in cells exposed to IFN-alpha & beta?
Protein Kinase R (PKR) is produced. In the presence of dsRNA, it phosphorylates two proteins: 1) RNAse L --> degrades RNA 2) eIF2 --> inhibits translation
280
What cell surface markers are found on T cells?
``` TCR CD3 CD28 If Th - CD4, CD40L If CTL - CD8 ```
281
What cell surface markers are found on B cells?
BCR (Ig) CD19,20,21,40 MHCII B7
282
What cell surface markers are found on macrophages?
CD14,40 MHCII B7 Fc & C3b receptors (for enhanced phagocytosis)
283
What cell surface markers are found on NK cells?
CD16 (binds Fc of IgG) | CD56 (unique to NK)
284
What receptors bind LPS?
CD14 & TLR-4 | macrophages
285
What is the half life of passive immunity?
3 weeks
286
What exposures require administration of passive immunity?
if you want "To Be Healed Rapidly" Tetanus toxin Botulinum toxin HBV Rabies virus
287
What types of responses are induced with a live-attenuated vaccine vs an inactivated one?
``` Live-attenuated = cellular response Killed = humoral response ``` Killed are safer but often requires boosters.
288
What does IL-12 do?
Induces differentiation of T cells into Th1 cells | Activates NK cells
289
What does TNF-alpha do?
Responsible for septic shock Activates endothelium Leukocyte recruitment Vascular permeability
290
What cytokines are secreted by macrophages?
IL-1,6,8,12, TNF-alpha
291
When does serum sickness occur?
5-10 days post-exposure
292
What are serum sickness & arthus reaction?
Both are type III hypersensitivity Serum sickness is systemic - fever, urticaria, proteinuria, LAD Arthus reaction is localized - edema, necrosis
293
What are the types of blood transfusion reactions?
Allergic (Type I) Anaphylactic Febrile nonhemolytic transfusion reaction (FNHTR; Type II) Acute hemolytic transfusion reaction (HTR; Type II)
294
Anti-dsDNA, anti-Smith
SLE
295
Antihistone Ab's
Drug-induced lupus
296
Rheumatoid factor | anti-CCP
Rheumatoid arthritis
297
Anticentromere Ab's
CREST syndrome
298
anti-DNA topoisomerase I
aka anti-Scl-70 | Scleroderma
299
Anti-mitochondrial Ab's
Primary biliary cirrhosis
300
IgA anti-endomysial | IgA anti-tissue transglutaminase
Celiac disease
301
Anti-basement membrane
Goodpasture's
302
Anti-desmoglein Ab's
Pemphigus vulgaris
303
Antimicrosomal | Antithyroglobulin
Hashimoto's
304
Anti-Jo-1 anti-SRP anti-Mi-2
Polymyositis/Dermatomyositis
305
Anti-Ro | Anti-La
aka Anti-SSA/Anti-SSB Sjogren's syndrome
306
Anti-U1 RNP
Mixed connective tissue disease
307
Anti-smooth muscle Ab's
Autoimmune hepatitis
308
Anti-glutamate decarboxylase Ab's
T1DM
309
PR3-ANCA
AKA c-ANCA Granulomatosis with polyangiitis
310
MPO-ANCA
AKA p-ANCA Microscopic polyangiitis Churg-Strauss syndrome
311
What infections are seen in granulocyte deficiency?
Staphylococcus Burkholderia cepacia Serratia Nocardia Candida Aspergillus
312
What infections are seen in complement deficiency?
Neisseria
313
What type of infections are typically seen in T vs. B cell deficiency?
T-cell deficiency = fungal & viral infections | B-cell deficiency = encapsulated bacteria, enteroviral, and giardia infection
314
What are the characteristics of axonal reaction?
Rounded cell body Nucleus displaced to periphery Central chromatolysis (dispersion of Nissl substance) These changes are secondary to Wallerian degeneration. They represent increased protein synthesis in order to regenerate a severed axon.
315
What cofactors are required for: alpha-Ketoacid Dehydrogenase Pyruvate Dehydrogenase alpha-Ketoglutarate Dehydrogenase
"Tender Loving Care For Nancy" ``` Thiamine pyrophosphate Lipoate Coenzyme A FAD NAD ```
316
What is the Ddx for pulsus paradoxis?
Pulsus paradoxis = pulses decrease with inspiration ``` Ddx: Cardiac tamponade Constrictive pericarditis Severe obstructive lung disease Restrictive cardiomyopathy ```
317
How do the endemic fungi appear microscopically?
``` Histo = small, within macrophages (Histo hides) Blasto = Blasto Buds Broadly; same size as RBC Coccidio = Large spherule filled with endospores (Coccidio crowds) ```
318
What other receptors can hCG bind to?
hCG is an LH analog & can also bind to TSH receptor if in high enough concentrations. Thus, patients with choriocarcinoma/teratoma can exhibit hyperthyroidism and men can sometimes show gynecomastia.
319
What can be used to detect colon cancer recurrence?
Serume carcinoembryonic antigen (CEA)
320
What is seen hematalogically with CVID?
Normal number of B cells, reduced plasma cells & Ig titers
321
What is seen in DiGeorge syndrome?
CATCH-22 ``` 22q11 deletion syndromes: Cleft palate Abnormal facies Thymic aplasia Cardiac defects Hypocalcemia (parathyroid aplasia) 22q11 deletion ``` ``` DiGeorge = TCH VeloCARDIOFACIAL = the cardiac and facial (including cleft palate) defects ```
322
What does a 22q11 deletion cause embryologically?
Malformation of: 3rd branchial pouch (inf. parathyroids & thymus) 4th branchial pouch (sup. parathyroids)
323
What infections are seen in IL-12 receptor deficiency?
Disseminated MTB infections | No Th1 response; no IFN-gamma
324
What are the symptoms of Job syndrome?
Hyper-IgE Syndrome = FATED ``` Facies (course) Abscesses (noninflammatory; Staph) Teeth (keep baby teeth) IgE Dermatologic problems (eczema) ```
325
What causes Job Syndrome?
Hyper-IgE Syndrome Caused by a failure of Th1 cells to release IFN-gamma --> no neutrophil chemotaxis
326
What causes chronic mucocutaneous candidiasis?
Various forms of T cell dysfunction
327
What are the most common causes of SCID?
Severe Combine Immunodeficiency: 1) IL-2 receptor mutation (X-linked) 2) Adenosine Deaminase deficiency (ADA)
328
How does SCID present?
Failure to thrive Recurrent infections (bact, viral, fungal, protozoa) Chronic diarrhea, thrush Absence of thymic shadow, germinal centers, and T cells Decreased T-cell Recombinant Excision Circles (TRECs)
329
What is the treatment for SCID?
Bubble baby then BMT
330
What is the mutation seen in IgAtaxia-telangiectasia?
ATM gene mutation | Normally involved in NHEJ
331
What is seen in IgAtaxia-Telangiectasia?
IgA deficiency Cerebellar defects Spider angiomas
332
What serum marker is seen in IgAtaxia-telangiectasia?
^AFP
333
What mutation causes Hyper-IgM Syndrome?
Defective CD40L on Th cells --> B cells cannot class switch
334
How does Hyper-IgM syndrome present?
Sever pyogenic infections early in life | ^ IgM, very low titers of all other classes
335
What mutation causes Wiskott-Aldrich Syndrome?
WASP gene (X-linked) Makes T cells unable to reorganize actin cytoskeleton
336
What abnormal labs are seen in Wiskott-Aldrich syndrome?
Thrombocytopenia | ^IgE & IgA, decreased IgM
337
How does Wiskott-Aldrich Syndrome present?
Wiskott-Aldrich's TIE Thrombocytopenic purpura Infections Eczema
338
What is mutated in Leukocyte Adhesion Deficiency?
CD18 (LFA-1 integrin on phagocytes)
339
What is the presentation of Leukocyte Adhesion Deficiency?
Delayed umbilical cord separation (usually caused by PMN's) Neutrophilia Recurrent bacterial infections No pus formation
340
What mutation causes Chediak-Higashi syndrome?
Lysosomal Trafficking Regulator gene (LYST): Causes microtubule dysfunction in phagosome-lysosome fusion
341
What is seen in Chediak-Higashi Syndrome?
``` Recurrent staph & strep pyogenic infections Albinism Giant granules in neutrophils Peripheral neuropathy Defective primary hemostasis Neutropenia ```
342
What is defective in Chronic Granulomatous Disease?
Lack of NADPH oxidase --> no ROS --> no respiratory burst in neutrophils
343
What is seen in Chronic Granulomatous Disease?
Infection with catalase+ organisms (PLACESS) Dx by either: Dihydrorhodamine (DHR) flow cytometry Nitroblue tetrazolium dye reduction test (no blue color --> CGD)
344
What causes hyperacute transplant rejection?
Preformed anti-donor Ab's --> vessel occlusion --> ischemia/necrosis
345
What causes acute transplant rejection? | Is it reversible?
Recipient CTL's reacting against donor MHC's --> vasculitis of graft vessels with dense lymphocytic infiltrate Reversible with immunosuppressants
346
What causes chronic transplant rejection? | Is it reversible?
Non-self MHCI is perceived as self MHCI with non-self antigen by recipient CTL's --> T-cell & Ab-mediated vascular damage --> fibrosis of graft vessels & tissue Irreversible
347
What organ transplants typically cause GvH disease?
Bone marrow & liver transplant
348
What is Hydroxychloroquine? | What toxicity is seen with it?
It's a weak lipophilic base used to treat malaria, RA, and lupus Can cause retinal damage or hemolysis
349
What is the mechanism of cyclosporine?
Binds cyclophilins --> inhibits calcineurin --> reduced production of IL-2 and IL-2R --> impaired T cell differentiation and activation
350
What drugs are often used as immunosuppressants in transplant recipients?
``` Cyclosporine Tacrolimus Sirolimus Azathioprine Muromonab-CD3 Corticosteroids ```
351
What toxicities are seen with cyclosporine?
``` Nephrotoxic Metabolic syndrome Tremor Gingival hyperplasia Hirsutism ```
352
What is the mechanism of Tacrolimus?
Binds FK-binding protein --> inhibits calcineurin --> decreased IL-2 secretion --> T cell suppression
353
What toxicities are seen with Tacrolimus?
Nephrotoxic Metabolic syndrome Tremor
354
What is the mechanism of Sirolimus (rapamycin)?
Inhibits mTOR --> T-cells unresponsive to IL-2
355
What toxicities are seen with Sirolimus?
Hyperlipidemia Thrombocytopenia Leukopenia
356
What is the mechanism of Azathioprine?
It's the precursor to 6-mercaptopurine --> inhibits purine synthesis
357
What toxicities are seen with Azathioprine?
Bone marrow suppression Toxic effects are increased by xanthine oxidase inhibitors (metabolized by XO normally)
358
What is the mechanism of Muromonab (OKT3)?
mAb against CD3 --> blocks CD3 --> no T cell activation
359
What toxicities are seen with Muromonab?
Cytokine release syndrome (cytokine storm)
360
What is recombinant G-CSF called? | GM-CSF?
``` rG-CSF = Filgrastim rGM-CSF = Sargrastim ```
361
What is Omalizumab used for?
Severe asthma. It is an Ab against IgE.
362
What measurement is determined by using a case-control study? Cohort study?
Case control --> Odds ratio (Casino odds) | Cohort --> Relative risk
363
Phase I trials: Who is enrolled? What is the purpose?
Small number of healthy volunteers Meant to assess safety, toxicity, pharmacokinetics
364
Phase II trials: Who is enrolled? What is the purpose?
Small number of diseased patients Assesses efficacy, optimal dosing, and adverse effects
365
Phase III trials: Who is enrolled? What is the purpose?
Large number of diseased patients randomly assigned (RCT) to treatment of interest or standard of care/placebo. Used to compare the new treatment to the standard of care
366
Phase IV trials: Who is enrolled? What is the purpose?
Postmarketing surveillance following FDA approval Detects rare or long-term effects
367
What is the value of a sensitive test vs a specific test?
SNOUT --> Sensitive test rules out | SPIN --> Specific test rules in
368
What is the equation for sensitivity?
Sn = True Positive/All disease | = 1- false negative
369
What is the equation for PPV?
PPV = TP/(TP + FP)
370
What is the equation for NPV?
NPV = TN/(FN + TN)
371
What test descriptors vary with prevalence?
PPV --> Increases with higher prevalence (pretest probability) NPV --> Decreases with higher prevalence (pretest probability) Sensitivity and Specificity are fixed properties of a test.
372
What are the equations for incidence & prevalence?
Incidence = (# new cases over a time period)/(Population at risk) Prevalence = (# of existing cases)/(Population at risk) Thus Prevalence ~ (Incidence rate)*(duration of disease)
373
Odds ratio formula
OR = AD/BC
374
What is the formula for relative risk?
RR = (A/A+B)/(C/C+D)
375
What is the formula for Attributable Risk?
AR = (A/A+B) - (C/C+D)
376
What is the formula for Absolute Risk Reduction?
ARR = risk following treatment - control risk Determines how much reduction in risk is due to a treatment.
377
What is the formula for number needed to treat?
NNT = 1 / Absolute Risk Reduction
378
What is the formula for number needed to harm?
NNH = 1 / Attributable risk
379
What types of error affect precision vs. accuracy?
Accuracy is affected by systematic error Precision is affected by random error
380
Using only hospital inpatients as subjects. | What type of bias is this?
Berkson's bias (a type of selection bias)
381
What is Hawthorne Effect?
The group knows it is being watched and changes its behavior. It's a form of measurement bias. "Dr. Hawthorne is watching you"
382
The researcher's belief in the efficacy of a treatment changes the outcome of that treatment. What type of bias is this?
Observer-expectancy bias
383
Subjects in different groups are not treated the same. | What type of bias is this?
Procedure bias
384
What can be done to avoid confounding bias?
Crossover studies | Goo matching of groups
385
Earlier detection by a new diagnostic test appears as increased survival. What type of bias is this?
Lead-time bias
386
Information gathered at wrong time (i.e. after half of the patients are dead) What kind of bias is this
Late-look bias
387
What % is contained in +/-1 Standard Deviation? 2? 3?
For a Gaussian distribution: 1 SD = 68% 2 SD = 95% 3 SD = 99.7%
388
What is the formula for for Standard Error of the Mean?
SEM = SD/(square root of n)
389
What is Type I & Type II error?
Type I error: Seeing something there when it's not (alpha accepts alternate) alpha is the probability of making Type I error We set significance at 0.05 to account for alpha error Type II error: Missing a difference that does actually exist beta is the probability of Type II error (beta blind to difference)
390
What is statistical power?
The probability of correctly rejecting the null hypothesis when there is a difference. ***Increases with larger sample (there is power in numbers) Power = 1 - beta "Alpha accepts, Beta blinds, Power perceives"
391
What is Z for a 95% confidence interval? | 99%?
95% CI --> Z = 1.96 | 99% CI --> Z = 2.58
392
What is the formula for confidence intervals?
It's a range from: | [mean - Z(SEM)] to [mean + Z(SEM)]
393
What does Pearson's correlation coefficient indicate?
Ranges from -1 to +1 Closer that it is to either -1 or +1 --> stronger the linear correlation of 2 variables
394
What is primary, secondary, and tertiary disease prevention?
Primary prevents Secondary screens Tertiary treats
395
With minors, when is parental consent not required?
Drugs, sex, and emergencies
396
What is assessed in Apgar scores?
Appearance, Pulse, Grimace, Activity, Respiration Assessed at 1 minute & 5 minutes. >7 = good >3 = assist and stimulate 3 or less = resuscitate
397
What is the cutoff for low birth weight?
Less than 2500g
398
When should children walk & talk? | Be potty trained?
Walk/talk at 1 200 words and 2 word phrases at 2 Pee at 3 (toilet training)
399
What are the sleep stages and associated waveforms?
EEG waveforms: "at night BATS Drink Blood" ``` Awake (eyes open) = Beta Awake (eyes closed) = Alpha Stage N1 = Theta Stage N2 = Sleep spindles & K complexes Stage N3 = Delta REM = Beta ``` Most of time spent in N2. Sleepwalking, night terrors, bedwetting is in N3.
400
How often does a REM cycle occur? | What is the predominant neurotransmitter?
REM occurs every 90 minutes ACh --> REM NE --> inhibits REM
401
How does the suprachiasmatic nucleus regulate melatonin release?
SCN --> NE release --> Pineal gland --> Melatonin release SCN is regulated by the environment (light)
402
What bases can be methylated?
Cytosine and Adenine
403
What amino acid may be methylated in histones? | Acetylated?
Methylation occurs at lysines & arginines --> histone more + --> heterochromatin Acetylation occurs at lysines --> euchromatin --> histone less + --> euchromatin
404
What base pairing is the strongest?
``` G-C = 3 H-bonds A-T = 2 H-bonds ```
405
What is the mechanism of Hydroxyurea?
Inhibits Ribonucleotide Reductase: UDP cannot --> dUDP Ultimately deprives cell of Thymidine
406
What is the mechanism of 5-Fluorouracil?
Inhibits Thymidylate Synthase (FU, TS): No dUMP --> dTMP Ultimately deprives cell of Thymidine (purine)
407
What are the Dihydrofolate Reductase inhibitors?
Methotrexate Trimethoprim (bacterial DHFR) Decrease availability of THF. N5,N10 THF required for dUMP --> dTMP (via thymidylate synthase)
408
What is the mechanism of 6-mercaptopurine?
Inhibits PRPP Amidotransferase (first step in dedicated purine synthesis) It is activated by HGPRT. Azathioprine is its prodrug.
409
What are the symptoms of orotic aciduria?
``` Megaloblastic anemia (not responsive to folate/B12) Orotic acid in the urine ```
410
What is the treatment for orotic aciduria?
Oral uridine supplementation
411
What does Adenosine Deaminase catalyze?
Adenosine --> Inosine This is part of the purine salvage pathway: Adenine --> AMP --> Adenosine --> Inosine --> Hypoxanthine --> IMP
412
How does ADA deficiency cause SCID?
1) Excess dATP feedback inhibits ribonucleotide reductase --> imbalance of nucleotide pool --> impaired DNA synthesis --> lymphocyte depletion 2) Toxic metabolites build up in lymphocytes
413
What defect is seen in Lesch-Nyhan Syndrome?
HGPRT deficiency. Normally it catalyzes: Hypoxanthine --> IMP Guanine --> GMP
414
What are the symptoms of Lesch-Nyhan Syndrome?
Mental retardation Self-mutilation Agression Hyperuricemia/gout
415
What are the common X-linked recessive disorders?
GOLD FOB Holds His Watch ``` G6PD deficiency OTC deficiency Lesch-Nyhan Duchenne's (& Becker's) muscular dystrophy Fabry's disease Ocular albinism Bruton's agammaglobulinemia Hemophilia A & B Hunter's syndrome Wiskott-Aldrich syndrome ```
416
What is seen in Purine Nucleoside Phosphorylase deficiency?
T cells are killed by buildup of dGTP (vs ADA deficiency which kills both B & T cells) Normal reactions: Guanosine --> Hypoxanthine Inosine --> Hypoxanthine
417
What are the types of mutations?
Silent --> same amino acid Missense --> different amino acid (conservative if similar AA) Nonsense --> early stop codon (UGA, UAA, UAG) Frameshift --> usually truncated & nonfunctional
418
What is the purpose of Nucleotide Excision repair?
Removes pyrimidine dimers & 6-4 photoproducts | bulky lesions that distort helix
419
What disease is caused by mutated nucleotide excision repair?
Xeroderma pigmentosum
420
What is the sequence in Base Excision Repair?
Glycosylases recognize damaged base --> create apurinic/apyrimidinic site --> endonuclease cuts 3' end --> lyase cuts 5' end --> DNAP-beta fills it in --> ligase seals it
421
What is Base Excision Repair used for?
``` Removes damaged bases: Deamination products (via nitrates or spontaneous) ```
422
What disease is associated with a mutation in Miscmatch Repair?
HNPCC ``` MLH1 = MutL MSH2 = MutS ``` **They find a mismatch, then find the nearest nick and recruit an endonuclease to degrade from the nick all the way to the mismatch and it is all replaced
423
What do the different RNAP synthesize?
RNAP I --> rRNA RNAP II --> mRNA RNAP III --> tRNA rampant, massive, tiny
424
What are the mRNA start and stop codons?
``` Start = AUG (met or f-met; inAUGural codon) Stop = UGA, UAA, UAG (U Go Away, U Are Away, U Are Gone) ```
425
How are "death cap mushrooms" toxic?
They contain alpha-amanitin --> hepatotoxic via inhibition of RNAP II
426
What is the signal for polyadenylation?
AAUAAA
427
How does anticodon sense relate to the codon?
An anticodon is reversed and complementary
428
Where do amino acids bind to the tRNA?
3' CCA
429
What ribosomes pertain to eukaryotes vs prokaryotes?
``` Eukaryotes = 40S + 60S --> 80S Prokaryotes = 30S + 50S --> 70S ```
430
What cell types are permanent cells?
Neurons skeletal muscle cardiac muscle
431
What cell types are stable (quiescent) cells?
Hepatocytes Lymphocytes Can be stimulated to enter G1 but normally reside in G0
432
What cell types are labile cells?
``` Bone marrow Gut epithelium Skin Hair follicles Germ cells ```
433
What signals proteins to traffic to lysosomes from the golgi?
Mannose-6-Phosphate
434
What causes I-cell disease?
Cells cannot add Mann-6-P to proteins intended for lysosomes. Instead the proteins are secreted into the plasma.
435
What is seen in I-cell disease?
Course facial features Clouded corneas Restricted joints High plasma levels of lysosomal enzymes
436
What are the various vesicular trafficking proteins and their purposed?
COPI = golgi --> ER (retrograde) COPII = ER --> goldi (anterograde Clathrin = trans-golgi --> lysosomes; PM --> endosomes (responsible for receptor-mediated endocytosis)
437
What are the functions of the peroxisome?
Catabolism of very long chain fatty acids (beta oxidation) | Catabolism of amino acids
438
What is the cause of Kartagener's Syndrome?
Dynein arm defect --> cilia dysfunction
439
What is seen in Kartagener's Syndrome?
Infertility (females have some but reduced) Bronchiectasis Recurrent sinusitis Often associated with situs inversus
440
What is Vimentin a stain for?
Connective tissue intermediate filaments
441
What is GFAP a stain for?
Neuroglial intermediate filaments
442
What is exchanged by the Na+/K+ ATPase?
Each ATP: 3 Na+ out 2 K+ in
443
What are the types of collagen & their location?
Type I = bONE, skin, tendon (90% of total) Type II = car-two-lage Type III = Reticulin (skin, blood vessels, uterus, fetal tissue) Type IV = Basement membrane (Type IV is the floor)
444
What causes osteogenesis imperfecta?
Autosomal dominant deficiency in Type I collagen
445
What is seen in Osteogenesis Imperfecta?
Multiple easy fractures Blue sclerae (choroidal veins seen through them) Early hearing loss Dental problems
446
What is seen in Ehlers-Danlos syndrome?
Hyperextensible skin Hypermobile joints Easy bruising Associated with joint dislocation, berry aneurysms, aortic dissection Caused by faulty COLLAGEN synthesis (vs. fibrillin for Marfan's)
447
What is seen in Alport syndrome?
Split renal basement membrane --> hereditary nephritis Deafness Ocular disturbances Due to abnormal Type IV collagen
448
What inhibits elastase?
alpha-1-Antitrypsin (A1AT)
449
What is the difference between direct & indirect ELISA?
Direct = tests for an Antigen in a patient's blood Indirect = tests for an Antibody in the patients blood
450
What is the Cre-lox system used for?
Targeted recombination or deletion of genes. Can be controlled by outside stimuli and targeted to cell types, etc. Useful if a global knockout mouse would die in-utero, etc.
451
What is variable expressivity?
Phenotype varies among individuals with the same genotype (i.e. 2 NF1 pts have varying severity)
452
What is pleiotropy?
One gene contributes to multiple (often seemingly unrelated) effects
453
What is loss of heteroygosity?
The two-hit hypothesis of tumor suppressor genes
454
What is linkage disequilibrium?
When 2 alleles occur together more often in a population that would be expected by chance
455
What is locus heterogeneity?
Mutations at different loci can produce the same phenotype
456
What is heteroplasmy?
Presence of both normal and mutated mitochondrial DNA --> variable expression of mitochondrial inherited disease
457
What is uniparental disomy?
Offspring gets 2 copies of a chromosome from 1 parent ``` Heterodisomy = meiosis 1 error Isodisomy = meiosis 2 error ``` Consider if a patient has an aut. recessive disease but only 1 parent is a carrier
458
What are the equations for Hardy-Weinberg?
p^2 + 2pq + q^2 = 1 and p + q = 1 Also 2pq = carrier frequency
459
What is the genetic basis for Prader-Willi & Angelman's Syndromes?
Prader-Willi: The deleted allele comes from the father or uniparental disomy of the maternal (imprinted) chromosome. Angelman's: The deleted allele comes from the mother or uniparental disomy of the paternal (imprinted) chromosome. They are the same genetic deletion (15q11-15) but are normally imprinted differently based on which parent they came from. This causes differing symptoms.
460
What is seen in Prader-Willi syndrome?
Mental retardation Hyperphagia/obesity Hypogonadism Hypotonia
461
What is seen in Angelman's syndrome?
Mental retardation Inappropriate laughter Seizures Ataxia
462
What is the pattern of X-linked recessive transmission on a pedigree?
Sons of carrier mothers have a 50% chance of showing disease.
463
How does an X-linked dominant disorder present on pedigree?
If mother carries the allele: 50% chance of all offspring getting it If father carries the allele: all daughters & no sons get it
464
What disorder is X-linked dominant?
Hypophosphatemic Rickets: | phosphate wasting at the proximal tubule --> rickets-like presentation that is resistant to Vit. D supplementation
465
What diseases are inherited via mitochondria?
Mitochondrial myopathies: Present with myopathy & CNS disease "Ragged red fibers" on muscle biopsy All children of affected mothers will be affected (heteroplasmy seen)
466
What is seen in Familial hypercholesterolemia?
``` Hyperlipidemia Type IIa: Autosomal dominant inheritance Homozygotes LDL = 700+ CVD early in life (often MI before 20) Tendon xanthomas ``` Heterozygotes LDL ~300
467
What is seen in Osler-Weber-Rendu syndrome?
(aka) Hereditary Hemorrhagic Telangiectasia: ``` Autosomal dominant inheritance Telangiectasia Recurrent epistaxis Skin discolorations AVM's ```
468
What chromosome is the huntingtin gene found on?
CAG repeats on chromosome 4 "Hunting 4 CAG's
469
What is seen in Marfan's syndrome?
``` Autosomal dominant fibrillin defect Affects skeleton, heart, eyes: Marfanoid habitus Hyperflexible joints Arachnodactyly Pectus excavatum Cystic medial necrosis of aorta --> aortic regurgitation & aortic dissection Mitral valve prolapse Subluxation of lens (ectopia lentis) ```
470
What chromosome is NF1 on?
Chromosome 17 von Recklinghausen = 17 letters
471
What is seen in Neurofibromatosis type 2? | What chromosome carries the NF2 gene?
Bilateral acoustic shwannomas juvenile cataracts On chromosome 22. Type 2, NF2 --> 22
472
What is the most common mutation in cystic fibrosis?
Deletion of Phe508 All CF stems from mutation of CFTR gene on chromosome 7
473
What is seen in cystic fibrosis?
Recurrent pneumonia (S. aureus, H. flu, & Pseudomonas) Pancreatic insuficiency (malabsorption & steatorrhea) Meconium ileus in newborns Bronchiectasis, chronic bronchitis Male infertility (absence of vas deferens) Cirrhosis (late) Nasal polyps Salty sweat
474
What is the pathogenesis of cystic fibrosis?
CFTR channel misfolded & degraded before getting to cell surface CFTR normally: - Secretes Cl- in lung, small intestine, & pancreas - Reabsorbs Cl- from sweat
475
What causes Duchenne's muscular dystrophy?
X-linked frameshift mutation --> deletion of dystrophin gene | Accelerated muscle breakdown is seen
476
What is seen in Duchenne's muscular dystrophy? | How is the diagnosis made?
``` Weakness beginning with pelvic girdle muscles (Progresses superiorly) Use of Gowers' maneuver Calf pseudohypertrophy Onset before 5y ``` Dx: ^ CPK & muscle biopsy
477
What is seen in Fragile X syndrome?
X-tra large testes, ears, jaw Autism Mitral valve prolapse
478
What causes Fragile X syndrome?
^CGG repeats in FMR1 gene It is X-linked & can be found in females but rarer
479
What is seen with fragile X premutation?
Females --> premature ovarian failure | Males --> tremor-ataxia in 40's-50's
480
What is seen in myotonic dystrophy?
Inability to release volitional contraction (aka unable to release handshake) Progressive weakness
481
What are the trinucleotide repeat disorders?
"Try Hunting for My Fried Eggs" ``` Trinucleotide disorders: Huntington's (CAG) Myotonic dystrophy (CTG) Friedrich's ataxia (GAA) Fragile X (CGG) ```
482
What is capable of inducing ARDS?
``` Trauma Sepsis Shock Aspiration Uremia DIC Hypersensitivity reactions Acute pancreatitis Amniotic fluid embolism ```
483
Rank the conduction speed of the various cardiac tissues
His-Purkinje > Atria > Ventricles > AV node
484
What disease are Auer rods seen in?
Acute promyelocytic leukemia (M3) t(15;17) "Hey (A)! 15 miles per Auer"
485
What occurs following ligand binding to a tyrosine kinase receptor?
Dimerization --> Cross-phosphorylation --> Intracellular active sites exposed --> phosphorylation of tyrosine residues on target proteins
486
What malignancies are seen with a germline mutated Rb gene?
Retinoblastoma Osteosarcoma *Requires knockout of other copy (2 hit hypothesis/loss of heterozygosity)
487
What are the mediators of the early-phase of an asthma attack?
Histamine | Leukotrienes C4, D4, E4
488
What is seen in Albright's Hereditary Osteodystrophy?
(aka) Pseudohypoparathyroidism: Autosomal dominant kidney unresponsiveness to PTH Symptoms: Hypocalcemia Short 4th/5th digits (knuckle, knuckle, dimple, dimple) Short stature
489
What is allelic heterogeneity?
Different mutations at the same locus cause similar variant phenotypes
490
What is seen in hemochromatosis?
``` "Bronze diabetes" Hepatomegaly --> Cirrhosis Diabetes Skin hyperpigmentation Testicular atrophy CHF ^ risk of hepatocellular carcinoma ```
491
What is the mutation in achondroplasia?
Activating mutation of FGFR-3 | chromosome 4
492
What type of signal sequence targets an mRNA to be translated bound to the ER?
Hydrophobic N-terminal signal sequences are recognized by signal recognition particles, which halt translation and carry the ribosome over to the RER to continue translation.
493
What are the CYP450 inhibitors?
MAGIC RACKS in GQ ``` Macrolides Amiodarone Grapefruit juice Isoniazid Cimetidine Ritonavir Acute alcohol Ciprofloxacin Ketoconazole Sulfonamides Gemfibrozil Quinidine ```
494
What are the CYP450 inducers?
"Carbemazepine By Pill Really Speeds Cyp450 Metabolism Greatly" ``` Carbemazipine Barbiturates Phenytoin Rifampin St. John's Wort Chronic alcohol Modafinil Griseofulvin ```
495
What part of the bone is generally affected in osteomalacia?
Unmineralized osteoid matrix surrounding the normally mineralized trabeculae
496
What causes Li-Fraumeni syndrome? | What is seen?
Germline p53 mutation 2nd hit causes: Early onset breast cancer Soft tissue sarcomas
497
Where do the recurrent laryngeal nerves loop around?
R. side loops around the R. subclavian | L. side loops around the aortic arch
498
Are efferent nerves myelinated or unmyelinated?
All are myelinated except for postganglionic autonomic neurons
499
Are afferent nerves myelinated or unmyelinated?
They are myelinated, with the exception of slow C fibers (heat & pain from free nerve endings).
500
What is the most common cause of aseptic meningitis?
Enteroviruses | poliovirus, coxsackie, echovirus, HepA
501
What occupies MHC molecules during their synthesis & processing?
MHCI --> beta-2-microglobulin | MHCII --> invariant chain
502
Who commonly gets molluscum contagiosum?
Children, sexually active adults, & immunodeficiency patients. It is a poxvirus
503
What is seen in the first trimester pregnancy screen for: Down syndrome? Edwards' syndrome?
Order goes alpha-fetoprotein, beta-hCG, estriol, inhibin-A Down: down-up-down-up Edwards: down-down-down-normal
504
What is seen on the FIRST-trimester pregnancy screen for Patau syndrome?
Decreased hCG Decreased PAPP-A Nuchal translucency
505
What is seen in Edwards' syndrome?
``` Trisomy 18: Severe mental retardation Rocker-bottom feet Low set ears Micrognothia (small jaw) Prominent occiput Clenched hands Congenital heart disease ``` Death usually occurs in <1y
506
What is seen in Patau syndrome?
``` Trisomy 13: Severe mental retardation Cleft lip/palate Microcephaly Holoprosencephaly Polydactyly Congenital heart disease ``` Death occurs in <1y Rocker-bottom feet
507
What chromosomes are at risk for Robertsonian translocations?
13, 14, 15, 21, 22 They are acrocentric chromosomes (1 large q arm and a tiny p arm) --> q arm is duplicated & p arm is lost --> normal if balanced, deletions/duplications if unbalanced
508
What causes cri-du-chat syndrome? | What is seen?
Microdeletion on 5p (short arm) ``` Symptoms: Mental retardation Crying/meowing (Cri-du-chat = cry of the cat) Epicanthal folds VSD Microcephaly ```
509
What causes Williams syndrome? | What is seen?
Microdeletion on long arm of chromosome 7 ``` Symptoms: Elfin facies Intellectual disability Vit. D sensitivity --> Hypercalcemia Extreme friendliness with strangers Cardiovascular problems (elastin is deleted) ```
510
What is seen in Reiter's syndrome?
``` aka Reactive arthritis Triad of: Conjunctivitis Urethritis Seronegative arthritis "Can't see, can't pee, can't climb a tree" ``` Associated with GU infection by: Chlamydia, Campylobacter, Salmonella, Shigella, or Yersinia HLA-B27
511
How does dermatitis herpetiformis present? | What is the cause?
Presents as pruritic lesions on the extensor surfaces | Celiac sprue --> IgA/IgG cross-react with reticulin in dermal papillary tips
512
What conditions are associated with acanthosis nigricans?
Insulin resistance | GI or lung malignancy
513
Where is eukaryotic DNA methylated? | Why?
At CpG islands. This causes genetic silencing of this region & is associated with imprinting, etc.
514
What sinuses are most commonly affected by URI?
Maxillary sinuses Because their drainage is superior, so gravity doesn't favor it.
515
What cancers can show mets to the left supraclavicular node?
Virchow's node drains the thoracic duct. Abdominal (GI) malignancies typically can show lymphatic dissemination to Virchow's node.
516
What lung pathogen is associated with blood clots?
Aspergillus
517
What does retinol do?
Antioxidant Constituent of visual pigments (retinal) Differentiates epithelial cells (ATRA for APL) Prevents SCC
518
What is seen in retinol deficiency?
Vitamin A deficiency --> Night blindness & dry skin
519
What is seen with hypervitaminosis A?
``` Teratogenic (cleft palate, cardiac abnormalities) Arthralgias Headache (cerebral edema) Alopecia Sore throat ```
520
What is B1 used for?
``` Thiamine --> TPP Used for decarboxylation reactions: Pyruvate dehydrogenase alpha-ketoglutarate dehydrogenase Transketolase Branched-chain amino acid dehydrogenase ```
521
What is seen in Thiamine deficiency?
1) Wernicke-Korsakoff syndrome - eyes, lies, capsize 2) Dry Ber1Ber1 - polyneuritis, symmetrical muscle wasting 3) Wet Ber1Ber1 - dilated cardiomyopathy, edema
522
What should be administered to an alcoholic with low glucose?
B1 FIRST, then glucose. Giving glucose in the setting of thiamine deficiency can worsen symptoms.
523
What is Vitamin B3 used for?
Niacin - part of NAD+ & NADP+ (B3 --> 3 ATP) Synthesized from tryptophan & requires B6
524
What is seen with B3 deficiency? | Excess?
Niacin deficiency --> Pellegra Pellegra (3 D's): Diarrhea Dermatitis (Casal's necklace) Dementia Excess --> facial flushing
525
What can result in B3 deficiency?
``` Niacin deficiency can result from: Hartnup disease Carcinoid syndrome (^ tryptophan metabolism) INH (can cause B6 deficiency) ```
526
What is Vit. B2 used for?
Riboflavin --> FAD & FMN (B2 --> 2 ATP)
527
What is seen in B2 deficiency?
Riboflavin deficiency (2 C's): Cheilosis (inflamed lips, fissures at the corners) Corneal vascularizaiton
528
What is Vit. B5 used for?
Pantothenate - a component of CoA & fatty acid synthase "Pentothenate" = B5
529
What is seen with B5 deficiency?
``` Pantothenate deficiency: Dermatitis Adrenal insufficiency Enteritis Alopecia ```
530
What is Vit. B6 used for?
Pyridoxine - converted to pyridoxal phosphate Used in: Transamination (ALT/AST) Decarboxylation Glycogen phosphorylase Synthesis of cystathionine, heme, niacin, histamine, serotonin, epinephrine, NE, GABA
531
What are the common causes of B6 deficiency? | What is seen?
``` Pyridoxine deficiency caused by - INH & OCP use Symptoms: Convulsions Hyper-irritability Peripheral neuropathy Sideroblastic anemia ```
532
What is Vit. B7 used for?
Biotin - cofactor for carboxylation enzymes: Pyruvate carboxylase Acetyl-CoA carboxylase Propionyl-CoA carboxylase
533
What is seen in B7 deficiency?
``` Biotin deficiency is rare (abx use or egg white over-ingestion) Symptoms: Dermatitis Enteritis Alopecia ```
534
What is the Vit. B9 used for?
Folic acid - converted to THF for methylation rxns in DNA/RNA synthesis Found in leafy green vegetables
535
What is seen in Vit. B9 deficiency?
Folic acid deficiency - seen in pregnancy & alcoholism Megaloblastic anemia Neural tube defects in pregnancy *Most common vitamin deficiency in the U.S.
536
What is B12 used for?
``` Cobalamin used for: Homocysteine methyltransferase (Homocysteine --> Methionine; also regenerates THF for thymidylate synthase) ``` Methylmalonyl-CoA mutase (Methylmalonyl-CoA (from odd-chain FA's) --> Succinyl-CoA)
537
What is seen in B12 deficiency? | What causes it?
Cobalamin deficiency: Megaloblastic anemia Hypersegmented PMN's Subacute combined degeneration (abnormal myelin) Caused by: Malabsorption Diphyllabothrium latum Lack of IF (pernicious anemia, gastric bypass) Lack of terminal ileum (Crohn's) Alcoholics Use Schilling test (radiolabeled B12) to determine cause
538
What is S-adenosyl methionine used for?
SAM (ATP + methionine) is used for conversion of NE --> EPI Requires Folic acid & B12 to regenerate Methionine (& thus SAM)
539
What is Vit. C used for?
Ascorbic acid: Antioxidant Aids in Fe2+ absorption Hydroxylation of proline & lysine in collagen synthesis Used in dopamine beta-hydroxylase (Dop --> NE)
540
What is seen in Vit. C deficiency?
``` Scurvy: Swollen gums Bruising Hemarthrosis Anemia Poor wound healing Weakened immune response ```
541
What is seen with Vit. C excess?
N/V Diarrhea Fatigue Iron toxicity
542
What are the forms of Vit. D and their uses?
D2 - ergocalciferol; ingested form plants D3 - cholecalciferol; ingested in milk & synthesized in skin 25-OH-D3 - storage form 1,25-OH-D3 - Calcitriol; active form
543
What is the function of Vit. E? | What is seen in deficiency?
Antioxidant for erythrocytes Deficiency --> hemolytic anemia & subacute combined degeration
544
What is the use of Vit. K?
K is for Koagulation Catalyzes gamma-carboxylation of glutamic acid residues on clotting proteins (II, VII, IX, X, C, S)
545
What is seen in Vit. K deficiency?
Hemorrhagic disease of the newborn (given shot @ birth) ^ PT & aPTT but normal bleeding time Can also be seen following prolonged use of broad-spectrum antibiotics (only acquired through intestinal flora synthesis)
546
What is seen in Zn deficiency?
Delayed wound healing Hypogonadism Anosmia/Dysgeusia
547
What is Fomepizole used for?
Fomepizole inhibits alcohol dehydrogenase - it is the antidote for methanol or ethylene glycol poisoning.
548
How does ethanol cause hypoglycemia?
It increases the NADH/NAD+ ratio in the liver --> inhibition of gluconeogenesis & ^ fatty acid synthesis (fatty liver).
549
What is Kwashiorkor? | Marasmus?
Kwashiorkor - lack of protein --> skin lesions, edema, fatty liver, anemia, protruberant abdomen, change in hair color Marasmus - lack of calories --> loss of subQ fat, loss of muscle, edema
550
What is the net ATP production by 1 molecule of glucose?
Aerobic (muscle; glycerol-3-P shuttle) - 30 ATP Aerobic (heart/liver; malate-aspartate shuttle) - 32 ATP Anaerobic glycolysis - 2 ATP
551
How are hexokinase & glucokinase different?
Hexokinase - ubiquitous, high affinity, low Vmax, inhibited by Glu-6-P Glucokinase - liver & pancreatic beta cells, low affinity, high Vmax, inhibited by Fru-6-P So at low [glu], sequestration in the tissues, and at high [glu], sequestration in the liver.
552
What is the net equation for glycolysis?
Glu + 2ADP + 2NAD+ --> 2pyruvate + 2ATP + 2NADH + 2H+ + 2H20
553
How do hormones affect Fructose-2,6-Bisphosphate levels?
Glucagon --> ^ cAMP --> ^ PKA --> phosphorylates to activate Fructose-2,6-Bisphosphatase --> less Fructose-2,6-BP --> inhibited PFK-1 --> inhibited glycolysis Insulin does the opposite, by decreasing cAMP --> eventual ^ in PFK-2 activity --> ^ Fru-2,6-BP --> ^ PFK-1 activity --> glycolysis
554
How does arsenic act as a poison? | What are the symptoms?
Arsenic inhibits lipoic acid --> inhibited functioning of pyruvate dehydrogenase Symptoms: Vomiting Rice water stools Garlic breath
555
What is the antidote to arsenic poisoning?
Dimercaprol It competes with pyruvate dehydrogenase thiols and binds up arsenic, which can then be excreted in the urine.
556
What is the treatment for pyruvate dehydrogenase complex deficiency?
Most mutations are X-linked --> neurologic defects from birth Treatment = ketogenic diet (fats, Lysine & Leucine)
557
What is the rate-limiting step in glycolysis? | How is it regulated?
Fructose-6-P --> Fructose-1,6-Bisphosphate (PFK-1) PFK-1 ^ by AMP & Fructose-2,6-Bisphosphate PFK-1 inhibited by ATP & Citrate
558
What steps in glycolysis require ATP?
Glucose --> Glucose-6-Phosphate (Hexokinase/Glucokinase) Fructose-6-Phosphate --> Fructose-1,6-Bisphosphate (PFK-1)
559
What steps in glycolysis liberate ATP?
1,3-Bisphosphoglycerate --> 3-Phosphoglycerate (2 ATP total) (Phosphoglycerate kinase) Phosphoenolpyruvate --> Pyruvate (2 ATP total) (Pyruvate kinase)
560
What are the possible fates of Pyruvate produced by glycolysis?
1) Pyruvate --> Alanine (ALT; Used to carry NH3 to the liver via the Alanine cycle) 2) Pyruvate + Co2 + ATP --> Oxaloacetate (Pyruvate carboxylase; Produces Oxaloacetate to replenish it in the TCA cycle or for gluconeogenesis) 3) Pyruvate --> Acetyl-CoA + NADH + CO2 (Pyruvate dehydrogenase; Transition from glycolysis to TCA cycle) 4) Pyruvate --> Lactate + NAD+ (LDH; The end of anaerobic glycolysis, regenerates NAD+; Lactate enters Cori cycle)
561
What tissues undergo anaerobic glycolysis as their major pathway?
``` RBC's Leukocytes Kidney medulla Lens Cornea Testes ```
562
What is produced by the TCA cycle?
``` Per 1 Acetyl-CoA: 3 NADH 1 FADH2 2 CO2 1 GTP ``` So 10 ATP total per Acetyl-CoA (x2 per glucose)
563
What are the intermediates of the TCA cycle?
Citrate Is Krebs' Starting Substrate For Making Oxaloacetate ``` Citrate Isocitrate alpha-Ketoglutarate Succinyl-CoA Succinate Fumarate Malate Oxaloacetate ```
564
What is cortisol's effect regarding vascular & bronchial smooth muscle?
Cortisol is permissive because it increases vascular & bronchial smooth muscle reactivity to catecholamines.
565
How can you identify the class of antiretroviral drug?
``` -navir = protease inhibitor Raltegravir = integrase inhibitor Enfuvertide = fusion inhibitor Nevirapine, Efavirenz, Delaverdine = NNRTI All others = NRTI ```
566
What is the mechanism of Triptan drugs?
Sumatriptan: Presynaptic 5-HT(1B/1D) agonist It inhibits functioning of trigeminal nerve & release of vasoactive peptide --> reduces neurogenic inflammation & causes vasoconstriction
567
Where is airway resistance the greatest?
Half of airway resistance comes from the upper airways (nose, pharynx) In the lower airways, resistance increases between the 2-5th bronchial generations due to turbulent flowbut then decreases due to ^ in cross-sectional area & laminar flow.
568
Describe the innervation of the larynx
Vagus nerve --> Superior laryngeal n. & recurrent laryngeal n. Superior laryngeal nerve: External branch --> motor to cricothyroid muscle Internal branch --> sensory above the vocal cords Recurrent laryngeal nerve --> motor to all other laryngeal muscles; sensory below the vocal cords
569
Describe the events of oxidative phosphorylation
Complex I: NADH is oxidized, 1 H+ is pumped (into intermembranous space) Complex II: FADH2 is oxidized, no H+ is pumped Complex III: 1 H+ is pumped Complex IV: 1 H+ is pumped Complex V: ATP synthase --> each proton allowed back into mitochondrial matrix = 1 ATP
570
What is the mechanism of Rotenone?
Inhibits ETC complex 1 (RotenONE) | Decreased proton gradient
571
What is the mechanism of cyanide?
Cyanide & CO inhibit Complex IV of the ETC | Decreased proton gradient
572
What is the mechanism of Antimycin A?
Inhibits complex III of the ETC | decreased proton gradient
573
What is the mechanism of Oligomycin?
Directly inhibits ATP synthase | ^ proton gradient
574
What are the irreversible enzymes in gluconeogenesis?
"Pathway Produces Fresh Glucose" Pyruvate carboxylase (mitochondria; Pyruvate --> Oxaloacetate) PEP Carboxykinase (cytosol; Oxaloacetate --> Phosphoenolpyruvate) Fructose-1,6-Bisphosphatase (cytosol) Glucose-6-Phosphatase (ER)
575
What are the primary gluconeogenic precursors?
``` Lactate Glycerol Alanine Glutamine (account for 90% of gluconeogenesis) ```
576
Where does gluconeogenesis occur?
Liver >> Kidney, Intestinal epithelium
577
What is the purpose of HMP shunt pathway?
The pentose phosphate pathway (HMP shunt) provides: Ribose-5-P for nucleotide synthesis (pentose) NADPH for reductive reactions (Phosphate) (used to reduce Glutathione, produces ROI for resp. burst)
578
What are the steps of the HMP shunt pathway?
Oxidative: Glu-6-P --> 2 NADPH + Ribulose-5-P (G6PD; Irreversible; Rate-limiting) Non-oxidative: Ribulose-5-P --> Ribose-5-P or Glycerol-3-P or Fructose-6-P (Phosphopentose isomerase transketolases; Reversible; Requires B1)
579
What are the steps involved in the synthesis of ROI's for respiratory burst?
1) O2 --> Superoxide (O2-) (NADPH Oxidase; Requires NADPH; Def. in CGD) 2) O2- --> H2O2 (Superoxide dismutase) 3) H2O2 + Cl- --> HOCl- (Myeloperoxidase; Produces hypochlorite/bleach) H202 can be inactivated by bacterial catalase or endogenous glutathione peroxidase
580
What are the common precipitants of hemolysis in a G6PD patient?
``` Infection Fava beans Sulfa drugs Primaquine Dapsone ```
581
What is seen on a blood smear in G6PD deficiency?
Heinz Bodies - Oxidized Hb | Bite cells - splenic macrophages take a bite out of RBC's to remove heinz bodies
582
What is the process of fructose metabolism to enter glycolysis?
Fructose --> Fructose-1-P (Fructokinase) Fructose-1-P --> DHAP + Glyceraldehyde (Aldolase B) Glyceraldehyde --> Glyceraldehyde-3-P (Triose kinase)
583
What is deficient in Essential fructosuria? | What is seen?
Aut. recessive defect in Fructokinase Fructose appears in blood & urine (Benign disease, as are both fructose disorders when compared to their galactose counterparts)
584
What causes Fructose intolerance? | What is seen?
Aut. recessive deficiency of Aldolase B Fructose-1-P accumulates, taking away phosphate groups from glycogenolysis & gluconeogenesis Symptoms: Hypoglycemia, jaundice, cirrhosis, vomiting
585
What is the treatment for fructose/galactose metabolism disorders?
Exclude from diet. Fructose --> exclude fructose & sucrose Galactose --> exclude galactose & lactose
586
What are the steps in the metabolism of galactose to glucose?
Galactose --> Gal-1-P (Galactokinase) Gal-1-P --> Glucose-1-P (Gal-1-P Uridyltransferase/GALT; Requires UDP-Glu from 4-epimerase)
587
What are the galactose metabolic disorders? | What is seen?
``` Galactokinase deficiency: Galactose in blood/urine Infantile cataracts (Galactose --> Galactitol shunt) ``` ``` Classic Galactosemia (GALT deficiency): Failure to thrive Jaundice E. coli sepsis Infantile cataracts Mental retardation Hepatomegaly ```
588
What is the polyol pathway? | What are its components?
It's an alternative method for trapping glucose within a cell (vs. hexokinase/glucokinase). Steps: Glucose --> Sorbitol (Aldose reductase) Sorbitol --> Fructose (Sorbitol dehydrogenase; Not present in Schwann, retina, kidney)
589
What tissues have Aldose reductase? | What tissues have insufficient Sorbitol dehydrogenase?
Both enzymes: Liver, lens, ovaries, seminal vesicles Insufficient Aldose Reductase: Retina, Schwann cells, kidneys
590
What does Aldose reductase catalyze?
Glucose --> Sorbitol | Galactose --> Galactitol
591
What are the essential amino acids?
PVT. TIM HALL ``` Phe Val Thr Trp Ile Met His Arg Leu Lys ``` Always Argues, never Tyres **His & Arg are semi-essential (required during growth)
592
What is the purpose of the cori cycle? | Alanine cycle?
Cori cycle: Converts lactate & pyruvate to glucose for export to tissues Alanine cycle: Alanine carries amino groups to liver
593
What is the function of ALT | AST?
ALT: Alanine + alpha-Ketoglutarate --> Pyruvate + Glutamate (Part of alanine cyle; Glutamate enters urea cycle; Pyruvate enters gluconeogenesis) AST: Glutamate + Oxaloacetate --> alpha-Ketoglutarate + Aspartate (alpha-Ketoglutarate can then be used in alanine or TCA cycle; Aspartate can enter urea cycle; Glutamate can also enter urea cycle directly through deamination)
594
What is the rate-limiting step in the urea cycle?
Carbamoyl phosphate synthetase I CO2 + NH4+ (+2ATP) --> Carbamoyl phosphate It's the first step
595
What are the intermediates in the urea cycle?
"Ordinarily, Careless Crappers Are Also Frivolous About Urination" ``` Ornithine +Carbamoyl phosphate Citrulline +Aspartate Arginosuccinate -Fumarate Arginine Urea ```
596
What is seen with ammonia intoxication? | What is the treatment?
Symptoms: Asterixis, slurred speech, somnolence, vomiting, cerebral edema, blurred vision Treament: Lactulose - acidifies GI lumen --> ^ excretion of NH4+ Limit dietary protein Benzoate/Phenylbutyrate --> bind AA's --> ^ excretion
597
``` What amino acids are these derived from? Dopamine/NE/EPI Serotonin/Melatonin Heme Histamine Creatine Urea NO GABA Glutathione ```
``` Dopamine/NE/EPI = Phenylalanine (converted to Tyr first) Serotonin/Melatonin = Tryptophan Heme = Glycine Histamine = Histidine Creatine/Urea/NO = Arginine GABA/Glutathione = Glutamate ```
598
What are the steps & enzymes involved in catecholamine synthesis?
Phenylalanine --> Tyrosine (Phenylalanine hydroxylase; requires tetrahydrobiopterin) Tyrosine --> DOPA (dihydroxyphenylalanine) (Tyrosine hydroxylase; requires tetrahydrobiopterin) DOPA --> Dopamine (Dopa decarboxylase; requires B6) Dopamine --> NE (Dopamine beta-hydroxylase; requires Vit. C) NE --> EPI (Phenylethanolamine N-methyltransferase; requires SAM)
599
What causes PKU? | What is seen in PKU?
Phenylalanine hydroxylase (Phe --> Tyr) deficiency or tetrahydrobiopterin (cofactor; 2% of cases) deficiency. ``` Symptoms: Mental retardation Growth retardation Fair skin Eczema Musty/Mousy body odor Phenylacetate/Phenyllactate/Phenylpyruvate in urine ```
600
What is the treatment for PKU?
``` Avoid phenylalanine (artificial sweeteners) Increase Tyrosine intake ```
601
What is the danger of maternal PKU?
If uncontrolled during pregnancy, child will be affected (mental/growth retardation, cardiac abn) despite the child being a heterozygote.
602
What causes Onchronosis? | What is seen?
Alkaptonuria = a congenital deficiency of homogentistic acid oxidase (Tyr --> Fumarate to degrade Tyr). Symptoms: Dark connective tissue (ears, nose, cheeks) & sclera Urine turns black when exposed to air Severe arthralgias
603
What causes albinism?
Ocular albinism = X-linked Generalized albinism = varies Defect is either in tyrosinase (Tyr --> melanin) or in Tyrosine transporters (decreased Tyr available). Can also result from a lack of neural crest cell migration.
604
What causes homocysteinuria? | What is the treatment?
Three forms: 1) Cystathione synthase deficiency Tx: Decreased Met in diet & ^ Cysteine, B12, Folate 2) Decreased affinity of cystathione synthase for B6 Tx: ^^ dietary B6 3) Homocysteine methyltransferase deficiency
605
What is seen in Homocystinuria?
``` ^^ Homocysteine in urine Cysteine is essential Mental retardation Osteoporosis Tall stature Kyphosis Lens subluxation Stroke/MI ```
606
What causes Cystinuria? What is seen? How is it treated?
Hereditary defect in renal reabsorption of COAL (Cysteine, Ornithine, Arginine, Lysine) --> Staghorn calculi Think of this if recurrent kidney stones seen in a young pt. Tx: Good hydration & urinary alkalinization
607
What causes Maple Syrup Urine Disease? | What is seen?
Congenital deficiency of alpha-ketoacid dehydrogenase --> no degradation of branched amino acids. "I Love Vermont maple syrup" (Ile, Leu, Val) Symptoms: CNS defects/Mental retardation Death Urine smells like burned sugar
608
How is Cistinuria diagnosed?
1) Hexagonal crystals in urine | 2) Sodium Cyanide-Nitroprusside test (detects sulfhydryl groups; purple --> + result)
609
What causes Hartnup disease? | What is seen?
Hereditary disorder preventing neutral amino acid transport in the kidney & intestine --> Tryptophan not absorbed in gut & ^ excretion in urine --> Pellegra (Niacin deficiency).
610
How is glyogenolysis stimulated/inhibited in muscle?
Glucagon (live only) or EPI (liver & muscle --> ^ cAMP --> PKA --> activated Glycogen phosphorylase kinase --> Activated Glycogen phosporylase Insulin --> Receptor tyrosine kinase --> Protein phosphatase --> inactive Glycogen phosphorylase **Ca2+/Calmodulin can also activate Glycogen phosphorylase kinase to coordinate glycogenolysis in active muscle
611
What type of bonding is seen in glycogen?
alpha (1,6) = straight | alpha (1,4) = branch
612
What are the names of the glycogen storage diseases?
"Viagra Pills Cause A Massive Hard-on" ``` Type I = von Gierke's II = Pompe's III = Cori's IV = Anderson's V = McArdle's VI = Her's ```
613
What causes von Gierke's disease? | How does it present?
Glucose-6-Phosphatase deficiency ``` Symptoms: Severe fasting hypoglycemia ^^ Liver glycogen --> Hepatomegaly ^ Blood lactate Fat cheeks, thin extremities ```
614
What causes Pompe's disease?
Lysosomal alpha-1,4-glucosidase deficiency Symptoms: Cardiomegaly (Pompe's trashes the pump) Systemic findings Early death
615
What causes Cori's disease? | What is seen?
Debranching enzyme (alpha-1,6-glucosidase) deficiency Symptoms: Milder form of Type I (gluconeogenesis intact) Hepatomegaly Fasting hypoglycemia
616
What causes McArdle's disease? | What is seen?
Skeletal muscle glycogen phosphorylase deficiency Symptoms: Painful muscle cramps & myoglobinuria with exercise Often presents in early 20's
617
Which LSD's are associated with Ashkenazi Jews?
Tay-Sachs Niemann Pick Gaucher's
618
Which LSD's are X-linked recessive?
Fabry's Hunter's All others are autosomal recessive
619
What causes Fabry's disease? | How does it present?
alpha-galactosidase A deficiency --> ceramide trihexoside accumulation Symptoms: Glove & stocking neuropathy Angiokeratomas Cardiovascular/renal disease
620
What causes Gaucher's disease? | What is seen?
Glucocerebrosidase deficiency --> glucocerebrosidase accum. ``` Symptoms: HSM Aseptic necrosis of femur Bone crises Gaucher's cells (crumpled macrophages) Thrombocytopenia & anemia ```
621
What causes Niemann-Pick disease? | What is seen?
Sphingomyelinase deficiency --> Spingomyelin accum. ``` Symptoms: HSM Progressive neurodegeneration/Developmental delay Cherry red spot on macula Foam cells ```
622
What causes Tay-Sach's disease? | What is seen?
Hexosaminidase A deficiency (Tay-SaX) --> GM2 ganglioside accum. ``` Symptoms: Progressive degeneration/Developmental delay Cherry red spot on macula Lysosomes with onion skin appearance NO HSM (vs. Niemann-Pick) ```
623
What causes Krabbe's disease? | What is seen?
Galactocerebrosidase deficiency ``` Symptoms: Peripheral neuropathy Developmental delay Optic atrophy Globoid cells ```
624
What causes Metachromatic Leukodystrophy disease? | What is seen?
Arylsulfatase A deficiency --> Cerebroside sulfate accum. Symptoms: Central & peripheral demyelination Ataxia Dementia
625
What causes Hurler's syndrome? | What is seen?
alpha-L-iduronidase deficiency --> Heparin & Dermatin sulfat accum. ``` Symptoms: Developmental delay Gargoylism Airway obstruction Corneal clouding HSM Gibbus deformity ```
626
What causes Hunter's syndrome? | What is seen?
Iduronate sulfase deficiency --> Heparin & Dermatin sulfate accum. Symptoms: Mild Hurler's dz but aggressive behavior & no corneal clouding Hunters are aggressive men & must see clearly
627
What intermediate is used to synthesize fatty acids?
Citrate --> Acetyl-CoA --> Malonyl-CoA --> Fatty acid
628
How does Carnitine deficiency present?
Inability to transport long chain FA's into the mitochondria for beta oxidation --> Weakness, hypoketotic hypoglycemia
629
What are the ketone bodies? | What are they formed from?
Acetoacetate Acetone beta-Hydroxybutyrate They are formed from the oxidation of FA's --> Acetyl-CoA. When TCA intermediates are depleted (Oxaloacetate) --> Ketogenesis instead
630
When are ketone bodies seen?
Prolonged starvation Alcoholics (Oxaloacetate is depleted due to --> to malate) DKA (Oxaloacetate is depleted from gluconeogenesis)
631
What is the progression of metabolic fuel usage during exercise?
ATP Creatinine (seconds) Anaerobic glycolysis (minutes-1h) Aerobic metabolism & FA oxidation (surpasses anaerobic glycolysis @ 1h)
632
How does Aspirin overdose cause fever?
Uncouples ETC --> electron transport continues but no ATP is produced --> Heat
633
``` What is the function of the following apolipoproteins? E A-I C-II B-48 B-100 ```
``` E --> mediates remnant uptake A-I --> Activates LCAT C-II --> Lipoprotein lipase cofactor B-48 --> Mediates chylomicron secretion B-100 --> Binds LDL receptor ```
634
What are the functions of the various Lipoproteins?
Chylomicron - Delivers dietary TG's to peripheral tissue & cholesterol to the liver. VLDL - Secreted by liver to deliver hepatic TG's to peripheral tissue IDL - Degradation product of VLDL; returns to liver LDL - Delivers hepatic cholesterol to peripheral tissues; taken up into target tissues via LDLR; formed via hepatic lipase modification of IDL HDL - Reverse cholesterol transport from periphery to liver; acts as repository for ApoC & ApoE; secreted by liver & intestine
635
What causes Type I familial dyslipidemia? | What is seen?
Lipoprotein lipase deficiency or altered C-II --> ^ chylomicrons, TG, cholesterol ``` Symptoms/Risks: Pancreatitis HSM Xanthomas No ^ risk for atherosclerosis ```
636
What causes Type IIa familial dyslipidemia? | What is seen?
Autosomal dominant. Absent or reduced LDLR --> ^^LDL, cholesterol Symptoms: Early atherosclerosis Achilles xanthomas Corneal arcus (blue ring @ outside of iris)
637
What causes Type IV familial dyslipidemia? | What is seen?
Autosomal dominant. Hepatic overproduction of VLDL. Causes pancreatitis
638
What causes Abetalipoproteinemia?
Aut recessive mutation in Microsomal TRG Transfer Protein (MTP) gene --> reduced B-48 & B-100 --> reduced chylomicron & VLDL synthesis
639
What are the symptoms of abetalipoproteinemia?
``` Seen in the first few months of life Failure to thrive Steatorrhea Acanthocytosis (spur cells; Vit. E) Ataxia Night blindness (Vit. A) ``` Intestinal biopsy - Lipid accumulation within enterocytes
640
What is seen with TCA overdose?
TCCAA ``` Temperature (hyperpyrexia) Convulsions Coma Arrhythmia Apnea ```
641
What type of foods often contain S. aureus food poisoning (pre-formed toxin)?
Picnic foods, especially containing mayonnaise.
642
How do eosinophils function in parasitic infections?
Their IgE-specific Fc receptors bind IgE on opsonized parasites & they degranulate (antibody-dependent cytotoxicity)
643
What types of drugs are typically hepatically eliminated?
Lipophilic drugs
644
Anti-CCP Antibodies
Anti-cyclic citrullinated peptide Ab's --> Rheumatoid arthritis
645
What signaling molecules use the JAK/STAT pathway?
Colony-simulating factors Prolactin Growth hormone
646
What is the most rapidly metabolized sugar via glycolysis?
Fructose This is because it enters the glycolytic pathway after the rate-limiting step of glycolysis (PFK-1)
647
Which penicillins are anti-pseudomonal? Cephalosporins? Aminoglycosides? Fluoroquinolones?
``` Pen = Ticarcillin, Piperacillin Ceph = Ceftazidime, Cefepime Aminogly = Tobramycin, Gentamycin, Amikacin FQ = Ciprofloxacin, Levofloxacin ``` Also Aztreonam, Imipenem, Meropenem
648
What is provided in chocolate agar?
Chocolate agar is required for H. influenzae culture. It provides: Factor V = hematin Factor X = NAD+
649
What is the urinary metabolite for serotonin?
5-HIAA | 5-Hydroxyindoleaceticacid
650
What anti-mycobacterial agents work on intracellular bacteria?
Pyrazinamide - it works best at acidic environments like within a phagosome The others (RIES) work on extracellular bacteria best.
651
What other abnormalities are commonly seen alongside imperforate anus?
Genitourinary abnormalities
652
What is seen in myeloperoxidase deficiency?
Candida infections
653
What is observed with apoptosis?
``` Pyknosis (shrinkage) Karyorrhexis (fragmentation) --> DNA laddering (180 bp intervals) Karyolysis (destruction of chromatin) Cell shrinkage Apoptotic body formation ```
654
What induces intrinsic apoptosis? | Extrinsic?
Intrinsic: Withdrawal of growth factor Injury (radiation, hypoxia) Extrinsic: FasL binding to Fas receptor Granzyme B (enters via perforin; CTL's)
655
Where does liquefactive necrosis occur? Fat necrosis? Fibrinoid necrosis? Gangrenous necrosis?
Liquefactive = brain, bacterial abscesses, pancreas Fat necrosis = peripancreatic fat, breast Fibrinoid necrosis = blood vessels (malignant HTN, vasculitis) Gangrenous necrosis = extremities & GI (Can be dry (mummy) or wet (bacterial infection)
656
What type of calcification is seen with normal Ca2+ levels?
Dystrophic calcification = normal serum [Ca2+] Metastatic calcification = ^^ [Ca2+]
657
What is seen with irreversible cell damage? | Reversible?
``` Irreversible: Disruption of 3 membranes -Plasma membrane -Mitochondrial membrane (Cyt. C) - Lysosomal membrane ``` Reversible: Cell swelling (due to reduced ATP --> reduced pump action) Ribosomal detachment Fatty change
658
What cells are affected in Hypoxic Ischemic Encephalopathy?
HIE affects: - Pyramidal cells of hippocampus - Purkinje cells of cerebellum
659
What are the cell-mediators of the cardinal signs of inflammation?
``` Calor/Rubor = Histamine, Prostaglandins, Bradykinin Tumor = Histamine, Tissue damage Dolor = Bradykinin & PGE2 ```
660
Where does leukocyte extravasation occur?
The post-capillary venules This is the site of vascular permeability
661
What are the steps of leukocyte extravasation & their mediators?
1) Margination (occurs due to vasodilation) 2) Rolling (P/E-selectin binds Sialyl-Lewis) 3) Adhesion (ICAM-1 binds LFA-1 = integrin) 4) Diapedesis (PECAM-1 binds itself) 5) Migration (CILK: C5a, IL-8, LTB4, Kallikrein)
662
What is released from Weibel-Palade bodies?
von Willebrand Factor | P-selectin
663
What are the stages of wound healing?
1) Inflammatory (immediate) 2) Proliferative (2 days; granulation tissue, collagen (III), contraction by myofibroblasts) 3) Remodeling (1 week; Type III collagen --> Type I)
664
What dietary deficiencies can slow wound healing?
Ascorbic acid - required for collagen hydroxylation --> crosslinks Copper - crosslinking enzyme requires it Zinc - collagenase requires it
665
What diseases cause granuloma formation?
``` MTB Fungal infections Syphilis Leprosy Bartonella hensalae (cat scratch disease) Sarcoidosis Crohn's disease Wegener's Churg-Strauss Berylliosis/silicosis ```
666
What causes exudative fluid?
Either inflammation or lymphatic obstruction
667
What is the specific gravity of transudate vs. exudate?
Transudate < 1.012 | Exudate > 1.020
668
What conditions can lower ESR?
Sickle cell Polycythemia CHF
669
What stain is used to detect amyloidosis?
Congo red stain Lights up with "apple green birefringence" under polarized light
670
What are the systemic amyloidoses and their causes
AL (primary) amyloisosis: Plasma cell dyscrasia or Multiple myeloma --> Ig light chain deposition AA (secondary) amyloisosis: Chronic inflammatory dz (RA, IBD, chronic infx), cancer, Familial Mediterranean fever --> Amyloid A (acute phase reactant)
671
What is seen with systemic amyloidosis?
``` Nephrotic syndrome Restrictive cardiomyopathy Easy bruising Hepatomegaly Neuropathy ```
672
What causes dialysis-related amyloidosis? | What is seen?
Beta-2-microglobulin deposits in joints --> carpal tunnel & joint issues
673
What causes Heritable amyloidosis? | What is seen?
MUTATED Transthyretin deposition in heart & brain
674
What causes Alzheimer's disease?
Deposition of beta-amyloid (from amyloid precursor protein) in the brain *APP is located on chromosome 21 (why Down's causes early-onset Alzheimer's)
675
By what means do cancers tend to metastasize?
``` Carcinomas = lymphatics Exceptions: -Renal cell carcinoma -HCC -Follicular thyroid carcinoma -Choriocarcinoma ``` Sarcomas = hematogenous
676
How can clonality of a possible neoplasia be determined?
Females - G6PD expression (alleles should be 1:1) | B-cell tumors = Ig light chain (normal k:L = 3:1)
677
What -plasia's are reversible & irreversible?
Reversible = Hyperplasia, Metaplasia, Dysplasia Irreversible = Anaplasia, Neoplasia, Desmoplasia
678
What cancers is ionizing radiation associated with?
Leukemias Papillary thyroid cancer Breast cancer
679
``` What are the characteristic intermediate filaments in the following tissues? Mesenchyme Neuroglia Muscle Neurons Epithelium ```
``` Mesenchyme = Vimentin Neuroglia = GFAP Muscle = Desmin Neurons = Neurofilaments Epithelium = Keratin ```
680
What is chromogranin a tumor marker for?
Neuroendocrine tumors
681
What cancers stem from myc dysfunction?
myc = a transcription factor c-myc --> Burkitt's lymphoma L-myc --> small cell Lung carcinoma N-myc --> Neuroblastoma
682
What cancer is c-kit found in?
GIST
683
What tumor is BRAF mutated in?
Melanoma
684
What do the Rb and p53 genes do?
Rb: Inhibits E2F G1-->S tumor suppressor p53: Transcription factor for p21 G1-->S tumor suppressor
685
What is CEA a tumor marker for?
Colorectal & Pancreatic carcinoma *Very nonspecific
686
What is AFP a tumor marker for?
Hepatocellular carcinoma | Yolk sac tumors
687
What is CA-125 a tumor marker for?
Ovarian cancer
688
What is S-100 a tumor marker for?
Melanoma Neural tumors Schwannoma
689
What is Alkaline phosphatase a tumor marker for?
Mets to bone | Paget's dz of the bone
690
What is Bombesin a tumor marker for?
Neuroblastoma | Lung/gastric cancer
691
What is TRAP a tumor marker for?
Tartrate-Resistant Acid Phosphatase | Hairy cell leukemia TRAP the hairy animal
692
What is CA-19-9 a tumor marker for?
``` Pancreatic adenocarcinoma (19-9 = 10 = the # of letters in "pancreatic") ```
693
What types of neoplasms can EBV cause?
Burkitt's lymphoma Hodgkin's lymphoma Nasopharyngeal carcinoma CNS lymphoma (immunocompromised pts)
694
What type of cancer can aflatoxins cause?
HCC
695
What type of cancer can vinyl chloride cause?
Liver angiosarcoma
696
What type of cancer can nitrosamines cause?
(smoked foods) --> gastric adenocarcinoma (intestinal type)
697
What type of cancers can cigarette use cause?
``` Mouth/Esophageal/larynx Lung Pancreas Kidney Bladder ```
698
What type of cancer(s) can arsenic cause?
SCC (skin; like the women who used it to lighten their skin) | Liver angiosarcoma
699
What type of cancer(s) can naphthalene cause?
Aniline dyes --> Transitional cell carcinoma (bladder)
700
What types of cancers are associated with alcohol?
SCC of the mouth & esophagus HCC (via cirrhosis) Pancreatic (not a risk factor; but chronic pancreatitis is)
701
What type of cancer is associated with the following paraneoplastic syndrome? ACTH
Clinically --> Cushing's syndrome Small cell lung carcinoma
702
What type of cancer is associated with the following paraneoplastic syndrome? ADH
SIADH Small cell lung carcinoma Intracranial neoplasms
703
What type of cancer is associated with the following paraneoplastic syndrome? PTHrp
Clinically --> Hypercalcemia Squamous cell lung cancer Renal cell cancer Breast cancer *The most common cause of clinically apparent hypercalcemia = cancer
704
What type of cancer is associated with the following paraneoplastic syndrome? Calcitriol
Clinically --> Hypercalcemia Lymphoma
705
What type of cancer is associated with the following paraneoplastic syndrome? EPO
Clinically --> Polycythemia Renal cell carcinoma Hemangioblastoma HCC Pheochromocytoma
706
What cancers are psamomma bodies seen in?
``` PSaMMoma: Papillary thyroid Serous cystadenocarcinoma Meningioma Mesothelioma ```
707
What are the leading cancers in incidence? | Deaths?
``` Incidence = Breast/Prostate, Lung, CRC Deaths = Lung, Breast/Prostate, CRC ``` Cancer is the 2nd leading cause of death in the U.S.
708
What mets go to brain?
Lung > Breast > GU > Osteosarcoma > Melanoma > GI 50% of brain cancer is mets (usually at gray/white junction)
709
What metastasizes to the liver?
CRC >> stomach > pancreas
710
What metastasized to the bone?
Prostate = Breast > lung > thyroid = testes
711
What families do each of the viral hepatitides belong to?
``` HAV = Picornavirus HBV = Hepadnavirus HCV = Flavivirus HDV = Deltavirus HEV = Hepevirus ```
712
What is the cause of propionic acidemia?
Propionyl-CoA carboxylase deficiency Normally Valine or Isoleucine --> Propionyl-CoA (branched chain alpha-ketoacid dehydrogenase) then Propionyl-CoA --> Methylmalonyl-CoA (propionyl-CoA carboxylase) then Methylmalonyl-CoA --> Succinyl-CoA (methylmalonyl-CoA isomerase) --> TCA cycle.
713
What are the endoscopic & microscopic findings for Candida, HSV-1, & CMV esophagitis?
Candida: grey/white pseudomembrane patches Yeast & pseudohyphae HSV-1: Punched out ulcers Molding, marginated, multinuclear w/ intranuclear inclusions CMV: Linear ulceration Both intranuclear & cytoplasmic inclusions
714
What does the Prussian blue stain detect?
Intracellular iron
715
What causes hemosiderin-laden macrophages in the alveoli?
These are heart failure cells Caused by left CHF --> ^ hydrostatic pressure in pulmonary capillaries --> RBC/heme leakage --> ingestion by macs
716
What is the first line drug for absence seizures? | What is the mechanism?
Ethosuximide It blocks T-type Ca2+ channels in the thalamus
717
What anticonvulsants prolong Na+ channel recovery?
Phenytoin Carbamazepine Valproic acid
718
How does botulism commonly present?
Diplopia, dysphagia, dysphonia within 12-46h of ingestion. Can be caused by: - Improperly canned food - Wound infection - Honey (infantile botulism)
719
What is seen in normal cardiac aging?
``` Decreased apex-to-base dimensions Sigmoid shaped interventricular septum Myocyte atrophy with interstitial fibrosis Lipofuscin accumulation (byproducts of lipid metabolism) ```
720
What cell surface markers are absent in PNH?
CD55 & CD59
721
What is recommended regarding GBS and pregnancy?
Vaginal/rectal culture at 35-37 weeks. If positive --> intrapartum ampicillin (or penicillin)
722
What anti-herpesvirus drugs require activation by viral enzymes?
Acyclovir- requires activation by HSV/VZV viral thymidine kinase Gancyclovir - requires activation by CMV viral kinase Cidofovir - does not require any activation *All are viral DNA polymerase inhibitors
723
How does Ondansetron work?
It's a 5-HT3 antagonist: 1) Blocks vagal nerve stimulation of the medulla oblongata 2) Blocks central chemoreceptor trigger zone
724
What is Km? | What determines Vmax?
Km = the concentration needed for 50% enzyme saturation (inversely related to affinity) Vmax is directly related to enzyme concentration
725
Lineweaver-Burk plots: What determines the X intercept? Y intercept? Slope?
X intercept = 1/(-Km) Y intercept = 1/Vmax Slope = Km/Vmax
726
What kinetics variable do competitive inhibitors affect? | Noncompetitive inhibitors?
Competitive inhibitors increase Km --> decrease potency Noncompetitive inhibitors decrease Vmax --> decrease efficacy
727
What is the equation for bioavailability?
F = (AUCoral)/(AUCiv)*100
728
What is the equation for volume of distribution?
Vd = Amount of drug in the body/plasma drug concentration It is the theoretical volume of fluid needed to maintain a constant dose at a constant plasma concentration.
729
What types of drugs have low vs high volumes of distribution?
Low Vd: Large/charged molecules Plasma protein bound High Vd: Lipophilic molecules Tissue protein bound
730
What is the equation for half life?
HL = (0.7*Vd)/CL *A property specific to first-order kinetics (constant % of drug eliminated)
731
What is the equation for drug clearance?
``` CL = (rate of elimination of the drug)/(plasma drug concentration) CL = Vd*Ke --------where Ke is the elimination constant ``` It's given in units of flow
732
How quickly do constant infusions of drugs reach steady state?
Takes 4-5 half lives Steady state is a function of half-life, therefore dosing frequency, size, loading doses, etc. have no effect on the time it takes to reach steady state
733
What is the equation for drug loading dose? | Maintenance dose?
LD = Target plasma concentration*(Vd/F) MD = Target plasma concentration*(CL/F) Renal or liver disease --> lower maintenance dose (only)
734
What drugs have zero-order elimination?
PEA (round like 0): Phenytoin Ethanol Aspirin
735
How can urine pH affect renal elimination of a drug?
Ionized form cannot traverse membranes: Weak acids are trapped in alkaline urine (give IV HCO3) Weak bases are trapped in acidic urine (give IV NH4Cl)
736
What occurs during Phase I & Phase II drug metabolism?
Phase I: REDOX, CYP450 --> slightly polar metabolites (often still active) Phase II: GAGS: Glucuronidation, Acetylation, Glutathione, Sulfation Usually yields inactive, very polar metabolites for urine excretion
737
What phase of drug metabolism decreases first with age?
Phase I is affected first
738
What is the equation for therapeutic index? | Therapeutic window?
TI = LD50/ED50 TW = min. effective dose - min. toxic dose
739
What sympathetic structures are innervated by ACh?
Sweat glands Adrenal medulla All others are preganglionic ACh & postganglionic NE or Dop.
740
What autonomic drugs act on what G-proteins & second messengers?
HAV 1 M&M = H1, a1, V1, M1, M3 --> Gq MAD 2's = M2, a2, D2 --> Gi All others = Gs
741
What are the sequences of the autonomic second messenger systems?
Gq --> Phospholipase C (cleaves PIP2) --> DAG & IP3 DAG --> PKC IP3 --> Ca2+ influx --> smooth muscle contraction Gi -/-> Adenylyl cyclase Gs --> Adenylyl cyclase --> ^ cAMP --> PKA --> --> ^Ca2+ (in heart) or ^MLCK activity
742
What do alpha adrenergic receptors control?
Alpha1: Vascular smooth muscle contraction Mydriasis Intestinal & bladder sphincter contraction ``` Alpha2: Lowers central sympathetic outflow Lowers insulin release Lowers lipolysis Causes platelet aggregation ```
743
Where do muscarinic receptors act?
``` M1 = CNS, enteric nervous system M2 = decreases HR & contractility of atria M3 = exocrine glad secretions, gut peristalsis, bladder contraction, bronchoconstriction, miosis, accommodation ```
744
Where do dopamine receptors act?
D1 = relaxes renal vascular smooth muscle | D2 modulates NT release in the brain
745
Where do histamine receptors act in the body?
H1 = nasal & bronchial mucus production, bronchiolar constriction, pruritis, pain H2 = gastric acid secretion
746
Where does vasopressin act in the body?
``` V1 = vascular smooth muscle contraction V2 = water reabsorption in the kidney ```
747
What is the mechanism of Bethanechol? | What is it used for?
Cholinomimetic used to activate bladder & bowels. | postop ileus, neurogenic ileus, urinary retention
748
What is the mechanism of Carbachol? | What is it used for?
Cholinomimetic used for glaucoma, pupillary contraction.
749
What is the mechanism of Pilocarpine? | What is it used for?
Cholinomimetic that stimulates sweat, tears, saliva. Used for open-angle and closed-angle glaucoma. "You cry, drool, and sweat on your pilo (pillow)"
750
What is the mechanism of Methacholine? | What is it used for?
It is a cholinomimetic used in the challenge test to diagnose asthma.
751
What is the mechanism of Neostigmine? | What is it used for?
It is an anticholinesterase inhibitor used for: Postop/neurogenic ileus & urinary retention Myasthenia gravis Postop reversal of neuromuscular junction blockade *Neo (no) CNS penetration
752
What is the mechanism of Pyridostigmine? | What is it used for?
It is a long acting anticholinesterase inhibitor used for myasthenia gravis treatment *It does not cross the BBB
753
What is the mechanism of Edrophonium? | What is it used for?
It is a short-acting acetylcholinesterase inhibitor used to diagnose myasthenia gravis.
754
What is the mechanism of Physostigmine? | What is it used for?
It is a acetylcholineesterase inhibitor used to treat anticholinergic overdose (crosses the BBB). "Physostigmine phyxes atropine overdose"
755
What is the mechanism of Donepezil? | What is it used for?
It is an acetylcholinesterase inhibitor used to treat Alzheimer's disease.
756
What is seen with cholinesterase inhibitor poisoning?
Due to compounds that irreversible bind AChE (organophosphates) ``` DUMBBELSS: Diarrhea Urination Miosis Bronchospasm Bradycardia Excitation of skeletal muscle & CNS Lacrimation Sweating Salivation ```
757
What is the mechanism of Benztropine? | What is it used for?
Muscarinic antagonist used for Parkinson's disease. "Park my Benz"
758
What is the mechanism of Scopalamine? | What is it used for?
Anti-muscarinic used for motion sickness.
759
What is the mechanism of Ipratropium? | What is it used for?
Ipratropium/Tiotropium are antimuscarinic drugs used in COPD & asthma.
760
What is the mechanism of Oxybutynin? | What is it used for?
It is an antimuscarinic drug used to reduce bladder spasms & alleviate urgency seen with mild cystitis
761
What is the mechanism of Glycopyrrolate? | What is it used for?
It is an antimuscarinic used preoperatively to reduce airway secretions
762
What is atropine used for? | What is seen with overdose?
Atropine is used to treat bradycardia & in ophthalmology. Overdose: "Hot as a hare, dry as a bone, red as a beet, blind as a bad, mad as a hatter"
763
What receptors does epinephrine act at? | What are its uses?
A1, A2, B1, B2 Used in anaphylaxis, open angle glaucoma, asthma, hypotension
764
What receptors does norepinephrine act at? | What are its uses?
A1, A2 > B1 Used for hypotension (but decreases renal perfusion)
765
What do beta adrenergic receptors control?
B1: ^ HR and contractility ^ renin release ^ lipolysis ``` B2: Vasodilation Bronchodilation ^ HR & contractility ^ lipolysis ^ insulin release Tocolysis Ciliary muscle relaxation ^ aqueous humor production ```
766
What are the effects of NE and Isopreterenol on heart rate?
NE --> alpha-adrenergic stimulation --> ^ BP --> reflex bradycardia Isopreterenol --> beta-adrenergic stimulation -->B2 causes decreased BP --> reflex tacycardia (plus B1 stimulation ^ HR)
767
What receptors does isopreterenol act at? | What are its uses?
B1, B2 It is used in Torsades & bradyarrhythmias Don't use if ischemia is suspected (lowers BP and ^ HR)
768
What receptors does Dopamine act at? | What are its uses?
D1 > B1, B2, > A1, A2 Used for shock (helps renal perfusion), heart failure Low dose = ^ RPF Medium dose = ^ CO High dose = lowered CO
769
What receptors does dobutamine act at? | What are its uses?
B1 >> B2, A1, A2 Used for heart failure, cardiac stress testing.
770
What receptors does phenylephrine act at? | What are its uses?
A1, A2 Used for hypotension, ocular procedures (mydriasis), rhinitis (decongestant)
771
What receptors does albuterol act at? | What are its uses?
B2 >> B1 Albuterol is used for acute asthma. Salmeterol is used for long-term asthma or COPD control. Terbutaline is used to reduce premature uterine contractions.
772
What receptors does Ritodrine act at? | What are its uses?
B2 Reduces premature contractions
773
What are the indirect sympathomimetics?
Amphetamine - releases stored catecholamines Ephedrine - same as amphetamine Cocaine - catecholamine reuptake inhibitor (never give beta blockers to cocaine abuser, can cause unopposed alpha stimulation --> malignant HTN)
774
What receptors does clonidine act at? | What are its uses?
It is a centrally acting A2 agonist --> reduces central sympathetic outflow. It's useful in HTN comorbid with renal disease because it does not reduce kidney perfusion.
775
What drug is used preoperatively for pheochromocytoma? | What is its mechanism?
Phenoxybenzamine is an irreversible alpha-adrenergic antagonist.
776
What is Phentolamine? | What is its use?
It is a reversible nonselective alpha-adrenergic antagonist. It is used to treat patients who are on MAOi's and eat Tyramine.
777
What is Terazosin? | What is it used for?
Prazosin, Terazosin, Doxazosin, Tamsulosin They are A1-selective adrenergic antagonists. They are used to treat HTN & urinary retention from BPH. They can cause 1st dose orthostatic hypotension, dizziness, headache.
778
What is Mirtazapine? | What is it used for?
It is an alpha-2 blocker used to treat depression.
779
What are the uses for beta blockers?
``` Angina MI SVT (AV node) HTN (acts on heart and juxtaglomerular app. cells) CHF Glaucoma ```
780
What are the side effects of beta blockers?
Impotence Asthma exacerbation Bradycardia or AV block CNS - seizures, sedation, sleep alteration Use with caution in diabetics (mask hypoglycemia) & asthmatics
781
What are the B1-selectinve beta blockers?
``` BEAM: Betaxolol (partial agonist) Esmolol (short-acting)\ Atenolol Metoprolol ```
782
What are the nonselective beta blockers?
``` "Please Try Not Being Picky" Propranolol Timolol Nadolol Beta blockers yo Pindolol ```
783
What are the nonselective alpha & beta blockers?
Carvedilol | Labetalol
784
What are the beta-adrenergic partial agonists?
Partial Agonist: Pindolol Acebutolol
785
What is the treatment for a beta blocker overdose?
Glucagon
786
What is the treatment for a digitalis overdose?
``` KLAM treatment K+ normalization Lidocaine Anti-dig Fab fragments Mg2+ ```
787
What is the treatment for a lead overdose?
EDTA Dimercaprol Penicillamine
788
What is the treatment for an iron overdose?
DeFeroxamine
789
What is the treatment for a Mercury overdose?
Dimercaprol | Succimer
790
What is the treatment for a copper overdose?
Penicillamine
791
What is the treatment for an arsenic/gold overdose?
Penicillamine Dimercaprol Succimer
792
What is the treatment for methemoglobin?
Methylene blue
793
What is the treatment for a benzodiazepine overdose?
Flumazenil
794
What is the treatment for a TCA overdose?
Alkalinize urine (NaHCO3)
795
What is the treatment for a heparin overdose?
Protamine sulfate
796
What is the treatment for a warfarin overdose?
Fresh frozen plasma | Vitamin K
797
What is the treatment for a tPA/streptokinas/urokinase overdose?
Aminocaproic acid
798
What is the treatment for a Theophylline overdose?
Beta blocker
799
What drugs cause flushing?
``` VANC: Vancomycin Adenosine Niacin Calcium channel blockers ```
800
What drugs can cause hemolysis in G6PD deficient patients?
``` "Hemolysis IS PAIN" INH Sulfa drugs Primaquine Aspirin Ibuprofen Nitrofurantoin ```
801
What drug can cause acute cholestatic hepatitis?
Erythromycin
802
What drugs can cause gynecomastia?
"Some Drugs Create Awkward Knockers" ``` Spironolactone Digitalis Cimetidine Alcohol (chronic) Ketoconazole ```
803
What drugs can cause hypothyroidism?
Lithium Amiodarone Sulfa drugs
804
What drugs can cause pulmonary fibrosis?
hard to BLAB when you have pulmonary fibrosis BLeomycin Amiodarone Busulfan
805
What drugs can cause gingival hyperplasia?
Phenytoin Verapamil Cyclosporine
806
What drugs can cause myopathies?
Fish N CHIPS Give myopathies ``` Fibrates Niacin Colchicine Hydroxychloroquine Interferon-alpha Penicillamine Statins Glucocorticoids ```
807
What drugs can cause photosensitivity?
SAT for a photo Sulfa drugs Amiodarone Tetracycline
808
What drugs can cause drug-induced lupus?
HIPP Hydralazine INH Procainamide Phenytoin
809
What drugs can cause seizures?
BITE MI tongue ``` Bupropion Imipenem Tramadol Enflurane Metaclopramide INH ```
810
What drugs are both nephrotoxic and ototoxic?
Aminoglycosides Vancomycin Loop diuretics Cisplatin
811
What are the sulfa drugs?
Popular FACTSSS ``` Probenecid Furosemide Acetazolamide Celecoxib Thiazides Sulfa antibiotics Sulfasalazine Sulfonylureas ```
812
Match the suffix with the drug class: - iptan - caine - azine - etine - ipramine/-triptyline - tidine - nib - cept
- iptan = 5-HT1B/1D agonist - caine = local anesthetic - azine = neuroleptic, antiemetic - etine = SSRI - ipramine/-triptyline = TCA - tidine = H2 antagonist - nib = kinase inhibitor - cept = receptor molecule
813
What is the upper limit of a normal ESR?
Men: age/2 Women: (age+10)/2
814
What causes senile systemic amyloidosis?
Deposition of normal TTR (Transthyretin)
815
What cancers is ret associated with?
MEN2A & MEN2B
816
Why is CVID unique?
Can be acquired in 20's-30's
817
What causes cauda equina syndrome? | What is seen?
Rupture of intervertebral disk --> compresses 2+ spinal nerves ``` Symptoms: Low back pain that radiates to legs Asymmetrical saddle anesthesia Loss of anocutaneous reflex Loss of knee & ankle jerk reflex (Generally unilateral) paraplegia ```
818
What causes conus medullaris syndrome? | What is seen?
Disk herniation, tumor, spinal fracture --> L2 lesion --> conus medullaris syndrome ``` Symptoms: Acute onset severe low back pain Urinary & fecal incontinence Impotence Saddle anesthesia (S3-S5) Generally spares legs (might see mild weakness) ```
819
What drugs can cause Torsades de pointes
``` Class III (Sotolol) & Class Ia (Quinidine) antiarrhythmics TCA's ```
820
What structures cease at the lung bronchi? | What cease at the terminal bronchioles?
Bronchi - Cartilage, goblet cells Terminal bronchioles - Pseudostratified ciliated columnar cells (mucociliary escalator) Smooth muscle of the airways
821
What type of epithelia are found in the lung?
Bronchi & terminal bronchioles = Pseudostratified ciliated columnar epithelium Respiratory bronchioles = cuboidal cells Alveolar ducts & alveoli = simple squamous
822
What is the equation for collapsing pressure of alveoli?
P = 2(surface tension)/radius
823
What test shows lung maturity in a fetus?
Lecithin:Spingomyelin ratio > 2.0 | <1.5 increases risk for NRDS
824
What are lamellar bodies?
Intracellular type 2 pneumocte inclusions. They contain phospholipids for production of surfactant.
825
What are club cells?
They are nonciliated columnar cells found in the small airways. They can degrade inhaled toxins (via CYP450) & act as reserve cells. They can also secrete substances to protect the bronchiolar lining.
826
Where do aspirates go in the lung?
Upright --> bottom of RLL Supine --> top of RLL
827
How are the pulmonary artery & bronchus organized in the lungs?
Pulmonary artery is RALS to the bronchus: Right anterior Left superior
828
At what level do the main structures penetrate the diaphragm?
"I ate ten eggs at noon" IVC = T8 Esophagus/Vagus = T10 Aorta/Azygous vein/Thoracic duct = T12
829
What are the accessory muscles of breathing?
Inspiration - External intercostals, SCM, scalenes Expiration - Rectus abdominus, obliques, internal intercostals
830
What is the equation for total lung volume?
LITER: | tLc = IRV + TV + ERV + RV
831
``` What are typical pulmonary values for: TV FRC RV TLC ```
``` TV = 500 mL FRC = 2200 mL RV = 1200 mL TLC = 6000 mL ```
832
What are the two forms of HbA & their affinity for oxygen?
``` T = taut = low affinity for O2 R = relaxed = 300x higher affinity for O2 ``` This is the basis for the positive cooperativity seen with Hb. Binding of O2 induces the relaxed form in the other Hb molecules.
833
What factors cause Hb to bind O2 less avidly in the tissues?
``` Increase in C-BEAT: CO2, Cl- BPG Exercise Acid/Altitude Temperature ``` "Taut in tissues, Relaxed in respiratory"
834
What causes HbF to have a higher affinity for O2 than HbA?
It does not efficiently bind 2,3-BPG
835
What causes Methemoglobinema? | How is it treated?
Nitrites oxidize Fe2+-->Fe3+ (cannot bind oxygen) Use METhyline blue for METhemoglobinemia
836
What is the main problem with CO poisoning?
O2 cannot be unloaded in tissues because CO (higher affinity than O2 for Hb) holds Hb in the relaxed form. Causes left shift in the Hb dissociation curve.
837
How does myoglobin differ from hemoglobin?
It is found within muscle cells & is monomeric (Hb is tetrameric).
838
What gases are perfusion limited in the lung? | Diffusion limited?
Perfusion limited = O2 (health), N2O, CO2 | Diffusion limited = CO
839
What is the equation for diffusion capacity?
V(gas) = (Area/Thickness)*Diff. constant*(P1-P2) Emphysema --> decreased area Fibrosis --> increased thickness
840
What is the cause of primary pulmonary hypertension?
Inactivating mutation in BMPR2 (normally inhibits vascular smooth muscle proliferation) Seen in young women.
841
What defines pulmonary hypertension? | What can cause secondary pulmonary hypertension?
Pulmonary artery pressure > 25 mmHg ``` Causes of secondary PHTN: COPD Mitral stenosis Recurrent PE's Autoimmune disease Left-to-right shunt Sleep apnea Living at high altitude ```
842
What is the equation for pulmonary vascular resistance?
PVR = (Pulm art. Pressure - Wedge pressure)/Cardiac output
843
What is a normal O2 binding capacity?
15g/dL of Hb * (1.34 mlO2/gHb) = ~20 mLO2/dL Cyanosis is seen when > 5g/dL of Hb is deoxygenated
844
What is the alveolar gas equation?
PAO2 = PIO2 - (PaCO2/R) ``` PIO2 = PO2 of inspired air = 150 mmHg R = respiratory quotient = CO2 produced/O2 consumed = 0.8 ```
845
What is a normal A-a gradient? | What can cause an increased A-a gradient?
Normal = 10-15 mmHg Increased in: Right-to-left shunt Diffusion limitation V/Q mismatch
846
What are the causes of hypoxemia? | Which show an increased A-a gradient?
Normal A-a gradient: High altitude Hypoventilation Increased A-a gradient: Right-to-left shunt Diffusion limitation V/Q mismatch
847
What type of V/Q mismatch improves with supplemental O2?
High V/Q (dead space) should improve with O2 V/Q of 0 (shunt) will not improve with O2
848
What are the alveolar, arterial, and venous pressures like throughout the lung?
``` Zone 1 (apex) = A > a > v Zone 2 = a > A > v Zone 3 (base) = a > v > A ```
849
What part of the lung has a high vs. low V/Q
High V/Q in apex (dead space) V/Q = 1 in the middle of the lung Low V/Q in base (shunt) *Note that the base is actually better ventilated than the apex, but it is much much better perfused --> low V/Q
850
How is CO2 carried in the blood?
1) Bicarbonate (90%) 2) HbCO2 (5%) 3) Dissolved CO2 (5%)
851
How does CO2 from the tissues cause O2 unloading?
CO2 --(carbonic anhydrase)--> H2CO3 H+ & HCo3- --> H+ binds Hb --> taut Hb --> O2 release. The opposite happens in the lung due to high amount of O2. CO2 is released.
852
What changes occur at high altitude?
Acute ^ in ventilation --> decreased O2 & CO2 Chronic: ^ EPO ^ 2,3-BPG ^ renal excretion of bicarb (compensates for resp. alk.) Chronic hypoxic vasoconstriction --> RVH
853
What changes in vascular O2 & CO2 are seen during exercise?
PaO2 & PaCO2 are unchanged | Venous O2 is decreased & venous CO2 is increased
854
What are the components of Virchow's triad of hemostasis?
1) Stasis 2) Hypercoagulability 3) Endothelial damage These predispose to coagulation.
855
What are the types of pulmonary emboli?
``` Fat (long bone fractures; hypoxemia + petechial rash) Gas (the bends, the chokes) Thrombus (95%) Bacteria Amniotic fluid (can cause DIC) Tumor ```
856
What defines chronic bronchitis?
Chronic productive cough lasting > 3 months total over the past 2 years
857
What is seen on spirometry in obstructive lung disease? | Restrictive lung disease?
Obstructive = LOW FEV1, low FVC --> lower FEV1/FVC Restrictive = low FEV1, LOW FVC --> FEV1/FVC > 80%
858
Who are blue bloaters & pink puffers? | Why?
Chronic bronchitis = blue bloaters They are constantly hypoxemic RHF --> edema Emphysema = pink puffers They must work hard to breath Puff their lips out when they exhale (PEEP)
859
What is the Reid index?
Thickness of the mucus gland layer in relation to the whole bronchial wall. It is > 50% in chronic bronchitis.
860
What causes centriacinar emphysema? Panacinar emphysema? Where are they seen?
Smoking --> centriacinar emphysema in upper lobes (more smoke) A1AT deficiency --> panacinar emphysema in lower lobes (more blood)
861
What chromosome is asthma associated with?
Chromosome 5
862
What is seen histologically with asthma?
Curschmann's spirals (shed epithelium plugs) | Charcot-Leyden crystals (from eosinophils)
863
What are the mediators of an asthma attack?
Early phase: Histamine, LTC4,D4,E4 Late phase: Inflammation; Major basic protein
864
What can cause bronchiectasis?
``` Cystic fibrosis Kartagener's Bronchial obstruction Smoking (poor ciliary motility) Bronchopulmonary aspergillosis ```
865
What are the causes of restrictive lung disease?
``` Idiopathic pulmonary fibrosis Pneumoconioses Hypersensitivity pneumonitis Sarcoidosis Drug toxicity (bleomycin, busulfan) Recurrent PE Also poor breathing mechanics (obesity, MG) ```
866
What lobes are affected in the pneumoconioses?
Anthracosis & silicosis --> upper lobes | Asbestosis --> lower lobes
867
What are the risks of the pneumoconioses?
Anthracosis - fibrosis Silicosis - fibrosis, TB infection, bronchogenic carcinoma Asbestosis - fibrosis, bronchogenic carcinoma, mesothelioma
868
When are adequate levels of surfactant produced in-utero?
Production begins at 28 weeks & is sufficient at 34 weeks
869
What are the risk factors for NRDS? | The treatment?
Risk factors: Prematurity Maternal diabetes (insulin decreases production) C-section (glucocoricoids ^ production) Treatment: Intrapartum dexamethasone Surfactant at birth
870
What complications are seen with NRDS?
Hypoxemia --> Necrotizing enterocolitis & PDA Supplemental O2 --> Bronchopulmonary dysplasia & Retinopathy of the newborn
871
What can cause ARDS?
``` Trauma Sepsis Shock DIC Aspiration Uremia Acute pancreatitis Amniotic fluid embolism Hypersensitivity ```
872
What is seen histologically with pulmonary hypertension?
Plexiform lesions Arteriosclerosis Hypertrophy of the media Intimal fibrosis
873
How does sleep apnea cause hypertension?
1) ^ release of catecholamines during night | 2) Hypoxia --> ^ EPO --> ^ erythropoiesis
874
What are the complications of lung cancer?
``` SPHERE of complications: SVC syndrome Pancoast tumor Horner's syndrome Endocrine (paraneoplastic) Recurrent laryngeal (hoarseness) Effusions ```
875
What lung cancers are most vs. least common?
``` Metastases (breast, colon, prostate, bladder) Adenocarcinoma Squamous cell carcinoma Small cell carcinoma Large cell carcinoma Bronchial carcinoid tumor ```
876
Where does lung cancer metastasize?
Adrenals Brain Bone Liver
877
What lung tumors are located centrally? | Peripherally?
Peripheral = adenocarcinoma, Large cell carcinoma Central = Small cell carcinoma, Squamous cell carcinoma
878
What is the most common lung cancer in nonsmokers and female smokers?
Adenocarcinoma
879
What mutation is commonly seen in lung adenocarcinoma?
k-ras mutation
880
What defines the Bronchioloalveolar subtype of lung cancer?
It is a subtype of adenocarcinoma. CXR resembles interstitial pneumonia because it grows along the alveolar septa. Seen in nonsmokers & Eastern Asian women.
881
What paraneoplastic syndromes are seen with lung cancers?
Hypercalcemia (PTHrP) --> Squamous ACTH; ADH --> Small cell Lambert-Eaton --> Small cell
882
How does bronchial carcinoid appear grossly? | Histologically?
Grossly they are a polyp within the bronchus. | Histologically they are nests of neuroendocrine cells chromogranin positive
883
Which lung cancers are neuroendocrine in origin?
``` Small cell (Kulchitsky cells) Carcinoid ```
884
What is a pancoast tumor?
Carcinoma in the apex of the lung: Horner's syndrome (cervical chain) +/- Arm weakness (brachial plexus)
885
What is SVC syndrome?
Compression of the SVC seen sometimes with lung cancer. Impairs blood drainage from the head, neck, & arms: Facial plethora (redness) JVD Upper extremity edema Is a medical emergency because it can raise ICP.
886
What antiplatelet factors are secreted by the endothelium?
PGI2 (prostacyclin) | NO
887
What is the treatment for status epilepticus?
1) IV lorazepam + load with phenytoin 2) (if still seizing) Phenobarbital 3) (if still seizing) Intubate & give general anesthesia
888
How are the right and left renal veins different?
The left renal vein drains the kidney, left testicle, and left adrenal. The Right testicular vein drains directly to the IVC. And the Right suprarenal vein also drains directly to the IVC.
889
How is menopause confirmed?
Serum FSH (>30 U/L)
890
What is the treatment for thrush in immunocompetent patients?
Nystatin swish & swallow
891
How many half lives does it take for a drug to be largely gone from the plasma?
5 half lives = ~97% cleared
892
What are the 3 types of pneumonia?
Lobar Bronchopneumonia Interstitial (atypical) pneumonia
893
What are the organisms that cause atypical pneumonia?
Mycoplasma Chlamydia Viruses (CMV, influenza) Coxiella (Q fever)
894
What are the common causes of lung abscess?
``` S. aureus Anaerobes: -Bacteroides -Fusobacterium -Peptostreptococcus ```
895
What are the common causes of hypersensitivity pneumonitis?
``` Moldy hay (farmers) Bird droppings (pigeon breeder's lung) ```
896
What is seen in hypersensitivity pneumonitis?
Mixed Type III/IV hypersensitivity reaction --> granulomatous reaction --> can lead to interstitial fibrosis
897
What is characteristic of transudate effusion? Exudate? Chylothorax?
``` Transudate = low protein content Exudate = high protein content Chylothorax = High TRG content ```
898
What causes chylothorax?
Thoracic duct injury from trauma or malignancy.
899
What causes spontaneous pneumothorax?
Rupture of apical blebs. Usually seen in tall, thin, young males.
900
What are 1st generation H1 blockers for?
Allergy, motion sickness, sleep aid. Some examples: Diphenhydramine Dimenhydrinate Chlorpheniramine
901
What are 2nd generation H1 blockers used for?
Allergy meds. Far less sedating than 1st gen due to less CNS penetration. ``` Some examples: Loratadine Fexofenadine Desloratadine Cetirizine ```
902
What is Theophylline used for? | What is its mechanism?
It is used to cause bronchodilation in asthmatics. It inhibits phosphodiesterase --> ^ cAMP in smooth muscle cells. It also blocks adenosine's action (bronchoconstriction) in the lung.
903
What muscarinic antagonist is first line in COPD?
Tiotropium | long-acting version of Ipratropium
904
What are the inhaled corticosteroids used in asthma?
Beclomethasone | Futicasone
905
What is Montelukast? | What is its use?
Montelukast & Zafirlukast are leukotriene receptor antagonists. They are used to treat asthma, especially aspirin-induced asthma.
906
What is Zileuton? | What is its use?
It is a 5-lipoxygenase pathway inhibitor (blocks conversion of arachidonic acid to leukotrienes). It is used to treat asthma.
907
What is N-acetylcysteine used for?
1) Mucolytic - loosens mucous plugs in CF patients | 2) Donates sulfhydryl groups to treat acetaminophen OD
908
What is Bosentan? | What is it used for?
It is an endothelin-1 receptor antagonist used to treat pulmonary hypertension.
909
What is Dextromethorphan? | What is it used for?
It is a synthetic codeine analog used as an antitussive.
910
What is phenylephrine? | What is it used for?
Phenylephrine & pseudoephedrine are alpha-agonists used as OTC nasal decongestants. They are stimulants & can cause anxiety or hypertension with toxicity.
911
What antiplatelet factors are secreted by the endothelium?
PGI2 (prostacyclin) | NO
912
What is the treatment for status epilepticus?
1) IV lorazepam + load with phenytoin 2) (if still seizing) Phenobarbital 3) (if still seizing) Intubate & give general anesthesia
913
How are the right and left renal veins different?
The left renal vein drains the kidney, left testicle, and left adrenal. The Right testicular vein drains directly to the IVC. And the Right suprarenal vein also drains directly to the IVC.
914
How is menopause confirmed?
Serum FSH (>30 U/L)
915
What is the treatment for thrush in immunocompetent patients?
Nystatin swish & swallow
916
How many half lives does it take for a drug to be largely gone from the plasma?
5 half lives = ~97% cleared
917
What are the 3 types of pneumonia?
Lobar Bronchopneumonia Interstitial (atypical) pneumonia
918
What are the organisms that cause atypical pneumonia?
Mycoplasma Chlamydia Viruses (CMV, influenza) Coxiella (Q fever)
919
What are the common causes of lung abscess?
``` S. aureus Anaerobes: -Bacteroides -Fusobacterium -Peptostreptococcus ```
920
What are the common causes of hypersensitivity pneumonitis?
``` Moldy hay (farmers) Bird droppings (pigeon breeder's lung) ```
921
What is seen in hypersensitivity pneumonitis?
Mixed Type III/IV hypersensitivity reaction --> granulomatous reaction --> can lead to interstitial fibrosis
922
What is characteristic of transudate effusion? Exudate? Chylothorax?
``` Transudate = low protein content Exudate = high protein content Chylothorax = High TRG content ```
923
What causes chylothorax?
Thoracic duct injury from trauma or malignancy.
924
What causes spontaneous pneumothorax?
Rupture of apical blebs. Usually seen in tall, thin, young males.
925
What are 1st generation H1 blockers for?
Allergy, motion sickness, sleep aid. Some examples: Diphenhydramine Dimenhydrinate Chlorpheniramine
926
What are 2nd generation H1 blockers used for?
Allergy meds. Far less sedating than 1st gen due to less CNS penetration. ``` Some examples: Loratadine Fexofenadine Desloratadine Cetirizine ```
927
What is Theophylline used for? | What is its mechanism?
It is used to cause bronchodilation in asthmatics. It inhibits phosphodiesterase --> ^ cAMP in smooth muscle cells. It also blocks adenosine's action (bronchoconstriction) in the lung.
928
What muscarinic antagonist is first line in COPD?
Tiotropium | long-acting version of Ipratropium
929
What are the inhaled corticosteroids used in asthma?
Beclomethasone | Futicasone
930
What is Montelukast? | What is its use?
Montelukast & Zafirlukast are leukotriene receptor antagonists. They are used to treat asthma, especially aspirin-induced asthma.
931
What is Zileuton? | What is its use?
It is a 5-lipoxygenase pathway inhibitor (blocks conversion of arachidonic acid to leukotrienes). It is used to treat asthma.
932
What is N-acetylcysteine used for?
1) Mucolytic - loosens mucous plugs in CF patients | 2) Donates sulfhydryl groups to treat acetaminophen OD
933
What is Bosentan? | What is it used for?
It is an endothelin-1 receptor antagonist used to treat pulmonary hypertension.
934
What is Dextromethorphan? | What is it used for?
It is a synthetic codeine analog used as an antitussive.
935
What is phenylephrine? | What is it used for?
Phenylephrine & pseudoephedrine are alpha-agonists used as OTC nasal decongestants. They are stimulants & can cause anxiety or hypertension with toxicity.
936
What are the stages of fetal kidney development?
Pronephros = week 4; degenerates Mesonephros = interim kidney during 1st trimester; forms Wolffian ducts in male Metanephros = Permanent; nephrogenesis runs from 5th week to 32-36 weeks.
937
What are the components of the metanephros? | What do they give rise to?
1) Ureteric bud: forms collecting system (collecting ducts, calyces, pelvises, ureters 2) Metanephric mesenchyme: forms glomerulus through DCT. Induced by interaction with the ureteric bud.
938
What is the last portion of the fetal kidney to canalize?
The ureteropelvic junction. This is why it is the most common site of obstruction (hydronephrosis) in the fetus.
939
What is Potter's syndrome?
Oligohydramnios --> limb/facial deformities, pulmonary hypoplasia, death in-utero Caused by: Bilateral renal agenesis, ARPKD
940
What is seen with horseshoe kidney?
Inferior poles are fused --> get caught on IMA & remain low in abdomen. It is associated with Turner syndrome.
941
What causes dysplastic kidney?
Abnormal interaction between ureteric bud and metanephric mesenchyme --> nonfunctional kidney composed of cysts and connective tissue. Usually unilateral, NON-inherited.
942
What is the course of the ureters?
"Water under the bridge" They pass under the uterine artery, ductus deferens, & gonadal artery/vein.
943
What is the body composition of water?
60-40-20 rule: 60% water 40% is intracellular 20% is extracellular 1/4 of that extracellular fluid is plasma, 3/4 is interstitial.
944
What is the glomerular filtration barrier composed of?
Fenestrated capillary endothelium Basement membrane with heparan sulfate (neg. charge) Epithelial barrier of podocyte foot processes
945
What is the equation for renal clearance of a substance?
CL = UV/P ``` U = Urine concentration of the solute V = Urine flow rate P = Plasma concentration of the solute ``` If CL > GFR --> net tubular secretion of the solute If CL < GFR --> net tubular reabsorption of the solute
946
What substance can be used to calculate GFR? | RPF?
Inulin = freely filtered, not reabsorbed/secreted --> GFR (Creatinine clearance approximates it but slightly overestimates) PAH = filtered & secreted completeley --> effective RPF (ERPF) (Underestimates true RPF by ~10%) By calculating the clearance of these substances (CL = UV/P), you can get the GFR & RPF.
947
What is the equation for renal blood flow?
RBF = RPF/(1 - HCT)
948
What is the equation for filtration fraction? | What is a normal FF?
FF = GFR/RPF Normal = 20%
949
What are the relative bloodflows to organs under resting conditions?
"Having Sex Brings Many Kids Later" ``` Heart = 250 mL/min = 5% Skin = 500 mL/min = 10% Brain = 750 mL/min = 15% Muscle = 1000 mL/min = 20% Kidney = 1250 mL/min = 25% Liver = 1500 mL/min = 30% ```
950
What are the effects of NSAIDs on the kidney? | ACE inhibitors?
NSAIDs constrict afferent arteriole (inhibit prostaglandins that normally dilate it) ACE inhibitors dilate efferent arteriole (AT-II preferentially constricts efferent arteriole)
951
How is the reabsorption/secretion of a substance (x) calculated?
Filtered load = GFR*P[x] Excreted load = V*U[x] Reabsorption rate = filtered - excreted Secretion rate = excreted - filtered
952
At what serum glucose does glucose appear in the urine?
>160 mg/dL --> glucosuria begins | >350 mg/dL --> all transporters saturated
953
What is the effect of PTH on the nephron?
Proximal tubule: PTH inhibits Na+/phosphate cotransport --> phosphate excretion Distal tubule: PTH increases Ca2+/Na+ exchange --> Ca2+ reabsorption
954
What is the effect of Angiotensin II in the nephron?
Proximal tubule: Stimulates Na+/H+ exchange --> ^ Na+/H2O/HCO3- reabsorption Also causes efferent arteriole constriction --> ^ FF
955
What is reabsorbed in the Proximal tubule?
``` In order of reabsorption rate: Glucose Amino acids HCO3- Phosphate Na+ (65-80%) K+ Cl- Urea ```
956
Why does silicosis increase susceptibility to MTB infection?
Macrophage phagolysosomes are disrupted.
957
What occurs in SMA syndrome?
The angle of the superior mesenteric artery and the aorta decreases, compressing & obstructing the transverse portion of the duodenum. This will cause symptoms of small bowel obstruction.
958
What causes SMA syndrome?
Any very catabolic state (weight loss, burns, long bedrest) leading to diminished mesenteric fat Low body weight Severe lordosis Surgical correction of scoliosis
959
What are the two types of MG exacerbation?
Myasthenic crisis: Too low of AChE inhibitor dose Responds to edrophonium (short-acting AChE inhibitor) Cholinergic crisis: Too high of AChE inhibitor dose (muscle cells depolarized too often & can't recover) Does not respond to edrophonium
960
What steps in collagen synthesis occur extracellularly?
Cleavage of N & C terminal peptides Spontaneous assembly of fibrils Covalent crosslinking by lysyl oxidase
961
What does glycerol kinase do? | Where is it located?
It catalyzes Glycerol (from beta oxidation) --> DHAP This allows glycerol to enter glycolysis or gluconeogenesis. It is specific to the liver.
962
What are the symptoms of lead poisoning?
Colicky abdominal pain (lead colic), constipation, headaches Lead line (blue pigment) at the gum-tooth line Wrist/foot drop due to peripheral neuropathy Microcytic hypochromic anemia with basophilic stippling
963
What is used to determine the proliferation rate of cancer cells?
Ki-67 fraction Ki-67 is an antigen that is only present on actively dividing cells. The closer to 100%, the more cells are actively dividing.
964
What is Cromylin sodium? | What is it used for?
It inhibits mast cell degranulation. | It is used for asthma prophylaxis.
965
What are the initiator caspases? | Executioner caspases?
Initiator caspases = 8 & 10 | Executioner caspases = 3 & 6
966
What is reabsorbed in the thick ascending limb?
Na+/K+/2Cl- (via symporter) Mg2+/Ca2+ (paracellular, pushed electrically by K+ backleak into tubule)
967
What is reabsorbed in the DCT?
Na+/Cl- (symporter) Ca2+ (antiporter with Na+ is at basolateral membrane & channels are at apical membrane)
968
What is reabsorbed in the Collecting tubules?
Reabsorb Na+ in exchange for K+ & H+ (Aldosterone) Water is reabsorbed via aquaporins (ADH)
969
How is total body water measured? Extracellular fluid? Plasma volume?
TBW - tritiated water ECF - inulin Plasma - radiolabeled albumin
970
What cell types are found in the nephron's collecting duct? | What are their purposes?
Principal cells: Reabsorb Na+ (Ald --> ENaC synthesis) Reabsorb H2O (ADH --> aquaporin insertion) Secrete K+ Intercalated cells: Secrete H+ (aldosterone stimulated ATPase)
971
What are the stimulators of renin?
Decreased BP Decreased NaCl delivery to DCT (sensed by macula densa cells) B1 adrenergic receptor simulation Secreted by JG cells
972
What are the effects of Angiotensin II?
AT-1 receptors --> systemic vasoconstriction Constricts efferent arteriole (^ FF) Aldosterone & ADH secretion Increases activity of PCT Na+/H+ antiporter Stimulates thirst in hypothalamus
973
Where is EPO synthesized?
EPO is made in the peritubular capillary cells of the renal cortex in response to hypoxia.
974
Where exactly is 25-OH-Vitamin D activated to Calcitriol?
Proximal tubule cells
975
What can cause potassium to shift out of cells?
DO LAB: ``` Digitalis Osmolarity ^ Lysis Acidosis Beta blockers ``` Whereas INsulin shifts K+ INto cells
976
What is seen with hypomagnesemia?
Decreased DTR's Lethargy Bradycardia & hypotension Hypocalcemia
977
How can a simple vs. mixed metabolic acidosis be determined?
Calculate the expected PCO2 & see if the true PCO2 is adequately compensating (+/- 2). Expected PCO2 = 1.5*(HCO3-) + 8 If the true PCO2 is within 2 either way --> simple If it is not within 2 --> mixed acid-base disorder
978
What causes contraction alkalosis?
Loss of blood volume --> Renin, AT, Ald system ATII: ^Na+/H+ exchange in PCT ^HCO3- reabsorption (due to ^H+ available in the lumen) Ald: ^ H+ ATPase activity --> H+ secretion
979
How is an anion gap calculated?
Na - (Cl + HCO3) Normal = 8-12
980
What can cause an increased anion gap metabolic acidosis?
MUDPILES: ``` Methanol Uremia DKA Propylene glycol INH or Iron tablets Lactic acidosis Ethylene glycol Salicylates (late) ```
981
What can cause a non-anion gap metabolic acidosis?
HARDASS: ``` Hyperalimentation (overeating, too much TPN) Addison's Renal tubular acidosis Diarrhea Acetazolamide Spironolactone Saline infusion ```
982
What can cause a metabolic alkalosis?
Loop diuretics Vomiting Antacids Hyperaldosteronism
983
What are the causes of the renal tubular acidoses?
Type 1 = Collecting duct cannot excrete H+ (distal) Type 2 = PCT cannot reabsorb HCO3 (proximal) Type 3 = Hypoaldosteronism or collecting duct not responsive to it --> hyperkalemia --> impaired ammoniagenesis --> urine cannot buffer titratable acids
984
What are the risks of the renal tubular acidoses?
"Stones, bones, and low aldosterones" Type 1 --> Nephrolithiasis Type 2 --> Hypophosphatemic rickets Type 3 --> hyperkalemic metabolic acidosis
985
What are the casts & their significance?
``` RBC casts WBC casts Fatty casts (oval fat bodies) = nephrotic syndrome Granular (muddy brown) casts = ATN Waxy casts = Chronic/advanced disease Hyaline casts = nonspecific ```
986
What is seen with nephrotic syndrome?
PALE: Proteinuria (> 3.5g/day) Albumin low Lipidemia/lipiduria Edema ATIII loss --> thromboembolism Ig loss --> infection
987
What is seen on microscopy with FSGS?
LM - focal (< half) & segmental sclerosis & hyalinosis EM - effacement of foot processes
988
What is seen on microscopy with Membranous nephropathy?
LM - diffuse GMB thickening ("membranous") EM - "spike & dome" appearance with subepithelial deposits
989
What is seen on microscopy with Minimal Change Disease?
LM - normal glomeruli EM - effacement of foot processes
990
What is seen on microscopy with Membranoproliferative Glomerulonephritis?
Type I - subendothelial immune complex deposits with "tram track" appearance; granular IF pattern Type II - intramembranous immune complex "dense deposits"
991
What is seen on microscopy with diabetic nephropathy?
LM - mesangial expansion, GBM thickening; Kimmelstiel-Wilson nodules`
992
What is seen on microscopy with Post-infectious glomerulonephritis?
LM - PMN's, lump-bumpy appearance EM - subepithelial humps of immune complexes IF - granular deposits along GBM
993
What is seen on microscopy with RPGN?
Crescents Composed of: Fibrin Plasma proteins Macrophages IF - can be linear (type 1), granular (type 2), or pauci-immune (type 3)
994
What is seen on microscopy with diffuse proliferative glomerulonephritis?
LM - "wire looping" of capillaries EM - subedothelial & sometimes intramembranous immune complexes IF - granular
995
What is seen on microscopy with Berger's disease?
LM - mesangial proliferation EM - mesangial immune complex deposits IF - IgA-based immune complex deposits in mesangium
996
What is associated with FSGS?
``` Most common nephrotic syndrome in Hispanic & Blacks HIV --> wall collapse variant Heroin Sickle cell Interferon treatment ```
997
What is associated with minimal change disease?
Most common nephrotic syndrome in children Selective loss of albumin (loss of GBM polyanions) Responds to corticosteroids Hodgkin's lymphoma
998
What is associated with membranoproliferative GM?
Type I - HBV, HCV Type II - C3 nephritic factor (Ig that stabilizes C3 convertase leading to overactivation of complement)
999
Which nephrotic syndrome causes are due to immune complex deposits?
Anything with "membrano-" in the name: Membranous = subepithelial IC deposits MPGN Type I = subendothelial IC deposits MPGN Type II = intramembranous IC deposits
1000
What is membranous nephropathy associated with?
Found in white adults SLE's nephrotic presentation Drugs, infections, solid tumors (caused by in-situ IC deposition)
1001
What is seen with nephritic syndrome?
PHAROH: ``` Proteinuria (<3.5 g/day) Hematuria & RBC casts Azotemia RBC casts Oliguria Hypertension ```
1002
What are the causes of RPGN?
Type I = Goodpasture's syndrome (Ig's against kidney & lung BM) Type II = Any of the other nephritic syndromes but worse Type III = pauci-immune (GPA, Churg-Strauss, MP)
1003
What is associated with Diffuse Proliferative GM?
It is the nephritic presentation of SLE or MPGN. It is the most common cause of death in SLE.
1004
How does Berger's disease present?
aka IgA nephropathy Associated with Henoch-Schonlein purpura It is the most common nephropathy. It presents in childhood with intermittent gross or microscopic hematuria (following URI or gastroenteritis).
1005
What causes Alport syndrome?
An X-linked mutation in Type-IV collagen --> triad of: Nephritic syndrome Deafness Ocular problems
1006
What are the 4 types of kidney stones?
Calcium (oxalate/phosphate) Ammonium-magnesium-phosphate (struvite) Uric acid Cystine
1007
Which kidney stones precipitate in acidic urine? Alkaline urine? Which stone is radiolucent?
``` Acidic = Cystine, Uric acid & Calcium oxalate Basic = Calcium phosphate & Struvite ``` Uric acid stones are radiolucent. All others are radiopaque.
1008
What causes struvite stones?
aka ammonium-magnesium-phosphate stones Caused by urease positive UTI's (Proteus, Klebsiella, Staph)
1009
What kidney stones for staghorn calculi?
Struvite & Cystine stones
1010
How does renal (clear) cell carcinoma present clinically?
``` Often silent for a long time. Symptoms: Triad - hematuria, flank pain, palpable mass Paraneoplastic syndromes (EPO, ACTH, PTHrp) Left-sided varicocele ```
1011
What mutation is present in Von Hippel-Lindau disease? | What is seen?
VHL gene deletion (chrom. 3) --> Constitutive HIF (angiogenic TF) Presentation: Bilateral renal cell carcinoma Hemangioblastomas of retina/cerebellum/medulla
1012
What is Wilms' tumor associated with?
aka nephroblastoma Most common renal cancer in children: Deletion of WT2 tumor suppressor gene on chromosome 11 May be part of: Beckwith-Wiedemann syndrome WAGR complex (Wilms', Aniridia, GU malformation, Retardation)
1013
What are the risk factors for transitional cell carcinoma?
cancer in your P SAC: Phenacetin Smoking Aniline dyes Cyclophosphamide
1014
What are the risk factos for squamous cell carcinoma of the bladder?
Must have squamous metaplasia first. - Schistosoma hematobium (Middle-Eastern male) - Chronic cystitis (older woman) - Longstanding nephrolithiasis
1015
What are the common causes of drug-induced interstitial nephritis?
``` Diuretics Penicillins Sulfa drugs Rifampin NSAIDs ```
1016
What is seen with acute interstitial nephritis?
``` AKI Fever Rash Hematuria Pyuria (eosinophils) ```
1017
What can cause renal papillary necrosis?
``` Diabetes Severe acute pyelonephritis Chronic phenacetin/Aspirin/Acetaminophen use Sickle cell anemia & trait Severe interstitial nephritis ```
1018
What are the phases of ATN & their risks?
1) Inciting event (ischemic or nephrotoxic) 2) Maintenance phase - 1-3 weeks; oliguria; risk of hyperkalemia 3) Recovery phase - polyuria; BUN & Cr return to baseline; risk of hypokalemia
1019
What is the BUN/Cr ratio in the various forms of AKI?
Prerenal: >20 Intrinsic renal: 15, chronic <15
1020
What are the most common causes of ATN?
``` Aminoglycosides Heavy metals Myoglobinuria Ethylene glycol Radiocontrast dye Urate (tumor lysis syndrome) ```
1021
What is seen with chronic renal failure?
``` Uremia Na+/H2O retention --> HTN Hyperkalemic metabolic acidosis Anemia Renal osteodystrophy Dyslipidemia ```
1022
What is seen with uremia?
``` Nausea & anorexia Pericarditis Asterixis Encephalopathy Platelet dysfunction ```
1023
What causes renal osteodystrophy?
CKD --> hyperphosphatemia alongside reduced Vit. D activation --> Secondary hyperparathyroidism --> subperiosteal thinning of bones & metastatic calcification
1024
What causes ADPKD? | What is it associated with?
Autosomal Dominant mutation in PKD1 or PKD2 ``` Associated with: CKD HTN (^ renin production) Berry aneurysms Mitral valve prolapse Benign hepatic cysts ```
1025
What is seen in ARPKD?
``` Potter's syndrome Congenital hepatic fibrosis HTN Portal HTN Progressive renal insufficiency ```
1026
What toxicities are seen with loop diuretics?
OH DANG ``` Ototoxicity Hypokalemia/Hypocalcemia Dehydration Allergy (sulfa) Nephritis (interstitial) Gout ```
1027
``` What is the mechanism of the diuretic classes? Acetazolamide Loop diuretics Thiazides Spironolactone Triamterine/Amiloride ```
``` Acetazolamide = carbonic anhydrase inhibitor Loop diuretics (furosemide, ethacrynic acid) = Na+/K+/2Cl- inhib. Thiazides = DCT NaCl transporter inhibitor Spironolactone = Aldosterone antagonists Triamterine/Amiloride = block cortical collecting duct ENaC's ```
1028
What toxicities are seen with thiazide diuretics?
``` Hypokalemic metabolic alkalosis Sulfa reaction hyperGLUC: Glycemia Lipidemia Uricemia Calcemia ```
1029
What are the potassium sparing diuretics?
Spironolactone Eplerenone Triamterine Amiloride
1030
What toxicities are seen with ACE Inhibitors?
Captopril's CATCHH Cough (bradykinin; not seen with ARB) Angioedema (bradykinin; not seen with ARB) Teratogen (fetal renal malformations) Creatinine ^ (decreased GFR due to efferent art. dilation) Hyperkalemia Hypotension
1031
What do integrins bind in the ECM?
Collagen Fibronectin Laminin
1032
How is fructose dealt with in patients that have essential fructosuria?
Fructose is converted to Fructose-6-P by hexokinase. This can then participate in glycolysis or glycogenesis. This is a minor pathway in normal individuals but becomes important in the setting of fructokinase deficiency.
1033
Where should thoracentesis be performed?
``` Midclavicular = 5th-7th rib Midaxillary = 7th-9th rib Paravertebral = 9th-11th rib ``` This is the location where there is pleura but no lung. It should always be performed above a rib so as not to damage the van driving under the rib.
1034
What are found in the granules of platelets?
Dense granules: ADP, Ca2+ alpha-granules: vWF, fibrinogen
1035
What are the relative abundancies of the various leukocytes?
``` Neutrophils Lymphocytes Monocytes Eosinophils Basophils ```
1036
What coagulation factors require Vitamin K?
II, VII, IX, X, C, S
1037
What is the mechanism of warfarin?
Inhibits epoxide reductase. | Normall reduces Vit. K so that it can act as a cofactor in factors II, VII, IX, X, C, S
1038
What can cause decrease in the ESR?
``` Polycythemia Sickle cell CHF Microcytosis Hypofibrinogenemia ```
1039
What can cause spherocytes on a peripheral blood smear?
Hereditary spherocytosis | Autoimmune hemolysis
1040
What can cause target cells on a peripheral blood smear?
HALT HbC disease Asplenia Liver disease Thalassemia
1041
What can cause Howell-Jolly bodies on peripheral blood smear?
Asplenia Functional asplenia Naphthalene ingestion (mothballs)
1042
What are the microcytic hypochromic anemias?
``` MCV < 80 Iron deficiency Anemia of chronic disease (late) Thalassemias Lead poisoning Sideroblastic anemias ```
1043
What is seen with Plummer-Vinson syndrome?
Plummer-Vinson triad: Iron-deficiency anemia Esophageal webs Atrophic glossitis
1044
What is seen in alpha-thalassemia?
4 gene deletion --> Hb Barts --> hydrops fetalis (death in-utero) 3 gene deletion --> HbH disease anemia 1-2 gene deletion --> asymptomatic
1045
What causes alpha-thalassemia?
Deletion of the alpha-globin genes. cis-deletion in Asians trans-deletion in Africans cis-deletion is associated with higher rates of hydrops fetalis in offspring.
1046
What is seen in beta-thalassemia?
Thalassemia minor (heterozygote): Asymptomatic Dx confirmed by HbA2 > 3.5% Thalassemia major (homozygote): Severe anemia requiring transfusions (hemochromatosis) Marrow expansion --> HSM, crew cut x-ray, chipmunk facies ^HbF & HbA2 Parvovirus infection --> aplastic crisis
1047
What is affected by lead poisoning? | What are the symptoms?
ALA dehydratase & Ferrochelatase are inhibited (heme synth) ``` Symptoms: LEEAADD: Lead lines on gingiva & metaphyses of long bones Encephalopathy Erythrocyte stippling Anemia (sideroblastic) Abdominal colic Drops (wrist & foot) Dimercaprol & EDTA first line ```
1048
What causes Sideroblastic anemia?
Defects in heme synthesis: Hereditary - X-linked defect in ALA Synthase Acquired - EtOH, lead poisoning, INH (B6 def.)
1049
How can Folate vs. B12 deficient megaloblastic anemia be differentiated?
B12 deficiency: ^Methylmalonic acid Subacute combined degeneration
1050
What are the causes of macrocytic anemia?
Megaloblastic: B12 def. Folate def. Orotic aciduria Non-megaloblastic: Alcoholism Liver disease Reticulocytosis
1051
What are the lab findings in extravascular vs. intravascular hemolysis?
Intravascular: ^LDH, depleted haptoglobin, Hb in urine Extravascular: ^LDH, ^unconjugated bilirubin
1052
What causes anemia of chronic disease? | What do iron studies show?
Chronic inflammation --> ^Hepcidin --> sequestration of iron in intestinal mucosa & macrophages --> normocytic anemia that progresses to microcytic. Decreased iron, decreased TIBC, increased ferritin
1053
What can cause aplastic anemia?
Drugs (chloramphenicol, alkylating agents, antimetabolites) Viruses (Parvovirus, EBV, HIV, HCV) Fanconi's anemia
1054
What is the formula for corrected reticulocyte count?
Corrected reticulocytes = (Absolute reticulocytes)*(HCT/45) If corrected > 3% --> adequate response If < 3% --> issue with the bone marrow
1055
What are the intravascular hemolytic anemias?
``` G6PD deficiency Paroxysmal nocturnal hemoglobinuria Cold agglutinins Microangiopathic anemia Macroangiopathic anemia Infections (malaria, Babesia) ```
1056
What are the extravascular hemolytic anemias?
``` Hereditary spherocytosis G6PD deficiency Pyruvate kinase deficiency Sickle cell anemia Warm agglutinins ```
1057
What causes hereditary spherocytosis? | What is seen?
Defect in RBC cytoskeletal anchoring proteins (ankyrin, spectrin) --> small pieces of membrane removed by splenic macs ``` Symptoms: Splenomegaly Aplastic crisis (Parvovirus) Small, round RBC's with no central pallor ^MCHC, ^ RDW Positive osmotic fragility test ```
1058
What is seen on a blood smear of a G6PD patient?
Heinz bodies | Bite cells
1059
How does pyruvate kinase deficiency present?
(AR) Hemolytic anemia in a newborn. Decreased ATP causes rigid RBC's
1060
What causes HbC disease? | What is seen?
AR mutation of beta chain. Glutamic acid --> LyCine at residue 6. Symptoms: Mild extravascular hemolytic anemia HbC crystals on blood smear (rectangular)
1061
What causes PNH? | What is seen?
Impaired formation of GPI anchors or DAF deficiency. It is an ACQUIRED mutation in a HSC line. ``` Symptoms: Pancytopenia Numerous DVT's; hepatic/portal/cerebral vein thromboses Hemolytic anemia Flow cytometry - CD55/59 negative RBC's 10% develop AML ```
1062
What is the treatment for PNH?
Eculizumab - C5 complement inhibitor
1063
What causes sickle cell anemia?
HbS point mutation in beta chain Glutamic acid --> Valine at position 6. Low O2, dehydration, or acidosis --> polymerization of deoxygenated HbS --> Sickling
1064
What is seen in sickle cell trait?
Resistance to malaria | Microinfarctions in kidney medulla --> eventual inability to concentrate urine
1065
What complications are seen with sickle cell anemia?
``` Salmonella osteomyelitis Autosplenectomy --> Infection with SHiNE SKiS (death in kids) Painful crises: --Dactylitis --Acute chest syndrome (death in adults) --Avascular necrosis Aplastic crisis (Parvovirus) Renal papillary necrosis ```
1066
What is the treatment for sickle cell anemia?
Hydroxyurea --> HbF | Bone marrow transplant
1067
What can trigger candida vaginitis?
``` Antibiotic use! Contraceptives Systemic corticosteroids Diabetes Immunosuppression ```
1068
What are the important glucose transporters and their locations?
``` GLUT-1 = insulin-independent; RBC's, brain GLUT-2 = bidirectional; Beta cells, liver, kidney small intestine GLUT-4 = insulin-dependent; Adipocytes, muscle cells ```
1069
What cancers are seen in Li-Fraumeni syndrome?
``` Early-onset cancers: Breast Brain Adrenal cortex Sarcomas Leukemias ```
1070
What is the epithelial cell type along the female reproductive tract?
Vagina = nonkeratinized stratified squamous Ectocervix = stratified squamous Endocervix = simple columnar Uterus = simple columnar; pseudostratified glands Fallopian tube = ciliated columnar Ovary = simple cuboidal
1071
How can tumor lysis syndrome be avoided?
It is due to ^^uric acid following rapid killing of tumor cells. Methods: Hydration Urine alkalinization Allopurinol
1072
What are the adverse effects of methotrexate?
Stomatitis Hepatotoxicity (hepatitis, fibrosis, cirrhosis) Myelosuppression
1073
What can cause autoimmune hemolytic anemia?
Warm agglutinins (IgG): SLE CLL Drugs (methyldopa) Cold agglutinins (IgM): CLL Mycoplasma pneumoniae Mononucleosis
1074
What normal process can increase TIBC?
Pregnancy or OCP use
1075
What substrates are used to produce heme?
Glycine Succinyl-CoA Iron (Fe2+) **B6 is required for the first step
1076
Where in the cell does heme synthesis occur?
First & last step in the mitochondria. The middle is in the cytosol.
1077
What accumulates in the blood following lead poisoning?
ALA (aminolevulonic acid) | Protoporphyrin
1078
What are the symptoms of Acute Intermittent Porphyria?
5 P's: Painful abdomen Port-wine colored urine (after it stands a while) Polyneuropathy Psychological disturbances Precipitated by drugs (CYP450 inducers --> ^Heme production)
1079
What causes Acute Intermittent Porphyria? | What accumulates?
Caused by Porphobilinogen deaminase deficiency Porphobilinogen, ALA, & uroporphyrin accumulate
1080
What is the treatment for acute intermittent porphyria?
Heme & Glucose --> both inhibit ALA synthase
1081
What causes Porphyria cutanea tarda? What accumulates? What is seen?
Uroporphyrinogen decarboxylase deficiency Uroporphyrin builds up Symptoms: Blistering cutaneous photosensitivity later on in life (cutanea tarda) Tea colored urine *The most common porphyria
1082
What are the qualitative platelet disorders & their causes?
Bernard-Soulier syndrome = GPIb defect (adhesion) Glanzmann's thrombasthenia = GP IIb/IIIa defect (aggregation) *BS = big suckers (PLT's turned over faster --> low PLT count & larger platelets)
1083
What is the cause of ITP?
Autoimmune Ab's against GPIIb/IIIa --> splenic consumption of platelets Tx: Steroids, IVIg, splenectomy (refractory)
1084
What is the cause of TTP & HUS?
TTP: ADAMTS13 deficiency (acquired via autoimmunity or congenital) --> circulating ^vWF multimers --> ^PLT aggregation & thrombosis HUS: Verotoxin damage of endothelium --> PLT aggregation & thrombosis
1085
What is seen with TTP-HUS?
``` Altered mental status Acute kidney injury Thrombocytopenia Microangiopathic hemolytic anemia Fever ```
1086
What is seen in von Willebrand's disease? | What is the treatment?
Autosomal dominant deficiency in vWF: ^bleeding time & ^PTT Abnormal ristocetin assay Tx: Desmopressin (DDAVP) --> ^vWF release from W-P bodies
1087
What are the causes of DIC?
"STOP Making New Thrombi Stupid" ``` Sepsis (gram-) Trauma Obstetric complications (tissue thromboplastin in amniotic fluid) Pancreatitis (acute) Malignancy Nephrotic syndrome Transfusion Snakebites (rattlesnake) ```
1088
What is the most common inherited bleeding disorder? | Hypercoagulable disorder?
Bleeding - vWD Hypercoagulable - Factor V Leiden (especially whites)
1089
What are the inherited thrombotic syndromes?
``` Factor V Leiden Prothrombin mutation (3' UTR mut. --> ^^production) Antithrombin deficiency (normalizes with heparin) Protein C or S deficiency (^coumadin-induced-necrosis) ```
1090
How can CML be differentiated from a leukemoid reaction?
``` CML: Myeloid:Erythroid > 10:1 Alkaline phosphatase negative ^Basophils t[9;22] ```
1091
What are the symptoms of Hodgkin's Lymphoma?
Localized group of LAD | "B" symptoms (fever, night sweats, weight loss)
1092
Reed-Sternberg cells | Diagnosis?
"Owl's eye" nuclei = mirror image bi-lobed or bi-nucleated with nucleolus. CD15+ & CD30+ Usually surrounded by reactive lymphocytes. Hodgkin's Lymphoma
1093
What types of Hodgkin's lymphoma have a better/worse prognosis?
Lymphocyte rich --> better prognosis | Lymphocyte depleted --> worse prognosis
1094
Popcorn cells | Diagnosis?
Nodular lymphocyte predominant hodgkin's lymphoma (NLPHL) Different from nodular sclerosing type (most commen; women = men)
1095
What type of lymphoma has a bimodal age distribution?
Hodgkin's = young adulthood & then > 55y Non-hodgkin's = 20-40y
1096
Lacunar cells | Diagnosis?
Reid-Sternberg cells that appear to be in a lake because there is a space around them. Nodular sclerosing hodgkin's lymphoma
1097
How can bands be distinguished by flow cytometry?
They have decreased CD16 (FcR) on their surface.
1098
What are the non-Hodgkin's lymphomas?
``` B-cell: Burkitt's Diffuse large B-cell Mantle cell Follicular ``` T-cell: Adult T-cell Mycosis fungoides/Sezary syndrome
1099
t(8;14) | Diagnosis?
Burkitt's lymphoma c-myc activation
1100
"Starry sky" appearance
Burkitt's lymphoma Sheets of basophilic B-cells with interspersed macrophages.
1101
What mutation is seen in mantle cell lymphoma? | What cell-marker is seen?
t(11;14) - Cyclin D1 translocated to Ig heavy chain location CD5+
1102
t(14;18) | Diagnosis?
Follicular lymphoma & DLCL Translocation of Ig heavy chain and bcl-2.
1103
How does adult T-cell lymphoma present?
Rash & lytic bone lesions Caused by HTLV-1 (seen in Japan, Africa, Caribbean)
1104
What can cause marginal zone lymphoma?
Marginal zone only seen in chronic inflammatory conditions: MALToma Hashimoto's Sjogren's
1105
T-cells with Cerebriform nuclei | Diagnosis?
Sezary syndrome (systemic mycosis fungoides) Mycosis fungoides --> Pautrier microabscesses in skin
1106
What are the symptoms of multiple myeloma?
``` CRAB: hyperCalcemia Renal insufficiency Anemia Bone lytic lesions --> back pain ```
1107
What is seen on labs with multiple myeloma? | What are the risks?
Punched out lytic bone lesions on x-ray M-spike on electrophoresis (IgG > IgA) Bence-Jones protein in urine (Ig light chain) Rouleaux formation Many plasma cells with "clock face" chromatin Risks: Susceptibility to infection (most common COD) Primary amyloidosis (AL)
1108
What is seen with Waldenstrom's macroglobulinemia?
M spike of IgM (macro) --> hyperviscosity symptoms only
1109
What is asymptomatic multiple myeloma?
Monoclonal Gammopathy of Undetermined Significance (MGUS) 1% chance per year of --> MM
1110
What are the symptoms of leukemia in general?
Marrow failure: Anemia (RBC's) Infection (WBC's) Hemorrhage (PLT's)
1111
What type of leukemia is seen in kids?
ALL
1112
What is the cell marker of ALL?
Acute Lymphoblastic Leukemia/Lymphoma All ALL are TdT+ ``` Pre-B = CD10/19/20+ Pre-T = CD1/2/5+ ```
1113
What is seen on a CLL/SLL peripheral blood smear? | How does it present?
Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma Smudge cells on peripheral smear Asymptomatic or autoimmune hemolytic anemia
1114
What cell marker is used for hair cell leukemia?
TRAP (tartrate-resistant acid phosphatase) "TRAP the hairy animal" It remains TRAPPED in red pulp (spleen) & bone marrow --> no LAD
1115
What is the treatment for hairy cell leukemia?
Cladribine It is a purine analog that inhibits ADA.
1116
How does APL present? | What is seen on peripheral smear?
APL (M3 subtype of AML) often presents with DIC because Auer rods (present on peripheral smear; crystallized MPO) can induce DIC.
1117
t(15;17) | Diagnosis?
M3 subtype of AML = APL Treat with ATRA
1118
t(9;22) | Diagnosis?
Chronic Myelogenous Leukemia (CML) t(9;22) = bcr-abl fusion
1119
How does CML present? | How is it treated?
^Basophils, neutrophils, metamyelocytes Splenomegaly May transform into AML > ALL Responds to Imatinib
1120
What leukemias are seen at increased rates in Down syndrome?
Before 5y --> Acute megakaryoblastic leukemia After 5y --> ALL
1121
What is seen in Langerhans histiocytosis?
Proliferation of dendritic cells --> lytic bone lesions & skin rash Birbeck granules (tennis rackets) S-100+ & CD1a+
1122
How does polycythemia vera present?
^RBC's, ^WBC's, ^PLT's DVT's #1 cause of bud-chiari Itching after a hot shower (^mast cells)
1123
Dacrocyte | Diagnosis?
Dacrocyte = teardrop cell --> Myelofibrosis "The marrow is crying because it's fibrosed" It is its own chronic myeloproliferative disorder and can also be seen late in polycythemia vera.
1124
What can cause inappropriate production of EPO?
``` Renal cell carcinoma Wilms' tumor Renal cysts HCC Hydronephrosis ```
1125
What is the mechanism of heparin?
Cofactor for Antithrombin --> inactivates Xa & IIa
1126
What is the mechanism of HIT?
Heparin-induced thrombocytopenia IgG Ab's against Hepatin-Platelet factor 4 --> Ab-Heparin-PF4 complex activates platelets --> thrombosis & thrombocytopenia
1127
What is Lepirudin?
Lepirudin & Bivalirudin are thrombin inhibitors used as an alternative to heparin following HIT
1128
What is the treatment for thrombolytic overdose?
Aminocaproic acid
1129
What are the thrombolytic drugs? What is their mechanism When are they contraindicated?
Alteplase (tPA), reteplase (rPA), tenecteplase (TNK-tPA) They cause activation of plasminogen --> plasmin --> cleaves thrombin & fibrin clots & fibrinogen Contraindicated in active bleeding, Hx of intracranial bleeding, severe HTN.
1130
What is the mechanism of aspirin? | What are its toxicities?
Irreversible COX-1 & COX-2 inhibitor. Endothelial cells can resynthesize COX-2 --> PGI2. Platelets cannot resynthesize COX-1 --> decreased TXA2. Toxicity: Gastric ulcers Chronic use - AKI, interstitial nephritis, UGI bleeds Reye's syndrome in post-viral children Overdose --> Resp. alkalosis --> eventual metabolic acidosis
1131
What are the ADP receptor inhibitors? | What are their toxicities?
Clopidogrel, Ticlopidine, Pasugrel, Ticagrelor Irreversibly bind ADP receptors --> PLT's don't activate or display GPIIb/IIIa --> cannot aggregate Ticlopidine --> neutropenia (Presents w/ fever & stomatitis)
1132
What are the Xa inhibitors? | IIa inhibitors?
Xa inhibitors have X in the name: Apixaban Rivaroxaban Idraparinux IIa inhibitors have a G in the name: Argatroban Dabigatran
1133
What are the phosphodiesterase III inhibitors? | What is their mechanism?
PDEIII inhibitors = Cilostazol, Dipyridamole PDE III inhibition --> ^cAMP --> PLT aggregation inhibited & vasodilation Used for intermittent claudication, angina prophylaxis
1134
What are the GPIIb/IIIa inhibitors?
GPIIb/IIIa inhibitors = Abciximab, Eptifbatide, Tirofiban Used for angioplasty & ACS
1135
What is the mechanism of Cytarabine?
Pyrimidine analog --> inhibitor of DNAP Can cause pancytopenia
1136
What is the mechanism of Dactinomycin? | What is its toxicity?
Dactinomycin intercalates into DNA Used for childhood tumors! (Wilms', Ewing's sarcoma, rhabdomyosarcoma) Can cause myelosuppression
1137
What is the mechanism of Doxorubicin? | What are its toxicities?
Doxorubin/Daunomycin intercalate into the DNA & inhibit TopoII Toxicities: Cardiotoxic (prevented with Dexrazoxane) Vesicant Myelosuppression
1138
What is the mechanism of Bleomycin? | What are its toxicities?
Bleomycin induces free-radical formation --> dsDNA breaks Toxicities: Pulmonary fibrosis Skin hyperpigmentation
1139
What is the mechanism of Cyclophosphamide? | What are its toxicities?
Cyclophosphamide/Ifosphamide are alkylating agents --> Crosslink DNA at guanine N-7 position Toxicities: Hemorrhagic cystitis (Mesna prevents) Myelosuppression Sterility
1140
What are the nitrosureas? | What is their use?
Carmustine, Lomustine, Semustine, Streptozocin They are alkylating agents that can cross the BBB --> used for brain tumors. They can cause CNS toxicities (ataxia, dizziness)
1141
What is the mechanism of Busulfan? | What are its toxicities?
Busulfan is an alkylating agent used for CML & ablation of bone marrow prior to BMT Toxicities: Pulmonary fibrosis Skin hyperpigmentation
1142
What is the mechanism of Vincristine? | What are its toxicities?
Vincristine & Vinblastine block polymerization of microtubules --> inhibit mitotic spindle formation. Toxicities: Vincristine - peripheral neuropathy Vinblastine - bone marrow suppression (blasts bone marrow)
1143
What is the mechanism of Cisplatin? | What are its toxicities?
Cisplatin, Carboplatin, & Oxaliplatin --> crosslink DNA Cisplatin - Nephrotoxic & ototoxic Carboplatin - Myelosuppression Oxaliplatin - Paresthesias & cold sensitivity
1144
What is the mechanism of Hydroxyurea? | What are its toxicities?
Hydroxyurea inhibits ribonucleotide reductase (UDP --> dUDP) Toxicities: Bone marrow suppression
1145
What is the mechanism of Imatinib? | What are its toxicities?
Imatinib is a bcr-able tyrosine kinase inhibitor Used in CML & GIST Toxicity: Fluid retention
1146
What is the mechanism of Rituximab? | What is it used for?
mAb against CD20 Used in Non-hodgkin's lymphoma, RA
1147
What is the mechanism of Vemurafenib? | What is its use?
Small molecule inhibitor of B-raf kinase with V600E mutation Used to treat metastatic melanoma
1148
What is the mechanism of Bevacizumab?
mAb against VEGF --> inhibits angiogenesis
1149
What bugscause bacteremia in a sickle cell patient?
Strep pneumo > H. flu They are functionally asplenic --> encapsulated organisms Also don't forget that they get Salmonella osteomyelitis
1150
What types of collagen are defective/deficient in inherited diseases?
Type I --> Osteogenesis imperfecta Type II Type III --> Ehlers-Danlos Syndrome Type IV --> Alport Syndrome
1151
``` How many calories are present in 1 gram of: Protein Fat Carbohydrate Ethanol ```
Protein & Carbs = 4 cal Fat = 9 cal Ethanol = 7 cal
1152
What is Clozapine used for? | What toxicities are seen with it?
It is an atypical antipsychotic used when other antipsychotics have all failed (it's the big gun). Toxicities: Agranulocytosis! Significant weight gain Seizures
1153
When are ACE inhibitors contraindicated?
Bilateral renal artery stenosis (these pts are dependent on ACE-mediated constriction of efferent arteriole to maintain GFR) --> ARF if administered Also Pregnancy (teratogen)
1154
What are the layers of the epidermis?
"Cancel Lab, Get Some Beer" ``` Corneum Lucidum (only present on thick skin) Granulosum Spinosum Basale ```
1155
Where are apocrine glands found?
Armpits, genitals, and nipples Secrete milky viscous fluid. Stinks because of bacteria.
1156
What are the epithelial cell junction types & their compositions?
Tight junctions = Claudins & Occludens Adherens junctions = Cadherins (link to actin) Desmosomes = Desmogleins (link to keratin) Gap junctions = Connexins form Connexons Hemidesmosomes (bind basement membrane) Integrins (bind laminin in BM)
1157
What landmark is used for a pudendal nerve block? | A lumbar puncture?
Pudendal n. block (childbirth) --> Ischial spine Lumbar puncture --> Iliac crest
1158
What are the rotator cuff muscles & their functions?
SITS: Supraspinatus --> initial abduction (most common RC injury) Infraspinatus --> laterally rotates arm (pitching injury) Teres minor --> adducts & laterally rotates Subscapularis --> adducts & medially rotates
1159
What nerve is affected by this injury? | Humerus surgical neck fracture or dislocation
Axillary nerve --> deltoid atrophy & numbness over deltoid
1160
What nerve is affected by this injury? | Subluxation of radius
Radial nerve (deep branch) --> wrist drop & numb dorsal hand/snuffbox
1161
What nerve is affected by this injury? | Midshaft humerus fracture
Radial nerve --> wrist drop & numb posterior hand/snuffbox
1162
What nerve is affected by this injury? | Supracondylar humerus fracture
Proximal Median nerve --> Pope's blessing & lateral hand numbness
1163
What nerves contribute to the branches of the brachial plexus?
C5, C6, C7, C8, T1 "The 3 Musketeers Assasinated 5 Rats, 5 Mice, & 2 Unicorns" ``` Musculocutaneous - C5,6,7 Axillary - C5,6 Radial - C5,6,7,8,T1 Median - C5,6,7,8,T1 Ulnar - C8,T1 ```
1164
What nerve is affected by this injury? | Lunate dislocation
Distal median nerve --> median claw (can't extend 2 & 3)
1165
What can cause distal ulnar injury? | What is seen?
Fractured hook of the hamate --> distal ulnar claw (cannot extent 4th & 5th digits)
1166
What causes Erb-Duchenne palsy?
Waiter's tip - caused by torn upper trunk of brachial plexus (C5,6) seen in infants following delivery
1167
What causes a Klumpke claw?
Torn lower trunk of brachial plexus (C8, T1). This can occur by pulling a baby out by its arms, falling and catching oneself on a tree branch, or thoracic outlet syndrome.
1168
What causes thoracic outlet syndrome? | What is seen?
Compression of the brachial plexus & subclavian vessels between the anterior & middle scalene (superior thoracic outlet). ``` Symptoms: Atrophy of intrinsic hand muscles Pain/paresthesias Decreased pulses in the arm Edema ```
1169
What carpal injuries can occur by falling on an outstretched hand? What are the complications?
Fractured scaphoid --> avascular necrosis Dislocated lunate --> acute carpal tunnel syndrome (median n.) Fractured hook of hamate --> ulnar n. injury
1170
When does clawing of the hand occur?
Clawing occurs with more distal lesions, because the deficit is pronounced even at rest. More proximal lesions present with deficits only when the muscle is used.
1171
What common drugs have antimuscarinic effects?
``` Atropine TCA's H1 blockers (diphenhydramine, etc.) Neuroleptics Antiparkinsonian drugs ```
1172
What are the contraindications of metformin?
``` Renal failure Liver failure CHF Alcoholism Sepsis ``` Anything that could predispose to lactic acidosis
1173
What is cataplexy?
Sudden loss of motor tone triggered by emotion. This is seen in narcolepsy.
1174
What substance is deficient in the CSF in narcolepsy?
Hypocretin-1 & Hypocretin-2 (Orexin-A & Orexin-B) Produced in the lateral hypothalamus, they promote wakefulness.
1175
What side effects are seen with low potency first generation antipsychotics? High potency?
Low potency --> blockade of: Cholinergic receptors --> anticholinergic Histamine receptors --> sedation Noradrenergic receptors --> orthostatic hypotension High potency: Extrapyramidal symptoms
1176
What receptors are antagonized by TCA's?
Histamine --> sedation Alpha adrenergic --> orthostatic hypotension Cholinergic --> anticholinergic effects
1177
What must be monitored in a pt on thiazolidinediones?
A pt on TZD's (Rosiglitazone, etc.) must have their LFT's monitored.
1178
What adverse effects are seen with Foscarnet?
Foscarnet binds the pyrophosphate binding site on viral RNA polymerase. Second line for CMV retinitis & HSV. Adverse effects: Nephrotoxicity Hypocalcemia Hypomagnesemia
1179
What are the long, intermediate, & short acting insulins?
Short: Lispro, Aspart, Glulisine Intermediate: NPH > Regular insulin Long: Glargine Detemir
1180
What is the equation for physiologic dead space?
Physiologic DS = anatomic DS + V/Q mismatch DS = TV*(PaCO2-PeCO2)/PaCO2
1181
What risks are associated with Common Variable Immunodeficiency?
Increased risk of autoimmune disease & lymphoma
1182
What is the outcome of vertical transmission of HBV?
Immature infant immune system: Mild initial illness High chance of chronic infection
1183
What is the treatment for toxoplasmosis?
Sulfadiazine & Pyrimethamine
1184
What are the most common causes of focal brain lesions in HIV patients?
Toxoplasma | Primary CNS lymphoma
1185
What is used to treat hypertension in pregnant women?
Methyldopa
1186
What is the adenoma-to-carcinoma progression of CRC?
Normal colon --> APC mutation --> Proliferation --> K-RAS mutation --> Adenoma --> p53 mutation --> Carcinoma Order is AK-53
1187
What is considered fever? What is dangerous to brain tissue? What causes death?
Fever > 101.0 (38.3) Neurological sequelae = 42 degrees Death = 43 degrees
1188
What is hyperpyrexia? | What is the treatment for hyperpyrexia?
Hyperpyrexia is a temperature > 40 degrees Treatment is NSAIDs/Acetaminophen & physical cooling of the body.
1189
What are the risk factors for cervical cancer?
Multiple sexual partners Cigarette smoking Early coitarche Low socioeconomic status
1190
What drugs improve mortality in CHF patients?
ACE inhibitors Beta blockers (unless decompensated) ARB's Spironolactone
1191
What is seen with left-sided CHF?
Pulmonary edema Paroxysmal nocturnal dyspnea Orthopnea Hemosiderin-laden macrophages
1192
What is seen with right-sided CHF?
Chronic passive congestion --> hepatosplenomegaly (nutmeg liver) Peripheral dependent pitting edema JVD
1193
What causes acute bacterial endocarditis? | Subacute?
Acute = S. aureus --> large vegetations on a normal valve Subacute = Viridans strep --> small vegetations on congenitally abnormal or diseased valves
1194
What can cause culture-negative endocarditis?
``` Malignancy Hypercoagulability Lupus (Marantic/Libman-Sacks) HACEK organisms: -Hemophilus -Actinobacillus -Cardiobacterium -Eikenella -Kingella ```
1195
What are the signs/symptoms of bacterial endocarditis?
bacteria FROM JANE ``` Fever Roth's spots (IC mediated) Osler's nodes (Painful; IC mediated) Murmur Janeway lesions (Painless; septic emboli) Anemia (of chronic disease) Nail bed hemorrhage Emboli ```
1196
What complications can result from bacterial endocarditis?
Chordae rupture Glomerulonephritis Suppurative pericarditis Emboli
1197
How is diagnosis of rheumatic fever made?
JONES criteria Joints (migratory polyarthritis) s chorea Minor criteria: Fever, ^ESR
1198
What is seen early in chronic rheumatic heart disease? | Late?
Early death = myocarditis Rheumatic heart disease affects Mitral > Aortic >> Tricuspid Early lesion - mitral regurgitation Late lesion - mitral stenosis
1199
What is seen histologically with rheumatic heart disease?
``` Aschoff bodies (granulomas) Anitschkow's cells (activated histiocytes) ``` Caused by a Type II hypersensitivity reaction from Ab's against M protein.
1200
What is seen with acute pericarditis?
Sharp pain (worse w/ inspiration, better w/ leaning forward) Friction rub ST elevation in all leads
1201
What are the types of pericarditis & their causes?
Fibrinous - Dressler's syndrome, uremia, radiation Serous - viral pericarditis, inflammatory diseases (SLE, RA, etc.) Suppurative - bacterial pericarditis (rare due to abx)
1202
What is seen with cardiac tamponade?
``` Beck's triad: -Hypotension -JVD -Distant heart sounds Pulsus paradoxus Electrical alternans ``` Pulsus paradoxus - systolic BP decreases by at least 10 mmHg upon inspiration
1203
What can cause pulsus paradoxus?
Pulsus paradoxus - systolic BP decreases by at least 10 mmHg upon inspiration ``` Caused by: Cardiac tamponade Pericarditis Asthma Obstructive sleep apnea Croup ```
1204
What cardiac tumors are seen?
Myxoma - Adults; ball-valve obstruction in the LA (syncope) Rhabdomyoma - Kids; associated with tuberous sclerosis Metastasis - Often to pericardium (effusion). Melanoma, lymphoma
1205
What is Kussmaul's sign? | What is it seen in?
^ in JVP upon inspiration rather than a decrease ``` Seen in: Constrictive pericarditis Restrictive cardiomyopathy RA or RV tumors Cardiac tamponade ```
1206
Who is classically affected by Takayasu's arteritis?
Asian females under 40
1207
What is seen in Takayasu's arteritis?
"Pulseless disease" (weak UE pulses) Visual & neurologic symptoms ^ESR Basically the same disease as Giant Cell arteritis but occurs at the aortic arch & proximal great vessels.
1208
What is seen with Polyarteritis Nodosa?
``` HBV seropositivity Fever, weight loss, Abdominal pain w/ melena Hypertension Renal damage Neurologic symptoms (wrist/foot drop) "String of pearls" on arteriogram Fatal if untreated ``` SPARES THE LUNGS
1209
Who is classically affected by polyarteritis nodosa? | What is seen histologically?
Young adults Transmural inflammation with fibrinoid necrosis
1210
What is seen in Kawasaki disease?
``` Fever Cervial LAD Conjunctivitis Strawberry tongue (mucocutaneous LN syndrome) Desquamating rash on palms & soles ``` Can lead to coronary MI or aneurysm/rupture
1211
What is the treatment for Kawasaki disease?
IVIg & high-dose aspirin
1212
What autoAb's are seen in Kawasaki disease?
Anti-endothelial or anti-smooth muscle Ab's
1213
What is seen with microscopic polyangiitis?
Hemoptysis RPGN Palpable purpura p-ANCA's NO granulomas, NO eosinophilia
1214
What is seen with Granulomatosis & Polyangiitis?
Wegener's ``` Granulomas Sinusitis/Nasopharyngeal ulceration Hemoptysis (CXR shows large nodular densities) RPGN c-ANCA's ```
1215
What is seen with Churg-Strauss syndrome?
``` Asthma, sinusitis Palpable purpura Peripheral neuropathy Granulomas with eosinophilia RPGN p-ANCA ^IgE ```
1216
What is seen with Henoch-Shonlein purpura?
``` Most common systemic childhoos vasculitis Follows an URI Palpable purpura on buttocks/legs Arthralgia Abdominal pain w/ melena IgA nephropathy ```
1217
What are c-ANCA's & p-ANCA's against?
c-ANCA = PR3-ANCA p-ANCA = MPO-ANCA
1218
What is seen with a strawberry hemangioma?
Benign capillary hemangioma of infancy. | Grows rapidly and regresses spontaneously by 5-8y
1219
What is seen with a cherry hemangioma?
Benign capillary hemangioma of the elderly | Does not regress
1220
What is a pyogenic granuloma?
Polypoid capillary hemangioma that can ulcerate & bleed. | Associated with trauma & pregnancy
1221
What is a cystic hygroma?
Cavernous lymphangioma of the neck | Associated with Turner syndrome
1222
What is a Glomus tumor?
Benign, painful red/blue tumor under the fingernail | Arises from glomus body
1223
What is a bacillary angiomatosis?
Benign capillary lesion found in AIDS patients Caused by Bartonella henselae infection Looks like Kaposi's sarcoma
1224
Where are angiosarcomas typically found?
Head, neck, & breast following radiation | Liver due to PVC, arsenic, & Thorotrast exposure
1225
What causes Kaposi's sarcoma?
Endothelial malignancy of skin, mouth, GI, & respiratory tract Caused by HHV-8 & HIV together Seen in old Eastern European males, HIV pts, & transplant pts
1226
What is seen with Sturge-Weber disease?
Port wine stain on face (trigeminal distribution) Ipsilateral (to port wine stain) leptomeningeal angioma Seizures Early-onset glaucoma It is a vascular disorder that affects the small vessels.
1227
What mutation causes Von Hippel-Lindau disease? | What neoplasms are seen in Von Hippel-Lindau disease?
Autosomal dominant deletion of VHL gene on chromosome 3p Cerebellar hemangioblastomas Clear cell renal carcinoma Pheochromocytomas
1228
What causes "housemaid's knee?"
Prepatellar bursitis. Seen in patients who do a lot of kneeling.
1229
How many organisms are required to establish cholera?
Usually 10^10 Anything that decreases gastric acid can cause susceptibility (PPI's, food ingestion, achlorhydria)
1230
What is the most common cause of aortic stenosis?
Senile calcific aortic stenosis Presents in 70's or earlier if bicuspid aortic valve is present.
1231
What are the triggers for Gilbert hyperbilirubinemia?
``` Fasting Stress Hemolysis Physical exertion Febrile illness Fatigue ```
1232
From where do the bronchial arteries arise? | Where do they empty?
Arise from the descending thoracic aorta. Most of the venous blood is returned via the pulmonary veins. This accounts for part of the A-a gradient.
1233
What type of glucose are the GLUT transporters selective for?
They are somewhat selective for D-glucose
1234
What are the insertion points of the SCM?
Sterno --> Sternum Cleido --> Clavicle (medial portion) Mastoid --> Mastoid process
1235
What drug is used to prevent cisplatin nephrotoxicity? | What is its mechanism?
``` Amifostine scavenges free radicals. Chloride diuresis (IV NaCl) is also used to keep cisplatin in a nonreactive state while in the kidney. ```
1236
What drug is given to prevent Doxorubicin/Daunomycin cardiotoxicity?
Dexrazoxane (iron chelating agent)
1237
What is the "female athlete triad"?
Eating disorder Amenorrhea Osteoporosis
1238
What is caused by rat poison? | What is the antidote?
Rat poison is like a long-acting warfarin. Thus, the treatment to prevent bleeding is fresh frozen plasma.
1239
What drug is used to treat bradycardia secondary to RCA infarction? What is a possible acute side effect?
Atropine can be used to block vagal outflow to the SA & AV nodes. This can cause acute closed-angle glaucoma in susceptible patients. It is absolutely contraindicated in a patient with preexisting glaucoma.
1240
How is congenital adrenal hyperplasia treated?
Low dose corticosteroids ---> inhibition of ACTH --> suppression of HPA axis
1241
Where do Cushing ulcers arise? | Curling ulcers?
Cushing ulcers can be in the esophagus, stomach, or duodenum. They arise due to ^ICP. Curling ulcers are found in the proximal duodenum only. They arise due to severe trauma/burns --> decreased oxygenation
1242
What can cause gallbladder hypomotility?
Pregnancy Rapid weight loss Long-term TPN or octreotide High spinal cord injuries
1243
Why is spironolactone beneficial in CHF patients?
It is likely due to inhibition of ventricular remodeling & cardiac fibrosis (normally facilitated by aldosterone).
1244
What are the effects of nitroprusside?
It is a balanced arterial & venodilator. This causes decreased preload and afterload.
1245
What are the eukaryotic promoters?
CAAT (-75) & TATA (-25)
1246
What are the advantages of COX 2 inhibitors?
Less risk of bleeding Less GI ulceration They have a higher risk of clotting though.
1247
What nerve injury is seen with subluxation of the radial head?
Damage to the deep branch of the radial nerve --> wrist drop without sensory deficit
1248
What is the function of P-glycoprotein?
It is an ATP-driven efflux pump that is often upregulated in tumor cells & can cause resistance to chemotherapy.
1249
What is the mechanism of shiga toxin?
Alter the 60S ribosomal subunit to prevent tRNA binding to the ribosome --> inhibit translation
1250
What is the most common risk factor for native valve endocarditis in the US?
Mitral valve prolapse
1251
What are the sizes of petechiae, purpura, & ecchymoses?
Petechiae < 5mm 5 mm < Purpura < 1 cm Ecchymoses >1cm
1252
What is the mechanism of diabetic neuropathy?
1) Aldose reductase conversion of intracellular glucose to sorbitol --> osmotic damage to axons & schwann cells 2) Hyaline arteriolosclerosis of endoneural arterioles --> ischemic nerve damage
1253
What bugs typically cause reactive arthritis?
``` Reider's syndrome is typically caused by: Chlamydia Campylobacter Salmonella Shigella Yersinia ```
1254
What signaling molecules work through increases in cGMP?
Atrial natriuretic peptide | Nitric oxide
1255
What hormones exert their effects through an increase in cAMP?
Glucagon TSH PTH
1256
What is seen in Diffuse Esophageal Spasm?
Painful, disorganized, sustained esophageal contractions "Corkscrew esophagus" on barium swallow Intermittent dysphagia & chest pain
1257
What is used as a serum marker of anaphylaxis?
Tryptase It is released (with histamine) from mast cells & is relatively specific to them.
1258
What is the intrapleural pressure at rest?
Intrapleural pressure at FRC = -5 cm H20
1259
What does Parvovirus interact with on host cells?
P antigen on immature erythroid cells | Can also infect mature ones but has tropism for immature
1260
What is the major determinant of E. coli virulence in neonatal meningitis?
K-1 capsular antigen
1261
What receptor is responsible for nicotine addiction?
alpha-4-beta-2 nicotinic ACh receptor --> downstream dopamine release
1262
What is the mechanism of Verenicline?
It is an alpha-4-beta-2 nicotinic receptor partial agonist used to reduce nicotine cravings & attenuating the reward pathway of nicotine
1263
What medications cause fat redistribution from the extremities to the abdomen?
``` Protease inhibitors (lipodystrophy) Corticosteroids (Cushing's) ```
1264
What vessels can cause compression of CNIII?
Posterior cerebral artery Superior cerebellar artery CNIII runs between these vessels & aneurysm in either one can cause compression of it.
1265
With CNIII palsy, what type of injury causes GVE symptoms? | GSE symptoms?
Compression tends to cause GVE symptoms --> pupillary reflexes Ischemia tends to cause GSE symptoms --> motor palsy
1266
What can cause autoimmune degeneration of the cerebellum?
Paraneoplastic syndromes from: Breast/ovary (anti-Yo) Lung (Anti-P/Q; Anti-Hu)
1267
Which pneumoconiosis is associated with eggshell calcification of hilar LN's?
Silicosis
1268
What causes bronchiolitis obliterans? | What is seen?
Chronic rejection of a transplanted lung --> lymphocytic inflammation & necrosis of bronchiolar walls --> granulation tissue & exudate in the lumen of the bronchioli --> fibrous tissue obliteration of small airways
1269
What can cause clubbing of the fingers?
Lung diseases - cancer, TB, CF, bronchiectasis, pulmonary HTN, any with hypoxia. This is the most common cause. Heart diseases - cyanotic (5 T's), bacterial endocarditis Other - IBD, hyperthyroidism, malabsorption
1270
What is the germ tube test?
Inoculate Candida albicans into serum at 37 degress for 3h --> true hyphae will sprout from the yeast (often look like little J's)
1271
What is the histology of a lung hamartoma?
Hyaline cartilage Fat, smooth muscle, clefts Lined by respiratory epithelium Generally present as a peripheral "coin lesion"
1272
What is derived from the foregut?
``` Everything coming off of the celiac trunk: Esophagus Stomach Liver Gallbladder Pancreas Upper duodenum ```
1273
What is derived from the midgut?
``` Everything supplied by the SMA: Lower duodenum Small intestine Ascending colon Proximal 2/3 of the transverse colon ```
1274
What is derived from the hindgut?
Everything supplied by the IMA: Distal 1/3 of the transverse colon Descending colon Sigmoid colon
1275
What can be seen with intestinal malrotation?
Intestinal obstruction --> bilious vomiting in first week of life Cecum in RUQ, fixed by Ladd's fibrous bands Midgut volvulus --> gangrene or perforation
1276
What is the equation for attributable risk percentage?
ARP = (RR-1)/RR
1277
What is Cheyne-Stokes breathing? | What is it seen in?
Repeating episodes of breathing followed by an apneic period Seen in advanced heart disease & neurologic disease. It is caused by a delayed response to increases in PaCO2.
1278
What is seen clinically with cerebral amyloid angiopathy?
Recurrent small hemorrhagic strokes in multiple brain locations, especially outside of the basal ganglia & in the hemispheres.
1279
What is the treatment for insomnia associated with jet lag?
Melatonin
1280
What are the portacaval anastomoses?
They link the portal vein to the systemic venous circulation 1) Left gastric vein --> Esophageal vein (Esophageal varices) 2) Superior rectal vein --> Middle & inferior rectal veins (Hemorrhoids) 3) Paraumbilical veins --> Superficial & inferior epigastric veins (Caput medusae)
1281
What species cause mucormycosis?
Mucor Rhizopus Absidia
1282
What bacterium requires media with cholesterol?
Ureaplasma urealyticum requires special media rich in urea & cholesterol to grow. It can cause urethritis.
1283
What can be caused by use of appetite suppressants?
Fenfluramine Phentermine (Fen-Phen) If ingested for >3 months, they can cause pulmonary hypertension. Classic presentation is dyspnea on exertion --> cor pulmonale (RVH) --> sudden cardiac death.
1284
When are MAOI's desirable?
Atypical depression
1285
What are the fever cycle lengths of the various species of Plasmodium?
vivax/ovale - 48h malariae - 72h falciparum - severe & disorganized
1286
What is the PMN count in a gouty joint?
Normally < 20,000 If the PMN count is much greater than this, indicates a septic arthritis.
1287
``` What molecule (contained in food) should be avoided in patients taking MAOI's? What is seen if it is ingested? ```
Tyramine should be avoided. It is a sympathomimetic usually degraded in the GI tract. If ingested, it can cause hypertensive crisis. This is the most commonly tested side effect on boards.
1288
What are the long acting benzodiazepines?
Diazepam Flurazepam Chlordiazepoxide Clorazepate
1289
What are the short-acting benzodiazepines?
Alprazolam Triazolam Oxazepam
1290
What should be considered if jejunal ulcers are present?
Gastrinoma Jejunal ulcers are almost never caused by your everyday PUD. Also consider if the gastrinoma is part of MEN1
1291
What are the possible sequelae of GAS impetigo? | Strep throat?
Impetigo --> only Acute post-strep GN only Strep throat --> Acute post strep GN or Rhematic fever Rheumatic fever can only follow strep pharyngitis.
1292
What breathing rates are adopted by patients with COPD?
Restrictive --> higher breathing rates of low tidal volume Obstructive --> lower breathing rates of high tidal volume This minimizes the work of breathing for them.
1293
Why are gallstones formed during pregnancy?
Estrogen --> ^HMG-CoA reductase activity --> ^Cholesterol Progesterone --> Gallbladder hypomotility & reduced bile acids Also seen with OCP use.
1294
What antiarrhythmic should be used in post-MI arrhythmia?
Lidocaine Mexiletine Tocainide They are selective for depolarized cells (ischemic myocardium). Amiodarone is the DOC for V-tach.
1295
What drug is the best treatment for AChE inhibitors?
Pralidoxime It can only be used early on. Atropine is an alternative, though it will only combat the muscarinic effects. The patient will still be at risk for muscle paralysis.
1296
What causes a Zenker's diverticulum?
Cricopharyngeal muscle dysfunction --> increased oropharyngeal pressure during swallowing --> herniation through the muscular wall
1297
What does a relative afferent pupillary defect indicate?
A positive swinging flashlight test indicates pathology involving the retina, optic nerve, optic chiasm, or optic tract. If the pathology was after this, the pupillary light reflex would be intact.
1298
What side effects are seen with Niacin?
``` High dose niacin (to treat dyslipidemia) can cause: Flushing Gout Hyperglycemia Hepatitis ```
1299
What step in the TCA cycle produces GTP?
Succinate thiokinase | Succinyl-CoA Synthetase
1300
Where is cryptococcus found in the environment?
Soil & bird (pigeon) droppings
1301
Who is at risk for being lactose intolerant?
Asian & African descent | Can also be secondary to viral gastroenteritis or any disease that damages the intestinal epithelium
1302
What is the mechanism of Niacin's effects on dyslipidemia?
Decreases the synthesis of hepatic TG's & VLDL. | Also increases HDL (drug of choice)
1303
How are patients screened if fat malabsorption is suspected?
Sudan III stain on a stool sample reveals presence of fat
1304
What coagulopathy is seen with uremic patients?
^bleeding time, normal PLT count, PT & aPTT It is a qualitative platelet defect due to under-excretion of platelet inhibitory factors. Resolves with dialysis.
1305
Where is a hydrocele located?
Within the tunica vaginalis of the testicle It is derived from the peritoneum and can remain in communication to the peritoneal cavity, leading to hydrocele.
1306
What is the basis for chloride shift seen in RBC's?
CO2 (tissues) --> H+ & HCO3- --> HCO3- diffuses out --> Cl- diffuses in to maintain charge The reverse happens in the lungs. Thus, RBC [Cl-] is high in venous blood and low in arterial blood.
1307
What drug is used in acute closed-angle glaucoma? | What drug is contraindicated?
Use Pilocarpine (cholinomimetic --> opens meshwork) Do NOT use epinephrine (effective at vasoconstricting to reduce production of aqueous humor but causes mydriasis --> blocks off canal of Schlemm)
1308
What is the mechanism of opioids?
Agonists at mu opioid receptors --> opens K+ channels & closes Ca2+ channels --> inhibition of synaptic transmission Inhibits release of ACh, NE, 5-HT, glutamate, substance P
1309
What toxicities are seen with opioids?
``` Addiction Respiratory depression Constipation Miosis Additive CNS depression with other drugs ``` OD treated with naloxone
1310
What is Butorphanol? | What is it used for?
It is a mu opioid receptor partial agonist used for severe pain. It has less respiratory depression than other opioids. If given to a pt who is dependent on a normal opioid, can cause withdrawal.
1311
What is the mechanism of Tramadol? | What toxicities are seen?
It is a weak opioid agonist that also inhibits NE & 5-HT reuptake. It is used for chronic pain. It has toxicities similar to opioids & can lower the seizure threshold.
1312
What is the drug of choice for partial seizures?
Carbamazepine Both simple & complex partial seizures.
1313
What is used to prevent status epilepticus?
Phenytoin
1314
What toxicities are seen with Carbemazepine?
``` Agranulocytosis Aplastic anemia SIADH Ataxia CYP450 induction Teratogenesis ```
1315
What toxicities are seen with Phenytoin?
``` Nystagmus Drug-induced Lupus Gingival hyperplasia Hirsutism Megaloblastic anemia LAD Ataxia CYP450 induction Teratogenic (fetal hydantoin syndrome) ```
1316
What toxicities are seen with valproic acid?
Tremor Weight gain Fatal hepatotoxicity (measure LFT's) Neural tube defects (don't give in pregnancy)
1317
What is the mechanism of barbiturates?
^DURATION of Cl- channel (GABAa) opening --> hyperpolarizes membrane --> decreases neuronal firing
1318
What is the mechanism of benzodiazepines?
Increase FREQENCY of GABAa channel opening --> influx of Cl- --> hyperpolarization of neurons
1319
What toxicities are seen with bezodiazepines? | What is the treatment for overdose?
``` CNS depression (seen in elderly; additive) Dependence (Triazolam, Oxazepam, Midazolam) ``` OD Tx: Flumazenil (antagonist)
1320
What are the common CNS depressants?
``` EtOH Benzodiazepines Barbiturates 1st generation antihistamines Neuroleptics ```
1321
What is the mechanism of Zolpidem?
Zolpidem, Zaleplon, & Eszopiclone They are selective GABA agonists at the BZ1 receptor subtype. Used for insomnia.
1322
What determines potency of an inhaled anesthetic?
^lipid solubulity Potency = 1/MAC
1323
What factors influence MAC?
Minimum alveolar concentration to anesthetize 50% of population Affected ONLY by: Age Body temperature
1324
What toxicity is seen with Halothane?
Hepatotoxicity: A few days after exposure: Eosinophilia, ^ALT, ^PT
1325
What toxicity is common to all inhaled anesthetics?
Malignant hyperthermia | not seen in N2O actually
1326
What inhaled anesthetic is pro-convulsant?
Enflurane
1327
What are the IV anesthetics?
``` Barbiturates Benzos Arylcyclohexamines (Ketamine) Opioids Propofol ```
1328
What barbiturate is used for anesthetic induction?
Thiopental It is highly lipid soluble (rapid onset) & redistributes rapidly (effects are short-lived)
1329
What is propofol used for? | What is its mechanism?
Sedation for mechanically ventilated patients Anesthesia induction Short procedures Potentiates GABAa
1330
What are the ester local anesthetics? | The amides?
Ester = Procaine, Cocaine, Tetracaine | Amide = Lidocaine, Mepivacaine, Bupivacaine Amide's have 2 I's in the name
1331
What can be coadministered to increase effectiveness of local anesthetics?
Epinephrine can be given to vasoconstrict --> ^local action & decrease systemic concentration
1332
What fibers are affected more by local anesthetics? | What sensations are lost first due to this?
Small myelinated > Small unmyelinated > Large myelinated > Large unmyelinated Loss of sensation: 1) Pain 2) Temperature 3) Touch 4) Pressure
1333
What is the mechanism of Dantrolene? | What is its use?
Blocks release of Ca2+ from the SR of skeletal muscle Used to treat: Malignant hyperthermia (inhaled anesthetics except N2O) Neuroleptic malignant syndrome
1334
What drugs are used to treat Parkinson's disease?
``` BALSA: Bromocriptine Amantadine Levodopa Selegiline (and COMT inhibitors) Antimuscarinics ```
1335
What is the mechanism of Bromocriptine? | What is it used for?
It is a D2 agonist used for Parkinson's disease & Prolactinomas
1336
What toxicities are seen with Levodopa/Carbidopa?
Anxiety, agitation, insomnia, confusion, hallucinations Arrhythmias Dyskinesia
1337
What is the mechanism of Selegiline?
Selective MAO-B inhibitor --> inhibits metabolism of Dopamine
1338
What drugs are used to treat Alzheimer's?
Memantine (NMDA antagonist) Donepezil, Ganatamine, Rivastigmine (AChE inhibitors) alpha-Tocopherol (Vit. E; Antioxidant)
1339
What drugs are used to treat Huntington's disease?
Tetrabenazine, Reserpine (inhibit VMAT -> dop not packaged) Haloperidol (Dopamine antagonist)
1340
What toxicities are seen with Sumatriptan?
Coronary vasospasm (contraindicated in Prinzmetal's angina)
1341
What is significant about Selective IgA Deficiency?
1) They have anaphylactic reactions to IgA-containing blood products 2) Can have false-positive hCG testing (heterophile Ab)
1342
Mutation in X-linked agammaglobulinemia
BTK (Bruton's Tyrosine Kinase) No B cell maturation seen --> less B cells & way less Ig's of all classes
1343
What is seen histologically with Dubin-Johnson syndrome?
Epinephrine metabolites are seen within the lysosomes Grossly, liver is black
1344
What immune cell predominance is seen in bronchoalveolar lavage of a sarcodosis patient?
Very high CD4:CD8 ratio
1345
What prokaryotic rRNA binds the Shine-Dalgarno sequence?
16S rRNA is complementary to the Shine-Dalgarno sequence (10 bp upstream from AUG)
1346
What is the mechanism of Aminoglycosides?
Bind 30S and inhibit formation of initiation complex
1347
What is the mechanism of Tetracyclines?
Bind 30S and prevent tRNA from entering acceptor site
1348
What is the mechanism of Chloramphenicol?
Binds 50S and inhibits peptidyl transferase
1349
What is the mechanism of Macrolides?
Bind 50S and prevent release of tRNA from the E site
1350
What illness is caused by B. pertussis?
Whooping cough 3 stages: 1) Catarrhal stage - flu-like; highly contagious 2) Paroxysmal stage - many coughs then large whooping inspiration 3) Recovery stage - coughing slowly subsides Whole process lasts ~3 months
1351
What is the mechanism of diphtheria toxin?
AB exotoxin ribosylates EF-2 --> inhibits protein synthesis leading to cell death
1352
What step is NAD+ needed for in glycolysis that is the basis for why it is regenerated in anaerobic glycolysis?
Glyceraldehyde-3-P --> 1-3-BPG
1353
What organism causes tinea versicolor?
aka pityriasis versicolor Caused by Malassezia furfur Symptoms: Light patches on skin Otherwise asymptomatic
1354
What is seen on KOH prep of Malassezia furfur?
Spaghetti & meatballs appearance Causes tinea versicolor
1355
What nutrients are not present in breast milk?
Vitamins D & K D levels will be adequate if the infant gets enough sunlight. Dark skinned children are at higher risk. K is administered at birth
1356
What are the possible complications of Diphtheria?
Suffocation due to pseudomembranes Heart pathology - myocarditis, arrhythmia, CHF CNS pathology - neuropathy, paralysis, coma
1357
What is the Diphtheria vaccine against?
The B portion of the AB exotoxin ``` A = active (ribosylates EF-2) B = binding ```
1358
What resting membrane potential do nerves typically sit at?
-70 mV
1359
What organisms cause nongonococcal urethritis?
Chlamydia trachomatis > Ureaplasma urealyticum >> Mycoplasma & Trichomonas Azithromycin is the treatment
1360
What is the drug of choice for the management of hypertensive pts who also have bradycardia?
Nifedipine It is a vascular-selective CCB that causes vasodilation and can cause compensatory tachycardia.
1361
What is the cause of hereditary pancreatitis?
Abnormal trypsin cannot be cleaved and thus inactivated by other trypsin. Patients suffer recurrent attacks of acute pancreatitis.
1362
What electrolyte disturbances can be caused by loop diuretics?
Hypokalemia Hypomagnesemia Hypocalcemia
1363
What is seen with fibromyalgia?
Diffuse musculoskeletal pain Insomnia Emotional disturbances Often seen in women 20-50
1364
What are the 3 dopaminergic pathways within the brain?
Mesolimbic-Mesocortical --> regulates behavior Nigrostriatal --> coordination of movement Tuberoinfundibular --> controls prolactin secretion
1365
What can persistent lymphedema lead to?
Lymphangiosarcoma Often seen after radical mastectomy
1366
What are the effects of AV shunts on the pressure-volume curve?
Increased preload and decreased afterload
1367
What effects do loop diuretics have on the renal arterioles?
In addition to their effects at the thick ascending limb, loop diuretics stimulate prostaglandin release, causing vasodilation of the afferent arteriole. This can be blocked by concurrent use of NSAIDs
1368
What antiarrhythmics demonstrate use-dependence?
Use-dependence = prolong the QRS more at high HR's ``` Class Ic (Na+ channel blockers) --> use-dependence Class III (K+ channel blockers --> reverse use-dependence ```
1369
What are the opioid partial agonists?
Pentazocine & Butorphanol They can cause withdrawal if full agonist opioids have been used.
1370
What beta blockers are safer to use in diabetics?
B1 selective The nonselective ones blunt the adrenergic symptoms normally seen with hypoglycemia. They also inhibit hepatic gluconeogenesis and peripheral glycogenolysis & lipolysis.
1371
What molecule are bisphosphonates analogs of?
Pyrophosphate (a component of hydroxyapatite)
1372
What is the cause of supine hypotension syndrome?
aka aortocaval compression syndrome Caused by the gravid (>20 weeks) uterus compressing the IVC upon lying down. This reduces venous return, which lowers CO --> hypotension & possible syncope
1373
How can the cause of a metabolic alkalosis be determined? | What are the causes?
Measurement of urinary [Cl-] & volume status Vomiting --> low urinary Cl- (saline responsive) Loop diuretics --> high urinary Cl- (saline responsive) Conn syndrome --> high urinary Cl- (saline unresponsive)
1374
What is defective in Hepatitis D virus?
It must be coated by the external coat antigen of HBV in order to survive & infect.
1375
What drugs are typically coadministered with direct arterial vasodilators?
Sympatholytics (to counteract compensatory tachycardia) Diuretics (to counteract sympathetic renin release --> edema)
1376
What antiarrhythmics cause prolongation of the QT interval?
Class Ia & Class III Amiodarone is the only drug of these classes that does not predispose to torsades.
1377
What nerve is at risk when food is caught in the piriform recess?
Internal branch of the superior laryngeal nerve Damage to it leave the patient with no sensation to the pharynx above the vocal cords --> no cough reflex
1378
What is Terbinafine used for? What is its mechanism? What toxicities are seen?
Terbinafine is used for dermatophyte infections. It inhibits fungal squalene epoxidase --> impaired synthesis of ergosterol. Toxicities: Abnormal LFT's Visual disturbances Not seen if used topically
1379
What is the drug of choice in treating hairy cell leukemia?
Cladribine It is an adenosine analog that is resistant to Adenosine Deaminase (usually a problem with treating HCL).
1380
What toxicities are seen with carbamazepine?
Bone marrow suppression Hepatotoxicity SIADH
1381
What drug is used to treat carcinoid syndrome?
Octreotide It is a synthetic somatostatin analog
1382
What is the treatment for cryptococcal meningitis?
Amphotericin B & flucytosine
1383
What is the main toxicity of antithyroid drugs?
PTU & Methimazole both cause agranulocytosis | Typically presents with fever & a sore throat
1384
Where are the leads placed for biventricular pacemakers?
Right atrium Right ventricle Left AV groove, within a vein of the coronary sinus
1385
When do the neural pores close?
Anterior - 25th day Posterior - 27th day Failure in either of these leads to neural tube defects.
1386
What is epidermal spongiosis?
Accumulation of edema in the epidermis --> intercellular bridges are stretched. If the bridges tear --> vesicle
1387
What can cause communicating hydrocephalus?
Meningitis Subarachnoid hemorrhage Intraventricular hemorrhage
1388
What PO2 corresponds to 50% saturation of Hb? | Myoglobin?
Hemoglobin - 26 mmHg Myoglobin - 1 mmHg Therefore myoglobin has much higher affinity for O2 than does hemoglobin. It also has no cooperativity, leading to a steep, non-sigmoidal dissociation curve. Monomeric Hb acts very similarly to Mb.
1389
What are neurofibromas composed of?
Schwann cell tumors They are rubbery and exhibit "buttonholing"
1390
What is released from the ventricles in response to volume overload?
BNP and even ANP can be released from the ventricles in severe volume overload/hypertrophy.
1391
What changes occur in the skin in response to chronic topical corticosteroid application?
Dermal atrophy Can present as loss of collagen, drying, cracking, tightening of the skin, telangiectasias & ecchymoses.
1392
What is seen on water deprivation testing in primary polydipsia?
Primary (psychogenic) polydipsia Strong increase in urine osmolarity due to water deprivation, then a weak increase following vasopressin injection.
1393
What are penicillins structural analogs of?
D-Ala-D-Ala
1394
What drugs can accumulate in slow-acetylators?
INH Dapsone Hydralazine Procainamide
1395
What is the mechanism of abciximab? | What is its use?
Anti-GPIIb/IIIa --> blocks platelet aggregation Used during angioplasty
1396
What are the effects of ANP?
1) Dilates afferent & constricts renal arterioles. Inhibits sodium reabsorption (PCT & CCD). Inhibits renin release. 2) Inhibits aldosterone secretion. 3) Peripheral vasodilation
1397
What is the mechanism of Ethambutol?
Inhibits arabinosyltransferase --> impaired carbohydrate polymerization --> impaired cell wall synthesis
1398
What can be coadministered to decrease the flushing seen with Niacin?
Aspirin This is because the side effect is mediated by prostaglandins.
1399
What is damaged in central diabetes insipidus?
Pituitary damage --> transient DI Hypothalamus damage --> permanent DI
1400
What is seen on liver biopsy of a patient with chronic HBV?
Ballooning degeneration Hepatocyte necrosis Portal inflammation "Ground glass" appearance (intracellular HBsAg proteins)
1401
What is seen on liver biopsy of a patient with chronic HCV?
Lymphoid aggregates in the portal tracts | Focal macrovesicular steatosis
1402
What is the mechanism of ammonia intoxication in the brain?
Astrocytes take up NH4+ and combine it with glutamate to make glutamine --> accumulation & astrocyte damage. Also the glutamate content in the brain is then decreased. Additionally, neurons can detoxify ammonia by combining it with alpha-Ketoglutarate --> glutamate. This depletes stores of alpha-Ketoglutarate --> impaired Krebs cycle within neurons.
1403
What is the glutamate-glutamine cycle?
Neurons convert glutamine --> glutamate (Glutaminase) --> release it into synaptic cleft as a NT Astrocytes take up glutamate and --> glutamine (Glutamine synthase) and release it for use by neurons.
1404
How long is it before an infarcted myocyte ceases contracting? How long until injury is irreversible?
Affected myocytes cease contracting within ~1 minute. Irreversible cell injury occurs at ~30 minutes. As ATP is depleted, it is degraded to ADP --> AMP --> adenosine. Adenosine can diffuse out of the cell and cause vasodilation. Following 30 minutes of ischemia, half of cellular adenine stores are gone and thus the cell cannot recover.
1405
What are the inhibitors of dihydrofolate reductase?
Methotrexate - cancer Trimethoprim - bacteria Pyrimethamine - malaria & toxoplasmosis
1406
What are the toxicities seen with statins?
Hepatotoxicity (check LFT's before prescribing) | Rhabdomyolysis
1407
What are the serum/urinary markers of osteoblastic activity? | Osteoclastic?
Osteoblasts - Alkaline phosphatase Osteoclasts - TRAP, Urinary hydroxyproline, urinary deoxypyridinoline
1408
What organisms show a positive Tzank smear?
``` Tzanck test = scrapinf of an ulcer base to look for Tzanck cells (multinucleate giant cells) seen in: HSV VZV CMV Pemphigus vulgaris ```
1409
What are the mechanisms by which combination OCP's function?
1) Feedback inhibition on the pituitary --> no LH spike 2) Thickened cervical mucus 3) No growth of endometrium --> not suitable for implantation
1410
What is the drug of choice for hypertensive crisis?
Nitroprusside
1411
What is the antidote for nitroprusside overdose?
Sodium thiosulfate It acts as a sulfur donor to enhance conversion of cyanide --> thiocyanate
1412
How do infantile & adult botulism differ?
Adults ingest preformed toxin, whereas infants ingest spores (honey) that germinate in the gut. Also, infantile is usually less severe.
1413
What is the psoas sign? | What is it indicative of?
Lying on their side, passive hyperextension of the hip causes pain. A positive psoas sign can be indicative of a psoas abscess or appendicitis (if on the right).
1414
What is the mechanism of INH?
It is a pyridoxine analog that inhibits mycolic acid synthesis in MTB.
1415
What occurs in tissues when a patient has CREST or systemic scleroderma?
Monoclonal T cell proliferation --> cytokine release (especially TGF-beta) --> excessive deposition of collagen by fibroblasts
1416
What vitamin can be used to treat measles?
Vitamin A They don't know why, the measles virus may induce a deficiency.
1417
What is the full name of PCP? | What is seen in pts who are on it?
Phencyclidine Vertical or horizontal nystagmus Violent behavior
1418
What is the mechanism of Phencyclidine?
PCP is an NMDA antagonist --> ^excitatory NT's --> hallucinations
1419
How does a glucagonoma present?
Migratory skin necrolysis Erythematous groin rash Diabetes Anemia
1420
What changes are seen in the adrenals in a pt with Cushing disease?
Cushing disease - ACTH must be coming from pituitary adenoma HyperPLASIA of both the zona fasciculata & reticularis
1421
What is seen with thalamic syndrome?
Thalamic stroke in the VPL/VPM area --> pure sensory stroke causing total sensory loss of the contralateral side of the body. No motor deficits but impaired proprioception can cause falls.
1422
What is used to create cystathionine? | What cofactor is needed?
Homocysteine + Serine --> Cystathionine --> Cysteine Both of these steps require Vit. B6
1423
What is the cycle for how THF is regenerated from 5-methyl-THF?
1) Homocysteine + 5-M-THF --> Methionine + THF (Methionine synthase/Homocysteine methyltransferase) (Requires Methylcobalamin) 2) Methionine + ATP --> S-adenosyl methionine (SAM) 3) SAM --> S-adenosyl homocysteine (Methyltransferase) 4) S-adenosyl homocysteine --> Homocysteine
1424
What is the mechanism of echinocandins?
Caspofungin & Micafungin (echinocandins) inhibit polysaccharide (glucan) synthesis --> inhibit fungal cell wall synthesis
1425
What is the mechanism of Griseofulvin?
It enters fungal cells, binds MT's & inhibits mitosis.
1426
What is seen on liver biopsy in halothane-associated hepatitis?
Massive centrilobular hepatic necrosis Mortality ~80%
1427
How does Crohn's disease lead to kidney stones?
Terminal ileum damage --> Bile acids not reabsorbed --> Steatorrhea --> loss of Ca2+ as soaps in stool --> Ca2+ not available to bind up oxalate in the gut --> ^Oxalate absorption --> kidney stones
1428
What is seen on liver biopsy of a patient with Reye's syndrome?
Microvesicular steatosis | No necrosis or inflammation
1429
What are the effects of Sotalol?
It has properties of a beta blocker & a Class III antiarrhythmic (K+ channel blocker) --> ^PR & ^QT
1430
What groups are at risk for Pellegra?
``` Populations that subsist on corn Alcoholics Carcinoid syndrome INH Hartnup disease ```
1431
What is B3 synthesized from endogenously?
Niacin is synthesized from Tryptophan
1432
What causes the outflow obstruction seen in HOCM?
Hypertrophied septum is too close to anterior mitral valve leaflet --> (paradoxical) systolic anterior motion of the anterior mitral valve leaflet --> leaflet moves closer to septum --> outflow obstruction
1433
What drugs display tachyphylaxis?
``` Topical antihistamines (endogenous NE is downregulated) Nitroglycerine (less NO release) ``` Both of these drugs require time off in order to maintain their efficacy.
1434
What is the reaction catalyzed by G6PD?
G6P + NADP --> 6-Phosphogluconate + NADPH
1435
What is used to treat TCA overdose?
Sodium bicarbonate
1436
What is the most common toxicity of aspirin?
GI bleeding This is seen even with low dose aspirin, but especially with high dose.
1437
What is the use of Fomepizole?
Inhibits alcohol dehydrogenase. It is used for ingestion of methanol, ethylene glycol, propylene glycol.
1438
What is seen on esophageal manometry in CREST syndrome?
Decreased peristalsis | Decreased LES tone
1439
What is the mechanism of macrolides? | Tetracyclines?
Macroslides --> elongation | Tetris --> blocks tRNA binding
1440
What causes a Bartholin cyst?
Obstruction of the Bartholin gland with subsequent infection. Seen in women of reproductive age. Unilateral painful cystic lesion just below the & adjacent to the vaginal vestibule.
1441
What causes condylomas?
HPV 6 & 11 infection
1442
What is seen with Lichen sclerosis? | What is the complication seen with it?
Leukoplakia with parchment-like vulvar skin in postmenopausal women. Slightly increased risk for SCC.
1443
What is seen with lichen simplex chronicus?
Chronic irritation & scratching --> leukoplakia w/ thick leathery skin No increased risk of SCC (only lichen sclerosis has ^risk)
1444
What are the causes of vulvar leukoplakia?
Lichen sclerosis Lichen simplex chronicus Squamous cell carcinoma
1445
What causes SCC of the vulva?
Two possible etiologies: 1) VIN due to HPV infection 2) Longstanding lichen sclerosis
1446
What causes hemolytic disease of the newborn?
Rh+ infant born to a (sensitized) Rh- mother ABO type is mild Rh type is severe but preventable
1447
What is used to prevent hemolytic disease of the newborn? | How does it work?
RhoGAM They are IgG against the Rh antigen that bind up & eliminate any fetal RBC's that are passed to mom. This prevents mom from mounting an immune response against the Rh antigen (fetal RBC's)
1448
What does the posterior descending coronary artery supply?
The posterior walls of the ventricles & posterior 1/3 of septum
1449
What is seen with congenital neutropenia?
Low neutrophil counts at birth or shortly afterward. Severe recurrent infections seen later w/ no pus.
1450
What ventricle is anterior & which is posterior?
The RV is anterior & right border | The LV is posterior & left lateral
1451
What gives cephalosporins their expanded spectrum?
They are less susceptible to beta-lactamases
1452
What are irradiated blood products used for?
To prevent Transfusion-Associated Graft vs. Host Disease (TA-GVHD) Blood that is given to immunodeficient patients should be irradiated prior to transfusion.
1453
What cranial nerve innervates the pharyngeal constrictor muscles?
CNX
1454
How does beta thalassemia minor present?
Usually asymptomatic Diagnosis is incidental by microcytic anemia on CBC Confirmation is via >3.5% of HbA2 on electrophoresis
1455
How is acetylcholinesterase poisoning treated?
Atropine - reverses central respiratory depression & other symptoms Pralidoxime - Reverses AChE poisoning Give both at once
1456
What is the equation for specificity?
Sp = TN /(TN + FP) Sp = 1 - false positive
1457
What is the equation for sensitivity?
Sn = TP/ (TP + FN) Sn = 1 - FN
1458
What is a wound contracture?
Excessive activity of metalloproteinases & myofibroblasts can cause a wound contracture, which can lead to deformity or limit the mobility of a joint.
1459
How are cirrhotic patients screened for HCC?
Serum AFP levels are monitored regularly
1460
What causes neonatal tetanus?
Colonization of the umbilical stump A common occurrence in developing countries.
1461
How does Parvovirus infection manifest in adults?
Arthritis in adults Erythema infectiosum in children
1462
Describe the mTOR pathway following growth factor binding.
Binding of growth factor --> Autophosphorylation of tyrosine kinase --> Activation of phosphoinositide-3 kinase --> Phosphorylation of PIP2 --> Activation of Protein kinase B --> Acivation of mTOR --> Translocation to nucleus to activity genes This pathway is active in many cancers. Sirolimus is an mTOR inhibitor.
1463
What causes cardiovascular dysphagia?
Enlargement of the left atrium --> compression of the esophagus
1464
What is the progression of CD4/8 expression of T cells in the thymus?
Double negative --> Double positive --> single positive
1465
How is resistance to INH developed?
Either decreased expression of catalase-peroxidase (required for INH activation) or modification of the mycolic acid synthesis enzyme.
1466
Why is the infectious dose of vibrio so high?
They prefer alkaline environments & are killed easily by acid.
1467
What is the best prognostic factor in meningococcemia?
Blood levels of lipooligosaccharide (LOS)
1468
What is the mechanism of Nystatin?
Binds ergosterol in the cell membrane and creates pores in the membrane --> leakage of ions from the cells --> cell lysis Same as Amphotericin B
1469
How are enterococci resistant to aminoglycosides?
They transfer chemical groups onto the drug (acetyl, adenyl, phosphate) that impair its binding to ribosomes.
1470
What is Trousseau's syndrome? | What causes it?
Migratory superficial thrombophlebitis seen with pancreatic, colon, or lung adenocarcinoma. These tumors can produce a thromboplastin-like (Tissue factor-like) substance.
1471
How is hereditary spherocytosis inherited?
Autosomal dominant
1472
What vitamin deficiency is commonly seen in patients with hemolytic anemia?
Folic acid deficiency This is because they have increased turnover of cells
1473
Why do Crohn's disease patients get gallstones?
Bile acids not absorbed in terminal ileum --> Decreased concentration of them in the bile --> Gallstones
1474
What is ANOVA used for?
It is like a T-test for more than 2 populations. It compares the numerical means of multiple populations.
1475
What is the effect of Tetrodotoxin?
Binds to Na+ channels on neurons & prevents them from opening. It is found in puffer fish.
1476
What causes Lymphogranuloma venereum? | What is seen?
Chlamydia trachomatis (L1-L3 serotypes) 1) Initial painless ulcer on genitals 2) Swollen painful inguinal nodes --> rupture (buboes) Treat with doxycycline
1477
What are the macrolides?
ACE Azithromycin Clarithromycin Erythromycin
1478
What injury is seen following humerus dislocation?
(Anterior) humerus dislocation --> axillary nerve injury It is the most commonly dislocated joint in the body.
1479
What is the mechanism of Enfuvertide?
Binds gp41 on HIV cell membrane --> prevents gp41 from undergoing conformational change & causing membrane fusion
1480
What are the effects of PPAR-gamma?
``` Increased synthesis of: Adiponectin Fatty acid transport protein Insulin receptor substrate GLUT4 ```
1481
Where are thyroid hormone receptors located?
In the nucleus. The other steroid hormone receptors are found in the cytosol & translocate to the nucleus upon binding.
1482
What stains can be used to detect cryptococcus neoformans?
Mucicarmine --> red stained polysaccharide capsule India ink stain --> Clearing around the cell (polysaccharide capsule)
1483
What would the approximate Vd be for these drugs? Large/Plasma protein bound/Highly charged Small & hydrophilic Small & hydrophobic
Large/Plasma protein bound/Highly charged --> ~4L (plasma) Small & hydrophilic --> ~15L (extracellular fluid & plasma) Small & hydrophobic --> ~41L (everywhere)
1484
What skin findings can be seen with Pseudomonas?
Ecthyma gangrenosum Cutaneous necrotic lesions caused by hematologic dissemination & subsequent local release of exotoxins.
1485
What does myelination do to the time constant & space constant?
Space (length) constant is increased (further transmission) | Time constant is decreased (faster transmission)
1486
What is the DOC for combined absence & tonic-clonic seizures?
Valproic acid
1487
What can cause lithium toxicity?
Drugs that affect Na+ handling in the kidney (thiazides) Drugs that affect GFR (ACEi, NSAIDs) Volume contraction (CHF, GI bleed, Cirrhosis) Li+ is handled just like Na+ is handled in the kidney. Thus, anything that could cause Na+ retention can cause Lithium toxicity. Treatment is cessation & hemodialysis.
1488
What embryologic layer do the branchial arches arise from?
Neural crest ectoderm
1489
What are the macrolides?
ACE Azithromycin Clarithromycin Erythromycin
1490
What injury is seen following humerus dislocation?
(Anterior) humerus dislocation --> axillary nerve injury It is the most commonly dislocated joint in the body.
1491
What is the mechanism of Enfuvertide?
Binds gp41 on HIV cell membrane --> prevents gp41 from undergoing conformational change & causing membrane fusion
1492
What are the effects of PPAR-gamma?
``` Increased synthesis of: Adiponectin Fatty acid transport protein Insulin receptor substrate GLUT4 ```
1493
What embryologic layer do the branchial arches arise from?
Neural crest ectoderm
1494
What can cause lithium toxicity?
Drugs that affect Na+ handling in the kidney (thiazides) Drugs that affect GFR (ACEi, NSAIDs) Volume contraction (CHF, GI bleed, Cirrhosis) Li+ is handled just like Na+ is handled in the kidney. Thus, anything that could cause Na+ retention can cause Lithium toxicity. Treatment is cessation & hemodialysis.
1495
What is the DOC for combined absence & tonic-clonic seizures?
Valproic acid
1496
What does myelination do to the time constant & space constant?
Space (length) constant is increased (further transmission) | Time constant is decreased (faster transmission)
1497
What skin findings can be seen with Pseudomonas?
Ecthyma gangrenosum Cutaneous necrotic lesions caused by hematologic dissemination & subsequent local release of exotoxins.
1498
What would the approximate Vd be for these drugs? Large/Plasma protein bound/Highly charged Small & hydrophilic Small & hydrophobic
Large/Plasma protein bound/Highly charged --> ~4L (plasma) Small & hydrophilic --> ~15L (extracellular fluid & plasma) Small & hydrophobic --> ~41L (everywhere)
1499
What stains can be used to detect cryptococcus neoformans?
Mucicarmine --> red stained polysaccharide capsule India ink stain --> Clearing around the cell (polysaccharide capsule)
1500
Where are thyroid hormone receptors located?
In the nucleus. The other steroid hormone receptors are found in the cytosol & translocate to the nucleus upon binding.
1501
What is torticollis? | What causes it?
It is congenital fibrosis of the SCM that causes tilting of the head & contralateral head turn, presenting at 2-4 weeks of age. It is caused by birth trauma or malposition of the head in utero. It can be treated with conservative therapy & stretching exercises.
1502
What is characteristic of CSF proteins in SSPE?
Oligoclonal bands of Ab's are found | None are against the M component of measles virus
1503
If patients are loss to follow up, what kind of bias is this?
Attrition bias (a type of selection bias)
1504
What portion of the aorta is most vulnerable to traumatic rupture (i.e. a car accident)?
The aortic isthmus Just distal to the branch point of the left subclavian, where the ascending & descending aorta meet.
1505
What causes Ménière's disease? | What is seen?
An increased volume of endolymph --> damage to vestibular & cochlear organs Triad of: Tinnitus Vertigo Hearing loss (sensorineural)
1506
What is seen on ECG in Wolff-Parkinson-White syndrome?
Recurrent paroxysmal SVT Baseline ECG: Shortened PR Delta wave before QRS Widened QRS
1507
What type of glands are found in the dermis of skin?
Eccrine (merocrine) - sweat glands Apocrine - genitals, axilla, areola; secrete onto hair follicles Sebaceous glands - holocrine secretion of sebum onto hair follicles
1508
What organism causes cat scratch disease? | What is seen?
Bartonella henselae Self-limited low fever, LAD Can cause bacillary angiomatosis in immunocompromised patients
1509
What is a test's reliability?
The same thing as precision. The ability to produce consistent results (whether or not they are accurate)
1510
What are P bodies?
They lie in the cytoplasm & play a role in mRNA translation regulation & degradation.
1511
What are the MAOi's?
Tranylcypromine Phenelzine Selegiline
1512
What type of renal damage is seen with prolonged NSAID use?
Papillary necrosis | Chronic interstitial nephritis
1513
What mutations cause HOCM? | What mutations cause hereditary dilated cardiomyopathy?
HOCM - Autosomal dominant mutations in myosin heavy chain Dilated CM - Autosomal dominant mutations in cytoskeleton (dystrophin) or mitochondrial enzymes
1514
What is the treatment of diphtheria?
1) Diphtheria antitoxin (passive immunity) 2) Penicillin or erythromycin 3) DPT vaccine Important to give antitoxin early since it cannot affect toxin that has reached the CNS or heart. Most common cause of death is cardiomyopathy (5-10% mortality).
1515
How can complete vs partial central diabetes insipidus be differentiated?
Response to vasopressin administration: >50% increase in urine osmolarity --> complete central DI partial central DI Partial central DI = some ADH is produced but not enough for normal kidney function.
1516
What antiarrhythmic classes affect PR & what affect QT?
1 and 3 --> long QT | 2 and 4 --> long PR
1517
What are the Class I antiarrhythmics?
"Double Quarter Pounder w/ Mayo Lettuce Tomato, and Fries Please" Ia: Disopyramide, Quinidine, Procainamide Ib: Mexiletine, Lidocaine, Tocainide Ic: Flecainide, Propafenone
1518
What causes the chronic myeloproliferative disorders?
CML = t(9;22) --> BCR-ABL fusion protein Essential thrombocytosis, Polycythemia vera, Primary myelofibrosis = JAK2 mutation --> constitutive tyrosine kinase activity
1519
What anticonvulsant is metabolized to other active anticonvulsants?
Primidone --> Phenobarbital & Phenylethylmalonamide Primidone can cause lethargy
1520
Anti-histone Ab's
Drug-induced lupus
1521
What is the most common cause of HCC?
Hepatitis B Vaccination is important
1522
What do Auer rods stain positive for?
Myeloperoxidase Seen in myelogenous leukemias
1523
What complications of Herpes Zoster are possible?
Herpes zoster ophthalmicus (if in V1 ganglion) --> visual probs Post-herpetic neuralgia: Persistent pain for months after resolution Increases with ^age
1524
What is the most common cause of death in diabetes?
Coronary heart disease
1525
What is the treatment for malignant hyperthermia? | What is its mechanism?
Malignant hyperthermia is caused by inhaled anesthetics & succinylcholine. Ryanodine receptors release large amounts of Ca2+ into the cytoplasm, exhausting ATP stores in an attempt to pump it back in to the SR. The treatment is Dantrolene - inhibits Ryanodine receptors to act as a muscle relaxant.
1526
Why is primary hyperaldosteronism not associated with edema?
Aldosterone escape: 1) Pressure natriuresis 2) Increased delivery of sodium to distal nephron overrides aldosterone's action there 3) ANP
1527
What are the causes of renal artery stenosis?
Atherosclerosis - older person with other atherosclerotic dz Fibromuscular dysplasia - young woman of childbearing age Both can cause HTN due to renal artery stenosis. Fibromuscular dysplasia shows a "beads on a string" appearance on angiography.
1528
What is the DDx for malabsorption?
"These Will Cause Lasting Absorption Problems" ``` Tropical sprue Whipple's disease Celiac sprue Lactose intolerance Abetalipoproteinemia Pancreatic insufficiency ``` Also infectious causes (giardia, etc.)
1529
What is seen in Whipple's disease?
"Foamy Whipped cream in a CAN" Foamy (PAS+) macrophages in SI lamina propria Cardiac symptoms, Arthralgias, Neurologic symptoms Most commonly occurs in older men.
1530
What changes are seen in the bowel from celiac disease?
Blunting of villi & crypt hypertrophy in the distal duodenum & proximal jejunum
1531
What complications are seen with celiac disease?
1) Vitamin deficiencies 2) Small bowel carcinoma 3) Enteropathy-Associated T-cell Lymphoma (EATL)
1532
What are the types of chronic gastritis?
Type A - autoimmune (Type IV) against parietal cells Type B - H. pylori infection
1533
What is seen with Menetrier's disease?
Gastric hypertrophy (look like brain gyri) Protein-losing gastropathy Parietal cell atrophy ^Mucous cells
1534
What cancers are associated with H. pylori infection?
Gastric adenocarcinoma MALT lymphoma Esophageal SCC (protective against esophageal adenocarcinoma)
1535
What risk factors are associated with gastric adenocarcinoma?
``` H. pylori infection (intestinal type only) Smoked foods (nitrosamines) Achlorhydria Chronic gastritis Type A blood ```
1536
What immune deficiency is seen in celiac patients?
IgA deficiency If present, must test for IgG against gliadin, endomysium, tTG
1537
What vessel bleeds in a duodenal ulcer hemorrhage? | What is the additional risk?
Posterior wall ulcer --> gastroduodenal artery bleed This can cause acute pancreatitis!
1538
What diseases are associated with p-ANCA?
Ulcerative colitis Microscopic polyangiitis Churg-Strauss disease
1539
What is seen grossly with Crohn's disease?
``` Transmural inflammation Cobblestone mucosa Creeping fat Linear ulceration Stricture (string sign on barium x-ray) ```
1540
What is seen grossly in Ulcerative Colitis?
Mucosal & submucosal inflammation only Pseudopolyps Loss of haustra --> lead pipe appearance on x-ray
1541
What is seen microscopically in Crohn's disease? | UC?
Crohn's: Noncaseating granulomas Lymphoid aggregates UC: Crypt abscesses
1542
What conditions are associated with ulcerative colitis?
``` PSC Toxic megacolon CRC Pyoderma gangrenosum Ankylosing spondulitis Uveitis ```
1543
What conditions are associated with Crohn's disease?
``` CRC Migratory polyarthritis Erythema nodosum Ankylosing spondylitis Uveitis Kidney stones (malabsorption --> Ca2+ excreted in feces) ```
1544
What is the association between smoking and IBD?
Smoking ^ risk of Crohns | Smoking protects against UC
1545
What is the treatment for Crohn's disease?
The same as for RA ``` Steroids Azathioprine MTX Infliximab Adalimumab ```
1546
What is the treatment for ulcerative colitis?
Sulfasalazine (ASA prep) 6-MP Infliximab Colectomy
1547
What can be seen with diverticulosis? | What are the complications?
Usually asymptomatic Hematochezia Diverticulitis Fistulas
1548
What causes pneumaturia?
Diverticulitis --> colovesical fistula
1549
What is seen with a Meckel's diverticulum?
Persistence of the Vitelline duct, leading to: Melena RLQ pain Intussusception, volvulus, obstruction Dx: Pertechnate study for ectopic gastric tissue
1550
What causes intussusception?
Children: Viral infection (adenovirus & rotavirus) --> LAD --> leading edge Adults: Tumor is leading edge
1551
Where do intussusception & volvulus occur?
Intussusception: Ileocecal valve Volvulus: Children = cecum, Adults = sigmoid colon
1552
How can the RTA's be distinguished on UA/Labs?
Type 1 (distal) --> hypokalemia, urine pH > 5.5 Type 2 (proximal) --> hypokalemia, urine pH < 5.5 Type 4 (aldosterone) --> hyperkalemic
1553
What is seen in chronic lung transplant rejection?
Bronchiolitis obliterans The small airways are inflamed & then fibrosed, rather than the vascular beds as in other organ transplants.
1554
How does H. pylori lead to gastric ulceration? | Duodenal ulceration?
Gastric = local inflammation Duodenal = impaired somatostatin release --> ^gastrin --> ^acid
1555
What cells mediate the fibrosis seen in atherosclerosis?
Smooth muscle cells respond to endothelial injury signals (PDGF). These reactive smooth muscle cells are responsible for intimal hyperplasia & fibrosis.
1556
What signaling molecules mediate atheroma formation?
PDGF (from platelets, endothelial cells, & macrophages) | TGF-beta (from platelets)
1557
What general medical conditions are associated with carpal tunnel syndrome?
Hypothyroidism Diabetes Rhematoid arthritis Dialysis-associated amyloidosis (beta-2 microglobulin)
1558
How does T-ALL present?
Mediastinal mass It can compress local structures leading to SVC syndrome, dysphagia, or respiratory symptoms. Also the standard symptoms of lymphoma will be present.
1559
What is carnitine used for?
To shuttle fatty acids into the mitochondria for beta oxidation.
1560
What is the capsule of H. flu type B composed of?
Polyribosyl-ribitol-phosphate (PRP) Antibodies against this protein are associated with immunity against HiB. The vaccine is composed of PRP conjugated to diphtheria or tetanus toxoid.
1561
What are the common dystonias?
Spadmodic torticollis (cervical dystonia) Blepharospasm (eyes held closed) Writer's cramp
1562
What are the Class I antiarrhythmics?
"Double Quarter Pounder w/ Lettuce, Tomato, Mayo & More Fries Please" Ia: Disopyramide Quinidine Procainide Ib: Lidocaine Tocainide Mexiletine Ic: MORicizine Flecainide Propafenone
1563
What patients are at risk for succinylcholine-induced hyperkalemia? How does this occur?
Burns Myopathies Crush injuries Denervating injuries Succinylcholine constantly stimulates the AChR (a nonselective cation channel), allowing both Na+ influx and K+ efflux. When open for a long time, this can cause a lot of K+ efflux from muscle cells --> hyperkalemia
1564
What, within the brain, is the cause of the extrapyramidal side effects seen with antipsychotics?
D2 blockade in the nigrostriatal pathway
1565
What causes thigh claudication?
Atherosclerosis of the external iliac or femoral arteries.
1566
What is the most common cause of death by drug overdose?
Opioids
1567
What is the most common elbow injury in young children? | What causes it?
Radial head subluxation (Nursemaid's elbow) Caused by a sudden pull on an extended arm that is pronated. The annular ligament slips over the radial head & into the radiohumeral joint. The child will hold his arm in a pronated & extended position close to the body & not want to move it. Reduction occurs by full supination & flexion of the elbow.
1568
What is seen with Theophylline overdose?
Theophylline = a PDE inhibitor & stimulant used for asthma Overdose: Seizures Tachyarrhythmia
1569
What protein is used to secrete conjugated bilirubin into the bile canaliculi?
MRP2 It is an organic anion transporter & is energy-dependent. Uptake of unconjugated bilirubin at the basolateral membrane is passive.
1570
What is Tommy John surgery?
Ulnar collateral ligament reconstruction This is an injury commonly seen in pitchers due to intense valgus stress on the elbow.
1571
What is seen with Salicylate overdose?
1) Early respiratory alkalosis | 2) Late metabolic acidosis superimposed on the early alkalosis, resulting in a mixed acid-base disturbance.
1572
How can E. coli O157:H7 be distinguished from other strains in culture?
Does not ferment sorbitol | Does not produce glucuronidase
1573
How does hyperparathyroidism present histologically in bone?
Subperiosteal thinning
1574
What is seen with Osler-Weber-Rendu syndrome?
Hereditary hemorrhagic telangiectasia Autosomal dominant disease Telangiectasias on all mucous membranes Recurrent mucosal bleeding (epistaxis, GI, hematuria)
1575
What ligaments form the lesser omentum?
Hepatogastric & Hepatoduodenal ligaments
1576
Where does Inhibin B feedback? | Testosterone?
Inhibin B --> pituitary (only) Tesosterone --> pituitary & hypothalamus
1577
What bacteria is pyrrolidonyl arylamidase positive?
Strep pyogenes is PYR+ It was used before bacitracin to determine if it was GAS or GBS
1578
What is the treatment for hemophilia A?
Desmopressin --> vWF release --> ^Factor VIII levels
1579
What cell type does renal cell carcinoma arise from?
PCT tubular cells
1580
How do benzodiazepines affect the GABA receptor? | Barbiturates?
Benzos - increase frequency of GABA opening Barbs - increase duration of GABA opening
1581
What are the stages of a lobar pneumonia?
Congestion (24h) Red hepatization (days 2-3) Gray hepatization (days 4-6) Resolution
1582
What is the most common cause of death in TCA overdose?
Refractory hypotension & cardiac arrhythmia due to inhibition of Na+ channels in the heart. Use NaHCO3 to resuscitate.
1583
What causes unilateral SVC syndrome?
Obstruction of the brachiocephalic vein on one side. Can be caused by thrombosis or by external compression (tumor, etc.)
1584
What is the function of cord factor?
It is a virulence factor found on mycobacteria ``` Causes: Neutrophil inhibition Mitochondrial destruction TNF release Also causes linear growth pattern of mycobacteria ```
1585
What cosmetic side effects are seen with Phenytoin?
``` Hirsutism Coarse facial features Acneiform skin rash Gingival hypertrophy LAD (pseudolymphoma) ```
1586
What substances can cause Serine phosphorylation leading to insulin resistance?
TNF-alpha Glucagon Fatty acids Glucocorticoids
1587
What portions of the nephron are most susceptible to ischemic injury?
Proximal tubule & thick ascending limb
1588
What toxicities are seen with NRTI's?
``` Bone marrow suppression Peripheral neuropathy Lactic acidosis (Zidovudine, Didanosine, Stavudine) Rash Anemia (Zidovudine) ```
1589
What drug is used to test for Prinzmetal angina?
Ergonovine It stimulates both alpha-adrenergic & 5-HT receptors --> spasm
1590
What substances can inhibit iodide uptake at the thyroid gland?
Competitive anion inhibitors: Perchlorate Pertechnetate Also iodide can
1591
What does the stapes transmit vibration to?
The oval window The round window is the one that just relieves pressure.
1592
What is the helicotrema?
The apex of the cochlea
1593
What processes use NADPH?
Fatty acid, cholesterol, steroid synthesis Drug metabolism Antioxidant in RBC's
1594
What CN's can be compressed by an acoustic neuroma?
CNV --> paralysis of muscles of mastication & loss of corneal rflx CNVII --> facial paralysis & hyperacusis CNVIII ---> tinnitus, vertigo
1595
What infections are caused by Pseudomonas?
``` PSEUDO: Pneumonia (CF) Sepsis (ecthyma gangrenosum) External otitis (diabetics, severe) UTI Drug users Osteomyelitis in diabetics ```
1596
Sudden vision loss & a pale retina & cherry red macula
Central retinal artery occlusion (CRAO)
1597
At what lung volume is PVR the least? | Why?
At FRC At high lung volumes, alveolar vessels are stretched longitudinally & narrowed. At low lung volumes, extra-alveolar vessels are not pulled open.
1598
How is Strongyloides infection diagnosed? | Why must it be treated?
Larvae in the stool is diagnostic Must be treated because it can autoinfect the same host over and over, leading to superinfection.
1599
What step in the TCA cycle requires thiamine?
Alpha-ketoglutarate dehydrogenase
1600
What drugs can niacin interact with?
``` Potentiates vasodilators (decrease meds) Increases insulin resistance (^meds) ```
1601
What is the most prominent side effect of Amphotericin B?
Renal toxicity Patients often require daily supplementation of potassium and magnesium.
1602
What physical exam finding can be used to rule out aplastic anemia?
Splenomegaly
1603
What can cause unilateral nasal hemianopia?
Aneurysm or calcification of the internal carotid. It can compress the lateral side of the optic chiasm.
1604
What drugs (aside from antidepressants) can cause serotonin syndrome when taken with SSRI's?
Tramadol Ondansetron Linezolid
1605
What are the most common causes of primary amenorrhea with normal gonadotropin & hormone levels?
Imperforate hymen | Müllerian duct abnormalities (back of vagina, uterus, fallopian)
1606
What substances require tetrahydrobiopterin as a cofactor for their synthesis?
``` Serotonin (from tryptophan) NO (from Arg) Tyrosine (from Phe) Dopamine NE/EPI ```
1607
What antibodies cause idiopathic membranous nephropathy?
Anti-Phospholipase A2 (PLA2)
1608
How is the lac operon regaulated?
^cAMP --> ^transcription (elevated in the absence of glucose) ^lactose --> ^transcription
1609
What enteric pathogens can cause disease with a low inoculum?
Shigella (10) Campylobacter (500) Entaoeba histolytic (1) Giardia (1)
1610
Where does the pentose phosphate pathway occur?
Exclusively in the cytoplasm
1611
What portions of the urea cycle take place in the mitochondria?
Carbamoyl phosphate synthetase I | OTC
1612
What toxicities are seen with mannitol?
Pulmonary edema Dehydration Contraindicated in CHF & anuria
1613
How are serotonin syndrome & neuroleptic malignant syndrome different?
NMS - rigidity 5-HT - myoclonus Otherwise they are pretty similar (hyperthermia, autonomic dysfunction, altered mental status)
1614
What patients is Allergic Bronchopulmonary Aspergillus (ABPA) seen in?
Asthmatics that take steroids
1615
What is seen in neonates who's mothers had poor glycemic control during pregnancy?
Macrosomia | Hypoglycemia (beta cell hyperplasia)
1616
What are the 3 types of responses that can be seen after frontal lobe injury?
1) Disorganized 2) Disinhibited 3) Apathetic
1617
What is seen with scarlet fever?
Fever Pharyngitis Sandpaper-like rash Strawberry tongue
1618
What artery can be damaged by a broken hip?
Broken hip = fractured femoral head The medial femoral circumflex artery provides the majority of blood to the femoral head & neck. Injury can cause avascular necrosis of the femoral head.
1619
What is seen histologically with herpes zoster reactivation?
Intranuclear inclusions in keratinocytes | Multinucleate giant cells
1620
What is seen on CXR in acute LV failure?
Cardiomegaly Pleural effusions Kerley C lines Vascular shadowing
1621
What amino acids are the repeats in collagen?
Gly-X-Y X & Y can be lysine or proline but glycine must be present every 3rd amino acid. Thus, it is the most prevalent.
1622
What drug is used to treat trigeminal neuralgia?
Carbamazepine
1623
What causes methylmalonic acid in the urine?
1) Deficiency in Methylmalonyl-CoA mutase | 2) B12 deficiency
1624
When does calcific aortic stenosis become symptomatic?
Normal patient = 70's | Bicuspid aorta = 60's
1625
What is seen with shaken baby syndrome?
Bilateral retinal hemorrhages | Subdural hematoma
1626
Where are the hormones produced that are released by the neurohypophysis?
"OPAS" ``` Oxytocin = Paraventricular ADH = Supraoptic ```
1627
What are neurophysins?
Proteins that modify ADH & OXY as they are being transported to the posterior pituitary within vesicles. They are released along with the hormones into the hypophysial vein --> systemic circulation.
1628
What deficiencies result specifically in disseminated MTB?
IL-12 receptor deficiency IFN-gamma receptor deficiency Patients require lifelong treatment with antimycobacterial agents.
1629
What occurs with heme's affinity for oxygen when methemoglobin is present?
Methemoglobin cannot bind O2 The remaining ferrous iron in the Hb molecule has a higher affinity for O2, causing a left shift.
1630
What types of cancer can cause nonbacterial thrombotic endocarditis?
Mucinous adenocarcinoma of the pancreas Adenocarcinoma of the lung These cancers produce mucin & are associated with a hypercoagulable state. This is the same pathogenesis as Trousseau's sign.
1631
What are the class III antiarrhythmics?
"AIDS" Amiodarone Ibutilide Dofetilide Sotalol
1632
What classes of antiarrhythmics can cause torsades?
Class Ia & Class III
1633
What toxicities are seen with Daptomycin?
^CPK levels | Myopathy
1634
What bacteria are beta hemolytic?
Listeria Staph aureus GAS GBS
1635
What cardiac defect is seen in normal adults?
Patent foramen ovale seen in 20-30% of normal adults Normally held closed by LA >> RA pressures
1636
How does multiple myeloma present?
Fatigue (anemia) Constipation (hypercalcemia) Bone pain Renal failure
1637
Where can viridans strep adhere to?
Fibrin They produce dextrans from sucrose. These aid their adherence to fibrin at sites of endothelial damage.
1638
What sites are affected in GvH disease?
Skin, liver, & intestine
1639
What drugs can be used to prevent tumor lysis syndrome?
IV Fluids Allopurinol Rasburicase - recombinant urate oxidase (converts urate to allantoin, which is 10x more soluble)
1640
What gives elastin its elasticity?
Inter-chain crosslinking of lysine residues This is performed by extracellular lysyl hydroxylase
1641
What is the mechanism of Miravaroc?
It is a CCR5 receptor inhibitor Prevents viral attachment of HIV
1642
What is Modafinil used for?
As a non-amphetamine daytime stimulant in narcolepsy It is a CYP450 inducer
1643
What shape are calcium-based kidney stones?
Tetrahedral | envelope
1644
What shape are struvite renal stones?
Coffin lid
1645
What shape are cystine renal stones?
Hexagonal
1646
What shape are urate renal stones?
Rhomboid
1647
What is used to treat bedwetting?
1) Desmopressin | 2) Imipramine
1648
What cell surface markers are found on Reed-Sternberg cells?
CD15, CD30
1649
What are the lymphoma translocations?
8urkitt's = t(8;14) Mantle ce11 = t(11;14) Follicular = Fourteen eighteen = t(14;18)
1650
What distinguishes acute leukemia vs chronic leukemia on a bone marrow smear?
>20% blasts = acute
1651
What translocations in ALL are indicative of prognosis?
t(12;21) --> Good prognosis; seen in children t(9;22) --> Philadelphia+ ALL; Poor prognosis; seen in adults
1652
What leukemia can present with DIC?
APL (M3 form of AML) Auer rods can cause DIC. Thus, it can also occur following treatment.
1653
t(15;17)
APL Give ATRA
1654
What leukemia infiltrates the gums?
Acute monocytic leukemia
1655
What is "blast crisis?"
When CML accelerates and transforms into AML or ALL.
1656
What causes increased basophils?
CML
1657
What is seen on coagulopathy labs in uremic patients?
Uremic patients (dialysis, etc.) show normal PT, PTT, & platelet counts but an increased bleeding time. There are dialyzable platelet inhibitory factors responsible for this, thus it will improve following dialysis.
1658
Where are the lung markings?
Anteriorly the 4th rib = horizontal fissure (Right side only) The 6th rib = oblique fissure
1659
Where is the abdominal neurovascular plane?
Between the internal oblique & transversus abdominus muscles. All of the deep abdominal wall arteries & nerves lie here.
1660
What artery travels with the recurrent laryngeal nerve?
Inferior thyroid artery RLN can be damaged during thyroidectomy
1661
What are the K+ sparing diuretics?
Amiloride Triamterene Spironolactone Eplerenone
1662
What drugs lower TG's best?
Fibrates > Niacin
1663
What drugs lower LDL the best?
Statins > Ezetimibe
1664
What is Diphenoxylate? | What is its use?
It is an opiate anti-diarrheal It is combine with atropine to prevent abuse (unpleasant anti-cholinergic effects)
1665
What is the treatment for lice (pediculosis capitis/pubis)?
Permethrin or Pyrethrin
1665
What coronary artery supplies the pacemaker nodes? | Bundle of his?
The RCA supplies BOTH the SA node & AV node. The LAD supplies the bundle of his (supplies anterior 2/3 of the interventricular septum)