standinpt2 Flashcards
Dermatitis, enteritis, alopecia, adrenal insufficiency
B5 defiency (lack of CoA)
Peripheral neuropathy of hands/feet, angiokeratomas, cardiovascular/renal disease. Disease and defiency and buildup and inheritance.
Fabry’s, alpha galactosidase A (enzyme), ceramide trihexose, XR
Central and peripheral demyelination with ataxia, dementia. Disease and defiency and buildup and inheritance.
Metachromatic leukodystrophy, arylsulfatase A, cerebroside sulfate, AR
Chylomicrons and picking up apolipoproteins
Start with B48, get E and C2 from HDL. E does uptake, C2 allows LPL to break down, B48 does nothing
What stains with Giemsa?
Chlamydia, borrelia, rickettsia, trypanosomes, plasmodium
What bug is cystine-tellurite plate
Diptheriae
What bug is lowenstein jensen agar
M TB
Cat positives?
Candida, listeria, aspergillus, pseudomonas, s aureus, serratia, e coli
Urease positive bugs?
Crypto, h pylori, proteus, klebsiella, ureaplasma, nocardia, s epidermidis, s saprophyticus
What secretes Protein A?
Binds Fc region of Igs to prevent opsonization. Secreted by S aureus
How does shiga toxin work?
Inactivates 60S ribosome by removing adenine from rRNA
Which toxins affect cAMP? How?
Anthrax –>Edema factor–> Mimics adenylate cyclase enzyme to increase cAMP. Cholera –> Gs always on (ETEC heat labile is same). Pertussis –> disables Gi.
5 toxins encoded in lysogenic phage
Shiga-like, botulinum, cholera, diptheria, erythrogenic of GAS
Only bacterium with polypeptide capsule
Anthrax (contains D-glutamate)
Classic triad of chorioretinitis, hydrocephalus, and intracranial calcifications
Congenital toxo
Which RNA viruses don’t replicate in the cytoplasm?
Influenza and retro
HIV structural genes. Which does attachment? Which does entry? Which is capsid? And which does reverse transcriptase, aspartate protease, and integrase?
Attachment = gp120
Fusion and entry = gp41
Capsid= gag (p24)
And those others = pol
Notched teeth, saddle nose, short maxilla, saber shins, CN VIII deafness
Congenital syphilis (though most die)
Macular rash over body after several days of high fever.
Roseola (HHV-6)
Lymphogranuloma venereum vs granuloma inguinale
Venereum= c trachomatus. Inguinale = Klebsiella inguinale. Boht are painless, but venereum is more lymphatics.
Toxo treatment
Pyrimethamine
Ribavirin
Guanine-like, inhibits IMP dehydrogenase. RSV and chronic hep C. Teratogenic
Draw out HIV antivirals
Do it.
Sulfonamides cause what teratogenic effect?
Kernicterus
CD14
Macs. This is what LPS activates
Ataxia, telangiectasia, what else would you expect to see?
Defects in ATM gene, coding for DNA repair enzymes. End up with IgA deficiency in addition to name, also increased risk of cancer. AR. Will see increased AFP!
Absent pus, delayed separation of umbilical cord. What is the problem?
Defect in integrin (CD18). LAD. Adhesion part is integrins
When do you use alpha interferon
Hep B and C, Kaposi’s sarcoma, leukemias, malignant melanoma
This part of the lymph node communicates with efferent lymphatics and contains reticular cells and macs.
Medulla
This part of the lymph node houses T cells and is underdeveloped in patients with DiGeorge
Paracortex. In between follicles and medulla
Anal canal above the pectinate line drains to which lymph node? Below pectinate line?
Internal iliac for above, below = superficial inguinal
HLA A3
Hemochromatosis
HLA DQ2/DQ8
Celiac
What is the second signal for T cell activation for CD4 and CD8?
CD4 is B7 and CD28 interaction. CD8 is IL2 from CD4 Th1 cells
What are the defenses to prevent complement activation on self cells? What happens when these go awry?
Decay accelerating factor (DAF)–> GPI anchored. What is deficient in paroxysmal nocturnal hemoglobinuria. And C1 esterase inhibitor (deficient in hereditary angioedema–> ACEi’s are contraindicated).
Cytokines secreted by macs
1, 6, 8, 12, TNF alpha.
CD3
Associated with TCR
Patient presents with coarse face, cold abscesses, retained primary teeth, dermatologic problems. What would you expect the labs to show and what’s going on?
Hyper-IgE syndrome. Th1 cells can’t produce IFN-gamma, so neutrophils don’t respond to chemotactic stimuli
Cyclosporine: MOA and AE’s
Binds to cyclophilins and makes a complex, then the complex blocks differentiation and activation of T cells by blocking calcineurin. Calcineurin is required for IL-2! Autoimmune. AE’s: nephrotoxicity, HTN, gingival hyperplasia and hirsutism (like phenyto
Tacrolimus: MOA and AE’s
Inhibits calcineurin by binding to FK-binding protein (another name for drug is FK-506). Same toxicity as cyclosporine - the phenytoin ones (no gingival hyperplasia or hirsutism)
Sirolimus (rapamycin): MOA and AE’s
Inhibits mTOR, blocking T cell proliferation in response to IL-2. AE’s: hyperlipidemia, thrombocytopenia, leukopenia. MINIMAL NEPHROTOXICITY! Differentiates from tacrolimus and cyclosporine.
Azathioprine
Antimetabolite precursor of 6MP that interferes with the metabolism and synthesis of nucleic acids. Immunosuppressant.
If you have a C3 deficiency, you have an increased susceptibility for what?
Type III hypersensitivity. Low C3b means low opsonization, so can’t remove complexes.
2 areas most susceptible to ischemia in the kidney
Straight segment of proximal tubule (medulla), thick ascending limb (medulla)
What are the factors responsible for neutrophil migration?
Chemotactic signals: C5a, IL-8, LTB4, kallikrein
Mechanism of carbon tetrachloride damage
P450 breaks CCl4 –> CCl3 radical –> lipid peroxidation and membrane degeneration –> loss of apolipoproteins –> fat can’t leave liver –> fatty cahnge
Free radical formation and handling in cells
O2 –> O2 radical by NADPH oxidase–> H2O2 by superoxide dismutase –> OH radical by catalase –> H2O by glutathione peroxidase I think. Note: H2O2 –> HOCl is done by MPO in neutrophils to kill stuff.
How does iron poisoning present and mechanism?
Acute–> gastric bleeding. Chronic –> metabolic acidosis, scarring leading to GI obstruction. Cell death due to peroxidation of membrane lipids
Senile amyloidosis
Due to deposition of transthyretin (TTR) in myocardium and other sites. Slow progression, often asymptomatic. This very same protein can be mutated and cause heritable amyloidosis leading to restrictive cardiomyopathy earlier in life.
What allows for adherence to BM in neoplasia?
Increased laminin
What allows for invasion of the BM in neoplasia?
Metalloproteinases and cathepsin D protease
Desmoplasia
Fibrous tissue formation in response to neoplasm
3 neoplasms associated with facial angiofibroma, seizures, and MR
Tuberous sclerosis: giant cell astrocytoma, renal angiomyolipoma, and cardiac rhabdomyoma
2 neoplasms associated with Paget’s disease of the bone
secondary osteosarcoma and fibrosarcoma
C-kit is for what type of tumor? Oncogene or tumor suppressor?
GIST. Oncogene
Genes involved in melanoma (2), tumor suppressor or oncogene?
p16 and BRAF. P16 is tumor suppressor (like p53), BRAF is oncogene
NF1 and 2, tumor suppressor or oncogene?
Tumor suppressor
Describe the Rb pathway and what goes wrong with cancer.
Rb is usually bound to E2F which blocks transcription (this is in unphosphorylated state). When growth factor activates cyclin D which activates cyclin dependent kinase 4, phosphorylates Rb protein and E2F pops off and now can have transcription of enzyme
Bombesin
Neuroblastoma, lung and gastric cancer marker
Nitrosamines hit the ______ and cause _______
Stomach and gastric cancer
Where does arsenic hit and what cancers does it cause?
Skin –> squamous cell and liver –> angiosarcoma. Arsenic fucks with lipoic acid to inhibit all the enzymes that need it (like pyruvate dehydrogenase) and oxidative phosphorylation, causing free radicals and decreased energy production
Alkylating agents cause cancer where?
Blood: leukemia
Which neoplasms make PTHrp?
Squamous of the lung, RCC, breast
Which cancers make too much vitamin D?
Hodgkins
Which cancers do EPO?
RCC, hemangioblastoma (associated with von hippel lindau), HCC, pheo
Metastases to brain
Lung>Breast>GU>osteosarcoma>melanoma>GI
