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Melson's Step 1

High Yield info for step 1

What stage of sleep is characterized by Bruxism and what EEG waveform is seen? Stage 2 with sleep spindles and K complexes
What stage of sleep is characterized by sleepwalking, night terrors and bedwetting and what EEG waveform is seen? Stage 3/4 and Delta waves are seen
What neurotransmitter initiates sleep and where does it do so? Serotonin through the Raphe Nucleus
What is the principle neurotransmitter involved in REM sleep? What neurotransmitter reduced REM sleep? ACh mediates it, NE reduces it
What region of the brain mediates EOM use during REM sleep? What else is it involved in? Paramedian Pontine Reticular Formation (PPRF), also does saccades and nystagmus
What drug is useful for treating enuresis? How does it do this? Imiprimine, decreases Stage 3/4 sleep
What drug is useful for treating night terrors and sleep walking? Benzos
What does depression do to sleep patterns? It increases REM sleep, decreases REM latency, and leads to multiple awakenings including early morning awakenings
What drugs can be used to help induce sleep in depressed patients? What should be considered prior to starting these drugs? Trazodone is good, as are TCAs like amitriptyline/doxepine but get EKG first to avoid arrhythmias
What drug can be used in long-term treatment of insomnia? Eszopiclone (Lunesta)
What drugs can be used to treat restless legs syndrome? Pramipexole or Ropinirole (Dopaminergics)
What diagnosis is conferred by the presence of cataplexy? How is it treated? What other symptoms are seen? Narcolepsy, Amphetamine (modafinil), and sodium oxybate (GHB), Visual hallucinations before bed (hyponogogic) and right before awakening (hyponopompic)
What age can you diagnose nocturnal enuresis? What age should you treat it? How should you treat it? At age 5 (chronologic and developmentally) it is diagnosed, at age 7 you treat, starting with behavioral (enuresis alarm) and THEN drugs (imipramine, ddAVP, indomethacin suppository)
What quick association is helpful for remembering the 1st branchial arch derivatives? What is the condition associated with a failure in development here? The M’s and T’s (Malleus, Mandible, Muscles of Mastication, Masseter, Tensor Tympani, Trigeminal nerve, Treacher-Collin’s syndrome)
What quick association is helpful for remembering the 2nd branchial arch derivatives? It is the S’s (Stapedius, Stylohyoid, Styloid process, Seventh CN, helps you SMILE)
What quick association is helpful for remembering the 3rd branchial arch derivatives? What is the condition associated with a failure in development here? The word pharyngeal (stylo-pharyngeal innervated by glossopharyngeal), failure = congenital pharyngo-cutaneous fistula (tonsil to cleft cyst)
What is the treatment regimen for poisoning with parathion? What is the mechanism of action(s) Atropine (anticholinergic) and pralidoxime (regenerates ACHase)
What are some used for octreotide? Pituitary excesses from tumors (acromegaly, Thyrotropinoma, ACTH tumor), Gi endocrine excesses (ZE syndrome, carcinoid, VIPoma, Glucagonoma, Insulinoma) and portal HTN (bleeding varices/peptic ulcer->reduces splanchninc circulation)
What is Malasma? What is the cause? ”The Mask of Pregnancy” caused by increased estrogen upregulating alpha-MSH release from the POMC of the anterior pituitary. This increases the pigment around the face of pregnant women
What are the symptoms of each congenital adrenal hyperplasia? 21-OHase deficiency: Masculinization, Hypotension, 17-OHase deficiency: Female Genotype, Hypertension. 11-OHase deficiency: masculinization and hypertension (no salt wasting)
What are the effects of cortisol? Thin skin, decreased immunity, ^WBCs, abd straiae, moon face, buffalo hump, DM, Osteoporosis, Acne, Peptic ulcer, psychosis, ^lipids, insomnia, bruises, glaucoma, truncal obesity
Patient with petechial rash and coagulopathy can lead to what adrenal issue? Waterhouse Friedrichsons disease = Neisseria Meningitis mediated adrenal hemorrhage->DIC
What is tertiary adrenal insufficiency? Iatrogenic adrenal insufficiency from immediate withdrawl of exogenous glucocorticoids after having been on them for >1 week
What are the differences between primary and secondary adrenal insufficiency? Primary = low renin, ^a-MSH = pigmentation, insufficient aldo. Secondary has sufficient ALDO, no salt wasting, no pigmentation
What are the breakdown products seen in urine of pheochromocytomas? How is it different than that of neuroblastoma? VMA, HVA and (nor)metanephrines as opposed to neuroblastoma = only HVA
What are the pathways of the G protein receptors? Which receptors are which type? Gq = PLC->PIP2->IP3/DAG->^Ca/PKC. Gs/i->AC->^cAMP->PLA. Gq = a1, H1, M1/3, V1. Gi = a2, d2, m2. Gs = the rest. HAV 1 M&M, MAD2s
What are the centrally acting a-2 agonists? What are they used for? What is special about each? Clonidine (works in ESRD), a-methyldopa (safe in pregnancy), Guanefasine (may shut down amygdala) all used for BP lowering, clonidine also for kids with ADHD
What is a common side effect of the alpha 1 inhibitors (-zosins)? First dose orthostatic hypotension, start low and go slow, dose at night before bed for this reason.
What is mertazipine, what are its unique side effects? Alpha 2 agonist that increases appetite & sedates. Good for treating HTN in depressed patients
Why do beta-blockers treat hypertension? They decrease CO and reduce renin release from JG cells
Which beta-blockers are b1 specific? Which are non-specific? Which are also alpha agonists? B1 specific = A-M Non specific = N-Z. Except for Labetalol and Carvedilol which are both alpha and beta antagonists
Which beta-blockers are cardioprotective? Carvedilol and Metoprolol
What is unique about nadolol? Long half-life?
Which beta-blockers are partial agonists? Pindolol, atenolol?
What drugs should not be given to a patient on cocaine overdose? Beta Blockers
What is the drug type of choice for aortic dissection? Why? Beta blockers, they reduce the rate of BP increase and they decrease the CO in general
What can beta-blockers be used for? HTN, CHF, Aortic Dissection, Glaucoma, Angina, SVTs, Post MI, Pheo control (with phenoxybenzamine)
What are the sensory, motor and taste inputs to the tongue based on location? Anterior 2/3 (Sensory from V3, Taste from VII) Posterior 1/3 (Sensory and Taste from 9/10), Motor always from 12
What is Osler-Weber-Rendu syndrome? What is the inheritance pattern? AD conditions where you get Telangiectasias everywhere and get GI bleeds, Epistaxis, Fe deficiency anemia, AVMs etc
What can cause gingival hyperplasia? Phenytoin (Cyclosporine and Calcium antagonists)
What is the most common malignant tumor of the salivary glands? Where does it most commonly occur? What other tumors are there in salivary glands? Mucoepidermiod Carcinoma, usually in the sublingual glands. There are also, Warthin Tumors and Pleomorphic adenomas
What is the disease with situs inversalis and reccurent URT infections? What else will they experience? Kartagener’s Syndrome, will also be infertile
What cancers are associated with RET mutation? MEN 2A/2B, Medullary and Papillary Carcinoma of thyroid
What drugs is used for anaphylactic shock? Cardiogenic shock? Septic shock? Epi, Dobutamine, NE
What type of hypersensitivity is Graves disease? Type 2 Hypersensitivity
What are the thioamides, what is their MOA and what are the dangerous side effects, what about in pregnancy? PTU and Methimazole, both prevent organification (PTU also blocks 5’-deiodinase), both can cause agranulocytosis and aplastic anemia (PTU = safer in pregnancy), PTU is nephrotoxic?
What drug requires that you check your PFTs, LFTs and TFTs? Amiodorone, pulm fibrosis, liver toxicity and Jod-Basedow (hyperthyroid) or Wolff-Chaikoff (hypothyroid)
If a patient has a radio-contrast scan and becomes hyperthyroid, what happened? Jod-Basedow reaction, too much iodine given to someone who was deficient led to hyperthyroidism
In what cancers can you see psammoma bodies? PSMM (Papillary Thyroid Ca, Serous CystadenoCA of ovary, Meningioma, and Malignant Mesothelioma
A person who was treated with radiation for leukemia as a child is most likely to present with what cancer as an adult? Papillary Thyroid Carcinoma
What cancer is associated with BRAF mutation, what other mutations is it associated with? Papillary Carcinoma of the thyroid, also associated with RET and NTRK1
What HLA subtypes are associated with diseases? Which diseases? HLA-A3 = hemochromatosis, HLA-B27 = Seronegative Spondyloarthropathies. HLA-DR3/4 = DM type 1
What are the four types of antigen presenting cells? Dendritic Cells, B-cells, M-cells, Macrophages
Where are Langerhans cells found? What do they do? Skin histiocytes, they are APCs for the skin
If you see a tennis racket shaped cell on EM, what is it called, what disease, what cell surface markers are present? Birbeck Granule, Langerhans Cell Histiocytosis, S-100 and CD1a positive
What cytokines affect T-cells, Macrophages and B-cells? T=IL-2, Mphages = IFN-gamma, B-cells = IL4,5
Where does positive selection occur for T-cell maturation? Negative Selection? Both take place in the corticomedullary junction of the thymus
What are the two signals needed for CD4 and CD8 t-cells to activate? CD4 needs MHCII and B7/CD28. CD8 needs MHCI and IL-2
What cytokines are produced by Th1 and Th2 cells? What cytokines inhibit each one from forming? Th1 is inhibited by IL-10, Th2 is inhibited by IFN-gamma
What cytokines activate NK cells? IFN-a,b, IL-12,
What are regulatory t-cells? What does each type help prevent? CD4 regs prevent autoimmune diseases through CD-12 and TGF-b, CD8 regs prevent graft rejection
What cell surface protein is unique to macrophages, what does it do? CD14, mediates inflammation via IL1,6 and TNFa
What does IFN-a,b do to neighboring cells? What is the mechanism by which is does this? IFN-a,b puts surrounding cells in an anti-viral state by upregulating ribonuclease which can cleave viral proteins/peptides
What cytokine activates macrophages? What cytokines do macrophages produce? IFN-a, produces: IL1,6,and TNFa
What amino acids are modified by the Golgi apparatus? Serine, Threonine, Aspartamine
What drugs have anti-cholinergic side effects? TCAs, Antihistamines, Amantadine,
What are the cell surface markers for B-cells? CD19,20,21, Both MHCs, CD40, IgM, IgD, B7
What are the two types of light chains that make up Abs? What is their normal ratio? What is happening if the ratio is thrown off? Kappa and Lambda, normally 2K:L, if not its possibly multiple myeloma
What is a thymus independent antigen? Why does it matter? Thymus independent antigens create an immune response but not a cell-mediated one and no future protection is conferred, must conjugate capsules and LPS to proteins to create lasting vaccination
What is Hydralazine? What are its side effects? ^GMP (lowers BP), causes drug induced lupus
What does IL-12 do? drives Th1 production and prepares for anti-viral state, activated NK-cells
What is the cause of PNH? What happens? Decay accelerating factor deficiency (CD55/59), can’t form GPI anchor on RBC to prevent compliment mediated attack. You get intravascular hemolysis of RBCs->PNH, Fe deficiency anemia, thrombosis
How do you diagnose PNH? How do you treat? Hamm’s test, RBCs lyse at a very low pH, activation of compliment. Or you could do flow cytometry and see lack of CD55/59. Rx = iron replacement, Warfarin, BM transplant eventually
What is the cause of hereditary angioedema? What drugs are contraindicated here? C1 esterase inhibitor deficiency, don’t give ACE-inhibitors, it can cause this alone
What compliment factors induce anaphylaxis? Which opsonize bacteria? Which does neutrophil chemotaxis? (what else does chemotaxis) C3a/C5a, C3b opsonizes, C5a does chemotaxis (with LTB4, IL-8 and Kallikrein)
What are some examples of type III hypersensitivities? SLE, RA, Polyarteritis Nodosum, Arthus reaction, Serum Sickness, Post-Strep Glomerularnephritis
What type of hypersensitivity reaction is DM type I? Arthus reaction? Contact dermatitis? Eczema? Goodpastures? PAN? MS? GVH disease? SLE? ITP? 4,3,4,1,2,3,4,4,3,2
What are the causes of eosinophilia? DNAAACP: Drugs, Neoplasms, Atopy/Asthma/allergies, addisons, acute interstitial nephritis, collagen vascular disease, parasites (helminthes)
What is Bruton’s Agammmaglobulinemia? X-linked, Boys, Poor B-cell maturation, defective BTK tyrosine kinase, low Igs, recurrent bacterial infections after age 6 months (when mom’s immunity goes away)
What is Digeorge’s syndrome? Thymic Aplasia, no t-cells, no PTs, tetany, heart/vessel defects, recurrent fungal, viral, protozoal infections, 90% have 22q11 deletion
What is SCID? defect in stem cell matur, many genes defects (adenosine deaminase deficiency), only line of defense is NK cells so you see, severe recurrent infection (candidiasis, RSV,VSV,HSV, measles, flu, PCP pneumonia), chronic diarrhea, FtT, no thymic shadow on CXR
What is the result of adenosine deaminase deficiency? SCID (infections, diarrhea, failure to thrive with lack of thymic shadow)
What is the disease with severe recurrent infections, chronic diarrhea and failure to thrive? SCID
What is Chronic Mucocutaenous Candidiasis? T-cell dysfunction with Candida, Rx = ketoconazole
What is Wiskott Aldrich Immunodeficiency? WAITER (Wiskott Aldrich Immunodeficiency, TCP (Purpura), Eczema, Recurrent pyogenic infections) No IgM against capsular bacteria, high IgA, x-linked
Which immunodeficiency syndromes are/can be X-linked? WBC (Wiskott, Brutons, Chronic Granulomatous disease, hyperIgM)
What are the hyperIgM inheritance patterns? XLR = No CD ligands, AR = No CD40, AD = NEMO
What is Ataxia-Telangiectasia? IgA deficiency with cerebellar ataxia, telangiectasias >5 y/o, poor smooth pursuit with eyes, ^ cancer risk (no x-rays!) DNA repair defect disorder, +/- ^AFP in kids, average age of death = 25 y/o
What are the selective immunoglobulin deficiencies? IgA deficiency is most common, most are healthy, with sinus and lung infections, associated with atopy/asthma, possible anaphylaxis to blood transfusions
What does IL-12 receptor deficiency cause? Increased mycobacterial infections
What are the phagocytes deficiency disease? Chronic granulomatous disease, Chediak-Higashi syndrome, Job syndrome, Leukocyte adhesion deficiency syndrome
What is Chronic Granulomatous Disease? Lack of NADPH oxidase leads to inability to form H2O2, susceptible to organisms with catalase (S. Aureus, E. Coli, Klebsiella, Aspergillus, Candida). Give them TMP/SMX prophylaxis and IFN-gamma
How can you diagnose Chronic Granulomatous Disease? Nitroblue Tetrazolium dye test, will be + (turn blue), if NADPH oxidase is present. So with CGD it is negative.
What is nitroblue tetrazolium dye test used to diagnose? What is the result in this disease? Chronic Granulomatous disease, it will be negative (no change from yellow to blue/black)
What is Chediak Higashi Syndrome? Defective Lysosomal transport LYST gene, see Giant granules in PMNs, with partial albinism, recurrent respiratory tract/skin infections and neurologic disorders (peripheral neuropathy, seizures etc)
What is Hyperimmunoglobulin E syndrome? Aka Job syndrome, deficient IFN-gamma (from t-cells) makes neutrophils fail to respond to chemotactic stimuli, high levels of IgE and eosinophils. You see, eczema, recurrent cold staph abscesses (Boils), course facial features. Retained primary teeth
What does a patient have if they have 2 rows of teeth? Job syndrome (HyperIgE syndrome)
What is leukocyte adhesion deficiency syndrome? Abnormal integrins lead to inability of phagocytes to leave circulation and you first see delayed separation of the umbilicus
If a child is born and retained his umbilicus for over a month, what is likely wrong? Leukocyte Adhesion deficiency
If a child gets recurrent staph abscesses, and neutrophils fail to respond to chemotaxis, what is the disease? HyperIgE aka Job’s Syndrome
If a child has pale skin, gets recurrent infections and has seizures, what is the diagnosis? Chediak Higashi’s syndrome
Ring enhancing lesions in an AIDS patients? Toxoplasmosis brain abscesses
Opportunistic infections in AIDS patients, superficial vascular proliferation? Chronic diarrhea? Encephalopathy? Abscesses? Meningnitis? Retinitis? Bacillary angiomatosis (Bartonella Henselae), Cryptosporidium, PML (JC Virus), Toxoplasmosis (ring enhancing), Cryptococcus (india ink), CMV
Opportunistic neoplasms in AIDS patients, superficial proliferation of vasculature? Hairy leukoplakia, non-hodgkin’s lymphoma, squamous cell CA, primary CNS lymphoma? HHV8 (kaposi’s sarcoma), EBV, EBV (often of oral cavity), anus (MSM) or cervical (HPV16/18), EBV (focal/multiple lesions)
Opportunistic respiratory infections in AIDS patients, interstitial infiltrates CD4<200? CD<50? Pleuritic pain and hemoptysis? Biopsy with intranuclear inclusion and pneumonia? PCP pneumonia, Mycobacterium Avium-intracellulare, invasive aspergillosis, CMV
What medication should be started for prophylaxis in CD4 count <200? TMP-SMX for PCP, Dapsone if sulfa allergy (careful for G6PD), aerosolized pentamadine
What medication should be started for prophylaxis in CD4 count <100? TMP-SMX for toxoplasmosis or dapsone + pentamidine +leucovorin (rescue)
What medication should be started for prophylaxis in CD4 count <50? Worry about MAC, give Azirthromycin once weekly
Which HIV drugs cause nephrolithiasis? Indinavir and Atazanavir (also get jaundice)
What is the drug class ending for protease inhibitors? -navir (Nevir tease a protease)
What are the HIV viral envelope proteins? What drugs target these specifically? gp41 (fusion and entry, targeted by Enfurvitide) and gp120 (binds to CD4 and either CCR5 or CXCR4, only R5 type is targeted by maraviroc)
What diagnostic tests are available for HIV? What order should they be done in? What are some special considerations regarding timing and age? ELISA = sensitive, Western Blot = specific, must do viral load though to catch in window period or to confirm newborn with positive tests (many false +)
What are the three structural genes that code for HIV proteins? pol (reverse transcriptase), env (gp120/41) and gag (p24 = capsid protein)
What HIV drug causes false positive on the Cannabis drug screening test? Efavorenz
What is the only drug available that blocks HIV viral integrase? What is its main side effect? Raltegravir, causes dyslipidemia
Which HIV drug (classes) fit the following side effect: Lactic acidosis, GI intolerance, pancreatitis, megaloblastic anemia, rash, metabolic derangements, BM suppression? NRTIs, Protease Inhibitors, Didanosine, Zidovadine, NNRTIs, Protease Inhibitors, Zidovadine
Which drugs should be used in the event of an HIV needle stick exposure? Zidovadine & Lamivudine
In a biopsy of esophagitis, you see large pink intranuclear inclusions and host cell chromatin that is pushed to the edge of the nucleus, what is it? HSV esophagitis
In a biopsy of the esophagus, you see enlarged cells, intranuclear and cytoplasmic inclusions and a clear perinuclear halo? CMV esophagitis
What condition is caused by anti-smooth muscle antibodies? Autoimmune hepatitis
If you see gastric rugae that are so hypertrophied they look like brain gyri, what is the cause and what is seen? Menetrier’s disease = gastric hypertrophy, parietal cell atrophy and increased mucus cells due to hypersecretion of acid. Precancerous lesion
How does metabolic acidosis/alkalosis affect potassium levels? Acidosis drives H+ into cells in exchange for K which leads to hyperkalemia. Alkalosis pulls H+ out of cells by pushing K into cells = hypokalemia
Biopsy reveals neutrophils above the BM, loss of surface epithelium and fibrin containing purulent exudate in the stomach, what is the problem? Acute gastritis (probably H. pylori, stress, NSAIDs, ulcer, ETOH etc)
Small intestine biopsy reveals small lymphocytes with irregular nuclear contours and proliferation of these lymphocytes into the mucosa and epithelial glands, what is the issue? MALToma
Stomach biopsy reveals lymphoid aggregates in the lamina propria with columnar absorptive cells and atrophy of glandular structures, what is the issue? Chronic Gastritis
What causes diffuse thickening of the gastric folds, elevated serum gastrin, and glandular hyperplasia without foveolar hyperplasia on biopsy? Zollinger Ellis syndrome
What drug could be given to directly counter a NSAID induced stomach ulcer? Misoprostol
What are the common areas of metastasis for gastric adenocarcinoma? L-supraclavicular node (Virchow’s), paraumbilical (Sister mary joseph nodule), ovaries (Krukenberg tumor), liver (Hematogenous)
What is the tumor marker for gastric cancer and what histological feature can you see in gastric adenocarcinoma cells (name the other cancer that also has this feature)? CEA = tumor marker, Signet ring cells are seen (as well as in lobular carcinoma in situ/lobular carcinoma of the breast)
What condition does a first born males that is 2 weeks old with hypokalemia and hypochloremia have? Pyloric stenosis, leads to excess vomiting, loss of Cl->metabolic alkalosis->hypokalemia (H/K exchange)
If you see a double bubble on X-ray, what genetic abnormality should you be looking for? Double bubble = duodenal atresia which is associated with down syndrome = trisomy 21
What is the embryonic origin of the spleen? What about its blood supply? The spleen is derived from mesoderm (not endoderm) and it comes from dorsal mesentery. Supplied by celiac artery however which supplies the rest of the foregut (endoderm)
What structures are retroperitoneal? SADPUCKER = Suprarenal (adrenal glands), aorta, duodenum (not 1st part), Pancreas (except tail), ureters, Colon (decending/ascending), Kidneys, Esophagus (lower 2/3), Rectum (upper 2/3)
What GI ligament contains the hepatic triad? What is in the hepatic triad? Hepataduodenal ligament contains the common bile duct, portal vein and hepatic arteries
What GI ligament would you cut into to access the lesser sac during surgery? Gastrohepatic ligament
What ligament(s) would need to be cut to remove the spleen? what structures are contained here? you would cut the gastrosplenic (short gastric arteries) and the splenorenal (splenic artery/vein)
What are the portosystemic anastomoses? What about arteries that would bypass abdominal aorta? Vein = esophageal, rectal, and umbilical. Arteries = epigastric (internal thoracic->external iliac), pancreaticoduodenal (celiac->SMA), middle colic/left colic (SMA->IMA), Superior rectal/middle (IMA->internal iliac)
How would you treat life threatening portal hypertension if pharmacologic therapy fails? TIPS procedure, Transjugular Intrahepatic Portosystemic Shunt
Which type of glucose load stimulates faster insulin release, IV or Oral? What mediates this effects? Oral glucose load stimulates Gastric Inhibitory Peptide (GIP) to cause the release of insulin and to inhibit gastric acid secretion
What drug should be used in diabetic patients with retained food in stomach? How does this drug work? What is the name of this condition? Gastroperesis can be treated with Metoclopramide (5-HT4 agonist, D2 antagonist)
What drugs are known for causing seizures (lowering the threshold)? Metoclopramide, Buproprion, Tramadol, enflurane, evening primrose oil
What anti-biotic can be used to stimulate the migrating motor complex? How does it do this? Macrolides such as erythromycin stimulate motilin which increases MMC activity
What are the common causes of acute pancreatitis? BADHHITS = Biliary, Alcohol, Drugs, HyperTG, HyperCa, Idiopathic, Trauma, Scorpion stings Also GETSMASHED= Gallstones, ethanol, trauma, steroids, mumps, autoimmune, scorpion sting, hyperCa/hyperTG, ERCP, drugs (sulfa, NRTIs didanosine, ratinovir)
What do scorpion stings cause? Black widow bites? Brown recluse bites? Acute pancreatitis, tetany (increase Ach release at nerve terminal), necrotoxicity
What pancreatic enzyme activates the other pancreatic enzymes for digestion? What activates this first enzyme? Trypsinogen is converted to trypsin via enterokinase/enteropeptidase on duodenal mucosa, trypsin then can activate the other zymogens (chymotrypsin, elastase, peptidases)
How do sugars get absorbed? They are broken down by salivary amylase and then oligosaccharide hydrolases (lactase, maltase etc). Glucose and Galactose use SGLT1, Fructose is passively diffused through GLUT-5, all then enter blood via GLUT-2
What anti-biotic would you not give to children because it causes cartilage issues? What condition would you consider giving it anyways and why? Fluroquinalones cause cartilage damage but in CF you might need to give it to fight pseudomonas that is resistant to other things already?
What is the antidote to acetaminophen overdose? What else can it be used for? N-acetylcysteine is an antidote and its used in Cystic fibrosis to break up plugs via cleavage of disulfide bonds in mucus plugs
What is the name for redness and tenderness on palpation of extremities that moves to different locations? What is it a sign of? Any other signs of this condition? Migratory Thrombophlebitis (Trousseau’s syndrome) is indicative of pancreatic cancer, can also so Courvoisier’s sign (obstructive jaundice with palpable gallbladder)
What causes acanthosis nigricans? Both visceral malignancies and insulin resistance
What are contraindications to metformin use? What potential side effect can they cause? Chronic renal failure or IV contrast dye can lead to messed up kidneys which can lead to life threatening Lactic Acidosis which is rare but very bad from Metformin. Liver disease and heart disease also not helpful
What do the sulfonylureas do? How? diabetes meds that block K channel and increase insulin release. Opposite of diazoxide
What do the glitazones do? How? Bind P-PAR gamma to increase insulin sensitivity by upregulating nuclear transcription factors. Cause weight gain, edema and can make CHF worse or cause MI’s rosaglit
What are the alpha-glucosidases? How do they work? Anti-diabetes drugs (acarbose and miglitol) that block oligosaccharide hydroxylases preventing glucose uptake in post-prandial period. Cause GI issues, not used often
What is Sitagliptin/Saxagliptin? How do they work? Anti-diabetes drugs that inhibit DDP-4, the enzyme which degrade incretins (endogenous chemicals that decrease glucagon, increase insulin and delay gastric empyting)
What are exenatide and liraglutide? How do they work? Anti-diabetes drugs that mimic incretins (endogenous chemicals that decrease glucagon, increase insulin and delay gastric empyting) Exenatide is similar to gila monster venom, liraglutide is synthetic human incretin (may cause pancreatitis)
What is pramlintide? How does it work? Anti-diabetes drug, it is an Amylin analog (amylin is usually released in same vesicle as insulin, it reduces glucagon and delays gastric empyting) can only be used along with insulin
What are the toxicities of insulin? lipodystrophy (at injection site), hypoglycemia, weight gain
If you see PAS+ foamy macrophages on intestinal biopsy of an old man, what is it? What will be the presentation? What about treatment? Whipple’s Disease (Tropheryma Whippelii) WHIPped cream in a CAN = Cardiac, Arthralgias and Neurologic symptoms, must be on TMP/SMX for 1 year after 10 days of antiobiotics
What are some features of celiac sprue? What anti-bodies are seen? What conditions are seen associated with it? Anti-gliadin, Anti-endomysial, Anti-tissue Transglutaminases all seen, blunting of microvilli in proximal small bowel with lymphocyte aggregation (^ t-cell lymphoma risk), associated with dermatitis herpetiformans
If a northern European female has GI issues and a large rash that has vesicles on a red background what is it and what is this called? Dermatitis Herpetiformans associated with celiac sprue
What condition prevents a person from forming chylomicrons and leads to fatty accumulation in the enterocytes? Abetalipoproteinemia
What condition is associated with migratory polyarthritis, erythema nodosum, kidney stones and granuloma formation on biopsy Crohns disease, transmural inflammation of intestinal wall w/ creeping fat, cobblestone appearance and string sign/skip lesions, rectal sparing
If a person comes in with 6 months of loose bowels and sudden urges that are relieved upon defecation, what is the diagnosis? What things must be ruled out and what symptoms would worry you? This is IBS, must rule out celiac sprue, IBD etc, should not have anorexia, pain at night, weight loss or malnutrition
What viral illness predisposes a child to having current jelly stool, what would be the cause? Adenovirus can cause inflammation of peyer’s patches which can cause intussceception
What two things can cause meconium ileus? Hirschbrungs and CF
If an elderly patients comes in with pain out of proportion to physical exam in abdominal with history of pain after meals and weight loss, what is the diagnosis? Ischemic colitis, usually at splenic flexure and distal colon
Where is angiodysplasia most often found in elderly people with GI issues? Right side, cecum, terminal ileum and ascending colon
If a tumor is found in the appendix that shows dense core granules on EM, what is it? What will it cause? It is a neuroendocrine tumor, likely carcinoid. It produces 5-HT but will not cause any symptoms unless it metastasizes because the liver breaks down serotonin. If it mets to liver you get BFRD, bronchospasm, flushing, R-sided value issues, Diarrhea
What are the symptoms of a carcinoid tumor? Where are the found? BFDR, bronchospasm, flushing, diarrhea, R-sided heart murmur, found in small intestine, appendix and lung. Don’t cause symptoms if they drain into liver first (metabolizes the serotonin)
If a patient comes in with wheezing, a red face, and complains of diarrhea. What should you include in your physical exam to confirm the diagnosis? Carcinoid syndrome is likely so auscultate their heart for R-sided heart murmur and look for edema/ascites
What is the most common glycolytic enzyme deficiency? How does it present? Pyruvate Kinase deficiency presents as hemolytic anemia (RBCs can’t get energy so they lyse)
How will Fructose-2,6-bisphosphatase levels change in the liver and muscle in response to sympathetic stimulation? It will decrease in the liver as you want to decrease glycolysis here, but it will increase in muscle as you want glycolysis to take place (sugar where you need it)
What enzyme drives glycolysis? What important enzyme feeds into it through a side path? PFK1 stimulates F6P to go to F1,6,BP but PFK2 makes F2,6BP which stimulates PFK 1 all of which drive glycolysis
What are the irreversible enzymes of gluconeogenesis? Which is rate limiting? pyruvate carboxylase, PEP carboxykinase, Fructose1,6,bisP (rate limiter), and G6P (not found in muscle so glucose stays trapped in)
Which glycogen storage disease can cause myoglobinuria? What enzyme is deficient? McArdle’s disease, Glucogen Phosphorylase deficiency causes glycogen to build up in muscle with exercise and draws in water which causes swelling and myolysis. Can lead to Rhabdyomyolysis also
What condition causes severe fasting hypoglycemia? What is happening here? Von-Guirks disease is G6Pase deficiency Lysosomal storage disease where liver, intestines, kidney can’t form glucose in last step in glycogenolysis or gluconeogenesis which leads to fasting hypoglycemia, it can’t share!
What things are increases in serum and on biopsy in Von-Guirks disease? Fasting hypoglycemia, ^blood lactate (damage cells), ^^glycogen in liver->hepatomegaly
What causes Pompe’s disease? What is the presentation? Lack of Lysosomal a1-4 glucosidase causes Lysosomal storage disease, cardiomegaly and early death in kids, respiratory failure and diaphragm issues in adults
What two amino acids are used to carry nitrogen from the blood to the liver? Alanine and Glutamine
What is the cause of garlic smelling breath with diarrhea/vomiting? Arsenic exposure, blocks the Lipoic acid aspect of pyruvates conversion to acetyl co-a. TLC For No-one
What are two forms of acquires pyruvate dehydrogenase deficiency? How does it manifest? Loss of any of the vitamins needed to make the complex, TLCForNo-one, esp Vit B1 deficiency in alcoholics but also arsenic exposure (blocks lipoic acid->Gastroenteritis with garlic breath)
What are 3 uncoupling agents and what do they do? 2,4 DNP (wood preservative), Aspirin (can cause hyperthermia) Thermogenin (brown fat)These drugs put a hole in the mitochondrial membrane allowing H+ to leak back out and causes hyperthermia.
How many ATP can you get from NADH? What about from FADH2? 3 ATP from NADH, 2ATP from FADH2
What does the chemical MPTP cause? Where does it come from? What other substances have this MOA? MPTP leads to MPP which leads to parkinsons syndrome, it comes from people who try to synthesize meperidine. Rotenone and Amytol both also block complex 1 of the electron transport chain
What things can block complex 4 of the Electron transport chain? Things that can be abbreviated, CO, CN-, H2S, sodium azide (NaN3)
What causes intense muscle cramps and dark urine after exercise? McArdles disease
What are the breakdown products of G6P when it enters the HMP shunt? What are these products good for? G6PD reduces G6P to NADPH and Ribulose 5-Phosphate. NADPH is needed for CHO/FA, 02 free radicals (both RBC protection and use for killing), and cytochrome P450. R5P is used to make PRPP for nucleotide synthesis
What is Ribulose 5-Phosphate? Where does it come from and what does it do? Byproduct of G6PD that creates NADPH and R5P, its used to make PRPP for nucleotide synthesis (DNA production)
What enzyme allows people with chronic granulomatous disease to break down catalase negative bacteria? Myeloperoxidase, the H2O2 produced by these bugs can be utilized to form HOCl (bleach) still even though NADPH Oxidase can’t be used to create O2 free radicals that SOD turns into H2O2.
What are the enzymes of the respiratory burst? O2->O2* via NADPH oxidase, O2*->H2O2 via SOD, H2O2->HOCl (bleach) via myeloperoxidase.
How do RBCs neutralize oxygen free radicals? RBCs utilize Catalase/Glutathione Peroxidase which must be reduced back to GSH from GSSG which requires NADPH. If you have no G6PD, you can’t regenerate NADPH, can’t reduce GSH and can’t neutralize H2O2->oxidative damage to RBCs
What happens in G6PD deficiency? Can’t make NADPH, without this you can’t reduce Glutathione to neutralize oxygen free radicals in RBCs so they undergo lysis when exposed to oxidative stress (Fava beans, sulfonamides, nitro drugs, antimalarial drugs)
What characteristic finding(s) will you see on peripheral blood smear in G6PD deficiency patients and what are they? Heinz bodies = oxidized hemoglobin in RBCs that ppt out and also bite cells from where spleen has taken them out
What occurs in fructose intolerance? What about essential fructosuria? Fructose intolerance is due to Aldose-B deficiency->F1P accumulation which decreases phosphate availability->hypoglycemia, jaundice, cirrhosis and vomiting. EF is fructokinase lack which is benign, just urinary fructose
What occurs in galactokinase deficiency? What about classic galactosemia? Galactokinase deficiency->^galactitol->infantile cataracts. Classic Galactosemia = lack of G1P uridyltransferase->failure to thrive, infantile cataracts, retardation, HSM, jaundice
How does one treat fructose intolerance? How about Classic Galactosemia? FI treat by removing all fructose AND sucrose from diet. CG treat by removing all galactose AND Lactose
What substances cause hemolytic anemia in patients with G6PD deficiency? Dapsone, fava beans, high dose aspirin, nitrofurantoin, primaquine, ibruprofen, naphthalene, isoniazid, Sulfa drugs
What are the four important pharmacokinetic equations? Vd=amount given IV/[plasma], LD = Cs*Vd t1/2 = 0.7*Vd/CL, MD = Cs*Cl
What is the antidote to methanol/ethylene glycol poisoning? Fomepizole (inhibits alcohol dehydrogenase) this prevents the breakdown of these poisons into their toxic metabolites
What findings are present in Kwashiorkor and what is the physiology? Skin depigmentation and lesions, edema (decreased oncotic pressure), fatty liver (decreased apo B-100), Anemia
What is the name for total caloric malnutrition, and what is seen? Marasmus is a lack of all calories and you see muscle wasting, subQ fat wasting, tissue wasting and variable edema
What is refeeding syndrome? The sudden introduction of substrate after 5+ days of starvation causes cells to go wild making proteins and storing minerals-> decreases in serum Phosphate, K+, and Mg2+, this leads to arrhythmias and ATP can also be depleted. Neurologic issues follow
What brain nuclei are affected by leptin? What does this do to them? Lateral nuclei in hypothalamus is inhibited by leptin as it causes hunger. VM nuclei in hypothalamus is stimulated by leptin->satiety
What are the diagnostic criteria categories for metabolic syndrome? Waist circumference, TGs, HDL, BP, fasting serum glucose
What is orlistat? Sibutramine? Both are weight loss drugs, orlistat inhibits pancreatic lipases, sibutramine is serotonin and NE reuptake inhibitor contraindicated in patients with CAD, CV disease, CHF and arrhythmias or on SSRIs/MAOIs
How is NASH treated? avoidance of alcohol, weight loss, controlling any DM, pioglitazone and metformin
What is phototherapy used to treat in the GI tract? How does it work? Jaundice (Crigler Najjar etc) is treated with blue light (not UVB) that converts unconj bilirubin in cutaneous blood vessels into isomer that is renal excreted.
What are the causes of hereditary hyperbilirubinemia? Gilbert’s (benign, shows up with stress), CN type I = no UDP-glucuronyl xferase, death early, Rx = phototherapy and plasmaphoresis, type II = less severe, Rx = phenobarbital (starts-up liver), Dubin-Johnson = ^conj bili, can’t excrete, grossly black liver
What is physiologic neonatal jaundice? How is it treated? Immature hepatocytes don’t produce enough UDP-glucuronyltransferase and unconj bili builds up, use phototherapy to prevent kernicterus
What is the cause of a grossly black looking liver? Dubin-johnson syndrome, can’t excrete conjugated bilirubin from hepatocytes which turns liver bile color (darkish/black)
What is wrong if you see intracytoplasmic eosinophilic inclusions on liver biopsy? Mallory bodies are seen in alcoholic hepatitis from long term hepatic steatosis. AST>ALT
What is the purpose of draining ascities fluid? What precautions should be taken? To prevent discomfort AND to reduce risk of spontaneous bacterial peritonitis (has poor prognosis). Be careful of hypotension though by giving albumin to help retain fluid in vascular space
What is gamma-glutamyl transpeptidase? GGT is a serum marker for liver disease (^ with heavy alcohol consumption)
What drugs would you give to someone with cirrhosis/liver failure? Propanolol/nadolol for varices (octreotide and banding if bleeding), diuretic +/- abx for ascites, vit K for coagulation, lactulose for detoxification (draws ammonia into colon to prevent encephalopathy)
What does elevated Alk phos indicate? What about ceruloplasmin? ^Alk phos means either bone disease (if all other liver labs are normal) or it means biliary obstruction. Low ceruloplasmin = wilsons disease
What causes Nutmeg liver? How could you differentiate the causes? Congestion of the liver, often due to R sided heart failure or budd-chiari syndrome. In RHF you get JVD, in Budd-Chiari you do not
What are the causes of budd-chiari syndrome? Hypercoaguable state, Virchow’s triad, polycythemia vera, pregnancy and hepatocellular carcinoma
What is Reye’s syndrome? Child with influenza or VSV that is given salicylates (aspirin) goes into hepatoencephalopathy, often fatal, reversible inhibition of mitochondrial enzymes reduces B-oxidation which decreases Acetyl-Coa necessary for TCA cycle
If a child has flu like symptoms and is given aspirin to treat his pain but starts vomiting and becomes disoriented what happened? Reye’s syndrome
If you have a person who went on a mission trip to sub-Saharan Africa and returns with RUQ pain and large lesions on their liver when they returned, what is the disease and how do you treat it? Entamoeba Histolytica likely from drinking water which led to liver abscesses, treat with Metronidazole
If a patient from mexico comes to you with HSM and low WBCs, RBCs and platelets and amastagotes are seen in their macrophages, what is the disease, how did they get it and how is it treated? Leishmania Donovani transmitted by the sand fly and treated with sodium stibogluconate
What meds can be used to treat Hep C? Hep B? C can be treated with a-IFN and Ribaviron, Hep B can be immunized but also treated with a-IFN but NOT ribaviron
Which antibodies can be present in autoimmune hepatitis? Anti-smooth muscle, ANA, Anti-Liver/Kidney Microsomal (Negative Anti-mitochondrial and Negative viral Hep Serology)
A patient with b-thalassemia comes in because of DOE and odd skin discoloration. What is the issue? What would lab results show? Rx? This is secondary hemochromatosis, would also see Cirrhosis, Bronze Diabetes and CHF. Labs would show ^Iron, ^Ferritin, ^Transferrin Sat, decreased TIBC, Rx = phlebotomy
If a patient came in with parkonsonian features but exhibited flapping of their arms when in full extension, what would be the cause and what would labs show/treatment? Wilson’s disease, copper accumulate leads to basal ganglia degeneration and asterixis due to liver damage. Decrease in ceruloplasmin is seen and penicillamine, deferoxamine is used to treat
If a 45 year old male comes in with emphysema and cirrhosis despite abstinence from cigarettes/alcohol, what is the issue and what would be seen on biopsy? a1AT deficiency is likely, you’d see PAS positive globules in the liver and panacinar emphysema in the lungs due to ^elastase activity
If a 25 y/o sexually active female comes in with vague RUQ pain and a liver mass is seen on imagining, what is the cause? What should be done? Likely a hepatic adenoma from OCP usage, follow with imagining and AFP to make sure HCCA doesn’t form, remove if >5cm, discontinue OCP
If a 28 y/o male weight lifter comes in with vague RUQ pain and elevated AFP, what is the issue? Likely a hepatic adenoma due to anabolic steroid usage that turned into HCCA and is releasing AFP.
What are the risk factors for hepatic angiosarcoma? Vinyl chloride and arsenic
In patients with Hep B, Hep C, hemochromatosis etc what lab value should you follow to check for HCCA development? Alpha Fetoprotein AFP
IF a patient shows up with tender HSP, an elevated H&H and jaundice, what is the diagnosis? HCCA
What causes yellowing of the palms and soles, orangish skin but not scleral icterus? Carotinemia, too many carrots, seriously
If a 40 y/o male with Ulcerative Colitis comes in with pruritus, dark urine and elevated alk phos/LFTs, what is the diagnosis? What is he at risk for, what will labs show? Primary Sclerosing Cholangitis is likely, will beads on a string Ultrasound, +PANCA likely, onion skinning of bile ducts and he is at risk for cholangioCA, Rx = liver transplant
If a 40 y/o female comes in with dark urine, light clay colored stools and pruritis, what is the diagnosis, what will labs show and what can it be treated with? Primary biliary cirrhosis, +Anti-mitochondrial antibodies w/ ^conj bili, ^CHO, ^Alk phos and Rx = Ursodiol (decrease liver CHO/bile production), liver transplant
What does ursodiol do? Decreases CHO production in the liver which decrease bile flow, used to treat primary biliary cirrhosis and gall stones.
What is charcot’s triad, what does it signify, what is the next level? Fever, RUQ pain and jaundice indicating Cholangitis, can go on to form pentad = those 3 + hypotension and altered mental status
What is positive murphy’s sign? What does it signify? Expiratory arrest with deep palpation of gallbladder due to pain with Cholecystis
What drug is used to treat fluke infections? What are the fluke infections? Praziquantel treats Shistasoma, Paragonamis Westermani and Clonorchis sinensis
If someone eats undercooked fish and develops pigmented gallstones what is the diagnosis and the treatment? Clonorchis Sinesis infects the biliary tree and praziquantel is the treatment
What are the familial dyslipidemias? What is deficient in each and what are the characteristic findings in each? type IIa = hypercholesterolemia due to deficiency LDL receptors = ^LDL->^CHO->accelerate atherosclerosis, tendon xanthomas, and corneal arcus. Type 4 = hyperTG, ^VLDL leads to pancreatitis
If you see acanthocytes in a peripheral blood smear, what is the diagnosis? What is the issue? Abetalipoproteinemia which leads to burr cells/mace looking RBCs. Due to lack of B-48 and B-100 which causes accumulation of lipids in enterocytes since they can’t get out (B-48)
What amino acid derivatives come from phenylalanine? PA->Tyrosine (thyroxine with addition of iodine), ->Dopa (melanin)->domapine->NE->Epi
What is phenylalanine converted directly into? What enzyme accomplishes this and how? Phenylalanine hydroxylase converts PA into tyrosine with the help of tetrahydrobioterin (THB)
How is heme made and what is the rate limiting step? What creates the precursor to heme? Porphyrins form heme with Aminolevulenate synthase. Prophyrins form from glycine with the help of B6
What is the most common urea cycle enzyme deficiency? What are the symptoms and labs Ornathine Transcarbamalyase deficiency which leads to a DECREASE in BUN (can’t make urea) and an increase in serum ammonia (hepatoencephalopathy = slurring, blurring, tremor, somnolence, vomiting, cerebral edema
What are some causes of hyperammonemia? How is it treated? Reye’s syndrome, OTC deficiency, liver damage, urea cycle issues (OTC). Rx = phenylbutyrate (binds AA and excretes), lactulose = colonic bacteria metabolize it and acidifies to trap NH4 for excretion
What is the rate limiting enzyme for the urea cycle? What is similar? Carbamoyl phosphate synthetase 1 is found in mitochondria, gets N from ammonium and does urea cycle. CPS2 does pyrimidine synthesis and is in cytoplasm and gets N from glutamine
What substrate and cofactor are necessary to make GABA? What happens if either are deficient? Glutamate and B6, you get convulsions! (no GABA = no inhibition)
What amino acid issue in mothers can cause microcephaly, growth/mental retardation and congenital heart defects in children? PKU, either lack of phenylalanine hydroxylase or lack of tetrahydrobiopterin
If a patient has brown pigmented sclera and their urine turns black, what is the issue and what is the pathophysiology? Alcaptonuria (ochronosis) is due to lack of homogentisic acid oxidase so they can’t break down tyrosine into fumarate (increased melanin)
What does lack of homogentisic acid oxidase cause? What is the pathophysiology? Alkaptonuria due to inability to break down tyrosine into fumarate
What are three possible causes of albinism? Lack of tyrosinase, lack of tyrosine transporters, or lack of migration of neural crest cells (similar to chediak higashi), all cause inability to make melanin from tyrosine
What are three reasons why you might have homocysteinuria? How do you treat? decreased cystathione synthase, decreased affinity of Cystathione synthase for cofactor pyridoxal phosphate (b6) or deficiency in homocysteine methytransferase (requires b12), Rx = B6,B12,folate and ^cysteine
What two genetic syndromes can cause lens subluxation? Marfans and homocysteineuria
What is S-adenosyl-methionine? Transfers methyl units, needed for phosphocreatine and Epi/NE. Made from ATP and methionine, requires B12 and folate to regenerate
What is the pathophysiology and treatment of cystinuria? Defective COLA transporter in PCT of kidneys, can’t transport Cysteine, Ornithine, Lysine or Arginine into tubule->^cysteine = kidney stones! Treat with acetazolamide (alkalinizes urine to prevent staghorn calculi)
Alpha-ketoacid dehydrogenase deficiency leads to what disease? Maple syrup urine disease, ^levels of isoleucine, leucine and valine (branch chain amino acids)
What is hartnip disease? defective neural amino acid transporter, leads to inability to absorb tryptophan->inability to make niacin = pellagra (vit B3 deficiency)
How would you treat salicylate poisoning with respect to urine pH? Salicylates = weak acid, you want alkaline urine to trap it ionized so you give NaHCO3 (sodium bicarb)
How would you treat amphetamine toxicitiy with respect to urine pH? Amphetamines = weak bases so you want to acidify urine to trap it in ionized state, give NH4Cl, ammonium chloride
What drugs are metabolized in a zero order elimination manner? Aspirin, Ethanol, Phenytoin (PEA)
What drugs induce P450? What drugs inhibit P450? Induce = BCGPQRS (Barbiturates, Chlorpromazine, Greisiofulvan, Phenytoin, Rifampin, Sulfonylureas. Inhibit = PICKEGS (PIs, Isoniazid, Cemetidine, Ketoconazole, Erythromycin, Grapefruit Juice, Sulfonamides
What coagulation test will be prolonged by Hemophilia A? what about B? Are these intrinsic or extrinsic pathways? Both Hemophilia A/B (factors 8 and 9) cause issues with the PTT, NOT the INR. PTT measures intrinsic pathways (not Tissue factor/extrinsic)
What coagulation factors are Vit K dependent? What cranial nerves are part of the pharyngeal arches? 2,7,9,10 AND ProteinC/S and 5,7,9,10
What is the result of a G20210A mutation? hypercoagulability, venous clots
If you give a patient heparin, which should be prolonged, PTT or PT? What happens if neither change? PTT should go up (inhibits factor X which is common but PTT is more accurate), if neither go up, the patient may have anti-thrombin deficiency, can’t accelerate antithrombin if its missing
IF you give a patient warfarin and they break out in skin necrosis and hemorrhage, what predisposed them to this condition? Protein C/S deficiency cause them to become hypercoaguable with the administration of warfarin which blocks C/S before it goes 2,7,9,10 and they already have low levels
What factors are blocked by protein C/S? Factors 5 and 8, S is a cofactor for C
If a patient is on heparin and suddenly develops low platelet count, what is happening? What are they at risk for? How do you treat? Heparin induced TCP (HIT) is due to platelet factor 4 complexes being attacked by antibodies that activate these platelets. They are at risk however for CLOTS, because they are activated (hypercoaguable). Use –rudins instead w/ warfarin
Is it possible to have a low platelet count yet be in a hypercoaguable state? What drug causes this to happen and how? Heparin induced TCP (HIT) is due to platelet factor 4 complexes being attacked by antibodies that activate these platelets and make the ppt out of blood (TCP). They are at risk however for CLOTS
What are the –rudins? When should they be used? These are heparin like drugs that work the same way but don’t cause HIT, so use them when people get HIT along with warfarin
How does warfarin work? It inhibits epoxide reductase which prevents the recycling of Vit K. This prevents gamma-carboxylation of vit K dependant clotting factors (2,7,9,10) AND protein C/S
What is enoxaparin? What is its MOA? LMW heparin, inhibits factor Xa, can’t follow with PTT, must check anti-Xa activity directly. Can be SubQ, longer halflife, weight based dosing, more convenient
When do you see Heinz bodies and bite cells? What causes them? G6PD deficiency leads to oxidative damage, Fe3+ causes Hgb to precipitate out (Heinz bodies) and spleen removes these with bite.
If a patient comes in with acute abdominal pain, psych disturbances and port-wine colored urine after starting a new drug, what is the issue and how would it be treated? Likely acute intermittent porphyria (uroporphyrinogen-synthase), treat with glucose and heme (this prevents ALA synthase from making more toxic intermediates by feedback inhibition)
What is the presentation of porphyria cutanea tarda? What enzyme is deficient? Hypertrichosis, facial hyperpigmentation, Hep C, alcoholism and blistering cutaneous photosensitivity. Often presents with elevated LFTs. Similar to a homeless man, uroporphyrinogen decarboxylase deficient
What enzymes are inhibited by lead poisoning? What environmental exposures cause this? ALA dehydratase and Ferrochelatase. Kids = lead paint. Adult = battery, ammunition and radiator factories
What are the symptoms and treatments for lead poisoning in adults and kids? Symptoms = lead lines on gums/long bones (kids), colicy abdominal pain, encephalopathy, wrist/foot drop, headache, memory loss etc. Rx in kids = succimer, adults = dimercaperol or EDTA
What are the structures of the following hemoglobins: HbA, HbA2, HbF, HbS, HbC, Hb Barts, HB H? a2b2, a2d2,a2g2, a2bs2,a2bc2,b4(alpha thal),g4(hydrops)
What is 2,3-bisphosphoglycerate? What effect does it have in fetus? this is 2,3 BPG which causes decreased affinity for O2 in blood, fetal hemoglobin has poor affinity ofr 2,3BPG and thus has higher affinity for O2 so it can steal it from mom
What is the rate limiting enzyme for de novo pyrimidine synthesis? Carbomyl Phosphate Synthetase II CPSII
What two enzyme deficiencies can lead to orotic aciduria with no elevated ammonia? What happens here? orotic acid phosphoribosyltransderase or orotidine 5’-phosphate decarboxylase deficiencies, ^orotic acid, can’t make UMP so you can’t make DNA and get megaloblastic anemia (does not improve with B12/folate), not OTC since low ammonia
What is the mechanism of action of hydroxyurea? What does it treat? Blocks ribonucleotide reductase (the converting enzyme for UMP->UDP) this treats cancer as well as sickle cell
What enzyme turns dUMP to dTMP? What drug blocks this? What cofactors are necessary? What drug(s) are involved in these cofactors? Thymidylate synthase is inhibited by 5-FU, it requires THF which is regenerated from DHF by the enzyme DHF reductase which is blocked by MTX in humans, TMP in prokaryotes
What drug blocks PRPP synthetase and what does this do? 6-MP blocks this enzyme which is needed for de novo purine base synthesis and salvage
What does allopurinol do? What drugs can this increase the toxicity of? Xanthine oxidase inhibitor, blocks the degradation of azathioprine and 6-MP which are blocking purine synthesis
What enzyme deficiency causes Lesch-Nyhan syndrome? What are the symptoms? Treatment? defective purine salvage enzyme HGPRT (He’s got purine recovery trouble). X-linked, ^uric acid (gout), lip-biting, aggression, choreaform movement. Rx = allopurinol
In what biochemical pathway is the enzyme that is deficient in SCID? Adenasine Deaminase is the deficient enzyme and it is in the purine salvage pathway (no thymic shadow, diarrhea, infections, failure to thrive)
What drugs target topoisomerase in humans and prokaryotes? What about antibodies? Fluoroquinalones target prokaryotes Topo, Atoposide does humans. Anti-topoisomerase Abs is seen in systemic scleroderma (anti-scl70)
What is the primary defect in xeroderma pigmentosa? Failure of nucleotide excision repair genes that allow Thymine-Thymine dimers to go un-repaired which leads to excessive skin cancer
What is the issue in ataxia-telangiectasia? DNA double strands have failure of nonhomologous end joining
What is Bloom syndrome? DNA repair defect that leads to increased risk of leukemia and lymphomas by age 25
What is wrong when a patient has a rash around anus, mouth and nose/eyes? Acrodermatitis Enteropathica, due to Zinc deficiency
What is diepoxybutane (DEB) test used for? Determines if Fanconi’s anemia is present, DNA repair issues that leads to chronic anemia
What are Bernard Soulier and Glanzmann’s thrombasthenia? Platelet malformations, BS has lacking gpIb which prevents platelets from adhering to endothelium (decreased PC seen) and GT is lacking gpIIb/IIIa which prevents platelet-platelet adhesion, no PC seen. Both increase BT
What is TTP? Thrombotic Thrombocytopenic Purpura. Due to ADAMTS13 zinc metaloprotease difficiency, can’t break down vWF multimeres -> platelet activation, decreased PC and thrombosis. Neuro/Renal sx, fever, TCP and anemia
What is ITP? Idiopathic Thrombocytopenic Purpura, antibodies to gpIIb/IIIa cause destruction of platelets, leading to low PC, increased BT and ^megakaryocytes on BM biopsy
What does ticlopidine do? What other drug does this too? Blocks ADP receptor on platelets preventing their activation and expression of gpIIb/IIIa. Clopidagrel also does this
What does abciximab do? What other drugs do this also? Binds to an blocks the gpIIb/IIIa receptors on platelets to prevent platelet adhesions. Other drugs include Tirofiban and Eptifibatide
What cancer is associated with hashimoto’s thyroiditis? Marginal Cell lymphoma.. you see hurthle cells and follicular centers w/ lymphoid aggregates which predisposes to Marginal cell lymphoma
What lymphoma fits the follow descriptions? Most common, Reed-Sternberg, EBV, Celiac disease, CLL, Starry Sky, sjogrens or H.pylori? Diffuse large B cells, Hodgkins, Hodgkins/Burkitts, Intestinal T-cell lymphoma, small lymphocytic lymphoma, burkitts, marginal cell
What are the translocations associated with the following lymphomas: Mantle cell, Follicular, Burkitts? t(11;14), t(14;18), t(8;14)
What is the cause of a red face/plethora, headaches and a palpable spleen? Polycythemia vera
If a patient has kidney stones, elevated creatinine, a low blood count and back pain, what is the diagnosis? Multiple Myeloma, hyper calcemia, renal insufficiency, anemia and back pain/lytic bone lesions
What are the chromosomal translocations for the following: CML, Burketts, Follicular, M3 type of ALL, ewings, Mantle Cell lymphoma? t(9;22), t(8;14), t(14;18), t(15;17), t(11;22), t(11;14)
What is alpha-tocopherol? Vit E
What types of drugs can cause stevens johnsons syndrome? Penecillins, Sulfa drugs, Seizure drugs and Allopurinol
What antibiotic would be the cause of an Achilles tendon rupture? Fluoroquinalones
How do you treat Salmonella gastroenteritis? You don’t give antibiotics! It prolongs the carrier state without really helping much.
What does Shigella Flexneri cause? Reiter syndrome, can’t pee can’t see can’t climb a tree
What GI infection can cause Guillan Barre? Campylobacter Jejuni
What bacteria mimics appendicitis? How is it obtained usually? Yersinia Enterocolitis is often obtained by puppies, pet fecal matter, milk/pork or day care centers
What drugs are often culprits of C. Diff colitis? Clindamycin and Amphicillin
What bug causes food poisoning from shell fish/ocean water? Vibrio Parahemolyticus
What bug can often be obtained from handling turtles? Salmonella
What bug can be found in cans of food that are bulging? C. Botulinum
Created by: amelson