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108 Cards in this Set

  • Front
  • Back
Girl with a short and thick neck, broad chest, and short fourth metacarpals will have what on echocardiogram? What kind of murmur?
Bicuspid aortic valve has an early systolic, high frequency click heard over right second interspace.
What is pulsion and what disease process is it involved in?
Increased intraluminal pressure created during strained bowel movements due to chronic constipation results in the formation of sigmoid colon false diverticula (mucosa and submucosa covered in serosa)
What would mutation of Fas or FasL in T cells result in?
Excessive accumulation of autoreactive T cells and development of autoimmune diseases like SLE due to inability to initiate the extrinsic apoptosis pathway.
Walk me through how Fas receptors initiate the extrinsic pathway of apoptosis?
Upon binding FasL, receptors trimerize and their death domains form a binding site for an adapter protein called Fas associated death domain (FADD). Receptor bound FADD stimulates activation of caspases 8 and 10 which begin the activation cascade culminating in activation of executioner caspases 3 and 6 (cleavage of DNA, nuclear fragmenation, organelle autodigestion, and plasma membrane blebbing follows.
If group B strep colonization is identified by vaginal rectal culture at 35-37 weeks gestation, what is required to prevent neonatal GBS sepsis, pneumonia, and meningitis?
Intrapartum antibiotics, either PCN or ampicillin
Which strep strain is beta hemolytic and bacitracin resistant?
S. agalactiae (GBS)
What is the presentation of debranching enzyme deficiency (Cori disease)? What does this deficiency lead to?
Hypoglycemia, ketoacidosis, hepatomegaly (fibrosis without fat infiltration separated from other GSDs), and muscle weakness with hypotonia due to cytosolic accumulation of glycogen with abnormally short outer chains (limit dextrins).
Lynch syndrome presentation? Defect? Genes involved? What proteins are coded for?
Family history of colon, endometrial and ovarian cancers and adenocarcinomas resulting from the autosomal dominant defect in DNA mismatch repair due to mutations in MSH2 and MLH1 which code for human MutS and MutL homologs.
Describe the step by step process of mismatch repair?
MutS homolog detects a mismatch on new daughter strand (has occasional nicks in the phosphodiester bonds), MutL homolg is recruited, and the resulting complex slides along the DNA molecule until 1 of the daughter strand nicks is encountered. Exonuclease 1 is loaded onto and activated by the repair complex and the daughter strand is degraded bckwards past the initial mismatch point, leaving a variable gap of single stranded DNA that is stabilized by ssDNA binding protein. Complex dissociates while DNA polymerase delta loads at the 3' end of the disontinuity and begins synthesizing a new daughter strand segment. Finally, DNA ligase I seals the remaining nick to complete the repair process.
When a patient is asking for pain medicine by name, what should you do?
clarify medication history by using prescription monitoring programs.
What is annular pancreas and what does it result from?
Pancreatic tissue encircling the descending duondenum results from the failure of the ventral pancreatic bud to properly migrate and fuse with the dorsal bud during the seventh and eighth week of fetal development often resulting in duodenal obstruction or pancreatitis.
What does the ventral pancreatic duct give rise to?
Uncinate process of the pancreatic head and the main pancreatic duct.
What does the dorsal pancreatic bud give rise to?
Pancreatic tail, body, and remainder of the head
Majority of annular pancreas patients have what symptoms?
Asymptomatic
Surgical repair of undescended testicle lodged in the inguinal canal involves reducing the testis through what physiologic opening and in what structure?
Superficial inguinal ring in the aponeurosis of the external oblique
What do you call the failure of one or both testes to descend into the scrotum before birth?
Cyoptorchidism
What is the function of calcineurin? What would an inhibitor of this substance be used for? Examples?
Cyclosporine and tacrolimus inhibit calcineurin activation. It is a protein phosphatase activated upon stimulation of the T cell receptor after which it dephosphorylates nuclear factor of activated t cells (NFAT) to allow it to enter the nucleus and bind to an IL-2 promoter, which can then stimulate the growth and differentiation of T cells.
If left ventricular pressure is higher than aortic pressure during systole, what kind of murmur would you hear and what would echocardiogram reveal?
Aortic stenosis would be heart as a holosytolic crescendo decrescendo murmur most intense during peak pressure of systole.
Statin treatment results in hepatocytes increasing what?
LDL receptor density, and thus uptake of circulating LDL
What cocci does the catalase test differentiate between? Coagulase test? Which one after this is novobiocin sensitive?
Strep (cat negative) from Staph (cat positive). Coag positive (s. aureus) and coag negative (S. epi, haemolyticus, sapro, etc.). Staph epi is the novobiocin sensitive one.
Why is clearance of PAH often used to estimate renal plasma flow?
It is both filtered and secreted by glomerulus and renal tubules
Can PAH filtration be saturated? What happens to tubular fluid PAH concentration as blood PAH concentration rises? Can PAH secretion be saturated?
No, filtration increases linearlyas plasma PAH concentration increases, but secretion can be saturated past a point of plasma PAH concentration
What does maximum secretion rate of PAH correspond with?
Transport maximum (Tmax) of secretion carrier enzymes
Lactose fermenting, indole positive gram negative rod is what organism? What if it was indole negative? What speed do both of these organisms ferment lactose at?
E coli, Enterobacter cloacae, and fast lactose fermenters.
In the case of an enveloped virus, whether or not the virus can attach to a specific host cel generally depends on what?
If the viral envelope glycoprotein binds with high affinity to the host cell glycoprotein. (mutation in this can affect the range of host cells that the virus can infect)
MacConkey agar separates gram negative rods into which two groups and by what feature?
Lactose fermenters (pink) and non fermenters (white)
Which gram negative rod turns white on macconkey agar and is oxidase positive? Does it ferment lactose?
Pseudomonas aeruginosa does not ferment lactose
Which oxidase negative lactose nonfermenter produces H2S on TSI agar? What color is it? What kind of bacteria is it?
Gram negative rod that is black due to H2S production can be Salmonella or Proteus
Which drug can reverse the toxicity of methotrexate in non cancerous cells of the GI mucosa and bonemarrow? How does it do it? Why would this same drug be used in colorectal cancer chemotherapy?
Folinic acid (leucovorin) rescues these cells as a form of folic acid that does not require DHFR activation (MTX blocks DHFR to prevent purine synthesis). When combined with 5-FU it potentiates cytotoxic effects by binding to thymidylate synthetase.
Allopurinol MOA and clinical use?
Noncompetitive inhibitor of xanthine oxidase used to treat gout and prevent tumor lysis syndrome
amifostine MOA and clinical use?
Cytoprotective free radical scavenger used to decrease nephrotoxicity associated with platinum containing and alkylating chemotherapeutic agents and to decrease dry mouth
MOA of dexrazoxane and its clinical use?
Iron chelating agent prevents anthracycline induced cardiotoxicity
Filgrastim MOA and clinical use?
Granulocyte colony stimulating factor analog used to stimulate proliferation and differentation of granulocytes in patients with neutropenia
Mesna MOA and clinical use?
Binds acrolein, the toxic metabolite formed by cyclophosphamide and ifosfamide, to prevent hemorrhagic cytitis.
Fever and sore throat in a patient with hyperthyroidism like Graves disease raises concern for what condition? Best next step and why?
Thionamide induced agranulocytosis can result from treatment with methimazole or PTU by inhibiting thyroid peroxidase to prevent iodine organification. They can cause agranulocytosis presenting in this way, so that's why you should get a white blood cell count with differential in this situation.
Describe the transport process mechanism for glucose entry into adipocytes?
Carrier mediated transport is facilitated with transmembrane proteins (facilitated diffusion without energy expenditure). Specifically, GLUT4 is stereoselective and preferentially catalyzes entrance of D-glucose instead of L-glucose in an insulin sensitive way (stored in cytoplasmic vesicles and incorporated into cell membrane under influence of insulin).
What is the function of GLUT2?
Facilitates export of glucose from the liver, small intestine, and kidneys into circulation and helps control insulin secretion in the pancreas.
Man with fatigue, rash, flushing, abdominal cramps is dizzy and lightheaded with syncope and a maculopapular rash showing large clusters of mast cells positive for KIT (CD117)- dx? How does the etiology of this condition explain the findings?
Systemic mastocytosis is a cloncal proliferation of mast cells in bone marrow, skin, and other organs due to a mutation in the KIT receptor tyrosine kinase yielding excessive histamine release resulting in syncope, flushing, hypotension, urticaria, etc. Also, the histamine will induce gastric acid secretion leading to gastric ulceration, inactivating pancreatic enzymes and causing diarrhea, NV, and cramps.
Teal colored, oderless, watery stools for 6 months with a total lack of gastric acid secretion in the stool promptly treated with administration of somatostain- dx?
VIPoma resulting in watery diarrhea, hypkalemia, and achlorhydria (WDHA) syndrome also known as pancreatic cholrea (pancreatic islet cell tumor that stimulates pancreatic bicarb and chloride secretion, binding to instestinal epithelial cells and leading to adenylate cyclase activation and increased cAMP production).
Which bone in the hand would be at risk for avascular necrosis in a fall onto an outstretched, extended hand?
Scaphoid bone has a tenuous blood supply
Congestive heart failure patient has an upset stomach, disturbed color perception, anorexia, NV, and diarrhea that has worsened- dx? What is the most serious complication of this condition?
Digoxin toxicity can lead to life threatening ventricular arrhythmia
Which lipoprotein drug would cause an increase in triglyceride levels? Why?
Bile acid resins like cholestyramine, colestipol, or colesevelam bind acids in the GI tract and inhibit enterohepatic circulation leading to diversion of hepatic cholesterol to synthesis of new bile acids, increasing uptake od LDL from the circulaiton, and thus reducing blood LDL levels. The main side effect of this drug is the increased hepatic production of triglycerides and increased release of triglyceride heavy VLDL particles into the circulation, leading to hypertriglyceridemia.
What would cancer of the parotid gland result in if untreated? Why?
Facial droop due to impingement of the extracranial portion of CN 7 which exits the skull through the stylomastoid foramen and courses within the parotid gland where it divides into its 5 branches.
MOA and clinical use of rasburicase? Another drug with this clincial use and its MOA?
Recombinant urate oxidase enzyme that catalyzes the conversion of uric acid to allontoin to make it more soluble and thus can be used to prevent and treat hyperuricemia and the renal manifestations of tumor lysis syndrome. (allopurinol inhibits uric acid formation during cell lysis)
Which type of lung cancer occurs in women and nonsmokers? Is it centrally or peripherally located?
Adenocarcinoma is the most common lung cancer in the general population as well as in these populations. It is located peripherally.
What does the Bcl-2 gene normally inhibit?
Apoptosis
Brain mass in a young boy has cystic spaces filled with thick, brownish yellow fluid that is rich in cholesterol. Diagnosis? What cell type does this originate from? Location?
Craniopharyngiomas are suprasellar tumors derived from Rathke's pouch which makes the anterior pituitary (adenohypophysis).
Profound cerebreal hypoperfusion leads to cerebral ischemia in what areas? How would this ischemia present and what is it called?
Watershed infarcts occur in ischemic hypoxic encephalopathy and present as bilateral wedge shaped strips of necrosis over the cerebral convexity, parallel and adjacent to the longitudinal cerebral fissure.
Female is treated with high dose folic acid for anemia which increases her Hgb level modestly, but she begins to have bilateral foot numbness and difficulty walking. What is going on?
She was likely treated for a megaloblastic anemia and thus was likely deficient in B12. Unfortunately, this has caused irreversible demyelination.
What kind of immunity underlies giant cell arteritis? Which cytokines drive this process, levels of which are correlated with severity of symptoms?
Cell mediated immunity driven by CD4 cells and macrophages involved IL-6, and thus a monoclonal antibody against this cytokine (tocilizumab) is an effective treatment.
Reduction in expiratory flow rate with increased total and residual long volumes are findings suggestive of which lung disease? What pathological findings are associated with this disease?
COPD has destruction of interalveolar walls resulting in airflow limitation
How do pregnancy and oral contraceptive use predispose to gallstone formation?
Estrogen increases cholesterol synthesis upregulating HMG-CoA reductase (bile becomes supersaturated with cholesterol), and progesterone reduces bil acid secretion and thus slows gallbladder emptying.
Describe the steps in inflammatory leukocyte accumulation and the key components involved with each?
Margination from increased vascular leakage improves contact of neutrophils with endothelial lining, Rolling from neutrophil Sialyl Lewis X binding to L selectins on neutrophils or E/P selectin on endothelial cells (expression of which is stimulated by cytokines), Activation of integrins by chemokines secreted by inflamed tissue, Tight adhesion and crawling of neutrophils as they firmly attach to the endothelium via binding of CD18 beta 2 integrins (Mac-1 and LFA-1) to intercellular adhesion molecule 1 (ICAM-1) on endothelial cells, and then finally, Transmigration out of the vasculature via integrin attachment to PECAM-1.
Man has a liver transplant as normal but presents a week later with NV, abdominal pain, diarrhea, a maculopapular rash over his neck, backand extremities and ulcerations of the intestinal mucosa. What is going on?
Graft versus host disease is due to the action of graft T cell sensitization against host MHC antigens which leads to these multisystem findings.
What happens in Guillain Barre syndrome?
Infectious agents with a ganglioside like substance in their lipopolysaccharide layer have antibodies form against them which cross react with ganglioside components of myelin which leads to segmental demyelination and endoneural inflammatory infiltrates of peripheral nerves. Ascending muscle weakness after the respiratory or GI infection (C. jejuni) is accompanied by deep tendon reflex loss and eventually paralysis ascending to cranial nerves and respiratory muscles.
Boy with hyperphenylalenemia is treated with a restricted diet and tyrosine supplementation but months later he comes back with normal serum phenylalanine and neuro abnormalities like hypotonia and microcephaly. He is noted to have elevated prolactin at this time, so what is the most likely diagnosis?
Likely, there is a deficiency of dihydrobiopterin reductase which is apparent due to the inability to synthesize dopamine which would otherwise inhibit prolactin release.
Describe the steps in converting phenylalanine to epinepherine and each enzyme and cofactor needed?
Phenylalanine to tyrosine via BH4 and phenylalanine hydroxylase. Tyrosine to DOPA via BH4 and tyrosine hydroxylase. DOPA to dopamine via dopa decarboxylase and then dopamine beta hydroxylase converts this to NE. PNMT and cofactor of SAM converts NE to Epinepherine.
Which drug in the standard TB therapy causes red orange discoloration of body fluids? What ist heir mechanism?
Rifamycins inhibit DNA dependent RNA polymerase to prevent transcription and lead to a deficiency of proteins necessary for the mycobacteria to survive.
What grade of tumor is a brain tumor forming giant cells?
high grade anaplastic
What synthesizes nitric oxide and from what amino acid? What does nitric oxide activate?
Endothelial nitric oxide synthase (eNOS) synthesizes nitric oxide from arginine. NO2 diffuses through smooth muscle where it activates guanylyl cyclase to increase formation of cyclic GMP.
MOA of canagliflozin? Side effects? Who should avoid taking these and what tests should be done before starting treatment with it?
Sodium glucose cotransporter 2 inhibitors (SGLT2) inhibitors cause urinary glucose loss which can cause UTIs and symptomatic hypotension and thus should be avoided in patients with renal impairment due to lack of efficacy (check renal function tests before starting).
What does the middle meningeal artery branch from? How does it enter the skull? What would its rupture result in?
Maxillary artery which enters the skull at the foramen spinosum and courses deep to the pterion, fracture of which results in epidural hematoma.
Definition and equations for filtration fraction? Normal value for this?
Fraction of plasma flowing through the glomeruli that is filtered across the glomerular capillaries into Bowman's space. FF=GFR/RPF or Creatinine clearance/PAH clearance. Usually equal to 20% in normal patients.
What is in the hepatoduodenal ligment?
Portal triad= hepatic artery, portal vein, and common bile duct.
If liver bleeding does not cease when portal triad is occluded, whhere is bleeding likely from?
Injury to IVC or hepatic veins.
Chest tube for drainage of a pleural effusion is placed at the midaxillary line at the 4/5th intercostal space and through which muscles?
Serratus anterior and intercostal muscles
If a dude has ear pain with trouble hearing, what cranial nerve is injured? What muscle is involved?
Stapedius muscle is innervated by the stapedius nerve which is a branch of the facial nerve (paralysis of this muscle leads to increased sensitivity to sound)
Bleeding history that is treated effectively with desmopressin- dx? How does this drug work to stop the bleeding?
Hemophilia A is a factor 8 deficiency and DDAVP increases circulating factor 8 levels (and also stimulates vWF secretion from endothelial cells).
MOA of methimazole and PTU?
Inhibition of thyroid peroxidase prevents coupling of iodotyrosines and iodine organification. PTU also decreases peripheral conversion of T4 to T3 but methimazole does not.
Describe the production of thyroid hormone?
Thyroid follicular cells take up idodide using a sodium iodide transporter against its concentration gradient. Iodide is released into the thyroid follicular lumen where it is oxidized to iodine radical by TPO, then iodine binds to tyrosine resides (organification) in thyroglobulin to form MIT and DIT. Couple of MIT and DIT produces T3 and T4.
First line treatment of toxoplasmosis gondi in an HIV patient?
Combo pyrimethamine and sulfadiazine (or clinda is sulfa allergy) along with leucovorin (folinic acid)
Primary B cell lymphoma in the CNS is caused by what in an HIV patient?
EBV
Late onset familial Alzheimers is associated with what genetic feature?
Apolipoprotein E4 allele
Early onset familil Alzheimers is associated with which 3 gene mutations?
APP on chromosome 21, presenilin 1 and 2
Incomplete fusion of urethral folds in a male results in what?
Hypospadias
What event causes epispadias?
Faulty positioning of the genital tubercle in 5th week of gestation
What is at the 3' end of an RNA that forms its secondary structures from base pairing within the chain? What kind of RNA is this?
tRNA has a CCA at the 3' end
Which portion of tRNA binds to the ribosome? Which portion facilitates correct tRNA recognition by aminoacyl tRNA synthetase?
T loop and D loop, respectively
What do inflammatory macrophages release in the intima to reduce plaque stability? What do these substances do?
Metalloproteinases which degrade collagen
How to treat acute delirium in an older woman with a UTI?
Low dose antipsychotic like haloperidol
If a patient likely has infective endocarditis caused by S. gallolyticus, what else should you look for in this patient? Or in any case of blood culture showing S. gallolyticus?
Colon cancer (GI lesions)
If a patient recently started taking diphenhydramine and now has blurred vision, what is going on?
First gen antihistamines like chlorpheniramine and diphenhydramine have antimuscarinic, anti alpha adrenergic, and anti serotonergic properties. The blurred vision is likely from the anticholinergic effects on ocular ciliary muscles, impairing accomodation and causing blurred vision for close objects.
Common side effect of amphotericin B? What would this result in?
Nephrotoxicity (renal tubular dysfunction) would cause hypokalemia and hypomagenesemia which could result in arrhythmias and weakness.
Patient with heart failure is on a drug that inhibits neutral endopeptidases and prlongs the action of an endogenous polypeptide hormone that increases urinary output and decreases TPR. What hormone is this and what endopeptidase is this? What are the other actions of this hormone?
A drug that inhibits neprilysin prevents the breakdown of ANP. ANP also dilates afferent arterioles, limits sodium reabsorption, inhibits renin secretion and aldosterone secretion, and finally, relaxes vascular smooth muscle causing vasodilation.
3 year old boy with oral bleeding after a tooth extraction had a maternal uncle with this same recurrent bleeding into joints, so what is likely abnormal in this patient?
Hemophilia is an X linked condition resulting in prolonged PTT
Where in erythrocytes does heme synthesis occur?
Partly in mitochondria and partly in cytoplasm. Mitchondria is obviously lost in mature RBCs.
What happens in vitiligo?
Partial or complete loss of epidermal melanocytes
An immigrant with chronic heart disease- dx? What happens to the mitral valve in this case?
Rheumatic heart disease causes valvular inflammation and scarring
What is the initiating step in the pathogenesis of infective endocarditis?
Deposition of fibrin and platelets on the soon to be infected heart valve (as in rheumatic heart disease)
Normal intestinal mucosal architecture but enterocytes have clear or foamy cytoplasm at the tips of the vili resutling in bulky and greasy stools- dx? What mutation causes this?
Abetalipoproteinemia (impaired formation of ApoB containing lipoproteins) results from an autosomal recessive loss of function in the MTP gene
Hemorrhagic destruction of both adrenals after septic shock- organism? What does this organism usually cause?
N. meningitidis usually causes meningitis.
Which drugs lead to drug induced lupus and what do they have in common? What is common to people who get these reactions?
Procainamide, hydralazine, and isoniazid are all metabolized by N-acetylation in the liver and individuals who are slow acetylators are at greatest risk for developing this.
What is health promotion?
Process of enabling people to increase control over their health and its determinants, and thereby improve their health
What could hCG mimic to induce ovulation in a female trying to conceive after administration of menotropin (human menopausal gonadotropin)?
Menotropin acts like FSH to trigger formation of the ovarian follicle and then ovulation is induced by hCG which mimics LH surge
The HTN, tachycardia, sweating, heat intolerance, weight loss, and hyperreflexia in Graves' disease is mediated by what receptor? What is give to control these manifestations while determining definitive treatment? Which feature of Graves would not be fixed with this treatment, and how does it arise?
Beta adrenergic receptors mediate these changes to administration of a beta blocker like propranolol could treat these symptoms. It would not help the exophthalmos which is caused by lymphocytic infiltration, extraocular myositis, fibroblast proliferation, and overproduction of mucopolysaccharides in response to anti thyroid antibodies.
Which prokaryotic enzyme has 5' to 3' exonuclease activity that function to remove the RNA primer created by RNA primase and repair damaged DNA?
DNA polymerase I
What enzyme does isoniazid inhibit and what cofactor does it normally produce, the active form of which is needed in the rate limiting step of heme synthesis (inhibition of this step leads to sideroblastic anemia)? What is this rate limiting step enzyme also?
Isoniazid inhibits pyridoxine phosphokinase leading to pyridoxine B6 deficiency, the active for of which is the cofactor for delta aminolevulinate synthase which is the rate limiting enzyme of heme synthesis.
Persistent patter of unstable relationships, mood lability, and impulsivity with suicidal ideation or behavior in the context of interpersonal crisis when they feel rejected or abandoned?
Borderline personality disorder
Predominant type of lymphocyte found in a condition with bilateral hilar adenopathy, elevated serum calcium, and elevated ACE levels? What condition is this and what is formed in this condition by these lymphocytes?
CD4 cells are seen in sarcoid granulomas
What condition has 46, XX female with normal ovaries and secondary sex characteristics but no upper vagina and variable uterine development?
Mullerian aplasia (Mayer Rokitansky Kuster Hauser syndrome)
Sunlight exposure catalyzes what conversion in the vitamin D pathway? How do you get active form of vitamin D?
7-dehydrocholesterol to cholecalciferol (D3). Subsequent 25-hydroxylation in the liver and 1-hydroxylation in the kidneys produces the active 1,25 form.
Best treatment choice for a pregnant woman with a DVT?
LMWH like enoxaparin
Pressure of what vein is elevated in liver cirrhosis?
Portal vein
How do you differentiate between the two causes of mononucleosis?
Heterophile antibody negative mononucleosis like syndrome means that you do the monospot test and put serum on horse or sheep tissue and check for heterophile antibodies but don't have any but you still have mono like symptoms so its probably CMV rather than EBV.
Reduced numbers of inhibitory ganglion cells in the esophageal wall is what condition if you also have decreased amplitude of peristalsis in the mid esophagus and increased tone and incomplete relaxation at the lower esophageal sphincter?
Achalasia
How to calculate probability of being a carrier if disease frequency is given?
q squared= mutant allele frequency and then 2q should give the probability of being a carrier as in hardy weinberg (p squared(frequency of normal individuals) + 2qp (carrier frequency) + q squared (frequency of diseased individuals))=1
What takes more organisms to infect, shigella or salmonella?
Salmonella needs a lot more, you only need like 10 for shigella
Cholesterol, bile acid, and phosphatidylcholine levels in a patient with gall stones?
Increased, decreased, and decreased.