Week of March 30 Flashcards
What causes the urinary incontience in normal pressure hydrocephalus
Triad: ataxic gait and urinary incontinence, then dementia
Expanded ventricles place traction on cortical efferent and afferent fibers (corona radiata) which disrupts the paraventricular pathways that transmit impulses from cortex to sacral micturition center. Later, lack of inhibition from cerebral cortex leads to frequent and uncontrolled micturtion (i.e. urge incontinence); no sensation of bladder fullness/control over bladder fxn. Voluntary relaxation of urethral sphincter remains intact. 17
How is the micturition reflex (urination) regulated (review of 17)
- Sacral micturtion center: responsible for bladder contraction; parasympathetic fibers travel from S2-S4 ventral whit ematter within pelvic nerves and stimulate cholinergic receptors in bladder wall
- Pontine micturition center: relaxation of external urethral sphincter with bladder contraction during voiding
- Cerebral cortex: inhibits sacral micturition center.
When do you use chi-square test
To test association between 2 categorical variables. In the case of an exposure status and a binomial outcome, patients are divided into 2 groups based on exposure and the number of patients that experience each outcome is recorded in a 2X2 table. 1184
Atypical lymphocytes observed in the peripheral smears of patients with infectious mononucleosis represent what?
Activated CD8+ cytotoxic T-lymphocytes (appear much larger than lymphocytes with abundant cytoplasm, an eccentrically-placed nucleus, and a cell membrane that apperas to conform to the borders of neighboring cells) which function to destroy virally-infected B-lymphocytes. 7643
NOTE: IM characterized by sore throat, malaise, LAD, myalgias, splenomegaly, and fever. After infecting pharyngeal mucosa and tonsillar crypts, EBV gains access to bloodstream where it preferentially infects B-lymphocytes by binding to CD21 cell surface receptor.
If migration of neural crest cells to the intestine is interrupted during the final week, which organ is most likely to be affected?
The arrest of migration of NCC causes Hirschsprung disease, in which a segment of colon is deprived of ganglion cells. NCC give rise to ganglion cells of the submucosal (Meissner) and myenteric (auerbach) plexi of the bowel wall. Since NCC migrate caudally, the RECTUM is always involved in Hirschsprung disease. The abscence of ganglion cells in colonic wall causes affected segment to be narrowed b/c it cannot relax. 330
Fracture of the 12th rib on the left side will most likely damage which structure and why?
The distal tip of the left 12th rib can be displaced into the retroperitoneum when fractured because both the 11th and 12th ribs are “floating ribs” that re not bound to anterior rib cage by cartilage. The 12th rib overlies the parietal pleura medially and kidney laterally. 1700
What caues shingles and how does it appear on LM
Herpes zoster (shingles) develops d/t reactivation of VZV (enveloped dsDNA virus) in the dorsal root ganglia (sensory neurons). On LM intranuclear inclusiosn in keratinocytes and multinucleated giant cells (positive Tzanck smear). Skin biopsy would show acantholysis (loss of intercellular connections) of keratinocytes and intraepidermal vesicles. 1042
What are the aortic and pharyngeal arch derivatives
During embryonic development, the head, neck, and upper thorax region develop from a set of arches known as pharyngeal arches. Each arch is associated with a CN and aortic arch.
- First: part of mxillary artery; CN V
- Second: stapedial artery (regresses); CN VII. Gives rise to the muscles of facial expression.
- Third: common carotid artery, proximal internal carotid; CN IX. Gives rise to parts of the hyoid bone as well as stylopharyngeal muscle.
- Fourth: Left (aortic arch), Right (right subclavian artery); superior laryngeal branch of vagus (CN X). Gives rise to the muscles of the pharynx and soft palate.
- Sixth: pulmonary arteries, ductus arteriosus; recurrent laryngeal branch of vagus (CN X). Gives rise to most of the muscles of the larynx.
PDA aortic arch derivative and symptoms
Ductus arteriosus is a derivative of the **sixth aortic arch. **Vessel usually closes after birth d/t decreased PGE2 levels and increased O2 consumption (can close with Indomethacin = PGE2 inhibitor). Patency results in L-R shunt that can cause LV volume overload and HF Sx (failure to thrive, resp distress). Continuous “machinery-like” murmur and palpable thrill over left intraclavicular region d/t turbulent blood flow thru PDA. 1751
Homeobox-containing genes code for which protein types?
Code for DNA-binding transcription factors (i.e. transcription regulators) which aler the expression of genes involved in morphogenesis.
A significant increase in blood oxygen saturation between the 2 right-sided vessels or chambers indicates what?
Left-to-right shunt. If such an oxygen step-up occurs b/w the RA and RV, a VSD is most likely which produces a holosystolic murmur that is loudest over the left mid-sternal border. 187
Triad of hemolytic anemia, hypercoaguability (e.g. hepatic vein thrombosis and Budd-Chiari), and pancytopenia suggest what?
Paroxysmal nocturnal hemoglobinuria (PNH) which results form an acquired mutation in the PIGA gene that causes abscence of the glycosylphosphatidylinositol (GPI) anchor and associated deficiency of CD55/59 complement inhibitor proteins –> complement-mediated hemolysis. 897
What are the features of vitamin A overuse
Acute: n/v, vertigo, blurred vision
Chronic: alopecia, dry skin, hyperlipidemia, hepatoxicity, HSM, visual difficulities. Papilledema when present is suggestive of cerebral edema in setting of benign intracranial HTN (pseudotumor cerebri)
Teratogenic: microcephaly, cardiac anomalies, fetal death (esp. in first trimester). 1048
What is one of the renal complications of tumor lysis syndrome?
Occurs when tumors with high cell turnover are treated with chemo. Lysis of tumor cells causes intracellular ions, such as K+ and phosphorus, and uric acid (metabolite of tumor nucleic acid) to be released into serum. Uric acid is soluble at physiologic pH but it can precipitate in normally acidic environment of distal tubules and collecting ducts. Prevention includes urine alkalinization and hydration, as high urine flow and high pH along nephron prevents crystallization and uric acid precipitation; allopurinol can alo reduce uric acid production. 1050
How can you diagnose a Strongyloides stercoralis infection?
Becgins following skin penetration by filariform (infectious) larva and can be dignosed by finding rhabditiform (noninfectious) larvae in the stool. Rhabditiform larvae can mature into filariform larva in the human GI tract, precipitating an autoinfeciton cycle –> hyperinfection syndrome characterized by massive dissemination of organism with multiorgan dysfunction and spetic shock. Pruritic, erythematous, linear streaks (larva currens) may occur on thighs and buttocks as teh larva migrate subcutaneously alway from the perianal region. Treatment: Ivermectin. 8873
What fungus can manifest with nodules that spread along lymphatics and commonly infects immunocompotent individuals?
Sporothrix schenckii: dimorphic fungus that cuases subcutaneous mycosis; transmitted by thorn prick.
Inital lesion is a reddish nodule that later ulcerates. Biopsy would reveal a granuloma. From the site of inoculation, the fungus spreads along the lymphatics forming subcutaneous nodules and ulcers. 270
Is unfractionated heparin or LMWH (enoxaparin) better at inactivating thrombin
Both unfractionated heparin and LMWH can bind to antithrombin to increase its activity against Factor Xa. Only unfractionated heparin is able to bind to antithrombin and thrombin to allow antithrombin to inactivate thrombin. 2132
What is the treatment and contraindication of treatment for an ST-segment elevation in inferior leads.
Diagnostic of inferior MI which are often d/t blockage of the RCA, the artery responsible for SA and AV node perfusion. Thus, inferior MIs are often associated with bradycardia.
Atropine blocks vagal influence on the SA and AV nodes and is effective in increasing HR in such patients. A common SE is increased intraocular pressure. In the eye, atropine causes mydriasis, resulting in narrowing of the anterior chamber angle and diminished outflow of aqeuous humor –> angle-closure glaucoma, with sx of unilateral severe eye pain and visual distrubances (e.g. halos). 1322
Segmental transmural inflammation with fibrinoid necrosis is most characteristic of what?
PAN which presents wtih malaise, weight loss, abdominal pain, and melena. Associated with hepatitis B infection.
What is the most common vasculitis from antibiotic use?
Microscopic polyangiitis (leukocytoclastic or hypersensitivity angiitis) which affects small vessels only. A type III immune reaction is the pathogenesis, and the antigen is often a medication (commonly penicillin)
Small cell carcinomas stain for which types of markers?
Tumor cells express neuroendocrine markers (e.g. neuron-specific enolase, chromogranin, synaptophysin, neurofilaments) and contain neurosecretory granules in the cytoplasm.
How do males with classic, non-salt-wasting 21-hydroxylase deficeincy present?
Present at age 2-4 years with early virilization, increased linear growth, and elevated levels of 17-hydroxyprogesterone and androgens. Females with classic 21-hydroxylase deficeincy (with or without salt-wasting) present with ambiguous genitalia at birth.
Treatment of congential adrenal hyperplasia involves low doses of exogenous corticosteroids to suppress excessive ACTH secretion and reduce stimulation of the adrenal cortex. 2081
How does acute hemorrhage into the pituitary gland (pituitary apoplexy) present?
Usually associated with a pre-existing pituitary adenoma. It presents acutely with severe HA, CN involvement (e.g. bitemporal hemianopsia, opthalmoplegia), and signs of meningeal irritation. Typically, chronic sx related to the pituitary tumor (e.g. HA, decreased libido) are present for months before the actual hemorrhage event.** CV collapse** can occur d/t ACTH deficeincy and subsequent adrenocortical insufficeincy. Tx: glucocorticoids (to prevent life-threatening hypotension). 225
What drugs are used in the treatment of hirsutism
Hirsutism occurs d/t increased testosterone secretion or increased conversion of testosterone to DHT via 5-alpha-reductase. Sironolactone has anti-androgenic properties and is used for treatment of hirsutism. Other drugs include antiandrogens flutamide (inhibits binding to testosterone receptors) and finasteride (5-alpha-reductase inhibitor). 656
Presence of capillary hemangioblastomas in teh retina and/or cerebellum, as well as congenital cysts and/or neoplasms in kidney, liver and pancreas
VHL disease. Also at increased risk of RCC, which cna be bilateral
Describe Sturge Weber Syndrome Presentation
Cutaneous facial angiomas as well as leptomeningeal angiomas. Skin involvement overlies the opthalmic (V1) and maxillary (V2) distributions of the trigeminal nerve. Associated with mental retardation, seizures, hemiplegia, and skull radiopacities. Skull radiographs may show characteristic “tram-track” calcifications.
Tuberous Sclerosis Presentation
AD. Kidney, liver, and pancreatic cysts. CNS involvment includes cortical and subepedymal hamartomas. Cutaneous angiofibromas (Adenoma sebaceum), visceral cysts, and a vairety of other hamartomas, as well as renal angiomyolipomas and cardiac rhabdomyomas. Seizures are a major complication.
What can be used to prevent the AE of cisplatin
Cisplatin: forms ROS that can form DNA crosslinks. SE is nephrotoxicity –> prevent with amifostine and establishment of a chloride diuresis (via IV saline) to keep cisplatin in a nonreactive state.
NOTE: Leucovorin (folinic acid) used in treament of MTX overdose. Dexrazoxane is an iron-chelating agent that can help prevent anthracycline-induced (i.e. doxorubicin) cardiotoxicity.
The avid uptake of bromodeoxyuridine (thymidine analog) by a tumor reflects what
Thymidine is a nucleotide used for DNA synthesis; therefore nucleotide uptake occurs in the S phase of the cell cycle. Reflects the high number of tumor cells in the S phase (preparing to divide) that are present in a tumor. A high proliferation rate suggests a high tumor grade and, therefore, a poor prognosis. 616
Describe hormone secretion in lung cancers
SCC: PTHrP and hypercalcemia
Small Cell: Cushing Syndrome and SIADH
What cells participate in CNS repair after ischemic infarction
During first week after an acute stroke, CT will show ischemic area to be hypodense. Microscopically, neurons display signs of irreversible damage during first 48h (“red neurons”). Neutrophils initially move into the area, followed by microglia 3-5d after onset of ischemia.
As the neurons disintegrate, there is extensive phagocytosis of myelin degradation products by microglia, causing an abundance of lipids to be seen in the cytoplasm. 20
What are the most common causes of osteomyelitis in patients with Sickle Cell
Patients have functional asplenia as a result of multiple infarctions of the spleen –> prone to infection by encapsulated organisms. Both Staph aureus and Salmonella (high fever, exquisite thigh pain, bacteremia d/t nonlactose-fermenting, oxidase-negative organism) are common causes of osteomyelitis. 1137
Contact dermatitis, granulomatous inflammation, the tuberculin skin test and the Candida extract skin reaction are all examples of what?
Delayed-type HSR, where antigen is taken up by dendritic cells and presented to CD4+ T-lymphocytes on MHC class II. These stimulates TH-lymphocytes (usually of the TH1 lineage) then release release IFN-y to cause recruitment and stimulation of macrophages. Take days to reach their peak activity. 544
Hallucinations of recently deceased relatives in children is…
part of a normal grief reaction and may not be indicative of major psychiatric illness. 10465
How is essential tremor treated?
Slowly progressive symmetric postural and/or kinetic tremor that most commonly affects upper extremities. First-line treatment is the nonspecific beta-adrenergic antagonist propranolol. 1946
The most important steps for prevention of cenral venous catheter infections are what?
Proper hand washing, full barrier precautions during insertion of central line, chlorhexidine for skin disinfection, avoidance of femoral insertion site, removal of cath when no longer needed. 8282
Describe concentrations of substances as fluid runs along the proximal tubule.
The concentrations of PAH, creatinine, inulin, and urea increase as fluid runs along the proximal tubule, while the concentrations of bicarbonate (actively reabsorbed d/t activity of carbonic anhydrase), glucose, and AAs decrease. 1617
A liver mass associated with increased AFP is a typical presentation associated with what
HCC which is strongly associated with HBV infection and often preceded by HBV-induced cirrhosis. Integration of viral DNA into the genome of host hepatocytes triggers neoplasic changes. Other risk factors for HCC include HCV, alcoholic cirrhosis, aflatoxins, and hemochromatosis. 822
Describe drug-induced liver injury
Inhaled anesthetics (e.g. halothane) can be associated with a highly lethal fulminant hepatitis that cannot be distinguished from acute viral hepatitis. In severe cases, extensive HCC damage causes liver to rapidly atrophy and appear **shrunken; **widspread centrilobular necrosis and inflammation of protal tracts. Patients have significally elevated aminotransferase levels d/t massive hpatocellular injury and prolonged prothrombin time (factor VII has shortest half-life) d/t failure of hepatic synthetic function. 369
What does PCR require
PCR requires primers that are complementary to the regions of DNA flanking the segment to be amplified (note that while PCR does not require that the sequence of target DNA be known, the flanking sequences must be known in order to make the primers). 2043
Thermostable DNA polymerase, deoxynucleotide triphosphates, and the target DNA template strand are also necessary.
What is the presentation of Henoch-Schonlein purpura
Generally affects young children and is classically preceeded by an URI. This IgA-mediated hypersensitivity (leukocytoclastic) vasculitis commonly causes abdominal pain, joint pain, lower extremity palpable purpra and hematuria.
What will not be affected by pancreatic insufficiency
Pancreatic amylases, along with salivary and brush border amylases, are required for degradation of polysacchraides into monosaccharides, as polysaccharides cannot be absorbed by the intestinal mucosa. D-xylose is a monosaccharide; thus, its absorption does not require pancreatic enzymes and is not affected by pancreatic insufficinecy. Oral administration of a fixed dose of D-xylose can be used to differentiate between malabosprtion of pancreatic vs. GI mucosal etiology. 1907
Explain the Southwestern blot
Used to detect DNA-binding proteins such as transcription factors, nucleases, and histones. The target protein binds to a labeled, dsDNA probe that is homolgous to the protein’s regulatory sequence. c-Jun and c-Fos are nuclear transcription factors that directly bind DNA via a leucine zipper motif; the genes that code for these are protooncogenes. 2044
What are interviewing strategies for dealing with patients experiencing intimate parter violence
- Ensure privacy (ask others to leave for interview)
- Nonjudgmental, empathic, open-ended questioning
- No pressure to disclose, report, press charges, leave partner
- Ask patient if she feels safe
- Determine if patient has an emergency safety plan/provide referrals for shelts, domestic violence agency, mental health as needed.
10659
Describe how one pathogen increases risk of the other in terms of pneumonia
Patients older than 65 are particuarly prone to developing secondary bacteria PN after influenza infection d/t virally induced damage to the mucocillary clearence mechanisms of the respiratory eptihelium. In order, the pathogens most often responsible for secondary bacteiral pneumonia are Strep pneumoniae, Staph aureus, and H. influenzae.
What is the most likely cuase of fatigue and new-onset cardiac murmur in a young adult?
Bacteiral endocarditis. Acute, diffuse, proliferative glomerulonephritis secodnary to circulating immune complexes may complicate BE and can result in acute renal insufficency. 229
What are the individual subunits of the hemoglobin molecule structurally analogous to?
If seperated, the monomeric subunits will demonstrate a hyperbolic oxygen-dissociation (compared to the normal sigmoid shape) curve simmilar to that of myoglobin. The partial pressure of oxygen at which 50% of myoglobin molecules are oxygen saturated (P50) is only 1mmHg, which is much lower than the P50 of hemoglobin (26 mmHg). 1413
Crescent formation on LM but a lack of immunoglobulins or complement deposits on IM indicates what?
Type 3 RPGN (“pauci immune”): most patients have ANCA in thier serum. This condition is associated with Wegner’s, but it can also be idiopathic. Patients present with renal failure, pulm sx (cough, dyspnea, hemoptysis), and URT sx (epistaxis, mucosal ulceration, chronic sinusitis). 13
Remember: Type I is characterized by anti-GBM antibodies with linear deposits of IgG and C3 on IM; foudn in association wtih Goodpasture syndrome. Type II is immune-complex mediated with a “lumpy bumpy”” granular pattern of staining on IM; can be a complication of poststrep glomerulonephritis, SLE, IgA nephropathy, or HSP.
What mediates poison ivy dermatitis
Form of allergic contact dermatitis, which is a type IV HSR mediated primarily by T lymphocytes. Poison ivy/oak/sumac all produce urushiol which causes an immune response when attached to proteins (i.e. a hapten). In urushiol-induced contact dermatitis, CD8 T cells are the primary effector cells and directly destroy keratinocytes expresseing haptenated proteins. It manifests as intesnely pruritic erythematous papules, vesicles or bullae that often form linear patterns. 1133
What is the drug of choice for oropharyngeal candidiasis in patients wihtout advanced immunodeficeincy?
Nystatin: polyene antifungal; acts by binding to ergosterol in the fungal cell membrane, cuasing the formation of pores and leakage of fungal cell contents. Not absorbd from GI tract and is adminsterd as an oral “swish and swallow” agent.
What is an opioid narcotic that has partial agonist and weak antagonist activity at mu receptors?
Pentazocine, which produces analgesic effects with little to no abuse potential. When used in combo with morphine or other opioids that are selective mu receptor agonists, it can competively inhibit mu receptors and produce antagonistic effects reducing opioid analgesic effects. When given to patients dependent on morphine, withdrawal sx can occur secondary to its antagonistic effect at mu receptors. 774
What is the cuase of bilateral loss of pain and temperature sensation
Syringomyelia: formation of a cavity (Syrinx) in the cervical region of the spinal cord which damages the ventral white commissure (area of decussation for fibers of the lateral spinothalamic tract), leading ot bilateral loss of pain and temp sensation that is limited to the affected levels (typcially arms and hnds); distal sensation is preserved. Destruction of the motor neurons in the ventral horsn (due to extension of the syrinx) causes flaccid paralysis and atrophy of the intrinsic muscles of the hand. 503
What are patients with Multiple Myeloma at greatest risk of?
AL amyloidosis; amyloid protein is derived from monoclonal immunoglobulin light chaisn, and oftne forms deposits in teh kidneys, heart, tonuge, and nervous system. AL amyloidosis contributes to the developmetn of renal failure in MM. 872
What is a common SE of thiazolidinedione (e.g. pioglitazone) therapy?
Exert their glucose-lowering effect by reducing insulin ressitance by bindign to PPAR-gamma. Fluid retention, with resultant weight gain and edema. This excess fluid can exacerbate underlying CHF.
Remember: hypoglycemia can occur with the use of sulfonylureas and insulin.
What are the treatments for CHF
Include positive inotropes such as digoxin, beta-adrenegic blockers, ACE-I, and diuretics (loop and potassium sparing). Potassium-sparing diurectics act primarily by antagonizing the effects of aldosterone on the late distal tubule and cortical collecting ducts. 2005
Hematogenous ostemyelitis is predominately a disease of…
Children that most frequently affects the long bones. Staph aureus is implicated in most cases secondary to a bacteremic event. Strep pyogenes (GAS) is the second most common cause. 646
What is the most common cause of hypothyroidism
Hashimoto’s thyroiditis: mononuclear, parenchymal infiltration wtih well-developed germinal centers is a characteristic histiologic finding. Presence of hypothyroidism (elevated TSH and low T4/T3) and elevated antithyroid peroxidase antibody. 769
Why can effective immunity against infection be confered with HCV envelope proteins?
Genetic variation has led to the developmetn of a hypervariable region of the envelope glycoprotein that is especially prone to frequent mutation. There is also no proofreading 3’ –> 5’ exonuclease in the RNA polymerase. Becuase of the variety in the antigenic structure of HCV envelope proteins, the productio of host antibodies lags behind the production of new mutant strains of HCV and effective immunity against infection is not conferred. 44
What are the findings of a water deprivation test in a patient wtih primary polydipsia
This is simply excessive (pathologic) water drinking. Significant increase in urine osmolality during water depriviation study and <10% change to vasopressin administration (perhaps d/t deficency in aquaporin channels). Patients will have low serum sodium levels and osmolality. Restriction of water intake normalizes urine output in patients. 212
How can you recognize tRNA
Small, noncoding form of RNA that contains unusal nucleosides suchas pseudouridine and thymidine. tRNA has a CCA sequence at its 3’ end that is used as a recognition sequence by proteins, and the 3’ terminal hydorxyl group of the CCA tail is used as the bindign site for the amino acid. 2037
What is the major virulence factor for Strep pyogenes?
Protein M: inhibits phagocytosis and complement activation, cytotoxic for neutrophils in the serum, mediates bacterial adherence, and is the target of type-specific humoral immunity tto S. pyogenes. 723
What reduces the risk of non-hereditary ovarian and emdoterial cancer?
Oral contraceptives. Multiparity and breast-feeding also decrease the risk of ovarian cancer.
What is the prophylaxis treatmetn for the various opportunistic infections of HIV
- P. jiroveci (CD4 <200): TMP-SMX
- Toxoplasma gondii (CD4 <100): TM-SMX
- M. avium and M. intracellulare (CD4 <50): Azithromycin. Marked anemia, HSM, and elevated ALP and LDH levels are more common with MAC than extrapulm TB d/t widespread involvemetn of the RES. In contrast to MTB, MAC grows well at high temps and will exhibit optimum growth at 41C.
- Histoplasma capsulatum (CD4 <150): Itraconazole
1312
What accounts for myocardial cells that show prominent intracytoplasmic granules that are tinged yellow-brown?
Lipofuscin is the product of lipid peroxidation, accumulation in aging cells (espeically in the heart and liver of aging or cachetic, malnourished patients)
How can you remember X-linked recessive disorders
Be Wise Fools GOLD Heeds Silly HOpe
- Bruton aggamaglobulinemia
- Wiskott-Aldrich
- Fabry Disease
- G6PD Deficeincy
- Ocular Albinism
- Lesch-Nyhan
- Duchenne (and Becker) MD
- Hunter Syndrome
- Hemophilia A and B
- Ornithine Transcarbamylase
What are the Trinucleotide Repeat Disorders
X-Girlfriends First Aid Helped Ace My Test
Fragile X = CGG
Friedrich ataxia = GAA
Huntington = CAG
Myotonic dystrophy = CTG
What is the disorder in which the DNA of patients are hypersensitive to ionizing radiation?
Ataxia-telangiectasia: AR; results from defect in DNA-repair genes. Sx: cerebellar ataxia (occurs in first years of life d/t cerebellar atrophy), oculocutaenous telangiectasias, repeated sinopulmonary infections, and increased incidence of malignancy. 673
Describe the inherited disorders that are caued by deficient DNA-repair enzymes (review of 673)
- Ataxia-telangiectasia: DNA hypersensitivity to ionizing radiation. Nonhomologous end joining (brings together 2 ends of DNA fragments to repair double-strandd breaks) is mutated
- Xeroderma pigmentosum: Defective nucleotide excision repair which prevents repair of pyrimidine dimers b/c of UV light exposure. Causes premature skin aging and increased risk of malignant melanoma and SCC
- Fanconi Anemia: hypersensitivty of DNA to cross-linking agents
- Bloom Syndrome: generalized chromosomal instability. Increased susceptibility to neoplasms.
- HNPCC: defect in DNA mismatch-repair enzymes. Leads to increased susceptibility to colon cancer.
Elevated amniotic fluid levels causes which fetal anomaly
Elevated amniotic fluid levels (polyhydramnios) can either be d/t to…
- Decreased fetal swallowing: GI obstruction (e.g. d/t duodenal, esophageal, or intestinal atresia) and anencephaly
- Increased fetal urination: high cardiac output d/t anemia or twin-to-twin transfusion syndrome
What is thalamic syndrome?
Vascular lesion of the thalamus (e.g. ischemic or hemorrhagic stroke) results in damage to thalamic VPL and VPM nuclei causing complete contralateral sensory loss. Although there are no motor deficits, proprioception is often profoundly affected and may lead to difficulty ambulating and falls. 2076
What are small (5mm) cavities located within the basal ganglia, posterior limb of internal capsule, pons, and cerebellum known as?
Lacunar Infarcts: results from occlusion of small penetrating arteries that supply these structures, most comonly d/t chronic uncontrolled HTN or diabetes.
Causes (2077)
- Lipohyalinosis: destructive vessel lesion with loss of normal arterial architecture, mural foam cells, and, in acute cases, evidence of fibrinoid vessel wall necrosis.
- Microatheromas: accumulation of lipid-laden macrophages within the intimal layer of a vessel. In lacunar infarcts, they cause occlusion of a penetrating artery at or near it origin off parent vessel.
Describe the lung volumes adn expiratory flow rates in restrictive lung diseases
Decreased lung volumes and increased expiratory flow rates at the corresponding lung volumes. Expiratory flow is increasd d/t decreased lung compliance (increased elastic recoil; compliane is the change in lung volume for a given change in pressure) and increased radial traction exerted on the conducting airways by the fibrotic lung. 1543
Airflow rate is inversely related to airway resistance. Airway resistance (like vascular) is inversely related to the radius of the conduncting vessel to the fourth power. Thus, as radius of airway increases, the resistance of that airway decreases exponentially. In normal individual, high lung volumes are associated with decreased airway resistance d/t increased radial traction (outward pulling) on conducting airways exerted by the inflated and stretched pulmonary interstitium.
What is the main site for digestion vs. absorption of dietary lipids (triglycerides, cholesterol, and phopsholipids)
The duodenum is the main site of digestion of dietary lipids, while the jejunum is the main site of lipid absorption. 1018
Fatty acids, cholesterol and monoacylglycerol diffuse through the brush border membrane of the intesitnal cells without the asistance of transporter proteins. Inside the cell, TGs and cholesteryl esters are reconstructed and combined with proteins to form chylomicrons. Bile acids are necessary for lipid absorption. Cholecystectomy has little effect on lipid digestion and absorption though patients may find it difficulty to eat a large fatty meal b/c they do not have ability to release a large amount of stored bile into the gut.
What are the ABX effective against P. aeruginosa?
- Anti-pseudomonal penicillins: ticeracillin, piperacillin
- Cephalosporins: ceftazidime (3rd gen), cefepime (4th gen)
- Aminoglycosides: amikacin, gentamicin, tobramycin
- Fluoroquinolones: cipro, levo
- Monobactams: aztreonam
- Carbapenems: imipenem, meropenem
What is the TATA box?
Promoter region that binds transcription factors and RNA polymerase II during the initiation of transcritpion. It is located 25 bases upstream from the beginning of the coding region.
What is the common pathologic morphology shared by Takayasu artertis and temporal arteritis
Granulomatous inflammation of the media.
Takayasu and temporal involve arterial vessels of different sizes and locations (aorta and proximal aortic arterial branch involvement vs. more distal carotid artery branch involvment, respectively)
What is a diuretic that can be used to treat acute angle-closure glaucoma?
Acetazolamide: inhibits carbonic anhydrase in the proximal tubule, which is responsible for catalyzing rxns necessary for NaHCO3 reabsorption. Inhibting CA results in enahanced HCO3- and water excretion. SE include somnolence, parasthesias, and urine alkalinization; rarely metabolic acidosis, dehydration, hypokalemia and hyponatremia. 682
CA is also found in eyes, pancreas, GI tract, CNS and RBC. In eyes, inhibtion of CA will decrease HCO3- and acqueous humor formation
What water-soluble vitamin must be replaced after total gastrectomy?
**Parenteral administration of vitamin B12. **
Parietal cells in the body and fundus of the stomach secrete IF (in adition to HCl) which binds to vitamin B12. This complex is then absorbed by enterocytes in the terminal ileum.
What is the MOA and mechanisms of resistance to Streptomycin
Aminoglycoside that inhibits protein synthesis by inactivating the small 30S RSU. Mutations that result in altered structure of bacterial ribosomal proteins are responsible for aminoglycoside resistance because they modify the ribosomal binding sites for these drugs.
Other mechanisms of resistance to MTB drugs include decreased activity of bacterial catalase-peroxidase (resistance to isoniazid) and structural alterations of enzymes involved in RNA synthesis such as DNA-dependent RNA polymerase (resistance to rifampin)
Primary biliary cirrhosis has similar histologic findings to what other disorder?
PBC: autoimmune destruction of intrahepatic bile ducts and cholestasis. In the pre-cirrhotic stage, interlobular bile ducts are destroyed by granulomatous inflammation (“florid duct lesion”) and heavy lymphocyte-predominant portal tract infiltrate. The simmilarity of these findings to those of graft vs. host disease suggest that immunolgoic injury is responsbile for disease manifestations. 402
Episodic HTN, tachycarida, HAs, diaphoresis, pallor or flushing and tremors are important clinical clues for the presence of what?
Catecholamine-secreting tumors (e.g. pheochromocytoma of adrenal medulla). Differential shoudl also include autonimc dysfunction, cocaine abuse, withdrawal from clonidine and beta-blockers, and panic attacks. 933
What type of study simultaneously measures exposure and outcome at a particular point in time?
Cross-sectional study (also known as a prevalence study). In other study designs, a certain time period seperates the exposure from the outcome. The major limitation of cross-sectional design is that a temporal relationship between exposure and outcome is not always clear. 1276
What is an efective add-on therapy for patients with severe allergic asthma that has been shown to be effective in reducing dependency on both oral and inhaled steroids?
Many asthmatics frequently have allergies as a trigger d/t a high IgE response in the body. Anti-IgE antibodies given as a SC injection (i.e., omalizumab) have been shown to be effective in patients with moderate-to-severe allergic asthma. Omalizumab is a recombinant humanized IgG1 monoclonal antibody that binds with IgE to inhibit the action of IgE with its receptor on mast cells, basophils, and other cell types and descrease the allergic response. 2128
What is the main immune mechanisms against Giardia?
Giardia lamblia causes injury to duodenal and jejunal mucosa by adhering to the intestinal bursh border and releasing molecules that induce a mucosal inflammatory response. Secretory IgA, which binds to trophozoites and impairs adherence, is the major component of adaptive immunity against infection. Conditions causign IgA deficeincy predipose patients to chronic giardiasis. 1596
Describe the pathway of aqueous humor production
Produced by the epithelial cells of the ciliary body –> excreted into posterior chamber and transferred through the pupil into anterior chamber –> trabecular meshwork through which aqueous humor diffuses into Schlemm’s canal –> episcleral and conjunctival veins. 1363
What types of drugs are used to treat Glaucoma?
Chronic eye disease characterized by increased IOP. Both narrow-angle and open-angle can ultimately lead to blindness. Drugs used to treat either decrease production of aqueous humor or increase its outflow. 1363
- Timolol (non-selective BBs): diminish secretion of aqueous humor by ciliary epithleium.
- Acetazolamide: CA inhibitor; decreases aqueous humor secretion by ciliary epithleium
- PGF2a (latanoprost, unoprostone, tavoprost) and cholinomimetics (pilocarpine, carbachol) decrease IOP by increasing outflow of aqueous humor.
Trochlear nerve (CN IV) palsy is characterized by what?
Vertical diplopia: most noticeable when the affected eye looks toward the nose, as occurs when reading teh newspaper or walking downstairs.
Tissue damage and resultant abscess formation is primarily caused by what?
Lysosmal enzyme release from neutrophils and macrophages. 302
What are the stabilizing forces for the various structures of proteins?
- Primary: sequence of AAs linked by covalent peptide bonds.
- Secondary: alpha-helix or beta-sheet; d/t hydrogen bonding
- Tertiary: compact folding of secondary structure; ionic bonds, hydrophobic interactions, H-bonds, and disulfide bonds. Disulfide bonds enhance a proteins ability to withstand denaturation.
What disease is associated with C-ANCAs that may target neutrophil proteinase 3?
Necrotizing vasculitis of the upper and lower respiratory tract (causing nasal ulcerations, sinusitis, hemoptysis) and rapidly progressive glomerulonephritis - producing a varaible degree of renal failure - is characteristic of granulomatosis with polyangitis (Wegners). 459
What is the preferred method for testing for type 1 and 2 diabetes
Fasting blood glucose (NOT oral glucose tolerance test, which is usually reserved for screenign pregnat women for gestational diabetes)
How can you distinguish teh pneumoconiosis that can casue exertional dyspnea nad interstital densities on CXR
- Silicosis: produces eggshell calcifications of hilar nodes and birefringent particles surrounded by fibrous tissues
- Asbestosis: calcified pleural plaques and ferruginous bodies. Interstital pattern of involvement, more prominent in lower lobes.
- Berylliosis and hypersenstiivty pneumonitis: noncaseating granulomas
- Coal miners lung: perilymphatic accumulations of coal dust-laden macrophages
What are the four major causes of hypoxemia (low PaO2)
- Alveolar hypoventiliation or inspiration of air with low pO2 (e.g. at high altitude).
- Ventilation-perfusion mismatch: poor ventilation of a well-perfused alveoli results in physiologic shunting, leading to an increased A-a gradient (e.g. pneumonia, obstructive pulm disease, pulm embolism)
- Diffusion impairment: diseases that cuase thickening of the alveolar capillary membranes (E.g. alveolar hyaline membrane diseases). Increased A-a as there is a normal concentration fo O2 in alveoli that cannot be transported into blood.
- Right-to-left shunting: cyanotic congential heart defects; venous blood bypasses lung and enters arterial circulation, thus decreasing PaO2.
The A-a gradient is normal with alveolar hypoventilation and helps to distinguish it from other forms of hypoxemia. Hypoventilation is common in patients with suppressed central respiratory drive (eg sedative overdose, sleep apnea) or those with disease that decrease the inspiratory capacity (eg myasthenia gravis, obesity). 1582