Kaplan Flashcards
complications of thyroidectomy
Hypocalcemia (due to PTH gland damage) resulting in cramps, paresthesias, tetany (trousseau’s sign and chvostek’s sign)
Hoarseness (recurrent laryngeal nerve damage)
Primary cell type responsible for wound contracture
myofibroblasts bc they express actin and myosin
fibroblasts only extracellular matrix, collagen, proteoglycan, and elastin
Signs and sx’s of scarlet fever. Associated bug and toxin
pharyngitis followed by blanching “sandpaper” rash (palms and soles spared), circumoral pallor, “strawberry tongue” and N/V
Strep pyogenes
fever and rash due to pyogenic exotoxins A-C which are phage-encoded
Which bugs have alpha toxins and how do they differ?
Staph aureus alpha toxin is pore forming - toxic shock syndrome with desquamating (skin peeling) rash
C. perfringens is lecithinase. bug causes gas gangrene
What is bacterial endotoxin?
LPS found in the outer leaflet of the outer membrane of G- bugs. Activates macs to overproduce IL-1, IL-6, and TNFa producing G- shock.
Hyaluronic acid capsule and M protein are both produced by which bug? function of each?
Strep pyogenes
Hyaluronic acid capsule non-immunogenic, makes it difficult for immune system to recognize pathogen
M protein is anti-phagocytic surface protein
Ulnar nerve damage at the level of the elbow results in what symptoms
weakened wrist flexion, loss of abduction and adduction of the digits, loss of the hypothenar muscles, and weakened flexion of the interphalangeal joints of the 4th and 5th digits. Clawing of medial 2 digits (may be worse with injury at the wrist). There may also be sensory loss on digit 5 and the medial half of digit 4.
Median nerve injury results in what symptoms
loss of function of lateral two lumbricals, flattening of the thenar eminence, and sensory loss on the palmar surface of digits 1, 2, 3, and the lateral half of 4.
Musculocutaneous nerve injury would result in what symptoms?
rarely happens but will result in loss of sensation to lateral forearm and decreased flexion at the elbow.
loss of wrist extension is due to what nerve injury? what are the common locations associated with this nerve injury?
Radial nerve injury, commonly mid-humoral fractures. additional sx’s include decreased extension of digits, weakened supination, and sensory loss to posterior forearm and hand.
The following describes what disease?
Autosomal dominant
Facial lesions (adenoma sebaceum)
Hypopigmented “ash-leaf” spots on the skin
Multiple hamartomas, including cortical tubers
Renal angiomyolipomas
Cardiac rhabdomyomas
Astrocytomas
Subependymal giant cell astrocytoma
Tuberous sclerosis
mutations in TSC1 or TSC2
Lisch nodules-what are they and what are they associated with
small, pigmented, nodular lesions (hamartomatous) associated with NF1
(also associated with NF1 are cafe-au-lait spots, axillary freckling, neurofibromas, scoliosis, pheochromocytoma, and optic gliomas)
Bilateral schwannomas of the 8th cranial nerve are associated with what disease?
NF2
What are the serologic markers for SLE.
anti-nuclear antibody (sensitive, not specific)
anti-dsDNA (specific, not sensitive)
anti-Smith (specific, not sensitive)
anti-histone (for drug induced lupus)
Why is lactulose given to patients with hepatic encephalopathy?
reduces ammonia absorption in the gut by acidifying colonic contents to convert NH3 to NH4 which will be trapped and excreted in feces.
Immunocomprimised patient from Eastern Great Lakes, Ohio, Mississippi, or Missouri, presenting with flu like symptoms and a rash should cause concern for what organism? what are characteristics of this organism?
Histoplasma capsulatum
intracellular yeast therefore non-communicable from person to person
acquired though inhalation of conidia or spores in soil or dust
What dangerous side effects are possible with Succinylcholine?
Hyperkalemia (tall T waves on EKG)
malignant hyprethermia
prolonged paralysis
respiratory depression
cardiac arrhythmias
rhabdomyolysis
myoglobinemia
Sickle cell nephropathy most commonly causes occlusion of what vessel producing what kind of symptoms?
Occlusion of vasa recta due to very high osmolarity and hypoxic environment (sickle cells predisposed to sickling in this environment)
ischemia results in patchy papillary necrosis, hematuria, proteinuria and sometimes cortical scarring.
A pt with history of emphysema and chronic bronchitis should be treated with what kind of antihypertensive?
Beta-1 receptor antagonist (selective)
A BEAM acebutolol betaxolol esmolol atenolol metoprolol
at low doses this will not exacerbate bronchoconstriction
Which antimicrobials should be avoided during pregnancy?
Sulfonamides-kernicterus
Aminoglycosides-ototoxicity
Flouroquinolones-cartilage damage
Clarithromycin-Embrotoxic
Tetracyclines-discolored teeth inhibition of bone growth
Ribavirin-teratogenic
Groseofulvin-teratogenic
Chloramphenicol-Gray Baby syndrome
“SAFe Children Take Really Good Care”
Name the layers of the anterior abdominal wall from outside to inside
Skin-Campers fascia-Scarpa fascia-external oblique-internal oblique-transversus abdominis muscle-transversalis fascia-parietal peritoneum
describe schizophreniform disorder
symptoms similar to schizophrenia lasting more than 1 month but less than 6 months
Describe schizoaffective disorder
symptoms of schizophrenia with mood changes like depression or mania
describe schizoid personality disorder
withdrawl from social relationships and flat affect
describe schizotypal personality disorder
eccentric behavior, odd beliefs like magic, difficulties with relationships
describe the clinical symptoms of myasthenia gravis
ptosis, diplopia, weakness, WORSENS with muscle use, improvement after edrophonium test
Describe Wiskott-Aldrich syndrome and characteristic lab findings
TRIAD: thrombocytopenia, eczema, and immunodeficiency (recurrent sinopulmonary infections)
IgM and IgG levels are depressed; IgE and IgA levels are normal to elevated
Name disease:
PAS positive inclusion bodies in neurons
UMN/LMN deficits but no sensory or bowel/bladder deficits
treatment for this disease?
Lou Gehrig aka Amyotrophic lateral sclerosis
treat with Riluzole (unclear mechanism that decreases gluatamate excitoxicity)
African/endemic burkitt lymphoma can present as masses in what areas of the body?
jaw, ovarian, breast, and GI tract
“get a JOB G”
What is the underlying cause of epithelial foot effacement in Minimal change disease? What are other conditions associated with MCD?
abnormal secretion of lymphokines by T cells, reduces the production of anions in the GBM, thereby increasing the glomerular permeability to albumin and other proteins
Hodgkin disease and T-cell lymphoma, are sometimes associated with minimal change disease.
Describe Delusional disorder
one or more non-bizarre delusions in the absence of other significant psychopathology (delusions are believable)
The patient is usually quite functional, without odd or bizarre behavior
Describe how to calculate APGAR? When are scores taken and what do they indicate?
Appearance, Pulse, Grimace, Activity, and Respiration.
The 1-minute score determines how well the newborn tolerated the birthing process and the 5-minute score determines how well the newborn is doing outside the mother’s womb
0-2 in each category max score 10: HR >100bpm, Respirations crying, color pink, tone active motion, and reflex irritability cry or active withdrawal.
What is familial gestational hyperthyroidism?
caused by increased responsiveness of thyroid-stimulating hormone (TSH) receptors to human chorionic gonadotropin (hCG) and/or luteinizing hormone (LH).
hCG rises early in pregnancy and then declines (which would cause elevated thyroid hormones in the serum the first 10 weeks).
Progesterone, estrogen, and human chorionic somatomammotropin increase throughout pregnancy and do not have the same effect on TSH receptors.
Causes of metabolic acidosis?
MUD PILES
M-Methanol U-Uremia (chronic kidney failure) D-Diabetic ketoacidosis P-Propylene glycol ("P" used to stand for Paraldehyde but this substance is not commonly used today) I-Infection, Iron, Isoniazid, Inborn errors of metabolism L-Lactic acidosis E-Ethylene glycol (Note: Ethanol is sometimes included in this mnemonic as well, although the acidosis caused by ethanol is actually primarily due to the increased production of lactic acid found in such intoxication.) S-Salicylates
**Recognize acute respiratory acidosis by a decrease in pH, an elevation in the PCO2, and a NEAR-NORMAL bicarbonate on arterial blood gas.
Which lysosomal storage disease presents with hepatosplenomegaly and cherry-red spot(s) on the macula
Neimman-Pick (sphingomyelinase deficiency)
sphingomyelin in histiocytes (macrophages) referred to as “sea blue” histiocytes
EM-> “zebra bodies”
higher in ashkenazi jews
Which condition should be suspected in infants with growth failure, cataracts, liver disease, aminoaciduria, and mental retardation?
what other condition causes cataracts in early life?
Classical galactosemia, autosomal recessive disease caused by a deficiency of galactose-1-phosphate uridyltransferase, which is necessary for the formation of glucose from dietary galactose, derived from milk lactose.
Treatment involves strict dietary lactose restriction,
Galactokinase deficiency can also cause catarcts in early life
What are the major microscopic findings in Alzheimer’s disease?
Neurofibrillary tangles:
Intraneuronal aggregates of insoluble cytoskeletal elements
Composed mainly of abnormally phosphorylated tau protein, forming paired helical filaments
During the first few minutes of exercise what happens to tissue levels of creatine phosphate, myoglobin oxygen, and ATP?
ALL will decrease to feed muscles as oxygen demands increase about 10 fold during exercise. Depletion of stores results in plataued increased level of oxygen consumption
What is the name and characteristics of the “rare protein losing gastropathy”?
Ménétrier disease, commonly seen in elderly patients, is a hyperplastic gastropathy characterized by enlarged rugal folds
marked hyperplasia of mucus-secreting cells (foveolar cells), leading to hypersecretion of mucus in such large quantities as to cause decreased protein absorption and losses through the gastrointestinal tract.
What are the four exceptions to informed consent rule:
Emergency
Waiver by the patient
Patient is incompetent
Therapeutic privilege (used if the patient is unconscious, confused, or the physician deprives patient of autonomy in interest of health).
ectopic pregnancy is a life threatening condition
Toxoplasmosis from toxoplasma gondii results in symptoms in what kinds of patients?
immunocompromised patients, the infection arises as a REACTIVATION of a latent infection, generally in the form of central nervous system infection (ring-enhancing lesions).
Primary infection during pregnancy may cause severe congenital infections (early infection) or long-term complications such as blindness (later infection).
Acid fast oocytes 4-6 microns in diameter in stool is what bug?
Cryptosporidium
Hypothalamic somatostatin acts on the anterior pituitary to inhibit the release of which hormones?
growth hormone (GH) and thyroid-stimulating hormone (TSH)
The most common cause of neonatal meningitis is? what are it’s distinguishing factors?
Group B streptococci or Streptococcus agalactiae (second is E.coli, third Listeria)
S. agalactiae are beta-hemolytic on blood agar positive with the CAMP test
Describe areas of drainage associated with thoracic and right lymphatic ducts.
thoracic duct drains lymph from left side of the head, neck and chest, the left upper limb and the entire body below the diaphragm.
The right lymphatic duct drains the right upper limb, the right side of the chest, and the right side of the head and neck
What are symptoms of Ataxia telangiectasia? What gene is it associated with? Gene function/consequence of mutation?
characterized by telangiectasias of the skin and eyes, variable immunodeficiency, and progressive ataxia
ATM codes for a DNA-dependent protein kinase responsible for recognizing and correcting DNA double strand breaks.
Mutation of ATM causes increased sensitivity to ionizing radiation and defective DNA repair
lymphoreticular malignancies (Hodgkin and non-Hodgkin lymphomas and leukemias) is increased
Which antidepressant is associated with ischemic priapism?
Trazodone
lesion of the oculomotor nerve can result in ?
Inability to adduct the eye (leading to horizontal diplopia)
Mydriasis (dilation)
Ptosis
Inability to constrict the eye
Loss of accommodation
What is Reiter syndrome?
a reactive arthritis, is triggered following urogenital or enteric infections
conjunctivitis, urethritis, and arthritis.
“can’t see, can’t pee, can’t climb a tree”
HLA-B27 is associated with this disease.
Aspirated joint fluid resembles that of rheumatoid arthritis (inflammatory arthritis with predominance of neutrophils)
CREST syndrome is associated with what autoantibodies?
Anticentromere
Chagas disease is associated with what symptoms? how do you contract the disease?
myocarditis or esophageal involvement, producing achalasia mega colon
spread by the reduviid bugs (“kissing bugs”)
What is the formula for volume of distribution when clearance and half life are known?
t1/2 = (0.7 x Vd)/CL
Will PE show up on a chest Xray?
No. but useful to rule out other disease
What is the most common site of intestinal involvement with Crohn’s disease?
Gross characteristics?
terminal ileum
harp demarcation of affected segments of bowel, which may show edema, stricture and fibrosis–risk obstuction
JVD indicates increased pressure in which part of the circulatory system?
central venous pressure is increased
ELEK test can be used for diagnosing what infection?
Diptheria vs non toxin producing normal flora diptheroid.
Can also use ELISA testing
Characteristics of Antisocial Personality Disorder?
Inability to conform to social norms (delinquency, theft, etc.)
Cannot hold a stable job
Prone to violence and crime
ecthyma gangrenosum, the hallmark lesion of?
Pseudomonas aeruginosa infection. Common nosocomial infection in cystic fibrosis, neutropenia, chronic granulomatous disease, or severe burns
Gram-negative, oxidase-positive bacillus
Endotoxin results in gram-negative shock in septicemia.
Exotoxin A inactivates elongation factor-2, inhibiting protein synthesis (like diphtheria toxin).
Describe location of cervical nerves in relation to vertebral bodies?
C1-C7 exit above C8 exits below C7
bilateral parotitis, orchitis, and pancreatitis is characteristic of what?
Mumps
Paramyxovirus: negative-sense, single-stranded RNA, helical, enveloped virus
which hypersensitivity is implicated in parasitic infections?
type I
eosinophilia and IgE mediated
Which drug should be used as first line in a type 2 Diabetic? When should this drug not be used? Which is the next drug in line to add to the first line drug when the diabetes is still out of control?
Usually 1st-Metformin should not be used in patients with renal insufficiency, heart failure, conditions predisposing to hypoxemia, or lactic acidosis.
Glimepiride is a second-generation sulfonylurea that undergoes hepatic clearance and is safer to use in patients with renal dysfunction than metformin.
Sulfonylureas are indicated in patients with type 2 diabetes when their diabetes cannot be controlled with metformin, diet and exercise alone.
What is a paradoxical embolism?
Emboli from venous circulation that bypasses the lungs and goes into systemic circulation. May cause ischemic stroke in the brain.
This can happen with persistent foramen ovale
What is a distinguishing CSF finding in viral meningitis?
normal glucose (45-80)
How can you distinguish bacterial CSF findings from mycobaterial/fungal?
In bacterial meningitis the distinguishing factor is the markedly elevated leukocytes (up to 90,000 neutrophils.
In mycobacterial/yeast meningitis you will see 100-1000 lymphocytes.
normal is <5
What is elevated in all forms of meningitis?
proteins (>50) and pressure (viral less than bacterial/mycobaterial/yeast)
eosinophilic granulomatosis with polyangiitis is also known as what? what are characteristics of this disease?
Churg-Strauss syndrome
necrotizing vasculitis affecting multiple organ systems.
history of asthma or allergic rhinitis, labs show eosinophilia and elevated p-ANCA
What is the inheritance pattern of familial hypercholesterolemia?
AD–> xanthomas
What is the drug of choice for pre-operative thyroidectomy?
Potassium iodide -hyperthyroidism in preoperative thyroidectomy patients. decreases vascularity for better surgery outcomes
X-linked recessive disorder that results from near-complete deficiency of the purine salvage enzyme hypoxanthine-guanine phosphoribosyl transferase (HPRT)
what would be increased in the serum of this pt?
Lesch Nyhan
intellectual disability, spastic cerebral palsy with compulsive biting of hands and lips, hyperuricemia (leading to gouty arthritis, urate crystal formation, tophi, and urate nephropathy), and death, often in the first decade
monosodium urate
Premature infant with respiratory distress and a biopsy showing surfactant-rich exudate and silver-staining cysts
Pneumocystis jirovecii
What are the medically important species undergo natural transformation?
Haemophilus species, Streptococcus species, Neisseria species and Helicobacter pylori.
Lead poisoning affects which enzymes in the heme biosynthetic pathway? Treatment for lead poisoning?
aminolevulinate (ALA) enzymes.
Treatment involves chelation of lead (EDTA, dimecaprol, succimer).
What is the formula to calculate constant steady state dose?
Css=Dosing rate/CL . Important to have same units!
Which bacteria acquire traits by lysogenic conversion?
COBEDS (Cholera toxin, O antigen of Salmonella, Botulinum toxin, erythrogenic exotoxins of Streptococcus pyogenes, diphtheria toxin, and shiga toxin)
When 2 people share a bed somebody gets a little pregnant [with phage]
Patients on MAO inhibitors (MAOIs) need to avoid what foods?
name some MAOIs
tyramine-containing foods (e.g., cheese, wine, beer) to prevent a sympathetic (or hypertensive) crisis
Phenelzine, tranylcypromine, and isocarboxazid Selegiline
(MAO Takes Pride In Shanghai)
tumors from the penis, skin of the labia, scrotum (not testes), and anal canal below the dentate line drain to what lymph nodes?
superficial group of inguinal lymph nodes
Attention-seeking, dramatic, seductive, extroverted
Unable to maintain long-lasting relationships
Are all characteristics of what personality disorder?
Histrionic
all personality disorders are ego-syntonic-they do not bother the pt but do bother others
What is the Mechanism of action of the pertussis toxin?
ADP-ribosylates the Gαi subunit of the heterotrimer G protein complex, resulting in an increase in cAMP levels inside the cell.
high-dose glutamate causes excitotoxic injury to neurons by binding what type of receptors?
Ligand-gated and voltage-gated cation channel
Glutamate also binds metabotropic (G-protein coupled: mGluR) but this would nt account for cytotoxic effects.
In a type III HS reaction, which component of the complement pathway is most important in deposition of immune complexes into tissues (aka removal from serum)
C3b.
C3b attaches to bacterial surfaces for opsonization by phagocytes
C3b is also critically involved and facilitates the removal of immune complexes from the serum when C3b-bound-complexes bind to complement receptors on phagocytic cells such as macrophages
Describe the difference in blood flow to tissues in dynamic/endurance exercise vs. static high intensity exercise?
During high-intensity static exercise, such as weight lifting, blood vessels are compressed by the skeletal muscle, thus increasing vascular resistance and decreasing blood flow (increases MAP)
During dynamic, endurance exercise, vascular resistance decreases and blood flow increases due to metabolic vasodilation of arterioles.
most common cause of non-traditional pneumonia in AIDS patients with CD4+ T cell counts above 400/μl?
counts of less than 100/μl?
Mycobacterium tuberculosis
M. avium-intracellulare (mostly below 50)
What are the features of SIADH?
Increased plasma ADH
Decreased serum osmolality
Increased urine sodium concentration
Normal serum potassium levels
Increased plasma atrial natriuretic peptide levels
Decreased plasma aldosterone levels
Clinical presentation of confusion, weakness, fatigue, and weight gain
What is the most common mutation in CF and what is the consequence of this mutation (i.e. specific type of mutation and result)
deletion of phenylalanine at position 508 (delta-F508),
interferes with proper protein folding and the post-translational processing of oligosaccharide side chains.
PPD is an example of what type of hypersensitivity?
Type IV Th1 secrete IFNg attract and activate macrophages
Difference between genetic drift vs shift?
Which associated with genetic reassortment?
Which associated with influenza pandemics?
Minor antigenic changes (antigenic drift) in surface antigens-reason for yearly tweak in flu vaccine
larger, abrupt changes in antigen expression (antigenic shift) as a consequence of reassortment-pandemic
What is the good samaritan law?
he Good Samaritan law says that physicians do not have to stop to help in a non-medical situation such as an automobile accident
If help is offered, the physician is shielded from liability, provided that:
Actions are within the physician’s competence
Only accepted procedures are performed
The physician remains at the scene until relieved by competent personnel
No compensation is accepted
What is Tacrolimus, mechanism, and indications?
macrolide immunosuppressant produced by Streptomyces tsukubaensis, which is a species of Actinobacteria
inhibits calcineurin-mediated transcription of IL-2. It binds to an immunophilin, termed the FK binding protein (FKBP) and inhibits the first phase of T-cell activation.
indicated for heart, liver, and kidney transplant prophylaxis
Pt presents with: Increased aldosterone secretion Hypertension Suppressed plasma renin activity Hypernatremia and hypokalemia
Conn syndrome (primary hyperaldosteronism)
What is the mechanism of the diptheria toxin? Which aspect of the cell is the toxin target? and the consequence?
inactivates eukaryotic elongation factor-2 (eEF-2) in via ADP-ribosylation. This inhibits cell protein synthesis. Directly affects translation elongation
X-linked SCID is associated with which gene mutations? How does the disease present?
mutations in the gene encoding the common γ chain shared by the receptors for IL-2, 4, 7, 9, and 15.
Recurrent infections, low to absent Tcells (CD3) and normal Bcells (CD19)
Defective CD40L causes what?
hyper IgM syndrome where Bcells cant do class switch
What are RAG genes responsible for? What lab findings would you see in a patient with a RAG deficiency/mutation?
responsible for VDJ recombination of lymphocytes (determines receptors)
would see absent Tcells (CD3) and Bcells (CD19) in the periphery
ADA deficiency will also show absent T/Bcells and pt usually dies by age 2 if no bone marrow transplant. accumulation of toxic metabolites results in death of T/B stem cells in the bone marrow
What is the function of the Sarco/endoplasmic reticulum calcium ATPase (SERCA)?
renormalization of intracellular calcium concentrations following muscle contraction
Velocity of shortening muscle fibers is associated with the what activity in the muscle cell?
Myosin ATPase
What drug should you give to stop a migraine and what is it’s mechanism of action?
Triptans (sumatriptan, almotriptan, rizatriptan, and zolmitriptan)
5-HT1B/1D agonists and are used in abortive therapy of migraine
They cause vasoconstriction and prevent the release of peptides such as CGRP and substance P from trigeminal afferents.
Typical pneumonia in alcoholics with red, gelatinous sputum?
Characteristics of the bug?
Treatment?
Klebsiella pneumoniae
gram-negative, oxidase-negative bacillus that is lactose-fermenting.
Treatment is with a third-generation cephalosporin with or without an aminoglycoside.
Which bugs would cause the following symptoms of pneumonia
blue-green color and a fruity odor
cause of pneumonia, pharyngitis, bronchitis, and otitis media, but does not typically cause lobar consolidation or bloody sputum.
“rusty,” or tinged with brown blood
salmon-colored sputum
gram-negative, oxidase-positive bacillus: Pseudomonas aeruginosa
gram-negative pleomorphic bacillus requiring factors X and V: Haemophilus influenzae
gram-positive, catalase-negative coccus: Streptococcus, of which S. pneumoniae is the most common cause of typical pneumonia in adults (including alcoholics)
gram-positive, catalase-positive coccus: Staphylococcus, of which S. aureus is a common cause of pneumonia. Associated with aspiration pneumonia in alcoholics
Idiopathic thrombocytopenic purpura (ITP) is an acquired bleeding disorder due to the development of which autoantibodies?
IgG autoantibodies against platelet membrane glycoproteins such as GPIIb/IIIa.
result in platelet destruction, resulting in thrombocytopenia
petechiae, purpura, and easy bruising are common. Epistaxis, gingival bleeding, and menorrhagia may also be the cause of presentation.
case-control study analysis- odds ratio is used
whereas for analysis of a cohort study- relative risk or attributable risk are used
What are the equations for each analysis?
Odds Ratio = (A/C) / (B/D) = (AD) / (BC)
(diseased and exposed/diseased and not exposed)/(healthy and exposed/healthy and not exposed)
relative risk = [A/(A+B)] / [C/C+D)]
Attributable risk = [A/(A+B)] - [C/C+D)]
List side effects of lithium and the necessary monitoring while prescribing this medication?
what organ is responsible for metabolism and excretion of lithium?
Acute side effects: tremor (propranolol may be used to treat this), nausea, polyuria (related to nephrogenic diabetes insipidus) and thirst, weight gain, and cognitive impairment.
Hypothyroidism and goiter (↓ thyroid-stimulating hormone effects and inhibits 5’-deiodinase). Thyroid function tests should be given 1 or 2 times during the first 6 months, and every 6 to 12 months thereafter in higher risk patients. If hypothyroidism develops, treat with T4.
Nephrogenic diabetes insipidus (↓ antidiuretic hormone effect), causing polyuria and dilute urine; amiloride is used to treat. Other renal dysfunction can also occur. Urinalysis, BUN and creatinine should be checked every 2 to 3 months during the first 6 months and every 6 to 12 months thereafter.
Teratogenicity: Ebstein’s anomaly (malformed tricuspid valve)
KIDNEY
In a pt with AML what drug should also be given when administerin chemo?
Allopurinol to prevent tumor lysis syndrome (hyperuricemia and uric acid stones) from all the purine breakdown caused by chemo
what are the findings of the swinging flashkught test in Marcus-Gunn pupil
Shine light in Marcus Gunn pupil ⇒ pupils do not constrict fully
Shine light in normal eye ⇒ pupils constrict fully
Shine light in affected pupil again ⇒ apparent dilation of both pupils because the stimulus carried by the optic nerve (CN II) of the affected eye is weaker than the normal eye.
A pressure differential of >20 mm Hg between upper and lower extremities is diagnostic of what?
Why do you see HTN?
coarctation of the aorta
activation of the renin-angiotensin-aldosterone system (RAAS)
What is Medication reconciliation?
process where an institution can accurately and completely reconcile medications across the continuum of care to prevent medication errors.
Chronic hemodialysis results in accumulation of what protein?
Joint aspiration produces synovial fluid that stains positively with Congo red and shows green birefringence with polarized light.
beta-2 microglobulin (beta-2-M) due to the inability of the kidney to eliminate it.
Beta-2-M deposits as an amyloid protein, primarily in muscles, joints, tendons, and bones.ood
Most common cause of Intracerebral (intraparenchymal) hemorrhage?
Sites of brain affected?
findings on autopsy?
Hypertension is the most frequent predisposing condition.
It can involve the basal ganglia, internal capsule, thalamus, pons, centrum semiovale, and/or cerebellum.
The internal capsule and basal ganglia are the most frequent sites for massive intraparenchymal hemorrhage.
Hypertensive hemorrhages are often massive, and frequently dissect through the brain parenchyma into the ventricular system (autopsy shows ventricles filled with blood
Lung cancer commonly metastasizes to the adrenals, resulting in adrenocortical insufficiency. List symptoms that would manifest.
hyperpigmentation of the skin and mucous membranes
fatigue, gastrointestinal distress, anorexia, weight loss, hypoglycemia hypotension
and in some cases salt wasting (hyponatremia and volume depletion), hyperkalemia, and metabolic acidosis.
Which adrenergic receptors are related to uterine contraction and relaxation?
Stimulation of α1 causes uterine contraction; stimulation of β2 receptors causes uterine relaxation.
β2 receptors stimulates Gs, increases cAMP, and activates protein kinase A (PKA). As a result of PKA phosphorylation and inactivation of myosin light-chain kinase (MLCK), uterine smooth muscle contractions are reduced
Stimulation of α1 receptors stimulates Gq, increases intracellular levels of IP3, and increases intracellular calcium, causing greater smooth muscle contraction.
Aldosterone synthesis and release is controlled by what substances?
Angiotensin II and Potassium. NOT ACTH!
High levels of aldosterone would present with hyper or hypo- kalemia and natremia?
hypokalemia and hypernatremia
How does pregnancy result in an increased total T4 count?
Pregnancy increases estrogen, which leads to an increased synthesis of TBG and an increase in total serum T4
Besides hypertension and proteinuria what would you see in preeclampsia? What is preeclampsia thought to be due to?
edema, elevated AST, elevated ALT, and low platelets
secondary to placental ischemia
Aside from mental retardation, trisomy 21 is associated with numerous other medical issues. List them.
acute lymphoblastic leukemia, cardiac malformations, Alzheimer-like changes, epilepsy, duodenal atresia, and visual problems
Pseudomyxoma peritonei is produced when a benign or malignant mucus-producing tumor (mucinous cystadenoma or mucinous cystadenocarcinoma) produces gelatin-like mucus that fills the peritoneal cavity “jelly belly”
The ovaries and appendix are the usual sites for these tumors.
What is a Ghon complex?
granulomatous lesions formed during an infection with Mycobacterium tuberculosis (seen in the lung)
Klebsiella pneumoniae mimics which other bug as a pulmonary pathogen? what would you see on CXR of pneumonia caused by Klebsiella?
Strep. pneumo
lobar pneumonia associated with cavitation
Pyruvate carboxylase is a mitochondrial enzyme requiring ?. It is activated by ? During gluconeogenesis it produces ? that can ultimately be converted to glucose.
A deficiency of pyruvate carboxylase leads to accumulation of ?.
What is the inheritance pattern and what lab findings would you see?
Biotin
acetyl CoA (from b oxidation FA)
oxaloacetate (OAA)
Accumulation of gluconeogenic precursors, alanine, pyruvate and lactate
Auto Rec - characterized by elevated levels of alanine, pyruvate, lactate, and ketoacids
The presence of ?-cells is key to the diagnosis of intraductal papilloma or any benign condition
myoepithelial cells
Consider ?? in a young woman who presents with hirsutism, amenorrhea, and obesity.
Confirm the diagnosis by demonstrating increased serum ?, decreased serum ?, increased testosterone, and cystic follicles in the ovary.
polycystic ovary syndrome
increased LH and decreased FSH
Which viruses possess segmented genomes and are therefore capable of undergoing genetic shift?
Viruses with segmented genome: ROBA (Reoviridae, Orthomyxoviridae, Bunyaviridae, Arenaviridae)
What are the signs of anticholinergic toxicity?
“Red as a beet”: Cutaneous vasodilation (flushing) occurs to dissipate heat to compensate for impaired sweat formation.
“Dry as a bone”: Muscarinic receptors are on sweat glands, so blockade of these receptors stops sweating (anhidrosis), causing dry skin.
“Hot as a hare”: Interference with sweating can lead to hyperthermia.
“Blind as a bat”: Blocking muscarinic receptors on the ciliary muscle prevents accommodation (causing blurry vision). Muscarinic receptors on the pupillary sphincter muscle are also blocked, causing pupillary dilation (mydriasis).
“Mad as a hatter”: Blockade of central muscarinic receptors can cause anxiety, agitation, delirium, and hallucinations. (Hallucinations can actually make people seem larger or smaller, hence the Alice in Wonderland reference.)
Other side effects: Sinus tachycardia, decreased bowel sounds, and urinary retention
If you ever suspect child abuse what is the first thing you must do?
separate the child from the authorities and then contact the authorities
Gross and histologic characteristics of yolk sac tumor?
What is used both to mark the tumor in histologic sections and as a serum tumor marker for metastatic pure yolk-sac tumor or mixed non-seminomatous germ cell tumors?
Yolk-sac tumors produce a yellow, mucinous mass that histologically shows endodermal sinus formation and presence of hyaline (eosinophillic) droplets
α-Fetoprotein (AFP)
What are the two legal categories related to the failure to obtain informed consent?
battery action and negligent nondisclosure.
A battery action is an action for a “touching” to which the patient did not agree and is not considered to be a standard of medical care
Negligent nondisclosure is when a physician discovers that their original diagnosis is inaccurate but chooses not to tell the patient about the error and/or alter the treatment regimen
disease characterized by an extreme sensitivity to sunlight, skin changes, and a predisposition to malignancy?
inheritance pattern?
xeroderma pigmentosum (XP), an autosomal recessive
UV light induces formation of pyrimidine dimers between adjacent thymines in the DNA.
A defective excision repair mechanism for elimination of these dimers results in xeroderma pigmentosum
In a pt with hx of gout what would you give for an acute attack vs long term and mechanisms of each?
Acute: colchicine. Inhibition of leukocyte migration and phagocytosis
Long: Allopurinol and febuxostat are both xanthine oxidase inhibitors given within one week of an acute attack and used for 1-3 weeks until uric acid levels return to normal. However in acute situation may worsen symptoms
Chronic gout is managed by Probenecid and sulfinpyrazone: uricosuric agents that inhibit organic anion transporters (OATs) in the proximal tubule. OATs normally reabsorb urate from the renal tubule, so inhibiting them increases the urinary excretion of uric acid
They are not useful in acute attacks of gouty arthritis and are likely to worsen the severity of the existing attack Do not give to pt with hx of stones!**
Shoulder droop
Inability to shrug the shoulder
Inability to raise the arm above the horizontal plane
What muscle and what nerve palsy?
Trapezius muscle, caused by spinal accessory nerve damage
findings in hemoglobin electrophoresis in beta-thalassemia are low or absent hemoglobin A and elevated hemoglobin F and hemoglobin A2
inheritance?
beta-thalassemia (+), there is decreased or absent ability to synthesize beta-globin chains, which are necessary to make adequate amounts of hemoglobin A (α2β2), normally the predominant hemoglobin after 6 months of age
auto recessive
What is relative risk and how do you calculate it?
The relative risk (RR) is the comparative probability asking “How much more likely?” is something in one group compared to another
incidence rate of the exposed group divided by the incidence rate of the unexposed group
How is multidrug resistance spread between E. coli?
plasmids via conjugation
during summer months what are most common modes of transmission of viral encephalitis?
arthropods and mosuitos
Atypical lymphocytosis in the peripheral blood due to mononucleosis or EBV shows what type of cells?
Downey type II cells (CD8-positive cytotoxic cells)
what is transferrin? Where do you find it? what would cause increase or decrease in synthesis?
Transferrin transports iron to and from tissues.
Serum iron is bound to transferrin
Transferrin synthesis increases when ferritin is low (tissues need more iron). Transferrin synthesis decreases when ferritin is high (tissues need less iron).
Presentation: older woman with headache, muscle aches, jaw claudication, tenderness of the temples, and elevated ESR.
How do you dx this disease?
what’s the worst complication?
Suspect temporal arteritis
Diagnosis by biopsy of the temporal artery reveals granulomatous lesion with giant cells.
If left untreated, temporal arteritis can result in blindness.
renal cysts (many in an adult) and often present with hypertension (almost 100%), hematuria, proteinuria, or renal insufficiency
What is the disease? why do they have HTN? what is the treatment?
Polycystic kidney disease leads to increased renin secretion, thereby causing overproduction of angiotensin II.
An ACE inhibitor lowers blood pressure by blocking the conversion of angiotensin I to angiotensin II and also provides a renoprotective effect not matched by any other antihypertensive drugs
What changes to arterial and venous PO2 will be seen in an anemic pt? how about CO, 2,3BPG and RBC H+ concentration?
because the arterial O2 content (not concentration) is decreased, the extraction of normal amounts of O2 by the tissues will result in lower-than-normal mixed venous PO2. (Arterial PO2 is normal!)
all others will be increased
autosomal recessive disease in which there is an absence of CD18 beta-2 subunits (the common beta-2 chain) of integrin molecules?
Leukocyte adhesion deficiency (LAD)
The beta-2 integrins are the most important high affinity binding molecule on neutrophils because it binds to ICAM-1 on the surface of vascular endothelial cells to facilitate extravasation from the bloodstream into the tissues
These patients suffer recurrent, chronic bacterial infections.
The hallmark of this condition is an inability to form pus or abscesses. Also may see extreme neutrophilia (5-10 times above normal due to WBC inability to extravaste tissue)
Lynch syndrome aka HNPPC-inheritance pattern and mutation?
Auto dom
DNA MMR
What drug do you give in actue angle closure glaucoma
topical timolol (beta-blocker), apraclonidine (alpha-2 agonist), pilocarpine (muscarinic agonist) and prednisolone (corticosteroid) are generally used first to decrease IOP and then acetazolamide is administered when symptoms persist
Epiglottitis is most commonly caused by ?
Describe the vaccine.
Haemophilus influenzae type B (HiB).
capsular polysaccharide of the organism is covalently coupled to a protein carrier such as diphtheria toxoid-making it available to Tcells
How do you calculate forced vital capacity? (FVC)
Forced vital capacity can be calculated from a forced expiratory diagram as the difference between total lung capacity (TLC) and residual volume (RV).
highly aggressive malignancy commonly found in the long bones of young patients. Radiographically, there is concentric laminated (onion-skin appearance) periosteal reaction. What is it? what do you see histologically?
Ewing sarcoma-pain in the region of involvement, usually worse at night
“small blue cell tumors.”
How do you calculate maintenance dose?
MD = Cl x Cpss/F, where: MD = maintenance dose; Cl = clearance; Cpss= plasma concentration at steady-state; and F = drug bioavailability.
drugs administered IV are 100% bioavailable (F=1). (F becomes important when drugs are given orally.)
Reed-Sternberg cells and mononuclear RS variants are positive for CD?
CD30 and CD15.
CD15, CD30 positive RS cells are seen in the following types of Hodgkin disease: nodular sclerosis, mixed cellularity, lymphocyte rich, and lymphocyte depleted
The proximal tubule reabsorbs most of the filtered load of phosphate, primarily by what located in the apical membrane?
Parathyroid hormone decreases Pi reabsorption by down-regulating Na+-Pi transporter expression. What increases transporter expression and thereby increases Pi reabsorption.
Na+-Pi cotransporters
Calcitriol
With genital herpes, where does virus lay latent between infections?
sacral ganglion
How do you treat treat atypical “walking” pneumonia caused by M. pneumoniae?
Macrolides: inhibit the translocation of peptidyl tRNA from the acceptor site to the donor site of the 50S subunit.
Ulnar nerve lesions can produce:
Clawing of the 4th and 5th digits
Wasting of the hypothenar eminence and dorsal interosseous muscles
Loss of sensation to the medial side of the hand (both palm and dorsal surface) and both the palmar and dorsal surfaces of the 5th digit and the medial half of the 4th digit
Describe what a case series study entails and why is it done?
A case series study is a type of observational study that collects information from patients with a certain disease and does not include controls.
It provides objective information about a new disease process, so that appropriate treatment can be administered.
Best test to order for suspicion of recent alcohol use?
Serum gamma glutamyl transferase (GGT) is a biologic marker of alcohol consumption over an extended period of time.
GGT is elevated before liver function tests (ALT, AST, and alkaline phosphatase concentrations) are significantly altered.
GGT is useful as a screening test for previous alcohol intoxication.
leukocoria, strabismus
Microscopy reveals small, round, primitive-appearing blue cells; may have rosettes.
What is it and what chromosome is it associated with/mutation/inheritance?
Retinoblastoma:
Caused by mutation in Rb gene on chromosome 13q
Most common malignant eye tumor of childhood
In the familial form of the disease, a single Rb mutation in inherited from a parent, which by itself, causes no symptoms. However, a second acquired mutation of Rb in a retinal cell results in loss of the tumor suppressor protein (Knudson’s two-hit hypothesis)
in the sporadic form of retinoblastoma, two independent Rb mutational events must occur, which is a much rarer event
What happens to the efferent arteriole when the kidney intereprets hypotension?
decreased NaCl to macula densa–> increased renin –> increased ANGII –> constriction of efferent arteriole
Vascular tumors (hemangioblastomas) of the retina, cerebellum, and/or medulla
About half of affected patients can develop bilateral renal cell carcinomas.
What disease is it and what gene and chromosome is it associated with?
Von Hippel Lindau (VHL) disease:
Associated with a deletion of VHL gene on chromosome 3p
Expain what kind of metabolic syndrome (acidosis or alkalosis) can result from hyperaldosteronism? What are the major cells involved and where in the nephron are they?
hyperaldosteronism causes hypernatremia and hypokalemia by increasing acitivity of Na/K ATPase on basolateral membrane of principal cells in DCT and increasing expression of ENaC and ROMK. (Na pumped into interstitium favoring uptake of Na from tubule and wasting of K+).
the hypokalemia will induce increased expression of apical H+/K+ ATPase in alpha intercalated cells favoring reuptake of K+ and excretion of H+ resulting in metabolic alkalosis.
When do you Kimmelstiel-Wilson nodules?
Describe the histology?
Why do these pt’s get proteinuria?
Kimmelstiel-Wilson nodules are a classic finding in diabetic glomerulosclerosis.
They appear as ovoid, hyaline and PAS-positive structures found in the mesangial core at the edge of the glomerulus
Elevated blood glucose also results in diabetic glomerulonephropathy due to glycosylation of glomerular matrix proteins and hyaline arteriosclerosis of renal vasculature. Loss of negative charge in the basement membrane results in proteinuria as one of the initial changes
Alcoholic with hep C going through withdrawal, what do you give for acute symptoms like nausea vomiting tremors?
Benzodiazepines such as diazepam, chlordiazepoxide, and lorazepam are used in alcohol withdrawal.
**Lorazepam, oxazepam, and temazepam are benzodiazepines that are preferred in **liver disease in general because they are metabolized by phase II processes, which are less affected by liver disease. Lorazepam and oxazepam are more likely to be used for alcohol withdrawal
Screening for HIV what is the order of tests
ELISA, Confirmatory test: antibody immunoassays for HIV1 and HIV2 antibodies.
When antibody immunoassay results are negative or inconclusive in conjunction with positive screening test results (ELISA), a RT-PCR must be performed.
Left-sided sensory neglect is typically caused by a lesion in the??
right parietal or frontal lobe (bc pt usually right handed)
All RNA viruses replicate in cytoplasm, except
Orthomyxoviruses and Retroviruses that have replicative stages in nuclei.
All DNA viruses require a nucleus,
except Poxviruses that can replicate in the cytoplasm.
Chlamydia trach: serotypes with sympoms above the waist and below? What is the elementary vs. reticulate body? Stain? Tx?
A-C above D-K below.
Elementary infections (inactive) enters cell replicated in endosome forming reticulate body that reorganizes into elementary and cell bursts
Stain with Geimsa or iodine
Tx azithromycin or doxy, add ceftriaxone for gonorrhea. neonate gets oral erythromycin for conjunctivitis or pneumonia
What are Krukenberg tumors?
gastric mucinous adenocarcinomas that have metastasized to the ovary.
Note usually both ovaries are involved compared to a primary ovarian cancer which would more likely be unilateral
What buzz word/cell is associated with this histo description?
cytoplasmic mucin droplet pushes aside the nucleus of an adenocarcinoma cell
Signet ring cell
inferior parathyroid glands and the thymus arise from which pouch?
superior parathyroid glands?
What syndrome is associated with abnormalities in both pouches listed above?
third pharyngeal pouch
fourth
DiGeorge=abnormal neural crest cell migration. presents with immunologic problems, hypocalcemia, cardiovascular defects, abnormal ears, and micrognathia.
most common inherited cause of mental retardation (hint due to chromosomal breakage)
Fragile X. X- linked Dominant
caused by expansion of CGG trinucleotide repeat in the fragile X mental retardation 1 (FMR1) gene, which leads to altered transcription of the gene.
When considering adding a medication to a pt who is taking Theophylline, what do you have to consider and avoid?
Theophylline extensively metabolized by CYP1A2 and 3A4 and has a very narrow therapeutic index so be care not to prescribe any drugs that are CYP inhibitors.
Sx’s of theophylline toxicity include nausea, vomiting, insomnia, irritability and transient diuresis. More serious reactions include seizures, arrhythmias, hypotensionand shock
Mycophenolate mofetil MOA and indication?
immunosuppressant agent that inhibits purine synthesis. It inhibits inosine monophosphate dehydrogenase
indicated for organ rejection prophylaxis in patients receiving allogeneic renal, cardiac, or hepatic transplants when used in conjunction with cyclosporine and a corticosteroid, such as prednisone.
Type I-V collagen, where do you find them and what are associated diseases?
Type I collagen: tensile strength and is found in skin, bone, tendons, and most other organs. Osteogenesis imperfecta (OI)- bones are brittle and fragile, and sclera have a blue discoloration.
Type III collagen in blood vessels, uterus, and skin. Ehlers-Danlos syndrome, vascular types 3 and 4, are example of collagen type III disorders.
Type IV collagen: basement membrane. Disorders that affect type IV collagen include Alport syndrome and Goodpasture syndrome.
Type V collagen is a minor component of interstitial tissues and blood vessels. Classic Ehlers-Danlos syndrome types 1 and 2 are collagen type V disorders.
clinical presentation of fever, chills, and fatigue following an outdoor hiking trip; identification of small rings in red blood cells. What what bug causes this?
How is it transmitted?
Babesia microti
Ixodes scapularis tick
Holoprosencephaly, cyclopia, cleft palate/lip, and proboscis
Polydactyly, rocker-bottom feet, congenital heart defects, and renal defects
Sonic hedgehog (SHH) gene mutation can cause loss of ventral midline development, resulting in holoprosencephaly
Patau syndrome trisomy 13
Most often, DS is due to a numerical alteration of chromosome 21, with lesser percentage due to what?
of structural alterations (Robertsonian translocation t14,21).
Pt’s with goodpasture syndrome may also suffer what other pulmonary sequelae?
Pulmonary Hemorrhage
Patients at risk for retinoblastoma are also at risk for what other disease?
Osteosarcome.
Rb gene chromosome 13