uWorld 8 Flashcards
what is the pathogenies of pulmonary hypertension due to left sided heart disease
passive increase in pulmonary capillary and arterial pressure secondary to PULMONARY VENOUS CONGESTION
this leads to decreased production of NITRIC OXIDE (vasodilator) and increased production of ENDOTHELIN (vasoconstrictor)
over time, remodeling of pulmonary vasculature occurs with increased smooth muscle cell proliferation (medial hypertrophy) and collagen and elastase deposition (intimal thickening and fibrosis)
remodeling is less than idiopathic pulm hypertension so its partially reversible
teenage boy who has to stop playing sports b/c of progressively worsening symptoms found to have kyphoscoliosis and high plantar arch. bother had neurologic disorder and died of heart failure at 25. whats he got
Fredreich Ataxia (AR)
caused by mutation in frataxin (FXN) gene that encodes for an essential mitochondria protein involved in the assembly of iron sulfur enzymes
GAA trinucleotide repeats on muted gene cousin decreased FXN expression
degeneration of the spinocerebellar tracts and lateral corticospinal tract (gait ataxia and spastic muscle weakness)
loss of position and vibration
hypertrophic cardiomyopathy and CHF
high arches (pes cavus)
kyphoscoliosis
diabetes mellitus
gait ataxia and spastic muscle weakness
what is seen in Charcot-Marie-Tooth disease
AD disorder associated with demyelination of peripheral nerves
distal leg weakness/atrophy with sensory deficits
pes Casus
kyphoscoliosis
how can confounding effects be negated
running separate analyses for the smokers and non-smokers (or whatever the group is)- a technique called STRATIFIED ANALYSIS
in case-control studies what are the two groups
cases- ppl with disease
control- ppl without disease
* DOES NOT CHANGE DUE TO EXPOSURE OR ANYTHING ITS SIMPLY PPL W DISEASE AND PPL W/O DISEASE*** dont fuck this up
what medication can be used to treat simple and complex (loss of memory during event and postictal state) PARTIAL seizures (this means only one part of body is affected)
carbamazepine (also used for generalized tonic-clonic seizures, bipolar, trigeminal neuralgia)
blocks voltage gated Na channels in cortical neurons- stabilizes channels in an inactivated state
ADRs: marrow suppression, SIADH, hepatotoxic
what is the Pringle maneuver
occlusion of the portal triad- surgical techniques used to distinguish the source of right upper quadrant bleeding
if bleeding persists after occlusion of portal train the IVC or hepatic veins likely to be injured
what is permissiveness
when one hormone allows another to exert its maximal effect
cortisol increases alpha adrenergic receptors allowing NE to have increased vascular responsiveness
in adrenal insufficiency how can LOW glucocorticoid levels contribute to hypotensive crisis
decrease vascular responsiveness to angiotensin II and norepinephrine
what is tachyphylaxis
decreased drug responsiveness in a short period following one or more dose (rapidly developing tolerance)
what do homeobox genes do
transcription regulators- code for TRANSCRIPTION FACTORS that bind to regulatory regions on DNA, altering expression of genes involved in the segmental organization of the embryo
ensures correct positions along CRANIO-CAUDAL axis
teenager w/ progressive skin rash for past year, long standing history of turing sensation in pals and soles exacerbated by stress and fatigue (severe after exercise- which he sweats minimally). clusters of non-blanching, red papule in the gluteal, inguinal, and umbilical areas. undetectable levels of alpha-galactosidase A
FABRY DISEASE (X-LINKED recessive) alpha-galactosidase A deficiency results in accumulation of globotriaosylceramide (Gb3 or ceramide trihexoside) neuropathic pain hypohydrosis angiokeratomas telangiectasis glomerular disease (proteinuria and polyuria from buildup of Gb3 in glomerulus and distal tubule) renal failure cerebrovascular disease (TIA, stroke) cardiac disease (LVH)
what are hypocretin 1 and 2
aka orexin A and B
produced only in neurons of the LATERAL HYPOTHALAMUS
function to promote wakefulness and inhibit REM sleep-teated phenomena
only detectable in CSF
CSF concentrations of homovanillic acid are also decreased in patients with waht
parkinsons
what causes hypocalcemia in patients who just received packed red blood cells as part of a resuscitation effort
infused citrate from the packed cells can chelate serum calcium
prior to storage, whole blood (for transfusions) is generally mixed with solutions containing citrate anticoagulant- packed cells derived from these blood collections also contain citrate
most likely to occur following MASSIVE TRANSFUSIONS of more than 5 or 6 liters of blood (total body blood volume) over 24 hours
what is NEPRILYSIN
a metalloprotease that INACTIVATES several peptide hormones: bradykinin, glucagon, enkephalins, and natriuretic peptides (ANP, BNP)
what are ANPs affects in the kidney, adrenal gland, blood vessels
kidney: dilates afferent arterioles, increases GFR and urinary excretion of sodium and water, limits sodium reabsorption and inhibits renin secretion
adrenal: restricts aldosterone secretion
blood vessel: relaxes vascular smooth muscle in arterioles and venules
what is sacubitril
neprilysin inhibit that prevents degradation of ANP, enhancing its beneficial hemodynamic effects in heart failure patients
what would acutely cause an increase in cardiac output and an increased venous return thus shifting both curves and causing an increased mean systemic pressure
DECEASED TPR due to AV fistula or exercise
what type of cartilage is found in scars, where else is it found
type 1 cartilage
bone, tendons, dermis, ligaments, dentin, cornea, blood vessels
PECAM-1 is involved in what part of leukocyte adhesion cascade
transmigration
p-selectin/e-selectin on endothelial cells and L-selectin on neutrophils are involved in what part of leukocyte adhesion cascade
rolling (LAD 2 happens here: impaired fucosylation of sialylated carbohydrate ligands prevents selection binding)
loose binding of leukocyte to endothelium via sialylated carbohydrate groups such as Sailyl Lewis X or PSGL-1, to L-selectin/P-selectin/E-selectin
cytokine stimulation greatly increases expression of the endothelial selections
tight adhesion and crawling of leukocyte adhesion is due to what
neutrophil become firmly attached to endothelium via binding of CD18 BETA 2 INTEGRINS (LFA-1, Mac01) to intercellular adhesion molecule-1 (ICAM-1) on endothelial cells
what is LAD type 3
similar to type 1 and causes severe recurrent bacterial infections, delayed separation of the umbilical cord, and bleeding complications
due to affected BETA-3 INTEGRINS on PLATELETS
what is LAD type 2
milder condition with no delay in separation of the umbilical cord and less severe and fewer infections
Swan-Ganz catheters traverse what artery
pulmonary artery
where is the left ventricular lead in biventricular pacemakers
courses through coronary sinus which resides int eh atrioventricular groove on the posterior aspect of the heart
what causes persistent central diabetes insipidus (what can cause transient)
loss of vasopressinergic neurons in the PARAVENTRICULAR and SUPRAOPTIC nuclei of the HYPOTHALAMUS due to trauma, surgery, infiltrative disease (histocytosis X) where the ADH is made will lead to permanent DI
damage to the posterior pituitary (where ADH is stored for release) or the infundibulum can cause transient DI (but the hypothalamic nuclei being intact, axonal regeneration and hypertrophy will allow adequate ADH release into circulation)
the genetic instability of hepatitis C is due to what
lack of 3’ to 5’ exonuclease activity in the viral RNA polymerase
also has hypervariable genomic regions, like those that case forts 2 envelope glycoproteins
sickling in sickle cell is promoted by what
conditions associated with low oxygen levels, increased acidy, or low blood volume (dehydration)
oxygen unloading in organs that have high metabolic demands (brain, muscles, placenta) promotes sickling
what is the pathogenesis of pulmonary hypertension in COPD patients
hypoxia-induced vasoconstriction and emphysematous obliteration of the vasculature
a large VSD will cause pulmonary hypertension due to what
pulmonary hypertension secondary to early pulmonary remodeling from an increase in pulmonary artery flow volume
effect modification results when?
results when external variable positively or negatively impacts the observed effect of a risk factor on disease status
when this occurs, stratified analysis will reveal a significant difference in risk b/w the stratified groups
hepatomegaly occurs in what lysosomal storage diseases
Niemann-Pick
Hunter
Hurler
optic atrophy, developmental regression, and seizures occur in what lysosomal storage disease
Krabbe
glactocerebrosidase deficiency
progressive demyelination leading to ataxia, peripheral neuropathy, seizures, and hypotonia are seen in what lysosomal storage disease
Metachromatic leukodytophy
arylsulfatase A deficiency
accumulation of cerebroside sulfate
excessive perspiration does what to acne
exacerbates (does not cause)
what happens to potassium in stored red blood cells
stored red cells gradually lose intracellular potassium to surrounding solution- potential to cause hyperkalemia in pt
nucleolus pulposus has what kind of collagen
type II
so does cartilage
granulation tissue has what type of collagen
type III (seen about 7 days after MI- replaced by type I as scar forms)
DAN glycosylase is important for what
base excision repair as it removes the nitrogen vase for the sugar-phosphate backbone
an endonuclease then competes the remainder of the repair
what is DAN primase
form of RNA polymerase
in bacteria it binds with DNA helices and synthesizes a short RNA primer to which nucleotides can be added by DNA polymerase
what is fibromyalgia
chronic disorder characterized by WIDESPREAD MUSCULOSKELETAL PAIN in an association with FATIGUE and neuropsychiatric disturbances (paresthesias, poor sleep, depression, difficulty concentrating)
common in women 20-55
tenderness at characteristic locations in the soft tissues and at bony prominence
how is fibromyalgia diagnosed
pts with chronic pain and fatigue for more than 3 months in the absence of physical or laboratory findings suggestive of an inflammatory etiology (synovial swelling, elevated ESR or c-reacive protein) or other chronic pain syndrome
the etiology of fibromyalgia is probably what and how can you improve function
abnormal central processing of painful stimuli
GRADUAL INCREMENAL AEROBIC EXERCISE is proven to reduce pain an improve function
what is polymyalgia rheumatica
inflammatory disorder that affects pts over 50 and causes subacute pain and stiffness in shoulders and hips, weight loss, fever, and malaise
patients with severe aortic stenosis and left concentric left ventricular hypertrophy, atrial contraction contributes significantly to left ventricular filling, they can get what kind of arrhythmia and what can happen b/c of this
atrial fibrillation can cause loss of atrial contraction-
sudden decrease in left ventricular preload and cardiac output sufficiently to cause systemic hypotension
decreased forward filling can lead to pulmonary edema
pt with worsening fatigue and no other medical problems with unremarkable physical but ALP is increased to 180, what should be checked next
gamma-glutamyl transpeptidase (GGT)
enzyme predominantly present in hepatocytes and biliary epithelia
BOTTOM LINE: moderate alk phos of unclear etiology should always be followed by GGT
chronic rheumatoid arthritis can involve the cervical spine, destruction of what joint occurs and what does this cause
ALANTOAXIAL JOINT is often involved and more prone to SUBLUXATION (vertebral malalignment) as the atlas (C1) has a high degree of mobility relative to the axis (C2 odontoid and body) with limited intrinsic bony stability
laxity/destruction of the transverse ligaments or progressive erosion or fracture of the odontoid process can increase anterior movement of the atlas and cause spinal cord compression due to posterior displacement of the odontoid
what are the symptoms of alantoaxial instability with subluxation (as can be seen in chronic rheumatoid arthritis)
neck pain
stiffness
neurological findings (radicular spin
endotracheal indication with extension of the neck can worsen with subluxation with possible acute compression of the spinal cord and/or vertebral arteries
pts can develop paralysis with decreased or absent reflexes below the level of compression (spinal shock), hypotension due to loss of sympathetic tone, and/or sudden death
cereal septic emboli are usually due to what
infective endocarditis of the left-sided heart valves
patients can develop neurologic findings with paralysis but do not usually have acute-onset quadriparesis
what is DRESS syndrome
DRUG REACTION with EOSINOPHILIA and SYSTEMIC SYMPTOMS
rare and potentially life-threatening drug reaction typically occurring 2-8 weeks after drug exposure
commonly associated with ANTICONVULSANTS, ALLOPURINOL, sulfonamides, antibiotics
FEVER, GENERALIZED LYMPHADENOPATHY, FACIAL EDEMA, and diffuse moriblliform skin rash that can progress to a confluent erythema with follicular accentuation
hepatomegaly, jaundice
acute interstitial nephritis
cough, dyspnea
what lab findings are seen in DRESS syndrome
EOSINOPHILA
typical lymphocytes
elevates serum alanine transaminase
what is cryoglobulinemia
small- to medium- vessel vasculitis caused by circulating immunoglobulin-complement complexes that precipitate on refrigeration
typically associated with chronic inflammatory states (SLE, hepC) and can present with systemic findings (fatigue, arthralgia, myalgias) and palpable purpura in the lower extremities due to cutaneous vasculitis
drug-induced antineutrophil cytoplasmic antibodies-associated vasculitis is most commonly linked to what meidcations
hyperthyroid (propylthiouracil, methimazole)
hydrazine
patients can develop constitutional symptom, arthralgia/arthritis, and cutaneous vasculitis
fever, sore throat, ulceration, or other signs of infection in a pt with hyperthyroidism (Graves, etc) treated with medical therapy should raise concern for what
THIONAMIDE-INDUCED AGRANULOCYTOSIS
check white blood cell count with differential
aspirin and ibuprofen should be avoided in hyperthyroidism why
they displace thyroid hormone
what is displacement
emotions are unconsciously transferred rom their actual target to someone or something that is less threatening
what spinal nerve is unique in that it only carries muscle fibers
C1
what is pituitary apoplexy
due to sudden hemorrhage into the pituitary, usual in setting of preexisting adenoma
acute severe headache, ophthalmoplegia, and altered sensorium
what is lymphocytic hypophysitis
most common inflammatory contain of the pituitary and typically occurs during late pregnancy or the early postpartum period
acute severe headache and visual field defects
structures arising from neural crest cells can be summarized by the pneumonic MOTEL PASS what does that stand for
Melanocytes Odontoblasts Tracheal cartilage Enterochromaffin cells Laryngeal cartilage Parafollicular cells of the thyroid Adrenal medulla and all ganglia Schwann cells Spiral membrane
what is seen in MEN1
primary hyperparathyroidism (hypercalemia) pituitary tumors (prolactin, visual defects) pancreatic tumors (especially gastriomas)
menin a tumor suppression on chromosome 11
what is seen in MEN2A
medullary thyroid cancer
pheochromocytoma (hypertension, flushing, diaphoresis, headaches)
parathyroid hyperplasia
RET gene on chromosome 10
what is seen in MEN2B
medullary thyroid cancer
pheochromocytoma (hypertension, flushing, diaphoresis, headaches)
mucosal neuromas/marfanoid habitus
RET gene on chromosome 10
