uWorld 14 Flashcards
what secondary changes can be seen in minimal change disease (and other nephrotic syndromes) that compensate for decreased plasma albumin concentration
liver increases synthesis of proteins, including LIPOPROTEINS (which is followed by lipiduria)
what happen to plasma aldosterone levels in nephrotic syndrome
increase (and thus sodium and water retention happen)
what is the most important source of protection in an Influenza A vaccine
humoral response- antibodies against hemagglutinin
can neutralize the virus and primarily block its binding to the host cells
what are the classical physical features of fragile X
mental retardation (MCC of inherited intellectual disability) macrocephaly, long narrow face, prominent forehead, jaw, chin ears, machroorchidism
due to CGG repeat in the fragile X mental retardation 1 (FMR1) gene on the long arm of ch X
what is the structure of fetal hemoglobin
alpha2gamma2
what is given to patients with aspirin allergies to prevent cardiovascular events
clopidogrel (irreversilby blocks P2Y12 component of ADP receptors on platelet surface)
what is cilostazol
PDE inhibitor that is occasionally used in pts with symptomatic peripheral vascular disease (claudication)
what is eptifibatide
platelet glycoprotein IIb/IIIa inhibitor that inhibits the final common pathway of lately aggregation
occasionally used in some patients with acute coronary syndrome, but it is not used for patients with stable CHD
what drugs can induce parkinsonism
antipsychotics (first generation more than second)
antiemetics/gastric motility agents (prochlorperazine, metoclopramide)
verapamil can be used for its vasodilatory properties and also affects cardiac contractility but has minimal effect on skeletal muscle. Which of the following properties of skeletal muscle is responsible for this resistance of the medication?
No dependence on extracellular calcium influx
cardiac myocytes depolarization causes L-type calcium channels on the plasma membrane to open and allow influx of extracellular calcium- this then ind to and activates RyR2 channels inducing release of calcium from the SR (CALCIUM-INDUCED CALCIUM RELEASE)
-smooth muscle is similar however calcium-calmodulin rather than calcium-troponin (like in cardiac) complex facilitates the interaction b.w actin and myosin
in skeletal muscle L-type calcium channels directly interact with RyR1 calcium channels to cause release of calcium from SR- there is NO significant influx of calcium across the plasma membrane L-type channel- PHYSICAL INTERACTION b/w the 2 channels that triggers sarcoplasmic calcium release
an elaborate T-tuble system is found in what types of muscle
skeletal and cardiac muscle but NOT smooth
T-tubles are a communication of the extracellular space that permits a more paid spread of depolarization throughout skeletal and cardiac muscles
what muscle cells depend on extracellular calcium influx into the cells via L-type calcium channels for excitation-contraction coupling
cardiac and smooth muscle NOT skeletal
why is skeletal muscle resistant to the effect of calcium channel blockers
does not require an initial influx of extracellular calcium for excitation-contraction coupling
pulmonary capillary wedge pressure is a measure of what and an isolated increase in it represents what
left atrial pressure and left end-diastolic pressure
increase in PCWP can represent MITRAL STENOSIS from increase in left atrial pressure that is reflected to the pulmonary veins
where is majority of water absorbed in the kidneys no matter what the hydration status is
PROXIMAL TUBULES REABSORB over 60% of water filtered by the glomeruli
how is the release of prolactin controlled
inhibitory control by dopamine secretion from the HYPOTHALAMUS
disruption of dopaminergic pathways or blockade of dopamine D2 receptors can cause hyperprolactinemia
what two branches of the external iliac artery branch before the inguinal ligament
INFERIOR EPIGASTRIC ARTERY
deep circumflex iliac
what is pantoprazole
proton pump inhibitor
used to great GERD
what is seen histologically in GERD
basal zone hyperplasia
lamina propria papillae
scattered eosinophilia and neutrophils
NOCTURNAL COUGH
eosinophilic esophagitis
an immune/antigen-mediated disease that typically presents with dysphagia and good impaction in atopic adults
characterized histologically by NUMEROUS superficially located intraepithelial eosinophils, which and hep differentiate it from reflux esophagitis
PPIs usually doe not work
what causes pill-induced esophagitis
tetracylcine antibiotics
potassium chloride
bisphosphonates
what is the most common malignant brain tumor of childhood
medulloblastoma (typically arises in cerebellum)
patients with what are at increased risk of developing ALL
Down syndrome
ataxia-telangiectasia
NF1
because the pentose phosphate pathways is the MAIN SOURCE of NADPH, the pathway is particularly active in:
cells experiencing OXIDATIVE STRESS (erythrocytes), where NADPH is used to REGENERATE GLUTATHIONE
organs (liver, adrenals) involved in REDUCTIVE BIOSYNTHESIS (FATTY ACIDS, CHOLESTEROL, STEROIDS) and cytochrome P450 metabolism
phagocytic cells generating a respiratory burst (NADPH oxidase)
an increase in mean corpuscular hemoglobin concentration (MCHC) and loss of central pallor is seen in what disease
hereditary spherocytosis
acquired spherocytosis- MCC is autoimmune hemolytic anemia
what is haptoglobin
acute phase reactant that combines with free hemoglobin in the citation to preserve body iron stores and prevent tissue damage
haptoglobin levels decrease in any form of hemolytic anemia (even with extravascular hemolysis, as some free hemoglobin will spill into the blood stream)
what are the skeletal findings in hyperparathyroidism
most commonly involve the CORTICAL (COMPACT) BONE) in the appendicular skeleton (the pectoral girdle, pelvic girdle, and limbs)
SUBPERIOSTEAL THINNING is a characteristic feature and appears radiologically as MUBPEROSTEAL EROSIONS in flanges of the hand, and a granular “SALT AND PEPPER” SKULL, and osteolytic cysts in the long bones (OSTEITIS FIBROSIS CYSTICA)
trabecular thinning with fewer interconnections is characteristic of what bone disease
OSTEOPOROSIS
osteoid matrix accumulation around trabeulae is seen in what bone disease
vitamin D DEFICIECNY
also see excessive un-mineralized osteoid with widened osteoid seams
patients typically have low urine calcium
persistence of the primary spongiosa in the medullary cavity with no mature trabeculae is a classic finding int hat
OSTEOPETROSIS (“marble bone disease”)
caused by decreased osteoclastic bone resorption, which resulting in accumulation of woven bone and diffuse skeletal thickening
what is the most common skeletal manifestation of primary hyperparathyroidism
OSTEITIS FIBROSIS CYSITCA
bone pain, subperiosteal erosions affecting the phalanges of the hand, and a “salt and pepper” skull, and BROWN TUMOR bone CYSTS
intimal streaks are the earlier lesion of what
atherosclerosis
aortic regurgitation in the presence of mediastinal widening suggests what
thoracic aortic aneurysm
what ANTIOXIDANT ENZYMES convert ROS to oxygen and water, neutralizing their capacity for cellular damage
SUERPOXIDE DISMUTASE
GLUTATHIONE PEROXIDASE
CATALASE
what is myeloperoxidase
found in neutrophils
converts hydrogen peroxide to hypochlorous acid (a bactericidal compound that also causes oxidative damage to host cells)
what is cytochrome oxidase
final component of the ETC and functions to cover molecular oxygen to water while establishing the proton gradient necessary for synthesis of ATP
what is the inheritance of PKU
AR
GPIIb/IIIa is either deficient or defective in patients with
Glanzmann thrombasthenia
what is seen on EM and immunoflourescen of PSGN
her cellular glomeruli fue to leukocyte infiltration (neutrophils and monocytes) and mesangial and endothelial cell proliferation
granular deposits of IgG, IgM, and C3
SUBEPITHELIAL HUMPS on EM
sub endothelial C1q deposits are characteristic of what renal disease
type 1 membranoproliferative GN
IgE deposits are sometimes seen in what renal disease
lupus nephritis
confined to the capillary wall
associated with a poorer prognosis
what are the clinical features of restless leg syndrome
uncomfortable urge to move the legs:
unpleasant sensation in the legs
onset with inactivity or at NIGHT
RELEIF with MOVEMENT (walking, stretching)
what are the causes of restless leg syndrome
idiopathic IRON DEFICIENCY uremia diabetes (esp w/ neuropathy) multiple sclerosis, Parkinson disease Drugs (antidepressants, metoclopramide)
what is the treatment of restless leg syndrome
avoidance of aggravating facts (alcohol, sleep deprivation) supportive measures: leg massage, exercise, heating pads DOPAMINE AGONISTS (PRAMIPEXOLE)
what drug is used to treat restless leg syndrome
PRAMIPEXOLE (dopamine agonist) or ropinirole
patients who have overdosed on beta blockers should be treated with what
glucagon (increases heart rate and contractility independent of adrenergic receptors)
what is Osgod-Schlatter disease (OSD)
an overuse injury of the SECONDARY OSSIFICATION CENTER (APOPHYSIS) of the TIBIAL TUBERCLE
very common cause of knee pain in young ADOLESCENTATHLETES after a recent growth spurt
presents as in and selling at the TIBIAL TUBERCLE, the insertion of the PATELLAR LIGAMENT (connects the tibia to the patella)
REPETATIVE QUADRICEPTS CONTRACTION (jumping) and CHRONIC AVULSION cause the proximal patellar tendon to separate fro the TIBIAL TUBERCLE
what does the COPPER REDUCTION TEST detect
presence of a REDUCING SUGAR (like FRUCTOSE, glucose, and galactose)
urine dipstick only positive if glucose present (not galactose or fructose)
a deficiency in acid alpha-glucosidase (aka acid maltase) causes what
glycogen storage disease type II (POMPE DISEASE)
affected infants have cardiomyopathy, muscle weakness, and hypotonia
what is essential in making the diagnosis of THROMBOTIC THROMBOCYTOPENIA PURPURA
microangiopathic HEMOLYTIC ANEMIA (↑ LDH, ↓ haptoglobin) with SCHISTOCYTES
THROMBOCYTOPENIA (↑ bleeding time, normal PT/PTT)
Tx: PLASMA EXCHANGE if life-saving and
in pregnancy DIC is mediated by what
TISSUE FACTOR (THOMBOPLASTIN)
what is seen on light microscopy of craniopharyngioma
cysts lined by cords/nests of stratified squamous epithelium with peripheral palisading and internal areas of lamellar “WET” KERATIN
what is seen in a cranipharyngioma
cystic or partially cystic with solid areas
cysts are filled with a brownish-yellow, viscous fluid that resembles machine oil due to the presence f protein and CHOLESTEROL CRYSTALS
DYSTROPHIC CALCIFICATION of the cysts is highly characteristic and may be detected on neuroimaging
