Test 85 Flashcards
RVH
Cor pulmonale
caused by pulmonary HTN seen in F 20-40
Essential HTN and aortic stenosis cause what type of hypertrophy
LEFT VH
Pica
compulsive consumption of nonfood
common in pregnancy
usually ice
CGD inheritance
X linked mutation that affects NADPH oxidase
reccurent bacterial and fungal infections
dx CGD
ABSENCE of normal BLUE and fluroscent GREEN pigment produced by NITROBLUE TETRAZOLIUM test and DIHYRORHODAMINE flow cytometry
Nitroblue tetrazolium testing
Add NBT to sample of pt’s neutrophils
properly functioning neutrophils produce ROS that can REDUCE the yellow NBT to DARK BLUE that preceipitates in cells
Dihydrorhodamine
assesses production of superoxide radicals by measuring conversion of DHR to RHOdamine which shoiuld come out fluorescent green
ADA def
AR
SECOND MC cause of SCID
PROFOUND decrease in B and T cells
mutlisubunit complex that acts as terminal enzyme of mitochondrial respiratory chain
Cyt C oxidase
MPO
Enzyme found in neutrophil azurophilic granules
catalyzes production of BLEACH from hydrogen peroxide and chlorine
Tx HIGH LDL
Hepatitis
myopathy
Statins
HMG CoA reductase
Tx LOW HDL Cutaneous vasodilation (Red flushed face) hyperglycemia (acanthosis nigricins) Hyperuricemia/ GOUT Hepatitis
Niacin
INHIBITS lipolysis in adipose
REDUCES hepatic VLDL synthesis
Tx HIGH TG
Gallstones
Myopathy (WORSE w/ Statins)
Fibrates (gemfibrozil, and ‘brates)
Upregulate LPL> increase TG clearance
Tx high LDL
Gi upset
hypertriglyceridemia
malabsorption
cholestyramine, colestipol, coleselvam
prevent intestinal esorption of bile acids, so liver must use choleseterol to make more
MO of Nitrate drugs
Mimic actions of NO
transformed to NO at SM membrane> INCREASED cGMP> DECREASEd intracellular Ca and myosin dephosphorylation
Receptor for insulin and ILGF
TK
leads to TRANSIENT recurrent pulmonary infiltrates and eventual proximal BRONCHIECTASIS that can complicate asthma
Aspergillus fumigatus
*often occurs in STEROID DEPENDENT asthmatics
pulmonary pathogen in pts w/ CF or neutropenia
p. aeruginosa
Colchicine
inhibits LEUKOCYTE migration and phagocytosis by blocking TUBULIN polymeratization
TX for acute gouty arthritis in ELDERLY pts or those w/ renal failure by INHIBITING phospholipiase A2
Glucocorticoids
Zieluton
blocks 5 lipoxygenase and inhibits leukotriene formation
Why does urinary pH DECREASE in metabolic acidosis?
Bicar is combletely resorbed from teh tubular fluid
to excrete H you need to the two most important renal buffers (NH3 and H2PO4)
CEREBELLAR HEMANGIOBLASTOMAS
CYSTS/CANCER in kidney, liver, pancrease
incrased risk for RCC (can be bilateral)
VHL disease
Neurofibromas
optic nerve gliomas
Lisch nodules
cafe au lait spots
NF1
AKA Von Recklinghaunsen’s disease
bilateral cranial nerve II SCHWANNOMAS
multiple MENINGIOMAS
NF 2
cutaneous FACIAL ANGIOMAS and LEPTOMENINGEAL ANGIOMAS
usually inolved V1 and V2 distribution of trigemnial nerve
TRAM TRACK calcifications on skull
Sturge Weber syndrome
think about weber test being on the head….
AD syndrome
cutaneous ANGIOFIBROMAS
HAMARTOMAS
CARDIAC RHABDOMYOMAS
RENAL ANIGIOMYOLIPOMAS
….seizures are major complication
Tuberous sclerosis
AD inhertiance of CONGENITAL TELANGIECTASIAS
osler weber rendu syndrome
rupture> epistaxis, GI bleeding, hematuria
Increase PT and PTT but DO NOT affect thrombin time
Direct factor XA inhibitors (apixaban, rivaroxaban)
Direct thrombin inhibotrs (dabigatran, bivalirudin, dabigatran)
prevent formation of THROMBIN (factor IIa) and lead to prolongation of aPTT, PT and TT
binds to ANTITHROMBINa nd causes inactivation of SEVERAL coag factors (THRoMBIN and FACTOR X)
Both PTT and TT are prolonged
Unfractionated heparin
Brain tumors in a child
Please take me to….
Animal kindgdom
Magic kingdom
Epcot
i’m dying of a brain tumor
pilocystic astrocytoma
medulloblastoma
ependyoma
craniopharyngioma
Cerebellar tumor in a child
Pilocytic astrocytoma (cystic and solid components)
Meduloblastomma (always solid)
MC brain tumor in child
arise from astrocytes
ROSENTHAL FIBERS
pilocytic astrocytomas
Second MC brain tumor of childhood
solid
composed of sheets of SMALL BLUE cells w/ hyperchromatic nuclei
malignant w/ poor prog
HOME WRIGHT ROSEETESS
Medulooblastoma
third MC brain tuor in children
Ependymal lining of 4th ventricle
obstruct CSF
hydrocephalus
PERIVASCULAR ROSSETES
EPENDYOMA
MC SUPRATENTORIAL TUMOR IN CHILDHOOD
causes bitemporal hemianopia
craniopharyngioma
multiple ring enhancing lesions w/ mass effect in pt w/ HIV
toxoplasmosis
tx for toxoplasmosis
pyrimethamine
sulfadizazine
cryptococcocal meningitis tx
amphotericin
listeria tx
ampicillin
CMV tx
gnciclovir
second MC cause of ring enhancing lesions w/ mass effect in pt w/ HIV
primary central nervous system lymphoma
B cell ORIGION d/t EBV infection
Nephrotic syndrome
INCREASED permeability of glomerular capillary wall
MASSIVE urine protein loss> decrase in serum albumin > DECREASE In OSMOTIC PRESSURE> EDEMA
SHIFT of water into interstitium> RAAS> Na nad Water retention
INCREASED liver lipoprotein syntehsis
Cyp450 INducers
Griseolfulvin Carbamezabine Phenobarbital (BARBS) Phenytoin Barbs) Rifampin St. JoHns Wort
CYP 450 inhibiotors
CImetidine Ritonavir Amiodarone Cipro Ketoconoazole Acute ETOH use Macrolides Isoniazid Grapefruit Juice Omeprazole Sulfonamides
What leads to a low Vd (3-5L)?
HIGH MW
HIGH PPB
HIGH charge
hydrophilicity
Gastrojejunostomy
IDA
Diagnostic for B12 def
Increased methylmalonic Acid
decreased in thiamine deficiency
RBC transketaolase levels
PE…
HYPOXEMIA
Respiratory alkalosis
ANG I to ANG II in HF
HF stimulates SNS and the RAAS to maintain effective intravascular volume
Ang I is converted to ANG II by ACE enzyme in the lungs
thunder clap HA seen in pt w/ ADPKD
Intracrnaial berry aneuryms in circle of WILLIS> subarachnoid hemorrhage
virus that can mimic RA but is usually self resolving
Parvo B 19
Congenital torticolis
noteted by 204 wks of age
child prefers to hold head tilted to one side
result of malposition of the head in utero or BIRTH TRAUMA
pathognomonic for acute pyelonephritis
WBC casts in tububules
MC cardiac abnormality that predisposes you to NVBE
mitral valve prolapse
Retroperitoneal hematoma in a stable pt
pancreatic injury
Rup[ture of abdominal aorta
massive nad rapid blood loss > hemorrhagic shock
Extrapyramidal SE of antipsychotic meds:
inner restlessness
inability to sit/stand in one positoin
Akathisia
involuntary and potentially irreversible movement disorder d/t prolonged antipsychoitc expsure
Tardive dyskonisia
relationship betweeen xanthelasma and yellowish eyelid papule
Primary biliary cirrhosis> hypercholesterolemia
Differential cyanosis restricted to the LOWER body in a child
PDA
Whole body cyanosis in a child
Septal defect
TOF
Coaractation of aorat
can limite exercise intolerance but does NOT cause cyanosis in children/adults
innervates the stapedius muscle and paralysis of hteis msucle can lead to hyperascusis (icnrased sensitivity to sound)
CN 7