March 16 Flashcards
Thiamine B1
Function
carbohydrate metabolism
decarboxylation of alpha-keto acids
pyruvate dehydrogenase cofactor
Deficiency:
Dry beriberi - polyneuritis and symmetrical muscle wasting
Wet beriberi - dilated cardiomyopathy, edema
Wernicke-Korsakoff Syndrome - confusion, ophthalmoplegia, ataxia, memory loss (permanent)
damage to mammillary bodies and medial dorsal nucleus of thalamus
tx: glucose AND thiamine
Riboflavin B2
Function
FAD
Deficiency
angular cheilosis, stomatitis, glossitis, corneal vascularization
normocytic anemia
Niacin B3
Function
NAD/NADP
tx: dyslipidemia (decrease VLDL, increase HDL)
side fx: flushing
Deficiency = Pellagra
dermatitis, dementia, diarrhea, peripheral neuropathy
Tryptophan can be used to synthesize Niacin B3 if not available in diet
Hartnup Disease - autosomal recessive, inability to absorb/resorb neutral amino acids –> tryptophan deficiency and Pellagra
Pyridoxine B6
Function
synthesis of heme, niacin, histamine, 5-HT, Epi, NE, DA, GABA
Deficiency
cheilosis, stomatitis, glossitis
convulsions, peripheral neuropathy
sideroblastic anemia due to impaired heme synth
must supplement with INH therapy
Folate B9
Function
DNA and RNA synthesis
Deficiency
macrocytic megaloblastic anemia
increased homocysteine
normal methylmalonic acid
acquired due to phenytoin, sulfonamides, methotrexate
Cobalamin B12
Function
cofactor for methionine synthase
Deficiency
macrocytic megaloblastic
degeneration of dorsal cloumn, lateral corticospinal, and spinocerebellar tract
increased homocysteine and increased methylmalonic acid
absorbed in the terminal ileum
Ascorbic Acid Vitamin C
Function
hydryoxylation of proline and lysine for proper collagen synthesis
Deficiency = Scurvy
petechial hemorrhage, gingival swelling, impaired wound healing, weak immune system
Aschoff Body
aka Interstitial Myocardial Granuloma
lymphocytes, macrophages, multinucleated giant cells
Acute Rheumatic Fever related myocarditis post untreated Group A Strep infection
Embryonic Defect: Agenesis
Complete absence of an organ
ex. renal agenesis
Embryonic Defect: Association
Multiple anomalies with an unknown unifying cause
ex. VACTERL
Embryonic Defect: Deformation
Structural abnormality due to extrinsic mechanical force
ex. adhesions causing finger malformations
Embryonic Defect: Dysplasia
Abnormal organization/cellular architecture in a tissue
ex. Osteogenesis Imperfecta
Embryonic Defect: Field Defect
Initial disturbance causes multiple malformations
ex. holoprosencephaly
can be genetic like DiGeorge or environmental like Accutane - isoretinoic acid
Alpha-1 antitrypsin deficiency
Autosomal Dominant
Z-allele - decreased secretion of alpha-1 antitrypsin due to abnormal protein folding
Affects LIVER and LUNGS
Lungs - panacinar emphysema due to unopposed destruction of alveolar walls
Liver - intrahepatocyte accumulation of polymerized misfolded AAT - stain red/pink with acid-Schiff
Pt with wheezing and poor air movement with increase AST and ALT
Midshaft Humerus Fracture
Damage:
Deep Brachial Artery
Radial Nerve –> wrist drop = inability to extend forearm (triceps) + cutaneous sensory of dorsal hand, forearm, upper arm
Chronic Granulomatous Disease
NADPH oxidase mutation
Dihydrorhodamine flow cytometry test - decreased fluorescence
Nitroblue tetrazolium test - does NOT turn blue
recurrent infection with catalase positive organisms
Candida Aspergillus T (no) Aureus (Staph) Listeria Aeruginosa (Pseudomonas) Serratia E. coli
Myeloperoxidase
blue-green heme-containing pigment that gives sputum its color
generates HCLO’ from H2O2 +Cl-
3’ end of tRNA
CCA
HbF
Fetal Hemoglobin
2 alpha
2 gamma
Canagliflozin
Dapagliflozin
SGLT2 inhibitors (Sodium GLucose coTransporter 2)
block Glucose/Na resorption from the lumen of the proximal tubule - increase glucose loss in urine
Contraindicated in patients with moderate-to-severe renal impairment
Check BUN and Cr first!
Short Acting Benzodiazepines
Half life <6hr
Triazolam, oxazepam, midazolam
Intermediate Benzos
Half life 6-24hr
Alprazolam, lorazepam, temazepam
Long Acting Benzos
Half life >24hr
Diazepam, Flurazepam, chlordiazepoxide
Urine Electrolytes in DKA pt
Urinary Acid Secretion accomplished by
free H+
(NH3 + H+) = NH4+
(PO4– +2H+) = H2PO4
decreased pH decreased HCO3- (increased bicarb resorption) increased H increased H2PO4 increased NH4+
Metastasis of Prostate Cancer
via prostatic venous plexus –> vertebral venous plexus
Most common benign tumor of the breast
Fibroadenoma
well demarcated
Most important steps for preventing central venous catheter infections
hand hygiene
full barrier precautions
chlorhexidine skin disinfection
removal of catheter when it is no longer needed
femoral vein has higher rate of infection
Hepatitis D
dependent on HBV because it needs its viral particles
Colonic Adenoma –> Carcinoma
COCKED AK 53
APC
COX-2
KRAS
p53
Effect of Nitrates
Venodilators
Decreased: Left ventricular end-diastolic pressure
Decreased: systemic vascular resistance
Increased: Peripheral venous capacitance
Portal Triad Contents
Common Bile Duct
Hepatic Artery
Hepatic Portal Vein
Tetanus route
Wound –> Motor neuron axon –> spinal cord
Phenylephrine
alpha1-agonist
increases peripheral vascular resistance and systolic blood pressure
this causes
reflex bradycardia and decreased pulse pressure
Homocystinuria
Autosomal Recessive deficiency of Cystathionine beta-synthase (required pyridoxine B6 cofactor)
dislocated lens (3-10years old)
intellectual disability
marfanoid habitus
thromboemoblic occlusion
restrict methionine
Warfarin-induced skin necrosis
Occurs in individuals with underlying
Protein C Deficiency
Protein S Deficiency
shortly after starting warfarin
tx: fresh frozen plasma
Angiotensin II Receptor Blocker
-sartans
increased renin increased AngI increased AngII decreased Aldo no change in bradykinin
Less addicting sleep aid?
Zolpidem
alpha-1 adrenergic
Organ - Effect
Peripheral vasculature - vasoconstriction - increase BP (systolic)
Bladder - contraction internal urinary sphincter
Eye - mydriasis (constriction of pupillary dilator)
Drugs: phenylephrine, epinephrine, norepinephrine
beta-1 adrenergic
Increase heart rate, contractility, conductance
Drugs: epinephrine, Dobutamine, Dopamine, Isoproterenol
beta-2 adrenergic
peripheral vasculature - vasodilation - decrease BP (diastolic)
bronchodilation
isoproterenol, terbutaline
Isoproterenol
beta-1 = increase cardiac contractility
beta-2 = decrease vascular resistance
Clonidine
alpha-2 = decreased sympathetic outflow @ CNS = decrease is peripheral vascular resistance, decrease heart rate, decrease blood pressure
Hepatic Processing of Bilirubin
1) carrier mediated uptake at hepatic sinusoids
2) conjugation with glucuronic acid by UGT (uridine diphosphate-glucuronyltransferase)
3) biliary excretion of water-soluble, nontoxic bilirubin glucuronides
Conjugated Bilirubin
“Direct Bili”
water soluble
loosely bound to albumin
excreted in the urine
Unconjugated Bilirubin
“Indirect Bili”
highly unsoluble in water
tightly bound to albumin
toxic
deposits in tissues (brain!)
Crigler-Najjar
Autosome Recessive lack of UGT enzyme - responsible for conjugating bilirubin
increased levels of circulating toxic unconjugated bilirubin
deposition in the brain causses kernicterus - potentially fatal
kernicterus - muscle rigidity, lethargy, seizures
t(14:18)
Follicular Lymphoma (b-cells)
18 = overexpression of Bcl-2 which is anti-apoptotic
14 = heavy-chain Ig
painless waxing/waning LAD in adults
t(8:14)
Burkitt lymphoma
8=c-myc
14= heavy chain Ig
associated with EBV
Jaw lesion in endemic form in Africa
pelvic or abdominal mass in sporadic form
‘starry sky’ appearace = sheets of lymphocytes
p53
tumor suppressor gene - decreased activity in cancers and tumors
Immunity to tetanus toxin
circulating antibodies that neutralize it :)
immunization with toxoid
Gaucher Disease
Autosomal Recessive lysosomal storage disorder common in Jews
beta-glucocerebrosidase deficiency
bone pain, easy bruising and bleeding, pallor and fatigue, hepatosplenomegaly
anemia/thrombocytopenia/leukopenia/pancytopenia
lipid laden macs = crumpled tissue paper
Lympatic Drainage of Lower Limb
Lateral track –> popliteal and deep inguinal/superolateral LN
medial track –> superficial/superomedial inguinal
Lymphatic Drainage: Prostate
internal iliac LN
Lymphatic Drainage: Scrotum
superficial inguinal LN
Lymphatic Drainage: Testes
para-aortic LN
Promoter Region
bind RNA Pol II and Transcription Factors directyl
TATA @-25bp
CAAT @ -70-80bp
Enhancer Region
Bind activator proteins, facilitates interaction with promoter region –> increased rate of transcription
can be up or down stream of gene
within intron or on separate chromosome
Familial erythrocytosis
reduced binding of 2,3-BPG at beta-globin
increased hemoglobin oxygen affinity - left shift
gamma-globin of fetal hemoglobin also has decreased binding of 2,3-BPG –> increased O2 affinity
Sarcoidosis
elevated serum calcium
elevated serum ACE
noncaseating granulomas
bilateral hilar adenopathy
in pulmonary - increased CD4+
Most likely side effect of SSRIs
sexual dysfunction - decreased libido, decreased arousal, anorgasmia
depression, anxiety disorder, bulemia, panic disorder, PTSD, OCD,
Minimal Change Disease
increased selective filtration of proteins
podocyte foot process fusion –> loss of negative charge –> loss of albumin in the urine aka selective albuminuria
symp: facial puffiness/edema
most common NEPHROTIC dz in children
Hemolytic Uremic Syndrome
post bloody diarrhea from EHEC (releases shiga-like toxin) or Shigella infection
acute renal failure
anemia, thrombocytopenia
smear: schistocytes
normal coag studies
Shiga Toxin
Shiga-Like Toxin
Shiga from Shigella
Shiga-like from EHC
inactivate 60S ribosome
precedes hemolytic uremic syndrome
Ovarian Torsion
sudden-onset unilateral pelvic pain +/- nausea, vomiting, fever
most commonly due to ovarian mass (cyst/neoplasm) which causes ovary to twist
infundibulopelvic ligament aka suspensory ligament of the ovary carries ovarian N. A. V. - becomes occluded in torsion
Complication of Left Atrial Enlargement + Atrial Fibrillation
thromboembolism, clot develops in Left Atrial Appendage (little cul-de-sac)
cause stroke.
Trochlear Nerve (CN IV) Palsy
vertical diplopia - most noticable when looking down towards nose
ex. up close reading and walking down stairs (but not a problem when looking up)
superior oblique muscle - causes eye to internally rotate and depress
patients may compensate by tucking in chin and tilting head away from affected eye
Actinomyces israelii
Gram + anaerobic branching filamentous not acid fast bacteria forms yellow ‘sulfur granules’
oral/facial abscesses
tx: Penicillin
Mallory-Weiss Tear
Esophageal tear caused by high intra-gastric pressure (vomiting)
may be seen with Metabolic Alkalosis due to loss of H+ during emesis
Cells with Neural Crest Origins
Melanocytes Odontoblasts Tracheal cartilage Enterochromaffin cells Laryngeal cartilage
Parafollicular C cells
Adrenal Medulla (Chromaffin cells)
Schwann Cells
Spiral Membrane
Marker of Osteoblast Activity?
bone-specific alkaline phosphatase
Haemophilus influenzae type b (Hib) infections
meningitis* #1
pneumonia
epiglottitis
sepsis
virulence factor: PRP capsule - acilitates invasion
PRP = polyribosyl ribitol phosphate
Hib Vaccine
cell wall polysacchride conjugated with protein toxoid from diphtheria or tetanus
given at 2 months
decreases meningitis (also pneumonia, bacteremia, epiglottitis)
Thionamides
Methimazole and Propylthiouracil
inhibit thyroid peroxidase - impairs iodine organification
Methimazole - teratogenic
PTU - decreased peripheral conversion of T4 –> T3
can cause agranulocytosis - pts taking these meds with signs of infection need WBC count with differential!
Congenital Hydrocephalus Symptoms
macrocephaly
bulging fontanelle
poor feeding
UMN lesion: muscle hypertonicity. spasticity, hypereflexia
developmental delay
seizures
Wolff-Parkinson-White Syndrome
accessory AV conduction pathway
recurrent supraventricular tachycardia in an otherwise healthy individual
shortened PR delta wave at the start of QRS widened QRS (not using His Purkinje system)
Acetazolamide
Proximal Convoluted Tubule Diuretic
inhibits carbonic anhydrase –> blocks NaHCO3 (and water) resorption –> decrease in total body HCO3- and alkaline urine
decrease aqueous humor synthesis via inhibition of carbonic anhydrase
tx: glaucoma, altitude sickness, metabolic alkalosis (more HCO3- in the pee)
ACIDazolamide causes ACIDosis
Antiretroviral Protease Inhibitors
-NAVIR (darunavir, ritonavir, lopinavir)
ARDs: hyperglycemia, CYP450 inhibition, buffalo hump fat deposition
Anesthetic with high peripheral tissue uptake
large arteriovenous gradient (lungs –> arterial circulation –> uptake by tissues –> venous circulation)
this means that it has high tissue solubility and will have a SLOWER onset of action
low peripheral tissue uptake = low tissue solubility = fast onset
Minimal Alveolar Concentration (MAC)
Defines POTENCY of an anesthetic
high potency = low MAC
Mac = alveolar concentration required to sedate 50% of general population
Migratory thrombophlebitis
aka Trousseau Syndrome
indicative of visceral cancers - esp adenocarcinoma of pancreas, colon, and lung
hypercoagulability due to visceral cancer
Orthopnea
LEFT-sided heart failure
Acyclovir
guanosine analog that is activated by virally-encoded thymidine kinase
treats: HSV-1, HSV-2, VZV
Ineffective: EBV, CMV
Physiologic Changes associated with Exercise
Increased HR
Increased SV
Increased CO
vasodilation in active skeletal muscles
Decreased systemic vascular resistance (^)
