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Last Test > NV > Flashcards

NV Flashcards

1
Q

Blood supply to liver?

A

2/3 from Portal v.-deoxygenated, rich in nutrients; 1/3 from hepatic a. - provides O2 to liver cells

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2
Q

Fxns of the liver?

A

Metabolism (carbs, lipids, AAs), Synthesis (albumin, clotting factors, VLDL, LDL, HDL, cholesterol, glycogen), Catabolism (ammonia–>urea; hormones, detoxifies foreign cmpds/drugs/chemicals), Storage (glycogen, TGs, Fe++, Cu++, fat-sol. vits), Excretion (bile, endogenous waste), Blood reservoir, Endocrine

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3
Q

Enzymes involved in AA metab., present in cytosol of hepatocytes, are elevated in hepatic injury

A

AST, ALT (serum transaminases)

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4
Q

ALP

A

enzyme removes PO4 groups, found in liver, bone and intestines as diff. isoenzymes, in bile duct/liver cells, elevated in cholestatic disorders

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5
Q

Most sensitive enzyme indicator of liver disease?

A

GGT (gamma glutamyl transpeptidase)- enzyme inv. in glutathione metab., and drug detox

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6
Q

When GGT and ALP are elevated together this indicates?

A

Hepatobiliary disease

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7
Q

Get decreased serum levels of this protein in liver disease but level does NOT correlate with severity of disease?

A

Albumin (protein produced by liver, maintains normal oncotic pressure)

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8
Q

Etiologies of Acute Hepatitis ?

A

Viral (72%), Acetaminophen OD, response to meds (ie Cipro), excess alcohol, Autoimmune, Metabolic disorders, circulatory disorders

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9
Q

Clinical Manif. of Severe Acute Hepatitis

A

Acute encephalopathy, coagulopathy, ARF, GI bleeding, Infection/Sepsis, Resp. failure, Cardiovascular collapse

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10
Q

Poss. outcomes of Acute hepatitis?

A

May: 1) Resolve w/ supportive therapy,

2) Proceed to Acute Liver Failure,
3) Develop into Chronic Hepatitis

(liver has large regen. capacity, hepatocytes divide even during necrosis/chronic injury)

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11
Q

What serum measurements are markers of Biliary exretory fxn?

A

Bilirubin, ALP, GGT

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12
Q

Histo features they look for when evaluating for liver hepatitis?

A

Spotty/lytic necrosis, Ballooning degen., Interface hepatitis, Confluent necrosis, bridging necrosis

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13
Q

Acute Hepatic Failure is clinical syndrome that results from?

A

inadequate liver fxn due to either diminished # of hepatocytes or impaired fxn; MC due to chronic liver disease, may result from compensated chronic disease w/ sudden flare of activity (acute-on-chronic LF)

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14
Q

Acute Hepatic Failure (AHF) → Hyperammonemia →?

A

Hepatic encephalopathy (behavioral changes, rigidity, hyperreflexia, Asterixis, EEG changes)

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15
Q

how does AHF → Coagulopathy?

A

decreased production of clotting factors →bleeding diathesis and incr PTT,

hypersplenism/marrow suppression → Thrombocytopenia,

liver fails to clear activated factors from circ → DIC

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16
Q

Microvesicular steatosis?

A

multiple tiny droplets of fat that do NOT displase the nucleus- seen in acute fatty liver of pregnancy and toxic rxn to drugs (such as tetracycline, valproate)

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17
Q

Lab findings of AHF?

A

elevated serum AST, ALT, hyperammonemia, hypoalbuminemia

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18
Q

Hepatorenal syndrome?

A

Functional RF w/o intrinsic morphologic abnormalities, decr renal perfusion, systemic vasodilation →compensatory renal vasoconstriction→ decr GFR,

Renal fxn normalizes if liver fxn returns to normal

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19
Q

Def of Chronic Liver Disease?

A

Various liver diseases + abnormal tests lasting 6+ months assoc. w/ progressive fibrosis, ultimately → cirrhosis

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20
Q

Pathogenesis of Liver Cirrhosis?!

A

Stimulation of Ito/stellate cell by ROS, Gfs, IL-1 produced by damaged hepatocyte→ myofibroblast-like cell: produces smooth m. actin, GFAP →→ collagen deposited→ fibrosis →cirrhosis

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21
Q

Etiologies of Chronic liver disease?

A

NAFLD, Hep B/C, Alcoholic LD, Hereditary hemochromatosis, alpha-1 antitrypsin deficiency, Wilson’s disease, PBC/PSC, Autoimmune hepatitis

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22
Q

Pathophys changes in liver cirrhosis that →alterations on microvasculature architecture ?!

A

Loss of Sinusoidal cell fenestrations, Shunt development, High-pressure, fast-flowing vessels WITHOUT solute exchange, loss of functional integrity

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23
Q

Regenerative nodules?

A

micro (<3mm) and macro (3+ mm) from regeneration of liver cells in canals of Hering (progenitors of parenchymal and bile duct cells), seen in Cirrhosis

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24
Q

Morph of Cirrhosis

A

Bridging fibrous septa: fibrosis c/o delicate bands or broad scars surrounding multiple adjacent lobules,

Disruption of ENTIRE liver architecture by fibrous scars,

Regenerative nodules,

alterations in microvasculature

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25
Q

Clinical manif of Cirrhosis

A

Silent or nonspecific Sx: weakness, anorexia, weight loss;

In advanced disease: progressive LF, Portal HTN

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26
Q

Cirrhosis is the MC cause of Portal HTN which → what major consequences?

A

Ascites, portosystemic venous shunts (→esophageal varices, hemorrhoids, caput medusae), Congestive splenomegaly, Hepatic encephalopathy

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27
Q

Fxns of bile?

A

Bile acids are needed for emulsification/ micelle formation for absorption of dietary fat, provides bicarb for neutralizing gastric acid, inv. in elimination of cholest., organic molecules, heavy metals, lipophilic drug metabolites

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28
Q

Unconjugated form of bilirubin?

A

water insoluble (cannot be excreted in urine), toxic, crosses BBB in infants→ kernicterus (presents as neurologic deficits/seizures)

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29
Q

Conjugated form of bilirubin?

A

water soluble, nontoxic, excreted in urine

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30
Q

Bile Salts? fxn?

A

are Bile Acids conjugated w/ taurine or glycine = Cholic acid, Chenodeoxycholic acid ;

Detergents - solubilize H2O-insoluble lipids

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31
Q

accum. of substances normally excreted in bile (ie. bilirubin, cholesterol, bile acids)/ bile pigment in hepatic parenchyma due to decr bile flow or impaired bile formation

A

Cholestasis (causes may be extrahepatic/intrahepatic obstruction of bile ducts, OR defects in hepatocyte bile secretion)

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32
Q

Histo feature seen in Cholestasis?

A

“Feathery degen.’ -clear cytoplasm w/ wispy cytoplasmic threads,

if intracellular- see bile in cytosol,

Bile plugs in dilated canaliculi

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33
Q

Clinical manif of Cholestasis

A
  • Incr serum bilirubin→Jaundice
  • Serum bile acids→ Pruritis,
  • Malabsorp. of fats/fat-sol vits→ Steatorrhea, hemorrhage, clotting disorders,
  • Incr serum cholesterol→ Xanthomas of skin
  • Elevated ALP and GGT
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34
Q

Phagiocephaly?

A

Assymetric head, deformation, can occur pre- or post- natally, can be corrected by helmets/positioning

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35
Q

Dysmorphic individual?

A

one who shows a problem w. generalized growth and/or in the growth/formation of atleast one structure of the body

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36
Q

what % of all newborns have a recognizable major congenital anomaly?

what % of these are due to a syndrome?

A

3%

(but will be seen in up to 7% of all births if followed to age 6);

25%

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37
Q

Defects that require medical or surgical intervention and will have a sign. impact on the health of the infant known as?

A

Major anomalies (ex: neural tube defects, cleft lip/palate)

(3+ minor anomalies incr suspicion of a poss. major anomaly)

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38
Q

3 Types of problems in morphogenesis that → congenital anomalies?

A

Malformation, Deformation, Disruption

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39
Q

Malformation?

Mechs?

exs?

A

Intrinsically abnormal developmental process→primary structural defect;

altered tissue formation, growth or differentiation due to genetic, enviornmental or a combo of factors ;

cleft lip, polydactyly

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40
Q

Deformation?

Mechs?

exs?

A

an abnormality of morphogenesis due to extrinsic force on a normally developing structure;

can be extrinsic (Fetal constraint) or intrinsic (fetal akinesia);

Clubfoot, plagiocephaly

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41
Q

What situations pre-dispose to deformations?

A

Uterine malformations or pathology (ie Bicornuate uterus, fibroid cysts), multiple fetuses, Breech position

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42
Q

Disruption?

Mechs?

A

Abnormality of morphogenesis due to destructive force acting upon the developing structure;

Cell death or tissue destruction due to vascular, infectious or mechanical force (trauma, compression, tearing)

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43
Q

MC cause of Disruption we see?

A

Amniotic bands that → vascular occlusion, another common cause of vascular disruption is cocaine abuse

(most disruptions are assymetrical!)

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44
Q

Gastroschisis is due to what type of congenital anomalie?

A

Disruption- occlusion of the omphalomesenteric a. → body wall defect w. herniation (get high AFP)

(young mothers who smoke at incr risk)

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45
Q

What are some vascular causes of Disruption?

A

Aberrant vessels, Vascular occlusion, hypoperfusion, vasoactive drugs (cocaine, amphetamines)

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46
Q

Porencephaly?

A

Congenital disorder due to vascular accident/disruption, occlusion of a cerebral a. → porencephalic cyst in the brain

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47
Q

Poland Anomaly ?

A

Subclavian a. disruption →absent pectoral m. defect and ipsilateral limb defects (reduced digits), MC on R side (if on L see heart defects), seen when mother is Diabetic

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48
Q

A cascade of effects stemming from a single localized abnormality in early morphogen. – the 1st defect may be a malformation, disruption or deformation?

A

Sequence

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49
Q

Holoprosencephaly is due to?

A

Malformation sequence - 1° malformation→ incomp. cleavage of prosencephalon and faulty bifacial devel. (most severe form: cyclopia w. proboscis)

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50
Q

Why are women on cholesterol lowering meds at a much higher risk of having baby w. brain anomalies?

A

Cholesterol activates ‘Shh’ which is a transcription promoting factor that needs to fxn properly to get normal brain devel.

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51
Q

Robin Sequence?

A

Sequence of anomalies initiated by either malformation (hypoplastic mandible) or deformation(mandibular constraint) → abnormal tongue position, U-shaped cleft palate, possible airway obstruction, Micrognathia

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52
Q

Potter Sequence?

A

Initiated by lack of amniotic fluid due to renal agenesis or from amniotic rupture (disruption) →fetal compression→ pulmonary hypoplasia, potter facies, positioning defects, breech position, growth deficiency

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53
Q

A combo of anomalies that occur together more frequently than by chance alone but underlying etiology is unknown, most cases sporadic?

A

Associations

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54
Q

Coloboma of eye, Heart defects, Choanal atresia, retardation of growth/devel., genital and ear anomalies?

A

CHARGE Syndrome from gene mutation on Chr 8

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55
Q

VATER?

A

an association c/o vertebral defects, imperforate anus, T-EF, renal or radial ray defects

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56
Q

MURCS

A

an association c/o Mullerian duct, Renal and Cervical vertebral defects

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57
Q

Anomalies of several diff. structures, all of which lie in the same body region during embryogenesis?

Ex of this?

A

Complex ;

OEIS complex= Omphalocele, exstrophy, imperforate anus, spinal defects

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58
Q

Multiple structural defects in one or more tissues thought to be due to a particular chromosomal, genetic, teratogenic or unknown insult that impairs mulriple tissues

A

Syndrome

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59
Q

See short stature, mental retardation, limb defects, classic facies (unibrow)

A

Cornealia de Lange Syndrome, caused by mutations in gene (NIPBL) on Chr 5

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60
Q

Multiple structural defects in one or more tissues thought to be due to a particular chromosomal, genetic, teratogenic, or unknown insult that impairs multiple tissues

A

Syndrome

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61
Q

Retinoic acid embryopathy?

A

Syndrome that results from maternal use of retenoids (MC Accutane) → ear/facial anomalies, CNS defects etc

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62
Q

Drug that disrupts growth of neural axons → Limb defects (Phocomelia)

A

Thalidomide

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63
Q

Steps inv in the work-up of the genetic pt.?

A

collect history (of pt., of pregnancy, FH),

examine pt., Testing, est. Dx, counseling for pt./family

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64
Q

FH of a genetics pt. should inc?

A

Generate a fam pedigree for 3 gens. -record miscarriages and causes of death, Ask about Consanguinity and ethnic origins

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65
Q

Pregnancy history of a genetics pt. should inc?

medical history should inc?

A

weight gained, fetal mvmt, any testing prior/during;

review labor/delivery, feeding history, medical probs, devel. milestones -ask about regression (which could be sign of a metabolic disorder that needs prompt Tx)

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66
Q

explain Free cell DNA testing

A

fetal DNA leaks into maternal circ. and is methylated diff. than adult DNA, so you can get a ratio of methyleted vs not, that should be 1:1 if baby has correct # of Chr.

pretty accurate, not perfect but much better than maternal serum screenings

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67
Q

PE of a genetics pt should inc?

A

observation- look for asymmetry, estimate devel. age,

Measurements- growth parameters, head size (inc. parents), anything else that look abnormal (ex: Canthal measurements)

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68
Q

Canthal measurements: What are some different findings?

A

Telecanthus: inner canthi is incr and outer canthi is normal, marker of FAS and Waardenburg Sx;

Hypertelorism: both measurements are incr, seen in a lot of disorders;

Hypotelorism: need to image brain for holoprosencephaly

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69
Q

If child has disorder/intelectual delays but you do not recognize a specific syndrome what testing should you start w.

A

Chr Microarray

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70
Q

When should a fam/individual be referred for genetic counseling?

A

Prior to conception when there is a FH or other risk factor that incr chance for an abnormal offspring and ASAP for newly diagnosed pts

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71
Q

What must you have to provide genetic counseling?

what info will be covered in session?

A

accurate Dx, complete FH, current info on condition, unbiased approach;

basic info on condition, inheritance, risk for others, options available (prenatal testing etc), prognosis, Tx options

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72
Q

Diff ways to communicate risk to pts in genetic counseling?

A

Mendelian trait (calculated risk), Polygenic/multifactorial traits- emperic risk (observed), and Bayes Theorem (use of both calc. risk w/ observed data)

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73
Q

what systemic viral infections can cause hepatitis?

A

EBV (infectious mono), CMV, HSV, Adenovirus (neonates, IC), yellow fever

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74
Q

Which hepatotropic viruses never cause chronic hepatitis?

A

HAV, HEV

(“vowels A,E = only AcuteE hepatitis, except HEV in IC/pregnant women)

(“Consonants B, C, D- cause Chronic disease)

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75
Q

HBV genome structure?

HBV 3 types of viral particles?

A

relaxed (noncovalently closed) circular, partially ds DNA, Polymerase attached to 5’ end of the - strand, RNA primer attached to 5’ end of the + strand, overlapping open reading frames;

complete spherical infectious virion (Dane particle), 2 types of noninfectious- filamentous/spherical particles have only envelope structure

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76
Q

How is HBV transmitted?

A

by “Blood, Birthing and Bonking”

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77
Q

only hepatotropic v. that is more often chronic than not, is almost never detected in acute form, 80+% develop chronic hep., 20% of whom develop cirrhosis?

A

HCV

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78
Q

Typical course of acute viral hepatitis?

A
  1. Incubation period: ASx
  2. Symptomatic Pre-icteric: malaise, fatigue, nausea, fever, 2wks post-exposure
  3. Icteric phase: jaundice, dark urine, clay-colored stools, hepatomegaly, 1-2wks after prodrome, lasts up to 6wks
  4. Covalescence: diminishing jaundice, 6-8wks post-exposure
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79
Q

when are pts w/ acute viral hepatitis most infectious?

A

last ASx days of INCUBATION period

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80
Q

Labs in acute viral hepatitis?

A

high levels of transaminases (>1000 U/L),

Hyperbilirubinemia (due to cholestatic jaundice/cholestasis),

Viral serology

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81
Q

Def of Chronic viral hepatitis

A

Symptomatic, biochemical or serological evidence of continuing/relapsing disease for longer than 6mos (26wks)

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82
Q

Carrier state of viral hepatitis

A

Harbors organisms without S/Sx, either +/- liver damage → reservoirs for future infection

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83
Q

Hepatocellular damage in viral hepatitis is due to?!

A

Host’s adaptive immune response to viral proteins expressed by infected cells- T-cells are activated after recognition → Necroinflamm. activity, Apoptosis (Immunopathology)

(NOT due to direct viral cytopthic effect)

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84
Q

HAV general features?

A

Fam=Picornaviridae, Genus- Hepatovirus,

small, naked, +sense, ss-RNA w/ a single ORF that encodes a single polypeptide- proteolysis of this →structural/regulatory proteins,

5’ non-coding region has an IRES for cap-indep. translation (No cap!),

3’ end has poly-A tail,

usually benign, self-limited course

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85
Q

HAV transmission?

A

fecal-oral, via contaminated food/H2O, contaminated shellfish concentrates virus- can infect if undercooked, (or blood transmission=minor route),

heat stable, acid and detergent (ie 1% SDS) resistant;

HAV shed in feces 2-3 weeks before, and 1 wk after the start of jaundice (can spread to close contacts during this time)

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86
Q

HAV prevalence/epidemiology?

A

endemic in developing countries (Asia, Africa, SA) w/ poor sanitation- kids under 6 affected, ASx/mild clinical disease, many have Abs by age 10;

in developed- better socioeconomy/hygeine but no herd immunity, older age group affected, more severe Sx, incidence very low in US due to vaccine

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87
Q

HAV serology?

A

IgM: rises w onset of Sx, marker of acute infection,

IgG: rises after months, when IgM declines, persists, confers lifelong immunity against reinfection w. all strains (no direct detection og IgG must measure total then subtract IgM to get IgG)

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88
Q

HBV gen features?

A

Hepadnavirus, spherical double shelled, partially ds relaxed circular DNA, very resistant to extreme temp/humidity, “Dane particle”=complete infectious virion, 4 open-reading frames:

Nucleocapsid core, capsid shell is made of: HbcAg

envelope glycoproteins (surface): HbsAg

Polymerase (Pol) w. DNA Pol and reverse transcriptase activity

HBx protein

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89
Q

HBV, serum markers of active replication?

A

HBV-DNA, HBsAg, HBeAg

(HBeAg is marker of active infection and infectivity)

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90
Q

Appears during peak Sx of Hep B, implies waning of acute infection?

A

Anti-HBe

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91
Q

HBV mode of transmission?

A

Contact w. contaminated body fluids (serum, urine, saliva…),

depends on geographics: high prevalence areas (ie Asia)- MC route is perinatal infection (both horizontal/vertical) - early childhood infection→HIGH rate of chronicity

Horizontal in intermediate prev. areas,

in low prev. areas (US): Sexual/IV drugs -low rate of chronicity

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92
Q

Clinical outcomes of Hepatitis B ?

A

Most cases (~90%)- self-limited, spontaneous resolution/recovery, majority w/ no Sx or jaundice, some w acute disease,

Chronic disease (5-10%)- may progress to cirrhosis +/- develop HCC,

Fulminant/acute liver failure (<0.5%)

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93
Q

What predicts chronicity of Hepatitis B?

A

Age at time of infection, younger= more likely to become chronic (ie vertical transm from mother→baby)

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94
Q

Why is complete cure of chronic Hepatitis B hard to achieve?

A

Virus inserts itself into host DNA →host cannot mount effective response via production of HbsAb→virus persists

(so Tx invs limiting progress of disease/damage to liver -prevent cirrhosis/HCC)

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95
Q

What determines disease outcome of Hepatitis B?

A

Host immune response, in early stage innate immune response (NK cells, IFN) protects, resolution if CD4+/CD8+ produce IFN-gamma, etc and clear infected cells;

If weak immune response: some infected cells not killed→CHRONICITY

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96
Q

Why is it difficult to make vaccine against HCV?!?

A

due to genomic instability, Ag variability, RNA Pol- poor fidelity in copying, thus many genotypes/subtypes (even in same pt.- quasispecies)

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97
Q

HCV gen features?

A

Flaviviridae, Small, + ssRNA virus, RNA codes for only 1 polyprotein- processed into fxnal proteins, E2 envelope protein (target of anti-HCV Abs) is the most VARIABLE region of genome, new virus strains can escape neutralizing Abs!

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98
Q

Why is there a HIGH RATE of chronicity of Hepatitis C?!

A

Anti-HCV IgG produced after infection does NOT confer immunity! → recurrent reactivation of preexisting infection or emergence of newly mutated strain

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99
Q

Why has there been such a dramatic decr in annual incidence of Hepatitis C infections?

A

mostly due to decr in transfusion-assoc infection

(blood transfusion and Factor VIII -no longer major routes)

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100
Q

Hepatitis C Risk Factors

A

IV drug abuse, multiple sex partners, surgery in last 6mos, needle stick [(1.8%= higher risk than HIV (0.3%)], multiple contacts, work in medical/dental fields

~1/3 of pts have unknown etiology

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101
Q

HCV clinical manif

A

Incubation period: 4-26wks, most pts ASx (85%)-bc there is weak/delayed immune responses (more mild than HBV), HCV RNA is detected in blood for 1-3wks together w/ incr AST/ALT ;

Majority develop chronic Hepatitis, Cirrhosis in up to 20% (HCV RNA remains in blood, AST/ALT never normalize)

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102
Q

HCV Serology

A

HCV-RNA- marker of acute infection w/ resolution or remains in blood in chronic disease despite presence of Anti-HCV

(Anti-HCV is just diagnostic, does NOT confer immunity)

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103
Q

HCV Tx? what does response depend on?!?

(usually a Q)

A

Pegylated IFN-alpha, ribavirin;

HCV genotype- 2, 3 best response!!,

and host genotype- IL28B gene (encodes IF-lambda)

104
Q

HDV infection?

A

it is a defective “satellite virus”/viroid- requires HBV as helper for infection, both self-limited EXCEPT in IV drug abusers→ acute liver failure, can be: co-infection of HBV and HDV at same time- acute, self-limited, OR

Superinfection: when HBV carrier gets HDV → severe acute hepatitis

105
Q

HDV gen features?

A

very small, covalently circular, - ss RNA, uses host’s RNA Pol to replicate via RNA-directed RNA syntheis, Delta Ag (HDAg) protein produced by virus

(HBsAg on both HBV and HDV)

106
Q

HEV mnemonic?

A

“Pregnant Pigs in EEndia”

in India- more sporadic HEV cases than HAV,

In Pig farms in developed world,

Unique: high mortality rate (up to 20%) in Pregnant women

107
Q

HEV features/clinical manif

A

naked, + ss RNA, Hepevirus, MC form of hepatitis in endemic regions, Virions shed during acute illnes,

Fecal-oral route, HEV RNA and virions in stool/serum before Sx appear,

S/Sx: elevated AST, ALT, IgM anti-HEV at same time, self-limited

108
Q

Along w/ clinical info and serology, assess hepatitis via biopsy which can?

A

Confirm clinical Dx, exclude simultaneous conditions, Grade extent of inflamm./injury, Stage the degree of fibrosis, monitor Tx effectiveness

(EXCEPTION: biopsy not done for Hep A)

109
Q

Characteristic Morph of Hep C?! (KNOW)

A

Lymphoid aggregates or follicles in portal tract!!

110
Q

Characteristic Morph of Hep B?! (KNOW)

A

Hepatocytes look like “Ground Glass”/ granular periphery of cell, ER is stuffed full of HbsAg)

111
Q

Which demographic has longest life expectancy?

lowest?

A

Hispanic women

Non-hispanic black males

112
Q

Homeostenosis?

A

Decr in reserve capacity w/ aging- as we age we egage more of our physiological reserves just to maintain homeostasis thus there are less reserves left to address challenges; A medical issue of aging

113
Q

Normal physiologic body composition changes w aging?

A

Loss of lean body mass, decr in skeletal m. mass, decr in bone mass, Incr in total body adipose tissue→accum. in mm. and organs- more fat but not necessarily more weight

114
Q

Importance of body composition changes w aging?

A

has major implications on prescribing meds- t1/2 of lipophilic drugs increases dramatically!, serious complications can arise from “normal” drug doses

115
Q

Normal physiologic changes seen in aging?

A

Temp: incr risk for both hyper-/hypo-thermia, difficulty mounting a fever in response to infection! (all conditions causing fever in young pts may present WITHOUT fever in elderly)

Body fluid: TBW decr, thirst sensation diminished,

BP: higher BPs or orthostatic hypotension,

Senses: Dark adaptation decr, near vision declines, high frequ hearing declines, sense of smell declines after 50yo

116
Q

Normal cardio changes seen in aging?

A

incr vessel wall thickness+ decr elastin= stiff vessels (makes it difficult to get accurate BP reading), incr BP but normally below threshold for HTN, adipose infiltration, decr pacemaker cells, calcifications of heart →murmurs, epi/NE levels are higher but tissue response to beta-adrenergic stimulation is decr

117
Q

Normal Neuro changes seen in aging?

A

slower encoding, slowed recall. decr ability to multi-task, decr #/size of motor neurons, decr sensation in feet, lack of ankle reflexes

118
Q

Normal Resp changes seen in aging?

GI changes?

A

decr elasticity, FEV1 and O2 sat ;

presbyesophagus may occur, hepatic metabolic fxn may decline

119
Q

Normal Renal changes seen in aging?

A

steady decline in most but not all, affects drug metabolism!!, must review all meds/adjust doses

120
Q

Normal Immune changes seen in aging?

A

Thymic involution- less naÏve lymphocytes to respond to new threats, decr T cell response, decr in some cytokines, decr Ab response to vaccines

121
Q

Normal skeletal changes seen in aging?

A

Loss of cortical thickness, incr in cortical porositym thinning of trabeculae w loss of connections ;

122
Q

Why do ppl get shorter?

A

Wear of discs btwn bones, changes in posture- ususally due to arthritis but can be from osteoporosis (NOT NORMAL AGING but diseases)

123
Q

So normal aging is heterogenous and marked by??

A

Decr in reserve capacity (homeostenosis) and diminished ability to respond to stress

⇒ Incr vulnerability!

124
Q

BPH leads to Sx of urinary frequ, hesitancy, dribbling, should avoid what type of drugs that will make this worse?

A

alpha-agonists (Decongestants)

125
Q

Diseases manifest themselves diff in elderly, which →

exs?

A

atypical presentation= incr risk of advers outcomes and delayed Dx→ incr morbidity/mortality;

expect more vague complaints such as acute confusion/delerium, “weak and dizzy”, refuse to eat/drink, malaise, fatigue, falling

126
Q

When an elderly pt. complains of pain, why must it be considered more urgent ?

HA is relatively uncommon as a new complaint and may be a sign of what in elderly?

A

Blunting of sensation of pain may occur, many elderly pts will minimize their complaints, and abdominal pain is much more likely to be due to a life threatening disease!;

temporal arteritis, trigeminal neuralgia, HZV, subdural hematoma, metastatic disease

127
Q

Anorexia/weight loss considerations in elderly?

A

malignancy/inflamm. disorders at top of list but also consider CHF, chronic lung disease, drug ADRs, depression, memory loss, hyperthyroidism

128
Q

Weakness/fatigue considerations in elderly?

A

Apathetic hyperthyroidism, true m. weakness represents specific disease, expect weakness for up to 3 wks after a period of immobility like a hospital stay- takes awhile to get back to baseline

129
Q

Common barriers for effective communication w elderly?!

A

Ageism, sensory problems ie hearing, cognitive problems, dementia

(tips- consider pts health literacy skills/cultural beliefs, RESPECT, dedicate time w. each pt., write down instructions, address pt w last name)

130
Q

Risk factors for HAV?

A

low socioeconomic status (poor sanitation),

Travel to endemic areas,

MSM,

Illicit drug use

131
Q

HAV liver tropism is attributed to?

A

1) Hepatocellular asiaglycoprotein receptor-mediated uptake of IgA-HAV cmplx,
2) Intracellular factors favoring viral replication

132
Q

HAV mechs of infection

A

Hosts: humans ans nonhuman primates,

Cellular receptor: HAVcr1/TIM1 (class 1 integral glycoprotein),

Auxiliary factor: IgA1-lambda- facilitates infection =special ligand of HAVcr1/TIM1, forms IgA-HAV cmplx and enhances receptors intxn w/ HAV

133
Q

Cellular Immune responses to HAV?

A

in acute phase- CD8 CTLs are the main lymphocytes in liver, NK cells may also lyse HAV infected hepatocytes;

Anti-HAV CD4 T cell response imp. for recovery- CD4 Tcells become main lymphocytes in liver in the covalescent/recovery phase

134
Q

HUMORAL Immune responses to HAV?

A

Abs against the conformational epitope formed by VP1 and VP3 are protective against infection→ life long immunity after recovery, no chronic disease; Vaccination very effective for prevention!

135
Q

HBV is an enveloped DNA virus, the envelope consists of?

A

3 types of surface Ags (HBsAg):

  1. The long form: (LHBsAg)- contains pre S1, pre S2, and S domains
  2. Medium form (MHBsAg)- Pre S2 and S domains
  3. Short form (SHBsAg)- contains S domain only
136
Q

Describe HBV entry into target cells

A

HBsAgs bind to receptor→ nuclear capsid released into cells by receptor mediated endocytosis, loss of envelope →transport of core particle to cell nuclear mem. → uncoating/release of RC DNA into nucleus

137
Q

Next step after HBV RC DNA is released into host cell’s nucleus?

A

RC DNA cnvtd to covalently closed circular DNA (cccDNA) by removal of viral Pol, RNA form RC DNA then DNA synthesis/ligation by cellular enzymes → formation of viral minichromosome (where transcription occurs)

138
Q

Viral variants of HBV due to?

A

HB Pol lacks proof reading capacity →genome prone to mutation

Thus exists in host as Quasi-species- a mixture of genetic variants

139
Q

What is the key determinant for vertical transmission of HBV?

A

HBeAg and HBV DNA levels in infected woman

(90% for HBeAg + vs 32% for HBeAg neg women)

(Breastfeeding is NOT a sign. mode of transmission)

140
Q

Humoral Immune response to HBV?

A

Anti-HBsAg Abs are protective

141
Q

Cellular immune responses to AHB?

A
  • noncytolytic clearance (drop of viral titers)- occurs after peak repl./before onset of hepatitis, Mediated by viral specific CTL-produced cytokines: IFN-y, TNF-alpha, IFN-alpha/beta
  • Inflamm. cell-mediated liver damage: High levels of CD4/CD8 T cell responses detected in blood, massive liver infiltration of T cells, NK cells and neutrophils
142
Q

HCV infection invs interaction btwn?

A

HCV E1, E2 heterodimer w/ multiple cellular surface proteins which can bind to E2 to form cmplx

143
Q

How is HDV transmitted?

A

Parenteral routes- drug addicts and hemophiliacs at high risk

144
Q

How is Anencephaly diagnosed?

A

prenatally by elevation of maternal AFP and fetal US,

usually go post-term, polyhydramnios

145
Q

Encephalocele

A

Defect in cranial vault→herniation of brain/meninges

146
Q

Rachischisis

A

worst form of spina bifida, neural tube never even forms you just have neural tissue

147
Q

where does HBV RNA transcription occur?

list HBV Subgenomic transcripts (and the proteins they form)

A

Viral RNA- is transcribed from viral miniChr then exported out of the nucleus for protein synthesis/genome repl.

Pre-S1 RNA (LHBsAg)

Pre-S2/S RNA (MHBsAg, SHBsAg)

X RNA (HBxAg)

148
Q

fxns of HBV pgRNA ?

A

=pregenomic RNA- encodes viral Pol and HBcAg and serves as template for genomic repl. (lacks the start codon for HBeAg)

149
Q

what catalyzes cnvsn of unconjugated to conjugated bilirubin in the liver?

A

UDPGT

150
Q

Physiologic jaundice is due to?

A

immature conjugation/excretion mechs in babies liver till 2 weeks of age (low UDPGT activity and low levels of ligandin binding protein), can also get more bilirubin b/c: shorter RBC lifespan, high RBC mass, birth trauma

151
Q

Physiologic jaundice Tx?

A

Phototherapy- cnvts to H2O soluble isomers that can be excreted into bile w/o conjugation→ excreted into stool/urine (most resolve in 2-3wks)

152
Q
A
153
Q

Breastmilk Jaundice?

Tx?

A

lasts longer than physiologic jaundice, breastmilk has beta-glucuronidase that deconjugates conjug’d bilirubin;

tempor. stop breastfeeding

154
Q

Kernicterus

A

brain damage in baby due to excessive levels of unconjug’d bilirubin that crosses BBB, toxic to brain, due to Neonatal hyperbilirubinemia, G6PD deficiency, spherocytosis

155
Q

hereditary disorders that →Unconjug’d hyperbilirubinemia?

A

Crigler-Najjar Syndromes type 1 and II, Gilbert syndrome

156
Q

hereditary disorders that → Conjug’d hyperbilirubinemia?

A

Dubin-Johnson and Rotor syndromes

(both AR)

157
Q

Hereditary hyperbilirubinemia due to absent UGT1A1 (gene for UDGPT) activity→ kernicterus and FATAL w/o liver transplant

A

Crigler-Najjar Syndrome type 1 (AR)

(see Sx of Kernicterus- hypotonia, deafness, oculomotor palsy, lethargy)

158
Q

Dubin-Johnson Syndrome

(Sxs, labs, gross morph?)

A

AR, due to mutated gene for MRP2 (transports conjug’d bilirubin from liver cells to canaliculi)→ benign relapsing conjug’d hyperbilirubinemia, non-pruritic jaundice in teens or ASx, normal AST/ALT, gross path: BLACK PIGMENTED LIVER , non-iron (melanin) pigment

159
Q

Hereditary hyperbilirubinemia due to decreased UGT1A1 activity→ mild jaundice, non-fatal ?

Dx by? Tx?

A

Crigler-Najjar Syndrome type II (only one that is AD!!!);

by HPLC/liver biopsy enzyme assay;

lifelong phototherapy or transplant in some

160
Q

Hereditary hyperbilirubinemia due to decr (30% of nml) UDPGT activity, no Tx needed- ASx or mild jaundice only w/ stress, infection, or fasting?

Lab findings?

A

Gilbert Syndrome (AR);

incr in Unconjug’d bilirubin, incr in ratio of urinary coproporphyrin I:III

161
Q

Causes of large Bile Duct obstruction → Cholestasis?

Tx?

A

Gallstones (extrahepatic), Malignancies of biliary tree/head of pancreas, strictures from surgery;

surgery to reverse extrahepatic obstruction to avoid biliary cirrhosis, but if cause is intrahepatic surgery is NOT helpful (unless transplant)

162
Q

Ascending cholangitis?

A

Subtotal/intermittent obstruction→ secondary bacterial infection by Coliforms/enterococci from gut → F/C, abd pain, jaundice,

severe form= Suppurative- bile pus fills bile ducts, SEPSIS dominates

163
Q

Sepsis-assoc. cholestasis?

A

response to microbial products in blood, esp in systemic G– infection, 2 types: Canalicular and Ductular

164
Q

Sepsis-assoc. Canalicular cholestasis morph.

A

centrilobular canalicular bile plugs, Kupffer cell activation, mild portal inflamm., scant/absent hepatocyte necrosis

165
Q

Sepsis-assoc. Ductular cholestasis morph?

A

Dilated canals of Hering and Bile ductules, bile plugs, septic shock

(worse path than canalicular)

166
Q

Prolonged conjug’d hyperbilirubinemia in newborn that needs to be evaluated for if jaundice lasts beyond 2-3wks?

Causes?

A

Neonatal Cholestasis;

Neonatal hepatitis (toxic, metabolic, infectious or idiopathic), Cholangiopathies- biliary atresia..

167
Q

Neonatal hepatitis (which can→ cholestasis) is due to ?

pathogenesis?

A

can clinically determine etiology as either toxic, metabolic or infectious 90% of the time, need Bx 10%, 10-15% idiopathic;

immature bile synthesis/secretion pathways decompensated by injury

168
Q

Neonatal Hepatitis- liver biopsy will show?!?

A

Necrosis, GIANT CELL transformation- multinucleated giant Hepatocytes!!! unique response of young liver to injury so only occurs in infants!!;

micro: apoptotic/acidophil bodies, extramedullary hematopoiesis

169
Q

Fetal/embryonal form (20%) of Biliary atresia ?

A

aberrant intrauterine devel. of extrahepatic biliary tree→obstruction, assoc w other anomalies (of abd organs, polysplenia, etc)

170
Q

Perinatal form (80%) of biliary atresia?

A

presumably normally developed biliary tree is destroyed at birth, invs. Viruses (rota-, reo-, echo- viruses, CMV) →continual inflamm/ obstruction

171
Q

Gross morph of liver cholestasis due to biliary atresia?

micro?

A

enlarged, dark green, hard liver, micronodular cirrhosis, dilated intrahepatic bile ducts w. inspissated bile ;

fibrosis (stains blue w trichrome stain), incr ductules, neutrophils, bile plugs in ductules

172
Q

Tx of Liver cholestasis due to biliary atresia?!

A

Kasai procedure (hepatoportoenterostomy)- bypasses atretic ducts to achieve bile flow, OR transplant - It is the MC reason for liver transplantation in kids!

173
Q

Iron overload in tissues and organs such as liver due to AR genetic mutations ?

A

Primary hemochromatosis

174
Q

Iron accumulates in body due to known sources of excess iron ie. multiple transfusions, ineffective erythropoiesis (thalassemias, sideroblastic anemia), incr iron intake (Bantu siderosis), cirrhosis, chronic liver disease

A

Secondary hemochromatosis

175
Q

HFE hemochromatosis?

A

due to mutation in HFE gene → decr hepcidin→ incr iron absorption/ defect in regulation of intestinal absorption→ more iron in blood/deposited in tissues

(MC is C282Y mutation)

176
Q

HFE hemochromatosis clinical manif?

A

classic triad: hepatomegaly, slate grey skin, DM, …plus pancreas fibrosis, cardiac dysfxn, abd pain, death due to cirrhosis/ cardiac failure, but normal life expec. w Tx= phlebotomy

have 200x risk of HCC (not dec by Tx)

177
Q

AR disorder due to mutation of ATP7B gene→defective ATPase→ impaired Cu++ excretion into bile/failure to incorporate into ceruloplasmin → accum of Cu++ at toxic levels in liver, brain, eye, etc

A

Wilson’s Disease

178
Q

Dx of Wilson’s Disease?

A

Liver Bx- qunatitation of Cu++,

Lab: urinary Cu++ excretion, serum ceruloplasmin,

easily missed clinically- presents in teens/20s w neuropsychiatric, liver, neuro, and eye abnormalities

KAYSER-FLEISHER RINGS (brown ring around cornea)

(many diverse mutations so no genetic testing)

179
Q

Wilson’s disease (AKA Hepatolenticular degen.) path?

Tx?

A

Glycogenated nuclei, Cu++ granules- stain red w. rhodanine stain

(liver changes resemble viral hepatitis and fatty changes);

Copper chelation or transplant

180
Q

Alpha-1 antitrypsin (AAT) deficiency?

A

AR disorder, mutated SERPINA1 gene on Chr 14→ low levels of AAT (a serine protease inhibitor)→uncontrolled proteases→ COPD, cirrhotic liver w. accum. of A1AT-Z in cytoplasmic globules (stain w PAS), poss HCC

181
Q

3 intrahepatic Biliary tract diseases?

A
  1. Primary Biliary Cirrhosis,
  2. Secondary Biliary Cirrhosis (due to prolonged obstruction of extrahepatic biliary tree)
  3. primary sclerosing cholangitis
182
Q

Autoimmune non-suppurative inflamm./destruction of med-sized intrahepatic bile ducts⇒ chronic progressive, often fatal cholestatic liver disease, may be ASx but with incr ALP or present w biliary cirrhosis?

assoc w.?!

A

Primary Biliary Cirrhosis (90% in females);

Celiac, Sjogren’s, MS, Raynaud’s !

183
Q

4 stages of Primary Biliary Cirrhosis?

A
  • Portal: portal inflamm., BD damage, +/- florid duct lesion
  • Peripheral: ductular prolif, periportal inflamm, fibrosis
  • Septal: bridging fibrosis, ductopenia
  • Cirrhosis: Biliary cirrhosis

(histo: fibrotic or granulomatous inflamm.)

184
Q

Primary Biliary Cirrhosis Dx?

Tx?

A

Liver Bx, labs will show elevated serum ALP, GGT and AMA!!!!(antimitochondrial Abs);

Ursodiol or transplant if advanced

185
Q

Chronic cholestatic disorder char. by inflamm./fibrosis that obliterates intra-and extra-hepatic BDs up to ampulla of vater, w/ dilation of preserved segments? how does this appear on imaging?

coexists w.?

A

primary sclerosing cholangitis (PSC);

“Beading”

IBD esp. UC;

186
Q

primary sclerosing cholangitis clinical manif?

A

persistent ASx elevated ALP, Cholestatic Sx (jaundice, pruritis- Tx w cholestyramine), CHD (weight loss, ascites, varices, enceph.)

187
Q

primary sclerosing cholangitis pathogen?

A

activated T-cells in GIT (assoc w. UC) go to liver and cross-react w. Ag in BD →immune-mediated injury to BDs, CONCENTRIC PERIDUCTAL FIBROSIS → fibrous obliteration of ducts, “ONION SKIN” scar

188
Q

primary sclerosing cholangitis gross morph?

pts at risk for developing?

A

Nodules of cirrhosis, areas of hypertrophy/hyperplasia, Fibrotic ;

cholangiocarcinoma, HCC, chronic/autoimmune pancreatitis (incr IgG4)

189
Q

Deaths from acute poisoning are due to ?

A

cardiovascular complications, depressed respiration -hypoxia (further decr. by mixing barbs, opiates, benzos or alcohol)

so supporrtive careis essential to maintain resp. and circulation

190
Q

MC poisonings in kids less than 5yo?

A

vits w. IRON, OTC meds, cleaning supplies, pesticides

191
Q

MC poisonings of adults due to?

A

analgesics, drugs of abuse, antidepressants, sedatives, anti-anxiety meds, alcohol, or combo of these

192
Q

Iron toxicity: primarily occurs in? .. from?

toxic dose is calc. based on?!

toxic amt is?!

A

young kids, multi-vits w/ iron;

elemental Fe content!

20+ Mg/Kg Elemental Fe!

193
Q

Syrup of Ipecac?!

A

Emetic, stimulates CTZ in brain and has direct effect on stomach → vomiting. good for terminating exposure to MANY DRUGS ie acetaminophen, antihistamines, vits, coldremedies, benzos, pesticides (paraquat)

194
Q

When is emesis (syrup of ipecac) contraindicated?

A

corrosive agents, loss of gag reflex, comatose, sharp objects, agents assoc w seizure (strychnine, TCAs, GHB), ingestion of hydrocarbons if risk of aspiration is greater than risk of systemic toxicity

195
Q

Activated charcoal -added to lavage fluid to enhance removal of toxins, what agents are well adsorbed by it?

A

acetaminophen, aspirin, amphetamines, benzos, digoxin, opiates, malathion, Nicotine, syrup of ipecac

it can be used in comatose pts., ineffective for acids, alkalis, most metals, petroleum distillates, do NOT use for corrosive/caustic agents

196
Q

what antagonist can be used for opiate overdose ?!

A

NALOXONE! = competitive reversible antagonist at opiate (mu) receptor, reverses resp. depression of opiate

197
Q

what antagonist can be used for Benzodiazepine overdose ?!

A

FLUMAZENIL! - competitive reversible antagonist, reverses resp. depression

(benzos are the “-pam” drugs- diazepam, oxazepam..)

198
Q

N-acetyl-benzoquinoneimine (NAPQI) ?!

A

Acetaminophen is metabolized by P450s to this hepatotoxic metabolite, NAPQI is detoxified by binding to glutathione, liver damage occurs when hepatic glutathione depletion occurs → NAPQI binding to proteins

199
Q

how does SODIUM BICARBONATE enhance excretion?!

A

alkalinizes urine to enhance excretion of ACIDIC drugs (ie. barbs, aspirin)

200
Q

Major toxicity of Petroleum distillates (gasoline, furniture polish, kerosene..)?

A

Aspiration and chemical pneumonitis

201
Q

Risk of aspiration of an agent depends on?

A
  • Viscosity, surface tension, volatality
  • low viscosity incrs potential to flow into airways
  • low surface tens allows rapid spread from mouth to trachea
  • high volatality→ rapid displacement of O2 from alveoli→hypoxia
202
Q

BEST determinant of aspiration potential of petroleum distillates?

A

VISCOSITY! (expressed as SUS units)

low viscosity= high aspiration risk !!

203
Q

Most deaths from abuse of aerosol propellants (huffing) due to?

huffing while pregnant causes ?

A

Cardiac arrest or hypoxia;

decr birth weight, incr fetal death, nerve damge in baby,

there’s no antidote or Tx

204
Q

What are the long term effects from abusing the aerosol propellant Toluene? (in whiteout)

A

ophthalmic and auditory n. damage - BLINDNESS and HEARING LOSS

205
Q

What are the long term effects from abusing the aerosol propellant Benzene- which is in gasoline?

A

LEUKEMIA

206
Q

Iron toxicity Tx?!

A

emesis if caught early, bicarb lavage to decr absorption, or if plasma iron 350µg/dl - Chelation therapy with Deferoxamine!

207
Q

there is an Excellent correlation of acetaminophen blood levels with?

A

time expired since ingestion, and prediction of toxicity

(thus can use a Nomogram to evaluate risk of toxicity)

208
Q

Antidote used for Acetaminophen toxicity!?

A

n-ACETYLCYSTEINE- taken up by the cell, provides CYS for glutathione synthesis

advantage of giving this orally is 100% goes to liver but it is not pleasant so can be given IV if necessary

209
Q

Salicylates follow what order kinetics?

A

1st order at HA dose, zero order at doses taken for arthritis/toxic doses

210
Q

mechs of Salicylate toxicity?!

A
  • Aspirin directly stimulates resp. centers in medulla→​Hyperventilation →resp. alkalosis
  • toxic levels uncouple oxidative phosphorylation which stimulates CO2 production, increases ketones, lactic and pyruvic acid → metabolic acidosis (usually only in kids)
211
Q

S/Sx of Salicylate toxicity?

A

HA, tinnitus, sweating, hyperventilation, drowsiness, fever (MC in kids), acid-base and electrolyte imbalance, dehydration, low serum K+ levels (renal excretion of bicarb and organic acids)

212
Q

Can use nomogram to predict severity of Salicylate toxicity since it has good correlation with the time of ingestion and ?!?

A

PLASMA SALICYLATE concentration

213
Q

Tx of Salicylate toxicity?!

A

emesis, lavage, +/- activated charcoal, alkalinize urine to enhance salicylic acid excretion 4-fold by giving IV bicarb, makes drug more ionized so less of it can get to brain,

for kids also need to- correct electrolytes/fluids, return body temp to nml

214
Q

Methanol toxicity!?

A

irreversible toxicity to eye- photophobia and BLINDNESS

methanol metabolized by alcohol dehydrogenase to the toxic metabolites formaldehyde and formic acid, formic acid →met. acidosis and BLINDNESS

215
Q

this alcohol induces inebriation/nausea, targets kidney → RF, oliguria, and obstruction due to?

what Tx is only used for this alcohol?

A

Ethylene glycol,

oxalate crystals deposit in lumen of kidney;

Fomepizole (inhibitor of alcohol dehydrogenase, used as an alternative to ethanol)

216
Q

Tx for both Ethylene glycol and methanol toxicity?

A

IV Ethanol -competes for alcohol dehydrogenase, has greater affinity than other alcohols

217
Q

why should we care about herbal agents?

A

~1/2 of US pop. uses some form, some agents have active ingredients that: are assoc. w drug-herbal intxns, may be palliative, may have sign. adverse effects

218
Q

Gov regulation of herbal products?

A

considered dietary supplements (NOT drugs), thus do NOT have to be proven safe oe effective

219
Q

Herb labeling

A
  • no std. for efficacy, safety or validation
  • Standardized- certain conc. is consistent btwn batches
  • NF on label, meets NF std. for purity, strength of marker cmpd and labeling
  • can NOT be marketed for prevention/Tx of a disease
220
Q

Garlic: is marketed for?

its effects?

A

lowering cholesterol, preventing heart attack ;

  • Lowers cholesterol (HMG CoA reductase inhibitor)
  • Inhibits platelet aggregation via Ajoene (Allicin metab.) and Diallyl trisulfide - inhibit platelet cyclooxygenase, reduce platelet TXA2
221
Q

Garlic: Cautions?

A
  • avoid high doses w/ antiplatelet drugs
  • Avoid prior to surgery!
  • Must store chopped garlic in oil in fridge or → Clostridium infection
222
Q

Active ingredients og Horse Chestnut and their action

A
  • Escin (good cmpnt) decreases: leg pain (4fold), swelling, and vascular permeability of venous capillaries!!
  • Aesculin cmpnt that incr risk of bleeding (gums, nose, etc), similar to Warfarin
223
Q

Horse Chestnut CIs?

A
  • Anticoagulants, aspirirn, NSAIDs (since it enhances bleeding)
  • Pregnancy (category X)
  • Breast feeding
  • Avoid before surgery
224
Q

Ginko Biloba actions?

A
  • improves perfusion of capillaries/ blood flow in skin,
  • Kaemferol: anti-oxidant, reduces free redical damage, may slow aging/ improve mental focus, improves utilization of ATP and glucose in brain
  • Kaemferol and Apigenin: inhibitors of MAO-A/B
225
Q

Ginko Biloba standardized for?

A

ginko flavonglycosides: quercetin, kaempferol, isorhamnetine

226
Q

Ginko Biloba Adverse effects?

A
  • BLEEDING times can double!
  • drug intxns w/ incr risk of bleeding: inhibits PAF, aspirin, NSAIDs, Warfarin, heparin, Platelet IIb/IIIa receptor antagonsits (clopidogrel)
  • Lethal ingestion of ginko seeds (contain ginko toxin- antivit. B6, interferes w/ AA metab)→ seizures/death
227
Q

St. John’s Wort: standardized for?

Adverse effects/ drug rxns?!

A

should contain min. of: 0.3% Hypericin and 3% Hyperforin;

HA, loss of appetite, induces P450 isozyme Cyp 3A4 so may need to incr dose of OCs, theophylline, warfarin and avoid before surgery, should NOT be taken w/ MAO inhibitors or SSRIs

228
Q

Poss. MOA of St. John’s Wort

A
  • Hyperforin SSRI
  • Hypericin and Hyperforin - MAO inhibitors (enhance mood)
  • activation of GABA (they are inhibited by flumazenil), anti-anxiety action
  • Alcohol extract: anti-depressant action (for mild depression, NOT mod/severe depression)
  • cumulative effect mediated by more than 1 MOA
229
Q

Valerian: used for? mech?

active ingredients?

A

Insomnia; binds to GABA-A receptor, opens Cl- channel→hyperpolarization/ decr neuronal activity (effect similar to benzos)

Valerenic acid, valtrate, Glutamine

230
Q
A
231
Q

Marketed to stimulate immune system but not much evidence for this, not effective to prevent colds but decr. cold Sx, no benefit in kids, avoid use w/ immunosuppresants

A

Echinacea

232
Q

May block the translocation of the cytosol androgen receptor to the nucleus, inhibitor of 5alpha-reductase!! (compared to Finasteride)?

A

Saw Palmetto!

233
Q

Ginesing decreases levels of cortisol/neurotransmitters during stress, may stimulate immune system and has what CV effects?

Active ingrediants?

A

Red ginseng may lower lipids and incr. HDLs,

Panax ginseng inhibits platelet aggregation,

Ginsenosides

234
Q

This herbal med causes nervousness, avoid taking it w. coffee or before bed, abusing it → HTN, excitability, sleeplessness, nervousness, diarrhea in AM

A

Ginseng

235
Q

KAVA is marketed for sedation/relaxation alternative to alcohol, MOA?

Adverse effects?

A

interacts w GABA receptor;

impairment of motor skills, sedation, yellow eyelids/nails (this is NOT jaundice), hepatic toxicity!!! (FDA warning, like a black box warning)

236
Q

Herb marketed for migraines/arthritis pain- Inhibits phospholipase A2, inhibits platelet serotonin release;

A

Feverfew;

drosi-/dizzi-ness, postfeverfew syndrome of insomnia, joint pain, m. aches when discont. it, heavier flow, mouth ulceration, may incr. bleeding so avoid before surgery

237
Q

6 Herbs to avoid prior to surgery?

A

Feverfew, Garlic, Ginko Biloba, Ginseng, Horse Chestnut, St. John’s Wort

238
Q

Herbs to avoid mixing w/ alcohol?

A

Valerian, KAVA

239
Q

Herbs to avoid mixing w MAO or serotonin re-uptake inhibitors?

A

St. John’s Wort, Valerian

240
Q

90% of cases of Congenital Adrenal Hyperplasia (CAH) due to?

incidence is 1/14,000 births world wide (classic) but higher where?

Nonclassic form is much MC (1-2/1000)

A

21-hydroxylase deficiency (AR);

Alaska (Yupik Eskimos), Reunion Island ;

(nonclassic more common in NYC)

241
Q

S/Sx of Classic CAH?

A

Large hyperplastic adrenals at birth, ambiguous genitalia (androgenital syndrome), high risk of adrenal insufficiency, early puberty, short stature;

Can be Simple/virilizing form (25%, have atleast 1% enzyme activity) or Salt-wasting form (75%, 0 enzyme activity)→Crisis- hypovolemic shock, hyperkalemia

242
Q

S/Sx on Nonclassic CAH

(have 20-50% enzyme activity)

A

Mild, no genital ambiguity, onset usually in adolescence, No adrenal insufficiency, androgen excess→ Oligomenorrhea, Hirsuitism, Acne

243
Q

Steroid biosynthetic pathway is a cascade that start w?

ends with?

A

Cjolesterol;

Mineralocorticoids (Aldo), Glucocorticoids, Gonadal steroids

244
Q

21-hydroxylase genes are on Chr 6 and are tightly linked w/ HLA,

what genetic changes are seen in the most severe/salt-wasting form of CAH?

A

point mutations in 75%, gene deletion (12%), Large gene cnvsns (12%)

245
Q

All states now screen newborns for 21-hydroxylase deficiency, can be effectively treated with?

A

Glucocorticoid and Mineralocorticoid Tx, genital reconstruction

246
Q

IRB- approved protocol for prenatal Dx/Tx for 21-hydroxylase deficiency to prevent female virilization

A

Start Dexamethasone when pregnancy is confirmed in at risk pts.,

Chorionic villus sampling at 8-10wks,

Amniocentesis at 16wks,

if baby is found to be Male or is an unaffected female- stop dexamethosone, only cont. in affected females

247
Q

MEN syndrome type 1

A

“3 Ps” Parathyroid neoplasia, Pituitary neoplasia, Pancreatic islet neoplasia, AD

248
Q

MEN syndrome type 2?

Genetic testing is est. in the Dx/Tx and enables?

A

“TPP”- Thyrocalcitonin (medullary carcinoma), Pheochromocytoma, Parathyroid neoplasia ;

prophylactic thyroidectomy and saves family members extensive prospective testing

249
Q

MEN 2A

genetics?

A
  • Medullary thyroid Ca. 100%
  • Pheochromocytoma 50%
  • Parathyroid neoplasia 10-20%

Point mutations of RET Proto-oncogene on Chr 10q which interacts w GDNF, site of mutation determines disease

250
Q

MEN 2A Variants?

A
  • Familial Medullary thyroid Ca. (FMTC)
  • MEN 2A w/ cutaneous lichen amyloidosis (MEN-2A/CLA)
  • MEN 2A w. Hirschsprung disease
251
Q

All pts with new medullary thyroid Ca. should be screened for??!

A

RET

252
Q

MEN 2B

A
  • Medullary thyroid Ca. 100% (early onset)
  • Pheochromocytoma 50%
  • No parathyroid disease
  • Marfinoid habitus nearly 100%
  • Intestinal ganglioneuromatosis and mucosal neuromas ~100%
253
Q

Neoplasm of parafollicular (C) cells, secrete calcitonin, CEA (can be used as markers), starts as hyperplasia→ Nodular hyperplasia → MicroCa.→ MacroCa.;

indolent in 80%, the rest- very aggresive, can metastasize early to liver, bone, lung (can obstruct airway)

A

Medullary Thyroid Ca.

(70% sporadic or can be familial- MEN 2A, MEN 2B, FMTC)

MEN2B is most aggressive then sporadic=MEN2A>FMTC

254
Q

Genetic testing for family members of pts w MEN 2A or familial medullary Thyroid Ca. (FMTC) w/ known mutation?

A
  • Normal RET analysis- excludes w/ ~100% certainty, repeat analysis in 3-5yrs (No catecholamine or Ca++ screening)
  • If they have RET mutation: Thyroidectomy before 5yo -OR- Annual Calcitonin and neck US until abnormal>thyroidectomy, cont. catecholamine and Ca++ screening
  • Screen all possibly affected fam members
255
Q

RET testing for FMTC?

A

no known RET mutation occurs in 5-8% of families so not finding mutation does not rule it out, need annual calcitonin and neck US, repeat genetic analysis when more mutations known

256
Q

RET testing for Sporadic MTC?

A

absence of FH does NOT exclude mutation, 6% germline mutation, If no mutation- hereditary excluded w 99% certainty, somatic mutations in 25%

Last Test (2 decks)

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