Path Test 3 Buzzwords Mccarver's Review Notes (Infancy, Liver 1,4,5)

Front 1

Alveoli develop when?

Back 1

wk 26-32, accelerated c corticosteroids

Front 2

APGAR

Back 2

Activity, Pulse, Grimace, Appearance, Respiration

Front 3

Caput Succedaneum vs Cephalhematoma

Back 3

Interstitial fluid vs hemorrhage into scalp

Front 4

Malformation vs Deformation

Back 4

M - intrinsic abnormalities occurring in the development process
D - usually from mechanical distruption, acts on an organ that was normal during development

Front 5

Oligohydramnios Sequence

Back 5

Dec amniotic fluid leading to fetal compression c flattened facies, positional abnormalities of hands/feet, hypoplastic lungs, low growth of chest wall, nodules in amnion

Front 6

CMV risk period and defects

Back 6

2nd trimester, mental retardation, microcephaly, deafness, hepatosplenomegaly

Front 7

Rubella risk period and defects

Back 7

Before conception --> 16th wk (1st 8 wks worse)
Cataracts + heart defects + deafness

Front 8

RDS defect

Back 8

Defect in pulmonary surfactant, hyaline membrane disease

Front 9

3 other complications of RDS after survival

Back 9

Ductus arteriosus, intraventricular hemorrhage, necrotizing enterocolitis

Front 10

TORCH

Back 10

Toxoplasma, Others, Rubella, Cytomegalovirus, Herpesvirus

Front 11

Necrotizing Enterocolitis (NEC)

Back 11

Preemies, sx @ first oral feeding, breakdown of mucosal barrier, migration of gut bacteria, inflammation, necrosis, sepsis/shock
Presents c bloody stools, ab distension, circulatory collapse, tx c resection of necrotic bowel

Front 12

Subependymal hemorrhage (intraventricular, germinal matrix)

Back 12

Secondary bleeing into ventricles, preemies, sudden increase in ICP + damage to brain, herniation of medulla or base of brain, fatal depression of medullary centers

Front 13

Lack of phenylalanine hydroxylase, AR

Back 13

PKU

Front 14

Normal @ birth, rising phenylalanine levels, @ 6 mo. severe mental retardation, restrict intake of phenylalanine

Back 14

PKU

Front 15

Disorder in epithelial transport affecting fluid secretion, gene located on chromosome 7

Back 15

CF

Front 16

CFTR gene, viscous mucus secretions c secondary obstruction and infection, bronchioles distended c thick mucus

Back 16

CF

Front 17

CF's 3 most common orgs of infection

Back 17

S. Aureus, H. Flu, P. Aeruginosa

Front 18

Exocrine pancreas insufficiency in 85-90% + 5-10% meconium ileus @ birth

Back 18

CF

Front 19

SIDS

Back 19

Sudden infant death under 1yo

Front 20

Portion of liver parenchyma closest to blood supply

Back 20

Zone 1

Front 21

Portion of liver parenchyma closest to THV

Back 21

Zone 3

Front 22

4 Primary insult diseases of the liver

Back 22

Viral hepatitis, alcoholic liver disease, non-alcoholic fattly liver disease, hepatocellular carcinoma

Front 23

Hepatic Conjugating Enzyme

Back 23

UGT1A1

Front 24

Most common causes of jaundice

Back 24

Bili overproduction, hepatitis, obstruction of flow of bile

Front 25

Crigler-Najjar Type 1 vs CN type 2?

Back 25

UGT1A1 absent in 1, severely reduced in 2

Front 26

Mild, fluctuating hyperbilirubinemia usually seen in periods of stress

Back 26

Gilbert Syndrome (unconjugated)

Front 27

Chronic conjugated hyperbilirubinemia + chronic recurrent jaundice asymptomatic, due to mutation in MRP2

Back 27

Dubin-Johnson syndrome (conjugated)

Front 28

Asymptomatic conjugated hyperbilirubinemia, jaundice but otherwise normal

Back 28

Rotor Syndrome

Front 29

3 Ominous complications of liver failure

Back 29

Hepatic encephalopathy, hepatorenal syndrome, hepatopulmonary syndrome

Front 30

Ass'd c elevated blood/CSF NH4 levels + rigidity, hyperreflexia, asterixis

Back 30

Hepatic encephalopathy

Front 31

Chronic liver disease + hypoxemia + intrapulmonary vascular dilations

Back 31

Hepatopulmonary syndrome

Front 32

Bridging fibrous septa, parenchymal nodules, disruption of architecture of liver

Back 32

Liver cirrhosis

Front 33

5 Major causes of Liver Cirrhosis

Back 33

Alcoholic liver disease, viral hepatitis, non alcoholic steatohepatitis, biliary disease, primary hemochromatosis

Front 34

Mechanisms of death c liver cirrhosis

Back 34

Progressive liver failure, portal HTN complications, development of hepatocellular carcinoma

Front 35

4 Major consequences of Portal HTN

Back 35

Ascites, Formation of portosystemic venous shunts, congestive splenomegaly, hepatic encephalopathy

Front 36

HELLP Syndrome

Back 36

Hemolysis, Elevated Liver enzymes, Low Platelets

Front 37

Syndrome that follows cytoreductive therapy given just before bone marrow transplant

Back 37

Liver toxicity after bone marrow transplant

Front 38

5 Effects of liver toxicity after BM transplant

Back 38

Wt gain, tender hepatomegaly, edema, ascites, hyperbilirubinemia

Front 39

2 Acute effects of GVH liver morphology

Back 39

Donor lymphos attacking epithelial cells of liver, hepatitis c necrosis + inflammation

Front 40

4 Chronic effects (>100 days post transplant) of GVD morphology of the liver

Back 40

Inflammation of portal tracts, bile duct destruction, fibrosis, endotheliitis

Front 41

2 Types of Nodular Liver Hyperplasias

Back 41

Focal Nodular Hyperplasia + Nodular Regenerative Hyperplasia

Front 42

Ass'd c anabolic steroids + oral contraceptives, well demarcated, poorly encapsulated nodule, central grey-white c depressed stellate scar, central portion contains large vessels

Back 42

Focal Nodular Hyperplasia

Front 43

Rough spherical nodules, no fibrosis, can lead to portal HTN

Back 43

Nodular Regenerative Hyperplasia

Front 44

2 Benign neoplasms of liver, which is more common, what are its features?

Back 44

Cavernous hemangioma, adenoma
CH - most common, discrete red-blue soft nodules under capsule, can be mistaken for mets

Front 45

Benign neoplasms from hepatocytes, ass'd c oral contraceptives, regresses after discontinuing OC's, can rupture (usually during pregnancy) causing severe intraperitoneal hemorrhage

Back 45

Liver Adenomas

Front 46

5 Types of Liver Malignant Tumors

Back 46

Hepatoblastoma, Angiosarcoma, Hepatocellular Carcinoma, Cholangiocarcinoma, Metastatic Tumors (M.A.C.H. HCC)

Front 47

Ass'd c vinyl chlroide, arsenic, thorotrast, may be latent for decades, highly aggressive

Back 47

Angiosarcoma of liver

Front 48

Most common primary liver CA

Back 48

Hepatocellular Carcinoma

Front 49

4 Associations c HCC

Back 49

Viral Infection (HBV, HCV), Chronic Alcoholism, Food contaminants (Aflotoxin), non alcoholic steatosis

Front 50

Previous exposure to thorotrast + invasion of biliary tract by liver fluke Opisthorchis Sinensis

Back 50

Cholangiocarcinoma

Front 51

HCC is associated c ______ infection

Back 51

HBV

Front 52

3 Gross morphologic patterns of HCC

Back 52

Unifocal, multifocal, diffusely infiltrative, paler than surrounding tissue

Front 53

What does HCC like to invade?

Back 53

Vascular channels, may invade portal vein or IVC

Front 54

Variant of HCC c the best prognosis

Back 54

Fibrolamellar variant, well differentiated cells in nests or cords separated by parallel lamellae of dense collagen bundles

Front 55

What protein is elevated in some HCC pts

Back 55

a-FP, sometimes CEA

Front 56

2 Types of Gallstones

Back 56

Cholesterol (80% is this type in western world)
Pigment (bilirubin calcium salts)

Front 57

Risks for both types of Gallstones

Back 57

Cholesterol - old, fat, female, native american, rapid weight loss, GB stasis, inborn diseases of bile acid metabolism
Pigment - Asian, chronic hemolytic syndromes, biliary infections, GI disorders

Front 58

Mucosal surface studded c minute, yellow flecks

Back 58

Strawberry GB

Front 59

Hematogenous mets to Lungs, Bones, Adrenals

Back 59

Cholangiocarcinoma

Front 60

Hematogenous spread less frequent, can recur despite liver transplant if venous invasion is found

Back 60

HCC

Front 61

GB enlarged, tense, fibrin covering on serosa, obstructing stone in neck/duct, GB lumen may contain other stones, can have a thickened GB wall or can progress to gangrenous cholecystitis

Back 61

Acute Cholecystitis

Front 62

Sequel to repeated bouts of acute cholecystitis, 90% of cases have cholelithiasis, smooth serosa, thickened GB wall

Back 62

Chronic cholecystitis

Front 63

Extensive dystrophic calcification of GB

Back 63

Porcelain GB, ass'd c marked increase in CA

Front 64

5 Complications of Acute/Chronic Cholecystitis

Back 64

Bacterial superinfection (cholangitis, sepsis), GB rupture c diffuse peritonitis, GB perforation c local abscess, Biliary enteric fiscula, aggravation of preexisting illness

Front 65

Presence of stones w/in the bile ducts in biliary tree

Back 65

Choledocholithiasis

Front 66

Bacterial infection of bile ducts, bacteria enters through sphincter of Oddi, pts have FV/chills/Ab pain/jaundice c acute inflammation of wall of bile duct

Back 66

Cholangitis

Front 67

2 Major forms of biliary atresia

Back 67

Fetal (20%, aberrant intrauterine development of extrahepatic biliary tree) and Perinatal (more common, normally developed biliary tree destroyed following birth)

Front 68

Carcinoma of GB etiology

Back 68

Woman > Men slightly, 70yo, usually a poor prognostic sign as it is found when it is not resectable and most have invaded liver when discovered

Front 69

Insidious, painless progressive jaundice, older people, risk increased c biliary tree fluke infection, primary sclerosing cholangitis, inflammatory bowel disease, choledochal cyst

Back 69

Extrahepatic Bile Duct Carcinoma