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Systems > GI & Hepatobiliary > Flashcards

GI & Hepatobiliary Flashcards

1
Q

Tracheoesopageal Fistula

A

Congenital - results in connection btwn esophagus & trachea

Proximal Atresia, Distal Fistula most common (85%)

=vomiting, polyhydramnios, abdominal distension & aspiration (4)

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2
Q

Esophageal Web

A

Thin protrusions of esophageal mucosa, most often in upper esophagus

=dysphagia w/ poorly chewed food

↑ risk of esophageal squamous cell carcinoma
-plummer-vinson syndrome

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3
Q

Plummer-Vinson Syndrome

A

Characterized by severe iron deficiency anemia, esophageal web, and beefy-red tongue due to atrophic glossitis

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4
Q

Zenker Diverticulum

A

False diverticululm of pharyngeal mucosa through an acquired defect in the muscular wall

=dysphagia, obstruction, halitosis

Arisis above the UES at junction of esophagus and pharynx (Killian’s triangle)

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5
Q

Mallory-Weiss Syndrome

A

Longitudinal laceration of mucosa at gastroesophageal junction; often caused by severe vomiting (bulimia, alcoholism)

=Painful hematemesis

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6
Q

Boerhaave Syndrome

A

rupture of esophagus leading to air in the mediastinum and subcutaneous emphysema

-hear crackles when you press on bubbles in the skin, and on auscultation of the heart

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7
Q

Esophageal Varices

A

Dilated submucosal veins in the lower esophagus secondary to portal HTN (L gastric vein backs up)

=Painless hematemesis

in 90% of cirrhotic patients, causes 1/2 of the deaths???

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8
Q

Achalasia

A

Disordered esophageal motility w/ inability to relax the LES

  • Due to damaged ganglion cells in the myenteric plexus
  • Idiopathic or 2° to insult (T. cruzi in Chagas disease)

=Dysphagia for solids and liquids, putrid breath, ‘bird-beak’ sign, high LES pressure on esophageal manometry
↑ risk of esophageal SCC

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9
Q

GERD

A

reflux of acid from the stomach due to reduced LES tone

Alcohol, tobacco, obesity, fat-rich diet, caffeine, hiatal hernia

=heartburn, asthma (adult onset) & cough, damage to teeth enamel, ulceration w/ stricture & Barrett’s esophagus (late)

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10
Q

Hiatal Hernia

A

stomach herniating through esophageal hiatus of the diaphragm

Sliding = hourglass appearance & GERD due to gastric tissue above LES/diaphragm

Paraesophageal = (less common), bowel sounds in lung fields; may lead to lung hypoplasia; can become strangulated*

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11
Q

Barrett Esophagus

A

Metaplasia of the lower esophagus from nonkeratinized stratified squamous epithelium→ nonciliated columnar epithelium w/ goblet cells (alcian blue stain)
-seen in 10% of PT w/ GERD

=response of esophageal stem cells to acidic stress & may progress to dysplasia and adenocarcinoma

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12
Q

Esophageal Adenocarcinoma

A

malignant proliferation of glands, most common esophageal carcinoma in West

Arises from preexisting Barret esophagus (=usually in lower 1/3 of esophagus)

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13
Q

Esophageal SCC

A

malignant proliferation of squamous cells, most common esophageal carcinoma worldwide

Arises from esophageal irritation (hot tea, achalasia, alcohol, tobacco, esophageal web, or injury) & usually in upper 2/3 or esophagus

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14
Q

Esophageal Carcinoma

A

Adenocarcinoma in lower 1/3, most common in West
SCC in upper 2/3 (middle 1/3 most common), worldwide

Lymph Node Spread
Upper 1/3 - cervical
Middle 1/3 - Mediastinal or trachebronchial
Lower 1/3 - celiac & gastric

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15
Q

Gastroschisis

A

Congenital malformation of the anterior abdominal wall leading to exposure of abdominal contents

= a hole - can clearly see the intestine

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16
Q

Omphalocele

A

Persistent herniation of bowel into the umbilical cord due to failure of herniated intestines to return to body cavity in development.

contents covered by peritoneum & amnion of umbilical cord

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17
Q

Pyloric Stenosis

A

Congenital hypertrophy of pyloric smooth muscle, more common in males and classically presents 2 weeks after birth

=projectile NONBILIOUS vominting, visible peristalsis, olive-like mass

tx-myotomy (removal of hypertrophic muscle)

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18
Q

Acute Gastritis

A

Acidic damage to the stomach mucosa due to an imbalance of acidic environment and mucosal defense

Acid damage results in superficial inflammation, erosion or ulcer

Risk factors = sever burn or shock (↓ blood supply), chemo, NSAIDS (↓PGE), heavy alcohol consumption, ↑ intracranial pressure (=vagal strain=ACh=↑acid production)

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19
Q

Chronic Gastritis

A

Chronic inflammation of stomach mucosa
1-Chronic H. pylori gastritis

2-Chronic autoimmune gastritis

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20
Q

Chronic H. Pylori Gastritis

A 
in antrum (90% of chronic gastritis)
=epigastric pain, ↑ulceration risk, MALT lymphoma, & gastric adenocarcinoma
-triple tx=PPI, clarithromycin, & amoxicillin or metrinidazole
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21
Q

Chronic Autoimmune Gastritis

A

in body/fundis
-autoimmune destruction of gastric parietal cells mediated by T cells (type IV hypersensitivity)
=atrophy of mucosa w/ intestinal metaplasia; achlorhydria w/ ↑ gastrin levels (G cell hyperplasia); Megaloblastic (pernicious) anemia; ↑risk of gastric adenocarcinoma (intestinal type)

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22
Q

Peptic Ulcer Disease

A

Solitary mucosal ulcer

-Proximal duodenum (90%) almost always due to H. pylori
=epigastric pain that improves w/ meals
-Usually anterior, but posterior rupture may lead to bleeding from gastroduodenal artery or acute pancreatitis
*almost never malignant

-Gastric (10%)
=epigastric pain that worsens w/ meals
-Usually in lesser curvature or antrum, rupture = risk of bleeding from L gastric artery
should always be biopsied (benign = small, punched-out & w/o heaping at margins)

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23
Q

Intestinal Type Gastric Carcinoma

A

presents as large, irregular ulcer w/ heaped up margins; most commonly at lesser curvature of the antrum; more common than diffuse type

RF-intestinal metaplasia, nitrosamines (smoked foods), Blood Type A

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24
Q

Diffuse Type Gastric Carcinoma

A

signet ring cells that diffusely infiltrate the gastric wall; desmoplasia results in thickening of stomach wall

*not associated w/ H. pylori, intestinal metaplasia or nitrosamines

signet ring cells= mucin production by tumor cells pushes nucleus to the outside of the cell
desmoplasia leads to Linitis plastica (from fibrous build up)

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25
Q

Gastric Carcinoma

A

Malignant proliferation of surface epithelial cells (intestinal type more common than diffuse type)

Pw/ WL, abdominal payin, anemia, early satiety, and rarely w/ acanthosis nigicans or Leser-Trelat sign (keratoses over skin)

spread to Virchow node (L supraclavicular), Sr. Mary Joseph nodule (periumbilical) w/ intestinal type
Krakenburg tumor (bilateral ovaries) w/ diffuse type
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26
Q

Duodenal Atresia

A

congenital failure of duodenum to canalize; associated w/ Downs

=polyhydramnios, ‘double bubble’ sign, BILIOUS vomiting

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27
Q

Meckel Diverticulum

A

TRUE diverticulum (outpouching of all 3 layers) due to failure of vitelline duct to involute

Rule of 2s: 2% of population, 2 inch long, w/in 2 feet of ileocecal valve, presents w/in 1st 2 years of life (but usually asymptomatic)

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28
Q

Volvulus

A

Twisting of bowel along its mesentary commonly in sigmoid colon (elderly) or cecum (young adults)

=obstruction and disruption of blood supply w/ infarction

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29
Q

Intussusception

A

telescoping - proximal segment of bowel into distal segment

Associated w/ a leading edge (focus of traction)
Child- terminal ileum into cecum from lymphoid hyperplasia
Adult - tumor is most common cause

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30
Q

Lactose Intolerance

A

↓ function of lactase enzyme in brush border of enterocytes; rarely congenital = usually acquired, can be temporary after small bowel infection

= abdominal distension & diarrhea upon consumption of dairy products because undigested lactose is osmotically active

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31
Q

Celiac Disease

A

Immune-mediated damage of small bowel villi due to gluten exposure by T helper cells (deaminated gliadin is presented)

*damage mainly in duodenum

Child - abdominal distenstion, diarrhea & failure to thrive
Adults - chronic diarrhea & bloating

Dermatitis herpetiformis may arise on skin

small bowel carcinoma & T-cell lymphoma are late complications that present as refractory disease despite good dietary control

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32
Q

Dermititis Herpetiformis

A

Seen in Celiac disease

=small, herpes-like vesicles arise on skin due to IgA deposition at the tips of dermal papillae; resolves w/ gluten-free diet

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33
Q

Tropical Sprue

A

Damage to small bowel villi due to an unknown organism resulting in malabsorption

Occurs in tropical regions and arises after infectious diarrhea = responds to antibiotics

*damage mainly in jejunum and ileum

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34
Q

Whipple disease

A

systemic tissue damage characterized by macrophages loaded w/ Tropheryma whippelii, which is only partially destroyed

=Foamy (Pas+) macrophages in small bowel lamina propria

Results in fat malabsorption & steatorrhea (macrophages compress lacteals = chylomicrons can’t be transferred to lymph)

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35
Q

Carcinoid Tumor

A

Low-grade malignancy; malignant proliferation of neuroendocrine cells (=granules positive for chromogranin)

Small Bowel = most common site

Often secrete seratonin which can lead to carcinoid syndrome if tumor metastasizes (and seratonin can bypass the liver)

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36
Q

Carcinoid Syndrome

A

=bouts of bronchospasm, diarrhea & flushing of skin which may be triggered by alcohol or emotional stress (stimulation of 5-HT release)

seen in some metastatic carcinoid tumors

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37
Q

Carcinoid Heart Disease

A

=right-sided valvular fibrosis leading to tricuspid regurgitation and pulmonary valve stenosis

(no left-sided lesions due to MAO in lungs)

seen in some metastatic carcinoid tumors

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38
Q

Acute Appendicitis

A

Acute inflammation of the appendix; related to obstruction of the appendix by lymphoid hyperplasia (children) or fecalith (adult)

=periumbilical pain which localizes to the RLQ (McBurney point), fever and nausea
rupture = peritonitis w/ guarding and rebound tenderness

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39
Q

Inflammatory Bowel Disease

A

Chronic, relapsing inflammation of the bowel; possibly due to abnormal immune response to enteric flora

Dx of exclusion; symptoms mimic other causes of bowel inflammation

40
Q

Ulcerative Colitis

A

IBD involving the mucosa & submucosa of the colon

  • begins in rectum & can extend proximally to the cecum
  • involvement is generally continuous

=LLQ pain w/ bloody diarrhea

  • crypt abscesses w/ neutrophils (GRANULOCYTES)
  • ‘lead-pipe’ sign on imaging

= toxic megacolon and ↑ risk of carcinoma

Smoking is protective for UC

41
Q

Crohn Disease

A

IBD w/ full-thickness inflammation w/ knife-like fissures anywhere in the GI tract from mouth to anus (terminal ileum most common, rectum least common) w/ skip lesions, can form fistulas

=RLQ pain w/ non-bloody diarrhea

  • lymphoid aggregates w/ GRANULOMAS
  • Cobblestone mucosa, creeping fat, strictures (‘string-sign’)

=nutrient malabsorption, calcium oxalate nephrolithiasis, fistulas, carcinoma (if colonic disease present)

Smoking ↑ risk of CD

42
Q

Hirschprung Disease

A

Defective relaxation and peristalsis of rectum and distal sigmoid colon due to congenital failure of ganglion cells (neural crest-derived) to descend into myenteric and submucosal plexus

Associated w/ Down Syndrome

=failure to pass meconium, empty rectal vault on digital exam, massive dilation of bowel proximal to obstruction w/ rupture risk

**rectal suction biopsy reveals lack of ganglion cells

Tx=resection of involved bowel

43
Q

Colonic Diverticula

A

False diverticulum (M & SM through MP), related to wall stress

Usually asymptomatic, complications include:
rectal bleeding (hematochezia) - bright red blood being passed
Diverticulitis w/ appendicitis-like symptoms (but LLQ pain)
Fistula-colovesicular fistula presents w/ air or stool in urine

44
Q

Angiodysplasia

A

Acquired malformation of mucosal & submucosal capillary beds
Usually arise in cecum & right colon due to high wall stress

Rupture classically presents as hematochezia in older adults

45
Q

Hereditary hemorrhagic telangiectasia

A

AD = thin-walled blood vessels, especially in the mouth and GI

Rupture presents w/ bleeding

can see dark spots on lips from ruptured telangiectasias

46
Q

Ischemic colitis

A

Ischemic damage to colon, usually at splenic flexure (watershed area of the SMA), SMA artherosclerosis = most common cause

=postprandial pain & WL, infarction results in pain & bloody diarrhea

47
Q

Irritable Bowel Syndrome

A

Relapsing abdominal pain w/ bloating, flatulence, & change in bowel habits that improves w/ defecation

Related to disturbed intestinal motility, no pathologic change IDed

↑ dietary fiber may improve symptoms

48
Q

Colonic Polyps

A

Raised protrusions of colonic mucosa

hyperplastic polyps w/ no malignant potential
adenomatous polyps which are benign, but premalignant

All polyps are removed and examined microscopically because both types (hyperplastic & Adenomatous) look identical on colonoscopy

49
Q

Hyperplastic polyps

A

due to hyperplasia of glands
*classically show ‘serrated’ appearance on microscopy

Most common type, usually arise in left colon

benign w/ no malignant potentials

50
Q

Adenomatous Polyps

A

due to neoplastic proliferation of glands

benign, but premalignant**

may progress to adenocarcinoma via Adenoma-carcinoma sequence:

  • APC mutation (↑risk of polyp formation)
  • K-ras mutation (leads to polyp formation)
  • p53 mutation & ↑COX (allow for progression to carcinoma)

greatest risk for progression to carcinoma is related to size (>2cm), also sessile growth & villous histology

51
Q

Familial Adenomatous Polyposis (FAP)

A

AD, characterized by 100s-1000s of adenomatous colonic polyps

Due to inherited APC mutation (on chromosome 5)

Colon and rectum removed prophylactically; otherwise, almost all patients develop carcinoma by 40

Gardner and Turcot syndrome involve additional symptoms

52
Q

Gardner Syndrome

A

FAP w/ fibromatosis & osteomas

fibromatosis = non-neoplastic proliferation of fibroblasts, arise in retroperitoneu & locally destroy tissue

osteoma - benign tumor of bone, usually in the skull

53
Q

Turcot syndrome

A

FAP w/ CNS tumors (medulloblastoma & glial tumors)

54
Q

Juvenile Polyposis

A

Sporadic hamartomatous polyp (benign) that arises in children <5 characterized by multiple juvenile polyps in the stomach/colon; large # increase the risk of progression to carcinoma

Usually presents as a solitary rectal polyp that prolapses & bleeds

55
Q

Puetz-Jeghers Syndrome

A

AD = hamartomatous polyps throughout the GI tract & mucocutaneous hyperpigmentation (freckle-like spots) on lips, oral mucosa & genital skin

↑risk for colorectal, breast & gynecologic cancer

56
Q

Colorectal Carcinoma

A

CA arising from colonic or rectal mucosa; 3rd most common site & 3rd most common cause of cancer-related deaths (M&W)

Most commonly arise from adenoma-carcinoma pathway, but microsatellite instability (ex HNPCC) is a 2nd important molecular pathway

can grow anywhere along the entire length of the colon

  • left-sided grow as ‘napkin-ring’ lesion & present w/ ↓ stool caliber, LLQ pain, & blood-streaked stool
  • right-sided grow as raised lesions & PW/ iron-deficiency anemia & vague pain

*older adults w/ iron deficiency anemia have colorectal carcinoma until proven otherwise**

Associated w/ and ↑ risk for Streptococcus bovis endocarditis

CEA is useful for assessing Tx response & recurrence
NOT useful for screening

57
Q

Annular Pancreas

A

Developmental malformation in which the ventral pancreatic bud abnormally encircles 2nd part of duodenum, forms a ring of pancreatic tissue that may cause duodenal narrowing & leads to risk of duodenal obstruction

58
Q

Acute Pancreatitis

A

=inflammation & hemorrhage of pancreas due to autodigestion of pancreatic parencyma by premature activation of pancreatic enzymes (spec. trypsin) resulting in liquefactive hemorrhagic necrosis of pancreas & fat necrosis of peripancreatic fat

Most commonly due to alcohol (contracts sphincter of oddi) & gallstones

  • Epigastric ab pain that radiates to back, N/V, periumbilical & flank hemorrhage (as necrosis spreads)
  • ↑ serum amylase & lipase (L more specific to pancreas)
  • hypocalcemia = poor prognostic indicator (Ca consumed during saponification of fat necrosis) as it indicates more severe necrosis
59
Q

Acute Pancreatitis Complications

A

Shock - due to peripancreatic hemorrhage (from digestion of vessels) % fluid sequestration

Pancreatic pseudocyst - formed by fibrous tissue surrounding liquefactive necrosis, Pw/ ab mass & persistently ↑ amylase

Pancreatic abcess - often due to E. coli Pw/ ab pain, high fever & persistently ↑ amylase

DIC & ARDS

60
Q

Chronic pancreatitis

A

= Fibrosis of pancreatic parenchyma, most often 2° to recurrent acute pancreatitis - most commonly due to alcohol (adult) or CF (child)

  • epigastric pain that radiates to the back
  • Pancreatic insufficiency = malabsorption w/ steatorrhea and ADEK deficiency *Amylase/lipase not useful markers as pancreas is not producing sufficient enzymes
  • Dystrophic calcification of pancreated parenchyma on imaging shows ‘chain of lake’ pattern from dilation of ducts between damaged/fibrotic areas *2° diabetes mellitus = late complication from islet destruction
  • ↑ risk of pancreatic carcinoma
61
Q

Pancreatic Carcinoma

A

Adenocarcinoma arising from the pancreatic ducts, most commonly seen in elderly (avg ~70yo)
Major RF = smoking & chronic pancreatitis

  • epigastric pain & WL
  • obstructive jaundice w/ pale stools and palpable gallbladder (head)
  • 2° diabetes mellitus (body/tail)
  • Pancreatitis
  • Migratory thrombophlebitis Pw/ swelling, erythema & tenderness in extremities (Trousseau sign)
  • CA19-9 = serum tumor marker
62
Q

Thin elderly patient Pw/ 2° diabetes mellitus

A

Not normal subset of 2° diabetes (middle age & obese)

=think pancreatic carcinoma causing diabetes from destruction of islets in body/tail of pancreas

63
Q

Biliary Atresia

A

Failure to form or early destruction of extrahepatic biliary tree

=biliary obstruction w/in the 1st 2 months of life

Pw/ jaundice & progression to cirrhosis

64
Q

Cholelithiasis

A

Solid, round stones in gallbladder due to cholesterol or bilirubin

Cholesterol stones (90% - yellow) usually radiolucent
RF=age (40s), estrogen, Native American ethnicity, CD, cirrhosis, clofibrate (↑ cholesterols)
Bilirubin stones (pigmented) usually radiopaque
RF=extravascular hemolysis & biliary tract infection (E. coli, Ascaris lumbricoides, Clohorchis sinesis)
65
Q

Cholelithiasis complications

A 
Usually asymptomatic:
1-biliary colic
2-acute & chronic cholecystitis
3-ascending cholagitis
4-gallstone ileaus
5-gallbladder cancer
66
Q

Biliary Colic

A

Waxing & waning RUQ pain due to gallbladder contracting against a stone lodged in the cystic duct

May result in acute pancreatitis or obstructive jaundice

Symptoms relieved if the stone passes

67
Q

Acute Cholecystitis

A

Acute inflammation of the gallbladder wall as an impacted stone in the cystic duct results in dilation w/ pressure ischemia, bacterial overgrowth (E. coli or Klebsiella) & inflammation

Pw/ RUQ pain (often radiates to R scapula), fever w/ ↑WBC, N/V, ↑serum ALP (from duct damage)

risk of rupture if left untreated

68
Q

Chronic Cholecystitis

A

Chronic inflammation of the gallbladder due to chemical irritation from longstanding cholelithiasis (w/ or w/o superimposed bouts of acute cholecystitis) resulting in herniation of gallbladder mucosa into the muscular wall (Rokitansky-Aschoff sinus)

Pw/ vague RUQ pain, especially after eating

Porcelin gallbladder = late complication (shrunken, hard gallbladder due to chronic inflammation, fibrosis & dystrophic calcification) = ↑ risk of carcinoma

Tx - cholecystectomy, especially if porcelain gallbladder present

69
Q

Ascending Cholangitis

A

Bacterial infection of the bile ducts usually due to ascending infection w/ enteric Gram - bacteria

Pw/ sepsis, jaundice & abdominal pain

↑ incidence w/ choledocholithiasis (stone in biliary duct) = ↓ bile flow allowing for bacteria to move up duct more easily

70
Q

Gallstone Ileus

A

Large gallstone enters and obstructs the small bowel due to cholecystitis w/ fistula formation btwn the gallbladder-small bowel

71
Q

Gallbladder Carcinoma

A

Adenocarcinoma arising from the glandular epithelium that lines the gall bladder wall - poor Px

RF = gallstones, esp if complicated by porcelain gallbladder

Classically Pw/ cholecystitis in an elderly woman

72
Q

Elderly woman presenting w/ cholecystitis

A

Think gallbladder carcinoma

cholecystitis is normally a disease of middle age (40-50s)

73
Q

Alcohol-Related Liver Disease

A

Damage to hepatic parenchyma due to consumption of alcohol
*↑ AST,ALT in 2:1 pattern
Fatty liver = accumulation of fat in hepatocytes (=heavy/greasy liver)

Alcoholic hepatitis - chemical injury to hepatocytes (binge drinking)
=swelling of hepatocytes w/ Mallory body formation, necrosis & acute inflammation; mediated by acetaldehyde
Pw/ painful hepatomegaly & ↑ liver enzymes

74
Q

Nonalcoholic Fatty Liver Disease

A

Fatty changes, hepatitis &/or cirrhosis that develop w/o exposure to alohol - associated w/ obesity

Dx of exclusion: ALT,AST in 1.15:1 pattern

75
Q

Hemochromatosis

A

AR defect in iron absorption (HFE gene) or chronic transfusion

=excess body iron leading to deposition in tissue (hemosiderosis) & organ damage (hemochromatosis)

Pw/ (late, in adults) classic triad: Cirrhosis, 2° diabetes mellitus, & Bronze skin

↑ risk HCC
Tx = phlebotomy
Labs = ↑ ferritin, serum iron & % sat, ↓ TIBC
Liver biopsy = accumulation of brown pigment in hepatocytes (prussian blue stain differentiates from lipofuscin)

76
Q

Wilson Disease

A

AR defect in ATP-mediated hepatocyte copper absorption (ATP7B)

=lack of copper transport into bile & incorporation into ceruloplasmin

Cu builds up in hepatocytes, leaks into serum & deposits into tissues = production of hydroxyl free radicals & tissue damage

Pw/ (in children) Cirrhosis, Kayser-Fleisher rings, & Neurologic manifestations (behavioral changes, dementia, chorea)

↑ risk of HCC
Tx= copper chelation w/ D-penicillamine
Labs = ↑urinary copper & copper in liver, ↓ ceruloplasmin

77
Q

Primary Biliary Cirrhosis

A

Autoimmune granulomatous destruction of intrahepatic bile ducts

Pw/ features of obstructive jaundice; cirrhosis = late complication

Classically arises in women (avg-40) & associates w/ other autoimmune diseases

Antimitochondrial Ab (AMA) present

78
Q

Primary Sclerosing Cholangitis

A

Inflammation & fibrosis of intra & extrahepatic bile ducts

Pw/ obstructive jaundice; cirrhosis = late complication

Periductal fibrosis w/ ‘onion-skin’ appearance & ‘beaded-string’ appearance of dilated uninvolved regions

Classically in men & associated w/ UC (p-ANCA often +)

↑ risk of cholangiocarcinoma

79
Q

Reye Syndrome

A

Fulminant liver failure & encephalopathy in child w/ viral illness (& fever) who take aspirin (likely related to mitochondrial damage in hepatocytes)

Pw/ hypoglycemia, elevated liver enzymes, N/V & may progress to coma and death

80
Q

Hepatic Adenoma

A

Benign tumor of hepatocytes

Associated w/ oral contraceptive use; regress upon drug cessation

Risk of rupture & intraperitoneal bleeding (esp in pregnancy) tumors are subcapsular & grow w/ exposure to estrogen

81
Q

Hepatocellular Carcinoma

A

Malignant tumor of hepatocytes - often detected late because symptoms are masked by cirrhosis = poor prognosis
α-fetoprotein = serum tumor marker
-hepatomegaly w/ a nodular free edge

RF = Chronic hepatitis, Cirrhosis, Aflatoxins (from Aspergillis)

↑ risk of Budd-Chiari syndrome

Metastasis to liver is more common than primary liver tumors, commonly come from colon, pancreas, lung & breast carcinomas

82
Q

Budd-Chiari Syndrome

A

Liver Infarction secondary to hepatic vein obstruction

Pw/ painful hepatomegaly & ascites

83
Q

Crigler-Najjar Syndrome type I

A

AR= absent UGT1A1 activity

=unable to conjugate bilirubin
Fatal in neonatal period

NOT responded to phenobarbital

84
Q

Crigler-Najjar Syndrome type II

A

AD (variable penetrance) = ↓ UGT1A1 activity

generally mild, occasionally kernicterus

responsive to phenobarbital

85
Q

Gilbert Syndrome

A

AR - ↓ UGT1A1 activity

Innocuous (jaundice when stressed)

86
Q

Dubin-Johnson Syndrome

A

AR - MRP2 mutation (canicular multidrug resistance protein2 )
Impaired excretion of bili glucuronides due to mutation

Black liver due to pigmented cytoplasmic globules

Innocuous, may have recurrent jaundice

Total urinary coproporphyrin normal w/ ↑ isomer 1

87
Q

Rotor Syndrome

A

AR = Decreased hepatic uptake and storage

Innocuous

↑ Total Urinary coproporphyrin, normal isomer 1

88
Q

Progressive Familial Intrahepatic Cholestasis

A

AR = defects in biliary epithelial transport

PFIC-1 (Byler Disease) = canicular ATPase deficiency (ATP8B1)
PFIC-2 = Bile salt export pump deficiency (ABCB11)
PFIC-3 = Phospatidylcholine (lecithan) transfer deficiency (ABCB4)

Pw/ progressive cholestasis in childhood leading to failure to thrive, hepatic failure, cirrhosis in adolescence = need liver transplant
=Cholestasis, Fat malabsorption, ADEK deficiency, Osteopenia

Histologically, see rosette formation of cells around bile

89
Q

Cholestasis

A

=Pruritis, jaundice, ‘clay-colored’ stools, dark urine, Xanthomas (from hyperlipidemia), Osteoporosis (from Vit D deficiency)

Hepatocellular Dysfunction = Virus & Alcoholic Liver Disease…
Post hepatic dysfunction = gallstones & pancreatic cancer…

90
Q

Cirrhosis

A

Hepatocyte necrosis, progressive fibrosis, w/ regenerative hepatocyte nodules = abnormal vascular connection & disruption of hepatocyte function

Alcoholic liver disease, viral hepatitis, obesity (fatty liver disease)

Mediated by TGF-β from stellate (Kuppfer) cells

Micronodular (3mm) - hepatitis B/C, Wilson Disease, α1-antitrypsin

91
Q

Cirrhosis (complications)

A

Decompensated Cirrhosis = Portal HTN, hepatorenal syndrome, liver failure & hepatic encephalopathy

Portal HTN = esophageal varices, hemorrhoids, Caput medusae, Ascites, congestive hypersplenomegaly

Ascites - check Serum Ascites Albumin Gradient (SAAG) >1.1= portal HTN, <1.1=something else (nephrotic syndrome, TB, peritoneal disease)

92
Q

Fulminant Hepatic Failure

A

Early onset w/ no previous hepatic disease

= coagulopathy, encephalopathy & multi-organ failure w/ high mortality

histologically - confluent necrosis w/ collapse of normal hepatic structure

e.x. ingestion of hepatotoxin, Hep E in pregnancy, Hep D in superinfection

93
Q

Hepatorenal Syndrome

A

Refers to acute renal failure that occurs in the setting of cirrhosis or fulminant liver failure

alteration in blood flow/vessel tone in intestinal system (vasodilation) results in altered blood flow to the kidneys (vasoconstriction)

↑BUN/creat, ↓ urinary [Na}, normal urinary sediment

Type 1 HRS - rapidly progressing, doubling of creat to >2.5 in 1.5 mg/dl

94
Q

hepatopulmonary syndrome

A

Clinical triad of chronic liver disease, hypoxemia & intra-pulmonary vascular dilation (NO appears to be key mediator)

=Ventilation-perfusion mismatch (due to rapid blood flow through dilated vessels) (=blood shunting in a way)

95
Q

Hepatic Encephalopathy

A

Neuropsychiatric abnormality in setting of acute/chronic liver failure

↑NH3 brain diffusion w/ edema = cause

= spatial perception distortion, sleep disturbances, personality change, asterixis, abnormal EEG, lethargy, coma, decerebrate posture

reversible in chronic liver disease
80% lethal w/o transplant in fulminant hepatitis

Lactulose used to treat (draws water and toxins into the colon)

Systems (4 decks)

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