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GI & Hepatobiliary Flashcards

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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
Gastric Carcinoma
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 ```
26
Duodenal Atresia
congenital failure of duodenum to canalize; associated w/ Downs =polyhydramnios, 'double bubble' sign, BILIOUS vomiting
27
Meckel Diverticulum
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)
28
Volvulus
Twisting of bowel along its mesentary commonly in sigmoid colon (elderly) or cecum (young adults) =obstruction and disruption of blood supply w/ infarction
29
Intussusception
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
30
Lactose Intolerance
↓ 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
31
Celiac Disease
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
32
Dermititis Herpetiformis
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
33
Tropical Sprue
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
34
Whipple disease
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)
35
Carcinoid Tumor
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)
36
Carcinoid Syndrome
=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
37
Carcinoid Heart Disease
=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
38
Acute Appendicitis
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
39
Inflammatory Bowel Disease
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
Ulcerative Colitis
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
Crohn Disease
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
Hirschprung Disease
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
Colonic Diverticula
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
Angiodysplasia
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
Hereditary hemorrhagic telangiectasia
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
Ischemic colitis
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
Irritable Bowel Syndrome
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
Colonic Polyps
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
Hyperplastic polyps
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
Adenomatous Polyps
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
Familial Adenomatous Polyposis (FAP)
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
Gardner Syndrome
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
Turcot syndrome
FAP w/ CNS tumors (medulloblastoma & glial tumors)
54
Juvenile Polyposis
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
Puetz-Jeghers Syndrome
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
Colorectal Carcinoma
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
Annular Pancreas
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
Acute Pancreatitis
=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
Acute Pancreatitis Complications
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
Chronic pancreatitis
= 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
Pancreatic Carcinoma
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
Thin elderly patient Pw/ 2° diabetes mellitus
Not normal subset of 2° diabetes (middle age & obese) =think pancreatic carcinoma causing diabetes from destruction of islets in body/tail of pancreas
63
Biliary Atresia
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
Cholelithiasis
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
Cholelithiasis complications
``` Usually asymptomatic: 1-biliary colic 2-acute & chronic cholecystitis 3-ascending cholagitis 4-gallstone ileaus 5-gallbladder cancer ```
66
Biliary Colic
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
Acute Cholecystitis
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
Chronic Cholecystitis
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
Ascending Cholangitis
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
Gallstone Ileus
Large gallstone enters and obstructs the small bowel due to cholecystitis w/ fistula formation btwn the gallbladder-small bowel
71
Gallbladder Carcinoma
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
Elderly woman presenting w/ cholecystitis
Think gallbladder carcinoma cholecystitis is normally a disease of middle age (40-50s)
73
Alcohol-Related Liver Disease
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
Nonalcoholic Fatty Liver Disease
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
Hemochromatosis
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
Wilson Disease
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
Primary Biliary Cirrhosis
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
Primary Sclerosing Cholangitis
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
Reye Syndrome
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
Hepatic Adenoma
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
Hepatocellular Carcinoma
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
Budd-Chiari Syndrome
Liver Infarction secondary to hepatic vein obstruction Pw/ painful hepatomegaly & ascites
83
Crigler-Najjar Syndrome type I
AR= absent UGT1A1 activity =unable to conjugate bilirubin Fatal in neonatal period NOT responded to phenobarbital
84
Crigler-Najjar Syndrome type II
AD (variable penetrance) = ↓ UGT1A1 activity generally mild, occasionally kernicterus responsive to phenobarbital
85
Gilbert Syndrome
AR - ↓ UGT1A1 activity Innocuous (jaundice when stressed)
86
Dubin-Johnson Syndrome
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
Rotor Syndrome
AR = Decreased hepatic uptake and storage Innocuous ↑ Total Urinary coproporphyrin, normal isomer 1
88
Progressive Familial Intrahepatic Cholestasis
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
Cholestasis
=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
Cirrhosis
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
Cirrhosis (complications)
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
Fulminant Hepatic Failure
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
Hepatorenal Syndrome
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
hepatopulmonary syndrome
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
Hepatic Encephalopathy
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)

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