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312 Cards in this Set

  • Front
  • Back
what side is the aorta on compared to the IVC?
what does the falciform ligament connet?
liver to anterior abdonminal wall
what does the falciform ligmanet contain?
ligamentum teres
what is the falciform ligament derived from?
the fetal umbilical vein
what does the hepatoduodenal ligament connect?
liver to duodenum; it also connect the greater and lesser sacs
what does the hepatoduodenal ligament contain?
portal triad: hepatic artery, portal vein and common bile duct
what does the gastrohepatic ligmanet connect?
liver to lesser curve of the stomach; separates the right greater and lesser sacs
what does the gastrohepatic ligament contain?
gastric arteries
what does the gastrocolic ligament connect?
greater curvature and transverse colon; it is part of the greater omentum
what does the gastrocolic ligament contain?
gastroepiploic arteries
what does the gastrosplenic ligament connect
greater curvature and spleen; separates left greater and lesser sacs
what does the gastrosplenic ligament contain?
short gastrics
what does the splenorenal ligament connect?
spleen to posterior abdmonial wall
what does the splenorenal ligament contain?
splenic artery and vein
what are the 4 layers of the gut wall?
1. mucosa, 2. submucosa, 3. muscularis externa, 4. serosa/adventitia
what is contained in the mucosa layer of the gut wall?
1. epithelium (absorption), 2. lamina propria (support), 3. muscularis mucosae (motility)
what is contained in the submucosa layer of the gut wall?
submucosal nerve plexus = Meissner's
what is contained in the muscularis externa?
Myenteric nerve plexus = Auerbach's
what is the frequency of basal electric rhythm of the stomach, duodenum and ileum?
Stomach = 3 waves/min; Duodenum = 12 waves/min; Ileum = 8-9 waves/min
what is the function of Auerbach's plexus?
Myenteric - controls motility along entire gut wall, contains cell bodies of PNS terminal effector neurons
where is Auerbach's plexus located?
between inner ciruclar and outer longitudinal layers of smooth muscle (Auerbach's is on the Autside)
what is the function of Meissner's plexus?
Submucosal - regulates local secretions, blood flow and absorption; contains cell bodies of some PNS ternimal effector neurons
where is Meissner's plexus located?
between mucosa and inner layer of smooth muscle
what structures are derived from the embryonic foregut?
stomach to proximal duodenum, liver, gallbladder and pancreas
what artery and nerve innervate the embryonic foregut?
celiac artery and the vagus nerve
what is the vertebral level of the foregut?
what structures are derived from the embryonic midgut?
distal duodenem to proxmial 2/3 of transverse colon
what artery and nerve innervate the embryonic midgut?
SMA and vagus nerve
what is the vertebral level of the midgut?
what structures are derived from the hindgut?
distal 1/3 of transverse colon to upper portion of rectum; splenic flexure is a watershed region
what artery and nerve innervate the hindgut?
IMA and pelvic nerve
what is the vertebral level of the hindgut?
what are the 3 branches of the celiac trunk?
common hepatic, splenic and left gastric
what forms the pectinate line?
it is formed where the hindgut meets ecoterm
what can occur above the pectinate line?
internal hemorroids, adenocarcinoma
what can occur below the pectinate line?
external hemorrhoids and SCC
what supplies and drains above the pectinate line?
superior rectal artery (branch of IMA) and drained by the superior rectal vein to the inferior mesenteric to the portal system = a sign of portal HTN
what supplies and drains below the pectinate line?
inferior rectal artery (branch of internal pudendal), drained by inferior rectal vein to the internal pudenal vein to the internal iliac vein to IVC
what does the apical surface of hepatocytes face?
bile canaliculi
what does the basolateral surface of hepatocytes face?
what are the 3 zones of the liver?
Zone I: periportal zone (1st affected by viral hepatitis), Zone II: intermediate zone; Zone III: pericentral vein (centrilobular zone): affected 1st by ischemia, contains P450 system, most senstiive to toxic injury and alcoholic hepatiits
what zone of the liver is most sensitive to viral hepatitis
Zone I: periportal zone
what zone of the liver is most sensitive to alcoholic hepatitis?
Zone 3: pericentral (centrilobular) vein zone
what zone of the liver is most senstiive to toxic injury?
Zone 3: pericentral (centrilobular) vein zone
what zone of the liver is affected 1st by ischemia?
Zone 3: pericentral (centrilobular) vein zone
what combines to form the common bile duct?
common hepatic duct and the cystic duct
how is the femoral region's vessels organized?
go lateral to medical to find your NAVEL: nerve-artery-vein-empty space-lymphmatics
what is contained in the femoral triangle?
femoral vien, artery and nerve
what is contained in the femoral sheath?
femoral vein, artery and canal (deep to inguinal lymph nodes) but NOT the femoral nerve
what is the most common hiatal hernia?
sliding; GE junction is displaced "hourglass stomach"
where is an indirect inguinal hernia found?
through the INternal (deep) inguinal ring, external (superficial) inguinal ring and INto the scrotum; enters lateral to the inferior epigastric artery
why does an indirect inguinal hernia form?
forms in INfants due to failure of processus vaginalis to close; much more common in males
where is a direct inguinal hernia found?
protrudes through the inguinal triangle (Hasselbach's), directly through the abdominal wall; goes through the external (superficial) inguinal ring only; occurs in older men
where does an indirect hernia lie in relation to the inferior epigastric artery?
MDs don't LIe: Medial = direct; Lateral = indirect
where does a direct hernia lie in relation to the inferior epigastric artery?
MDs don't LIe: Medial = direct; Lateral = indirect
where is a femoral hernia found?
protrudes below inguinal ligament through the femoral canal below and lateral to the pubic tubercle; more common in women
which hernia is more common in women?
femoral hernia
what outlines Hasselbach's triangle?
1 inferior epigastric artery, 2. lateral border of rectus abdmonis, 3. inguinal ligament
what are Peyer's patches?
unencapsulated lymphoid tissue in the lamina propria and submucosa of the small intestin; contains speclized M cells that take up antigens
where does saliva scretions come from?
parotid - serious mostly; submandibular, submaxillary and sublingual - mostly mucus secretions
what is the function of saliva?
alpha-amylase - begins to digest start, inactivated by low pH; bicarb neutralizes bacterial acids; mucins lubricate food; antibacterial secretory products; GFs that promote epithelial renewal
what type of solution is secreted when saliva has a low flow rate?
hypotonic - more time to reabsorb H20
what type of solution is secreted when saliva has a high flow rate?
isotonic - less time to reabsorb H20
where does CN7 run?
through the parotid gland
what stimulates saliva secretion?
both SNS (T1-T3 superior cervical ganglion) and PNS (faical and glossopharyngeal nerve)
what are Brunner's glands?
secrete alkaline mucus to neutralize acid contents enterin gthe duodenum from the stomach; located in duodenal submucosa (only GI submucosal glands)
when is hypertrophy of Brunner's glands seen?
in peptic ulcer disease
what do parietal cells secrete?
IF and gastric acid
what stimulates gastric acid secretion?
histamine, ACh and gastrin
what inhibits gastric acid secretion?
somatostatin, GIP and prostaglandin
what is secreted by chief cells?
what stimulates pepsin secretion?
vagal stimulation and local acid
what is screted by mucosal cells?
what stimulates HCO3- secretion?
where are parietal cells and chief cells located in the stomach?
in the body of the stumach
where are mucus cells and G cells located in the stomach?
in the antrum
which cells are blocked by atropine?
parietal cells, not G cells even though both are stimulated by the vagus nerve; G cells works via GRP, not ACh
what does cimetidine do?
blocks histamine from stimulating gastric acid secretion
what activates trypsinogen?
converted to trypsin by enterokinase/enteropeptidase which is secreted by the duodenal mucosa
what type of bond does salivary amylase break?
hydrolyzes alpha-1,4 linkages to yield disaccharides
what is the rate limiting step of carbohydrate digestion?
oligosaccharide hydrolases located at the brush border of the intestine
what do enterocytes absorb?
only monosaccharides glucose and galactose by SGLT1 (Na dependent) and fructose by GLUT5
where is iron absorbed?
as Fe2+ in the duodenum
where is folate absorbe?
in the jejunum
where is B12 absorbe?
in the terminal ileum with bile acids
what is bile composed of?
bile saids (bile acids conjugated to glycine or taurine = water insoluble), phopholipids, cholesterol, bilirubin, watera nd ions
how is cholesterol excreted?
in bile
where do most salivary gland tumors occur?
in the parotid gland
what is the most common salivary gland tumor?
pleomorphic adenoma: painless, movable mass, benign with high rate or recurrence
what is warthin's tumor?
benign salivary tumor due to heterotorphic salivary galnd tissue traped in a laymp node and surrounded with lymphatic tissue
what is the most common malignant salivary gland tumor?
mucoepidermoid carcinoma
define achalasia
failure of relaxation of the LES due to loss of myenteric (Auerbach's plexus)
barium swallow shows dilated esophagus with an area of distal stenosis
what is associated with achalasia?
increased risk of esophageal carcinoma
"bird's beak" on barium swallow
what can cause secondary achalasia?
chagas disease
what type of esophageal dysmotility is associated with scleroderma (CREST)?
low pressure proximal to LES
heartburn and regurg upon lying down
painless bleeding of submucosal veins of the lower 1/3 esophagus
esophageal varices
painful mucosal lacerations at the GE junction due to severe vomiting
Mallory-Weiss syndrome
what populations is mallory weiss syndrome common in?
alcoholics and bulimics
transmural esophageal rupture due to violet retching
BoerHaave syndrome ("Been Heaving syndrome")
what is associated with lye ingestion?
esophageal stricutres
what is associated with esophagitis?
reflux, infections (HSV-1, CMV, Candida) or chemical ingestion
Triad: 1. dysphagia, 2. glossitis, 3. iron deficiency anemia
Plummer-Vinson syndrome (dysphagia due to esophageal webs)
define Barrett's esophagus
glandular metaplasia: replacement of nonkeratinized (stratified) squamous epithelium with intestinal (columnar) epithelium in the distal esophagus; caused by chronic acid reflux
what type of cancer is associated with Barrett's esophagus?
what are the risk factors for esophageal cancer?
ABCDEF: alcohol/achalasia, Barrett's esophagus, Cigarettes, Diverticuli (zenker's), Esophageal web/esophagitis, Familial
which part of the esophagus is affected by SCC?
upper and middle 2/3
which part of the esophagus is affected by adenocarcinoma?
lower 1/3
what are the symptoms of malabsorption syndromes?
diarrhea, steatorrhea, weight loss and weakness
AutoAb to gluten (gliadin)
celiac sprue
what part of the bowel does celiac sprue affect?
proximal small bowel primarily - jejunem
how does tropical sprue differ from celiac sprue?
tropical sprue may be infectious and responds to antibiotics; unlike celiac, it can affect the entire small bowel
infection with Tropheryma whippelii
Whipple's disease
PAS+ macrophages in intestinal lamina propria and mesenteric nodes
Whipple's disease
malabsorption syndrome with arthralgias, cardiac and neurological symtpoms
Whipple's disease - often occurs in older men
what is the most common lactase deficiency?
disaccharide deficiency - milk intolerance
normal vili with osmotic diarrhea
disaccharide deficiency - milk intolerance
what can cause disaccharide deficiency?
self-limiting lactase deficiency can occur following injury (viral diarrhea) because lactase is located at the tips of intestinal villi
what causes malabsorpiton of fat and fat-soluble vitamins?
pancreatic insufficiency
what causes pancreatic insufficiency?
CF, obstructing cancer and chronic pancreatitis
anti-gliadin Ab
celiac sprue
blunted villi and lymphocytes in the lamina propria
celiac sprue
what is associated with celiac sprue?
dermatitis herpetiformis; and moderately increased risk of malignancy - T cell lymphomas
what causes acute (erosive) gastritis
disruption of the mucosal barrier leads to inflammation
what are the etiologies of acute (erosive) gastritis?
1. stress, 2. NSAIDs, 3. alcohol, 4. uricemia, 5. burns (Curling's ulcer), 6. brain injury (Cushing's ulcer)
what is type A chronic (nonerosive) gastritis?
autoimmune disorder characterized by 1. Auto-Ab to parietal cells, pernicious Anemia and Achlorhydria that affects the body/fundus
what part of the stomach is affected by type A chronic gastritis?
what is type B chronic gastritis?
caused by H. pylori infection in the Antrum
what is associated with type B chronic gastritis?
increased risk of MALT lymphoma
gastric hypertrophy with protein loss, pariteal cell atrophy and increased mucous cells
Menetrier's disease - rugae are so hypertrophied they look like brain gyri
what is a consequence of menetrier's disease?
It is precancerous
what type of cancer most often affects the stomach?
what is associated with stomach cancer?
dietary nitrosamines (smoked foods), achlorhydria, chronic gastritis, type A blood
what is Krukenberg's tumor?
bilateral ovarian metastasis from stomach cancer; contains abundant mucus and signet ring cells
what are common histological features of stomach cancer?
signet ring cells and acathosis nigricans
what is linitis plastica?
stomach cancer that is diffusely infiltrative (thickened, rigide, "leather bottle" appearance
gastric pain made worse by meals
gastric ulcer - pain leads to weight loss
what is associated with gastric ulcers?
H. pylori infection - 70%; chronic NSAID use also implicated
what is the cause of gastric ulcers?
decreased mucosal protection against gastric acid
pain decreases with meals
duodenal ulcers - leads to weight gain
what is assoicated with duodenal ulcers?
H. pylori infection - 100%
what causes duodenal ulcers?
increased gastric acid secretion (zollinger-ellison syndrome) or decreased mucosal proteciotn
hypertrophy of bruner's glands
duodenal ulcer
what differs between the histology of duodenal ulcers and carcinoma?
DUs have clean "punched out" margins
what are the complications of DUs?
bleeding, penetration into pancreas, performation and obstruction (not intrinsically precancerous)
how do the etiologies of Crohn's disease and ulcerative colitis differ?
CD- postinfectious; UC- autoimmune
how do the locations of Crohn's vs. ulcerative colitis differ?
CD- any portion of GI, usually terminal ileum and colon with 'skip' lesions and rectal sparing; UC- colon, continuous lesions always has rectal involvement
how does the gross morphology of Crohn's vs. ulcerative colitis differ?
CD- transmural inflammation, cobblestone mucosa and creeping fat, bowel wall thickening, linear ulcers, fissures and fistuals;
UC - mucosal and submucosal inflammation only; friable mucosal pseudopolyps with freely hanign mesentery
how does the imaging of Crohn's vs. ulcerative colitis differ?
CD -"string sign" on barium swallow x-ray;
UC- "lead pipe" on imaging
how does the histology of Crohn's vs. ulcerative colitis differ?
CD - noncaseating granulomas and lymphoid aggregates; UC - crypt abscesses and ulcers, bleeding, no granulomas
what are the complications of Crohn's disease?
strictures, fistulas, perianal disease, malabsorption, nutritional depletion
what are the complications of ulcerative colitis?
colorectal carcinoma, stenosis and toxic megacolon
how does the initial manifestation of Crohn's vs. ulcerative colitis differ?
CD- diarrhea w/wo blood; UC- bloodly diarrhea
what are the extraintestinal manifestations of Crohn's disease?
migratory polyarthritis, erythema nodosum, ankylosing spondylitis, uveitis and immunlogic disorders
what are the extraintestinal manifestations of ulcerative colitis?
pyoderma gangrenosum, primary sclerosing cholangitis
what is the treatment of Crohn's disease?
steroids and infliximab
what is the treatment of ulcerative colitis?
sulfasalazine, infliximab and colectomy
what is the differential for appendicitis?
elderly - diverticulitis; younger women - ectopic pregnancy (hCG can rule that out)
blind pouch popping out of the alimentary tract that communicates with the lumen of the gut
where are diverticulum most often located?
sigmoid colon
define "true" diverticulum
all 3 gut wall layers outpouch
define "false" diverticulum
only mucosa and submucosa outpouch, occur especially where vasa recta perforate muscularis externa
what population is diverticulosis common in?
50% are in pts > 60 yoa; associated with a low-fiber diet
what causes diverticulosis?
increased intraluminal pressure and focal weakness in colonic wall
how does diverticulosis present?
often asymptomatic or assoicated with vague discomfort and/or painless rectal bleeding
inflammation of diverticula classically causing LLQ pain, fever and leukocytosis
what are the consequences of diverticulitis?
may perforate - peritonitis, abscess formation or bowel stenosis; can cause colovesical fistual (fistula with bladder) - pneumaturia
how do you treat diverticulitis?
how does diverticulitis present
pain, fever, leukocytosis; can cause bright red rectal bleeding
what is zenker's diverticulum?
false diverticulum (only mucosa and submucosa outpouch) - herniation of mucosal tissue at the junction of the pharynx and esophagus
what are the presenting symptoms of zenker's diverticulum?
halitosis, dysphagia and obstruction
what is Meckel's diverticulum?
persistence of the vitelline duct or yolk stalk
what is the most common congenital anomaly of the GI tract?
meckel's diverticulum
what can be contained in Meckle's diverticulum?
ectopic acid-secreting gastric mucosa and or pacreatic tissue
what are the consequences of Meckle's diverticulum?
bleeding, intussusceptoin, volvulus or obstruction near the terminal ileum
how does Meckle's diverticulum differ from omphalomesenteric cyst?
omphalomesenteric cyst is a cystic dilation of the vitelline duct
what is the rule of 2s for Meckle's diverticulum?
2 inches long, 2 feet from the ileocecal valve, 2% of the population, presents in first 2 years of life, may have 2 types of epithelia - gastric and pancreatic
telescoping of a bowel segment into a distal segment
what are the consequences of intussusception?
can compromise blood supply
what causes intussusception?
intraluminal mass, usually in infants
twisting of portion of bowel around its mesentery
what are the consequences of volvulus?
can lead to obstruction and infarction
where and when are volvulus usually seen?
in the sigmoid colon (where there is redundant mesentery) and in the elderly
what characterized Hirschsprung's disease?
lack of ganglion cells/enteric nervous plexuses (auerbach'as and meissner's) due to failure of neural crest cell migration
gut abnormality due to failure of neural crest cell migration
Hirschsprung's disease
how does Hirschsprung's present?
chronic constipation (failure to pass mesconium) early in life; dilated colon proximal to aganglionic segment resulting in a 'transistional zone'; involves rectum
what is associated with Hirschsprung's?
down syndrome
early bilious vomiting with proximal stomach distention
duodenal atresia = "double bubble"
failure of recanalization of small bowel
duodenal atresia
what is assoicated with duodenal atresia?
down syndrome
what is meconium ilues?
in CF, meconium plug obstructs intestine preventing stool passage at birth
necrosis of intestinal mucosa and possible perforation
necrotizing enterocolitis - colon is usually involved but can involve entire GI tract; more common in premature infants
where does ischemic colitits usually occur?
at the splenic flexure, typically in the elderly
what is intestinal adhesion?
causes acute bowel obstruction, commonly forma recent surgery; can have well-demarcated necrotic zone
what is angiodysplasia?
tortuous dilation of vessels leads to bleeding
where does angiodysplasia occur?
often in the cecum and ascending colon; more common in older pts
what are the majority of colonic polyps?
90% are benign hyperplastic hamartomas, not neoplasms; the more villous the polyp, the more likely it is to be malignant
what do colonic polyps look like?
sawtooth' appereance - grow into the lumen, often rectosigmoid colon
what is the cause of most colorectal cancer?
most are sporadic due to chromsomal instability (85%) or microsatellite instability (15%)
how do left vs. right sided colon cancers present?
left side obstructs, right side bleeds
what are the risk factors for colon cancer?
1. colorectal villous adenomas, 2. chronic IBD (especially ulcerative colitis), 3. FAP, 4. HNPCC, 5. family hsitory
"apple core" lesion on barium enema x-ray
colorectal cancer
what is the tumor marker for colorectal cancer?
what causes familial adenomatous polyposis (FAP)?
AD mutation of the APC gene on 5q (2 hit hypothesis)
thousands of polyps, pancolonic and always involving the rectum
familial adenomatous polyposis
what is Gardner's syndrome?
FAP with ossesous and soft tissue tumors and retinal hyperplasia
what is turcot's syndrome?
FAP with possible brain involvmenet (gliomas and medulloblastomas)
what causes Lynch syndrome?
mutatios of DNA mismatch repair genes, 80% progress to colorectal cancer; proximal colon always involved
what is Peutz-Jeghers syndrome?
a benign polyposis syndrome associated with increased risk of colorectal cancer and other visceral malignancies (pancreas, breast, stomach, ovary)
hamartomatous polyps of colon and small intestine, hyperpigmented mouth, lips, hands and genitalia
Peutz-Jeghers syndrome
what is carcinoid tumor?
tumor of endocrine cells that contain "dense core bodies" on EM and often produce 5HT which can lead to carcinoid syndrome
where do carcinoid tumors occur?
small intestine - they make up 50% of small bowel tumors;
wheezing, right sided heart murmurs, diarrhea, and flushing
carcinoid tumor
what happens if a carcinoid tumor is completely confined to the GI system?
no carcinoid syndrome occurs due to liver metabolzism of 5HT
define liver cirrhosis
diffuse fibrosis of liver that destroys normal architecture, with nodular regneration
what causes micronodular liver cirrhosis?
nodules <3cm, uniform size; due to metabolic insult - alcohol, hemochromatosis, Wilson's disease
what causes macronodular cirrhosis of the liver?
nodules >3cm, varied in size; due to significant liver injury leading to necrosis (postinfectious or drug induced hepatitis)
which increases the risk of hepatocellular carcinoma, micro- or macronodular liver cirrhosis?
what can relieve portal HTN?
portacaval shunt between splenic vein and left renal vein
what disease is ALT a good marker for?
viral hepatitis (ALT > AST)
what disease is AST a good marker for?
alcoholic hepatitis (AST > ALT) and MI
what disease is GGT (gamma-glutamyl transpeptidase) a good marker for?
increases with heavy alcohol consumption
what disease is alkaline phosphatase a good marker
obstructive liver disease (hepatocellular carcinoma), bone disease, and bile duct disease
what disease is amylase a good marker for?
acute pancreatitis, mumps
what disease is lipase a good marker for?
acute pancreatitis
what disease is ceruloplasmin a good marker for?
decreased in Wilson's disease
what is Reye's syndrome?
rare, often fatal childhood hepatoencephalopathy
findings: fatty liver, hypoglycemia nd coma
Reye's syndrome
what is reye's syndrome associated with?
viral infection (VZV and influenza B) that has been treated with salicylates - aspirin is not recommended for children!
what are the 3 types of alcoholic liver disease?
1. hepatic steatosis, 2. alcoholic hepatitis, 3. alcoholic cirrhosis
define hepatic steatosis
short term cahnge with moderate alcohol intake; macrovesicular fatty change that can be reverse when you stop drinking
what causes alcoholic hepatitis?
sustained, long term consuption
define alcoholic hepatitis
swollen and necrotic hepatocytes with neutrophilic infiltration and mallory bodies (intracytoplasmic eosinophilic inclusions
what is the AST/ALT ratio in alcoholic hepatitis?
AST > ALT, ratio usually greater than 1.5
define alcoholic cirrhosis
final and irrversible form of alcoholic liver disease - has presentation of liver disease; micronodular irregularly shrunken liver with "hobnail" appereance and sclerosis around the central vein
what is the most common primary malignant tumor of the liver in adults?
hepatocellular carcinoma/hepatoma
what are risk factors for hepatocellular carcinoma?
HBV and HCV, Wilson's disease, hemochromatosis, alpha1-antityrpsin deficiency, alcoholic cirrhosis and carcinogens
how does hepatocellular carcinoma spread?
by hematogenous dissemination
findings: tender hepatomegaly, ascities, polycythemia and hypoglycema
hepatocellular carcinoma/hepatoma
what causes nutmeg liver?
backup of blood into the liver, commonly due ot RCHF and Budd-Chiari syndrome
what is increased in hepatocellular carcinoma?
alpha-fetoprotein, can lead to Budd Chiari syndrome
what can result from nutmeg liver?
centrilobular congestion and necrosis can result in cardiac cirrhosis
what is budd-chiari syndrome?
occlusion of IVC or hepatic veins with coentrilobular congestion and necrosis that leads to congestive liver disease
what are the findings in budd-chiari syndrome?
varices, visible abdmonial and back veins, and absent JDV
what is associated with budd-chiari syndrome?
polycythemia vera, pregnancy and hepatcellular carcinoma
what is alpha1-anti-trypsin deficiency?
misfolded gene product protein accumulates in the hepatocellular EF; decreaed elastic tisssue in the lungs leads to panacinar emphysema; PAS+ globules are found in the liver
what are the findings of hetaocellular jaudice: hyperbilirubinemia, urine bilirubin and urine urobilinogen
hyperbilirubienima: conjugated/unconjugated, urine bilirubin: incrased; urine urobilinogen: normal or low
what are the findings of obstructive jaudice: hyperbilirubinemia, urine bilirubin and urine urobilinogen
hyperbilirubienima: conjugated, urine bilirubin: incrased; urine urobilinogen: low
what are the findings of hemolytic jaudice: hyperbilirubinemia, urine bilirubin and urine urobilinogen
hyperbilirubienima: unconjugated, urine bilirubin: absent; urine urobilinogen: increased
what is Gilbert's syndrome?
mildly decreased UDP-glucuronyl transferase or decrased bilirubin uptake. Elevated unconjugated bilirubin without overt hemolysis; associated with stress, asymptomatic with no clinical consequences
what disease has absent UDP-glucuronyl transferase?
Crigler-Najjar syndrome, type 1
findings: jaundice, kernicterus, increased unconjugated bilirubin
Crigler-Najjar syndrome, type 1
what are the consequences of crigler-najjar syndrome, type 1?
present early in life, pts die wihtin a few years
what is crigler-najjar syndrome, type 2?
less severe than type 1, and responds to phenobarbital which increaes liver enzyme synthesis
what is the treatment for crigler-najjar syndrome, type 1?
plasmapheresis and phototherapy
what disease causes a grossly black liver?
dubin-Johnson syndrome
what causes dubin-johnson syndrome?
conjugated hyperbilirubinemia due to defective liver excretion
what is Rotor's syndrome?
a milder form of dubin-johnson syndrome that does not cause black liver
how do Gilbert's syndrome, Crigler-najjar and dubin-johnson syndromes differ?
gilberts: problem with bilirubin uptake, crigler najjar: problem with bilirubin conjugation, dubin-johnson: problem with excreted of conjugated bilirubin
which of the hereditary hyperbilirubinemias presents with conjugated bilirubinemia?
dubin-Johnson syndrome
define Wilson's disease
AR disease of inadequate heaptic copper excretion and failure of copper to enter circulation as ceruoplasmin
where does copper accumulate in Wilson's disease?
liver, brain, cornea, kidneys and joints
how do you treat Wilson's disease?
what characterizes Wilson's diseas?
ABCD: Asterixis, Basal ganglia degernation (parkinsonian), Ceruloplasmin decreased, cirrhosis, corneal depostisi, copper acculumation, carcinoma; Dementia, dyskinesia and dysarthria; hemolytic anemia
Kayser-Fleischer rings
Wilson's disease
what is hemochromatosis?
the deposition of hemosiderin (iron)
micronodular cirrhosis, DM and skin pigmentation
hemochromatosis, also called "bronze" diabetes
what are the consequences of hemochromatosis?
CHF and increased risk of hepatocellular carcinoma
hoe do you get hemochromatosis?
primary - AR, or secondary to chronic transfusion therapy
increased ferritin, increased iron, decreased TIBC, increased transferrin saturation
hemochromatosis, also called "bronze" diabetes
how do you treat hereditary hemochromatosis?
repeated phleobotomy, deferoxamine
what HLA is hemochromatosis associated with
concentric "onion skin" bile duct fibrosis
primary sclerosing cholangitis
what is primary sclerosing cholangitis?
both intra- and extra-hepatic, inflammation and fibrosis of the bile ducts, has an increase in ALP
alternating strictures and dilation with "beading" on ERCP
primary sclerosing cholangitis
what is assoicated with primary sclerosing cholangitis?
associated with ulcerative colitis, can lead to secondary biliary cirrhosis
what is primary biliary cirrhosis?
intrahepatic, autoimmune desorder that causes severe obstructive jaundice, steatorrhea, pruritus and hypercholesterolemia (zanthoma)
what disease is associated with increased ALP and increased serum mitochondrial Ab
primary biliary cirrhosis
what diseases are associated with primary biliary cirrhosis?
scleroderma and CREST
what causes secondary biliary cirrhosis?
extrahepatic biliary obstruction - incrased pressure in the intrahepatic ducts results in injury and fibrosis
what disease is assoicated with increased ALP and increased conjugated bilirubin?
secondary biliary cirrhosis
what complicates secondary biliary cirrhosis?
ascending choangitis (bacterial infection) bile stasis and "bile lakes"
when are gallstones (cholelithiasis) formed?
when solubilizing bile acids and lecithin are overwhelmed by increased cholesterol and or bilirubin
which type of stone is most common?
cholesterol stones
what are cholesterol stones associated with ?
obesity, crohn's disease, CF, advanced age, clofibrate, estrogens, multipartiy, rapid weight loss and native america origin
what gallstones are radiolucent?
cholesterol stones - 10-20% opaque due to calcifications
what are the 4 risk factors for gallstones?
female, fat, fertile, forty
triad: 1. Jaundice, 2. fever, 3. RUQ pain
charcot's triad of cholangitis
which gallstones are radiopaque?
pigment stones
what is assoicated with pigment stones?
chronic RBC hemolysis, alcoholic cirrhosis, advanced age and biliary infection
what are the complications of gallstones?
ascending cholangitis, acute pancreatitis, bile stasis and cholecystitis; can cause biliary colic, or cause a fistula between the gallbladder and the small intestine
what causes air to be present in the biliary tree on imaging?
if gallstones obstruct the ileocecal valve
how do you diagnose and treat gallstones (cholelithiasis)?
diagnose by ultrasound, treat with cholecystectomy
what is Murphy's sign?
inspiratory arrest on deep palaption, caused by gallstones
what is cholecystitis?
inflammation of the gallbladder - either infectious (CMV, crypto) or due to gallstone complication
what is a marker of ascending cholangitis?
increased ALP when bile duct becomes involved
define acute pancreatitis
autodigestion of pancreas by pancreatic enzymes
what are the causes of acute pancreatitis?
GET SMASHeD: 1. gallstones, 2. ethanol, 3. trauma, 4. steroids, 5. mumps, 6. autoimmune disease, 7. scorpion sting, 8. hypercalcemia/Hyperlipidemia, 9. drugs (sulfa)
epidgastric abdominal pain radiating to back, anorexia and nausea
acute pancreatitis
what lab values are associated with acute pancreatitis?
elevated amylase and lipase (higher specificity)
what are the consequences of acute pancreatitis?
can lead to DIC, ARDS, diffuse fat necrosis, hypocalcemia, pseudocyst formaiton, hemorrhage and infection
what are the consequences of chronic pancreatitis?
preacreatic insufficiency - steatorrhea, fat-soluble vitamin deficiency and DM
what type of chronic pancreatic injury is associated with alcoholism?
chronic calcifying pancreatitis
what location of the pancrease are tumors more commonly found?
in the head, can lead to obstructive jaundice
what cancer is associated with markers: CEA and CA-19-9?
pancreatic adenocarcinoma
what is a risk factor for developing pancreatic adenocarcinoma?
cigarettes but NOT EtOH
decribe the prognosis of pancreatic adenocarcinoma
very aggressive, prognosis is less than 6 months, usually already metastasized at presentation; there is an increased risk in jews and black males
abdmonial pain radiating to the back, weight loss, migratory thrombophlebitis (redness and tenderness on palaption of extremities) and obstructive juadnice with palpable gallbladder?
pancreatic adenocarcinoma
what is Trousseau's syndrome?
migratory thrombophlebitis - redness and tnederness on palaption of extremities, seen in pancreatic adenocarcinoma
what is courvoisier's sign?
obstructive jaundice with plapable gallbladder, seen in pancreatic adenocarcinoma