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312 Cards in this Set
- Front
- Back
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what side is the aorta on compared to the IVC?
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right
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what does the falciform ligament connet?
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liver to anterior abdonminal wall
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what does the falciform ligmanet contain?
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ligamentum teres
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what is the falciform ligament derived from?
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the fetal umbilical vein
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what does the hepatoduodenal ligament connect?
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liver to duodenum; it also connect the greater and lesser sacs
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what does the hepatoduodenal ligament contain?
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portal triad: hepatic artery, portal vein and common bile duct
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what does the gastrohepatic ligmanet connect?
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liver to lesser curve of the stomach; separates the right greater and lesser sacs
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what does the gastrohepatic ligament contain?
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gastric arteries
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what does the gastrocolic ligament connect?
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greater curvature and transverse colon; it is part of the greater omentum
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what does the gastrocolic ligament contain?
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gastroepiploic arteries
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what does the gastrosplenic ligament connect
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greater curvature and spleen; separates left greater and lesser sacs
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what does the gastrosplenic ligament contain?
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short gastrics
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what does the splenorenal ligament connect?
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spleen to posterior abdmonial wall
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what does the splenorenal ligament contain?
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splenic artery and vein
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what are the 4 layers of the gut wall?
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1. mucosa, 2. submucosa, 3. muscularis externa, 4. serosa/adventitia
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what is contained in the mucosa layer of the gut wall?
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1. epithelium (absorption), 2. lamina propria (support), 3. muscularis mucosae (motility)
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what is contained in the submucosa layer of the gut wall?
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submucosal nerve plexus = Meissner's
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what is contained in the muscularis externa?
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Myenteric nerve plexus = Auerbach's
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what is the frequency of basal electric rhythm of the stomach, duodenum and ileum?
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Stomach = 3 waves/min; Duodenum = 12 waves/min; Ileum = 8-9 waves/min
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what is the function of Auerbach's plexus?
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Myenteric - controls motility along entire gut wall, contains cell bodies of PNS terminal effector neurons
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where is Auerbach's plexus located?
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between inner ciruclar and outer longitudinal layers of smooth muscle (Auerbach's is on the Autside)
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what is the function of Meissner's plexus?
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Submucosal - regulates local secretions, blood flow and absorption; contains cell bodies of some PNS ternimal effector neurons
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where is Meissner's plexus located?
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between mucosa and inner layer of smooth muscle
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what structures are derived from the embryonic foregut?
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stomach to proximal duodenum, liver, gallbladder and pancreas
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what artery and nerve innervate the embryonic foregut?
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celiac artery and the vagus nerve
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what is the vertebral level of the foregut?
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T12/L1
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what structures are derived from the embryonic midgut?
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distal duodenem to proxmial 2/3 of transverse colon
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what artery and nerve innervate the embryonic midgut?
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SMA and vagus nerve
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what is the vertebral level of the midgut?
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L1
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what structures are derived from the hindgut?
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distal 1/3 of transverse colon to upper portion of rectum; splenic flexure is a watershed region
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what artery and nerve innervate the hindgut?
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IMA and pelvic nerve
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what is the vertebral level of the hindgut?
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L3
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what are the 3 branches of the celiac trunk?
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common hepatic, splenic and left gastric
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what forms the pectinate line?
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it is formed where the hindgut meets ecoterm
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what can occur above the pectinate line?
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internal hemorroids, adenocarcinoma
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what can occur below the pectinate line?
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external hemorrhoids and SCC
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what supplies and drains above the pectinate line?
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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
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what supplies and drains below the pectinate line?
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inferior rectal artery (branch of internal pudendal), drained by inferior rectal vein to the internal pudenal vein to the internal iliac vein to IVC
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what does the apical surface of hepatocytes face?
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bile canaliculi
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what does the basolateral surface of hepatocytes face?
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sinusoids
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what are the 3 zones of the liver?
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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
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what zone of the liver is most sensitive to viral hepatitis
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Zone I: periportal zone
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what zone of the liver is most sensitive to alcoholic hepatitis?
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Zone 3: pericentral (centrilobular) vein zone
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what zone of the liver is most senstiive to toxic injury?
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Zone 3: pericentral (centrilobular) vein zone
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what zone of the liver is affected 1st by ischemia?
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Zone 3: pericentral (centrilobular) vein zone
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what combines to form the common bile duct?
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common hepatic duct and the cystic duct
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how is the femoral region's vessels organized?
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go lateral to medical to find your NAVEL: nerve-artery-vein-empty space-lymphmatics
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what is contained in the femoral triangle?
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femoral vien, artery and nerve
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what is contained in the femoral sheath?
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femoral vein, artery and canal (deep to inguinal lymph nodes) but NOT the femoral nerve
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what is the most common hiatal hernia?
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sliding; GE junction is displaced "hourglass stomach"
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where is an indirect inguinal hernia found?
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through the INternal (deep) inguinal ring, external (superficial) inguinal ring and INto the scrotum; enters lateral to the inferior epigastric artery
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why does an indirect inguinal hernia form?
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forms in INfants due to failure of processus vaginalis to close; much more common in males
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where is a direct inguinal hernia found?
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protrudes through the inguinal triangle (Hasselbach's), directly through the abdominal wall; goes through the external (superficial) inguinal ring only; occurs in older men
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where does an indirect hernia lie in relation to the inferior epigastric artery?
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MDs don't LIe: Medial = direct; Lateral = indirect
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where does a direct hernia lie in relation to the inferior epigastric artery?
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MDs don't LIe: Medial = direct; Lateral = indirect
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where is a femoral hernia found?
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protrudes below inguinal ligament through the femoral canal below and lateral to the pubic tubercle; more common in women
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which hernia is more common in women?
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femoral hernia
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what outlines Hasselbach's triangle?
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1 inferior epigastric artery, 2. lateral border of rectus abdmonis, 3. inguinal ligament
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what are Peyer's patches?
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unencapsulated lymphoid tissue in the lamina propria and submucosa of the small intestin; contains speclized M cells that take up antigens
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where does saliva scretions come from?
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parotid - serious mostly; submandibular, submaxillary and sublingual - mostly mucus secretions
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what is the function of saliva?
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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
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what type of solution is secreted when saliva has a low flow rate?
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hypotonic - more time to reabsorb H20
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what type of solution is secreted when saliva has a high flow rate?
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isotonic - less time to reabsorb H20
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where does CN7 run?
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through the parotid gland
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what stimulates saliva secretion?
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both SNS (T1-T3 superior cervical ganglion) and PNS (faical and glossopharyngeal nerve)
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what are Brunner's glands?
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secrete alkaline mucus to neutralize acid contents enterin gthe duodenum from the stomach; located in duodenal submucosa (only GI submucosal glands)
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when is hypertrophy of Brunner's glands seen?
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in peptic ulcer disease
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what do parietal cells secrete?
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IF and gastric acid
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what stimulates gastric acid secretion?
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histamine, ACh and gastrin
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what inhibits gastric acid secretion?
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somatostatin, GIP and prostaglandin
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what is secreted by chief cells?
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pepsin
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what stimulates pepsin secretion?
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vagal stimulation and local acid
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what is screted by mucosal cells?
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HCO3-
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what stimulates HCO3- secretion?
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secretin
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where are parietal cells and chief cells located in the stomach?
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in the body of the stumach
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where are mucus cells and G cells located in the stomach?
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in the antrum
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which cells are blocked by atropine?
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parietal cells, not G cells even though both are stimulated by the vagus nerve; G cells works via GRP, not ACh
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what does cimetidine do?
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blocks histamine from stimulating gastric acid secretion
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what activates trypsinogen?
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converted to trypsin by enterokinase/enteropeptidase which is secreted by the duodenal mucosa
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what type of bond does salivary amylase break?
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hydrolyzes alpha-1,4 linkages to yield disaccharides
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what is the rate limiting step of carbohydrate digestion?
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oligosaccharide hydrolases located at the brush border of the intestine
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what do enterocytes absorb?
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only monosaccharides glucose and galactose by SGLT1 (Na dependent) and fructose by GLUT5
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where is iron absorbed?
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as Fe2+ in the duodenum
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where is folate absorbe?
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in the jejunum
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where is B12 absorbe?
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in the terminal ileum with bile acids
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what is bile composed of?
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bile saids (bile acids conjugated to glycine or taurine = water insoluble), phopholipids, cholesterol, bilirubin, watera nd ions
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how is cholesterol excreted?
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in bile
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where do most salivary gland tumors occur?
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in the parotid gland
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what is the most common salivary gland tumor?
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pleomorphic adenoma: painless, movable mass, benign with high rate or recurrence
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what is warthin's tumor?
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benign salivary tumor due to heterotorphic salivary galnd tissue traped in a laymp node and surrounded with lymphatic tissue
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what is the most common malignant salivary gland tumor?
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mucoepidermoid carcinoma
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define achalasia
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failure of relaxation of the LES due to loss of myenteric (Auerbach's plexus)
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barium swallow shows dilated esophagus with an area of distal stenosis
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achalasia
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what is associated with achalasia?
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increased risk of esophageal carcinoma
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"bird's beak" on barium swallow
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achalasia
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what can cause secondary achalasia?
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chagas disease
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what type of esophageal dysmotility is associated with scleroderma (CREST)?
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low pressure proximal to LES
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heartburn and regurg upon lying down
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GERD
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painless bleeding of submucosal veins of the lower 1/3 esophagus
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esophageal varices
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painful mucosal lacerations at the GE junction due to severe vomiting
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Mallory-Weiss syndrome
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what populations is mallory weiss syndrome common in?
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alcoholics and bulimics
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transmural esophageal rupture due to violet retching
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BoerHaave syndrome ("Been Heaving syndrome")
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what is associated with lye ingestion?
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esophageal stricutres
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what is associated with esophagitis?
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reflux, infections (HSV-1, CMV, Candida) or chemical ingestion
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Triad: 1. dysphagia, 2. glossitis, 3. iron deficiency anemia
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Plummer-Vinson syndrome (dysphagia due to esophageal webs)
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define Barrett's esophagus
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glandular metaplasia: replacement of nonkeratinized (stratified) squamous epithelium with intestinal (columnar) epithelium in the distal esophagus; caused by chronic acid reflux
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what type of cancer is associated with Barrett's esophagus?
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adenocarcinoma
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what are the risk factors for esophageal cancer?
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ABCDEF: alcohol/achalasia, Barrett's esophagus, Cigarettes, Diverticuli (zenker's), Esophageal web/esophagitis, Familial
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which part of the esophagus is affected by SCC?
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upper and middle 2/3
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which part of the esophagus is affected by adenocarcinoma?
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lower 1/3
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what are the symptoms of malabsorption syndromes?
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diarrhea, steatorrhea, weight loss and weakness
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AutoAb to gluten (gliadin)
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celiac sprue
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what part of the bowel does celiac sprue affect?
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proximal small bowel primarily - jejunem
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how does tropical sprue differ from celiac sprue?
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tropical sprue may be infectious and responds to antibiotics; unlike celiac, it can affect the entire small bowel
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infection with Tropheryma whippelii
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Whipple's disease
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PAS+ macrophages in intestinal lamina propria and mesenteric nodes
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Whipple's disease
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malabsorption syndrome with arthralgias, cardiac and neurological symtpoms
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Whipple's disease - often occurs in older men
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what is the most common lactase deficiency?
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disaccharide deficiency - milk intolerance
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normal vili with osmotic diarrhea
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disaccharide deficiency - milk intolerance
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what can cause disaccharide deficiency?
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self-limiting lactase deficiency can occur following injury (viral diarrhea) because lactase is located at the tips of intestinal villi
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what causes malabsorpiton of fat and fat-soluble vitamins?
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pancreatic insufficiency
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what causes pancreatic insufficiency?
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CF, obstructing cancer and chronic pancreatitis
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anti-gliadin Ab
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celiac sprue
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blunted villi and lymphocytes in the lamina propria
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celiac sprue
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what is associated with celiac sprue?
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dermatitis herpetiformis; and moderately increased risk of malignancy - T cell lymphomas
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what causes acute (erosive) gastritis
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disruption of the mucosal barrier leads to inflammation
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what are the etiologies of acute (erosive) gastritis?
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1. stress, 2. NSAIDs, 3. alcohol, 4. uricemia, 5. burns (Curling's ulcer), 6. brain injury (Cushing's ulcer)
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what is type A chronic (nonerosive) gastritis?
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autoimmune disorder characterized by 1. Auto-Ab to parietal cells, pernicious Anemia and Achlorhydria that affects the body/fundus
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what part of the stomach is affected by type A chronic gastritis?
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body/fundus
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what is type B chronic gastritis?
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caused by H. pylori infection in the Antrum
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what is associated with type B chronic gastritis?
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increased risk of MALT lymphoma
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gastric hypertrophy with protein loss, pariteal cell atrophy and increased mucous cells
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Menetrier's disease - rugae are so hypertrophied they look like brain gyri
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what is a consequence of menetrier's disease?
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It is precancerous
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what type of cancer most often affects the stomach?
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adenocarcinoma
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what is associated with stomach cancer?
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dietary nitrosamines (smoked foods), achlorhydria, chronic gastritis, type A blood
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what is Krukenberg's tumor?
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bilateral ovarian metastasis from stomach cancer; contains abundant mucus and signet ring cells
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what are common histological features of stomach cancer?
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signet ring cells and acathosis nigricans
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what is linitis plastica?
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stomach cancer that is diffusely infiltrative (thickened, rigide, "leather bottle" appearance
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gastric pain made worse by meals
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gastric ulcer - pain leads to weight loss
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what is associated with gastric ulcers?
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H. pylori infection - 70%; chronic NSAID use also implicated
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what is the cause of gastric ulcers?
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decreased mucosal protection against gastric acid
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pain decreases with meals
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duodenal ulcers - leads to weight gain
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what is assoicated with duodenal ulcers?
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H. pylori infection - 100%
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what causes duodenal ulcers?
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increased gastric acid secretion (zollinger-ellison syndrome) or decreased mucosal proteciotn
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hypertrophy of bruner's glands
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duodenal ulcer
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what differs between the histology of duodenal ulcers and carcinoma?
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DUs have clean "punched out" margins
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what are the complications of DUs?
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bleeding, penetration into pancreas, performation and obstruction (not intrinsically precancerous)
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how do the etiologies of Crohn's disease and ulcerative colitis differ?
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CD- postinfectious; UC- autoimmune
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how do the locations of Crohn's vs. ulcerative colitis differ?
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CD- any portion of GI, usually terminal ileum and colon with 'skip' lesions and rectal sparing; UC- colon, continuous lesions always has rectal involvement
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how does the gross morphology of Crohn's vs. ulcerative colitis differ?
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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 |
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how does the imaging of Crohn's vs. ulcerative colitis differ?
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CD -"string sign" on barium swallow x-ray;
UC- "lead pipe" on imaging |
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how does the histology of Crohn's vs. ulcerative colitis differ?
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CD - noncaseating granulomas and lymphoid aggregates; UC - crypt abscesses and ulcers, bleeding, no granulomas
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what are the complications of Crohn's disease?
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strictures, fistulas, perianal disease, malabsorption, nutritional depletion
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what are the complications of ulcerative colitis?
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colorectal carcinoma, stenosis and toxic megacolon
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how does the initial manifestation of Crohn's vs. ulcerative colitis differ?
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CD- diarrhea w/wo blood; UC- bloodly diarrhea
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what are the extraintestinal manifestations of Crohn's disease?
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migratory polyarthritis, erythema nodosum, ankylosing spondylitis, uveitis and immunlogic disorders
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what are the extraintestinal manifestations of ulcerative colitis?
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pyoderma gangrenosum, primary sclerosing cholangitis
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what is the treatment of Crohn's disease?
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steroids and infliximab
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what is the treatment of ulcerative colitis?
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sulfasalazine, infliximab and colectomy
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what is the differential for appendicitis?
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elderly - diverticulitis; younger women - ectopic pregnancy (hCG can rule that out)
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blind pouch popping out of the alimentary tract that communicates with the lumen of the gut
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diverticulum
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where are diverticulum most often located?
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sigmoid colon
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define "true" diverticulum
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all 3 gut wall layers outpouch
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define "false" diverticulum
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only mucosa and submucosa outpouch, occur especially where vasa recta perforate muscularis externa
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what population is diverticulosis common in?
|
50% are in pts > 60 yoa; associated with a low-fiber diet
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what causes diverticulosis?
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increased intraluminal pressure and focal weakness in colonic wall
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how does diverticulosis present?
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often asymptomatic or assoicated with vague discomfort and/or painless rectal bleeding
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inflammation of diverticula classically causing LLQ pain, fever and leukocytosis
|
diverticulitis
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what are the consequences of diverticulitis?
|
may perforate - peritonitis, abscess formation or bowel stenosis; can cause colovesical fistual (fistula with bladder) - pneumaturia
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how do you treat diverticulitis?
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antibiotics
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how does diverticulitis present
|
pain, fever, leukocytosis; can cause bright red rectal bleeding
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what is zenker's diverticulum?
|
false diverticulum (only mucosa and submucosa outpouch) - herniation of mucosal tissue at the junction of the pharynx and esophagus
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what are the presenting symptoms of zenker's diverticulum?
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halitosis, dysphagia and obstruction
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what is Meckel's diverticulum?
|
persistence of the vitelline duct or yolk stalk
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what is the most common congenital anomaly of the GI tract?
|
meckel's diverticulum
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what can be contained in Meckle's diverticulum?
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ectopic acid-secreting gastric mucosa and or pacreatic tissue
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what are the consequences of Meckle's diverticulum?
|
bleeding, intussusceptoin, volvulus or obstruction near the terminal ileum
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how does Meckle's diverticulum differ from omphalomesenteric cyst?
|
omphalomesenteric cyst is a cystic dilation of the vitelline duct
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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
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telescoping of a bowel segment into a distal segment
|
intussusception
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what are the consequences of intussusception?
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can compromise blood supply
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what causes intussusception?
|
intraluminal mass, usually in infants
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twisting of portion of bowel around its mesentery
|
volvulus
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what are the consequences of volvulus?
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can lead to obstruction and infarction
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where and when are volvulus usually seen?
|
in the sigmoid colon (where there is redundant mesentery) and in the elderly
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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
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gut abnormality due to failure of neural crest cell migration
|
Hirschsprung's disease
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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
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what is associated with Hirschsprung's?
|
down syndrome
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early bilious vomiting with proximal stomach distention
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duodenal atresia = "double bubble"
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|
failure of recanalization of small bowel
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duodenal atresia
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what is assoicated with duodenal atresia?
|
down syndrome
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what is meconium ilues?
|
in CF, meconium plug obstructs intestine preventing stool passage at birth
|
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|
necrosis of intestinal mucosa and possible perforation
|
necrotizing enterocolitis - colon is usually involved but can involve entire GI tract; more common in premature infants
|
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where does ischemic colitits usually occur?
|
at the splenic flexure, typically in the elderly
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what is intestinal adhesion?
|
causes acute bowel obstruction, commonly forma recent surgery; can have well-demarcated necrotic zone
|
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what is angiodysplasia?
|
tortuous dilation of vessels leads to bleeding
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where does angiodysplasia occur?
|
often in the cecum and ascending colon; more common in older pts
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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
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what do colonic polyps look like?
|
sawtooth' appereance - grow into the lumen, often rectosigmoid colon
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what is the cause of most colorectal cancer?
|
most are sporadic due to chromsomal instability (85%) or microsatellite instability (15%)
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how do left vs. right sided colon cancers present?
|
left side obstructs, right side bleeds
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what are the risk factors for colon cancer?
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1. colorectal villous adenomas, 2. chronic IBD (especially ulcerative colitis), 3. FAP, 4. HNPCC, 5. family hsitory
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"apple core" lesion on barium enema x-ray
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colorectal cancer
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what is the tumor marker for colorectal cancer?
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CEA
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what causes familial adenomatous polyposis (FAP)?
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AD mutation of the APC gene on 5q (2 hit hypothesis)
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thousands of polyps, pancolonic and always involving the rectum
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familial adenomatous polyposis
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what is Gardner's syndrome?
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FAP with ossesous and soft tissue tumors and retinal hyperplasia
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what is turcot's syndrome?
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FAP with possible brain involvmenet (gliomas and medulloblastomas)
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what causes Lynch syndrome?
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mutatios of DNA mismatch repair genes, 80% progress to colorectal cancer; proximal colon always involved
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what is Peutz-Jeghers syndrome?
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a benign polyposis syndrome associated with increased risk of colorectal cancer and other visceral malignancies (pancreas, breast, stomach, ovary)
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hamartomatous polyps of colon and small intestine, hyperpigmented mouth, lips, hands and genitalia
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Peutz-Jeghers syndrome
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what is carcinoid tumor?
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tumor of endocrine cells that contain "dense core bodies" on EM and often produce 5HT which can lead to carcinoid syndrome
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where do carcinoid tumors occur?
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small intestine - they make up 50% of small bowel tumors;
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wheezing, right sided heart murmurs, diarrhea, and flushing
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carcinoid tumor
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what happens if a carcinoid tumor is completely confined to the GI system?
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no carcinoid syndrome occurs due to liver metabolzism of 5HT
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define liver cirrhosis
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diffuse fibrosis of liver that destroys normal architecture, with nodular regneration
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what causes micronodular liver cirrhosis?
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nodules <3cm, uniform size; due to metabolic insult - alcohol, hemochromatosis, Wilson's disease
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what causes macronodular cirrhosis of the liver?
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nodules >3cm, varied in size; due to significant liver injury leading to necrosis (postinfectious or drug induced hepatitis)
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which increases the risk of hepatocellular carcinoma, micro- or macronodular liver cirrhosis?
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macronodular
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what can relieve portal HTN?
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portacaval shunt between splenic vein and left renal vein
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what disease is ALT a good marker for?
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viral hepatitis (ALT > AST)
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what disease is AST a good marker for?
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alcoholic hepatitis (AST > ALT) and MI
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what disease is GGT (gamma-glutamyl transpeptidase) a good marker for?
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increases with heavy alcohol consumption
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what disease is alkaline phosphatase a good marker
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obstructive liver disease (hepatocellular carcinoma), bone disease, and bile duct disease
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what disease is amylase a good marker for?
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acute pancreatitis, mumps
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what disease is lipase a good marker for?
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acute pancreatitis
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what disease is ceruloplasmin a good marker for?
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decreased in Wilson's disease
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what is Reye's syndrome?
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rare, often fatal childhood hepatoencephalopathy
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findings: fatty liver, hypoglycemia nd coma
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Reye's syndrome
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what is reye's syndrome associated with?
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viral infection (VZV and influenza B) that has been treated with salicylates - aspirin is not recommended for children!
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what are the 3 types of alcoholic liver disease?
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1. hepatic steatosis, 2. alcoholic hepatitis, 3. alcoholic cirrhosis
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define hepatic steatosis
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short term cahnge with moderate alcohol intake; macrovesicular fatty change that can be reverse when you stop drinking
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what causes alcoholic hepatitis?
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sustained, long term consuption
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define alcoholic hepatitis
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swollen and necrotic hepatocytes with neutrophilic infiltration and mallory bodies (intracytoplasmic eosinophilic inclusions
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what is the AST/ALT ratio in alcoholic hepatitis?
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AST > ALT, ratio usually greater than 1.5
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define alcoholic cirrhosis
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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
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what is the most common primary malignant tumor of the liver in adults?
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hepatocellular carcinoma/hepatoma
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what are risk factors for hepatocellular carcinoma?
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HBV and HCV, Wilson's disease, hemochromatosis, alpha1-antityrpsin deficiency, alcoholic cirrhosis and carcinogens
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how does hepatocellular carcinoma spread?
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by hematogenous dissemination
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findings: tender hepatomegaly, ascities, polycythemia and hypoglycema
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hepatocellular carcinoma/hepatoma
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what causes nutmeg liver?
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backup of blood into the liver, commonly due ot RCHF and Budd-Chiari syndrome
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what is increased in hepatocellular carcinoma?
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alpha-fetoprotein, can lead to Budd Chiari syndrome
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what can result from nutmeg liver?
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centrilobular congestion and necrosis can result in cardiac cirrhosis
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what is budd-chiari syndrome?
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occlusion of IVC or hepatic veins with coentrilobular congestion and necrosis that leads to congestive liver disease
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what are the findings in budd-chiari syndrome?
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varices, visible abdmonial and back veins, and absent JDV
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what is associated with budd-chiari syndrome?
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polycythemia vera, pregnancy and hepatcellular carcinoma
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what is alpha1-anti-trypsin deficiency?
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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
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what are the findings of hetaocellular jaudice: hyperbilirubinemia, urine bilirubin and urine urobilinogen
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hyperbilirubienima: conjugated/unconjugated, urine bilirubin: incrased; urine urobilinogen: normal or low
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what are the findings of obstructive jaudice: hyperbilirubinemia, urine bilirubin and urine urobilinogen
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hyperbilirubienima: conjugated, urine bilirubin: incrased; urine urobilinogen: low
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what are the findings of hemolytic jaudice: hyperbilirubinemia, urine bilirubin and urine urobilinogen
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hyperbilirubienima: unconjugated, urine bilirubin: absent; urine urobilinogen: increased
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what is Gilbert's syndrome?
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mildly decreased UDP-glucuronyl transferase or decrased bilirubin uptake. Elevated unconjugated bilirubin without overt hemolysis; associated with stress, asymptomatic with no clinical consequences
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what disease has absent UDP-glucuronyl transferase?
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Crigler-Najjar syndrome, type 1
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findings: jaundice, kernicterus, increased unconjugated bilirubin
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Crigler-Najjar syndrome, type 1
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what are the consequences of crigler-najjar syndrome, type 1?
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present early in life, pts die wihtin a few years
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what is crigler-najjar syndrome, type 2?
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less severe than type 1, and responds to phenobarbital which increaes liver enzyme synthesis
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what is the treatment for crigler-najjar syndrome, type 1?
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plasmapheresis and phototherapy
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what disease causes a grossly black liver?
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dubin-Johnson syndrome
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what causes dubin-johnson syndrome?
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conjugated hyperbilirubinemia due to defective liver excretion
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what is Rotor's syndrome?
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a milder form of dubin-johnson syndrome that does not cause black liver
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how do Gilbert's syndrome, Crigler-najjar and dubin-johnson syndromes differ?
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gilberts: problem with bilirubin uptake, crigler najjar: problem with bilirubin conjugation, dubin-johnson: problem with excreted of conjugated bilirubin
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which of the hereditary hyperbilirubinemias presents with conjugated bilirubinemia?
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dubin-Johnson syndrome
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define Wilson's disease
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AR disease of inadequate heaptic copper excretion and failure of copper to enter circulation as ceruoplasmin
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where does copper accumulate in Wilson's disease?
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liver, brain, cornea, kidneys and joints
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how do you treat Wilson's disease?
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penicillamine
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what characterizes Wilson's diseas?
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ABCD: Asterixis, Basal ganglia degernation (parkinsonian), Ceruloplasmin decreased, cirrhosis, corneal depostisi, copper acculumation, carcinoma; Dementia, dyskinesia and dysarthria; hemolytic anemia
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Kayser-Fleischer rings
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Wilson's disease
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what is hemochromatosis?
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the deposition of hemosiderin (iron)
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micronodular cirrhosis, DM and skin pigmentation
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hemochromatosis, also called "bronze" diabetes
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what are the consequences of hemochromatosis?
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CHF and increased risk of hepatocellular carcinoma
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hoe do you get hemochromatosis?
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primary - AR, or secondary to chronic transfusion therapy
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increased ferritin, increased iron, decreased TIBC, increased transferrin saturation
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hemochromatosis, also called "bronze" diabetes
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how do you treat hereditary hemochromatosis?
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repeated phleobotomy, deferoxamine
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what HLA is hemochromatosis associated with
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HLA-A3
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concentric "onion skin" bile duct fibrosis
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primary sclerosing cholangitis
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what is primary sclerosing cholangitis?
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both intra- and extra-hepatic, inflammation and fibrosis of the bile ducts, has an increase in ALP
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alternating strictures and dilation with "beading" on ERCP
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primary sclerosing cholangitis
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what is assoicated with primary sclerosing cholangitis?
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associated with ulcerative colitis, can lead to secondary biliary cirrhosis
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what is primary biliary cirrhosis?
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intrahepatic, autoimmune desorder that causes severe obstructive jaundice, steatorrhea, pruritus and hypercholesterolemia (zanthoma)
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what disease is associated with increased ALP and increased serum mitochondrial Ab
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primary biliary cirrhosis
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what diseases are associated with primary biliary cirrhosis?
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scleroderma and CREST
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what causes secondary biliary cirrhosis?
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extrahepatic biliary obstruction - incrased pressure in the intrahepatic ducts results in injury and fibrosis
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what disease is assoicated with increased ALP and increased conjugated bilirubin?
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secondary biliary cirrhosis
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what complicates secondary biliary cirrhosis?
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ascending choangitis (bacterial infection) bile stasis and "bile lakes"
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when are gallstones (cholelithiasis) formed?
|
when solubilizing bile acids and lecithin are overwhelmed by increased cholesterol and or bilirubin
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which type of stone is most common?
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cholesterol stones
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what are cholesterol stones associated with ?
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obesity, crohn's disease, CF, advanced age, clofibrate, estrogens, multipartiy, rapid weight loss and native america origin
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what gallstones are radiolucent?
|
cholesterol stones - 10-20% opaque due to calcifications
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what are the 4 risk factors for gallstones?
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female, fat, fertile, forty
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triad: 1. Jaundice, 2. fever, 3. RUQ pain
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charcot's triad of cholangitis
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|
which gallstones are radiopaque?
|
pigment stones
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what is assoicated with pigment stones?
|
chronic RBC hemolysis, alcoholic cirrhosis, advanced age and biliary infection
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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
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what causes air to be present in the biliary tree on imaging?
|
if gallstones obstruct the ileocecal valve
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how do you diagnose and treat gallstones (cholelithiasis)?
|
diagnose by ultrasound, treat with cholecystectomy
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what is Murphy's sign?
|
inspiratory arrest on deep palaption, caused by gallstones
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what is cholecystitis?
|
inflammation of the gallbladder - either infectious (CMV, crypto) or due to gallstone complication
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what is a marker of ascending cholangitis?
|
increased ALP when bile duct becomes involved
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|
define acute pancreatitis
|
autodigestion of pancreas by pancreatic enzymes
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|
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)
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|
|
epidgastric abdominal pain radiating to back, anorexia and nausea
|
acute pancreatitis
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what lab values are associated with acute pancreatitis?
|
elevated amylase and lipase (higher specificity)
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what are the consequences of acute pancreatitis?
|
can lead to DIC, ARDS, diffuse fat necrosis, hypocalcemia, pseudocyst formaiton, hemorrhage and infection
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what are the consequences of chronic pancreatitis?
|
preacreatic insufficiency - steatorrhea, fat-soluble vitamin deficiency and DM
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what type of chronic pancreatic injury is associated with alcoholism?
|
chronic calcifying pancreatitis
|
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|
what location of the pancrease are tumors more commonly found?
|
in the head, can lead to obstructive jaundice
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|
what cancer is associated with markers: CEA and CA-19-9?
|
pancreatic adenocarcinoma
|
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|
what is a risk factor for developing pancreatic adenocarcinoma?
|
cigarettes but NOT EtOH
|
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|
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
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what is Trousseau's syndrome?
|
migratory thrombophlebitis - redness and tnederness on palaption of extremities, seen in pancreatic adenocarcinoma
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what is courvoisier's sign?
|
obstructive jaundice with plapable gallbladder, seen in pancreatic adenocarcinoma
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