Gastrointestinal Flashcards

1
Q

Midgut development

A

The midgut is the lower duodenum to the 2/3 transverse colon

6th week- physiologic midgut herniates though umbilical ring

10th week-returns to abdominal cavity and rotates around superior mesenteric artery for total 270 counterclockwise

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2
Q

Where is the defect in gastroschisis and omphaloceles during development?

A

lateral fold closure

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3
Q

extrusion of abdominal contents through abdominal folds (R of umbilicus) NOT covered by peritoneum or amnion?

A

Gastroschisis

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4
Q

Failure of lateral walls to migrate at umbilical ring results in persistent midline herniation of abdominal contents into umbilical cord. Gut contents are surrounded by peritoneum

A

Omphaloceles

“O” surrounds the gut

related to congenital anomalies

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5
Q

What is the most common tracheoesophageal anomaly?

A

Esophageal atresia with distal tracheoesophageal fistulae

presents with polyhydramnios in utero because unable to swallow

vomit with first feeding

Air can enter the stomach compared to a pure EA where the stomach is gas less

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6
Q

Pt is a 1-2 day old baby with downs syndrome that presents with bilious vomiting and abdominal distention. What is this baby likely to have? what is the xray sign?

A

duodenal atresia due to failure to recanalze

double bouble on xray

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7
Q

Pt presents with bilious vomiting and abdominal distention within first 1-2 days of life and the apple peel sign on xray

A

jejunal and ileal atresia

disruption of mesenteric vessels leading to ischemic necrosis and segmental resorption (bowel discontinuity)

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8
Q

What is the most common cause of gastric outlet obstruction in infants?

A

hypertrophic pyloric stenosis

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9
Q

Infant presents with a palpable olive shaped mass in the epigastric region and nonbillious projectile vomiting with visible peristaltic waves at 2-6 wks old

A

Hypertrophic pyloric stenosis

more in firstborn males
associated withe exposure to macrolides

vomiting empties gastric acid and results in hypokalemic and hypochloremic metabolic alkalosis

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10
Q

What becomes the body, tail, isthmus and accessory pancreatic duct?

A

dorsal pancreatic bud

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11
Q

What becomes the pancreatic head?

A

Both dorsal and ventral pancreatic bud

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12
Q

What becomes the uncinate process and the main pancreatic duct?

A

ventral pancreatic bud

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13
Q

Annular pancreas

A

abnormal rotation of ventral pancreatic bud results in a ring formation that encircles the second part of the duodenum causing duodenal narrowing and vomiting

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14
Q

When the ventral and dorsal buds fail to fuse at 8 weels

A

pancreas divisum

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15
Q

What is the spleen derived from?

A

mesoderm but has foregut supply from celiac trunks splenic artery

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16
Q

retroperitoneal organs

A
suprarenal/adrenal glands
Aorta/IVC
Duodenum (2nd through 4th part)
Pancreas (except tail)
Ureters
Colon (descending and ascending)
Kidneys
Esophagus (thoracic portion)
Rectum (partially)

“SAD PUCKER”

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17
Q

Falciform ligament

A

liver to anterior abdominal wall

contains the ligmentum teres hepatis and patent paraumbilical veins

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18
Q

Hepatoduodenal ligament

A

Liver to duodenum

contains the portal triad: proper hepatic artery, portal vein, common bile duct

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19
Q

Pringle manuver

A

Hepatoduodenal ligament may be compressed between thumb and index finger placed in omental foramen to control bleeding

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20
Q

Gatrohepatic ligament

A

liver to less curvature of stomach

contains the gastric vessels

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21
Q

Gatrocolic ligament

A

connects greater curvature and transverse colon

contains the gastroepiploic arteries

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22
Q

Gastrosplenic ligament

A

connects the greater curvature and spleen

contains teh short gastrics, left gastroepiploic vessels

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23
Q

Splenorenal ligament

A

connects the spleen to posterior abdominal wall

contains the splenic artery and vein, tail of pancreas

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24
Q

Layers of gut wall

A

Mucosa- epithelium –> lamina propria–> muscularis mucosa

submucosa- submucosal nerve plexus (meissner)

Muscularis externa - inner circular mm, myenteric nerve plexus (auerbach), outer longitudinal mm

Serosa if intraperitoneal/adventitia if retroperitoneal

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25
Cell type in the esophagus?
nonkeratinized stratified squamous epithelium
26
Brunners glands
In duodenum HCO3 secreting cells of submucosa
27
Crypts of lieberkuhn
In duodenum, jejunum, ileum, colon contains stem cells ( in duodenum they can replace enterocytes/goblet cells and paneth cells tat secrete defensins, lysozyme, and TNF)
28
Plicae circulares
In distal duodenum, jejunum, proximal ileum
29
Peyers patches
in ileum lymphoid aggregates in lamina propria and submucosa
30
Largets number of goblet cells in the small intestine is in the?
ileum Colon also has abundant goblet cells
31
Vertebral levels of major abdominal arteries - celiac trunk, SMA, IMA? Bifurcation of aorta?
celiac- T12 SMA-L1 IMA-L3 Bifurcation of aorta at L4 "biFOURcation"
32
In low body weight patients, we worry about superior mesenteric artery syndrome...which is?
characterized by intermittent intestinal obstruction symptoms primarily post prandial pain when SMA and aorta compress transverse (3rd) part of duodenum
33
Where is the colon prone to ischemia?
at watershed areas where you get distal arterial branches 1) splenic flexure -SMA and IMA 2) rectosigmoid junction - the last sigmoid arterial branch from the IMA and superior rectal artery
34
blood supply and innervation of foregut? midgut? hindgut?
foregut is celiac artery with parasymp innervation from vagus Midgut is SMA with parasympathetic innervation from vagus Hindgut is IMA with pelvic parasympathetics
35
Branches of celiac trunk?
hepatic, splenic, left gastric
36
Posterior duodenal ulcers run the risk of penetrating what artery and causing hemorrhage?
gastroduodenal artery
37
What is the risk with anterior duodenal ulcers?
perforate into the anterior abdominal cavity and potentiall lead to pneumoperitoneum
38
portal systemic anastomoses: esophagus
left gastric with azygos clinical sign is esophageal varices
39
portal systemic anastomoses: umbilicus
paraumbilical with small epigastric veins of ant ab wall clinical sign is caput medisae
40
portal systemic anastomoses: rectum
superior rectal with middle and inferior rectal clinical sign is anorectal varices
41
Anorectal varices, caput medusae, esophageal varices are commonly seen with
portal HTN TX then with transjugular intrahepatic portosystemic shunt (TIPS) between portal and hepatic vein that shunts blood to the systemic circulation, bypassing liver and reducing portal HTN
42
What is the pectinate/dentate line? why is it important?
where the endoderm (hindgut) meets ectoderm above line: internal hemorrhoids (not painful because visceral innervation), adenocarcinoma below line: external hemorrhoids (painful because somatic innervation from inferior rectal branch of pudendal nerve), anal fissures, squamous cell carcinoma
43
Anal fissures are most commonly located?
below pectinate line posteriorly because poorly perfused
44
Liver tissue function unit and struture
the functional unit of the liver is made up of hexagonally arranged lobules surrounding the central vein with portal triads on the edges (consisting of portal vein, hepatic artery, bile ducts, lymphatics)
45
Kupffer cells
specialized macrophages in liver
46
Hepatic stellate (Ito) cells
in space of Disse store vitamin A and produce extracellular matrix when activated --> hepatic fibrosis
47
Liver zones
Zone I: perioportal zone - affected first by viral hepatitis and ingested toxins Zone II: intermediate zone - related to yellow fever Zone III: pericentral vein (Centrilobular)zone - affected first by ischemia, high cytochrome P450, most sensitive to metabolic toxins, site of alcoholic hepatitis
48
Tumors of the head of the pancrease commonly cause?
obstruction of the common bile duct that results in enlarged gallbladder with painless jaundice (courvoisier sign) commonly andenocarcinoma
49
Femoral structures from lateral to medial? Whats in the femoral triangle? Femoral sheath?
- Nerve-Artery-Vein-Lymphatics - "NAVeL" - NAV is in the femoral triangle - "Venous near the penis" - sheath that is below the inguinal ligament that contains femoral vein, artery, and canal (deep inguinal lymph nodes) but not femoral nerve
50
Muscles of the inguinal canal
Transversus abdominis mm Internal oblique mm which fuse into a conjoined tenden
51
Spermatic cord structure
ICE tie Internal spermatic fascia (transversalis fascia) Cremasteric muscle and fascia (internal oblique) External Spermatic fascia (external oblique)
52
Hesselbach triangle
Inguinal triangle - inferior epigastric - lateral border of ther ectus abdominis - inguinal ligament
53
Diaphragmatic hernia? where is it more common?
abd structures enter the thorax. mostly on the left side because the liver protects the right side
54
Most common diaphragmatic hernia? What is the other type?
- sliding hiatal hernia is most common. The GE junction is displaced upward as gastric cardia slides into the esophageal hiatus . "hourglass stomach" - paraesophageal hiatal hernia is when the gastric fundus herniates
55
This type of hernia follows the path of descent of the testes and is covered by all 3 layers of spermatic fascia
Indirect inguinal hernia - Lateral to inferior epigastrics (MDs dont LIe - medial is direct hernia, Lateral is indirect) goes through the internal (deep) inguinal ring, external (superficial) inguinal ring, and into the scrotum
56
What causes an indirect inguinal hernia?
caused by failure of processus vaginalis to close and can form a hydrocele more common in males
57
This type of hernia protrudes through the inguinal triangle
Direct inguinal hernia bulges directly though parietal peritoneum medial to the inferior epigastric vessels but lateral to rectus abdominis ONLY goes through external (superficial) inguinal ring
58
This hernia protrudes below the inguinal ligament through the femoral canal below and lateral to pubic tubercles
Femoral hernia mostly females more likely to present with incarceration or strangulation than inguinal hernias
59
Gastrin
- From G cells of the antrum of stomach and duodenum - increases H+ gastric secretion - Increases gastric mucosa growth and motility effect is due to enterochromaffin-like cells (ECL) that leads to histamine release rather than through its direct effect on parietal cells
60
Somatostatin
- From D cells in pancreatic islets and GI mucosa - Decrease gastric acid and pepsinogen secretion - Decrease pancreatic and small intestine fluid secretion - Decrease gallbladder contraction - Decrease insulin and glucagon release high acidity induces release
61
Cholecystokinin
- From I cells in duodenum and jejunum - Increase pancreatic secretion - Increase gallbladder contraction - Decrease gastric emptying - Increase sphincter of Oddi relaxation
62
Secretin
- From S cells in the duodenum - Increase pancreatic HCO3 secretion - decrease gastric acid secretion - increase bile secretion
63
Glucose-dependent insulinotropic peptide/gastric inhibitory peptide (GIP)
- From K cells (duodenum, jejunum) - exocrine: decrease gastric H+ secretion - endocrine: increase insulin release
64
Motilin
- From small intestine | - Produces migrating mtoor xomplexes (MMCs)
65
Vasoactive Intestinal Polypeptide
- From parasympathetic ganglia in sphincters, gallbladder, small intestine - Increase intestinal water and electrolyte secretion - Increase relaxation of intestinal smooth mm and sphincters
66
Nitric Oxide
- increase smooth muscle relaxation, including lower esophageal sphincter - Loss of NO related to achalasia's increase lower esophageal sphincter tone
67
Ghrelin
- From stomach | - Increases appetite
68
Intrinsic factor
- From parietal cells of stomach | - Vitamin B12 binding protein (required for B12 uptake in terminal ileum)
69
Gastric acid
- From parietal cells of stomach - Decreases stomach pH - Regulation: increases with histamine, vagal stimulation (ACh), gastrin. Decreases with somatostatin, GIP, secretin
70
Pepsin
-From chief cells (Stomach) -Protein digestion REgulation: increase by vagal stimulation (ACh)
71
Bicarbonate
- From mucosal cells in stomach, duodenum, salivary glands, pancreas and brunners glands in duodenum - Neutralizes acid
72
Pancreatic alpha amylase
starch digestion released in active form
73
Pancreatic lipases
fat digestion
74
Panctreatic proteases
protein digestion trypsin, chymotrypsin, elastase, carboxypeptidases which are secreted as proenzymes
75
Pancreatic trypsinogen
converted to active trypsin (by enterokinase/enteropeptidase on brush border) which then goes on to activate other proenzymes and cleaving of additional trypsinogen molecules into active trypsin
76
Where is Fe absorbed? Folate (B9)? B12?
- duodenum for Fe - small bowel for folate - terminal ileum for B12
77
Peyers patches
- unencapsulated lymphoid tissue found in lamina propria and submucosa of ileum - specialized M cells sample and present antigens to immune cells - B cells in germinal centers differentiate into IgA secreting plasma cells which will reside in lamina propria - IgA receives protective secretory component and is then transported across epithelium to deal with intraluminal antigens
78
What are bile acids conjugates to
glycine | taurine
79
What is teh rate limiting step of bile acid synthesis
cholesterol 7alpha hydroxylase
80
Low bile salt absorption can result in what kind of stones and why
calcium oxalate kidney stones Ca which normally binds oxalate, binds fat instead and so free oxalate is absorbed by gut and causes stones
81
Bilirubin pathway
1) macrophages break down RBCs and release heme which is metabolized by heme oxygenase to biliverdin which is reduced to bilirubin 2) unconjugated bilirubin/indirect is water insoluble and is in macrophages 3) enters bloodstream and joins albumin to form unconjugated bilirubin albumin complex 4) enters liver and via UDP-glucuronosyl-transferase --> conjugated bilirubin/direct bilirubin/water soluble 5) gut bacteria turns this into urobilinogen which 80% excreted by feces as stercobilin (brown color) and rest either excreted in urine as urobilin (yellow color) or go to liver via enterohepatic circulation
82
Sialolithiasis
Stones in salivary gland single stone more common in submandibular gland (wharton duct) caused by dehydration or trauma
83
Sialadenitis
inflammation of salivary gland
84
Salivary gland tumors
most commonly benign and in parotid gland presents as painless mass/swelling Facial pain or paralysis suggests malignant involvement of CN VII
85
The most common salivary gland tumor
pleomorphic adenoma- benign mixed tumor of chondromyxoid stroma and epithelium
86
The most common malignant tumor of salivary gland
Mucoepidermoid carcinoma has mucinous and squamous components
87
A benign cystic tumor of salivary gland with germinal centers
warthin tumor (papillary cystadenoma lymphomatosum) seen in smokers Bilateral in 10% Multifocal in 10%
88
Barium swallow shows dilated esophagus with an area of distal stenosis "birds beak sign"
Achalasia-Failure of LES to relax due to loss of myenteric (auerbach) plexus due to loss of postganglionic inhibitory neurons (which contain NO and VIP) Increased risk of esophageal cancer
89
Pt presents with crepitus felt in neck region and chest wall
Boerhaave syndrome Transmural (compared with mallory weiss which is non transmural) Distal esophageal rupture with pneumomediastinum due to violent retching subq emphysema may be due to dissecting air
90
Infiltration of eosinophils in the esophagus
Eosinophilic esophagitis esophageal rings and linear furrows often seen on endoscopy unresponsive to GERD therapy
91
Esophageal strictures
Associated with caustic ingestion (Strong acid/base) and acid reflux
92
Esophagitis
reflux, infection in immunocompromised (candida causes pseudomembrane, HSV-1 causes punched out lesion, CMV causes linear ulcers), caustic ingestion, or pill esophagitis
93
Mallory Weiss syndrome
partial thickness mucosal lacerations at gastroesophageal junction due to severe vomiting alcoholics and bulimics
94
pt presents with triad of dysphagia, iron deficiency anemia, and esophageal webs
Plummer vinson syndrome increased risk of esophageal squamous cell carcinoma "Plumbers DIE"
95
Pt presents with esophageal smooth muscle atrophy as part of CREST syndrome
Sclerodermal esophageal dysmotility decreased LES pressure and dysmotility --> acid reflux and dysphagia --> stricture, barrett esophagus, and aspiration
96
The replacement of nonkeratinized stratified squamous epithelium with intestinal epithelium (nonciliated columnar with goblet cells in distal esophagus)
Barrett esophagus can cause esophageal adenocarcinoma
97
Esophageal cancer that affects the upper 2/3rd of the esophagus and is most common worldwide
squamous cell carcinoma due to alcohol, hot liquids, caustic strictures, smoking, achalasia
98
Esophageal cancer that affects the lower 1/3 of the esophagus and is most common in america
Adenocarcinoma due to chronic GERD, Barrett esophagus, obesity, smoking, achalasia
99
Why do NSAIDs cause erosions in acute gastritis?
they decrease PGE2 which decreases gastric mucosa protection
100
Curling ulcer in acute gastritis
Burns cause curling ulcer which has hypovolemia --> mucosal ischemia "burned by the curling iron"
101
Cushings ulcer in acute gastritis
Brain injury causes increase in vagal stimulation causes increase in ACh and thus H+ production "always cushion the brain"
102
hypochlorhydria
refers to states where the production of hydrochloric acid in gastric secretions of the stomach and other digestive organs is absent or low,
103
H pylori in chronic gastritis
affects antrum first and spreads to body of stomach worry about MALT lymphoma
104
Menetrier disease
Hyperplasia of gastric mucosa causes hypertrophied rugae and causes excess mucus production with resultant protein loss and parietal cell atrophy with decrease acid production precancerous pt presents with epigastric pain, anorexia, weight loss, vomiting, edema (due to protein loss)
105
Gastric cancers are commonly _____
adenocarcinoma can present with acanthosis nigricans and leser trelat sign Associated with blood type A
106
Diffuse gastric cancer
signet ring cells (mucin filled cells with peripheral nuclei) stomach wall grossly thickened and leathery (linitis plastica)
107
Intestinal gastric cancer
commonly on lesser curvature and looks like ulcer with raised margins
108
Virchow node
involvement of left supraclavicular node by metastasis from stomach
109
Krukenberg tumor
bilateral metastases to ovaries. abundant mucin secreting signet ring cells
110
Sister Mary joseph nodule
subq periumbilical metastasis
111
affect of meals on gastric ulcer? duodenal ulcer?
Gastric ulcer - greater pain with meals + weight loss | duodenal ulcer-paind ecreases with meals + weight gain
112
Ruptured gastric ulcer on the lesser curvature of stomach
bleeding from left gastric artery
113
Ruptured ulcer on the posterior wall of duodenum
bleeding from gastroduodenal artery
114
Duodenal hemorrhhaging is more common in post or ant?
post>ant
115
Duodenal perforation is more common in aant or post?
ant>post
116
Celiac disease/gluten sensitive enteropathy/celiac sprue
autoimmune mediated intolerance of gliadin protein in gluten --> malabsorption and steatorrhea. Can get dermatitis herpetiformis
117
Celiac dz is associated with what HLA?
HLA-DQ2 HLA-DQ8 couldnt eat her 28 DQ icecreams
118
pt presents with IgA anti tissue transglutaminase (IgA tTG), anti-endomysial, anti deamidated gliadin peptide antibodies
Celiac dz - villous atrophy - crypt hyperplasia - intrapeithelial lymphocytosis primarly affects distal duodenum and or proximal jejunum
119
Dxylose test
passive absorbed in proximal small intestine; blood and urine levels decrease with mucosa defects or bacterial overgrowth
120
Lactose hydrogen breath test
positive for lactose malabsorption if post lactose breath hydrogen value rises>20 ppm compared with baseline
121
Tropical sprue
similar to celiac sprue except responds to antibiotics associated with megaloblastic anemia due to folate deficiency and later B12 deficiency
122
Disease caused by Tropheryma Whipplei, an intracellular gram +
PAS + foamy macrophages in intestinal lamina propria Whipple disease causes mostly in older men cardiac symptoms Arthralgias Neurologic symptoms "foamy whipped cream in a CAN"
123
Crohns disease
- Any portion of GI tract can be affected but mostly ileum and colon - Skip lesions with rectal sparring - transmural inflammation causing fistulas - Cobblestone mucosa, creeping fat, bowel wall thickening causes string sign on barium swallow xray - non caseating granulomas and lymphoid aggregates - Th1 mediated - kidney stones due to calcium oxalate and gallstones - + anti-saccharomyces cerevisiae antibodies (ASCA)
124
Ulcerative colotis
- Continuous colonic lesions that extend proximally - Rectal involvement - mucosal and submucosal only - superficial and deep ulcerations - loss of haustra causes lead pipe appearance on imaging - bloody diarrhea - no granulomas - Th2 mediated - toxic megacolon - associated with primary sclerosing cholangitis (p-ANCA)
125
Cause of appendicitis in adults? in children?
in adults its fecalith | In children its lymphoid hyperplasia
126
Diverticulum
blind pouch protruding from the alimentary tract that communicates with the lumen of the gut
127
Only mucosa and submucosa outpuch. Especially where the vasa recta perforate muscularis externa
if acquired then its a "false diverticula" or pseudodiverticulum
128
All gut layers outpouch
True diverticulum (i.e. Meckels)
129
Diverticulosis is common in the
sigmoid colon and caused by increase intraluminal pressure and focal weakness in colonic wall can get painless hematochezia or diverticulitis --> LLQ, fever, leukocytosis
130
Patient is an elder male with dysphagia, obstruction, gurgling, aspiration, foul breath, and a neck mass
Zenker diverticulum - pharyngoesophageal false diverticulum esophageal dysmotility causes hernaition of mucosal tissue at killian triangle between the thyropharyngeal and cricopharyngeal parts of the inferior pharygneal constrictor
131
Meckels diverticulum
true diverticulum due to the persistence of the vitelline (omphalomesenteric) duct findings: hematochezia, melena, RLQ pain, intussusception, volvulus, or obstruction near terminal ileum
132
Rules of 2 for Meckels diverticulum
``` 2x more in males 2 inches long 2 feet from ileocecal valve 2% of population first 2 years of life 2 types of epithelia (gastric and pancreatic) ```
133
Patient presents with bilious emesis, abdominal distention, and failure to pass meconium within 48 hours (chronic constipation). A portion of the colon is dialated. Patient mentions having explosive expulsion of feces (squirt sign) and now has an empty rectum on digital exam
Hirschsprung disease Congential megacolon that lacks gangion cells/enteric nervous plexus (auerbach and meissner plexuses) in distal segment of colon. dilated portion is near the aganglionic segment and results in transition zone risk increases with down syndrome RET mutation int he RecTum
134
Treatment for hirschsprung disease
is resection
135
What is seen in the anomaly of improper midgut rotation
malrotation will show small bowel clumped on right side and colon on left there is also formation of fibrous bands called ladd bands can lead to volvulus and duodenal obstruction
136
What causes the coffee bean sign on xray
sigmoid volvulus twisting of portion of bowel around its mesentery which can lead to obstruction and infarction
137
Type of volvulus seen in children? in elderly?
children see midgut volvulus | elderly see sigmoid volvulus which causes the coffee bean sign
138
What is intussusception and where is it commonly seen?
telescoping of proximal bowel segment into the distal segment, commonly at ileocecal junction
139
Patient presents with intermittent abdominal pain with currant jelly stools. Exam found a sausage shaped mass and the ultrasound showed the "target sign"
Intussusception commonly due to Meckels diverticulum (children) or intraluminal mass (adults). Mostly children can be related to Henoch Schonlein purpura, rotavirus, and recent viral infection where peyer patch hypertrophy creates lead point
140
Patient presents with abdominal pain thats out of proportion to physical findings, also you note red currant jelly stools
Acute mesenteric ischemia - critical blockage of intestinal blood flow often due to embolic occlusion of SMA --> causing small bowel necrosis
141
Patient has postprandial epigastric pain. She now has food aversion and weight loss
Chronic mesenteric ischemia - intestinal angina atheroslerosis of celiac artery, SMA, or IMA causes intestinal hypoperfusion
142
Pt has crampy abdominal pain followed by hematochezia. On imaging you notice a thumbprint sign due to mucosal edema/hemorrhage
colonic ischemia - reduction of intestinal blood flow causes ischemia. commonly at watershed areas (splenic flexure and distal colon) elderly
143
Angiodysplasia
- tortuous dilation of vessels --> hematochezia - mostly found in R. sided colon - older patients - associated with aortic stenosis and von willebrand disease - confirmed with angiography
144
What is the most common cause of small bowel obstruction?
adhesions
145
Ileus
intestinal hypomotility without obstruction
146
Meconium ileus is commonly seen in _____
in CF, meconium plug obstructs intestine, preventing stool passage at birth
147
Necrotizing enterocolitis
Seen in premature formula fed infants with immature immune system primarily colonic necrosis with possible perforation can lead to pneumatosis intestinalis, free air in abdomen, portal venous gas
148
Hamartomatous polyps
solitary lesions no significant risk normal colonic tissue with distorted architecture associated with peutz-jeghers syndome and juvenile polyposis
149
Inflammatory pseudopolyps are seen in ___
inflammatory bowel disease - mucosal erosions
150
submucosal polyp examples
lipoma leiomyomas fibroma
151
What polyp type is most common
hyperplastic polyps that can evolve into serratted polyps and more advanced lesions mostly in rectosigmoid region
152
adenomatous polyps
malignant potential (villous on histo > maligant potential than tubular) mutations in APC and KRAS cause chromosomal instability
153
Serrated polyps
Premalignant --> 20% colorectal cancer "sawtooth pattern" of crypts on biopsy CpG island methylator phenotype (CIMP; cytosine base followed by guanine) Defect may silence the MMR gene. Mutations lead to microsatellite instability and mutations in BRAF
154
thousands of polyps arise starting after puberty in a pancolonic fashion and always involves the rectum
Familial adenomatous polyposis --> 100% CRC Autosomal dominant mutation of APC tumor suppressor gene on chromosome 5q21 give prophylactic colectomy
155
Gardner syndrome (familial colorectal polyposis)
1) FAP + osseous and soft tissue tumors 2) congential hypertorphy of retinal pigment epithelium 3) impacted/supernumerary teeth
156
Turcot syndrome
FAP/Lynch syndrome +malignant CNS tumor
157
Autosomal dominant syndrome featuring numerous hamartomas throughout GI tract. Pt also has hyperpigmented mouth, lips, hands, genitalia
Peutz-Jeghers syndrome associated with increased risk of breast and GI cancers
158
Autosomal dominant syndrome in children <5 yo featuring numerous hamartomatous polyps in the colon, stomach, small bowel
Juvenile polyposis syndrome associated with increased risk of CRC
159
Lynch syndrome
Hereditary nonpolyposis colorectal cancer (HNPCC) autosomal dominant mutation of DNA mismatch repair genes with subsequent microsatellite instability 80% progress to CRC and proximal colon is always involved
160
Lynch syndrome is commonly associated with what cancers
- endometrial - ovarian - skin cancers
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Colorectal cancer is most common in
Rectosigmoid>ascending>descending if on the right side we see bleeding If on the left side we see obstruction see "apple core" on barium enema xray
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ascending colorectal cancer
exophytic mass Iron deficieny anemia weight loss if man >50 yo or postmenopausal woman has iron deficiency anemia, you have high suspicion
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Descending colorectal cancer
infiltrating mass partial obstruction colicky pain hematochezia
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Screening for colorectal cancer
at 40 yo if family history or 10 years before the family members presentation normally starts at 50 yo use CEA marker for monitoring recurrence
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Molecular pathogenesis of colorectal cancer
chromosomal instability pathway: mutations in APC cause normal colon to be at risk. Then a KRAS mutation will result in adenoma. Loss of tumor suppressor genes like p53 will result in carcinoma APC loss causes decrease intercellular adhesion and increase proliferation KRAS mutation causes unregulated intracellular signaling Loss of tumor suppressor causes increase in tumorigenesis
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Mutations in FAP? mutations in lynch?
FAP is related to APC mutation (chromosomal instability pathway) Lynch syndrome is mutation or methylation of mismatch repair genes like MLH1 (microsatellite instability pathway)
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This is a common and potentially fatal bacterial infection in patients with cirrhosis and ascites
spontaneous bacterial peritonitis also known as primary bacterial peritonitis mostly aerobic gram negative orgnaisms paracentesis of ascitic fluid absolute neutrophil count (ANC) >250 first line treatment is 3rd generation cephalosporin
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aspartate aminotransferase (AST)
AST>ALT in alcoholic liver disease if AST>ALT in non alcoholic liver disease then worry it has progressed to advanced fibrosis or cirrhosis
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alanine aminotransferase (ALT)
ALT>AST in liver disease
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alkaline phosphatase
increases in cholestasis,infiltrative disorders, bone disease
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gamma glutamyl transpeptidase
increases in various liver and biliary diseases but not in bone disease associated with alcohol use
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Bilirubin
increases in liver dz
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Albumin
decreases in advanced liver dz
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Prothrombin time
increases in advanced liver disease because decrease in production of clotting factors
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Platelets
decrease in advanced liver disease (drop in thrombopoietin) portal hypertension (splenomegaly/splenic sequestration)
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Rare often fatal childhood hepatic encephalopathy
reye syndrome "reye of sunSHINE" ``` S-steatosis of liver/hepatocytes H-hypoglycemia/hepatomegaly I-Infection (VZV, influenza that has been treated with aspirin) N-not awake (coma) E (encephalopathy) ``` mitochondrial abnormalities fatty liver due to microvesicular fatty change
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Why does aspirin increase the risk of reyes syndrome
aspirin decreases beta oxidation by reversible inhibition of mitochondrial enzymes avoid in children unless have kawasaki disease
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Alcoholic hepatitis histo findings
swollen and necrotic hepatocytes with neutrophilic infiltration mallory bodies- intracytoplasmic eosinophilic inclusions of damaged keratin filaments
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alcoholic liver disease progression
hepatic steatosis --> alcoholic hepatitis --> alcoholic cirrhosis
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Non alcoholic fatty liver disease
ALT>AST metabolic syndrome (insulin resistance) obesity --> fatty infiltration of hepatocytes --> cellular ballooning and eventual necrosis may cause cirrhosis and HCC independent of alcohol use
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hepatic encephalopathy
cirrhosis causes a portosystemic shunt which decreases NH3 metabolism --> neuropsychiatric dysfunction due to increased production and absorption OR decreased NH3 removal treatment: lactulose if theres increased generation and rifaximin or neomycin to decrease NH3 producing gut bacteria
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Findings seen in hepatocellular carcinoma/hepatoma and how to dx
``` jaundice tender hepatomegaly ascites polycythemia anorexia spreads hematogenously ``` dx due to increase alpha feto protein, ultrasound, contrast CT/MRI, biopsy
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The most common benign liver tumor that occurs at 30-50 yo. Do not biopsy because of risk of _____
cavernous hemangioma do not because of hemorrhage risk
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What benign liver tumor is often related to oral contraceptibe or anabolic steroid use
hepatic adenoma may regress or rupture (abd pain and shock)
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Liver tumor that malignant and related to arsenic and vinyl chloride exposure. Endothelial origin
Angiosarcoma
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Thrombosis or compression of hepatic veins with centrilobular congestion and necrosis resulting in congestive liver disease. Risk in hepatocellular carcinoma/hepatoma, hypercoagulable states etc.
Budd chiari syndrome ``` hepatomegaly ascites varices abd pain liver failure absence of JVD may cause a nutmeg liver ```
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alpha1-antitrypsin deficiency
results in misfolded gene product protein aggregates in hepatocellular ER --> cirrhosis with PAS + globules in liver young pt, liver damage, dyspnea, no history of smoking
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how does alpha1-antitrypsin deficiency affect the lings?
decrease in alpha1-antitrypsin causes uninhibited elastaste in alveoli and this decrease in elastic tissue and panacinar emphysema
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Jaundice is due to an increase in ______ and is caused by _______, ______, ______, ________
high bilirubin think HOT liver H-hemolysis O-obstruction T-Tumor Liver disease
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Unconjugated (indirect) hyperbilirubinemia
hemolytic physiologic (newborns) crigler-najjar Gilbert syndrome
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conjugated (direct) hyperbilirubinemia
``` biliary tract obstruction biliary disease (primary sclerosing cholangitis and primary biliary cholangitis) excretion defects (dubin johnson syndrome or rotor syndrome) ```
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Mixed (direct and indirect) hyperbilirubinemia
hepatitis | cirrhosis
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Physiologic neonatal jaundice is due to immature _____
immature UDP-glucuronosyltransferase --> unconjugated hyperbilirubinemia --> jaundice/kernicterus (deposition of unconjugated lipid soluble bilirubin in the brain, particularly basal ganglia)
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Treatment for physiologic neonatal jaundice
phototherapy (non-UV) isomerizes unconjugated bilirubin to water soluble form occurs after first 24 hours of life and usually resolves without treatment in 1-2 weeks
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______ are converted to bilirubin. _______ Bilirubin is bound to serum albumin and transferred to the liver where it is turned into _______ by ________. ______ is excreted into bile
Heme molecules (from hemoglobin) Unconjugated (or indirect) --> water insoluble conjugated (direct)/bilirubin diglucuronide --> water soluble UDP-glucuronosyl transferase Conjugated (direct) bilirubin is excreted into bile.
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All hereditary hyperbilirubinemias are ______
autosomal recessive
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Gilbert syndrome
Mild decrease in UDP-glucuronosyltransferase conjugation and impaired bilirubin uptake increases unconjugated bilirubin without overt hemolysis
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Crigler-Najjar syndrome, type I
absent UDP-glucuronosyltransferase causes increase in unconjugated bilirubin presents early and die within few years. Need liver transplant to cure. Type II is less severe and responds to phenobarbital which increases liver enzyme synthesis jaundice kernicterus - bilirubin deposition in brain
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Dubin Johnson syndrome
conjugated hyperbilirubinemia due to defective liver excretion grossly black dark liver benign
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Rotor syndrome
similar to dubin johnson syndrome milder and without black liver due to impaired hepatic uptake and excretion
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Wilsons disease (hepatolenticular degeneration) is _____ inheritence and a mutation in _______. Describe the pathophys
autosomal recessive mutation in hepatocyte copper transporting ATPase (ATP7B gene; chromosome 13) decrease copper incorporation into apoceruloplasmin and excretion into bile --> drop in serum ceruloplasmin copper accumulates in liver, brain, cornea, kidneys
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Kayser fleischer rings
deposits in descemet membrane of cornea in wilsons disease
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deficits in wilsons disease
- neurologic - liver - psychiatric - kayser fleischer rings - hemolytic anemia - renal disease
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tx for wilsons disease
chelation with penicillamine or trientine, oral zinc
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Hemochromatosis inheritence, mutation, HLA type
autosomal recessive C282Y mutation on HFE gene of chromosome 6 associated with HLA A3
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Abnormal iron sensing and increased intestinal absorption that causes iron overload. What is this disease? where does iron like to accumulate?
hemochromatosis in liver, pancreas, skin, heart, pituitary, joints iron can be identified with prussian blue stain
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Patient is 40 yo and has liver cirrhosis, diabetes mellitus, and is now complaining of increase skin pigmentation. on blood work you notice there is >20 g total body iron. What is the pt diagnosis and what else are they at risk for? What is the common cause of their death? treatment?
hemochromatosis restrictive cardiomyopathy (classic) or dilated cardiomyopathy (revesible) hypogonadism arthropathy due to calcium pyrophosphate deposition, esp in metacarpophalangel joints high risk of HCC death
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Treatment for hemochromatosis
repeated phlebotomy chelation with deferasirox deferoxamine oral deferiprone
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Pt presents with pruritis, jaundice, dark urine, and light colored stool. You also notice hepatosplenomegaly. Labs show high conjugated bilirubin, cholesterol, and ALP
Biliary tract disease
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"onion skin" bile duct fibrosis
primary sclerosing cholangitis bile duct fibrosis causes alternating strictures and dilation with beading of intra and extrahepatic bile ducts on ERCP classically seen in middle aged men with IBD associated with ulcerative cholitis, p-ANCA +, and high igM
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Primary biliary chilangitis
autoimmune reaction that causes lymphocytic infiltrate + granulomas --> destruction of lobular bile ducts classically seen in middle aged women anti-mitochondrial antibody + and high igM
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secondary biliary cholangitis
extrahepatic biliary obstruction that increases pressure in intrahepatic ducts --> injury/fibrosis and bile stasis usually in patients with known obstructive lesions and may be complicated with ascending cholangitis
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high cholesterol, high bilirubin, low bile salts, and gallbladder stasis can cause ____? What are the main risk factors for this disease?
Gallstones (cholelithiasis) 80% are cholesterol stones (radiolucent unless calcified) Can also have pigment stones (black is radiopaque and brown is radiolucent) 4 Fs- female, fat, fertile (multiparity), forty
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Biliary colic
pain occurs due to a gallstone temporarily blocking the bile duct.
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choledocholithiasis
presence of gallstones in common bile duct elevated ALP,GGT, direct bilirubin, and or AST/ALT
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gallstone ileus
fistula between gallbladder and GI tract causes stone to enter GI lumen and obstruct at the ileocecal valve (narrowest point) can see air in biliary tree (pneumobilia)
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What kind of cancer is common in the porcelain gallbladder
calcified gallbladder has high risk of gallbladder adenocarcinoma have prophylactic cholecystectomy
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Charcot triad of cholangitis
jaundice fever RUQ pain
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Reynolds pentad of cholangitis
charcots triad of cholangitis + altered mental status and shock ``` jaundice Fever RUQ pain altered mental status shock (hypotension) ```
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acute pancreatitis
autodigestion of pancreas by pancreatic enzymes on imaging the pancreas is surrounded by edema causes: - idiopathic - gallstones - Ethanol - Trauma - Steroids - Mumps - Autoimmune - Scorpion sting - Hypercalcemia/hypertriglyceridemia (>1000) - ERCP - Drugs "I GET SMASHED"
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Diagnosis criteria for acute pancreatitis
2 of the 3 - acute epigastric pain that radiates to the back - high serum amylase or lipase (more specific) to 3x upper limit of normal - characteristic imaging findings
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complications of acute pancreatitis
``` pseudocyst lined by granulation tissue not epithelium abscess necrosis hemorrhage infection organ failure hypocalcemia ```
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Chronic pancreatitis
chronic inflammation, atrophy, calcification of the pancreas caused by alcohol abuse and genetic predisposition complicated by pancreatic insufficiency ( <10% function and may manifest with steatorrhea and fat soluble vitamin deficiency, diabetes mellitus) and pseudo cysts (surrounded by granulation tissue not epithelium)
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Pancreatic adenocarcinoma
very aggressive tumor arising from pancreatic ducts (disorganized glandular structure with cellular infiltration) often metastatic at presentation associated with CA19-9 tumor marker and also CEA
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patients presents with weight loss, redness and tenderness on palpation of extremities, and pain that radiates to the back
pancreatic adenocarcinoma weight loss is due to malabsorption and anorexia migratory thrombophlebitis (red and tender) is trousseau syndrome courvoisier sign
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courvoisier sign
obstructive jaundice with palpable, nontender gallbladder