1
Q
A
A: normal gastroesophageal junction
B: Barrett esophagus; note the small islands of paler squamous mucosa within the Barrett mucosa
2
Q
describe the image
A
C: histologic appearance of the gastroesophageal junction in Barrett esophagus; note the transition between esophageal squamous mucosa (left) and metaplastic mucosa containing goblet cells (right)
3
Q
describe the etiology of the condition seen in the image
A
- long-standing acid reflux esophagitis → GERD
- more common in males
- more common in whites
- GERD is caused by:
- obesity
- limited scleroderma (CREST)
- E = esophageal dysmotility → GERD
- anti-centromere positive, hiatal hernia
4
Q
describe the pathogenesis of the condition seen in the image
A
- decreased tone in LES → genetic reprogramming of stem cells in the lower 1/3 of the esophagus
- proliferation of progenitor cells for healing which then differentiate into columnar cells → more resistant to peptic acid injury
5
Q
describe the presentation of the condition seen in the image
A
- presentation is similar to reflux
- heart burn → worse when lying down
- dyspepsia
- waterbrush (bad metallic taste of acid in mouth)
- epigastric pain
- substernal discomfort relieved by antacids
6
Q
definitive diagnosis of the condition seen in the image is accomplished by ____
A
definitive diagnosis of the condition seen in the image is accomplished by upper GI endoscopy and biopsy
- endoscopy: normal pearly white esophageal squamous mucosa → velvety pink columnar mucosa
7
Q
describe complications of the condition seen in the image
A
- # 1 risk factor for dysplasia → adenocarcinoma of the esophagus
- ALWAYS d/t Barrett’s
- progressive dysphagia and odynophagia
- melena → iron deficiency anemia
8
Q
describe the etiology of the condition seen in the image
A
- more common in US
- precursor lesion = GERD, Barrett esophagus (dysplasia)
- lower 1/3 of esophagus
9
Q
_____ is the precursor lesion to the condition seen in the image
A
GERD → Barrett esophagus is the precursor lesion to the condition seen in the image
10
Q
describe the risk factors for the condition seen in the image
A
- white men, smokers, obese, previous radiation therapy
11
Q
describe the presentation of the condition seen in the image
A
- presentation:
- signs and symptoms similar to SCC
- dysphagia, initially to solid then to liquid
- odynophagia
- weight loss → cachexia
- chest pain
- vomiting
12
Q
describe the complications of the condition seen in the image
A
- complications:
- melena → iron deficency anemia
- TEF → aspiration pneumonia → lung abscess
- invade heart → pericarditis → percardial effusion
13
Q
describe the image
A
squamous cell carcinoma composed of nests of malignant cells that partially recapitulate the stratified organization of squamous epithelium
14
Q
describe risk factors of the condition seen in the image
A
- etiology:
- fungal contamination
- nitrites (smoked food)
- alcohol
- tobacco use
- GERD
- achalasia
- Tylosis: oral leukoplakia, SCCE, hyperkeratosis of palms and soles
- Plummer-Vinson
- characterized by difficulty in swallowing, iron-deficiency anemia, glossitis, cheilosis and esophageal webs
- Celiac disease
15
Q
describe the pathogenesis of the condition seen in the image
A
- usually in upper 2/3 of esophagus (middle 1/3 = more likely)
- begins as in-situ lesion in the form of squamous dysplasia
- growth pattern; exophytic, excavated (ulcerative), infiltrative
16
Q
describe the presentation of the condition seen in the image
A
- presentation:
- progressive dysphagia (to solids then to liquids)
- odynophagia
- cachexia
- fatigue (d/t melena → iron deficiency anemia)
- hematemesis
- hoarse voice and cough
17
Q
describe investigations for the condition seen in the image
A
- investigations:
- GI endoscopy w/ biopsy: malignant squamous cells invading into the submucosa & muscularis propria
- barium swalllow: shows obstruction of lumen
18
Q
describe complications of the condition seen in the image
A
- complications:
- can obstruct
- bleed (melena) → IDA
- perforate → mediastinitis
- form a TEF (food can get into lungs → aspiration pneumonia → lung abscess)
- can spread to cervical, mediastinal, paratracheal, tracheobronchial, gastric and celiac nodes depending on site of tumor
- direct metastasis to adjacent mediastinal structures including trachea and heart
19
Q
describe the image
A
lymphoid aggregates with germinal centers and abundant subepithelial plasma cells within the superficial lamina propria are characteristic of H. pylori gastritis
20
Q
describe the image
A
spiral-shaped H. pylori bacilli are highlighted with Warthin-Starry silver stain. Organisms are abundant within surface mucus
21
Q
the condition seen in the image is caused by chronic infection with ___ at the ____ region of lesser curvature
A
the condition seen in the image is caused by chronic infection with H. pylori at the antro-pyloric region of lesser curvature
22
Q
describe the histology of the condition seen in the image
A
- histology:
- reactive lymphoid aggregates
- chronic inflammatory infiltrate (lymphocytes, plasma cells) in lamina propria
- H. pylori is G-ve and not invasive
- therefore always seen on luminal surface
- addition of neutrophils = chronic ACTIVE gastritis
23
Q
describe the pathogenesis of the condition seen in the image
A
- produces urease (urea → ammonia to neutralize acid) and phospholipase (destroys phospholipid bilayer in mucosa of stomach) → diffuse effacement of the mucosa by lymphocytes → chronic gastritis/peptic ulcers
- cytotoxin-associated gene A → increases risk for peptic ulcer disease and adenocarcinoma
- flagella → motility in mucoid environment
24
Q
describe investigations of the condition seen in the image
A
- upper GI endoscopy + biopsy with Steiner Silver Stain
- black = organism
- microscopy:
- reactive lymphoid aggregates found just below epithelial lining (**hallmark of H. pylori**)
- inflammatory infiltrate in lamina propria
- PMNs in surface epithelium and glandular lumen
- intestinal metaplasia and glandular atrophy +/- dysplasia
25
in the condition seen in the image, ____ are found just below the epithelial lining which is a hallmark of \_\_\_\_\_
in the condition seen in the image, **reactive lymphoid aggregates** are found just below the epithelial lining which is a hallmark of **H. pylori**
26
in the condition seen in the image, confirmation of the etiologic agent would be supported by \_\_\_\_\_
in the condition seen in the image, confirmation of the etiologic agent would be supported by **regression of tumor with antibiotics**
27
describe the complications of the condition seen in the image
* chronic inflammation → intestinal metaplasia → dysplasia → **intestinal gastric adenocarcinoma**
* lymphoid aggregates → uncontrolled prolif. of B cells → **MALToma** (gastric lymphoma)
* peptic ulcer disease
28
describe the image
diffuse gastric cancers display an infiltrative growth pattern and are composed of discohesive cells with **large mucin vacuoles** that expand the cytoplasm and **push the nucleus to the periphery**, creating a **signet ring cell** morphology
29
describe the etiology of the diffuse form of the condition seen in the image
* etiology:
* mutation = **E-cadherin (CDH1)** → signet ring cells that contain mucin
* no intestinal metaplasia
30
describe the morphology of the **diffuse** form of the condition seen in the image
* morphology:
* **"leather bottle stomach"/****linitis****plastica** becuase signet ring cell infiltrates the stomach wall
* no gland formation: single cells, sheets, clusters
31
describe the complications of the **diffuse** form of the condition seen in the image
* complications:
* **ovarian metastasis → Krukenberg tumor → bilateral (only with diffuse type)**
* Krukenberg: from diffuse gastric adenocarcinoma, invasive lobular carcinoma of breast, and colon cancer
32
describe the etiology of the **intestinal** form of the condition seen in the image
* etiology:
* H. pylori = most common
* autoimmune
* smoked foods (nitrosamines)
33
describe the morphology of the **intestinal** form of the condition seen in the image
* morphology:
* neoplastic cells form glands
* histology: malignant glands that make mucin → invading into the submucosa & muscularis propria
34
describe complications of the **intestinal** form of the condition seen in the image
* complications:
* bleed → IDA
* **left supraclavicular/Virchow's LN metastasis**
* one of the nodes that drains the stomach
* periumbilical metastasis (intestinal) → **Sister Mary Joseph nodule**
35
\_\_\_\_ is a prognostic indicator of the **intestinal** form of the condition seen in the image
**depth of invasion and nodal status** is a prognostic indicator of the **intestinal** form of the condition seen in the image
36
\_\_\_ is the tumor marker for the condition seen in the image
**CEA** is the tumor marker for the condition seen in the image
37
GIST tumors are derived from \_\_\_\_
GIST tumors are derived from **interstitial cells of Cajal**
38
a majority of GIST tumors express ____ and have mutations in \_\_\_\_
a majority of GIST tumors express **CD117** and have mutations in **c-Kit**
39
describe the histology of cells seen in GIST
spindle-shaped tumor cells
40
GIST is treated with \_\_\_\_
GIST is treated with **TKI (Imatinic/Gleevec)**
## Footnote
**same treatment as CML (9,22 translocation)**
41
describe predisposing factors for the condition seen in the image
* predisposing factors:
* tobacco chewing (most common)
* alcohol
* HPV 16 & 18
* jagged teeth
* ill-fitting dentures
42
describe what is seen on biopsy of the condition seen in the image
* investigations:
* biopsy = malignant squamous cells w/ keratin pearls
43
describe complications of the condition seen in the image
* complications:
* spreads via lymphatics → **anterior cervical lymph nodes**
44
describe the image seen
**celiac sprue/disease**
complete loss of villi or total villous atrophy
dense plasma cell infiltrates in the lamina propria
45
describe the etiology of the condition seen in the image
* etiology = **gluten** (wheat, barley, oats, rye)
* specifically **gliadin** protein
* associated with **HLA-DQ2** (more common) & **HLA-DQ8**
46
describe the morphology seen in the condition in the image
* morphology:
* increased intraepithelial lymphocytes (CD8 T cells) in lamina propria
* elongated and hyperplastic crypts
* marked atrophy (flattening) and loss of villi → decreased SA for absorption
47
describe the pathogenesis of the condition seen in the image
* pathogenesis:
* inappropriate immune cell mediated Type IV HS response to gliadin in the proximal small intestine (**duodenum**) →
* gluten is deamidated to form **gliadin by tissue transglutaminase (tTG)** → phagocytosed by APC with **HLA DQ2/DQ8** → presentation to CD4 T cells in lamina propria → cytokine production → destruction of villi
48
describe the pathogenesis of the condition seen in the image
* presentation:
* malabsorption → steatorrhea (foul-smelling, pale bulky stools)
* weight loss
* flatulence
49
the condition in the image mainly affects the \_\_\_\_
the condition in the image mainly affects the **duodenum**
50
describe complications of the condition seen in the image
* **T-cell lymphoma** (**EATL** = enteropathy-associated T-cell lymphoma)
* classic scenario = patient adherent to gluten-free diet with worsening symptoms
* increased risk for small bowel carcinoma
51
in the condition seen in the image, too much IgA causes \_\_\_\_\_
in the condition seen in the image, too much IgA causes **dermatitis herpetiformis (deposition of IgA in the dermal papillae)**
52
describe the image
villi are stout and packed with foamy macrophages
lipid particles --\> dilated lacteals
PAS stain --\> foamy particles in LP
53
describe the etiology of the condition seen in the image
G+ve sickle-shaped actinomycete **Trophyeryma whippeli** (PAS +)
54
describe the pathogenesis of the condition seen in the image
phagocytosis and incomplete degradation G+ve Trophyerma whipplei, which accumulate inside lysosomes of the macrophages (foamy-looking) → **mechanical lymphatic (lacteal obstruction)** → distended/flattened villi → malabsorption
55
list the extra-intestinal manifestations of condition seen in the image
* brain: dementia/seizures
* skin: hyperpigmentation
* lymphadenopathy: intestinal/mesenteric & peripheral
* joints: migratory polyarthritis
* heart: infective endocarditis and aortic valve regurg.
* eyes: uveitis → blindness
56
describe the investigations of the condition seen in the image
* investigations:
* small bowel biopsy:
* distended PAS positive (red) foamy macrophages in lamina propria (mucosa)
* EM → rod-shaped bacilli
57
describe the etiology of the condition seen in the image in adults vs. children
* adult: fecolith obstruction (obstruction of lumen)
* children: d/t lymphoid hyperplasia in the lymphoid follicles of the appendix (follicles are aggregations of lymphoid cells)
58
describe the pathogenesis of the condition seen in the image
* pathogenesis:
* obstruction → continued secretion of mucinous fluid → **increased intraluminal pressure** → **collapse of draining veins** → ischemic injury → bacterial proliferation → **inflammation and edema**
59
describe the investigations of the condition seen in the image
* investigation:
* CBC: increased neutrophils and increased band cells
* biopsy: presence of neutrophils all the way to muscularis propria
* positive for Rovsing, Psoas and Obturator signs
60
describe complications of the condition seen in the image
* complications:
* perforation → peritonitis → septicemia
* peri-appendiceal abscess → liver abscess, bacteremia
61
on biopsy of the condition seen in the image, there are ____ all the way to the \_\_\_\_\_
on biopsy of the condition seen in the image, there are **neutrophils** all the way to the **muscularis propria**
62
describe the image
**diverticulosis**
sigmoid diverticulum showing **protrusion of the mucosa and submucosa** through the muscularis propria
63
in the condition in the image, there is herniated of the ___ and ___ which makes it a \_\_\_\_
in the condition in the image, there is herniated of the **mucosa** and **submucosa** which makes it a **false diverticulum (pseudodiverticulum)**
64
describe where the condition in the image are located (anatomical weakness)
diverticula are located on the **mesenteric border where the vasa recta penetrate the muscle wall** (anatomic weakness site)
65
describe the morphology of the condition seen in the image
* morphology:
* acquired **pseudo-diverticulum** (involves mucosa & a little submucosa -- NOT the entire wall)
* most commonly seen as **flask-like structure in the sigmoid colon**
66
describe the predisposing factors of the condition seen in the image
* predisposing factors:
* elderly on a **low-fiber diet**
* long-standing history of constipation
67
describe the pathogenesis of the condition seen in the image
* pathogenesis:
* decreased dietary fiber → **sustained bowel contractions** and **increased intraluminal pressure** → herniation of colonic wall at sites of focal defects
68
describe the presentation of the condition seen in the image
* presentation:
* asymptomatic, incidental finding on colonoscopy, can cause painless bleeding
69
list complications of the condition seen in the image
* complications:
* lower GI bleed → hematochezia
* perforate → sepsis
* acute diverticulitis (mimics appendicitis) → inflammation of diverticulum
* **enterovesical (colovesical) fistula → fistula with bladder due to perforation**
* chronic diverticulitis → strictures from narrowing of lumen from fibrosis → perforate → septic shock and DIC
* NO malignant potential
70
describe the image
ulcerative colitis
total colectomy with pancolitis showing active disease, with red, granular mucosa in the cecum (left) and smooth, atrophic mucosa distally (right)
71
describe the image
ulcerative colitis
the disease is limited to the **mucosa + submucosa**
72
the condition seen in the image is associated with HLA-\_\_\_ as well as \_\_\_\_\_
the condition seen in the image is associated with HLA-**DRB1** as well as **primary sclerosing cholangitis (p-ANCA)**
73
describe the classic features seen in the condition in the image
* classic features:
* **always starts the rectum** & moves proximally; continuous
* **no skipped lesions** → curable by surgery
74
describe the histology of the condition see in the image
* histology:
* **mucosal and submucosal** involvement
* architectural distortion
* dense chronic inflammation with **basal plasmacytosis**
* basal plasmacytosis = presence of plasma cells between the base of the crypts and the muscularis mucosae
* **crypt abscesses** (PMNs in the lumen of crypts)
* no granulomas
75
describe the gross appearance of the condition seen in the image
* gross:
* mucosa red, granular and friable
* **broad-based ulcers**
* isolated islands of intervening regenerating mucosa bulge creating **pseudopolyps**
76
during a barium enema while investigating the condition in the image, a ____ appearance is seen due to \_\_\_\_
during a barium enema while investigating the condition in the image, a **lead-pipe** appearance is seen due to **loss of haustra**
77
flare-ups of the condition seen in the image is associated with \_\_\_\_
flare-ups of the condition seen in the image is associated with **physical and mental stress**
78
in the condition in the image, in severe cases of pancolitis, the ____ can be affected as well, which is called \_\_\_\_\_
in the condition in the image, in severe cases of pancolitis, the **ileum** can be affected as well, which is called **backwash ileitis**
79
the condition in the image leads to a higher risk of \_\_\_\_
the condition in the image leads to a higher risk of **colon cancer**
80
describe the image
Crohn's disease
linear mucosal ulcers and thickened intestinal wall
81
describe the image
Crohn's disease
haphazard crypt organization results from repeated injury and regeneration
82
describe the image
Crohns disease
transmural Crohn disease with submucosal and serosal non-caseating granulomas
83
the condition in the image is associated with HLA- \_\_\_\_
the condition in the image is associated with **HLA-DR7 and HLA-DQ4**
84
describe the histology of the condition in the image
cobblestone appearance
* sharply delimited & transmural involvement
* non-caseating granulomas
* mucosal fissuring with involvement of fistulas
* skipped lesions that spare the rectum
85
describe the gross appearance of the condition
* gross:
* **linear (deep) ulcers** & **creeping mesenteric fat** due to fibrosis
* thick wall due to edema, hypertrophy, fibrosis and inflammation
* long narrow thickened segments of small intestine
* string sign on radiography
86
the most common location of the condition in the image is \_\_\_\_
the most common location of the condition in the image is **the terminal ileum**
* malabsorption: vit. B12 deficiency, malabsorption of bile salts
* may have non-bloody diarrhea due to malabsorption
87
on barium enema in the condition in the image, _____ is seen due to \_\_\_\_\_
on barium enema in the condition in the image, **string-sign** is seen due to **narrowing of lumen from fibrosis (aka strictures)**
88
on biopsy of the condition seen in the image, there is ____ involvement with ____ and inflammatory infiltrate
on biopsy of the condition seen in the image, there is **transmural** involvement with **non-caseating granulomas** and inflammatory infiltrate
89
describe the extra-intestinal features of the condition seen in the image
* **migratory** **polyarthriris** = most common
* **erythema nodosum** = inflammation of fat under skin (usually shin)
* **gallstones**: malabsorption of bile acids → decreased bile solubility → cholecystitis
* kidney stones
* **ankylosing spondylitis**
* **uveitis**
90
describe complications of the condition seen in the image
smoking can trigger a flare (unlike UC where it is protective)
* **intestinal obstruction** due to fibrosis → perforation → peritonitis
* **malabsorption** if small bowel is affected
* **strictures, fissures** (deep ulcers)
* **fistulas**:
* perianal
* abdominal
* bladder → enterovesical
* **colon cancer** ONLY when colon is involved
91
describe the image
**pseudomembranous colitis**
typical pattern of neutrophils emanating from a crypt is reminiscent of a volcanic eruption
92
describe the etiology of the condition seen in the image
* hospitalized patients on **broad-spectrum antibiotics** (clindamycin; disruption of normal flora) → **C. difficile** exotoxin mediated damage
93
describe the morphology of the condition seen in the image
* **yellow plaques** covering mucosal surface (mucosal itself is not eroded)
* **pseudomembrane**
* fibrinopurulent-necrotic debris, inflammatory cells (mainly neutrophil), necrotic epithelium & mucus
* congested vessels
94
describe the pathogenesis of the condition seen in the image
* exotoxin mediated → denuded surface epithelium & **superficially damaged crypts** distended by mucopurulent exudate erupt to form a **mushrooming cloud** → coalescence of clouds to produce **pseudomembrane** → consists of **inflammatory cells, necrotic debris and fibrin**
* most common location = **rectosigmoid**
95
describe the presentation of the condition seen in the image
* fever, lower abdominal pain, cramps, massive bloody & mucoid diarrhea (dysentery)
96
describe investigations for the condition seen in the image
**assay stool for exotoxin**
97
list complications for the condition seen in the image
* perforate → peritonitis → E. coli sepsis → septic shock
* mucoid diarrhea → hypovolemic shock, hypokalemia & peripheral edema
* hypokalemia → arrhythmias
* toxic megacolon (but hallmark complication of UC)
98
describe the right vs. left sided presentation of the condition in the image
* _right sided = proximal/ascending colon due to **microsatellite instability**_
* **iron deficiency anemia**
* weight loss
* exophytic tumors polypoidal lesion → obstruction is uncommon
* _left sided = distal/descending → adenoma-carcinoma seq._
* LLQ pain
* blood streaked stool w/ a change in stool caliber
* **circumferential lesions → annular, encircling napkin ring constrictions → obstruction and altered bowel movements**
99
describe the etiology of the condition in the image
* gene = **APC (tumor suppressor gene)** which requires 2 hits → inherit 1 bad hit and get 2nd hit later in life → form polyps → **mutations in KRAS** → mutation in **p53** → adenocarcinoma
100
describe the gross morphology of the condition in the image
* **pedunculated morphology** → tubular adenoma
* numerous benign polyps on **left** **side of** **colon** (sigmoid, descending)
* **benign tumor with dysplastic cells** and therefore can become cancer
101
describe the presentation of the condition in the image
* asymptomatic in early stages
* later stages: **bright red bloody & mucoid diarrhea**, intestinal obstruction and LLQ pain
* screen with **sigmoidoscopy** **(screen at age 12)** and **offer prophylactic colectomy**
102
describe the complication of the condition in the image
* diameter of left colon is smaller → napkin ring constriction → intestinal obstruction
103
explain the variants of the condition seen in the image
* **Gardner's syndrome** = polyposis of colon + extra-intestinal signs and symptoms
* multiple osteomas (especially mandible)
* skin cysts: epidermal cysts, fibromas, lipomas
* CT growth → desmoid tumors
* hypertrophy of retinal pigment
* supernumerary teeth
* papillary thyroid cancer
* **Turcot's syndrome** = FAP + CNS gliomas and medulloblastomas
104
describe the image
**carcinoid tumor**
carcinoid tumors often form a **submucosal nodule composed of tumor cells embedded in dense fibrous tissue**
105
describe the image
**carcinoid tumor**
bland cytology that typifies neuroendocrine tumors
the chromatin texture, with fine and coarse clumps, frequently assumes a **"salt-and-pepper" pattern**
106
the condition in the image is most commonly found in the ____ (layer) of the ____ and \_\_\_\_
the condition in the image is most commonly found in the **submucosa** of the **SI** and **appendix**
107
describe the origin of the condition seen in the image
origin **= enterochromaffin/Kulchitsky/neuroendocrine cells**
108
describe type I of the condition seen in the image
* type I: gastric atrophy and achlorydia
* **autoimmune chronic gastritis**
* hypergastrinemia → ECL cell hyperplasia
* may be multiple, but usually benign
109
describe type II of the condition seen in the image
* type II: gastrinoma/Zollinger-Ellison syndrome (gastrinoma of the pancreas)
* **usually in MEN-1 syndrome**
* ZE: hypergastrinemia → increased acid but no negative feedback → multiple, large duodenal ulcers
110
describe type III of the condition seen in the image
* type III: sporadic
* malignant → very aggressive, sporadic
111
describe the the condition in the image when it affects the small intestine and appendix
* small, occult primary tumors can metastasize widely leading to carcinoid syndrome **if serotonin bypasses the liver**, avoiding degradation to 5-HIAA by MAO
* serotonin leaks out hepatic tributaries and can lead to **carcinoid heart disease → R-sided valvular fibrosis (tricuspid** **regurg****. &****pulm****. valve stenosis)**
* **L-side of heart unaffected** since lungs contain MAO and COMT
112
describe what is seen on biopsy of the condition seen in the image
* biopsy: uniform cells with **stippled oval nuclei** & **salt and pepper** appearance in the **submucosa**
113
\_\_\_\_ is increased in the urine in the condition seen in the image
**5-HIAA**
114
list the tumor markers for the condition seen in the image
synaptophysin, chromogranin, CD56 (origin of cells)
115
describe the image
**cirrhosis**
thick bands of collagen separate rounded cirrhotic nodules
116
describe the hepatic diseases that can lead to the condition seen in the image
* etiology: irreversible diffuse fibrosis of the liver with formation of regenerative nodules
* hepatic diseases
* viral hepatitis → **B & C**
* auto-immune hepatitis → **ANA, anti-smooth muscle Ab**
* steatohepatitis: alcohol
* see Mallory bodies (intermediate filaments as eosinophilic cytoplasmic inclusions)
117
describe the biliary diseases that can lead to the condition seen in the image
* biliary diseases
* **primary biliary cirrhosis = anti-mt Ab**
* **primary sclerosing cholangitis** = causes strictures; beaded appearance; p-ANCA positive
* can be caused by UC
118
describe the **metabolic** diseases that can lead to the condition seen in the image
* metabolic diseases:
* **hemochromatosis**: increased iron; increased ferritin; decreased TIBC (total iron binding capacity)
* diabetes mellitus
* increased skin pigmentation
* cardiomyopathy
* **Wilson's disease**: decreased serum ceruloplasmin, increased hepatic Cu, increased urinary Cu excretion and KF rings
* **A1AT deficiency** caused by PiZZ
119
describe the pathogenesis of the condition in the image
* activate ito/stellate cells (store vit. A) to deposit type III collagen and type I collagen in space of Disse
* **loss of fenestrations** in endothelial cells → impaired secretion of proteins (albumin, clotting factors)
* new vascular channels in **fibrous septae** → shunting of blood
* **obstruction of biliary channels** → jaundice
120
describe the presentation of the condition seen in the image
* estrogen metabolism impairment:
* palmar erythema
* spider nevi/angioma
* testicular atrophy
* gynecomastia
* finger clubbing, jaundice, leukonychia (white nails due to low albumin), Dupuytren contracture, xanthomas
121
describe the investigations of the condition seen in the image
* biopsy with trichrome stain (stains collagen blue)
* increased AST, increased bilirubin, decreased clotting factors, decreased albumin
122
list complications of the condition seen in the image
* hepatic failure
* synthetic function abnormalities (albumin, clotting factors)
* portal HTN → ascites, varices, splenomegaly, hepatic encephalopathy
* hepatic encephalopathy because of defective urea cycle → build-up of ammonia in the brain
* asterixis (hand flapping tremor) due to increased ammonia
* HCC
* increased bleeding
* loss of coagulation cascade proteins and vit. K
123
describe the etiology of the condition seen in the image
* global distribution strongly related to the prevalence of HBV
* cirrhosis of any etiology
* most common = alcohol and HBV
* Aspergillus flavus (aflatoxin → cirrhosis)
* A1AT def.
* NAFLD/NASH
124
describe the histological morphology of the condition seen in the image
* histology:
* trabecular, sinusoidal, or pseudoacinar pattern
* **hallmark: bile production by tumor cells** → increased bile output → seen as cytoplasmic inclusion (increased globules of bile in cytoplasm)
125
describe the investigations of the condition seen in the image
* increased AFP as well as a sudden increase in ALP and GGT
126
describe the complications of the condition seen in the image
* spreads to:
1. lungs
2. bone (via blood)
* increased risk of Budd-Chiari
127
describe the trabecular type of the condition seen in the image
**nests and cords of hepatocytes**
128
describe the fibrolamellar type of the condition seen in the image
* fibrolamellar type
* **no association with viral hepatitis or cirrhosis = idiopathic**
* **well differentiated polygonal cells in cords or nests separated by fibrous septa**
* best prognosis of all types
129
describe the risk factors for the condition seen in the image
* RF:
* 6 F's: female, fat, forty, fertile (oral contraceptives), fair skin, family history
* reduced bile salts due to poor absorption or underproduction:
* Crohns = inflammation of ileum → malabsorption of bile salts
* cirrhosis = decreased bile salt synthesis
130
describe the etiology of the condition seen in the image
* etiology:
* gallbladder stasis
* inborn error of bile salt metabolism
* hyperlipidemia syndromes
131
the condition in the image is ___ and is therefore not seen on \_\_\_\_
the condition in the image is **radiolucent** and is therefore not seen on **x-ray**
132
describe the etiology of the condition seen in the image
**pigment stones = bilirubin calcium salts**
* etiology:
* **chronic hemolytic anemia**
* HS, B-thalassemia, SCD
* biliary infections → bacteria have glucorinidases that convert bilirubin → unconjugated
* liver cirrhosis or chronic liver disease → lack of conjugation of bilirubin
133
describe the pathogenesis of the condition seen in the image
* pathogenesis:
* bile is supersaturated with cholesterol → **bile hypomotility** promotes nucleation (precipitation of cholesterol from bile into vesicles)
* **mucous hypersecretion traps the crystal** permitting aggregation into stones → acts like glue
134
list complications of the condition seen in the image
* complications:
* empyema
* acute cholecystitis
* **chronic cholecystitis**
* porcelain gallbladder = extensive dystrophic calcification → gallbladder cancer
* **gallstone ileus** → fistula with small bowel → gallstone obstructs the ileocecal valve
* acute pancreatitis → obstruction of the common bile duct (biliary tree)
* acute cholangitis → obstruction of the common bile duct (biliary tree)
* **gallbladder adenocarcinoma**
* most common predisposing factor
135
describe the risk factors for the condition seen in the image
all RFs for cholangiocarcinoma cause chronic inflammation and cholestasis
* primary sclerosing cholangitis
* parasitic infections
* Clonorchis sinensis, Opisthorchis viverini
* cystic dilatations of biliary system → Caroli's disease
* gallstones
* chemicals
* benidene
* nitrosamines
136
describe the presentation of the condition seen in the image
* presentation:
* patients typically have non-cirrhotic livers & present with obstructive symptoms
* malaise, weight loss, jaundice
* ascending cholangitis
* **Charcot's triad:** jaundice, fever, chills
* **Raynaud pentad**: Charcot triad + hypothension and mental status changes
* poor prognosis
* more likely to spread beyond liver than HCC
137
primary biliary cholangitis is a non-\_\_\_\_, ____ destruction of \_\_\_\_-sized bile ducts
primary biliary cholangitis is a non-**suppurative**, **granulomatous** destruction of **medium**-sized bile ducts
138
describe the pathogenesis of PBC and name a differential
chronic **non-suppurative (non-caseating granulomatous) granulomatous inflammation** caused by autoimmune **CD4 T cell-mediated** destruction of the ***_intrahepatic_* bile ducts**
PSC = intrahepatic AND extrahepatic bile ducts
139
describe the presentation of PBC
* pruritus (itching) caused by bile salts
* increased bile acids deposited in skin
* steatorrhea
* liver tries to compensate by making cholesterol → **xanthomas**, xanthelasma
140
PBC is associated with anti-\_\_\_\_\_
PBC is associated with anti-**mitochondrial antibodies**
141
list complications of PBC
* **ductopenia**
* malabsorption of fat and fat-soluble vitamins
* **hypercholesterolemia → xanthomas**
142
describe the hallmarks of primary sclerosing cholangitis
* inflammation, fibrosis, strictures and dilatations of intra- AND extrahepatic ducts
* associated with UC and is p-ANCA positive
143
on ERCP of suspected PSC, there is ___ of the biliary tree
on ERCP of suspected PSC, there is **beading** of the biliary tree
144
describe the histology in PSC
periductal fibrosis → onion-skin fibrosis → obliterating of bile ducts
145
describe the etiology of the condition seen in the image
* MCC = **alcohol and gallstones**
* I GET SMASHED
* Idiopathic, Gallstones, Ethanol, Trauma, Steroids, Mumps/Malignancy, Autoimmune, Scorpion sting, Hypercalcemia, ERCP, Drugs (diuretics, estrogen)
146
describe the morphology of the condition seen in the image
* focal **fat necrosis** in pancreas and peripancreatic tissue and abdominal cavity
* calcium deposition in these areas → appear radiopaque on radiographs
* pancreatic injury → release of amylase & lipase → breaks down lipids to release FAs which combine with Ca2+ → saponification
* **liquefactive necrosis** of the exocrine pancreas
147
describe the pathogenesis of the condition seen in the image
* acinar cell injury → enzymatic auto-digestion of pancreas → trypsin → constant activation of all other enzymes
148
describe the investigations of the condition seen in the image
* _24-48 hours_: measure **amylase** (highly sensitive, not specific)
* _72-96 hours_: measure **lipase** (specific, not sensitive)
149
describe the complications of the condition seen in the image
* **ARDS**
* hemolysis with peripheral vascular collapse
* hypovolemic shock → **acute tubular necrosis** → acute renal failure
* secondary infection by bacteria → sepsis → DIC
* hypocalcemia → tetany and heart murmurs
* **pancreatic abscess** = infection of pancreatic pseudocyst most commonly by intestinal bacterial (like E. coli)
150
describe the image
**acute pancreatitis**
microscopy shows a region of fat necrosis (right) and focal pancreatic parenchymal necrosis (center)
151
in the condition seen in the image, there is ___ of parenchyma due to repeated \_\_\_\_
in the condition seen in the image, there is **fibrosis** of parenchyma due to repeated **bouts of acute pancreatitis**
152
describe the etiology of the condition seen in the image
* adults: **most common cause = chronic alcoholism**
* children: **CF**, pancreatic divisum, mumps
153
describe the presentation of the condition seen in the image
* repeated attacks or persistence of moderately severe **abdominal pain and back pain**
* possible progression to **pancreatic insufficiency** and **diabetes**
* malabsorption (b/c no amylase or lipase) & steatorrhea & jaundice
* malabsorption corrected by pancreatic enzyme supplements
* **intraluminal hydrolysis of fats, proteins, carbs by enzymes is defective** → malabsorption
154
describe what would be seen on CT/x-ray of the condition in the image
**fibrotic pancreas** w/ **dystrophic calcification** of pancreas
155
the most important prognostic indicator of the condition seen in the image is \_\_\_\_
the most important prognostic indicator of the condition seen in the image is **hypocalcemia due to malabsorption of vit. D** → ***cardiac arrhythmias***
156
describe the complications of the condition seen in the image
* **pancreatic pseudocyst**: fluid-filled cavity NOT lined by epithelium; fibrous scar
* **pancreatic insufficiency**: diabetes (if islets are damaged), fat malabsorption, steatorrhea, fat-soluble vit. deficiencies
* **pancreatic carcinoma** → esp. with alcohol
157
the most common location for the condition seen in the image is \_\_\_\_
what can this affect?
the most common location for the condition seen in the image is **the head of the pancreas**
* adenocarcinoma of the head of the pancreas/ampulla **obstructs bile flow**
* jaundice → increase ALP, light colored stools, palpable gallbladder (Courvoisier sign)
158
list the predisposing factors for the condition seen in the image
1. smoking (most common)
* familial relapsing chronic pancreatitis
* KRAS mutation
159
describe the presentation of the condition seen in the image
* **majority** **are silent until late**
* therefore classically called "**painless jaundice**"
* the first symptom is pain due to invasion of the posterior abdominal wall and nerves (perineural)
160
in the condition seen in the image, the first symptom is ____ due to \_\_\_
the first symptom is **pain** due to **invasion** **of the posterior abdominal wall and nerves (perineural)**
161
describe the histology of the condition seen in the image
* majority ductal type adenocarcinomas
* dense stromal fibrosis → **desmoplasia**
* propensity for perineural invasion
162
there is no single specific marker for the condition seen in the image, but ___ is raised sometimes
there is no single specific marker for the condition seen in the image, but **CA 19-9** is raised sometimes
163
describe the complications of the condition seen in the image
* Trousseau's sign: recurrent migratory thrombophlebitis
* due to release of platelet factors and procoagulants from tumor
* renal vein thrombosis
* diabetes (if islets are destroyed)
* perineural invasion
* metastasis to the mesenteric lymph nodes and liver
