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Panctreaticobiliary and Liver Flashcards

1
Q

What cyst is this and how to treat?

A

Type I.

Resection with Whipple.

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2
Q

Mutation in JAG1 and NOTCH?

A

Alagille syndrome

Lack of bile ducts. Cholestatic jaundice.

Triangular face

Butterfly vertebrae

Heart murmur

20-30% liver transplant

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3
Q

Mutation f Alagille syndrome?

A

AD

JAG1 and NOTCH

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4
Q

Liver histo with concentric periportal fibrosis

A

PSC

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5
Q

Liver histo with rosettes, lymphocytes, plasma cells, interface hepatitis

A

AIH

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6
Q

Liver histo with PAS positive, diastase resistent granules

A

A1AT

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7
Q

Defect of ATP7B

A

Wilsons Disease

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8
Q

Wilsons gene defect?

A

ATP7B

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9
Q

Defect ATP8B1

A

progressive familial intrahepatic cholestasis

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10
Q

Liver bx with mixed portal tract inflammation with portal venule endothelialitis

A

Acute cellular rejection

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11
Q

Acute cellular rejection liver bx results

A

mixed portal tract inflammation with portal venule endothelialitis

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12
Q

hereditary pancreatitis in an autosomal dominant pattern due to premature activation of trypsin

A

PRSS1

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13
Q

nherited in an autosomal recessive pattern and causes pancreatitis through lowering the levels of trypsin inhibitor

A

SPINK1

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14
Q

failure of alkalization of the acinar cells with resulting retention of zymogens in the pancreatic duct and digestion of pancreatic tissue

A

CFTR

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15
Q

Which choledococele requires endoscopic sphincterotomy?

What other abnormality is assoiciated with and what should you recommend in these patients?

A

Type III

APBJ

Needs cholecystectomy

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16
Q

Which choledococyst has high malignancy potential?

A

I and IV

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17
Q

FAP patient with hx colectomy presenting witha cute pancreatitis?

A

Think NSAID sulindac

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18
Q

Seizures and bipolar with acute pancreatitis?

A

Think valproic acid

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19
Q

Bilateral gynecomastea and acute pancreatitis?

A

Think marijuana

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20
Q

What skin lesion is found in glucagonoma?

A

Necrolytic migratory erythema

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21
Q

Pt with obstruction, what is this a sign of?

A

Annular pancreas

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22
Q

What liver injury does valproic acid cause on histology?

What other things cause this pattern?

A

Microvescicular steatosis of liver

Acute fatty liver of pregnancy

Tetracycline

MTX

Amiodarone

Tamoxifen

Reyes syndrome

Mitichondrial disorders

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23
Q

Chronic diarrhea with IDA and recurrent pancreatitis?

Why cause this?

A

Celiac disease

Papillary outflow obstruction

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24
Q

What size important to remember sporatic ampullary adenoma treatment?

A

<2-3cm and no lymph node involement can resect endoscopically

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25
When not to use LR for pancreatitis?
Hypercalciumeia pancreatitis
26
Indication to perform ERCP in gallstone pancreatitis?
Cholangitis
27
When is SOD manometry abnormal pressure?
\>40 mmHg
28
If patient has divism and recurrent pancreatitis what should you do?
ERCP and minor papillotomy
29
Which pancreatic cystic neoplasm presents in woman aged 20-30? Worried for malignancy?
Solid pseudopapillary neoplasm Moderate to high malignancy potential range Bloody fluid with cytology showing finger-like projections (pseudopapillae)
30
Pancreatic cyst with aspiration showing elevated CEA and amylase? 3 risk factors for resection?
IPMN \>3, dilated PD, nodule/solid components
31
What type of cells of pancreatic cyst are cuboidal?
Serous cystadenoma
32
What type of cells from pancretic cyst and culumnar?
IPMN
33
Cyst in female with low CEA and low amalyse? Management?
Serous cystadenoma No surveilance needed!
34
Difference in IPMN vs mucinous cystadenoma?
IPMN males+femals, MCA females only IPMN more likely head MCA more likely body/tail
35
Difference between AIP type I and type II? Cell type differences? Which one is associated with IgG4 elevation? Which one associated with IBD?
Type I IgG4 Type I Lymphocytoplastic Type II granulocytic and epithelial IBD with type II
36
Why do celiac patients have chronic pancreatitis?
Effacement of duodenal cells leads to loss of CCK and secretic which stimulate enzyme secretion and production
37
Unresectable pancreatic mass with biliary obstuction?
EUS hepaticogastrostomy vs SEMS if \>3 months to live If \<3 months, plastic is okay
38
Malignent gastroduodenal obstruction? \>6 months? \< 6 months?
\>6 months, GJ \< 6 months, enteral stent Biliary decompression first if hepatic obstruction present
39
What kind of gallstone? Sickle cell, HHS
Black pigment (bilirubin)
40
What kind of gallstone? Cirrhosis
Black pigment (bilirubin)
41
What kind of gallstone? Recurrent pyogenic cholangitis
Brown pigment stones
42
What kind of gallstone? TPN
Cholesterol
43
What kind of gallstone? Pregnent, OCP, female?
Cholesterol
44
What kind of gallstone? Neufibromatosis - diarrhea, diabetes, biliary dyskenesia
Somatostatinoma with cholesterol stone
45
What helps cholestatsis in cystic fibrosis?
UDCA
46
What kind of gallstone? Ceftriaxone
Crystal calcium ceftriaxone stones
47
Secretory diarrhea, dermatitis (necrolytic migratory erythema), diabetes
glucagonoma Dx: glucagon \>500 Tx: local surgical resection mets octreotide
48
Mass in tail of pancreas with watery diarrhea with hypokalemia and hypochloremia?
VIPoma \>75
49
MCC mets to pancreas (2)?
RCC and melenoma
50
Stool gap and meaning 290 - 2(Na+K)
\<50 secretory \>50 osmotic (carb mal, laxatives)
51
How to differentiate hypoglyemia from insulinoma vs insulin administration? What syndrome is it related to?
C-peptide is HIGH with insulinoma MEN1 (AD; chrome 11 melin) Hyperprolactonemia (gynecomastia), Hyperparathyroidism (elevated calcium),
52
What is related to von Hippel Lindau?
AD PNETs Pheos Hemangioblastomas RCC
53
What is related to Nuerofibromatosis 1?
Somatostatinoma (mild diabetes mellitus, steatorrhoea and gallstones, and achlorhydria, basic stomach contents) Pheos Cafe au lait Neurofibromas
54
What is related to tuberous sclerosis?
**Insulinoma** Angiolipoma Renal cysts Rhabdomyoma
55
Classic XR finding with annular pancreas?
"Double bubble" with dilation of duodenum and stomach
56
WHen would Peustow procedure be benificial for chronic pancreatitis patient?
If PD duct is dilated \>6mm
57
Liver graft failure in patients with HCV not controlled?
Fibrosing cholestatic hepatitis
58
RUQ pneumatosis think...
Enphesematous cholecystitis Tx: Abx and perc chole (same as acalculous cholecystitis)
59
Asian woman with round nodules in gallbadder and cholecysttis
Xanthogranulomatous cholecytits
60
All patients with porcelain gallbladder get what
Cholecystectomy
61
What is next step in transect bile duct?
Percutaneous transhepatic cholangiogram then surgical or endoscopic HJ
62
Difference between PSC and HIV cholangiopathy on MRCP?
AIDS cholangiopathy has ampullary stenosis
63
Air with pain in retroperitoneal space?
Duodenal perforation (can be from ERCP) Tx: often can be treated conservatively
64
What injury is common when "shortening the scope" on ERCP?
Lateral wall perforation which needs to be treated surgically
65
Patient crashing with hypotension and hypoxia during ERCP?
Air embolism Put in Trendelenburg and call emergency team
66
Oily skin lesions + weight loss + pantreatitis
Acinar cell carcinoma of pancreas
67
ALT:LDH level is what important number?
\<1.5 is more likely shock or DILI
68
Giant hemangioma is associated with what?
DIC Kasabach-Merritt syndrome
69
What is Kasaback Merritt syndrome?
DIC and giant hemangioma
70
Young women with fever, elevated LFTs and RUQ pain with normal US? Think of...
Fitz-Hugh-Curtis and Chlamydia PID infection - get pelvic US and IV ceftriaxone
71
Injury zone in Budd-Chiari?
Zone 3 portal lobules Compensatory caudate lobe hypertrophy because of accessory hepatic vein drainage directly into IVC
72
Acute ascites and abdominal pain in pregnent woman?
Think Budd-Chiari
73
Diagnosis for Pa02 and A-a gradient in HPS? and what level precludes transplant? What about contrast TTE results? What about TcMaa level? an what level precludes transplant?
Pa02 \<70 or Aa gradient \>20 No transplant \<50 Contrast in cardiac cycle \>3 = intrapulm shunt \<3 = intracardiac shunt TcMAA \>5% = intrapulm shunt \>40% precludes transplant
74
What is routine screen on pregnent patient with cirrhosis? What size for surgery? What else should you screen for?
Doppler US to look for splenic artery aneurysm \>2cm Varices
75
What type of delivery is best for cirrhotic patient with varices?
Vaginal
76
2 risks for HCV vertical transmission?
Fetal scalp monitoring Prolonged laboring (membrane rupture)
77
Can you breastfeed in HepC? What about HepB?
Yes to both
78
Best HepB for pregnancy treatment?
tenofovir Give if \>200,000 at 28 weeks
79
What HBV genotype responds best to PEG and which one worst?
A best (african american) D is worst (eastern european and asian)
80
Which HBV genotype has worst prognosis?
C B is better, C is crummy in Asians
81
What test is validated to detect subacute encephalopathy?
Number connection test \>30 is +
82
What does Rifaximin due for encephalopathy?
Reduce hospital admissions
83
Who can you not give PEG interferon to?
cirrhotics
84
Genetic defect in Wilsons? Classic lab finding in LFTs?
ATP 7B on chrome 13 ALP \<40
85
Wilsons diagnosis? Treatment
Ceroloplasim \<20 24 hr collection \>100 Hepatic concentration \>250 mcg/g Tx: Trientine or penicillamine. Take Zinc. Liver transplant cures. Neuropsych symptoms persist. Check well water copper level
86
How to diagnose portopulmoary pressure? When cannot do transplant?
RHC showing PA pressure \>25 \>50
87
Independent markers of varices presence?
Portal vein diameter \>13 Plt \<88 Elevated INR Splenomegaly
88
What disease rpesents with increased wedge and free pressure?
RHF
89
Liver bx with phospholipd layden lysomal lamellar bodies
Amiodarine
90
What abx has AIH type pattern?
Nitrofurantoin
91
Which NSAID causes liver failure?
Diclofenac
92
Other drugs with AIH injury?
Diclofenac Nitrofuretin Minocycline Phenytoin PPU
93
94
What can anabolic steroids and AIDS cause?
Peliosis - multiple blood filled cystic spaces throughout the liver Elevated ALP/GGT but bilirubin normal AIDS Bartonella infection (tx erythromycin 2g daily)
95
Lipid-filled stellate cells
VitA toxicity
96
What does phlebotomy NOT help with hemochromatosis?
97
Two side main side effects of Riboviron?
Hemolytic anemia and rash
98
Difference between fibrolamellar HCC and FNH?
Both have central scar Calicifications with HCC C in HCC
99
ALT above what in pancreatitis is suggestive of gallstone panc?
150
100
Complications of PBC?
Osteoporosis HLD Hypothyroidism Nodular regeneative hyperplasia causing elevated portal pressures (screen for varices if platelets are dropping)
101
Wilsons fact: what can happen after starting treatment?
Nuero symptoms and liver tests may initially worsen and improve after 6 months Screen first degree relatives
102
What are some signs of GOOD prognosis in acute liver failure?
Hypophosphetemia Elevated FV Leiden Elevated AFP
103
Persistent unexplained hypotension in acute liver failure?
Think adrenal insufficiency
104
ICP goal in acute liver failure?
\<20
105
Fibrous-obliteation of the bile duct with concentric connective tissue replacement
PSC
106
New diagnosis of PCS what lab test should be checked?
IgG4 to rul eout IgG4 associated cholangitis BECAUSE IT RESPONDS TO STEROIDS
107
Liver parasite association: Sheepherder
Fascioliasis Echinococcosis
108
Liver parasite association: Strong male predominance
Amebiasis
109
Liver parasite association: Serum sickness
Schistisimiasis
110
Liver parasite association: Solitary lesion in R lobe
Echinococcosis Amebiasis
111
Liver parasite association: Transverse myelitits
Schistosomiasis
112
Liver parasite association: Epilepsy
Scistosomiasis
113
Liver parasite association: Undercooked fish
Clonorchiasis
114
Liver parasite association: Gallstones and biliary ductal fibrosis
Clonorchiasis
115
Liver parasite association: Tortuous tracts on CT
Fasciolisasis
116
Coffee protects you from what?
HCC, elevated AST/ALT and cirrhosis
117
Dude eats mushroom and goes into liver failure. Whats the treatment?
Amanita PCN G and silymarin (milk thistle extract)
118
Vitamins: Problems balancing while ambulating
Vitamin E
119
Vitamins: Night blindness
Vitamin A
120
Vitamins: Persistent bleeding after polypectomy
VitK deficiency
121
How are AMA+/AIH and AMA negative PBC/AIH (autoimmune cholangiopathy) treated differently?
Add UDCA to AMA NEGATIVE PBC
122
AD polycystic kidney disease has what colonic complication?
Diverticulitis
123
MCC skin cancer after liver transplant?
SCC | (normally it BCC in normal people)
124
Three causes of high SAAG high protein?
Budd-Chiari Hypothyroidism (myedema) Cardiac
125
Low AlkPhos + Macrocytosis + Early graying
Pernicious anemia | (anti-paritial cell antibody)
126
Crohn's and dry skin rash with low alk phos
Zinc deficiency
127
Subcapsular liver lesion in female?
Hepatic adenoma Resect if over 5cm or if symptomatic or if going to get pregnent
128
3 liver things with pregnancy that are normal?
Small varices Palmar erythema/spider angioma Elevated ALP (from placenta)
129
Can draw on skin + flushing with ETOH
mastocytosis
130
Which Wilsons treatment is less tolerable but more potent?
D-Penicillimase Can get early severe hypersensitivity reaction
131
Nutmeg liver
Congestive hepatopathy or Budd-Chiari
132
Ground glass hepatocytes
HepB infection
133
How to tell primary hemochromatosis vs iron overload on liver biopsy?
HHE: iron in hepatocytes Secondary: iron in Kupffer cells (eating up the iron)
134
Cirrhosis with new TR and Loud P2 click What pressures can you trasnplant?
think porto-pulmonary hypertension Dx \>25 with wedge \<15 transplant between 25-35mmHg; def not \>50
135
Blood risk of intrahepatic cholestasis of pregnancy? How to treat?
Vit K deficiency from steatorrhea from cholestasis Tx: UDCA
136
Why does acute fatty liver in preganncy occur?
Fetus homozygoud for LCHAD mutation and mother is heterozygous and can't break down long chain fatty acids (intramitochondrial fatty acid oxidation) MONITER fetus for hypoglycemia
137
When should you surgically remove echinococcosis cyst?
10cm
138
Risky 6-MMP level for risk of hepatotoxicity
5700
139
Heterogenous pancreatic mass: Tail and body of young 30s Asian or AA woman?
Solid and Papillary Epithelial Neoplasm Resect
140
Pancreatic cyst: Central fibrotic scar
Serous cystadenoma
141
Pancreatic cyst: Middle aged woman: Ovarian-like stroma w/estrogen and progesterone receptors
Biliary cystadenoma Tx: Surgical resection
142
What cell is glucagonoma released?
alpha-2 cells
143
Gallbladder get vascular supply from what two vessels?
R hepatic artery GDA collaterals
144
LDL uptake is stimulate by what two things?
Cholesterol diet Estrogen
145
4 drugs that increase cholesterol gallstone risk?
Clofibrate Ceftriaxone Octreotide Estrogen
146
What type of gallstone common in Crohn's?
Black pigment stone | (calcium bilirubinate)
147
Which PFIC has elevated GGT?
PFIC type III ABC B4
148
Defect ATP 7B?
Wilsons
149
Defect in ATP8 B1?
PFIC I
150
Defect in ACB11?
Type II PFIC
151
Fatty liver gene defect?
PNPLA3
152
Difference between Crigler-Najjar type I and II and how are they treated?
I: complete - liver transplant II: partial - phenobarbitol
153
Liver biopsy with lipid laden macrophages?
Gauchers disease Increased HCC risk, cirrhosis rare Hepatomegaly with bone pain and fractures
154
Why is iron overload in HH?
Defect in hepcidin (which is low) Normal if mutation is in ferroportin gene
155
Severe SOS post BMT?
Defibrotide
156
What kind of gallstone after ileum resection but colon intact? How to treat?
Calcium oxylate stone Decrease fat and increase calcium
157
One drug to treat bulemia?
Fluoxetine
158
Drug for binge eating disorder?
Vyanse amphetamine
159
Chylous ascites and primary lymphangiectasa are treated the same, how?
Low fat high protein diet Medium chain fatty acids
160
Juvinille polyposis syndrome mutation? Associated with what?
SMAD4 Hemorrhagic telectangias
161
Dermatomyositis (weakness) + agancathiosis nigrans + mirgratory thrombophlebitis = what sign of?
Gastric cancer
162
Peuta Jager surveilance?
Annual Pap, Breast, Pelvic US MRCP/EUS q 2 years at 35 Colon EGD SB imaging q 3 years
163
Lynch surveilance
Screen annually at 20-25 yo Endometrial anual screen @30 EGD @ 30 Urinalysis @ 30
164
FAP surveilance
APC mutation as infant: HCC screen til 5 years old hemangioblastoma Sigmoid at 10 Side Viewing @ 30
165
What is Croflelener used for?
Diarrhea in HIV patients
166
Mechanism of bile acid diarrhea?
Decreased **fibroblast growth factor** 19 (FGF19) secretion by terminal ileum enterocytes can lead to dysfunctional feedback inhibition of CYP7A1, the rate limiting enzyme in bile acid synthesis in the liver. The result is an expanded bile acid pool that allows increased amounts of bile acids to reach the colon.
167
Which bacteria chronic carrier in gallbladder increases risk of cancer?
Salmonella typhi Abx don't work.
168
Adiponectin levels low?
Low levels of circulating adiponectin, accompanied by insulin resistance, characterize metabolic syndrome and non-alco - holic fatty liver disease
169
Tell me about hepcidin?
Secreted by liver to downregulare intestinal iron absorption Defective in HH (low blood levels)
170
Tell me about Adiponutrin (PNPLA3)?
Associated with increaed liver fat deposit
171
Tell me about bone morphogenetic protein 1?
up-regulation of hepatic hepcidin production and control of intestinal iron absorption. Hemojuvelin is a hepatic co-receptor for this peptide.
172
Tell me about Ferroportin?
Transporter in intestine to uptake iron. Downregulated by hepcidin.
173
Target of AMA?
lipoic acid moiety of the pyruvate dehydrogenase complex in **bile ductular epithelial cells**
174
Taget of AMLK?
hepatic cyto-chrome **P450** 2E6
175
Who are candidate for early TIPS?
Candidates for early (preemptive) TIPS are patients with variceal hemorrhage who are Child C (score 10-13) or Child B (score 7-9) with active hemorrhage at endoscopy.
176
What medications should be avoided for constipation in pregnancy?
Mineral oil and castor oil (also avoid in elderly patients with aspiration)
177
Acute fatty liver of pregnancy mechanism?
The G1528C and E474Q mutations of the mitochondrial trifunctional protein (MTP) are the cause of deficiency in long chain 3-hydroxyacylCoA dehydrogenase (LCHAD), which is the most investigated fetal fatty acid oxidation defect that causes acute fatty liver of pregnancy (AFLP).
178
Patients require a colon in continuity to develop calcium oxalate kidney stones as a consequence of fat malabsorption.
ie IC resection
179
Anorexia nervosa stomach motility does what?
Decreases
180
Leptin in obesity?
Obesity thought to be leptin resistant state
181
When are obesity medications indicated?
Anti-obesity medications are appropriate for patients with BMI greater than or equals 27 kg/ m2 with obesity related complications, or BMI greater than or equal to 30 kg/m2
182
When is surgical weight loss indicated?
Surgical weight loss treatment is appropriate for patients with BMI greater than or equal to 35 kg/ m2 with obesity related complications or BMI greater than or equal to 40 kg/m2
183
When can you initiate anti-TNF therapy with latent TB?
Prefer to wai ttill after but emergently can use after 4 weeks
184
What two mutations do SSA have?
BRAF mutations and CpG island methylation.
185
Fundic gland polyps malignancy potential?
NONE EVEN WITH LOW GRADE DYSPLASIA Common in FAP
186
How can inappropriately stored food increase risk of HCC?
Aflatoxin B1 is a fungal toxin produced by certain Aspergillus species.
187
Cyclical vomting syndrome for children \<5 and \>5 years old?
\<5: cyproheptadine \>5: amitryptalene
188
Antiretroviral HBV therapy in ALF?
Does not change mortality outcome, this patient needs a liver transplant
189
Copper urine goal of WIlson with or without Zinc?
Urine copper: 200-500 WTH ZINC: 75
190
What GI lesions do Cowdens disease produce?
hamartomas and ganglioneuromas Also increase in breast cancer, thyroid cancer, endometrial cancer, renal cellcancer and melanoma Non-malignant features of Cowden’s syndrome include glycogenic acanthosis of the esophagus (as in the endoscopy picture above), as well as macrocephaly and skin lesions called trichilemmomas.
191
Pancreatic cyst with columnar epithelium surrounded by ovarian-type stroma
Mucinous cystic neoplasms Once removed, no surveilance needed. Surveilance is needed for IPMN
192
Gastric hamartomas in JPS? Mutation? How different from Cronkhite-Canada syndrome?
glandular dilation with an inflammatory infiltrate BMPR1A and SMAD4 Also at risk for hereditary hemorrhagic telangiectasias Cronkhite-Canada syndromehas hair loss and nail growth problems and is non-heriditary "Walter Kronkite had bad hair and brittle nails"
193
What features of malignent polyp require surgery?
High risk features a polyp with cancer invading through the muscularis mucosa and into the submucosa. Unfavorable histologic features of malignant polyps include: poorly differentiated, presence of angiolymphatic invasion and/or positive margin of resection (defined as tumor less than one to two mm from the transected margin or tumor cells present at the margin).
194
What type of polyps in the stomach do have increase risk of malignancy compared to fundic gland polyps?
Hyperplastic \>1 cm and pedunculated have higher risks Endoscopic removal Adneomas in stomach should also be removed and follow p should occur in 1 year
195
Eliquis (abixiban) holding for CCl \>60 \>30 \<30
1-2 days 3 days 4 days
196
Timing of holding Xerelto (rivaroxiban) CCl \>90 \>60 \>30 \<30
1 2 3 4
197
Numbness and ataxia think of what dificiency?
Zinc
198
What defieincy causes neutrapenia?
Copper
199
Liraglutinide contraindications?
GLP-1 personal or family history of medullary thyroid carcinoma or MENII Pancreatitis risk
200
Lorcaserin (Belvique) contraindications?
Valolopathy Attension or memory distrubance
201
Phentiramine/ topiramate can cause what side effect?
Kidney stones
202

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