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Pancreas function Flashcards

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1
Q

List clinical sx and labs suggestive of EPI

A

Symptoms: FTT, weight loss, steatorrhea..etc

Labs:
anemia
low alb
prolonged PT
HypoK, HypoCa, HypoMg
FSV deficiency
low level TG, cholesterol

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2
Q

How can you test for EPI?

A

Indirect test:
1. Feccal elastase-1
2. stool trypsin/chemotryspin
3. serum immuno-reactive trypsinogen (tryspin precursor in serum)
4. 72 hr fecal fat collection (GOLD STD)
5. CO2 breath test

Direct:
1. Dreiling tube pancreatic function test
(IV CCK/Secretin followed by duodenal collection of PA fluids)
2. Endoscopic pancreatic function test
3. Secretin stimulated MRCP

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3
Q

List inherited disorders associated with EPI

A
  1. CF
  2. Schwachman-Diamond syndrome
  3. Pearson bone marrow-pancrease syndrome
  4. Johanson Blizzard syndrome
  5. Isolated pancreatic enzyme deficiency
  6. Pancreatic agenesis/hypoplasia
  7. Jeune syndrome
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4
Q

what is PA divisum?

A

Failure of the ventral and dorsal PA ducts–> two separate systems

The dorsal PA drains into minor papilla

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5
Q

Symptoms of PA divisum?

A

Acute PA
Chronic PA
Papillary inflammation –>stricture–>stenosis

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6
Q

why do you get papillary inflammation in PA divisum?

A

large volume of PA secretions/fluids pass through small papilla –> allows PA enzymes activation –>inflammation

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7
Q

Dx & Rx of pancreatic divisum

A

CT/MRI, MRCP, ERCP

Endoscopic dilation of minor papilla + stenting

Endoscopic sphincterotomy

Surgical sphincterotomy or sphincteroplasty

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8
Q

what is ectopic PA?

A

PA tissues without with absent VASCULAR or CONTINUITY with pancreas

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9
Q

where can you find ectopic PA?

A

Stomach (pre-pyloric)
Meckle’s diverticulum
Colon

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10
Q

Sx of ectopic PA

A

asymptomatic
abdominal pain
dyspepsia
pyloric obstruction
GIB

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11
Q

Dx and Rx of ectopic PA

A

Dx: incidental, xray & EGD

Rx: monitoring
removal via snare
surgical resection

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12
Q

what is annular PA?

A

Anomaly which the head of the PA completely or partially encircles the 2nd part of the duodenum

how? incomplete rotation

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13
Q

Associations with annular PA?

A

T21
Congenital heart disease
Esophageal and duodenal atresia
Tracheoesophageal fistula
imperforate anus
Meckle’s diverticulum
polyhydroamnios in utero

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14
Q

Symptoms of annular PA

A

small bowel obstruction
feeding intolerance
bilious vomiting
abdominal distension
postprandial fullness
acute/chronic PA

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15
Q

Criteria for acute PA

A
  1. abdominal pain (epigastric region w/ or w/O radiation to the back)
  2. elevation in amylase and/or lipase 3x UNL
  3. radiologic evidence of PA (US, CT)
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16
Q

3 Physiological phases of PA

A
  1. triggering event (virus)
  2. acinar cell injury and activation of digestive enzymes
  3. cell injury –> inflammatory mediators –> local inflammation
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17
Q

severe acute pancreatitis

A
  1. Release of vasoactive substances–>third spacing of fluids
  2. Involves SIRS and multi-organ failure
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18
Q

why do we prefer serum lipase over amylase?

A

Amylase:
1. less specific, 33-45% in PA and rest is salivary
2. increase in 2-12hr and remains high 3-5 days
3. can increase in DKA, mumps, renal failure

Lipase:
1. more specific to PA
2. increase in 4-8hr and remains high 8-14 days

** Elevated LFTs and GGT suggest biliary obstruction

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19
Q

Risk factors for severe PA

A
  1. age < 7
  2. weight <23
  3. WBC >18,500
  4. LDH >2000
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20
Q

Predictors of severe disease within 48hr

A
  1. low Ca <8.3
  2. low alb <2.6
  3. high BUN >5
  4. high fluid replacement <75ml/kg
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21
Q

DDx systemic illness associated with PA

A
  1. HUS
  2. Systemic Lupus
  3. HSP
  4. Kawasaki disease
  5. IBD
  6. JRA
  7. CF
  8. Shock/hypo-perfusion
  9. SCD
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22
Q

DDx biliary disease associated with PA

Study These Flashcards
A
  1. gallstones
  2. choledocal malformations
  3. biliary sludge
23
Q

DDx medications associated with PA

Study These Flashcards
A
  1. VAP
  2. L-asparginase
  3. Azathioprine
  4. 6-MP
  5. Steroids
  6. Mesalamine
  7. metronidazole
  8. cytarabine
  9. Furosemide
24
Q

DDx anatomic associated with PA

Study These Flashcards
A

PA divisum
Annular PA
Obstruction: ampullary tumor, duodenal ulcer or cronh’s disease, mass or tumor

25
DDx infection associated with PA
mycoplasma Coxsackie virus Mumps virus Rubeola adenovirus varicella HSV CMV HIV Hep A/B Influenza A/B
26
DDx genetic mutations associated with PA
1. CFTR 2. PRSS1 (AD, inc risk of CA) 3. SPINK-1
27
DDx metabolic causes of PA
hyperCa hypertriglyceriemia malnutrition
28
DDx toxins associated with PA
Tylenol overdose heroin amphetamine organophosphates alcohol spider/scorpion venom
29
Management of acute PA
1. hydration 1.5-2x maintenance 2. pain control (parenteral narcotics) 3. Early feeding
30
Benefits of early feeding in acute PA
Decrease 1. risk of infection 2. surgical intervention 3. hospital stay
31
complications of pancreatitis
1. pseudocyst: >4wk with thin surrounding wall 2. acute peripancreatic fluid collection: <4wks from onset 3. local fat necrosis 4. necrotizing pancreatic abscess 5. abscess extension to nearby organs 6. systemic: sepsis, shock, coagulopathy, renal failure, electrolyte disturbance diabetes
32
what is PRSS-1
AD mutation of cationic trypsinogen proteolytic enzyme by acinar cells--> increase activation of trypsin --> auto-digestion recurrent PA increase risk of DM and PA adenocarcinoma
33
what is SPINK-1
AR Serine Protase Inhibitor Kazal -1 Pancreatic secretory trypsin inhibitor that prevents premature activation of trypsin Alone not enough to cause chronic PA This mutation acts as co-factor
34
CFTR and chronic pancreatitis
Thickened PA secretions --> ductular obstruction --> can't secrete PA fluids --> activated PA enzymes --> autodigestion
35
CTRC mutation
Chymotryspin C proteolytic enzyme responsible for degradation of trypsin Loss of function--> over activation of trypsin
36
DDx of chronic pancreatitis
1. genetic mutation (SPINK-1, PRSS-1, CTRC, CFTR) 2. Anatomic abnormalities: PA divisum, annular PA, choledocal mal., stones, sludge 3. Syndormes: SDS, JBS, PBPS 4. autoimmune PA 5. toxins/meds 6. trauma 7. sphincter of oddi dysfunction 8. strictures (ductal ca, surgery) 9. repeated recurrent acute PA
37
Autoimmune PA
Marker: IgG4 Histology: ductal and periductal lymphocytic infiltrates + plasma cells narrowing of PA duct PA enlargment repsonds to steroids
38
Criteria of chronic pancreatitis
characteristic imaging findings + one or more of: 1. Abdo pain of pancreatic origin 2. EPI (fecal elastase, fecal fat, dreiling tube, endoscopic PA function test) 3. Endocrine PA insufficiency (ogtt)
39
Characteristic imaging findings for chronic pancreatitis
Ductal: irregular contour (beading) of main PA duct, intraductal filling defects, calculi, stricture, dilation parenchymal: generalized or focal enlargment, cavities, calcification, irregular contour, heterogenous echotexture
40
Testing for patients with SPINK-1, PRSS-1, CFTR, CTRA?
1. MRCP or EUS/CT/ERCP 2. RUQ US searching for stones 3. serum Ca 4. Fasting lipids 5. sweat chloride
41
Management of chronic PA
1. Hydration 2. PRET + FSV 3. Pain control (1st tylenol and NSAIDs then opioids) 4. endoscopic for obstruction, sphincterotomy/stent 5. surgery
42
Clinical features of JBS
1. EPI 2. Nasal wing hypoplasia 3. Oligodontia (absence of teeth) Other: 1. Imperforate anus 2. Renal abnormalities 3. Genital malformation 4. congenital heart defects 5. IUGR 6. short stature 7. GH or pahhypopituitarisim 8. hypothyroidism 9. cognitive impairment 10. hearing impairment 11. Diabetes
43
How differentiate be JBS and SDS?
Skeletal abnormalities found in SDS
44
US of JBS
Normal size fatty pancreas
45
what is the pathophysiology of JBS
genetic mutation leading to near absence of acinar cells --> replaced by connective tissue and fatty infiltrates
46
Main features of SDS
1. EPI 2. Skeletal abnormalities (metaphyseal dystosis, short stature, thoracic cage, SCFE) 3. Bone marrow dysfunction (low Hb, PLT, WBC, pancytopenia, cyclic neutropenia) Other: 1. delayed dentition/carries/enamel 2. development: delayed speech 3. behavioural problem 4. growth is good with PERT, 50% on 3rd%ile 5. liver: micro or macro steatosis
47
differenced between CF & SDS
CF SDS Sweat CL: High N IR trypsinogen: N birth, low, birth low >1yr N>3 ye Histology: duct fibrosis N ducts fibrosis fatty ectasia infiltrates PA enzyme output Inc over output: depends time. on genotype No genotype dependance
48
US and histology of SDS
US: Normal sized fatty pancreas Histo: acinar duct absence or aplasia Intact duct architecture VS abnormal in CF
49
List organs affected by CF
1. lungs 2. pancreas (EPI) 3. Liver/biliary system (steatosis, focal fibrosis, cholestasis, sludge, stones, PSC, small GB) 4. GI tract (Meconium ileus, DIOS) 5. vas deferense (infertility)
50
what is the test used for CF newborn screen?
Immunoreactive trypsinogen FP: hypoxia, res. distress, low apgar, Trisomies, renal dysfunction, contaminated filter paper, early specimen collection FN: severe meconium ileus, older infant, PA sufficient CF variant
51
Gold STD testing for CF?
Sweat chloride >60 --> CF 40-60--> borderline --> repeat + genetics <40 --> normal FP: (skin dehydration stuff) anorexia nervosa addison disease nephrogenic diabetes insipidus hypothyroidism FN: edema
52
Adj/Disadvantages of fecal elastase-1 and fecal fat collection?
FE: less specific with mild PI Affected by diarrhea not affected PERT FFC: Difficult test, especially in diapered child Affected by dietary fat Different coefficient fecal fat absorption cutoff for diff age (<7% abnormal, >15% <6 mo)
53
GI complications in CF?
1. Meconium ileus 2. DIOS 3. GERD 4. CFLD 5. Increase GI malignancy 6. Nutritional deficiencies 7. electrolyte abnormalities

Pancreas (1 decks)

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