GI Zebras

Question 1

pt presents with skin flushing and telangiectasis, diarrhea, and bronchospasm and deramatitis

Answer 1

features of carcinoid syndrome

Question 2

GI manifestations of Carcinoid syndrome

Answer 2

diarrhea, abdominal cramping

Question 3

niacin deficiency and dermatitis and diarrhea and dementia

Answer 3

seen with carcinoid syndrome

Question 4

skin manifestations of carcinoid syndrome

Answer 4

flushing, telangiectasias and cyanosis

Question 5

pulmonary manifestation of carcinoid syndrome

Answer 5

bronchospasm

Question 6

cardiac manifestation of carcinoid syndrome

Answer 6

valvular lesions (right > left side)

Question 7

diagnosis of carcinoid syndromes

Answer 7

elevated 24 hr urinary excretion of 5 HIAA CT/MRI of abdomen and pelvis to localize tumor octreoscan to detect metastasis echocardiogram (see if there is heart disease present)

Question 8

treatment of carcinoid syndrome

Answer 8

octreotide for symptomatic patients and prior to surgery and anesthesia surgery for liver metastasis.

Question 9

what is carcinoid tumor?

Answer 9

slow growing neuroendocrine tumors found most commonly in the distal small intestine nad proxmal colon and lung. Can secrete up to 40 products (serotonin, histamine, vasoactive intestinal peptide) Can spread to liver and prevent this hepatic inactivation which can cause carcinoid syndrome] can also see extraintestinal tumors can have direct access to the systemic circulation without immediate hepatic clearance of their products.

Question 10

what is carcinoid syndrome?

Answer 10

syndrome of espidoic flushing and secretory diarrhea and cutaneous telangiectasis and bronchospasm and tricuspid regurgitation from carcinoid tumors.

Question 11

what is systemic mastocytosis

Answer 11

increased mast cells and see skin involvement most commonly seen with pruritis and urticaria pigmentosa (yellow red raised papules and nodules in the upper or lower extremities).

Question 12

What are VIPomas

Answer 12

secrete vasoactive intestinal peptide tp have large volume secretory watery diarrhea and elctrolytes disturbances (hypokalemia, hypochlorhydria) and see flushing in 20% of pts with VIPomas.

Question 13

What is the carcinoid crisis?

Answer 13

life threatening complication fro ma tumor manipulation (surgery or biopsy or anesthesia) which causes the release of large amounts of active products.

Question 14

how to prevent carcinoid crisis?

Answer 14

give somatostatin analogs or 5HIAA analogs to provide relief and prevent a carcinoid crisis.

Question 15

What is pellagra?

Answer 15

diarrhea, dermatitis, and dementia (seen with niacin deficiency) and can see this with deficiency of tryptophan and niacin deficiency when you have wide metastasis of carcinoid tumor.

Question 16

Drug induced liver injury treatment

Answer 16

if no encephalopathy or coagulopathy need to stop offending drug and watchful waiting.

Question 17

Most common drugs that cause drug induced liver injuryare:

Answer 17

CNS agents like an antiepileptic or psychotropic drugs (amoxicillin-clavulante drug induced liver injury can occur following cessation of drug

Question 18

mean onset of juandice from amoxicillin cessation is about

Answer 18

15 days in cases of drug induced liver injury but range is about 2-55 days.

Question 19

4 D’s of niacin deficiency?

Answer 19

pellagra - see dermatitis, diarrhea, and dementia and this can lead to death.

Question 20

Zollinger Ellison syndrome

Answer 20

see gastrinomas that secrete gastrin and cause peptic ulcers.

Question 21

what medical condition is associated with Zollinger Ellison syndrome

Answer 21

MEN1 (20%) of cases

Question 22

when do we see Zollinger Ellison syndrome

Answer 22

20-50 yrs old with dyspepsia, reflux symtpoms, abdominal pain, weight loss and frank GI bleeding

Question 23

What is the screening test for Zollinger Ellison syndrome and what is seen on EGD

Answer 23

EGD findings: thickened gastric folds, multiple peptic ulcers, refractory uclers despite PPI and ulcers distal to duodenum in the jejunum. Screening test: check gastrin level. Gastrin level <110 pg/ml rules gastrinoma out

Question 24

what level of gastrin is highly suggestive of Zollinger Ellison syndrome What to do after high gastrin?

Answer 24

gastrin level >1000 pg/ml need to check gastric pH with pH>4 ruling out gastrinoma pH<4 suggests gastrinoma and requires further testing with a EUS and somatostain receptor sintigraphy and CT or MRI for localization

Question 25

what if gastrin level is 110-1000?

Answer 25

needs asecretin stimulation test. Negative secretin test rules out gastrinoma, positive test needs localization testing.

Question 26

Gastrinoma in association with MEN1 responds to

Answer 26

PPI

Question 27

How to treat sporadic gastrinomas or MEN-1 pts with uncontrolled symptoms on medical therapy?

Answer 27

surgery

Question 28

Where does gastrinoma commonly occur in the GI tract?

Answer 28

70% in the duodenum and less commonly at hte head of hte pancreas.

Question 29

Cancer screening recommendations for Peutz Jegher’s Syndrome?

Answer 29

Colonoscopy at 18 yrs, EGD and small bowel series at 8 yrs old. need early mammograms and testicular examination in boys under 12 yrs old

Question 30

Evaluation of suspected gastrinoma

Answer 30

Question 31

acute intermittent porphyria

Answer 31

caused by elevated prophobilinogen and alpha aminolevulinic acid in tissues due to a heme pathway enzyme deficiency (hepatic PBG deaminase).

Factors causing AIP are: starvation, stress, infection, ETOH intake, infection, drugs (sulfonamides and hormones) and low carb diet.

Question 32

Peutz Jegher’s Syndrome or PJS

genetic inheritance

skin manifestations.

Answer 32

rare autosomal dominant disorder with multiple hamartomatous GI polyps and pigmented mucocutaneous papules and seen in both men and women and across races.

Skin lesions are present with 95% at birth and are flat gray to brown spots about 1-5 mm and are on lips, perioral area, buccal mucosa, hands and feet. These are different from freckles.

can have greater risk for small bowel cancer

greater risk for breast, gynecological cancers, pancreatic cancer and risk for GI bleeding and intussuception and small bowel obstruction.

Question 33

GI complications with Peutz Jegher’s syndrome or PJS.

Answer 33

Can have lots of GI polyps everhwere in small intestine, colon, stomach, and rectum. Can see obstruction (intussusception or occlusion by polyp) around age 10-30 yrs. See abdominal pain and rectal bleeding.

There is also a risk for malignancy with the polyps. Aggressive surveillance is needed.

Colonoscopy at 18 yrs, EGD and small bowel series at 8 yrs old. need early mammograms and testicular examination in boys under 12 yrs old

Greater risk for breast, gynecological and pancreatic cancers.

Question 34

someone is <40 yrs and unexplained liver dx, test for:

Answer 34

Wilson’s dx. Autosomal recessive metabolic disorder

serum ceruloplasmin measurement as screen. Will be low

confirmation test: 24 hr urine copper excrement - should be high.

Question 35

acute intermittent porphyria presentation:

Answer 35

recurrent episodes of severe abdominal pain, seen with signs of constipation, signs of ileus (nausea, abdominal distention, decreased bowel sounds due to autonomic neuropathy

See bladder dysfunction resulting in urinary retention, incontinence, dysuria

see dark or reddish urine.

can have sensory neuropathy early on but motor neuropathy and painful flaccid paralysis is a late presentation.

Question 36

acute intermittent porphyria labs

Answer 36

see reddish or dark urine

hyponatremia - due to SIADH and see increased risk for seizure

Tachycardia and hypertension due to catecholamine release during attack from pain are common

Question 37

diagnosis of acute intermittent porphyria

Answer 37

elevated urinary porphobilinogen measurement confirms diagnosis. also will see elevated urine heptacrboxyl prophyrin level.

best measured in acute attack.

Question 38

Treatment of acute intermittent porphyria

Answer 38

symptomatic therapy

glucose loading

IV hemin - reconstitutes regulartoy heme pool in the liver to lower PBG

Future attacks are prevented by avoiding starvation, stress, infection, ETOH intake, drugs (hormones and sulfonamides)

Question 39

what increases gastrin levels?

Answer 39

PPI so if suspecting gastrinoma, should test before therapy

Question 40

MEN1 is associated with

Answer 40

3 P’a

pituitary adenoma

parathyroid hyperplasia

pancreatic or gastrinoma

Question 41

after diagnosing porphyria cutanea tarda what to also check?

Answer 41

rule out hemochromatosis, genetic testing, HIV, and hep C and hepatic tumors wiht U/S

Treatment: reduce sun exposure , eliminate risk factors, phlebotomy for iron overload and if needed administration of antimalarial agents.