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ZOLLINGER-ELLISON
SYNDROME
(Mr. Lim case problem)
MD-2508 (DIGESTIVE SYSTEM)
PROBLEM BASED LEARNING (PBL)
DR. FATMA S.A. SAGHIR
TRIGGER 1
• Mr. Lim (Man), 47 Y/O (Adult), House painting
business
• Main complain : abdominal pain ass. Diarrhea
• Referred to Physician, endoscopy shows ulcer
in duodenal bulb
• Ct scan shows 3cm mass on head of pancrease
• Thought to be gastrinoma
• Omeprazole prescribed
• Undergo laparoscopic surgery
TRIGGER 2
TRIGGER 3
9 questions to ask about
pain
9 questions to ask about pain
S – Site of the pain
O – Onset of the pain
C – Characteristic of the pain
R – Radiation of the pain
A – Association of the pain
T – Time course of the pain
E – Exacerbating/relieving factor
S – Severity of the pain
Sign of
GIT
SIGN OF GIT
Inspection
- Abnormal abdominal
structure
- Skin have
hemangioma or
dilated vein
- Mass
- Asymmetry motion
during respiration
- Herniation during
cough
- Anal fissures or
hemorrhoids
Auscultation
- Abnormal bowel
sounds
- Rub and bruits
sound
Palpation
- Tenderness of
visceral organ
- Nodularity on
liver
- Mass
- Change in size of
visceral organ
Percussion
- Ascites
Dehydration
- Decrease in
skin turgor
- Dry mucous
membranes
-Less tears and
urine (babies)
- Increased pulse
- Decreased
blood pressure
-Delayed
capillary refill
GENERAL GI
SYMPTOMS
General symptom GIT disorder
• Constipation (painful bowel movement)
• Abdominal pain (indigestion)
• Diarrhoea (acute & chronic)
• Gas in GI tract (bloating)
• GI bleeding ( peptic ulcer )
• Nausea
• Vomiting
DIFFERENT
BETWEEN
GASTRIC ULCER
AND
DUODENAL ULCER
Gastric Ulcer Duodenal Ulcer
Occur in the stomach. Occur in the duodenum.
Abdominal pain cannot be
relieve by eating.
Abdominal pain can be relieve
by eating.
Epigastric pain 1-2 hours after
eating.
Epigastric pain 2-5 hours after
eating.
Can cause melena or
haemetemesis.
Can cause melena or
hematochezia.
Pain are less likely awaken
patient at night (30-40%.)
Pain often awaken patient
during the night (50-80%).
Underlying
causes of peptic
ulcer &
duodenal ulcer
Peptic
ulcer
Chronic use
of NSAID or
aspirin
Steroids
Smoking
Helicobacter
pylori
Duodenal
ulcer
Helicobacter
pylori
↑ gastric
secretion
↑ rate of
gastric
emptying
Blood group
o
Cirrhosis
COPD
Differential Diagnosis
Signs &
Symptoms
Zollinger –
Elison
Syndrome
Peptic Ulcer Pyloric
Obstruction
Gastric
cancer
Indigestion / / / /
Loss of appetite / / / /
Abdominal pain / /
Oily diarrhoea /
Ulcer in
duodenum
/ / /
High gastrin
level
/ /
PAIN THAT
WORSEN
AFTER EATING
1. Cholecystitis
• inflammation of the gallbladder (mostly by gall
stones)
• Gallstones stuck in the cystic duct
• When eating, bile is produced then builds up
in the gallbladder
• bladder becomes stretched then lead to
inflammation
2. Diverticulitis
Diverticulitis happens when feces get trapped in
the pouches (diverticula). This allows bacteria
to grow in the pouches. This can lead to
inflammation or infection.
causes are idiopathic,
Diverticulitis can be very painful.
PAIN THAT
RELIEVE BY
EATING
1. duodenal Ulcer
• Imbalance amount of acid produced and
mucosal barrier of duodenum acid damage
duodenum ulcer develop food ingested
food contain some bases neutralise acid at
duodenum decrease Inflammation
2. Gastritis
• Imbalance amount of acid produced and
mucosal barrier of stomach acid damage
stomach food ingested gastric acid act
on food decrease Inflammation
Noted: Mr Lim suffered diarrhea (looked oily)
STEATORRHEA
Definition :
Passing of stools that contain a high amount of fat.
Symptom :
• Fatty Stools
• Float stools
• Pale Color
• Foul Smell
• Weight Loss
• Heavy Stools
Causes :
• Bile Acid Deficiency
• Defects of Pancreatic Enzymes
- Pancreatitis - Inflammatory bowel disease
- Cystic fibrosis - Gastrointestinal surgery
• Indigestible Fats
• Medicines
- Drugs like Orlistat can block the enzymes that digest
fat components in the diet..
• Poor Absorption of Nutrients
• Excessive Alcohol Consumption
- Too much consumption of alcohol for a very long time can
damage tissues in the pancreas and cause
scarring and swelling
LAB REPORT
(investigation)
• Gastrin hormone test (Mr Lim got high level : 800pg/ml)
-to detect excess production of gastrin.
-to diagnose gastrin producing tumor, peptic ulser and G-
cells
hyperplasia.
-low: usually are off concern.
high: ZE syndrome, G-cells hyperplasia.
low after surgery then high: recurrance of tumor.
• Basal Gastric H+ secretion (Mr Lim got high rate : 100mEq/hr)
- Measure acid secretion in fasting state.
- Completely absence: pernicious anemia.
- Decreased: gastric cancer.
- Increase: ZE syndrome.
• Pentagastrin stimulation test (Mr Lim: No increased in H+
secretion )
- to test basal and stimulated acid production by the
parietal cells of the stomach.
- Low: achlorhydia*.
High: ZE syndrome
• Secretin stimulation test (Mr Lim: the serum gastrin increased
to 1,100pg/mL)
- To measure pancrease ability towards secretin.
- stimulates gastrin release in patients with gastrinoma.
- small changes in serum gastrin concentrations occur
patients with peptic ulcer.
Causes of tumor
& Gastrinoma
What causes tumor ?
GASTRINOMA (gastrin-secreting
tumor)
 Gastrinoma is one of the type of neuroendocrine
tumor
 This tumor will produce large amounts of gastrin
 Neuroendocrine tumours (NETs) are tumours that
develop in cells that are triggered by nerve cells to
produce hormones.
 People who have a rare family cancer syndrome
called Multiple Endocrine Neoplasia Type 1 (MEN 1)
have a higher risk of gastrinoma
Manifestation
1. Usually idiopathic PUD (75%)
- Haemorrhage, perforation and obstruction are common
complications.
2. May get diarrhoea (25%)
- from the acid (destroys lipase and produces steatorhoea).
Often delayed diagnosis
- mean time of symptoms to diagnosis is 5 years.
Suspect if:
Diarrhoea, pain and weight loss.
Recurrent or refractory ulcers
Prominent rugal folds (trophic effect of gastrin)
GI symptoms in an MEN-1 patient
Large amount of gastrin produced
Too much stomach acid produced
damage to the mucosal lining of the GI tract
May form peptic ulcer in stomach or duodenum
acid inactivates pancreatic enzymes, which contributes to
the diarrhoea, steatorrhoea, and malabsorption
COMPLICATION OF
ZOLLINGER-ELLISON
SYNDROME
COMPLICATION OF ZES
• May have only one gastrinoma or may have
several.
• ZES patients who have multiple endocrine
neoplasia type 1, can cause tumors in the
pituitary and parathyroid glands.
• Gastrinomas can be malignant (cancerous).
These malignant gastrinomas can spread to
other parts of the body, including the liver,
lymph nodes, spleen, bones, or skin.
• Bleeding, perforation, and obstruction
antacid
ANTACIDS
• Neutralize acidity ,, acidity
• Relieve sign & symptom(pain)
• Treat DU(naturally in high dose&sufficient
duration)
• Less effective in Gastric ulcer
• Systemic-NaHCO3
• Non systemic-(Mg,Al,Ca)compund
• Over counter antacid-Eno,Gaviscon,Alucid
• At hospital,pharmacy-Mg
trisilicate,Omelon,Zantac
zantac
omelon
• omelon-omeprazole,Zantac-ranitidine(has
antacid action)
omeprazole
NAME TRADE NAME: Acimax, Antra, Aspra, Gastroloc, Losec, Losectil, Lozeprel, Mopral, Olex, Omepral,
Omez, Opal, Ozid, Prilosec, Rome 20, Segazole, Ulcozol, Zegacid, Zegerid.
CHEMICAL NAME: 6-methoxy-2-[(4-methoxy-3,5-dimethylpyridin-2-yl)methanesulfinyl]-1H-1,3-
benzodiazole
DOSE 20mg once daily for 4 weeks in DU, 8 weeks in GU
40mg once daily for recurrent or severe case
ALTERNATIVE Esomoprazole, lansoprazole, pentoprazole,rabeprazole,
SIDE EFFECT - Nausea, abdominal pain, constipation
- Subacute myopathy, arthralgia, headache, skin rash
MECHANISM OF
ACTION
Permanent
(irriversable)
inhibition of H+,
K+ -ATPase
(proton pump) of
gastric parietal
cells, selectively
inhibit gastric
mucosal carbonic
anhydrase

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ZOLLINGER-ELLISON SYNDROME

  • 1. ZOLLINGER-ELLISON SYNDROME (Mr. Lim case problem) MD-2508 (DIGESTIVE SYSTEM) PROBLEM BASED LEARNING (PBL) DR. FATMA S.A. SAGHIR
  • 2. TRIGGER 1 • Mr. Lim (Man), 47 Y/O (Adult), House painting business • Main complain : abdominal pain ass. Diarrhea • Referred to Physician, endoscopy shows ulcer in duodenal bulb • Ct scan shows 3cm mass on head of pancrease • Thought to be gastrinoma • Omeprazole prescribed • Undergo laparoscopic surgery TRIGGER 2 TRIGGER 3
  • 3. 9 questions to ask about pain
  • 4. 9 questions to ask about pain S – Site of the pain O – Onset of the pain C – Characteristic of the pain R – Radiation of the pain A – Association of the pain T – Time course of the pain E – Exacerbating/relieving factor S – Severity of the pain
  • 6. SIGN OF GIT Inspection - Abnormal abdominal structure - Skin have hemangioma or dilated vein - Mass - Asymmetry motion during respiration - Herniation during cough - Anal fissures or hemorrhoids Auscultation - Abnormal bowel sounds - Rub and bruits sound Palpation - Tenderness of visceral organ - Nodularity on liver - Mass - Change in size of visceral organ Percussion - Ascites Dehydration - Decrease in skin turgor - Dry mucous membranes -Less tears and urine (babies) - Increased pulse - Decreased blood pressure -Delayed capillary refill
  • 8. General symptom GIT disorder • Constipation (painful bowel movement) • Abdominal pain (indigestion) • Diarrhoea (acute & chronic) • Gas in GI tract (bloating) • GI bleeding ( peptic ulcer ) • Nausea • Vomiting
  • 10. Gastric Ulcer Duodenal Ulcer Occur in the stomach. Occur in the duodenum. Abdominal pain cannot be relieve by eating. Abdominal pain can be relieve by eating. Epigastric pain 1-2 hours after eating. Epigastric pain 2-5 hours after eating. Can cause melena or haemetemesis. Can cause melena or hematochezia. Pain are less likely awaken patient at night (30-40%.) Pain often awaken patient during the night (50-80%).
  • 12. Peptic ulcer Chronic use of NSAID or aspirin Steroids Smoking Helicobacter pylori Duodenal ulcer Helicobacter pylori ↑ gastric secretion ↑ rate of gastric emptying Blood group o Cirrhosis COPD
  • 14. Signs & Symptoms Zollinger – Elison Syndrome Peptic Ulcer Pyloric Obstruction Gastric cancer Indigestion / / / / Loss of appetite / / / / Abdominal pain / / Oily diarrhoea / Ulcer in duodenum / / / High gastrin level / /
  • 16. 1. Cholecystitis • inflammation of the gallbladder (mostly by gall stones) • Gallstones stuck in the cystic duct • When eating, bile is produced then builds up in the gallbladder • bladder becomes stretched then lead to inflammation
  • 17. 2. Diverticulitis Diverticulitis happens when feces get trapped in the pouches (diverticula). This allows bacteria to grow in the pouches. This can lead to inflammation or infection. causes are idiopathic, Diverticulitis can be very painful.
  • 19. 1. duodenal Ulcer • Imbalance amount of acid produced and mucosal barrier of duodenum acid damage duodenum ulcer develop food ingested food contain some bases neutralise acid at duodenum decrease Inflammation
  • 20. 2. Gastritis • Imbalance amount of acid produced and mucosal barrier of stomach acid damage stomach food ingested gastric acid act on food decrease Inflammation
  • 21. Noted: Mr Lim suffered diarrhea (looked oily) STEATORRHEA
  • 22. Definition : Passing of stools that contain a high amount of fat. Symptom : • Fatty Stools • Float stools • Pale Color • Foul Smell • Weight Loss • Heavy Stools
  • 23. Causes : • Bile Acid Deficiency • Defects of Pancreatic Enzymes - Pancreatitis - Inflammatory bowel disease - Cystic fibrosis - Gastrointestinal surgery • Indigestible Fats • Medicines - Drugs like Orlistat can block the enzymes that digest fat components in the diet.. • Poor Absorption of Nutrients • Excessive Alcohol Consumption - Too much consumption of alcohol for a very long time can damage tissues in the pancreas and cause scarring and swelling
  • 25. • Gastrin hormone test (Mr Lim got high level : 800pg/ml) -to detect excess production of gastrin. -to diagnose gastrin producing tumor, peptic ulser and G- cells hyperplasia. -low: usually are off concern. high: ZE syndrome, G-cells hyperplasia. low after surgery then high: recurrance of tumor. • Basal Gastric H+ secretion (Mr Lim got high rate : 100mEq/hr) - Measure acid secretion in fasting state. - Completely absence: pernicious anemia. - Decreased: gastric cancer. - Increase: ZE syndrome.
  • 26. • Pentagastrin stimulation test (Mr Lim: No increased in H+ secretion ) - to test basal and stimulated acid production by the parietal cells of the stomach. - Low: achlorhydia*. High: ZE syndrome • Secretin stimulation test (Mr Lim: the serum gastrin increased to 1,100pg/mL) - To measure pancrease ability towards secretin. - stimulates gastrin release in patients with gastrinoma. - small changes in serum gastrin concentrations occur patients with peptic ulcer.
  • 27. Causes of tumor & Gastrinoma
  • 29. GASTRINOMA (gastrin-secreting tumor)  Gastrinoma is one of the type of neuroendocrine tumor  This tumor will produce large amounts of gastrin  Neuroendocrine tumours (NETs) are tumours that develop in cells that are triggered by nerve cells to produce hormones.  People who have a rare family cancer syndrome called Multiple Endocrine Neoplasia Type 1 (MEN 1) have a higher risk of gastrinoma
  • 30. Manifestation 1. Usually idiopathic PUD (75%) - Haemorrhage, perforation and obstruction are common complications. 2. May get diarrhoea (25%) - from the acid (destroys lipase and produces steatorhoea). Often delayed diagnosis - mean time of symptoms to diagnosis is 5 years. Suspect if: Diarrhoea, pain and weight loss. Recurrent or refractory ulcers Prominent rugal folds (trophic effect of gastrin) GI symptoms in an MEN-1 patient
  • 31. Large amount of gastrin produced Too much stomach acid produced damage to the mucosal lining of the GI tract May form peptic ulcer in stomach or duodenum acid inactivates pancreatic enzymes, which contributes to the diarrhoea, steatorrhoea, and malabsorption
  • 33. COMPLICATION OF ZES • May have only one gastrinoma or may have several. • ZES patients who have multiple endocrine neoplasia type 1, can cause tumors in the pituitary and parathyroid glands. • Gastrinomas can be malignant (cancerous). These malignant gastrinomas can spread to other parts of the body, including the liver, lymph nodes, spleen, bones, or skin. • Bleeding, perforation, and obstruction
  • 35. ANTACIDS • Neutralize acidity ,, acidity • Relieve sign & symptom(pain) • Treat DU(naturally in high dose&sufficient duration) • Less effective in Gastric ulcer • Systemic-NaHCO3 • Non systemic-(Mg,Al,Ca)compund • Over counter antacid-Eno,Gaviscon,Alucid • At hospital,pharmacy-Mg trisilicate,Omelon,Zantac
  • 38. NAME TRADE NAME: Acimax, Antra, Aspra, Gastroloc, Losec, Losectil, Lozeprel, Mopral, Olex, Omepral, Omez, Opal, Ozid, Prilosec, Rome 20, Segazole, Ulcozol, Zegacid, Zegerid. CHEMICAL NAME: 6-methoxy-2-[(4-methoxy-3,5-dimethylpyridin-2-yl)methanesulfinyl]-1H-1,3- benzodiazole DOSE 20mg once daily for 4 weeks in DU, 8 weeks in GU 40mg once daily for recurrent or severe case ALTERNATIVE Esomoprazole, lansoprazole, pentoprazole,rabeprazole, SIDE EFFECT - Nausea, abdominal pain, constipation - Subacute myopathy, arthralgia, headache, skin rash MECHANISM OF ACTION Permanent (irriversable) inhibition of H+, K+ -ATPase (proton pump) of gastric parietal cells, selectively inhibit gastric mucosal carbonic anhydrase

Notas do Editor

  1. Skin have hemangioma and dilated vein : skin of abdomen, it indicates severe liver disease Assymmetry motion during respiration : Due to muscular rigidity from underlying inflammation Bowel sound (on small intestine region): Bowel sounds are created when food, bowel gas and feces move through the intestines. Most bowel sounds are normal - Increased bowel sound : obstruction Decrease bowel sound : Ileus Bruit Sound : found on spleen indicate Change in size: Increased liver size: hepatitis, metastasic carcinoma, cirrhosis Decrease liver size : cirrhosis Nodular on liver : metastatic carcinoma, cirrhosis Increase spleen size: viral infection,portal hypertension Ascites: Ascites is excess fluid in the space between the membranes lining the abdomen and abdominal organs.
  2. Indigestion, also called dyspepsia, is a common cause for abdominal pain. This condition results from the incomplete or abnormal digestion of food. Indigestion can be caused by many different GI disorders; as well as by eating too much, too fast, or in a stressful situation; by a high intake of fat, alcohol, or caffeine; by certain drugs; or by emotional problems (e.g., anxiety, depression) Nausea is an unpleasant sensation in the stomach or the back of the throat. Nausea can be described as feeling queasy or squeamish, or as an upset stomach. This condition often occurs prior to retching or vomiting (emesis).Symptoms that may accompany nausea include dizziness and lightheadedness, difficulty swallowing, increased saliva production, rapid heart rate, and changes in skin temperature. Retching without producing vomit is called dry vomiting or dry heaves. Q & A: Do u hv any difficulty in excrete? Do u hv any unpleasant stomach condition? Any bloating or abdominal distention? do u hv diarrhoea? If yes, how frequent? Any feeling of queasy or squeamish? Do u vomit? If yes is there any blood in it?
  3. Melena: The black color (feaces) is caused by the hemoglobin in the blood Haemetemesis:  vomiting of blood Hematochezia:  the passage of fresh blood through the anus, usually in or with stools 
  4.  achlorhydia: states where the production of gastric acid in the stomach is absent or low, respectively. It is associated with various other medical problems.
  5.  ZES : tumor in pancreas that stimulates the acid-secreting cells of the stomach (parietal cells) to maximal activity  ZES may due to the autosomal dominant familial syndrome (multiple endocrine neoplasia type 1)
  6. Omeprazole : irreversible proton pump inhibitor. It suppresses stomach acid secretion by specific inhibition of the H +/K + ATPase system found at the secretory surface of gastric parietal cells. Because this enzyme system is regarded as the acid (proton, or H+) pump within the gastric mucosa, omeprazole inhibits the final step of acid production.