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

soundPalpation- Tenderness of

visceral organ- Nodularity on

liver- Mass- Change in size of

visceral organPercussion- 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 relieveby eating.

Epigastric pain 1-2 hours aftereating.

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 gastrinlevel

/ /

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 ulcersProminent 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 OFZOLLINGER-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&sufficientduration)• 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