Updated: 12/26/2018

Zollinger-Ellison (ZE) Syndrome

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Snapshot
  • A 42-year-old male executive complains of upper abdominal pain. He says he is under considerable stress at work and smokes a pack of cigarettes daily. He has no history of shortness of breath, swelling of the feet, chest pain, or allergies. Further history reveals that the pain is worse at night and often wakens him from sleep. It occurs between meals and is subsided by drinking milk and avoiding spicy foods. On one occasion he vomited blood. Testing reveals a basal acid output (BA) of 60 mEq (normal <5 mEq) and a serum gastrin level of 1000 pg/ml (normal <300 pg/ml)
Introduction
  • Caused by a malignant duodenal or pancreatic islet cell tumor that ectopically secretes excessive amounts of gastrin 
    • leads to increased secretion of acid by parietal cells
    • peptic ulcer disease
      • ulcers are in unusual positions, without H. pylori infection or NSAID use
  • Associated with MEN type I 
Presentation
  • Symptoms
    • similar to PUD, GERD 
    • diarrhea
      • acidity in the duodenum inactivates pancreatic enzymes
Evaluation
  • BAO:MAO ratio 
    • increased
  • Serology
    • increased insulin, glucagon, gastrin 
Treatment
  • Medical
    • PPIs
    • chemotherapy
  • Surgical
    • surgical resection of tumor
 

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Questions (4)
Lab Values
Blood, Plasma, Serum Reference Range
ALT 8-20 U/L
Amylase, serum 25-125 U/L
AST 8-20 U/L
Bilirubin, serum (adult) Total // Direct 0.1-1.0 mg/dL // 0.0-0.3 mg/dL
Calcium, serum (Ca2+) 8.4-10.2 mg/dL
Cholesterol, serum Rec: < 200 mg/dL
Cortisol, serum 0800 h: 5-23 μg/dL //1600 h:
3-15 μg/dL
2000 h: ≤ 50% of 0800 h
Creatine kinase, serum Male: 25-90 U/L
Female: 10-70 U/L
Creatinine, serum 0.6-1.2 mg/dL
Electrolytes, serum  
Sodium (Na+) 136-145 mEq/L
Chloride (Cl-) 95-105 mEq/L
Potassium (K+) 3.5-5.0 mEq/L
Bicarbonate (HCO3-) 22-28 mEq/L
Magnesium (Mg2+) 1.5-2.0 mEq/L
Estriol, total, serum (in pregnancy)  
24-28 wks // 32-36 wks 30-170 ng/mL // 60-280 ng/mL
28-32 wk // 36-40 wks 40-220 ng/mL // 80-350 ng/mL
Ferritin, serum Male: 15-200 ng/mL
Female: 12-150 ng/mL
Follicle-stimulating hormone, serum/plasma Male: 4-25 mIU/mL
Female: premenopause: 4-30 mIU/mL
midcycle peak: 10-90 mIU/mL
postmenopause: 40-250
pH 7.35-7.45
PCO2 33-45 mmHg
PO2 75-105 mmHg
Glucose, serum Fasting: 70-110 mg/dL
2-h postprandial:<120 mg/dL
Growth hormone - arginine stimulation Fasting: <5 ng/mL
Provocative stimuli: > 7ng/mL
Immunoglobulins, serum  
IgA 76-390 mg/dL
IgE 0-380 IU/mL
IgG 650-1500 mg/dL
IgM 40-345 mg/dL
Iron 50-170 μg/dL
Lactate dehydrogenase, serum 45-90 U/L
Luteinizing hormone, serum/plasma Male: 6-23 mIU/mL
Female: follicular phase: 5-30 mIU/mL
midcycle: 75-150 mIU/mL
postmenopause 30-200 mIU/mL
Osmolality, serum 275-295 mOsmol/kd H2O
Parathyroid hormone, serume, N-terminal 230-630 pg/mL
Phosphatase (alkaline), serum (p-NPP at 30° C) 20-70 U/L
Phosphorus (inorganic), serum 3.0-4.5 mg/dL
Prolactin, serum (hPRL) < 20 ng/mL
Proteins, serum  
Total (recumbent) 6.0-7.8 g/dL
Albumin 3.5-5.5 g/dL
Globulin 2.3-3.5 g/dL
Thyroid-stimulating hormone, serum or plasma .5-5.0 μU/mL
Thyroidal iodine (123I) uptake 8%-30% of administered dose/24h
Thyroxine (T4), serum 5-12 μg/dL
Triglycerides, serum 35-160 mg/dL
Triiodothyronine (T3), serum (RIA) 115-190 ng/dL
Triiodothyronine (T3) resin uptake 25%-35%
Urea nitrogen, serum 7-18 mg/dL
Uric acid, serum 3.0-8.2 mg/dL
Hematologic Reference Range
Bleeding time 2-7 minutes
Erythrocyte count Male: 4.3-5.9 million/mm3
Female: 3.5-5.5 million mm3
Erythrocyte sedimentation rate (Westergren) Male: 0-15 mm/h
Female: 0-20 mm/h
Hematocrit Male: 41%-53%
Female: 36%-46%
Hemoglobin A1c ≤ 6 %
Hemoglobin, blood Male: 13.5-17.5 g/dL
Female: 12.0-16.0 g/dL
Hemoglobin, plasma 1-4 mg/dL
Leukocyte count and differential  
Leukocyte count 4,500-11,000/mm3
Segmented neutrophils 54%-62%
Bands 3%-5%
Eosinophils 1%-3%
Basophils 0%-0.75%
Lymphocytes 25%-33%
Monocytes 3%-7%
Mean corpuscular hemoglobin 25.4-34.6 pg/cell
Mean corpuscular hemoglobin concentration 31%-36% Hb/cell
Mean corpuscular volume 80-100 μm3
Partial thromboplastin time (activated) 25-40 seconds
Platelet count 150,000-400,000/mm3
Prothrombin time 11-15 seconds
Reticulocyte count 0.5%-1.5% of red cells
Thrombin time < 2 seconds deviation from control
Volume  
Plasma Male: 25-43 mL/kg
Female: 28-45 mL/kg
Red cell Male: 20-36 mL/kg
Female: 19-31 mL/kg
Cerebrospinal Fluid Reference Range
Cell count 0-5/mm3
Chloride 118-132 mEq/L
Gamma globulin 3%-12% total proteins
Glucose 40-70 mg/dL
Pressure 70-180 mm H2O
Proteins, total < 40 mg/dL
Sweat Reference Range
Chloride 0-35 mmol/L
Urine  
Calcium 100-300 mg/24 h
Chloride Varies with intake
Creatinine clearance Male: 97-137 mL/min
Female: 88-128 mL/min
Estriol, total (in pregnancy)  
30 wks 6-18 mg/24 h
35 wks 9-28 mg/24 h
40 wks 13-42 mg/24 h
17-Hydroxycorticosteroids Male: 3.0-10.0 mg/24 h
Female: 2.0-8.0 mg/24 h
17-Ketosteroids, total Male: 8-20 mg/24 h
Female: 6-15 mg/24 h
Osmolality 50-1400 mOsmol/kg H2O
Oxalate 8-40 μg/mL
Potassium Varies with diet
Proteins, total < 150 mg/24 h
Sodium Varies with diet
Uric acid Varies with diet
Body Mass Index (BMI) Adult: 19-25 kg/m2
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(M1.GI.25) A 41-year-old female complains of frequent diarrhea and abdominal pain between meals. Endoscopy reveals a duodenal ulcer distal to the duodenal bulb. CT scan of the abdomen demonstrates a pancreatic mass, and subsequent tissue biopsy of the pancreas reveals a malignant islet cell tumor. Which of the following hormones is likely to be markedly elevated in this patient: Review Topic

QID: 101082
1

Gastrin

68%

(13/19)

2

Cholecystokinin

5%

(1/19)

3

Secretin

11%

(2/19)

4

Vasoactive intestinal peptide

11%

(2/19)

5

Motilin

0%

(0/19)

M1

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PREFERRED RESPONSE 1

(M1.GI.174) A 45-year-old male patient with a history of recurrent nephrolithiasis and chronic lower back pain presents to the ER with severe, sudden-onset, upper abdominal pain. The patient is febrile, hypotensive, and tachycardic, and is rushed to the OR for exploratory laporotomy. Surgery reveals that the patient has a perforated gastric ulcer. Despite appropriate therapy, the patient expires, and subsequent autopsy reveals multiple ulcers in the stomach, duodenum, and jejunum. The patient had been complaining of abdominal pain and diarrhea for several months but had only been taking ibuprofen for his lower back pain for the past 3 weeks. What is the most likely cause of the patient's presentation? Review Topic

QID: 101231
1

A gastrin-secreting tumor of the pancreas

93%

(14/15)

2

A vasoactive-intestinal-peptide (VIP) secreting tumor of the pancreas

0%

(0/15)

3

Cytomegalovirus infection

0%

(0/15)

4

H. pylori infection

0%

(0/15)

5

Chronic NSAID use

7%

(1/15)

M1

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PREFERRED RESPONSE 1
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