Baylor University Medical Center Proceedings April 2017 - 177

Benign pancreatic hyperenzymemia (Gullo syndrome),
histamine intolerance, and carbohydrate malabsorption
Wolfgang J. Schnedl, MD, Dietmar Enko, MD, Harald Mangge, MD, Michael Schenk, MD, Sonja Lackner, MSc,
and Sandra J. Holasek, PhD

Benign pancreatic hyperenzymemia (Gullo syndrome) is characterized
by a more than threefold increase of the pancreatic enzymes lipase and
amylase in the absence of a pancreatic disease over a period of more
than 1 year, with elevations and significant undulations of pancreatic
enzyme serum concentrations occurring on a day-to-day basis for 5
consecutive days. Nonspecific abdominal complaints may be caused
by carbohydrate and/or protein malabsorption. We report a patient with
benign pancreatic hyperenzymemia with lactose and histamine malabsorption; the symptoms of gastrointestinal malabsorption were treated
successfully with an individually tailored lactose- and histamine-free diet.

enign pancreatic hyperenzymemia (Gullo syndrome)
is characterized by a more than threefold increase of
the serum pancreatic enzymes lipase and amylase in
the absence of a pancreatic disease (1). Gastrointestinal malabsorption is caused mainly by carbohydrates (lactose
and fructose), proteins (gluten), and biogenic amines (e.g.,
histamine) and frequently manifests in nonspeciﬁc abdominal
complaints. We tested a patient with Gullo syndrome for gastrointestinal malabsorption and diagnosed lactose and histamine
malabsorption. This patient with nonspeciﬁc abdominal complaints due to lactose and histamine malabsorption recovered
with an individually tailored diet free of lactose and histamine
within a few days (2).
CASE DISCUSSION
A 71-year-old white man was examined for repeatedly
elevated lipase and amylase serum values over a time period of
3 years and presented with sustained nonspeciﬁc abdominal
complaints. His symptoms were postprandial abdominal discomfort in the right upper quadrant, bloating, diﬀuse abdominal pain, and semisolid stools. Physical examination showed a
bloated abdomen, and anamnesis revealed no weight loss.
During the 3 years before presentation, the elevation of serum
lipase and amylase levels reached 450 U/L (normal <60) and
367 U/L (normal 20-100), respectively. Abdominal sonography
revealed gallbladder stones, and subsequently a laparoscopic cholecystectomy was performed. Repetitive abdominal computed
tomography and magnetic resonance imaging demonstrated no
abdominal or pancreatic abnormalities. Gastroscopy ruled out
Proc (Bayl Univ Med Cent) 2017;30(2):177-178

Helicobacter pylori infection or other abnormalities, and colonoscopy disclosed no abnormalities. The serum total cholesterol
was 253 mg/dL; low-density lipoprotein cholesterol, 166 mg/dL;
glutamate-oxaloacetate-transaminase, 49 U/L (normal <35); and
glutamate-pyruvate-transaminase, 52 U/L (normal <45). Using
a radio extraction assay, the diamine oxidase in serum (Sciotec
Diagnostic Technologies, Tulln, Austria) was 2.6 U/mL (normal
>10 U/mL) (3). Triglycerides, calcium, IgG4, carcinoembryonic
antigen, carbohydrate antigen 19-9, fecal pancreatic elastase test,
and C-reactive protein were normal. The erythrocyte sedimentation rate was <20 mm in 2 hours.
Hydrogen breath tests were performed to detect lactose and
fructose malabsorption (Gastrolyzer, Bedfont Scientiﬁc Inc.,
Kent, England). During the breath test with a drink containing 50 g lactose dissolved in 200 mL water, the end-expiratory
exhalation of hydrogen was measured every 30 minutes for a
period of 150 minutes. The hydrogen value increased from the
baseline 10 up to 19 parts per million (ppm) (normal <20). This
test demonstrated decreasing blood glucose levels from a fasting
value of 105 mg/dL to 88 and 86 mg/dL after 1 and 2 hours,
respectively (normal is an increase >20) (4). In the breath test
with a drink containing 25 g fructose load dissolved in 200 mL of
water, the end-expiratory exhalation of hydrogen was <20 ppm.
Antibodies against tissue transglutaminase were not found.
A registered dietitian developed an individually tailored diet
for this patient, which resulted in the improvement of symptoms
within a few days. At 6 months, the patient was still symptom
free. Written informed consent was obtained for all procedures,
which were in accordance with the Declaration of Helsinki and
the recommendations of the local ethics committee.
From Practice for General Internal Medicine, Bruck, Austria (Schnedl); Institute of
Pathophysiology, Centre for Molecular Medicine, Medical University of Graz, Graz,
Austria (Schnedl, Lackner, Holasek); Institute of Laboratory Medicine, General
Hospital Steyr, Steyr, Austria (Enko); Clinical Institute of Medical and Chemical
Laboratory Diagnosis, Medical University of Graz, Graz, Austria (Mangge); and
Das Kinderwunsch Institut Schenk GmbH, Dobl, Austria (Schenk).
Competing interests: Wolfgang J. Schnedl received speaking honoraria from
Sciotec Diagnostic Technologies. The other authors declare no competing interests. The authors have received no funding for this manuscript.
Corresponding author: Wolfgang J. Schnedl, MD, Professor of Internal Medicine,
Practice for General Internal Medicine, Theodor Körnerstrasse 19b, A-8600
Bruck/Mur, Austria (e-mail: w.schnedl@dr-schnedl.at).
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