Gullo's Syndrome Research Articles

“Gullo syndrome: A case report”

“Gullo syndrome: A case report”

A clinical case of asymptomatic pancreatic hyperenzymemia on the background of taking a Glucagon-like peptide-1 analogue (GLP-1)

According to current statistics, the incidence of type 2 diabetes has increased significantly over the past few years. The number of drugs prescribed to correct carbohydrate metabolism is also increasing, and new groups of hypoglycemic drugs are appearing. This prompts a more detailed study and analysis of the possible side effects of the prescribed therapy. One of the modern groups of medications f or treating type 2 diabetes are analogues of glucagon-like peptide-1 receptor agonists, which, in addition to correcting carbohydrate metabolism, have a beneficial effect on the risk of developing cardiovascular events. Despite this, there is evidence that their use may be associated with the development of undesirable adverse effects from the pancreas, particularly acute pancreatitis, pancreatopathy, or asymptomatic hyperenzymemia. The aim of our work was the analysis of a clinical case of an asymptomatic increase in the level of pancreatic enzymes (Gullo's syndrome) in a patient with type 2 diabetes, who used a glucagon-like peptide-1 receptor analogue for treatment according to the usual scheme (dulaglutide 0.75 mg subcutaneously once a week during 2 years). As a result, the patient developed a side effect that could be related to taking this medicine. This did not lead to the withdrawal of the drug but required a more thorough examination of the patient and regular further screening for the timely detection of the development of possible organic pathology of the pancreas in the future. So, after analyzing this clinical case, it is possible to see the possibility of developing asymptomatic pancreatic hyperenzymopathy during the treatment of type 2 diabetes mellitus with glucagon-like peptide-1 receptor agonist analogues, which should be taken into account when determining the treatment tactics.

Approach to Hyperamylasemia and Hyperlipasemia

Hyperamylasemia and hyperlipidemia occur when the amounts of amylase and lipase that enter the blood overweigh the kidney and liver reticuloendothelial system clearance of the enzymes. In normal homeostasis, most of these enzymes are excreted from the apical membrane of the acinar cells into the ductal system of the gland. However, in pathological conditions, this apical exocytosis is blocked, and the leakage of these enzymes at the basolateral membrane is increased. Therefore, these enzymes are released into the bloodstream. Many different pancreatic pathologies and Gullo syndrome can cause this type of leakage in the pancreas. This mechanism can also cause basolateral leakage in other organs that secrete amylase and lipase. In small bowel inflammatory conditions, amylase and lipase are absorbed through the inflamed mucosa and introduced into the bloodstream due to increased vascular permeability. Regardless of the enzymes that enter the blood circulation, several kidney and liver diseases prevent the excretion and metabolism of serum amylase and lipase. When the amylase and lipase are combined with immunoglobulins, they escape normal glomerular filtration due to the development of macroenzymes and accumulate in the blood. These situations related to the clearance of the enzymes are also crucial mechanisms of serum enzyme elevation that should not be overlooked.

Food intolerance/malabsorption may occur in rare diseases.

Sugars including lactose and fructose, or proteins (gluten), or biogenic amines (histamine), and combinations thereof may cause food intolerance/malabsorption. However, in usually asymptomatic patients with rare diseases, who present with functional, non-specific, non-allergic gastrointestinal (GI) complaints the etiologic factors of food intolerance/malabsorption need to be evaluated. We summarize patients with rare diseases, such as primary epiploic appendagitis, beta-thalassemias minor, Gullo syndrome and anomaly of the inferior vena cava, who presented functional, non-specific, non-allergic GI complaints. As conclusion, these GI symptoms in patients with otherwise asymptomatic, rare diseases were due to fructose malabsorption, histamine-, lactose intolerance and Helicobacter pylori (H.p.) infection. A registered and experienced dietician was employed to design an individually-tailored diet which ensured effective treatments and H.p. infection was accordingly eradicated.

Benign pancreatic hyperenzymemia: Gullo's syndrome

Carlo, a 2-year-old boy, was referred to our Unit for a chronic diarrhoea. His medical history revealed a San Diego hemoglobinopathy and Gilbert disease, family history was also positive for celiac disease. Clinical examination was normal. Laboratory investigations revealed unexpected hyperamylasemia (total amylase 257 U/L and pancreatic amylase 196 U/L), hyperlipasemia (199 U/L), and mild hyperbilirubinemia, mainly indirect. Celiac disease was excluded. Further investigation as TORCH and EBV serology, coagulation, insulin, C-peptide and islet cell antibodies, erythrocyte sedimentation rate, C-reactive protein, autoimmunity, sweat test and urinary amylase were negative. Abdominal echography and MRI were normal. During the 5 years follow-up, laboratory investigations confirmed the hyperamylasemia with fluctuating values, but always above the standard values for age. Excluding pancreatic and extra pancreatic pathologies, Gullo's syndrome has been suggested. This condition may occur sporadically or in a familial form. It is asymptomatic and the hyperenzymemia can persist for years. Etiology is still not clear, it has been reported defects in the passage of enzymes from Golgi network to the basolateral cell membrane. As in our patient, it has been reported in the literature that subjects with benign pancreatic hyperenzymemia have also a Gilbert's syndrome. The relationship is still unclear. A proper diagnosis of Gullo's syndrome is important to aid clinician in the management of this children and to reassure family about the benignancy of the disease. The recognition of this syndrome may also prevent multiple and complex diagnostic evaluations or hospital admissions for children.

Benign Pancreatic Hyperenzymemia (Gullo Syndrome), Histamine Intolerance, and Carbohydrate Malabsorption

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.

Pancreatic morpho-functional imaging as a diagnostic approach for chronic asymptomatic pancreatic hyperenzymemia

BackgroundMagnetic resonance cholangio-pancreatography (MRCP) findings in people with chronic asymptomatic pancreatic hyperenzymemia (CAPH) have shifted the hypothesis that CAPH is always non-pathological. However, there have been no studies including both secretin-MRCP (S-MRCP) and endoscopic ultrasonography (EUS) to examine the pancreatic morphology in these subjects. AimTo prospectively assess the diagnostic approach for CAPH using both pancreatic EUS and S-MRCP. MethodsIn a case-control prospective study from January 2010 to December 2014, 68 consecutive subjects with CAPH were scheduled to undergo S-MRCP and EUS (CAPH group) in a tertiary care setting. In the same period, the EUS findings of this group were compared with 68 patients examined by EUS alone for submucosal lesions of the gastric fundus, matched for sex and age (control group). ResultsEUS detected pancreatic alterations in 60.3% of the CAPH group and 13.2% of controls (p<0.001). S-MRCP showed pancreatic alterations in 51.5% in the CAPH group. With the combined procedures, pancreatic abnormalities were detected in 63.3%. The diagnoses established by the two techniques were concordant in 51 (75%) of the 68 CAPH subjects; in the remaining 17 (25%) the two methods gave additional information. ConclusionsIn people with CAPH S-MRCP and EUS are both recommended in order to detect pancreatic abnormalities before this biochemical alteration is confirmed as benign CAPH, or Gullo's syndrome.

Healthy Chinese with benign pancreatic hyperenzymemia

Benign pancreatic hyperenzymemia, or Gullo's syndrome, is an uncommon syndrome characterized by a long-term increase of serum pancreatic enzyme in the absence of pancreatic diseases. It is primarily discovered incidentally and occurs in either sporadic or familial form. Herein, we report the first case of benign pancreatic hyperenzymemia in Taiwan. A 57-year-old Chinese male was incidentally noted with elevated serum amylase and lipase levels during a health check-up and was diagnosed with benign pancreatic hyperenzymemia using a series of image and serological tests. Although this is the first case of benign pancreatic hyperenzymemia in Taiwan, its prevalence may be underestimated due to the diagnostic difficulties. Correct diagnosis of this disease is important to avoid costly test duplication, unfounded anxieties, and multiple consultations.

Two rare cases of benign hyperlipasemia in children

Gullo's syndrome is a newly identified condition characterized by a chronic elevation of pancreatic amylase and/or lipase in the absence of pancreatic disease. Until now, only one case of benign isolated hyperlipasemia in children has been recorded. We describe two children with benign and not familial increase of serum lipase. Case 1: a six year old girl presented with occasional discovery of serum lipase elevation. Medical history was silent for pancreatic hyperenzymemia. The screening for possible causes for elevated lipase (genetic, autoimmune and infectious diseases) was normal. The serum lipase increased three fold over the upper limit (193 U/L; reference range 0-60 U/L), with daily fluctuation of values. Both ultrasound scan and magnetic resonance imaging were normal. The genetic mutation associated with chronic pancreatitis was negative. We followed up this patient for two years with blood tests every six months and she did not show any signs or symptoms of pancreatic disease, except for the high level of lipase serum. Case 2: an eight year old girl complained of nausea, vomiting and severe abdominal pain in the epigastric region after eating for the last two weeks. Full blood count, electrolytes, C-reactive protein, liver and renal function were normal. Serum lipase was 96 U/L (reference range 0-60 U/L). The screening for the possible causes of pancreatic disease was negative. Endoscopy of the upper gastrointestinal tract, ultrasound, computed tomography scan and magnetic resonance imaging were normal. One year after the presentation of the symptoms, the patient became asymptomatic although the level of serum lipase continued to be high.

Benign pancreatic hyperenzymemia or Gullo's syndrome

Benign pancreatic hyperenzymemia is a newly identified syndrome characterized by an abnormal increase in serum pancreatic enzymes in the absence of pancreatic disease. The hyperenzymemia can occur sporadically or in a familial form, and all of the pancreatic enzymes show elevations. Although the condition is persistent, the enzyme elevations fluctuate considerably, even temporarily returning to normal levels at times. In this review the main characteristics of this syndrome are described.