Familial chylomicronemia syndrome related chronic pancreatitis: a single-center study

Hepatobiliary Pancreat Dis Int. 2014 Apr;13(2):209-14. doi: 10.1016/s1499-3872(14)60033-3.

Abstract

Background: Hypertriglyceridemia induces acute recurrent pancreatitis, but its role in the etiology of chronic pancreatitis (CP) is controversial. This study aimed to evaluate the clinical, laboratory and radiological findings of 7 patients with CP due to type 1 hyperlipidemia compared to CP patients with other or undefined etiological factors.

Methods: We retrospectively analyzed the clinical, laboratory and radiological findings of 7 CP patients with type 1 hyperlipidemia compared to CP patients without hypertriglyceridemia. These 7 patients had multiple episodes of acute pancreatitis and had features of CP on abdominal CT, endoscopic retrograde cholangiopancreatography and/or endoscopic ultrasonography.

Results: All CP patients were classified into two groups: a group with type 1 hyperlipidemia (n=7) and a group with other etiologies (n=58). The mean triglyceride level was 2323+/-894 mg/dL in the first group. Age at the diagnosis of CP in the first group was significantly younger than that in the second group (16.5+/-5.9 vs 48.3+/-13.5, P<0.001). The number of episodes of acute pancreatitis in the first group was significantly higher than that in the second group (15.0+/-6.8 vs 4.0+/-4.6, P=0.011). The number of splenic vein thrombosis in the first group was significantly higher than that in the second group (4/7 vs 9/58, P=0.025). Logistic regression analysis found that younger age was an independent predictor of CP due to hypertriglyceridemia (r=0.418, P=0.000).

Conclusions: Type 1 hyperlipidemia appears to be an etiological factor even for a minority of patients with CP. It manifests at a younger age, and the course of the disease might be severe.

Publication types

  • Comparative Study

MeSH terms

  • Adolescent
  • Adult
  • Age Factors
  • Biomarkers / blood
  • Chi-Square Distribution
  • Child
  • Cholangiopancreatography, Endoscopic Retrograde
  • Endosonography
  • Female
  • Humans
  • Hyperlipoproteinemia Type I / blood
  • Hyperlipoproteinemia Type I / complications*
  • Hyperlipoproteinemia Type I / diagnosis
  • Logistic Models
  • Male
  • Middle Aged
  • Odds Ratio
  • Pancreatitis, Chronic / diagnosis
  • Pancreatitis, Chronic / etiology*
  • Predictive Value of Tests
  • Prognosis
  • Retrospective Studies
  • Risk Factors
  • Severity of Illness Index
  • Tomography, X-Ray Computed
  • Triglycerides / blood
  • Turkey
  • Young Adult

Substances

  • Biomarkers
  • Triglycerides