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Incidence, Prevalence, Etiology, and Prognosis of First-Time Chronic Pancreatitis in Young Patients: A Nationwide Cohort Study

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Abstract

Background/Aims

Publications on etiology of chronic pancreatitis (CP) are infrequent. Etiologies today encompass genetic disorders. We wanted to describe etiologies of today and identify patients with genetic disorders like hereditary pancreatitis (HP), mutations in Serine Protease Inhibitor Kazal type1 (SPINK1), and the Cystic Fibrosis Transmembrane Conductance Regulator gene (CFTR) among patients formerly considered to have idiopathic CP.

Methods

Data on patients diagnosed with first-time CP < 30 years of age in Denmark identified in the Danish National Registry of Patients were retrieved. Patients previously considered to have idiopathic pancreatitis were offered genetic counseling and evaluation for HP, SPINK1, and CFTR mutations.

Results

In the period 1980–2004, 580 patients < 30 years of age presented with CP, the standardized prevalence ratio of CP increased from 11.7 per 100,000 person years in 1980–1984 to 17.0 per 100,000 in 2000–2004 (p < 0.001). The odds ratio (OR) having gallstone-related CP increased in the latter time period, especially in women, that of alcohol-induced CP decreased over time. OR having idiopathic CP increased in the latter period; 50% of patients with idiopathic pancreatitis accepted genetic reevaluation; 28 patients had a genetic mutation that totally or partly could explain their pancreatitis, nine of these had two, and 11 patients had HP.

Conclusion

The prevalence of CP, especially in women, increased over time. Genetic causes that partly or totally could explain the CP were found in 54.90% (95% CI (40.45–68.62)) of those with idiopathic CP, as a minimum estimation 1.9% (95% CI (1.00–3.47)) of the total cohort had HP.

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Acknowledgments

We thank the patients participating in the study and secretaries who have been helpful in scrutinizing hospital files. A special thanks to our laboratory technicians Henriette Hansen, Steen Gregersen, and Lene Byriel and molecular biologist Anders Aamann Rasmussen who have set up the CFTR analysis.

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Authors and Affiliations

  1. Department of Medical Gastroenterology S, Odense University Hospital, University of Southern Denmark, Sdr. Boulevard 29, 5000, Odense C, Denmark

    Maiken Joergensen & Ove B. Schaffalitzky de Muckadell

  2. Department of Biochemistry, Pharmacology and Genetics, Odense University Hospital, University of Southern Denmark, Sdr. Boulevard 29, 5000, Odense C, Denmark

    Klaus Brusgaard & Anne-Marie Gerdes

  3. Department of Clinical Genetics, Vejle Hospital, University of Southern Denmark, Kabbeltoft 25, 7100, Vejle, Denmark

    Dorthe Gylling Crüger

Authors
  1. Maiken Joergensen
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  2. Klaus Brusgaard
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  3. Dorthe Gylling Crüger
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  4. Anne-Marie Gerdes
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  5. Ove B. Schaffalitzky de Muckadell
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Corresponding author

Correspondence to Maiken Joergensen.

Additional information

The study was supported by grants from Kontorchef Svend Coles Frederiksen og hustrus fond, director Jacob Madsens & og hustru Olga Madsens Fond, Inge og Jørgen Larsens Mindelegat and Thora og Viggo Grove’s Mindelegat.

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Joergensen, M., Brusgaard, K., Crüger, D.G. et al. Incidence, Prevalence, Etiology, and Prognosis of First-Time Chronic Pancreatitis in Young Patients: A Nationwide Cohort Study. Dig Dis Sci 55, 2988–2998 (2010). https://doi.org/10.1007/s10620-009-1118-4

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  • DOI: https://doi.org/10.1007/s10620-009-1118-4

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