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Wiener klinische Wochenschrift

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28.04.2021 | original article

Hereditary pancreatitis in childhood: course of disease and complications

verfasst von: Regina Prommer, Melanie Kienbauer, Simon Kargl, Prim. Univ.-Prof. Dr. Rainer Schöfl

Erschienen in: Wiener klinische Wochenschrift | Ausgabe 13-14/2021

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Summary

Background

Hereditary pancreatitis is rare. Pain therapy for juvenile symptom onset, child development and the risk of pancreatic carcinoma in adulthood must be considered.

Patients, material and methods

Data from a cohort of 11 patients with disease onset in childhood (< 16 years) were analyzed retrospectively. The gene encoding cationic trypsinogen (PRSS1), serine protease inhibitor Kazal type 1 (SPINK1) and cystic fibrosis transmembrane conductance regulator (CFTR) genes were investigated as genetic factors. Treatment concept and complications were registered. Prognosis, treatment success and quality of life were objectified using the chronic pancreatitis prognosis score and a standardized questionnaire (KIDSCREEN-10 index).

Results

The mean age of symptom onset was 7.5 ±4.2 years. The PRSS1 and SPINK1 mutations each occurred with 36.4%, 3 patients had a pancreas divisum and 2 a long common channel. The course of pancreatitis was obstructive in 90.9%. Exocrine pancreatic insufficiency occurred in seven patients so far (mean age 12.5 years). Stenting was performed in 72.7% and 18.2% needed pancreatic surgery. Currently the chronic prognosis score is on average 7.5 points, pain on numerical rating scale 0 (no pain). The mean KIDSCREEN‑T score of 66.9 confirms a very good quality of life.

Conclusion

Patients with genetically caused chronic pancreatitis are rare. Their care ranges from pain therapy in childhood and adolescence to questions concerning family planning and pancreatic cancer prevention from mid-adulthood onward. The disease is challenging for the interdisciplinary cooperation. We found the step-up strategy to be a good option for pain therapy. A national registry monitored by scientific societies with active recruitment for screening examinations will further improve and ensure care in the long term.

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Titel: Hereditary pancreatitis in childhood: course of disease and complications
verfasst von: Regina Prommer
Melanie Kienbauer
Simon Kargl
Prim. Univ.-Prof. Dr. Rainer Schöfl
Publikationsdatum: 28.04.2021
Verlag: Springer Vienna
Erschienen in: Wiener klinische Wochenschrift / Ausgabe 13-14/2021
Print ISSN: 0043-5325
Elektronische ISSN: 1613-7671
DOI: https://doi.org/10.1007/s00508-021-01869-0

Springer Medizin Österreich

Summary

Background

Patients, material and methods

Results

Conclusion

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