Skip to main content
main-content

Tipp

Weitere Artikel dieser Ausgabe durch Wischen aufrufen

Erschienen in: Wiener klinische Wochenschrift 13-14/2021

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

Einloggen, um Zugang zu erhalten
share
TEILEN

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.

Sie möchten Zugang zu diesem Inhalt erhalten? Dann informieren Sie sich jetzt über unsere Produkte:

Abo für kostenpflichtige Inhalte

Literatur
12.
Zurück zum Zitat Hidaka E, Yanagisawa A, Seki M, et al. High frequency of K‑ras mutations in biliary duct carcinomas of cases with a long common channel in the papilla of Vater. Cancer Res. 2000;60(3):522–4. PubMed Hidaka E, Yanagisawa A, Seki M, et al. High frequency of K‑ras mutations in biliary duct carcinomas of cases with a long common channel in the papilla of Vater. Cancer Res. 2000;60(3):522–4. PubMed
Metadaten
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