Total pancreatectomy with islet cell autotransplantation in a 2-year-old child with hereditary pancreatitis due to a PRSS1 mutation.
autotransplantation
clinical decision-making
clinical research/practice
genetics
islet isolation
islet transplantation
pediatrics
Journal
American journal of transplantation : official journal of the American Society of Transplantation and the American Society of Transplant Surgeons
ISSN: 1600-6143
Titre abrégé: Am J Transplant
Pays: United States
ID NLM: 100968638
Informations de publication
Date de publication:
11 2021
11 2021
Historique:
revised:
02
06
2021
received:
04
05
2021
accepted:
13
06
2021
pubmed:
17
6
2021
medline:
12
11
2021
entrez:
16
6
2021
Statut:
ppublish
Résumé
Acute recurrent and chronic pancreatitis in children carries high morbidity and burden. Compared to adults, ~75% of the cases of chronic pancreatitis in children are associated with underlying genetic mutations. The decision to intervene and the optimal timing poses unique challenges. Total pancreatectomy and islet cell autotransplantation (TPIAT) provides definitive therapy to relieve pain and improve quality of life while minimizing the risk of pancreatogenic diabetes. Substantial clinical data are available for adults; however, information on clinical outcomes in children remains scarce, particularly for very young children. Herein, we present an unusual, complex case of a 2-year-old child that underwent a successful TPIAT due to hereditary pancreatitis with an underlying mutation in PRSS1 gene, complicated by unremitting pancreatic ascites, hemorrhage, and sepsis. This is the youngest case to be reported in the literature. We provide a comprehensive report of the course and procedures implemented in this patient to guide other teams when considering these extraordinary measures in similar cases.
Identifiants
pubmed: 34132023
doi: 10.1111/ajt.16723
pii: S1600-6135(22)08813-X
doi:
Substances chimiques
PRSS1 protein, human
EC 3.4.21.4
Trypsin
EC 3.4.21.4
Types de publication
Case Reports
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Pagination
3790-3793Informations de copyright
© 2021 The American Society of Transplantation and the American Society of Transplant Surgeons.
Références
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