Long-Term Outcomes of Total Pancreatectomy and Islet Auto Transplantation for Hereditary/Genetic Pancreatitis

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• 作者：Srinath Chinnakotla ; MD^a ; ^b ; ^{chinni@umn.edu" class="auth_mail} ; David M. Radosevich ; RN ; PhD^a ; Ty B. Dunn ; MD ; FACS^a ; Melena D. Bellin ; MD^b ; ^c ; Martin L. Freeman ; MD^d ; Sarah J. Schwarzenberg ; MD^b ; A.N. Balamurugan ; PhD^a ; Josh Wilhelm ; MS^c ; Barbara Bland ; MS ; RN^a ; Selwyn M. Vickers ; MD ; FACS^a ; Gregory J. Beilman ; MD ; FACS^a ; David E.R. Sutherland ; MD ; PhD^a ; ^c ; Timothy L. Pruett ; MD ; FACS^a
• 关键词：CFTR ; cystic fibrosis transmembrane conductance regulator ; CL ; confidence limits ; CP ; chronic pancreatitis ; HGP ; hereditary/genetic pancreatitis ; HRQoL ; health-related quality of life ; IAT ; islet autogeneic transplantation ; IEQ ; islet cell equivalent ; MCS ; Mental Component Summary ; OR ; odds ratio ; PCS ; Physical Component Summary ; PRSS1 ; protease trypsin 1 ; SPINK1 ; serine protease inhibitor Kazal type 1 ; TP ; total pancreatectomy
• 刊名：Journal of the American College of Surgeons
• 出版年：April, 2014
• 年：2014
• 卷：218
• 期：4
• 页码：530-543
• 全文大小：1793 K

文摘

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Background

Chronic pancreatitis is a debilitating disease resulting from many causes. The subset with hereditary/genetic pancreatitis (HGP) not only has chronic pain, but also an increased risk for pancreatic cancer. Long-term outcomes of total pancreatectomy (TP) and islet autogeneic transplantation (IAT) for chronic pancreatitis due to HGP are not clear.

Study Design

We reviewed a prospectively maintained database of 484 TP-IATs from 1977 to 2012 at a single center. The outcomes (eg, pain relief, narcotic use, 尾-cell function, health-related quality of life measures) of patients who received TP-IAT for HGP (protease trypsin 1, n聽= 38; serine protease inhibitor Kazal type 1, n聽= 9; cystic fibrosis transmembrane conductance regulator, n聽= 14; and familial, n聽= 19) were evaluated and compared with those with non鈭抙ereditary/nongenetic causes.

Results

All 80 patients with HGP were narcotic dependent and failed endoscopic management or direct pancreatic surgery. Post TP-IAT, 90% of the patients were pancreatitis pain free with sustained pain relief; >65% had partial or full 尾-cell function. Compared with nonhereditary causes, HGP patients were younger (22 years old vs 38 years old; p 鈮?0.001), had pancreatitis pain of longer duration (11.6 卤 1.1 years vs 9.0 卤 0.4 years; p聽= 0.016), had a higher pancreas fibrosis score (7 卤 0.2 vs 4.8 卤 0.1; p 鈮?0.001), and trended toward lower islet yield (3,435 卤 361 islet cell equivalent vs 3,850 卤 128 islet cell equivalent; p聽= 0.28). Using multivariate logistic regression, patients with non-HGP causes (p聽= 0.019); lower severity of pancreas fibrosis (p < 0.001); shorter duration of years with pancreatitis (p聽= 0.008); and higher transplant islet cell equivalent per kilogram body weight (p 鈮?0.001) were more likely to achieve insulin independence (p < 0.001). There was a significant improvement in health-related quality of life from baseline by RAND 36-Item Short Form Health Survey and in physical and mental component health-related quality of life scores (p < 0.001). None of the patients in the entire cohort had cancer of pancreatic origin in the liver or elsewhere develop during 2,936 person-years of follow-up.

Conclusions

Total pancreatectomy and IAT in patients with chronic pancreatitis due to HGP cause provide long-term pain relief (90%) and preservation of 尾-cell function. Patients with chronic painful pancreatitis due to HGP with a high lifetime risk of pancreatic cancer should be considered earlier for TP-IAT before pancreatic inflammation results in a higher degree of pancreatic fibrosis and islet cell function loss.