Use of hyperglycemic clamp to assess pancreatectomy and islet cell autotransplant in patient with heterotaxy syndrome and dorsal pancreas agenesis leading to chronic pancreatitis. Issue 12 (23rd June 2020)

• Record Type: Journal Article
• Authors: De Paep, Diedert L.; Gillard, Pieter; Ling, Zhidong; Verbeke, Hilde; Maleux, Geert; Vandecaveye, Vincent; Debaveye, Yves; Keymeulen, Bart; van der Merwe, Schalk; Pipeleers, Daniel; Pirenne, Jacques; van Malenstein, Hannah; Jacobs‐Tulleneers‐Thevissen, Daniel
• Is Part Of: American journal of transplantation. Volume 20:Issue 12(2020)
• Page Start: 3662
• Page End: 3666
• Publication Date: 2020-06-23
• Subjects: anatomy -- autotransplantation -- clinical research/practice -- diabetes -- endocrinology/diabetology -- islet isolation -- islet transplantation; Transplantation of organs, tissues, etc -- Periodicals; 617.95
• DOI: 10.1111/ajt.16084
• Languages: English
• ISSNs: 1600-6135

Abstract

Abstract : Patients with heterotaxy syndrome (HS) can present with an associated complete dorsal pancreas agenesis (DPA). They are considered to be at increased risk for developing diabetes due to a reduced functional beta cell mass (FBM) as well as for chronic pancreatitis leading to unmanageable pain. We report the case of a young woman with chronic pancreatitis due to HS and associated DPA. She presented with a severe persisting upper abdominal pain refractory to nonsurgical treatment. Unlike in previously reported cases, she had a high FBM (ie, 150% of normoglycemic controls) as determined by hyperglycemic clamp. She underwent a total pancreatectomy followed within 24 hours by an intraportal autologous islet cell transplant containing 4 × 10 6 beta cells (4700 islet equivalent)/kg body weight. After surgery, the pain resolved, eliminating the need for analgesics. The intraportal implant established an adequate FBM (72% of controls at posttransplant month 2), achieving glycemic control without need for insulin administration. A hyperglycemic clamp can assess the utility and efficacy of an intraportal islet cell autotransplant following total pancreatectomy in patients with HS and complete DPA. Abstract : Total pancreatectomy followed by islet autotransplantation is a treatment option for chronic pancreatitis due to heterotaxy syndrome and dorsal pancreas agenesis.