UNLABELLED: Cystic fibrosis (CF) is an inherited disorder that presents in childhood as a multisystem disease. Pulmonary failure and pancreatic insufficiency, including CF related diabetes (CFRD) and exocrine insufficiency, are common complications of this disease. In this report we review the first three simultaneous lung and pancreas transplantations in CF patients with diabetes. METHODS: All three CF patients presented for evaluation for lung transplantation and had pancreatic insufficiency requiring enzyme supplementation and CFRD requiring insulin. All were severely malnourished and required nutritional supplementation. SURGICAL TECHNIQUE: In each case, the allografts were procured from a single cadaveric donor. Bilateral lung transplantation was performed first using two separate thoracic incisions. The pancreas transplant was performed with systemic venous drainage and enteric exocrine drainage. RESULTS: The pancreas allografts all functioned normally with normoglycemia independent of insulin. As a result of the enteric drainage of the pancreas allograft, supplemental pancreatic enzymes were no longer required. Despite several complications detailed in the manuscript, all three remain independent of supplemental oxygen, insulin and pancreatic enzyme replacement at 4, 6 and 14 months of follow-up. CONCLUSION: Simultaneous lung and pancreas transplantation in patients with CF can be performed successfully and provides the advantages of normoglycemia and improves nutrition for patients requiring lung transplantation.
UNLABELLED: Cystic fibrosis (CF) is an inherited disorder that presents in childhood as a multisystem disease. Pulmonary failure and pancreatic insufficiency, including CF related diabetes (CFRD) and exocrine insufficiency, are common complications of this disease. In this report we review the first three simultaneous lung and pancreas transplantations in CFpatients with diabetes. METHODS: All three CFpatients presented for evaluation for lung transplantation and had pancreatic insufficiency requiring enzyme supplementation and CFRD requiring insulin. All were severely malnourished and required nutritional supplementation. SURGICAL TECHNIQUE: In each case, the allografts were procured from a single cadaveric donor. Bilateral lung transplantation was performed first using two separate thoracic incisions. The pancreas transplant was performed with systemic venous drainage and enteric exocrine drainage. RESULTS: The pancreas allografts all functioned normally with normoglycemia independent of insulin. As a result of the enteric drainage of the pancreas allograft, supplemental pancreatic enzymes were no longer required. Despite several complications detailed in the manuscript, all three remain independent of supplemental oxygen, insulin and pancreatic enzyme replacement at 4, 6 and 14 months of follow-up. CONCLUSION: Simultaneous lung and pancreas transplantation in patients with CF can be performed successfully and provides the advantages of normoglycemia and improves nutrition for patients requiring lung transplantation.