title: Combined heterotopic liver-pancreas transplantation as a curative treatment for liver cirrhosis and diabetes mellitus in cystic fibrosis.
abstract: Cystic fibrosis (CF) is an inherited disease with a defect in epithelial chloride transport that results in a multisystem disease. Although pulmonary disease remains the primary cause of morbidity and mortality, focal biliary cirrhosis and portal hypertension may develop in up to 8% of these patients. Liver transplantation (TX) is an accepted therapy and shows good results. We report on a patient with cystic fibrosis homozygous for the most common CFTR mutation delta F 508 who received a combined heterotopic liver and pancreas transplantation at the age of 18 yr. He suffered from CFRD, which untypically required high doses of insulin. In addition, the patient had pulmonary complications, was chronically colonized with multiresistant Pseudomonas aeruginosa (MBL) and had an allergic bronchopulmonary aspergillosis (ABPA). The patient remained in stable health for 54 months post-TX and was able to live a nearly normal life. With a follow-up of five yr, the function of the liver and pancreas allografts was excellent. However, and sadly, his pulmonary function continued to deteriorate from progression of his CF, and he died of respiratory failure due to a severe pneumonia and septicemia at the age of 23 yr and five months.
authors.affiliation: Department of Women and Child Health, Hospital for Children and Adolescents, University Hospital Leipzig, Leipzig, Germany.
authors.forename: Constance
authors.initials: C
authors.lastname: Henn
authors.forename: Thomas
authors.initials: T
authors.lastname: Kapellen
authors.forename: Freerk
authors.initials: F
authors.lastname: Prenzel
authors.forename: Manuela
authors.initials: M
authors.lastname: Siekmeyer
authors.forename: Hans-Michael
authors.initials: HM
authors.lastname: Hau
authors.forename: Wieland
authors.initials: W
authors.lastname: Kiess
authors.forename: Michael
authors.initials: M
authors.lastname: Bartels
mesh_terms: D001229:Aspergillosis, Allergic Bronchopulmonary / Q000150:complications; D003550:Cystic Fibrosis / Q000150:complications / Q000628:therapy*; D048909:Diabetes Complications / Q000628:therapy; D003920:Diabetes Mellitus / Q000628:therapy*; D018450:Disease Progression; D017809:Fatal Outcome; D006085:Graft Survival; D006801:Humans; D016031:Liver Transplantation / Q000379:methods*; D008171:Lung Diseases / Q000382:microbiology; D008297:Male; D016035:Pancreas Transplantation / Q000379:methods*; D011552:Pseudomonas Infections / Q000150:complications / Q000628:therapy; D011550:Pseudomonas aeruginosa; D055815:Young Adult
keywords: CF-related diabetes mellitus; combined liver-pancreas transplantation; cystic fibrosis; heterotopic graft placement; liver failure