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Combined heterotopic liver–pancreas transplantation as a curative treatment for liver cirrhosis and diabetes mellitus in cystic fibrosis
Author(s) -
Henn Constance,
Kapellen Thomas,
Prenzel Freerk,
Siekmeyer Manuela,
Hau HansMichael,
Kiess Wieland,
Bartels Michael
Publication year - 2014
Publication title -
pediatric transplantation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.457
H-Index - 69
eISSN - 1399-3046
pISSN - 1397-3142
DOI - 10.1111/petr.12157
Subject(s) - medicine , cystic fibrosis , cirrhosis , allergic bronchopulmonary aspergillosis , gastroenterology , liver transplantation , pulmonary function testing , transplantation , diabetes mellitus , pneumonia , liver function , pancreatic disease , liver disease , surgery , pancreas , immunology , immunoglobulin e , antibody , endocrinology
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.