In:
Pediatric Transplantation, Wiley, Vol. 18, No. 1 ( 2014-02)
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.
Type of Medium:
Online Resource
ISSN:
1397-3142
,
1399-3046
DOI:
10.1111/petr.2013.18.issue-1
Language:
English
Publisher:
Wiley
Publication Date:
2014
detail.hit.zdb_id:
2008614-3