Combined heterotopic liver-pancreas transplantation as a curative treatment for liver cirrhosis and diabetes mellitus in cystic fibrosis.

[allergic bronchopulmonary aspergillosis]

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 .

Diseases
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Diseases presenting "untypically required high doses" symptom

allergic bronchopulmonary aspergillosis

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