Simultaneous bilateral lung and pancreas transplantation in recipient with cystic fibrosis

J A Fridell1, T C Wozniak, J A Powelson

  • 1Indiana University School of Medicine, Indianapolis, Indiana 46202, USA. jfridell@iupui.edu

Insights

Simultaneous lung and pancreas transplants offer a safe solution for cystic fibrosis (CF) patients with multi-organ complications. This procedure improved glucose control and nutritional status, freeing patients from insulin and enzyme dependence.

Area of Science:

  • Transplantation Surgery
  • Pulmonology
  • Endocrinology

Background:

  • Cystic fibrosis (CF) is a genetic disorder causing multisystem disease, notably pulmonary failure and pancreatic insufficiency.
  • CF-related diabetes (CFRD) and exocrine insufficiency are major contributors to morbidity and mortality in CF patients.

Observation:

  • A 25-year-old male with CF presented with bronchiectasis, recurrent pneumonia, pancreatic exocrine insufficiency, and CFRD.
  • The patient had normal hepatic and renal function prior to transplantation.

Findings:

  • Simultaneous lung and pancreas transplantation was successfully performed using grafts from a single donor.
  • The patient experienced excellent recovery, with the pancreas allograft normalizing blood glucose and eliminating the need for insulin.
  • Enteric drainage of the pancreas allograft resolved the need for supplemental pancreatic enzymes, improving nutritional status.

Implications:

  • Simultaneous lung and pancreas transplantation is a viable and safe option for select CF patients with advanced disease.
  • This combined approach addresses both pulmonary and metabolic complications, significantly improving patient outcomes and quality of life.
Abstract

Related Concept Videos

Cystic Fibrosis: Pathogenesis01:23

Cystic Fibrosis: Pathogenesis

Cystic fibrosis (CF), an autosomal recessive disorder, significantly affects the function of exocrine glands. This genetically inherited disease is characterized by the production of thick and sticky mucus, which can severely affect various organs and systems in the body.
CF is primarily caused by a genetic mutation in a chromosome 7 gene coding for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. The most common gene mutation leading to CF is the ΔF508 mutation, but...
Cystic Fibrosis: Management01:24

Cystic Fibrosis: Management

Cystic fibrosis (CF) is an autosomal recessive disorder that predominantly affects individuals of Northern European descent, occurring at a rate of 1 in 3500. It is caused by a genetic mutation in a gene on chromosome 7, most commonly the ΔF508 mutation, that codes for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. This results in thicker mucus secretions and obstruction pathologies in multiple organs, including the lungs and sinuses.
Sinus disease and chronic sinusitis...
Chronic Pancreatitis II: Collaborative Care01:29

Chronic Pancreatitis II: Collaborative Care

The management of chronic pancreatitis is multifaceted, involving a comprehensive approach that includes thorough assessment, diagnostic testing, and a variety of management strategies.
Assessment:
Kidney Transplant II: Surgical Procedure01:26

Kidney Transplant II: Surgical Procedure

Preoperative ManagementThe primary goals of preoperative management in kidney transplantation are to optimize the patient’s metabolic state and prepare them for surgery through diet adjustments, necessary dialysis, and tailored medical treatment. This phase also involves comprehensive infection screening and patient education about the surgical procedure and postoperative care to improve outcomes and adherence.Medical ManagementA comprehensive evaluation is required for both the living donor...