Combined lung-liver-pancreas transplantation in a recipient with cystic fibrosis

Barbas, Andrew S.; Dib, Martin J.; Al‐Adra, David P.; Goldaracena, Nicolás; Waddell, Thomas K.; Keshavjee, Shaf; Selzner, Nazia; Chaparro, Cecilia; Cattral, Mark S.

doi:10.1016/j.jcf.2017.05.004

Cited by 19 publications

(11 citation statements)

References 23 publications

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“…As hydration and mucin viscosity in the airways is dependent on the ionic equilibrium across the epithelial barrier, lack of CFTR function reduces airway mucus fluidity, trapping inhaled bacteria [6]. Although CFTR mutations also have negative effects on the function of other organs, such as the pancreas, CF patients receiving nutritional supplementation are able to manage these limitations [7]. However, the inflammation and chronic infection associated with CF lung disease limits quality of life and ultimately longevity [8].…”

Section: Gram-negative Bacteria and Lung Inflammation: Cystic Fibrosismentioning

confidence: 99%

Pseudomonas aeruginosa and Klebsiella pneumoniae Adaptation to Innate Immune Clearance Mechanisms in the Lung

Riquelme¹,

Ahn²,

Prince³

2018

J Innate Immun

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Many different species of gram-negative bacteria are associated with infection in the lung, causing exacerbations of chronic obstructive pulmonary disease, cystic fibrosis (CF), and ventilator-associated pneumonias. These airway pathogens must adapt to common host clearance mechanisms that include killing by antimicrobial peptides, antibiotics, oxidative stress, and phagocytosis by leukocytes. Bacterial adaptation to the host is often evident phenotypically, with increased extracellular polysaccharide production characteristic of some biofilm-associated organisms. Given the relatively limited repertoire of bacterial strategies to elude airway defenses, it seems likely that organisms sharing the same ecological niche might also share common strategies to persistently infect the lung. In this review, we will highlight some of the major factors responsible for the adaptation of Pseudomonas aeruginosa to the lung, addressing how growth in biofilms enables persistent infection, relevant to, but not limited to, the pathogenesis of infection in CF. In contrast, we will discuss how carbapenem-resistant Klebsiella pneumoniae evade immune clearance, an organism often associated with ventilator-associated pneumonia and health-care-acquired pneumonias, but not a typical pathogen in CF.

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Section: Gram-negative Bacteria and Lung Inflammation: Cystic Fibrosismentioning

confidence: 99%

Pseudomonas aeruginosa and Klebsiella pneumoniae Adaptation to Innate Immune Clearance Mechanisms in the Lung

Riquelme¹,

Ahn²,

Prince³

2018

J Innate Immun

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“…There is 1 case report in the literature of a 19-year-old male undergoing simultaneous lung-liver-pancreas transplantation in Toronto, Canada. (60) At 1 year of follow-up, he continued to do well.…”

Section: Simultaneous Liver-lung Transplantation and Liver Transplantmentioning

confidence: 89%

“…There was an improvement in body mass index and nutritional status in the patients who completed the SLPT. There is 1 case report in the literature of a 19‐year‐old male undergoing simultaneous lung‐liver‐pancreas transplantation in Toronto, Canada . At 1 year of follow‐up, he continued to do well.…”

Section: Management Of End‐stage Organ Failure With Transplantationmentioning

confidence: 99%

Cystic Fibrosis–Associated Liver Disease in Lung Transplant Recipients

Mallea¹,

Bolan²,

Cortese

et al. 2019

Liver Transpl

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Cystic fibrosis (CF) is an autosomal recessive disease characterized by mutations in the gene that encodes for the cystic fibrosis transmembrane conductance regulator protein (CFTR). CFTR gene mutations manifest as epithelial cell dysfunction in the airways, biliary tract, pancreas, gut, sweat glands, paranasal sinuses, and genitourinary tract. The clinical manifestations of this dysfunction include respiratory tract infections, bronchiectasis, pancreatic insufficiency, malabsorption, intestinal obstruction, liver disease, and male infertility. The liver disease manifestations of CF can include biliary disease, multilobular cirrhosis, and portal hypertension with and without cirrhosis. Pulmonary disease is the main cause for morbidity and mortality in individuals with CF, and according to the International Society for Heart and Lung Transplantation, CF is the third most common indication for lung transplantation in adults, accounting for 16% of procedures performed. The survival after lung transplantation in individuals with CF continues to improve and is now the highest among end‐stage lung diseases requiring transplant. The survival rate at 10 years is close to 50%. Given the potential presence of liver disease in CF patients undergoing an evaluation for lung transplantation and in lung transplant recipients, it is important to understand the manifestations of liver disease in CF patients and the recommended workup and follow‐up. This review aims to discuss the current literature and provide guidance in the management of these patients.

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“…255 Otros tratamientos descritos en la literatura, aunque en casos extremadamente seleccionados es el trasplante pulmonar y pancreático en pacientes con FQ. 256 Al igual que con la DM, nuestro estudio no encuentra una relación con una menor supervivencia de los pacientes afectos con IPE (p = 0,06).…”

Section: Características Demográficasunclassified

El trasplante pulmonar en la fibrosis quística y la influencia de la circulación extracorpórea

Deu

Romero

Román

et al. 2018

Archivos de Bronconeumología

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Combined lung-liver-pancreas transplantation in a recipient with cystic fibrosis

Cited by 19 publications

References 23 publications

<b><i>Pseudomonas aeruginosa</i></b> and <b><i>Klebsiella</i></b> <b><i>pneumoniae</i></b> Adaptation to Innate Immune Clearance Mechanisms in the Lung

<b><i>Pseudomonas aeruginosa</i></b> and <b><i>Klebsiella</i></b> <b><i>pneumoniae</i></b> Adaptation to Innate Immune Clearance Mechanisms in the Lung

Cystic Fibrosis–Associated Liver Disease in Lung Transplant Recipients

El trasplante pulmonar en la fibrosis quística y la influencia de la circulación extracorpórea

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