scholarly journals The use of elexacaftor/tezacaftor/ivacaftor in patients with cystic fibrosis post-liver transplant: a case series

2021 ◽  
Author(s):  
Hunter Ragan ◽  
Elizabeth Autry ◽  
Taryn Bomersback ◽  
Jennifer Hewlett ◽  
Lauren Kormelink ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Liver Transplantation ◽  
Wide Spectrum ◽  
Case Series ◽  
Retrospective Case ◽  
Clinical Benefits ◽  
Tacrolimus Trough Concentration ◽  
Life Improvement ◽  
Immunosuppressant Medications ◽  
Related Liver Disease

Introduction Cystic fibrosis (CF) related liver disease (CFLD) manifests as a wide spectrum of hepatobiliary disease and can progress to need liver transplantation. Elexacaftor/tezacaftor/ivacaftor (elx/tez/iva) is a cystic fibrosis transmembrane conductance regulator (CFTR) modulator which has superior efficacy compared to previously approved modulators. Use of elx/tez/iva, should be approached with caution in individuals with CFLD or following liver transplantation due to possible increases in LFTs and drug-drug interactions with several immunosuppressant medications. Objective The purpose of this case series was to explore if the use of elx/tez/iva is safe and tolerable in patients with CF post-liver transplantation. Methods A retrospective case series including patients prescribed elx/tez/iva following liver transplantation and an immunosuppressive regimen consisting of drug therapy metabolized by P-glycoprotein was completed. Results Ten patients at six CF centers with a median age of 22.1 years (range 14-43.4 years) and median time from transplant of 6.9 years (range 0.6-22 years) were included. Most patients (8, 80%) received a reduced or full dose of elx/tez/iva for a mean duration of 10.4 months (range 7-12 months). Fluctuations in LFTs occurred in all patients (10, 100%) and led to therapy discontinuation in two patients (20%). Elx/tez/iva initiation resulted in elevations in tacrolimus trough concentration in 7 patients (70%). Most patients who tolerated elx/tez/iva had symptomatic and quality of life improvement, increased body-mass-index, and maintained or improved lung function. Conclusion Initiation of elx/tez/iva in patients with CF who received a liver transplantation may be safe with clinical benefits.

Pregnancy after liver transplantation. How safe? A retrospective case-series study in a large tertiary hospital

2015 ◽  
Vol 29 (13) ◽  
pp. 2120-2124 ◽  
Author(s):  
Chrysoula Margioula-Siarkou ◽  
Ioannis Kalogiannidis ◽  
Stamatios Petousis ◽  
Constantin Kubanangidi ◽  
Ioannis Fouzas ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Case Series ◽  
Tertiary Hospital ◽  
Retrospective Case ◽  
Retrospective Case Series ◽  
Case Series Study ◽  
Series Study

30 Liver transplantation for cystic fibrosis-related liver disease

2003 ◽  
Vol 35 ◽  
pp. A10
Author(s):  
M.L. Melzi ◽  
A. Callegaro ◽  
R. Adam ◽  
M.G. Valsecchi ◽  
B.M. Assael ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Liver Transplantation ◽  
Liver Disease ◽  
Related Liver Disease

scholarly journals Development of bacterial resistance during treatment with topical gentamicin for chronic rhinosinusitis in patients with cystic fibrosis and primary ciliary dyskinesis. Retrospective case series

2020 ◽  
Vol 74 (2) ◽  
pp. 1-8 ◽  
Author(s):  
Marta Kisiel ◽  
Isabella Sjölander ◽  
Agnes Klar ◽  
Monika Asplund Stenkvist ◽  
Göran Laurell
Keyword(s):  
Cystic Fibrosis ◽  
Chronic Rhinosinusitis ◽  
Nasal Spray ◽  
Bacterial Resistance ◽  
Bacterial Species ◽  
Case Series ◽  
Retrospective Case ◽  
Pre Treatment ◽  
Ciliary Dyskinesia ◽  
Microbiological Data

Background: The management of chronic rhinosinusitis (CRS) in patients with cystic fibrosis (CF) and primary ciliary dyskinesia (PCD) is still a challenge. At our institution we have used gentamycin nasal spray, extemporaneously produced, for prophylactic treatment of moderate-to-severe CRS. The aim of this study was to investigate the gentamycin susceptibility of bacteria in sputum samples in CF and PCD patients treated for CRS. Methodology: Patients with CF and PCD who were prescribed gentamycin nasal spray for CRS and had sputum bacterial cultures taken pre-treatment and followed-up at least once after ≥6 months were retrospectively included. Microbiological data were descriptively analysed in terms of bacterial species and resistance to gentamycin. Results: A case series of 17 CF and 12 PCD patients passed the inclusion criteria. Of those cases, three (18%) CF patients and one (8%) PCD patient developed resistance to gentamycin during treatment with gentamycin nasal spray. In all four cases, the resistant bacterial isolates were <i>P. aeruginosa</i>. Additionally, two CF patients already had <i>P. aeruginosa </i> isolates resistant to gentamycin in the pre-treatment culture. In further two CF patients, the multi-resistant <i>Burgdorferi cepacia </i>complex, including gentamycin resistance, was identified. <i>P. aeruginosa </i> and <i>S. aureus </i> in CF and <i>P. aeruginosa</i> and <i>H. influenza </i> in PCD were the predominant bacterial species. Conclusions: The study showed that there was moderate incidence of gentamycin resistance in CF and PCD patients at our institution. However, further prospective studies are needed to confirm the outcomes.

scholarly journals Clinical significance in non-cystic fibrosis bronchiectasis followed in a real practice

2013 ◽  
Vol 26 (4) ◽  
pp. 895-904
Author(s):  
Newton Santos de Faria Júnior ◽  
Amilcar Marcelo Bigatão ◽  
Sérgio Ricardo Santos ◽  
Fernando Sérgio Studart Leitão Filho ◽  
José Roberto Jardim ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Breathing Pattern ◽  
Functional Characterization ◽  
Case Series ◽  
Lung Parenchyma ◽  
Adult Patients ◽  
Retrospective Case ◽  
Chronic Disorder ◽  
Case Series Study ◽  
Real Practice

INTRODUCTION: Bronchiectasis is a chronic disorder characterized by permanent dilation of the bronchi and bronchioles accompanied by inflammatory changes in the walls of these structures and adjacent lung parenchyma. OBJECTIVE: The aim of the present study was to perform a clinical and functional characterization of adult patients with non-cystic fibrosis bronchiectasis. METHODS: A clinical, descriptive, retrospective, case-series study was carried out involving 232 patients with non-cystic fibrosis bronchiectasis treated at a lung ambulatory between 2004 and 2012. RESULTS: The sample consisted of 232 patients (134 females; mean age: 52.9 years ± 17.7; body mass index: 23.5 kg/m² ± 4.4). The predominant symptoms were cough (91.4%), expectoration (85.8%) and dyspnea (76.3%). The majority of cases were of a non-tuberculosis etiology (64.7%). Regarding lung function, the obstructive breathing pattern was predominant (43.5%). The most common comorbidities were of a cardiovascular origin (51.0%). CONCLUSIONS: Adult patients with non-cystic fibrosis bronchiectasis (mainly post-infection or post-tuberculosis in origin) are characterized by a low educational level, excessive cough, sputum, dyspnea, muscle fatigue, an obstructive breathing pattern with frequent hypoxemia and multiple comorbidities, mainly of a cardiovascular origin. However, our patients have a low index of exacerbations and hospitalizations that can be assigned to a clinical protocol for monitoring.

scholarly journals P012 Preliminary experience of the use of oral posaconazole and terbinafine to treat lomentospora prolificans and scedosporium apiospermum in children with cystic fibrosis

2019 ◽  
Vol 104 (7) ◽  
pp. e2.15-e2
Author(s):  
Sian Bentley ◽  
IM Balfour-Lynn ◽  
SB Carr
Keyword(s):  
Cystic Fibrosis ◽  
Lung Function ◽  
Case Series ◽  
Term Treatment ◽  
Scedosporium Apiospermum ◽  
Retrospective Case ◽  
Positive Clinical Effect ◽  
Long Term Treatment ◽  
Pseudomonas Aeruginosa Infection ◽  
Nutritional Complications

BackgroundItraconazole and voriconazole are the drugs of choice for Lomentospora prolificans and Scedosporium apiospermum. Posaconazole, is often substituted when there is intolerance or lack of efficacy to first line agents. Terbinafine, an allylamine antifungal, is recommended with an azole for the treatment of L.prolificans, though there is no published use of this combination in children with cystic fibrosis (CF).AimTo evaluate the safety, tolerability and efficacy of this regimen in CF children.MethodsRetrospective case note review of CF children receiving terbinafine and posaconazole, from Nov 2015 to Nov 2016. Children were identified from pharmacy records and clinical data collected from case notes and laboratory records.ResultsThere were 4 children (all girls), median age 15 years (range 10–16), with a median FEV1% predicted of 70.5% (range 55–88%). 2 children chronically isolated L.prolificans, 2 isolated S.apiospermum. 3 also had CF related diabetes and chronic Pseudomonas aeruginosa infection. 1 child received treatment for 6 weeks. 3 children are taking long- term treatment (median 50 weeks; range 35–59). 2 children improved FEV1% predicted with treatment by 14% and 15%; one was stable. Importantly the trend graphs for lung function in these 3 children appear to stabilise post initiation of treatment. One child did not improve her lung function but also had recurrent MRSA infections and significant nutritional complications. No adverse effects from the combination were reported. Posaconazole levels were therapeutic (>1 mg/l) in all children (range 1.22–3.85 mg/l). Terbinafine levels were not measured.ConclusionIn this small case series, combination treatment with posaconazole and terbinafine was well tolerated and a positive clinical effect on lung function was evident. This is the first report on the use of this regimen for this indication in CF children and we will continue to use it, whilst gathering safety and efficacy data.

scholarly journals Pneumothorax in cystic fibrosis: a retrospective case series

2006 ◽  
Vol 91 (11) ◽  
pp. 924-925 ◽  
Author(s):  
G M Hafen ◽  
O C Ukoumunne ◽  
P J Robinson
Keyword(s):  
Cystic Fibrosis ◽  
Case Series ◽  
Retrospective Case ◽  
Retrospective Case Series
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