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Article ; Online: An imaging pitfall: misdiagnosis of pulmonary embolism in a patient with advanced cystic fibrosis and bronchiectasis.

Shteinberg, Michal / Kassem, Sameer / Adir, Yochai / Livnat, Galit / Goldberg, Natalia

2023 Volume 401, Issue 10373, Page(s) 303

MeSH term(s)	Humans ; Cystic Fibrosis/complications ; Cystic Fibrosis/diagnosis ; Bronchiectasis/complications ; Bronchiectasis/diagnostic imaging ; Pulmonary Embolism/complications ; Pulmonary Embolism/diagnostic imaging ; Diagnostic Imaging ; Diagnostic Errors
Language	English
Publishing date	2023-01-25
Publishing country	England
Document type	Journal Article
ZDB-ID	3306-6
ISSN	1474-547X ; 0023-7507 ; 0140-6736
ISSN (online)	1474-547X
ISSN	0023-7507 ; 0140-6736
DOI	10.1016/S0140-6736(22)02165-1
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Article: Pneumonia in the presentation of Kawasaki disease: The syndrome or a sequence of two diseases?

Khoury, Lana / Livnat, Galit / Hamad Saied, Mohamad / Yaacoby-Bianu, Karin

Clinical case reports

2022 Volume 10, Issue 12, Page(s) e6676

Abstract: Two cases of Kawasaki disease (KD) presented as persistent lung consolidation associated ... ...

Abstract	Two cases of Kawasaki disease (KD) presented as persistent lung consolidation associated with
Language	English
Publishing date	2022-12-05
Publishing country	England
Document type	Case Reports
ZDB-ID	2740234-4
ISSN	2050-0904
ISSN	2050-0904
DOI	10.1002/ccr3.6676
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Article: Respiratory healthcare professionals' views on long-term recommendations of interventions to prevent acute respiratory illnesses after the COVID-19 pandemic.

Yaacoby-Bianu, Karin / Livnat, Galit / Chalmers, James D / Shteinberg, Michal

ERJ open research

2022 Volume 8, Issue 4

Abstract: Respiratory professionals support the continuing use of protective measures for respiratory patients following the #COVID19 pandemic. The optimal use of these measures should be considered in clinical guidelines and public health recommendations. ...

Abstract	Respiratory professionals support the continuing use of protective measures for respiratory patients following the #COVID19 pandemic. The optimal use of these measures should be considered in clinical guidelines and public health recommendations.
Language	English
Publishing date	2022-10-04
Publishing country	England
Document type	Journal Article
ZDB-ID	2827830-6
ISSN	2312-0541
ISSN	2312-0541
DOI	10.1183/23120541.00114-2022
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Article ; Online: Respiratory healthcare professionals’ views on long-term recommendations of interventions to prevent acute respiratory illnesses after the COVID-19 pandemic

Karin Yaacoby-Bianu / Galit Livnat / James D. Chalmers / Michal Shteinberg

ERJ Open Research, Vol 8, Iss

2022 Volume 4

Keywords	Medicine ; R
Language	English
Publishing date	2022-10-01T00:00:00Z
Publisher	European Respiratory Society
Document type	Article ; Online
Database	BASE - Bielefeld Academic Search Engine (life sciences selection)

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Article ; Online: Exhaled Breath Condensate and Respiratory Sequelae in Children Post-COVID-19.

Fireman Klein, Einat / Yaacoby-Bianu, Karin / Orlin, Ido / Zetser, Anna / Purits, Nona / Livnat, Galit

Respiration; international review of thoracic diseases

2023 Volume 102, Issue 7, Page(s) 479–486

Abstract: Background: Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) causes an acute respiratory illness. A substantial proportion of adults experience persistent symptoms. There is a paucity of data on respiratory sequelae in children. Exhaled ... ...

Abstract	Background: Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) causes an acute respiratory illness. A substantial proportion of adults experience persistent symptoms. There is a paucity of data on respiratory sequelae in children. Exhaled breath condensate (EBC) is a non-invasive tool used to assess airway inflammation. Objectives: This study aimed to evaluate EBC parameters, respiratory, mental and physical ability among children post COVID-19 infection. Methods: Observational study of confirmed SARS-CoV-2 infection cases among children, aged 5-18 years, evaluated once, 1-6 months post positive SARS-CoV-2 PCR testing. All subjects performed spirometry, 6-min walk test (6MWT), EBC (pH, interleukin-6), and completed medical history questionnaires, Depression, Anxiety, and Stress Scale (DASS-21), and physical activity scores. Severity of COVID-19 disease was classified according to WHO criteria. Results: Fifty-eight children were included and classified asymptomatic (n = 14), mild (n = 37), and moderate (n = 7) disease. The asymptomatic group included younger patients compared to the mild and moderate groups (8.9 ± 2.5y vs. 12.3 ± 3.6y and 14.6 ± 2.5y, respectively, p = 0.001), as well as lower DASS-21 total scores (3.4 ± 4 vs. 8.7 ± 9.4 and 8.7 ± 0.6 respectively, p = 0.056), with higher scores in proximity to positive PCR (p = 0.011). No differences were found between the 3 groups regarding EBC, 6MWT, spirometry, body mass index percentile, and activity scores. Conclusions: COVID-19 is an asymptomatic-mild disease in most young healthy children, with gradually diminishing emotional symptoms. Children without prolonged respiratory symptoms revealed no significant pulmonary sequelae as evaluated by EBC markers, spirometry, 6MWT, and activity scores. Larger studies are required to assess long-term pediatric consequences of post SARS-CoV-2 infection, to assess the need for pulmonology surveillance.
MeSH term(s)	Adult ; Humans ; Child ; Asthma/diagnosis ; COVID-19/complications ; COVID-19/diagnosis ; Respiratory Function Tests ; SARS-CoV-2 ; Lung ; Disease Progression ; Breath Tests ; Exhalation
Language	English
Publishing date	2023-06-30
Publishing country	Switzerland
Document type	Observational Study ; Journal Article
ZDB-ID	206674-9
ISSN	1423-0356 ; 0025-7931
ISSN (online)	1423-0356
ISSN	0025-7931
DOI	10.1159/000530971
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Article ; Online: Improvement in BMI z-score following adenotonsillectomy in adolescents aged 12-18 years: a retrospective cohort study.

Nathan, Keren / Livnat, Galit / Feraru, Liat / Pillar, Giora

BMC pediatrics

2021 Volume 21, Issue 1, Page(s) 184

Abstract: Background: Characteristics of obstructive sleep apnea (OSA) changes with age. Infants, toddlers and prepubertal children with OSA are usually underweight and may suffer from failure to thrive (FTT). Adenotonsillectomy (T&A) is the first line of ... ...

Abstract	Background: Characteristics of obstructive sleep apnea (OSA) changes with age. Infants, toddlers and prepubertal children with OSA are usually underweight and may suffer from failure to thrive (FTT). Adenotonsillectomy (T&A) is the first line of treatment for OSA in childhood. In adults OSA is commonly associated with obesity and the metabolic syndrome. The change in body mass index (BMI) in adolescents with OSA following T&A was only sporadically studied. Thus, we peruse to examine the BMI z-score change following T&A in adolescents. Methods: Clalit Health Services is the largest health care organization in Israel with the largest patient registry (more than 50% of the population). Two hundred and forty two adolescents aged 12-18 who underwent T&A between 2006 and 2015 were identified in the Clalit registry and their characteristics including height and weight were retrieved. The BMI z-score of these adolescents at baseline (up to 3 months prior to T&A) and during the consecutive 3 years after T&A were analyzed and compared. Results: Changes in BMI Z-score were observed to all directions following T&A with overall small increase, not statistically significant (P = 0.26) from a median of 0.79 prior to T&A to a median of 0.835 after it. There was a minimal trend toward BMI z-score reduction in overweight children (n = 74) from 1.508 to 1.48 following T&A (p = NS), and in obese children (n = 33) from 2.288 to 2.000 (P = 0.06, 2 tailed). Interestingly thin individuals (n = 6) increased their BMI z-score following T&A from - 2.4 to - 0.59 (p = 0.046). Conclusions: Adolescents show variable changes in their BMI z-score following T&A. In this aspect their BMI z-score change is closer to the change seen in adults treated for OSA and not that of young children. The changes observed show a trend toward normalization of the BMI z-score such that overweight children tend to decrease their BMI z-score while thin individuals tend to increase it.
MeSH term(s)	Adenoidectomy ; Adolescent ; Body Mass Index ; Child ; Child, Preschool ; Humans ; Infant ; Israel/epidemiology ; Polysomnography ; Retrospective Studies
Language	English
Publishing date	2021-04-20
Publishing country	England
Document type	Journal Article
ZDB-ID	2041342-7
ISSN	1471-2431 ; 1471-2431
ISSN (online)	1471-2431
ISSN	1471-2431
DOI	10.1186/s12887-021-02634-y
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Article ; Online: Phenotypic Alteration of an Established Human Airway Cell Line by Media Selection.

Livnat, Galit / Meeker, Jessica D / Ostmann, Alicia J / Strecker, Lauren M / Clancy, John P / Brewington, John J

International journal of molecular sciences

2023 Volume 24, Issue 2

Abstract: Cystic Fibrosis (CF) is caused by mutations in the CF transmembrane conductance regulator (CFTR), a chloride/bicarbonate channel. Many studies utilize human airway cell models (cell lines and primary cells) to study different aspects of CFTR biology. ... ...

Abstract	Cystic Fibrosis (CF) is caused by mutations in the CF transmembrane conductance regulator (CFTR), a chloride/bicarbonate channel. Many studies utilize human airway cell models (cell lines and primary cells) to study different aspects of CFTR biology. Media selection can alter the growth and differentiation of primary cells, yet the impact on stable airway cell lines is unclear. To determine the impact of media and growth conditions on CFBE41o- cells stably transduced with wild-type or F508del CFTR, we examined four commonly used growth media, measuring epithelial and mesenchymal markers, as well as CFTR expression, maturation, and function. The selection of growth media altered the expression of epithelial and mesenchymal markers in the cell lines, and significantly impacted CFTR expression and subsequent function. These results highlight the importance of media selection to CFTR and cell line behavior and should be considered in both studies of primary human airway cells and stable cell lines.
MeSH term(s)	Humans ; Cystic Fibrosis Transmembrane Conductance Regulator/genetics ; Cystic Fibrosis Transmembrane Conductance Regulator/metabolism ; Epithelial Cells/metabolism ; Cystic Fibrosis/metabolism ; Cell Line ; Mutation
Chemical Substances	Cystic Fibrosis Transmembrane Conductance Regulator (126880-72-6)
Language	English
Publishing date	2023-01-08
Publishing country	Switzerland
Document type	Journal Article
ZDB-ID	2019364-6
ISSN	1422-0067 ; 1422-0067 ; 1661-6596
ISSN (online)	1422-0067
ISSN	1422-0067 ; 1661-6596
DOI	10.3390/ijms24021246
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Article: Optimizing CFTR modulator therapy management for cystic fibrosis through the ReX platform.

Yaacoby-Bianu, Karin / Cohen-Cymberknoh, Malena / Shoseyov, David / Lavi, Tal / Ostrovski, Ana / Shteinberg, Michal / Livnat, Galit

Frontiers in pediatrics

2023 Volume 11, Page(s) 1300968

Abstract: Background: Cystic fibrosis (CF) is a chronic multi-systemic disease that requires a complex daily treatment regimen. Therefore, there is sub-optimal adherence to CF therapies, and it was shown to impact its clinical and economic burden. Cystic fibrosis ...

Abstract	Background: Cystic fibrosis (CF) is a chronic multi-systemic disease that requires a complex daily treatment regimen. Therefore, there is sub-optimal adherence to CF therapies, and it was shown to impact its clinical and economic burden. Cystic fibrosis transmembrane conductance regulator modulators (CFTRm) are high-cost medications that demonstrated significant benefit in clinical trials. The aim of this study was to evaluate the safety, usability, and efficacy of the ReX platform in medication management of CFTRm for the treatment of people with CF (pwCF). Methods: ReX is a patient engagement platform consisting of a cloud-based management system and a cell-enabled handheld device intended to dispense oral medication into the patient's mouth, following a pre-programmed treatment protocol. It provides real-time adherence data to caregivers and timely, personalized reminders to patients. This is a prospective multi-center open study for pwCFs older than 12 years, who had been prescribed CFTRm [elexacaftor/tezacaftor/ivacaftor (ETI) or tezacaftor/ivacaftor (TI)], and provided consent to use ReX platform to receive CFTRm and record their health condition. Study duration was 12-24 months, with clinic visits where physical examination, body mass index (BMI), and pulmonary function tests were performed, and user experience questionnaires were filled in. Results: Ten pwCFs from two CF centers in Israel were included. The mean age was 31.5 years (range 15-74 years); eight were taking ETI and two TI. Median adherence to CFTRm was 97.5% (range 70%-100%) in the first year and 94% (range 84%-99%) in the second year, which is higher than the previously reported CFTRm adherence of ∼80%. No adverse events related to the use of the platform were reported. Patients reported ReX to be valuable to their treatment management and user friendly. Estimated mean forced expiratory volume in 1 s (FEV Conclusions: Using the ReX platform in medication management of pwCF treated by CFTRm is safe, easy to use, and effective in improving the adherence to treatment and the clinical outcomes. Consequently, this device may potentially reduce costs to healthcare providers. Further larger and long-term studies are required to examine the clinical benefits of the ReX platform.
Language	English
Publishing date	2023-12-19
Publishing country	Switzerland
Document type	Journal Article
ZDB-ID	2711999-3
ISSN	2296-2360
ISSN	2296-2360
DOI	10.3389/fped.2023.1300968
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Article ; Online: Real life evaluation of the multi-organ effects of Lumacaftor/Ivacaftor on F508del homozygous cystic fibrosis patients.

Yaacoby-Bianu, Karin / Schnapp, Zeev / Koren, Ilana / Ilivitzki, Anat / Khatib, Mohamed / Shorbaji, Nadeem / Shteinberg, Michal / Livnat, Galit

BMC pharmacology & toxicology

2022 Volume 23, Issue 1, Page(s) 80

Abstract: Background: Lumacaftor/Ivacaftor (LUM-IVA), a cystic fibrosis transmembrane conductance regulator (CFTR) protein corrector-potentiator combination, improves lung function and reduces pulmonary exacerbations (PEx) in F508del homozygous CF patients. ... ...

Abstract	Background: Lumacaftor/Ivacaftor (LUM-IVA), a cystic fibrosis transmembrane conductance regulator (CFTR) protein corrector-potentiator combination, improves lung function and reduces pulmonary exacerbations (PEx) in F508del homozygous CF patients. However, the systemic effects of LUM-IVA outside the respiratory system have not yet been thoroughly investigated. Methods: A prospective, real-world, yearlong study was performed on F508del homozygous adult CF patients who commenced treatment with LUM-IVA. Pancreatic function, bone metabolism, fertility status, nutritional and pulmonary factors were evaluated. Results: Twelve patients, mean age 28.3 years (18.6-43.9) were recruited. Following 12 months of treatment, no changes were detected in glucose, insulin, c-peptide or BMI values. A significant relative decrease in mean alkaline-phosphatase levels (122.8 U/L vs 89.4, p = 0.002) and a trend toward an increase in calcium levels (9.5 vs 9.9 mg/dL, p = 0.074) were observed. A non-significant improvement in mean DEXA spine t-score after a year of treatment (-2.1 vs -1.6, n = 4, p = 0.11) was detected. Sweat chloride concentrations decreased significantly (-21.4 mEq/L; p = 0.003). Pulmonary outcome revealed improvement in spirometry values during the first three months (FEV Conclusions: After one year of treatment, stabilization was observed in the pancreatic indices, nutritional status, structure and function of the lungs, with a beneficial effect on bone mineral metabolism and CFTR function. Additional studies should investigate the effect of CFTR modulators on extra-pulmonary manifestations.
MeSH term(s)	Adult ; Humans ; Anti-Bacterial Agents ; C-Peptide ; Calcium ; Chlorides ; Cystic Fibrosis/drug therapy ; Cystic Fibrosis/genetics ; Cystic Fibrosis Transmembrane Conductance Regulator/genetics ; Drug Combinations ; Glucose ; Mutation ; Prospective Studies ; Adolescent ; Young Adult ; Chloride Channel Agonists/therapeutic use
Chemical Substances	Anti-Bacterial Agents ; C-Peptide ; Calcium (SY7Q814VUP) ; Chlorides ; Cystic Fibrosis Transmembrane Conductance Regulator (126880-72-6) ; Drug Combinations ; Glucose (IY9XDZ35W2) ; ivacaftor (1Y740ILL1Z) ; lumacaftor (EGP8L81APK) ; Chloride Channel Agonists
Language	English
Publishing date	2022-10-20
Publishing country	England
Document type	Journal Article ; Research Support, Non-U.S. Gov't
ZDB-ID	2680259-4
ISSN	2050-6511 ; 2050-6511
ISSN (online)	2050-6511
ISSN	2050-6511
DOI	10.1186/s40360-022-00624-z
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Article ; Online: Eradication of Nontuberculous Mycobacteria in People with Cystic Fibrosis Treated with Elexacaftor/Tezacaftor/Ivacaftor: A Multicenter Cohort Study.

Wiesel, Vered / Aviram, Micha / Mei-Zahav, Meir / Dotan, Miri / Prais, Dario / Cohen-Cymberknoh, Malena / Gur, Michal / Bar-Yoseph, Ronen / Livnat, Galit / Goldbart, Aviv / Hazan, Guy / Hazan, Itai / Golan-Tripto, Inbal

Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society

2023 Volume 23, Issue 1, Page(s) 41–49

Abstract: Background: The prevalence of nontuberculous mycobacteria (NTM) infections is rising in people with cystic fibrosis (pwCF). NTM infection, especially infection with Mycobacterium abscessus complex (MABC), is commonly associated with severe lung ... ...

Abstract	Background: The prevalence of nontuberculous mycobacteria (NTM) infections is rising in people with cystic fibrosis (pwCF). NTM infection, especially infection with Mycobacterium abscessus complex (MABC), is commonly associated with severe lung deterioration. The current treatment modalities, including multiple intravenous antibiotics, frequently fail to achieve airway eradication. Although treatment with elexacaftor/tezacaftor/ivacaftor (ETI) has been shown to modulate the lung microbiome, data regarding its role in eradicating NTM in pwCF is lacking. Our aim was to evaluate the impact of ETI on the rate of NTM eradication in pwCF. Methods: This retrospective multicenter cohort study included pwCF from five CF centers in Israel. PwCF aged older than 6 who had at least one positive NTM airway culture in the past two years and were treated with ETI for at least one year were included. The annual NTM and bacterial isolations, pulmonary function tests, and body mass index were analyzed before and after ETI treatment. Results: Fifteen pwCF were included (median age 20.9 years, 73.3% females, 80% pancreatic insufficient). In nine patients (66%) NTM isolations were eradicated following treatment with ETI. Seven of them had MABC. The median time between the first NTM isolation and treatment with ETI was 2.71 years (0.27-10.35 years). Eradication of NTM was associated with improved pulmonary function tests (p<0.05). Conclusions: For the first time, we report successful eradication of NTM, including MABC, following treatment with ETI in pwCF. Additional studies are needed to assess whether treatment with ETI can result in the long-term eradication of NTM.
MeSH term(s)	Female ; Humans ; Aged ; Young Adult ; Adult ; Male ; Nontuberculous Mycobacteria ; Cystic Fibrosis/complications ; Cystic Fibrosis/drug therapy ; Cystic Fibrosis/epidemiology ; Cohort Studies ; Mycobacterium abscessus ; Mycobacterium Infections, Nontuberculous/diagnosis ; Mycobacterium Infections, Nontuberculous/drug therapy ; Mycobacterium Infections, Nontuberculous/epidemiology ; Cystic Fibrosis Transmembrane Conductance Regulator ; Benzodioxoles ; Indoles ; Aminophenols ; Pyrazoles ; Pyridines ; Pyrrolidines ; Quinolones
Chemical Substances	elexacaftor (RRN67GMB0V) ; ivacaftor (1Y740ILL1Z) ; tezacaftor ; Cystic Fibrosis Transmembrane Conductance Regulator (126880-72-6) ; Benzodioxoles ; Indoles ; Aminophenols ; Pyrazoles ; Pyridines ; Pyrrolidines ; Quinolones
Language	English
Publishing date	2023-05-10
Publishing country	Netherlands
Document type	Multicenter Study ; Journal Article
ZDB-ID	2084724-5
ISSN	1873-5010 ; 1569-1993
ISSN (online)	1873-5010
ISSN	1569-1993
DOI	10.1016/j.jcf.2023.05.003
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