Cardiopulmonary assessment of patients diagnosed with Gaucher’s disease type I
Аутори
Bjelobrk, MarijaLakočević, Milan
Damjanović, Svetozar
Petakov, Milan
Petrović, Milan
Bosnić, Zoran
Arena, Ross
Popović, Dejana
Чланак у часопису (Објављена верзија)
Метаподаци
Приказ свих података о документуАпстракт
Background: Understanding the basis of the phenotypic variation in Gaucher's disease (GD) has proven to be challenging for efficient treatment. The current study examined cardiopulmonary characteristics of patients with GD type 1. Methods: Twenty Caucasian subjects (8/20 female) with diagnosed GD type I (GD-S) and 20 age- and sex-matched healthy controls (C), were assessed (mean age GD-S: 32.6 ± 13.1 vs. C: 36.2 ± 10.6, p >.05) before the initiation of treatment. Standard echocardiography at rest was used to assess left ventricular ejection fraction (LVEF) and pulmonary artery systolic pressure (PASP). Cardiopulmonary exercise testing (CPET) was performed on a recumbent ergometer using a ramp protocol. Results: LVEF was similar in both groups (GD-S: 65.1 ± 5.2% vs. C: 65.2 ± 5.2%, p >.05), as well as PAPS (24.1 ± 4.2 mmHg vs. C: 25.5 ± 1.3 mmHg, p >.05). GD-S had lower weight (p <.05) and worse CPET responses compared to C, including peak values of heart rate, oxygen consumption, carbo...ndioxide production (VCO2), end-tidal pressure of CO2, and O2 pulse, as well as HR reserve after 3 min of recovery and the minute ventilation/VCO2 slope. Conclusions: Patients with GD type I have an abnormal CPET response compared to healthy controls likely due to the complex pathophysiologic process in GD that impacts multiple systems integral to the physiologic response to exercise.
Кључне речи:
cardiopulmonary exercise testing / echocardiography / Gaucher's diseaseИзвор:
Molecular Genetics and Genomic Medicine, 2021, 9, 8Издавач:
- John Wiley and Sons Inc
DOI: 10.1002/mgg3.1757
ISSN: 2324-9269
WoS: 000674061600001
Scopus: 2-s2.0-85110391790
Институција/група
PharmacyTY - JOUR AU - Bjelobrk, Marija AU - Lakočević, Milan AU - Damjanović, Svetozar AU - Petakov, Milan AU - Petrović, Milan AU - Bosnić, Zoran AU - Arena, Ross AU - Popović, Dejana PY - 2021 UR - https://farfar.pharmacy.bg.ac.rs/handle/123456789/3930 AB - Background: Understanding the basis of the phenotypic variation in Gaucher's disease (GD) has proven to be challenging for efficient treatment. The current study examined cardiopulmonary characteristics of patients with GD type 1. Methods: Twenty Caucasian subjects (8/20 female) with diagnosed GD type I (GD-S) and 20 age- and sex-matched healthy controls (C), were assessed (mean age GD-S: 32.6 ± 13.1 vs. C: 36.2 ± 10.6, p >.05) before the initiation of treatment. Standard echocardiography at rest was used to assess left ventricular ejection fraction (LVEF) and pulmonary artery systolic pressure (PASP). Cardiopulmonary exercise testing (CPET) was performed on a recumbent ergometer using a ramp protocol. Results: LVEF was similar in both groups (GD-S: 65.1 ± 5.2% vs. C: 65.2 ± 5.2%, p >.05), as well as PAPS (24.1 ± 4.2 mmHg vs. C: 25.5 ± 1.3 mmHg, p >.05). GD-S had lower weight (p <.05) and worse CPET responses compared to C, including peak values of heart rate, oxygen consumption, carbondioxide production (VCO2), end-tidal pressure of CO2, and O2 pulse, as well as HR reserve after 3 min of recovery and the minute ventilation/VCO2 slope. Conclusions: Patients with GD type I have an abnormal CPET response compared to healthy controls likely due to the complex pathophysiologic process in GD that impacts multiple systems integral to the physiologic response to exercise. PB - John Wiley and Sons Inc T2 - Molecular Genetics and Genomic Medicine T1 - Cardiopulmonary assessment of patients diagnosed with Gaucher’s disease type I VL - 9 IS - 8 DO - 10.1002/mgg3.1757 ER -
@article{ author = "Bjelobrk, Marija and Lakočević, Milan and Damjanović, Svetozar and Petakov, Milan and Petrović, Milan and Bosnić, Zoran and Arena, Ross and Popović, Dejana", year = "2021", abstract = "Background: Understanding the basis of the phenotypic variation in Gaucher's disease (GD) has proven to be challenging for efficient treatment. The current study examined cardiopulmonary characteristics of patients with GD type 1. Methods: Twenty Caucasian subjects (8/20 female) with diagnosed GD type I (GD-S) and 20 age- and sex-matched healthy controls (C), were assessed (mean age GD-S: 32.6 ± 13.1 vs. C: 36.2 ± 10.6, p >.05) before the initiation of treatment. Standard echocardiography at rest was used to assess left ventricular ejection fraction (LVEF) and pulmonary artery systolic pressure (PASP). Cardiopulmonary exercise testing (CPET) was performed on a recumbent ergometer using a ramp protocol. Results: LVEF was similar in both groups (GD-S: 65.1 ± 5.2% vs. C: 65.2 ± 5.2%, p >.05), as well as PAPS (24.1 ± 4.2 mmHg vs. C: 25.5 ± 1.3 mmHg, p >.05). GD-S had lower weight (p <.05) and worse CPET responses compared to C, including peak values of heart rate, oxygen consumption, carbondioxide production (VCO2), end-tidal pressure of CO2, and O2 pulse, as well as HR reserve after 3 min of recovery and the minute ventilation/VCO2 slope. Conclusions: Patients with GD type I have an abnormal CPET response compared to healthy controls likely due to the complex pathophysiologic process in GD that impacts multiple systems integral to the physiologic response to exercise.", publisher = "John Wiley and Sons Inc", journal = "Molecular Genetics and Genomic Medicine", title = "Cardiopulmonary assessment of patients diagnosed with Gaucher’s disease type I", volume = "9", number = "8", doi = "10.1002/mgg3.1757" }
Bjelobrk, M., Lakočević, M., Damjanović, S., Petakov, M., Petrović, M., Bosnić, Z., Arena, R.,& Popović, D.. (2021). Cardiopulmonary assessment of patients diagnosed with Gaucher’s disease type I. in Molecular Genetics and Genomic Medicine John Wiley and Sons Inc., 9(8). https://doi.org/10.1002/mgg3.1757
Bjelobrk M, Lakočević M, Damjanović S, Petakov M, Petrović M, Bosnić Z, Arena R, Popović D. Cardiopulmonary assessment of patients diagnosed with Gaucher’s disease type I. in Molecular Genetics and Genomic Medicine. 2021;9(8). doi:10.1002/mgg3.1757 .
Bjelobrk, Marija, Lakočević, Milan, Damjanović, Svetozar, Petakov, Milan, Petrović, Milan, Bosnić, Zoran, Arena, Ross, Popović, Dejana, "Cardiopulmonary assessment of patients diagnosed with Gaucher’s disease type I" in Molecular Genetics and Genomic Medicine, 9, no. 8 (2021), https://doi.org/10.1002/mgg3.1757 . .