Is sirolimus a therapeutic option for patients with progressive pulmonary lymphangioleiomyomatosis?

Background Lymphangioleiomyomatosis (LAM) is a rare lung disease characterised by progressive airflow obstruction. No effective medical treatment is available but therapy with sirolimus has shown some promise. The aim of this observational study was to evaluate sirolimus in progressive LAM. Methods...
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Autor*in:	Neurohr, Claus [verfasserIn] Hoffmann, Anna L Huppmann, Patrick Herrera, Vivian A Ihle, Franziska Leuschner, Stefan von Wulffen, Werner Meis, Tobias Baezner, Carlos Leuchte, Hanno Baumgartner, Rainer Zimmermann, Gregor Behr, Juergen

Format:	E-Artikel
Sprache:	Englisch

Erschienen:	2011

Schlagwörter:	Force Vital Capacity Sirolimus Tuberous Sclerosis Complex Airflow Obstruction Tuberous Sclerosis Complex Patient

Anmerkung:	© Neurohr et al; licensee BioMed Central Ltd. 2011

Übergeordnetes Werk:	Enthalten in: Respiratory research - London : BioMed Central, 2001, 12(2011), 1 vom: 01. Dez.
Übergeordnetes Werk:	volume:12 ; year:2011 ; number:1 ; day:01 ; month:12

Links:	Volltext

DOI / URN:	10.1186/1465-9921-12-66

Katalog-ID:	SPR028512685

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245	1	0	\|a Is sirolimus a therapeutic option for patients with progressive pulmonary lymphangioleiomyomatosis?
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520			\|a Background Lymphangioleiomyomatosis (LAM) is a rare lung disease characterised by progressive airflow obstruction. No effective medical treatment is available but therapy with sirolimus has shown some promise. The aim of this observational study was to evaluate sirolimus in progressive LAM. Methods Sirolimus (trough level 5 - 10 ng/ml) was administered to ten female patients (42.4 ± 11.9 years) with documented progression. Serial pulmonary function tests and six-minute-walk-distance (6-MWD) assessments were performed. Results The mean loss of $ FEV_{1} $ was -2.30 ± 0.52 ml/day before therapy and a significant mean gain of $ FEV_{1} $ of 1.19 ± 0.26 ml/day was detected during treatment (p = 0.001). Mean $ FEV_{1} $ and FVC at baseline were 1.12 ± 0.15 l (36.1 ± 4.5%pred.) and 2.47 ± 0.25 l (69.2 ± 6.5%pred.), respectively. At three and six months during follow-up a significant increase of $ FEV_{1} $ and FVC was demonstrated (3 months $ ΔFEV_{1} $: 220 ± 82 ml, p = 0.024; 6 months $ ΔFEV_{1} $: 345 ± 58 ml, p = 0.001); (3 months ΔFVC: 360 ± 141 ml, p = 0.031; 6 months ΔFVC: 488 ± 138 ml, p = 0.006). Sirolimus was discontinued in 3 patients because of serious recurrent lower respiratory tract infection or sirolimus-induced pneumonitis. No deaths and no pneumothoraces occurred during therapy. Conclusions Our data suggest that sirolimus might be considered as a therapeutic option in rapidly declining LAM patients. However, sirolimus administration may be associated with severe respiratory adverse events requiring treatment cessation in some patients. Moreover, discontinuation of sirolimus is mandatory prior to lung transplantation.
650		4	\|a Force Vital Capacity \|7 (dpeaa)DE-He213
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700	1		\|a Leuschner, Stefan \|4 aut
700	1		\|a von Wulffen, Werner \|4 aut
700	1		\|a Meis, Tobias \|4 aut
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700	1		\|a Leuchte, Hanno \|4 aut
700	1		\|a Baumgartner, Rainer \|4 aut
700	1		\|a Zimmermann, Gregor \|4 aut
700	1		\|a Behr, Juergen \|4 aut
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allfields	10.1186/1465-9921-12-66 doi (DE-627)SPR028512685 (SPR)1465-9921-12-66-e DE-627 ger DE-627 rakwb eng Neurohr, Claus verfasserin aut Is sirolimus a therapeutic option for patients with progressive pulmonary lymphangioleiomyomatosis? 2011 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © Neurohr et al; licensee BioMed Central Ltd. 2011 Background Lymphangioleiomyomatosis (LAM) is a rare lung disease characterised by progressive airflow obstruction. No effective medical treatment is available but therapy with sirolimus has shown some promise. The aim of this observational study was to evaluate sirolimus in progressive LAM. Methods Sirolimus (trough level 5 - 10 ng/ml) was administered to ten female patients (42.4 ± 11.9 years) with documented progression. Serial pulmonary function tests and six-minute-walk-distance (6-MWD) assessments were performed. Results The mean loss of $ FEV_{1} $ was -2.30 ± 0.52 ml/day before therapy and a significant mean gain of $ FEV_{1} $ of 1.19 ± 0.26 ml/day was detected during treatment (p = 0.001). Mean $ FEV_{1} $ and FVC at baseline were 1.12 ± 0.15 l (36.1 ± 4.5%pred.) and 2.47 ± 0.25 l (69.2 ± 6.5%pred.), respectively. At three and six months during follow-up a significant increase of $ FEV_{1} $ and FVC was demonstrated (3 months $ ΔFEV_{1} $: 220 ± 82 ml, p = 0.024; 6 months $ ΔFEV_{1} $: 345 ± 58 ml, p = 0.001); (3 months ΔFVC: 360 ± 141 ml, p = 0.031; 6 months ΔFVC: 488 ± 138 ml, p = 0.006). Sirolimus was discontinued in 3 patients because of serious recurrent lower respiratory tract infection or sirolimus-induced pneumonitis. No deaths and no pneumothoraces occurred during therapy. Conclusions Our data suggest that sirolimus might be considered as a therapeutic option in rapidly declining LAM patients. However, sirolimus administration may be associated with severe respiratory adverse events requiring treatment cessation in some patients. Moreover, discontinuation of sirolimus is mandatory prior to lung transplantation. Force Vital Capacity (dpeaa)DE-He213 Sirolimus (dpeaa)DE-He213 Tuberous Sclerosis Complex (dpeaa)DE-He213 Airflow Obstruction (dpeaa)DE-He213 Tuberous Sclerosis Complex Patient (dpeaa)DE-He213 Hoffmann, Anna L aut Huppmann, Patrick aut Herrera, Vivian A aut Ihle, Franziska aut Leuschner, Stefan aut von Wulffen, Werner aut Meis, Tobias aut Baezner, Carlos aut Leuchte, Hanno aut Baumgartner, Rainer aut Zimmermann, Gregor aut Behr, Juergen aut Enthalten in Respiratory research London : BioMed Central, 2001 12(2011), 1 vom: 01. Dez. (DE-627)326646485 (DE-600)2041675-1 1465-993X nnns volume:12 year:2011 number:1 day:01 month:12 https://dx.doi.org/10.1186/1465-9921-12-66 kostenfrei Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_702 GBV_ILN_2001 GBV_ILN_2003 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2008 GBV_ILN_2009 GBV_ILN_2010 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2015 GBV_ILN_2020 GBV_ILN_2021 GBV_ILN_2025 GBV_ILN_2031 GBV_ILN_2038 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2056 GBV_ILN_2057 GBV_ILN_2061 GBV_ILN_2111 GBV_ILN_2113 GBV_ILN_2153 GBV_ILN_2190 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 12 2011 1 01 12
spelling	10.1186/1465-9921-12-66 doi (DE-627)SPR028512685 (SPR)1465-9921-12-66-e DE-627 ger DE-627 rakwb eng Neurohr, Claus verfasserin aut Is sirolimus a therapeutic option for patients with progressive pulmonary lymphangioleiomyomatosis? 2011 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © Neurohr et al; licensee BioMed Central Ltd. 2011 Background Lymphangioleiomyomatosis (LAM) is a rare lung disease characterised by progressive airflow obstruction. No effective medical treatment is available but therapy with sirolimus has shown some promise. The aim of this observational study was to evaluate sirolimus in progressive LAM. Methods Sirolimus (trough level 5 - 10 ng/ml) was administered to ten female patients (42.4 ± 11.9 years) with documented progression. Serial pulmonary function tests and six-minute-walk-distance (6-MWD) assessments were performed. Results The mean loss of $ FEV_{1} $ was -2.30 ± 0.52 ml/day before therapy and a significant mean gain of $ FEV_{1} $ of 1.19 ± 0.26 ml/day was detected during treatment (p = 0.001). Mean $ FEV_{1} $ and FVC at baseline were 1.12 ± 0.15 l (36.1 ± 4.5%pred.) and 2.47 ± 0.25 l (69.2 ± 6.5%pred.), respectively. At three and six months during follow-up a significant increase of $ FEV_{1} $ and FVC was demonstrated (3 months $ ΔFEV_{1} $: 220 ± 82 ml, p = 0.024; 6 months $ ΔFEV_{1} $: 345 ± 58 ml, p = 0.001); (3 months ΔFVC: 360 ± 141 ml, p = 0.031; 6 months ΔFVC: 488 ± 138 ml, p = 0.006). Sirolimus was discontinued in 3 patients because of serious recurrent lower respiratory tract infection or sirolimus-induced pneumonitis. No deaths and no pneumothoraces occurred during therapy. Conclusions Our data suggest that sirolimus might be considered as a therapeutic option in rapidly declining LAM patients. However, sirolimus administration may be associated with severe respiratory adverse events requiring treatment cessation in some patients. Moreover, discontinuation of sirolimus is mandatory prior to lung transplantation. Force Vital Capacity (dpeaa)DE-He213 Sirolimus (dpeaa)DE-He213 Tuberous Sclerosis Complex (dpeaa)DE-He213 Airflow Obstruction (dpeaa)DE-He213 Tuberous Sclerosis Complex Patient (dpeaa)DE-He213 Hoffmann, Anna L aut Huppmann, Patrick aut Herrera, Vivian A aut Ihle, Franziska aut Leuschner, Stefan aut von Wulffen, Werner aut Meis, Tobias aut Baezner, Carlos aut Leuchte, Hanno aut Baumgartner, Rainer aut Zimmermann, Gregor aut Behr, Juergen aut Enthalten in Respiratory research London : BioMed Central, 2001 12(2011), 1 vom: 01. Dez. (DE-627)326646485 (DE-600)2041675-1 1465-993X nnns volume:12 year:2011 number:1 day:01 month:12 https://dx.doi.org/10.1186/1465-9921-12-66 kostenfrei Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_702 GBV_ILN_2001 GBV_ILN_2003 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2008 GBV_ILN_2009 GBV_ILN_2010 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2015 GBV_ILN_2020 GBV_ILN_2021 GBV_ILN_2025 GBV_ILN_2031 GBV_ILN_2038 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2056 GBV_ILN_2057 GBV_ILN_2061 GBV_ILN_2111 GBV_ILN_2113 GBV_ILN_2153 GBV_ILN_2190 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 12 2011 1 01 12
allfields_unstemmed	10.1186/1465-9921-12-66 doi (DE-627)SPR028512685 (SPR)1465-9921-12-66-e DE-627 ger DE-627 rakwb eng Neurohr, Claus verfasserin aut Is sirolimus a therapeutic option for patients with progressive pulmonary lymphangioleiomyomatosis? 2011 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © Neurohr et al; licensee BioMed Central Ltd. 2011 Background Lymphangioleiomyomatosis (LAM) is a rare lung disease characterised by progressive airflow obstruction. No effective medical treatment is available but therapy with sirolimus has shown some promise. The aim of this observational study was to evaluate sirolimus in progressive LAM. Methods Sirolimus (trough level 5 - 10 ng/ml) was administered to ten female patients (42.4 ± 11.9 years) with documented progression. Serial pulmonary function tests and six-minute-walk-distance (6-MWD) assessments were performed. Results The mean loss of $ FEV_{1} $ was -2.30 ± 0.52 ml/day before therapy and a significant mean gain of $ FEV_{1} $ of 1.19 ± 0.26 ml/day was detected during treatment (p = 0.001). Mean $ FEV_{1} $ and FVC at baseline were 1.12 ± 0.15 l (36.1 ± 4.5%pred.) and 2.47 ± 0.25 l (69.2 ± 6.5%pred.), respectively. At three and six months during follow-up a significant increase of $ FEV_{1} $ and FVC was demonstrated (3 months $ ΔFEV_{1} $: 220 ± 82 ml, p = 0.024; 6 months $ ΔFEV_{1} $: 345 ± 58 ml, p = 0.001); (3 months ΔFVC: 360 ± 141 ml, p = 0.031; 6 months ΔFVC: 488 ± 138 ml, p = 0.006). Sirolimus was discontinued in 3 patients because of serious recurrent lower respiratory tract infection or sirolimus-induced pneumonitis. No deaths and no pneumothoraces occurred during therapy. Conclusions Our data suggest that sirolimus might be considered as a therapeutic option in rapidly declining LAM patients. However, sirolimus administration may be associated with severe respiratory adverse events requiring treatment cessation in some patients. Moreover, discontinuation of sirolimus is mandatory prior to lung transplantation. Force Vital Capacity (dpeaa)DE-He213 Sirolimus (dpeaa)DE-He213 Tuberous Sclerosis Complex (dpeaa)DE-He213 Airflow Obstruction (dpeaa)DE-He213 Tuberous Sclerosis Complex Patient (dpeaa)DE-He213 Hoffmann, Anna L aut Huppmann, Patrick aut Herrera, Vivian A aut Ihle, Franziska aut Leuschner, Stefan aut von Wulffen, Werner aut Meis, Tobias aut Baezner, Carlos aut Leuchte, Hanno aut Baumgartner, Rainer aut Zimmermann, Gregor aut Behr, Juergen aut Enthalten in Respiratory research London : BioMed Central, 2001 12(2011), 1 vom: 01. Dez. (DE-627)326646485 (DE-600)2041675-1 1465-993X nnns volume:12 year:2011 number:1 day:01 month:12 https://dx.doi.org/10.1186/1465-9921-12-66 kostenfrei Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_702 GBV_ILN_2001 GBV_ILN_2003 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2008 GBV_ILN_2009 GBV_ILN_2010 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2015 GBV_ILN_2020 GBV_ILN_2021 GBV_ILN_2025 GBV_ILN_2031 GBV_ILN_2038 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2056 GBV_ILN_2057 GBV_ILN_2061 GBV_ILN_2111 GBV_ILN_2113 GBV_ILN_2153 GBV_ILN_2190 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 12 2011 1 01 12
allfieldsGer	10.1186/1465-9921-12-66 doi (DE-627)SPR028512685 (SPR)1465-9921-12-66-e DE-627 ger DE-627 rakwb eng Neurohr, Claus verfasserin aut Is sirolimus a therapeutic option for patients with progressive pulmonary lymphangioleiomyomatosis? 2011 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © Neurohr et al; licensee BioMed Central Ltd. 2011 Background Lymphangioleiomyomatosis (LAM) is a rare lung disease characterised by progressive airflow obstruction. No effective medical treatment is available but therapy with sirolimus has shown some promise. The aim of this observational study was to evaluate sirolimus in progressive LAM. Methods Sirolimus (trough level 5 - 10 ng/ml) was administered to ten female patients (42.4 ± 11.9 years) with documented progression. Serial pulmonary function tests and six-minute-walk-distance (6-MWD) assessments were performed. Results The mean loss of $ FEV_{1} $ was -2.30 ± 0.52 ml/day before therapy and a significant mean gain of $ FEV_{1} $ of 1.19 ± 0.26 ml/day was detected during treatment (p = 0.001). Mean $ FEV_{1} $ and FVC at baseline were 1.12 ± 0.15 l (36.1 ± 4.5%pred.) and 2.47 ± 0.25 l (69.2 ± 6.5%pred.), respectively. At three and six months during follow-up a significant increase of $ FEV_{1} $ and FVC was demonstrated (3 months $ ΔFEV_{1} $: 220 ± 82 ml, p = 0.024; 6 months $ ΔFEV_{1} $: 345 ± 58 ml, p = 0.001); (3 months ΔFVC: 360 ± 141 ml, p = 0.031; 6 months ΔFVC: 488 ± 138 ml, p = 0.006). Sirolimus was discontinued in 3 patients because of serious recurrent lower respiratory tract infection or sirolimus-induced pneumonitis. No deaths and no pneumothoraces occurred during therapy. Conclusions Our data suggest that sirolimus might be considered as a therapeutic option in rapidly declining LAM patients. However, sirolimus administration may be associated with severe respiratory adverse events requiring treatment cessation in some patients. Moreover, discontinuation of sirolimus is mandatory prior to lung transplantation. Force Vital Capacity (dpeaa)DE-He213 Sirolimus (dpeaa)DE-He213 Tuberous Sclerosis Complex (dpeaa)DE-He213 Airflow Obstruction (dpeaa)DE-He213 Tuberous Sclerosis Complex Patient (dpeaa)DE-He213 Hoffmann, Anna L aut Huppmann, Patrick aut Herrera, Vivian A aut Ihle, Franziska aut Leuschner, Stefan aut von Wulffen, Werner aut Meis, Tobias aut Baezner, Carlos aut Leuchte, Hanno aut Baumgartner, Rainer aut Zimmermann, Gregor aut Behr, Juergen aut Enthalten in Respiratory research London : BioMed Central, 2001 12(2011), 1 vom: 01. Dez. (DE-627)326646485 (DE-600)2041675-1 1465-993X nnns volume:12 year:2011 number:1 day:01 month:12 https://dx.doi.org/10.1186/1465-9921-12-66 kostenfrei Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_702 GBV_ILN_2001 GBV_ILN_2003 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2008 GBV_ILN_2009 GBV_ILN_2010 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2015 GBV_ILN_2020 GBV_ILN_2021 GBV_ILN_2025 GBV_ILN_2031 GBV_ILN_2038 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2056 GBV_ILN_2057 GBV_ILN_2061 GBV_ILN_2111 GBV_ILN_2113 GBV_ILN_2153 GBV_ILN_2190 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 12 2011 1 01 12
allfieldsSound	10.1186/1465-9921-12-66 doi (DE-627)SPR028512685 (SPR)1465-9921-12-66-e DE-627 ger DE-627 rakwb eng Neurohr, Claus verfasserin aut Is sirolimus a therapeutic option for patients with progressive pulmonary lymphangioleiomyomatosis? 2011 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © Neurohr et al; licensee BioMed Central Ltd. 2011 Background Lymphangioleiomyomatosis (LAM) is a rare lung disease characterised by progressive airflow obstruction. No effective medical treatment is available but therapy with sirolimus has shown some promise. The aim of this observational study was to evaluate sirolimus in progressive LAM. Methods Sirolimus (trough level 5 - 10 ng/ml) was administered to ten female patients (42.4 ± 11.9 years) with documented progression. Serial pulmonary function tests and six-minute-walk-distance (6-MWD) assessments were performed. Results The mean loss of $ FEV_{1} $ was -2.30 ± 0.52 ml/day before therapy and a significant mean gain of $ FEV_{1} $ of 1.19 ± 0.26 ml/day was detected during treatment (p = 0.001). Mean $ FEV_{1} $ and FVC at baseline were 1.12 ± 0.15 l (36.1 ± 4.5%pred.) and 2.47 ± 0.25 l (69.2 ± 6.5%pred.), respectively. At three and six months during follow-up a significant increase of $ FEV_{1} $ and FVC was demonstrated (3 months $ ΔFEV_{1} $: 220 ± 82 ml, p = 0.024; 6 months $ ΔFEV_{1} $: 345 ± 58 ml, p = 0.001); (3 months ΔFVC: 360 ± 141 ml, p = 0.031; 6 months ΔFVC: 488 ± 138 ml, p = 0.006). Sirolimus was discontinued in 3 patients because of serious recurrent lower respiratory tract infection or sirolimus-induced pneumonitis. No deaths and no pneumothoraces occurred during therapy. Conclusions Our data suggest that sirolimus might be considered as a therapeutic option in rapidly declining LAM patients. However, sirolimus administration may be associated with severe respiratory adverse events requiring treatment cessation in some patients. Moreover, discontinuation of sirolimus is mandatory prior to lung transplantation. Force Vital Capacity (dpeaa)DE-He213 Sirolimus (dpeaa)DE-He213 Tuberous Sclerosis Complex (dpeaa)DE-He213 Airflow Obstruction (dpeaa)DE-He213 Tuberous Sclerosis Complex Patient (dpeaa)DE-He213 Hoffmann, Anna L aut Huppmann, Patrick aut Herrera, Vivian A aut Ihle, Franziska aut Leuschner, Stefan aut von Wulffen, Werner aut Meis, Tobias aut Baezner, Carlos aut Leuchte, Hanno aut Baumgartner, Rainer aut Zimmermann, Gregor aut Behr, Juergen aut Enthalten in Respiratory research London : BioMed Central, 2001 12(2011), 1 vom: 01. Dez. (DE-627)326646485 (DE-600)2041675-1 1465-993X nnns volume:12 year:2011 number:1 day:01 month:12 https://dx.doi.org/10.1186/1465-9921-12-66 kostenfrei Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_702 GBV_ILN_2001 GBV_ILN_2003 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2008 GBV_ILN_2009 GBV_ILN_2010 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2015 GBV_ILN_2020 GBV_ILN_2021 GBV_ILN_2025 GBV_ILN_2031 GBV_ILN_2038 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2056 GBV_ILN_2057 GBV_ILN_2061 GBV_ILN_2111 GBV_ILN_2113 GBV_ILN_2153 GBV_ILN_2190 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 12 2011 1 01 12
language	English
source	Enthalten in Respiratory research 12(2011), 1 vom: 01. Dez. volume:12 year:2011 number:1 day:01 month:12
sourceStr	Enthalten in Respiratory research 12(2011), 1 vom: 01. Dez. volume:12 year:2011 number:1 day:01 month:12
format_phy_str_mv	Article
institution	findex.gbv.de
topic_facet	Force Vital Capacity Sirolimus Tuberous Sclerosis Complex Airflow Obstruction Tuberous Sclerosis Complex Patient
isfreeaccess_bool	true
container_title	Respiratory research
authorswithroles_txt_mv	Neurohr, Claus @@aut@@ Hoffmann, Anna L @@aut@@ Huppmann, Patrick @@aut@@ Herrera, Vivian A @@aut@@ Ihle, Franziska @@aut@@ Leuschner, Stefan @@aut@@ von Wulffen, Werner @@aut@@ Meis, Tobias @@aut@@ Baezner, Carlos @@aut@@ Leuchte, Hanno @@aut@@ Baumgartner, Rainer @@aut@@ Zimmermann, Gregor @@aut@@ Behr, Juergen @@aut@@
publishDateDaySort_date	2011-12-01T00:00:00Z
hierarchy_top_id	326646485
id	SPR028512685
language_de	englisch
fullrecord	<?xml version="1.0" encoding="UTF-8"?><collection xmlns="http://www.loc.gov/MARC21/slim"><record><leader>01000caa a22002652 4500</leader><controlfield tag="001">SPR028512685</controlfield><controlfield tag="003">DE-627</controlfield><controlfield tag="005">20230519212210.0</controlfield><controlfield tag="007">cr uuu---uuuuu</controlfield><controlfield tag="008">201007s2011 xx \|\|\|\|\|o 00\| \|\|eng c</controlfield><datafield tag="024" ind1="7" ind2=" "><subfield code="a">10.1186/1465-9921-12-66</subfield><subfield code="2">doi</subfield></datafield><datafield tag="035" ind1=" " ind2=" "><subfield code="a">(DE-627)SPR028512685</subfield></datafield><datafield tag="035" ind1=" " ind2=" "><subfield code="a">(SPR)1465-9921-12-66-e</subfield></datafield><datafield tag="040" ind1=" " ind2=" "><subfield code="a">DE-627</subfield><subfield code="b">ger</subfield><subfield code="c">DE-627</subfield><subfield code="e">rakwb</subfield></datafield><datafield tag="041" ind1=" " ind2=" "><subfield code="a">eng</subfield></datafield><datafield tag="100" ind1="1" ind2=" "><subfield code="a">Neurohr, Claus</subfield><subfield code="e">verfasserin</subfield><subfield code="4">aut</subfield></datafield><datafield tag="245" ind1="1" ind2="0"><subfield code="a">Is sirolimus a therapeutic option for patients with progressive pulmonary lymphangioleiomyomatosis?</subfield></datafield><datafield tag="264" ind1=" " ind2="1"><subfield code="c">2011</subfield></datafield><datafield tag="336" ind1=" " ind2=" "><subfield code="a">Text</subfield><subfield code="b">txt</subfield><subfield code="2">rdacontent</subfield></datafield><datafield tag="337" ind1=" " ind2=" "><subfield code="a">Computermedien</subfield><subfield code="b">c</subfield><subfield code="2">rdamedia</subfield></datafield><datafield tag="338" ind1=" " ind2=" "><subfield code="a">Online-Ressource</subfield><subfield code="b">cr</subfield><subfield code="2">rdacarrier</subfield></datafield><datafield tag="500" ind1=" " ind2=" "><subfield code="a">© Neurohr et al; licensee BioMed Central Ltd. 2011</subfield></datafield><datafield tag="520" ind1=" " ind2=" "><subfield code="a">Background Lymphangioleiomyomatosis (LAM) is a rare lung disease characterised by progressive airflow obstruction. No effective medical treatment is available but therapy with sirolimus has shown some promise. The aim of this observational study was to evaluate sirolimus in progressive LAM. Methods Sirolimus (trough level 5 - 10 ng/ml) was administered to ten female patients (42.4 ± 11.9 years) with documented progression. Serial pulmonary function tests and six-minute-walk-distance (6-MWD) assessments were performed. Results The mean loss of $ FEV_{1} $ was -2.30 ± 0.52 ml/day before therapy and a significant mean gain of $ FEV_{1} $ of 1.19 ± 0.26 ml/day was detected during treatment (p = 0.001). Mean $ FEV_{1} $ and FVC at baseline were 1.12 ± 0.15 l (36.1 ± 4.5%pred.) and 2.47 ± 0.25 l (69.2 ± 6.5%pred.), respectively. At three and six months during follow-up a significant increase of $ FEV_{1} $ and FVC was demonstrated (3 months $ ΔFEV_{1} $: 220 ± 82 ml, p = 0.024; 6 months $ ΔFEV_{1} $: 345 ± 58 ml, p = 0.001); (3 months ΔFVC: 360 ± 141 ml, p = 0.031; 6 months ΔFVC: 488 ± 138 ml, p = 0.006). Sirolimus was discontinued in 3 patients because of serious recurrent lower respiratory tract infection or sirolimus-induced pneumonitis. No deaths and no pneumothoraces occurred during therapy. Conclusions Our data suggest that sirolimus might be considered as a therapeutic option in rapidly declining LAM patients. However, sirolimus administration may be associated with severe respiratory adverse events requiring treatment cessation in some patients. 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author	Neurohr, Claus
spellingShingle	Neurohr, Claus misc Force Vital Capacity misc Sirolimus misc Tuberous Sclerosis Complex misc Airflow Obstruction misc Tuberous Sclerosis Complex Patient Is sirolimus a therapeutic option for patients with progressive pulmonary lymphangioleiomyomatosis?
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topic_title	Is sirolimus a therapeutic option for patients with progressive pulmonary lymphangioleiomyomatosis? Force Vital Capacity (dpeaa)DE-He213 Sirolimus (dpeaa)DE-He213 Tuberous Sclerosis Complex (dpeaa)DE-He213 Airflow Obstruction (dpeaa)DE-He213 Tuberous Sclerosis Complex Patient (dpeaa)DE-He213
topic	misc Force Vital Capacity misc Sirolimus misc Tuberous Sclerosis Complex misc Airflow Obstruction misc Tuberous Sclerosis Complex Patient
topic_unstemmed	misc Force Vital Capacity misc Sirolimus misc Tuberous Sclerosis Complex misc Airflow Obstruction misc Tuberous Sclerosis Complex Patient
topic_browse	misc Force Vital Capacity misc Sirolimus misc Tuberous Sclerosis Complex misc Airflow Obstruction misc Tuberous Sclerosis Complex Patient
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title	Is sirolimus a therapeutic option for patients with progressive pulmonary lymphangioleiomyomatosis?
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title_full	Is sirolimus a therapeutic option for patients with progressive pulmonary lymphangioleiomyomatosis?
author_sort	Neurohr, Claus
journal	Respiratory research
journalStr	Respiratory research
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author_browse	Neurohr, Claus Hoffmann, Anna L Huppmann, Patrick Herrera, Vivian A Ihle, Franziska Leuschner, Stefan von Wulffen, Werner Meis, Tobias Baezner, Carlos Leuchte, Hanno Baumgartner, Rainer Zimmermann, Gregor Behr, Juergen
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author-letter	Neurohr, Claus
doi_str_mv	10.1186/1465-9921-12-66
title_sort	is sirolimus a therapeutic option for patients with progressive pulmonary lymphangioleiomyomatosis?
title_auth	Is sirolimus a therapeutic option for patients with progressive pulmonary lymphangioleiomyomatosis?
abstract	Background Lymphangioleiomyomatosis (LAM) is a rare lung disease characterised by progressive airflow obstruction. No effective medical treatment is available but therapy with sirolimus has shown some promise. The aim of this observational study was to evaluate sirolimus in progressive LAM. Methods Sirolimus (trough level 5 - 10 ng/ml) was administered to ten female patients (42.4 ± 11.9 years) with documented progression. Serial pulmonary function tests and six-minute-walk-distance (6-MWD) assessments were performed. Results The mean loss of $ FEV_{1} $ was -2.30 ± 0.52 ml/day before therapy and a significant mean gain of $ FEV_{1} $ of 1.19 ± 0.26 ml/day was detected during treatment (p = 0.001). Mean $ FEV_{1} $ and FVC at baseline were 1.12 ± 0.15 l (36.1 ± 4.5%pred.) and 2.47 ± 0.25 l (69.2 ± 6.5%pred.), respectively. At three and six months during follow-up a significant increase of $ FEV_{1} $ and FVC was demonstrated (3 months $ ΔFEV_{1} $: 220 ± 82 ml, p = 0.024; 6 months $ ΔFEV_{1} $: 345 ± 58 ml, p = 0.001); (3 months ΔFVC: 360 ± 141 ml, p = 0.031; 6 months ΔFVC: 488 ± 138 ml, p = 0.006). Sirolimus was discontinued in 3 patients because of serious recurrent lower respiratory tract infection or sirolimus-induced pneumonitis. No deaths and no pneumothoraces occurred during therapy. Conclusions Our data suggest that sirolimus might be considered as a therapeutic option in rapidly declining LAM patients. However, sirolimus administration may be associated with severe respiratory adverse events requiring treatment cessation in some patients. Moreover, discontinuation of sirolimus is mandatory prior to lung transplantation. © Neurohr et al; licensee BioMed Central Ltd. 2011
abstractGer	Background Lymphangioleiomyomatosis (LAM) is a rare lung disease characterised by progressive airflow obstruction. No effective medical treatment is available but therapy with sirolimus has shown some promise. The aim of this observational study was to evaluate sirolimus in progressive LAM. Methods Sirolimus (trough level 5 - 10 ng/ml) was administered to ten female patients (42.4 ± 11.9 years) with documented progression. Serial pulmonary function tests and six-minute-walk-distance (6-MWD) assessments were performed. Results The mean loss of $ FEV_{1} $ was -2.30 ± 0.52 ml/day before therapy and a significant mean gain of $ FEV_{1} $ of 1.19 ± 0.26 ml/day was detected during treatment (p = 0.001). Mean $ FEV_{1} $ and FVC at baseline were 1.12 ± 0.15 l (36.1 ± 4.5%pred.) and 2.47 ± 0.25 l (69.2 ± 6.5%pred.), respectively. At three and six months during follow-up a significant increase of $ FEV_{1} $ and FVC was demonstrated (3 months $ ΔFEV_{1} $: 220 ± 82 ml, p = 0.024; 6 months $ ΔFEV_{1} $: 345 ± 58 ml, p = 0.001); (3 months ΔFVC: 360 ± 141 ml, p = 0.031; 6 months ΔFVC: 488 ± 138 ml, p = 0.006). Sirolimus was discontinued in 3 patients because of serious recurrent lower respiratory tract infection or sirolimus-induced pneumonitis. No deaths and no pneumothoraces occurred during therapy. Conclusions Our data suggest that sirolimus might be considered as a therapeutic option in rapidly declining LAM patients. However, sirolimus administration may be associated with severe respiratory adverse events requiring treatment cessation in some patients. Moreover, discontinuation of sirolimus is mandatory prior to lung transplantation. © Neurohr et al; licensee BioMed Central Ltd. 2011
abstract_unstemmed	Background Lymphangioleiomyomatosis (LAM) is a rare lung disease characterised by progressive airflow obstruction. No effective medical treatment is available but therapy with sirolimus has shown some promise. The aim of this observational study was to evaluate sirolimus in progressive LAM. Methods Sirolimus (trough level 5 - 10 ng/ml) was administered to ten female patients (42.4 ± 11.9 years) with documented progression. Serial pulmonary function tests and six-minute-walk-distance (6-MWD) assessments were performed. Results The mean loss of $ FEV_{1} $ was -2.30 ± 0.52 ml/day before therapy and a significant mean gain of $ FEV_{1} $ of 1.19 ± 0.26 ml/day was detected during treatment (p = 0.001). Mean $ FEV_{1} $ and FVC at baseline were 1.12 ± 0.15 l (36.1 ± 4.5%pred.) and 2.47 ± 0.25 l (69.2 ± 6.5%pred.), respectively. At three and six months during follow-up a significant increase of $ FEV_{1} $ and FVC was demonstrated (3 months $ ΔFEV_{1} $: 220 ± 82 ml, p = 0.024; 6 months $ ΔFEV_{1} $: 345 ± 58 ml, p = 0.001); (3 months ΔFVC: 360 ± 141 ml, p = 0.031; 6 months ΔFVC: 488 ± 138 ml, p = 0.006). Sirolimus was discontinued in 3 patients because of serious recurrent lower respiratory tract infection or sirolimus-induced pneumonitis. No deaths and no pneumothoraces occurred during therapy. Conclusions Our data suggest that sirolimus might be considered as a therapeutic option in rapidly declining LAM patients. However, sirolimus administration may be associated with severe respiratory adverse events requiring treatment cessation in some patients. Moreover, discontinuation of sirolimus is mandatory prior to lung transplantation. © Neurohr et al; licensee BioMed Central Ltd. 2011
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title_short	Is sirolimus a therapeutic option for patients with progressive pulmonary lymphangioleiomyomatosis?
url	https://dx.doi.org/10.1186/1465-9921-12-66
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author2	Hoffmann, Anna L Huppmann, Patrick Herrera, Vivian A Ihle, Franziska Leuschner, Stefan von Wulffen, Werner Meis, Tobias Baezner, Carlos Leuchte, Hanno Baumgartner, Rainer Zimmermann, Gregor Behr, Juergen
author2Str	Hoffmann, Anna L Huppmann, Patrick Herrera, Vivian A Ihle, Franziska Leuschner, Stefan von Wulffen, Werner Meis, Tobias Baezner, Carlos Leuchte, Hanno Baumgartner, Rainer Zimmermann, Gregor Behr, Juergen
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up_date	2024-07-03T19:54:14.803Z
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No effective medical treatment is available but therapy with sirolimus has shown some promise. The aim of this observational study was to evaluate sirolimus in progressive LAM. Methods Sirolimus (trough level 5 - 10 ng/ml) was administered to ten female patients (42.4 ± 11.9 years) with documented progression. Serial pulmonary function tests and six-minute-walk-distance (6-MWD) assessments were performed. Results The mean loss of $ FEV_{1} $ was -2.30 ± 0.52 ml/day before therapy and a significant mean gain of $ FEV_{1} $ of 1.19 ± 0.26 ml/day was detected during treatment (p = 0.001). Mean $ FEV_{1} $ and FVC at baseline were 1.12 ± 0.15 l (36.1 ± 4.5%pred.) and 2.47 ± 0.25 l (69.2 ± 6.5%pred.), respectively. At three and six months during follow-up a significant increase of $ FEV_{1} $ and FVC was demonstrated (3 months $ ΔFEV_{1} $: 220 ± 82 ml, p = 0.024; 6 months $ ΔFEV_{1} $: 345 ± 58 ml, p = 0.001); (3 months ΔFVC: 360 ± 141 ml, p = 0.031; 6 months ΔFVC: 488 ± 138 ml, p = 0.006). Sirolimus was discontinued in 3 patients because of serious recurrent lower respiratory tract infection or sirolimus-induced pneumonitis. No deaths and no pneumothoraces occurred during therapy. Conclusions Our data suggest that sirolimus might be considered as a therapeutic option in rapidly declining LAM patients. However, sirolimus administration may be associated with severe respiratory adverse events requiring treatment cessation in some patients. 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Is sirolimus a therapeutic option for patients with progressive pulmonary lymphangioleiomyomatosis?

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