Id |
Subject |
Object |
Predicate |
Lexical cue |
T49 |
0-200 |
Sentence |
denotes |
In two small retrospective studies, ERT has been reported to stabilize or even ameliorate the burden of pulmonary involvement in Fabry disease, mainly bronchial obstruction and/or sleep apnea [20,21]. |
T50 |
201-343 |
Sentence |
denotes |
An interventional study of six patients receiving either ERT or placebo showed an increased exercise tolerance in patients receiving ERT [22]. |
T51 |
344-465 |
Sentence |
denotes |
Lung function parameter in five patients (2 males) on ERT were followed longitudinally for an average of five years [17]. |
T52 |
466-670 |
Sentence |
denotes |
In three patients, FVC and FEV1 increased during the treatment period, in one patient slight decrease of FVC and FEV1 were registered and in one patient a slight decrease in FVC, with an increase in FEV1. |
T53 |
671-779 |
Sentence |
denotes |
Recently, Franzen et al. [23] reported the change in z-scores of FEV1 and FEV1/FVC over time in 53 patients. |
T54 |
780-1035 |
Sentence |
denotes |
In the same study, they investigated whether factors related to age, sex, tobacco, phenotype, residual α-gal activity, age at ERT initiation, Mainz Severity Score Index (MSSI) and lyso-Gb3 levels in plasma may affect change in FEV1 and FEV1/FVC over time. |
T55 |
1036-1084 |
Sentence |
denotes |
Median spirometric follow-up time was 7.7 years. |
T56 |
1085-1169 |
Sentence |
denotes |
When considering z-scores, 27 patients (51%) developed airflow limitation over time. |
T57 |
1170-1373 |
Sentence |
denotes |
Factors including male sex, active tobacco or past history of smoking, cardiac involvement and increased patient age at ERT initiation were associated with faster FEV1 decline in the univariate analysis. |
T58 |
1374-1491 |
Sentence |
denotes |
These results suggest that earlier ERT initiation may help to stabilize FEV1 decline and preserve pulmonary function. |