PMID- 36774597
OWN - NLM
STAT- MEDLINE
DCOM- 20230214
LR  - 20230214
IS  - 1669-9106 (Electronic)
IS  - 0025-7680 (Linking)
VI  - 83
IP  - 1
DP  - 2023
TI  - Analyzing respiratory muscle weakness and thoracopulmonary restriction in 
      subjects with Duchenne muscular dystrophy.
PG  - 52-58
AB  - OBJECTIVE: To analyze the underlying components of reduced maximal static 
      inspiratory (MIP) and expiratory (MEP) pressures in subjects with Duchenne 
      muscular dystrophy. METHODS: Forty-three subjects were assessed based on routine 
      pulmonary function tests. MIP and MEP were measured the subjects performed 
      maximal expirations and inspirations using a snorkel mouthpiece. Lung volumes 
      were measured using the helium dilution technique. RESULTS: The mean age was 13 
      years (range, 7-20 years). Median total lung capacity (TLC) and residual volume 
      (RV) were 78.0 (49.0-94.0) and 27.0 (19.7-30.1) of the predicted values 
      respectively. The RV/TLC relationship was 35.3% (28.1-47.7). Thirty-five subjects 
      had a TLC below the lower limit of normal, while 31 had an RV/TLC ratio above the 
      upper limit of normal. The median (IQR) MIP and MEP values were -53.0 (-65.5 to 
      -41.8) and 58.0 (41.5-74.8) cmH2O respectively. MIP and MEP in percent of the 
      predicted values (predicted TLC and RV) were 42.6 (33.3-50.8) and 33.7 
      (23.9-44.5). MIP in percent of the RV reached for Group A (7-11 years old) was 
      higher (p 0.025) while MEP in percent of the TLC reached for Group B (12-16 
      years) and C (17-20 years) were higher too (0.031). CONCLUSIONS: In subjects with 
      Duchenne muscular dystrophy, the intrinsic weakness of respiratory muscles and 
      mechanical disadvantage lead to inadequate maximal static pressure generation. 
      Maximal static pressures should be interpreted cautiously as they overestimate 
      respiratory muscle weakness when compared to predicted values obtained at TLC and 
      RV. Our results provide additional data supporting absolute values use rather 
      than predicted values.
FAU - De Vito, Eduardo L
AU  - De Vito EL
AD  - Instituto de Investigaciones Medicas Alfredo Lanari, Facultad de Medicina, 
      Universidad de Buenos Aires, Buenos Aires, Argentina. E-mail: eldevito@gmail.com.
FAU - Arce, Santiago C
AU  - Arce SC
AD  - Instituto de Investigaciones Medicas Alfredo Lanari, Facultad de Medicina, 
      Universidad de Buenos Aires, Buenos Aires, Argentina.
FAU - Sobrino, Edgardo M
AU  - Sobrino EM
AD  - Instituto de Investigaciones Medicas Alfredo Lanari, Facultad de Medicina, 
      Universidad de Buenos Aires, Buenos Aires, Argentina.
FAU - Monteiro, Sergio G
AU  - Monteiro SG
AD  - Instituto de Investigaciones Medicas Alfredo Lanari, Facultad de Medicina, 
      Universidad de Buenos Aires, Buenos Aires, Argentina.
LA  - eng
PT  - Journal Article
TT  - Analisis de la debilidad de los musculos respiratorios y de la restriccion 
      toracopulmonar en sujetos con distrofia muscular de Duchenne.
PL  - Argentina
TA  - Medicina (B Aires)
JT  - Medicina
JID - 0204271
SB  - IM
MH  - Humans
MH  - Adolescent
MH  - Child
MH  - *Muscular Dystrophy, Duchenne/complications
MH  - Respiratory Function Tests
MH  - Respiratory Muscles
MH  - Muscle Weakness/etiology
OTO - NOTNLM
OT  - Duchenne muscular dystrophy
OT  - maximal respiratory pressures
OT  - muscle weakness
OT  - neuromuscular diseases
OT  - respiratory muscles
EDAT- 2023/02/13 06:00
MHDA- 2023/02/15 06:00
CRDT- 2023/02/12 14:06
PHST- 2023/02/12 14:06 [entrez]
PHST- 2023/02/13 06:00 [pubmed]
PHST- 2023/02/15 06:00 [medline]
PST - ppublish
SO  - Medicina (B Aires). 2023;83(1):52-58.