Home > Journals > European Journal of Physical and Rehabilitation Medicine > Past Issues > Europa Medicophysica 2001 June;37(2) > Europa Medicophysica 2001 June;37(2):71-81





A Journal on Physical Medicine and Rehabilitation after Pathological Events

Official Journal of the Italian Society of Physical and Rehabilitation Medicine (SIMFER), European Society of Physical and Rehabilitation Medicine (ESPRM), European Union of Medical Specialists - Physical and Rehabilitation Medicine Section (UEMS-PRM), Mediterranean Forum of Physical and Rehabilitation Medicine (MFPRM), Hellenic Society of Physical and Rehabilitation Medicine (EEFIAP)
In association with International Society of Physical and Rehabilitation Medicine (ISPRM)
Indexed/Abstracted in: CINAHL, Current Contents/Clinical Medicine, EMBASE, PubMed/MEDLINE, Science Citation Index Expanded (SciSearch), Scopus
Impact Factor 1,827




Europa Medicophysica 2001 June;37(2):71-81


language: English

Assessment of the respiratory function in individuals with Duchenne Muscular Dystrophy

Lissoni A.

From the Division of Rehabilitative Medicine Valduce Hospital, Como, Italy Rehabilitation Centre “Villa Beretta” Costa Masnaga (Lecco), Italy


Background. The time­ly diag­no­sis and the def­i­ni­tion of the ­grade of res­pir­a­to­ry insuf­fi­cien­cy in indi­vid­u­als affect­ed ­with Duchenne Muscular Dystrophy (DMD) is hin­dered by the com­plex clin­i­cal and func­tion­al con­di­tion of ­these ­patients and by the rel­a­tive rar­ity of the dis­ease ­which ­means ­that phy­si­cians do not ­always ­have an in-­depth knowl­edge of ­these prob­lems. For the ­same rea­sons dif­fi­cul­ties are encoun­tered in accom­plish­ing ade­quate fol­low-up dur­ing the pro­gres­sion of the res­pir­a­to­ry func­tion, in intro­duc­ing sig­nif­i­cant and time­ly treat­ment (usu­al­ly ­some ­form of assist­ed mechan­i­cal ven­ti­la­tion) and in mon­i­tor­ing the effec­tive­ness of the ­same. The aim of ­this ­study is to ­define the diag­nos­tic val­ue and the prog­nos­tic reli­abil­ity of sev­er­al meth­ods of func­tion­al assess­ment and to rec­og­nise ­their lim­i­ta­tions in rela­tion to pro­gres­sive ven­til­a­to­ry and res­pir­a­to­ry insuf­fi­cien­cy; pro­pos­ing, if pos­sible, an opti­mum diag­nos­tic ­path.
Methods. The ­results of func­tion­al ­tests car­ried out ­over a peri­od of ­more ­than 15 ­years on 153 DMD ­patients ­were ana­lysed. The ­data, gath­ered ­over the ­course of ­over 700 hos­pi­tal­isa­tions for diag­nos­tic-ther­a­peu­tic rea­sons, ­refer to 658 spi­rom­e­tries, 695 oxim­e­tries dur­ing ­sleep, 596 meas­ure­ments of Pimax and 350 of Pemax, 453 hae­mo­gas anal­y­ses. The ­results of the dif­fer­ent ­tests ­were ­grouped on the ­basis the ­subjects’ age in a con­tin­u­um of ­between the ­ages of 11 and 31. Means and stan­dard devi­a­tions ­were cal­cu­lat­ed, for ­each age ­group, ­from val­ues record­ed in the res­pir­a­to­ry func­tion ­tests ­most com­mon­ly ­used in the ­study of ­these ­patients. On ­this ­basis, ­graphs ­were plot­ted ­that illus­trate the ­trend ­over ­time of the ­mean val­ues of VC, VC%, MVV, MVV%, Pimax, Pemax, PaO2 and PaCO2.
Results. By com­par­ing the evo­lu­tion of the ­data col­lect­ed ­over ­time and relat­ing it to the ­basic dis­ease, the pro­gres­sive dete­ri­ora­tion of the res­pir­a­to­ry func­tion was con­firmed: the diag­nos­tic-prog­nos­tic sig­nif­i­cance of the indi­vid­u­al ­tests was ­also ver­i­fied as ­well as how the lat­ter chang­es at dif­fer­ent ­ages. In ­this way the ­most appro­pri­ate ­tests ­were iden­ti­fied on the ­basis of the ­stage of pro­gres­sion as ­well as ­those ­with less­er sig­nif­i­cance. Moreover, ­from the anal­y­sis of the graph­ic ­plots of noc­tur­nal oxim­e­tries it was pos­sible to dis­tin­guish ­which ­patients had nor­mal ­sleep pat­terns, ­which dem­on­strat­ed ­initial noc­tur­nal hypo­ven­ti­la­tion and ­which ­patients suf­fered ­from chron­ic noc­tur­nal hypo­ven­ti­la­tion: ­these ­groups record­ed dif­fer­ent con­cen­tra­tions at dif­fer­ent ­ages and con­firmed not ­only the pro­gres­sive dete­ri­ora­tion of ven­ti­la­tion dur­ing ­sleep but ­also the diag­nos­tic and prog­nos­tic use­ful­ness of the ­test.
Conclusions. Respiratory func­tion assess­ment of ­these ­patients is par­tic­u­lar­ly jus­ti­fied ­from the age of 11-12 ­years; ­between the ­ages of 11 and 20, VC val­ues, in par­tic­u­lar VC%, and Pimax val­ues are use­ful to deter­mine the resid­u­al valid­ity of the ven­ti­la­tion ­pump. Haemogas anal­y­sis, ­already ­known to be use­ful ­after the age of 17-18, ­becomes a guid­ing cri­te­ria in res­pir­a­to­ry func­tion test­ing ­after the age of 20. Studying ­sleep pat­terns ­using oxim­e­tries is use­ful ­from the age of 13 and ­vital ­after the age of 15 in sub­jects ­that do not ­have noc­tur­nal ven­til­a­to­ry assis­tance ­whilst it rep­re­sents a val­id con­fir­ma­tion of the effec­tive­ness of the treat­ment in assist­ed ­patients.

top of page

Publication History

Cite this article as

Corresponding author e-mail