Artículo
Andrada, L.E.; De Vito, E.L. "Clinical and spirometric alterations in Duchenne muscular dystrophy " (1996) Medicina. 56(5 I):463-471
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Abstract:
In 36 patients with Duchenne muscular dystrophy we studied the growth pattern, the type and severity of the spirometric abnormalities, the evolution of the Motor Functional Class (MFC), the infectious complications and treatments. Their age ranged from 6 to 19 years and the MFC was from 1 to 9. Regarding height, up to 12 years we verified a slope of 5.69 ± 0.58 cm/year (r 0.872 p < 0.001) and a posterior detention was observed. Of the 36 patients, 24 were below the percentile 5. The restrictive disorder prevailed. The forced vital capacity (FVC) expressed in % of the theoretical value showed a lineal fall with age, with a negative correlation (r 0.51, p < 0.01) of - 3.5 ± 0.83%/year. The deterioration of the MFC was marked starting from 6 years; with a slope of 0.84 ± 0,14 points between 6 to 12 years (r 0.73 p × 0.001). Up to 14 years, the slope was 0.212 ± 0.084 (r 0.49, p < 0.05). Patients older than 14 years had reached a greater CFM of 7; starting from this MFC a progressive fall of the VC was observed with a slope of - 15.29 ± 3.39% of CVF/CF (r 0.56, p < 0.001). Nine patients with respiratory infections were documented. Four were pneumonia and 3 of them required mechanical ventilation and died. Only 50% of the patients accepted rehabilitating treatment. Four patients accepted surgery of the alterations of the feet while the patients with deformation of the column underwent spinal stabilization.
Registro:
| Documento: | Artículo |
| Título: | Clinical and spirometric alterations in Duchenne muscular dystrophy |
| Autor: | Andrada, L.E.; De Vito, E.L. |
| Filiación: | Inst. Munic. Rehabilitacion P., Buenos Aires, Argentina Inst. de Invest. Med. Alfredo Lanari, Universidad de Buenos Aires, Argentina Servicio de Clinica Médica, Inst. Munic. Rehabilitacion P., Echeverría 955, 1428 Buenos Aires, Argentina |
| Palabras clave: | adolescent; adult; age; article; breathing; breathing disorder; child; human; male; motor activity; muscular dystrophy; pathophysiology; spirometry; Adolescent; Adult; Age Factors; Child; Humans; Male; Motor Activity; Muscular Dystrophies; Respiration; Respiration Disorders; Spirometry |
| Año: | 1996 |
| Volumen: | 56 |
| Número: | 5 I |
| Página de inicio: | 463 |
| Página de fin: | 471 |
| Título revista: | Medicina |
| Título revista abreviado: | Medicina (Argentina) |
| ISSN: | 00257680 |
| CODEN: | MEDCA |
| Registro: | https://bibliotecadigital.exactas.uba.ar/collection/paper/document/paper_00257680_v56_n5I_p463_Andrada |
Referencias:
- Cervera Radigales, C., Codina Puiggros, A., Alberca Serrano, R., Miopatias (1994) Tratado de Neurología, pp. 867-909. , Codina Puiggross A. Barcelona: Ed. Libro del an̄o
- Schmalbruch, H., The muscular dystrophies (1982) Skeletal Muscle Pathology, pp. 253-265. , Mastaglia FL, Walton Y (eds). Edinburgh: Churchill Livingstone
- Rideau, J., Jankowsky, L.W., Grellet, J., Respiratory function in the muscular dystrophies (1981) Muscle Nerve, 4, pp. 155-164
- Hapke, E.J., Meek, J.C., Jacobs, J., Pulmonary function in progressive muscular dystrophy (1972) Chest, 61, pp. 41-47
- Baydur, A., Gilgoff, I., Prentice, W., Carlson, M., Fisher, D.A., Decline in respiratory function and experience with long term assisted ventilation in advanced Duchenne's muscular dystrophy (1990) Chest, 97, pp. 884-889
- Edwards, P.R., Howard, P., Methods and prognosis of non invasive ventilation in neuromuscular disease (1993) Monaldi Arch Chest Dis, 48, pp. 176-182
- Mohr, C.H., Hill, N.S., Long-term follow-up of nocturnal ventilatory assistance in patients with respiratory failure due to Duchenne muscular dystrophy (1990) Chest, 97, pp. 91-96
- Gilroy, J., Cahalan, J.L., Berman, R., Newman, M., Cardiac and pulmonary complications in Duchenne's progressive muscular dystrophy (1963) Circulation, 27, pp. 484-493
- Smith, P.E.M., Calverley, M.B., Edwards, R.H.T., Evans, G.A., Campbell, E.J.M., Practical problems in the respiratory care of patients with muscular dystrophy (1987) N Engl J Med, 316, pp. 1197-1205
- Inkley, S.R., Oldenburg, F.C., Vignos Jr., P.J., Pulmonary function in Duchenne muscular dystrophy. Related to stage of disease (1974) Am J Med, 56, pp. 297-306
- Fukunaga, H., Sonoda, Y., Atsuchi, H., Osame, M., Respiratory failure and its care in Duchenne muscular dystrophy (1991) Rinsho-Shinkeigaku, 31, pp. 154-158
- Forst, R., Kronchen-Kaufmann, A., Forst, J., Duchenne muscular dystrophy: Contracture preventive operations of the lower extremities with special reference to anesthesiologic aspects (1991) Klin Pediatr, 203, pp. 24-27
- Arendar, G.M., Canelo, S., Paladino, D., Mendyirzycki, F., Casini, E., Aichenbaun, S., Cirugía de los miembros inferiores en la distrofia muscular progresiva. «Enfermedad de Duchenne» (1993) Rev Asoc Arg Ortop y Traumatol, 58, pp. 298-306
- Williams, E.A., Read, L., Ellis, A., Morris, P., Galasko, C.S.B., The management of equinus deformity in Duchenne muscular dystrophy (1984) J Bone Joint Surg, 66 B, pp. 546-550
- Galasko, C.S., Delaney, C., Morris, P., Spinal stabilisation in Duchenne muscular dystrophy (1992) J Bone Joint Surg, 74, pp. 210-214
- Miller, R.G., Chalmers, A.C., Dao, H., The effect of spine fusion on respiratory function in Duchenne muscular dystrophy (1991) Neurology, 41, pp. 38-40
- Kennedy, J.D., Staples, A.J., Brook, P.D., Effect of spinal surgery on lung function in Duchenne muscular dystrophy (1995) Thorax, 50, pp. 1173-1178
- Rideau, Y., Glorion, B., Delaubier, A., Tarlé, O., Bach, J., The treatment of scoliosis in Duchenne muscular dystrophy (1984) Muscle Nerve, 7, pp. 281-286
- Sansome, A., Royston, P., Dubowitz, V., Corticosteroid in Duchenne muscular dystrophy: Pilot study of a new low-dosage schedule (1993) Neuromuscul Disord, 3, pp. 567-569
- Angelini, C., Pegoraro, E., Turella, E., Intino, M.T., Pini, A., Costa, C., Deflazacort in Duchenne dystrophy: Study of long-term effect (1994) Muscle Nerve, 17, pp. 386-391
- Brooke, M.H., Fenichel, G.M., Griggs, R.C., Clinical investigation of Duchenne muscular dystrophy. Interesting results in a trial of prednisone (1987) Arch Neurol, 44, pp. 812-817
- Mesa, L.E., Dubrovsky, A.L., Corderi, J., Marco, P., Flores, D., Steroid in Duchenne muscular dystrophy-Deflazacort trial (1991) Neuromuscl Disord, 4, pp. 261-266
- Huard, J., Bouchard, J.P., Roy, R., Human myoblast transplantation: Preliminary results of 4 cases (1992) Muscle Nerve, 15, pp. 550-560
- Partridge, T.A., Davies, K.E., Myoblast-based gene therapies (1995) Br Med Bull, 51, pp. 123-137
- Nicholson, L.V.B., Davison, K., Falkous, G., Dystrophin in skeletal muscle. 1. Western blot analysis using a monoclonal antibody (1989) N Neurol Sci, 94, pp. 125-136
- Wallgren-Pettersson, C., Jasani, B., Rosse, L.G., Lazarou, L.P., Nicholson, L.V.B., Clarcke, A., Immunohistological evidence for second or somatic mutations as the underliying cause of distrophin expressio by isolated fibres in Xp21 muscular dystrophy of Duchenne-type severity (1993) J Neurol Sci, 118, pp. 56-63
- Brooke, M.H., Griggs, R.C., Mandell, J.R., Fenichel, G.M., Shumate, J.B., Pellegrino, R.J., Clinical trial in Duchenne dystrophy. 1. the design of the protocol (1981) Muscle Nerve, 4, pp. 186-197
- Hyser, C.L., Mendell, Y.R., Recent advance in Duchenne and Becker muscular dystrophy (1988) Neurologic Clinics. Muscle Disease, pp. 429-453. , Riggs YE (Guest Editor). Philadelphia: WB Saunders
- Vignos, P.J., Spencer, G.E., Archivald, K.C., Management of progressive muscular dystrophy of childhood (1963) JAMA, 184, pp. 89-110
- Johnson, B.E., Westgate, H.D., Methods of predicting Vital Capacity in patients with thoracic scoliosis (1970) J Bone Joint Surg, 52, pp. 1433-1439
- Polgar, G., Prohar, V., (1971) Pulmonary Function Testing in Children. Techniques and Standards, , Philadelphia: WB Saunders
- Perloff, J.K., Roberts, W.C., De Leon, A.C., O'Doherty, D., The distinctive electrocardiogram of Duchenne's progressive muscular dystrophy (1967) Am J Med, 42, pp. 179-188
- Perloff, J.K., De Leon, A.C., O'Doherty, D., The cardiomyopathy of progressive muscular dystrophy (1966) Circulation, 33, pp. 625-648
- Fukunaga, H., Okubo, R., Moritoyo, T., Kawashima, N., Osame, M., Long-term follow-up of patients with Duchenne muscular dystrophy receiving ventilatory support (1993) Muscle Nerve, 16, pp. 554-558
- Brooke, M.H., Fenichel, G.M., Griggs, R.C., CIDD Group: Clinical investigation in Duchenne dystrophy. 2. Determination of the «Power» of therapeutic trial based on the natural history (1983) Muscle Nerve, 6, pp. 91-103
Citas:
---------- APA ----------
Andrada, L.E. & De Vito, E.L. (1996) . Clinical and spirometric alterations in Duchenne muscular dystrophy . Medicina, 56(5 I), 463-471.Recuperado de https://bibliotecadigital.exactas.uba.ar/collection/paper/document/paper_00257680_v56_n5I_p463_Andrada [ ]
---------- CHICAGO ----------
Andrada, L.E., De Vito, E.L. "Clinical and spirometric alterations in Duchenne muscular dystrophy " . Medicina 56, no. 5 I (1996) : 463-471.Recuperado de https://bibliotecadigital.exactas.uba.ar/collection/paper/document/paper_00257680_v56_n5I_p463_Andrada [ ]
---------- MLA ----------
Andrada, L.E., De Vito, E.L. "Clinical and spirometric alterations in Duchenne muscular dystrophy " . Medicina, vol. 56, no. 5 I, 1996, pp. 463-471.Recuperado de https://bibliotecadigital.exactas.uba.ar/collection/paper/document/paper_00257680_v56_n5I_p463_Andrada [ ]
---------- VANCOUVER ----------
Andrada, L.E., De Vito, E.L. Clinical and spirometric alterations in Duchenne muscular dystrophy . Medicina (Argentina). 1996;56(5 I):463-471.Available from: https://bibliotecadigital.exactas.uba.ar/collection/paper/document/paper_00257680_v56_n5I_p463_Andrada [ ]
