Relation between height and clinical course in Duchenne muscular dystrophy.
We have evaluated the relation between height and rate of clinical progression in boys with Duchenne muscular dystrophy (DMD). In all, 111 DMD patients with age ranging from 2 to 23 years (mean 8.2 +/- 3.4 years) were assessed; of these patients, 92 had their height measured. Clinical course was determined through Vignos scale of functional disability, motor ability, and timed functional tests. All patients had grossly elevated serum creatine-kinase (CK) and pyruvate-kinase (PK) levels. When height was adjusted for patients' age, a statistically significant correlation was found between height and clinical course (positive with Vignos scale and negative with motor ability), suggesting that smaller boys have a better clinical course than taller patients of comparable age. These results support our previous hypothesis and suggest that growth inhibition seems to be effective in diminishing the progression of DMD.
Zatz
M
M
Departamento de Biologia, Universidade de São Paulo, Brazil.
Rapaport
D
D
Vainzof
M
M
Rocha
J M
JM
Pavanello
R de C
Rde C
Colletto
G M
GM
Peres
C A
CA
D000367:Age Factors; D001835:Body Weight; D006130:Growth Disorders / Q000150:complications; D006801:Humans; D009043:Motor Activity; D009136:Muscular Dystrophies / Q000473:pathology* / Q000503:physiopathology