PMID- 17222213
OWN - NLM
STAT- MEDLINE
DCOM- 20070220
LR  - 20181113
IS  - 0959-9673 (Print)
IS  - 1365-2613 (Electronic)
IS  - 0959-9673 (Linking)
VI  - 87
IP  - 6
DP  - 2006 Dec
TI  - The effects of glucocorticoid therapy on the inflammatory and dendritic cells in 
      muscular dystrophies.
PG  - 451-61
AB  - Various clinical trials have documented the therapeutic benefit of 
      glucocorticoids (GCs) in enhancing muscle strength and slowing disease 
      progression of Duchenne and Becker muscular dystrophies (DMD/BMD). We 
      hypothesized that GCs may have relevance to the differential anti-inflammatory 
      effect on mononuclear inflammatory cells (MICs) and Dendritic cells (DCs) 
      infiltrating the dystrophic muscles. In this prospective study, two muscle 
      biopsies were obtained (before and after 6-month prednisone therapy) from 30 
      patients with dystrophies (DMD = 18; BMD = 6; and limb girdle muscular 
      dystrophies (LGMD) = 6). MICs and DCs infiltrating the muscles were examined 
      using mouse monoclonal antibodies and immunoperoxidase staining methods. Muscle 
      strength was evaluated monthly by manual testing, motor ability and timed tests. 
      Prednisone therapy was associated with: (i) functional improvement of overall 
      motor disability, in upper limbs of DMD (P < 0.001) and BMD (P < 0.01) and lower 
      limbs of DMD (P < 0.001) and BMD (P < 0.05); (ii) histological improvement such 
      as fibre size variation (DMD, P < 0.01; BMD, P < 0.05), internalization of nuclei 
      (DMD, P < 0.05), degeneration and necrosis (DMD and BMD, P < 0.01), regeneration 
      (DMD, P < 0.001; BMD, P < 0.01) and endomysial connective tissue proliferation 
      (DMD, P < 0.01; BMD, P < 0.05) and (iii) reduction of total MICs (P < 0.01) and 
      DCs (P < 0.01). There was a positive correlation between the degree of 
      improvement in overall motor disability and reduction of DCs numbers (In upper 
      limbs; r = 0.638, P < 0.01 for DMD and r = 0.725, P < 0.01 for BMD, in Lower 
      limbs; r = 0.547, P < 0.05 for DMD and r = 0.576, P < 0.05 for BMD). Such 
      improvements and changes of MICs/DCs were absent in LGMD. In DMD/BMD, prednisone 
      therapeutic effect was associated with reduced MICs and DCs numbers. Whether this 
      therapeutic effect reflects targeting of the deleterious immune response produced 
      by these cells mandates further investigations.
FAU - Hussein, Mahmoud R
AU  - Hussein MR
AD  - Department of Pathology, Assiut University, Assiut, Egypt. mrh17@swissinfo.org
FAU - Hamed, Sherifa A
AU  - Hamed SA
FAU - Mostafa, Mohammed G
AU  - Mostafa MG
FAU - Abu-Dief, Eman E
AU  - Abu-Dief EE
FAU - Kamel, Nageh Fouly
AU  - Kamel NF
FAU - Kandil, Mahmoud R
AU  - Kandil MR
LA  - eng
PT  - Comparative Study
PT  - Journal Article
PL  - England
TA  - Int J Exp Pathol
JT  - International journal of experimental pathology
JID - 9014042
RN  - 0 (Glucocorticoids)
RN  - VB0R961HZT (Prednisone)
SB  - IM
MH  - Adolescent
MH  - Adult
MH  - Analysis of Variance
MH  - Biopsy
MH  - Child
MH  - Child, Preschool
MH  - Dendritic Cells/*immunology
MH  - Female
MH  - Glucocorticoids/*therapeutic use
MH  - Humans
MH  - Immunohistochemistry/methods
MH  - Male
MH  - Muscle, Skeletal/*immunology
MH  - Muscular Dystrophies/*drug therapy/*immunology
MH  - Muscular Dystrophies, Limb-Girdle/drug therapy/immunology
MH  - Muscular Dystrophy, Duchenne/drug therapy/immunology
MH  - Prednisone/*therapeutic use
MH  - Prospective Studies
MH  - Treatment Outcome
PMC - PMC2517389
EDAT- 2007/01/16 09:00
MHDA- 2007/02/21 09:00
PMCR- 2006/12/01
CRDT- 2007/01/16 09:00
PHST- 2007/01/16 09:00 [pubmed]
PHST- 2007/02/21 09:00 [medline]
PHST- 2007/01/16 09:00 [entrez]
PHST- 2006/12/01 00:00 [pmc-release]
AID - IEP470 [pii]
AID - 10.1111/j.1365-2613.2006.00470.x [doi]
PST - ppublish
SO  - Int J Exp Pathol. 2006 Dec;87(6):451-61. doi: 10.1111/j.1365-2613.2006.00470.x.