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Intravenous Infusions of Purified Alpha-1 Antitripsyn Effectively Controls Symptoms and Reverts Muscle Biopsy Changes in an MZ Alpha-1 Antitripsyn Deficiency and Fibromyalgia Syndrome Patient

Blanco Blanco, Ignacio; Astudillo, Aurora; Dominguez Iglesias, Francisco; Janciauskiene, Sabina LU ; Carcaba Fernandez, Victoriano; Gallo Alvaro, Cesar; Canto Argiz, Hortensia; de Serres, Frederick Joseph and Fernandez Bustillo, Enrique (2010) In Journal of Musculoskeletal Pain 18(2). p.167-172
Abstract
Background: An MZ phenotype alpha-1 antitrypsin [AAT] deficiency and fibromyalgia syndrome [FMS] patient participated in a trial with AAT-intravenous augmentationtherapy [AAT-IV-AT] after failure of conventional therapeutic measures. Three quadricep biopsies were performed at different stages. The first one showed large aggregates of AAT and ubiquitin in myocytes and blood vessels, and moderate muscle atrophy, before AAT-IV-AT. The two remaining biopsies were performed while receiving AAT-IV-AT. Findings: Remarkably good clinical response and histological improvement in the follow-up biopsies. Conclusions: The clinical and histopathological efficacy of AAT-IV-AT evidenced in this patient should open new perspectives of research and... (More)
Background: An MZ phenotype alpha-1 antitrypsin [AAT] deficiency and fibromyalgia syndrome [FMS] patient participated in a trial with AAT-intravenous augmentationtherapy [AAT-IV-AT] after failure of conventional therapeutic measures. Three quadricep biopsies were performed at different stages. The first one showed large aggregates of AAT and ubiquitin in myocytes and blood vessels, and moderate muscle atrophy, before AAT-IV-AT. The two remaining biopsies were performed while receiving AAT-IV-AT. Findings: Remarkably good clinical response and histological improvement in the follow-up biopsies. Conclusions: The clinical and histopathological efficacy of AAT-IV-AT evidenced in this patient should open new perspectives of research and management of AAT deficiency and FMS patients. (Less)
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author
organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
alpha-1 antitrypsin augmentation therapy, deficiency, alpha-1 antitrypsin, Fibromyalgia syndrome
in
Journal of Musculoskeletal Pain
volume
18
issue
2
pages
167 - 172
publisher
Haworth Press
external identifiers
  • wos:000279801800009
  • scopus:77952489805
ISSN
1540-7012
DOI
10.3109/10582452.2010.483962
language
English
LU publication?
yes
id
ec24f600-d0e5-40ef-a5e7-cd2107740de4 (old id 1657408)
date added to LUP
2010-08-30 13:13:38
date last changed
2018-05-29 10:02:10
@article{ec24f600-d0e5-40ef-a5e7-cd2107740de4,
  abstract     = {Background: An MZ phenotype alpha-1 antitrypsin [AAT] deficiency and fibromyalgia syndrome [FMS] patient participated in a trial with AAT-intravenous augmentationtherapy [AAT-IV-AT] after failure of conventional therapeutic measures. Three quadricep biopsies were performed at different stages. The first one showed large aggregates of AAT and ubiquitin in myocytes and blood vessels, and moderate muscle atrophy, before AAT-IV-AT. The two remaining biopsies were performed while receiving AAT-IV-AT. Findings: Remarkably good clinical response and histological improvement in the follow-up biopsies. Conclusions: The clinical and histopathological efficacy of AAT-IV-AT evidenced in this patient should open new perspectives of research and management of AAT deficiency and FMS patients.},
  author       = {Blanco Blanco, Ignacio and Astudillo, Aurora and Dominguez Iglesias, Francisco and Janciauskiene, Sabina and Carcaba Fernandez, Victoriano and Gallo Alvaro, Cesar and Canto Argiz, Hortensia and de Serres, Frederick Joseph and Fernandez Bustillo, Enrique},
  issn         = {1540-7012},
  keyword      = {alpha-1 antitrypsin augmentation therapy,deficiency,alpha-1 antitrypsin,Fibromyalgia syndrome},
  language     = {eng},
  number       = {2},
  pages        = {167--172},
  publisher    = {Haworth Press},
  series       = {Journal of Musculoskeletal Pain},
  title        = {Intravenous Infusions of Purified Alpha-1 Antitripsyn Effectively Controls Symptoms and Reverts Muscle Biopsy Changes in an MZ Alpha-1 Antitripsyn Deficiency and Fibromyalgia Syndrome Patient},
  url          = {http://dx.doi.org/10.3109/10582452.2010.483962},
  volume       = {18},
  year         = {2010},
}