Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Clinical Trial
. 2016 Jun;137(6):1913-1915.e2.
doi: 10.1016/j.jaci.2016.01.033. Epub 2016 Apr 4.

Pioglitazone as a novel therapeutic approach in chronic granulomatous disease

Maddalena Migliavacca  1 , Andrea Assanelli  2 , Francesca Ferrua  1 , Maria Pia Cicalese  1 , Alessandra Biffi  1 , Marta Frittoli  1 , Paolo Silvani  3 , Giovanna Chidini  4 , Edoardo Calderini  4 , Anna Mandelli  5 , Anna Camporesi  5 , Raffaella Milani  6 , Giada Farinelli  7 , Roberto Nicoletti  8 , Fabio Ciceri  2 , Alessandro Aiuti  9 , Maria Ester Bernardo  1
Affiliations
Clinical Trial

Pioglitazone as a novel therapeutic approach in chronic granulomatous disease

Maddalena Migliavacca et al. J Allergy Clin Immunol. 2016 Jun.
No abstract available

PubMed Disclaimer

Figures

Fig 1
Fig 1
Chest computed tomography scan before (A) and after (B) pioglitazone treatment. Fig 1, A, Large left pulmonary consolidation involving the inferior lobe and, partly, the dorsal and lingular segments of the superior lobe, with multiple internal air bronchograms: pneumonia. Similar pulmonary consolidation is present in the right lung and involves the dorsal segments of the inferior lobe. Fig 1, B, Remarkable reduction in the areas of consolidation in the left and right lungs; residual pulmonary lesions are visible in the left and right inferior lobes.
Fig 2
Fig 2
DHR fluorescence after stimulation with the protein kinase C activator, phorbol 12-myristate 13-acetate (PMA), before and after pioglitazone treatment for the patient (PT) and a healthy donor (HD). DHR analysis was performed on granulocytes by Phagoburts (BDBiosciences, Milan, Italy) according to manufacturer's instructions and analyzed by flow-cytometry. Red line represents the unstimulated condition; blue line indicates the PMA-stimulated condition. A, DHR results before pioglitazone treatment; representative histograms for neutrophils show 0.06% response. MFI on total granulocytes (fold increase): 0.90 in patient vs 43.02 in HD. B, DHR results at day+10 at target dose demonstrate 12.90% granulocytes with increased DHR fluorescence. MFI on total granulocytes (fold increase): 2.87 in patient vs 149.7 in HD. MFI, Mean fluorescent intensity.
See this image and copyright information in PMC

References

    1. Holland S.M. Chronic granulomatous disease. Clin Rev Allergy Immunol. 2010;38:3–10. - PubMed
    1. Di Matteo G., Giordani L., Finocchi A., Ventura A., Chiriaco M., Blancato J., IPINET (Italian Network for Primary Immunodeficiencies) Molecular characterization of a large cohort of patients with chronic granulomatous disease and identification of novel CYBB mutations: an Italian multicenter study. Mol Immunol. 2009;46:1935–1941. - PubMed
    1. Cole T., Pearce M.S., Cant A.J., Cale C.M., Goldblatt D., Gennery A.R. Clinical outcome in children with chronic granulomatous disease managed conservatively or with hematopoietic stem cell transplantation. J Allergy Clin Immunol. 2013;132:1150–1155. - PubMed
    1. Güngör T., Teira P., Slatter M., Stussi G., Stepensky P., Moshous D., Inborn Errors Working Party of the European Society for Blood and Marrow Transplantation Reduced-intensity conditioning and HLA-matched haemopoietic stemcell transplantation in patients with chronic granulomatous disease: a prospective multicentre study. Lancet. 2014;383:436–448. - PubMed
    1. Gennery A.R., Slatter M.A., Grandin L., Taupin P., Cant A.J., Veys P., Inborn Errors Working Party of the European Group for Blood and Marrow Transplantation; European Society for Immunodeficiency Transplantation of hematopoietic stem cells and long-term survival for primary immunodeficiencies in Europe: entering a new century, do we do better? J Allergy Clin Immunol. 2010;126:602–610. e1-11. - PubMed