Surfactant protein c deficiency hydroxychloroquine

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  1. kest131 Guest

    Surfactant protein c deficiency hydroxychloroquine


    The exact physiopathology of this disease is not known and there is no specific treatment for it. There is merely news from a few previous descriptions in the literature about the use of hydroxychloroquine for surfactant protein C deficiency with satisfactory results.

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    Focusing on immunomodulation and aggressive anti-inflammatory therapy, a triple-drug combination was chosen with hydroxychloroquine, azithromycin, and methylprednisolone pulse based on sporadic success in surfactant protein C deficiency. Surfactant proteins A, B, C, and D are specialized proteins that make up about 5% of the pulmonary surfactant. Surfactant protein B SP-B and C SP-C are mainly involved in preventing alveolar collapse while surfactant protein A SP-A and D SP-D play a role in the lung’s immune defense. Surfactant protein B SP-B and C SP-C are mainly involved in preventing alveolar collapse while surfactant protein A SP-A and D SP-D play a role in the lung’s immune defense. ABCA3 is a protein that transports surfactant within the alveolar Type II cell, the cell type in the lung that produces pulmonary surfactant.

    These data seem to cast some doubt on the efficacy of hydroxychloroquine, and they suggest that the clinical improvement was simply the natural evolution of the disease. Two years after the treatment was withdrawn, the twins were evaluated once again: they presented no new infections, growth and general state were normal and chest CT showed a notable additional reduction in the interstitial pneumopathy.

    Surfactant protein c deficiency hydroxychloroquine

    Childhood interstitial lung disease due to surfactant., Surfactant Deficiency chILD Foundation

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  5. Hydroxychloroquine and Surfactant Protein C Deficiency to the editor We wish to report initial success in treating a boy with surfactant protein C deficiency.

    • Hydroxychloroquine and Surfactant Protein C Deficiency.
    • Surfactant mutations chILD Foundation.
    • Is Treatment With Hydroxychloroquine Effective in..

    Hydroxychloroquine therapeutic use Infant; Lung diagnostic imaging Lung Diseases, Interstitial drug therapy etiology Male; Pulmonary Surfactant-Associated Protein C deficiency Radiography; 0 Enzyme Inhibitors 0 Pulmonary Surfactant-Associated Protein C The diagnosis of a surfactant protein C mutation c.545 GC.pGly182Arg was confirmed by genetic testing once available. Patient 1 had a family history of a maternal uncle dying of pneumonia at 6 months of age and a maternal grandfather dying of lung disease at 49 years of age, but neither one had genetic studies performed. Nogee LM, Brody AS, et al. Surfactant protein deficiency in familial interstitial lung disease. J Pediatr. 2001;1-92. 28. Rabach I, Poli F, Zennaro F, Germani C, Ventura A, Barbi E. Is treatment with hydroxychloroquine effective in surfactant protein C deficiency? Arch Bronconeumol. 2013;3-215. 29. Beers MF.

     
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