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Lucie Boulière

Hospices Civils de Lyon

3 Quai des Célestins

Lyon, 69002

France

  • Hospices Civils de Lyon

SCHOLARLY PAPERS

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Scholarly Papers (1)

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Plasma KL-6 Reflects Pulmonary Severity and Longitudinal Response to Enzyme Replacement Therapy in Acid Sphingomyelinase Deficiency Type B

Number of pages: 10 Posted: 18 Feb 2026
Karla Cifuentes-Uribe, Nathalie Guffon, Lucie Boulière, Ségolène Turquier, Cecile Acquaviva-Bourdain, Roseline Froissart and Magali Pettazzoni
Hospices Civils de Lyon, Hospices Civils de Lyon, Hospices Civils de Lyon, Hospices Civils de Lyon, Hospices Civils de Lyon, Hospices Civils de Lyon and Hospices Civils de Lyon
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Abstract:

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Acid sphingomyelinase deficiency, Alveolar epithelial injury, Biomarker, Enzyme replacement therapy, Interstitial lung disease, KL-6, Pulmonary diffusing capacity