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Plasma KL-6 reflects pulmonary severity and longitudinal response to enzyme replacement therapy in acid

Karla Cifuentes-Uribe1, Nathalie Guffon1, Lucie Boulière2

  • 1National Reference Centre for Hereditary Metabolic Diseases, Hospices Civils de Lyon, France.

Molecular Genetics and Metabolism Reports
|August 9, 2026
PubMed

Keywords:
Acid sphingomyelinase deficiencyAlveolar epithelial injuryBiomarkerEnzyme replacement therapyInterstitial lung diseaseKL-6Pulmonary diffusing capacity

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