Publication date: Sep 01, 2026
Acid sphingomyelinase deficiency (ASMD) type B is frequently complicated by interstitial lung disease without validated circulating pulmonary biomarkers. In this retrospective longitudinal study of five patients treated with enzyme replacement therapy, plasma KL-6 was evaluated in relation to CT-based severity, DLCO, and treatment response. Higher pre-treatment KL-6 concentrations were observed with higher exploratory CT severity grades, and KL-6 levels generally decreased or remained stable during follow-up. A transient rise occurred during SARS-CoV-2 infection. KL-6 may reflect dynamic alveolar epithelial injury in ASMD.
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Semantics
| Type | Source | Name |
|---|---|---|
| disease | MESH | acid sphingomyelinase deficiency |
| disease | MESH | ASMD |
| disease | MESH | interstitial lung disease |
| disease | MESH | SARS-CoV-2 infection |
| pathway | REACTOME | SARS-CoV-2 Infection |
| disease | MESH | injury |
| disease | MESH | Metabolic Diseases |
| disease | MESH | dyslipidemia |
| drug | DRUGBANK | Carbon monoxide |
| drug | DRUGBANK | Olipudase alfa |
| drug | DRUGBANK | Esomeprazole |
| disease | MESH | included |
| pathway | REACTOME | Metabolism |
| drug | DRUGBANK | Coenzyme M |
| disease | MESH | lung inflammation |
| disease | MESH | repeated injury |
| disease | MESH | image |
| disease | MESH | dissociation |
| drug | DRUGBANK | Ezogabine |
| disease | MESH | Dis |
| disease | MESH | sphingolipidoses |
Original Article
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