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J Cyst Fibros ; 20(5): 824-827, 2021 09.
Artigo em Inglês | MEDLINE | ID: mdl-33814321

RESUMO

Optical measurement of CFTR-dependent sweat secretion stimulated by a beta-adrenergic cocktail (C-phase) vs. CFTR-independent sweat secretion induced by methacholine (M-phase) can discriminate cystic fibrosis (CF) patientts from controls and healthy carriers by the ratio of sweat rate in the C-phase vs. the M-phase (C/M ratio). However, image analysis is experimentally demanding and time-consuming. Here, sweat droplet number (SDN) in the C-phase, corresponding to the number of sweat-secreting glands, was a statistically significant predictor for detecting the effects of CFTR-targeted therapy. We show that in 44 non-CF subjects and 110 CF patients, SDN in the C-phase provides a linear readout of CFTR function that is more sensitive than that using the C/M ratio. In CF patients, increased SDN in the C-phase during treatment with (LUMA/IVA) was associated with a trend toward improved lung function (FEV1). Our method is suitable for multicenter monitoring of the effects of CFTR modulators.


Assuntos
Aminofenóis/uso terapêutico , Aminopiridinas/uso terapêutico , Benzodioxóis/uso terapêutico , Regulador de Condutância Transmembrana em Fibrose Cística/fisiologia , Fibrose Cística/diagnóstico , Fibrose Cística/tratamento farmacológico , Quinolonas/uso terapêutico , Glândulas Sudoríparas/metabolismo , Suor/metabolismo , Combinação de Medicamentos , Humanos , Óptica e Fotônica , Suor/efeitos dos fármacos , Glândulas Sudoríparas/efeitos dos fármacos
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