The value of serum creatinine as biomarker of disease progression in spinal and bulbar muscular atrophy (SBMA)

被引:7
作者
Blasi, Lorenzo [1 ]
Sabbatini, Daniele [1 ]
Fortuna, Andrea [1 ]
Querin, Giorgia [1 ,2 ]
Martinelli, Ilaria [1 ,3 ]
Vianello, Sara [1 ]
Bertolin, Cinzia [1 ,4 ]
Pareyson, Davide [5 ]
Pennuto, Maria [6 ]
Pegoraro, Elena [1 ]
Bello, Luca [1 ]
Soraru, Gianni [1 ]
机构
[1] Univ Padua, Neuromuscular Ctr, Dept Neurosci, I-35128 Padua, Italy
[2] Hop La Pitie Salpetriere, Inst Myol, I Mot Adult ClinicalTrials Platform, Paris, France
[3] Azienda Osped Univ Modena, Dept Neurosci, Neurol Unit, Modena, Italy
[4] Univ Padua, Dept Womens & Childrens Hlth, Clin Genet Unit, IRP Citta Speranza, Padua, Italy
[5] Fdn IRCCS Ist Neurol Carlo Besta, Dept Clin Neurosci, Unit Rare Neurodegenerat & Neurometab Dis, Milan, Italy
[6] Univ Padua, Dept Biomed Sci, Padua, Italy
关键词
PLASMA CREATININE; ANDROGEN RECEPTOR; MUSCLE MASS; NATURAL-HISTORY; SCALE;
D O I
10.1038/s41598-023-44419-6
中图分类号
O [数理科学和化学]; P [天文学、地球科学]; Q [生物科学]; N [自然科学总论];
学科分类号
07 ; 0710 ; 09 ;
摘要
Serum creatinine has been indicated as a potential marker of motor function in SBMA and results form previous longitudinal studies pointed to its decline over time. This is a longitudinal retrospective study investigating creatinine changes over a 36-month-period in 73 patients with SBMA. Severity and progression of the disease was assessed according to serum creatine kinase (CK) values, manual muscle testing (MMT), SBMA functional rating scale (SBMAFRS) score, 6-min-walk test (6MWT) value, and spirometry (forced vital capacity, fVC%) obtained at the baseline and at each of the annual follow-up visits. Baseline serum creatinine concentrations positively correlated with 6MWT, the MMT megascore score of both the upper (ULM) and lower (LLM) limbs and SBMAFRS. No correlation was found with CK or fVC% values. Similar correlation results were achieved at all the subsequent time points. Longitudinal assessments conducted by the generalized estimating equations (GEE) method returned significant changes for SBMAFRS (- 1.41 points per year, p < 0.001), ULM and LLM (- 0.69, p = 0.01; and - 1.07, p < 0.001, respectively), 6MWT (- 47 m, p < 0.001) but not for creatinine (- 0.82, p > 0.05). We also observed that creatinine levels at baseline did not correlate with changes in the other measures from baseline at each annual visit. Our data do not support a role for serum creatinine as sensitive biomarker of disease progression, and possibily prognosis, in SBMA.
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页数:6
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