Abstract
Kidney structural abnormalities are common features of Bardet-Biedl syndrome (BBS) patients that lead to a progressive decline in renal function. Magnetic resonance diffusion tensor imaging (DTI) provides useful information on renal microstructures but it has not been applied to these patients. This study investigated using DTI to detect renal abnormalities in BBS patients with no overt renal dysfunction. Ten BBS subjects with estimated glomerular filtration rates over 60 ml/min/1.73m2 and 14 individuals matched for age, gender, body mass index and renal function were subjected to high-field DTI. Fractional anisotropy (FA), and mean, radial and axial diffusivity were evaluated from renal cortex and medulla. Moreover, the corticomedullary differentiation of each DTI parameter was compared between groups. Only cortical FA statistically differed between BBS patients and controls (p = 0.033), but all the medullary DTI parameters discriminated between the two groups with lower FA (p < 0.001) and axial diffusivity (p = 0.021) and higher mean diffusivity (p = 0.043) and radial diffusivity (p < 0.001) in BBS patients compared with controls. Corticomedullary differentiation values were significantly reduced in BBS patients. Thus, DTI is a valuable tool for investigating microstructural alterations in renal disorders when kidney functionality is preserved.
Highlights
Kidney structural abnormalities are common features of Bardet-Biedl syndrome (BBS) patients that lead to a progressive decline in renal function
The water movement analysis provided with the diffusion tensor imaging (DTI) allows the measurement of quantitative parameters, such as mean diffusivity (MD) and fractional anisotropy (FA), which reflect relevant features strictly related to the functional integrity of renal structures, such as tubules, collecting ducts and v essels[18,19]
The mean body mass index (BMI) was of 26.96 kg/m2 in BBS individuals, which was not significantly different from that of the controls
Summary
Kidney structural abnormalities are common features of Bardet-Biedl syndrome (BBS) patients that lead to a progressive decline in renal function. In addition to the clinical utility of MRI examinations for the assessment of several structural kidney abnormalities (e.g., autosomal dominant polycystic kidney d isease[8] and renal c ancer9,10), recent studies have focused on the ability of kidney MRI to provide quantitative functional biomarkers for both diagnostic and prognostic purposes[11,12,13,14,15,16] In this field, diffusion tensor imaging (DTI) provides useful insights into in vivo kidney analyses by measuring, noninvasively, physical properties related to kidney tissue microstructural integrity[17]. Corticomedullary differentiation (CMD) obtained from DTI parameters was measured in patients and controls, and the right and left kidney were analysed separately using DTI parameters to assess possible asymmetrical alterations
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