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The aim of this study was to evaluate the feasibility of using intravoxel incoherent motion (IVIM) imaging for noninvasive assessment of pathologic changes in chronic kidney disease (CKD). Material/Methods: Thirty-four patients with CKD and 20 healthy volunteers were examined on a 1.5 T magnetic resonance imaging (MRI) unit. The examination consisted of morphologic sequences and diffusion-weighted echo-planar sequence with 10 b values. Diffusion parameters were calculated with the use of mono-(apparent diffusion coefficient, ADC) and bi-exponential model: pure diffusion coefficient (D) and perfusion fraction (Fp). Blood samples to assess the serum creatinine level were taken immediately before examination. Ultrasound guided biopsies were performed in less than 30 days from MRI and were scored by an experienced nephropathologist. Parametrical unpaired t-test and ROC curve analysis were used to investigate differences in diffusion parameters in relation to estimated glomerular filtration rate (eGFR). Pearson's correlation coefficients were calculated to assess relationship between diffusion parameters and laboratory and histopathological markers of renal damage. P-value <0.05 indicated statistical significance. Results: Both ADC and D correlated positively with eGFR (respective r 0.74 and 0.72), however D showed a more significant correlation with histopathology: while D correlated negatively with parameters reflecting chronic glomerular (r-0.48) and tubulo-interstitial changes (r-0.47), ADC correlated only with interstitial infiltrations (r-0.44). Flow-related diffusion parameters showed high standard deviation. Conclusions: IVIM imaging is sensitive to functional and morphologic changes in CKD. The separation of influence of Fp from true diffusion improves the assessment of chronic changes in renal parenchyma.

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Atypical hemolytic uremic syndrome (aHUS) is a rare disease triggered by dysregulation of the alternative complement pathway, consisting of a characteristic triad of nonimmune hemolytic anemia, thrombocytopenia, and renal failure. The risk of aHUS onset, recurrence, and allograft loss depends on the genetic background of a patient. We show a series of cases from a single family whose five members were affected by aHUS and presented distinct clinical outcomes. Next-generation sequencing revealed combined mutations in both complement factor H and membrane cofactor protein CD46. Out of eight siblings, aHUS affected three adult brothers, and, subsequently, affected two children of an unaffected sister. The first patient died due to aHUS, and two other brothers underwent successful kidney transplantation with no aHUS recurrence. The younger, 10-month-old child presented with a severe course of the disease with cardiac involvement and persistent hemolytic anemia limited by eculizumab, while the 2-year-old recovered completely on eculizumab. The study shows a highly variable disease penetrance.