X Yin1, W K Prince2, J D Blumenfeld3, W Zhang4, S Donahue5, W O Bobb5, H Rennert6, G Askin7, I Barash3, M R Prince8. 1. Shanghai General Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China; Department of Radiology, Weill Cornell Medicine, New York, NY, USA. 2. Department of Radiology, Weill Cornell Medicine, New York, NY, USA. 3. Rogosin Institute, New York, NY, USA; Department of Medicine, Weill Cornell Medicine, New York, NY, USA. 4. Department of Radiology, Weill Cornell Medicine, New York, NY, USA; Shanghai General Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China. 5. Rogosin Institute, New York, NY, USA. 6. Department of Pathology, Weill Cornell Medicine, New York, NY, USA. 7. Department of Healthcare Policy and Research, Weill Cornell Medicine, New York, NY, USA. 8. Department of Radiology, Weill Cornell Medicine, New York, NY, USA; Columbia College of Physicians and Surgeons, New York, NY, USA. Electronic address: map2008@med.cornell.edu.
Abstract
AIM: To evaluate splenic phenotype in autosomal dominant polycystic kidney disease (ADPKD) including presence of cysts and splenomegaly to determine if these are ADPKD related or represent unrelated incidental findings. MATERIALS AND METHODS: The axial/coronal T2-weighted images of ADPKD patients (n=215) and age/gender-matched controls (n=215) were evaluated for the presence of T2-bright splenic lesions by three blinded observers. Spleen volume (SV) was evaluated in the context of clinical and imaging features as well as results of gene testing for PKD1 and PKD2 mutations. RESULTS: T2-bright splenic lesions were found in 16 of 215 (7%) ADPKD patients compared to 11 of 215 (5%) control patients (p=0.32) and their prevalence was similar in patients with either PKD1 or PKD2 mutations. Median SV was significantly higher in ADPKD patients than controls (236 [182; 313 ml] versus 176 [129; 264 ml], p<0.0001). In multivariable analysis, height-adjusted SV (htSV) was not associated with the presence of liver cysts, haemorrhagic cysts, or infections; however, htSV was directly associated with height-adjusted total kidney volume (htTKV), a biomarker for ADPKD disease severity. CONCLUSIONS: The prevalence of T2-bright splenic lesions is similar in ADPKD patients and non-ADPKD controls, suggesting no relation to the diagnosis of ADPKD; however, splenic enlargement in ADPKD compared to controls could not be explained by liver cystic involvement, by infection/inflammatory conditions, or by haemorrhagic renal cysts. This combined with direct correlation of htSV with htTKV, a biomarker of ADPKD severity, suggests splenomegaly may be related to the pathogenesis of ADPKD.
AIM: To evaluate splenic phenotype in autosomal dominant polycystic kidney disease (ADPKD) including presence of cysts and splenomegaly to determine if these are ADPKD related or represent unrelated incidental findings. MATERIALS AND METHODS: The axial/coronal T2-weighted images of ADPKDpatients (n=215) and age/gender-matched controls (n=215) were evaluated for the presence of T2-bright splenic lesions by three blinded observers. Spleen volume (SV) was evaluated in the context of clinical and imaging features as well as results of gene testing for PKD1 and PKD2 mutations. RESULTS: T2-bright splenic lesions were found in 16 of 215 (7%) ADPKDpatients compared to 11 of 215 (5%) control patients (p=0.32) and their prevalence was similar in patients with either PKD1 or PKD2 mutations. Median SV was significantly higher in ADPKDpatients than controls (236 [182; 313 ml] versus 176 [129; 264 ml], p<0.0001). In multivariable analysis, height-adjusted SV (htSV) was not associated with the presence of liver cysts, haemorrhagic cysts, or infections; however, htSV was directly associated with height-adjusted total kidney volume (htTKV), a biomarker for ADPKD disease severity. CONCLUSIONS: The prevalence of T2-bright splenic lesions is similar in ADPKDpatients and non-ADPKD controls, suggesting no relation to the diagnosis of ADPKD; however, splenic enlargement in ADPKD compared to controls could not be explained by liver cystic involvement, by infection/inflammatory conditions, or by haemorrhagic renal cysts. This combined with direct correlation of htSV with htTKV, a biomarker of ADPKD severity, suggests splenomegaly may be related to the pathogenesis of ADPKD.
Authors: Xiaorui Yin; Jon D Blumenfeld; Sadjad Riyahi; Xianfu Luo; Hanna Rennert; Irina Barash; Martin R Prince Journal: Kidney Int Rep Date: 2020-11-01
Authors: Arman Sharbatdaran; Dominick Romano; Kurt Teichman; Hreedi Dev; Syed I Raza; Akshay Goel; Mina C Moghadam; Jon D Blumenfeld; James M Chevalier; Daniil Shimonov; George Shih; Yi Wang; Martin R Prince Journal: Tomography Date: 2022-07-13