Background and objectives: Nephrolithiasis (LIT) is more prevalent in patients with autosomal dominant polycystic kidney disease (ADPKD) than in the general population. Renal ultrasonography may underdetect renal stones because of difficulties imposed by parenchymal and/or cyst wall calcifications.Design, setting, participants, & measurements: A total of 125 patients with ADPKD underwent ultrasonography and unenhanced computed tomography (CT) scan, routine blood chemistry, and spot and 24-h urine collections.Results: CT scan detected calculi in 32 patients, including 20 whose previous ultrasonography revealed no calculi. The percentage of hypocitraturia was high but not statistically different between patients with ADPKD؉LIT or ADPKD. Hyperuricosuria and distal renal tubular acidosis were less prevalent but also did not differ between groups, whereas hyperoxaluria was significantly higher in the former. Hypercalciuria was not detected. Renal volume was significantly higher in patients with ADPKD؉LIT versus ADPKD, and a stepwise multivariate logistic regression analysis showed that a renal volume >500 ml was a significant predictor of LIT in patients with ADPKD and normal renal function, after adjustments for age and hypertension.Conclusions: CT scan was better than ultrasonography to detect LIT in patients with ADPKD. Larger kidneys from patients with ADPKD were more prone to develop stones, irrespective of the presence of metabolic disturbances.

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Background: Nephrolithiasis has been associated with bone loss and vascular calcification (VC), reflecting abnormal extraosseous calcium deposition. Fetuin-A (Fet-A) acts as a potent inhibitor of ectopic mineralization. The aim of the present study was to evaluate the prevalence of VC in stone formers (SF) and non-stone formers (NSF) and to investigate potential determinants of VC among SF, including circulating levels of Fet-A and bone microarchitecture parameters. Methods: Abdominal aortic calcification (AAC) was assessed using available computed tomography in SF and in age-, sex-, and BMI-matched NSF (potential living kidney donors). Serum Fet-A was measured in stored blood samples from SF. Bone microarchitecture parameters were obtained as a post hoc analysis of a cross-sectional cohort from young SF evaluated by high-resolution peripheral quantitative computed tomography (HR-pQCT). Results: A total of 62 SF (38.0 [28.0–45.3] years old) and 80 NSF (40.0 [37.0–45.8] years old) were included. There was no significant difference in AAC scores between SF and NSF. However, when dividing SF according to mean AAC score, below <5.8% (n = 33) or above ≥5.8% (n = 29), SF with higher AAC presented significantly higher BMI and tibial cortical porosity (Ct.Po) and significantly lower serum HDL, klotho, Fet-A, and eGFR. Urinary calcium did not differ between groups, but fractional excretion of phosphate was higher in the former. Upon multivariate regression, BMI, serum Fet-A, and tibial Ct.Po remained independently associated with AAC. Conclusions: This study suggests an association between reduced circulating Fet-A levels and increased bone Ct.Po with VC in SF.

Introduction: Renal impairment in Fabry disease is widely known, but the occurrence of renal cysts as a manifestation of the disease should still be highlighted among radiologists and other physicians. Objectives:To compare the presence of parapelvic cysts between Fabry disease and other causes of chronic kidney disease (CKD). Methods:We evaluated demographic, clinical and laboratory data, as well as kidney ultrasound (US) findings in 91 patients with Fabry disease (n=37) and different glomerulopathies (n=54).Results: Both groups were similar in age (p = 0.29), gender (p = 0.98), eGFR (p = 0.10) and CKD stages (p = 0.19). Presence of parapelvic cysts differed significantly between those two groups (p < 0.0001 in right and left kidneys; Cohen's h = 1.15). When present, diameters of the parapelvic cysts were similar. Both groups had signs of CKD such as corticomedullary undifferentiation. None of the mutations we found in GLA gene was associated with a higher prevalence of parapelvic cysts. Conclusions:When compared to different glomerulopathies, parapelvic cysts were more frequently found in Fabry disease despite age, gender and stage of CKD. Parapelvic cysts on ultrasound can raise suspicion on Fabry disease in patients with kidney disease of unknown etiology especially in the context of a familial nephropathy.

O índice de dose em tomografia computadoriza (CTDI) é empregado como indicador da dose de radiação recebida pelos pacientes submetidos a exames nessa modalidade de diagnóstico por imagem. Recentemente, organizações internacionais têm recomendado a adequação desse índice considerando o biótipo do paciente (estimativa de dose especificada pelo tamanho – SSDE) para fins de gerenciamento das doses. Este trabalho teve como objetivo a determinação desse novo indicador de dose baseado no tamanho do paciente (SSDE), calculando a espessura antero-posterior (AP) do paciente por meio de parâmetro geométrico (altura da mesa) extraído do cabeçalho DICOM dos arquivos de imagem do exame. Foram selecionados 101 exames de CT de abdome de pacientes adultos realizados em um equipamento Philips modelo Ingenuity CT com 64 canais, instalado no setor de radiologia de um hospital especializado de grande porte. Além do parâmetro de altura da mesa, foram extraídos do cabeçalho DICOM: data de nascimento e peso do paciente, tensão (kVp) e corrente (mA) aplicados no tubo, valor de CTDIvole localização do corte tomográfico. Os dados foram coletados por programa dedicado, desenvolvido em linguagem C. A espessura AP do paciente também foi determinada diretamente a partir da imagem correspondente a um corte tomográfico na região do abdome. Os resultados de SSDE encontrados indicam que 98% dos valores de CTDI estão subestimados. Considerando o tamanho dos pacientes analisados, a dose associada a esse indicador aumentou em ate 60%. Não houve diferença estatisticamente significante entre o valor estimado pela altura da mesa e pela espessura AP medida diretamente na imagem. Os resultados demonstram que os atuais valores de CTDI estão subestimados, sugerindo que a implementação prática dos valores de SSDE pode ser efetuada utilizando o valor da altura da mesa para uma estimativa mais realista da dose recebida pelos pacientes.