Objetivos: Avaliar a reprodutibilidade dos critérios de cistos minimamente complexos, diagnosticados pela ultrassonografia, bem como sua história natural e necessidade de estudo complementar. Materiais e métodos: Levantamento dos cistos renais diagnosticados por US no Centro de Ciências da Imagem e Física Médica (CCIFM) da faculdade de Medicina de Ribeirão Preto - Universidade de São Paulo (USP), no período de janeiro de 2003 a julho de 2014, num total de 2.259 casos. Dentre estes, 143 foram descritos como cistos complexos ou minimamente complexos. Após aplicação dos critérios de inclusão e exclusão, 99 cistos foram selecionados para análises das imagens, evolução natural e levantamento de dados clínicos, que foram realizados por dois observadores principais e outros três colaboradores. Resultados: Entre as 99 lesões analisadas, 51 (51,5%) foram consideradas como cistos minimamente complexos e 48 (48,5%) como complexos. A concordância interobservador, avaliada pelo método de Kappa foi de 0.704, com intervalo de confiança de 95% entre 0.517 e 0.892, p< 0.001 (tabela 1). A idade média dos pacientes foi de 59,2 anos, variando de 21 a 93 anos. 40 pacientes eram do gênero masculino (78,4%) e 11 do gênero feminino (21,6%). Dos 51 cistos classificados como minimamente complexos, 11 foram classificados como Bosniak I pela TC/RM, 27 como Bosniak II e 6 como Bosniak 2F. Sete lesões não tiveram correlação com métodos de imagem, porém foram seguidas por pelo menos 3 anos. 25 lesões foram acompanhados por pelo menos 3 anos e nenhuma das lesões apresentou crescimento significativo no período ou mudança em sua arquitetura interna. Conclusão: Os resultados apontam que a avaliação de cistos minimamente complexos, pela US, tem boa reprodutibilidade e a complementação desta avaliação pela tomografia computadorizada ou pela ressonância magnética não é necessária, bastando o seguimento ultrassonográfico, considerando-se a evolução natural destas lesões e a melhor resolução de contraste da US em relação à TC para caracterização dos septos finos. / Objectives: To evaluate the reproducibility of minimally complex cysts criteria, diagnosed by ultrasound as well as its natural history and the need for further study. Methods: Survey of renal cysts diagnosed by US found in Centro de Ciências da Imagem e Física Médica (CCIFM) da Faculdade de Medicina de Ribeirão Preto - Universidade de São Paulo (USP), from January 2003 to July 2014, in a total of 2259 study cases. Among these, 143 cases were described as complex or minimally complex cysts. After applying the inclusion and exclusion criteria, 99 cysts were selected to image analyses, natural history and clinical data, that were performed by two main observers and three cooperators. Results: The mean age of patients was 59.2 years, ranging from 21 to 93 years. 40 patients were male (78.4%) and 11 were females (21.6%). Among the 99 analyzed lesions, 51 (51.5%) were considered as minimally complex cysts and 48 (48.5%) as a complex. The interobserver agreement assessed by the Kappa method was 0.704, with a 95% confidence interval between 0.517 and 0.892, p <0.001 (Table 1). Of the 51 cysts classified as minimally complexes, 11 were classifieds as Bosniak I by CT/ MRI, 27 as Bosniak II and 6 as Bosniak IIF. Seven cysts were not correlated with crosssectional methods, however were followed for at least 3 years. Twenty-five lesions were followed for at least three years and none of the injuries showed significant growth or internal architecture changes. Conclusion: Our results show that the evaluation of minimally complex cysts, by US, showed good reproducibility and the completion of this evaluation by CT scan or MRI is not required, since a follow up using ultrasound is safe, in view of the low malignancy rate of these lesions and US better contrast resolution compared to CT for thin septa (hair-like) characterization.

Objetivos: Avaliar a reprodutibilidade dos critérios de cistos minimamente complexos, diagnosticados pela ultrassonografia, bem como sua história natural e necessidade de estudo complementar. Materiais e métodos: Levantamento dos cistos renais diagnosticados por US no Centro de Ciências da Imagem e Física Médica (CCIFM) da faculdade de Medicina de Ribeirão Preto - Universidade de São Paulo (USP), no período de janeiro de 2003 a julho de 2014, num total de 2.259 casos. Dentre estes, 143 foram descritos como cistos complexos ou minimamente complexos. Após aplicação dos critérios de inclusão e exclusão, 99 cistos foram selecionados para análises das imagens, evolução natural e levantamento de dados clínicos, que foram realizados por dois observadores principais e outros três colaboradores. Resultados: Entre as 99 lesões analisadas, 51 (51,5%) foram consideradas como cistos minimamente complexos e 48 (48,5%) como complexos. A concordância interobservador, avaliada pelo método de Kappa foi de 0.704, com intervalo de confiança de 95% entre 0.517 e 0.892, p< 0.001 (tabela 1). A idade média dos pacientes foi de 59,2 anos, variando de 21 a 93 anos. 40 pacientes eram do gênero masculino (78,4%) e 11 do gênero feminino (21,6%). Dos 51 cistos classificados como minimamente complexos, 11 foram classificados como Bosniak I pela TC/RM, 27 como Bosniak II e 6 como Bosniak 2F. Sete lesões não tiveram correlação com métodos de imagem, porém foram seguidas por pelo menos 3 anos. 25 lesões foram acompanhados por pelo menos 3 anos e nenhuma das lesões apresentou crescimento significativo no período ou mudança em sua arquitetura interna. Conclusão: Os resultados apontam que a avaliação de cistos minimamente complexos, pela US, tem boa reprodutibilidade e a complementação desta avaliação pela tomografia computadorizada ou pela ressonância magnética não é necessária, bastando o seguimento ultrassonográfico, considerando-se a evolução natural destas lesões e a melhor resolução de contraste da US em relação à TC para caracterização dos septos finos. / Objectives: To evaluate the reproducibility of minimally complex cysts criteria, diagnosed by ultrasound as well as its natural history and the need for further study. Methods: Survey of renal cysts diagnosed by US found in Centro de Ciências da Imagem e Física Médica (CCIFM) da Faculdade de Medicina de Ribeirão Preto - Universidade de São Paulo (USP), from January 2003 to July 2014, in a total of 2259 study cases. Among these, 143 cases were described as complex or minimally complex cysts. After applying the inclusion and exclusion criteria, 99 cysts were selected to image analyses, natural history and clinical data, that were performed by two main observers and three cooperators. Results: The mean age of patients was 59.2 years, ranging from 21 to 93 years. 40 patients were male (78.4%) and 11 were females (21.6%). Among the 99 analyzed lesions, 51 (51.5%) were considered as minimally complex cysts and 48 (48.5%) as a complex. The interobserver agreement assessed by the Kappa method was 0.704, with a 95% confidence interval between 0.517 and 0.892, p <0.001 (Table 1). Of the 51 cysts classified as minimally complexes, 11 were classifieds as Bosniak I by CT/ MRI, 27 as Bosniak II and 6 as Bosniak IIF. Seven cysts were not correlated with crosssectional methods, however were followed for at least 3 years. Twenty-five lesions were followed for at least three years and none of the injuries showed significant growth or internal architecture changes. Conclusion: Our results show that the evaluation of minimally complex cysts, by US, showed good reproducibility and the completion of this evaluation by CT scan or MRI is not required, since a follow up using ultrasound is safe, in view of the low malignancy rate of these lesions and US better contrast resolution compared to CT for thin septa (hair-like) characterization.

Indiana University-Purdue University Indianapolis (IUPUI) / Type II nephronophthisis (NPHP2) is an autosomal recessive renal cystic disorder characterized by mutations in the inversin gene. Humans and mice with mutations in inversin have enlarged cystic kidneys. Increased kidney size in NPHP2 may involve altered cell growth, apoptosis, electrolyte transport and fluid accumulation in the cysts. To test this hypothesis, histology and transcriptome analysis were performed on one-day old wild-type and inv/inv mice to uncover molecular pathways altered in the mutant mice. Histology of inv/inv mice kidneys showed dilated cystic tubules compared to wild type. Pathway analysis of transcriptome data showed that inversin exerts its effects on kidneys, at least in part, through the transcriptional regulation of genes implicated in inflammation, immune response, cellular metabolism, cell cycle and ion transport. Genes involved in inflammation or immune response were upregulated whereas the genes involved in cell cycle progression and ion transport were downregulated. To validate the array findings from inv/inv mice kidneys, functional consequence of inversin loss on transepithelial ion transport was measured by electrophysiological techniques in shRNA mediated inversin-depleted renal cell type isolated from mouse cortical collecting duct (mCCD). Depletion of inversin decreased vasopressin-induced Na+ absorption, but did not alter Cl- secretion in mCCD cells. Addition of amiloride, a specific blocker of the epithelial sodium channel (ENaC), abolished basal ion transport in both inversin knockdown and control cells indicating ENaC involvement. Loss of inversin decreased Na+ absorption and this effect, in part, was mediated by transcriptional and post-translational regulation of ENaC mediators. To better understand inversin function in renal cells, transcriptome analysis was performed in control and inversin-depleted mCCD cells. Pathway analysis showed that inversin-depletion altered the genes represented in cell cycle, cellular assembly and organization, DNA replication, cell proliferation and ion transport in this isolated renal cell type. In concordance with the array data from inv/inv mice kidneys, a decrease in the expression of cell cycle, ion transport and apoptotic genes were observed accompanied by an upregulation of genes implicated in inflammatory or immune response indicating a direct effect of inversin on renal cells. Together, this study utilized a combination of transcriptome and functional analyses to unravel the role of inversin in renal cells. These data demonstrate that loss of inversin can cause a delay in cell cycle progression with a decrease in cell proliferation and apoptosis which in turn can perturb the development of the renal tubule. Also, a decrease in Na+ reabsorption together with differential regulation of other ion transporters can result in altered electrolyte transport contributing to cystogenesis, cyst growth, fluid accumulation and cyst expansion in NPHP2.

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