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A 5-year-old female Persian cat was presented with the owner’s complaint about lethargy, anorexia, and no defecation. Physical examination revealed relatively poor general health status. Also, marked azotemia was one of the prominent findings of clinical pathology. Abdominal ultrasound revealed smooth-walled anechoic cysts in both kidneys, characteristic of feline polycystic kidney disease. Three weeks after the initiation of supportive therapy, percutaneous aspiration of large cysts was performed under the guidance of ultrasonography. The contents of the cysts were hypocellular and did not contain infectious or neoplastic cells. Following the re-examination of the patient after 30 days, the clinical and hematological parameters were notably improved. Therefore, aspiration of cysts guided by ultrasonography can be beneficial in alleviating some of the complications of polycystic kidney disease and improving the life quality of affected cats. However, it may be temporary, and this method needs to be repeated periodically.

Multicystic dysplastic kidney is a congenital anomaly typically diagnosed in infancy, rare in adults, in our case of a 25-year-old Sudanese female presented with left flank pain. Imaging showed the left kidney replaced by cysts, consistent with MCDK, and ovarian cyst. She underwent a successful open simple nephrectomy; and histopathology confirmed MCDK. Our case highlights diagnostic and management challenges of MCDK in adults. Conservative management is increasing, especially for asymptomatic patients with normal contralateral kidney function. Healthcare barriers in Sudan, including limited awareness and political unrest, complicate timely diagnosis and treatment. Multidisciplinary care and improved healthcare access are essential.

This report presents the first case of nodular dermatofibrosis with renal cysts (NDRC) in a beagle. In this atypical case, the gene mutation associated with the disease was not present, the renal cysts showed dynamic changes in size and number, and the patient has greatly surpassed the NDRC life expectation. [ABSTRACT FROM AUTHOR]

Introduction and Objectives: Congenital hepatic fibrosis (CHF) is a rare condition characterized by biliary tract changes and a geographic pattern of liver fibrosis. Liver biopsy is essential to confirm its diagnosis. The absence of specific clinical indicators in adults often leads to delays in diagnosis and management, while the natural history has not been well described. We sought to define the presentation and outcomes of adults with biopsy-proven CHF. Materials and Methods: A retrospective chart review was conducted of patients diagnosed with CHF by liver biopsy. Continuous variables were summarized with the sample median and range. Categorical variables were summarized with number and percentage of patients. Results: We identified 24 patients evaluated over a 20-year period, with a median age of 51 years (range 22-72 years) at initial presentation; 14 were male. The most common imaging findings were renal cysts (91.3%), splenomegaly (69.6%), and a cirrhotic-appearing liver (60.9%). The most commonly treated liver-related complications were cholangitis (45.8%), varices (45.8%), and hepatic encephalopathy (25%). Two patients died with a median length of follow-up of 2.9 years (range: 0.0-20.0 years). Two patients underwent transjugular intrahepatic portosystemic shunt (TIPS) placement to manage bleeding esophageal varices. Eight patients underwent liver transplantation (LT), the most common indication being decompensated disease (50%). Conclusions: CHF should be considered when patients present with cholangitis and/or complications of portal hypertension and have a cirrhotic appearing liver and renal cysts on imaging. Depending upon the disease severity, interventions such as TIPS or LT may be required. [ABSTRACT FROM AUTHOR]

Background: Nephronophthisis (NPHP) is a rare autosomal recessive inherited tubulointerstitial nephropathy, the most prevalent genetic cause of end-stage renal disease (ESRD) in children. Convincing evidence indicated that the overall prevalence of NPHP in adult-onset ESRD is very likely to be an underestimation. Therefore, understanding the genetic background and clinicopathologic features of adult-onset NPHP is warranted. Case presentation: we reported one intriguing case with concurrent NPHP3 c.2694-2_2694-1delAG (splicing) variant and c.1082C > G (p.S361C) variant. A 48-year-old male was admitted to our hospital, complained about renal dysfunction for 10 years, and found right renal space-occupying lesion for 1 week. One of the most interesting clinical features is adult-onset ESRD, which differs from previous cases. Another discovery of this study is that the NPHP harboring NPHP3 deletion may be associated with clear cell renal cell carcinoma. Conclusion: In conclusion, we report two mutations in the NPHP3 gene that cause NPHP with adult-onset ESRD and renal clear cell carcinoma in a Chinese family, enriching the clinical features of NPHP.

Abstract Background In 2019, the Bosniak classification system for cystic renal lesions underwent modifications aimed at addressing the limitations of the original classification. Results The revised 2019 version demonstrated notable differences from its predecessor. Specifically, it showed an increased proportion of class IIF cystic lesions (31% compared to 16.7%) and a decreased proportion of class III cystic lesions (27.4% compared to 45.2%). Additionally, the malignancy rate for class III cystic renal lesions was lower in the 2019 version (37.8% vs. 42.2%). When it came to diagnosing malignancies, the 2019 version exhibited higher specificity (74.4% compared to 41.03%) while maintaining a comparable sensitivity (97.8% vs. 100%) compared to the original Bosniak system. Conclusions The Bosniak 2019 version demonstrated enhanced specificity and diagnostic accuracy for malignancies in comparison to the original Bosniak system, all while maintaining an equivalent sensitivity.

Renal cyst progression in autosomal dominant polycystic kidney disease (ADPKD) is highly dependent on agents circulating in blood. We have previously shown, using different in vitro models, that one of these agents is the hormone ouabain. By binding to Na+-K+-ATPase (NKA), ouabain triggers a cascade of signal transduction events that enhance ADPKD cyst progression by stimulating cell proliferation, fluid secretion, and dedifferentiation of the renal tubular epithelial cells. Here, we determined the effects of ouabain in vivo. We show that daily administration of ouabain to Pkd1RC/RC ADPKD mice for 1–5 mo, at physiological levels, augmented kidney cyst area and number compared with saline-injected controls. Also, ouabain favored renal fibrosis; however, renal function was not significantly altered as determined by blood urea nitrogen levels. Ouabain did not have a sex preferential effect, with male and female mice being affected equally. By contrast, ouabain had no significant effect on wild-type mice. In addition, the actions of ouabain on Pkd1RC/RC mice were exacerbated when another mutation that increased the affinity of NKA for ouabain was introduced to the mice (Pkd1RC/RCNKAα1OS/OS mice). Altogether, this work highlights the role of ouabain as a procystogenic factor in the development of ADPKD in vivo, that the ouabain affinity site on NKA is critical for this effect, and that circulating ouabain is an epigenetic factor that worsens the ADPKD phenotype. NEW & NOTEWORTHY This work shows that the hormone ouabain enhances the progression of autosomal dominant polycystic kidney disease (ADPKD) in vivo. Ouabain augments the size and number of renal cysts, the kidney weight to body weight ratio, and kidney fibrosis in an ADPKD mouse model. The Na+-K+-ATPase affinity for ouabain plays a critical role in these effects. In addition, these outcomes are independent of the sex of the mice. [ABSTRACT FROM AUTHOR]

Background: In 2019, the Bosniak classification system for cystic renal lesions underwent modifications aimed at addressing the limitations of the original classification. Results: The revised 2019 version demonstrated notable differences from its predecessor. Specifically, it showed an increased proportion of class IIF cystic lesions (31% compared to 16.7%) and a decreased proportion of class III cystic lesions (27.4% compared to 45.2%). Additionally, the malignancy rate for class III cystic renal lesions was lower in the 2019 version (37.8% vs. 42.2%). When it came to diagnosing malignancies, the 2019 version exhibited higher specificity (74.4% compared to 41.03%) while maintaining a comparable sensitivity (97.8% vs. 100%) compared to the original Bosniak system. Conclusions: The Bosniak 2019 version demonstrated enhanced specificity and diagnostic accuracy for malignancies in comparison to the original Bosniak system, all while maintaining an equivalent sensitivity. [ABSTRACT FROM AUTHOR]

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Simple Summary: The present work deals with the different imaging procedures in the surveillance of renal cysts. The accuracy, interobserver variability, strengths and weaknesses of CT, MRI and CEUS in the classification of renal cysts are compared. The analysis refers to the original classification by MA Bosniak 1986, the update by GM Israel and MA Bosniak 2005, the version by SG Silverman et al. 2019, and the EFSUMB CEUS proposal by V Cantisani et al. 2021. The authors come to the conclusion that benign renal cysts, which are subject to surveillance, can and should be examined with contrast-enhanced ultrasound. CEUS is equivalent in the evaluation of renal cysts requiring surveillance compared with CE-CT and MRI. Appropriate qualification of the investigators is an unconditional requirement. MRI and CT can be used to investigate specific questions or for staging in case of surgery. It is time for a change. CEUS is an established method that should be much more actively included in renal cyst monitoring strategies. This review compares the accuracies, strengths, and weaknesses of CEUS, CECT, and MRI in the classification of renal cysts. In order to avoid overstaging by CEUS, a further differentiation of classes IIF, III, and IV is required. A further development in the refinement of the CEUS-Bosniak classification aims to integrate CEUS more closely into the monitoring of renal cysts and to develop new and complex monitoring algorithms. [ABSTRACT FROM AUTHOR]

Abstract This report presents the first case of nodular dermatofibrosis with renal cysts (NDRC) in a beagle. In this atypical case, the gene mutation associated with the disease was not present, the renal cysts showed dynamic changes in size and number, and the patient has greatly surpassed the NDRC life expectation.

Introduction and Objectives: Congenital hepatic fibrosis (CHF) is a rare condition characterized by biliary tract changes and a geographic pattern of liver fibrosis. Liver biopsy is essential to confirm its diagnosis. The absence of specific clinical indicators in adults often leads to delays in diagnosis and management, while the natural history has not been well described. We sought to define the presentation and outcomes of adults with biopsy-proven CHF. Materials and Methods: A retrospective chart review was conducted of patients diagnosed with CHF by liver biopsy. Continuous variables were summarized with the sample median and range. Categorical variables were summarized with number and percentage of patients. Results: We identified 24 patients evaluated over a 20-year period, with a median age of 51 years (range 22–72 years) at initial presentation; 14 were male. The most common imaging findings were renal cysts (91.3%), splenomegaly (69.6%), and a cirrhotic-appearing liver (60.9%). The most commonly treated liver-related complications were cholangitis (45.8%), varices (45.8%), and hepatic encephalopathy (25%). Two patients died with a median length of follow-up of 2.9 years (range: 0.0–20.0 years). Two patients underwent transjugular intrahepatic portosystemic shunt (TIPS) placement to manage bleeding esophageal varices. Eight patients underwent liver transplantation (LT), the most common indication being decompensated disease (50%). Conclusions: CHF should be considered when patients present with cholangitis and/or complications of portal hypertension and have a cirrhotic appearing liver and renal cysts on imaging. Depending upon the disease severity, interventions such as TIPS or LT may be required.

Multiple alterations of cellular metabolism have been documented in experimental studies of autosomal dominant polycystic kidney disease (ADPKD) and are thought to contribute to its pathogenesis. To elucidate the molecular pathways and transcriptional regulators associated with the metabolic changes of renal cysts in ADPKD, we compared global gene expression data from human PKD1 renal cysts, minimally cystic tissues (MCT) from the same patients, and healthy human kidney cortical tissue samples. We found gene expression profiles of PKD1 renal cysts were consistent with the Warburg effect with gene pathway changes favoring increased cellular glucose uptake and lactate production, instead of pyruvate oxidation. Additionally, mitochondrial energy metabolism was globally depressed, associated with downregulation of gene pathways related to fatty acid oxidation (FAO), branched-chain amino acid (BCAA) degradation, the Krebs cycle, and oxidative phosphorylation (OXPHOS) in renal cysts. Activation of mTORC1 and its two target proto-oncogenes, HIF-1α and MYC, was predicted to drive the expression of multiple genes involved in the observed metabolic reprogramming (e.g., GLUT3, HK1/HK2, ALDOA, ENO2, PKM, LDHA/LDHB, MCT4, PDHA1, PDK1/3, MPC1/2, CPT2, BCAT1, NAMPT); indeed, their predicted expression patterns were confirmed by our data. Conversely, we found AMPK inhibition was predicted in renal cysts. AMPK inhibition was associated with decreased expression of PGC-1α, a transcriptional coactivator for transcription factors PPARα, ERRα, and ERRγ, all of which play a critical role in regulating oxidative metabolism and mitochondrial biogenesis. These data provide a comprehensive map of metabolic pathway reprogramming in ADPKD and highlight nodes of regulation that may serve as targets for therapeutic intervention.

ARL13B is a small GTPase enriched in cilia. Deletion of Arl13b in mouse kidney results in renal cysts and an associated absence of primary cilia. Similarly, ablation of cilia leads to kidney cysts. To investigate whether ARL13B functions from within cilia to direct kidney development, we examined kidneys of mice expressing an engineered cilia-excluded ARL13B variant, ARL13BV358A. These mice retained renal cilia and developed cystic kidneys. Because ARL13B functions as a guanine nucleotide exchange factor (GEF) for ARL3, we examined kidneys of mice expressing an ARL13B variant that lacks ARL3 GEF activity, ARL13BR79Q. We found normal kidney development with no evidence of cysts in these mice. Taken together, our results show that ARL13B functions within cilia to inhibit renal cystogenesis during mouse development, and that this function does not depend on its role as a GEF for ARL3. [Display omitted] • Cilia-excluded ARL13B results in cystic kidneys. • ARL13B's GEF activity for ARL3 is not required for cyst inhibition. • Ciliary enrichment of ARL3 and TULP3 are altered when ARL13B is excluded from cilia. [ABSTRACT FROM AUTHOR]

Background: Screening for primary aldosteronism is based on measuring aldosterone-to-renin ratio. Non-suppressed renin may cause false negative screening results, and such patients may miss focused, potentially curable treatment. We investigated the association between renal cysts and non-suppressed plasma renin. Methods: Altogether, 114 consecutive patients with confirmed primary aldosteronism undergoing adrenal vein sampling were prospectively recruited between October 7, 2020 and December 30, 2021. During the procedure, plasma samples for renin analyses were collected from the right and left renal veins and the inferior vena cava. Renal cysts were identified using contrast-enhanced computed tomography. Results: Renal cysts were found in 58.2% of the 114 patients. Neither screening nor renal vein renin concentrations were significantly different in patients with and without cysts, or when the kidneys with and without cysts were evaluated. However, cysts were significantly more prevalent in the "high-normal renin" group (cut point 23.0 mU/L) than in the "low to low-normal renin" group (90.9%, n = 11 vs. 56.0%, n = 102, P = .027, respectively). All patients ≤50 years of age in the "high-normal renin" group had renal cysts. Strong correlations were found between renin concentrations in the right and left renal veins (r = .984), and between renin concentration and renin activity in the inferior vena cava (r = .817). Conclusion: Renal cysts are found in the majority of patients with primary aldosteronism, and they may interfere with diagnostics, especially in patients aged 50 years or less. In patients with non-suppressed renin due to renal cysts, aldosterone-to-renin ratio below the diagnostic threshold does not always exclude the diagnosis of primary aldosteronism. [ABSTRACT FROM AUTHOR]

Renal cysts have become more commonly recognized in asymptomatic patients due to the improvement and frequency of the use of advanced imaging modalities, such as CT and MRI. The purpose of the following review is to illustrate the main and most important aspects of renal cysts, including a checklist of top differential diagnoses and also, their further management and treatment options. Some renal cysts can have significant clinical implications; this is why increased awareness of these renal lesions is of the utmost importance in order to avoid diagnostic pitfalls or further complications. [ABSTRACT FROM AUTHOR]

Abstract Background Bosniak classification improves sensitivity and specificity for malignancy among cystic renal masses characterized with MRI. The quantitative parameters derived from diffusion-weighted imaging, and contrast enhancement, can be used in distinguishing between benign and malignant cystic renal masses. Methods This prospective observational study included 58 patients (39 male and 19 female) with complicated cystic renal mass initially diagnosed by US or CT. All patients underwent multiparametric MRI study (Pre- and Post-Gd-enhanced T1WI, T2WI and DWI) by using 3 Tesla MRI scanner. Each cystic renal lesion was assigned a category based on Bosniak classification. Demographic data were recorded. ADC ratio, dynamic enhancement parameters in both corticomedullary and nephrographic phases as well as absolute washout were calculated and compared using ROC curve analysis. Results The sensitivity, specificity, positive predictive value, negative predictive value, and overall accuracy of the multiparametric MRI in categorization of cystic renal masses according to Bosniak classification version 2019 were 90.32%, 100%, 100%, 90% and 94.83%, respectively, which was higher compared to biparametric MRI and conventional MRI. Conclusions Multiparametric MRI can be utilized to confidently evaluate cystic renal masses, overcoming the traditional limitations of overlapping morphological imaging features. Quantitative parameters derived from multiparametric MRI allow better evaluation of complex cystic renal tumors to distinguish between benign and malignant complex cystic renal lesions.

Background: Bosniak classification improves sensitivity and specificity for malignancy among cystic renal masses characterized with MRI. The quantitative parameters derived from diffusion-weighted imaging, and contrast enhancement, can be used in distinguishing between benign and malignant cystic renal masses. Methods: This prospective observational study included 58 patients (39 male and 19 female) with complicated cystic renal mass initially diagnosed by US or CT. All patients underwent multiparametric MRI study (Pre- and Post-Gd-enhanced T1WI, T2WI and DWI) by using 3 Tesla MRI scanner. Each cystic renal lesion was assigned a category based on Bosniak classification. Demographic data were recorded. ADC ratio, dynamic enhancement parameters in both corticomedullary and nephrographic phases as well as absolute washout were calculated and compared using ROC curve analysis. Results: The sensitivity, specificity, positive predictive value, negative predictive value, and overall accuracy of the multiparametric MRI in categorization of cystic renal masses according to Bosniak classification version 2019 were 90.32%, 100%, 100%, 90% and 94.83%, respectively, which was higher compared to biparametric MRI and conventional MRI. Conclusions: Multiparametric MRI can be utilized to confidently evaluate cystic renal masses, overcoming the traditional limitations of overlapping morphological imaging features. Quantitative parameters derived from multiparametric MRI allow better evaluation of complex cystic renal tumors to distinguish between benign and malignant complex cystic renal lesions. [ABSTRACT FROM AUTHOR]

Mutations in the HNF-1β gene have been found to be associated with renal cysts and diabetes syndrome (RCAD), also known as MODY5. The mutation is inherited in an autosomal dominant fashion, although sporadic mutations can be seen. Pediatric cases of HNF - 1β mutations are more likely to present with renal involvement like renal failure or renal hypoplasia. In young individuals, the detection of renal abnormalities usually pre-date the diagnosis of diabetes with a mean age of 24 years. We report a 5 year old, end stage kidney disease patient with renal cysts and hypertriglyceridemia (in the absence of overt diabetes) with a known pathogenic mutation in the Hepatocyte Nuclear Factor-1β (HNF-16) gene on chromosome 17q12. This case expands the clinical spectrum of HNF-1β mutation disorders with a take home message, that end stage renal disease patients with unexplained hypertriglyceridemia (even in absence of diabetes mellitus) should alert a clinician for HNF-1β mutational analysis. [ABSTRACT FROM AUTHOR]

Purpose: To assess the diagnostic accuracy of contrast-enhanced ultrasound (CEUS) and computed tomography (CT) within Bosniak IIF/III categories. Methods: After cystic renal mass diagnosis by contrast-enhanced CT, all patients with Bosniak score ≥ II also underwent CEUS between March 2017 and March 2019. Their exams were retrospectively analyzed. One experienced uro-radiologist performed every CEUS and reviewed the exams according to the EFSUMB 2020 Position Statement, while blinded to clinical data. CT Bosniak scores were retrospectively given blindly by two uro-radiologists (CT 1 and CT 2). We compared CEUS, CT 1 and CT 2 scores to clinical findings and histological tests. Clinical performance characteristics and area under the receiver operating characteristic (ROC) curves (AUCs) were determined separately for CEUS and CT, and then compared. Results: 101 cystic masses were analyzed. In Bosniak categories IIF and III, the AUCs were 0.854 for CT 1, 0.779 for CT 2, and 0.746 for CEUS. Conclusion: Despite some statistical limitations, this study confirms that among cystic renal masses, those classified as Bosniak IIF and III are the most difficult to assess. The diagnostic performances of CEUS and CT are similar within this group. However, in experienced hands, CEUS could be valuable in further evaluation of ambiguous cystic masses, and in more ductile, safer, and cost-effective surveillance of those classified as Bosniak IIF and III. When challenging cystic renal masses occur, CEUS is a useful tool for clinical management and for the follow-up of non-surgical lesions. [ABSTRACT FROM AUTHOR]

It is time for a change. CEUS is an established method that should be much more actively included in renal cyst monitoring strategies. This review compares the accuracies, strengths, and weaknesses of CEUS, CECT, and MRI in the classification of renal cysts. In order to avoid overstaging by CEUS, a further differentiation of classes IIF, III, and IV is required. A further development in the refinement of the CEUS-Bosniak classification aims to integrate CEUS more closely into the monitoring of renal cysts and to develop new and complex monitoring algorithms.

Yan Jing Chen,* Qin Yi,* Yi Ming Li, Li Li Department of Dermatology and Venereology, West China Hospital, Sichuan University, Chengdu, Sichuan, People’s Republic of China*These authors contributed equally to this workCorrespondence: Li LiDepartment of Dermatology and Venereology, West China Hospital, Sichuan University, Guoxue Alley No. 37, Wuhou District, Chengdu, Sichuan, 610041, People’s Republic of ChinaTel +86 18980601692Email lilihx_scu@scu.edu.cnAbstract: Nevoid basal cell carcinoma syndrome (NBCCS) is a rare autosomal dominant-inherited disease characterized by multiple basal cell carcinomas, multiple keratocystic odontogenic tumors, palmar and/or plantar pits. A 50-year-old male patient presented to our hospital with multiple plaques and maculopapular lesions on his face and trunk for more than 20 years. A skin biopsy revealed a number of discrete nests of basaloid cells in the dermis where the peripheral cells are arrayed like a palisade. Multiple odontogenic keratocysts and falx cerebri calcification were found. The diagnosis of NBCCS was made. We treated this patient with 5-aminolevulinic acid photodynamic therapy (ALA-PDT) with red light activation, 5% imiquimod cream and surgical excision for the basal cell carcinomas. All the skin lesions on his face improved substantially after eight sessions of red-light ALA-PDT from clinical observation. Red-light ALA-PDT proved to be a good therapeutic method for NBCCS in this case.Keywords: nevoid basal cell carcinoma syndrome, basal cell carcinomas, ALA-PDT, renal cysts

Renal cysts are fluid-filled cavities on the surface of kidneys which may be solitary or multiple. They may present as incidental finding during routine radiological investigations for chronic renal failure, end-stage renal disease or due to associated abdominal symptoms. During routine cadaveric dissection at the Institute, multiple cysts were observed on the surface of left kidney in a 67-year-old male cadaver. The cysts were carefully observed for gross appearance and histological examination of the cysts and biochemical analysis of cystic fluid was done. The right kidney was normal in gross appearance but was associated with double renal arteries. The left kidney, which appeared smaller, showed polycystic appearance with total 14 cysts on the surface of left kidney and distorted histology. Knowledge of renal cysts and variations in renal vascular anatomy should be kept in mind for planning effective treatment.

Crizotinib is a tyrosine kinase inhibitor that has been found to be effective in the treatment of c-ros oncogene 1-positive non-small cell lung cancer. Although this targeted agent for treating cancer has shown superiority to standard chemotherapy in some ways, this drug has adverse effects, such as the development of renal abscesses. Some associated renal damage may disappear with crizotinib withdrawal. Hence, we present the case of a 58-year-old man with non-small cell lung cancer on crizotinib therapy who developed bilateral renal abnormal space-occupying lesions, successively which were difficult to identify using various imaging methods; even PET-CT highly suspected the right renal masses as malignant. Finally, the right renal lesions were confirmed as renal abscesses by postoperative pathology. The left renal lesion was considered as renal cysts through the lesion disappearing after crizotinib withdrawal. There have been very few reports in this respect, especially proved by various methods and confirmed by postoperative pathology. It is important to recognize this drug-related complication in order to avoid incorrect diagnosis and inadequate therapy. It is necessary to monitor renal changes after taking crizotinib. [ABSTRACT FROM AUTHOR]

Purpose: Renal cysts are a frequent incidental finding on cross-sectional radiographic imaging. While most cysts are indolent, individuals with such cysts are frequently monitored for interval growth and potential malignant transformation, which is ultimately rare. In this study, we aimed to assess patients' values and preferences (believes and attitudes) about renal cysts. Methods: We deployed a cross-sectional survey to a random sample of patients with a diagnosis of renal cysts who were identified by billing code and self-identification. We collected data about demographics, insurance status, family history and overall health, and characteristics of patients with renal cysts. We performed a binary regression analysis (adjusted for age, gender, family history of cancer and kidney disease, and treatment plan for renal cysts) to determine anxiety predictors in patients with renal cysts. Results: We included 301 respondents in whom billing code and self-identification corresponded; of these, 138 had renal cysts and 163 did not. In an adjusted regression analysis, there was a suggestion that a clear management plan (OR = 0.49, 95% CI [0.22–1.11]) (p value 0.08) may be associated with less anxiety and a family history of renal disease may be associated with more anxiety (OR = 1.94 [0.76–4.94]) (p value 0.17). Family history of cancer also did not significantly predict anxiety (OR = 0.54 [0.24–1.19]) (p value 0.13). All these results were not statistically significant and had wide confidence intervals of the effect estimates make the results imprecise. Conclusion: Findings of this pilot study suggest a clear management plan for the renal cyst(s) management may be associated with a lower level of anxiety, thereby by emphasizing the importance of good communication, patient engagement and evidence-based guidance. More definitive, adequately powered studies are needed to evaluate this finding further. In addition, further studies exploring differences in imaging practices, patient symptomatology and patient engagement by different provider types would be insightful. Ultimately, tools to improve shared decision-making are needed to provide more patient-centered care. [ABSTRACT FROM AUTHOR]

Zellweger spectrum disorder (ZSD) is a rare, debilitating genetic disorder of peroxisome biogenesis that affects multiple organ systems and presents with broad clinical heterogeneity. Although severe, intermediate, and mild forms of ZSD have been described, these designations are often arbitrary, presenting difficulty in understanding individual prognosis and treatment effectiveness. The purpose of this study is to conduct a scoping review and meta-analysis of existing literature and a medical chart review to determine if characterization of clinical findings can predict severity in ZSD. Our PubMed search for articles describing severity, clinical findings, and survival in ZSD resulted in 107 studies (representing 307 patients) that were included in the review and meta-analysis. We also collected and analyzed these same parameters from medical records of 136 ZSD individuals from our natural history study. Common clinical findings that were significantly different across severity categories included seizures, hypotonia, reduced mobility, feeding difficulties, renal cysts, adrenal insufficiency, hearing and vision loss, and a shortened lifespan. Our primary data analysis also revealed significant differences across severity categories in failure to thrive, gastroesophageal reflux, bone fractures, global developmental delay, verbal communication difficulties, and cardiac abnormalities. Univariable multinomial logistic modeling analysis of clinical findings and very long chain fatty acid (VLCFA) hexacosanoic acid (C26:0) levels showed that the number of clinical findings present among seizures, abnormal EEG, renal cysts, and cardiac abnormalities, as well as plasma C26:0 fatty acid levels could differentiate severity categories. We report the largest characterization of clinical findings in relation to overall disease severity in ZSD. This information will be useful in determining appropriate outcomes for specific subjects in clinical trials for ZSD. [ABSTRACT FROM AUTHOR]

Background: Tuberous sclerosis complex (TSC) results in neurodevelopmental phenotypes, benign tumors, and cysts throughout the body. Recent studies show numerous rare findings in TSC. Guidelines suggest routine abdominal and chest imaging to monitor these thoracoabdominal findings, but imaging is not uniformly done across centers. Thus, the prevalence of many findings is unknown. To answer this, we categorized the clinical reads of 1398 thoracoabdominal scans from 649 patients of all ages in the Cincinnati Children's Hospital TSC Repository Database.Results: Typical TSC findings were present in many patients: kidney cysts (72%), kidney fat-containing angiomyolipomas (51%), kidney lipid-poor angiomyolipomas (27%), liver angiomyolipomas (19%), and lung nodules thought to represent multifocal micronodular pneumocyte hyperplasia (MMPH) (18%). While many features were more common in TSC2 patients, TSC1 patients had a higher prevalence of MMPH than TSC2 patients (24% versus 13%, p = 0.05). Many rare findings (e.g., lymphatic malformations and liver masses) are more common in TSC than in the general population. Additionally, most thoracoabdominal imaging findings increased with age except kidney cysts which decreased, with the 0-10 years age group having the highest percentage (69% 0-10 years, 49% 10-21 years, 48% 21 + years, p < 0.001). Finally, in our population, no patients had renal cell carcinoma found on abdominal imaging.Conclusions: These results show that regular thoracoabdominal scans in TSC may show several findings that should not be ignored or, conversely, over-reacted to when found in patients with TSC. Female sex, TSC2 mutation, and age are risk factors for many thoracoabdominal findings. The data suggest novel interactions of genetic mutation with pulmonary nodules and age with renal cysts. Finally, in agreement with other works, these findings indicate that several rare thoracoabdominal imaging findings occur at higher rates in the TSC population than in the general population. This work supports obtaining detailed thoracoabdominal imaging in patients with TSC. [ABSTRACT FROM AUTHOR]

Purpose: Simple renal cysts are common benign lesions that arise from the renal parenchyma. Cyst growth can lead to confusion as well as concern from patients and referring providers about the need for imaging follow-up or additional evaluation. The purpose of this study was to evaluate the natural history of simple renal cysts and determine the best metric to characterize cyst evolution. Methods: 222 simple renal cysts in 182 adults (age = 58.4 ± 6.0 years) were longitudinally evaluated on non-contrast CT examinations over a mean interval of 7.5 ± 2.8 years. Axial long axis, surface area, and volume were evaluated at baseline and follow-up CT examinations. Absolute and percent annualized growth rates were computed between CT studies for each parameter. Results: At baseline CT examinations, mean (± SD) axial long axis, surface area, and volume measurements were 2.5 ± 1.7 cm, 2.5 ± 4.5 cm2, and 17.6 ± 52.5 ml, respectively. On follow-up examinations, measurements were 3.4 ± 2.0 cm, 4.2 ± 5.9 cm2, and 34.4 ± 92.3 ml, respectively. Significant differences (p < 0.01) were found between baseline and follow-up values for each parameter. The absolute growth rate of each parameter was + 0.1 ± 0.1 cm/year, + 2.1 ± 3.4 cm2/year, and + 2.0 ± 5.6 ml/year, respectively. The percent annualized growth rate for each parameter was +6.5 ± 7.3%/year, +18 ± 24%/year, and +46 ± 100%/year, respectively. Overall, 86% (190/222) of cysts increased in size over time; most notably 78% (174/222) increased by ≥ 6% in volume per year. None of the simple cysts developed septations or solid components on follow-up examinations. Conclusion: The majority of simple renal cysts increase in size over time, which was not associated with the development of complex features. Surface area and volume are the parameters most indicative of cyst growth or regression over time. In patients with enlarging asymptomatic simple renal cysts, no follow-up imaging is indicated. [ABSTRACT FROM AUTHOR]

A high prevalence of AA-amyloidosis was identified in a breeding colony of northern tree shrews (Tupaia belangeri) in a retrospective analysis, with amyloid deposits in different organs being found in 26/36 individuals (72%). Amyloid deposits, confirmed by Congo red staining, were detected in kidneys, intestines, skin, and lymph nodes, characteristic of systemic amyloidosis. Immunohistochemically, the deposited amyloid was intensely positive with anti-AA-antibody (clone mc4), suggesting AA-amyloidosis. The kidneys were predominantly affected (80%), where amyloid deposits ranged from mild to severe and was predominantly located in the renal medulla. In addition, many kidneys contained numerous cysts with atrophy of the renal parenchyma. There was no significant association between concurrent neoplastic or inflammatory processes and amyloidosis. The lack of distinctive predisposing factors suggests a general susceptibility of captive T. belangeri to develop amyloidosis. Clinical and laboratory findings of a female individual with pronounced kidney alterations were indicative of renal failure. The observed tissue tropism with pronounced kidney alterations, corresponding renal dysfunction, and an overall high prevalence suggests amyloidosis as an important disease in captive tree shrews. [ABSTRACT FROM AUTHOR]

There are currently several strategies for the treatment of symptomatic simple renal cysts, such as aspiration with sclerosants and laparoscopic deroofing. However, no clear indication exists for choosing between them. Here, we carried out a systematic review and a meta-analysis of studies, which compared symptomatic and radiological success between aspiration with sclerotherapy and laparoscopic deroofing. Results were reported as relative risk (RR) and 95% confidence interval (95% CI) using laparoscopic deroofing as control group. The symptomatic and radiological successes were evaluated by 6 and 3 studies, respectively. Notably, aspiration with sclerotherapy was associated with higher risk of failure than laparoscopic deroofing (RR = 2.82; 95% CI = 1.84–4.31 for symptomatic failure; RR = 8.31; 95% CI = 4.22–16.38 for radiological failure). On the other hand, however, aspiration with sclerotherapy was associated with less frequent complications, shorter treatment duration and post-treatment hospital stay, and lower costs. Thus, our work underlines benefits and drawbacks of each intervention, raising the need for further studies to design guidelines for the management of simple renal cysts. [ABSTRACT FROM AUTHOR]

Abstract Background To compare the clinical efficiency between aspiration-sclerotherapy (AS) and laparoscopic de-roofing (LD) in the management of renal cysts through meta-analysis of comparative studies. Method A comprehensive literature search was performed by PubMed, MEDLINE, Ovid and Web of Science for relevant studies published up to January 2020. The statistical analyses were conducted with Review Manager 5.3.0 and Stata 15.1. The sensitivity analysis was also carried out to confirm the reliability of this Meta-analysis. Results Our searches of literature generated 6 studies (1547 patients incorporated) comparing AS with LD in the impacts of renal cyst therapy. Of these, 6 studies contained 1106 and 441 patients who were treated with AS and LD, respectively. The outcome of this meta-analysis indicated that LD group was superior in symptomatic successful rate [Odds Ratio (OR): 0.28; 95%Confidence Interval (CI): 0.09 to 0.86; P = 0.03), radiological successful rate (OR: 0.06; 95%CI: 0.02 to 0.15; P

Pietro Di Marino,1 Gianluca Mannetta,2 Consiglia Carella,3 Antonino Grassadonia,3 Nicola Tinari,3 Clara Natoli,3 Michele De Tursi3 1Clinical Oncology Unit, S.S. Annunziata Hospital, Chieti, Italy; 2Department of Neuroscience, Imaging and Clinical Sciences, “G. d’Annunzio” University, Chieti, Italy; 3Department of Medical, Oral and Biotechnological Sciences University “G. D’Annunzio”, Chieti-Pescara, ItalyCorrespondence: Pietro Di MarinoClinical Oncology Unit, PO SS Annunziata, Via Dei Vestini 5, Chieti 66100, ItalyTel +39 871 35 8005Fax +39 871 35 8476Email pietrodimarino@gmail.comBackground: Crizotinib is the first tyrosine kinase inhibitor approved for the treatment of anaplastic lymphoma kinase (ALK) rearranged non-small cell lung cancer (NSCLC). An increased incidence of renal cysts has been described during the crizotinib treatment.Case Presentation: We herein report the case of a 74-year-old woman who received crizotinib for metastatic ALK-positive NSCLC. During the crizotinib treatment, complex renal cystic lesions with invasion of perirenal spaces and iliopsoas muscle appeared; two complex hepatic cysts were also observed. Almost all lesions disappeared after switching to alectinib, a second-generation ALK inhibitor.Conclusion: It would seem that alectinib is able to reduce in size and number hepatic and renal cysts caused by the crizotinib treatment. Nevertheless, further studies are needed to clarify the role of both crizotinib in the onset of renal and hepatic cysts and alectinib in their disappearance.Keywords: anaplastic lymphoma kinase, crizotinib, alectinib, non-small-cell lung cancer, renal cysts, hepatic cysts

Background: Hypertension is one of the major world health problems. Ultrasonography plays a useful role in the assessment of morphologicalchanges at the kidneys in hypertensive patients. Aim: To assess sonographic findings of the kidneys in hypertensive patients’ co-morbidities with type 2 diabetes mellitus and ischemic heart disease (IDH). Materials and Methods: This was a prospective cross-sectional study involved 100 participants with primary hypertension selected by a method of simple convenient sampling. The patients were examined using ultrasonography to assess the sonographic findings of the kidneys. The renal length, corticomedullary differentiation (CMD), and renal artery diameters were assessed. Statistical Package for the Social Sciences (SPSS version 23.0) was used in data analysis. Results: The length of the right kidney was 8.9850 ± 1.01 cm and 9.48 ± 0.98 cm for the left kidney. Among the hypertensive patients, the incidence of hypertension was highest in housewives (27%) and students (23%) as compared with the other groups. The sonographic findings were 18% affected with simple renal cysts, 7% poor CMD, and the majority had normal kidneys. Significant correlation was found between age and sonographic findings of kidneys (r = 0.21, P value = 0.033). Conclusion: Simple renal cysts, poor CMD, and stenosis of renal arteries were most common sonographic findings in hypertension. Simple renal cystshad a significant association with hypertension.

Objective: To evaluate the relationship between lesion size and malignancy rates in surgically removed Bosniak 3 renal cysts. Material and Methods: Patients who underwent radical / partial nephrectomy for cystic renal mass between January 2010 and December 2018 were retrospectively analyzed. Fifty two patients who were diagnosed as Bosniak 3 renal cyst in preoperative computed tomography (CT) and / or Magnetic Resonance Imaging (MRI) images were included in the study. ROC Curve analysis was performed among Bosniak 3 lesions and tumor size cut-off value predicting malignancy was found. Patients with tumor size below and above this value were compared in terms of patient characteristics and histopathological data. Results: The mean patient age was 54.2 ± 13.1 years and the mean follow-up period was 39.9 ± 25.2 months. The malignancy rate for all Bosniak 3 lesions was 63.5%. Male gender and low tumor size were correlated with malignancy in both univariate and multivariate analysis in comparison of patients with malignant and benign pathology. In the ROC Curve analysis, the cut-off value of the cyst, which predicted malignancy, was 45 mm (AUC = 0.735, p = 0.005). Patients were divided into two groups according to tumor size ≤45 mm (Group 1) and > 45 mm (Group 2). The malignancy rate was 77.8% and 48% in Group 1 and 2, respectively (p = 0.026, OR: 0.26, CI: 0.079-0.875). Conclusion: In Bosniak 3 renal cysts, low tumor size was found to be associated with malignancy.

Multiple alterations of cellular metabolism have been documented in experimental studies of autosomal dominant polycystic kidney disease (ADPKD) and are thought to contribute to its pathogenesis. To elucidate the molecular pathways and transcriptional regulators associated with the metabolic changes of renal cysts in ADPKD, we compared global gene expression data from human PKD1 renal cysts, minimally cystic tissues (MCT) from the same patients, and healthy human kidney cortical tissue samples. We found gene expression profiles of PKD1 renal cysts were consistent with the Warburg effect with gene pathway changes favoring increased cellular glucose uptake and lactate production, instead of pyruvate oxidation. Additionally, mitochondrial energy metabolism was globally depressed, associated with downregulation of gene pathways related to fatty acid oxidation (FAO), branched-chain amino acid (BCAA) degradation, the Krebs cycle, and oxidative phosphorylation (OXPHOS) in renal cysts. Activation of mTORC1 and its two target proto-oncogenes, HIF-1α and MYC, was predicted to drive the expression of multiple genes involved in the observed metabolic reprogramming (e.g., GLUT3 , HK1/HK2 , ALDOA , ENO2 , PKM , LDHA/LDHB , MCT4 , PDHA1 , PDK1/3 , MPC1/2 , CPT2 , BCAT1 , NAMPT ); indeed, their predicted expression patterns were confirmed by our data. Conversely, we found AMPK inhibition was predicted in renal cysts. AMPK inhibition was associated with decreased expression of PGC-1α, a transcriptional coactivator for transcription factors PPARα, ERRα, and ERRγ, all of which play a critical role in regulating oxidative metabolism and mitochondrial biogenesis. These data provide a comprehensive map of metabolic pathway reprogramming in ADPKD and highlight nodes of regulation that may serve as targets for therapeutic intervention.

Background: Up until now, no research has been reported on the association between the clinical growth rate of multilocular cystic renal neoplasm of low malignant potential (MCRNLMP) and computed tomography (CT) imaging characteristics. Our study sought to examine the correlation between them, with the objective of distinguishing unique features of MCRNLMP from renal cysts and exploring effective management strategies., Aim: To investigate optimal management strategies of MCRNLMP., Methods: We retrospectively collected and analyzed data from 1520 patients, comprising 1444 with renal cysts and 76 with MCRNLMP, who underwent renal cyst decompression, radical nephrectomy, or nephron-sparing surgery for renal cystic disease between January 2013 and December 2021 at our institution. Detection of MCRNLMP utilized the Bosniak classification for imaging and the 2016 World Health Organization criteria for clinical pathology., Results: Our meticulous exploration has revealed compelling findings on the occurrence of MCRNLMP. Precisely, it comprises 1.48% of all cases involving simple renal cysts, 5.26% of those with complex renal cysts, and a noteworthy 12.11% of renal tumors coexisting with renal cysts, indicating a statistically significant difference ( P = 0.001). Moreover, MCRNLMP constituted a significant 22.37% of the patient population whose cysts demonstrated a rapid growth rate of ≥ 2.0 cm/year, whereas it only represented 0.66% among those with a growth rate below 2.0 cm/year. Of the 76 MCRNLMP cases studied, none of the nine patients who underwent subsequent nephron-sparing surgery or radical nephrectomy following renal cyst decompression experienced recurrence or metastasis. In the remaining 67 patients, who were actively monitored over a 3-year postoperative period, only one showed suspicious recurrence on CT scans., Conclusion: MCRNLMP can be tentatively identified and categorized into three types based on CT scanning and growth rate indicators. In treating MCRNLMP, partial nephrectomy is preferred, while radical nephrectomy should be minimized. After surgery, active monitoring is advisable to prevent unnecessary nephrectomy., Competing Interests: Conflict-of-interest statement: All authors have no conflicts of interest to disclose., (©The Author(s) 2024. Published by Baishideng Publishing Group Inc. All rights reserved.)

Renal ciliopathies are a group of genetic disorders that affect the function of the primary cilium in the kidney, as well as other organs. Since primary cilia are important for regulation of cell signaling pathways, ciliary dysfunction results in a range of clinical manifestations, including renal failure, cyst formation, and hypertension. We summarize the current understanding of the pathophysiological and pathological features of renal ciliopathies in childhood, including autosomal dominant and recessive polycystic kidney disease, nephronophthisis, and Bardet-Biedl syndrome, as well as skeletal dysplasia associated renal ciliopathies. The genetic basis of these disorders is now well-established in many cases, with mutations in a large number of cilia-related genes such as PKD1 , PKD2 , BBS , MKS , and NPHP being responsible for the majority of cases. Renal ciliopathies are broadly characterized by development of interstitial fibrosis and formation of multiple renal cysts which gradually enlarge and replace normal renal tissue, with each condition demonstrating subtle differences in the degree, location, and age-related development of cysts and fibrosis. Presentation varies from prenatal diagnosis of congenital multisystem syndromes to an asymptomatic childhood with development of complications in later adulthood and therefore clinicopathological correlation is important, including increasing use of targeted genetic testing or whole genome sequencing, allowing greater understanding of genetic pathophysiological mechanisms., Competing Interests: Declaration of Conflicting InterestsThe authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Background: Cystic lesions of the kidney are relatively frequent and may represent benign cysts, premalignantlesions, or malignant neoplasms. When asingle layer of cuboidal or flattened cells lines a cystic lesion, they are considered simple renal cortical cysts. Aim Of The Work: Our study aims to determine the frequency and associated risk factors of renal cysts. Patients&Methods: A cross-sectional study including 242 subjects undergoing abdominal CT, aging more than 18 years, and with no history of medical diseases. All involved patients were subjected to Routine investigations, including Random bloodsugar, postprandial bloodglucose., HbA1c, Complete bloodpicture, Serum creatinine, urea, andcholesterol in addition to Complete Urineanalysis. All participants were subjected to Computed tomography (CT)abdomen. Results:18.6% of the studied group had Pyuria, 29.5% had Urate crystal, 14% had microscopic hematuria, 1.2% had gross hematuria, and 29.3% had proteinuria. 20.2% (49 cases) had cyst among the studied population. Cyst cases were significantly associated with older age, male sex, smoking, diabetes mellitus, hypertension, and longer duration of treatment of DM and HTN. RBS, 2PP BS, HbA1C, Cholesterol, Cr, and Urea were significantly higher among cases with the cyst. Also, cases with cysts were significantly associated with Pyuria and Proteinuria. Conclusion: Significant risk factors for the occurrence of renal cysts include older age, male sex, hypertension, diabetes mellitus, smoking, longer DM or HTN TTTduration. Serum Urea, Creatinine, Cholesterol, urinary Pus, and Proteinuria are also significant factors affecting renalcysts. However, BMI, chronic use of NSAIDs, CBC findings, urate crystals, and hematuria are not statistically significant factors affecting renalcysts. [ABSTRACT FROM AUTHOR]

UnlabelledNephrotoxicity is a common adverse effect of many chemotherapeutic agents. The agents most commonly associated with chemotherapy-associated nephrotoxicity are methotrexate, semustine, streptozocin, mithramycin, and cisplatin. Certain chemotherapeutic agents have adverse effects on the kidneys and urothelium that can be visualized radiographically, including cystic change, interstitial nephritis, papillary necrosis, urothelial changes, haemorrhagic cystitis, acute tubular necrosis, and infarction. This review focuses on imaging features identifying complications of chemotherapy in the kidneys and collecting system and provides didactic cases to alert referring clinicians.Teaching points• Nephrotoxicity is a common adverse effect of many chemotherapeutic agents. • Chemotherapies have adverse renal and urothelial effects that can be visualized radiographically. • Crizotinib use can result in the development of complex renal cysts.

Abstract Non‐small cell lung cancer patients with anaplastic lymphoma kinase or c‐ros oncogene 1 mutations who are treated with the tyrosine kinase inhibitor crizotinib rarely develop crizotinib‐associated renal cysts (CARCs). Here, we present a case report and review of the literature supporting the hypothesis that CARCs may correlate positively with progression‐free survival.

Polycystic kidney disease with Tuberous sclerosis is a disease caused by the deletions of the TSC2‐PKD1 gene. The disease is rarely reported and the characterized manifestation is severe polycystic kidney growth. The diagnosis can be made by molecular analysis. We report the first case of PKDTS discovered in infancy in China with typical neurological and renal manifestations. The patient has infantile spasm, polycystic kidney, skin damage, hypertension, and hematuria after infection. After effective treatment of Rapamycin, the seizures were completely controlled. There was not been any renal function damage in the patient. At the same time, we review the related literature and further elaborate on the variety of clinical manifestations, treatment, and prognosis. [ABSTRACT FROM AUTHOR]