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Objective: To compare the efficacy and safety of percutaneous polidocanol sclerotherapy and laparoscopic deroofing in the management of pediatric symptomatic simple renal cysts. Methods: Forty-six ...

Objective: To compare the percutaneous and laparoscopic treatment for renal cyst in order to determine the optimal therapy for patients with renal cyst. Methods: A systematic search of PubMed, Coch...

BACKGROUND Renal cysts and diabetes (RCAD) syndrome is an autosomal dominant diabetic renal disease. Precise molecular diagnosis of RCAD syndrome has proven valuable for understanding its mechanism and personalized therapy. CASE SUMMARY A RCAD patient and her family were studied to investigate potential responsible genes by the whole exome sequencing (WES). Candidate pathogenic variants were validated by Sanger sequencing. The clinical characteristics of RCAD patient were collected from medical records. Unlike those typical RCAD patients, we observed renal manifestation and prediabetes phenotype, but not reproductive organ phenotype and hypomagnesaemia. A novel 7-bp deletion mutation in exon 4 of the hepatocyte nuclear factor 1B, NM_000458: c.882_888del (p.V294fs), was identified by WES and confirmed by Sanger sequencing. CONCLUSION This novel mutation identified in a Chinese family with RCAD syndrome might be the molecular pathogenic basis of this disorder.

Purpose: To compare the capabilities and evaluate the effectiveness of gray-scale B-mode, Doppler mapping and contrast enhanced in the assessment of cystic renal lesions.Material and methods: Ultrasound examination (US) was performed in 61 patients with cystic kidney formations (category Bosniak ≥ II). Cysts of categories Bosniak ≥ III were histologically verified, rest (categories II–IIF) were under follow up. All patients underwent gray-scale ultrasound, color Doppler imaging and contrast enhanced (CEUS).Results: The efficiency of the B mode was: sensitivity 55.6 %; specificity 72.1 %; accuracy 62.3 %, in the CDI mode these indicators were 52.8; 80.1; 63.9 %, respectively. Contrast ultrasound significantly increased the capabilities of the method, and also made it possible to evaluate cystic formations according to the Bosniak criteria with indicators of the effectiveness of the method up to 100.0; 92.0; 96.7 %, respectively.Conclusions: CEUS demonstrated high informative value in the assessment of renal cystic formations in comparison with native ultrasound and Doppler modes, and therefore the technique should be considered as promising for inclusion in the algorithm of examination of complex renal cysts.

Prior studies have suggested a higher prevalence of simple renal cysts (SRC) among patients with aortic disease, including abdominal aortic aneurysms (AAA). Thus, the aim of this study was to systematically review all currently available literature and investigate whether patients with AAA are more likely to have SRC.This study was performed according to the PRISMA guidelines. A meta-analysis was conducted with the use of random effects modeling and the I-square was used to assess heterogeneity. Odds ratios (OR) and the corresponding 95% confidence intervals (CI) were synthesized to compare the prevalence of several patients' characteristics between AAA vs. no-AAA cases.Eleven retrospective studies, 9 comparative (AAA vs. no-AAA groups) and 3 single-arm (AAA group), were included in this meta-analysis, enrolling patients (AAA: N = 2,297 vs. no-AAA: N = 35,873) who underwent computed tomography angiography as part of screening or preoperative evaluation for reasons other than AAA. The cumulative incidence of SRC among patients with AAA and no-AAA was 55% (95% CI: 49%-61%) and 32% (95% CI: 22%-42%) respectively, with a statistically higher odds of SRC among patients with AAA (OR: 3.02; 95% CI: 2.01-4.56; P0.001). The difference in SRC prevalence remained statistically significant in a sensitivity analysis, after excluding the study with the largest sample size (OR: 2.71; 95% CI: 1.91-3.84; P0.001).Our meta-analysis demonstrated a 3-fold increased prevalence of SRC in patients with AAA compared to no-AAA cases, indicating that the pathogenic processes underlying SRC and AAA could share a common pathophysiologic mechanism. Thus, patients with SRC could be considered at high risk for AAA formation, potentially warranting an earlier AAA screening.

Objective: To investigate the outcomes of retrograde flexible ureteroscopy in managing parapelvic renal cysts and speculate the factors affecting therapeutic efficacy. Methods: Thirty-eight patient...

Blood cysts of the heart are unusual primary cardiac tumor-like masses, which are usually located on the cardiac valves. These are very rare in adults, especially in a non-valvular location. Autosomal-dominant polycystic kidney disease (ADPKD) typically presents with multiple bilateral renal cysts, resulting in chronic kidney disease. Whilst many of the extra-renal manifestations of ADPKD are well-documented, associated cardiac masses are extremely rare: and cardiac blood cyst has not been reported in a patient with APKD to date. We present a 57-year man with a history of ADPKD and end-stage renal disease with a 2-cm-diameter right ventricular blood cyst, which was detected on multimodality imaging. Key words: Autosomal dominant polycystic kidney disease, Cardiac blood cyst, Extra-renal manifestations.

High levels of hormones secreted by the adrenal cortex lead to hypercortisolism, among whose complications is Cushing's syndrome. Thyroid carcinoma is one of the malignant neoplasms of the thyroid gland, the most common type of which is Papillary thyroid carcinoma (PTC), which is more common in women in the age group of 30-50 years. The course of the patient's symptoms ranges from asymptomatic to foreign body sensation, dysphagia, neck swelling, and voice change. The prognosis of patients with surgical procedures is good, and the five-year survival of patients is 90%. The concurrentincidence of several diseases in one person is a rare phenomenon. The reported article is the first very rare case of Cushing's syndrome, papillary thyroid carcinoma, and renal cysts.

Simple renal cysts are common benign lesions of the kidney with up to 4% of patients developing symptoms necessitating intervention including pain and haematuria. We conducted a systematic review of the literature to determine the efficacy and safety of aspiration–sclerotherapy of symptomatic simple renal cysts. A systematic review using Cochrane guidelines was conducted on published literature from 1990 to 2020. RCTs, cohort studies and case series meeting the inclusion criteria were reviewed and cumulative analysis of outcomes was performed. A total of 4071 patients from 57 studies underwent aspiration ± sclerotherapy for their simple renal cysts. 87.7% of patients who had aspiration with sclerotherapy demonstrated ‘treatment success’ with a >50% reduction in cyst size and complete resolution of symptoms. 453 minor, transient complications occurred (11.2%) whilst a major complication rate of less than 0.1% (4 patients) was reported. Pooled analysis of all available current literature demonstrates that aspiration–sclerotherapy is a safe and effective first-line therapy for symptomatic simple renal cysts. Although we are unable to compare those undergoing aspiration alone to aspiration–sclerotherapy, it is evident the use of a sclerosing agent is integral to treatment success; however, the optimum agent, volume, injection frequency, and dwelling time are yet to be defined. Cyst size should be considered when discussing treatment options; however, we conclude it reasonable for aspiration–sclerotherapy to be used in the first instance in all cases of symptomatic simple renal cysts. Furthermore, we propose definitions of treatment outcome measures in order to allow direct comparative analysis across future studies.

Introduction Laparoscopic renal cyst decortication is currently the best choice for the treatment of simple renal cysts and is widely used in clinical practice Aim To investigate the safety and clinical efficacy of two-trocar mini-laparoscopic decortication of adult renal cysts. Material and methods A total of 90 patients were enrolled in the study and randomly divided into two groups: a two-trocar mini-laparoscopic treatment group (M group) and a three-trocar standard laparoscopic treatment group (S group), with 45 patients in each group. Results The average length of hospital stay was shorter, and the demand for postoperative analgesics was less in the M group than in the S group (p < 0.05). The proportion of “very satisfied” patients in the patient physical recovery satisfaction survey was significantly higher in the M group than in the S group (p < 0.05). Of the 45 patients in the M group, 40 successfully underwent surgery. In 3 patients, the two-trocar procedure was converted to a three-trocar procedure due to difficulty in separating perirenal adhesion for visualization. Mini-laparoscopic surgery was converted to classic laparoscopic surgery in 2 patients. In the S group, 44 patients successfully underwent the renal cyst decortication procedure. One patient underwent partial renal resection due to an intraoperative diagnosis of multilocular cystic renal cell carcinoma. Postoperative urine leakage was reported in 3 patients in the M group and two in the S group. Conclusions Two-trocar mini-laparoscopic treatment of renal cysts is as safe and effective as traditional laparoscopy but is associated with less cosmetic damage, leading to a better physical appearance.

Renal lymphangiectasia is a rare entity occurring both in children and adults with no specific age preference. It may be unilateral or bilateral and has no gender predilection. The clinical symptoms are non-specific ranging from no complaints with incidental diagnosis to pain abdomen. Radiological appearance may be mistaken with peripelvic cysts, renal cysts, and perinephric abscess, or collection. Hence, here lies the importance of familiarity with imaging appearance of this rare condition. We describe the case of a 35-year-old male who presented with pain abdomen. The radiological investigations revealed bilateral perinephric collections which led to the diagnosis of bilateral renal lymphangiectasia by exclusion, which was confirmed by aspiration of chylous fluid.

PURPOSE: Contrast-enhanced-ultrasound (CEUS) has been frequently used in assessment of cystic renal lesions. OBJECTIVE: The aim of this study was to investigate the Bosniak classification in CEUS compared to CT and MRI in a multi-center setting. METHODS: Bosniak classification in CEUS examinations of cystic renal lesions were compared to imaging findings in computed-tomography (ceCT) and magnetic-resonance-imaging (ceMRI). Imaging results were correlated to histopathological reports. All examinations were performed by experts (EFSUMB level 3) using up-to-date CEUS examination-protocols. RESULTS: Overall, 173 cystic renal lesions were compared to subgroups CT (n = 87) and MRI (n = 86). Using Bosniak-classification 64/87 renal cysts (73.6%) were rated equal compared to CT with upgrade of four lesions (4.6%) and downgrade of 19 lesions (21.8%) by CT (Intra-class-correlation [ICC] coefficient of 0.824 [p

Objective The objective was to assess the interobserver agreement rate, progression rates and malignancy rates in the assessment of complex renal cysts (≥ Bosniak IIF) using a population-based database. Methods A regional database identified 452 complex renal cysts in 415 patients between 2009 and 2019. Each patient was tracked and followed up using a unique identifier and deterministic linkage methodology. The interobserver agreement rate between radiologists was calculated using a weighted kappa statistic. Progression and malignancy rates of cysts (Bosniak ≥IIF) over the 11-year period were calculated. Results The linear-weighted kappa value was 0.69 for all complex cysts. The rate of progression and regression of Bosniak IIF cysts was 4.6% (7/151) and 3.3% (5/151), respectively. All malignant IIF cysts progressed within 16 months of diagnosis. The malignancy rate of surgically resected Bosniak III and IV cysts was 79.3% (23/29) and 84.5% (39/46), respectively. Of all malignant tumours, 73.8% and 93.7% were of low ISUP grade and low stage, respectively. Conclusions This study further confirms that there is a good degree of agreement between radiologists in classifying complex renal masses using the Bosniak classification. The progression rate of Bosniak IIF cysts is low, but the malignancy rates of surgically resected Bosniak IIF, III and IV cysts are high. Benign cysts are frequently resected, and a very high proportion of histopathologically confirmed cancers in complex renal cysts are of low grade and stage. Key Points •There is a good degree of agreement between radiologists in classifying complex renal masses using the Bosniak classification. •The rate of progression of Bosniak IIF cysts is low, and malignant cysts progress early during surveillance. Although the malignancy rates of resected Bosniak IIF, III and IV cysts are high, the rate of benign cyst resection is significant.

OBJECTIVE To explore the current practice patterns for the management of renal cysts internationally among pediatric urologists and nephrologists. MATERIALS AND METHODS A survey composed of 21 questions and 4 clinical scenarios was distributed to pediatric urologists and nephrologists. Survey questions evaluated optimal imaging modality, management, and follow-up period. Interspecialty comparisons were made using chi-square analysis where appropriate. RESULTS A total of 183 respondents completed the survey (128 pediatric urologists, 37 pediatric nephrologists, and 19 other specialists). Most (57%) respondents agreed or strongly agreed with using an ultrasound based Bosniak classification to categorize renal cysts in children. The most commonly used follow-up intervals were 6-12 months for pediatric urologists and 1-2 years for pediatric nephrologists. Symptomatic mass effect (80.9%), gross hematuria (79.2%), or family history were the most common reasons for escalating surveillance. Pediatric nephrologists were more likely to increase follow-up with development of bilateral simple renal cysts (P = .008) or chronic kidney disease (P = .027) when compared to pediatric urologists. Laparoscopic marsupialization (39.4%) was the most common treatment for a simple renal cyst in a symptomatic child. Modified Bosniak III cysts had more heterogeneity in treatment based on the physician responses. CONCLUSION There is currently no consensus on the optimal protocol for the surveillance, imaging, or treatment of renal cysts in children. Most respondents agree that using an ultrasound-based Bosniak classification is reasonable. A call to action is therefore necessary for the development of registries and guidelines on the management of pediatric renal cysts and their associated malignancies.

Background: Percutaneous aspiration with sclerotherapy (PAS) and laparoscopic marsupialization (LM) are minimally invasive treatment modalities for renal cysts. We aimed to compare the efficacy and...

Solitary renal cysts frequently occur in children. Minimally invasive methods of treatment are often used in this case. Effectiveness of their usage was not compared. Purpose . To improve renal solitary cyst treatment outcomes in children when using minimally invasive procedures. Materials and methods . Comparative analysis of puncture and sclerosing method of treatment (TG1 = 96 children) and laparoscopic excision of cysts with argon coagulation of the cystic mucous along the renal parenchyma (TG2 = 44 patients) was performed. US, MSCT, excretory urography were used to diagnose a disease and control treatment quality. Three 5 mm trocars were applied during surgery in TG2. In laparoscopy, port injection sites were selected depending on the renal cyst localization. Results . Puncture sclerosing treatment is advantageous by treatment duration (TG1=14.3 ±1.4 min, TG2= 26.6±2.3 min) and operational trauma of the abdominal wall (TG1=1–2 mm, TG2= 15 mm). Criteria of postoperative complications support laparoscopic treatment. It was found out in the long term that 7.29% of children from TG1 had a cicatricial deformity of the renal parenchyma and adjacent pararenal fat body. Conclusion : puncture sclerosing treatment of renal solitary cysts is more traumatic by the integrity of postoperative complications and remote treatment outcomes. It doesn’t provide for complete recovery following a single procedure.

Introduction: This study aimed to investigate the safety and efficacy of mini-laparoscopy for renal cyst unroofing.Material and methods: Eighty-six patients for treatment of renal cysts that met the selection criteria were included in this study. They were divided into two groups. Forty-five patients underwent cyst unroofing via mini-laparoscopy (Group M), and 43 patients underwent cyst unroofing via standard laparoscopy (Group S). There were no differences between the two groups in terms of sex, age, body mass index or clinical data. Data from the groups were recorded and analyzed.Results: The average hospital stays were shorter (p = .039) and postoperative painkiller demand was lower (p = .031) in Group M than in Group S. Forty-one out of 45 procedures in Group M were successful, and all 43 cases in Group S were successfully. With a follow-up period of 0.5 to 5.5 years, there was no significant difference in recovery rate (p = .213). Questionnaires showed that patients in Group M were significantly more satisfied with their cosmetic results than were patients in Group S (p = .041).Conclusion: Our findings suggest that renal cyst decortications with mini-laparoscopic instruments are as safe and effective as procedures using standard laparoscopic instruments. Cosmetically, the results are better with mini-laparoscopy than with standard laparoscopic unroofing.

Purpose: To present the outcomes of the laparoscopic and robotic treatment of pediatric simple renal cysts with two novel modifications: the indocyanine green (ICG) fluorescence and the perirenal f...

Crizotinib is one of the first generations of tyrosine kinase inhibitors targeting anaplastic lymphoma kinase(ALK) and is recently found to be associated with the development of complex renal cysts with inconclusive explanation up to this time. Hereby, we discuss the hypothesis of Crizotinib-associated complex renal cyst development and coexisting renal impairment after initiation of the treatment in a 75-year-old man with ALK-positive non-small cell lung cancer whose complex renal cysts evolved after initiation and cessation of Crizotinib treatment. The coexistence as renal impairment persisted even after switching from Crizotinib to Ceritinib.

Background 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.

Evaluate the fluid percentage (FP) and enhancing solid volume (SV) of small (

Renal cysts in pediatric patients are uncommon lesion. A modified Bosniak classification system for renal cysts based on US has been developed to evaluate pediatric renal cysts to identify the simple cyst or cystic tumour. Nevertheless, it is not widely used. In this retrospective study, all incidentally detected renal cysts by ultrasound performed in children and the reproducibility of modified Bosniak classification to guide the radiological and clinical follow up.

Introduction: Kidney cancer represents 2% to 3% of malignant tumors, with an incidence of 5.8/100,000 in Western countries, a very specific subgroup of renal tumors that are cystic tumors. In fact, almost 50% of the population over 50 has a renal cyst. Materials and Methods: The objective of this study is to establish a correlation between the preoperative Bosniak score and the postoperative anatomopathological analysis in order to answer this question, the use of contrast ultrasound or MRI should be systematically performed before an atypical renal cyst? This is a retrospective study of 50 patients treated for renal cysts between January 2010 and March 2016. All patients have an abdominopelvic ultrasound and an abdominal scanner. Cysts have been classified according to the Bosniak classification. We treated our patients based on the radiological examination urocsanner and we classified our patients according to the classification of Bosniak, and uro MRI was performed in cases of atypical cysts or diagnostic doubt before considering a therapeutic attituderding to the classification of Bosniak. Results: In our work, the Uro MRI was sharper for atypical cysts (type IIF and Type III cyst) with sensitivity greater than that of the scanner but with a specificity similar to that of the scanner. And therefore be a key examination in the atypical kidney cysts. Conclusions: URO MRI is currently in 2020 a key examination of atypical bonsiak IIf and III cysts before management of the therapeutic load.

Nierentumoren werden unter anderem durch die technischen Fortschritte der Ultraschalldiagnostik und durch die flachendeckende Verbreitung von Ultraschallgeraten in Deutschland immer fruher erkannt. Die Ultraschalldiagnostik stellt meist das erste bildgebende Verfahren in der Hand des Urologen dar. Bei Nierenlasionen > 1 cm sollte mittels CT/MRT-Diagnostik (Computertomographie/Magnetresonanztomographie) der Fettgehalt gemessen werden, um die Verdachtsdiagnose eines Angiomyolipoms zu bestatigen bzw. das weitere Procedere bei malignomsuspekter Nierenlasion im interdisziplinaren Tumorboard unter Einbeziehung samtlicher Bildgebung festzulegen. Als weiterer Schritt kann besonders bei entzundlichen Lasionen oder Lymphomverdacht eine Biopsie indiziert werden, anderweitige solide Tumoren werden einer tumorstadiengerechten urologischen Therapie zugefuhrt. Mit der kontrastverstarkten Sonographie kann gegenwartig nicht sicher zwischen benignen und malignen Nierentumoren unterschieden werden. Dennoch ist die Methode im klinischen Alltag hilfreich zum Ausschluss von „Parenchymzapfen“ und anderen Pseudolasionen, als nierenschonende, kontrastverstarkte Bildgebung bei entzundlichen Lasionen und fur Verlaufskontrollen traumatischer Pseudolasionen sowie zur Differenzialdiagnose von untypischen Nierenzysten in Abgrenzung zum Nierentumor. Die kontrastverstarkte Sonographie kann ferner die Mikroperfusion von Nierentumoren aufzeigen und so Hinweise fur die Unterscheidung zwischen klarzelligen und papillaren Nierenzellkarzinomen liefern. Auch fur kontrastmittelsonographisch gesteuerte Nierentumorpunktionen ist die Methode optimal geeignet. Die kontrastverstarkte Sonographie erganzt die CT/MRT-Bildgebung durch eine Echtzeitinformation uber die Durchblutung des Nierentumors und kann so in der interdisziplinaren Nierentumorkonferenz mit der Perfusionsinformation eine mitunter therapieentscheidende Hilfe sein, deshalb sollten die Ultraschalluntersuchungen dort als Video prasentiert und kommentiert werden.

Objective: The aim of this research was to verify the efficacy and complication of flexible ureteroscopic holmium laser incision for simple renal cysts (SRCs). Patients and Methods: We ret...

Background: To explore and analyse the curative effects of a holmium laser under a flexible ureteroscope in the treatment of endogenous renal cysts complicated with renal calculi. Methods: Seven patients with endogenous renal cysts and renal calculi were admitted to our hospital from April 2019 to April 2021. The renal calculi was smashed by a holmium laser under a flexible ureteroscope, and the kidney cysts were opened by holmium laser endotomy. Window decompression was performed, indwelling the ureteral double-J tube for 1 month after the operation. After 1 month, when the patient was hospitalized and the double-J tube was removed, a flexible ureteroscope was inserted into the renal pelvis to confirm the effect of window opening and lithotripsy, and CT or B-ultrasound was reviewed. Results: Among the 7 patients with endogenous renal cysts and kidney stones, one patient was scheduled to undergo an internal incision after successful lithotripsy. The renal cyst wall tissue was surrounded by the renal artery, and the internal incision was terminated. One patient had an unsuccessful lithotripsy due to the poor angle of the kidney stone but was successfully treated for the renal cyst. The remaining 5 patients successfully underwent lithotripsy and internal incision surgery, of which 2 cases had difficulty finding kidney cysts and stones during the operation and needed assistance in locating kidney cysts and kidney stones under B-ultrasound monitoring. The average operation time was 42 minutes. When the catheter was extubated, the diameter of the renal cyst window was 0.5 cm to 2.5 cm under a flexible ureteroscope. The stone discharge was fair. There were residual gravels in the lower calyx of the kidney in two patients, and the stone basket was removed. After extubation, the maximum diameter of the renal cyst was reduced by more than half by CT or B-ultrasound, the effect of fenestration and decompression was achieved, and there were no obvious residual stones. Conclusions: A holmium laser under a flexible ureteroscope has a reliable curative effect in the simultaneous treatment of endogenous renal cysts with kidney stones, with little damage, quick recovery, and low cost. It can be used as the first choice for the treatment of endogenous renal cysts with kidney stones.

Objective This study was designed to evaluate the feasibility, efficacy, and safety of percutaneous ureteroscopy laser unroofing as an ambulatory surgery for symptomatic simple renal cysts under multilevel paravertebral nerve block anesthesia. Methods From December 2015 to September 2017, 33 simple renal cyst patients who had surgical indications were enrolled. Under ultrasound guidance, the T10/T11, T11/T12, and T12/L1 paravertebral spaces were identified, and 7–10 mL of 0.5% ropivacaine was injected at each segment. Then a puncture needle was placed inside the cyst cavity under ultrasonic monitoring. A guidewire was introduced followed by sequential dilation up to 28/30 Fr. The extra parenchymal portion of the cyst wall was dissociated and incised using a thulium laser, and a pathological examination was performed. Results Sensory loss to pinprick from T8 to L1 and sensory loss to ice from T6 to L2 were observed in all patients. None of the patients complained of pain during surgery. No serious complications occurred perioperatively. After the surgery, all patients immediately recovered their lower limb muscle strength, got out of bed, resumed oral feeding, and left the hospital within 24 h of admission. The pathologic diagnosis of all cyst walls was a simple renal cyst. The average follow-up was 35.8 months. At the end of follow-up, the cyst units were reduced in size by more than 50% compared to their preoperative size, and no patient experienced a recurrence. Conclusion Multilevel paravertebral nerve block for percutaneous ureteroscopy laser unroofing as an ambulatory surgery mode is feasible, safe, and effective for the treatment of simple renal cysts in selected patients.

Birt-Hogg-Dubé (BHD) syndrome is a rare autosomal-dominant disorder, affecting multiple organs, mostly the skin, lungs, and kidneys. The prevalence of BHD syndrome is difficult to define given the rarity of the disease. Patients present most often with primary spontaneous pneumothorax. Renal tumors are a characteristic finding in BHD, and are often bilateral and multifocal and of the chromophobe and oncocytoma variant. Very scarce reports have highlighted the presence of simple renal cysts, as the only phenotypical renal manifestation, in BHD patients. Herein, we highlight two novel cases of bilateral multiple renal and pelvic cysts, in two females with genetically proven BHD syndrome, doubting a potential association with BHD syndrome.

Background The main treatment of parapelvic cysts is flexible ureteroscope currently. Considering the intraoperative localization of the cyst may fail with flexible ureteroscope, we tend to use an innovative method by ultrasound-guided for easily locating cystic wall during flexible ureteroscopic surgery Methods We retrospectively reviewed 17 consecutive cases of parapelvic renal cysts treated by ultrasound-guided flexible ureteroscope between March 2017 and May 2020. The differences of simple flexible ureteroscopic technique and ultrasound-guided flexible ureteroscopic technique were compared. The surgical procedures, postoperative complications, results and patients’ follow-ups were evaluated. Results The cysts wall were seen clearly in 10 patients with ureteroscopic vision. Another 7 patients changed to ultrasound-guided flexible ureteroscopic surgery since it was difficult to identify the cyst wall. Mean operative time were 25.9 ± 8.7 minutes and 37.1 ± 10.1 minutes for conventional and modified technique respectively (P =0.004), of which 17.6 ± 5.8 minutes and 26.5 ± 8.4 minutes to search the cysts, respectively (P = 0.002), and the mean time of the incising were 7.1 ± 4.9 minutes and 12.1 ± 5.6 minutes, respectively (P = 0.000). All of the patients were followed-up 12 months, there were no serious complications and recurrence observed. Conclusions We demonstrated that it is feasible and safe to treat parapelvic renal cyst by ultrasound-guided flexible ureteroscopic incision and drainage. The less sample size and further studies were the limitations of our study.

International audience; Autosomal dominant polycystic kidney disease (ADPKD), characterized by progressive cyst formation/expansion, results in enlarged kidneys and often end stage kidney disease. ADPKD is genetically heterogeneous; PKD1 and PKD2 are the common loci (∼78% and ∼15% of families) and GANAB, DNAJB11, and ALG9 are minor genes. PKD is a ciliary-associated disease, a ciliopathy, and many syndromic ciliopathies have a PKD phenotype. In a multi-cohort/-site collaboration, we screened ADPKD-diagnosed families that were naive to genetic testing (n = 834) or for whom no PKD1 and PKD2 pathogenic variants had been identified (n = 381) with a PKD targeted next-generation sequencing panel (tNGS; n = 1,186) or whole-exome sequencing (WES; n = 29). We identified monoallelic IFT140 loss-of-function (LoF) variants in 12 multiplex families and 26 singletons (1.9% of naive families). IFT140 is a core component of the intraflagellar transport-complex A, responsible for retrograde ciliary trafficking and ciliary entry of membrane proteins; bi-allelic IFT140 variants cause the syndromic ciliopathy, short-rib thoracic dysplasia (SRTD9). The distinctive monoallelic phenotype is mild PKD with large cysts, limited kidney insufficiency, and few liver cysts. Analyses of the cystic kidney disease probands of Genomics England 100K showed that 2.1% had IFT140 LoF variants. Analysis of the UK Biobank cystic kidney disease group showed probands with IFT140 LoF variants as the third most common group, after PKD1 and PKD2. The proximity of IFT140 to PKD1 (∼0.5 Mb) in 16p13.3 can cause diagnostic confusion, and PKD1 variants could modify the IFT140 phenotype. Importantly, our studies link a ciliary structural protein to the ADPKD spectrum.

INTRODUCTION AND OBJECTIVE:Hereditary Leiomyomatosis and Renal Cell Carcinoma (HLRCC) is a hereditary condition in which affected individuals are at risk for the development of renal cysts, renal t...

Introduction and objective: Over the last decade laparoscopic surgery has been popularized and developed to such an extent that it can be considered gold standard for many types of procedures in urology. Currently the majority of operations in urologic field can be performed by laparoscopy. This is because it is as effective as open surgery, but associated with less postoperative pain, shorter hospital stay, faster recovery and has better cosmetic result. The aim of the study is to evaluate the results of our experience of treating symptomatic renal cysts by laparoscopy. Materials and methods: This prospective study was conducted from January 2015 to December 2017 in the Department of Urology in a single unit of Dhaka Medical College Hospital. All the patients were admitted through out-patient department. Among them those who fulfilled the criteria were selected for laparoscopic surgery. All patients were diagnosed by ultrasonography and computed tomography to determine the Bosniak classification of the cyst & informed written consent was taken. Total five laparoscopic decortication of renal cysts were performed and the results of our experience were compared with data from published article. Pain and cyst recurrence were assessed during the follow-up. Results: Our study described the results of 05 laparoscopic decortication of renal cysts. All procedures were completed successfully by transperitoneal approach, with no major intraoperative and postoperative complications. There was a placement of drain tube in one patient. The mean (range) operative duration was 68 (40–110) min, affected by the site and number of cysts decorticated. The mean post operative hospital stay was 1.8 (1- 3) days. All patients were symptom-free and no sign of recurrence during the follow-up. Conclusion: Laparoscopic decortication of symptomatic renal cysts should be the standard of care and it is feasible with conventional laparoscopic instruments and gives a better cosmetic outcome. Bangladesh Journal of Urology, Vol. 21, No. 2, July 2018 p.137-139

Kidney cancer is one of the most common cancer with a 2.02% risk in men and 1.02% in women. While several attempts are being made in improving early diagnosis, artificial intelligence has been successful in prediction of cancer progression. This paper utilizes artificial intelligence to classify genome-wide CpG regions for the identification of novel genes in the pathogenesis of benign and malignant cancer. Briefly, random forest is utilized for narrowing down the number of genes and later classify them with the aim of generating a diagnostic and prognostic biomarker signature. Characterizing molecular pathophysiology of identified genes with linking the methylation changes and its causative effects in renal cysts and masses development and progression is important for validation of proposed technique.

The growing availability of modern-day imaging has resulted in an increase in the number of renal cysts detected in the pediatric population. Few publications have reported outcomes of these childhood cysts. In this study we assessed the prevalence and evolution of renal cysts in children, and described clinical characteristics, mode of presentation and ultimate outcomes.Our institutional ultrasound database was searched for all abdominal ultrasound reports from 2006 to 2017. These reports were then cross-referenced with a manual retrospective chart review. Clinical characteristics including mode of presentation, cyst characteristics, and outcomes were analyzed using descriptive and nonparametric statistical methods.Of 70,500 abdominal ultrasound scans during the study period 1,531 (2.2%) met the study inclusion criteria. Overall 26% of cysts were complex and 10.1% of cases were associated with hydronephrosis. Echogenic kidneys were more likely to be associated with simple cysts (p=0.0001). There was no difference between cyst diameter and symptomatology (p=0.82). The conversion of simple to complex renal cysts was less than 1% and 1.8% of complex cysts developed renal cell carcinoma.In a large cohort of children who underwent abdominal imaging we found a 10-year renal cyst prevalence of 2.2%. Given that nearly all cysts follow a benign course and that simple cysts will invariably grow within 2 years, we believe that these cases could be safely discharged after that point. We continue to recommend surveillance for patients with cysts larger than 15 mm, complex cysts, family history of adult polycystic kidney disease or those with concomitant genitourinary anomalies requiring ongoing followup.

Polycystic kidney disease (PKD) is characterized by slowly expanding renal cysts that damage the kidney, typically resulting in renal failure by the fifth decade. The most common cause of death in these patients, however, is cardiovascular disease. Expanding cysts in PKD induce chronic kidney injury that is accompanied by immune cell infiltration, including macrophages, which we and others have shown can promote disease progression in PKD mouse models. Here, we show that monocyte chemoattractant protein-1 [MCP-1/chemokine (C-C motif) ligand 2 (CCL2)] is responsible for the majority of monocyte chemoattractant activity produced by renal PKD cells from both mice and humans. To test whether the absence of MCP-1 lowers renal macrophage concentration and slows disease progression, we generated genetic knockout (KO) of MCP-1 in a mouse model of PKD [congenital polycystic kidney ( cpk) mice]. Cpk mice are born with rapidly expanding renal cysts, accompanied by a decline in kidney function and death by postnatal day 21. Here, we report that KO of MCP-1 in these mice increased survival, with some mice living past 3 mo. Surprisingly, however, there was no significant difference in renal macrophage concentration, nor was there improvement in cystic disease or kidney function. Examination of mice revealed cardiac hypertrophy in cpk mice, and measurement of cardiac electrical activity via ECG revealed repolarization abnormalities. MCP-1 KO did not affect the number of cardiac macrophages, nor did it alleviate the cardiac aberrancies. However, MCP-1 KO did prevent the development of pulmonary edema, which occurred in cpk mice, and promoted decreased resting heart rate and increased heart rate variability in both cpk and noncystic mice. These data suggest that in this mouse model of PKD, MCP-1 altered cardiac/pulmonary function and promoted death outside of its role as a macrophage chemoattractant.

Objective: To evaluate progression rate of Bosniak category IIF complex renal cysts and the malignancy rate among surgically resected cysts. Materials and Methods: We performed a database search for complex renal cysts classified as Bosniak category IIF on computed tomography or magnetic resonance imaging between January 2008 and April 2016. Follow-up examinations (computed tomography or magnetic resonance imaging) were used in order to evaluate progression (Bosniak category reclassification) and stability, the latter being defined as remaining stable for a minimum of six months. Pathology reports were used as the reference to assess the malignancy rate of surgically resected cysts. Results: A total of 152 cysts in 143 patients were included in the final analysis. Seven cysts (4.6%) were reclassified on follow-up studies, and mean time to progression was 20 months (range, 1 month to 4 years). Three cysts were surgically resected. All three were diagnosed as low-grade malignant renal cell carcinomas (RCCs): one clear cell RCC and two papillary RCCs. The remaining 145 cysts remained unchanged after a mean follow-up period of 28 months (range, 6 to 118 months). Conclusion: The progression rate in Bosniak category IIF cysts was low. Even lesions that were upgraded on follow-up remained stable, indicating an indolent behavior. Our data support the idea of conservative management of Bosniak IIF renal cyst. Resumo Objetivo: Avaliar a taxa de progressão das lesões císticas renais complexas Bosniak II-F e a taxa de neoplasia maligna nas lesões ressecadas cirurgicamente. Materiais e Métodos: Realizamos uma pesquisa no banco de dados da nossa instituição reunindo lesões císticas renais complexas classificadas como Bosniak II-F em exames de tomografia computadorizada ou ressonância magnética, de janeiro de 2008 a abril de 2016. Foram utilizados exames de acompanhamento (tomografia computadorizada ou ressonância magnética) para avaliar a progressão na categoria Bosniak, com um mínimo de seis meses de estabilidade. Os relatórios de patologia foram utilizados como referência para avaliar a taxa de neoplasia maligna de lesões ressecadas cirurgicamente. Resultados: Um total de 152 lesões em 143 pacientes foi incluído na análise final. Sete lesões foram reclassificadas em estudos de acompanhamento (4,6%) e o tempo médio de progressão foi de 20 meses (1 mês a 4 anos). Três pacientes tiveram cistos ressecados cirurgicamente, todos diagnosticados como carcinoma de células renais: um do subtipo células claras e dois papilíferos, todos de baixo grau. As 145 lesões restantes permaneceram inalteradas, com seguimento médio de 28 meses (6 a 118 meses). Conclusão: A taxa de progressão nos cistos categoria Bosniak II-F foi baixa. Mesmo as lesões que tiveram upgrade da categoria de Bosniak no seguimento permaneceram estáveis, indicando um comportamento indolente. Nossos dados apoiam a ideia de tratamento conservador do cisto renal Bosniak II-F.

The objective of our study was to evaluate the cost-effectiveness of active surveillance (AS) versus nephron-sparing surgery (NSS) in patients with a Bosniak IIF or III renal cyst.Markov models were developed to estimate life expectancy and lifetime costs for 60-year-old patients with a Bosniak IIF or III renal cyst (the reference cases) managed by AS versus NSS. The models incorporated the malignancy rates, reclassification rates during follow-up, treatment effectiveness, complications and costs, and short- and long-term outcomes. An incremental cost-effectiveness analysis was performed to identify management preference under an assumed $75,000 per quality-adjusted life-year (QALY) societal willingness-to-pay threshold, using data from studies in the literature and the 2015 Medicare Physician Fee Schedule. The effects of key parameters were addressed in a multiway sensitivity analysis.The prevalence of malignancy for Bosniak IIF and III renal cysts was 26% (25/96) and 52% (542/1046). Under base case assumptions for Bosniak IIF cysts, the incremental cost-effectiveness ratio of NSS relative to AS was $731,309 per QALY for women, exceeding the assumed societal willingness-to-pay threshold, and AS outperformed NSS for both life expectancy and cost for men. For Bosniak III cysts, AS yielded greater life expectancy (24.8 and 19.4 more days) and lower lifetime costs (cost difference of $12,128 and $11,901) than NSS for men and women, indicating dominance of AS over NSS. Superiority of AS held true in sensitivity analyses for men 46 years old or older and women 57 years old or older even when all parameters were set to favor NSS.AS is more cost-effective than NSS for patients with a Bosniak IIF or III renal cyst.

Objectives: To determine the efficacy and safety of laparoscopic deroofing of symptomatic simple renal cysts. Methods: A Cochrane style systematic review was conducted on published literature from 1990 to 2017, to include case series and randomised controlled trials. A pooled meta-analysis was conducted. Results: A total of 696 patients who had laparoscopic deroofing of their simple renal cysts were identified. Complete radiological resolution was seen in 96% of patients with 95% of patients completely symptoms free. Less than 1% of patients had intra-operative complications and 1.4% developed postoperative complications. Conclusions: Cumulative analysis of the literature examining the role of laparoscopic decortication in the management of symptomatic simple renal cysts reveals that the procedure is highly efficacious to relieve symptoms. The procedure was also associated with a significant radiological success rate (96.3%). It was also shown to be safe, with a low complication rate. We deduced a protocol following this systematic review for the management of symptomatic renal cyst in an aim to help select patients that would benefit from laparoscopic decortication.

Objective The aim of this study was to determine which apparent diffusion coefficient-derived texture features are associated with malignancy in Bosniak IIF and III renal cystic lesions. Methods Twenty benign and 7 malignant Bosniak IIF (22) or III (5) renal cysts, as evaluated with magnetic resonance imaging, were assessed for progression to pathology-confirmed malignancy. Whole-cyst volumes of interest were manually segmented from apparent diffusion coefficient maps. Texture features were extracted from each volume of interest, including first-order histogram-based features and higher-order features, and data were analyzed with the Mann-Whitney U test to predict malignant progression. Results Eleven of 17 first-order features were significantly greater in benign compared with malignant cysts. Eight higher-order gray-level co-occurrence matrix (GLCM) texture features were significantly different between groups, 5 of which were greater in the benign population. Conclusions Apparent diffusion coefficient-derived texture measures may help differentiate between benign and malignant Bosniak IIF and III cysts.

To determine the effects of different reconstruction algorithms on histogram and texture features in different targets.Among 3620 patients, 480 had normal liver parenchyma, 494 had focal solid liver lesions (metastases = 259; hepatocellular carcinoma = 99; hemangioma = 78; abscess = 32; and cholangiocarcinoma = 26), and 488 had renal cysts. CT images were reconstructed with filtered back-projection (FBP), hybrid iterative reconstruction (HIR), and iterative model reconstruction (IMR) algorithms. Computerized histogram and texture analyses were performed by extracting 11 features.Different reconstruction algorithms had distinct, significant effects. IMR had a greater effect than HIR. For instance, IMR had a significant effect on five features of liver parenchyma, nine features of focal liver lesions, and four features of renal cysts on portal-phase scans and four, eight, and four features, respectively, on precontrast scans (p 0.05). Meanwhile, different algorithms had a greater effect on focal liver lesions (six in HIR and nine in IMR on portal-phase, three in HIR, and eight in IMR on precontrast scans) than on liver parenchyma or cysts. The mean attenuation and standard deviation were not affected by the reconstruction algorithm (p .05). Most parameters showed good or excellent intra- and interobserver agreement, with intraclass correlation coefficients ranging from 0.634 to 0.972.Different reconstruction algorithms affect histogram and texture features. Reconstruction algorithms showed stronger effects in focal liver lesions than in liver parenchyma or renal cysts.• Imaging heterogeneities influenced the quantification of image features. • Different reconstruction algorithms had a significant effect on histogram and texture features. • Solid liver lesions were more affected than liver parenchyma or cysts.

Cystic kidney diseases such as autosomal recessive or dominant polycystic kidney disease (ARPKD and ADPKD) are associated with high prevalence of arterial hypertension. On the contrary, studies on hypertension in children with renal cysts and diabetes (RCAD) syndrome caused by abnormalities in the HNF1B gene are rare. Therefore, the aim of our study was to investigate the prevalence of hypertension in children with RCAD syndrome due to HNF1B gene abnormalities and to search for possible risk factors for development of hypertension. Data on all children with genetically proven RCAD syndrome from three pediatric nephrology tertiary centers were retrospectively reviewed (office blood pressure (BP), ambulatory blood pressure monitoring (ABPM), creatinine clearance, renal ultrasound, echocardiography, albuminuria/proteinuria). Hypertension was defined using the current ESH 2016 criteria and/or by the use of antihypertensive drugs. Thirty-two children with RCAD syndrome were investigated. Three children received ACE-inhibitors for hypertension and/or proteinuria. Hypertension was diagnosed using office BP in 22% of the children (n = 7) In the 7 performed ABPM 1 child (14%) was diagnosed with hypertension. Creatinine clearance, proteinuria, albuminuria, body mass index, enlargement or hypodysplasia of the kidneys and prevalence of HNF1B-gene deletion or mutation were not significantly different between hypertensive and normotensive children. Conclusion: Hypertension is rare in children with RCAD syndrome. The low prevalence of hypertension seems to be the hallmark of the disease.