Ureteroscopy vs laparoscopic ureterolithotomy for large proximal ureteric stone: a randomised trial.

OBJECTIVE: To compare the outcomes of retrograde flexible ureteroscopy (fURS) with retroperitoneal laparoscopic ureterolithotomy (RLU) for large proximal ureteric stones. PATIENTS AND METHODS: A prospective randomised trial was conducted from January 2018 through December 2022 including patients with impacted proximal ureteric stones of 15-25 mm. Patients underwent fURS or RLU. Primary outcome was the stone-free rate. Demographic data, stone features, and complications rates were also compared between groups. RESULTS: A total of 64 patients were enrolled, 32 in each group. The mean impacted stone time was similar between groups, as well as stone size (17 mm) and stone density (>1000 Hounsfield Units). The ureteric stone-free rates between the two groups (93.7% in fURS vs 96.8% in RLU; odds ratio [OR] 0.72, 95% confidence interval [CI] -1.72 to 3.17; P = 0.554), and overall success rates, which take into account residual fragments in the kidney (84.3% in fURS vs 93.7% in RLU; OR 1.02, 95% CI -0.69 to 2.74; P = 0.23), were similar. Operative time was also not statistically significantly different between groups (median 80 vs 82 min; P = 0.101). There was no difference in hospital length of stay. Retropulsion rate was higher with fURS (65.6% vs 3.1%; p < 0.001). Residual hydronephrosis (34.3% each group) and complication rates did no differ according to treatment. CONCLUSION: Flexible URS and RLU are both highly efficient and present low morbidity for large impacted proximal ureteric stone treatment. RLU is not superior to fURS.

Clinical and Pathological Predictors of Death for Adrenocortical Carcinoma.

Adrenocortical carcinoma (ACC) is a rare and lethal disease with a poor prognosis. This study aims to share our 41-year experience as a referral center, focusing on identifying risk factors associated with ACC mortality. Our retrospective analysis included a cohort of 150 adult patients with ACC in all stage categories, treated between 1981 and 2022. Tumor hormonal hypersecretion was observed in 78.6% of the patients, and the median age of diagnosis was 40 years. The majority presented as European Network for the Study of Adrenal Tumors (ENSAT) III or IV (22.9% and 31.2%, respectively), and the overall mortality rate was 54.6%. Independent predictors of death were elevated secretion of cortisol (HR = 2.0), androstenedione (HR = 2.2), estradiol (HR = 2.8), 17-OH progesterone (HR = 2.0), and 11-deoxycortisol (HR = 5.1), higher Weiss (HR = 4.3), modified Weiss (HR = 4.4), and Helsinki scores (HR = 12.0), advanced ENSAT stage (HR = 27.1), larger tumor size (HR = 2.7), higher Ki-67 percentage (HR = 2.3), and incomplete surgical resection (HR = 2.5). Mitosis greater than 5/50 high-power field (HR = 5.6), atypical mitosis (HR = 2.3), confluent necrosis (HR = 15.4), venous invasion (HR = 2.8), and capsular invasion (HR = 2.4) were also identified as independent predictors of death. Knowing the risk factors for ACC's mortality may help determine the best treatment option.

Renal Function Evolution and Hypoaldosteronism Risk After Unilateral Adrenalectomy for Primary Aldosteronism.

Few studies demonstrated a percentage decrease in the estimated glomerular filtration rate (eGFR) at a single time and the rate of hypoaldosteronism after adrenalectomy for primary aldosteronism (PA). Our aim was to investigate the evolution of renal function and the hypoaldosteronism risk after adrenalectomy for PA. Aldosterone, renin, eGFR, and electrolyte levels were determined before and at 1 week, 1, 3 and 6 months after unilateral adrenalectomy in 94 PA patients (40 men and 54 women). The main outcome was the postoperative eGFR decline using analysis of covariance with the preoperative eGFR as a covariate. eGFR decreased during first postoperative week compared to 3 months before surgery. During the first 6 months, eGFR remained stable at similar levels to the first week after surgery. Age (p=0.001), aldosterone levels (p=0.021) and eGFR 3 months before surgery (p+<+0.0001) had a significant correlation with eGFR during first postoperative week. High aldosterone levels at diagnosis were correlated with decline in renal function in the univariate model (p=0.033). In the multivariate analysis, aldosterone levels at diagnosis had a tendency to be an independent predictor of renal function after surgery (p=0.059). Postoperative biochemical hypoaldosteronism was diagnosed in 48% of the cases after adrenalectomy, but prolonged hyperkalemia occurred in only 4 cases (4.5%). Our findings showed a decrease of eGFR after unilateral adrenalectomy for PA. Additionally, aldosterone levels at diagnosis correlated with postoperative renal function. Postoperative biochemical hypoaldosteronism occurred in almost half of the patients, but prolonged hyperkalemia with fludrocortisone replacement was less frequent.

The effect of hormonal secretion on survival in adrenocortical carcinoma: A multi-center study.

BACKGROUND: Current evidence suggests that cortisol secreting adrenocortical carcinoma has worse prognosis compared to non-secreting adrenocortical carcinoma. However, the effect of other secretory subtypes is unknown. METHODS: This multicenter study within the American-Australian-Asian Adrenal Alliance included adults with adrenocortical carcinoma (1997-2020). We compared overall survival and disease-free survival among cortisol secreting, mixed cortisol/androgen secreting, androgen secreting, and non-secreting adrenocortical carcinoma. RESULTS: Of the 807 patients (mean age 50), 719 included in the secretory subtype analysis: 24.5% were cortisol secreting, 13% androgen secreting, 28% mixed cortisol/androgen, 32.5% non-secreting, and 2% were mineralocorticoid secreting. Median overall survival and disease-free survival for the entire cohort were 60 and 9 months, respectively. Median overall survival was 36 months for cortisol, 30 for mixed, 60 for androgen secreting, and 115 for non-secreting adrenocortical carcinoma, P < .01. Median disease-free survival was 7 months for cortisol, 8 for mixed, 10 for androgen, and 12 for non-secreting adrenocortical carcinoma, P = .06. On multivariable analysis of age, sex, Ki67%, secretory subtype, stage, resection, and adjuvant therapy, predictors of worse overall survival were older age, higher Ki67%, stage IV, mixed secreting, R1, and no adjuvant therapy, P < .05. On subgroup analysis of R0 resection, predictors of worse overall survival included older age and higher Ki67%. Ki67% ≥40, stage III and cortisol secretion were associated with worse disease-free survival. CONCLUSION: Mixed cortisol/androgen secreting adrenocortical carcinoma was associated with worse overall survival, while cortisol or androgen secreting alone were not. Notably, among patients after R0 resection, secretory subtype did not affect overall survival. Cortisol secreting adrenocortical carcinoma demonstrated worse disease-free survival. Ki67% remained a strong predictor of worse overall survival and disease-free survival independent of stage.

Efficacy of Oral Furosemide Test for Primary Aldosteronism Diagnosis.

Context: Confirmatory tests represent a fundamental step in primary aldosteronism (PA) diagnosis, but they are laborious and often require a hospital environment due to the risks involved. Objective: To evaluate the efficacy of oral furosemide as a new confirmatory test for PA diagnosis. Methods: We prospectively evaluated the diagnostic performance of 80 mg of oral furosemide in 64 patients with PA and 22 with primary hypertension (controls). Direct renin concentration (DRC) was measured before, and 2 hours and 3 hours after the oral furosemide. In addition, the oral furosemide test was compared with 2 other confirmatory tests: the furosemide upright test (FUT) and saline infusion test (SIT) or captopril challenge test (CCT) in all patients with PA. Results: The cut-off of 7.6 µU/mL for DRC at 2 hours after oral furosemide had a sensitivity of 92%, specificity of 82%, and accuracy of 90% for PA diagnosis. In 5 out of 6 controls with low-renin hypertension, which might represent a PA spectrum, renin remained suppressed. Excluding these 6 controls with low-renin hypertension, the DRC cut-off of 10 µU/mL at 2 hours after oral furosemide had a sensitivity of 95.3%, specificity of 93.7% and accuracy of 95% for PA diagnosis. DRC after 3 hours of oral furosemide did not improve diagnostic performance. Using the cut-off of 10 µU/mL, the oral furosemide test and the FUT were concordant in 62 out of 64 (97%) patients with PA. Only 4 out of 64 cases with PA (6.4%) ended the oral furosemide test with potassium <3.5 mEq/L. Hypotension was not evidenced in any patient with PA during the test. Conclusion: The oral furosemide test was safe, well-tolerated and represents an effective strategy for PA investigation.

Laparoscopic Ureterocalicostomy Technique

ABSTRACT Purpose: Ureterocalicostomy is a technique that was first described by Neuwirt in 1948 (1) The laparoscopic access was initiated in 2003 by Cherullo et al. (2), following the established principles of open surgery. In 2004, Gill et al. had two patients with UPJO treated with laparoscopic ureterocalicostomy, with success (3). In 2014, Arap et. al. presented a case series with good results in adults and children in our service (4). There are factors that prepare the surgeon for an ureterocalicostomy, such as the renal cortex thickness, although the decision is mainly taken during the procedure (5). Material and Methods: A 24 years-old female patient with right lumbar pain was referred to our institution. She already had a right open pyeloplasty two years ago. The CT scan presented a right hydronephrotic kidney, DMSA scan with 30% of relative function and a DTPA scan with an obstructive pattern. Results: A laparoscopic ureterocalicostomy was performed due to the intra-operative findings (inferior kidney pole thickness and challenging access to the uretero-pelvic junction). The overall time was 130 minutes with no complications. The patient was discharged in two days and the double J was withdrawn in four weeks. The CT scan within one year demonstrates a reduction of the hydronephrosis. She had no more lumbar pain. Conclusion: In complex cases, the laparoscopic ureterocalicostomy proves to be a safe and efficient procedure, with a free tension-free anastomosis and the advantages of the laparoscopic access.

Laparoscopic Ureterocalicostomy Technique.

PURPOSE: Ureterocalicostomy is a technique that was first described by Neuwirt in 1948 (1) The laparoscopic access was initiated in 2003 by Cherullo et al. (2), following the established principles of open surgery. In 2004, Gill et al. had two patients with UPJO treated with laparoscopic ureterocalicostomy, with success (3). In 2014, Arap et. al. presented a case series with good results in adults and children in our service (4). There are factors that prepare the surgeon for an ureterocalicostomy, such as the renal cortex thickness, although the decision is mainly taken during the procedure (5). MATERIAL AND METHODS: A 24 years-old female patient with right lumbar pain was referred to our institution. She already had a right open pyeloplasty two years ago. The CT scan presented a right hydronephrotic kidney, DMSA scan with 30% of relative function and a DTPA scan with an obstructive pattern. RESULTS: A laparoscopic ureterocalicostomy was performed due to the intra-operative findings (inferior kidney pole thickness and challenging access to the uretero-pelvic junction). The overall time was 130 minutes with no complications. The patient was discharged in two days and the double J was withdrawn in four weeks. The CT scan within one year demonstrates a reduction of the hydronephrosis. She had no more lumbar pain. CONCLUSION: In complex cases, the laparoscopic ureterocalicostomy proves to be a safe and efficient procedure, with a free tension-free anastomosis and the advantages of the laparoscopic access.

Evidence for a Founder Effect of SDHB Exon 1 Deletion in Brazilian Patients With Paraganglioma.

CONTEXT: Limited information is available concerning the genetic spectrum of pheochromocytoma and paraganglioma (PPGL) patients in South America. Germline SDHB large deletions are very rare worldwide, but most of the individuals harboring the SDHB exon 1 deletion originated from the Iberian Peninsula. OBJECTIVE: Our aim was to investigate the spectrum of SDHB genetic defects in a large cohort of Brazilian patients with PPGLs. METHODS: Genetic investigation of 155 index PPGL patients was performed by Sanger DNA sequencing, multiplex ligation-dependent probe amplification, and/or target next-generation sequencing panel. Common ancestrality was investigated by microsatellite genotyping with haplotype reconstruction, and analysis of deletion breakpoint. RESULTS: Among 155 index patients, heterozygous germline SDHB pathogenic or likely pathogenic variants were identified in 22 cases (14.2%). The heterozygous SDHB exon 1 complete deletion was the most frequent genetic defect in SDHB, identified in 8 out of 22 (36%) of patients. Haplotype analysis of 5 SDHB flanking microsatellite markers demonstrated a significant difference in haplotype frequencies in a case-control permutation test (P = 0.03). More precisely, 3 closer/informative microsatellites were shared by 6 out of 8 apparently unrelated cases (75%) (SDHB-GATA29A05-D1S2826-D1S2644 | SDHB-186-130-213), which was observed in only 1 chromosome (1/42) without SDHB exon 1 deletion (X2 = 29.43; P < 0.001). Moreover, all cases with SDHB exon 1 deletion had the same gene breakpoint pattern of a 15 678 bp deletion previously described in the Iberian Peninsula, indicating a common origin. CONCLUSION: The germline heterozygous SDHB exon 1 deletion was the most frequent genetic defect in the Brazilian PPGL cohort. Our findings demonstrated a founder effect for the SDHB exon 1 deletion in Brazilian patients with paragangliomas.

The influence of interstitial cells of Cajal density in the outcomes of pyeloplasty in adults: A prospective analysis.

PURPOSE: To evaluate if the density of interstitial cells of Cajal (ICC) in the ureteropelvic junction (UPJ) influences the outcomes of pyeloplasty in adults. METHODS: Twenty-three patients with the diagnosis of ureteropelvic junction obstruction (UPJO) that underwent laparoscopic dismembered pyeloplasty were included. ICC density was measured using immunohistochemistry reaction for c-KIT expression in the resected UPJ segment. Pyeloplasty outcome was evaluated by patient self-report pain, urinary outflow using DTPA renogram and hydronephrosis assessment using ultrasound (US) at 12 months of follow-up. A logistic regression analysis was performed to assess the association of pyeloplasty outcomes and ICC density. RESULTS: Low, moderate, and high ICC density were present in 17.4%, 30.4%, and 52.2% of the patients, respectively. Complete pain resolution was observed in 100%, 85.7%, and 75% of patients with low, moderate and high ICC density, respectively (p = 0.791). DTPA renogram improved in 75%, 85.7%, and 91.7% of patients with low, moderate and high ICC density, respectively (p = 0.739). Hydronephrosis improved in 25%, 85.7%, and 91.7% of patients with low, moderate and high ICC density, respectively (p = 0.032). CONCLUSIONS: Patients with high ICC density have a significant amelioration of hydronephrosis after pyeloplasty. However, ICC density is not associated with functional outcomes.

Intra-individual Variability of Serum Aldosterone and Implications for Primary Aldosteronism Screening.

CONTEXT: Primary aldosteronism (PA) screening relies on an elevated aldosterone to renin ratio with a minimum aldosterone level, which varies from 10 to 15 ng/dL (277-415.5 pmol/L) using immunoassay. OBJECTIVE: To evaluate intra-individual coefficient of variation (CV) of aldosterone and aldosterone to direct renin concentration ratio (A/DRC) and its impact on PA screening. METHODS: A total of 671 aldosterone and DRC measurements were performed by the same chemiluminescence assays in a large cohort of 216 patients with confirmed PA and at least 2 screenings. RESULTS: The median intra-individual CV of aldosterone and A/DRC was 26.8% and 26.7%. Almost 40% of the patients had at least one aldosterone level <15 ng/dL, 19.9% had at least 2 aldosterone levels <15 ng/dL, and 16.2% had mean aldosterone levels <15 ng/dL. A lower cutoff of 10 ng/dL was associated with false negative rates for PA screening of 14.3% for a single aldosterone measurement, 4.6% for 2 aldosterone measurements, and only 2.3% for mean aldosterone levels. Considering the minimum aldosterone, true positive rate of aldosterone thresholds was 85.7% for 10 ng/dL and 61.6% for 15 ng/dL. An A/DRC >2 ng/dL/µIU/mL had a true positive rate for PA diagnosis of 94.4% and 98.4% when based on 1 or 2 assessments, respectively. CV of aldosterone and A/DRC were not affected by sex, use of interfering antihypertensive medications, PA lateralization, hypokalemia, age, and number of hormone measurements. CONCLUSION: Aldosterone concentrations had a high CV in PA patients, which results in an elevated rate of false negatives in a single screening for PA. Therefore, PA screening should be based on at least 2 screenings with concomitant aldosterone and renin measurements.

Cytoreductive Surgery of the Primary Tumor in Metastatic Adrenocortical Carcinoma: Impact on Patients' Survival.

CONTEXT: The role of cytoreduction of adrenocortical carcinoma (ACC) remains poorly understood. OBJECTIVE: To analyze the impact of cytoreductive surgery of the primary tumor in patients with metastatic ACC. DESIGN AND SETTING: We performed a multicentric, retrospective paired cohort study comparing the overall survival (OS) in patients with metastatic ACC who were treated either with cytoreductive surgery (CR group) or without cytoreductive surgery (no-CR group) of the primary tumor. Data were retrieved from 9 referral centers in the American-Australian-Asian Adrenal Alliance collaborative research group. PATIENTS: Patients aged ≥18 years with metastatic ACC at initial presentation who were treated between January 1, 1995, and May 31, 2019. INTERVENTION: Performance (or not) of cytoreductive surgery of the primary tumor. MAIN OUTCOME AND MEASURES: A propensity score match was done using age and the number of organs with metastasis (≤2 or >2). The main outcome was OS, determined from the date of diagnosis until death or until last follow-up for living patients. RESULTS: Of 339 patients pooled, 239 were paired and included: 128 in the CR group and 111 in the no-CR group. The mean follow-up was 67 months. Patients in the no-CR group had greater risk of death than did patients in the CR group (hazard ratio [HR] = 3.18; 95% CI, 2.34-4.32). Independent predictors of survival included age (HR = 1.02; 95% CI, 1.00-1.03), hormone excess (HR = 2.56; 95% CI, 1.66-3.92), and local metastasis therapy (HR = 0.41; 95% CI, 0.47-0.65). CONCLUSION: Cytoreductive surgery of the primary tumor in patients with metastatic ACC is associated with prolonged survival.

Videolaparoscopic prostatectomy in porcine model for training residents

ABSTRACT Introduction: Surgical training models prepare the resident for a more ethical surgical practice as well as providing a less steep learning curve. In urology, there are well-known models of pyeloplasty simulation, urethro-vesical anastomosis and nephrectomy, which have helped in the training of urology residents (1-3). Learning laparoscopic prostatectomy is a difficult surgery and requires advanced surgical skill from the surgeon (4), requires operate without a direct view of the surgical field in a two-dimensional space and with longer instruments (5). Laparoscopic prostatectomy step by step makes the surgeon's learning curve less difficult, lead to less intraoperative complications, such as blood loss, while also enabling shorter operative time and less positive surgical margins (6). The objective of surgical models is to simulate surgical procedures in a reliable way thus preparing the surgeon for his daily practice, surgical simulations in animal models have been described to compensate for inadequate clinical exposure (7). The canine model of prostate cancer has many similarities with humans. Despite trying to develop a model that is as credible as possible, there are ethical issues in several countries, such as Brazil, that do not allow the use of live dogs for scientific experimentation and there is a difficulty in not standardizing the animals used (8, 9). The swine surgical training model is widely known, accepted and used as a valuable tool in the teaching of new surgeons (10). The porcine video laparoscopic prostatectomy model allows the urologist in training to exercise the skills required in a real surgical situation, practicing them in a single session (10). We will present an experimental model in pigs for training urology residents in laparoscopic radical prostatectomy with current techniques (11-13). The limitations found are that the prostate has no limits as well defined as in humans, the urethra is long and coiled, the fat surrounding the pelvic organs is scarce and there is no postoperative follow-up for evaluating functionality after the procedure, as well as the effectiveness of the surgery with surgical margins. However, it is similar in surgical model presented, it is reproducible and can provide a realistic simulation environment to the beginner surgeon. Material and Methods: In this paper, according to the institutional protocol approved by the institutional ethics and research committee FMUSP n° 964/2017 and protocol was in accordance with current international regulations for the use of animals in Research: Reporting In Vivo Experiments (ARRIVE) guide. Ten male pigs weighing 20 to 22kg were used. The animals were anesthetized with a combination of Telazol (5mg/kg), Xylazine (1.5mg/kg), Cetamine (22mg/kg) and Atropine (0.04mg/kg) for orotracheal intubation followed by Isoflurane (2%). Animals were euthanized at the end of the procedure with a lethal dose of KCl (2mEq/kg). The trocar insertion points were marked using the epigastric vessels and umbilical region as reference points. Initially, urethral catheterization was performed using a hydrophilic Nitinol guidewire, followed by a perineal incision to dissect the tortuous urethra of the porcine model. A malleable urethral catheter 8Fr was inserted into their bladder. The animal was placed in the Trendelenburg position inserted and 12mm trocars were inserted in its umbilical region, utilizing 10mm in the surgeon's dominant hand, 5mm in his non-dominant hand of the surgeon, and 5mm in the first assistant's trocar. The surgeon replicates the steps performed in a laparoscopic radical prostatectomy in humans, including the bladder catheterization, dissection of the anterior bladder plane, the vesicular and prostatic dissection, the suture of the dorsal venous plexus, a prostatectomy, an urethral vesical anast omosis, as well as the waterproof test, even including the performing of surgical steps using current concepts of anterior urethral suspension as the reconstruction of the posterior plane of the rhabdosphincter. Results: All steps of surgery could be reproduced in all ten porcine cases. No significant bleeding was observed and the surgical time was gradually reduced fifty percent from case one to last cases. Conclusions: The porcine model allowed the surgeon to replicate all the steps usually performed in a laparoscopic radical prostatectomy. The junior surgeons are better prepared to such difficult surgery. However, further studies will be necessary to prove the impact of the animal model presented in urological clinical practice.

MiR-200c-3p expression may be associated with worsening of the clinical course of patients with COVID-19.

COVID-19 represents a public health emergency, whose mechanism of which is not fully understood. It is speculated that microRNAs may play a crucial role in host cells after infection by SARS-CoV-2. Thus, our study aimed to analyze the expression of miR-200c-3p in saliva samples from patients with COVID-19. One handred eleven samples from patients with COVID-19 were divided into 4 groups. Group I: 39 patients negative for Covid-19; Group II: 37 positive and symptomatic patients, with no indication of hospitalization; Group III: 21 patients with respiratory disorders (hospitalized); Group IV: 14 patients with severe conditions (oxygen therapy). The expression levels of miR-200c-3p were determined using qPCR. We found greater expression of miR-200c-3p in patients in group IV (p<0.0001), and also verified that patients aged ≥42 years had a higher expression of this miR (p=0.013). Logistic regression analysis revealed that the expression of miR-200c-3p and systemic arterial hypertension are factors independently associated with patients in group IV (p<0.0001). Our results suggest that miR-200c-3p is a predictor of severity independent of COVID-19 risk factors, which could represent a way of screening patients affected by SARS-CoV-2.

Shorter leukocyte telomere length is associated with severity of COVID-19 infection.

The infection by COVID-19 is a serious global public health problem. An efficient way to improve this disease's clinical management would be to characterize patients at higher risk of progressing to critically severe infection using prognostic biomarkers. The telomere length could be used for this purpose. Telomeres are responsible for controlling the number of maximum cell divisions. The telomere length is a biomarker of aging and several diseases. We aimed to compare leukocyte telomere length (LTL) between patients without COVID-19 and patients with different clinical severity of the infection. Were included 53 patients who underwent SARS-CoV-2 PCR divided in four groups. The first group was composed by patients with a negative diagnosis for COVID-19 (n = 12). The other three groups consisted of patients with a confirmed diagnosis of COVID-19 divided according to the severity of the disease: mild (n = 15), moderate (n = 17) and severe (n = 9). The LTL was determined by Q-PCR. The severe group had the shortest LTL, followed by the moderate group. The negative and mild groups showed no differences. There is an increase of patients with hypertension (p = 0.0099) and diabetes (p = 0.0067) in moderate and severe groups. Severe group was composed by older patients in comparison with the other three groups (p = 0.0083). Regarding sex, there was no significant difference between groups (p = 0.6279). In an ordinal regression model, only LTL and diabetes were significantly associated with disease severity. Shorter telomere length was significantly associated with the severity of COVID-19 infection, which can be useful as a biomarker or to better understand the SARS-CoV-2 pathophysiology.

Pyeloplasty in Adults With Ureteropelvic Junction Obstruction in Poorly Functioning Kidneys: A Systematic Review.

OBJECTIVES: To systematically summarize the available evidence concerning the impact of pyeloplasty on symptoms and differential renal function (DRF) in adults with unilateral UPJO in poorly functioning kidneys (PFK), and to identify potential predictors of kidney function recovery that could help clinicians select candidates for pyeloplasty. METHODS: A literature search (MEDLINE, Embase, Google Scholar, Scopus, ClinicalTrials.gov, and the WHO Clinical Trials Registry) and systematic review were performed up to September 2020 according to the PRISMA guidelines. PFK were defined as a baseline DRF ≤30% on renal scintigraphy. The primary endpoints were symptom relief and postoperative scintigraphic DRF. Predictors of kidney function recovery were evaluated and compared among studies. RESULTS: Nine studies comprising 731 patients met the inclusion criteria and were included for evidence synthesis. A DRF increase >5% occurred in 13.3%-53.8% of 160 patients with a pre- and postoperative renal scan. Symptoms improved in 73.3%-93.3% of 141 adults after pyeloplasty. Neither patient's age, baseline DFR, comorbidities, degree of hydronephrosis, kidney parenchymal thickness, nor kidney biopsy findings consistently predicted a significant DRF increase among 375 patients undergoing pyeloplasty. CONCLUSION: Based on a low level of evidence, pyeloplasty may relieve symptoms and stabilize kidney function in adults with UPJO in PFK. A significant number of patients showed a DRF increase >5%, yet no consistent predictor of kidney function recovery was identified. Until more evidence becomes available, pyeloplasty could be considered for selected cases after accounting for the risks of a failure requiring a future nephrectomy.

Genetic and clinical aspects of paediatric pheochromocytomas and paragangliomas.

OBJECTIVE: Few and conflicting reports have characterized the genetics of paediatric pheochromocytomas and paragangliomas (PPGLs). This study aimed to investigate the clinical and genetic features of Brazilian children with PPGL. PATIENTS AND METHODS: This study included 25 children (52% girls) with PPGL. The median age at diagnosis was 15 years (4-19). The median time of follow-up was 145 months. The genetic investigation was performed by Sanger DNA sequencing, multiplex ligation-dependent probe amplification and/or target next-generation sequencing panel. RESULTS: Of the 25 children with PPGL, 11 (44%), 4 (16%), 2 (8%), 1 (4%) and 7 (28%) had germline VHL pathogenic variants, SDHB, SDHD, RET and negative genetic investigation, respectively. Children with germline VHL missense pathogenic variants were younger than those with SDHB or SDHD genetic defects [median (range), 12 (4-16) vs. 15.5 (14-19) years; P = .027]. Moreover, 10 of 11 cases with VHL pathogenic variants had bilateral pheochromocytoma (six asynchronous and four synchronous). All children with germline SDHB pathogenic variants presented with abdominal paraganglioma (one of them malignant). The two cases with SDHD pathogenic variants presented with head and neck paraganglioma. Among the cases without a genetic diagnosis, 6 and 2 had pheochromocytoma and paraganglioma, respectively. Furthermore, metastatic PPGL was diagnosed in four (16%) of 25 PPGL. CONCLUSIONS: Most of the paediatric PPGL were hereditary and multifocal. The majority of the affected genes belong to pseudohypoxic cluster 1, with VHL being the most frequently mutated. Therefore, our findings impact surgical management and surveillance of children with PPGL.

Short-term outcomes of pyeloplasty vs. nephrectomy in adult patients with ureteropelvic junction obstruction and differential renal function ≤15.

OBJECTIVES: To compare symptom resolution and short-term renal function after pyeloplasty or nephrectomy in adults with ureteropelvic junction obstruction (UPJO) in poorly functioning renal units (PFRU). METHODS: Retrospective analysis of adult patients with UPJO and differential renal function (DRF) ≤ 15% who underwent laparoscopic pyeloplasty or nephrectomy. Primary endpoints included symptom resolution and estimated glomerular filtration rate (eGFR) at 12 months. Surgical complications were compared between groups. A secondary analysis was performed comparing baseline and postoperative DRF to evaluate the PFRU recovery potential after pyeloplasty. RESULTS: Sixty-three patients were included; 19 underwent pyeloplasty and 44 underwent nephrectomy. The mean age of the cohort was 39.5 ± 13.8 years. Nephrectomy was associated with significantly higher intra-operative blood loss (p = 0.02). Operative time and length of hospital stay were not significantly different between groups. There were three complications in the nephrectomy group, and none in the pyeloplasty group (p = 0.34). Symptom resolution rates were equivalent between groups (73% vs. 76%; p = 0.78). The eGFR variation was not statistically different after pyeloplasty or nephrectomy (+6.2 vs. +0.1 mL/min/1.73m2, respectively; p = 0.18). Patients undergoing pyeloplasty had no significant change in the mean DRF (baseline 9.5 vs. 10%; p = 0.99). CONCLUSION: Pyeloplasty can be considered for selected patients with UPJO in PFRU as an organ-sparing alternative to nephrectomy. Although there was no significant gain in mean DRF, pyeloplasty prevented further functional loss and relieved symptoms in most cases in the short-term with at least the same complication rates of nephrectomy.