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Objective: To characterize clinical, hemodynamic, imaging, and pathologic findings in children with pulmonary arterial hypertension (PAH) and variants in SRY-box transcription factor 17 (SOX17), a novel risk gene linked to heritable and congenital heart disease-associated PAH., Study Design: We assembled a multi-institutional cohort of children with PAH and SOX17 variants enrolled in the Pediatric Pulmonary Hypertension Network (PPHNet) and other registries. Subjects were identified through exome and PAH gene panel sequencing. Data were collected from registries and retrospective chart review., Results: We identified 13 children (8 female, 5 male) aged 1.6-16 years at diagnosis with SOX17 variants and PAH. Seven patients had atrial septal defects and 2 had patent ductus arteriosus. At diagnostic cardiac catheterization, patients had severely elevated mean pulmonary artery (PA) pressure (mean 78, range 47-124 mmHg) and markedly elevated indexed pulmonary vascular resistance (mean 25.9, range 4.9-55 WU∗m 2 ). No patients responded to acute vasodilator testing. Catheter and computed tomography angiography imaging demonstrated atypical PA anatomy including severely dilated main pulmonary arteries, lack of tapering in third and fourth order pulmonary arteries, tortuous 'corkscrewing' pulmonary arteries, and abnormal capillary 'blush.' Several children had PA stenoses and 2 had systemic arterial abnormalities. Histologic examination of explanted lungs from 3 patients disclosed plexiform arteriopathy and extensive aneurysmal dilation of alveolar septal capillaries., Conclusions: SOX17-associated PAH is a distinctive genetic syndrome characterized by early onset severe PAH, extensive pulmonary vascular abnormalities, and high prevalence of congenital heart disease with intracardiac and interarterial shunts, suggesting a role for SOX17 in pulmonary vascular development., Competing Interests: Declaration of Competing Interest The PPHNet Registry was supported by grant U01 HL12118 (Data Fusion: A Sustainable, Open Source Registry Advancing Pediatric Pulmonary Vascular Disease Research; K.D. Mandl and S.H. Abman) from the National Heart, Lung, and Blood Institute (NHLBI) of the National Institutes of Health (NIH) Work for this paper was also supported by a philanthropic grant for SOX17 research to the Boston Children's Hospital Pulmonary Hypertension Program, the Jayden de Luca Foundation, the Manton Center for Orphan Disease Research/Manton Foundation, and by NIH/NCATS Colorado CTSA Grant Number UL1 TR002535. Contents are the authors' sole responsibility and do not necessarily represent official NIH or NHLBI views., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Background: Bronchiectasis is one of the most common comorbidities in severe asthma. However, the mechanisms by which asthma promotes the development and progress of this condition are not well defined. This study aimed to analyze the inflammatory phenotypes and quantify the expression of proinflammatory and remodeling cytokines in asthma patients with and without bronchiectasis., Methods: The study sample comprised individuals with severe asthma and bronchiectasis (group AB, n=55) and a control population of individuals with severe asthma without bronchiectasis (group AC, n=45). Induced sputum samples were obtained and cell types determined by differential cell count. Proinflammatory and bronchial remodeling cytokines (IL-8, neutrophilic elastase, TGFβ1, VEGF, IFN-γ, TNF-α, and GM-CSF) were analyzed by immunoassay in sputum supernatant., Results: Neutrophilic inflammation was the primary phenotype in both asthma groups. Higher levels of TGFβ1, VEGF and IFN-γ were observed in asthma patients with bronchiectasis (group AB) than in controls (group AC) (15 vs 24pg/ml, p=0.014; 183 vs 272pg/ml, p=0.048; 0.85 vs 19pg/ml, p<0.001, respectively). Granulocyte-macrophage colony-stimulating factor (GM-CSF) levels were significantly lower in the AB group than in the AC group (1.2 vs 4.4pg/ml, p<0.001). IL-8, neutrophil elastase and TNF-α did not present significant differences between the groups., Conclusions: Raised levels of TGFβ1 and VEGF cytokines may indicate airway remodeling activation in asthma patients with bronchiectasis. The type of inflammation in asthma patients did not differ according to the presence or absence of bronchiectasis., (Copyright © 2024 SEPAR. Published by Elsevier España, S.L.U. All rights reserved.)

Objetive: To assess the ability of an artificial intelligence software to detect pneumothorax in chest radiographs done after percutaneous transthoracic biopsy., Material and Methods: We included retrospectively in our study adult patients who underwent CT-guided percutaneous transthoracic biopsies from lung, pleural or mediastinal lesions from June 2019 to June 2020, and who had a follow-up chest radiograph after the procedure. These chest radiographs were read to search the presence of pneumothorax independently by an expert thoracic radiologist and a radiodiagnosis resident, whose unified lecture was defined as the gold standard, and the result of each radiograph after interpretation by the artificial intelligence software was documented for posterior comparison with the gold standard., Results: A total of 284 chest radiographs were included in the study and the incidence of pneumothorax was 14.4%. There were no discrepancies between the two readers' interpretation of any of the postbiopsy chest radiographs. The artificial intelligence software was able to detect 41/41 of the present pneumothorax, implying a sensitivity of 100% and a negative predictive value of 100%, with a specificity of 79.4% and a positive predictive value of 45%. The accuracy was 82.4%, indicating that there is a high probability that an individual will be adequately classified by the software. It has also been documented that the presence of Port-a-cath is the cause of 8 of the 50 of false positives by the software., Conclusions: The software has detected 100% of cases of pneumothorax in the postbiopsy chest radiographs. A potential use of this software could be as a prioritisation tool, allowing radiologists not to read immediately (or even not to read) chest radiographs classified as non-pathological by the software, with the confidence that there are no pathological cases., (Copyright © 2023 SERAM. Published by Elsevier España, S.L.U. All rights reserved.)

Background/aim: The robotic retroperitoneal approach for renal mass surgery was introduced in 2018 at the Department of Urology in the clinic of Leverkusen, Germany. Clinical criteria for the choice of the access site (trans- vs. retroperitoneal) are not clearly defined. The aim of this study was to explore the learning curve and the impact of the access site on clinical outcome, in order to elucidate which preoperative clinical criteria should be taken into account when choosing the renal approach site., Patients and Methods: This retrospective study included 107 patients who underwent robotic tumor surgery between June 2018 and March 2022 at the Department of Urology in the Clinic of Leverkusen, Germany. Data from 86 patients with transperitoneal robotic surgery of the kidney and 21 patients with retroperitoneal access were available for analysis. We evaluated the data of patients in a trans- and a retroperitoneal access group. The preoperative clinical data included anthropomorphic data, the Body Mass Index (BMI) as well as the Preoperative Aspects and Dimensions Used for Anatomical Classification of Renal Masses (PADUA) - score. Intraoperative and postoperative data such as blood loss, clamping time, renal function and the learning curve of the surgeons was used to evaluate the outcomes of the two groups., Results: Operation time in the retroperitoneal group was significantly shorter (p=0.015). Operation-specific variables showed no significant difference between the two groups. PADUA score and hilar clamping time showed no difference (p=0.345 and p=0.130, respectively). The learning curve in the retroperitoneal access group unveiled a noticeable difference in the experience and mastery of the involved surgeons., Conclusion: Mastery of the retroperitoneal approach is readily possible for surgeons with previous experience in robotic renal surgery without compromising the operative morbidity. The PADUA-score seems most suitable as a preoperative clinical criterion for choosing the renal approach site., (Copyright © 2023 International Institute of Anticancer Research (Dr. George J. Delinasios), All rights reserved.)

Introduction: Since the beginning of the pandemic in 2020, COVID-19 has changed the medical landscape. International recommendations for localized prostate cancer (PCa) include deferred treatment and adjusted therapeutic routines., Materials and Methods: To longitudinally evaluate changes in PCa treatment strategies in urological and radiotherapy departments in Germany, a link to a survey was sent to 134 institutions covering two representative baseline weeks prior to the pandemic and 13 weeks from March 2020 to February 2021. The questionnaire captured the numbers of radical prostatectomies, prostate biopsies and case numbers for conventional and hypofractionation radiotherapy. The results were evaluated using descriptive analyses., Results: A total of 35% of the questionnaires were completed. PCa therapy increased by 6% in 2020 compared to 2019. At baseline, a total of 69 radiotherapy series and 164 radical prostatectomies (RPs) were documented. The decrease to 60% during the first wave of COVID-19 particularly affected low-risk PCa. The recovery throughout the summer months was followed by a renewed reduction to 58% at the end of 2020. After a gradual decline to 61% until July 2020, the number of prostate biopsies remained stable (89% to 98%) during the second wave. The use of RP fluctuated after an initial decrease without apparent prioritization of risk groups. Conventional fractionation was used in 66% of patients, followed by moderate hypofractionation (30%) and ultrahypofractionation (4%). One limitation was a potential selection bias of the selected weeks and the low response rate., Conclusion: While the diagnosis and therapy of PCa were affected in both waves of the pandemic, the interim increase between the peaks led to a higher total number of patients in 2020 than in 2019. Recommendations regarding prioritization and fractionation routines were implemented heterogeneously, leaving unexplored potential for future pandemic challenges., Competing Interests: The authors have declared that no competing interests exist.

Background: To investigate infectious and non-infectious complications after transperineal prostate biopsy (TPB) without antibiotic prophylaxis in a multicenter cohort. Secondly, to identify whether increasing the number of cores was predictive for the occurrence of complications. Thirdly, to examine the relation between TPB and erectile dysfunction., Methods: We analyzed a retrospective multicenter cohort of 550 patients from three different urological centers undergoing TPB without antibiotic prophylaxis. The median number of cores was 26. Demographic and clinical data were extracted by reviewing patients' electronic medical records and follow-up data such as postoperative complications obtained by structured phone interviews. To investigate the influence of the number of cores taken on the occurrence of complications, we performed univariate and multivariate mixed effects logistic regression models., Results: There was no case of sepsis reported. Overall, 6.0% of patients (33/550) presented with any complication besides mild macrohematuria. In all, 46/47 (98%) complications were ≤Grade 2 according to Clavien-Dindo. In multivariate regression analyses, an increased number of cores was associated with overall complications (odds ratio (OR) 1.08, 95% confidence interval (CI) 1.02-1.14, P = 0.01) and specifically bleeding complications (OR 1.28, 95% CI 1.11-1.50, P = 0.01) but not with infectious complications (OR 1.03, 95% CI 0.97-1.10, P = 0.67). A total of 14.4% of patients referred impairment of erectile function after TPB. Of note, 98% of these men were diagnosed with prostate cancer., Conclusions: This is the first multicenter trial to investigate complications after TPB without antibiotic prophylaxis. In our study, we found no case of sepsis. This underlines the safety advantage of TPB even without antibiotic prophylaxis and supports the ongoing initiative to abandon TRB of the prostate. A higher number of cores were associated with an increase in overall complications specifically bleeding complications, but not with infectious complications. Post-biopsy erectile dysfunction was mainly present in patients diagnosed with PCa., (© 2021. The Author(s).)

Though asthma and bronchiectasis are two different diseases, their coexistence has been demonstrated in many patients. The aim of the present study is to compare the characteristics of asthmatic patients with and without bronchiectasis and to assess risk factors for the development of this condition. Two hundred and twenty-four moderate-severe asthmatic patients were included. The severity of bronchiectasis was assessed by Reiff and FACED parameters. Logistic regression was used to identify independent factors associated with bronchiectasis. Bronchiectasis was identified in 78 asthma patients. In severe asthma patients, its prevalence was 56.9%. Bronchiectasis was defined as mild in81% of patients using modified Reiff criteria and in 74% using FACED criteria. Asthmatic patients with bronchiectasis had decreasing FEV1, FVC and FEV1/FVC (p = 0.002, 0.005 and 0.014 respectively), presented more frequent asthma exacerbations (p < 0.001) and worse asthma control (ACT 21 vs 16pts, p < 0.001). Factors independently associated with bronchiectasis were older age (42-65 years: OR, 3.99; 95% CI 1.60 to 9.95, P = 0.003; ≥ 65 years: OR, 2.91; 95% CI 1.06 to 8.04, P = 0.039), severe asthma grade (OR, 8.91; 95% CI 3.69 to 21.49; P < 0.001) and frequency of asthma exacerbations (OR, 4.43; 95% CI 1.78 to 11.05; P < 0.001). In patients with severe asthma, age of asthma onset (OR, 1.02; 95% CI 1.01 to 1.04; P = 0.015) and asthma exacerbations (OR, 4.88; 95% CI 1.98 to 12.03; P = 0.001) were independently associated with the development of bronchiectasis. The prevalence of bronchiectasis in severe asthmatic patients is high. Age of asthma onset and exacerbations were independent factors associated with the occurrence of bronchiectasis., (© 2021. The Author(s).)

Introduction: The aim of the study is to compare length of hospital stay, transfusion rates, and re-intervention rates during hospitalization for transurethral resection of the prostate (TUR-P), open prostatectomy (OP), and laser therapy (LT) for surgical treatment of benign prostatic obstruction (BPO)., Methods: URO-Cert is an organization, in which clinical data of prostatic diseases from 2 university, 19 public, and 3 private hospitals and 270 office-based urologists are collected in order to document treatment quality. Data on diagnostics, therapy, and course of disease are recorded web based. The analysis includes datasets from 2005 to 2017., Results: Of 10,420 patients, 8,389 were treated with TUR-P, 1,334 with OP, and 697 with LT. Median length of hospital stay was 6 days (IQR: 4-7) for TUR-P, 9 days (IQR: 7-11) for OP, and 5 days (IQR: 4-6) for LT (p < 0.001). Risk for a hospital stay ≥7 days was higher for OP versus TUR-P (OR: 7.25; 95% CI = 6.27-8.36; p < 0.001) and LT (OR: 17.89; 95% CI = 14.12-22.65; p < 0.001) and higher for TUR-P versus LT (OR: 2.47; 95% CI = 2.03-3.01; p < 0.001). OP had a significantly higher risk for transfusions than TUR-P (OR: 2.44; 95% CI = 1.74-3.41; p < 0.001) and LT (OR: 3.32; 95% CI = 1.56-7.01; p < 0.001). Transfusion rates were not significantly different between TUR-P and LT (OR: 1.36; 95% CI = 0.66-2.79; p = 0.51). Risk of re-intervention was not different between all 3 approaches., Conclusion: OP was associated with higher transfusion rates and longer hospital stay than TUR-P and LT. Risk of transfusion was not different between TUR-P and LT, but TUR-P was inferior to LT concerning length of hospital stay. Re-intervention rates during hospitalization did not differ between the groups., (© 2021 S. Karger AG, Basel.)

Objectives: To evaluate retrospectively the surgical, symptomatic and oncological outcomes of pelvic exenteration surgery (PES) in men with significant intrapelvic complications of locally advanced castration-sensitive (CSPC) and castration-resistant prostate cancer (CRPC)., Patients and Methods: A total of 103 patients with locally advanced progressive and symptomatic CSPC or CRPC underwent PES (radical cystoprostatectomy, n = 71 [68.9%]; radical prostatectomy with continent vesicostomy, n = 9 [8.7%]; total exenteration, n = 23 [22.3%]). All patients underwent local staging via magnetic resonance imaging, cystoscopy and rectoscopy. Systemic staging was carried out with chest, abdominal and pelvic computed tomography scans and bone scans. Peri-operative complications were assessed according to Clavien-Dindo classification. Symptom-free and overall survival were evaluated using the Kaplan-Meier method. Statistical tests were two-tailed with a P value <0.05 taken to indicate statistical significance., Results: After a median (range) follow-up of 36.5 (3-123) months, the symptom-free survival rate at 1 and 3 years was 89.2% (n = 89) and 64.1% (n = 66), respectively. The median symptom-free survival was 27.9 months. A total of 78.6% of the patients were symptom-free during their remaining lifetime. The overall survival rate at 1 and 3 years was 92.2% and 43.7%, respectively, and the median overall survival was 33.6 months. Clavien-Dindo grades 2, 3 and 4 complications developed in 31 (30.6%), 12 (11.6%) and eight patients (8.1%), respectively., Conclusion: Pelvic exenteration surgery is technically feasible in well-selected patients, resulting in symptom relief in >90% of patients, covering 80% of their remaining lifetime., (© 2020 The Authors BJU International © 2020 BJU International Published by John Wiley & Sons Ltd.)

Competing Interests: Erklärung zu finanziellen InteressenForschungsförderung erhalten: nein; Honorar/geldwerten Vorteil für Referententätigkeit erhalten: ja, von einer anderen Institution (Pharma- oder Medizintechnikfirma usw.); Bezahlter Berater/interner Schulungsreferent/Gehaltsempfänger: nein; Patent/Geschäftsanteile/Aktien (Autor/Partner, Ehepartner, Kinder) an Firma (Nicht-Sponsor der Veranstaltung): nein; Patent/Geschäftsanteile/Aktien (Autor/Partner, Ehepartner, Kinder) an Firma (Sponsor der Veranstaltung): nein. Erklärung zu nichtfinanziellen InteressenDie Autorinnen/Autoren geben an, dass kein Interessenkonflikt besteht.

Background/aim: The aim of this study was to evaluate the diagnostic sensitivity, specificity and accuracy of endorectal magnetic resonance imaging (e-MRI), as a preoperative staging modality in the diagnosis of lymph node metastasis (LNM) in patients with prostate cancer (PCa)., Patients and Methods: Retrospectively, we analyzed data from N=168 patients who underwent radical prostatectomy (RP) between 2004 and 2013 at two tertiary medical centres. Prior to RP all patients underwent an e-MRI. Inclusion criteria were: PSA levels >20 ng/ml or Gleason score >7. Examinations were performed on a closed 1.0-T system combined with an endorectal body phased-array coil, imaging results were correlated with histopathology., Results: 10.7% (N=18 patients) had histologically-proven LNM. e-MRI was true-positive in N=6 (33.3%) and false-negative N=12 patients (66.6%). N=150 (89.3%) patients without LNM e-MRI were true-negative in 96% and false-positive in 4%. Sensitivity was 96%, specificity was 33%, accuracy was 64.5%., Conclusion: e-MRI can be considered a useful preoperative staging modality in diagnosis of LNM., (Copyright© 2018, International Institute of Anticancer Research (Dr. George J. Delinasios), All rights reserved.)

Vesicouterine fistulas (VUFs) are a pathological communication between uterus and urinary bladder, which are mainly related to iatrogenic lesions during caesarean sections or occur during vaginal deliveries after a previous caesarean section. The main symptoms are menstruation through the bladder (menouria), amenorrhea and urinary incontinence. Treatment can be conservative, hormonal or surgical; however, a spontaneous closure of the fistula is rare (5 %). We report a case of a female VUF with menouria after caesarean section with a successful surgical fistula excision. Based on this case report, we analyse the causes, symptoms, diagnostics and treatment of VUF as reported in the literature., Competing Interests: Es besteht kein Interessenkonflikt. Der korrespondierende Autor versichert, dass keine Verbindungen mit einer Firma, deren Produkt in dem Artikel genannt ist, oder einer Firma, die ein Konkurrenzprodukt vertreibt, bestehen., (© Georg Thieme Verlag KG Stuttgart · New York.)

Urethral strictures are independent of a patient's age and can happen in every life period. They are often iatrogenic, resulting from a transurethral surgical procedure or traumatic catheterisation. Endoscopic slotting is the surgery of choice; although this method is frequently associated with recurrence. Retrograde urethrography or cystourethrography continue to play a central role in diagnostic evaluation and treatment. There is no standardised procedure regarding the surgical technique, perioperative management, and postoperative follow-up. Evidence-based recommendations or guidelines do not exist. For a successful surgical treatment of the urethra it is important to have an excellent knowledge of the urethral and urogenital anatomy. The permanent removal of urethral strictures can only be achieved by open reconstructive surgery. Direct end-to-end urethroplasty is used, in general, for short bulbar urethral strictures, while urethroplasty with oral mucosa is used for longer bulbar and penile strictures. Urethral reconstruction using a patient-specific autologous cell transplant with MukoCell ® is an alternative to the conventional transplantation with native oral mucosa. Urethral reconstruction should be performed in specialised centres with appropriate expertise., Competing Interests: Interessenkonflikt: Die Autoren geben an, dass kein Interessenkonflikt vorliegt., (© Georg Thieme Verlag KG Stuttgart · New York.)

A female urethral diverticulum (UD) is a rare condition with a prevalence of 0.6 - 4.7 %. Possible aetiologies include chronic infection of the periurethral glands, childbirth trauma, and iatrogenic lesions after urethral manipulation. The main symptoms are recurrent urinary tract infections, post-void dribbling and leakage of urine or purulent discharge by movement, which is caused by the emptying of the diverticular lumen (paradoxical incontinence). As this may imitate stress urinary incontinence, the final diagnosis is a challenge for urologists. We report the case of a female UD containing multiple calculi, which were diagnosed during diagnostic work-up of incontinence. Based on this case report, we want to present and analyse the symptoms as well as the diagnostic evaluation and treatment of UD as reported in the literature. Also we wish to emphasise that the differential diagnosis between a complicated diverticulum and stress urinary incontinence requires a precise knowledge of the symptoms and the diagnostic algorithm for the detection of a DU because the symptoms of these two conditions are similar., Competing Interests: Interessenkonflikt: Die Autoren geben an, dass kein Interessenkonflikt besteht., (© Georg Thieme Verlag KG Stuttgart · New York.)

Objectives: The aim of this report is to identify the radiological findings of unilateral tuberculous lung destruction (UTLD)., Materials and Methods: Thirteen patients with (UTLD) were reviewed from 1999 to 2014. Only patients with radiological evidence of absence of pulmonary parenchyma preserved were included. Clinical and demographic data were obtained and radiological studies (chest radiograph and CT) were retrospectively reviewed., Results: The left lung was more commonly involved (85%). The following radiological findings were found in all cases: a decrease in the diameter of the pulmonary vessels of the affected lung, herniation of the contralateral lung and hypertrophy of the ribs and/or thickening of extrapleural fat. Two radiological patterns were identified: UTLD with cystic bronchiectasis (85%) and UTLD without residual cystic bronchiectasis (15%). Forty-six per cent of cases had respiratory infection symptoms with presence of air-fluid levels in the affected lung as the most common finding in these patients., Conclusions: Total unilateral post-tuberculous lung destruction is an irreversible complication with the following main radiological features: predominantly left-sided location, decreases in the diameter of the ipsilateral pulmonary vessels, herniation of the contralateral lung and hypertrophy of the ribs and/or thickening of extrapleural fat., Teaching Points: • Unilateral tuberculous lung destruction is an irreversible complication of tuberculosis. • Left-side predominance and herniation of the contralateral lung are characteristic. • Decreased diameter of the ipsilateral pulmonary vessels occurred in all patients. • The pattern with residual cystic bronchiectasis is the most frequent. • Superimposed non-tuberculous infections may affect the destroyed lung.

Background: Malignancies and cisplatin-based chemotherapy are both known to correlate with a high risk of venous thrombotic events (VTT). In testicular cancer, the information regarding the incidence and reason of VTT in patients undergoing cisplatin-based chemotherapy is still discussed controversially. Moreover, no risk factors for developing a VTT during cisplatin-based chemotherapy have been elucidated so far., Patients and Methods: We retrospectively analyzed 153 patients with testicular cancer undergoing cisplatin-based chemotherapy at our institution for the development of a VTT during or after chemotherapy. Clinical and pathological parameters for identifying possible risk factors for VTT were analyzed. The Khorana risk score was used to calculate the risk of VTT. Student t test was applied for calculating the statistical significance of differences between the treatment groups., Results: Twenty-six out of 153 patients (17%) developed a VTT during chemotherapy. When we analyzed the risk factors for developing a VTT, we found that Lugano stage ≥IIc was significantly (p = 0.0006) correlated with the risk of developing a VTT during chemotherapy. On calculating the VTT risk using the Khorana risk score model, we found that only 2 out of 26 patients (7.7%) were in the high-risk Khorana group (≥3)., Conclusion: Patients with testicular cancer with a high tumor volume have a significant risk of developing a VTT with cisplatin-based chemotherapy. The Khorana risk score is not an accurate tool for predicting VTT in testicular cancer., (© 2017 S. Karger AG, Basel.)