A 6-year review of acute post-streptococcal glomerulonephritis at a public children's hospital in Cape Town, South Africa.

BACKGROUND: Acute post-streptococcal glomerulonephritis (APSGN) is the most common cause of acute nephritis in children globally and, in some cases, may be associated with progressive kidney injury and failure, cumulating in the need for long-term dialysis and/or kidney transplantation. METHODS: Our retrospective study describes the occurrence of APSGN among children (< 14 years) admitted to a tertiary children's hospital in Cape Town, South Africa, from January 2015 to December 2020. RESULTS: Of 161 children who presented with acute nephritis (haematuria, oedema, oliguria, and hypertension), 100 met the inclusion criteria. Demographic, clinical features, laboratory findings, management, and outcome data were collected. APSGN was defined by the clinical presentation of at least two clinical signs of acute nephritis, and low serum complement 3 (C3) level or evidence of a recent streptococcal infection. Most cases of APSGN were associated with streptococcal skin infections: 55/100 (55%); 10/100 (10%) children presented with hypertensive seizures; C3 levels were low in 86/92 (93.5%) children; 94/94 (100%) children had elevated anti-deoxyribonuclease-B (anti-DNase-B) levels; and 80/94 (85%) also had elevated anti-streptolysin O titre (ASOT) at presentation. Eleven (11%) children had a percutaneous kidney biopsy; 4/11 (36%) showed histological features of post-infectious nephritis, and 7/11(64%) also had crescentic glomerulonephritis with immune complex deposits. Sixty-two (62%) children confirmed recovered, and five (5%) progressed to kidney failure, but 29 presumed recovered as they did not return for follow-up to our institution. CONCLUSIONS: Childhood APSGN remains an important health problem in South Africa (SA) with favourable outcomes in most, apart from those with crescentic glomerulonephritis who progressed to kidney failure.

Detection of Alport gene variants in children and young people with persistent haematuria.

BACKGROUND: Genetic kidney disease is an important cause of persistent microscopic haematuria in children and young people. We aimed to determine the frequency of variants in the Alport syndrome genes (COL4A3, COL4A4 or COL4A5) in individuals under 18 years of age presenting with persistent microscopic haematuria to a single specialist centre in the UK over a 10-year period. METHODS: We conducted a retrospective longitudinal study of individuals referred to a tertiary paediatric nephrology service with persistent microscopic haematuria between April 2012 to 2022. RESULTS: A total of 224 individuals (female 51.8%) were evaluated with persistent microscopic haematuria of greater than 6 months duration. The age at presentation was 7.5 ± 4.3 years (mean ± SD) with a duration of follow-up of 6.8 ± 4.6 years (mean ± SD). Targeted exome sequencing was performed in 134 individuals and 91 (68%) had a pathogenic or likely pathogenic variant in COL4A3, COL4A4 or COL4A5. Only 49.5% of individuals with identified variants had a family history of microscopic haematuria documented and 37.4% (34/91) had additional proteinuria at presentation. COL4A5 was the most common gene affected and missense variants affecting glycine residues were the most common variant type. CONCLUSION: Over two-thirds of children and young people who underwent genetic testing had an identifiable genetic basis for their microscopic haematuria and over half did not have a documented family history. Genetic testing should be part of the evaluation of persistent microscopic haematuria despite a negative family history.

Kidney outcomes associated with haematuria and proteinuria trajectories among patients with IgA nephropathy in real-world clinical practice: The Japan Chronic Kidney Disease Database.

AIM: Among patients with Immunoglobulin A (IgA) nephropathy, we aimed to identify trajectory patterns stratified by the magnitude of haematuria and proteinuria using repeated urine dipstick tests, and assess whether the trajectories were associated with kidney events. METHODS: Using a nationwide multicentre chronic kidney disease (CKD) registry, we analysed data from 889 patients with IgA nephropathy (mean age 49.3 years). The primary outcome was a sustained reduction in eGFR of 50% or more from the index date and thereafter. During follow-up (median 49.0 months), we identified four trajectories (low-stable, moderate-decreasing, moderate-stable, and high-stable) in both urine dipstick haematuria and proteinuria measurements, respectively. RESULTS: In haematuria trajectory analyses, compared to the low-stable group, the adjusted hazard ratios (HRs) (95% confidence interval [CI]) for kidney events were 2.59 (95% CI, 1.48-4.51) for the high-stable, 2.31 (95% CI, 1.19-4.50) for the moderate-stable, and 1.43 (95% CI, (0.72-2.82) for the moderate-decreasing groups, respectively. When each proteinuria trajectory group was subcategorized according to haematuria trajectories, the proteinuria group with high-stable and with modest-stable haematuria trajectories had approximately 2-times higher risk for eGFR reduction ≥50% compared to that with low-stable haematuria trajectory. CONCLUSION: Assessments of both haematuria and proteinuria trajectories using urine dipstick could identify high-risk IgA nephropathy patients.

Prevalence of prostate cancer in men with haematuria: a systematic review and meta-analysis.

OBJECTIVES: To investigate the prevalence of prostate cancer in men attending evaluation for haematuria, as this could help healthcare providers to determine whether men with haematuria should have prostate examinations performed. METHODS: The study was performed according to a pre-specified protocol uploaded to the International Prospective Register of Systematic Reviews (PROSPERO; CRD42022299383). A systematic search of MEDLINE, Ovid and Google Scholar was performed in December 2021. Two independent researchers evaluated all titles, available abstracts, and full texts. We included studies on adult men (aged ≥18 years) describing haematuria and prostate cancer. RESULTS: We screened 4252 titles and abstracts when available and assessed 350 studies in full text. In total, 65 studies were included and 42 was summarised in a meta-analysis. In total, 18 752 men with haematuria were included, and the pooled prevalence (95% confidence interval [CI]) of prostate cancer was 3.0% (2.0-4.1%). In men with macroscopic haematuria, the pooled prevalence (95% CI) of prostate cancer was 5.9% (2.9-9.9%; n = 265/5373). In men with microscopic haematuria, the pooled prevalence (95% CI) of prostate cancer was 1.4% (0.8-2.2%; n = 71/6642). CONCLUSION: Our findings indicate that the prevalence of prostate cancer is considerable in men attending evaluation for haematuria. Therefore, digital rectal examination and prostate-specific antigen measurement should become a standard procedure for all men with haematuria, especially for men with macroscopic haematuria.

Clinical and diagnostic utility of genomic sequencing for children referred to a Kidney Genomics Clinic with microscopic haematuria.

BACKGROUND: Microscopic haematuria in children is associated with the risk of progression to chronic kidney disease. Genetic disease is an important potential aetiology. Genomic sequencing presents the most effective diagnostic route for these conditions, but access remains inequitable internationally. METHODS: We conducted a retrospective review of the electronic medical records of a Kidney Genomics Clinic (KGC) from January 2016 to December 2021. RESULTS: Sixty patients were referred to the KGC with haematuria over this period. Forty-three percent of patients had analysis of a limited haematuria panel (COL4A1, COL4A3, COL4A4, COL4A5, MYH9) with 58% receiving a genetic diagnosis. Forty-two percent of referred patients had further analysis of genes implicated in the development of kidney disease, and 36% received a diagnosis. Eight percent of patients underwent cascade testing for a known familial variant, and all received a diagnosis. Children with the highest levels of haematuria (> 500 × 106/L red blood cells) had the highest diagnostic yield (67%). Proteinuria, defined as a urinary protein to creatinine ratio > 20, increased the diagnostic yield from 31 to 65%. Importantly, negative genetic analysis can still have significant clinical utility for patients by altering surveillance and further management; the genetic result had clinical utility in 60% of patients. CONCLUSIONS: Our KGC review highlights the substantial clinical utility and diagnostic yield of genomic analysis for microscopic haematuria in paediatric patients. Whilst the management of variants of uncertain significance can be challenging, a multidisciplinary team including genetic counselling can help ensure these patients are followed up meaningfully. A higher resolution version of the Graphical abstract is available as Supplementary information.

The unknown silent drug reaction in acne patients: rare case of isotretinoin-induced haematuria.

BACKGROUND: Acne vulgaris is one of the most frequent visits to primary care physicians and dermatologists alike. Isotretinoin is the backbone of acne treatment. In most countries, depending on the health care system, isotretinoin is prescribed by dermatologists but primary care physicians are a part of the follow-up and interpreting analysis. Adverse effects of isotretinoin on the kidney and urinary system are mostly limited to sparse case reports. Specifically, gross and microscopic haematuria is not mentioned to be associated with isotretinoin. Lack of data regarding these adverse effects can lead to doubt regarding further patient management not only with dermatologists but also primary care physicians. OBJECTIVE: We report a 16-year-old male patient with isotretinoin-induced haematuria with multiple episodes and subsequent challenge and de-challenge. No personal or familial history of nephrological disease was present. Ultrasound imaging and nephrology workup was within normal limits. Other aetiologies were excluded. Nephrology consult stated there was no contraindication for isotretinoin use and was reinstated at 0.6 m/kg/day. More frequent observation was indicated until completion of isotretinoin. CONCLUSION: Our case raises awareness to other dermatologists and primary care physicians that haematuria can be secondary to isotretinoin but not a contraindication for further use if asymptomatic and microscopic. More extensive evaluation and monitoring should be done if the patient is symptomatic with other abnormalities and symptoms. Urinalysis should be a part of routine follow-up monitoring in patients on isotretinoin. Furthermore, delineating and differentiating when to refer to a nephrologist is essential for physicians, patients, and the health care system overall.

Red blood cells in the urine (called haematuria), whether seen by the eye or seen only on urinalysis can be caused by many diseases and/or drugs. The most effective treatment of acne is isotretinoin and its side effects are for the most part known. Renal and/or urinary side effects are extremely rare. We report a 16-year-old male patient with isotretinoin-induced haematuria with multiple episodes and subsequent challenge and de-challenge. When isotretinoin was discontinued, no red blood cells were seen in the urine. When isotretinoin was reinstituted, red blood cells were seen once again in the urine. It is important for physicians to know of this rare side effect as it prevents unnecessary referrals to nephrologists, while on the other hand raises awareness of the connection and helps in understanding when isotretinoin should be potentially discontinued and patients referred.

Optimizing Upper-Tract Imaging for Non-Visible Haematuria.

INTRODUCTION: Non-visible haematuria (NVH) is associated with a small risk of upper-tract urothelial carcinoma (UTUC), though there is little consensus on its investigation, particularly with regard to upper-tract imaging. This study aimed to determine whether the presentation of UTUC can guide investigation of NVH in patients under 60 years old. METHODS: All patients investigated at our one-stop haematuria clinics under a cancer pathway were reviewed during a 5-year period, with all patients undergoing cystoscopy and upper-tract imaging. Retrospective analysis of all UTUC cases from our urological cancer multidisciplinary team meeting database over a 10-year period was also undertaken. RESULTS: 2,129 patients with a median age of 67 years underwent urgent investigation for haematuria between March 2015 and February 2020. 449 cases presented with NVH, of whom 124 (27.6%) were under 60. Out of 21 cases of UTUC, only 2 presented with NVH; both were over the age of 60 years. Factors that independently predicted diagnosis with urinary-tract malignancy were age ≥60 (OR 3.70, p < 0.001), visible haematuria (OR 2.50, p = 0.006), and suspicious cystoscopic findings (OR 58.06, p < 0.001). Review of all 119 UTUC cases over 10 years found 6 cases (5.0%) presenting with NVH, with one (0.8%) also presenting under 60 years. CONCLUSION: Diagnosis with UTUC is rare in patients presenting with NVH under the age of 60 years. Routine use of CTU in this low-risk group is best avoided, with ultrasonography constituting a safer first-line upper-tract imaging modality. Guidelines that risk-stratify NVH patients may be effective in reducing unnecessary investigations.

Arteriovenous malformation of the prostatic median lobe: a rare case treated by transurethral resection.

Arteriovenous malformation localised to the prostate is rare. Until recently, the gold standard for diagnosis was angiography; however, this changed with the use of computed tomography and magnetic resonance imaging, which quickly became the first-line diagnostic tool. Common complaints are haematuria and lower urinary tract symptoms, for which there are no well-defined management guidelines. We present the case of a 53-year-old male patient who was treated for clotted haematuria. While the bleeding was thought to originate from an enlarged prostate, cystoscopy displayed a non-pulsatile, exophytic, active bleeding mass on the median lobe. The mass was resected transurethrally and diagnosed as arteriovenous malformation. This case shows an aberrant presentation of a vascular malformation in the prostate. The mass seemed to be constrained to a compact area without a visible plurality of arterial feeders. Since the prostate is a rare location for arteriovenous malformation, there are no well-defined treatment options. Nevertheless, the mass appears to have been successfully extracted by transurethral resection.

Pseudodominant Alport syndrome caused by pathogenic homozygous and compound heterozygous COL4A3 splicing variants.

Alport syndrome is a genetic disorder affecting the basement membranes of the kidney, ear and eye, and represents a leading cause of monogenic kidney disease. Alport syndrome is genetically heterogeneous with three key genes involved (COL4A3-5) and several transmission patterns, including monogenic X-linked, autosomal recessive/dominant and digenic. We report a consanguineous family where 13 individuals presented variable features of Alport syndrome including kidney failure on two generations and male-to-male transmission, suggesting autosomal dominant inheritance. COL4A3-5 gene panel analysis surprisingly reveals two distinct, confirmed splice-altering variants in COL4A3 (NM_000091.4: c.1150+5G>A and c.4028-3C>T) present in homozygous or compound heterozygous state in individuals with kidney failure. This adds a further mode of transmission for Alport syndrome where, in a consanguineous family, the independent segregation of two variants at the same locus may create a pseudodominant transmission pattern. These findings highlight the importance of a molecular diagnosis in Alport syndrome for genetic risk counselling, given the variable modes of inheritance, but also the pitfalls of assuming identity by descent in consanguineous families.

A prospective evaluation of the fourth national Be Clear on Cancer 'Blood in Pee' campaign in England.

OBJECTIVE: To assess the impact of the fourth Be Clear on Cancer (BCoC) 'Blood in Pee' (BiP) campaign (July to September 2018) on bladder and kidney cancer symptom awareness and outcomes in England. METHODS: In this uncontrolled before and after study, symptom awareness and reported barriers to GP attendance were assessed using panel and one-to-one interviews. The Health Improvement Network (THIN), National Cancer Registration and Analysis Service (NCRAS) and NHS Cancer Waiting Times (CWT) data were analysed to assess the impact on GP attendances, urgent cancer referrals, cancer diagnoses and 1-year survival. Analyses used Poisson, negative binomial and Cox regression. RESULTS: Symptom awareness and intention to consult a GP after one episode of haematuria increased following the campaign. GP attendance with haematuria (rate ratio (RR) 1.17, 95% confidence interval (CI): 1.07-1.28) and urgent cancer referrals (RR 1.18 95% CI: 1.08-1.28) increased following the campaign. Early-stage diagnoses increased for bladder cancer (difference in percentage 2.8%, 95% CI: -0.2%-5.8%), but not for kidney cancer (difference -0.6%, 95% CI: -3.2%-2.1%). CONCLUSIONS: The fourth BCoC BiP campaign appears to have been effective in increasing bladder cancer symptom awareness and GP attendances, although long-term impacts are unclear.

The IDENTIFY study: the investigation and detection of urological neoplasia in patients referred with suspected urinary tract cancer - a multicentre observational study.

OBJECTIVE: To evaluate the contemporary prevalence of urinary tract cancer (bladder cancer, upper tract urothelial cancer [UTUC] and renal cancer) in patients referred to secondary care with haematuria, adjusted for established patient risk markers and geographical variation. PATIENTS AND METHODS: This was an international multicentre prospective observational study. We included patients aged ≥16 years, referred to secondary care with suspected urinary tract cancer. Patients with a known or previous urological malignancy were excluded. We estimated the prevalence of bladder cancer, UTUC, renal cancer and prostate cancer; stratified by age, type of haematuria, sex, and smoking. We used a multivariable mixed-effects logistic regression to adjust cancer prevalence for age, type of haematuria, sex, smoking, hospitals, and countries. RESULTS: Of the 11 059 patients assessed for eligibility, 10 896 were included from 110 hospitals across 26 countries. The overall adjusted cancer prevalence (n = 2257) was 28.2% (95% confidence interval [CI] 22.3-34.1), bladder cancer (n = 1951) 24.7% (95% CI 19.1-30.2), UTUC (n = 128) 1.14% (95% CI 0.77-1.52), renal cancer (n = 107) 1.05% (95% CI 0.80-1.29), and prostate cancer (n = 124) 1.75% (95% CI 1.32-2.18). The odds ratios for patient risk markers in the model for all cancers were: age 1.04 (95% CI 1.03-1.05; P < 0.001), visible haematuria 3.47 (95% CI 2.90-4.15; P < 0.001), male sex 1.30 (95% CI 1.14-1.50; P < 0.001), and smoking 2.70 (95% CI 2.30-3.18; P < 0.001). CONCLUSIONS: A better understanding of cancer prevalence across an international population is required to inform clinical guidelines. We are the first to report urinary tract cancer prevalence across an international population in patients referred to secondary care, adjusted for patient risk markers and geographical variation. Bladder cancer was the most prevalent disease. Visible haematuria was the strongest predictor for urinary tract cancer.

Does non-visible haematuria require urgent assessment? A retrospective cohort study from a university teaching hospital.

OBJECTIVES: It is not certain from current evidence which patient groups with non-visible haematuria (NVH) require urgent investigation and which investigations are sufficient. We report referral outcomes data from Scotland to identify patient groups who will benefit from urgent assessment to rule out urological cancer (UC) and whether full set of investigations are necessary in all referred patients. MATERIALS AND METHODS: Data were collected from electronic patient records for patients referred with NVH to secondary care urology services between July 2017 and May 2020. The correlations between risk factors and final diagnosis were assessed using categorical variables in a multivariate logistic regression analysis and using chi-squared models. Statistical analysis was performed using IBM SPSS data editor version 25. RESULTS: Our study cohort comprised 525 patients (43.4% males; median age 66 years), in which UC was diagnosed in 25 patients (4.8%). Age > 60 years had sensitivity and NPV for UC of 92% and 99%, respectively. Univariate and multivariate analysis showed male sex, age ≥ 60 years and smoking were significant predictors of UC in patients with NVH (p < 0.05). There was no significant difference in UC in patients with history of LUTS, anticoagulation and previous UC. CONCLUSION: The risk of urologic cancer in NVH patients is significant and male gender, age ≥ 60 years and smoking are significant predictors of UC. Patients with risk factors of UC require complete assessment of both the upper and lower urinary tract; however, in the absence of risk factors, patients do not require urgent or complete assessment.

Transarterial Embolization in the Management of Intractable Haemorrhage.

INTRODUCTION: The purpose of this study was to evaluate the effectiveness and long-term results of selective transarterial iliac embolization (STIE) in patients with intractable bladder haemorrhage (IBH). METHODS: Twenty-five patients with a median age of 84 (range 65-94) years underwent STIE because of IBH between 2002 and 2020. The median follow-up time was 3 (mean 13.9) months. Patients were treated because of bleeding bladder or prostate cancer, radiation-induced haemorrhagic cystitis, and other conditions. Success was defined as technical success (feasibility to embolize bilateral hypogastric arteries or neoplastic arteries) and as clinical success (absence of further or additional therapy). RESULTS: Twenty-five patients with a median age of 84 years with a median hospital stay of 7 days were embolized at our institution. In total, 60% required additional therapy. Only 20% had minor complications, but no complication major was seen; 60% needed an additional therapy because of continuous bleeding. Our 30-day, 90-day, 6-month, and 12-month mortality rates were 28, 44, 64, and 76%, respectively. CONCLUSIONS: STIE in IBH is a safe, well-tolerated, and feasible procedure for palliating haematuria patients in poor general condition. Major complications are very rarely seen. However, patients often need additional therapy after STIE.

Uretero-arterial fistula: Six new cases and systematic review of the literature.

AIM: Secondary uretero-arterial fistulas (SUAF) are uncommon, underrated and threatening for any patient. Gross hematuria is a clinical symptom of this pathology for patients with history of pelvic radiotherapy, complex pelvic surgery or long-term ureteral stenting. The purpose of this work is to assess risk factors, diagnosis and treatment of SUAF. METHODS: Monocentric and retrospective series of 6 new cases illustrated by a literature review through MedLine and Pubmed using the keywords "arterio-ureteral fistula", "arterio iliac fistula" and "ilio-ureteral fistula". We excluded uretero-arterial fistula following vascular surgery. RESULTS: Our series included 4 men and 2 women. All patients had a history of complex pelvic surgery and long-term ureteral stenting. Three patients had history of pelvic radiotherapy. They all had inaugural macroscopic haematuria episode. Two fistula cases were diagnosed on 5 repeated CT-scans. In 2 out of 5 cases, arteriography highlighted the fistula. Fistulas were generally located at the left common iliac artery. An endovascular stent was placed in 5 out of 6 cases. One patient needed open surgery. After treatment, 3 patients remained alive, 3 patients died either by a fistula relapse or by complications late in the treatment. CONCLUSION: SUAF are uncommon, but serious. Today, there is no specific recommendation regarding complex treatment of these fistulas. Endovascular stents seem to be a good therapeutic option. LEVEL OF PROOF: 3.

Imaging protocols for CT urography: results of a consensus conference from the French Society of Genitourinary Imaging.

OBJECTIVES: To develop technical guidelines for computed tomography urography. METHODS: The French Society of Genitourinary Imaging organised a Delphi consensus conference with a two-round Delphi survey followed by a face-to-face meeting. Consensus was strictly defined using a priori criteria. RESULTS: Forty-two expert uro-radiologists completed both survey rounds with no attrition between the rounds. Ninety-six (70%) of the initial 138 statements of the questionnaire achieved final consensus. An intravenous injection of 20 mg of furosemide before iodinated contrast medium injection was judged mandatory. Improving the quality of excretory phase imaging through oral or intravenous hydration of the patient or through the use of an abdominal compression device was not deemed necessary. The patient should be imaged in the supine position and placed in the prone position only at the radiologist's request. The choice between single-bolus and split-bolus protocols depends on the context, but split-bolus protocols should be favoured whenever possible to decrease patient irradiation. Repeated single-slice test acquisitions should not be performed to decide of the timing of excretory phase imaging; instead, excretory phase imaging should be performed 7 min after the injection of the contrast medium. The optimal combination of unenhanced, corticomedullary phase and nephrographic phase imaging depends on the context; suggestions of protocols are provided for eight different clinical situations. CONCLUSION: This expert-based consensus conference provides recommendations to standardise the imaging protocol for computed tomography urography. KEY POINTS: • To improve excretory phase imaging, an intravenous injection of furosemide should be performed before the injection of iodinated contrast medium. • Systematic oral or intravenous hydration is not necessary to improve excretory phase imaging. • The choice between single-bolus and split-bolus protocols depends on the context, but split-bolus protocols should be favoured whenever possible to decrease patient irradiation.

Uptake of further investigations following universal urinary screening among elementary and junior high school students in Shiga Prefecture, Japan: A retrospective cohort study.

AIM: This study aimed to investigate the current progression status from screening phase to further investigation phase in the Japanese school urine mass screening (SUS) project. METHODS: This retrospective cohort study on the SUS project across the Shiga Prefecture during 2012 to 2017 analysed data from school life instruction sheets, which are principal documents in the SUS project, regarding urinalysis, attendance at follow-up and diagnoses. RESULTS: Between the years 2012 to 2017, a median of 107 out of 83 749 elementary school students (aged 6-11 years) and 215 out of 42 870 junior high students (aged 12-14 years) had urine abnormalities identified for the first time in the SUS project. Among those with urine abnormalities, a mean of 4.2% of elementary school and 1.8% of junior high school students, respectively, were diagnosed with suspected glomerulonephritis for the first time. Overall, 5.9% (95% confidence interval [CI] 4.1, 7.7) and 23.6% (95% CI 21.3, 25.9) of proteinuria-positive elementary and junior high school students, respectively, did not undergo further investigations. The probability of a student undergoing further investigations was not affected by the local availability of medical care benefits. CONCLUSION: In the current SUS project, screening frequently does not lead to further investigation, especially among junior high school students. To maintain the integrity of the SUS project and to prevent the progression of renal disease in young students, efforts including elucidation of barriers to further investigations should be made to reduce the proportions of students not undergoing further investigations for abnormal urinalysis findings.

[Super-selective embolisation of bladder arteries for treatment of patients with bladder tumors complicated by recurrent macrohematuria].

The prevalence of bladder cancer in Russia over the past 10 years has increased from 49.6 to 74.1 patients per 100,000 population. Hematuria is a life-threatening complication and one of the common causes of mortality in advanced stages of bladder cancer. Conservative methods of hemostasis do not always allow to achieve a stable effect. In addition, in the cases of intractable bleeding, the patients may require surgical treatment. Due to the prevalence of patients with a high anesthetic risk group in bladder cancer patients cohort, the minimally invasive hemostasis technologies are more preferable. This review is devoted to one of such methods - superselective embolization of the urinary bladder arteries.

Development and validation of a haematuria cancer risk score to identify patients at risk of harbouring cancer.

BACKGROUND: A lack of consensus exists amongst national guidelines regarding who should be investigated for haematuria. Type of haematuria and age-specific thresholds are frequently used to guide referral for the investigation of haematuria. OBJECTIVES: To develop and externally validate the haematuria cancer risk score (HCRS) to improve patient selection for the investigation of haematuria. METHODS: Development cohort comprise of 3539 prospectively recruited patients recruited at 40 UK hospitals (DETECT 1; ClinicalTrials.gov: NCT02676180) and validation cohort comprise of 656 Swiss patients. All patients were aged >18 years and referred to hospital for the evaluation of visible and nonvisible haematuria. Sensitivity and specificity of the HCRS in the validation cohort were derived from a cut-off identified from the discovery cohort. RESULTS: Patient age, gender, type of haematuria and smoking history were used to develop the HCRS. HCRS validation achieves good discrimination (AUC 0.835; 95% CI: 0.789-0.880) and calibration (calibration slope = 1.215) with no significant overfitting (P = 0.151). The HCRS detected 11.4% (n = 8) more cancers which would be missed by UK National Institute for Health and Clinical Excellence guidelines. The American Urological Association guidelines would identify all cancers with a specificity of 12.6% compared to 30.5% achieved by the HCRS. All patients with upper tract cancers would have been identified. CONCLUSION: The HCRS offers good discriminatory accuracy which is superior to existing guidelines. The simplicity of the model would facilitate adoption and improve patient and physician decision-making.

Screening urinalysis demonstrates that haematuria is a frequent finding in persons with haemophilia treated at a paediatric haemophilia treatment centre.

INTRODUCTION: Haematuria is a recognized complication of haemophilia A and B (HA, HB). Adult persons with haemophilia (PWH) have a higher prevalence of renal disease than the general population. There is limited literature investigating the prevalence of haematuria in paediatric PWH. AIM: Our paediatric haemophilia treatment centre (HTC) had previously used quality improvement methods to increase the frequency of screening urinalyses at annual comprehensive visits. We retrospectively reviewed the data collected to determine the prevalence of haematuria and explore for associations in those with haematuria. METHODS: Retrospective chart review to identify the frequency of haematuria on screening urinalysis in all male PWH ≥2 years old. Haematuria was defined as ≥3 red blood cells (RBCs) per high power field. Univariate logistic regression was performed to evaluate for associations with haematuria. RESULTS: A total of 93 patients met eligibility criteria. Sixty-seven with HA (11 mild, 17 moderate, 39 severe) and 26 with HB (three mild, 16 moderate, seven severe). Forty-two of ninety-three (45%) patients were identified as having haematuria (median RBCs 7, mean RBCs 332). Of those with haematuria, 76% were identified by screening UA, as opposed to clinical symptoms, and 52% had recurrent haematuria. Older age and HA were associated with an increased likelihood of haematuria. CONCLUSIONS: Our study demonstrated that the prevalence of haematuria was high in PWH treated at our paediatric HTC. Future investigation is needed to determine the population-wide prevalence of haematuria in paediatric PWH and its impact on renal function.

Does urinary cytology have a role in haematuria investigations?

OBJECTIVES: To determine the diagnostic accuracy of urinary cytology to diagnose bladder cancer and upper tract urothelial cancer (UTUC) as well as the outcome of patients with a positive urine cytology and normal haematuria investigations in patients in a multicentre prospective observational study of patients investigated for haematuria. PATIENT AND METHODS: The DETECT I study (clinicaltrials.gov NCT02676180) recruited patients presenting with haematuria following referral to secondary case at 40 hospitals. All patients had a cystoscopy and upper tract imaging (renal bladder ultrasound [RBUS] and/ or CT urogram [CTU]). Patients, where urine cytology were performed, were sub-analysed. The reference standard for the diagnosis of bladder cancer and UTUC was histological confirmation of cancer. A positive urine cytology was defined as a urine cytology suspicious for neoplastic cells or atypical cells. RESULTS: Of the 3 556 patients recruited, urine cytology was performed in 567 (15.9%) patients from nine hospitals. Median time between positive urine cytology and endoscopic tumour resection was 27 (IQR: 21.3-33.8) days. Bladder cancer was diagnosed in 39 (6.9%) patients and UTUC in 8 (1.4%) patients. The accuracy of urinary cytology for the diagnosis of bladder cancer and UTUC was: sensitivity 43.5%, specificity 95.7%, positive predictive value (PPV) 47.6% and negative predictive value (NPV) 94.9%. A total of 21 bladder cancers and 5 UTUC were missed. Bladder cancers missed according to grade and stage were as follows: 4 (19%) were ≥ pT2, 2 (9.5%) were G3 pT1, 10 (47.6%) were G3/2 pTa and 5 (23.8%) were G1 pTa. High-risk cancer was confirmed in 8 (38%) patients. There was a marginal improvement in sensitivity (57.7%) for high-risk cancers. When urine cytology was combined with imaging, the diagnostic performance improved with CTU (sensitivity 90.2%, specificity 94.9%) superior to RBUS (sensitivity 66.7%, specificity 96.7%). False positive cytology results were confirmed in 22 patients, of which 12 (54.5%) had further invasive tests and 5 (22.7%) had a repeat cytology. No cancer was identified in these patients during follow-up. CONCLUSIONS: Urine cytology will miss a significant number of muscle-invasive bladder cancer and high-risk disease. Our results suggest that urine cytology should not be routinely performed as part of haematuria investigations. The role of urine cytology in select cases should be considered in the context of the impact of a false positive result leading to further potentially invasive tests conducted under general anaesthesia.