Primary Sclerosing Cholangitis in Children With Inflammatory Bowel Diseases Is Associated With Milder Clinical Activity But More Frequent Subclinical Inflammation and Growth Impairment.

BACKGROUND & AIMS: Although inflammatory bowel diseases (IBD) associated with primary sclerosing cholangitis (PSC) have been well characterized in adults, there have been few pediatric studies, and these were small and produced conflicting results. We investigated features of PSC-IBD in children, compared with children with IBD without PSC. METHODS: We performed a retrospective study of 74 children with PSC-IBD, diagnosed from 2000 through 2018, who were each matched with 2 children with ulcerative colitis or IBD-unclassified (controls) based on sex, date of birth, and type of IBD. We compared IBD distribution and clinical activity (remission, medication use, hospitalization, or colectomy) and patient growth between groups. Data were extracted from each hospital contact and analyzed using mixed effects analyses or Cox proportional hazards regression, adjusting for time-dependent medication exposure. RESULTS: Higher proportions of children with PSC-IBD had backwash ileitis, pancolitis, and rectal sparing, and more severe right-sided disease, than controls (P < .05). Patients with PSC-IBD were more likely to be treated with only 5-ASA, compared with controls (odds ratio [OR], 3.04; 95% CI, 1.44-6.41) and to have IBD in clinical remission (OR, 2.94; 95% CI, 1.78-4.87). Risk of colectomy or treatment with a biologic agent was lower in patients with PSC-IBD than controls (hazard ratio, 0.24; 95% CI, 0.12-0.52). However, determination of IBD severity based on symptoms underestimated severity based on endoscopic activity in patients with PSC-IBD. Among patients with IBD in clinical remission, those with PSC were less likely to have endoscopic remission (OR, 0.44; 95% CI, 0.20-0.96). Patients with PSC-IBD were shorter and had lower weight over time, compared with controls. CONCLUSIONS: In a retrospective study, we found that features of IBD differed between children with vs without PSC, similar to adults. Despite the mild clinical activity of IBD in patients with PSC, lack of symptoms does not always indicate lack of mucosal inflammation. Children with PSC-IBD have greater growth impairments compared with children with ulcerative colitis or IBD-unclassified.

Outcome and safety of targeted liver biopsies for indeterminate lesions in patients with chronic liver disease: A single centre experience.

INTRODUCTION: To evaluate the histopathological and safety outcomes of indeterminate lesions in patients at high risk for developing hepatocellular carcinoma (HCC) who underwent ultrasound-guided biopsies. METHODS: Ultrasound-guided targeted liver biopsies for indeterminate lesions performed in a 10-year period at our institution were reviewed retrospectively for lesion characteristics, biopsy techniques, histopathological results and post procedural complications. RESULTS: A total of 172 biopsies were performed in 152 patients. Most common background liver disease included hepatitis C, hepatitis B, alcoholic and non-alcoholic steatohepatitis. 65.1% had known cirrhosis at time of biopsy. HCC was the most common histopathological finding accounting for 55.8% of all biopsies, followed by cholangiocarcinoma, dysplastic nodule and metastasis. Rarer lesions including lymphoma, neuroendocrine tumour and angiomyolipoma were also encountered. No mortality, clinically significant bleeding or tumour seeding was detected. CONCLUSIONS: Ultrasound-guided liver biopsies of indeterminate lesions in patients at high risk of HCC yield important histopathological findings, important for management options including the provision of curative treatments and assisting future novel therapies such as immunotherapy and targeted therapies. The low complication rates confirm its safety and the procedure should not be avoided for fear of complications.

Comparative sensitivity and specificity of imaging modalities in staging bladder cancer prior to radical cystectomy: a systematic review and meta-analysis.

PURPOSE: The detection of lymph node metastases in bladder cancer has a significant impact on treatment decisions. Multiple imaging modalities are available to clinicians including magnetic resonance imaging, computed tomography and positron emission tomography. We aimed to investigate the utility of alternate imaging modalities on pre-cystectomy imaging in bladder cancer for the detection of lymph node metastases. METHODS: We performed systematic search of Web of Science (including MEDLINE), EMBASE and Cochrane libraries in accordance with the PRISMA statement. Studies comparing lymph node imaging findings with final histopathology were included in our analysis. Sensitivity and specificity data were quantified using patient-based analysis. A true positive was defined as a node-positive patient on imaging and node positive on histopathology. Meta-analysis of studies was performed using a mixed-effects, hierarchical logistic regression model. RESULTS: Our systematic search identified 35 articles suitable for inclusion. MRI and PET have a higher sensitivity than CT while the specificity of all modalities was similar. The summary MRI sensitivity = 0.60 (95% CI 0.44-0.74) and specificity = 0.91 (95% CI 0.82-0.96). Summary PET/CT sensitivity = 0.56 (95% CI 0.49-0.63) and specificity = 0.92 (95% CI 0.86-0.95). Summary CT sensitivity = 0.40 (95% CI 0.33-0.49) and specificity = 0.92 (95% CI 0.86-0.95). CONCLUSION: MRI and PET/CT provides superior sensitivity compared to CT for detection of positive lymph nodes in bladder cancer prior to cystectomy. There is variability in the accuracy that current imaging modalities achieve across different studies. A number of other factors impact on detection accuracy and these must be considered.

Factors affecting the timeliness and adequacy of haematuria assessment in bladder cancer: a systematic review.

OBJECTIVES: To review the literature to identify factors affecting haematuria assessment in bladder cancer. METHODS: We performed a systematic review in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Publications indexed in EMBASE and Medline (PubMed) in March 2016 were searched, using the keywords 'hematuria', 'urinary bladder neoplasm(s)' and 'bladder tumor'. Studies evaluating the timeliness and adequacy of haematuria assessment in the context of bladder cancer were included. Exclusion criteria included age <18 years, animal studies and non-English articles. RESULTS: Following our search strategy, a total of 17 articles were included in our study. All 17 studies commented on gender, with female gender associated with delayed and inadequate haematuria evaluation. Women waited longer than men for urological review (three studies) and bladder cancer diagnosis (three studies). Women were also less likely to be referred to urology (two studies), receive imaging (three studies) or have cystoscopy (two studies). In all, 10 studies commented on age, with the impression that advancing age is associated with a more thorough assessment. Smokers and those with microscopic haematuria appear to undergo a less thorough evaluation. CONCLUSION: Female gender is associated with sub-optimal haematuria evaluation, while older patients are evaluated more thoroughly. Smokers paradoxically undergo less comprehensive assessment. Further research on the impact of other factors is required.

Predictors of delay to cystoscopy and adequacy of investigations in patients with haematuria.

OBJECTIVE: To identify factors that impact on the timeliness and adequacy of haematuria evaluation. PATIENTS AND METHODS: We undertook a retrospective cohort study identifying patients who underwent cystoscopy for investigation of haematuria at our institution between 1 January 2015 and 31 December 2015. Data on patient demographics, smoking status, anticoagulation, type of haematuria and referring clinician were collected. Exclusion criteria included patient age <18 years, known history of bladder/urinary tract/renal cancer, cystoscopy for indications other than haematuria and unknown date of urology consultation. Primary outcome measures were: i) time from general practitioner (GP) referral to urology consultation, ii) time from urology consultation to cystoscopy, and iii) receipt of investigations in the 180 days prior to cystoscopy. Comparisons between risk factors were carried out using negative binomial regression for count outcomes and chi-square test for categorical outcomes. RESULTS: Over the study period, 305 eligible cases (225 men, 80 women) were identified, of which 196 (64%) were referred by a GP. Patients waited a median of 38 days from GP referral to urology consultation and 28 days from urology consultation to cystoscopy. The median time to urology consultation was 65 days for women and 33.5 days for men (P = 0.020). However, the observed difference between men and women was no longer statistically significant on multivariable regression, with the only independent predictors of a shorter interval being visible haematuria and imaging suspicious for cancer. Anticoagulated patients were more likely to have imaging studies, in particular renal tract ultrasonography (P = 0.006), while only 61% of patients with visible haematuria received imaging. No significant differences in recent investigations between genders were observed. CONCLUSIONS: Gender is not a significant predictor of delayed haematuria assessment or receipt of recent investigations. Anticoagulated patients are more likely to receive imaging than patients without anticoagulation and patients with visible haematuria are not adequately imaged. Improved clinician and public education is required to ensure that all patients are evaluated appropriately.