ANCA-negative Churg-Strauss Syndrome

A rare and a disease of unknown etiology, Churg-Strauss syndrome (CSS) is a granulomatous necrotizing small vessel vasculitis characterized by the presence of asthma, sinusitis, and hypereosinophilia, which is initially described by Churg and Strauss in 1951. Because of its clinical and pathological features that overlap with those of the other anti-neutrophil antibody (ANCA)-associated systemic vasculitides (AASVs) and now the disease is classified as AASVs. The ANCA status may dictate the clinical phenotype. ANCA-positive patients are significantly more likely to have disease manifesta­tions associated with small-vessel vasculitis, including oecrotising glomemlonephritis, mononeuritis and purpura, whereas ANCA-negative cases predominantly likely to have cardiac and lung involvement. The objective of this case report is to point out the possibility of vasculitic rash in ANCA-negative CSS in a 35-year-old man and the disease rarely occurs in Bangladeshi population. We analyze the history, clinical examinations and relevant investigations related to the patient to establish the diagnosis in our department. The clinical scenario and biopsy help us to attain the diagnosis. But due to unavailability of patients' cohort we have limitations of comparison of ANCA status in Bangladeshi populations. Though ANCA-positive and ANCA-negative CSS differ phenotypically, primary therapy for both the conditions is systemic glucocorticoids. Additional immunosuppressive agents like cyclophosphamide, mycophenolate mofetil, azathioprine, rituxin1ab are occasionally added in patients with more advanced or refractory disease

The “Is mpMRI Enough” or IMRIE Study: A Multicentre Evaluation of Prebiopsy Multiparametric Magnetic Resonance Imaging Compared with Biopsy

Background: Multiparametric magnetic resonance imaging (mpMRI) is now recommended prebiopsy in numerous healthcare regions based on the findings of high-quality studies from expert centres. Concern remains about reproducibility of mpMRI to rule out clinically significant prostate cancer (csPCa) in real-world settings. / Objective: To assess the diagnostic performance of mpMRI for csPCa in a real-world setting. / Design, setting, and participants: A multicentre, retrospective cohort study, including men referred with raised prostate-specific antigen (PSA) or an abnormal digital rectal examination who had undergone mpMRI followed by transrectal or transperineal biopsy, was conducted. Patients could be biopsy naïve or have had previous negative biopsies. / Outcome measurements and statistical analysis: The primary definition for csPCa was International Society of Urological Pathology (ISUP) grade group (GG) ≥2 (any Gleason ≥7); the accuracy for other definitions was also evaluated. / Results and limitations: Across ten sites, 2642 men were included (January 2011–November 2018). Mean age and PSA were 65.3 yr (standard deviation [SD] 7.8 yr) and 7.5 ng/ml (SD 3.3 ng/ml), respectively. Of the patients, 35.9% had “negative MRI” (scores 1–2); 51.9% underwent transrectal biopsy and 48.1% had transperineal biopsy, with 43.4% diagnosed with csPCa overall. The sensitivity and negative predictive value (NPV) for ISUP GG ≥ 2 were 87.3% and 87.5%, respectively. The NPVs were 87.4% and 88.1% for men undergoing transrectal and transperineal biopsy, respectively. Specificity and positive predictive value of MRI were 49.8% and 49.2%, respectively. The sensitivity and NPV increased to 96.6% and 90.6%, respectively, when a PSA density threshold of 0.15 ng/ml/ml was used in MRI scores 1–2; these metrics increased to 97.5% and 91.2%, respectively, for PSA density 0.12 ng/ml/ml. ISUP GG ≥ 3 (Gleason ≥4 + 3) was found in 2.4% (15/617) of men with MRI scores 1–2. They key limitations of this study are the heterogeneity and retrospective nature of the data. / Conclusions: Multiparametric MRI when used in real-world settings is able to rule out csPCa accurately, suggesting that about one-third of men might avoid an immediate biopsy. Men should be counselled about the risk of missing some significant cancers. / Patient summary: Multiparametric magnetic resonance imaging (MRI) is a useful tool for ruling out prostate cancer, especially when combined with prostate-specific antigen density (PSAD). Previous results published from specialist centres can be reproduced at smaller institutions. However, patients and their clinicians must be aware that an early diagnosis of clinically significant prostate cancer could be missed in nearly 10% of patients by relying on MRI and PSAD alone

Finishing the euchromatic sequence of the human genome

The sequence of the human genome encodes the genetic instructions for human physiology, as well as rich information about human evolution. In 2001, the International Human Genome Sequencing Consortium reported a draft sequence of the euchromatic portion of the human genome. Since then, the international collaboration has worked to convert this draft into a genome sequence with high accuracy and nearly complete coverage. Here, we report the result of this finishing process. The current genome sequence (Build 35) contains 2.85 billion nucleotides interrupted by only 341 gaps. It covers ∼99% of the euchromatic genome and is accurate to an error rate of ∼1 event per 100,000 bases. Many of the remaining euchromatic gaps are associated with segmental duplications and will require focused work with new methods. The near-complete sequence, the first for a vertebrate, greatly improves the precision of biological analyses of the human genome including studies of gene number, birth and death. Notably, the human enome seems to encode only 20,000-25,000 protein-coding genes. The genome sequence reported here should serve as a firm foundation for biomedical research in the decades ahead

Quotations illustrating “Acceptance”.

Background‘Expressed Emotion (EE)’ captures ways in which emotions are expressed within a family environment. Research has found that EE in families has an impact on psychiatric illness, in particular psychosis, such that it increases risk of relapse. EE was conceptualised by research conducted in the UK. Thus, behaviours defined as pathological were largely based on white samples adhering to UK norms. Cross-cultural variations have been found in EE and its relationship with clinical outcomes. A more culturally appropriate understanding of norms surrounding the EE across cultures is required.AimsThis study aims to use a bottom-up approach to provide a culturally specific understanding of family relationships and EE across ‘non-clinical’ UK-based South Asian families.MethodsSemi-structured interviews were conducted with 18 South Asian participants to explore their relationships with a significant other. Interviews were analysed using thematic analysis.ResultsFour main themes were generated: expression of love, setting boundaries, inter-generational differences and acceptance.ConclusionThe findings indicate considerable cultural variability within EE and highlight the need to interpret EE in the context of socio-cultural norms. Whilst certain domains of EE that are considered pathological in Western contexts are present in the UK-based South Asian diaspora, these are perceived as less problematic, indicative of varying cultural norms.</div

Thematic map.

Main themes: Blue; Intermediary themes: Orange; Subthemes: Yellow.</p

Quotations illustrating “Inter-generational differences”.

Quotations illustrating “Inter-generational differences”.</p

Quotations illustrating “Expression of love”.

Background‘Expressed Emotion (EE)’ captures ways in which emotions are expressed within a family environment. Research has found that EE in families has an impact on psychiatric illness, in particular psychosis, such that it increases risk of relapse. EE was conceptualised by research conducted in the UK. Thus, behaviours defined as pathological were largely based on white samples adhering to UK norms. Cross-cultural variations have been found in EE and its relationship with clinical outcomes. A more culturally appropriate understanding of norms surrounding the EE across cultures is required.AimsThis study aims to use a bottom-up approach to provide a culturally specific understanding of family relationships and EE across ‘non-clinical’ UK-based South Asian families.MethodsSemi-structured interviews were conducted with 18 South Asian participants to explore their relationships with a significant other. Interviews were analysed using thematic analysis.ResultsFour main themes were generated: expression of love, setting boundaries, inter-generational differences and acceptance.ConclusionThe findings indicate considerable cultural variability within EE and highlight the need to interpret EE in the context of socio-cultural norms. Whilst certain domains of EE that are considered pathological in Western contexts are present in the UK-based South Asian diaspora, these are perceived as less problematic, indicative of varying cultural norms.</div

Quotations illustrating “Setting boundaries”.

Background‘Expressed Emotion (EE)’ captures ways in which emotions are expressed within a family environment. Research has found that EE in families has an impact on psychiatric illness, in particular psychosis, such that it increases risk of relapse. EE was conceptualised by research conducted in the UK. Thus, behaviours defined as pathological were largely based on white samples adhering to UK norms. Cross-cultural variations have been found in EE and its relationship with clinical outcomes. A more culturally appropriate understanding of norms surrounding the EE across cultures is required.AimsThis study aims to use a bottom-up approach to provide a culturally specific understanding of family relationships and EE across ‘non-clinical’ UK-based South Asian families.MethodsSemi-structured interviews were conducted with 18 South Asian participants to explore their relationships with a significant other. Interviews were analysed using thematic analysis.ResultsFour main themes were generated: expression of love, setting boundaries, inter-generational differences and acceptance.ConclusionThe findings indicate considerable cultural variability within EE and highlight the need to interpret EE in the context of socio-cultural norms. Whilst certain domains of EE that are considered pathological in Western contexts are present in the UK-based South Asian diaspora, these are perceived as less problematic, indicative of varying cultural norms.</div

Table of themes before and after iteration process.

Table of themes before and after iteration process.</p