Publications
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Kousios A, Blakey S, Moran L, et al., 2023, Non-crystalline light chain proximal tubulopathy, a morphologically protean entity., Nephrology Dialysis Transplantation, ISSN: 0931-0509
BACKGROUND: Light chain proximal tubulopathy (LCPT) is a rare form of paraprotein-related disease, occurring in two main histopathological forms; crystalline and non-crystalline. The clinicopathological features, treatment strategies and outcomes, especially of the non-crystalline form, are not well described. METHODS: Single-centre retrospective case series of 12 LCPT patients, 5 crystalline and 7 non-crystalline, between 2005-2021. OBSERVATIONS: Median age was 69.5 years (range, 47-80). 10 patients presented with CKD and significant proteinuria (median eGFR of 43.5 ml/min/1.73m2; uPCR 328 mg/mmol). Only 6 patients had known haematological disease at the time of renal biopsy. Multiple myeloma (MM) was diagnosed in 7 cases and MGRS in 5. A clone was detected in all cases combining serum/urine electrophoresis and free LC assays. Crystalline and non-crystalline variants had similar clinical presentations. For the non-crystalline variant, a diagnosis was reached based on combination of CKD without other cause, haematological work-up, LC restriction on IF and abnormalities on EM. Nine of 12 patients received clone-directed treatment. Patients who achieved haematological response (including all non-crystalline LCPT) had improved renal outcomes over a median follow-up of 79 months. CONCLUSIONS: The non-crystalline variant may go unrecognised because of its subtle histopathological features and requires EM to distinguish it from "excessive LC resorption without tubular injury". Clone-directed treatment with good haematological response improves renal outcomes in both variants but limited data exist in MGRS. Multi-centre prospective studies are needed to better define the clinico-pathological characteristics associated with poor outcomes and optimize treatment strategies in patients with MGRS.
Gulati K, Edwards H, Prendecki M, et al., 2021, Combination treatment with rituximab, low-dose cyclophosphamide and plasma exchange for severe antineutrophil cytoplasmic antibody-associated vasculitis, Kidney International, Vol: 100, Pages: 1316-1324, ISSN: 0085-2538
Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis can present with life-threatening lung-kidney syndromes. However, many controlled treatment trials excluded patients with diffuse alveolar hemorrhage or severely impaired glomerular filtration rates, and so the optimum treatment in these cases is unclear. In this retrospective cohort study, we report the outcomes of 64 patients with life-threatening disease treated with a combination regimen of rituximab, low-dose intravenous cyclophosphamide, oral glucocorticoids, and plasma exchange. At entry, the median estimated glomerular filtration rate was 9 mL/min, 47% of patients required dialysis, and 52% had diffuse alveolar hemorrhage. All patients received a minimum of seven plasma exchanges, and the median cumulative doses of rituximab, cyclophosphamide, and glucocorticoid were 2, 3, and 2.6 g, respectively, at six months. A total of 94% of patients had achieved disease remission (version 3 Birmingham Vasculitis Activity Score of 0) at this time point, and 67% of patients who required dialysis recovered independent kidney function. During long-term follow-up (median duration 46 months), overall patient survival was 85%, and 69% of patients remained free from end-stage kidney disease, which compares favorably to a historic cohort with severe disease treated with a conventional induction regimen. Combination treatment was associated with prolonged B cell depletion and low rates of relapse; 87% of patients were in continuous remission at month 36. The serious infection rate during total follow-up was 0.28 infections/patient/year, suggesting that combination treatment is not associated with an enduring risk of infection. Thus, we suggest that combination immunosuppressive therapy may permit glucocorticoid avoidance and provide rapid and prolonged disease control in patients with severe ANCA-associated vasculitis.
Boharoon H, Tomlinson J, Limback-Stanic C, et al., 2021, A case series of patients with isolated IgG4-related hypophysitis treated with rituximab, Journal of the Endocrine Society, Vol: 4, Pages: 1-9, ISSN: 2472-1972
ContextThe acute presentation of Immunoglobulin G4 (IgG4)-related hypophysitis can be indistinguishable from other forms of acute hypophysitis and histology remains the diagnostic gold standard. The high recurrence rate necessitates long term immunosuppressive therapy. Rituximab (RTX) has been shown to be effective in systemic IgG4-related disease (IgG4-RD), but experience with isolated pituitary involvement remains limited.Case descriptionWe report three female patients with MRI findings suggestive of hypophysitis. All patients underwent transsphenoidal biopsy and fulfilled diagnostic criteria for IgG4-related hypophysitis. Treatment with GCs (GC) resulted in good therapeutic response in patients 1 and 2, but the disease recurred on tapering doses of GCs. GC treatment led to emotional lability in Patient 3 necessitating dose reduction. All three patients received RTX and Patients 2 and 3 received further courses when symptoms returned and B-cells repopulated. Patient 3 did not receive RTX until 12 months from onset of symptoms. Patient 1 was not able to have further RTX treatments due to an allergic reaction when receiving the second dose. RTX treatment resulted in sustained remission and full recovery of anterior pituitary function in Patients 1 and 2 with complete resolution of pituitary enlargement. By contrast, Patient 3 only showed symptomatic response following RTX treatment, but pituitary enlargement and hypofunction persisted.ConclusionRTX treatment for IgG4-related hypophysitis resulted in sustained remission in two patients treated early in the disease process, but only achieved partial response in a patient with chronic disease suggesting that early therapeutic intervention may be crucial to avoid irreversible changes.
Thind A, Beckwith H, Dattani R, et al., 2021, Resuming deceased donor kidney transplantation in the COVID-19 era: what do patients want?, Transplantation Direct, Vol: 7, Pages: 1-6, ISSN: 2373-8731
Background: The rapidly evolving novel coronavirus 2019 (COVID-19) pandemic bought many kidney transplant (KT) programmes to a halt. Integral to resuming KT activity is understanding the perspectives of potential transplant candidates during this highly dynamic time. Methods: From June 1st to July 7th 2020, a telephone survey of KT candidates on the deceased donor waiting list at Imperial College Renal and Transplant Centre in West London was conducted. The survey captured ongoing COVID-19 exposure risks and patients’ views on wait list (WL) reactivation and undergoing transplantation. Results: 207 responses were received. Of the respondents 180 patients (87%) were happy to be reactivated onto the WL; with 141 patients (68%) willing to give consent to transplantation currently, whilst 53 patients (26%) felt unsure, and 13 patients (6%) would decline a KT. The vast majority of patients had no concerns. In the responses from those who were uncertain or who would decline a KT, concerns about COVID-19 infection and the need for reassurance from transplant units dominated. Universally patients wanted more information about COVID-19 infection risk with KT and the precautions being taken to reduce this risk. Conclusions: The majority of surveyed patients are in favour of reactivation and receiving a KT despite the ongoing COVID-19 pandemic. Reactivation of candidates cannot be assumed and should take an individualised approach, incorporating clinical risk with patient perspectives. Improved communication with KT candidates is highly requested.
Nikolopoulou A, Teixeira C, Cook H, et al., 2021, Membranous nephropathy associated with viral infection, Clinical Kidney Journal, Vol: 14, Pages: 876-883, ISSN: 2048-8505
BackgroundMembranous nephropathy (MN) can be associated with hepatitis infection and less commonly with human immunodeficiency virus (HIV) infection. The significance of anti-phospholipase A2 receptor (PLA2R) and anti-thrombospondin type 1 domain-containing 7A (THSD7A) antibodies in this setting is unclear.MethodsWe describe the clinical, histopathological and outcome data of 19 patients with MN and hepatitis B virus (HBV), hepatitis C virus (HCV) or HIV infection identified through our renal biopsy database and the association with anti-PLA2R antibodies and anti-THSD7A antibodies.ResultsThe cohort consisted of 19 patients, 8 male and 11 female, with a median age of 42 years (range 23–74). HBV infection was found in six cases, HCV in four and HIV in nine (two HIV patients had HBV co-infection and one HCV co-infection). PLA2R staining on biopsy was positive in 10/19 patients: 4 with HBV-MN, 3 with HCV-MN and 3 with HIV-MN and circulating anti-PLA2R antibodies were detected in 7/10 cases. THSD7A staining on biopsy was positive in three PLA2R-negative cases, one with HBV-MN and two with HIV-MN. Mean proteinuria was higher in the PLA2R-positive group and the median urinary protein:creatinine ratio (uPCR) was 963 mg/mmol (range 22–2406) compared with the PLA2R-negative group [median uPCR 548 mg/mmol (range 65–1898); P = 0.18 Mann–Whitney]. Spontaneous remission occurred in 6/19 patients and after-treatment remission occurred in 7/11 patients. Renal function was preserved in all but two patients who required haemodialysis 2 and 11 years from diagnosis.ConclusionsWe describe a cohort of patients with MN associated with viral infection, including rare cases of HIV-MN with PLA2R and THSD7A positivity. The mechanism of coincidental or viral-related MN needs to be investigated further.
Povey J, Rutherford E, Levy J, et al., 2021, Relapse of treated anti-GBM disease following hair dye use, BMJ CASE REPORTS, Vol: 14
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Levy JB, Kong E, Johnson N, et al., 2021, The mixed reality medical ward round with the MS HoloLens 2: Innovation in reducing COVID-19 transmission and PPE usage., Future Healthc J, Vol: 8, Pages: e127-e130, ISSN: 2514-6645
BACKGROUND: The COVID-19 pandemic necessitated changes to the traditional medical ward round to protect staff and patients. This study investigated the value and acceptability of using the Microsoft HoloLens 2 mixed reality headset in a COVID-19 renal medicine ward. METHODS: The HoloLens 2 was used during the height of the COVID-19 pandemic and it was compared with the days prior to its introduction. Staff exposure to COVID-19 and PPE usage were measured, and staff and patients were surveyed on the HoloLens 2 experience. RESULTS: The average ward round was significantly shorter with the use of the HoloLens 2 (94 minutes vs 137 minutes; p=0.006). With the HoloLens 2, only the consultant was in direct contact with COVID-19 patients compared with up to seven staff members on a normal ward round. Personal protective equipment usage was reduced by over 50%. Both staff and patients were positive about its use but raised some important concerns. CONCLUSION: The HoloLens 2 mixed reality technology is an innovative solution to the challenges posed by COVID-19 to the traditional medical ward round.
Willicombe M, Gleeson S, Clarke C, et al., 2021, Identification of Patient Characteristics Associated With SARS-CoV-2 Infection and Outcome in Kidney Transplant Patients Using Serological Screening, TRANSPLANTATION, Vol: 105, Pages: 151-157, ISSN: 0041-1337
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Clarke C, Lucisano G, Prendecki M, et al., 2021, Informing the risk of kidney transplantation versus remaining on the wait list in the COVID-19 era, Kidney International Reports, Vol: 6, Pages: 46-55, ISSN: 2468-0249
Introduction: There is limited data pertaining to comparative outcomes of remaining on dialysis versus kidney transplantation as the threat of COVID-19 remains. This study aims to delineate the differential risks involved using serological methods to help define exposure rates. Methods: From a cohort of 1433 patients with ESKD, we analysed COVID-19 infection rates and outcomes in 299 wait list patients compared with 237 transplant recipients within their first year post-transplant. Patients were followed over a 68-day period from the time our transplant programme closed due to COVID-19. Results: The overall mortality rate in wait list and transplant populations were equivalent, p=0.69. However, COVID-19 infection was more commonly diagnosed in the wait list patients, p=0.001, who were more likely to be tested by RT-PCR, p=0.0004. Once infection was confirmed, mortality risk was higher in the transplant patients, p=0.015. The seroprevalence in dialysis and transplant patients with undetected infection was 18.3% and 4.6% respectively, p=0.0001. After adjusting for a potential screening bias, the relative risk of death following a diagnosis of COVID-19 remained higher in transplant recipients, HR: 3.36 (1.19-9.50), p=0.022. Conclusions: In conclusion, whilst COVID-19 infection was more common in the wait list patients, a higher COVID-19 associated mortality rate was seen in transplant recipients, resulting in comparable overall mortality rates.
Corbett RW, Blakey S, Nitsch D, et al., 2020, Epidemiology of COVID-19 in an urban dialysis center, Journal of the American Society of Nephrology, Vol: 31, Pages: 1815-1823, ISSN: 1046-6673
Background During the coronavirus disease 2019 (COVID-19) epidemic, many countries have instituted population-wide measures for social distancing. The requirement of patients on dialysis for regular treatment in settings typically not conducive to social distancing may increase their vulnerability to COVID-19.Methods Over a 6-week period, we recorded new COVID-19 infections and outcomes for all adult patients receiving dialysis in a large dialysis center. Rapidly introduced control measures included a two-stage routine screening process at dialysis entry (temperature and symptom check, with possible cases segregated within the unit and tested for SARS-CoV-2), isolated dialysis in a separate unit for patients with infection, and universal precautions that included masks for dialysis nursing staff.Results Of 1530 patients (median age 66 years; 58.2% men) receiving dialysis, 300 (19.6%) developed COVID-19 infection, creating a large demand for isolated outpatient dialysis and inpatient beds. An analysis that included 1219 patients attending satellite dialysis clinics found that older age was a risk factor for infection. COVID-19 infection was substantially more likely to occur among patients on in-center dialysis compared with those dialyzing at home. We observed clustering in specific units and on specific shifts, with possible implications for aspects of service design, and high rates of nursing staff illness. A predictive epidemic model estimated a reproduction number of 2.2; cumulative cases deviated favorably from the model from the fourth week, suggesting that the implemented measures controlled transmission.Conclusions The COVID-19 epidemic affected a large proportion of patients at this dialysis center, creating service pressures exacerbated by nursing staff illness. Details of the control strategy and characteristics of this epidemic may be useful for dialysis providers and other institutions providing patient care.
Medjeral-Thomas NR, Lawrence C, Condon M, et al., 2020, Randomized, controlled trial of tacrolimus and prednisolone monotherapy for adults with de novo minimal change disease: a multicenter, randomized, controlled trial (vol 15, pg 209, 2020), Clinical Journal of the American Society of Nephrology, Vol: 15, Pages: 1027-1027, ISSN: 1555-9041
Tan PG, O'Brien J, Bedi R, et al., 2020, TREATMENT EFFICACY OF BIOSIMILAR RITUXIMAB (TRUXIMA) COMPARED TO THE ORIGINATOR (MABTHERA) IN PATIENTS WITH ANCA ASSOCIATED VASCULITIS, 57th ERA-EDTA Congress, Publisher: OXFORD UNIV PRESS, Pages: 680-680, ISSN: 0931-0509
Tan PG, O'Brien J, Griffith M, et al., 2020, VALIDATION OF THE ANCA RENAL RISK SCORE IN A LONDON COHORT: POTENTIAL IMPACT OF TREATMENT ON PREDICTION OUTCOME, 57th ERA-EDTA Congress, Publisher: OXFORD UNIV PRESS, Pages: 662-662, ISSN: 0931-0509
Tan PG, O'Brien J, Griffith M, et al., 2020, THE SAFETY PROFILE OF REPEAT RITUXIMAB TREATMENT IN ANCA-ASSOCIATED VASCULITIS - A 10 YEAR SINGLE CENTRE STUDY, 57th ERA-EDTA Congress, Publisher: OXFORD UNIV PRESS, Pages: 104-104, ISSN: 0931-0509
Medjeral-Thomas NR, Lawrence C, Condon M, et al., 2020, Randomized, controlled trial of tacrolimus and prednisolone monotherapy for adults with De Novo minimal change disease: a multicenter, randomized, controlled trial, Clinical Journal of the American Society of Nephrology, Vol: 15, Pages: 209-218, ISSN: 1555-9041
BACKGROUND AND OBJECTIVES: Minimal change disease is an important cause of nephrotic syndrome in adults. Corticosteroids are first-line therapy for minimal change disease, but a prolonged course of treatment is often required and relapse rates are high. Patients with minimal change disease are therefore often exposed to high cumulative corticosteroid doses and are at risk of associated adverse effects. This study investigated whether tacrolimus monotherapy without corticosteroids would be effective for the treatment of de novo minimal change disease. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: This was a multicenter, prospective, open-label, randomized, controlled trial involving six nephrology units across the United Kingdom. Adult patients with first presentation of minimal change disease and nephrotic syndrome were randomized to treatment with either oral tacrolimus at 0.05 mg/kg twice daily, or prednisolone at 1 mg/kg daily up to 60 mg daily. The primary outcome was complete remission of nephrotic syndrome after 8 weeks of therapy. Secondary outcomes included remission of nephrotic syndrome at 16 and 26 weeks, rates of relapse of nephrotic syndrome, and changes from baseline kidney function. RESULTS: There were no significant differences between the tacrolimus and prednisolone treatment cohorts in the proportion of patients in complete remission at 8 weeks (21 out of 25 [84%] for prednisolone and 17 out of 25 [68%] for tacrolimus cohorts; P=0.32; difference in remission rates was 16%; 95% confidence interval [95% CI], -11% to 40%), 16 weeks (23 out of 25 [92%] for prednisolone and 19 out of 25 [76%] for tacrolimus cohorts; P=0.25; difference in remission rates was 16%; 95% CI, -8% to 38%), or 26 weeks (23 out of 25 [92%] for prednisolone and 22 out of 25 [88%] for tacrolimus cohorts; P=0.99; difference in remission rates was 4%; 95% CI, -17% to 25%). There was no significant difference in relapse rates (17 out of 23 [74%] for prednisolone and 16 out of 22
Ashby D, Borman N, Burton J, et al., 2019, Renal association clinical practice guideline on haemodialysis, BMC Nephrology, Vol: 20, Pages: 1-36, ISSN: 1471-2369
This guideline is written primarily for doctors and nurses working in dialysis units and related areas of medicine in the UK, and is an update of a previous version written in 2009. It aims to provide guidance on how to look after patients and how to run dialysis units, and provides standards which units should in general aim to achieve. We would not advise patients to interpret the guideline as a rulebook, but perhaps to answer the question: “what does good quality haemodialysis look like?”The guideline is split into sections: each begins with a few statements which are graded by strength (1 is a firm recommendation, 2 is more like a sensible suggestion), and the type of research available to back up the statement, ranging from A (good quality trials so we are pretty sure this is right) to D (more like the opinion of experts than known for sure). After the statements there is a short summary explaining why we think this, often including a discussion of some of the most helpful research. There is then a list of the most important medical articles so that you can read further if you want to – most of this is freely available online, at least in summary form.A few notes on the individual sections:1.This section is about how much dialysis a patient should have. The effectiveness of dialysis varies between patients because of differences in body size and age etc., so different people need different amounts, and this section gives guidance on what defines “enough” dialysis and how to make sure each person is getting that. Quite a bit of this section is very technical, for example, the term “eKt/V” is often used: this is a calculation based on blood tests before and after dialysis, which measures the effectiveness of a single dialysis session in a particular patient.2.This section deals with “non-standard” dialysis, which basically means anything other than 3 times per week. For example, a few people need 4 or more ses
Poo SX, Tham CSW, Smith C, et al., 2019, IgG4-related disease in a multi-ethnic community: Clinical characteristics and association with malignancy, QJM: An International Journal of Medicine, Vol: 112, Pages: 763-769, ISSN: 1460-2393
BackgroundImmunoglobulin-G4-related disease (IgG4-RD) is a recently recognised fibro-inflammatory condition that can affect multiple organs. Despite growing interest in this condition, the natural history and management of IgG4-RD remain poorly understood.AimTo describe the clinical characteristics, treatment and outcomes of IgG4-RD in a multi-ethnic UK cohort, and investigate its possible association with malignancy.DesignRetrospective analysis of case-note and electronic data.MethodsCases were identified from sub-specialty cohorts and a systematic search of an NHS trust histopathology database using ‘IgG4’ or ‘inflammatory pseudotumour’ as search terms. Electronic records, imaging and histopathology reports were reviewed.Results66 identified cases of IgG4-RD showed a similar multi-ethnic spread to the local population of North West London. The median age was 59 years and 71% of patients were male. Presenting symptoms relating to mass effect of a lesion were present in 48% of cases and the mean number of organs involved was 2.4. 10 patients had reported malignancies with 6 of these being haematological. 83% of those treated with steroids had good initial response, however 50% had relapsing-remitting disease. Rituximab was administered in 11 cases and all achieved an initial serological response. Despite this, 7 patients subsequently relapsed after a mean duration of 11 months and 4 progressed despite treatment.ConclusionsWe report a large UK-based cohort of IgG4-RD that shows no clear ethnic predisposition and a wide range of affected organs. We discuss the use of serum IgG4 concentrations as a disease marker in IgG4-RD, the association with malignant disease and outcomes according to differing treatment regimens.
Nikolopoulou A, Condon M, Turner-Stokes T, et al., 2019, Mycophenolate mofetil and tacrolimus versus tacrolimus alone for the treatment of idiopathic membranous glomerulonephritis: A randomised controlled trial., BMC Nephrology, Vol: 20, Pages: 1-9, ISSN: 1471-2369
Background: Tacrolimus (TAC) is effective in treating membranous nephropathy (MN);however relapses are frequent after treatment cessation. We conducted a randomisedcontrolled trial to examine whether the addition of mycophenolate mofetil (MMF) to TACwould reduce relapse rate.Methods: 40 patients with biopsy proven idiopathic MN and nephrotic syndrome wererandomly assigned to receive either TAC monotherapy (n=20) or TAC combined with MMF(n=20) for 12 months. When patients had been in remission for 1 year on treatment the MMFwas stopped and the TAC gradually withdrawn in both groups over 6 months. Patients alsoreceived supportive treatment with angiotensin blockade, statins, diuretics andanticoagulation as needed. Primary endpoint was relapse rate following treatmentwithdrawal. Secondary outcomes were remission rate, time to remission and change in renalfunction.Results: 16/20 (80%) of patients in the TAC group achieved remission compared to 19/20(95%) in the TAC/MMF group (p=0.34). The median time to remission in the TAC groupwas 54 weeks compared to 40 weeks in the TAC/MMF group (p=0.46). There was nodifference in the relapse rate between the groups: 8/16 (50%) patients in the TAC grouprelapsed compared to 8/19 (42%) in the TAC/MMF group (p=0.7). The addition of MMF toTAC did not adversely affect the safety of the treatment.Conclusions: Addition of MMF to TAC does not alter the relapse rate of nephrotic syndromein patients with MN.
Kousios A, Duncan N, Charif R, et al., 2019, Autologous stem cell transplant for the treatment of type I crystal cryoglobulinaemic glomerulonephritis caused by monoclonal gammopathy of renal significance (MGRS), Kidney International Reports, Vol: 4, Pages: 1342-1348, ISSN: 2468-0249
Cryoglobulins (CGs) are immunoglobulins that precipitate at temperatures below 37°C and dissolve again after rewarming. Cryoglobulinemia may be asymptomatic or cause end-organ damage by CG precipitation in small- to medium-sized blood vessels.1 In their seminal work, Brouet et al.2 classify cryoglobulinemias into 3 subgroups according to CG composition and clonality. In type II cryoglobulinemia there is a mixture of monoclonal IgM with rheumatoid factor activity and polyclonal IgG. In type III, CGs consist of polyclonal IgM and IgG.1 Type II and III cryoglobulinemias are also referred to as mixed cryoglobulinemias and are often caused by chronic hepatitis C infection and less frequently by autoimmune diseases or other viral infections (hepatitis B infection, HIV).3CGs in type I cryoglobulinemia are monoclonal Igs (MIg), also known as paraproteins, commonly IgG, IgM subtypes, or free light chains. The underlying pathological process is a plasma cell or B-cell lymphoproliferative disease, such as multiple myeloma (MM), Waldenström macroglobulinemia, chronic lymphocytic leukemia, or other B-cell non-Hodgkin lymphoma. However, in approximately 40% of symptomatic cases, the plasma cell or B-cell clone is too small to fulfill the diagnostic criteria of MM or overt lymphoma. The term monoclonal gammopathy of undetermined significance (MGUS) used for these cases is a misnomer, as the MIg causes disease regardless of the size and tumor burden.4 For cases with renal involvement, the International Kidney and Monoclonal Gammopathy Research Group introduced the term monoclonal gammopathies of renal significance (MGRS).5 The updated MGRS definition includes monoclonal gammopathies that cause renal disease but have low tumor burden and thus treatment from the hematological standpoint is not imminently indicated.6 These patients may have fewer than 10% plasma cells in bone marrow biopsy, smoldering myeloma, or low-grade lymphomas.7 MGRSs are not of undetermined significanc
Radhakrishnan ST, Ruban A, Uthayakumar AK, et al., 2019, Haemolytic Uraemic Syndrome - a rare case report of bloody diarrhoea in adults, BMC Gastroenterology, Vol: 19, Pages: 1-4, ISSN: 1471-230X
Background: Haemolytic uraemic syndrome is a rarely seen in adults often leading to critical illness.This case highlights how difficult it can be to establish a diagnosis and treat when a patient presentswith bloody diarrhoea.Case Presentation: A 17-year-old Iraqi man presented to the emergency department with abdominalpain and bloody diarrhoea. He was initially treated as acute appendicitis, undergoing anappendectomy but following a recurrence in his symptoms a colonoscopy was performed. Adiagnosis of shiga toxin-producing Escherichia coli leading to HUS was suspected following histologyobtained at colonoscopy and this was confirmed on antibody testing. Despite intravenous fluids andsupportive therapy the patient's symptoms and condition deteriorated. He developed seizures andacute renal failure requiring intubation and plasma exchange in the intensive care setting. Heeventually required treatment with ecluzimab therapy; a monoclonal antibody and subsequentlymade a full recovery.Conclusions: Haemolytic uraemic syndrome is a triad of progressive renal failure, thrombocytopeniaand haemolytic anaemia which is a condition rarely seen in adults. It is usually associated with an E.coli infection and supportive therapy remains the mainstay of treatment.
Mcadoo S, Tanna A, Kang A, et al., 2019, LONG TERM OUTCOMES OF PATIENTS WITH ANCA-ASSOCIATED VASCULITIS PRESENTING WITH SEVERE RENAL DYSFUNCTION, 19th International Vasculitis and ANCA Workshop, Publisher: OXFORD UNIV PRESS, Pages: 115-115, ISSN: 1462-0324
Sargsyan M, Kwame I, Levy J, et al., 2019, COMPARATIVE ANALYSIS OF COCAINE-ASSOCIATED LIMITED AUTOIMMUNE DISEASE WITH A PRIMARY LOCALIZED GPA COHORT, 19th International Vasculitis and ANCA Workshop, Publisher: OXFORD UNIV PRESS, Pages: 119-119, ISSN: 1462-0324
Gulati K, Mcadoo S, Tanna A, et al., 2019, PLASMAPHERESIS, RITUXIMAB AND LOW-DOSE CYCLOPHOSPHAMIDE FOR REMISSION INDUCTION THERAPY IN SEVERE ANCA-ASSOCIATED VASCULITIS, 19th International Vasculitis and ANCA Workshop, Publisher: OXFORD UNIV PRESS, ISSN: 1462-0324
Pepper RJ, McAdoo SP, Moran SM, et al., 2019, A novel glucocorticoid-free maintenance regimen for anti-neutrophil cytoplasm antibody-associated vasculitis (vol 58, pg 260, 2019), RHEUMATOLOGY, Vol: 58, Pages: 373-373, ISSN: 1462-0324
Pepper RJ, McAdoo SP, Moran SM, et al., 2019, A novel glucocorticoid-free maintenance regimen for anti-neutrophil cytoplasm antibody-associated vasculitis, Rheumatology, Vol: 58, Pages: 260-268, ISSN: 1462-0324
Objectives: Glucocorticoids (GCs) are a mainstay of treatment for patients with ANCA-associated vasculitis (AAV) but are associated with significant adverse effects. Effective remission induction in severe AAV using extremely limited GC exposure has not been attempted. We tested an early rapid GC withdrawal induction regimen for patients with severe AAV. Methods: Patients with active MPO- or PR3-ANCA vasculitis or ANCA-negative pauci-immune glomerulonephritis were included. Induction treatment consisted of two doses of rituximab, 3 months of low-dose CYC and a short course of oral GC (for between 1 and 2 weeks). Clinical, biochemical and immunological outcomes as well as adverse events were recorded. Results: A total of 49 patients were included, with at least 12 months of follow-up in 46. All patients achieved remission, with decreases observed in creatinine, proteinuria, CRP, ANCA level and BVAS. Three patients requiring dialysis at presentation became dialysis independent. Two patients required the introduction of maintenance GC for treatment of vasculitis. Overall outcomes were comparable to those of two matched cohorts (n = 172) from previous European Vasculitis Society (EUVAS) trials, but with lower total exposure to CYC and GCs (P < 0.001) and reduced rates of severe infections (P = 0.02) compared with the RITUXVAS (rituximab versus cyclophosphamide in AAV) trial. We found no new cases of diabetes in the first year compared with historic rates of 8.2% from the EUVAS trials (P = 0.04). Conclusion: Early GC withdrawal in severe AAV is as effective for remission induction as the standard of care and is associated with reduced GC-related adverse events.
McAdoo SP, Medjeral-Thomas N, Gopaluni S, et al., 2019, Long-term follow-up of a combined rituximab and cyclophosphamide regimen in renal anti-neutrophil cytoplasm antibody-associated vasculitis, Nephrology Dialysis Transplantation, Vol: 34, Pages: 63-73, ISSN: 0931-0509
Background: Current guidelines advise that rituximab or cyclophosphamide should be used for the treatment of organ-threatening disease in anti-neutrophil cytoplasm antibody (ANCA)-associated vasculitis (AAV), although few studies have examined the efficacy and safety of these agents in combination. Methods: We conducted a single-centre cohort study of 66 patients treated with a combination of oral corticosteroids, rituximab and low-dose pulsed intravenous cyclophosphamide followed by a maintenance regimen of azathioprine and tapered steroid for the treatment of biopsy-proven renal involvement in AAV. Patients were followed for a median of 56 months. Case-control analysis with 198 propensity-matched cases from European Vasculitis Study Group (EUVAS) trials compared long-term differences in relapse-free, renal and patient survival. Results: At entry, the median Birmingham Vasculitis Activity Score (BVAS) was 19 and estimated glomerular filtration rate was 25 mL/min. Cumulative doses of rituximab, cyclophosphamide and corticosteroids were 2, 3 and 4.2 g, respectively, at 6 months. A total of 94% of patients achieved disease remission by 6 months (BVAS < 0) and patient and renal survival were 84 and 95%, respectively, at 5 years. A total of 84% achieved ANCA-negative status and 57% remained B cell deplete at 2 years, which was associated with low rates of major relapse (15% at 5 years). The serious infection rate during long-term follow-up was 1.24 per 10 patient-years. Treatment with this regimen was associated with a reduced risk of death {hazard ratio [HR] 0.29 [95% confidence interval (CI) 0.125-0.675], P = 0.004}, progression to end-stage renal disease (ESRD) [HR 0.20 (95% CI 0.06-0.65), P = 0.007] and relapse [HR 0.49 (95% CI 0.25-0.97), P = 0.04] compared with propensity-matched patients enrolled in EUVAS trials. Conclusions: This regimen is potentially superior to current standards o
Rawson T, Sivakumaran P, Lobo R, et al., 2018, Development of a web-based tool for undergraduate engagement in medical research; the ProjectPal experience, BMC Medical Education, Vol: 18, ISSN: 1472-6920
BackgroundWe report the development and evaluation of a web-based tool designed to facilitate student extra-curricular engagement in medical research through project matching students with academic supervisors.UK based university students were surveyed to explore their perceptions of undergraduate research, barriers and facilitators to current engagement. Following this, an online web-based intervention (www.ProjectPal.org) was developed to support access of students to research projects and supervisors. A pilot intervention was undertaken across a London-based university in January 2013 to February 2016. In March 2016, anonymised data were extracted from the prospective data log for analysis of website engagement and usage. Supervisors were surveyed to evaluate the website and student outputs.ResultsFifty-one students responded to the electronic survey. Twenty-four (47%) reported frustration at a perceived lack of opportunities to carry out extra-curricular academic projects. Major barriers to engaging in undergraduate research reported were difficulties in identifying suitable supervisors (33/51; 65%) and time pressures (36/51; 71%) associated with this. Students reported being opportunistic in their engagement with undergraduate research. Following implementation of the website, 438 students signed up to ProjectPal and the website was accessed 1357 times. Access increased on a yearly basis. Overall, 70 projects were advertised by 35 supervisors. There were 86 applications made by students for these projects. By February 2016, the 70 projects had generated 5 peer-review publications with a further 7 manuscripts under peer-review, 14 national presentations, and 1 national prize.ConclusionThe use of an online platform to promote undergraduate engagement with extra-curricular research appears to facilitate extra-curricular engagement with research. Further work to understand the impact compared to normal opportunistic practices in enhancing student engagement is now
Wilson H, Turner-Stokes T, Griffith M, et al., 2018, Twenty years of lupus nephritis management - evaluation of a multi ethnic patient cohort to identify factors affecting outcome, 55th Congress of the European-Renal-Association (ERA) and European-Dialysis-and-Transplantation-Association (EDTA), Publisher: Oxford University Press (OUP), ISSN: 0931-0509
Gulati K, McAdoo S, Galliford J, et al., 2018, PLASMAPHERESIS, RITUXIMAB AND LOW-DOSE CYCLOPHOSPHAMIDE FOR REMISSION INDUCTION THERAPY IN SEVERE ANCA-ASSOCIATED VASCULITIS, 55th Congress of the European-Renal-Association (ERA) and European-Dialysis-and-Transplantation-Association (EDTA), Publisher: OXFORD UNIV PRESS, ISSN: 0931-0509
Rashid T, Perinpathasan K, Casley C, et al., 2018, The use of alternative renal monitoring in people living with HIV (PLWH) over the age of 50 to predict renal disease, European AIDS conference, Publisher: WILEY, Pages: S87-S87, ISSN: 1464-2662
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