One-fifth of all individuals commencing Atripla will need to switch therapy, often for adverse events. The commonest reason for switch in our cohort was CNS toxicity, which although it may develop shortly after initiation may persist, ultimately leading to discontinuation of Atripla months or years later.
Aims Technetium-99m-labelled 3,3-diphosphono-1,2-propanodicarboxylic acid (99mTc-DPD scintigraphy) is recognized as highly accurate for the non-invasive diagnosis of transthyretin (ATTR) cardiac amyloidosis (CA). A proportion of patients with immunoglobulin light chain (AL) CA have also been reported to show cardiac 99mTc-DPD uptake. Herein, we assessed the frequency and degree of cardiac 99mTc-DPD uptake and its clinical significance among patients with AL CA. Methods and results Between 2010 and 2017, 292 consecutive patients with AL CA underwent 99mTc-DPD scintigraphy and were included in this study: 114 (39%) had cardiac 99mTc-DPD uptake: grade 1 in 75%, grade 2 in 17%, and grade 3 in 8% of cases. Patients with cardiac 99mTc-DPD uptake had poorer cardiac systolic function and higher N-terminal pro-brain natriuretic peptide. No differences were noted in cardiac magnetic resonance parameters between patients with and without cardiac 99mTc-DPD uptake (N = 19 and 42, respectively). Patients with cardiac 99mTc-DPD uptake showed a trend to worse survival than those with no uptake (log-rank P = 0.056). Among 22 patients who underwent serial 99mTc-DPD scintigraphy, 5 (23%) showed reduction in the grade of cardiac uptake. Conclusions In this large cohort of patients with AL CA, 99mTc-DPD scintigraphy ∼40% of cases showed cardiac uptake, including grade 2–3 in 10% of all patients (25% of those with cardiac 99mTc-DPD uptake). Cardiac 99mTc-DPD uptake was associated with poorer cardiac function and outcomes. These data highlight the critical importance of ruling out AL amyloidosis in all patients with cardiac 99mTc-DPD uptake to ensure such patients are not assumed to have ATTR CA.
The development of antibody responses to SARS-CoV-2 is an indicator of seroprevalence and may afford protection from infection. It has been presumed that antibody responses to SARS-CoV-2 will be impaired in patients with aggressive haematological malignancy (PHM) due to underlying immunological dysfunction caused by malignancy or systemic anti-cancer treatment (SACT), placing them at increased risk. Here we analysed longitudinal serum samples from ten hospitalised PHM with aggressive disease and on SACT, collected up to 103 days post-onset of COVID-19 symptoms. We found that the majority (8/9) of PHM with confirmed SARS-CoV-2 infection seroconverted and developed antibodies to the major SARS-CoV-2 antigens (S1 and N) with most (6/8) produced neutralising antibody responses. Furthermore, the dynamics of antibody responses were broadly similar to that reported for the general population, except for a possible delay to seroconversion. Our finding that PHM on SACT can make functional antibody responses to SARS-CoV-2 has important implications for patient management and serological monitoring of SARS-CoV-2 in high-risk groups.
Background and aims Sickle cell anaemia (SCA) is an autosomal recessive disorder caused by point mutation of the β-globin gene, resulting in abnormal forms of hemoglobin that cause increased red blood cell rigidity and hemolysis. It is one of the most common hereditary blood conditions, affecting over 14,000 adults in the UK (Dormandy el al, 2017). One of the manifestations of SCA is vaso-occlusive crises. These typically cause severe pain that may require emergency department (ED) attendance for pain management, typically with opioids. Pain relief should be given quickly and response to this assessed on a regular basis to ensure pain management is optimized. Those patients in whom pain relief is not well-controlled are at risk of further complications including acute chest syndrome. The UK National Institute of Clinical Excellence (NICE) published a quality standard in 2014 stating that patients presenting to hospital with an acute painful sickle cell episode should have a pain assessment, a clinical assessment and appropriate analgesia within 30 minutes of presentation (NICE, 2014). This study was performed to assess the Whittington Hospital's compliance to national recommendations and to establish which aspects of care in ED contributed to delays in management. Methods If a Whittington SCA patient attends ED, an automated email is generated that notifies the haematology team of the attendance. This system was used to identify acute sickle cell presentations to ED. Criteria for inclusion in the study was Whittington SCA patients that presented to ED with acute painful sickle cell crises between August 2017 to January 2018. Patients who received analgesia in the ambulance and patients with no documentation available were excluded. The time of presentation, analgesia prescription and administration for each attendance were noted from ED documentation. Results A total of 104 ED SCA attendances were included. 41% of patients presenting with an acute painful sickle cell crisis received analgesia during their first 30 minutes in ED. The average wait for analgesia was 47 minutes, with 75% of SCA patients receiving analgesia within 1 hour of arrival. The time taken to triage SCA patients was on average 8 minutes (range 0 to 29 minutes). Time from arrival to prescription of pain relief was much more variable with an average wait 40 minutes (range 10 minutes to 2 hours 22 minutes). Time from prescription to administration also varied, with 56% administered within 10 minutes of prescription and 87% within 30 minutes Patients who frequently attended Whittington ED (defined as 7 or more attendances within the 6 month period studied) had a shorter average wait for analgesia. Analgesia was given 36 minutes after arrival on average for frequent attenders, whereas patients presenting 6 or fewer times had an average wait of 53 minutes. Conclusions We are not currently meeting our audit standard for provision of analgesia in the emergency department, and performance appears to have worsened progressively since our earliest available date from 2012 (although methodological differences may have contributed to this). In 2015 49% of patients received analgesia within 30 minutes compared to 41% in 2017/18. Most of the delay appears to be due to the time taken for medication to be prescribed, although the time taken to triage the patient and administer the medication was also not insignificant and often amounted to greater than 30 minutes. It is unclear what is contributing to this delay, although it appears that performance is improved when the patient is a repeat attender and therefore known to the department. Educations sessions with the ED department and availability of a SCA specialist nurse may improve management of SCA painful crises in ED. Disclosures Shah: Novartis: Honoraria, Speakers Bureau; Sobi/Apotex: Honoraria; Celgene Corp: Other: Steering committee; Roche: Other: Advisory board meeting.
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