Clinical medicine (London, England)
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Case Reports
Lessons of the month 1: Longitudinal extensive transverse myelitis following AstraZeneca COVID-19 vaccination.
Longitudinal extensive transverse myelitis (LETM) is a rare but recognised complication of vaccination. We report the case of a 58-year-old man admitted to hospital 10 days after his first AstraZeneca COVID-19 vaccination with progressive neurological symptoms and signs, and investigations and imaging consistent with LETM. This case reviews the literature and the investigative process behind excluding other diagnoses given the patient's background of pulmonary sarcoidosis. It is unique in being the first UK report of a case of LETM with a strong temporal link to COVID-19 vaccination.
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The COVID-19 pandemic has led to adaptations being made to all aspects of the NHS, including general practice, acute medical specialties and oncology. This has presented unique challenges to acute oncology services (AOSs) in how to provide continuity of care while maintaining the safety of patients and staff. ⋯ The patient population reviewed in April 2020 (at the initial peak of the pandemic in the UK) was markedly different to one reviewed in April 2019, with 55% more patients presenting with a new diagnosis of cancer via an emergency route. Finally, we suggest changes to AOSs for future waves of the pandemic.
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Case Reports
Lessons of the month 2: Meningococcal epiglottitis and connective tissue disease associated with C2 deficiency.
A 68-year-old woman was referred for immunological investigation following an episode of meningococcal epiglottitis with associated septicaemia. Several years previously, she had been diagnosed with undifferentiated connective tissue disease. On investigation, alternative pathway complement function was normal; however, classical pathway complement activation was reduced. C1q, C3 and C4 levels were all measured and found to be within their respective normal ranges, but C2 levels were low. Sequencing of the C2 gene was subsequently performed, confirming a diagnosis of type 1 C2 deficiency (C2D). ⋯ C2D is usually hereditary and inherited in an autosomal recessive manner. C2D is often asymptomatic, however, some patients suffer from infections with encapsulated bacteria and/or autoimmune diseases, particularly systemic lupus erythematosus. Recognition of complement pathway deficiency is important due to the predisposition to severe and/or recurrent infections by encapsulated bacteria. Immunisations have the potential to reduce both mortality and morbidity not only for the patient but also for any affected relatives.
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A significant proportion of COVID-19 patients show evidence of hyperinflammation (HI), of which secondary haemophagocytic lymphohistiocytosis (sHLH) is the most severe manifestation and diagnosed with HScore. Using a COVID-relevant modification of the HScore (%HScore), we set out to determine the prevalence of sHLH in 567 COVID-19 inpatient cases. The overall incidence of individuals with an 80% probability of sHLH in our COVID-19 cohort was 1.59% on admission and only rose to 4.05% if calculated at any time during admission. ⋯ Overall, %HScores were lower in older patients (p<0.0001) and did not reliably predict outcome at any cut-off value (AUROC 0.533, p=0.211, odds ratio 0.99). Our study demonstrates that a modified version (%HScore) of the conventional sHLH scoring system (HScore) does not enable risk stratification in people hospitalised with COVID. We propose further work is needed to develop novel approaches to predict HI and improve trial stratification for HI directed therapy in people with COVID-19.
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The National Patient Safety Alert supporting early recognition and treatment of adrenal crisis is a vital new component of care for adults affected by primary adrenal insufficiency. Benefits for patients with secondary and tertiary adrenal insufficiency need to be weighed alongside other considerations such as security of the diagnosis, relative likelihood of adrenal crisis and potential for anxiety and distress from assigning 'physical dependency' in relation to glucocorticoid therapy. All clinicians must be vigilant for and responsive to managing risks of adrenal crisis in at-risk patients, while avoiding diagnostic anchoring in the context of acute illness. More research is required to help define who is at greatest risk of adverse outcomes (including avoidance of therapeutic glucocorticoid therapy for fear of adrenal insufficiency) and a cross-specialty approach is advocated.