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We report the case of a young female adult in her early 20s, who had COVID-19 infection for 8 weeks and COVID-19 vaccination 4 weeks prior to presentation with an extensive rash associated with erythema multiforme, resembling varicella zoster on initial presentation. After initial acyclovir therapy with no improvement, systemic corticosteroid treatment dramatically resolved the patient’s skin rash.

ObjectivesGiven the fundamental role of newborn bloodspot screening (NBS) to enable prompt diagnosis and optimal clinical management of individuals with sickle cell disease (SCD), we sought to systematically assess enablers and barriers to implementation of NBS programmes for SCD in Africa using established qualitative research methods.SettingChildbirth centres and NBS laboratories from six countries in East, West and Southern Africa.ParticipantsEight programme leaders involved with establishing and operating NBS programmes for SCD in Angola, Democratic Republic of Congo, Ghana, Liberia, Nigeria and Tanzania.Primary and secondary outcome measuresData obtained through a structured, phased interview approach were analysed using a combination of inductive and deductive codes and used to determine primary themes related to the implementation and sustainability of SCD NBS programmes.ResultsFour primary themes emerged from the analysis relating to governance (eg, pragmatic considerations when deploying overcommitted clinical staff to perform NBS), technical (eg, design and execution of operational processes), cultural (eg, variability of knowledge and perceptions of community-based staff) and financial (eg, issues that can arise when external funding may effectively preclude government inputs) aspects. Key learnings included perceived factors that contribute to long-term NBS programme sustainability.ConclusionsThe establishment of enduring NBS programmes is a proven approach to improving the health of populations with SCD. Organising such programmes in Africa is feasible, but initial implementation does not assure sustainability. Our analysis suggests that future programmes should prioritise government partner participation and funding from the earliest stages of programme development.

Objective To determine the prevalence, correlates and prognostic implications of anaemia and iron deficiency (ID) in patients with heart failure (HF) in Tanzania. Method This was a cross-sectional and prospective observational study conducted at Muhimbili National Hospital in Dar es Salaam, Tanzania. Patients were ≥18 years of age, with HF defined according to the Framingham criteria. The primary outcome was anaemia and the secondary outcome was a composite of hospitalisation for HF or all-cause mortality. Results A total of 401 HF patients (median age 56 years, IQR 41–67 years; women 51%) were included. The prevalence of anaemia was 57%. The overall prevalence of ID was 49% distributed as 69% versus 21% in subjects with and without anaemia (p

Summary points Sickle cell disease is characterised by unpredictable episodes of acute illness, progressive organ damage, and a lack of effective treatments. It is one of the most common inherited conditions, although its prevalence varies widely. Median life expectancy is currently 40-60 years in high income countries but much less in low income areas.1 2 It is associated with protean clinical complications. Patients present to all medical specialties and increasingly to general practitioners. This review aims to provide an evidence based update on how to manage patients with this disease in the community. It does not consider sickle cell trait, which is largely asymptomatic. #### Sources and selection criteria We searched Medline, the Cochrane Database, and ClinicalTrials.gov using the term “sickle” together with certain complications including pain, infection, enuresis, renal failure, spleen, acute chest syndrome, leg ulcers, and stroke. Preference was given to randomised clinical trials, and when these were unavailable large case series were sought. We prioritised newer studies over older ones. Sickle cell disease …

Aim To test the hypothesis that low iron status in children with Sickle Cell Anaemia (SCA), not receiving regular blood transfusions, is associated with lower nocturnal haemoglobin oxygen saturation (SpO 2 ) and more sleep-disordered breathing. Methods 32 children (16 boys, 50%), homozygous for SCA (HbSS; mean age 8.0 years, range 3.6–15.3 years) underwent motion resistant nocturnal oximetry (Masimo Radical). Iron status was assessed by transferrin saturation and averaged steady state haematology indices from the previous year. Only studies with a minimum of 4 h of artifact-free data were included in the analysis. Analysis software yielded standard measures including: mean and minimum SpO2; δ-12s index and desaturation index of 3% or greater from baseline. Results 28% (9/32) had low transferrin saturation ( Conclusions These data suggest that higher iron availability, assessed by transferrin saturation, is associated with nocturnal chronic and intermittent hypoxia. Whether transferrin saturation is aetiologically related to or simply a marker of the observed haemoglobin oxygen desaturation remains to be determined. The longer term consequences of iron status and haemoglobin oxygen desaturation in SCA warrant further detailed investigation in view of the preliminary clinical evidence of a link with unfavourable clinical course.

Sickle cell disease (SCD) is the most common genetic disorder in the UK. Increasing evidence suggests that autonomic function is altered in SCD, and if sympathetic drive is increased, this might increase the vascular tone of the intracranial arteries, leading to narrowing and increased velocities. Deep inspiratory gasps generate a vasomotor reflex characterised by sympathetic mediated transient vasoconstriction of skin arterioles. The aim of this study was to assess the relationship between autonomic reactivity, oxygen saturation (SpO 2 ) and cerebral blood flow velocity (CBFV) in children with SCD. Methods Autonomic reactivity was assessed using the inspiratory gasp vascular responses (IGVR) in 71 children with SCD (median age 7.8, range 2.8–15.3) years and 26 controls (7.7, 3.2–12.8). Cutaneous blood flux was measured by laser Doppler fluximetry (VMS and VP1T probe, Moor Instruments UK) on the pulp of the index finger of the non-dominant hand at rest and during three deep inspiratory gasps. CBFV was measured in the middle cerebral artery (MCA) and basilar artery (BA) using transcranial Doppler (DopplerBox, ScanMed, UK); daytime and minimum nocturnal SpO 2 using pulse oximetry (Masimo, Irvine CA). Results Mean resting blood flux (MRBF), IGVR, CBFV in the right and left MCA and BA were all higher in SCD compared to controls (p=0.002, p=0.001, p 2 and minimum nocturnal SpO 2 were lower (p 2 (r=−0.286, −0.269; p=0.011, 0.019). Left and right MCA and BA velocities were correlated with MRBF (r=0.241, 0.364, 0.283; p=0.47, 0.002, 0.022, respectively) and IGVR (r=0.420, 0.307, 0.451; p 2 was inversely correlated with maximum CBFV in MCA and BA (r= −0.223, −0.276; p=0.044, 0.015). In multiple linear regression, IGVR predicted maximum MCA CBFV (r 2 =0.097, p=0.011); SpO 2 predicts IGVR (r 2 =0.058 p=0.037) but not CBFV. IGVR predicts CBFV independently of SpO 2 in a stepwise model. Conclusion The study provides evidence of increased MRBF, sympathetic tone and CBFV in children with SCD and low SpO 2 which may improve understanding of the pathogenesis and prevention of cerebrovascular disease and neurological complications.