Northern General Hospital

Spontaneous coronary artery dissection (SCAD) is a cause of acute myocardial infarction predominantly affecting adult women. A proportion of SCAD cases are associated with rare heritable connective tissue disorders. Vascular EDS (vEDS), due to deleterious variants in COL3A1, is one of the most common of these. Our aim was to identify specificfeatures of SCAD in vEDS which may aid patient selection for genetic testing. A systematic review of published cases of individuals with SCAD and vEDS was conducted. Additionally, patients with SCAD and genetically confirmed vEDS (SCAD-vEDS) were identified through the UK national EDS service and UK SCAD registry. Data were collected on presentation, management and extra-cardiac findings. Angiography was compared with an age and sex-matched, exome sequenced, control cohort with SCAD but without vEDS (SCAD-nonvEDS). Data from ten SCAD-vEDS patients were identified. There was a lower average age of SCAD and higher proportion of males in individuals with SCAD-vEDS, however differences should be interpreted carefully given cohort size. Fifty-six cases of SCAD-vEDS were identified through systematic review. Systemic features were present in most but not all cases. This report presents a new, angiographically characterised case-control cohort along with a systematic review of the current literature. Whilst clinical differences appear between the SCAD-vEDS and SCAD-nonvEDS groups, these are insufficient to accurately distinguish SCAD-vEDS from the general SCAD population. All individuals with SCAD should be evaluated for underlying vEDS but clinical assessment will miss some cases. Wider genetic testing in some SCAD patients may be merited to enable appropriate management. Systematic review registration: https://www.crd.york.ac.uk/prospero/536751 Identifier: 536751.

In the United Kingdom and Sweden, men aged 65 are offered screening for Abdominal Aortic Aneurysm (AAA). Men with small AAA enter a surveillance programme to monitor growth until the AAA is large enough for referral for treatment. Some men develop anxiety related to having an AAA or being in surveillance. The original aim was to develop and assess the acceptability of a new intervention to help men in surveillance manage anxiety. As the study progressed, the aim changed to developing an information booklet to address men's uncertainties about AAA and surveillance because uncertainties might lead to anxiety. An intervention development study was undertaken, following guidance in three phases: 1. Identifying need using surveys of screening staff and men, and qualitative interviews with men and family members; 2. Co-design of an intervention using a literature review, programme theory development, and two workshops with men, family members, and a patient representative; 3. Assessing the acceptability of the intervention using a telephone survey of 23 men in AAA surveillance. Although the original aim was to develop an intervention to help men manage anxiety, men and screening staff identified the need for an information-based intervention to address men's uncertainties about AAA. Published evidence identified that uncertainty about health conditions or treatment can lead to anxiety, so the intervention taken forward was an information booklet to address men's uncertainties about AAA. A 16-page A5 booklet was developed with men and their family members, addressing why men had to wait for AAA to become large before referral for treatment, the risk of rupture for different sizes of AAA, and how to reduce the risk of rupture. In the telephone survey, 20/23 men in the AAA surveillance who read the draft booklet found it helpful because it addressed their uncertainties. They suggested minor refinements. A refined version of the prototype booklet was produced based on this feedback. A co-designed information booklet is available that addresses the uncertainties of men with AAA in surveillance. Future research should measure the impact of the information booklet on AAA-related anxiety. We set up a patient panel specifically for this study. We identified five men from different sources including asking the vascular clinicians on the team to invite patients to consider joining the panel and approaching existing health research studies patient panels to identify men who had AAA. One member of our team was the patient representative on the research committee for the NHS AAA Screening Programme in England. He attended all our patient panel meetings. The panel reviewed all the documents we used to invite men to different parts of the study, offered advice about recruitment, and gave feedback about the findings. They offered advice about the prototypes of the information booklet.

Granulomatosis with polyangiitis (GPA) is an uncommon autoimmune anti-neutrophilic cytoplasmic antibody-associated vasculitis. Although it was first recognized nearly a century ago, its etiology is still under investigation. Numerous previous studies have widely suggested that neutrophils play a central role in its pathogenesis. This granulomatous necrotizing inflammation has historically been recognized to commonly affect the ear, nose, throat, lungs, and kidneys; however, other organ involvement, including hepatic involvement, has also been occasionally observed. Some older retrospective case studies have reported fatal outcomes associated with liver involvement due to GPA, highlighting the diagnostic challenges it presents. Hence, we present a case report describing a middle-aged gentleman who presented with persistent constitutional symptoms and continuously deranged liver function tests (LFTs) and was ultimately diagnosed with GPA with hepatic involvement.

Frostbite remains a common cold injury in military units operating in severe climates, yet the epidemiology and physiologic screening markers for vulnerable personnel are not fully characterized. We conducted a cross-sectional study of 1,250 male servicemen stationed in northern subarctic/alpine regions, combining a structured questionnaire with anthropometry (skinfolds), estimated maximal oxygen uptake (VO2 max; Bruce protocol), and a standardized hand/foot cold-induced vasodilation (CIVD) protocol. Frostbite was reported by 688 participants (55.0%), predominantly first-degree; feet (78.1%) and hands (75.0%) were most affected, with incidents clustering between November and February at -20 °C to -50 °C. In multivariable models, recruit status, southern origin before enlistment, and participation in field operational training were associated with higher odds of frostbite, whereas subcutaneous fat and VO2 max were not independent predictors. A unitless CIVD index summarizing response timing and magnitude discriminated frostbite status with an area under the ROC curve (AUC) of 0.778; at the Youden cut-off, sensitivity was 70.5% and specificity was 85.5%. These findings indicate a high frostbite burden concentrated during winter operational activities and support the practical utility of a simple CIVD index to identify high-risk individuals for targeted prevention and training. Prospective studies that integrate detailed exposure metrics and protective-equipment use are warranted to refine prediction and evaluate prevention strategies.

<h3>Background</h3> Clinical outcomes for NTM-PD are often poor. The NTM Network UK Standards of Care were launched in 2024 to support consistent, high-quality practice including pharmacological and non-pharmacological treatment. We aimed to document NTM-PD management prior to this to identify opportunities to improve care. <h3>Methods</h3> Centres were invited via the NTM Network UK to complete an online retrospective survey. At each site, anonymised clinical data were recorded from three consecutive adults (≥18) newly diagnosed with NTM-PD from 1 January 2022. <h3>Results</h3> 48 centres participated (Northern England, n=12; Southern England, n=12; London, n=9; Wales, n=7; Midlands, n=6; Scotland, n=2) They reported having the following resources: physiotherapy for airway clearance (n=42/48, 88%), dietitian and pulmonary rehabilitation (each n=32, 67%), specialist nurse (n=30, 63%), and health psychology (n=9, 19%). Respondents contributed data on 137 patients. 72 (53%) were female and 115 (84%) identified as White-British. 45 (34%) were aged 61–70. 62 (45%) had COPD and 55 (40%) bronchiectasis. Common radiological findings were nodular-bronchiectatic change (n=62, 45%), fibro-cavitary disease (n=40, 29%), and emphysema (n=39, 28%). <i>Mycobacterium avium complex</i> (MAC) was identified in 106 (77%), <i>M. kansasii</i>(n=14, 10%) and <i>M. abscessus</i> (n=9, 7%). Eighty-one (59%) patients were discussed in an MDT. Treatment plans included involvement of specialist nurses (n=91, 66%), physiotherapists (n=52, 38%), dietitians (n=34, 25%), and pulmonary rehabilitation (n=16, 12%). Forty-two (31%) were advised on airway clearance using Active Cycle Breathing, 25 (18%) Positive Expiratory Pressure devices, 17 (12%) mucoactive drugs, and 12 (9%) hypertonic saline. Written resources were provided to 39 (28%) patients, 22 (16%) were directed to patient-focused websites, and 13 (10%) signposted to peer-support networks. During a median follow-up from diagnosis of 21 (IQR 14–29) months, 124 (91%) started antibiotics for NTM-PD. Forty-five (33%) remain on follow-up and using antibiotics, 53 (39%) are not on antibiotics, 26 (19%) were discharged, and 12 (9%) died. <h3>Conclusion</h3> This UK-wide assessment of NTM-PD management reveals significant variation in available services and care provided. Most patients started specific antimicrobials, though non-medical health care support and patient information provision were limited. We have identified important areas for re-audit against the 2024 Standards.