Author: Jason Curtis

Coronary heart disease mortality in severe vs. non-severe familial hypercholesterolaemia in the Simon Broome Register (2019)

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Type of publication:
Journal article

Author(s):
Humphries, Steve E; Cooper, Jackie A; *Capps, Nigel; Durrington, Paul N; Jones, Ben; McDowell, Ian F W; Soran, Handrean; Neil, Andrew H W; Simon Broome Familial Hyperlipidaemia Register Group

Citation:
Atherosclerosis. 2019 Feb;281:207-212

Abstract:
BACKGROUND AND AIMS The International Atherosclerosis Society (IAS) has proposed that patients with "severe" FH (SFH) would warrant early and more aggressive cholesterol-lowering treatment such as with PCSK9 inhibitors. SFH is diagnosed if LDL-cholesterol (LDLC)>10mmol/L, or LDLC >8.0mmol/L plus one highrisk feature, or LDLC >5mmol/L plus two high-risk features. Here we compare CHD mortality in SFH and nonSFH (NSFH) patients in the UK prospective Simon Broome Register since 1991, when statin use became routine.METHODS 2929 definite or possible PFH patients (51% women) aged 20-79 years were recruited from 21 UK lipid clinics and followed prospectively between 1992 and 2016. The excess CHD standardised mortality ratio (SMR) compared to the England and Wales population was calculated (with 95% confidence intervals).RESULTS1982 (67.7%) patients met the SFH definition. Compared to the non-SFH, significantly (p < 0.001) more SFH patients had diagnosed CHD at baseline (24.6% vs. 17.5%), were current smokers (21.9% vs 10.2%) and had a BMI>30kg/m2 (14.9% vs. 7.8%). The SMR for CHD mortality was significantly (p=0.007) higher for SFH (220 (184-261) (34,134 person years, 129 deaths observed, vs. 59 expected) compared to NSFH of 144 (98-203) (15,432 person years, 32 observed vs. 22 expected). After adjustment for traditional risk factors, the Hazard Ratio for CHD mortality in SFH vs. NSFH was 1.22 (0.80-1.87) p=0.36, indicating that the excess risk was largely accounted for by these factors.CONCLUSIONS CHD mortality remains elevated in treated FH, especially for SFH, emphasising the importance of optimal lipid-lowering and management of other risk factors.

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Lymphangioma circumscriptum of the vulva clinical picture and surgical management (2018)

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Type of publication:
Journal article

Author(s):
*Papoutsis D.; Haefner H.K

Citation:
Archives of Hellenic Medicine 35(6):809-810 · November 2018

Abstract:
Vulvar lymphangioma circumscriptum is a rare entity that may mimic many other diseases of the vulva. It presents with the non-specific symptoms of persistent vulvar itching and soreness, and the diagnosis is confirmed through vulvar biopsy. Surgical treatment has the lowest recurrence rates compared with other treatment modalities. The case is presented here of a woman diagnosed with lymphangioma circumscriptum of the vulva and its surgical management.

Audit of 2-week wait referrals to the Gynecology Department in District General Hospital and investigating patient awareness of the reasons and importance behind the referral (2018)

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Type of publication:
Conference abstract

Author(s):
Wilkinson M.; *Sahu B

Citation:
BJOG: An International Journal of Obstetrics and Gynaecology; Dec 2018; vol. 125 ; p. 48

Abstract:
Introduction Two-week wait referrals to gynecology services are at a premium with pressure on numbers. Referrals can be inappropriate. Patients are often unaware the appointment is for suspected cancer. Methods Two-week wait referrals were examined during a six month period. The gynecologist in clinic collected data. Patients were asked about their 2-week wait referral, gauging awareness around their referral. Clinical symptoms and signs were compared to those in referral. Appropriateness of the referral was assessed by symptoms fitting the 2-week wait criteria or clinical findings on referral not consistent with the presenting symptoms and findings in clinic. Results A total of 172 patients were referred under the 2-week wait criteria; mean age was 58 years, range (17-95). Referrals were from 50 separate primary care practices. Suspected cancer referrals were composed of 111 endometrial, 15 ovarian, 22 cervical, 15 vulva/vaginal and 9 of mixed pathology. There was awareness of referral for cancer in 90 cases (52%), awareness of "2-week wait" in 124 cases (72%) and aware that the appointment could be at either hospital within the trust in 96 cases (56%). The referral was considered appropriate for 2-week wait referral in 123 (72%) of cases. Conclusion The majority of patients were referred correctly. A wide range of pathologies was seen. A significant number could have been referred as routine referrals or advice requested. Patient awareness of it being a referral due to suspected cancer was poor with better appreciation of the urgency of referral.

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Cancelled operations: a 7-day cohort study of planned adult inpatient surgery in 245 UK National Health Service hospitals (2018)

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Type of publication:
Journal article

Author(s):
Wong, D.J.N.; Harris, S.K.; Moonesinghe, S.R

Collaborators at Shrewsbury and Telford Hospital NHS Trust:
*Jane Wright, *Paul Jones, *Riquella Abbott, *Lisa Bacon, *Christopher Godden, *David Howe, *Angela Loughlin, *Helen Moore, *Samuel Passey, *Rebekah Rodgers

Citation:
BJA: The British Journal of Anaesthesia; Oct 2018; vol. 121 (no. 4); p. 730-738

Abstract:
Background: Cancellation of planned surgery impacts substantially on patients and health systems. This study describes the incidence and reasons for cancellation of inpatient surgery in the UK NHS. Methods: We conducted a prospective observational cohort study over 7 consecutive days in March 2017 in 245 NHS hospitals. Occurrences and reasons for previous surgical cancellations were recorded. Using multilevel logistic regression, we identified patient- and hospital-level factors associated with cancellation due to inadequate bed capacity.Results: We analysed data from 14 936 patients undergoing planned surgery. A total of 1499 patients (10.0%) reported previous cancellation for the same procedure; contemporaneous hospital census data indicated that 13.9% patients attending inpatient operations were cancelled on the day of surgery. Non-clinical reasons, predominantly inadequate bed capacity, accounted for a large proportion of previous cancellations. Independent risk factors for cancellation due to inadequate bed capacity included requirement for postoperative critical care [odds ratio (OR)=2.92; 95% confidence interval (CI), 2.12-4.02; P<0.001] and the presence of an emergency department in the treating hospital (OR=4.18; 95% CI, 2.22-7.89; P<0.001). Patients undergoing cancer surgery (OR=0.32; 95% CI, 0.22-0.46; P<0.001), obstetric procedures (OR=0.17; 95% CI, 0.08-0.32; P<0.001), and expedited surgery (OR=0.39; 95% CI, 0.27-0.56; P<0.001) were less likely to be cancelled.Conclusions: A significant proportion of patients presenting for surgery have experienced a previous cancellation for the same procedure. Cancer surgery is relatively protected, but bed capacity, including postoperative critical care requirements, are significant risk factors for previous cancellations.

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The current standard of care and the unmet needs for axial spondyloarthritis (2018)

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Type of publication:
Journal article

Author(s):
*Noureldin, Basil; Barkham, Nick

Citation:
Rheumatology; Volume 57, Issue suppl_6, November 2018, Pages vi10–vi17

Abstract:
The aim of this article is to explore the benefits and limitations of the established treatments for axial SpA (axSpA), including physiotherapy, NSAIDs, conventional synthetic DMARDs and biologic DMARDs such as TNF inhibitors (TNFis). It also briefly discusses the emerging role of anti-IL-17 therapy, which could be used as a valuable alternative to first-line biologic DMARD treatment or as a second-line treatment for patients who are inadequate responders to TNFi therapy, as evidenced by various studies. Exercise programmes improve health related quality of life and hydrotherapy improves disease activity and functional parameters in AS. NSAIDs have been proven to substantially relieve symptoms in 70–80% of patients and enhance physiotherapy by reducing pain and stiffness. The role of NSAIDs in preventing radiographic progression remains unclear. The use of conventional synthetic DMARDs (csDMARDs) is limited to peripheral arthritis; there is insufficient evidence to support the use of csDMARDs for axial disease. TNFi therapy reduces the disease activity of axSpA, however, as not all patients respond to treatment in the same way, it is good to have other therapeutic options available. Finally, this article explores the potential for IL-17 inhibition in AS and introduces clinical data for secukinumab, a fully human monoclonal antibody targeting IL-17A.

Charcot Marie Tooth disease type 2S with late onset diaphragmatic weakness: An atypical case (2018)

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Type of publication:
Journal article

Author(s):
Kulshrestha, Richa; Forrester, Natalie; Antoniadi, Thalia; Willis, Tracey; *Sethuraman, Sethil Kumar; Samuels, Martin

Citation:
Neuromuscular disorders : NMD; Dec 2018; vol. 28 (no. 12); p. 1016-1021

Abstract:
Immunoglobulin-helicase-μ-binding protein 2 (IGHMBP2) mutations are associated with partial continuum between two extremes of rapidly lethal disorder of spinal muscular atrophy with respiratory distress type 1 (SMARD1), with infantile axonal neuropathy, diaphragmatic weakness and commonly death before 1 year of age, and Charcot-Marie-Tooth disease (CMT) type 2S with slowly progressive weakness and sensory loss but no significant respiratory compromise. We present an atypical case of CMT2S. A 9 month old boy presented with bilateral feet deformities and axonal neuropathy. Genetic testing revealed two heterozygous variants in the IGHMBP2 gene: c.1156 T>C p.(Trp386Arg) in exon 8 and c.2747G>A p.(Cys916Tyr) in exon 14, that were inherited from his father and mother respectively. At 9 years, he developed diaphragmatic weakness, following which he was established on non-invasive ventilation. Our case emphasizes the importance of life long respiratory surveillance for patients with CMT2S and expands the phenotype of this condition.

A patient-centred model to quality assure outputs from an echocardiography department: consensus guidance from the British Society of Echocardiography (2018)

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Type of publication:
Journal article

Author(s):
*Ingram, Thomas E; Baker, Steph; Allen, Jane; Ritzmann, Sarah; Bual, Nina; Duffy, Laura; Ellis, Chris; Bunting, Karina; Black, Noel; Peck, Marcus; Hothi, Sandeep S; Sharma, Vishal; Pearce, Keith; Steeds, Richard P; Masani, Navroz

Citation:
Echo research and practice; Dec 2018; vol. 5 (no. 4); p. G25

Abstract:
Background Quality assurance (QA) of echocardiographic studies is vital to ensure that clinicians can act on findings of high quality to deliver excellent patient care. To date, there is a paucity of published guidance on how to perform this QA. The British Society of Echocardiography (BSE) has previously produced an Echocardiography Quality Framework (EQF) to assist departments with their QA processes. This article expands on the EQF with a structured yet versatile approach on how to analyse echocardiographic departments to ensure high-quality standards are met. In addition, a process is detailed for departments that are seeking to demonstrate to external bodies adherence to a robust QA process. Methods The EQF consists of four domains. These include assessment of Echo Quality (including study acquisition and report generation); Reproducibility & Consistency (including analysis of individual variability when compared to the group and focused clinical audit), Education & Training (for all providers and service users) and Customer & Staff Satisfaction (of both service users and patients/their carers). Examples of what could be done in each of these areas are presented. Furthermore, evidence of participation in each domain is categorised against a red, amber or green rating: with an amber or green rating signifying that a quantifiable level of engagement in that aspect of QA has been achieved. Conclusion The proposed EQF is a powerful tool that focuses the limited time available for departmental QA on areas of practice where a change in patient experience or outcome is most likely to occur.

Link to full-text [NHS OpenAthens account required]

Screening for colorectal cancer in defunctioned colons (2018)

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Type of publication:
Journal article

Author(s):
*Akbar, Fayyaz; Quyn, Aaron; Steele, Robert

Citation:
Journal of medical screening; Dec 2018; vol. 25 (no. 4); p. 178-182

Abstract:
OBJECTIVES Population-based colorectal (bowel) cancer screening using faecal occult blood tests leads to a reduction in cause-specific mortality. However, in people where the colon is defunctioned, the use of standard faecal occult blood test is not appropriate. The aim of this study was to examine the current trends of clinical practice for colorectal cancer screening in people with defunctioned colons.METHODS An online survey was performed using SurveyMonkey. All members of the Association of Coloproctology of Great Britain and Ireland were invited by email to participate. Reminders were sent to non-responders and partial responders till six weeks. All responses were included in our analysis. RESULTS Of the 206 (34.59%) questionnaires completed, all questions were answered in 110 (55.8%). Among responders, 94 (85.4%) were colorectal consultant surgeons, 72% had worked in their current capacity for more than five years, and 105 (50.9%) had encountered colorectal cancer in defunctioned colons during their career. Some 72.2% of responders stated that a screening test for colorectal cancer in patients with defunctioned colons was currently not offered, or that they did not know whether or not it was offered in their area.CONCLUSIONS Bowel screening in the United Kingdom is currently not offered to 72.2% of the age appropriate population with defunctioned colons. Among responding colorectal surgeons, 50% had encountered colorectal cancer in such patients. There is considerable variability in clinical practice regarding the optimal age for onset of screening, time interval, and the optimal modality to offer for screening in such cases.

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Self-inflicted bilateral ocular and eyelid injuries in an unsuspected individual (2019)

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Type of publication:
Journal article

Author(s):
*Samia-Aly, Emma; *Ha, Jerome; *Sagili, Suresh

Citation:
Scottish medical journal; May 2019; vol. 64 (no. 2); p. 78-82

Abstract:
BACKGROUND AND AIMS This report aims to illustrate a case of self-inflicted ocular and orbital injury, resulting in severe tissue loss and ophthalmoplegia in a patient with no known history of mental illness.METHODS AND RESULTS A 71-year-old male initially presented to the emergency department with significant tissue loss from his left upper and lower lids, orbital tissue loss and complete ophthalmoplegia, after reportedly tripping and falling onto his desk. He subsequently attended the emergency department on two further occasions with similar injuries, affecting the same and contralateral eye, whilst maintaining a traumatic cause for his injuries. He was eventually admitted to a psychiatric ward for mental health assessment. This report covers his progress as well as illustrating his injuries with images. CONCLUSION Self-harm is an important differential diagnosis in cases where the mechanism of injury does not correspond to the extent of injury or tissue loss. It can, however, be difficult to differentiate from accidental injury and even with repeated assessments, a formal psychiatric diagnosis may not be possible.