Adult North Star Network (ANSN): Consensus Guideline For The Standard Of Care Of Adults With Duchenne Muscular Dystrophy (2021)

Type of publication:
Journal article

Author(s):
Quinlivan, R; Messer, B; Murphy, P; Astin, R; Mukherjee, R; Khan, J; Emmanuel, A; Wong, S C; Kulshresha, R; Willis, T; Pattni, J; *Willis, D; Morgan, A; Savvatis, K; Keen, R; Bourke, J; Marini Bettolo, C; Hewamadduma, C; ANSN

Citation:
Journal of Neuromuscular Diseases; Sep 2021 [epub ahead of print]

Abstract:
There are growing numbers of adults with Duchenne Muscular Dystrophy living well into their fourth decade. These patients have complex medical needs that to date have not been addressed in the International standards of care. We sought to create a consensus based standard of care through a series of multi-disciplinary workshops with specialists from a wide range of clinical areas: Neurology, Cardiology, Respiratory Medicine, Gastroenterology, Endocrinology, Palliative Care Medicine, Rehabilitation, Renal, Anaesthetics and Clinical Psychology. Detailed reports of evidence reviewed and the consensus building process were produced following each workshop and condensed into this final document which was approved by all members of the Adult North Star Network including service users. The aim of this document is to provide a framework to improve clinical services and multi-disciplinary care for adults living with Duchenne Muscular Dystrophy.

Primary acinic cell carcinoma in a young female patient: a case report (2021)

Type of publication:
Journal article

Author(s):
*Venkatasami, M ; *Harrison, K

Citation:
Oral Surgery, Oral Medicine, Oral Pathology & Oral Radiology; Jul 2021; vol. 132 (no. 1)

Abstract:
Background: Acinic cell carcinoma (ACC) is a rare, low-grade tumor, accounting for about 5% of all primary salivary gland malignancies. ACCs predominate in the parotid gland, are seen in the fifth and sixth decades of life, and have a female predilection. Well-differentiated and low-grade tumors are associated with a favorable prognosis.
Description: We report a case of a 16-year-old female patient presenting with a right parotid lump that had been slowly increasing over the past year. Clinical examination revealed a 1.5-cm right parotid nodule with no facial nerve involvement and presence of cervical lymphadenopathy. Her past medical history was unremarkable.
Findings: Radiological investigations revealed a well-circumscribed, lobulated lesion in the anterior right parotid gland with presence of reactive lymph nodes bilaterally. Fine-needle aspiration showed granular cells with a differential diagnosis of oncocytoma, Warthin's tumor, or acinic cell carcinoma. Histologic examination showed a multinodular appearance with some marked granular cytoplasm with oncolytic and lymphoid infiltrate. The results of immunohistochemistry were negative for S100 and positive with DOG-1. There were widespread periodic acid-Schiff diastase-resistant granules throughout the tumor cells, with a Ki67 proliferation index of approximately 5%.
Outcome: The tumor was completely excised, and the patient made a full recovery and was considered to have a good long-term prognosis.
Conclusions: This is an unusual case of ACC seen in this age group and gender, where less than 4% of cases of ACC have been reported in patients younger than 20. It is important to consider differential diagnoses of salivary gland pathology, such as ACC, in the young female patient.

Craniofacial osteosarcoma: a case report (2021)

Type of publication:
Journal article

Author(s):
*Venkatasami, M ; *Harrison, K

Citation:
Oral Surgery, Oral Medicine, Oral Pathology & Oral Radiology; Jul 2021; vol. 132 (no. 1)

Abstract:
Background: Osteosarcoma is the most common primary bone tumor, with 10% of cases affecting the head and neck. Demographics of head and neck osteosarcoma are different from those elsewhere in the musculoskeletal system. Prognosis is strongly dependent on negative resection margins with the use of neoadjuvant chemoradiotherapy in select cases.
Description: We present a case of a 58-year-old male nonsmoker patient who presented with a lump in his left upper jaw. Clinical examination revealed an exophytic mass in the upper left tuberosity of the maxilla suspicious for squamous cell carcinoma with no associated lymphadenopathy.
Findings: Radiological investigations revealed a metabolically active left maxillary lesion with destruction of the maxillary sinus. Histologic examination of a superficial biopsy initially suggested a proliferative fibro-osseous lesion; however, a second deeper biopsy was diagnostic of osteosarcoma, and the patient was referred to a sarcoma center. Immunohistochemistry showed AE1/AE3 and CK(MNF.116) positivity in occasional cells with a Ki67 proliferation index of 60%. This was diagnostic of grade 2-3 osteosarcoma. Multidisciplinary management of the patient included neoadjuvant chemotherapy and total maxillectomy and dental prosthetic rehabilitation. The patient is still under follow-up.
Conclusions: This case of primary osteosarcoma of the maxilla is rare and scarcely reported in the literature. Clinical differential diagnoses include squamous cell carcinoma, and histologic differential diagnosis includes fibro-osseous proliferative lesions in undersampled cases. It is important to consider osteosarcoma in destructive lesions, as it requires prompt and early specialist intervention to maximize the chances of negative surgical margins, which is the mainstay of treatment for this disease for prognosis.

A rare case of isolated laryngeal metastasis 23 years after nephrectomy for clear cell renal carcinoma (2021)

Type of publication:
Journal article

Author(s):
*Eastwood, Michael J ; *Ahsan, Syed F; *Harris, Richard

Citation:
British Journal of Hospital Medicine; Aug 2021; vol. 82 (no. 8); p. 1-3

Abstract:
The article describes the case of isolated laryngeal metastasis 23 years after nephrectomy for clear cell renal
carcinoma in an 84-year-old man.

The experience of implementing a discovery system in a pandemic (2021)

Type of publication:
Journal article

Author(s):
*Curtis, Jason

Citation:
HLG Nursing Bulletin; Jan 2021; vol. 40 (no. 1); p. 6-11

Abstract:
The article explores the transition of Shropshire Health Libraries from a legacy library management system
(OLIB) to a full discovery system using Worldshare Management Services and WorldCat Discovery in 2020.
Topics discussed include the advantages of the discovery system such as access to the WorldCat global catalog
for co-operative cataloguing, project management training given by Online Computer Library Center (OCLC) to
Shropshire, and disadvantages of WorldShare for Shropshire.

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Predictors of in-hospital mortality in Covid-19: A study across two peripheral district general hospitals in UK (2021)

Type of publication:
Journal article

Author(s):
Samanta N.K.; Bandyopadhyay S.K.; *Herman D.; Chakraborty B.; *Marsh A.; *Kumaran S.; *Burnard L.; *Gnanaseelan G.; *Gibson S.; *Florence B.; Ganguly S.

Citation:
British Journal of Medical Practitioners; Jul 2021; vol. 14 (no. 1)

Abstract:
Aim-The mortality from Coronavirus Disease 2019 (COVID-19) has remained a significant medical challenge. Internationally, patient demographics and pre-existing co-morbidities are significant determinants of mortality from COVID-19. The mortality-risk in a local population is difficult to determine. The objective of our study is to examine the risk posed by epidemiological and demographic variables, and co-morbidities in our local population. Method-A retrospective, observational study was conducted on confirmed COVID-19 patients, identified from the local microbiology database. A search of the electronic patient records was performed to collect demographic details and co-morbidities. Chi-square test and logistic regression analysis of the demographic variables and co-morbidities were utilised to calculate the predictive-risk for in-hospital mortality of adult COVID-19 patients. Results-Final analysis included 263 samples. Univariate logistic regression analysis was performed using age as an independent categorical predictor with two cohorts – those <60 and those >=60 years old. Age (2=17.12, p<0.001) was found to be an independent predictor of mortality – this was independent of sex (2=1.784, p<0.182). Charlson Comorbidity Index (CCI) score was found to be a significant predictor of adverse outcome. The odds of death for patients with CCI scores 0-4 was less than half (44.8%) of those with CCI scores >=5 (p=0.005). Patients with no pre-existing medical conditions had a lower mortality-risk (OR=0.181, p=0.022) than those with known medical conditions. Pre-existing renal disease predicted a poor outcome (OR=1.996, p=0.027). The odds of death for the patients coming from their own-home was only 26% of the odds for those from a long-term care-home. Long-term care facility, advanced age (OR=1.058, p <0.001), and long-term oral steroid (OR=3.412, p=0.016) use were all associated with a poor prognosis. Conclusion People aged >=60 years, residence in a long-term care-home, pre-existing renal diseases, a high CCI score and long-term oral steroids use were associated with an increased mortality-risk.

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Upgrading of hospital discharge summary software to optimise COVID-19 documentation and safeguard infection prevention in the community (2021)

Type of publication:
Journal article

Author(s):
*Donati-Bourne J.; *Lo N.; *Selvan M.; *O'dair J.; Mohamed W.; Kasmani Z.

Citation:
British Journal of Medical Practitioners; Jul 2021; vol. 14 (no. 1)

Abstract:
Aims: Early review of 50 discharge summaries at Royal Shrewsbury Hospital (SATH) in April 2020 revealed only 27% documented the patient's in-hospital COVID-19 test result and 2% outlined any recommended self isolation advice following hospital discharge. This had potential adverse implications for community infection control as well as medico-legal sequalae for the Trust were the discharged patient to spread COVID-19 to other cohabitants. The urology team worked with SATH IT to amend the existing discharge summary software, to add two tabs to make COVID-19 test result and self-isolation documentation mandatory for successful sign-off. The aim of this quality improvement project was to evaluate the impact of updating the discharge summary software on documentation accuracy related to COVID-19 on discharge paperwork.
Method(s): Following the implementation of the modified software, 50 consecutive discharge summaries for patients admitted under the urology team starting 1st October 2020 were retrospectively reviewed for documentation of COVID-19 result and self-isolation advice.
Result(s): 90% of discharge summaries included COVID-19 test result and 100% included self-isolation advice for the patient, or alternatively confirmed that no self-isolation was required.
Conclusion(s): This simple modification of an existing IT system greatly improved compliance with COVID-19 discharge summary documentation. We propose all hospitals consider adopting similar measures in the interest of infection prevention, public safety and potential medico-legal sequalae.

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Results of DARS: a randomised phase III trial of dysphagia-optimised intensity modulated radiotherapy (DO-IMRT) versus standard IMRT (S-IMRT) in oropharyngeal (OPC) and hypopharyngeal (HPC) cancer (2021)

Type of publication:
Conference abstract

Author(s):
Nutting C.; Roe J.; Tyler J.; Bhide S.; Rooney K.; Foran B.; *Pettit L.; Beasley M.; Finneran L.; Sydenham M.; Emson M.; Hall E.; Petkar I.; Frogley R.

Citation:
Oral Oncology; Jul 2021; vol. 118 Supplement ; p. 10-11

Abstract:
Presented by: Chris Nutting (Chris.Nutting@rmh.nhs.uk) Introduction Most newly diagnosed OPC & HPC are treated with (chemo)RT with curative intent but at the consequence of adverse effects on quality of life. DARS (ISRCTN:25458988) tested if using DO-IMRT to reduce RT dose to the dysphagia/aspiration related structures (DARS) improved swallowing function compared to S-IMRT. Materials and Methods Patients with T1-4, N0-3, M0 OPC/HPC were randomised 1:1 to S-IMRT (65 Gray (Gy)/30 fractions (f) to primary & nodal tumour; 54 Gy/ 30f to remaining pharyngeal subsite & nodal areas at risk of microscopic disease) or DO-IMRT. The volume of the superior & middle pharyngeal constrictor muscle (PCM) (OPC) or inferior PCM (HPC) lying outside the high-dose target volume was set a mandatory mean dose constraint in DO-IMRT. Treatment allocation was by minimisation balanced by centre, use of induction/concomitant chemotherapy, tumour site & AJCC stage. Primary endpoint was mean MD Anderson Dysphagia Inventory (MDADI) composite score 12 months after RT with 102 patients needed to detect a 10 point improvement (assuming S-IMRT score of 72, standard deviation (SD) 13.8; 90% power, 2-sided 5% alpha). Patients were blind to treatment allocation. Secondary endpoints assessing swallowing function included University of Washington (UW)-Qol & Performance Status Scale Head & Neck (PSS-HN) scores. Results 112 patients (56 S-IMRT, 56 DO-IMRT) were randomised from 22 UK centres from 06/2016 to 04/2018. Mean age was 57 years; 80% were male; 97% had OPC; 90% had AJCC stage 3&4 disease; 86% had concomitant chemotherapy only, 4% induction & concomitant and 10% no chemotherapy. 111/112 had RT doses as prescribed (1 patient died before RT). Median of the mean inferior PCM dose was SIMRT 49.8 Gy (IQR 47.1-52.4) vs. DO-IMRT 28.4 Gy (21.3-37.4), p < 0.0001; superior & middle PCM dose was
S-IMRT 57.2 Gy (56.3-58.3) vs. DO-IMRT 49.7 Gy (49.4-49.9), p < 0.0001. At 12 months, DO-IMRT had significantly higher MDADI scores: S-IMRT mean: 70.5 (SD 17.3) vs. DO-IMRT 77.7 (16.1), p = 0.037. At 12 months the proportion of patients reporting UW-QoL as being able to swallow "as well as ever" was 40.4% for DO-IMRT & 15.2% for S-IMRT; scores of?>50 were reported for PSS-HN normalcy of diet by 70.6% DO-IMRT & 58.1% S-IMRT patients & for eating in public scores by 84.3% DO-IMRT & 74.4% S-IMRT. Conclusions DOIMRT reduced RT dose to the DARS and improved patient reported swallowing function compared with S-IMRT. This is the first randomised study to demonstrate functional benefit of swallow-sparing IMRT in OPC.