Multidisciplinary team approach to diagnosing lymphangioleiomyomatosis (2021)

Type of publication:
Journal article

Author(s):
Okoh, Magnus; Khan, Rosina; *Ahmad, Nawaid

Citation:
BMJ Case Reports; Aug 2021; vol. 14 (no. 8)

Abstract:
A 42-year-old woman with chronic obstructive pulmonary disease was referred to the respiratory team due to shortness of breath on exertion and significant deterioration in pulmonary function tests. Her symptoms were progressively getting worse. This prompted a referral to the specialist team where further investigations were undertaken including a high-resolution CT scan followed by lung biopsy, which eventually revealed a diagnosis of lymphangioleiomyomatosis (LAM). Successful referral to the National LAM Centre in Nottingham provided the key therapeutic approach required to manage this rare condition. Diagnosing this rare condition was due to the multidisciplinary team approach, which involved input from the general practitioner, radiologist and
respiratory consultant. The patient has been making good progress with pharmacological management.

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An unusual presentation of dysarthria in a young patient, a stroke mimic (2021)

Type of publication:
Journal article

Author(s):
*Simpson D.; *David O.; Nasr F

Citation:
Acute Medicine; Jun 2021; vol. 20 (no. 2); p. 140-143

Abstract:
Internal carotid artery dissection commonly affects younger patients. We present a case of a previously fit and well 43-year-old gentleman who presented with a sudden onset of slurring of speech, with right-sided tongue deviation and fasciculation on examination. Signs and symptoms began following participation in a home workout class. Magnetic resonance angiography revealed right-sided extracrainal internal carotid artery dissection leading to right-sided unilateral twelfth cranial nerve palsy.

X Chromosome Contribution to the Genetic Architecture of Primary Biliary Cholangitis (2021)

Type of publication:
Journal article

Author(s):
Asselta R.; Paraboschi E.M.; Cardamone G.; Duga S.; Gerussi A.; Ciaccio A.; Cristoferi L.; D'Amato D.; Malinverno F.; Mancuso C.; Massironi S.; Milani C.; O'Donnell S.E.; Ronca V.; Barisani D.; Carbone M.; Invernizzi P.; Cordell H.J.; Mells G.F.; Sandford R.N.; Jones D.E.; Nakamura M.; Ueno K.; Tokunaga K.; Hitomi Y.; Kawashima M.; Nishida N.; Kawai Y.; Kohn S.-S.; Nagasaki M.; Gervais O.; Tanaka A.; Takikawa H.; Tang R.; Xiong M.; Li Z.; Shi Y.; Liu X.; Hirschfield G.; Siminovitch K.A.; Gershwin M.E.; Seldin M.F.; Walker E.; Xie G.; Mason A.; Myers R.; Peltekian K.; Ghent C.; Atkinson E.; Juran B.; Lazaridis K.; Lu Y.; Gu X.; Jing K.; Amos C.; Affronti A.; Brunetto M.; Coco B.; Spinzi G.; Elia G.; Ferrari C.; Lleo A.; Muratori L.; Muratori P.; Portincasa P.; Colli A.; Bruno S.; Colloredo G.; Azzaroli F.; Andreone P.; Bragazzi M.; Alvaro D.; Cardinale V.; Cazzagon N.; Rigamonti C.; Floreani A.; Rosina F.; Lampertico P.; Donato F.; Fagiuoli S.; Almasio P.L.; Giannini E.; Cursaro C.; Colombo M.; Valenti L.; Miele L.; Andriulli A.; Niro G.A.; Grattagliano I.; Morini L.; Casella G.; Vinci M.; Battezzati P.M.; Crosignani A.; Zuin M.; Mattalia A.; Calvaruso V.; Colombo S.; Benedetti A.; Marzioni M.; Galli A.; Marra F.; Tarocchi M.; Picciotto A.; Morisco F.; Fabris L.; Croce L.S.; Tiribelli C.; Toniutto P.; Strazzabosco M.; Ch'ng C.L.; Thomas C.; Rahman M.; Yapp T.; Sturgess R.; Harrison M.; Healey C.; Galaska R.; Czajkowski M.; Kendall J.; Whiteman J.; Gunasekera A.; Lawlor C.; Gray C.; Gyawali P.; Premchand P.; Kapur K.; Elliott K.; Marley R.; Foster G.; Watson A.; Dias A.; Subhani J.; Harvey R.; McCorry R.; Ramanaden D.; Gasem J.; Mulvaney-Jones C.; Hobson L.; Evans R.; Mathialahan T.; Shorrock C.; Van Duyvenvoorde G.; Lipscomb G.; Loftus A.; Southern P.; Seward K.; Tibble J.; Gorard D.; Penn R.; Palegwala A.; Maiden J.; Damant R.; Jones S.; Dawwas M.; Alexander G.; Dolwani S.; Cloudsdale R.; Prince M.; Foxton M.; Silvestre V.; Elphick D.; Glenn S.; Mitchison H.; Dungca E.; Gooding I.; Wheatley N.; Karmo M.; Doyle H.; Saksena S.; Kent M.; Mendall M.; Patel M.; Hamilton C.; Braim D.; Ede R.; Austin A.; Paton A.; Sayer J.; Lancaster N.; Hankey L.; Hovell C.; Fisher N.; Carter M.; Desousa P.; Koss K.; Piotrowicz A.; Muscariu F.; Musselwhite J.; Grimley C.; Neal D.; Lim G.; Tan L.; Levi S.; Ala A.; Broad A.; Saeed A.; Wood G.; Flahive K.; Brown J.; Nambela E.; Townshend P.; Ford C.; Holder S.; Wilkinson M.; Gordon H.; Palmer C.; Ramage J.; Ridpath J.; Featherstone J.; Ngatchu T.; Grover B.; Nasseri M.; Shaukat S.; Shidrawi R.; Sadeghian J.; Abouda G.; Ali F.; Rolls S.-A.; Rees I.; Salam I.; Narain M.; Brown A.; Crossey M.; Taylor-Robinson S.; Williams S.; Stansfield G.; MacNicol C.; Grellier L.; Wilkins J.; Banim P.; Das D.; Chilton A.; Raymode P.; Heneghan M.; Lee H.-J.; Curtis H.; Gess M.; Drake I.; Durant E.; Aldersley M.; Davies M.; Jones R.; Bishop R.; McNair A.; Srirajaskanthan R.; Pitcher M.; Tripoli S.; Sen S.; Bird G.; Casey R.; Barnardo A.; Kitchen P.; Cowley C.; Yoong K.; Miller R.; Chirag O.; Sivaramakrishnan N.; MacFaul G.; Jones D.; Shah A.; Wright F.; Evans C.; Saha S.; Pollock K.; Bramley P.; Mukhopadhya A.; Fraser A.; Williams D.; Mills P.; Shallcross C.; Campbell S.; Bathgate A.; Shepherd A.; Dillon J.; Rushbrook S.; Przemioslo R.; Macdonald C.; Metcalf J.; Shmueli U.; Davis A.; Naqvi A.; Lee T.; Ryder S.D.; Collier J.; Klass H.; Kent L.; Ninkovic M.; Cramp M.; Sharer N.; Aspinall R.; Goggin P.; Ghosh D.; Douds A.; Hoeroldt B.; Booth J.; Williams E.; Gunter E.; Dewhurst H.; Hussaini H.; Stableforth W.; Ayres R.; Thorburn D.; Marshall E.; Burroughs A.; Mann S.; Lombard M.; Richardson P.; Patanwala I.; Maltby J.; Brookes M.; Mathew R.; Vyas S.; Singhal S.; Gleeson D.; Misra S.; *Butterworth J.; George K.; Harding T.; Douglass A.; Tregonning J.; Panter S.; Sanghi P.; Shearman J.; Bray G.; Butcher G.; Forton D.; Mclindon J.; Cowan M.; Whatley G.; Mandal A.; Gupta H.; Jain S.; Pereira S.; Prasad G.; Watts G.; Wright M.; Neuberger J.; Gordon F.; Unitt E.; Grant A.; Delahooke T.; Higham A.; Brind A.; Cox M.; Ramakrishnan S.; King A.; Collins C.; Whalley S.; Li A.; Fraser J.; Bell A.; Hughes M.; Wong V.S.; Singhal A.; Gee I.; Ang Y.; Ransford R.; Gotto J.; Millson C.; Bowles J.; Hails J.; Wooldridge H.; Abrahams R.; Gibbins A.; Hogben K.; McKay A.; Foale C.; Brighton J.; Williams B.; Hynes A.; Duggan C.; Wilhelmsen E.; Ncube N.; Houghton K.; Ducker S.; Bird B.; Baxter G.; Keggans J.; Grieve E.; Young K.; Ocker K.; Hines F.; Martin K.; Innes C.; Valliani T.; Fairlamb H.; Thornthwaite S.; Eastick A.; Tanqueray E.; Morrison J.; Holbrook B.; Browning J.; Walker K.; Congreave S.; Verheyden J.; Slininger S.; Stafford L.; O'Donnell D.; Ainsworth M.; Lord S.; March L.; Dickson C.; Simpson D.; Longhurst B.; Hayes M.; Shpuza E.; White N.; Besley S.; Pearson S.; Wright A.; Jones L.; Fouracres A.; Farrington L.; Graves L.; Marriott S.; Leoni M.; Tyrer D.; Dalikemmery L.; Lambourne V.; Green M.; Sirdefield D.; Amor K.; Orpe J.; Colley J.; Shinder B.; Jones J.; Mills M.; Carnahan M.; Taylor N.; Boulton K.; Brown C.; Clifford G.; Archer E.; Hamilton M.; Curtis J.; Shewan T.; Walsh S.; Warner K.; Netherton K.; Mupudzi M.; Gunson B.; Gitahi J.; Gocher D.; Batham S.; Pateman H.; Desmennu S.; Conder J.; Clement D.; Gallagher S.; Chan P.; Currie L.; O'Donohoe L.; Oblak M.; Morgan L.; Quinn M.; Amey I.; Baird Y.; Cotterill D.; Cumlat L.; Winter L.; Greer S.; Spurdle K.; Allison J.; Dyer S.; Sweeting H.; Kordula J.; Aiba Y.; Nakamura H.; Abiru S.; Nagaoka S.; Komori A.; Yatsuhashi H.; Ishibashi H.; Ito M.; Migita K.; Ohira H.; Katsushima S.; Naganuma A.; Sugi K.; Komatsu T.; Mannami T.; Matsushita K.; Yoshizawa K.; Makita F.; Nikami T.; Nishimura H.; Kouno H.; Ota H.; Komura T.; Nakamura Y.; Shimada M.; Hirashima N.; Komeda T.; Ario K.; Nakamuta M.; Yamashita T.; Furuta K.; Kikuchi M.; Naeshiro N.; Takahashi H.; Mano Y.; Tsunematsu S.; Yabuuchi I.; Shimada Y.; Yamauchi K.; Sugimoto R.; Sakai H.; Mita E.; Koda M.; Tsuruta S.; Kamitsukasa H.; Sato T.; Masaki N.; Kobata T.; Fukushima N.; Higuchi N.; Ohara Y.; Muro T.; Takesaki E.; Takaki H.; Yamamoto T.; Kato M.; Nagaoki Y.; Hayashi S.; Ishida J.; Watanabe Y.; Kobayashi M.; Koga M.; Saoshiro T.; Yagura M.; Hirata K.; Zeniya M.; Abe M.; Onji M.; Kaneko S.; Honda M.; Arai K.; Arinaga-Hino T.; Hashimoto E.; Taniai M.; Umemura T.; Joshita S.; Nakao K.; Ichikawa T.; Shibata H.; Yamagiwa S.; Seike M.; Honda K.; Sakisaka S.; Takeyama Y.; Harada M.; Senju M.; Yokosuka O.; Kanda T.; Ueno Y.; Kikuchi K.; Ebinuma H.; Himoto T.; Yasunami M.; Murata K.; Mizokami M.; Shimoda S.; Miyake Y.; Takaki A.; Yamamoto K.; Hirano K.; Ichida T.; Ido A.; Tsubouchi H.; Chayama K.; Harada K.; Nakanuma Y.; Maehara Y.; Taketomi A.; Shirabe K.; Soejima Y.; Mori A.; Yagi S.; Uemoto S.; Tanaka T.; Yamashiki N.; Tamura S.; Sugawara Y.; Kokudo N.

Citation:
Gastroenterology; Jun 2021; vol. 160 (no. 7); p. 2483

Abstract:
Background & Aims: Genome-wide association studies in primary biliary cholangitis (PBC) have failed to find X chromosome (chrX) variants associated with the disease. Here, we specifically explore the chrX contribution to PBC, a sexually dimorphic complex autoimmune disease. Method(s): We performed a chrX-wide association study, including genotype data from 5 genome-wide association studies (from Italy, United Kingdom, Canada, China, and Japan; 5244 case patients and 11,875 control individuals). Result(s): Single-marker association analyses found approximately 100 loci displaying P < 5 x 10-4, with the most significant being a signal within the OTUD5 gene (rs3027490; P = 4.80 x 10-6; odds ratio [OR], 1.39; 95% confidence interval [CI], 1.028-1.88; Japanese cohort). Although the transethnic meta-analysis evidenced only a suggestive signal (rs2239452, mapping within the PIM2 gene; OR, 1.17; 95% CI, 1.09-1.26; P = 9.93 x 10-8), the population-specific meta-analysis showed a genome-wide significant locus in East Asian individuals pointing to the same region (rs7059064, mapping within the GRIPAP1 gene; P = 6.2 x 10-9; OR, 1.33; 95% CI, 1.21-1.46). Indeed, rs7059064 tags a unique linkage disequilibrium block including 7 genes: TIMM17B, PQBP1, PIM2, SLC35A2, OTUD5, KCND1, and GRIPAP1, as well as a superenhancer (GH0XJ048933 within OTUD5) targeting all these genes. GH0XJ048933 is also predicted to target FOXP3, the main T-regulatory cell lineage specification factor. Consistently, OTUD5 and FOXP3 RNA levels were up-regulated in PBC case patients (1.75- and 1.64-fold, respectively). Conclusion(s): This work represents the first comprehensive study, to our knowledge, of the chrX contribution to the genetics of an autoimmune liver disease and shows a novel PBC-related genome-wide significant locus.

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COVID-19 disease and cardiac involvement-a local experience (2021)

Type of publication:
Conference abstract

Author(s):
*Ahmed M.R.; *Islam S.; *Challinor E.; *Ingram T.; *Khan A.

Citation:
Heart; Jun 2021; vol. 107

Abstract:
Aims The aim of this review to assess cardiac involvement in patients with severe COVID-19 patients. We review all patients with COVID 19 disease admitted in our trust requiring transthoracic echocardiograms on their clinical indications. Background Cardiac involvement in COVID-19 disease has been found to be prognostic factor and has been related with higher mortality and morbidity. In a large series with COVID-19 those with heart disease had a fatality rate around 10.5%.1 2 Methods All adult patients who were COVID-19 positive on PCR admitted between March 2020 and February 2021, who had an echocardiogram, were identified through our local database. Their demographics, co-morbid, troponin levels and Pro NT-BNP were analysed. All echocardiograms reports which were finalised by the imaging cardiologist were included in our analysis. Results There were a total of 41 patients who had echocardiograms during their stay in the hospital with COVID-19 disease. Mean age was 70 (range 45-90) years old. There were 70% male and 30% female patients. 12% were diabetic, 49% hypertensive and 40% had previous heart disease. Pulmonary embolism diagnosed in 10% of patients by CT pulmonary angiogram. 56% of patients required high flow oxygen and 21% need mechanical ventilation. Almost all patients had troponin and CRP levels on admission. Mean troponin level 215 and mean CRP levels were 197. Mean D dimer levels 1130, and mean creatinine levels were 138. 92% had evidence of lung involvement in chest X-ray. 13% patients had new evidence of a diagnosis of left ventricular dysfunction on echocardiography. Similarly, 27% had a new diagnosis of right ventricular dysfunction. Mean left ventricular diastolic dimension were 4.6 cm and systolic dimension. 2% had echo diagnosis of left ventricular thrombus echocardiographic studies. Mean PA pressure on echocardiography were 35 mmHg and mean E/A ratio was 1.2. 17% of patients were found to have pericardial effusion but none causing haemodynamic compromise. Conclusion This data suggests high incidence of right and left ventricular involvement in patients with severe COVID-19 disease. We recommend that all patients with COVID-19 disease admitted to hospital and requiring oxygen should have transthoracic echocardiograms during their admission.

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Non-contrast MRI for assessment of thoracic aorta dimension (2021)

Type of publication:
Conference abstract

Author(s):
*Gupta M.; *Ingram T.; *Clarke H.; *Pakala V.; *Lee E.; Hargreaves O.; *Otun J.

Citation:
Heart; Jun 2021; vol. 107

Abstract:
Introduction Multi-modality imaging plays a significant role in evaluating and interval monitoring of patients with aortopathies. Echocardiogram is the first screening test followed by Computerised Tomography (CT) and/ or Magnetic Resonance Imaging (MRI). Most patients require repeated scans at interval. Both CT and MRI require contrast administration and furthermore, radiation exposure in CT. Locally, we have c adopted surveillance scanning with non-contrast MR to overcome the above limitations. This is not widely practised. Aim The aim of the study is to compare inter-modality agreement between CT (gold standard) and non-contrast MRI measurements of ascending aortic dimensions. Methods 126 consecutive patients underwent non-contrast
MRI thoracic aorta our hospitals between 2017 and 2021. Thirty-eight patients (61% males, age 61+/-14 years) have had both CT and MRI. A retrospective analysis was conducted to assess the inter-modality agreement of ascending aorta measurements. Statistical analysis was done using R programme (R studio). A Bland-Altman graph was used to assess inter-modality agreement of ascending aorta measurements. Differences in measurements of the two modalities were reported as mean and 95% confidence interval. Results There is good linear correlation (Pearson's R=0.86, p<0.05) between CT and MRI measurements. Mean difference between CT and MRI measurements was 2.39mm, 95% confidence interval 6.5mm to 8.4mm, see figure 1. Conclusion
There is good inter-modality agreement of ascending aorta measurements between CT non contrast MRI in our experience. Non contrast MRI has the advantage of requiring no radiation and no need for contrast. This is desirable particularly in young patients requiring long term surveillance.

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Suitability for low-dose rivaroxaban based on compass trial: A district general hospital perspective (2021)

Type of publication:
Conference abstract

Author(s):
*Asad M.; *Irfan Kazi S.; *Makan J.; *Gupta M.; Alaguraja P.; McCaughey D

Citation:
Heart; Jun 2021; vol. 107

Abstract:
Introduction COMPASS trial has recommended that low-dose rivaroxaban reduces major adverse cardiac and limb events among patients with stable atherosclerotic vascular disease. In the real-world practice, the recommendations from COMPASS trial can be used as a standard to recognise potentially suitable patients. The objective of our study was to establish the cohort of patients identified as COMPASS-eligible for low dose rivaroxaban. Methods A health service evaluation of Cardiology Outpatients from Shrewsbury and Telford Hospital NHS Trust (SaTH) was carried out. The specific characteristics of the selected cohort included known stable atherosclerotic vascular disease while the inclusion and exclusion criteria incorporated in the COMPASS
trial was used as a standard. The SaTH clinical databases from January 2021 were utilized to conduct a retrospective analysis to identify patients who could prospectively benefit from low-dose rivaroxaban. Results Among the 99 patients who were found to have stable atherosclerotic vascular disease, 34 patients were deemed eligible for low dose rivaroxaban. Patients in our COMPASS-eligible group included 26 patients who were >=65years of age while 8 patients were noted to be <65 years of age. Further analysis revealed that 94% of the patients had coronary artery disease as compared with only 6% found to have peripheral artery disease. In this cohort of patients, 79 % of the non-eligible patients were excluded due to underlying atrial fibrillation. Conclusion About one-third of our cohort of patients met the COMPASS criteria and could potentially benefit from low dose rivaroxaban therapy. There is certainly a strong mandate for introduction of rivaroxaban
following the COMPASS trial recommendations. Local protocols should be established to ensure that this window of opportunity to prevent major adverse cardiovascular and limb events is not missed in the clinical practice.

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Emergency management of neck stoma patients during the coronavirus pandemic: a national nurse survey (2021)

Type of publication:
Journal article

Author(s):
Senior A.; *Chan J.; Brookes K.; *Jolly K.; *Darr A.; *Ameen R.

Citation:
British Journal of Nursing; Jun 2021; vol. 30 (no. 12); p. 742-746

Abstract:
BACKGROUND: Neck stoma patient care involves significant clinical complexity. Inadequate staff training, equipment provision and infrastructure have all been highlighted as causes for avoidable patient harm.
AIMS: To establish the perception of knowledge and confidence levels relating to the emergency management of neck stomas among UK nurses during the COVID-19 pandemic.
METHOD(S): A nationwide prospective electronic survey of both primary and secondary care nurses via the Royal College of Nursing and social media. FINDINGS: 402 responses were collated: 81 primary care and 321 secondary care; the majority (n=130) were band 5. Forty-nine per cent could differentiate between a laryngectomy and a tracheostomy; ENT nurses scored highest (1.56; range 0-2) on knowledge. Fifty-seven per cent could oxygenate a tracheostomy stoma correctly and 54% could oxygenate a laryngectomy stoma correctly. Sixty-five per cent cited inadequate neck stoma training and 91% felt inclusion of neck stoma training was essential within the nursing curriculum.
CONCLUSION(S): Clinical deficiencies of management identified by nurses can be attributed to a lack of confidence secondary to reduced clinical exposure and education.

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Effective implementation of an advanced clinical practitioner role in breast imaging (2021)

Type of publication:
Conference abstract

Author(s):
*Deane L.; *Williams S.; *Cielecki L.; *Burley S.

Citation:
Breast Cancer Research 2021, 23(Suppl 1):P57

Abstract:
Background: Due to the immense pressure to provide capacity for women with breast symptoms, to be seenĀ  within two weeks, a new innovative role has been created to provide increased capacity. Introduction: The breast services see many women with conditions that are benign and easily identified upon ultrasound. The majority of these conditions occur in women under the age of 40years. The role of an advanced clinical practitioner was created to answer a service need. This role requires a highly specialised cohort of skills combining breast image interpretation, breast ultrasound and breast biopsying alongside a range of clinical competences enabling autonomous practice within clear governance.
Method(s): A new clinic was created for under 40 aged women only requiring only a breast clinical specialist and an advanced clinical practitioner, using ultrasound for assessment. Unexpected findings suspicious upon ultrasound-would be redirected to the next consultant led clinic for full imaging assessment and biopsy.
Result(s): Increased capacity was achieved, without increased costs. Anxiety levels were reduced due to these patients seen within these clinics and more specialist skills could be directed to more complex cases in the traditional cancer clinics.
Conclusion(s): The use of this specialist role has proven to be innovative and specialised in answering capacity issues within the workforce. The ACP role is utilised as a support to all clinics working alongside consultant radiographers as well as in an autonomous role, thereby freeing up the consultants for cases requiring specialist skills. The stability of the breast service has been ensured

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Male breast ultrasound: 2019 audit results (2021)

Type of publication:
Conference abstract

Author(s):
*Ozcan U.A.; *Williams S.; *Metelko M

Citation:
Breast Cancer Research 2021, 23(Suppl 1):O3.3

Abstract:
Background and Purpose: Male breast cancer is rare whereas gynaecomastia is very common. Only asymmetrical gynaecomastia require breast imaging and focal lumps are amenable to clinical core biopsy. So the use of ultrasound in the assessment of male breast should be limited. The aim of this study is to audit the referral indications and ultrasound outcomes in male breast US (MBUS) patients against local guidelines.
Method(s): In the last 5 years, 968 patients were referred for MBUS in our Trust. This audit includes the patients between 02/01/2019-04/12/2019. The duplicate patients and follow-ups were excluded from the study. In total, 197 patients were analysed (mean age: 58 (8-90) retrospectively. Referral diagnosis, age, US grading and clinical outcomes were noted.
Result(s): Of the 197 patients, 79% were gynecomastia (133), lipoma (21) or fat necrosis (2), and 15% (30) were normal. There was 1 chest wall lymphoma and 1 DCIS, and 9 (5%) patients had benign breast disease (fibroepithelial lesions, abscess, papilloma, sebaceous cysts, haematoma). In 122 patients (62%) clinical grade was not given, 66 had P2, 8 had P3, 1 had P5. 2 patients were scored as U4 and 4 patients as U3.
Conclusion(s): These results clearly show that 99% of the patients referred to MBUS were benign. And also 95% of the patients were clinically benign or not assessed. The excessive use of MBUS without a clinical indication leads to patient anxiety, increased waiting times and might delay the proper imaging to the patients who should have the priority in terms of clinical indication. Careful clinical assessment before ultrasound referral is mandatory for better care.

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