Recent Submissions

  • Rare Cutaneous Manifestations of Erdheim Chester Disease: A Case Report and Literature Review.

    Vasandani, Nikhil; Low, Jing Er; Liau, Yun Hui; Ergun, Alexander; Balakrishnan, Theogren (2023-06-20)
    Erdheim Chester disease (ECD) is a rare and complex non-Langerhans histiocytic systemic disease that affects multiple organ systems, including the bones, heart, lungs, and central nervous system. Fewer than 1,000 cases have been reported in the medical literature and dermatological manifestations of the disease are rare but can provide valuable diagnostic clues for this challenging disease. The cutaneous manifestations of ECD can take many forms, including nodules, plaques, papules, and xanthomas. These lesions can occur on any part of the body and may be solitary or multiple. Cutaneous manifestations of ECD have been reported to occur in up to 20% of cases, but the true prevalence may be higher, as many cases may go undiagnosed. We present the case of a 62-year-old gentleman with a history of ECD currently on vemurafenib who presented with multiple painless subcutaneous nodules on his back after an excision biopsy under local anesthetic revealed histological features of ECD. The objective of this case report is to raise awareness of ECD and its dermatological manifestations. Further research is warranted to better understand the pathogenesis and morphology of cutaneous involvement in ECD.
  • Preoperative Breast Magnetic Resonance Imaging as a Predictor of Response to Neoadjuvant Chemotherapy.

    McAnena, Peter; O'Halloran, Niamh; Moloney, Brian M; Crilly, Emily; Kerin, Michael J; Lowery, Aoife J; Browne, Robert (2022-06-24)
    Introduction: The ability to accurately predict pathologic complete response (pCR) after neoadjuvant chemotherapy (NAC) in breast cancer would improve patient selection for specific treatment strategies, would provide important information for patients to aid in the treatment selection process, and could potentially avoid the need for more extensive surgery. The diagnostic performance of magnetic resonance imaging (MRI) in predicting pCR has previously been studied, with mixed results. Magnetic resonance imaging performance may also be influenced by tumour and patient factors. Methods: Eighty-seven breast cancer patients who underwent NAC were studied. Pre-NAC and post-NAC MRI findings were compared with pathologic findings postsurgical excision. The impact of patient and tumour characteristics on MRI accuracy was evaluated. Results: The mean (SD) age of participants was 48.7 (10.3) years. The rate of pCR based on post-NAC MRI was 19.5% overall (19/87). The sensitivity, specificity, positive predictive value (PPV), negative predictive value, and accuracy in predicting pCR were 52.9%, 77.1%, 36.0%, 87.1%, and 72.4%, respectively. Positive predictive value was the highest in nonluminal versus Luminal A disease (45.0% vs 25.0%, P < .001), with higher rates of false positivity in nonluminal subtypes (P = .002). Tumour grade, T category, and histological subtype were all independent predictors of MRI accuracy regarding post-NAC tumour size. Conclusion: Magnetic resonance imaging alone is insufficient to accurately predict pCR in breast cancer patients post-NAC. Magnetic resonance imaging predictions of pCR are more accurate in nonluminal subtypes. Tumour grade, T category, and histological subtype should be considered when evaluating post-NAC tumour sizes.
  • Caplacizumab Use in a TTP Case Unresponsive to Conventional Therapy.

    William, J; McCellistrim, C; Nuñez, Y (2022-09-15)
    Introduction Thrombotic Thrombocytopenic Purpura (TTP) is a rare but life-threatening disorder caused by severely reduced activity of ADAMTS13, causing platelet adhesion and formation of small-vessel platelet-rich thrombi, thrombocytopenia, and microangiopathic haemolytic anaemia. Diagnosis A 48-year-old female presented with acute generalized petechial rash, bruises, and fatigue. Bloods revealed thrombocytopenia, anaemia, 10% schistocytes. Her plasmic score was seven, and ADAMT13 was &lt;5. Treatment Patient initially responded to plasma exchange and steroids, but thrombocytopenia recurred on day six of treatment, needing the addition of further immunosuppressive drugs and Caplacizumab. Conclusion TTP cases unresponsive to conventional regimens can represent a challenging situation; however, poor outcomes could potentially be avoided with a novel therapy like Caplacizumab. In our patient, this medication was well tolerated, and platelet count normalized after two days of its introduction.
  • A Surgical Presentation of Churg-Strauss Syndrome.

    Vasandani, Nikhil; Isaac, Martha; Bajwa, Amrit; Sheehan, Margaret; Nugent, Emmeline (2022-04-21)
    Case presentation: Eosinophilic granulomatosis with polyangiitis (EGPA) or Churg-Strauss syndrome (CSS) is a rare, autoimmune vasculitis usually affecting small and medium-sized blood vessels in its later phases. It is a diffuse, systemic, multisystem disease that is reported to present with gastrointestinal manifestations but very rarely as an acute abdomen secondary to eosinophilic peritonitis. A 28-year-old relatively healthy male with a pre-existing diagnosis of inactive pulmonary sarcoidosis presented to the emergency department with an acute abdomen. After an exploratory laparotomy, multi-specialty involvement, and extensive investigations to exclude other differentials, a diagnosis of EGPA was made. The patient was treated with systemic glucocorticoids initially, followed by a tapering course of steroids and anti-interleukin 5 monoclonal antibodies as maintenance upon remission. EGPA can manifest in a myriad of ways including an acute abdomen, and medical treatment is useful in managing this presentation. Surgeons should be aware of the atypical causes of acute abdomen and should routinely broaden their differential diagnosis to include medical pathologies.
  • Stylohyoid Eagle syndrome and EXTracranial INternal Carotid arTery pseudoaneurysms (EXTINCT) with internal jugular vein nutcracker syndrome: a challenging clinical scenario.

    Sultan, Sherif; Acharya, Yogesh; Soliman, Osama; Hynes, Niamh (2022-04-21)
    Case report: We report a 4-year delay in diagnosing a combined carotid arterial and jugular venous styloid compression. The symptoms, which included dull neck pain, dizziness, intermittent diplopia, tinnitus, severe incapacitating right side headache and eye bloating, were challenging and wrongly attributed initially to various facial neuralgias. The patient presented during COVID-19 pandemic and was labelled as ‘carotidynia’ first and later as a transient perivascular inflammation of carotid artery syndrome. Combined targeted duplex ultrasonography and CT angiography with 3D reconstruction revealed a long styloid process and its tendinous-ligamentous attachments, injuring the internal carotid artery. Moreover, there was substantial internal jugular vein compression on a long C1 transverse process with a nutcracker syndrome. Release of the tendinous portion of the long styloid process and repair of the carotid artery pseudoaneurysm ended the patient’s complaints and allowed him to have a better quality of life.
  • Instrumentation failure during microlaryngoscopy.

    van den Berg, Nadia; Corbett, Mel; Cleere, Eoin; Keogh, Ivan (2022-04-25)
    Equipment failure can be a cause of morbidity during surgical procedures. We present two cases where a broken surgical instrument, a heart-shaped curved micro-grasper, colloquially termed ‘sweetheart’ micro-forceps, compromised patient safety during microlaryngoscopy. We discuss the importance of thorough safety protocols and communication between team members to mitigate this risk. Microinstruments used during microlaryngoscopy are at risk of failure or breakage due to their small size. Surgeons must be alert to the risk of equipment failure during surgery as a potential cause of patient morbidity. In our patient, loss of the broken instrument in the respiratory tract could have resulted in iatrogenic aspiration, respiratory infection and would have necessitated rigid bronchoscopy for removal. Instruments and equipment must checked and communicated by the surgical team before and after use to prevent potential patient morbidity.
  • Candida glabrata infection of a pancreatic pseudocyst in a COVID-19 patient: A case report and review of the literature.

    Khan, Mohammad Aasim; Almas, Talal; Ullah, Muneeb; Alkhattab, Maha; Shaikh, Fathema; Shaikh, Sufyan; Bagwe, Isha; Antony, Meetty; Khedro, Tarek; Nagarajan, Vikneswaran Raj; et al. (2022-04-19)
    Introduction Pancreatic pseudocysts remain a feared complication of acute or chronic pancreatitis and are often characterized by collections of fluids due to underlying damage to the pancreatic ducts, culminating in a walled-off region bereft of an epithelial layer but surrounded by granulation tissue. While fungal infections of pancreatic pseudocysts are rarely encountered, candida albicans remains the most frequently implicated organism. Case presentation A 55-year-old male presented with pain in the left-hypochondriac region, accompanied by non-bilious emesis and nausea. Interestingly, the patient also tested positive for a COVID-19 infection. Investigative workup divulged enhancing pancreatic walls with a radiologic impression consistent with a pancreatic pseudocyst. An ultrasound-guided external drainage was performed; the drainage was conducted unremarkably, with the resultant fluid collection revealing the presence of Candida Glabrata. The patient was commenced on antifungal therapy and continues to do well to date. Discussion Infectious ailments of pancreatic pseudocysts remain a widely known complication of acute pancreatitis. While it is rare, fungal infection is a crucial consideration for patients with pancreatic pseudocysts, especially in the context of a lack of an adequate response to antibiotics, deterioration, comorbidities, and immunocompromised states. Conclusion Rapid identification of the microbe responsible for pancreatic pseudocyst infection is vital for time-sensitive treatment and a more rapid recovery, curbing associated morbidity and mortality.
  • Perioperative Levosimendan Infusion in Patients With End-Stage Heart Failure Undergoing Left Ventricular Assist Device Implantation.

    Abdelshafy, Mahmoud; Elsherbini, Hagar; Elkoumy, Ahmed; Simpkin, Andrew J; Elzomor, Hesham; Caliskan, Kadir; Soliman, Osama (2022-04-28)
    Left ventricular assist device (LVAD) therapy has been instrumental in saving lives of patients with end-stage heart failure (HF). Recent generation devices have short-to-mid-term survival rates close to heart transplantation. Unfortunately, up to 1 in 4 patients develop a life-threatening right-sided HF (RHF) early post LVAD implantation, with high morbidity and mortality rate, necessitating prolonged ICU stay, prolonged inotropic support, and implantation of a right-ventricular assist device. Pre-operative optimization of HF therapy could help in prevention, and/or mitigation of RHF. Levosimendan (LEVO) is a non-conventional inotropic agent that works by amplifying calcium sensitivity of troponin C in cardiac myocytes, without increasing the intra-cellular calcium or exacerbating ischemia. LEVO acts as an inodilator, which reduces the cardiac pre-, and after-load. LEVO administration is associated with hemodynamic improvements. Despite decades long of the use of LVAD and more than two decades of the use of LEVO for HF, the literature on LEVO use in LVAD is very limited. In this paper, we sought to conduct a systematic review to synthesize evidence related to the use of LEVO for the mitigation and/or prevention of RHF in patients undergoing LVAD implantation.
  • Dermatofibrosarcoma protuberans of the scalp.

    Al-Khattab, Maha; Kennedy, Sharon; Jones, Deirdre (2022-04-26)
    Dermatofibrosarcoma protuberans is a rare entity. Due to its high propensity for local recurrence, knowledge of the appropriate management, both surgical and medical, is important for optimal patient outcomes.
  • Keeping an Eye on Bisphosphonate Therapy in Myeloma: A Case Report of Ocular Inflammation Postzoledronic Acid Infusion.

    Faryal, Rehman; Hayat, Amjad (2021-02-13)
    Bisphosphonates have evolved over the past decades from oral to more potent intravenous preparations. Along with significant paradigm shift in the management of myeloma over the past years, stronger nitrogen-containing bisphosphonates, due to their antiresorptive action on the bones, have found their way as a key and integral part in the management of bone disease in myeloma. Multiple randomized controlled trials have established efficacy of bisphosphonates in reducing skeletal-related events in myeloma. Some well-documented adverse events include acute-phase reactions, esophageal irritation, and osteonecrosis of the jaw. Across all clinical indications, the incidence of inflammatory eye reactions after bisphosphonate infusion ranges from 0.046% to 1%. However, data from myeloma patients are extrapolated from few reported cases in literature with varying management strategies including discontinuation, switching to different forms, and rechallenging with steroid cover. Inflammatory eye reactions can vary from self-limiting conjunctivitis and episcleritis to serious uveitis and vision-threatening orbital inflammation. We present a similar case of a patient with IgG kappa myeloma who developed flu-like symptoms followed by severe orbital inflammation within 48-72 hours after receiving zoledronic acid infusion. The patient was successfully managed with intravenous methyl prednisolone followed by oral tapering dose of steroids and discontinuation of further bisphosphonate therapy. A complete recovery was noted in a week's time.
  • Community Occupational Therapy for people with dementia and family carers (COTiD-UK) versus treatment as usual (Valuing Active Life in Dementia [VALID]) study: A single-blind, randomised controlled trial.

    Wenborn, Jennifer; O'Keeffe, Aidan G; Mountain, Gail; Moniz-Cook, Esme; King, Michael; Omar, Rumana Z; Mundy, Jacqueline; Burgess, Jane; Poland, Fiona; Morris, Stephen; et al. (2021-01-04)
    We aimed to estimate the clinical effectiveness of Community Occupational Therapy for people with dementia and family carers-UK version (Community Occupational Therapy in Dementia-UK version [COTiD-UK]) relative to treatment as usual (TAU). We hypothesised that COTiD-UK would improve the ability of people with dementia to perform activities of daily living (ADL), and family carers' sense of competence, compared with TAU.
  • Human Monocyte Subset Distinctions and Function: Insights From Gene Expression Analysis.

    Cormican, Sarah; Griffin, Matthew D (2020-06-04)
    Monocytes are a highly plastic innate immune cell population that displays significant heterogeneity within the circulation. Distinct patterns of surface marker expression have become accepted as a basis for distinguishing three monocyte subsets in humans. These phenotypic subsets, termed classical, intermediate and nonclassical, have also been demonstrated to differ in regard to their functional properties and disease associations when studied in vitro and in vivo. Nonetheless, for the intermediate monocyte subset in particular, functional experiments have yielded conflicting results and some studies point to further levels of heterogeneity. Developments in genetic sequencing technology have provided opportunities to more comprehensively explore the phenotypic and functional differences among conventionally-recognized immune cell subtypes as well as the potential to identify novel subpopulations. In this review, we summarize the transcriptomic evidence in support of the existence of three separate monocyte subsets. We also critically evaluate the insights into subset functional distinctions that have been garnered from monocyte gene expression analysis and the potential utility of such studies to unravel subset-specific functional changes which arise in disease states.
  • Mixed infected laryngocoele presenting as airway obstruction: a case report.

    James, Danielle L; Garry, Stephen; Corbett, Mel; Lang, John (2021-02-28)
    Laryngocoele is a rare entity, defined as an abnormal cystic dilatation of saccule of the laryngeal ventricle. Three types of laryngocele have been described, based on their relation to the thyrohyoid membrane: internal, external or mixed type. Symptoms are variable, including neck swelling, shortness of breath, dysphonia and fever, if the laryngocoele becomes infected. Patients may also present in extremis with airway obstruction. We present the case of a healthy 34-year-old gentleman with acute airway obstruction due to a mixed infected laryngocoele. Flexible nasoendoscopy showed a large cystic swelling arising from the laryngeal ventricle. Computed tomography of neck confirmed a right paraglottic collection extending into the ventricle and glottis, causing significant airway compromise. The patient was managed with microlaryngoscopy and cystic decompression. At outpatient follow up, he was completely asymptomatic and is currently under surveillance. Endoscopic decompression is a safe and effective initial management for mixed laryngocoele.
  • Awake Proning as an Adjunctive Therapy for Refractory Hypoxemia in Non-Intubated Patients with COVID-19 Acute Respiratory Failure: Guidance from an International Group of Healthcare Workers.

    Stilma, Willemke; Åkerman, Eva; Artigas, Antonio; Bentley, Andrew; Bos, Lieuwe D; Bosman, Thomas J C; de Bruin, Hendrik; Brummaier, Tobias; Buiteman-Kruizinga, Laura A; Carcò, Francesco; et al. (2021-03-11)
    Non-intubated patients with acute respiratory failure due to COVID-19 could benefit from awake proning. Awake proning is an attractive intervention in settings with limited resources, as it comes with no additional costs. However, awake proning remains poorly used probably because of unfamiliarity and uncertainties regarding potential benefits and practical application. To summarize evidence for benefit and to develop a set of pragmatic recommendations for awake proning in patients with COVID-19 pneumonia, focusing on settings where resources are limited, international healthcare professionals from high and low- and middle-income countries (LMICs) with known expertise in awake proning were invited to contribute expert advice. A growing number of observational studies describe the effects of awake proning in patients with COVID-19 pneumonia in whom hypoxemia is refractory to simple measures of supplementary oxygen. Awake proning improves oxygenation in most patients, usually within minutes, and reduces dyspnea and work of breathing. The effects are maintained for up to 1 hour after turning back to supine, and mostly disappear after 6-12 hours. In available studies, awake proning was not associated with a reduction in the rate of intubation for invasive ventilation. Awake proning comes with little complications if properly implemented and monitored. Pragmatic recommendations including indications and contraindications were formulated and adjusted for resource-limited settings. Awake proning, an adjunctive treatment for hypoxemia refractory to supplemental oxygen, seems safe in non-intubated patients with COVID-19 acute respiratory failure. We provide pragmatic recommendations including indications and contraindications for the use of awake proning in LMICs.
  • Subacromial impingement by a lipoma arborescens.

    Elamin, Mohamed; Yeluri, Venkatramana; Khatir, Hisham; O'Grady, Paul; Bennani, Fadel (2021-03-11)
    Subacromial impingement syndrome (SIS) is the leading cause of shoulder pain. A systemic approach for abnormal causes of SIS is recommended to avoid misdiagnosing rare or sinister pathologies. To our knowledge, only nine cases of subacromial lipoma arborescens associated with impingement syndrome have been reported in the literature. In this report, we briefly discuss histopathologic and radiological signs of an unusual case of impingement syndrome caused by subacromial "lipoma arborescens" and describe arthroscopic synovectomy after the failure of conservative management. The patient remains symptom-free five years after surgery.
  • Camptodactyly and DiGeorge syndrome: A rare hand anomaly.

    Hurley, C M; McHugh, N; Carr, S; Kelly, J L (2021-03-19)
    The most common deletion syndrome is 22q11.2 and it effects an estimated 1 in 3000 live births. Major features of this multisystem condition include congenital abnormalities, developmental delay, learning difficulties, immunodeficiency, endocrine anomalies and an array of psychiatric disorders. However, variability in phenotype and severity may cause the diagnosis to be overlooked. Early clinical recognition and treatment of DiGeorge syndrome has been shown to increase early life survival, decrease complications and enhance overall quality of life. Skeletal anomalies are infrequently described in 22q11.2 but a subset of patients exhibit upper and lower limb deformities. We present the case of a 5 year-old girl with bilateral fifth digit camptodactyly caused by a fibrous band, and the surgical management of this condition. The current report adds to the body of evidence that camptodactyly is a rare clinical feature of 22q11.2 deletion syndrome, and may serve as a diagnostic aid in these patients.
  • The impact of COVID-19 on a cohort of patients treated with clozapine.

    Fahy, Y; Dineen, B; McDonald, C; Hallahan, B (2021-04-05)
    Objectives: To examine the psychological and social impact of the COVID-19 pandemic and its associated restrictions on a cohort of patients with severe and enduring mental illness treated with clozapine. Methods: Semi-structured interviews were conducted with 63 individuals attending a clozapine clinic within the Galway-Roscommon Mental Health Services to determine the impact of COVID-19 restrictions on anxiety and depressive symptoms, social and occupational functioning and quality of life, by utilising Likert scale data. The Beck Anxiety Inventory (BAI) and Hamilton Anxiety Rating Scale (HAM-A) were additionally utilised to measure anxiety symptoms cross-sectionally. Results: Anxiety symptoms were low with a median BAI score of 4.0 and HAM-A score of 4.0. Likert scale measurements recorded only a modest adverse impact of COVID-19 restrictions on anxiety and depressive symptoms, quality of life and occupational and social functioning. Free-text comments from patients (n = 55), were grouped into five themes: neutral impact (n = 22), negative psychological impact (n = 13), negative social impact (n = 11), positive psychological impact (n = 5) and media coverage inducing anxiety (n = 4). Conclusions: Three months into the COVID-19 pandemic and its restrictions, the impact on individuals with treatment-resistant psychotic disorders attending a clozapine clinic has been modest, with preliminary evidence demonstrating minimal increases in subjective symptoms of anxiety and reduced social functioning. Reduced social engagements and supports attainable both within the community and from mental health services were noted by some participants.
  • Death in hospital following ICU discharge: insights from the LUNG SAFE study.

    Madotto, Fabiana; McNicholas, Bairbre; Rezoagli, Emanuele; Pham, Tài; Laffey, John G; Bellani, Giacomo (2021-04-13)
    Background: To determine the frequency of, and factors associated with, death in hospital following ICU discharge to the ward. Methods: The Large observational study to UNderstand the Global impact of Severe Acute respiratory FailurE study was an international, multicenter, prospective cohort study of patients with severe respiratory failure, conducted across 459 ICUs from 50 countries globally. This study aimed to understand the frequency and factors associated with death in hospital in patients who survived their ICU stay. We examined outcomes in the subpopulation discharged with no limitations of life sustaining treatments ('treatment limitations'), and the subpopulations with treatment limitations. Results: 2186 (94%) patients with no treatment limitations discharged from ICU survived, while 142 (6%) died in hospital. 118 (61%) of patients with treatment limitations survived while 77 (39%) patients died in hospital. Patients without treatment limitations that died in hospital after ICU discharge were older, more likely to have COPD, immunocompromise or chronic renal failure, less likely to have trauma as a risk factor for ARDS. Patients that died post ICU discharge were less likely to receive neuromuscular blockade, or to receive any adjunctive measure, and had a higher pre- ICU discharge non-pulmonary SOFA score. A similar pattern was seen in patients with treatment limitations that died in hospital following ICU discharge. Conclusions: A significant proportion of patients die in hospital following discharge from ICU, with higher mortality in patients with limitations of life-sustaining treatments in place. Non-survivors had higher systemic illness severity scores at ICU discharge than survivors.
  • Cardiorenal syndrome in COVID-19.

    Ali, Uzair Akbar; Sadiq, Muhammad Sajjad; Yunus, Muhammad Jawad (2021-04-23)
    SARS-CoV-2 preferentially targets the human's lungs, but it can affect multiple organ systems. We report a case of cardiorenal syndrome in a 37-year-old man who had symptoms of fever, myalgia and cough. He tested positive for COVID-19 and presented 5 days later with acute heart failure. Work up was done including echocardiography showing reduced ejection fraction. Later in the hospital course he developed acute renal failure and was treated with intermittent renal replacement therapy. No other definite cause of cardiorenal complications was identified during the course of the disease. A possible link with COVID-19 was considered with underlying mechanisms still needed to be explored. This case highlights the potential of SARS-CoV-2 affecting heart and kidneys. The disease not only involves the organs directly but can exacerbate the underlying comorbid illness.
  • Mutation Underlying Silent Stroke.

    Faryal, Rehman; Lee Tokar, Lisa; Langabeer, Stephen E; Krawczyk, Janusz (2021-06-01)

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