TITLE OF CASE Multiple Emergency Department Encounters for Acute Musculoskeletal Presentation with an Existing Mental Health Diagnosis SUMMARY Reconceptualising acute Musculoskeletal (MSK) injuries with both stress- and tissue- based factors is required to consider prior influences of mental health disorders on acute persistent musculoskeletal pain presentations involving an extremity. This report highlights repeated emergency presentations for acute persistent musculoskeletal pain involving an extremity for an individual in their 20s living with mental health diagnoses ranging across Depression, Mood Disorders and an eating disorder. This person also had mental health related inpatient admissions that were not captured under the retrospective record review for a large district hospital emergency department using the Systematized Nomenclature of Medicine Clinical Terms (SNOMED CT) classification system. This case report attempts to demonstrate that improving the understanding of pre-existing vulnerabilities and mental health diagnoses may assist with informing healthcare design to develop specialised care pathways for acute injury presentations with triage settings. BACKGROUND Acute MSK pain represents a common cause for seeking emergent health care (1),(2). While the majority of acutely injured people should expect to recover spontaneously, 50% will transition from acute to chronic pain and disability (3, 4). Data from robust clinical trials (5) and prevalence studies have failed to enable us to adequately identify individuals at risk of delayed recovery from MSK injury, and interventions targeting known risk factors have yielded, at best, only modest effects (6, 7). Furthermore, the most recent output from the global burden of disease in 2019 suggest MSK conditions are a leading cause of civilian years lived with disability. Alarmingly, this has not changed since 1990, suggesting research has had little effect on reducing the burdens of acute and chronic MSK conditions. Perhaps critical to this long-standing problem is that research has not generated new mechanistic knowledge into why some people recover and others do not following their acute MSK presentation. (8). Perhaps reconceptualising the acute MSK injury (be it a slip-and-fall, motor vehicle collision, fracture, muscle strain affecting the spine or extremities) as both a stress- and tissue-based injury is required to integrate and consider how pre-existing diatheses such as mental health disorders (9) influence the process of recovery. By identifying patients who may be vulnerable to costly negative chronic outcomes, appropriate early screening tools and preventative treatments can be offered to improve clinical outcomes and avoid harmful secondary effects, such as opioid dependency, stigma, poor return to work outcomes, withdrawal from valued life roles, long-term reliance on ineffective and costly management options and repeat emergency department (ED) encounters. For example, people living with mental health conditions tend to experience adverse physical health outcomes and significantly more medical conditions as compared to others without a history of mental health disorders. This is not to suggest the presence of a mental health disorder(s) predisposes a person to a life of chronic pain following adulthood injury requiring emergent care. While it is acknowledged that the Emergency Department environment presents a challenge, if not a trigger, to both busy, time-strapped, clinicians, and the patients themselves, knowledge of pre-existing diatheses could inform and streamline new clinical pathways for acute MSK injury on a patient-by-patient basis. The case of a Caucasian woman in her twenties seeking repeated ED management over a 7 year period is used to highlight the challenges for both the patient and the healthcare providers in, and beyond, the ED. CASE PRESENTATION The repeated voluntary ED presentations (38 visits over 7 years, from January 2015 – February 2021) were observed to be for persistent musculoskeletal pain, involving an extremity and upper limb pain. Pre-existing diagnoses of Mood Disorder, Depression, and an Eating Disorder (Anorexia Nervosa) were recorded at each ED presentation of persistent MSK pain involving an extremity, and while considered, did not feature in the clinical work-up. The retrospective record review was approved by the Northern Sydney Local Health District Human Research Ethics Committee, ethics approval number – 2021/STE02301: SSA. Design Retrospective interrogation of electronic medical records obtained from January 2015 until July 2021 capturing relevant data for acute presentations of MSK pain intersecting with mental health admissions over the preceding 12-month periods at a district hospital Emergency Department. Setting Emergency department triage facility for a large urban district hospital serving a catchment of over 1.5 million people. Subject A case report of an individual in their twenties, with multiple ED presentations (38 visits) over a 7 year period, classified by the treating ED physician/ clinicians using the SNOMED CT system at each presentation. The SNOMED CT is defined as a standardised, multilingual vocabulary of clinical terminology containing more than 300,000 medical concepts used by health care providers within the electronic exchange of clinical health information (10, 11) . The SNOMED CT is made up of the numerical codes, known as concepts, used to identifying clinical information. The number of concepts used are largely if not completely dependent on the clinical setting and patient census. In this case, the number of concepts available in a busy urban ED with level 1 trauma status is in the thousands. The concepts are divided into different groups such as body structure, clinical findings, geography, location and biological products represented by different concepts based on the complexity of the presenting condition. The terminology classifies presentations under findings, disorders, diagnoses and similar with individual numbers. SNOMED CT classifies “findings” as observations which may be objective or subjective information from a primary source, including human observation whereas the term “disorder” refers to as an abnormal clinical state and are classified under the hierarchy of disease (10). SNOMED CT however also tends to be subjective and have the same description while referring to different concepts due to the ambiguity dependant on the triage (12). The ED admission data captured the date, the patient’s reason for the visit to ED, MSK diagnosis provided at triage, and the pre-existing MH diagnosis. Under SNOMED CT, findings refer to observations that exist at the time of recording, while disorder suggests an abnormal and underlying psycho-physical pathological process that remains a vulnerability even post completion of treatment (11). As summarised in Table 1, the repeated MSK/ acute pain related presentations observed over the 2-year period were for persistent musculoskeletal pain involving an extremity(11). There were multiple mental health related admissions separate to the acute MSK pain presentation at ED over this period recorded initially for an unspecified mood (affective) disorder, progressing to Dysthymia/ Mood Disorder, followed by a separate admission for Post-traumatic stress disorder (PTSD), a further mental health admission for Dissociative convulsions, and the last captured admission was for Anorexia Nervosa (classified under Eating Disorder/s). Information regarding social circumstances, such as living independently or in supported accommodation, employment or education status, social supports both informal and formal including community mental health services, General Practitioner and any non-governmental organisations involvement was not available.

Title: Sirolimus-induced DRESS syndrome in a stem cell transplant patient.Authors: Fortunato CASSALIA1, Alice SPILLER1, Roberto SALMASO1, Francesca CAROPPO1,2, Anna BELLONI FORTINA1,2Affiliations:Dermatology Unit, Department of Medicine (DIMED), University of Padua, Padua, Italy.Pediatric Dermatology Unit, Department of Women and Children’s Health, University of Padua, Padua, Italy.Corresponding Author: Francesca Caroppo, MD Unit of Dermatology, Department of Medicine University of Padova, Italy Via Vincenzo Gallucci 4, 35121, Padova, Italy e-mail: [email protected]:Word count: 1329Tables: NoneFigure: 3References: 9Conflicts of interest: NoneAcknowledgements: NoneAuthor Contributions: All authors contributed to designing and conducting the work, drafting, and revising the manuscript and approved the final version for submission.ABSTRACTBACKGROUND: We present a case of sirolimus-induced DRESS syndrome in a stem cell transplant patient. Sirolimus is an immunosuppressive drug that inhibits the mTOR pathway. It is commonly used in organ transplants to prevent rejection. While no sirolimus-induced DRESS cases have been reported, allergic reactions with everolimus, a similar drug, have been documented. DRESS syndrome is a severe drug reaction characterized by fever, rash, and organ involvement. Diagnosis is based on clinical findings and laboratory tests. Early recognition, discontinuation of the drug, and supportive care are crucial in managing DRESS syndrome, often involving systemic corticosteroids.CASE REPORT A 24-year-old man who had undergone haplo-TESE transplantation for acute lymphatic leukaemia presented with diffuse itchy eczematous lesions. Initially diagnosed as atopic dermatitis, he received topical steroid therapy and NB-UVB phototherapy, but his condition worsened. Two months later, he returned to the emergency department with eczematous patches, xerosis, fever, chills, and generalized edema. His medical history included relapses of leukaemia, acute cutaneous graft-versus-host disease (GVHD), and Evans syndrome. He had been on sirolimus immunosuppressive therapy before the onset of symptoms. A skin biopsy revealed spongiotic dermatitis with dermal eosinophils, suggestive of drug reaction or atopic reaction. Based on the severity of the symptoms and histological findings, the patient was diagnosed with sirolimus-induced DRESS syndrome. Sirolimus was discontinued, and oral steroid therapy was initiated, leading to significant improvement. At the one-month follow-up, the patient was symptom-free and had lost the gained weight.CONCLUSION Although no cases of sirolimus-induced DRESS syndrome have been reported, allergic reactions with eosinophilia induced by everolimus have been documented. And since sirolimus and everolimus, both mTOR inhibitors, share a common mechanism of action, therapeutic indications, pharmacokinetics, adverse effects and drug interactions, it cannot be ruled out that sirolimus may trigger DRESS syndrome in patients with risk factors. In our case, the patient’s history characterized by stem cell transplantation and multiple immunosuppressive therapies may have contributed to the development of DRESS syndrome after beginning sirolimus therapy. This case may be the first evidence of sirolimus-induced DRESS syndrome in a stem cell transplant patient and highlights how early diagnosis, discontinuation of the culprit drug and appropriate management are crucial for a favourable outcome.BACKGROUND We present a case of sirolimus-induced DRESS syndrome1in a stem cell transplant patient. Sirolimus, also known as rapamycin, is an immunosuppressive and antiproliferative drug that inhibits the mammalian target of rapamycin (mTOR) pathway2. It has a wide range of clinical applications and has been extensively studied in various fields of medicine. Sirolimus is commonly used in solid organ transplants to prevent acute rejection and improve transplant survival with the advantage of reducing the nephrotoxicity associated with calcineurin inhibitors3. Although no cases of sirolimus-induced DRESS syndrome have been reported, allergic reactions with eosinophilia induced by everolimus, a similar drug of the mTOR inhibitor family, have been documented. In particular, cases of drug reaction with eosinophilia and systemic symptoms (DRESS) caused by an everolimus-eluting stent have been reported4. DRESS syndrome, also known as drug-induced hypersensitivity syndrome (DIHS), is a severe adverse drug reaction characterized by fever, skin rash, multi-organ involvement and eosinophilia. The pathogenesis of the DRESS syndrome remains unclear, involving a complex interaction between drug metabolism, immune dysregulation and genetic factors. Skin manifestations vary from maculopapular eruptions to severe exfoliative dermatitis, while organ involvement often involves the liver, kidneys, lungs and haematological system. Other systemic symptoms may include lymphadenopathy, myocarditis and interstitial nephritis. The diagnosis of DRESS syndrome is based on the recently validated RegiSCAR score5 which considers clinical findings, temporal relationship with drug exposure and blood tests. Early recognition and discontinuation of the involved drug are crucial for the management of DRESS syndrome. Supportive care and careful monitoring of organ function are essential, while symptomatic treatment aims to improve symptoms. Systemic corticosteroids are often administered to suppress the immune response6.CASE REPORTA 24-year-old boy, who had previously undergone haplo-TESE transplantation (transplantation of haploidentical haematopoietic stem cells for acute lymphatic leukaemia, presented to the dermatology outpatient clinic for the onset of a diffuse eruption with itchy eczematous lesions. The initial clinical presentation was diagnosed as atopic dermatitis and topical steroid therapy was recommended. In the following days, due to the lack of clinical response and the worsening of the skin eruption, about two months later the patient returned to the emergency department complaining of diffuse xerosis mixed with eczematous, itchy, finely scaling patches. In addition, the patient complained of fever and chills and significant and consistent oedema all over his body. He also reported a weight gain of 9 kg in the last month and eosinophilia >20% with leukopenia (Figure 1). The medical history revealed that the patient was diagnosed with acute lymphatic leukaemia in 2003 and underwent treatment according to the AIEOP LLA 2000 protocol (Prednisone, Vincristine, Daunorubicin, L-asparaginase, methotrexate, 6-Mercaptopurine, Cyclophosphamide, Cytarabine, Dexamethasone). In 2015, the patient developed a relapse for which he was treated according to the AIEOP protocol BFM 2009 concluded in 2017 (Prednisone, Vincristine, Daunorubicin, L-asparaginase, methotrexate, 6-Mercaptopurine, Cyclophosphamide, Cytarabine, Dexamethasone). However, in 2018, the patient developed a new relapse and therefore underwent haplo-TESE stem cell transplantation. Unfortunately, in 2019, the patient suffered an acute cutaneous GVHD for which he underwent treatment with oral cyclosporine in combination with tacrolimus that led to a rapid improvement of the skin manifestations. Unfortunately, in 2020 he was diagnosed with Evans syndrome and was treated with oral steroids; once the acute phase was over, the patient started immunosuppressive therapy with sirolimus 2 mg/die. Given the history and the severity of the clinical picture, the patient was hospitalized and a skin biopsy with histological examination was performed. The result of the histological examination revealed a hyperkeratosis with focally confluent spongiosis and irregular acanthosis of the epidermide. The underlying superficial dermis shows a modest infiltrate of lymphocytes, plasma cells and eosinophils, the latter also observed in the deep dermis. Specific histochemical stains did not reveal the presence of mucins and fungi (Alcial Blue and PAS) while immunohistochemical reactions for T and B lymphocytes excluded the clinical hypothesis of GVHD. The morphological picture depicts a spongiotic dermatitis with a discrete presence of dermal eosinophils suggesting the possibility of drug reaction vs. atopic reaction (Figure 2). Considering the clinical picture and the histological examination, the diagnosis of DRESS syndrome induced by sirolimus was made7. The drug was withdrawn and scaled-up oral steroid therapy was instituted, after 3 weeks of therapy the patient ceased taking the oral steroid. At the follow-up visit after one month the oedema was in remission, the patient no longer complained of any symptoms and had lost the previously accumulated kg (Figure 3).DISCUSSION The case described presents a 24-year-old boy with a complex medical history, including a previous diagnosis of acute lymphatic leukaemia and subsequent relapses, which required intensive treatments such as stem cell transplantation and immunosuppressive therapy. The patient presented to the dermatology outpatient clinic with diffuse itchy eczematous lesions, initially diagnosed as atopic dermatitis. Despite topical steroid therapy, the patient’s condition worsened with dry, itchy patches mixed with xerosis concomitant with fever, swelling, weight gain and abnormal blood results. Skin biopsy ruled out the diagnosis of graft-versus-host disease (GVHD). Based on the clinical presentation, histological findings and history of sirolimus therapy, the diagnosis of sirolimus-induced DRESS (Drug Rash with Eosinophilia and Systemic Symptoms) syndrome was made. Sirolimus was discontinued and the patient started oral steroid therapy, which was gradually reduced and finally discontinued. After three weeks of treatment, the patient’s symptoms improved, including remission of oedema, and at the one-month follow-up visit the patient was asymptomatic and had lost the weight gained during the illness. DRESS syndrome is a severe form of drug reaction in which skin manifestations and systemic involvement are associated. The onset time is usually longer than in other delayed skin reactions, on average 6-8 weeks after the introduction of the responsible drug. It is a severe idiosyncratic T-cell mediated reaction, classified as a type Vb and sometimes IVc delayed hypersensitivity reaction. DRESS is presumed to result from a complex interaction between drug exposure (such as vaccines or biological drugs), genetic predisposition and/or viral reactivation, and the development of this serious clinical condition would appear to be the result of the cumulative effect of aligned risks1. Early recognition and discontinuation of the culprit drug are crucial for the management of DRESS syndrome. Systemic corticosteroids are often used to suppress the inflammatory response and supportive care is provided for any organ involvement6. Although no cases of sirolimus-induced DRESS syndrome are reported in the literature, allergic reactions with eosinophilia induced by everolimus have been described. In particular, cases of drug reaction with eosinophilia and systemic symptom syndrome caused by an everolimus-eluting stent have been reported4. Sirolimus and everolimus both belong to the class of drugs called mTOR inhibitors and share several features8, including: (1) Mechanism of action: Sirolimus and everolimus act by inhibiting the mammalian target of rapamycin (mTOR); (2) therapeutic indications: both are used in immunosuppressive therapy to prevent organ rejection in transplant patients. (3) Pharmacokinetics: both are administered orally and are rapidly absorbed. They have a large volume of distribution and are extensively metabolised in the liver. (4) Adverse effects: They have common adverse effects including immunosuppression, which may increase the risk of infection, delay wound healing and altered response to vaccines. Other potential side effects include hyperlipidaemia (elevated blood lipid levels), peripheral oedema, gastrointestinal disorders, and metabolic abnormalities; (5) drug interactions: both drugs are metabolised by cytochrome P450 enzymes, which may lead to potential drug interactions with other drugs that act on these enzymes9. It is important to consider these interactions when prescribing or administering these drugs. In our case, the patient’s history of previous intensive treatments, stem cell transplantation and immunosuppressive therapies may have contributed to immune system dysregulation and the onset of DRESS syndrome following the initiation of sirolimus therapy. Timely diagnosis, discontinuation of the drug and appropriate management led to the resolution of symptoms and general improvement of the patient’s condition. The case emphasizes the importance of careful monitoring and consideration of potential adverse drug reactions in patients undergoing complex treatment regimes. This case could be the first evidence of DRESS syndrome induced by sirolimus in a stem cell transplant patient.FIGURE 1 Eczematous, itchy, finely scaling patches and significant and consistent oedema.FIGURE 2 Flap of skin with hyperkeratosis, focally confluent spongiosis and irregular acanthosis of the epider-mide. Modest infiltrate of lymphocytes, plasma cells and eosinophils, in the deep dermis.FIGURE 3 One month after withdrawal of SirolimusREFERENCESRamirez, G. A., Ripa, M., Burastero, S., et al . (2023). Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS): Focus on the Pathophysiological and Diagnostic Role of Viruses. Microorganisms, 11(2), 346.https://doi.org/10.3390/microorganisms11020346Mao, B., Zhang, Q., Ma, L., et al. (2022). Overview of Research into mTOR Inhibitors. Molecules (Basel, Switzerland), 27(16), 5295.https://doi.org/10.3390/molecules27165295Hahn, D., Hodson, E. M., Hamiwka, L. A., et al. (2019). Target of rapamycin inhibitors (TOR-I; sirolimus and everolimus) for primary immunosuppression in kidney transplant recipients. The Cochrane database of systematic reviews, 12(12), CD004290.https://doi.org/10.1002/14651858.CD004290.pub3Bianchi, L., Caraffini, S., & Lisi, P. (2014). Drug reaction with eosinophilia and systemic symptoms syndrome caused by an everolimus-eluting stent. International journal of dermatology, 53(4), e286–e288.https://doi.org/10.1111/ijd.12112Kardaun, S. H., Sekula, P., Valeyrie-Allanore, L., et al (2013). Drug reaction with eosinophilia and systemic symptoms (DRESS): an original multisystem adverse drug reaction. Results from the prospective RegiSCAR study. The British journal of dermatology, 169(5), 1071–1080. https://doi.org/10.1111/bjd.12501Calle, A. M., Aguirre, N., Ardila, et al. (2023). DRESS syndrome: A literature review and treatment algorithm. The World Allergy Organization journal, 16(3), 100673. https://doi.org/10.1016/j.waojou.2022.100673Jindal, R., Chugh, R., Chauhan, P., et al (2022). Histopathological Characterization of Drug Rash with Eosinophilia and Systemic Symptoms (DRESS) and Comparison with Maculopapular Drug Rash (MPDR). Indian dermatology online journal, 13(1), 32–39.https://doi.org/10.4103/idoj.idoj_452_21Yan, X., Huang, S., Yang, Y., et al (2022). Sirolimus or Everolimus Improves Survival After Liver Transplantation for Hepatocellular Carcinoma: A Systematic Review and Meta-Analysis. Liver transplantation : official publication of the American Association for the Study of Liver Diseases and the International Liver Granata, S., Dalla Gassa, A., Carraro, A., et al (2016). Sirolimus and Everolimus Pathway: Reviewing Candidate Genes Influencing Their Intracellular Effects. International journal of molecular sciences, 17(5), 735. https://doi.org/10.3390/ijms17050735

INTRODUCTIONCharles Bonnet Syndrome(CBS) was first described in 1760 by Naturalist and philosopher Charles Bonnet, who first observed lifelike complex visual hallucinations in his grandfather Charles Lullis after bilateral cataract surgery(1); it was a neurologist George de Morsier who coined the term in 1938 after Charles Bonnet himself developed the condition(2). CBS, also known as ”phantom image”, is characterized by recurrent or persistent complex visual hallucinations in people with a disease of the visual system with intact insight, intellectual function and normal cognitive functioning without primary psychiatric disorders(3,4). With limited knowledge of CBS among physicians, the prevalence of CBS is underreported(4) even though 41-59% of the visually impaired experience elementary visual phenomena, and 11-15% exhibit complex hallucinations, due to a medical condition or artificially produced, such as in preparation for cataract surgery(5,6). The presence of varying inclusion criteria, inconsistent depth of questioning and limited patient disclosure for fear of being ridiculed may all contribute to underreporting(3,5). Visual hallucinations could be a sign of psychiatric disorders, neurological diseases, metabolic abnormalities, and the use or abuse of specific drugs(7). Both elementary forms(3) and complex visual hallucinations are reported in CBS(8); while glaucoma,cataract and age-related macular degeneration are the most common associated disorders, any ophthalmological conditions can lead to CBS(7), especially in significant visual impairment(9). A pituitary adenoma is rarely associated with CBS (10); however, reduced visual acuity or a visual field deficit can occur from the benign tumour’s compressing optic chiasm affecting one or both eyes(11).There are three main theories associated with the pathogenesis of CBS, and these are; the sensory deprivation or deafferentiation theory related to spontaneous excitation due to a loss of visual input to the brain(12), the release theory associated with excessive excitation and the consequent release of visual hallucinations(13,14), and the ”irritative theory” due to distal provocative injuries transmit abnormal input to the visual cortex leading to abnormal excitatory activity to the temporal and occipital lobes(15,16).Charles Bonnet syndrome is treated multifacetedly with pharmacotherapy, psychosocial therapy, maintaining appropriate eye care, and sensory stimulation. Generally, CBS is treated with antipsychotics and antidepressants(5), and in some cases, antiepileptic medications have also been used with variable benefits (17).An 81-year-old blind male presented with a three-year history of visual hallucinations after a recurrence of pituitary macroadenoma. His past psychiatric history is uneventful, and he has intact insight with no cognitive impairment. He improved on a low haloperidol dosage, with a few relapses, when he stops the medications.

Teduglutide in Amyloidosis-associated Intestinal FailureClara Luhn§(1), Hermine Agis§(2), Elisabeth Hütterer(3), Ingrid Simonitsch-Klupp(4), Christopher Dawoud(1), Anton Stift(1), Felix Harpain(1)(1) Department of Surgery, Division of General Surgery, Medical University Vienna, Austria(2) Department of Internal Medicine I, Division of Hematology and Hemostaseology, Medical University of Vienna, Austria(3) Department of Internal Medicine I, Division of Oncology, Medical University of Vienna, Austria(4) Department of Pathology, Medical University of Vienna, Austria§ contributed equally

IntroductionCarotidodynia or Fay’ syndrome is characterized as neck pain near the carotid bifurcation (1). Three main differential diagnoses can be responsible for carotidodynia : carotid dissection, cervical osteoarthritis and large vessel vasculitis ie Takayasu disease, giant cell arteritis. The definition of carotidodynia has evolved in the past century: initially classified in the International Classification of Headache Disorders (ICHD) in 1988 and then declassified due to unspecific clinical signs in 2004 (2).Over the last decade, reports of perivascular inflammation of the carotid artery have been described in patients with neck pain called TIPIC syndrome: Transient Perivascular Inflammation of the Carotid Artery. The diagnostic criteria for this rare vascular disorder were established by Lecler et al. in 2017 : acute neck pain directly around the level of the carotid bifurcation, eccentric pericarotidian infiltration on imaging, exclusion of another vascular or nonvascular diagnosis with imaging and improvement within 14 days either spontaneously or with anti-inflammatory treatment (1). A recent multicenter retrospective study of 72 patients with TIPIC syndrome confirmed the benign nature of this disorder and that recurrence may occur in up to 20% of cases (Micieli E et al. Transient perivascular inflammation of the carotid artery (TIPIC) syndrome; Vasa 2022). However, TIPIC pathophysiology and triggers remain poorly understood.We report herein the case of a myelodysplastic syndrome (MDS) patient with granulocyte colony stimulating factor (GCSF) induced TIPIC syndrome.

INTRODUCTION Pheochromocytoma, arising from the chromaffin cells of the adrenal medulla, can cause excessive catecholamines secretion and trigger pheochromocytoma crisis, characterized by high mortality rate up to 13%1. Pheochromocytoma crisis induced catecholaminergic cardiomyopathy may lead to multiple organ system damage or dysfunction, myocardial infarction, acute left ventricular failure, cardiogenic shock, and other fatal outcomes, commonly in undiagnosed or well-controlled pheochromocytoma2. Surgical resection is the primary treatment; however, the instability of perioperative hemodynamics poses a high risk of anesthesia and poses great challenges in treatment3. Currently, research on the diagnosis, treatment, and nursing care of such patients is mainly based on case reports. Veno-arterial extracorporeal membrane oxygenation (VA -ECMO) has been reported to stabilize circulation and extend the surgical window for patients with severe myocardial injury or circulatory failure4. In June 2021, we successfully treated a patient with pheochromocytoma presenting with cardiogenic shock as the first manifestation of multiple organ failure. This case report presents our experience in diagnosis, treatment, and nursing care of pheochromocytoma with various severe complications.

Characteristics and treatments of patients with significantly elevated creatine kinase levels induced by seizures: case report and literature reviewKai Wang, Jinwei Yang, Wenhao Xu, Lei Wang, Yu Wang*The First Affiliated Hospital of Anhui Medical UniversityCorrespondence: Yu WangDepartment of Neurology, The First Affiliated Hospital of Anhui Medical UniversityJixi Road 218, Shushan District, Hefei, Anhui Province 230032,People’s Republic of China. Email [email protected]

Clival Ectopic Pituitary Prolactinoma was Successfully Managed by Transsphenoidal Surgery: A Rare Case Report

Introduction The general term for non-carious loss of dental tissue (non-carious cervical lesion (NCCL)) is tooth wear which describes the loss of hard dental tissue due to various etiological factors, including chemical and mechanical processes(1). According to Loomans et al., tooth wear, when abnormal for the age of the patient, causes pain and discomfort, functional issues, or decline in aesthetic appearance, and if progressing, may result in complex undesirable complications. Tooth wear can be classified as erosion, abrasion, attrition, and abfraction (2). The clinical observations have shown that the wear mechanisms do not act alone but interact with each other and usually with synergic effect on each other to cause the destruction of dental surfaces(3). The increased occurrence of erosive wear has been reported in young populations due to dietary habits, a growing tendency to consume soft and energy drinks, and the impact of stress. Regardless of the causative factors, tooth wear may affect oral health, personal comfort, loss of vertical dimension of occlusion (VDO), tooth sensitivity, hyperactive muscles, temporomandibular joint problems and pulpal involvement(4). However, aesthetic complaints exist, particularly in young adult patients. The incisal edges may also show signs of wear, shortening due to the loss of enamel support, and subsequent fracture, depending on the progression. A high prevalence of tooth wear has been reported in the young population, which can be reflective of the future dental problems in this generation. Also, in elderly population complete loss of dentition is now second to severe tooth wears as dental problem. For successful treatment of the above cases, paying close attention to the anterior guidance ,posterior contacts and TMJ is essential(5).The significance of anterior guidanceFollowing centric relation (CR), anterior guidance is the most significant factor that must be determined when restoring an occlusion. Aside from its key role in aesthetics, anterior guidance is a crucial factor in protecting the posterior teeth. This protective role of anterior guidance is so important that if the posterior teeth are not protected from lateral and protrusive stresses by the separating effect of the anterior teeth, they will be subjected to high and non-vertical stress over time which can lead to worn dentition. Patient comfort is determined by how accurately the anterior guidance is coordinated with the functional pattern of other parts of masticatory system (6).Posterior-anterior contactsThe following formula should be kept in mind when analyzing the anterior guidance: “Lines in the front and dots in the back. ” The dots in the back merely show the contact points on posterior teeth which means maximum Intercuspation (MI) contacts are coincide with CR. The lines in the front demonstrate the role of anterior teeth in separating the posterior teeth during all eccentric movements. Thus, the stable contacts of anterior teeth in CR and sliding contacts in lateral movements are fundamental issues in extensive restorations (7). This is why this scheme is called “mutually protected system”.Treatment optionsThe biggest problem with restoring worn dentition is there is no space for restorative material to provide optimum resistance and retention form. A variety of treatment options have been proposed, including elective endodontic treatment, surgical crown lengthening for restoration of worn teeth with insufficient restorative space, and prosthetic treatments(8). However, these conventional methods are very costly, time-consuming, and invasive. They destroy a considerable amount of tooth structure and are accounted as an irreversible path for both the patient and dentist. Another problem with these invasive treatment plans is they are heavily dependent on laboratory precision, in fact if dentist has no access to a skillful laboratory whole treatment can lead to a failure. With advances in adhesion and dental bonding agents, minimally invasive restorations have been introduced to preserve the residual tooth structure. More invasive approaches as the treatment options can be postponed until more advanced ages.An alternative treatment option is occlusal veneers which are considered a conservative approach to increase the VDO in cases with severely worn teeth. The durability of these restorations and their ease of construction make them an appropriate conservative treatment option(9). But they are costly and still needs a skillful ceramist.An ultra-conservative and simple treatment is proposed using a combination of composite resin and the Dahl principle to resolve the anterior teeth wear. The Dahl approach involves the creation of inter-occlusal space through axial movement of the teeth via an appliance or the restorations placed in the supra-occlusion and subsequently re-creating the occlusal contacts of the full arch over a time span. The primary Dahl appliance is based on a metal cobalt-chromium appliance cemented on the palatal surfaces of the upper anterior teeth(10). However, the quality and long-term esthetical properties of directly bonded restorations are more dependent on the operator, they have been used recently to create the proper space with low to moderate durability compared to indirect restorations (11). Concerning the directly bonded restorations, as stated by Craig, while using the composites for the restoration of worn teeth is superior to using ceramics due to their lower modulus of elasticity and seemingly satisfactory clinical performance, they require maintenance owing to their limited mechanical and physical properties(12).

Massive circulating metastatic cells: a case of carcinocythemiaCarine Farkh1, Jérome Debus1, Valérie Andrieu1, Juliette Gay11Laboratoire Hématologie, AP-HP, Hôpital Bichat-Claude Bernard, F-75018 Paris, France.

A Case of Trichotillomania (TTM) and Bulimia Nervosa (BN) in a Patient with Adult-Onset Attention-Deficit/Hyperactivity Disorder (ADHD)

Imaging findings of succinate dehydrogenase-deficient renal cell carcinoma

Staphylococcal scalded skin syndrome (SSSS) in premature infants is a rare condition. We present SSSS in a preterm infant, who developed all signs and symptoms of SSSS at birth, with a fatal outcome due to fungemia caused by Candida parapsilosis. The clinical presentation was challenging to differential diagnosis. SSSS diagnosis was

IntroductionIBD is a chronic systemic inflammatory disease that predominantly involves the gastrointestinal tract. At the same time, it exerts numerous cardiovascular manifestations, like atherosclerotic cardiovascular disease and thromboembolic events, due to a hypercoagulable state. Although the disease mechanism is unclear, factors responsible, like the disruption of the normal coagulation cascade, hyperhomocysteinemia, abnormalities in platelet-endothelial cell interactions, and increased fibrinolysis, are implicated.

Castleman Disease Presenting as Lymphadenopathy in a Female with Systemic Lupus Erythematosus: A rare case report

Meningitis and Endocarditis as a Sequela of Streptococcus Pneumoniae Mastoiditis: A Case Report Running Title: Meningitis, Endocarditis, Mastoiditis  as a Variant of Austrian SyndromeDr. Mitchell Peebles, DO, PGY-1 Internal Medicine Resident at Texas Health Harris Methodist Hospital, Fort Worth, TexasEmail Address: [email protected]. Mehnaz Roshani, MD, Internal Medicine Core Faculty at Texas Health Harris Methodist Hospital, Fort Worth, TexasEmail Address: [email protected] Srivastava, MS3 at Anne Marion Burnett School of Medicine at TCU, Fort Worth, TexasEmail Address: [email protected] Author: Kumaraman SrivastavaEthical Statement: The authors of this study had the required consent from the patient involved in this case report.Data Availability Statement: All data can be found in the article and no other data or materials were used.Acknowledgements: N/AConflicts of Interest: Authors state no conflicts of interest.Background: Streptococcus pneumoniae is responsible for more than 50% of all bacterial meningitis and has a case fatality rate of 22% in adults1. In addition, Streptococcus pneumoniae is also one of the most common causes of acute otitis media, a known cause of mastoiditis. However, in conjunction with bacteremia and endocarditis, limited evidence is able to be identified2. This sequence of infections also closely relates to Austrian syndrome. Otherwise known as Osler’s triad, Austrian syndrome is a rare phenomenon of meningitis, endocarditis, and pneumonia secondary to Streptococcus pneumoniae bacteriemia that was first delineated by Robert Austrian in 19563. The incidence of Austrian syndrome is reported to be less than <0.0001% per year and has decreased significantly since the initial usage of penicillin in 19414. Despite this, the mortality rate of Austrian syndrome is still around 32%5. Despite an extensive literature review, we were unable to find any reported cases of variants of Austrian syndrome that include mastoiditis as the primary insult. As such, we present a unique presentation of Austrian syndrome with mastoiditis, endocarditis, and meningitis with complex medical management that led to resolution for the patient.Objective: To discuss the presentation, progression, and complex medical management of a rare triad of mastoiditis, meningitis, and endocarditis occurring in a patient.Key Clinical Message: Austrian Syndrome classically consists of meningitis, endocarditis, and pneumonia due to Streptococcus pneumoniae bacteremia. A literature review, however, does not show variants of this triad. Our case highlights a unique variant of Austrian Syndrome with mastoiditis, meningitis, and endocarditis which requires immediate recognition and treatment to prevent devastating patient outcomes.Case report: A 78-year-old man with past medical history of resected prostate cancer, pheochromocytoma, and obstructive sleep apnea presented to the emergency department due to altered mental status, right-sided whole-body weakness, and expressive aphasia. The patient’s wife reported that the patient was shivering and lethargic and his last known normal was determined to be 14 hours prior to presentation. The patient’s recent medical history was remarkable for planned procedure to undergo bilateral eustachian tube placement. The patient developed a fever up to 103 F in the emergency department and initial labs revealed a leukocytosis of 12 with lactic acid of 1.67. Further imaging included CT scan of the head as well as CTA head and neck which did not reveal any acute abnormalities other than a right-sided tympanomastoid effusion FIGURE 1. The physical exam on admission was significant for altered mental status, agitation, and subjective nuchal rigidity. Moreover, the patient was incoherent, a significant decline from his baseline independence according to his wife. The patient received a fluid bolus and Cefepime in the ED prior to hospital admission. An attempt to obtain a lumbar puncture in the ED was also unsuccessful due to the patient’s increased agitation. Upon admittance to the hospital, Vancomycin and Ceftriaxone at central nervous system dosing were administered to the patient for empiric coverage of meningitis. Furthermore, blood cultures were positive for Streptococcus pneumoniae . On hospital day 2, another attempt at lumbar puncture under fluoroscopy was attempted but was unsuccessful due to repeated patient agitation. Lumbar puncture was finally obtained on hospital day 3 under sedation with the following CSF findings consistent with bacterial meningitis: FIGURE 2CSF cultures showed no growth and ENT was consulted for further evaluation of right tympanomastoid effusion secondary to mastoiditis. Patient underwent right mastoidectomy with bilateral myringotomy with ear tube insertion on hospital day 3. The mastoid bone was noted to be sclerotic and the mastoid air cells were filled with inflammatory tissue. Vancomycin was also discontinued on hospital day 4 after cultures revealed sensitivity to Ceftriaxone.Further workup included a transthoracic echocardiogram which revealed an immobile mitral valve echo-density measuring 0.7 cm in diameter associated with chordal structures of the anterior mitral leaflet without evidence of mitral regurgitation or mitral stenosis FIGURE 3. This strongly suggested endocarditis and infectious diseases and cardiology were consulted and determined transesophageal echocardiogram was not required to confirm the diagnosis. As such, the decision was made to extend the patient’s ceftriaxone for 6 weeks for treatment of endocarditis. However, the patient developed significant surgical site bleeding on hospital day 6 which was presumed to be due to ceftriaxone-induced thrombocytopenia, so ceftriaxone was stopped, and Vancomycin was restarted. The patient also had elevated fibrinogen suggesting possible disseminated intravascular coagulation so a dose of cryoprecipitate was given, and the patient’s platelet and fibrinogen levels stabilized after hospital day 9. The patient had deconditioned significantly from his baseline status so physical therapy was initiated and the patient responded very well. On hospital day 10, a peripherally inserted central catheter line was placed so that the patient could continue to receive IV antibiotics at home. The patient was stable for home discharge on hospital day 11.Discussion/Conclusion:Austrian’s syndrome has been typically described as meningitis, endocarditis, and pneumonia with multiple case reports and case series describing the difficulties of managing this complex disease. There is, however, no published cases that demonstrate a variant of Austrian syndrome with the triad of mastoiditis, endocarditis, and meningitis. In this patient’s case, the tympanomastoid effusion may have been the initial insult that led to mastoiditis. Moreover, meningitis has been described in several cases as a rather uncommon complication of mastoiditis6,7. Furthermore, the etiology of the patient’s endocarditis was likely due to meningitis8, but there are some case reports that suggest that mastoiditis may also directly cause endocarditis9. Our patient initially presented with altered mental status with multiple failed attempts at a lumbar puncture due to agitation, so we initiated empiric coverage of meningitis with vancomycin and ceftriaxone10. Notably, we did not add ampicillin because our clinical suspicion ofListeria monocytogenes was extremely low. Initial CT scans revealed a right tympanomastoid effusion which we believed was the nidus of infection. Prompt ENT intervention surgically removed the right mastoid bone to alleviate the probable bacterial source, but our patient unfortunately developed post-operative DIC which is a known complication of both meningitis and head/neck surgery11,12. Ceftriaxone was stopped due to incidences of ceftriaxone-induced thrombocytopenia and cryoprecipitate was given with resolution of the patient’s uncontrolled bleeding. Our patient endured several complications due to his complex interaction of diseases, but we were able to treat him effectively. As such, this case report presents an exceptionally distinct variant of a rare phenomenon in Austrian syndrome with complex medical management that resulted in complete recovery of the patient.

A 2-month-old boy presented to the former emergency department with a fever of 38 ° C and hypoxemia and respiratory failure. He was transferred to our hospital on suspicion of myocarditis because he had an enlarged chest X-ray, a 30% EF, a decrease in left heart function, and an increase in myocardial deviation enzyme(CK-MB 149.8 IU/L, TnT-1（＋）). Echocardiography revealed dilation and poor contraction of left ventricle and no abnormalities in the origin of the coronary arteries (Figure1A, B), and treatment with cardiac stimulants, antibiotics, and IVIG was started for myocarditis. After that, his cardiac function did not improve, and contrast-enhanced CT was taken on day 12. He was diagnosed with anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA). The LCA diverged from the right PA instead of the main PA (Figure1C). Coronary transplantation was performed on day 13. The postoperative course was uneventful, and he was transferred to our hospital on the 21st day after the operation.ALCAPA mostly originates from the left posterior sinus at the root of the PA, which can lead to coronary steal syndrome and myocardial ischemia, and the incidence of the disease is approximately 1/3,00,000, accounting for 0.25% to 0.5% of congenital heart disease(1, 2). In this case, the LCA diverged from the right PA instead of the main PA, which is extremely rare and has never been reported before. This difference in bifurcation might make the diagnosis of ALCAPA on echocardiography more difficult.ALCAPA should be considered in the differential diagnosis of myocarditis, and contrast-enhanced CT or catheterization should be considered even if coronary artery abnormalities are not detected on echocardiography.

Delayed cerebellar ataxia induced by Plasmodium falciparum malaria: A rare complicationEmmanuel Edwar Siddig1,2,*, Sarah Misbah EL-Sadig3, Hala Fathi Eltigani1, Ahmed Mudawi Musa4, Nouh Saad Mohamed5, Ayman Ahmed4,6,71 Mycetoma Research Centre, University of Khartoum, Khartoum, Sudan2 Faculty of Medical Laboratory Sciences, University of Khartoum, Khartoum, Sudan.3 Faculty of Medicine, University of Khartoum, Khartoum, Sudan4 Institute of Endemic Disease, University of Khartoum, Khartoum, Sudan5 Molecular Biology Unit, Sirius Training and Research Centre, Khartoum, Sudan6 Swiss Tropical and Public Health Institute (Swiss TPH), Allschwil 4123, Switzerland7 University of Basel, Petersplatz 1, Basel 4001, Switzerland* Correspondance: Emmanuel Edwar Siddig; University of Khartoum, Khartoum, Sudan.Email: [email protected]: 0000-0001-6314-7374