Chicken Coop-Induced Hemophagocytic Lymphohistiocytosis

Introduction: Hemophagocytic lymphohistiocytosisis (HLH) is a rare and often fatal condition characterized by an overactive but ineffective response of the immune system. There have been several documented causes, which include genetic predisposition, malignancy, infection, autoimmune disease, and chronic immunosuppressive therapy. Case Presentation: A 42 year-old woman with a two year history of ankylosing spondylitis, who was recently initiated on Infliximab, presented with abdominal pain, fevers, and jaundice. She had a temperature of 104F and a leukocytosis. She was suspected to have acute cholangitis and was initially treated with antibiotics, however, several imaging studies, including ultrasound, HIDA, and MRCP did not appreciate intra or extra hepatic duct dilatation, bile duct wall thickening, or cholelithiasis/choledocholithiasis. She remained febrile and had persistent elevation of her liver enzymes and bilirubin. Her clinical condition worsened, progressing to renal failure, cerebral edema and new onset seizures. Liver biopsy was obtained and demonstrated acute granulomatous hepatitis secondary to fungal organisms, morphologically consistent with histoplasmosis. Further history from the patient revealed that she had been maintaining a chicken coop in her backyard for the past several years. Due to her persistent fevers, cytopenia and a ferritin level of 20,308, the suspicion for HLH continued to grow. Bone marrow biopsy was performed which noted hemophagocytic cells, as well as fungal yeast forms, as seen with use of GMS stain. She met six of the eight diagnostic criteria for HLH based on the Histiocyte Society’s Guidelines (HLH-2004) - [Fever, bi-cytopenia, hypertriglyceridemia, elevated ferritin, elevated soluble IL-2 receptor, and hemophagocytosis on bone marrow biopsy]. It was believed that her HLH was secondary to disseminated histoplasmosis. She was started on liposomal Amphotericin B for treatment of disseminated histoplasmosis, as well as Etoposide and Dexamethasone for treatment of HLH. Her clinical condition improved, and within 3 weeks of initial diagnosis of HLH, she was ultimately discharged with continued treatment with Itraconazole, as well as a four week taper of Dexamethasone. Discussion: This case illustrates a patient, who after initiation of treatment with an anti-TNF agent for ankylosing spondylitis, developed HLH due to disseminated infection with Histoplasma capsulatum. HLH is an under-recognized, severely aggressive syndrome with high mortality rates. It is typically provoked by disruptions in immune homeostasis, such as infection or immunosuppression. It is most commonly associated to viral infections, Histoplasma is more commonly associated with advanced HIV infection in high endemic areas. Prompt recognition and diagnosis of this disease is crucial to ensure timely treatment, however due to its rarity, and non-specific clinical presentation, this may be of great obstacle.https://scholarlycommons.henryford.com/merf2020caserpt/1068/thumbnail.jp

An Atypical Presentation of Disseminated CMV in an Immunocompromised Patient

Discussion: We present a case of disseminated CMV manifesting as cutaneous eruptions, viremia, retinitis, and encephalopathy in an immunocompromised patient. This patient was admitted for multifocal pneumonia, but continued to spike fevers and remained encephalopathic even after resolution of the pneumonia. Despite extensive evaluation for other infectious etiologies he failed to improve until he was placed on ganciclovir. Encephalopathy in HIV patients requires evaluation for Toxoplasma, primary CNS lymphoma, progressive multifocal leukoencephalopathy, and also HIV encephalopathy. This patient had never been on anti-retroviral therapy, and imaging was concerning for HIV encephalopathy; however, he had drastic improvement in mentation upon initiating ganciclovir therapy. Disseminated CMV is not uncommon in AIDS patients, but is traditionally associated with visceral organ involvement (retinitis, colitis, meningitis, and hepatitis). Cutaneous manifestations, and encephalopathy are less common presentations. Conclusion: Due to the immunocompromised nature of AIDS patients, there are multiple infectious etiologies that can present with encephalopathy, and cutaneous manifestations. As such, a thorough history, physical exam, and a systematic approach is necessary to identify the cause. Disseminated CMV can present with encephalopathy as well as cutaneous lesions that can be co-infected with HSV 1 and 2. This patient showed dramatic improvement in mentation and skin lesions while being on IV ganciclovir.https://scholarlycommons.henryford.com/merf2019caserpt/1051/thumbnail.jp

Severe COVID 19 Case with Atypical Presentation

Introduction: COVID-19 was brought to the attention of the WHO on December 31st, 2019 and classified a global pandemic on March 11th. As of March 26th, there were 2,856 cases and 60 deaths in Michigan, with 851 cases and 15 deaths in Detroit. Efforts to characterize risk factors for severe disease may improve clinical outcomes and inform resource allocation. Better understanding of the epidemiological and clinical characteristics of COVID-19 are essential to slowing transmission and treating patients. Below we detail the clinical features of a COVID-19 positive patient seen in early March, 2020. Case Report: An 80-year-old female presented to the ED with fevers. She endorsed worsening fevers, watery diarrhea, abdominal pain, and myalgias of one week. She was lethargic and presyncopal for one day prior to presentation. She endorsed contact with sick members at home and denied travel history. Her past medical history was significant for resected colon cancer, T2DM, COPD, HTN, and CAD. She was a former smoker. On exam she was febrile and had lower abdominal tenderness. Her labs showed lymphopenia, thrombocytopenia, and mild hyponatremia. Influenza swab, viral panel, and legionella urine antigen were negative, prompting COVID-19 testing. Chest x-ray showed diffuse reticular opacities. Antibiotics were started and she was admitted on hospital day 2. She developed dyspnea, rales, and increasing oxygen demand through her hospitalization. COVID-19 testing resulted positive by day 4. Infectious disease recommended ribavirin and lopinavir-ritonavir. Her son was informed, and all contacts were advised to isolate for two weeks. On days 5 and 6 she improved clinically, though was not discharged due to concern she would not abide by self-isolation recommendations. Overnight, she had increasing oxygen demand and repeat chest x-ray revealed worsening infiltrates. She was intubated and transferred to the MICU on day 7. Inflammatory markers including LDH, CRP, procalcitonin, lactate, anion gap, aPTT, INR, and D-dimer were elevated. ABG revealed low PaO2 and low pH. Her IL-6 and fibrinogen levels were normal. She continued to decompensate with concern for septic shock, and had worsening bradycardia and hypotension, unresponsive to three vasopressors. On day 7, she expired. Discussion: Our report of a COVID-19 patient that ended in their mortality provides important lessons for providers. The transmission mode was local spread, reflecting high transmissibility among family groups. Fevers are reliably present over the illness course, though seen in under half on presentation. Fatigue is common and was observed in our patient. Cough is common, though was absent here. Diarrhea is an uncommon presenting symptom, reducing initial clinical suspicion and potentially delaying diagnosis. Other characteristics seen in our patient reflect a growing body of evidence supporting high rate of morbidity and mortality in patients with COVID-19. Such populations, including critically ill elderly population, require ICU level care, with marked lymphopenia on admission labs, and elevated inflammatory markers across their hospitalization. Also, investigative treatments including Lopinavir-ritonavir, ribavirin, hydroxychloroquine, and azithromycin have yet to demonstrate clinical efficacy in large randomized controlled trials.https://scholarlycommons.henryford.com/merf2020caserpt/1105/thumbnail.jp

Delaying escalation of care for a COVID-19 patient

Background: Since being reported on December 31st 2019, COVID-19 has become a pandemic. In Detroit, there are 1075 cases and 23 deaths, as of March 28th, 2020. Rapid identification of the disease is vital as preliminary reports show that multiple ED and clinic visits are associated with worse outcomes, likely due to delayed treatment. Our report describes the course of a COVID-19 patient who required multiple visits prior to diagnosis, and rapidly deteriorated.Case Report: A 63-year old African American man presented to his PCP with sore throat, cough, and body aches. Patient endorsed symptoms for 4 days, no sick contacts, and flu swab was negative. Patient was diagnosed with a viral syndrome and prescribed rest and symptomatic care. The following day he went to the ED with worsening symptoms and hypotension and was sent home. The next day, patient went for a CXR, where he developed SOB. Due to his distress and presence of bilateral pneumonia, he was sent to the ED rule out COVID. At the ED, patient endorsed a fever, SOB, and chills. Patient’s past medical history included asthma, hypertension, and diabetes. On exam, he was febrile but hemodynamically stable. Patient was ill-appearing, with decreased breath sounds on the left. Labs showed leukopenia, lymphopenia, and an AKI. COVID testing was sent. Patient was admitted, with airborne plus precautions, and antibiotics were started.On hospital day 3, patient became persistently febrile and hypoxic. ABG was done which showed a PaO2 of 55.9. Due to worsening respiratory status, patient was intubated and transferred to the MICU. CXR was repeated and showed worsening airspace opacities bilaterally, and small pleural effusions. COVID test came back positive and treatment began with Hydroxychloroquine, and use of remdesivir pending. On hospitalization day 7, patient received remdesivir and tocilizumab, with hopes that reduced systemic inflammation would lead to improvement of his ARDS. Overnight, patient was hypotensive and nonresponsive to fluids. Levophed was begun, and vent settings were increased. On days 9 and 10, due to worsening hypoxia and inability to follow commands, patient was paralyzed to allow for more time to improve inflammation. Patient was placed on max vent settings, and his PaO2 sat was 57. CXR was repeated due to worsening vent requirements and showed no change. Subsequently, patient became tachycardic in the 120s, and hypotensive to the 80s. D-dimer was elevated, and patient desaturated when turned or repositioned. On day 11, patient was found to be hyperkalemic. Nephrology was consulted and determined the hyperkalemia was due to hemolysis secondary to DIC, but the patient was not a candidate for ultrafiltration or intermittent dialysis. Due to absence of clinical improvement, patient was transferred to comfort care, and expired.Discussion: Our report elucidates the importance of rapid identification of a patient with COVID. Our patient had a standard presentation with cough, fever, body aches and sore throat, indicating that the possibility of COVID as the cause for the patient’s presentation should have been considered. During a pandemic it is vital to practice with a high of index of suspicion. The importance of prompt identification of the illness becomes even more salient considering that current treatment approach is primarily symptomatic management, due to lack of clinically effective curative treatments. It may seem overly simplified, but the sooner a patient is able to receive these services, the more likely they are to recover. While our patient had multiple risk factors for deterioration due to COVID, such as HTN, and T2DM, our patient had two opportunities for escalation of care and identification of his underlying pathology that could have improved his prognosis. Further, our report is in line with preliminary findings that African Americans and patientshttps://scholarlycommons.henryford.com/merf2020caserpt/1103/thumbnail.jp

Necrotizing Cavitary Lung Mass in Patient with AIDS: A Rare Manifestation of PCP

Introduction: Pulmonary infections in immunocompromised hosts encompass a wide differential diagnosis. Of the possible etiologies, Pneumocystis jirovecii presents as a rare cause of necrotizing cavitary pneumonia, particularly in patients with advanced HIV and AIDS. We present the case of a cavitating lung lesion in a patient with AIDS, as an example of this rare atypical manifestation of Pneumocystis, hoping to increase clinical awareness and assist in prompt diagnosis and management. Case: A 50-year-old male patient presented with progressively worsening cough productive of green blood-tinged sputum of 2-week duration, associated with pleuritic chest pain, night sweats, and diarrhea. Past medical history was significant for AIDS due to HIV-1 with CD-4 count 73/mm3 not on antiretroviral therapy. He was admitted to an outside hospital two months prior for similar symptoms, and chest x-ray at that time demonstrated no acute process (figure 1). On presentation he was tachycardic, tachypneic, and afebrile. Oxygen saturation was 98-100% on room air. Physical examination revealed inspiratory crackles on right upper lung field auscultation with associated dullness to percussion, tenderness in the epigastrium and right upper quadrant of the abdomen, and oral thrush. CXR demonstrated diffuse airspace opacification throughout the right upper lobe (RUL), with internal lucent areas consistent with cavitation (figure 2). A follow-up chest computed tomography (CT) with intravenous contrast demonstrated cavitation of the RUL, with cystic spaces and air fluid levels (figure 3,4). Sputum culture was grossly contaminated with oropharyngeal flora. Three consecutive acid-fast bacilli (AFB) sputum smears were initially negative. Pneumocystis qualitative PCR was positive, with B-D glucan assay (Fungitell) of 239 pg/mL (ref range: \u3c60 pg/mL). Histoplasma urine antigen was negative. Cryptococcus serum testing was negative. A bronchoscopy with bronchoalveolar lavage (BAL) demonstrated findings consistent with necrotizing cavitary pneumonia. Pneumocystis was again identified on qualitative PCR. Anaerobic and aerobic culture, AFB culture, Nocardia, Legionella, and fungal culture were negative. No malignant cells were visualized. After 4 weeks of incubation, the AFB culture from sputum sampling on admission demonstrated Mycobacterium avium complex (MAC) growth in 1 of 3 samples consistent with contamination.Discussion: This case highlights the complexity of cavitary pulmonary lesion diagnosis in a patient with AIDS. The diagnosis of Pneumocystis pneumonia (PCP) was made through sputum and BAL qualitative PCR testing, with fungitell assay testing. The atypical radiographic presentation necessitated exclusion of alternate diagnoses. The possibility of superimposed anaerobic infection was not entirely ruled out, however the location of cavitation with multiple negative cultures on sputum and BAL sampling made this less likely.Conclusion: Pneumocystis jirovecii can present as a necrotizing cavitary pneumonia, and increased awareness regarding this atypical and rare presentation is critical for accurate diagnosis and prompt management.https://scholarlycommons.henryford.com/merf2019caserpt/1045/thumbnail.jp

COVID-19 Presenting with Neurological Symptoms

Introduction: Healthcare officials of Wuhan City in China became aware of several pneumonia cases with an unknown etiology in December 2019. A novel coronavirus, SARS-CoV-2, was identified as cause of the disease named Coronavirus disease-19 (COVID-19). SARS-CoV-2 enters cells through a receptor found on pneumocytes, and there is also evidence this receptor is located on neurons and glial cells. Recently, the neurological manifestations of COVID-19 have been described. However, to our knowledge, there are currently no known cases of COVID-19 presenting as cerebellar dysfunction and essential tremor, which we describe here. Case: A 77-year-old obese man with a past medical history of hypertension and essential tremor presented with decreased mentation, episodes of blank staring, bradykinesia, and worsening bilateral hand tremors. He also reported shortness of breath with a cough productive of clear sputum. Physical exam was within normal limits except for tachypnea requiring 4 liters of oxygen, a bilateral essential tremor and dysmetria. All labs were within normal limits. A chest x-ray suggested multifocal pneumonia. A Computed Tomography (CT) scan demonstrated scattered ground glass opacities and multi-lobar pneumonia, while a CT scan of the head was negative. Treatment was initiated for community acquired pneumonia with azithromycin and ceftriaxone. Upon admission, the patient continued to be orientated only to person and place. Subsequent laboratory investigation demonstrated elevated ferritin (1,200 ng/mL), Lactate Dehydrogenase (613 IU/L), C-Reactive Protein (13.2 mg/dL), and low procalcitonin ( Discussion: The respiratory systems – cough, sputum production, and dyspnea – and imaging findings of COVID-19 are well described.7–11 The presented case is unique because, while the patient did display the established symptoms of COVID-19, he also had encephalopathy, bilateral essential tremor, and cerebellar dysfunction. Neurological imaging was negative, and patients with these symptoms would traditionally receive further investigation. Patients with neurological dysfunction secondary to COVID-19 can demonstrate some significant findings on magnetic resonance imaging (MRI) such as enhancement in the leptomeningeal region and bilateral frontotemporal hypoperfusion on perfusion imaging.5However, because of his concomitant respiratory symptoms, and in context of the ongoing COVID-19 pandemic, our patient did not receive further neurological imaging as there was a high index of suspicion. The central and peripheral nervous system manifestations of COVID-19 are documented, but this case demonstrates that COVID-19 can result in cerebellar dysfunction as well. Conclusion: COVID-19 is caused by SARS-CoV-2, a virus that enters host cells via a receptor primarily found on pneumocytes but also neurons and glial cells. As such, the most common presenting symptoms are respiratory. Here we present a case of a patient presenting with neurological dysfunction in addition to the established respiratory symptoms. Highlights: Our patient presented with encephalopathy, worsened essential tremor, and cerebellar dysfunction, which is an unusual presentation of COVID-19.-In an ongoing pandemic, it is important to have a high index of suspicion, even when patients present with atypical symptoms -It is unclear at this time if hydroxychloroquine and azithromycin should be the treatment of choice for COVID-19, but the regimen was successful in this patient.https://scholarlycommons.henryford.com/merf2020caserpt/1049/thumbnail.jp

A Curriculum for Enhancing Physician Teaching Skills: The Value of Physician-Educator Partnerships

Developing as a physician requires an enormous amount of complex training, and quality of instruction greatly affects training outcomes. But while physicians are expected to teach trainees within the clinic, they often do not receive formal training in effective instructional practices. Providing faculty development programs is one way that institutions can help physicians develop teaching skills, but these programs often are developed without the input of educational specialists and not based in educational theory. In this methodology paper, we describe a 5-module curriculum that was developed in a cross-disciplinary collaboration between instructional designers and physician faculty. By merging educational and medical expertise and using adult learning theory with the Charlotte-Danielson educational framework, an essentials for clinical teaching educational endorsement program (ECTEEP) was created as a feature of the institutional curriculum within a large, urban teaching hospital. Here we describe how the program was developed through a physician-educator partnership, outline the program\u27s key content, and highlight essential aspects of successful implementation. The ECTEEP incorporates active learning approaches within an abbreviated format, distilling 5 critical aspects of effective teaching that are relevant to the clinical environment: cultural humility and safe learning environments, instruction practices for engaging learners, instruction and assessment strategies, receiving and giving feedback, and mentorship and coaching. A central feature of the program is that facilitators actively model the teaching behaviors they are conveying, which underscores the critical importance of facilitator preparation and skill. Our curriculum is offered here as a basic template for institutions that may want to establish a program for enhancing physician teaching skill

Project #37: Management Guidelines for Patients with COVID-19: Rapid Cycle Improvement

Project’s purpose is to rapidly devise, continually improve, educate, and implement a live and changing COVID-19 management guideline based upon emerging best available evidence. This project also aims to optimize the care of patients with COVID-19 and improve patient outcomes.https://scholarlycommons.henryford.com/qualityexpo2022/1004/thumbnail.jp

Diffuse idiopathic skeletal hyperostosis causing progressive dysphagia

Background: There are a multitude of extrinsic and intrinsic etiologies of dysphagia, including mechanical obstruction, infections, neuromuscular conditions, and malignancies. Diffuse idiopathic skeletal hyperostosis (DISH) of the cervical and upper thoracic spine, a relatively common finding in spinal imaging, is rarely associated with upper esophageal pathology. We present a case of progressive dysphagia due to DISH. Case Presentation: A 64-year-old male presents to the emergency department with a three-day history of chest pain associated with cough, and vomiting. The patient admits to an extensive history of alcohol abuse with current everyday use and a 30-year smoking history. Upon further review, the patient noted increased weight loss and progressive inability to swallow liquids and solids associated with concurrent retrosternal chest pain and occasional vomiting. Initial cardiac workup was negative for any acute abnormalities. Follow up right upper quadrant ultrasound and HIDA scan were negative for acute cholecystitis. Dynamic swallow study showed silent aspiration of all consistencies. Follow up esophagogastroduodenoscopy was concerning for external compression of the esophagus at the upper esophageal sphincter. Subsequent imaging of the spine revealed bulky, flowing osteophytes in the cervical and thoracic spine consistent with diffuse idiopathic hyperostosis with varying degrees of airway and esophageal indentation throughout. At the time of publication, patient remained nil per os with nutrition supplementation through a nasogastric tube pending neurosurgical evaluation. Conclusions: Diffuse idiopathic skeletal hyperostosis, also known as Forestier’s disease, is an abnormal calcification of the anterolateral aspects of the spinal ligaments, and less commonly, the appendicular skeleton. The formation of bridging osteophytes through at least four consecutive vertebral bodies is required for diagnosis. Patients with DISH are usually asymptomatic, but can present with limited cervical mobility, or neck and back pain. Dysphagia is a relatively uncommon finding in DISH, but has been estimated to occur in up to 20% of cases. Mild cases are treated conservatively with physical therapy and pain control; however, progressively worsening symptoms or focal deficits require surgical management. Studies have shown recurrence of osteophytic lesions after surgical management is very common.https://scholarlycommons.henryford.com/merf2019caserpt/1082/thumbnail.jp