Antithrombotic Therapy for Heart Failure in Sinus Rhythm - A Concise Review.

Heart failure (HF) is a common disorder associated with significant morbidity and mortality. It can increase the risk of thromboembolic events, which subsequently lead to increased risk of stroke, ischemic heart disease, thromboembolism, and death. Antithrombotic therapy has been investigated as a potential management strategy for HF patients in sinus rhythm, but its efficacy remains uncertain. Current guidelines do not recommend the routine use of antithrombotics in patients with HF in sinus rhythm without any other indication for their use. Several randomized controlled trials have investigated the efficacy of antithrombotics in HF patients in sinus rhythm. This article provides a concise review of the existing literature to assess the evidence supporting the use of antithrombotics in HF patients in sinus rhythm. The use of warfarin or other anticoagulants has demonstrated a lower risk of stroke but an increased risk of bleeding. The studies demonstrate that anticoagulant therapy in HF patients in sinus rhythm does not provide significant benefits in terms of overall ischemic events or death.

Intravenous Immunoglobulin-Associated Severe Hemolytic Anemia.

Intravenous immunoglobulin (IVIG) is used to treat immunodeficiency conditions, neuro-immunological, infection-related, autoimmune, and inflammatory disorders and is typically well tolerated. A hematological adverse reaction such as hemolytic anemia and neutropenia is known to occur with IVIG, which is usually transient and subclinical. However, severe hemolytic anemia is known to occur in some cases. We present a case of a 66-year-old man who developed severe symptomatic hemolytic anemia after receiving IVIG for acute inflammatory demyelinating polyneuropathy (AIDP). The patient had known risk factors such as non-O blood group, high cumulative dose of IVIG, and underlying autoimmune condition, which would have put him at high risk for developing hemolytic anemia after IVIG. Therefore, it is prudent for clinicians to have increased awareness regarding the potential for severe hemolysis and closely monitor these patients with risk factors after treatments to identify this adverse reaction before more severe complications occur.

Stroke in a Young Patient Due to Moyamoya Disease.

Moyamoya is an uncommon, chronic vasocclusive disease of brain which affects the terminal portion of the internal carotid artery. Moyamoya disease is a disease of young with peak incidence of around 10-40 years. It can present as a transient ischemic attack (TIA), stroke or intracerebral hemorrhage in the pediatric or young adults. Treatment usually includes medical therapy or surgery. Surgical treatment generally involves use of external carotid system for blood supply which are spared in this disease process. We present a case of a young male patient with stroke from Moyamoya disease, who underwent successful EDAS (encephaloduroateriosynangiosis) procedure with complete resolution of his stroke symptoms.

A Retrospective Study Comparing the Effect of Conventional Coagulation Parameters Vs. Thromboelastography-Guided Blood Product Utilization in Patients With Major Gastrointestinal Bleeding.

Background: The use of thromboelastography (TEG) has demonstrated decreased blood product utilization in patients with specific etiologies of major gastrointestinal bleeding (GIB), such as variceal and non-variceal bleeding in cirrhosis patients; however, in a non-cirrhosis patient with GIB, there is far less evidence in the literature. Our retrospective study compares the effect of TEG-guided blood product utilization in patients with major GIB with all etiologies, including cirrhosis, admitted to medical intensive care unit (MICU). Methods: A retrospective chart review was conducted on patients admitted to the MICU of a tertiary academic medical center diagnosed with GIB using ICD-9/10 codes from 2014 to 2018. A total of 1,889 patients were identified, and validation criteria such as "GI or hepatology consult note", type and screen, pantoprazole, or octreotide drip" were used, which resulted in 997 patients, out of which 369 had a diagnosis of cirrhosis. Propensity score matching was done for baseline variables (age, sex, and race), ICU length of stay, hospital length of stay, ventilator days, and vasopressor use. As a result, 88 patients were included in the final analysis, with 44 in TEG and 44 in non-TEG group. A sub-group analysis was done in 46 patients with cirrhosis, 23 in TEG group and 23 in non-TEG group after propensity score matching. Results: There was significantly higher total blood volume (4,207 mL vs. 2,568 mL, P = 0.04) in the TEG group as compared to the non-TEG group, including total volume of cryoprecipitate (80 mL vs. 55 mL, P = 0.03) and total volume of platelet (543 mL vs. 327 mL, P = 0.03). In the cirrhosis sub-group, there was no significant difference in the amount of blood products transfused between the two groups. Conclusion: This study revealed that TEG is not superior to conventional coagulation parameters in limiting the volume of blood product transfusion in major GIB patients in ICU settings.

Interesting case of incidental diagnosis of pulmonary embolism by endobronchial ultrasonography.

Pulmonary embolism (PE) is the obstruction of the pulmonary artery or its branches, usually by a thrombus that originates in the lower extremity veins. PE is associated with high mortality risk. Here, we present the case of a patient who initially presented with dysphagia. Chest radiography revealed a lung nodule. Endobronchial ultrasonography (EBUS) was performed to evaluate the nodule, which revealed a pulmonary embolus. Subsequently, CT angiography of the chest was performed to confirm the diagnosis of PE. Anticoagulation therapy was initiated. The biopsy results were positive for lung adenocarcinoma. There are only few reported cases of PE diagnosed using EBUS. Here, the patient had not presented with the signs and symptoms of PE. Had PE not been diagnosed by EBUS, our patient could have potentially had a disastrous outcome. Moreover, this case shows that EBUS may be used for diagnosing PE.

Experience With Dexmedetomidine Use in the Treatment of Dysautonomic Crisis in Familial Dysautonomia: An Off-Label Use.

Familial dysautonomia is a rare genetic neurodevelopmental disorder characterized by episodes of hyperautonomic state known as dysautonomic crises. The features of dysautonomic crises are hypertension, tachycardia, vomiting, sweating, flushing, and behavioral changes. The etiology of such crises is supposed to be a consequence of the inability to control sympathetic overflow due to damage to the afferent neurons carrying baroreceptor inputs to the central nervous system. A 19-year-old male with a known history of familial dysautonomia and frequent dysautonomic crises presented to the Emergency Department with intractable nausea and vomiting for six hours. He was hypertensive and tachycardic on presentation. The patient had tried oral labetalol and clonidine at home with no improvement. In the emergency room, the patient received intravenous labetalol, diazepam, and clonidine which were ineffective. He was then treated with intravenous dexmedetomidine, and his symptoms resolved within a few hours. The patient was discharged home on the same day. The mainstay of treatment for dysautonomic crises is benzodiazepines and clonidine. The use of these treatment modalities has its challenges. Here, we present a case of a dysautonomic crisis that was resistant to the conventional treatment, treated safely and successfully with dexmedetomidine.

A Case of Severe Hypoxia Caused by Phenazopyridine-Induced Methemoglobinemia: A near Fatal Event from Over-the-Counter Medication Use.

Methemoglobinemia is a rare blood disorder characterized by the oxidation of heme iron from ferrous (Fe2+) to ferric (Fe3+) state, which increases oxygen affinity and impairs oxygen release to the tissue causing hypoxia. It can be congenital or acquired; however, most cases are acquired and caused by exogenous substances such as medications, chemicals, and environmental substances. Phenazopyridine is an over-the-counter urinary analgesic medication commonly used for symptomatic relief of dysuria and has been reported to cause methemoglobinemia. However, only a handful of cases of phenazopyridine-induced methemoglobinemia have been reported. We present a case of an 89-year-old female who presented with severe hypoxia, shortness of breath, headache, nausea, and dizziness caused by phenazopyridine-induced methemoglobinemia. She was found to have a methemoglobin level of 21.5% and was treated with methylene blue, leading to a rapid improvement of her symptoms. She was taking one over-the-counter phenazopyridine 200 mg tablet three times daily for two weeks for her chronic dysuria. This case highlights the need to have a high index of suspicion of phenazopyridine-induced methemoglobinemia in a patient presenting with unexplained shortness of breath with a history of phenazopyridine use as it could lead to severe methemoglobinemia with hypoxia that could potentially be fatal if not promptly diagnosed.

An Unusual Presentation of Tuberculosis With Dysphagia.

About a quarter of the world's population is infected with tuberculosis (TB). It is one of the leading causes of death worldwide. However, the prevalence of TB in the United States is rare. Pulmonary TB is the commonest form of TB. Most patients with TB present with pulmonary symptoms. Extrapulmonary TB usually presents with symptoms related to the organ system involved and can present with very unusual symptoms. TB presenting with dysphagia is uncommon, and spinal TB presenting with dysphagia is very unusual. A 57-year-old woman presented to the emergency department with a four-month history of dysphagia and chest pain. She was undergoing an outpatient workup of her symptoms that included esophagogastroduodenoscopy, gastric emptying study, and computed tomography (CT) scan of the chest, which showed incidental findings of a focus in the thoracic spine. It was followed by a bone scan, and the results were concerning for malignancy. She was awaiting an oncology appointment when she presented to us. A basic workup after her presentation that includes complete blood count, comprehensive metabolic panel, troponin, chest x-ray, and electrocardiogram was unrevealing. Magnetic resonance imaging (MRI) of the thoracic spine showed findings suggestive of tuberculous spondylitis, tuberculous paraspinal, and prevertebral abscesses. Chest CT was repeated, which showed mass effect and erosion on the posterior esophageal wall with anterior displacement of the esophagus. Tissue biopsy revealed acid-fast bacilli (AFB) on AFB stain, and the culture grew Mycobacterium tuberculosis. She was successfully treated with the antitubercular regimen of rifampin, isoniazid, ethambutol, and pyrazinamide. TB can present with a myriad of symptoms, and although rare, it can present with symptoms like dysphagia. In patients with a history of travel to or immigration from an endemic region, previous infection, and immunosuppression, TB should be considered as one of the differential diagnoses even for unusual symptoms like dysphagia.

Use of Magnetic Resonance Imaging to Identify Immune Checkpoint Inhibitor-Induced Inflammatory Arthritis.

Importance: Immune checkpoint inhibitors (ICIs) have transformed the treatment paradigm for an ever-increasing number of cancers. However, their use has also led to the emergence of immune-related adverse events, such as ICI-induced inflammatory arthritis. A reproducible, reliable, and accessible modality is needed to assess and distinguish early ICI-induced inflammatory arthritis and help in management. Magnetic resonance imaging (MRI) of joints may be helpful for early diagnosis, guiding therapeutic decision-making, and identifying patients at high risk for erosive disease. Objective: To assess the role of MRI of joints in patients with ICI-induced inflammatory arthritis. Design, Setting, and Participants: This retrospective case series included patients enrolled at the National Institutes of Health Clinical Center in Bethesda, Maryland. Patients were evaluated by the rheumatology consultation service between December 27, 2016, and May 28, 2019. A retrospective health record review was performed to determine demographic characteristics, clinical characteristics of inflammatory arthritis and malignant tumors, and imaging findings. Inclusion criteria were patients who were enrolled on various institutional review board-approved protocols of ICIs, developed joint-related symptoms, and had MRI data for at least 1 joint. Data were analyzed from June 1, 2019, to September 1, 2019. Exposures: Undergoing MRI of at least 1 joint. Main Outcomes and Measures: All MRIs were reviewed for synovitis, tenosynovitis, bone marrow edema, and soft tissue conditions. Results: A total of 8 patients (mean [SD] age, 58.8 [5.2] years; 6 women and 2 men) between the ages of 50 and 65 years who were undergoing ICI therapy for a variety of malignant tumors were included in this study. Only 1 patient was receiving combined ICI therapy. The results of 13 separate MRI examinations were reviewed. The most commonly performed MRIs were of the hands and wrists (9 MRIs), followed by knee examinations (3 MRIs). Tenosynovitis and synovitis were frequently seen in the hands and wrists. Bone marrow edema and erosions were also found in 3 patients, suggesting early damage. In larger joints (ie, knees and ankles), joint effusions and synovial thickening were characteristic. Most patients (5 patients) were treated with corticosteroids and had good responses. In patients with high-risk features on MRI imaging (eg, bone marrow edema, erosions), disease-modifying antirheumatic drug therapy was also discussed as a treatment option. Conclusions and Relevance: These findings suggest that advanced imaging may help to distinguish ICI-induced inflammatory arthritis from other causes of joint pain, aid in identifying patients at increased risk of joint damage, and provide utility in monitoring inflammatory arthritis treatment response in patients receiving ICI therapy.

Improving Blood Transfusion Practices in a Community Hospital Setting: Our Experience with Real-Time Clinical Decision Support.

There is good evidence that 50% or more of red blood cell (RBC) transfusions are unnecessary. To curtail inappropriate RBC transfusions at our hospital, real-time clinical decision support was implemented in our electronic medical record (EMR) that alerts clinicians to the patient's most recent pretransfusion hemoglobin value upon order entry and provides Best Practice Advisory. This is a soft pop-up alert which is activated when the hemoglobin exceeds 7 g/dL. The ordering clinician can either honor (by cancelling the order) or override the alert. We studied the impact of the alert on blood utilization during a 3-month period (November 2016 to January 2017). For patients who were transfused despite the alert, a retrospective review of the medical chart was performed to determine whether or not the transfusion was clinically indicated. During the study period, 178 of the 895 RBC transfusion orders (20%) triggered the alert. After excluding duplicates, 144 orders were included in our analysis. Most of these orders (124/144, 86%) were carried out despite the alert. According to our chart review, 48% of the alert transfusions could be considered inappropriate, with hemodynamically stable, asymptomatic anemia being the leading indication. Of clinical services, orthopedic surgery had the highest rate of overriding the alert with no clinical justification (70%). The number of RBC transfusions dropped from 313.5 units per month (preintervention period) to 293.2 units per month (postintervention period)-a 6.5% decrease. Real-time clinical decision support may reduce the number of inappropriate RBC transfusions in a community hospital setting, though in our study, the decrease in blood utilization (6.5%) did not reach statistical significance.

Pharmacotherapy Pearls in Rheumatology for the Care of Older Adult Patients: Focus on Oral Disease-Modifying Antirheumatic Drugs and the Newest Small Molecule Inhibitors.

Providing safe and effective pharmacotherapy to geriatric patients with rheumatologic disorders is challenging. Multidisciplinary care involving rheumatologists, primary care physicians, and other specialties can optimize benefit and reduce adverse outcomes. Oral disease-modifying antirheumatic drugs, including methotrexate, hydroxychloroquine, sulfasalazine, and leflunomide, and the small molecule inhibitors tofacitinib and apremilast have distinctive monitoring requirements and specific adverse reaction profiles. This article provides clinically relevant pearls for use of these interventions in older patients.

RS3PE revisited: a systematic review and meta-analysis of 331 cases.

OBJECTIVES: Remitting seronegative symmetrical synovitis with pitting oedema (RS(3)PE) syndrome is a rare inflammatory arthritis, characterised by symmetrical distal synovitis, pitting oedema of the hands and feet, absence of rheumatoid factor, and favourable response to glucocorticoids. The aim of our study is to further delineate the clinical and laboratory features, and response to treatment. METHODS: We performed a systematic electronic search of Medline, PubMed, EMBASE, ACR and EULAR databases for case reports, case series, and related articles of RS(3)PE. Statistical analysis was done comparing categorical variables with Chi-square tests and frequencies of means via t-tests. Binary logistic regression analysis was performed to identify predictors of erosions, recurrence, malignancy and rheumatologic disorders. RESULTS: 331 cases of RS(3)PE were identified from 121 articles. RS(3)PE was found in older patients (71±10.42 years) predominantly in males (n= 211, 63.36%), was symmetrical (n=297/311, 95.50%) involved the hands (n=294/311, 94.53%) A concurrent rheumatologic condition was reported in 22 cases (6.65%), and malignancy in 54 cases (16.31%). Radiographic joint erosions were found in 5.5%. Most patients responded to medium-dose glucocorticoids (16.12±9.5 mg/day). Patients with concurrent malignancy requiring non-significantly higher doses of prednisone (18.12 vs. 15.76 mg, p 0.304) and higher likelihood of recurrence of disease (OR 4.04, 95% CI 1.10-14.88, p=0.03). CONCLUSIONS: The symptoms and unique findings that make up RS(3)PE appear to represent a steroid-responsive disease that may be a harbinger of an underlying malignancy. More study is needed to understand the molecular origins of RS(3)PE in order to determine whether it is a separate disease process. Patients with concurrent cancer tend to have more severe presentations and higher rates of recurrence.

Effect of mycophenolate mofetil on the white blood cell count and the frequency of infection in systemic lupus erythematosus.

Leukopenia is a common manifestation of SLE. Addition of immunosuppressive therapy in a SLE patient who is already leukopenic is a clinical concern. It could worsen leukopenia, increase the risk of infection, or both. The aim of this study was to analyze the immediate effect of mycophenolate mofetil on the white blood cell count and the rate of infection in SLE patients. Two hundred and forty-four patients within the Hopkins Lupus Cohort who were newly started on mycophenolate mofetil were included in the study. The white blood cell count and interval infection history on the day mycophenolate mofetil was started were compared with the white blood cell count and interval infection history at the next visit. The study was based on 244 patients who began taking mycophenolate mofetil in the cohort. The study population included 47 % African Americans, 44 % Caucasians, and 9 % other ethnicities. There was a slight but not statistically significant increase in the white blood cell count (6.63 vs. 7.01), after starting mycophenolate mofetil. Patients with a baseline white blood cell count <3000/mm(3) did have a statistically significant increase in the white blood cell count after starting mycophenolate mofetil (2.57 vs. 5.13, P = 0.0047). We also found a statistically significant increase in the risk of bacterial infection (but not viral infection) after starting mycophenolate mofetil (4 vs. 9 %, P = 0.0036). Leukopenia does not worsen with mycophenolate mofetil. However, mycophenolate mofetil appears to slightly increase the rate of bacterial (but not viral) infection.