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This case series highlights the occurrence of hemodynamically significant ventricular septal defects (VSDs) in two patients presenting with ST-elevation myocardial infarction (STEMI) during the COVID-19 pandemic. This paper aims to emphasize the delayed presentation of cardiac emergencies, such as STEMI, due to concerns about contracting COVID-19. This delay has led to an increased risk of rare complications, including VSD, associated with STEMI. The first case involves a 92-year-old male with a history of hypertension, hyperlipidemia, chronic kidney disease, and coronary artery disease. He presented with acute chest pain, and diagnostic tests revealed ST elevations and a VSD. Despite intervention efforts, including hemodynamic support, the patient's condition deteriorated, and he passed away due to advanced age and high surgical risk. The second case involves a 62-year-old female with a medical history of diabetes, hypertension, and hyperlipidemia. She presented with left-sided chest pain, and an angiogram revealed a mid-right coronary artery stenosis and a thrombus. During the procedure, the patient experienced hypotension, requiring hemodynamic support. Subsequent evaluations identified a large VSD with right ventricular dysfunction. The patient underwent a series of interventions, including a ventricular assist device and VSD closure, but experienced multi-organ failure and ultimately passed away. VSDs following acute myocardial infarction (MI) are rare but life-threatening complications. Early revascularization is crucial in preventing the development of VSDs. These cases demonstrate the importance of prompt diagnosis and intervention, as delayed presentation increases the risk of mechanical complications. Surgical closure remains the definitive treatment for postinfarction VSDs.
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Pertuzumab is a targeted therapy drug that is employed in the management of human epidermal growth factor receptor 2 (HER2)-positive breast cancer and works by blocking the ability of cancer cells to receive growth and proliferation signals. Toxic epidermal necrolysis (TEN) is a severe cutaneous manifestation characterized by widespread erythema, necrosis, and bullous detachment of the skin involving more than 10% of the body surface area (BSA) and may be precipitated by an immunologic response to the administration of certain medications. However, TEN development as a consequence of HER2 inhibitor therapy has not been described in the existing literature. A 44-year-old female with a history of metastatic breast cancer to the liver presented with a diffuse blistering rash following a first-time administration of pertuzumab three days prior. Her rash began as painful and pruritic blisters 12 hours after the last infusion of pertuzumab and progressed to involve her arms, chest, groin, and thighs with a positive Nikolsky sign. She was managed supportively with high-dose steroids and antihistamines, and although her hospital course was complicated by hypotension requiring pressor support, she gradually made a full recovery and was released to a rehabilitation facility.
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Anaplastic large-cell lymphoma (ALCL) is an aggressive subtype of non-Hodgkin lymphoma. There are two forms of ALCL: primary and secondary. Primary can be systemic, affecting multiple organs, or cutaneous, affecting mainly the skin. A secondary form occurs when another lymphoma undergoes an anaplastic transformation. ALCL rarely presents as initial symptoms of respiratory failure. In most of these situations, the trachea or bronchial involved with an obstruction was present. We present an unusual case of ALCL where the patient rapidly progressed to acute hypoxic respiratory failure with a patent bronchus and trachea. Unfortunately, the patient rapidly deteriorated and died before diagnosis. Only upon at autopsy, it was found that his lung parenchyma was diffusely involved with ALCL. The autopsy report showed that the patient had CD-30 anaplastic lymphoma kinase (ALK)-negative ALCL diffusely involving all lung fields.
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When considering tumors of the bone, metastatic disease from a distant primary is more common than primary tumors of the bone itself. The commonest sites to which skeletal metastasis occur are in the axial skeleton, and with regard to the appendicular skeleton, metastasis to the forearm bones is uncommon. Almost a third of patients who present with skeletal metastases do not have any evidence of their primary tumor at presentation. We report a case of a 68-year-old female diagnosed with lung adenocarcinoma after presenting with metastatic deposits involving the right radius as the first clinical manifestation of her disease. She presented initially complaining of painful swelling of her right forearm for a duration of one year. Imaging investigations of her right forearm showed an expansile mixed lytic and sclerotic lesion involving the full length of the right radius. A contrast-enhanced computed tomography scan of her chest to investigate the possible site of primary malignancy showed a peripherally located, well-defined, irregularly shaped mass lesion with enlarged mediastinal lymph nodes. A fluorodeoxyglucose positron emission tomography (FDG-PET) bone scan also noted oligometastatic disease in her right proximal humerus. She was started on palliative docetaxel for six cycles with palliative external beam radiotherapy. Although a variety of tumors metastasize to the bone, metastasis to the appendicular skeleton, and in particular the forearm bones, is a rare phenomenon that is poorly described in the existing literature. Skeletal metastasis may also be the primary presenting feature in a minority of cases. Lung cancer is among the more commonly associated primary sites, and further workup should include appropriate imaging to evaluate for a lung primary as well as an FDG-PET/CT or a bone scan to detect occult metastatic disease.
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Introduction Sickle cell anemia (SCA) is a hemoglobinopathy that arises from a point mutation in the beta-globin gene, which causes the polymerization of deoxygenated hemoglobin that leads to a wide variety of clinical complications. Deaths in patients with SCA most commonly arise from renal, cardiovascular disease, infections, and stroke. In-hospital cardiac arrest has been found to be more common in older patients and those on ventilatory life support, among others. This study aims to provide more insight into how SCA affects the risk of in-hospital mortality in post-cardiac arrest patients. Methods The National Inpatient Survey database years 2016 to 2019 was utilized. The International Classification of Diseases, Tenth Revision, Procedure Coding System (ICD-10 PCS) codes for cardiopulmonary resuscitation were used to identify in-hospital cardiac arrest (IHCA) patients. ICD-10 Clinical Modification (CM) codes were used to identify SCA and other medical comorbidities. Categorical data was compared using Person's chi-square test, and continuous variables were compared using the independent samples t-test. Multinomial logistic regression was used to study the effects of SCA on post-arrest in-hospital mortality controlling for age, Charlson comorbidity score, and demographic variables. Binomial logistic regression models for dichotomous variables were utilized in the subgroup and secondary outcomes analysis. Results In patients with IHCA, patients who had SCA were found to have a significantly increased risk of in-hospital mortality adjusted for baseline characteristics and Charlson comorbidity score (OR: 1.16, 95% CI: 1.02-1.32, p=0.0025). Patient characteristics most strongly associated with an increased risk of in-hospital mortality in this cohort were found to be Black race (OR: 1.92, 95% CI: 1.87-1.97, p<0.001) and self-payer status (OR: 2.14, 95% CI: 2.06-2.22, p<0.001). Subgroup analysis revealed only patients with sickle cell disease had a statistically significant increased risk of in-hospital mortality in this cohort (OR: 4.41, 95% CI: 3.5-5.55, p<0.001), and patients with sickle cell trait did not. Conclusion In patients with IHCA, SCA is associated with an increased risk of in-hospital mortality. This risk was confined to patients with sickle cell disease and not patients with sickle cell trait.
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INTRODUCTION: Acute myeloid leukemia [AML] is a heterogenous group of primary hematopoietic neoplasms arising from myeloid precursor cells. Up to 50% of patients failed to achieve remission with initial therapy and go on to develop refractory AML. Whenever possible, enrollment in a clinical trial in view of the paucity of evidence surrounding a clearly superior treatment modality is recommended, and the therapy which provides the best chance for cure post remission is allogeneic hematopoietic stem cell transplantation [HCT], with much of everyday clinical decision-making in relapsed/refractory (R/R) AML surrounding the choice of the least toxic regimen that could achieve remission and enable prompt HCT. DISCUSSION: We discuss a variety of treatment modalities employed in the R/R AML setting beginning with traditional cytotoxic regimens. We then turn our attention to targeted therapies that have shown efficacy in specific patient populations such as the IDH inhibitors and FLT3 inhibitors and lastly, we turn our attention to immunotherapeutic agents employed in the R/R in the setting, such as CD33 inhibitors and bispecific antibodies. CONCLUSION: It appears increasingly clear that approaching AML as a homogenous disease entity is unsatisfactory in view of the variations in such disease factors as cytogenetic and molecular markers, age, and disease severity at presentation; all of which contribute significantly to heterogeneity of the disease. Moving forward, treating AML would likely require tailored therapy following advances in technology such as molecular profiling, drug sensitivity and resistance testing.
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Leucemia Mieloide Aguda , Humanos , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Transplante de Células-Tronco Hematopoéticas/métodos , Leucemia Mieloide Aguda/tratamento farmacológico , Leucemia Mieloide Aguda/terapia , Recidiva , Terapia de SalvaçãoRESUMO
Myocarditis refers to inflammation of the heart muscle and may occur individually or together with pericarditis, which refers to inflammation of the saclike tissue layer that surrounds the heart. They may have infectious or non-infectious etiologies. Campylobacter jejuni, a major cause of gastroenteritis worldwide, may also cause myocarditis in rare situations. We present two cases highlighting this rare complication of diarrheal disease caused by Campylobacter jejuni infection and subsequent development of myocarditis. Both patients presented with chest pain and multiple episodes of watery diarrhea, with initial EKGs showing ST segment changes, as well as elevated inflammatory markers and elevated troponins. GI panels for both patients were positive for Campylobacter jejuni. Based on their presentations and investigative findings, they were diagnosed with myocarditis secondary to Campylobacter infection, and their symptoms subsided with appropriate management. It is unclear if the myocardial damage, in this case, is a direct effect of the toxin on cardiac myocytes or secondary to an immunologic phenomenon. Regardless, Campylobacter jejuni-associated myocarditis remains a rare phenomenon and needs to be considered in the differential of patients presenting with concurrent chest pain and diarrheal symptoms.
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Membranous nephropathy (MN) is an autoimmune disease resulting in nephrotic syndrome. Neural epidermal growth factor-like 1 protein (NELL-1) has been shown to cause a rare form of MN that is more likely to be associated with malignancy. We present a case of a 73-year-old female who was found to have a NELL-1-associated segmental MN. She presented complaining of generalized weakness, chills, and poor appetite, worsening over a one-week duration. Her kidney functions were noted to be markedly deranged, with a computed tomography scan of the abdomen showing evidence of chronic kidney disease. Further testing confirmed heavy proteinuria, although the etiology was still uncertain. A kidney biopsy revealed granular subepithelial immunoglobulin G deposits with subsequent immunohistochemical staining for NELL-1 antigen being positive. She improved with supportive care over the next few days. Despite an extensive workup, no underlying malignancy was found. NELL-1 is a rare yet recognized antigen target for the development of MN. Up to a third of patients with NELL-1-associated MN have associated cancer, thus requiring evaluation for underlying malignancy in this cohort.
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Background One of the biggest hurdles in treating pediatric patients is managing dental fear and anxiety. Some factors that contribute to an increase in dental anxiety are fear of pain, the presence of unknown individuals, a change in the setting of an environment, and separation from parents. Aim The aim of this study was to evaluate dental fear and anxiety in pediatric patients, between the ages of 6 and 12 years, visiting private and public dental hospitals using the Children's Fear Survey Schedule-Dental Subscale (CFSS-DS). Methods A total of 280 children, 140 in a private dental hospital setting and 140 in a public dental hospital setting, were enrolled in this study. The purpose of the study was explained to the accompanying guardian of the patient and written consent was taken. The CFSS-DS was explained verbally in Urdu and the questionnaire was given to guardians alongside the patients which they were asked to fill out following their dental treatment. Result The data obtained from the questionnaires were analyzed using the unpaired t-test. The highest dental fear mean scores and standard deviation in a private dental hospital were for "choking" (3.25 ± 1.21), "the noise of the dentist drilling" (3.24 ± 1.04), and "having somebody put instruments in your mouth" (3.19 ± 1.06), whereas, for a public dental hospital, the highest fear score was recorded in "choking" (3.17 ± 1.69), "injections" (3.07 ± 1.72), and "people in white uniforms" (1.90 ± 1.21). Conclusion The study showed a higher prevalence of dental fear and anxiety in a private dental setting when compared to a public dental setting. Factors responsible for an increase in dental fear need to be assessed for each patient and then treatment given accordingly.
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The BK virus is a polyomavirus known to particularly affect transplant recipients. An important complication caused by BK virus infection in bone marrow transplant patients is hemorrhagic cystitis. We present a 31-year-old male with a history of bone marrow transplantation complicated by graft-versus-host disease (GVHD) and was diagnosed with BK virus-related hemorrhagic cystitis. He presented with gross hematuria and suprapubic and penile pain for one week. He has a significant past medical history of acute B-cell lymphocytic leukemia for which he has successfully undergone allogenic bone marrow transplantation, which was complicated by GVHD. Imaging revealed significant bladder wall thickening which prompted an evaluation for BK virus-induced hemorrhagic cystitis. A urinary specimen was sent for BK virus polymerase chain reaction (PCR) which was strongly positive, confirming the infection. He was managed supportively throughout his hospitalization and improved with symptomatic management alone. Our case demonstrates one of the main complications caused by the BK virus in allogeneic bone marrow transplant patients in the setting of GVHD and is an important differential to keep in mind when treating patients presenting with hematuria after bone marrow transplantation.
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Adrenocortical carcinoma (ACC) is a rare endocrine malignancy with a poor prognosis. Surgical resection may be curative if localized disease is identified, although recurrence is common. Research shows that the use of adjuvant therapeutic regimens such as EDP-M (combination of mitotane, etoposide, doxorubicin, and cisplatin) in high-risk patients has survival benefits. A 75-year-old female was incidentally found to have a right adrenal heterogeneous internal enhancement measuring 5.0 x 3.7cm. The workup confirmed autonomous adrenal production of corticosteroids and she was referred to surgery for an adrenalectomy. A T2 ACC with positive margins and lympho-vascular invasion was resected, following which she was started on external beam radiation followed by four cycles of carboplatin and etoposide. Despite initial treatments, she was diagnosed with refractory metastatic disease at subsequent follow-ups. Pembrolizumab immunotherapy was started, but disease progression continued, and she was eventually transitioned to mitotane 1g twice daily. She continued to worsen and was eventually transitioned to hospice care. The management of ACC remains diagnostically challenging, especially because most patients do not present until an advanced stage of disease. Surgery is commonly employed with a curative intent, and opinions regarding adjuvant cytotoxic therapy and/or radiotherapy remain mixed.
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Hemophagocytic lymphohistiocytosis (HLH) is characterized by immune dysregulation with extensive inflammation and tissue destruction due to abnormal immune activation. Post-COVID-19 patients who have recovered with negative serologic tests may also present with secondary HLH, an unusual finding that our case demonstrates. A 73-year-old male with a notable past medical history of fall COVID-19 infection approximately 11 months prior presented initially to emergency services with a chief complaint of high fevers, lethargy, and progressive changes in mentation gradually progressive over the last 5 months' duration. This presentation was concerning for HLH in view of the patient's high H score and clinical suspicion for HLH, and he was initiated on dexamethasone 20 mg daily. intravenous immune globulin (IVIG) protocol was also trialed, despite which the patient continued to deteriorate before expiring during the course of the hospitalization. sHLH following COVID-19 infection remains a poorly understood phenomenon. The severity of the COVID-19 infection does not appear to be related to one's predisposition to develop sHLH. The mortality of HLH remains high even with appropriate therapy.
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Acute liver failure (ALF) is a potentially fatal condition that adversely affects multiple organs and has a high mortality rate. ALF due to hepatic infiltration is rare even though it is a common place for cancer to metastasize. Patients with ALF usually present with abdominal pain and elevated liver function tests. We report a case of a 65-year-old male that developed ALF due to miliary liver metastasis of non-small cell carcinoma from an unknown primary origin. The patient initially presented with a couple of episodes of coffee-ground emesis and epigastric pain. Upon further evaluation, along with computed tomography (CT) scans and liver biopsy, the diagnosis was established. The patient passed away on the 12th day of hospitalization.
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Multiple myeloma (MM) is a neoplasm of plasma cell origin characterized by the proliferation of immunoglobulin-producing plasma cells in the bone marrow. Extramedullary disease (EMD) occurs in approximately 10% of patients with MM. Myelomatous pleural effusion (MPE) is a possible manifestation of EMD and has been associated with a poorer prognosis. A 66-year-old female was evaluated after an abnormal serum protein electrophoresis that showed a 2.1 g/dL M-spike in the gamma region, highly suggestive of plasma cell dyscrasia. Imaging subsequently confirmed the bony metastasis. A bone marrow biopsy confirmed plasmablastic MM, and she was started on chemotherapy. She presented three months later with bilateral pleural effusions, with cytology revealing neoplastic plasmacytoid cells. Despite transitioning to dexamethasone, cyclophosphamide, etoposide, and cisplatin (the V-DCEP regimen) due to disease progression, her myeloma remained refractory to treatment, and she expired one month later. MPE in MM is associated with a poor prognosis, with a median overall survival (OS) of 13 months in MPE compared to 37 months in other EMDs. A higher tumor burden and greater multisite extra-medullary lesions are also characteristics of MPE in MM. There is no standard of care for the management of EMD, and salvage regimens such as RVD and V-DCEP are commonly employed. The management of MM with EMD remains a challenge, and more investigation is required before effective treatment regimens may be employed in this setting.