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Immunoglobulin G4-related disease (IgG4-RD) is a fibroinflammatory autoimmune disease characterized by tissue infiltration by dense lymphoplasmacytic infiltrate composed of T cells, activated B-cells, and plasma cells expressing IgG4 and has varied presentations with similar histopathology. It can involve visceral organs, glands, aorta, lymph nodes, and retroperitoneal tissue. In our case, a 68-year-old male with a past history of Hodgkin's lymphoma and in remission presented for investigation of polyclonal gammopathy. Serum electrophoresis showed increased free kappa light chains, free lambda light chains, and kappa lambda ratio; immunoglobulin G (IgG) levels were also increased. A positron emission tomography (PET) scan and magnetic resonance imaging (MRI) thoracic spine suggested a hypermetabolic prevertebral soft tissue density. Biopsy of the mass suggested IgG4-related disease (IgG4-RD). He also had a compression fracture of the T7 vertebra. He was started on intravenous methylprednisolone and rituximab, following which he had a significant decrease in the size of the mass along with a decline in the levels of IgG, kappa, and lambda chains.
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Essential thrombocythemia (ET) is a myeloproliferative neoplasm involving the clonal proliferation of platelets. It is Philadelphia negative and is associated with Janus kinase 2 (JAK2), calreticulin (CALR), or myeloproliferative leukemia virus oncogene (MPL) mutations. The resultant platelets have quantitative and qualitative defects, making them more sticky and prone to thromboembolism. However, ET does not only affect platelet survival, it also has a low leukemogenic potential. It's more common in the elderly, 60 years or more, but can be seen in all age groups, including children. Patients with ET have an increased risk of vascular events like hemorrhage and thromboses like cerebrovascular events, myocardial infarction, superficial thrombophlebitis, deep vein thrombosis, and pulmonary embolism. Cardiovascular risk factors like hypertension, diabetes, and smoking can lead to increased thromboembolism and atherosclerosis. The management of ET focuses primarily on the prevention of thrombosis and hemorrhage. It involves cardiovascular risk management and antiplatelet and cytoreductive therapy according to the risk stratification. Low-risk ET patients are treated with low-dose aspirin, and high-risk ET patients are treated with cytoreductive therapy with hydroxyurea. Interferon (IFN) and anagrelide are reserved for young patients or pregnant women. This case report discusses a 40-year-old male, a known smoker presenting with myocardial infarction and left anterior descending artery (LAD) blockage without any prior history. His high platelets and the relative absence of cardiovascular risk factors helped reach the diagnosis, and bone marrow analysis and mutation analysis confirmed the diagnosis. The patient was started on hydroxyurea, which decreased the total platelet count.
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Oxaliplatin is widely used in chemotherapeutic regimens for colorectal carcinoma, its recurrence, and metastasis, and is associated with better outcomes. However, oxaliplatin use is associated with injury to hepatic sinusoidal endothelium and the development of nodular regenerative hyperplasia (NRH) in the liver, which can be differentiated from nodular hyperplasia of cirrhosis by the presence of diffuse micronodular transformation without a fibrous band and the lack of perinuclear collagen tissue. This causes non-cirrhotic portal hypertension (NCPH), which presents with splenomegaly and variceal bleeding and preserved synthetic liver function. Its treatment revolves around managing variceal bleeding with banding, sclerotherapy, and beta blockers. Some patients may end up requiring liver transplantation because of recurrent variceal bleeding. We present the case of a 46 years old female who presented with recurrent variceal bleeding due to NCPH and NRH six years after treatment of colon carcinoma with oxaliplatin.
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Pneumoconiosis is an occupational disease found in workers with environmental exposure to organic and inorganic dust, as in mining, sandblasting, pottery, stone masonry, and farming. The inflammatory response of the lung to respirable dust causes the formation of macules, nodules, and fibrosis, and higher silica content in inhaled dust is associated with increased fibrosis. Mixed dust pneumoconiosis (MDP) is characterized by exposure to dust containing 10-20% silica, and its lung imaging show irregular opacities. Histopathology plays a vital role in the diagnosis of MDP. Though it has a favorable outcome, it evolves slowly over many years of constant exposure and is characterized by worsening dyspnea and cough gradually progressing to cor pulmonale. The only effective treatment is removing exposure, which makes it essential to recognize the disease early for a favorable outcome. We present a case of mixed dust pneumoconiosis in a farmer from South America who had asthma. He presented with worsening dyspnea and multiple nodules in both lungs on imaging and cor pulmonale. An extensive workup was done, and it ruled out any malignancy and tuberculosis. Analysis of video-assisted thoracoscopic surgery (VATS) biopsy samples confirmed the diagnosis of mixed dust pneumoconiosis. He had a confluence of irregular nodes in the upper lobes of the lungs, and the largest was 2.1 cm. This fits the International Labour Organization (ILO) definition of progressive massive fibrosis. This, along with cor pulmonale present in him, gives it a poor prognosis even after he is removed from dust exposure. He received steroids, which led to symptomatic improvement, and he was discharged to follow up with the pulmonologist.
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BACKGROUND: Lower gastrointestinal bleeds (LGIB) is a very common inpatient condition in the United States. Gastrointestinal bleeds have a variety of presentations, from minor bleeding to severe hemorrhage and shock. Although previous studies investigated the efficacy of colonoscopy in hospitalized patients with LGIB, there is limited research that discusses disparities in colonoscopy utilization in patients with LGIB in urban and rural settings. AIM: To investigate the difference in utilization of colonoscopy in lower gastrointestinal bleeding between patients hospitalized in urban and rural hospitals. METHODS: This is a retrospective cohort study of 157748 patients using National Inpatient Sample data and the Healthcare Cost and Utilization Project provided by the Agency for Healthcare Research and Quality. It includes patients 18 years and older hospitalized with LGIB admitted between 2010 and 2016. This study does not differentiate between acute and chronic LGIB and both are included in this study. The primary outcome measure of this study was the utilization of colonoscopy among patients in rural and urban hospitals admitted for lower gastrointestinal bleeds; the secondary outcome measures were in-hospital mortality, length of stay, and costs involved in those receiving colonoscopy for LGIB. Statistical analyses were all performed using STATA software. Logistic regression was used to analyze the utilization of colonoscopy and mortality, and a generalized linear model was used to analyze the length of stay and cost. RESULTS: Our study found that 37.9% of LGIB patients at rural hospitals compared to approximately 45.1% at urban hospitals received colonoscopy, (OR = 0.730, 95%CI: 0.705-0.7, P > 0.0001). After controlling for covariates, colonoscopies were found to have a protective association with lower in-hospital mortality (OR = 0.498, 95%CI: 0.446-0.557, P < 0.0001), but a longer length of stay by 0.72 d (95%CI: 0.677-0.759 d, P < 0.0001) and approximately $2199 in increased costs. CONCLUSION: Although there was a lower percentage of LGIB patients that received colonoscopies in rural hospitals compared to urban hospitals, patients in both urban and rural hospitals with LGIB undergoing colonoscopy had decreased in-hospital mortality. In both settings, benefit came at a cost of extended stay, and higher total costs.
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Atrial fibrillation (AF) is one of the most common cardiac arrhythmias encountered. Aggressive and appropriate management, along with identification and modification of risk factors, remains at the forefront of evidence-based practice. Thrombus formation (primarily in the left atrial appendage) and consequent thromboembolism are risks associated with AF. Anticoagulation is utilized to prevent and reduce AF-induced complications such as stroke, heart failure, and death. However, in instances when the risk of bleeding from anticoagulation outweighs the benefits of stroke prevention, other modalities such as left atrial appendage closure (LAAC) devices like the WATCHMAN device (Boston Scientific, MA) are utilized. LAAC devices, such as the WATCHMAN device, are also not without significant risks, one of them being device-related thrombus (DRT) formation. We present a case of device-related thrombus formation post WATCHMAN implantation and a subsequent embolic cerebrovascular accident (CVA).
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Hemoglobin A1c (HbA1c) can be unreliable (falsely elevated or lowered) in certain conditions, including hemoglobinopathies, anemia, lead poisoning, chronic alcoholism, and opioid use. Hemoglobin Wayne is a rare variant of hemoglobin (Hgb) that can also result in a false elevation of HbA1c. Hence, clinicians should be aware of these underlying causes before diagnosing and treating diabetes mellitus to avoid unexpected consequences. We are reporting a case of falsely elevated HbA1c in a female in her early 60s due to a rare variant of Hgb called hemoglobin Wayne. The patient presented with a consistently elevated HbA1c ranging from 10.3% to 10.7% for two years, which did not correlate with her fasting blood glucose levels ranging between 80 and 100. The continuous glucose monitoring (CGM) profile was also within the normal range. The hemoglobin electrophoresis technique was used to confirm the diagnosis of hemoglobin Wayne in this patient and the initial treatment of metformin was discontinued upon confirmation.
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Myxomas are benign tumors of mesenchymal origin, containing a few pluripotent cells in the myxomatous stroma. They usually present at 30-40 years of age and are more common in females than males. These tumors mostly arise in the atria and protrude into the atrial lumen. They cause constitutional symptoms like fever and weight loss and obstructive symptoms related to outflow obstruction in the heart. Some tumors are more fragile and cause embolism and may present as stroke. Mostly sporadic but familial cases and myxomas associated with Carney syndrome (CNC) tend to be multiple. Here, we report a case of a 40-year-old female with a stroke due to embolization from multiple myxomas. She had no family history of myxoma and had no skin findings or other tumors associated with CNC. She also had an atrophied horseshoe kidney with renal failure. The association of a horseshoe kidney with myxoma is rarely reported. In an extensive literature search, we could only find only one other case. Atrial myxomas were detected while investigating the cause of stroke. Our patient gradually improved and was advised surgical removal of the myxomas, which is the treatment of choice.
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Posterior reversible encephalopathy syndrome (PRES) refers to white matter vasogenic edema primarily affecting the brain's posterior occipital and parietal lobes, causing acute neurological symptoms like headaches, visual symptoms, seizures, and altered mental status. We present the case of a 32-year-old male with uncontrolled hypertension, altered mental status, and left-sided weakness. He had a rapid neurological decline, and a computed tomography (CT) head showed blurring of gray-white matter interfaces in the right posterior parietal lobe, suggesting infarction or PRES. Magnetic resonance imaging (MRI) of the brain suggested worsening with acute-early subacute infarction involving the right temporal, parietal, and occipital lobes and diffuse cerebral edema causing compression of the right ventricle with diffuse sulcal effacement and central downward herniation. There were flair hyperintensities in the bifrontal, pons, and cerebellum. Given the history of uncontrolled hypertension, the right hemispheric infarction and edema were thought to be due to secondary complications of severe PRES. He underwent urgent bilateral craniectomies with dural augmentation and external ventricular drain placement to control the intracranial pressure the next day. His mental status, as well as neurologic function, showed gradual improvement in the next few months. A high index of suspicion and rapid treatment can pave the way for a quick recovery and help reduce morbidity and death.
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There are few major adverse events after the coronavirus disease 2019 (COVID-19) vaccination. However, increasing cases of myocarditis and pericarditis are being reported to the Vaccine Adverse Event Reporting System (VAERS) in young people, primarily after the second dose of messenger RNA (mRNA) COVID-19 vaccines. We present a case series of myopericarditis post mRNA (Moderna) and myocarditis post vector-based (Johnson & Johnson) COVID-19 vaccines. We intend to highlight the importance of early diagnosis and treatment of vaccine-related myocarditis to reduce mortality and morbidity.
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May-Thurner syndrome (MTS) is a relatively uncommon clinical condition characterized by venous blockage in the left lower leg. Compression of the left common iliac vein by the right common iliac artery and the underlying vertebral body causes obstruction. We report a case of MTS with extensive venous thrombosis in a 44-year-old female who remained clinically silent until she used combined oral contraceptive pills (OCPs) for more than three years.
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Gallbladder hydrops or mucocele is usually due to the obstruction of the gallbladder by a gallstone. It is usually characterized by an increase in gallbladder volume, which remains clinically silent and is often incidentally diagnosed during exploratory laparotomy or laparoscopy. We report a rare case of acute calculous cholecystitis with gallbladder hydrops (measuring 17 cm in maximum dimension) due to the obstruction of the cystic duct by a gallstone in a 67-year-old female. We highlight the importance of early magnetic resonance imaging (MRI) in patients with right upper quadrant (RUQ) pain to rule out gallbladder hydrops especially in those with a history of gallstones.
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Chronic steroid use causes aberrant fat deposition in the epidural space, which may in rare cases result in spinal epidural lipomatosis (SEL). We discuss the case of a 79-year-old female who had been on steroids for a long time, initially for polymyalgia rheumatica (PMR), then for adrenal insufficiency. Her dose was raised with a few steroid stress doses to control the flare of adrenal insufficiency. The patient presented with complaints of intractable lumbosacral pain and was subsequently diagnosed with SEL and foraminal and spinal canal stenosis based on magnetic resonance imaging of the lumbar spine. She successfully underwent laminectomy.
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During an ongoing pandemic of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), a novel virus, new discoveries about its complications and treatment are made every day. Bowel perforation is another rarely reported complication due to the virus itself leading to ischemia or can be due to the treatment with antiviral drugs that reduces the integrity of epithelial barriers. This makes the bowel more prone to perforation even in patients with no prior history of bowel disease. We report a case of bowel perforation in a 72-year-old male patient with severe COVID-19 infection.
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Immune checkpoint inhibitors are a novel class of immunotherapy drugs that have improved the prognosis of melanoma, renal cell carcinoma, non-small cell lung cancer, urothelial carcinoma, and various other solid tumors. Nivolumab is an immune checkpoint inhibitor that acts by inhibiting programmed death. Its use is associated with significant immune-related adverse events, such as pneumonitis, thyroiditis, hepatitis, pruritus, vitiligo, and diarrhea. However, adrenal insufficiency and checkpoint inhibitor-related autoimmune diabetes mellitus are extremely rare adverse events related to nivolumab treatment. Here, we are highlighting cases of adrenal insufficiency and diabetes inspidus as a result of nivolumab. These rare adverse events in our case series are to raise awareness that this medication also may be the cause for this illness among oncologists, endocrinologists, internists, and other clinicians.
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Statins are a widely prescribed medication that lowers serum cholesterol by inhibiting the HMG-CoA reductase enzyme, a rate-limiting step in cholesterol synthesis. Myopathy is one of the well-known adverse effects of statins, mainly when prescribed with the fibrates. However, statin-induced autoimmune necrotizing myopathy (SIANM) is an infrequent and severe complication. Hence, all clinicians should be more vigilant regarding this complication and treat it early to prevent acute kidney injury (AKI).
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Warfarin is associated with anticoagulant-related nephropathy (ARN), one of the potential side effects. This is evidenced by a progressively increasing number of detected cases of deterioration in the kidney function even in patients with normal baseline function (GFR {glomerular filtration rate}) in addition to the chronic kidney disease (CKD) patients who are already vulnerable to ARN. There has been a clinical correlation in a rapid decline of kidney function and international normalized ratio (INR) levels of greater than three. ARN is a significant but underdiagnosed complication of anticoagulation that is associated with increased renal morbidity and all-cause mortality. We want to emphasize the importance of monitoring kidney function regularly and adjusting the appropriate doses of warfarin. We present a case of a 57-year-old female who was on warfarin for the mechanical aortic valve, presented with acute kidney injury and supratherapeutic INR. Her renal biopsy showed diffuse mesangial proliferative glomerulonephritis.
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[This corrects the article DOI: 10.7759/cureus.28883.].
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Aldosterone is a mineralocorticoid hormone that maintains sodium and potassium homeostasis. Excess aldosterone secretion causes sodium influx and potassium outflow, leading to hypertension and in some cases hypokalemia. Conn's syndrome, or primary aldosteronism, is the most common cause of secondary hypertension, accounting for 20% or more of people with resistant hypertension. We present a young male with hypertension, blurry vision in the right eye, and hypokalemia who was on further investigation found to have an aldosterone-secreting adrenal adenoma. He was treated with retroperitoneoscopic right-sided adrenalectomy and his blood pressure improved. Conn's syndrome should be suspected in any hypertensive patient with hypokalemia. Adrenal venous sampling is the best investigation to measure aldosterone levels and also to lateralize the source. Surgical resection is the treatment of choice.
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Polycystic liver disease (PLD) is a condition that most often occurs in patients with autosomal dominant polycystic kidney disease (ADPKD) and less commonly as isolated liver disease. The presence of both conditions has proven to be a therapeutic challenge. Patients with ADPKD can suffer from significant renal and extra-renal complications and symptoms as a result of space-occupying cysts from polycystic kidney and liver enlargement. We present a case of ADPKD in a 56-year-old Caucasian female who developed pelvic organ prolapse, a rare complication, while also dealing with multiple other complications of ADPKD. Despite the high prevalence of ADPKD, complications such as pelvic organ prolapse have seldom been reported and discussed in the literature. The care team should do a prompt gynecological examination when they realize the burden of cysts becomes so large.