Phenytoin-Associated Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Overlap in Focus: A Case Report.

Stevens-Johnson syndrome and toxic epidermal necrolysis overlap is a rare but severe cutaneous hypersensitivity reaction that can lead to death if not treated aggressively and adequately. Drug-induced hypersensitivity reactions are often related to drug exposure, with sulfonamides, anti-epileptics, fluoroquinolones, cephalosporins, and nonsteroidal anti-inflammatory drugs being the most common culprits. This case report describes a 10-year-old boy who was administered phenytoin at a local clinic to manage his seizures. This treatment led to the onset of SJS-TEN overlap, ultimately resulting in his demise.

Unlocking the Potential of Immunotherapy in Cardiovascular Disease: A Comprehensive Review of Applications and Future Directions.

Immunotherapy has emerged as a pioneering therapeutic approach that harnesses the immune system's abilities to combat diseases, particularly in the field of oncology where it has led to significant advancements. However, despite its significant impact in the field of oncology, the potential of immunotherapy in the context of cardiovascular disease (CVD) has not been thoroughly investigated. The purpose of this narrative review is to address the existing knowledge and potential uses of immunotherapy in the field of cardiovascular disease (CVD), with the intention of filling the existing gap in understanding. Furthermore, the review thoroughly examines the future prospects of this swiftly advancing field, providing insights into the aspects that necessitate further investigation and addressing the forthcoming challenges. The review is organized into four distinct sections to enhance comprehension. The first section introduces immunotherapy, presenting the fundamental concepts and principles. The second section explores the immunomodulatory mechanisms in cardiovascular disease (CVD), with a specific focus on the intricate interplay between the immune system and the development of cardiovascular pathogenesis. The utilization of immunotherapy in specific cardiovascular conditions will be examined, investigating the application of immunotherapy in the context of different cardiovascular diseases. The future prospects and challenges in immunotherapy for cardiovascular diseases will be discussed, highlighting the potential areas for future research and addressing the barriers that must be overcome to effectively implement immunotherapeutic interventions in the management of cardiovascular diseases.

A Very Rare Case of Diabetes Mellitus Occurring in a Patient With Hyperinsulinism Hyperammonemia Syndrome.

Background/Objective: To illustrate an unusual case of type 2 diabetes mellitus (T2DM) developing many years after the diagnosis of hyperinsulinism hyperammonemia (HI/HA) syndrome. Case Report: This article reports about a 36-year-old female with a history of congenital hyperinsulinism due to HI/HA syndrome, which was diagnosed in infancy. The patient presented with hypoglycemia and seizures as an infant and was treated with diazoxide and a low-protein diet for many years with reduction in her hypoglycemic events. She subsequently developed T2DM >30 years later. Genetic analysis was positive for a glutamate dehydrogenase 1 gene (GLUD1) alteration. She was treated with metformin and a glucagon-like peptide 1 agonist, with significant improvement in her blood glucose control and weight loss. Discussion: HI/HA syndrome is a rare genetic syndrome that manifests in childhood with signs and symptoms of hypoglycemia and neurologic symptoms. This is the first case reported in the literature of a patient with HI/HA syndrome due to a GLUD1 alteration who developed T2DM much later in life. Patients with this disorder usually have recurrent hypoglycemia and require long-term medical therapy or very occasionally may have a resolution. She had class 3 obesity and evidence of insulin resistance, which likely contributed to her risk of diabetes. Conclusion: This is a rare case of T2DM presenting in a patient with HI/HA syndrome. This should be considered a possible outcome in patients with this disorder, especially in the presence of obesity.

Generation of a Spiral Ganglion Neuron Degeneration Mouse Model.

Spiral ganglion neurons (SGNs) can be injured by a wide variety of insults. However, there still is a lack of degeneration models to specifically damage the SGNs without disturbing other types of cells in the inner ear. This study aims to generate an SGN-specific damage model using the Cre-LoxP transgenic mouse strains. The Cre-inducible diphtheria toxin receptor (iDTR+/+ ) knock-in mouse strain was crossed with a mouse strain with Cre activity specific to neurons (Nefl CreER/CreER ). Expression of the Cre-recombinase activity was evaluated using the reporter mouse strain Ai9 at pre-hearing, hearing onset, and post-hearing stages. Accordingly, heterozygous Nefl CreER/+;iDTR+/- mice were treated with tamoxifen on postnatal days 1-5 (P1-5), followed by diphtheria toxin (DT) or vehicle injection on P7, P14, and P21 to evaluate the SGN loss. Robust tamoxifen-induced Cre-mediated Ai9 tdTomato fluorescence was observed in the SGN area of heterozygous Nefl CreER/+;Ai9+/- mice treated with tamoxifen, whereas vehicle-treated heterozygote mice did not show tdTomato fluorescence. Compared to vehicle-treated Nefl CreER/+;iDTR+/- mice, DT-treated Nefl CreER/+;iDTR+/- mice showed significant auditory brainstem response (ABR) threshold shifts and SGN cell loss. Hair cell count and functional study did not show significant changes. These results demonstrate that the Nefl CreER/CreER mouse strain exhibits inducible SGN-specific Cre activity in the inner ear, which may serve as a valuable SGN damage model for regeneration research of the inner ear.

Salmonella aortitis successfully treated with antibiotics without surgery.

Aortitis is an inflammation of the aorta that is linked to large vessel vasculitis and other rheumatologic cases. Less often, an infectious etiology of aortitis is diagnosed. Aortitis is associated with high mortality and morbidity and requires a high index of suspicion. Here we present a rare case of aortitis secondary to Salmonella Septicemia treated with six weeks of antibiotics in the hospital without and remained asymptomatic and inflammatory markers normalized at 2 weeks follow up (ESR, CRP, and WBCs).

A case of unusual presentation of acute esophageal necrosis with pneumonia.

Acute esophageal necrosis (AEN) is a rare and often fatal pathology of unclear etiology affecting the distal two-thirds of the esophagus. Typically, elderly patients with multiple comorbidities present with signs of upper gastrointestinal (GI) hemorrhage. On endoscopy, the mucosa is black due to ischemic necrosis, resulting in the commonly used term "black esophagus." We present a rare case of a 61-year-old male presenting with shortness of breath and hematemesis diagnosed as AEN through endoscopy. This case illustrates the importance of considering AEN as part of differential diagnoses in a rising elderly population with multiple comorbidities that present with upper GI hemorrhage. Treatment should be aimed at maintaining hemodynamic stability with high-dose proton pump inhibitors.

Recurrent Kikuchi-Fujimoto Disease Masquerading as Lymphoma Successfully Treated by Anakinra.

Kikuchi-Fujimoto disease (KFD), also known as histiocytic necrotizing lymphadenitis, is a rare and benign disease that usually presents in middle-aged women of Oriental-Asian ethnicity. This condition was described in Japan for the first time in 1972. Though the clinical course is benign, KFD has been misdiagnosed as malignancy (e.g. lymphoma) or infection. The most common presentation of KFD is with localized or generalized lymphadenopathy, fever, fatigue, weight loss, hepatosplenomegaly, and rash. A definitive diagnosis of KFD can be made by excisional lymph node biopsy, as immunohistochemical analysis is necessary. We present here an interesting case of a 20-year-old Hispanic female who was diagnosed with KFD who failed therapy with steroids and was subsequently treated successfully with the interleukin-1 (IL-1) inhibitor - anakinra.

Chronic Pancreatitis Leading to Pancreatogenic Diabetes Presenting in Diabetic Ketoacidosis: A Rare Entity.

Diabetes mellitus type 3c (DM3c) is an uncommon cause of diabetes due to pancreatic pathology. Its prevalence reaches about 5-10% among all diabetics in the Western world, largely due to chronic pancreatitis. DM3c occurs due to the destruction of the endocrine islet cells. Glucagon and insulin levels are both decreased due to the destruction of alpha and beta cells, respectively. This makes the development of diabetic ketoacidosis (DKA) a rare process in patients with DM3c because of the destruction of glucagon, which facilitates ketone production. We report a case of DM3c presenting with DKA. The patient presented with a history of chronic pancreatitis and was on pancreatic enzyme replacement therapy. Prior records revealed that HbA1c levels were normal. Prior computed tomography evidence revealed diffuse pancreatic calcifications. The patient was admitted for DKA, presenting with hyperglycemia, blood glucose of 703 mg/dL, bicarbonate of 16 mmol/L, ketones in the urine and acetone in the blood. The patient's anion gap corrected for albumin was 27. The patient was admitted to the medical intensive care unit where he was treated with intravenous (IV) insulin and IV hydration. Once the anion gap closed, the patient was transitioned to long-acting insulin. HbA1c level on admission was elevated, autoimmune causes of diabetes were sent and were negative, ruling out late onset type 1 diabetes. This shows that although it is a rare phenomenon, diabetics with DM3c can present in DKA.

Enigma of recurrent strokes with literature review.

Metastatic tumors are the most common tumors affecting the heart. Primary tumors are rare, with myxomas being the most common of the primary cardiac tumors. The incidence of primary cardiac tumors is 0.02%, about 200 cases has been reported in 1 million autopsies. Most of primary cardiac tumors have been detected incidentally on diagnostic modalities: computed tomography (CT), cardiac magnetic resonance imaging (MRI), or echocardiography. Majority of primary-origin cardiac tumors are benign, of which the most common type of primary tumor is cardiac myxoma. Fibroelastoma is a rare benign tumor and the 2nd most common cause of primary cardiac tumors. In the past fibroelastoma has been detected on autopsy findings. With the development of more advanced imaging modalities fibroelastoma is more frequently detected as a cause of stroke, myocardial infarction (MI), angina episodes, and systemic embolization. Echocardiogram is the best diagnostic modality to diagnose primary cardiac tumors although transthoracic echocardiogram (TTE) can miss primary cardiac tumors; transesophageal echocardiography (TEE) has been more labeled more accurate in the diagnosis of primary cardiac tumors. We present here a case of a 21-year-old male with the history of multiple strokes secondary to cardiac papillary fibroelastoma (CPF).