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Amiodarone-Induced Electrical Storm: A Nightmare in the Emergency Room
Full text linkDrug-induced long QT syndrome (LQTS) is defined as prolonged corrected QT interval (QTc ≥460 ms) plus polymorphic ventricular arrhythmia fitting the description of torsades de pointes temporally associated with the administration of a drug or combination of drugs. Amiodarone therapy is a known uncommon cause of acquired QT interval prolongation that should not be underestimated. We present a case of an iatrogenic electrical storm with atrial fibrillation (AF) in which amiodarone was administered to attempt chemical cardioversion, resulting in an unnoticed prolongation of the QT interval, with subsequent repeated polymorphic ventricular tachycardia, managed with isoproterenol. Concomitant drugs and slight electrolyte disturbances potentiated this phenomenon. Given the widespread use of this drug in the emergency department, our case highlights a pertinent matter for all medical emergency practitioners. Additionally, it stresses the significance of potential precipitating factors, such as electrolyte imbalances, which are clinical conditions very frequent in the emergency context, along with the importance of recognizing drug interactions. Finally, this case also emphasizes the vital importance of closely monitoring the patient's receiving amiodarone.info:eu-repo/semantics/publishedVersio
Synergic Difficulties in an Anticipated Physiologically and Anatomically Difficult Airway in a Trauma Patient: A Case Report
Full text linkThe American Society of Anesthesiologists (ASA) defines a difficult airway as a clinical situation in which a physician who is trained in anesthesiology experiences difficulty or fails in either face mask ventilation, laryngoscopy, using a supraglottic airway, tracheal intubation, extubation, or front-of-neck airway. Classically, this has been defined in relation to anatomic factors, but the concept of a physiologically difficult airway has been growing in relevance, in which physiologic factors, such as hypoxemia and hypercapnia, act to reduce safe apnea times. The case reports on a trauma patient with an unstable thoracic vertebral fracture requiring correction via the posterior approach. Our patient had multiple anatomical difficult airway predictors, namely, a short neck, greatly limited neck mobility, and a Mallampati class IV airway, among others, and multiple physiological difficult airway predictors, such as a baseline hypoxemic respiratory failure and severe sleep apnea, in addition to the restrictions on mobility imposed by the fracture itself. We describe a successful perioxygenation strategy, using high-flow nasal oxygen (HFNO) during the preoxygenation, intubation, extubation, and post-anesthesia care phases, and with an awake fiberoptic intubation technique for securing the airway.info:eu-repo/semantics/publishedVersio
Recurrent Appendicitis in Children: The Impact of a Poorly Known Disease
Full text linkChronic and recurrent appendicitis is rare in pediatric patients and can be easily misdiagnosed due to its unusual presentation and low incidence rate. We present the case of an 11-year-old male with recurrent right lower quadrant (RLQ) pain persisting for 19 months. The patient experienced pain flare-ups accompanied by paleness and gait limp, without fever or other symptoms. Despite extensive medical examinations, including imaging and endoscopy, a definitive diagnosis remained elusive. As serial abdominal ultrasounds reported an appendix at the upper limit of the normal caliber and symptoms persisted despite medical therapy, a diagnostic laparoscopy was performed, revealing a congested ileocecal appendix with erosions and granulocytic inflammatory infiltrate, consistent with appendicitis. Post-appendectomy, the patient's symptoms resolved, significantly improving his quality of life (QoL), as evidenced by the DISABKIDS Chronic Generic Module (DCGM). This case underscores the challenges in diagnosing chronic and recurrent appendicitis, emphasizing the need for improved awareness, case definitions, and research to better understand and manage these conditions. Moreover, the report highlights the substantial impact of such conditions on patients' physical, social, and psychological well-being using the only health-related QoL instrument developed across cultures for children with chronic diseases: the DCGM.info:eu-repo/semantics/publishedVersio
An Immunological Non-responder Human Immunodeficiency Virus/Hepatitis C Virus Coinfected Patient: Considerations About a Clinical Case
Full text linkHuman immunodeficiency virus (HIV) and hepatitis C virus (HCV) infections are two chronic viral infections that share the same mode of transmission, making HIV/HCV coinfection frequent. Highly active antiretroviral therapy (HAART) was a turning point in HIV treatment and has been shown to successfully restore immune function and reduce the frequency of opportunistic infections. Despite a virological response to HAART, a proportion of patients fail to achieve substantial immune recovery, as measured by peripheral CD4 cell counts. Herein, we present the case of a patient with HIV/HCV coinfection who did not achieve successful immune function restoration despite HIV suppression and HCV treatment. Our goal is to promote discussion. Despite considerable advances in the understanding of the impact of HCV on HIV disease progression, there are many individual variables that influence a patient's immune function. In addition, we consider hypogammaglobulinemia as a possible contributor. Further understanding and improvement of immune reconstitution in patients infected with HIV remain an important field of scientific research.info:eu-repo/semantics/publishedVersio
2023 ACR/EULAR antiphospholipid syndrome classification criteria
Full text linkObjective: To develop new antiphospholipid syndrome (APS) classification criteria with high specificity for use in observational studies and trials, jointly supported by the American College of Rheumatology (ACR) and EULAR.
Methods: This international multidisciplinary initiative included four phases: (1) Phase I, criteria generation by surveys and literature review; (2) Phase II, criteria reduction by modified Delphi and nominal group technique exercises; (3) Phase III, criteria definition, further reduction with the guidance of real-world patient scenarios, and weighting via consensus-based multicriteria decision analysis, and threshold identification; and (4) Phase IV, validation using independent adjudicators' consensus as the gold standard.
Results: The 2023 ACR/EULAR APS classification criteria include an entry criterion of at least one positive antiphospholipid antibody (aPL) test within 3 years of identification of an aPL-associated clinical criterion, followed by additive weighted criteria (score range 1-7 points each) clustered into six clinical domains (macrovascular venous thromboembolism, macrovascular arterial thrombosis, microvascular, obstetric, cardiac valve, and hematologic) and two laboratory domains (lupus anticoagulant functional coagulation assays, and solid-phase enzyme-linked immunosorbent assays for IgG/IgM anticardiolipin and/or IgG/IgM anti-β2-glycoprotein I antibodies). Patients accumulating at least three points each from the clinical and laboratory domains are classified as having APS. In the validation cohort, the new APS criteria vs the 2006 revised Sapporo classification criteria had a specificity of 99% vs 86%, and a sensitivity of 84% vs 99%.
Conclusion: These new ACR/EULAR APS classification criteria were developed using rigorous methodology with multidisciplinary international input. Hierarchically clustered, weighted, and risk-stratified criteria reflect the current thinking about APS, providing high specificity and a strong foundation for future APS research.info:eu-repo/semantics/publishedVersio
Medical Challenges of a Common Variable Immunodeficiency With a TNFRSF13B Gene Mutation in a Simultaneous Kidney and Pancreas Transplant Recipient
Full text linkCommon variable immune deficiency (CVID) is a primary immunodeficiency disorder, with hypogammaglobulinemia and increased susceptibility to recurrent infections, autoimmune disorders, granulomatous diseases and malignancy. Among the solid organ transplant (SOT) recipient population, those with primary immunodeficiency disorders under chronic immunosuppression therapy can theoretically be at higher risk of atypical infections, autoimmune complications and disease recurrence with suboptimal long term graft survival, but literature is scarce. Here, we report a 27-year-old female with type 1 diabetes mellitus, complicated with nephropathy that progressed to end-stage renal disease (ESRD), who had a history of a chronic inflammatory response dysregulation, with chronic monoarthritis, persistent elevation of inflammation markers, recurrent infections, low immunoglobulin G (IgG) and A (IgA) serum levels, a slightly decreased population of memory B cells at flow cytometric immunophenotyping, and a confirmed pathological heterozygous mutation in the tumor necrosis factor receptor superfamily 13B (TNFRSF13B), with a suspected diagnosis of CVID. Whilst on hemodialysis, she received a simultaneous kidney and pancreas transplant from a standard criteria donor (SCD), and our induction and maintenance immunosuppression protocol and prophylaxis regimen allowed for a successful transplant with immediate pancreatic function, with no evidence of renal graft rejection upon biopsy in the early post-transplant period, and no novel episodes of serious infectious complications were recorded during a follow-up period of six monthsinfo:eu-repo/semantics/publishedVersio
Intrasphenoidal Rathke’s Cleft Cyst: An Uncommon Feat
Full text linkUsually occurring entirely intrasellarly or extending suprasellarly (intra-suprasellar), Rathke's cleft cysts (RCCs) can present with an intrasphenoidal location. Extrasellar positions are rare. To date, only seven patients with intrasphenoidal RCC have been reported in the literature. Despite the rarity of the condition and the lack of pathognomonic radiological features, preoperative diagnosis remains challenging. A trans-sphenoidal approach can be adopted to treat this type of cyst, which has great clinical relevance. Awareness of this different presentation of RCC before respective management may be of value in its approach. Intrasphenoidal RCC should be diagnosed preoperatively and the surgical approach should be changed accordingly by aspiration and partial removal before the histological examination.info:eu-repo/semantics/publishedVersio
Estimating the Glomerular Filtration Rate in Pediatric Patients With Neurogenic Bladder: A Comparison Between Creatinine- and Cystatin C-Equations
Full text linkBackground and objective Patients with neurogenic bladder (NB) are at a higher risk of developing chronic kidney disease (CKD). Due to their lower muscle mass, the estimated glomerular filtration rate (eGFR) based on creatinine (Cr) may be overestimated and delay the diagnosis of renal failure. This study compared eGFR calculated with different equations based on Cr and/or cystatin C (CysC) in children with NB, and the differences between patients with lower muscle mass (underdeveloped lower limbs) and those with independent gait (less muscle depletion). Methods We calculated the eGFR in pediatric patients with NB and CKD stages 1 and 2 by using the following equations: Chronic Kidney Disease in Children equation for serum creatinine (CKiD-Cr), CKiD-CysC, CKiD combined-Cr/CysC, Zappitelli-CysC, and Zappitelli combined-Cr/CysC. Results We evaluated a total of 47 patients, 74.5% with CKD stage 1, with a median age of 14.1 years. Of these participants, 59.6% had lipo/myelomeningocele. The CKiD-Cr and CysC-based equations led to significantly lower calculated eGFR (p<0.05), specifically CKiD-CysC (p<0.001), Zappitelli-CysC (p<0.001), CKiD-Cr/CysC (p<0.001), and Zappitelli combined-Cr/CysC (p<0.05). When CKiD-CysC was used, 68% of the patients moved to a more advanced CKD stage. In patients without independent gait, with lower muscle mass (55.3%), the median eGFR calculated using the CKiD-Cr and CKiD combined-Cr/CysC equations was significantly higher (p<0.05). However, there were no differences between the two groups when using the other CysC-based equations. Conclusion In patients with NB and poor muscle mass, the CKiD-Cr equation may overestimate renal function. CysC-based equations seem more reliable in these patients, especially in those with greater muscular atrophy.info:eu-repo/semantics/publishedVersio
A Case Report of Extreme Oral Lesions: A Rare Indicator of Bullying-Associated Non-suicidal Self-Injury
Full text linkBullying has reached epidemic proportions, affecting one in three students worldwide. A pervasive issue that carries profound physical, mental, and social consequences, significantly increasing the risk of non-suicidal self-injury (NSSI) and suicidal behaviors among those who experience this type of harassment and hazing. While physicians and most caregivers are fully aware and competent in identifying signs of self-harming behavior such as scratching, cutting, or burning the skin, oral self-injury is often overlooked as a potential indicator and is associated with unintentional soft tissue biting or specific conditions. We present a rare case of a 14-year-old male who sought medical attention due to severe bilateral tongue ulcers, leading to his admittance to the emergency department (ED) with excruciating pain and feeding difficulties. In the reported case, although the traumatic biting of the tongue emerged as the most probable etiological factor, a specific underlying motive and contextual comprehension were initially absent. It was only after successfully establishing a foundation of trust with the patient, enabling an honest response, that it became evident that the observed lesions represented a manifestation of bullying-induced non-suicidal self-injury. However, patients rarely openly acknowledge intentional self-inflicted lesions and/or their experiences of bullying, underscoring the necessity to maintain vigilance for alternative indicators such as behavioral changes or a noticeable decline in academic productivity. The significance of this case also goes beyond its presentation, highlighting the largely unexplored issue of how a patient's dentofacial features can serve as substantial catalysts for bullying. Therefore, it is only through equally prioritizing awareness of uncommon signs, symptoms, and context that one can expedite early diagnosis and intervention, emphasizing the essential need for comprehensive and timely management of such cases.info:eu-repo/semantics/publishedVersio
Diagnosis of Asymptomatic Biliary Ascariasis by Abdominal Ultrasound in a Non-Endemic Area
Full text linkBiliary ascariasis is rare in non-endemic areas. This infection is associated with severe complications of the biliary tract, which can become a medical emergency. Treatment with oral anthelmintics is often effective, but, in some cases, surgery is required. We describe an unusual case of ultrasound diagnosis of biliary ascariasis in the gallbladder in a patient who, besides residing in a low-prevalence area of the infection, did not present with biliary tract manifestations. We intend to raise awareness of this clinical entity in non-endemic areas, where this diagnosis is not usually considered. A brief review of the subject is also presented.info:eu-repo/semantics/publishedVersio