Clinicoepidemiological Profile of Ciguatera Cardiotoxicity: A Systematic Review.

Patients with ciguatera poisoning commonly present with gastrointestinal and neurologic symptoms, but its cardiotoxicity has been largely unrecognized. This systematic review is the first to summarize the evidence regarding clinical characteristics of cardiotoxicity from ciguatera poisoning to provide the illness script and pertinent knowledge for clinicians. Following the Preferred Reporting Items for Systematic reviews and Meta-Analyses statement, we searched MEDLINE and EMBASE for all peer-reviewed articles using keywords including "ciguatera" and "cardiotoxicity" from their inception to January 2024. We included 20 articles, including 148 cases, in this systematic review. Among the included cases, the median age was 54 years with male predominance (62.2%). Based on the WHO designation, 68.5% were reported from the Western Pacific Region. Common symptoms included hypotension (75.0%) and bradycardia (67.6%), and chest pain or syncope were less commonly reported (2.7% and 1.4%, respectively). Sinus bradycardia was the most common electrocardiogram abnormality (35.9%), followed by atrioventricular blocks (15.4%). Symptomatic treatments such as atropine, dopamine, and epinephrine were commonly used, and only 4.1% required intensive care unit admission. None expired due to cardiotoxicity from ciguatera. This review summarizes the current evidence and the characteristics of cardiotoxicity from ciguatera. Although ciguatera cardiotoxicity is currently underrecognized, increased awareness of the condition in clinicians is crucial because the clinical outcomes of the patients could be benign as long as it is identified and intervened early.

The Successful Management of Stroke in Evans Syndrome by Anticoagulation with Warfarin, Intravenous Immunoglobulin (IVIG), and High-Dose Corticosteroid.

Evans syndrome (ES) is a rare autoimmune disorder characterised by autoimmune haemolytic anaemia (AIHA), immune thrombocytopenia and autoimmune neutropenia. The precise pathogenesis of ES remains unclear, but it is believed to involve immune-mediated destruction of erythrocytes and platelets. Thrombotic complications, such as stroke, are critical yet largely unrecognised in ES. Here, we present a case of an 80-year-old male with ES who developed multiple strokes, emphasising the complex management challenges associated with this condition. The patient, known for stage IIB lung adenocarcinoma, presented with right-sided weakness and was diagnosed with a stroke of undetermined aetiology. He was started on warfarin for secondary prevention alongside intravenous immunoglobulin (IVIG) and corticosteroids for ES. Stroke in ES is rarely reported, and the optimal management remains inconclusive due to its rarity. The patient's management was guided by existing guidelines for stroke prevention and anticoagulation in the setting of antiphospholipid syndrome. While anticoagulants are recommended for venous thromboembolism prophylaxis in AIHA, there are no clear guidelines for stroke prevention in ES. This case underscores the necessity of individualised treatment approaches and highlights the gaps in evidence regarding stroke management in ES. Future research is essential to determine the optimal management of stroke in this complex clinical scenario. LEARNING POINTS: Evans syndrome is a rare autoimmune disorder characterised by the coexistence of autoimmune haemolytic anaemia and immune thrombocytopenia, which potentially increase venous and arterial thrombotic risk.Managing strokes in Evans syndrome remains challenging due to its rarity and lack of definitive guidelines, necessitating individualised treatment approaches.Future prospective studies are warranted to determine the optimal patient population that needs secondary prevention with anticoagulants following a stroke in the context of Evans syndrome.

Left Ventricular Outflow Tract Obstruction Due to Cardiac Hamartoma.

Hamartoma of mature cardiac myocytes (HMCM) is a rare, benign cardiac tumor. We report a case of a 19-year-old female with an atypical presentation, including significant weight loss and abnormal electrocardiogram. A transthoracic echocardiogram (TTE) revealed a mass causing left ventricular outflow tract (LVOT) obstruction, confirmed by cardiac magnetic resonance (CMR) imaging showing a 5 x 3 cm mass contiguous with the right ventricular free wall and exhibiting heterogeneous, diffuse late gadolinium enhancement. The patient subsequently underwent sternotomy for surgical biopsy and septal myectomy, with histology of the mass being consistent with HMCM. The patient remained asymptomatic during her 6-month follow-up.

Atypical lymphoplasmacytic and immunoblastic proliferation: A Systematic Review.

Atypical lymphoplasmacytic and immunoblastic proliferation (ALPIBP) was first reported in 1984 as characteristic histological findings in lymph nodes associated with autoimmune diseases, but it has not been clearly defined to date. To summarize the histological characteristics and clinical diagnoses associated with ALPIBP, we searched MEDLINE and EMBASE for all peer-reviewed articles using keywords including "atypical lymphoplasmacytic and immunoblastic lymphadenopathy" from their inception to December 27, 2023. We also summarized the courses of three cases with a pathological diagnosis of ALPIBP. Nine articles with 52 cases were included. Among the total of 55 cases, including the three from our institution, the median age of the cases was 63.5 years with a female predominance (69.5%). Lymphadenopathy was generalized in 65.6% and regional in 34.4% of cases. RA (24.4%), SLE (24.4%), and autoimmune hemolytic anemia (20.0%), were common clinical diagnoses. A combination of cytotoxic chemotherapy was used in 15.6% of cases due to the suspicion of malignancy. Nodal T-follicular helper cell lymphoma, angioimmunoblastic type, methotrexate-associated lymphoproliferative disorders, and IgG4-related diseases were listed as important diseases that need to be pathologically differentiated from ALPIBP. This review summarizes the current understanding of the characteristics of ALPIBP. Given that underrecognition of ALPIBP could lead to overdiagnosis of hematological malignancy and unnecessary treatment, increased awareness of the condition in pathologists and clinicians is crucial.

Tonsillar Metastasis of Hepatocellular Carcinoma: A Case Report and Review of the Literature.

A 63-year-old male with stage IV hepatocellular carcinoma (HCC), accompanied by lung and adrenal metastases, presented with oral bleeding. Physical examination disclosed bleeding from the tonsillar mass. A head and neck computed tomography identified a 2.4 cm enhancing lesion in the right anterior ethmoidal sinus, extending to the nasal region and medial orbit. Tonsillar mass biopsy confirmed HCC metastasis, immunopositive for Hepatocyte Paraffin 1 (HepPar1) and Arginase. He was treated with local radiotherapy (30 fractions). The unique presentation of severe bleeding from a tonsillar biopsy-proven HCC metastatic lesion underscores the rarity of head and neck involvement. Extrahepatic metastasis, particularly to the head and neck area likely due to hematogenous spread, may be a major independent predictor of poor outcomes in HCC patients. Local radiotherapy to achieve local hemostasis and reduce tumor bulk should be considered. In patients with known HCC having new oropharyngeal symptoms, HCC metastasis should be considered for a timely diagnosis. Despite its rarity, this manifestation signifies an unfavorable prognosis, reinforcing the imperative for a multidisciplinary approach to enhance therapeutic outcomes in these complex scenarios.

Reversible Cerebral Vasoconstriction Syndrome Mimicking Eclampsia.

This report describes the case of an 18-year-old Micronesian pregnant woman at 32 weeks gestation, initially presumed to have eclampsia but later diagnosed with reversible cerebral vasoconstriction syndrome (RCVS). She presented with seizures, altered mental status, nystagmus, lower extremity weakness, and absent reflexes. An extensive workup ruled out infectious and autoimmune causes, but a computed tomography angiogram (CTA) revealed severe cerebral vasoconstriction. Treatment included levetiracetam, intravenous magnesium, and nimodipine. The case highlights the challenge of differentiating RCVS from eclampsia in the postpartum period, emphasizing the importance of considering alternative diagnoses and brain CTA when RCVS is suspected, with calcium channel blockers potentially contributing to favorable neurological outcomes.

Blastoid Mantle Cell Lymphoma Presenting as an Oropharyngeal Mass.

Blastoid mantle cell lymphoma (MCL) is an extremely rare neoplasm with a dismal prognosis. MCL with an initial presentation in the oral cavity has been rarely reported. This report describes a 75-year-old male who presented with an oropharyngeal mass causing dysphonia and intermittent hypoxia. A biopsy and immunophenotyping confirmed MCL, favoring the blastoid variant. Imaging showed a 4.2 cm left oropharyngeal polypoid mass with extensive lymphadenopathy. His prognosis was considered unfavorable with elevated Ki-67 index, blastoid morphology, and p53 positivity of malignant cells. There was no central nervous system involvement. He received palliative radiation, resulting in profound tumor reduction and resolution of symptoms. An intensive chemoimmunotherapy was not deemed beneficial due to age, comorbidities, absence of TP53 mutation, and a personal preference for a less aggressive treatment. This case highlights the importance of risk-adapted and personalized management approaches in a very unique presentation of blastoid MCL.

Burden of depressive disorders in Vietnam from 1990 to 2019: A secondary analysis of the Global Burden of Disease Study 2019.

Depressive disorders are among the leading causes of disability globally. However, information on the burden of depressive disorders in Vietnam is limited. We aimed to analyse the burden of depressive disorders in Vietnam from 1990 to 2019. Using data from the Global Burden of Disease Study 2019, prevalence and disability-adjusted life-years (DALYs) were used as indicators to analyse the burden of depressive disorders by age and sex. In 2019 in Vietnam, depressive disorders comprised 2629.1 thousand (95% uncertainty interval (UI): 2233.3-3155.9) estimated cases and 380.6 thousand (95% UI: 258.9-533.8) estimated DALYs. The crude prevalence rate of depressive disorders was higher among females than among males. The DALYs of depressive disorder accounted for a higher percentage of the total all-cause DALYs in the 10-64-year age group than in other age groups. Major depressive disorder was the largest contributor to the burden of depressive disorders. From 1990 to 2019, the crude prevalence and DALY rates per 100 000 population due to depressive disorders increased significantly, whereas age-standardised rates of prevalence and DALYs decreased significantly; the respective average annual percent changes were 0.88% (95% confidence interval: 0.87 to 0.89), 0.68% (0.66 to 0.70), -0.20% (-0.21 to -0.19), and -0.27% (-0.28 to -0.25). Although the age-standardised prevalence rate was lower than that seen globally, depressive disorders were considerable mental health issues in Vietnam. This study will help governments and policymakers to establish appropriate strategies to reduce the burden of these disorders by identifying the priority areas and individuals.

Diagnostic challenges of the idiopathic plasmacytic lymphadenopathy (IPL) subtype of idiopathic multicentric Castleman disease (iMCD): Factors to differentiate from IgG4-related disease.

AIMS AND METHODS: Idiopathic multicentric Castleman disease (iMCD) is currently considered to be classified into three clinical subtypes, including idiopathic plasmacytic lymphadenopathy (IPL), thrombocytopaenia, anasarca, fever, reticulin fibrosis/renal dysfunction, organomegaly (TAFRO) and not otherwise specified (NOS). Among the three, iMCD-IPL closely mimics IgG4-related disease (IgG4-RD). In diagnosing IgG4-RD, it is sometimes challenging to distinguish iMCD-IPL patients that also meet the histological diagnostic criteria for IgG4-RD. In this study, we focused on the number of IgG4-positive cells in the lymph nodes and analysed the relationship with laboratory findings to distinguish iMCD-IPL from IgG4-RD. Thirty-nine patients with iMCD-IPL and 22 patients with IgG4-RD were included. RESULTS: Among the cases considered to be iMCD-IPL, 33.3% (13/39) cases also met the histological diagnostic criteria for IgG4-RD and serum IgG4 levels were not different between the two groups. However, the serum IgG4/IgG ratio was significantly higher in IgG4-RD, with a cut-off value of 19.0%. Additionally, a significant positive correlation between serum IgG levels and the number of IgG4-positive cells was observed in iMCD-IPL (p=0.001). The serum IgG cut-off value for distinguishing iMCD-IPL meeting histological criteria for IgG4-RD from other iMCD-IPL was 5381 mg/dL. CONCLUSIONS: iMCD-IPL cases with high serum IgG levels (>5000 mg/dL) were likely to meet the diagnostic criteria for IgG4-RD because of the numerous IgG4-positive cells observed. A combination of clinical presentations, laboratory values including the serum IgG4/IgG ratios and histological analysis is crucial for diagnosis of IgG4-RD and iMCD-IPL.

Analysis of Notch1 protein expression in methotrexate-associated lymphoproliferative disorders.

Methotrexate (MTX)-associated lymphoproliferative disorder (MTX-LPD) is a lymphoproliferative disorder in patients treated with MTX. The mechanism of pathogenesis is still elusive, but it is thought to be a complex interplay of factors, such as underlying autoimmune disease activity, MTX use, Epstein-Barr virus infection, and aging. The NOTCH genes encode receptors for a signaling pathway that regulates various fundamental cellular processes, such as proliferation and differentiation during embryonic development. Mutations of NOTCH1 have been reported in B-cell tumors, including chronic lymphocytic leukemia/lymphoma, mantle cell lymphoma, and diffuse large B-cell lymphoma (DLBCL). Recently, it has also been reported that NOTCH1 mutations are found in post-transplant lymphoproliferative disorders, and in CD20-positive cells in angioimmunoblastic T-cell lymphoma, which might be associated with lymphomagenesis in immunodeficiency. In this study, to investigate the association of NOTCH1 in the pathogenesis of MTX-LPD, we evaluated protein expression of Notch1 in nuclei immunohistochemically in MTX-LPD cases [histologically DLBCL-type (n = 24) and classical Hodgkin lymphoma (CHL)-type (n = 24)] and de novo lymphoma cases [DLBCL (n = 19) and CHL (n = 15)]. The results showed that among MTX-LPD cases, the expression of Notch1 protein was significantly higher in the DLBCL type than in the CHL type (P < 0.001). In addition, among DLBCL morphology cases, expression of Notch1 tended to be higher in MTX-LPD than in the de novo group; however this difference was not significant (P = 0.0605). The results showed that NOTCH1 may be involved in the proliferation and tumorigenesis of B cells under the use of MTX. Further research, including genetic studies, is necessary.

Long-term trends in mortality from adverse effects of medical treatment in older populations in the United States from 1990 to 2019.

BACKGROUND: Adverse Effects of Medical Treatment (AEMT) refer to unintended harm caused by medical care and are a significant public health concern. OBJECTIVE: This study utilizes the Global Burden of Disease database to investigate AEMT mortality trends among older adults in the United States from 1990 to 2019, focusing on crude mortality rates and age-standardized mortality rate trends by age group and sex. METHODS: The study employs cause-of-death ensemble modeling and statistical analysis to examine crude and age-standardized mortality rates (ASRs) for AEMT in older age groups and identify trends in mortality due to AEMTs in those over 65 years of age in the United States. Trends in the ASR of AEMT were analyzed using the Joinpoint regression model. RESULTS: AEMT mortality rates increased among older adults from 2012 to 2019, with the highest increase observed in the 95 years or older age group. Significant differences were noted in AEMT mortality rates between older men and women, with older men having higher rates and showing an upward trend, while rates among older women decreased from 1990 to 2019. CONCLUSION: The study highlights an overall increase in ASR related to AEMT among older adults in the United States, with men shown to have a greater susceptibility to death from AEMT. Increased attention toward the detrimental impact of AEMT on our aging population, particularly for men, in conjunction with reinforcement of health policies and education, is warranted.

Cliff diving leading to commotio cordis in a patient with Ebstein's anomaly.

In this case report, we describe a 23-year-old male with Ebstein's anomaly who experienced out-of-hospital cardiac arrest due to commotio cordis following cliff diving. The patient previously underwent a Cone procedure and re-do reduction tricuspid valvuloplasty. Comprehensive investigations revealed no new ischemic events or structural abnormalities. He received an implantable cardioverter-defibrillator during an uneventful outpatient visit. This is the first reported case of commotio cordis in a patient with Ebstein's anomaly, suggesting a potential increased risk in individuals with congenital heart diseases. This highlights the significance of tertiary prevention in such cases. Learning objective: Through this case, readers may be able to review the incidence and electrical abnormalities leading to sudden cardiac death in patients with commotio cordis, the clinical presentation and mechanism of injury, and the current consensus regarding the management of commotio cordis.

Defining optimal blood pressure control for pre-transplant end-stage renal disease patients: scoping review.

BACKGROUND: Strict blood pressure control is essential to prevent cardiovascular disease and is associated with decreased mortality. However, in patients with end-stage renal disease awaiting renal transplantation, the level of optimal blood pressure control is not yet defined. METHODS: Following the PRISMA Extension for Scoping Reviews, we searched MEDLINE and EMBASE for all peer-reviewed articles using keywords including 'end-stage renal disease', 'blood pressure', and 'pre-transplant' from their inception to 7 August 2022. RESULTS: Seven observational studies, including one population-based study, were included in the review. Most studies investigated factors associated with post-transplant graft failure or mortality. There was considerable heterogeneity in defining optimal pre-transplant blood pressure measurement frequency among studies (average of three measurements vs. single measurement). One study suggested that low pre-transplant diastolic blood pressure (<50 mmHg) was associated with lower odds of delayed graft failure and mortality. Two studies noted that pre-transplant hypertension, or clinical criteria of hypertension that were present prior to transplant, was associated with post-transplant adverse outcomes. In contrast, one study noted that pre-transplant sustained hypotension with mean blood pressure <80 mmHg, was associated with a higher frequency of delayed graft failure. CONCLUSION: This systematic review summarizes the current evidence regarding the relationship between pre-transplant blood pressure control and post-transplant outcomes in end-stage renal disease patients. While the results from the included studies are mixed, more stringent blood pressure control than currently practiced may be beneficial to decrease graft failure and mortality in this patient population.

Intravenous Iron for Heart Failure: Updated Systematic Review and Meta-Analysis.

While the administration of intravenous (IV) iron to those with heart failure has been implicated to be associated with a possible reduction in hospitalizations and improvement in symptoms, a recent large multicenter trial only showed modest benefits in reducing hospitalization, necessitating the updated systematic review. We conducted a systematic review and meta-analysis, searching the MEDLINE and EMBASE database until January 9, 2023. Outcomes included total heart failure hospitalizations, first heart failure hospitalization, six-minute walk test (6MWT) distance, and incidence of infection. There were 13 studies with 3410 participants (1,790 with IV iron). Pooled analysis that reported the incidence of cardiovascular death showed that patients with IV iron did not have significantly lower odds of cardiovascular death or first heart failure hospitalization. In contrast, those who received IV iron had significantly lower total heart failure hospitalization (pooled odds ratio (OR) 0.63, 95% confidence interval (CI) 0.44-0.90, I2 59.0%, P = .017) and a composite of cardiovascular death and first heart failure hospitalization (pooled OR 0.55, 95% CI 0.47-0.64, I2 0%, P = .656). While the efficacy is modest, IV iron therapy could be associated with reduced hospitalization for heart failure without significant adverse events.

Eosinophilic myocarditis: systematic review.

OBJECTIVE: In clinical practice, patients with eosinophilic myocarditis (EM) may forgo the gold standard diagnostic procedure, endomyocardial biopsy (EMB), although it is highly recommended in guidelines. This systematic review aims to summarise current approaches in diagnosing and treating EM with a particular emphasis on the utilisation and value of alternative diagnostic methods. METHODS: Following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses statement, we searched MEDLINE and EMBASE for all peer-reviewed articles using the keywords "eosinophilic myocarditis" from their inception to 10 September 2022. RESULTS: We included 239 articles, including 8 observational studies and 274 cases, in this review. The median patient age was 45 years. Initial presentations were non-specific, including dyspnoea (50.0%) and chest pain (39.4%). The aetiologies of EM were variable with the most common being idiopathic (28.8%) and eosinophilic granulomatosis polyangiitis (19.3%); others included drug-induced (13.1%) and hypereosinophilic syndrome (12.8%). 82.4% received an EM diagnosis by EMB while 17.6% were diagnosed based on clinical reasoning and cardiac MRI (CMR). CMR-diagnosed patients exhibited a better risk profile at diagnosis, particularly higher left ventricular ejection fraction and less need for inotropic or mechanical circulatory supports. Glucocorticoids were the primary treatment with variability in dosages and regimens. CONCLUSION: EMB is the mainstay for diagnostic testing for EM. CMR is potentially helpful for screening in appropriate clinical scenarios. Regarding treatment, there is no consensus regarding the optimal dosage of corticosteroids. Large clinical trials are warranted to further explore the utility of CMR in the diagnosis of EM and steroid regimen in treating EM.

Clinical Characteristics of Retroperitoneal Fibrosis Patients at a Tertiary Hospital in Japan.

Retroperitoneal fibrosis (RPF) is a rare cause of hydronephrosis and progressive renal dysfunction with unidentified origin. RPF is categorized into idiopathic RPF with/without immunoglobulin G4 (IgG4)-related disease (IgG4-RD), and secondary RPF. Identifying the underlying cause is challenging and often associated with delayed diagnosis or therapeutic interventions. We investigated RPF's clinical characteristics based on different etiologies and factors that may help distinguish the underlying causes. We analyzed the cases of 49 patients with RPF that was radiographically diagnosed at our institution (2008-2022). The cohort was 77.6% males; 75.5% had idiopathic RPF and 24.5% had secondary RPF. Among the idiopathic patients, 54.1% had IgG4-RD. The patients were likely to have abdominal pain, lower back pain/lumbago, and constitutional symptoms including generalized fatigue and fever. The idiopathic patients were likely to have higher serum IgG4 and IgG levels and lower serum C3 levels compared to secondary RPF. The IgG4-RPF patients were likely to have higher serum IgG4 levels and lower serum C-reactive protein, ferritin, and C3 levels compared to the idiopathic RPF patients without IgG4-RD. These findings might reflect underlying systemic inflammatory responses. Comprehensive laboratory testing, including serum inflammatory markers and immunological panels, is recommended for radiologically diagnosed RPF patients.

Factors Associated with Favorable Outcomes in Cardiac Arrest and Target Temperature Management.

Current guidelines strongly recommend providing targeted temperature management (TTM) after cardiac arrest, but hypothalamic dysregulation may confound TTM's impact on a patient's ultimate outcome. Although time to reach target temperature has largely been viewed as a process measure for TTM protocols, the difference between initial presenting temperature and target temperature (Δ-temperature) may be a potential surrogate marker of hypothalamic dysregulation. We performed a retrospective observational study to explore whether Δ-temperature was associated with neurologic outcomes and mortality. We included 86 patients (53 with out-of-hospital cardiac arrest [OHCA] and 33 with in-hospital cardiac arrest [IHCA]) in our analysis; more than half of the patients were cooled to 33°C (56.9% in OHCA and 57.6% in IHCA). In univariate logistic regression analysis, Δ-temperature alone did not appear to be statistically associated with mortality or neurologic outcomes regardless of target temperature. In exploratory analysis, longer time from TTM initiation-to-target was associated with worse neurological outcomes in the 33°C target (odds ratio = 0.996, 95% confidence interval = 0.992-1.000). Further research investigating the impact of hypothalamic dysregulation and Δ-temperature as well as the rate of cooling may be warranted to elucidate additional factors contributing to outcomes after cardiac arrest. In addition, our study population was noted to have a higher proportion of Asians and Native Hawaiians/Pacific Islanders, with a potential disparity in outcomes. Future studies may be warranted to ensure generalizability of TTM protocols and findings across populations.

The risk of new heart failure associated with protease inhibitor: Systematic scoping review.

BACKGROUND: Protease inhibitors (PIs) have contributed to the long-term survival of persons with human immunodeficiency virus (PHIV). While there is a concern linking protease inhibitors to an increased risk of heart failure (HF), the evidence linking protease inhibitors and heart failure has been uncertain. METHODS: Following the PRISMA Extension for Scoping Reviews, we searched MEDLINE and EMBASE for peer-reviewed articles using keywords including "protease inhibitor," "heart failure," and "human immunodeficiency virus" from their inception to December 21, 2022. RESULTS: Five articles, including three observational studies and two randomized controlled trials, were included in the review. While protease inhibitors seem to be associated with atherosclerotic cardiovascular disease through their effects on metabolic markers, there is scarce evidence suggesting a direct association between protease inhibitors and heart failure. Although one study showed a possible correlation between protease inhibitor use and lower left ventricular ejection fraction and increased heart failure admission, the results were subject to confounders, and participants had poor medication adherence. CONCLUSION: Although current data are conflicting, there could be an association between PIs and HF in PHIV. Future prospective studies are warranted to evaluate the incidence of heart failure stratified on the generation of PIs and with adjustment for other metabolic risk factors.