Clinical Presentation Research Articles

Understanding Arrhythmia-Induced Cardiomyopathy: Symptoms and Treatments.

Arrhythmia-induced cardiomyopathy is a complex condition that causes a decline in heart function as a result of irregular heart rhythms. This disorder highlights the link between irregular heart rhythm and heart failure, necessitating prompt identification and intervention. It often occurs due to ongoing fast heart rhythms like atrial fibrillation or tachycardia. Understanding the mechanisms, symptoms, and available treatments is essential for enhancing patient outcomes given the complicated nature of the condition. This article delves into various aspects of arrhythmia-induced cardiomyopathy, including pathogenesis, clinical presentation, diagnostic methods, epidemiology, typical arrhythmias associated with the condition, and management options. It assesses patients' future outlook and necessary follow-up, aiming to provide healthcare providers with a comprehensive understanding of how to handle this intricate condition. The article emphasizes the important effect an integrative approach can have on both patients' lives and the clinical consequences of diagnosing and treating this condition. This extensive understanding enhances the resources at the disposal of physicians, enabling targeted treatments that enhance cardiomyopathy by targeting arrhythmia regulation. More research and development are needed in the field of cardiomyopathy and arrhythmia relationship. The presentation urges the medical field to delve deeper into the complexities of illness by emphasizing the need for continuous research and a multifaceted treatment plan. By combining these understandings, our goal is to enhance patient outcomes and create opportunities for further studies on cardiovascular wellness.

Histopathological Investigation of Progressive Encephalomyelitis with Rigidity and Myoclonus: An Autopsy Case Characterized by Oculomotor Dysfunction and Autonomic Failure.

Progressive encephalomyelitis with rigidity and myoclonus (PERM) is a rare disease associated with the presence of anti-glycine receptor (GlyR) antibodies. We herein report an autopsy case of an 80-year-old man diagnosed with anti-GlyR antibody-positive PERM who presented with symptoms of oculomotor dysfunction and autonomic failure. Despite intensive immunotherapy, the neurological symptoms showed almost no improvement, and the patient succumbed to aspiration pneumonia and bacterial translocation. Postmortem pathology revealed mild inflammatory changes and neuronal loss that were disproportionate to a severe clinical presentation. These results suggest that the clinical symptoms of PERM may result from antibody-mediated GlyR internalization, leading to neuronal disinhibition, rather than a neuroinflammatory signature.

Acute primary angle closure during the Omicron outbreak

AIM: To investigate Omicron's impact on clinical presentation of acute primary angle closure (APAC) in China. METHODS: A consecutive case series with historical controls was conducted at Shenzhen Eye Hospital, the largest specialized hospital in Shenzhen, China. Medical records from a two-month period during the Omicron pandemic (December 1, 2022, to January 31, 2023) were compared with records from two control groups (12/2018–1/2019 and 12/2021–1/2022) before pandemic. Patients with APAC were included, and the prevalence of APAC and demographic characteristics in Omicron-infected and non-infected patients were compared. RESULTS: Seventy-one (23.43%) out of 303 patients were diagnosed with APAC in the pandemic cohort, which was 2.98 and 2.61 times higher than that in control cohorts (7.87% in 2019, 8.96% in 2022, P<0.001). The pandemic cohort has significantly higher Omicron-infected rate (78.87% vs 0 vs 0; P<0.001), lower proportion of glaucoma history (16.90% vs 42.86% vs 41.67%, P=0.005), higher surgical rate (95.77% vs 83.33% vs 78.57%, P=0.024), higher total medical costs and larger pupil diameter (5.63±0.15 vs 4.68±0.15 vs 4.69±0.22 mm, P<0.01). In 83% Omicron-infected patients, ocular symptoms appeared within 3d after systemic symptoms onset. In multivariate analysis, Omicron infection (P<0.001) was the only independent predictor of pupil diameter. CONCLUSION: In the Omicron epidemic in China, there is an increase of prevalence and severity of APAC, particularly focusing on the first 3d following infection.

Retinitis pigmentosa and stem cell therapy

Retinitis pigmentosa (RP) is a group of genetic disorders characterized by progressive degeneration of photoreceptors and retinal pigment epithelium (RPE) cells. Its main clinical manifestations include night blindness and progressive loss of peripheral vision, making it a prevalent debilitating eye disease that significantly impacts patients' quality of life. RP exhibits significant phenotypic and genetic heterogeneity. For instance, numerous abnormal genes are implicated in RP, resulting in varying clinical presentations, disease progression rates, and pathological characteristics among different patients. Consequently, gene therapy for RP poses challenges due to these complexities. However, stem cells have garnered considerable attention in the field of RPE therapy since both RPE cells and photoreceptors can be derived from stem cells. In recent years, a large number of animal experiments and clinical trials based on stem cell transplantation attempts, especially cord blood mesenchymal stem cell (MSC) transplantation and bone marrow-derived MSC transplantation, have confirmed that stem cell therapy can effectively and safely improve the outer retinal function of the RP-affected eye. However, stem cell therapy also has certain limitations, such as the fact that RP patients may involve multiple types of retinal cytopathia, which brings great challenges to stem cell transplantation therapy, and further research is needed to solve various problems faced by this approach in the clinic. Through comprehensive analysis of the etiology and histopathological changes associated with RP, this study substantiates the efficacy and safety of stem cell therapy based on rigorous animal experimentation and clinical trials, while also highlighting the existing limitations that warrant further investigation.

Clinical Presentation and Outcomes of Diabetic Ketoacidosis in Pregnancy.

To examine the presentation, management, and outcomes of pregnancies complicated by diabetic ketoacidosis (DKA) in a contemporary obstetric population. This is a case series of all admissions for DKA during pregnancy at a single Midwestern academic medical center over a 10-year period. Diabetic ketoacidosis was defined per the following diagnostic criteria: anion gap more than 12 mEq/L, pH less than 7.30 or bicarbonate less than 15 mEq/L, and elevated serum or urine ketones. Demographic information, clinical characteristics, and maternal and neonatal outcomes were assessed. Patient characteristics and clinical outcomes were compared between individuals with type 1 and those with type 2 diabetes mellitus. Between 2012 and 2021, there were 129 admissions for DKA in 103 pregnancies in 97 individuals. Most individuals (n=75, 77.3%) admitted for DKA during pregnancy had type 1 diabetes. The majority of admissions occurred in the third trimester (median gestational age 29 3/7 weeks). The most common precipitating factors were vomiting or gastrointestinal illness (38.0%), infection (25.6%), and insulin nonadherence (20.9%). Median glucose on admission was 252 mg/dL (interquartile range 181-343 mg/dL), and 21 patients (17.6%) were admitted with euglycemic DKA. Fifteen admissions (11.6%) were to the intensive care unit. Pregnancy loss was diagnosed during admission in six individuals (6.3%, 95% CI, 2.3-13.7%). Among pregnant individuals with at least one admission for DKA, the median gestational age at delivery was 34 6/7 weeks (interquartile range 33 2/7-36 3/7 weeks). Most neonates (85.7%, 95% CI, 76.8-92.2%) were admitted to the neonatal intensive care unit and required treatment for hypoglycemia. The cesarean delivery rate was 71.9%. Despite similar hemoglobin A1C values before pregnancy and at admission, individuals with type 1 diabetes had higher serum glucose (median [interquartile range], 256 mg/dL [181-353 mg/dL] vs 216 mg/dL [136-258 mg/dL], P=.04) and higher serum ketones (3.78 mg/dL [2.13-5.50 mg/dL] vs 2.56 mg/dL [0.81-4.69 mg/dL] mg/dL, P=.03) on admission compared with those with type 2 diabetes. Individuals with type 2 diabetes required intravenous insulin therapy for a longer duration (55 hours [29.5-91.5 hours] vs 27 hours [19-38 hours], P=.004) and were hospitalized longer (5 days [4-9 days] vs 4 days [3-6 days], P=.004). Diabetic ketoacidosis occurred predominantly in pregnancies affected by type 1 diabetes. Individuals with type 1 diabetes presented with greater DKA severity but achieved clinical resolution more rapidly than those with type 2 diabetes. These results may provide a starting point for the development of interventions to decrease maternal and neonatal morbidity related to DKA in the modern obstetric population.

Paediatric Uveitis - the uniqueness in clinical presentation and the efficacy of biologics treatment

AimsTo evaluate unique clinical characteristics of paediatric uveitis in our locality and treatment outcomes especially the efficacy of biologics.MethodsThis was a retrospective cohort.Results37 paediatric uveitis cases involving 67 eyes were included. Male-to-female ratio was 1:1.3. Mean age of uveitis onset was 11 ± 3.7 (4–18). 81.1% cases suffered from bilateral uveitis. 75.7% cases were chronic uveitis. Nearly half of the cases (40.5%) presented with anterior uveitis. The predominant diagnosis of uveitis in our cohort was idiopathic. Unlike studies from other populations, the associated systemic conditions in this mostly Chinese cohort were Behçet’s disease (8.1%), tubulointerstitial nephritis and uveitis (8.1%) and HLA-B27 associated uveitis (8.1%). Steroid response was a common phenomenon, observed in 40.5% of cases. The most common complication was posterior synechiae (45.9%), followed by cataract (37.8%), glaucoma (27.0%), band keratopathy (18.9%) and macular oedema (13.5%). 3/37 patients encountered either first attack of uveitis or flare after receiving COVID-19 vaccine. 54.1% of patients required systemic steroid for disease control. The majority required steroid sparing immunotherapy, including Methotrexate (43.2%), Mycophenolate Mofetil (24.3%), Cyclosporine A (8.1%), Azathioprine (5.4%) and Tacrolimus (2.7%). Resistant cases required biologics including tumour necrosis factor alpha inhibitors (Adalimumab 32.4%, Infliximab 2.7%) and interleukin-6 inhibitors (Tocilizumab 2.7%).ConclusionsClinical presentation of the local paediatric uveitis differs from previously described features in Caucasian and other populations. According to our experience as a tertiary eye centre, Behçet’s disease, tubulointerstitial nephritis and uveitis and HLA-B27 associated uveitis were more often encountered than Juvenile Idiopathic Arthritis associated uveitis. Our report evaluated the efficacy of immunomodulatory therapy and biologics in controlling uveitis and reducing ocular complications.

Neurological manifestations of ehrlichiosis among a cohort of patients: prevalence and clinical symptoms

BackgroundEhrlichiosis is a potentially fatal tick-borne disease that can progress to involve the central nervous system (CNS) (i.e., neuro-ehrlichiosis), particularly in cases where diagnosis and treatment are delayed. Despite a six-fold national increase in the incidence of ehrlichiosis over the past 20 years, recent data on the prevalence and manifestations of neuro-ehrlichiosis are lacking.MethodsWe conducted a retrospective chart review of all patients tested for ehrlichiosis at University of North Carolina Health facilities between 2018 and 2021 and identified patients who met epidemiological criteria for ehrlichiosis as established by the Council of State and Territorial Epidemiologists and employed by the Centers for Disease Control and Prevention. We estimated the prevalence of neurological symptoms and described the spectrum of neurological manifestations in acute ehrlichiosis, documenting select patient cases in more detail in a case series.ResultsOut of 55 patients with confirmed or probable ehrlichiosis, five patients (9.1%) had neurologic symptoms, which is notably lower than previous estimates. Neurological presentations were highly variable and included confusion, amnesia, seizures, focal neurological deficits mimicking ischemic vascular events, and an isolated cranial nerve palsy, though all patients had unremarkable neuroimaging at time of presentation. All but one patient had risk factors for severe ehrlichiosis (i.e., older age, immunosuppression).ConclusionsNeuro-ehrlichiosis may lack unifying patterns in clinical presentation that would otherwise aid in diagnosis. Clinicians should maintain a high index of suspicion for neuro-ehrlichiosis in patients with acute febrile illness, diverse neurological symptoms, and negative neuroimaging in lone star tick endemic regions.

High disease burden and healthcare resource usage in patients with acute porphyria-A population-based analysis.

Acute porphyria is a chronic recurrent disease with late diagnosis, heterogeneous clinical presentations and potentially devastating complications. The study aimed at providing real-world evidence on the natural course of acute porphyria, patient characteristics, disease burden, and healthcare utilization before diagnosis. This observational study used anonymized claims data covering 8 365 867 persons from German statutory health insurance, spanning 6 years (2015-2020). Patients with at least one diagnosis of acute porphyria during the index period (2019-2020) were classified into three groups by attack frequency. These findings were compared with two age- and sex-adjusted reference groups: the general population and fibromyalgia patients. Prevalence over the index period was calculated for all porphyria patients and those with active acute porphyria. We revealed a prevalence of 79.8 per 1 000 000 for acute porphyria, with 12.9 per 1 000 000 being active cases. Acute porphyria patients, particularly with frequent attacks, demonstrated a higher comorbidity burden compared to the general population. Within the year before the recorded diagnosis, patients with acute porphyria required a median of 23.0 physician visits, significantly higher than the general population's 16.0. Additionally, 33.8% were hospitalized at least once during this period, a notably higher proportion than the general population (19.3%). This study's findings, collected before the introduction of givosiran, as the first approved preventive therapy for acute porphyria in Europe, highlight the need for healthcare strategies and policies tailored to the complex needs of acute porphyria patients. The significant healthcare demands, heightened comorbidity burden, and increased healthcare system utilization emphasize the urgency of developing a comprehensive support infrastructure for these patients. Also, these acute porphyria real-world findings provide additional insights on disease characteristics in Germany.

Management of metastatic pheochromocytomas and paragangliomas: when and what

Recently, the treatment landscape for metastatic pheochromocytomas and paragangliomas (MPPGL) has seen both progress and setbacks. We provide an up-to-date review of the multimodality management of MPPGL and discuss novel opportunities and current challenges in the treatment landscape. Given the unique clinical presentation of MPPGL, we discuss the management of hormone-related clinical sequelae and traditional modalities of therapy. Advances in the understanding of the molecular biology of these diverse tumors have enabled novel strategies such as augmenting DNA damage by targeted delivery of radionuclides such as 131I and 177Lu, abrogating tumor angiogenesis, hypoxia resistance, and DNA damage repair. Despite progress, we address the significant challenges still faced by patients and researchers engaged in efforts to improve outcomes in these rare cancers.

Ineffective esophageal motility: Characterization and outcomes across pediatric neurogastroenterology and motility centers in the United States.

Ineffective esophageal motility (IEM) on high-resolution manometry (HRM) is not consistently associated with specific clinical syndromes or outcomes. We evaluated the prevalence, clinical features, management, and outcomes of pediatric IEM patients across the United States. Clinical and manometric characteristics of children undergoing esophageal HRM during 2021-2022 were collected from 12 pediatric motility centers. Clinical presentation, test results, management strategies, and outcomes were compared between children with IEM and normal HRM. Of 236 children (median age 15 years, 63.6% female, 79.2% Caucasian), 62 (23.6%) patients had IEM, and 174 (73.7%) patients had normal HRM, with similar demographics, medical history, clinical presentation, and median symptom duration. Reflux monitoring was performed more often for IEM patients (25.8% vs. 8.6%, p = 0.002), but other adjunctive testing was similar. Among 101 patients with follow-up, symptomatic cohorts declined in both groups in relation to the initial presentation (p > 0.107 for each comparison) with management targeting symptoms, particularly acid suppression. Though prokinetics were used more often and behavioral therapy less often in IEM (p ≤ 0.015 for each comparison), symptom outcomes were similar between IEM and normal HRM. Despite a higher proportion with residual dysphagia on follow-up in IEM (64.0% vs. 39.1%, p = 0.043), an alternate mechanism for dysphagia was identified more often in IEM (68.8%) compared to normal HRM (27.8%, p = 0.017). IEM is a descriptive manometric pattern rather than a clinical diagnosis requiring specific intervention in children. Management based on clinical presentation provides consistent symptom outcomes.

Evolving Trends in the Diagnosis and Management of Keratoconus Over 3 Decades.

To report the trends in the clinical presentation, diagnosis, and management of keratoconus (KC) in patients presenting to a multitier hospital network over 33 years. This retrospective study included patients with KC presenting from 1987 to 2020. Patients with KC in at least 1 eye were included. The data of 20,576 patients included in this study were retrieved from the electronic medical record database. Data on patient demographics, ocular diagnosis, contact lens usage, and surgical history were collected and exported for analysis. Descriptive statistics and chi square test were used for statistical analysis. A total of 20,576 KC cases were seen from 1987 to 2020. Mean age at diagnosis reduced from 29.3 years (1987-1991) to 22.2 years (2016-2020). Adults (76.64%) were commonly affected with a majority being male (61.25%). Use of contact lenses increased from 4.34% (2002-2006) to 11.23% (2016-2020). Of the total number of surgical interventions, collagen crosslinking (CXL) constituted more than 1/5th (22.28%) between 2007 and 2011, which increased to more than half between 2012 and 2016 (53.61%) and almost 3/4th (72.53%) between 2017 and 2020. Advances in lamellar surgery have led to more patients undergoing deep anterior lamellar keratoplasty (DALK) as compared with penetrating keratoplasty. In the last 8 years, 17.2% patients underwent surgery and the most common was CXL (14.77%) followed by DALK (1.72%) and penetrating keratoplasty (1.04%). We report the trends in the clinical presentation and management of KC over 3 decades. In the last 33 years, advances in diagnostics have helped diagnose KC earlier. Contact lenses and advances in CXL have reduced the number of keratoplasties in KC, DALK being more common.

Multicenter registry of multisystem inflammatory syndrome in children (MIS-C) and Paired comparison with Kawasaki disease

ObjectivesThis study aimed to identify clinical characteristics to differentiate multisystem inflammatory syndrome in children (MIS-C) and Kawasaki disease (KD) in Taiwan, an island with a delayed cluster of MIS-C and a high incidence of KD. Additionally, we studied risk factors for developing severe complications in patients with MIS-C. MethodsWe conducted a retrospective, multicenter, cohort, and observational study that linked data on patients with MIS-C between May and December 2022 and patients with KD between 2019 and 2021 from 12 medical centers. Hemodynamic compromise, defined as the need for inotropic support or fluid challenge, was recorded in patients with MIS-C. We also evaluated maximal coronary Z-scores before treatment and one month after disease onset. ResultsA total of 83 patients with MIS-C and 466 patients with KD were recruited. A 1:1 age and gender-matched comparison of 68 MIS-C and KD pairs showed that MIS-C patients had a lower percentage of positive BCG red halos, lower leukocyte/platelet counts, more gastrointestinal symptoms, and a higher risk of hemodynamic compromise. In Taiwan, 38.6% of MIS-C patients experienced hemodynamic compromise, with presence of conjunctivitis and elevated levels of procalcitonin (>1.62 ng/mL) identified as independent risk factors. ConclusionsWe identified two independent risk factors associated with hemodynamic compromise in MIS-C patients. The comparison between matched MIS-C and KD patients highlighted significant differences in clinical presentations, like BCG red halos, which may aid in the differential diagnosis of the two disease entities, especially in regions with a high incidence rate of KD.

Rheumatoid meningitis: a case series report and review of modern therapeutic schemes and outcome.

Rheumatoid meningitis (RM) is an extremely rare extra-articular complication of rheumatoid arthritis (RA), with approximately 165 cases reported world-wide. RM exhibits a broad range of symptoms, with stroke-like episodes and seizures being the most common manifestations. The primary differential diagnoses include vascular and infectious diseases. The influence of immunomodulatory medications on the pathophysiology of RM remains unclear. There are no consensus guidelines on therapeutic regimen. We present four patients with prior history of RA that developed different neurological syndromes in correlation to radiological leptomeningitis. Clinical presentations, comorbid conditions, supplementary diagnostic assessments, treatments, and prognosis are provided. A literature review of recent immunosuppressive management in RM patients was performed. Three patients presented to hospital with recurrent focal seizures. Only two suffered meningism, reporting headache and fever. Magnetic resonance imaging (MRI) showed different grades of leptomeningitis across all cases. Notably, three cases demonstrated bilateral involvement extending to the pachymeninges. Two patients exhibited pronounced CSF mononuclear inflammation while extended microbiological evaluations yielded negative results. Two patients required biopsy for confirmation. The initiation of immunosuppressive therapy marked a turning point for three patients who previously exhibited progressive deterioration. Mortality was absent in all cases. Our experience remarks the elusive nature of RM. Rigorous exclusionary diagnostics are imperative to differentiate RM from mimicking conditions. Clinical manifestations oscillate between transient episodes and progressive neurological impairments, punctuated by frequent epileptic seizures. In scenarios where clinical worsening persists or where clinical and radiological evaluations are inconclusive, aggressive immunosuppressive therapy is recommended.

Sleep EEG signatures in mouse models of 15q11.2-13.1 duplication (Dup15q) syndrome

BackgroundSleep disturbances are a prevalent and complex comorbidity in neurodevelopmental disorders (NDDs). Dup15q syndrome (duplications of 15q11.2-13.1) is a genetic disorder highly penetrant for NDDs such as autism and intellectual disability and it is frequently accompanied by significant disruptions in sleep patterns. The 15q critical region harbors genes crucial for brain development, notably UBE3A and a cluster of gamma-aminobutyric acid type A receptor (GABAAR) genes. We previously described an electrophysiological biomarker of the syndrome, marked by heightened beta oscillations (12-30 Hz) in individuals with Dup15q syndrome, akin to electroencephalogram (EEG) alterations induced by allosteric modulation of GABAARs. Those with Dup15q syndrome exhibited increased beta oscillations during the awake resting state and during sleep, and they showed profoundly abnormal NREM sleep. This study aims to assess the translational validity of these EEG signatures and to delve into their neurobiological underpinnings by quantifying sleep physiology in chromosome-engineered mice with maternal (matDp/ + mice) or paternal (patDp/ + mice) inheritance of the full 15q11.2-13.1-equivalent duplication, and mice with duplication of just the UBE3A gene (Ube3a overexpression mice; Ube3a OE mice) and comparing the sleep metrics with their respective wildtype (WT) littermate controls.MethodsWe collected 48-h EEG/EMG recordings from 35 (23 male, 12 female) 12–24-week-old matDp/ + , patDp/ + , Ube3a OE mice, and their WT littermate controls. We quantified baseline sleep, sleep fragmentation, spectral power dynamics during sleep states, and recovery following sleep deprivation. Within each group, distinctions between Dup15q mutant mice and WT littermate controls were evaluated using analysis of variance (ANOVA) and student’s t-test. The impact of genotype and time was discerned through repeated measures ANOVA, and significance was established at p < 0.05.ResultsOur study revealed that across brain states, matDp/ + mice mirrored the elevated beta oscillation phenotype observed in clinical EEGs from individuals with Dup15q syndrome. Time to sleep onset after light onset was significantly reduced in matDp/ + and Ube3a OE mice. However, NREM sleep between Dup15q mutant and WT littermate mice remained unaltered, suggesting a divergence from the clinical presentation in humans. Additionally, while increased beta oscillations persisted in matDp/ + mice after 6-h of sleep deprivation, recovery NREM sleep remained unaltered in all groups, thus suggesting that these mice exhibit resilience in the fundamental processes governing sleep-wake regulation.ConclusionsQuantification of mechanistic and translatable EEG biomarkers is essential for advancing our understanding of NDDs and their underlying pathophysiology. Our study of sleep physiology in the Dup15q mice underscores that the beta EEG biomarker has strong translational validity, thus opening the door for pre-clinical studies of putative drug targets, using the biomarker as a translational measure of drug-target engagement. The unaltered NREM sleep may be due to inherent differences in neurobiology between mice and humans. These nuanced distinctions highlight the complexity of sleep disruptions in Dup15q syndrome and emphasize the need for a comprehensive understanding that encompasses both shared and distinct features between murine models and clinical populations.

Psychopathological and Organic Features of Atypical Anorexia Nervosa in Developmental Age: A Systematic Review

Purpose: This study aimed to comprehensively report the epidemiological and clinical features of atypical anorexia nervosa (AAN) in children and adolescents. Methods: In May 2024, a systematic review was performed using Medline, Cochrane Library, ClinicalTrials.gov, and relevant websites. Following PRISMA guidelines, 234 articles were screened for studies on DSM-5-defined AAN. A standardized checklist—the JBI critical appraisal tool—was adopted in assessing methodology, and 13 retained studies passed the screening and critical appraisal process for the final review. The Newcastle–Ottawa Scale was utilized to assess the risk of bias in cohort and case–control studies, ensuring a comprehensive evaluation of methodological quality. Results: AAN prevalence in young age groups is 2.8%, with a cumulative 2.8% incidence over 8 years. Incidence is 366 per 100,000 person-years, and the average episode duration is 11.6 months, with a 71% remission rate. Diagnostic persistence for AAN is less stable than other restrictive feeding and eating disorders (FEDs). AAN individuals exhibit higher EDE-Q scores, more severe distress, and distinct BMI differences compared to those with anorexia nervosa and controls. The diagnostic transition from the DSM-IV to the DSM-5 shows that AAN patients are predominantly female, slightly older, and with higher weight. Conclusions: This study yields concrete insights into the features of AAN in the developmental age, highlighting demographic variations, clinical presentations, and treatment outcomes. Recognizing the unique challenges faced by AAN individuals is vital for tailoring effective interventions and improving overall care within the FED spectrum.

Influence of gender on clinical presentation, management practices and outcomes of ocular syphilis

Ocular syphilis is a re-emerging inflammatory eye disease with a clear gender imbalance, disproportionately affecting men. We investigated the impact of gender on the presentation, management practices and clinical outcomes of this condition. Data generated from a study of patients consecutively diagnosed with ocular syphilis who attended a subspecialist uveitis service at one of four hospitals in Brazil over a 30-month period were disaggregated for analysis by gender. Two-hundred and fourteen eyes (161 men and 53 women) of 127 patients (96 men and 31 women) were included. Posterior uveitis was the most common presentation in both men and women (80.1% vs. 66.7%, p > 0.05), but men were significantly more likely to have vitritis as a feature of their disease (49.4% versus 28.8%, p = 0.019). Three eyes of women had nodular anterior scleritis (p = 0.015). Men were more likely to undergo a lumbar puncture to assess for neurosyphilis (71.9% vs. 51.6%, p = 0.048), but men and women undergoing a lumbar puncture were equally likely to have a cerebrospinal fluid abnormality (36.2% vs. 25.0%, p = 0.393). All patients were treated with aqueous penicillin G or ceftriaxone, and there was a trend towards more men receiving adjunctive systemic corticosteroid treatment as part of their management (65.2% vs. 46.7%, p = 0.071). There were no significant differences in the age of presentation, bilaterality of disease, anatomical classification of uveitis, initial or final visual acuity, and rates of ocular complications between men and women. Our findings indicate that ocular syphilis has comparable outcomes in men and women, but that there are differences in the type of ocular inflammation and management practices between the genders.

Study on Clinical Profile and Treatment Outcome of Acute Paraquat Poisoning in an Intensive Care Unit in Bangladesh

Background: Paraquat, one of the most widely used herbicides, is a major cause of self-harm related death in Bangladesh resulting from multiple organ failure. The primary objective aimed to identify the demographic &amp; clinical characteristics of paraquat poisoning and the outcome of different treatment modalities. Materials and Methods: A prospective observational study was conducted over twenty-four months at the Intensive Care Unit of Dhaka Medical College Hospital, a tertiary care referral hospital.Between March 2022 and March 2024, patients presenting with acute paraquat toxicity who fulfilled inclusion and exclusion criteriawere selected by purposive sampling. A total of 42 patients were enrolled in the study.Demographic, clinical, and treatment particulars were collected. The study evaluated the outcomes, including length of stay in the intensive care unit (ICU), the need for mechanical ventilation&amp;hemodialysisandin-hospital mortality. SPSS version 25.0 was used for statistical analysis and reporting. Results: Among the patients, 54.8% were men with a median age of 23.31 years residing in rural areas (85.7%). All cases were suicidal, primarily due to family disputes (40.5%) and personal relationship issues (21.4%). Symptoms included shortness of breath (40.5%), disorientation (28.6%), difficulty in swallowing (14.3%), vomiting (9.5%), and low urine output (7.1%). Most ICU admissions occurred after 24 hours postingestion (59.5%). Organ involvement included acute kidney injury (57.14%), acute respiratory distress syndrome (50%), hepatic impairment (14.3%), pulmonary fibrosis (14.3%), and septic shock (2.4%). Methylprednisolone was administered to 21 patients (50%), with an equal number also receiving methylprednisolone plus cyclophosphamide (50%). Only 10 (23.80%) patients received gastric lavage.The median ICU stay was 6 days (IQR 4-9), while the Median hospital stay was 8 days (IQR 1-12). In-hospital mortality was high, and 41 out of 42 patients (97.8%) died. Conclusion: The rural population is mostly affected by paraquat poisoning for its easy availability,and clinical presentations, particularly pulmonary, renal and multi-systeminvolvement, are significantly associated with high mortality with the limited role of different treatment approaches. J MEDICINE 2024; 25: 129-135

Extrauterine leiomyomas in uncommon locations: two case reports and literature review

ObjectivesThis study aims to provide an overview of the diagnosis, treatment, and follow-up management of vulvar and vaginal leiomyomas through the presentation of two rare cases.MethodsDetailed clinical presentations, surgical procedures, histopathological examinations, and follow-up outcomes of two cases of vulvar and vaginal leiomyomas are described. Relevant literature is also reviewed to contextualize the findings.ResultsBoth patients underwent successful surgical excision of the leiomyomas with no perioperative or postoperative complications. Histopathological examinations confirmed the diagnosis of leiomyoma based on characteristic microscopic features and immunohistochemical analyses.ConclusionVulvar and vaginal leiomyomas are rare benign tumors that require careful evaluation for accurate diagnosis and appropriate management. Surgical excision remains the primary treatment modality, and long-term follow-up is essential for monitoring recurrence and ensuring favorable outcomes.

Clinical spectrum and risk factors of severe dengue among hospitalized patients - a cross-sectional study during the 2023 dengue epidemic in Bangladesh

Introduction: Dengue is a significant public health concern for Bangladesh with recurrent epidemics. This study aimed to investigate the clinical and biochemical presentations and identify risk factors for severe dengue among patients admitted to a tertiary care hospital during the 2023 epidemic in Bangladesh. Methods: This cross-sectional study was conducted at a tertiary care hospital in Bangladesh between July and September 2023. Data were collected from 176 adult patients with serologically confirmed dengue infection. Clinical and laboratory parameters were recorded, and severity of dengue was categorized according to WHO guidelines. Logistic regression was performed to identify predictors of severe dengue. Results: Of the 176 patients included, 113 had severe dengue and 63 had non-severe dengue. The mean age was 34.5 (SD 14.3) years, with a male-female ratio of 1:1.07. Conventional symptoms including fever, myalgia, headache, and retro-orbital pain were present in majority of patients, with warning signs present in twothirds of them. Laboratory findings showed leukocytopenia (53%), thrombocytopenia (25.6%), elevated hematocrit level (4.5%), elevated ALT, AST and ferritin level (36%, 24% and 50% respectively). Logistic regression identified persistent vomiting (aOR 3.22, 95% CI 1.41–8.04), clinical fluid accumulation (aOR 2.23, 95% CI 1.13–5.74), and elevated serum AST (aOR 3.13, 95% CI 1.30–7.79) as predictors of severe dengue. Conclusion: Most dengue cases presented with typical symptoms and warning signs, with associated laboratory abnormalities. Persistent vomiting, clinical fluid accumulation, and elevated serum AST levels upon admission could predict severe dengue. J MEDICINE 2024; 25: 121-128

Heat Shock Protein 70 Constitutes a Promising Novel Biomarker in Differential Diagnosis between Takotsubo Syndrome and Non-ST-Segment Elevation Myocardial Infarction

(1) Background: Due to similar clinical presentation and a lack of specific biomarkers, initial differentiation between Takotsubo syndrome (TTS) and non-ST-segment elevation myocardial infarction (NSTEMI) remains challenging in daily practice. Heat Shock Protein 70 (HSP70) is a novel biomarker that is recognized for its potential in the diagnosis and differentiation of cardiovascular conditions. (2) Methods: Data from a total of 156 patients were analyzed (32.1% NSTEMI, 32.7% TTS, and 35.3% controls). Serum concentrations of HSP70 were determined using ELISA and compared between patients and controls. ROC curve analysis, logistic regression analysis and propensity-score-weighted logistic regression were conducted. (3) Results: Concentrations of HSP70 were highest in patients with TTS (median 1727 pg/mL vs. ACS: median 1545 pg/mL vs. controls: median 583 pg/mL, p &lt; 0.0001). HSP70 was predictive for TTS in binary logistic regression analysis (B(SE) = 0.634(0.22), p = 0.004), which even remained significant after correction for possible confounders in propensity-score-weighted analysis. ROC curve analysis also revealed a significant association of HSP70 with TTS (AUC: 0.633, p = 0.008). (4) Conclusions: Based on our findings, HSP70 constitutes a promising biomarker for discrimination between TTS and NSTEMI, especially in combination with established cardiovascular biomarkers like pBNP or high-sensitivity cardiac troponin.