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This case describes a pediatric patient with a history of ichthyosis vulgaris and global anhidrosis who was diagnosed with erythema ab igne (EAI), a rare dermatosis resulting from chronic heat exposure. After developing progressive, reticulated brown patches on his extremities and abdomen, extensive diagnostic investigations were conducted to rule out autoimmune, vascular, and genetic etiologies. Bloodwork was unrevealing and biopsies showed histologic features closely resembling keratosis lichenoides chronica. Ultimately, after discovering the patient had prolonged exposure to a space heater, the diagnosis of EAI was made. This case underscores the diagnostic challenges in pediatric EAI cases and emphasizes the importance of careful history taking as part of a comprehensive evaluation.
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Purpose: This study aimed to describe healthcare resource utilization and costs among individuals with vitiligo who were diagnosed with ≥1 psychosocial comorbidity, using data from US claims databases. Patients and Methods: A retrospective, observational cohort analysis of the IBM MarketScan Commercial and Medicare supplemental claims databases for US individuals with vitiligo aged ≥12 years and a first vitiligo claim between January 1 and December 31, 2018, was undertaken to assess psychosocial burden, including mental and behavioral health comorbidities. Results: Of the 12,427 individuals included in the analysis, nearly 1 in 4 (23.5%) who had vitiligo were also diagnosed with ≥1 psychosocial comorbidity. A greater percentage of these individuals versus those who were not diagnosed with a psychosocial comorbidity had a vitiligo-related prescription claim (50.2% vs 45.4%; P<0.0001), especially for oral corticosteroids (25.4% vs 16.6%; P<0.0001) and low-potency topical corticosteroids (9.0% vs 7.6%; P<0.05). Total vitiligo-related healthcare resource utilization and costs were consistent among individuals with and without psychosocial comorbidity despite significantly (P<0.05) higher vitiligo-related ER visit utilization and expenditure among those with psychosocial comorbidity. Furthermore, individuals diagnosed with vitiligo and ≥1 psychosocial comorbidity had significantly (P<0.0001) greater utilization of all-cause mean prescription claims (25.0 vs 12.8), outpatient services (other than physician and ER visits: 19.5 vs 11.3), outpatient physician visits (10.1 vs 6.4), inpatient stays (0.6 vs 0.1), and ER visits (0.4 vs 0.2) and incurred significantly higher mean (SD) direct medical expenditures ($18,804 [$46,621] vs $9833 [$29,094] per patient per year; P<0.0001). Conclusion: Individuals with vitiligo who were diagnosed with ≥1 psychosocial comorbidity incurred greater total all-cause but not vitiligo-related healthcare resource utilization and expenditures than those without diagnosis of psychosocial comorbidities. Identification of psychosocial comorbidities in individuals with vitiligo may be important for multidisciplinary management of vitiligo to reduce overall burden for individuals with vitiligo.
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[This corrects the article DOI: 10.3389/fneur.2023.1270767.].
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Background: The incidence of microorganisms with extended-spectrum beta-lactamase (ESBL) is on the rise, posing a significant public health concern. The current application of machine learning (ML) focuses on predicting bacterial resistance to optimize antibiotic therapy. This study employs ML to forecast the occurrence of bacteria that generate ESBL and demonstrate resistance to multiple antibiotics (MDR). Methods: Six popular ML algorithms were initially trained on antibiotic resistance test patient reports (n = 489) collected from Al-Hussein/Salt Hospital in Jordan. Trained outcome models predict ESBL and multidrug resistance profiles based on microbiological and patients' clinical data. The results were utilized to select the optimal ML method to predict ESBL's most associated features. Results: Escherichia coli (E. coli, 82%) was the most commonly identified microbe generating ESBL, displaying multidrug resistance. Urinary tract infections (UTIs) constituted the most frequently observed clinical diagnosis (68.7%). Classification and Regression Trees (CART) and Random Forest (RF) classifiers emerged as the most effective algorithms. The relevant features associated with the emergence of ESBL include age and different classes of antibiotics, including cefuroxime, ceftazidime, cefepime, trimethoprim/ sulfamethoxazole, ciprofloxacin, and gentamicin. Fosfomycin nitrofurantoin, piperacillin/tazobactam, along with amikacin, meropenem, and imipenem, had a pronounced inverse relationship with the ESBL class. Conclusion: CART and RF-based ML algorithms can be employed to predict the most important features of ESBL. The significance of monitoring trends in ESBL infections is emphasized to facilitate the administration of appropriate antibiotic therapy.
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In this study, TiO2 supported over embryonic Beta zeolite (BEA) was prepared for the photocatalytic degradation of Tetracycline (TC) antibiotic under visible light. The immobilization of sol-gel TiO2 over the zeolite increased its surface area from 33 (m2/g) to 226 (m2/g) and enhanced its adsorption efficiency from 8 % to 18 %. In order to expand the photocatalytic activity of TiO2 towards the visible light region (i.e. λ > 380 nm), two different metal sensitization techniques with Iron ions from aqueous solution of FeCl3 were explored. In the ion-exchange method, the substitutional cations within the TiO2/BEA structure were exchanged with Fe3+. Whereas, in the doping technique, solgel TiO2 was doped with Fe3+ during its synthesis and before its immobilization over Zeolite. Four different samples with 20, 40, 60, and 100 % w/w of TiO2/BEA ratio were prepared. After testing the various ion-exchanged photocatalysts under blue and white lights, only Fe-60%TiO2/BEA showed better activity compared to pure TiO2 under white light at TC initial concentration, C o = 20 ppm. For the doped immobilized Titania with 60 wt% TiO2/BEA, three different doped photocatalysts were prepared with 3 %, 7 %, and 10 % per mole Fe/TiO2. All the Fe-doped TiO2/BEA photocatalysts showed better activity compared to pure TiO2 under white light. Under solar irradiations, the 3 % Fe-doped TiO2/BEA was able to degrade all TC within 120 min, while Fe-60%TiO2/BEA needed 200 min, and TiO2 needed more than 300 min. This enhanced performance was a result of both increased surface area due to immobilization over BEA as well as iron doping by Fe3+ that simultaneously increased the visible light absorption of TiO2 and minimized the charge carrier recombination effect.
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While multiple sclerosis (MS) commonly manifests with optic nerve involvement, it can also masquerade as diverse cranial nerve (CN) palsies. We present the case of a young male initially diagnosed with Bell's palsy based on unilateral facial nerve paralysis. Despite the presence of typical clinical features, the patient's evaluation took an unexpected turn. Subsequent brain MRI revealed demyelinating lesions, ultimately confirming the diagnosis of MS. This case underscores the importance of maintaining vigilance in diagnosing atypical presentations of MS, illustrating how meticulous evaluation and neuroimaging play pivotal roles in uncovering underlying pathologies when conventional diagnoses such as Bell's palsy raise uncertainties.
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Introduction: The effects of COVID-19 lockdowns and the discontinued face-to-face clinical practicum had negative consequences on nursing and midwifery students at many levels. The clinical learning environment includes all the training and learning experiences that nursing students undergo during their clinical practicum. Objectives: This study aimed to assess the effects of the COVID-19 lockdowns on the nursing and midwifery students' practicum training in governmental and private universities and academic faculties of nursing in Jordan. Methods: A descriptive cross-sectional study of 1025 nursing and midwifery students from academic faculties of nursing of both governmental and private Jordanian universities was conducted in September 2021. The research group from two Jordanian universities designed and validated a 13-item survey to determine and evaluate the impact of mass lockdowns on nursing and midwifery students' practicum training. The responses were assessed using descriptive and inferential analyses. Results: The findings revealed that the lockdown had a negative impact on nursing and midwifery students' self-confidence and competency in performing nursing procedures. The academic year of the students was a significant independent predictor of their self-confidence and competency levels in performing nursing procedures. Conclusion: The study concluded that nursing and midwifery students were dissatisfied with their clinical education during the COVID-19 pandemic, which led to poor self-confidence in performing nursing procedures. The study group recommended repeating the clinical practicum, incorporating extensive laboratory and hospital courses, and implementing a one-year internship for newly graduated nurses and midwives to address the training gap and enhance self-confidence in clinical procedures.
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With the rapid growth of information and communication technologies, governments worldwide are embracing digital transformation to enhance service delivery and governance practices. In the rapidly evolving landscape of information technology (IT), secure data management stands as a cornerstone for organizations aiming to safeguard sensitive information. Robust data modeling techniques are pivotal in structuring and organizing data, ensuring its integrity, and facilitating efficient retrieval and analysis. As the world increasingly emphasizes sustainability, integrating eco-friendly practices into data management processes becomes imperative. This study focuses on the specific context of Pakistan and investigates the potential of cloud computing in advancing e-governance capabilities. Cloud computing offers scalability, cost efficiency, and enhanced data security, making it an ideal technology for digital transformation. Through an extensive literature review, analysis of case studies, and interviews with stakeholders, this research explores the current state of e-governance in Pakistan, identifies the challenges faced, and proposes a framework for leveraging cloud computing to overcome these challenges. The findings reveal that cloud computing can significantly enhance the accessibility, scalability, and cost-effectiveness of e-governance services, thereby improving citizen engagement and satisfaction. This study provides valuable insights for policymakers, government agencies, and researchers interested in the digital transformation of e-governance in Pakistan and offers a roadmap for leveraging cloud computing technologies in similar contexts. The findings contribute to the growing body of knowledge on e-governance and cloud computing, supporting the advancement of digital governance practices globally. This research identifies monitoring parameters necessary to establish a sustainable e-governance system incorporating big data and cloud computing. The proposed framework, Monitoring and Assessment System using Cloud (MASC), is validated through secondary data analysis and successfully fulfills the research objectives. By leveraging big data and cloud computing, governments can revolutionize their digital governance practices, driving transformative changes and enhancing efficiency and effectiveness in public administration.
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Long COVID-19, also known as post-acute sequelae of SARS-CoV-2 infection (PASC), is characterized by persistent symptoms after COVID-19 onset. This article explores the challenges, management strategies, and recommendations for addressing long COVID-19 in primary care settings. The epidemiology of long COVID-19 reveals significant variability, with a substantial portion of COVID-19 survivors experiencing post-acute symptoms. Pathophysiological mechanisms include viral persistence, endothelial dysfunction, autoimmunity, neurological dysregulation, and gastrointestinal dysbiosis. Multiple risk factors, including age, sex, pre-existing comorbidities, smoking, BMI, and acute COVID-19 severity, influence the development of long COVID-19. Effective management requires proactive measures such as vaccination, identification of high-risk populations, public awareness, and post-infection vaccination. Collaboration of primary care physicians with specialists is essential for holistic and individualized patient care. This article underscores the role of primary care physicians in diagnosing, managing, and mitigating the long-term effects of COVID-19. (AU)
La COVID-19 prolongada, también conocida como secuela postaguda de la infección por SARS-CoV-2, se caracteriza por síntomas persistentes después de la aparición de la COVID-19. Este artículo explora los desafíos, las estrategias de manejo y las recomendaciones para abordar la COVID-19 prolongada en entornos de atención primaria. La epidemiología de la COVID-19 prolongada revela una variabilidad significativa, y una parte sustancial de los supervivientes de la COVID-19 experimentan síntomas postagudos. Los mecanismos fisiopatológicos incluyen persistencia viral, disfunción endotelial, autoinmunidad, desregulación neurológica y disbiosis gastrointestinal. Múltiples factores de riesgo, como la edad, el sexo, las comorbilidades preexistentes, el tabaquismo, el IMC y la gravedad aguda de la COVID-19, influyen en el desarrollo de la COVID-19 prolongada. Una gestión eficaz requiere medidas proactivas, como la vacunación, la identificación de poblaciones de alto riesgo, la concienciación pública y la vacunación posterior a la infección. La colaboración de los médicos de atención primaria con los especialistas es esencial para una atención holística e individualizada al paciente. Este artículo subraya el papel de los médicos de atención primaria en el diagnóstico, el tratamiento y la mitigación de los efectos a largo plazo de la COVID-19. (AU)
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Humanos , /diagnóstico , /epidemiología , Atención Primaria de Salud , AutoinmunidadRESUMEN
Background Plastic surgery has developed to benefit in a variety of challenging areas formerly handled by other disciplines. Medical students do not have a clear picture of plastic surgery as a career due to lacking scope, clinical practice, and understanding of plastic surgery as a clinical area of expertise, including general practitioners, nursing staff, medical trainees, and the general public, and misconceptions about the extent of reconstructive and plastic surgery. Methods A cross-sectional observational study was conducted on Kuwait University Medical students (2nd-7th Years) over a period of 1 month. A questionnaire and a consent form were provided to eligible students. The inclusion criteria were Kuwait University Medical students from 2nd to 7th Years with signed consent form. The response was collected via email sent in coordination with the Vice Dean of Student Affairs in the Faculty of Medicine. Using statistical package for the social sciences, responses were statistically analyzed. Pearson's chi-square test was used to calculate p -values, where p < 0.05 was considered statistically significant. Results A total of 244 eligible medical students, 121 males and 123 females, were included in the study, with a mean age of 21 (±2) years. Similarly, 126 (51.6%) were preclinical students (2nd-4th-year students), while 118 (48.4%) were clinical students (5th-7th-year students). About 79.8% of medical students believed that plastic surgery plays an essential role in trauma management, whereas 9.2% did not consider plastic surgery significant for trauma management. This study found that only 15.5% of medical students were interested in enrolling in plastic surgery residency after graduation, while 47.1% of students did not consider plastic surgery residency after graduation. However, 37.4% were uncertain. The two most driving factors in deciding on plastic surgery residency were expected income (61.8%) and lifestyle (14.3%). Conclusion Improving medical students' education quality can enhance their perception and awareness of plastic surgery. Students should be taught the broader scope of plastic surgery. The inclusion of formal training during undergraduation is the essence of time and should be added to or improved during plastic surgery rotations with more emphasis on reconstructive and hand/peripheral nerve surgery. Student-led interest groups can be a useful tool for educating students about their specialty.
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BACKGROUND: Little is known about the relationship between cytomegalovirus (CMV) infections and donor-derived cell-free DNA (dd-cfDNA) in heart transplant recipients. METHODS: In our study, CMV and dd-cfDNA results were prospectively collected on single-organ heart transplant recipients. If the CMV study was positive, a CMV study with dd-cfDNA was repeated 1-3 months later. The primary aim was to compare dd-cfDNA between patients with positive and negative CMV results. RESULTS: Of 44 patients enrolled between August 2022 and April 2023, 12 tested positive for CMV infections, 25 were included as controls, and seven patients with a viral infection without CMV were excluded. Baseline characteristics did not differ significantly between CMV-positive and CMV-negative patients with the exception of a later median time post-transplant in the CMV-positive group (253 days vs. 120 days, p = .03). Dd-cfDNA levels were significantly higher in patients with CMV infections compared to those without (p < .001) with more patients in the CMV positive group showing dd-cfDNA results ≥.12% (75% vs. 8%, p < .001) and ≥.20% (58% vs. 8%, p = .002). Each 1 log10 copy/ml reduction in CMV viral load from visit 1 to visit 2 was associated with a.23% reduction in log10 dd-cfDNA (p = .002). CONCLUSION: Our findings suggest that active CMV infections may raise dd-cfDNA levels in patients following heart transplantation. Larger studies are needed to validate these preliminary findings.
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Ácidos Nucleicos Libres de Células , Infecciones por Citomegalovirus , Trasplante de Corazón , Humanos , Citomegalovirus/genética , Donantes de Tejidos , Receptores de Trasplantes , Rechazo de InjertoRESUMEN
Importance: Current left bundle branch block (LBBB) criteria are based on animal experiments or mathematical models of cardiac tissue conduction and may misclassify patients. Improved criteria would impact referral decisions and device type for cardiac resynchronization therapy. Objective: To develop a simple new criterion for LBBB based on electrophysiological studies of human patients, and then to validate this criterion in an independent population. Design, Setting, and Participants: In this diagnostic study, the derivation cohort was from a single-center, prospective study of patients undergoing electrophysiological study from March 2016 through November 2019. The validation cohort was assembled by retrospectively reviewing medical records for patients from the same center who underwent transcatheter aortic valve replacement (TAVR) from October 2015 through May 2022. Exposures: Patients were classified as having LBBB or intraventricular conduction delay (IVCD) as assessed by intracardiac recording. Main Outcomes and Measures: Sensitivity and specificity of the electrocardiography (ECG) criteria assessed in patients with LBBB or IVCD. Results: A total of 75 patients (median [IQR] age, 63 [53-70.5] years; 21 [28.0%] female) with baseline LBBB on 12-lead ECG underwent intracardiac recording of the left ventricular septum: 48 demonstrated complete conduction block (CCB) and 27 demonstrated intact Purkinje activation (IPA). Analysis of surface ECGs revealed that late notches in the QRS complexes of lateral leads were associated with CCB (40 of 48 patients [83.3%] with CCB vs 13 of 27 patients [48.1%] with IPA had a notch or slur in lead I; P = .003). Receiver operating characteristic curves for all septal and lateral leads were constructed, and lead I displayed the best performance with a time to notch longer than 75 milliseconds. Used in conjunction with the criteria for LBBB from the American College of Cardiology/American Heart Association/Heart Rhythm Society, this criterion had a sensitivity of 71% (95% CI, 56%-83%) and specificity of 74% (95% CI, 54%-89%) in the derivation population, contrasting with a sensitivity of 96% (95% CI, 86%-99%) and specificity of 33% (95% CI, 17%-54%) for the Strauss criteria. In an independent validation cohort of 46 patients (median [IQR] age, 78.5 [70-84] years; 21 [45.7%] female) undergoing TAVR with interval development of new LBBB, the time-to-notch criterion demonstrated a sensitivity of 87% (95% CI, 74%-95%). In the subset of 10 patients with preprocedural IVCD, the criterion correctly distinguished IVCD from LBBB in all cases. Application of the Strauss criteria performed similarly in the validation cohort. Conclusions and Relevance: The findings suggest that time to notch longer than 75 milliseconds in lead I is a simple ECG criterion that, when used in conjunction with standard LBBB criteria, may improve specificity for identifying patients with LBBB from conduction block. This may help inform patient selection for cardiac resynchronization or conduction system pacing.
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Bloqueo de Rama , Electrocardiografía , Humanos , Bloqueo de Rama/fisiopatología , Bloqueo de Rama/diagnóstico , Bloqueo de Rama/terapia , Femenino , Masculino , Anciano , Persona de Mediana Edad , Estudios Prospectivos , Estudios RetrospectivosRESUMEN
A male patient was evaluated by the dermatology inpatient consult service after a 5-week history of a skin lesion on the right anterior thigh with intermittent itching and mild tenderness to palpation. What is your diagnosis?
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Inmunocompetencia , Humanos , Biopsia , Eritema/diagnóstico , Eritema/patologíaRESUMEN
This paper presents an eco-design approach to the synthesis of a highly efficient Cr(VI) adsorbent, utilizing a positively charged surface mesoporous FDU-12 material (designated as MI-Cl-FDU-12) for the first time. The MI-Cl-FDU-12 anion-exchange adsorbent was synthesized via a facile one-pot synthesis approach using sodium silicate extracted from sorghum waste as a green silica source, 1-methyl-3-(triethoxysilylpropyl) imidazolium chloride as a functionalization agent, triblock copolymer F127 as a templating or pore-directing agent, trimethyl benzene as a swelling agent, KCl as an additive, and water as a solvent. The synthesis method offers a sustainable and environmentally friendly approach to the production of a so-called "green" adsorbent with a bimodal micro-/mesoporous structure and a high surface area comparable with the previous reports regarding FDU-12 synthesis. MI-Cl-FDU-12 was applied as an anion exchanger for the adsorption of toxic Cr(VI) oxyanions from aqueous media and various kinetic and isotherm models were fitted to experimental data to propose the adsorption behavior of Cr(VI) on the adsorbent. Langmuir model revealed the best fit to the experimental data at four different temperatures, indicating a homogeneous surface site affinity. The theoretical maximum adsorption capacities of the adsorbent were found to be 363.5, 385.5, 409.0, and 416.9 mg g-1 at 298, 303, 308, and 313 K, respectively; at optimal conditions (pH=2, adsorbent dose=3.0 mg, and contact time of 30 min), surpassing that of most previously reported Cr(VI) adsorbents in the literature. A regeneration study revealed that this adsorbent possesses outstanding performance even after six consecutive recycling.
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Long COVID-19, also known as post-acute sequelae of SARS-CoV-2 infection (PASC), is characterized by persistent symptoms after COVID-19 onset. This article explores the challenges, management strategies, and recommendations for addressing long COVID-19 in primary care settings. The epidemiology of long COVID-19 reveals significant variability, with a substantial portion of COVID-19 survivors experiencing post-acute symptoms. Pathophysiological mechanisms include viral persistence, endothelial dysfunction, autoimmunity, neurological dysregulation, and gastrointestinal dysbiosis. Multiple risk factors, including age, sex, pre-existing comorbidities, smoking, BMI, and acute COVID-19 severity, influence the development of long COVID-19. Effective management requires proactive measures such as vaccination, identification of high-risk populations, public awareness, and post-infection vaccination. Collaboration of primary care physicians with specialists is essential for holistic and individualized patient care. This article underscores the role of primary care physicians in diagnosing, managing, and mitigating the long-term effects of COVID-19.
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COVID-19 , Humanos , COVID-19/terapia , Síndrome Post Agudo de COVID-19 , SARS-CoV-2 , Enfermedad Crónica , Atención Primaria de Salud , Progresión de la EnfermedadRESUMEN
BACKGROUND: Immunotherapies have changed the landscape of late-stage melanoma; however, data evaluating timely access to immunotherapy are lacking. METHODS: A retrospective cohort study utilizing the National Cancer Database was conducted. Stage III and IV melanoma cases diagnosed between 2011 and 2018 that received systemic treatment with either immunotherapy or chemotherapy were included. Chemotherapy included BRAF/MEK inhibitors. Multivariable logistic regression models were utilized to evaluate factors associated with the likelihood of receiving immunotherapy as primary systemic treatment relative to chemotherapy; additionally, Cox proportional hazards models were utilized to incorporate time from diagnosis to primary systemic therapy into the analysis. RESULTS: The study population was comprised of 14,446 cases. The cohort included 12,053 (83.4%) immunotherapy and 2393 (16.6%) chemotherapy cases. In multivariable logistic regression analysis, factors significantly associated with immunotherapy receipt included population density, circle distance, year of diagnosis, Breslow thickness, and cancer stage. Immunotherapy timing was evaluated using multivariable Cox regression analysis. Minorities were less likely to receive timely immunotherapy than non-Hispanic Whites (HR 0.83, CI 0.74-0.93, p = 0.001). Patients at circle distances of 10-49 miles (HR 0.94, CI 0.89-0.99, p = 0.02) and ≥50 miles (HR 0.83, CI 0.77-0.90, p < 0.001) were less likely to receive timely immunotherapy. CONCLUSION: Patients traveling ≥10 miles and minorities have a decreased likelihood of receiving timely immunotherapy administration for primary systemic treatment. Future research is needed to identify what barriers and approaches can be leveraged to address these inequities.
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Inmunoterapia , Melanoma , Estadificación de Neoplasias , Tiempo de Tratamiento , Humanos , Melanoma/tratamiento farmacológico , Melanoma/terapia , Melanoma/patología , Melanoma/inmunología , Femenino , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Anciano , Tiempo de Tratamiento/estadística & datos numéricos , Inmunoterapia/métodos , Neoplasias Cutáneas/terapia , Neoplasias Cutáneas/patología , Neoplasias Cutáneas/tratamiento farmacológico , Neoplasias Cutáneas/inmunología , Adulto , Estados UnidosRESUMEN
Length of stay (LoS) prediction is deemed important for a medical institution's operational and logistical efficiency. Sound estimates of a patient's stay increase clinical preparedness and reduce aberrations. Various statistical methods and techniques are used to quantify and predict the LoS of a patient based on pre-operative clinical features. This study evaluates and compares the results of Bayesian (simple Bayesian regression and hierarchical Bayesian regression) models and machine learning (ML) regression models against multiple evaluation metrics for the problem of LoS prediction of cardiac patients admitted to Tabba Heart Institute, Karachi, Pakistan (THI) between 2015 and 2020. In addition, the study also presents the use of hierarchical Bayesian regression to account for data variability and skewness without homogenizing the data (by removing outliers). LoS estimates from the hierarchical Bayesian regression model resulted in a root mean squared error (RMSE) and mean absolute error (MAE) of 1.49 and 1.16, respectively. Simple Bayesian regression (without hierarchy) achieved an RMSE and MAE of 3.36 and 2.05, respectively. The average RMSE and MAE of ML models remained at 3.36 and 1.98, respectively.
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BACKGROUND: Patients with partial DiGeorge syndrome (pDGS) can present with immune dysregulation, the most common being autoimmune cytopenia (AIC). There is a lack of consensus on the approach to type, combination, and timing of therapies for AIC in pDGS. Recognition of immune dysregulation early in pDGS clinical course may help individualize treatment and prevent adverse outcomes from chronic immune dysregulation. OBJECTIVES: Objectives of this study were to characterize the natural history, immune phenotype, and biomarkers in pDGS with AIC. METHODS: Data on clinical presentation, disease severity, immunological phenotype, treatment selection, and response for patients with pDGS with AIC were collected via retrospective chart review. Flow cytometric analysis was done to assess T and B cell subsets, including biomarkers of immune dysregulation. RESULTS: Twenty-nine patients with the diagnosis of pDGS and AIC were identified from 5 international institutions. Nineteen (62%) patients developed Evan's syndrome (ES) during their clinical course and twenty (69%) had antibody deficiency syndrome. These patients demonstrated expansion in T follicular helper cells, CD19hiCD21lo B cells, and double negative cells and reduction in CD4 naïve T cells and regulatory T cells. First-line treatment for 17/29 (59%) included corticosteroids and/or high-dose immunoglobulin replacement therapy. Other overlapping therapies included eltrombopag, rituximab, and T cell immunomodulators. CONCLUSIONS: AIC in pDGS is often refractory to conventional AIC treatment paradigms. Biomarkers may have utility for correlation with disease state and potentially even response to therapy. Immunomodulating therapies could be initiated early based on early immune phenotyping and biomarkers before the disease develops or significantly worsens.
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Citopenia , Síndrome de DiGeorge , Humanos , Síndrome de DiGeorge/diagnóstico , Síndrome de DiGeorge/terapia , Estudios Retrospectivos , Antígenos CD19 , Progresión de la EnfermedadRESUMEN
The respiratory variations in mitral valve (MV) Doppler flow in hemodialysis (HD) patients have not been investigated and the normal echocardiographic value is used as a reference for HD patients. The present study evaluated the respiratory variation in MV Doppler flow in HD patients to determine if it has a unique pattern. In this prospective cohort study, echocardiography was performed before and 6 h after dialysis. The transmitral spectral Doppler E wave was measured during inspiratory and expiratory phases. The percent changes in the E wave were calculated pre- and post-dialysis. The means of the percent variation in the MV inspiratory and expiratory E wave pre- and post-dialysis were 56 ± 7% and 44 ± 1.1%, respectively, with a significant reduction after dialysis (P = .000). There was a significant positive correlation between post-dialysis ∆E wave % change and post-dialysis % change in weight (r = .318; P = .000). The respiratory changes in the MV E wave in HD patients were higher than the normal reference values. This marked variation could be explained by fluid overloading in HD patients.