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1.
Pediatr Transplant ; 28(2): e14707, 2024 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-38419558

RESUMEN

Epstein-Barr Virus (EBV) is a ubiquitous herpes type virus that is associated with post-transplant lymphoproliferative disorder (PTLD). Usual management includes reduction or cessation of immunosuppression and in some cases chemotherapy including rituximab. However, limited therapies are available if PTLD is refractory to rituximab. Several clinical trials have investigated the use of EBV-directed T cells in rituximab-refractory patients; however, data regarding response is scarce and inconclusive. Herein, we describe a patient with EBV-PTLD refractory to rituximab after orthotopic heart transplantation (OHT) requiring EBV-directed T-cell therapy. This article aims to highlight the unique and aggressive clinical presentation and progression of PTLD with utilization of EBV-directed T-cell therapy for management and associated pitfalls.


Asunto(s)
Infecciones por Virus de Epstein-Barr , Trasplante de Corazón , Trasplante de Células Madre Hematopoyéticas , Trastornos Linfoproliferativos , Humanos , Preescolar , Herpesvirus Humano 4 , Rituximab/uso terapéutico , Infecciones por Virus de Epstein-Barr/terapia , Infecciones por Virus de Epstein-Barr/tratamiento farmacológico , Trastornos Linfoproliferativos/diagnóstico , Trastornos Linfoproliferativos/etiología , Trastornos Linfoproliferativos/terapia , Tratamiento Basado en Trasplante de Células y Tejidos
2.
Med Teach ; : 1-4, 2024 Jan 25.
Artículo en Inglés | MEDLINE | ID: mdl-38270187

RESUMEN

BACKGROUND AND OBJECTIVES: The rational for the Somaliland national harmonised curriculum (NHC) was driven by shared concern about the quality of medical education. PROCESS: The Ministry of Education and Science and the Ministry of Health Development produced a Medical Education Policy 2018. Policy objectives included the development of the NHC and accreditation for medical schools that met the standards of the World Federation for Medical Education (WFME). Two bodies were asked to oversee these aims: the National Health Professions' Commission (NHPC) and the National Commission of Higher Education (NCHE). Between 2018 and 2020, a collaborative approach between the Somaliland government, medical school stakeholders, King's Global Health Partnership's (KGHP) volunteers and the Tropical Health Education Trust (THET) team was used to design the 6-years NHC. The NHC structure, content and delivery were grounded by WFME standards, health needs of the local population, student focused and active learning methods, and feasibility of implementation in medical schools. OUTCOMES: The NHC comprises details about the educational outcomes, curriculum model and framework, educational principles, instructional and learning methods, core as well as optional content, and assessment strategy. CONCLUSIONS: The approach used to develop the NHC ensured it is bespoken for Somaliland. Ongoing evaluation of patient and population needs, each medical school's review of programme implementation and outcomes will inform continuous revision and renewal.

3.
Emerg Radiol ; 31(2): 187-192, 2024 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-38340256

RESUMEN

BACKGROUND AND PURPOSE: Suppurative retropharyngeal lymphadenitis is a retropharyngeal space infection almost exclusively seen in the young (4-8 years old) pediatric population. It can be misdiagnosed as a retropharyngeal abscess, leading to unnecessary invasive treatment procedures. This retrospective study aims to assess radiology residents' ability to independently identify CT imaging findings and make a definitive diagnosis of suppurative retropharyngeal lymphadenitis in a simulated call environment. MATERIALS AND METHODS: The Wisdom in Diagnostic Imaging Emergent/Critical Care Radiology Simulation (WIDI SIM) is a computer-aided emergency imaging simulation proven to be a reliable method for assessing resident preparedness for independent radiology call. The simulation included 65 cases across various imaging modalities of varying complexity, including normal studies, with one case specifically targeting suppurative retropharyngeal adenitis identification. Residents' free text responses were manually scored by faculty members using a standardized grading rubric, with errors subsequently classified by type. RESULTS: A total of 543 radiology residents were tested in three separate years on the imaging findings of suppurative retropharyngeal lymphadenitis using the Wisdom in Diagnostic Imaging simulation web-based testing platform. Suppurative retropharyngeal lymphadenitis was consistently underdiagnosed by radiology residents being tested for call readiness irrespective of the numbers of years in training. On average, only 3.5% of radiology residents were able to correctly identify suppurative retropharyngeal lymphadenitis on a contrast-enhanced computed tomography (CT). CONCLUSIONS: Our findings underscore a potential gap in radiology residency training related to the accurate identification of suppurative retropharyngeal lymphadenitis, highlighting the potential need for enhanced educational efforts in this area.


Asunto(s)
Internado y Residencia , Linfadenitis , Radiología , Humanos , Niño , Preescolar , Estudios Retrospectivos , Radiología/educación , Competencia Profesional , Linfadenitis/diagnóstico por imagen
4.
J ECT ; 38(2): 141-143, 2022 06 01.
Artículo en Inglés | MEDLINE | ID: mdl-35220357

RESUMEN

ABSTRACT: A 72-year-old man suffering with severe depression with psychotic symptoms, marked apathy, and psychomotor retardation was treated with electroconvulsive therapy (ECT) after resistance to treatment with psychotropic medications. His age, comorbidities, and dysexecutive syndrome prompted consideration of a diagnosis of frontotemporal dementia (FTD), and a 99mTechnetium-labeled hexamethyl propylene amine oxime single-photon emission computed tomography (SPECT) brain perfusion scan showed bilateral perfusion defects that were most pronounced in the frontal regions of the brain. The scan was judged to be abnormal and in keeping with a neurodegenerative dementia. We reasoned that the fluctuation in symptoms was inconsistent with a diagnosis of FTD and that his severe depression could be improved with ECT even if he had FTD, so we decided to proceed with this treatment. A course of 12 sessions of ECT resulted in remission of his psychiatric symptoms and improvement in cognitive performance. A repeat SPECT scan 5 weeks after the last ECT demonstrated a substantial improvement in cerebral blood flow, favoring the diagnosis of depression, rather than dementia. Similar case reports from the literature suggest that ECT does reverse brain hypoperfusion in severe cases of depression and catatonia. Clinicians should be aware that abnormal SPECT findings are nonspecific and can be caused by various conditions, including psychiatric illness, and are not necessarily diagnostic of a neurodegenerative disease.


Asunto(s)
Catatonia , Trastorno Depresivo Mayor , Terapia Electroconvulsiva , Demencia Frontotemporal , Enfermedades Neurodegenerativas , Anciano , Catatonia/complicaciones , Catatonia/terapia , Depresión/complicaciones , Depresión/terapia , Humanos , Masculino
5.
J Stroke Cerebrovasc Dis ; 29(12): 105294, 2020 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-32992195

RESUMEN

Hyperintense reperfusion marker (HARM) on post-contrast magnetic resonance imaging (MRI) fluid attenuated inversion recovery (FLAIR) represents gadolinium contrast extravasation in the setting of acute ischemic stroke and is a common finding after revascularization therapies. Clinically, it is a marker of blood brain barrier (BBB) disruption, predictor of hemorrhagic transformation, and predictor of poor clinical outcome in ischemic stroke. Here, we describe a case where a patient underwent mechanical thrombectomy and was later found to have evidence of contrast extravasation on CT imaging, in the same locations found on the post-contrast FLAIR MRI, demonstrating that MRI-HARM and CT contrast extravasation may mimic similar phenomena. Thus, this case demonstrates that we may be able to extrapolate what we know about HARM detected on MRI to a CT imaging biomarker that would be more readily obtainable in most stroke patients.


Asunto(s)
Medios de Contraste/administración & dosificación , Extravasación de Materiales Terapéuticos y Diagnósticos/diagnóstico por imagen , Infarto de la Arteria Cerebral Media/terapia , Trombectomía/efectos adversos , Tomografía Computarizada por Rayos X , Anciano , Circulación Cerebrovascular , Extravasación de Materiales Terapéuticos y Diagnósticos/etiología , Femenino , Humanos , Infarto de la Arteria Cerebral Media/diagnóstico por imagen , Infarto de la Arteria Cerebral Media/fisiopatología , Imagen por Resonancia Magnética , Valor Predictivo de las Pruebas , Resultado del Tratamiento
6.
BJU Int ; 120(5B): E59-E63, 2017 11.
Artículo en Inglés | MEDLINE | ID: mdl-28317247

RESUMEN

OBJECTIVE: To establish the pattern of isotope bone scan (BS) positivity in a large contemporaneous cohort of patients with newly diagnosed localised prostate cancer and compare with the European Association of Urology (EAU) guidelines, as imaging guidelines and clinical practice for using BS to stage newly diagnosed patients with intermediate-risk localised prostate cancer are not uniform in the literature. PATIENTS AND METHODS: All patients with newly diagnosed prostate cancer were discussed in a specialist multidisciplinary team meeting and were prospectively entered in a database. Patients were categorised based on D'Amico classification. All intermediate- and high-risk patients had pelvic magnetic resonance imaging and BS unless contraindicated. The BS positivity in each group was analysed and the negative predictive value (NPV) calculated. A cohort of 2720 patients between 2002 and 2015 was retrospectively analysed. RESULTS: Of 976 patients in the D'Amico intermediate-risk category, 99 had primary Gleason pattern 4. Only one of the 99 patients had a positive BS and there were no positive BS in patients with Gleason primary pattern 3 in the intermediate-risk category. On subgroup analysis, based on prostate-specific antigen (PSA) level and Gleason grade alone, the BS-positivity rate in patients with a PSA level of <20 ng/mL and Gleason primary pattern 4 vs 3 was 6% and 0%, respectively, resulting in 100% NPV for a positive BS with Gleason primary pattern 3 and a PSA level of <20 ng/mL. The importance of clinical T stage (cT) was also noted, as eight of 146 patients had positive BS, who were high risk on cT stage, with a PSA level of <20 ng/mL and Gleason score <8. All eight patients had Gleason primary pattern 4. By limiting BS to the population at risk (all high-risk + intermediate-risk with primary pattern 4), 68 BS per year could have been avoided in a single centre. A limitation was that there was no histological confirmation of bony metastases. Extending the BS recommendation considering the new Gleason Grade Grouping is discussed. CONCLUSION: This study confirms that a staging BS can be safely avoided in patients with intermediate-risk prostate cancer with Gleason primary pattern 3 and to limit performing BS in all high-risk prostate cancer and in the intermediate-risk group when the primary Gleason pattern is 4, thereby reinforcing the current recommendations of the EAU guidelines.


Asunto(s)
Neoplasias Óseas/diagnóstico por imagen , Neoplasias Óseas/secundario , Huesos/diagnóstico por imagen , Huesos/patología , Neoplasias de la Próstata/diagnóstico , Neoplasias de la Próstata/patología , Cintigrafía , Biomarcadores de Tumor , Estudios de Cohortes , Europa (Continente) , Adhesión a Directriz/estadística & datos numéricos , Humanos , Masculino , Clasificación del Tumor , Guías de Práctica Clínica como Asunto , Valor Predictivo de las Pruebas , Antígeno Prostático Específico/sangre , Neoplasias de la Próstata/sangre , Neoplasias de la Próstata/diagnóstico por imagen , Reino Unido
8.
Magn Reson Imaging Clin N Am ; 32(2): 253-275, 2024 May.
Artículo en Inglés | MEDLINE | ID: mdl-38555140

RESUMEN

Toxic leukoencephalopathy and myelopathy are common neurologic complications of a wide range of chemotherapeutic and substance abuse agents. During the last decade, there has been a significant change in the profile of white matter toxins, primarily driven by the development and usage of new chemotherapeutic and immunotherapeutic agents and by the continuous increase in illicit drug abuse with contaminants. Neuroimaging in the form of MR imaging forms the cornerstone in the diagnosis of these entities, many of which are reversible and amenable to rapid correction. Chronic white matter changes are also seen with these toxins with gradually progressive clinicoradiological findings.


Asunto(s)
Leucoencefalopatías , Imagen por Resonancia Magnética , Humanos , Imagen por Resonancia Magnética/métodos , Encéfalo/diagnóstico por imagen , Leucoencefalopatías/inducido químicamente , Leucoencefalopatías/diagnóstico por imagen , Neuroimagen
9.
Artículo en Inglés | MEDLINE | ID: mdl-38609160

RESUMEN

CONTEXT: Patients with PCOS are at high risk of depression, anxiety, and metabolic syndrome (MetSyn), a key predictor of cardiovascular disease. The impact of depression and/or anxiety on MetSyn is unknown in this population. OBJECTIVE: To compare the risk of developing MetSyn in patients with PCOS with and without a history of depression and/or anxiety. DESIGN: Retrospective longitudinal cohort study (2008-2022) with median follow-up of 7 years. SETTING: Tertiary care ambulatory practice. PATIENTS OR OTHER PARTICIPANTS: Patients with hyperandrogenic PCOS and at least 2 evaluations for MetSyn ≥3 years apart (n=321). INTERVENTION(S): N/A. MAIN OUTCOME MEASURE(S): The primary outcome was risk of developing MetSyn. We hypothesized that this risk would be higher with a history of depression and/or anxiety. RESULTS: At the first visit, 33.0% had a history of depression and/or anxiety, with a third prescribed antidepressants or anxiolytics. Depression and/or anxiety increased risk of developing MetSyn during the study period (adjusted hazard ratio [aHR] 1.45, 95% CI 1.02-2.06, p=0.04) with an incidence of MetSyn of 75.3 compared to 47.6 cases per 100 person-years among those without (p=0.002). This was primarily driven by depression (aHR 1.56, 95% CI 1.10-2.20, p=0.01). CONCLUSIONS: Patients with PCOS and depression and/or anxiety have a high risk of developing MetSyn, with a stronger association between depression and MetSyn. Our findings highlight the urgent need for guideline-directed screening for depression and anxiety at time of diagnosis of PCOS as well as screening at subsequent visits to facilitate risk stratification for metabolic monitoring and early intervention in this high-risk group.

10.
Magn Reson Imaging Clin N Am ; 32(2): 233-251, 2024 May.
Artículo en Inglés | MEDLINE | ID: mdl-38555139

RESUMEN

For over two centuries, clinicians have been aware of various conditions affecting white matter which had come to be grouped under the umbrella term multiple sclerosis. Within the last 20 years, specific scientific advances have occurred leading to more accurate diagnosis and differentiation of several of these conditions including, neuromyelitis optica spectrum disorders and myelin oligodendrocyte glycoprotein antibody disease. This new understanding has been coupled with advances in disease-modifying therapies which must be accurately applied for maximum safety and efficacy.


Asunto(s)
Neuromielitis Óptica , Humanos , Neuromielitis Óptica/diagnóstico por imagen , Glicoproteína Mielina-Oligodendrócito/metabolismo , Acuaporina 4 , Imagen por Resonancia Magnética/métodos , Autoanticuerpos
11.
Medicine (Baltimore) ; 102(39): e35265, 2023 Sep 29.
Artículo en Inglés | MEDLINE | ID: mdl-37773871

RESUMEN

Progressive multifocal leukoencephalopathy (PML) is a central nervous system disease caused by the human polyomavirus 2 that usually occurs in a setting of immunodeficiency. PML without overt immunosuppression is considered a rare occurrence but has been described in multiple previous case reports and series. Its prevalence, overall frequency, and prognosis are largely unknown. This is a single-center retrospective review of all University of Florida cases with the ICD10 PML diagnosis code (A81.2). PML without overt immunosuppression was defined as absence of human immunodeficiency virus (HIV) infection, hematological malignancy, immunomodulatory/-suppressive medications, autoimmune conditions with a propensity for PML (sarcoidosis, systemic lupus erythematosus). Cases that did not fulfill criteria for clinically or histologically definite PML were excluded. Of 52 patients with the ICD10 code A 81.2, 17 fulfilled definite diagnostic criteria for PML. Overt immunosuppression was identified in 15/17 (88.2%) cases (10/17 (58.8%): human immunodeficiency virus; 5/17 (29.4%): immunomodulatory/-suppressive medication). Two/seventeen (11.8%) cases were consistent with PML without overt immunosuppression. Possible contributing factors were a preceding dog bite and mild hypogammaglobulinemia M (39 mg/dL) in case 1 and significant alcohol use without evidence for liver disease in case 2. Both cases were fatal within 6 (case 1) and 2 (case 2) months. The results suggest that PML without overt immunosuppression may be more common than previously described. Therefore, PML should be considered even in the absence of overt immunosuppression if clinical and radiographic findings are suggestive of the diagnosis.


Asunto(s)
Enfermedades Autoinmunes , Infecciones por VIH , Leucoencefalopatía Multifocal Progresiva , Lupus Eritematoso Sistémico , Animales , Perros , Humanos , Enfermedades Autoinmunes/complicaciones , Infecciones por VIH/complicaciones , Tolerancia Inmunológica , Leucoencefalopatía Multifocal Progresiva/diagnóstico , Leucoencefalopatía Multifocal Progresiva/etiología , Lupus Eritematoso Sistémico/tratamiento farmacológico
12.
J Child Neurol ; 37(8-9): 727-737, 2022 08.
Artículo en Inglés | MEDLINE | ID: mdl-35673711

RESUMEN

BACKGROUND: The identification of immunoglobulin G antibodies against the aquaporin-4 channel (AQP-IgG) in the majority of adult patients differentiates neuromyelitis optica as a distinct disease entity. The high specificity of AQP4-IgG for neuromyelitis optica has allowed the identification of seropositive patients with atypical presentations of this disease. Neuromyelitis optica spectrum disorder has been increasingly recognized in children who demonstrate patterns of clinical involvement beyond the traditional boundaries of the optic pathways and spinal cord. METHODS: This is a single-center, retrospective review comparing demographic, clinical/paraclinical, and laboratory features of children and adults with a serologically confirmed diagnosis of AQP4-IgG-positive neuromyelitis optica spectrum disorder. RESULTS: Of 151 reviewed patient charts, 12 pediatric-onset and 31 adult-onset patients had AQP4-IgG-positive neuromyelitis optica spectrum disorder. The mean age of pediatric-onset neuromyelitis optica spectrum disorder was 12 ± 3.58 years with a female predilection (3:1). Pediatric patients showed more frequent involvement of the brainstem (6/12 [50%]); P = .008) and diencephalon (3/12 [25%]; P = .018). A preceding infection was identifiable in only 3 of 12 (25%) pediatric-onset patients. Moreover, disability as calculated on the expanded disability status scale was less severe in pediatric-onset cases compared to adult-onset cases in their most recent assessment (0 [0-9]) vs 6.5 [0-10]; P = .005). Pediatric-onset patients were also more likely to respond to treatment of acute episodes with corticosteroids ± intravenous immunoglobulin and/or plasmapheresis (Clinical Global Impression-Change scale: 2.5 [1-4] vs 4 [1-6], P = .009). INTERPRETATION: This retrospective study was able to compare and contrast pediatric- and adult-onset neuromyelitis optica spectrum disorder. Relative to their adult counterparts, pediatric-onset neuromyelitis optica spectrum disorder patients were more likely to respond to treatment and less likely to be disabled from their disease at follow-up. Therefore, pediatric-onset disease may represent a less virulent form of neuromyelitis optica spectrum disorder.


Asunto(s)
Acuaporina 4 , Autoanticuerpos , Inmunoglobulina G , Neuromielitis Óptica , Adolescente , Adulto , Edad de Inicio , Acuaporina 4/inmunología , Autoanticuerpos/sangre , Niño , Femenino , Humanos , Inmunoglobulina G/sangre , Masculino , Neuromielitis Óptica/diagnóstico por imagen , Neuromielitis Óptica/inmunología , Estudios Retrospectivos , Pruebas Serológicas
13.
Surgery ; 172(5): 1563-1568, 2022 11.
Artículo en Inglés | MEDLINE | ID: mdl-35927077

RESUMEN

BACKGROUND: A major challenge in the study of high-impact, low-frequency procedures in trauma is the lack of accurate data for time-sensitive processes of care. Trauma video review offers a possible solution, allowing investigators to collect extremely granular time-stamped data. Using resuscitative thoracotomy as a model, we compared data collected using review of audiovisual recordings to data prospectively collected in real time with the hypothesis that data collected using video review would be subject to less missingness and bias. METHODS: We conducted a prospective cohort study of patients undergoing resuscitative thoracotomy at a single urban academic level 1 trauma center. Key data on the timing and completion of procedural milestones of resuscitative thoracotomy were collected using video review and prospective collection. We used McNemar's test to compare proportions of missing data between the 2 methods and calculated bias in time measurements for prospective collection with respect to video review. Statistical analyses were performed using Stata v. 15.0 (College Station, TX). RESULTS: We included 51 subjects (88% Black, 82% male, 90% injured by gunshot wounds) over the study period. Missingness in resuscitative thoracotomy procedural milestone time measurements ranged from 34% to 63% for prospective collection and 0 to 8% for video review and was less missing for video review for all key variables (P < .001). When not missing, bias in data collected by prospective collection was 10% to 43% compared with data collected by video review. CONCLUSIONS: The data collected using video review have less missingness and bias than prospective collection data collected by trained research assistants. Audiovisual recording should be the gold standard for data collection for the study of time-sensitive processes of care in resuscitation.


Asunto(s)
Toracotomía , Heridas por Arma de Fuego , Recolección de Datos , Femenino , Humanos , Masculino , Estudios Prospectivos , Resucitación/métodos , Centros Traumatológicos
14.
Clin Imaging ; 73: 139-145, 2021 May.
Artículo en Inglés | MEDLINE | ID: mdl-33406475

RESUMEN

BACKGROUND: Glioblastoma (GBM) is the most common primary brain malignancy, but much remains unknown about the histogenesis of these tumors. In the great majority of cases, GBM is a purely glial tumor but in rare cases the classic-appearing high-grade glioma component is admixed with regions of small round blue cells with neuronal immunophenotype, and these tumors have been defined in the WHO 2016 Classification as "glioblastoma with a primitive neuronal component." METHODS: In this paper, we present two cases of GBM-PNC with highly divergent clinical courses, and review current theories for the GBM cell-of-origin. RESULTS AND CONCLUSIONS: GBM-PNC likely arises from a cell type competent to give rise to glial or neuronal lineages. The thesis that GBM recapitulates to some extent normal neurodevelopmental cellular pathways is supported by molecular and clinical features of our two cases of GBM-PNC, but more work is needed to determine which cellular precursor gives rise to specific cases of GBM. GBM-PNC may have a dramatically altered clinical course compared to standard GBM and may benefit from specific lines of treatment.


Asunto(s)
Neoplasias Encefálicas , Glioblastoma , Glioma , Encéfalo , Neoplasias Encefálicas/diagnóstico por imagen , Glioblastoma/diagnóstico por imagen , Humanos
15.
Mult Scler Relat Disord ; 48: 102718, 2021 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-33388560

RESUMEN

BACKGROUND: The discovery of two immunoglobulin G (IgG) antibodies against aquaporin 4 (anti-AQP4) and myelin oligodendrocyte glycoprotein (anti-MOG) has led to the distinction of the disorders anti-AQP4 immunoglobulin G positive neuromyelitis spectrum disorder (AQP4-IgG+ NMOSD) and anti-MOG associated disorder (MOGAD). Different clinical and radiological features have been proposed to distinguish these two demyelinating CNS diseases. METHODS: This is a single-center retrospective review at the University of Florida (UF) including all patients with the diagnostic code ICD G36 ("other acute disseminated demyelination") from October 2015 to January 2020 (n=141) and all charts included in the clinical NMOSD database of the UF Division of Neuroimmunology (n=36). A total of 151 cases were reviewed for presence of anti-MOG and anti-AQP4 antibodies and NMOSD diagnostic criteria. Differences in MOGAD and AQP4-IgG+ NMOSD were compared. RESULTS: Of the 151 reviewed patient charts, 11 were consistent with MOGAD and 43 with AQP4-IgG+ NMOSD. Patients with MOGAD were significantly younger at symptom onset compared to patients with AQP4-IgG+ NMOSD (14 [1-33] years vs. 37 [6-82] years; p=0.005). In comparison with AQP4-IgG+ NMOSD, optic neuritis in MOGAD was more frequently associated with bilateral optic nerve involvement: (6/11 [54.5%] vs. 6/43 [13.9%]; p=0.009) and fundoscopic presence of optic disc edema (5/11 [45.5%] vs. 3/43 [7%]; p=0.006). Perineuritis was a common radiological feature in MOGAD (present in 4 cases). In case of myelitis, there was more frequent involvement of the conus medullaris in MOGAD (4/11 [36.4%] vs. 2/43 [4.7%]; p=0.012). Symptomatic cerebral syndrome with supratentorial white matter lesions was seen in MOGAD patients with pediatric onset (pediatric onset: 4/6 [66.7%] vs. adult onset: 0/5 [0%]. In MOGAD, evidence for combined central and peripheral demyelination and increased intracranial pressure was present in one patient each. A preceding inciting event (illness/postpartum) was more frequently identifiable in MOGAD (4/11 [36.4%] vs. 4/43 [7%]; p=0.045). Disability as calculated on the Expanded Disability Status Scale was less severe in MOGAD compared to AQP-IgG+ NMOSD (most severe presentation: 5 [2-7] vs. 7 [1-10]; p=0.015; most recent assessment: 2 [0-5] vs. 5 [0-10]; p=0.045) and patients were more likely to respond to treatment of acute attacks with corticosteroids and/or plasmapheresis (Clinical Global Impression-Global Change scale: 1 [1-4] vs. 3 [1-6]; p=0.001). INTERPRETATION: The study confirms that simultaneous bilateral optic neuritis, presence of optic disc edema, transverse myelitis with conus involvement and a less severe disease course are distinctive features of MOGAD.


Asunto(s)
Neuromielitis Óptica , Adulto , Acuaporina 4 , Autoanticuerpos , Niño , Femenino , Humanos , Inmunoglobulina G , Glicoproteína Mielina-Oligodendrócito , Neuromielitis Óptica/diagnóstico por imagen , Estudios Retrospectivos
16.
J Nanosci Nanotechnol ; 10(2): 1278-85, 2010 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-20352789

RESUMEN

The paper will discuss the effect of pulsing single or two unbalanced magnetrons in a closed magnetic field configuration on the structure and properties of tribological Cr-Al-N coatings. Nanocrystalline Cr-Al-N coatings were reactively deposited from Cr and Al elemental targets using two unbalanced magnetrons, which were powered in both dc, pulsing only Al target and asynchronously pulsing both Cr and Al targets at 100 kHz and 50% duty cycle conditions. The ion energy distributions of these deposition and pulsing conditions were characterized using a Hiden Electrostatic QuadruPole Plasma Analyzer. It was found that pulsing two magnetrons asynchronously at 100 kHz and 50% duty cycle produced higher ion energies and significant increased ion fluxes than pulsing none or pulsing only one (Al) target. The structure and properties of Cr-Al-N coatings synthesized under different dc and pulsing conditions were investigated using X-ray diffraction, scanning electron microscopy, nanoindentation and ball-on-disk wear test, and were correlated with the effects of ion energies and ion flux regimes observed in the plasma diagnostics. The advantages of using pulsed magnetron sputtering producing different energetic ion regimes to enhance the ion bombardment on the growing films and therefore achieving the improved density, refinement of grain size and properties are illustrated.

17.
J Acoust Soc Am ; 127(1): 309-15, 2010 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-20058978

RESUMEN

An approach for time delay estimation, based on phase difference detection, is presented. A multiple-frequency short continuous wave pulse is used to solve the well-known phase ambiguity problem when the maximum distance exceeds a full wavelength. Within an unambiguous range defined with the lowest frequency difference between components, the corresponding phase difference is unique and any distance within this range can be determined. Phase differences between higher frequency components are used to achieve a finer resolution. The concept will be presented and the effectiveness of the approach will be investigated through theoretical and practical examples. The method will be validated using underwater acoustic measurements, simulating noisy environments, demonstrating resolutions better than a 50th of a wavelength, even in the presence of high levels (-5 dB) of additive Gaussian noise. Furthermore, the algorithm is simple to use and can be easily implemented, being based on phase detection using the discrete Fourier transform.

18.
Pediatr Neurol ; 108: 117-120, 2020 07.
Artículo en Inglés | MEDLINE | ID: mdl-32248988

RESUMEN

BACKGROUND: In Pompe disease, glycogen deposition results in an augmentation of blood flow and abnormal remodeling, with resultant weakening of the arterial walls, which may result in pathologic dilatation of the cerebral arteries. This complication is rare in patients with late-onset Pompe disease, but it has not been well-described in infantile-onset Pompe disease. The effect of enzyme replacement therapy on this process is not known. METHODS: We examined clinical and imaging data on two patients who exhibit cerebrovascular arteriopathy: a 14-year-old boy with infantile-onset Pompe disease on enzyme replacement therapy and a 23-year-old woman with late-onset Pompe disease who was also receiving enzyme replacement therapy. RESULTS: Our 14-year-old patient exhibits cerebrovascular arteriopathy, primarily proximal and vertebrobasilar, while the 23-year-old patient has a more diffuse pattern. The 14-year-old patient is unique because cerebral dolichoectasias have not been described in infantile-onset Pompe disease. The 23-year-old patient is notable given the age and history of enzyme replacement therapy since age 15 years. CONCLUSIONS: Dilative cerebral arteriopathy in infantile-onset Pompe disease is novel and similarly atypical is the diffuse vascular dilation seen in our young patient with late-onset Pompe disease, both receiving enzyme replacement therapy. We should be cognizant of the risk of cerebrovascular disease in Pompe disease regardless of the disease variant and enzyme replacement therapy status.


Asunto(s)
Enfermedades Arteriales Cerebrales/etiología , Enfermedades Arteriales Cerebrales/patología , Enfermedad del Almacenamiento de Glucógeno Tipo II/complicaciones , Adolescente , Adulto , Edad de Inicio , Dilatación Patológica/etiología , Dilatación Patológica/patología , Terapia de Reemplazo Enzimático , Femenino , Enfermedad del Almacenamiento de Glucógeno Tipo II/tratamiento farmacológico , Humanos , Masculino , Adulto Joven
19.
Asia Ocean J Nucl Med Biol ; 8(1): 18-26, 2020.
Artículo en Inglés | MEDLINE | ID: mdl-32064279

RESUMEN

OBJECTIVES: Cardiovascular disease is a leading cause of morbimortality with over half cardiovascular events occurring in the asymptomatic population by traditional risk stratification. This preliminary study aimed to evaluate systemic plaque vulnerability in patients with prior Coronary Artery Disease (CAD) with a single Whole Body [FDG] PET-CT scan in terms of plaque inflammation and calcifications. METHODS: Twenty-two patients referred for oncological evaluation and with prior history of advanced CAD or age and gender matched controls without cardiovascular disease, underwent a Whole Body PET-CT scan 90 min after injection of 18F-FDG. A total of 975 transaxial PET images were retrospectively analysed to assess plaque inflammation using a standardized method of analysis with averaged Target-to-Background Ratios (TBRs) at different levels, in the thoracic and abdominal aorta, carotids, LAD, common iliac and femoral arteries, and were correlated with calcium scores from the CT images. RESULTS: TBRs from the thoracic aorta were higher in male patients than controls (1.49±0.11, p<0.05) and a gradient was observed (ascending > descending > aortic arch), and were also higher in the carotids in female patients (1.43±0.07) versus controls (p<0.05). A tendency for higher levels of plaque inflammation in the abdominal aorta was noted in all groups, but no significant FDG uptake was found either in the iliac or femoral arteries in any group. Plaque inflammation was also higher in the LAD in males but with large variations. Higher levels of calcifications were noted in the LAD, infra-renal abdominal aorta and common iliac arteries, but without significant correlation with plaque inflammation except sporadic overlapping. CONCLUSION: Patients with advanced CAD are at risk for vulnerable inflamed atheromas in other territories such as the thoracic aorta and carotid arteries, underpinning the systemic nature of the atherosclerotic disease. Coexistence with calcifications is rare, suggesting a different functional status of the plaques and different stages of the disease. Evaluation of subclinical systemic plaque vulnerability in CAD with a Whole Body [FDG] PET-CT scan is feasible and a potentially useful biomarker to assess subclinical vascular risk for risk stratification and treatment optimization, but further studies are needed.

20.
Acad Radiol ; 27(7): 1016-1024, 2020 07.
Artículo en Inglés | MEDLINE | ID: mdl-32402787

RESUMEN

RATIONALE AND OBJECTIVES: To further characterize empirical data from a full-resolution simulation of critical care imaging coupled with post hoc grading of resident's interpretations by senior radiologists. To present results from estimating the random effects terms in a comprehensive mixed (hierarchical) regression model. MATERIALS AND METHODS: After accounting for 9 fixed effects detailed in Part 1 of this paper, we estimated normally distributed random effects, expressed in terms of score offsets for each case, resident, program, and grader. RESULTS: The fixed effects alone explained 8.8% of score variation and adding the random effects increased explanatory power of the model to account for 36% of score variation. As quantified by intraclass correlation coefficient (ICC = 28.5%; CI: 25.1-31.6) the majority of score variation is directly attributable to the case at hand. This "case difficulty" measure has reliability of 95%. Individual residents accounted for much of the remaining score variation (ICC = 5.3%; CI: 4.6-5.9) after adjusting for all other effects including level of training. The reliability of this "resident competence" measure is 82%. Residency training program influence on scores was small (ICC = 1.1%; CI: 0.42-1.7). Although a few significantly high and low ones can be identified, reliability of 73% militates for caution. At the same time, low intraprogram variation is very encouraging. Variation attributable to differences between graders was minimal (ICC = 0.58%; CI: 0.0-1.2) which reassures us that the method of scoring is reliable, consistent, and likely extensible. CONCLUSION: Full resolution simulation based evaluation of critical care radiology interpretation is being conducted remotely and efficiently at large scale. A comprehensive mixed model of the resulting scores reliably quantifies case difficulty and resident competence.


Asunto(s)
Internado y Residencia , Radiología , Competencia Clínica , Cuidados Críticos , Humanos , Reproducibilidad de los Resultados
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