Clinical Features and Therapeutic Outcomes Comparing Primary Mediastinal Large B-cell Lymphoma to Mediastinal Hodgkin Disease.

Currently, there is limited data available comparing Primary Mediastinal Large B-cell Lymphoma (PMBL) and mediastinal Hodgkin disease, nodular sclerosis type (HDNS). This is a retrospective cohort study that compares the clinical features, histology through immunohistochemistry (IHC) and treatment outcomes of 19 cases of PMBL and 39 cases of HDNS diagnosed over 13 years at a single institution in San Juan, PR. Superior Vena Cava syndrome (SVCS) and elevated Lactate Dehydrogenase (LDH) levels were more frequently seen in the PMBL cohort. At the median follow-up visit, of 74 months, no significant difference was seen in overall survival or progression free survival between PMBL and HDNS. Almost all of the relapses in the PMBL group occurred within 12 months of diagnosis. Our data suggests that PMBL and HDNS differ in their clinical presentation and have a favorable prognosis.

Resultados sensoriales y motores en pacientes miopes tratados con técnicas de superficies / Sensory and Motor Outcomes in Myopic Patients Treated with Surface Techniques

Objetivo: Evaluar los resultados sensoriales y motores poscirugía refractiva con técnicas de superficie (LASEK-MMC o PRK-MMC) en pacientes miopes con o sin astigmatismo asociado. Método: Se realizó estudio experimental controlado aleatorizado abierto, en el cual fueron incluidos 160 pacientes (320 ojos), de ellos tratados con LASEK-MMC (80 pacientes) y con PRK-MMC (80 pacientes), seguidos por 3 meses. Las principales variables evaluadas fueron: edad, tipo de error refractivo, equivalente esférico, ángulo Kappa, anisometropía, estereopsis, punto próximo de convergencia y su anomalía, amplitud de convergencia y de divergencia (cerca y lejos) y magnitud de la desviación del alineamiento ocular. Resultados: El grupo tratado con PRK-MMC tenía edad media de 26,48 años ± 4,47 y equivalente esférico preoperatorio de -3,27 ±1,54 que disminuyó significativamente (p <0,05) a -0,04 ± 0,23 dioptrías (D) a los tres meses de la cirugía. El grupo tratado con LASEK-MMC tenía edad media de 26,31 años ± 4,86 y equivalente esférico preoperatorio de -3,34 ± 1,66 dioptrías (D) que disminuyó significativamente (p <0,05) a -0,06 ± 0,26 D a los tres meses de la cirugía. Además, disminuyó la anisometropía, mejoró la estereopsis y disminuyó la amplitud la amplitud de convergencia y divergencia para lejos. El alineamiento ocular también mejoró. Conclusiones: Las técnicas de superficies para tratar pacientes con miopía o astigmatismo miópico compuesto disminuyen la anisometropía, mejoran la estereopsis y disminuyen la amplitud de convergencia y divergencia para lejos, con mayor proporción de pacientes con ortoforia después de la operación(AU)

Purpose : To evaluate sensory and motor outcomes after refractive surgery with surface techniques (LASEK-MMC or PRK-MMC) in myopic patients with or without associated astigmatism. Methods : An open randomized controlled experimental study was performed, in which 160 patients (320 eyes) were included, treated with LASEK-MMC (80 patients) and with PRK-MMC (80 patients), followed up during 3 months. The main variables evaluated were: age, type of refractive error, spherical equivalent, Kappa angle, anisometropia, stereopsis, near convergence point and its anomaly, convergence and divergence amplitude (near and far) and magnitude of ocular alignment deviation. Results : The group treated with PRK-MMC had an average age of 26.48 years ± 4.47 and preoperative spherical equivalent of -3.27 ± 1.54 which decreased significantly (p < 0.05) to -0.04 ± 0.23 diopters (D) three months after surgery. The LASEK-MMC treated group had an average age of 26.31 years ± 4.86 and preoperative spherical equivalent of -3.34 ± 1.66 diopters (D) which decreased significantly (p < 0.05) to -0.06 ± 0.26 D three months after surgery. In addition, anisometropia decreased, stereopsis improved, Kappa angle increased, and convergence and divergence amplitude (for far) decreased; ocular alignment also improved. Conclusions : Surface techniques to treat patients with myopia or compound myopic astigmatism decrease anisometropia, improve stereopsis, increase Kappa angle, and decrease near point anomaly and convergence and divergence amplitude (for far), with higher proportion of patients with orthophoria postoperatively(AU)

Failure of human rhombic lip differentiation underlies medulloblastoma formation.

Medulloblastoma (MB) comprises a group of heterogeneous paediatric embryonal neoplasms of the hindbrain with strong links to early development of the hindbrain1-4. Mutations that activate Sonic hedgehog signalling lead to Sonic hedgehog MB in the upper rhombic lip (RL) granule cell lineage5-8. By contrast, mutations that activate WNT signalling lead to WNT MB in the lower RL9,10. However, little is known about the more commonly occurring group 4 (G4) MB, which is thought to arise in the unipolar brush cell lineage3,4. Here we demonstrate that somatic mutations that cause G4 MB converge on the core binding factor alpha (CBFA) complex and mutually exclusive alterations that affect CBFA2T2, CBFA2T3, PRDM6, UTX and OTX2. CBFA2T2 is expressed early in the progenitor cells of the cerebellar RL subventricular zone in Homo sapiens, and G4 MB transcriptionally resembles these progenitors but are stalled in developmental time. Knockdown of OTX2 in model systems relieves this differentiation blockade, which allows MB cells to spontaneously proceed along normal developmental differentiation trajectories. The specific nature of the split human RL, which is destined to generate most of the neurons in the human brain, and its high level of susceptible EOMES+KI67+ unipolar brush cell progenitor cells probably predisposes our species to the development of G4 MB.

Presión de perfusión ocular en sujetos sin enfermedad ocular / Ocular Perfusion Pressure in Subjects Without Ocular Disease

Objetivo: Describir el comportamiento de la presión de perfusión ocular en sujetos sin enfermedad ocular. Métodos: Se realizó un estudio descriptivo transversal con sujetos sin enfermedad ocular de la consulta externa de Oftalmología del Hospital Universitario "General Calixto García", entre enero y diciembre de 2019. Resultados: La mediana de presión intraocular del ojo derecho osciló entre 13,0-16,0 mmHg, y del ojo izquierdo entre 13,0-15,5 mmHg, durante todo el estudio. El valor máximo para ambos ojos se obtuvo a las 6:00 a. m. (madrugada). La mediana de presión arterial sistólica osciló entre 129,0-138,5 mmHg, y de diastólica entre 79,5-81,5 mmHg. El valor mínimo de presión arterial diastólica fue 53 mmHg a las 6:00 a. m. La mediana de presión de perfusión ocular del ojo derecho osciló entre 46,4 mmHg (12:00 a. m.) y 50,8 mmHg; y del ojo izquierdo entre 47,3 mmHg (6:00 a. m.) y 51,9 mmHg. El valor mínimo específico de presión de perfusión ocular fue 35 mmHg para ambos ojos, a las 6:00 a. m. Conclusiones: La hipertensión arterial es el antecedente patológico personal más frecuente en la población estudiada y en ocasiones se producen cifras fuera de la normalidad, tanto elevadas como disminuidas, capaces de afectar la presión de perfusión ocular, sobre todo la diastólica baja. Sin embargo, las presiones de perfusión ocular se mantuvieron normales, probablemente, por mecanismos de autorregulación individuales(AU)

Objective: To describe the behavior of ocular perfusion pressure in subjects without ocular disease. Methods: A cross-sectional descriptive study was performed with subjects without ocular disease from the Ophthalmology outpatient clinic of the University Hospital "General Calixto García", between January and December 2019. Results: The median intraocular pressure of the right eye ranged between 13.0-16.0 mmHg, and of the left eye between 13.0-15.5 mmHg, throughout the study. The maximum value for both eyes was obtained at 6:00 a. m. (early morning). The median systolic blood pressure ranged from 129.0-138.5 mmHg, and diastolic from 79.5-81.5 mmHg. The minimum diastolic blood pressure value was 53 mmHg at 6:00 a.m. The median ocular perfusion pressure of the right eye ranged from 46.4 mmHg (12:00 a.m.) to 50.8 mmHg; and of the left eye from 47.3 mmHg (6:00 a.m.) to 51.9 mmHg. The minimum specific ocular perfusion pressure value was 35 mmHg for both eyes, at 6:00 a.m. Conclusions: Arterial hypertension is the most frequent personal pathologic antecedent in the population studied, and both elevated and decreased out-of-normal figures capable of affecting ocular perfusion pressure, especially low diastolic, occasionally occur. However, ocular perfusion pressures remained normal, probably due to individual autoregulatory mechanisms(AU)

Sensibilidad al contraste y aberrometría ocular en pacientes operados de miopía con técnicas de superficie / Contrast Sensitivity and Ocular Aberrometry in Patients Operated on for Myopia with Surface Techniques

Objetivo: Determinar el cambio de la sensibilidad al contraste en pacientes operados de miopía con técnicas de superficie y su relación con la aberrometría ocular. Métodos: Se realizó un estudio experimental con 324 ojos de 162 pacientes miopes atendidos en la consulta de Cirugía Refractiva del Instituto Cubano de Oftalmología "Ramón Pando Ferrer" en el período de enero 2019 y marzo de 2020 y que fueron operados de cirugía refractiva con láser de excímero, técnicas de superficie y perfil de ablación asférico. La sensibilidad al contraste fue la variable principal de comparación entre ambas técnicas quirúrgicas. Se formaron dos grupos de pacientes, cuya asignación fue secuencial, al primer grupo, se le realizó la técnica quirúrgica PRK-MMC (81) y al segundo LASEK-MMC (81) con un seguimiento de 3 meses. Resultados: Predominaron las mujeres con miopía leve y edades entre 21 y 28 años. A los tres meses la sensibilidad al contraste, agudeza visual sin corrección, equivalente esférico y el valor cuadrático medio total mejoraron de manera significativa, mostrando una relación positiva con la sensibilidad al contraste en ambos grupos, sin diferencias entre ellos. Conclusiones: La sensibilidad al contraste mejora con técnicas de superficie y posee una relación positiva y significativa con los resultados visuales, refractivos y aberrométricos. El estudio de las aberraciones oculares proporciona un arma fundamental para valorar la calidad óptica del ojo humano, información que permite conocer la calidad visual que se puede esperar en los pacientes evaluados(AU)

Objective: To determine the change in contrast sensitivity in patients operated on for myopia with surface techniques and its relationship with ocular aberrometry. Methods: An experimental study was carried out with 324 eyes of 162 myopic patients treated at the Refractive Surgery Clinic of the Cuban Institute of Ophthalmology "Ramón Pando Ferrer" between January 2019 and March 2020 who underwent refractive surgery with excimer laser and surface techniques and aspheric ablation profile. Contrast sensitivity was the main variable of comparison between both surgical techniques. Two groups of patients were sequentially assigned, the first group underwent PRK-MMC (81) and the second LASEK-MMC (81) with a 3-month follow-up. Results: Women with mild myopia and ages between 21 and 28 years predominated. After 3 months contrast sensitivity, uncorrected visual acuity, spherical equivalent and total average square value improved significantly, showing a positive relationship with contrast sensitivity in both groups, with no differences between them. Conclusions: Contrast sensitivity improves with surface techniques and has a positive and significant relationship with visual, refractive and aberrometric outcomes. The study of ocular aberrations provides a fundamental tool to assess the optical quality of the human eye, information that allows us to know the vision quality that can be expected in the patients evaluated(AU)

Is a Non-Caloric Sweetener-Free Diet Good to Treat Functional Gastrointestinal Disorder Symptoms? A Randomized Controlled Trial.

Background: A diet containing non-caloric sweeteners (NCS) could reduce calorie intake; conversely, some animal studies suggest that NCS consumption may increase functional gastrointestinal disorder symptoms (FGDs). This study aimed to compare the effect of consuming a diet containing NCS (c-NCS) versus a non-caloric sweetener-free diet (NCS-f) on FGDs. Methods: We conducted a randomized, controlled, parallel-group study using two different diets for five weeks: the c-NCS diet contained 50−100 mg/day NCS, whereas the NCS-f diet had less than 10 mg/day NCS. At the beginning of the study (PreTx) and at the end (PostTx), we assessed FGDs, dietary intake, and NCS consumption. Results: The percentage of participants with diarrhea (PreTx = 19% vs. PstTx = 56%; p = 0.02), post-prandial discomfort (PreTx = 9% vs. PstTx = 39%; p = 0.02), constipation (PreTx = 30% vs. PostTx = 56%; p < 0.01), and burning (PreTx = 13% vs. PostTx = 33%; p < 0.01) increased in the c-NCS diet group. Conversely, abdominal pain (PreTx = 15% vs. PostTx = 3%; p = 0.04), post-prandial discomfort (PreTx = 26% vs. PostTx = 6%; p = 0.02), burning (PreTx = 15% vs. PostTx = 0%; p = 0.02), early satiety (PreTx = 18% vs. PostTx = 3%; p < 0.01), and epigastric pain (PreTx = 38% vs. PostTx = 3%; p < 0.01) decreased in the NCS-f diet group. Conclusion: A c-NCS diet is associated with increased FGDs, including diarrhea, post-prandial discomfort, constipation, and burning or retrosternal pain. The NCS-f diet also decreased FGDs, as well as abdominal pain, post-prandial discomfort, burning or retrosternal pain, early satiety, and epigastric pain.

Resultados refractivos y visuales en cirugía fotoablativa en pacientes miopes / Refractive and visual results in photoablative surgery in myope patients

Objetivo: Evaluar los resultados refractivos y visuales post cirugía refractiva corneal con láser de excímero (LASEK-MMC o PRK-MMC) en pacientes miopes con o sin astigmatismo asociado. Método: Se realizó un estudio preexperimental del tipo antes y después, en el que fueron incluidos 81 pacientes (162 ojos) tratados con LASEK-MMC (65 pacientes) o PRK-MMC (16 pacientes) seguidos por tres meses. Se les realizó un examen oftalmológico completo y se evaluaron las variables: edad, sexo, diagnóstico y tipo de miopía, así como agudeza visual, esfera, cilindro, equivalente esférico precirugía y poscirugía. Además, se analizó la función visual y los resultados de la refracción. Resultados: El valor de la mediana de edad fue de 24,0 años en los pacientes tratados con LASEK-MMC y en el grupo con PRK-MMC fue de 23,0 años. En ambos grupos existió un predominio del sexo femenino. La totalidad de los pacientes tratados presentaba un astigmatismo miópico compuesto con niveles de miopía leve. A los tres meses el 96,9 por ciento de los pacientes tratados con LASEK-MMC y el 93,8 por ciento de los tratados con PRK-MMC tenían una agudeza visual no corregida de 20/20 o más y todos tenían una visión de 20/40 o más. Conclusiones: La cirugía fotoablativa con láser tiene buenos resultados refractivos y visuales ya que la mayoría de los pacientes quedaron emétropes con una marcada mejoría de la agudeza visual sin corrección y de la refracción en el posoperatorio(AU)

Objective: To evaluate refractive and visual outcomes after excimer laser corneal refractive surgery (laser-assisted subepithelial keratectomy [LASEK] or photorefractive keratectomy [PRK], with intraoperative application of mitomycin-C [MMC]) in myopic patients with or without associated astigmatism. Methods: A preexperimental, before-and-after study was carried out with 81 patients (162 eyes) treated with LASEK-MMC (65 patients) or PRK-MMC (16 patients) and followed for three months. A complete ophthalmologic examination was done and the following variables age, sex, diagnosis and type of myopia were assessed, as well as pre-surgery and post-surgery visual acuity, sphere, cylinder and spherical equivalent. In addition, visual function and the refraction results were analyzed. Results: The median age was 24.0 years in the LASEK-MMC group and 23.0 years in the PRK-MMC group. In both groups there was a predominance of the female sex. All treated patients had compound myopic astigmatism with mild myopia. At three months, 96.9 percent of patients treated with LASEK-MMC and 93.8 percent of patients treated with PRK-MMC had uncorrected visual acuity of 20/20 or better, and all had vision of 20/40 or better. Conclusions: Laser photoablative surgery has good refractive and visual outcomes as most patients became emmetropic with marked improvement in uncorrected visual acuity and refraction postoperatively(AU)

Early versus late response to daratumumab-based triplet therapies in patients with multiple myeloma: a pooled analysis of trials POLLUX, CASTOR and MAIA.

In multiple myeloma (MM), it is unclear whether early and late responders to daratumumab have similar outcomes. We pooled individual-level data from phase 3 trials and divided them into early and late response groups based on median time to response. Altogether 670 and 213 patients achieved very good partial response (VGPR) or better and minimal residual disease (MRD) negativity, respectively. Among VGPR or better, there was no significant difference of modified progression-free survival (mPFS, hazard ratio [HR] 1.00, 95% confidence interval [CI] 0.69-1.44) or duration of response (DOR) (HR 1.02, 95%CI 0.68-1.53). Among relapsed/refractory MM (RRMM) achieving MRD negativity, late responders had significantly longer mPFS (p = 0.038) and DOR (p = 0.043). These results support that for patients who failed to achieve an early response to daratumumab, therapies should be continued with the goal of achieving ongoing and stepwise improvement of response.

Impact of Consensus Definitions on Identification of Glomerular Lesions by Light and Electron Microscopy.

INTRODUCTION: In 2020, a working group of 13 renal pathologists published consensus definitions for 47 individual glomerular lesions found on light microscopy (LM) and 47 glomerular lesions and 9 normal structures found on electron microscopy (EM). METHODS: To test the impact of these definitions on identification of these lesions and structures, 2 surveys were circulated to all members of the Renal Pathology Society (RPS), each having 32 images (19 LM, 13 EM) and accompanying questions with 5 multiple-choice answers, one being the consensus choice of the working group. The first survey (survey 1 [S1]), answered by 297 RPS members, was sent in September 2020, before publication of the consensus definitions. The second (survey 2 [S2]), with images of the same lesions and structures (but not the same images) and the same questions and multiple choices in different order, was sent in April 2020, 5 months after the publication of the definitions. RESULTS: S2 was taken by 181 RPS members; 64% also took S1 and 61% reported having read the definitions paper (def. paper). Mean agreement with the consensus answers increased modestly between the 2 surveys (65.2% vs. 72.0%, P = 0.097); the increase was greater and significant when only respondents to S2 who read the def. paper were considered (65.2% vs. 74.8%, P = 0.026). Furthermore, in S2 agreement with consensus answers was greater among respondents who read this paper versus those who did not (66.9% vs. 74.8%, P < 0.0001). CONCLUSIONS: Publication of the consensus definitions modestly improved interobserver agreement in identification of glomerular lesions.