VEXAS syndrome unveiled: a multidimensional journey from clinical presentation to management.

An 83-year-old man with a history of monoclonal gammopathy of unknown significance, macrocytic anemia, and cytopenias presented with hemorrhagic bullae on his left hand, alongside intermittent fevers and joint pain. Laboratory findings indicated anemia, elevated mean corpuscular volume, thrombocytopenia, leukopenia, and a high C-reactive protein level. A biopsy of the bullae showed neutrophilic dermatosis, and computed tomography scans of the thorax revealed lung opacities and mediastinal lymphadenopathy, suggesting neutrophilic alveolitis. Bone marrow examination found hypercellularity with myeloid and histiocytic hyperplasia, vacuolated precursors, and 3% blasts of an immature myelomonocytic lineage. Genetic testing uncovered a UBA1 mutation at an 81% allele frequency, confirming a diagnosis of VEXAS syndrome. Treatment commenced with prednisone, initiated at 60 mg daily and tapered to 10 mg, with tocilizumab considered for future symptom management. This regimen has successfully maintained remission, as observed in follow-up appointments. This case highlights the diagnostic complexity and effective management of VEXAS syndrome, underscoring the importance of genetic testing in guiding treatment decisions.

VEXAS syndrome, initially identified in 2020, is a rare disease characterized by overlapping hematologic and rheumatologic conditions, primarily affecting middle-aged to older men.Our case presents a unique instance of VEXAS syndrome, featuring inflammatory symptoms alongside pancytopenia and a history of monoclonal gammopathy of unknown significance, highlighting the diverse clinical spectrum of this condition.The diagnosis was confirmed through bone marrow biopsy revealing vacuoles in myeloid precursors and a UBA1 mutation, reaffirming the diagnostic criteria outlined for VEXAS syndrome.Management challenges persist, with current treatment options limited to glucocorticoids for symptomatic relief, underscoring the need for further research into targeted therapies such as ruxolitinib to improve patient outcomes.

Venous thromboembolism and cannabis consumption, outcomes among hospitalized patients in the United States: A nationwide analysis.

Venous Thromboembolism (VTE) carries significant clinical implications, and with the rise in cannabis consumption, its potential influence on VTE outcomes warrants investigation. Using the National Inpatient Sample (NIS) database (2016-2019), we analyzed 2,217,184 hospitalized VTE patients. Among these, 1.8 % (38,810) reported cannabis use. We compared demographics, comorbidities, in-hospital outcomes, and quality metrics between cannabis users and non-users with VTE. Cannabis users were chiefly younger males (average age 45 in cannabis users vs. 62 in non-cannabis users) from lower-income brackets. Notably, 5.4 % discharged against medical advice. Although in-hospital mortality was initially lower for cannabis users (2.8 % vs. 5.1 %, OR 0.6, 95 % CI 0.69-0.94, p = 0.008), this difference became non-significant post-propensity-score matching (aOR 0.9, 95% CI 0.72-1.10, p = 0.3). Non-users faced higher in-hospital complications, a trend that persisted post-PSM. Among cannabis users, key mortality predictors were peripheral vascular disease, acute kidney injury, vasopressor use, cardiogenic shock, myocardial infarction, invasive ventilation, and surgical embolectomy. Cannabis users also had a shorter hospital stay (4.2 vs. 5.4 days) and slightly reduced costs ($27,472.95 vs. $31,660.75). The significantly younger age of VTE patients who use cannabis, coupled with the considerable proportion discharging against medical advice, underscores the urgency for tailored care interventions. Additional research is vital to comprehensively understand the interplay between cannabis consumption and VTE outcomes.

Unraveling Bacillus Calmette-Guérin (BCG) Therapy Side Effects in Bladder Cancer: A Tale of Triumph Over Treatment Challenges.

This case report presents a 66-year-old male with a complex medical history, including testicular cancer, chronic obstructive pulmonary disease, obstructive sleep apnea, tobacco use disorder, erectile dysfunction, and obesity. The patient exhibited recurrent gross hematuria, leading to a comprehensive workup. Cystoscopy revealed a bladder tumor, prompting transurethral resection and mitomycin C instillation. Subsequent intravesical Bacillus Calmette-Guérin (BCG) therapy was initiated but resulted in severe sepsis during maintenance. Despite initial suspicion of BCG-induced sepsis, further evaluation suggested a reaction with chemical cystitis. Treatment involved brief antimicrobial therapy, and the patient's condition improved. This case highlights the challenges in managing BCG therapy complications, emphasizing the need for prompt intervention, careful monitoring, and consideration of risk factors. Patient education and vigilant follow-ups are crucial for addressing potential long-term effects.

Características clínicas de gestantes en trabajo de parto con infección SARSCoV-2 en la altura: serie de casos / Clinical characteristics of pregnant women in labor with SARS-CoV-2 infection at high altitude: A case series

Resumen Introducción. A gran altitud, se ha observado menos casos y menos letalidad de COVID-19 en comparación con cifras reportadas a nivel del mar. Actualmente no hay publicaciones que informen el comportamiento clínico de COVID-19 en mujeres embarazadas adaptadas a la altura e hipoxia crónica en el Perú. Métodos. Estudio retrospectivo mediante revisión de historias clínicas del 6 de marzo de 2020 al 15 de junio de 2020. Se describe los primeros trece casos de gestantes con COVID-19 atendidos en el Hospital COVID-19 Simón Bolívar, ubicado a 2 750 msnm. Los casos procedían de altitudes entre 2 035 msnm y 3 502 msnm. El análisis estadístico se realizó con SPSS, versión 19.0. Resultados. Trece casos de gestantes con COVID-19 confirmado por IGM para SARS-CoV-2, fueron tratadas a 2 750 msnm (9 022,31 pies), en los Andes peruanos. El parto fue por cesárea en ocho casos (61,5%) y por vía vaginal en cinco (38,5%). Hubo dos casos (15,4%) de preeclampsia, uno de ellos diagnosticado como síndrome HELLP, prematuridad y muerte fetal. Tres casos (23,1%) desarrollaron hipotonía uterina posparto y requirieron sutura de Hayman o B-Lynch. Dos casos (15,4%) se complicaron con oligohidramnios y otros dos casos con infección urinaria. Los niveles de hemoglobina variaron entre 11,1 y 16 g/dL. Solo un caso (7,7%) presentó sintomatología, con dolor faríngeo leve. No se observó transmisión vertical detectada por IgM/IgG para SARS-CoV-2. La evolución clínica fue favorable en los trece casos y el alta fue a los 2 a 4 días, para continuar la cuarentena en domicilio. Conclusiones. En el presente estudio preliminar, las gestantes en trabajo de parto con COVID-19 por prueba rápida IgM para SARS-CoV-2 en la altura fueron generalmente asintomáticas; no hubo transmisión vertical. Se presentó alto porcentaje de otras complicaciones obstétricas.

Abstract Introduction: Fewer COVID-19 cases and less lethality have been observed at high altitude compared to cases reported at sea level. There are currently no publications reporting clinical behavior of pregnant women with COVID-19 at high altitude. Methods: This is a retrospective study with review of medical records between March 6, 2020 and June 15, 2020. The first thirteen cases of pregnant women with COVID-19 who were attended at Simón Bolívar COVID-19 Hospital, located at 2 750 meters above sea level, are described. The cases came from altitudes between 2 035 and 3 502 meters above sea level (masl). Statistical analysis used SPSS, version 19.0. Results: Thirteen cases of pregnant women with COVID-19 confirmed by IgM for SARS-CoV-2 were attended at 2 750 masl (9 022.31 feet) in the Peruvian Andes. Delivery by cesarean section occurred in eight cases (61.5%) and five (38.5%) delivered vaginally. There were two cases (15.4%) of preeclampsia, one with diagnosis of HELLP syndrome, prematurity and fetal death. Three cases (23.1%) developed uterine hypotonia that required Hayman or B-Lynch suture. Two cases (15.38%) were complicated with oligohydramnios and two with urinary infection. Hemoglobin levels were between 11.1 and 16 g/dL. Only one case (7.7%) was symptomatic, with mild pharyngeal pain. No vertical transmission was detected by IgM/IgG for SARS-CoV-2. Clinical evolution was favorable in the thirteen cases and they were discharged after 2 to 4 days hospitalization to continue home quarantine. Conclusions: Results in this short study show pregnant women in labor with COVID-19 by rapid IgM test for SARS-CoV-2 at high altitude were mostly asymptomatic; there was no vertical transmission, but high presence of other obstetrical complications.

Simulación versus exposición convencional en la enseñanza de preeclampsia severa

Objective: To determine if simulation is more effective than conventional exposition in the improvement in nurses' knowledge of severe preeclampsia. Methods: Crosssectional quasi-experimental study in two private clinics in Cajamarca, Peru. We compared the improvement in nurses' knowledge of severe preeclampsia: 13 attended to a conventional exposition about severe preeclampsia (Exconv) and 16, the experimental group, had the same exposition and four simulation training sessions (Sim). We conducted a pretest in both groups and a posttest in 20 participants; these were composed of multiple choice questions previously validated by experts; Spearman-Brown reliability was 0.76. The simulation training sessions were recorded in video and evaluated by an expert with a checklist adapted from the American College of Obstetricians and Gynecologists. Data was processed with SPSS 20.0. We used Spearman-Brown correlation tests and Student's t test for paired samples to compare the scores obtained in pretest and posttest, p <0.05. Results: The control group's (Exconv) pretest and posttest scores were 11.69 ± 2.53 and 13.85 ± 2.27, p = 0.02, and pedagogical gain was 2.15 ± 2.88. The experimental group's (Sim) pretest and posttest scores were 10.25 ± 2.79 and 14.69 ± 1.89, with a pedagogical gain of 4.44 ± 2.22, p = 0.000. The pedagogical gain of the experimental group was greater than that of the control group, p = 0.002. Conclusions: Greater improvement in knowledge was obtained by teaching severe preeclampsia with simulation training sessions than with conventional exposition solely.

Objetivo. Determinar si la simulación es más efectiva que la exposición convencional en la mejora cognitiva de enfermeras en preeclampsia severa. Métodos. Investigación transversal cuasiexperimental realizada en dos clínicas de Cajamarca, Perú. Se comparó las mejoras cognitivas de enfermeras sobre la preeclampsia severa: 13 fueron sometidas a exposición convencional sobre preeclampsia severa (Exconv) y 16, asignadas al grupo experimental, tuvieron una exposición y 4 sesiones de simulación (Sim). Ambos grupos respondieron un pretest y, luego de las sesiones, un postest de 20 preguntas de opción múltiple, previamente validado por expertos y con confiabilidad de Spearman-Brown de 0,76. Todas las sesiones de simulación fueron guardadas en vídeo y evaluadas por un experto, con lista de verificación adaptada del American College of Obstetricians and Gynecologists. Los datos fueron procesados con el programa SPSS 20.0. Se usaron las pruebas Spearman-Brown de correlación, t de student para muestras relacionadas para comparar los puntajes obtenidos en pretest y postest, con p< 0,05. Resultados. Los puntajes del grupo control (Exconv) en el pretest y postest fueron: 11,69 ± 2,53 y 13,85 ± 2,27, p=0,02, con ganancia pedagógica de 2,15 ± 2,88. Los puntajes de pretest y postest del grupo experimental (Sim) fueron 10,25 ± 2,79 y 14,69 ± 1,89, con ganancia pedagógica de 4,44 ± 2,22 con p=0,000. Las ganancias pedagógicas del grupo experimental fueron mayores que las del grupo control, con p=0,002. Conclusión. La enseñanza de preeclampsia severa con simulación obtuvo mejoras cognitivas mayores que con la exposición convencional.

Enfermedad cerebrovascular hemorrágica en la eclampsia asociada al síndrome HELLP / Stroke in HELLP syndrome-associated eclampsia

Introduction: Eclampsia (E) and HELLP syndrome (H) are two complications of preeclampsia that increase maternal morbidity and mortality. The main complication and the main cause of death of this EH / HE association is the hemorrhagic cerebrovascular disease (HCD). Objectives: To determine differences between women with EH / HE who presented HCD and those who did not. To define the types of HCD in women with EH / HE. Design: Cross-sectional comparative study. Patients: Cases of EH / HE at the Hospital Regional Docente de Cajamarca, Peru, 2015. Interventions: Patients with EH / HE were divided into two groups: those without HCD and those with HCD. SPSS 20.0 was used. The comparison of groups was done with Mann Whitney U and chi square tests. Significant differences were when p <0.05. Results: There were 23 women with EH / HE: 18 (78.3%) without HCD and 5 (21.7%) with HCD. We compared women with EH / HE who had HCD and those who did not. HCD had OR = 4.44 (95% CI 1.19-16.55) p = 0.043 for risk of death, and platelets less than 30 000 had OR = 4.44 (95% CI 1.19-16,55) with p = 0.043 risk for HCD. In addition, the stay in ICU was longer in those with HCD than without HCD. Mortality was 60%; 80% of the HCD had ventricular compromise. There was 66.7% of the patients presented subarachnoid hemorrhage and 66.7%, intraventricular hemorrhage (IVH). In IVH, 100% had lobar compromise of which 66.7% had occipital compromise. Conclusions: Hemorrhagic cerebrovascular disease in women with HELLP syndrome associated with eclampsia is related to lower platelet levels; this increases the risk of death and prolongs stay in the intensive care unit.

Introducción. La eclampsia (E) y el síndrome HELLP (H) son dos complicaciones de la preeclampsia que, asociadas, pueden aumentar la morbimortalidad materna. La principal complicación y la causa principal de muerte de esta asociación EH/ HE es la enfermedad cerebrovascular hemorrágica (ECH). Objetivos. Determinar diferencias entre las mujeres con EH/HE que presentan ECH y las que no. Definir los tipos de ECH en mujeres con EH/HE. Diseño. Estudio transversal comparativo. Pacientes. Casos de EH/HE en el Hospital Regional Docente de Cajamarca, Perú, 2015. Intervenciones. Se dividió las pacientes con EH/HE en dos grupos: sin ECH y con ECH. Se utilizó el software SPSS 20.0. La comparación de los grupos se realizó con la U de Mann Whitney y chi cuadrado. Fueron diferencias significativas cuando p< 0,05. Resultados. Hubo 23 mujeres con EH/HE: 18 (78,3%) sin ECH y 5 (21,7%) con ECH. Comparamos las mujeres con EH/HE que tenían o no ECH. ECH tuvo OR 4,44 (IC95% 1,19 a 16,55) con p=0,043 de riesgo de muerte, y las plaquetas menores de 30 000 tuvieron OR 4,44 (IC95% 1,19 a 6,55) con p=0,043 de riesgo de ECH. Además, la permanencia en UCI fue mayor que en las que no tuvieron ECH. La mortalidad fue 60%. El 80% de las ECH mostró compromiso ventricular. Hubo 66,7% de hemorragia subaracnoidea y 66,7% de hemorragia intraventricular (HIC). En las HIC, el 100% tuvo compromiso lobar, de las cuales 66,7% mostró compromiso occipital. Conclusiones. La ECH en mujeres con síndrome HELLP asociado a eclampsia estuvo relacionado a valores más bajos de plaquetas y aumentó el riesgo de muerte y estancia en cuidados intensivos.

Características moleculares y susceptibilidada antibióticos de cepas de Staphylococcusaureus colonizantes de pacientes conpatologías nasales procedentes de Cartagena(Colombia), 2015: estudio observacional / Molecular characterization and antibiotic susceptibility of Staphylococcus aureus strains colonizing patients with nasal pathology from Cartagena (Colombia), 2015: observational study

Staphylococcus aureus es uno de los principales patógenos causantes deinfecciones tanto a nivel hospitalario como comunitario. El objetivo de este estudio fuedeterminar características moleculares y los perfiles de resistencia a antibióticos deaislamientos de Staphylococcus aureus colonizantes de pacientes con patología nasalatendidos en el servicio de Otorrinolaringología del Hospital Universitario del Caribede la ciudad de Cartagena, Colombia. Métodos: los aislamientos de Staphylococcusaureus obtenidos de pacientes colonizados fueron sometidos a extracción de ADNy ensayos de PCR para determinar la presencia de los genes mecA y lukS/F-PV.Se determinó la pertenencia de las cepas a los complejos clonales CC5 y CC8, y sedeterminaron las relaciones clonales de los aislamientos mediante análisis de PFGE.A las cepas SARM se les tipificó y sub-tipificó el casete cromosomal estafilocócicoSCCmec mediante PCR múltiple. Resultados: la prevalencia de colonización encontradapara Staphylococcus aureus fue 22.8% y 5.26% para SARM. Del total de aislamientosSARM, 33.3% estuvieron relacionados con el clon CC8-SARM-IVc y 22.28%con CC8-SARM-IVa. Los genes para la leucocidina PVL se identificaron en 66.7% deaislamientos SARM y 40% de aislamientos sensibles a meticilina (SASM)...

Comercializacion de sucedaneos de la leche materna en farmacias de la ciudad de La Paz y El Alto, 1996 / Commerce of succedaneous maternal milk in drug strores in the cities of La Paz and El Alto, 1996

El estudio muestra los resultados de una encuesta realizada en las ciudades de la Paz y El Alto, sobre comercializacion de sucedaneos de la leche materna (todo alimento comercializado o de otro modo presentado como substituto parcial o toal de la leche materna, sea o no adecuado para este fin) y alimentos complentarios (todo alimento manufacturado o preparado localmente, que convenga como complemento de la leche matrna o de las preparaciones para lactantes, cuando aquella o estas resulten insuficientes para satisfacer las necesidades nutricionales del lactante. Este tipo de alimento se suele llamar tambien "alimento de destete" o suplemento de la leche materna) en 78 farmacias de estas ciudades. Los encuestadores personificaron familiares con problemas para amamantar a su bebe, solicitando orientacion sobre acciones mas aconsejables. Preguntas tales como si es necesario visitar a un medico, suspender la lactancia materna, iniciar alimentacion con leche artificial o formula infantil, precio del sucedaneo, tiempo de administracion, producto aconsejado, edad de incio de alimentos complementarios al bebe, fueron planteadas. Se indago sobre el criterio de los responsables de farmacias sobre la utilidad o no de los estimulantes del apetito y/o vitaminas, las marcas de biberones (mamaderas) y chupones (tetinas), si ellos los recomiendan y si las leches maternizadas son saludables, sus desventajas y ventajas. Los resultados de estas interrogantes y otras se presentan y discusion en el presente articulo