Therapeutic inertia in the management of neuromyelitis optica spectrum disorder.

Introduction and objective: Limited information is available on how neurologists make therapeutic decisions in neuromyelitis optica spectrum disorder (NMOSD), especially when new treatments with different mechanisms of action, administration, and safety profile are being approved. Decision-making can be complex under this uncertainty and may lead to therapeutic inertia (TI), which refers to lack of treatment initiation or intensification when therapeutic goals are not met. The study aim was to assess neurologists' TI in NMOSD. Methods: An online, cross-sectional study was conducted in collaboration with the Spanish Society of Neurology. Neurologists answered a survey composed of demographic characteristics, professional background, and behavioral traits. TI was defined as the lack of initiation or intensification with high-efficacy treatments when there is evidence of disease activity and was assessed through five NMOSD aquaporin-4 positive (AQP4+) simulated case scenarios. A multivariate logistic regression analysis was used to determine the association between neurologists' characteristics and TI. Results: A total of 78 neurologists were included (median interquartile range [IQR] age: 36.0 [29.0-46.0] years, 55.1% male, median [IQR] experience managing demyelinating conditions was 5.2 [3.0-11.1] years). The majority of participants were general neurologists (59.0%) attending a median (IQR) of 5.0 NMOSD patients (3.0-12.0) annually. Thirty participants (38.5%) were classified as having TI. Working in a low complexity hospital and giving high importance to patient's tolerability/safety when choosing a treatment were predictors of TI. Conclusion: TI is a common phenomenon among neurologists managing NMOSD AQP4+. Identifying TI and implementing specific intervention strategies may be critical to improving therapeutic decisions and patient care.

Coronavirus disease 2019 and lung cancer: where are we?

Oncology patients are more susceptible to severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection due to hospital contact and an immunological system that can be compromised by antineoplastic therapy and supportive treatments. Certain similarities have been described in the physiopathology of coronavirus disease 2019 (COVID-19) and lung cancer (LC) that may explain the higher probability of these patients of developing a more serious disease with more frequent hospitalizations and even death, especially with the addition of smoking, cardiovascular and respiratory comorbidities, old age and corticosteroids use. Pre-existing lesions and cancer therapies change the normal architecture of the lungs, so diagnostic scales such as COVID-19 Reporting and Data System (CO-RADS) are of vital importance for a correct diagnosis and patient homogenization, with a high inter-observer correlation. Moreover, anticancer treatments have required an adaptation to reduce the number of visits to the hospital [hypofractionated radiotherapy (RT), larger intervals between chemotherapy cycles, delay in follow-up tests, among others]. In a way, this has also caused a delay in the diagnosis of new cancers. On the other hand, vaccination has had a positive impact on the mortality of these patients, who maintain a similar seroprevalence to the rest of the population, with a similar impact in mortality.

Real-world persistence to first-line DMTs in relapsing-remitting multiple sclerosis.

BACKGROUND AND PURPOSE: disease-modifying treatments (DMT) for Multiple Sclerosis (MS) have expanded in recent years making the shared-decision process challenging. Moreover, no head-to-head studies are available within the first-line options. Our aim is to compare therapeutic persistence within first-line DMT: teriflunomide (TER), dimethyl fumarate (DMF), and injectable drugs (INJ) in a real-world setting. METHODS: Retrospective observational study analyzing diagnosed with Relapsing-Remitting Multiple Sclerosis (RRMS) who started DMT between January 2015 and April 2022 (TER=117, DMF=117, INJ=123). Clinical, radiological, and demographic variables were collected. The primary outcome was the median time to discontinuation of any DMT. Dropout was defined as discontinuation for 6 months for any reason. RESULTS: Of the total of 357 patients, 155 withdraw with a median time-to-discontinuation of 1.427 years (IQR 2.410). The discontinuation rate was higher in the injectable group, 49.6%; compared to teriflunomide 40.2%, and dimethyl fumarate 39.8% (p = 0.201). The most frequent reason of discontinuation differs within groups (lack of efficacy in TER, 63.8%, and adverse effects in DMF and INJ (40.4% and 40.9% respectively). No difference in persistence was observed between DMT (p = 0.30). After 2018 there has been a tendency to treat in a quick and early manner (lower EDSS; relapse rate and number of naïve patients), statistically significant for TER (p = 0.005, p = 0.010, and p = 0.045). CONCLUSIONS: Our study demonstrated no differences in persistence between the actual first-line DMT in a real-world setting, although a trend to favor oral-DMT was seen. Reasons for discontinuation differs within groups.

Oligometastatic Hormone-Sensitive Prostate Cancer. Why Radiotherapy?

Androgen deprivation therapy (ADT) has been considered for years the standard initial treatment for patients with metastatic prostate cancer (mPC). Recently published results support the use of taxanes, second-generation antiandrogens or radiotherapy to the primary tumor as part of the treatment in these patients, considering ADT alone as suboptimal. Metastasis-directed therapy (MDT) is used as part of the treatment for oligometastatic patients in different tumor types. In oligometastatic hormone-sensitive prostate cancer the role of MDT is being studied with promising results. In the present review we assess the available evidence for radiotherapy to the primary tumor in newly diagnosed mPC and for MDT in oligometastatic prostate cancer, as well as future directions in this clinical setting.

Development of a scale for the evaluation of the quality of the shared decision process in multiple sclerosis patients.

In the last years, therapeutic decisions in multiple sclerosis (MS) have become challenging due to expanded options with different treatment profiles attending to efficacy, safety, and route and frequency of administration. Moreover, patients with multiple sclerosis (PwMS) increasingly wish to be involved in their therapeutic decision process. Therefore, a new, patient-centric shared decision model (SDM), is gaining relevance. However, validated scales oriented to assess the quality of the process itself are lacking. The AGA-25 scale is a fit-for-purpose 25-item scale based on two validated scales in MS (Treatment Satisfaction Questionnaire for Medication (TSQM) and Decisional Conflict Scale (DCS)). The aim of this work is to develop and validate the AGAS-25 in Spanish. Two hundred and three PwMS (aged 17 to 67; 155 [76.4%] females) undergoing stable disease modifying treatment in the last 6 months were consecutively recruited. The Principal Component Analysis suggested a four-factor structure for the 25-item version of the questionnaire: 1) satisfaction with the SDM process 2) adverse events with the DMT, 3) convenience of the chosen-DMT and 4) information reliability. The internal consistency of the measurement was adequate (Cronbach's alpha = 0.88). Our results support the use of the AGAS-25 scale to assist SDM in Spanish-speaking PwMS.

Miositis carcinomatosa secundaria a cáncer gástrico / Carcinomatous myositis secondary to gastric cancer

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Hematoma epidural neonatal. Tres casos y revisión de la literatura / Neonatal epidural haematoma. Presentation of 3 cases and a literature review

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Fingolimod: efectividad y seguridad en la práctica clínica habitual. Estudio observacional, retrospectivo y multicéntrico en Galicia / Fingolimod: effectiveness and safety in routine clinical practice. An observational, retrospective, multi-centre study in Galicia

INTRODUCCIÓN: La efectividad y seguridad del fingolimod en pacientes con esclerosis múltiple remitente recurrente (EMRR) se demostró en ensayos clínicos. Sin embargo, por las limitaciones de éstos, es importante saber cómo se comporta en condiciones de práctica clínica habitual. Así, el objetivo de este estudio es evaluar la efectividad y seguridad del fingolimod después de 12 meses de uso en la práctica clínica en Galicia. PACIENTES Y MÉTODOS: Estudio retrospectivo y multicéntrico (n = 8) de pacientes con EMRR y tratados con una o más dosis de fingolimod, 0,5 mg/día. Se evaluó la efectividad -tasa anualizada de brotes (TAB), cambio en la puntuación de la escala expandida del estado de discapacidad (EDSS), porcentaje de pacientes libres de brotes, libres de progresión de discapacidad y libres de actividad en resonancia- para el total de pacientes y según tratamiento previo. Se evaluó la seguridad a partir del porcentaje de pacientes que discontinuaron y que presentaron efectos adversos. RESULTADOS: Después de 12 meses de uso, el fingolimod redujo un 87% la TAB (de 1,7 a 0,23; p < 0,0001) y, en consecuencia, un 81% de pacientes estuvo libre de brotes. La puntuación de la EDSS disminuyó un 9%. Un 91% de pacientes estuvo libre de progresión de discapacidad y un 72%, libre de actividad en resonancia. En el 43% de los pacientes no se evidenciaron signos de la actividad de la enfermedad. La mayoría de los beneficios del fingolimod difirieron según el tratamiento previo. Alrededor de un tercio de los pacientes comunicaron efectos adversos, pero sólo el 2% discontinuó debido a ellos. CONCLUSIONES: La mayoría de los resultados de efectividad de los ensayos clínicos del fingolimod se observa durante los 12 primeros meses de tratamiento en la práctica clínica. Se observó un mejor perfil de seguridad al comunicado en los ensayos clínicos

INTRODUCTION: The effectiveness and safety of fingolimod in patients with relapsing-remitting multiple sclerosis (RRMS) have been proven in clinical trials. Yet, due to their limitations, it is important to know how it behaves under everyday clinical practice conditions. Hence, the aim of this study is to evaluate the effectiveness and safety of fingolimod after 12 months' usage in clinical practice in Galicia. PATIENTS AND METHODS: We conducted a retrospective, multi-centre study (n = 8) of patients with RRMS who were treated with one or more doses of fingolimod, 0.5 mg/day. Effectiveness was assessed -annualised relapse rate (ARR), changes in the score on the Expanded Disability Status Scale (EDSS), percentage of patients free from relapses, free from progression of disability and free from activity in resonance- for the total number of patients and according to previous treatment. Safety was assessed based on the percentage of patients who withdrew and presented adverse side effects. RESULTS: After 12 months' use, fingolimod reduced the ARR by 87% (1.7 to 0.23; p < 0.0001) and, consequently, 81% of patients were free from relapses. The score was reduced by 9%. In all, 91% of patients were free from progression of disability and 72% were free from resonance activity. No signs of disease activity were found in 43% of the PATIENTS: Most of the benefits of fingolimod differed depending on previous treatment. About a third of the patients reported adverse side effects, but only 2% of them withdrew for this reason. CONCLUSIONS: In clinical practice, most of the results on the effectiveness of the clinical trials conducted with fingolimod were observed during the first 12 months of treatment. A better safety profile was observed than that reported in the clinical trials (AU)

Trends of Respiratory Syncytial Virus Infections in Children under 2 Years of Age in Puerto Rico.

OBJECTIVE: The respiratory syncytial virus (RSV) is the most significant viral pathogen causing bronchiolitis and pneumonia in infants, today. In tropical climates the RSV infection may occur throughout the year. The purpose of this study was to asses RSV infections during the 2009‒2010 RSV season in children under 2 years of age and to evaluate the trend of positive RSV tests in the period of 2007 to 2009. METHODS: A retrospective review of data collected from 6 hospitals in Puerto Rico was performed. Patients with confirmed RSV bronchiolitis were included in the study. RESULTS: A total of 4,678 patients were included. The mean age of the patients was 7 months. Data showed that RSV infection occurred throughout the studied months. CONCLUSION: Data confirms a year-round presence of RSV in Puerto Rico. The RSV surveillance system needs to be reinforced to establish and understand the epidemiology of RSV and to review the current immunoprophylaxis guidelines.

Palivizumab compliance by infants in Puerto Rico during the 2009-2010 respiratory syncytial virus season.

Respiratory syncytial virus (RSV) is the leading viral pathogen responsible for bronchiolitis and pneumonia in infants. We assessed palivizumab prophylaxis compliance for infants in Puerto Rico. We retrospectively studied data from 868 infants (409 females, 459 males) during the 2009-2010 RSV season. The infants had a mean gestational age of 33 weeks (range 23-41) and a mean birth weight of 1,767 g (range 509-4,120). Only 74 % of the infants with indications received prophylaxis. The main reasons for noncompliance were non-approval by the medical insurance (53 %), parents' unavailability (31 %), and infant sickness (11 %). Infants with the government medical insurance were more likely to be denied prophylaxis and to receive fewer doses. Parents need to be educated on the importance of RSV prophylaxis. Physicians should be aware that many infants are not being dosed appropriately and that strategies need to be established to improve compliance.

Neutropenia and thrombocytopenia in very low birth weight infants of women with preeclampsia.

OBJECTIVE: Determine the incidence of neutropenia and thrombocytopenia during the first week of life in very low birth weight (VLBW) infants born to mothers with preeclampsia. METHODS: Medical records of infants born to mothers with preeclampsia admitted to NICU from 2005-2007 were reviewed. RESULTS: A total of 93 infants were included with a mean birth weight of 988 g and gestational age of 29 weeks. Neutropenia was present in 49% of the infants and thrombocytopenia in 56%. Infants with neutropenia had lower birth weights (p<0.01) and were born at lower gestational ages (p<0.01). Neutropenia was associated to a higher prevalence of positive blood cultures during the first week of life especially coagulase negative organisms. CONCLUSIONS: In this group of VLBW infants born to mothers with preeclampsia, neutropenia was common and it was associated to increase risk of bacterial infections during the first week of life.

Mortality and risk factors for invasive fungal infections after the implementation of a fluconazole prophylaxis protocol in very low birth weight infants.

UNLABELLED: Fungal infections are a common cause of late-onset sepsis in very low birth weight infants and can impact mortality and morbidity. The purpose of this study is to determine the impact of systemic fluconazole prophylaxis in the prevalence of fungemia. METHODS: Subjects included 402 very low birth weight premature infants with birth weights between 500 and 1,250 grams admitted to the University Pediatric Hospital NICU over a six year period. The period before and after prophylaxis was retrospectively compared RESULTS: There was a marginal decrease in fungemia in the prophylaxis group (2.6% vs. 6.7%, p = 0.0525). Factors associated to fungemia were chorioamnionitis (p = 0.0240), H2-receptor inhibitors use (p = 0.0109), mechanical ventilati (p = 0.0049), prolonged antibiotic t py (p = 0.0015), and parenteral nutriti (p = 0.0048). Infants with fungemia had longer lengths of stay (p = 0.0143) and lower survival (p = 0.002). CONCLUSION: Prophylaxis with fluconazole decreases mortality and morbidity in an environment with a high incidence of late onset fungal infections. Early identification and prevention of risk factors must be reinforced.

Improvement in knowledge of nursing staff after an educational intervention about control of newborn temperature.

UNLABELLED: Newborn infants are at risk for hypothermia. Providing an adequate thermal environment is fundamental OBJECTIVE: To assess nurses knowledge about temperature control in newborns before and after an educational intervention and its effect in the prevalence of hypothermia. METHODS: Registered nurses working in a NICU received an educational intervention. They answered a pretest and posttest. Prevalence of hypothermia was recorded. RESULTS: Subjects included 55 nurses (89% females, 11% males). The subjects showed improvement in knowledge (81% correct answers before. 88% after, p < 0.01) Prevalence of hypothermia was similar before and after the intervention. CONCLUSION: An educational intervention in small group format was successful in improving nurse knowledge about temperature control in newborns. There was no effect in the prevalence of hypothermia. The intervention was aimed to registered nurses but neonatal hypothermia prevention requires interactions by all caregivers, thus educational interventions should be aimed to all personnel involved in neonatal care.

Atypical presentation of congenital cytomegalovirus infection in a neonate admitted to the neonatal intensive care unit: a case report.

Cytomegalovirus is a common cause of congenital infection in developed countries, affecting approximately 1% of live births. We report the case of a baby boy born with Cytomegalovirus pneumonitis dying from complications of persistent pulmonary hypertension of the newborn.

Non-familial stiff baby syndrome cases in the northern central area of Puerto Rico.

Hyperekplexia or Stiff Baby Syndrome is an uncommon disorder of non-epileptic movements with an autosomal dominant inheritance pattern. Neonates present exaggerated startle response to sensory stimuli and temporary muscular rigidity. We report two neonates from the northern center of Puerto Rico who presented involuntary movements, hypertonia and apnea during first days of life, being treated with anticonvulsants without improvement. Both were diagnosed with hyperekplexia due to the clinical evolution associated to normal EEG and head radiologic studies. Electromyography showed persistent muscular activity with periods of rest. Both were managed with clonazepam satisfactorily. We emphasized prompt recognition to prevent seizures misdiagnosis and administering appropriate treatment due to complications of sudden death secondary to stiffness and apnea.

Oseltamivir therapy in two low birth-weight premature infants born to women with H1N1 influenza.

The influenza A (H1N1) virus may cause severe illness in humans, especially children less than two years of age. The neuraminidase enzyme inhibitor, oseltamivir, is a potent medication for the management of patients with this infection. Minimal information exists regarding the immediate and long term effects of oseltamivir therapy in low birth weight premature infants born to mothers with this viral illness. This is a review of two premature male infants born to mothers with influenza A (H1N1), their presentation, clinical course, administration of oseltamivir, medical management and long term follow up through the first 18 months of life.

Propionic acidemia in a male newborn with Noonan syndrome: a case report.

We report a case of preterm infant born with prenatal diagnosis of cystic hygroma in the neck and congenital heart defect. Physical exam showed a hypotonic infant with dysmorphic features. Noonan syndrome was diagnosed. This newborn presented with progressive encephalopathy leading to a workup for metabolic disorders. The findings of elevated glycine levels associated with ketosis prompted the diagnosis of propionic acidemia. This case demonstrates that metabolic disorders can co-exist with other genetic syndromes and a high index of suspicion is needed in order to make the diagnosis and determine available treatment options. Genetic counseling should be offered to the parents as these disorders may affect future.

Congenital leukemia with leukemia cutis: a case report.

Congenital leukemia is a rare malignancy. Most of the neonatal cases reported have acute nonlymphoblastic leukemia. A number of chromosomal translocations and deletions have been found carrying an unfavorable prognosis. We report a newborn with congenital leukemia with leukemia cutis. The patient died before chemotherapy was started. The chromosome analysis showed 46, XY, t (4:11) (q21;q23). FISH for 11q23 MLL gene rearrangement showed positivity in 58% of interphase cells examined. Flow cytometry was reported with precursor B-cell acute lymphoblastic leukemia. This is a rare case of congenital leukemia with findings associated with poor prognosis.