Evolution of genome space occupation in ferns: linking genome diversity and species richness.

BACKGROUND AND AIMS: The dynamics of genome evolution caused by whole genome duplications and other processes are hypothesized to shape the diversification of plants and thus contribute to the astonishing variation in species richness among the main lineages of land plants. Ferns, the second most species-rich lineage of land plants, are highly suitable to test this hypothesis because of several unique features that distinguish fern genomes from those of seed plants. In this study, we tested the hypothesis that genome diversity and disparity shape fern species diversity by recording several parameters related to genome size and chromosome number. METHODS: We conducted de novo measurement of DNA C-values across the fern phylogeny to reconstruct the phylogenetic history of the genome space occupation in ferns by integrating genomic parameters such as genome size, chromosome number and average DNA amount per chromosome into a time-scaled phylogenetic framework. Using phylogenetic generalized least square methods, we determined correlations between chromosome number and genome size, species diversity and evolutionary rates of their transformation. KEY RESULTS: The measurements of DNA C-values for 233 species more than doubled the taxon coverage from ~2.2 % in previous studies to 5.3 % of extant diversity. The dataset not only documented substantial differences in the accumulation of genomic diversity and disparity among the major lineages of ferns but also supported the predicted correlation between species diversity and the dynamics of genome evolution. CONCLUSIONS: Our results demonstrated substantial genome disparity among different groups of ferns and supported the prediction that alterations of reproductive modes alter trends of genome evolution. Finally, we recovered evidence for a close link between the dynamics of genome evolution and species diversity in ferns for the first time.

Impact of N-acetylcysteine (NAC) and calcium hydroxide intracanal medications in primary endodontic infection: a randomized clinical trial.

OBJECTIVES: This RCT investigated the impact of N-acetylcysteine (NAC) and calcium hydroxide [Ca(OH)2] intracanal medications (ICMs) in primary endodontic infection with apical periodontitis (PEIAP). MATERIALS AND METHODS: Thirty-six teeth with PEIAP were randomly divided into groups according to the ICM: NAC, Ca(OH)2 + saline solution (SSL), and Ca(OH)2 + 2% chlorhexidine-gel (2% CHX-gel) (all, n = 12). Root canal samples (RCSs) were collected before (s1) and after instrumentation (s2) and after 14 days of ICM (s3). Chemomechanical preparation (CMP) was performed with a Reciproc file and 2.5% NaOCl. Checkerboard DNA-DNA hybridization was used to assess 40 target bacteria species. RESULTS: At s1, bacterial DNA was detected in 100% of RCSs (36/36). All 40 bacterial species were found in PEIAP. The mean number of species per RCS was 17.92 ± 13.18. The most frequent bacteria were S. mitis (65%), E. nodatum (63%), E. faecalis (63%), F. nucl sp vicentii (58%), T. forsythia (58%), and F. periodonticum (56%). CMP reduced the mean number of species per RCS to 6.8 ± 2.36 (p < 0.05). At s3, the intragroup analysis revealed a broader antimicrobial activity for Ca (OH)2 + 2% CHX-gel and NAC than Ca(OH)2 + SSL (p < 0.05). NAC eliminated 8/12 bacteria species resistant to both Ca (OH)2 ICMs, including P. micra, P. nigrescens, T. denticola, A. israelii, P. endodontalis, P. acnes, C. ochracea, and E. corrodens. CONCLUSIONS: Ca (OH)2 + 2% chlorhexidine gel (2% CHX gel) showed a greater bacterial elimination over the number of bacterial species; however, NAC eliminated 8/12 bacteria species resistant to both Ca (OH)2 ICMs (RBR-3xbnnn). CLINICAL RELEVANCE: The use of intracanal medication with a broad antimicrobial activity can optimize root canal disinfection. Ca(OH)2 + 2% CHX gel and NAC showed a broader antimicrobial activity than Ca(OH)2 + SSL against endodontic pathogens in primary root canal infection. TRIAL REGISTRATION: Brazilian Registry of Clinical Trials (REBEC), No. RBR-3xbnnn.

MicroBooNE and the ν_{e} Interpretation of the MiniBooNE Low-Energy Excess.

A new generation of neutrino experiments is testing the 4.7σ anomalous excess of electronlike events observed in MiniBooNE. This is of huge importance for particle physics, astrophysics, and cosmology, not only because of the potential discovery of physics beyond the standard model, but also because the lessons we will learn about neutrino-nucleus interactions will be crucial for the worldwide neutrino program. MicroBooNE has recently released results that appear to disfavor several explanations of the MiniBooNE anomaly. Here, we show quantitatively that MicroBooNE results, while a promising start, unquestionably do not probe the full parameter space of sterile neutrino models hinted at by MiniBooNE and other data, nor do they probe the ν_{e} interpretation of the MiniBooNE excess in a model-independent way.

Can physiological stimulation prior to ileostomy closure reduce postoperative ileus? A prospective multicenter pilot study.

BACKGROUND: The aim of this study was to assess the impact of ileostomy closure following preoperative physiological stimulation (PPS) on postoperative ileus (POI) in patients with loop ileostomy after low anterior resection for rectal cancer. METHODS: Patients who underwent ileostomy closure between January 2017 and February 2020 in two tertiary referral centers were prospectively included. PPS stimulation was compared to standard treatment. Stimulation was carried out daily during the 15 days prior to ileostomy closure by the patient's self-instillation of 200 ml of fecal contents from the ileostomy bag via the efferent loop, using a rectal catheter. Standard treatment (ST) consisted of observation. Outcomes measures were POI, morbidity, stimulation feasibility, and predictors to ileus. RESULTS: A total of 58 patients were included [42 males and 16 females, median age 67 (43-85) years]. PPS was used in 24 patients, who completed the entire stimulation process, and ST in 34 patients. No differences in preoperative factors were found between the two groups. POI was significantly lower in the PPS group (4.2%) vs the ST group (32.4%); p < 0.01, OR: 0.05 (CI 95% 0.01-0.65). The PPS group had a shorter time to restoration of bowel function (1 day vs 3 days) p = 0.02 and a shorter time to tolerance of liquids (1 day vs 2 days), p = 0.04. Age (p = 0.01), open approach at index surgery, p = 0.03, adjuvant capecitabine (p = 0.01). and previous abdominal surgeries (p = 0.02) were associated with POI in the multivariate analysis. C-reactive-protein values on the 3rd (p = 0.02) and 5th (p < 0.01) postoperative day were also associated with POI. CONCLUSIONS: PPS for patients who underwent ileostomy closure after low anterior resection for rectal cancer is feasible and might reduce POI.

Attitudes towards risk-reducing early salpingectomy with delayed oophorectomy for ovarian cancer prevention: a cohort study.

OBJECTIVE: To determine risk-reducing early salpingectomy and delayed oophorectomy (RRESDO) acceptability and effect of surgical prevention on menopausal sequelae/satisfaction/regret in women at increased ovarian cancer (OC) risk. DESIGN: Multicentre, cohort, questionnaire study (IRSCTN:12310993). SETTING: United Kingdom (UK). POPULATION: UK women without OC ≥18 years, at increased OC risk, with/without previous RRSO, ascertained through specialist familial cancer/genetic clinics and BRCA support groups. METHODS: Participants completed a 39-item questionnaire. Baseline characteristics were described using descriptive statistics. Logistic/linear regression models analysed the impact of variables on RRESDO acceptability and health outcomes. MAIN OUTCOMES: RRESDO acceptability, menopausal sequelae, satisfaction/regret. RESULTS: In all, 346 of 683 participants underwent risk-reducing salpingo-oophorectomy (RRSO). Of premenopausal women who had not undergone RRSO, 69.1% (181/262) found it acceptable to participate in a research study offering RRESDO. Premenopausal women concerned about sexual dysfunction were more likely to find RRESDO acceptable (odds ratio [OR] = 2.9, 95% CI 1.2-7.7, P = 0.025). Women experiencing sexual dysfunction after premenopausal RRSO were more likely to find RRESDO acceptable in retrospect (OR = 5.3, 95% CI 1.2-27.5, P < 0.031). In all, 88.8% (143/161) premenopausal and 95.2% (80/84) postmenopausal women who underwent RRSO, respectively, were satisfied with their decision, whereas 9.4% (15/160) premenopausal and 1.2% (1/81) postmenopausal women who underwent RRSO regretted their decision. HRT uptake in premenopausal individuals without breast cancer (BC) was 74.1% (80/108). HRT use did not significantly affect satisfaction/regret levels but did reduce symptoms of vaginal dryness (OR = 0.4, 95% CI 0.2-0.9, P = 0.025). CONCLUSION: Data show high RRESDO acceptability, particularly in women concerned about sexual dysfunction. Although RRSO satisfaction remains high, regret rates are much higher for premenopausal women than for postmenopausal women. HRT use following premenopausal RRSO does not increase satisfaction but does reduce vaginal dryness. TWEETABLE ABSTRACT: RRESDO has high acceptability among premenopausal women at increased ovarian cancer risk, particularly those concerned about sexual dysfunction.

Use of pulmonary ultrasound to predict in-hospital mortality in patients with COVID-19 infection.

INTRODUCTION: Lung ultrasound (LUS) implementation in patients with COVID-19 can help to establish the degree of pulmonary involvement, evaluate treatment response and estimate in-hospital outcome. OBJECTIVE: To evaluate the application of a LUS protocol in patients with COVID-19 infection to predict in-hospital mortality. METHODS: The study was carried out from April 1 to August 1, 2020 in patients with COVID-19 infection admitted to the Intensive Care Unit. Lung evaluation was carried out by physicians trained in critical care ultrasonography. RESULTS: Most patients were males, median age was 56 years, and 59 % required mechanical ventilation. In-hospital mortality was 39.4 %, and in those with a LUS score ≥ 19, mortality was higher (50 %). The multiple logistic regression model showed that a LUS score ≥ 19 was significantly associated with mortality (hazard ratio = 2.55, p = 0.01). CONCLUSIONS: LUS is a safe and fast clinical tool that can be applied at bedside in patients with COVID-19 infection to establish the degree of parenchymal involvement and predict mortality.

INTRODUCCIÓN: La implementación del ultrasonido pulmonar (LUS) en los pacientes con COVID-19 puede ayudar a establecer el grado de afectación pulmonar, evaluar la respuesta al tratamiento y estimar el desenlace intrahospitalario. OBJETIVO: Evaluar la aplicación de un protocolo LUS en pacientes con infección por COVID-19 para predecir mortalidad intrahospitalaria. MÉTODOS: El estudio se realizó del 1 de abril al 1 de agosto de 2020 en pacientes con infección por COVID-19, ingresados en la Unidad de Terapia Intensiva. Se realizó evaluación pulmonar por médicos entrenados en ultrasonografía crítica. RESULTADOS: La mayoría de los pacientes fue del sexo masculino, la edad mediana fue de 56 años y 59 % requirió ventilación mecánica. La mortalidad intrahospitalaria fue de 39.4 % y en aquellos con puntuación de LUS ≥ 19, de 50 %. El modelo de regresión logística múltiple mostró que la puntuación de LUS ≥ 19 se asoció significativamente a mortalidad (cociente de riesgo = 2.55, p = 0.01). CONCLUSIONES: El LUS es una herramienta clínica segura y rápida que puede realizarse al lado de la cama de los pacientes con infección por COVID-19, para establecer el grado de afectación parenquimatosa y predecir la mortalidad.

Psychological impact of multigene cancer panel testing in patients with a clinical suspicion of hereditary cancer across Spain.

OBJECTIVE: Patients' psychological reactions to multigene cancer panel testing might differ compared with the single-gene testing reactions because of the complexity and uncertainty associated with the different possible results. Understanding patients' preferences and psychological impact of multigene panel testing is important to adapt the genetic counselling model. METHODS: One hundred eighty-seven unrelated patients with clinical suspicion of hereditary cancer undergoing a 25-gene panel test completed questionnaires after pretest genetic counselling and at 1 week, 3 months, and 12 months after results to elicit their preferences regarding results disclosure and to measure their cancer worry and testing-specific distress and uncertainty. RESULTS: A pathogenic variant was identified in 38 patients (34 high penetrance and 4 moderate penetrance variants), and 54 patients had at least one variant of uncertain significance. Overall, cancer panel testing was not associated with an increase in cancer worry after results disclosure (P value = .87). Twelve months after results, carriers of a moderate penetrance variant had higher distress and uncertainty scores compared with carriers of high penetrance variants. Cancer worry prior to genetic testing predicted genetic testing specific distress after results, especially at long term (P value <.001). Most of the patients reported the wish to know all genetic results. CONCLUSIONS: Our results suggest that patients can psychologically cope with cancer panel testing, but distress and uncertainty observed in carriers of moderate penetrance cancer variants in this cohort warrant further research.

Distribution of Ophioglossum reticulatum L. in South America. A case of long-distance jump dispersal?

The objective of this exploratory work is to test the hypothesis that South American populations of Ophioglossum reticulatum L. derive from Africa. Spores cross the Atlantic transported by wind and arrive in South America in recurrent migration. Three-dimensional (backward and forward) trajectories of spores between Africa and South America were calculated using the Hybrid Single Particle Lagrangian Integrated Trajectory Model (HYSPLIT4). The model showed possible backward trajectories in the low troposphere arriving in South America with probable origin in Northwestern Africa. The results support the hypothesis of long-distance dispersal of the studied species. Including vertical motion in the model runs allowed obtaining valuable and novel information about the migration routes. The trade winds combined with the South American monsoon could be a dispersal vehicle for the disseminules from Northwestern Africa to the eastern slopes of the Andes. As the monsoon is a periodic regional atmospheric circulation pattern, transcontinental migration can be assumed to be a recurring phenomenon that provides genetic exchange and prevents speciation by reproductive isolation. Modelled forward trajectories connect the neotropics with Africa-Madagascar, but they seem to be less effective due to their travelling altitudes. This hypothesis might explain the absence of infraspecific taxa restricted to different geographic locations.

Osteoporotic vertebral fractures: Natural history and impact. / Fracturas vertebrales osteoporóticas: historia natural e impacto.

Osteoporosis, and the consequences derived from it, such as fragility fractures, constitute a growing public health problem. Suffering from a fracture of this nature is the main risk factor for suffering a new fracture. It is documented that vertebral compression fractures lead to significant morbidity and mortality, in the short and long term, as well as other complications, such as sagittal imbalance and hyperkyphosis of the segment. However, we have not found documentation that analyzes the medium and long-term consequences of these injuries, assessing the type of treatment used, and the economic impact they represent. The purpose of this review is to analyze the main recent literature on the subject and make a breakdown of the consequences of these fractures in various spheres, such as economic, quality of life, sagittal balance and radiographic parameters, pain or mortality; as well as a brief analysis of epidemiology and natural history. CONCLUSION: Osteoporotic fractures constitute an emerging problem, both in the medical and economic fields. The consequences and sequelae on the patient are multiple and although surgical options offer good long-term results, it is necessary to properly select the patient, through multidisciplinary teams, to try to minimize potential complications.

Prospective, multi-centric, international, single-arm, cohort study to assess a synthetic polyamide suture material in oral surgery to close the mucosa - MUCODA study.

OBJECTIVES: The aim was to collect different clinical parameters systematically and proactively regarding safety, effectiveness, and performance of a nylon monofilament suture under routine clinical practice for oral surgery. METHODS: The study design was prospective, bicentric, international, single-armed, and observational. A non-absorbable suture was applied to close the mucosa after different dental surgical interventions. Main objective was the incidence of combined postoperative complications until suture removal. The 95 % Confidence Interval (Agresti-Coull method) was used to prove the non-inferiority with a pre-specified upper margin of 21.9 %. Secondary variables were intraoperative suture handling, patient pain and satisfaction, wound healing, aesthetic appearance, and bacterial contamination. RESULTS: 105 patients were enrolled. Complication rate was low (1.9 %), 2 swellings occurred. Pain was present for 1.61 days ± 1.42 after various dental interventions with an average pain level of 20.98 ± 22.60 (VAS). Patients with impacted third molar extraction showed the longest pain duration (6 days) combined with the highest mean pain level of 35.33 ± 30.45 (VAS). Intraoperative suture handling was very good to excellent. Suture removal was done after an average duration of 7.56 ± 2.09 days. Patient's satisfaction was high, and an excellent wound healing was reported by the dentists. Aesthetic appearance only performed in implant patients was rated by oral surgeons with an average of 96.19 ± 3.79 points [min. 80 - max. 100] at 5 months postoperatively. Thread bacterial analysis showed that F. nucleatum was the most present species. CONCLUSIONS: Our findings indicate that the non-absorbable, nylon-based monofilament suture used is safe and quite suitable for oral mucosal closure after various dental surgical interventions such as tooth extraction, implant placement and impacted third molar extraction. CLINICAL SIGNIFICANCE: This study showed the safe use of a non-absorbable, nylon-based monofilament suture for different oral surgical interventions under daily routine clinical practice.

The Anti-SARS-CoV-2 IgG1 and IgG3 Antibody Isotypes with Limited Neutralizing Capacity against Omicron Elicited in a Latin Population a Switch toward IgG4 after Multiple Doses with the mRNA Pfizer-BioNTech Vaccine.

The aim of this study was to analyze the profiles of IgG subclasses in COVID-19 convalescent Puerto Rican subjects and compare these profiles with those of non-infected immunocompetent or immunocompromised subjects that received two or more doses of an mRNA vaccine. The most notable findings from this study are as follows: (1) Convalescent subjects that were not hospitalized developed high and long-lasting antibody responses. (2) Both IgG1 and IgG3 subclasses were more prevalent in the SARS-CoV-2-infected population, whereas IgG1 was more prevalent after vaccination. (3) Individuals that were infected and then later received two doses of an mRNA vaccine exhibited a more robust neutralizing capacity against Omicron than those that were never infected and received two doses of an mRNA vaccine. (4) A class switch toward the "anti-inflammatory" antibody isotype IgG4 was induced a few weeks after the third dose, which peaked abruptly and remained at high levels for a long period. Moreover, the high levels of IgG4 were concurrent with high neutralizing percentages against various VOCs including Omicron. (5) Subjects with IBD also produced IgG4 antibodies after the third dose, although these antibody levels had a limited effect on the neutralizing capacity. Knowing that the mRNA vaccines do not prevent infections, the Omicron subvariants have been shown to be less pathogenic, and IgG4 levels have been associated with immunotolerance and numerous negative effects, the recommendations for the successive administration of booster vaccinations to people should be revised.

Therapeutic intervention with virtual reality in patients with Parkinson's disease for upper limb motor training: A systematic review.

To analyze the scientific evidence regarding changes in upper extremities in patients with Parkinson's disease who use virtual reality as part of their neurological rehabilitation treatment. The search was carried out in the following databases: MEDLINE, Cochrane Library, PEDROs and SCOPUS. The following inclusion criteria were applied to a total of 106 articles: Randomized clinical trials with an age of 5 years, the sample had to be of patients with PD (regardless of their state of involvement) and who had performed RV in the treatment of limbs superiors. A total of n=7 articles were used, in which the variables corresponding to upper limb motor control were measured, such as: fine motor dexterity, gross motor dexterity, strength, tremor, functionality and speed. Virtual Reality as tool in neurorehabilitation in patients with Parkinson's disease shows positive effects in all measurements related to upper limb motor control.

On the causes and consequences of the free-roaming dog problem in southern Chile.

Free-roaming dogs are an important concern for public health, livestock production and the environment. Human behaviors-such as allowing pets to roam, abandoning dogs, or feeding stray animals-could influence free-roaming dog abundance and the frequency of occurrence of dog-caused problems. Here we aim to determine patterns of free-roaming dog abundance in urban and rural areas, to reveal spatial variation in human behaviors underlying the free-roaming dog problem, and to test for associations between free-roaming dog abundance and related problems. We conducted our study in Chile, where dogs are a major environmental issue. In Chile, as in many other Global South countries, many people leave their dogs to roam, partly due to norms and to lax enforcement of dog control laws. To address our objectives, we counted dogs in 213 transects in urban and rural areas to model dog abundance using N-mixture models. Then we conducted interviews in 553 properties around the transects to determine people's dog management, their behavior towards free-roaming dogs and the prevalence of dog-caused problems. Dog abundance was higher in transects where a higher number of owned dogs was allowed to roam, as well as in lower-income neighborhoods (based on property tax valuation). Meanwhile, rural citizens were more likely to let their dogs' roam. Dog abandonment was reported more frequently in lower-income urban neighborhoods and rural areas. Not surprisingly, we found that several problems-such as dog bites-were more frequent where we detected more free-roaming dogs. Our results highlight that the owned dog population is a central component of the free-roaming dog problem, and that human behavior is the key driver underlying the problem. Dog management programs should promote responsible dog-ownership, with a strong message focused on keeping dogs inside properties and preventing abandonment.

[Translated article] The role of minimally invasive spine surgery in the treatment of vertebral metastasis: A narrative review.

BACKGROUND: Spinal metastases are a very common problem which dramatically affects the quality of life of cancer patients. The objective of this review is to address the issue of how minimally invasive surgery can play an important role in treating this pathology. METHODS: A literature review was performed, searching in the Google Scholar, PubMed, Scopus and Cochrane databases. Relevant and quality papers published within the last 10 years were included in the review. RESULTS: After screening the 2184 initially identified registers, a total of 24 articles were included for review. CONCLUSION: Minimally invasive spine surgery is specially convenient for fragile cancer patients with spinal metastases, because of its reduced comorbidity compared to conventional open surgery. Technological advances in surgery, such as navigation and robotics, improve accuracy and safety in this technique.

[Translated article] The role of minimally invasive spine surgery in the treatment of vertebral metastasis (Part 1): A clinical review.

Spinal metastases represent a significant burden on the quality of life in patients affected by active oncological disease due to the high incidence of pain syndromes, spinal deformity, and neurological impairment. Surgery plays a decisive role in improving quality of life by controlling pain, restoring neurological function and maintaining spinal stability, as well as contributing to the response to medical therapy. Minimally invasive surgery (MIS) is a treatment option in certain patients with high surgical risk since it has a low rate of complications, intraoperative bleeding, hospital stay, and offers similar results to open surgery. In this review, we present the role of MIS in this pathology and some cases treated in our hospital.

The role of minimally invasive spine surgery in the treatment of vertebral metastasis (part 1): A clinical review. / El papel de la cirugía mínimamente invasiva de columna en el tratamiento de las metástasis vertebrales (parte 1): una revisión clínica.

Spinal metastases represent a significant burden on the quality of life in patients affected by active oncological disease due to the high incidence of pain syndromes, spinal deformity, and neurological impairment. Surgery plays a decisive role in improving quality of life by controlling pain, restoring neurological function and maintaining spinal stability, as well as contributing to the response to medical therapy. Minimally invasive surgery (MIS) is a treatment option in certain patients with high surgical risk since it has a low rate of complications, intraoperative bleeding, hospital stay, and offers similar results to open surgery. In this review, we present the role of MIS in this pathology and some cases treated in our hospital.

The role of minimally invasive spine surgery in the treatment of vertebral metastasis: A narrative review. / El papel de la cirugía mínimamente invasiva de columna en el tratamiento de las metástasis vertebrales: una revisión narrativa.

BACKGROUND: Spinal metastases are a very common problem which dramatically affects the quality of life of cancer patients. The objective of this review is to address the issue of how minimally invasive surgery can play an important role in treating this pathology. METHODS: A literature review was performed, searching in the Google Scholar, PubMed, Scopus and Cochrane databases. Relevant and quality papers published within the last 10 years were included in the review. RESULTS: After screening the 2184 initially identified registers, a total of 24 articles were included for review. CONCLUSION: Minimally invasive spine surgery is specially convenient for fragile cancer patients with spinal metastases, because of its reduced comorbidity compared to conventional open surgery. Technological advances in surgery, such as navigation and robotics, improve accuracy and safety in this technique.

Widespread Presence of Domestic Dogs on Sandy Beaches of Southern Chile.

Dogs on sandy beaches are a threat to shorebirds. Managing this problem requires understanding the factors that influence the abundance of dogs in these ecosystems. We aimed to determine the proportion of beaches used by dogs and the effects of human presence on dog abundance on sandy beaches of southern Chile. We conducted dog counts and recorded the presence of tracks on 14 beaches. We used zero-inflated generalized linear mixed models to determine if the number of people, number of households, and other covariates were associated with dog abundance. We detected dog tracks on all the beaches, and dog sightings on most of them. Dogs were frequently not supervised (45%) and only 13% of them were leashed. The number of people on the beach and the number of houses near the beach were positively associated with the number of dogs on beaches. Finally, when dogs co-occurred with whimbrels (Numenius phaeopus), the probability of dog harassment was high (59%). Our work reveals that human presence determines the abundance of dogs on sandy beaches. Therefore, our study suggests that any strategy aiming at reducing dog harassment of shorebirds requires changes in those human behaviors that favor the presence of free-ranging dogs at beaches.

[Idursulfase desensitization in a child with Hunter syndrome (mucopolysaccharidosis II)]. / Desensibilización con idursulfase en un niño con síndrome de Hunter (mucopolisacaridosis II).

Enzyme replacement therapy with idursulfase decreases morbidity and improves quality of life of patients with mucopolysaccharidosis ii. Immediate hypersensitivity reactions to this drug have been described. Desensitization is a treatment that induces temporary tolerance to a culprit drug, allowing the allergic patient to receive the medication. We present the case of a 7-year-old patient diagnosed with Hunter syndrome who presented, after 4 years of treatment, two episodes of anaphylaxis during the infusion of idursulfase. Detection of specific immunoglobulin E was carried out using skin tests, with intradermal reaction at a 1/10 dilution (0.2 mg/ml) being positive. A 12-step desensitization protocol was performed without presenting adverse events. The allergological evaluation and the possibility of desensitization were useful tools in the management of our patient.

La terapia de reemplazo enzimático disminuye la morbilidad y mejora la calidad de vida de los pacientes con mucopolisacaridosis ii. Se han descrito reacciones de hipersensibilidad inmediata a este fármaco. La desensibilización es un tratamiento que induce la tolerancia temporaria a una droga y permite al paciente alérgico recibir la medicación. Se presenta el caso de un niño de 7 años con diagnóstico de síndrome de Hunter que, luego de 4 años de tratamiento con idursulfase, tuvo dos episodios de anafilaxia durante la infusión del fármaco. Se detectó inmunoglubulina E específica mediante pruebas cutáneas, y fue positiva la intradermorreacción con dilución 1/10 (0,2 mg/ml). Se realizó un protocolo de desensibilización de 12 pasos, sin presentar eventos adversos. La evaluación alergológica y la posibilidad de desensibilización constituyeron herramientas útiles en el manejo de nuestro paciente.