Case report: desensitization of hypersensitivity against the antisense oligonucleotide volanesorsen

Familial chylomicronemia syndrome (FCS) is a rare autosomal recessive metabolic disorder that causes extremely elevated plasma triglyceride levels, with limited therapeutic options. Volanesorsen is an antisense oligonucleotide approved for its treatment. A 24-year-old woman with genetically diagnosed FCS secondary to a pathogenic variant in APOA5 and a history of recurrent hypertriglyceridemia-induced pancreatitis episodes was being treated with volanesorsen, 285 mg every 2 weeks. Treatment with volanesorsen achieved normalization of triglycerides to <200 mg/dl. However, after the fifth dose of the medication, the patient developed urticaria and volanesorsen was discontinued. In the absence of alternative pharmacological treatments, the patient received a novel desensitization protocol for volanesorsen that allowed continuation of therapy, without evidence of hypersensitivity reactions after subsequent administrations. FCS requires aggressive multimodal therapy and close follow-up. Volanesorsen has shown great efficacy, but a significant rate of discontinuation due to side effects has been observed. Here, the patient presented an immediate hypersensitivity reaction to volanesorsen, but the provision of a desensitization protocol was effective, facilitating continued treatment and impacting the survival and quality of life of the patient

Case report: desensitization of hypersensitivity against the antisense oligonucleotide volanesorsen

Familial chylomicronemia syndrome (FCS) is a rare autosomal recessive metabolic disorder that causes extremely elevated plasma triglyceride levels, with limited therapeutic options. Volanesorsen is an antisense oligonucleotide approved for its treatment. A 24-year-old woman with genetically diagnosed FCS secondary to a pathogenic variant in APOA5 and a history of recurrent hypertriglyceridemia-induced pancreatitis episodes was being treated with volanesorsen, 285 mg every 2 weeks. Treatment with volanesorsen achieved normalization of triglycerides to &lt;200 mg/dl. However, after the fifth dose of the medication, the patient developed urticaria and volanesorsen was discontinued. In the absence of alternative pharmacological treatments, the patient received a novel desensitization protocol for volanesorsen that allowed continuation of therapy, without evidence of hypersensitivity reactions after subsequent administrations. FCS requires aggressive multimodal therapy and close follow-up. Volanesorsen has shown great efficacy, but a significant rate of discontinuation due to side effects has been observed. Here, the patient presented an immediate hypersensitivity reaction to volanesorsen, but the provision of a desensitization protocol was effective, facilitating continued treatment and impacting the survival and quality of life of the patient

La situación de la mortalidad materna en Argentina

En esta hoja informativa se presenta un análisis del problema de las muertes maternas en la Argentina con el propósito de promover el debate social informado y facilitar la toma de decisiones sobre una plataforma común de información.Fil: Romero, Mariana. Consejo Nacional de Investigaciones Científicas y Técnicas; Argentina. Centro de Estudios de Estado y Sociedad; ArgentinaFil: Chapman, Evelina. Academia Nacional de Medicina de Buenos Aires. Instituto de Investigaciones Epidemiológicas; ArgentinaFil: Ramos, Silvina. Centro de Estudios de Estado y Sociedad; ArgentinaFil: Ábalos, Edgardo. Centro Rosarino de Estudios Perinatales; Argentin

La situación de la mortalidad materna en la Argentina

En esta hoja informativa se presenta un análisis del problema de las muertes maternas en la Argentina con el propósito de promover el debate social informado y facilitar la toma de decisiones sobre una plataforma común de informaciónÁrea de Salud, Economía y Socieda

Hyperoxemia and excess oxygen use in early acute respiratory distress syndrome : Insights from the LUNG SAFE study

Publisher Copyright: © 2020 The Author(s). Copyright: Copyright 2020 Elsevier B.V., All rights reserved.Background: Concerns exist regarding the prevalence and impact of unnecessary oxygen use in patients with acute respiratory distress syndrome (ARDS). We examined this issue in patients with ARDS enrolled in the Large observational study to UNderstand the Global impact of Severe Acute respiratory FailurE (LUNG SAFE) study. Methods: In this secondary analysis of the LUNG SAFE study, we wished to determine the prevalence and the outcomes associated with hyperoxemia on day 1, sustained hyperoxemia, and excessive oxygen use in patients with early ARDS. Patients who fulfilled criteria of ARDS on day 1 and day 2 of acute hypoxemic respiratory failure were categorized based on the presence of hyperoxemia (PaO2 > 100 mmHg) on day 1, sustained (i.e., present on day 1 and day 2) hyperoxemia, or excessive oxygen use (FIO2 ≥ 0.60 during hyperoxemia). Results: Of 2005 patients that met the inclusion criteria, 131 (6.5%) were hypoxemic (PaO2 < 55 mmHg), 607 (30%) had hyperoxemia on day 1, and 250 (12%) had sustained hyperoxemia. Excess FIO2 use occurred in 400 (66%) out of 607 patients with hyperoxemia. Excess FIO2 use decreased from day 1 to day 2 of ARDS, with most hyperoxemic patients on day 2 receiving relatively low FIO2. Multivariate analyses found no independent relationship between day 1 hyperoxemia, sustained hyperoxemia, or excess FIO2 use and adverse clinical outcomes. Mortality was 42% in patients with excess FIO2 use, compared to 39% in a propensity-matched sample of normoxemic (PaO2 55-100 mmHg) patients (P = 0.47). Conclusions: Hyperoxemia and excess oxygen use are both prevalent in early ARDS but are most often non-sustained. No relationship was found between hyperoxemia or excessive oxygen use and patient outcome in this cohort. Trial registration: LUNG-SAFE is registered with ClinicalTrials.gov, NCT02010073publishersversionPeer reviewe

Multidifferential study of identified charged hadron distributions in ZZ-tagged jets in proton-proton collisions at s=\sqrt{s}=13 TeV

Jet fragmentation functions are measured for the first time in proton-proton collisions for charged pions, kaons, and protons within jets recoiling against a $Z$ boson. The charged-hadron distributions are studied longitudinally and transversely to the jet direction for jets with transverse momentum 20 $< p_{\textrm{T}} < 100$ GeV and in the pseudorapidity range $2.5 < \eta < 4$. The data sample was collected with the LHCb experiment at a center-of-mass energy of 13 TeV, corresponding to an integrated luminosity of 1.64 fb$^{-1}$. Triple differential distributions as a function of the hadron longitudinal momentum fraction, hadron transverse momentum, and jet transverse momentum are also measured for the first time. This helps constrain transverse-momentum-dependent fragmentation functions. Differences in the shapes and magnitudes of the measured distributions for the different hadron species provide insights into the hadronization process for jets predominantly initiated by light quarks.Comment: All figures and tables, along with machine-readable versions and any supplementary material and additional information, are available at https://cern.ch/lhcbproject/Publications/p/LHCb-PAPER-2022-013.html (LHCb public pages

Desensitization in patients with hypersensitivity to haem arginate: A case report

Background: Porphyria comprises a group of metabolic disorders caused by the irregular activities of enzymes within the haem biosynthetic pathway. This disease can provoke a large variety of symptoms. Acute porphyria attacks need to be treated urgently to avoid prolonged illness and fatal complications. Haem arginate, a concentrated haem solution stabilized with arginine, is the only preparation available for treatment in Europe and South America. This report describes a safe desensitization protocol for patients who require such treatment and have haem arginate hypersensitivity. Case presentation: A 25-year-old female patient diagnosed with acute intermittent porphyria, who had an anaphylactic reaction while receiving haem arginate. The patient was treated with a desensitization protocol for patients with hypersensitivity to haem arginate. Conclusion: Porphyria is a disease that can significantly compromise a patient's quality of life. The desensitization protocol for patients with hypersensitivity to haem arginate is a safe and effective treatment option for patients with a history of haem arginate allergies, to whom it is not possible to administer haematin. Keywords: Porphyria, Hypersensitivity to haem arginate, Hemin arginat

Intervenciones destinadas a reducir la mortalidad materna

En esta hoja informativa se resumen las intervenciones orientadas a la prevención y el manejo de las principales causas de muertes maternas. Se focaliza, principalmente, en las intervenciones clínicas y no clínicas provistas por los prestadores de salud (como por ejemplo, intervenciones educativas hacia las mujeres y/o sus familias por parte del personal de la salud), y se mencionan algunas estrategias en la organización de los sistemas y servicios de salud necesarios para su implementación. Serán motivo de otra hoja informativa la compilación y presentación de la evidencia acerca de las mejores vías de entrega, de financiamiento, de gobernanza, y de implementación, así como las formas de monitoreo y evaluaciónÁrea de Salud, Economía y Socieda

Two case reports of desensitization in patients with hypersensitivity to iron

Abstract Background Iron deficiency anemia is a disease that can significantly compromise a patient’s quality of life. Desensitization is a safe and effective treatment option for iron-deficient anemic patients who require intravenous iron despite their hypersensitivity to iron. This report describes a safe desensitization protocol for patients with iron hypersensitivity who require iron treatment for their clinical improvement. Case presentation Two patients of 20 and 46-year-old diagnosed with secondary iron deficiency anemia hipermenorreas and a clinical history of fail treatment with oral iron, who presented a reaction of the anaphylactic type while they receive iron parenteral sucrose. Therefore, the patients were treated with the desensitization protocol applied for patients with hypersensitivity to iron. Conclusion Iron deficiency anemia is a disease that can significantly compromise the quality of life of patients. The desensitization protocol for patients with hypersensitivity to iron is a safe and effective treatment option for patients with a history of allergy to intravenous iron. This case report shows the usefulness to use the desensitization protocol for patients with hypersensitivity to iron