Recommendations on NMOSD treatment in Latin America: Utilizing consensus-based RAND/UCLA methodology.

Introduction: Neuromyelitis optica spectrum disorder (NMOSD) is a serious condition affecting people worldwide, including Latin America (LATAM). Healthcare disparities and economic limitations make effective treatment access challenging. It is crucial to consider the best practice therapeutic decision-making, including emerging long-term preventive therapies, to ensure patients in LATAM and elsewhere can effectively manage their disease all over the world.

Improvement of gastrointestinal symptoms in a significant proportion of male patients with classic Fabry disease treated with agalsidase beta: A Fabry Registry analysis stratified by phenotype.

Background: Fabry disease is an inherited disorder of glycolipid metabolism with progressive involvement of multiple organs, including the gastrointestinal tract, in classically affected male patients. Clinical presentations in males with later-onset Fabry phenotypes are more heterogeneous and largely dependent on the level of residual α-galactosidase A activity.

Methods: We assessed agalsidase beta treatment outcomes of gastrointestinal symptoms in adult males with classic or later-onset Fabry disease.

The clinical profiles of female patients with Fabry disease in Latin America: A Fabry Registry analysis of natural history data from 169 patients based on enzyme replacement therapy status.

Background: Fabry disease is an X-linked lysosomal storage disorder with heterogeneous clinical expression in female patients ranging from asymptomatic to severe clinical presentations as in classic males. We assessed clinical profiles and compared natural history data of female patients eventually initiated on enzyme replacement therapy ("ERT-recipients") with those remaining untreated ("ERT-naïve").

Methods: We analyzed Fabry Registry data from 93 ERT-recipients, collected prior to ERT initiation, and 76 ERT-naïve females with classic or unclassified phenotypes from four Latin American countries and evaluated Fabry symptoms, interventricular septum thickness, left ventricular posterior wall thickness, estimated glomerular filtration rate, and severe clinical events.

Improvement of Fabry Disease-Related Gastrointestinal Symptoms in a Significant Proportion of Female Patients Treated with Agalsidase Beta: Data from the Fabry Registry.

Fabry disease, an X-linked inherited lysosomal storage disorder, is caused by mutations in the gene encoding α-galactosidase, GLA. In patients with Fabry disease, glycosphingolipids accumulate in various cell types, triggering a range of cellular and tissue responses that result in a wide spectrum of organ involvement. Although variable, gastrointestinal symptoms are among the most common and significant early clinical manifestations; they tend to persist into adulthood if left untreated.

[Cost and outcome of hip's arthroplasty nosocomial infection. Case and control study].

Case-control study for evaluation of cost and outcome of nosocomial surgical site infection in primary hip arthroplasty during a period of 5 years. Mean hospitalization time in the orthopedic service was 54 days for cases, and 13 days for control (p<0.05).

[Malignant infarct of the middle cerebral artery in a patient with bacterial meningitis].

The mortality of acute bacterial meningitis (BM) has remained stable in the last decades in spite of the use of new antibiotics, probably due to vascular complications. We report a 68-year-old woman with BM who had a malignant infarction of left middle cerebral artery territory 72 hours after admission to the hospital. The patient experienced a bad evolution and died four days after admission.