Classic and Atypical Late Infantile Neuronal Ceroid Lipofuscinosis in Latin America: Clinical and Genetic Aspects, and Treatment Outcome with Cerliponase Alfa.

Introduction: Late infantile neuronal ceroid lipofuscinosis type 2 (CLN2), is a neurodegenerative autosomal recessive disease caused by gene variants with a spectrum of classic and atypical phenotypes. The aim of treatment is to slow functional decline as early as possible in an attempt to improve quality of life and survival. This study describes the clinical characteristics as well as the response to treatment with cerliponase alfa.

Knowledge regarding extracorporeal membrane oxygenation management among Brazilian pediatric intensivists: a cross-sectional survey.

Objective: To assess Brazilian pediatric intensivists' general knowledge of extracorporeal membrane oxygenation, including evidence for its use, the national funding model, indications, and complications.

Methods: This was a multicenter cross-sectional survey including 45 Brazilian pediatric intensive care units. A convenience sample of 654 intensivists was surveyed regarding their knowledge on managing patients on extracorporeal membrane oxygenation, its indications, complications, funding, and literature evidence.

Mental Health and Emotional Disorders During the COVID-19 Pandemics: Prevalence and Extent in PICU Staff.

Objectives: To assess the prevalence of burnout, anxiety and depression symptoms, and posttraumatic stress disorder (PTSD) in PICU workers in Brazil during the first peak of the COVID-19 pandemic. To compare the results of subgroups stratified by age, gender, professional category, health system, and previous mental health disorders.

Design: Multicenter, cross-sectional study using an electronic survey.

Validation of sedation scores in mechanically ventilated children admitted to a tertiary pediatric intensive care unit.

Objectives: Sedation scores are important tools for use in pediatric intensive care units. The Comfort-Behavior scale is a valid method for the assessment of children although it is considered an extensive scale. The motor activity assessment scale is validated for an adult population.

Year in review 2006: Critical Care--Paediatrics.

In 2006, paediatric intensive care-related subjects were discussed in a number of papers published in various journals, including Critical Care. Because they focused on the cardiovascular system and its support, we summarize them here. In particular, these papers highlighted the management of refractory septic shock, extracorporeal support, outcome markers in sepsis, and outcome after cardiac arrest.

Meningococcal disease and meningitis.

Objective: To review the literature relevant to diagnosis and management of meningococcal disease (MD).

Sources: Non-systematic review of medical literature through the MEDLINE database using the terms meningococcal, septic shock, diagnosis, and treatment. Articles were selected according to their relevance to the objective of the study and according to the authors' opinion.