RESUMO
Vanishing white matter disease (VWMD) is an under-diagnosed condition that affects the brains white matter at all ages, especially in the pediatric age group. It belongs to a clinically and genetically heterogeneous group of disorders, collectively known as eukaryotic initiation factor 2B-related disorders. The disorder has been described in different ethnic groups. Here, we describe a case of VWMD from Saudi Arabia.
RESUMO
OBJECTIVE: To develop pediatric guidelines for the use of citrate as a regional anticoagulant for continuous renal replacement therapy (CRRT) using a neonatal piglet model. DESIGN: Prospective observational study. SETTING: Animal laboratory in the research center of a tertiary-level children's hospital. SUBJECTS: Ten neonatal piglets. INTERVENTIONS AND MEASUREMENTS: Using a venovenous CRRT circuit and filter, we randomly altered the filter blood flow rate, replacement flow rate, and citrate flow rate over conventional pediatric ranges. Measured end points were prefilter serum ionized calcium and citrate levels. MAIN RESULTS: A prefilter serum citrate concentration of 6 mmol/L is required to maintain the prefilter ionized calcium < or =0.4 mmol/L. Using multiple regression analysis on collected data, we derived a formula to predict prefilter serum citrate for combinations of replacement flow rate, blood flow rate, and citrate flow rate. CONCLUSIONS: The available literature and our past experience indicate that a prefilter ionized calcium < or =0.4 mmol/L is required to anticoagulate a CRRT circuit; a prefilter serum citrate concentration of 6 mmol/L is required to achieve this. Our multiple regression analysis can be expressed graphically to allow easy calculation of the required citrate flow rate, given the knowledge of the replacement flow rate and blood flow rate. Our results provide the first guidelines for the use of citrate as a regional anticoagulant in a pediatric-size model of CRRT.