ABSTRACT. Human carnitine palmitoyl transferase (CTP) deficiency results in two different clinical variants, one with "hepatic" and one with "muscular" symptoms. We studied CPT activity and long-chain fatty acid oxidation in fibroblast cell lines from four patients, two from each group. Overall CPT activity was deficient in patients' fibroblasts with the hepatic presentation, as previously demonstrated in patients' fibroblasts with the muscular presentation. The hepatic patients' fibroblasts displayed a CPTl deficiency which resulted in impaired long-chain fatty acid oxidation. In contrast, CPTl activity and palmitate oxidation were normal in muscular patients' fibroblasts. In these latter patients, the mutation presumably involved CPT2 activity. These data suggest that CPT deficiency is due to at least two different mutations, resulting in two distinct patterns of clinical and biochemical abnormalities. (Pediatr Res 24: 308-31 1, 1988)

Objectives To assess the appropriateness of empirical antimicrobial therapy for sepsis and septic shock and determine factors associated with patient treatment outcomes at a Vietnamese national hospital. Methods A cross-sectional study was conducted on 134 patients diagnosed with sepsis and/or septic shock at Thong-Nhat Hospital, Ho Chi Minh City, Vietnam, from January 2018 to June 2018. Appropriateness of antimicrobial therapy was defined as physician adherence to antimicrobial guidelines using the Sanford Guide to Antimicrobial Therapy and the Vietnam national guidelines. Bayesian model averaging technique was used to identify the related factors associated with patient treatment outcomes. Results The median age of patients was 70 years. Organisms were identified in 54.5% of cases and predominated by Escherichia coli and staphylococci. Appropriate empirical antimicrobial agents were initiated in 56.6% (n = 73) of all cases. Of these patients, 31 cases (42.5%) and 61 cases (83.6%) received the antimicrobials in accordance with recommendations related to dosage and route of administration, respectively, bringing the overall rate of appropriate empirical antimicrobial therapy down to 23.3%. Patients who progressed to septic shock, received inappropriate antimicrobial therapy and required ICU admission were more likely to suffer treatment failure. Conclusions The study findings suggest that clinicians should appropriately adhere to antimicrobial guidelines, especially in patients with septic shock and those who require ICU care, to improve treatment outcomes.