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Aim: This study was aimed at characterising the prevalence, management and outcomes of paediatric severe sepsis and septic shock in tertiary paediatric intensive care units (PICUs) in Turkey. Methods: A point prevalence study was conducted on 5 days over the course of 1 year in 29 PICUs in Turkey. Outcomes included severe sepsis and septic shock point prevalence, therapies used, duration of PICU stay and mortality at day 28. Results: Of the 1757 children who were admitted to the PICU during the study period, 141 (8.0%) children met the consensus criteria for severe sepsis and 23 (1.3%) children met the criteria for septic shock. Paediatric severe sepsis and septic shock accounted for 8% and 1.3% of all PICU admissions, respectively. The median age of the patients was 2.6 years (interquartile range [IQR], 0.7–8.6 years). Enteral nutrition (79.3%) was preferred compared to parenteral nutrition (31.1%) for the first 3 days after PICU admission. A total of 39 patients died while in the PICU, for a 23.8% mortality rate, which did not vary by age. Conclusion: The mortality rate was similar to that in other studies. Hematologic‐immunologic comorbidity, parenteral nutrition and the use of vasoactive drugs were independently associated with mortality. [ABSTRACT FROM AUTHOR]

Mitochondrial 3‐hydroxy‐3‐methylglutaryl‐CoA synthase (mHS) deficiency is a very rare autosomal recessive inborn error of ketone body synthesis and presents with hypoketotic hypoglycemia, metabolic acidosis, lethargy, encephalopathy, and hepatomegaly with fatty liver precipitated by catabolic stress. We report acute presentation of two patients from unrelated two families with novel homozygous c.862C>T and c.725‐2A>C mutations, respectively, in HMGCS2 gene. Affected patients had severe hypoketotic hypoglycemia, lethargy, encephalopathy, severe metabolic and lactic acidosis and hepatomegaly after infections. Surprisingly, molecular screening of the second family showed more affected patients without clinical findings. These cases expand the clinic spectrum of this extremely rare disease. [ABSTRACT FROM AUTHOR]