HIBCH deficiency in a patient with phenotypic characteristics of mitochondrial disorders.

HIBCH (3-hydroxyisobutyryl-CoA hydrolase) deficiency (MIM #250620) is a rare autosomal recessive inborn error of metabolism, leading to a block in the catabolic pathway of the amino acid valine and presumably to accumulation of toxic valine metabolites in mitochondria. Only three families with HIBCH deficiency and biallelic HIBCH mutations have been described. We report on a further patient, first child of healthy consanguineous parents, with severe developmental delay, seizures, hyperintensities of the basal ganglia on magnetic resonance imaging (MRI), progressive brain atrophy, optic nerve atrophy, repeatedly elevated blood lactate, and respiratory chain complexes I, I + III and cytochrome c oxidase deficiencies with borderline depletion of mitochondrial DNA in muscle tissue.

Prospective assessment of complications associated with ultrasound activated resorbable pin osteosynthesis in pediatric craniofacial surgery: preliminary results.

Ultrasound activated resorbable pin osteosynthesis (UARPO) has recently shown favourable results in operations on children suffering for craniosynostosis. However, data on complications coming with this new technique in children suffering from craniosynostoses are scarce and have only been assessed retrospectively so far. It has been the aim of the present study to prospectively follow up children undergoing craniosynostosis surgery with a focus on complications related to UARPO materials.

Drug utilisation on a preterm and neonatal intensive care unit in Germany: a prospective, cohort-based analysis.

Purpose: This study aims to describe the drug use on a Neonatal Intensive Care Unit (NICU) at a University Children's Hospital in Germany, to investigate the licensing status of the drugs used and to conclude critical areas in neonatal intensive care to support prioritisation of future research.

Methods: An 11-month, prospective cohort study was conducted on the NICU at the University Children's Hospital Erlangen, Germany. All products prescribed during the study period were analysed whether or not the SPC contains information on term and preterm neonates.

Reintegrating seriously violent and personality-disordered offenders from a supermaximum security institution into the general offender population.

Offenders who have committed serious violent acts while incarcerated are often segregated and housed in supermaximum security facilities. Given the highly restricted regime under which they are detained, it is often difficult to decide if they are safe enough to be discharged. However, there is a need to reintegrate them into the general offender population in a lower security institution for humane, correctional, and financial reasons.