Beta-ketothiolase deficiency: A case with unusual presentation of nonketotic hypoglycemic episodes due to coexistent probable secondary carnitine deficiency.

Beta-ketothiolase (T2, mitochondrial acetoacetyl-CoA thiolase) deficiency is an autosomal recessive disorder of isoleucine catabolism and ketone body metabolism that is characterized by increased urinary excretion of 2-methylacetoacetate, 2-methyl-3-hydroxybutyrate, and tiglylglycine. Most patients with T2 deficiency develop their first severe ketoacidotic events between 5 and 24 months of age. We encountered a case of T2 deficiency who developed the first hypoglycemic crisis without ketosis during her neonatal period and repeated such nonketotic hypoglycemic crisis during her infancy and early childhood. This is a very atypical clinical phenotype in T2 deficiency. We finally realized that she also has severe carnitine deficiency which might suppress beta-oxidation resulting in nonketotic hypoglycemia. After carnitine supplementation, she actually developed episodes with ketonuria. Her carnitine deficiency was probably a secondary deficiency which is rare in T2 deficiency but if present, may modify the clinical manifestation of T2 deficiency from ketoacidotic events to hypoketotic hypoglycemic events.

Changes in the anti-HBc profile of infants born to HBV infected mothers from Iran.

From 2007 to 2014, 328 infants born to hepatitis B surface antigen (HBsAg) positive mothers, who received passive-active immunization against hepatitis B at birth were tested for HBsAg, antibody to hepatitis B core antigen (anti-HBc) and antibody to hepatitis B surface antigen (anti-HBs) at 12-15months of age. Thirteen (4%) cases were HBsAg positive. Forty-four infants who were anti-HBs, anti-HBc positive (group 1) and twenty-one infants who were anti-HBc positive alone (group 2) were identified. Both groups were followed-up annually for testing anti-HBs and anti-HBc to verify if anti-HBc was of maternal origin. In group 1, anti-HBc disappeared in 41 cases at month 24, and it disappeared from the remaining 3 cases at month 36. In group 2, anti-HBc disappeared in 18 cases at month 24 and in the remaining 3 cases at month 36. The results show that maternal anti-HBc may persist up to 3years in some children.

Effect of surfactant type on platinum nanoparticle size of composite Pt/α-Al2O3 catalysts synthesized by a microemulsion method.

A facile method for the generation of platinum-on-alumina hybrid materials with high-surface area is presented, employing a microemulsion-based synthesis of metal nanoparticles. Pt nanoparticles (NPs) supported on α-Al2O3 were prepared by the reduction of metal ions in water-in-oil microemulsion systems stabilized by a range of different surfactants with cationic, anionic and nonionic headgroups, namely AOT, CTAB, Tween80 and TX-100. The synthesized materials were characterized using Fourier transform infrared spectroscopy (FT-IR), X-ray diffraction (XRD) and scanning electron microscopy (SEM). It is demonstrated that choice of surfactant can be used to tailor the size of the generated Pt nanoparticles, and seen that surfactant charge has a determining role in this process. Pt NPs formed in microemulsion systems based on charged surfactants (AOT and CTAB) are smaller than those prepared in nonionic microemulsion systems (TX-100 and Tween80). A solvent-induced demixing process was used to cleanly obtain the hybrid materials from the reaction medium at low energy cost.