Pancreatic involvement in patients with inborn errors of metabolism.

BACKGROUND: Repeated inflammation of the pancreas can cause pancreatitis or diabetes. It is well recognized that the organic acidemias may be complicated by pancreatitis but less recognized are other metabolic disorders in which pancreatitis can occur. This study shows that long-term follow-up of patients with various metabolic disorders in Korea revealed several with episodes of isolated pancreatitis or diabetes concomitantly with pancreatitis. RESULTS AND DISCUSSION: In this study, two patients with methylmalonic aciduria (MMA), two with propionic acidemia (PPA), one with fatty acid oxidation disorder (FAOD), and one with hyperornithinemia, gyrate atrophy, and juvenile onset diabetes mellitus (DM) were clinically followed for up to 10 - 21 years. Two Korean siblings with MMA showed recurrent pancreatitis from the age of 15 and 19, respectively. The frequency of admission due to pancreatitis was up to 11 times. One patient with MMA developed diabetes mellitus at the age of 20. The other patient with MMA developed recurrent pancreatitis at 4 years and diabetes at 8 years of age. One of the patients with PPA presented with diabetic ketoacidosis. The other PPA patient died of cardiac arrest at age 10. The patient with FAOD presented with pancreatitis at 10 years and died at the age of 15 years due to cardiac arrest. A 35-year-old woman with hyperornithinemia/gyrate atrophy was diagnosed with juvenile onset diabetes at the age of 7 years. No pancreatitis occurred during the follow-up period. CONCLUSIONS: We conclude that various metabolic disorders can trigger acute or chronic pancreatitis. Proper and prompt multidisciplinary management of metabolic derangement is crucial for preventing pancreatic damage. Further clinical and investigational studies are required to elucidate the pathogenesis of pancreatitis and diabetes mellitus in patients with inborn errors in metabolism.

Simultaneous detection of Streptococcus pneumoniae, S. mitis, and S. oralis by a novel multiplex PCR assay targeting the gyrB gene.

A multiplex PCR (mPCR) protocol was developed for simultaneous detection of the gyrB gene in Streptococcus pneumoniae, Streptococcus mitis, and Streptococcus oralis, and the specificity was evaluated using 141 coccus strains. Genomic DNAs purified from S. pneumoniae, S. mitis, and S. oralis strains were efficiently detected with size differences, whereas no PCR products were amplified from any of the reference strains tested. A pilot study of 47 human oral swab specimens was conducted in parallel, and the mPCR assay identified S. pneumoniae in 1 sample, S. mitis in 8 samples, and S. oralis in 2 samples, providing a powerful means for characterization at the level of species compared with traditional culture analysis. Our results suggest that the mPCR protocol presented here is a sensitive and promising tool for the rapid detection and discrimination of S. pneumoniae, S. mitis, and S. oralis from clinical specimens.

Development of micro-heaters with optimized temperature compensation design for gas sensors.

One of the key components of a chemical gas sensor is a MEMS micro-heater. Micro-heaters are used in both semiconductor gas sensors and NDIR gas sensors; however they each require different heat dissipation characteristics. For the semiconductor gas sensors, a uniform temperature is required over a wide area of the heater. On the other hand, for the NDIR gas sensor, the micro-heater needs high levels of infrared radiation in order to increase sensitivity. In this study, a novel design of a poly-Si micro-heater is proposed to improve the uniformity of heat dissipation on the heating plate. Temperature uniformity of the micro-heater is achieved by compensating for the variation in power consumption around the perimeter of the heater. With the power compensated design, the uniform heating area is increased by 2.5 times and the average temperature goes up by 40 °C. Therefore, this power compensated micro-heater design is suitable for a semiconductor gas sensor. Meanwhile, the poly-Si micro-heater without compensation shows a higher level of infrared radiation under equal power consumption conditions. This indicates that the micro-heater without compensation is more suitable for a NDIR gas sensor. Furthermore, the micro-heater shows a short response time of less than 20 ms, indicating a very high efficiency of pulse driving.