Propionic acidemia is a rare autosomal recessive disorder affecting the catabolism of branched-chain amino acids because of a genetic defect in PCC. Despite the improvements in medical treatment with protein restriction, sufficient caloric intake, supplementation of l-carnitine, and metronidazole, patients with the severe form of propionic acidemia have life-threatening metabolic acidosis, hyperammonemia, and cardiomyopathy, which results in serious neurologic sequelae and sometimes death. This study retrospectively reviewed three children with neonatal-onset propionic acidemia who received LDLT. Between November 2005 and December 2010, 148 children underwent LDLT, with an overall patient survival of 90.5%, in our center. Three patients were indicated for transplantation because of propionic acidemia. All recipients achieved a resolution of metabolic derangement and better quality of life with protein restriction and medication, although urine methylcitrate and serum propionylcarnitine levels did not decrease markedly. LT can reduce the magnitude of progressive cardiac/neurologic disability as a result of poor metabolic control. Further evaluation is therefore required to determine the long-term suitability of this treatment modality.

A 4-month-old boy, with late-onset argininosuccinate lyase (ASL) deficiency with hepatomegaly, was treated by protein restricted diet and arginine supplementation; he was followed for 3 years. Hepatomegaly and mild liver dysfunction persisted without significant hyperammonemia. He maintained normal psychomotor development to the age of 12 months, but, at 3 years of age, his developmental status is in the borderline normal range. Liver biopsy performed at 12 months of age demonstrated swollen and pale hepatocytes with abnormal glycogen deposition and mild periportal fibrosis. A subsequent liver biopsy at 3 years of age showed progressive liver fibrosis in the periportal and central areas, which extended into the liver lobule. These findings suggest that liver impairment in ASL deficiency may advance without significant hyperammonemia and underline the importance of repeated liver biopsy in this disorder, even when the plasma ammonia level is well controlled.

Fabrication and electrical characterization of high aspect ratio silicon field emitter arrays Fabrication and characterization of silicon field emitter arrays with focusing electrode by the chemical mechanical polishing processWe fabricated hafnium carbide ͑HfC͒ coated Si field emitter arrays ͑HfC FEAs͒ with an extraction-gate electrode to improve the emission characteristics of Si FEAs. Hafnium carbide thin film was deposited by inductively coupled plasma-assisted magnetron sputtering. The HfC film was characterized by x-ray photoelectron spectroscopy and x-ray diffraction measurement, and was found to be ͑111͒-oriented polycrystalline film. The HfC FEAs exhibited superior performance. An emission of more than 10 mA could be obtained from the 16 000 tip array, which is 20 times higher than that for Si FEAs. The operational voltage for emission of 1 A decreased from 61 to 45 V due to the HfC coating. The long-term emission characteristics were also measured. Si FEAs degraded rapidly even in an ultrahigh vacuum chamber. However, the emission degradation in the HfC FEAs was much slower. The number of active tips was counted using an electrostatic-lens projector, and the results revealed that the HfC FEAs had six times as many tips as the Si FEAs had.

A graphene-oxide-semiconductor (GOS) planar-type electron source was fabricated by direct synthesis of graphene on an oxide layer via low-pressure chemical vapor deposition. It achieved a maximum electron emission efficiency of 32.1% by suppressing the electron inelastic scattering within the topmost gate electrode using graphene electrode. In addition, a 100-mA/cm 2 electron emission current density was observed at 16.2-% electron emission efficiency. The electron energy spread was well fitted to Maxwell-Boltzmann distribution, which indicates that the emitted electrons are thermally equilibrium state within the electron source. The full-width at half-maximum energy spread of the emitted electrons was approximately 1.1 eV. The electron emission efficiency did not deteriorate after more

This note describes an a x . conipa,rator circuit \.vliich uses a Hall generator for nieasurement of d.c. magnetic fields. The accuracy is to I?/, between 1 80 and 12030 gauss.

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Citrin deficiency is an autosomal recessive disorder caused by mutations in the SLC25A13 gene. The disease can present with age‐dependent clinical manifestations: neonatal intrahepatic cholestasis by citrin deficiency (NICCD), failure to thrive, and dyslipidemia by citrin deficiency (FTTDCD), and adult‐onset type II citrullinemia (CTLN2). As a nationwide study to investigate the clinical manifestations, medical therapy, and long‐term outcome in Japanese patients with citrin deficiency, we collected clinical data of 222 patients diagnosed and/or treated at various different institutions between January 2000 and December 2019. In the entire cohort, 218 patients were alive while 4 patients (1 FTTDCD and 3 CTLN2) had died. All patients <20 years were alive. Patients with citrin deficiency had an increased risk for low weight and length at birth, and CTLN2 patients had an increased risk for growth impairment during adolescence. Liver transplantation has been performed in only 4 patients (1 NICCD, 3 CTLN2) with a good response thereafter. This study reports the diagnosis and clinical course in a large cohort of patients with citrin deficiency and suggests that early intervention including a low carbohydrate diet and MCT supplementation can be associated with improved clinical course and long‐term outcome.

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