Strain and dynamic measurements using fiber optic sensors embedded into graphite/epoxy tubes

Graphite/epoxy tubes were fabricated with embedded optical fibers to evaluate the feasibility of monitoring strains with a fiber optic technique. Resistance strain gauges were attached to the tubes to measure strain at four locations along the tube for comparison with the fiber optic sensors. Both static and dynamic strain measurements were made with excellent agreement between the embedded fiber optic strain sensor and the strain gauges. Strain measurements of 10(exp -7) can be detected with the optical phase locked loop (OPLL) system using optical fiber. Because of their light weight, compatibility with composites, immunity to electromagnetic interference, and based on the static and dynamic results obtained, fiber optic sensors embedded in composites may be useful as the sensing component of smart structures

Successful domino liver transplantation in maple syrup urine disease using a related living donor

Maple syrup urine disease (MSUD) is an autosomal recessive disease associated with high levels of branched-chain amino acids. Children with MSUD can present severe neurological damage, but liver transplantation (LT) allows the patient to resume a normal diet and avoid further neurological damage. The use of living related donors has been controversial because parents are obligatory heterozygotes. We report a case of a 2-year-old child with MSUD who underwent a living donor LT. The donor was the patient’s mother, and his liver was then used as a domino graft. The postoperative course was uneventful in all three subjects. DNA analysis performed after the transplantation (sequencing of the coding regions of BCKDHA, BCKDHB, and DBT genes) showed that the MSUD patient was heterozygous for a pathogenic mutation in the BCKDHB gene. This mutation was not found in his mother, who is an obligatory carrier for MSUD according to the family history and, as expected, presented both normal clinical phenotype and levels of branched-chain amino acids. In conclusion, our data suggest that the use of a related donor in LT for MSUD was effective, and the liver of the MSUD patient was successfully used in domino transplantation. Routine donor genotyping may not be feasible, because the test is not widely available, and, most importantly, the disease is associated with both the presence of allelic and locus heterogeneity. Further studies with this population of patients are required to expand the use of related donors in MSUD

Behavioural Responses of Western Flower Thrips, Frankliniella occidentalis (Pergande), to Volatiles from Three Aromatic Plants

The behavioural responses of adult female western flower thrips, Frankliniella occidentalis (Pergande) to volatiles from meadow-sweet (Filipendula ulmaria), bay laurel (Laurus nobilis) and sage (Salvia officinalis) were investigated in laboratory bioassays. Volatiles collected by entrainment of a solvent extract of F. ulmaria were more attractive than was the original extract. Frankliniella occidentalis was also significantly attracted to volatiles from L. nobilis and S. officinalis. Analysis by gas chromatography and mass spectrometry identified 1,8-cineole (eucalyptol) as one of the main volatile components of all three plant species. In coupled gas chromatography-electroantennography studies with F. ulmaria, both 1,8-cineole and methyl salicylate elicited responses from F. occidentalis. Eucarvone was identified as the major component of F. ulmaria volatiles, but showed no electrophysiological activity. Behavioural responses of thrips to a range of concentrations of 1,8-cineole and methyl salicylate were tested using a modified Pettersson 'star' olfactometer. 1,8-cineole showed some attractant activity for the thrips at 0.01 mg, but methyl salicylate was repellent at all the concentrations tested. RÉSUMÉ La réponse comportementale de femelles adultes de thrips (Frankliniella occidentalis) aux émissions volatiles de trois plantes aromatiques, la filipendule (Filipendula ulmaria), le laurier-sauce (Laurus nobilis) et la sauge (Salvia officinalis), a été étudiée dans des essais au laboratoire. Les produits volatils obtenus par collecte d'effluves d'un extrait par solvant de F. ulmaria se sont montrés plus attractifs que l'extrait original. Frankliniella occidentalis a aussi été attiré significativement par les émissions volatiles de L. nobilis et S. officinalis. Des analyses par chromatographie en phase gazeuse et spectrométrie de masse ont permis d'identifier le 1,8-cineole (eucalyptol) comme l'un des composés volatils majeurs des trois espèces végétales. Dans des études par couplage chromatographie en phase gazeuse-électroantennographie, avec un extrait de F. ulmaria, le 1,8-cineole et le methyl salicylate ont tous deux induit des réponses chez F. occidentalis. L'eucarvone a été identifié comme le composé majoritaire des émissions volatiles de F. ulmaria, mais n'a induit aucune activité électrophysiologique. Les réponses comportementales des thrips à une gamme de concentrations de 1,8-cineole et de methyl salicylate ont été évaluées dans un olfactomètre de Pettersson modifé. Le 1,8-cineole a induit une certaine attraction chez les thrips à la concentration de 0,01 mg, mais le methyl salicylate a été répulsif à toutes les concentrations testées

Successful domino liver transplantation in maple syrup urine disease using a related living donor

Maple syrup urine disease (MSUD) is an autosomal recessive disease associated with high levels of branched-chain amino acids. Children with MSUD can present severe neurological damage, but liver transplantation (LT) allows the patient to resume a normal diet and avoid further neurological damage. The use of living related donors has been controversial because parents are obligatory heterozygotes. We report a case of a 2-year-old child with MSUD who underwent a living donor LT. The donor was the patient’s mother, and his liver was then used as a domino graft. The postoperative course was uneventful in all three subjects. DNA analysis performed after the transplantation (sequencing of the coding regions of BCKDHA, BCKDHB, and DBT genes) showed that the MSUD patient was heterozygous for a pathogenic mutation in the BCKDHB gene. This mutation was not found in his mother, who is an obligatory carrier for MSUD according to the family history and, as expected, presented both normal clinical phenotype and levels of branched-chain amino acids. In conclusion, our data suggest that the use of a related donor in LT for MSUD was effective, and the liver of the MSUD patient was successfully used in domino transplantation. Routine donor genotyping may not be feasible, because the test is not widely available, and, most importantly, the disease is associated with both the presence of allelic and locus heterogeneity. Further studies with this population of patients are required to expand the use of related donors in MSUD

The lysosomal storage disorders mucolipidosis type II, type III alpha/beta, and type III gamma : update on GNPTAB and GNPTG mutations

Mutations in the GNPTAB and GNPTG genes cause mucolipidosis (ML) type II, type III alpha/beta, and type III gamma, which are autosomal recessively inherited lysosomal storage disorders. GNPTAB and GNPTG encode the α/β‐precursor and the γ‐subunit of N‐acetylglucosamine (GlcNAc)‐1‐phosphotransferase, respectively, the key enzyme for the generation of mannose 6‐phosphate targeting signals on lysosomal enzymes. Defective GlcNAc‐1‐phosphotransferase results in missorting of lysosomal enzymes and accumulation of non‐degradable macromolecules in lysosomes, strongly impairing cellular function. MLII‐affected patients have coarse facial features, cessation of statural growth and neuromotor development, severe skeletal abnormalities, organomegaly, and cardiorespiratory insufficiency leading to death in early childhood. MLIII alpha/beta and MLIII gamma are attenuated forms of the disease. Since the identification of the GNPTAB and GNPTG genes, 564 individuals affected by MLII or MLIII have been described in the literature. In this report, we provide an overview on 258 and 50 mutations in GNPTAB and GNPTG, respectively, including 58 novel GNPTAB and seven novel GNPTG variants. Comprehensive functional studies of GNPTAB missense mutations did not only gain insights into the composition and function of the GlcNAc‐1‐phosphotransferase, but also helped to define genotype‐phenotype correlations to predict the clinical outcome in patients