Kondo-Induced Giant Isotropic Negative Thermal Expansion.

Negative thermal expansion is an unusual phenomenon appearing in only a handful of materials, but pursuit and mastery of the phenomenon holds great promise for applications across disciplines and industries. Here we report use of x-ray spectroscopy and diffraction to investigate the 4f-electronic properties in Y-doped SmS and employ the Kondo volume collapse model to interpret the results. Our measurements reveal an unparalleled decrease of the bulk Sm valence by over 20% at low temperatures in the mixed-valent golden phase, which we show is caused by a strong coupling between an emergent Kondo lattice state and a large isotropic volume change. The amplitude and temperature range of the negative thermal expansion appear strongly dependent on the Y concentration and the associated chemical disorder, providing control over the observed effect. This finding opens avenues for the design of Kondo lattice materials with tunable, giant, and isotropic negative thermal expansion.

Plasmon modes in single gold nanodiscs.

Optical properties of single gold nanodiscs were studied by scanning near-field optical microscopy. Near-field transmission spectra of a single nanodisc exhibited multiple plasmon resonances in the visible to near-infrared region. Near-field transmission images observed at these resonance wavelengths show wavy spatial features depending on the wavelength of observation. To clarify physical pictures of the images, theoretical simulations based on spatial correlation between electromagnetic fundamental modes inside and outside of the disc were performed. Simulated images reproduced the observed spatial structures excited in the disc. Mode-analysis of the simulated images indicates that the spatial features observed in the transmission images originate mainly from a few fundamental plasmon modes of the disc.

Kv1.3 blockers ameliorate allergic contact dermatitis by preferentially suppressing effector memory T cells in a rat model.

BACKGROUND: The Kv1.3 voltage-gated potassium channel is selectively upregulated upon activation in effector memory T (TEM ) cells in inflamed tissue, and plays an important role in maintenance of T-cell activation. Although Kv1.3 blockers have been shown to ameliorate allergic contact dermatitis (ACD) in a rat model, it remains unknown whether the effect of Kv1.3 blockers on ACD is mediated by suppressing TEM cell function and/or whether naive T-cells or central memory T (TCM ) cells are influenced. AIM: To analyse the detailed mechanism of Kv1.3 blockers in a rat model of ACD. METHODS: We examined the effects of a Kv1.3 blocker on inflammation and production of the effector cytokine interferon (IFN)-γ in inflamed tissue in rat ACD. Single-cell suspensions were isolated from inflamed rat ears (TEM cells), and regional lymph nodes (naive T/TCM cells), and the effect of Kv1.3 blockers on anti-CD3-stimulated IFN-γ production in vitro was measured. RESULTS: The Kv1.3 blocker significantly suppressed ear inflammation and IFN-γ production at the protein level in vivo. It also suppressed in vitro IFN-γ production from TEM cells from inflamed tissues, but did not suppress the function of naive T/TCM cells from lymph nodes. CONCLUSIONS: We found that the Kv1.3 blocker ameliorated ACD by inhibiting TEM cell functions only, thus Kv1.3 blockers could be a potentially selective therapeutic agent for TEM cell-mediated inflammatory skin diseases without producing harmful side-effects.

Association of T-cell receptor Vbeta haplotypes with dry skin in DS-Nh mice.

BACKGROUND: Although dry skin and T cell-dependent disease exacerbation are characteristic features of atopic dermatitis (AD), the involvement of T cells in the development of dry skin remains unclear. AIMS: We aimed to elucidate the role of T cells in the development of dry skin in DS-Nh mice as a model for AD, and to evaluate this skin condition pharmacologically. METHODS: We prepared DS-Nh mice harbouring a T-cell receptor (TCR)Vbeta(a) haplotype with a central deletion in the TCRBV gene segments, and mice harbouring a TCRVbeta(b) haplotype without any deletion. We analysed the TCRVbeta chain usage and cytokine response to antimouse CD3 monoclonal antibodies in the splenocytes from the two mouse substrains. Transepidermal water loss (TEWL) was measured, and histochemical examination of these mice was carried out. Finally, a pharmacological analysis using loratadine was also performed to evaluate the features of spontaneous dry skin in DS-Nh mice as a model of AD. RESULTS: Although the deletion of TCRBV gene segments in the TCRVbeta(a) haplotype yielded different representations of each TCRVbeta mRNA, this deletion did not evoke distinct cytokine profiles in the splenocytes compared with those of mice with the TCRVbeta(b) haplotype. Furthermore, our results indicated that the onset of dry skin occurred earlier in mice with TCRVbeta(b) than in those with TCRVbeta(a). Pharmacologically, AD-like dry skin in DS-Nh with TCRVbeta(b) mice is susceptible to an H1 blocker. CONCLUSIONS: A specific lymphocyte subpopulation bearing T-cell receptors may be responsible for loratadine-responsive dermatitis in DS-Nh mice.

Raman and near-field spectroscopic study on localized surface plasmon excitation from the 2D nanostructure of gold nanoparticles.

We have studied electromagnetic field on the surface of two-dimensional nanostructure of gold nanoparticles through two-photon-induced photoluminescence images by using scanning near-field optical microscope and far-field surface-enhanced Raman scattering measurements. The near-field two-photon-induced photoluminescence image shows that strong inhomogeneous enhancement in two-photon-induced photoluminescence occurs over the two-dimensional nanostructure, representing localized surface plasmon excitations. The results suggest that the local structure of the two-dimensional nanostructure influences the distribution of localized surface plasmon excitation.

Discovery of superconductivity in quasicrystal.

Superconductivity is ubiquitous as evidenced by the observation in many crystals including carrier-doped oxides and diamond. Amorphous solids are no exception. However, it remains to be discovered in quasicrystals, in which atoms are ordered over long distances but not in a periodically repeating arrangement. Here we report electrical resistivity, magnetization, and specific-heat measurements of Al-Zn-Mg quasicrystal, presenting convincing evidence for the emergence of bulk superconductivity at a very low transition temperature of [Formula: see text] K. We also find superconductivity in its approximant crystals, structures that are periodic, but that are very similar to quasicrystals. These observations demonstrate that the effective interaction between electrons remains attractive under variation of the atomic arrangement from periodic to quasiperiodic one. The discovery of the superconducting quasicrystal, in which the fractal geometry interplays with superconductivity, opens the door to a new type of superconductivity, fractal superconductivity.

Hypercalcemia associated with infantile fibrosarcoma producing parathyroid hormone-related protein.

We describe a 7-month-old boy who manifested severe hypercalcemia associated with mesenchymal neoplasm. A huge hypervascular tumor on the neck had been detected in prenatal ultrasonography. Surgical removal of the entire tumor at birth was not indicated, because the tumor was diagnosed as hemangioma. Chemotherapy and radiotherapy were attempted, but there was no effect on tumor growth. When the infant was 6 months old, the serum calcium level increased rapidly, associated with the expansion of the tumor. Hypophosphatemia due to phosphaturia was also observed. Serum PTH was undetectable, whereas the serum concentration of carboxyl-terminal (C-terminal) fragments of PTH-related protein (PTH-rP) was markedly elevated. Northern blot analysis and immunostaining demonstrated the expression of PTH-rP in the tumor. The tumor was transplantable to nude mice and caused elevation of circulating PTH-rP in the animals. Histological examination of the patient's bone revealed an increased number of osteoclasts. These findings were consistent with humoral hypercalcemia of malignancy caused by the excess production of PTH-rP. The tumor was identified histologically as infantile fibrosarcoma, which has not been reported as a cause of humoral hypercalcemia of malignancy to date. The expression of PTH/PTH-rP receptor messenger ribonucleic acid was detected in the tumor by the RT-PCR, suggesting that PTH-rP may have exerted its effect in the tumor in an autocrine/paracrine manner. In addition to the systemic effect of PTH-rP manifested as hypercalcemia, the PTH-rP secreted from the neoplasm could have been a local factor involved in the growth of the tumor.

Comparison of the fates of ingested leucine and ingested 2-ketoisocaproate in rats.

We previously reported that the ratio, R, of 14C to 3H in the leucine of whole body protein, measured 6 h after ingestion of [3H]leucine and [1-14C]2-ketoisocaproate is equal to ratio of the dose of leucine to the dose of 2-ketoisocaproate (KIC) (on a leucine-free diet) required to achieve the same rate of growth. To determine whether R is dependent on the interval between injection and sampling, R was measured at intervals in purified whole body protein after oral injection of these isotopes in groups of rats; it was constant from 1 h onward for 1 wk, averaging 0.64 +/- 0.01 (means +/- SEM). Thus, the extent of incorporation into the leucine of whole body protein of ingested KIC remains close to 64% of the incorporation of ingested leucine administered as such simultaneously, from 1 h onward for at least 1 wk.

Results of single and double autografts for high-risk neuroblastoma patients.

Although intensive therapy with autologous bone marrow transplantation (ABMT) has improved the outcome of advanced neuroblastoma, nearly half the patients with this disease still relapse after a single ABMT. In our previous study, 10 of 22 patients relapsed within 16 months post-transplantation. Predictive risk-factors for relapse were the presence of bone lesions at diagnosis, and a minor response or progressive disease at transplantation. In order to improve the outcome of these high-risk patients, we tested the feasibility of double autografts. To date, eight patients have been treated, and no treatment-related deaths were observed. Six remain in CR or with stable disease for 6 to 29 months. Although more cases and longer observation are needed to draw conclusions, these results are encouraging.

Successful double autografts for patients with relapsed clear cell sarcoma of the kidney.

Although the prognosis of clear cell sarcoma of the kidney (CCSK) has improved, when metastases occur the probability of cure is very low. We have treated two pediatric patients with relapsed CCSK, one with multiple bone metastases and another with brain metastases. After one or two courses of re-induction chemotherapy and radiation therapy to the sites of metastasis, they received double high-dose chemotherapy with autologous bone marrow rescue. Conditioning regimens were ifosphamide plus melphalan for the first autograft and busulfan plus thiotepa for the second. Hematological recovery was prompt, and no severe complications were observed. They are doing well without evidence of recurrence at 19 and 49 months after the second autograft, respectively.

Studies on the composition of phospholipids in rat small intestinal smooth muscle.

The phospholipid composition of rat small intestinal smooth muscle was investigated in comparison with those of the mucosa and liver. Phospholipid content per g of the wet smooth muscle was almost identical with that of the mucosa and was about 1/4 of that in the liver. The phospholipid/protein ratio of the smooth muscle was about 1/2 of the value in the liver. Sphingomyelin content was significantly high and amounted to 18% of total phospholipids. This value was about twice that in the mucosa and 4 times higher than that in the liver. On the other hand, the percent distribution of phosphatidylcholine was lowest in the smooth muscle. Distribution patterns of phosphatidylserine and phosphatidylinositol in the smooth muscle as well as in the mucosa were different from those in the liver. The occurrence of vinyl-ether and ether phospholipids was clearly demonstrated in the smooth muscle as well as in the mucosa. A major part of the ether lipids was detected in the phosphatidylethanolamine fraction, in which they amounted to about 50%; 40% as alkenyl-acyl type and 12% as alkyl-acyl type. A high content of ether lipids was also observed in the phosphatidylethanolamine fraction from mucosa, but the distribution was reversed, that is, 14% alkenyl-acyl type and 28% alkyl-acyl type. Fatty aldehydes, fatty alcohols, and fatty acids were also determined by gas-liquid chromatography. The compositions of fatty aldehydes in the phosphatidylethanolamine fraction from smooth muscle and from mucosa were similar, whereas the compositions of long chain fatty alcohol and fatty acids were clearly different. The compositions of fatty alcohols and fatty acids of the phosphatidylcholine fraction from smooth muscle showed significantly different patterns from those of the phosphatidylethanolamine fraction and from those of the same phospholipid fraction in the mucosa.

Clinical characteristics of congenital esophageal stenosis distal to associated esophageal atresia.

BACKGROUND: Congenital esophageal stenosis (CES) has been reported to be a rare association in patients with esophageal atresia or tracheoesophageal fistula, or both (EA-TEF). This study investigates the clinical characteristics of this association with special reference to its treatment. METHODS: A retrospective review of medical and radiographic records of 81 patients who underwent primary repair of EA-TEF was performed. An association of CES was diagnosed when they showed histologic evidence or persistent radiographic images of esophageal narrowing since the neonatal period. RESULTS: Eleven of 81 EA-TEF patients (14%) were identified with CES. Their symptoms were difficulty in swallowing solid food, food impaction, emesis, stridor, repeated respiratory infection, and failure to thrive. Two patients had a recurrence of TEF. Three patients were diagnosed with CES in the neonatal period-2 at the time of primary repair of EA-TEF and 1 on the initial postoperative esophagram. The remaining 8 patients were diagnosed between the ages of 2 months and 3 years. Although esophageal dilatation was attempted in 9 patients, its effectiveness was temporary in all except 2 patients and esophageal laceration occurred in 4 patients. Surgical repair including myotomy and resection of the narrow segment was performed in 7 patients, in 6 of whom Nissen/Collis-Nissen fundoplication was added. A histologic examination was performed in 5 patients, revealing that their CES was due to fibro-muscular hypertrophy (2) or tracheobronchial remnants (3). The long-term outcome was satisfactory in all patients except one who showed wrap herniation. CONCLUSIONS: The association between CES and EA-TEF is not rare. Esophageal dilatation was not universally effective and carried with it a considerable incidence of esophageal leakage. An antireflux operation concomitant with repair of CES may be useful to prevent postoperative gastroesophageal reflux in patients with a narrowing close to the esophagogastric junction.

Rate of whole-body protein synthesis in the rat as calculated from fractional oxidation of leucine, valine, or methionine.

In the steady state, the ratio of the rate of utilization for whole-body protein synthesis of any essential amino acid to its rate of oxidation should be the same as the ratio of the peak fraction of a dose of tracer incorporated into protein (F) to the fraction oxidized (1-F) provided that negligible tracer remains in the free amino acid pool or remains unabsorbed in the gut. The total rate of amino acid catabolism (C) can be estimated from the rate of urinary excretion of urea nitrogen (N) plus ammonia N. Hence the rate of whole-body protein synthesis (S) can be estimated as CF/(1-F). This method, which is not new, was explored as follows: (1) Radioactivity in the leucine of whole-body protein of rats after intravenous (IV) injection of labeled leucine was shown to plateau from three to nine hours. (2) The fractions of labeled leucine, valine, and methionine remaining in the gut six hours after enteral injection were 1.2 +/- 0.4% (SD), 1.2 +/- 0.4%, and 7.1 +/- 2.9%, respectively; thus, enterally administered methionine cannot be used for this purpose. (3) Oxidation of [1-14C]-labeled leucine or valine, measured from 14CO2 excretion, was found to be the same whether these isotopes were given IV or enterally. (4) Negligible radioactivity remained in the free leucine of plasma within one hour after injection of labeled leucine.(ABSTRACT TRUNCATED AT 250 WORDS)

Amino acid metabolism in pediatric patients.

As with energy requirements, protein requirements are relatively much greater in infants and decline progressively with age. Amino acid metabolism in pediatric patients is characterized by the following differences. The requirement for essential amino acids in neonates is larger than that in adults. Because of low activity of phenylalanine hydroxylase and cystathionase, hyperphenylalaninemia and hypermethioninemia tend to occur, whereas tyrosine and cysteine tend to be deficient. In addition to cysteine and tyrosine, histidine, lysine, arginine and taurine are considered as semiessential amino acids. Nowadays there are different kinds of amino acid formulas to satisfy these specific requirements, and most of these formulas are intended to normalize the plasma aminogram. However, the nutritional benefit of these formulas for growth and development is still not completely proven, and the pharmacological use for specific diseases is expected with some modification of these formulas.

Effects of dietary histidine and methionine loading in rats with a portacaval shunt.

To elucidate disturbances of brain amine metabolism in hepatic coma, the effects of dietary histidine and methionine loading on the brain levels of amino acids and transmitter amines and on behavior were examined in rats with a portacaval shunt (PCS rats). Surgical construction of a portacaval shunt exaggerated the increase in brain histamine caused by dietary histidine loading 4 weeks after operation. Although the marked increase in the brain level of methionine itself did not affect on the brain levels of catecholamines, serotonin, and histamine, brain level of 5-HIAA was decreased in PCS rats on methionine-enriched diet. Diminished spontaneous activity was observed in PCS rats on either diet, which could not be related to the disturbance of brain amine metabolism caused by excess histidine and methionine. These results indicated that histamine was involved in the derangement of brain amine metabolism in PCS rats, and that direct effect of dietary methionine loading on the brain levels of transmitter amines was not observed in PCS rats. The etiological relation of these substances to hepatic coma should be further investigated.

Hyperbilirubinemia in neonates associated with total parenteral nutrition.

To identify the factors responsible for total parenteral nutrition (TPN) associated jaundice in the neonate, 77 newborns who had been started on TPN during the past 12 years had their charts reviewed. Forty-four (57%) of these infants developed jaundice during the 1st month of life. The incidence of jaundice was significantly higher in the presence of those diseases which were associated with impaired intestinal passage such as congenital duodenal atresia, jejunal atresia, etc, and those with an abnormal rotation of the gut such as diaphragmatic hernia, gastroschisis, etc. Thirty-two (42%) of these 77 infants had accompanying infectious signs, and 28 (88%) of those 32 infants with infectious signs developed jaundice. This incidence was significantly higher than that (36%) among those who had no infectious signs. Of the possible etiologic factors other than infection, neither the length of intrauterine life nor birth weight showed significant correlation with the incidence of jaundice. The incidence of jaundice tended to be higher in infants started on TPN at a younger age. There was no significant correlation between the incidence of jaundice and the duration of TPN or fasting period. Infants receiving 110 cal/kg/day or more during TPN developed jaundice significantly more frequently than those receiving fewer calories. No definite correlation was obtained between the incidence of jaundice and the amount of amino acids administered.

Clinical studies on a newly devised amino acid solution for neonates.

In 97 neonates receiving total parenteral nutrition in the postoperative period, clinical assessment was made for a newly devised amino acid solution (PF-I-III) from the standpoint of plasma amino acid profile and nutritional effect. These amino acid solutions prepared are characterized by the high concentration of branched-chain amino acids up to 40%, increased arginine and decreased glycine, phenylalanine and methionine as compared with commercially available solutions. In the PF group, each amino acid was kept within the range of standard value. Correlation between plasma amino acid profiles and the dose of each amino acid administered was obtained, from which minimum, standard, and maximum doses for each amino acid was determined. Based on these values, we proposed new formula for neonates which elicits no abnormal plasma amino acid pattern even when amino acids are administered at the dosage level of 1.5-2.5 g/kg/day.

Prenatal diagnosis of congenital diaphragmatic hernia and perinatal care: assessment of lung hypoplasia.

To assess the severity of lung hypoplasia, we have attempted to measure the lung thorax transverse area ratio (L/T) by using ultrasonic echography and to select immediate surgery after delivery by caesarean section. The evaluation of L/T for an index of lung hypoplasia was made by arterial blood gas data and clinical courses. Of 14 fetuses diagnosed, 13 had left sided CDH and one right sided case, from 11 to 38 weeks of pregnancy, 10 cases survived. The L/T in 14 fetuses with CDH was from 0.08 to 0.36 (mean 0.2 +/- 0.073) and was significantly lower than that of the controls. L/T was correlated best with data of arterial pH, PCO2, preductal A-aDO2 before operation and the duration of mechanical ventilation in survivors. Although L/T was also significantly low in the cases with severe grade, diaphragmatic patch closure and ECMO therapy, no significant differences were noted in L/T between survivors and non-survivors. These results indicated that L/T may predict the severity of lung hypoplasia in CDH and that the combination of perinatal management bases on prenatal diagnosis of CDH and ECMO support may improve the outcome of fatal CDH with severe lung hypoplasia.

Posterior migration of cervical spinal cord between split laminae as a complication of laminoplasty.

STUDY DESIGN: This is a case report. OBJECTIVE: To focus attention on a possible neurologic complication of laminoplasty. SUMMARY OF BACKGROUND DATA: An iatrogenic spinal cord lesion developed after laminoplasty as an operative complication of unknown cause. METHODS: Two patients had iatrogenic damage to the spinal cord from an unknown cause after laminoplasty of the cervical spine, but recovered soon after an additional laminectomy. They were evaluated with regard to the mechanism of damage and prevention. RESULTS: The spinal cord damage occurred when the spinal cord migrated posteriorly and deformed between the split laminae at the apex of the lordosis, where the maximum posterior displacement of the spinal cord was achieved. CONCLUSION: This damage was caused by a relatively narrower canal resulting from the split laminae of the apex of the adjacent laminae.