Above 25†nm emission wavelength shift in blue-violet InGaN quantum wells induced by GaN substrate misorientation profiling: towards broad-band superluminescent diodes.

We report a thorough study of InGaN quantum wells spatially modified by varying the local misorientation of the GaN substrate prior to the epitaxial growth of the structure. More than 25 nm shift of emission wavelength was obtained, which is attributed to indium content changes in the quantum wells. Such an active region is promising for broadening of the emission spectrum of (In,Al,Ga)N superluminescent diodes. We observed that the light intensity changes with misorientation, being stable around 0.5° to 2° and decreasing above 2°. This relation can be used as a base for future device designing.

Evaluation of the non-functional tooth contact in patients with temporomandibular disorders by using newly developed electronic system.

The aims of this study were to introduce a novel electronic system for reliable evaluation of the non-functional tooth contact in patients with temporomandibular disorders (TMDs) and investigate the possible associations between the non-functional tooth contact and some characteristics of the patients with TMD. We designed and installed a software program to send emails regarding the non-functional tooth contact to the subjects' preregistered cellular phones at intervals of 20 ± 9 min daily for 10 consecutive days. Twelve patients with TMD and 12 gender- and age-matched healthy subjects responded via emails to one of 3 choices: no tooth contact, tooth contact during oral functions or tooth contact not associated with oral functions. The influence of subjective stress, anxiety, depression, personality and daily activities on tooth contact was then assessed. The frequency of the non-functional tooth contact was significantly higher in the patients with TMD than in the healthy subjects (35·0% vs. 9·6%, P < 0·001), while no significant group difference was found for the frequency of functional tooth contact, the stress, anxiety, depression and personality.

Instrumentation for dual-probe scanning near-field optical microscopy.

To investigate local carrier motions, we developed a dual-probe scanning near-field optical microscope (SNOM) with two fiber probes where one is for photoexcitation and the other is for light collection. This instrumentation is based on two important techniques: the design of probe structures and distance control between the sample surface and the two probes. A finite-difference time-domain method numerically analyzed and optimized the design for high efficiency photoexcitation and light collection, while a dual band modulation realized distance control. Real time detection of the oscillations of the probe tips using different frequencies independently controls the distance between the probe tip and the sample surface as well as the distance between the two probes. Thus, the collection probe can be scanned around an illumination probe without destroying the probe tips. To demonstrate our SNOM, we performed photoluminescence spectroscopy under the dual-probe configuration and observed carrier motions in an InGaN quantum well.

Single mode emission and non-stochastic laser system based on disordered point-sized structures: toward a tuneable random laser.

As an advantage, random lasers may be elaborated from a large variety of materials and do not require any cavity oscillators that usually necessitate complicated and expensive fabrication techniques. Since the feedback process of those non-conventional laser systems is provided by light interference in a disordered medium, spectral and temporal uncertainties are usually considered as an intrinsic part of their optical proprieties. We investigated random laser action under two photon absorption experiments through an auto-organized InGaN/GaN quantum-disks ensemble. Thanks to our experimental approach, we evidence random lasing based on a gain medium constituted by point-sized structures. In such context, a stabilised and individual emission mode is observed as for conventional semiconductor lasers. By controlling the emission energy of these nanostructures, a tuneable and stable random laser may be built. Moreover, our findings suggest that disordered medium should play an important role in the conception of low cost quantum dot and up conversion laser systems.

Optical gain spectra for near UV to aquamarine (Al,In)GaN laser diodes.

Optical gain spectra presented for (Al,In)GaN laser diodes with lasing wavelength ranging from UV (375 nm) to aquamarine (470 nm) show a strong increase in inhomogeneous broadening, caused by Indium composition and quantum well width fluctuations which increase with Indium mole fraction. These gain spectra provides a standard data set for the calibration of microscopic many-body simulations. We demonstrate by comparison with basic simulations that the different assumptions of a global constant carrier density or of global constant quasi-Fermi levels for electrons and holes lead to a strikingly different dependency of optical gain on carrier density. For constant quasi-Fermi levels the threshold carrier density becomes insensitive to inhomogeneous broadening for realistic parameters. This is in agreement with the observation that the threshold current is nearly independent over the wavelength range from near UV to aquamarine.

Direct ligand-receptor complex interaction controls Brassica self-incompatibility.

Many higher plants have evolved self-incompatibility mechanisms to prevent self-fertilization. In Brassica self-incompatibility, recognition between pollen and the stigma is controlled by the S locus, which contains three highly polymorphic genes: S-receptor kinase (SRK), S-locus protein 11 (SP11) (also called S-locus cysteine-rich protein; SCR) and S-locus glycoprotein (SLG). SRK encodes a membrane-spanning serine/threonine kinase that determines the S-haplotype specificity of the stigma, and SP11 encodes a small cysteine-rich protein that determines the S-haplotype specificity of pollen. SP11 is localized in the pollen coat. It is thought that, during self-pollination, SP11 is secreted from the pollen coat and interacts with its cognate SRK in the papilla cell of the stigma to elicit the self-incompatibility response. SLG is a secreted stigma protein that is highly homologous to the SRK extracellular domain. Although it is not required for S-haplotype specificity of the stigma, SLG enhances the self-incompatibility response; however, how this is accomplished remains controversial. Here we show that a single form of SP11 of the S8 haplotype (S8-SP11) stabilized with four intramolecular disulphide bonds specifically binds the stigma membrane of the S8 haplotype to induce autophosphorylation of SRK8, and that SRK8 and SLG8 together form a high-affinity receptor complex for S8-SP11 on the stigma membrane.

Upshaw-Schulman syndrome revisited: a concept of congenital thrombotic thrombocytopenic purpura.

Upshaw-Schulman syndrome (USS) is a congenital bleeding disorder characterized by repeated episodes of thrombocytopenia and microangiopathic hemolytic anemia that respond to infusions of fresh frozen plasma. Inheritance of USS has been thought to be autosomal recessive, because 2 siblings in the same family are often affected but their parents are asymptomatic. Recently, chronic relapsing thrombotic thrombocytopenic purpura (CR-TTP), reported almost exclusively in adults, was shown to be caused by inherited or acquired deficiency in the activity of a plasma von Willebrand factor-cleaving protease (vWF-CPase). The pathogenesis of USS is unknown, and a relationship between CR-YEP and USS has not been reported. We studied 3 unrelated USS patients (ST, SY, and KI) who presented with severe indirect neonatal hyperbilirubinemia. All 3 patients had undetectable vWF-CPase activity, and the inhibitors to vWF-CPase were all negative. In their parents with no clinical symptoms, vWF-CPase activities as a percentage of control samples (mother/father) were 17/20 for ST, 60/45 for SY, and 36/5.6 for KI. Thus, USS and vWF-CPase activity appear to be coinherited as autosomal recessive traits. Transfusion of fresh frozen plasma in 2 patients (ST and SY) resulted in the expected maximal increment of approximately 7% to 8% in vWF-CPase activity at 1 to 4 hours, but the levels became less than 3% within 2 days. After this decrease, platelet counts increased, plateaued in the normal range at 10 to 12 days, and declined thereafter. Thus, the 2 to 3 weeks of therapeutic benefit from plasma infusions will be discussed in relation to the intravascular lifetime of vWF-CPase.

A pollen coat protein, SP11/SCR, determines the pollen S-specificity in the self-incompatibility of Brassica species.

Many flowering plants have evolved self-incompatibility (SI) systems to prevent inbreeding. In the Brassicaceae, SI is genetically controlled by a single polymorphic locus, termed the S-locus. Pollen rejection occurs when stigma and pollen share the same S-haplotype. Recognition of S-haplotype specificity has recently been shown to involve at least two S-locus genes, S-receptor kinase (SRK) and S-locus protein 11 or S-locus Cys-rich (SP11/SCR). SRK encodes a polymorphic membrane-spanning protein kinase, which is the sole female determinant of the S-haplotype specificity. SP11/SCR encodes a highly polymorphic Cys-rich small basic protein specifically expressed in the anther tapetum and in pollen. In cauliflower (B. oleracea), the gain-of-function approach has demonstrated that an allele of SP11/SCR encodes the male determinant of S-specificity. Here we examined the function of two alleles of SP11/SCR of B. rapa by the same approach and further established that SP11/SCR is the sole male determinant of SI in the genus Brassica sp. Our results also suggested that the 522-bp 5'-upstream region of the S9-SP11 gene used to drive the transgene contained all the regulatory elements required for the unique sporophytic/gametophytic expression observed for the native SP11 gene. Promoter deletion analyses suggested that the highly conserved 192-bp upstream region was sufficient for driving this unique expression. Furthermore, immunohistochemical analyses revealed that the protein product of the SP11 transgene was present in the tapetum and pollen, and that in pollen of late developmental stages, the SP11 protein was mainly localized in the pollen coat, a finding consistent with its expected biological role.

Prospective study of mother-to-infant transmission of hepatitis C virus.

BACKGROUND: Mother-to-infant transmission of hepatitis C virus (HCV) could become the main route of HCV infection in the future because there are no methods available to prevent vertical infection. The aim of this study was to determine the incidence of mother-to-infant transmission in infants born to mothers who tested positive for anti-HCV antibodies and to elucidate associated risk factors for transmission. METHODS: Screening was conducted for 16,800 pregnant women with an anti-HCV antibodies test, and 154 mothers were positive. From the positive group 141 mothers were enrolled in the study and their 147 infants were followed from birth for serum alanine aminotransferase activity, anti-HCV antibodies and HCV RNA. HIV infection was tested in 73 of 141 mothers, all of whom were negative. RESULTS: Thirty-three infants were dropped from the study because they were followed for <6 months or were not tested adequately. Of the 114 infants finally evaluated 9 (7.8%) had detectable HCV RNA. The transmission rate was not influenced by the mode of delivery [vaginal delivery, 8 of 90 vs. cesarean section, 1 of 24 (P = 0.396)] or by the type of feeding [9 of 98 for breast-fed infants vs. 0 of 16 for formula-fed infants (P = 0.243)]. All infected infants were born to mothers who had HCV viremia at the delivery (P = 0.040) and to those with a high viral load (P = 0.019). CONCLUSIONS: Our prospective study showed that the transmission rate of mother-to-infant HCV infection was 7.8% in anti-HCV antibody-positive mothers. Risk was related to the presence of maternal HCV viremia at delivery and a high viral load in the mothers.

Measurement of bone mineral density of lumbar spine and whole body in low-birth-weight infants: comparison of two methods.

In the present study, we compared lumbar spinal and whole-body bone mineral density (BMD) measurements to determine which is more suitable for evaluating the bone mineral status of low-birth-weight (LBW) infants. Lumbar spinal and whole-body BMD were assessed simultaneously in a prospective series including 152 Japanese LBW infants (birth weight 453-2400 g, gestational age 24-38 weeks) from the age of 40 weeks post-conception to 2 years of age. Lumbar spinal BMD at 40 weeks post-conception was significantly correlated with birth weight (r = 0.74; P < 0.0001), but wholebody BMD was not correlated with birth weight. No correlation was found between lumbar spinal and whole-body BMD at 40 weeks post-conception. However, after 40 weeks post-conception, a significant correlation was found between lumbar spinal and whole-body BMD (r = 0.65; P < 0.0001). For infants with a body weight of 4 kg or less at the time of measurement, no correlation was found between lumbar spinal and whole-body BMD. However, for infants with a body weight above 4 kg, a significant correlation was found between lumbar spinal and whole-body BMD (r = 0.65; P < 0.0001). Thus, lumbar spinal BMD is more suitable than whole-body BMD for evaluation of the bone mineral status of LBW in early infancy. Therefore, lumbar spinal BMD should be used for serial evaluation of changes in the bone mineral status of LBW infants.

A novel mutation (296 del G) of the SOX90 gene in a patient with campomelic syndrome and sex reversal.

The human SOX9 gene is responsible for the campomelic syndrome (CMPS) and sex reversal. This gene encodes a transcription factor containing a DNA binding domain homologous to the SRY high mobility group (HMG) domain. A novel mutation of SOX9, i.e. a single G deletion in one allele at nt 296 from A of the first ATG in the open reading frame, was identified in a patient with CMPS with sex reversal. The deletion resulted in a frameshift mutation upstream of the HMG box and a stop codon 30 bp downstream of the HMG box. The predicted truncated SOX9 protein contained 108 amino acids instead of the 509 amino acids of the normal SOX9 protein, removing nearly 80% of the SOX9 protein, including the HMG and the C-terminal transactivation domain. Most patients with CMPS reported previously died within the neonatal period. Our findings that the patient has survived, although has been in daily need of mechanical ventilation support for 5 years and 3 months despite a severely impaired SOX9 protein, do not support a linear relationship between the type of mutation and severity of the clinical outcome.

Bone mineral density of the lumbar spine in very-low-birth-weight infants: a longitudinal study.

UNLABELLED: To examine osteopenia in very low birth weight (VLBW) infants we used repeated dual-energy X-ray absorptiometry in a prospective study of lumbar spinal bone mineral density (BMD) in Japanese VLBW infants (birthweight 426-1498 g; n = 61, group 1) aged 40 weeks postconception to 3 years of age. Control subjects were Japanese infants with birthweight 1500-1999 g (group 2), 2000-2499 g (group 3), or more than 2500 g (group 4). BMD in group 1 during the early period after birth was very low, increased rapidly for 1 year, and then gradually increased until 3 years of age (r = 0.931, P < 0. 0001). BMD at the age of 40 weeks postconception was 0.085 +/- 0.026, 0.132 +/- 0.039, 0.178 +/- 0.042, and 0.196 +/- 0.046 g/cm(2) in groups 1, 2, 3, and 4, respectively (P < 0.0001). However, at 1 and 2 years of age no differences were observed among the groups in BMD. CONCLUSION: This study shows that lumbar spinal BMD in VLBW infants can normalize by the age of 2 years.

Correlation between magnetic resonance imaging and clinical profiles of periventricular leukomalacia.

Magnetic resonance imaging (MRI) findings of 70 children with periventricular leukomalacia (PVL), examined between 1 year 2 months and 8 years of age (mean: 2 years 4 months of age), were analysed. Neurological assessments were made between 1 year 3 months and 15 years (mean: 4 years 9 months). The possible correlations between MRI findings and clinical profiles of PVL were investigated using three parameters of the MRI findings. The grade of ventriculomegaly correlated well with the severity of cerebral palsy (CP) but not with the severity of mental impairment. The grade of reduction of periventricular white matter correlated well with the severity of CP and mental impairment, and is the most reliable parameter for neurological prognosis. The degree of periventricular hyperintensity on T2-weighted images did not correlate well with severity of CP, but correlated to some degree with mental impairment. There was a significantly lower degree of periventricular hyperintensity in children at less than 28 weeks of gestation than at 28 or more weeks of gestation, but no significant difference in other parameters. The periventricular hyperintensity should be evaluated in view of the gestational age.

Transient increase in cerebral blood flow in a patient with Sturge-Weber syndrome.

We report the case of an infant with Sturge-Weber syndrome whose regional cerebral blood flow (rCBF) showed an increase and a subsequent decrease. As compared with simultaneous CT and MR findings, we speculate that the transient increase in rCBF reflects the neuronal reaction to hypoxia caused by congestion.

[A case of acute necrotizing encephalopathy of childhood with a good outcome].

We experienced a case of acute necrotizing encephalopathy of childhood with a good outcome. The disease continued for about three weeks, and the patient recovered with a sequela of mild left-hemiparesis. On the brain magnetic resonance imaging, abnormal intensity areas in the thalamus and putamen reduced size at an early stage of the disease. After 1 year, his mental and motor development seemed to be almost normal, but brain single photon emission computed tomography and electroencephalography were abnormal. Subclinical functional abnormalities persisted longer than had been expected.

National survey of periventricular leukomalacia in Japan.

BACKGROUND: Clarification of the present status of periventricular leukomalacia (PVL) in Japan. METHODS: Questionnaires were mailed to the leading neonatal intensive care units (NICU) and rehabilitation centers for children. RESULTS: The incidence of PVL in the group of surviving preterm infants of gestational ages less than 33 weeks was 4.8-4.9% on ultrasound and 7.7-7.9% on magnetic resonance imaging and/or computed tomography. The incidence of PVL did not decrease between 1990 and 1994. The incidence of PVL in the NICU varied from 0 to 47.1%. Infants from multiple pregnancies had a higher incidence (9.1%) of PVL than those from single pregnancies (6.2%). The proportion of PVL in all patients in rehabilitation centers with cerebral palsy (CP) has increased recently. CONCLUSIONS: It was roughly calculated that about 750 cases of CP with PVL occurred annually in Japan, thus accounting for about one-third of the total number of cases of CP.

Trends in neonatal exchange transfusions at Yodogawa Christian Hospital.

A review was conducted to determine the trends in exchange transfusion (ET) of newborn infants at the Yodogawa Christian Hospital during the past 18 years. At that hospital in 1957, the first ET was performed on a term infant with severe hemolytic jaundice caused by rhesus factor (Rh) incompatibility. By 1989, ET had been performed in more than 1400 newborn infants. These cases of newborns who had had ET were retrospectively reviewed, with a focus on every 3 year period from 1974 to 1992. The total number of infants requiring ET noticeably decreased from 68 cases (14.0% of total admissions) in 1974 to 19 cases (6.1% of total admissions) in 1992. (chi 2, P < 0.001) There were three major significant changes in ET during those years. The first was a change in the subjects for ET. The incidence of ET for term infants showed a marked decrease, while the incidence of ET for preterm infants, especially for very low birthweight (VLBW) infants (< 1500 g), noticeably increased. The second was a change in indications for ET. There was a marked decrease in the need for ET as a result of hyperbilirubinemia, while the incidence of ET because of other etiologies, such as septicemia and/or disseminated intravascular coagulopathy, noticeably increased. The third was a change in the technical methods of ET. Now at the Yodogawa Christian Hospital, 100% of the infants are given ET with an automated peripheral two-site method, instead of the Diamond method. Although ET might still be a useful treatment for severe hyperbilirubinemia and other acute problems, the total number of ET noticeably decreased in accord with a decrease in the number of severe hyperbilirubinemia in term newborns. On the other hand, the incidence of ET in preterm infants increased relatively, accompanied by an increase in the survival of VLBW infants. The automated two-site method is the preferred technique for ET at the Yodogawa Christian Hospital, rather than the Diamond method. Further changes in ET might occur in accord with new alternative measures in future.

[Quality of life of children with chronic neurological diseases: introductory remarks].

Recent advances in medical technology, such as an assisted ventilation, have made a big impact on pediatrics. With such a progress, many children with intractable diseases have survived intact. On the other hand, chronically ill children with handicaps have also been increasing. Some of them have been artificially supported by a respirator to maintain life in a hospital or at home. Under these social conditions, we should establish a system for total care of these children, to promote their quality of lives, in collaboration with medicine, health & welfare, and education.

Haemodynamic effects of differing blood transfusion rates in infants less than 1500 g.

OBJECTIVE: To investigate whether the haemodynamic effects of the standard 2-3 h blood transfusion increases the risk for intraventricular haemorrhage (IVH) and patent ductus arteriosus (PDA) in very low birthweight infants. METHODOLOGY: In a randomized controlled study, haemodynamic changes using slow and rapid transfusion were compared. Twenty-seven very low birthweight infants were divided between 12h (n = 14) and 3h (n = 13) transfusion groups. Blood pressure, ejection fraction (EF), anterior cerebral artery pulsatility index (PI), blood gases, serum electrolytes and haematocrit were measured pre- and post-transfusion. Infectious status was also monitored. RESULTS: Blood pressure (48.1/25.5 vs 55.7/30.2 mmHg) and EF (0.68 vs 0.73) increased significantly during rapid transfusion (P<0.01) but remained stable with slow transfusion. Serum potassium, base excess and incidence of infection did not increase in either group. CONCLUSIONS: Slow transfusion causes less haemodynamic disturbance than rapid transfusion, thereby preventing the potential risk for IVH and PDA.