[Congenital spindle cell/sclerosing rhabdomyosarcoma: a clinicopathological analysis].

Objective: To investigate the clinicopathological features, immunophenotype and molecular genetic characteristics of congenital spindle cell/sclerosing rhabdomyosarcoma. Methods: Sixteen cases (including 10 consultation cases) of congenital spindle cell/sclerosing rhabdomyosarcoma diagnosed at the Beijing Children's Hospital, Capital Medical University, Beijing China, from April 2017 to January 2022 were collected. These cases were evaluated for clinical profiles, histomorphological features, immunophenotype and molecular characteristics. Results: Among the 16 patients, 9 were male and 7 were female. Five cases were present during maternal pregnancy and 11 cases were found immediately after birth. The tumors were located in the chest wall, low back, retroperitoneum, extremities or perineum. The tumors consisted of fasciculated spindle-shaped cells with localized mesenchymal sclerosis and vitreous metaplasia. Immunohistochemistry showed that the tumor cells expressed Desmin, Myogenin, MyoD1, SMA, CD56 and ALK to varying degrees, but not other markers such as CD34, CD99, pan-TRK, S-100 and BCOR. FISH analyses with NCOA2 (8q13) and VGLL2 (6q22) gene breakage probes revealed a breakage translocation in chromosome NCOA2 (8q13) in 4 cases (4/11). In the 6 cases subject to sequencing, a mutation at the p.L122R locus of MYOD1 gene was detected in 1 case (1/6). Two cases were examined by electron microscopy, which showed bundle-arranged myofilaments with some primitive myofilament formation. Five cases were resected with simple surgery, 2 cases were biopsied and followed up with observation only, and 9 cases were treated with surgery and adjuvant chemotherapy. Follow-up was available in 12 cases. At the end of the follow-up, 2 of the 12 patients developed local recurrences and 2 patients survived with disease. Conclusions: Congenital spindle cell/sclerosing rhabdomyosarcoma is a rare subtype of congenital rhabdomyosarcoma. It more commonly occurs in the chest, back and lower limbs of infants than other sites. NCOA2/VGLL2 gene fusion seems to be the most common genetic change. Its prognosis is better than other subtypes of rhabdomyosarcoma and those in adolescents and adults with the same subtype. Analysis and summary of its clinicopathological features can help differentiate it from other soft tissue tumors in infants and children and provide the information for appropriate treatments.

Identification and diversity of Y-chromosome haplotypes in Qinghai yak populations.

The aim of the present study was to perform a preliminary analysis of the characterization and diversity of Y-chromosome haplotypes/haplogroups in yak of Qinghai Province, China. A total of 322 male yaks from nine populations belonging to three officially recognized breeds (Gaoyuan, Huanhu and Datong) were sampled. Animals were genotyped using six previously reported Y-SNPs present in the SRY, USP9Y, UTY, AMELY and OFD1Y genes and four new Y-SNPs in the OFD1Y gene (g.569A>C, g.578A>C, g.608G>T and g.653G>C) identified in this study. Seven Y-chromosome haplotypes (H1-H7) were identified according to the combination of the 10 Y-SNPs. H1, H2 and H6 were the most common and shared haplotypes across all yak populations/breeds. Private haplotypes H3 and H7 were detected in the Datong breed; H4 in Guoleimude, Qumalai, Qilian, Tianjun and Ganglong populations; and H5 in Qumalai of Gaoyuan breed. Haplotype clustering and network analyses inferred two haplogroups, Y1 and Y2, indicating two divergent lineages of paternal origins of Qinghai yak. The analysis of molecular variance showed a significant difference among individuals (P < 0.0001) with more than 93% of the total genetic variation present within populations, suggesting a weak genetic structure among Qinghai yak populations. The overall Y-haplotype diversity was 0.538 ± 0.028, showing a relatively high diversity in Qinghai yak. The Gaoyuan and Datong breeds had similar haplotype diversities (0.547 ± 0.030 and 0.553 ± 0.083, respectively), which were higher than that of the Huanhu breed (0.441 ± 0.098). Our results support the conservation and sustainable use of unique yak genetic resources in Qinghai.

Element-selective resonant state in M-doped SnTe (M = Ga, In, and Tl).

The band engineering of M-doped SnTe (M = Ga, In, and Tl) is investigated by using first-principles calculations. Being consistent with experimental measurements, our calculations found that Ga doping hardly changes the valence band, while In doping introduces an obvious resonant state near the Fermi level. The resonant state is demonstrated to be from the anti-bonding of In-s and Te-p orbitals. Unexpectedly, no resonant state was observed in Tl-doped SnTe, indicating the nonmonotonic behavior of the Ga-In-Tl series. We show that the absence of the resonant state in Tl-doped SnTe is due to the downward shift of the Tl-s orbital, which may be attributed to the effect of lanthanide contraction. The increase of the Seebeck coefficient in In-doped SnTe is numerically confirmed by Boltzmann transport calculations. Moreover, we find that the mutually matched resonant state location and valence band separation is the key precondition for the combination of the resonant state and band convergence in SnTe. A further enhanced Seebeck coefficient (∼230 µV K(-1)) and ZT value (1.8 at 920 K) are predicted in codoped SnTe by In-Hg, owing to the synergy of two kinds of band engineering.

Band engineering and improved thermoelectric performance in M-doped SnTe (M = Mg, Mn, Cd, and Hg).

The electronic structures of M-doped SnTe (M = Mg, Mn, Cd, and Hg) are investigated by using first-principles calculations including spin-orbit coupling. It is found that Sn vacancy plays an important role in the band engineering of SnTe, showing a different property from its related compound PbTe. The enlarged band gap and reduced energy separation between two valence bands are in good agreement with experimental measurements. Both of the two band modifications lead to the increase of Seebeck coefficients, which is explicitly confirmed by the followed Boltzmann transport calculations. The calculated Seebeck coefficients for Mn-doped SnTe agree well with the experimental data in a broad range of carrier concentration. Owing to the improved Seebeck coefficients, Mn- and Cd-doped SnTe exhibit promising thermoelectric properties with ZT = 1.32 and 1.65 at around 800 K, respectively.

[The application of autologous cartilago auriculae in posterior wall of external auditory canal reconstruction and tympanoplasty].

Objective:To evaluate the value of of autologous cartilago auriculae in posterior wall of external auditory canal reconstruction and tympanoplasty after modified radical mastoidectomy.Method:To determine the outcome of surgery for chronic suppurative otitis media with choleteatoma. The open mastoidectomy and tympanoplasty were performed on sixty patients aged 20-62 years old, and the average age was（43.16±9.43）years old, which includes 31 males and 27 females at our department in the period from September 2011 to September 2014. 58 patients all underwent modified radical mastoidectomy.Study group chose collected intraoperative cortical mastoid bone as a filler combined with autologous cartilago auriculae and temporal fascia muscle-periosteal flap covering the cavity and tympanoplasty.Control group chose collected intraoperative cortical mastoid bone as a filler temporal fascia muscle-periosteal flap covering the cavity. The hearing improvement at the time of achieving dry ear,3 months and 6 months after surgery were evaluated for both groups.Result:There are significant differeence between two groups in improvement of hearing and dry ear time,according to follow-up and analysis of therapeutic efficacy(P <0.05), There was no recurrence of cholesteatoma in 2 groups.Conclusion:The application of autologous cartilago auriculae in posterior wall of external auditory canal reconstruction and tympanoplastycan that can get more in line with the external auditory canal and the volume of physiological structure state of near normal tympanic cavity, dry ear and hearing improvement was satisfied.The autologous cartilago curiculae is suitable for repairing materials of open radical operation.

Y-STR INRA189 polymorphisms in Chinese yak breeds.

To further explore Y-STR INRA189 polymorphisms in the yak, and to determine the genetic differences among yak breeds, genotyping analysis of INRA189 in 102 male yak individuals from three yak breeds in Qinghai Province of China was performed. Genotyping revealed the presence of four alleles, with sizes of 149, 155, 157, and 159 bp, respectively. Of these, the 157-bp allele, which was found with the highest frequency in the three yak breeds, was the dominant allele. Interestingly, the 149-bp allele was only detected in the Gaoyuan breed, and the 159-bp allele was only found in the Huanhu and Datong breeds. Only the 157- and 155-bp alleles were found in all three yak breeds. Taking the three yak breeds as a single population, the frequency of these four alleles was 0.0294, 0.0686, 0.8628, and 0.0392, respectively. The average polymorphism information content in the three yak breeds was 0.2379, indicating that the INRA189 was a low polymorphic Y-STR marker in yak.

Double-resonance enhanced intersubband second-order nonlinear optical susceptibilities in GaN/AlGaN step quantum wells.

Second-order nonlinear optical susceptibilities for second harmonic generation (SHG) associated with intersubband transitions in GaN/AlGaN single quantum well and step quantum well have been studied theoretically by solving Schrödinger and Poisson equations self-consistently. The calculated results suggest that due to the very large polarization-induced field in the quantum well, the potential profile becomes asymmetrical, leading to large second-order susceptibilities. A high value about 4 × 10-7 m/V can be obtained in single quantum well structure. Furthermore, by adopting step quantum well structure to increase the asymmetry degree of the potential profile and manipulate the energy levels for double-resonance, a significant enhancement of second-order susceptibility can occur in step quantum well. Specifically, the susceptibility can be as large as 4 × 10-6 m/V with structure optimization, about an order of magnitude greater than that in single quantum well. The results indicate that nonlinear optical elements based on GaN/AlGaN step quantum wells are very promising for SHG in a wide range of wavelengths from telecommunication to mid-infrared, especially effective in longer wavelength.

Postsynaptic density-93 deficiency protects cultured cortical neurons from N-methyl-D-aspartate receptor-triggered neurotoxicity.

It has been reported that N-methyl-D-aspartate receptor (NMDAR)-triggered neurotoxicity is related to excessive Ca(2+) loading and an increase in nitric oxide (NO) concentration. However, the molecular mechanisms that underlie these events are not completely understood. NMDARs and neuronal NO synthase each binds to the scaffolding protein postsynaptic density (PSD)-93 through its PDZ domains. In this study, we determined whether PSD-93 plays a critical role in NMDAR/Ca(2+)/NO-mediated neurotoxicity. We found that the targeted disruption of the PSD-93 gene attenuated the neurotoxicity triggered by NMDAR activation, but not by non-NMDAR activation, in cultured mouse cortical neurons. PSD-93 deficiency reduced the amount of NMDAR subunits NR2A and NR2B in synaptosomal fractions from the cortical neurons and significantly prevented NMDA-stimulated increases in cyclic guanosine 3',5'-monophosphate and Ca(2+) loading in the cortical neurons. These findings indicate that PSD-93 deficiency could block NMDAR-triggered neurotoxicity by disrupting the NMDAR-Ca(2+)-NO signaling pathway and reducing expression of synaptic NR2A and NR2B. Since NMDARs, Ca(2+), and NO play a critical role during the development of brain trauma, seizures, and ischemia, the present work suggests that PSD-93 might contribute to molecular mechanisms of neuronal damage in these brain disorders.

[Roles of Galphaq/11 mediated- and platelet-derived growth factor mediated-signal transduction pathways in rat aorta restenosis].

To observe the roles of Galphaq/11 mediated- and platelet-derived growth factor (PDGF) mediated-signal transduction pathways in proliferation and migration of vascular smooth muscle cells (VSMC) after arterial injury, a vascular cell proliferation model was established by balloon injury in rat aorta and the morphologic changes in injured vascular walls after the injury were studied. Activities of angiotensin converting enzyme (ACE) and aorta phospholipase C (PLC) were tested, and levels of platelet-derived growth factor receptor-beta (PDGFR-beta) and Galphaq/11 protein were measured by Western blot analysis. At l day after operation, injured aortic segments showed denudation in endothelial cells. Medial VSMC proliferation and intimal hyperplasia were not observed. As compared with the sham group, ACE activities were increased by 382.7 percent; (P<0.0l), but the expression of PDGFR-beta and PLC activities did not show significant changes (P>0.05). In addition, the level of Galphaq/11 protein was decreased by 20.0 percent; (P<0. 05). At l4 days after operation, sections of injured aorta showed marked intimal thickening with large numbers of VMSCs proliferating throughout intima and media. In comparison with the sham group, ACE activity, PLC activity and the level of PDGFR-beta were increased by 420.2 percent; (P<0.01), 186.2 percent; (P<0.05) and 85.0 percent; (P<0.05), respectively. While the level of Galphaq/11 protein was decreased by 33.1 percent; (P<0.01). The above data suggest that the PDGF-mediated signal transduction pathway plays an important role in VSMC proliferation.

[The changes of cell structure in the courses of Armillaria mellea penetrating the nutritional stems of Gastrodia elata].

OBJECTIVE: To study the cell structure changes of Gastrodia elata after Armillaria mellea infection as well as the nutritional resource of this important medicinal plant. METHOD: Observation of the serial sections on the nutritional stems of G. elata, and measure new corms of G. elata when cut off the old corms connection with rhizomorph of A. mellea. RESULT: After G. elata nutritional stems infected by A. mellea, the rhizomorph of A. mellea separate into several hyphal layers and penetrate hyphal stream which infect into the cells of cortex layer in the direction of outside and infect directly into layer cells in the direction of inside, these hypha can be used the nutrition of G. elata the new corms will be stop growth if cut off the rhizomorph of A. mellea connected with G. elata. CONCLUSION: After the rhizomorph of A. mellea infected G. elata nutritional stems, hyphal coils and the hyphal stream will breakthrough passage cells as well as large cells surround the whole nutritional stems, so the passage cells is the key nutritional resource in the whole growth period of G. elata.

Experimental studies on penetrating heterokeratoplasty with human corneal grafts in monkey eyes.

In nine rhesus monkeys, human corneal grafts were used for penetrating keratoplasty in one eye of each. The data were divided into three groups according to the size of grafts. Group 1 (n = 3) received 5.5-mm human corneal grafts in 5.0-mm recipient beds; group 2 (n = 3) received 6.2-mm human corneal grafts in 6.0-mm beds; and group 3 (n = 3) received 7.5-mm grafts in 7.0-mm beds. Five of nine eyes in groups 1 and 2 (55%) maintained graft clarity for more than 1 year, but only two of the three in group 1 maintained clear human corneal grafts for 32 and 36 months, respectively. However, the three grafts in the third group maintained transparency for only 4-7 months. Our findings suggest that xenogeneic corneal grafts may have a greater success rate in humans than previously believed.

Effects of anti-inflammatory and immunosuppressive drugs on the heterolamellar corneal transplantation in rabbits.

We have studied the graft rejection of bovine lamellar cornea transplanted onto the rabbit cornea. In addition, the effect of inhibitors of arachidonic acid metabolism and immunosuppressive agents on this heterolamellar corneal transplant was investigated. The corneal lamellar transplant treated with saline were rejected at 9 +/- 1.4 day with 100% rejection rate. The postsurgical conjunctival hyperemia and edema occurred within 2-3 days. Corneal neovascularization took 5 to 9 days to reach the region of the graft. Intramuscular cyclosporin A (25mg/kg/day) and dexamethasone (0.1%) applied topically for 20 days (t.i.d.) inhibited the corneal opacity, edema, neovascularization and graft rejection. Topical Dexamethasone in the ipsilateral eye prolonged survival of grafts in the contralateral (untreated) eye. Cyclosporin A and dexamethasone probably inhibited the graft rejection process by their immunosuppressive action. Indomethacin and BW755C, respective cyclooxygenase and lipoxygenase inhibitors, applied topically did not inhibit graft rejection process while inhibiting postsurgical inflammatory response.

Immobilization of callus tissue cells of Salvia miltiorrhiza and the characteristics of their products.

The Salvia miltiorrhiza callus tissue cells were entrapped with 3% alginate. The immobilized cells were incubated in medium with LS + KT 0.1 + NAA 1.0 containing 3% sucrose at 25 degrees C for nearly one month. After incubation, the medium free from cells was extracted with ether 3 times. After evaporation, the residue of the ether extract was employed to determine the content on TLC and HPLC. The results showed that the incubation system mentioned above could continuously secrete the main components of S. miltiorrhiza, tanshinone IIA and cryptotanshinone, which were almost the same as the extract of S. miltiorrhiza roots. In addition, suspension incubation of callus tissue cells, the conditions of immobilization with alginate, the stability of immobilized cells and the characteristics of products were also examined.

[Studies on the life cycle of Gastrodia elata].

Seeds of G. elata are strongly reduced small structures. They lack nutrient tissues entirely. A small embryo is surrounded by a single-layered seed coat with a very loose structure. Digestion of certain kinds of fungi (Mycena osmundicola, etc) which have invaded the embryonic cells provides the necessary nutrition to sprout. Protocorms obtain their nutrition from invading Armillaria mellea and then go on to vegetative propagation, becoming small, white G. elata within a year. The commercial arrow G. elata can be obtained late in the year. Over the winter, it will bud, blossom and bear fruit, following in the next year. Taking three years to complete its life cycle, G. elasta depends upon the digestion of certain kinds of fungi----Mycena osmundicola, A. mellea, etc.