Parkinsonism after ventriculoperitoneal shunt for hydrocephalus.

BACKGROUND: Parkinsonism after ventriculoperitoneal shunt in patients with hydrocephalus is a rare and profound complication that is often misdiagnosed, causing treatment to be delayed. To date, the characteristics of this disease have not been well described and summarized. Here, we report a rare case of parkinsonism after ventriculoperitoneal shunt; symptoms were aggravated by antipsychotic drugs but showed a good response to Madopar. Such cases have rarely been reported previously. CASE PRESENTATION: A 44-year-old man presented with parkinsonism, bilateral pyramidal tract signs, and oculomotor impairment four years after a successful ventriculoperitoneal shunt for idiopathic aqueduct stenosis resulting in obstructive hydrocephalus. Brain magnetic resonance imaging and computed tomography showed fluctuations in the lateral ventricle and the third ventricle without any intervention. The patient's condition was aggravated by antipsychotic drugs but showed a good response to Madopar. CONCLUSION: This observation suggests that parkinsonism in this patient was caused by reversible dysfunction of the presynaptic nigrostriatal dopaminergic pathway due to fluctuations in the lateral ventricle, representing the first hit to the dopaminergic signalling pathway, and antipsychotic drugs had an antagonistic effect on dopamine D2 receptors, representing the second hit. In addition, we summarize the pathophysiological mechanisms, clinical manifestations, treatments, and prognoses of this complication in 38 patients who met the inclusion criteria in 24 previous studies to increase neurologists' understanding of the disease.

[Endovascular abdominal aortic aneurysm repair with a new stent graft:early results from a multicenter study].

Objective: To examine the safety and effectiveness of a new stent graft system for endovascular repair of abdominal aortic aneurysm(AAA). Methods: This is a prospective,multi-center,single-arm clinical trial. The patients with AAA treated with a new stent graft system were enrolled at 21 centers from September 2018 to September 2019 in China. Follow-up was performed before discharge, and at 30, 180, 360 days after operation, respectively. The primary safety endpoint was the incidence of major adverse events(MAE) within 30 days. The primary efficacy endpoint was the success rate of AAA treatment at 360 days. Secondary safety endpoints were the incidence of perioperative access complications and acute lower limb ischemia,all-cause mortality, AAA related mortality and incidence of serious adverse events (SAE) at 180 and 360 days. Secondary efficacy endpoints were the incidence of type Ⅰ or Ⅲ endoleak,stent displacement,and conversion to open surgery or re-intervention at 180 and 360 days. Results: One hundred and fifty-six patients were enrolled,including 137 males and 19 females. The age was (68.9±6.9) years (range:48.2 to 84.6 years).Maximum aneurysm diameter was (50.8±11.2) mm (range:25.0 to 85.0 mm),diameter of proximal landing zone was (21.2±2.5) mm (range:17.0 to 29.5 mm),and length of proximal landing zone was (31.4±13.0) mm (range:11.0 to 75.0 mm).The incidence of MAE was 1.3% (2/156) at 30 days,both were all-cause death cases. The success rate of AAA treatment was 88.5% (138/156) at 360 days. No perioperative access complication and acute lower limb ischemia occurred. All-cause mortality was 2.0% (3/154) at 180 days and 2.6% (4/153) at 360 days,and there was no AAA related death. The incidence of SAE was 23.0%(35/152) at 180 days and 30.5%(46/151) at 360 days, and no device-related SAE occurred. The incidence of type Ⅰor Ⅲ endoleak was 3.4% (5/147) at 180 days and 3.5% (5/144) at 360 days. Conclusion: The new stent graft system is easy to operate,and early-term safety and effectiveness results are expected.

[Observation and quantification of diabetic keratopathy in type 2 diabetes patients using in vivo laser confocal microscopy].

Objective: To study the diabetic keratopathy in type 2 diabetes patients with retinopathy by in vivo laser confocal microscopy. Methods: This was a case-control study. Ninety type 2 diabetes patients were involved in this study from May 2015 to December 2019 in Qingdao Eye Hospital. According to the diabetic retinopathy clinical stage, these patients were divided into the non-proliferative diabetic retinopathy (NPDR) group (30 cases), early stage proliferative diabetic retinopathy (PDR) group (30 cases) and intermediate to late stage PDR group (30 cases). Thirty non-diabetic healthy volunteers were included in the control group. The central cornea was observed with an in vivo laser confocal microscope. The corneal nerve fiber density, nerve fiber length, nerve branch density, and nerve fiber tortuosity were compared between groups. The corneal Langerhans cells, epithelial cells, stromal cells and endothelial cells were also compared. Results: There were more nerve fibers and branches in the control group than the other three diabetic groups. The nerve fiber length in the control group, NPDR group, early stage PDR group and intermediate to late stage PDR group was (21.55±2.57), (14.73±1.56), (11.23±1.40) and (8.02±1.33) mm/mm2, respectively, and there were statistically significant differences between the groups (F=316.17, P=0.00). In the nerve fiber density, nerve branch density and curvature, there were statistically significant differences between the groups (F=345.72, 479.46, 167.00, all P=0.00). The basal cell density in the control group, NPDR group, and two PDR groups was (5 761±303), (5 336±367), (4 146±379) and (3 658±365) cells/mm2, respectively, and there were statistically significant differences between the groups (F=234.94, P=0.00). The anterior stromal cell density in the four groups was (836±30), (727±57), (544±59) and (360±47) cells/mm2, respectively, and there were statistically significant differences between the groups (F=535.08, P=0.00). The hexagonal endothelium cell rate in the four groups was 62.0%±5.5%, 51.1%±3.7%, 40.2%±4.0% and 27.8%±3.9%, respectively, and the Langerhans cell density was (1.5±0.6), (4.2±1.3), (6.8±2.1) and (10.9±2.1) cells/mm2, respectively; there were statistically significant differences between the groups (F=342.28, 179.78, all P=0.00). There was no statistically significant difference between the groups in the corneal endothelial cell density (F=1.58, P=0.20). Conclusions: In type 2 diabetes patients with diabetic retinopathy, the corneal nerve fiber and branch density can be significantly reduced, and the density of the hexagonal corneal endothelial cells, epithelial basal cells and anterior stromal cells can also decrease. Langerhans cells may be involved in the development diabetic keratopathy. (Chin J Ophthalmol, 2020, 56: 754-760).

PPP1R21 homozygous null variants associated with developmental delay, muscle weakness, distinctive facial features, and brain abnormalities.

We present 3 children with homozygous null variants in the PPP1R21 gene. A 3-year-old girl had profound developmental delay, hypotonia and weakness, poor feeding, recurrent chest infections and respiratory failure, rotatory nystagmus, absent reflexes, and a homozygous nonsense variant c.2089C>T (p.Arg697*). A 2-year-old boy had profound developmental delay, weakness and hypotonia, recurrent chest infections and respiratory distress, undescended testes, rotatory nystagmus, hyporeflexia, and a homozygous nonsense variant c.427C>T (p.Arg143*). An 11-year-old girl with profound developmental delay, weakness and hypotonia, stereotypic movements, growth failure, hyporeflexia, and a homozygous frameshift variant c.87_88delAG (p.Gly30Cysfs*4). In addition, these children shared common facial features (thick eyebrows, hypertelorism, broad nasal bridge, short nose with upturned nasal tip and broad low-hanging columella, thick lips, low-set ears, and coarse facies with excessive facial hair), and brain abnormalities (cerebellar vermis hypoplasia, ventricular dilatation, and reduced white matter volume). Although PPP1R21 has not yet been linked to human disease, the consistency in the phenotype of individuals from unrelated families, the nature of the variants which result in truncated proteins, and the expected vital role for PPP1R21 in cellular function, all support that PPP1R21 is a novel disease-associated gene responsible for the phenotype observed in these individuals.

Pressure-Induced Superconductivity in Elemental Ytterbium Metal.

Ytterbium (Yb) metal is divalent and nonmagnetic (4f^{14} configuration). Under pressure its valence increases significantly leading to the expectation that magnetic instabilities and other highly correlated electron effects may appear before a stable trivalent state is reached (4f^{13} configuration). We carried out electrical resistivity and ac magnetic susceptibility measurements to 179 GPa over the temperature range 1.4-295 K. No evidence for magnetic order is observed. However, Yb becomes a superconductor at 86 GPa with T_{c}≃1.4 K, increasing to 4.6 K at 179 GPa. X-ray absorption spectroscopy shows that Yb remains mixed valent to at least 125 GPa, pointing to an active role of f electrons in the emergence of superconductivity in this simple, elemental solid.

Decomposition Kinetics of Omethoate in Blood.

OBJECTIVES: To study the decomposition kinetics of omethoate in blood. METHODS: The acetonitrile precipitated protein was added into the blood, with the chromatographic column of a Waters BEH C18 column （2.1 mm×50 mm, 1.7 µm）, the mobile phase of 5 mmol/L ammonium acetate aqueous solution-methanol, and the gradient elution with a flow rate of 0.3 mL/min and injection volume of 2 µL. With electrospray ionization （ESI） source and positive ion detection, qualitative and quantitative analyses were taken using multi-reaction monitoring mode. Omethoate standard was added into blank human blood to the mass concentrations of 0.78, 1.40, 2.30, 4.50, and 7.20 µg/mL, and each mass concentration was preserved at 3 temperatures of -20 ℃, 4 ℃, and 20 ℃, respectively. The content of omethoate was detected at different time points （0, 1, 3, 4, 7, 11, 15, 24, 32, 40, 48, 64, 80, 96, and 120 d）. RESULTS: Different concentrations of omethoate all showed a descended trend in human blood under different temperature conditions. The decomposition in storage environment of -20 ℃, 4 ℃, and 20 ℃ was fit to a one-compartment open model with a first-order kinetic process, which could be expressed as Ct=Coe-αt, with the calculated theoretical values of omethoate concentration close to the measured values. CONCLUSIONS: All concentrations of omethoate are decomposed in the blood, which vary a lot in different preservation conditions. It is suggested that blood samples should be frozen and detected timely in suspected omethoate poisoning cases.

Decreased microRNA-198 expression and its prognostic significance in human glioma.

Previous evidence has shown the association of aberrant miR-198 expression with tumorigenesis and progression of many human malignancies. However, its involvement in human glioma is still unclear. Therefore, the aim of the current study was to investigate the expression and function of miR-198 in human gliomas. Using real-time quantitative RT-PCR, we examined miR-198 expression in 122 pairs of human gliomas and matched non-neoplastic brain tissues. The association of miR-198 expression with clinicopathological factors was also analyzed. Then, the effects of miR-198 on the biological behavior of glioma cells in vitro were evaluated. Our results showed that miR-198 expression was significantly downregulated in gliomas compared with corresponding non-neoplastic brain tissues (P < 0.001). Furthermore, low levels of miR-198 were associated with a higher WHO grade and lower Karnofsky performance status (KPS) score. A multivariate Cox regression analysis identified decreased miR-198 expression as an independent factor predicting poor prognosis for glioma patients. Lastly, in vitro functional analysis revealed that overexpression of miR- 198 in U87 cells reduced cell proliferation, promoted cell apoptosis, and inhibited cell invasion and migration. Taken together, these findings indicate that miR-198 may act as a tumor suppressor in human glioma, and may serve as a novel target for molecular therapies of this disease.

Decreased expression of miR-874 and its tumor suppressive function in human colorectal cancer.

Dysregulation of miRNAs is associated with cancer development and progression. For example, aberrant expression of miR-874 has been found in some types of cancer. However, miR-874 expression and its clinical significance in colorectal cancer (CRC) have not yet been explored. The aim of the current study was to explore the effects of miR-874 in CRC tumorigenesis and development. Quantitative reverse-transcription PCR was performed to evaluate miR-874 levels in CRC cell lines and in 135 pairs of primary human CRC specimens and adjacent noncancerous tissues. The association of miR-874 expression with clinicopathological factors and prognosis was also analyzed. Furthermore, the effects of miR-874 on the biological behavior of CRC cells in vitro were investigated. Our results revealed that miR-874 expression was significantly downregulated in CRC cancer tissues and cell lines. Decreased miR-874 expression was significantly associated with larger tumor size, deeper invasion depth, and advanced TNM stage in vivo. Additionally, low miR-874 expression in CRC was an independent predictor of poor survival. Moreover, overexpression of miR-874 inhibited cell proliferation, invasion, and migration, and promoted cell apoptosis of the SW620 CRC cell line in vitro. Taken together, these findings indicate that miR-874 may act as a tumor suppressor in CRC, and may serve as a novel therapeutic target for miR-based therapy.

[Effect of moluodan on gastric secretion in atrophic gastritis rats].

Objective: To investigate the effect of moluodan on gastric secretion and the underlying mechanism of moluodan in treating atrophic gastritis. Method: According to the random number table, 120 healthy male specific-pathogen-free (SPF) Sprague-Dawley rats were divided into 4 groups: control group, model group, moluodan low-dose group, and moluodan high-dose group, with 30 rats in each group. The control group was administered with normal saline 2 ml/d by gavage, the other three groups were administered with 2% sodium salicylate 1 ml/d, 20 mol/L sodium deoxycholate 1 ml/d, and N-methyl-N'-nitro-N-nitrosoguanidine (MNNG) 200 mg/kg for every 10 days. And 16 weeks later, the control group and model group were treated with normal saline 2 ml/d by gavage, meanwhile the moluodan low-dose group was treated with moluodan 0.9 g·kg-1·d-1and the high-dose group was treated with moluodan 1.8 g·kg-1·d-1, continuously for 12 weeks. Ten rats of each group were sacrificed at the end of 4, 8, 12 weeks. The effect of moluodan on atrophic gastritis was observed. The secretion function of gastric mucosa was assessed through detecting the numbers of gastrin-secreting cells (G cells) and somatostatin-secreting cell (D cells) in gastric mucosa using immunochemical staining, and measuring the serum levels of gastrin (GAS) and somatostatin (SS) using enzyme-linked immunosorbent assay (ELISA). Results: After 8 weeks, the numbers of G and D cells in gastric mucosa in the moluodan high-dose group significantly increased compared with the model group[(0.617±0.114) vs (0.476±0.116) cells/mm2, (0.504±0.084) vs (0.369±0.148) cells/mm2, both P<0.05]; the numbers of G and D cells in gastric mucosa in the low-dose group increased after 12-week's treatment[(0.674±0.129) vs (0.528±0.103) cells/mm2, (0.526±0.087) vs (0.371±0.058) cells/mm2, both P<0.05]. The serum GAS levels increased markedly after 8 weeks in the moluodan high-dose group and after 12 weeks in the low-dose group[(1.313±0.080) ng/ml vs (0.964±0.080) ng/ml, (1.202±0.124) ng/ml vs (0.909±0.054) ng/ml, both P<0.01]; the serum SS levels in both high- and low-dose groups were significantly lower than in the model group after 8-week's treatment[(2.376±0.199) ng/ml, (2.238±0.155) ng/ml vs (2.605±0.183) ng/ml, both P<0.05]. Conclusion: Moluodan may treat atrophic gastritis by repairing G and D cells in gastric mucosa and thus increasing serum levels of GAS.

Superior semicircular canal dehiscence in relation to the superior petrosal sinus: a potential cause of pulsatile tinnitus.

AIM: To examine the association between superior semicircular canal dehiscence (SSCD) and pulsatile tinnitus (PT). MATERIALS AND METHODS: Two SSCD groups included 408 unilateral persistent PT patients, and 511 controls undergoing head and neck dual-phase contrast-enhanced computed tomography (DP-CECT) for reasons other than PT. The prevalence of type I (no the superior petrosal sinus running through the dehiscence) and type II (superior semicircular canal dehiscence in relation to the superior petrosal sinus) SSCD was analysed using chi-square test. RESULTS: SSCD was identified in 5.1% (21/408) of PT ears, significantly different from 2% (8/408) of non-PT ears and 0.7% (7/1022) of controls. There was no significant difference in SSCD prevalence between non-PT ears in the PT group and controls. In the PT group, 15/21 ears were type II SSCD; 6/21 ears were type I. Fifteen combined non-PT and control ears with SSCD included two type II and 13 type I SSCD. The prevalence of type II SSCD in PT ears was significantly higher than that of non-PT ears in both groups, and the prevalence of type I SSCD in PT ears was similar to that of non-PT ears in both groups. CONCLUSION: Compared with type I SSCD, there may be a causal relationship between type II SSCD and PT.

Mechanisms for pressure-induced crystal-crystal transition, amorphization, and devitrification of SnI4.

The pressure-induced amorphization and subsequent recrystallization of SnI4 have been investigated using first principles molecular dynamics calculations together with high-pressure (119)Sn nuclear resonant inelastic x-ray scattering measurements. Above ∼8 GPa, we observe a transformation from an ambient crystalline phase to an intermediate crystal structure and a subsequent recrystallization into a cubic phase at ∼64 GPa. The crystalline-to-amorphous transition was identified on the basis of elastic compatibility criteria. The measured tin vibrational density of states shows large amplitude librations of SnI4 under ambient conditions. Although high pressure structures of SnI4 were thought to be determined by random packing of equal-sized spheres, we detected electron charge transfer in each phase. This charge transfer results in a crystal structure packing determined by larger than expected iodine atoms.

Improved efficacy of warm-white light-emitting diode luminaires.

We present a novel approach to address one of the technical hurdles the current light-emitting diode (LED) lighting field faces: the packaged efficacy of warm-white LEDs is 20%-30% lower than that of cool-white LEDs depending on the color rendering index. With a differentiated luminaire design in combination with a new class of nano materials, we have greatly improved the efficacy of warm white by 15% at the luminaire system level, which translates to less energy being required to achieve the same light output, and thus offers a more energy-efficient solution. Reliability test of the luminaire shows no performance degradation within the tested period of more than 3000 h. A modeling has been developed to predict the optical performance of luminaires incorporating the nano materials, which agrees well with the experimental data and serves as a powerful tool for designing luminaires with targeted performance.

The ratio of transforming growth factor-ß1/bone morphogenetic protein-7 in the progression of the epithelial-mesenchymal transition contributes to rat liver fibrosis.

This study was designed to show whether rat liver epithelial cells could undergo epithelial-mesenchymal transition (EMT), thereby directly contributing to liver fibrosis. The role of the ratio of transforming growth factor-ß1 (TGF-ß1)/bone morphogenetic protein-7 (BMP-7) was evaluated in the progression of EMT or mesenchymal-epithelial transition. Primary rat liver epithelial cells were stimulated with different ratios of TGF-ß1/BMP-7 and examined for evidence of transition to a mesenchymal or epithelial phenotype. Liver sections were labeled to detect antigens associated with liver epithelial cells [E-cadherin (E-cad)], EMT [fibroblast-specific protein-1 (FSP-1), vimentin], myofibroblasts [α-smooth muscle actin (α-SMA)], and intracellular signal-transduction mediated by forming liver fibrosis undergo EMT, resulting in the formation of invasive fibroblasts; this process may be driven or impeded by a response to local TGF-ß1 or BMP-7. BMP-7 downregulated α-SMA and phosphorylated Smad2/3. Stimulation of cultured cells with TGF-ß1 induced the expression of pSmad2/3, FSP-1, and α-SMA. Stimulation of cultured cells with BMP-7 induced the expression of E-cad. We demonstrated that the cells upregulated E-cad release compared with untreated cells, but TGF-ß1 was different. We found that the equilibrium of the ratio of TGF-ß1/BMP-7 was 1/10. In summary, the mechanism for this process was not determined. Demonstration of the contribution of what the ratio of TGF-ß1/BMP-7 induced to EMT to the chronic liver diseases would provide a new basis for understanding pathogenesis and potential treatment.

Large-scale analysis of factors influencing nonalcoholic fatty liver disease and its relationship with liver enzymes.

Serum liver enzyme levels are often used effectively for the evaluation of nonalcoholic fatty liver disease (NAFLD). We aimed to investigate the associations between serum liver enzyme levels and risks for NAFLD in over 8000 cases in a large-scale analysis. A cross-sectional survey with multiple stages and random samplings was performed from May 2007 to May 2009 on 8102 workers at Tongji University. A questionnaire was given, assessments of physical measurements, plasma glucose, lipid profiles, and liver enzymes were made, and real-time liver ultrasounds conducted. The prevalence of NAFLD in Tongji University was 22.2%. It was higher in males than in females (P = 0.0023). The body mass index, waist-to-hip ratio, serum total triglycerides, serum total cholesterol, alanine aminotransferase (ALT), aspartate aminotransferase (AST), and gamma-glutamyl transferase (GGT) values were all higher in the NAFLD group than in the control group. For moderate and severe NAFLD patients, the ALT, AST and GGT values were significantly increased, high density lipoprotein cholesterol was decreased, and drinking much, heavy entertainment and less exercise were more prevalent (P < 0.001). There were strong correlations between serum liver enzyme levels and NAFLD (P < 0.001), with GGT being a more sensitive marker for NAFLD than ALT or AST. ALT and GGT were independent predictors for NAFLD, and GGT was a better predictor than ALT for NAFLD.

Association of CD14-260 polymorphisms, red-complex periodontopathogens and gingival crevicular fluid cytokine levels with cyclosporine A-induced gingival overgrowth in renal transplant patients.

UNLABELLED: BACKGROUD AND OBJECTIVE: Genetic factors may influence the colonization of pathogenic bacteria, therefore increasing the risk for the initiation and development of periodontal disease. The present study was carried out to investigate the association of CD14-260 polymorphisms, subgingival microbiota, and gingival crevicular fluid (GCF) cytokine levels with cyclosporine A (CsA)-induced gingival overgrowth (GO) in renal transplant patients. MATERIAL AND METHODS: A total of 204 patients were dichotomized into two groups: 124 with GO and 80 without GO. The CD14-260 polymorphisms were measured using an allele-specific PCR method. The levels of periodontal pathogens were determined by real-time PCR of subgingival samples. GCF levels of IL-1ß and sCD14 were detected by ELISA. RESULTS: The frequency of CD14-260 genotype CT + TT was found to be similar in both groups. Patients with GO presented increased prevalence of Pg, Td, and Tf (red complex) and significantly higher levels of interleukin -1ß than those without GO. Patients with GO carrying CT + TT genotypes were found to have higher frequencies of Pg, Td, and Tf than those carrying the CC genotype. Furthermore, in the presence of red complex, CT + TT genotypes were associated with higher interleukin -1ß levels and severe GO. Multiple logistic regression analysis demonstrated that the severity of GO is not dependent on age, gender and pharmacological variables, being only associated with CD14-260 genotype and red complex periodontopathogens. CONCLUSION: No association between CD14-260 polymorphisms and the prevalence of GO was revealed in renal transplant patients administered CsA. However, CD14-260 CT + TT genotypes are associated with the prevalence of red complex periodontopathogens in patients with GO, and may thus play some role in the development of severe CsA-induced GO.

Sox9 is required for cartilage formation.

Chondrogenesis results in the formation of cartilages, initial skeletal elements that can serve as templates for endochondral bone formation. Cartilage formation begins with the condensation of mesenchyme cells followed by their differentiation into chondrocytes. Although much is known about the terminal differentiation products that are expressed by chondrocytes, little is known about the factors that specify the chondrocyte lineage. SOX9 is a high-mobility-group (HMG) domain transcription factor that is expressed in chondrocytes and other tissues. In humans, SOX9 haploinsufficiency results in campomelic dysplasia, a lethal skeletal malformation syndrome, and XY sex reversal. During embryogenesis, Sox9 is expressed in all cartilage primordia and cartilages, coincident with the expression of the collagen alpha1(II) gene (Col2a1) . Sox9 is also expressed in other tissues, including the central nervous and urogenital systems. Sox9 binds to essential sequences in the Col2a1 and collagen alpha2(XI) gene (Col11a2) chondrocyte-specific enhancers and can activate these enhancers in non-chondrocytic cells. Here, Sox9 is identified as a regulator of the chondrocyte lineage. In mouse chimaeras, Sox9-/- cells are excluded from all cartilages but are present as a juxtaposed mesenchyme that does not express the chondrocyte-specific markers Col2a1, Col9a2, Col11a2 and Agc. This exclusion occurred cell autonomously at the condensing mesenchyme stage of chondrogenesis. Moreover, no cartilage developed in teratomas derived from Sox9-/- embryonic stem (ES) cells. Our results identify Sox9 as the first transcription factor that is essential for chondrocyte differentiation and cartilage formation.

MRCP and 3D LAVA imaging of extrahepatic cholangiocarcinoma at 3 T MRI.

Extrahepatic cholangiocarcinoma (CCA) is a primary bile duct malignant tumour with poor prognosis. Familiarity with their varied imaging characteristics can be helpful in developing a correct diagnosis and in optimal treatment planning, and thus contribute to a better prognosis. The purpose of this article is to illustrate the typical appearances of extrahepatic CCA on magnetic resonance cholangiopancreatography (MRCP) and three-dimensional (3D) LAVA (liver acquisition with volume acceleration) sequences at 3 T magnetic resonance imaging (MRI), and to discuss the superiority of the two techniques in the diagnosis of CCA.

[A comparative study on the radiographs of spiral CT and cone-beam CT in temporomandibular joint osteoarthrosis].

Objective: To observe and compare the radiographs of spiral CT and cone-beam CT (CBCT) in the imaging of temporomandibular joint osteoarthrosis (TMJOA) and to explore the difference between CBCT and spiral CT in detection accuracy so as to provide references for clinical diagnosis and treatment. Methods: A total of 52 patients with TMJOA diagnosed in the Department of Oral and Maxillofacial Surgery, General Hospital of Chinese PLA, from January 2018 to December 2019 were selected. There were 10 males and 42 females, with an average age of 38.6 years (21-70 years). All patients underwent spiral CT and CBCT examinations. Two oral radiologists and two oral and maxillofacial surgeons measured and evaluated the joint spaces and condylar bone lesions of each side of temporomandibular joint. According to the presence or absence of osteoarthrosis, the patients were divided into osteoarthrosis group (92 sides) and non osteoarthrosis group (12 sides). The mean size of joint spaces and the detection rate of lesions were compared between the two groups. SPSS 20.0 was used to analyze the data. Results: There was no significant difference between the measurements of joint space size and joint position in the spiral CT group and the CBCT group (P>0.05). The mean size of the anterior space and the ratio of the posterior condyle in the osteoarthrosis side were larger than that in the normal side. The LR index was smaller in the osteoarthrosis side than that in the normal side indicating that the position of the posterior condyle in the osteoarthrosis side was deviated. However, the difference was not statistically significant (P>0.05). Both spiral CT and CBCT showed good consistency in displaying condylar osteopathy. The most common types of condylar osteopathy was surface defect. The detection rates of defects by spiral CT were surface erosion (85.6%, 89/104), articular surface flattening and shortening (82.7%, 86/104), subcortical sclerosis (40.4%, 42/104), osteophyte (40.4%, 42/104) and subcortical cyst (11.5%, 12/104) respectively. The detection rates of defects by CBCT were surface erosion (88.5%, 92/104), articular surface flattening and shortening (86.5%, 90/104), subcortical sclerosis (35.6%, 37/104), osteophyte (41.3%, 43/104) and subcortical cyst (11.5%, 12/104). There was no statistical difference between the two groups (P>0.05), respectively. Conclusions: Both spiral CT and CBCT showed good accuracies in displaying the osteopathy of TMJOA and the sizes of the joint spaces measured by spiral CT and CBCT were basically the same. Both spiral CT and CBCT could be used as a routine diagnostic method for TMJOA.

Therapeutic cancer vaccines for pediatric malignancies: advances, challenges, and emerging technologies.

Though outcomes for pediatric cancer patients have significantly improved over the past several decades, too many children still experience poor outcomes and survivors suffer lifelong, debilitating late effects after conventional chemotherapy, radiation, and surgical treatment. Consequently, there has been a renewed focus on developing novel targeted therapies to improve survival outcomes. Cancer vaccines are a promising type of immunotherapy that leverage the immune system to mediate targeted, tumor-specific killing through recognition of tumor antigens, thereby minimizing off-target toxicity. As such, cancer vaccines are orthogonal to conventional cancer treatments and can therefore be used alone or in combination with other therapeutic modalities to maximize efficacy. To date, cancer vaccination has remained largely understudied in the pediatric population. In this review, we discuss the different types of tumor antigens and vaccine technologies (dendritic cells, peptides, nucleic acids, and viral vectors) evaluated in clinical trials, with a focus on those used in children. We conclude with perspectives on how advances in combination therapies, tumor antigen (eg, neoantigen) selection, and vaccine platform optimization can be translated into clinical practice to improve outcomes for children with cancer.

Microdeletions including YWHAE in the Miller-Dieker syndrome region on chromosome 17p13.3 result in facial dysmorphisms, growth restriction, and cognitive impairment.

BACKGROUND: Deletions in the 17p13.3 region are associated with abnormal neuronal migration. Point mutations or deletion copy number variants of the PAFAH1B1 gene in this genomic region cause lissencephaly, whereas extended deletions involving both PAFAH1B1 and YWHAE result in Miller-Dieker syndrome characterised by facial dysmorphisms and a more severe grade of lissencephaly. The phenotypic consequences of YWHAE deletion without deletion of PAFAH1B1 have not been studied systematically. METHODS: We performed a detailed clinical and molecular characterization of five patients with deletions involving YWHAE but not PAFAH1B1, two with deletion including PAFAH1B1 but not YWHAE, and one with deletion of YWHAE and mosaic for deletion of PAFAH1B1. RESULTS: Three deletions were terminal whereas five were interstitial. Patients with deletions including YWHAE but not PAFAH1B1 presented with significant growth restriction, cognitive impairment, shared craniofacial features, and variable structural abnormalities of the brain. Growth restriction was not observed in one patient with deletion of YWHAE and TUSC5, implying that other genes in the region may have a role in regulation of growth with CRK being the most likely candidate. Using array based comparative genomic hybridisation and long range polymerase chain reaction, we have delineated the breakpoints of these nonrecurrent deletions and show that the interstitial genomic rearrangements are likely generated by diverse mechanisms, including the recently described Fork Stalling and Template Switching (FoSTeS)/Microhomology Mediated Break Induced Replication (MMBIR). CONCLUSIONS: Microdeletions of chromosome 17p13.3 involving YWHAE present with growth restriction, craniofacial dysmorphisms, structural abnormalities of brain and cognitive impairment. The interstitial deletions are mediated by diverse molecular mechanisms.