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Background Progressive encephalomyelitis with rigidity and myoclonus (PERM) is a rare and life-threatening autoimmune disease of the central nervous system. So far, only ten cases of PERM have been reported in children worldwide, including the one in this study. Case presentation We report a case of an 11-year-old boy with PERM with an initial presentation of abdominal pain, skin itching, dysuria, urinary retention, truncal and limb rigidity, spasms of the trunk and limbs during sleep, deep and peripheral sensory disturbances, and dysphagia. A tissue-based assay using peripheral blood was positive, demonstrated by fluorescent staining of mouse cerebellar sections. He showed gradual and persistent clinical improvement after immunotherapy with intravenous immunoglobulin, steroids, plasmapheresis and rituximab. Conclusions We summarized the diagnosis and treatment of a patient with PERM and performed a literature review of pediatric PERM to raise awareness among pediatric neurologists. A better comprehension of this disease is required to improve its early diagnosis, treatment, and prognosis.

Background Atrial septal defect often become more severe when encountered in genetic syndromes. Congenital disorder of glycosylation type 1a is an inherited metabolic disorder associated with mutations in PMM2 gene and can affect almost all organs. Cardiac abnormalities vary greatly in congenital disorder of glycosylation type 1a and congenital heart defects have already been reported, but there is little knowledge about the effect of this inherited disorder on an existing congenital heart defect. Herein we report for the first time on a baby with congenital disorder of glycosylation type 1a with atrial septal defect and make a comparison of changes in atrial septal defect by follow-ups to the age of 3. Case presentation Our patient was an 8-month-old Han Chinese boy. At the initial visit, he presented with recurrent lower respiratory infection, heart murmur, psychomotor retardation, inverted nipples, and cerebellar atrophy. Echocardiography revealed a 8 mm secundum atrial septal defect with left-to-right shunt (Qp/Qs ratio 1.6). Enzyme testing of phosphomannomutase 2 demonstrated decreased levels of phosphomannomutase 2 activities in fibroblasts. Whole exon sequencing showed he was heterozygous for a frameshift mutation (p.I153X) and a missense mutation (p.I132T) in PMM2 gene. The diagnosis of congenital disorder of glycosylation type 1a with atrial septal defect was issued. Now, he is 3-years old at the time of this writing, with the development of congenital disorder of glycosylation type 1a (cerebellar atrophy become more severe and the symptom of nystagmus emerged), the size of atrial septal defect increased to 10 mm and the Qp/Qs ratio increased to 1.9, which suggested exacerbation of the atrial septal defect. Congenital heart defect-associated gene sequencing is then performed and shows there are no pathogenic mutations, which suggested intrinsic cardiac factors are not the cause of exacerbation of the atrial septal defect in our patient and it is reasonable to assume congenital disorder of glycosylation type 1a can worsen the situation of the existing atrial septal defect. Conclusions This report highlights the view that congenital disorders of glycosylation type 1a should be excluded when faced with congenital heart defect with cerebellar atrophy or neurodevelopmental delay, especially when the situation of congenital heart defect becomes more and more severe.

For a non-Gaussian Levy model, it is shown that if the model exists a trivial arbitrage-free interval, option pricing by mean correcting method is always arbitrage-free, and if the arbitrage-free interval is non-trivial, this pricing method may lead to arbitrage in some cases. In the latter case, some necessary and sufficient conditions under which option price is arbitrage-free are obtained.

Based on the best wavelet packet decomposition of images, a new universal steganalysis method with high detection correct ratio is proposed. First, the best wavelet packet decomposition of image based on the Shannon entropy information cost function is made. Second, high order absolute characteristic function moments of histogram extracted from the coefficient subbands obtained by best wavelet packet decomposition are regarded as features. Finally, these features are processed and a back-propagation （BP） neural network is designed to classify original and stego images. Three different steganalysis algorithms for three different cases of background and application condition are presented. To validate the performance of the proposed method, a series of experiments are made for six kinds of typical steganography methods, i.e. LSB, LTSB, PMK, Jsteg, F5 and JPHide. Results show that, the average detection accuracy of the proposed method exceeds at least 6.4% and up to 15.4% and has a better universal performance than its closest competitors. Furthermore, the proposed method can provide reference for designing the pattern recognition and classification algorithm based on best wavelet packet decomposition.

This paper investigates the detection of adaptive image steganography. Firstly, the sample pair analysis model of multiple least-significant bits (MLSB) replacement is analyzed, and the conditions under which the adaptive steganography can be detected via this model are given. For the category of adaptive steganography satisfying these conditions, a general quantitative steganalysis method is presented based on specific areas and sample pair analysis. Then, for a typical adaptive steganography, some concrete methods are proposed to select specific areas and trace sets of sample pairs, and estimate the stego modification ratios. Experimental results show that the proposed methods can estimate the stego modification ratio accurately. This verifies the validity of the proposed general quantitative steganalysis method.

The methylcytosine dioxygenases TET proteins （TET1, TET2, and TET3） play important regulatory roles in neural function. In this study, we investigated the role of TET proteins in neuronal differentiation using Neuro2a cells as a model. We observed that knockdown of TET1, TET2 or TET3 promoted neuronal differentiation of Neuro2a cells, and their overexpression inhibited VPA （valproic acid）-induced neuronal differentiation, suggesting all three TET proteins negatively regulate neu- ronal differentiation of Neuro2a cells. Interestingly, the inducing activity of TET protein is independent of its enzymatic activity. Our previous studies have demon- strated that srGAP3 can negatively regulate neuronal differentiation of Neuro2a cells. Furthermore, we revealed that TET1 could positively regulate srGAP3 expression independent of its catalytic activity, and srGAP3 is required for TET-mediated neuronal differentiation of Neuro2a cells. The results presented here may facilitate better understanding of the role of TET proteins in neuronal differentiation, and provide a possible therapy target for neuroblastoma.

Terahertz (THz=1012Hz) radiation has attracted wide attention for its unprecedented sensing ability and its noninvasive and nonionizing properties. Tremendous strides in THz instrumentation have prompted impressive breakthroughs in THz biomedical research. Here, we review the current state of THz spectroscopy and imaging in various biomedical applications ranging from biomolecules, including DNA/RNA, amino acids/peptides, proteins, and carbohydrates, to cells and tissues. We also address the potential biological effects of THz radiation during its biological applications and propose future prospects for this cutting-edge technology. THz spectroscopy has proven to be an innovative tool for providing new insights into the hydration shell in the solvation dynamics of protein solutions. THz in-line digital holography, THz near-field imaging modality, and THz endoscope prototypes have been utilized to identify abnormal tissues faster and more accurately. Increasing applications of artificial modeling and numerical computation are becoming essential supplements for THz biological effect studies.

Elemene is a second-line broad-spectrum anti-tumour drug that has been used in China for more than two decades. However, its main anti-tumour ingredient, β-elemene, has disadvantages, including excessive lipophilicity and relatively weak anti-tumour efficacy. To improve the anti-tumour activity of β-elemene, based on its minor molecular weight character, we introduced furoxan nitric oxide (NO) donors into the β-elemene structure and designed six series of new generation β-elemene NO donor hybrids. The synthesised compounds could effectively release NO , displayed significant anti-proliferative effects on U87MG, NCI-H520, and SW620 cell lines. In the orthotopic glioma model, compound significantly and continuously suppressed the growth of gliomas in nude mice, and the brain glioma of the treatment group was markedly inhibited (>90%). In short, the structural fusion design of NO donor and β-elemene is a feasible strategy to improve the anti-tumour activity of β-elemene.

Heat shock protein 60 (HSP60) is a chaperonin with essential functions for cell physiology and survival, and its expression correlates with prognosis in a number of malignancies. The aim of this study is to determine the relationship of HSP60 status with clinicopathological parameters and prognosis in gastric cancer. The levels of HSP60 and matrix metallopeptidase 9 (MMP-9) antigen was evaluated by immunohistochemistry in 223 gastric carcinoma samples. The association between HSP60 and MMP-9, clinicopathological parameters, and prognosis of gastric cancer was examined. The level of HSP60 protein was significantly associated with depth invasion, lymph node metastasis and stage of disease (all P<0.05). Both univariate and multivariate analyses revealed that HSP60 was an independent prognostic factor for both overall survival (OS) and recurrence-free survival (RFS) (both P<0.05). Furthermore, HSP60 overexpression was associated with a poor prognosis in patients with advanced gastric cancer in different risk groups. Moreover, HSP60 was significantly correlated with MMP-9 among 223 gastric cancer tissues (P<0.001). Patients who had HSP60 overexpression, in which tumor cells displayed high invasiveness, had poor OS and shorter RFS. HSP60 plays an important role on tumor aggressiveness and prognosis, and may act as a promising target for prognostic prediction.

In rotating machinery, the condition of rolling bearings is paramount, directly influencing operational integrity. However, the literature on the fault evolution of rolling bearings in their nascent stages is notably limited. Addressing this gap, our study establishes an innovative nonlinear dynamic model for early fault evolution of rolling bearings based on collision impact. Firstly, considering the fault evolution characteristics, the influence of the rolling element and fault structure, the dynamic model of early fault evolution between the rolling element and the local fault is established. Secondly, according to the Hertzian contact deformation theory, a nonlinear dynamic model of rolling bearings expressed as mass-spring is established. Thirdly, the energy contribution method is used to integrate the fault evolution model and the nonlinear dynamic model of the rolling bearing. A nonlinear dynamic model of early fault evolution of the rolling bearing is proposed by using the Lagrangian equation. Comparing the simulation results of the nonlinear dynamic with the experimental results, it can be seen that the numerical model can effectively predict the evolution process and vibration characteristics of the fault evolution of rolling bearings in the early stage.