[Responses of blood parameters and hemoglobin subtypes in plateau zokors and plateau pikas to different altitude habitats].

The plateau zokor (Myospalax baileyi) and plateau pika (Ochotona curzoniae) are native species unique to the Qinghai-Tibetan Plateau with successful adaptation to the hypoxic environment. In this study, the number of red blood cells, hemoglobin concentration, mean hematocrit and mean volume of red blood cells were measured in plateau zokors and plateau pikas at different altitudes. Hemoglobin subtypes of two plateau animals were identified by mass spectrometry sequencing. The forward selection sites in two animals' hemoglobin subunits were analyzed by PAML4.8 program. Homologous modeling was used to analyze the effect of forward selection sites on the affinity of hemoglobin to oxygen. The adapting strategies of plateau zokors and plateau pikas to hypoxia at different altitudes were analyzed through comparing blood parameters between the two species. The results indicated that, with increasing altitudes, plateau zokors responded to hypoxia by increasing red blood cell count and decreasing red blood cell volume, while plateau pikas took the opposite strategies to plateau zokors. In erythrocytes of plateau pikas, both adult α2ß2 and fetal α2ε2 hemoglobins were identified, while erythrocytes of plateau zokors only had adult α2ß2 hemoglobin, however the affinities and the allosteric effects of the hemoglobin of plateau zokors were significantly higher than those of plateau pikas. Mechanistically, in the α and ß subunits of hemoglobin of plateau zokors and pikas, the numbers and the sites of the positively selected amino acids as well as the side chain groups polarities and orientations of the amino acids differed significantly, which may result in the difference of the affinities to oxygen of hemoglobin between plateau zokors and pikas. In conclusion, the adaptive mechanisms to respond to hypoxia in blood properties of plateau zokors and plateau pikas are species-specific.

[Comparison of the composition and content of pulmonary surfactant among plateau zokors, plateau pikas and rats].

In the present study, the composition and content of pulmonary surfactant (PS) were analyzed to explore the hypoxia adaptation mechanism in plateau zokors (Myospalax baileyi) and plateau pikas (Ochotona curzoniae). 36 plateau zokors and plateau pikas were trapped alive at the Laji Mountain in Guide County, Qinghai Province (at the altitude of about 3 600 m), and 36 Sprague-Dawley (SD) rats were purchased from the experimental animal center of Lanzhou University (at the altitude of about 1 500 m). All animals were lavaged after laboratory anesthesia, the blood in lung tissues was fully washed out and the lung tissues were then taken out to obtain the bronchoalveolar lavage fluid by bronchoalveolar lavage. The composition and content of phospholipids in the PS of three different kinds of animals were analyzed by using high performance liquid chromatography; the protein composition, content and types in the PS were analyzed by G-250 Coomassie brilliant blue method, polyacrylamide gel electrophoresis (PAGE) and mass spectrometry; the dissolved oxygen in the PS solutions were determined by using dissolved oxygen electrode. The results showed that the total contents of phospholipids in the PS were successively increased among plateau zokors, plateau pikas and SD rats (P < 0.05), while the total content of proteins successively decreased (P < 0.05). There were five phospholipids identified in the PS, including linoleic palmitoylphosphatidylcholine (LPPC), dipalmitoylphosphatidylcholine (DPPC), phosphatidylglyerol (PG), phosphatidylinositol (PI) and phosphatidylserine (PSe), but the relative contents of these phospholipids were different. The relative content of LPPC was successively increased among plateau zokors, plateau pikas and SD rats (P < 0.01). The relative contents of DPPC, PG and PI in the PS of plateau zokors were significantly higher than those of plateau pikas (P < 0.01), while insignificant differences between plateau pikas and SD rats (P > 0.05). The relative content of PSe had no significant differences between plateau zokors and plateau pikas (P > 0.05), but both were significantly higher than that of SD rats (P < 0.01). The serum albumin (SA) was identified in the PS of three kinds of animals, including homologous tetramer protein containing heme, which is composed of hemoglobin ß subunit, in plateau zokors and plateau pikas. Immunoglobulin (Ig) heavy chain was found in PS of plateau zokors and SD rats. The content of Ig heavy chain in plateau zokor was significantly higher than that in SD rats (P < 0.01), and the content of protein containing heme was significantly higher than that in plateau pikas (P < 0.05). The amount of dissolved oxygen was successively decreased in the PS among plateau zokors, plateau pikas and SD rats (P < 0.01), but it was significantly higher than that in saline (P < 0.01). These results suggest that the total content of proteins in the PS of plateau zokors and plateau pikas was significantly higher, while the total content of phospholipids was significantly decreased. There were high content of homologous tetramer protein containing heme in the PS of plateau zokors and plateau pikas. The relative content of DPPC, the main component of phospholipids, was significantly increased in plateau zokors. The changes of PS component and content improve the adaptability of the two plateau animals in hypoxia environment.

Neuromyelitis optica spectrum disorders with multiple brainstem manifestations: a case report.

BACKGROUND: Multiple brainstem manifestations have been rarely reported during the same attack in neuromyelitis optica spectrum disorders (NMOSD). CASE PRESENTATION: We describe a 39-year-old Asian woman presenting multiple brainstem manifestations including intractable nausea and vomiting, vertigo, diplopia, facial palsy, hypogeusia, ophthalmoplegia, hemiplegia, dysphagia and tonic spasm during the same attack. Hypogeusia was transient and recovered without any immunotherapy. The brain MRIs showed progressive multiple lesions in the brainstem. NMO-IgG (aquaporin4-antibody, AQP4-Ab) were positive in both serum and cerebral spinal fluid. The symptoms and signs were controlled after immunosuppressive therapy. No relapse happened during the 15-month follow-up. CONCLUSION: This report emphasizes multiple brainstem manifestations during the same attack in NMOSD and the most characteristic symptom was reversible hypogeusia.

Transient, recurrent, white matter lesions in x-linked Charcot-Marie-tooth disease with novel mutation of gap junction protein beta 1 gene in China: a case report.

BACKGROUND: Transient white matter lesions have been rarely reported in X-linked Charcot-Marie-Tooth disease type 1. CASE PRESENTATION: We describe a 15-year-old boy who presented transient and recurrent weakness of the limbs for 5 days. His mother, his mother's mother and his mother's sister presented pes cavus. MRI and electrophysiology were performed in the proband. Gap junction protein beta l gene was analyzed by PCR-sequencing in the proband and his parents. The electrophysiological studies showed a mixed demyelinating and axonal sensorimotor neuropathy. MRI showed white matter lesions in the internal capsule, corpus callosum and periventricular areas, which showed almost complete resolution after two months. T278G mutation in Gap junction protein beta l gene was detected in the proband and his mother. CONCLUSION: This case report highlights that the novel T278G mutation of Gap junction protein beta l maybe could result in X-linked Charcot-Marie-Tooth disease type 1 with predominant leucoencephalopathy. The white matter changes in MRI of X-linked Charcot-Marie-Tooth disease type 1 patient are reversible.

AI-based green technology implementation simulation for achieving carbon neutrality: exploring the role of subsidies and knowledge management.

This study investigates the role of green technology implementation (GTI) based on artificial intelligence (AI) at the household level to achieve carbon neutrality by addressing gaps in the existing research. The research focuses on understanding how education on green consumption preferences, green invention and emission impacts can optimally influence AI-based GTI decisions. Through behavioural analysis at the household level, this study quantifies the effects of education and preferences on emissions and proposes subsidies as accelerators for carbon-neutral transitions. Furthermore, the study employs regression analysis and simulation-based optimisation, which are then validated against prior methodologies, with a focus on Punjab, Pakistan. Utilising a simple random sampling technique, approximately 1000 households were surveyed to represent the province's diverse demographics comprehensively. Findings reveal that higher education levels correlate with less enthusiasm for AI-based GTI. Simulations measured optimal subsidy levels by striking a balance between encouraging green behaviour and technological adoption. By integrating diverse factors and AI-based GTI optimisation, this study defines important thresholds for education and subsidies, thus highlighting their pivotal role in advancing AI-based green technologies and sustainable household practices. This research significantly enhances the understanding of the complex relationship between AI-based GTI decisions and educational influences, thereby contributing to the advancement of environmental sustainability.

Correlation between serum inflammatory factors and cognitive function in patients with high-altitude polycythemia: A case-control study.

The purpose of this study is to investigate the serum inflammatory factors in patients with high-altitude polycythemia (HAPC) and their correlation with cognitive function. The subjects were recruited and placed into a HAPC group and control group. Serum samples were collected, and inflammatory factors (interleukin-1beta [IL-1ß], monocyte chemoattractant protein-1 [MCP-1], and tumor necrosis factor-alpha [TNF-α]) were measured using ELISA kits. The mini-mental State Examination (MMSE) was used to assess cognitive function. According to the MMSE scores, HAPC group was further divided into normal cognitive function group (HNCF) and cognitive dysfunction group (HCDF). In comparison with the control group, the MMSE scores in the HAPC group were significantly low (P < .05), whereas the serum levels of IL-1ß, MCP-1, and TNF-α were significantly high (P < .01). Among the HAPC group (n = 60), 21 belonged to the HCDF and 39 belonged to the HNCF. Compared with the HNCF, the IL-1ß, MCP-1, and TNF-α in the HCDF were significantly increased (P < .01). The Pearson correlation analysis showed that inflammatory factors were positively correlated with hemoglobin, and negatively correlated with MMSE. Serum inflammatory cytokines IL-1, MCP-1, and TNF-α were increased in HAPC, and HAPC exhibited cognitive dysfunction. Considering chronic hypoxia environment influences the change of the red blood cell metabolic and inflammatory factor, red blood cells and inflammatory factor in plateau is likely to be affected by patients with vascular lesions, increase cognitive impairment.

An 85-year-old woman with Miller Fisher syndrome.

Miller Fisher's syndrome (MFS) commonly presents in the fourth and fifth decades and are rare in people over 70 years. An 85-year-old female with no significant medical history presented with upper extremity anesthesia, ptosis, and unsteady gait. The patient had a history of hypertension and diabetes mellitus. Physical examination showed bilateral total external ophthalmoplegia, areflexia, and cerebellar ataxia. Radiological and laboratory studies were unremarkable. Lumbar puncture showed albuminocytological dissociation. The combined history, physical examination, and lumbar puncture results established a presumptive diagnosis of MFS. Intravenous immunoglobulin was given for 5 days. The patient gradually improved 10 days after the onset of symptoms. Ophthalmoplegia had fully recovered after 6 months. To the best of our knowledge, this case represented the oldest patient with MFS.

[Clinical features and antimicrobial resistance of community-acquired pneumonia caused by Klebsiella pneumoniae in infants].

OBJECTIVE: To study the clinical features and antimicrobial resistance of community-acquired pneumonia caused by Klebsiella pneumoniae in infants. METHODS: The clinical data of 65 infants with community-acquired pneumonia caused by Klebsiella pneumoniae between 2007 and 2011 were retrospectively studied. RESULTS: Of the 65 infants, 37 cases (57%) were aged ≤3 months, 17 cases (26%) over 4 months, 7 cases (11%) over 7 months and 4 cases (6%) between 13 and 24 months. There were no significant differences in clinical manifestations and chest X-ray features between the infants with community-acquired pneumonia caused by Klebsiella pneumoniae and those with other bacterial pneumonia. Forty strains (62%) of ESBLs-producing Klebsiella pneumoniae were detected. Klebsiella pneumoniae was 100% sensitive to imipenem, meropenem and amikacin but resistant to penicillins and cephalosporins. The resistance rates of ESBLs-producing strains to penicillins, cephalosporins, amoxicillin/clavulanic acid, ampicillin/sulbactam, compound sulfamethoxazole, gentamycin, ciprofloxacin and aztreonam were significantly higher than for non-ESBLs-producing strains. ESBLs-producing strains also showed multiple-drug resistance. CONCLUSIONS: Community-acquired pneumonia caused by Klebsiella pneumoniae is common in infants aged ≤3 months. ESBLs-producing strains are prevalent in community-acquired pneumonia caused by Klebsiella pneumoniae and demonstrate both high rates of drug resistance and multiple-drug resistance.

Response to Different Oxygen Partial Pressures and Evolution Analysis of Apoptosis-Related Genes in Plateau Zokor (Myospalax baileyi).

The plateau zokor (Myospalax baileyi) is a native species of the Qinghai-Tibet Plateau that spends its entire life underground in sealed burrows with hypoxic conditions. The present study aimed to assess the sequence characteristics of apoptosis-related genes and the response to different oxygen partial pressures (pO2) in plateau zokor and Sprague-Dawley rats. The sequences of the p53-induced protein with a death domain (Pidd), p53-upregulated modulator of apoptosis (Puma), insulin-like growth factor binding protein 3 (Igfbp3), and apoptosis protease-activating factor 1 (Apaf1) were evaluated concerning homology and convergent evolution sites, and their mRNA levels were evaluated in different tissues under 14.13 (3,300 m) and 16.12 kPa (2,260 m) pO2 conditions. Our results showed that, (1) the sequences of the apoptosis-related genes in plateau zokor were highly similar to those of Nannospalax galili, followed by Rattus norvegicus; (2). Pidd, Puma, Igfbp3, and Apaf1 of plateau zokor were found to have five, one, two, and five convergent sites in functional domains with N. galili, respectively. Lastly (3), under low pO2, the expression of Pidd and Puma was downregulated in the lung of plateau zokors. In turn, Igfbp3 and Apaf1 were upregulated in the liver and lung, and Puma was upregulated in the skeletal muscle of plateau zokor under low pO2. In Sprague-Dawley rats, low pO2 downregulated Puma and Apaf1 expression in the liver and downregulated Igfbp3 and Puma in the lung and skeletal muscle separately. In contrast, low pO2 upregulated Pidd expression in the liver and skeletal muscle of Sprague-Dawley rats. Overall, the expression patterns of Apaf1, Igfbp3, and Puma showed the opposite pattern in the liver, lung, and skeletal muscle, respectively, of plateau zokor as compared with Sprague-Dawley rats. In conclusion, for the long-time adaptation to hypoxic environments, Pidd, Puma, Igfbp3, and Apaf1 of plateau zokor underwent convergent evolution, which we believe may have led to upregulation of their levels under low oxygen partial pressures to induce apoptosis, so as to suppress tumorigenesis under hypoxic environments in plateau zokor.

Vitamin D3 Metabolic Enzymes in Plateau Zokor (Myospalax baileyi) and Plateau Pika (Ochotona curzoniae): Expression and Response to Hypoxia.

Vitamin D3 (D3) is produced endogenously from 7-dehydrocholesterol by irradiation and is an important secosteroid for the absorption of calcium and phosphate. Lithocholic acid (LCA) increases intestinal paracellular calcium absorption in a vitamin D receptor-dependent manner in vitamin D-deficient rats. The plateau zokor (Myospalax baileyi), a strictly subterranean species, and plateau pika are endemic to the Qinghai-Tibet Plateau. To verify whether the zokors were deficient in D3 and reveal the effects of hypoxia on D3 metabolism in the zokors and pikas, we measured the levels of 25(OH)D3, calcium, and LCA, and quantified the expression levels of D3 metabolism-related genes. The results showed an undetectable serum level of 25(OH)D3 and a significantly higher concentration of LCA in the serum of plateau zokor, but its calcium concentration was within the normal range compared with that of plateau pika and Sprague-Dawley rats. With increasing altitude, the serum 25(OH)D3 levels in plateau pika decreased significantly, and the mRNA and protein levels of CYP2R1 (in the liver) and CYP27B1 (in the kidney) in plateau pika decreased significantly. Our results indicate that plateau zokors were deficient in D3 and abundant in LCA, which might be a substitution of D3 in the zokor. Furthermore, hypoxia suppresses the metabolism of D3 by down-regulating the expression of CYP2R1 and CYP27B1 in plateau pika.

A homotetrameric hemoglobin expressed in alveolar epithelial cells increases blood oxygenation in high-altitude plateau pika (Ochotona curzoniae).

The plateau pika (Ochotona curzoniae) is native to the Qinghai-Tibet Plateau. In this study, the gene that encodes a heme-binding protein in the pulmonary surfactant (PS) of the pika is identified. The protein is a homotetrameric hemoglobin (δ4) encoded by HBD (δ). HBD is expressed in alveolar epithelial type II (ATII) and type I (ATI) cells, upregulated by hypoxia. δ4 is secreted into alveolar cavities through osmiophilic multilamellar bodies. HBD expression is downregulated by RNAi, which significantly increases hypoxia-inducible factor 1α expression in lung tissue and red blood cells and hemoglobin and blood lactate concentrations but significantly decreases arterial partial pressure of oxygen (PaO2). Our results indicate that plateau pikas physiologically show hypoxemia when HBD expression is downregulated. Therefore, specific HBD expression in the lungs helps plateau pikas to obtain oxygen under hypoxia by maintaining higher PaO2. These findings provide insights into the adaptive mechanisms of plateau pikas to withstand high-altitude environments.

A New Homotetramer Hemoglobin in the Pulmonary Surfactant of Plateau Zokors (Myospalax Baileyi).

The plateau zokor (Myospalax baileyi) is a native species to the Qinghai-Tibetan Plateau, inhabiting hypoxia and hypercapnia sealed subterranean burrows that pose several unique physiological challenges. In this study, we observed a novel heme-containing protein in the pulmonary surfactant (PS) of plateau zokor, identified the encoding gene of the protein, predicted its origination and structure, verified its expression in alveolar epithelial cells, and determined the protein's affinity to oxygen and its effect on the oxygen-dissolving capability in the PS of plateau zokors. The protein is an unusual homotetramer hemoglobin consisting of four γ-like subunits, and the subunit is encoded by a paralog gene of γ, that is γ-like. The divergence time of γ-like from γ is estimated by the molecular clock to be about 2.45 Mya. The generation of γ-like in plateau zokors might well relate to long-time stress of the high land hypoxia. Unlike γ, the γ-like has a hypoxia response element (HRE) and a lung tissue-specific enhancer in its upstream region, and it is expressed specifically in lung tissues and up-regulated by hypoxia. The protein is named as γ4-like which is expressed specifically in Alveolar epithelial type II (ATII) cells and secreted into the alveolar cavities through the osmiophilic multilamellar body (LBs). The γ4-like has a higher affinity to oxygen, and that increases significantly oxygen-dissolving capability in the PS of plateau zokors by its oxygenation function, which might be beneficial for the plateau zokors to obtain oxygen from the severe hypoxia environments by facilitating oxygen diffusion from alveoli to blood.

Anti-N-methyl-D-aspartate receptor encephalitis with serum anti-thyroid antibodies and IgM antibodies against Epstein-Barr virus viral capsid antigen: a case report and one year follow-up.

BACKGROUND: Anti-N-methyl-D-aspartate receptor encephalitis is an increasingly common autoimmune disorder mediated by antibodies to certain subunit of the N-methyl-D-aspartate receptor. Recent literatures have described anti-thyroid and infectious serology in this encephalitis but without follow-up. CASE PRESENTATION: A 17-year-old Chinese female patient presented with psychiatric symptoms, memory deficits, behavioral problems and seizures. She then progressed through unresponsiveness, dyskinesias, autonomic instability and central hypoventilation during treatment. Her conventional blood work on admission showed high titers of IgG antibodies to thyroglobulin, thyroid peroxidase and IgM antibodies to Epstein-Barr virus viral capsid antigen. An immature ovarian teratoma was found and removal of the tumor resulted in a full recovery. The final diagnosis of anti-N-methyl-D-aspartate receptor encephalitis was made by the identification of anti-N-methyl-D-aspartate receptor antibodies in her cerebral spinal fluid. Pathology studies of the teratoma revealed N-methyl-D-aspartate receptor subunit 1 positive ectopic immature nervous tissue and Epstein-Barr virus latent infection. She was discharged with symptoms free, but titers of anti-thyroid peroxidase and anti-thyroglobulin antibodies remained elevated. One year after discharge, her serum remained positive for anti-thyroid peroxidase and anti-N-methyl-D-aspartate receptor antibodies, but negative for anti-thyroglobulin antibodies and IgM against Epstein-Barr virus viral capsid antigen. CONCLUSIONS: Persistent high titers of anti-thyroid peroxidase antibodies from admission to discharge and until one year later in this patient may suggest a propensity to autoimmunity in anti- N-methyl-D-aspartate receptor encephalitis and support the idea that neuronal and thyroid autoimmunities represent a pathogenic spectrum. Enduring anti-N-methyl-D-aspartate receptor antibodies from admission to one year follow-up but seroreversion of Epstein-Barr virus viral capsid antigen IgM may raise the important issue of elucidating the triggers and boosters of anti- N-methyl-D-aspartate receptor encephalitis.

[Expression of tau-related protein in spinal cord of patients with Alzheimer's disease].

OBJECTIVE: To study the expression of tau-related protein in spinal cord of Chinese patients with Alzheimer's disease. METHODS: Gallays-Braak stain and immunohistochemical study for tau protein (AT8) were carried out in the spinal cord tissue (T2, T8, T10, L2 and S2 segments) of 3 Chinese patients with Alzheimer's disease. Seven age-matched cases without evidence of dementia or neurologic disease were used as controls. RESULTS: Neurofibrillary tangles were identified in the neurons of anterior horn in 2 Alzheimer's disease cases but none was observed in the controls. Tau-positive axons and astroglia were detected in all Alzheimer's disease cases. Tau immunoreactivity in spinal cord of the patients correlated with that in brain tissue. CONCLUSION: The expression of tau-related protein is demonstrated in the spinal cord of Alzheimer's disease patients suggesting that axonal transport defect may play a role in the pathogenesis of Alzheimer's disease.

Does high risk mean high loss: Evidence from flood disaster in southern China.

Southern China has suffered from flood disasters for over sixty years, which results in tremendous socio-economic loss. With the development of economy and the improvement of disaster reduction, both the exposure and potential loss of flood disaster are increasing. However, previous studies only focus on risk assessment, few has examined the comparison of potential risk and the actual losses caused by it. To this end, a method combing entropy weight and TOPSIS based on flood data (2008 to 2018) in China's national and provincial disaster database is applied to analysis flood risk and resulting loss in southern China. By using disaster system dimensions of hazard, exposure and vulnerability, the effect of natural, economic and social factors on flood risk are also examined. Results indicate that: (1) flood risk in southern China is relatively low from 2008 to 2014 and becomes severe since 2016; (2) the resulting losses of flood disasters in southern China are optimistic during most of the selected years in the study period; (3) flood risk is not always in line with the resulting loss; and (4) flood disasters in southern China are categorized into high-risk and low-loss situation, low-risk and high-loss situation, and the situation with the same level of risk and loss. To the best of our knowledge, this is the first study to assess southern China on a regional scale from both temporal and spatial perspectives, and has compensated for the lack of comparative research on flood risk and the resulting loss. In practice, our findings can protrude the priorities of flood prevention both in flood-prone areas and specific measures, which is conducive to improve the efficiency of resource allocation.

A Novel Variation in the Mitochondrial Complex I Assembly Factor NDUFAF5 Causes Isolated Bilateral Striatal Necrosis in Childhood.

Background: Bilateral striatal necrosis (BSN) is characterized by symmetrical degeneration, predominantly of the caudate and putamen nucleus, in the basal ganglia. It is associated with numerous acquired and hereditary neuro-developmental and motor dysfunction-related pathological conditions. BSN results in high morbidity and mortality among infants and children, and its diagnosis is clinically challenging due to several overlapping disease phenotypes. Therefore, a precise genetic diagnosis is urgently needed for accurate genetic counseling and improved prognostic outcomes as well. Objective: To identify novel missense mutations in the NDUFAF5 gene as a cause of childhood BSN in members of a Chinese family and summarize the clinical characteristics of patients with the NDUFAF5 gene mutations. Methods: This study included a large family living in a remote northwestern area of China. Three siblings developed a neurological disorder characterized by generalized dystonia within the first decade of their lives. Cerebral computed tomography (CT) and magnetic resonance imaging (MRI) showed bilateral lesions of the putamen. Biochemical and genetic approaches were used to identify the cause of BSN. Results: Sequence analysis showed no pathogenic variation in PANK2, SLC25A19, SLC19A3, and NUP62 genes and in the entire mitochondrial genome as well. Whole-exome sequencing revealed compound heterozygous mutations consisting of NDUFAF5:c.425A > C(p.E142A) and c.836T > G (p.M279R). The father, a healthy sister, and a healthy brother of the affected siblings carried the c.836T > G mutation, and the mother carried the c.425A > C mutation. These variants were absent in 100 ethnically matched non-BSN controls. In silico analysis demonstrated that the E142A and M279R mutations in NDUFAF5 protein significantly perturbed the normal conformation of the protein due to alterations in the hydrogen bonding patterns around the evolutionarily conserved catalytic domains, leading to its loss of function in the early stage of mitochondrial complex I assembly. Conclusions: We identified a novel compound heterozygous mutation (c.425A > C and c.836T > G) in the NDUFAF5 gene as the potential cause of autosomal recessive childhood BSN, which extended the pathogenic variation spectrum of the NDUFAF5 gene. This study provides substantial evidence for further improvement of genetic counseling and better clinical management of BSN affected individuals.

Corrigendum: A Novel Variation in the Mitochondrial Complex I Assembly Factor NDUFAF5 Causes Isolated Bilateral Striatal Necrosis in Childhood.

[This corrects the article DOI: 10.3389/fneur.2021.675616.].

[Microbiological etiology in children with community acquired pneumonia].

OBJECTIVE: To study the distribution of pathogenic microorganisms of community acquired pneumonia (CAP) in children. METHODS: Respiratory secretion and blood specimens were collected in 1167 children with CAP within 4 hrs of admission. Mycoplasma pneumonia and Chlamydia trachomatis were detected by RT-PCR in respiratory secretion specimens. Respiratory syncytial virus (RSV-IgM) and Adenovirus (ADV-IgM) were tested using ELISA in blood samples. RESULTS: A total of 308 strains of bacteria were isolated from the respiratory tract secretions, with gram positive strains of 53.6% and gram negative strains of 46.4%. The top five bacteria strains detected were Streptococcus pneumoniae (35.1%), Escherichia coli (11.7%), Staphylococcus aureus (8.8%), Klebsiella pneumonia (6.5%) and Moraxelle catarrhalis (5.8%) in turn. Beta-lactamase and ESBLs producing strains accounted for 30.1% in the top five bacteria strains. The non-bacteria pathogens were found in 281 specimens (24.1%). Respiratory syncytial virus accounted for the most prevalent pathogen (19.3%). The mixed infection of respiratory syncytial virus and Streptococcus pneumoniae was common (35.2%). The infection rate from most of pathogenic microorganisms among children under the age of one was higher than that in children over one year old. CONCLUSIONS: Respiratory syncytial virus and Streptococcus pneumoniae are the major pathogens of CAP in children. The risk of pathogenic microorganism infections in children under the age of one is higher than that of children over one year old.

Naphtho[2,3-b]furan-4,9-dione synthesis via palladium-catalyzed reverse hydrogenolysis.

A reverse hydrogenolysis process has been developed for two-site coupling of 2-hydroxy-1,4-naphthoquinones with olefins to produce naphtha[2,3-b]furan-4,9-diones and hydrogen (H2). The reaction is catalyzed by commercially available Pd/C without oxidants and hydrogen acceptors, thereby providing an intrinsically waste-free approach for the synthesis of functionalized and potentially biologically relevant naphtha[2,3-b]furan-4,9-diones.

Cell death and proliferation in NF-kappaB p50 knockout mouse after cerebral ischemia.

The transcription factor NF-kappaB is a key regulator of inflammation and cell survival. NF-kappaB activation increases following cerebral ischemia. We previously showed accelerated aging process in NF-kappaB p50 subunit knockout (p50 -/-) mice under physiological condition. The present investigation concerned the role of NF-kappaB p50 gene in ischemia-induced neuronal cell death. In an animal model of permanent middle cerebral artery occlusion (MCAO), infarct formation, apoptotic cell death and cell proliferation were examined in adult wild type (WT) and p50-/- mice. The ischemic infarct volume was significantly larger in p50-/- mice than that in WT mice. Consistently, the numbers of cells in the penumbra region positive to terminal deoxynucleotidyltransferase (TdT)-mediated dUTP-biotin nick end-labeling (TUNEL) and caspase-3 staining were significantly more in p50-/- mice than that in WT mice. To identify proliferation after cerebral ischemia, bromodeoxyurindine (BrdU) was intraperitoneal injected daily after MCAO. Ischemia increased BrdU positive cells in the penumbra, subventricular zone, corpus callosum, and cerebral cortex, while cell proliferation was hampered in p50-/- mice. These results suggest that NF-kappaB signaling is a neuroprotective mechanism and may play a role in cell proliferation in the stroke model of permanent MCAO.