[Research progress of brain-computer interface application paradigms based on rapid serial visual presentation].

Rapid serial visual presentation (RSVP) is a type of psychological visual stimulation experimental paradigm that requires participants to identify target stimuli presented continuously in a stream of stimuli composed of numbers, letters, words, images, and so on at the same spatial location, allowing them to discern a large amount of information in a short period of time. The RSVP-based brain-computer interface (BCI) can not only be widely used in scenarios such as assistive interaction and information reading, but also has the advantages of stability and high efficiency, which has become one of the common techniques for human-machine intelligence fusion. In recent years, brain-controlled spellers, image recognition and mind games are the most popular fields of RSVP-BCI research. Therefore, aiming to provide reference and new ideas for RSVP-BCI related research, this paper reviewed the paradigm design and system performance optimization of RSVP-BCI in these three fields. It also looks ahead to its potential applications in cutting-edge fields such as entertainment, clinical medicine, and special military operations.

A Novel ANK1 Mutation in a Neonatal Hereditary Spherocytosis Case: Diagnostic Challenges and Familial Genetic Analysis.

Hereditary spherocytosis (HS) is a congenital disease in which erythrocyte membranes are abnormal, with ANK1 defects as the main cause. The diagnosis of neonatal HS is difficult due to poor phenotypic specificity. Therefore, a detailed inquiry into family history may be helpful for diagnosis. Here, we describe a familial case of HS caused by a novel mutation in ANK1. The proband is a premature infant of Chinese Han ethnicity characterized by progressive aggravation of anemia and jaundice. The disease was caused by a frameshift mutation (c.3392delT/p.Leu1131Argfs*15) of ANK1 that was identified by genetic testing. In vitro functional experiments showed that this variant may seriously affect the protein expression and further expanded the mutation spectrum of ANK1-HS. In this case, we emphasize the diagnostic value of early-intervention genetic testing for neonatal hemolytic anemia with a family history.

The Chinese version of the Quality of Life in Childhood Epilepsy Questionnaire-16-C (QOLCE-16-C): translation, validity, and reliability.

BACKGROUND: Epilepsy is one of the most common chronic neurological diseases that adversely impact the quality of life of patients and their families. The "Quality of Life of Childhood Epilepsy Questionnaire" (hereinafter referred to as "QOLCE-16") is a 16-item measure that was designed to assess health-related quality of life (HRQOL) among children with epilepsy. The purpose of the study was to translate and evaluate the psychometric properties of the QOLCE-16. METHODS: The 10 steps of Principles of Good Practices for translation and cultural adaptation of measures were adopted to translate the QOLCE-16 into Chinese. After that, item analysis, floor effect and ceiling effect, internal consistency, test-retest reliabilities, content validity and construct validity were conducted to test its applicability in children with epilepsy in China. A total of 435 native Chinese-speaking parents with children who had epilepsy from one children's hospital were invited to take part in the study, including a cognitive interview sample of 5 and a validation sample of 430. RESULTS: A total of 414 objects were enrolled in our study for psychometric testing. The results of the item analysis revealed QOLCE-16-C to have good discrimination, the floor effect and ceiling effect were 0.2% and 1.0% respectively, and each item was significantly related to the total scale (P < 0.001). The Cronbach's α value was 0.938 and the test-retest reliability was 0.724. For validity, results showed that the QOLCE-16-C had good content validity. Exploratory factor analysis indicated it was reasonable that the QOLCE-16-C consists of four dimensions after rotation. Confirmatory factor analysis demonstrated good construct validity (χ2/df = 1.698, GFI = 0.913, CFI = 0.974, RMSEA = 0.058). CONCLUSION: The Chinese version of QOLCE-16-C appears to be a culturally appropriate, valid and reliable tool to assess the health-related quality of life of children with epilepsy in China.

The influence of soil particle size distribution and clay minerals on ammonium nitrogen in weathered crust elution-deposited rare earth tailing.

Even after being abandoned for many years, a large number of weathered crust elution-deposited rare earth (WCED-RE) tailings continue to release ammonia nitrogen (AN) pollution into their surrounding environments. However, the influences of particle size distribution and clay minerals on AN pollution caused by these tailings have been insufficiently studied, and its causes are poorly understood. In this study, soil samples at different depths (5, 7, 9, 11 and 14 m) were collected from a rare earth tailing in Ganzhou City, Jiangxi Province, China. Particles were screened by size into six groups (2-1, 1-0.5, 0.5-0.25, 0.25-0.1, 0.1-0.075 and < 0.075 mm), and AN forms were extracted. The results showed that as soil particle size decreases, both soil specific surface area and clay content increase, leading to stronger AN enrichment ability. With increased sampling depth, the distribution of clay across the six particle fractions became more uniform, such that the accumulation of AN in soil with fine particle size was less obvious. Clay minerals with different capacities for AN enrichment vary with sampling depth. This variation is responsible for the profile of AN distribution in the mine, where AN first increases and then decreases as vertical depth is increased. Although AN content was highest at 11 m, water soluble AN content was higher in the upper part of the completely weathered layer (5 and 7 m), which poses a higher environmental risk. This study provides significant information to deepen our understanding of the distribution characteristics of AN and its main influencing factors, as well as a foundation for the prevention and remediation of nitrogen pollution from WCED-RE tailings.

[Analysis of SLC35A2 gene variant in a child with congenital disorder of glycosylation type IIm].

OBJECTIVE: To investigate the clinical features and SLC35A2 variant of a case of congenital disorder of glycosylation type IIm (CDG-IIm), and to identify the possible causes of the disease. METHODS: Trio-whole exome sequencing (WES) was used to analyze the gene variant of the children and their parents. The suspicious gene variants were screened for Sanger verification and the bioinformatics prediction was used to analyze the hazard of variant. RESULTS: The clinical manifestations of the child were epilepsy, global growth retardation, nystagmus, myocarditis and other symptoms. MRI showed brain dysplasia such as wide frontal temporal sulcus and subarachnoid space on both sides. Echocardiography showed left ventricular wall thickening and patent foramen ovale. According to the results of gene detection, there was a heterozygous missense variant c.335C>A (p.Thr112Lys) in SLC35A2 gene. The parents were wild-type at this locus, which was a de novo variant. At the same time, there was no report of this variant in the relevant literature, which was a novel variant in SLC35A2 gene. According to the genetic variant guidelines of American College of Medical Genetics and Genomics, SLC35A2 gene c.335C>A (p.Thr112Lys) variant was predicted to be likely pathogenic (PS2+PM2+PP3). CONCLUSION: The variant of SLC35A2 gene c.335C>A(p.Thr112Lys) may be the cause of the disease in the child.

Clinical outcomes of warfarin anticoagulation after balloon dilation alone for the treatment of Budd-Chiari syndrome complicated by old inferior vena cava thrombosis.

BACKGROUND: To evaluate the safety and clinical efficacy of warfarin anticoagulation after balloon dilation alone for the treatment of Budd-Chiari syndrome (BCS) complicated by old inferior vena cava (IVC) thrombosis. METHODS: From January 2008 to November 2013, 19 BCS patients complicated with old IVC thrombosis were treated with balloon dilation followed by oral administration of anticoagulant warfarin. Follow-up was performed at 1 week, then 1, 2, 3, 6, and 12 months after balloon dilation, and then annually thereafter. IVC patency and morphologic changes of the old thrombus were examined by ultrasound, and clinical symptoms and signs were determined by clinical examinations during follow-up. RESULTS: Successful IVC balloon dilation was achieved in the 19 patients (100%). Inferior vena cavography demonstrated the patency of IVC lumen, and the size of the old thrombus was not altered. The mean pressure gradient between IVC and the right atrium was reduced from 27.5 ± 3.0 cm H2O (range, 22-35) before treatment to 5.4 ± 1.3 cm H2O (range: 2-7) after treatment (t = 41.6, P < 0.05; 1 cm H2O = 0.098 kPa). Patients were followed up as outpatients for an average of 15.9 ± 14.4 months (range, 3-66). Anticoagulation with warfarin was well tolerated in all patients after balloon dilation alone. Of the 19 patients, complete resolution of the old thrombus was achieved in 12 patients and partial resolution was achieved in 7 patients. Color Doppler ultrasound showed that 17 patients had IVC lumen patency, and 2 patients had IVC reocclusion. None of the patients had recurrence of thrombosis, symptomatic pulmonary embolism, and bleeding complications throughout the follow-up period. CONCLUSIONS: Our results indicate that warfarin anticoagulation after balloon dilation alone is a safe and effective therapy for BCS patients with old IVC thrombosis.

Comparison of diagnostic criteria for sepsis-associated acute kidney injury in the pediatric intensive care unit: a retrospective cohort study.

Background: pRIFLE (Pediatric Risk, Injury, Failure, Loss, End Stage Renal Disease), KDIGO (Kidney Disease Improving Global Outcomes) and pROCK (Pediatric Reference Change Value Optimized for AKI) are diagnostic criteria used for acute kidney injury (AKI) incidence evaluation. The aim of this study was to explore the diagnostic consistency, incidence and mortality rate, clinical signs, and influencing factors of renal injury related to sepsis in children diagnosed by three different AKI diagnostic criteria, and then evaluate which one was more valuable. Methods: A retrospective analysis was performed on the clinical data of children with severe sepsis. The patients were diagnosed and staged according to the 2007 pRIFLE standard, the 2012 KDIGO standard, and the 2018 pROCK standard. The clinical characteristics and prognosis of children with different stages of sepsis were compared between the three diagnostic standards. Results: A total of 62 patients with sepsis were included. Blood stream infection is common (11 cases, 17.74%). According to pRIFLE, KDIGO, and pROCK standards, the incidence of sepsis-associated AKI (SA-AKI) was 74.2%, 67.7%, and 56.5%, respectively. The pRIFLE had the highest diagnostic rate of early detection of SA-AKI. There was no statistical difference in SA-AKI incidence or staging consistency between the pRIFLE and KDIGO groups (κ=0.0671; κ>0.60); the consistency of SA-AKI diagnoses across the three standards was good (all P values <0.05), and pROCK demonstrated a higher specificity. A high Pediatric Risk of Mortality (PRISM) score and high procalcitonin level were independent risk factors. Shock and renal replacement therapy were independent risk factors for SA-AKI death. Death from admission to 28 days after admission was used as an endpoint to draw a survival graph, which revealed that the AKI group had a significantly higher risk of death than did the non-AKI group. Conclusions: The consistency of diagnosing SA-AKI across the three classification criteria was similar, and mortality rate increased with increased SA-AKI staging. The pRIFLE criteria were more sensitive in the early detection of SA-AKI, while the pROCK had higher specificity. There was no significant difference between the pRIFLE and KDIGO in terms of incidence, diagnosis, or staging of SA-AKI.

Successful management of Mycobacterium abscessus pneumonia in a 53-day-old immunocompetent infant.

Pulmonary infection due to Mycobacterium abscessus complex (MABC) usually occurs in children with underlying risk factors including cystic fibrosis (CF), chronic lung disease, and immunocompromised status, but rarely in immunocompetent children without underlying lung disease, especially in infants. We present a case of MABC pulmonary disease (MABC-PD) in an otherwise healthy 53-day-old male infant with one week of cough and respiratory distress. Computed tomography showed multiple masses across both lungs. Isolated mycobacteria from his bronchoalveolar lavage fluid were identified as MABC. We describe our complete evaluation, including immunodeficiency evaluation incorporating whole exome sequencing and our therapeutic process given complicated susceptibility pattern of the M. abscessus isolate, and review literature for MABC-PD in immunocompetent children. The infant was successfully treated through prolonged treatment with parenteral Amikacin, Cefoxitin, Linezolid, and Clarithromycin, combined with inhaled Amikacin.

Long-term variations in surface ozone at the Longfengshan Regional Atmosphere Background Station in Northeast China and related influencing factors.

Surface ozone (O3) is a crucial air pollutant that affects air quality, human health, agricultural production, and climate change. Studies on long-term O3 variations and their influencing factors are essential for understanding O3 pollution and its impact. Here, we conducted an analysis of long-term variations in O3 during 2006-2022 at the Longfengshan Regional Atmosphere Background Station (LFS; 44.44°N, 127.36°E, 330.5 m a.s.l.) situated on the northeastern edge of the Northeast China Plains. The maximum daily 8-h average (MDA8) O3 fluctuated substantially, with the annual MDA8 decreasing significantly during 2006-2015 (-0.62 ppb yr-1, p < 0.05), jumping during 2015-2016 and increasing clearly during 2020-2022. Step multiple linear regression models for MDA8 were obtained using meteorological variables, to decompose anthropogenic and meteorological contributions to O3 variations. Anthropogenic activities acted as the primary drivers of the long-term trends of MDA8 O3, contributing 73% of annual MDA8 O3 variability, whereas meteorology played less important roles (27%). Elevated O3 at LFS were primarily associated with airflows originating from the North China Plain, Northeast China Plain, and coastal areas of North China, primarily occurring during the warm months (May-October). Based on satellite products of NO2 and HCHO columns, the O3 photochemical regimes over LFS revealed NOx-limited throughout the period. NO2 increased first, reaching peak in 2011, followed by substantial decrease; while HCHO exhibited significant increase, contributing to decreasing trend in MDA8 O3 during 2006-2015. The plateauing NO2 and decreasing HCHO may contribute to the increase in MDA8 O3 in 2016. Subsequently, both NO2 and HCHO exhibited notable fluctuations, leading to significant changes in O3. The study results fill the gap in the understanding of long-term O3 trends in high-latitude areas in the Northeast China Plain and offer valuable insights for assessing the impact of O3 on crop yields, forest productivity, and climate change.

Accuracy of SIRS, age-adapted pSOFA, and quick SOFA scoring systems for predicting outcomes in paediatric patients with sepsis: a meta-analysis.

BACKGROUND: Sepsis is the leading cause of mortality in children. Several scoring systems are used to predict outcome and mortality for pediatric patients with sepsis, but how they compare to each other in terms of sensitivity and specificity is unclear. METHODS: The systematic literature review was performed following PRISMA guidelines. Publically accessible search engines and study databases such as PubMed, CENTRAL (Cochrane Central Register of Controlled Trials), and Google Scholar were scanned for articles published from January 1990 to March 2021 using relevant key words. All relevant studies were analyzed separately by two reviewers. A random-effects model was used to calculate the pooled sensitivity and pooled specificity with a 95% confidence interval (CI). Heterogeneity was evaluated using I2, which estimates the percentage of variation between study results due to heterogeneity rather than sampling error. RESULTS: Eleven studies met inclusion criteria and evaluated the SOFA scoring system. The pooled sensitivity, specificity, and SROC for prediction of mortality were 83% (95% CI: 76%-88%), 72% (95% CI: 60%-81%), and 85% (95% CI: 82%-88%), respectively. Six studies examined the SIRS system. Pooled sensitivity, specificity, and SROC were 80% (95% CI: 64%-90%), 36% (95% CI: 23%-51%), and 59% (95% CI: 55%-63%), respectively. CONCLUSION: This meta-analysis shows that SOFA was superior to SIRS for predicting mortality in PICU patients with sepsis. Additional prospective multi-centric studies are needed to better evaluate and validate this finding.

Toxicity prediction of dioxins and dioxins-like compounds based on the molecular fragments variable connectivity index.

The toxicity of 95 doxins and dioxin-like compounds was investigated by quantitative structure-activity relationship (QSAR) with the molecular fragments variable connectivity index (mfVCI). For each of the four homologues, the models have good fitting (R² > 0.80) and predictive (Q²EXT > 0.80) ability. The models developed from more than one homologues are also satisfactory with R² > 0.80 and Q²EXT > 0.77. The molecular fragments weights have the ability to diagnose the contribution of the molecular fragments to the toxicity of the compounds. The mfVCI may play an important role in the development of molecular descriptors in further QSAR research.

Thrombotic microangiopathy led to acute kidney injury in an infant with hemophagocytic lymphohistiocytosis: A case report.

Hemophagocytic lymphohistiocytosis (HLH) is a rare but fatal clinical syndrome frequently complicated by acute kidney injury (AKI) and acute tubular necrosis. Renal thrombotic microangiopathy (TMA) is a specific pathological feature of childhood HLH and few cases have been reported among infants. The present study presents a rare case of HLH with TMA in an infant. A 15-month-old infant with a week-long history of fever was admitted to hospital. The infant presented with AKI and subsequently a reduction in platelet and hemoglobin levels. TMA was diagnosed by kidney biopsy and the clinical, laboratory and bone marrow biopsy findings met the criteria of HLH. Due to a progressive increase in serum creatinine levels, hemodialysis was initiated on the second day following admission. Dexamethasone was administered to treat both the fever and HLH. The patient's body temperature returned to a normal range and platelet and hemoglobin levels were stable after 14 days of admission. Renal function stabilized on day 21. The results of genetic testing did not identify any disease-related variations. Childhood HLH is a severe condition and mortality can be reduced by early diagnosis and correct treatment. For patients with HLH and AKI, the possible role of TMA should be considered. Renal biopsy can help to identify the cause of AKI and can be performed when the patient's condition is stable.

[Comparison of two tracing method of transplanted mouse embryonic stem cell].

OBJECTIVE: To trace the embryonic stem (ES) cells transplanted into rat brain by labeling the cells with green fluorescent protein (GFP) and by mouse neuronal specific antibody Thy-1 and compare their features. METHODS: For GFP labeling,transfect pEGFP-N1 plasmid containing GFP and anti-neomycin sequences into embryonic stem cell and add neomycin for more than 10 passages. To test the GFP expression in vivo, the GFP-ES was transplanted into healthy rat brain, and the frozen sectioned slides were observed under fluorescence microscope and laser con-focal microscope 21 days later. For the antibody labeling,embryonic stem cells were directly transplanted into the rat brain. The specific mouse thy-1 antibody was used in immunostaining of transplanted cells. For both of the two labeling method, the slides were also examined by double labeling with the antibodies,neuronal nuclei (NeuN) or glial fibrillary acidic protein (GFAP) to identify the differentiation of transplanted cells. RESULTS: Both single ES cell and cell pellets expressed bright green fluorescence the day after plasmid transfection, and more than 30% ES cells were labeled. The GFP-labeled cells could still be found gathered around the infusion channel at least 21 days later, but the GFP fluorescent could not be overlapped with NeuN or GFAP staining. On the contrary, Thy-1 antibody overlapped well with NeuN or GFAP staining. CONCLUSIONS: Liposome-helped plasmid GPF transfection is effective in labeling mouse embryonic stem cell in vivo,but is not effective in showing the differentiated cells. On the contrary, Thy-1 antibody can not only show the transplanted cells, but also trace the transplanted cells after their differentiation.

Fatal congenital tuberculosis owing to late diagnosis of maternal tuberculosis: case report and review of congenital tuberculosis in China.

Congenital tuberculosis (TB) is rare and the prognosis is poor if not detected early. The diagnosis is often delayed owing to non-specific clinical presentation, misdiagnosis and undiagnosed maternal TB during pregnancy. A 12-day-old girl presented with a 5-day history of fever, cough, poor feeding and respiratory distress. Her mother had a cough and fever at 30 weeks gestation which was managed empirically as community-acquired pneumonia without a TB workup. Immediately postpartum, her mother developed a high fever and shortness of breath and required admission to the intensive care unit. The infant was separated from her mother after delivery. The infant's chest radiograph showed bilateral miliary nodules. Thoracic and abdominal computed tomography (CT) showed multiple enlarged lymph nodes and congenital TB was suspected. Early morning gastric aspirate and sputum (obtained through a suction tube) were positive for acid-fast bacilli on smear microscopy and subsequently Mycobacterium tuberculosis was cultured from both specimens. Lumbar puncture was performed and cerebrospinal fluid (CSF) was compatible with TB meningitis. TB-polymerase chain reaction (TB-PCR) was positive. Her mother was diagnosed with miliary TB on postpartum day 17. Both were given anti-TB chemotherapy. Unfortunately, despite the treatment, the infant died from multiple organ dysfunction syndrome (MODS) caused by congenital TB at the age of 14 days. This case highlights the importance of screening pregnant women for TB in regions where it is highly prevalent. A high index of suspicion of maternal and congenital TB is critical to early diagnosis, especially in such regions.

A progressive neurological condition with acquired sea-blue histiocytosis further the diagnosis of Niemann-Pick type C1 in a 10-year-old boy.

Sea-blue histiocytes in bone marrow can be associated with a number of conditions and have indeed often been reported in Niemann-Pick diseases, mostly in Niemann-Pick type B, but also Niemann-Pick type C. Rarely, it was reported to be related to a progressive neurological condition. In this work, early bone marrow aspirations in a boy following the discovery of hepatosplenomegaly at 1 month of age and later isolated splenomegaly did not reveal abnormal cells (which is not uncommon). Numerous sea-blue histiocytes were found in a repeated exam when the child was 10-year old, at a time he had developed a progressive neurological condition with frequent falls, clumsiness, slow and slurred speech, intellectual disability, dystonic movements, and dysphagia. Acquired sea-blue histiocytes should be considered initially on the basis of clinical symptoms. Whole-exome sequencing identified two variants in the NPC1 gene, leading to the diagnosis of Niemann-Pick type C1. This case points out the presence of sea-blue histiocytes in the bone marrow and has helped to reach a diagnosis of NPC1 which was very difficult to establish even after years of study. Given the rarity of this pathology and the variety of clinical presentations, it is important to communicate the possible forms of presentation of this syndrome.

Efficacy of fiberoptic bronchoscopy and bronchoalveolar lavage in childhood-onset, complicated plastic bronchitis.

BACKGROUND: Plastic bronchitis (PB) is a rare, variable, and potentially fatal disease. This study aimed to assess the efficacy of fiberoptic bronchoscopy (FOB) and bronchoalveolar lavage (BAL) in treating children with PB. METHODS: In total, 15 children with PB, between 2012 and 2020, were enrolled in our study. Within 12 hours of admission, FOB and BAL were performed and reviewed under local anesthesia and sedation. Before and after FOB, clinical findings and chest imaging were evaluated. RESULTS: Regarding the onset of symptoms before FOB, all cases had prominent cough for 7.00 ± 4.55 days, and 14 had persistent high fever. In total, 13 cases had complete obstruction from bronchial casts, consistent with consolidated lesions; 2 had partial airway obstruction. Within 3 days, complete resolution was revealed in nine cases. Overall, six cases underwent repeated FOB (range, 2-3 times) for persistent atelectasis and airway obstruction. Except for two cases with type 2 PB, cast histology confirmed type 1 PB for all cases. Only eight children had minor intra- and post-procedure complications. Reverse transcription-polymerase chain reaction for Mycoplasma pneumoniae in sputum and BAL samples were positive in 13 cases. Next-generation sequencing of the BAL samples was positive for adenovirus and Human parainfluenza virus in one case, respectively. During 1 month to 7 years of follow-up, no patient developed PB recurrence, asthmatic attacks, or chronic cough. CONCLUSIONS: Early FOB and BAL were effective in alleviating clinical findings, atelectasis, and airway obstruction. Serial FOB could be performed in patients with recurrent symptoms.

[Effects of different sedation regimens on sedation and inflammatory response in critically ill children with multiple trauma].

OBJECTIVE: To compare the sedation and anti-inflammatory effects of dexmedetomidine and midazolam on critical ill children with multiple trauma. METHODS: A prospective randomized controlled trial was conducted. Sixty-five critical ill children with multiple trauma admitted to pediatric intensive care unit (PICU) of Anhui Province Children's Hospital from January 2014 to September 2016 were enrolled, who were randomly divided into dexmedetomidine group (33 cases) and midazolam group (32 cases). Children of both groups received sufentanil for analgesia. Children in dexmedetomidine group firstly received 1.0 µg/kg intravenous bolus of dexmedetomidine for 10 minutes, then continuous infusion of 0.2-0.7 µg×kg-1×h-1, while in midazolam group children received 1-5 µg×kg-1×min-1 of midazolam in continuous infusion. The goal of sedation was to maintain a Richmond agitation-sedation scale (RASS) score of -1 to 0. The level of serum interleukin (IL-6, IL-8, IL-10, IL-1ß), tumor necrosis factor-α (TNF-α) and C-reactive protein (CRP) were detected by enzyme linked immunosorbent assay (ELISA) at 24, 48, 72 hours after treatment, and the duration of mechanical ventilation, ratio of continuous renal replacement therapy (CRRT), length of stay in the PICU, ratio of sepsis and multiple organ failure (MOF) and mortality were also recorded. RESULTS: Compared with midazolam, dexmedetomidine decreased the level of pro-inflammatory cytokines and increased the level of anti-inflammatory cytokines. At 24 hours after treatment, the levels of serum IL-1ß, TNF-α significantly decreased and IL-10 significantly increased [IL-1ß (ng/L): 6.48±2.89 vs. 8.07±3.14, TNF-α (µg/L): 11.25±5.21 vs. 15.44±5.97, IL-10 (ng/L): 12.10±5.35 vs. 9.58±4.71, all P < 0.05]. At 48 hours after treatment, the levels of serum IL-6, IL-8, IL-1ß, TNF-α and CRP significantly decreased and IL-10 significantly increased [IL-6 (ng/L): 209.67±80.49 vs. 336.31±123.94, IL-8 (ng/L): 229.09±80.81 vs. 298.28±90.25, IL-1ß (ng/L): 7.31±3.02 vs. 8.74±3.17, TNF-α (µg/L): 12.52±4.79 vs. 16.58±5.98, CRP (g/L): 47.82±24.92 vs. 72.35±31.71, IL-10 (ng/L): 12.90±5.42 vs. 10.01±4.79, all P < 0.05]. At 72 hours after treatment, the levels of serum IL-8 and CRP significantly decreased [IL-8 (ng/L): 234.64±96.24 vs. 290.28±103.97, CRP (g/L): 53.24±29.12 vs. 86.58±38.30, both P < 0.05]. Compared with midazolam, dexmedetomidine could significantly reduce the duration of mechanical ventilation (days: 4.7±1.3 vs. 6.6±2.1), length of PICU stay (days: 9.5±2.7 vs. 12.3±3.9, both P < 0.05), and the ratio of sepsis (33.3% vs. 53.1%, P < 0.05). But there were no significant differences in ratio of CRRT (18.2% vs. 18.8%), MOF (9.1% vs. 18.8%) and mortality (6.1% vs. 12.5%) between two groups (all P > 0.05). CONCLUSIONS: Compared with midazolam, dexmedetomidine had better efficacy in the treatment of severe multiple trauma in children and reduce the level of inflammation.

[Report of a case with late-onset ornithine transcarbamylase deficiency with gas chromatography-mass spectrometry and DNA sequencing confirmation and literatures review].

OBJECTIVE: To discuss the clinical characteristics, diagnosis and treatment management of ornithine transcarbamylase deficiency (OTCD). METHOD: Data of the clinical diagnosis and treatment of a case with OTCD were analyzed, and the domestic and international literature was reviewed. RESULT: (1) The case was a boy, 8 years old; and the initial symptoms were vomiting and reduced consciousness for a day after eating a lot of eggs as previous similar history. The level of blood ammonia was 2 500 µmol/L. The patient was treated with fasting, high-calorie fluid intravenous infusion, reducing blood ammonia. However, the disease further aggravated and the patient died due to brain hernia and central cardiovascular failure. Finally, he was confirmed as OTCD through urine testing by gas chromatography-mass spectrometry, plasma amino acid examination (plasma arginine and citrullineurine reduced, orotic acid raised) and genetic testing (OTC c.386G>A p. (Arg129His)). (2) Data of 55 case reports about diagnosis and treatment outcome including 65 OTCD cases' clinical data in domestic and abroad reports in nearly 5 years. Their ages ranged from 3 days to 49 years; the common symptoms were vomiting and encephalopathy; both urine tests were positive in 52 cases; pathogenic genes had less repeated loci and located at Xp21.1 in 42 cases detected by OTC gene test; all of which had gene mutation. CONCLUSION: The acute onset OTCD begins with the symptoms of vomiting and unconsciousness, with rapid rise of blood ammonia level. OTCD may cause high mortality.

[Analysis of serious respiratory complications in PICU in children with measles].

OBJECTIVE: To evaluate the characteristics of the serious complications of 17 cases with measles in ICU. METHOD: Seventeen cases with measles with serious complications in ICU in Anhui Provincial Children's Hospital were recruited from May 2012 to May 2013. Clinical characteristics, image finding, and prognosis were analyzed retrospectively. IgM antibody was positive in all the 17 cases, which included 9 male cases, 8 female cases, and their age was from 2 months to 10 years. RESULT: All the 17 patients received mechanical ventilation because of severe respiratory distress within 1 week of onset, of which 14 cases were complicated with acute respiratory distress syndrome (acute ARDS), 6 cases of tension pneumothorax, 3 cases were complicated with bronchitis, laryngeal obstruction III degrees, and totally 7 cases died. The survived 10 patients were followed up for 1 year, 1 patient with localized pneumothorax, bronchopleural fistula, 1 case of mild pulmonary fibrosis, 1 case of acute laryngitis with persistent hoarseness, and mild inspiratory dyspnea were found. The remaining 7 cases fully recovered. Fourteen cases failed to inoculate measles vaccine. CONCLUSION: A higher risk of death and poor prognosis were found in children with measles who needed to be treated in PICU, especially for ARDS with pneumothorax. Laryngitis needed long time to cure and had weaning difficulties, and the case associated with pleural effusion or bronchopleural fistula must be treated with surgery, and the patients easily develop secondary bacterial infection.