Effect of dominant vertebral artery angle on basilar artery curvature and plaque.

Background: Quantification of vertebral arteries can provide insights into basilar curvature and plaque. Therefore, this retrospective study aimed at identifying the dominant vertebral artery (VA) causing basilar artery (BA) curvature and to further quantify the effect of dominant VA angle on BA curvature and BA plaque using high-resolution magnetic resonance imaging (HRMRI) and 3-dimensional time-of-flight magnetic resonance angiography (3D-TOF-MRA). Methods: This retrospective analysis included 521 participants who underwent HRMRI in the China-Japan Friendship Hospital from November 2015 to October 2021 for neurological symptoms or signs. The VA angle more related to BA curvature was defined as the dominant VA angle. Multivariable linear regression analysis was used to evaluate the relationship between the dominant VA angle and mid-BA angle, while multivariable logistics regression was used to evaluate the influence of the dominant VA angle and clinical risk factors on BA plaque. Results: In total, 259 participants were included in this study (mean age 53.71±13.12 years; 146 males). The balanced-type participants had a significantly lower probability of BA plaques (P<0.001). The Chi-squared test showed that the BA curvature direction was significantly associated with the side with larger VA diameter (P<0.001) and larger VA angle (P<0.001). As a result, the VA angle on the side with the larger diameter or the larger VA angle when the diameters were similar was considered to be the dominant VA angle. The dominant VA angle was independently correlated with the mid-BA angle (P<0.001). In addition, the dominant VA angle was also an independent risk factor for BA plaque. Additionally, 80° was the cutoff value of the dominant VA angle, and when the dominant VA angle was greater than 80°, the risk of BA plaque increased about 18-fold [odds ratio, 18.951; 95% confidence interval (CI): 4.545-79.026; P<0.001]. Conclusions: The dominant VA angle was independently associated with BA plaque, and a dominant VA angle greater than 80° may be a marker for a high risk of posterior circulation atherosclerosis.

Correlation between CT Score and KL-6: A Severity Assessing in Juvenile Dermatomyositis Associated Interstitial Lung Disease.

Background: There is no radiological measurement to estimate the severity of pediatrics juvenile dermatomyositis (JDM) with interstitial lung disease (ILD). We validated the effectiveness of CT scoring assessment in JDM patients with ILD. Aim: To establish a CT scoring system and calculate CT scores in JDM patients with ILD and to determine its reliability and the correlation with Krebs von den Lungen-6 (KL-6). Methods: The study totally enrolled 46 JDM-ILD patients and 16 JDM without ILD (non-ILD, NILD) patients. The chest CT images (7.0 ± 3.6 years; 32 male and 30 female) were all analyzed. CT scores of six lung zones were retrospectively calculated, included image pattern score and distribution range score. Image pattern score was defined as follows: increased broncho-vascular bundle (1 point); ground glass opacity (GGO) (2 points); consolidation (3 points); GGO with bronchiectasis (4 points); consolidation with bronchiectasis (5 points); and honeycomb lung (6 points). Distribution range score was defined as no infiltrate (0 point); <30% (1 point); 30%-60% (2 points); and ≥60% (3 points). Two pediatric radiologists reviewed all CT images independently. The ROC curve was established, and the optimal cutoff score for severity discrimination was set. Results: The agreement between two observers was excellent, and the ICC was 0.930 (95% CI 0.882-0.959, p < 0.01). CT score and KL-6 level had a positive linear correlation (r = 0.784, p < 0.01). However, the correlation between CT scores of different lung zone and KL-6 level was different. The KL-6 cut off level suggested for JDM with ILD was 209.0 U/ml, with 73.9% sensitivity and 87.5% specificity, and the area under curve was (AUC) 0.864 (p < 0.01). Conclusion: The CT scoring system we established, as a semiquantitative method, can effectively evaluate ILD in JDM-PM patients and provide reliable evidence for treatment.

Mowing mitigated the sensitivity of ecosystem carbon fluxes responses to heat waves in a Eurasian meadow steppe.

The heat waves (HW) will be more frequent and intense in the future with increased human activity and uncertain implications for ecosystem carbon fluxes. The semi-arid Eurasian grassland is sensitive to climate change and under frequent HWs attacks. Mowing as one of the most common human practices in this region, combining with HW can have comprehensive effects on plant communities, biomass, and nutrient cycling. Hence, a 3-year (2019-2021) field manipulation experiment was conducted to assess how mowing influenced the carbon cycling under HWs, and the interactions between HWs and mowing on carbon fluxes at the community and ecosystem levels in a Eurasian meadow steppe. Over the three years, HW significantly reduced net ecosystem CO2 exchange (NEE) and gross ecosystem production (GEP) by 28 % and 8 % (P < 0.05), respectively, whereas ecosystem respiration (Re) did not show significant changes. Moderate mowing (stubble height was set at 6-8 cm) for harvest effectively mitigated ecosystem sensitivity to HWs and significantly increased ecosystem carbon fluxes (NEE, Re, and GEP), biomass and the number of species. Mowing reduced the negative impact of HWs on ecosystem carbon fluxes by about 15 % compared to HWs alone, contributing to the invasion of species such as Thalictrum squarrosum and Vicia amoena, and increased the indirect effect of HW on NEE in the structural equation model. In addition, the higher soil water content (SWC) was another effective way to reduce the impact of HWs. Therefore, mowing and higher SWC would be effective ways to counteract the negative effects of HWs on carbon fluxes in future grassland management.

Extreme wet precipitation and mowing stimulate soil respiration in the Eurasian meadow steppe.

The imbalance of terrestrial carbon (C) inputs versus losses to extreme precipitation can have consequences for ecosystem carbon balances. However, the current understanding of how ecosystem processes will respond to predicted extreme dry and wet years is limited. The current study was conducted for three years field experiment to examine the effects of environmental variables and soil microbes on soil respiration (Rs), autotrophic respiration (Ra) and heterotrophic respiration (Rh) under extreme wet and dry conditions in mowed and unmowed grassland of Inner Mongolia. Across treatments (i.e. control, dry spring, wet spring, dry summer and wet summer), the mean of Rs was increased by 24.9 % and 24.1 % in the wet spring and wet summer precipitation treatments, respectively in mowed grassland. In other hand, the mean of Rs was decreased by -22.1 % and -3.5 % in dry spring and dry summer precipitation treatments, respectively in mowed grassland. The relative contribution of Rh and Ra to Rs showed a significant (p < 0.05) change among simulated precipitation treatments with the highest value (76.18 %) in wet summer and 26.41 % in dry summer, respectively under mowed grassland. Rs was significantly (p < 0.05) affected by the interactive effect of extreme precipitation and mowing treatments in 2020 and 2021. The effects of precipitation change via these biotic and abiotic factors explained by 52 % and 81 % in Ra and Rh, respectively in mowed grassland. The changes in microbial biomass carbon (MBC) and nitrogen (MBN) had significant (p < 0.05) direct effects on Rh in both mowed and unmowed grasslands. The influence of biotic and abiotic factors on Rs was stronger in mowed grasslands with higher standardized regression weights than in unmowed grassland (0.78 vs. 0.69). These findings highlight the importance of incorporating extreme precipitation events and mowing in regulating the responses of C cycling to global change in the semiarid Eurasian meadow steppe.

Clinical Outcome Discrimination in Pediatric ARDS by Chest Radiograph Severity Scoring.

Background: There is no accurate radiological measurement to estimate the severity of pediatrics acute respiratory distress syndrome (PARDS). We validated the effectiveness of an adult radiographic assessment of lung edema (RALE) score in PARDS. Aim: To assess the severity and prognosis of PARDS based on a chest radiograph (CXR) RALE scoring method. Methods: Pediatric Acute Lung Injury Consensus Conference (PALICC) criteria were used to diagnose PARDS. General demographics, pulmonary complications, and 28-day mortality of the patients were recorded. Subgroups were compared by prognosis (survive and death) and etiology (infection and noninfection). Two observers calculated RALE independently. Each quadrant of CXR was scored by consolidation scores 0 (none alveolar opacity), 1 (extent <25%), 2 (extent 25%-50%), 3 (50%-75%), and 4 (>75%) and density scores 1 (hazy), 2 (moderate), and 3 (dense). Quadrant score equals consolidation score times density score. Total score equals to the sum of four quadrants scores. The ROC curve and survival curve were established, and the optimal cutoff score for discrimination prognosis was set. Results: 116 PARDS (72 boys and 44 girls) and 463 CXRs were enrolled. The median age was 25 months (5 months, 60.8 months) and with a mortality of 37.9% (44/116). The agreement between two independent observers was excellent (ICC = 0.98, 95% CI: 0.97-0.99). Day 3 score was independently associated with better survival (p < 0.001). The area under the curve of ROC was 0.773 (95% CI: 0.709-0.838). The cutoff score was 21 (sensitivity 71.7%, specificity 76.5%), and the hazard ratio (HR) was 9.268 (95% CI: 1.257-68.320). The pulmonary complication showed an HR of 3.678 (95% CI: 1.174-11.521) for the discrimination. Conclusion: CXR RALE score can be used in PARDS for discriminating the prognosis and has a better agreement among radiologist and pediatrician. PARDS with pulmonary complications, day 3 score whether greater than 21 points, have a better predictive effectiveness.

Diagnosis of Hirschsprung disease by hydrocolonic sonography in children.

OBJECTIVES: To compare hydrocolonic sonography with histopathology for diagnosing children with symptoms highly suggestive of Hirschsprung disease (HD). METHODS: In this prospective study, patients presenting refractory constipation highly suggestive of HD underwent hydrocolonic sonography with retrograde infusion of saline into the colon. The dilated segments, narrowed segments, luminal diameter ratio, transition zone (TZ), thickening, and blood perfusion of the upstream bowel were evaluated. The sensitivity and specificity of combined and single parameters were determined in comparison with biopsy. RESULTS: One hundred and three children were included in this study; 49 were confirmed to have HD. The luminal diameter ratio showed superiority over other parameters. An area under the curve (AUC) of 0.969 (95% confidence interval [CI]: 0.936-1.000) and a cutoff value of 1.51 were established by receiver operating characteristic (ROC) curve analysis of the luminal diameter ratio (sensitivity: 89.8%; specificity: 96.3%). By combining the luminal diameter ratio as the major criterion with two minor criteria, hydrocolonic sonography showed the same sensitivity (91.8%) and better specificity (96.3% vs 87%) than contrast enema, but this difference was not statistically significant (p = 0.063). Consistency analysis showed a kappa value of 0.825 (p < 0.001), indicating excellent agreement between hydrocolonic sonography and contrast enema. CONCLUSIONS: Hydrocolonic sonography is a valuable diagnostic tool with both high sensitivity and specificity for HD diagnosis, allowing morphological and vascular assessments of the colon, and correlates well with contrast enema. In the appropriate setting, hydrocolonic sonography may be an alternative screening method for HD in a large group of children with constipation. Key Points • Hydrocolonic sonography is a simple, well-tolerated diagnostic tool with both high sensitivity and specificity for HD diagnosis. • Hydrocolonic sonography allows morphological and vascular assessments of the colon, and correlates well with contrast enema. • Hydrocolonic sonography is a possible alternative modality for paediatric patients highly suggestive of HD.

Spatial patterns and driving factors of aboveground and belowground biomass over the eastern Eurasian steppe.

The Eurasian steppe is the largest steppe region in the world and is an important part of the global grassland ecosystem. The eastern Eurasian steppe has favorable hydrothermal conditions and has the highest productivity and the richest biodiversity. Located in the arid and semi-arid region, the eastern Eurasian steppe has experienced large-scale grassland degradation due to dramatic climate change and intensive human activities during the past 20 years. Hence, accurate estimation of aboveground biomass (AGB, gC m-2) and belowground biomass (BGB, gC m-2) is necessary. In this study, plenty of AGB and BGB in-situ measurements were collected among dominated grassland types during summer in 2013 and 2016-2018 in the eastern Eurasian steppe. Vegetation indices from the Moderate Resolution Imaging Spectroradiometer (MODIS), Digital Elevation Model (DEM) and climate variables were chosen as independent variables to establish predictive models for AGB and BGB with random forest (RF). Both AGB (R2 = 0.47, MAE = 21.06 gC m-2, and RMSE = 27.52 gC m-2) and BGB (R2 = 0.44, MAE = 173.02 gC m-2, and RMSE = 244.20 gC m-2) models showed acceptable accuracy. Then the RF models were applied to generate spatially explicit AGB and BGB estimates for the study area over the last two decades (2000-2018). Both AGB and BGB showed higher values in the Greater Khingan Mountains and decreased gradually to the east and west sides. The mean values for AGB and BGB were 62.16 gC m-2 and 531.35 gC m-2, respectively. The climatic factors were much more important in controlling biomass than anthropogenic drivers, and shortage of water and raising temperature were the main limiting factor of AGB and BGB, respectively, in the peak growth season. These findings provide scientific data for the scientific management of animal husbandry and can contribute to the sustainable development of grassland ecology in the eastern Eurasian steppe.

Surgical approach and functional outcome of redo pull-through for postoperative complications in Hirschsprung's disease.

AIM: To review our surgical experience and outcome of redo pull-through for various postoperative complications of Hirschsprung's disease. METHODS: A retrospective study was performed on children who underwent redo pull-through from 2016 to 2019. Operative methods and functional outcomes were compared between those with anastomotic complications (stricture and fistula, n = 12) and patients without anastomotic complications (n = 24) such as residual aganglionosis/transition zone, twisted pull-through and tight soave cuff. RESULT: 36 Patients (29 male and 7 female) were included with median age 6 (0.1-54) months at primary and 36 (9-144) months at redo pull-through. A transanal rectal mucosectomy and partial internal anal sphincterectomy (TRM-PIAS) pull-through with laparoscopic (n = 10, 27.8%) or laparotomy (n = 26, 72.2%) assisted techniques were performed for all patients during redo procedure. Patients with anastomotic complications had lower incidence of successful laparoscopic pull-through (0%), higher postoperative complications (25%) after redo surgery, but similar functional outcomes compared to those without anastomotic complications (41.6% underwent laparoscopic surgery, 4.2% complications). Patients with partial colectomy had significantly less soiling (36.4%) and enterocolitis (0%) compared to those with subtotal/total colectomy (79.2% soiling and 58.3% enterocolitis). CONCLUSION: TRM-PIAS with/without laparoscopic-assisted redo pull-through was effective in treating various complications after primary pull-through. The functional outcome is strongly associated with the length of residual colon after redo pull-though.

Novel MNX1 mutations and genotype-phenotype analysis of patients with Currarino syndrome.

BACKGROUND: Currarino syndrome (CS) is a specific complex of congenital caudal anomalies, including anorectal malformations, presacral mass and sacral anomalies. Mutations in the MNX1 gene are closely related to CS and occur in almost all familial cases and less than half of sporadic patients. We investigated the spectrum of MNX1 pathogenic variants and associated clinical features in Chinese patients with CS. RESULTS: Seventeen index patients from 16 families were recruited from 2015 to 2018. All patients were diagnosed with CS and treated at the Capital Institute of Pediatrics Affiliated Children's Hospital. Genetic testing was applied to identify mutations in CS patients and their relatives by whole-exome sequencing and Sanger sequencing. Functional verification was performed for a recurrent noncanonical splice site variant in MNX1 with a minigene splicing assay. In 17 CS patients, 14 were complete CS and 3 were mild CS. Nine variants in MNX1 were identified in 11 patients, and these included two frameshift mutations (p.Leu223Leufs*61, p.X402Serfs*70), four nonsense mutations (p.Gly42X, p.Cys88X, p.Gln24X, p.Cys241X), one missense mutation (p.Trp288Leu), one splice region variant (c.691 + 3G > T) and one polyalanine polymorphism (p.Ala135insAlaAla). Seven of these nine variants have never been reported. Pathogenic MNX1 mutations were found in 100% (4/4) of familial and 46% (6/13) of sporadic patients. CONCLUSION: Our study expanded the mutation spectrum of MNX1 and provided clinical and genetic analyses of seventeen CS patients from mainland China.

Laparoscopic-Assisted Anorectal Pull-Through for Currarino Syndrome.

Objective: Currarino syndrome (CS) is characterized by the triad of sacral anomalies, presacral tumor, and anorectal malformation (ARM). This study evaluates the feasibility and outcomes of laparoscopic-assisted anorectal pull-through (LAARP) for CS. Materials and Methods: Children admitted for primary or redo repair of CS through LAARP between 2016 and 2019 were reviewed. The indication of redo included constipation with megarectosigmoid, residual presacral mass, anastomosis leak, and secondary fistula. ARM was corrected by excision of rectal stenosis, fistula, and its associated megarectosigmoid. This was followed by a complete surgical resection of the presacral tumor, with subsequent pull-through and anocolic anastomosis. This was done with a combined laparoscopic and transanal approach. The detailed surgical techniques, early postoperative complications, and mid-term functional outcomes were summarized. Results: Fourteen patients underwent LAARP for primary (N = 4) and redo (N = 10) repair of CS. Four of them had colostomy previously. Mean age at operation was 20.7 ± 13.9 months. Mean operative time was 120 ± 25 minutes. Median hospital stay was 8 days (range 7-9 days) postoperatively. None of the patients developed early postoperative complications such as anastomotic leaks, presacral abscesses, recurrent fistulas, and residual mass. Bowel function was assessed 1 year after LAARP in 10 patients. Mean follow-up time was 15.9 months. The frequency of bowel movements was 3.3 ± 1.5/day. Constipation occurred in 2 patients. Occasional soiling (<3 times/week) was reported in 4 patients and frequent soiling (>3 times/week) was in 1. Conclusion: LAARP for CS is safe and effective.

Pulmonary acute graft-versus-host disease and infections after allogeneic hematopoietic stem cell transplantation in pediatric recipients: A comparative study on CT.

OBJECTIVE: To compare the chest CT patterns of acute graft-versus-host disease (aGVHD) and infections within 100 days after allogeneic hematopoietic stem cell transplantation (allo-HSCT) in pediatric recipients to help hematologist make definitive diagnosis as early as possible. METHODS: A total of 143 pediatric recipients from January 2015 to June 2019 who were diagnosed pulmonary aGVHD or infections within 100 days after allo-HSCT were enrolled in this study. Two observers evaluated the extent and distribution (unilateral, bilateral) of the CT patterns. The patterns were then classified as ground-glass opacity (GGO) (localized, patchy, diffuse), consolidation (localized, patchy, diffuse), reticulation (localized, patchy, diffuse), nodules (localized, multiple), bronchiectasis, pleural effusion, air trapping, tree-in-bud sign, and pneumomediastinum. The onset time and radiological patterns of the two cohorts were statistically compared. RESULTS: The mean onset time of aGVHD (n = 85) and infections group (viral n = 29, bacterial n = 22, fungal n = 7, total n = 58) was 36.89 ± 24.34 (range, 10-99 days) and 23.48 ± 20.65 days (range, 4-94 days) with a significant difference (P = .001). The top three underlying diseases were acute lymphoblastic leukemia (ALL) (n = 49, 57.6%); acute myeloid leukemia (AML) (n = 24, 28.2%); and aplastic anemia (AA) (7.1%) in aGVHD group and hemophagocytic syndrome (HPS) (n = 33, 56.9%); AA (n = 9, 15.5%); and ALL (n = 6, 10.3) in infection group. GGO (41.2%) in aGVHD prevailed on CT, whereas GGO (53.4%) and consolidations (43.1%) were more prevalent in infections. The distribution of GGO showed more diffuse in aGVHD (P = .031) and symmetric while patchier GGO prefers infections (P < .001). No differences were found in the reticulation. Nodules were more common in infections (P = .004) while pleural effusion was more common in aGVHD group (P < .035). CONCLUSION: Imaging patterns of aGVHD on CT differ substantially from that of infections. Physicians and radiologists should be aware of such radiological differences in order to give accurate treatment. Notably, definite diagnosis should be made in combination with clinical manifestations, signs, and laboratory tests.

Grazing affects snow accumulation and subsequent spring soil water by removing vegetation in a temperate grassland.

By altering plant and soil properties and microclimate environments, grazing has a profound influence on the structure and function of grassland ecosystems. However, few studies have addressed the potential grazing effects on snow accumulation and subsequent spring soil water after snow melting and soil thawing. In this study, vegetation properties, snow accumulation and soil water were measured in experimental plots subjected to 8 years of cattle grazing comprising six different grazing intensity treatments in a typical temperate grassland in eastern Eurasia. The results indicated that heavy grazing reduced the snow depth by 51% and the snow mass by 40%. Snow accumulation first rapidly increased but then remained relatively stable with increases in both the aboveground biomass and canopy height. An obvious inflection point occurred at approximately 200 g m-2 aboveground biomass and at a 12.5 cm canopy height. The obvious difference in soil water content between the heavily grazed and ungrazed treatments occurred mainly in the spring after snow melting and soil thawing. The spring soil water content (0-30 cm) reached 31.5% in the ungrazed treatment (G0), which was 1.7 times that in the heavily grazed treatment (G0.92). The soil water content increased exponentially with increasing vegetation properties (aboveground biomass, canopy height and canopy cover), and a similar trend occurred with increasing snow mass and snow depth. Our structural equation modeling showed that both vegetation properties and snow accumulation had significant positive effects on spring soil water. By removing vegetation, grazing at increased intensities had significant, indirect suppressive effects on snow accumulation and spring soil water. Therefore, to obtain increased amounts of snow accumulation and spring soil water, land managers should consider reducing the grazing intensity or leaving some plots ungrazed.

[Clinical and genetic features of Mowat-Wilson syndrome: an analysis of 3 cases].

Mowat-Wilson syndrome (MWS) is a rare autosomal dominant genetic disease caused by zinc finger E-box-binding homeobox 2 (ZEB2) gene mutation and has various clinical manifestations including intellectual disability/global developmental delay, unusual facies and multiple congenital malformations. This article reports the clinical features and gene mutations of three children diagnosed with MWS by ZEB2 gene analysis. All three children had Hirschsprung disease and unusual facies. One child died of severe heart failure and pneumonia at the age of 4 months. Global developmental delay was not discovered by her parents due to her young age. The other two children had severe global developmental delay. All three children carried a de novo heterozygous nonsense mutation in the ZEB2 gene, among which c.756C>A (p.Y252X) had not been reported before. Such mutations produced truncated proteins and were highly pathogenic. MWS is presented with strong clinical and genetic heterogeneity. Clinicians should consider the possibility of MWS when a child has unusual facies of MWS, intellectual disability/global developmental delay and multiple congenital malformations. Gene detection helps to make a confirmed diagnosis.

Sixteen Chinese pediatric brucellosis patients onset of fever in non-epidemic areas and 8 developed with osteoarticular involvement.

The purpose of this study is to summarize the manifestations, diagnosis, differential diagnosis, and treatment of childhood brucellosis in non-epidemic areas of China. A retrospective review of 16 admitted children patients with brucella's disease who were diagnosed of brucellosis during the period from 2011 to 2016 was performed. Diagnostic criteria, clinical presentations, and outcomes were recorded. The most common symptom was fever. Osteoarticular involvement was found in 50% of the patients. They were infected by contacting with infected animals or consuming of unpasteurized milk or meat of sheep or goats, also. Standard agglutination test was positive in all patients and blood culture in 10 (62.5%) patients as well as medulloculture in 3 (18.8%) patients were positive. A combination of antibiotic treatment with rifampin plus cotrimoxazole showed good response and all clinical manifestations improved. Brucellosis is misdiagnosed frequently and should be considered in the differential diagnosis when patients do not respond to standard treatment. Blood culture, together with brucella serology test, is important and helpful in the diagnosis. MRI is a good method in differentiating those with symptoms of arthritis.

Experience of Mowat-Wilson syndrome prenatal diagnosis for a Chinese family.

Mowat-Wilson syndrome (MWS) is a complex developmental disorder. We report the first prenatal diagnosis provided for a family in mainland China after identifying the causal mutation for the proband. Special focus on MWS-related organs during prenatal ultrasound scan is described which is extremely important for genetic counseling of parents.

Annular pancreas in Trichorhinophalangeal syndrome type II with 8q23.3-q24.12 interstitial deletion.

BACKGROUND: Trichorhinophalangeal syndrome type II (TRPS II, OMIM # 150230) is a rare autosomal dominant genetic disorder characterized by craniofacial and skeletal abnormalities. Loss of functional copies of the TRPS1 gene at 8q23.3 and the EXT1 gene at 8q24.11 are considered to be responsible for the syndrome. CASE PRESENTATION: Herewith, we report an 8-year-old girl with sparse scalp hair, bulbous nose, thin upper lip, broad eyebrows, phalangeal abnormalities of both hands/toes, multiple exostoses, mild intellectual impairment and severe malnutrition. In addition, the patient also had annular pancreas, a rare co-existing feature in patients with TRPS II. CONCLUSIONS: A contiguous 5.47 Mb deletion involving 8q23.3-q24.12 was detected by array comparative genomic hybridization (aCGH), leading to haploinsufficiency of 10 protein coding genes, 1 long non-coding RNA and 1 microRNA. Quantitative PCR (qPCR) examination confirmed half-reduced DNA copy of the patient and normal expression of both parents, indicating a de novo origin of the deletion and complete penetrance of the mutation.

[Effect of grazing on the temperature sensitivity of soil respiration in Hulunber meadow steppe].

Grazing is one of the major human activities which lead to disturbance on grassland ecosystem. Quantifying the effect of grazing on the temperature sensitivity of soil respiration ( Q10 ) is essential for accurate assessment of carbon budget in grassland ecosystem. This study was conducted on the grazing gradients experiment platform in Hulunber meadow steppe. Soil respiration was measured by a dynamic closed chamber method (equipped with Li 6400-09, Lincoln, NE, USA) during the growing season in 2011. The results showed that soil respiration had significant seasonal variation and the maximum occurred in July, which was mainly dominated by temperature. The order of average soil respiration during the period from May to September in different treatments was G1 > GO > G2 > G3 > G4 > G5. Comparing with non-grazing treatment, Q10 under heavy grazing conditions (0. 92 Au hm-2) was reduced by about 10% , and was increased a little under light grazing conditions (0. 23 Au hm-2). There was a significant negative correlation between Q15 and grazing intensities (r = 0. 944, P <0. 05) . Grazing could decrease the temperature sensitivity of soil respiration to different degrees. The Q10 under different grazing gradients had positive linear regression relationships with aboveground biomass, belowground biomass, soil organic carbon and soil moisture. They could explain 71.0%-85.2% variations of Q10. It was suggested that the variation of Q10 was mainly determined by the change of biotic and environmental factors due to grazing.

[Chest CT features and outcome of necrotizing pneumonia caused by Mycoplasma pneumoniae in children (report of 30 cases)].

OBJECTIVE: To summarize the chest CT features and outcome of necrotizing pneumonia (NP) caused by Mycoplasma pneumoniae in children and to review the changes of common inflammatory parameters in NP patients to help clinicians understand the proper timing of CT scan. METHOD: The imaging data from 30 cases of Mycoplasma pneumoniae pneumonia in NP group and 24 cases with non-necrotizing Mycoplasma penumoniae pneumonia (control group) were analyzed retrospectively. The changes of common inflammatory parameters in NP group and control group were compared. RESULT: (1) The chest CT findings of NP (30 cases): 28 cases showed unilateral pneumonia, and 20 cases showed single lobar consolidation, 10 cases had multiple lobes involvement; pulmonary cavities were seen in 27 cases. There were decreased enhancement areas in the consolidation (22 cases). (2) The dynamic changes of CT signs during follow-up: The CT scan performed during the 1 - 2 months after onset of disease (23 cases) showed that pulmonary consolidation in 2 cases (9%) were absorbed, 18 cases (78%)had cavities in lung, 16 cases (70%) had pleural thickening, 2 cases (9%) atelectasis and 1 case (4%) bronchopleural fistula;the CT scan performed during the 2 - 3 months after onset of disease (11 cases) showed pulmonary consolidation in 7 cases (64%) were absorbed, 10 cases (91%) pleural thickness, 7 cases (64%) with cavities in lung, 5 cases (45%) atelectasis, 2 cases (18%) pulmonary lobe cysts and 1 case bronchopleural fistula. The CT scan performed at 3.5 years of disease course (10 cases) showed that there were no pulmonary consolidation in any of the cases, 4 cases had atelectasis, 4 cases had pulmonary cysts, and 1 case had band-like scars. (3) There were significant differences between NP group and control group in the maximum peripheral blood WBC, proportion of neutrophil and C-reactive protein(CRP, mg/L) (P < 0.01, 0.01, 0.001, respectively), and there was significant difference between the 2 groups in the duration of fever, abnormal WBC(d) and CRP(d) (P < 0.001). CONCLUSION: The chest CT features of NP caused by Mycoplasma pneumoniae in children were single lobular consolidation in most cases, NP had decreased parenchymal enhancement and cavity in the consolidation, and recovery was slow, the outcome included recovery, atelectasis or lobar cystic degeneration. The clinicians should pay more attention to the common inflammatory parameters when they suspect the Mycoplasma pneumoniae pneumonia is progressing into necrosis and make correct decision for chest CT examination.

[Preliminary study on normal aerification of paranasal sinuses in children].

OBJECTIVE: To explore the normal aerification of paranasal sinuses in Chinese children with magnetic resonance imaging. METHODS: Two hundred and eighty Chinese children aged from 17 days to 14 years without any symptoms related to sinusitis were statistically analyzed in MRI features, including counting the number of paranasal sinus pneumatization and the maximum axial and sagittal area of the left maxillary. RESULTS: The pneumatization rate of maxillary sinus was 85% in children aged from 0 to 1 years. Until 3 years the pneumatization rate of maxillary sinus was 95% and there was no significant difference in boys and girls (χ(2) = 0.741, P = 0.389). The pneumatization rate of maxillary sinus reached 100% after 4 years old. The pneumatization rate of ethmoid sinus was 100% in this study. The pneumatization rate of sphenoid sinus was 0 within 1 year old, 49% within 4 years old and 100% after 7 years old. There was no significant difference in boys and girls on the pneumatization rate of sphenoid sinus (χ(2) = 2.452, P = 0.117). The pneumatization rate of frontal sinus was 0 within 5 years old, 62% within 9 years old and 95% after 10 years old. There was no significant difference in boys and girls on the pneumatization rate of frontal sinus (χ(2) = 0.124, P = 0.724). The axial and sagittal maximum area of maxillary sinus was (689.28 ± 221.79) and (659.76 ± 263.31) mm(2) in girls and (668.13 ± 206.38) and (638.60 ± 207.67) mm(2) in boys. The differences were significant (t = -19.78, P < 0.001; t = -19.89, P < 0.001). CONCLUSION: The study of the development and normal aerification of paranasal sinuses of children can help radiologist make correct diagnosis of paranasal sinuses in children.

[Chest radiographic findings in children with 2009 influenza A (H1N1) virus infection].

OBJECTIVE: To evaluate chest radiographic findings of children with 2009 influenza (H1N1) virus infection. METHOD: Data of 235 patients who had microbiologically confirmed H1N1 infection and available chest radiograph obtained between May 1(st) 2009 and Jan. 31(st) 2010 were retrospectively analyzed. The final study group was divided on the basis of clinical course [group 1 mild, outpatients without hospitalization (n = 172); group 2 moderate, inpatients with brief hospitalization (n = 49); group 3 severe, ICU admission (n = 14)]. Four pediatric radiologists reviewed all the chest radiographs of lung parenchyma, airway, pleural abnormalities and also anatomic distribution of the disease. RESULT: No significant sex or age differences were found among the study groups (P > 0.05). The mean interval between the onset of clinical symptom and the initial chest radiography was (5.91 ± 1.64) days (group 1), (3.60 ± 1.43) days (group 2) and (1.21 ± 0.41) days (group 3), respectively. The differences among the three groups were significant statistically (χ(2) = 13.368, P < 0.01). The ratio of abnormality presented at initial chest X-ray was 79.7% in group 1, 91.8% in group 2 and 100% in group 3. Radiographically, there were prominent peribronchial markings (group 1, 55.2%; group 2, 83.7%; and group 3, 78.6%), consolidation (group 1, 34.3%; group 2, 69.4%; and group 3, 100.0%), hyperinflation (group 1, 22.1%; group 2, 44.9%; and group 3, 50.0%) and ground glass opacity (group 1, 0.6%; group 2, 2.0%; and group 3, 14.3%) in the chest radiographs. The differences of presenting were statistically significant (P < 0.01). In the severe group, the lesions distributed diffusely and asymmetrically with multi-lobe involvements. CONCLUSION: In children with 2009 influenza A H1N1 viral infection, the interval between the onset of clinical symptom and initial chest radiography, the ratio of abnormality presented at initial chest X-ray film and the severity of chest film are parallel to their clinical situation.