[Genetic characteristics and survival analysis of 27 cases of juvenile myelomonocytic leukemia].

Objective: To investigate the genetic and genomic profiling of juvenile myelomonocytic leukemia (JMML) and factors affecting its survival rate. Methods: Clinical characteristics, cytogenetics, molecular biology results and survival status of children with 27 JMML cases admitted to the Hematology Department of Children's Hospital, Capital Institute of Pediatrics from December 2012 to December 2021 were analyzed retrospectively, and the outcomes of the children were followed up. Kaplan-Meier method was used for survival analysis. Univariate analysis was used for analyzing factors affecting the overall survival (OS) rates of patients who received hematopoietic stem cell transplantation (HSCT). Log-Rank test was used for comparison of survival curves. Results: Among 27 JMML cases, there were 11 males and 16 females. The age of disease onset was 28 (11,52) months. There are 20 cases of normal karyotype, 4 cases of monosomy 7, 1 case of trisomy 8,1 case of 11q23 rearrangement and 1 case of complex karyotype. A total of 39 somatic mutations were detected.Those involved in RAS signal pathway were the highest (64%(25/39)), among which PTPN11 mutation was the most frequent (44% (11/25)). A total of 17 cases (63%) received HSCT, 8 cases (30%) did not receive HSCT, and 2 cases (7%) lost follow-up. For children receiving transplantation, the follow-up time after transplantation was 47 (11,57) months. The 1-year OS rate of high-risk transplantation group (17 cases) and high-risk non transplantation group (6 cases) was (88±8)% and (50±20)% respectively, with a statistically significant difference (χ2=5.01, P=0.025). The 5-year OS rate of the high-risk transplantation group was (75±11)%. The survival time of those who relapsed or progressed to acute myeloid leukemia after transplantation was significantly shorter than that of those who did not relapse (χ2=6.80, P=0.009). The OS rate of patients with or without PTPN11 mutation was (81±12) % and (67±19)% respectively (χ2=0.85, P=0.356). Conclusions: The main pathogenesis involved in JMML is gene mutation related to RAS signaling pathway, and the most common driver gene of mutation is PTPN11. Allogeneic HSCT can significantly improve the survival rate of high-risk JMML patients. The recurrence or progression after transplantation was related to poor prognosis.

[Analysis of early diagnosis and prevention techniques for occupational pneumoconiosis based on patent perspectives in China's coal industry].

Occupational pneumoconiosis in the coal industry is an occupational disease that seriously endangers coal miners' health. Early diagnosis and prevention techniques are significant in controlling its incidence rate and reducing its harm. This article takes the patent data related to the early diagnosis and prevention of occupational pneumoconiosis in the coal industry, published from 1985 to 2021, as the research object. It uses tools such as the Derwent Data Analyzer (DDA) software, the Patyee Database, and the PatSnap Database to analyze the technological development trend and characteristics in this field from aspects of growth trend, primary patent holders, IPC classification layout, value, and research and development hotspots. The results show that the number of patent applications in this field indicates a rapid growth trend, mainly in the hands of Shandong Energy Group Co. Ltd., Shandong University of Science and Technology, China University of Mining and Technology, and other institutions or enterprises. Patent technology mainly involves spray dust reduction, dust removal fans, dust masks, and other aspects with high patent value and innovation ability. This article provides a new perspective and reference for preventing, diagnosing, and treating occupational pneumoconiosis in the coal industry.

[Clinical study of causes and outcomes in pediatric liver retransplantation].

Objective: To investigate the etiology,clinical features and prognosis of pediatric liver retransplantation. Methods: The data of 1 024 cases of pediatric liver transplantation (<18 years old) from January 2014 to December 2019 operated at Tianjin First Central Hospital were collected,retrospectively. Retransplantation was performed in 26 cases,among which 25 cases received secondary liver transplantation and 1 case received a third liver transplantation. There were 13 male and 12 female patients among the 25 patients. The median age was 12.9(20.5) months(range: 5.8 to 134.8 months), the body weight was 8.0(5.6) kg(range: 5.0 to 30.0 kg) at the time of retransplantation. The pediatric end-stage liver disease(PELD) score was 17.0(21.3) (range: 0 to 45) before retransplantation. The etiology of retransplantation was biliary complications in 7 cases,primary nonfunction of liver graft in 5 cases,antibody-mediated rejection in 4 cases,hepatic artery thrombosis in 3 cases,portal vein thrombosis in 3 cases,concomitant hepatic artery and portal vein thrombosis in 2 cases,thrombogenesis of inferior Vena Cava in 1 case and sinusoidal obstruction syndrome in 1 case. The patients were divided into two groups according to the time interval(30 days) between two liver transplantations,8 patients were classified into early-retransplantation(≤30 days) group and 18 patients were classified into late-retransplantation (>30 days) group. The etiology of liver retransplantation,pre-transplant score,time interval between two transplantations,surgical aspects,major complications and survival rates were compared between the two groups. Continuous variables with normal distribution were compared with t test,while Mann-Whitney U test was applied to compare variables without normal distribution. Categorical variables were compared with chi-square test. The survival curves were created by Kaplan-Meier method and compared by Log Rank test. Results: The median follow-up time was 26.8(30.2) months(range: 1 day to 85.7 months), and the incidence of retransplantation was 1.9%. In the early-retransplantation group,the duration of surgery was (439.8±151.0)minutes,the graft-to-recipient weight ratio was 5.0(1.8)%(range:3.6% to 6.1%),the main cause for retransplantation were primary nonfunction and vascular complications. In the late-retransplantation group,the duration of surgery was (604.4±158.0)minutes,the graft-to-recipient weight ratio was 3.4(2.1)%(range:1.4% to 5.3%),the main cause for retransplantation were biliary complications,antibody mediated rejection and vascular complications.The 3-month,1-year and 2-year recipient survival rates in the early-retransplantation group were all 62.3%,while the recipient survival rates in the late-retransplantation group were 100%,93.8% and 93.8%,respectively. The difference of recipient survival rates was significant between the early-retransplantation group and the late-retransplantation group(P=0.019). The overall 3-month,1-year and 3-year recipient survival rates after the primary liver transplantation were 97.1%,95.4%,94.1%,respectively. Conclusions: The vascular complications,biliary complications,primary nonfunction and antibody-mediated rejection are the main causes of liver retransplantation.The PELD score is higher in patients receiving early retransplantation,while the surgery is relatively more complex in patients receiving late retransplantation,which is reflected by longer duration of surgeries. Patients in the late-retransplantation group showed similar recipient survival rates with primary liver transplantation recipients,and the survival rates are superior to those of patients in the early-retransplantation group. Infection and multiple organ failure are the most common fatal causes after retransplantation.

Effects of pterostigma structure on vibrational characteristics during flight of Asian ladybird Harmonia axyridis (Coleoptera: Coccinellidae).

The hind wings of beetles are deployable and play an essential role in flight. In the Asian ladybird Harmonia axyridis (Coleoptera: Coccinellidae), the pterostigma (pst) is found in the middle of the hind wing instead of at the tip of the hind wing. This paper investigates the effect of the pst on the vibrational characteristics during the flight of H. axyridis. Based on cross sections of the pst and veins as well as the morphology and nanomechanical properties of the hind wing, including the wing membrane and veins, three three-dimensional coupling models, Models I-III, of hind wings with/without pst structures and veins with varying or uniform reduced moduli are established. Modal analysis results for these three models show that the vibrational characteristics and deformation tendencies change the flight performance of the hind wing models with pst structures compared with that of the other models. The results in this paper reveal that the pst structure has an important influence on vibrational characteristics and deformation tendencies and, hence, on flight performance; the relationships between the body mass and the area of the hind wing, which have significant implications for the design of biomimetic deployable wing structures for micro air vehicles (MAVs), are also analyzed.

Solitary fibrous tumor of the sinonasal cavity: CT and MR imaging findings.

SUMMARY: SFT is a rare lesion of the sinonasal cavity. We retrospectively reviewed 5 patients with histopathologically proved sinonasal SFTs to determine their CT and MR imaging features. All patients underwent paranasal sinus CT and MR imaging. Four SFTs occurred in the nasal cavity, and 1, in the maxillary sinus. All SFTs had well-defined margins, and the mean maximum diameter was 55 mm. On nonenhanced CT, 5 SFTs appeared homogeneously isoattenuating to gray matter. The most common manifestations of bony involvement were bony remodeling and thinning. On MR imaging, 5 SFTs were isointense to gray matter on T1-weighted images, and the lesions were isointense in 3 and hypointense in 2 patients on T2-weighted images. The lesions showed heterogeneously marked enhancement on postenhanced MR images. Four patients underwent dynamic contrast-enhanced MR imaging, and the TICs showed a washout pattern. A familiarity with the imaging findings of sinonasal SFT may help to diagnose this entity.

[Flow cytometry in diagnosis and treatment of bladder tumor].

The cellular DNA contents of 139 bladder irrigations from 52 cases of bladder tumors were determined by flow cytometry. The results were compared with those of pathological grading and exfoliative cytology. It is suggested that cytological results were less accurate than DNA contents. High aneuploid DNA contents in tumor cells were marked by low grade and deep penetration of the bladder tumor. Total cystectomy was needed for bladder tumor with high aneuploid DNA, and partial cystectomy and TURBT for that tumor showed high recurrence with instillation of BCG. The results showed that DNA content of tumor is correlated with the nuclear grade, especially in poorly differentiated neoplasms, but flow cytometry can not replace cystoscopy examination.

[Effects of ciwujia (Acanthopanax senticosus Harms) on reperfusion-induced arrhythmia and action potential alterations in the isolated rat heart].

By means of Langendorff method and standard microelectrode techniques the effects of Ciwujia on reperfusion induced arrhythmias and action potential alterations were studied in isolated rat heart with transient coronary occlusion. Inclusion of Ciwujia extract (equivalent to 1.2 and 2.4 mg crude drug/ml) was found helpful in reducing reperfusion induced ventricular fibrillation and ventricular tachycardia. Associated with this reduction in rhythm disturbances was an increase in the total duration of normal sinus rhythm during the 3 min reperfusion period. With the administration of Ciwujia the number of cells with abnormal action potential configurations was significantly reduced. This confirms that Ciwujia can protect myocardium from electrophysiological abnormalities, and therefore reduces the incidence of malignant arrhythmias.

[Cholangiothoracic fistulae].

Eight cholangio-thoracic fistula patients were seen from 1981 to 1987 in our hospital. The clinical features of the cases were classified into four types, i.e. bronchobiliary fistula (BBF, 3 cases), bronchopleurobiliary fistula (BPBF, 2 cases), pleurobiliary fistula (PBF, 2 cases) and pericardiobiliary fistula (PCBF, 1 case). In addition to a history of cholangitis or cholelithiasis and continuous or intermittent secondary biliary tract infection, the principle manifestations of BBF included: acute, subacute or chronic biliary empyema; biliary pneumonia or biliary lung abscess; and biliptysis. The empyema perforating into trachea, bronchus, pericardium and large vessels in the thoracic cavity may result in serious consequences. Asphyxiation, pericardial tamponade and abrupt massive intrathoracic hemorrhage may also be fatal. It is often difficult to locate the fistula by bronchoscopy, however the bile found in the respiratory tract may suggest BBF. Surgical management of BBF and PCBF are discussed.

Lysine-mediated tissue osmication in combination with a tannin-osmium conductive staining method for non-coated scanning electron microscopy of biological specimens.

Glutaraldehyde fixed rat kidney blocks showed no charging effect when treated with lysine and osmic acid and viewed in a scanning electron microscope using an acceleration voltage of 5-30 kV and a specimen current of 1 X 10(-10) A. The podocyte processes and endothelial micropores of the glomerulus were visible without metal coating. Glutaraldehyde fixed, tannin-osmium impregnated and lysine-osmium treated specimens also showed no charging effect in the scanning electron microscope, yielding instead much clearer scanning images which were comparable to those obtained from gold-coated specimens or from tannin-osmium-thiocarbohydrazide-osmium impregnated specimens (Murakami and Jones, 1980).