Objective To summarize the CT features of pulmonary mucormycosis in post hematopoietic stem cell transplantation (HSCT) patients with leukemia,to provide timely and accurate guidance for clinical treatment.Methods 9 pulmonary mucormycosis in post HSCT patients with leukemia confirmed by surgery,biopsy and sputum culture were analyzed retrospectively.Distribution,morphology and CT features of the disease were analyzed and summarized.All patients were underwent non-enhanced MSCT.Results Reversed halo sign (n=7);patchy ground glass opacity (GGO) (n =5);bilateral multiple pulmonary nodules and nodular GGO (n=3);bilateral multiple pulmonary micro-cysts with spiculate boundary (n=1);pleural effusion (n=2);pneumo-mediastinum (n 1) were seen.Two or more than two CT signs were seen in some patients.The interval time between the appeared reversed halo sign and the transplantation were 0.5-19 months in 7 patients,which median time was 10 months and 6 cases (85.7％) appeared within 18months.8 patients (88.9 ％) presented cough,expectorated white or yellow phlegm,among them,4 cases (50 ％) presented bloodstained sputum or hemoptysis.3 cases (37.5 ％) presented left chest pain.1 case was asymptomatic.6 patients (66.7 ％) presented significant increased body temperature with the range from 38.4 ℃ to 39.6 C,the median was 39 C.3 patients (33.3％) showed normal temperature.The WBC counting was between 0.16-3.31 × 109/L,the median was 0.7 × 109/L.The neutrophil cell counting was between 0-2.46 × 109/L,with the median of 0.09 × 109/L.Among them,7 patients (77.8％) found reversed halo sign with neutrophil counting between 0-1.63 × 109/L,and the median was 0.09 × 109/L.Conclusion Various imaging manifestations of pulmonary mucormycosis are seen in post HSCT patients with leukemia.When patients is in agranulocytosis phase after transplantation HSCT,it is highly suggested pulmonary mucormycosis infection when the characteristic reversed halo sign is found.

Objective:To explore the efficacy and safety of eltrombopag in children with chronic primary immune thrombocytopenia (cITP).Methods:The clinical data of 13 children with cITP in Children′s Hospital of Soochow University from July 2017 to December 2021 were retrospectively analyzed.Results:Among 13 children with cITP, 7 males and 6 females, the median age was 5 years old, the median duration of illness was 2.2 years. The median therapy duration of eltrombopag was 5 months, the median maintenance dose was 25.0 mg/d, the median time to response was 4 weeks; complete response was in 5 cases, partial response was in 2 cases; 2 cases had skin disease, 1 case had arthralgia, 1 case had liver function abnormal, all complications did not cause serious impact.Conclusions:Eltrombopag was an effective and safe therapeutic option for children with cITP.

Objective To explore the significance of GBA gene mutation and gene detection in diagnosis of Gaucher disease. Method The clinical data and genetic testing results of 3 probands from 3 unrelated Gaucher families and their family members were analyzed. Results A compound heterozygous mutation of c.907C>A and c.1448T>C was found in the proband of the first family, which was inherited from parents respectively. Another complex heterozygous mutation of c.1174delC and c.1226A>G was found in the proband of second family, which was inherited from parents respectively, and the variant c.1174delC was a new mutation, which has not been reported in the literature according to the search by HGMD. The homozygous nucleotide variation of c.1342G>C and heterozygous nucleotide variation of c.1263_1317del was found in the proband of the third family and the c.1263_1317del heterozygous mutation was inherited from father. Conclusion The mutation of GBA gene was the cause of Gaucher disease in these 3 families and Gaucher disease can be diagnosed by molecular genetics in clinic.

Objective:To evaluate the effect of propofol on right ventricular hypertrophy induced by pulmonary arterial hypertension (PAH) in rats.Methods:Twenty-two clean-grade healthy adult male Wistar rats, weighing 250-280 g, were divided into 3 groups using a random number table method: control group (group C, n=8), PAH group (group PH, n=6) and propofol group (group P, n=8). In PH and P groups, monocrotaline 60 mg/kg was injected intraperitoneally to establish the model of PAH, while the equal volume of normal saline was administered in group C. Propofol 100 mg/kg was injected intraperitoneally twice a week for 6 consecutive weeks starting from 2 weeks after establishment of the model in group P. The weight of rats was measured before establishment of model and after administration, and the weight difference (△BW=weight after administration-weight before administration) was calculated.At the end of administration, the right ventricular end-diastolic dimension (RVEDD), right ventricular wall thickness in diastole (RVWTd), intraventricular septum in diastole (IVSd), left ventricular posterior wall in diastole (LVPWd) and maximal velocity of pulmonic valve (PV) were measured using cardiac ultrasound.The animals were then sacrificed, and the lungs and hearts were removed for examination of the pathological changes (after haematoxylin and eosin staining) and for determination of the degree of myocardial fibrosis in right ventricular (by Masson staining), expression of interleukin-6 (IL-6) and tumor necrosis factor-alpha (TNF-α) in lung tissues and myocardial tissues of the right ventricle (by immunohistochemistry). Results:Compared with group C, △BW and PV were significantly decreased, RVWTd, IVTd and RVEDD were increased, the thickness of the wall of pulmonary arterioles and myocardial cell penetration area in right ventricular were increased, the expression of IL-6 and TNF-α in lung tissues and myocardial tissues of right ventricle was up-regulated ( P<0.05 or 0.01), inflammatory cell infiltration and structural disorders were found in lung tissues, and intercellular spaces were widened, and the myocardial tissue was extensively fibrotic in group PH.Compared with group PH, △BW and PV were significantly increased, RVWTd, IVTd and RVEDD were decreased, the thickness of the wall of pulmonary arterioles and myocardial cell penetration area (the area of cardiomyocytes in which the nucleus located in the middle) were decreased, the expression of IL-6 and TNF-α in lung tissues and myocardial tissues of right ventricle was down-regulated ( P<0.05 or 0.01), inflammatory cell infiltration, structural disorders, intercellular spaces and degree of fibrosis were improved in group P. Conclusion:Propofol can alleviate right ventricular hypertrophy induced by PAH, and the mechanism is probably related to reduction of inflammatory responses in rats.

ObjectiveBased on ultra performance liquid chromatography-mass spectrometry（UPLC-MS） and non-targeted metabolomics technology to discuss the central regulatory effect of Chaishao Liujuntang on chronic atrophic gastritis（CAG） rats with liver-depression and spleen-deficiency， and to look for the correlation between cerebral cortex， hypothalamus and metabolic status of gastric tissues. MethodA CAG rat model with liver-depression and spleen-deficiency was established by chemical induction， hunger and satiety disorders， chronic restraint and tail clamping stimulation， lasting for 16 weeks. Twenty-eight Wistar rats were randomly divided into a blank group of 8 rats and a model group of 20 rats. After the completion of modeling， 4 rats in the model group were taken to observe the pathological changes of gastric mucosa. The remaining model rats were randomly divided into a model group of 8 rats and a Chaishao Liujuntang group of 8 rats. Chaishao Liujuntang group rats were given 5.1 g·kg^-1 by gavage， and the remaining rats were given equal volume sterilized water by gavage for 4 weeks. Macroscopic characteristics， behavioral indicators and histopathological changes of the gastric mucosa of rats in each group were observed and compared. UPLC-MS non-targeted metabolomics was used to explore the metabolic regulation effect of Chaishao Liujuntang on the cerebral cortex， hypothalamus and stomach tissues of CAG rats with liver-depression and spleen-deficiency. Pearson correlation coefficient method was used to analyze the correlation between different tissue metabolites. ResultCompared with the model group， the macroscopic characteristics of rats in Chaishao Liujuntang group were improved， such as hair color， mental state and stool properties， and the number of times of crossing and standing in the open field experiment was significantly increased， and the static time of forced swimming was significantly reduced（P<0.01）， and the gastric mucosa atrophy was reduced. The metabolic data from the cerebral cortex of rats in each group identified a total of 3 common potential biomarkers， but not enriched in pathways， 26 common potential biomarkers were identified in the hypothalamus， and the key metabolic pathways involved were mainly enriched in purine metabolism， glycerol phospholipid metabolism， D-glutamine and D-glutamic acid metabolism. Seventeen common potential biomarkers were identified in the stomach， and the key metabolic pathways involved were mainly enriched in thiamine metabolism， valine， leucine and isoleucine biosynthesis， and taurine and taurine metabolism. Correlation analysis of metabolites in different tissues revealed that multiple amino acids and their derivatives mediated metabolic connections between the cerebral cortex， hypothalamus and stomach of rats. ConclusionThe metabolic disorders in the cerebral cortex， hypothalamus and stomach of CAG rats with liver-depression and spleen-deficiency have their own characteristics， mainly manifested by changes in the content of glycerol phospholipids， fatty acids and bile acid metabolites. Moreover， Chaishao Liujuntang may play a central regulatory role in CAG rats with liver-depression and spleen-deficiency by correcting the metabolic disorders of amino acids.

ObjectiveThis article aims to investigate the association between hypertension and the risk of GSD by conducting a national multicenter study， a systematic review， and a meta-analysis. MethodsThe study was conducted in three stages. In the first stage， subjects were recruited for health examination in four hospitals in Chengdu， Tianjin， Beijing， and Chongqing， China， from 2015 to 2020， and the multivariate logistic regression analysis was used to investigate the association between hypertension and the risk of GSD in each center. In the second stage， Embase， PubMed， Wanfang Data， VIP， and CNKI databases were searched for related studies published up to May 2021， and a meta-analysis was conducted to further verify such association. In the third stage， the random effects model was used for pooled analysis of the results of the multicenter cross-sectional study and the findings of previous literature. ResultsA total of 633 948 participants were enrolled in the cross-sectional study， and the prevalence rate of GSD was 7.844%. The multivariate logistic regression analysis showed that hypertension was positively associated with the risk of GSD（P<0.05）. Subgroup analysis showed that there was no significant difference in the association between hypertension and GSD between individuals with different sexes， ages， and subtypes of GSD. A total of 80 articles were included in the systematic review and the meta-analysis， and the results showed that the risk of GSD was increased by 1.022 times for every 10 mmHg increase in diastolic pressure and 1.014 times for every 10 mmHg increase in systolic pressure. ConclusionHypertension significantly increases the risk of GSD， and the findings of this study will provide a basis for the etiology of GSD and the identification of high-risk groups.