CT and MR imaging features of soft tissue rhabdoid tumor: compared with rhabdomyosarcoma in children.

Objective: To assess the computed tomography (CT) and magnetic resonance (MR) imaging characteristics of soft tissue rhabdoid tumors (RT) and compare them with those of rhabdomyosarcoma (RMS). Methods: We conducted a retrospective analysis of 49 pediatric patients from 2011 to 2022, comprising 16 patients with soft tissue RT and 33 patients with RMS who underwent CT or MRI scans. Key imaging features, as well as clinical and pathological data, were compared between the two groups. The multivariate logistic regression analysis was used to determine independent differential factors for distinguishing soft tissue RT from RMS, and the model was established. The final prediction model was visualized by nomograms and verified internally by using a bootstrapped resample 1,000 times. The diagnostic accuracy of the combined model was assessed in terms of discrimination, calibration, and clinical utility. Results: Age, sex, number of lesions, and primary locations were similar in both groups. The imaging characteristics, including margin, calcification, surrounding blood vessels, and rim enhancement, were associated with the two groups of soft tissue tumors, as determined by univariate analysis (all p < 0.05). On multivariate logistic regression analysis, the presence of unclear margin (p-value, adjusted odds ratio [95% confidence interval]: 0.03, 7.96 [1.23, 51.67]) and calcification (0.012, 30.37 [2.09, 440.70]) were independent differential factors for predicting soft tissue RT over RMS. The presence of rim enhancement (0.007, 0.05 [0.01, 0.43]) was an independent differential factor for predicting RMS over soft tissue RT. The comprehensive model established by logistic regression analysis showed an AUC of 0.872 with 81.8% specificity and 81.3% sensitivity. The decision curve analysis (DCA) curve displayed that the model achieved a better net clinical benefit. Conclusion: Our study revealed that the image features of calcification, indistinct margins, and a lack of rim enhancement on CT and MRI might be reliable to distinguish soft tissue RT from RMS.

The value of oxygen index and base excess in predicting the outcome of neonatal acute respiratory distress syndrome

Abstract Objective This study aimed to identify the predictors and threshold of failure in neonatal acute respiratory distress syndrome. Methods Newborns with severe acute respiratory distress syndrome aged 0-28 days and gestational age ≥36 weeks were included in the study if their cases were managed with non-extra corporal membrane oxygenation treatments. Patients were divided into two groups according to whether they died before discharge. Predictors of non-extra corporal membrane oxygenation treatment failure were sought, and the threshold of predictors was calculated. Results A total of 103 patients were included in the study. A total of 77 (74.8%) survived hospitalization and were discharged, whereas 26 (25.2%) died. Receiver operating characteristic analysis of oxygen index, pH, base excess, and combinations of these indicators demonstrated the advantage of the combination of oxygen index and base excess over the others variables regarding their predictive ability. The area under the curve for the combination of oxygen index and base excess was 0.865. When the cut-off values of oxygen index and base excess were 30.0 and −7.4, respectively, the sensitivity and specificity for predicting death were 77.0% and 84.0%, respectively. The model with base excess added a net reclassification improvement of 0.090 to the model without base excess. Conclusion The combination of oxygen index and base excess can be used as a predictor of outcomes in neonates receiving non-extra corporal membrane oxygenation treatment for acute respiratory distress syndrome. In neonates with acute respiratory distress syndrome, if oxygen index >30 and base excess <−7.4, non-extra corporal membrane oxygenation therapy is likely to lead to death.

Familial Hemophagocytic Lymphohistiocytosis Type 2 in a Chinese Infant with PRF1 Homozygous Mutation: a Case Report.

BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening condition of hyperinflammation caused by uncontrolled proliferation of activated lymphocytes and histiocytes. Familial HLH (fHLH) is an autosomal recessive disease. METHODS: We report a case of fHLH in a 45-day-old Chinese female infant presenting with fever, hepatosplenomegaly, and pancytopenia. Typical laboratory findings were detected for the infant, and fHLH was diagnosed according to the HLH-2004 guidelines. The infant was initially treated with antibiotics and ganciclovir prior to diagnosis of HLH. No response to glucocorticoid and intravenous immunoglobulin treatment was observed, and the infant died of disseminated intravascular coagulation. RESULTS: A mutation in the perforin gene was identified in this patient. Direct sequencing analysis revealed a deleterious homozygous mutation of PRF1, c.65delC [p.P22Rfs*29], and her parents were heterozygous carriers of the mutant alleles. CONCLUSIONS: Therefore, familial HLH is still a potentially lethal childhood illness, and appropriate individualized therapies, including cell therapy and gene targeting therapy, need to be established.

Blocking of thromboxane A2 receptor attenuates airway mucus hyperproduction induced by cigarette smoke.

Cigarette smoking is one of the risk factors for chronic obstructive pulmonary disease (COPD). In this study, we investigated the effects of thromboxane A2 (TxA2) receptor antagonists on airway mucus production induced by cigarette smoke. Rats were exposed to cigarette smoke 1h/day, 6 days/week for 4 weeks. Seratrodast (2, 5, 10mg/kg day) was administered intragastrically prior to smoke exposure. Thromboxane B2 (TxB2) in the bronchoalveolar lavage fluid and lung tissues was determined by enzyme immunoassay. Airway mucus production was determined by alcin-blue/periodic acid sthiff (AB-PAS) staining, Muc5ac immunohistochemical staining, and RT-PCR. The phosphorylation of ERK and p38 was evaluated by Western blotting. Seratrodast reduced the overproduction of TxB2 in both bronchoalveolar lavage fluid and lung tissues. Cigarette smoke exposure markedly increased AB/PAS-stained goblet cells and rat Muc5ac expression in the airway, which was significantly attenuated by seratrodast administration. The induced phosphorylation of ERK and p38 was also attenuated by seratrodast. TxA2 receptor antagonist could reduce Muc5ac production induced by cigarette smoke in vivo, possibly through the mitogen-activated protein kinases (MAPK) signaling pathway.

Blockade of advanced glycation end product formation attenuates bleomycin-induced pulmonary fibrosis in rats.

BACKGROUND: Advanced glycation end products (AGEs) have been proposed to be involved in pulmonary fibrosis, but its role in this process has not been fully understood. To investigate the role of AGE formation in pulmonary fibrosis, we used a bleomycin (BLM)-stimulated rat model treated with aminoguanidine (AG), a crosslink inhibitor of AGE formation. METHODS: Rats were intratracheally instilled with BLM (5 mg/kg) and orally administered with AG (40, 80, 120 mg/kg) once daily for two weeks. AGEs level in lung tissue was determined by ELISA and pulmonary fibrosis was evaluated by Ashcroft score and hydroxyproline assay. The expression of heat shock protein 47 (HSP47), a collagen specific molecular chaperone, was measured with RT-PCR and Western blot. Moreover, TGFbeta1 and its downstream Smad proteins were analyzed by Western blot. RESULTS: AGEs level in rat lungs, as well as lung hydroxyproline content and Ashcroft score, was significantly enhanced by BLM stimulation, which was abrogated by AG treatment. BLM significantly increased the expression of HSP47 mRNA and protein in lung tissues, and AG treatment markedly decreased BLM-induced HSP47 expression in a dose-dependent manner (p < 0.05). In addition, AG dose-dependently downregulated BLM-stimulated overexpressions of TGFbeta1, phosphorylated (p)-Smad2 and p-Smad3 protein in lung tissues. CONCLUSION: These findings suggest AGE formation may participate in the process of BLM-induced pulmonary fibrosis, and blockade of AGE formation by AG treatment attenuates BLM-induced pulmonary fibrosis in rats, which is implicated in inhibition of HSP47 expression and TGFbeta/Smads signaling.

[Effects of phosphodiesterase-4 inhibitor on the mucin secretion in airway stimulated with acrolein: experiment with rats].

OBJECTIVE: To examine the effects of YM976, a phosphodiesterase (PDE)4 inhibitor, on mucin hypersecretion of airway stimulated with acrolein. METHODS: Forty-eight SD rat were randomly divided into 6 equal groups. Twenty-four rats underwent gastric perfusion of YM976 0.5, 1.5, or 4.5 mgxkg(-1)xd(-1) (n=8 for each group) and then underwent aerosol inhalation of 3.0 ppm acrolein 6 hours per day for 12 days so as to establish rat models of airway mucus hypersecretion. Eight rats underwent aerosol inhalation of 3.0 ppm acrolein only for 12 days to establish rat models of airway mucus hypersecretion without any drug intervention (acrolein model group). Eight rats underwent gastric perfusion of 5% methylcellulose and then acrolein inhalation for 12 days (methylcellulose group), and another 8 rats underwent gastric perfusion of YM976 4.5 mg/kg once a day and then inhalation of normal saline for 12 days (YM976 control group). On the 13th day the rats were killed. Bronchoalveolar lavage fluid (BALF) was acquired for cell count, and ELISA was used to detect the levels of tumor necrosis factor (TNF)-alpha and cytokine-induced neutrophil chemoattractant (CINC)-1 in the BALF. The mucin level in the airway mucous membrane was detected by alcian blue(AB)/periodic acid-Schiff(PAS) method. Muc5ac protein was measured by immunohistochemical staining and Western-blotting, and Muc5ac mRNA was detected by RT-PCR. RESULTS: The percentages of positive AB/PAS staining of the acrolein model group and the 3 YM976 groups (0.5, 1.5, and 4.5 mgxkg(-1)xd(-1)) were 23.65+/-2.86, 22.63+/-2.12, 16.34+/-1.72, and 5.03+/-0.72 respectively. The integral optical density (IOD) levels of Muc5ac immunohistochemical staining of the methylcellulose group and the 3 YM976 groups were 0.54+/-0.05, 0.49+/-0.06, 0.32+/-0.04, and 0.22+/-0.04 respectively. The IOD of Muc5ac protein expression by Western-blotting of the methylcellulose group and the 3 YM976 groups were 1.177+/-0.190, 0.806+/-0.180, 0.303+/-0.061, and 0.134+/-0.035 respectively. The IOD of Muc5ac mRNA expression by RT-PCR of the methylcellulose group and the 3 YM976 groups were 0.246+/-0.021, 0.223+/-0.020, 0.161+/-0.012, and 0.097+/-0.011 respectively. The TNF-alpha and CINC-1 levels in the BALF of the methylcellulose group and the 3 YM976 groups were (96.77+/-2.31), (87.65+/-2.75), (73.56+/-2.62), and (52.23+/-2.79) microg/L respectively, and (145.75+/-4.43), (139.73+/-5.51), (95.34+/-5.13), and (65.74+/-5.62) microg/L respectively, all significantly higher than those of the normal control group [4.38+/-0.32, 0.12+/-0.02, 0.058+/-0.024, 0.061+/-0.006, (18.23+/-2.32) microg/L and (33.56+/-3.43) microg/L respectively, all P<0.05], but those of the YM976 1.5 mgxkg(-1)xd(-1) and 4.5 mgxkg(-1)xd(-1) groups being significantly lower than those of the methylcellulose group (both P<0.05). CONCLUSION: YM976 inhibits airway inflammatory response and airway mucin hypersecretion induced by acrolein.

[Effect of transfection of hEndostatin gene on CNE2 cell xenograft growth in nude mice].

BACKGROUND & OBJECTIVE: Tumor angiogenesis play an important role in growth and metastasis of cancer. Angiogenesis inhibitors induce apoptosis in cancer by inhibiting tumor angiogenesis and have strong inhibiting effect on both growth and metastasis of cancer. This study was designed to explore the effect of transfection of human endostatin gene on nasopharyngeal carcinoma CNE2 cells xenograft growth in nude mice. METHODS: The plasmids (pBlast-hIL-hEndostatin, pBlast-hEndostatin, and pBlast-MCS) were transfected and lipofectin-mediated into the CNE2 cell line. The biological activity of secreted hEndostain from gene-transferred cell lines was determined using MTT method in vitro. Then the transfected CNE2 cells were injected into the nude mice and tumorigenicity of CNE2 was observed in vivo. RESULTS: The supernatant of CNE2 cell transfected with pBlast-hIL-hEndostatin effectively inhibited the growth of endothelial cell (ECV304). The volume and the weight of tumor in pBlast-hIL -hEndostatin transfecting cells group were less than those in control group (P< 0.01). The growth speed of tumor in pBlast-hIL-hEndostatin transfecting cells group was slower than that in control group. CONCLUSION: Transfection of hEndostatin gene could inhibit CNE2 cell growth in nude mice.