A Unique Case of a 12-Year-Old Boy With Noonan Syndrome Combined With Noncompaction of the Ventricular Myocardium.

A 12-year-old Chinese boy was admitted with dyspnea after exercise. Based on his clinical features, echocardiography tests, and family history, he was diagnosed with Noonan syndrome (NS) combined with noncompaction of the ventricular myocardium (NVM). Noonan syndrome (NS) is a common syndrome, but to the best of our knowledge, our case is the first reported case of NS combined with NVM. In our case, the detected mutated genes may be inherited and unreported genes caused NS or NVM. Our research may enrich our knowledge about NS and contribute to furthering our understanding of the pathogenesis and treatment. In summary, we present a unique case of NS combined with NVM.

[Effects of Fluoxetine on Nogo Expression and Collagen Production with Decrease of Pulmonary Artery Pressure in Rats with Right Ventricular Failure.]

OBJECTIVES: To investigate the effect of fluoxetine on neurite growth inhibitor (Nogo) expession and collagen production of cardiac tissue in rats with right heart failure and pulmonary hypertension. METHODS: Thirty one male SD rats were randomly divided into the treatment group,right heart failure group and normal control group.The rats in the treatment group and right heart failure group received intrapertioneal injection of monocrotaline (MCT,60 mg/kg) to induce pulmonary hypertension and right heart failure.After 21 days,the rats in treatment group were given fluoxetine of 10 mg/(kg×d) by gavage per day for 21 days,the rats in the other two groups were given saline.HE staining was used to observe the pulmonary artery and right ventricular myocardial tissue in rats.The collagen formation in right ventricular myocardium was observed by Masson staining.The expressions of Nogo-A, Nogo-B,type1collagen and type 3 collagen mRNA in myocardium were measured by real-time fluorescence quantitative PCR,while the semi quantitative measurement of Nogo protein level was detected by Western blot. RESULTS: After the intervention of fluoxetine,pulmonary artery stenosis was significantly reduced,myocardial tissue lesion decreased,collagen synthesis decreased in right ventricular myocardium.RT-PCR showed that mRNA of Nogo-A decreased,and mRNA of Nogo-B increased (P<0.05).Western blot showed that the expression of Nogo-A protein decreased,while Nogo-B1 protein expression increased (P<0.05),Nogo-B2 expression was not significantly changed (P>0.05). CONCLUSIONS: Nogo may affect the collagen synthesis in right heart failure,and partly involved in myocardial fibrosis.

HSP90/LSD1 dual inhibitors against prostate cancer as well as patient-derived colorectal organoids.

The rational installation of pharmacophores targeting HSP90 and LSD1 axes has achieved significant anti-cancer capacity in prostate and colorectal cancer. Among the series of hybrids, inhibitor 6 exhibited remarkable anti-proliferative activity against prostate cancer cell lines PC-3 and DU145, with GI50 values of 0.24 and 0.30 µM, respectively. It demonstrated notable efficacy in combinatorial attack and cell death initiation towards apoptosis. The cell death process was mediated by PARP induction and γH2AX signaling, and was also characterized as caspase-dependent and Bcl-xL/Bax-independent. Notably, no difference in eye size or morphology was observed in the zebrafish treated with compound 6 compared to the reference group (AUY922). The profound treatment response in docetaxel-resistant PC-3 cells highlighted the dual inhibitory ability in improving docetaxel sensitivity. Additionally, at a minimum concentration of 1.25 µM, compound 6 effectively inhibited the growth of patient-derived colorectal cancer (CRC) organoids for up to 10 days in vitro. Together, the designed HSP90/LSD1 inhibitors present a novel route and significant clinical value for anti-cancer drug therapy.

[Clinical analysis of post pancreatoduodenectomy hemorrhage].

OBJECTIVE: To explore the factors of post pancreatoduodenectomy hemorrhage. METHODS: The clinical data of 263 cases between January 1998 and April 2008 underwent pancreatoduodenectomy were analyzed prospectively. RESULTS: The overall mortality rate was 4.94% (13/263). Postoperative bleeding occurred in 23 patients (8.75%), with 8 episodes ending fatally (34.8%). The tumor size, Child classification, caput total resection and pancreatic leakage were identified as significant risk factors for post pancreatoduodenectomy hemorrhage by means of univariate analysis. The multivariate Logistic regression analysis revealed that all of the five factors turned out to be the independent risk factors. CONCLUSIONS: The prevention of these bleeding complications depends in the first place on meticulous hemostatic technique. The pancreatic leakage is also one of the most important factors due to postoperative bleeding. The prophylactic use of somatostatin is not necessary.

Application of three-dimensional visualization technique in preoperative planning of progressive hilar cholangiocarcinoma.

OBJECTIVE: This study aims to investigate the role of three-dimensional visualization technique in the diagnosis and treatment of progressive hilar cholangiocarcinoma. METHODS: From January 2014 to February 2017, a three-dimensional visualization model was set up in 23 patients with progressive hilar cholangiocarcinoma. The distributions and variations of the hepatic portal ducts were observed. The tumors were classified based on Bismuth classification. The simulation operation was performed and the operation plan was established. RESULTS: All 23 patients revealed a clear relationship between the intrahepatic and extrahepatic ducts, as well as the tumors and ducts. An individualized surgery program was established through the accurate calculation of liver volume and residual liver volume. Among these patients, 13 patients completed radical resection of hilar cholangiocarcinoma combined with massive hepatectomy. No bile leakage occurred and no operative death was found. CONCLUSION: For patients with progressive hilar cholangiocarcinoma, the optimized three-dimensional visualization technique can accurately demonstrate the dilated biliary tract system, provide a new standard to determine the presence of tumor and peripheral vascular invasion, help in establishing a reasonable individualized operation plan, reduce the incidence of bile leakage and liver failure after the operation, and improve the success rate of operation.

Improved outcomes after complete resection of underlying tumors for patients with paraneoplastic pemphigus: a single-center experience of 22 cases.

BACKGROUND: Paraneoplastic pemphigus (PNP) is an autoimmune-related acquired bullous disease. Delayed diagnosis and treatment of this clinically rare disease often result in poor prognosis. METHODS: Between January 1999 and December 2009, 22 patients with confirmed PNP who underwent surgical resection of underlying tumors were enrolled in this study. Clinicopathologic characteristics, treatment options, and perioperative and long-term results were analyzed. RESULTS: Among 22 patients, 2 patients died of severe infection several weeks after surgery. Postoperative major complications included pulmonary infections (n = 10) and septicemia (n = 4). Respiratory symptoms persisted in 13 patients. Tumors were completely resected in 20 patients. Two patients whose tumors were not completely resected died of relapse 2 and 32 months after surgery. Two patients with completely resected tumors died of respiratory failure 10 and 24 months after surgery, respectively. One patient whose pathological result was follicular dentritic cell sarcoma had a relapse recently. The remaining 15 patients have survived till now. CONCLUSIONS: Early detection, prompt treatment, and complete resection of PNP can effectively decrease the mortality and speed up the recovery.

Prognostic factors after pancreatoduodenectomy for distal bile duct cancer.

Prognostic factors influencing long-term survival after radical resection for distal bile duct cancer have not been well established because of the rarity of this malignancy. The goal of this study was to identify main prognostic factors in patients undergoing pancreatoduodenectomy for distal bile duct carcinoma. A retrospective study consisting of 122 patients with distal bile duct cancer who underwent pancreatoduodenectomy in three major university hospitals was performed to identify the main prognostic factors. Major surgical complications occurred in 40 patients (32.8%), of whom eight died (6.6%) in the hospital. Overall actuarial survival (excluding hospital deaths) at 1-, 3-, and 5-year follow-up was 82.9, 49.4, and 32.7 per cent, respectively, with a median survival of 36 months. Univariate analysis showed that papillary tumor (P = 0.045), negative surgical margin (R0 resection, P = 0.005), earlier pT (P = 0.005), pTNM stage (P < 0.001), and absence of lymph node involvement (P < 0.0001) were significant predictors of survival. On multivariate analysis, only lymph node metastasis was shown to be an independent prognostic factor of survival (P = 0.036). Lymph node involvement was the most important survival predictor after a Whipple resection in patients with distal cholangiocarcinoma.

Carcinoma of the ampulla of Vater: factors influencing long-term survival of 127 patients with resection.

INTRODUCTION: The prognosis for patients with carcinoma of the ampulla of Vater is improved relative to other periampullary neoplasms. Identification of independent prognostic factors in ampullary carcinomas has been limited by the small number of tumors resected. The aim of the present study was to determine the clinicopathologic factors that influence long-term survival in patients with resected ampullary carcinoma. METHODS: Clinicopathologic data were retrospectively reviewed for patients with ampullary carcinomas radically resected between March 1987 and September 2002. The correlation between clinicopathologic variables and survival of patients after resection was examined by the Kaplan-Meier method, the log-rank test, and Cox proportional hazards regression. Ampullary carcinomas were radically resected in 127 patients either by pancreaticoduodenectomy (n = 124) or local resection (n = 3). RESULTS: Hospital mortality was 9.7%. The overall actuarial survival rates (including hospital deaths) at 1, 3, 5, and 10 years were 76.2%, 46.8%, 43.3%, and 35.7%, respectively. Factors that significantly influenced survival were lymph node status (P < 0.001), depth of tumor infiltration (P = 0.029), and TNM stage (P < 0.001) on univariate analysis. On multivariate analysis, both depth of infiltration and lymph node status were the independent determinants of survival after resection (P = 0.003, P = 0.005, respectively). CONCLUSIONS: Carcinoma of the ampulla of Vater has a higher resectability rate and a much better survival rate than pancreatic cancer. Pancreaticoduodenectomy is the treatment of choice for this tumor. Long-term survival was independently influenced by the depth of tumor infiltration and lymph node metastasis.

[Study on production of acrylamide by microbial method (II)--enzyme catalytic kinetics and de-active dynamics of nitrile hydratase].

The hydration reaction by microbial method is the crisis of the procedure of acrylamide production from acrylonitrile. This research studied the enzyme catalytic kinetics and de-active kinetics of nitrile hydratase in the type of free cell. Firstly, the effects of the concentration of cells, the temperature, pH value, the concentration of acrylonitrile and the concentration of acrylamide on the activity of nitrile hydratase was investigated. The result is that the temperature and the concentration of acrylamide are the most important among these factors. The activity of the nitrile hydratase was 5659 u/mL (broth) at 28 degrees C; the counterpart was only 663 u/mL (broth) at 5 degrees C. And the activity of NHase in solution of 45% acrylamide was just about half of that in solution of 5% acrylamide. After study on the relation of temperature and the reaction speed, It was found that the activation energy of the hydration of NHase was 65.57 kJ.mol-1. This paper studied the effects of concentration of cells, temperature, pH value, concentrations of acrylonitrile and acrylamide on the deactivation of Nhase, as well as the related enzyme de-active kinetics. The result also showed that the temperature and the concentration of acrylamide are the most important among these factors. In solution of 35% acrylamide, the residual activity was about 0% of the original value after 55 h; but in solution of 10% acrylamide, after the same period of time, the residual activity was 50% of the original one. It was also found that the concentration of acrylonitrile had little effect on the stability of NHase. The coefficient of deactivation at 28 degrees C was 21.77 times of the one at 5 degrees C. Correlating the temperature and the coefficient of deactivation, the activation energy of the de-active reaction was found to be 92.28 kJ.mol-1.

[Study on production of acrylamide by microbial method (I)--Culture of bacterium cells and expression of high activity of nitrile hydratase].

The cultural conditions for the growth of Norcardia cell were studied in this paper. Controlling pH value, adding nutrient and optimizing the quantity of inducer during cultivation, the activity of nitrile hydratase reached 6567 u/mL (culture medium), which was the highest value appeared in native journals. In the farther hydratase experiments, no by-product, crylic acid, was detected. It showed that the activity of amidase was not promoted obviously while the activity of nitrile hydratase was increased greatly. The results set a strong foundation for the industrial application and the research on new technology.