In order to investigate the potential anti-leukemic effect of cyclosporin A(CsA), MTT method and cell viability assay in vitro were carried out in this study to observe the effect of CsA on the proliferation and cell viability of various leukemic cell lines, such as T-cell Jurkat, Molt-4, CCRF-CEM, Nalm-6, K562 and multi-drug-resistant leukemic cell line K562/AO2. The results fully showed that CsA did possess the same cytotoxic action on all the leukemic cell lines, particularly including multi-drug-resistant leukemic cell line,and could then inhibit the proliferation and cell viability of these leukemic cells, thereby indicating that CsA might be applied as one of the new, safe and effective anti-leukemic agents when used with clinically adoptable dosage in leukemias.

Objective To study the application of fusion of CT and MRI images in X-knife treatment for intracranial lesions.Methods Total 25 patients included:3 gliomas,3 acoustic neuroma,2 pituitary adenoma,one craniopharygioma to be remained or recurred postoperatily,2 pituitary micro-adenoma,5 AVM,4 cavernous angioma,3 metastatic tumour,one neoplasm located pituitary stem and midbrain respectively.Before the fusion of CT and MRI images MRI scan and CT scan for location of X-knife were performed respectively,then MR and CT image were transferred to workstation for the fusion of images.Results All lesions were showed clearly on fusional images and more nodules were observed in 3 metastatic tumour.Skull,soft tissue constructures and the profile of lesions were completely overlaped on overlaped images of CT and MR with an error less 1.0 mm.Conclusion CT and MRI images of head can be accurately registered.The images can show the radiologic informations more clearly than conventional CT image.It provides a safe,effective and little harmful method for X-knife treatment of intracranial lesions.

Objective To observe the clinical characteristics of 2 patients with mucolipidosis type Ⅲ. Methods Two sibling patients (a sister and a brother) with mucolipidosis type Ⅲ symptoms and other family members were the research objects, and the changes of their features of hand bone imaging, blood indexes [blood glucose, cholesterol, triacylglycerol (TG), total protein (TP), albumin (Alb)] and body composition were analyzed. Results Except the 2 patients, the bone morphology, blood indexes and body compositions in other 7 family members were under normal conditions. The phalanx intervals of both hands in 2 patients with mucolipidosis type Ⅲwere widened significantly, among them the thumb manifestation was more obvious; the distal segments of phalanxes in both hands became pointed and curved presenting a "claw-like hand" deformity; the metacarpal and distal carpal metaphysis were obviously enlarged, and scaphoid, lunate, trianglar, orbicular, and trapezium and trapezoid bones were loosely arranged at the wrist; the distal ends of ulna and radius were markedly enlarged. Compared to healthy people, the triglyceride levels of serum in the 2 patients were obviously reduced (the percentage of reduction: 57.14% and 41.07% respectively); body mass indexes (BMI), total fat and visceral fat were significantly lowered (BMI reduction percentage:26.81% and 14.55%, total fat reduction percentage: 38.12% and 44.95%, visceral fat reduction percentage: 62.25% and 67.74%, respectively) in the two patients. Conclusion The purpose of studying the biochemistry indexes, imaging characteristics and body compositions is to more deeply understand the clinical symptoms and signs of the 2 sibling patients with mucolipidosis type Ⅲ in a family to provide a theoretical reference.

Primary hyperhidrosis is a chronic disease that does not meet the normal body temperature regulation and causes excessive sweating. The common sweating parts include the head, palm, armpit, chest and back, and the soles of the feet, which can seriously affect the patient's learning, socialization, and jobs. Radiofrequency thermocoagulation is a safe and effective treatment for primary hyperhidrosis. This article reviews the research progress of radiofrequency thermocoagulation in the treatment of primary hyperhidrosis, so as to provide some references for clinical workers.

Objective:To study the treatment efficacy of percutaneous radiofrequency thermocoagulation in patients with hyperhidrosis of heads and palms.Methods:Thirty patients with primary hyperhidrosis of heads and palms, admitted to our hospital from June 2017 to May 2019, were chosen in our study. CT-guided percutaneous puncture of T 3 or T 4 and sympathetic ganglion radiofrequency thermocoagulation were given to all patients. The evaluation of curative effects and complications of these patients were summarized during the 12 months of follow up. Results:The symptoms of hyperhidrosis in 24 patients got significant improvement, enjoying postoperative satisfaction rate of 80%. During the surgery, 5 patients suffered thoracic and lung puncture injury, including 4 with pneumothorax and one with hemothorax. Seven patients experienced pain and numbness in the chest, back, armpit or upper arm after surgery; 10 patients developed compensatory hyperhidrosis of the back, and two patients developed compensatory hyperhidrosis of the back and bilateral feet.Conclusion:Percutaneous radiofrequency is an effective treatment for hyperhidrosis that provides excellent immediate and long-term effect, as well as low complication rate.

OBJECTIVEMucopolysaccharidosis(MPS) is a congenital hereditary disease. Only a few patients with this disease can be controlled by enzyme replacement therapy. Most of them are short of effective interference. To exploit the effect of treatment with allogenic hematopoietic stem cell transplantation, two children were treated with the transplantation.

METHODSThe two patients included a 23 month MPS-IH and an 18 month old MPS-VI at the time of transplantation. Busulfan of 20 mg/kg plus 200 mg of Cyclophosphamide were used as the conditioning regimen. Peripheral stem cells were collected from a 9/10 high resolution matched unrelated donor and a matched sibling carrier donor, respectively. The heart and lung were affected in the patient with MPS-IH. Medium obstructed pulmonary impairment was found by pulmonary function test at the time of transplantation. Medium mitral valve countercurrent and patent ductus arteriosis(PDA) were found by Doppla examination.

RESULTSThe number of hematopoietic stem cells was comparative between the two donors with total nucleated cells and CD34+ cells of 11 x 10(8)/kg and 17 x 10(8)/kg, and 7.6 x 10(6)/kg and 7.2x 10(6)/kg respectively. Neutrophil engrafted at day 11. The process of transplantation in the MPS-VI patient went smoothly with grade II graft versus host disease(GVHD) briefly and only 1 U RBC and 2 U platelet were transfused. For the MPS-IH patient, the process of transplantation was tough with platelet reaching to 20 x 10(9)/L till day 40 and 5 U RBC and 7 U platelet were transfused during transplantation. Grade III GVHD was resolved by steroid, mycophenolate mofetil (MMF) and CD25 antibody. Pneumonia recurred 3 times with 2 times rescued by trachea intubation and mechanical ventilation because of accompanying acute heart failure. At day 14 the lymphocytes in both patients were 100% from donors as evidenced by short tandem repeat-PCR(STR-PCR). MPS associated enzyme activity was increased to 70 nmol/h.mg and 66 nmol/h.mg at 3 month and still remained 50.9 nmol/h.mg and 44.5 nmol/h.mg at 2 years post transplantation. Till now the 2 patients have been followed up for 25 months and 28 months with good general condition. The cardiac and pulmonary functions have improved obviously in the MPS-IH patient. The cornea became clear in this patient.

CONCLUSIONAllogeneic hematopoietic stem cell transplantation is an effective measure to treat patient with MPS-IH and MPS-VI. Transplantation at earlier stage of age can decrease transplant related complications. It requires longer time follow up for observing the clinical effects for these patients.

Female ; Follow-Up Studies ; Graft vs Host Disease ; drug therapy ; etiology ; Hematopoietic Stem Cell Transplantation ; adverse effects ; methods ; Humans ; Infant ; Intraoperative Complications ; drug therapy ; etiology ; Male ; Mucopolysaccharidoses ; enzymology ; pathology ; physiopathology ; surgery ; Recovery of Function ; Transplantation, Homologous

Hippo signaling pathway is highly conservative in evolution. MST1/2, LATS1/2, and the effector protein YAP/TAZ are the core members of this signaling pathway in mammalian cells. There have been many studies on YAP/TAZ and its downstream, however, the upstream regulatory factors of the Hippo signaling pathway remain unclear, and become one of the hot research directions of this pathway at present. In addition, Hippo signaling pathway can cross-talk with other signaling pathways such as Wnt and Notch signaling pathways, and plays an important role in controlling organ size, maintaining tissue homeostasis, and promoting tissue repair and regeneration. Abnormal Hippo signaling pathway may lead to the occurrence of a variety of tumors, especially gastrointestinal cancers such as liver cancer, colorectal cancer and gastric cancer. The abnormal expression of its members in gastrointestinal cancers is related to cancer cell proliferation, apoptosis, invasion and migration. Hippo signaling pathway is vital for liver repair and regeneration. Its inactivation will lead to the occurrence of primary liver cancer. The mechanism of YAP in liver cancer mainly depends on TEAD-mediated gene transcription. Hippo signaling pathway is also important for maintaining intestinal homeostasis, and its imbalance can lead to the occurrence and recurrence of colorectal cancer. In primary and metastatic gastric cancer, the expression of YAP/TAZ is significantly up-regulated, but the specific molecular mechanism is unclear. This article summarizes the recent progress on Hippo signaling pathway and its upstream regulatory factors, its roles in the development of gastrointestinal cancers and related molecular mechanisms; and also discusses the future research directions of Hippo signaling pathway.
Apoptosis ; physiology ; Cell Proliferation ; physiology ; Gastrointestinal Neoplasms ; physiopathology ; Humans ; Signal Transduction