骨纤维异常增殖症的X线诊断与鉴别诊断

目的了解骨纤维异常增殖症在各部位上的X线表现及鉴别诊断。方法对本院确诊病例进行回顾性分析研究。结果骨纤维异常增殖症在各骨上的X线表现不尽相同。按其形态及内部骨质改变情况大致分为5种类型。磨玻璃型:患骨膨胀增粗,病患区显示密度均匀一致的无小梁结构区,呈磨玻璃样改变;囊肿型:表现为囊状透光区;丝瓜瓤型:在囊状膨胀性改变的背景上,可见不规则的粗大骨小梁,粗大骨小梁和沿纵轴或不规则排列,前后重叠交织,呈“丝瓜瓤样”改变;虫噬型:呈多发的点状溶骨性破坏,边缘锐利如虫噬一样;硬化型:表现为骨质密度增高,范围较大。可数种并存以其中一种改变为主,也可单独存在。结论骨纤在人体各部位均可发生,X线表现虽有不同,但也有其特征性表现。

BCR-ABL1基因阴性骨髓增殖性肿瘤临床分析

目的通过对骨髓增殖性肿瘤(MPN)骨髓活检形态学、基因突变检测及临床特征的病例分析,为提高疾病的诊断水平,减少临床漏诊和误诊提供可靠依据。方法回顾性分析183例BCR-ABL1基因阴性的骨髓增殖性肿瘤患者骨髓细胞形态学、骨髓病理学、基因突变检测及临床资料。结果骨髓粒系细胞比例、红系细胞比例及巨核细胞计数,在3组疾病间比较差异无统计学意义(P>0.05)。JAK2 V617F体细胞突变发生在100%的真性红细胞增多症(PV)、57%的原发性血小板增多症(ET)、50%的原发性骨纤(PMF);CALR基因突变发生在35%的ET、46%的PMF,MPL基因突变在7%的ET、2%的PMF。PV以Ⅲ型少分叶核巨核细胞为主,ET以Ⅳ型(巨大)多分叶核巨核细胞为主,PMF以Ⅰ型核固缩巨核细胞为特征。结论 MPN的诊断必须要结合临床病史、骨髓形态学、骨髓活检组织病理学、融合基因和分子生物学等相关检验结果进行综合判断。

Giant monostotic osteofibrous dysplasia of the ilium: A case report and review of literature

BACKGROUNDOsteofbrous dysplasia （OFD） is a developmental ske-letal disorder, and cases with a giant affected area in the pelvis are rare.CASE SUMMARYIn this case report, a 48-year-old man presented with a large tumor in the right iliac region that turned out to be OFD. The patient had rebound tenderness in his right hip. After radiography examination, magnetic resonance imaging examinations and some physical examination, extensive bone destruction in the right ilium was confirmed. Moreover, changes in bone mi-neral density and peripheral cortical bone sclerosis with surrounding soft tissue swelling were observed. Thus, this patient was considered to have giant monostoticOFD of the ilium. The tumor-related area was removed completely by surgery, and the remaining cavity was filled by artificial bones from the opposite ilium. According to the results of follow-up, the patient had normal fexion and extension activities of the right hip joint, and there was no evidence of recurrence of the tumor.CONCLUSION Suture of iliopsoas and gluteus medius muscle following focus curettage and bone grafting is a promising and effective method to treat giant OFD of the ilium. It is a feasible way to fll a large cavity after removing a lesion like the one is this case.

Multiple esophageal variceal ruptures with massive ascites due to myelofibrosis-induced portal hypertension

A 75-year old man had been diagnosed at 42 years of age as having polycythemia vera and had been monitored at another hospital. Progression of anemia had been recognized at about age 70 years, and the patient was thus referred to our center in 2008 where secondary myelofibrosis was diagnosed based on bone marrow biopsy findings. Hematemesis due to rupture of esophageal varices occurred in January and February of 2011. The bleeding was stopped by endoscopic variceal ligation. Furthermore, in March of the same year, hematemesis recurred and the patient was transported to our center. He was in irreversible hemorrhagic shock and died. The autopsy showed severe bone marrow fibrosis with mainly argyrophilic fibers, an observation consistent with myelofibrosis. The liver weighed 1856 g the spleen 1572 g, indicating marked hepatosplenomegaly. The liver and spleen both showed extramedullary hemopoiesis. Myelofibrosis is often complicated by portal hypertension and is occasionally associated with gastrointestinal hemorrhage due to esophageal varices. A patient diagnosed as having myelofibrosis needs to be screened for esophageal/gastric varices. Myelofibrosis has a poor prognosis. Therefore, it is necessary to carefully decide the therapeutic strategy in consideration of the patient's concomitant conditions, treatment invasiveness and quality of life.

Effects of Guiyuanfang and autologous transplantation of bone marrow stem cells on rats with liver fibrosis

AIM: To investigate the therapeutic effects of Guiyuanfang and bone marrow stem cells (BMSCs) on rats with liver fibrosis.METHODS: Liver fibrosis model was induced by carbon tetrachloride, ethanol, high lipid and assessed biochemically and histologically. Liver function and hydroxyproline contents of liver tissue were determined.Serum hyaluronic acid (HA) level and procollagen Ⅲ level were performed by radioimmunoassay. The VG staining was used to evaluate the collagen deposit in the liver.Immunohistochemical SABC methods were used to detect transplanted BMSCs and expression of urokinase plasminogen activator (uPA).RESULTS: Serum transaminase level and liver fibrosis in rats were markedly reduced by Guiyuanfang and BMSCs. HA level and procollagen Ⅲ level were also reduced obviously,compared to model rats (HA: 47.18±10.97 ng/mL,48.96±14.79 ng/mL; PCⅢ: 22.48±5.46 ng/mL, 26.90±3.35ng/mL; P＜0.05).Hydroxyproline contents of liver tissue in both BMSCs group and Guiyuanfang group were far lower than that of model group (1 227.2±43.1 μg/g liver tissue, 1390.8±156.3 μg/g liver tissue; P＜0.01). After treatment fibrosis scores were also reduced. Both Guiyuanfang and BMSCs could increase the expression of uPA. The transplanted BMSCs could engraft, survive, and proliferate in the liver.CONCLUSION: Guiyuanfang protects against liver fibrosis.Transplanted BMSCs may engraft, survive, and proliferate in the fibrosis livers indefinitely. Guiyuanfang may synergize with BMSCs to improve recovery from liver fibrosis.

Role of fibroblast growth factor receptor 1 in the bone development and skeletal diseases

Accumulating data suggest that FGFs/FGFR1 plays essential roles in the bone development and human skeletal diseases. Conditional inactivation of fgfrl caused different phenotypes displaying in different cells or specific organs and revealed some novel functions of FGFR1 in bone development. Fgfrl mutation mainly induced 2 types of human skeletal diseases, craniosynostosis syndrome and dysplasias. Similar mutation of fgfrl in mouse model just mimicked the phenotype that happened in human. These fa- cilitate the investigation on the underlying mechanism of the diseases. Here we mainly focused on the ad- vance of FGFR1 function in the bone development and its mutation caused skeletal diseases.

Electromagnetic Stimulation Combined with Aloe vera Increases Collagen Reorganization in Burn Repair

Objectives： Burns are shown as a clinical problem for their severity and multiple complications due to the time required to heal. Therapies that improve their healing are of great importance, especially for being minimally invasive, of low cost and best performance, all related to the speed and quality of healing. This study investigated the effects of the magnetic electro stimulator Haihuá CD9 isolated or in association with Aloe vera in rats skin burns. Methods： Experimental groups （n = 30/group） were： （C） Carbopol gel; （F） A. vera/Carbopol gel; （H） Haihuá＋Carbopol gel; （H＋F） Haihuá＋A. vera/Carbopol gel. Samples were collected on the 7th, 14th, and 21st experimental days for structural and morphometric analysis, hydroxyproline and glycosaminoglycans quantification, zymography for MMP-2 and MMP-9 and Western Blotting for TGF-β1, VEGF, Collagen I and III. Key findings： The expression of TGF-β1 in H＋F was increased on the 7th day and of MMP-9 on the 7th and 14th days. The expression of VEGF increased in the first experimental periods and decreased in the last for the treated groups. There was an increase in the fibroblasts and birefringent collagen fibers in groups treated with Haihuá isolated or in association with A. vera in all periods. The quantification of collagen I increased, while collagen III decreased in H＋F. The higher amount of GAGs and MMP-2 active isoform was detected in H and H＋F during all periods. Conclusions： Considering the results of the present study, electromagnetic stimulation in association with the A. vera extract promoted an increase in the number of fibroblasts, GAGs content, MMP-2 activity, the deposition and organization of collagen fibers, favoring the repair of injuries to second degree burns, and may also present therapeutic potential in this injury type.

Effect of polypropylene fiber and coarse aggregate on the ductility and fluidity of cemented tailings backfill

Adding polypropylene(PP)fibers and coarse aggregates has become a popular way to enhance the strength and stability of the cemented tailings backfilling(CTB)body.It is essential to explore the influence of tailings-aggregate ratio and fiber content on the mechanical properties of CTB samples.The comprehensive tests of the unconfined compressive strength(UCS),slump and microstructure were designed,and the regression models were established to characterize the effect of the strength,ductility and fluidity.The results indicate that the tailings-aggregate ratio of 5:5 and PP fiber content of 0.5 kg/m^(3) are the optimum point considering the UCS,cracking strain,peak strain and post-peak ductility.The tailings-aggregate ratio is consistent with the unary quadratic to the UCS and a linear model with a negative slope to the slump.Microstructural analysis indicates that PP fiber tends to bridge the cracks and rod-mill sand to serve as the skeleton of the paste matrix,which can enhance the compactness and improve the ductility of the CTB.The results presented here are of great significance to the understanding and application of coarse aggregates and fibers to improve the mechanical properties of CTB.

Therapy with bone marrow cells reduces liver alterations in mice chronically infected by Schistosoma mansoni

AIM： To investigate the potential of bone marrow mononuclear cells （BM-MCs） in the regeneration of hepatic lesions induced by Schistosoma mansoni （S.mansoni） chronic infection. METHODS： Female mice chronically infected with S.rnansoni were treated with BM-MCs obtained from male green fluorescent protein （GFP） transgenic mice by intravenous or intralobular injections. Control mice received injections of saline in similar conditions, Enzyme-linked immunosorbent assay （ELISA） assay for transforming growth factor-beta （TGF-β）, polymerase chain reaction （PCR） for GFP DNA, immunofluorescence and morphometric studies were performed. RESULTS： Transplanted GFP^＋ cells migrated to granuloma areas and reduced the percentage of liver fibrosis. The presence of donor-derived cells was confirmed by Fluorescence in situ hybridization （FISH） analysis for detection of cells bearing Y chromosome and by PCR analysis for detection of GFP DNA. The levels of TGF-β, a cytokine associated with fibrosis deposition, in liver fragments of mice submitted to therapy were reduced. The number of oval cells in liver sections of S.mansoni-infected mice increased 3-4 fold after transplantation. A partial recovery in albumin expression, which is decreased upon infection with S.mansoni, was found in livers of infected mice after cellular therapy. CONCLUSION： In conclusion, transplanted BMCs migrate to and reduce the damage of chronic fibrotic liver lesions caused by S.mansoni.

Progress in non-invasive detection of liver fibrosis

Liver fibrosis is an important pathological precondition for hepatocellular carcinoma.The degree of hepatic fibrosis is positively correlated with liver cancer.Liver fibrosis is a series of pathological and physiological process related to liver cell necrosis and degeneration after chronic liver injury,which finally leads to extracellular matrix and collagen deposition.The early detection and precise staging of fibrosis and cirrhosis are very important for early diagnosis and timely initiation of appropriate therapeutic regimens.The risk of severe liver fibrosis finally progressing to liver carcinoma is＆gt;50%.It is known that biopsy is the gold standard for the diagnosis and staging of liver fibrosis.However,this method has some limitations,such as the potential for pain,sampling variability,and low patient acceptance.Furthermore,the necessity of obtaining a tissue diagnosis of liver fibrosis still remains controversial.An increasing number of reliable non-invasive approaches are now available that are widely applied in clinical practice,mostly in cases of viral hepatitis,resulting in a significantly decreased need for liver biopsy.In fact,the noninvasive detection and evaluation of liver cirrhosis now has good accuracy due to current serum markers,ultrasound imaging,and magnetic resonance imaging quantification techniques.A prominent advantage of the non-invasive detection and assessment of liver fibrosis is that liver fibrosis can be monitored repeatedly and easily in the same patient.Serum biomarkers have the advantages of high applicability（〉95%）and good reproducibility.However,their results can be influenced by different patient conditions because none of these markers are liver-specific.The most promising techniques appear to be transient elastography and magnetic resonance elastography because they provide reliable results for the detection of fibrosis in the advanced stages,and future developments promise to increase the reliability and accuracy of the staging of hepatic fibrosis.This article aims to describe the recent progress in the development of non-invasive assessment methods for the staging of liver fibrosis,with a special emphasize on computer-aided quantitative and deep learning methods.

Endoscopic Management in Fibrous Dysplasia of Ethmoid Sinus:One Case Report and Literatures Review

Introduction Fibrous dysplasia （FD） is a sporadic real bone structures and marrow are skeletal disorder in which norreplaced by fibro-osseous tissuet. It is classified into three types： monostotic FD, polyostotic FD, and McCune-Albright syndrome. The monostotic is the most common form of FD. The disease primarily affects the long bones of the patients, and may also develop in the craniofacial region. However, FD in the paranasal sinuses is uncommon. The case in this report was diagnosed as FD in the ethmoid sinus which is a rarely seen case.

Portal hypertension secondary to myelofibrosis with myeloid metaplasia:A study of 13 cases

AIM:To describe the clinical presentation and complications of portal hypertension (PH) secondary to myelofibrosis with myeloid metaplasia (MMM).METHODS: Medical records for 123 patients with MMM were reviewed.RESULTS: Thirteen patients with PH secondary to MMM were identified. Median ages at time of MMM and PH diagnosis were 61 and 66 years, respectively. The interval from MMM diagnosis to presentation with one of the PH features ranged from 1 to 11 years. Variceal bleeding and ascites were the most common presentations. Of the eight patients who presented with variceal bleeding, six patients underwent endoscopic variceal ligation (EVL) with no variceal recurrence or hematological worsening during a 12-mo follow up period.CONCLUSION: Patients with MMM might develop PH. Exact mechanisms leading to PH in MMM are still controversial. As in other etiologies, variceal bleeding and ascites are the most common presentations. Anemia may correlate with, and/or predict, the severity of the PH presentation in these patients. EVL can successfully control variceal bleeding in MMM. Further clinical studies are required.

Microtubular elements of cortex in Euplotes Woodruffi

The cortical ciliature microtubular organelles of the ciliated protozoan Euplotes Woodruffi were analyzed with FLUTAX and anti-a-tubulin antibody. It showed that the cortical cytoskeleton was composed of non-ciliature structure, ciliature structure including adoral zone of membranelles （AZM）, undulating membranes （UM）, frontal-ventral-transverse cirri （FVTC）, caudal cirri （CC）, dorsal kineties, as well as base-associated microtubules. The silverline system is composed of longitudinal and transverse microtubules, concave structure appeared on the cell dorsal side, the base of DK containing rosette-like skeleton structure. All these suggested that the non-ciliature structure, ciliature structure of Euplotes be quite different from that of other species of ciliate, the silverline-system be true pare of cortical microtubute cytoskeleton in Euplotes, rosette structure be part of the basecytoskeleton.

Expression of Neuropilin-1 Gene in Bone Marrow Stromal Cells from Patients with Myeloid Leukemia and Normal Individuals

Objective: To investigate the expression of neuropilin-1 (NP-1) gene in bone marrow stromal cells (BMSCs) from myeloid leukemia (AML and CML) and normal individuals. Methods: Mononuclear cells were isolated from bone marrow (BM) of CML (14 cases), AML (12 cases) and normal individuals (20 cases). Adherent cells (i.e. BMSCs) were collected after long-term culture in vitro. The expression of NP-1 gene in three groups was detected respectively by reverse-transcription polymerase chain reaction (RT-PCR). Results: The long-term culture of BMSCs was successfully established. The expression level of NP-1 gene was significantly lower in BMSCs from AML (47.1%) and CML (50%) than in normal individuals (85%). Conclusion: NP-1 gene is expressed in BMSCs from some AML or CML patients and most normal individuals. The low-expression of NP-1 gene in BMSCs from AML or CML patients might be related with abnormality of regulation in hematopoiesis.

An exceptional case of myelodysplastic syndrome with myelofibrosis following combination chemotherapy for squamous cell lung cancer

A 60-year-old woman with squamous cell carcinoma in the right lung was successfully treated with four cycles of combination chemotherapy after surgery, and complete remission was achieved. However, the patient developed myelodysplastic syndrome (MDS) RAEB-2 with myelofibrosis after remission, possibly because of chemotherapy or DNA methylation. The patient responded well to dacitabine (Dacogen), suggesting that DNA hypomethylation agents can be a promising therapy to retard the progression of a second tumor or carcinoma.

EFFECTS OF ALVEOLAR MACROPHAGE CONDITIONED MEDIA FROM INTERSTITIAL LUNG DISEASE PATIENTS ON THE PROCOLLAGEN mRNA EXPRESSION IN HUMAN LUNG FIBROBLASTS

Progressive inflammation and fibrosis are the central processes in the pathogenesis of pulmonary fibrosis. It is believed that macrophages in areas of chronically inflamed lung play a key role in fibrotic response. Therefore, we investigated the effects of alveolar macrophage (AmΦ) conditioned media from interstitial lung disease (ILD) patients on lung fibroblast proliferation and procollagen mRNA expression. After stimulating with AmΦ conditioned media from ILD patients, the fibroblast proliferation increased 71. 4 % compared with the control, but for media from bronchial carcinoma (BC) patients, it just increased 14. 3%. There is a significant difference between the two groups (P<0. 05). The procollagen a1 (Ⅰ) mRNA in fibroblasts stimulated with AmΦ conditioned media from ILD patients was increased 21. 3 %, and a1 ( Ⅲ) was 37. 2% higher than control (P<0.05). It increased 6. 8% and 12. 8% for media from BC patients respectively, but there was no difference when compared to the control. We considered that AmΦ from ILD patients might be in an activated state and could release some growth factors to stimulate fibroblast proliferation and promote collagen DNA expression.

Intravenous injection of mesenchymal stem cells is effective in treating liver fibrosis

AIM: To compare the influence of different transplant sites in bone marrow mesenchymal stem cell (MSC)-based therapy for liver fibrosis. METHODS: MSCs isolated from Sprague Dawley (SD) rats were induced into hepatocyte-like cells. Liver fibrosis in SD rats was induced with carbon tetrachloride. Following hepatocyte induction in vitro, 4',6-diamidino- 2-phenylindole (DAPI)-labeled MSCs were transplanted by intravenous, intrahepatic, and intraperitoneal injection. Histopathological staining, immunohistochemistry, and biochemical analysis were used to compare the morphological and functional liver regeneration among different MSC injection modalities. The expression differences of interleukins, growth factor, extracellular matrix, matrix metalloproteinases, and tissue inhibitor of metalloproteinase were examined by real-time reverse transcription-polymerase chain reaction (RT-PCR) andenzyme linked immunosorbent assay (ELISA). RESULTS: Four days after exposure to hepatocyte differentiation medium, MSCs that did not express hepatocyte markers could express α-fetoprotein, albumin, and cytokeratin 18. The results of histopathological staining, immunohistochemistry, and biochemical analysis indicated that intravenous injection is more effective at rescuing liver failure than other injection modalities. DAPI-labeled cells were found around liver lobules in all three injection site groups, but the intravenous group had the highest number of cells. PCR and ELISA analysis indicated that interleukin-10 (IL-10) was highest in the intravenous group, whereas il1β, il6, tnfα and tgfβ, which can be regulated by IL10 and are promoters of liver fibrosis, were significantly lower than in the other groups. CONCLUSION: MSC administration is able to protect against liver fibrosis. Intravenous injection is the most favorable treatment modality through promotion of IL10 expression.

Expression of BMP-7 and its receptors in renal tubolo-interstitial fibrosis induced with unilateral ureteral obstruction in rats

Objective:To study the changes of the expression of bone morphogenetic protein-7(BMP-7) and its receptors(BMPR-Ⅱ,ALK2,ALK3 and ALK6) in the renal tubulointerstitial fibrosis induced with unilateral ureteral obstruction in rats. Methods: Sixty Wistar male rats were divided randomly into the normal control,sham-operation and unilateral ureteral obstruction(UUO) groups and the rats were killed on the 1st,3rd,7th and 14th postoperative days respectively.The mRNA level of BMP-7,BMPR-Ⅱ,ALK2,ALK3 and ALK6 was determined with RT-PCR.The site and level of protein expression of BMP-7 were observed with immunohistochemical staining. Results: The mRNA level of BMP-7,BMPR-Ⅱ,ALK2 and ALK3 was significantly decreased in the rats of UUO group than in those of the sham-operation group but the mRNA level of ALK6 showed no obvious changes in all the rats.Immunohistochemical staining revealed that the protein of BMP-7 was mainly expressed in the renal tubules and interstitial tissue of the kidneys in normal rats but it was decreased gradually along with the unilateral ureteral obstruction. Conclusion: It is found that the loss of BMP-7 and its receptors including BMPR-Ⅱ,ALK2 and ALK3 occurs in the early phase of renal tubulointerstitial fibrosis before the appearance of other pathological changes in the kidney and may play an important role in the occurrence and progress of renal tubulointerstitial fibrosis.

Effects of Electromagnetic Field and Basic Fibroblast Growth Factor on Osteoblast's Growth

Osteoblasts of rat cultured in vitro were stimulated with pulsed 50 Hz electromagnetic field and basic fibroblast growth factor(bFGF).The MTT method,flow cytometry and histochemistry staining were used to detect cell proliferation?cell cycle and alkaline phosphatase .The results indicated: after stimulated by 1 mT electromagnetic field,the cells are more abundant,have more S phase percentages,2 mT electromagnetic field have no evident effect on cells’growth;compared with electromagnetic field,the cells stimulated by bFGF are more abundant and have larger S phase ratios.Electromagnetic field and bFGF have no effect on cells,alkaline phosphatase.Therefore ,we concluded that electromagnetic field can enhance osteoblasts growth like some growth factor such as basic fibroblast growth factor,and the osteoblasts’,characteristics was not changed.

Wnt5a participates in hepatic stellate cell activation observed by gene expression profile and functional assays

AIM:To identify differentially expressed genes in quiescent and activated hepatic stellate cells(HSCs)and explore their functions.METHODS:HSCs were isolated from the normal Sprague Dawley rats by in suit perfusion of collagenase and pronase and density Nycodenz gradient centrifugation.Total RNA and mRNA of quiescent HSCs,and cultureactivated HSCs were extracted,quantified and reversely transcripted into cDNA.The global gene expression profile was analyzed by microarray with Affymetrix rat genechip.Differentially expressed genes were annotated with Gene Ontology(GO)and analyzed with Kyoto encyclopedia of genes and genomes(KEGG)pathway using the Database for Annotation,Visualization and Integrated Discovery.Microarray data were validated by quantitative real-time polymerase chain reaction(qRTPCR).The function of Wnt5a on human HSCs line LX-2 was assessed with lentivirus-mediated Wnt5a RNAi.The expression of Wnt5a in fibrotic liver of a carbon tetrachloride(CCl4)-induced fibrosis rat model was also analyzed with Western blotting.RESULTS:Of the 28 700 genes represented on this chip,2566 genes displayed at least a 2-fold increase or decrease in expression at a P<0.01 level with a false discovery rate.Of these,1396 genes were upregulated,while 1170 genes were downregulated in culture-activated HSCs.These differentially expressed transcripts were grouped into 545 GO based on biological process GO terms.The most enriched GO terms included response to wounding,wound healing,regulation of cell growth,vasculature development and actin cytoskeleton organization.KEGG pathway analysis revealed that Wnt5a signaling pathway participated in the activation of HSCs.Wnt5a was significantly increased in cultureactivated HSCs as compared with quiescent HSCs.qRTPCR validated the microarray data.Lentivirus-mediated suppression of Wnt5a expression in activated LX-2 resulted in significantly impaired proliferation,downregulated expressions of typeⅠcollagen and transforming growth factor-β1.Wnt5a was upregulated in the fibrotic liver of a CCl4-induced fibrosis rat model.CONCLUSION:Wnt5a is involved in the activation of HSCs,and it may serve as a novel therapeutic target in the treatment of liver fibrosis.