OBJECTIVETo study the clinicopathological features, immnophenotype and differential diagnosis of low-grade fibromyxoid sarcoma (LGFMS).

METHODSThe clinical, radiological and pathological features of 9 cases of LGFMS were reviewed.

RESULTSThe patients consisted of six males and three females with ages ranging from 11 to 65 years (mean 31.4 years). Clinically, most cases presented as slowly growing painless masses located in the extremities, trunk and neck. Two cases had a history of a rapid recent enlargement. Three cases presented with recurrent diseases after incomplete resection. Ultrasound showed heterogeneous signal. Precontrast CT and T1-weighted MRI showed a nodular mass of low to isodensity, while contrast-enhanced CT and T2-weighted MRI demonstrated contrasting zonal areas of hypo/hyperintensity. The tumors measured 3 to 16 cm (mean 7.7 cm), with a fibrous to myxoid appearance on cut surface. Histologically, they were composed of alternating collagenous and myxoid areas. A transition between the two areas could be identified. Giant rosette-like structures were observed in 3 cases. The center of the giant rosettes was composed of eosinophilic collagen, which showed centrifugal in arrangement. There was also gradual transition between the giant rosettes and the fibromyxoid component. On high magnification, the tumor was composed of ovoid to spindle-shaped cells with hyperchromatic nuclei. Nuclear atypia was inconspicuous. The tumor cells were arranged mostly in interlacing fascicles or whorls. Vessels were not prominent and necrosis was absent. Immunohistochemically, the tumor cells showed unique staining for vimentin, consistent with a fibroblastic differentiation.

CONCLUSIONSLGFMS is a distinctive low grade fibroblastic sarcoma of young adults. Recognizing the characteristics of the rare entity may help to avoid misdiagnosis. Wide local excision is recommended to avoid local recurrences.

Adolescent ; Adult ; Aged ; Child ; Diagnosis, Differential ; Extremities ; pathology ; Female ; Fibrosarcoma ; diagnosis ; metabolism ; pathology ; surgery ; Head and Neck Neoplasms ; diagnosis ; metabolism ; pathology ; surgery ; Humans ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Myxosarcoma ; pathology ; Soft Tissue Neoplasms ; diagnosis ; metabolism ; pathology ; surgery ; Vimentin ; metabolism ; Young Adult

Five new megastigmanes (1-5) were isolated from a decoction of Uncaria rhynchophylla by separation techniques of column chromatography using a combination of multiple stationary phases, including macroporous adsorbent resin, MCI resin, silica gel, Sephadex LH-20, and Toyopearl HW-40F, and reversed phase HPLC. Their structures were characterized by spectroscopic data analysis of HR-ESI-MS, NMR, and CD, in combination with Mosher's mothed as well as ECD and NMR calculations. The new compounds were named uncarphyllonone A (1), uncarphyllonols A (2) and B (3), and uncarphabscisic acids A (4) and B (5). Although the structures of 3 and 4 were previously reported, the reported NMR spectroscopic data were incorrect or do not support the assigned structures in literatures. This is also the first report of discovery of new megastigmane natural products from the Uncaria genus.

Adult ; Factor VII Deficiency ; complications ; Female ; Humans ; Liver Cirrhosis, Biliary ; complications

Objective To evaluate the efficacy and safety of multi-target treatment of multi glycosides of tripterygium wilfordii combined small dose of tacrolimus on idiopathic membranous nephropathy.Methods A total of 40 cases with idiopathic membranous nephropathy were selected in our hospital and were randomly divided into two groups, 20 cases in control and treatment group, respectively.Patients in treatment group were given tacrolimus 0.05 mg· kg-1 · d-1 for the first time, maintaining the blood drug concentration at 2 to 5 ng · mL-1 , at the same time, tripterygium wilfordii 60 mg· d -1 were additionally given.After 6 months, the dose of tripterygium wilfordii was gradually reduced or stopped.While patients in control group were only administrated with tacrolimus with the initial dose of 0.1 mg· kg -1 · d-1 , maintaining the blood drug concentration at 5 to 10 ng· mL-1.After 6 months, the using of tacrolimus began to re-duce to maintain the concentration at 2 to 5 ng· mL-1.The data of cura-tive effect, adverse reaction and the concentration of tacrolimus in two groups were observed in 12 months.Results Compared with before treatment, the data of 24 h urinary protein, serum albumin and triglyceride were significantly improved in two groups ( P<0.05).After 12 -month of treatment, the data of 24 h urinary protein and serum albumin were statistically different between two groups ( P<0.05).After 6 months follow-up, the complete remission rate was 55% in treat-ment group, significantly higher than 30%in control group ( P<0.05).After 12 months follow-up, the recurrence rate in treatment group (17%) was lower than that in control group (41%, P<0.05).The incidence rate of adverse reactions between the two groups had no significant difference( P>0.05).Conclusion The multi-target treatment of small dose of tacrolimus combined tripterygium wilfordii for idiopathic membranous nephropathy has been confirmed with a faster onset, higher clinical remission rate, lower relapse rate, and patients are well tolerated.

OBJECTIVETo study the effects of nucleotides on apoptosis of thymocytes in mice.

METHODSApoptosis model in vivo was first established and 25 KM mice, 4 weeks old, were randomly divided into 5 groups. One group was control, and the others were test groups. Mice in test groups were injected with DEX (25 mg/kg) and the controls were treated with normal saline. 4, 8, 16, and 24 hours later the thymus and spleen were weighed and lymphocytes in thymus were separated. The apoptosis of lymphocytes was analyzed by using DNA electrophoresis and flow cytometry. 16 hours later lymphocytes apoptosis reached a peak and lasted 24 hours. Methods used to establish apoptosis model in vivo were: mice (4 weeks old) were injected with DEX (25 mg/kg), and thymus lymphocytes were separated 16 hours later and analyzed. The effects of nucleotides on apoptosis of mice thymocytes were investigated in experiment 2. Sixty KM mice, 20 g +/- 2g, 4 weeks old, were divided into four treatments: negative control group (NC), positive control group (PC), nucleotides-additive group 1 (NTS1) and nucleotides-additive group 2 (NTS2).

RESULTSBody weight gained in NST1 and NST2 were 3.71 g, 4.01 g respectively, significantly higher than NC (2.74 g) (P < 0.01) and in NST2 was significantly higher than in PC (2.96 g) (P < 0.01). Thymus index and spleen index were decreased significantly (P < 0.01), and no difference was found with the supplementation of nucleotides (P > 0.05). [Ca2+]i increased to 167.37 nmol/L, 191.16 nmol/L, 180.78 nmol/L in PC, NST1 and NST2 with DEX, being significantly higher than in NC (103.76 nmol/L) (P < 0.01). The percent of apoptosised thymocytes in groups were 0.31%, 11.93%, 9.82%, 11.15%, respectively. Thymus index and spleen index, cell apoptosis and [Ca2+]i were not differed significantly among PC, NTS1 and NTS2 groups.

CONCLUSIONNucleotides should have no significant effects on apoptosis of thymocytes in mice in vivo.

Animals ; Apoptosis ; drug effects ; Dexamethasone ; pharmacology ; Lymphocytes ; cytology ; Male ; Mice ; Nucleotides ; pharmacology ; Random Allocation ; Thymus Gland ; cytology

This study was aimed to investigate the correlation between HLA gene distribution and allele frequency of the patients with leukemia. PCR-SSP technique was used to detect the HLA genotype of 2994 umbilical cord blood units from healthy newborns (as control), the detecting result of which was compared with HLA genotypes of 1246 patients with leukemia searched in our cord blood bank. The differences between two groups were compared and analyzed. The results indicated that as compared with the control group, the allele frequencies of HLA-B*56 (0.56%), B*70 (0.24%) obviously increased (RR = 2.2546, 6.2598, χ(2) = 5, 5.98, P < 0.05), while the allele frequencies of HLA-A*03 (3.45%), A*30 (4.86%), B*13 (8.75%), B44* (3.25%), B61* (5.70%), DRB1*07 (8.23%), DRB1*15 (14.21%) obviously decreased in patients with leukemia (RR = 0.5889, 0.7187, 0.7359, 0.5713, 0.7127, 0.6242, 0.7976, χ(2) = 19.23, 9.82, 14.33, 20.48, 11.99, 33.21, 11.56, P < 0.01). It is concluded that HLA-B*56, B*70 alleles seem to be characterized by the genetic susceptibility to leukemia and may be served as risk markers for leukemia occurrence, while the HLA-A*03, A*30, B*13, B*44, B*61, DRB1*07, DRB1*15 can be considered as genetic indicators for resistance of leukemia.
Adolescent ; Adult ; Alleles ; Case-Control Studies ; Child ; Child, Preschool ; Female ; Fetal Blood ; Gene Frequency ; Genotype ; HLA-A Antigens ; genetics ; HLA-B Antigens ; genetics ; HLA-DRB1 Chains ; genetics ; Humans ; Infant, Newborn ; Leukemia ; genetics ; Middle Aged ; Young Adult

：【Objective】Toinvestigatetheeffectsofinterleukin-17（IL-17）ontheproliferationandmigrationof bronchialsmoothmusclecells（BSMC）andtheroleofJAK/STAT3signalingpathwayinthisprocess.【Methods】BSMC weretreatedwithdifferentconcentrationsofIL-17fordifferenttimestodeterminethebestoftheexperimentalcondition. ThenMTTassaywasusedtodetectcellviability.CellproliferationstatesweredetectedbyBrdUstaining，andthecell cyclewasassessedbyPIstainingusingaflowcytometer.Transwellcellmigrationassaywasfurtherusedtodetectcell migrationability.TheexpressionofJAK，p-JAK，STAT3andp-STAT3inBSMCafterbeingtreatedwithIL-17was detectedbyWesternblotting.JAK/STAT3signalingpathwayspecificblockerAG490wasusedtoinvestigatetheroleof JAK/STAT3signalingpathwayinIL-17-inducedBSMCproliferationandmigration.TheeffectsofIL-17oncellproliferation， migration and JAK/STAT3 signaling pathway related protein expression were evaluated after blocking the JAK/STAT3 signaling.【Results】IL-17enhancedtheproliferation（P<0.05），promotedthecellcycletransitions（P<0.05）andsig⁃nificantlyincreasesthemigrationability（P<0.05）inBSMC.ThisprocesswasaccompaniedbytheenhancementofJAK/ STAT3signalingpathwayinBSMC（P<0.05）.InhibitionofJAK/STAT3signalingpathwayalleviatedBSMCproliferation andmigrationinducedbyIL-17（P<0.05） .【Conclusions】JAK/STAT3signalingpathwayparticipatesinthestimulation processofIL-17ontheproliferationandmigrationofBSMC.AG490inhibitstheenhancementofJAK/STAT3signaling pathwayinBSMCinducedbyinterleukin-17.

OBJECTIVETo study the clinicopathologic features, diagnosis and differential diagnosis of systemic Epstein-Barr virus (EBV)-positive T-cell lymphoproliferative disease of childhood (CSEBV(+)T-LPD).

METHODSThirty cases of CSEBV(+)T-LPD were retrospectively studied by light microscopy, immunohistochemistry and in-situ hybridization for EBV-encoded RNA (EBER). The clinical information and follow-up data were analyzed.

RESULTSNineteen of the 30 patients were males and 11 females. The median age of disease onset was 9 years (range = 1.5 to 32 years). The average duration between disease onset and diagnosis was 14 months. The major clinical manifestations were fever (96.7%), lymphadenopathy (83.3%) and hepatosplenomegaly (66.7%). Cutaneous manifestations were not uncommon, which included hypersensitivity to mosquito bite (13.3%) and skin rash (20.0%). Six of the 20 patients died on follow up. Histologically, the lymph nodes showed expansion of T zone, with diminished or effaced lymphoid follicles. The lymphoid cells were of small to medium size. Scattered large lymphoid cells were also identified in the expanded T zone. Furthermore, the liver and spleen showed mild to marked sinusoidal infiltration. In some cases, various degrees of sinus histiocytosis with erythrophagocytosis were present. Skin biopsies showed mild to marked degree of lymphocytes infiltration in dermis. Immunohistochemical study and in-situ hybridization showed that the EBER-positive cells were of T lineage and CD3 positive. They also expressed cytotoxic molecules granzyme B and TIA-1. Seven of the 8 cases examined were CD8 positive, while the remaining case was mainly CD4 positive. Thirteen of 15 cases were shown to be CD56 negative. The number of EBER-positive cells ranged from 5 to more than 500 per high-power field. These cells included small to large lymphoid cells located mostly in the expanded T zone and sometimes in the germinal centers. Nine of the 30 cases, which consisted mainly of medium to large-sized lymphoid cells, were also EBER positive.

CONCLUSIONSSystemic EBV-positive T-cell lymphoproliferative disease of childhood occurs most often in children and young adults, with a median age of 9 years. It has a subacute or chronic clinical course. Most of the patients have evidence of systemic disease, often with lymph node, liver, spleen and skin involvement. It carries a poor clinical outcome and can be life-threatening. The disease is characterized by a clonal proliferation of EBV-infected T cells with cytotoxic immunophenotype. Definitive diagnosis requires correlation between clinical, pathologic and ancillary investigation findings.

Adolescent ; Adult ; CD3 Complex ; metabolism ; CD8 Antigens ; metabolism ; Child ; Child, Preschool ; Epstein-Barr Virus Infections ; genetics ; metabolism ; pathology ; virology ; Female ; Follow-Up Studies ; Gene Rearrangement, T-Lymphocyte ; Granzymes ; metabolism ; Herpesvirus 4, Human ; isolation & purification ; Humans ; Infant ; Lymph Nodes ; metabolism ; pathology ; Lymphoproliferative Disorders ; genetics ; metabolism ; pathology ; virology ; Male ; Poly(A)-Binding Proteins ; metabolism ; Prognosis ; RNA, Viral ; metabolism ; Retrospective Studies ; T-Cell Intracellular Antigen-1 ; T-Lymphocytes ; metabolism ; pathology ; virology ; Young Adult

OBJECTIVETo observe the changes of pulmonary surfactant (PS) in rats with acute lung injury(ALI) induced by lipopolysaccharide (LPS) and to explore the effects of hydrogen sulfide (H2S) on PS.

METHODSFourty- eight male rats were randomly divided into six groups (n = 8). They were control group, LPS group, LPS+ NaHS low, middle, high dose groups and LPS+ PPG group. Saline was administrated in Control group. LPS was administrated in LPS group. In LPS + NaHS low, middle, high dose groups or LPS + PPG group, sodium hydrosulfide (NaHS) of different doses or DL-propargylglycine (PPG) were respectively administrated when the rats were administrated of LPS after 3 hours. All the rats were killed at 6 hours after administration of Saline or LPS. The morphological changes of alveolar epithelial type II cells (AEC-II) were respectively observed by transmission electron microscopes. The content of H2S in plasma and activity of cystathionine-gamma-lyase (CSE) in lung tissues were respectively detected. The contents of total protein (TP) and total phospholipids (TPL) in bronchoalveolar lavage fluid (BLAF) were respectively measured. The pulmonary surfactant protein A (SP-A), surfactant protein B (SP-B) and surfactant protein-C (SP-C) mRNA expressions in lung tissues were analysed.

RESULTS(1) Compared with control group, the content of H2S in plasma, activity of CSE, content of TPL, and SP-A, SP-B and SP-C mRNA expressions were respectively decreased in LPS group (P < 0.05 or P < 0.01). But the content of TP was increased in LPS group (P < 0.01); (2) Compared with LPS group, the content of H2S, activity of CSE and SP-A mRNA expression were significantly increased in LPS + NaHS low, middle and high dose groups (P < 0.05). The SP-B mRNA expression and content of TPL were significantly increased in LPS + NaHS Middle and High dose groups (P < 0.05). The content of TP was decreased in LPS + NaHS High dose group (P < 0.05). The SP-C mRNA expression was not altered in LPS+ NaHS low, middle and high dose groups (P > 0.05); (3) Compared with LPS group, the content of H2S, activity of CSE, content of TPL, and SP-A, SP-B and SP-C mRNA expressions were respectively decreased, but content of TP was increased in LPS + PPG group (P < 0.05).

CONCUSIONThe decrease of PS is the important physiopathologic process of ALI induced by LPS. Exogenously applied H2S could attenuate the process of ALI that possibly because H2S could adjust the compose and secretion of PS.

Acute Lung Injury ; chemically induced ; metabolism ; Animals ; Hydrogen Sulfide ; metabolism ; pharmacology ; Lipopolysaccharides ; Male ; Pulmonary Surfactants ; metabolism ; Rats ; Rats, Sprague-Dawley

BACKGROUNDSpinocerebellar ataxias (SCAs) are a group of neurodegenerative disorders that primarily cause the degeneration in the cerebellum, spinal cord, and brainstem. We study the clinical characteristics, radiological features and gene mutation in Chinese families with SCAs.

METHODSIn this study, we investigated 10 SCAs Chinese families with SCA1, SCA3/Machado-Joseph disease (MJD), SCA7, SCA8. There were 27 people who were genetically diagnosed as SCA, of which 21 people showed clinical symptoms, and 6 people had no clinical phenotype that we called them presymptomatic patients. In addition, 3 people with cerebellar ataxia and cataracts were diagnosed according to the Harding diagnostic criteria but failed to be recognized as SCAs on genetic testing. Clinical characteristic analyses of each type of SCAs and radiological examinations were performed.

RESULTSWe found that SCA3/MJD was the most common subtype in Han population in China, and the ratio of the pontine tegmentum and the posterior fossa area was negatively correlated with the number of cytosine-adenine-guanine (CAG) repeats; the disease duration was positively correlated with the International Cooperative Ataxia Rating Scale score; and the CAG repeats number of abnormal alleles was negatively correlated with the age of onset.

CONCLUSIONSCollectively our study is a systematic research on SCAs in China, which may help for the clinical diagnosis and prenatal screening of this disease, and it may also aid toward better understanding of this disease.

Adult ; DNA Repeat Expansion ; genetics ; Female ; Humans ; Machado-Joseph Disease ; genetics ; pathology ; Male ; Mutation ; genetics ; Spinocerebellar Ataxias ; genetics ; pathology ; Trinucleotide Repeat Expansion ; genetics