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Sample records for core usw g-4

  1. Petrochemical variation of Topopah Spring tuff matrix with depth (stratigraphic level), drill hole USW G-4, Yucca Mountain, Nevada

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    Byers, F.M. Jr.

    1985-12-01

    This study describes and interprets petrochemical variation of the matrix (excluding fractures and large gas cavities) of the Topopah Spring Member of the Paintbrush Tuff. This tuff includes the candidate host rock for a high-level nuclear waste repository at Yucca Mountain on the Nevada Test Site. Cored hole USW G-4, near the site of a potential exploratory shaft at Yucca Mountain, penetrated 359.4 m (1179 ft) of the member within the unsaturated zone. This study shows that petrographic textures and chemistry of the matrix vary systematically within recognizable lithologic subunits related to crystallization (cooling) zones, welding (compaction) zones, and compositional zones (rhyolite versus quartz latite). The methods used for this study include petrographic modal thin section analysis using an automated counter and electron microprobe analysis of the groundmass. Distinctive textural categories are defined, and they can be ranked from finest to coarsest as vitrophyre (glass), cryptocrystalline groundmass, spherulites, granophyre, lithic fragments, and phenocrysts. The two main groundmass compositions are also defined: rhyolite high silica) and quartz latite. The value of these petrochemical studies lies in providing microscopic criteria for recognizing the zonal subunits where they may have greatly limited exposure, as in mined drifts and in core from horizontal drill holes. For example, the lower nonlithophysal zone can be distinguished microscopically from the middle nonlithophysal zone by (1) degree of compaction, (2) amount of quartz, and (3) amount of lithic fragments. The variability between these textural categories should also be considered in designing physical and chemical tests of the Topopah Spring.

  2. Preliminary stratigraphic and petrologic characterization of core samples from USW-G1, Yucca Mountain, Nevada

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    Waters, A.C.; Carroll, P.R. (eds.)

    1981-11-01

    Tuffs of the Nevada Test Site are currently under investigation to determine their potential for long-term storage of radioactive waste. As part of this program, hole USW-G1 was drilled to a depth of 6000 ft below the surface, in the central part of the Yucca Mountain area, Nevada Test Site, Nevada. Petrographic study of the USW-G1 core is presented in this report and shows the tuffs (which generally were variably welded ash flows) are partly recrystallized to a variety of secondary minerals. The important alteration products are zeolites (heulandite, clinoptilolite, mordenite and analcime), smectite clays with minor interstratified illite, albite, micas, potassium feldspar, and various forms of silica. Iijima`s zeolite zones I through IV of burial metamorphism can be recognized in the core. Zeolites are first observed at about the 1300-ft depth, and the high-temperature boundary of zeolite stability in this core occurs at about 4350 ft. Analcime persists, either metastably or as a retrograde mineral, deeper in the core. The oxidation state of Fe-Ti oxide minerals, through most of the core, increases as the degree of welding decreases, but towards the bottom of the hole, reducing conditions generally prevail. Four stratigraphic units transected by the core may be potentially favorable sites for a waste repository. These four units, in order of increasing depth in the core, are (1) the lower cooling unit of the Topopah Spring Member, (2) cooling unit II of the Bullfrog Member, (3) the upper part of the Tram tuff, and (4) the Lithic-rich tuff.

  3. G4 Aptamers: Trends in Structural Design.

    Science.gov (United States)

    Varizhuk, Anna; Ilyinsky, Nikolay; Smirnov, Igor; Pozmogova, Galina

    2016-01-01

    Many potent DNA aptamers are known to contain a G-quadruplex (G4) core. Structures and applications of the majority of such aptamers have been reviewed previously. The present review focuses on the design and optimization of G4 aptamers. General features of bioactive G4s are analyzed, and the main strategies for construction of aptamers with desired properties and topologies, including modular assembly, control of an aptamer folding and some others, are outlined. Chemical modification as a method for post-SELEX G4 aptamer optimization is also discussed, and the effects of loop and core modifications are compared. Particular attention is paid to the emerging trends, such as the development of genomic G4- inspired aptamers and the combinatorial approaches which aim to find a balance between rational design and selection.

  4. Geology of the USW SD-12 drill hole Yucca Mountain, Nevada

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    Rautman, C.A. [Sandia National Labs., Albuquerque, NM (United States); Engstrom, D.A. [Spectra Research Institute, Albuquerque, NM (United States)

    1996-11-01

    Drill hole USW SD-12 is one of several holes drilled under Site Characterization Plan Study 8.3.1.4.3.1, also known as the {open_quotes}Systematic Drilling Program,{close_quotes} as part of the U.S. Department of Energy characterization program at Yucca Mountain, Nevada, which has been proposed as the potential location of a repository for high-level nuclear waste. The SD-12 drill hole is located in the central part of the potential repository area, immediately to the west of the Main Test Level drift of the Exploratory Studies Facility and slightly south of midway between the North Ramp and planned South Ramp declines. Drill hole USW SD-12 is 2166.3 ft (660.26 m) deep, and the core recovered essentially complete sections of ash-flow tuffs belonging to the lower half of the Tiva Canyon Tuff, the Pah Canyon Tuff, and the Topopah Spring Tuff, all of which are part of the Miocene Paintbrush Group. A virtually complete section of the Calico Hills Formation was also recovered, as was core from the entire Prow Pass Tuff formation of the Crater Flat Group.

  5. Geology of the USW SD-7 drill hole Yucca Mountain, Nevada

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    Rautman, C.A. [Sandia National Laboratories, Albuquerque, NM (United States); Engstrom, D.A. [Spectra Research Inst., Albuquerque, NM (United States)

    1996-09-01

    The USW SD-7 drill hole is one of several holes drilled under Site Characterization Plan Study 8.3.1.4.3.1, also known as the Systematic Drilling Program, as part of the U.S. Department of Energy characterization program at Yucca Mountain, Nevada. The Yucca Mountain site has been proposed as the potential location of a repository for high-level nuclear waste. The SD-7 drill hole is located near the southern end of the potential repository area and immediately to the west of the Main Test Level drift of the Exploratory Studies Facility. The hole is not far from the junction of the Main Test Level drift and the proposed South Ramp decline. Drill hole USW SD-7 is 2675.1 ft (815.3 m) deep, and the core recovered nearly complete sections of ash-flow tuffs belonging to the lower half of the Tiva Canyon Tuff, the Pah Canyon Tuff, and the Topopah Spring Tuff, all of which are part of the Miocene Paintbrush Group. Core was recovered from much of the underlying Calico Hills Formation, and core was virtually continuous in the Prow Pass Tuff and the Bullfrog Tuff. The SD-7 drill hole penetrated the top several tens of feet into the Tram Tuff, which underlies the Prow Pass and Bullfrog Tuffs. These latter three units are all formations of the Crater Flat Group, The drill hole was collared in welded materials assigned to the crystal-poor middle nonlithophysal zone of the Tiva Canyon Tuff; approximately 280 ft (85 m) of this ash-flow sheet was penetrated by the hole. The Yucca Mountain Tuff appears to be missing from the section at the USW SD-7 location, and the Pah Canyon Tuff is only 14.5 ft thick. The Pah Canyon Tuff was not recovered in core because of drilling difficulties, suggesting that the unit is entirely nonwelded. The presence of this unit is inferred through interpretation of down-hole geophysical logs.

  6. Geology of the USW SD-7 drill hole Yucca Mountain, Nevada

    Energy Technology Data Exchange (ETDEWEB)

    Rautman, C.A. [Sandia National Laboratories, Albuquerque, NM (United States); Engstrom, D.A. [Spectra Research Inst., Albuquerque, NM (United States)

    1996-09-01

    The USW SD-7 drill hole is one of several holes drilled under Site Characterization Plan Study 8.3.1.4.3.1, also known as the Systematic Drilling Program, as part of the U.S. Department of Energy characterization program at Yucca Mountain, Nevada. The Yucca Mountain site has been proposed as the potential location of a repository for high-level nuclear waste. The SD-7 drill hole is located near the southern end of the potential repository area and immediately to the west of the Main Test Level drift of the Exploratory Studies Facility. The hole is not far from the junction of the Main Test Level drift and the proposed South Ramp decline. Drill hole USW SD-7 is 2675.1 ft (815.3 m) deep, and the core recovered nearly complete sections of ash-flow tuffs belonging to the lower half of the Tiva Canyon Tuff, the Pah Canyon Tuff, and the Topopah Spring Tuff, all of which are part of the Miocene Paintbrush Group. Core was recovered from much of the underlying Calico Hills Formation, and core was virtually continuous in the Prow Pass Tuff and the Bullfrog Tuff. The SD-7 drill hole penetrated the top several tens of feet into the Tram Tuff, which underlies the Prow Pass and Bullfrog Tuffs. These latter three units are all formations of the Crater Flat Group, The drill hole was collared in welded materials assigned to the crystal-poor middle nonlithophysal zone of the Tiva Canyon Tuff; approximately 280 ft (85 m) of this ash-flow sheet was penetrated by the hole. The Yucca Mountain Tuff appears to be missing from the section at the USW SD-7 location, and the Pah Canyon Tuff is only 14.5 ft thick. The Pah Canyon Tuff was not recovered in core because of drilling difficulties, suggesting that the unit is entirely nonwelded. The presence of this unit is inferred through interpretation of down-hole geophysical logs.

  7. IgG4 related sclerosing mastitis: expanding the morphological spectrum of IgG4 related diseases.

    Science.gov (United States)

    Chougule, Abhijit; Bal, Amanjit; Das, Ashim; Singh, Gurpreet

    2015-01-01

    IgG4 related disease (IgG4RD) is a recently recognised condition characterised by mass forming lesions associated with storiform fibrosis, obliterative phlebitis, lymphoplasmacytic infiltrate rich in IgG4 positive plasma cells and elevated serum IgG4 levels. Although rare, mammary involvement has been reported as IgG4 related sclerosing mastitis, the morphological counterpart of a growing family of IgG4 related diseases. A total of 17 cases belonging to mass forming benign inflammatory breast lesions such as plasma cell mastitis, granulomatous lobular mastitis, non-specific mastitis and inflammatory pseudotumour were investigated as a possible member of IgG4 related sclerosing mastitis. Clinical, radiological, histopathological and immunohistochemistry findings were noted in all cases. Cases diagnosed as inflammatory pseudotumour showed all the histopathological features of IgG4RD along with increased number of IgG4 positive plasma cells and IgG4/IgG ratio >40%. However, only a few IgG4 positive cells were seen in plasma cell mastitis, granulomatous lobular mastitis and non-specific mastitis cases. These cases also did not fulfill the morphological criteria for the diagnosis of IgG4 related diseases. IgG4RD should be excluded in plasma cell rich lesions diagnosed on core biopsies by IgG4 immunostaining. This can avoid unnecessary surgery as IgG4 related diseases respond to simple and effective steroid treatment.

  8. Mineralogic variation in drill holes USW NRG-6, NRG-7/7a, SD-7, SD-9, SD-12, and UZ{number_sign}14: New data from 1996--1997 analyses

    Energy Technology Data Exchange (ETDEWEB)

    Chipera, S.J.; Vaniman, D.T.; Bish, D.L.; Carey, J.W.

    1997-05-30

    New quantitative X-ray diffraction (QXRD) mineralogic data have been obtained for samples from drill holes NRG-6, NRG-7/7A, SD-7, SD-9, SD- 12, and UZ{number_sign}14. In addition, new QXRD analyses were obtained on samples located in a strategic portion of drill hole USW H-3. These data improve our understanding of the mineral stratigraphy at Yucca Mountain, and they further constrain the 3-D Mineralogic Model of Yucca Mountain. Some of the unexpected findings include the occurrence of the zeolite chabazite in the vitric zone of USW SD-7, broad overlap of vitric and zeolitic horizons (over vertical ranges up to 70 m), and the previously unrecognized importance of the bedded tuft beneath the Calico Hills Formation as a subunit with generally more extensive zeolitization than the Calico Hills Formation in the southern part of the potential repository area. Reassessment of data from drill hole USW H-5 suggests that the zeolitization of this bedded unit occurs in the northwestern part of the repository exploration block as well. Further analyses of the same interval in USW H-3, however, have not permitted the same conclusion to be reached for the southwestern part of the repository block because of the much poorer quality of the cuttings in H-3 compared with those from H-5. X-ray fluorescence (XRF) chemical data for drill holes USW SD-7, 9, and 12 show that the zeolitic horizons provide a >10 million year record of retardation of Sr transport, although the data also show that simplistic models of one-dimensional downward flow in the unsaturated zone (UZ) are inadequate. Complex interstratification of zeolites and glass, with highly variable profiles between drill cores, point to remaining problems in constructing detailed mineral stratigraphies. However, the new data in this report provide important information for constructing bounding models of zeolite stratigraphy for transport calculations.

  9. Immunoglobulin G4-Related Disease Mimicking Asthma

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    Hiroshi Sekiguchi

    2013-01-01

    Full Text Available Immunoglobulin (Ig G4-related disease (also known as ‘IgG4-related sclerosing disease’, ‘IgG4-related systemic disease’ or ‘hyper-IgG4-disease’ is a recently recognized systemic fibroinflammatory disease associated with IgG4-positive plasma cells in tissue lesions. IgG4-related disease was initially described as autoimmune pancreatitis, but it is now known to affect virtually any organ. The authors describe a patient presenting with multi-organ manifestations, including airway inflammation mimicking asthma, pulmonary parenchymal infiltrates, intrathoracic lymphadenopathy, submandibular gland swelling and a kidney mass.

  10. Analysis of aquifer tests conducted in boreholes USW WT-10, UE-25 WT No. 12, and USW SD-7, 1995-96, Yucca Mountain, Nevada

    Energy Technology Data Exchange (ETDEWEB)

    O`Brien, G.M.

    1997-09-01

    Single-borehole aquifer tests were conducted in three boreholes in the Yucca Mountain area between March 1995 and January 1996 to obtain estimates of borehole specific capacity and aquifer transmissivity. Analysis of aquifer testing in borehole USW SD-7 also resulted in an estimate of reservoir volume. Aquifer-test data were analyzed with the Cooper and Jacob straight-line method, two modified Theis nonequilibrium equation solutions, and a modified reservoir-limit solution. The highest estimates of transmissivity were in borehole USW WT-10, completed in the Topopah Spring Tuff. Mean transmissivity, based on the results of three drawdown tests, was 1,600 meters squared per day. Mean specific capacity in borehole USW WT-10 after 5 hours of pumping was 1,100 meters squared per day, and was estimated to be 740 meters squared per day after 24 hours of pumping. Aquifer testing in borehole UE-25 WT No. 12 appeared to be significantly affected by well losses. A mean transmissivity of 7 meters squared per day was obtained on the basis of analysis of three drawdown tests in borehole UE-25 WT No. 12. Mean specific capacity in borehole UE-25 WT No. 12, after 24 hours of pumping, was 7 meters squared per day. Borehole UE-25 WT No. 12 seemed to be producing water from fractures that could provide only a limited amount of water to the borehole.

  11. IgG4 Related Sclerosing Cholangitis

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    D. Joshi

    2014-01-01

    Full Text Available IgG4 related disease (IgG4-RD is a multisystemic disorder which has only recently been recognized. IgG4 related sclerosing cholangitis (IgG4-SC is the biliary manifestation of the disease, often in association with autoimmune pancreatitis (AIP. In this review, we provide an overview of IgG4-RD, with a focus on the biliary manifestations. In particular, we describe the important differential diagnoses of IgG4-SC that need to be considered, namely, primary sclerosing cholangitis (PSC and cholangiocarcinoma, and provide a management algorithm. Finally, we highlight future directions and unanswered questions which will provide new insights into this exciting and evolving disease entity.

  12. IgG4-associated vasculitis.

    Science.gov (United States)

    Perez Alamino, Rodolfo; Martínez, Carlos; Espinoza, Luis R

    2013-08-01

    Elevated IgG4 is characteristic of cases of IgG4-RD, a newly recognized systemic disease. However, several chronic inflammatory conditions, including rheumatic diseases, can also be associated with increased levels of IgG4. There have also recently been several reports describing an increased IgG4 immune response to some vasculitis syndromes, in particular Churg-Strauss syndrome and granulomatosis with polyangiitis. To avoid misdiagnosis, clinicians must be aware that the clinical manifestations of IgG4-RD and ANCA-associated vasculitis may overlap. The meaning of these observations is not yet understood, and more studies are needed to determine the true significance of the increased IgG4 response to vasculitis syndromes, especially anti-neutrophil cytoplasmatic antibodies (ANCA)-associated vasculitis.

  13. IgG4-related sclerosing disease

    Institute of Scientific and Technical Information of China (English)

    Terumi Kamisawa; Atsutake Okamoto

    2008-01-01

    Based on histological and immunohistochemical examination of various organs of patients with autoimmune pancreatitis (AIP), a novel clinicopathological entity of IgG4-related sclerosing disease has been proposed. This is a systemic disease that is characterized by extensive IgG4-positive plasma cells and T-lymphocyte infiltration of various organs. Clinical manifestations are apparent in the pancreas, bile duct, gallbladder, salivary gland, retroperitoneum, kidney, lung, and prostate, in which tissue fibrosis with obliterative phlebitis is pathologically induced. AIP is not simply pancreatitis but, in fact, is a pancreatic disease indicative of IgG4-related sclerosing diseases. This disease includes AIP, sclerosing cholangitis, cholecystitis, sialadenitis, retroperitoneal fibrosis, tubulointerstitial nephritis, interstitial pneumonia, prostatitis, inflammatory pseudotumor and lymphadenopathy, all IgG4-related. Most IgG4-related sclerosing diseases have been found to be associated with AIP, but also those without pancreatic involvement have been reported. In some cases, only one or two organs are clinically involved, while in others, three or four organs are affected. The disease occurs predominantly in older men and responds well to steroid therapy. Serum IgG4 levels and immunostaining with anti-IgG4 antibody are useful in making the diagnosis. Since malignant tumors are frequently suspected on initial presentation, IgG4-related sclerosing disease should be considered in the differential diagnosis to avoid unnecessary surgery.

  14. IgG4-Related Disease without Overexpression of IgG4: Pathogenesis Implications

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    Naoshi Nishina

    2012-01-01

    Full Text Available IgG4-related disease is a new disease group that affects multiple organs. It is characterized by high serum IgG4 and abundant infiltration of IgG4-bearing plasma cells in the affected organ. Here, we describe an intriguing case that suggested that IgG4-related disease might present without IgG4 overexpression or infiltration, at least during a relapse. A 47-year-old man had been diagnosed with systemic lupus erythematosus 15 years. He was admitted due to a pituitary mass, systemic lymphadenopathy, and multiple nodules in the lungs and kidneys. The serum IgG4 level was normal and histopathological examination of the pituitary mass showed abundant lymphocyte and plasma cell infiltration with very few IgG4-positive cells. When we examined specimens preserved from 15 years ago, we found high serum IgG4 levels and IgG4-bearing plasma cell infiltration. This resulted in a diagnosis of IgG4-related disease, and we considered the current episode to be a relapse without IgG4 overexpression. This case indicated that, to clarify the pathogenesis of IgG4-related disease, current cases should repeat specimen evaluations over the course of IgG4-related disease to define diagnostic markers.

  15. IMac G4/800 (Flat Panel)

    CERN Multimedia

    2002-01-01

    Apple introduced the iMac G4/800 on January 7, 2002. The total cost was about $2000 (base price of $1799 plus RAM upgrade). The iMac G4/800 has an 800 MHz G4 processor. The G4/800 has the following ports: three USB 1.1 ports, two Fire Wire 400-Mbps ports, one RJ-45, 10/100BASE-T Ethernet port, one RJ-11 56K V.90 modem port, one Mini-VGA output port, one speaker jack, and one headphone jack. There is a built in microphone set on the front of the monitor in the bottom left corner. There is a single internal SuperDrive capable of reading and writing CDs and DVDs. The disk drive is on the front of the computer. It opens by pushing the disk drive button on the iMac's keyboard. The monitor size is 15 inches. The G4/800 has a NVIDIA GeForce2 MX graphics processor with AGP 2X support that provides 32 MB of dedicated Double Data Rate (DDR) video memory. Native resolution is 1024 x 768, but the built in monitor is also capable of 640 x 480 and 800 x 600. RAM can be changed by removing a panel on the bottom of the chas...

  16. IgG4-Related Perineural Disease

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    Dai Inoue

    2012-01-01

    Full Text Available Aims. To elucidate characteristics of IgG4-related disease involving the peripheral nervous system. Methods. Retrospective review of 106 patients with IgG4-related disease identified 21 peripheral nerve lesions in 7 patients. Clinicopathological and radiological features were examined. Results. Peripheral nerve lesions were commonly identified in orbital or paravertebral area, involving orbital (=9, optic (=4, spinal (=7, and great auricular nerves (=1. The predominant radiological feature was a distinct perineural soft tissue mass, ranging 8 to 30 mm in diameter. Histologically, the epineurium was preferentially involved by massive lymphoplasmacytic infiltration rich in IgG4+ plasma cells. All lesions were neurologically asymptomatic and steroid-responsive at the first presentation, but one recurrent lesion around the optic nerve caused failing vision. Conclusion. IgG4-related disease of the peripheral nervous system is characterized by orbital or paravertebral localization, perineural mass formation, and rare neurologic symptoms. The term “IgG4-related perineural disease” seems appropriate to describe this entity.

  17. IgG4-related prostatitis progressed from localized IgG4-related lymphadenopathy

    Science.gov (United States)

    Li, Dujuan; Kan, Yunzhen; Fu, Fangfang; Wang, Shuhuan; Shi, Ligang; Liu, Jie; Kong, Lingfei

    2015-01-01

    Immunoglobulin G4-related disease (IgG4-RD) is a recently described inflammatory disease involving multiple organs. Prostate involvement with IgG4-RD is very rare. In this report, we describe a case of IgG4-related prostatitis progressed from localized IgG4-related lymphadenopathy. This patient was present with urine retention symptoms. MRI and CT examination revealed the prostatic enlargement and the multiple lymphadenopathy. Serum IgG4 levels were elevated. Prostatic tissue samples resected both this time and less than 1 year earlier showed the same histological type of prostatitis with histopathologic and immunohistochemical findings characteristic of IgG4-RD. The right submandibular lymph nodes excised 2 years earlier were eventually proven to be follicular hyperplasia-type IgG4-related lymphadenopathy. This is the first case of IgG4-RD that began as localized IgG4-related lymphadenopathy and progressed into a systemic disease involving prostate and multiple lymph nodes. This patient showed a good response to steroid therapy. This leads us to advocate a novel pathogenesis of prostatitis, and a novel therapeutic approach against prostatitis. Pathologists and urologists should consider this disease entity in the patients with elevated serum IgG4 levels and the symptoms of prostatic hyperplasia to avoid ineffective medical or unnecessary surgical treatment. PMID:26617921

  18. IgG4-Related Nasal Pseudotumor

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    L. K. Døsen

    2015-01-01

    Full Text Available IgG4-related disease is recognized as one form of autoimmune pancreatitis. During the last ten years, it has also been described in several other organs. We present two patients with lesions showing a histological picture of fibrosis and lymphoplasmacytic infiltrations with abundant IgG4 positive plasma cells at hitherto unreported symmetrical nasal locations. The symmetrical complex consisted of one central lesion in the anterior nasal septum and the two others in each of the lateral nasal walls. The lesions extended from the anterior part of the inferior concha into the vestibulum and caused severe nasal obstruction.

  19. IgG4-associated cholangitis.

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    Nath, Vikas; Lewin, Jack; Subramony, Charu; Shenoy, Veena

    2014-12-01

    We report a young female patient with IgG4-associated cholangitis (IAC) who presented with common bile duct (CBD) stricture and review the features that distinguish IAC from both primary sclerosing cholangitis (PSC) and other types of secondary sclerosing cholangitis (SSC). IAC is a biliary manifestation of IgG4-related sclerosing disease, an autoimmune condition characterized by elevated serum IgG4 and infiltrates containing lymphocytes and IgG4-positive plasma cells, accompanied by sclerosis. On endoscopic retrograde cholangiopancreatography, IAC consists of segmental biliary strictures with a predilection for the distal CBD, whereas in PSC the strictures are more band-like; other types of SSC often demonstrate unifocal ductal obstructions, sometimes associated with choleliths. On histologic examination, the bile duct wall in IAC contains a denser lymphocytic infiltrate and sparser sclerosis than in PSC; other types of SSC can be distinguished histologically by the types of inflammatory cells present. Unlike those of PSC, IAC-related strictures are reversible with corticosteroids.

  20. IgG4 Related Lung Disease

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    Mihir Patel

    2016-01-01

    Full Text Available IgG4 related disease is a poorly understood immune mediated condition. Lung involvement is rare and difficult to diagnose and can mimic primary lung malignancy on imaging. A patient who was found to have an incidental lung lesion with risk factors for primary pulmonary malignancy is reported.

  1. Gravitational Waves in G4v

    CERN Document Server

    Mead, Carver

    2015-01-01

    Gravitational coupling of the propagation four-vectors of matter wave functions is formulated in flat space-time. Coupling at the momentum level rather than at the "force-law" level greatly simplifies many calculations. This locally Lorentz-invariant approach (G4v) treats electromagnetic and gravitational coupling on an equal footing. Classical mechanics emerges from the incoherent aggregation of matter wave functions. The theory reproduces, to first order beyond Newton, the standard GR results for Gravity-Probe B, deflection of light by massive bodies, precession of orbits, gravitational red shift, and total gravitational-wave energy radiated by a circular binary system. Its predictions of total radiated energy from highly eccentric Kepler systems are slightly larger than those of similar GR treatments. G4v predictions differ markedly from those of GR for the gravitational-wave radiation patterns from rotating massive systems, and for the LIGO antenna pattern. The predicted antenna patterns have been shown t...

  2. Immunoglobulin G4-related cholangitis: a variant of IgG4-related systemic disease.

    Science.gov (United States)

    Novotný, Ivo; Dítě, Petr; Trna, Jan; Lata, Jan; Husová, Libuše; Geryk, Edvard

    2012-01-01

    IgG4-related sclerosing cholangitis as part of IgG4 systemic-related diseases is commonly associated with autoimmune pancreatitis. Major clinical manifestations of IgG4-related sclerosing diseases are apparent in the organs in which tissue fibrosis with obstructive phlebitis is pathologically induced. IgG4-related sclerosing cholangitis is included within the heterogeneous group of 'sclerosing cholangitis'. Sclerosing cholangitis may be associated with choledocholithiasis, infection or biliary malignancies. Sclerosing cholangitis of unknown etiology is called primary sclerosing cholangitis (PSC). Conservative therapy of PSC is usually unsuccessful, the disease involves extra- and/or intrahepatic biliary tree, and the end point of this disease is liver cirrhosis. Typically, PSC is identified at the age of 30 to 40 years, and the disease is frequently associated with inflammatory bowel diseases. On the other hand, IgG4-related sclerosing cholangitis is not associated with inflammatory bowel diseases. In patients with IgG4-related sclerosing cholangitis, a first symptom can be obstructive jaundice, whereas obstructive jaundice is rarely present in PSC. Clinically, patients with IgG4-related sclerosing cholangitis are older at diagnosis compared to patients with PSC. A typical diagnostic feature of IgG4-related sclerosing cholangitis is elevation of serum immunoglobulin G4. In patients with IgG4-related sclerosing cholangitis, response to steroid therapy is high; in patients with PSC corticosteroid therapy is unsuccessful. Histochemically abundant infiltration of IgG4-positive plasma cells is detected in the biliary duct wall. Histologically, we can identify dense lymphoplasmacytic infiltration of the bile duct wall, transmural fibrosis, lymphoplasmacytic infiltration and fibrosis in the periportal area of the liver - a typically obliterative phlebitis. The biliary epithelium is usually intact in contrast to PSC, where mucosal erosion is often present. Steroids are the

  3. IgG4相关性疾病%IgG4-related Disease

    Institute of Scientific and Technical Information of China (English)

    林玮; 张文

    2010-01-01

    IgG4相关性疾病是一种与IgG4淋巴细胞密切相关的慢性、系统性疾病,该类疾病以血清IgG4水平升高以及IgG4阳性细胞浸润多种器官和组织为特征,常见受累器官包括泪腺、胰腺和腹膜后间隙等,累及的器官或组织由于慢性炎症及纤维化进程可导致弥漫性肿大.该类疾病对皮质激素治疗反应良好.

  4. Statistical test of reproducibility and operator variance in thin-section modal analysis of textures and phenocrysts in the Topopah Spring member, drill hole USW VH-2, Crater Flat, Nye County, Nevada

    Energy Technology Data Exchange (ETDEWEB)

    Moore, L.M.; Byers, F.M. Jr.; Broxton, D.E.

    1989-06-01

    A thin-section operator-variance test was given to the 2 junior authors, petrographers, by the senior author, a statistician, using 16 thin sections cut from core plugs drilled by the US Geological Survey from drill hole USW VH-2 standard (HCQ) drill core. The thin sections are samples of Topopah Spring devitrified rhyolite tuff from four textural zones, in ascending order: (1) lower nonlithophysal, (2) lower lithopysal, (3) middle nonlithophysal, and (4) upper lithophysal. Drill hole USW-VH-2 is near the center of the Crater Flat, about 6 miles WSW of the Yucca Mountain in Exploration Block. The original thin-section labels were opaqued out with removable enamel and renumbered with alpha-numeric labels. The sliders were then given to the petrographer operators for quantitative thin-section modal (point-count) analysis of cryptocrystalline, spherulitic, granophyric, and void textures, as well as phenocryst minerals. Between operator variance was tested by giving the two petrographers the same slide, and within-operator variance was tested by the same operator the same slide to count in a second test set, administered at least three months after the first set. Both operators were unaware that they were receiving the same slide to recount. 14 figs., 6 tabs.

  5. IgG4-related disease%IgG4相关疾病

    Institute of Scientific and Technical Information of China (English)

    蒲佳曦

    2014-01-01

    IgG4相关疾病(IgG4-RD)是一种新发现的累及全身多器官损伤的少见临床疾病.IgG4-RD的发病机制尚未明确,尤其IgG4是直接介导组织损伤还是旁观效应仍不清楚.IgG4-RD表现为各种器官同时或非同时的器官肿大或结节/增生病变,相关检查主要包括血清学,病理学和影像学.IgG4-RD综合诊断标准的诊断算法是用综合诊断标准结合器官特异性标准而制定的.治疗方面虽无指南可循但仍然推荐首选激素治疗,必要时考虑运用免疫抑制剂和生物治疗.

  6. Cutoff Values of Serum IgG4 and Histopathological IgG4+ Plasma Cells for Diagnosis of Patients with IgG4-Related Disease

    Directory of Open Access Journals (Sweden)

    Yasufumi Masaki

    2012-01-01

    Full Text Available IgG4-related disease is a new disease classification established in Japan in the 21st century. Patients with IgG4-related disease display hyper-IgG4-gammaglobulinemia, massive infiltration of IgG4+ plasma cells into tissue, and good response to glucocorticoids. Since IgG4 overexpression is also observed in other disorders, it is necessary to diagnose IgG4-related disease carefully and correctly. We therefore sought to determine cutoff values for serum IgG4 and IgG4/IgG and for IgG4+/IgG+ plasma cells in tissue diagnostic of IgG4-related disease. Patients and Methods. We retrospectively analyzed serum IgG4 concentrations and IgG4/IgG ratio and IgG4+/IgG+ plasma cell ratio in tissues of 132 patients with IgG4-related disease and 48 patients with other disorders. Result. Serum IgG4 >135  mg/dl demonstrated a sensitivity of 97.0% and a specificity of 79.6% in diagnosing IgG4-related disease, and serum IgG4/IgG ratios >8% had a sensitivity and specificity of 95.5% and 87.5%, respectively. IgG4+cell/IgG+ cell ratio in tissues >40% had a sensitivity and specificity of 94.4% and 85.7%, respectively. However, the number of IgG4+ cells was reduced in severely fibrotic parts of tissues. Conclusion. Although a recent unanimous consensus of all relevant researchers in Japan recently established the diagnostic criteria for IgG4-related disease, findings such as ours indicate that further discussion is needed.

  7. Immunoglobulin G4-Associated Cholangitis: One Variant of Immunoglobulin G4-Related Systemic Disease

    NARCIS (Netherlands)

    Y.A. Alderlieste; B.D.J. van den Elzen; E.A.J. Rauws; U. Beuers

    2009-01-01

    IgG4-associated cholangitis (IAC) is a recently defined disease entity which shares a number of clinical, biochemical, and radiological features with primary sclerosing cholangitis (PSC). In contrast to PSC, IAC responds to immunosuppressive treatment, is not associated with inflammatory bowel disea

  8. G4S fuajee ruumiinstallatsioon = G4S lobby spatial installation / Ville Lausmäe

    Index Scriptorium Estoniae

    Lausmäe, Ville, 1981-

    2013-01-01

    Turvafirma G4S büroohoone (Paldiski mnt 80, Tallinn) fuajee sisekujundusest. Autorid: Ville Lausmäe, Kadi Karmann (sisearhitektuuribüroo VLS). Kultuuriministeeriumi kunstinõuniku Maria-Kristiina Soomre arvamus. Lühidalt sisearhitektuuribüroost VLS

  9. G4S fuajee ruumiinstallatsioon = G4S lobby spatial installation / Ville Lausmäe

    Index Scriptorium Estoniae

    Lausmäe, Ville, 1981-

    2013-01-01

    Turvafirma G4S büroohoone (Paldiski mnt 80, Tallinn) fuajee sisekujundusest. Autorid: Ville Lausmäe, Kadi Karmann (sisearhitektuuribüroo VLS). Kultuuriministeeriumi kunstinõuniku Maria-Kristiina Soomre arvamus. Lühidalt sisearhitektuuribüroost VLS

  10. G4CEP: A G4 theory modification by including pseudopotential for molecules containing first-, second- and third-row representative elements.

    Science.gov (United States)

    Silva, Cleuton de Souza; Pereira, Douglas Henrique; Custodio, Rogério

    2016-05-28

    The G4CEP composite method was developed from the respective G4 all-electron version by considering the implementation of compact effective pseudopotential (CEP). The G3/05 test set was used as reference to benchmark the adaptation by treating in this work atoms and compounds from the first and second periods of the periodic table, as well as representative elements of the third period, comprising 440 thermochemical data. G4CEP has not reached a so high level of accuracy as the G4 all-electron theory. G4CEP presented a mean absolute error around 1.09 kcal mol(-1), while the original method presents a deviation corresponding to 0.83 kcal mol(-1). The similarity of the optimized molecular geometries between G4 and G4CEP indicates that the core-electron effects and basis set adjustments may be pointed out as a significant factor responsible for the large discrepancies between the pseudopotential results and the experimental data, or even that the all-electron calculations are more efficient either in its formulation or in the cancellation of errors. When the G4CEP mean absolute error (1.09 kcal mol(-1)) is compared to 1.29 kcal mol(-1) from G3CEP, it does not seem so efficient. However, while the G3CEP uncertainty is ±4.06 kcal mol(-1), the G4CEP deviation is ±2.72 kcal mol(-1). Therefore, the G4CEP theory is considerably more reliable than any previous combination of composite theory and pseudopotential, particularly for enthalpies of formation and electron affinities.

  11. G4CEP: A G4 theory modification by including pseudopotential for molecules containing first-, second- and third-row representative elements

    Science.gov (United States)

    Silva, Cleuton de Souza; Pereira, Douglas Henrique; Custodio, Rogério

    2016-05-01

    The G4CEP composite method was developed from the respective G4 all-electron version by considering the implementation of compact effective pseudopotential (CEP). The G3/05 test set was used as reference to benchmark the adaptation by treating in this work atoms and compounds from the first and second periods of the periodic table, as well as representative elements of the third period, comprising 440 thermochemical data. G4CEP has not reached a so high level of accuracy as the G4 all-electron theory. G4CEP presented a mean absolute error around 1.09 kcal mol-1, while the original method presents a deviation corresponding to 0.83 kcal mol-1. The similarity of the optimized molecular geometries between G4 and G4CEP indicates that the core-electron effects and basis set adjustments may be pointed out as a significant factor responsible for the large discrepancies between the pseudopotential results and the experimental data, or even that the all-electron calculations are more efficient either in its formulation or in the cancellation of errors. When the G4CEP mean absolute error (1.09 kcal mol-1) is compared to 1.29 kcal mol-1 from G3CEP, it does not seem so efficient. However, while the G3CEP uncertainty is ±4.06 kcal mol-1, the G4CEP deviation is ±2.72 kcal mol-1. Therefore, the G4CEP theory is considerably more reliable than any previous combination of composite theory and pseudopotential, particularly for enthalpies of formation and electron affinities.

  12. Clinical study on IgG4-related autoimmune pancreatitis

    Institute of Scientific and Technical Information of China (English)

    吴庆军

    2013-01-01

    Objective To investigate the clinical features of IgG4-related autoimmune pancreatitis(IgG4-related AIP). Methods A prospective cohort study on IgG4 related disease(IgG4-RD) was carried out in Peking Union Medical College Hospital during December 2010 to June

  13. Stratigraphy, structure, and some petrographic features of Tertiary volcanic rocks in the USW G-2 drill hole, Yucca Mountain, Nye County, Nevada

    Science.gov (United States)

    Maldonado, Florian; Koether, S.L.

    1983-01-01

    A continuously cored drill hole designated as USW G-2, located at Yucca Mountain in southwestern Nevada, penetrated 1830.6 m of Tertiary volcanic strata composed of abundant silicic ash-flow tuffs, minor lava and flow breccias, and subordinate volcaniclastic rocks. The volcanic strata penetrated are comprised of the following in descending order: Paintbrush Tuff (Tiva Canyon Member, Yucca Mountain Member, bedded tuff, Pah Canyon Member, and Topopah Spring Member), tuffaceous beds of Calico Hills, Crater Flat Tuff (Prow Pass Member, Bullfrog Member, and Tram unit), lava and flow breccia (rhyodacitic), tuff of Lithic Ridge, bedded and ash-flow tuff, lava and flow breccia (rhyolitic, quartz latitic, and dacitic), bedded tuff, conglomerate and ash-flow tuff, and older tuffs of USW G-2. Comparison of unit thicknesses at USW G-2 to unit thicknesses at previously drilled holes at Yucca Mountain indicate the following: (1) thickening of the Paintbrush Tuff members and tuffaceous beds of Calico Hills toward the northern part of Yucca Mountain; (2) thickening of the Prow Pass Member but thinning of the Bullfrog Member and Tram unit; (3) thinning of the tuff of Lithic Ridge; (4) presence of approximately 280 m of lava and flow breccia not previously penetrated by any drill hole; and (5) presence of an ash-flow tuff unit at the bottom of the drill hole not previously intersected, apparently the oldest unit penetrated at Yucca Mountain to date. Petrographic features of some of the units include: (1) decrease in quartz and K-feldspar and increases in biotite and plagioclase with depth in the tuffaceous beds of Calico Hills; (2) an increase in quartz phenocrysts from the top to the bottom members of the Crater Flat Tuff; (3) a low quartz content in the tuff of Lithic Ridge, suggesting tapping of the magma chamber at quartz-poor levels; (4) a change in zeolitic alteration from heulandite to clinoptilolite to mordenite with increasing depth; (5) lavas characterized by a rhyolitic

  14. Cholangiocarcinoma with respect to IgG4 Reaction

    Directory of Open Access Journals (Sweden)

    Kenichi Harada

    2014-01-01

    Full Text Available IgG4 reactions marked by infiltration of IgG4-positive plasma cells in affected organs occur in cancer patients and in patients with IgG4-related diseases. Extrahepatic cholangiocarcinomas including gall bladder cancer are often accompanied by significant IgG4 reactions; these reactions show a negative correlation with CD8-positive cytotoxic T cells, suggesting that the evasion of immune surveillance is associated with cytotoxic T cells. The regulatory cytokine IL-10 may induce IgG4-positive plasma cell differentiation or promote B cell switching to IgG4 in the presence of IL-4. Cholangiocarcinoma cells may function as nonprofessional antigen presenting cells that indirectly induce IgG4 reactions via the IL-10-producing cells and/or these may act as Foxp3-positive and IL-10-producing cells that directly induce IgG4 reactions. Moreover, IgG4-related disease is a high-risk factor for cancer development; IgG4-related sclerosing cholangitis (IgG4-SC cases associated with cholangiocarcinoma or its precursor lesion biliary intraepithelial neoplasia (BilIN have been reported. IgG4-positive cell infiltration is an important finding of IgG4-SC but is not a histological hallmark of IgG4-SC. For the diagnosis of IgG4-SC, its differentiation from cholangiocarcinoma remains important.

  15. IgG4检测在IgG4相关性疾病的新进展%Value of serum IgG4 in IgG4-related disease

    Institute of Scientific and Technical Information of China (English)

    荆红运; 关秀茹

    2016-01-01

    Immunoglobulin G4(IgG4)-related disease (IgG4-RD) is a new systemic and chronic autoimmune disease.It is characterized by increased serum levels of IgG 4 and infiltration of abundant IgG 4-positive cells in various organs and tissues .It is needed to distinguish IgG 4-RD and other diseases which will induce high level of serum IgG4 and infiltration of abundant IgG4-positive cells in tissues.This study described the value of serum IgG4 in IgG4-RD.%IgG4相关性疾病( IgG4-RD)是一种新的系统性、慢性自身免疫性疾病。临床表现为IgG4阳性浆细胞及淋巴细胞浸润多种器官和组织,并伴有血清IgG4水平升高。此外,一些其他疾病也会出现血清IgG4升高或组织中IgG4阳性浆细胞浸润,因此需要进行鉴别诊断。本文论述了血清IgG4对IgG4-RD诊断及鉴别诊断的价值。(中华检验医学杂志,2016,39:817-819)

  16. Detailed petrographic descriptions and microprobe data for tertiary silicic volcanic rocks in drill hole USW G-1, Yucca Mountain, Nevada

    Energy Technology Data Exchange (ETDEWEB)

    Caporuscio, F.A.; Warren, R.G.; Broxton, D.E.

    1985-12-01

    This report contains detailed petrographic descriptions of 74 thin sections from drill hole USW G-1 at Yucca Mountain, Nevada. These descriptions are keyed to the distinctions between devitrified, vitrophyre, vitric, and zeolitized intervals below the Topopah Spring Member repository horizon. The petrographic features of the zeolitized intervals down through the Crater Flat tuff, as well as the sorption properties determined from these intervals, suggest that these zeolite occurrences may each have comparable sorptive capability.

  17. Pathologies Associated with Serum IgG4 Elevation

    Directory of Open Access Journals (Sweden)

    Mikael Ebbo

    2012-01-01

    Full Text Available Statement of Purpose. IgG4-related disease (IgG4-RD is usually associated to an increase of serum IgG4 levels. However other conditions have also been associated to high serum IgG4 levels. Methods. All IgG subclasses analyses performed in our hospital over a one-year period were analyzed. When IgG4 level were over 1.35 g/L, the patient’s clinical observation was analyzed and both final diagnosis and reason leading to IgG subclasses analysis were recorded. Only polyclonal increases of IgG4 were considered. Summary of the Results. On 646 IgG subclass analysis performed, 59 patients had serum IgG4 over 1.35 g/L. The final diagnosis associated to serum IgG4 increase was very variable. Most patients (25% presented with repeated infections, 13.5% with autoimmune diseases, and 10% with IgG4-RD. Other patients presented with cancer, primary immune deficiencies, idiopathic interstitial lung disease, cystic fibrosis, histiocytosis, or systemic vasculitis and 13.5% presented with various pathologies or no diagnosis. Mean IgG4 levels and IgG4/IgG ratio were higher in IgG4-RD than in other pathologies associated to elevated IgG4 levels. Conclusions. Our study confirms that elevation of serum IgG4 is not specific to IgG4-RD. Before retaining IgG4-RD diagnosis in cases of serum IgG4 above 1.35 g/L, several other pathological conditions should be excluded.

  18. Detection of serum IgG4 levels in patients with IgG4-related disease and other disorders.

    Directory of Open Access Journals (Sweden)

    Yuying Su

    Full Text Available Elevated serum IgG4 levels are an important hallmark for diagnosing IgG4-related disease (IgG4-RD, but can also be observed in other diseases. This study aimed to compare two different testing methods for IgG4: ELISA and nephelometric assay. Both assays were used to measure serum IgG4 concentrations, and to assess the prevalence of high serum IgG4 levels in both IgG4-RD and non-IgG4-RD diseases.A total of 80 serum samples were tested using the nephelometric assay and ELISA method that we established. Serum IgG4 concentrations were determined by ELISA for 957 patients with distinct diseases, including 12 cases of IgG4-RD and 945 cases of non-IgG4-RD.IgG4 levels from 80 selected serum samples examined by ELISA were in agreement with those detected using the nephelometry assay. Meanwhile, the serum IgG4 concentrations measured by ELISA were also consistent with the clinical diagnoses of patients with IgG4-RD during the course of disease. The Elevated levels of serum IgG4 (>1.35 g/L were detected in all IgG4-RD (12/12 patients, and the prevalence of high IgG4 serum levels was 3.39% in non-IgG4-RD cases. Among them, the positive rates of serum IgG4 were 2.06% in patients with carcinoma and 6.3% in patients with other non-IgG4 autoimmune diseases.Our established ELISA method is a reliable and convenient technique, which could be extensively used in the clinic to measure serum IgG4 levels. High levels of IgG4 were observed in IgG4-RD. However, this phenomenon could also be observed in other diseases, such as carcinomas and other autoimmune diseases. Thus, a diagnosis of IgG4 disease cannot only be dependent on the detection of elevated serum IgG4 levels.

  19. Immunglobulin G4-relateret sygdom er en ny inflammatorisk sygdom

    DEFF Research Database (Denmark)

    Næser, Esben Kure; Marcussen, Niels; Gregersen, Jon Waarst

    2012-01-01

    IgG4-related disease is a newly recognized inflammatory disease characterized by tissue infiltrates of IgG4 positive plasma cells. The disease was first recognized in pancreas but has now been described in nearly every organ. The diagnosis is based on the presence of dense lymphoplasmocytic...... infiltrates rich in IgG4 positive plasma cells. We describe a case of a 76-year-old man with IgG4-related disease involving the kidneys....

  20. softinex, inlib, exlib, ourex, ioda, g4view, g4exa, wall

    Science.gov (United States)

    Barrand, Guy

    2014-06-01

    Softinex names a software environment targeted to do data analysis and visualization. It covers the C++ inlib and exlib "header only" libraries that permit, through GL-ES and a maximum of common code, to build applications deliverable on the Apple AppStore (iOS), GooglePlay (Android), traditional laptops/desktops under MacOSX, Linux and Windows, but also deliverable as a web service able to display in various web browsers compatible with WebGL. In this paper we explain the coarse graining ideas, choices, code organization of softinex along a short presentation of some applications done so far (ioda, g4view, etc...). At end we present the "wall" programs that permit to visualize HEP data (plots, geometries, events) on a large display surface done with an assembly of screens driven by a set of computers. The web portal for softinex is http://softinex.lal.in2p3.fr.

  1. IgG4 Inflammatory Pseudotumor of the Kidney

    Directory of Open Access Journals (Sweden)

    Ahmed N. Alkhasawneh

    2012-01-01

    Full Text Available Hyper-IgG4 disease is a rare systemic disorder that usually affects middle age males. It is characterized by elevated serum IgG4 levels and infiltration of organs by IgG4 positive plasma cells associated with fibrosis. Patients usually present with mass or masses in the involved organ that mimic neoplasia. While initially described in the pancreas, IgG4-related inflammatory tumors have been now described in many organs. We describe an unusual case of an IgG4-related pseudotumor of the kidney.

  2. IgG4-related pleuritis with chylothorax.

    Science.gov (United States)

    Kato, Eisuke; Takayanagi, Noboru; Ishiguro, Takashi; Kagiyama, Naho; Shimizu, Yoshihiko; Sugita, Yutaka

    2014-01-01

    Presently, 6 cases of IgG4-related pleuritis have been reported. We encountered a patient who developed chylothorax due to IgG4-related disease. To our knowledge, such patients have not been reported. This patient developed right-sided chylothorax and left-sided non-chylothorax lymphocyte-predominant pleuritis. Elevated serum and pleural IgG4 concentrations and histopathological analysis of pleural biopsy confirmed the diagnosis of IgG4-related pleuritis. Left-sided pleuritis improved with corticosteroid therapy, but right-sided chylothorax persists. IgG4-related disease can be one cause of chylothorax.

  3. IgG4-related Disease of the Genitourinary Tract

    Directory of Open Access Journals (Sweden)

    Mukul K. Divatia

    2014-02-01

    Full Text Available IgG4-related disease (IgG4-RD is a recently established albeit well recognized fibro-inflammatory condition with distinctive features including a characteristic histopathological appearance; a propensity to develop tumefactive lesions in multiple body sites; and oft elevated serum IgG4 levels. The consensus statement on IgG-4 RD equips practicing pathologists with a set of working guidelines for the diagnosis of pathologic lesions identified in a host of different organ system affected with this disease. The diagnosis of IgG4-RD requires the combined presence of the characteristic histopathological appearance and increased numbers of IgG4-positive plasma cells. The essential histopathological features include a dense lymphoplasmacytic infiltrate, a storiform pattern of fibrosis, and obliterative phlebitis. Tissue IgG4-positive plasma cell counts and IgG4: IgG ratios are significant ancillary aids in establishing the diagnosis. The spectrum of IgG4-RD continues to expand and involve multiple body sites. The genitourinary system comprising of the kidneys, ureters, urinary bladder, urethra, prostate gland, testes and penis is one of the multiple organ systems to be affected by IgG4-RD. This review describes the clinical and histopathologic patterns of involvement of the genitourinary system by IgG4-RD, in association with serologic and radiological features. [J Interdiscipl Histopathol 2014; 2(1.000: 3-18

  4. Development of an IgG4-RD Responder Index

    Directory of Open Access Journals (Sweden)

    Mollie N. Carruthers

    2012-01-01

    Full Text Available IgG4-related disease (IgG4-RD is a multiorgan inflammatory disease in which diverse organ manifestations are linked by common histopathological and immunohistochemical features. Prospective studies of IgG4-RD patients are required to clarify the natural history, long-term prognosis, and treatment approaches in this recently recognized condition. Patients with IgG4-RD have different organ manifestations and are followed by multiple specialties. Divergent approaches to the assessment of patients can complicate the interpretation of studies, emphasizing the critical need for validated outcome measures, particularly assessments of disease activity and response to treatment. We developed a prototype IgG4-RD Responder Index (IgG4-RD RI based on the approach used in the development of the Birmingham Vasculitis Activity Score for Wegener’s granulomatosis (BVAS/WG. The IgG4-RD RI was refined by members of the International IgG4-RD Symposium Organizing Committee in a paper case exercise. The revised instrument was applied retrospectively to fifteen IgG4-RD patients at our institution. Those scores were compared to physician’s global assessment scale for the same visits. This paper describes the philosophy and goals of the IgG4-RD RI, the steps in the development of this instrument to date, and future plans for validation of this instrument as an outcome measure.

  5. Immunoglobulin G4-related disease: autoimmune pancreatitis and extrapancreatic manifestations*

    Science.gov (United States)

    Fernandes, Daniel Alvarenga; Kido, Ricardo Yoshio Zanetti; Barros, Ricardo Hoelz de Oliveira; Martins, Daniel Lahan; Penachim, Thiago José; Caserta, Nelson Marcio Gomes

    2016-01-01

    We present a case of immunoglobulin G4 (IgG4)-related disease with pancreatic and extrapancreatic involvement, including the biliary and renal systems. Given the importance of imaging methods for the diagnosis of IgG4-related disease and its differentiation from pancreatic adenocarcinoma, we emphasize important abdominal computed tomography and magnetic resonance imaging findings related to this recently recognized systemic autoimmune disease. PMID:27141136

  6. Immunoglobulin G4-related pancreatic and biliary diseases

    OpenAIRE

    Hisham Al-Dhahab; Julia McNabb-Baltar; Said Al-Busafi; Alan N Barkun

    2013-01-01

    BACKGROUND: Autoimmune pancreatitis and autoimmune cholangitis are new clinical entities that are now recognized as the pancreaticobiliary manifestations of immunoglobulin (Ig) G4-related disease.OBJECTIVE: To summarize important clinical aspects of IgG4-related pancreatic and biliary diseases, and to review the role of IgG4 in the diagnosis of autoimmune pancreatitis (AIP) and autoimmune cholangitis (AIC).METHODS: A narrative review was performed using the PubMed database and the following k...

  7. Immunoglobulin G4-related disease: autoimmune pancreatitis and extrapancreatic manifestations

    Directory of Open Access Journals (Sweden)

    Daniel Alvarenga Fernandes

    2016-04-01

    Full Text Available Abstract We present a case of immunoglobulin G4 (IgG4-related disease with pancreatic and extrapancreatic involvement, including the biliary and renal systems. Given the importance of imaging methods for the diagnosis of IgG4-related disease and its differentiation from pancreatic adenocarcinoma, we emphasize important abdominal computed tomography and magnetic resonance imaging findings related to this recently recognized systemic autoimmune disease.

  8. IgG4-related disease and the kidney.

    Science.gov (United States)

    Cortazar, Frank B; Stone, John H

    2015-10-01

    IgG4-related disease (IgG4-RD) is a systemic fibroinflammatory condition that involves almost every organ system. In this Review, we summarize current knowledge of IgG4-RD and its most frequent manifestations in the kidney—IgG4-related tubulointerstitial nephritis (TIN) and membranous glomerulonephropathy (MGN). Diagnosis of IgG4-RD relies on histopathology: the typical features are a dense lymphoplasmacytic infiltrate and storiform fibrosis. A high percentage of plasma cells observed within lesions stain positively for IgG4. IgG4-related TIN bears the hallmark pathological findings of IgG4-RD; distinctive radiographic characteristics are also frequently observed with use of contrast-enhanced CT. MGN secondary to IgG4-RD seems to be distinct from idiopathic MGN. Humoral and cell-mediated immunity seem to have roles in the pathophysiology of IgG4-RD, but the details of these roles remain unclear. The IgG4 molecule itself is unlikely to be the primary driver of inflammation; rather, it probably downregulates the immune response. Fibrosis might be caused by activation of innate immune cells by polarized CD4(+) T cells. Glucocorticoids are the standard initial treatment for IgG4-RD, but their long-term adverse effects and the high frequency of relapse and renal damage associated with use of this treatment has prompted a search for more effective options. B-cell depletion and the targeting of plasmablasts are both promising approaches.

  9. Immunoglobulin G4-Related Pancreatic and Biliary Diseases

    Directory of Open Access Journals (Sweden)

    Hisham Al-Dhahab

    2013-01-01

    Full Text Available BACKGROUND: Autoimmune pancreatitis and autoimmune cholangitis are new clinical entities that are now recognized as the pancreaticobiliary manifestations of immunoglobulin (Ig G4-related disease.

  10. Geohydrology of rocks penetrated by test well USW H-6, Yucca Mountain, Nye County, Nevada

    Energy Technology Data Exchange (ETDEWEB)

    Craig, R.W. [Geological Survey, Denver, CO (United States); Reed, R.L. [Fenix and Scisson, Inc., Tulsa, OK (United States)

    1991-12-01

    Test well USW H-6 is one of several wells drilled in the Yucca Mountain area near the southwestern part of the Nevada Test Site for investigations related to isolation of high-level nuclear waste. This well was drilled to a depth of 1,220 meters. Rocks penetrated are predominantly ash-flow tuffs of Tertiary age, with the principal exception of dacitic(?) lave penetrated at a depth from 877 to 1,126 meters. The composite static water level was about 526 meters below the land surface; the hydraulic head increased slightly with depth. Most permeability in the saturated zone is in two fractured intervals in Crater Flat Tuff. Based on well-test data using the transitional part of a dual-porosity solution, an interval of about 15 meters in the middle part of the Bullfrog Member of the Crater Flat Tuff has a calculated transmissivity of about 140 meters squared per day, and an interval of about 11 meters in the middle part of the Tram Member of the Crater Flat Tuff has a calculated transmissivity of about 75 meters squared per day. The upper part of the Bullfrog Member has a transmissivity of about 20 meters squared per day. The maximum likely transmissivity of any rocks penetrated by the test well is about 480 meters squared per day, based on a recharge-boundary model. The remainder of the open hole had no detectable production. Matrix hydraulic conductivity ranges from less than 5 {times} 10{sup {minus}5} to 1 {times} 10{sup {minus}3} meter per day. Ground water is a sodium bicarbonate type that is typical of water from tuffaceous rock of southern Nevada. The apparent age of the water is about 14,6000 years. 29 refs., 26 figs., 5 tabs.

  11. IgG4相关性疾病的研究进展%Immunoglobulin G4-related disease

    Institute of Scientific and Technical Information of China (English)

    张雪英; 乔建军

    2016-01-01

    IgG4相关性疾病是近年来提出的一种新病种,本病可累及人体任何组织和器官.IgG4相关性疾病的显著特征为血清IgG4水平升高以及多种器官和组织中IgG4阳性浆细胞浸润.近年来发现,IgG4相关性疾病可有皮肤改变,皮损可表现为多种皮肤病的症状,包括Mikulicz病、皮肤浆细胞增多症、假性淋巴瘤、血管淋巴样增生伴嗜酸细胞增多和木村病等.IgG4相关性疾病患者对系统糖皮质激素治疗反应较好.%Immunoglobulin G4 (IgG4)-related disease is a recently proposed entity, and can affect any tissue or organ in the human body.It is characterized by increased serum levels of IgG4 and infiltration of abundant IgG4-positive cells in various organs and tissues.Recent studies have found that IgG4-related disease may present with various skin manifestations, such as Mikulicz's disease, cutaneous plasmacytosis, pseudolymphoma, angiolymphoid hyperplasia with eosinophilia and Kimura's disease.Systemic glucocorticoids are effective for the treatment of IgG4-related disease.

  12. 26 CFR 54.4980G-4 - Calculating comparable contributions.

    Science.gov (United States)

    2010-04-01

    ... 26 Internal Revenue 17 2010-04-01 2010-04-01 false Calculating comparable contributions. 54.4980G-4 Section 54.4980G-4 Internal Revenue INTERNAL REVENUE SERVICE, DEPARTMENT OF THE TREASURY... calendar year. (c) Model notice. Employers may use the following sample language as a basis in...

  13. IgG4-Related Disease: A Multispecialty Condition

    Directory of Open Access Journals (Sweden)

    Iuri Usêda Santana

    2014-01-01

    Full Text Available IgG4-related disease (IgG4-RD is a recently recognized group of conditions, characterized by tumor-like swelling of involved organs, lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells, variable degrees of fibrosis, and elevated serum IgG4 concentrations. Currently IgG4-RD is recognized as a systemic condition that can affect several organs and tissues. Herein we report the case of a 34-year-old male patient who was admitted to our hospital with diffuse abdominal pain, weight loss, and painful stiffness in his neck. He had a history of tumoral mass of the left maxillary region, right palpebral ptosis with protrusion of the eyeball, and chronic dry cough for about 6 years. Laboratory tests revealed polyclonal hypergammaglobulinemia and increased serum IgG4 levels. Immunohistochemical staining of the maxillary biopsy was compatible with IgG4-RD. He had an excellent response to corticosteroid therapy. This case highlights that IgG4-RD should be included in the differential diagnosis with multisystem diseases.

  14. IgG4-Related Disease Presenting as Isolated Scleritis

    Science.gov (United States)

    Arnon, Ella; Yaakobi, Alona; Cohen, Yuval; Tiosano, Beatrice

    2017-01-01

    A rare case of IgG4-related disease (IgG4-RD) manifesting as nodular scleritis is presented in a 20-year-old female. Patient complained of left eye pain and redness for one week. Ocular examination together with ancillary testing led to the diagnosis of nodular scleritis. Since the patient did not show apparent improvement after one week of systemic steroidal treatment, she underwent a biopsy of the affected area revealing histopathological characteristics of IgG4-RD. Long-term treatment with corticosteroids and a steroid-sparing agent (methotrexate) led to significant improvement in signs and symptoms. This case highlights the significance of IgG4-RD in the differential diagnosis of scleritis and raises the question as to whether various organs affected by IgG4-RD may have different underlying pathophysiological mechanisms in which pathogenic T cells play a role. PMID:28149653

  15. IgG4-Seronegative Autoimmune Pancreatitis and Sclerosing Cholangitis

    Directory of Open Access Journals (Sweden)

    Allon Kahn

    2015-01-01

    Full Text Available IgG4-related disease is a relatively novel clinical entity whose gastrointestinal manifestations include type 1 autoimmune pancreatitis (AIP and IgG4-associated sclerosing cholangitis. The presence of elevated serum IgG4 is suggestive but not essential for the diagnosis of type 1 AIP and is a pervasive feature of the proposed diagnostic criteria. The differential diagnosis of type 1 AIP includes malignant conditions, emphasizing the importance of a deliberate, comprehensive evaluation. Management of patients with a suggestive clinical presentation, but without serum IgG4 elevation, is difficult. Here we present three cases of IgG4-seronegative AIP and sclerosing cholangitis that responded to empiric steroid therapy and discuss approach considerations. These cases demonstrate the value of meticulous application of existing diagnostic algorithms to achieve a clinical diagnosis and avoid surgical intervention.

  16. Immunoglobulin G4-related disease presenting with obstructive jaundice

    Directory of Open Access Journals (Sweden)

    Yuan-Rung Li

    2014-06-01

    Full Text Available A 48-year-old male presented with diffuse abdominal fullness for 1 month and tea-colored urine for 10 days. Abdominal computed tomography/magnetic resonance cholangiopancreatography revealed diffuse enlargement of the pancreas and unusual soft tissue density around the left ureter. Endoscopic retrograde cholangiopancreatography demonstrated lumen narrowing of the distal common bile duct and irregularity of the pancreatic duct. Markedly elevated serum immunoglobulin G4 (IgG4 was also noted. Biopsy of soft tissue from the area surrounding the left ureter identified lymphoplasmacytic infiltration with high concentrations of IgG4-positive plasma cells accompanied by obliterative phlebitis, compatible with IgG4-related disease. The patient was administered steroid therapy and his symptoms improved. Clinicians should be aware of possible IgG4-related disease in a patient presenting with obstructive jaundice and diffuse pancreatic enlargement because glucocorticoid administration can achieve good response.

  17. TREATMENT OF IgG4-RELATED DISEASE

    Directory of Open Access Journals (Sweden)

    E. V. Sokol

    2016-01-01

    Full Text Available IgG4-related disease (IgG4-RD is a fibroinflammatory condition characterized by the occurrence of tumor-like foci in different organs with a unique histological pattern (moirо-like fibrosis, obvious lymphoplasmacytic infiltration with large numbers of IgG4+ plasma cells, and obliterating phlebitis and elevated serum IgG4 levels in the majority of patients. Its first-line therapy is glucocorticoids at a starting dose of 0.6 mg/kg/day (equivalent to prednisolone; however, this treatment entails a great number of adverse events and high recurrence rates. The paper provides a review of today's literature on the treatment of IgG4-RD; particular emphasis is laid on the description of therapy with glucocorticoids and rituximab.

  18. IgG4-Related Disease Presenting as Isolated Scleritis

    Directory of Open Access Journals (Sweden)

    Eran Berkowitz

    2017-01-01

    Full Text Available A rare case of IgG4-related disease (IgG4-RD manifesting as nodular scleritis is presented in a 20-year-old female. Patient complained of left eye pain and redness for one week. Ocular examination together with ancillary testing led to the diagnosis of nodular scleritis. Since the patient did not show apparent improvement after one week of systemic steroidal treatment, she underwent a biopsy of the affected area revealing histopathological characteristics of IgG4-RD. Long-term treatment with corticosteroids and a steroid-sparing agent (methotrexate led to significant improvement in signs and symptoms. This case highlights the significance of IgG4-RD in the differential diagnosis of scleritis and raises the question as to whether various organs affected by IgG4-RD may have different underlying pathophysiological mechanisms in which pathogenic T cells play a role.

  19. G4beamline Particle Tracking in Matter Dominated Beam Lines

    Energy Technology Data Exchange (ETDEWEB)

    T.J. Roberts, K.B. Beard, S. Ahmed, D. Huang, D.M. Kaplan

    2011-03-01

    The G4beamline program is a useful and steadily improving tool to quickly and easily model beam lines and experimental equipment without user programming. It has both graphical and command-line user interfaces. Unlike most accelerator physics codes, it easily handles a wide range of materials and fields, being particularly well suited for the study of muon and neutrino facilities. As it is based on the Geant4 toolkit, G4beamline includes most of what is known about the interactions of particles with matter. We are continuing the development of G4beamline to facilitate its use by a larger set of beam line and accelerator developers. A major new feature is the calculation of space-charge effects. G4beamline is open source and freely available at http://g4beamline.muonsinc.com

  20. IgG4相关性甲状腺疾病%IgG4 Related Thyroid Diseases

    Institute of Scientific and Technical Information of China (English)

    汪洵理; 黄秋实; 朱信心; 杜镇鸿; 陈强; 邓巧莲; 孙映荷; 陈锐; 魏涛

    2013-01-01

    目的 总结IgG4相关性甲状腺病与IgG4关系的研究进展.方法 收集近年来国内外关于IgG4相关性甲状腺病的病理特征及其与IgG4关系的相关文献并作综述.结果 IgG4相关性疾病是一类新近被认识的与IgG4淋巴细胞密切相关的慢性、系统性疾病,可单发,也可累及甲状腺及全身多个器官.在桥本甲状腺炎、里德尔甲状腺炎及甲状腺乳头状癌病灶中均发现了IgG4的表达.结论 IgG4相关性甲状腺病是对甲状腺炎疾病的一种新的认识,对其的了解有助于对甲状腺炎患者提供合理的治疗手段.

  1. IgG4-related sclerosing diseases%IgG4相关的硬化性疾病

    Institute of Scientific and Technical Information of China (English)

    孙希印; 燕丽; 刘萍; 李新功

    2016-01-01

    IgG4-related sclerosing diseases are newly recognized, which are considered to be related to au-toimmunity, including cellular and humoral immune abnormity. IgG4 levels in serum are higher than normal. Histopathological findings include fibrosis with significant inflammatory cells, mainly IgG4(+) plasma cells. Lesions can involve many internal organs. Skin lesions are mostly found in head and neck, presenting with erythema, patches or subcutaneous nodules accompanying itching. Glucocorticoid is effective in the treatment of IgG4-related skin diseases.%IgG4相关硬化性疾病是近年被认识的一种疾病,被认为与自身细胞、体液免疫反应异常有关。患者血清IgG4水平升高,病变器官纤维化,并有显著IgG4阳性浆细胞浸润。病变可以累及许多器官,部分病例伴有皮肤的病变。皮肤病变以头颈部多见,可为红斑、斑片或皮下结节,有痒感。 IgG4相关皮肤疾病糖皮质激素治疗有效。

  2. [IgG4-related kidney disease. Diagnosis and treatment].

    Science.gov (United States)

    Kawano, Mitsuhiro; Mizushima, Ichiro; Yamada, Kazunori; Taniguchi, Yoshinori; Saeki, Takako

    2015-01-01

    IgG4-related disease (IgG4-RD) is a systemic inflammatory disorder that can affect most organs/tissues like sarcoidosis. The kidney is one of the most frequently affected organs. While tubulointerstitial nephritis (TIN) with characteristic imaging findings is the representative lesion of IgG4-related kidney disease (IgG4-RKD), a variety of glomerular lesions, particularly membranous nephropathy, sometimes overlap on TIN. Clinically, either decreased renal function and/or characteristic imaging findings such as multiple low-density lesions on contrast-enhanced computed tomography are typical presenting features. Histologically, plasma cell (PC)-rich TIN accompanied by characteristic fibrosis called storiform fibrosis with dense IgG4-positive PC infiltration is a typical finding. Although a swift response to corticosteroid is a very important feature of IgG4-RKD, in cases with moderately to severely decreased renal function before therapy, only partial recovery of renal function is obtained. This review provides a comprehensive overview of IgG4-RKD from the clinical, laboratory, imaging, and histological aspects and also addresses some of the therapeutic issues concerning it.

  3. [Immunoglobulin G4-associated to multiorganic lymphoproliferative disease].

    Science.gov (United States)

    Bourlon, María T; Chapa, Mónica; Chablé Montero, Fredy; Hernández Calleros, Jorge

    2011-01-01

    We report a case of a woman with lymphoproliferative multiorganic immunoglobulin G4 (IgG4) related disease with extensive involvement showing dacryoadenitis, sialoadenitis, parotiditis, pancreatitis, pneumonitis, lymphadenopathy and immune thrombocytopenic purpura. Serum elevation of acute phase reactant, polyclonal hypergammaglobulinemia, positivity for antinuclear antibodies and rheumatoid factor was found. Hystologically plasma cell infiltration was demonstrated on glandular and lymphatic tissue and immunochemistry was positive for IgG4 in > 30%. Immunosuppressive treatment with steroids and azathioprine was given with an excellent clinical response, the marked radiologic evidence of improvement and the decrease in inflammatory makers that conducted to symptom remission are shown in the text.

  4. IgG4-RELATED DISEASE. CLINICAL NOTES

    Directory of Open Access Journals (Sweden)

    Vladimir Ivanovich Vasilyev

    2013-01-01

    Full Text Available IgG4-related diseases are a new nosological entity that encompasses a few previously known diseases. IgG4-related systemic disease is diagnosed if two or more affected organs are detected. This group of diseases has two similar signs: serological (elevated serum IgG4 subclass concentrations and histological (organ and tissue infiltration from plasmo-cytes secreting IgG4, and eosinophils, and the development of fibrosclerosis and phlebitis obliterans. The paper describes two cases. In one case, a multisystemic disease was observed virtually at its onset whereas in the other this lesion was diagnosed several years after the natural course of the disease.

  5. IgG4-related sclerosing diseases%IgG4相关的硬化性疾病

    Institute of Scientific and Technical Information of China (English)

    黄文斌; 周晓军

    2008-01-01

    IgG4相关的硬化性疾病(IgG4-related sclerosing disease),又称IgG4相关的自身免疫性疾病(IsG4-related autoimmune disease)或高IgG4疾病(hyper-ISG4 disease),是新近认识的一种疾病,其名称尚未统一。该疾病主要累及胰腺,但也可累及胰腺外其他器官,有时胰腺可无病变。ISG4相关的硬化性疾病多与自身免疫有关,且对类固醇激素治疗有效。由于IgG4相关的硬化性疾病国内文献报道相对较少,人们对其尚未引起足够的重视,临床症状缺乏特异性,极易误诊或漏诊。本文主要介绍IgG4相关的硬化性疾病的临床特征、各器官的组织病理学特征、鉴别诊断和治疗。

  6. Immunoglobulin G4-related disease with recurrent obstructive jaundice

    Directory of Open Access Journals (Sweden)

    Yu-Hsiang Chiu

    2016-03-01

    Full Text Available A 51-year-old man was referred to our clinic for recurrent obstructive jaundice and underwent pylorus-preserving pancreaticoduodenectomy for a suspected malignancy. The pathology showed immunoglobulin G4 positive plasma cell infiltrated at the pancreas and the gallbladder. We discuss the cost-effectiveness of serum immunoglobulin G4 level prior to arranging for a pancreaticoduodenectomy, which would reduce the possibility of surgical complications as well as costs.

  7. g4re: Harnessing GCC to Reverse Engineer C++ Applications

    OpenAIRE

    Kraft, Nicholas A.; Malloy, Brian A.; Power, James F.

    2005-01-01

    In this paper, we describe g4re, our tool chain that exploits GENERIC, an intermediate format incorporated into the gcc C++ compiler, to facilitate analysis of real C++ applications. The gcc GENERIC representation is available through a file generated for each translation unit (TU), and g4re reads each TU file and constructs a corresponding Abstract Semantic Graph (ASG). Since TU files can be prohibitively large, ranging from 11 megabytes for a "hello world" program, t...

  8. Localized IgG4-related Cholecystitis Mimicking Gallbladder Cancer.

    Science.gov (United States)

    Inoue, Tadahisa; Okumura, Fumihiro; Mizushima, Takashi; Nishie, Hirotada; Iwasaki, Hiroyasu; Anbe, Kaiki; Ozeki, Takanori; Kachi, Kenta; Fukusada, Shigeki; Suzuki, Yuta; Watanabe, Kazuko; Sano, Hitoshi

    2015-01-01

    We encountered a case of localized IgG4-cholecystitis mimicking gallbladder cancer with focal/segmental type1 autoimmune pancreatitis (AIP). In this case, we were unable to exclude a diagnosis of gallbladder cancer and thus performed radical cholecystectomy. Type1 AIP is often associated with gallbladder lesions, accompanied by generally diffuse, circumferential thickening of the gallbladder wall. Although localized IgG4-related cholecystitis is extremely rare, differentiating this condition from gallbladder cancer is often very difficult.

  9. IgG4-Related Disease in a Urachal Tumor

    Directory of Open Access Journals (Sweden)

    Travis W. Dum

    2014-01-01

    Full Text Available IgG4-related disease is a newly recognized fibroinflammatory disorder that has the ability to affect nearly every organ system. It is characterized by tumefactive lesions and fibrosis and closely mimics neoplasms. Only one case of IgG4-related bladder mass has been reported in the literature, but there are no reports of IgG4-related disease in a urachal mass. Herein, we report a 26-year-old male who initially presented with symptoms of recurrent UTI. Work-up revealed a 6 cm urachal tumor, a 1.4 cm pulmonary lesion, and mediastinal lymphadenopathy; all metabolically active on PET scan and suspicious for urachal adenocarcinoma. Lung lesion fine needle aspiration and TURBT pathology revealed inflammation but no evidence of malignancy. The patient underwent a partial cystectomy and umbilectomy with pathology demonstrating dense plasmacytic cells, a high rate of immunohistochemistry staining positive for IgG4 plasma cells, a storiform pattern of fibrosis, and an obliterative phlebitis. Furthermore, the patient had an elevated serum IgG4 level of 227 mg/dL (range 2.4–121 mg/dL. IgG4-related disease is a newly recognized fibroinflammatory disorder that can mimic neoplastic processes and a high index of suspicion and accurate tissue pathology is necessary for an accurate diagnosis.

  10. Immunoglobulin G4 related lung diseases%IgG4相关性肺疾病

    Institute of Scientific and Technical Information of China (English)

    施举红

    2013-01-01

    IgG4相关性疾病是一组临床病理综合征,病因尚不清.IgG4相关性肺疾病在组织病理学表现为IgG4阳性浆细胞及淋巴细胞浸润,伴有间质纤维组织增生.在没有系统器官受累的情况下,IgG4相关性肺疾病在影像学上与非特异性间质性肺炎(NSIP)及肺部肿瘤很难鉴别,因此须依靠病理表现作出确定诊断.此类疾病对糖皮质激素治疗反应良好.

  11. A case of non-lacrimal immunoglobulin G4 (IgG4)-related orbital disease with mastitis.

    Science.gov (United States)

    Farooq, Tahir Ali; Mudhar, Hardeep; Sandramouli, S

    2016-01-01

    IgG4-related orbital disease is a recognised cause for orbital inflammation. As its awareness increases and diagnostic accuracy improves there will be an increased number of cases being identified. This unique case demonstrates for the first time, with histological evidence, a case of a non-lacrimal IgG4-related orbital disease with concurrent IgG4-related mastitis. We describe a 47 year old who presented with a supraorbital swelling and mass. This was initially successfully treated with oral steroids and was later excised on recurrence. Immunohistochemical and blood serum analysis confirmed IgG4-related orbital disease. On systemic enquiry she was found to have a mass of the breast, which was shown to be IgG4-related mastitis. She is currently asymptomatic with no sign of recurrence and is under long-term surveillance. This case highlights the importance of systemic work up in patients presenting with orbital foci of IgG4 disease.

  12. IgG4相关性皮肤病%IgG4-related skin disease

    Institute of Scientific and Technical Information of China (English)

    林尽染; 陈连军; 张乔安; 徐金华

    2015-01-01

    报告IgG4相关性皮肤病(IgG4-RSD)1例.患者男,25岁.躯干、面颈部瘙痒性红色至褐色丘疹、斑块及结节6年.皮损组织病理示真皮内胶原纤维增生,细血管周围小团块状或结节状淋巴细胞、浆细胞浸润.免疫组化示浆细胞表达CD38、CD138、CD19、K--轻链、λ-轻链、IgG、IgG4均阳性,IgG4+/IgG+浆细胞比值>40%,每高倍视野下IgG4+浆细胞>10个.该文对IgG4-RSD的诊断、鉴别诊断和治疗进行讨论.

  13. [Immunoglobulin-G4-related disease: a challenging diagnosis].

    Science.gov (United States)

    Banc, Ana; Stan, Cristina

    2014-01-01

    Immunglobulin G4 (IgG4)-related disease was recently described and represents a systemic lymphoproliferative disorder. The orbital form of the disease manifests as chronic lid swelling and proptosis. Visual disturbances may occur due to apical orbital lesions. A 65-year old pacient presents with the impossibility of maintaining his right eye open due to a progressive swelling of the upper lid. The general clinical examination shows adenopathy at 3 lymph node stations of the head. On ophthalmologic examination, a large tumor of the upper lid is observed in the right eye and proptosis and central retinal vein occlusion are noted in the left eye. The serum levels of the IgG are very high. A lymph node biopsy was performed. The differential diagnosis between the IgG4-related orbital disease and non-Hodgkin lymphoma is discussed. CONCLUSION The diagnosis criteria for IgG4-related disease are both the high serum levels of IgG4 and the specific immunohistochemistry stains.

  14. Lipid-modified G4-decoy oligonucleotide anchored to nanoparticles

    DEFF Research Database (Denmark)

    Cogoi, S; Jakobsen, U; Pedersen, E B

    2016-01-01

    KRAS is mutated in >90% of pancreatic ductal adenocarcinomas. As its inactivation leads to tumour regression, mutant KRAS is considered an attractive target for anticancer drugs. In this study we report a new delivery strategy for a G4-decoy oligonucleotide that sequesters MAZ, a transcription...... factor essential for KRAS transcription. It is based on the use of palmitoyl-oleyl-phosphatidylcholine (POPC) liposomes functionalized with lipid-modified G4-decoy oligonucleotides and a lipid-modified cell penetrating TAT peptide. The potency of the strategy in pancreatic cancer cells is demonstrated...

  15. Acute Painful Ptosis Secondary to IgG4 Dacryoadenitis

    Directory of Open Access Journals (Sweden)

    Rumana Hussain

    2016-02-01

    Full Text Available A 48-year-old lorry driver presented with 3 weeks of blurred vision, pain and diplopia. There was a right upper lid ptosis with some restriction of eye movements. A CT revealed an enlarged lacrimal gland and lacrimal gland biopsy showed IgG4-positive plasma cells. The patient responded to oral prednisolone and fully recovered. As a condition which mimics a number of diseases, an IgG4-related disease presents a diagnostic challenge and ought to be considered in both acute and chronic presentations.

  16. CORE

    DEFF Research Database (Denmark)

    Krigslund, Jeppe; Hansen, Jonas; Hundebøll, Martin

    2013-01-01

    different flows. Instead of maintaining these approaches separate, we propose a protocol (CORE) that brings together these coding mechanisms. Our protocol uses random linear network coding (RLNC) for intra- session coding but allows nodes in the network to setup inter- session coding regions where flows...... intersect. Routes for unicast sessions are agnostic to other sessions and setup beforehand, CORE will then discover and exploit intersecting routes. Our approach allows the inter-session regions to leverage RLNC to compensate for losses or failures in the overhearing or transmitting process. Thus, we...... increase the benefits of XORing by exploiting the underlying RLNC structure of individual flows. This goes beyond providing additional reliability to each individual session and beyond exploiting coding opportunistically. Our numerical results show that CORE outperforms both forwarding and COPE...

  17. CORE

    DEFF Research Database (Denmark)

    Krigslund, Jeppe; Hansen, Jonas; Hundebøll, Martin

    2013-01-01

    different flows. Instead of maintaining these approaches separate, we propose a protocol (CORE) that brings together these coding mechanisms. Our protocol uses random linear network coding (RLNC) for intra- session coding but allows nodes in the network to setup inter- session coding regions where flows...... intersect. Routes for unicast sessions are agnostic to other sessions and setup beforehand, CORE will then discover and exploit intersecting routes. Our approach allows the inter-session regions to leverage RLNC to compensate for losses or failures in the overhearing or transmitting process. Thus, we...... increase the benefits of XORing by exploiting the underlying RLNC structure of individual flows. This goes beyond providing additional reliability to each individual session and beyond exploiting coding opportunistically. Our numerical results show that CORE outperforms both forwarding and COPE...

  18. [Raise awareness of IgG4 relative ocular disease].

    Science.gov (United States)

    Wei, Shihui; Li, Hongyang

    2015-12-01

    Purpose IgG4-related ocular disease is a chronic systemic disease with lymphocyte abnormal. The lacrimal glands, extraocular muscles and infraorbital nerve were often involved which was often the first symptom of systemic disease. While ophthalmologists did not know this disease well. They usually misdiagnosed it as idiopathic inflammatory pseudotumor, thyroid-associated ophthalmopathy etc, which resulted in delayed treatments. Here pathogenesis, clinical features and treatment methods of IgG4-relative ocular disease were described in order to improve awareness of this ocular disease, reduce clinical misdiagnosis, improve disease prognosis and standardized treatment. As the incidence of this disease increased in recent years, it is very necessary to improve awareness of the disease for ophthalmologists.

  19. Data of evolutionary structure change: 1JKCA-2G4NF [Confc[Archive

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  20. Data of evolutionary structure change: 1CKCA-2G4NF [Confc[Archive

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  11. Data of evolutionary structure change: 1IWTA-2G4NF [Confc[Archive

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  12. Data of evolutionary structure change: 1B5WA-2G4NF [Confc[Archive

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  13. Data of evolutionary structure change: 1B7NA-2G4NF [Confc[Archive

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  14. Data of evolutionary structure change: 1JJ3B-2G4NF [Confc[Archive

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  15. Data of evolutionary structure change: 1JKDA-2G4NF [Confc[Archive

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  16. Data of evolutionary structure change: 1EQEA-2G4NF [Confc[Archive

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  17. Data of evolutionary structure change: 1CJ8A-2G4NF [Confc[Archive

    Lifescience Database Archive (English)

    Full Text Available 1CJ8A-2G4NF 1CJ8 2G4N A F KVFERCELARTLKRLGMDGYRGISLANWMCLAKWESGYN... HHHH -GGGG -- EVID>EVID> 0 2G4N F 2G4NF IVQNN-...n> 0.7294750213623047 1.3636130094528198 EVID> 1 2G4N F 2G4NF

  18. Immunogenetics of IgG4-Related AIP.

    Science.gov (United States)

    Ota, Masao; Umemura, Takeji; Kawa, Shigeyuki

    2017-01-01

    Autoimmune pancreatitis (AIP) is a unique form of chronic pancreatitis characterized by high serum IgG4 concentration and a variety of complicating extra-pancreatic lesions. AIP has the features of a complex disease that is caused by multifactorial genes. However, the genetic factors underlying AIP have not been elucidated conclusively. Association studies by the candidate-gene approach and genome-wide association studies (GWAS) have revealed several susceptibility genes for AIP, including HLA DRB1*04:05-DQB1*04:01, FCRL3, CTLA4, and KCNA3, albeit in small-scale analyses. Thus, GWAS of large sample sizes and multinational collaborative meta-analyses are needed to identify the precise genetic variants that are associated with AIP onset. Systems genetics approaches that integrate DNA sequencing, expression quantitative trait locus (eQTL) mapping, proteomics, and metabolomics will also be useful in clarifying the pathogenesis of AIP.

  19. Nucleolin inhibits G4 oligonucleotide unwinding by Werner helicase.

    Directory of Open Access Journals (Sweden)

    Fred E Indig

    Full Text Available BACKGROUND: The Werner protein (WRNp, a member of the RecQ helicase family, is strongly associated with the nucleolus, as is nucleolin (NCL, an important nucleolar constituent protein. Both WRNp and NCL respond to the effects of DNA damaging agents. Therefore, we have investigated if these nuclear proteins interact and if this interaction has a possible functional significance in DNA damage repair. METHODOLOGY/PRINCIPAL FINDINGS: Here we report that WRNp interacts with the RNA-binding protein, NCL, based on immunoprecipitation, immunofluorescent co-localization in live and fixed cells, and direct binding of purified WRNp to nucleolin. We also map the binding region to the C-terminal domains of both proteins. Furthermore, treatment of U2OS cells with 15 µM of the Topoisomerase I inhibitor, camptothecin, causes the dissociation of the nucleolin-Werner complex in the nucleolus, followed by partial re-association in the nucleoplasm. Other DNA damaging agents, such as hydroxyurea, Mitomycin C, and aphidicolin do not have these effects. Nucleolin or its C-terminal fragment affected the helicase, but not the exonuclease activity of WRNp, by inhibiting WRN unwinding of G4 tetraplex DNA structures, as seen in activity assays and electrophoretic mobility shift assays (EMSA. CONCLUSIONS/SIGNIFICANCE: These data suggest that nucleolin may regulate G4 DNA unwinding by WRNp, possibly in response to certain DNA damaging agents. We postulate that the NCL-WRNp complex may contain an inactive form of WRNp, which is released from the nucleolus upon DNA damage. Then, when required, WRNp is released from inhibition and can participate in the DNA repair processes.

  20. Distribution of serum immunoglobulin G4 levels in Hashimoto's thyroiditis and clinical features of Hashimoto's thyroiditis with elevated serum immunoglobulin G4 levels.

    Science.gov (United States)

    Takeshima, Ken; Ariyasu, Hiroyuki; Inaba, Hidefumi; Inagaki, Yuko; Yamaoka, Hiroyuki; Furukawa, Yasushi; Doi, Asako; Furuta, Hiroto; Nishi, Masahiro; Akamizu, Takashi

    2015-01-01

    Immunoglobulin G4-related disease (IgG4-RD) is characterized by elevated serum IgG4 levels, IgG4-positive plasmacytes, and lymphocyte infiltration into multiple organs. IgG4 thyroiditis is a subset of patients with Hashimoto's thyroiditis (HT) who exhibited histopathological features of IgG4-RD; its source of serum IgG4 is suggested to be the thyroid gland. Although a relationship between IgG4-RD and IgG4 thyroiditis has been reported, the meaning of serum IgG4 in HT is uncertain. In this report, we prospectively evaluated serum IgG4 levels and clinical features of patients with HT. A total of 149 patients with HT were prospectively recruited into this study. According to the comprehensive diagnostic criteria of IgG4-RD, patients were divided into two groups: elevated IgG4 (>135 mg/dL) and non-elevated IgG4 (≤135 mg/dL). Median serum IgG4 levels of HT patients were 32.0 mg/dL (interquartile range, 20.0-65.0), with a unimodal non-normal distribution. Six patients (4.0%) had elevated serum IgG4 levels above 135 mg/dL. The elevated IgG4 group was older and exhibited enlarged hypoechoic areas in the thyroid gland, as revealed by ultrasonography, relative to the non-elevated IgG4 group. Levothyroxine (L-T4) replacement doses and titers of anti-thyroid antibodies did not differ significantly between the two groups. Two out of six HT patients with elevated serum IgG4 levels had extra-thyroid organ involvement as seen in IgG4-RD. In conclusion, HT patients with elevated serum IgG4 levels shared clinical features with both IgG4-RD and IgG4 thyroiditis. Longer follow-up periods and histopathological assessments are needed to further understand the meaning of elevated serum IgG4 levels in HT.

  1. Value of serum IgG4 in the diagnosis of IgG4-related disease and in differentiation from rheumatic diseases and other diseases.

    Science.gov (United States)

    Yamamoto, Motohisa; Tabeya, Tetsuya; Naishiro, Yasuyoshi; Yajima, Hidetaka; Ishigami, Keisuke; Shimizu, Yui; Obara, Mikiko; Suzuki, Chisako; Yamashita, Kentaro; Yamamoto, Hiroyuki; Hayashi, Toshiaki; Sasaki, Shigeru; Sugaya, Toshiaki; Ishida, Tadao; Takano, Ken-Ichi; Himi, Tetsuo; Suzuki, Yasuo; Nishimoto, Norihiro; Honda, Saho; Takahashi, Hiroki; Imai, Kohzoh; Shinomura, Yasuhisa

    2012-06-01

    IgG4-related disease (IgG4-RD) is a novel disease entity that includes Mikulicz's disease, autoimmune pancreatitis (AIP), and many other conditions. It is characterized by elevated serum IgG4 levels and abundant IgG4-bearing plasmacyte infiltration of involved organs. We postulated that high levels of serum IgG4 would comprise a useful diagnostic tool, but little information is available about IgG4 in conditions other than IgG4-RD, including rheumatic diseases. Several reports have described cutoff values for serum IgG4 when diagnosing IgG4-RD, but these studies mostly used 135 mg/dL in AIP to differentiate from pancreatic cancer instead of rheumatic and other common diseases. There is no evidence for a cutoff serum IgG4 level of 135 mg/dL for rheumatic diseases and common diseases that are often complicated with rheumatic diseases. The aim of this work was to re-evaluate the usual cutoff serum IgG4 value in AIP (135 mg/dL) that is used to diagnose whole IgG4-RD in the setting of a rheumatic clinic by measuring serum IgG4 levels in IgG4-RD and various disorders. We therefore constructed ROC curves of serum IgG4 levels in 418 patients who attended Sapporo Medical University Hospital due to IgG4-RD and various rheumatic and common disorders. The optimal cut-off value of serum IgG4 for a diagnosis of IgG4-RD was 144 mg/dL, and the sensitivity and specificity were 95.10 and 90.76%, respectively. Levels of serum IgG4 were elevated in IgG4-RD, Churg-Strauss syndrome, multicentric Castleman's disease, eosinophilic disorders, and in some patients with rheumatoid arthritis, systemic sclerosis, chronic hepatitis, and liver cirrhosis. The usual cut-off value of 135 mg/dL in AIP is useful for diagnosing whole IgG4-RD, but high levels of serum IgG4 are sometimes observed in not only IgG4-RD but also other rheumatic and common diseases.

  2. Increased IgG4-Positive Plasma Cells in Granulomatosis with Polyangiitis: A Diagnostic Pitfall of IgG4-Related Disease

    Directory of Open Access Journals (Sweden)

    Sing Yun Chang

    2012-01-01

    Full Text Available Granulomatosis with polyangiitis (Wegener’s (GPA may mimic IgG4-related disease (IgG4-RD on histologic examination of some biopsies, especially those from head and neck sites. IgG4 immunostain is often performed in this context for differential diagnosis with IgG4-RD. However, the prevalence of IgG4+ cells in GPA has not been explored. We examined the IgG4+ cells in 26 cases confirmed as GPA by a thorough clinical and pathologic assessment. Twenty-six biopsies consisted of 14 sinonasal/oral cavity/nasopharynx, 7 orbit/periorbital, 3 lung/pleura, 1 iliac fossa/kidney, and 1 dura specimens. Eight of 26 (31% biopsies revealed increased IgG4+ cells (>30/HPF and >40% in IgG4+/IgG+ ratio. The IgG4+ cells and IgG4+/IgG+ ratio ranged 37–137/hpf and 44–83%, respectively. Eight biopsies with increased IgG4+ cells were from sinonasal (n=4 or orbital/periorbital (n=4 sites. In conclusion, increased IgG4+ cells are not uncommonly seen in sinonasal or orbital/periorbital biopsies of GPA, which could pose as a diagnostic pitfall.

  3. 血清IgG4对IgG4相关性疾病的诊断价值及该病的眼部表现%Value of serum IgG4 for the diagnosis of IgG4-related disease and the ocular manifestations of IgG4-related disease

    Institute of Scientific and Technical Information of China (English)

    杨慧敏; 王晓栋; 叶霜; 华静; 王琴; 李劲

    2015-01-01

    目的 评价血清IgG4浓度对IgG4相关性疾病的鉴别诊断价值,并探讨IgG4相关性疾病的眼部表现.方法 收集IgG4相关性疾病患者29例,血清IgG4浓度升高的其他疾病患者161例(其中自身免疫性肝病47例,急性胰腺炎15例,原发性肾小球病及肾功能不全61例,炎症性肠病38例),正常对照190例;以免疫散射比浊法测定血清IgG4和IgG浓度,比较各疾病组患者的检测结果.以所有患者的血清IgG4浓度绘制受试者工作特征(ROC)曲线,评价血清IgG4浓度的诊断价值,并确定其诊断IgG4相关性疾病的最佳临界值.同时对IgG4相关性疾病患者进行眼科检查,评估其眼部表现.结果 IgG4相关性疾病组血清IgG4浓度与原发性肾小球病及肾功能不全组、炎症性肠病组、自身免疫性肝病组、急性胰腺炎组比较,差异有统计学意义(P<0.01).经ROC曲线分析,当IgG4为2.37 g/L时,Youden指数最大,该诊断界值的敏感度为93.1%,特异度为84.3%,曲线下面积为0.939.29例IgG4相关性疾病患者中,2例有眼眶占位性病变,其余患者眼部表现无特异性.结论 血清IgG4浓度对于诊断IgG4相关性疾病具有较高的敏感度和特异度,但仅能作为诊断IgG4相关性疾病的依据之一,提示IgG4相关性疾病的可能;该病眼部表现无特异性.

  4. IgG4相关性淋巴结病与淋巴瘤%IgG4-related lymphadenopathy and lymphoma

    Institute of Scientific and Technical Information of China (English)

    王文生

    2015-01-01

    IgG4在IgG中是最小的亚群,近年来一种并不多见的以淋巴结及一系列结外部位出现硬化性损害伴随受累部位IgG4亚型浆细胞增多、血清IgG4水平升高以及经常出现自身抗体等表现为特征的系统性疾病经常被报道,该病现在一般被称为IgG4相关性疾病(IgG4-RD),对糖皮质激素治疗反应良好;一些研究者认为IgG4-RD有可能最终发展为淋巴瘤。本综述阐述了与IgG4-RD相关的淋巴结病变,探讨了病变淋巴结中 IgG4阳性浆细胞增多的意义,同时结合文献讨论了与IgG4-RD相关的淋巴瘤以及IgG4-RD患者发生淋巴瘤风险。%IgG4 is one of the IgG subtypes, but it is the most uncommon. In recent years an relatively rare systemic disorder characterized by some manifestations that may include the sclerosing lesions in a wide variety of extranodal sites, lymphadenopathy, appearance of increased numbers of plasma cells of IgG4 subtype in affected area, elevated serum IgG4 and autoantibodies may positive has been described. This disease has a good response to steroid therapy and now known as IgG4-related disease (IgG4-RD). Some researchers believe that IgG4-RD may eventually develop to lymphoma. This review described lymphadenopathy associated with the IgG4-RD, discusseed the significance of increased IgG4+ plasma cells in the isolated lymphadenopathy. It also discussed the IgG4-RD related lymphoma and the risk of progression to lymphoma in IgG4-RD patients.

  5. IgG4-related epididymo-orchitis associated with bladder cancer: possible involvement of BAFF/BAFF-R interaction in IgG4-related urogenital disease.

    Science.gov (United States)

    Migita, Kiyoshi; Miyashita, Taiichiro; Mizuno, Aya; Jiuchi, Yuka; Ito, Masahiro; Matsuo, Manabu; Izumi, Yasumori; Takeoka, Atsushi; Nishino, Ayako; Hayashi, Mikio

    2014-01-01

    We describe herein a patient who presented with immunoglobulin G4-related disease (IgG4-RD) involving the testis and prostate as well as the submandibular glands. Massive infiltration of IgG4-expressing plasma cells was observed in testis and prostate tissues. Serum concentrations of B cell activating factor belonging to the tumor necrosis factor family (BAFF) were elevated in parallel with serum IgG4 concentrations, and infiltration of BAFF-receptor (BAFF-R)-expressing B cells and BAFF-expressing lymphoid cells was observed around the ectopic lymphoid foci in the affected urogenital tissues. To date, testicular involvement in a patient diagnosed with IgG4-RD had not been reported, making this the first reported case of IgG4-related epididymo-orchitis. These findings suggest that the immune mechanism underlying ectopic lymphoneogenesis in IgG4-RD may involve enhanced BAFF/BAFF-R interactions among lymphoid cells.

  6. Data of evolutionary structure change: 1FKQA-2G4QA [Confc[Archive

    Lifescience Database Archive (English)

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  7. The Digestive Involvement of IgG4-related Disease%消化内镜与IgG4相关消化系统疾病

    Institute of Scientific and Technical Information of China (English)

    杨爱明; 于航

    2016-01-01

    IgG4相关疾病(IgG4-RD)是一种慢性炎症硬化性疾病,特点为大量IgG4阳性浆细胞浸润、致密纤维化、闭塞性静脉炎及血清IgG4水平升高等.IgG4-RD可以累积于多种脏器,其常见的消化系统受累表现包括自身免疫性胰腺炎、IgG4相关硬化性胆管炎、IgG4相关自身免疫性肝炎、IgG4相关硬化性胆囊炎、自身免疫性胰腺炎相关性胃炎等.消化内镜,包括超声内镜、ERCP、ERCP结合IDUS、EUS-FNA在IgG4相关消化系统疾病的诊断,尤其是胰腺、胆道系统方面都有重要作用.IgG4-RD对激素治疗敏感,但容易复发,对疾病复发的患者可再次激素治疗或联合免疫调节治疗.

  8. Renal manifestations of IgG4-related systemic disease%IgG4相关系统性疾病的肾脏损害

    Institute of Scientific and Technical Information of China (English)

    黄倩

    2012-01-01

    IgG4相关系统性疾病(IgG4-related systemic disease,IgG4-RSD)是一组最近认识的临床综合征,可累及多个器官,胰腺最常受累,主要表现为血清IgG4升高,受累组织弥漫IgG4阳性浆细胞浸润.本文主要介绍IgG4-RSD累及肾脏时的临床表现、影像学、实验室检查、病理特点及治疗情况.%IgG4-related systemic disease represents a recently recognized group of multi-organ diseases characterized by a high level of serum IgG4 and dense infiltration of IgG4-positive cells into multiple organs. The most commonly involved organ was pancreas. In this article,the relevant literatures about the renal involvements of IgG4-related systemic disease will be reviewed.

  9. A small subgroup of Hashimoto's thyroiditis is associated with IgG4-related disease.

    Science.gov (United States)

    Jokisch, Friedrich; Kleinlein, Irene; Haller, Bernhard; Seehaus, Tanja; Fuerst, Heinrich; Kremer, Marcus

    2016-03-01

    IgG4-related disease is a newly identified syndrome characterized by high serum IgG4 levels and increased IgG4-positive plasma cells in involved organs. The incidence of IgG4-related thyroiditis in the Caucasian population of Europe is unknown. We investigated formalin-fixed thyroid gland samples of 216 patients (191 Hashimoto's thyroiditis, 5 Riedel's thyroiditis, and 20 goiters, as controls), morphologically, and immunohistochemically. Cases were divided into two groups: IgG4-related Hashimoto's thyroiditis (24 cases) together with Riedel thyroiditis (1 case) and 171 non-IgG4-related thyroiditis. Compared to the non-IgG4-related cases, IgG4-related thyroiditis showed a higher IgG4/IgG ratio (0.6 vs. 0.1, p Hashimoto's thyroiditis was diagnosed in 23 of the 24 IgG4-related cases (96 %) and in 13 of 167 (18 %, p > 0.001) non-IgG4-related cases. The single case of IgG4-related Riedel's thyroiditis also showed a higher median IgG4 plasma cell count (56.3 vs. 14.3) and a higher IgG4/IgG ratio (0.5 vs. 0.2) than the four cases of non-IgG4-related Riedel's thyroiditis. Our data suggests the incidence of IgG4-related disease (IgG4-RD) of the thyroid gland in Europe is considerably lower than that observed in other studies. A significant elevation of IgG4-positive plasma cells was only found in a small group of Hashimoto's thyroiditis and then accompanied by intense fibrosis, indicating an association with IgG4-RD. Morphologically, IgG4-RD of the thyroid gland differs from that in other organ systems, exhibiting a dense fibrosis without intense eosinophilia or obliterative phlebitis.

  10. Rapid Industrial Prototyping and SoC Design of 3G/4G Wireless Systems Using an HLS Methodology

    OpenAIRE

    Andres Takach; Cavallaro, Joseph R.; Dennis McCain; Yuanbin Guo

    2006-01-01

    Many very-high-complexity signal processing algorithms are required in future wireless systems, giving tremendous challenges to real-time implementations. In this paper, we present our industrial rapid prototyping experiences on 3G/4G wireless systems using advanced signal processing algorithms in MIMO-CDMA and MIMO-OFDM systems. Core system design issues are studied and advanced receiver algorithms suitable for implementation are proposed for synchronization, MIMO equalization, and detectio...

  11. Are Classification Criteria for IgG4-RD Now Possible? The Concept of IgG4-Related Disease and Proposal of Comprehensive Diagnostic Criteria in Japan

    Directory of Open Access Journals (Sweden)

    Kazuichi Okazaki

    2012-01-01

    Full Text Available Recent studies suggest simultaneous or metachronous lesions in multiorgans characterized by elevated serum levels of IgG4 and abundant infiltration of IgG4-positive plasma cells with various degrees of fibrosis. Two Japanese research committees for IgG4-RD, one from fibrosclerosis (Okazaki team and the other from lymph proliferation (Umehara team supported by the “Research Program for Intractable Disease” of the Ministry of Health, Labor, and Welfare of Japan, have agreed with the unified nomenclature as “IgG4-RD” and proposed the comprehensive diagnostic criteria (CDC for IgG4-RD. Validation of the CDC demonstrated satisfactory sensitivity for the practical use of general physicians and nonspecialists but low sensitivity in the organs to be difficult in taking biopsy specimens such as type1 autoimmune pancreatitis (IgG4-related AIP, compared with IgG4-related sialadenitis/dacryoadenitis (Mikulicz's disease and IgG4-related kidney disease. Although the diagnostic criteria covering all IgG4-RD are hard to be established, combination with the CDC and organ-specific diagnostic criteria should improve sensitivity.

  12. Clinicopathological features of IgG4-related sialadenitis%IgG4相关性涎腺炎的临床病理特点

    Institute of Scientific and Technical Information of China (English)

    葛莉

    2013-01-01

    Immunoglobubin G4-related disease (IgG4-RD) is an emerging disease with unknown etiology and multiorgan involvement.IgG4-related sialadenitis is easily to be misdiagnosed as Sj(o)gren's syndrome,chronic nonspecific sialadenitis,or lymphoma.Thus many patients cannot be treated timely and even undergo many unnecessary surgery.It is important to improve our knowledge on IgG4-related sialadenitis.In this review,we summarized the clinicopathological characteristics of IgG4-related sialadenitis.%IgG4相关性疾病作为一种全身系统性疾,可累及多个器官及组织.发生于涎腺的IgG4相关性涎腺炎容易与干燥综合征、慢性非特异性涎腺炎、淋巴瘤等多种疾病混淆,延误治疗并给患者带来诸多不必要的手术负担.因此有必要提高对IgG4相关性涎腺炎的认识.本文就IgG4相关性涎腺炎的临床及病理组织学等特点进行综述.

  13. IgG4相关性疾病的发病机制及进展%Progress in pathogenesis of IgG4-related disease

    Institute of Scientific and Technical Information of China (English)

    苏玉莹; 王晨琼; 董凌莉

    2014-01-01

    IgG4-related disease, a newly recognized systemic entity, is characterized by elevated serum levels of IgG4 and a dense lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells in involved tissues. Its complicated clinical manifestation and multidiscipline involvement make it easy to be misdiagnosed or miss diagnosed. In order to better understand this disease, this review elaborated on update in pathogenesis of IgG4-related disease including genetics, infection and molecular mimicry, immunology and so on.%IgG4相关性疾病是一种多器官、多系统受累的新被认识的疾病,其主要特征为:血清IgG4水平升高和病变组织IgG4+浆细胞浸润。IgG4相关性疾病临床表现复杂多样,可涉及多个学科,临床易漏诊和误诊。本篇综述将从基因遗传、感染和分子模拟、免疫学角度等多方面阐述IgG4相关性疾病的发病机制,以加深临床医师对该病的了解。

  14. The research progress of IgG4-related kidney disease%IgG4相关性肾病研究进展

    Institute of Scientific and Technical Information of China (English)

    李正东; 杜晓刚

    2014-01-01

    IgG4-related kidney diseases is an autoimmune disease that was found in recent years, which can present with acute or chronic renal insufficiency and proteinuria and high level of serum IgG4. Iconography examination always shows a renal organic lesions. Tubulointerstitial nephritis with dense infiltration of IgG4-positive plasma cells is the classic pathological feature. This disease alway responds to steroid therapy with a good effect. This review will focus on the progress of clinical diagnose and treatment for IgG4-related kidney diseases.%IgG4相关性肾病(IgG4-RKD)是近年来发现的一种自身免疫性疾病,多表现为急性或慢性肾功能不全、蛋白尿,血清IgG4升高,影像学上可见肾实质损害,组织病理表现为小管间质性肾炎,以间质大量 IgG4阳性浆细胞浸润为主要特征,对糖皮质激素治疗反应较好。本文将对IgG4-RKD的临床诊治进展作一综述。

  15. Diagnosis and treatment advances of IgG4-related disease%IgG4相关性疾病的诊治及进展

    Institute of Scientific and Technical Information of China (English)

    高丽霞

    2016-01-01

    IgG4相关性疾病(IgG4 related disease,IgG4-RD)是一类病因不明的慢性系统性自身免疫性疾病.IgG4-RD临床谱广泛,常累及全身多部位腺体、腹膜后组织、肾脏、垂体及淋巴结.IgG4-RD的主要临床特征是受累器官肿胀、纤维化和硬化,血清IgG4水平明显升高,受累组织和器官中大量淋巴细胞和IgG4阳性的浆细胞浸润.IgG4-RD的确诊主要依赖组织病理学活检,该疾病的组织病理学特征为:大量淋巴细胞和浆细胞浸润、组织席纹状纤维化和硬化以及闭塞性静脉炎.糖皮质激素是诱导缓解的首选药物,有些患者需要联合免疫抑制剂或生物制剂.%IgG4 related disease (IgG4-RD)is a kind of chronic systemic autoimmune disease,whose etiology is unknown.The clinical spectrum of IgG4-RD is wide. The disease always involves systemic glandular organs, retroperitoneum,kidney,pituitary gland and lymph nodes.IgG4-RD is characterized by swelling and fibrosclerosing change of affected organs,elevated serum IgG4 level,a large number of lymphocytes and IgG4 positive plasma cells infiltration in involved organ and tissue.The diagnosis is confirmed by biopsy,the histopathological features include:dense lymphocytes and plasmacytes infiltration,storiform fibrosis and sclerosis,obliterative phlebitis.Glucocorticoids are the first-line agent for remission induction,some IgG4-RD patients require the use of immunosuppressive drugs or biological agents.

  16. IgG4相关唾液腺炎研究热点概述%Research hotspot in IgG4-related sialadenitis

    Institute of Scientific and Technical Information of China (English)

    王琼; 平飞云; 潘恒标

    2016-01-01

    IgG4相关疾病累及唾液腺称为IgG4相关唾液腺炎,中老年好发,男女性均可发病.IgG4相关唾液腺炎以唾液腺无痛性肿胀、血清IgG4升高、组织内大量特征性IgG4阳性浆细胞浸润伴纤维化为主要特点,糖皮质激素治疗反应良好.通过复习国内外文献,着重于该病近年来的研究热点阐述并分析多种免疫细胞和细胞因子、上皮间质转化参与的致病过程、正确看待血清IgG4浓度升高和组织IgG4阳性浆细胞浸润的临床病理意义及其诊断价值、与唾液腺癌和淋巴瘤的相关性、正电子发射型计算机断层摄影(positron emission tomography,PET)-CT的应用、唇腺活检对病理诊断的意义及治疗预后等问题.%IgG4-related disease is a novel clinical entity which can affect single or multiple organs.IgG4-related sialadenitis is referred to the salivary gland involvement of IgG4-related disease, with or without other organ involvement.IgG4-related sialadenitis is characterized by painless swelling or enlargement of salivary glands, high serum IgG4 level, abundant IgG4+ plasma cells infiltration with fibrosis histologically, and good response to glucocorticoids.With review of related articles, highlight and provide an overview of the most recent and focused findings and concepts of this disease, including the most significant pathogenic process based on kinds of immunocytes, cytokines, as well as participation of epithelial-mesenchymal transition, the clinical value of elevated serum IgG4 concentration and pathological role of infiltrated IgG4+ plasma cells, the potential relationship with salivary gland malignant tumor, the applying and usefulness of positron emission tomography-CT, the diagnostic utility of lip biopsy, treatment, prognosis, and also future perspectives.

  17. Clinicopathological features of IgG4-related lymphadenopathy%IgG4相关性淋巴结病的临床病理特点

    Institute of Scientific and Technical Information of China (English)

    张卉; 冯瑞娥

    2012-01-01

    Immunoglobulin G4-related disease (lgG4-RD) is an emerging disease entity of unknown etiology with multiorgan involvement. IgG4-related lymphadenopathy may be easily confused with multicentric Castleman's disease, follicular hyperplasia, or malignant lymphoma due to its lack of special clinicopathological features. Although IgG4-related lymphadenopathy shows a dramatic response to steroid therapy, some cases may be potentially associated with the development of lymphoma. Thus it is desirable to improve our knowledge of IgG4 related lymphadenopathy. In this review, we summarize the clinicopathological characteristics of IgG4-related lymphadenopathy.%IgG4相关性疾病作为一种全身系统性疾病,可累及多个器官及组织.发生于淋巴结的IgG4相关性淋巴结病由于其临床病理特点缺乏特异性,需与多种病变鉴别,如Castleman's病样、淋巴滤泡反应性增生、淋巴瘤等.尽管IgG4相关性疾病经激素治疗效果显著,但是某些患者可发展成淋巴瘤.因此有必要提高对IgG4相关性淋巴结病的认识.本文就IgG4相关性淋巴结病的临床及病理组织学等特点进行综述.

  18. IgG4-related interstitial lung disease%IgG4相关间质性肺疾病

    Institute of Scientific and Technical Information of China (English)

    荣令; 李家树; 周新

    2010-01-01

    IgG4-related interstitial lung disease is proposed based on the findings of systemic IgG4-related sclerosing disease. The diagnosis of IgG4-related interstitial lung disease requires the combination of clinical-radiological-pathological features. The therapeutic response of IgG4-related interstitial lung disease to glucocorticoid is good,and follow-up is necessary for the detection of recurrence.%IgG4相关间质性肺疾病的提出是基于对系统性IgG4相关硬化性疾病的研究发现.IgG4相关间质性肺疾病的诊断需要临床医师结合临床-影像学-病理学特征疑及此病.IgG4相关间质性肺疾病对糖皮质激素治疗反应良好,监测随访对于发现复发是必要的.

  19. Therapeutic IgG4 antibodies engage in Fab-arm exchange with endogenous human IgG4 in vivo

    NARCIS (Netherlands)

    A.F. Labrijn; A.O. Buijsse; E.T.J. van den Bremer; A.Y.W. Verwilligen; W.K. Bleeker; S.J. Thorpe; J. Killestein; C.H. Polman; R.C. Aalberse; J. Schuurman; J.G.J. van de Winkel; P.W.H.I. Parren

    2009-01-01

    Two humanized IgG4 antibodies, natalizumab and gemtuzumab, are approved for human use, and several others, like TGN1412, are or have been in clinical development. Although IgG4 antibodies can dynamically exchange half-molecules(1), Fab-arm exchange with therapeutic antibodies has not been demonstrat

  20. Data of evolutionary structure change: 1ELTA-2G4UA [Confc[Archive

    Lifescience Database Archive (English)

    Full Text Available 1ELTA-2G4UA 1ELT 2G4U A A VVGGRVAQPNSWPWQISLQYKSGSSYYHTCGGSLIRQGW...VGGTEAQRNSWPSQISLQYRSGSSWAHTCGGTLIRQNWVMTAAHCVDRELTFRVVVGEHNLNQNDGTEQYVGVQKIVVHPY...E EEEEEEEEEE EEEEEEGG HHHHHHHHH 0 1ELT... A 1ELTA

  1. Data of evolutionary structure change: 1ELTA-2G4TA [Confc[Archive

    Lifescience Database Archive (English)

    Full Text Available 1ELTA-2G4TA 1ELT 2G4T A A VVGGRVAQPNSWPWQISLQYKSGSSYYHTCGGSLIRQGW...VGGTEAQRNSWPSQISLQYRSGSSWAHTCGGTLIRQNWVMTAAHCVDRELTFRVVVGEHNLNQNDGTEQYVGVQKIVVHPY...E EEEEEEEEEEEE EEEEEEGG HHHHHHHHH 0 1ELT... A 1ELTA

  2. Selection for the G4 DNA motif at the 5' end of human genes.

    Science.gov (United States)

    Eddy, Johanna; Maizels, Nancy

    2009-04-01

    Formation of G4 DNA may occur in the course of replication and transcription, and contribute to genomic instability. We have quantitated abundance of G4 motifs and potential for G4 DNA formation of the nontemplate strand of 5' exons and introns of transcripts of human genes. We find that, for all human genes, G4 motifs are enriched in 5' regions of transcripts relative to downstream regions; and in 5' regulatory regions relative to coding regions. Notably, although tumor suppressor genes are depleted and proto-oncogenes enriched in G4 motifs, abundance of G4 motifs in the 5' regions of transcripts of genes in these categories does not differ. These results support the hypothesis that G4 motifs are under selection in the human genome. They further show that for tumor suppressor genes and proto-oncogenes, independent selection determines potential for G4 DNA formation of 5' regulatory regions of transcripts and downstream coding regions.

  3. An IgG4-related sclerosing mediastinitis in posterior mediastinum: CT findings

    Directory of Open Access Journals (Sweden)

    Xiaolong Liu

    2015-03-01

    Full Text Available Immunoglobulin G4 (IgG4-related sclerosing disease, an uncommon disease entity, is known to involve various organs. To our knowledge, few reports have been presented on IgG4-related sclerosing diseases involving the mediastinum, especially the posterior mediastinum. We present a case of IgG4-related sclerosing disease of the posterior mediastinum with imaging findings on computed tomography (CT.

  4. 16 CFR Appendix G4 to Part 305 - Mobile Home Furnaces

    Science.gov (United States)

    2010-01-01

    ... 16 Commercial Practices 1 2010-01-01 2010-01-01 false Mobile Home Furnaces G4 Appendix G4 to Part 305 Commercial Practices FEDERAL TRADE COMMISSION REGULATIONS UNDER SPECIFIC ACTS OF CONGRESS RULE... Appendix G4 to Part 305—Mobile Home Furnaces Manufacturer's rated heating capacities (Btu's/hr.) Range of...

  5. Overlapping Morphologic and Immunohistochemical Features of Hashimoto Thyroiditis and IgG4-Related Thyroid Disease.

    Science.gov (United States)

    Raess, Philipp W; Habashi, Arlette; El Rassi, Edward; Milas, Mira; Sauer, David A; Troxell, Megan L

    2015-05-01

    Immunoglobulin G4-related disease (IgG4-RD) is an emerging clinicopathologic entity characterized by both IgG4+ plasma cell infiltration and fibrosis in one or more organs, prototypically pancreas or salivary/lacrimal glands. IgG4-RD in the thyroid (IgG4-RTD) is an area of active study, and the relationship between IgG4-RTD and Hashimoto thyroiditis is not fully delineated due to their overlapping histologic features. Retrospective review was performed of all thyroidectomy cases demonstrating lymphocytic inflammation at a single institution over a 4-year period. Approximately half (23/38) of patients had a clinical diagnosis of Hashimoto thyroiditis (HT). Nine of the 38 patients had increased absolute and relative numbers of IgG4+ plasma cells. Patients with a clinical diagnosis of HT had increased lymphoplasmacytic inflammation, but the relative proportion of IgG4+ plasma cells was not increased compared to patients without HT. There was no correlation between IgG4 levels and the amount of fibrosis in patients with or without HT. Patients identified as having the fibrosing variant of HT were not more likely to have increased levels of IgG4+ plasma cells than those without. There is significant morphologic and immunohistochemical overlap between HT and IgG4-RTD. Future studies to identify specific characteristics of IgG4-RTD involving the thyroid are necessary to accurately define this entity.

  6. Immunoglobulin G4-related prostatitis: A case-control study focusing on clinical and pathologic characteristics

    NARCIS (Netherlands)

    J. Buijs (Jorie); L. Maillette De Buy Wenniger (Lucas); G.J.H.L. Leenders (Geert); J. Verheij (Joanne); I. van Onna (Ilze); B.E. Hansen (Bettina); M. van Heerde (Marianne); N.C. Krak (Nanda); U. Beuers (Ulrich); M.J. Bruno (Marco); H.R. van Buuren (Henk)

    2014-01-01

    markdownabstractOBJECTIVE To evaluate the occurrence and histopathologic characteristics of immunoglobulin G4 (IgG4)- related prostatic involvement in patients diagnosed with autoimmune pancreatitis. METHODS Nine cases of IgG4-related prostatitis were identified among 117 men in the autoimmune pan

  7. Comparative Electrostatic Force Microscopy of Tetra- and Intra-Molecular G4-DNA

    CERN Document Server

    Livshits, Gideon I; Borovok, Natalia; Kotlyar, Alexander B; Porath, Danny

    2014-01-01

    Two forms of G4-DNA, with parallel and pairwise anti-parallel strands, are studied using atomic force microscopy. The directionality of the strands affects the molecules' structural properties (different height and length) and their electrical polarizability. Parallel G4-DNA is twice as polarizable as anti-parallel G4-DNA, suggesting it is a better electrical wire for bio-nanoelectronics.

  8. Data of evolutionary structure change: 1B7QA-2G4QA [Confc[Archive

    Lifescience Database Archive (English)

    Full Text Available 1B7QA-2G4QA 1B7Q 2G4Q A A KVFERCELARTLKRLGMDGYRGISLALWMCLAKWESGYN...GRCELAAAMKRHGLDNYRGYSLGNWVCAAKFESNFNTQATNRNTD-GSTDYGILQINSRWWCNDGRTPGSRNLCNIPCSALLSSDITASVNCAKKIVSDGNGMNAWVAWRNRCKGTDVQA... A 2G4QA NRNTD-GSTDY 1B7Q A 1B7QA

  9. Data of evolutionary structure change: 1D6QA-2G4QA [Confc[Archive

    Lifescience Database Archive (English)

    Full Text Available 1D6QA-2G4QA 1D6Q 2G4Q A A KVFERCELARTLKRLGMDGYRGISLANWMCLAKWESGYN...GRCELAAAMKRHGLDNYRGYSLGNWVCAAKFESNFNTQATNRNTD-GSTDYGILQINSRWWCNDGRTPGSRNLCNIPCSALLSSDITASVNCAKKIVSDGNGMNAWVAWRNRCKGTDVQA...bID> A 2G4QA NRNTD-GS...> 1D6Q A 1D6QA

  10. Immunoglobulin G4-Related Inflammatory Abdominal Aortic Aneurysm Associated With Myasthenia Gravis, With Contained Rupture.

    Science.gov (United States)

    Jun, Heungman; Jung, Cheol Woong

    2016-11-01

    Immunoglobulin (Ig) G4-related disease is reportedly among the various causes of inflammatory abdominal aortic aneurysm (IAAA). Many IgG4-related diseases are closely related to allergic constitution and autoimmune disease. We report a case of a 72-year-old man with IgG4-related IAAA associated with myasthenia gravis, with contained rupture.

  11. 17 CFR 240.17g-4 - Prevention of misuse of material nonpublic information.

    Science.gov (United States)

    2010-04-01

    ... 17 Commodity and Securities Exchanges 3 2010-04-01 2010-04-01 false Prevention of misuse of material nonpublic information. 240.17g-4 Section 240.17g-4 Commodity and Securities Exchanges SECURITIES... Organizations § 240.17g-4 Prevention of misuse of material nonpublic information. (a) The written policies...

  12. 75 FR 32317 - Proposed Revocation of Colored Federal Airway G-4; AK

    Science.gov (United States)

    2010-06-08

    ... Federal Aviation Administration 14 CFR Part 71 Proposed Revocation of Colored Federal Airway G-4; AK...: This action proposes to remove Colored Federal Airway Green 4 (G-4) from the National Airspace System...) part 71 by removing Colored Federal airway G-4 associated with the planned BTS NDB decommissioning...

  13. 75 FR 47737 - Proposed Revocation of Colored Federal Airway G-4; AK

    Science.gov (United States)

    2010-08-09

    ... G-4; AK AGENCY: Federal Aviation Administration (FAA), DOT. ACTION: Notice of proposed rulemaking... Airway Green (G-4) in Alaska. The FAA has determined that the withdrawal is warranted since the proposed... NPRM proposing to remove Federal Airway G-4 (75 FR 32317), Docket No. FAA- 2010-0453. A review...

  14. Isolated Mass-Forming IgG4-Related Cholangitis as an Initial Clinical Presentation of Systemic IgG4-Related Disease.

    Science.gov (United States)

    Kim, Seokhwi; Bae, Hyunsik; Choi, Misun; Kim, Binnari; Heo, Jin Seok; Kim, Ho Seong; Choi, Seung Hee; Jang, Kee-Taek

    2016-07-01

    IgG4-related disease (IgG4-RD) may involve multiple organs. Although it usually presents as diffuse organ involvement, localized mass-forming lesions have been occasionally encountered in pancreas. However, the same pattern has been seldom reported in biliary tract. A 61-year-old male showed a hilar bile duct mass with multiple enlarged lymph nodes in imaging studies and he underwent trisectionectomy under impression of cholangiocarcinoma. Gross examination revealed a mass-like lesion around hilar bile duct. Histopathologically, dense lymphoplasmacytic infiltration and storiform fibrosis were identified without evidence of malignancy. Immunohistochemical stain demonstrated rich IgG4-positive plasma cell infiltration. Follow-up imaging studies disclosed multiple enlarged lymph nodes with involvement of pancreas and perisplenic soft tissue. The lesions have been significantly reduced after steroid treatment, which suggests multi-organ involvement of systemic IgG4-RD. Here, we report an unusual localized mass-forming IgG4-related cholangitis as an initial presentation of IgG4-RD, which was biliary manifestation of systemic IgG4-related autoimmune disease.

  15. Isolated Mass-Forming IgG4-Related Cholangitis as an Initial Clinical Presentation of Systemic IgG4-Related Disease

    Directory of Open Access Journals (Sweden)

    Seokhwi Kim

    2016-07-01

    Full Text Available IgG4-related disease (IgG4-RD may involve multiple organs. Although it usually presents as diffuse organ involvement, localized mass-forming lesions have been occasionally encountered in pancreas. However, the same pattern has been seldom reported in biliary tract. A 61-year-old male showed a hilar bile duct mass with multiple enlarged lymph nodes in imaging studies and he underwent trisectionectomy under impression of cholangiocarcinoma. Gross examination revealed a mass-like lesion around hilar bile duct. Histopathologically, dense lymphoplasmacytic infiltration and storiform fibrosis were identified without evidence of malignancy. Immunohistochemical stain demonstrated rich IgG4-positive plasma cell infiltration. Follow-up imaging studies disclosed multiple enlarged lymph nodes with involvement of pancreas and perisplenic soft tissue. The lesions have been significantly reduced after steroid treatment, which suggests multi-organ involvement of systemic IgG4-RD. Here, we report an unusual localized mass-forming IgG4-related cholangitis as an initial presentation of IgG4-RD, which was biliary manifestation of systemic IgG4-related autoimmune disease.

  16. Emphasizing differential diagnosis of immunoglobulin G4 related diseases%重视IgG4相关性疾病的鉴别诊断

    Institute of Scientific and Technical Information of China (English)

    张文

    2013-01-01

    IgG4相关性疾病(IgG4-RD)是近年来新认识的一种由免疫介导的慢性自身炎症伴纤维化的疾病,该病的共同特点是多器官、系统受累;受累器官或组织肿胀伴硬化;受累组织中大量淋巴细胞和IgG4阳性浆细胞浸润;血清IgG4显著增高.由于一些疾病(如肿瘤、自身免疫性疾病或感染)也可出现血清IgG4升高或组织中IgG4阳性浆细胞浸润,因此需要与该病进行鉴别诊断.该文强调了IgG4-RD鉴别诊断的重要性.

  17. The strong edge coloring of graphs with A(G)=4%△(G)=4的图的强边染色

    Institute of Scientific and Technical Information of China (English)

    朱海洋

    2008-01-01

    针对1985年Erd6s和Negetǐil提出的强边一染色猜想:令G为图,若△(G)为偶数,则Sx'(G)≤5△2(G)/4;若△(G)为奇数,则Sx'(G)≤5△2(G)/4-△(G)/2+1/4.证明了对于令G为△(G)=4的图,若δ(G)≤3或围长g(G)≤4,则SX'(G)≤21.

  18. Spectrum of Disorders Associated with Elevated Serum IgG4 Levels Encountered in Clinical Practice

    Directory of Open Access Journals (Sweden)

    Jay H. Ryu

    2012-01-01

    Full Text Available IgG4-related disease (IgG4-RD is a recently described systemic fibroinflammatory disease associated with elevated circulating levels of IgG4 and manifests a wide spectrum of clinical presentations. Although serum IgG4 level has been described to be the most sensitive and specific laboratory test for the diagnosis of IgG4-RD, it is recognized that an elevated serum IgG4 level can be encountered in other diseases. In this study, we sought to identify the frequency of IgG4-RD and other disease associations in patients with elevated serum IgG4 levels seen in clinical practice. Among 3,300 patients who underwent IgG subclass testing over a 2-year period from January 2009 to December 2010, 158 (4.8% had an elevated serum IgG4 level (>140 mg/dL. IgG4 subclass testing was performed for evaluation of suspected IgG4-RD or immunodeficiency. Twenty-nine patients (18.4% had definite or possible IgG4-RD. Among those patients without IgG4-RD, a broad spectrum of biliary tract, pancreatic, liver, and lung diseases, as well as systemic vasculitis, was diagnosed. We conclude that patients with elevated serum IgG4 levels encountered in clinical practice manifest a wide array of disorders, and only a small minority of them has IgG4-RD.

  19. Immunohistochemical Characteristics of IgG4-Related Tubulointerstitial Nephritis: Detailed Analysis of 20 Japanese Cases

    Directory of Open Access Journals (Sweden)

    Mitsuhiro Kawano

    2012-01-01

    Full Text Available Although tubulointerstitial nephritis with IgG4+ plasma cell (PC infiltration is a hallmark of IgG4-related kidney disease (IgG4-RKD, only a few studies are available about the minimum number of IgG4+ PC needed for diagnosis along with IgG4+/IgG+ PC ratio in the kidney. In addition, the significance of the deposition of IgG or complement as a reflection of humoral immunity involvement is still uncertain. In this study, we analyzed 20 Japanese patients with IgG4-RKD to evaluate the number of IgG4+ PCs along with IgG4+/IgG+ PC ratio and involvement of humoral immunity. The average number of IgG4+ PCs was 43.8/hpf and the average IgG4+/IgG+ or IgG4+/CD138+ ratio was 53%. IgG and C3 granular deposits on the tubular basement membrane (TBM were detected by immunofluorescence microscopy in 13% and 47% of patients, respectively. Nine patients had a variety of glomerular lesions, and 7 of them had immunoglobulin or complement deposition in the glomerulus. In conclusion, we confirmed that infiltrating IgG4+ PCs > 10/hpf and/or IgG4/IgG (CD138+ PCs > 40% was appropriate as an item of the diagnostic criteria for IgG4-RKD. A relatively high frequency of diverse glomerular lesions with immunoglobulin or complement deposits and deposits in TBM may be evidence of immune complex involvement in IgG4-related disease.

  20. Tumefactive immunoglobulin G4-related disease involving the dura mater: A case report and literature review

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Jeong Hoon; Lee, Ji Hoon; Ko, Yong; Paik, Seoung Sam; Lee, Young Jun [Dept. of Radiology, Hanyang University Hospital, Seoul (Korea, Republic of); Park, Dong Woo [Dept. of Radiology, Hanyang University Hospital, Seoul (Korea, Republic of)

    2015-08-15

    Immunoglobulin G4 (IgG4)-related disease is a well-known disorder characterized by an inflammatory reaction with an increase in the number of IgG4-positive plasma cells associated with sclerosis. IgG4-related disease often affects the dura mater with a pattern of diffuse thickening when the central nervous system is involved. However, some nodular dural thickening requires discrimination from tumors because of obviously different treatment options. We report of a case of IgG4-related disease with tumefactive dural involvement.

  1. Appendiceal immunoglobulin G4-related disease mimicking appendiceal tumor or appendicitis: A case report

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Hyun Soo; Kang, Won Kyung; Chung, Dong Jin [Yeouido St. Mary' s Hospital, The Catholic University of Korea, Seoul (Korea, Republic of)

    2016-02-15

    Immunoglobulin G4 (IgG4)-related disease is an autoimmune disease that forms tumorous lesions. Several cases involving various organs are reported, however, IgG4-related disease involving appendix has not been reported yet. In this report, we presented a case of IgG4-related disease of appendix, which raised a suspicion of appendiceal tumor or usual appendicitis and, therefore, led to unnecessary surgical resection. IgG4-related disease should be considered in the differential diagnosis for a mass-like swelling of the appendix, in order to avoid unnecessary surgery.

  2. A case of immunoglobulin G-4 related sclerosing disease mimicking lung cancer

    Energy Technology Data Exchange (ETDEWEB)

    Kwon, Soo Hee; Lee, Young Kyung; Shim, Mi Suk; Lee, Hyang Im [Seoul Medical Center, Seoul (Korea, Republic of)

    2013-07-15

    Immunoglobulin (Ig) G4-related sclerosing disease is a recently described systemic fibro-inflammatory disease associated with an elevated circulating level of IgG4 and extensive IgG4-positive lymphoplasmacytic infiltration, resulting in sclerosing inflammation involving various body organs. We experienced one case where surgery confirmed IgG4-related sclerosing disease as a solitary lung mass mimicking lung cancer. We report radiologic findings including chest computed tomography and positron emission tomography computed tomography, with clinical manifestations of IgG4-related sclerosing disease.

  3. The Utility of Serum IgG4 Concentrations as a Biomarker

    Directory of Open Access Journals (Sweden)

    Shigeyuki Kawa

    2012-01-01

    Full Text Available IgG4-related disease is a new disease entity involving IgG4 in its clinical presentation and having 6 characteristic features: (1 systemic involvement; (2 solitary or multiple lesions showing diffuse or localized swelling, masses, nodules, and/or wall thickening on imaging; (3 high serum IgG4 concentration >135 mg/dL; (4 abundant infiltration of lymphoplasmacytes and IgG4-bearing plasma cells; (5 a positive response to corticosteroid therapy; and (6 complications of other IgG4-related diseases. To date, most IgG4-related diseases have been recognized as extrapancreatic lesions of autoimmune pancreatitis. This paper will discuss the utility of IgG4 as a biomarker of IgG4-related diseases, including in the diagnosis of autoimmune pancreatitis and its differentiation from pancreatic cancer, in the prediction of relapse, in the long-term follow-up of patients with autoimmune pancreatitis and normal or elevated IgG4 concentrations, and in patients with autoimmune pancreatitis and extrapancreatic lesions, as well as the role of IgG4 in the pathogenesis of IgG4-related disease.

  4. Elevated serum IgG4 levels in two cases of paragonimiasis

    Science.gov (United States)

    Saeki, Sho; Horio, Yuko; Hirosako, Susumu; Ichiyasu, Hidenori; Fujii, Kazuhiko; Kohrogi, Hirotsugu

    2015-01-01

    Paragonimiasis is a parasitic pleuropulmonary infection caused by eating raw crustaceans and wild boar meat and this infection is endemic in Asia. We herein report two cases of pulmonary Paragonimus westermani infection associated with elevated levels of serum immunoglobulin (Ig) G4 and dense infiltration of IgG4-positive plasma cells in the lung lesions. Treatment with praziquantel resolved the pulmonary lesions and decreased the serum levels of IgG4. IgG4-related disease is a systemic disease occasionally involving the lungs and leads to increased serum levels of IgG4. Our findings suggest that P. westermani infection requires a differential diagnosis from IgG4-related diseases and the serum IgG4 level may be a potentially useful marker of P. westermani infection. PMID:26392854

  5. A case of immunoglobulin G4-related respiratory disease with multiple lung cysts: A case report

    Directory of Open Access Journals (Sweden)

    Hironori Mikumo

    2017-01-01

    Full Text Available A 48-year-old man was admitted for evaluation of abnormal shadows on chest radiograph. Chest computed tomography (CT showed cysts, nodules, and cervical and axillary lymphadenopathies. Elevated serum levels of IgG4 and interleukin (IL-6 suggested IgG4-related disease (IgG4-RD or multicentric Castleman's disease (MCD. Histologic findings of the cervical lymph node and right lung S6 biopsies revealed numerous IgG4-positive plasma cells. Although CT findings of the lungs were atypical for IgG4-RD, consistent histologic findings, clinical symptoms, and laboratory data made us conclude IgG4-RD. Because histologic findings of IgG4-RD and MCD have similarities, differentiating between the two diseases should consider the clinical presentation.

  6. A case of IgG4-related lymphadenopathy, pericarditis, coronary artery periarteritis and luminal stenosis.

    Science.gov (United States)

    Hourai, Ryoto; Miyamura, Masatoshi; Tasaki, Ryunosuke; Iwata, Akiko; Takeda, Yoshihiro; Morita, Hideaki; Hanaoka, Nobuharu; Tanigawa, Jun; Shibata, Kensaku; Takeshita, Atsushi; Kawano, Mitsuhiro; Sato, Yasuharu; Hirose, Yoshinobu; Ishizaka, Nobukazu

    2016-10-01

    Immunoglobulin G4 (IgG4)-related disease is an emerging new clinicopathological disorder that is characterized by elevation of serum IgG4 levels and histological findings of IgG4-positive plasmacytic infiltration. IgG4-related disease may appear synchronously or metachronously in a wide variety of organs. The current patient was found to have pericardial effusion and retroperitoneal fibrosis. He was subsequently diagnosed with coronary artery stenosis. (18)F-FDG positron emission tomography showed enhanced FDG uptake in lymph nodes as well as pericardial and peri-aortic tissue. Histopathology of the mediastinal lymph node showed the infiltration of numerous IgG4-positive cells, leading to the diagnosis of IgG4-related lymphadenopathy with pericardial and periarterial involvement.

  7. Gastric calcifying fibrous tumor: A case of suspected immunoglobulin G4-related gastric disease

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    Hejun Zhang

    2015-01-01

    Full Text Available Gastrointestinal lesions resulting from immunoglobulin G4-related disease are classified into two types: One is a gastrointestinal lesion showing marked thickening of the wall, and the other is an IgG4-related pseudotumor. We report the case of a woman with gastric calcifying fibrous tumor undergoing endoscopic resection that contained 62 IgG4+ plasma cells per high-power field and an IgG4-to-IgG ratio of 41% in lesional plasma cells, which shared clinical and histopathological features associated with gastric IgG4-related pseudotumor. So, we postulate that calcifying fibrous tumor as part of the spectrum of IgG4-related disease might be the unifying concept with IgG4-related pseudotumor. Meanwhile, the patient had coexistent autoimmune diseases, including autoimmune atrophic gastritis, Hashimoto's thyroiditis, and possible primary biliary cirrhosis. The clinical follow-up evaluation was uneventful.

  8. Orbital Pseudotumor: Uncommon Initial Presentation of IgG4-Related Disease

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    Teresa Carbone

    2015-01-01

    Full Text Available IgG4-related disease (IgG4-RD encompasses a group of fibroinflammatory conditions recognized in recent times. The main clinical features include variable degrees of tissue fibrosis, tumorlike expansions, perivascular lymphocytic infiltration rich in IgG4-positive plasma cells, and elevated serum IgG4. A case has been reported of an elderly patient with an unexplained unilateral exophthalmia; biopsy was performed and revealed lymphocytic infiltration, suggesting IgG4-RD. High serum levels of IgG4, in association with a good response to steroid therapy and to the exclusion of other diagnoses, confirmed the hypothesis of orbital pseudotumor by IgG4-RD.

  9. IgG4与风湿免疫性疾病

    Institute of Scientific and Technical Information of China (English)

    黄安芳; 庄越; 张伶姝; 刘毅

    2015-01-01

    近年来,随着对IgG4分子的逐步认识,其临床意义也得到了关注和重视。目前临床工作中发现IgG4水平不仅对IgG4相关性疾病(IgG4-related disease,IgG4-RD)诊断有重要价值,部分风湿免疫性疾病患者也存在IgG4水平的异常。本文主要就IgG4分子生物学特点及与风湿免疫性疾病相关性作一综述。

  10. A Case of IgG4-Related Lung Disease Presenting as Interstitial Lung Disease.

    Science.gov (United States)

    Ahn, Jee Hwan; Hong, Sun In; Cho, Dong Hui; Chae, Eun Jin; Song, Joon Seon; Song, Jin Woo

    2014-08-01

    Intrathoracic involvement of immunoglobulin G4 (IgG4)-related disease has recently been reported. However, a subset of the disease presenting as interstitial lung disease is rare. Here, we report a case of a 35-year-old man with IgG4-related lung disease with manifestations similar to those of interstitial lung disease. Chest computed tomography showed diffuse ground glass opacities and rapidly progressive pleural and subpleural fibrosis in both upper lobes. Histological findings showed diffuse interstitial lymphoplasmacytic infiltration with an increased number of IgG4-positive plasma cells. Serum levels of IgG and IgG4 were also increased. The patient was diagnosed with IgG4-related lung disease, treated with anti-inflammatory agents, and showed improvement. Lung involvement of IgG4-related disease can present as interstitial lung disease and, therefore, should be differentiated when evaluating interstitial lung disease.

  11. IgG4相关性腹膜后纤维化CT诊断%Diagnosis of IgG4-related retroperitoneal fibrosis

    Institute of Scientific and Technical Information of China (English)

    田芳; 朴雪梅; 周自明; 常时新; 丁永生

    2012-01-01

    Objective: To study and summarize the clinical features and the imaging manifestations of IgG4-related retroperitoneal fibrosis and to improve the understanding of IgG4-related sclerosing disease. Methods: A case of IgG4-related retroperitoneal fibrosis was reported,including clinical features,laboratory reports,imaging manifestations and pathological results,and relative literatures were reviewed. Results: The disease had no distinctive clinical features. Symptoms include vague pain in the lower back or abdomen,anorexia,fatigue,fever and weight loss. The imaging manifestation was soft tissue mass. The level of serum IgG (especially IgG4) was strikingly high. The histological manifestation in the involved organs showed diffuse infiltration of lymphoplasma cells and fibrosis. Abundant IgG4-positive plasma cells could be found and the treatment with glucocorticoid was effective. Conclusion:IgG4-related retroperitoneal fibrosis lacks specifity. It is essential to improve its understanding to avoid misdiagnosis.%目的:总结IgG4相关腹膜后纤维化的临床表现及影像特点,提高时IgG4相关的硬化性疾病的认识.方法:报告1例IgC4相关腹膜后纤维化的临床表现、实验室检查、影像学资料、病理结果等,并复习相关文献.结果:该病临床症状无明显特异性,可有腹痛、背痛、疲乏、食欲下降等,影像表现为腹膜后肿块,该病与自身免疫性胰腺炎关系密切,血清IgG尤其是IgG4显著升高;受累脏器组织学表现为弥漫性淋巴浆细胞漫润和纤维化,大量IgG4阳性浆细胞;糖皮质激素治疗有效.结论:IgG4相关腹膜后纤维化临床症状缺乏特异性,极易误诊或漏诊,需提高对该病的认识.

  12. Elevated Serum IgG4 Defines Specific Clinical Phenotype of Rheumatoid Arthritis

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    Le-Feng Chen

    2014-01-01

    Full Text Available Objectives. To explore the correlation of serum IgG4 (sIgG4 with clinical manifestations or therapeutic response in rheumatoid arthritis (RA. Methods. Consecutive 136 RA patients were recruited and followed up at regular interval. SIgG4 was detected by immunonephelometry. Serial synovial tissue sections from 46 RA patients were stained immunohistochemically for IgG4. Results. Forty-six percent of 136 RA patients had elevated sIgG4. Patients with elevated sIgG4 had higher sIgG4/sIgG ratio, C-reactive protein, erythrocyte sedimentation rate, rheumatoid factor, and anticyclic citrullinated peptide antibodies than those with normal sIgG4 (all P<0.05. Among 45 patients who received methotrexate and leflunomide therapy, 50% (9/18 of patients with elevated sIgG4 and 85% (23/27 of patients with normal sIgG4 reached therapeutic target (disease activity score of 28 joints < 3.2 at 6-month visit (χ2=6.508, P=0.011. IgG4-positive plasma cell count correlated positively with sIgG4, total synovitis score, and CD3-, CD20-, and CD38-positive cell counts (all P<0.05. Conclusions. Our results showed that elevated sIgG4 in RA is common and disproportional to total IgG and RA with elevated sIgG4 may be a specific clinical phenotype with higher disease activity, higher level of autoantibodies, and poor response to methotrexate and leflunomide therapy.

  13. IgG4-Related Autoimmune Prostatitis: Is It an Unusual or Underdiagnosed Manifestation of IgG4-Related Disease?

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    María T. Bourlon

    2013-01-01

    Full Text Available IgG4-related disease (IgG4-RD encompasses a wide range of extrapancreatic manifestations. Albeit some are relatively well known, others such as autoimmune prostatitis remain poorly described. We present a 61-year-old Latin-American male with autoimmune pancreatitis (AIP who presented with lower urinary tract symptoms (LUTS, normal prostate specific antigen (PSA test, and prostate enlargement attributed to benign prostatic hyperplasia (BPH. He underwent a transurethral resection of the prostate (TURP after which symptoms were resolved. On histopathology, prostatic stroma had a dense inflammatory infiltrate rich in plasma cells and lymphocytes; immunohistochemical morphometric assessment showed >10 IgG4-positive plasma cells/high power field (HPF. The diagnosis of IgG4-related prostatitis was postoperatively. We compared the patient characteristics with those of previous reports on Asian patients. Shared findings included prostate enlargement, LUTS (symptoms that can be confused with BPH, and PSA within normal limits or mild elevations. IgG4-related prostatitis is rarely considered as a preprocedural diagnosis, even in patients with evidence of IgG4-RD. Involved prostate zones include mainly central and transitional zones and less frequently the peripheral. Currently, there is insufficient data about the natural history and outcome. Whether steroids, transurethral resection, or both are the treatment of choice needs to be elucidated.

  14. Clinical analysis of 49 cases with IgG4-related disease%IgG4相关性疾病49例分析

    Institute of Scientific and Technical Information of China (English)

    马玲瑛; 马莉莉; 纪宗斐; 纪元; 侯英勇; 姜林娣

    2015-01-01

    Objective To investigate the clinical manifestations,radiographic presentations,histopathological features,treatment and clinical follow-up of IgG4 related disease (IgG4-RD).Methods Forty-nine cases in our hospital were enrolled from 2009 to 2012 and were followed up.Results Of the 49 patients with IgG4-RD,the male to female ratio was 2.1∶1,the mean age was (53±15) years,and the serum IgG4 ranged between 0.39 to 20.8 g/L.The most commonly affected organ was pancreas.Two or more organs were involved in 15 patients.Histopathological findings included tissue infiltration by lymphocytes,IgG+ plasma cells and IgG4+ plasma cells and diffuse fibrosis among 45 patients.The IgG4+ plasma cells were over 10/high power fields (HPF).Responses to glucocorticoid in combination with immunosuppressants were good except 3 patients suffered relapses.Conclusion IgG4-RD is an autoimmune disease affecting multiple organs,and the increased IgG4 positive plasma cells is characteristic.This disease can be effectively alleviated with prompt use of glucocorticoid.%目的 总结IgG4相关性疾病(IgG4-RD)的临床特点、影像学特征、组织病理学表现及治疗预后.方法 回顾性收集2009-2012年在我院诊治的49例患者临床资料,并进行随访.结果 49例IgG4-RD平均年龄(53±15)岁,其中男女比例为2.1∶1,血清IgG4水平在0.39~20.8 g/L之间.胰腺受累患者最多,15例患者伴有多部位受累.共有45例患者进行组织病理学检查,主要表现为淋巴浆细胞浸润及纤维化形成,IgG4+细胞在每高倍镜视野下>10个.43例患者进行糖皮质激素联合免疫抑制剂治疗,激素减量后,3例患者疾病复发.结论 IgG4-RD是多器官受累的自身免疫病,受累脏器IgG4阳性浆细胞浸润是其主要特点,及时应用糖皮质激素可有效缓解病情.

  15. Report on one case of IgG4-related retroperitoneal fibrosis and literatures review for IgG4-related disease%IgG4相关性腹膜后纤维化一例及IgG4相关性疾病文献复习

    Institute of Scientific and Technical Information of China (English)

    牛强; 丁倩倩; 陈勤奋

    2013-01-01

    目的 探讨IgG4相关性腹膜后纤维化(IgG4-RRPF)和IgG4相关性疾病(IgG4-RD)的临床表现、诊断和治疗方法.方法 对1例IgG4-RRPF患者的临床资料进行回顾性分析,复习相关文献并介绍一个新的临床病种IgG4-RD.结果 患者,52岁,男性,以左下腹痛和左肾积液为表现,影像学显示左侧腰大肌内侧异常软组织影,PET-CT显示该软组织影脱氧葡萄糖代谢摄取增高,经肿块切取活检术,病理诊断符合IgG4-RD,诊断为IgG4-RRPF,予泼尼松治疗后症状缓解,肿块缩小.复习最新文献发现IgG4-RRPF即以往所称的特发性腹膜后纤维化(IRF).结论 IgG4-RRPF是IgG4-RD谱中的一种罕见类型,以腹膜后组织慢性炎症和显著纤维化为特征,常包绕输尿管或其他腹部脏器,血液学检查显示血清IgG4浓度升高(≥1.35 g/L),组织病理学检查显示明显淋巴细胞和IgG4+浆细胞浸润伴纤维化.一线治疗给予糖皮质激素.%Objective To explore the clinical manifestation,diagnosis and treatment of IgG4-related retroperitoneal fibrosis (IgG4-RRPF) and IgG4-related disease (IgG4-RD).Methods The clinical data of a case of IgG4-RRPF was analyzed retrospectively,the related literatures were reviewed,and a novel clinical entity IgG4-RD was introduced.Results The patient was a 52-year-old man with the complaint of left lower abdominal pain due to left hydronephrosis.CT and MRI revealed a soft tissue mass at the left psoas major muscle.PET-CT showed increased FDG uptake.After biopsy,the pathological diagnosis was consistent with IgG4-RD.The patient was diagnosed as IgG4-RRPF and treated with prednisone.The symptoms improved and retroperitonea] mass reduced.According to review the latest literatures,it is found that IgG4-RRPF was socalled idiopathic retroperitoneal fibrosis (IRF) formerly.Conclusion IgG4-RRPF is a rare disease in IgG4-RD spectrum,characterised by the presence of a retroperitoneal tissue,consisting of chronic inflammation and marked

  16. IgG4-Associated Cholangitis--A Mimic of PSC.

    Science.gov (United States)

    Beuers, Ulrich; Hubers, Lowiek M; Doorenspleet, Marieke; Maillette de Buy Wenniger, Lucas; Klarenbeek, Paul L; Boonstra, Kirsten; Ponsioen, Cyriel; Rauws, Erik; de Vries, Niek

    2015-01-01

    IgG4-associated cholangitis (IAC) is an inflammatory disorder of the biliary tract representing a major manifestation of IgG4-related disease (IgG4-RD) often with elevation of serum IgG4 levels, infiltration of IgG4+ plasma cells in the affected tissue and good response to immunosuppressive treatment. Its first description may go back to 150 years ago. The clinical presentation of IAC is often misleading, mimicking other biliary diseases such as primary sclerosing cholangitis (PSC) or cholangiocarcinoma. The HISORt criteria--histopathological, imaging, and serological features (sIgG4), other organ manifestations of IgG4-RD and response to treatment--are the standard for the diagnosis of IAC. In this overview of a recent lecture, we summarize our original findings on IgG4-RD that (i) dominant IgG4+ B-cell clones identified by advanced next generation sequencing (NGS) are highly specific for IgG4-RD (meanwhile confirmed by others), are a highly accurate diagnostic marker to distinguish IgG4-RD from PSC and biliary/pancreatic malignancies and may be crucial in unravelling the pathophysiology of IgG4-RD; (ii) sIgG4/sIgG1 >0.24 have additional diagnostic value in comparison to sIgG4 in differentiating IAC from PSC; (iii) blood IgG4 mRNA is a highly accurate diagnostic marker comparable to NGS and may become an easily available and affordable diagnostic standard for distinguishing IgG4-RD from PSC and biliary/pancreatic malignancies; and (iv) 'blue collar work' with long-term exposure to solvents, paints, oil products or industrial gases may be a risk factor for development of IgG4-RD. These findings may contribute to the understanding of the pathophysiology and to the early diagnosis and adequate treatment of IgG4-RD.

  17. IgG4-related disease-experience of 100 consecutive cases from a specialist centre.

    Science.gov (United States)

    Bateman, Adrian C; Culver, Emma L

    2017-04-01

    To describe the features of 100 consecutive cases referred to a single UK institution in which a diagnosis of IgG4-related disease (IgG4-RD) was under consideration. The histological features were reviewed by a single histopathologist, and cases were categorized according to the 2012 Boston criteria: Category 1-histologically highly suggestive of IgG4-RD; Category 2-probable histopathological features of IgG4-RD; and Category 3-insufficient histopathological evidence of IgG4-RD. A 'global assessment' was performed with the available clinical information: Assessment group 1-'definite/very likely IgG4-RD'; Assessment group 2-'possible IgG4-RD'; Assessment group 3-'not IgG4-RD'; and Assessment group 4-insufficient information. The mean IgG4+ plasma cell count and IgG4+/IgG+ ratio were highest in Category 1 [134/high-power field (HPF); 57%] and Assessment group 1 (113/HPF; 52%), and lowest in Category 3 (11/HPF; 18%) and Assessment group 3 (43/HPF; 31%) (Category comparison of IgG4+ count and ratio, both P < 0.001; Assessment group comparison of IgG4+ count, P < 0.0002; and Assessment group comparison of ratio, P = 0.04). A non-IgG4-RD diagnosis was rare in Category 1 (7%) but common in Category 2 (60%) and Category 3 (47%). Stromal reactions to neoplasia and chronic oral ulceration were simulants of IgG4-RD. The Boston criteria are linked to the likelihood of IgG4-RD. Other conditions may show some histological features of IgG4-RD. The likelihood of IgG4-RD is much greater when the histological features reach the threshold for Category 1 than when they reach the thresholds for Categories 2 and 3. Despite the utility of the Boston criteria, this study highlights the crucial importance of careful clinicopathological correlation when a diagnosis of IgG4-RD is under consideration. © 2016 John Wiley & Sons Ltd.

  18. Clinical analysis of patients with IgG4-related disease%IgG4相关性疾病临床特征分析

    Institute of Scientific and Technical Information of China (English)

    姚春容; 崔贝贝; 林辉; 薛丽佳; 朱利君; 刘毅

    2016-01-01

    目的 针对IgG4相关性疾病(IgG4-RD)的临床特点、影像学特征、组织病理学表现及预后进行分析,为IgG4-RD的诊断及治疗提供依据.方法 回顾性收集2011~2015年在四川大学华西医院诊治的48例诊断为IgG4相关性疾病患者临床资料,统计分析该病患者症状,血清IgG和IgG4、肿瘤血清学标志物及自身抗体表达,总结该病患者CT、MRI、超声等影像学特点,随访疾病预后.结果 48例IgG4-RD平均年龄(50±16)岁,其中男女比例为1.5∶1,血清IgG4水平在0.05~80.5 g/L之间.淋巴结受累患者最多,14例患者伴有多部位受累.共有47例患者进行组织病理学检查,主要表现为淋巴浆细胞浸润及纤维化形成,IgG4+细胞>10个高倍视野.38例患者进行糖皮质激素联合免疫抑制剂治疗,激素减量后,2例患者疾病复发.结论 IgG4-RD是多器官受累的自身免疫病,受累脏器IgG4阳性浆细胞浸润是其主要特点,及时应用糖皮质激素及免疫抑制剂可有效缓解病情.

  19. Immunoglobulin G4-related disease preceded by lung involvement: A case report.

    Science.gov (United States)

    Abo, Miki; Takato, Hazuki; Watanabe, Satoshi; Kase, Kazumasa; Sakai, Tamami; Koba, Hayato; Hara, Johsuke; Sone, Takashi; Kimura, Hideharu; Kasahara, Kazuo

    2017-06-01

    Immunoglobulin G4-related disease (IgG4-RD) is a systemic condition involving various organs and vessels including the pancreas, bile duct, salivary glands, periorbital tissues, kidneys, lungs, lymph nodes, meninges, and aorta. Recently, some cases of IgG4-RD have been reported, in which only pulmonary lesions were present. It is not known whether IgG4-RD can be diagnosed on the basis of pulmonary lesions only, because increases in serum IgG4 levels and infiltration of IgG4-positive plasma cells into the lung tissue also occur in other inflammatory conditions. A case of IgG-RD that was followed-up for 7 years after onset is described. Initially, only pulmonary lesions were present; however, other lesions in the submandibular glands, pancreas, periarterial region, and other areas occurred over time, with a gradual increase in serum IgG4 levels. Histopathology results from the patient's submandibular gland confirmed the diagnosis of IgG4-RD. Following diagnosis, the patient was treated with corticosteroids immediately, and his symptoms disappeared rapidly. Because other diseases, including malignancies, mimic IgG4-RD in clinical and histopathological features, an absolute diagnosis is necessary to avoid missing the presence of underlying diseases. This case more provides insight into the clinical pathology of IgG4-RD.

  20. IgG4-related mastitis, a rare disease, can radiologically and histologically mimic malignancy.

    Science.gov (United States)

    Yamada, Rin; Horiguchi, Shin-ichiro; Yamashita, Toshinari; Kamisawa, Terumi

    2016-03-23

    IgG4-related disease (IgG4-RD) is characterised by high serum concentrations of IgG4, dense lymphoplasmacytic infiltrates, storiform fibrosis and increased IgG4-positive plasma cells in tissues. This systemic disease occurs in various organs metachronously, but IgG4-related mastitis appears extremely rare. We report a case of IgG4-related mastitis, radiologically considered to represent breast cancer mainly composed of intraductal component and requiring histological differentiation from mucosa-associated lymphoid tissue (MALT) lymphoma. The breast mass disappeared with steroid therapy. When patients have a breast mass, regardless of the presence or absence of IgG4-RD, IgG4-related mastitis should be considered in addition to breast cancer. If histological findings show dense lymphoplasmacytic infiltrates, IgG4-related mastitis should be suspected in addition to malignant lymphoma, and lack of monoclonality should be confirmed. To avoid unnecessary surgery or chemotherapy, knowledge and accurate diagnosis of the entity of IgG4-related mastitis is necessary. 2016 BMJ Publishing Group Ltd.

  1. Serum levels of IgG and IgG4 in Hashimoto thyroiditis.

    Science.gov (United States)

    Kawashima, Sachiko-Tsukamoto; Tagami, Tetsuya; Nakao, Kanako; Nanba, Kazutaka; Tamanaha, Tamiko; Usui, Takeshi; Naruse, Mitsuhide; Minamiguchi, Sachiko; Mori, Yusuke; Tsuji, Jun; Tanaka, Issei; Shimatsu, Akira

    2014-03-01

    Although IgG4-related disease is characterized by extensive infiltration of IgG4-positive plasma cells and lymphocytes of various organs, the details of this systemic disease are still unclear. We screened serum total IgG levels in the patients with Hashimoto thyroiditis (HT) to illustrate the prevalence of IgG4-related thyroiditis in HT. Twenty-four of 94 patients with HT (25.5%) had elevated serum IgG levels and their serum IgG4 was measured. Five of the 24 cases had more than 135 mg/dL of IgG4, which is the serum criterion of IgG4-related disease. One was a female patient who was initially treated as Graves' disease and rapidly developed a firm goiter and hypothyroidism. The biopsy of her thyroid gland revealed that follicular cells were atrophic with squamous metaplasia, replaced with fibrosis, which was compatible with the fibrous variant of HT. Immunohistochemical examination revealed diffuse infiltration of IgG4-positive plasma cells, and the serum IgG4 level was 179 mg/dL. The levels of IgG and IgG4 were positively correlated with the titers of anti-thyroglobulin antibody or anti-thyroid peroxidase antibody. In conclusion, at least a small portion of patients with HT with high titers of anti-thyroid antibodies may overlap the IgG4-related thyroiditis.

  2. Recognizing Immunoglobulin G4-Related Overlap Syndromes in Patients with Pancreatic and Hepatobiliary Diseases

    Directory of Open Access Journals (Sweden)

    Aldo J Montano-Loza

    2008-01-01

    Full Text Available The first description of autoimmune pancreatitis and elevated serum immunoglobulin-G4 (IgG4 in 2001 heralded further reports of several related autoimmune diseases with raised IgG4 levels. It is now recognized that a spectrum of overlap syndromes associated with increased IgG4 and biopsy evidence of IgG4-producing plasma cells, which has now been convincingly linked with cholangitis, autoimmune hepatitis, Sjögren’s syndrome, nephritis and retroperitoneal fibrosis. Collectively, this disease cluster is referred to as IgG4-related systemic disease. The importance of making the correct diagnosis is underscored by the management of individuals with IgG4-related systemic disease. In the first instance, patients generally have a dramatic response to immunosuppressive therapy, whereas patients with other forms of cholangitis and pancreatitis do not. Also, surgical management of pancreatic malignancy can be avoided once the correct diagnosis of IgG4-related disease has been made. In the present review, an overview of the current information regarding the role of IgG4 and IgG4-positive cells affecting the biliary system, pancreas and liver is provided.

  3. Pancreatic cancer with a high serum IgG4 concentration

    Institute of Scientific and Technical Information of China (English)

    Terumi Kamisawa; Pong Yui Chen; Yuyang Tu; Hitoshi Nakajima; Naoto Egawa; Kouji Tsuruta; Atsutake Okamoto; Tsunekazu Hishima

    2006-01-01

    Differentiation between autoimmune pancreatitis and pancreatic cancer is sometimes difficult. It has been reported that serum IgG4 concentrations are significantly elevated and particularly high (>135 mg/dL) in autoimmune pancreatitis. Measurement of serum IgG4 has become a useful tool for differentiating between autoimmune pancreatitis and pancreatic cancer. However,we present a 74-year-old female with a markedly elevated serum IgG4 (433 mg/dL) who underwentpancreaticoduodenectomy for pancreatic cancer. Elevated serum IgG4 levels continued after the resection. On histology, adenocarcinoma of the pancreas accompanied with moderate lymphoplasmacytic infiltration infiltrated the lower bile duct and duodenum, but there were no findings of autoimmune pancreatitis. Although a small metastasis was detected in one parapancreatic lymph node, regional lymph nodes were swollen. Abundant IgG4-positive plasma cells infiltrated the cancerous areas of the pancreas, but only a few IgG4-positive plasma cells were detected in the noncancerous areas.Pancreatic cancer cells were not immunoreactive for IgG4. An abundant infiltration of IgG4-positive plasma cells was detected in the swollen regional lymph nodes and in the duodenal mucosa. We believe that the serum IgG4 level was elevated in this patient with pancreatic cancer as the result of an IgG4-related systemic disease that had no clinical manifestations other than lymphadenopathy.

  4. IgG4相关性眼病的研究进展

    Institute of Scientific and Technical Information of China (English)

    杨慧敏; 李劲; 张琳; 柳林

    2015-01-01

    IgG4相关性疾病(IgG4 related disease,IgG4-RD)是一组独特的疾病群,以血清IgG4浓度升高和受累器官IgG4+浆细胞浸润、伴不规则纤维化为特征.IgG4相关性眼病(IgG4 related eyedisease,IgG4-RED)是隶属于IgG4-RD的一类疾病,可以发生在任一眼附属器,包括泪腺、泪囊、眼外肌、眼眶脂肪组织、眶周膜和眼睑,临床症状取决于受损部位;主要侵袭中老年人群,症状相对温和,由于器官肿胀或损害,这种情况常引起临床关注.激素疗法对大多数IgG4相关性眼病患者有效.

  5. A case of progressively transformed germinal center-type IgG4-related lymphadenopathy.

    Science.gov (United States)

    Seki, Nobuhiko; Yamazaki, Norikazu; Koizumi, Jun-ichi; Takano, Ken-ichi; Abe, Ayumi; Ikeda, Tatsuru; Noguchi, Hiroko; Himi, Tetsuo

    2015-08-01

    Progressively transformed germinal centers (PTGC), a lymph node process unfamiliar to most otolaryngologists, is a morphological variant of reactive lymphofollicular hyperplasia of lymph nodes. Immunoglobulin (Ig)G4-related disease (IgG4-RD) is a newly identified condition, characterized by hyper-IgG4-γ-globulinemia and mass-forming or hypertrophic lesions associated with infiltration of IgG4(+) plasma cells in the affected organs. Recently, a case study of PTGC was reported that fulfilled the diagnostic criteria of IgG4-RD (IgG4(+) PTGC) [1]. A 68-year-old male was referred to our hospital with swelling in the left submandibular region. Palpation revealed swollen lymph nodes, the largest of which measured 5cm in diameter. (18)F-fluorodeoxyglucose (FDG)-positron emission tomography/computed tomography identified lymphadenopathy with high (18)F-FDG uptake in the left submandibular region. We strongly suspected malignant lymphoma, and excisional biopsy of the submandibular lymph node was performed under general anesthesia. Pathological findings were consistent with IgG4(+) PTGC, and serological examination demonstrated elevated levels of IgG4. These findings were consistent with IgG4-RD. The patient did not have systemic lesions; therefore, he has not undergone corticosteroid therapy. IgG4(+) PTGC should be considered as a differential diagnosis for cervical lymphadenopathy by otolaryngologists as well as pathologists.

  6. [Review of ear and nose and throat involvement in IgG4-RD].

    Science.gov (United States)

    Tao, Xiaofeng; Liu, Chang; Song, Bo

    2015-11-01

    IgG4-related disease (IgG4-RD) is a newly recognized disease entity. IgG4-RD is characterized by a single or multiple masses in one or more organs; a lymphoplasmacytic infiltrate with a high percentage of plasma cells within the lesion staining for IgG4; a peculiar pattern of fibrosis known as "storiform" fibrosis; and elevated serum IgG4 concentrations. IgG4-RD can occur in various organs, including pancreas, kidneys, lungs, retroperitoneum, and prostate gland. The head and neck involvements of IgG4-RD have been chiefly described in Mikulicz disease (MD), Küttner's tumor, orbital? inflammatory pseudotumor, and idiopathic hypertrophic pachymeningitis (IHP) previously. Recent studies reported that IgG4-RD could also involve ear, nose and throat. Here we reviewed the literatures about ear, nose and throat involvement by IgG4-RD, in order to provide some theoretical bases for the diagnosis and treatment of IgG4-RD.

  7. Synthesis of Macrocyclic Hexaoxazole (6OTD Dimers, Containing Guanidine and Amine Functionalized Side Chains, and an Evaluation of Their Telomeric G4 Stabilizing Properties

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    Keisuke Iida

    2010-01-01

    Full Text Available Structure-activity relationship studies were carried out on macrocyclic hexaoxazole (6OTD dimers, whose core structure stabilizes telomeric G-quadruplexes (G4. Two new 6OTD dimers having side chain amine and guanidine functional groups were synthesized and evaluated for their stabilizing ability against a telomeric G4 DNA sequence. The results show that the 6OTD dimers interact with the DNA to form 1:1 complexes and stabilize the antiparallel G4 structure of DNA in the presence of potassium cation. The guanidine functionalized dimer displays a potent stabilizing ability of the G4 structure, as determined by using a FRET melting assay (ΔTm=14 °C.

  8. IgG4升高的肾病:IgG4相关性肾病与特发性膜性肾病的研究进展%Advances in Kidney Diseases with Elevated IgG4 Levels:IgG4 -related Kidney Disease and Idiopathic Membranous Nephropathy

    Institute of Scientific and Technical Information of China (English)

    申嫒文; 林鹭; 郁胜强

    2016-01-01

    IgG4相关性疾病是以血清IgG4升高、组织IgG4浆细胞浸润以及纤维性病变为特征的多系统疾病,可累及胰腺、肾脏、唾液腺等多种器官,累及肾脏时称为IgG4相关性肾病,主要表现为IgG4相关性肾小管间质性肾炎,累及肾小球时以膜性肾病多见。而特发性膜性肾病的免疫复合物沉积也以IgG4为主,在临床上区分IgG4相关性疾病继发的膜性肾病与特发性膜性肾病非常重要。本文从发病机制、临床表现、诊断及治疗等方面探讨IgG4相关性肾病与特发性膜性肾病的区别,为临床的诊治提供一定的理论基础。%IgG4 -related disease is a multi -system disease , which often manifests as elevated serum IgG 4 concentrations , IgG4-positive plasma cell infiltration and fibrosis lesions .IgG4-related disease can affect many organs , such as pancreas, kidney, and salivary glands.When kidney is involved, we call it IgG4-related kidney disease, and its most common renal manifestation is IgG4 -related tubulointerstitial nephritis.In addition, glomerulus involvement , most commonly membranous nephropathy (MN), could also be found.Meanwhile, idiopathic membranous nephropathy is associated with high IgG4.It is important to distinguish between the MN secondary to IgG 4-related disease and idiopathic membranous nephropathy , which is a separate disorder .In this paper , through the discussion from pathogenesis , clinical manifestation , diagnosis and treatment, we compared the differences between IgG 4-related kidney disease and idiopathic membranous nephropathy , in order to provide some theoretical foundations for clicinal diagnosis and treatment .

  9. [IgG4-related disease is a rare differential diagnosis of malignant and autoimmune diseases].

    Science.gov (United States)

    Storgaard, Anders; Detlefsen, Sönke

    2015-04-06

    Immunoglobulin G4-related disease (IgG4-RD) is an inflammatory and fibrotic disease with the potential to produce diffuse enlargement, massforming lesions or stenoses in a wide range of organs. Elevation of serum IgG4 concentration and high levels of IgG4-positive cells in the inflamed tissue are common denominators. Type 1 autoimmune pancreatitis is one of the main manifestations, and its recognition preceded the definition of IgG4-RD as a novel clinical entity. The aetiology, pathophysiology, epidemiology and clinical long-term outcome of IgG4-RD are not fully elucidated. Steroids are effective in most patients, sometimes combined with other antiinflammatory drugs.

  10. Normal pressure hydrocephalus, a possible complication in IgG4-related disease

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    Hiroshi Kobayashi

    2016-09-01

    Full Text Available We report a 75-year-old man with glucocorticoid-responsive normal pressure hydrocephalus (NPH complicated with tubulointerstitial renal disease, protein-losing gastroenteropathy (PLG and elevated serum IgG4, which might be a possible subset of IgG4-related disease (IgG4-RD. Although either PLG or NPH, especially in combination, has rarely been reported in IgG4-RD, the glucocorticoid-responsive nature of every abnormality observed in the patient supports the diagnosis of IgG4-RD. Of course, pathological confirmation is essential to fulfill the recently raised diagnostic criteria, however, such invasive procedure might not always be indicated in high-risk patients. Our report illustrates that IgG4-RD might be considered as one of the underlying causes and/or the aggravating factors of NPH before shunt operation.

  11. First case of IgG4-related sclerosing cholangitis associated with autoimmune hemolytic anemia.

    Science.gov (United States)

    Masutani, Hironori; Okuwaki, Kosuke; Kida, Mitsuhiro; Yamauchi, Hiroshi; Imaizumi, Hiroshi; Miyazawa, Shiro; Iwai, Tomohisa; Takezawa, Miyoko; Koizumi, Wasaburo

    2014-07-14

    To our knowledge, patients with immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) associated with autoimmune hemolytic anemia (AIHA) have not been reported previously. Many patients with IgG4-SC have autoimmune pancreatitis (AIP) and respond to steroid treatment. However, isolated cases of IgG4-SC are difficult to diagnose. We describe our experience with a patient who had IgG4-SC without AIP in whom the presence of AIHA led to diagnosis. The patient was a 73-year-old man who was being treated for dementia. Liver dysfunction was diagnosed on blood tests at another hospital. Imaging studies suggested the presence of carcinoma of the hepatic hilus and primary sclerosing cholangitis, but a rapidly progressing anemia developed simultaneously. After the diagnosis of AIHA, steroid treatment was begun, and the biliary stricture improved. IgG4-SC without AIP was thus diagnosed.

  12. What are the characteristics of asthma patients with elevated serum IgG4 levels?

    Science.gov (United States)

    Flament, T; Marchand-Adam, S; Gatault, P; Dupin, C; Diot, P; Guilleminault, L

    2016-03-01

    IgG4 has recently been a subject of great interest in human pathology. No data are available about the characteristics of asthma patients with elevated IgG4 levels. An observational study was conducted from January 2006 to March 2015 in a difficult-to-treat population of asthma patients. Twenty-six difficult-to-treat asthma patients with elevated serum IgG4 levels (IgG4/IgG ratio up to 10%) were compared with a control population of 98 difficult-to-treat asthma patients with normal serum IgG4. Blood eosinophilia, total IgE and FeNO were compared between groups to better characterize asthma patients with elevated serum IgG4 levels. Median IgG4 concentrations were 1.72 g/l [1.19-2.36] and 0.22 g/l [0.10-0.49] in the elevated IgG4 group and normal Ig4 group, respectively. Median blood eosinophilia was more than three times higher in patients with elevated serum IgG4 levels than in controls (0.75 10(9)/L [IQR 0.54-1.78] vs 0.22 10(9)/L [IQR 0.09-0.54] respectively, p ppb vs 35 [IQR 23-51] ppb, p < 0.001). Allergic broncho-pulmonary aspergillosis (ABPA) and eosinophilic granulomatosis with polyangiitis (EGPA) were observed in the asthma patients with elevated serum IgG4. Ten patients had unexplained increased blood eosinophilia. Asthma patients with elevated IgG4 levels have significantly higher blood eosinophilia, total IgE and FeNO. ABPA and EGPA are observed in patients with elevated serum IgG4. Copyright © 2016 Elsevier Ltd. All rights reserved.

  13. Bilateral Vision Loss Secondary to Pachymeningitis in a Patient with IgG4-Related Disease

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    Lucas eRamirez

    2014-10-01

    Full Text Available IgG4-Related Disease is a recently recognized fibroinflammatory condition associated with disease in nearly every organ, including the meninges. A proportion of Idiopathic Hypertrophic Pachymeningitis cases may involve a component of meningeal IgG4-RD. We present a patient with severe bilateral vision loss found to have thickening of the dura mater on MRI, and subsequently diagnosed with IgG4-RD after dural biopsy.

  14. IgG4 immunostaining and its implications in orbital inflammatory disease.

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    Amanda J Wong

    Full Text Available OBJECTIVE: IgG4-related disease is an emerging clinical entity which frequently involves tissue within the orbit. In order to appreciate the implications of IgG4 immunostaining, we analyzed gene expression and the prevalence of IgG4- immunostaining among subjects with orbital inflammatory diseases. METHODS: We organized an international consortium to collect orbital biopsies from 108 subjects including 22 with no known orbital disease, 42 with nonspecific orbital inflammatory disease (NSOI, 26 with thyroid eye disease (TED, 12 with sarcoidosis, and 6 with granulomatosis with polyangiitis (GPA. Lacrimal gland and orbital adipose tissue biopsies were immunostained for IgG4 or IgG secreting plasma cells. RNA transcripts were quantified by Affymetrix arrays. RESULTS: None of the healthy controls or subjects with TED had substantial IgG4 staining. Among the 63 others, the prevalence of significant IgG4-immunostaining ranged from 11 to 39% depending on the definition for significant. IgG4 staining was detectable in the majority of tissues from subjects with GPA and less commonly in tissue from subjects with sarcoidosis or NSOI. The detection of IgG4+ cells correlated with inflammation in the lacrimal gland based on histology. IgG4 staining tissue expressed an increase in transcripts associated with inflammation, especially B cell-related genes. Functional annotation analysis confirmed this. CONCLUSION: IgG4+ plasma cells are common in orbital tissue from patients with sarcoidosis, GPA, or NSOI. Even using the low threshold of 10 IgG4+ cells/high powered field, IgG4 staining correlates with increased inflammation in the lacrimal gland based on histology and gene expression.

  15. Refractory gastric ulcer with abundant IgG4-positive plasma cell infiltration: A case report

    Institute of Scientific and Technical Information of China (English)

    Takayoshi; Fujita; Takafumi; Ando; Masatoshi; Sakakibara; Waki; Hosoda; Hidemi; Goto

    2010-01-01

    We describe a 77-year-old man with refractory gastric ulcer that worsened after Helicobacter pylori eradication therapy.Pathology showed marked infiltration of IgG4-positive plasma cells in the gastric lesions,which led us to suspect IgG4-related sclerosing disease.To the best of our knowledge,this is the first report of IgG4-related gastric ulcer without the main manifestation of autoimmune pancreatitis.

  16. [Two cases of IgG4-related disease with pleural effusion].

    Science.gov (United States)

    Suzuki, Nobuaki; Saeki, Takako; Shimaoka, Yuichi; Kuriyama, Hideyuki; Nishibori, Takeaki; Ebe, Yusuke; Sato, Kazuhiro

    2011-02-01

    We describe 2 cases of IgG4-related disease with pleural effusion in elderly men. Both patients had elevated serum IgG4 levels, and the characteristics of their pleural effusion were similar. Patient 1 had pericardial effusion and retroperitoneal fibrosis, and a biopsy specimen from the pericardium showed infiltration of abundant IgG4-positive plasma cells with fibrosis. Because his pleurisy, pericarditis and retroperitoneal fibrosis responded to steroid therapy, we diagnosed pleurisy associated with IgG4-related disease. Patient 2 had been treated with steroids because of IgG4-related sialadenitis and interstitial pneumonitis, but pleural effusion developed. Although histopathological examination of the pleura showed infiltration of abundant IgG4-positive plasma cells with fibrosis, Mycobacterium tuberculosis was cultured from the pleural effusion, and histologic examination also showed epithelioid granuloma. Chemotherapy for tuberculosis was effective for the pleurisy, and we diagnosed tuberculous pleurisy as a complication of IgG4-related disease. In cases of IgG4-related disease associated with pleural effusion, the clinical course should be considered together with the serum IgG4 levels and pleural histology.

  17. Distinct histopathological features of Hashimoto's thyroiditis with respect to IgG4-related disease.

    Science.gov (United States)

    Li, Yaqiong; Zhou, Gengyin; Ozaki, Takashi; Nishihara, Eijun; Matsuzuka, Fumio; Bai, Yanhua; Liu, Zhiyan; Taniguchi, Emiko; Miyauchi, Akira; Kakudo, Kennichi

    2012-08-01

    A form of Hashimoto's thyroiditis with lymphoplasmacytic sclerosing changes and increased numbers of IgG4-positive plasma cells has recently been reported in the literature. These histopathological features suggest that this subtype of Hashimoto's thyroiditis may be closely related to IgG4-related disease. Therefore, this unique form of IgG4-related Hashimoto's thyroiditis, which is referred to as IgG4 thyroiditis, has its own clinical, serological, and sonographic features that are distinct from those associated with non-IgG4 thyroiditis. IgG4 thyroiditis shares similarities with the well-known fibrous variant of Hashimoto's thyroiditis; however, the detailed histopathological features of IgG4 thyroiditis have not been well established. Based on immunostaining results, 105 patients with Hashimoto's thyroiditis were divided into an IgG4 thyroiditis group (n=28) and a non-IgG4 thyroiditis group (n=77). As in our previous reports, IgG4 thyroiditis was associated with a patient population of a younger age, a lower female-to-male ratio, rapid progression, higher levels of thyroid autoantibodies, subclinical hypothyroidism, and diffuse sonographic echogenicity. Histopathologically, this group revealed severe lymphoplasmacytic infiltration, dense stromal fibrosis, marked follicular cell degeneration, numerous micro-follicles, and notable giant cell/histiocyte infiltration. Importantly, the IgG4-related group did not completely overlap with fibrous variant of Hashimoto's thyroiditis. Four cases (14%) in the IgG4 thyroiditis group presented only mild fibrosis in the stroma, whereas 29 cases (38%) in the non-IgG4 thyroiditis group met the diagnostic criteria for fibrous variant of Hashimoto's thyroiditis. Furthermore, we observed three patterns of stromal fibrosis in Hashimoto's thyroiditis: interfollicular fibrosis, interlobular fibrosis, and scar fibrosis. The IgG4 thyroiditis group was significantly associated with the presence of predominant interfollicular fibrosis. In

  18. Small bowel obstruction caused by peritoneal immunoglobulin G4-related disease mimicking carcinomators: Case report

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    Coulier, Bruno; Montfort, Luc; Pierard, Frederic [Clinique St. Luc, Bouge (Belgium); Beniuga, Gabrique; Gielen, Lsabelle [Institute of Pathology and Genetics, Gosselies (Belgium)

    2014-02-15

    We hereby report a case of diffuse pelvic peritoneal involvement by immunoglobulin G4-related disease (IgG4-RD). Numerous pelvic masses and nodules showing delayed enhancement on enhanced abdominal CT were found to congregate in the pelvic organs of a 57-year-old female presenting with intestinal subocclusion. The differentiation between peritoneal IgG4-RD and pelvic peritoneal carcinomatosis was only made by histopathology and immunohistochemistry performed after surgical resection. Autoimmune pancreatitis represents the historical prototype of IgG4-RD, but the spectrum of manifestations involving various organs has expanded during the last decade. In this report, we shortly review this clinical entity.

  19. Histologically confirmed isolated IgG4-related hypophysitis: two case reports in young women

    OpenAIRE

    Sosa, Gabriela Alejandra; Bell, Soledad; Christiansen, Silvia Beatriz; Pietrani, Marcelo; Glerean, Mariela; Loto, Monica; Lovazzano,Soledad; Carrizo, Antonio; Ajler, Pablo; Fainstein Day, Patricia

    2014-01-01

    Summary IgG4-related hypophysitis is a recently described entity belonging to the group of IgG4-related diseases. Many other organs can also be affected, and it is more common in older men. To date, 32 cases of IgG4-related hypophysitis have been reported in the literature, 11 of which included confirmatory tissue biopsy and the majority affecting multiple organs. The aim of this report is to present two cases of biopsy-proven IgG4-related hypophysitis occurring in two young female patients w...

  20. Immunoglobulin G4 sinusitis in association with aspirin-exacerbated respiratory disease.

    Science.gov (United States)

    Johal, Kirti; Welch, Kevin; Peters, Anju

    2017-09-01

    Immunoglobulin G4 (IgG4) related disease is a systemic inflammatory disease characterized by tumor-like tissue infiltration with IgG4 positive (IgG4+) plasma cells. Aspirin-exacerbated respiratory disease (AERD) is defined as asthma, chronic rhinosinusitis with nasal polyposis, and hypersensitivity to cyclooxygenase-1 inhibitors. We described a case of a non-smoking 61-year-old male with prior NSAID sensitivity who presented with a 1-year history of left eye proptosis associated with chronic nasal symptoms, ultimately identified as concurrent AERD and IgG4 sinusitis. The patient was evaluated in the clinic and diagnosed by using clinical, radiographic, and surgical biopsy findings. Although initial concern was greatest for malignancy, a biopsy specimen confirmed the presence of a dense lymphoplasmacytic infiltrate and storiform fibrosis, associated with increased IgG4+ plasma cells. Therefore, IgG4-related disease (RD) was identified in this patient with AERD. Shared type II inflammation may be responsible for the coexistence of IgG4-RD and AERD as observed in our patient. Health care workers must be cognizant of the simultaneous presentation of both IgG4-RD and AERD.

  1. Prevalence of IgG4-Related Disease in Japan Based on Nationwide Survey in 2009

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    Kazushige Uchida

    2012-01-01

    Full Text Available The number of patients with autoimmune pancreatitis who visited hospitals in Japan in 2007 was approximately 2709 (95% confidence interval; range 2540–3040. Because IgG4-related disease is a new clinical entity, there are no data with regard to its prevalence. To estimate the number of patients with IgG4-related disease in Japan, we randomly selected hospitals using stratification and asked them how many patients they had with IgG4-related disease in 2009. The number of patients with Mikulicz’s disease, IgG4-related retroperitoneal fibrosis, IgG4-related renal disease, IgG4-related pulmonary disease, and IgG4-related lymphadenopathy who visited hospitals in Japan in 2009 was approximately 4304 (95% confidence interval; range 3360–5048, 272 (95% confidence interval; range 264–306, 57 (95% confidence interval; range 47–66, 354 (95% confidence interval; range 283–424, and 203 (95% confidence interval; range 187–240, respectively. The total number of patients with IgG4-related disease without autoimmune pancreatitis in Japan was approximately 5190 (95% confidence interval; range 4141–6084. The male : female ratio was 1 : 0.77, and the average of age of disease onset was 58.8 years. The total number of patients with IgG4-related disease in Japan in 2009, including autoimmune pancreatitis, was approximately 8000.

  2. Severe IgG4-Related Disease in a Young Child: A Diagnosis Challenge

    Directory of Open Access Journals (Sweden)

    Susana Corujeira

    2015-01-01

    Full Text Available Immunoglobulin G4-related disease (IgG4-RD is an increasingly recognized syndrome that can appear with multiple organ involvement, typically with tumor-like swelling, lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells, and elevated serum IgG4 concentrations. We report the case of a 22-month-old female child with failure to thrive and recurrent respiratory tract infections since 8 months of age. Physical examination was normal except for pulmonary auscultation with bilateral crackles and wheezes. Laboratory tests revealed elevated erythrocyte sedimentation rate, and elevated serum IgG and IgG4 with polyclonal hypergammaglobulinemia. Thoracic CT and MRI showed multiple mediastinal lymphadenopathies and a nodular posterior mediastinal mass in right paratracheal location with bronchial compression. Initial fine needle aspiration biopsy was compatible with reactive lymphadenopathy but after clinical worsening a thoracoscopic partial resection of the mass was performed and tissue biopsy revealed lymphoplasmacytic infiltrate and increased number of IgG4-positive plasma cells and a ratio of IgG4/IgG positive cells above 40%. Glucocorticoids therapy was started with symptomatic improvement, reduction in the size of the mass, and decrease of serum IgG4 levels after 6 weeks. There are very few reports of IgG4-RD in children. Long-term follow-up is necessary to monitor relapses and additional organ involvement.

  3. Immunoglobulin G4-Related Sclerosing Disease Involving the Urethra: Case Report

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    Choi, Jin Woo; KIm, Sang Youn; Cho, Jeong Yeon; Kim, Seung Hyup [Dept. of Radiology, Seoul National University College of Medicine, and Institute of Radiation Medicine, Seoul National University Medical Research Center, Seoul (Korea, Republic of); Moon, Kyung Chul [Dept. of Pathology, Seoul National University College of Medicine, Seoul (Korea, Republic of)

    2012-11-15

    Immunoglobulin G4 (IgG4)-related sclerosing disease is a systemic disease characterized by extensive IgG4-positive plasma cells and T-lymphocyte infiltration in various organs. We described the imaging findings of an IgG4-related inflammatory pseudotumor in the urethra. The urethral mass showed isoattenuation on unenhanced CT images, delayed enhancement on enhanced CT images, iso- to slight hyper-intensity on T1 and T2 weighted magnetic resonance images, diffusion restriction on diffusion weighted images, and heterogeneously low echogeneity on ultrasonography.

  4. Diagnostic Value of Serum IgG4 for IgG4-Related Disease: A PRISMA-compliant Systematic Review and Meta-analysis.

    Science.gov (United States)

    Hao, Mingju; Liu, Min; Fan, Gaowei; Yang, Xin; Li, Jinming

    2016-05-01

    Many studies about serum IgG4 for the diagnosis of IgG4-related disease (IgG4-RD) have been reported. However, these studies had relatively small sample sizes and the diagnostic accuracy values varied much between them.The aim of this study was to perform a meta-analysis to evaluate the diagnostic value of serum IgG4 for IgG4-RD.We conducted a search of relevant articles using MEDLINE, EMBASE, Web of Science, SCOPUS, and Cochrane Library databases published before December 2015.Studies those assessed the diagnostic accuracy of serum IgG4 for IgG4-RD and those provided the cut-off value for serum IgG4 were included.Data were synthesized using the random-effect model. Statistical analysis was performed using STATA with the MIDAS module and Meta-DiSc 1.4 software.A total of 9 case-control studies were analyzed, which included 1235 patients with IgG4-RD and 5696 overall controls. The pooled estimate, for a cut-off value ranged from 135 to 144 mg/dL, produced a sensitivity of 87.2% (95% CI, 85.2-89.0%) and a specificity of 82.6% (95% CI, 81.6-83.6%). The positive likelihood ratio (PLR), negative likelihood ratio (NLR), and diagnostic odds ratio (DOR) were 6.48 (95% CI, 3.98-10.57), 0.14 (95% CI, 0.09-0.21), and 45.15 (95% CI, 23.41-87.06), respectively. The area under the curve (AUC) of the summary receiver operating characteristic curve (SROC) was 0.94 (0.92-0.96). When a cut-off value of 2-fold the upper limit of normal was used (ranged from 270 to 280 mg/dL), the pooled sensitivity was 63% (95% CI, 60.0-66.0%), and the specificity was 94.8% (95% CI, 94.1-95.4%). The PLR, NLR, and DOR were 13.3 (95% CI, 7.39-24.0), 0.41 (95% CI, 0.29-0.58) and 33.42 (95% CI, 13.88-80.43), respectively. The AUC of the SROC was 0.92 (0.90-0.94).Only a relatively small number of studies were included, and significant heterogeneity was observed in this meta-analysis.Serum IgG4 is a modestly effective marker to diagnose IgG4-RD. Doubling the cut-off value for IgG4 could not improve the

  5. IgG4相关性疾病研究进展%Research advances in immunoglobulin G4 related sclerosing diseases

    Institute of Scientific and Technical Information of China (English)

    韩英; 朱疆依

    2013-01-01

    IgG4相关性疾病是新近认识的一种疾病,表现为多灶性纤维硬化性改变、血清IgG4升高、组织中大量IgG4阳性浆细胞浸润,该病发病机制尚不明确,目前也没有统一的诊断标准,其临床诊断主要是多方面综合判断的结果.明确诊断有助于规范治疗,避免不必要的手术.激素等免疫抑制剂治疗预后良好.

  6. IgG4相关性胃病临床分析%Clinical Analysis of IgG4-related Gastric Disease

    Institute of Scientific and Technical Information of China (English)

    郑松柏; 李小雯; 肖立; 沈达明; 季大年

    2015-01-01

    背景:IgG4相关性胃病较罕见,临床上因对该病认识不足,易致错误诊断和治疗.目的:提高IgG4相关性胃病的诊治水平.方法:结合复旦大学附属华东医院近期1例IgG4相关性胃病与既往文献报道的8例病例,对该病作分析、归纳和讨论.结果:IgG4相关性胃病患者以中老年居多,主要临床表现为腹部不适、纳差、恶心、呕吐、贫血、体质量下降等,少数患者可伴有其他器官免疫性疾病.胃镜下表现可分为增生型和溃疡型,组织病理学特征为大量IgG4阳性淋巴细胞、浆细胞浸润,淋巴滤泡形成,可累及胃壁各层、神经周围间隙、小血管壁、区域淋巴结,基本无胃外器官受累.诊断可参考日本IgG4相关性疾病综合诊断标准,但多数病例血清IgG4正常.治疗以手术为主,包括局部切除、胃部分切除和全胃切除,患者预后良好.结论:有限的病例报道显示IgG4相关性胃病的诊治并不十分困难,关键是提高对该病的警惕性和认识并积累更多病例,以深入探讨其发病机制和临床特点.

  7. Types of Organ Involvement in Patients with Immunoglobulin G4-related Disease

    Institute of Scientific and Technical Information of China (English)

    Yu Chen; Ji-Zhi Zhao; Rui-E Feng; Ju-Hong Shi; Xue-Mei Li; Yun-Yun Fei; Yang Shi

    2016-01-01

    Background:Immunoglobulin G4-related disease (IgG4-RD) is a newly recognized systemic disease that can involve multiple organs and various clinical phenotypes.The purpose of this study was to analyze different types of organ involvement in IgG4-RD patients in China.Methods:We conducted a prospective cohort study on IgG4-RD patients to analyze the clinical manifestations and rare features of IgG4-RD.Patients were grouped into different types according to organ involvement regarding organ number and organ site.The constituent ratio in different types was also analyzed.Results:A total of 200 IgG4-RD patients,with a male:female ratio of 2.08:1,were grouped into different types.Cases having involvement of two or three organs were the most common whereas the fewest number of patients had multi-organ (≥4) involvement.Serum IgG4 and IgE levels,IgG4/IgG ratio,and percentage of eosinophils increased as the number of involved organs increased.In addition,constituent ratio analysis revealed that patients with salivary gland/lacrimal gland swelling,who also constituted the largest number of IgG4-RD patients,had higher serum IgG4 concentrations and IgG4/IgG values,had higher percentage of Eos,and were more likely to have had a history of allergies relative to patients with internal organ involvement.Conclusions:The characteristic feature of IgG4-RD is multiple organ involvement with various clinical manifestations and different types.Although serum IgG4 levels increased with the number of involved organs,serum IgG4 levels were higher for those patients with salivary gland/lacrimal gland swelling compared with those with intemal organ involvement.Thus,valuable clues to the differential diagnosis of IgG4-RD could be obtained by examining the clinical patterns of organ involvement.

  8. Cell uptake mechanisms of PAMAM G4-FITC dendrimer in human myometrial cells

    Energy Technology Data Exchange (ETDEWEB)

    Oddone, Natalia; Zambrana, Ana I.; Tassano, Marcos [Instituto de Investigaciones Biologicas Clemente Estable, Laboratorio de Senalizacion Celular y Nanobiologia (Uruguay); Porcal, Williams [Universidad de la Republica, Grupo de Quimica Medicinal, Instituto de Quimica Biologica, Facultad de Ciencias-Facultad de Quimica (Uruguay); Cabral, Pablo [Universidad de la Republica, Laboratorio de Radiofarmacia, Centro de Investigaciones Nucleares, Facultad de Ciencias (Uruguay); Benech, Juan C., E-mail: benech@iibce.edu.uy [Instituto de Investigaciones Biologicas Clemente Estable, Laboratorio de Senalizacion Celular y Nanobiologia (Uruguay)

    2013-07-15

    The high incidence and severity of diseases which involve smooth muscle dysfunction dictates the need of continued search for novel therapeutic strategies to treat these conditions. Dendrimers are branched macromolecules with multiple end-groups that can be functionalized for applications which include drug delivery. There is no data regarding the cellular uptake mechanisms used by dendrimers in smooth muscle human myometrial cells (HMC). Polyamidoamine G4 dendrimers were conjugated with fluorescein isothiocyanate (FITC) and the resulting conjugate (G4-FITC) was characterized using high-performance liquid chromatography, nuclear magnetic resonance, and atomic force microscopy. G4-FITC showed to have no significant effect on the primary culture HMC viability up to 48 h. HMC incubated with G4-FITC were analyzed by laser confocal microscopy. Peri-nuclear fluorescence distribution was observed at 5 h of incubation or more (24, 36, and 48 h). At 24 h, G4-FITC partially co-localized with lysotracker. Uptake of G4-FITC by HMC was slightly inhibited by filipin (8.0 {+-} 3.9 %) and significantly inhibited by chlorpromazine (63.5 {+-} 3.7 %). In non-electroporated HMC, G4-FITC was never observed inside the cell nucleus. Interestingly, we detected G4-FITC inside the nuclear domain of some electroporated cells. Thus, electroporation changed intracellular G4-FITC localization. Isolated nuclei of HMC incubated with G4-FITC showed fluorescence signal inside the nuclear domain. The results suggest that in HMC, G4-FITC is taken up by clathrin-mediated endocytosis with endosomal and lysosomal localization at 24 h. The combination of electroporation and dendrimers could be an interesting technology to electrotransfer drugs into smooth muscle cells cytosol and nuclei.

  9. Fibrosing variant of Hashimoto thyroiditis is an IgG4 related disease.

    Science.gov (United States)

    Deshpande, Vikram; Huck, Amelia; Ooi, Esther; Stone, John H; Faquin, William C; Nielsen, G Petur

    2012-08-01

    Hashimoto thyroiditis (HT) and the fibrosing variant of Hashimoto thyroiditis (FVHT) are immune-mediated tumefactive lesions of the thyroid. Immunoglobulin G4-related disease (IgG4-RD) is now a widely recognised multi-organ system disease characterised by elevated serum and tissue concentrations of IgG4. In this study, the authors address several unresolved questions pertaining to the relationship between HT and FVHT, and the association of each of these diseases with IgG4-RD. The authors evaluated 28 consecutive cases of HT and nine cases of FVHT. The clinical, demographic and serological data were recorded. The slides were stained immunohistochemically using antibodies to IgG4 and IgG and the quantitative analysis was recorded. Data on thyroid function tests were available on seven cases of FVHT and 14 cases of HT. Based on the availability of data, hypothyroidism was noted in 62% (9/14) of HT and 86% of FVHT (6/7). FVHT demonstrated an exaggerated lobular pattern with lobules separated by cellular storiform-type fibrosis, resembling fibrosis seen in other forms of IgG-RD. The median IgG4 counts per high power field (×40) in HT and FVHT were 2.3 and 22, respectively. The median IgG4:IgG ratios in HT and FVHT were 0.11 and 0.58, respectively. The authors propose that FVHT belongs to the spectrum of IgG4-RD. Although a proportion of cases of HT show elevated numbers of IgG4 positive plasma cells, these cases lack the histological features typically associated with IgG4-RD, and thus the relationship between HT and IgG4-RD remains unproven.

  10. Cell uptake mechanisms of PAMAM G4-FITC dendrimer in human myometrial cells

    Science.gov (United States)

    Oddone, Natalia; Zambrana, Ana I.; Tassano, Marcos; Porcal, Williams; Cabral, Pablo; Benech, Juan C.

    2013-07-01

    The high incidence and severity of diseases which involve smooth muscle dysfunction dictates the need of continued search for novel therapeutic strategies to treat these conditions. Dendrimers are branched macromolecules with multiple end-groups that can be functionalized for applications which include drug delivery. There is no data regarding the cellular uptake mechanisms used by dendrimers in smooth muscle human myometrial cells (HMC). Polyamidoamine G4 dendrimers were conjugated with fluorescein isothiocyanate (FITC) and the resulting conjugate (G4-FITC) was characterized using high-performance liquid chromatography, nuclear magnetic resonance, and atomic force microscopy. G4-FITC showed to have no significant effect on the primary culture HMC viability up to 48 h. HMC incubated with G4-FITC were analyzed by laser confocal microscopy. Peri-nuclear fluorescence distribution was observed at 5 h of incubation or more (24, 36, and 48 h). At 24 h, G4-FITC partially co-localized with lysotracker. Uptake of G4-FITC by HMC was slightly inhibited by filipin (8.0 ± 3.9 %) and significantly inhibited by chlorpromazine (63.5 ± 3.7 %). In non-electroporated HMC, G4-FITC was never observed inside the cell nucleus. Interestingly, we detected G4-FITC inside the nuclear domain of some electroporated cells. Thus, electroporation changed intracellular G4-FITC localization. Isolated nuclei of HMC incubated with G4-FITC showed fluorescence signal inside the nuclear domain. The results suggest that in HMC, G4-FITC is taken up by clathrin-mediated endocytosis with endosomal and lysosomal localization at 24 h. The combination of electroporation and dendrimers could be an interesting technology to electrotransfer drugs into smooth muscle cells cytosol and nuclei.

  11. 慢性硬化性颌下腺炎IgG4的表达观察%IgG4 expression in chronic sclerosing submaxillaritis

    Institute of Scientific and Technical Information of China (English)

    孙柯; 王丽君; 姚洪田; 向华; 丁伟

    2012-01-01

    Objective To analyze the clinicopathologic features of chronic sclerosing submaxillaritis (CSS).Methods The clinical and pathological characteristics of 9 CSS were analyzed.Results In the 9 patients,there were 6 males and 3 females.The age of patients ranged from 51-77 years old.All of the tumors were located in the submandibular gland,presenting with painless and firm mass.Histologically,a well-defined mass lesion with extensive lymphocytes and plasma cells infiltration,preservation of lobular architecture,with acinar atrophy.The reactive hyperplasia of lymphoid follicles may be found in CSS.The phlebitis and obliterating phlebitis also formed.Immunohistochemistry showed evidence of diffuse infiltration of plasma cells.The mean number of IgG4-positive plasma cell per high-power field (HPF) was 186,mean value of the IgG4∶ IgG ratio was 0.71.Three of these 9 cases had manifestations of IgG4-associated systemic disease.Conclusions CSS is considered as a part of IgG4-related sclerosing diseases,recognition of which is very essential for a successful treatment.When diagnosis is made,it is necessary to ascertain whether lesion occurs within salivary gland only or in combination with outside IgG4-related sclerosing disease.The establishment of follow-up is also necessary.Some patients show good response to steroid therapy.%目的 分析慢性硬化性颌下腺炎的临床病理特点及IgG4的表达,为临床诊断和治疗提供参考.方法 收集术后病理诊断为慢性硬化性颌下腺炎的病例9例,男性6例,女性3例,年龄51 ~77岁,中位年龄66岁.对其临床资料、手术标本病理组织学特征及免疫表型总结分析.结果 组织学表现为颌下腺小叶结构保存,致密的淋巴细胞和浆细胞浸润,大量淋巴滤泡反应性增生,腺泡不同程度萎缩,小叶间隔明显纤维化,静脉炎及闭塞性静脉炎形成.免疫组织化学检测显示病变组织中IgG4及IgG阳性的浆细胞均明显增加,每一高

  12. Concept, objectives and methodological tools in the design of USW in the municipality of San Marcos (Guipuzcoa, ES); Concepto, objetivos y elementos metodologicos en el diseno de la recogida selectiva de RSU de la mancomunidad de San Marcos

    Energy Technology Data Exchange (ETDEWEB)

    Abel Casado, J.; Eguiluz Saenz, I. [Ein, S.L. (Spain); Castillo, I.

    1995-12-31

    The authors describe the principles followed by the association of municipalities of San Marcos (Guipuzcoa, Spain) to set up a separated collection Urban Solid Waste Plan. The policy of this association has three basic goals: first, maximum profitable use of USW by recovering materials, obtaining biogas; second, waste treatment on a sanitary landfill and third, elimination control and following those points used for uncontrolled delivery of wastes.

  13. An inflammatory condition with different faces : Immunoglobulin G4-Related disease

    NARCIS (Netherlands)

    Karim, A. F.; Verdijk, R. M.; Guenoun, J.; van Hagen, P. M.; van Laar, J. A M

    2016-01-01

    Background: Immunoglobulin G4-related disease (IgG4-RD) is a systemic fibro-inflammatory condition with involvement of different organs. The pathophysiological mechanism is unclear, but fibrosis is the hallmark of this disease. Early recognition is critical to avoid irreversible organ damage. Recent

  14. Prevalence of atopy, eosinophilia, and IgE elevation in IgG4-related disease.

    Science.gov (United States)

    Della Torre, Emanuel; Mattoo, Hamid; Mahajan, Vinay S; Carruthers, Mollie; Pillai, Shiv; Stone, John H

    2014-02-01

    IgG4-related disease (IgG4-RD) is a fibroinflammatory disorder that can affect virtually every organ system. T-helper type 2 responses have been presumed to be pathogenic in this disease, and a high proportion of patients with IgG4-RD are reported to have longstanding allergies, peripheral blood eosinophilia, and serum IgE elevation. It has therefore been proposed that allergic mechanisms drive IgG4-RD. However, no epidemiological assessment of atopy, peripheral blood eosinophilia, and serum IgE concentrations has ever been undertaken in patients with IgG4-RD. In this study, we evaluated these parameters in a large cohort of patients with IgG4-RD in whom a wide range of organs were affected by disease. Our results demonstrate that the majority of patients with IgG4-RD are nonatopic. Nevertheless, a subset of nonatopic subjects exhibit peripheral blood eosinophilia and elevated IgE, suggesting that processes inherent to IgG4-RD itself rather than atopy per se contribute to the eosinophilia and IgE elevation observed in the absence of atopy. © 2013 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  15. Serum total IgG and IgG4 levels in thyroid eye disease

    Directory of Open Access Journals (Sweden)

    Sy A

    2016-10-01

    Full Text Available Aileen Sy, Rona Z Silkiss Department of Ophthalmology, California Pacific Medical Center, San Francisco, CA, USA Purpose: To investigate the relationship between immunoglobulin G (IgG4-related disease (IgG4-RD and thyroid eye disease (TED with respect to IgG levels. Patients and methods: A retrospective review of total IgG, IgG subclass, and thyroid stimulating immunoglobulin (TSI levels in 24 patients with TED. Results: Five patients (20.8% demonstrated serum IgG4 levels consistent with IgG4-RD without any additional systemic disease. Total IgG and IgG subclass levels were found to be an inadequate proxy for TSI elevation. Conclusion: There may be a subtype of TED patients with elevated IgG4 in the absence of IgG4-RD systemic findings. Keywords: thyroid eye disease, IgG subclass, IgG4, Graves’ disease, Graves’ ophthalmopathy, IgG4-RD

  16. Specific IgG(4) responses during chronic and transient antigen exposure in aspergillosis

    NARCIS (Netherlands)

    Tomee, JFC; Dubois, AEJ; Koeter, GH; Beaumont, F; vanderWerf, TS; Kauffman, HF

    1996-01-01

    The factors that lead to increased production of specific IgG subclasses are still largely unknown. Recent studies suggest that increased IgG(4) responses may be related to prolonged antigen exposure. We present data showing that increased IgG(4) responses are found under conditions of chronic expos

  17. IgG4-related inflammatory pseudotumor of the renal pelvis involving renal parenchyma, mimicking malignancy.

    Science.gov (United States)

    Park, Ho Gyun; Kim, Kyoung Min

    2016-01-22

    IgG4-related disease is a recently recognized systemic disease characterized by storiform fibrosis with infiltration of IgG4-positive plasma cells. In rare incidences, IgG4-related renal disease can present as a solitary mass lesion at renal pelvis and can pose a diagnostic challenge since these lesions mimic malignancy. Herein, we present a rare case of IgG4-related disease presenting as inflammatory pseudotumor lesion, involving the renal pelvis and also neighboring renal parenchyma. A 75-year-old man with no history of IgG4-related disease underwent computed tomography (CT) scan for evaluation of prostatic cancer. The CT scan incidentally revealed a mass lesion located at the right renal pelvis. Radiologic findings were highly suggestive of malignancy. Therefore, the patient underwent right nephroureterectomy. Microscopically, the mass lesion showed storiform fibrosis with diffuse and intense inflammatory cell infiltration. Infiltrating cells were mainly histiocytes and plasma cells. Tubulointerstitium adjacent to the lesion also showed fibrosis with abundant plasmacytic infiltration. Immunohistochemical staining revealed the presence of IgG4-positive plasma cells in both the mass lesion and tubulointerstitium (mean of 94/HPF per field). Considering these findings, we diagnosed the mass lesion as IgG4-related inflammatory pseudotumor of the renal pelvis. In patients with renal pelvic masses, IgG4-related inflammatory pesudotumor should be considered in the differential diagnosis to avoid unnecessary surgical intervention.

  18. Data of evolutionary structure change: 1DUIA-2G4VA [Confc[Archive

    Lifescience Database Archive (English)

    Full Text Available 1DUIA-2G4VA 1DUI 2G4V A A --AKCVSYGVSQIKAPALHS-----QGYTGSNVKVAVID...H - HHHHHHHHHH EEE - 0 1DUI... A 1DUIA GTVLA----VAPSA ALA CA 375 ARG CA 304 ASN CA 261 1DUI... A 1DUIA AGNEGTSGSSSTV

  19. 78 FR 21151 - G4 Products, LLC a Subsidiary of G4 Holdings, Inc. Including Workers Whose Wages are Paid Under...

    Science.gov (United States)

    2013-04-09

    ... Whose Wages are Paid Under CPS Ventures, LLC, Crestline, Ecoeverywhere, LLC, G4 Services, LL, Geiger Brothers, Geiger Group, Livgeiger, and Sun Graphix and Including On-Site Leased Workers from OSW and Maine..., LLC, Geiger Brothers, Geiger Group, Livgeiger, LLC, and Sun Graphix. The subject worker group does...

  20. Perivascular fibrosis and IgG4-related disease: a case report

    Directory of Open Access Journals (Sweden)

    S. Monti

    2014-11-01

    Full Text Available Immunoglobulin G4-related disease (IgG4-RD is a newly recognized fibroinflammatory condition which can potentially involve any organ. Some characteristic histopathologic features with lymphoplasmacytic infiltrate, an increased number of IgG4+ cells, storiform fibrosis and obliterative phlebitis are the mainstay for diagnosis. Serum IgG4 levels often increase. We report the case of a patient with perivascular fibrotic lesions involving the aortic arch and the splenic hilum, with a surgical biopsy-proven diagnosis of IgG4-related disease. The patient is now undergoing a low-dose corticosteroid maintenance therapy without evidence of new localizations of the disease. This case highlights the need for increasing awareness and recognition of this new, emerging clinical condition.

  1. A Case of Immunoglobulin G4-Related Disease with Extensive Multiorgan Involvements

    Directory of Open Access Journals (Sweden)

    Kazuhiko Higashioka

    2015-01-01

    Full Text Available We report a case of IgG4-related disease (IgG4-RD with multiple ten-organ involvement. This case showed many clinical findings, such as bilateral swelling of salivary and lacrimal glands, autoimmune pancreatitis, interstitial nephritis, retroperitoneal fibrosis, periaortitis, systemic swelling of lymph nodes, pulmonary lesions, splenomegaly, and jejunal lesions. He was suspected as having SLE or malignant lymphoma but diagnosed as having IgG4-RD by the elevated serum IgG4 level and histological findings from kidney and lymph node. We report a case of IgG4-RD with multiple ten-organ involvement that was successfully treated with prednisolone therapy.

  2. Anti-inflammatory activity of human IgG4 antibodies by dynamic Fab arm exchange.

    Science.gov (United States)

    van der Neut Kolfschoten, Marijn; Schuurman, Janine; Losen, Mario; Bleeker, Wim K; Martínez-Martínez, Pilar; Vermeulen, Ellen; den Bleker, Tamara H; Wiegman, Luus; Vink, Tom; Aarden, Lucien A; De Baets, Marc H; van de Winkel, Jan G J; Aalberse, Rob C; Parren, Paul W H I

    2007-09-14

    Antibodies play a central role in immunity by forming an interface with the innate immune system and, typically, mediate proinflammatory activity. We describe a novel posttranslational modification that leads to anti-inflammatory activity of antibodies of immunoglobulin G, isotype 4 (IgG4). IgG4 antibodies are dynamic molecules that exchange Fab arms by swapping a heavy chain and attached light chain (half-molecule) with a heavy-light chain pair from another molecule, which results in bispecific antibodies. Mutagenesis studies revealed that the third constant domain is critical for this activity. The impact of IgG4 Fab arm exchange was confirmed in vivo in a rhesus monkey model with experimental autoimmune myasthenia gravis. IgG4 Fab arm exchange is suggested to be an important biological mechanism that provides the basis for the anti-inflammatory activity attributed to IgG4 antibodies.

  3. Immunoglobulin G4-positive multi-organ lymphoproliferative syndrome with antiphospholipid antibody syndrome.

    Science.gov (United States)

    Kawakami, Nobuyo; Kawai, Kazuhiro; Baba, Naoko; Ohshima, Kouichi; Kanekura, Takuro

    2012-07-01

    We report immunoglobulin (Ig)G4-positive multi-organ lymphoproliferative syndrome (IgG4(+) -MOLPS) with antiphospholipid antibody syndrome (APS) in a 56-year-old Japanese man presenting with purpuric patches on his legs. Skin biopsy revealed leukocytoclastic vasculitis. Laboratory tests demonstrated high levels of serum IgG and IgG4, hypocomplementemia and anticardiolipin antibody. Echography of the lower limbs and pulmonary scintigraphy showed a thrombus in the left soleal vein and multiple emboli in the basal part of both inferior pulmonary arteries. Computed tomography revealed systemic lymphadenopathy. Histologically, there was reactive paracortical hyperplasia with proliferation of histiocytes and infiltration of IgG4-positive plasma cells. We made a diagnosis of IgG4(+) -MOLPS with APS. To our knowledge, this complication has not been reported previously.

  4. The clinical significance of serum IgG4 in the diagnosis of IgG4-related diseases%血清IgG4检测在IgG4相关性疾病诊断中的临床意义

    Institute of Scientific and Technical Information of China (English)

    李萍; 陈华; 吴庆军; 吴子燕; 张蜀澜; 张文; 张奉春; 李永哲

    2014-01-01

    Objective To evaluate the clinical applications of serum IgG4 for the diagnosis of IgG4-related disease (IgG4-RD).Methods In this retrospective study,160 adult patients with IgG4-RD who in their first time visit in Peiking Union Medical College Hospital between 2011 to 2013 were reviewed.All patients had detailed clinical reference and final clear diagnosis.Meantime we selected 126 patients with other non-IgG4-related immune disease and 125 healthy subjects as controls from Peiking Union Medical College Hospital physical checkup center,IgG4 was detected by nephelometry,takeing the first testing result for analysis.Statistical analysis was performed using SPSS13.0 software.Results Serum IgG4 levels higher than 1 350 mg/L were seen in 82.5% of the patients (132/160) with IgG4-RD and 20.6% of the patients (26/126) with other diseases respectively.The serum concentrations of IgG4 in IgG4-RD group were significantly higher than other disease group and healthy control group (x2 =110.8,158.6,P < 0.05).There was no significant difference between other disease group and the healthy control group(x2 =1.964,P =0.161 > 0.05).The optimal diagnostic cut-off values for IgG4-RD was 1575mg / L.Conclusion The best diagnostic cut valuse of serum IgG4 for Chinese IgG4-RD is different from the current international standard,and realize that to establish reference vange of Chinese for diagnosis of IgG4-RD has very important significance.%目的 评估血清IgG4检测对诊断IgG4相关性疾病(IgG4-RD)的临床意义.方法 回顾性研究2011年2月至2013年3月首次在北京协和医院就诊的成年人IgG4-RD患者160例,均有详细的临床资料和最终明确诊断,同时选取126例患者为其他非血清IgG4相关的免疫性疾病对照组,125名来自北京协和医院体检中心的健康体检者作为健康对照组.采用散射比浊法测定血清IgG4,160例患者的血清IgG4结果以首次就诊时检测结果为准.所有数据采用SPSS13.0统计学

  5. Analysis of clinical feature of IgG4 related disease%IgG4相关性疾病临床特征分析

    Institute of Scientific and Technical Information of China (English)

    李燕明; 王和; 方芳; 张旻; 居阳; 孙铁英

    2015-01-01

    Objective To summarize the clinical characteristics of IgG 4 related disease.Methods A retrospective analysis of clinical manifestation , radiography and pathological information of patients diagnosed with IgG4 related disease in Beijing Hospital.Results Patients recruited in this study were 7 males and 7 females at the average age of (64.3 ±10.0) years old.Equal or greater than two organs were suffered in 10 patients.In these involved organs , pancreas was the most suffered organ with 10 times, bile duct and salivary gland was suffered 5 times respectively , lung was suffered 4 times and lacrimal gland was suffered 2 times.Laboratory examination of these 14 cases showed that 5 of these cases were with increased level of plasma IgG and all of the cases were with increased level of IgG 4, 7 patients were with increased level of eosinophil count and 9 patients in 12 cases were with the increased level of IgE.All of the diagnosed patients were eusemia and 13 of them received the treatment of glucocorticoid.After usage of glucocorticoid , the clinical symptom and radiography findings were at great improvement , and the levels of IgG4 were decreased , but only two cases were down to normal level.Conclusions IgG4 related disease is a kind of disease with various manifestation and multiple organs are commonly suffered.The increase of IgG , IgE levels and eosinophil count could be considered as diagnosis clues.%目的 分析IgG4相关性疾病的临床特征.方法 回顾分析2011年12月至2015年1月北京医院确诊的14例IgG4相关性疾病患者的临床表现、影像和病理学资料.结果 14例患者中男、女各7例,年龄(64.3 ±10.0)岁.10例存在≥2个器官受累,受累器官包括胰腺10例次,胆管和涎腺各5例次,肺4例次,泪腺2例次.14例患者中5例血清IgG水平升高,14例血清IgG4水平均升高,7例血嗜酸粒细胞绝对值升高,12例检查IgE的患者中9例升高.14例患者预后均良好,其中有13例应用糖皮质激

  6. IgG4-related disease simulating Hodgkin lymphoma in a child

    Directory of Open Access Journals (Sweden)

    D. Eric Ewing, MD

    2016-06-01

    Full Text Available Immunoglobulin (Ig G4-related disease is a recently described syndrome characterized by mass forming lymphoplasmacytic tissue infiltration and elevated serum IgG4 concentrations usually affecting middle-aged or older individuals. Lymphadenopathy is frequently observed and is sometimes the first or only manifestation of the disease. We report a case of IgG4-related disease mimicking Hodgkin lymphoma in a 13-year-old girl. The patient presented with progressive unilateral cervical lymphadenopathy of several months duration. Biopsy showed follicular hyperplasia with progressive transformation of germinal centers. Interfollicular areas were expanded by small lymphocytes, histiocytes, eosinophils and fibrosis with occasional CD30 positive cells initially concerning for interfollicular Hodgkin lymphoma. Immunohistochemical analysis revealed an intrafollicular plasmacytosis with an IgG4-positive/IgG-positive plasma cell ratio of 50% supporting a diagnosis of IgG4-related lymphadenopathy, progressively transformed germinal centers type. Laboratory studies were supportive with elevated serum IgG4 (178 mg/dL and IgE (30.40 kU/L levels along with an elevated serum IgG4/IgG ratio (0.16. Very few cases of IgG4-related disease have been described in children. Within this age group, there is considerable clinical overlap between IgG4-related disease associated lymphadenopathy and Hodgkin lymphoma. In addition, lymphadenopathy secondary to IgG4-related disease demonstrates substantial histologic diversity with the potential to simulate the inflammatory background and fibrosis of Hodgkin lymphoma. The importance of accurate diagnosis is underscored by the prognostic implications considering the marked response of the syndrome to steroid therapy. In addition, appropriate follow up is critical to monitor for relapse and additional organ involvement.

  7. Histopathologic Overlap between Fibrosing Mediastinitis and IgG4-Related Disease

    Directory of Open Access Journals (Sweden)

    Tobias Peikert

    2012-01-01

    Full Text Available Fibrosing mediastinitis (FM and IgG4-related disease (IgG4-RD are two fibroinflammatory disorders with potentially overlapping clinical and radiological features. In this paper, we looked for histopathologic features of IgG4-RD and enumerated infiltrating IgG4-positive plasma cells within mediastinal tissue biopsies from FM patients. We identified 15 consecutive FM surgical mediastinal tissue biopsies between 1985 and 2006. All patients satisfied the clinical and radiological diagnostic criteria for FM. All patients had either serological or radiological evidence of prior histoplasmosis or granulomatous disease, respectively. Formalin-fixed paraffin-embedded tissue sections of all patients were stained for H&E, IgG, and IgG4. Three samples met the predefined diagnostic criteria for IgG4-RD. In addition, characteristic histopathologic changes of IgG4-RD in the absence of diagnostic numbers of tissue infiltrating IgG4-positive plasma cells were seen in a number of additional cases (storiform cell-rich fibrosis in 11 cases, lymphoplasmacytic infiltrate in 7 cases, and obliterative phlebitis/arteritis in 2 cases. We conclude that up to one-third of histoplasmosis or granulomatous-disease-associated FM cases demonstrate histopathological features of IgG4-RD spectrum. Whether these changes occur as the host immune response against Histoplasma or represent a manifestation of IgG4-RD remains to be determined. Studies to prospectively identify these cases and evaluate their therapeutic responses to glucocorticoids and/or other immunosuppressive agents such as rituximab are warranted.

  8. Mitochondrial-dependent Autoimmunity in Membranous Nephropathy of IgG4-related Disease

    Directory of Open Access Journals (Sweden)

    Simona Buelli

    2015-05-01

    Full Text Available The pathophysiology of glomerular lesions of membranous nephropathy (MN, including seldom-reported IgG4-related disease, is still elusive. Unlike in idiopathic MN where IgG4 prevails, in this patient IgG3 was predominant in glomerular deposits in the absence of circulating anti-phospholipase A2 receptor antibodies, suggesting a distinct pathologic process. Here we documented that IgG4 retrieved from the serum of our propositus reacted against carbonic anhydrase II (CAII at the podocyte surface. In patient's biopsy, glomerular CAII staining increased and co-localized with subepithelial IgG4 deposits along the capillary walls. Patient's IgG4 caused a drop in cell pH followed by mitochondrial dysfunction, excessive ROS production and cytoskeletal reorganization in cultured podocytes. These events promoted mitochondrial superoxide-dismutase-2 (SOD2 externalization on the plasma membrane, becoming recognizable by complement-binding IgG3 anti-SOD2. Among patients with IgG4-related disease only sera of those with IgG4 anti-CAII antibodies caused low intracellular pH and mitochondrial alterations underlying SOD2 externalization. Circulating IgG4 anti-CAII can cause podocyte injury through processes of intracellular acidification, mitochondrial oxidative stress and neoantigen induction in patients with IgG4 related disease. The onset of MN in a subset of patients could be due to IgG4 antibodies recognizing CAII with consequent exposure of mitochondrial neoantigen in the context of multifactorial pathogenesis of disease.

  9. Concurrent systemic AA amyloidosis can discriminate primary sclerosing cholangitis from IgG4-associated cholangitis.

    Science.gov (United States)

    Kato, Takehiro; Komori, Atsumasa; Bae, Sung-Kwan; Migita, Kiyoshi; Ito, Masahiro; Motoyoshi, Yasuhide; Abiru, Seigo; Ishibashi, Hiromi

    2012-01-14

    Chronic hepatobiliary inflammatory diseases are not widely acknowledged as underlying disorders of systemic AA amyloidosis, except epidemic schistosomiasis. Among them, primary sclerosing cholangitis (PSC) might initiate amyloid A protein deposition in diverse tissues, giving rise to systemic amyloidosis, due to a progressive and unresolved inflammatory process, and its possible association with inflammatory bowel diseases. Nevertheless, only one such case has been reported in the literature to date. We report a 69-year-old Japanese woman with cirrhosis who was diagnosed with PSC complicated with systemic AA amyloidosis, without any evidence of other inflammatory disorders. As a result of cholestasis in conjunction with biliary strictures and increased serum IgG4, the presence of IgG4(+) plasma cells was examined systemically, resulting in unexpected documentation of Congo-red-positive amyloid deposits, but not IgG4(+) plasma cells, in the liver, stomach and salivary glands. Elevated serum IgG4 is the hallmark of IgG4-related disease, including IgG4-associated cholangitis, but it has also been demonstrated in certain patients with PSC. Amyloid A deposits in multiple organs associated with an indolent clinical course that progresses over many years might have a diagnostic value in discriminating PSC from IgG4-associated cholangitis.

  10. Utility of FDG PET/CT in IgG4-related systemic disease

    Energy Technology Data Exchange (ETDEWEB)

    Nakatani, K., E-mail: koyakn@kuhp.kyoto-u.ac.jp [Department of Diagnostic Imaging and Nuclear Medicine, Kyoto University Graduate School of Medicine, Sakyo-ku, Kyoto (Japan); Nakamoto, Y.; Togashi, K. [Department of Diagnostic Imaging and Nuclear Medicine, Kyoto University Graduate School of Medicine, Sakyo-ku, Kyoto (Japan)

    2012-04-15

    IgG4-related systemic disease (IgG4-RSD) is an emerging clinical entity about which much remains to be elucidated, in terms of its aetiology, pathogenesis, diagnosis, treatment and outcome. Autoimmune pancreatitis (AIP) and Mikulicz disease (MD) are the two major, well-studied constituents of IgG4-RSD. AIP and MD have common characteristics of forming tumour-mimicking lesions that consist of lymphoplasmacytic infiltrates and fibrosclerosis with numerous immunoglobulin G4 (IgG4)-positive plasma cells, as well as various multi-organ manifestations of IgG4-RSD. 2-[{sup 18}F]-fluoro-2-deoxy-D-glucose positron-emission tomography/ computed tomography (FDG PET/CT) enables the acquisition of whole-body images and provides functional information about disease activity; as such it has a valuable role in staging extent of disease, guiding biopsy, and monitoring response to treatment. However, FDG PET/CT is likely to be only one component of the management strategy, and clinical, laboratory, imaging and histological findings are crucial in the overall diagnosis of the condition. At present FDG PET/CT does not have a well-established role in the assessment of patients with IgG4-RSD and future prospective studies are required to define the cost-effectiveness and clinical impact in this patient group more accurately.

  11. Concurrent systemic AA amyloidosis can discriminate primary sclerosing cholangitis from IgG4-associated cholangitis

    Institute of Scientific and Technical Information of China (English)

    Takehiro Kato; Atsumasa Komori; Sung-Kwan Bae; Kiyoshi Migita; Masahiro Ito; Yasuhide Motoyoshi; Seigo Abiru; Hiromi Ishibashi

    2012-01-01

    Chronic hepatobiliary inflammatory diseases are not widely acknowledged as underlying disorders of systemic AA amyloidosis, except epidemic schistosomiasis. Among them, primary sclerosing cholangitis (PSC) might initiate amyloid A protein deposition in diverse tissues, giving rise to systemic amyloidosis, due to a progressive and unresolved inflammatory process, and its possible association with inflammatory bowel diseases. Nevertheless, only one such case has been reported in the literature to date. We report a 69-yearold Japanese woman with cirrhosis who was diagnosed with PSC complicated with systemic AA amyloidosis, without any evidence of other inflammatory disorders. As a result of cholestasis in conjunction with biliary strictures and increased serum IgG4, the presence of IgG4+ plasma cells was examined systemically, resulting in unexpected documentation of Congo-red-positive amyloid deposits, but not IgG4+ plasma cells, in the liver, stomach and salivary glands. Elevated serum IgG4 is the hallmark of IgG4-related disease, including IgG4-associated cholangitis, but it has also been demonstrated in certain patients with PSC. Amyloid A deposits in multiple organs associated with an indolent clinical course that progresses over many years might have a diagnostic value in discriminating PSC from IgG4-associated cholangitis.

  12. Is MuSK myasthenia gravis linked to IgG4-related disease?

    Science.gov (United States)

    Raibagkar, Pooja; Ferry, Judith A; Stone, John H

    2017-04-15

    Immunoglobulin (Ig) G4-related disease (IgG4-RD) is an immune-mediated inflammatory condition that affects a wide variety of sites, including the nervous system, where it can involve the meninges or the pituitary gland, and cause perineural mass lesions. A large subset of acetylcholine receptor antibody (Ab)-negative myasthenia gravis (MG) patients has muscle-specific tyrosine kinase (MuSK) Abs, generally of the IgG4 subclass. There has not been any association found between IgG4-RD and MuSK MG yet. We report the first case of MuSK MG associated with lymphadenopathy with histopathology consistent with IgG4-RD. A 54-year-old woman with MuSK MG developed eight compression fractures related to steroid therapy. Eighteen months after initial presentation she was found to have retroperitoneal lymphadenopathy with biopsy findings consistent with IgG4-RD. She was started on rituximab with clinical improvement and ability to taper immunomodulatory agents for the first time. Our case raises number of questions regarding a potential link between MuSK MG and IgG4-RD which may shed further light on the pathophysiology and management of these diseases. Copyright © 2017 Elsevier B.V. All rights reserved.

  13. IgG4-related Hashimoto's thyroiditis--a new variant of a well known disease.

    Science.gov (United States)

    Luiz, Henrique Vara; Gonçalves, Diogo; Silva, Tiago Nunes da; Nascimento, Isabel; Ribeiro, Ana; Mafra, Manuela; Manita, Isabel; Portugal, Jorge

    2014-11-01

    Hashimoto's thyroiditis (HT) has been characterized for many years as a well-defined clinicopathologic entity, but is now considered a heterogeneous disease. IgG4-related HT is a new subtype characterized by thyroid inflammation rich in IgG4-positive plasma cells and marked fibrosis. It may be part of the systemic IgG4-related disease. We report a case of a 56-year-old Portuguese man who presented with a one-month history of progressive neck swelling and dysphagia. Laboratory testing revealed increased inflammatory parameters, subclinical hypothyroidism and very high levels of thyroid autoantibodies. Cervical ultrasound (US) demonstrated an enlarged and heterogeneous thyroid gland and two hypoechoic nodules. US-guided fine needle aspiration cytology was consistent with lymphocytic thyroiditis. The patient was submitted to total thyroidectomy and microscopic examination identified typical findings of HT, marked fibrosis limited within the thyroid capsule and lymphoplasmacytic infiltration, with >50 IgG4-positive plasma cells per high-power field and an IgG4/IgG ratio of >40%. After surgery, serum IgG4 concentration was high-normal. Symptoms relief and reduction in laboratory inflammatory parameters were noticed. Thyroid function is controlled with levothyroxine. To our knowledge we report the first case of IgG4-related HT in a non-Asian patient. We also perform a review of the literature regarding IgG4-related disease and IgG4-related HT. Our case highlights this new variant of the well known HT, and helps physicians in recognizing its main clinical features, allowing for proper diagnosis and treatment.

  14. Clinicopathological features of Riedel's thyroiditis associated with IgG4-related disease in Japan.

    Science.gov (United States)

    Takeshima, Ken; Inaba, Hidefumi; Ariyasu, Hiroyuki; Furukawa, Yasushi; Doi, Asako; Nishi, Masahiro; Hirokawa, Mitsuyoshi; Yoshida, Akira; Imai, Ryoukichi; Akamizu, Takashi

    2015-01-01

    Riedel's thyroiditis (RT) is a rare chronic fibrosing disorder characterized by a hard, infiltrative lesion in the thyroid gland, which is often associated with multifocal fibrosclerosis. Immunoglobulin G4-related disease (IgG4-RD) is typified by infiltration of IgG4-positive plasma cells into multiple organs, resulting in tissue fibrosis and organ dysfunction. In order to evaluate the clinicopathological features of RT and its relationship with IgG4-RD, we performed a Japanese literature search using the keywords "Riedel" and "Riedel's thyroiditis." We used the electronic databases Medline and Igaku Chuo Zasshi, the latter of which is the largest medical literature database in Japan. The diagnosis of RT was based on the presence of a fibroinflammatory process with extension into surrounding tissues. Only 10 patients in Japan fulfilled RT diagnostic criteria during the 25-year period between 1988 and 2012. Two patients with confirmed IgG4/IgG immunohistochemical findings demonstrated 43 and 13 IgG4-positive plasma cells per high-power field, respectively, and the IgG4-positive/IgG-positive plasma cell ratios of 20% and less than 5%. Of the 10 patients with RT, two received glucocorticoids, one of whom experienced marked shrinkage of the thyroid lesion. One patient had extra-thyroid involvement in the form of retroperitoneal fibrosis. Although the clinicopathological features of RT suggest that IgG4-RD may be the underlying condition in some cases, further investigation is needed to clarify the etiology of RT in relation to IgG4-RD.

  15. High Serum IgG4 Concentrations in Patients with Hashimoto’s Thyroiditis

    Directory of Open Access Journals (Sweden)

    Anna Popławska-Kita

    2015-01-01

    Full Text Available Purpose. Since recent reports suggest that Hashimoto thyroiditis (HT may be associated with IgG4-related disease, we aimed to find out whether the measurement of serum IgG4 allows for the identification of distinct types of HT, with different clinical, sonographic, and serologic characteristics. Methods. The group studied consisted of 53 patients with HT and 28 healthy individuals who underwent thyroid ultrasonography and body composition analysis. Serum concentrations of IgG4, TSH, anti-peroxidase antibodies (TPOAb, anti-TSH receptor antibodies, TNF-α, TGF-β1, Fas Ligand, TRAIL, and chemokines (CXCL9, CXCL11, and CXCL10 were measured by ELISA or radioimmunoassay. Results. The group with IgG4 level >135 IU/ml accounted for 32.5% of the patients. The signs of fibrosis were present in 27.0% of the high-IgG4 patients and in 9.1% of the normal-IgG4 group. The patients with elevated IgG4 required higher doses of L-thyroxine and had significantly lower level of TPOAb (P=0.02 than the non-IgG4-HT individuals and higher TNF-α level in comparison with the controls (P=0.01. Conclusions. Our results suggest that the measurement of serum IgG4 allows for an identification of patients with more rapid progression of HT, requiring higher doses of L-thyroxine. Low TPOAb level and the absence of coexisting autoimmune diseases may suggest distinct pathomechanism of this type of thyroiditis.

  16. Association between IgG4-related disease and progressively transformed germinal centers of lymph nodes.

    Science.gov (United States)

    Sato, Yasuharu; Inoue, Dai; Asano, Naoko; Takata, Katsuyoshi; Asaoku, Hideki; Maeda, Yoshinobu; Morito, Toshiaki; Okumura, Hirokazu; Ishizawa, Shin; Matsui, Shoko; Miyazono, Takayoshi; Takeuchi, Tamotsu; Kuroda, Naoto; Orita, Yorihisa; Takagawa, Kiyoshi; Kojima, Masaru; Yoshino, Tadashi

    2012-07-01

    Progressively transformed germinal centers is a benign condition of unknown pathogenesis characterized by a distinctive variant form of reactive follicular hyperplasia in lymph nodes. We recently reported Ig G4-related disease in progressively transformed germinal centers. However, no large case series has been reported and clinicopathologic findings remain unclear. Here, we report 40 Japanese patients (28 men, 12 women; median age, 56 years) with progressively transformed germinal centers of the lymph nodes who fulfilled the histological diagnostic criteria for IgG4-related disease (IgG4(+) progressively transformed germinal centers), with asymptomatic localized lymphadenopathy involving the submandibular nodes in 24, submandibular and cervical nodes in 14, cervical nodes only in 1, and cervical and supraclavicular nodes in 1. In all, 16 (52%) of 31 examined patients had allergic disease. Histologically, the lymph nodes demonstrated uniform histological findings, namely marked follicular hyperplasia with progressively transformed germinal centers, and localization of the majority of IgG4(+) plasma cells in the germinal centers. Serum IgG4, serum IgE and peripheral blood eosinophils were elevated in 87%, 92% and 53% of examined patients, respectively. Eighteen patients subsequently developed extranodal lesions (including five who developed systemic disease), which on histological examination were consistent with IgG4-related disease. IgG4(+) progressively transformed germinal centers presents with uniform clinicopathological features of asymptomatic localized submandibular lymphadenopathy, which persists and/or relapses, and sometimes progresses to extranodal lesions or systemic disease. Nine patients were administered steroid therapy when the lesions progressed, to which all responded well. We suggest that IgG4(+) progressively transformed germinal centers should be included in the IgG4-related disease spectrum.

  17. Colovesical fistula caused by glucocorticoid therapy for IgG4-related intrapelvic mass

    OpenAIRE

    2015-01-01

    IgG4-related disease (IgG4-RD) is an immune-mediated fibroinflammatory disorder that can occur in almost all systemic organs and generally responds to corticosteroid treatment. We report a rare case of an IgG4-related intrapelvic mass lesion that responded to steroid therapy but caused a fistula between the sigmoid colon and bladder. A 71-year-old man was followed after treatment for hepatocellular carcinoma. Follow-up computed tomography (CT) incidentally depicted left hydronephrosis with an...

  18. Immunoglobulin G4-related Lung Disease: A Disease with Many Different Faces

    Directory of Open Access Journals (Sweden)

    Philip Hui

    2013-01-01

    Full Text Available Immunoglobulin (Ig G4-related lung disease is a fibroinflammatory entity that presents in protean ways. Diagnostically, IgG4-related lung disease requires a high clinical index of suspicion complemented by elevated serum IgG4 levels and/or biopsy that shows the characteristic pathological features. The disease is almost always responsive to systemic corticosteroids. However, relapse is common following their discontinuation. The authors present three cases to highlight the diverse clinical features, and to illustrate the diagnostic and therapeutic approaches to this disease.

  19. IgG4-Related Hypophysitis: Case Report and Literature Review.

    Science.gov (United States)

    Decker, Lauren; Crawford, Angela M; Lorenzo, Gamaliel; Stippler, Martina; Konstantinov, Konstantin N; SantaCruz, Karen

    2016-12-01

    IgG4-related hypophysitis is a rare, inflammatory process of the pituitary that mimics more commonly seen pituitary tumors. We report a case of IgG4-related hypophysitis in a 16-year-old female with diabetes insipidus who was found to have IgG4-related hypophysitis based on tissue diagnosis. This entity has not been previously described in a pediatric patient. Recognition of certain inflammatory processes of the pituitary may lead to alternative means of diagnosis and medical management without a biopsy.

  20. IgG4相关性肾病发病机制和治疗新进展

    Institute of Scientific and Technical Information of China (English)

    何一鸣; 陈理换; 蔡珺; 彭艳霞; 陈秋华; 谢彤; 刘华锋; 潘庆军

    2016-01-01

    IgG4相关性肾病(IgG4-related kidney disease,IgG4-RKD)属于IgG4相关性疾病(IgG4-related disease,IgG4-RD),可累及全身多个器官,特别是肾脏.IgG4-RKD主要包括IgG4相关的小管间质性肾炎(IgG4-related tubulointerstitial nephritis,IgG4-TIN)和IgG4相关的膜性肾病(IgG4-related membranous nephropathy,IgG4-MN).目前IgG4-RKD发病机制仍存争议,如高水平的IgG4是伴随现象还是致病因素;IgG4-RKD是否为自身免疫性疾病,其抗原性如何等.IgG4-RKD的治疗方面,公认有效的治疗药物单一,糖皮质激素为一线药物,但其用药方案仍存争议.利妥昔单抗等生物制剂是较有前景的治疗药物.本文综述IgG4-RKD的发病机制及治疗新进展.

  1. Research on Marketing Combination Strategy of G+4P of Tianshui Tourism%天水旅游G+4P营销组合策略研究

    Institute of Scientific and Technical Information of China (English)

    李少华

    2013-01-01

    针对目前天水旅游业的现状,以G+4P观念为主线,从旅游产品策略,渠道策略,价格策略,促销策略以及政府营销5个角度进行具体的营销策略研究,同时提出营销策略实施的要点,并创新性地引入了微博营销,口碑营销,情感营销,交叉营销等新的营销理念,以提高发展地区旅游产业为主要目标,提出G+4P天水旅游营销组合策略.

  2. 提高对IgG4相关性眼病的认识%Raise awareness of IgG4 relative ocular disease

    Institute of Scientific and Technical Information of China (English)

    魏世辉; 李红阳

    2015-01-01

    Purpose IgG4-related ocular disease is a chronic systemic disease with lymphocyte abnormal.The lacrimal glands, extraocular muscles and infraorbital nerve were often involved which was often the first symptom of systemic disease.While ophthalmologists did not know this disease well.They usually misdiagnosed it as idiopathic inflammatory pseudotumor, thyroid-associated ophthalmopathy etc,which resulted in delayed treatments.Here pathogenesis, clinical features and treatment methods of IgG4-relative ocular disease were described in order to improve awareness of this ocular disease, reduce clinical misdiagnosis, improve disease prognosis and standardized treatment.As the incidence of this disease increased in recent years, it is very necessary to improve awareness of the disease for ophthalmologists.%IgG4相关性眼病是一种与淋巴细胞密切相关的慢性系统性疾病,泪腺、眼外肌和眶下神经常常受累,常常为全身病变的首发症状.眼科医师对该疾病尚缺乏足够的认识,经常误诊为炎性假瘤、甲状腺相关性眼病等而延误治疗.本文通过对IgG4相关性眼病的发病机制、临床特点及治疗方法进行阐述,旨在提高临床医师对该眼病的认识,降低该眼病的临床误诊率,提升疾病标准化治疗和预后的质量.由于该疾病近年发病率有所提高,因此提高临床医师对该疾病的认识十分必要.

  3. IgG4-related cholecystitis presenting as biliary malignancy: report of three cases.

    Science.gov (United States)

    Feely, Michael M; Gonzalo, David H; Corbera, Montserrat; Hughes, Steven J; Trevino, Jose G

    2014-09-01

    An increased awareness of IgG4-related diseases has led to an escalation in the number of sites known to be involved by this fibroinflammatory disease. We report three cases of IgG4-related cholecystitis which were thought to represent biliary malignancies both clinically and radiographically. All three cases underwent surgery tailored towards presumed malignant neoplasms. Only following pathologic examination was the true nature of the disease identified. Recognition of the clinical, radiographic, and pathologic presentation of IgG4-related cholecystitis is essential for the consideration of this disease process prior to surgical management for suspected gallbladder malignancies. However, the pre-operative diagnosis remains challenging and extensive surgical intervention is often necessary given the distressing presentation of IgG4-related cholecystitis.

  4. Immunoglobin G4-related disease: Two case reports and review of the recent literature

    Directory of Open Access Journals (Sweden)

    Urmila Anandh

    2015-01-01

    Full Text Available IgG4-related disease (IgG4-RD is a new, multiorgan and constantly evolving disease characterized by IgG4-positive plasma cells in the affected organ. This disease often affects the elderly. The pancreas is the main target organ affected, with almost all organs in the body being affected. A large amount of studies have been conducted to understand the pathogenesis of the disease and its spectrum. For accurate diagnosis of the condition, an adequate knowledge of imaging findings, clinical presentations and laboratory reports is essential. We report two cases of the IgG4-RD and review the recent literature about this increasingly recognized entity.

  5. IgG4-related intraocular inflammation masquerading as ciliary body melanoma in a young girl

    Directory of Open Access Journals (Sweden)

    Dipankar Das

    2016-01-01

    Full Text Available Immunoglobulin G4 (IgG4-related diseases affects various tissues and organs of the human body. Orbital, adnexal, and scleral inflammations were already reported in the medical literature. To the best of our knowledge, we report the first case of intraocular IgG4-associated inflammatory mass in the ciliary body mimicking as a melanoma in a 23-year-old female from Northeast India. Characteristic histopathology, immunohistochemistry in the tissue, protein chemistry, and raised serum IgG4 were supportive for the diagnosis. As this newly diagnosed disease has multi-organ affection and little is known about its pathogenesis particularly in eye and adnexa, the present case will open many challenges in clinico-pathological diagnosis and research in the future.

  6. Radiologic Findings of Immunoglobulin G4 Related Sclerosing Esophagitis: A Case Report

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Mi Sun; Kim, Su Young; Lee, Byung Hoon; Hwang, Yoon Joon; Han, Yoon Hee [Dept. of Radiology, Ilsan Paik Hospital, Inje University College of Medicine, Koyang (Korea, Republic of)

    2012-02-15

    We describe a case of immunoglobulin G4 (IgG4)-related sclerosing esophagitis occurring in a 63-year-old man with progressive dysphagia and 10-kg weight loss over 9 months. An esophagoscopy revealed significant stricture with diffuse mucosal friability and ulceration at mid esophagus level. Barium esophagogram showed diffuse stenosis at the mid and lower esophagus levels with ulcerations and irregularity of the mucosa. Multidetector computed tomography revealed diffuse edematous and circumferential thickening of the submucosa and muscle layer of this esophageal segment. Fluorine 18 fluorodeoxyglucose positron emission tomography (FDG PET) revealed diffuse mild FDG uptake in mid to lower esophagus. Although benign inflammatory lesion was suspected based on the imaging findings, the patient underwent surgery for worsening esophageal stricture and the esophageal lesion was pathologically confirmed as IgG4-related sclerosing esophagitis. Radiologic benignancy and high clinical suspicion for IgG4-related sclerosing disease may help making a proper decision and avoiding unnecessary operation.

  7. [An IgG4-related pancreatitis mimicking an adenocarcinoma: A case report].

    Science.gov (United States)

    Courcet, Emilie; Beltjens, Françoise; Charon-Barra, Céline; Guy, France; Orry, David; Ghiringhelli, François; Arnould, Laurent

    2015-12-01

    Type 1 auto-immune pancreatitis (type 1 AIP) is the pancreatic manifestation of IgG4-related systemic disease (IgG4-RD). This disease has recently been individualized and is characterized by elevated serum IgG4 levels and extrapancreatic lesions with common histologic characteristic: dense infiltration of lymphocytes, IgG4-positive plasma cells and storiforme fibrosis. Obliterative phlebitis is frequently detected. The pancreas is frequently involved in this disease. As approach to the pancreas for histological examination is generally difficult, AIP is diagnosed using a combination of clinical, serological, morphological and histopathological features. In pseudotumoral cases, AIP can be misdiagnosed as pancreatic cancer. Since AIP responds dramatically to steroid therapy, accurate diagnosis of AIP can avoid unnecessary laparotomy or pancreatic resection. We report here a case of a patient who underwent surgery for presumed pancreatic cancer. The final diagnosis was type 1 AIP.

  8. Cometabolic degradation of trichloroethylene by Burkholderia cepacia G4 with poplar leaf homogenate.

    Science.gov (United States)

    Kang, Jun Won; Doty, Sharon Lafferty

    2014-07-01

    Trichloroethylene (TCE), a chlorinated organic solvent, is one of the most common and widespread groundwater contaminants worldwide. Among the group of TCE-degrading aerobic bacteria, Burkholderia cepacia G4 is the best-known representative. This strain requires the addition of specific substrates, including toluene, phenol, and benzene, to induce the enzymes to degrade TCE. However, the substrates are toxic and introducing them into the soil can result in secondary contamination. In this study, poplar leaf homogenate containing natural phenolic compounds was tested for the ability to induce the growth of and TCE degradation by B. cepacia G4. The results showed that the G4 strain could grow and degrade TCE well with the addition of phytochemicals. The poplar leaf homogenate also functioned as an inducer of the toluene-ortho-monooxygenase (TOM) gene in B. cepacia G4.

  9. 血清IgG4水平在IgG4相关性疾病及与风湿免疫性疾病鉴别诊断中的应用价值%Value of serum IgG4 in diagnosis of IgG4-related disease and in differentiation from rheumatic diseases

    Institute of Scientific and Technical Information of China (English)

    贺琤雯; 唐古生; 鲁琼; 沈茜

    2012-01-01

    Objective To investigate the value of serum IgG4 in diagnosis of IgG4-RD and in differentiation from rheumatic diseases.Methods Total of 23 patients with IgG4-RD and 502 patients with rheumatic diseases were enrolled,who presented at Changhai Hospital in 2010 to 2011.In the study,rheumatic diseases were categorized into groups of Sj(o)gren syndrome (n =26),ankylosing spondylitis (n-50),systemic sclerosis (n =3),rhcumatoid arthritis (RA,n =125),mixed connective tissue disease (n =15),systemic lupus erythematosus (SLE,n =212),adult onset still disease (n =20),Behcet syndrome (n =17),polymyositis (n =12),dermatomyositis (n =12),polymyalgiarheumatica (n =10).Serum IgG and IgG4 levels were measured by a rate nephelometer assay.The ROC curves were constructed to identify the optimal serum IgG4 cutoff value for diagnosing IgG4-RD and evaluate its sensitivity and specificity.Results The mean levels of serum lgG4 in the group with IgG4-RD were 11.4(5.0-14.8) g/L.In about 95.6% IgG4-RD patients,the serum IgG4 level was higher than > 1.4 g/L and other rheumatic diseases (U values were 6.0,21.0,0,58.5,0,9.0,3.0,4.0,0,3.0,3.5,P <0.01).The levels of serum IgG4 with RA was 0.6(0.3-1.2) g/L,the levels of serum IgG4 with SLE was 0.2 (0.1-0.4) g/L.There were statistical differences between RA and SLE (U value was 5847,P < 0.01).At the same time,some patients with other rheumatic diseases were found serum IgG4 level higher than > 1.4 g/L,which was about 10% in the patients whith RA,ankylosing spondylitis,adult onset still disease and polymyalgiarheumatica.According to the ROC constructed the cut off value in present study was 2.2 g/L,and sensitivity and specificity were 95.7% and 97.4%,respectively.Area under the cerve (AUC) was 0.995.There were no significant differences between the sensitivity and specificity values obtained with a cutoff value of 2.2 g/L.In patients with other rheumatic diseases,the ratio of high serum IgG4 level (> 2.2 g/L) were declined

  10. IgG4-related disease and other causes of inflammatory meningeal disease.

    Science.gov (United States)

    Carruthers, Robert; Carruthers, Mollie; Della-Torre, Emanuel

    2014-09-01

    Immunoglobulin-4 (IgG4-) related disease is a newly described treatable condition that has recently expanded the differential diagnosis of inflammatory meningeal disorders. This review will discuss the main clinical and pathophysiological features of IgG4-related meningeal disease in the context of meningeal inflammatory disorders in general. Particular attention will be dedicated to the differential diagnosis and the different therapeutic approaches.

  11. IgG4-related disease: a systemic condition with characteristic microscopic features

    DEFF Research Database (Denmark)

    Detlefsen, Sönke

    2013-01-01

    diseases on their own, others have been included under the umbrella of "multifocal fibrosclerosis". Biopsies or resection specimens from affected organs in IgG4-RD reveal several common microscopic features irrespective of the site of the lesion. Cellular and storiform fibrosis, lymphoplasmacytic...... on the microscopic features, diagnosis and differential diagnosis of the different organ manifestations of IgG4-RD, and the current concepts of its pathogenesis will also be addressed....

  12. Enzyme-Assisted Extraction of Oil from Wet Microalgae Scenedesmus sp. G4

    OpenAIRE

    Shuhao Huo; Zhongming Wang; Fengjie Cui; Bin Zou; Pengxiang Zhao; Zhenhong Yuan

    2015-01-01

    The enzyme-assisted aqueous extraction of oil from wet microalgae was employed to avoid the energy consumption of a dewatering process. In this paper, oil-rich microalgae Scenedesmus sp. G4 was hydrolyzed by enzyme mixtures for oil extraction. The results showed that the algae concentration had the greatest influence on yield of extracted oil, and the temperature and the ratio of enzyme mixtures affected the results as follows: the maximum yield of oil extracted from Scenedesmus sp. G4 reache...

  13. IgG4-related disease: current challenges and future prospects

    Directory of Open Access Journals (Sweden)

    Lang D

    2016-02-01

    Full Text Available David Lang,1 Jochen Zwerina,2 Herwig Pieringer1,3 1Second Department of Internal Medicine, Kepler University Clinic, Linz, 2First Department of Internal Medicine, Ludwig Boltzmann-Institute of Osteology, Hanusch Hospital, Vienna, 3Paracelsus Private Medical University, Salzburg, Austria Abstract: Immunoglobulin G4-related disease (IgG4-RD represents an immune-mediated fibroinflammatory condition with a characteristic histopathological appearance that can affect various organs. Although numerous single-organ manifestations have been described more than a century ago, its systemic nature and unique features were only discovered in the last 2 decades, when IgG4-RD emerged as a new entity of disease. IgG4-RD is usually considered a rare disease, but its true epidemiology has not yet been fully clarified. Also, despite recent advances in the identification of the underlying immunological processes, its pathophysiology is only incompletely understood till now. The diagnostic workup of IgG4-RD is complex and usually requires a combination of clinical examination, imaging, histological, and serological analyses. However, no finding alone is specific for IgG4-RD. Therefore, its diagnosis requires careful interpretation of examination results in context with the patient’s clinical appearance as well as the exclusion of a broad variety of differential diagnoses. The past years brought rapid advances concerning this novel disease entity: diagnostic criteria, further insights into the underlying immunological processes, new biomarkers, and novel therapeutic approaches were proposed and widened the knowledge in the field of IgG4-RD. Still, a greater number of questions remain unanswered, and many recent developments require further discussion and proof from clinical trials. This review should give an overview on current knowledge and future perspectives in epidemiology, pathophysiology, diagnosis, and therapy of IgG4-RD. Keywords: PET/CT, autoimmune

  14. [IgG4-related kidney disease: what the nephrologist needs to know].

    Science.gov (United States)

    Galeano, Dario; Zanoli, Luca; Scarfia, Viviana Rosalia; L'Imperio, Vincenzo; Malatino, Lorenzo; Fatuzzo, Pasquale; Granata, Antonio

    2016-01-01

    IgG4 related disease is a systemic fibro-inflammatory disorder characterized by multiple organ and multiple tissue lesions. The real pathogenesis is currentlyactually unknown. For these reasons many authors compare IgG4 related disease to sarcoidosis. Lesions are often localized in the pancreas, salivary and lacrimal glands, biliary ducts, retroperitoneum and in many other organs. The diagnosisis difficult because of mild symptoms and the possibility of mimicking other severe diseases. Therefore, histopathology together with clinical and radiological typical findings are mandatory tools for diagnosis. Steroidtherapy usually enables disappearance of tumor like lesions and complete recovery. Kidney has an extensive organ involvement in the contextof IgG4-related disease. Historically, tubule - interstitial nephritis(TIN) is considered the main renal feature of renal lesions, however recent studies extend the spectrum of renal lesions also to glomerular tuft. These findings allow to introduce in the nosography the term of IgG4related kidney disease (IgG4 RKD). This review focuses on renal involvement in IgG4related disease, in order to help nephrologists to improve their clinical, diagnostic and therapeutic approach to this emerging pleiotropic clinical pattern.

  15. Development of novel electrical characterization methods and measurements of G4-DNA and DNA Derivatives

    CERN Document Server

    Livshits, Gideon I

    2015-01-01

    This dissertation presents an investigation into the electrical properties of two types of G4-DNA and several DNA-based molecules, targeting them as candidates for molecular wires and devices. Atomic force microscopy (AFM) and electrostatic force microscopy (EFM) comparison of co-deposited tetra- and intra-molecular G4-DNA reveals variations in morphology and different sensitivity to the applied electric field, suggesting that the folding orientation of the strands affects the molecular structure, i.e. either the tetrad unit or the tetrad-tetrad stacking or both, and therefore the charge mobility. Tetra-molecular G4-DNA is found to be twice as polarizable as intra-molecular G4-DNA, suggesting it has greater charge mobility. These promising results motivated direct electrical transport measurements on tetra-molecular G4-DNA using a special conductive AFM setup, profiling the conductance along the molecule. Reproducible currents of tens to over 100 pA were measured in many tetra-molecular G4-DNA molecules over ...

  16. Increased lymphangiogenesis in Riedel thyroiditis (Immunoglobulin G4-related thyroid disease).

    Science.gov (United States)

    Cameselle-Teijeiro, José; Ladra, María Jesús; Abdulkader, Ihab; Eloy, Catarina; Soares, Paula; Barreiro, Francisco; Sobrinho-Simões, Manuel; Beiras-Iglesias, Andrés

    2014-09-01

    The present study describes in depth a case of Riedel thyroiditis (RT) to clarify its pathogenesis and its putative inclusion in the spectrum of IgG4-related disease. We report the clinicopathological, immunohistochemical, and ultrastructural features of a case of RT in a 39-year-old white Spanish woman, admitted with a hard goiter and cold nodule in the left thyroid lobe. This case represents 0.05 % of a series of 1,973 consecutive thyroidectomies performed in our hospital. More than 80 % of the left thyroid lobe was effaced by fibrosis and inflammation (lymphocytes, 57 IgG4+ plasma cells per 1 high-power field, an IgG4/IgG ratio of 0.67, and eosinophils) with extension into the surrounding tissues and occlusive phlebitis. Immunostaining for podoplanin (D2-40) detected signs of increased lymphangiogenesis in the fibroinflammatory areas that were confirmed by electron microscopy. A strong, diffuse stain for podoplanin and transforming growth factor ß1 was also detected in the same areas. The increased number of lymphatic vessels in RT is reported for the first time. Our findings support the inclusion of RT within the spectrum of IgG4-related thyroid disease (IgG4-RTD). Although the etiology and physiopathology of IgG4-RTD still remain elusive, the results obtained in the present case suggest the participation of lymphatic vessels in the pathogenesis of RT.

  17. Colovesical fistula caused by glucocorticoid therapy for IgG4-related intrapelvic mass.

    Science.gov (United States)

    Yabuuchi, Yohei; Matsubayashi, Hiroyuki; Matsuzaki, Masato; Shiomi, Akio; Moriguchi, Michihisa; Kawamura, Ichiro; Ito, Ichiro; Ono, Hiroyuki

    2015-12-16

    IgG4-related disease (IgG4-RD) is an immune-mediated fibroinflammatory disorder that can occur in almost all systemic organs and generally responds to corticosteroid treatment. We report a rare case of an IgG4-related intrapelvic mass lesion that responded to steroid therapy but caused a fistula between the sigmoid colon and bladder. A 71-year-old man was followed after treatment for hepatocellular carcinoma. Follow-up computed tomography (CT) incidentally depicted left hydronephrosis with an ill-demarcated intrapelvic mass lesion. This lesion was histologically diagnosed as IgG4-RD by open biopsy, and peroral steroid therapy was initiated. One month after starting steroids, a colovesical fistula was detected by follow-up CT. A colostomy and urethral catheterization were emergently performed. The patient recovered and the mass lesion was drastically minimized by the initiation of glucocorticoids; however, he still needs urethral catheterization. IgG4-RD develops in various systemic organs and generally responds well to steroids. Clinicians must be watchful for the complications of responses to corticosteroids, such as fistulization, when the mass lesion of IgG4-RD is adjacent to multiple luminal organs.

  18. An aortoduodenal fistula as a complication of immunoglobulin G4-related disease

    Institute of Scientific and Technical Information of China (English)

    Momir Sarac; Ivan Marjanovic; Mihailo Bezmarevic; Uros Zoranovic; Stanko Petrovic; Miodrag Mihajlovic

    2012-01-01

    Most primary aortoduodenal fistulas occur in the presence of an aortic aneurysm,which can be part of immunoglobulin G4 (IgG4)-related sclerosing disease.We present a case who underwent endovascular grafting of an aortoduodenal fistula associated with a high serum IgG4 level.A 56-year-old male underwent urgent endovascular reconstruction of an aortoduodenal fistula.The patient received antibiotics and other supportive therapy,and the postoperative course was uneventful,however,elevated levels of serum IgG,IgG4 and C-reactive protein were noted,which normalized alter the introduction of steroid therapy.Control computed tomography angiography showed no endoleaks.The primary aortoduodenal fistula may have been associated with IgG4-related sclerosing disease as a possible complication of IgG4-related inflammatory aortic aneurysm.Endovascular grafting of a primary aortoduodenal fistula is an effective and minimally invasive alternative to standard surgical repair.

  19. Evolution of human G4P[8] group A rotavirus strains circulating in Italy in 2013.

    Science.gov (United States)

    Ianiro, Giovanni; Delogu, Roberto; Fiore, Lucia; Ruggeri, Franco M

    2015-06-02

    Group A rotaviruses (RVA) are the leading cause of acute gastroenteritis in young (humans worldwide are associated with the five major G/P combinations G1P[8], G2P[4], G3P[8], G4P[8] and G9P[8]. During RVA gastroenteritis surveillance in Italy, a total of 1112 samples collected from children hospitalized with acute gastroenteritis in 2013 were RVA positive and were genotyped following standardized protocols from the EuroRotaNet. Most strains analyzed belonged to the five major human genotypes. Among these common strains, 22 G4P[8] RVA strains from different Italian regions were subjected to nucleotide sequencing of their VP4, VP6, VP7 and NSP4 genes to investigate their evolution. The phylogenetic analysis showed that the Italian strains belonged to lineage G4-I for VP7 and to lineage P[8]-III for VP4, in line with the modern G4P[8] RVA strains detected in children worldwide. The phylogenetic trees revealed high degrees of nucleotide identity between the RVA strains involved in this study and G4P[8] strains detected previously in Europe, Asia and Africa, but also demonstrated at least three separate evolution clusters within the same lineage. Based on the amino acid sequences deduced for their hypervariable regions, both the VP7 and VP8* proteins of the Italian G4P[8] RVA strains presented amino acid substitutions near known neutralizing epitopes.

  20. Increased IgG4 responses to multiple food and animal antigens indicate a polyclonal expansion and differentiation of pre-existing B cells in IgG4-related disease

    OpenAIRE

    Culver, EL; Vermeulen, E.; Makuch, M; Leeuwen, A. van; Sadler, R.; Cargill, T; Klenerman, P; Aalberse, RC; van Ham, SM; Barnes, E; Rispens, T

    2015-01-01

    Background: IgG4-related disease (IgG4-RD) is a systemic fibroinflammatory condition, characterised by an elevated serum IgG4 concentration and abundant IgG4-positive plasma cells in the involved organs. An important question is whether the elevated IgG4 response is causal or a reflection of immune-regulatory mechanisms of the disease. Objectives: To investigate if the IgG4 response in IgG4-RD represents a generalised polyclonal amplification by examining the response to common environmental ...

  1. Unusual IgG4-related hypophysitis: one case report and analysis of clinicopathological characteristics

    Directory of Open Access Journals (Sweden)

    Zhen-qi LI

    2014-10-01

    Full Text Available Background Immunoglobulin G4 (IgG4-related disease is a recently characterized autoimmune disease entity marked by elevated serum IgG4 levels and tissue infiltration by IgG4-positive plasma cells in multiple involved organs. Hypophysitis is a rare inflammatory disorder and IgG4-related sclerosing disease involving the ituitary alone is especially rare. Imaging studies may reveal a mass lesion in the sellar area or a thickening of pituitary stalk, mimicking a pituitary tumor. Due to its rarity and non-specific appearance in radiological examination, it is a diagnostic challenge for clinicians and histopathologists to differentiate solitary IgG4-related hypophysitis from other pituitary lesions. The aim of this study is to summarize the clinicopathological features of unusual IgG4-related hypophysitis and discuss the differential diagnosis of histologically similar inflammatory lesions in pituitary. Methods The clinical manifestation of a patient with solitary IgG4-related hypophysitis was presented retrospectively. Resected mass was routinely paraffin-embedded and stained with Hematoxylin and Eosin. Dako EnVision immunohistochemical staining system was used to detect the tumor antigen expressions, including vimentin (Vim, S-100 protein (S-100, pan cytokeratin (PCK, epithelial membrane antigen (EMA, CD3, CD20, CD68, CD1a, κ-light chain, λ-light chain and progestrone receptor (PR.  Results A 47-year-old male patient presented with 1-year history of mild limb weakness and hyposexuality. Laboratory examination revealed hypopituitarism with low levels of serum testosterone, cortisol, luteinizing hormone (LH and follicle stimulating hormone (FSH, although his serum IgG4 level was high. MRI of the pituitary gland revealed a mass lesion in the sellar area with T1WI mild hyperintense and homogeneous enhancement after gadolinium administration. The patient underwent a transsphenoidal mass resection of the pituitary gland. Histological examination

  2. Acute tubulointerstitial nephritis with severe renal impairment associated with multisystem IgG4-related disease.

    Science.gov (United States)

    Beltrame, Rafael Coimbra Ferreira; Friderichs, Maurício; Fior, Bárbara Rayanne; Schaefer, Pedro Guilherme; Thomé, Gustavo Gomes; Silva, Dirceu Reis da; Barros, Elvino José Guardão; Seligman, Renato; Veronese, Francisco Veríssimo

    2016-01-01

    The IgG4-related disease has a wide clinical spectrum where multiple organs can be affected, and the diagnosis depends on typical histopathological findings and an elevated IgG4 expression in plasma cells in the affected tissue. We describe the clinical presentation and evolution of a patient with acute tubulointerstitial nephritis, severe kidney failure and systemic manifestations such as lymphadenomegaly and chronic pancreatitis. The diagnosis was confirmed by the clinical picture and kidney and lymph node histopathology, in which immunohistochemistry of the lymphoid tissue showed policlonality and increased expression of IgG4, with a IgG4/total IgG ratio > 80%. The patient was treated with prednisone at a dose of 60 mg/day, followed by mycophenolate mofetil, and showed clinical and renal function improvement at 6 months of follow-up. The high index of suspicion of IgG4-related disease with multisystem involvement and the early treatment of this condition are essential to improve the prognosis of affected patients. Resumo A doença relacionada à IgG4 tem um espectro clínico amplo em que múltiplos órgãos podem ser afetados, e o diagnóstico depende de achados histopatológicos típicos e elevada expressão de IgG4 em plasmócitos no tecido afetado. Descrevemos o quadro clínico e a evolução de um paciente com nefrite túbulo-intersticial aguda, insuficiência renal grave e manifestações sistêmicas como linfoadenomegalias e pancreatite crônica. O diagnóstico foi confirmado pelas características clínicas e pela histopatologia renal e de linfonodo, na qual a imunohistoquímica mostrou tecido linfoide com policlonalidade e expressão aumentada de IgG4, com uma relação IgG4/IgG total > 80%. O paciente foi tratado com prednisona na dose de 60 mg/dia, seguido de micofenolato mofetil, e apresentou melhora clínica e da função renal depois de 6 meses de tratamento. O alto índice de suspeição da doença relacionada ao IgG4 com comprometimento multissist

  3. Immunglobulin G4-relateret sygdom er en sjælden differentialdiagnose til maligne og autoimmune sygdomme

    DEFF Research Database (Denmark)

    Storgaard, Anders; Detlefsen, Sönke

    2015-01-01

    Immunoglobulin G4-related disease (IgG4-RD) is an inflammatory and fibrotic disease with the potential to produce diffuse enlargement, massforming lesions or stenoses in a wide range of organs. Elevation of serum IgG4 concentration and high levels of IgG4-positive cells in the inflamed tissue...

  4. Role of endoscopy in the diagnosis of autoimmune pancreatitis and immunoglobulin G4-related sclerosing cholangitis.

    Science.gov (United States)

    Kamisawa, Terumi; Ohara, Hirotaka; Kim, Myung Hwan; Kanno, Atsushi; Okazaki, Kazuichi; Fujita, Naotaka

    2014-09-01

    Autoimmune pancreatitis (AIP) must be differentiated from pancreatic carcinoma, and immunoglobulin (Ig)G4-related sclerosing cholangitis (SC) from cholangiocarcinoma and primary sclerosing cholangitis (PSC). Pancreatographic findings such as a long narrowing of the main pancreatic duct, lack of upstream dilatation, skipped narrowed lesions, and side branches arising from the narrowed portion suggest AIP rather than pancreatic carcinoma. Cholangiographic findings for PSC, including band-like stricture, beaded or pruned-tree appearance, or diverticulum-like outpouching are rarely observed in IgG4-SC patients, whereas dilatation after a long stricture of the bile duct is common in IgG4-SC. Transpapillary biopsy for bile duct stricture is useful to rule out cholangiocarcinoma and to support the diagnosis of IgG4-SC with IgG4-immunostaining. IgG4-immunostaining of biopsy specimens from the major papilla advances a diagnosis of AIP. Contrast-enhanced endoscopic ultrasonography (EUS) and EUS elastography have the potential to predict the histological nature of the lesions. Intraductal ultrasonographic finding of wall thickening in the non-stenotic bile duct on cholangiography is useful for distinguishing IgG4-SC from cholangiocarcinoma. Endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA) is widely used to exclude pancreatic carcinoma. To obtain adequate tissue samples for the histological diagnosis of AIP, EUS-Tru-cut biopsy or EUS-FNA using a 19-gauge needle is recommended, but EUS-FNA with a 22-gauge needle can also provide sufficient histological samples with careful sample processing after collection and rapid motion of the FNA needles within the pancreas. Validation of endoscopic imaging criteria and new techniques or devices to increase the diagnostic yield of endoscopic tissue sampling should be developed.

  5. Prohibitin Is Involved in Patients with IgG4 Related Disease.

    Directory of Open Access Journals (Sweden)

    Hongwu Du

    Full Text Available IgG4-related disease (IgG4-RD is a chronic systemic disease involved in many organs and tissues. As only limited autoantigens have been found since the beginning of this century, the aim of this study was to reveal new candidate autoantigens of IgG4-RD.Multiple cell lines including HT-29, EA.hy926, HEK 293 and HepG2 were used to test the binding ability of circulating autoantibodies from IgG4-RD sera. The amino-acid sequence was then analyzed by matrix-assisted laser desorption/ionization time-of-flight tandem (MALDI-TOF/TOF mass spectrometry. After the cloning and expression of recombinant putative autoantigen in a bacterial expression system, the corresponding immuno assay was set up and utilized to observe the prevalence of serum autoantibodies in a large set of confirmed clinical samples.One positive autoantigen was identified as prohibitin. ELISA analysis showed that a majority of patients with IgG4-RD have antibodies against prohibitin. Anti-prohibitin antibodies were present in the sera of patients with definite autoimmune pancreatitis (25/34; 73.5%, Mikulicz's disease (8/15; 53.3%, retroperitoneal fibrosis (6/11; 54.5%, other probable IgG4-RD (26/29; 89.7% and Sjögren's syndrome (4/30; 13.3% but not in apparently healthy donors (1/70; 1.4%.An association between prohibitin and patients with some IgG4-RD was observed, although the results were quite heterogeneous among different individuals within autoimmune pancreatitis, Mikulicz's disease and retroperitoneal fibrosis.

  6. Idiopathic granulomatous orchitis: morphology and evaluation of its relationship to IgG4 related disease.

    Science.gov (United States)

    Karram, Sarah; Kao, Chia-Sui; Osunkoya, Adeboye O; Ulbright, Thomas M; Epstein, Jonathan I

    2014-04-01

    Idiopathic granulomatous orchitis (IGO) is rare, thought to result from an autoimmune reaction to spermatogenic elements. Its relationship to IgG4-related disease (IgG4-RD) has not been evaluated. Sixteen orchiectomy specimens (1984-2012) with a prominent intratubular granulomatous reaction were reviewed: IGO (n = 6); intratubular germ cell neoplasia unclassified (IGCNU) with a granulomatous reaction and associated seminoma (GS, n = 6); and unclassified intratubular granulomatous orchitis not fitting into a specific entity (UGO, n = 4). Men with IGO were 32 to 86 years old, presenting with a mass suspicious for malignancy. Only one patient had a history of an inflammatory disease. Clinical follow-up was available for 2 patients with IGO, and both had no evidence of systemic IgG4-RD. All IGO cases had an epithelioid granulomatous reaction confined to seminiferous tubules, an extensive interstitial lymphoplasmacytic inflammation, 3 of 6 had prominent interstitial fibrosis, and 3 of 6 cases had plasma cells with an IgG4+/IgG+ ratio >40%. In GS, 10% to 100% of tubules with IGCNU had a granulomatous reaction, which in 3 cases replaced IGCNU cells. In contrast to IGO, GS had more intratubular multinucleated giant cells, more peritubular sclerosis, fewer interstitial plasma cells, and no interstitial fibrosis. Of the 4 UGO cases, most had predominantly interstitial with less intratubular granulomatous inflammation. Only 1 non-IGO case had elevated tissue IgG4 (GS case). It is critical and sometimes difficult to distinguish GS from IGO. IGO shares some features with IgG4-RD, yet current evidence does not support its classification as a localized manifestation of IgG4-RD occurring in the testis.

  7. Increased IgG4 responses to multiple food and animal antigens indicate a polyclonal expansion and differentiation of pre-existing B cells in IgG4-related disease

    Science.gov (United States)

    Culver, Emma L; Vermeulen, Ellen; Makuch, Mateusz; van Leeuwen, Astrid; Sadler, Ross; Cargill, Tamsin; Klenerman, Paul; Aalberse, Rob C; van Ham, S Marieke; Barnes, Eleanor; Rispens, Theo

    2015-01-01

    Background IgG4-related disease (IgG4-RD) is a systemic fibroinflammatory condition, characterised by an elevated serum IgG4 concentration and abundant IgG4-positive plasma cells in the involved organs. An important question is whether the elevated IgG4 response is causal or a reflection of immune-regulatory mechanisms of the disease. Objectives To investigate if the IgG4 response in IgG4-RD represents a generalised polyclonal amplification by examining the response to common environmental antigens. Methods Serum from 24 patients with IgG4-RD (14 treatment-naive, 10 treatment-experienced), 9 patients with primary sclerosing cholangitis and an elevated serum IgG4 (PSC-high IgG4), and 18 healthy controls were tested against egg white and yolk, milk, banana, cat, peanut, rice and wheat antigens by radioimmunoassay. Results We demonstrated an elevated polyclonal IgG4 response to multiple antigens in patients with IgG4-RD and in PSC-high IgG4, compared with healthy controls. There was a strong correlation between serum IgG4 and antigen-specific responses. Responses to antigens were higher in treatment-naive compared with treatment-experienced patients with IgG4-RD. Serum electrophoresis and immunofixation demonstrated polyclonality. Conclusions This is the first study to show enhanced levels of polyclonal IgG4 to multiple antigens in IgG4-RD. This supports that elevated IgG4 levels reflect an aberrant immunological regulation of the overall IgG4 response, but does not exclude that causality of disease could be antigen-driven. PMID:25646372

  8. [PAL-1 5G/4G polymorphism in patients with systemic lupus erythematosus].

    Science.gov (United States)

    Savov, A; Andonova, S; Tanev, D; Robeva, R; Marincheva, Ts; Tomova, A; Kumanov, Ph; Rashkov, R; Kolarov, Zl

    2014-01-01

    Systemic lupus erythematosus (SLE) is a connective tissue disease affecting predominantly women that has been widely associated with obstetric complications. Inherited thrombophilias are significant risk factors for pregnancy loss, but their role in patients with SLE, and especially in those without concomitant secondary antiphospholipid syndrome (APS) has not been clarified. The aim of the present study was to study PAI-1 5G/4G polymorphism in women with lupus. A total of 103 SLE patients as well as 69 healthy volunteers were genotyped for PAI-1 5G/4G (rs1799889). No significant differences in the PAI-1 5G/4G genotype prevalence between patients and controls were found. After exclusion of the women with secondary APS, the frequency of pregnancies and spontaneous abortions, as well as the number of live births were similar in the studied patients with different PAI-1 genotype (p> 0.05). PAI-1 5G/4G polymorphism was not significantly related to any of the lupus ACR criteria or disease activity (p > 0.05), but it could influence the platelet number in the studied patients (263.52 ± 91.10 [5G/5G genotype] versus 210.12 ± 71.79 [4G/4G genotype], p = 0.023). In conclusion, our results showed that PAI-1 4G/5G polymorphism did not worsen the reproductive outcome in SLE women without secondary APS.

  9. Isolated Liver Hilar Infiltration by IgG4 Inflammation Mimicking Cholangiocarcinoma

    Science.gov (United States)

    Bochatay, Laurent; Majno, Pietro; Giostra, Emiliano; Frossard, Jean Louis

    2016-01-01

    IgG4-related disease represents a heterogeneous group of disease characterized by infiltration of various tissues by IgG4 plasmocytes. In case of liver infiltration, this condition classically mimics primary sclerosing cholangitis or multifocal cholangiocarcinoma due to inflammation that preferentially affects the intra- and extrahepatic bile duct. Diagnostic criteria have recently been reviewed in order to better define the disease and help physicians make the diagnosis. Herein, we present the case of a patient who died after liver surgery for suspected cholangiocarcinoma that finally turned out to be IgG4-associated liver disease, a condition being out of current consensual criteria. The patient presented with progressive cholestasis identified by MR cholangiography as an isolated hilar mass responsible for dilatation of the left and right intrahepatic bile duct suspicious for a Klatskin tumor. The IgG4 blood level was normal as was biliary cytology. The patient underwent right portal embolization followed by right extended hepatectomy. Pathologic examination found no tumor but intense fibrosclerotic infiltration with a marked inflammatory infiltrate characterized by IgG4-positive plasmocytes. Despite immunosuppressive treatment, cholestasis was never controlled and successive biopsies of the remaining liver showed progressive cholestasis, liver infiltrate and no bile duct regeneration. The patient finally presented an upper gastrointestinal hemorrhage leading to death 4 months after hepatectomy and appropriate immunosuppressive therapy. PMID:27843427

  10. Acute tubulointerstitial nephritis with severe renal impairment associated with multisystem IgG4-related disease

    Directory of Open Access Journals (Sweden)

    Rafael Coimbra Ferreira Beltrame

    Full Text Available Abstract The IgG4-related disease has a wide clinical spectrum where multiple organs can be affected, and the diagnosis depends on typical histopathological findings and an elevated IgG4 expression in plasma cells in the affected tissue. We describe the clinical presentation and evolution of a patient with acute tubulointerstitial nephritis, severe kidney failure and systemic manifestations such as lymphadenomegaly and chronic pancreatitis. The diagnosis was confirmed by the clinical picture and kidney and lymph node histopathology, in which immunohistochemistry of the lymphoid tissue showed policlonality and increased expression of IgG4, with a IgG4/total IgG ratio > 80%. The patient was treated with prednisone at a dose of 60 mg/day, followed by mycophenolate mofetil, and showed clinical and renal function improvement at 6 months of follow-up. The high index of suspicion of IgG4-related disease with multisystem involvement and the early treatment of this condition are essential to improve the prognosis of affected patients.

  11. Isolated Liver Hilar Infiltration by IgG4 Inflammation Mimicking Cholangiocarcinoma

    Directory of Open Access Journals (Sweden)

    Laurent Bochatay

    2016-10-01

    Full Text Available IgG4-related disease represents a heterogeneous group of disease characterized by infiltration of various tissues by IgG4 plasmocytes. In case of liver infiltration, this condition classically mimics primary sclerosing cholangitis or multifocal cholangiocarcinoma due to inflammation that preferentially affects the intra- and extrahepatic bile duct. Diagnostic criteria have recently been reviewed in order to better define the disease and help physicians make the diagnosis. Herein, we present the case of a patient who died after liver surgery for suspected cholangiocarcinoma that finally turned out to be IgG4-associated liver disease, a condition being out of current consensual criteria. The patient presented with progressive cholestasis identified by MR cholangiography as an isolated hilar mass responsible for dilatation of the left and right intrahepatic bile duct suspicious for a Klatskin tumor. The IgG4 blood level was normal as was biliary cytology. The patient underwent right portal embolization followed by right extended hepatectomy. Pathologic examination found no tumor but intense fibrosclerotic infiltration with a marked inflammatory infiltrate characterized by IgG4-positive plasmocytes. Despite immunosuppressive treatment, cholestasis was never controlled and successive biopsies of the remaining liver showed progressive cholestasis, liver infiltrate and no bile duct regeneration. The patient finally presented an upper gastrointestinal hemorrhage leading to death 4 months after hepatectomy and appropriate immunosuppressive therapy.

  12. IgG4-related multiorgan disease: report of the first autopsy case

    Science.gov (United States)

    Ochoa, Minerva Lazos; Gabiño López, Belem; Cabello, Raúl Romero; Feregrino, Raúl Romero

    2013-01-01

    IgG4-related disease (IgG4RD) is a chronic recurring fibro-inflammatory pathology that is considered to be of autoimmune origin. Histopathology is considered to be the gold standard method for diagnosis. IgG4RD affects multiple organs. IgG4RD was first identified in the pancreas and was called autoimmune pancreatitis (AIP). During the following years, the disease spectrum was expanded and it was realised that the extrapancreatic lesions can precede, coexist or appear after the diagnosis of AIP. At present, several illnesses such as Mikulicz disease, Küttner tumour, multifocal fibrosclerosis, etc, are considered to be part of the IgG4RD spectrum. The symptoms of the disease tend to appear over months and years and diagnosis is achieved on average 13.5 months (4–60 months) after the onset. The purpose of this report was to provide information about a case that was sadly fatal but that permitted a complete histopathological study of the damaged tissues. PMID:23645656

  13. Natural Mosquito-Pathogen Hybrid IgG4 Antibodies in Vector Borne Diseases: A Hypothesis

    Directory of Open Access Journals (Sweden)

    Berlin L. Londono-Renteria

    2016-09-01

    Full Text Available Chronic exposure to antigens may favor the production of IgG4 antibodies over other antibody types. Recent studies have shown that up to a 30% of normal human IgG4 is bi-specific and is able to recognize two antigens of different nature. A requirement for this specificity is the presence of both eliciting antigens in the same time and at the same place where the immune response is induced. During transmission of most vector-borne diseases, the pathogen is delivered to the vertebrate host along with the arthropod saliva during blood feeding and previous studies have shown the existence of IgG4 antibodies against mosquito salivary allergens. However, there is very little ongoing research or information available regarding IgG4 bi-specificity with regards to infectious disease, particularly during immune responses to vector-borne diseases such as malaria, filariasis or dengue virus infection. Here, we provide background information and present our hypothesis that IgG4 may not only be a useful tool to measure exposure to infected mosquito bites, but that these bi-specific antibodies may also play an important role in modulation of the immune response against malaria and other vector-borne diseases in endemic settings.

  14. Autoimmune Pancreatitis and IgG4 Related Disease in Three Children

    Science.gov (United States)

    Chong, Sze Yee; Coleman, Lee; MacGregor, Duncan; Hardikar, Winita; Oliver, Mark R.

    2016-01-01

    We report 3 children who presented with fever and abdominal pain, deranged liver function tests, and on abdominal ultrasound were found to have an enlarged pancreas, substantial abdominal lymphadenopathy, and extrahepatic biliary duct dilatation. After ruling out malignancy, probable immunoglobulin G4-related disease (IgG4RD) associated with autoimmune pancreatitis was considered. This condition was first described in the adults and often mimics pancreatic cancer. It can involve multiple organs, either synchronously or metachronously, and is rarely reported in children. The disorder mostly responds to corticosteroid therapy and other immune suppression. We highlight the difficulty in diagnosing autoimmune pancreatitis/IgG4-related disease in children and illustrate the difference between pediatric and adult presentation. PMID:27622194

  15. Autoimmune pancreatitis metachronously associated with retroperitoneal fibrosis with IgG4-positive plasma cell infiltration

    Institute of Scientific and Technical Information of China (English)

    Terumi Kamisawa; Pong Yui Chen; Yuyang Tu; Hitoshi Nakajima; Naoto Egawa

    2006-01-01

    Retroperitoneal fibrosis is an uncommon disorder characterized by the formation of a dense plaque of fibrous tissue in the retroperitoneum, and its etiology remains unknown. Autoimmune pancreatitis is a rare type of chronic pancreatitis characterized by fibrosis with abundant infiltration of IgG4-positive plasma cells and lymphocytes and obliterative phlebitis in the pancreas. We present a case of autoimmune pancreatitis that developed 10 mo after the occurrence of retroperitoneal fibrosis. Histological findings of the resected retroperitoneal mass were marked periureteral fibrosis with abundant infiltration of IgG4-positive plasma cells and lymphocytes and obliterative phlebitis.These findings suggest a common pathophysiological mechanism for retroperitoneal fibrosis and autoimmune pancreatitis in this case. Some cases of retroperitoneal fibrosis might be a retroperitoneal lesion of IgG4-related sclerosing disease.

  16. Topiramate-induced maculopathy in IgG4-related disease

    Directory of Open Access Journals (Sweden)

    DaCosta J

    2016-06-01

    Full Text Available Joanna DaCosta,1,2 Saad Younis1 1Ophthalmology Department, Imperial College Healthcare NHS Trust, Western Eye Hospital, 2Barts Health NHS Trust, Whipps Cross University Hospital, London, UK Abstract: This report describes a case of reversible topiramate-induced maculopathy in a 32-year-old female patient with IgG4-related disease. The patient presented with decreased vision associated with anterior uveitis and cystoid macula edema, which was unresponsive to oral and topical steroids. Following topiramate cessation, both cystoid macula edema and vision improved. The ocular side effects of topiramate and putative pharmacological mechanisms for topiramate-induced maculopathy in the context of IgG4-related disease are discussed. This report highlights that neurologists and ophthalmologists should be aware that patients presenting with topiramate-associated maculopathy should be advised to discontinue topiramate promptly to prevent irreversible loss of vision. Keywords: IgG4 disease, maculopathy, topiramate

  17. Hypocholesterolaemic effect of yoghurt containing Bifidobacterium pseudocatenulatum G4 or Bifidobacterium longum BB536.

    Science.gov (United States)

    Al-Sheraji, Sadeq Hasan; Ismail, Amin; Manap, Mohd Yazid; Mustafa, Shuhaimi; Yusof, Rokiah Mohd; Hassan, Fouad Abdulrahman

    2012-11-15

    The effect of a yoghurt supplement containing Bifidobacterium pseudocatenulatum G4 or Bifidobacterium longum BB536 on plasma lipids, lipid peroxidation and the faecal excretion of bile acids was examined in rats fed a cholesterol-enriched diet. After 8 weeks, the rats in the positive control (PC) group who were fed the cholesterol-enriched diet showed significant increases in plasma total cholesterol (TC), low-density lipoprotein (LDL) cholesterol, and malondialdehyde (MDA). However, groups fed a cholesterol-enriched diet supplemented with yoghurt containing B. pseudocatenulatum G4 or B. longum BB536 had significantly lower plasma TC, LDL-C, very-low-density lipoprotein (VLDL) cholesterol, and MDA than had the PC group after 8 weeks of treatment. In addition, faecal excretion of bile acids was markedly increased in the rats fed the yoghurt containing B. pseudocatenulatum G4 or B. longum BB536 as compared to the PC and NC groups.

  18. Membranous glomerulopathy and massive cervical lymphadenopathy due to immunoglobulin G4-disease

    Directory of Open Access Journals (Sweden)

    Kamel El-Reshaid

    2017-01-01

    Full Text Available A 32-year-old male presented with acute and severe nephrotic syndrome as well as massive right cervical lymphadenopathy for <2 years. Computed tomography scan of the chest, abdomen, and pelvis did not reveal any lymphadenopathy. Histopathology and immunohistochemical testing of his lymph node biopsy showed infiltrate enriched with immunoglobulin G4 (IgG4-positive plasma cells. His kidney biopsy showed granular membranous deposits of IgG4 in the basement membrane without interstitial infiltrate. Antiphospholipid 2 receptor antibodies were absent excluding its "idiopathic" nature. Since he was allergic to rituximab, he was treated with corticosteroids for two months and a combination of tacrolimus and mycophenolate. His lymphadenopathy disappeared, and his proteinuria abated. The dose of the latter two medications was reduced to half after four months and will be maintained for a minimum of two years to prevent relapse of his disease.

  19. Contactin 1 IgG4 associates to chronic inflammatory demyelinating polyneuropathy with sensory ataxia.

    Science.gov (United States)

    Miura, Yumako; Devaux, Jérôme J; Fukami, Yuki; Manso, Constance; Belghazi, Maya; Wong, Anna Hiu Yi; Yuki, Nobuhiro

    2015-06-01

    A Spanish group recently reported that four patients with chronic inflammatory demyelinating polyneuropathy carrying IgG4 autoantibodies against contactin 1 showed aggressive symptom onset and poor response to intravenous immunoglobulin. We aimed to describe the clinical and serological features of Japanese chronic inflammatory demyelinating polyneuropathy patients displaying the anti-contactin 1 antibodies. Thirteen of 533 (2.4%) patients with chronic inflammatory demyelinating polyneuropathy had anti-contactin 1 IgG4 whereas neither patients from disease or normal control subjects did (P = 0.02). Three of 13 (23%) patients showed subacute symptom onset, but all of the patients presented with sensory ataxia. Six of 10 (60%) anti-contactin 1 antibody-positive patients had poor response to intravenous immunoglobulin, whereas 8 of 11 (73%) antibody-positive patients had good response to corticosteroids. Anti-contactin 1 IgG4 antibodies are a possible biomarker to guide treatment option.

  20. Unusual Polarization Properties of Supernova Remnant G4.8+6.2 at 1400 MHz

    Institute of Scientific and Technical Information of China (English)

    ZHANG Xi-Zhen(张喜镇); R.G.Strom; W.Reich

    2003-01-01

    Unusual polarization properties of the high galactic latitude supernova remnant (SNR) G4.8+6.2 are reported.The percentage polarization is larger than 70% over the two main shells of G4.8+6.2 at 1400MHz. It is the second SNR with such high polarization at such a Iow frequency among the known 225 SNRs. We show that this is very unusual for an SNR. Its morphology and polarization are very similar to those of the supernova remnant DA530 (G93.0+6.5), which is another high Galactic latitude SNR.

  1. Immunoglobulin G4 -related kidney disease: A comprehensive pictorial review of the imaging spectrum, Mimickers, and clinicopathological characteristics

    Energy Technology Data Exchange (ETDEWEB)

    Seo, Ni Eun; Kim, Jin Hee; Byun, Jae Ho; Lee, Seung Soo; Kim, Hyoung Jung; Lee, Moon Gyu [Dept. of Radiology and Research Institute of Radiology, Asan Medical Center, University of Ulsan College of Medicine, Seoul (Korea, Republic of)

    2015-10-15

    Immunoglobulin G4 (IgG4)-related kidney disease (IgG4-KD) has recently been demonstrated to be an important part of IgG4-related sclerosing disease (IgG4-SD). However, since IgG4-KD is still relatively unfamiliar to radiologists and physicians as compared to IgG4-SD involving other organs, it could, therefore, be easily missed. In this article, we present a comprehensive pictorial review of IgG4-KD with regards to the imaging spectrum, mimickers, and clinicopathologic characteristics, based on our clinical experience with 48 patients during the past 13 years, as well as a literature review. Awareness of the broad imaging spectrum of IgG4-KD and differential diagnosis from its mimickers will thus facilitate its early diagnosis and treatment.

  2. 人体端粒G4-D NA稳定剂的研究进展%Study of human telomere G4-DNA stabilizers

    Institute of Scientific and Technical Information of China (English)

    郑小辉; 刘海英; 夏立新; 毛宗万

    2016-01-01

    Telomere maintenance plays a critical role in cancer progression.Approximately 85% human cancer cells maintain their telomere length through activation of telomerase.Other 15%of cancers maintain telomere length independently of telom-erase by alternative lengthening of telomeres (ALT)pathway. Both events are equally important for telomere length mainte-nance of cancer cells.Human telomere consists of a series of G rich DNA sequences,which could form G-quadruplex.The for-mation of this structure can block the extension of telomeres by telomerase or ALT,resulting in cancer cell death.Thereby,G-quadruplex has been one of the focuses of anticancer therapy in recent years.This review focuses on the latest progress of G-quadruplex stabilizers.%端粒长度的维持在肿瘤细胞的永生化过程中起到至关重要的作用。约85%的人体肿瘤细胞通过端粒酶延伸端粒,从而获得持续的增殖能力。另外,15%的人体肿瘤细胞通过端粒替代延伸机制(alternative lengthening of telomeres, ALT)延伸端粒。这两种机制对于维持肿瘤细胞中端粒的长度具有同等重要的意义。人体端粒由富含鸟嘌呤(G)的DNA重复序列组成,该序列在特定的条件下可以形成G-四链体(G4)的结构。此结构的形成可以从根本上抑制端粒酶和ALT对端粒的延伸而达到抗肿瘤的目的。因此,人体端粒G4-DNA作为抗肿瘤靶点的研究是近年来抗肿瘤研究的重要前沿领域之一。该文重点综述人体端粒G4-DNA稳定剂研发的最新研究进展。

  3. Recent advances in IgG4-related lung disease%免疫球蛋白G4相关性肺疾病研究进展

    Institute of Scientific and Technical Information of China (English)

    张卉; 冯瑞娥

    2011-01-01

    @@ 近年来随着对免疫球蛋白G4(immunoglobulin G4,IgG4)相关性硬化性疾病的关注,越来越多的研究结果表明,IgG4相关性硬化性疾病是一组系统性疾病,除胰腺外,还可累及多个器官及组织,关于肺的IgG4相关性疾病也逐渐出现相关报道.IgG4相关性疾病以血清IgG4浓度升高、IgG4阳性浆细胞浸润及不规则纤维化为特征.

  4. Three Cases of Bronchial Asthma Preceding IgG4-Related Autoimmune Pancreatitis

    Directory of Open Access Journals (Sweden)

    Satoru Ito

    2012-01-01

    Discussion: It is possible that atopy and increased Th2 cell activity are related to a higher coincidence of IgG4-related diseases such as type 1 autoimmune pancreatitis. Because the present cases are few in number, further studies are necessary.

  5. Comparable profiles of serum histamine and IgG4 levels in allergic beekeepers.

    Science.gov (United States)

    Chliva, C; Aggelides, X; Makris, M; Katoulis, A; Rigopoulos, D; Tiligada, E

    2015-04-01

    Growing evidence identifies histamine as a key player in allergic responses, but the reports relating serum histamine to tolerance are scarce and inconclusive. This study investigated the relationship of circulating histamine to the tolerant phenotype in allergic beekeepers. The results showed a positive correlation between the serum levels of histamine and specific immunoglobulin G4 (sIgG4) to honeybee venom, but not with total IgE or sIgE. Interestingly, both sIgG4 and histamine levels were negatively correlated with the time since the last bee sting. In contrast to total IgE and sIgE, serum sIgG4 and histamine levels followed comparable patterns, being higher in tolerant/recently stung individuals and lower in the least frequently stung subgroup. The data obtained in this pilot study associated, for the first time, serum histamine levels with allergen tolerance in allergic individuals and provided the lead for further considering the putative immunomodulatory properties of histamine in allergic responses.

  6. Isolated IgG4-related cholecystitis mimicking gallbladder cancer: a case report.

    Science.gov (United States)

    Shin, Sang-Wook; Kim, Yongsoo; Jeong, Woo Kyoung; Kim, Jinoo; Kim, Min Yeong; Oh, Young Ha; Pyo, Ju Yeon

    2013-01-01

    A 58-year-old man with right upper quadrant pain was referred to the radiology department. The patient underwent computed tomography and magnetic resonance imaging, which suggested the diagnosis of gallbladder cancer invading the liver. After surgical removal of the gallbladder, and the adjacent liver parenchyma was performed, the histologic diagnosis of IgG4-related cholecystitis was made.

  7. IgG4-Related Disease Presenting as Recurrent Mastoiditis With Central Nervous System Involvement

    Directory of Open Access Journals (Sweden)

    April L. Barnado MD

    2013-09-01

    Full Text Available We report a case of a 43-year-old female who presented with right ear fullness and otorrhea. She was initially diagnosed with mastoiditis that was not responsive to multiple courses of antibiotics and steroids. She was then diagnosed with refractory inflammatory pseudotumor, and subsequent treatments included several mastoidectomies, further steroids, and radiation therapy. The patient went on to develop mastoiditis on the contralateral side as well as central nervous system involvement with headaches and right-sided facial paresthesias. Reexamination of the mastoid tissue revealed a significantly increased number of IgG4-positive cells, suggesting a diagnosis of IgG4-related disease. The patient improved clinically and radiographically with rituximab and was able to taper off azathioprine and prednisone. IgG4-related disease should be considered in patients with otologic symptoms and be on the differential diagnosis in patients with inflammatory pseudotumor. Staining for IgG and IgG4 is essential to ensure a prompt diagnosis and treatment.

  8. Relationship between chronic sclerosing dacryoadenitis with high level of IgG4 and Castleman disease

    Directory of Open Access Journals (Sweden)

    Toshiyuki Oshitari

    2010-12-01

    Full Text Available Toshiyuki Oshitari1, Jiro Yotsukura1, Kaoru Asahagi1, Takayuki Baba1, Takashi Kishimoto2, Shuichi Yamamoto11Department of Ophthalmology and Visual Science, 2Department of Molecular Pathology, Chiba University Graduate School of Medicine, Chiba, JapanAbstract: The purpose of this study is to present a case of chronic sclerosing dacryoadenitis with high level of IgG4 in a patient diagnosed earlier with Castleman disease. A 79-year-old man noticed a swelling of his lower left jaw that was first seen 8 years earlier. He was diagnosed with Castleman disease from the histopathological examination of a biopsy of the submandibular gland. Since then, the size of the gland had not changed, and he had no systemic inflammatory signs or symptoms. He developed diplopia a year earlier, and CT scans showed bilateral swelling of the lacrimal glands. He was referred to our hospital for further examinations. The patient underwent partial dacryoadenectomy. From the histopathological examinations, he was diagnosed with chronic sclerosing dacryoadenitis with high level of the serum IgG4. He underwent oral steroid therapy and the swollen lacrimal glands were significantly improved. The results suggest that there may be pathological links between IgG4-related dacryoadenitis and Castleman disease.Keywords: IgG4, dacryoadenitis, Castleman disease, steroid

  9. IgG4-related diseases and the common medical imaging diagnosis for IgG4-related pancreatitis%IgG4相关疾病概述及IgG4相关性胰腺炎的常见医学影像学诊断

    Institute of Scientific and Technical Information of China (English)

    王宝华

    2015-01-01

    IgG4相关疾病(IgG4-related disease,IgG4-RD)是一组2010年首次命名的系统性炎症纤维化疾病,已受到国内外学者的广泛关注,是一组原因不明的慢性进行性自身免疫性疾病.其受累器官广泛,几乎可累计全身各个器官,多器官同时或相继受累,也可只累及某一器官.影像学检查是IgG4-RD诊断与鉴别诊断的重要手段之一,为使影像医师掌握该疾病的相关知识和影像学表现,结合国内外有关文献现对其进行论述.

  10. Increased RP105-Negative B Cells in IgG4-Related Disease.

    Science.gov (United States)

    Koarada, S; Tashiro, S; Nagao, N; Suematsu, R; Ohta, A; Tada, Y

    2013-01-01

    Four patients with IgG4-related disease (IgG4-RD) showed increased percentages of RP105-negative B cells in the peripheral blood. Case 1: A 66-year-old man having retroperitoneal fibrosis had 18.8% of RP105-negative B cells. Oral prednisolone improved the affected lesions and the percentage of RP105-negative B cells decreased (3.2%) after the treatment. Case 2: A 53-year-old man with retroperitoneal fibrosis had 27.9% of RP105-negative B cells. Case 3: A 38-year-old man with follicular hyperplasia showed increased percentage of RP105-negative B cells (8.3%). Case 4: A 60-year-old man with interstitial nephritis had 27.5% of RP105-negative B cells. The treatment decreased the numbers of RP105-negative B cells. Increased numbers of RP105-negatvie B cells is possibly associated with disease activity of IgG4-RD. Analysis of expression of RP105 on B cells may be helpful in evaluation of disease activity of IgG4-RD.

  11. Exponential Optical Model for Attenuation in Tiles in G4 Simulation

    CERN Document Server

    Lawlor, D

    2006-01-01

    Changes made to the ATLAS Tile Calorimeter simulation to include the effect of light attenuation within the scintillating tiles are presented. The effect of attenuation was studied using data from the test beam as well as 90Sr source scans. A comparison with the default G4 simulation is presented, as are instructions to use this model in Athena release 12.0.0.

  12. Is IgG4-Related Disease a Cause of Xerostomia? A Cohort Study of 60 Patients

    Directory of Open Access Journals (Sweden)

    M. Hermet

    2012-01-01

    Full Text Available Objective. Immunoglobulin-G4-(IgG4- related disease (IgG4 RD is a fibrosing process characterized by a significant infiltration of IgG4-secreting plasma cells. IgG4 RD can affect almost all organs including salivary glands. Whether IgG4 RD plays a role in the development of sicca syndrome and particularly dry mouth syndrome remains to be investigated. Methods. We conducted a monocentric cohort study for two years to search for IgG4 RD features in patients with dry mouth syndrome using immunostainings of labial salivary gland specimens with anti-IgG4 antibody. Results. Among 60 patients presenting with dry mouth syndrome who underwent labial salivary gland biopsy, 18 showed positive immunostaining with the anti-IgG4 antibody including 4 patients with typical systemic IgG4 RD. Five also fulfilled criteria for Sjögren's syndrome. Conclusion. These findings suggest that clinical forms of IgG4 RD salivary involvement without salivary swelling may occur. This salivary involvement is probably overlooked in everyday practice and could represent a mild form of IgG4 RD.

  13. Chemotaxis of Pseudomonas stutzeri OX1 and Burkholderia cepacia G4 toward chlorinated ethenes.

    Science.gov (United States)

    Vardar, Gönül; Barbieri, Paola; Wood, Thomas K

    2005-03-01

    The chemotactic responses of Pseudomonas putida F1, Burkholderia cepacia G4, and Pseudomonas stutzeri OX1 were investigated toward toluene, trichloroethylene (TCE), tetrachloroethylene (PCE), cis-1,2-dichloroethylene (cis-DCE), trans-1,2-dichloroethylene (trans-DCE), 1,1-dichloroethylene (1,1-DCE), and vinyl chloride (VC). P. stutzeri OX1 and P. putida F1 were chemotactic toward toluene, PCE, TCE, all DCEs, and VC. B. cepacia G4 was chemotactic toward toluene, PCE, TCE, cis-DCE, 1,1-DCE, and VC. Chemotaxis of P. stutzeri OX1 grown on o-xylene vapors was much stronger than when grown on o-cresol vapors toward some chlorinated ethenes. Expression of toluene-o-xylene monooxygenase (ToMO) from touABCDEF appears to be required for positive chemotaxis attraction, and the attraction is stronger with the touR (ToMO regulatory) gene.

  14. The complete mitochondrial genome of the acid-tolerant fungus Penicillium ShG4C

    Directory of Open Access Journals (Sweden)

    Andrey V. Mardanov

    2016-12-01

    Full Text Available Complete mitochondrial genome of the acid-tolerant fungus Penicillium ShG4C, isolated from oxidized sediments of an abandoned polymetallic mine site, has been sequenced using high-throughput sequencing approach. The mitochondrial genome represents a circular DNA molecule with size of 26,725 bp. It encodes a usual set of mitochondrial genes, including 15 protein coding genes, large and small ribosomal RNAs and 27 tRNA genes. All genes are located on H-strand DNA and transcribed in one direction. Taxonomic analysis based on concatenated sequences of mitochondrial proteins confirmed taxonomic position of this fungus within the genus Penicillium. The sequence of the complete mitochondrial genome of Penicillium ShG4C was deposited in DBBJ/EMBL/GenBank under accession number KX931017.

  15. Design and Simulation of a Programmable Memory/Multiplier Array Using G4-FET Technology

    Science.gov (United States)

    2007-07-01

    Arrays........................ 12 4.2 Primitive Cell Design and Configuration ......................................................... 13 4.3...CMOS devices. 4.2 Primitive Cell Design and Configuration Using the cell architecture of the G4-FET adder as a guide, we developed metal mask...layout of the primitive cell template, as well as the cell configured as a majority gate and as 2 memory cells. The template contains the mask

  16. The changing faces of IgG4-related disease: Clinical manifestations and pathogenesis.

    Science.gov (United States)

    Islam, Arshia Duza; Selmi, Carlo; Datta-Mitra, Ananya; Sonu, Rebecca; Chen, Mingyi; Gershwin, M Eric; Raychaudhuri, Siba P

    2015-10-01

    Since the earliest reports in 2001, immunoglobulin G4 (IgG4)-related disease has been defined as an autoimmune systemic disease characterized by the lymphoplasmacytic infiltration of affected tissues leading to fibrosis and obliterative phlebitis along with elevated serum IgG4 levels. Prior to this unifying hypothesis, a plethora of clinical manifestations were considered as separate entities despite the similar laboratory profile. The pathology can be observed in virtually all organs and may thus be a challenging diagnosis, especially when the adequate clinical suspicion is not present or when obtaining a tissue biopsy is not feasible. Nonetheless, the most frequently involved organs are the pancreas and exocrine glands but these may be spared. Immunosuppressants lead to a prompt clinical response in virtually all cases and prevent histological sequelae and, as a consequence, an early differential diagnosis from other conditions, particularly infections and cancer, as well as an early treatment should be pursued. We describe herein two cases in which atypical disease manifestations were observed, i.e., one with recurrent neck lymph node enlargement and proptosis, and one with jaundice. Our understanding of the pathogenesis of IgG4-related disease is largely incomplete but data support a significant role for Th2 cytokines with the contribution of innate immunity factors such as Toll-like receptors, macrophages and basophils. Further, macrophages activated by IL4 overexpress B cell activating factors and contribute to chronic inflammation and the development of fibrosis. We cannot rule out the possibility that the largely variable disease phenotypes reflect different pathogenetic mechanisms and the tissue microenvironment may then contribute to the organ involvement.

  17. Secure Military Communications on 3G, 4G and WiMAX

    Science.gov (United States)

    2013-09-01

    securely leverage three cellular technologies, 3G , 4G/LTE and WiMAX, through an analysis of their security features. The security architectures of these...This chapter provides information regarding the security architecture of Third Generation ( 3G ), Long Term Evolution (LTE), and WiMAX cellular systems...Figure 1. UMTS security architecture , from [3] • Network access security (class 1): enables the user to securely access a 3G network and provide

  18. IgG4-related Sclerosing Mesenteritis in a 7-year-old Saudi Girl

    OpenAIRE

    Hasosah, Mohammed Y.; Satti, Mohamed B.; Yousef, Yasmin A.; Alzahrani, Daifullah M.; Almutairi, Sajdi A.; Ashraf F Alsahafi; Sukkar, Ghassan A.; Alzaben, Abdullah A.

    2014-01-01

    Sclerosing mesenteritis (SM) is a rare, benign inflammatory disorder of unknown etiology, affecting the membranes of the digestive tract that involves lymphoplasmacytic inflammation, fat necrosis, and fibrosis of the mesentery. We report a child patient with a history of recurrent abdominal pain and fever who was found to have an intra-abdominal mass suspicious for malignancy. A tissue biopsy revealed the diagnosis of SM associated with IgG4-related systemic disease. The patient is currently ...

  19. [IgG4- and MPO-ANCA-associated hypertrophic pachymeningitis].

    Science.gov (United States)

    Aragonès, Josep M; Arias-Rivero, Montserrat; García-Barrionuevo, Joan M; Lucchetti, Gianni

    2015-11-16

    Introduccion. La paquimeningitis hipertrofica idiopatica es una enfermedad fibroinflamatoria de la duramadre. Su diagnostico requiere la exclusion de enfermedades infecciosas, tumorales y otras enfermedades inflamatorias. En los ultimos años se han descrito nuevas entidades que pueden presentarse con paquimeningitis hipertrofica: la enfermedad relacionada con IgG4 y la paquimeningitis MPO-ANCA+ como forma de vasculitis limitada al sistema nervioso central. Caso clinico. Varon de 64 años con cefalea y cervicalgia de predominio nocturno y clinica de compresion medular. Tras el diagnostico de paquimeningitis hipertrofica craneocervical facilitado por el estudio de resonancia magnetica, se realizo un estudio etiologico. Se descartaron enfermedades infecciosas y tumorales. La clinica no mostraba afectacion sistemica y en la analitica presentaba IgG4 elevada y MPO-ANCA+. Tras tratamiento con corticoides presento una rapida mejoria de la clinica. Conclusiones. La enfermedad relacionada con IgG4 y la vasculitis asociada a MPO-ANCA limitada al sistema nervioso central pueden representar un alto porcentaje de las paquimeningitis hipertroficas que se consideraban idiopaticas, y su diagnostico requiere biopsia y estudio histologico.

  20. Enzyme-Assisted Extraction of Oil from Wet Microalgae Scenedesmus sp. G4

    Directory of Open Access Journals (Sweden)

    Shuhao Huo

    2015-08-01

    Full Text Available The enzyme-assisted aqueous extraction of oil from wet microalgae was employed to avoid the energy consumption of a dewatering process. In this paper, oil-rich microalgae Scenedesmus sp. G4 was hydrolyzed by enzyme mixtures for oil extraction. The results showed that the algae concentration had the greatest influence on yield of extracted oil, and the temperature and the ratio of enzyme mixtures affected the results as follows: the maximum yield of oil extracted from Scenedesmus sp. G4 reached 86.1% under optimal conditions with an algae concentration at 2.5 g/L, temperature 30/50 °C, pH 3.5/4.5 and a cellulase:pectinase:hemicellulase ratio of 1:1:1 or 1:2:1 (w/w/w. The results also indicated that the enzyme mixtures had a significant impact on the integrity of microalgae cells and the crystallization index increased from 30.7% to around 36.0% after enzymatic hydrolysis treatment. The cell wall of Scenedesmus sp. G4 has a high content of cellulose Iα and low crystallization, which is beneficial to the oil extraction by the enzyme-assisted hydrolysis method.

  1. [Central nervous system in IgG4-related disease: case report and literature review].

    Science.gov (United States)

    Vanegas-Garcia, A L; Calle-Lopez, Y; Zapata, C H; Alvarez-Espinal, D M; Saavedra-Gonzalez, Y A; Arango-Viana, J C

    2016-08-01

    Introduccion. La enfermedad relacionada con IgG4 es una entidad clinica multisistemica recientemente descrita y que se presenta con diferentes manifestaciones clinicas. Los organos que estan afectados con mayor frecuencia son el pancreas, la via biliar y las glandulas salivales, y es menos frecuente la afeccion del sistema nervioso central. Caso clinico. Mujer de 33 años con alteraciones cognitivas, alucinaciones, cefalea, sindrome convulsivo, sinusitis maxilar con afeccion osea y evidencia de paquimeningitis y panhipopituitarismo, con biopsia meningea que confirmo una enfermedad relacionada con IgG4, tras haberse descartado causas secundarias. Se inicio tratamiento con glucocorticoides y azatioprina, sin recaidas despues de 12 meses de seguimiento. Conclusiones. Se debe considerar el diagnostico de enfermedad relacionada con IgG4 en casos de paquimeningitis hipertrofica e hipofisitis, incluso sin que se acompañen de otras manifestaciones sistemicas, siempre que se hayan descartado otras causas mas frecuentes. El tratamiento de eleccion son los glucocorticoides, y puede ser necesario añadir otro inmunosupresor como ahorrador de esteroides y para evitar las recaidas. Se necesitan estudios prospectivos para evaluar las diferentes manifestaciones clinicas y paraclinicas y establecer los resultados del tratamiento a largo plazo.

  2. Solely lung-involved IgG4-related disease : a case report and review of the literature.

    Science.gov (United States)

    Zhang, Xiao-Qin; Chen, Guo-Ping; Wu, Sheng-Chang; Yu, Sa; Wang, Hong; Chen, Xuan-Yi; Ren, Zhuo-Chao

    2016-12-23

    By analyzing the clinical data of 1 case of IgG4-related lung disease(IgG4-RLD) and the review of literature, the author investigated the clinical characteristics of IgG4-RLD. IgG4-RLD is a rare disease characterized by significant elevation of serum IgG4 and infiltration of a large number of IgG4+ plasma cells. The clinical manifestations of the disease were nonspecific, and the imaging features were mixed with several types. The disease can only be involved in the lung, but also multiple organ involvement. Solely lung-involved IgG4-RD is not only extremely rare but also easily misdiagnosed as tuberculosis, lung cancer, lymphoma and other common pulmonary diseases. Histopathological examination is the key to the diagnosis of the disease. Corticosteroids are the first choice of treatment, and the overall prognosis is good.

  3. A case of tubulointerstitial nephritis in IgG4-related systemic disease with markedly enlarged kidneys.

    Science.gov (United States)

    Mise, Naobumi; Tomizawa, Yutaka; Fujii, Akiko; Yamaguchi, Yutaka; Sugimoto, Tokuichiro

    2009-06-01

    IgG4-related systemic disease, including autoimmune pancreatitis, is a multi-organ disorder characterized by elevated serum immunoglobulin G4 (IgG4) concentration and IgG4-positive plasma cell infiltration. We report the case of a 67-year-old man with IgG4-related tubulointerstitial nephritis, presenting with markedly enlarged kidneys and renal dysfunction. The serum IgG4 level was elevated with 4200 mg/dl and pathological examination revealed patchy, clearly fringed areas of IgG4-positive plasma cell infiltration and advanced fibrosis in the renal parenchyma, perirenal tissue and lymph nodes. With oral prednisolone at a dose of 60 mg daily, a contraction of the kidneys and an improvement of renal function were observed. No recurrence of the disease was observed during the reduction of prednisolone to 2 mg daily over 4 years.

  4. Recent advances in the concept and diagnosis of autoimmune pancreatitis and IgG4-related disease.

    Science.gov (United States)

    Okazaki, Kazuichi; Uchida, Kazushige; Koyabu, Masanori; Miyoshi, Hideaki; Takaoka, Makoto

    2011-03-01

    Recent studies have suggested the existence of two subtypes of autoimmune pancreatitis (AIP): type 1 AIP, related to IgG4 (lymphoplasmacytic sclerosing pancreatitis); and type 2 AIP, related to a granulocytic epithelial lesion (idiopathic duct-centric chronic pancreatitis). Compared with type 2 AIP, the clinicopathological features of type 1 AIP, with increased serum IgG4/IgE levels, abundant infiltration of IgG4 + plasmacytes and lymphocytes, autoantibodies, and steroid responsiveness, are more suggestive of abnormal immunity such as allergy or autoimmunity. Moreover, patients with type 1 AIP often have extrapancreatic lesions, such as sclerosing cholangitis, sclerosing sialadenitis, or retroperitoneal fibrosis, showing pathological features similar to those of the pancreatic lesions. Based on these findings, an international concept of and diagnostic criteria for AIP have been proposed recently. Of interest, many synonyms have been proposed for the conditions of AIP and extrapancreatic lesions associated with IgG4, such as "multifocal idiopathic fibrosclerosis," "IgG4-related autoimmune disease," "IgG4-related sclerosing disease," "systemic IgG4-related plasmacytic syndrome (SIPS)," and "IgG4-related multiorgan lymphoproliferative syndrome," all of which may refer to the same conditions. Therefore, the Japanese Research Committee for "Systemic IgG4-Related Sclerosing Disease" proposed a disease concept and clinical diagnostic criteria based on the concept of multifocal fibrosclerosing disease, in 2009, in which the term "IgG4-related disease" was agreed upon as a minimal consensus to cover these conditions. Although the significance of IgG4 in the development of "IgG4-related disease" remains unclear, we have proposed a hypothesis for the development of type 1 AIP, one of the IgG4-related diseases. The concept and diagnostic criteria of "IgG4-related disease" will be changed in accordance with future studies.

  5. Antineutrophil cytoplasmic antibody-associated vasculitides and IgG4-related disease: A new overlap syndrome.

    Science.gov (United States)

    Danlos, François-Xavier; Rossi, Giovanni Maria; Blockmans, Daniel; Emmi, Giacomo; Kronbichler, Andreas; Durupt, Stéphane; Maynard, Claire; Luca, Luminita; Garrouste, Cyril; Lioger, Bertrand; Mourot-Cottet, Rachel; Dhote, Robin; Arlet, Jean-Benoit; Hanslik, Thomas; Rouvier, Philippe; Ebbo, Mikael; Puéchal, Xavier; Nochy, Dominique; Carlotti, Agnès; Mouthon, Luc; Guillevin, Loïc; Vaglio, Augusto; Terrier, Benjamin

    2017-10-01

    Atypical manifestations have been described in patients with ANCA-associated vasculitides (AAV), such as pachymeningitis, orbital mass or chronic periaortitis. Because these manifestations have been associated to the spectrum of IgG4-related disease (IgG4-RD), we hypothesized that both diseases could overlap. We conducted a European retrospective multicenter observational study including patients fulfilling ACR and Chapel Hill criteria for AAV and IgG4-RD Comprehensive Diagnostic Criteria. Eighteen patients were included (median age 55.5years, 13 men). AAV and IgG4-RD were diagnosed concomitantly in 13/18 (72%) patients; AAV preceded IgG4-RD in 3/18 (17%) while IgG4-RD preceded AAV in 2/18 (11%). AAV diagnoses included granulomatosis with polyangiitis in 14 (78%), microscopic polyangiitis in 3 (17%), and eosinophilic granulomatosis with polyangiitis in one case. IgG4-RD diagnosis included definite IgG4-RD in 5 (28%) cases, probable IgG4-RD in 5 (28%) and possible IgG4-RD in 8 (44%). IgG4-RD manifestations were chronic periaortitis in 9/18 (50%) patients, orbital mass and tubulointerstitial nephritis in 4 (22%) cases, prevertebral fibrosis in 3 (17%), pachymeningitis and autoimmune pancreatitis in 2 (11%) cases. Patients required median number of 2 (range 0-4) lines of immunosuppressants in combination with glucocorticoids. During the follow-up (median 49,8months, range 17,25-108months), AAV manifestations relapsed in 10/18 (56%) cases and IgG4-RD lesions in 5/18 (28%). When used, mainly for relapses, rituximab showed response in all cases. AAV and IgG4-RD may overlap. Clinicians should consider that atypical manifestations during AAV could be related to IgG4-RD rather than to refractory granulomatous or vasculitic lesions. Copyright © 2017 Elsevier B.V. All rights reserved.

  6. The S228P mutation prevents in vivo and in vitro IgG4 Fab-arm exchange as demonstrated using a combination of novel quantitative immunoassays and physiological matrix preparation.

    Science.gov (United States)

    Silva, John-Paul; Vetterlein, Olivia; Jose, Joby; Peters, Shirley; Kirby, Hishani

    2015-02-27

    Human immunoglobulin G isotype 4 (IgG4) antibodies (Abs) are potential candidates for immunotherapy when reduced effector functions are desirable. IgG4 Abs are dynamic molecules able to undergo a process known as Fab arm exchange (FAE). This results in functionally monovalent, bispecific antibodies (bsAbs) with unknown specificity and hence, potentially, reduced therapeutic efficacy. IgG4 FAE is suggested to be an important biological mechanism that provides the basis for the anti-inflammatory activity attributed to IgG4 Abs. To date, the mechanism of FAE is not entirely understood and studies measuring FAE in ex vivo matrices have been hampered by the presence and abundance of endogenous IgG4 wild-type (WT) Abs. Using representative humanized WT IgG4 monoclonal Abs, namely, anti-IL-6 and anti-TNF, and a core-hinge stabilized serine 228 to proline (S228P) anti-IL-6 IgG4 mutant, it is demonstrated for the first time how anti-IgG4 affinity chromatography can be used to prepare physiologically relevant matrices for assessing and quantifying FAE. A novel method for quantifying FAE using a single MSD immunoassay is also reported and confirms previous findings that, dependent on the redox conditions, the S228P mutation can prevent IgG4 FAE to undetectable levels both in vitro and in vivo. Together, the findings and novel methodologies will allow researchers to monitor and quantify FAE of their own IgG4 molecules in physiologically relevant matrices.

  7. Current clinical research of immunoglobulin G4-related orbital disease%免疫球蛋白G4相关性眼眶病的临床研究进展

    Institute of Scientific and Technical Information of China (English)

    王洋; 周慧芳; 范先群

    2016-01-01

    lmmunoglobulin G4 - related disease ( lgG4 - related disease ) has received lots of attention in medical community as a recently recognized fibro-inflammatory condition. lt is characterized by infiltration of lgG4 -immunopositive plasmacytes and concentration of elevated serum lgG4. lgG4-related disease shows organ enlargement or nodular/hyperplastic lesions in various organs including the pancreas, hepatobiliary tract and orbit, which is called lgG4-related orbital disease. The diagnostic criteria for lgG4-related disease and lgG4-related orbital disease has recently been established, which is based on clinical, imaging and histopathologic features of the orbital lesions. Besides, attention should be drawn to the differentiation from other diseases. The treatment is empirical including corticosteroids, immunosuppressive drugs, radiotherapy, and rituximab. This article reviews clinical progression of lgG4-related orbital disease.%近年来,免疫球蛋白G4( IgG4)相关性疾病作为一种新的自身免疫性疾病,受到国内外医学界的的广泛关注。IgG4相关性疾病以IgG4+浆细胞浸润和血清IgG4浓度升高为特征,同时伴有因全身多器官的纤维化而发生肿大、结节性或增生性病变的自身免疫性疾病。当累及眼眶时称为IgG4相关性眼眶病。目前提出了IgG4相关性眼眶病的相对完善的诊断标准,然而治疗方面还没有公认有效的手段,目前首选激素治疗。本文就IgG4相关性眼眶病的临床特征、诊断和治疗的研究进展进行综述。

  8. IgG4相关硬化性胆管炎的研究进展%Recent advances in research of immunoglobulin G4-related sclerosing cholangitis

    Institute of Scientific and Technical Information of China (English)

    黄颖秋

    2012-01-01

    免疫球蛋白G4相关硬化性胆管炎(immunoglobulin G4-related sclerosing cholangitis,IgG4-SC)是一种新近认识的以血清IgG4升高、慢性进行性阻塞性黄疸、弥漫性或局限性IgG4阳性浆细胞和淋巴细胞组织浸润、纤维化及闭塞性静脉炎为特征的慢性炎症性疾病,常并发自身免疫性胰腺炎(autoimmune pancreatitis,AIP),其临床、生化及影像学特征与原发性硬化性胆管炎(primary sclerosing cholangitis,PSC)或胆管癌(cholangiocarcinoma,CC)相似.类固醇激素是IgG4-SC的主要治疗手段,而肝移植是PSC唯一的有效治疗方法,CC则需外科手术治疗.因此,IgG4-SC与PSC或CC间的准确鉴别是目前面临的一个十分重要的课题.本文详尽地阐述了免疫球蛋白G4(immunoglobulin G4,IgG4)的特征和功能,IgG4-SC的诊断和治疗,IgG4-SC与AIP、PSC及CC之间关系等研究进展,为IgG4-SC的精确诊断和治疗提供了新的思路.%Immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) is a recently defined disease entity characterized by elevated serum IgG4, chronic progressive obstructive jaundice, and diffuse or mass-forming inflammatory reaction rich in IgG4-positive plasma cells and lymphocytes associated with fibrosclerosis and obliterative phlebitis, which shares a number of clinical, biochemical, and radiological features with primary sclerosing cholangitis (PSC) or cholangiocarcino-ma (CC). IgG4-SC is commonly associated with autoimmune pancreatitis (AIP). Steroid therapy comprises the mainstay of treatment for IgG4-SC patients. However, liver transplantation is the only useful treatment for PSC patients, and CC patients require surgical therapy. Therefore, the accurate discrimination between IgG4-SC and PSC or CC is a very important issue. In this article, we will review the features and role of immunoglobulin G4 (IgG4), the diagnosis and therapy of IgG4-SC, and the relations between IgG4-SC and AIP, PSC or CC.

  9. Hypermethylation of MST1 in IgG4-related autoimmune pancreatitis and rheumatoid arthritis

    Energy Technology Data Exchange (ETDEWEB)

    Fukuhara, Takataro; Tomiyama, Takashi [Division of Gastroenterology and Hepatology, The Third Department of Internal Medicine, JST CREST, Kansai Medical University, 2-5-1 Shin-machi, Hirakata, Osaka 573-1010 (Japan); Yasuda, Kaneki [Department of Urology and Andrology, Kansai Medical University, 2-5-1 Shin-machi, Hirakata, Osaka 573-1010 (Japan); Ueda, Yoshihiro [Department of Molecular Genetics, Institute of Biomedical Science, and JST CREST, Kansai Medical University, 2-5-1 Shin-machi, Hirakata, Osaka 573-1010 (Japan); Ozaki, Yoshio; Son, Yonsu; Nomura, Shosaku [Department of the First Department of Internal Medicine, Kansai Medical University, 2-5-1 Shin-machi, Hirakata, Osaka 573-1010 (Japan); Uchida, Kazushige; Okazaki, Kazuichi [Division of Gastroenterology and Hepatology, The Third Department of Internal Medicine, JST CREST, Kansai Medical University, 2-5-1 Shin-machi, Hirakata, Osaka 573-1010 (Japan); Kinashi, Tatsuo, E-mail: kinashi@takii.kmu.ac.jp [Department of Molecular Genetics, Institute of Biomedical Science, and JST CREST, Kansai Medical University, 2-5-1 Shin-machi, Hirakata, Osaka 573-1010 (Japan)

    2015-08-07

    The serine/threonine kinase Mst1 plays important roles in the control of immune cell trafficking, proliferation, and differentiation. Previously, we reported that Mst1 was required for thymocyte selection and regulatory T-cell functions, thereby the prevention of autoimmunity in mice. In humans, MST1 null mutations cause T-cell immunodeficiency and hypergammaglobulinemia with autoantibody production. RASSF5C(RAPL) is an activator of MST1 and it is frequently methylated in some tumors. Herein, we investigated methylation of the promoter regions of MST1 and RASSF5C(RAPL) in leukocytes from patients with IgG4-related autoimmune pancreatitis (AIP) and rheumatoid arthritis (RA). Increased number of CpG methylation in the 5′ region of MST1 was detected in AIP patients with extrapancreatic lesions, whereas AIP patients without extrapancreatic lesions were similar to controls. In RA patients, we detected a slight increased CpG methylation in MST1, although the overall number of methylation sites was lower than that of AIP patients with extrapancreatic lesions. There were no significant changes of the methylation levels of the CpG islands in the 5′ region of RASSF5C(RAPL) in leukocytes from AIP and RA patients. Consistently, we found a significantly down-regulated expression of MST1 in regulatory T cells of AIP patients. Our results suggest that the decreased expression of MST1 in regulatory T cells due to hypermethylation of the promoter contributes to the pathogenesis of IgG4-related AIP. - Highlights: • Mst1 controls immune cells trafficking, cell proliferation and differentiation. • Autoimmune pancreatitis (AIP) is an idiopathic pancreatitis affecting multiple organs. • Decreased MST1 expression and increased CpG methylation of promoter of MST1 in AIP. • Slight increased CpG methylation of MST1 in rheumatoid arthritis patients. • MST1 contributes pathogenesis of IgG4-related AIP.

  10. IgG4-Related Disease Is Not Associated with Antibody to the Phospholipase A2 Receptor

    Directory of Open Access Journals (Sweden)

    Arezou Khosroshahi

    2012-01-01

    Full Text Available Patients with IgG4-related disease (IgG4-RD share histopathological characteristics that are similar across affected organs. The finding of infiltration with IgG4+ plasma cells in the proper clinical and histopathological contexts connects a large number of clinical entities that were viewed previously as separate conditions. The renal involvement in IgG4-RD is usually characterized by tubulointerstitial nephritis, but membranous nephropathy has also been reported to be one of the renal complications of IgG4-RD. The recent discovery that a high proportion of patients with idiopathic membranous nephropathy (IMN have IgG4 autoantibodies to the M-type phospholipase A2 receptor (PLA2R in the circulation and glomerular immune deposits, together with the profound IgG4 hypergammaglobulinemia and occasional reports of membranous nephropathy in IgG4-RD, raised the question of a common antigen. To assess the presence of anti-PLA2R antibody in patients with IgG4-RD, we screened sera from 28 IgG4-RD patients by immunoblot. None of the patients in this cohort had detectable circulating anti-PLA2R antibodies. This study suggests that despite some clinical and serological overlaps between IgG4-RD and IMN,anti-PLA2R antibodies do not play a role in the pathogenesis of IgG4-RD. Additional studies of IgG4-RD with evidence of membranous nephropathy are important to exclude any definite relationship.

  11. IgG4-related disease: a novel lymphoproliferative disorder discovered and established in Japan in the 21st century.

    Science.gov (United States)

    Masaki, Yasufumi; Kurose, Nozomu; Umehara, Hisanori

    2011-01-01

    IgG4-related disease is a novel lymphoproliferative disorder that shows hyper-IgG4-γ-globulinemia and IgG4-producing plasma cell expansion in affected organs with fibrotic or sclerotic changes. Patients show systemic inflammatory conditions and various symptoms depending on the affected organ. Since the first report of patients with elevated serum IgG4 in sclerosing pancreatitis in 2001, various systemic disorders described by many names have been reported. Despite similarities in the organs involved in IgG4-related Mikulicz's disease and Sjögren's syndrome, there are marked clinical and pathological differences between these conditions. Most patients diagnosed with autoimmune pancreatitis in Japan have IgG4-related pancreatitis [Type 1 autoimmune pancreatitis (AIP), lymphoplasmacytic sclerosing pancreatitis (LPSP)], a disease distinct from some of the western type [Type 2 AIP, idiopathic duct-centric chronic pancreatitis (IDCP), autoimmune pancreatitis with granulocytic epithelial lesions (GEL)]. Diagnosis of IgG4-related disease is characterized by both elevated serum IgG4 (>135 mg/dL) and histopathological features including lymphocyte and IgG4(+) plasma cell infiltration (IgG4(+) plasma cells/IgG(+) plasma cells>40%). Differential diagnosis from other distinct disorders, such as sarcoidosis, Castleman's disease, Wegener's granulomatosis, lymphoma, cancer, and other existing conditions associated with high serum IgG4 level or abundant IgG4-bearing plasma cells in tissues is necessary. We have begun a clinical prospective study to establish a treatment strategy (Phase II prospective treatment study for IgG4-multiorgan lymphoproliferative syndrome: UMIN R000002311).

  12. IgG4-Related Tubulointerstitial Nephritis Pattern in 18F-FDG PET/CT.

    Science.gov (United States)

    Bélissant, Ophélie; Guernou, Mohamed; Rouvier, Philippe; Compain, Caroline; Bonardel, Gérald

    2015-10-01

    A 17-year-old adolescent girl was admitted with chronic arthralgia, Raynaud phenomenon, pericarditis, and evidences of chronic diffuse inflammation. F-FDG PET/CT scan was performed to search systemic vasculitis and showed diffuse moderate uptake in the kidneys. We suggested the existence of a nephritis, but the ultrasonography result was normal, and no treatment was introduced. Another F-FDG PET/CT scan was performed 7 months later to explore abdominal pain. It showed again diffuse intense uptake in both kidneys. A proteinuria was highlighted, and renal biopsy allowed to diagnose IgG4-related disease.

  13. Antigen recognition by IgG4 antibodies in human trichinellosis

    Directory of Open Access Journals (Sweden)

    Pinelli E.

    2001-06-01

    Full Text Available The antibody isotype response to Trichinella spiralis excretory/secretory (ES products of muscle larva was examined using sera from patients with confirmed trichinellosis. Using Western blots we identify components of the ES antigen that are recognized by IgM and IgG antibodies. A 45 kDa component was strongly recognized by different antibody classes and subclasses. We observed a 45 kDa-specific lgG4 response that was detected exclusively using sera of patients with trichinellosis and not of patients with echinococcosis, filariasis, cysticercosis, ascariasis, strongyloidiasis or toxocariasis. These results are relevant for the diagnosis of human trichinellosis.

  14. Rapid Industrial Prototyping and SoC Design of 3G/4G Wireless Systems Using an HLS Methodology

    Directory of Open Access Journals (Sweden)

    Cavallaro JosephR

    2006-01-01

    Full Text Available Many very-high-complexity signal processing algorithms are required in future wireless systems, giving tremendous challenges to real-time implementations. In this paper, we present our industrial rapid prototyping experiences on 3G/4G wireless systems using advanced signal processing algorithms in MIMO-CDMA and MIMO-OFDM systems. Core system design issues are studied and advanced receiver algorithms suitable for implementation are proposed for synchronization, MIMO equalization, and detection. We then present VLSI-oriented complexity reduction schemes and demonstrate how to interact these high-complexity algorithms with an HLS-based methodology for extensive design space exploration. This is achieved by abstracting the main effort from hardware iterations to the algorithmic C/C++ fixed-point design. We also analyze the advantages and limitations of the methodology. Our industrial design experience demonstrates that it is possible to enable an extensive architectural analysis in a short-time frame using HLS methodology, which significantly shortens the time to market for wireless systems.

  15. Rapid Industrial Prototyping and SoC Design of 3G/4G Wireless Systems Using an HLS Methodology

    Directory of Open Access Journals (Sweden)

    Andres Takach

    2006-07-01

    Full Text Available Many very-high-complexity signal processing algorithms are required in future wireless systems, giving tremendous challenges to real-time implementations. In this paper, we present our industrial rapid prototyping experiences on 3G/4G wireless systems using advanced signal processing algorithms in MIMO-CDMA and MIMO-OFDM systems. Core system design issues are studied and advanced receiver algorithms suitable for implementation are proposed for synchronization, MIMO equalization, and detection. We then present VLSI-oriented complexity reduction schemes and demonstrate how to interact these high-complexity algorithms with an HLS-based methodology for extensive design space exploration. This is achieved by abstracting the main effort from hardware iterations to the algorithmic C/C++ fixed-point design. We also analyze the advantages and limitations of the methodology. Our industrial design experience demonstrates that it is possible to enable an extensive architectural analysis in a short-time frame using HLS methodology, which significantly shortens the time to market for wireless systems.

  16. The role of IgG4 (+) plasma cells in the association of Hashimoto's thyroiditis with papillary carcinoma.

    Science.gov (United States)

    Taşli, Funda; Ozkök, Güliz; Argon, Asuman; Ersöz, Didem; Yağci, Ayşe; Uslu, Adam; Erkan, Nazif; Salman, Tarik; Vardar, Enver

    2014-12-01

    Hashimoto's thyroiditis (HT) is considered to be a risk factor for the formation of papillary carcinoma. The association of IgG4-related sclerosing disease with tumor is reported to be as sporadic cases in many organs. In this study, it was intended to re-classify the HT diagnosed cases on the basis of the existence of IgG4 (+) plasma cells; to investigate the clinicopathologic and histopathologic features of the both groups; and in addition, to evaluate the papillary carcinoma prevalence in IgG4 (+) and IgG4 (-) HT cases as well as the prognostic parameters between these groups. Totally 59 cases between the years 2008-2013, 29 of which contain Hashimoto thyroiditis diagnosis in total thyroidectomy materials, and 30 of which contain the diagnosis of HT+papillary carcinoma, were included in the study. The materials were immunohistochemically applied IgG and IgG4; and the cases were classified in two groups as IgG4-positive HT and IgG4-negative HT containing cases, on the basis of IgG4/IgG rate. All histopathologic and clinicopathologic parameters between these two groups, as well as their association with papillary carcinoma were investigated. Thirty eight (64.4%) of total 59 cases were NonIgG4 thyroiditis, and 21 (35.5%) were IgG4 thyroiditis. Tumors were detected in 14 (36.8%) of the NonIgG4 thyroiditis cases, and in 16 (76.1%) of the IgG4 thyroiditis cases. The association of IgG4 thyroiditis with tumor is statistically significant (p thyroiditis cases. Perithyroidal extension was detected in six of the cases with tumor, and five of the six cases were IgG4 thyroiditis cases. The association of IgG4 (+) HT cases with increased papillary carcinoma prevalence is suggestive of that IgG4 (+) plasma cells can play a role in carcinogenesis in papillary carcinomas developed in HTs, without a chronic sclerosing ground. In addition, although the number of cases is limited, the high-association of IgG4 (+) plasma cells with adverse prognostic parameters such as

  17. [Hashimoto's thyroiditis(chronic thyroiditis), IgG4-related thyroiditis].

    Science.gov (United States)

    Itoh, Mitsuyasu

    2012-11-01

    Hashimoto's thyroiditis emerges in patients who have genetic preponderance such as SNPs of CTLA-4 and risk factors such as excess intake of iodine, pregnancy or postpartum period, and smoking. Such risk factors also affect the entire clinical course. One of the major outcomes in Hashimoto's thyroiditis appears to be increased in cardio-vascular risks through subclinical hypothyroidism and concomitant metabolic syndrome, but in most cases, treatment with L-T4 has little effects on cardio-vascular benefit or quality of life. The pregnant women also have risks for obstetric complications and postpartum thyroid dysfunction. The women who have anti-TPO antibodies, type 1 diabetes, or previous history of post-partum thyroid dysfunction are recommended to be measured their TSH. It is noteworthy that Hashimoto's thyroiditis is sometimes complicated with encephalopathy, papillary carcinoma, or IgG4-related thyroiditis. IgG4-related thyroiditis is partly similar but partly discerned from a variant of Hashimoto's thyroiditis. The pathogenetic roles of this variant on autoimmune-based thyroiditis remain unclear.

  18. Renal cell carcinoma mimicking IgG4-related pseudotumor in autoimmune pancreatitis.

    Science.gov (United States)

    Khan, Muhammad Ali; Kamal, Sehrish; Ahmad, Usman; Chowdhury, Mohammed Andaleeb; Nawras, Ali

    2014-09-28

    Autoimmune pancreatitis is classified into two distinct clinical profiles. Type 1 autoimmune pancreatitis (AIP) is considered to be a manifestation of a novel clinicopathological entity called IgG4 related sclerosing disease, diagnosed using the Mayo Clinic HISORt criteria. Extra-pancreatic manifestations can include involvement of bile ducts, salivary gland, lung nodules, thyroiditis, tubulointerstitial nephritis, renal masses, and retroperitoneal fibrosis. Type 2 autoimmune pancreatitis on the other hand is confirmed by histologically seen duct centric pancreatitis without elevation of IgG4 or involvement of other organs. In type 1 autoimmune pancreatitis, extrapancreatic manifestations like bile duct strictures, tubulointerstitial nephritis, renal nodules, retroperitoneal fibrosis respond to steroid therapy. We present a case of type 1 autoimmune pancreatitis in which the renal mass did not respond to steroid therapy and was later on found to be renal cell carcinoma. To the best of our knowledge this is only the third reported case of autoimmune pancreatitis in which the patient had renal cell carcinoma. Our case highlights the importance of close follow up of lesions that do not respond to steroid treatment which in this case proved to be renal cell cancer.

  19. Immunglobulin G4-relateret sygdom er en sjælden differentialdiagnose til maligne og autoimmune sygdomme

    DEFF Research Database (Denmark)

    Storgaard, Anders; Detlefsen, Sönke

    2015-01-01

    Immunoglobulin G4-related disease (IgG4-RD) is an inflammatory and fibrotic disease with the potential to produce diffuse enlargement, massforming lesions or stenoses in a wide range of organs. Elevation of serum IgG4 concentration and high levels of IgG4-positive cells in the inflamed tissue...... are common denominators. Type 1 autoimmune pancreatitis is one of the main manifestations, and its recognition preceded the definition of IgG4-RD as a novel clinical entity. The aetiology, pathophysiology, epidemiology and clinical long-term outcome of IgG4-RD are not fully elucidated. Steroids are effective...... in most patients, sometimes combined with other antiinflammatory drugs....

  20. Hyalinizing cholecystitis with features of immunoglobulin G4-related disease-coincidence or an unrecognized association? A case report.

    Science.gov (United States)

    Gupta, Rajib K; Patton, Kurt T

    2015-04-01

    Hyalinizing cholecystitis (HC) is a recently described rare subtype of chronic cholecystitis characterized by dense, paucicellular collagenous transmural fibrosis, which usually replaces the mucosa and muscularis propria. Immunoglobulin (Ig)G4-associated cholecystitis is also a newly described cholecystitis variant characterized by transmural or extramural lymphoplasmacytic inflammation, lymphoid follicles, storiform fibrosis, phlebitis, and increased tissue IgG4-positive plasma cells. We describe a case of cholecystitis in an elderly white man who harbored features of both HC and IgG4-associated cholecystitis. In retrospect, the patient also had a significantly elevated serum IgG4 level. To the best of our knowledge, an association between HC and IgG4-related disease has not been previously described in the literature. Although not entirely conclusive, our observations raise the possibility that some cases of HC represent the end stage of IgG4-related disease.

  1. An immunoglobulin G-4 related sclerosing disease of the small bowel: CT and small bowel series findings

    Energy Technology Data Exchange (ETDEWEB)

    Ko, Young Hwan; Hwang, Dae Hyun; Min, Seon Jeong [Dept. of Radiology, Hallym University Dongtan Sacred Heart Hospital, Hwaseong (Korea, Republic of); Woo, Ji Young; Kim, Jeong Won; Hong, Hye Sook; Yang, Ik; Lee, Yul [Hallym University Kangnam Sacred Heart Hospital, Seoul (Korea, Republic of)

    2013-10-15

    Immunoglobulin G4 (IgG4)-related sclerosing disease is rare and is known to involve various organs. We present a case of histologically proven IgG4-related sclerosing disease of the small bowel with imaging findings on computed tomography (CT) and small bowel series. CT showed irregular wall thickening, loss of mural stratification and aneurysmal dilatation of the distal ileum. Small bowel series showed aneurysmal dilatations, interloop adhesion with traction and abrupt angulation.

  2. Advances in the diagnosis and treatment of IgG4-related sclerosing cholangitis:a review

    Institute of Scientific and Technical Information of China (English)

    Shunda Du; Gang Liu; Yilei Mao

    2015-01-01

    Immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) is an IgG4-related disease characterized by bile duct fibroinflammatory wal-thickening and stenosis, resulting in obstruction jaundice, weight loss. Dif-ferent regions of the bile duct can be involved, with the distal region being the most common. IgG4-SC can also have other organ involvement, such as the pancreas, urinary tract, salivary glands and lacrimal glands. In clinical practice, the manifestation of IgG4-SC is very similar to cholangiocarcinoma (CC) and primary sclerosing cholangitis (PSC), as wel as pancreatic malignancies, while the treatment and prognosis are totaly diferent. Japanese researchers ever established the clinical diagnostic criteria in 2012: (1) charac-teristic biliary imaging findings; (2) elevated serum IgG4 concentrations; (3) the coexistence of IgG4-related diseases except those of the biliary tract; and (4) characteristic histopathological features. According to our observations, IgG4-SC can be distinguished from CC with 100% specificity only at a cutof of six times the upper normal limit. Imaging findings have low specificity for diagnosis, with the exception of intraductal ultrasonography, which can reflect the lesion with relatively high specificity. IgG4 plasma cel infiltration can be found in bile duct biopsy tissue, although this procedure is dificult. According to recent studies, the treatment of IgG4-SC relies mainly on corticosteroids. Folowing steroid treatment, most IgG4-SC patients can recover and their symptoms are resolved although a few patients relapse after steroid withdrawal. Maintenance of steroid therapy or alternative drugs is necessary in such cases. There is, however, no strong evidence for malignant transformation in IgG4-SC.

  3. Analysis of clinicopathological characters for IgG4-related disease%IgG4相关性疾病临床病理学特征分析

    Institute of Scientific and Technical Information of China (English)

    钟卫祥; 孙柯; 滕晓东

    2015-01-01

    目的:探讨IgG4相关性疾病的病理学形态、免疫表型特征。方法观察12例IgG4相关性疾病的镜下特点,结合免疫组化EnVision两步法染色检测IgG、IgG4、CD138、CD34的表达,分析IgG4相关性疾病的病理学形态特征。结果 IgG4相关性疾病主要表现为组织弥漫性纤维化;伴大量淋巴细胞、浆细胞浸润,围绕血管神经分布;闭塞性静脉炎形成,免疫组化EnVision两步法染色IgG4阳性浆细胞与IgG阳性浆细胞比例>40%。结论 IgG4相关性疾病临床特点和影像学无特异性,易误诊为肿瘤,术前血清IgG4检测可作为疑似病例的首选方法。%Purpose To study the clinicopathological features and immunohistochemical characteristics of IgG4-related disease. Meth-ods The microscopic characteristics and immunohistochemical staining (EnVision) of IgG, IgG4, CD138 and CD34 have been per-formed on 12 cases of IgG4-related disease. Results IgG4-related disease were characterized by diffuse fibrosis, accompanied with in-filtrating of dense lymphocytes and plasma cells surrounding neurovascular and occlusive phlebitis. Immunohistochemical staining re-sults showed the ratio of IgG4+/IgG+ cells were over 40%. Conclusions IgG4-related disease is absent of characteristic clinical and radiographic features and is easily misdiagnosed as tumor. Preoperative serum IgG4 detection could be used as the prior examina-tion for the suspected cases.

  4. The G4Foam Experiment: global climate impacts of regional ocean albedo modification

    Science.gov (United States)

    Gabriel, Corey J.; Robock, Alan; Xia, Lili; Zambri, Brian; Kravitz, Ben

    2017-01-01

    Reducing insolation has been proposed as a geoengineering response to global warming. Here we present the results of climate model simulations of a unique Geoengineering Model Intercomparison Project Testbed experiment to investigate the benefits and risks of a scheme that would brighten certain oceanic regions. The National Center for Atmospheric Research CESM CAM4-Chem global climate model was modified to simulate a scheme in which the albedo of the ocean surface is increased over the subtropical ocean gyres in the Southern Hemisphere. In theory, this could be accomplished using a stable, nondispersive foam, comprised of tiny, highly reflective microbubbles. Such a foam has been developed under idealized conditions, although deployment at a large scale is presently infeasible. We conducted three ensemble members of a simulation (G4Foam) from 2020 through to 2069 in which the albedo of the ocean surface is set to 0.15 (an increase of 150 %) over the three subtropical ocean gyres in the Southern Hemisphere, against a background of the RCP6.0 (representative concentration pathway resulting in +6 W m-2 radiative forcing by 2100) scenario. After 2069, geoengineering is ceased, and the simulation is run for an additional 20 years. Global mean surface temperature in G4Foam is 0.6 K lower than RCP6.0, with statistically significant cooling relative to RCP6.0 south of 30° N. There is an increase in rainfall over land, most pronouncedly in the tropics during the June-July-August season, relative to both G4SSA (specified stratospheric aerosols) and RCP6.0. Heavily populated and highly cultivated regions throughout the tropics, including the Sahel, southern Asia, the Maritime Continent, Central America, and much of the Amazon experience a statistically significant increase in precipitation minus evaporation. The temperature response to the relatively modest global average forcing of -1.5 W m-2 is amplified through a series of positive cloud feedbacks, in which more

  5. The G4Foam Experiment: Global Climate Impacts of Regional Ocean Albedo Modification

    Energy Technology Data Exchange (ETDEWEB)

    Gabriel, Corey; Robock, Alan; Xia, Lili; Zambri, Brian; Kravitz, Benjamin S.

    2017-01-12

    Reducing insolation has been proposed as a geoengineering response to global warming. Here we present the results of climate model simulations of a unique Geoengineering Model Intercomparison Project Testbed experiment to investigate the benefits and risks of a scheme that would brighten certain oceanic regions. The National Center for Atmospheric Research CESM-CAM4-CHEM global climate model was modified to simulate a scheme in which the albedo of the ocean surface is increased over the subtropical ocean gyres in the Southern Hemisphere. In theory, this could be accomplished using a stable, nondispersive foam, comprised of tiny, highly reflective microbubbles. Such a foam has been developed under idealized conditions, although deployment at a large scale is presently infeasible. We conducted three ensemble members of a simulation (G4Foam) from 2020 through 2069 in which the albedo of the ocean surface is set to 0.15 (an increase of 150%) over the three subtropical ocean gyres in the Southern Hemisphere, against a background of the RCP6.0 (representative concentration pathway resulting in +6 W m-2 radiative forcing by 2100) scenario. After 2069, geoengineering is ceased, and the simulation is run for an additional 20 years. Global mean surface temperature in G4Foam is 0.6 K lower than RCP6.0, with statistically significant cooling relative to RCP6.0 south of 30°N. There is an increase in rainfall over land, most pronouncedly in the tropics during the June-July-August season, relative to both G4SSA (specified stratospheric aerosols) and RCP6.0. Heavily populated and highly cultivated regions throughout the tropics, including the Sahel, Southern Asia, the Maritime Continent, Central America and much of the Amazon, experience a statistically significant increase in precipitation minus evaporation. The temperature response to the relatively modest global average forcing of –1.5 W m-2 is amplified through a series of positive cloud feedbacks, in which more shortwave

  6. 头颈部IgG4相关性疾病的临床及影像学诊断%Clinical and imaging diagnosis of IgG4-related disease in the head and neck

    Institute of Scientific and Technical Information of China (English)

    余长亮; 刘斌; 余永强

    2013-01-01

    头颈部IgG4相关性疾病是新认识的一种多器官系统病变,临床上以血清IgG4升高、广泛的IgG4阳性淋巴浆细胞组织浸润、组织纤维化及皮质类固醇激素治疗有效为特征.在CT和MRI上也可呈现一些相对特征的表现,CT和MRI不仅能为头颈部IgG4相关性疾病的诊断及鉴别诊断提供有价值的信息,而且能为临床治疗方案的选择及其疗效、预后的评估提供帮助.%IgG4-related disease in the head and neck is a newly recognized multi-organ system disease characterized by elevated serum IgG4,infiltration of numerous IgG4-positive plasma cells,tissue fibrosis,and dramatic response to corticosteroid treatment.IgG4-related disease of the head and neck has some relative characteristics on CT and MRI,which can provide valuable information for the diagnosis and differential diagnosis,and are helpful for the clinical treatment,evaluation of therapeutic effects and prediction of prognosis.

  7. IgG4酶联免疫分析的建立及初步应用%Development and Primary Application of IgG4 Enzyme Linked Immunoassay

    Institute of Scientific and Technical Information of China (English)

    张凯; 颜光涛; 郝秀华; 薛辉; 王录焕; 林季

    2006-01-01

    用兔抗人IgG4的多克隆抗体(IgG4Ab)包被成固相板,以识别结合待测标本中的IgG4.用生物素标记IgG4Ab得Bio-IgG4Ab;用亲和素标记辣根酶得HRP-A.在已包被IgG4Ab的固相板中加入IgG4标准品(或待测样品),反应、洗涤后,加入Bio-IgG4Ab,反应、洗涤后,加入HRP-A,反应、洗涤后,板上形成IgG4Ab-IgG4-Bio-IgG4Ab-HRP-A复合物,加酶底物显色,用酶标仪在490 nm波长测定OD值,作标准曲线,根据标准曲线,查出标本中IgG4含量.该法测定范围:2.5~200ng/mL,最低检出量2.1ng/mL,批内和批间CV分别8.4%和11.2%.测得血清IgG4含量:青年人(30名)为37.7±2.3ng/mL;30名献血员为42.7±9.9ng/mL,49例重症监护患者明显升高为71.2±9.2ng/mL;49例肺部感染患者明显降低为28.4±9.2 ng/mL.结果表明该法稳定,灵敏度适于检出人血清IgG4水平.

  8. The usefulness of 18F-FDG PET/CT imaging in immunoglobulin G4-related disease%18F-FDG PET/CT在IgG4相关性疾病中的应用进展

    Institute of Scientific and Technical Information of China (English)

    朱冬灵; 邹思娟; 朱小华

    2015-01-01

    As a newly identified systemic syndrome characterized by a high level of serum immunoglobulin G4 ( IgG4 ) and dense infiltration of IgG4-positive plasma cells into multiple organs including lacrimal gland, salivary gland, lung, pancreas, liver, biliary duct, kidney, prostate, retroperitoneum and so on, IgG4-related disease(IgG4-RD)has attracted growing attention recently. Timely and accurate diagnosis is an essential prerequisite for early intervention leading to better recovery and outcome in patients with IgG4-RD. 18F-FDG PET/CT has been served as an important imaging tool for patients with IgG4-RD in assessing organ involvement, guiding biopsy and monitoring therapeutic response. This review will summarize the state-of-the-art on applications of 18F-FDG PET/CT in patients with IgG4-RD.%免疫球蛋白G4(IgG4)相关性疾病是近几年新发现和确定的一种复合性免疫系统疾病,其特征为血清IgG4水平升高、受累组织IgG4阳性浆细胞浸润。该病累及范围广,包括泪腺、唾液腺、肺、胰腺、肝脏、胆管、肾脏、前列腺和后腹膜等,临床表现为同时或不同时间各脏器肿大、结节或肥厚。全面、准确地诊断有利于该病的早期治疗和预后改善。18F-FDG PET/CT显像在IgG4相关性疾病中的诊断、疗效评估与随访监测等方面具有重要作用。笔者将对18F-FDG PET/CT在IgG4相关性疾病中的应用及研究进展进行综述。

  9. High Expression of Galectin-3 in Patients with IgG4-Related Disease: A Proteomic Approach

    Directory of Open Access Journals (Sweden)

    Adeeb Salah

    2017-01-01

    Full Text Available Objectives. Immunoglobulin G4-related disease (IgG4-RD is a multiorgan condition manifesting itself in different forms. This study aimed to investigate protein expression profiles and to find the possible biomarker for IgG4-RD by liquid chromatography mass spectrometry (LC-MS using tissue sections in IgG4-RD patients. Methods. Protein expression profiles in five IgG4-related pancreatitis and three normal pancreatic samples were compared using LC-MS and were validated by quantitative real-time PCR (qRT-PCR, immunoblotting, and immunohistochemistry. ELISA was employed in the serum of 20 patients with systemic IgG4-RD before and during steroid treatment. Results. LC-MS indicated that the levels of 17 proteins were significantly higher and 12 others were significantly lower in IgG4-related pancreatitis patients compared to controls. Among these proteins, galectin-3 levels were 13-fold higher in IgG4-related pancreatitis (P<0.01. These results were confirmed by immunoblotting and qRT-PCR. The average number of galectin-3 + cells in various organs of IgG4-RD patients, including salivary glands, lungs, and lymph nodes, was higher than in controls. Galectin-3 was detectable in macrophages, dendritic cells, and stromal myofibroblast-like cells, but not in lymphocytes by immunofluorescence staining. Serum galectin-3 levels were higher in patients with IgG4-RD compared with healthy donors and remained high during steroid therapy. Conclusion. Galectin-3 was overexpressed in IgG4-RD and the levels were indirectly related to clinical activity.

  10. 基于IgG4分类的桥本甲状腺炎临床意义探讨%Clinical significance of IgG4 related Hashimoto thyroiditis

    Institute of Scientific and Technical Information of China (English)

    钱伟; 杜婷婷; 吴汉妮

    2015-01-01

    目的:探讨IgG4相关性桥本甲状腺炎( HT)患者血清IgG4水平及其临床意义。方法:酶联免疫吸附法测定129例HT患者血清IgG4水平,并根据IgG4水平将患者分为IgG4组(IgG4≥135 mg/dl)和非IgG4组(IgG4<135 mg/dl);电化学发光法测定血清甲状腺激素、甲状腺过氧化物酶抗体( TPOAb)、甲状腺球蛋白抗体( TgAb);甲状腺超声影像检查。结果:HT患者血清TPOAb与同时测定的IgG4水平(r=0.4371,P=0.0127)和IgG4/IgG比值(r=0.3962,P=0.0235)呈显著正相关。与非IgG4组(97例)比较,IgG4组(32例):①平均患病年龄趋年轻化(P=0.0293);②较高的血清TPOAb(P=0.0021)、TgAb(P=0.0128)水平;③超声影像:更易形成甲状腺结节(P=0.0226);④多因素Logistic回归分析结果提示,血清IgG4、TPOAb水平是患甲状腺结节危险因素,OR值分别为1.672(P=0.021)、2.549(P=0.014)。结论:IgG4相关性HT患者存在相应的临床特征。对于血清中IgG4水平明显升高的HT患者,应更加密切监测甲状腺功能及形态变化。%Objective:To explore the serum IgG4 level in patients with IgG4-related Hashimoto thyroiditis(IgG4 HT),and its clinical implications.Methods:The serum IgG4 was determined in 129 patients with HT using enzyme-linked immunosorbent assays and classified into two subgroups based on IgG4 level:IgG4 HT group(IgG4≥135 mg/dl)and non-IgG4 HT group(IgG4<135 mg/dl).And the levels of serum thyroid hormone and thyroid peroxidase antibodies(TPOAb)and thyroglobulin antibodies(TgAb)were measured by electro-chemiluminescence immunoassay.Ultrasonic imaging of the thyroid gland were detected.Results:The TPOAb levels correlated significantly with both serum IgG4 levels(r=0.437 1,P=0.012 7)and IgG4/IgG ratios(r=0.396 2,P=0.023 5)in the patients with HT.Compared with that of non-IgG4 HT group(n=97),IgG4 HT group(n=32):①The mean age was lower

  11. Serum Immunoglobulin Free Light Chain Assessment in IgG4-Related Disease

    Directory of Open Access Journals (Sweden)

    Aurélie Grados

    2013-01-01

    Full Text Available Immunoglobulin free light chains are produced in excess during normal antibody synthesis. Their evaluation is commonly used in case of a monoclonal gammopathy. In polyclonal hypergammaglobulinemia related to the Sjögren syndrome or systemic lupus, erythematosus serum free light chain levels are increased and could correlate with disease activity. We show here that the κ ( and λ ( free light chains and the κ : λ ratio ( are increased in sixteen patients with IgG4-related disease when compared to healthy controls. The increase of κ and λ free light chains probably reflects the marked polyclonal B cell activation of the disease. We could not assess in this small cohort of patients a significative correlation of serum free light chain levels and disease activity or extension.

  12. IgG4-related sclerosing mesenteritis in a 7-year-old Saudi Girl

    Directory of Open Access Journals (Sweden)

    Mohammed Y Hasosah

    2014-01-01

    Full Text Available Sclerosing mesenteritis (SM is a rare, benign inflammatory disorder of unknown etiology, affecting the membranes of the digestive tract that involves lymphoplasmacytic inflammation, fat necrosis, and fibrosis of the mesentery. We report a child patient with a history of recurrent abdominal pain and fever who was found to have an intra-abdominal mass suspicious for malignancy. A tissue biopsy revealed the diagnosis of SM associated with IgG4-related systemic disease. The patient is currently maintained on 5 mg prednisone daily and no recurrence of symptoms was noted during the 24-month follow-up period. We emphasize, therefore, that SM can present clinical challenges and the presence of SM should cue clinicians to search for other coexisting autoimmune disorders that can have various outcomes.

  13. G4-Tetra DNA Duplex Induce Lung Cancer Cell Apoptosis in A549 Cells

    Science.gov (United States)

    Xu, Xiaobo; Zhao, YiZhuo; Lu, Hu; Fu, Cuiping; Li, Xiao; Jiang, Liyan; Li, Shanqun

    2016-10-01

    The specific DNA is typically impermeable to the plasma membrane due to its natural characters, but DNA tetra structures (DTNs) can be readily uptake by cells in the absence of transfection agents, providing a new strategy to deliver DNA drugs. In this research, the delivery efficiency of tetrahedral DNA nanostructures was measured on adenocarcinomic human alveolar basal epithelial (A549) cells via delivering AS1411 (G4). The DNA tetra-AS1411 complex was rapidly and abundantly uptake by A549 cells, and the induced apoptosis was enhanced. Furthermore, biodistribution in mouse proved the rapid clearance from non-targeted organs in vivo. This study improved the understanding of potential function in DNA-based drug delivery and proved that DTNs-AS1411 could be potentially useful for the treatment of lung cancer.

  14. IgG4-related sclerosing mesenteritis in a 7-year-old Saudi girl.

    Science.gov (United States)

    Hasosah, Mohammed Y; Satti, Mohamed B; Yousef, Yasmin A; Alzahrani, Daifullah M; Almutairi, Sajdi A; Alsahafi, Ashraf F; Sukkar, Ghassan A; Alzaben, Abdullah A

    2014-01-01

    Sclerosing mesenteritis (SM) is a rare, benign inflammatory disorder of unknown etiology, affecting the membranes of the digestive tract that involves lymphoplasmacytic inflammation, fat necrosis, and fibrosis of the mesentery. We report a child patient with a history of recurrent abdominal pain and fever who was found to have an intra-abdominal mass suspicious for malignancy. A tissue biopsy revealed the diagnosis of SM associated with IgG4-related systemic disease. The patient is currently maintained on 5 mg prednisone daily and no recurrence of symptoms was noted during the 24-month follow-up period. We emphasize, therefore, that SM can present clinical challenges and the presence of SM should cue clinicians to search for other coexisting autoimmune disorders that can have various outcomes.

  15. Oral lichen sclerosus expressing extracellular matrix proteins and IgG4-positive plasma cells.

    Science.gov (United States)

    De Aquino Xavier, Flavia Calo; Prates, Alisio Alves; Gurgel, Clarissa Araujo; De Souza, Tulio Geraldo; Andrade, Rodrigo Guimaraes; Goncalves Ramos, Eduardo Antonio; Pedreira Ramalho, Luciana Maria; Dos Santos, Jean Nunes

    2014-09-16

    Lichen sclerosus (LS) is a mucocutaneous disease with uncommon oral involvement. The etiology is not yet well understood, but LS has been associated with autoimmune, genetic, and immunological factors. We report a 47-year-old man with LS that exhibited an asymptomatic white plaque with red patches on the maxillary alveolar mucosa extending to the labial mucosa. He had no other skin disease. Positive immunostaining for tenascin and scarcity of fibronectin suggested extracellular matrix reorganization. Elastin immunostaining indicated a reduction of elastic fibers. Immunoexpression of collagen IV in blood vessels and its absence in the epithelial basement membrane, together with diffuse MMP-9 immunoexpression, suggested altered proteolytic activity. Mast cell staining bordering areas of sclerosis indicated a possible role in the synthesis of collagen. IgG4 positivity in plasma cells suggested a role in the fibrogenesis. This is an unusual presentation of oral LS and we discuss immunohistochemical findings regarding cellular and extracellular matrix components.

  16. Thermodynamic analysis of biogenic and synthetic polyamines conjugation with PAMAM-G4 nanoparticles.

    Science.gov (United States)

    Chanphai, P; Tajmir-Riahi, H A

    2016-02-01

    We report the thermodynamic analysis of the bindings of poly(amidoamine) (PAMAM-G4) nanoparticles with biogenic polyamines spermine (spm), spermidine (spmd) and synthetic polyamines 3,7,11,15-tetrazaheptadecane·4HCl (BE-333) in aqueous solution at physiological conditions. Multiple spectroscopic methods, thermodynamic parameters and molecular modelling were used to analyse polyamine bindings to PAMAM dendrimers. Thermodynamic parameters ΔS, ΔH and ΔG parameters showed that polyamines bind polymer through H-bonding and van der Waals contacts with biogenic polyamines form more stable conjugates than synthetic polyamines. Modelling showed that polyamines are located at the surface of PAMAM with the free binding energy of -3.56 (spermine), -3.88 (spermidine) and -3.13 kcal/mol (BE-333), indicating spontaneous polyamine-polymer interaction at room temperature.

  17. IgG4-related retroperitoneal fibrosis: A case report and literature review%IgG4相关性腹膜后纤维化病例报告并文献复习

    Institute of Scientific and Technical Information of China (English)

    王聪; 赵忺; 田建立; 巩路

    2015-01-01

    Clinical data of one patient diagnosed as IgG4-related retroperitoneal fibrosis was analyzed."IgG4" and "retroperitoneal fibrosis" were used as the key words to search literatures from PubMed and Wanfang Database.Clinical data of 27 cases with IgG4 retroperitoneal fibrosis,including clinical manifestation,diagnostic basis,therapeutic method and prognosis,which were retrieved through literatures,were analyzed.A 82-year-old male patient presented with urinary tract obstruction and renal insufficiency.Imaging examination revealed a retroperitoneal irregular lump shadow,and showed chronic inflammation of soft tissues in posterior peritoneum with fibrosis by biopsy pathology.The IgG4-positive plasmocyte infiltration was found by immunohistochemical method.The serum level of IgG4 was significantly increased,which was in accord with IgG4-related retroperitoneal fibrosis.After treatment with methylprednisolone,retroperitoneal lump shadow was obviously dissipated.The serum level of IgG4 became normal.27 cases with IgG4-related retroperitoneal fibrosis were searched through literature retrieval,with 19 confirmed cases,3 suspected cases,and 5 suspicious cases.Male accounted for 92.6% (25/27),and the average age was 67.6 years old.The cases with the involvement of extraperitoneal organs accounted for 37.0% (10/27).The misdiagnosis rate was 55.6% (15/27).23 cases took effective therapy with glucocorticoids.IgG4-related retroperitoneal fibrosis is an orphan disease,which can be misdiagnosed easily.The diagnosis is made with histopathological examination and determination on the serum level of IgG4.The therapy with glucocorticoids is effective.

  18. Giant hepatic artery aneurysm associated with immunoglobulin G4-related disease successfully treated using a liquid embolic agent

    Energy Technology Data Exchange (ETDEWEB)

    Rossi, Michele; Virgilio, Edoardo; Laurino, Florindo; Orgera, Gianluigi; Mene, Paolo; Pirozzi, Nicola; Ziparo, Vincenzo; Cavallini, Marco [St. Andrea Hospital, Rome (Italy)

    2015-08-15

    The occurrence of a giant hepatic artery aneurysm (GHAA) in a patient with systemic vasculitis is very rare. Herein, we describe our endovascular treatment experience of a GHAA associated with immunoglobulin G4-related disease (IgG4-RD) consisting primarily of a liquid embolic injection and deployment of a vascular plug.

  19. Current research of immunoglobulin G4-related orbital disease%免疫球蛋白G4相关性眼眶病的研究进展

    Institute of Scientific and Technical Information of China (English)

    李静

    2012-01-01

    In recent years,immunoglobulin G4-related disease (IgG4-related disease) has received increasingly attention in medical community as a novel clinical entity.Although its clinical manifestation varies,high serum IgG4 concentration and numerous IgG4-positive plasma c(c)ll infiltration are the common characteristics.IgG4-related disease involves many human organs,and most one is pancreas,followed by parotid gland,bile duct,liver,lung,lymph node and so on,and orbital involvement is relatively rare.Current initial research shows that there is certain association between orbital disease with IgG4,and these diseases mainly include benign lymphoepithelial lesion,idiopathic orbital inflammatory pseudotumor,Castleman ' s disease,xanthogranuloma,Rosai-Dorfman disease etc..The researching progression in the relationship between IgG4 and orbital diseases is reviewed.%近年来,免疫球蛋白G4(IgG4)相关性疾病作为一种新的临床疾病正逐渐受到医学界的广泛关注.尽管该病临床表现多种多样,但是血清IgG4质量浓度增高和组织中IgG4阳性浆细胞的大量浸润是其共有的特征.该病可累及全身多个器官,最常见的是胰腺,其次为腮腺、胆管、肝脏、肺脏和淋巴结等,累及眼眶者较为少见.目前研究初步显示,良性淋巴上皮病变、特发性眼眶炎性假瘤、Castleman病、黄色肉芽肿、RosaiDorfman病等几种眼眶病具有IgG4相关性疾病的特征.就IgG4相关性眼眶病的研究进展进行综述.

  20. Association Between HLA Haplotypes and Increased Serum Levels of IgG4 in Patients With Primary Sclerosing Cholangitis.

    Science.gov (United States)

    Berntsen, Natalie L; Klingenberg, Olav; Juran, Brian D; Benito de Valle, Maria; Lindkvist, Björn; Lazaridis, Konstantinos N; Boberg, Kirsten Muri; Karlsen, Tom H; Hov, Johannes Roksund

    2015-05-01

    Increased serum levels of IgG4 have been reported in 9%-15% of patients with primary sclerosing cholangitis (PSC); it is not clear whether this increase contributes to pathogenesis. We performed genetic analyses of the HLA complex in patients with PSC from Norway, Sweden, and from the United States. We found an association between levels of IgG4 above the upper reference limit and specific HLA haplotypes. These patients had a significantly lower frequency of the strongest PSC risk factor, HLA-B*08, than patients without increased IgG4, and significantly higher frequencies of HLA-B*07 and HLA-DRB1*15. HLA genotype therefore might affect the serum concentration of IgG4, and increased IgG4 might be a marker of a distinct phenotype of PSC.

  1. Die Suche nach dem verschollenen Großsteingrab G4 'Onner es' (Onnen, Prov. Groningen)

    NARCIS (Netherlands)

    Groenendijk, Hendrik; Lanting, JN; Woldring, H

    2014-01-01

    On the eastern hillside of the Hondsrug near the hamlet Onnen (Gr.), a scatter of surface finds led to the belief that a megalithic grave (hunebed) must have been present on the Onner es. Through soil radar, test excavation, pollen analysis and coring, the former location of the presumed hunebed was

  2. Mg2+对Bloom综合症解旋酶与G4DNA结合的影响研究%Study on the effects of Mg2+ on the binding between Bloom syndrome helicase and G4DNA

    Institute of Scientific and Technical Information of China (English)

    骆衡; 蔡明娟; 陈祥; 丁玫; 李坤; 许厚强

    2012-01-01

    利用荧光偏振技术检测了Mg2+对G4DNA、BLM-G4DNA复合物和BLM642-1290解旋酶与G4DNA结合的影响.结果表明,G4DNA荧光偏振值随着Mg2+浓度的增加而增加(P<0.01);BLM-G4DNA复合物的荧光偏振值随着Mg2+浓度的增加出现下降—升高—下降的变化趋势(P<0.01);G4DNA与BLM642-1290解旋酶结合的荧光偏振值随着Mg2+浓度的增加而逐渐下降(P<0.01);分析不同Mg2+浓度下两种分子结合的Kd值,发现Mg2+浓度为3.0 mmol/L时,BLM642-1290解旋酶与G4DNA最容易结合,表明适量Mg2+浓度会促进BLM642-1290与G4DNA的结合,但会引起两种分子结合的形状、流动性和电荷等性质的改变.这些结果可为进一步研究BLM解旋酶对G4DNA的作用机理提供相关资料.%This paper studied the effects of Mg2+ on G4DNA, BLM-G4DNA complex, and the binding of BLM642-1290 helicase and G4DNA using fluorescence anisotropy technology. The results indicated that the fluorescence anisotropy of G4DNA increased with Mg2+ concentration (P< 0.01); The anisotropy of BLM-G4DNA complex showed varied tendency of dccreasc-increasc-decrease with Mg2+ concentration (P<0. 01) ;The anisotropy of the binding between the helicase and G4DNA gradually declined following Mg2+ concentration (PG4DNA when Mg2+ concentrations was 3. 0 mmol/L. which suggested that the suitable amount of Mg2+ could promote the binding of the helicase and G4DNA but that could lead to the change of the binding properties of two molecules including the shape, flow ability, and charge. These results provided useful data for studying the interaction mechanism between BLM helicase and G4DNA.

  3. A brief contextualization on IgG4 tubulointerstitial nephritis based on a case report in south Brazil.

    Science.gov (United States)

    Pêgas, Karla Lais; Cambruzzi, Eduardo; Lobato, Gisele

    2016-06-01

    IgG4-related disease (IgG4RD) is a recent inflammatory process of supposed autoimmune etiology, which is characterized by elevated serum IgG4 levels, dense lymphoplasmacytic infiltration rich in IgG4-positive plasma cells and storiform fibrosis. Tubulointerstitial nephritis is the most common renal manifestation, with different degrees of kidney dysfunction and variable clinical findings. Herein, the authors describe a new case of IgG4 tubulointerstitial nephritis (IgG4TN), and discuss clinic and pathologic criteria. Male patient, 72 years-old, was admitted on hospital service with clinical complaint of asthenia, loss of strength, emaciation, and anosmia. Previous history included type 2 diabetes mellitus. Laboratorial data included normochromic anemia, proteinuria, and creatinine elevation. Bilateral kidney ultrassonography/computed tomography revealed a heterogenous parenchyma, with diffuse irregular dense zones, areas of fibrosis on upper poles, and hydronephrosys. Kidney biopsy showed a dense interstitial lymphoplasmacytic infiltrate, with more than 50 plasma cell per high power field, irregular areas of fibroblastic and collagenous fibrosis, focal tubulitis, and normal glomeruli. Immunofluorescence revealed mild granular deposition of C3c and IgG in the tubular basement membrane. Immunohistochemestry was positive for CD138, lambda and Kappa light chains, and IgG4 (around forty five IgG4 positive plasma cells per high power field). IgG4 serum level was increased. The diagnosis of IgG4TN was then established. The patient received corticotherapy and strict control of glycemia with insulin, with marked improvement of symptoms and creatinine levels.

  4. IgG4-related kidney disease: MRI findings with emphasis on the usefulness of diffusion-weighted imaging

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Bohyun; Kim, Jin Hee, E-mail: kimjhrad@amc.seoul.kr; Byun, Jae Ho; Kim, Hyoung Jung; Lee, Seung Soo; Kim, So Yeon; Lee, Moon-Gyu

    2014-07-15

    Objectives: To investigate the imaging findings of immunoglobulin G4 (IgG4)-related kidney disease (IgG4-KD) on magnetic resonance imaging (MRI) including diffusion-weighted imaging (DWI) and to evaluate the usefulness of DWI in lesion detection. Methods: This retrospective cohort study included 31 patients with IgG4-KD who underwent MRI covering both kidneys. Two radiologists reviewed in consensus the MR images to determine the distribution pattern (location, laterality, and multiplicity) and the visually assessed signal intensity (hypointense, isointense or hyperintense) of the renal lesions compared to the normal renal parenchyma on each sequence. Per-patient sensitivity for detecting IgG4-KD and the number of detectable lesions were compared in T2-weighted images, DWI, and dynamic contrast-enhanced images. Results: IgG4-KD typically manifested as bilateral (83.9%), multiple (93.5%), and renal parenchymal (87.1%) nodules appearing isointense (93.5%) on T1-weighted images, hypointense (77.4%) on T2-weighted images, hyperintense (100%) on DWI (b = 1000), and hypointense (83.3%) in the arterial phase and with a progressive enhancement pattern on dynamic contrast-enhanced images. The sensitivity of DWI for detecting IgG4-KD was significantly higher than that of T2-weighted images (100% vs. 77.4%, P = 0.034). The median number of detectable lesions was significantly greater in DWI (n = 9) than in T2-weighted images (n = 2) and dynamic contrast-enhanced images (n = 5) (P ≤ 0.008). Conclusions: The characteristic MRI findings of IgG4-KD were bilateral, multiple, renal parenchymal nodules with T2 hypointensity, diffusion restriction, and a progressive enhancement pattern. As DWI was useful in the detection of IgG4-KD, adding DWI to conventional MRI for patients suspected of having IgG4-KD may enhance the diagnosis.

  5. Concomitant occurrence of IgG4-related pleuritis and periaortitis: a case report with review of the literature.

    Science.gov (United States)

    Ishida, Mitsuaki; Hodohara, Keiko; Furuya, Aya; Fujishiro, Aya; Okuno, Hiroko; Yoshii, Miyuki; Horinouchi, Akiko; Shirakawa, Ayaka; Harada, Ayumi; Iwai, Muneo; Yoshida, Keiko; Kagotani, Akiko; Yoshida, Takashi; Okabe, Hidetoshi

    2014-01-01

    IgG4-related sclerosing disease is an established disease entity with characteristic clinicopathological features. Some recent reports have demonstrated that this disease can occur in the respiratory system including the pleura. Herein, we describe the first documented case of concomitant occurrence of IgG4-related pleuritis and periaortitis. A 71-year-old Japanese female with a history of essential thrombocythemia presented with persistent cough and difficulty in breathing. Computed tomography demonstrated thickening of the right parietal pleura, pericardium, and periaortic tissue and pleural and cardiac effusions. Histopathological study of the surgical biopsy specimen of the parietal pleura revealed marked fibrous thickening with lymphoplasmacytic infiltration. Phlebitis was noted, however, only a few eosinophils had infiltrated. Immunohistochemical study revealed abundant IgG4-positive plasma cell infiltration and high ratio of IgG4-/IgG-positive plasma cells (84%). Therefore, a diagnosis of IgG4-related pleuritis was made with consideration of the elevated serum IgG4 level (684 mg/dL). Recently, the spectrum of IgG4-related sclerosing disease has expanded, and this disease can occur in the pleura, pericardium, and periaortic tissue. Although histopathological analysis of the pericardium and periaortic tissue was not performed in the present case, it was suspected that thickening of the pericardium and periaortic tissue was clinically due to IgG4-related sclerosing disease. Our clinicopathological analyses of IgG4-related pleuritis and pericarditis reveal that this disease can present as dyspnea and pleural and pericardial effusion as seen in the present case, therefore, it is important to recognize that IgG4-related sclerosing disease can occur in these organs for accurate diagnosis and treatment.

  6. The application of PET/CT in immunoglobulin G4-related disease%免疫球蛋白G4相关性疾病的PET/CT应用

    Institute of Scientific and Technical Information of China (English)

    张慧玮; 赵军

    2013-01-01

    Immunoglobulin G4 (IgG4)-related disease is a kind of new systemic entity characterized by mass-forming lesions in various organs that consist of lymphoplasmacytic infiltrates and fibrosclerosis with numerous IgG4-positive plasma cells.PET/CT has been widely used in clinical as a whole body examination that earned plenty of successful experience,especially in multiple locations and organs cancer such as lymphoma.PET/CT also has a potential application in IgG4-relatcd diseases as its feature of systemic.%免疫球蛋白G4(IgG4)相关性疾病是一种以器官肿胀、IgG4水平升高和IgG4阳性淋巴浆细胞的组织浸润为特征的纤维炎症性疾病,该疾病可累及多器官.PET/CT作为一种全身检查现已广泛应用于临床,特别是在以淋巴瘤为代表的累及多部位、多器官疾病的应用中已经取得了成功的经验.又因IgG4相关性疾病具有系统性疾病的特点,PET/CT在该疾病中可能具有广阔的应用前景.

  7. IgG4-associated parotitis:case report and review of the literature%IgG4相关硬化性腮腺炎1例报告及文献复习

    Institute of Scientific and Technical Information of China (English)

    张青; 方利华; 平金良

    2013-01-01

    A case of immunoglobulin G4 (IgG4)-associated parotitis was reported and related literatures were reviewed,in order to improve the recognization of this systemic diseases and reduce the misdiagnosis and mistreatment in clinical practice for the stomatologists.%通过对1例IgG4相关硬化性腮腺炎疾病的诊治的介绍和相关文献的回顾复习,增加国内口腔科医师对该类疾病的认识,以减少临床误诊误治.

  8. 肺受累的IgG4相关硬化性疾病1例%A case report of IgG4-related sclerosing disease with lung involvement

    Institute of Scientific and Technical Information of China (English)

    张颖健; 赵金霞; 刘蕊; 刘湘源

    2012-01-01

    IgG4-related sclerosing disease ( IgG4-RSD) is a kind of lymphoplasmacytic disease with multi-organ involvement and is characterized by serum IgG4 elevation and tissue IgG4 positive plasma cell infiltration. Autoimmune pancreatitis, sclerosing cholangitis, sclerosing sialadenitis, retroperitoneal fibrosis and lymphadenopathy make up its main clinical manifestations. This difficult case was a middle-aged female with onset as muiltiple lymph nodes and glands enlargement, including lacrimal gland, salivary glands and pancreas. Meanwhile, repeated examinations of auto-antibodies and serum IgG4 were all negative. The patient didn' t respond well to glucocorticoid therapy, and further progressed to rare lung involvement presenting as lung nodule. This complex entity was eventually diagnosed as IgG4-RSD by the support of histopathology evidence of IgG4 immunohistochemistry stain. Though IgG4-RSD has been known for years, it is still underappreciated in China and case reports are scarce. The case report here with literature review is just to enhance the recognition of this disease regarding its pathogenesis, various clinical manifestations, diagnosis and therapy.%IgG4相关硬化性疾病(IgG4 related sclerosing disease,IgG4-RSD)是一种累及多器官、以血清IgG4升高、组织IgG4阳性浆细胞浸润为特点的淋巴浆细胞病,主要表现为自身免疫性胰腺炎、硬化性胆管炎、硬化性涎腺炎、腹膜后纤维化和淋巴结病.本例患者以泪腺、唾液腺、淋巴结肿大起病,多种自身抗体阴性,外院应用激素效果不佳,多次查血清IgG4阴性,为诊断带来困难,患者在此基础上出现以肺内结节为主要表现的肺受累更为罕见,最终通过组织活检IgG4免疫组织化学染色确诊.IgG4-RSD作为一种新的临床疾病逐渐被广泛认识,但国内报道仍较少.报道这一病例并进行相关文献复习旨在提高对IgG4-RSD多种临床表现的认识并对其诊疗及发病机制进行探讨.

  9. Paying attention to the research on IgG4 related orbital disease%重视IgG4相关性眼眶疾病的研究

    Institute of Scientific and Technical Information of China (English)

    马建民; 李静

    2015-01-01

    IgG4 related disease (IgG4-RD) is a systemic disease which was named and accepted in recent years.IgG4-RD is a multiorgan-involved disease, mainly including pancrease, parotid gland, bile duct, liver, etc.The study on the relationship between IgG4 and orbital disease is relatively few.In recent years, our study group aimed directly to the relationship between IgG4 and common orbital disease, including benign lymphoepithelial lesion of lacrimal gland,idiopathic orbital inflammatory pseudotumor, orbital lymphoma, etc.We found that IgG4 was associated with the pathogenesis and progression of these diseases.This article addressed systematically the pathogenesis of IgG4 related orbital disease and evaluated the values of IgG4 in diagnosis,differential diagnosis,treatment and montoring of these orbital diseases based on related literature and reseaching results from our study group,in order to deepen the recognition of IgG4 related orbital diseases in ophthalmologists.%IgG4相关性疾病是近年被国际医学界逐渐认可并接受的一种全身性疾病的统称,其可累及全身多个组织器官,常见的受累器官为胰腺、腮腺、胆管和肝脏等,但目前有关IgG4与眼眶疾病之间关系的研究较为少见.近年来我们课题组主要针对IgG4与泪腺良性淋巴上皮病变、眼眶炎性假瘤、眼眶淋巴瘤等常见眼眶疾病之间关系进行研究,发现上述疾病的发生及发展与IgG4之间存在一定联系.在结合相关文献及我们课题组研究结果的基础上,本文将系统阐述IgG4相关性眼眶疾病的发病机制以及IgG4在IgG4相关性眼眶疾病诊断、鉴别诊断、治疗和预后中的作用,以期提高眼科医师对IgG4相关性眼眶疾病的认识.

  10. IgG4相关性疾病合并恶性肿瘤的病例对照研究%A retrospective case-control study of immunoglobulin G4-related disease combined with malignancy

    Institute of Scientific and Technical Information of China (English)

    冯云路; 吴东; 张晟瑜; 吴晰; 舒慧君; 杨爱明; 钱家鸣

    2016-01-01

    IgG4-related disease (IgG4-RD) has been proved to be associated with malignancy.The incidence and risk factors of malignancy development in IgG4-RD were not clear.Nine IgG4-RD patients with malignancies and 27 IgG4-RD control cases were analyzed for risk factors and clinical features.The incidence of malignancy in IgG4-RD was 3.3%,higher than age-controlled general population.Smoking history was significantly more common in patients with malignancies than in the control group (9/9 vs 16/27,P < 0.05).A total of 6/9 malignancies occurred within the first year after the diagnosis of IgG4-RD.Colorectal,biliary and thyroid cancers were the leading types.Smoking history is a risk factor for IgG4-RD associated malignancy.Careful vigilance to monitor malignancy needs to be paid during follow-up.%加深对IgG4相关性疾病(IgG4-RD)合并恶性肿瘤高危人群的认识,提高早期诊断率.回顾分析我院确诊的9例IgG4-RD合并恶性肿瘤患者(恶性肿瘤组),并根据性别和年龄匹配27例未合并恶性肿瘤IgG4-RD患者(对照组),进行对照分析.IgG4-RD患者合并恶性肿瘤的发生率为3.3%,高于同年龄组一般人群.恶性肿瘤组9例患者均吸烟,比例明显高于对照组(9/9比16/27,P<0.05),两组之间受累器官数目、饮酒史、血清IgG4水平、糖皮质激素治疗情况和原发病复发率的差异均无统计学意义.恶性肿瘤组中,诊断IgG4-RD后1年内发现恶性肿瘤者占6/9;肿瘤按发生率高低依次为结直肠癌、胆系肿瘤和甲状腺癌.吸烟史是IgG4-RD合并恶性肿瘤的高危因素.在诊断IgG4-RD后,应保持警惕并注意筛查和随访,及时发现恶性肿瘤.

  11. 自身免疫性肝病患者血清IgG4水平分析%Serum IgG4 levels in patients with autoimmune liver diseases

    Institute of Scientific and Technical Information of China (English)

    张敏; 朱争艳; 韩涛; 聂彩云; 王伟; 杜卫杰; 周淑芬; 王棕覃; 孙扬; 丁贤

    2014-01-01

    To study the differences of serum IgG4 levels in various autoimmune liver diseases and to analyze the clinical characterization of patients with high serum IgG4 levels. Methods 65 patients with autoimmune liver diseases were enrolled for this study including 11 patients with autoimmune hepatitis (AIH),47 patients with primary biliary cirrhosis (PBC) and 7 patients with overlap syndrome of AIH and PBC. The serum IgG4 levels was detected by immuno-scatter turbidmetry. The differences of serum IgG4 levels in the three groups were studied. The clinical features of patients with autoimmune liver disease were analyzed according to their serum IgG4 levels. The significance of inter-group differences of measurement data was assessed by Student’s t-test or Mann-Whitney U test and enumeration data by Fisher’s exact test. Results The serum IgG4 levels in patients with AIH was 642.2 mg/L(97.7 mg/L~1687.0 mg/L),a little higher than in patients with PBC [153.9 mg/L(78.9 mg/L~400.3 mg/L),P=0.076] and in with overlap syndrome [229.9 mg/L(154.9 mg/L ~417.9 mg/L),P=0.388];Three patients with AIH had high serum IgG4 levels (≥1350 mg/L) out of 11 patients with AIH. The ratio of IgG4/IgG was higher in the three patients with higher as compared to 8 with relatively low serum IgG4 levels (P﹤0.05);The three patients with elevated IgG4 levels had diabetes mellitus type 2 and one of them was also complicated with rheumatoid arthritis,whereas the other 8 patients with low serum IgG4 levels were not;14 patients with PBC with higher serum IgG4 levels (≥200 mg/L) had higher total serum bilirubin levels as com-pared to those in 33 patients with relatively low serum IgG4 levels [(45.09 ±74.85)μmol/L vs.(26.38 ±23.03)μmol/L,P=0.05)]. Conclusion Compared with patients with PBC or overlap syndrome of AIH and PBC,the pa-tients with AIH have a higher serum IgG4 levels. The patients with AIH with high serum IgG4 levels might be complicated with other autoimmune or metabolic diseases.%探

  12. Using G4FETs as a Data Router for In-Plane Crossing of Signal Paths

    Science.gov (United States)

    Fijany, Amir; Vatan, Farrokh; Mojarradi, Mohammad; Toomarian, Nikzad; Johnson, Travis; Kolawa, Elizabeth; Blalock, Benjamin; Cristoloveanu, Sorin; Chen, Suheng; Akarvardar, Kerem

    2007-01-01

    Theoretical analysis and some experiments have demonstrated that siliconon- insulator (SOI) 4-gate transistors the type known as G(exp 4)FETs could be efficiently used for in-plane crossing of signal paths. Much of the effort of designing very-large-scale integrated (VLSI) circuits is focused on area-efficient routing of signals. The main source of difficulty in VLSI signal routing is the requirement to prevent crossing, in the same plane, of wires that are meant to be kept electrically insulated from each other. Consequently, it often becomes necessary to design and build VLSI circuits in multiple layers with vias (connections between conductors in different layers at selected locations). Suitable devices that would prevent, or at least sufficiently suppress, undesired electrical coupling (cross-talk) between wires crossing in the same plane would enable compact, simpler implementation complex interconnection networks with in-plane crossings that, heretofore, have not been possible in VLSI circuitry. The use of G4FETs as in-plane signal-crossing devices or routers, in combination with the use of G(exp 4)FETs as universal programmable logic gates, would create opportunities for reducing complexity in VLSI design.

  13. Optimization and in vivo toxicity evaluation of G4.5 PAMAM dendrimer-risperidone complexes.

    Directory of Open Access Journals (Sweden)

    Maria Jimena Prieto

    Full Text Available Risperidone is an approved antipsychotic drug belonging to the chemical class of benzisoxazole. This drug has low solubility in aqueous medium and poor bioavailability due to extensive first-pass metabolism and high protein binding (>90%. Since new strategies to improve efficient treatments are needed, we studied the efficiency of anionic G4.5 PAMAM dendrimers as nanocarriers for this therapeutic drug. To this end, we explored dendrimer-risperidone complexation dependence on solvent concentration, pH and molar relationship. The best dendrimer-risperidone incorporation (46 risperidone molecules per dendrimer was achieved with a mixture of chloroform:methanol 50∶50 v/v solution pH 3. In addition, to explore the possible effects of this complex, in vivo studies were carried out in the zebrafish model. Changes in the development of dopaminergic neurons and motoneurons were studied using tyrosine hydroxylase and calretinin, respectively. Physiological changes were studied through histological sections stained with hematoxylin-eosin to observe possible morphological brain changes. The most significant changes were observed when larvae were treated with free risperidone, and no changes were observed when larvae were treated with the complex.

  14. Optimization and in vivo toxicity evaluation of G4.5 PAMAM dendrimer-risperidone complexes.

    Science.gov (United States)

    Prieto, Maria Jimena; del Rio Zabala, Nahuel Eduardo; Marotta, Cristian Hernán; Carreño Gutierrez, Hector; Arévalo Arévalo, Rosario; Chiaramoni, Nadia Silvia; del Valle Alonso, Silvia

    2014-01-01

    Risperidone is an approved antipsychotic drug belonging to the chemical class of benzisoxazole. This drug has low solubility in aqueous medium and poor bioavailability due to extensive first-pass metabolism and high protein binding (>90%). Since new strategies to improve efficient treatments are needed, we studied the efficiency of anionic G4.5 PAMAM dendrimers as nanocarriers for this therapeutic drug. To this end, we explored dendrimer-risperidone complexation dependence on solvent concentration, pH and molar relationship. The best dendrimer-risperidone incorporation (46 risperidone molecules per dendrimer) was achieved with a mixture of chloroform:methanol 50∶50 v/v solution pH 3. In addition, to explore the possible effects of this complex, in vivo studies were carried out in the zebrafish model. Changes in the development of dopaminergic neurons and motoneurons were studied using tyrosine hydroxylase and calretinin, respectively. Physiological changes were studied through histological sections stained with hematoxylin-eosin to observe possible morphological brain changes. The most significant changes were observed when larvae were treated with free risperidone, and no changes were observed when larvae were treated with the complex.

  15. Viability of Bifidobacterium Pseudocatenulatum G4 after Spray-Drying and Freeze-Drying

    Directory of Open Access Journals (Sweden)

    Stephenie Wong

    2010-04-01

    Full Text Available Viability of Bifidobacterium pseudocatenulatum G4 following spray-drying and freeze-drying in skim milk was evaluated. After spray-drying, the strain experienced over 99% loss in viability regardless of the air outlet temperature (75 and 85 °C and the heat-adaptation temperature (45 and 65 °C, 30 min. The use of heat-adaptation treatment to improve the thermotolerance of this strain was ineffective. On the other hand, the strain showed a superior survival at 71.65%–82.07% after freeze-drying. Viable populations of 9.319–9.487 log10 cfu/g were obtained when different combinations of skim milk and sugar were used as cryoprotectant. However, the addition of sugars did not result in increased survival during the freeze-drying process. Hence, 10% (w/v skim milk alone is recommended as a suitable protectant and drying medium for this strain. The residual moisture content obtained was 4.41% ± 0.44%.

  16. PAI-1 gene: pharmacogenetic association of 4G/4G genotype with bleeding after cardiac surgery--pilot study.

    Science.gov (United States)

    Sirgo, Gonzalo; Morales, Pablo; Rello, Jordi

    2009-05-01

    To investigate whether the 4G/4G genotype of the PAI-1 gene is associated with bleeding after cardiac surgery and whether it may influence the use of antifibrinolytic drugs. After a case-control association study to compare the distribution of genotypes of the 4G/5G polymorphism of the PAI-1 gene (4G/4G, 4G/5G and 5G/5G) between cardiac surgery patients (n = 260) and nonhospitalized age-matched controls (n = 111), we have evaluated the possible association of genotype homozygous 4G/4G (considered procoagulant) in two cohorts of cardiac surgery patients (treated with aprotinin or tranexamic acid) with postoperative bleeding and transfusion requirements. Chest tube output was measured at 6 h and 24 h and then total blood output. Genotypes were typed using restriction fragment length polymorphism analysis. The PAI-1 4G/4G genotype was not associated with bleeding except in the subgroup of patients treated with aprotinin in whom blood loss was significantly lower than in nonhomozygous 4G/4G patients at 6 h and 24 h [mean 135.9 ml (SD 101.8 ml) vs. mean 227.6 ml (SD 218.2 ml), P bleeding in the general patient population. The 4G/4G genotype of the PAI-1 gene was associated with less bleeding after cardiac surgery only in the subgroup of patients treated with aprotinin.

  17. 超声诊断IgG4相关疾病的应用研究进展

    Institute of Scientific and Technical Information of China (English)

    周洁莹; 唐杰

    2012-01-01

    @@ IgG4相关疾病(immunoglobulin G4-related disease,IgG4-RD)是近10年来新发现的一种疾病实体,其主要特征为多克隆性IgG4阳性浆液细胞浸润全身多系统,导致全身多脏器纤维化、硬化等改变.该类疾病经激素治疗后效果良好甚至可以完全缓解.由于长期以来临床上对此类疾病认识不足,常常误诊为恶性病变导致不必要的手术,因此准确的诊断此类疾病有较大临床意义. 形态学信息是诊断IgG4相关疾病的必要条件,超声检查因具有简捷、灵活、费用低等优势,常作为首选的影像学方法为临床提供诊断信息.部分IgG4相关疾病可以通过特异性的超声表现明确诊断,超声引导下穿刺活检及IgG4染色对鉴别诊断IgG4相关疾病与恶性病变有较大价值.本文对IgG4相关疾病超声诊断价值及超声表现作一综述.

  18. Clinical and Pathological Features of IgG4-related Sclerosing Pachymeningitis%IgG4相关性硬化性脑膜炎临床及病理特点(附8例报告)

    Institute of Scientific and Technical Information of China (English)

    袁松英; 朱德茂; 文秋元

    2011-01-01

    [目的]探讨IgG4相关性硬化性脑膜炎的临床及病理特点.[方法]对8例IgG4相关性硬化性脑膜炎患者的临床资料结合形态特点以及IgG4表达阳性细胞进行分析.[结果]8例均表现头痛,各种脑神经受累6例、癫痫3例、呕吐2例.CT及MRI显示病变部位脑膜增厚,增强明显强化.血清学IgG4增高(2/2).病理表现为淋巴-浆细胞炎症反应,纤维化和闭塞性静脉炎,病变可弥漫或局限形成局部肿块.本组病例中3例IgG4阳性(IgG4+)浆细胞数20~30个/HPF,5例高于50个/HPF,IgG4+浆细胞数占IgG+细胞总数的40%以上.[结论]IgG4相关性硬化性脑膜炎是一种与自身免疫相关的疾病,可单发或伴有其他全身性病变.诊断有赖于病理组织学检查.%[Objective] To explore the clinical and pathological features of IgG4-related sclerosing pachymenin-gitis. [Methods]The clinical data, morphology features and IgG4-positive cells in 8 cases of IgG4-related sclerosing pachymeningitis were analyzed. [Results] All 8 patients had headache. There were 6 cases of various kinds of cerebral nerve involvement, 3 cases of epilepsy and 2 cases of vomiting. CT and MRI revealed meningeal thickening and obvious enhancement in lesions. Serum IgG4 increased. Pathological manifestation was lymphoplasmacytic inflammatory response, fibrosis and obstructive phlebitis. The lesions were suffused or localized into local mass. The number of IgG4 + plasma cells in 3 cases was 20 - 30/HPF and that in 5 cases was more than 50/HPF. The number of IgG4 + plasma cells occupied more than 40% of IgG+ cells. [Conclusion] IgG4 -related sclerosing pachymeningitis is an autoimmune-associated disease, which may be single or complicated with other systemic diseases. The diagnosis of IgG4 -related sclerosing pachymeningitis depends on histopathologic examination.

  19. Clinical analysis of 36 cases of IgG4-related disease%36例IgG4相关性疾病的临床分析

    Institute of Scientific and Technical Information of China (English)

    张伟; 薛峰; 刘孟春; 李红; 时维平; 邵乐平; 刘丽秋

    2016-01-01

    目的 总结IgG4相关性疾病(IgG4-RD)的临床特点,提高认识,做到早期诊断及合理治疗.方法 对在本院住院期间通过病理及临床特征确诊的36例IgG4-RD患者的临床资料进行回顾性分析,并使用IgG4-RD应答指数(RI)进行评估.结果 36例患者中男性30例,女性6例,平均发病年龄65.1岁.36例患者均存在活动性病变,其中颌下腺、淋巴结、腹膜后组织是本组病例最常见受累器官.77.7%患者血清IgG4明显升高,44.4%患者血清补体C3明显降低.血清IgG4升高组与正常组相比,RI值更高、受累器官更多(P<0.01).血清IgG4浓度与RI明显相关(r=0.737,P<0.01).累及肾脏患者低补体血症发生率高于累及其他器官患者(P<0.01).28例患者使用糖皮质激素治疗,治疗后RI值、IgG4浓度均明显降低(P<0.05).结论 IgG4-RD RI和IgG4浓度均可作为IgG4-RD病情活动度和治疗疗效的评估指标;IgG4-RD的诊断应以病理为基础,联合临床特点综合分析.%Objective To explore the clinical characteristics of IgG4-related disease (IgG4-RD) in Chinese by detailed clinicopathological and laboratory assessments.Methods The baseline features of 36 patients with biopsy-proven disease were reviewed.The diagnosis was confirmed by pathology review according to consensus diagnostic criteria and clinicopathologic correlation.Disease activity and damage were assessed by the IgG4-RD responder index (RI).Results Thirty (83.3%) of the patients were male,while six were female,and the average age of onset was 65.1 years.All of the 36 patients had active disease,in which submandibular gland,lymph nodes,retroperitoneal tissue were the most common affected organs in this group of patients.Among 36 patients,77.7% had elevated serum IgG4 concentrations and 44.4% had hypocomplementemia.Patients with elevated serum IgG4 had a higher RI,a greater number of organs involved (P < 0.01 for all comparisons).The correlation between serum IgG4 level

  20. Expression of IgG4 in Rosai-Dorfman disease and its significance%IgG4在 Rosai-Dorfman 病组织中的表达及意义

    Institute of Scientific and Technical Information of China (English)

    王秋鹏; 甘梅富; 翁寿向; 周涛; 郑海红; 陈汉松; 谢彩萍

    2015-01-01

    目的:探讨Rosai-Dorfman病病变组织免疫球蛋白G4(IgG4)阳性浆细胞表达情况及其与IgG4相关硬化性疾病( IgG4-SD)的相关性。方法收集来源于9例Rosai-Dorfman病患者12处病变组织(其中11处淋巴结外,1处淋巴结内)的临床及病理组织学资料,光镜下观察病变组织纤维化程度及闭塞性静脉炎,并采用免疫组织化学EnVision二步法标记其病变组织中IgG4阳性及IgG阳性的浆细胞,用医学图像分析系统定量分析IgG4阳性和IgG阳性的浆细胞含量。结果9处病变组织中有不同程度纤维化(轻度4处,中度1处,重度4处),2处病变组织中见闭塞性静脉炎。12处病变组织中IgG4阳性浆细胞浸润程度如下:重度(>50/HPF )4处,中度[(30~50)/HPF ]2处,轻度[(10~29)/HPF]3处,阴性(<10/HPF)3处,经统计学分析各组间差异有统计学意义(P<0.01),其中3处病变组织符合IgG4-SD组织学诊断标准,即IgG4阳性浆细胞>50/HPF、IgG4阳性/IgG阳性浆细胞>40%,包括左面部皮肤1处、左眼眶外上方1处、右腮腺1处。结论 Rosai-Dorfman病中部分病例符合IgG4-SD IgG4阳性浆细胞的诊断标准,并具有IgG4-SD的病理学组织特征,可能为IgG4-SD谱系中的一员,检测Rosai-Dorfman病组织中IgG4阳性浆细胞可能具有一定的临床病理意义。%Objective To study the prevalence of IgG 4-positive plasma cells in Rosai-Dorfman disease and to assess the association between Rosai-Dorfman disease and IgG4-related sclerosing disease (IgG4-SD) .Methods The clinicopathologic features of 12 tissue samples of Rosai-Dorfman disease (11 extranodal and one nodal) from nine patients were reviewed.The degree of fibrosis and occlusive phlebitis was studied by HE staining .The expression of IgG4 and IgG in plasma cells were studied by immunohistochemistry ( EnVision ) and quantitatively analyzed by medical

  1. Steroid-responsive IgG4-related disease with isolated prostatic involvement: An unusual presentation with elevated serum PSA

    Directory of Open Access Journals (Sweden)

    Vikas Jain

    2016-01-01

    Full Text Available Autoimmune prostatitis is known to occur as a part of multisystem fibro-inflammatory disorder known as IgG4 related disease (IgG4 RD. The usual presentation is with symptoms of gastro-intestinal disease with prostatic involvement presenting as lower urinary tract symptoms. The disease responds to corticosteroids. We report an asymptomatic young man who was diagnosed to have IgG4 related prostatitis on TRUS-guided prostate biopsy done for elevated serum PSA, in the absence of any other systemic involvement. The treatment with steroid resulted in normalization of S PSA levels.

  2. 用U盘启动G4L备份还原linux系统%Using Usb Start the Application of G4L to Back-up Copy and Restore Linux System

    Institute of Scientific and Technical Information of China (English)

    白万清

    2011-01-01

    人们用Ghost备份还原Windows操作系统由来已久,版本较多,而且应用得心应手。Linux也有其备份还原工具,G4L就是其中的一种。现在的版本是V0.38,主要是光盘版,需要刻录成光盘来运行。本文介绍如何制作G4LU盘,用u盘启动G4L完成Linux系统的备份还原的操作方法。%Ghost for Back-up Copying windows system has been applied widely and perfectly in many different versions.G4L is one of Back-up Copying And Restoring in Linux System.V 0.38 mainly CD,requiring to be recorded CD.This paper introduces how to make G4L USB,start application of G4L by USB,complete the operation of Back-up Copying And Restoring in Linux System.

  3. Clinical analysis of 9 cases of IgG4-associated biliary and pancreatic diseases%IgG4相关胆胰疾病九例临床分析

    Institute of Scientific and Technical Information of China (English)

    姚仁玲; 徐桂芳; 吕瑛; 于成功; 邹晓平

    2015-01-01

    Objective To analyze the clinical features of patients with IgG4-associated biliary and pancreatic diseases,and to improve the understanding of these diseases.Methods Nine cases of patients with IgG4-associated pancreatitis and cholangitis were analyzed retrospectively,which included clinical manifestations,serological examination,imaging test,pathology,treatment and prognosis.Results Of the 9 patients,8 were male,1 was female,the average age was 61 years old.Four cases were presented with jaundice,2 cases with jaundice and abdominal pain,2 cases with abdominal pain,and 1 case with diabetes.Serum IgG4 levels were 3.46-21.3 g/L (mean 9.743 g/L) in 6 cases,and which were higher than normal value,3 patients did not undergo blood test of IgG4.Nineteen auto-antibodies (including ANCA) were all negative in 9 cases.CA19-9 was increased in 4 cases.CT manifestations showed 5 cases with low density pancreas mass (2 cases with biliary tract dilatation);1 case had pancreas swelling with dilatation of bile duct,and 2 cases with bile duct dilatation only,and CT findings in 1 case were negative.Four patients underwent EUS-FNA,and EUS features included hypoechoic lesions without peripancreatic lymph nodes.FNA results indicated 2 cases with IgG4 related chronic inflammation,2 cases with chronic inflammation with negative IgG4.Seven cases were confirmed to have IgG4 related pancreatitis,and 2 cases with IgG4 related cholangitis.Six patients received glucocorticoid treatment,and the dose ranged from 8-40 mg;3 patients underwent surgery and the surgical pathology indicated IgG4 positive plasma cell.Follow-up showed the serum IgG4 returned to normal,clinical symptoms improved remarkably,and pancreatic mass decreased.Conclusions IgG4 positive plasma cell infiltration is the main feature of IgG4-associated cholangitis and pancreatitis.The diagnosis should combine image,serology with pathology.Glucocorticoid is an effective treatment.%目的 分析IgG4相关胆胰疾病患者的临床特

  4. Importance should be attached to IgG4-related autoimmune diseases%应重视IgG4相关性自身免疫性疾病

    Institute of Scientific and Technical Information of China (English)

    李坤鹏; 黄烽

    2012-01-01

    @@ IgG4相关性疾病(IgG4-related disease)是新近被重新认识的一种自身免疫性、纤维炎症性疾病,特征包括肿瘤样增生、席纹状纤维化、大量IgG4阳性淋巴细胞浆细胞浸润、血清IgG4 水平显著升高以及糖皮质激素治疗反应良好.既往认为独立的疾病都可能是IgG4相关性疾病中某一器官的表现,如米库利兹综合征、慢性硬化唾液腺炎、Riedel甲状腺炎都有可能划分至IgG4 相关性疾病谱中.

  5. 如何打造中国联通3G/4G一体化精品网络%How to Build China Unicom 3G/4G Integrated Network of Quality

    Institute of Scientific and Technical Information of China (English)

    李萌; 闫震; 门里

    2015-01-01

    It analyses 3G/4G network of quality from the aspects of user and owner,explains 3G/4G integrated network of quality from 7 dimensions. Final y,for China Unicom,how to do the 7 dimensions wel ,it gives the macro analysis from the aspects of net-work construction scheme,coverage means,user perception,3G/4G interoperation,LTE voice solution,intel igent channel, power saving measures,network robustness,big data construction and the function of big data platform.%首先从使用者和拥有者的角度对3G/4G精品网进行了解读,然后通过7个维度对什么是3G/4G一体化精品网络进行了解释,最后对中国联通应该如何做好这7个维度从网络建设策略、覆盖手段、用户感知、3G/4G互操作、LTE语音解决演进、智能管道、节电措施、网络健壮性、大数据建设、大数据平台的作用等方面进行了宏观分析。

  6. IgG4-related disease:a retrospective analysis of 20 cases%IgG4相关性疾病20例临床特征分析

    Institute of Scientific and Technical Information of China (English)

    李坤鹏; 朱剑; 赵伟; 邓小虎; 张江林; 黄烽

    2012-01-01

    目的 总结IgG4相关性疾病临床特征及预后,提高认识,做到早期诊断及合理治疗.方法 对在我院住院明确诊断的20例IgG4相关性疾病患者资料进行回顾性分析.结果 20例患者中男性14例,女性6例,平均年龄58岁,胰腺和胆管为最常见受累器官,17例患者(85%)有≥2个器官受累.所有患者血清IgG4均明显升高(>1350 mg/L).12例患者行组织病理学检查,主要表现为组织弥漫性纤维化,伴有大量淋巴细胞、浆细胞浸润,免疫组织化学可见CD38阳性浆细胞浸润,IgG4阳性浆细胞浸润,IgG4阳性浆细胞占IgG阳性浆细胞比例均>40%.20例患者中,除1例患者死于急性化脓性胆管炎及1例未治疗,其余患者病情均明显缓解.结论 IgG4相关性疾病临床症状无特异性,血清IgG4可作为疑似病例的首选检查方法,及时应用糖皮质激素可迅速缓解病情.%Objective To understand the features of immunoglobulin G4-related diseases and to improve its diagnosis and treatment.Methods The clinical data of 20 cases discharged from our hospital were analyzed retrospectively.Results Twenty cases with IgG4 related diseases were reviewed retrospectively.IgG4-related diseasesis more prevalent in male than in female (14∶6),and the mean age was 58 years.Pancreas and bile ducts were the most commonly affected organs,2 and more than 2 organs were involved in 17 patients.All patients had significantly elevated serum immunoglobulin G4.Histopathological findings included diffuse tissue fibrosis with extensive lymphoplasmacytes infiltration.On immunohistochemical staining,the ratio of IgG4-positive plasma cells to IgG-positive plasma cells was higher than 40%.One patient deceased and one patient was left untreated,other patients responded well to the treatment.Conclusion There is no characteristic clinical symptoms for IgG4-related diseases,and serum immunoglobulin G4 is the essential diagnostic method in suspected cases.IgG4-related

  7. Arctic cryosphere response in the Geoengineering Model Intercomparison Project G3 and G4 scenarios

    Science.gov (United States)

    Berdahl, Mira; Robock, Alan; Ji, Duoying; Moore, John C.; Jones, Andy; Kravitz, Ben; Watanabe, Shingo

    2014-02-01

    We analyzed output from the Geoengineering Model Intercomparison Project for the two most "realistic" scenarios, which use the representative concentration pathway of 4.5 Wm-2 by 2100 (RCP4.5) as the control run and inject sulfate aerosol precursors into the stratosphere. The first experiment, G3, is specified to keep RCP4.5 top of atmosphere net radiation at 2020 values by injection of sulfate aerosols, and the second, G4, injects 5 Tg SO2 per year. We ask whether geoengineering by injection of sulfate aerosols into the lower stratosphere from the years 2020 to 2070 is able to prevent the demise of Northern Hemispere minimum annual sea ice extent or slow spring Northern Hemispere snow cover loss. We show that in all available models, despite geoengineering efforts, September sea ice extents still decrease from 2020 to 2070, although not as quickly as in RCP4.5. In two of five models, total September ice loss occurs before 2060. Spring snow extent is increased from 2020 to 2070 compared to RCP4.5 although there is still a negative trend in 3 of 4 models. Because of the climate system lag in responding to the existing radiative forcing, to stop Arctic sea ice and snow from continuing to melt, the imposed forcing would have to be large enough to also counteract the existing radiative imbalance. After the cessation of sulfate aerosol injection in 2070, the climate system rebounds to the warmer RCP4.5 state quickly, and thus, any sea ice or snow retention as a result of geoengineering is lost within a decade.

  8. 75 FR 1009 - Termination of Ineligible Status and Statutory Debarment Pursuant to Section 38(g)(4) of the Arms...

    Science.gov (United States)

    2010-01-07

    ....k.a. Earlene Larson Christenson; Earlene Larson) ACTION: Notice. SUMMARY: Notice is hereby given... Christenson (a.k.a. Earlene Larson Christenson; Earlene Larson), pursuant to section 38(g)(4) of the...

  9. Immunoglobulin G4-related sclerosing disease manifesting as bilateral tonsillar hypertrophy on MRI images: A case report

    Energy Technology Data Exchange (ETDEWEB)

    Park, Mee Hyun; Woo, Ji Young; Lee, Yul; Yoon, Dae Young; Hong, Hye Sook; Hong, Min Eui [Hallym University College of Medicine, Kangnam Sacred Heart Hospital, Seoul (Korea, Republic of)

    2016-02-15

    Immunoglobulin G4-related sclerosing disease (IgG4-SD) is currently recognized as a distinct systemic disease involving various organs. We reported the imaging findings of a case of pathologically confirmed IgG4-SD involving bilateral palatine tonsils. CT and MRI showed diffuse enlargement of both palatine tonsils with homogeneous contrast enhancement. Focal contour bulging was noted in the right palatine tonsil. Lesions appeared as isointense on T1-weighted and slightly hyperintense on T2-weighted MRI images, as compared with muscle. The T2-weighted MRI image showed a striated pattern in both tonsils. Despite its rare occurrence, IgG4-SD should be included in the differential diagnoses of patients with symptomatic bilateral tonsillar hypertrophy that is non-responsive to medication.

  10. [Expression of human IL-35-IgG4 (Fc) fusion protein in CHO/DG44 cells].

    Science.gov (United States)

    Tang, Jing; Gao, Wenda; Zhang, Qing; Zhang, Dawei; Chen, Yang; He, Bo; Liu, Quansheng

    2009-01-01

    We constructed the eukaryotic expression vector of human IL-35-IgG4 (Fc)-pOptiVEC-TOPO by gene recombination technique and expressed the fusion protein human IL-35-IgG4 (Fc) in CHO/DG44 cells. The two components of the newly discovered cytokine human IL-35, EBI3 and IL-12p35, were amplified by PCR from the cDNA library derived from the KG-I cells after LPS induction. The two PCR-amplified cDNA fragments of human IL-35 were linked by over-lapping PCR and then cloned into the IgG4 (Fc)-pOptiVEC-TOPO vector. The constructed plasmid with the recombinant cDNA IL-35-IgG4 (Fc) was verified by restriction enzyme digestion analysis, PCR and DNA sequencing. The verified plasmid with the recombinant cDNA was transfected into CHO/DG44 cells using Lipofectamine 2000. The success of the transfection was examined and confirmed by RT-PCR. After selection in alpha-MEM (-) medium, the IL-35-Ig G4 (Fc) positive CHO/DG44 clones were chosen and the media from these positive clones were collected to be used to purify the fusion protein. The positive CHO/DG44 clones were further cultured in increasing concentrations of MTX and the expression levels of the fusion protein IL-35-Ig G4 (Fc) were repetitively induced by MTX-induced gene amplification. The IL-35-IgG4 (Fc) fusion protein was purified from the media collected from the positive CHO/DG44 clones by protein G affinity chromatography and then identified by SDS-PAGE and Western blotting. The results showed that one protein band was found to match well with the predicted relative molecular mass of human IL-35-IgG4 (Fc) and this protein could specifically bind to anti-human IgG4 (Fc) monoclonal antibody. In conclusion, our study successfully established an IL-35-IgG4 (Fc) positive DG44 cell line which could stably express IL-35-IgG4 (Fc) fusion protein.

  11. Screening of a chemical library by HT-G4-FID for discovery of selective G-quadruplex binders.

    Science.gov (United States)

    Largy, Eric; Saettel, Nicolas; Hamon, Florian; Dubruille, Sylvie; Teulade-Fichou, Marie-Paule

    2012-01-01

    Due to the lack of structural guidelines about G-quadruplex ligands, rational design cannot be the only approach to discover potent G4-ligands. As a complementary approach, screening of chemical library may provide interesting scaffolds known as hits provided that specific tools are available. In this work, the Institut Curie-CNRS chemical library was firstly screened by chemoinformatics methods. Similarity estimations by comparison with reference compounds (Phen-DC3, 360A, MMQ12) provided a set of molecules, which were then evaluated by high-throughput G4-FID (HT-G4-FID) against various G-quadruplex DNA. A full investigation of the most interesting molecules, using the HT-G4-FID assay and molecular modeling, supplied an interesting structure-activity relationship confirming the efficiency of this general approach. Overall, we demonstrated that HT-G4-FID coupled with screening of chemical libraries is a powerful tool to identify new G4-DNA binding scaffolds.

  12. Fluorescence intercalator displacement assay for screening G4 ligands towards a variety of G-quadruplex structures.

    Science.gov (United States)

    Tran, Phong Lan Thao; Largy, Eric; Hamon, Florian; Teulade-Fichou, Marie-Paule; Mergny, Jean-Louis

    2011-08-01

    The potential formation of G-quadruplexes in many regions of the genome makes them an attractive target for drug design. A large number of small molecules synthesized in recent years display an ability to selectively target and stabilize G-quadruplexes. To screen for G4 ligands, we modified a G4-FID (G-quadruplex Fluorescent Intercalator Displacement) assay. This test is based on the displacement of an "on/off" fluorescence probe, Thiazole Orange (TO), from quadruplex or duplex DNA matrices by increasing amounts of a putative ligand. Selectivity measurements can easily be achieved by comparing the ability of the ligand to displace TO from various quadruplex and duplex structures. G4-FID requires neither modified oligonucleotides nor specific equipment and is an isothermal experiment. This test was adapted for high throughput screening onto 96-well plates allowing the comparison of more than twenty different structures. Fifteen different known G4 ligands belonging to different families were tested. Most compounds showed a good G4 vs duplex selectivity but exhibited little, if any, specificity for one quadruplex sequence over the others. The quest for the "perfect" specific G4 ligand is not over yet! Copyright © 2011 Elsevier Masson SAS. All rights reserved.

  13. IgG4相关性疾病的临床及病理学表现%Clinical and Pathologic Characteristics of Immunoglobulin G4 Related Disease

    Institute of Scientific and Technical Information of China (English)

    王咏梅; 杨庆婵(综述); 章明放(审校)

    2016-01-01

    IgG4相关性疾病( IgG4-RD)是一种新被认识的系统性、纤维炎症性疾病,该病以多位点形成肿瘤样脱髓鞘病变、大量IgG4阳性淋巴细胞和浆细胞浸润以及纤维化为特征,常累及胰腺、胆管、唾液腺、眶周组织、淋巴结及腹膜后间隙等。各器官的病理特征相似,诊断该病时,需结合临床表现、实验室、组织病理及影像学等检查结果,并积极排除其他疾病如肿瘤后作出诊断。该病对激素等免疫抑制剂治疗反应良好,预后较好。%IgG4-related disease(IgG4-RD) is a newly recognized systemic fibro-inflammatory disorder characterized with a tendency to form tumefactive lesions in multiple sites ,massive IgG4-bearing lymphocyte and plasma cell infiltration and fibrosis.The most commonly involved the pancreas,the biliary tree,salivary glands,periorbital tissues,lymph nodes,retroperitoneal space and so on.The histopathological features are similar across the involved organs.Combination of the clinical manifestations,laboratory examination,patholog-ic and imaging findings is recommended for diagnosis of IgG4-RD,while other disorders such as malignancy should be excluded to make a final diagnosis.Treatments with corticosteroid and other immunosuppressive drugs are effective and the prognosis is favorable.

  14. IgG4相关性肺疾病1例及文献复习%A review of 1 case of IgG4-related lung disease

    Institute of Scientific and Technical Information of China (English)

    李燕; 黄妹; 蔡后荣; 孙琦; 苗立云; 孟凡青; 肖永龙

    2016-01-01

    目的:提高对IgG4相关性肺疾病的临床特征、胸部影像学和病理组织学的认识。方法对1例经病理证实的IgG4相关性肺疾病的临床资料进行分析,并结合文献进行回顾总结。结果患者男,62岁,以胸腔积液起病,在外院先后行胸膜活检、胸腔镜肺活检等均未能明确诊断,后至我院行CT引导下经皮肺穿刺活检术,结果示活检组织内纤维组织增生伴淋巴细胞、浆细胞等炎症细胞浸润,免疫组化示大量浆细胞(+),IgG4阳性,浆细胞最密集计数约为40个/高倍视野,血清IgG4浓度示4.07 g/L(0.03 g/L~2 g/L)。诊断IgG4相关性肺疾病,给予糖皮质激素治疗,2个月后复查胸部CT示肺部病灶较前局部吸收、好转。结论IgG4相关性疾病是一种累及多器官、以血清IgG4水平升高、组织IgG4阳性浆细胞浸润为特点的淋巴浆细胞病。目前国内IgG4相关性肺疾病的报道很少,报道这一病例并进行文献复习有助于提高对IgG4相关性肺疾病的认识。%Objective To describe the clinical characteristics of IgG4-related lung disease. Methods A case with pathologically diagnosed as IgG4-related lung disease was reviewed. The clinical manifestation, laboratory examination results, chest computed tomography ( CT) characteristics, histopathological features, and treatment of IgG4-related lung disease were summarized through literature reviewing. Results The initial clinical manifestation of a 62-year-old man was pleural effusion. He received pleural biopsy and thoracoscopic lung biopsy successively in oth-er two hospitals where failed to get definite diagnosis. He was admitted to our department afterwards and received per-cutaneous lung puncture biopsy guided by CT. The pathological examination of the biopsy tissue found proliferation of fibrous tissue, with lymphoplasmacytic infiltration. The immunohistochemical staining of lung tissues revealed pre-dominant plasma cells. The highest number of IgG4

  15. IgG4-related systemic diseases: a report of eight cases%IgG4相关系统性疾病八例临床分析

    Institute of Scientific and Technical Information of China (English)

    郑可; 李雪梅; 蔡建芳; 文煜冰

    2012-01-01

    Objective To explore the clinical features of IgG4-related systemic diseases (IgG4-RSD)Methods A total of 8 inpatients with IgG4-RSD diagnosed at our hospital during August 2010 to December 2011 were recruited.We analyzed the clinical data,laboratory profiles,radiological and pathologic features and prognostic factors of these patients with IgG4-RSD.Results There were 5 males and 3 females with a mean onset age of 52.5 years.IgG4-related diseases were described in multiple organ systems:pancreas (n =5),biliary tree (n =3),kidneys (n =6),lungs (n =3),aorta and periaortic tissue,retroperitoneum (n =5) and lymph nodes (n =6).Hyperglobulinemia,elevated serum levels of IgG and IgG4,anemia(n =6),renal dysfunctions(n =5) and obstructive jaundice (n =2) were common laboratory findings.Lymphoplasmacytic infiltration and fibrosis were common pathologic findings.A diffuse infiltration of plasma cells with over 30 IgG4-positive cells per high-power field provided compelling evidences of IgG4-related disease.Patients with IgG4-RSD responded well to glucocorticoids.Conclusion With heterogeneous clinical characteristics,IgG4-RSD is found in various organ systems.The prominent histopathologic features of IgG4-RSD include a diffuse infiltration of plasma cells with over 30 IgG4-positive cells per high-power field.And the therapy of glucocorticoids is efficacious.%目的 总结IgG4相关系统性疾病(IgG4-RSD)的临床特点,提高对该疾病的认识.方法 回顾性分析符合IgG4-RSD诊断的8例患者的临床表现、实验室检查、影像学结果、病理表现和治疗及预后情况.结果 8例IgG4-RSD患者中,男5例,女3例,中位年龄52.5(36 ~72)岁;临床首发表现多样化;受累器官包括胰腺(5例)、胆道(3例)、肾脏(6例)、肺部(3例)、后腹膜和腹主动脉(5例)、淋巴结(6例)等;实验室检查中所有患者均存在高球蛋白血症,IgG及IgG4亚型水平升高,部分患者存在贫血(6例)、肾功能异常(5

  16. g4c2c: A Model for Citizen Engagement at Arms’ Length from Government

    Directory of Open Access Journals (Sweden)

    Axel Bruns

    2011-03-01

    Full Text Available Normal 0 false false false EN-AU X-NONE X-NONE MicrosoftInternetExplorer4 /* Style Definitions */ table.MsoNormalTable {mso-style-name:"Table Normal"; mso-tstyle-rowband-size:0; mso-tstyle-colband-size:0; mso-style-noshow:yes; mso-style-priority:99; mso-style-qformat:yes; mso-style-parent:""; mso-padding-alt:0cm 5.4pt 0cm 5.4pt; mso-para-margin:0cm; mso-para-margin-bottom:.0001pt; mso-pagination:widow-orphan; font-size:11.0pt; font-family:"Calibri","sans-serif"; mso-ascii-font-family:Calibri; mso-ascii-theme-font:minor-latin; mso-fareast-font-family:"Times New Roman"; mso-fareast-theme-font:minor-fareast; mso-hansi-font-family:Calibri; mso-hansi-theme-font:minor-latin; mso-bidi-font-family:"Times New Roman"; mso-bidi-theme-font:minor-bidi;} The recognition that Web 2.0 applications and social media sites will strengthen and improve interaction between governments and citizens has resulted in a global push into new e-democracy or Government 2.0 spaces. These typically follow government-to-citizen (g2c or citizen-to-citizen (c2c models, but both these approaches are problematic: g2c is often concerned more with service delivery to citizens as clients, or exists to make a show of ‘listening to the public’ rather than to genuinely source citizen ideas for government policy, while c2c often takes place without direct government participation and therefore cannot ensure that the outcomes of citizen deliberations are accepted into the government policy-making process. Building on recent examples of Australian Government 2.0 initiatives, we suggest a new approach based on government support for citizen-to-citizen engagement, or g4c2c, as a workable compromise, and suggest that public service broadcasters should play a key role in facilitating this model of citizen engagement.

  17. Purification and characterization of toluene 2-monooxygenase from Burkholderia cepacia G4.

    Science.gov (United States)

    Newman, L M; Wackett, L P

    1995-10-31

    Recent in vivo studies indicate that ring monooxygenation is a widespread mechanism by which bacteria metabolize aromatic hydrocarbons and obtain carbon and energy. In this study, toluene 2-monooxygenase from Burkholderia (formerly Pseudomonas) cepacia G4 was purified to homogeneity and found to be a three-component enzyme system. The reconstituted enzyme system oxidized toluene to o-cresol and o-cresol to 3-methylcatechol, an important intermediate for growth of the bacterium on toluene. Steady-state kinetic parameters measured for the water-soluble substrate o-cresol were a Km of 0.8 microM and a Vmax of 131 nmol min-1 (mg of hydroxylase protein)-1. The three protein components were (1) a 40 kDa polypeptide containing one FAD and a [2Fe2S] cluster, (2) a 10.4 kDa polypeptide that contained no identifiable metals or organic cofactors, and (3) a 211 kDa alpha 2 beta 2 gamma 2 component containing five to six iron atoms. The 40 kDa flavo-iron-sulfur protein oxidized NADH and transferred electrons to cytochrome c, dyes, and the alpha 2 beta 2 gamma 2 component. It is analogous to other NADH oxidoreductase components found in a wide range of bacterial mono- and dioxygenases. The 10.4 kDa component, added to the other two components and NADH, increased toluene oxidation rates 10-fold. The alpha 2 beta 2 gamma 2 component was indicated to contain the site for toluene binding and hydroxylation by the following observations: (1) tight binding to a toluene affinity column; (2) oxidation of toluene after reduction of the protein with dithionite and adding O2; (3) H2O2-dependent toluene oxidation and catalase activity; and (4) spectroscopic studies of the iron atoms in the component. The alpha 2 beta 2 gamma 2 component had no significant absorbance in the visible region. EPR spectroscopy yielded a signal at g = 16 upon addition of > 2 equiv of electrons per 2 Fe atoms. Taken with the quantitation of five to six iron atoms, the data suggest that the alpha 2 beta 2 gamma 2

  18. 9G4 autoreactivity is increased in HIV-infected patients and correlates with HIV broadly neutralizing serum activity.

    Directory of Open Access Journals (Sweden)

    James J Kobie

    Full Text Available The induction of a broadly neutralizing antibody (BNAb response against HIV-1 would be a desirable feature of a protective vaccine. Vaccine strategies thus far have failed to elicit broadly neutralizing antibody responses; however a minority of HIV-infected patients do develop circulating BNAbs, from which several potent broadly neutralizing monoclonal antibodies (mAbs have been isolated. The findings that several BNmAbs exhibit autoreactivity and that autoreactive serum antibodies are observed in some HIV patients have advanced the possibility that enforcement of self-tolerance may contribute to the rarity of BNAbs. To examine the possible breakdown of tolerance in HIV patients, we utilized the 9G4 anti-idiotype antibody system, enabling resolution of both autoreactive VH4-34 gene-expressing B cells and serum antibodies. Compared with healthy controls, HIV patients had significantly elevated 9G4+ serum IgG antibody concentrations and frequencies of 9G4+ B cells, a finding characteristic of systemic lupus erythematosus (SLE patients, both of which positively correlated with HIV viral load. Compared to the global 9G4-IgD--memory B cell population, the 9G4+IgD--memory fraction in HIV patients was dominated by isotype switched IgG+ B cells, but had a more prominent bias toward "IgM only" memory. HIV envelope reactivity was observed both in the 9G4+ serum antibody and 9G4+ B cell population. 9G4+ IgG serum antibody levels positively correlated (r = 0.403, p = 0.0019 with the serum HIV BNAbs. Interestingly, other serum autoantibodies commonly found in SLE (anti-dsDNA, ANA, anti-CL did not correlate with serum HIV BNAbs. 9G4-associated autoreactivity is preferentially expanded in chronic HIV infection as compared to other SLE autoreactivities. Therefore, the 9G4 system provides an effective tool to examine autoreactivity in HIV patients. Our results suggest that the development of HIV BNAbs is not merely a consequence of a general breakdown in

  19. CT,MRI imaging features of IgG4-related disease in the chest and abdominal%IgG4相关性疾病胸腹部 CT、MRI 影像表现

    Institute of Scientific and Technical Information of China (English)

    梁璐; 蒋涛; 彭朋; 李敏; 于洋; 高帅

    2015-01-01

    目的:分析 IgG4相关性疾病(IgG4-RD)的影像特征,提高对 IgG4-RD 的影像诊断水平。方法回顾性分析临床诊治的11例 IgG4-RD 的患者影像资料,包括 CT 增强扫描5例,MRI 增强扫描9例,胸部高分辨 CT 7例,MRCP 4例。结果(1)胰腺受累11例:8例胰腺弥漫性肿大,2例胰头局限性肿大,1例萎缩,4例有封套征,5例胰管呈弥漫性不规则狭窄;(2)胰腺外受累包括:胆管受累6例;肺间质改变2例;双肾受累1例,腹膜后纤维化1例;腹膜后淋巴结肿大1例,胰周淋巴结肿大2例,肝门区淋巴结肿大1例,纵隔淋巴结肿大2例。结论认识 IgG4-RD 多器官受累的影像表现,有助于早期诊断。%Objective To analyse image features of IgG4-related disease and improve its diagnostic accuracy. Methods Retro-spectively review imaging data in 1 1 cases of clinical diagnosed of IgG4-related diseases,including 5 enhanced CT scannings,9 en-hanced MR scannings,7 High-Resolution CT scannings,and 4 MRCPs.Results (1)All the 1 1 patients showed pancreas damage, in which 8 had diffuse enlargement pancreas,2 enlargement of head of pancreas,1 atrophy,4 envelope syndrome,5 irregular stric-ture of pancreatic duct.(2)The imaging findings of extrapancreatic lesions included:6 IgG4-related sclerosis cholangitis,2 pulmona-ry interstitial change,1 both-kidney damage,1 retroperitoneal fibrosis,1 retroperitoneal lymph nodes enlargement,2 peripancreatic lymph nodes enlargement,1 hilar lymph nodes enlargement,2 mediastinal lymph nodes enlargement.Conclusion IgG4-related dis-ease often had multiple organ damage.Recognize the imaging features can help us make diagnosis early.

  20. 眼眶IgG4相关疾病的临床病理学特点及其鉴别诊断%A clinicopathological study of IgG4-related ocular disease and differentiated diagnosis with non-IgG4-related ocular disorders

    Institute of Scientific and Technical Information of China (English)

    吴霞; 罗清礼; 李甘地

    2016-01-01

    目的 探讨眼眶IgG4相关疾病(IgG4-ROD)的临床及组织病理学特点及其与眼眶非IgG4相关疾病(Non-IgG4-ROD)的鉴别诊断.方法 回顾性系列病例研究.收集2008年11月至2014年8月四川大学华西医院病理科的43例患者的临床资料.分析其临床表现、实验室检查、影像学结果、治疗情况及组织病理学特点.根据2014年IgG4-ROD的诊断标准和相关文献报道,本组研究病例分为2组:IgG4-ROD(18例,23份眼眶标本)和Non-IgG4-ROD(25例,31份眼眶标本).男女构成、淋巴滤泡的形成、导管及血管周围的纤维化、眼眶的病变范围采用Pearson x2检验,年龄、免疫组织化学IgG4+/HPF浆细胞数、IgG+/HPF浆细胞数、IgG4+/IgG+浆细胞比值采用两样本比较的t检验,病变累及的结构组织及形态学指标嗜酸性粒细胞和淋巴细胞和浆细胞的浸润采用配对设计的符号秩和检验.结果 两组病例在以下参数中的差异具有统计学意义:IgG4-ROD组每个高倍视野IgG4+浆细胞数(>40个/HPF)(t=12.17,P<0.01),IgG+浆细胞数(>100个/HPF) (t=2.91,P<0.01),IgG4+/IgG+浆细胞比值(≥40%)(t=16.98,P<0.01),淋巴细胞和浆细胞的浸润(F=3.62,P=0.03),导管周围的纤维化(F=4.71,P=0.03),眼眶病变范围(t=1.11,P=0.01).但是在平均年龄(t=1.17,P=0.28),男女构成(t=1.09,P=0.58)的差异无统计学意义.IgG4-ROD需要与眼眶非特异性的炎性病变、淋巴瘤等鉴别.结论 眼眶IgG4相关疾病主要发生在中老年男性,双侧眼眶无痛性肿胀为眼部常见症状,病变组织特点为大量IgG4+浆细胞浸润,并可以累及眼眶多种结构组织,以泪腺为主,但未见典型的闭塞性静脉炎改变.充分结合临床表现、影像学发现、实验室检查和组织病理学改变有助于与Non-IgG4-ROD及其他疾病进行鉴别诊断.%Objective To study the clinicopathologic features of IgG4-related ocular disease and differentiated diagnosis with non-IgG4-related ocular

  1. Recent advances in research of immunoglobulin G4-related autoimmune pancreatits%IgG4相关性自身免疫性胰腺炎的研究进展

    Institute of Scientific and Technical Information of China (English)

    石振东

    2016-01-01

    自身免疫性胰腺炎(autoimmune pancreatits,AIP)是一种少见的慢性胰腺炎,属于免疫球蛋白G4(immunoglobulin G4,IgG4)相关性疾病谱.组织学表现为胰管周围大量淋巴细胞及浆细胞浸润、席纹状纤维化、闭塞性静脉炎和IgG4阳性浆细胞显著增多.临床表现为梗阻性黄疸、复发性胰腺炎,伴胆管、涎腺等其他器官受累,实验室可见血清IgG4明显增高.影像学表现为胰腺“腊肠样”外观,伴主胰管弥漫性不规则狭窄.AIP对糖皮质激素治疗敏感,但容易复发,对疾病复发的患者再次应用激素治疗或联合免疫调节剂治疗是有效的.

  2. The clinicopathological analysis of 4 cases of IgG4-related nonspecific interstitial pneumonia%IgG4相关非特异性间质性肺炎四例临床病理分析

    Institute of Scientific and Technical Information of China (English)

    张卉; 施举红; 冯瑞娥; 田欣伦; 徐作军; 许文兵; 刘鸿瑞; 刘彤华

    2012-01-01

    Objectives To observe the immunohistochemical staining of IgG4 in nonspecific interstitial pneumonia (NSIP) and to study the clinicopathological features of IgG4-related NSIP.Methods Retrospective analysis was carried out for 32 patients with NSIP who had been admitted into Peking Union Medical College Hospital from November 2002 to October 2010.The diagnosis of NSIP was established by surgical lung biopsy and all specimens were fixed in neutral formalin and embedded in paraffin.Sections were cut for HE and immunohistochemical stain.According to the diagnostic criteria for IgG4-related disease,4 cases were confirmed to be IgG4-related NSIP.The clinicopathological features including clinical history,laboratory examination,and pathologic evaluation were studied.Results The 4 patients with IgG4-related NSIP included 1 man and 3 women,with a median age of 48 years (range,44-56 years).The presenting symptoms were dry cough or shortness of breath.One patient (1/4,25.0%)was found to have a positive autoantibody but no cases showed positive RF in serum.The histological finding of the 4 cases was characterized by inflammatory cell infiltration in interstitium with fibrosis,and 1 case showed obliterative arteritis.The numbers of IgG4-positive plasma cells in the 4 cases were 42/hpf,22/hpf,11/hpf,and 33/hpf respectively,while the percentages of IgG4-positive to IgG-positive plasma cells were 70%,71%,57%,43% respectively.Conclusions IgG4-related interstitial pulmonary disease can be characterized as the NSIP pattern.The pathological features of IgG4-related NSIP include infiltration of lymphoplasmacytes and eosinophils in interstitium with fibrosis,and lymphoid follicles are frequently identified in the area of lymphocyte aggregation,but obliterative arteritis is infrequently identified in the lesion.Immunohistochemical staining of IgG and IgG4 is very helpful for a definite diagnosis of IgG4-related disease.%目的 观察非特异性间质性肺炎(NSIP)病变组织中IgG

  3. IgG4-related lung disease: a case report and review of the literature%IgG4相关性肺疾病一例并文献复习

    Institute of Scientific and Technical Information of China (English)

    刘涌; 孙永昌; 冯瑞娥; 刘晓芳; 刘广杰; 刘红刚

    2012-01-01

    目的 分析1例IgG4相关性肺疾病的临床资料并进行文献复习,探讨该病的诊断及治疗经验.方法 分析北京同仁医院呼吸科收治的1例IgG4相关性肺疾病患者的临床表现、实验室检查结果、影像学及组织病理学资料.以“IgG4相关疾病”和“肺”为关键词,自Pubmed共计检索到IgG4相关性肺疾病65例,对呼吸系统症状、肺外器官受累情况、血清IgG4水平、影像学表现及组织标本中IgG4阳性浆细胞数量以及糖皮质激素治疗反应等进行分析.结果 患者41岁,男,体检时X线朐片发现肺部阴影,入院后查胸部CT显示以双侧中肺野中内带为主的磨玻璃影,伴有纤维条索影及蜂窝样改变.经胸腔镜肺活检病理显示肺泡间隔及支气管血管束周围大量炎症细胞浸润,主要为淋巴浆细胞,并伴有胶原纤维沉积,肺泡间隔增厚,可见闭塞性静脉炎,免疫组织化学染色可见大量IgG4阳性浆细胞.血清IgG4明显升高(3.07 g/L).诊断IgG4相关性肺疾病,给予泼尼松口服治疗,4个月后复查胸部CT示磨玻璃影基本吸收,仪残余纤维条索影及蜂窝样改变,IgG4水平降至1.99 g/L.文献检索到IgG4相关性肺疾病共65例,其中单纯肺部受累者27例,合并肺外表现者38例,其中累及胰腺者21例.36例测定血清IgG4水平,其中34例高于正常.影像学分类显示实性结节型占55.4% (36/65),肺泡间质型占26.2%(17/65),支气管血管束型占13.8% (9/65),圆形磨玻璃影型占4.6%(3/65).应用糖皮质激素治疗者23例,疗效良好,仅1例治疗失败.结论 IgG4相关性肺疾病是一少见疾病,临床易于忽视,可与其他硬化性疾病并发,也可单发,诊断有赖于血清IgG4水平测定及组织病理学检查,糖皮质激素治疗有效,预后良好.%Objective To describe the clinical features of IgG4-related lung disease.Method The clinical symptoms,laboratory tests,radiographic patterns,histopathological features and

  4. Pseudotumors due to IgG4 immune-complex tubulointerstitial nephritis associated with autoimmune pancreatocentric disease.

    Science.gov (United States)

    Cornell, Lynn D; Chicano, Sonia L; Deshpande, Vikram; Collins, A Bernard; Selig, Martin K; Lauwers, Gregory Y; Barisoni, Laura; Colvin, Robert B

    2007-10-01

    Autoimmune pancreatitis (AIP) is a mass-forming chronic fibroinflammatory condition centered on the pancreatobiliary system and characterized by predominant immunoglobulin G4 (IgG4)-positive plasma cells. Recent reports have brought to light the multiorgan involvement of this disease. We describe a series of 5 cases of tubulointerstitial nephritis (TIN) associated with AIP and characterize the clinical, pathologic, ultrastructural, and immunopathologic features of TIN. The specimens consisted of 4 biopsies and 1 nephrectomy. The average patient age was 64 years (range 45 to 78) and the male to female ratio was 4:1. All had histologic and/or clinical and radiographic evidence of AIP, mass-forming sclerosing cholangitis, or both. The clinical impression in 4 patients was a renal mass or vasculitis. Two patients had renal insufficiency. Histologic preparations revealed a dense tubulointerstitial lymphoplasmacytic infiltrate. Eosinophils were often numerous. Tubulitis and tubular injury were present, along with tubular atrophy with focally thickened tubular basement membranes (TBMs). The histologic appearance ranged from a cellular, inflammatory pattern without tubular atrophy to a striking expansive interstitial fibrosis with tubular destruction. The nephrectomy specimen demonstrated a masslike nodular pattern of inflammation with normal renal tissue elsewhere. Glomeruli were uninvolved. By immunohistochemistry or immunofluorescence, numerous plasma cells in the infiltrate were positive for IgG4. TBM granular IgG deposits, predominantly of the IgG4 subclass, were detected in 4 of 5 cases by either immunofluorescence or immunohistochemistry. By electron microscopy, corresponding amorphous electron-dense deposits were present in the TBM in these cases. This type of TIN, typically characterized by a masslike lesion consisting of a lymphoplasmacytic infiltrate with eosinophils and prominent IgG4-positive plasma cells and immune-complex deposits in the TBM, may be part of

  5. 累及肾盂的IgG4相关性疾病1例报告并文献复习%Renal pelvis involved IgG4-related disease (A case report and literature review)

    Institute of Scientific and Technical Information of China (English)

    吴兴成; 纪志刚; 严维刚; 毛全宗

    2016-01-01

    目的:分析1例累及肾盂的IgG4相关性疾病的临床资料并进行文献复习,探讨该病的诊断和治疗经验,以提高对IgG4相关性肾盂疾病的认识及诊治水平.方法:分析北京协和医院收治的1例IgG4相关性肾盂疾病患者的临床表现、实验室检查、影像学及组织病理学资料.以“IgG4相关性疾病”和“肾盂”为关键词,自Pubmed共计检索到累及肾盂的IgG4相关性疾病4例,对临床症状、肾盂外器官受累情况、血清IgG4水平、影像学表现和组织学以及治疗等进行分析.结果:患者女性,53岁,B超发现右肾盂占位,无临床不适,体重近半年减重3 kg.尿路CT成像示右肾盂及中下部肾盏团块样软组织密度影.逆行造影未见右肾盂内明确充盈缺损.PET/CT示右肾盂软组织密度影放射性摄取增高.行腹腔镜右肾输尿管全长切除术,病理符合IgG4相关性疾病.血清IgG4明显升高(3 240 mg/L),给以泼尼松口服治疗,3个月后复查IgG4水平降至1 890 mg/L.文献报道IgG4相关性肾盂疾病共4例,合并其他器官受累3例.2例行患侧肾输尿管全长切除,1例行肾切除.应用糖皮质激素治疗3例,疗效良好,1例因合并严重消化道出血死亡.结论:IgG4相关性疾病是一少见疾病,累及肾盂罕见,临床易误诊为肾盂癌而行不必要的手术切除,可同时累及其他器官,诊断有赖于血清IgG4水平测定及组织病理学检查,糖皮质激素治疗有效,预后良好.

  6. Drilling technique practice and recogonition of Gabon G4-188 Block%加蓬G4-188区块钻井技术实践认识

    Institute of Scientific and Technical Information of China (English)

    石强

    2008-01-01

    加蓬G4-188区块是一个新的海外勘探区块,在钻井过程中遇到了井壁垮塌、气侵、井斜控制困难等诸多难题,影响了钻井施工的速度.针对这些难题,实施了解决G4-188区块钻井施工难点的各项技术措施,为G4-188区块后续钻井施工提供了有益的技术支持.

  7. Relationship between IgG4 and Connective Tissue Disease with Interstitial Lung Disease%IgG4与结缔组织病合并间质性肺病的相关性研究

    Institute of Scientific and Technical Information of China (English)

    鲁芙爱; 刘媛; 安燕; 王永福

    2013-01-01

    Objective To discuss the significance of IgG4 in the pathogenesis of connective tissue disease with interstitial lung disease and its relationship with activity of the disease. Methods Totally 205 patients with connective tissue diseases were divided into two groups, with 93 with interstitial lung disease and 112 without interstitial lung disease. Serum IgG4 levels of all patients were detected by immune nephelometry quantitative method, and IgG4 level changes between the two groups were compared. At the same time, the levels of ESR, C - reactive protein ( CRP ), IgA, IgG and IgM were detected in 93 patients with interstitial lung disease, and the correlation of the activity of these indices with IgG4 was analyzed. Results No significant difference in serum IgG4 level was found between the connective tissue disease patients with interstitial lung disease and the patients without interstitial lung disease ( P >0. 05 ). And in the patients with interstitial lung disease there was no significant correlation between the level of serum IgG4 and the levels of ESR, CRP, IgM, IgA and IgG ( P >0. 05 ) . Conclusion The IgG4 level is not correlated with connective tissue disease complicated by interstitial lung disease, suggesting that IgG4 - related diseases and connective tissue disease are two independent immune system diseases, and serum IgG4 level cannot be used to determine the activity of connective tissue disease with interstitial lung disease.%目的 探讨IgG4在结缔组织病合并间质性肺病发病中的意义及与其病情活动的关系.方法 选取205例结缔组织病患者,分为两组,其中合并间质性肺病组的患者93例,无间质性肺病组的患者112例.采用动态免疫散射比浊法定量检测两组患者血清中IgG4的水平,比较两组之间IgG4水平的变化;对合并间质性肺病的93例患者同时进行了红细胞沉降率(ESR)、C反应蛋白(CRP)、免疫球蛋白A(IgA)、免疫球蛋白G(IgG)、免疫球蛋白M(IgM)的

  8. 眼眶IgG4相关疾病的临床病理观察%Clinical pathology observation on orbit IgG4 related disease

    Institute of Scientific and Technical Information of China (English)

    郭继华; 田艳明; 马敏丽; 刘莹; 高晓唯

    2015-01-01

    AIM:To discuss clinical pathological features of orbit IgG4 related disease ( IgG4-RD) . METHODS: The clinical pathological materials of 23 patients ( 35 eyes ) with orbit IgG4-RD were collected. They were observed in terms of histology and immunohistochemistry, and its clinical and pathologic characteristics were summarized. RESULTS:There were 23 patients (35 eyes) with orbit IgG4-RD (8 male patients, 9 eyes;15 female patients, 26 eyes), with an average age of 52. 1 year-old (from age 28 to 72). 19 patients (30 eyes) occured in lacrimal gland and 4 cases (5 eyes) in other places, and they went to hospital for lacrimal gland cyst or exophthalmos. There were 11 cases in one side and 12 cases in both sides. The disease lasted from 1mo to 10a, averaging 27mo. It recureded in one patient (1 eye) after 1mo. In general inspection: Gray nodular goiter, thin fibrous coat wrapping around the lacrimal gland could be observed. Histologic characteristics: lacrimal gland bubble and catheter group shrinked or even disappeared, substituted by lymphocyte, plasma cells and lymphoid follicle and accompanied with fibrosis. Immunohistochemical staining:IgG4 positive plasma cells of 23 cases (35 eyes) was >50/HPF, and IgG4/IgG ratio of positive plasma cells was >40%. CONCLUSION: Orbit IgG4 - RD mainly occures in lacrimal gland tissue, and expression of IgG4 can be detected through histologic characteristics and immunohistochemical staining. IgG4 - RD should be screened, prevented and treated in the early phase.%目的:探讨眼眶 IgG4相关疾病( IgG4-RD )的临床病理特点。  方法:收集整理23例35眼眼眶IgG4-RD患者的临床病理资料,对其进行组织学和免疫组织化学观察,总结其临床和病理特点。  结果:眼眶IgG4-RD患者23例35眼,其中男8例9眼,女15例26眼,年龄28~72(平均52.1)岁。19例30眼来源于泪腺,4例5眼来源于眶内其他部位。以泪腺区肿胀或眼球突出就诊。单侧11例,双侧12例。病程1 mo

  9. 2个芹菜品种泛变应原Api g 4基因的克隆与分析%Cloning and analysis of panallergen Api g 4 gene from two cultivars of Apium graveolens

    Institute of Scientific and Technical Information of China (English)

    李梦瑶; 王枫; 侯喜林; 蒋倩; 马静; 熊爱生

    2013-01-01

    从芹菜(Apium graveolens Linn.)品种‘津南实芹’(‘Jinnanshiqin’)和‘美国西芹’(‘Meiguoxiqin’)中分别克隆获得泛变应原基因Api g 4;2个品种的Api g4基因均包含1个长度为405 bp的开放阅读框,二者间有3个核苷酸位点的差异.2个品种的Api g4基因均能编码134个氨基酸,但二者编码的氨基酸序列有2个位点的差异.多重比对以及进化树分析结果均表明:2个芹菜品种Api g 4基因编码的氨基酸序列与其他植物的泛变应原氨基酸序列同源性较高,氨基酸序列高度保守;与同科植物欧芹[Petroselinum crispum (Miller) Nyman ex A.W.Hill]和胡萝卜(Daucus carota Linn.)的泛变应原氨基酸序列的同源性均达到90%以上,在进化树上也归为同一支.2个品种的泛变应原Api g 4均为疏水性蛋白,具有相似的三维空间结构,均包含3个α螺旋和7个β折叠.实时定量PCR分析结果显示:Api g 4基因在‘津南实芹’和‘美国西芹’根中的表达水平均最高,在茎和茎尖分生组织中的表达水平相对较低,在叶中的表达水平很弱,且2个品种间同一组织的Api g4基因表达水平也有差异,表明Api g 4基因的表达具有明显的组织特异性.

  10. Usefulness of biopsying the major duodenal papilla to diagnose autoimmune pancreatitis: A prospective study using TgG4-immunostaining

    Institute of Scientific and Technical Information of China (English)

    Terumi Kamisawa; Yuyang Tu; Hitoshi Nakajima; Naoto Egawa; Kouji Tsuruta; Atsutake Okamoto

    2006-01-01

    AIM: To examine the histological and immunohistochemical findings of biopsy specimens taken from the major duodenal papilla of autoirnrnune pancreatitis (AIP)patients.METHODS: The major duodenal papilla in the resected pancreas of 3 patients with AIP and of 5 control patients [pancreatic carcinoma (n = 3) and chronic alcoholic pancreatitis (n = 2)] was irnrnunostained using anti-CD4-T cell, CD8-T cell and IgG4 antibodies. Forceps biopsy specimens taken from the major duodenal papilla of 2patients with AIP and 5 control patients with suspected papillitis were prospectively taken during duodenoscopy and immunohistochernically examined.RESULTS: Moderate or severe lyrnphoplasrnacytic infiltration including many CD4-positive or CD8-positive T lymphocytes and IgG4-positive plasma cells (≥10/HPF),was observed in the major duodenal papilla of all 3 patients with AIP. The same findings were also detected in the biopsy specimens taken from the major duodenal papilla of 2 patients with AIP, but in controls, there were only a few (≤3/HPF) IgG4-positive plasma cells infiltrating the major duodenal papilla.CONCLUSIONS: An abundant infiltration of IgG4-positive plasma cells is specifically detected in the major duodenal papilla of patients with AIP. Although this is a preliminary study, IgG4-irnmunostaining of biopsy specimens taken from the major duodenal papilla may support the diagnosis of AIP.

  11. A Case Report of an Atypical Presentation of IgG4-Related Disease and Idiopathic CD4 Lymphocytopenia

    Directory of Open Access Journals (Sweden)

    Francesco Rapisarda

    2015-01-01

    Full Text Available The IgG4-related disease is a fibroinflammatory disease characterized by tumefactive lesions, a dense lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells, storiform fibrosis, and, often but not always, elevated serum levels of IgG4. Idiopathic CD4 lymphocytopenia is a heterogenic and rare syndrome characterized by the detection of a persistent absolute CD4 T cells count <300 cells/mm3 (or <20% of total T cells in more than one occasion and no evidence of HIV infection in absence of immunodeficiency or therapy associated with depressed levels of CD4 T cells. We report the case of a 50-year-old man with a multiorgan IgG4-related disease presenting in a temporal association with a profound and symptomatic idiopathic CD4 lymphocytopenia. Both clinical pictures improved after steroid treatment. Idiopathic CD4 lymphocytopenia has been associated with a number of autoimmune conditions but, to the best of our knowledge, this is the first case in which an association with the IgG4-related disease is reported.

  12. Natural Mosquito-Pathogen Hybrid IgG4 Antibodies in Vector-Borne Diseases: A Hypothesis

    Science.gov (United States)

    Londono-Renteria, Berlin; Cardenas, Jenny C.; Troupin, Andrea; Colpitts, Tonya M.

    2016-01-01

    Chronic exposure to antigens may favor the production of IgG4 antibodies over other antibody types. Recent studies have shown that up to a 30% of normal human IgG4 is bi-specific and is able to recognize two antigens of different nature. A requirement for this specificity is the presence of both eliciting antigens in the same time and at the same place where the immune response is induced. During transmission of most vector-borne diseases, the pathogen is delivered to the vertebrate host along with the arthropod saliva during blood feeding and previous studies have shown the existence of IgG4 antibodies against mosquito salivary allergens. However, there is very little ongoing research or information available regarding IgG4 bi-specificity with regard to infectious disease, particularly during immune responses to vector-borne diseases, such as malaria, filariasis, or dengue virus infection. Here, we provide background information and present our hypothesis that IgG4 may not only be a useful tool to measure exposure to infected mosquito bites, but that these bi-specific antibodies may also play an important role in modulation of the immune response against malaria and other vector-borne diseases in endemic settings. PMID:27746778

  13. Primate-specific evolution of noncoding element insertion into PLA2G4C and human preterm birth

    Directory of Open Access Journals (Sweden)

    Fellman Vineta

    2010-12-01

    Full Text Available Abstract Background The onset of birth in humans, like other apes, differs from non-primate mammals in its endocrine physiology. We hypothesize that higher primate-specific gene evolution may lead to these differences and target genes involved in human preterm birth, an area of global health significance. Methods We performed a comparative genomics screen of highly conserved noncoding elements and identified PLA2G4C, a phospholipase A isoform involved in prostaglandin biosynthesis as human accelerated. To examine whether this gene demonstrating primate-specific evolution was associated with birth timing, we genotyped and analyzed 8 common single nucleotide polymorphisms (SNPs in PLA2G4C in US Hispanic (n = 73 preterm, 292 control, US White (n = 147 preterm, 157 control and US Black (n = 79 preterm, 166 control mothers. Results Detailed structural and phylogenic analysis of PLA2G4C suggested a short genomic element within the gene duplicated from a paralogous highly conserved element on chromosome 1 specifically in primates. SNPs rs8110925 and rs2307276 in US Hispanics and rs11564620 in US Whites were significant after correcting for multiple tests (p PLA2G4C activity. Conclusions Our findings suggest that variation in PLA2G4C may influence preterm birth risk by increasing levels of prostaglandins, which are known to regulate labor.

  14. A Case Report of an Atypical Presentation of IgG4-Related Disease and Idiopathic CD4 Lymphocytopenia.

    Science.gov (United States)

    Rapisarda, Francesco; Zanoli, Luca; Portale, Grazia; Scuto, Salvo; Castellino, Pietro

    2015-01-01

    The IgG4-related disease is a fibroinflammatory disease characterized by tumefactive lesions, a dense lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells, storiform fibrosis, and, often but not always, elevated serum levels of IgG4. Idiopathic CD4 lymphocytopenia is a heterogenic and rare syndrome characterized by the detection of a persistent absolute CD4 T cells count <300 cells/mm(3) (or <20% of total T cells) in more than one occasion and no evidence of HIV infection in absence of immunodeficiency or therapy associated with depressed levels of CD4 T cells. We report the case of a 50-year-old man with a multiorgan IgG4-related disease presenting in a temporal association with a profound and symptomatic idiopathic CD4 lymphocytopenia. Both clinical pictures improved after steroid treatment. Idiopathic CD4 lymphocytopenia has been associated with a number of autoimmune conditions but, to the best of our knowledge, this is the first case in which an association with the IgG4-related disease is reported.

  15. 累及肾和淋巴结的IgG4相关性疾病病理特点%Pathologic features of IgG4 related diseases involving kidney and lymph node

    Institute of Scientific and Technical Information of China (English)

    何同梅; 曲利娟; 谢飞来; 郑智勇

    2013-01-01

    Objective To explore the clinical and pathological features,differential diagnosis,treatment and prognosis of IgG4-related disease.Methods One case of IgG4-related diseases involving the kidneys and lymph node was studied by light microscopy,electron microscopy and immunohistochemistry,and review of literature to explore clinical pathological features of the disease.Results In the kidney biopsy a large number of plasma cells were noted in the renal interstitial infiltration,and these plasma cells mainly were IgG4 positive cells (> 10/HPF) ; increasing giomerular capillary wall became diffusely thick,and segments of double-track formation were observed.Axillary lymph node biopsy showed lymphoid follicles scattered,some follicles was shrinking; interfollicular zone expanded significantly with a large number of plasma cells in the interfollicular region,and mixed with more small lymphocytes and a small amount of immunoblasts.Immunohistochemistry showed that most of plasma cells were IgG positive (> 50/HPF),of which > 50% of the cells were IgG4-positive.Conclusions IgG4-related disease is a chronic systemic disease entity with unknown pathogenesis,multiple organ involvement,and extensive infiltration of IgG4-positive cells.The diagnosis relies on clinical,pathological and serological examination.There is no effective treatment,but the response to steroid therapy is better,and it can recur after treatment.%目的 探讨IgG4相关性疾病的临床病理学特点、鉴别诊断、治疗及预后.方法 对1例累及肾和淋巴结的IgG4相关性疾病病理活检标本进行光镜、电镜及免疫组化染色(采用EliVision法),并结合文献探讨该病的临床病理学特点.结果 患者肾穿刺活检见肾间质中大量浆细胞浸润,主要为IgG阳性细胞,其中IgG4阳性细胞(>10个/HPF);肾小球毛细血管壁弥漫增厚,并节段双轨形成.腋下淋巴结活检显示,淋巴滤泡散在分布,部分滤泡呈萎缩状态,滤泡间区明

  16. Structure of full-length human anti-PD1 therapeutic IgG4 antibody pembrolizumab.

    Science.gov (United States)

    Scapin, Giovanna; Yang, Xiaoyu; Prosise, Winifred W; McCoy, Mark; Reichert, Paul; Johnston, Jennifer M; Kashi, Ramesh S; Strickland, Corey

    2015-12-01

    Immunoglobulin G4 antibodies exhibit unusual properties with important biological consequences. We report the structure of the human full-length IgG4 S228P anti-PD1 antibody pembrolizumab, solved to 2.3-Å resolution. Pembrolizumab is a compact molecule, consistent with the presence of a short hinge region. The Fc domain is glycosylated at the CH2 domain on both chains, but one CH2 domain is rotated 120° with respect to the conformation observed in all reported structures to date, and its glycan chain faces the solvent. We speculate that this new conformation is driven by the shorter hinge. The structure suggests a role for the S228P mutation in preventing the IgG4 arm exchange. In addition, this unusual Fc conformation suggests possible structural diversity between IgG subclasses and shows that use of isolated antibody fragments could mask potentially important interactions, owing to molecular flexibility.

  17. IgG4-Related Mikulicz's Disease Associated with Thyroiditis:a Case Report and Review of the Literature

    Institute of Scientific and Technical Information of China (English)

    Yujiao Zhang; Yi Du; Kaijun Li; Jianfeng He

    2014-01-01

    Purpose:To report an unusual case of IgG4-related Mikulicz's disease associated with thyroiditis. Case report:.We describe a 25-year-old Chinese man who presented with bilateral,.painless swellings of the lachrymal glands,.parotid glands,.and thyroid nodules..The patient un-derwent left-sided dacryoadenectomy and the diagnosis of IgG4-related Mikulicz's disease was pathologically confirmed. The size of the right-sided lachrymal gland and parotid glands recovered fundamentally after one month of glucocorticoid therapy. Conclusion:.IgG4-related Mikulicz's disease associated with thyroiditis should be considered in the differential diagnosis of bilateral swellings of lachrymal glands, salivary glands, and thyroid nodules. Surgical excision is recommended in order to treat the tumor and to ensure the pathological diagnosis. Glu-cocorticoid therapy should be considered in association with surgery after removal. (Eye Science 2014; 29:47-52).

  18. Congruence between nuclear and mitochondrial genes in Demospongiae: a new hypothesis for relationships within the G4 clade (Porifera: Demospongiae).

    Science.gov (United States)

    Morrow, C C; Picton, B E; Erpenbeck, D; Boury-Esnault, N; Maggs, C A; Allcock, A L

    2012-01-01

    The current morphological classification of the Demospongiae G4 clade was tested using large subunit ribosomal RNA (LSU rRNA) sequences from 119 taxa. Fifty-three mitochondrial cytochrome oxidase 1 (CO1) barcoding sequences were also analysed to test whether the 28S phylogeny could be recovered using an independent gene. This is the largest and most comprehensive study of the Demospongiae G4 clade. The 28S and CO1 genetrees result in congruent clades but conflict with the current morphological classification. The results confirm the polyphyly of Halichondrida, Hadromerida, Dictyonellidae, Axinellidae and Poecilosclerida and show that several of the characters used in morphological classifications are homoplasious. Robust clades are clearly shown and a new hypothesis for relationships of taxa allocated to G4 is proposed.

  19. Listeria monocytogenes Meningitis in an Immunosuppressed Patient with Autoimmune Hepatitis and IgG4 Subclass Deficiency

    DEFF Research Database (Denmark)

    Gaini, Shahin

    2015-01-01

    A 51-year-old Caucasian woman with Listeria monocytogenes meningitis was treated and discharged after an uncomplicated course. Her medical history included immunosuppressive treatment with prednisolone and azathioprine for autoimmune hepatitis. A diagnostic work-up after the meningitis episode...... revealed that she had low levels of the IgG4 subclass. To our knowledge, this is the first case report describing a possible association between autoimmune hepatitis and the occurrence of Listeria monocytogenes meningitis, describing a possible association between Listeria monocytogenes meningitis...... and deficiency of the IgG4 subclass and finally describing a possible association between Listeria monocytogenes meningitis and immunosuppressive therapy with prednisolone and azathioprine....

  20. Spectroscopic and calorimetric studies on the interaction between PAMAM G4-OH and 5-fluorouracil in aqueous solutions

    Science.gov (United States)

    Buczkowski, Adam; Urbaniak, Pawel; Piekarski, Henryk; Palecz, Bartlomiej

    2017-01-01

    The results of spectroscopic measurements (an increase in solubility, equilibrium dialysis, 1H NMR titration) and calorimetric measurements (isothermal titration ITC) indicate spontaneous (ΔG solution. PAMAM G4-OH dendrimer bonds about n = 8 ± 1 molecules of the drug with an equilibrium constant of K = 70 ± 10. The process of saturating the dendrimer active sites by the drug molecules is exothermal (ΔH 0). The parameters of binding 5-fluorouracil by PAMAM G4-OH dendrimer were compared with those of binding this drug by the macromolecules of PAMAM G3-OH and G5-OH.

  1. Competitive binding exchange between alkali metal ions (K+, Rb+, and Cs+) and Na+ ions bound to the dimeric quadruplex [d(G4T4G4)]2: a 23Na and 1H NMR study.

    Science.gov (United States)

    Cesare Marincola, Flaminia; Virno, Ada; Randazzo, Antonio; Mocci, Francesca; Saba, Giuseppe; Lai, Adolfo

    2009-12-01

    A comparative study of the competitive cation exchange between the alkali metal ions K+, Rb+, and Cs+ and the Na+ ions bound to the dimeric quadruplex [d(G4T4G4)]2 was performed in aqueous solution by a combined use of the 23Na and 1H NMR spectroscopy. The titration data confirm the different binding affinities of these ions for the G-quadruplex and, in particular, major differences in the behavior of Cs+ as compared to the other ions were found. Accordingly, Cs+ competes with Na+ only for the binding sites at the quadruplex surface (primarily phosphate groups), while K+ and Rb+ are also able to replace sodium ions located inside the quadruplex. Furthermore, the 1H NMR results relative to the CsCl titration evidence a close approach of Cs+ ions to the phosphate groups in the narrow groove of [d(G4T4G4)]2. Based on a three-site exchange model, the 23Na NMR relaxation data lead to an estimate of the relative binding affinity of Cs+ versus Na+ for the quadruplex surface of 0.5 at 298 K. Comparing this value to those reported in the literature for the surface of the G-quadruplex formed by 5'-guanosinemonophosphate and for the surface of double-helical DNA suggests that topology factors may have an important influence on the cation affinity for the phosphate groups on DNA.

  2. McAb-ELISA检测丝虫特异IgG4的应用研究%Study on Application of McAb-ELISA to Filaria Surveillance by Detecting Filaria-specSifie IgG4

    Institute of Scientific and Technical Information of China (English)

    陈锡欣; 邓绪礼; 李桂萍; 徐凤全; 傅斌; 刘新; 高长兰; 赵中平

    2002-01-01

    目的探讨McAb-ELISA检测丝虫特异IgG4在丝虫感染监测中的应用价值.方法利用生化免疫技术,经细胞融合,建立起7株对IgG4表现为单一反应性的单克隆抗体(McAb).以此为探针,采用ELISA方法检测丝虫特异IgG4.结果检测班氏微丝蚴血症者血清的敏感性为96.3%(104/108),测得IgG4的最低限量为0.01ul/ml;检测丝虫病非流行区健康者、肠道线虫感染者、华支睾吸虫感染者和囊虫病患者等血清均呈阴性,未出现交叉反应,特异性为100%.经现场应用研究,同样获得良好的实用性,有效地反映出当地丝虫病流行与感染状况.结论该技术是监测丝虫感染的理想方法.

  3. 1例IgG4相关硬化性疾病临床病理分析及文献复习%IgG4-related sclerosing disease:A case report and review of literature

    Institute of Scientific and Technical Information of China (English)

    荣娜; 魏日胞; 王远大

    2011-01-01

    Objective To report a rare case of IgG4- related sclerosing disease involving multiple organs in order to improve our understanding of it. Methods A rare case of IgG4- related sclerosing disease admitted to our hospital in November 2010 with its diagnosis established by pathology was analyzed and its clinical manifestations, diagnosis, treatment, and prognosis were discussed according to its domestic and foreign literature. Results The patient's clinical manifestations included diabetes mellitus, impaired renal function, enlargement of lymph nodes in the neck and groin, high IgG and IgG4 level, and enlargement of pancreas. Abdominal CT showed enlargement of pancreas and interstitial pneumonia. Biopsy of lymph nodes revealed infiltration of IgG4 -positive plasma cells. Biopsy of kidney tissue displayed focal sclerosing glomerulonephritis. The renal function became normal and the size of pancreas and lymph nodes was decreased 3 weeks after treatment with corticosteroids. The serum IgG and IgE levels returned to normal and diabetes mellitus was well controlled 20 weeks after treatment with corticosteroids. Conclusion IgG4-related sclerosing disease is a very rare systemic disease, which can be controlled with prompt use of corticosteroids.%目的 报告1例罕见的IgG4相关硬化性疾病多器官受累病例,提高该病的认识水平.方法 分析我院2010年11月诊治并经病理学确诊的IgG4相关硬化性疾病1例,并结合国内外文献,对本病的临床表现、诊断、治疗及预后进行分析讨论.结果 患者临床表现为糖尿病、肾功能受损,颈部、腹股沟等全身淋巴结肿大;血清IgG、IgG4异常增高,CT检查胰腺肿大、间质性肺炎,淋巴结活检可见IgG4阳性浆细胞浸润,肾脏活检病理为局灶硬化性肾小球肾炎;激素治疗3周肾功能正常,胰腺及淋巴结形态明显缩小,20周血IgG、IgE正常,糖尿病控制良好.结论 IgG4相关硬化性疾病是一种非常罕见的全身系统

  4. Phylogenetic analysis of porcine rotavirus in Argentina: increasing diversity of G4 strains and evidence of interspecies transmission.

    Science.gov (United States)

    Parra, Gabriel I; Vidales, Graciela; Gomez, Jorge A; Fernandez, Fernando M; Parreño, Viviana; Bok, Karin

    2008-01-01

    Group A rotaviruses are one of the most frequently detected viral agents associated with neonatal diarrhea in piglets. In order to characterize rotavirus (RV) strains circulating in Argentinean swine, four porcine production farms located in Buenos Aires were studied. RV strains genotyped as P[6]G4, P[6]G8 and P[1]G6 were found in piglets under 30 days of age, without diarrhea. Phylogenetic and sequence analysis of the VP7 gene from G4 strains available in databases, reveals five porcine new lineages (III-VII) and three sublineages (VIIa-VIIc). The G4 porcine Argentinean strains were grouped with a porcine RV strain isolated in Brazil and another RV strain isolated from a child with diarrhea in Mexico, constituting an American lineage (VII). On the other hand, porcine G6 and G8 were closely related to RV's circulating in Argentinean cattle and South-American camelids, respectively. The fact that G4 porcine lineages were epidemiologically related to human strains, and G6 and G8 Argentinean porcine strains were found related to bovine and South-American camelids, respectively, suggests that pigs might play a crucial role as reservoir and generator of newly adapted emerging RV strains for human and other species.

  5. Impacts, Effectiveness and Regional Inequalities of the GeoMIP G1 to G4 Solar Radiation Management Scenarios

    Energy Technology Data Exchange (ETDEWEB)

    Yu, Xiaoyong; Moore, John; Cui, Xuefeng; Rinke, Annette; Ji, Duoying; Kravitz, Benjamin S.; Yoon, Jin-Ho

    2015-06-01

    We evaluate the regional effectiveness of solar radiation management (SRM) to compensate for simultaneous changes in temperature and precipitation induced by increased greenhouse gas concentrations. We analyze results from multiple earth system models under four Geoengineering Model Intercomparison Project(GeoMIP) experiments with a modified form of the Residual Climate Response approach. Under the solar dimming geoengineering experiments G1(4xCO2) and G2(increasing CO2 by 1% per year), global average temperature is successfully restored to pre-industrial level over 50 years simulations. However, these two SRM experiments also produce a robust global precipitation decrease. The stratospheric aerosol GeoMIP geoengineering experiment, G4 has significantly greater regional inequality and lower effectiveness for compensating temperature change than G1 and G2. G4 also has significantly larger regional inequality for compensating precipitation change than G1and G2. However, there is no significant difference between precipitation change compensation effectiveness of G4 and G2, though there is much larger across model variability in G4 results. G3 has significant greater regional inequality for compensating temperature change than G1 and G2, and has significant lower effectiveness than G1. The effectiveness of four SRMs to compensate for temperature change is much higher than for precipitation. The large cross-model variation in adjustment percentage of compensated SAT and precipitation change by SRM to achieve optimal compensation effectiveness shed a light on the uncertainty accumulation effect in optimizing compensation effectiveness of SRM.

  6. Cometabolic Degradation of Trichloroethylene by Pseudomonas cepacia G4 in a Chemostat with Toluene as the Primary Substrate

    NARCIS (Netherlands)

    Landa, Andrew S.; Sipkema, E. Marijn; Weijma, Jan; Beenackers, Antonie A.C.M.; Dolfing, Jan; Janssen, Dick B.

    1994-01-01

    Pseudomonas cepacia G4 is capable of cometabolic degradation of trichloroethylene (TCE) if the organism is grown on certain aromatic compounds. To obtain more insight into the kinetics of TCE degradation and the effect of TCE transformation products, we have investigated the simultaneous conversion

  7. 77 FR 31611 - Proposed CERCLA Section 122(g)(4) Administrative Agreement and Order on Consent for the Mercury...

    Science.gov (United States)

    2012-05-29

    ... AGENCY Proposed CERCLA Section 122(g)(4) Administrative Agreement and Order on Consent for the Mercury... the Mercury Refining Superfund Site (``Site'') located in the Towns of Guilderland and Colonie, Albany... Hazardous Substance Superfund Mercury Refining Superfund Site Special Account, which combined total...

  8. Effect of alginate and chitosan on viability and release behavior of Bifidobacterium pseudocatenulatum G4 in simulated gastrointestinal fluid.

    Science.gov (United States)

    Kamalian, Nikoo; Mirhosseini, Hamed; Mustafa, Shuhaimi; Manap, Mohd Yazid Abd

    2014-10-13

    The main aim of this study was to investigate the effect of different coating materials (i.e. Na-alginate and chitosan) on the viability and release behavior of Bifidobacterium pseudocatenulatum G4 in the simulated gastric fluid (SGF) and simulated intestinal fluid (SIF). This study reports the viability of encapsulated B. pseudocatenulatum G4 coated using different alginate (2-4 g/100mL) and chitosan (0.2-0.8 g/100mL) concentrations. The results indicated that the highest concentration of alginate (4.4142 g/100mL) along with 0.5578 g/100mL chitosan resulted in the highest viability of B. pseudocatenulatum G4. The release behavior of the encapsulated probiotics in SGF (pH 1.5) in 2h followed by 4h in SIF (pH 7.4) was also assessed. The resistance rate of alginate-chitosan capsule in SGF was higher than SIF. The alginate-chitosan encapsulated cells had also more resistance than alginate capsules. The current study revealed that alginate encapsulated B. Pseudocatenulatum G4 exhibited longer survival than its free cells (control).

  9. Is immunotherapy-induced birch-pollen-specific IgG4 a marker for decreased allergen-specific sensitivity?

    DEFF Research Database (Denmark)

    Bodtger, U; Ejrnaes, A M; Hummelshoj, L

    2005-01-01

    The role of IgG4 during allergen-specific immunotherapy (SIT) is still controversial. The available studies present paramount differences in in vitro techniques, allergens, and clinical outcome parameters. By implementing a sensitive method, and pivotal clinical outcome parameters, we wanted...

  10. Outcome and Genetic Factors in IgG4-Associated Autoimmune Pancreatitis and Cholangitis: A Single Center Experience

    Directory of Open Access Journals (Sweden)

    Matthias Buechter

    2017-01-01

    Full Text Available Introduction. Most investigations on autoimmune pancreatitis (AIP were published on Asian cohorts while those on Caucasians are limited. However, there might be differences related to the origin. Patients and Methods. We analyzed 36 patients and compared type 1 (AIP1 with type 2 (AIP2. Results. The majority of patients suffered from AIP1 (55.6%. AIP1 patients were significantly older than AIP2 patients (54.4 versus 40.8 years. Moreover, 85.0% of AIP1 patients had concurrent autoimmune cholangitis (AIC while 18.8% of AIP2 patients suffered from overlap to ulcerative colitis (UC. However, AIP1 patients revealed a cholestatic course and had significantly higher immunoglobulin G4 levels (IgG4. When compared to allele frequencies in healthy controls, in patients with AIP1 HLA-B8 reached statistical significance. Response to steroids was excellent in both groups, but we noticed high rates of relapse especially in AIP1 patients. Finally, 3 patients with AIP1 were diagnosed with cholangiocellular carcinoma (CCC. Conclusion. In contrast to Asian studies, we found an almost equal distribution of AIP1 and AIP2 patients in our German cohort. AIP2 patients were younger and mostly of female gender whereas AIP1 patients revealed higher IgG4 levels and involvement of the biliary tract in sense of IgG4-associated cholangitis.

  11. Role of IgG4 in histamine release from human basophil leucocytes. I. Sensitization of cells from normal donors

    DEFF Research Database (Denmark)

    Poulsen, L K; Stahl Skov, P; Mosbech, H

    1988-01-01

    Several conflicting reports on the ability of IgG4 to mediate type I allergic reactions have appeared lately. We have developed a model system for testing this possibility, using passive sensitization of basophil leucocytes from normal individuals. At first, the system was optimized with regard...

  12. Degradation of toluene and trichloroethylene by Burkholderia cepacia G4 in growth-limited fed-batch culture

    NARCIS (Netherlands)

    Mars, Astrid E.; Houwing, Joukje; Dolfing, Jan; Janssen, Dick B.

    1996-01-01

    Burkholderia (Pseudomonas) cepacia G4 was cultivated in a fed-batch bioreactor on either toluene or toluene plus trichloroethylene (TCE), The culture was allowed to reach a constant cell density under conditions in which the amount of toluene supplied equals the maintenance energy demand of the cult

  13. A Retrospectively Diagnosed Case of IgG4-Related Tubulointerstitial Nephritis Showing Good Renal Outcome and Pathological Progress

    Directory of Open Access Journals (Sweden)

    Qiong Wu

    2013-01-01

    Full Text Available A 74-year-old man was hospitalized for diabetic nephropathy evaluation and assessment of the effect of treatment on his tubulointerstitial nephritis (TIN. When he was 62 years old, he developed polyarthralgia and had superficial lymph node swelling, mildly increased serum creatinine concentration, hypergammaglobulinemia, hypocomplementemia, high serum IL-2R level, and positive titer of antinuclear antibody. Several tissues were biopsied. Mild chronic sialadenitis and reactive lymphadenitis were identified. Renal specimen showed mild glomerular ischemia, extensive storiform fibrosis, and abundant infiltrating monocytes and plasma cells. He was treated with oral prednisolone and cyclophosphamide. After the treatment, most of his clinical parameters quickly returned to within the reference range. However, he developed diabetes mellitus soon after steroid therapy. At the time of rebiopsy, a high level of serum IgG4 was detected. The second renal biopsy showed diabetic nephropathy without any tubulointerstitial damage. The first biopsied tissues were retrospectively investigated. Large numbers of IgG4-positive plasma cells were detected in the kidneys and lymph nodes. A retrospective diagnosis of IgG4-related TIN with lymph node involvement was made. In conclusion, this paper describes a retrospectively diagnosed case of IgG4-related TIN with lymph node involvement, showing good clinical and pathological prognosis.

  14. Sjögren’s syndrome versus IgG4-related diseases – classification difficulties and treatment progress

    Directory of Open Access Journals (Sweden)

    Anna Nowakowska-Płaza

    2014-09-01

    Full Text Available Sjögren’s syndrome (SS is a chronic autoimmune disorder characterized by lymphocytic infiltration in exocrine glands mainly salivary and lacrimal which affects impairment of their functions. Some patients develop extraglandular symptoms such as chronic fatigue, arthralgia, or lung, renal, central or peripheral nervous system involvement. Recent decades have brought understanding of some pathogenetic mechanisms and offered new therapeutic options by depleting B cells. Furthermore, the American College of Rheumatology proposed a new set of classification criteria based on objective symptoms. IgG4-related diseases are new nosological entities. The clinical course similarities of SS to Mikulicz’s disease (a subtype of IgG4-related disease result in diagnostic difficulties. Typical conditions of them are: an increased IgG4 level and infiltrations of parenchymal organs by plasmatic cells. This review summarizes classification difficulties, pathogenesis and treatment strategies of SS and IgG4-related diseases.

  15. Characterizing IgG4-related disease with {sup 18}F-FDG PET/CT: a prospective cohort study

    Energy Technology Data Exchange (ETDEWEB)

    Zhang, Jingjing; Ma, Yanru; Niu, Na; Wang, Xinwei; Li, Fang; Zhu, Zhaohui [Chinese Academy of Medical Sciences and Peking Union Medical College, Department of Nuclear Medicine, Peking Union Medical College Hospital, Beijing (China); Chen, Hua; Lin, Wei; Zhang, Fengchun; Zhang, Wen [Chinese Academy of Medical Sciences and Peking Union Medical College, Department of Rheumatology, Peking Union Medical College Hospital, Beijing (China); Xiao, Yu; Liang, Zhiyong [Chinese Academy of Medical Sciences and Peking Union Medical College, Department of Pathology, Peking Union Medical College Hospital, Beijing (China)

    2014-08-15

    IgG4-related disease (IgG4-RD) is an increasingly recognized clinicopathological disorder with immune-mediated inflammatory lesions mimicking malignancies. A cohort study was prospectively designed to investigate the value of {sup 18}F-fluorodeoxyglucose (FDG) positron emission tomography/computed tomography (PET/CT) in characterizing IgG4-RD. Thirty-five patients diagnosed with IgG4-RD according to the consensus criteria were enrolled with informed consent. All patients underwent baseline {sup 18}F-FDG PET/CT evaluation. Among them, 29 patients underwent a second {sup 18}F-FDG PET/CT scan after 2 to 4 weeks of steroid-based therapy. All 35 patients were found with {sup 18}F-FDG-avid hypermetabolic lesion(s); 97.1 % (34/35) of these patients showed multi-organ involvement. Among the 35 patients, 71.4 % (25/35) patients were found with more organ involvement on {sup 18}F-FDG PET/CT than conventional evaluations including physical examination, ultrasonography, and computed tomography (CT). {sup 18}F-FDG PET/CT demonstrated specific image characteristics and pattern of IgG4-RD, including diffusely elevated {sup 18}F-FDG uptake in the pancreas and salivary glands, patchy lesions in the retroperitoneal region and vascular wall, and multi-organ involvement that cannot be interpreted as metastasis. Comprehensive understanding of all involvement aided the biopsy-site selection in seven patients and the recanalization of ureteral obstruction in five patients. After 2 to 4 weeks of steroid-based therapy at 40 mg to 50 mg prednisone per day, 72.4 % (21/29) of the patients showed complete remission, whereas the others exhibited > 81.8 % decrease in {sup 18}F-FDG uptake. F-FDG PET/CT is a useful tool for assessing organ involvement, monitoring therapeutic response, and guiding interventional treatment of IgG4-RD. The image pattern is suggested to be updated into the consensus diagnostic criteria for IgG4-RD. (orig.)

  16. Recent advances in the concept and pathogenesis of IgG4-related disease in the hepato-bilio-pancreatic system.

    Science.gov (United States)

    Okazaki, Kazuichi; Yanagawa, Masahito; Mitsuyama, Toshiyuki; Uchida, Kazushige

    2014-09-01

    Recent studies have proposed nomenclatures of type 1 autoimmune pancreatitis (AIP) (IgG4-related pancreatitis), IgG4-related sclerosing cholangitis (IgG4-SC), IgG4-related cholecystitis, and IgG4-related hepatopathy as IgG4-related disease (IgG4-RD) in the hepato-bilio-pancreatic system. In IgG4-related hepatopathy, a novel concept of IgG4-related autoimmune hepatitis (AIH) with the same histopathological features as AIH has been proposed. Among organs involved in IgG4-RD, associations with pancreatic and biliary lesions are most frequently observed, supporting the novel concept of "biliary diseases with pancreatic counterparts." Targets of type 1 AIP and IgG4-SC may be periductal glands around the bile and pancreatic ducts. Based on genetic backgrounds, innate and acquired immunity, Th2-dominant immune status, regulatory T (Treg) or B cells, and complement activation via a classical pathway may be involved in the development of IgG4-RD. Although the role of IgG4 remains unclear in IgG4-RD, IgG4-production is upregulated by interleukin 10 from Treg cells and by B cell activating factor from monocytes/basophils with stimulation of toll-like receptors/nucleotide-binding oligomerization domain-like receptors. Based on these findings, we have proposed a hypothesis for the development of IgG4-RD in the hepato-bilio-pancreatic system. Further studies are necessary to clarify the pathogenic mechanism of IgG4-RD.

  17. A high number of IgG4-positive cells in gastric cancer tissue is associated with tumor progression and poor prognosis.

    Science.gov (United States)

    Miyatani, Kozo; Saito, Hiroaki; Murakami, Yuki; Watanabe, Joji; Kuroda, Hirohiko; Matsunaga, Tomoyuki; Fukumoto, Yoji; Osaki, Tomohiro; Nakayama, Yuji; Umekita, Yoshihisa; Ikeguchi, Masahide

    2016-05-01

    IgG4-related disease is a newly defined disease characterized by elevated serum IgG4 levels and infiltration of affected organs by IgG4-positive plasma cells. Recently, increased IgG4 levels were reported to be closely related with malignancy. To assess the relationship between IgG4 and the progression of gastric cancer, we immunohistochemically stained in this study gastric cancer tissue samples for IgG4-positive cells using an anti-IgG4 antibody. In addition, pre- and postoperative serum concentrations of IgG4 were measured, using an enzyme-linked immunosorbent assay. In gastric cancer samples, the number of CD138-positive plasma cells was significantly lower and the number of IgG4-positive cells significantly higher than in non-cancerous gastric mucosa. The number of IgG4-positive cells was significantly correlated with gross tumor appearance, tumor depth, lymph node metastasis, venous invasion, and lymphatic invasion. Prognosis was significantly poorer in patients with a high number of IgG4-positive cells than in those with a low number. Multivariate analysis indicated that both the number of IgG4-positive cells and the depth of tumor invasion were independently prognostic of survival. In conclusion, in gastric cancer, the number of IgG4-positive cells is increased and this is closely associated with gastric cancer progression.

  18. [Hypertrophic pachymeningitis secondary to IgG4-related disease: case report and review of the literature].

    Science.gov (United States)

    Rodríguez-Castro, Emilio; Fernández-Lebrero, Aida; López-Dequidt, Iria A; Rodríguez-Osorio, Xiana; López-González, Francisco J; Suárez-Peñaranda, José M; Arias, Manuel

    2015-10-01

    Introduccion. La paquimeningitis hipertrofica es un trastorno infrecuente que produce un engrosamiento focal o difuso de la duramadre. Puede ser idiopatica o secundaria a procesos infecciosos, autoinmunes o neoplasicos. La recientemente descrita 'enfermedad relacionada con IgG4' podria ser la causa de bastantes cuadros considerados criptogenicos. Caso clinico. Mujer de 54 años, con historia de asma bronquial, que consulto por cefalea, vertigo y perdida de audicion por su oido izquierdo. En la resonancia magnetica cerebral con gadolinio se objetivo engrosamiento y realce dural, que se extendia desde la pared lateral del seno cavernoso izquierdo y la parte medial del lobulo temporal al angulo pontocerebeloso y parte del tentorio homolaterales. El liquido cefalorraquideo presentaba 10 leucocitos/µL (90% mononucleares), con 1 g/L de proteinas y sin consumo de glucosa. El estudio anatomopatologico mostro fibrosis y un infiltrado linfoplasmocitario, con 16 celulas plasmaticas IgG4+ por campo de gran aumento. El resto de estudios analiticos y microbiologicos resultaron normales o negativos. La tasa plasmatica de IgG4 estaba dentro de los limites normales. Tratada con esteroides, se produjo mejoria clinica acompañada de la practica desaparicion de las alteraciones detectadas en la neuroimagen. Conclusiones. La paquimeningitis hipertrofica como manifestacion de la enfermedad relacionada con IgG4 puede diagnosticarse basandose en los hallazgos de la resonancia magnetica si la IgG4 plasmatica esta elevada. En casos dudosos, habra que recurrir a la biopsia meningea. La corticoterapia suele ser eficaz y representa la primera linea terapeutica.

  19. IgG4相关性肺疾病二例并文献复习%IgG4-related lung disease:a clinical analysis and literature review

    Institute of Scientific and Technical Information of China (English)

    单兴华; 聂小蒙; 勇晓; 汪莹; 陈亮; 何妙霞; 姚小鹏; 李强; 黄怡

    2015-01-01

    目的 总结IgG4相关性肺疾病的临床特点及诊治经验,提高临床对该病的认识,减少误诊误治.方法 分析第二军医大学附属长海医院呼吸内科收治的两例IgG4相关性肺疾病的一般情况、临床表现、实验室检查、组织病理学、治疗及预后,并进行相关文献复习.结果 两例患者均为男性,血清IgG4水平均显著升高(2.25 g/L;10 g/L).1例肺部影像学呈实性结节型改变,并有回盲部受累,糖皮质激素治疗有效.另1例肺部影像学呈支气管血管束型改变,对糖皮质激素治疗依赖.文献复习69例IgG4相关性肺疾病,因呼吸道症状就诊者39例(56.5%),伴有肺外受累者41例(59.4%).48例检测了血清IgG4水平,均显著升高(307 ~ 52 500 mg/L).胸部影像学表现实性结节型35例(50.7%).31例(44.9%)患者接受了糖皮质激素治疗,预后良好.结论 IgG4相关性肺疾病是临床少见的免疫性疾病,临床表现缺乏特异性,临床诊断应综合分析其血清免疫学检查、影像学表现及组织病理学结果,糖皮质激素治疗效果良好.%Objective To summarize the clinical features,diagnosis and treatment experience of IgG4-related lung disease for the sake of improving clinical understanding of this disease and reducing the misdiagnosis and mistreatment rates.Methods To analyze the general situation,clinical manifestation,laboratory examination,histopathology,therapy and prognosis of 2 patients with IgG4-related lung disease,who were admitted in the department of respiratory diseases at Changhai Hospital.Publications related to IgG4 lung disease were reviewed.Results Both patients were male with elevated serum IgG4 level(2.25 g/L and 10 g/L respectively).In one patient,radiologic finding showed solid nodules in the lung with ileocecal involvement.He responded well to glucocorticoid.The other patient's computed tomography of lung demonstrated bronchovascular type.Glucocorticoid therapy was effective to both

  20. Clinical analysis of primary orbital IgG4-related disease%原发眼眶IgG4相关性疾病的临床分析

    Institute of Scientific and Technical Information of China (English)

    俞丹洋; 魏锐利; 蔡季平; 吴联群; 程金伟

    2015-01-01

    目的 总结原发眼眶IgG4相关性疾病的临床症状、体征、诊断、治疗和预后.方法 回顾2013年1月至2014年1月在上海长征医院眼科就诊为原发眼眶IgG4相关性疾病患者9例,对其临床症状、眼部体征、实验室及影像学检查、诊断及治疗进行分析.结果 该病好发于中老年人,男性多见.临床主要表现为眼球突出伴眶组织肿胀,可伴眼球运动受限.影像学检查多表现泪腺组织病变,可累及眼周边附属器组织.实验室检查结果显示血清IgG4> 1.35 g/L.术中见8例瘤体无明显包膜,1例包膜完整.术后组织病理学检查见淋巴细胞、浆细胞浸润及纤维化;IgG4浆细胞浸润,且IgG4细胞超过10个/HPF.随访8~20个月有2例复发.结论 眼眶IgG4病易发生于老年人,多侵犯泪腺及其周边组织,血清学及组织病理检查诊断可以明确.%Objective To analyze the clinical manifestations of disease, diagnosis, treatment and prognosis of primary orbital IgG4-related disease.Methods Aretrospective study of 9 cases with primary orbital IG4 related diseases, and the clinical data, including clinical symptoms and signs, laboratory and radiological examination results, and treatment were analyzed.Results The disease usually occurred in elder men.The main clinical manifestations were proptosis and eye lid swelling.Imaging often showed enlarged lacrimal gland tissue lesions, partly may involve ocular adnexal surrounding tissue.Lesions from 8 cases had no obvious capsules, but one had.Recurrences were observed in 2 cases during the 8-20 months follow-up time.Conclusions IgG4-related diseases are prone to occur in the elderly, usually involve the lacrimal gland and its surrounding tissue.Serological and histopathological examinations are necessary to make the final diagnosis.

  1. IgG4相关性胆胰疾病的诊断和治疗%Diagnosis and Treatment of IgG4-related Biliary and Pancreatic Diseases

    Institute of Scientific and Technical Information of China (English)

    白文元; 戴胜兰

    2014-01-01

    近年来发现,IgG4相关性疾病可累及全身多系统多器官,目前研究提示IgG4相关性胆胰疾病主要有IgG4相关性硬化性胆管炎和自身免疫性胰腺炎(AIP).前者是一种免疫发病机制未明、生化学特点以及胆管影像学表现与原发性硬化性胆管炎(PSC)相似,对糖皮质激素应答良好的疾病.后者临床表现为复发性胰腺炎、梗阻性黄疸,伴胆管、涎腺等其他器官受累.实验室检查血清和组织学IgG4明显升高.影像学表现为胰腺呈“腊肠样”外观,伴主胰管弥漫不规则狭窄,同样对激素治疗敏感.二者常同时受累、关系密切,易误诊为胆管癌、硬化性胆管炎或胰腺癌,本文将当前国内外研究现状做一介绍,供临床诊治参考.

  2. IgG4相关硬化性疾病30例病理特点%Pathological Features of 30 Cases IgG4 Related Sclerosing Disease

    Institute of Scientific and Technical Information of China (English)

    陈志忠; 陈小岩

    2014-01-01

    Objective To explore the IgG4 related to the pathological features of disease, to improve the recognition of clinical and pathologic doctor for this disease. Methods Collect the pathological diagnosis of IgG4 related pathological 30 cases, disease changes to the histological and immunohistochemical characteristics and so on carries on the observation and analysis, and review the relevant literature. Results 30 cases of IgG4 related mean age, 52.0 years, patients with sclerosing disease than for men and women:3:2, af ected organs including nasopharyngeal 4 cases, 5 cases of submandibular gland, sublingual gland in 1 case, 1 cases of parotid gland, tear ducts or surrounding tissue in 3 cases, 1 case of spleen, lung, 4 cases, 2 cases of liver, lymph nodes in 1 case, 1 case of abdominal wall, 1 cases of retroperitoneal, 1 cases of pelvic floor, 2 cases and left temporal maxil ary neoplasm, knee, back the mass in 1 case;Histological changes of the af ected tissue lymphocytic and plasma cell infiltrates in great quantities, part with lymphoid fol icular formation, fibrous tissue hyperplasia, some cases visible inherent gland atrophy, infringement of vascular, hyperplasia of vascular occlusive vasculitis and existence;Immunohistochemical staining showed IgG4 + 30 ~ 100 / HPF plasma cellcounts, IgG4 + / IgG ratio > 40%. Conclusion IgG4 related sclerosing disease is a rare autoimmune disease, etiology is unknown and involving multiple systems, lack of characteristic clinical manifestations, diagnosis combined with clinical manifestation, laboratory examination and histopathology of comprehensive analysis, among them, the laboratory examination, tissue morphological features and immunohistochemical expression is an important diagnostic basis.%目的探讨IgG4相关硬化性疾病的病理特征,提高临床和病理医生对本病的认识。方法收集病理诊断为IgG4相关硬化性疾病的病理30例,对其组织学改变及免疫组化特点等进

  3. Listeria monocytogenes Meningitis in an Immunosuppressed Patient with Autoimmune Hepatitis and IgG4 Subclass Deficiency

    Directory of Open Access Journals (Sweden)

    Shahin Gaini

    2015-01-01

    Full Text Available A 51-year-old Caucasian woman with Listeria monocytogenes meningitis was treated and discharged after an uncomplicated course. Her medical history included immunosuppressive treatment with prednisolone and azathioprine for autoimmune hepatitis. A diagnostic work-up after the meningitis episode revealed that she had low levels of the IgG4 subclass. To our knowledge, this is the first case report describing a possible association between autoimmune hepatitis and the occurrence of Listeria monocytogenes meningitis, describing a possible association between Listeria monocytogenes meningitis and deficiency of the IgG4 subclass and finally describing a possible association between Listeria monocytogenes meningitis and immunosuppressive therapy with prednisolone and azathioprine.

  4. Visual detection of filaria-specific IgG4 in urine using red-colored high density latex beads.

    Science.gov (United States)

    Nagaoka, Fumiaki; Itoh, Makoto; Samad, Mohammad S; Takagi, Hidekazu; Weerasooriya, Mirani V; Yahathugoda, Thishan C; Hossain, Moazzem; Moji, Kazuhiko; Kimura, Eisaku

    2013-02-01

    The use of urine for the immunodiagnosis of lymphatic filariasis has a definite advantage: the sample collection is not invasive and thus well accepted by people. Urine-based ELISA to detect filaria-specific IgG4 has been used successfully. However, ELISA requires equipment such as a microplate reader, which is often not available in most endemic areas. We have developed a new visual immunodiagnosis that detects urinary IgG4 using red-colored latex beads (bead test). The sensitivity was 87.2% when ICT antigen test positive people were regarded as the standard (136/156), and the specificity was 97.2% with the non-endemic people in Japan and Bangladesh, and the urine ELISA negatives in Sri Lanka (1264/1300). In a prevalence study, the bead test could detect filarial infection more effectively than ICT test among young children in Sri Lanka, indicating the usefulness of the visual test in epidemiological studies.

  5. Contrast enhanced ultrasonography in the diagnosis of IgG4-negative autoimmune pancreatitis: A case report

    Science.gov (United States)

    Zhang, Min-Min; Zou, Duo-Wu; Wang, Yin; Zheng, Jian-Ming; Yang, Hua; Jin, Zhen-Dong

    2011-01-01

    Autoimmune pancreatitis (AIP) is a rare disorder frequently manifesting as a mass-like lesion that may lead to obstructive jaundice. We report here a case of pancreatic obstruction with painless jaundice, and elevation of CA 19-9 without elevation of serum IgG4. Contrast enhanced ultrasonography (CE US) revealed the possibility of AIP, and the final pathological findings confirmed the diagnosis. PMID:22586534

  6. A patient with hypereosinophilic syndrome that manifested with acquired hemophilia and elevated IgG4: a case report

    Directory of Open Access Journals (Sweden)

    Nagao Yoshiro

    2012-02-01

    Full Text Available Abstract Introduction Hypereosinophilic syndrome is defined as a prolonged state (more than six months of eosinophilia (greater than 1500 cells/μL, without an apparent etiology and with end-organ damage. Hypereosinophilic syndrome can cause coagulation abnormalities. Among hypereosinophilic syndrome types, the lymphocytic variant (lymphocytic hypereosinophilic syndrome is derived from a monoclonal proliferation of T lymphocytes. Here, we describe the case of a patient with lymphocytic hypereosinophilic syndrome who presented with a coagulation abnormality. To the best of our knowledge, this is the first such report including a detailed clinical picture and temporal cytokine profile. Case presentation A 77-year-old Japanese man presented to our facility with massive hematuria and hypereosinophilia (greater than 2600 cells/μl. His eosinophilia first appeared five years earlier when he developed femoral artery occlusion. He manifested with multiple hematomas and prolonged activated partial thromboplastin time. His IgG4 level was remarkably elevated (greater than 2000 mg/dL. Polymerase chain reaction tests of peripheral blood and bone marrow identified lymphocytic hypereosinophilic syndrome. His prolonged activated partial thromboplastin time was found to be due to acquired hemophilia. Glucocorticoids suppressed both the hypereosinophilia and coagulation abnormality. However, tapering of glucocorticoids led to a relapse of the coagulation abnormality alone, without eosinophilia. Tumor necrosis factor α, interleukin-5, and/or eotaxin-3 may have caused the hypereosinophilia, and interleukin-10 was correlated with the coagulation abnormality. Conclusions To the best of our knowledge, this is the first case in which lymphocytic hypereosinophilic syndrome and IgG4-related disease have overlapped. In addition, our patient is only the second case of hypereosinophilic disease that manifested with acquired hemophilia. Our patient relapsed with the

  7. An autopsy case of acute pancreatitis with a high serum IgG4 complicated by amyloidosis and rheumatoid arthritis

    Institute of Scientific and Technical Information of China (English)

    Tatsuki Ichikawa; Kazuhiko Nakao; Keisuke Hamasaki; Kazuaki Ohkubo; Kan Toriyama; Katsumi Eguchi

    2005-01-01

    We report an autopsy case of acute pancreatitis with a high serum IgG4 concentration complicated by systemic amyloid A amyloidosis and rheumatoid arthritis (RA). The patient was a 42-year-old Japanese female with a 22-year history of rheumatoid arthritis. She was diagnosed with myasthenia gravis when she was 31-year old. At the onset of pancreatitis, the patient was anti-nuclear antibody-positive,and had high serum gamma globulin and IgG4 levels.Dexamethasone and conventional therapy induced clinical remission and significantly decreased the serum IgG4 and gamma globulin. However, despite the decreased disease parameters, the patient developed a bleeding pseudocyst and died of cardiac failure. In the autopsy examination, it was determined that pancreatitis was probably caused by ischemia due to vascular obstruction caused by amyloid deposition in the pancreas. Even though acute pancreatitis is a rare complication in RA patients, we speculate that an autoimmune pancreatitis-related mechanism and ischemia due to vascular obstruction by amyloid deposition might be attributable to a single source that leads to acute pancreatitis in our particular case.

  8. Recent advances in understanding and managing IgG4-related disease [version 1; referees: 4 approved

    Directory of Open Access Journals (Sweden)

    Anna R. Wolfson

    2017-02-01

    Full Text Available IgG4-related disease was only recently discovered, so its description, management, and new discoveries related to its etiology are rapidly evolving. Because IgG4 itself is a unique antibody which is intimately related to the diagnosis of the disease, the role of plasmablasts in the pathophysiology remains an active area of discussion. Recent studies have uncovered a possible role for CD4-positive cytotoxic T lymphocytes, T follicular helper cells, and M2 macrophages. The clinical presentation is variable and can be vague, as this disease affects many organs and new presentations are continuing to be described. The diagnosis depends on clinical and histopathological assessment. The mainstay of treatment is with glucocorticoids, but rituximab has recently shown promise. Monitoring disease activity using imaging modalities (including positron emission tomography and serum markers is imperative, as relapses are common. IgG4-related disease spans many medical disciplines but is a treatable condition with which all clinicians should be familiar.

  9. Experimental Performance Evaluation of Multihop IEEE 802.15.4/4g/4e Smart Utility Networks in Outdoor Environment

    Directory of Open Access Journals (Sweden)

    Chin-Sean Sum

    2017-01-01

    Full Text Available This paper presents the experimental performance evaluation results of the IEEE 802.15.4/4g/4e Smart Utility Networks (SUN in applications suited for outdoor environment. SUN is an advanced wireless communications network designed for reliable, low data rate, and low energy consumption networks for command-and-control applications like utility service, sensor network, and so on. IEEE 802.15.4g/4e is the international standard for SUN supported by multiple utility providers and product vendors. In this paper, a comprehensive field test was conducted by employing the implementation we have developed to evaluate the performance of the SUN devices based on IEEE 802.15.4/4g/4e standard. The output power of the implementation is 250 mW for extended range, reducible to 20 mW for short-range scalability and battery preservation. Results showed that in an outdoor line-of-sight environment, the achievable one-hop range of a 50 kbps SUN device was 450 m. Next, in a non-line-of-sight environment involving typical residential concrete building, the communications could be established penetrating obstructions to reach above the 11th storey, reaching the performance degradation limits at the 20th storey. Next, the network of the SUN system was proven to be capable of supporting a typical multihop tree network in a dense populated building, meeting the required performance by the standard.

  10. Proteomics Core

    Data.gov (United States)

    Federal Laboratory Consortium — Proteomics Core is the central resource for mass spectrometry based proteomics within the NHLBI. The Core staff help collaborators design proteomics experiments in a...

  11. Proteomics Core

    Data.gov (United States)

    Federal Laboratory Consortium — Proteomics Core is the central resource for mass spectrometry based proteomics within the NHLBI. The Core staff help collaborators design proteomics experiments in...

  12. G4 Advanced Education.

    Science.gov (United States)

    1987-06-29

    various social sectors. The various levels of educational administrative and management departments must strengthen and improve the macroscopic guidance...long existed: the state appropriates money and assigns per- sonnel, running the whole show. Therefore, as far as various levels of educational ... administrative ministries are concerned, the cost for cul- tivating one student is none of their business, and after all, it is the state that bear’s the

  13. Progress in diagnosis and treatment of IgG4-related hepatobiliary diseases%lgC4相关肝胆疾病诊疗进展

    Institute of Scientific and Technical Information of China (English)

    李萍; 李永哲

    2014-01-01

    IgG4相关性疾病是一类以淋巴浆细胞性炎症为主,伴血清和组织中IgG4增多并累及多器官或组织的慢性进行性全身性炎性疾病,累及肝胆胰腺系统时称之为IgG4相关肝胆疾病。简述了IgG4相关自身免疫性胰腺炎、IgG4相关硬化性胆管炎、IgG4相关自身免疫性肝炎的临床表现和实验室检查等方面。准确识别IgG4相关肝胆疾病的各个实验室结果,并正确诊断此类疾病,有助于避免不必要的手术和错误的治疗。%IgG4-related diseases are a class of chronic,progressive,systemic inflammatory disorders that are characterized by lymphoplas-macytic inflammation,as well as elevated IgG4 levels in serum and tissue,and may involve multiple organs or tissues.When involving the pancreas,liver,and biliary tracts,they are called IgG4-related hepatobiliary diseases.IgG4-related autoimmune pancreatitis,IgG4-re-lated sclerosing cholangitis,and IgG4-associated autoimmune hepatitis are reviewed in terms of their clinical manifestations and laboratory findings.Accurate identification of the laboratory results for IgG4-related hepatobiliary diseases and correct diagnosis of these diseases help to avoid unnecessary surgery and wrong treatment.

  14. The research about the relationship between membranous nephropathy and Phospholipase A2R、 IgG4%膜性肾病与磷脂酶A2R、IgG4的相关性

    Institute of Scientific and Technical Information of China (English)

    王琳

    2015-01-01

    据现有的文献资料显示,我国膜性肾病发病率较低,约占原发性肾小球疾病的9.9% ~ 13.5%[1],居于IgA肾病、系膜增生性肾小球肾炎之后,位列第三.在临床工作和文献报道中,近年来膜性肾病呈现出逐年增高的趋势,并且多见于老年人.目前对于膜性肾病发病机制的研究仍旧是一个焦点,对于IgG4、PLA2R的研究有可能为鉴别特发性膜性肾病和继发性膜性肾病以及膜性肾病病情活动及复发提供无创性手段.因此对于膜性肾病与PLA2R、IgG4相关性分析的研究,仍是目前国内外肾内科学的热门话题.

  15. The application of drilling fluid technology in Gabon G4-188 block%加蓬G4-188区块钻井液技术应用

    Institute of Scientific and Technical Information of China (English)

    赵炬肃; 马春松

    2011-01-01

    加蓬G4-188区块钻井施工中遇到的技术难点和问题较多,主要反映在表层流砂层垮塌、盐岩层垮塌、地层研磨性强等方面.经几口井的施工和研究分析,摸索出该区块地层压力系统分布情况和井眼失稳的基本规律,通过钻井液技术优化配方和确定的技术措施,采用聚合醇两性离子钻井液与完井液体系,较好地解决了G4-188区块易塌地层井壁失稳技术难题,为钻井施工提供了技术保障.

  16. Impacts, effectiveness and regional inequalities of the GeoMIP G1 to G4 solar radiation management scenarios

    Science.gov (United States)

    Yu, Xiaoyong; Moore, John C.; Cui, Xuefeng; Rinke, Annette; Ji, Duoying; Kravitz, Ben; Yoon, Jin-Ho

    2015-06-01

    We evaluate the effectiveness and the regional inequalities of solar radiation management (SRM) in compensating for simultaneous changes in temperature and precipitation caused by increased greenhouse gas concentrations. We analyze the results from Earth System Models under four Geoengineering Model Intercomparison Project (GeoMIP) experiments with a modified form of the Residual Climate Response approach. Each experiment produces 50 model yrs of simulations: 13 models completed experiment G1 (offsetting 4 × CO2 via solar reduction); 12 models completed experiment G2 (offsetting CO2 that increased by 1% per year); 3 models completed experiment G3 (offsetting increasing radiative forcing under RCP4.5 with increasing stratospheric aerosol); and 7 models completed experiment G4 (injection of 5 Tg SO2 a- 1 into the stratosphere). The regional inequalities in temperature and precipitation compensation for experiments G1, G3 and G4 are significantly different from their corresponding noise backgrounds for most models, but for G2 they are not significantly different from noise. Differences in the regional inequalities and the actual effectiveness among the four SRM scenarios are not significant for many models. However, in more than half of the models, the effectiveness for temperature in the solar dimming geoengineering scenarios (G1 and G2) is significantly higher than that in the SO2 geoengineering scenarios (G3 and G4). The effectiveness of the four SRM experiments in compensating for temperature change is considerably higher than for precipitation. The methodology used highlights that a large across-model variation in the treatment of key geoengineering processes (such as stratospheric aerosols) and the quantification of damage caused by climate change creates significant uncertainties in any strategies to achieve optimal compensation effectiveness across different regions.

  17. Molecular characterization of a human immunoglobulin G4 antibody specific for the major birch pollen allergen, Bet v 1.

    Science.gov (United States)

    Flicker, S; Steinberger, P; Eibensteiner, P B; Lebecque, S; Kraft, D; Valenta, R

    2008-02-01

    Allergen-specific IgG4 antibodies induced by specific immunotherapy are thought to represent a protective immune response. Objective Our aim was the molecular characterization of a human IgG4 antibody (BAB5) specific for the major birch pollen allergen Bet v 1 that was derived from an immunotherapy-treated patient. The cDNA coding for BAB5 was obtained by reverse transcriptase-PCR from the BAB5-producing cell line, compared with the germ line sequences and was expressed as a soluble antibody fragment in Escherichia coli. The epitope specificity and cross-reactivity of BAB5 were investigated with recombinant and synthetic Bet v 1 fragments and Bet v 1 homologous allergens from pollen. The ability of BAB5 to block allergic patients IgE was determined by competition experiments and sandwich ELISA. BAB5 is an affinity-matured Bet v 1-specific IgG4 antibody that reacts exclusively with Bet v 1 but not with Bet v 1-related allergens. Unlike an earlier-described monoclonal IgG1-blocking antibody, BAB1, which had been isolated from the same patient, BAB5 did not block allergic patients' IgE reactivity to Bet v 1. Our study demonstrates that not all allergen-specific IgG antibodies inhibit IgE recognition of allergens and can contribute to the success of immunotherapy. The epitope specificity and affinity of IgG antibodies but not their isotype are decisive for their protective activity.

  18. Infraorbital nerve involvement on magnetic resonance imaging in European patients with IgG4-related ophthalmic disease: a specific sign

    Energy Technology Data Exchange (ETDEWEB)

    Soussan, J. Ben; Sadik, J.C.; Savatovsky, J.; Heran, F.; Lecler, A. [Fondation Ophtalmologique Adolphe de Rothschild, Department of Radiology, Paris (France); Deschamps, R. [Fondation Ophtalmologique Adolphe de Rothschild, Department of Neurology, Paris (France); Deschamps, L. [Bichat Hospital, APHP, Department of Pathology, Paris (France); Puttermann, M. [Necker-Enfants Malades Hospital, APHP, Department of Pathology, Paris (France); Zmuda, M.; Galatoire, O. [Fondation Ophtalmologique Adolphe de Rothschild, Department of Orbitopalpebral Surgery, Paris (France); Picard, H. [Fondation Ophtalmologique Adolphe de Rothschild, Clinical Research Unit, Paris (France)

    2017-04-15

    To measure the frequency of infraorbital nerve enlargement (IONE) on magnetic resonance imaging (MRI) in European patients suffering from an IgG4-related ophthalmic disease (IgG4-ROD) as compared to patients suffering from non-IgG4-related ophthalmic disease (non-IgG4-ROD). From January 2006 through April 2015, 132 patients were admitted for non-lymphoma, non-thyroid-related orbital inflammation. Thirty-eight had both pre-therapeutic orbital MRI and histopathological IgG4 immunostaining. Fifteen patients were classified as cases of IgG4-ROD and 23 patients as cases of non-IgG4-ROD. Two readers performed blinded analyses of MRI images. The main criterion was the presence of an IONE, defined as the infraorbital nerve diameter being greater than the optic nerve diameter in the coronal section. IONE was present in 53% (8/15) of IgG4-ROD cases whereas it was never present (0/23) in cases of non-IgG4-ROD (P < 0.0001). IONE was only present in cases where, on MRI, the inflammation of the inferior quadrant was present and in direct contact with the ION canal. In European patients suffering from orbital inflammation, the presence of IONE on an MRI is a specific sign of IgG4-ROD. Recognition of this pattern may facilitate the accurate diagnosis for clinicians and allow for the adequate management and appropriate care of their patients. (orig.)

  19. IgG4相关自身免疫性胰腺炎的临床特点%Clinical features of IgG4 related autoimmune pancreatitis

    Institute of Scientific and Technical Information of China (English)

    张锐; 曾弘; 余先焕; 唐启彬; 王捷; 刘超

    2014-01-01

    目的:探讨IgG4相关自身免疫性胰腺炎(AIP)的临床特点。方法回顾性研究2003年1月至2012年12月在中山大学孙逸仙纪念医院肝胆胰外科接受诊治并经病理学确诊的12例IgG4相关AIP患者临床资料。所有患者均签署知情同意书,符合医学伦理学规定。患者均为男性,平均年龄为(54±13)岁,均被误诊为胰头癌行胰头十二指肠切除术。收集患者临床表现及实验室、影像学、病理学检查等资料。患者术后接受随访,观察治疗情况及疗效。结果12例患者中腹痛7例、黄疸7例、消瘦6例、无明显症状1例,合并糖尿病3例、慢性下颌下腺炎1例。患者血、尿淀粉酶均正常,血GGT升高12例、糖链抗原19-9(CA19-9)轻度升高9例、癌抗原(CA)125升高3例、癌胚抗原(CEA)升高2例。增强CT示胰头局部肿大11例,伴胰管轻度扩张3例。MRI示胰体呈“腊肠样”改变4例,胰周呈“包鞘样”改变5例。磁共振胰胆管成像(MRCP)示胰头段胰管及胆总管下段狭窄,其中4例远端胰管轻度扩张。影像学检查未发现局部侵犯。病理学检查表现为淋巴浆细胞硬化性胰腺炎,免疫组织化学方法(免疫组化法)染色IgG4阳性。12例术后患者间断出现腹痛,其中7例需服用止痛药。1例患者经泼尼松治疗后症状缓解。结论 IgG4相关AIP临床表现与胰腺癌相似,易误诊为胰腺癌。其主要临床特点为血CA19-9轻度升高;影像学检查见胰头局部肿大、胰体呈“腊肠样”、胰周呈“包鞘样”改变,且无局部侵犯;病理学检查为淋巴浆细胞硬化性胰腺炎,免疫组化法染色IgG4阳性;肾上腺皮质激素治疗有效。%Objective To investigate the clinical features of IgG4 related autoimmune pancreatitis (AIP). Methods Clinical data of 12 patients pathologically diagnosed as IgG4 related AIP in Department of Hepatopancreatobiliary Surgery

  20. Multi-organ involvement of an immunoglobulin G4-related inflammatory pseudotumor of the urogenital tract: A case report

    Energy Technology Data Exchange (ETDEWEB)

    Chun, Ka Young; Jung, Yoon Young; Kim, Yun Jung; Joo, Jong Eun; Yoo, Tag Keun; You, Myung Won; Choi, Yun Sun [Eulji Hospital, Eulji University School of Medicine, Seoul (Korea, Republic of)

    2015-12-15

    Inflammatory pseudotumor (IPT) is a rare solid tumor of unknown etiology that can arise in most organs. IPT usually presents as a single, benign lesion. In the urogenital tract, IPT frequently occurs in the bladder, but in rare instances, IPT may originate in the kidney, prostate, or ureter. We describe a highly unusual case of multi-organ IPT that included the periureteral area, paravesical space, and prostate. The diagnosis was confirmed by computed tomography imaging, and by pathology testing that detected prominent immunoglobulin G4-positive plasma cells.

  1. An IgG4-Related Salivary Gland Disorder: A Case Series Presenting with a Different Clinical Setting

    Directory of Open Access Journals (Sweden)

    Masayuki Ishida

    2011-01-01

    Full Text Available Küttner tumor is a chronic inflammatory disease that presents with a firm swelling of the submandibular gland and often mimics a neoplasm. Recently evidence suggests that Küttner tumor may be a type of disorder characterized by IgG4-related inflammations. Herein, we report 3 cases of submandibular gland swellings with severe fibrosis, inflammation with marked lymphoplasmacytic infiltration; this pathology mimics clinical manifestation of a malignant tumor in 18-fluorodeoxyglucose positron emission tomography (FDG-PET findings.

  2. Molecular mechanisms for maintenance of G-rich short tandem repeats capable of adopting G4 DNA structures

    Energy Technology Data Exchange (ETDEWEB)

    Nakagama, Hitoshi [Biochemistry Division, National Cancer Center Research Institute, 5-1-1 Tsukiji, Chuo-ku, Tokyo 104-0045 (Japan)]. E-mail: hnakagam@gan2.res.ncc.go.jp; Higuchi, Kumiko [Biochemistry Division, National Cancer Center Research Institute, 5-1-1 Tsukiji, Chuo-ku, Tokyo 104-0045 (Japan); Tanaka, Etsuko [Biochemistry Division, National Cancer Center Research Institute, 5-1-1 Tsukiji, Chuo-ku, Tokyo 104-0045 (Japan); Tsuchiya, Naoto [Biochemistry Division, National Cancer Center Research Institute, 5-1-1 Tsukiji, Chuo-ku, Tokyo 104-0045 (Japan); Nakashima, Katsuhiko [Biochemistry Division, National Cancer Center Research Institute, 5-1-1 Tsukiji, Chuo-ku, Tokyo 104-0045 (Japan); Katahira, Masato [Biochemistry Division, National Cancer Center Research Institute, 5-1-1 Tsukiji, Chuo-ku, Tokyo 104-0045 (Japan); Fukuda, Hirokazu [Biochemistry Division, National Cancer Center Research Institute, 5-1-1 Tsukiji, Chuo-ku, Tokyo 104-0045 (Japan)

    2006-06-25

    Mammalian genomes contain several types of repetitive sequences. Some of these sequences are implicated in various specific cellular events, including meiotic recombination, chromosomal breaks and transcriptional regulation, and also in several human disorders. In this review, we document the formation of DNA secondary structures by the G-rich repetitive sequences that have been found in several minisatellites, telomeres and in various triplet repeats, and report their effects on in vitro DNA synthesis. d(GGCAG) repeats in the mouse minisatellite Pc-1 were demonstrated to form an intra-molecular folded-back quadruplex structure (also called a G4' structure) by NMR and CD spectrum analyses. d(TTAGGG) telomere repeats and d(CGG) triplet repeats were also shown to form G4' and other unspecified higher order structures, respectively. In vitro DNA synthesis was substantially arrested within the repeats, and this could be responsible for the preferential mutability of the G-rich repetitive sequences. Electrophoretic mobility shift assays using NIH3T3 cell extracts revealed heterogeneous nuclear ribonucleoprotein (hnRNP) A1 and A3, which were tightly and specifically bound to d(GGCAG) and d(TTAGGG) repeats with K {sub d} values in the order of nM. HnRNP A1 unfolded the G4' structure formed in the d(GGCAG) {sub n} and d(TTAGGG) {sub n} repeat regions, and also resolved the higher order structure formed by d(CGG) triplet repeats. Furthermore, DNA synthesis arrest at the secondary structures of d(GGCAG) repeats, telomeres and d(CGG) triplet repeats was efficiently repressed by the addition of hnRNP A1. High expression of hnRNPs may contribute to the maintenance of G-rich repetitive sequences, including telomere repeats, and may also participate in ensuring the stability of the genome in cells with enhanced proliferation. Transcriptional regulation of genes, such as c-myc and insulin, by G4 sequences found in the promoter regions could be an intriguing field of

  3. G4-DNA formation in the HRAS promoter and rational design of decoy oligonucleotides for cancer therapy.

    Directory of Open Access Journals (Sweden)

    Alexandro Membrino

    Full Text Available HRAS is a proto-oncogene involved in the tumorigenesis of urinary bladder cancer. In the HRAS promoter we identified two G-rich elements, hras-1 and hras-2, that fold, respectively, into an antiparallel and a parallel quadruplex (qhras-1, qhras-2. When we introduced in sequence hras-1 or hras-2 two point mutations that block quadruplex formation, transcription increased 5-fold, but when we stabilized the G-quadruplexes by guanidinium phthalocyanines, transcription decreased to 20% of control. By ChIP we found that sequence hras-1 is bound only by MAZ, while hras-2 is bound by MAZ and Sp1: two transcription factors recognizing guanine boxes. We also discovered by EMSA that recombinant MAZ-GST binds to both HRAS quadruplexes, while Sp1-GST only binds to qhras-1. The over-expression of MAZ and Sp1 synergistically activates HRAS transcription, while silencing each gene by RNAi results in a strong down-regulation of transcription. All these data indicate that the HRAS G-quadruplexes behave as transcription repressors. Finally, we designed decoy oligonucleotides mimicking the HRAS quadruplexes, bearing (R-1-O-[4-(1-Pyrenylethynyl phenylmethyl] glycerol and LNA modifications to increase their stability and nuclease resistance (G4-decoys. The G4-decoys repressed HRAS transcription and caused a strong antiproliferative effect, mediated by apoptosis, in T24 bladder cancer cells where HRAS is mutated.

  4. Retroperitoneal fibrosis associated with immunoglobulin g4-related disease in the differential diagnosis in retroperitoneal tumors. Case report.

    Science.gov (United States)

    Ruiz Mar, Gabriela; Cárdenas Serrano, Óscar E; Roldan García, Jorge; Cañavera-Constantino, A; Menéndez Trejo, Víctor M; Chapa Azuela, Óscar

    2017-07-19

    The patient was a 55-year-old woman with a 4-month history of pain in left flank that irradiated to the lumbar region. Computed tomography revealed a cystic tumor in the retroperitoneal region that compresses the ureter and left renal pelvis in contact with pancreatic body and tail. Surgery was performed and the definitive pathological report diagnosed nodular retroperitoneal fibrosis associated with IgG4; Ki67-positive in germinal centers (5%) and IgG4-positive (40 plasma cells in 3 fields of 40x) by immunohistochemistry. Retroperitoneal fibrosis is a rare disease, that develops gradually and has an excellent response to steroid management. Surgical treatment is reserved for cases that compromise adjacent structures. Thus, identifying it when studying a retroperitoneal tumor leads to better prognosis and survival. Copyright © 2017 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved.

  5. Shedding lights on the flexible-armed porphyrins: Human telomeric G4 DNA interaction and cell photocytotoxicity research.

    Science.gov (United States)

    Sun, Xiang-Yu; Zhao, Ping; Jin, Shu-Fang; Liu, Min-Chao; Wang, Xia-Hong; Huang, Yu-Min; Cheng, Zhen-Feng; Yan, Si-Qi; Li, Yan-Yu; Chen, Ya-Qing; Zhong, Yan-Mei

    2017-08-01

    DNA polymorphism exerts a fascination on a large scientific community. Without crystallographic structural data, clarification of the binding modes between G-quadruplex (G4) and ligand (complex) is a challenging job. In the present work, three porphyrin compounds with different flexible carbon chains (arms) were designed, synthesized and characterized. Their binding, folding and stabilizing abilities to human telomeric G4 DNA structures were comparatively researched. Positive charges at the end of the flexible carbon chains seem to be favorable for the DNA-porphyrin interactions, which were evidenced by the spectral results and further confirmed by the molecular docking calculations. Biological function analysis demonstrated that these porphyrins show no substantial inhibition to Hela, A549 and BEL 7402 cancer cell lines under dark while exhibit broad inhibition under visible light. This significantly enhanced photocytotoxicity relative to the dark control is an essential property of photochemotherapeutic agents. The feature of the flexible arms emerges as critical influencing factors in the cell photocytotoxicity. Moreover, an ROS-mediated mitochondrial dysfunction pathway was suggested for the cell apoptosis induced by these flexible-armed porphyrins. It is found that the porphyrins with positive charges located at the end of the flexible arms represent an exciting opportunity for photochemotherapeutic anti-cancer drug design. Copyright © 2017. Published by Elsevier B.V.

  6. IgE and IgG4 Epitope Mapping of Food Allergens with a Peptide Microarray Immunoassay.

    Science.gov (United States)

    Martínez-Botas, Javier; de la Hoz, Belén

    2016-01-01

    Peptide microarrays are a powerful tool to identify linear epitopes of food allergens in a high-throughput manner. The main advantages of the microarray-based immunoassay are the possibility to assay thousands of targets simultaneously, the requirement of a low volume of serum, the more robust statistical analysis, and the possibility to test simultaneously several immunoglobulin subclasses. Among them, the last one has a special interest in the field of food allergy, because the development of tolerance to food allergens has been associated with a decrease in IgE and an increase in IgG4 levels against linear epitopes. However, the main limitation to the clinical use of microarray is the automated analysis of the data. Recent studies mapping the linear epitopes of food allergens with peptide microarray immunoassays have identified peptide biomarkers that can be used for early diagnosis of food allergies and to predict their severity or the self-development of tolerance. Using this approach, we have worked on epitope mapping of the two most important food allergens in the Spanish population, cow's milk and chicken eggs. The final aim of these studies is to define subsets of peptides that could be used as biomarkers to improve the diagnosis and prognosis of food allergies. This chapter describes the protocol to produce microarrays using a library of overlapping peptides corresponding to the primary sequences of food allergens and data acquisition and analysis of IgE- and IgG4-binding epitopes.

  7. Assessment of pathologically diagnosed patients with Castleman's disease associated with diffuse parenchymal lung involvement using the diagnostic criteria for IgG4-related disease.

    Science.gov (United States)

    Ogoshi, Takaaki; Kido, Takashi; Yatera, Kazuhiro; Oda, Keishi; Kawanami, Toshinori; Ishimoto, Hiroshi; Sakamoto, Noriho; Sano, Arisa; Yoshii, Chiharu; Shimajiri, Shohei; Mukae, Hiroshi

    2013-12-01

    IgG4-related disease (IgG4RD) is a recently recognized disease entity. Differentiating IgG4RD from plasma cell type Castleman's disease (PCD) is important but also difficult using only pathological findings. In addition, little is known about the association between these two diseases with diffuse parenchymal lung involvement. We analyzed the serum IgG4 levels and the ratio of IgG4/IgG-positive plasmacytes in the lung and lymph node specimens of eight patients previously pathologically diagnosed of PCD with diffuse parenchymal lung involvement (DL-PCD). We also compared the clinical and laboratory findings observed in these patients. Six of the eight patients exhibited abundant IgG4-positive plasmacytes in the lung and lymph node tissues and elevated serum IgG4 levels, thereby fulfilling the diagnostic criteria of IgG4RD with DL (DL-IgG4RD) in addition to having obstructive phlebitis and massive lymphoplasmacytic infiltration with fibrosis. However, three of these six patients exhibited higher levels of serum interleukin-6 and were still diagnosed with DL-PCD. Accordingly, three of these eight patients were considered as IgG4RD with DL (DL-IgG4RD), and the other five patients were ultimately given a diagnosis of DL-PCD. These two diseases have different characteristics in terms of age, symptoms, serum levels of C-reactive protein, and IgA, complicating allergic disorders, response to corticosteroids, and prognosis. This is the first report to show a high prevalence of DL-IgG4RD in DL-PCD patients, although additional large investigations are necessary. Clinical and laboratory findings are important for distinguishing between these two diseases in other organs, as previously described.

  8. Hepatic nodular lymphoid lesion with increased IgG4-positive plasma cells associated with primary biliary cirrhosis: a report of two cases.

    Science.gov (United States)

    Calvo, Jessica; Carbonell, Nicolas; Scatton, Olivier; Marzac, Christophe; Ganne-Carrie, Nathalie; Wendum, Dominique

    2015-11-01

    The nodular lymphoid lesion of the liver known as reactive lymphoid hyperplasia or pseudolymphoma is rare and its pathogenesis is unknown. We report two cases of nodular lymphoid lesions of the liver with numerous IgG4-positive plasma cells in patients with primary biliary cirrhosis. Histologically, in both cases, the lesion showed a dense lymphoplasmacytic infiltrate with lymphoid follicles and granulomas. Fibrous tissue was scarce and without a storiform pattern. Obliterative phlebitis was not identified. The IgG4+ plasma cell counts were 82 and 76 per high power field, with an IgG4/IgG ratio of 75 and 64 %, respectively, which qualifies the lesions according to the diagnostic criteria for IgG4-related disease as « probable histological feature of IgG4-related disease ». There were no rearrangements of immunoglobulin heavy-chain genes and plasma cells had a polytypic pattern of kappa and lambda light-chain expression. The non-tumor liver showed primary biliary cirrhosis with destructive cholangitis without IgG4 plasma cells. In both cases, IgG4-related disease was not found in other organs neither at the time of diagnosis nor 3 years later. Serum IgG4 levels normalized after local ablation of the lesions. It seems unlikely that these lesions are a manifestation of IgG4-related disease. However, because the pathogenesis of both nodular lymphoid lesions and IgG4-related disease remains unclear, further studies are needed to elucidate a potential link between nodular lymphoid lesions of the liver and an increased number of IgG4 plasma cells. More definite conclusions will be possible when the pathogenesis of IgG4-related disease has been clarified.

  9. Characteristics of oil and gas distribution of belt WZ in Gabon block G4-188%加蓬G4-188区块WZ构造带含油气特征

    Institute of Scientific and Technical Information of China (English)

    吴德军

    2011-01-01

    加蓬G4-188区块WZ构造带有良好的油气显示,现已从勘探工作阶段转入准开发的关键时期.基于区域地质资料,结合现场地质录井、测井及后期试油等相关资料,对该构造带各层位的油气显示及分布特征进行了总结,探讨了每一层位的含油气特征及区域分布特点.研究表明:PG组为非油气产层;U.PC组为典型的产气层;L.PC组和Anguille组油气显示良好,且地层压力较高,为典型的高压油层.为该区的开发和邻区的进一步勘探提供建议.%Oil and gas is abundant in belt WZ of Garbon block G4-188. The focus is moving from exploration to exploitation which is more important. Based on the areal geology data, combining with data of drill program and mudlogging,analyze oil and gas shows and distribution in every position. The results show that PG group isn't oil and gas producing formation, U. PC group is typical gas producing formation. L. PC group and Anguille group are typical high pressure reservoirs which have better oil and gas shows and formation pressure is higher. The study provides the reference for the development of this block and the exploration of other blocks.

  10. [IgG4-related disease and clonal B-cell lymphoid proliferation: Description of two clinical cases and a review of literature].

    Science.gov (United States)

    Sokol, E V; Vasilyev, V I; Kovrigina, A M; Safonova, T N; Nasonov, E L

    2015-01-01

    IgG4-related disease (IgG4-RD) is a systemic immune-related disease that may involve the pancreas, liver, retroperitoneal space, biliary tract, salivary and lacrimal glands, eye socket, lung, and kidney. In term of pathomorphogenesis, it is a fibroinflammatory disease manifesting as a tumor-like lesion of organs, elevated serum IgG4 levels, and a morphofunctional substrate - the development of marked fibrosis and lymphoplasmacytic infiltration in the tissues with the high content of IgG4-positive plasma cells. The detection of a tumor-like nodule frequently leads to that the patients with IgG4-RD undergo major traumatic surgery for presumed cancer. At the same time, a number of investigations show the association of IgG4-RD with the development of cancer and lymphoproliferative diseases. The paper describes two clinical cases: Russia's first diagnosis of MALT lymphoma of the lacrimal gland, IgG4-positive and IgG4-RD with a rare onset with a destruction focus in the cervical vertebrae, multiple organ dysfunction, B-cell clonality in salivary gland tissue and PIgMκ secretion. It also reviews world literature on the development of lymphoproliferative diseases in the presence of IgG4-RD.

  11. @@%IgG4相关性肾病与特发性膜性肾病的关系

    Institute of Scientific and Technical Information of China (English)

    张沛

    2014-01-01

    @@%目前研究认为,IgG4相关性肾病(IgG4-related kidney disease,IgG4-RKD)与特发性膜性肾病(idiopathic membranous nephropathy,IMN)的发病过程均有IgG4参与,二者存在一定的区别与联系.本文试从发病机制、临床表现、病理改变、治疗策略等方面进行综述,对两种疾病的关系进行探讨.

  12. A case of proteinase 3 anti-neutrophil cytoplasmic antibody (PR3-ANCA positive/IgG4-related lung disease

    Directory of Open Access Journals (Sweden)

    Hirokazu Touge

    2017-01-01

    Full Text Available IgG4-related lung disease (IgG4-RLD is a rare and chronic progressive autoimmune disease. We report a case of IgG4-related inflammatory pseudo-tumor of the lung that was seropositive for proteinase 3 anti-neutrophil cytoplasmic antibody (PR3-ANCA. A 61-year-old male had a mass lesion in the right lower lung field in chest X-ray. Transbronchial lung biopsy resulted in a pathological diagnosis of IgG4-RLD. The condition was improved by hormonal therapy.

  13. IgG4-related disease presenting as a lung mass and weight loss: Case report and review of the literature

    Directory of Open Access Journals (Sweden)

    Kevin Grewal

    2016-01-01

    Full Text Available We describe a case of IgG4-related lung disease presenting as a lung mass with associated weight loss. IgG4-related disease is a systemic sclerosing disorder that causes fibrotic, often tumor-like manifestations that variably effect different organ systems. The clinical presentation of IgG4-related disease is protean. Timely recognition and diagnosis requires awareness on the part of clinicians and pathologists to the variable manifestations of this newly recognized disorder. We offer a concise review of the pulmonary manifestations, diagnosis and treatment of IgG4-related lung disease.

  14. Performance evaluation of a CyberKnife® G4 image-guided robotic stereotactic radiosurgery system

    Science.gov (United States)

    Antypas, Christos; Pantelis, Evaggelos

    2008-09-01

    The aim of the current work was to present the performance evaluation procedures implemented at our department for the commissioning of a G4 CyberKnife system. This system consists of a robotic manipulator, a target-locating system and a lightweight 6-MV linac. Individual quality assurance procedures were performed for each of the CyberKnife subsystems. The system was checked for the mechanical accuracy of its robotic manipulator. The performance of the target-locating system was evaluated in terms of mechanical accuracy of both cameras' alignment and quality assurance tests of the x-ray generators and the flat-panel detectors. The traditional linac 6-MV beam characteristics and beam output parameters were also measured. Results revealed a manipulator mechanical mean accuracy of ~0.1 mm, with individual maximum position uncertainties less than 0.25 mm. The target-locating system mechanical accuracy was found within the acceptance limits. For the most clinically used parameters in the CyberKnife practice, e.g. 100-120 kV and 50-200 ms, kV and exposure time accuracy error were measured as less than 2%, while the precision error of the kV was determined as less than 1%. The acquired images of the ETR grid pattern revealed no geometrical distortion while the critical frequency f50 values for cameras A and B were calculated as 1.5 lp mm-1 and 1.4 lp mm-1, respectively. Dose placement measurements were performed in a head and neck phantom. Results revealed sub-millimeter beam delivery precision whereas the total clinical accuracy of the system was measured equal to 0.44 ± 0.12 mm, 0.29 ± 0.10 mm and 0.53 ± 0.16 mm for the skull, fiducial and Xsight spine tracking methods, respectively. The results of this work certify the G4 CyberKnife SRS system capable of delivering high dose distributions with sub-millimeter accuracy and precision to intracranial and extracranial lesions. Moreover, total clinical accuracy of the investigated G4 system was found to be improved for

  15. Performance evaluation of a CyberKnife (registered) G4 image-guided robotic stereotactic radiosurgery system

    Energy Technology Data Exchange (ETDEWEB)

    Antypas, Christos; Pantelis, Evaggelos [Medical Physics Department, Iatropolis, Magnitiki Tomografia, Clinic and Diagnostic Center, Ethnikis Antistaseos 54-56, 15231 Athens (Greece)], E-mail: cantypas@med.uoa.gr

    2008-09-07

    The aim of the current work was to present the performance evaluation procedures implemented at our department for the commissioning of a G4 CyberKnife system. This system consists of a robotic manipulator, a target-locating system and a lightweight 6-MV linac. Individual quality assurance procedures were performed for each of the CyberKnife subsystems. The system was checked for the mechanical accuracy of its robotic manipulator. The performance of the target-locating system was evaluated in terms of mechanical accuracy of both cameras' alignment and quality assurance tests of the x-ray generators and the flat-panel detectors. The traditional linac 6-MV beam characteristics and beam output parameters were also measured. Results revealed a manipulator mechanical mean accuracy of {approx}0.1 mm, with individual maximum position uncertainties less than 0.25 mm. The target-locating system mechanical accuracy was found within the acceptance limits. For the most clinically used parameters in the CyberKnife practice, e.g. 100-120 kV and 50-200 ms, kV and exposure time accuracy error were measured as less than 2%, while the precision error of the kV was determined as less than 1%. The acquired images of the ETR grid pattern revealed no geometrical distortion while the critical frequency f{sub 50} values for cameras A and B were calculated as 1.5 lp mm{sup -1} and 1.4 lp mm{sup -1}, respectively. Dose placement measurements were performed in a head and neck phantom. Results revealed sub-millimeter beam delivery precision whereas the total clinical accuracy of the system was measured equal to 0.44 {+-} 0.12 mm, 0.29 {+-} 0.10 mm and 0.53 {+-} 0.16 mm for the skull, fiducial and Xsight spine tracking methods, respectively. The results of this work certify the G4 CyberKnife SRS system capable of delivering high dose distributions with sub-millimeter accuracy and precision to intracranial and extracranial lesions. Moreover, total clinical accuracy of the investigated G4

  16. Decreased Expression of Innate Immunity-Related Genes in Peripheral Blood Mononuclear Cells from Patients with IgG4-Related Disease.

    Directory of Open Access Journals (Sweden)

    Akio Nakajima

    Full Text Available IgG4-related disease (IgG4-RD is a new clinical entity of unknown etiology characterized by elevated serum IgG4 and tissue infiltration by IgG4-positive plasma cells. Although aberrancies in acquired immune system functions, including increases in Th2 and Treg cytokines observed in patients with IgG4-RD, its true etiology remains unclear. To investigate the pathogenesis of IgG4-RD, this study compared the expression of genes related to innate immunity in patients with IgG4-RD and healthy controls.Peripheral blood mononuclear cells (PBMCs were obtained from patients with IgG4-RD before and after steroid therapy and from healthy controls. Total RNA was extracted and DNA microarray analysis was performed in two IgG4-RD patients to screen for genes showing changes in expression. Candidate genes were validated by real-time RT-PCR in 27 patients with IgG4-RD and 13 healthy controls.DNA microarray analysis identified 21 genes that showed a greater than 3-fold difference in expression between IgG4-RD patients and healthy controls and 30 genes that showed a greater than 3-fold change in IgG4-RD patients following steroid therapy. Candidate genes related to innate immunity, including those encoding Charcot-Leyden crystal protein (CLC, membrane-spanning 4-domain subfamily A member 3 (MS4A3, defensin alpha (DEFA 3 and 4, and interleukin-8 receptors (IL8R, were validated by real-time RT-PCR. Expression of all genes was significantly lower in IgG4-RD patients than in healthy controls. Steroid therapy significantly increased the expression of DEFA3, DEFA4 and MS4A3, but had no effect on the expression of CLC, IL8RA and IL8RB.The expression of genes related to allergy or innate immunity, including CLC, MS4A3, DEFA3, DEFA4, IL8RA and IL8RB, was lower in PBMCs from patients with IgG4-RD than from healthy controls. Although there is the limitation in the number of patients applied in DNA microarray, impaired expression of genes related to innate immunity may be

  17. Interleukin-33 produced by M2 macrophages and other immune cells contributes to Th2 immune reaction of IgG4-related disease

    Science.gov (United States)

    Furukawa, Sachiko; Moriyama, Masafumi; Miyake, Kensuke; Nakashima, Hitoshi; Tanaka, Akihiko; Maehara, Takashi; Iizuka-Koga, Mana; Tsuboi, Hiroto; Hayashida, Jun-Nosuke; Ishiguro, Noriko; Yamauchi, Masaki; Sumida, Takayuki; Nakamura, Seiji

    2017-01-01

    IgG4-related disease (IgG4-RD) is characterized by elevated serum IgG4 and marked infiltration of IgG4-positive cells in multiple organs. Interleukin-33 (IL-33) is a recently described cytokine that is secreted by damaged epithelial cells, macrophages, and dendritic cells, and potently activates helper T type 2 (Th2) immune responses, which have been suggested to play a major role in IgG4 production of IgG4-RD. Here, we assessed the expression of IL-33 and related molecules in the salivary glands (SGs) of patients with IgG4-RD versus that in patients with Sjögren’s syndrome (SS) and controls. Expression of IL-33 and its receptor (ST2) was strongly detected around ectopic germinal centers (GCs) in the SGs from patients with IgG4-RD, whereas IL-33 was expressed only in epithelial cells in patients with SS and controls. Moreover, IL-33 and CD68+/CD163+ macrophages were mainly distributed around ectopic GCs in patients with IgG4-RD. Double immunofluorescence staining showed that IL-33 expression co-localized with CD68+/CD163+ macrophages. Finally, mRNA expression levels of IL-33 showed a positive correlation to those of Th2 cytokines (IL-4 and IL-13) in patients with IgG4-RD. Our data suggest that IL-33 produced by M2 macrophages might contribute to the pathogenesis of IgG4-RD via aberrant activation of Th2 immune responses. PMID:28205524

  18. Immunohistochemical analysis of IgA expression differentiates IgG4-related disease from plasma cell-type Castleman disease.

    Science.gov (United States)

    Manabe, Akihiro; Igawa, Takuro; Takeuchi, Mai; Gion, Yuka; Yoshino, Tadashi; Sato, Yasuharu

    2017-03-01

    Plasma cell-type Castleman disease (PCD) is often encountered when differentiating IgG4-related disease (IgG4-RD). Given that serum IgA is often elevated in Castleman disease, we investigated whether IgA expression levels in histological specimens can be used to differentiate between the two diseases. Lymph node lesions obtained from 12 IgG4-RD and 11 PCD patients were analysed by immunohistochemistry with anti-IgG, -IgG4, and -IgA antibodies. In addition to all 12 cases of IgG4-RD, 8/11 cases (72.7 %) of PCD also met the diagnostic criteria of IgG4-RD (serum IgG4 ≥135 mg/dl and IgG4/IgG-positive cells ≥40 %). IgA-positive cells were sparsely and densely distributed in IgG4-RD and PCD cases, respectively. The median number of IgA-positive cells ± SD in all 12 cases of IgG4-RD was 31 ± 37 cells per three high-powered fields (3HPFs) (range 4-118 cells/3HPFs). In contrast, the median number of IgA-positive cells, which was significantly higher in all 11 cases of PCD, was 303 ± 238 cells/3HPFs (range 74-737 cells/3HPFs) (P IgA immunostaining can be used for differential diagnosis of IgG4-RD.

  19. Ice cores

    DEFF Research Database (Denmark)

    Svensson, Anders

    2014-01-01

    Ice cores from Antarctica, from Greenland, and from a number of smaller glaciers around the world yield a wealth of information on past climates and environments. Ice cores offer unique records on past temperatures, atmospheric composition (including greenhouse gases), volcanism, solar activity......, dustiness, and biomass burning, among others. In Antarctica, ice cores extend back more than 800,000 years before present (Jouzel et al. 2007), whereas. Greenland ice cores cover the last 130,000 years...

  20. Ice cores

    DEFF Research Database (Denmark)

    Svensson, Anders

    2014-01-01

    Ice cores from Antarctica, from Greenland, and from a number of smaller glaciers around the world yield a wealth of information on past climates and environments. Ice cores offer unique records on past temperatures, atmospheric composition (including greenhouse gases), volcanism, solar activity......, dustiness, and biomass burning, among others. In Antarctica, ice cores extend back more than 800,000 years before present (Jouzel et al. 2007), whereas. Greenland ice cores cover the last 130,000 years...

  1. Autoimmune pancreatitis with IgG4-positive plasma cell infiltration in salivary glands and biliary tract

    Institute of Scientific and Technical Information of China (English)

    Masashi Taguchi; Gentaro Aridome; Shintaro Abe; Keiichiro Kume; Mitsuo Tashiro; Mitsuyoshi Yamamoto; Yasuyuki Kihara; Hayato Nakamura; Makoto Otsuki

    2005-01-01

    A 62-year-old male was referred to our hospital because of liver dysfunction, diffuse pancreatic swelling, and trachelophyma. At admission, the patient was free of pain.Physical examination showed enlarged and palpable bilateral submandibular masses, but no palpable mass or organomegaly in the abdomen. Laboratory findings were as follows: total protein 90 g/L with γ-globulin of 37.3% (33 g/L), total bilirubin 4 mg/L, aspartate aminotransferase 39 IU/L, alanine aminotransferase 67 IU/L, γ-glutamyl transpeptidase 1 647 IU/L, and amylase 135 IU/L. Autoantibodies were negative, and tumor markers were within the normal range. Serum IgG4 level was markedly elevated (18 900 mg/L). Computed tomography (CT) showed diffuse swelling of the pancreas and dilatation of both common and intra-hepatic bile ducts. Endoscopic retrograde pancreatography (ERP) revealed diffuse irregular and narrow main pancreatic duct and stenosis of the lower common bile duct. Biopsy specimens from the pancreas, salivary gland and liver showed marked periductal IgG4-positive plasma cell infiltration with fibrosis. We considered this patient to be autoimmune pancreatitis (AIP) with fibrosclerosis of the salivary gland and biliary tract, prescribed prednisolone at an initial dose of 40 mg/d. Three months later, the laboratory data improved almost to normal. Abdominal CT reflected prominent improvement in the pancreatic lesion. Swelling of the salivary gland also improved. At present, the patient is on 10 mg/d of prednisolone without recurrence of the pancreatitis. We present here a case of AIP with fibrosclerosis of salivary gland and biliary tract.

  2. [Lymphocytic hypophysitis and hypertrophic pachymeningitis: description of a possible case associated to IgG4 pathologies].

    Science.gov (United States)

    Blanco-Cantó, M Empar; Dávila-González, Pablo; López de Silanes, Carlos; Cuadrado-Pérez, M Luz; Ortega, Gloria; Porta-Etessam, Jesús

    2015-06-01

    Introduccion. Las patologias relacionadas con la infiltracion de linfocitos y celulas inflamatorias se clasifican, segun su topografia, en patologia orbitaria idiopatica, paquimeningitis hipertrofica idiopatica, sindrome de Tolosa-Hunt e hipofisitis linfocitaria. Presentamos a una paciente que comenzo con una hipofisitis linfocitaria hace ocho años y ha presentado varios episodios de paquimeningitis hipertrofica variables en su localizacion. Caso clinico. Mujer de 55 años, en seguimiento por cefalea de dos meses de evolucion, que ingreso por empeoramiento clinico con paralisis del III par derecho completo, lagrimeo y rinorrea. Se realizo una resonancia magnetica cerebral que demostro la presencia de una lesion sellar compatible con hipofisitis linfocitaria. Se inicio tratamiento con corticoides con mejoria inicial, pero, tras dos años, la paciente presento empeoramiento clinico con cefalea y parestesias perioculares derechas. Se repitio la resonancia, donde se observaron varias lesiones extraaxiales, siendo el resto de estudios normales, por lo que se diagnostico paquimeningitis hipertrofica idiopatica y se inicio tratamiento de nuevo con corticoides. En el seguimiento posterior, la paciente presento nuevas lesiones en diferentes localizaciones y mal control algico, por lo que se añadio tratamiento inmunomodulador. Ante la asociacion de hipofisitis linfocitaria y paquimeningitis hipertrofica, se completo el estudio con determinacion de la IgG4 en el suero, que resulto negativa. Conclusiones. La asociacion de hipofisitis linfocitaria y paquimeningitis hipertrofica con las patologias relacionadas con la IgG4 se ha descrito recientemente. A pesar de que en nuestra paciente no se ha confirmado el diagnostico, se debe considerar esta relacion en aquellos casos idiopaticos y, sobre todo, si se asocian otras manifestaciones sistemicas.

  3. Idealized marine cloud brightening experiments G4cdnc from the geoengineering model intercomparison project GeoMIP

    Science.gov (United States)

    Weum Stjern, Camilla; Egill Kristjánsson, Jón; Boucher, Olivier; Cole, Jason N. S.; Jones, Andy; Kravitz, Ben; Niemeier, Ulrike; Muri, Helene; Phipps, Steven J.; Watanabe, Shingo

    2017-04-01

    Climate engineering could be considered as part of a response portfolio to contribute to reach such ambitious climate targets as those set by the Paris Agreement. Marine cloud brightening (MCB) is one of these techniques, which falls into the category of solar radiation management, or albedo modification, and aims to cool the climate by increasing the amount of solar radiation reflected by clouds. Existing model assessments of MCB have very different experimental set-ups, making comparison difficult. Therefore, the experiment G4cdnc was designed, in which several Earth system models performed the same perturbation of cloud properties, to assess the climate impacts. The G4cdnc experiment starts in year 2020 in the RCP4.5 scenario and dictates a 50% increase in cloud droplet number concentrations of low level clouds over global oceans for a duration of 50 years. Many of the models significantly underestimate low level cloud amounts; nevertheless, all the models simulate a cooling effect from MCB. The resulting net radiative forcing is of -1.8 Wm^-2 in the ensemble mean with large inter-model spread. The ensemble mean global cooling achieved is of -0.95 K with a particularly strong cooling over low latitude land masses. There is a global precipitation decrease of -0.08 mm/day due to a cooler climate, but in low latitudes there is a 0.03 mm/day increase over land from circulation changes. Inter-model differences can be partly explained by different cloud susceptibilities, but more studies are needed to fully understand the mechanisms involved.

  4. Comprehensive Analysis of the Therapeutic IgG4 Antibody Pembrolizumab: Hinge Modification Blocks Half Molecule Exchange In Vitro and In Vivo.

    Science.gov (United States)

    Yang, Xiaoyu; Wang, Fengqiang; Zhang, Ying; Wang, Larry; Antonenko, Svetlana; Zhang, Shuli; Zhang, Yi Wei; Tabrizifard, Mohammad; Ermakov, Grigori; Wiswell, Derek; Beaumont, Maribel; Liu, Liming; Richardson, Daisy; Shameem, Mohammed; Ambrogelly, Alexandre

    2015-12-01

    IgG4 antibodies are evolving as an important class of cancer immunotherapies. However, human IgG4 can undergo Fab arm (half molecule) exchange with other IgG4 molecules in vivo. The hinge modification by a point mutation (S228P) prevents half molecule exchange of IgG4. However, the experimental confirmation is still expected by regulatory agencies. Here, we report for the first time the extensive analysis of half molecule exchange for a hinge-modified therapeutic IgG4 molecule, pembrolizumab (Keytruda) targeting programmed death 1 (PD1) receptor that was approved for advanced melanoma. Studies were performed in buffer or human serum using multiple exchange partners including natalizumab (Tysabri) and human IgG4 pool. Formation of bispecific antibodies was monitored by fluorescence resonance energy transfer, exchange with Fc fragments, mixed mode chromatography, immunoassays, and liquid chromatography-mass spectrometry. The half molecule exchange was also examined in vivo in SCID (severe combined immunodeficiency) mice. Both in vitro and in vivo results indicate that the hinge modification in pembrolizumab prevented half molecule exchange, whereas the unmodified counterpart anti-PD1 wt showed active exchange activity with other IgG4 antibodies or self-exchange activity with its own molecules. Our work, as an example expected for meeting regulatory requirements, contributes to establish without ambiguity that hinge-modified IgG4 antibodies are suitable for biotherapeutic applications.

  5. The blocking activity of birch pollen-specific immunotherapy-induced IgG4 is not qualitatively superior to that of other IgG subclasses

    DEFF Research Database (Denmark)

    Ejrnaes, Anne M; Bødtger, Uffe; Larsen, Jørgen N;

    2004-01-01

    blocking activity was found in the purified IgG4 fraction. There was no significant difference in the binding avidities (1/K(d)) measured in the two IgG fractions. Thus, it appears that SIT-induced specific IgG4 contributes to the IgG blocking of allergen binding to IgE in a simple quantitative manner...

  6. IgG4相关硬化性疾病头颈部病变的研究进展

    Institute of Scientific and Technical Information of China (English)

    吕晶; 刘红刚

    2012-01-01

    IgG4相关硬化性疾病(IgG4-related sclerosing disease,IgG4-SD)是新近认识的一种不同于一般慢性炎症和其他自身免疫性疾病的独立临床病理实体.该疾病主要累及胰腺,也可与胰腺外多个器官病变并存,头颈部IgG4-SD主要表现为颌下腺(Kuttner瘤)、泪腺和腮腺(Mikulicz病)以及脑下垂体(淋巴浆细胞性垂体炎)疾病等.目前头颈部IgG4-SD的诊断以及鉴别诊断已取得一定的研究进展.

  7. IgG4-related pleural disease diagnosed by a re-evaluation of chronic bilateral pleuritis in a patient who experienced occasional acute left bacterial pleuritis.

    Science.gov (United States)

    Yamamoto, Hiroshi; Suzuki, Toshiro; Yasuo, Masanori; Kobayashi, Orie; Tsushima, Kenji; Ito, Michiko; Urushihata, Kazuhisa; Yamazaki, Yoshitaka; Hanaoka, Masayuki; Koizumi, Tomonobu; Uehara, Takeshi; Kawakami, Satoshi; Hamano, Hideaki; Kawa, Shigeyuki; Kubo, Keishi

    2011-01-01

    A 78-year-old man with cryptogenic chronic bilateral lymphoplasmacytic pleuritis, diagnosed based on left parietal pleural biopsy specimens obtained by pleuroscopy, developed acute left bacterial pleuritis. The left pleural effusion was neutrophil dominant, however, the right pleural effusion showed lymphoplasmacytic infiltration. Laboratory examinations revealed that his serum IgG4 concentration was increased, with a higher level of IgG4 in the right pleural effusion. Re-evaluation of the previous biopsy specimens using an immunostaining method revealed numerous IgG4-positive plasma cell infiltrations with IgG4-positive/IgG-positive plasma cells at 85.4%. Accordingly, the new diagnosis of this patient was considered to be chronic bilateral IgG4-related pleuritis.

  8. Successful treatment of pediatric IgG4 related systemic disease with mycophenolate mofetil: case report and a review of the pediatric autoimmune pancreatitis literature

    Directory of Open Access Journals (Sweden)

    Cron Randy Q

    2011-01-01

    Full Text Available Abstract Autoimmune pancreatitis is frequently associated with elevated serum and tissue IgG4 levels in the adult population, but there are few reports of pediatric autoimmune pancreatitis, and even fewer reports of IgG4 related systemic disease in a pediatric population. The standard of care treatment in adults is systemic corticosteroids with resolution of symptoms in most cases; however, multiple courses of corticosteroids are occasionally required and some patients require long term corticosteroids. In these instances, steroid sparing disease modify treatments are in demand. We describe a 13-year-old girl with IgG4 related systemic disease who presented with chronic recurrent autoimmune pancreatitis resulting in surgical intervention for obstructive hyperbilirubinemia and chronic corticosteroid treatment. In addition, she developed fibrosing medianstinitis as part of her IgG4 related systemic disease. She was eventually successfully treated with mycophenolate mofetil allowing for discontinuation of corticosteroids. This is the first reported use of mycophenolate mofetil for IgG4 related pancreatitis. Although autoimmune pancreatitis as part of IgG4 related systemic disease is rarely reported in pediatrics, autoimmune pancreatitis is also characterized as idiopathic fibrosing pancreatitis. All pediatric autoimmune pancreatitis cases reported in the world medical literature were identified via a PUBMED search and are reviewed herein. Twelve reports of pediatric autoimmune pancreatitis were identified, most of which were treated with corticosteroids or surgical approaches. Most case reports failed to report IgG4 levels, so it remains unclear how commonly IgG4 related autoimmune pancreatitis occurs during childhood. Increased evaluation of IgG4 levels in patients with autoimmune pancreatitis may shed further light on the association of IgG4 with pancreatitis and the underlying pathophysiology.

  9. 加蓬G4-188区块钻井液技术难点与对策%Gabon Block G4-188 Drilling Fluid Technology Difficulties and Countermeasures

    Institute of Scientific and Technical Information of China (English)

    金军斌; 宋明全; 鲍洪志; 于玲玲; 豆宁辉

    2010-01-01

    加蓬G4-188区块存在表层严重漏失引起基础沉降、UPC井段缩径严重阻卡、LPC和ANG井段垮塌掉块扩径、地层承压能力分布不均引起的井漏等钻井液技术难点.为此,提出不同开次采用不同钻井液体系及不同工程技术措施的技术对策:一开采用加入随钻堵漏材料的高黏高切钻井液;二开采用强包被抑制性钻井液;三开采用抑制防塌钻井液;钻进坍塌掉块缩径井段采取防阻卡和防扩径技术措施;钻至预计漏层前采取防漏技术措施;钻进高压地层时采取控制流变性和膨润土含量的技术措施.这些技术措施在SAWZ-5井、SAWZ-6(2)井和SAWZ-7井的现场施工中取得了良好的应用效果.

  10. Topology and regulation of the human eIF4A/4G/4H helicase complex in translation initiation

    Science.gov (United States)

    Marintchev, Assen; Edmonds, Katherine A.; Marintcheva, Boriana; Hendrickson, Elthea; Oberer, Monika; Suzuki, Chikako; Herdy, Barbara; Sonenberg, Nahum; Wagner, Gerhard

    2009-01-01

    Summary The RNA helicase eIF4A plays a key role in unwinding of mRNA and scanning during translation initiation. Free eIF4A is a poor helicase and requires the accessory proteins eIF4G and eIF4H. However, the structure of the helicase complex and the mechanisms of stimulation of eIF4A activity have remained elusive. Here we report the topology of the eIF4A/4G/4H helicase complex, which is built from multiple experimentally observed domain-domain contacts. Remarkably, some of the interactions are continuously rearranged during the ATP binding/hydrolysis cycle of the helicase. We show that the accessory proteins modulate the affinity of eIF4A for ATP by interacting simultaneously with both helicase domains and promoting either the closed, ATP-bound conformation or the open, nucleotide-free conformation. The topology of the complex and the spatial arrangement of the RNA-binding surfaces offer insights into their roles in stimulation of helicase activity and the mechanisms of mRNA unwinding and scanning. PMID:19203580

  11. A novel read-through transcript JMJD7-PLA2G4B regulates head and neck squamous cell carcinoma cell proliferation and survival

    Science.gov (United States)

    Cheng, Yingduan; Wang, Yi; Li, Jiong; Chang, Insoon; Wang, Cun-Yu

    2017-01-01

    Recent findings on the existence of oncogenic fusion genes in a wide array of solid tumors, including head and neck squamous cell carcinoma (HNSCC), suggests that fusion genes have become attractive targets for cancer diagnosis and treatment. In this study, we showed for the first time that a read-through fusion gene JMJD7-PLA2G4B is presented in HNSCC, splicing neighboring jumonji domain containing 7 (JMJD7) and phospholipase A2, group IVB (PLA2G4B) genes together. Ablation of JMJD7-PLA2G4B significantly inhibited proliferation of HNSCC cells by promoting G1 cell cycle arrest and increased starvation-induced cell death compared to JMJD7-only knockdown HNSCC cells. Mechanistically, we found that JMJD7-PLA2G4B modulates phosphorylation of Protein Kinase B (AKT) to promote HNSCC cell survival. Moreover, JMJD7-PLA2G4B also regulated an E3 ligase S-phase kinase-associated protein 2 (SKP2) to control the cell cycle progression from G1 phase to S phase by inhibiting Cyclin-dependent kinase inhibitor 1 (p21) and 1B (p27) expression. Our study provides novel insights into the oncogenic control of JMJD7-PLA2G4B in HNSCC cell proliferation and survival, and suggests that JMJD7-PLA2G4B may serve as an important therapeutic target and prognostic marker for HNSCC development and progression. PMID:28030848

  12. Case report of anti-transcription intermediary factor-1-γ/α antibody-positive dermatomyositis associated with gastric cancer and immunoglobulin G4-positive pulmonary inflammatory pseudotumor.

    Science.gov (United States)

    Ito, Kotaro; Imafuku, Shinichi; Hamaguchi, Yasuhito; Fujimoto, Manabu; Nakayama, Juichiro

    2013-07-01

    Dermatomyositis is a rare connective tissue disease often associated with internal malignancy and interstitial pneumonitis. Serologically, various auto-antibodies (Ab) are associated with dermatomyositis. Anti-transcription intermediary factor-1-γ/α (TIF-1-γ/α) Ab was recently identified as an auto-Ab and was observed mostly in cancer-associated dermatomyositis. IgG4-related disease is a newly described entity characterized by increased serum IgG4 levels and IgG4-positive plasma cell infiltration with fibrosis in organs such as the pancreas and parotid gland. IgG4-related disease also includes inflammatory pseudotumors in various organs. We report herein a 59-year-old Japanese man who had dermatomyositis complicated with a gastric cancer and an IgG4-related pulmonary inflammatory pseudotumor. He manifested typical classical Gottron's papules on the fingers, V-sign erythema on the chest, flagellate erythema on the back, nail fold bleeding and facial erythema. Serum levels of anti-TIF-1-γ/α Ab were positive as assessed by immunoprecipitation assay. He also had bilateral swelling of the parotid gland, and an excised specimen of the lung showed inflammatory pseudotumor with IgG4-positive plasma cells. As far as we know, this case is the first to report the association of IgG4-related disease and TIF-1-γ/α-positive dermatomyositis. Further accumulation of such cases is required to elucidate the mechanism of this association.

  13. Occurrence of anaplastic large cell lymphoma following IgG4-related autoimmune pancreatitis and cholecystitis and diffuse large B-cell lymphoma.

    Science.gov (United States)

    Ishida, Mitsuaki; Hodohara, Keiko; Yoshida, Keiko; Kagotani, Akiko; Iwai, Muneo; Yoshii, Miyuki; Okuno, Hiroko; Horinouchi, Akiko; Nakanishi, Ryota; Harada, Ayumi; Yoshida, Takashi; Okabe, Hidetoshi

    2013-01-01

    IgG4-related sclerosing disease is an established disease entity with characteristic clinicopathological features. Recently, the association between IgG4-related sclerosing disease and the risk of malignancies has been suggested. IgG4-related autoimmune pancreatitis with pancreatic cancer has been reported. Further, a few cases of extraocular malignant lymphoma in patients with IgG4-related sclerosing disease have also been documented. Herein, we describe the first documented case of anaplastic large cell lymphoma (ALCL) following IgG4-related autoimmune pancreatitis and cholecystitis and diffuse large B-cell lymphoma (DLBCL). A 61-year-old Japanese male, with a past history of DLBCL, was detected with swelling of the pancreas and tumorous lesions in the gallbladder. Histopathological study of the resected gallbladder specimen revealed diffuse lymphoplasmacytic infiltration with fibrosclerosis in the entire gallbladder wall. Eosinophilic infiltration and obliterative phlebitis were also noted. Immunohistochemically, many IgG4-positive plasma cells had infiltrated into the lesion, and the ratio of IgG4/IgG-positive plasma cells was 71.6%. Accordingly, a diagnosis of IgG4-related cholecystitis was made. Seven months later, he presented with a painful tumor in his left parotid gland. Histopathological study demonstrated diffuse or cohesive sheet-like proliferation of large-sized lymphoid cells with rich slightly eosinophilic cytoplasm and irregular-shaped large nuclei. These lymphoid cells were positive for CD30, CD4, and cytotoxic markers, but negative for CD3 and ALK. Therefore, a diagnosis of ALK-negative ALCL was made. It has been suggested that the incidence of malignant lymphoma may be high in patients with IgG4-related sclerosing disease, therefore, intense medical follow-up is important in patients with this disorder.

  14. Alternative Pathway Dysregulation and the Conundrum of Complement Activation by IgG4 Immune Complexes in Membranous Nephropathy

    Science.gov (United States)

    Borza, Dorin-Bogdan

    2016-01-01

    Membranous nephropathy (MN), a major cause of nephrotic syndrome, is a non-inflammatory immune kidney disease mediated by IgG antibodies that form glomerular subepithelial immune complexes. In primary MN, autoantibodies target proteins expressed on the podocyte surface, often phospholipase A2 receptor (PLA2R1). Pathology is driven by complement activation, leading to podocyte injury and proteinuria. This article overviews the mechanisms of complement activation and regulation in MN, addressing the paradox that anti-PLA2R1 and other antibodies causing primary MN are predominantly (but not exclusively) IgG4, an IgG subclass that does not fix complement. Besides immune complexes, alterations of the glomerular basement membrane (GBM) in MN may lead to impaired regulation of the alternative pathway (AP). The AP amplifies complement activation on surfaces insufficiently protected by complement regulatory proteins. Whereas podocytes are protected by cell-bound regulators, the GBM must recruit plasma factor H, which inhibits the AP on host surfaces carrying certain polyanions, such as heparan sulfate (HS) chains. Because HS chains present in the normal GBM are lost in MN, we posit that the local complement regulation by factor H may be impaired as a result. Thus, the loss of GBM HS in MN creates a micro-environment that promotes local amplification of complement activation, which in turn may be initiated via the classical or lectin pathways by subsets of IgG in immune complexes. A detailed understanding of the mechanisms of complement activation and dysregulation in MN is important for designing more effective therapies. PMID:27199983

  15. Feasibility of utilizing the SD BIOLINE Onchocerciasis IgG4 rapid test in onchocerciasis surveillance in Senegal.

    Science.gov (United States)

    Dieye, Yakou; Storey, Helen L; Barrett, Kelsey L; Gerth-Guyette, Emily; Di Giorgio, Laura; Golden, Allison; Faulx, Dunia; Kalnoky, Michael; Ndiaye, Marie Khemesse Ngom; Sy, Ngayo; Mané, Malang; Faye, Babacar; Sarr, Mamadou; Dioukhane, Elhadji Mamadou; Peck, Roger B; Guinot, Philippe; de Los Santos, Tala

    2017-10-03

    As effective onchocerciasis control efforts in Africa transition to elimination efforts, different diagnostic tools are required to support country programs. Senegal, with its long standing, successful control program, is transitioning to using the SD BIOLINE Onchocerciasis IgG4 (Ov16) rapid test over traditional skin snip microscopy. The aim of this study is to demonstrate the feasibility of integrating the Ov16 rapid test into onchocerciasis surveillance activities in Senegal, based on the following attributes of acceptability, usability, and cost. A cross-sectional study was conducted in 13 villages in southeastern Senegal in May 2016. Individuals 5 years and older were invited to participate in a demographic questionnaire, an Ov16 rapid test, a skin snip biopsy, and an acceptability interview. Rapid test technicians were interviewed and a costing analysis was conducted. Of 1,173 participants, 1,169 (99.7%) agreed to the rapid test while 383 (32.7%) agreed to skin snip microscopy. The sero-positivity rate of the rapid test among those tested was 2.6% with zero positives 10 years and younger. None of the 383 skin snips were positive for Ov microfilaria. Community members appreciated that the rapid test was performed quickly, was not painful, and provided reliable results. The total costs for this surveillance activity was $22,272.83, with a cost per test conducted at $3.14 for rapid test, $7.58 for skin snip microscopy, and $13.43 for shared costs. If no participants had refused skin snip microscopy, the total cost per method with shared costs would have been around $16 per person tested. In this area with low onchocerciasis sero-positivity, there was high acceptability and perceived value of the rapid test by community members and technicians. This study provides evidence of the feasibility of implementing the Ov16 rapid test in Senegal and may be informative to other country programs transitioning to Ov16 serologic tools.

  16. Transformer core

    NARCIS (Netherlands)

    Mehendale, A.; Hagedoorn, Wouter; Lötters, Joost Conrad

    2010-01-01

    A transformer core includes a stack of a plurality of planar core plates of a magnetically permeable material, which plates each consist of a first and a second sub-part that together enclose at least one opening. The sub-parts can be fitted together via contact faces that are located on either side

  17. Transformer core

    NARCIS (Netherlands)

    Mehendale, A.; Hagedoorn, Wouter; Lötters, Joost Conrad

    2008-01-01

    A transformer core includes a stack of a plurality of planar core plates of a magnetically permeable material, which plates each consist of a first and a second sub-part that together enclose at least one opening. The sub-parts can be fitted together via contact faces that are located on either side

  18. IN SITU BIOREMEDIATION OF TRICHLOROETHYLENE USING BURKHOLDERIA CEPACIA G4 PR1: ANALYSIS OF MICROBIAL ECOLOGY PARAMETERS FOR RISK ASSESSMENT (RESEARCH BRIEF)

    Science.gov (United States)

    The introduction of bacteria into aquifers for bioremediation purposes requires monitoring of the persistence and activity of microbial populations for efficacy and risk assessment purposes. Burkholderia cepacia G4 PR1 constitutively expresses a toluene ortho-monooxygenase (tom) ...

  19. Presence and gradual disappearance of filaria-specific urinary IgG4 in babies born to antibody-positive mothers: a 2-year follow-up study.

    Science.gov (United States)

    Weerasooriya, Mirani V; Itoh, Makoto; Islam, Mohammad Z; Aoki, Yoshiki; Samarawickrema, Wilfred A; Kimura, Eisaku

    2008-09-01

    A total of 14 Sri Lankan pregnant women, who were anti-Brugia pahangi urinary IgG4 positive, and their 14 newborn babies were followed up for the urinary antibody for 2 years by enzyme-linked immunosorbent assay. Eight babies showed positive IgG4 reaction, at least once within 4 months after birth. Urinary antibody titers of mothers and their babies measured around the perinatal period showed a significant positive correlation, suggesting that baby's IgG4 was transferred from the mother through the placenta. The IgG4 decreased gradually and became negative in all positive babies by day 339.3 after birth. The present result provides a basis to judge if a positive urine ELISA test among babies is due to a new filarial infection.

  20. IgE and allergen-specific immunotherapy-induced IgG4 recognize similar epitopes of Bet v 1, the major allergen of birch pollen.

    Science.gov (United States)

    Groh, N; von Loetzen, C S; Subbarayal, B; Möbs, C; Vogel, L; Hoffmann, A; Fötisch, K; Koutsouridou, A; Randow, S; Völker, E; Seutter von Loetzen, A; Rösch, P; Vieths, S; Pfützner, W; Bohle, B; Schiller, D

    2017-05-01

    Allergen-specific immunotherapy (AIT) with birch pollen generates Bet v 1-specific immunoglobulin (Ig)G4 which blocks IgE-mediated hypersensitivity mechanisms. Whether IgG4 specific for Bet v 1a competes with IgE for identical epitopes or whether novel epitope specificities of IgG4 antibodies are developed is under debate. We sought to analyze the epitope specificities of IgE and IgG4 antibodies from sera of patients who received AIT. 15 sera of patients (13/15 received AIT) with Bet v 1a-specific IgE and IgG4 were analyzed. The structural arrangements of recombinant (r)Bet v 1a and rBet v 1a_11x , modified in five potential epitopes, were analyzed by circular dichroism and nuclear magnetic resonance spectroscopy. IgE binding to Bet v 1 was assessed by ELISA and mediator release assays. Competitive binding of monoclonal antibodies specific for Bet v 1a and serum IgE/IgG4 to rBet v 1a and serum antibody binding to a non-allergenic Bet v 1-type model protein presenting an individual epitope for IgE was analyzed in ELISA and western blot. rBet v 1a_11x had a Bet v 1a - similar secondary and tertiary structure. Monomeric dispersion of rBet v 1a_11x was concentration and buffer-dependent. Up to 1500-fold increase in the EC50 for IgE-mediated mediator release induced by rBet v 1a_11x was determined. The reduction of IgE and IgG4 binding to rBet v 1a_11x was comparable in 67% (10/15) of sera. Bet v 1a-specific monoclonal antibodies inhibited binding of serum IgE and IgG4 to 66.1% and 64.9%, respectively. Serum IgE and IgG4 bound specifically to an individual epitope presented by our model protein in 33% (5/15) of sera. Patients receiving AIT develop Bet v 1a-specific IgG4 which competes with IgE for partly identical or largely overlapping epitopes. The similarities of epitopes for IgE and IgG4 might stimulate the development of epitope-specific diagnostics and therapeutics. © 2016 John Wiley & Sons Ltd.

  1. 累及多部位的IgG4相关硬化性疾病1例并文献复习

    Institute of Scientific and Technical Information of China (English)

    郭雪西; 李彦敏; 胡占东; 李艳; 蔡文娟

    2014-01-01

    目的 观察罕见的IgG4相关硬化性疾病(IgG4-related sclerosing disease,IgG4-SD)的病理学特征、诊断及鉴别诊断.方法 对1例IgG4-SD进行病理学和免疫组化染色观察,并复习相关文献.结果 患者男性,60岁,2009年发现肾周肿物,胰腺饱满,体积增大;2011年发现双侧颌下腺及淋巴结肿大.镜检:肾周肿物、胰腺及颌下腺均显示纤维组织、淋巴组织增生,并伴大量成熟浆细胞浸润,淋巴结于滤泡间见较多浆细胞浸润.免疫表型:浆细胞呈IgG、IgG4阳性,IgG4阳性细胞计数约137个/HPF,IgG4阳性/IgG阳性细胞比值约60%.结论 IgG4-SD是近年来逐渐被认识的疾病之一,主要累及胰腺,也可累及其它器官.病理诊断需与Castleman病、一般慢性炎症相鉴别.

  2. IgE vs IgG4 epitopes of the peanut allergen Ara h 1 in patients with severe allergy

    DEFF Research Database (Denmark)

    Bøgh, Katrine Lindholm; Nielsen, H.; Eiwegger, T.;

    2013-01-01

    Background: Development and maintenance of tolerance to food allergens may be associated with increased levels of specific IgG4. It has been suggested that co-localisation of IgG4 and IgE binding epitopes may be of great significance for the tolerance, where IgG4 may act by blocking IgE binding...... to the allergen. However, recent studies have demonstrated the very importance of the IgG4-epitope affinity for the blocking ability. Studies comparing IgE and IgG4 binding epitopes mainly focus on the identification of linear epitopes. Peanut allergy is one of the most severe and persistent forms of food allergy....... The importance of conformational epitopes, of the major peanut allergen Ara h 1, has been demonstrated. The aim of this study was to compare Ara h 1-specific epitope patterns for IgE and IgG4 in patients with severe peanut allergy applying a method suitable to identify both linear and conformational epitopes...

  3. Specific IgE, IgG and IgG4 antibodies against house dust mite in patients with bronchial asthma.

    Directory of Open Access Journals (Sweden)

    Okazaki,Morihiro

    1991-08-01

    Full Text Available Serum levels of total IgE, specific IgE, IgG and IgG4 against house dust mite were measured in mite-sensitive asthma patients receiving immunotherapy with house dust. Serum levels of total IgE, mite specific IgE and IgG did not significantly change during the course of hyposensitization. Increased levels of mite specific IgG4 were observed in patients during immunotherapy. The increase in specific IgG4 was dependent on the total dose of house dust administered in both children (r = 0.636, p less than 0.001 and adults (r = 0.629, p less than 0.01. However, the increase of specific IgG