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Sample records for lymphomatoid granulomatosis recurrent

  1. Lymphomatoid granulomatosis: two cases with skin involvement.

    Science.gov (United States)

    Camisa, C

    1989-04-01

    Lymphomatoid granulomatosis is a systemic disease marked by a polymorphous cellular infiltrate that is both angiocentric and angiodestructive. The predominant organs of involvement are lungs, skin, central nervous system, and kidneys. I describe two cases of lymphomatoid granulomatosis in association with cutaneous manifestations, stressing to the dermatologist the importance of early recognition and diagnosis of this entity.

  2. Pulmonary lymphomatoid granulomatosis in a 4-year-old girl

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Subin; Kang, Mi-Jin; Lee, Jihae [Inje University Sanggye Paik Hospital, Department of Radiology, 1342, Dongilro, Nowon-gu, Seoul (Korea, Republic of); Kim, Hyun-Jung [Inje University Sanggye Paik Hospital, Department of Pathology, 1342, Dongilro, Nowon-gu, Seoul (Korea, Republic of); Kim, Hyo-Bin [Inje University Sanggye Paik Hospital, Department of Pediatrics, 1342, Dongilro, Nowon-gu, Seoul (Korea, Republic of)

    2015-07-15

    Lymphomatoid granulomatosis is a rare lymphoproliferative disease associated with the Epstein-Barr virus that commonly affects the lung. There is limited literature on cases of pediatric lymphomatoid granulomatosis. Half of all cases of lymphomatoid granulomatosis develop during the treatment of leukemia. Herein, we describe a case of lymphomatoid granulomatosis in a previously healthy child without leukemia. (orig.)

  3. Isolated neurological involvement of lymphomatoid granulomatosis

    Institute of Scientific and Technical Information of China (English)

    HU Yu-hua; SHAO En-de; WU Jian-liang; MENG Xian-bing

    2010-01-01

    @@ Lymphomatoid granulomatosis (LG) is a rare EpsteinBarr virus (EBV)-associated systemic granulomatous disease that is characterized by an angiocentric and occasionally angiodestructive polymorphic cellular infiltrate. It most frequently affects the lungs, kidneys,and brain.1,2 Usually the lesions are multi-systemic and multifocal. Central nervous system (CNS) involvement occurs in approximately 30% of affected patients, and patients may present with nonspecific neurological symptoms.3 Although CNS lesions usually appear as a secondary metastasis of pulmonary lesions, in rare conditions, it may be the initial or only manifestation of the disease. In this report, we describe two cases presenting with unifocal mass in the brain, histologically confirmed to be LG,

  4. Orbital lymphomatoid granulomatosis - a rare cause of proptosis

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    Du Toit, Jacqueline; Kilborn, Tracy [Department of Radiology, Red Cross Children' s Hospital, Rondebosch (South Africa); Eyssen, Ann van [Department of Oncology, Red Cross Children' s Hospital, Rondebosch (South Africa); Pillay, Komala [Department of Pathology, Red Cross Children' s Hospital, Rondebosch (South Africa)

    2015-07-15

    A 1-year-old girl with unilateral proptosis was found to have primary orbital lymphomatoid granulomatosis - a condition rarely occurring in children. This multisystem angiocentric, angiodestructive, lymphoproliferative disease typically involves the lungs, with ocular involvement being extremely uncommon. Our case serves to illustrate the imaging findings of this unusual condition and highlight a rare cause of proptosis. (orig.)

  5. Lymphomatoid granulomatosis mimicking interstitial lung disease.

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    Braham, Emna; Ayadi-Kaddour, Aïda; Smati, Belhassen; Ben Mrad, Sonia; Besbes, Mohammed; El Mezni, Faouzi

    2008-11-01

    Lymphoid granulomatosis is a rare form of pulmonary angiitis. This case report presents a patient with lymphoid granulomatosis in whom the clinical presentation, radiological features and the partial response to corticosteroid therapy mimicked interstitial lung disease. Lymphoid granulomatosis was only diagnosed at post-mortem examination. The range of reported clinical presentations, diagnostic approaches and outcomes are described.

  6. A Rare Presentation of a Rare Disease: Pulmonary Lymphomatoid Granulomatosis

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    Ghulam Rehman Mohyuddin

    2012-01-01

    Full Text Available A 70-year-old female presented with a 4-week history of dry cough and wheezing. Chest radiograph showed a 10.5 cm mass-like density in the anterior mediastinum which had not been previously visualized. Computed tomography scan (CT of the chest showed a right hilar mass encasing and narrowing right upper lobe bronchus and right mainstem bronchus and secondary atelectatic changes. Biopsy was consistent with a diagnosis of lymphomatoid granulomatosis Grade 3. She responded well clinically and radiologically to therapy. Lymphomatoid granulomatosis is a rare EBV-associated disorder which is considered a lymphoproliferative disease. The most common radiographic feature is multiple lung nodules. An isolated hilar mass is an exceptionally rare presentation of this rare disease.

  7. Pulmonary angiocentric lymphoma (lymphomatoid granulomatosis) in a donkey.

    Science.gov (United States)

    du Toit, N; Genovese, L M; Dalziel, R G; Smith, S H

    2012-01-01

    A 36-year-old donkey developed dyspnoea, pyrexia, hypoalbuminaemia and oedema. Following continued clinical deterioration the donkey was humanely destroyed. Grossly, there were numerous nodules (5-10mm) scattered throughout the lung. Microscopically, the lung was infiltrated by an angiocentric and bronchocentric to diffuse mixed population of small mature and atypical lymphocytes, histiocytes, plasma cells and fewer eosinophils. The infiltrate was composed of numerous small mature and fewer atypical CD3(+) T lymphocytes. Low numbers of CD20(+) and CD79a(+) B cells, some atypical, accompanied the T cells. These infiltrates were consistent with an angiocentric lymphoma and resembled lymphomatoid granulomatosis, an Epstein-Barr virus (EBV)-associated human tumour. Immunohistochemistry for EBV latent membrane protein and polymerase chain reaction analysis for equine gamma herpesvirus DNA were negative. To the authors' knowledge this is the first case of angiocentric lymphoma reported in a donkey and the first case of lymphomatoid granulomatosis-type disease in an animal in which possible concurrent infection with a gamma herpesvirus has been investigated.

  8. Lymphomatoid Granulomatosis: CT and FDG-PET Findings

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    Chung, Jonathan H. [National Jewish Health, Denver (United States); Wu, Carol C.; Gilman, Matthew D.; Palmer, Edwin L.; Hasserjian, Robert P.; Sphepar, Jo-Anne O. [Massachusetts General Hospital, Boston (United States)

    2011-11-15

    Lymphomatoid granulomatosis (LG) is a rare, aggressive extranodal Epstein-Barr virus (EBV)-positive B-cell lymphoproliferative disease. The purpose of our study was to analyze the CT and fluorodeoxyglucose positron emission tomography (FDG-PET) findings of pulmonary LG. Between 2000 and 2009, four patients with pathologically proven pulmonary LG and chest CT were identified. Two of these patients also had FDG-PET. Imaging features of LG on CT and PET were reviewed. Pulmonary nodules or masses with peribronchovascular, subpleural, and lower lung zonal preponderance were present in all patients. Central low attenuation (4 of 4 patients), ground-glass halo (3 of 4 patients), and peripheral enhancement (4 of 4 patients) were observed in these nodules and masses. An air-bronchogram and cavitation were seen in three of four patients. FDG-PET scans demonstrated avid FDG uptake in the pulmonary nodules and masses. Pulmonary LG presents with nodules and masses with a lymphatic distribution, as would be expected for a lymphoproliferative disease. However, central low attenuation, ground-glass halo and peripheral enhancement of the nodules/masses are likely related to the angioinvasive nature of this disease. Peripheral enhancement and ground-glass halo, in particular, are valuable characteristic not previously reported that can help radiologists suggest the diagnosis of pulmonary LG.

  9. DOCK 8 Deficiency, EBV+ Lymphomatoid Granulomatosis, and Intrafamilial Variation in Presentation.

    Science.gov (United States)

    Dimitriades, Victoria R; Devlin, Vincent; Pittaluga, Stefania; Su, Helen C; Holland, Steven M; Wilson, Wyndham; Dunleavy, Kieron; Shah, Nirali N; Freeman, Alexandra F

    2017-01-01

    Dedicator of cytokinesis 8 (DOCK8) deficiency is an autosomal recessive, combined immunodeficiency within the spectrum of hyper-IgE syndromes. Epstein-Barr virus-positive lymphomatoid granulomatosis (LYG) (EBV + LYG) is a rare diagnosis and a previously unreported presentation of DOCK8 deficiency. A 10-year-old girl was initially evaluated for mild eczema and recurrent sinopulmonary infections. She had normal immunoglobulins with elevated IgE, poor polysaccharide response with low switched memory B cells, low CD4 count, and normal mitogen and antigen responses. Despite clinical improvement following immunoglobulin replacement, a prolonged cough prompted a CT scan, which showed nodules. Biopsy identified a Grade 2 EBV + LYG. Due to an inadequate response with chemotherapy, further workup for primary immunodeficiency was performed. With her symptoms of eczema and IgE elevation, along with her brother's history of recurrent sinopulmonary infections and warts, targeted sequencing of DOCK8 was performed revealing compound heterozygous mutations for the two siblings. Both patients were successfully transplanted with resolution of the LYG and warts, respectively. This is the first reported case of LYG in DOCK8 deficiency. The EBV-driven lymphoproliferative disease along with the infection history in the brother led to the diagnosis of DOCK8 deficiency and curative hematopoietic stem cell transplants.

  10. Pulmonary lymphomatoid granulomatosis: Report of a case and review of literature

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    D B Olusina

    2011-01-01

    Full Text Available Lymphomatoid granulomatosis (LYG is a rare angiocentric lymphoproliferative process predominantly affecting the lung. The diagnosis of this condition is often difficult as the physical signs, history, chest x-ray, and routine laboratory investigations are usually non-specific. Nevertheless, it is important to establish a tissue diagnosis, as this lymphoproliferative disorder can be refractory to treatment and even progress to overt lymphoma. We report a case of pulmonary LYG in a 52-year old Nigerian man of Ibo extraction treated in our centre in 2001 and followed up for a year. The difficulty in making diagnosis is highlighted and treatment modality discussed.

  11. Arthritis in lymphomatoid granulomatosis: Report of a case and review of literature

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    Agarwal Vikas

    2004-02-01

    Full Text Available Lymphomatoid granulomatosis (LG is a rare systemic vasculitis caused by Epstein Barr virus induced transformation of the B-cells in a T-cell rich environment. The predominant clinical presentations are confined to the pulmonary system however; extra-pulmonary manifestations can sometimes be the main feature of the disease. Here in we describe a 52-year-old female who presented with symmetric polyarthritis and generalized stiffness for 7 months and papular lesions over extremities for 3 months duration. She in addition had generalized lymphadenopathy. Histopathological examination of the cutaneous lesions confirmed LG. Patient died despite therapy with cyclophosphamide and prednisolone. This is the first report of LG mimicking rheumatoid arthritis from India.

  12. A clinicopathologic analysis of primary central nervous system lymphomatoid granulomatosis: case report and literature review

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    FU Yong-juan

    2012-10-01

    Full Text Available Objective To investigate the clinical, neuroimaging and histopathological features of primary central nervous system lymphomatoid granulomatosis (LG. Methods The clinical manifestation, neuroimaging, histopathological and biological features of a patient with primary central nervous system LG were presented, and the related literatures were reviewed. Results A 57-year-old male presented with memory impairment, weak in orientation, calculation, apprehension and judgment for 3 months. Magnetic resonance imaging (MRI showed space-occupying lesions in bilateral frontal lobes, with T1WI isointensity and T2WI hyperintensity, and the enhancement was irregular. The lesion was slight expansive with yellow surface and gray-white section in color and soft texture and abundant blood supply. Microscopically, the lesion was characterized by angiocentric and angiodestructive lymphoproliferation, partly showed the structure of LG characterized by T cell predominant proliferation, macrophage infiltration, astrocyte activation, small vessel proliferation and hyalinization, and partly showed the structure of lymphoma characterized by diffuse atypical B cell proliferation, with IgK monoclonal production. Epstein-Barr virus (EBV was negative. Conclusion As a precursor disease of lymphoma, LG should be considered in the differential diagnosis of both diffuse and multifocal lesions of the central nervous system. The relavance between primary central nervous system LG and EBV infection should be further discussed.

  13. Lymphomatoid Granulomatosis of Central Nervous System and Lung Driven by Epstein Barr Virus Proliferation: Successful Treatment with Rituximab

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    Ruben Fernandez

    2014-02-01

    Full Text Available Lymphomatoid granulomatosis (LYG is a very rare Epstein-Barr virus (EBV associated B-cell lymphoproliferative disorder. We report the case of a 41-year-old man who presented with fever and respiratory symptoms. Computed tomography showed multiple nodules in both lung fields. Polymerase chain reaction (PCR analysis of the bronchoalveolar lavage was positive for EBV and biopsy of lung node yielded a diagnosis of LYG, grade III. Shortly after initiation of treatment with chemotherapy, neurologic deterioration appeared. Radiologic findings revealed hydrocephalus and PCR analysis of the cerebrospinal fluid (CSF was positive for EBV. Treatment with intravenous rituximab led to rapid reduction of EBV load in CSF, along with radiological and clinical improvement. After completion of treatment with immunochemotherapy, an autologous stem cell transplantation was performed. Patient stays in remission one year after diagnosis.

  14. Recurrence of Intravenous Talc Granulomatosis following Single Lung Transplantation

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    Richard C Cook

    1998-01-01

    Full Text Available Advanced pulmonary disease is an unusual consequence of the intravenous injection of oral medications, usually developing over a period of several years. A number of patients with this condition have undergone lung transplantation for respiratory failure. However, a history of drug abuse is often considered to be a contraindication to transplantation in the context of limited donor resources. A patient with pulmonary talc granulomatosis secondary to intravenous methylphenidate injection who underwent successful lung transplantation and subsequently presented with recurrence of the underlying disease in the transplanted lung 18 months after transplantation is reported.

  15. Clinical features and diagnosis of primary cerebral lymphomatoid granulomatosis%原发性颅内淋巴瘤样肉芽肿病的临床特点及诊断

    Institute of Scientific and Technical Information of China (English)

    卫华; 李存江; 朴月善; 卢德宏; 周静安

    2014-01-01

    目的:总结原发性颅内淋巴瘤样肉芽肿病的临床特点、影像学及组织病理学特点。方法报告我院收治的1例原发性颅内淋巴瘤样肉芽肿病病例,并对相关文献进行复习。结果患者,女,28岁,癫痫发作3年余,头颅MRI显示散在多发病灶,环形或结节状强化,病灶周围水肿明显。除颅内多发病灶外其他器官未受累。脑组织活检病理学提示淋巴瘤样肉芽肿病。结论原发性颅内淋巴瘤样肉芽肿病影像学有一定特点,但无特异性,因此颅内占位性病变或散在、浸润性病变患者需注意和本病鉴别。单纯颅内淋巴瘤样肉芽肿病预后较好。%Objective To report a case of primary brain lymphomatoid granulomatosis (LYG), and summarize its imaging, histopathological characteristics and prognosis. Methods The clinical, pathological, imaging and laboratory data of the patient were presented, and the pertinent literature were reviewed to discuss the importance of this rare entity. Re-sults A 28-year-old woman had been suffering from epilepsia for more than 3 years. MRI revealed multiple focal in-tra-parenchymal lesions, with heterogeneous enhancement surrounded by perilesional edema in the left frontal and tempo-ral lobe. The focal resection of the lesion in the left frontal was performed and lymphomatoid granulomatosis was diagnosed based on the histopathological examination. Conclusion CNS-LYG is a rare disease that should be considered in the dif-ferential diagnosis of both diffuse and space-occupying cerebral lesions. Primary cerebral LYG appears to have a better prognosis than systemic LYG with CNS localization.

  16. Recurrent Septic Arthritis Due to Achromobacter xylosoxidans in a Patient With Granulomatosis With Polyangiitis.

    Science.gov (United States)

    Patel, Payal K; von Keudell, Arvind; Moroder, Philipp; Appleton, Paul; Wigmore, Robin; Rodriguez, Edward K

    2015-12-01

    We report a case of recurrent Achromobacter xylosoxidans infections including bacteremia, sepsis, septic joints and endocarditis in a 72 year old female with granulomatosis with polyangiitis. Achromobacter xylosoxidans is a gram negative bacteria increasingly identified in immunocompromised patients. Surgical and medical therapy may need to be combined.

  17. Genetically Modified T-cell Infusion Following Peripheral Blood Stem Cell Transplant in Treating Patients With Recurrent or High-Risk Non-Hodgkin Lymphoma

    Science.gov (United States)

    2017-01-27

    Adult Grade III Lymphomatoid Granulomatosis; Cutaneous B-cell Non-Hodgkin Lymphoma; Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Intraocular Lymphoma; Nodal Marginal Zone B-cell Lymphoma; Post-transplant Lymphoproliferative Disorder; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Grade III Lymphomatoid Granulomatosis; Recurrent Adult Immunoblastic Large Cell Lymphoma; Recurrent Adult Lymphoblastic Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Small Lymphocytic Lymphoma; Refractory Hairy Cell Leukemia; Small Intestine Lymphoma; Splenic Marginal Zone Lymphoma; Testicular Lymphoma; Waldenström Macroglobulinemia

  18. Pulmonary lymphomatoid granulomatosis: an immunohistochemical and gene rearrangement study%肺淋巴瘤样肉芽肿病的免疫表型及基因重排研究

    Institute of Scientific and Technical Information of China (English)

    冯瑞娥; 刘鸿瑞; 刘彤华; 陈杰; 凌庆; 师晓华; 钟定荣; 罗玉风; 曹金伶

    2011-01-01

    目的 观察肺淋巴瘤样肉芽肿病的细胞组成、免疫表型和分子生物学改变.方法 回顾性分析北京协和医院9例肺淋巴瘤样肉芽肿病患者的临床病理情况.其中5例为开胸肺活检标本,3例为肺叶切除标本,1例尸检.标本经4%甲醛固定,石蜡包埋,常规切片,HE染色.免疫组织化学EnVision法染色(抗体包括CD20、CD3、CD56),原位杂交检测EB病毒,采用聚合酶链反应进行Ig和TCR基因重排检测.结果 9例肺淋巴瘤样肉芽肿患者,年龄3~59岁,男∶女=3:6.9例患者肺组织内病变分布均显示以血管为中心的淋巴细胞浸润为特点.免疫组织化学显示以CD3阳性的T淋巴细胞占绝对优势,散在不等数量的CD20阳性的B细胞,CD56均为阴性.8例行EB病毒原位杂交,4例阳性细胞数20%,1例为15%.按照WHO的3级分级方法,Ⅰ级为4例,Ⅱ级1例,Ⅲ级4例.6例行基因重排检测,3例显示有Ig基因重排阳性,其中,1例为Ⅱ级病变,2例为Ⅲ级病变.6例TCR重排检测均为阴性.随访时间0.5~6.5年不等,9例患者中3例死亡,2例存活,4例失访.结论 部分肺淋巴瘤样肉芽肿病,特别是Ⅱ、Ⅲ级患者,有B淋巴细胞克隆性增生,提示其为淋巴瘤性病变.%Objective To study the immunophenotype and gene rearrangement pattern of pulmonary lymphomatoid granulomatosis. Methods Nine cases of pulmonary lymphomatoid granulomatosis, included 5 cases of open lung biopsy, 3 cases of lobectomy specimen and 1 case of autopsy, were retrospectively analyzed by immunohistochemistry, in-situ hybridization for Epstein-Barr virus-encoded RNA, immunoglobulin and T-cell receptor gene rearrangement studies. Results The Histologically, all cases showed lymphocytic infiltration surrounding the blood vessels and in the perivascular areas. Most of these lymphoid cells expressed T-cell marker CD3. There were also variable numbers of CD20-positive B cells. The staining for CD56 was negative. According to the WHO

  19. Vorinostat With or Without Isotretinoin in Treating Young Patients With Recurrent or Refractory Solid Tumors, Lymphoma, or Leukemia

    Science.gov (United States)

    2014-06-16

    Childhood Acute Promyelocytic Leukemia (M3); Childhood Atypical Teratoid/Rhabdoid Tumor; Childhood Burkitt Lymphoma; Childhood Chronic Myelogenous Leukemia; Childhood Diffuse Large Cell Lymphoma; Childhood Immunoblastic Large Cell Lymphoma; Juvenile Myelomonocytic Leukemia; Recurrent Childhood Acute Lymphoblastic Leukemia; Recurrent Childhood Acute Myeloid Leukemia; Recurrent Childhood Grade III Lymphomatoid Granulomatosis; Recurrent Childhood Large Cell Lymphoma; Recurrent Childhood Lymphoblastic Lymphoma; Recurrent Childhood Medulloblastoma; Recurrent Childhood Small Noncleaved Cell Lymphoma; Recurrent Childhood Supratentorial Primitive Neuroectodermal Tumor; Recurrent Neuroblastoma; Recurrent/Refractory Childhood Hodgkin Lymphoma; Relapsing Chronic Myelogenous Leukemia; Unspecified Childhood Solid Tumor, Protocol Specific

  20. Lymphomatoid papulosis with a natural killer-cell phenotype

    NARCIS (Netherlands)

    Bekkenk, MW; Kluin, PM; Jansen, PM; Meijer, CJLM; Willemze, R

    Lymphomatoid papulosis (LyP) is defined as a recurrent self-healing papulonodular eruption with the histological features of a (CD30+) cutaneous T-cell lymphoma. The atypical cells usually have a CD3+/-, CD4+/-, CD8-, CD30+, CD56- T-cell phenotype. We report an unusual case of LyP, in which the

  1. Orofacial granulomatosis

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    Srinivas Kandula

    2016-01-01

    Full Text Available Orofacial granulomatosis (OFG is a rare disease, usually presenting as a persistent swelling of the soft tissues in the orofacial region, predominantly lips (cheilitis granulomatosa. The treatment of OFG can be challenging as frequent recurrences are very common. Here, we present a case of an Asian female patient with persistent swelling of the upper lip from the past 6 months. After establishing the diagnosis, intralesional triamcinolone injection (10 mg on a weekly schedule was planned for a period of 6 weeks. Complete resolution of the lesion was observed. This article presents a unique case of cheilitis granulomatosa (CG with a brief highlight on the various treatment modalities and a structured algorithm discussing the various differential diagnoses, aiding in formulating an accurate diagnosis and effective treatment.

  2. Clinicopathological diagnosis of orofacial granulomatosis.

    Science.gov (United States)

    Afsar, Fatma Sule; Duran, Hatice Demirlendi; Yilmaz, Gungor; Ermete, Murat

    2017-01-01

    Orofacial granulomatosis is a rare chronic inflammatory disorder characterized by persistent or recurrent soft tissue swellings, oral ulceration, and other orofacial features in the absence of an identifiable granulomatous disease. We report a case of a 61-year-old woman with recurrent ulcerations and swellings in her oral mucosa. She was diagnosed as orofacial granulomatosis based upon clinicopathological correlation after exclusion of other granulomatous diseases and showed a favorable response to systemic corticosteroid treatment.

  3. 原发于中枢神经系统的淋巴瘤样肉芽肿:病例报告并文献分析%A clinicopathologic analysis of primary central nervous system lymphomatoid granulomatosis:case report and literature review

    Institute of Scientific and Technical Information of China (English)

    付永娟; 朴月善; 陈莉; 李存江; 卢德宏

    2012-01-01

    Objective To investigate the clinical, neuroimaging and histopathological features of primary central nervous system lymphomatoid granulomatosis (LG). Methods The clinical manifestation, neuroimaging, histopathological and biological features of a patient with primary central nervous system LG were presented, and the related literatures were reviewed. Results A 57-year-old male presented with memory impairment, weak in orientation, calculation, apprehension and judgment for 3 months. Magnetic resonance imaging (MRI) showed space-occupying lesions in bilateral frontal lobes, with TiWI isointensity and T2WI hyperintensity, and the enhancement was irregular. The lesion was slight expansive with yellow surface and gray-white section in color and soft texture and abundant blood supply. Microscopically, the lesion was characterized by angiocentric and angiodestructive lymphoproliferation, partly showed the structure of LG characterized by T cell predominant proliferation, macrophage infiltration, astrocyte activation, small vessel proliferation and hyalinization, and partly showed the structure of lymphoma characterized by diffuse atypical B cell proliferation, with IgK monoclonal production. Epstein-Barr virus (EBV) was negative. Conclusion As a precursor disease of lymphoma, LG should be considered in the differential diagnosis of both diffuse and multifocal lesions of the central nervous system. The relavance between primary central nervous system LG and EBV infection should be further discussed.%目的 探讨原发于中枢神经系统的淋巴瘤样肉芽肿的临床表现、影像学及病理学特点.方法 回顾分析一例原发于中枢神经系统的淋巴瘤样肉芽肿患者的临床及影像学表现、组织学及分子生物学特点,并复习相关文献.结果 男性患者,57岁.表现为记忆力减退3个月,地点定向障碍,计算力和理解判断力下降.影像学检查显示双侧额叶占位性病变,呈等T1、长T2信号,强化不均匀.

  4. Lymphomatoid granulomatosis of the brain: A case report

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    Edgardo Quinones

    2016-01-01

    Conclusions: This rare lesion in an adult, immunocompetent patient, debuting with motor seizures represents a challenge in terms of diagnosis and treatment. After surgical and medical treatment, the patient had a satisfactory recovery. Clinical features, imaging, differential diagnosis, and pathology are discussed.

  5. Pityriasis lichenoides and lymphomatoid papulosis.

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    Rogers, M

    1992-03-01

    The clinical features, histopathology, immunopathology, and management of pityriasis lichenoides and lymphomatoid papulosis are discussed, with particular emphasis on the pediatric aspects of these conditions. The difficulties in logically separating pityriasis lichenoides into an acute (pityriasis lichenoides et varioliformis acuta) and a chronic (pityriasis lichenoides chronical) form are addressed. The development of lymphoreticular malignancy in patients with lymphomatoid papulosis has been well documented, but pityriasis lichenoides has characteristically been regarded as a benign condition. However, recent reports of the development of large plaque parapsoriasis in patients with pityriasis lichenoides have led to a reconsideration. Some of these patients were in the pediatric age group. Although there are significant clinical, histopathological, and immunopathological differences between pityriasis lichenoides and lymphomatoid papulosis, the demonstration of similar clonal T cell receptor gene rearrangements and the confirmation of the potentially premalignant nature of both suggests that there may indeed be an interrelationship between these two controversial entities. Close follow-up of patients with both of these conditions is recommended, with observation being discontinued only when the patient has been free of lesions for several years.

  6. Orofacial Granulomatosis.

    Science.gov (United States)

    Al-Hamad, Arwa; Porter, Stephen; Fedele, Stefano

    2015-07-01

    Orofacial granulomatosis (OFG) is an uncommon chronic inflammatory disorder of the orofacial region. It is characterized by subepithelial noncaseating granulomas and has a spectrum of possible clinical manifestations ranging from subtle oral mucosal swelling to permanent disfiguring fibrous swelling of the lips and face. Etiopathogenesis is unknown. A range of systemic granulomatous disorders, including Crohn disease and sarcoidosis, may cause orofacial manifestations that cannot be distinguished from those of OFG. Treatment of OFG has proven difficult and unsatisfactory, with no single therapeutic model showing consistent efficacy in reducing orofacial swelling and mucosal inflammation.

  7. Vorinostat and Bortezomib in Treating Young Patients With Refractory or Recurrent Solid Tumors, Including Central Nervous System Tumors and Lymphoma

    Science.gov (United States)

    2013-07-01

    Childhood Burkitt Lymphoma; Childhood Central Nervous System Choriocarcinoma; Childhood Central Nervous System Germ Cell Tumor; Childhood Central Nervous System Germinoma; Childhood Central Nervous System Mixed Germ Cell Tumor; Childhood Central Nervous System Teratoma; Childhood Central Nervous System Yolk Sac Tumor; Childhood Choroid Plexus Tumor; Childhood Craniopharyngioma; Childhood Diffuse Large Cell Lymphoma; Childhood Immunoblastic Large Cell Lymphoma; Childhood Medulloepithelioma; Childhood Meningioma; Childhood Mixed Glioma; Childhood Nasal Type Extranodal NK/T-cell Lymphoma; Childhood Oligodendroglioma; Recurrent Childhood Anaplastic Large Cell Lymphoma; Recurrent Childhood Brain Stem Glioma; Recurrent Childhood Central Nervous System Embryonal Tumor; Recurrent Childhood Cerebellar Astrocytoma; Recurrent Childhood Cerebral Astrocytoma; Recurrent Childhood Ependymoma; Recurrent Childhood Grade III Lymphomatoid Granulomatosis; Recurrent Childhood Large Cell Lymphoma; Recurrent Childhood Lymphoblastic Lymphoma; Recurrent Childhood Malignant Germ Cell Tumor; Recurrent Childhood Medulloblastoma; Recurrent Childhood Pineoblastoma; Recurrent Childhood Small Noncleaved Cell Lymphoma; Recurrent Childhood Subependymal Giant Cell Astrocytoma; Recurrent Childhood Supratentorial Primitive Neuroectodermal Tumor; Recurrent Childhood Visual Pathway and Hypothalamic Glioma; Recurrent Childhood Visual Pathway Glioma; Recurrent/Refractory Childhood Hodgkin Lymphoma; Unspecified Childhood Solid Tumor, Protocol Specific

  8. A child with mastocytosis and lymphomatoid papulosis

    DEFF Research Database (Denmark)

    Lange, Henriette Juel; Agertoft, Lone; Møller, Michael Boe;

    2016-01-01

    A change in clinical behavior of a disease should prompt search for differential diagnoses. Here, the appearance of ulcerated skin nodules in a preexisting cutaneous mastocytosis revealed a concurrent lymphomatoid papulosis - a CD30+ lymphoproliferative skin disease with histological features of ...

  9. Alisertib in Combination With Vorinostat in Treating Patients With Relapsed or Recurrent Hodgkin Lymphoma, B-Cell Non-Hodgkin Lymphoma, or Peripheral T-Cell Lymphoma

    Science.gov (United States)

    2016-07-12

    Adult B Acute Lymphoblastic Leukemia; Adult T Acute Lymphoblastic Leukemia; Anaplastic Large Cell Lymphoma; Angioimmunoblastic T-Cell Lymphoma; Chronic Lymphocytic Leukemia; Cutaneous B-Cell Non-Hodgkin Lymphoma; Extranodal Marginal Zone Lymphoma of Mucosa-Associated Lymphoid Tissue; Hepatosplenic T-Cell Lymphoma; Intraocular Lymphoma; Lymphomatous Involvement of Non-Cutaneous Extranodal Site; Mature T-Cell and NK-Cell Non-Hodgkin Lymphoma; Nodal Marginal Zone Lymphoma; Recurrent Adult Acute Lymphoblastic Leukemia; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Grade III Lymphomatoid Granulomatosis; Recurrent Adult Hodgkin Lymphoma; Recurrent Adult Immunoblastic Lymphoma; Recurrent Adult Lymphoblastic Lymphoma; Recurrent Adult T-Cell Leukemia/Lymphoma; Recurrent Cutaneous T-Cell Non-Hodgkin Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Mycosis Fungoides and Sezary Syndrome; Recurrent Small Lymphocytic Lymphoma; Refractory Chronic Lymphocytic Leukemia; Refractory Hairy Cell Leukemia; Small Intestinal Lymphoma; Splenic Marginal Zone Lymphoma; T-Cell Large Granular Lymphocyte Leukemia; Testicular Lymphoma; Waldenstrom Macroglobulinemia

  10. Orofacial granulomatosis in children: think about Crohn's disease.

    Science.gov (United States)

    Lazzerini, Marzia; Martelossi, Stefano; Cont, Gabriele; Bersanini, Chiara; Ventura, Giovanna; Fontana, Massimo; Zuin, Giovanna; Ventura, Alessandro; Taddio, Andrea

    2015-04-01

    The term orofacial granulomatosis is conventionally used to describe patients with granulomatous lesions affecting the orofacial tissues, in absence of intestinal lesions. Lip swelling and facial swelling are the most common clinical signs. Despite the fact that histologically it is not distinguishable from Crohn's disease, and that both diseases have a chronic/recurrent course, the relationship between orofacial granulomatosis and Crohn's disease is still debated. Herein we present five cases of orofacial granulomatosis. All patients presented concomitant Crohn's disease, supporting the hypothesis that orofacial granulomatosis and Crohn's disease may be one single disease. Thalidomide was effective in inducing remission of oral and intestinal symptoms in all five cases and could be considered a valid treatment opportunity for these patients. Orofacial granulomatosis and Crohn's disease may be part of the same disease; both may respond to thalidomide. Copyright © 2014 Editrice Gastroenterologica Italiana S.r.l. Published by Elsevier Ltd. All rights reserved.

  11. A Case of Mycosis Fungoides and Lymphomatoid Papulosis Occurring Simultaneously in a Child

    OpenAIRE

    Queller, Jenna N.; Bognet, Rachel A.; Kozic, Heidi; Lee, Jason B.; Sahu, Joya; Hyde, Patrice M.

    2012-01-01

    Several studies have reported an association between lymphomatoid papulosis and other lymphomas, such as mycosis fungoides, Hodgkin’s disease, and anaplastic large cell lymphoma. The association between lymphomatoid papulosis and mycosis fungoides has been reported to be between seven and 39 percent. Although a relationship is acknowledged between lymphomatoid papulosis and mycosis fungoides, our understanding is limited. The authors report a case of mycosis fungoides and lymphomatoid papulos...

  12. Orofacial granulomatosis: clinical signs of different pathologies.

    Science.gov (United States)

    Troiano, Giuseppe; Dioguardi, Mario; Giannatempo, Giovanni; Laino, Luigi; Testa, Nunzio Francesco; Cocchi, Roberto; De Lillo, Alfredo; Lo Muzio, Lorenzo

    2015-01-01

    Orofacial granulomatosis (OFG) is an uncommon disease characterized by persistent or recurrent soft tissue enlargement, oral ulceration and a variety of other orofacial features. It could be an oral manifestation of a systemic disease. For a correct differential diagnosis, local and systemic conditions characterized by granulomatous inflammation should be excluded using appropriate clinical and laboratory investigations. In fact, the diagnosis of OFG may be confirmed only by histopathological identification of noncaseating granulomas. The literature from 1943 to 2014 was reviewed with emphasis on the etiology of OFG and on clinical manifestations of systemic pathologies associated with OFG. The precise cause of OFG is still unknown, although several theories have been suggested, such as infection, hereditary factors and allergy. OFG is a disease that has a wide spectrum of presentation, which may include the oral manifestation of a systemic condition such as Crohn's disease, sarcoidosis, granulomatosis with polyangiitis and Melkersson-Rosenthal syndrome.

  13. Lymphomatoid papulosis type C in a child%儿童淋巴瘤样丘疹病(C型)一例

    Institute of Scientific and Technical Information of China (English)

    惠云; 郑冰洁; 陈浩; 王千秋; 张国毅

    2013-01-01

    A 9-year-old boy was admitted to the hospital for recurrent papules,pustules,crust and necrosis all over the body for 8 years.A diagnosis of lymphomatoid papulosis type C was made according to histopathological and immunohistochemical findings.Lymphomatoid papulosis,as a kind of self-healing primary cutaneous CD30+ lymphoproliferative disorder,can be histopathologically divided into 3 types,i.e.,A,B and C,and little is known about its pathogenesis.Lymphomatoid papulosis is often misdiagnosed as pityriasis lichenoides et varioliformis acuta.Lymphomatoid papulosis type C should be pathologically differentiated from primary cutaneous CD30+ anaplastic large cell lymphoma.%患者男,9岁,因全身丘疹、脓疱、结痂、坏死8年余就诊.皮损组织病理结合免疫组化符合淋巴瘤样丘疹病(C型).淋巴瘤样丘疹病属于原发性皮肤CD30+淋巴增生性疾病,具有自限性,病因研究较少,组织病理学可分为A、B、C三型.临床上易误诊为急性痘疮样苔藓样糠疹,C型组织病理学与原发性皮肤CD30+间变性大细胞性淋巴瘤相似,故鉴别诊断尤为重要.

  14. Uncommon inflammatory swelling of the lips: orofacial granulomatosis.

    Science.gov (United States)

    Hafiz, Abdul; Mufeed, Abdulla; Kandasamy, Gopinath; Krishnapillai, Rekha

    2016-01-12

    Orofacial granulomatosis (OFG) is an unusual condition associated with permanent or recurrent swelling of orofacial tissues together with oral mucosal ulceration and a variety of orofacial characteristics. The chronic inflammation inherent to OFG often displays granulomas in the subepithelial stroma. We present a case of OFG and its management. The patient responded to intralesional injections of corticosteroids.

  15. Massive intracerebral hemorrhage associated with Wegener granulomatosis.

    Science.gov (United States)

    Ceri, Mevlut; Ortabozkoyun, Levent; Unverdi, Selman; Kirac, Mustafa; Duranay, Murat

    2012-06-01

    Wegener granulomatosis (WG) is a necrotizing granulomatous vasculitis that predominantly affects airways and kidneys. But central nervous system involvement (7-11%) is an uncommon. Massive ICH may occur in the course of WG, and this serious condition is related with high risk of mortality. Therefore, the new treatment strategies may be considered in addition to classical practices in serious organ involvement and recurrent attack. Here, we present an adult patient with WG whose disease was complicated by a massive intracerebral hemorrhage (ICH), which subsequently led to death.

  16. Staphylococcus aureus and Wegener's granulomatosis

    NARCIS (Netherlands)

    Popa, ER; Stegeman, CA; Kallenberg, CGM; Tervaert, JWC

    2002-01-01

    Wegener's granulomatosis (WG) is a form of systemic vasculitis. It is characterized by granulomatous inflammation in the upper and lower airways, vasculitis and necrotizing glomerulonephritis, and is strongly associated with antineutrophil cytoplasmic antibodies against proteinase 3, Since the etiol

  17. T cell lymphomatoid contact dermatitis: a challenging case and review of the literature.

    Science.gov (United States)

    Knackstedt, Thomas J; Zug, Kathryn A

    2015-02-01

    Lymphomatoid contact dermatitis is a pseudolymphoma with clinical and histological features of allergic contact dermatitis and cutaneous T cell lymphoma. Incorrect diagnosis may lead to unnecessary testing, unnecessary treatment, or patient harm. The objective of this study is to present a case to demonstrate the diagnostic challenge and overlap between allergic contact dermatitis and cutaneous T cell lymphoma in a patient with lymphomatoid contact dermatitis caused by methylchoroisothiazolinone/methylisothiazolinone and paraben mix, and to review the existing literature in order to summarize the demographics, clinical features, allergens and treatments reported for lymphomatoid contact dermatitis. A search of major scientific databases was conducted for English-language articles reporting cases of lymphomatoid contact dermatitis or additional synonymous search headings. Nineteen articles with a total of 23 patients were analysed. Lymphomatoid contact dermatitis was more common in men, with an average age of 58.5 years. Fourteen unique allergens were identified and confirmed by patch testing. However, no single test or study was diagnostic of lymphomatoid contact dermatitis. Allergen avoidance was the most useful management tool, but selected patients required topical or systemic immunosuppression. In conclusion, without specific diagnostic features, evaluation for lymphomatoid contact dermatitis should include a thorough history and examination, patch testing, and biopsy with immunohistochemistry and clonality studies.

  18. Idiopathic orofacial granulomatosis in a young patient: A rare entity

    Directory of Open Access Journals (Sweden)

    Gurumoorthy Kaarthikeyan

    2012-01-01

    Full Text Available The granular enlargement of the gingiva may be the first clinical manifestation of Orofacial granulomatosis, preceding other local or systemic manifestations. The term Idiopathic Orofacial granulomatosis (OFG refers to conditions restricted to the oral region without any identifiable systemic granulomatous diseases. We report a case of nine year old female patient with gingival enlargement as the sole manifestation without any systemic involvement. Laboratory investigations were done to rule out tuberculosis, sarcoidosis, allergic hypersensitivity, Crohn′s disease. The patient was treated by gingivectomy and there is complete remission of the lesion without any recurrence. Thus the early investigations and diagnosis of OFG can help in the prevention of future systemic complications.

  19. Lymphomatoid papulosis with pseudocarcinomatous hyperplasia in a 7-year-old girl: a case report.

    Science.gov (United States)

    Xiong, Jingshu; Ma, Yiping; Chen, Hao; Xu, Xiulian; Sun, Jianfang

    2016-05-01

    Lymphomatoid papulosis (LyP) belongs to the group of cutaneous CD30+ lymphoproliferative disorders. Pseudocarcinomatous hyperplasia has rarely been reported in patients with LyP. In this report, we describe a case of LyP presenting as pseudocarcinomatous hyperplasia. The patient was a 7-year-old girl who presented with a recurrent papulonodular eruption on her face and trunk for 2 months. Histopathologic examination revealed an irregular growth of hyperkeratotic epidermis into the whole dermal layer with marked nests of squamous cells in the background of diffuse atypical lymphoid cells, eosinophils and neutrophils. The large atypical cells were positive for CD30 and CD3, but negative for CD4, CD5, CD8, CD20 and CD56. A TCR-γ clone was identified by polymerase chain reaction (PCR). The correct diagnosis in cases of LyP with overlying pseudocarcinomatous epithelial hyperplasia can be very difficult both clinically and histopathologically. Clinical and histopathologic characteristics should be integrated to avoid an erroneous diagnosis of squamous cell carcinoma or keratoacanthoma.

  20. Type D Lymphomatoid Papulosis: An uncommon Variant. A case report and review of the literature

    Directory of Open Access Journals (Sweden)

    Gladys Alejandra Paguaga

    2014-10-01

    Full Text Available Lymphomatoid papulosis (LyP is an indolent form of primary cutaneous T-cell lymphoma, currently classified together with primary cutaneous anaplastic large T-cell lymphoma within the spectrum of CD30-positive lymphoproliferative disorders. It is characterized by presenting as a clinically benign but histopathological malignant disease. Clinical features consist in recurrent waxing and waning red papules. Histopathologically, there are 4 variants recognized, Type A or Hystiocitic type, being the most frecuent of all, Type B or Mycosis fungoides-like, Type C or Anaplastic large-cell lymphoma-like and Type D, the most recently described and uncommon variant with features similar to Cutaneous Aggressive CD8-Positive Cytotoxic T-Cell Lymphoma. We present a case of a 22-year-old female with multiple papules and nodules in trunk and limbs that after histopathological and immunochemical examination was compatible with Type D LyP. It is important to report this case, as a perfect example of an uncommon variant of LyP, with emphasis in its typical clinical, histopathological and inmunohistochemical findings and review of the literature.

  1. Lymphomatoid papulosis - making sense of the alphabet soup: a proposal to simplify terminology.

    Science.gov (United States)

    Kempf, Werner; Mitteldorf, Christina; Karai, Laszlo J; Robson, Alistair

    2017-04-01

    Clinically, lymphomatoid papulosis (LYP) is characterized by recurrent papulonodular lesions. Unlike this stereotypical clinical presentation, the histological spectrum of LYP is very wide, comprising distinct growth patterns, variably sized neoplastic cells, and different immunophenotypes. The revised 2016 WHO classification includes the histological LYP types A to E as well as another type characterized by a specific chromosomal alteration. In addition, new LYP types are going to be proposed, based not only on histological but also on clinical and genetic features. The ensuing expansion of the alphabetical list of histological types will add to the complexity of the terminology of LYP, thereby potentially increasing the risk of complicating rather than facilitating the diagnostic approach to the disease. Moreover, there may be overlap between individual disease types. This development raises the question as to how to simplify the terminology of LYP while still respecting its histological complexity. Herein, we advocate a practical approach to the terminology of LYP based on descriptive terms rather than the designation of LYP types by alphabetical characters. Our proposal aims to contribute to a pragmatic and user-friendly approach, thus not only facilitating the diagnostic process but also the communication between clinicians and pathologists.

  2. High-Dose Busulfan and High-Dose Cyclophosphamide Followed By Donor Bone Marrow Transplant in Treating Patients With Leukemia, Myelodysplastic Syndrome, Multiple Myeloma, or Recurrent Hodgkin or Non-Hodgkin Lymphoma

    Science.gov (United States)

    2010-08-05

    Adult Acute Myeloid Leukemia; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Grade III Lymphomatoid Granulomatosis; Recurrent Adult Hodgkin Lymphoma; Recurrent Adult Immunoblastic Large Cell Lymphoma; Recurrent Adult Lymphoblastic Lymphoma; Recurrent Adult Non-Hodgkin Lymphoma; Recurrent Adult T-cell Leukemia/Lymphoma; Recurrent Childhood Acute Lymphoblastic Leukemia; Recurrent Childhood Acute Myeloid Leukemia; Recurrent Cutaneous T-cell Non-Hodgkin Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Mycosis Fungoides/Sezary Syndrome; Recurrent Small Lymphocytic Lymphoma; Refractory Multiple Myeloma; Relapsing Chronic Myelogenous Leukemia; Secondary Myelodysplastic Syndromes; Small Intestine Lymphoma; Splenic Marginal Zone Lymphoma; Testicular Lymphoma; Waldenstrom Macroglobulinemia

  3. Lymphohistiocytoid mesothelioma: a rare lymphomatoid variant of predominantly sarcomatoid mesothelioma

    Energy Technology Data Exchange (ETDEWEB)

    Henderson, D.W.; Attwood, H.D.; Constance, T.J.; Shilkin, K.B.; Steele, R.H.

    1988-01-01

    Of 394 ''definite'' mesotheliomas entered in the Australian Mesothelioma Surveillance Program, three bore a striking resemblance to malignant lymphoma by conventional light microscopy, and each was misinterpreted at some stage as lymphoma. The lymphoma-like morphology was a combined result of intense lymphoplasmacytic infiltration and the histiocytoid appearances of the underlying neoplastic cell population. Immunocytochemical analysis demonstrated cytokeratins coexpressed with vimentin within the tumor cells, whereas immunoreactivity for leukocyte common antigen was confined to the smaller lymphoid cells. Electron microscopy of two cases revealed a polymorphous population of fibrohistiocytic cells resembling those typical of malignant fibrous histiocytoma, admixed with lymphocytes and plasma cells, but sporadic cells expressed mesothelial properties in the form of sinuous villiform processes, intracytoplasmic neolumina lined by microvilli, and intermediate filaments that were aggregated into tonofilament bundles in some cells. The ultrastructural appearances, the localization of the tumors to the pleura, with effusion, and absence of anterior mediastinal mass lesions facilitated exclusion of lymphocyte-rich thymoma. In addition, a history of prior occupational exposure to asbestos was elicited in each instance. There was no apparent response to radiotherapy or chemotherapy, and the patients died at 4, 5, and 8 months after presentation. Our observations suggest that immunocytochemical or ultrastructural evaluation is mandatory for accurate diagnosis of all pleura-based lymphomatoid lesions with a mixed large and small cell pattern. 42 references.

  4. Eosinophilic Granulomatosis with Polyangiitis and Diffuse Gastrointestinal Involvement

    Directory of Open Access Journals (Sweden)

    Diana L. Franco

    2014-10-01

    Full Text Available Eosinophilic granulomatosis with polyangiitis (EGPA, formerly named Churg-Strauss syndrome, is a rare systemic small- and medium-sized-vessel vasculitis, characterized by the presence of severe asthma as well as blood and tissue eosinophilia. Gastrointestinal (GI symptoms, like diarrhea and abdominal pain, are common; however, there are few reports of histologic evidence of GI involvement. We report the case of a patient on treatment for EGPA who presented with recurrent small bowel obstruction and choledocholithiasis. Biopsies of the esophagus, small bowel and common bile duct showed diffuse eosinophilia, with clear EGPA in the GI tract. Improved awareness of GI EGPA may allow for timely management of this disorder.

  5. Wegener's granulomatosis--an etiology of acute pancreatitis.

    Science.gov (United States)

    Joshipura, Vismit P; Haribhakti, Sanjiv P; Pandya, Sapan C; Soni, Harshad N; Patel, Nitin R

    2007-01-01

    Wegener's granulomatosis is a systemic disease that usually involves the upper respiratory tract and kidneys. We report a 47-year-old man with Wegener's granulomatosis that presented as acute pancreatitis.

  6. Oxaliplatin and Irinotecan in Treating Young Patients With Refractory Solid Tumors or Lymphomas

    Science.gov (United States)

    2013-06-04

    Childhood Burkitt Lymphoma; Childhood Central Nervous System Germ Cell Tumor; Childhood Diffuse Large Cell Lymphoma; Childhood Grade III Lymphomatoid Granulomatosis; Childhood Immunoblastic Large Cell Lymphoma; Recurrent Childhood Brain Stem Glioma; Recurrent Childhood Cerebellar Astrocytoma; Recurrent Childhood Cerebral Astrocytoma; Recurrent Childhood Ependymoma; Recurrent Childhood Grade III Lymphomatoid Granulomatosis; Recurrent Childhood Large Cell Lymphoma; Recurrent Childhood Liver Cancer; Recurrent Childhood Lymphoblastic Lymphoma; Recurrent Childhood Malignant Germ Cell Tumor; Recurrent Childhood Medulloblastoma; Recurrent Childhood Rhabdomyosarcoma; Recurrent Childhood Small Noncleaved Cell Lymphoma; Recurrent Childhood Soft Tissue Sarcoma; Recurrent Childhood Supratentorial Primitive Neuroectodermal Tumor; Recurrent Childhood Visual Pathway Glioma; Recurrent Colon Cancer; Recurrent Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Recurrent Melanoma; Recurrent Nasopharyngeal Cancer; Recurrent Neuroblastoma; Recurrent Osteosarcoma; Recurrent Wilms Tumor and Other Childhood Kidney Tumors; Recurrent/Refractory Childhood Hodgkin Lymphoma; Unspecified Childhood Solid Tumor, Protocol Specific

  7. Granulomatosis with polyangiitis in Tunisia

    Directory of Open Access Journals (Sweden)

    I. Ben Ghorbel

    2017-05-01

    Full Text Available Granulomatosis with polyangiitis (GPA is more frequent in Northern rather than Southern countries. Very few studies have been conducted in Africa. We have performed a retrospective descriptive study including clinical and laboratory profiles of 30 Tunisian GPA patients seen at the department of Internal Medicine of the University Hospital of la Rabta from 2000 to 2014. Mean age at initial GPA diagnosis was 46±12 years, and the average number of months between the onset of symptoms and diagnosis was 25. Seventeen (56% were male, and 13 (44% were female. Ear/nose/throat involvement occurred in 83%. Lung and renal involvement were observed in respectively 70% and 56% followed by mucocutaneous (50%, neurological (50%, ocular (33%, vascular (20%, ureteral (16%, and cardiac involvement in 10%. Cytoplasmic pattern-antineutrophil cytoplasmic antibodies (ANCA was detected in 27 (90% patients. Induction therapy consisted of intravenous cyclophosphamide pulses in 27 patients (90% and oral methotrexate in 3 patients (10%. Trimethoprime-sulfamethoxazole was used in 26 patients (86%. Maintenance therapy consisted of azathioprine in 17 cases and methotrexate in 13 cases. Relapses occurred in 36%. Eighteen patients had favorable outcome and 12 died. Our patients had a distinct phenotype with high prevalence of pleural involvement, lymph node enlargement, sensorimotor neuropathy and ureter stenosis. ENT symptoms were less frequent as inaugural presentation. Overall 2-year survival was 60%.

  8. Granulomatose de Wegener Wegener's granulomatosis

    Directory of Open Access Journals (Sweden)

    Telma Antunes

    2005-07-01

    Full Text Available A granulomatose de Wegener caracteriza-se por vasculite necrosante granulomatosa que acomete preferencialmente vias aéreas superiores, inferiores e rins. Seu diagnóstico é feito associando-se as manifestações clínicas, radiológicas (multiplos nódulos escavados e os achados anatomopatológicos e o anticorpo anticitoplasma de neutrófilos positivo. O tratamento com corticosteróides e ciclofosfamida leva a 90% de remissão da doença em 1 ano.Wegener's granulomatosis is characterized by granulomatous necrotizing vasculitis that primarily affects the airways (upper and lower and the kidneys. The diagnosis is made by analyzing the clinical and radiological manifestations (multiple pulmonary cavitations, together with the pathological findings and results of the test for cytoplasmic-pattern antineutrophil cytoplasmic antibodies. Treatment consists of corticosteroids and cyclophosphamide and leads to remission of the disease within one year in 90% of cases.

  9. Pulmonary Localization Revealing Wegener's Granulomatosis

    Directory of Open Access Journals (Sweden)

    Mona Mlika

    2010-01-01

    Full Text Available Wegener's granulomatosis (WG is the most frequent antineutrophil cytoplasmic antibody (ANCA–associated vasculitis. It affects mainly the upper airways, lungs, and kidneys. Two forms are identified: systemic and limited. We describe three cases of limited WG diagnosed during a 7-year period. Our aim is to report three localized forms of WG and to put emphasis on the necessity of differentiating localized from systemic forms because of their different prognoses and manner of management. Our study contained two men and one woman with a mean age of 43 years. All our patients were symptomatic and presented with nonspecific respiratory signs. The cANCA were positive in all patients. The imaging findings consisted of cavitary masses. The diagnosis was based on surgical lung biopsy in all cases. All patients were put on cyclophosphamide and prednisolone. Only one patient presented with renal complications after a 2-year follow-up period. The two other patients did not present complications after, respectively, 1 month and 1 year of follow-up. These case reports put emphasis on a rare form of WG, the limited form. The low number of patients, due to the rarity of this disease, does not allow us to delineate the characteristics and the differences between this form and the systemic form, but we highlight the necessity of future investigations in order to explore the pathogenesis, therapeutic, and prognosis differences between these two subsets.

  10. [The first clinical description of granulomatosis with polyangiitis (known before as Wegener's granulomatosis)].

    Science.gov (United States)

    Mercado, Ulises

    2017-01-01

    Before 1950, cases of necrotizing vasculitis were commonly published in journals of pathology. Most of these cases were designated as polyarteritis nodosa. In 1952, the pathologist Pearl Zeek critically reviewed and summarized the literature dealing with polyarteritis nodosa and first grouped the different types of necrotizing vasculitis. But she omitted some types of not well-characterized vasculitis, among them granulomatosis with polyangiitis (Wegener's granulomatosis).

  11. Granulomatosis with Polyangiitis (GPA) Mimicking Tuberculosis.

    Science.gov (United States)

    Haridas, Vikram; Haridas, Kiran

    2017-03-01

    Granulomatosis with Polyangiitis (GPA) is a rare disease with varied clinical manifestations. We present a case of GPA which manifested initially with symptoms suggestive of meningeal tuberculosis. High index of suspicion and collective review of all clinical features helped in the correct diagnosis. Treatment of this case with rituximab provided significant symptomatic relief. © Journal of the Association of Physicians of India 2011.

  12. Characteristics of patients with orofacial granulomatosis.

    LENUS (Irish Health Repository)

    McCartan, B E

    2011-10-01

    Orofacial granulomatosis has mostly been described in reports of very small numbers of cases. Few large case groups have been described. The aim of this study was to describe the demographics, symptoms, clinical features and laboratory findings in a large cohort of cases.

  13. Postirradiation pulmonary fibrosis complicated by aspergilloma and bronchocentric granulomatosis

    Energy Technology Data Exchange (ETDEWEB)

    Makker, H.; McConnochie, K.; Gibbs, A.R. (Univ. of Wales College of Medicine, Llandough Hospital, Penarth (UK))

    1989-08-01

    An asthmatic patient requiring cortico-steroid treatment developed a pulmonary aspergilloma in an area of postmastectomy radiation fibrosis. At necropsy bronchocentric granulomatosis was also found. (author).

  14. Paget's disease of the breast in a male with lymphomatoid papulosis: a case report

    Directory of Open Access Journals (Sweden)

    Fouad Dina

    2011-01-01

    Full Text Available Abstract Introduction Paget's disease is an eczematous skin change of the nipple that is usually associated with an underlying breast malignancy. Male breast cancer represents only 1-3% of all breast malignancies and Paget's disease remains very rare. Case presentation We present the case of a 67-year-old Caucasian man with lymphomatoid papulosis who was diagnosed with Paget's disease of the nipple and who was treated successfully with surgery alone. We discuss the presentation, investigations, management and pathogenesis of Paget's disease of the nipple. Conclusion The case highlights the need to be vigilant when new skin lesions arise in the context of an underlying chronic skin disorder.

  15. Kidney involvement in a wegener granulomatosis case

    Directory of Open Access Journals (Sweden)

    Gioacchino Li Cavoli

    2012-01-01

    Full Text Available Wegener Granulomatosis is a systemic Anti-Neutrophil Cytoplasmic Autoantibody- associated Vasculitis, affecting small-to-medium vessels. Clinical presentation with simultaneous involvement of kidney and upper and lower respiratory tract is unusual. We report an instructive case of WG, analyzing clinical course, laboratory, and radiological features, kidney, lung, and larynx histological pictures. Besides renal biopsy, nephrology team performed larynx and lung biopsies because of unusual clinical presentation, computed tomography chest examination, and relevant malignancy risk regarding following immunosuppressant therapy.

  16. Treatment of Wegener’s granulomatosis

    Directory of Open Access Journals (Sweden)

    G. Valesini

    2011-09-01

    Full Text Available Treatment of Wegener’s granulomatosis, often a life-threatening disease, has greatly improved, considering that before corticosteroids and immunosuppressives were available, the average survival time of patients amounted to no more than 5 months. The management of Wegener’s granulomatosis can be divided in two stages: induction of remission and maintenance of remission. The standard regimen for the induction of remission consists of cyclophosphamide, 2 mg/kg/day orally, in combination with prednisone, 1mg/Kg/day orally, with a gradual tapering once remission has been obtained. To lower the overall cumulative dose, monthly intravenous pulses of cyclophosphamide have been evaluated. Other alternative treatments as high doses of corticosteroids, methotrexate, or plasmapheresis have been proposed, together as prophylaxis with trimethoprim-sulfamethoxazole. To minimize toxicity, for maintenance therapy other drugs are also used such as methotrexate, azathioprine, cyclosporine. Frequent therapeutic changes are needed due to the great variability of the disease; while important aspects are the recognition and treatment of relapse, and include not only the management of resistant disease, but also some particular aspects such as disease in chronic dialysis, renal transplant, pregnancy. Other cytotoxic drugs like leflunomide or mycofenolic mofetil appear to be promising, while new efforts to identify more effective and less toxic therapies include biologic products, such as high-dose immunoglobulin, TNF antagonists and other monoclonal antibodies. Many different kind of clinical trials are going on to better evaluate the real efficacy and safety of these treatments in Wegener’s granulomatosis.

  17. Diagnostic Consideration for Sinonasal Wegener's Granulomatosis Clinically Mistaken for Carcinoma

    Science.gov (United States)

    La Rosa, Cristina; Emmanuele, Carmela; Tranchina, Maria Grazia; Ippolito, Massimo; Cosentino, Sebastiano; Saita, Vincenzo; Fraggetta, Filippo

    2013-01-01

    We report a case of Wegener's granulomatosis clinically mistaken for carcinoma in a 21-year-old girl presenting with an ulcerated mass of the nasopharynx associated with enlarged laterocervical nodes. The lesion was clinically suspected as malignant on the basis of clinical and radiological findings (namely, computed tomography scan and positron emission tomography). However, multiple biopsies were not conclusive for malignancy showing histological change suggestive of Wegener's granulomatosis. A serum determination of cANCA supported the diagnosis of Wegener's granulomatosis. Clinical findings and image studies suggested an erroneous diagnosis of malignancy whereas a definitive diagnosis of Wegener's granulomatosis was achieved only after repeated biopsies thus leading to a correct therapeutic approach. The Wegener granulomatosis must be added to the list of the differential diagnoses of the masses of the nasopharynx associated with or without enlarged laterocervical nodes. PMID:24106630

  18. Deoxyspergualin in relapsing and refractory Wegener's granulomatosis

    DEFF Research Database (Denmark)

    Flossmann, O; Baslund, B; Bruchfeld, A

    2008-01-01

    at entry and prednisolone doses adjusted according to clinical status. Deoxyspergualin, 0.5 mg/kg per day, was self-administered by subcutaneous injection in six cycles of 21 days with a 7-day washout between cycles. Cycles were stopped early for white blood count less than 4000 cells/mm(3). The primary......-threatening (> or = grade 3) treatment-related adverse events occurred in 24 (53%) patients mostly due to leucopaenias. CONCLUSIONS: Deoxyspergualin achieved a high rate of disease remission and permitted prednisolone reduction in refractory or relapsing Wegener's granulomatosis. Adverse events were common but rarely led...

  19. Lymphomatoid contact dermatitis associated with textile dye at an unusual location.

    Science.gov (United States)

    Uzunçakmak, Tuğba Kevser; Akdeniz, Necmettin; Özkanlı, Şeyma; Türkoğlu, Zafer; Zemheri, Ebru Itır; Ka Radağ, Ayşe Serap

    2015-12-01

    Lymphomatoid contact dermatitis (LCD) is a rare variant of noneczematous allergic contact dermatitis, which can mimick parapsoriasis or early-stage mycosis fungoides with its atypical clinical and histopathological manifestation. Many different haptens have been reported to be associated with this reaction. Histopathological examination, immunhistochemistry, clonality tests, and patch tests are mandatory for diagnosis and differential diagnosis. We present a 48-year-old male with a four years history of a relapsing erythematous plaque on the glans penis. Topical corticosteroids had been prescribed but he complained of relapse upon withdrawal. Histopathological examination was consistent with LCD. Thin layer rapid use epicutaneous patch test result was (++) for disperse blue and nickel sulfate. We present this case because of its rarity and unusual localization. This kind of allergic contact dermatitis should be remembered in differential diagnosis of nonspesific pruritic plaques over the genital region.

  20. Lymphomatoid contact dermatitis associated with textile dye at an unusual location

    Directory of Open Access Journals (Sweden)

    Tuğba Kevser Uzunçakmak

    2015-01-01

    Full Text Available Lymphomatoid contact dermatitis (LCD is a rare variant of noneczematous allergic contact dermatitis, which can mimick parapsoriasis or early-stage mycosis fungoides with its atypical clinical and histopathological manifestation. Many different haptens have been reported to be associated with this reaction. Histopathological examination, immunhistochemistry, clonality tests, and patch tests are mandatory for diagnosis and differential diagnosis. We present a 48-year-old male with a four years history of a relapsing erythematous plaque on the glans penis. Topical corticosteroids had been prescribed but he complained of relapse upon withdrawal. Histopathological examination was consistent with LCD. Thin layer rapid use epicutaneous patch test result was (++ for disperse blue and nickel sulfate. We present this case because of its rarity and unusual localization. This kind of allergic contact dermatitis should be remembered in differential diagnosis of nonspesific pruritic plaques over the genital region.

  1. Lymphomatoid contact dermatitis associated with textile dye at an unusual location

    Science.gov (United States)

    Uzunçakmak, Tuğba Kevser; Akdeniz, Necmettin; Özkanlı, Şeyma; Türkoğlu, Zafer; Zemheri, Ebru Itır; Ka Radağ, Ayşe Serap

    2015-01-01

    Lymphomatoid contact dermatitis (LCD) is a rare variant of noneczematous allergic contact dermatitis, which can mimick parapsoriasis or early-stage mycosis fungoides with its atypical clinical and histopathological manifestation. Many different haptens have been reported to be associated with this reaction. Histopathological examination, immunhistochemistry, clonality tests, and patch tests are mandatory for diagnosis and differential diagnosis. We present a 48-year-old male with a four years history of a relapsing erythematous plaque on the glans penis. Topical corticosteroids had been prescribed but he complained of relapse upon withdrawal. Histopathological examination was consistent with LCD. Thin layer rapid use epicutaneous patch test result was (++) for disperse blue and nickel sulfate. We present this case because of its rarity and unusual localization. This kind of allergic contact dermatitis should be remembered in differential diagnosis of nonspesific pruritic plaques over the genital region. PMID:26904444

  2. Orofacial granulomatosis in a patient with Crohn's disease.

    NARCIS (Netherlands)

    Scheur, van de M.R.; Waal, van der RI; Volker - Dieben, H.J.M.; Knol, E.C.; Starink, T.M.; Waal, van der I.

    2003-01-01

    Orofacial granulomatosis encompasses the previously recognized clinical entities Melkersson-Rosenthal syndrome and cheilitis granulomatosa. We report the case of a 39-year-old patient with cheilitis granulomatosa, intestinal Crohn's disease, and optic neuropathy. Cheilitis granulomatosa and optic ne

  3. Linfoma de Burkitt en un portador de granulomatosis de Wegener

    Directory of Open Access Journals (Sweden)

    Kryssia Rodríguez-Castro

    2005-10-01

    Full Text Available Se reporta el caso de un paciente costarricense portador de granulomatosis de Wegener, en tratamiento con ciclofosfamida y prednisona, quien desarrolla un linfoma de Burkitt que lo lleva a la muerte. Se menciona la posible relación entre estas dos patologías.This is a report of a costarrican male with Wegener' s granulomatosis undergoing treatment with prednisone and cyclophosphamide, he developed a fatal Burkitt' s lymphoma. The possible relationship between these two disorders is addressed.

  4. Sinonasal Wegener's granulomatosis: CT characteristics

    Energy Technology Data Exchange (ETDEWEB)

    Benoudiba, F.; Marsot-Dupuch, K.; Rabia, Hadj M.; Lasjaunias, P. [Neuroradiology Department, Bicetre Hospital, 78 rue du General Leclerc, 94275 Le Kremlin Bicetre (France); Cabanne, J. [Department of Internal Medecine, Saint-Antoine Hospital, 184 rue du Faubourg Saint-Antoine, 75012 Paris (France); Bobin, S. [Head and Neck Department, Bicetre Hospital, 78, rue du General Leclerc, 94275 Le Kremlin Bicetre (France)

    2003-02-01

    Wegener's granulomatosis (WG) is a severe and potentially lethal granulomatosis. Even though no specific radiological criteria exist, CT may suggest the correct diagnosis at an early stage. Recent improvement in the prognosis is related to earlier diagnosis, allowing the initiation of efficient and specific treatment before any severe complications occur. We reviewed a series of WG cases in order to establish the CT diagnostic criteria. (orig.)

  5. Wegener's granulomatosis simulated by a T cell lymphoma of the lung

    OpenAIRE

    1991-01-01

    A case of primary T cell lymphoma of the lung associated with antineutrophil cytoplasmic antibody simulated Wegener's granulomatosis, the patient having features compatible with but not diagnostic of Wegener's granulomatosis.

  6. Successful endonasal dacryocystorhinostomy in a patient with Wegener’s granulomatosis

    Directory of Open Access Journals (Sweden)

    P Eloy

    2009-11-01

    Full Text Available P Eloy, E Leruth, B Bertrand, Ph RombauxENT and HNS department, Cliniques Universitaires de Mont-Godinne, Université Catholique de Louvain, 5530, Yvoir, BelgiumAbstract: Wegener’s granulomatosis (WG is one form of idiopathic autoimmune vasculitis. The disease has a predilection for the upper and lower respiratory tracts (lungs, nose, sinus, and kidneys. WG may be systemic, severe, and potentially lethal, but it may also be limited to the otolaryngological area or to the eyes and the orbits. Obstruction of the lacrimal pathway is a possible complication of the disease that affects approximately 7% of patients with WG. It usually occurs as a direct extension of sinonasal disease and typically is a late manifestation. Management of such a condition is generally viewed as difficult. We report the case of a patient with a quiescent WG limited to the otolaryngological area. This patient presented a bilateral obstruction of the nasolacrimal ducts caused by bilateral extensive adhesions in the nasal cavity. Because she had several episodes of left-side acute dacryocystitis which necessitated several courses of broad-spectrum antibiotics, she successfully underwent an endonasal endoscopic dacryocystorhinostomy using a diode laser and powered instrumentation. The authors describe the clinical case, the surgical technique, and review the literature.Keywords: Wegener’s granulomatosis, recurrent dacryocystitis, endonasal DCR, diode, laser, powered instrumentation

  7. Large Pyoderma Gangrenosum-Like Ulcers: A Rare Presentation of Granulomatosis with Polyangiitis

    Directory of Open Access Journals (Sweden)

    Basheer Tashtoush

    2014-01-01

    Full Text Available Granulomatosis with polyangiitis (GPA, formerly known as Wegener's granulomatosis (WG, is a rare systemic vasculitis that classically manifests as necrotizing granulomas of the upper and lower respiratory tract, kidneys, and blood vessels; however, it may affect any organ system, including the skin. Cutaneous manifestations occur in up to 45% of patients during the disease course, and are the presenting feature in 9% to 14% of patients. The most common skin lesion specific to GPA is palpable purpura, with the histopathologic correlate of leukocytoclastic vasculitis. However, a wide range of clinical and histologic features may be seen. We herein report a case of a previously healthy 52-year-old Caucasian man who presented with multiple progressively enlarging painful ulcers on his face, upper extremities, back, and abdomen over a two-month period. Skin biopsies revealed pyoderma gangrenosum-like features. Serological tests were positive for PR3/c-ANCA. Six months later, the patient developed recurrent episodes of sinusitis associated with nasal bleeds and eventually nasal septum perforation. Despite aggressive treatment with Cyclophosphamide and steroids over one year, the patient had persistent nonhealing large ulcers and developed multiple lung nodules with cavitary lesions.

  8. Miescher's granulomatosis (granulomatosis disciformis chronica et progressiva in a non-diabetic patient – case report

    Directory of Open Access Journals (Sweden)

    Brzezińska-Wcisło Ligia

    2009-08-01

    Full Text Available Abstract Introduction Necrobiosis lipoidica diabeticorum is a rare disease of unclear etiology, that occurs in about 1% of diabetic patients. Case report We present case of granulomatosis disciformis chronica et progressiva Miescher with good response to systemic corticosteroids therapy. Patient 45 years old woman, with primary yellow-brown areas skin lesions, with foci well separated from surroundings on both lower legs, that occurred 5 years ago. In laboratory tests there was no abnormalities. Because of advance suggestion (after last admit in dermatological ward of observation according to xantogranuloma necrobioticum tests for paraproteinemia were made. Immunoelectrophoresis, IgG, IgM, IgA levels, kappa light chain, lambda heavy chain; were correct, Bence-Johns protein-negative. During hospitalization in Clinic methylprednisolone in dose of 32 mg od, vascular drugs and local steroidotherapy was applied with good therapeutic response. Conclusion We described case of typical clinical and histological characters of necrobiosis lipoidica. without diabetes-granulomatosis disciformis chronica et progressiva Miescher that despite of suspicion of proper diagnosis for a long time was not treat effective.

  9. Wegener's granulomatosis in childhood

    Energy Technology Data Exchange (ETDEWEB)

    McHugh, K.; Manson, D. (Hospital for Sick Children, Toronto, ON (Canada). Dept. of Diagnostic Imaging); Eberhard, B.A.; Shore, A.; Laxer, R.M. (Hospital for Sick Children, Toronto, ON (Canada). Div. of Rheumatology)

    1991-12-01

    The authors reviewed the medical histories and radiological examinations of five pediatric patients with a histologic diagnosis of Wegener's granulomatosis (WG) seen over a six year period in whom a total of 22 thoracic CT scans were performed. Involvement of both the upper and lower respiratory tracts was seen in all patients at presentation. One patient had subglottic stenosis necessitating tracheotomy. Pulmonary hemorrhage occurred in three patients at initial diagnosis. Classic cavitary lung nodules were seen in two patients - one at initial presentation, the other at relapse. The plain radiographic lower respiratory tract manifestations of pediatric WG were protean both at initial presentation and during follow up. Similarly, disease expression was highly variable on thoracic CT examinations but, overall multifocal parenchymal infiltrates with or without small peripheral nodules were the commonest thoracic CT manifestations. As a consequence of cytotoxic and corticosteroid therapy the long-term prognosis of WG has improved considerably in recent years. Knowledge of the varied patterns of the primary disease and potential for iatrogenic complications are necessary for successful radiologic assessment of pediatric patients with WG. (orig.).

  10. [Teutschlaender lipo-calcino-granulomatosis or tumoral calcinosis of Inclan (author's transl)].

    Science.gov (United States)

    Barrière, H; Welin, J; Lenne, Y; Visset, J; Vigier, P

    1977-02-01

    The present report describes a typical case of tumoral calcinosis. Differential diagnosis was initially directed towards sarcoma suggested by fibrous connective tissue surrounding cystlike cavities. The course of this disease led to chronic multiple fistulae with secondary infection in spite of two attempts of surgical excision. This case adds further support to the results of earlier reports showing no specific biological abnormalities. The present sutyd indicates otherwise that tumoral calcinosis and lipo-calcino-granulomatosis of Teutschlaender are the same condition. The pathogenesis of this affection remains enigmatic; there is no evidence that the lipidic excess should be the beginning of the process, but this disease must be considered as a distinctive form of calcinosis. The surgical exeresis is the only possible treatment, even if it doesn't prevent recurrence.

  11. Yttrium Y 90 Ibritumomab Tiuxetan, Fludarabine, Radiation Therapy, and Donor Stem Cell Transplant in Treating Patients With Relapsed or Refractory Non-Hodgkin's Lymphoma

    Science.gov (United States)

    2016-03-21

    B-cell Chronic Lymphocytic Leukemia; Nodal Marginal Zone B-cell Lymphoma; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Grade III Lymphomatoid Granulomatosis; Recurrent Adult Immunoblastic Large Cell Lymphoma; Recurrent Adult Lymphoblastic Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Small Lymphocytic Lymphoma; Splenic Marginal Zone Lymphoma; Waldenström Macroglobulinemia

  12. A case of fever of unknown origin: necrotizing sarcoid granulomatosis.

    Science.gov (United States)

    Unlü, G; Onyılmaz, T A; Barış, S A; Turhan, N; Vural, C; Başyiğit, I; Boyacı, H

    2014-01-01

    Necrotizing sarcoid granulomatosis is a rare type of vasculitis; its etiology and pathogenesis are still unknown. The disease primarily affects the lungs, although extra-pulmonary involvement has been reported. The typical symptoms are cough, chest pain, dyspnea, and weight loss; high temperatures have been reported in rare cases. We present the case of a 65-year-old woman who was diagnosed with lymph node tuberculosis, for which she received treatment for six months. The patient experienced no improvement in her symptoms, which included fever, weakness and dyspnea. A re-evaluation of previously collected thoracoscopic biopsy material revealed compatibility with necrotizing sarcoid granulomatosis.

  13. Eosinophilic granulomatosis with polyangiitis: an overview

    Directory of Open Access Journals (Sweden)

    Andrea eGioffredi

    2014-11-01

    Full Text Available Eosinophilic granulomatosis with polyangiitis (EGPA is a multisystemic disorder, belonging to the small vessel ANCA-associated vasculitis, defined as a eosinophil-rich and necrotizing granulomatous inflammation often involving the respiratory tract, and necrotizing vasculitis predominantly affecting small to medium-sized vessels, associated with asthma and eosinophilia. EGPA pathogenesis is not well known: HLA-DRB1*04 and *07, HLA-DRB4 and IL10.2 haplotype of the IL-10 promoter gene are the most studied genetic determinants. Among the acquired pathogenetic factors, the exposure to different allergens, infections, vaccinations, drugs and silica exposure have been involved.Eosinophils are the most characteristic cells in EGPA and different studies have demonstrated their role as effector and immunoregulatory cells.EGPA is considered a disease with a prevalent activation of the Th2 cellular-mediated inflammatory response but also humoral immunity plays an important role. A link between B and T inflammatory responses may explain different disease features. EGPA typically develops into three sequential phases: the allergic phase, distinguished by the occurrence of asthma, allergic rhinitis and sinusitis, the eosinophilic phase, in which the main pathological finding is the eosinophilic organ infiltrations (e.g. lungs, heart and gastrointestinal system and the vasculitic phase, characterized by purpura, peripheral neuropathy and constitutional symptoms.ANCA (especially pANCA anti-MPO are present in 40-60% of the patients. An elevation of IgG4 is frequently found. Corticosteroids and cyclophosphamide are classically used for remission induction, while azathioprine and methotrexate are the therapeutic options for remission maintenance. B-cell depletion with rituximab has shown promising results for remission induction.

  14. Ispinesib in Treating Young Patients With Relapsed or Refractory Solid Tumors or Lymphoma

    Science.gov (United States)

    2013-01-15

    Childhood Burkitt Lymphoma; Childhood Central Nervous System Germ Cell Tumor; Childhood Choroid Plexus Tumor; Childhood Craniopharyngioma; Childhood Grade I Meningioma; Childhood Grade II Meningioma; Childhood Grade III Meningioma; Childhood High-grade Cerebral Astrocytoma; Childhood Infratentorial Ependymoma; Childhood Low-grade Cerebral Astrocytoma; Childhood Spinal Cord Neoplasm; Childhood Supratentorial Ependymoma; Recurrent Childhood Brain Stem Glioma; Recurrent Childhood Brain Tumor; Recurrent Childhood Cerebellar Astrocytoma; Recurrent Childhood Cerebral Astrocytoma; Recurrent Childhood Ependymoma; Recurrent Childhood Grade III Lymphomatoid Granulomatosis; Recurrent Childhood Large Cell Lymphoma; Recurrent Childhood Lymphoblastic Lymphoma; Recurrent Childhood Medulloblastoma; Recurrent Childhood Small Noncleaved Cell Lymphoma; Recurrent Childhood Supratentorial Primitive Neuroectodermal Tumor; Recurrent Childhood Visual Pathway and Hypothalamic Glioma; Unspecified Childhood Solid Tumor, Protocol Specific

  15. "Wegener’s granulomatosis in a patient with Rheumatoid arthritis "

    Directory of Open Access Journals (Sweden)

    "Ahmadi Nejad Z

    2001-06-01

    Full Text Available Wegener’s Granulomatosis is a necrotizing granulomatous vasculitis involving small and medium sized vessels. The syndrome is classically defined as having involvement of kidney, lungs and upper respiratory tract (i.e. sinuses. Wegener’s Granulomatosis may be present in other autoimmune or inflammatory diseases, particulary systemic lupus erythematosis (SLE, but most frequently has been associated with polyarteritis and glumerulonephritis.We present a case of wegener’s Granulomatosis (WG in a middle age lady with Rheumatiod Arthritis (RA; and discuss the implications of these two conditions co-existing in one patient. As for as we are aware, through medline and interent research, this is probably the fourth case with such as association of WG and RA and the fist one in Iran.Presentation of new uncontrollable sing and symptoms, in a previously well controlled RA patient, might suggest a new overlapping syndrome like Wegener’s Granulomatosis besides to flare up of previous disease as differential diagnosis

  16. Patch testing for food-associated allergies in orofacial granulomatosis.

    LENUS (Irish Health Repository)

    Fitzpatrick, Laura

    2011-01-01

    Food-associated allergies, especially to benzoates and cinnamon-related compounds, have been associated with orofacial granulomatosis and both standard and urticarial patch testing have been used to detect such allergies. Elimination diets have also been shown to be effective in some patients.

  17. Bronchoplastic procedure for an unusual indication--Wegener's granulomatosis.

    LENUS (Irish Health Repository)

    Soo, Alan

    2009-09-01

    Wegener\\'s granulomatosis (WG) is a systemic vasculitic condition that commonly affects the lung and kidneys. With improvement in medical therapy, airway complications are increasingly encountered and are difficult to manage. Here, we present a case whereby a patient presenting with airway complication is successfully treated with surgery.

  18. A Case of Wegener’s Granulomatosis Presenting with Unilateral Facial Nerve Palsy

    Directory of Open Access Journals (Sweden)

    Roy Ujjawal

    2016-01-01

    Full Text Available Wegener’s granulomatosis or granulomatosis with polyangiitis is a necrotizing vasculitis affecting both arterioles and venules. The disease is characterized by the classical triad involving acute inflammation of the upper and lower respiratory tracts with renal involvement. However, the disease pathology can affect any organ system. This case presents Wegener’s granulomatosis presenting with facial nerve palsy as the first manifestation of the disease, which is rarely reported in medical literature.

  19. Fludarabine Phosphate, Low-Dose Total-Body Irradiation, and Donor Stem Cell Transplant Followed by Cyclosporine, Mycophenolate Mofetil, Donor Lymphocyte Infusion in Treating Patients With Hematopoietic Cancer

    Science.gov (United States)

    2016-08-01

    Acute Undifferentiated Leukemia; Adult Nasal Type Extranodal NK/T-cell Lymphoma; Anaplastic Large Cell Lymphoma; Angioimmunoblastic T-cell Lymphoma; Childhood Burkitt Lymphoma; Childhood Diffuse Large Cell Lymphoma; Childhood Grade III Lymphomatoid Granulomatosis; Childhood Immunoblastic Large Cell Lymphoma; Childhood Myelodysplastic Syndromes; Childhood Nasal Type Extranodal NK/T-cell Lymphoma; Chronic Myelomonocytic Leukemia; Cutaneous B-cell Non-Hodgkin Lymphoma; de Novo Myelodysplastic Syndromes; Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Hepatosplenic T-cell Lymphoma; Intraocular Lymphoma; Juvenile Myelomonocytic Leukemia; Mast Cell Leukemia; Myelodysplastic/Myeloproliferative Neoplasm, Unclassifiable; Myeloid/NK-cell Acute Leukemia; Nodal Marginal Zone B-cell Lymphoma; Noncutaneous Extranodal Lymphoma; Peripheral T-cell Lymphoma; Post-transplant Lymphoproliferative Disorder; Previously Treated Myelodysplastic Syndromes; Primary Systemic Amyloidosis; Recurrent Adult Acute Lymphoblastic Leukemia; Recurrent Adult Acute Myeloid Leukemia; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Grade III Lymphomatoid Granulomatosis; Recurrent Adult Hodgkin Lymphoma; Recurrent Adult Immunoblastic Large Cell Lymphoma; Recurrent Adult Lymphoblastic Lymphoma; Recurrent Adult T-cell Leukemia/Lymphoma; Recurrent Childhood Acute Lymphoblastic Leukemia; Recurrent Childhood Acute Myeloid Leukemia; Recurrent Childhood Anaplastic Large Cell Lymphoma; Recurrent Childhood Grade III Lymphomatoid Granulomatosis; Recurrent Childhood Large Cell Lymphoma; Recurrent Childhood Lymphoblastic Lymphoma; Recurrent Childhood Small Noncleaved Cell Lymphoma; Recurrent Cutaneous T-cell Non-Hodgkin Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma

  20. Wegener's granulomatosis: description of a case with oral manifestation.

    Science.gov (United States)

    Reboll-Ferrer, Rosa Maria; Zapater-Latorre, Enrique; Calabuig-Crespo, Consuelo; Basterra-Alegría, Jorge

    2010-07-01

    Wegener's granulomatosis is a multisystemic granulomatous vasculitis that predominantly affects the airways and the kidneys, but may affect any organ. Otorhinolaryngological manifestations may be oral ulcers, gingival swelling or septal perforations that can cause saddle nose deformities, rhinitis, sinusitis and hearing loss. The oral ulcers usually occur when the disease is advanced. Renal involvement is characterized by focal and segmental glomerulosclerosis, and determines the evolution. The diagnosis is made by clinical symptoms and signs, the presence of c-ANCA and a positive biopsy. The anatomic pathology is characterized by vasculitis, granulomatous inflammation with multinuclear giant cells and necrosis. The prognosis has improved as a result of treatment with immunosuppressants associated with corticosteroids. We report a case of a 53-year-old patient with Wegener's granulomatosis with oral manifestation, which began as chronic mastitis.

  1. Wegener′s granulomatosis disease mimicking pulmonary tuberculosis

    Directory of Open Access Journals (Sweden)

    Naveen Pandhi

    2015-01-01

    Full Text Available Wegener′s granulomatosis (WG is an uncommon autoimmune disease with multi-system involvement that manifests as vasculitis, granulomatosis, and necrosis. While its standard form involves the upper and lower respiratory tracts and kidneys, it may essentially involve any organ. We present a case in a young female patient presenting with symptoms of cough with expectoration, fever, dyspnea and chest discomfort, having cavitating lesion in right upper lobe and nodule in left upper lobe with bilateral paranasal sinus involvement on computed tomography. Allergic rhino-sinusitis with pulmonary tuberculosis was suspected, and patient was investigated further. Upon further investigation, renal involvement was detected, and serology revealed cytoplasmic antineutrophil cytoplasmic antibody. WG was suspected and was proven by histopathology of nasal tissue which revealed necrotizing granulomas.

  2. Case of granulomatosis with polyangiitis (Wegener's granulomatosis) manifested with asymptomatic intracerebral hemorrhage.

    Science.gov (United States)

    Takaoka, Hirokazu; Hashimoto, Atsushi; Nogi, Shinichi; Iwata, Kanako; Futami, Hidekazu; Arinuma, Yoshiyuki; Shimada, Kota; Nakayama, Hisanori; Komiya, Akiko; Furukawa, Hiroshi; Matsui, Toshihiro; Tohma, Shigeto

    2013-01-01

    A 46-year-old man, who had had sinusitis, developed bilateral omalgia, petechiae on his lower extremities and a congested right eye. A blood test detected elevated serum C-reactive protein level. Computed tomography incidentally found an acute lesion of thalamic hemorrhage without neurological symptoms and no specific therapy was given at the time. Thereafter, he developed vertigo, vomiting and pneumonia for which antibiotics were ineffective. He was referred and admitted to our hospital. Further, aural and renal lesions, and presence of serum proteinase 3-antineutrophil cytoplasmic antibody (PR3-ANCA) confirmed his diagnosis of granulomatosis with polyangiitis (Wegener's) (GPA). With corticosteroid and cyclophosphamide therapy, his symptoms disappeared in two months along with faded PR3-ANCA. Afterward he showed neither new cerebral lesion nor symptom. This is a rare case of GPA manifested with asymptomatic intracerebral hemorrhage. It should be noted that GPA could cause various manifestations in central nervous system such as a fatal or an asymptomatic hemorrhagic lesion, which might respond to immunosuppressive therapy.

  3. Granulomatosis with Polyangiitis Presenting as a Parotid Gland Abscess

    Directory of Open Access Journals (Sweden)

    Blenda Dias

    2015-01-01

    Full Text Available Granulomatosis with polyangiitis (GPA is a small-vessel vasculitis consisting of necrotizing granulomatous lesions in airways and focal necrotizing glomerulonephritis. However, it may affect other sites such as the skin, central nervous system, eyes, heart, gastrointestinal tract, and liver. We describe a rare case of GPA in which the initial manifestation was the involvement of the parotid gland mimicking a pyogenic abscess.

  4. Wegener's granulomatosis : description of a case with oral manifestation

    OpenAIRE

    Reboll Ferrer, Rosa María; Zapater Latorre, Enrique; Calabuig Crespo , Consuelo; Basterra Alegría, Jorge

    2010-01-01

    Wegener?s granulomatosis is a multisystemic granulomatous vasculitis that predominantly affects the airways and the kidneys, but may affect any organ. Otorhinolaryngological manifestations may be oral ulcers, gingival swelling or septal perforations that can cause saddle nose deformities, rhinitis, sinusitis and hearing loss. The oral ulcers usually occur when the disease is advanced. Renal involvement is characterized by focal and segmental glomerulosclerosis, and determines the evolutio...

  5. Meningeal involvement in Wegener's granulomatosis is associated with localized disease.

    Science.gov (United States)

    Di Comite, G; Bozzolo, E P; Praderio, L; Tresoldi, M; Sabbadini, M G

    2006-01-01

    Meningeal involvement is a rare occurrence in Wegener's Granulomatosis (WG). A Medline search uncovered only 48 previously reported cases. Here we describe the clinical features of meningeal involvement in WG and to evaluate the association with systemic disease extension. Through a systematic literature review of papers concerning meningeal involvement in WG, we collected and analysed data about sex, age, disease extension, symptoms, cerebrospinal fluid examination, imaging, ANCA and histology about previously reported patients. Headache is almost always the first symptom of meningeal involvement in WG. Later in the course of the disease other abnormalities may develop. Among them cranial nerve palsy, seizures and encephalopathy are the most frequent. Diagnosis is obtained by neuroimaging, which may disclose two distinct patterns of meningeal thickening: diffuse or focal. 62.9% of patients tests positive for ANCA. Histology typically shows necrotizing granulomatosis. Meningeal involvement is by far more frequent in the setting of localized WG. Meningitis is a rare complication of WG. It usually develops in patients with localized disease who are more likely to have destructive lesions of the upper airways. It may be recognized by a constellation of clinical and radiological findings and by histological signs of necrotizing granulomatosis, with little or no vasculitis.

  6. Acute Pancreatitis as the First Presentation of Wegener's Granulomatosis

    Directory of Open Access Journals (Sweden)

    Mohammed Abu-Hilal

    2008-05-01

    Full Text Available Context Wegener’s granulomatosis is a systemic vasculitis with prominent involvement of the respiratory tract and kidney. An association between acute pancreatitis and Wegener's granulomatosis is rarely reported and is even rarer as the first presentation. This can result in diagnostic difficulty and may allow severe pancreatitis to develop with potentially poor outcome. Case report We report a rare case with fatal outcome of vasculitis consistent with Wegener’s granulomatosis presenting as acute pancreatitis in a 20-year-old female. The patient was admitted with worsening abdominal pain associated with nausea and loss of appetite. Accepted causes of acute pancreatitis were excluded and granulomatous vasculitis of the pancreas was confirmed from immunological profile, computed tomography and histology. As the disease progressed the patient experienced cutaneous, pulmonary, renal and severe gut involvement. Thirteen months from diagnosis the patient died of multi-organ failure despite appropriate surgical and immunosuppressive therapy. Conclusion Vasculitic disease of the pancreas is rare but should be considered when other causes have been appropriately ruled out. Careful radiological, immunological and histological diagnosis is necessary and early immunosuppressant therapy in conjunction with advice from immunologists is essential to avoid the poor outcome reported in this and other case reports.

  7. Computed tomographic characteristics of eosinophilic pulmonary granulomatosis in five dogs.

    Science.gov (United States)

    Fina, Caroline; Vignoli, Massimo; Terragni, Rossella; Rossi, Federica; Wisner, Erik; Saunders, Jimmy H

    2014-01-01

    Canine pulmonary eosinophilic granulomatosis is a rare inflammatory pulmonary disease characterized by formation of eosinophilic granulomas that tend to obliterate the normal pulmonary architecture. The purpose of this retrospective study was to describe the CT characteristics of confirmed idiopathic pulmonary eosinophilic granulomatosis in a group of dogs. Five dogs met inclusion criteria. All patients were young adult dogs of variable breeds. No dog had concurrent occult heartworm disease. Computed tomographic characteristics most commonly included pulmonary masses and nodules of variable size, and lesions were most commonly located in the caudal lung lobes. Four dogs had large pulmonary masses with or without additional nodules and one dog had nodular lesions disseminated throughout the entire lung parenchyma. All large eosinophilic granulomas were smoothly margined, heterogeneous pulmonary masses displaying heterogeneous contrast enhancement. A honeycomb-like enhancement pattern was observed in all but one mass and consisted of multiple hyperattenuating rims delineating central hypoattenuating areas, suggestive of bronchiectatic lung with peripheral enhancing airway walls and fluid-filled, necrotic bronchial lumen. One dog had evidence of tracheobronchial lymphadenopathy. Findings indicated that canine eosinophilic pulmonary granulomatosis should be included as a differential diagnosis for dogs with CT characteristics of multiple pulmonary masses and/or nodules in caudal lung lobes, and a honeycomb-like enhancement pattern in masses after intravenous administration of iodinated contrast medium.

  8. Occult Renal Granulomatous Inflammatory Lesions in Granulomatosis with Polyangiitis Detected by 18F-FDG PET/CT.

    Science.gov (United States)

    Fu, Zhanli; Liu, Meng; Li, Ziao; Zhang, Jin; Li, Qian

    2017-09-01

    A 33-year-old woman with a 7-month history of granulomatosis with polyangiitis F-FDG PET/CT scan due to low-grade intermittent fever and elevation of serum inflammatory markers, which revealed multiple hypermetabolic lesions in bilateral kidneys. Biopsy of a lesion showed granulomatosis with polyangiitis, consistent with renal involvement of granulomatosis with polyangiitis.

  9. Malignancies in Wegener's granulomatosis: incidence and relation to cyclophosphamide therapy in a cohort of 293 patients

    DEFF Research Database (Denmark)

    Faurschou, Mikkel; Sørensen, Inge Juul; Mellemkjaer, Lene

    2008-01-01

    To describe the incidence of malignancies in a cohort of Danish patients with Wegener's granulomatosis (WG) and to investigate the cancer risk associated with cyclophosphamide (CYC) -therapy in WG.......To describe the incidence of malignancies in a cohort of Danish patients with Wegener's granulomatosis (WG) and to investigate the cancer risk associated with cyclophosphamide (CYC) -therapy in WG....

  10. Diagnostic Consideration for Sinonasal Wegener’s Granulomatosis Clinically Mistaken for Carcinoma

    Directory of Open Access Journals (Sweden)

    Cristina La Rosa

    2013-01-01

    Full Text Available We report a case of Wegener’s granulomatosis clinically mistaken for carcinoma in a 21-year-old girl presenting with an ulcerated mass of the nasopharynx associated with enlarged laterocervical nodes. The lesion was clinically suspected as malignant on the basis of clinical and radiological findings (namely, computed tomography scan and positron emission tomography. However, multiple biopsies were not conclusive for malignancy showing histological change suggestive of Wegener’s granulomatosis. A serum determination of cANCA supported the diagnosis of Wegener’s granulomatosis. Clinical findings and image studies suggested an erroneous diagnosis of malignancy whereas a definitive diagnosis of Wegener’s granulomatosis was achieved only after repeated biopsies thus leading to a correct therapeutic approach. The Wegener granulomatosis must be added to the list of the differential diagnoses of the masses of the nasopharynx associated with or without enlarged laterocervical nodes.

  11. The use of abatacept in debilitating cavitating lung disease associated with rheumatoid arthritis, bronchocentric granulomatosis and aspergillosis.

    LENUS (Irish Health Repository)

    Neff, K

    2010-06-01

    A case of debilitating cavitating lung disease associated with rheumatoid arthritis and bronchocentric granulomatosis, which failed to respond to conventional medical or surgical treatment, is described. The patient was treated over 10 years with steroids, antimicrobial agents, disease-modifying antirheumatoid drugs and surgery. Lung function continued to decline and the patient presented for admission with recurrent pneumonia. Abatacept was initiated to modify the underlying immunopathology. Following 12 months of treatment with abatacept the patient has demonstrable improvement in lung function and lung anatomy, and has not presented to hospital with pneumonia. She has tolerated the treatment without complication. The use of abatacept has stabilised the lung disease in this case in the medium term and prevented readmission to hospital. These results suggest a larger role for abatacept in those with such disease in the future and may warrant further investigation.

  12. [Hereditary systemic autoinflammatory diseases. Part II: cryopyrin-associated periodic syndromes, pediatric systemic granulomatosis and PAPA syndrome].

    Science.gov (United States)

    Aróstegui, Juan I; Yagüe, Jordi

    2008-03-29

    Hereditary systemic autoinflammatory diseases result from a genetically-based dysregulated inflammatory process, and are clinically characterized by recurrent or persistent systemic inflammatory episodes, which typically occur in the absence of infectious, neoplastic or autoimmune etiology. Elucidation of their molecular basis has enabled the use of genetic analyses to achieve an accurate and definitive diagnosis, and to establish a tailored treatment. The present review is the second and last part of an updated and comprehensive overview of hereditary systemic autoinflammatory diseases, and will introduce persistent, non-periodic autoinflammatory diseases, such as: a) the group of cryopyrin-associated periodic syndromes (CAPS), which includes familial cold-induced autoinflammatory syndrome (FCAS), Muckle-Wells syndrome, and CINCA-NOMID syndrome; b) the group of pediatric systemic granulomatosis, which includes both Blau syndrome and early-onset sarcoidosis, and c) the pyogenic sterile arthritis, pyoderma gangrenosum and acne (PAPA) syndrome.

  13. Kaposi's sarcoma following immune suppressive therapy for Wegener's granulomatosis.

    Science.gov (United States)

    Deschênes, Isabelle; Dion, Louise; Beauchesne, Claude; de Brum-Fernandes, Artur

    2003-03-01

    The association between Kaposi's sarcoma and infection with human herpesvirus 8 is now well recognized. Immunologic impairment is associated with 2 forms of Kaposi's sarcoma, epidemic [associated with human immunodeficiency virus (HIV) infection] and iatrogenic (associated with immunosuppressive treatment); both forms have become more common during the last decade. We describe an HIV negative 54-year-old man who developed Kaposi's sarcoma 2 months after the beginning of immuno-suppressive therapy for Wegener's granulomatosis (WG). With tapering of medication, complete remission of Kaposi's sarcoma was achieved in one year. To our knowledge, this is the second reported case of iatrogenic Kaposi's sarcoma in a patient with WG.

  14. Infectious complication or exacerbation of granulomatosis with polyangiitis?

    Science.gov (United States)

    Masiak, Anna; Struk-Panfill, Małgorzata; Zdrojewski, Zbigniew

    2015-01-01

    Granulomatosis with polyangiitis (GPA) is a primary, systemic small vessel vasculitis. The respiratory tract is typically involved in the course of the disease. Abnormalities on the chest radiograph are noted in more than 70% patients at some point during their disease history. In some clinical situations it is difficult to distinguish whether symptoms result from the underlying disease or are a symptom of infection. In these clinical situations, chest computed tomography (CT) can be very useful. We present a patient with GPA localized mainly in the respiratory tract with sudden deterioration of the general state and new abnormalities revealed in the CT of the chest.

  15. Pancreatic mass as an initial presentation of severe Wegener's granulomatosis

    Science.gov (United States)

    Valerieva, Yana; Golemanov, Branimir; Tzolova, Nadezhda; Mitova, Rumiana

    2013-01-01

    Acute pancreatitis or a pancreatic mass is a very rare initial presentation of Wegener's granu-lomatosis. A 62-year-old woman presented with tumor-like pancreatitis and otitis media Abdominal ultrasound and magnetic resonance suggested the presence of pancreatic tumor. Ultrasound-guided fine needle aspiration was negative. Distal pancreatic resection and splenectomy were performed and histopathology proved Wegener's vasculitis of the pancreas and spleen. Azathioprine and steroids were subsequently started and six months later the patient was asymptomatic. Involvement of the pancreas could be considered as a presenting symptom of Wegener's vasculitis. PMID:24714250

  16. A case of exorbitism in association with Wegener's granulomatosis with renal involvement.

    Science.gov (United States)

    Beji, S; Fatma, L Ben; Chebbi, A; Rais, L; Krid, M; Smaoui, W; Maiz, H Ben; Zouaghi, K; Moussa, F Ben

    2012-03-01

    Wegener's granulomatosis (WG) is a necrotizing granulomatous vasculitis involving the nose, paranasal sinuses, lungs, and kidneys. Ocular involvement can occur in about 50% of cases. There are very few reports of WG with orbital inflammation and exorbitism. We report a case of a female patient who presented with exorbitism related to orbital inflammation secondary to WG, with renal involvement. A 29-year-old woman with a previous history of recurrent pan-sinusitis presented with bilateral exophthalmos and renal failure with rapidly progressive glomerulonephritis. Computed tomography showed extensive bilateral soft tissue in the retro-orbital area. Immunologic tests showed the presence of type-C anti-neutrophil cytoplasmic antibodies and renal biopsy revealed pauci immune crescentic glomerulonephritis. The patient was treated with corticosteroids and pulses of cyclophosphamide followed by azathioprine and trimethoprim-sulfamethoxazole. After a follow-up of 10 months, the renal outcome was favorable with improvement of renal function but there was persistence of exorbitism and loss of visual function. Our case suggests that WG should be considered in the differential diagnosis of persistent bilateral exophthalmos. Prompt recognition of this early manifestation is important for the institution of early treatment.

  17. A case of exorbitism in association with Wegener′s granulomatosis with renal involvement

    Directory of Open Access Journals (Sweden)

    S Beji

    2012-01-01

    Full Text Available Wegener′s granulomatosis (WG is a necrotizing granulomatous vasculitis invol-ving the nose, paranasal sinuses, lungs, and kidneys. Ocular involvement can occur in about 50% of cases. There are very few reports of WG with orbital inflammation and exorbitism. We report a case of a female patient who presented with exorbitism related to orbital inflammation secondary to WG, with renal involvement. A 29-year-old woman with a previous history of recurrent pan-sinusitis presented with bilateral exophthalmos and renal failure with rapidly progressive glo-merulonephritis. Computed tomography showed extensive bilateral soft tissue in the retro-orbital area. Immunologic tests showed the presence of type-C anti-neutrophil cytoplasmic antibodies and renal biopsy revealed pauci immune crescentic glomerulonephritis. The patient was treated with corticosteroids and pulses of cyclophosphamide followed by azathioprine and trimethoprim-sulfamethoxazole. After a follow-up of 10 months, the renal outcome was favorable with improvement of renal function but there was persistence of exorbitism and loss of visual function. Our case suggests that WG should be considered in the differential diagnosis of persistent bila-teral exophthalmos. Prompt recognition of this early manifestation is important for the institution of early treatment.

  18. Pulmonary manifestations of wegener granulomatosis: CT findings in 57 patients and a review of the literature

    Energy Technology Data Exchange (ETDEWEB)

    Lohrmann, Christian; Uhl, Markus; Kotter, Elmar; Burger, Dieter; Ghanem, Nadir; Langer, Mathias

    2005-03-01

    Wegener granulomatosis is a multisystem disease of unknown cause characterized by a necrotizing granulomatous vasculitis. In comparison to other vasculitides, the lung is the most common organ involved in wegener granulomatosis presenting with a very aggressive airways pathology and chronic relapsing course. Chest radiographs fail to describe the pattern and distribution of thoracic pathology sufficiently, and CT has shown to be more sensitive for detecting lung involvement. We present the CT findings of 57 patients with wegener granulomatosis and a review of the literature.

  19. Rituximab in Treating Patients Undergoing Donor Peripheral Blood Stem Cell Transplant for Relapsed or Refractory B-cell Lymphoma

    Science.gov (United States)

    2015-11-23

    B-cell Adult Acute Lymphoblastic Leukemia; B-cell Childhood Acute Lymphoblastic Leukemia; B-cell Chronic Lymphocytic Leukemia; Childhood Burkitt Lymphoma; Childhood Diffuse Large Cell Lymphoma; Childhood Immunoblastic Large Cell Lymphoma; Cutaneous B-cell Non-Hodgkin Lymphoma; Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Intraocular Lymphoma; Nodal Marginal Zone B-cell Lymphoma; Post-transplant Lymphoproliferative Disorder; Recurrent Adult Acute Lymphoblastic Leukemia; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Grade III Lymphomatoid Granulomatosis; Recurrent Adult Hodgkin Lymphoma; Recurrent Adult Immunoblastic Large Cell Lymphoma; Recurrent Adult Lymphoblastic Lymphoma; Recurrent Childhood Acute Lymphoblastic Leukemia; Recurrent Childhood Grade III Lymphomatoid Granulomatosis; Recurrent Childhood Large Cell Lymphoma; Recurrent Childhood Lymphoblastic Lymphoma; Recurrent Childhood Small Noncleaved Cell Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Small Lymphocytic Lymphoma; Recurrent/Refractory Childhood Hodgkin Lymphoma; Refractory Chronic Lymphocytic Leukemia; Refractory Hairy Cell Leukemia; Small Intestine Lymphoma; Splenic Marginal Zone Lymphoma; Testicular Lymphoma; Waldenström Macroglobulinemia

  20. Fludarabine Phosphate, Melphalan, and Low-Dose Total-Body Irradiation Followed by Donor Peripheral Blood Stem Cell Transplant in Treating Patients With Hematologic Malignancies

    Science.gov (United States)

    2016-10-26

    Accelerated Phase Chronic Myelogenous Leukemia; Adult Acute Lymphoblastic Leukemia in Remission; Adult Acute Myeloid Leukemia in Remission; Adult Acute Myeloid Leukemia With 11q23 (MLL) Abnormalities; Adult Acute Myeloid Leukemia With Del(5q); Adult Acute Myeloid Leukemia With Inv(16)(p13;q22); Adult Acute Myeloid Leukemia With t(15;17)(q22;q12); Adult Acute Myeloid Leukemia With t(16;16)(p13;q22); Adult Acute Myeloid Leukemia With t(8;21)(q22;q22); Adult Grade III Lymphomatoid Granulomatosis; Adult Nasal Type Extranodal NK/T-cell Lymphoma; Anaplastic Large Cell Lymphoma; Angioimmunoblastic T-cell Lymphoma; Aplastic Anemia; Burkitt Lymphoma; Childhood Acute Lymphoblastic Leukemia in Remission; Childhood Acute Myeloid Leukemia in Remission; Childhood Chronic Myelogenous Leukemia; Childhood Diffuse Large Cell Lymphoma; Childhood Grade III Lymphomatoid Granulomatosis; Childhood Immunoblastic Large Cell Lymphoma; Childhood Myelodysplastic Syndromes; Childhood Nasal Type Extranodal NK/T-cell Lymphoma; Chronic Myelomonocytic Leukemia; Chronic Phase Chronic Myelogenous Leukemia; Congenital Amegakaryocytic Thrombocytopenia; Diamond-Blackfan Anemia; Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Hepatosplenic T-cell Lymphoma; Juvenile Myelomonocytic Leukemia; Myelodysplastic/Myeloproliferative Neoplasm, Unclassifiable; Nodal Marginal Zone B-cell Lymphoma; Paroxysmal Nocturnal Hemoglobinuria; Peripheral T-cell Lymphoma; Polycythemia Vera; Post-transplant Lymphoproliferative Disorder; Previously Treated Myelodysplastic Syndromes; Primary Myelofibrosis; Recurrent Adult Acute Lymphoblastic Leukemia; Recurrent Adult Acute Myeloid Leukemia; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Grade III Lymphomatoid Granulomatosis; Recurrent Adult Hodgkin Lymphoma; Recurrent Adult Immunoblastic Large Cell

  1. Cardiopatía valvular en un paciente con granulomatosis de Wegener Valvular cardiopathy in a patient with Wegener's granulomatosis

    Directory of Open Access Journals (Sweden)

    Luis E Silva

    2012-01-01

    Full Text Available La granulomatosis de Wegener es una vasculitis necrotizante autoinmune cuya prevalencia reportada es de 3/100.000 habitantes. Tiene compromiso multisistémico, principalmente el tracto respiratorio superior e inferior, y el sistema nervioso central y renal. La frecuencia del compromiso cardiaco varía según las series estudiadas, pero oscila alrededor de 6%; sin embargo, las manifestaciones clínicas son poco frecuentes. El compromiso valvular se reporta como insuficiencia aórtica o mitral, secundaria a infiltración de las valvas o dilatación de la raíz aórtica. El tratamiento se basa en el control de la enfermedad, el manejo de la falla cardiaca y la intervención quirúrgica de la válvula comprometida según la indicación.Wegener's granulomatosis is an autoimmune necrotizing vasculitis with a reported prevalence of 3/100.000 inhabitants. It is a multisystemic disease, involving mainly the upper and lower respiratory tract, the central nervous system and the kidneys. The frequency of cardiac involvement varies depending on the series studied, but oscillates around 6%; however, the clinical manifestations are rare. The valvular involvement is reported as aortic or mitral insufficiency secondary to infiltration of the leaflets, or as aortic root dilatation. Treatment is based on the control of the disease, the management of heart failure and surgical intervention of the involved valve, as directed.

  2. Prevalence of hearing impairment in patients with rheumatoid arthritis, granulomatosis with polyangiitis (GPA, Wegener's granulomatosis), or systemic lupus erythematosus.

    Science.gov (United States)

    Rahne, Torsten; Clauß, Franziska; Plontke, Stefan K; Keyßer, Gernot

    2017-07-01

    Hearing loss in patients with autoimmune diseases, such as systemic lupus erythematosus (SLE), granulomatosis with polyangiitis (GPA, Wegener's granulomatosis), or rheumatoid arthritis (RA), is controversial. Many studies lack measurements of bone-conduction thresholds to sufficiently differentiate between sensorineural hearing loss and conductive hearing loss. In addition, many studies lack control groups or comparisons to an age-related normal hearing threshold. This study investigates hearing performance with an extended audiological battery using psychoacoustic and objective measures. A total of 22 adults with RA, 16 with GPA, 20 with SLE, and two age- and gender-matched control groups (n = 34 for GPA and RA and n = 42 for SLE) were included. Pure-tone hearing thresholds, speech perception in quiet and noise, tympanometry, and high-resolution otoacoustic emissions were assessed. GPA patients exhibited impaired pure-tone hearing compared to the control group, whereas SLE and RA patients did not. In GPA patients, a larger air-bone gap indicated conductive hearing loss. In addition, speech perception was reduced exclusively in GPA patients. A significant correlation was found between hearing loss and both the cumulative steroid dose and number of organ manifestations in GPA and SLE patients. Our data indicate that GPA and SLE patients are at moderate-to-high risk of conductive hearing loss. In contrast, RA patients are at low risk of disease-associated hearing loss.

  3. Diabetes Insipidus as a Complication of Wegener's Granulomatosis and Its Treatment with Biologic Agents

    Directory of Open Access Journals (Sweden)

    Joanna Rosalind Cunnington

    2009-01-01

    Full Text Available Wegener's granulomatosis of the pituitary gland resulting in diabetes insipidus is a rare complication of the disease. Standard treatment for Wegener's granulomatosis involves a combination of prednisolone and cylophosphamide, however biologic agents are now being used in refractory cases. We report three cases of patients with diabetes insipidus as a complication of Wegener's granulomatosis who were treated with biologic agents. All three cases showed clinical response to treatment with biologic agents including rituximab and alemtuzumab and two cases demonstrated improvement in pituitary gland abnormalities by MRI. Clinicians should be aware that diabetes insipidus can present as a complication of Wegener's granulomatosis and that biologic therapies may be effective in refractory cases.

  4. A disease activity score for ENT involvement in granulomatosis with polyangiitis (Wegener's)

    DEFF Research Database (Denmark)

    Del Pero, Marcos Martinez; Chaudhry, Afzal; Rasmussen, Niels

    2013-01-01

    Accurate assessment of disease activity in patients with otorhinolaryngological manifestations of granulomatosis with polyangiitis (Wegener's) (ENT/GPA) is necessary for treatment decisions and clinical trials. We have designed a disease activity score (ENT/GPA DAS) for this purpose....

  5. Wegener's granulomatosis patients show an adequate antibody response to influenza vaccination

    NARCIS (Netherlands)

    Holvast, A.; Stegeman, C. A.; Benne, C. A.; Huckriede, A.; Wilschut, J. C.; Palache, A. M.; Kallenberg, C. G. M.; Bij, M.

    2009-01-01

    Objectives: Wegener's granulomatosis (WG) is a systemic vasculitis characterised by relapsing and remitting disease activity. Immunosuppressive drugs are used to control disease, but increase susceptibility to infection. Therefore, influenza vaccination should be considered in WG patients. This stud

  6. Impaired health-related quality of life in patients treated for Wegener's granulomatosis

    DEFF Research Database (Denmark)

    Faurschou, Mikkel; Sigaard, Lene; Bjørner, Jakob Bue;

    2010-01-01

    To investigate whether patients with Wegener's granulomatosis (WG) experience reduced health-related quality of life (HRQOL) after accomplishment of remission, and to study the influence of WG-associated organ damage on HRQOL.......To investigate whether patients with Wegener's granulomatosis (WG) experience reduced health-related quality of life (HRQOL) after accomplishment of remission, and to study the influence of WG-associated organ damage on HRQOL....

  7. /sup 67/Ga scanning in talc-induced pulmonary granulomatosis

    Energy Technology Data Exchange (ETDEWEB)

    Brown, D.G.; Aguirre, A.; Weaver, A.

    1980-04-01

    We describe a case of pulmonary granulomatosis in a user who habitually injected methylphenidate (Ritalin) intravenously; symptomatic and objective improvement occurred with corticosteroid therapy. A scan of the lungs using raioactive /sup 67/Ga showed an increased concentration of /sup 67/Ga throughout both lungs. There was a reduction in abnormal accumulation of /sup 67/Ga, improvement in the arterial oxygen pressure and the diffusing capacity for carbon monoxide, and a reduction in the infiltrate on the chest x-ray film two months after the institution of therapy. Before treatment the patient's symptoms and arterial deoxygenation increased despite the cessation of her drug abuse, thus raising the question of a self-perpetuating inflammatory process in a case of pulmonary deposition of talc.

  8. Characteristics of hypertrophic pachymeningitis in patients with granulomatosis with polyangiitis.

    Science.gov (United States)

    Choi, Hyun Ah; Lee, Mi Ji; Chung, Chin-Sang

    2017-04-01

    Hypertrophic pachymeningitis (HP) is an important neurologic complication of granulomatosis with polyangiitis (GPA, formerly Wegener's granulomatosis). The aim of this study is to investigate the clinical features, radiological findings, and diagnostic pitfalls of GPA-related HP. A retrospective chart review was performed to screen patients diagnosed with GPA at Samsung Medical Center between 1997 and 2016. Neurologic manifestation, laboratory findings, neuroimaging data, and clinical course were evaluated in all patients. Characteristics of patients with HP were compared to those of patients without HP. Sixty-five patients with GPA were identified. Twenty-five of these patients had central nervous system involvement. HP (N = 9, 36%) was the second most common radiologic finding. Other neurologic findings included stroke (N = 7, 28%) and granulomatous disease (N = 10, 40%). Patients with HP had lower incidences of systemic manifestations (N = 2, 22.2% vs. N = 38, 67.9%, p = 0.013 in the lung and N = 1, 11.1% vs. N = 28, 50.0%, p = 0.030 in the kidney) than those without HP. Six patients with GPA-related HP were MPO-ANCA positive (66.7%) and two had PR3-ANCA (22.2%). Most of the patients with HP presented with headache (N = 8, 88.9%) at a rate that is similar to those of primary headache disorders (migraine, tension-type, and stabbing) and other secondary headache disorders (postural type and meningitis). Patients with HP rarely had neurologic deficits (N = 3, 37.5%). Different clinical or radiologic features may be observed in GPA-related HP. Early recognition and accurate diagnosis of GPA-related HP are needed in addition to neuroimaging findings.

  9. Recurrent recurrent gallstone ileus.

    Science.gov (United States)

    Hussain, Z; Ahmed, M S; Alexander, D J; Miller, G V; Chintapatla, S

    2010-07-01

    We describe the second reported case of three consecutive episodes of gallstone ileus and ask the question whether recurrent gallstone ileus justifies definitive surgery to the fistula itself or can be safely managed by repeated enterotomies.

  10. Etoposide, Filgrastim, and Plerixafor in Improving Stem Cell Mobilization in Treating Patients With Non-Hodgkin Lymphoma

    Science.gov (United States)

    2016-09-15

    Adult Acute Lymphoblastic Leukemia in Remission; Adult Grade III Lymphomatoid Granulomatosis; Adult Nasal Type Extranodal NK/T-cell Lymphoma; Anaplastic Large Cell Lymphoma; Angioimmunoblastic T-cell Lymphoma; Cutaneous B-cell Non-Hodgkin Lymphoma; Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Hepatosplenic T-cell Lymphoma; Nodal Marginal Zone B-cell Lymphoma; Noncutaneous Extranodal Lymphoma; Peripheral T-cell Lymphoma; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Grade III Lymphomatoid Granulomatosis; Recurrent Adult Immunoblastic Large Cell Lymphoma; Recurrent Adult Lymphoblastic Lymphoma; Recurrent Adult T-cell Leukemia/Lymphoma; Recurrent Cutaneous T-cell Non-Hodgkin Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Mycosis Fungoides/Sezary Syndrome; Recurrent Small Lymphocytic Lymphoma; Refractory Chronic Lymphocytic Leukemia; Refractory Hairy Cell Leukemia; Small Intestine Lymphoma; Splenic Marginal Zone Lymphoma; T-cell Large Granular Lymphocyte Leukemia; Testicular Lymphoma; Waldenström Macroglobulinemia

  11. Characterisation of a Swedish cohort with orofacial granulomatosis with or without Crohn's disease.

    Science.gov (United States)

    Gale, G; Östman, S; Rekabdar, E; Torinsson Naluai, Å; Högkil, K; Hasséus, B; Saalman, R; Jontell, M

    2015-01-01

    To compare oral manifestations in a Swedish cohort of patients with orofacial granulomatosis with or without Crohn's disease and to assess NOD2 polymorphisms in the two groups. Twenty-nine patients with orofacial granulomatosis were included. Demographics, disease history, clinical features and concurrent Crohn's disease were recorded. DNA was extracted from buccal swabs and examined for NOD2 variants Arg702Trp, Gly908Arg and Leu1007fsinsC, all previously linked to gastrointestinal Crohn's disease. Twelve of 29 patients were diagnosed with coexisting gastrointestinal Crohn's disease, and of whom 21 were males. Symptom duration was significantly longer for the orofacial granulomatosis group com-pared to the group with coexisting Crohn's disease (P orofacial granulomatosis patients also perceived their overall discomfort, aesthetic problems and social discomfort as more severe. No significant differences in the clinical presentation of oral lesions between the two groups were found. None of the patients with orofacial granulomatosis carried any of the NOD2 variations, whereas four of the 12 patients with coexisting Crohn's disease had a NOD2 variant (Arg702Trp). The two patient groups had similar phenotypic characteristics but seemed to have genotypic differences regarding NOD2. The Swedish cohort differed in their clinical characteristics from patients reported in other geographical regions. © 2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  12. Massive Alveolar Hemorrhage During Wegener Granulomatosis: a Case Report

    Directory of Open Access Journals (Sweden)

    Gökhan Perincek

    2011-11-01

    Full Text Available This is a presentation of Wegener Granulomatosis (WG disease. Even though the lungs are rarely affected. massive alveolar hemorrhage is seen which leads to mortality. The patient was a 28 year old man. His illness was diagnosed as WG and glomerulonephritis a year previously and he was treated by administration of methylprednisolone orally. He had been treated irregularly. He applied to the emergency service with hemoptysis and asthma complaints two days earlier. After the results of his examination Hb: 3.6 gr/dl, Htc:10.3%, Üre:131 mg /dl, kreatini: 7.7 mg/dl, pH: 7.41, pO2: 55 mmHg, pCO2:33 mmHg, and being diagnosed as alveolar consolidation on lung X-ray, he was taken to the intensive care unit with a diagnosis of a massive alveolar hemorrhagei. He was intubated and attached to mechanical ventilation. He was treated with parenteral 1 mg/kg/day methylprednisolone and, siklofosfamid 2 mg/kg/day. He was extubated on the 21st day. He was taken to the chest service department on 24th day. He is still being treated.

  13. Renal cancer and Wegener's granulomatosis: a case report

    Directory of Open Access Journals (Sweden)

    Bumbasirevic Uros

    2011-12-01

    Full Text Available Abstract Wegener's granulomatosis (WG is a systemic disorder characterized by necrotizing vasculitis involving the respiratory tract, and in most cases, the kidneys. The most common manifestation of WG in the kidneys is segmental necrotizing glomerulonephritis. The presence of a renal mass as a manifestation of WG is rare. We report a patient with WG in whom a CT scan revealed an infiltrating mass in the lower portion of the left kidney. After surgical exploration, we performed an open radical nephrectomy. Histopathology showed clear cell type renal cell carcinoma (RCC. RCC associated with WG has been reported in only a few cases, and in most of them, the diseases started simultaneously, suggesting common pathogenetic pathways. Long-term immunosuppressive treatment is a known risk factor in the development of malignancies, so occurrence of RCC in WG has been proposed as a side effect of cyclophosphamide treatment. Furthermore, it is important to make a differential diagnosis between RCC and pseudotumors in WG as they cannot be distinguished solely on basis of imaging findings. Due to the higher risk of urologic malignancies, more frequent checkups and screening of WG patients should be considered.

  14. Hemorrhagic acneiform lesions in a teenager as the initial presentation of granulomatosis with polyangiitis.

    Science.gov (United States)

    Dosal, Jacquelyn; Good, Erica; Alshaiji, Jasem; De Solo, Santiago; Ricotti, Carlos; Alvarez-Connelly, Elizabeth

    2014-01-01

    A 19-year-old girl presented with hemorrhagic acneiform lesions on the face for several months that was unresponsive to conventional acne treatment. A biopsy revealed a noninfectious suppurative granulomatous dermatitis with hemorrhage, possibly representing a ruptured folliculitis. A second biopsy revealed chronic granulomatous dermal inflammation and hemorrhage with foreign body giant cells non-infectious by stains. No vasculitis was noted in either biopsy. Later in her course she developed a severe sinusitis and eventually presented with severe fevers, rapid weight loss, sinusitis, and cough. Further workup produced the diagnosis of granulomatosis with polyangiitis (Wegener's granulomatosis). She rapidly improved with intravenous steroids and rituximab. To date, acneiform lesions have only been reported in young adult patients and may represent a clinical manifestation of granulomatosis with polyangiitis unique to this age group, as illustrated in our patient.

  15. Wegener’s granulomatosis mimicking inflammatory bowel disease and presenting with chronic enteritis

    Directory of Open Access Journals (Sweden)

    Shahedi K

    2013-10-01

    Full Text Available Kamyar Shahedi,1,2 Ramy Magdy Hanna,1,2 Oleg Melamed,1,2 James Wilson2,31Department of Medicine Olive-View UCLA Medical Center, Sylmar, CA, 2David Geffen School of Medicine at UCLA, Los Angeles, CA, 3UCLA Medical Center-UCLA Stone Center, Los Angeles, CA, USAAbstract: Wegener’s granulomatosis, also known as anti-neutrophil cytoplasmic antibody (ANCA-associated vasculitis, is a small vessel vasculitis with primarily pulmonary, renal, and sinus disease manifestations. The prevalence of Wegener’s granulomatosis is three cases per 100,000 patients. Cardiovascular, neurologic, cutaneous, and joint manifestations have been reported in many case reports and case series. Gastrointestinal manifestations are less noted in Wegener’s granulomatosis, although they have been previously reported in the form of intestinal perforation and intestinal ischemia. Additionally, there are characteristic findings of vasculitis that are noted with active Wegener’s granulomatosis of the small bowel. We report a case of an elderly patient who presented with weight loss, diarrhea, and hematochezia. His symptoms were chronic and had lasted for more than 1 year before diagnosis. Inflammatory bowel disease or chronic enteritis due to Salmonella arizonae because of reptile exposure originally were suspected as etiologies of his presentation. The findings of proteinuria, renal failure, and pauci-immune glomerulonephritis on renal biopsy, in conjunction with an elevated c-ANCA titer, confirmed the diagnosis of Wegener’s granulomatosis with associated intestinal vasculitis. This case demonstrates an atypical presentation of chronic duodenitis and jejunitis secondary to Wegener’s granulomatosis, which mimicked inflammatory bowel disease.Keywords: ANCA-associated vasculitis, Wegener’s syndrome, pauci-immune glomerulonephritis, Salmonella arizonae, inflammatory bowel disease

  16. Refractory Granulomatosis with Polyangiitis Presenting as Facial Paralysis and Bilateral Sudden Deafness.

    Science.gov (United States)

    Kim, Sang Hoon; Jung, A Ra; Kim, Su Il; Yeo, Seung Geun

    2016-04-01

    Granulomatosis with polyangiitisis [(GPA) or Wegener granulomatosis] is a multi-system disease characterized by granuloma formation and necrotizing vasculitis. GPA classically shows involvement of the respiratory tracts and the renal system. However, locoregional disease is common and may include otologic manifestations. Although otologic involvement can occur during the course of GPA, no report has described facial palsy with sudden sensorineural total deafness with vertigo as the presenting feature of GPA. This case describes a patient with multiorgan involving resistant form of GPA initially presenting with bilateral profound sudden sensorineural hearing loss and left facial paralysis with vertigo. The condition responded well to treatment with rituximab.

  17. Coexistence of granulomatosis with polyangiitis (GPA) and Crohn's disease or multiorgan manifestation of the same disease?

    Science.gov (United States)

    Jóźwiak, Lucyna; Ławnicka, Izabela; Książek, Andrzej

    2016-01-01

    Granulomatosis with polyangiitis (GPA) is a systemic necrotizing vasculitis of unknown aetiology, often related to the antineutrophil cytoplasmic antibody (ANCA). GPA was previously named Wegener's granulomatosis (WG). The disease frequently has multisystemic presentation, targeting mainly the respiratory tract and kidneys, but gastrointestinal involvement is uncommon. Crohn's disease (CD) is an inflammatory bowel disease (IBD) with many extraintestinal manifestations. Clinically, symptoms of WG and CD can mimic each other. In this paper a case of GPA manifested initially by severe multiorgan damage including colitis, regarded to be coexistent CD, is presented. The case illustrates the difficulties in establishing the diagnosis when symptoms of the diseases mimic each other.

  18. Kaposi's sarcoma in an elderly man with Wegener's granulomatosis treated with cyclophosphamide and corticosteroids.

    Science.gov (United States)

    Erban, S B; Sokas, R K

    1988-05-01

    The association of Kaposi's sarcoma with malignant lymphoreticular diseases and immunosuppressive therapy is well documented. This report describes an elderly man who presented with fulminant Wegener's granulomatosis that responded to treatment with cyclophosphamide and corticosteroids. Rapidly progressing cutaneous Kaposi's sarcoma developed ten weeks after the start of immunosuppressive therapy yet regressed on discontinuation of the corticosteroid therapy, despite continuation of cyclophosphamide therapy. To our knowledge, this is the first reported case of Kaposi's sarcoma occurring in association with Wegener's granulomatosis. The literature on Kaposi's sarcoma in immunosuppressed patients is reviewed.

  19. Long-term outcome of lung transplantation in previous intravenous drug users with talc lung granulomatosis.

    Science.gov (United States)

    Weinkauf, J G; Puttagunta, L; Nador, R; Jackson, K; LaBranche, K; Kapasi, A; Mullen, J; Modry, D L; Stewart, K C; Thakrar, M; Doucette, K; Lien, D C

    2013-01-01

    Talc lung granulomatosis results from the intravenous use of medication intended for oral use. Talc (magnesium silicate) acts as filler in some oral medications; when injected intravenously, it deposits in the lungs leading to airflow obstruction and impaired gas exchange. Allocation of donor lungs to previous intravenous drug users is controversial. After a careful selection process, 19 patients with talc lung granulomatosis have received lung allografts in our program. Long-term survival for these patients is excellent and our results suggest the previous use of intravenous drugs should not necessarily preclude lung transplantation.

  20. Refractory Granulomatosis with Polyangiitis Presenting as Facial Paralysis and Bilateral Sudden Deafness

    Science.gov (United States)

    Kim, Sang Hoon; Jung, A Ra; Kim, Su Il

    2016-01-01

    Granulomatosis with polyangiitisis [(GPA) or Wegener granulomatosis] is a multi-system disease characterized by granuloma formation and necrotizing vasculitis. GPA classically shows involvement of the respiratory tracts and the renal system. However, locoregional disease is common and may include otologic manifestations. Although otologic involvement can occur during the course of GPA, no report has described facial palsy with sudden sensorineural total deafness with vertigo as the presenting feature of GPA. This case describes a patient with multiorgan involving resistant form of GPA initially presenting with bilateral profound sudden sensorineural hearing loss and left facial paralysis with vertigo. The condition responded well to treatment with rituximab. PMID:27144236

  1. Flavopiridol in Treating Patients With Relapsed or Refractory Lymphoma or Multiple Myeloma

    Science.gov (United States)

    2016-06-27

    Adult Lymphocyte Depletion Hodgkin Lymphoma; Adult Lymphocyte Predominant Hodgkin Lymphoma; Adult Mixed Cellularity Hodgkin Lymphoma; Adult Nodular Sclerosis Hodgkin Lymphoma; Anaplastic Large Cell Lymphoma; Angioimmunoblastic T-cell Lymphoma; Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Nodal Marginal Zone B-cell Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Grade III Lymphomatoid Granulomatosis; Recurrent Adult Hodgkin Lymphoma; Recurrent Adult T-cell Leukemia/Lymphoma; Recurrent Cutaneous T-cell Non-Hodgkin Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Mycosis Fungoides/Sezary Syndrome; Recurrent Small Lymphocytic Lymphoma; Refractory Multiple Myeloma; Splenic Marginal Zone Lymphoma; Stage I Multiple Myeloma; Stage II Multiple Myeloma; Stage III Multiple Myeloma; Waldenström Macroglobulinemia

  2. Wegener’s granulomatosis and environmental factors in Western Montana

    Directory of Open Access Journals (Sweden)

    Andrew Samuel Zeft

    2010-12-01

    Full Text Available The objective of our study was to determine whether exposure to silica or other environmental factors is associated with developing Wegener’s granulomatosis (WG, in a geographically isolated region of Western Montana. We sought to identify and interview all cases of WG diagnosed during 1993-2006 among residents of a ten-county region of Western Montana, as well as a group of demographically similar controls (n=39 without autoimmune disease. In the interview, we ascertained occupational silica and other exposures (metals, solvents, pesticides, tobacco. We enumerated 32 cases of WG, of whom 27 were included in the case-control study. Overall, a history of silica exposure was not associated with WG (OR = 0.68, 95% CI: 0.13-3.27, although there was a suggestion of increase in risk among persons with relatively recent (OR=2.67, 95% CI: 0.54-17.2, heavy (OR=1.82, 95% CI: 0.09-112.9, and prolonged (OR=1.53, 95% CI: 0.16-20.0 exposures. A history of having worked in the mining industry was associated with WG (six cases including three with no silica exposure, zero controls, lower 95% CI: 1.53. Risk was not associated with occupational or aerial pesticide exposure, but with residential rodenticide use (OR=12.15, 95% CI: 1.54-552. Occupational exposure to metals or solvents was not associated with WG, nor was a history of cigarette smoking. Results of earlier studies of WG support the hypothesis that silica exposure adversely influences the risk of developing WG. Our small study of WG failed to identify an association with silica overall, but the results are compat­ible with an increased risk in persons with relatively heavy, prolonged, and/or recent exposure.

  3. Detailed Follow-up Study of Pediatric Orofacial Granulomatosis Patients.

    Science.gov (United States)

    Haaramo, Anu; Alapulli, Heikki; Aine, Liisa; Saarnisto, Ulla; Tuokkola, Jetta; Ruuska, Tarja; Sipponen, Taina; Pitkäranta, Anne; Kolho, Kaija-Leena

    2017-10-01

    Orofacial granulomatosis (OFG) is a chronic inflammatory condition affecting the orofacial area. Its connection to Crohn disease (CD) is debated. Our aim was to describe a cohort of pediatric patients with OFG in detail, study the long-term behavior of OFG, and evaluate factors predicting CD in patients with OFG. We invited patients diagnosed with OFG at 2 university hospitals, Finland for a follow-up appointment. Patients (n = 29) were examined by a dentist and an otorhinolaryngologist using a structural schema. Orofacial findings were also recorded using digital photographing. Patients filled in questionnaires about general health and special diets. Patients' nutrition was evaluated from food records. The findings were compared between patients with OFG only and OFG with CD. Patients with CD had more findings in the orofacial area (total score for orofacial findings median 11) compared to patients with OFG only (total score median 7.5). There was no statistically significant difference in the type of lesions between these groups, except the upper lip was more often affected in patients with CD (n = 11) than in patients with OFG only (n = 0). Most of the patients had normal otorhinolaryngological findings. All patients with elevated anti-Saccharomyces cerevisiae antibody A levels had CD (n = 6) and they presented with more orofacial findings (total score) than patients with normal levels of anti-S cerevisiae antibody A (P = 0.0311). Long-term follow-up of pediatric-onset patients with OFG shows good prognosis. Patients with OFG do not seem to have otorhinolaryngological comorbidity. Anti-S cerevisiae antibody A may serve as a factor to indicate the possible presence of underlying CD in patients with OFG, but further studies are requested.

  4. Recurrent varicocele

    Directory of Open Access Journals (Sweden)

    Katherine Rotker

    2016-01-01

    Full Text Available Varicocele recurrence is one of the most common complications associated with varicocele repair. A systematic review was performed to evaluate varicocele recurrence rates, anatomic causes of recurrence, and methods of management of recurrent varicoceles. The PubMed database was evaluated using keywords "recurrent" and "varicocele" as well as MESH criteria "recurrent" and "varicocele." Articles were not included that were not in English, represented single case reports, focused solely on subclinical varicocele, or focused solely on a pediatric population (age <18. Rates of recurrence vary with the technique of varicocele repair from 0% to 35%. Anatomy of recurrence can be defined by venography. Management of varicocele recurrence can be surgical or via embolization.

  5. Persistent expansion of CD4(+) effector memory T cells in Wegener's granulomatosis

    NARCIS (Netherlands)

    Abdulahad, W. H.; van der Geld, Y. M.; Stegeman, C. A.; Kallenberg, C. G. M.

    2006-01-01

    In order to test the hypothesis that Wegener's granulomatosis (WG) is associated with an ongoing immune effector response, even in remission, we examined the distribution of peripheral naive and memory T-lymphocytes in this disease, and analyzed the function-related phenotypes of the memory T-cell p

  6. Incidence of granulomatosis with polyangiitis (Wegener`s) in Greenland and the Faroe Islands

    DEFF Research Database (Denmark)

    Faurschou, M; Helleberg, M; Obel, N

    2013-01-01

    Previous studies suggest that the incidence of granulomatosis with polyangiitis (Wegener's; GPA) increases along a south-north gradient in the Northern Hemisphere with an incidence of 8.0/million/year reported for the population of Northern Norway. In the present study, we assessed the incidence ...

  7. Plasma levels of soluble endothelial cell protein C receptor in patients with Wegener's granulomatosis

    NARCIS (Netherlands)

    Boomsma, MM; Stearns-Kurosawa, DJ; Stegeman, CA; Raschi, E; Meroni, PL; Kurosawa, S; Tervaert, JWC

    Elevated soluble thrombomodulin (sTM) levels are an accepted marker of endothelial damage. The physiological significance of plasma endothelial protein C receptor (sEPCR) levels is not known. To assess the relevance of this plasma protein in Wegener's granulomatosis (WG), sEPCR levels were measured

  8. Wegener´s granulomatosis in a young patient preceded by localized cutaneous manifestations

    Directory of Open Access Journals (Sweden)

    Jesper Smit

    2011-10-01

    Full Text Available Wegener´s granulomatosis (WG is a rare, systemic vasculitis involving multiple organs. The clinical presentation is highly diverse, and there is considerable risk of mortality if diagnosis and treatment are delayed. We present a case illustrating that patients with WG may initially present with localized cutaneous symptoms and signs.

  9. Cranial nerve palsy in Wegener's granulomatosis--lessons from clinical cases

    DEFF Research Database (Denmark)

    Nowack, Rainer; Wachtler, Paul; Kunz, Jürgen;

    2009-01-01

    The problem of diagnosing vasculitic neuropathy is discussed based on case reports of two patients with Wegener's granulomatosis. One patient developed de novo 6(th) nerve palsy as an isolated relapse manifestation and the second patient a sequence of multiple cranial nerve palsies. Brain imaging...

  10. Incidence of granulomatosis with polyangiitis (Wegener`s) in Greenland and the Faroe Islands

    DEFF Research Database (Denmark)

    Faurschou, M; Helleberg, M; Obel, N

    2013-01-01

    Previous studies suggest that the incidence of granulomatosis with polyangiitis (Wegener's; GPA) increases along a south-north gradient in the Northern Hemisphere with an incidence of 8.0/million/year reported for the population of Northern Norway. In the present study, we assessed the incidence ...

  11. Dilatation tracheoscopy for laryngeal and tracheal stenosis in patients with Wegener's granulomatosis

    NARCIS (Netherlands)

    Schokkenbroek, Ada A.; Franssen, Casper F. M.; Dikkers, Frederik G.

    2008-01-01

    Wegener's granulomatosis (WG) frequently involves the subglottis and trachea and may compromise the upper airway. The objective of this study is to evaluate retrospectively the effect of treatment of subglottic stenosis (SGS) and tracheal stenosis (TS) by dilatation tracheoscopy (DT) in patients wit

  12. Enhanced endothelium-dependent microvascular responses in patients with Wegener's granulomatosis

    NARCIS (Netherlands)

    Nienhuis, Hans L.A.; De Leeuw, Karina; Smit, Andries J.; Bijzet, Johan; Stegeman, Coen A.; Kallenberg, Cees G.M.; Bijl, Marc

    2007-01-01

    Objective. To assess endothelial cell (EC) function of the cutaneous microcirculation in patients with Wegener's granulomatosis (WG) and to relate EC function to EC activation and presence of atherosclerosis. Methods. We studied 28 WG patients with inactive disease and 28 age and sex matched control

  13. Wegener's granulomatosis with unusual cavernous sinus and sella turcica extension

    Energy Technology Data Exchange (ETDEWEB)

    Hermann, M.; Bobek-Billewicz, B. [Dept. of Radiology, Medical University of Gdansk (Poland); Bullo, B.; Hermann, A.; Rutkowski, B. [Dept. of Nephrology, Medical University of Gdansk (Poland)

    1999-07-01

    Intracerebral extension of Wegener's granulomatosis (WG) is rare. We present a patient with oculomotor and trochlear nerve palsy with histologically proved WG. An MR examination revealed granulomatous tissue in nasal cavity, paranasal sinuses with meningeal infiltration, and uncommon penetration into cavernous sinus and sella turcica. The MR images before and during pharmacological therapy are presented. (orig.)

  14. Detection of immune deposits in skin lesions of patients with Wegener's granulomatosis

    NARCIS (Netherlands)

    Brons, RH; de Jong, MCJM; de Boer, NK; Stegeman, CA; Kallenberg, CGM; Tervaert, JWC

    2001-01-01

    Background-Wegener's granulomatosis (WG) is considered a pauci-immune systemic vasculitis based on the absence of immune deposits in renal biopsies of patients with active disease. In animal models of antineutrophil cytoplasmic antibody (ANCA) associated glomerulonephritis, immune deposits along the

  15. Alemtuzumab, Fludarabine Phosphate, and Total-Body Irradiation Followed by Cyclosporine and Mycophenolate Mofetil in Treating Patients Who Are Undergoing Donor Stem Cell Transplant for Hematologic Cancer

    Science.gov (United States)

    2016-06-13

    Acute Undifferentiated Leukemia; Adult Acute Lymphoblastic Leukemia in Remission; Adult Acute Myeloid Leukemia in Remission; Adult Nasal Type Extranodal NK/T-cell Lymphoma; Anaplastic Large Cell Lymphoma; Angioimmunoblastic T-cell Lymphoma; Atypical Chronic Myeloid Leukemia, BCR-ABL1 Negative; Childhood Acute Lymphoblastic Leukemia in Remission; Childhood Acute Myeloid Leukemia in Remission; Childhood Burkitt Lymphoma; Childhood Chronic Myelogenous Leukemia; Childhood Diffuse Large Cell Lymphoma; Childhood Immunoblastic Large Cell Lymphoma; Childhood Myelodysplastic Syndromes; Childhood Nasal Type Extranodal NK/T-cell Lymphoma; Chronic Myelomonocytic Leukemia; Chronic Phase Chronic Myelogenous Leukemia; Cutaneous B-cell Non-Hodgkin Lymphoma; Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Hepatosplenic T-cell Lymphoma; Intraocular Lymphoma; Juvenile Myelomonocytic Leukemia; Mast Cell Leukemia; Myelodysplastic/Myeloproliferative Neoplasm, Unclassifiable; Nodal Marginal Zone B-cell Lymphoma; Noncutaneous Extranodal Lymphoma; Peripheral T-cell Lymphoma; Previously Treated Myelodysplastic Syndromes; Recurrent Adult Acute Lymphoblastic Leukemia; Recurrent Adult Acute Myeloid Leukemia; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Grade III Lymphomatoid Granulomatosis; Recurrent Adult Hodgkin Lymphoma; Recurrent Adult Immunoblastic Large Cell Lymphoma; Recurrent Adult Lymphoblastic Lymphoma; Recurrent Adult T-cell Leukemia/Lymphoma; Recurrent Childhood Acute Lymphoblastic Leukemia; Recurrent Childhood Acute Myeloid Leukemia; Recurrent Childhood Anaplastic Large Cell Lymphoma; Recurrent Childhood Grade III Lymphomatoid Granulomatosis; Recurrent Childhood Large Cell Lymphoma; Recurrent Childhood Lymphoblastic Lymphoma; Recurrent Childhood Small Noncleaved Cell Lymphoma; Recurrent Cutaneous T

  16. [Pulmonar pseudotumor in granulomatosis with polyangiitis (GPA). Pulmonary cancer and/or GPA? Diagnostic implications of pulmonary nodules].

    Science.gov (United States)

    Horta-Baas, Gabriel; Meza-Zempoaltecatl, Esteban; Pérez-Cristóbal, Mario; Barile-Fabris, Leonor Adriana

    2016-01-01

    Granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis, is a systemic necrotizing vasculitis, which affects small and medium sized blood vessels and is often associated with cytoplasmic anti-neutrophil cytoplasmic antibodies (ANCA). Inflammatory pseudotumor is a rare condition characterized by the appearance of a mass lesion that mimics a malignant tumor both clinically and on imaging studies, but that is thought to have an inflammatory/reactive pathogenesis. We report a patient with a GPA which was originally diagnosed as malignancy.

  17. Orofacial granulomatosis in children can be the initial manifestation of systemic disease: a presentation of two cases

    DEFF Research Database (Denmark)

    Simonsen, Anne Birgitte; Deleuran, Mette

    2014-01-01

    Orofacial granulomatosis is a chronic granulomatous condition characterized by relapsing and remitting lip swelling and oral involvement that may include deep ulcers, tags and cobblestone formation. It occurs as an independent entity but also in conjunction with systemic diseases such as tubercul......Orofacial granulomatosis is a chronic granulomatous condition characterized by relapsing and remitting lip swelling and oral involvement that may include deep ulcers, tags and cobblestone formation. It occurs as an independent entity but also in conjunction with systemic diseases...

  18. Voriconazole-induced periostitis causing arthralgias mimicking a flare of granulomatosis with polyangiitis.

    Science.gov (United States)

    Gladue, Heather S; Fox, David A

    2013-12-01

    We describe a case of voriconazole-induced periostitis that occurred in a 68-year-old woman with granulomatosis with polyangiitis. Our patient presented with months of severe hip pain limiting her daily activities, which was initially felt to be a flare of her granulomatosis with polyangiitis. However, upon further review, she had an elevated alkaline phosphatase and periostitis on her hip radiograph; voriconazole was held, and within 2 days she had marked improvement in her pain. Although this clinical syndrome is well documented in transplant patients, it is a rare complication in patients with autoimmune disorders. However, it is important because it may cause severe arthralgias that can mimic a flare of rheumatic diseases.

  19. Chronic meningitis with multiple cranial neuropathies: A rare initial presentation of Wegener′s granulomatosis

    Directory of Open Access Journals (Sweden)

    Vikas Gupta

    2013-01-01

    Full Text Available Wegener′s granulomatosis (WG is a systemic necrotizing vasculitis that affects the small blood vessels. It mainly affects the upper and lower respiratory tract and kidneys. Central nervous system (CNS involvement is rare, and has been reported only in about 8% of cases during the course of illness. Initial presentation with neurologic affection, particularly chronic hypertrophic meningitis is very unusual. We report the case of a 34 year old male who presented with chronic hypertrophic meningitis and multiple cranial nerve involvement as the initial manifestation, without respiratory and renal symptoms. This case highlights the difficulties in diagnosing a rare disease with rarer presentation, and at the same time illustrates that Wegener′s granulomatosis should be considered in the differential diagnosis of chronic meningitis.

  20. Polymicrobial Infections in the Early Period of Kidney Transplantation in a Case with Wegener's Granulomatosis

    Directory of Open Access Journals (Sweden)

    Funda COŞKUN

    2011-01-01

    Full Text Available Wegener's granulomatosis (WG is a disorder that causes necrotizing granulomatosis vasculitis particularly of the upper respiratory tract, lung and kidney. A 43-year-old male who had been treated with hemodialysis because of renal insufficiency due to WG underwent live donor renal transplantation. Pulmonary infiltrates were detected on the postoperative 4th day and antibiotic therapy was started with a diagnosis of sepsis and pulmonary infection. Dialysis treatment was also started due to the degradation of renal function for the patient who was intubated during follow-up. Non-invasive mechanical ventilation (BiPAP treatment was started after extubation. The graft and respiratory function improved during clinical follow-up. Resistant hospital infections, causing respiratory failure and systemic complications, were facilitated by a long history of hospitalization before transplantation, the presence of WG and immunosupression and were successfully treated with a multidisciplinary approach.a

  1. GRANULOMATOSIS WITH POLYANGIITIS, WHICH IS PATHOGENETICALLY ASSOCIATED WITH ANTINEUTROPHIL CYTOPLASMIC ANTIBODIES: CLINICAL FEATURES

    Directory of Open Access Journals (Sweden)

    Tatiana Valentinovna Beketova

    2012-01-01

    Full Text Available The author studied in detail the specific features of the clinical manifestations reflecting a granulomatous inflammatory response and necrotizing polyangiitis, by using her experience in following up 70 patients with granulomatosis and polyangiitis (Wegener's, and analyzed their early symptoms, premorbid background, and possible predictors. Granulomatous inflammation and its related clinical manifestations (primarily orbital pseudotumor and subfold granuloma of the larynx was demonstrated to tend over time to occupy a dominant place in the clinical picture of the disease while the proportion of the symptoms apparently related to necrotizing vasculitis was generally decreased. Interpretation of mechanisms for the development of granulomatosis with polyangiitis (Wegener's may be of value for the further elaboration of an optimal treatment strategy.

  2. Granulomatosis with Polyangiitis with Bilateral Facial Palsy and Severe Mixed Hearing Loss

    Directory of Open Access Journals (Sweden)

    Agnieszka Wawrzecka

    2016-01-01

    Full Text Available Granulomatosis with polyangiitis is autoimmune and rare disease. It affects many organs, but the most often affected organs are the nose, lungs, and kidneys. It is part of vasculitis and causes an autoimmune attack by an abnormal type of circulating antibody termed ANCAs against small blood vessels. Disease concerns both men and women with a peak age of presentation in the sixth and seven decades. Typically upper and lower respiratory tract and kidneys are involved. Otitis externa, otitis media, or mastoiditis rarely occurs in granulomatosis with polyangiitis. Deafness is the most dangerous aural complication. Histological examination of biopsy is often not specific. A case of GPA with bilateral otitis media, bilateral deafness, and bilateral facial palsy with fatal course is presented.

  3. Limited Wegener's granulomatosis presenting as lung nodules in a patient with rheumatoid arthritis: a case report.

    Science.gov (United States)

    Pai, Sushma; Panda, Mukta

    2008-12-23

    Rheumatoid arthritis has varied pleuroparenchymal manifestations. Wegener's granulomatosis can develop in an established case of rheumatoid arthritis and this association although previously reported is very rare. A 60-year-old lady had been diagnosed with rheumatoid arthritis on the basis of her clinical symptoms and serological tests which were positive RA factor and anti-CCP antibodies. Her rheumatoid arthritis activity had been mild and well controlled with hydroxychloroquine and low dose prednisone. She presented with a productive cough and right-sided pleuritic chest pain. CT scan of the chest showed three lung nodules with increased uptake on PET CT scan, raising concerns for an inflammatory or malignant process. The differential diagnosis included rheumatoid nodules, infections or malignancy. A CT-guided needle biopsy of the largest nodule was undertaken which showed vasculitis typical of Wegener's granulomatosis. Stains and cultures of the biopsy specimen were negative for bacteria, fungi and acid fast bacilli. A panel of serological tests for vasculitis were checked and showed elevated titers of cANCA and anti-proteinase 3 antibodies. Urine analysis and CT scan of paranasal sinuses was normal. Since the upper respiratory tract and the kidneys were spared a diagnosis of limited Wegener's granulomatosis affecting only the lungs was made. Due to the toxicity of cyclophosphamide, her relatively mild disease sparing the kidneys and the underlying rheumatoid arthritis, weekly methotrexate was started and low dose prednisone was continued. She had marked symptomatic improvement and complete resolution of the nodules was documented on subsequent imaging. Wegener's granulomatosis developing in a patient with rheumatoid arthritis is very rare but should be considered as it warrants a different and possibly more aggressive treatment approach.

  4. Two cases of extrapulmonary onset granulomatosis with polyangiitis which caused diffuse alveolar haemorrhage

    Directory of Open Access Journals (Sweden)

    Halide Kaya

    2014-01-01

    Full Text Available Granulomatosis with polyangiitis (GPA is a rare form of vasculitis. Multidisciplinary therapeutic approach and early diagnosis assume vital importance in management of patients with diffuse alveolar haemorrhage caused by GPA, which is a rare complication. The purpose of this study was to present the diagnostic and therapeutic challenges experienced by clinicians in management of two severe cases of GPA with insidious extrapulmonary manifestations which rapidly progressed into acute kidney injury, alveolar haemorrhage and acute respiratory failure.

  5. Incidence of granulomatosis with polyangiitis (Wegener's) in Greenland and the Faroe Islands

    DEFF Research Database (Denmark)

    Faurschou, M; Helleberg, M; Obel, N

    2013-01-01

    Previous studies suggest that the incidence of granulomatosis with polyangiitis (Wegener's; GPA) increases along a south-north gradient in the Northern Hemisphere with an incidence of 8.0/million/year reported for the population of Northern Norway. In the present study, we assessed the incidence...... of GPA in the predominantly Inuit population of Greenland and in the Caucasian population of the Faroe Islands....

  6. Testicular Involvement in Wegener Granulomatosis: Case Report and Review of the literature

    Directory of Open Access Journals (Sweden)

    Mehmet Nuri TURAN

    2012-05-01

    Full Text Available Wegener granulomatosis (WG is a type of necrotising vasculitis that affects mainly the respiratory tract, paranasal sinuses and the kidneys. Urogenital involvement is rare; mainly affecting the prostate gland. Testicular involvement in WG is exceptional. We report a 50-year-old male patient with hematuria, proteinuria and testicular pain and renal failure diagnosed as WG by testicular and kidney biopsy. We also provide a review of the literature.

  7. Wegener`s granulomatosis and mucoromycosis: A case study and review of literature

    Directory of Open Access Journals (Sweden)

    Sayyed Mojtaba Abtahi

    2012-01-01

    Full Text Available Mucormycosis is a fatal invasive infection which mostly involves diabetic or immunosuppressed patients. Early diagnosis, improving immunosuppression, systemic antifungal therapy, and surgical debridement are necessary for successful treatment. In this case study, we represent a known case of Wegener′s granulomatosis (WG, with concomitant sinusal mucormycosis mimicking vasculitic disease relapse, which was successfully treated with surgical debridement, amphotericine, and intravenous immunoglobuline.

  8. Pachymeningitis in Granulomatosis with Polyangiitis: A Case Report and a Review of the Literature

    Directory of Open Access Journals (Sweden)

    Grigorios T. Sakellariou

    2013-01-01

    Full Text Available Central nervous involvement, mainly with symptoms of cranial neuropathies, occurs in 2–8% of patients with granulomatosis with polyangiitis (GPA. Meningeal involvement, with persistent and severe headache as main manifestation and abnormal thickening and enhancement of the dural mater on postcontrast magnetic resonance imaging, is extremely rare. We present a case of pachymeningitis due to limited GPA, providing simultaneously a literature review.

  9. An atypical case of Wegener`s granulomatosis complicated by sepsis and coxitis; Ziarniniakowatosc Wegenera o nietypowej lokalizacji powiklana posocznica i ropnym zapaleniem stawu biodrowego

    Energy Technology Data Exchange (ETDEWEB)

    Makowski, A.; Faflik, J. [Centrum Zdrowia Matki Polki, Lodz (Poland)

    1993-12-31

    There is presented an atypical case Wegener`s granulomatosis of maxillo-nasal region without bony destruction. The case is unusual because of sepsis and purulent coxitis. The patient responded well to treatment with vincristine and cyclophosphamide. ANCA (anti-neutrophil cytoplasmic antibodies) assays have very good sensitivity and specificity for Wegener`s granulomatosis. (author). 17 refs, 2 figs.

  10. Recurrent vulvovaginitis.

    Science.gov (United States)

    Powell, Anna M; Nyirjesy, Paul

    2014-10-01

    Vulvovaginitis (VV) is one of the most commonly encountered problems by a gynecologist. Many women frequently self-treat with over-the-counter medications, and may present to their health-care provider after a treatment failure. Vulvovaginal candidiasis, bacterial vaginosis, and trichomoniasis may occur as discreet or recurrent episodes, and have been associated with significant treatment cost and morbidity. We present an update on diagnostic capabilities and treatment modalities that address recurrent and refractory episodes of VV.

  11. Refractory optic perineuritis due to granulomatosis with polyangiitis successfully treated with methotrexate and mycophenolate mofetil combination therapy

    Science.gov (United States)

    Kimura, Yoshitaka; Asako, Kurumi; Kikuchi, Hirotoshi; Kono, Hajime

    2017-01-01

    Optic perineuritis is an uncommon inflammatory disorder of the optic sheath that causes visual loss or eye pain. There are few case reports of optic perineuritis associated with granulomatosis with polyangiitis. Herein we report the case of a 37-year-old male with granulomatosis with polyangiitis and who presented with headache, blurred vision in the right eye, diplopia, and numbness in the right forehead. Brain magnetic resonance images (MRI) findings revealed hypertrophic pachymeningitis and refractory optic perineuritis. These were manageable only by means of weekly methotrexate and mycophenolate mofetil combination therapy but not with methotrexate, mycophenolate mofetil, intravenous cyclophosphamide, rituximab, azathioprine, or cyclosporine individually. PMID:28293459

  12. Wegener's granulomatosis: chest computed tomography findings; Granulomatose de Wegener: aspectos na tomografia computadorizada de torax

    Energy Technology Data Exchange (ETDEWEB)

    Monteiro, Evelise de Azevedo; Marchiori, Edson; Martins, Erick Malheiro Leoncio [Universidade Federal Fluminense, Niteroi, RJ (Brazil). Dept. de Radiologia]. E-mail: edmarchiori@zipmail.com.br; Souza Junior, Arthur Soares [Faculdade de Medicina de Sao Jose do Rio Preto (FAMERP), SP (Brazil). Hospital de Base; Cerqueira, Elza Maria F.P. de [Universidade Estadual de Campinas, SP (Brazil). Faculdade de Ciencias Medicas. Dept. de Radiologia; Irion, Klaus L. [Pavilhao Pereira Filho, Porto Alegre, RS (Brazil); Araujo Neto, Cesar de [Bahia Univ., Salvador, BA (Brazil). Dept. de Radiologia; Souza, Rodrigo Azeredo de [Universidade Federal, Rio de Janeiro, RJ (Brazil). Faculdade de Medicina. Dept. de Radiologia

    2003-03-01

    Wegeners granulomatosis is an inflammatory systemic necrotizing idiopathic vasculitis affecting mainly small vessels. In this paper we analyzed the computed tomography findings of seven patients with Wegeners granulomatosis. The most common findings were masses and nodules (71.4%), areas of ground glass attenuation (57.1%), consolidation (42.8%), halo sign (42.8%), cavitation (42.8%), interlobular septa thickening (14.2%), bronchial wall thickening (14.2%) and pleural effusion (14.2%). Although radiological findings are mostly nonspecific, computed tomography and particularly high-resolution computed tomography are important tools in the evaluation of disease activity when evaluated in association with bronchoscopy findings and clinical data. (author)

  13. Plasma exchange for induction and cyclosporine A for maintenance of remission in Wegener's granulomatosis--a clinical randomized controlled trial

    DEFF Research Database (Denmark)

    Szpirt, Wladimir M; Heaf, James G; Petersen, Jørgen

    2011-01-01

    The use of plasma exchange (PE) for induction treatment of anti-neutrophil cytoplasm autoantibody (ANCA)-associated vasculitis (AAV), including Wegener's granulomatosis (WG), is still controversial. The use of PE in AAV is not commonly accepted in patients with a plasma creatinine......The use of plasma exchange (PE) for induction treatment of anti-neutrophil cytoplasm autoantibody (ANCA)-associated vasculitis (AAV), including Wegener's granulomatosis (WG), is still controversial. The use of PE in AAV is not commonly accepted in patients with a plasma creatinine...

  14. Successful application of extracorporeal membrane oxygenation due to pulmonary hemorrhage secondary to granulomatosis with polyangiitis

    Directory of Open Access Journals (Sweden)

    Hohenforst-Schmidt W

    2013-07-01

    Full Text Available Wolfgang Hohenforst-Schmidt,1 Arndt Petermann,2 Aikaterini Visouli,3 Paul Zarogoulidis,4 Kaid Darwiche,5 Ioanna Kougioumtzi,6 Kosmas Tsakiridis,3 Nikolaos Machairiotis,6 Markus Ketteler,2 Konstantinos Zarogoulidis,4 Johannes Brachmann11II Medical Clinic, Coburg Clinic, University of Wuerzburg, Coburg, Germany; 2Division of Nephrology, Coburg Clinic, University of Wuerzburg, Coburg, Germany; 3Cardiothoracic Surgery Department, “Saint Luke” Private Hospital, Thessaloniki, Greece; 4Pulmonary Department, “G Papanikolaou” General Hospital, Aristotle University of Thessaloniki, Thessaloniki, Greece; 5Department of Interventional Pneumology, Ruhrlandklinik, West German Lung Center, University Hospital, University of Duisburg-Essen, Essen, Germany; 6Surgery Department (National Health System, University General Hospital of Alexandroupolis, Alexandroupolis, GreeceAbstract: Extracorporeal membrane oxygenation (ECMO is increasingly applied in adults with acute refractory respiratory failure that is deemed reversible. Bleeding is the most frequent complication during ECMO support. Severe pre-existing bleeding has been considered a contraindication to ECMO application. Nevertheless, there are cases of successful ECMO application in patients with multiple trauma and hemorrhagic shock or head trauma and intracranial hemorrhage. ECMO has proved to be life-saving in several cases of life-threatening respiratory failure associated with pulmonary hemorrhage of various causes, including granulomatosis with polyangiitis (Wegener’s disease. We successfully applied ECMO in a 65-year-old woman with acute life-threatening respiratory failure due to diffuse massive pulmonary hemorrhage secondary to granulomatosis with polyangiitis, manifested as severe pulmonary-renal syndrome. ECMO sustained life and allowed disease control, together with plasmapheresis, cyclophosphamide, corticoids, and renal replacement therapy. The patient was successfully weaned from ECMO

  15. Pituitary involvement in Wegener ' s granulomatosis: a case report and review of the literature

    Institute of Scientific and Technical Information of China (English)

    陶建瓴; 董怡

    2003-01-01

    Wegener ' s Granulomatosis (WG) is a multi-system disorder characterized by necrotizing granulomas in the upper and/or lower respiratory tract, with or without focal necrotizing glomerulonephritis and other systemic vasculitis.1 Clinical manifestations may vary with the site of involvement and the extension of the lesions. Pituitary involvement is rare, and to our knowledge no more than ten cases with this complication have been published in the literature in English. We report here an additional WG case complicated by pan-hypopituitarism.

  16. Increased morbidity from ischemic heart disease in patients with Wegener's granulomatosis

    DEFF Research Database (Denmark)

    Faurschou, Mikkel; Mellemkjaer, Lene; Sørensen, Inge Juul;

    2009-01-01

    OBJECTIVE: Experimental studies indicate that patients with Wegener's granulomatosis (WG) experience accelerated atherosclerosis. The purpose of this study was to investigate whether the occurrence of overt ischemic heart disease (IHD) is increased in WG. METHODS: A total of 293 WG patients were...... MI were found in patients who were > or =50.0 years of age at the time of diagnosis of WG, in male patients, and in patients who received high cumulative doses of cyclophosphamide. CONCLUSION: Compared with the background population, WG patients seem to experience an increased number of both early...

  17. Characteristics and Outcomes of Granulomatosis With Polyangiitis (Wegener) and Microscopic Polyangiitis Requiring Renal Replacement Therapy

    DEFF Research Database (Denmark)

    Hruskova, Zdenka; Stel, Vianda S; Jayne, David;

    2015-01-01

    for microscopic polyangiitis (prevailing in Southern Europe). Kidney transplantation was performed in 558 (22.2%) patients with vasculitis. The 10-year probability for survival on RRT after day 91 was 32.5% (95% CI, 29.9%-35.1%) in patients with vasculitis. Survival on RRT after day 91 did not differ between AAV...... manifestations, treatment, and relapses. CONCLUSIONS: Geographical differences in the incidence of RRT for kidney failure due to granulomatosis with polyangiitis and microscopic polyangiitis copied their distribution in the general population. Overall survival on RRT after day 91 for patients with AAV...

  18. Lingual infarction in Wegener's Granulomatosis: A case report and review of the literature

    Directory of Open Access Journals (Sweden)

    Brizman Eitan

    2008-08-01

    Full Text Available Abstract Wegener's granulomatosis (WG is a multi-system disease, characterised by the triad of necrotising granulomata affecting the upper and lower respiratory tracts, disseminated vasculitis and glomerulonephritis. Oral lesions are associated with up to 50% of cases, although are rare as a presenting feature. The most common oral lesions associated with WG are ulceration and strawberry gingivitis. We review the literature regarding oral manifestations of WG and present a case of lingual infarction, an extremely rare oral lesion associated with WG, in a severe, rapidly progressive and ultimately fatal form of the disease.

  19. Pulmonary sarcoid-like granulomatosis induced by aluminum dust: report of a case and literature review

    Institute of Scientific and Technical Information of China (English)

    CAI Hou-rong; CAO Min; MENG Fan-qing; WEI Jing-yi

    2007-01-01

    @@ Awide range of pulmonary pathology is attributed to aluminum dust exposure, including interstitial fibrosis,1-5 desquamative interstitial pneumonia,6 and pulmonary alveolar proteinosis.7 To our knowledge,granulomatous lung disease induced by aluminum dust is rare, only two cases were reported in literatures.8,9 We had the opportunity to make a diagnosis of pulmonary sarcoid-like granulomatosis in a patient exposed to aluminum dust. Herein, we report the clinical history,radiographic and histopathological findings in this patient,and the detailed mineralogical analyses performed on lung tissue obtained by open lung biopsy.

  20. Granulomatous renal pseudotumor in Wegener's granulomatosis: imaging findings in one case

    Energy Technology Data Exchange (ETDEWEB)

    Verswijvel, G.; Eerens, I.; Oyen, R. [Department of Radiology, University Hospitals. Catholic University of Leuven, Leuven (Belgium); Messiaen, T. [Department of Nephrology, University Hospitals. Catholic University of Leuven, Leuven (Belgium)

    2000-08-01

    Wegener's granulomatosis is a clinico-pathological entity characterized by necrotizing granulomatous angiitis involving the upper- and/or lower respiratory tract and the kidneys. Renal involvement is usually characterized by a rapidly progressive necrotizing glomerulonephritis. A case is presented of a patient who developed renal failure and presented a solitary pseudotumoral lesion in the upper pole of the left kidney. Imaging characteristics on US, CT and MR imaging are discussed. The diagnosis was confirmed with ultrasound-guided needle biopsy. (orig.)

  1. A Rare Manifestation of Crohn's Disease: Sinonasal Granulomatosis. Report of a Case and Review of Literature

    Directory of Open Access Journals (Sweden)

    Lilia Baili

    2014-10-01

    Full Text Available Crohn’s disease is a granulomatous inflammatory bowel disease. Its pathologic findings include non-contiguous chronic inflammation and non-caseating granulomas, sometimes with extra-intestinal localizations. Sinonasal manifestations of Crohn’s disease are quite rare and only a few cases have been reported up to date in the worldwide literature. They are characterized by chronic mucosal inflammation, obstruction, bleeding and occasionally septal perforation. We report a case of sinonasal granulomatosis revealing Crohn’s disease in a 22-year-old woman and go over the available literature on sinonasal involvement in Crohn’s disease.

  2. A rare case of primary orofacial granulomatosis of gingiva during pregnancy

    Directory of Open Access Journals (Sweden)

    Vaibhavi Joshipura

    2015-01-01

    Full Text Available Orofacial granulomatosis (OFG comprises a group of diseases characterized by noncaseating granulomatous inflammation affecting the soft tissues of the oral and maxillofacial region. It is important to establish the diagnosis accurately because this condition is sometimes a manifestation of many systemic conditions like Crohn's disease or sarcoidosis. The clinical outcome of OFG patients continues to be unpredictable. Current therapies remain unsatisfactory. This article reports a rare case of isolated OFG with langhans type giant cells and inflammatory infiltrate without any systemic involvement, in which the condition was a manifestation of pregnancy. The diagnostic approach to and the treatment of OFG are reviewed.

  3. A rare case of primary orofacial granulomatosis of gingiva during pregnancy.

    Science.gov (United States)

    Joshipura, Vaibhavi; Mahantesha, S; Subbaiah, Shobha Krishna; Lakkasetty, Yogesh T

    2015-01-01

    Orofacial granulomatosis (OFG) comprises a group of diseases characterized by noncaseating granulomatous inflammation affecting the soft tissues of the oral and maxillofacial region. It is important to establish the diagnosis accurately because this condition is sometimes a manifestation of many systemic conditions like Crohn's disease or sarcoidosis. The clinical outcome of OFG patients continues to be unpredictable. Current therapies remain unsatisfactory. This article reports a rare case of isolated OFG with langhans type giant cells and inflammatory infiltrate without any systemic involvement, in which the condition was a manifestation of pregnancy. The diagnostic approach to and the treatment of OFG are reviewed.

  4. Wegener′s granulomatosis presenting as spontaneous pneumothorax in young adult

    Directory of Open Access Journals (Sweden)

    Sunil Kumar

    2014-01-01

    Full Text Available Pulmonary involvement in Wegener′s granulomatosis (WG usually starts with nonspecific symptoms such as cough, dyspnea, hemoptysis, and pleuritis. Spontaneous pneumothorax as initial presentation is extremely rare. Although its real incidence is unknown, according to different classic series, it ranges between 3 and 5% of the cases. In this case, a 28-year-old male presented with complaints of epistaxis and breathlessness, which was diagnosed as WG with pneumothorax on the basis of chest X-ray and computed tomography (CT chest and pathological confirmation by high level of serum cytoplasmic antineutrophil cytoplasmic antibody (ANCA.

  5. [Systemic diseases--the significance of early diagnosis exemplified by systemic lupus erythematosus and Wegener's granulomatosis].

    Science.gov (United States)

    Türk, H

    1993-12-01

    New therapeutic modalities have shown remarkable advances in the fields of systemic lupus erythematosus and Wegener's granulomatosis. For an optimal clinical outcome therapy has to be started early and must be adapted to disease activity. Concerning these two points early diagnosis is essential. This aim can be reached by a detailed evaluation of the patient's history by focusing on early symptoms and on typical clinical constellations, taking into account that both diseases show a great variability and sometimes even an insidious course. Immunological tests, capillary microscopy, echocardiography and computed tomography can be very helpful in the diagnosis of these diseases.

  6. Features of coexisting granulomatosis with polyangiitis in a patient with pemphigus vulgaris: a case report.

    Science.gov (United States)

    Cala, Cather M; Sami, Naveed

    2015-01-01

    Pemphigus vulgaris (PV) and granulomatosis with polyangiitis (Wegener's or GPA) are two rare autoimmune disorders. Both can involve the upper airways, and diagnosis can be difficult in the absence of extra-airway symptoms. We report the case of a patient with well-controlled PV but persistent upper respiratory tract symptoms. Further evaluation revealed perforation of the nasal septum and elevated serologies consistent with GPA. The patient improved with rituximab treatments. This case demonstrates that alternative concomitant diagnosis should be considered in patients with symptoms suggestive of recalcitrant PV and/or GPA, since these patients may require more aggressive initial treatment.

  7. Recurrent fevers.

    Science.gov (United States)

    Isaacs, David; Kesson, Alison; Lester-Smith, David; Chaitow, Jeffrey

    2013-03-01

    An 11-year-old girl had four episodes of fever in a year, lasting 7-10 days and associated with headache and neck stiffness. She had a long history of recurrent urticaria, usually preceding the fevers. There was also a history of vague pains in her knees and in the small joints of her hands. Her serum C-reactive protein was moderately raised at 41 g/L (normal <8). Her rheumatologist felt the association of recurrent fevers that lasted 7 or more days with headaches, arthralgia and recurrent urticaria suggested one of the periodic fever syndromes. Genetic testing confirmed she had a gene mutation consistent with one of tumour necrosis factor receptor-associated periodic syndrome.

  8. Lenalidomide After Donor Bone Marrow Transplant in Treating Patients With High-Risk Hematologic Cancers

    Science.gov (United States)

    2016-12-26

    Adult Acute Myeloid Leukemia in Remission; Adult Acute Myeloid Leukemia With Inv(16)(p13.1q22); CBFB-MYH11; Adult Acute Myeloid Leukemia With t(16;16)(p13.1;q22); CBFB-MYH11; Adult Acute Myeloid Leukemia With t(8;21); (q22; q22.1); RUNX1-RUNX1T1; Adult Acute Myeloid Leukemia With t(9;11)(p22.3;q23.3); MLLT3-KMT2A; Adult Acute Promyelocytic Leukemia With PML-RARA; Adult Grade III Lymphomatoid Granulomatosis; Adult Nasal Type Extranodal NK/T-Cell Lymphoma; Alkylating Agent-Related Acute Myeloid Leukemia; Anaplastic Large Cell Lymphoma; Angioimmunoblastic T-Cell Lymphoma; Cutaneous B-Cell Non-Hodgkin Lymphoma; Extranodal Marginal Zone Lymphoma of Mucosa-Associated Lymphoid Tissue; Hepatosplenic T-Cell Lymphoma; Intraocular Lymphoma; Lymphomatous Involvement of Non-Cutaneous Extranodal Site; Mature T-Cell and NK-Cell Non-Hodgkin Lymphoma; Nodal Marginal Zone Lymphoma; Post-Transplant Lymphoproliferative Disorder; Prolymphocytic Leukemia; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Grade III Lymphomatoid Granulomatosis; Recurrent Adult Immunoblastic Lymphoma; Recurrent Adult Lymphoblastic Lymphoma; Recurrent Adult T-Cell Leukemia/Lymphoma; Recurrent Cutaneous T-Cell Non-Hodgkin Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Mycosis Fungoides and Sezary Syndrome; Recurrent Small Lymphocytic Lymphoma; Refractory Chronic Lymphocytic Leukemia; Refractory Hairy Cell Leukemia; Richter Syndrome; Small Intestinal Lymphoma; Splenic Marginal Zone Lymphoma; T-Cell Large Granular Lymphocyte Leukemia; Testicular Lymphoma; Waldenstrom Macroglobulinemia

  9. Association study with Wegener granulomatosis of the human phospholipase Cγ2 gene

    Directory of Open Access Journals (Sweden)

    Csernok Elena

    2005-02-01

    Full Text Available Abstract Background Wegener Granulomatosis (WG is a multifactorial disease of yet unknown aetiology characterized by granulomata of the respiratory tract and systemic necrotizing vasculitis. Analyses of candidate genes revealed several associations, e.g. with α(1-antitrypsin, proteinase 3 and with the HLA-DPB1 locus. A mutation in the abnormal limb mutant 5 (ALI5 mouse in the region coding for the hydrophobic ridge loop 3 (HRL3 of the phospholipaseCγ2 (PLCγ-2 gene, corresponding to human PLCγ-2 exon 27, leads to acute and chronic inflammation and granulomatosis. For that reason, we screened exons 11, 12 and 13 coding for the hydrophobic ridge loop 1 and 2 (HRL1 and 2, respectively and exon 27 of the PLCγ-2 protein by single strand conformation polymorphism (SSCP, sequencing and PCR/ restriction fragment length polymorphism (RFLP analyses. In addition, we screened indirectly for disease association via 4 microsatellites with pooled DNA in the PLCγ-2 gene. Results Although a few polymorphisms in these distinct exons were observed, significant differences in allele frequencies were not identified between WG patients and respective controls. In addition, the microsatellite analyses did not reveal a significant difference between our patient and control cohort. Conclusion This report does not reveal any hints for an involvement of the PLCγ-2 gene in the pathogenesis of WG in our case-control study.

  10. Place in therapy of rituximab in the treatment of granulomatosis with polyangiitis and microscopic polyangiitis.

    Science.gov (United States)

    Shah, Shivani; Geetha, Duvuru

    2015-01-01

    Granulomatosis with polyangiitis and microscopic polyangiitis are small vessel vasculitides characterized by circulating antineutrophil circulating antibodies. Standard treatment for active severe disease has consisted of cyclophosphamide with glucocorticoids with or without plasmapheresis, which achieves approximately 75% sustained remission, but carries significant adverse effects such as malignancy, infertility, leukopenia, and infections. The role of B cells in the pathogenesis of anti-neutrophil circulating antibodies-associated vasculitis has been established, and as such, rituximab, a monoclonal anti-CD20 antibody, has been studied in treatment of active granulomatosis with polyangiitis and microscopic polyangiitis (induction) and in maintaining remission. Rituximab has been shown to be effective in inducing remission in several retrospective studies in patients with refractory disease or cyclophosphamide intolerance. The RAVE and RITUXVAS trials demonstrated rituximab is a noninferior alternative to standard cyclophosphamide-based therapy; however, its role in elderly patients and patients with severe renal disease warrants further investigation. Rituximab has been compared with azathioprine for maintaining remission in the MAINRITSAN trial and may be more efficacious in maintaining remission in patients treated with cyclophosphamide induction. Rituximab is not without risks and carries a similar adverse event risk rate as cyclophosphamide in randomized control trials. However, its use can be considered over cyclophosphamide in patients who have relapsing or refractory disease or in young patients seeking to preserve fertility.

  11. Granulomatosis with polyangiitis: rapidly progressive necrotizing glomerulonephritis in a pediatric patient

    Directory of Open Access Journals (Sweden)

    Luna M

    2014-04-01

    Full Text Available Mariana Luna,1 Victoria Bocanegra,3 Patricia G Vallés1,21Nephrology Division, Pediatric Department, Dr Humberto Notti Pediatric Hospital, Mendoza, Argentina; 2Pathophysiology Area, Pathology Department, School of Medicine, National Cuyo University, Mendoza, Argentina; 3National Council of Scientific and Technical Research of Argentina (CONICET, Buenos Aires, ArgentinaAbstract: Granulomatosis with polyangiitis (GPA is associated with a broad range of clinical manifestations including renal disease. It is a systemic vasculitis that is rarely encountered in children. We present a 14-year-old girl who suffered from pharyngitis 1 week before admittance to hospital. She was admitted for macroscopic hematuria and oliguria, under the possibility of nephritic syndrome. Renal failure with rapidly progressive glomerulonephritis occurred within 24 hours. Immunologic tests showed the presence of type-C anti-neutrophil cytoplasmic antibodies (c-ANCA with antiproteinase 3 specificity and renal biopsy revealed pauci-immune crescentic focal necrotizing glomerulonephritis. Treatment including methylprednisolone and cyclophosphamide intravenous pulses allowed renal recovery after 3 weeks. The clinical, hematological, and biochemical parameters improved substantially, achieving remission. Granulomatosis with polyangiitis, although rare in children, should be considered in the above clinical scenario. This case underlines that knowledge of renal histology diagnosis and early aggressive immunosuppressive therapy are essential for the management of these patients.Keywords: acute renal failure, vasculitis, crescentic pauci-immune glomerulonephritis-Type-C, antineutrophil cytoplasmic antibodies (c-ANCA-macroscopic hematuria

  12. Atypical Endobronchial Carcinoid with Postobstructive Pneumonia Obscuring the Diagnosis of Granulomatosis with Polyangiitis

    Science.gov (United States)

    Ali, Robert; Baldeo, Candice; Onyenekwe, Jesse; Lala, Roshan; Landa, Cristian; Siddiqi, Anwer

    2015-01-01

    Granulomatosis with polyangiitis (GPA), previously termed Wegener's Granulomatosis, is an autoimmune small vessel vasculitis which is highly associated with antineutrophil cytoplasmic antibodies (ANCA) and has varied clinical manifestations. Diagnosis hinges on identifying a combination of clinical features of systemic vasculitis, positive ANCA serology, and histological evidence of necrotizing vasculitis, necrotizing glomerulonephritis, or granulomatous inflammation from a relevant organ biopsy. The American College of Rheumatology has also developed a classification criteria focusing specifically on nasal or oral inflammation, abnormal chest radiograph, and abnormal urinary sediment, along with granulomatous inflammation, which helps to distinguish GPA from other forms of systemic vasculitis. In the case presented below, the diagnosis of GPA was delayed as the patient had a concomitant atypical endobronchial carcinoid which predisposed to postobstructive pneumonia. Fortunately, the papular lesions that developed across her lower limbs prompted further investigations. The return of appropriate serology coincided with progression to alveolar hemorrhage, offering a more complete clinical picture, and when she responded to the combination of steroid, cyclophosphamide, and plasma exchange, the diagnosis of GPA was cinched. PMID:26347844

  13. Atypical Endobronchial Carcinoid with Postobstructive Pneumonia Obscuring the Diagnosis of Granulomatosis with Polyangiitis

    Directory of Open Access Journals (Sweden)

    Robert Ali

    2015-01-01

    Full Text Available Granulomatosis with polyangiitis (GPA, previously termed Wegener’s Granulomatosis, is an autoimmune small vessel vasculitis which is highly associated with antineutrophil cytoplasmic antibodies (ANCA and has varied clinical manifestations. Diagnosis hinges on identifying a combination of clinical features of systemic vasculitis, positive ANCA serology, and histological evidence of necrotizing vasculitis, necrotizing glomerulonephritis, or granulomatous inflammation from a relevant organ biopsy. The American College of Rheumatology has also developed a classification criteria focusing specifically on nasal or oral inflammation, abnormal chest radiograph, and abnormal urinary sediment, along with granulomatous inflammation, which helps to distinguish GPA from other forms of systemic vasculitis. In the case presented below, the diagnosis of GPA was delayed as the patient had a concomitant atypical endobronchial carcinoid which predisposed to postobstructive pneumonia. Fortunately, the papular lesions that developed across her lower limbs prompted further investigations. The return of appropriate serology coincided with progression to alveolar hemorrhage, offering a more complete clinical picture, and when she responded to the combination of steroid, cyclophosphamide, and plasma exchange, the diagnosis of GPA was cinched.

  14. Cutaneous granulomatosis and combined immunodeficiency revealing Ataxia-Telangiectasia: a case report

    Directory of Open Access Journals (Sweden)

    Antoccia Antonio

    2010-04-01

    Full Text Available Abstract Ataxia-telangiectasia (A-T is a complex multisystem disorder characterized by progressive neurological impairment, variable immunodeficiency and oculo-cutaneous telangiectasia. A-T is a member of chromosomal breakage syndromes and it is caused by a mutation in the ataxia-telangiectasia mutated (ATM gene. Because of a wide clinical heterogeneity, A-T is often difficult to diagnose in children. We report an unusual case of a 3-year-old boy affected by A-T who presented exclusively with extensive cutaneous granulomatosis and severe combined immunodeficiency, without neurological abnormalities, at the time of diagnosis. This case clearly emphasizes the variable presentation of A-T syndrome and highlights the difficulties in the early diagnosis of A-T. A-T should be considered in children with evidence of combined humoral and cellular immunodeficiency associated with unexplained skin granulomatous lesions, even in the absence of the classic features of this syndrome.

  15. Granulomatosis de células de Langerhans: Presentación de un caso

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    . Caridad Nazco Ríos

    1998-12-01

    Full Text Available Se reporta un inusual caso de granulomatosis de células de Langerhans localizado en maxilares en un paciente de 30 meses de edad, quien presentaba además de las lesiones líticas óseas, lesiones de aspecto tumoral en encía palatina y lingual de ambas arcadas dentarias. El examen óseo no arrojaba compromiso de otros huesos. El diagnóstico histológico fue corroborado por el Centro Nacional de Referencia en Anatomía Patológica.A unusual case of Langerhans´ cells is reported, located in maxillae of a 30 months old patients, who, apart from bone lithic injuries, showed injuries of tumoral type in lingual and palatine gingiva of both dental archs. According to bone examination, there weren´t involvement of other bones. Histologic diagnosis was corroborated by National Reference Center in Pathologic Anatomy.

  16. Meningeal involvement in Wegener granulomatosis: case report and review of the literature.

    Science.gov (United States)

    Soriano, A; Lo Vullo, M; Casale, M; Quattrocchi, C C; Afeltra, A

    2012-01-01

    Wegener Granulomatosis (WG) is a multisystem autoimmune disorder characterized by necrotizing granulomatous vasculitis that most commonly involves the upper respiratory tract, lungs, and kidneys. The involvement of the central nervous system (CNS) is infrequent and can cause stroke, cranial nerve abnormalities, cerebrovascular events, seizures, and meningeal involvement. Meningeal involvement is rare and may occur due to local vasculitis, directly spread from adjacent disease in the skull base, paranasal or orbital region. We describe the case of a 20-year-old Caucasian man who was diagnosed with sinonasal WG with frontal focal meningeal involvement. A literature review on diagnosis and treatment of meningeal involvement in course of WG was carried out. The importance of an early diagnosis and treatment of localized WG has been emphasized, in order to avoid the progression to a severe form of disease, especially in younger patients and in paucisymptomatic cases.

  17. Pronounced Risk of Fractures among Elderly Men Affected by Granulomatosis with Polyangiitis

    DEFF Research Database (Denmark)

    Faurschou, Mikkel; Baslund, Bo; Obel, Niels

    2015-01-01

    OBJECTIVE: It is unknown whether patients affected by granulomatosis with polyangiitis (GPA) are at increased risk of fractures, and whether the fracture risk in GPA varies with age and sex. The aim of the present study was to compare the fracture risk among patients with GPA with that among age......- and sex-matched population controls. METHODS: We established a monocentric cohort of patients treated for GPA at a Danish tertiary care center from 1995 to 2010 (n = 159) and a register-derived GPA cohort identified from the Danish National Hospital Register (n = 402). Each patient was matched with 7...... population controls. The occurrence of fractures among patients was compared with that among controls by calculation of incidence rate ratios (IRR). RESULTS: In the monocentric cohort, an increased fracture risk was observed among men aged ≥ 55 years at the time of first hospitalization for GPA (IRR 3.5, 95...

  18. Risk of Diabetes Mellitus among Patients Diagnosed with Giant Cell Arteritis or Granulomatosis with Polyangiitis

    DEFF Research Database (Denmark)

    Faurschou, Mikkel; Ahlström, Magnus G; Lindhardsen, Jesper

    2017-01-01

    patients diagnosed with giant cell arteritis (GCA) or granulomatosis with polyangiitis (GPA), i.e., patients with relatively common forms of systemic vasculitis. METHODS: We used Danish healthcare registries to identify 1682 patients diagnosed with GCA and 342 patients diagnosed with GPA from 1997 to 2015.......7-10.6) in the GPA cohort. During the first year after diagnosis of vasculitis, the IRR for DM was 7.0 (95% CI 5.2-9.3) among patients with GCA and 10.4 (95% CI 4.4-24) among patients with GPA. IRR for DM were not significantly increased in either cohort during later followup periods. Within the first year......, treatment with high cumulative prednisolone/PRED doses was associated with new-onset DM among the patients with vasculitis. CONCLUSION: Patients diagnosed with GCA or GPA have a markedly increased risk of new-onset DM during early treatment phases....

  19. Suppurative dacroadenitis causing ocular sicca syndrome in classic Wegener′s granulomatosis

    Directory of Open Access Journals (Sweden)

    Khanna Dhanita

    2011-01-01

    Full Text Available Wegener′s granulomatosis (WG is a multisystem vasculitic disorder which can commonly afflict various components of the eye. Here we describe some unusual ocular manifestations of the disease in one patient. A young male with history of upper respiratory tract symptoms including epistaxis, nasal stuffiness and maxillary sinus pain presented with bilateral lacrimal gland abscess and ptosis. Lacrimal gland biopsy revealed granulomatous vasculitis. Lung cavities, positive cytoplasmic-antineutrophil cytoplasmic antibodies and high titers of serine proteinase-3 antibodies confirmed the diagnosis of WG. The patient developed dry eyes after a month of first presentation. There was no dryness of mouth, suggesting the absence of salivary gland involvement, and antinuclear antibodies as well as antibodies against Ro and La antigens classical of primary Sjogren′s syndrome were absent. Granulomatous vasculitis of lacrimal gland leading to abscess formation and dryness of eyes has not been described in WG and reflects the aggressive nature of inflammatory process in this disease.

  20. Granulomatosis with polyangiitis and facial palsy: Literature review and insight in the autoimmune pathogenesis.

    Science.gov (United States)

    Iannella, Giannicola; Greco, Antonio; Granata, Guido; Manno, Alessandra; Pasquariello, Benedetta; Angeletti, Diletta; Didona, Dario; Magliulo, Giuseppe

    2016-07-01

    Granulomatosis with polyangiitis (GPA) is an autoimmune systemic necrotizing small-vessel vasculitis associated with the presence of anti-neutrophil cytoplasmic antibodies (ANCA). Oto-neurological manifestations of ANCA-associated vasculitis according to PR3-ANCA positivity and MPO-ANCA positivity are usually reported. Facial nerve palsy is usually reported during the clinical course of the disease but it might appear as the presenting sign of GPA. Necrotizing vasculitis of the facial nerve 'vasa nervorum' is nowadays the most widely accepted etiopathogenetic theory to explain facial damage in GPA patients. A central role for PR3-ANCA in the pathophysiology of vasculitis in GPA patients with oto-neurological manifestation is reported. GPA requires prompt, effective management of the acute and chronic manifestations. Once the diagnosis of GPA has been established, clinicians should devise an appropriate treatment strategy for each individual patient, based on current clinical evidence, treatment guidelines and recommendations.

  1. Combined CNS and pituitary involvement as a primary manifestation of Wegener granulomatosis.

    Science.gov (United States)

    Spísek, Radek; Kolouchová, Elena; Jensovský, Jirí; Rusina, Robert; Fendrych, Pavel; Plas, Jaroslav; Bartůnková, Jirina

    2006-09-01

    Wegener granulomatosis (WG) is a systemic vasculitis of small and medium vessels. It predominantly affects the upper and/or lower respiratory airway and kidneys. Its pathogenesis is not fully understood. WG relatively frequently affects the nervous system (in 30-50% according to the different studies). Most frequently, it manifests as necrotizing vasculitis that leads to the peripheral neuropathies or to the cranial nerves palsy. Impairment of the central nervous system (CNS) is less frequent and occurs in 2-8% of patients. Three major pathogenetic mechanisms were described: CNS vasculitis, spreading of granulomas from the adjacent anatomical areas (paranasal cavities, orbit etc.), and new formation of granulomas in brain tissue. This case report describes patients in whom WG manifested in the form of localized skin involvement and combined CNS involvement that included pituitary gland. Atypical presentation of WG impedes and slows down the process of diagnosis and emphasizes the need for collaboration between medical specialists.

  2. Cardiopatía valvular en un paciente con granulomatosis de Wegener

    Directory of Open Access Journals (Sweden)

    Luis E. Silva, MD

    2012-01-01

    Full Text Available La granulomatosis de Wegener es una vasculitis necrotizante autoinmune cuya prevalencia reportada es de 3/100.000 habitantes. Tiene compromiso multisistémico, principalmente el tracto respiratorio superior e inferior, y el sistema nervioso central y renal. La frecuencia del compromiso cardiaco varía según las series estudiadas, pero oscila alrededor de 6%; sin embargo, las manifestaciones clínicas son poco frecuentes. El compromiso valvular se reporta como insuficiencia aórtica o mitral, secundaria a infiltración de las valvas o dilatación de la raíz aórtica. El tratamiento se basa en el control de la enfermedad, el manejo de la falla cardiaca y la intervención quirúrgica de la válvula comprometida según la indicación.

  3. [Tracheal resection for post-intubation subglottic stenosis in a patient with granulomatosis with polyangiitis (Wegener)].

    Science.gov (United States)

    Stoica, Radu; Negru, Irina; Matache, Radu; MirunaTodor

    2014-01-01

    Granulomatosis with polyangiitis (GPA or Wegener) is a systemic autoimmune disease with inflammation of small- and medium-size vessels. It can affect practically any organ or system, but renal, respiratory andjoint systems are most frequently damaged. Positive pANCA antibodies can raise the suspicion of diagnosis. Subglottic stenosis is relatively frequent, in a quarter of patients, especially in the third decade women. The case presented is of an 80-year-old woman, recently diagnosed with pulmonary, renal and systemic manifestations of GPA and with a subglottic stenosis rapidly evolving towards endotracheal intubation, tracheostomy with mechanical ventilation and renal failure. Further evolution has been favorable under corticoid therapy. After weaning from the mechanical ventilation and30 days after the suppression of the tracheostomy, the patient developed a tracheal stenosis with mixed etiology, secondary to vasculitis and prolonged intubation with tracheostomy. Tracheal resection with termino-terminal anastomosis was performed in emergency with simple post-operative evolution and without late complications.

  4. [Tracheal resection for post-intubation subglottic stenosis in a patient with granulomatosis with polyanaiitis (Wegener)].

    Science.gov (United States)

    Stoica, Radu; Negru, Irina; Matache, Radu; MirunaTodor

    2014-01-01

    Granulomatosis with polyangiitis (GPA or Wegener) is a systemic autoimmune disease with inflammation of small- and medium-size vessels. It can affect practically any organ or system, but renal, respiratory andjoint systems are most frequently damaged. Positive pANCA antibodies can raise the suspicion of diagnosis. Subglottic stenosis is relatively frequent, in a quarter of patients, especially in the third decade women. The case presented is of an 80-year-old woman, recently diagnosed with pulmonary, renal and systemic manifestations of GPA and with a subglottic stenosis rapidly evolving towards endotracheal intubation, tracheostomy with mechanical ventilation and renal failure. Further evolution has been favorable under corticoid therapy. After weaning from the mechanical ventilation and30 days after the suppression of the tracheostomy, the patient developed a tracheal stenosis with mixed etiology, secondary to vasculitis and prolonged intubation with tracheostomy. Tracheal resection with termino-terminal anastomosis was performed in emergency with simple post-operative evolution and without late complications.

  5. Airway Surgery in Tracheostomised Patients with Wegener Granulomatosis Leading to Subglottic Stenosis.

    Science.gov (United States)

    Altun, Demet; Sivrikoz, Nükhet; Çamcı, Emre

    2015-10-01

    Wegener granulomatosis (WG) is a multisystemic disorder characterised by granulomatous inflammation of the respiratory system. The growing of proliferative tissue towards the larynx and trachea may cause airway obstruction on account of subglottic stenosis. In this situation, the surgical goal is to eliminate the airway obstruction by providing natural airway anatomy. While mild lesions do not require surgical intervention, in fixed lesions, surgical intervention is required, such as tracheostomy, laser resection and dilatation. In tracheostomised patients, granuloma formation surrounding the tracheostomy cannula may occur in the trachea. Inflammation and newly formed granulation tissue result in severe stenosis in the airways. During surgical treatment of such patients, airway management is important. In this case report, we will discuss gas exchange and airway management with jet ventilation (JV) during excision of the granulation tissue with endolaryngeal laser surgery, leading to subglottic stenosis in tracheostomised patients in WG.

  6. Fatal Cytomegalovirus Disease after Combination Therapy with Corticosteroids and Rituximab for Granulomatosis with Polyangiitis

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    Talal Hilal

    2015-01-01

    Full Text Available The association of cytomegalovirus (CMV with autoimmune disease is poorly understood with suggested causality and reported viral reactivation coinciding with active inflammation. We report a case of a patient who presented with diffuse alveolar hemorrhage and acute renal failure from rapidly progressive glomerulonephritis ultimately diagnosed with granulomatosis with polyangiitis (GPA. She was acutely managed with plasmapheresis to reduce antibody-mediated end-organ damage, hemodialysis for worsening hyperkalemia and acidosis, and high-dose intravenous methylprednisolone. She was transitioned to oral prednisone and started on weekly rituximab with resultant remission induction over a three-week period at which point she developed reactivation of CMV causing severe fatal lung disease and viremia. The case highlights the multiple factors associated with CMV reactivation in cases of severe systemic inflammatory states and the need for further research to help establish practice guidelines regarding antimicrobial prophylaxis in patients with autoimmune diseases on prolonged courses of corticosteroids and biologic agents.

  7. Granulomatosis de Wegener Semejando Cáncer Epidermoide de Nasofaringe y Metástasis Pulmonares.

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    Gustavo Adolfo Martín Small

    2011-01-01

    Full Text Available La granulomatosis de Wegener (GW es una enfermedad idiopática, con posible componente autoinmune, que aparece generalmente en la quinta década de vida, caracterizándose por lesiones granulomatosas necrotizantes y vasculitis en vías aéreas y riñón. Paciente femenino de 54 años, quien desde Noviembre del 2008, presenta rinorrea, prurito y eritema en borde nasal inferior izquierdo, recibe antibióticos sin mejoría de los síntomas. En las radiografías torácicas, se observan dos radiopacidades redondeadas, de 4 cm de diámetro, sugestivas de lesiones tumorales en ambos campos pulmonares. La biopsia reporta cáncer epidermoide de alto grado, sospechándose primario en nasofaringe. Es remitida, por deterioro de condiciones, al Servicio de Neumonología del Hospital Universitario de Caracas el 18/03/2009, presentando disnea, tos productiva, placas purpúricas dolorosas en manos y pies, hipoacusia, hiperpigmentación del paladar duro, leucoplaquias y lesiones costrosas en lengua, insuficiencia renal (creatinina en 6,11 mg/dL y trombocitopenia. En TAC de tórax, se evidencian tumores mayores de 5 cm de diámetro, algunos con bordes bien definidos y otros mal delimitados con broncograma aéreo. Diagnostico definitivo de GW por serología. La GW con su afectación sistémica puede simular otras entidades como neoplasias de cabeza y cuello con metástasis pulmonares. Cuando las manifestaciones sistémicas son floridas debe sospecharse GW como diagnóstico diferencial. Palabras claves: Wegener, Granulomatosis, Vasculitis, Cáncer epidermoide.

  8. Histopathological features predictive of a clinical diagnosis of ophthalmic granulomatosis with polyangiitis (GPA).

    Science.gov (United States)

    Isa, Hazlita; Lightman, Sue; Luthert, Philip J; Rose, Geoffrey E; Verity, David H; Taylor, Simon R J

    2012-01-01

    The limited form of Granulomatosis with Polyangiitis (GPA), formerly known as Wegener's Granulomatosis (WG) primarily involves the head and neck region, including the orbit, but is often a diagnostic challenge, particularly as it commonly lacks positive anti-neutrophil cytoplasm antibody (ANCA) titres or classical features on diagnostic orbital biopsies. The purpose of this study was to relate biopsy findings with clinical outcome and to determine which histopathological features are predictive of a clinical diagnosis of GPA. Retrospective case series of 234 patients identified from the database of the UCL Institute of Ophthalmology Department of Eye Pathology as having had orbital biopsies of orbital inflammatory disorders performed between 1988 and 2009. Clinical records were obtained for the patients and analysed to see whether patients had GPA or not, according to a standard set of diagnostic criteria (excluding any histopathological findings). Biopsy features were then correlated with the clinical diagnosis in univariate and multivariate analyses to determine factors predictive of GPA. Of the 234 patients, 36 were diagnosed with GPA and 198 with other orbital pathologies. The majority of biopsies were from orbital masses (47%). Histology showed a range of acute and chronic inflammatory pictures in all biopsies, but the presence of neutrophils (PGPA. In a multivariate analysis, only tissue neutrophils (OR=3.6, P=0.01) and vasculitis (OR=2.6, P=0.02) were independently associated with GPA, in contrast to previous reports associating eosinophils and necrosis with the diagnosis. Neutrophil, eosinophil and macrophage infiltration of orbital tissues, together with vasculitis and necrosis, are all associated with a clinical diagnosis of GPA, but only neutrophil infiltration and vasculitis are independently associated with this diagnosis. These features may assist in the establishing the diagnosis of limited GPA among patients with early orbital disease, particularly

  9. Wegener’s granulomatosis and pyoderma gangrenosum – rare causes of facial ulcerations

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    Karolina Kędzierska

    2016-03-01

    Full Text Available Background: Pyoderma gangrenosum (PG is caused by immune system dysfunction, and particularly improper functioning of neutrophils. At least half of all PG patients also suffer from autoimmunological diseases, one of which is Wegener granulomatosis (WG. The purpose of this article was to compare cases of patients with WG and PG in terms of their clinical course, histopathology, and applied treatment. In both, histopathological features are not fully distinct. Data from microbiological and immunological evaluation and clinical presentation are required to establish the diagnosis. We also present the case of a patient with WG and deep facial skin lesions not responding to standard treatment. Methods: Systematic review of the literature in PubMed using the search terms “Wegener granulomatosis AND Pyoderma gangrenosum” and case report. Results: The finding of 22 reports in the literature (PubMed suggests that it is a rare phenomenon. This study revealed a similar rate of comorbidity of WG and PG in both genders and an increased incidence of both diseases after the age of 50. Among skin lesions there was a dominance of ulceration, most often deep and painful, covering a large area with the presence of advanced necrosis and destruction of the surrounding tissue. The most common location proved to be the cervical-cephalic area. The most popular treatment included steroids with cyclophosphamide. Discussion: The rarity of the coexistence of these two diseases results in a lack of effective therapy. In such cases sulfone derivatives are still effective and provide an alternative to standard immunosuppression methods. Hyperbaric therapy and plasmapheresis can also play an important complementary role.

  10. Place in therapy of rituximab in the treatment of granulomatosis with polyangiitis and microscopic polyangiitis

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    Shah S

    2015-08-01

    Full Text Available Shivani Shah, Duvuru Geetha Department of Medicine, Division of Nephrology, Johns Hopkins University School of Medicine, Baltimore, MD, USA Abstract: Granulomatosis with polyangiitis and microscopic polyangiitis are small vessel vasculitides characterized by circulating antineutrophil circulating antibodies. Standard treatment for active severe disease has consisted of cyclophosphamide with glucocorticoids with or without plasmapheresis, which achieves approximately 75% sustained remission, but carries significant adverse effects such as malignancy, infertility, leukopenia, and infections. The role of B cells in the pathogenesis of anti-neutrophil circulating antibodies-associated vasculitis has been established, and as such, rituximab, a monoclonal anti-CD20 antibody, has been studied in treatment of active granulomatosis with polyangiitis and microscopic polyangiitis (induction and in maintaining remission. Rituximab has been shown to be effective in inducing remission in several retrospective studies in patients with refractory disease or cyclophosphamide intolerance. The RAVE and RITUXVAS trials demonstrated rituximab is a noninferior alternative to standard cyclophosphamide-based therapy; however, its role in elderly patients and patients with severe renal disease warrants further investigation. Rituximab has been compared with azathioprine for maintaining remission in the MAINRITSAN trial and may be more efficacious in maintaining remission in patients treated with cyclophosphamide induction. Rituximab is not without risks and carries a similar adverse event risk rate as cyclophosphamide in randomized control trials. However, its use can be considered over cyclophosphamide in patients who have relapsing or refractory disease or in young patients seeking to preserve fertility. Keywords: rituximab, ANCA-associated vasculitis, GPA, MPA, induction therapy, maintenance therapy

  11. Dose Monitoring of Busulfan and Combination Chemotherapy in Hodgkin or Non-Hodgkin Lymphoma Undergoing Stem Cell Transplant

    Science.gov (United States)

    2015-08-12

    Adult Grade III Lymphomatoid Granulomatosis; Adult Nasal Type Extranodal NK/T-cell Lymphoma; Anaplastic Large Cell Lymphoma; Angioimmunoblastic T-cell Lymphoma; Childhood Burkitt Lymphoma; Childhood Diffuse Large Cell Lymphoma; Childhood Grade III Lymphomatoid Granulomatosis; Childhood Immunoblastic Large Cell Lymphoma; Childhood Nasal Type Extranodal NK/T-cell Lymphoma; Contiguous Stage II Adult Burkitt Lymphoma; Contiguous Stage II Adult Diffuse Large Cell Lymphoma; Contiguous Stage II Adult Diffuse Mixed Cell Lymphoma; Contiguous Stage II Adult Diffuse Small Cleaved Cell Lymphoma; Contiguous Stage II Adult Immunoblastic Large Cell Lymphoma; Contiguous Stage II Adult Lymphoblastic Lymphoma; Contiguous Stage II Grade 1 Follicular Lymphoma; Contiguous Stage II Grade 2 Follicular Lymphoma; Contiguous Stage II Grade 3 Follicular Lymphoma; Contiguous Stage II Mantle Cell Lymphoma; Contiguous Stage II Marginal Zone Lymphoma; Contiguous Stage II Small Lymphocytic Lymphoma; Cutaneous B-cell Non-Hodgkin Lymphoma; Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Hepatosplenic T-cell Lymphoma; Intraocular Lymphoma; Nodal Marginal Zone B-cell Lymphoma; Noncontiguous Stage II Adult Burkitt Lymphoma; Noncontiguous Stage II Adult Diffuse Large Cell Lymphoma; Noncontiguous Stage II Adult Diffuse Mixed Cell Lymphoma; Noncontiguous Stage II Adult Diffuse Small Cleaved Cell Lymphoma; Noncontiguous Stage II Adult Immunoblastic Large Cell Lymphoma; Noncontiguous Stage II Adult Lymphoblastic Lymphoma; Noncontiguous Stage II Grade 1 Follicular Lymphoma; Noncontiguous Stage II Grade 2 Follicular Lymphoma; Noncontiguous Stage II Grade 3 Follicular Lymphoma; Noncontiguous Stage II Mantle Cell Lymphoma; Noncontiguous Stage II Marginal Zone Lymphoma; Noncontiguous Stage II Small Lymphocytic Lymphoma; Noncutaneous Extranodal Lymphoma; Peripheral T-cell Lymphoma; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult

  12. Altered Liver Proteoglycan/Glycosaminoglycan Structure as a Manifestation of Extracellular Matrix Remodeling upon BCG-induced Granulomatosis in Mice.

    Science.gov (United States)

    Kim, L B; Shkurupy, V A; Putyatina, A N

    2017-01-01

    Experimental BCG-induced granulomatosis in mice was used to study changes in the dynamics of individual liver proteoglycan components reflecting phasic extracellular matrix remodeling, determined by the host-parasite interaction and associated with granuloma development. In the early BCG-granulomatosis period, the increase in individual proteoglycan components promotes granuloma formation, providing conditions for mycobacteria adhesion to host cells, migration of phagocytic cells from circulation, and cell-cell interaction leading to granuloma development and fibrosis. Later, reduced reserve capacity of the extracellular matrix, development of interstitial fibrosis and granuloma fibrosis can lead to trophic shortage for cells within the granulomas, migration of macrophages out of them, and development of spontaneous necrosis and apoptosis typical of tuberculosis.

  13. Estenosis subglótica y granulomatosis con poliangeítis (Wegener) en dos casos: Report of two cases

    OpenAIRE

    Massa,María; Nicholas C. Emery; Bosio, Martín; Finn,Bárbara C; Bruetman,Julio E; Young,Pablo

    2014-01-01

    Granulomatosis with polyangiitis (GPA) or Wegener's disease is characterized by a granulomatous vasculitis of the upper and lower airways and kidney. It involves the lower respiratory tract causing subglottic tracheal stenosis, which occurs in approximately 22% of patients. We report two females aged 40 and 52 years, admitted to the hospital with a subglottic tracheal stenosis. Their symptoms and management are reviewed. The frst patient responded to rituximab. The second patient required a t...

  14. Wegener's Granulomatosis

    Science.gov (United States)

    ... to) Wegener’s, but no specific infection (bacterial, viral, fungal, or other) has been identified. How is Wegener’s ... ANCAs. Some patients with inflammatory bowel disease, rheumatoid arthritis, or SLE may have atypical P-ANCA test ...

  15. Estenose subglótica como manifestação clínica da granulomatose de Wegener em adolescentes: relato de caso e revisão de literatura Subglottic stenosis as a clinical manifestation of Wegener's granulomatosis in adolescents: report of a case and review of literature

    Directory of Open Access Journals (Sweden)

    Márcia C. Machado

    2003-10-01

    Full Text Available A Granulomatose de Wegener é uma patologia caracterizada por vasculite sistêmica e inflamação granulomatosa necrotizante que compromete o trato respiratório alto, pulmões e rins, cujas manifestações iniciais, na maioria das vezes, ocorre em maiores de 20 anos de idade. As queixas clínicas otorrinolaringológicas estão, com freqüência, presentes nas fases iniciais da doença, sendo rinite, sinusopatia de repetição e epistaxe as mais comuns. A estenose subglógica parece estar correlacionada com a Granulomatose de Wegener em adolescentes. Relatamos o caso de uma paciente portadora de Granulomatose de Wegener cujo início dos sintomas foi na infância, tendo evoluído com estenose laríngea durante o curso da doença.Wegener's Granulomatosis is a well-characterized systemic vasculitis and necrotising granulomatous inflammation of the upper, lower respiratory tracts and the kidneys. The initial manifestations of the disease usually occur in patients over than twenty years old. The otolaryngologic symptoms like rhinorrhea, recurrent sinusitis and epistaxis are commonly present in early course of the disease. It seems that subglottic stenosis is correlated to Wegener's granumatosis in adolescents. We describe a case of a patient that developed subglottic stenosis associated with Wegener's granulomatosis in childhood during the course of the disease.

  16. Wegener's granulomatosis with renal involvement: patient survival and correlations between initial renal function, renal histology, therapy and renal outcome.

    Science.gov (United States)

    Andrassy, K; Erb, A; Koderisch, J; Waldherr, R; Ritz, E

    1991-04-01

    Patient survival and renal outcome were followed in 25 patients with biopsy confirmed Wegener's granulomatosis and renal involvement. Fourteen out of 25 patients required dialysis on admission, 11/25 patients did not. All patients were treated with a novel protocol comprising methylprednisolone and cyclophosphamide. The median follow-up observation was 36 months (12-113 months). With the exception of 1 patient (who died from causes not related to Wegener's granulomatosis) all patients are alive. Among the patients initially requiring dialysis (n = 14) 4 are in terminal renal failure after 0, 7, 21 and 38 months respectively. In the nondialysis group (n = 11) only 1 patient subsequently required chronic dialysis 30 months after clinical admission. Renal failure was due to non-compliance with immunosuppressive therapy in at least 2 patients. Percentage of obsolescent glomeruli and the degree of tubulointerstitial lesions, but not active glomerular lesions (crescents, necroses) predicted renal outcome. The major cause of renal functional impairment was relapse of Wegener's granulomatosis usually within 2 years after clinical remission. Therefore prolonged treatment with cyclophosphamide for at least 2 years after clinical remission is recommended. Two patients with initially negative immunohistology had a second renal biopsy which revealed de novo appearance of mesangial IgA deposits.

  17. Gamma-Secretase Inhibitor RO4929097 in Treating Young Patients With Relapsed or Refractory Solid Tumors, CNS Tumors, Lymphoma, or T-Cell Leukemia

    Science.gov (United States)

    2014-11-04

    Childhood Atypical Teratoid/Rhabdoid Tumor; Childhood Central Nervous System Choriocarcinoma; Childhood Central Nervous System Germinoma; Childhood Central Nervous System Mixed Germ Cell Tumor; Childhood Central Nervous System Teratoma; Childhood Central Nervous System Yolk Sac Tumor; Childhood Choroid Plexus Tumor; Childhood Craniopharyngioma; Childhood Ependymoblastoma; Childhood Grade I Meningioma; Childhood Grade II Meningioma; Childhood Grade III Meningioma; Childhood Infratentorial Ependymoma; Childhood Medulloepithelioma; Childhood Mixed Glioma; Childhood Oligodendroglioma; Childhood Supratentorial Ependymoma; Gonadotroph Adenoma; Pituitary Basophilic Adenoma; Pituitary Chromophobe Adenoma; Pituitary Eosinophilic Adenoma; Prolactin Secreting Adenoma; Recurrent Childhood Acute Lymphoblastic Leukemia; Recurrent Childhood Anaplastic Large Cell Lymphoma; Recurrent Childhood Brain Stem Glioma; Recurrent Childhood Central Nervous System Embryonal Tumor; Recurrent Childhood Cerebellar Astrocytoma; Recurrent Childhood Cerebral Astrocytoma; Recurrent Childhood Ependymoma; Recurrent Childhood Grade III Lymphomatoid Granulomatosis; Recurrent Childhood Large Cell Lymphoma; Recurrent Childhood Lymphoblastic Lymphoma; Recurrent Childhood Medulloblastoma; Recurrent Childhood Pineoblastoma; Recurrent Childhood Small Noncleaved Cell Lymphoma; Recurrent Childhood Spinal Cord Neoplasm; Recurrent Childhood Subependymal Giant Cell Astrocytoma; Recurrent Childhood Supratentorial Primitive Neuroectodermal Tumor; Recurrent Childhood Visual Pathway and Hypothalamic Glioma; Recurrent Childhood Visual Pathway Glioma; Recurrent Pituitary Tumor; Recurrent/Refractory Childhood Hodgkin Lymphoma; T-cell Childhood Acute Lymphoblastic Leukemia; T-cell Large Granular Lymphocyte Leukemia; TSH Secreting Adenoma; Unspecified Childhood Solid Tumor, Protocol Specific

  18. High-Dose Chemotherapy With or Without Total-Body Irradiation Followed by Autologous Stem Cell Transplant in Treating Patients With Hematologic Cancer or Solid Tumors

    Science.gov (United States)

    2016-11-07

    Adult Acute Lymphoblastic Leukemia in Remission; Adult Acute Myeloid Leukemia in Remission; Adult Acute Myeloid Leukemia With 11q23 (MLL) Abnormalities; Adult Acute Myeloid Leukemia With Del(5q); Adult Acute Myeloid Leukemia With Inv(16)(p13;q22); Adult Acute Myeloid Leukemia With t(15;17)(q22;q12); Adult Acute Myeloid Leukemia With t(16;16)(p13;q22); Adult Acute Myeloid Leukemia With t(8;21)(q22;q22); Adult Nasal Type Extranodal NK/T-cell Lymphoma; Childhood Acute Lymphoblastic Leukemia in Remission; Childhood Acute Myeloid Leukemia in Remission; Childhood Burkitt Lymphoma; Childhood Diffuse Large Cell Lymphoma; Childhood Immunoblastic Large Cell Lymphoma; Childhood Nasal Type Extranodal NK/T-cell Lymphoma; Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor (PNET); Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Hepatosplenic T-cell Lymphoma; Intraocular Lymphoma; Nodal Marginal Zone B-cell Lymphoma; Peripheral T-cell Lymphoma; Plasma Cell Neoplasm; Primary Systemic Amyloidosis; Recurrent Adult Acute Lymphoblastic Leukemia; Recurrent Adult Acute Myeloid Leukemia; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Grade III Lymphomatoid Granulomatosis; Recurrent Adult Hodgkin Lymphoma; Recurrent Adult Immunoblastic Large Cell Lymphoma; Recurrent Adult Lymphoblastic Lymphoma; Recurrent Childhood Acute Lymphoblastic Leukemia; Recurrent Childhood Acute Myeloid Leukemia; Recurrent Childhood Anaplastic Large Cell Lymphoma; Recurrent Childhood Grade III Lymphomatoid Granulomatosis; Recurrent Childhood Large Cell Lymphoma; Recurrent Childhood Lymphoblastic Lymphoma; Recurrent Childhood Small Noncleaved Cell Lymphoma; Recurrent Cutaneous T-cell Non-Hodgkin Lymphoma; Recurrent Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2

  19. MicroRNA-941 Expression in Polymorphonuclear Granulocytes Is Not Related to Granulomatosis with Polyangiitis

    DEFF Research Database (Denmark)

    Svendsen, Jesper Brink; Baslund, Bo; Cramer, Elisabeth Præstekjær

    2016-01-01

    granulomatosis with polyangiitis (GPA) have lower expression of miR-941 than healthy control donors as a biological cause for higher JMJD3 levels. We found no significant difference in the degree of maturation of PMNs from GPA patients (n = 8) and healthy controls (n = 11) as determined from cell surface...... expression of the neutrophil maturation marker CD16 and gene expression profile of FCGR3B. The expression of PRTN3 and KDM6B mRNAs and miR-941 was not significantly different in GPA patients and healthy controls. Transfection of pre-miR-941 into the neutrophil promyelocyte cell line PLB-985 cells did...... not result in reduction of the KDM6B mRNA level as shown previously in a hepatocellular carcinoma cell line. The amount of PR3 in PMNs from GPA patients and healthy controls was comparable. In conclusion, we found that PRTN3 mRNA, KDM6B mRNA, and miR-941 expression levels in PMNs do not differ between GPA...

  20. Impact of pre-existing co-morbidities on mortality in granulomatosis with polyangiitis

    DEFF Research Database (Denmark)

    Faurschou, Mikkel; Ahlström, Magnus Glindvad; Lindhardsen, Jesper

    2016-01-01

    OBJECTIVE: To assess the impact of pre-existing co-morbidities on mortality among patients affected by granulomatosis with polyangiitis (GPA). METHODS: By means of the Danish National Hospital Register, we identified a cohort of patients hospitalized for GPA during 1994-2010 (n = 308). The burden...... throughout 2010. Cox regression analyses were used to calculate mortality rate ratios (MRRs). RESULTS: The median duration of follow-up in the GPA cohort was 5.8 years (interquartile range 2.3-10.0). Compared with their matched population controls, the MRR for patients presenting with a CCI score of 0 (n...... = 246) was 3.9 (95% CI 2.0, 7.5) during years 0-2 and 1.4 (95% CI 0.9, 2.0) from the second year of follow-up onwards. The corresponding MRRs were 13.3 (95% CI 5.8, 31) and 1.9 (95% CI 1.1, 3.6) for patients with a CCI score ⩾1 (n = 62). In a direct comparison, GPA patients with a CCI score ⩾1 were...

  1. Eosinophilic granulomatosis with polyangiitis complicated by cholecystitis: a case report and review of the literature.

    Science.gov (United States)

    Ye, Lu; Lu, Xiaoyong; Xue, Jing

    2016-01-01

    The objectives are to report an atypical case of eosinophilic granulomatosis with polyangiitis (EGPA) associated with cholecystitis and to review the main clinical features, therapy, and prognosis of it. We present a 49-year-old male with non-classic clinical manifestations of EGPA and EGPA-related cholecystitis. EGPA was diagnosed by histology of the gallbladder after cholecystectomy. In addition, 11 cases of EGPA-associated cholecystitis have been reported and were described in details in this article. Gallbladder involvement is very uncommon in EGPA. All cases reviewed showed multiple organs involved as well as obviously elevated eosinophilic granulocyte proportion with inflammatory index, although antineutrophil cytoplasmic antibody may be negative. All patients in this cohort that showed gallbladder involvement were eventually confirmed with EGPA by histology examination after cholecystectomy. The pathological change could be infiltration of inflammatory mononuclear cells of small- and medium-sized vessels. Of the cases, 91.7% responded well to steroid and immunosuppressant therapy. Gallbladder involvement is a very rare comorbid condition in EGPA. However, it is an important symptom or secondary condition to alert physicians the diagnosis of EGPA. Moreover, timely diagnosis and correct administration in the early stage of this disease could obviously improve the prognosis.

  2. Efficiency of using rituximab in a patient with generalized granulomatosis with polyangiitis: A case report

    Directory of Open Access Journals (Sweden)

    Gulazyk Malikovna Koilubaeva

    2014-01-01

    Full Text Available Systemic vasculitides (SVs are characterized by inflammation of the blood vessels wall; the spectrum of their clinical manifestations depends on the type, extent, and location of affected vessels and the activity of systemic inflammation. The etiology of most primary SVs is unknown. Antineutrophil cytoplasmic antibodies (ANCAs are implicated in its pathogenesis. The presence of ANCAa in patients' serum and the correlation of their level with the severity of clinical manifestations served as a basis for identifying a subgroup of systemic necrotizing vasculitides associated with ANCA synthesis: granulomatosis with polyangiitis (GPA, microscopic polyangiitis, and Churg – Strauss syndrome. GPA is characterized by systemic granulomatous necrotizing vasculitis involving the small vessels of the upper respiratory tract, lung, and kidney.The paper describes a case of difficult diagnosis and successful rituximab (RTM treatment of generalized GPA in a 45-year-old female patients. The disease occurred with local damage to the upper respiratory tract, granulomatous inflammation of the pulmonary vessels to form multiple infiltrates with lung tissue destruction elements and necrotizing glomerulonephritis. Despite intensive immunosuppressive treatment, there was a rapid GPA progression with the further development of respiratory failure, which had been induced by stenotic laryngitis subglottica leading to tracheostoma. Damage to the organ of vision could lead to severe complications, including amaurosis. RMT was shown to be effective in treating generalized GPA with a poor prognosis.

  3. Comparison of the Phenotype and Outcome of Granulomatosis with Polyangiitis Between UK and Japanese Cohorts.

    Science.gov (United States)

    Furuta, Shunsuke; Chaudhry, Afzal N; Arimura, Yoshihiro; Dobashi, Hiroaki; Fujimoto, Shouichi; Homma, Sakae; Rasmussen, Niels; Jayne, David R

    2017-02-01

    There are differences in the frequencies of antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis subgroups between different geographic regions, and we have reported differences in the phenotype of microscopic polyangiitis between Europe and Japan. In this retrospective observational study, we compared phenotypes and outcomes of granulomatosis with polyangiitis (GPA) between the United Kingdom and Japan. We identified 128 UK and 82 Japanese patients with GPA diagnosed between 2000 and 2012. We evaluated baseline characteristics including ANCA status and organ involvement, treatment, patient and renal survival, and time to first relapse. Median age at onset was higher in Japan than in the UK (62.2 yrs vs 57.5 yrs, p UK (61.0% vs 85.2%, p UK (34.1% vs 8.6%, p UK (68.1 μmol/l vs 101.0 μmol/l, p UK (69.5% vs 40.6%, p UK, respectively (p = 0.03). At 60 months the cumulative relapse rates were 37.1% and 68.1% in Japan and the UK, respectively (p UK patients. The relapse-free survival rate was higher in Japan than the United Kingdom.

  4. [Subglottic stenosis in Wegener's granulomatosis--a diagnostic and therapeutic problem].

    Science.gov (United States)

    Wierzbicka, Małgorzata; Gaweqcki, Wojciech; Pastusiak, Tomasz; Szyfter, Witold

    2010-01-01

    Wegener's granulomatosis (WG) is a disease caused by necrotising vasculitis of small and middle blood vessels of upper and lower respiratory tract and kidneys of unknown etiology. ENT symptoms develop in more than 90% of patients and tracheobronchial involvement occurs in about 20% of patients, most often as a subglottic stenosis. Subglottic stenosis occurs usually as a late complication of disease, but sometimes it may be an early isolated symptom. It is usually irregular, no longer than 2-4 cm and affects mucosa and submucosa but sometimes also cartilage. The diagnosis is based on clinical symptoms, X-ray of the chest, urine analysis, c-ANCA detection and histological examination of the granulation from the stenosis. The main treatment of subglottic stenosis in WG is a causal immunosuppressive treatment, however an equally important is a preservation of respiratory tract passage, because a dominant symptom in this form of WG is problem with breathing or even dyspnoea. In this publication basing on literature review different methods of treatment of laryngotracheal stenosis and its importance in WG are described and discussed. In literature presently less invasive operations are recommended. The treatment of choice is endoscopic treatment with repeated dilatations and injections of steroid into the stenosis. In big stenosis in immunologically active disease patient sometimes requires tracheotomy and after remission of disease and maturation of the subglottic scar laryngotracheal resection can be considered.

  5. [Sarcoidosis--granulomatosis: the modern view of the etiology and pathogenesis with clinical cases review].

    Science.gov (United States)

    Kazmirchuk, V E; Tsarik, V V; Mal'tsev, D V; Ishchenko, M I

    2014-01-01

    Diagnostic criteria of sarcoidosis were offered in the 60-ies of XX century, however today the problem of sarcoidosis is difficult for understanding the different specialists and early detection. The development of laboratory diagnostic of viral infections and introduction of polymerase chain reaction (PCR) has greatly improved the level of diagnosis of herpes infections, reveal the previously unknown etiology of many diseases: sarcoidosis (granulomatosis), migraine, multiple sclerosis, cystic prenatal brain damage, convulsions, Hodgkin's disease and others. Sarcoidosis is the set of clinical symptoms (fatigue, shortness of breath, coughing, heaviness in the chest), laboratory, radiological and histopathological data that allow the doctor to diagnose, predict prognosis and treatment policy. Most often, sarcoidosis affects the lungs and thoracic lymph nodes. In the last period for 2011-2013 in 2930 immunologically tested patients the sarcoidosis was confirmed in 146. Primarily these patients were exposed to different diagnosis--COPD, pneumonia, tuberculosis, lung cancer. Among patients with sarcoidosis on the first place in frequency of detection were EBV and HHV-6. We were the first in 2000, described the Epstein-Barr virus as the causative factor of sarcoidosis, and has been hypothesized the immunopathology of sarcoidosis and principles of individual immunotherapy with a resolution of the granulomatous process in 92 % of cases. Subsequently, this association has been and illustrates the relationship to other viruses (HHV-6, HHV-8) demonstrated by other authors.

  6. Granulomatosis with Polyangiitis Presenting as Pauci-Immune Crescentic Glomerulonephritis in Pregnancy

    Directory of Open Access Journals (Sweden)

    Ryan Kunjal

    2016-01-01

    Full Text Available Antineutrophil cytoplasmic antibody (ANCA associated vasculitis rarely affects females of reproductive age. A 28-year-old African American woman presented at 8 weeks of gestation with intractable vomiting attributed to hyperemesis gravidarum. She was found to have acute kidney injury that was unresponsive to vigorous fluid resuscitation and urine sediment examination was suggestive of an underlying glomerulonephritis. Serum c-ANCA and PR3 were elevated and there was no peripheral eosinophilia. During her course she also developed one episode of small volume hemoptysis with right upper lobe infiltrates on CT Chest. There were no cutaneous manifestations of vasculitis or upper respiratory symptoms. Renal biopsy revealed a pauci-immune crescentic glomerulonephritis (PICGN. The diagnosis was consistent with granulomatosis with polyangiitis (GPA. Management initially comprised teratogen sparing agents; steroids, intravenous immunoglobulin; and plasma exchange. The response was suboptimal and she became dependent on daily renal replacement therapy. Ultimately the pregnancy was terminated allowing for traditional treatment approaches with dramatic effect. This is the first case of GPA presenting as PICGN in pregnancy and highlights the challenges of its management.

  7. Goodpasture syndrome involving overlap with Wegener's granulomatosis and anti-glomerular basement membrane disease.

    Science.gov (United States)

    Kalluri, R; Meyers, K; Mogyorosi, A; Madaio, M P; Neilson, E G

    1997-11-01

    A 68-year-old Caucasian woman presented to the hospital with nodular pulmonary infiltrates and acute renal failure. Wegener's granulomatosis was initially considered to be most likely because of the presence of increased serum levels of c-anti-neutrophil cytoplasmic antibodies (c-ANCA). A consultation through the Internet after a renal biopsy demonstrated crescentic, necrotizing glomerulonephritis and linear deposits of immunoglobulin G (IgG) and complement C3, typical of anti-glomerular basement membrane (GBM) disease. Hemodialysis was instituted; however, the patient suddenly developed a massive cerebral hemorrhage and died before full therapy could take effect. Postmortem analysis of the patient's sera revealed high titers of IgG against the alpha 3 NC1 domain of type IV collagen. Serologic evidence of both p-ANCA and anti-GBM antibodies are becoming more frequently recognized in the setting of rapidly progressive glomerulonephritis. The patient reported here had the unusual combination of c-ANCA antibodies with anti-GBM disease, and this association raises complex questions regarding the pathogenesis of this type of renal injury.

  8. Granulomatosis with Polyangiitis Presenting as Pauci-Immune Crescentic Glomerulonephritis in Pregnancy

    Science.gov (United States)

    Kunjal, Ryan; Makary, Raafat; Poenariu, Andreea

    2016-01-01

    Antineutrophil cytoplasmic antibody (ANCA) associated vasculitis rarely affects females of reproductive age. A 28-year-old African American woman presented at 8 weeks of gestation with intractable vomiting attributed to hyperemesis gravidarum. She was found to have acute kidney injury that was unresponsive to vigorous fluid resuscitation and urine sediment examination was suggestive of an underlying glomerulonephritis. Serum c-ANCA and PR3 were elevated and there was no peripheral eosinophilia. During her course she also developed one episode of small volume hemoptysis with right upper lobe infiltrates on CT Chest. There were no cutaneous manifestations of vasculitis or upper respiratory symptoms. Renal biopsy revealed a pauci-immune crescentic glomerulonephritis (PICGN). The diagnosis was consistent with granulomatosis with polyangiitis (GPA). Management initially comprised teratogen sparing agents; steroids, intravenous immunoglobulin; and plasma exchange. The response was suboptimal and she became dependent on daily renal replacement therapy. Ultimately the pregnancy was terminated allowing for traditional treatment approaches with dramatic effect. This is the first case of GPA presenting as PICGN in pregnancy and highlights the challenges of its management. PMID:27293925

  9. Serum biomarkers in patients with relapsing eosinophilic granulomatosis with polyangiitis (Churg-Strauss.

    Directory of Open Access Journals (Sweden)

    Christian Dejaco

    Full Text Available Previous studies suggest a role for eotaxin-3, TARC/CCL17 and IgG4 in newly-diagnosed patients with eosinophilic granulomatosis with polyangiitis (EGPA, Churg-Strauss with highly active disease. The role of these biomarkers in relapsing disease is unclear.Serum levels of TARC/CCL17, eotaxin-3, IgG4, and IgG4/IgG ratio were determined in serum samples from a longitudinal cohort of patients with EGPA (105 visits of 25 patients. Epidemiological, clinical and laboratory data were available for all visits.At the first visit, 80% of patients were using glucocorticoids and 68% additional immunosuppressive drugs. Disease flares were seen at 18 visits. The median BVAS and BVAS/WG scores at time of relapse were 4 and 2, respectively. None of the biomarkers tested were useful to discriminate between active disease and remission. Patients treated with prednisone had lower eotaxin-3 and eosinophil levels compared to patients not taking glucocorticoids irrespective of disease activity. Use of immunosuppressive agents was not associated with biomarker levels.Serum levels of TARC/CCL17, eotaxin-3, IgG4, and IgG4/IgG ratio do not clearly differentiate active and inactive disease in established EGPA. Defining biomarkers in EGPA remains a challenge especially during times of glucocorticoid use.

  10. Prolonged risk of specific malignancies following cyclophosphamide therapy among patients with granulomatosis with polyangiitis

    DEFF Research Database (Denmark)

    Faurschou, Mikkel; Mellemkjaer, Lene; Voss, Anne

    2015-01-01

    -melanoma skin cancers (NMSCs) and 11 were bladder carcinomas. A high occurrence of NMSC was observed from the second year of follow-up onwards, with a SIR of 7.0 (95% CI 2.3, 16) for cases diagnosed ≥20 years after GPA. The incidence of bladder cancer increased after 5-9, 10-14 and 15-19 years of follow......OBJECTIVE: The long-term cancer risk for patients treated for granulomatosis with polyangiitis (GPA) is not well characterized. We assessed the risk of early and late-occurring cancers among 293 patients diagnosed with GPA from 1973 to 1999 and followed throughout 2010. METHODS: Cancer incidence...... in the cohort was determined by linkage with the Danish Cancer Registry and compared with that in the general population by calculation of standardized incidence ratios (SIRs). RESULTS: The median duration of follow-up was 9.7 years (range 0-36). Seventy-three cancers occurred, of which 30 were non...

  11. γδ T-cell-rich variants of pityriasis lichenoides and lymphomatoid papulosis: benign cutaneous disorders to be distinguished from aggressive cutaneous γδ T-cell lymphomas.

    Science.gov (United States)

    Martinez-Escala, M E; Sidiropoulos, M; Deonizio, J; Gerami, P; Kadin, M E; Guitart, J

    2015-02-01

    T cells with a γδ phenotype have been associated with aggressive lymphomas. Yet, inflammatory skin disorders and low-grade lymphoproliferative disorders have rarely been described with a predominant γδ T-cell infiltrate. To review our experience and determine the clinical relevance of the γδ T-cell phenotype in lymphomatoid papulosis (LyP) and pityriasis lichenoides (PL). A retrospective dermatopathology file review looking for LyP and PL characterized by a γδ T-cell phenotype was performed. Clinical manifestations and course, histological features and molecular data were analyzed. Six of 16 cases of LyP and four of 23 cases diagnosed as PL during a 5-year period (2009-14) were identified. The median follow-up for the whole group was 16 months (range 3-64), showing an indolent clinical course in all cases. The detection of a predominantly γδ T-cell phenotype in papular lymphoid-rich infiltrates in the absence of other lesions is not associated with a clinically aggressive course. γδ T-cell-rich variants of LyP and PL may reflect a spectrum of related conditions. This is a single academic centre retrospective chart review of a relatively small sample. © 2014 British Association of Dermatologists.

  12. Successful Use of Plasma Exchange in the Treatment of Corticosteroid-Refractory Eosinophilic Granulomatosis with Polyangiitis Associated with Gastrointestinal Manifestations

    Directory of Open Access Journals (Sweden)

    Kohei Tsujimoto

    2016-01-01

    Full Text Available We describe the case of a 33-year-old woman having corticosteroid-refractory eosinophilic granulomatosis with polyangiitis (EGPA who presented with abdominal pain and responded dramatically to plasma exchange. Eosinophilia, asthma history, neuropathy, pulmonary infiltrates, and paranasal sinus abnormalities confirmed the diagnosis of EGPA. Treatment was initiated with 1 g/day of methylprednisolone pulse therapy for 3 days followed by 60 mg/day of intravenous prednisolone without relieving abdominal pain. Then, plasma exchange was performed thrice. Abdominal pain disappeared after the first plasma exchange. Indication of plasma exchange for EGPA remains controversial; however, it may represent a valid option in cases with gastrointestinal involvement.

  13. Low-Dose Total Body Irradiation and Donor Peripheral Blood Stem Cell Transplant Followed by Donor Lymphocyte Infusion in Treating Patients With Non-Hodgkin Lymphoma, Chronic Lymphocytic Leukemia, or Multiple Myeloma

    Science.gov (United States)

    2015-10-30

    Adult Nasal Type Extranodal NK/T-cell Lymphoma; Anaplastic Large Cell Lymphoma; Angioimmunoblastic T-cell Lymphoma; Cutaneous B-cell Non-Hodgkin Lymphoma; Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Hepatosplenic T-cell Lymphoma; Intraocular Lymphoma; Nodal Marginal Zone B-cell Lymphoma; Noncutaneous Extranodal Lymphoma; Peripheral T-cell Lymphoma; Recurrent Adult Acute Lymphoblastic Leukemia; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Grade III Lymphomatoid Granulomatosis; Recurrent Adult Immunoblastic Large Cell Lymphoma; Recurrent Adult Lymphoblastic Lymphoma; Recurrent Adult T-cell Leukemia/Lymphoma; Recurrent Cutaneous T-cell Non-Hodgkin Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Mycosis Fungoides/Sezary Syndrome; Recurrent Small Lymphocytic Lymphoma; Refractory Chronic Lymphocytic Leukemia; Refractory Hairy Cell Leukemia; Refractory Multiple Myeloma; Small Intestine Lymphoma; Splenic Marginal Zone Lymphoma; Stage II Multiple Myeloma; Stage III Multiple Myeloma; Testicular Lymphoma; Waldenström Macroglobulinemia

  14. Monoclonal Antibody Therapy Before Stem Cell Transplant in Treating Patients With Relapsed or Refractory Lymphoid Malignancies

    Science.gov (United States)

    2015-12-07

    Adult Nasal Type Extranodal NK/T-cell Lymphoma; Anaplastic Large Cell Lymphoma; Angioimmunoblastic T-cell Lymphoma; Cutaneous B-cell Non-Hodgkin Lymphoma; Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Hepatosplenic T-cell Lymphoma; Intraocular Lymphoma; Nodal Marginal Zone B-cell Lymphoma; Noncutaneous Extranodal Lymphoma; Peripheral T-cell Lymphoma; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Grade III Lymphomatoid Granulomatosis; Recurrent Adult Hodgkin Lymphoma; Recurrent Adult Immunoblastic Large Cell Lymphoma; Recurrent Adult Lymphoblastic Lymphoma; Recurrent Adult T-cell Leukemia/Lymphoma; Recurrent Cutaneous T-cell Non-Hodgkin Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Mycosis Fungoides/Sezary Syndrome; Recurrent Small Lymphocytic Lymphoma; Refractory Hairy Cell Leukemia; Small Intestine Lymphoma; Splenic Marginal Zone Lymphoma; T-cell Large Granular Lymphocyte Leukemia; Testicular Lymphoma; Waldenström Macroglobulinemia

  15. Genetically Modified Peripheral Blood Stem Cell Transplant in Treating Patients With HIV-Associated Non-Hodgkin or Hodgkin Lymphoma

    Science.gov (United States)

    2015-05-06

    Adult Nasal Type Extranodal NK/T-cell Lymphoma; AIDS-related Diffuse Large Cell Lymphoma; AIDS-related Diffuse Mixed Cell Lymphoma; AIDS-related Diffuse Small Cleaved Cell Lymphoma; AIDS-related Immunoblastic Large Cell Lymphoma; AIDS-related Lymphoblastic Lymphoma; AIDS-related Peripheral/Systemic Lymphoma; AIDS-related Small Noncleaved Cell Lymphoma; Anaplastic Large Cell Lymphoma; Angioimmunoblastic T-cell Lymphoma; Cutaneous B-cell Non-Hodgkin Lymphoma; Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Hepatosplenic T-cell Lymphoma; HIV-associated Hodgkin Lymphoma; Intraocular Lymphoma; Nodal Marginal Zone B-cell Lymphoma; Noncutaneous Extranodal Lymphoma; Peripheral T-cell Lymphoma; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Grade III Lymphomatoid Granulomatosis; Recurrent Adult Hodgkin Lymphoma; Recurrent Adult Immunoblastic Large Cell Lymphoma; Recurrent Adult Lymphoblastic Lymphoma; Recurrent Adult T-cell Leukemia/Lymphoma; Recurrent Cutaneous T-cell Non-Hodgkin Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Mycosis Fungoides/Sezary Syndrome; Recurrent Small Lymphocytic Lymphoma; Refractory Hairy Cell Leukemia; Small Intestine Lymphoma; Splenic Marginal Zone Lymphoma; Stage I AIDS-related Lymphoma; Stage II AIDS-related Lymphoma; Stage III AIDS-related Lymphoma; Stage IV AIDS-related Lymphoma; T-cell Large Granular Lymphocyte Leukemia; Testicular Lymphoma; Waldenström Macroglobulinemia

  16. Results of endoscopic surgery and intralesional steroid therapy for airway compromise due to tracheobronchial Wegener's granulomatosis

    Directory of Open Access Journals (Sweden)

    S. A. R. Nouraei

    2008-04-01

    Full Text Available Background: Upper airway compromise due to tracheobronchial stenosis commonly occurs in patients with Wegener's Granulomatosis (WG. There is at present no consensus on the optimal management of this life-threatening condition. Objective: To assess the results of laryngo-tracheo-bronchoscopy, intralesional steroid therapy, laser surgery, and dilatation in managing obstructive tracheobronchial WG. Methods: Records of eighteen previously-untreated stridulous patients with obstructive tracheobronchial WG, treated between 2004 and 2006 were prospectively recorded on an airway database and retrospectively reviewed. Information about patient and lesion characteristics and treatment details were recorded. Treatment progress was illustrated using a timeline plot, and intervention-free intervals were calculated with actuarial analysis. Results: There were nine males and the average age at presentation was 40 (16 years [range 13–74]. There were thirteen patients with tracheal, and five patients with tracheal and bronchial lesions. The average tracheal lesion height was 8 (3 mm, located 23 (9 mm below the glottis. There were 1, 10 and 7 Myer-Cotton grade I, II and III lesions respectively. Mean intervention-free interval following minimally-invasive treatment was 26 (2.8 months. Following endobronchial therapy the median intervention-free interval was 22 months (p>0.8 vs. tracheal lesions. No patient required a tracheostomy or endoluminal stenting. Conclusions: Intralesional steroid therapy and conservative endoluminal surgery is an effective strategy for treating airway compromise due to active tracheal and bronchial WG, obviating the need for airway bypass or stenting. We recommend the combination of endotracheal dilatation, conservative laser surgery and steroid therapy as the standard of care for treating airway compromise due to obstructive tracheobronchial WG.

  17. Granulomatosis de Wegener Semejando Cáncer Epidermoide de Nasofaringe y Metástasis Pulmonares.

    Directory of Open Access Journals (Sweden)

    Gustavo Adolfo Martín Small

    2009-01-01

    Full Text Available La granulomatosis de Wegener (GW es una enfermedad idiopática, con posible componente autoinmune, que aparece generalmente en la quinta década de vida, caracterizándose por lesiones granulomatosas necrotizantes y vasculitis en vías aéreas y riñón. Paciente femenino de 54 años, quien desde Noviembre del 2008, presenta rinorrea, prurito y eritema en borde nasal inferior izquierdo, recibe antibióticos sin mejoría de los síntomas. En las radiografías torácicas, se observan dos radiopacidades redondeadas, de 4 cm de diámetro, sugestivas de lesiones tumorales en ambos campos pulmonares. La biopsia reporta cáncer epidermoide de alto grado, sospechándose primario en nasofaringe. Es remitida, por deterioro de condiciones, al Servicio de Neumonología del Hospital Universitario de Caracas el 18/03/2009, presentando disnea, tos productiva, placas purpúricas dolorosas en manos y pies, hipoacusia, hiperpigmentación del paladar duro, leucoplaquias y lesiones costrosas en lengua, insuficiencia renal (creatinina en 6,11 mg/dL y trombocitopenia. En TAC de tórax, se evidencian tumores mayores de 5 cm de diámetro, algunos con bordes bien definidos y otros mal delimitados con broncograma aéreo. Diagnostico definitivo de GW por serología. La GW con su afectación sistémica puede simular otras entidades como neoplasias de cabeza y cuello con metástasis pulmonares. Cuando las manifestaciones sistémicas son floridas debe sospecharse GW como diagnóstico diferencial.

  18. Genetics and pathophysiology of granulomatosis with polyangiitis (GPA) and its main autoantigen proteinase 3.

    Science.gov (United States)

    Relle, Manfred; Föhr, Bernd; Fasola, Federica; Schwarting, Andreas

    2016-12-01

    Granulomatosis with polyangiitis (GPA) is a severe autoimmune disease and one of the small vessel anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides. Although its etiology and pathophysiology are still widely unknown, it is accepted that infections, environmental factors, epigenetic modifications, and a genetic predisposition provide the basis for this systemic disorder. GPA typically evolves into two phases: an initial phase characterized by ear, nose and throat (ENT) manifestations, such as chronic sinusitis and otitis, ulceration of the oral cavity and pharynx, as well as pulmonary nodules and a severe generalized phase, defined by the occurrence of rapidly progressive glomerulonephritis, pulmonary hemorrhage, and arthritis. ANCAs, directed against the neutrophilic enzymes proteinase 3 and myeloperoxidase, are present in up to 90% of the affected patients in the systemic phase. As the humoral immunity is predominantly directed against neutrophilic antigens, it is apparent that neutrophils play a critical role in GPA both as target and effector cells. Although GPA pathogenesis is not well known, some susceptibility genes and loci have been identified by candidate gene approaches, genome-wide association studies, and meta-analyses, as well as familial association studies. Such genes are CTLA4, PTPN22, COL11A2, SERPINA1, and the MHC class II gene cluster. This review highlights the clinical, pathophysiological, and genetic background of GPA and aims to give an overview of recent efforts to identify GPA susceptibility genes. We point out the genetic basis of the main autoantigen PR3 and why it is so difficult to establish a murine GPA model. Copyright © 2016 Elsevier Ltd. All rights reserved.

  19. IgA antibodies to myeloperoxidase in patients with eosinophilic granulomatosis with polyangiitis (Churg-Strauss).

    Science.gov (United States)

    Oommen, Esha; Hummel, Amber; Allmannsberger, Lisa; Cuthbertson, David; Carette, Simon; Pagnoux, Christian; Hoffman, Gary S; Jenne, Dieter E; Khalidi, Nader A; Koening, Curry L; Langford, Carol A; McAlear, Carol A; Moreland, Larry; Seo, Philip; Sreih, Antoine; Ytterberg, Steven R; Merkel, Peter A; Specks, Ulrich; Monach, Paul A

    2017-01-01

    To determine the prevalence of anti-myeloperoxidase (MPO) antibodies of IgA (IgA anti-MPO) isotype in patients with eosinophilic granulomatosis with polyangiitis (EGPA), and the association of the IgA antibodies with IgG anti-MPO and with disease activity. Serum samples from patients with EGPA followed in a multicenter longitudinal cohort were tested by ELISA for the presence of IgA anti-MPO and IgG anti-MPO antibodies. Sera from 87 healthy controls were used to define a positive test. Sera from 168 patients with EGPA (298 samples) were tested. Frequencies of positive testing for IgA anti-MPO were compared between patients with active EGPA, patients in remission, and controls. IgA anti-MPO was detected in 10 of 168 (6%) patients with EGPA (11 of 298 serum samples) compared to 1 of 87 (1%) healthy controls (p=0.10). All 11 samples testing positive for IgA anti-MPO also tested positive for IgG anti-MPO. Ninety samples tested positive for IgG anti-MPO but negative for IgA. Samples taken during active EGPA were positive for IgA anti-MPO in 6/72 cases (8%), compared to 5/226 (2%) during remission (p=0.03). Among samples taken during moderate or high disease activity, 5/41 were positive (12%, p=0.01 compared to remission). Although IgA anti-MPO antibodies are detectable in some patients with EGPA and may be detectable more frequently during active disease, their presence seems unlikely to provide information beyond what is obtained from conventional IgG anti-MPO.

  20. Azathioprine is effective for oral involvement in Crohn's disease but not for orofacial granulomatosis alone.

    Science.gov (United States)

    Mentzer, A; Goel, R; Elliott, T; Campbell, H; Hullah, E; Patel, P; Challacombe, S; Escudier, M; Sanderson, J D

    2016-04-01

    There have been no previous reports assessing the effectiveness of azathioprine (AZA) in the treatment of orofacial granulomatosis (OFG). This report is a review of patients receiving AZA for active OFG with or without concomitant gut Crohn's disease (CD) in a specialist tertiary referral centre. Clinical response was defined by Global Physician Assessment at 4-, 12- and 24-month follow-up and a standardised oral disease activity score (ODAS). Sixty of 215 patients seen with OFG in our clinic over a 12-year period were treated with AZA. Of these, 22 had concomitant CD. The proportion of patients responding to AZA with a diagnosis of CD/OFG vs. OFG only at 4, 12 and 24 months were 54% vs. 21% (P = 0.03), 59% vs. 21% (P = 0.003) and 41% vs. 24% (P = 0.16), respectively. A statistically significant difference was seen between starting and follow-up ODAS scores at 4 months in the CD/OFG group which was not observed in the OFG only group. Factors predicting a need for AZA included a diagnosis of intestinal CD, sulcal swelling, sulcal ulcers and upper lip involvement. The factor predicting response to treatment was a diagnosis of CD at 12 months of follow-up. No difference in the number of adverse effects was observed between the two groups of patients. AZA is significantly more effective in the treatment of oral disease with a concurrent diagnosis of CD rather than in the treatment of OFG alone. © 2015 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  1. Lenalidomide With or Without Rituximab in Treating Patients With Progressive or Relapsed Chronic Lymphocytic Leukemia, Small Lymphocytic Lymphoma, Prolymphocytic Leukemia, or Non-Hodgkin Lymphoma Previously Treated With Donor Stem Cell Transplant

    Science.gov (United States)

    2017-07-24

    Adult Nasal Type Extranodal NK/T-cell Lymphoma; Anaplastic Large Cell Lymphoma; Angioimmunoblastic T-cell Lymphoma; Cutaneous B-cell Non-Hodgkin Lymphoma; Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Hepatosplenic T-cell Lymphoma; Intraocular Lymphoma; Nodal Marginal Zone B-cell Lymphoma; Noncutaneous Extranodal Lymphoma; Peripheral T-cell Lymphoma; Prolymphocytic Leukemia; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Grade III Lymphomatoid Granulomatosis; Recurrent Adult Immunoblastic Large Cell Lymphoma; Recurrent Adult Lymphoblastic Lymphoma; Recurrent Adult T-cell Leukemia/Lymphoma; Recurrent Cutaneous T-cell Non-Hodgkin Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Mycosis Fungoides/Sezary Syndrome; Recurrent Small Lymphocytic Lymphoma; Refractory Chronic Lymphocytic Leukemia; Refractory Hairy Cell Leukemia; Small Intestine Lymphoma; Splenic Marginal Zone Lymphoma; T-cell Large Granular Lymphocyte Leukemia; Testicular Lymphoma; Waldenström Macroglobulinemia

  2. Fuzzy recurrence plots

    Science.gov (United States)

    Pham, T. D.

    2016-12-01

    Recurrence plots display binary texture of time series from dynamical systems with single dots and line structures. Using fuzzy recurrence plots, recurrences of the phase-space states can be visualized as grayscale texture, which is more informative for pattern analysis. The proposed method replaces the crucial similarity threshold required by symmetrical recurrence plots with the number of cluster centers, where the estimate of the latter parameter is less critical than the estimate of the former.

  3. Evaluation of capture ELISA for detection of antineutrophil cytoplasmic antibodies directed against proteinase 3 in Wegener's granulomatosis : first results from a multicentre study

    NARCIS (Netherlands)

    Csernok, E; Holle, J; Hellmich, B; Willem, J; Tervaert, C; Kallenberg, CGM; Limburg, PC; Niles, J; Pan, GL; Specks, U; Westman, K; Wieslander, J; Gross, WL

    2004-01-01

    Objective: To evaluate the performance characteristics of direct and capture ELISA for the detection of PR3-ANCA in Wegener's granulomatosis (WG) in international ANCA reference laboratories. Methods: Serum samples were derived from patients with histological and clinical diagnosis of WG (n = 60), r

  4. RELEVANCE OF CLASSIC ANTINEUTROPHIL CYTOPLASMIC AUTOANTIBODY (C-ANCA)-MEDIATED INHIBITION OF PROTEINASE 3-ALPHA-1-ANTITRYPSIN COMPLEXATION TO DISEASE-ACTIVITY IN WEGENER-GRANULOMATOSIS

    NARCIS (Netherlands)

    DOLMAN, KM; STEGEMAN, CA; VANDEWIEL, BA; HACK, CE; BORNE, AEGKV; KALLENBERG, CGM; GOLDSCHMEDING, R

    1993-01-01

    In the sera of patients with Wegener's granulomatosis (WG), C-ANCA can be detected that are directed against proteinase 3 (PR3). We have previously observed that C-ANCA interfere with PR3 proteolytic activity and with complexation of PR3 with its major physiologic inhibitor, alpha1-antitrypsin (alph

  5. The prevalence of the Staphylococcus aureus tst gene among community- and hospital-acquired strains and isolates from Wegener's Granulomatosis patients

    NARCIS (Netherlands)

    Deurenberg, Ruud H; Nieuwenhuis, Rutger F; Driessen, Christel; London, Nancy; Stassen, Frank R; van Tiel, Frank H; Stobberingh, Ellen E; Vink, Cornelis

    2005-01-01

    To allow rapid identification of toxic shock syndrome toxin-1 (TSST-1)-producing Staphylococcus aureus strains, a real-time PCR assay for the detection of the tst gene, which encodes TSST-1, was developed. The assay was applied to S. aureus isolates from patients with Wegener's Granulomatosis (WG),

  6. PREDOMINANCE OF IGG1 AND IGG4 SUBCLASSES OF ANTINEUTROPHIL CYTOPLASMIC AUTOANTIBODIES (ANCA) IN PATIENTS WITH WEGENERS GRANULOMATOSIS AND CLINICALLY RELATED DISORDERS

    NARCIS (Netherlands)

    BROUWER, E.; Tervaert, J.W.C.; HUITEMA, M.G.; VANDERGIESSEN, M.; Limburg, Piet; Kallenberg, Cees; Horst, G.

    1991-01-01

    In view of the supposed hypersensitivity, the elevated levels of IgE, and the occurrence of eosinophilia reported in Wegener's granulomatosis and related conditions, we studied the IgG subclass distribution of ANCA directed against a 29-kD serine protease and and myeloperoxidase (MPO) in 41 untreate

  7. A Diagnostic Approach to Recurrent Orofacial Swelling: A Retrospective Study of 104 Patients.

    Science.gov (United States)

    Miest, Rachel Y; Bruce, Alison J; Comfere, Nneka I; Hadjicharalambous, Elena; Endly, Dawnielle; Lohse, Christine M; Rogers, Roy S

    2017-07-01

    To identify patients evaluated in an outpatient setting at our institution with a presentation of recurrent orofacial swelling and to review the spectrum of causes to outline a diagnostic approach. A retrospective study of 104 patients with more than 1 episode of orofacial swelling lasting for more than 5 days identified through a keyword search of the electronic health record from January 2, 2000, through July 5, 2011. Patients were categorized according to final cause of orofacial swelling: idiopathic orofacial granulomatosis, solid facial edema due to rosacea and acne vulgaris, Crohn disease, contact dermatitis, sarcoidosis, exfoliative cheilitis, lichen planus, actinic cheilitis, cheilitis glandularis, lymphedema, miscellaneous, and multifactorial. Granulomatous inflammation was noted on biopsy in 40 of 85 patients (47%). Oral involvement was associated with Crohn disease (Porofacial swelling underscores the diagnostic challenge and importance of a thorough multidisciplinary evaluation to identify underlying causes. Copyright © 2017 Mayo Foundation for Medical Education and Research. Published by Elsevier Inc. All rights reserved.

  8. Recurrent zosteriform herpes simplex

    Directory of Open Access Journals (Sweden)

    Inamadar Arun

    1992-01-01

    Full Text Available A 25-year-old man had recurrent zosteriform herpes simplex for past 6 years. The attacks were precipitated by prolonged exposure to sunlight. Pain was mild and lesions used to subside each time in about 7 days. Clinical features which help in differentiating recurrent herpes simplex from recurrent herpes zoster are summarized.

  9. Necrotizing RPGN with linear anti IgG deposits in a patient with history of granulomatosis with polyangiitis: a case report

    Directory of Open Access Journals (Sweden)

    Parekh N

    2014-11-01

    Full Text Available Ninad Parekh, Edward Epstein, Suzanne El-Sayegh Department of Medicine, Division of Nephrology, Staten Island University Hospital, Staten Island, NY, USA Introduction: Diagnosing the etiology of a rapidly progressive glomerulonephritis is of vital importance to guide appropriate therapeutic management. This case highlights the complexity involved in establishing diagnosis when presentation is atypical. In certain cases diagnosis cannot be established based on clinical presentation or biopsy findings alone, and critical analysis of biopsy findings in context of clinical presentation is crucial to guide the clinical decision-making process.Case presentation: A 47-year-old Hispanic male with history of granulomatosis with polyangiitis (GPA in remission on azathioprine, presented with fatigue and lethargy. Physical examination was unremarkable. Laboratory data revealed elevated creatinine and otherwise normal electrolytes. Urinalysis showed numerous dysmorphic red blood cells with few red cell casts. His serologic results were all negative except anti-proteinase-3 antibody at very low titers. Kidney biopsy showed necrotizing crescentic glomerulonephritis with linear immunoglobulin G staining along the basement membrane.Conclusion: This case presented conflicting serologic and histopathologic findings. The presence of anti-proteinase-3 antibody supported diagnosis of recurrence of GPA. However, linear staining of immunoglobulin G (IgG on immunofluorescence (IF staining of renal biopsy supported anti-glomerular basement membrane (GBM disease. The treatment of anti-GBM disease and GPA both involve immunosuppression with prednisone and cyclophosphamide. However, patients with anti-GBM disease are also treated with plasmapheresis early in the disease presentation to prevent further damage. The patient with GPA, on the other hand, was shown to benefit from plasmapheresis only in the case of severe renal disease (serum creatinine level more than 5 mg/dL or

  10. EULAR/PRINTO/PRES criteria for Henoch-Schönlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part II: Final classification criteria

    DEFF Research Database (Denmark)

    Ozen, Seza; Pistorio, Angela; Iusan, Silvia M

    2010-01-01

    To validate the previously proposed classification criteria for Henoch-Schönlein purpura (HSP), childhood polyarteritis nodosa (c-PAN), c-Wegener granulomatosis (c-WG) and c-Takayasu arteritis (c-TA)....

  11. Aortic {sup 18}F-FDG uptake in patients suffering from granulomatosis with polyangiitis

    Energy Technology Data Exchange (ETDEWEB)

    Kemna, Michael J. [Maastricht University Medical Center, Department of Nephrology and Clinical Immunology, Maastricht (Netherlands); Maastricht University, Cardiovascular Research Institute Maastricht (CARIM), Maastricht (Netherlands); Bucerius, Jan [Maastricht University, Cardiovascular Research Institute Maastricht (CARIM), Maastricht (Netherlands); Maastricht University Medical Center, Department of Nuclear Medicine, Maastricht (Netherlands); University Hospital RWTH Aachen, Department of Nuclear Medicine, Aachen (Germany); Drent, Marjolein [Maastricht University, Department of Pharmacology and Toxicology, Maastricht (Netherlands); Voeoe, Stefan [Maastricht University, Cardiovascular Research Institute Maastricht (CARIM), Maastricht (Netherlands); Maastricht University Medical Center, Department of Nuclear Medicine, Maastricht (Netherlands); Veenman, Martine [Maastricht University, Cardiovascular Research Institute Maastricht (CARIM), Maastricht (Netherlands); Paassen, Pieter van [Maastricht University Medical Center, Department of Nephrology and Clinical Immunology, Maastricht (Netherlands); Tervaert, Jan Willem Cohen [Maastricht University, Cardiovascular Research Institute Maastricht (CARIM), Maastricht (Netherlands); Sint Franciscus Gasthuis, Noordoever Academy, Rotterdam (Netherlands); Kroonenburgh, Marinus J.P.G. van [Maastricht University Medical Center, Department of Nuclear Medicine, Maastricht (Netherlands)

    2015-08-15

    The objective of the study was to systematically assess aortic inflammation in patients with granulomatosis with polyangiitis (GPA) using {sup 18}F-2-deoxy-2-[{sup 18}F]fluoro-D-glucose (FDG) positron emission tomography (PET)/CT. Aortic inflammation was studied in PET/CT scans obtained from 21 patients with GPA; 14 patients with sarcoidosis were included as disease controls, 7 patients with stage I or II head and neck carcinoma ascertained during routine clinical practice were used as healthy controls (HC) and 5 patients with large vessel vasculitis (LVV) were used as positive controls. Aortic {sup 18}F-FDG uptake was expressed as the blood-normalized maximum standardized uptake value (SUV{sub max}), known as the target to background ratio (mean TBR{sub max}). The mean TBR{sub max} (interquartile range) of the aorta in patients with GPA, sarcoidosis, HC and LVV were 1.75 (1.32-2.05), 1.62 (1.54-1.74), 1.29 (1.22-1.52) and 2.03 (1.67-2.45), respectively. The mean TBR{sub max} was significantly higher in patients suffering from GPA or LVV compared to HC (p < 0.05 and p < 0.005, respectively) and tended to be higher in patients suffering from sarcoidosis, but this did not reach statistical significance (p = 0.098). The mean TBR{sub max} of the most diseased segment was significantly higher compared to HC [1.57 (1.39-1.81)] in LVV patients [2.55 (2.22-2.82), p < 0.005], GPA patients [2.17 (1.89-2.83), p < 0.005] and patients suffering from sarcoidosis [2.04 (1.88-2.20), p < 0.05]. In GPA patients, the mean TBR{sub max} of the aorta was significantly higher in patients with previous renal involvement [2.01 (1.69-2.53)] compared to patients without renal involvement in the past [1.60 (1.51-1.80), p < 0.05]. Interrater reproducibility with a second reader was high (all intraclass correlation coefficients >0.9). Patients suffering from GPA show marked aortic FDG uptake. (orig.)

  12. [A case of rapidly progressive glomerulonephritis in the course of Wegener's granulomatosis].

    Science.gov (United States)

    Idasiak-Piechocka, I; Oko, A; Łochyńska, K; Woźniak, A; Czekalski, S

    2000-01-01

    Wegener's granulomatosis (WG) is characterized by granulomatous vasculitis of the respiratory tract and glomerulonephritis (GN). Prognosis of this disease is poor and about 20% of untreated patients die after one year from the onset. WG was recognized in 45-year-old patient on the basis of: 1) clinical symptoms (joint pain and swollen, purpura on the skin which appeared one week after respiratory tract infection, ulceration of the tonsils and lingula), 2) results of additional testing (X-chest-ray-infiltrates of both lungs), positive results of the cANCA (titre 1:640) and rapidly progressive renal failure [the increase of serum creatinine level (Pcr) from 123.7 to 707 mumol/l (1.4 to 8.0 mg/dl) during one week]. Renal biopsy revealed extracapillary GN (cellular crescents in 7 out of 8 glomeruli and scattered foci of fibrinoid necrosis of capillary walls in all). At the beginning of the treatment Pcr raised to 884 mumol/l (10 mg/dl) and the patient required hemodialysis. He was treated with methylprednisolone (M) at flash doses of 1000 mg/24 h by three days followed by 125 mg/24 h i.v.--because of peptic ulcer, with cyclophosphamide (C-150 mg/24 h p.p.), with trimetoprim/sulphametoxazole, with pentoxifylline and omeprazol. After six weeks of the treatment in the control kidney biopsy sclerotic changes in 10 out of 13 glomeruli and diffuse interstitial fibrosis were found. However, during the same time, we observed clinical remission of the disease and the decrease of Pcr to 176.8 mumol/l (2 mg/dl). The M dosis was reduced by 5 mg every weeks and the C dosis--to 50 mg (because of the increase of aminotransferase levels) After six months of the treatment Pcr was 132.6 mumol/l (1.5 mg/dl) and CANCA titer was 1:16. In this case of RPGN, despite off the progression of the morphological changes in the kidney, we obtained the clinical remission of the disease and significant decrease of Pcr level. These results suggest that aggressive treatment of WG is justified even in

  13. MicroRNA-941 Expression in Polymorphonuclear Granulocytes Is Not Related to Granulomatosis with Polyangiitis

    Science.gov (United States)

    Svendsen, Jesper Brink; Baslund, Bo; Cramer, Elisabeth Præstekjær; Rapin, Nicolas; Borregaard, Niels

    2016-01-01

    Jumonji Domain-Containing Protein 3 (JMJD3)/lysine demethylase 6B (KDM6B) is an epigenetic modulator that removes repressive histone marks on genes. Expression of KDM6B mRNA is elevated in leukocytes from patients with ANCA-associated vasculitis (AAV) and has been suggested to be the reason for higher proteinase 3 (PR3) mRNA expression in these cells due to derepression of PRTN3 gene transcription. MicroRNA-941 (miR-941) has been shown to target KDM6B mRNA and inhibit JMJD3 production. We therefore investigated whether polymorphonuclear granulocytes (PMNs) from patients suffering from granulomatosis with polyangiitis (GPA) have lower expression of miR-941 than healthy control donors as a biological cause for higher JMJD3 levels. We found no significant difference in the degree of maturation of PMNs from GPA patients (n = 8) and healthy controls (n = 11) as determined from cell surface expression of the neutrophil maturation marker CD16 and gene expression profile of FCGR3B. The expression of PRTN3 and KDM6B mRNAs and miR-941 was not significantly different in GPA patients and healthy controls. Transfection of pre-miR-941 into the neutrophil promyelocyte cell line PLB-985 cells did not result in reduction of the KDM6B mRNA level as shown previously in a hepatocellular carcinoma cell line. The amount of PR3 in PMNs from GPA patients and healthy controls was comparable. In conclusion, we found that PRTN3 mRNA, KDM6B mRNA, and miR-941 expression levels in PMNs do not differ between GPA patients and healthy controls, and that miR-941 does not uniformly regulate KDM6B mRNA levels by inducing degradation of the transcript. Thus, decreased miR-941 expression in PMNs cannot be part of the pathogenesis of GPA. PMID:27755585

  14. Association between orofacial granulomatosis and Crohn's disease in children: systematic review.

    Science.gov (United States)

    Lazzerini, Marzia; Bramuzzo, Matteo; Ventura, Alessandro

    2014-06-21

    To review pediatric cases of orofacial granulomatosis (OFG), report disease characteristics, and explore the association between OFG and Crohn's disease. We conducted a systematic review according to the PRISMA guidelines. We searched Medline, LILACS, Virtual Health Library, and Web of Knowledge in September 2013 for cases of OFG in the pediatric age range (< 18 years), with no language limitations. All relevant articles were accessed in full text. The manual search included references of retrieved articles. We extracted data on patients' characteristics, disease characteristics, association with other diseases, and treatment. We analyzed the data and reported the results in tables and text. We retrieved 173 reports of OFG in children. Mean age at onset was 11.1 ± 3.8 years (range: 2.0-18 years). Prevalence in males was significant higher than in females (P < 0.001), with a male:female ratio of 2:1. Gastrointestinal signs or symptoms were present in 26.0% of children at the time of OFG diagnosis. Overall, 70/173 (40.4%) children received a concomitant diagnosis of Crohn's disease. In about half (51.4%) of the cases the onset of OFG anticipated the diagnosis of Crohn's disease, with a mean time between the two diagnoses of 13.1 ± 11.6 mo (range: 3-36 mo). Overall, 21/173 (12.1%) of the children with OFG had perianal disease, while 11/173 (6.4%) had a family history of Crohn's disease. Both perianal disease and a family history of Crohn's disease were significantly associated with a higher risk of Crohn's disease diagnosis in children with OFG [relative risk (RR) = 3.10, 95% confidence interval (CI): 2.46-3.90; RR = 2.74, 95%CI: 2.24-3.36, P < 0.0001 for both). Treatment of OFG included steroids (70.8% of children) and other immunosuppressive drugs (42.7%), such as azathioprine, thalidomide and infliximab. High prevalence of Crohn's disease in children with OFG suggests that OFG may be a subtype of Crohn's disease.

  15. Late recurrence of medulloblastoma.

    Science.gov (United States)

    Stevens, Brittney; Razzaqi, Faisal; Yu, Lolie; Craver, Randall

    2008-01-01

    We present a child with a cerebellar medulloblastoma, diagnosed at age three, treated with near total surgical resection, radiotherapy, and chemotherapy, that recurred 13 years after the initial diagnosis. This late recurrence exceeds the typical 10-year survival statistics that are in common use, and exceeds the Collins rule. Continued follow-up of these children is justified to increase the likelihood of detecting these late recurrences early and to learn more about these late recurrences.

  16. Recurrent intracerebral hemorrhage

    Institute of Scientific and Technical Information of China (English)

    Shen jinsong; Lu jianhong

    2000-01-01

    Objective: In order to study the clinical manifestation and risk factor of recurrent intracerebral hemorrhage(ICH).Methods:The 256 patients were analysed who admitted to our hospital for intracerebral hemorrhage between 1995 and 1997.The 15(5 .86%)patients had a recurrent ICH.There were 9 men and 6 women and the mean age of the patients was 63.5 ± 6.4years at the first bleeding episode and 67.8± 8. 5 years at the second. The mean interval between the two bleeding episodes was 44.6 ± 12.5 months. The 73.3%patients were hypertensive .′The site of the first hemorrhage was ganglionic in 8 patients , ]ohar in six paients and brainstem in one .The recurrent hemorrhage occurred at a different location from the previous ICH.The most common pattern of recurrence was “ganglionic -ganglionic” (7 patients), lobar - ganglionic (3 patients), lobar-lobar(three patients), which was always observed in hypertensive patients. The outcome after the recurrent hemorrhage was usually poor. By comparison with 24 patients followed up to average 47.5± 18.7 months with isolated ICH without recurrence .Only lobar hematoma and a younger age were risk factors for recurrences whereas sex and previous hypertension were not. The mechanism of recurrence of ICH were multiple(hypertension, cerebral amyloid angiopathy).Contral of blood pressure and good living habit after the first hemorrhage may prevent ICH recurrences.

  17. Optimal Recurrence Grammars

    CERN Document Server

    Graben, Peter beim; Fröhlich, Flavio

    2015-01-01

    We optimally estimate the recurrence structure of a multivariate time series by Markov chains obtained from recurrence grammars. The goodness of fit is assessed with a utility function derived from the stochastic Markov transition matrix. It assumes a local maximum for the distance threshold of the optimal recurrence grammar. We validate our approach by means of the nonlinear Lorenz system and its linearized stochastic surrogates. Finally we apply our optimization procedure to the segmentation of neurophysiological time series obtained from anesthetized animals. We propose the number of optimal recurrence domains as a statistic for classifying an animals' state of consciousness.

  18. High risk of pulmonary embolism and deep venous thrombosis but not of stroke in granulomatosis with polyangiitis (Wegener's)

    DEFF Research Database (Denmark)

    Faurschou, Mikkel; Obel, Niels; Baslund, Bo

    2014-01-01

    OBJECTIVE: To assess the incidence of stroke, pulmonary embolism (PE), and deep venous thrombosis (DVT) in granulomatosis with polyangiitis (Wegener's) (GPA). METHODS: Patients diagnosed with GPA at a Danish tertiary care center during 1993-2011 were identified (n = 180). Each patient was matched...... with 19 population controls (n = 3,420). Information on hospitalizations for stroke, PE, and DVT was obtained from the Danish National Hospital Register. The occurrence of vascular events in the GPA cohort was compared with that in the control group by calculation of incidence rate ratios (IRRs). RESULTS......: The median duration of followup was 7.2 years (interquartile range 3.1-11.7 years) in the GPA cohort. Within the first 2 years following the diagnosis of vasculitis, the incidences of PE and DVT were substantially increased among the patients (IRR 25.7 [95% confidence interval (95% CI) 6.9-96] for PE and IRR...

  19. Comparing Presenting Clinical Features in 48 Children With Microscopic Polyangiitis to 183 Children Who Have Granulomatosis With Polyangiitis (Wegener's)

    DEFF Research Database (Denmark)

    Cabral, David A; Canter, Debra L; Muscal, Eyal

    2016-01-01

    OBJECTIVE: To uniquely classify children with microscopic polyangiitis (MPA), to describe their demographic characteristics, presenting clinical features, and initial treatments in comparison to patients with granulomatosis with polyangiitis (Wegener's) (GPA). METHODS: The European Medicines Agency...... with GPA, whose diagnoses had been classified according to both adult- and pediatric-specific criteria. Descriptive statistics were used for comparisons. RESULTS: In total, 231 of 440 patients (64% female) fulfilled the classification criteria for either MPA (n = 48) or GPA (n = 183). The median time...... to diagnosis was 1.6 months in the MPA group and 2.1 months in the GPA group (ranging to 39 and 73 months, respectively). Patients with MPA were significantly younger than those with GPA (median age 11 years versus 14 years). Constitutional features were equally common between the groups. In patients with MPA...

  20. Tumour necrosis factor-alpha (TNF), lymphotoxin and TNF receptor levels in serum from patients with Wegener's granulomatosis

    DEFF Research Database (Denmark)

    Jónasdóttir, O; Petersen, J; Bendtzen, K

    2001-01-01

    -R), and these receptors are often found in soluble forms (sTNF-R), which can modulate TNFalpha actions. To evaluate the clinical importance of the TNF family of cytokines, the serum levels of TNFalpha, TNFbeta, now termed lymphotoxin (LTalpha), and sTNF-R1 and sTNF-R2 were measured by ELISA in 8 patients with WG during......Wegener's granulomatosis (WG) is a systemic inflammatory disease with vasculitis as the key feature. Abnormal expression of tumour necrosis factor alpha (TNFalpha) is considered of prime pathogenic importance in several inflammatory diseases. The effects of TNFa are mediated by TNF receptors (TNF...... of the relative amounts of TNFalpha and sTNF-R indicated that TNFalpha was mostly bound to its soluble receptors. In WG, the serum levels of sTNF-R1 and sTNF-R2 were dramatically increased (p...

  1. Recurrent Takotsubo Cardiomyopathy Related to Recurrent Thyrotoxicosis.

    Science.gov (United States)

    Patel, Keval; Griffing, George T; Hauptman, Paul J; Stolker, Joshua M

    2016-04-01

    Takotsubo cardiomyopathy, or transient left ventricular apical ballooning syndrome, is characterized by acute left ventricular dysfunction caused by transient wall-motion abnormalities of the left ventricular apex and mid ventricle in the absence of obstructive coronary artery disease. Recurrent episodes are rare but have been reported, and several cases of takotsubo cardiomyopathy have been described in the presence of hyperthyroidism. We report the case of a 55-year-old woman who had recurrent takotsubo cardiomyopathy, documented by repeat coronary angiography and evaluations of left ventricular function, in the presence of recurrent hyperthyroidism related to Graves disease. After both episodes, the patient's left ventricular function returned to normal when her thyroid function normalized. These findings suggest a possible role of thyroid-hormone excess in the pathophysiology of some patients who have takotsubo cardiomyopathy.

  2. On the Wegener granulomatosis associated region on chromosome 6p21.3

    Directory of Open Access Journals (Sweden)

    Csernok Elena

    2006-03-01

    Full Text Available Abstract Background Wegener granulomatosis (WG belongs to the heterogeneous group of systemic vasculitides. The multifactorial pathophysiology of WG is supposedly caused by yet unknown environmental influence(s on the basis of genetic predisposition. The presence of anti-neutrophil cytoplasmic antibodies (ANCA in the plasma of patients and genetic involvement of the human leukocyte antigen system reflect an autoimmune background of the disease. Strong associations were revealed with WG by markers located in the major histocompatibility complex class II (MHC II region in the vicinity of human leukocyte antigen (HLA-DPB1 and the retinoid X receptor B (RXRB loci. In order to define the involvement of the 6p21.3 region in WG in more detail this previous population-based association study was expanded here to the respective 3.6 megabase encompassing this region on chromosome 6. The RXRB gene was analysed as well as a splice-site variation of the butyrophilin-like (BTNL2 gene which is also located within the respective region. The latter polymorphism has been evaluated here as it appears as a HLA independent susceptibility factor in another granulomatous disorder, sarcoidosis. Methods 150–180 German WG patients and a corresponding cohort of healthy controls (n = 100–261 were used in a two-step study. A panel of 94 microsatellites was designed for the initial step using a DNA pooling approach. Markers with significantly differing allele frequencies between patient and control pools were individually genotyped. The RXRB gene was analysed for single strand conformation polymorphisms (SSCP and restriction fragment length polymorphisms (RFLP. The splice-site polymorphism in the BTNL2 gene was also investigated by RFLP analysis. Results A previously investigated microsatellite (#1.0.3.7, Santa Cruz genome browser (UCSC May 2004 Freeze localisation: chr6:31257596-34999883, which was used as a positive control, remained associated throughout the whole two

  3. Recurrent Abdominal Pain

    Science.gov (United States)

    Banez, Gerard A.; Gallagher, Heather M.

    2006-01-01

    The purpose of this article is to provide an empirically informed but clinically oriented overview of behavioral treatment of recurrent abdominal pain. The epidemiology and scope of recurrent abdominal pain are presented. Referral process and procedures are discussed, and standardized approaches to assessment are summarized. Treatment protocols…

  4. The eternal recurrence today

    CERN Document Server

    Neves, J C S

    2015-01-01

    In this work we have carried out an approach between the nonsingular scientific cosmologies (without the initial singularity, the big bang), specially the cyclic models, and the Nietzsche's thought of the eternal recurrence. Moreover, we have pointed out reasons for the Nietzsche's search for scientific proofs about the eternal recurrence in the decade of 1880's.

  5. Recurrent aphthous stomatitis

    Directory of Open Access Journals (Sweden)

    L Preeti

    2011-01-01

    Full Text Available Recurrent aphthous ulcers are common painful mucosal conditions affecting the oral cavity. Despite their high prevalence, etiopathogenesis remains unclear. This review article summarizes the clinical presentation, diagnostic criteria, and recent trends in the management of recurrent apthous stomatitis.

  6. Acute recurrent polyhydramnios

    DEFF Research Database (Denmark)

    Rode, Line; Bundgaard, Anne; Skibsted, Lillian

    2007-01-01

    Acute recurrent polyhydramnios is a rare occurrence characterized by a poor fetal outcome. This is a case report describing a 34-year-old woman presenting with acute recurrent polyhydramnios. Treatment with non-steroidal anti-inflammatory drugs (NSAID) and therapeutic amniocenteses was initiated ...

  7. FAU in Treating Patients With Advanced Solid Tumors or Lymphoma

    Science.gov (United States)

    2014-01-06

    Adult Grade III Lymphomatoid Granulomatosis; Adult Nasal Type Extranodal NK/T-cell Lymphoma; Anaplastic Large Cell Lymphoma; Angioimmunoblastic T-cell Lymphoma; Cutaneous B-cell Non-Hodgkin Lymphoma; Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Nodal Marginal Zone B-cell Lymphoma; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Grade III Lymphomatoid Granulomatosis; Recurrent Adult Hodgkin Lymphoma; Recurrent Adult Immunoblastic Large Cell Lymphoma; Recurrent Adult Lymphoblastic Lymphoma; Recurrent Adult T-cell Leukemia/Lymphoma; Recurrent Cutaneous T-cell Non-Hodgkin Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Mycosis Fungoides/Sezary Syndrome; Recurrent Small Lymphocytic Lymphoma; Small Intestine Lymphoma; Splenic Marginal Zone Lymphoma; Stage III Adult Burkitt Lymphoma; Stage III Adult Diffuse Large Cell Lymphoma; Stage III Adult Diffuse Mixed Cell Lymphoma; Stage III Adult Diffuse Small Cleaved Cell Lymphoma; Stage III Adult Hodgkin Lymphoma; Stage III Adult Immunoblastic Large Cell Lymphoma; Stage III Adult Lymphoblastic Lymphoma; Stage III Adult T-cell Leukemia/Lymphoma; Stage III Cutaneous T-cell Non-Hodgkin Lymphoma; Stage III Grade 1 Follicular Lymphoma; Stage III Grade 2 Follicular Lymphoma; Stage III Grade 3 Follicular Lymphoma; Stage III Mantle Cell Lymphoma; Stage III Marginal Zone Lymphoma; Stage III Mycosis Fungoides/Sezary Syndrome; Stage III Small Lymphocytic Lymphoma; Stage IV Adult Burkitt Lymphoma; Stage IV Adult Diffuse Large Cell Lymphoma; Stage IV Adult Diffuse Mixed Cell Lymphoma; Stage IV Adult Diffuse Small Cleaved Cell Lymphoma; Stage IV Adult Hodgkin Lymphoma; Stage IV Adult Immunoblastic Large Cell

  8. Granulomatose de Wegener e estenose subglótica: descrição de caso Wegener's granulomatosis and subglottic stenosis: case description

    Directory of Open Access Journals (Sweden)

    Fernanda Guidolin

    2004-06-01

    Full Text Available Descreve-se o caso de um paciente com Granulomatose de Wegener, em tratamento com esteróides e ciclofosfamida, que, apesar do tratamento, evoluiu com dispnéia progressiva e rouquidão. A investigação mostrou tratar-se de uma estenose subglótica, sendo a dispnéia prontamente aliviada com a traqueostomia. São discutidos diagnósticos diferenciais e modalidades de tratamentos de estenose subglótica em portadores de Wegener.We describe a patient with Wegener Granulomatosis being treated with steroids and cyclofosfamide that developed progressive dyspnea and hoarseness despite of treatment. Investigation showed a subglotic stenosis. The dyspnea was relieved by tracheotomy. We discuss the differential diagnosis as well as a number of treatment modalities in patients with Wegener granulomatosis and subglottic stenosis.

  9. Pityriasis lichenoides et varioliformis acuta with numerous CD30(+) cells: a variant mimicking lymphomatoid papulosis and other cutaneous lymphomas. A clinicopathologic, immunohistochemical, and molecular biological study of 13 cases.

    Science.gov (United States)

    Kempf, Werner; Kazakov, Dmitry V; Palmedo, Gabriele; Fraitag, Sylvie; Schaerer, Leo; Kutzner, Heinz

    2012-07-01

    Pityriasis lichenoides comprises a clinicopathologic spectrum of cutaneous inflammatory disorders, with the 2 most common variants being pityriasis lichenoides et varioliformis acuta (PLEVA) and pityriasis lichenoides chronica. The aim of the study was to describe 13 cases of a unique PLEVA variant characterized in the conspicuous CD30 component and thus mimicking lymphomatoid papulosis (LyP), a condition currently classified in the spectrum of CD30 lymphoproliferative disorders. The cohort included 10 female and 3 male patients whose ages at diagnosis ranged from 7 to 89 years (mean 41 y; median 39 y). The clinical manifestation was that of PLEVA, with small erythematous macules quickly evolving into necrotic papules. No waxing and waning was seen on follow-up in any of the cases. Histopathologically, typical features of PLEVA were present, but an unusual finding was occurrence of a considerable number of CD30 small lymphocytes as detected immunohistochemically. Over half of the cases also displayed a large number of CD8 cells and showed coexpression of CD8 and CD30 in the intraepidermal and dermal component of the infiltrate. Of the 11 cases of PLEVA studied for T-cell receptor gene rearrangement, 6 evidenced a monoclonal T-cell population, and 5 were polyclonal. Parvovirus B19 (PVB19) DNA was identified in 4 of 10 cases investigated, and positive serology was observed for PVB19 in 2 patients, altogether suggesting that PVB19 is pathogenetically linked to PLEVA at least in a subset of cases. The presence of CD30 lymphocytes and CD8 lymphocytes would be consistent with an inflammatory antiviral response, as CD30, even atypically appearing lymphoid cells have been identified in some viral skin diseases. The main significance of the PLEVA variant is, however, its potential confusion with LyP or some cytotoxic lymphomas. Admittedly, the CD30 PLEVA variant described herein and LyP show considerable overlap if one takes into account all known variations of the 2

  10. Manifestaciones clínicas de la granulomatosis de Wegener y la poliangeítis microscópica en Santiago-Chile, 1990-2001

    OpenAIRE

    Cisternas M,Marcela; Soto S,Lilian; Jacobelli G,Sergio; Marinovic M,María Angélica; Vargas B,Alex; Sobarzo V,Elizabeth; Saavedra M,Jorge; Chauan I,Karina; Meléndez T,Gabriela; Foster B,Carolina; Pacheco R,Daniel; Wainstein G,Eduardo

    2005-01-01

    Background: Systemic vasculitis are a group of heterogeneous diseases characterized by inflammation and necrosis of blood vessel walls. The etiology is not known, but geographic and environmental factors are implicated. Aim: To describe the clinical features of microscopic polyangiitis (MPA) and Wegener's granulomatosis (WG) in a Chilean cohort of patients. Patients and methods: Retrospective review of the medical records of 123 patients with the diagnosis of systemic vasculitis (65 MPA and 5...

  11. Predictors of Recurrent AKI.

    Science.gov (United States)

    Siew, Edward D; Parr, Sharidan K; Abdel-Kader, Khaled; Eden, Svetlana K; Peterson, Josh F; Bansal, Nisha; Hung, Adriana M; Fly, James; Speroff, Ted; Ikizler, T Alp; Matheny, Michael E

    2016-04-01

    Recurrent AKI is common among patients after hospitalized AKI and is associated with progressive CKD. In this study, we identified clinical risk factors for recurrent AKI present during index AKI hospitalizations that occurred between 2003 and 2010 using a regional Veterans Administration database in the United States. AKI was defined as a 0.3 mg/dl or 50% increase from a baseline creatinine measure. The primary outcome was hospitalization with recurrent AKI within 12 months of discharge from the index hospitalization. Time to recurrent AKI was examined using Cox regression analysis, and sensitivity analyses were performed using a competing risk approach. Among 11,683 qualifying AKI hospitalizations, 2954 patients (25%) were hospitalized with recurrent AKI within 12 months of discharge. Median time to recurrent AKI within 12 months was 64 (interquartile range 19-167) days. In addition to known demographic and comorbid risk factors for AKI, patients with longer AKI duration and those whose discharge diagnosis at index AKI hospitalization included congestive heart failure (primary diagnosis), decompensated advanced liver disease, cancer with or without chemotherapy, acute coronary syndrome, or volume depletion, were at highest risk for being hospitalized with recurrent AKI. Risk factors identified were similar when a competing risk model for death was applied. In conclusion, several inpatient conditions associated with AKI may increase the risk for recurrent AKI. These findings should facilitate risk stratification, guide appropriate patient referral after AKI, and help generate potential risk reduction strategies. Efforts to identify modifiable factors to prevent recurrent AKI in these patients are warranted. Copyright © 2016 by the American Society of Nephrology.

  12. Recurrent Intracerebral Hemorrhage

    DEFF Research Database (Denmark)

    Schmidt, Linnea Boegeskov; Goertz, Sanne; Wohlfahrt, Jan;

    2016-01-01

    the use of any of the investigated medicines with antithrombotic effect (ATT, SSRI's, NSAID's) and recurrent ICH. CONCLUSIONS: The substantial short-and long-term recurrence risks warrant aggressive management of hypertension following a primary ICH, particularly in patients treated surgically...... treatment and renal insufficiency were associated with increased recurrence risks (RR 1.64, 95% CI 1.39-1.93 and RR 1.72, 95% CI 1.34-2.17, respectively), whereas anti-hypertensive treatment was associated with a reduced risk (RR 0.82, 95% CI 0.74-0.91). We observed non-significant associations between...

  13. RECURRENT CROUP IN CHILDREN

    Directory of Open Access Journals (Sweden)

    S. L. Piskunova

    2014-01-01

    Full Text Available The article presents the results of examination of 1849 children, entering children's infectioushospitalofVladivostokwith the clinical picture of croup of viral etiology. The clinical features of primary and recurrent croup are described. Frequency of recurrent croup inVladivostokis 8%. Children with a recurrent croup had the burdened premorbid background, and also persistent herpetic infections (cytomegalic infection in 42,9% cases, cytomegalic infection in combination with the herpes simplex virus -1. Frequency of croups substantially rose in the period of epidemic of influenza.

  14. Recurrent Breast Cancer

    Science.gov (United States)

    ... that can help you cope with distress include: Art therapy Dance or movement therapy Exercise Meditation Music ... mayoclinic.org/diseases-conditions/recurrent-breast-cancer/basics/definition/CON-20032432 . Mayo Clinic Footer Legal Conditions and ...

  15. Multifocal recurrent periostitis

    Energy Technology Data Exchange (ETDEWEB)

    Kozlowski, K.; Anderson, R.; Tink, A.

    1981-11-01

    Two case reports of recurrent multifocal periostitis in two girls aged 15 and 16 are added to the eight cases already reported in the literature. The disease is characterised clinically by recurrent mesomelic swelling of the extremities and radiologically by periosteal thickening and sclerosis of underlying bone. Hyperglobulinaemia is the most constant biochemical finding. The bone biopsy shows no typical features. The possibility of a viral etiology is discussed.

  16. Recurrences of strange attractors

    Indian Academy of Sciences (India)

    E J Ngamga; A Nandi; R Ramaswamy; M C Romano; M Thiel; J Kurths

    2008-06-01

    The transitions from or to strange nonchaotic attractors are investigated by recurrence plot-based methods. The techniques used here take into account the recurrence times and the fact that trajectories on strange nonchaotic attractors (SNAs) synchronize. The performance of these techniques is shown for the Heagy-Hammel transition to SNAs and for the fractalization transition to SNAs for which other usual nonlinear analysis tools are not successful.

  17. Recurrent Fever in Children.

    Science.gov (United States)

    Torreggiani, Sofia; Filocamo, Giovanni; Esposito, Susanna

    2016-03-25

    Children presenting with recurrent fever may represent a diagnostic challenge. After excluding the most common etiologies, which include the consecutive occurrence of independent uncomplicated infections, a wide range of possible causes are considered. This article summarizes infectious and noninfectious causes of recurrent fever in pediatric patients. We highlight that, when investigating recurrent fever, it is important to consider age at onset, family history, duration of febrile episodes, length of interval between episodes, associated symptoms and response to treatment. Additionally, information regarding travel history and exposure to animals is helpful, especially with regard to infections. With the exclusion of repeated independent uncomplicated infections, many infective causes of recurrent fever are relatively rare in Western countries; therefore, clinicians should be attuned to suggestive case history data. It is important to rule out the possibility of an infectious process or a malignancy, in particular, if steroid therapy is being considered. After excluding an infectious or neoplastic etiology, immune-mediated and autoinflammatory diseases should be taken into consideration. Together with case history data, a careful physical exam during and between febrile episodes may give useful clues and guide laboratory investigations. However, despite a thorough evaluation, a recurrent fever may remain unexplained. A watchful follow-up is thus mandatory because new signs and symptoms may appear over time.

  18. Recurrent Fever in Children

    Directory of Open Access Journals (Sweden)

    Sofia Torreggiani

    2016-03-01

    Full Text Available Children presenting with recurrent fever may represent a diagnostic challenge. After excluding the most common etiologies, which include the consecutive occurrence of independent uncomplicated infections, a wide range of possible causes are considered. This article summarizes infectious and noninfectious causes of recurrent fever in pediatric patients. We highlight that, when investigating recurrent fever, it is important to consider age at onset, family history, duration of febrile episodes, length of interval between episodes, associated symptoms and response to treatment. Additionally, information regarding travel history and exposure to animals is helpful, especially with regard to infections. With the exclusion of repeated independent uncomplicated infections, many infective causes of recurrent fever are relatively rare in Western countries; therefore, clinicians should be attuned to suggestive case history data. It is important to rule out the possibility of an infectious process or a malignancy, in particular, if steroid therapy is being considered. After excluding an infectious or neoplastic etiology, immune-mediated and autoinflammatory diseases should be taken into consideration. Together with case history data, a careful physical exam during and between febrile episodes may give useful clues and guide laboratory investigations. However, despite a thorough evaluation, a recurrent fever may remain unexplained. A watchful follow-up is thus mandatory because new signs and symptoms may appear over time.

  19. Prolonged B cell depletion with rituximab is effective in treating refractory pulmonary granulomatous inflammation in granulomatosis with polyangiitis (GPA).

    Science.gov (United States)

    Henderson, Scott R; Copley, Susan J; Pusey, Charles D; Ind, Philip W; Salama, Alan D

    2014-12-01

    Pulmonary nodule formation is a frequent feature of granulomatosis with polyangiitis (GPA). Traditional induction therapy includes methotrexate or cyclophosphamide, however, pulmonary nodules generally respond slower than vasculitic components of disease. Efficacy of rituximab (RTX) solely for the treatment of pulmonary nodules has not been assessed. In this observational cohort study, we report patient outcomes with RTX in GPA patients with pulmonary nodules who failed to achieve remission following conventional immunosuppression. Patients (n = 5) with persistent pulmonary nodules were identified from our clinic database and retrospectively evaluated. Systemic manifestations, inflammatory markers, disease activity, concurrent immunosuppression, and absolute B cell numbers were recorded pre-RTX and at 6 monthly intervals following treatment. Chest radiographs at each time point were scored by an experienced radiologist, blinded to clinical details. Five patients with GPA and PR3-ANCA were evaluated (2 male, 3 female), mean age 34 (22-52) years. Pulmonary nodules (median 4, range 2-6), with or without cavitation were present in all patients. RTX induced initial B cell depletion (GPA following prolonged B cell depletion.

  20. Orofacial granulomatosis affecting lip and gingiva in a 15-year-old patient: A rare case report

    Directory of Open Access Journals (Sweden)

    Monika Bansal

    2015-01-01

    Full Text Available Orofacial granulomatosis (OFG is a rare disorder affecting the orofacial region, and clinically characterized by diffuse, nontender, soft to firm, painless swelling restricted to one or both lips and intraoral sites such as tongue, gingiva and buccal mucosa. Histologically, OFG is characterized by noncaseating granulomatous inflammation. The early diagnosis of OFG is essential for the better prognosis of the lesion. Delay in diagnosis of OFG results into formation of indurated and permanent swelling of the lip that not only compromises esthetic appearance but also causes impairment in function such as speaking and eating. Early diagnosis of OFG is challenging to the health care professionals due to clinical and histological resemblance to other chronic granulomatous disorders. Thus, dentists may act as a first person to diagnose the lesion and play an important role in the multidisciplinary treatment of granulomatous disorders. Here, we present a case of OFG affecting lips and gingiva in a 15-year-old patient without any identifiable systemic or local causes.

  1. Epidemiology and etiology of wegener granulomatosis, microscopic polyangiitis, churg-strauss syndrome and goodpasture syndrome: vasculitides with frequent lung involvement.

    Science.gov (United States)

    Gibelin, Aude; Maldini, Carla; Mahr, Alfred

    2011-06-01

    This review focuses on the epidemiological characteristics and etiologies of four primary systemic vasculitides with frequent lung involvement, namely Wegener granulomatosis (WG), microscopic polyangiitis (MPA), Churg-Strauss syndrome (CSS), and Goodpasture syndrome (GPS). Elucidation of the mechanisms underlying these vasculitides with frequent lung involvement is complicated by their rarity, which hampers the undertaking of large-scale studies; difficulties in classification; and their multifaceted clinical presentations, which infer the existence of several etiologic pathways. Notwithstanding, epidemiological research showed some evidence for international, interethnic, and temporal variations of the frequencies of these four vasculitides; led to the identification of several genetic and environmental risk factors; and provided insight on the extent to which genes and environment might contribute to their development. Available data support the concept that WG, MPA, CSS, and GPS have unique and shared risk determinants. Although the precise causes of these vasculitides are not yet fully understood and the development of prevention strategies is out of our reach at present, current knowledge enables the formulation of etiologic hypotheses to provide caregivers and their patients with valuable information on the nature of these rare entities. © Thieme Medical Publishers.

  2. Recurrent wheezing in children

    Science.gov (United States)

    Piazza, Michele; Piacentini, Giorgio

    2016-01-01

    Recurrent wheezing have a significant morbidity and it’s estimated that about one third of school-age children manifest the symptom during the first 5 years of life. Proper identification of children at risk of developing asthma at school age may predict long-term outcomes and improve treatment and preventive approach, but the possibility to identify these children at preschool age remains limited. For many years authors focused their studies to identify early children with recurrent wheezing at risk to develop asthma at school age. Different phenotypes have been proposed for a more precise characterization and a personalized plan of treatment. The main criticism concerns the inability to define stable phenotypes with the risk of overestimating or underestimating the characteristics of symptoms in these children. The aim of this review is to report the recent developments on the diagnosis and treatment of recurrent paediatric wheezing. PMID:26835404

  3. Recurrence of angular cheilitis.

    Science.gov (United States)

    Ohman, S C; Jontell, M; Dahlen, G

    1988-08-01

    The incidence of recurrence of angular cheilitis following a successful antimicrobial treatment was studied in 48 patients. Clinical assessments including a microbial examination were carried out 8 months and 5 yr after termination of treatment. Eighty percent of the patients reported recurrence of their angular cheilitis on one or more occasions during the observation period. Patients with cutaneous disorders associated with dry skin or intraoral leukoplakia had an increased incidence of recrudescence. Neither the presence of denture stomatitis nor the type of microorganisms isolated from the original lesions of angular cheilitis, i.e. Candida albicans and/or Staphylococcus aureus, were associated with the number of recurrences. The present observations indicate that treatment of the majority of patients with angular cheilitis should be considered in a longer perspective than previously supposed, due to the short lasting therapeutic effects of the antimicrobial therapy.

  4. Recurrent Novae - A Review

    CERN Document Server

    Mukai, Koji

    2014-01-01

    In recent years, recurrent nova eruptions are often observed very intensely in wide range of wavelengths from radio to optical to X-rays. Here I present selected highlights from recent multi-wavelength observations. The enigma of T Pyx is at the heart of this paper. While our current understanding of CV and symbiotic star evolution can explain why certain subset of recurrent novae have high accretion rate, that of T Pyx must be greatly elevated compared to the evolutionary mean. At the same time, we have extensive data to be able to estimate how the nova envelope was ejected in T Pyx, and it turns to be a rather complex tale. One suspects that envelope ejection in recurrent and classical novae in general is more complicated than the textbook descriptions. At the end of the review, I will speculate that these two may be connected.

  5. Recurrent parotitis in children

    Directory of Open Access Journals (Sweden)

    Bhattarai M

    2006-01-01

    Full Text Available Recurrent parotitis is an uncommon condition in children. Its etiological factors have not been proved till date although causes due to genetic inheritance, local autoimmune manifestation, allergy, viral infection and immunodeficiency have been suggested. The exact management of this disorder is not yet standardized, but a conservative approach is preferred and all affected children should be screened for Sjogren′s syndrome and immune deficiency including human immunodeficiency virus. We report a 12 years female child who presented with 12 episodes of non-painful recurrent swellings of the bilateral parotid gland in the past 3 years.

  6. Tackling a recurrent pinealoblastoma.

    Science.gov (United States)

    Palled, Siddanna; Kalavagunta, Sruthi; Beerappa Gowda, Jaipal; Umesh, Kavita; Aal, Mahalaxmi; Abdul Razack, Tanvir Pasha Chitraduraga; Gowda, Veerabhadre; Viswanath, Lokesh

    2014-01-01

    Pineoblastomas are rare, malignant, pineal region lesions that account for <0.1% of all intracranial tumors and can metastasize along the neuroaxis. Pineoblastomas are more common in children than in adults and adults account for <10% of patients. The management of pinealoblastoma is multimodality approach, surgery followed with radiation and chemotherapy. In view of aggressive nature few centres use high dose chemotherapy with autologus stem cell transplant in newly diagnosed cases but in recurrent setting the literature is very sparse. The present case represents the management of pinealoblastoma in the recurrent setting with reirradiation and adjuvant carmustine chemotherapy wherein the management guidelines are not definitive.

  7. Lung Cancer Indicators Recurrence

    Science.gov (United States)

    This study describes prognostic factors for lung cancer spread and recurrence, as well as subsequent risk of death from the disease. The investigators observed that regardless of cancer stage, grade, or type of lung cancer, patients in the study were more

  8. Chronic recurrent multifocal osteomyelitis

    NARCIS (Netherlands)

    Wedman, Jan; van Weissenbruch, Ranny

    2005-01-01

    We report what is, to our best knowledge, the first case of chronic recurrent multifocal osteomyelitis (CRMO) in which the frontal and sphenoid bones were involved. Characterized by a prolonged and fluctuating course of osteomyelitis at different sites, CRMO is self-limited, although sequelae can oc

  9. Recurrent Gliosarcoma in Pregnancy

    OpenAIRE

    2014-01-01

    Gliosarcoma is a rare tumor of the central nervous system and it constitutes about 1 to 8% of all malignant gliomas. In this report we are presenting a recurrent gliosarcoma case during a pregnancy in a 30-year-old woman. This is the first report presenting gliosarcoma in the pregnancy.

  10. Recurrent gallstone ileus.

    Science.gov (United States)

    Hayes, Nicolas; Saha, Sanjoy

    2012-11-01

    Mechanical small bowel obstructions caused by gallstones account for 1% to 3% of cases. In these patients, 80% to 90% of residual gallstones in these patients will pass through a remaining fistula without consequence. Recurrent gallstone ileus has been reported in 5% of patients. We report the case of a woman, aged 72 years, who presented with mechanical small bowel obstruction caused by gallstone ileus. After successful surgical therapy for gallstone ileus, the patient's symptoms recurred, and she was diagnosed with recurrent gallstone ileus requiring a repeat operation. While management of gallstone ileus can be achieved through a single-stage operation including enterolithotomy and cholecystectomy with repair of biliary-enteric fistula or by enterolithotomy alone, the literature supports enterolithotomy alone as the treatment of choice for gallstone ileus due to decreased mortality and morbidity. However, the latter approach does not obviate potential recurrence. We present this case of recurrent gallstone ileus to elucidate and review the pathogenesis, presentation, diagnosis, and consensus recommendations regarding management of this disorder.

  11. Acute Recurrent Pancreatitis

    Directory of Open Access Journals (Sweden)

    Glen A Lehman

    2003-01-01

    Full Text Available History, physical examination, simple laboratory and radiological tests, and endoscopic retrograde cholangiopancreatography (ERCP are able to establish the cause of recurrent acute pancreatitis in 70% to 90% of patients. Dysfunction of the biliary and/or pancreatic sphincter, as identified by sphincter of Oddi manometry, accounts for the majority of the remaining cases. The diagnosis may be missed if the pancreatic sphincter is not evaluated. Pancreas divisum is a prevalent congenital abnormality that is usually innocuous but can lead to recurrent attacks of acute pancreatitis or abdominal pain. In select cases, endoscopic sphincterotomy of the minor papilla can provide relief of symptoms and prevent further attacks. A small proportion of patients with idiopathic pancreatitis have tiny stones in the common bile duct (microlithiasis. Crystals can be visualized during microscopic analysis of bile that is aspirated at the time of ERCP. Neoplasia is a rare cause of pancreatitis, and the diagnosis can usually be established by computerized tomography or ERCP. A wide variety of medications can also cause recurrent pancreatitis. ERCP, sphincter of Oddi manometry, and microscopy of aspirated bile should be undertaken in patients with recurrent pancreatitis in whom the diagnosis is not obvious.

  12. Recurrent Spatial Transformer Networks

    DEFF Research Database (Denmark)

    Sønderby, Søren Kaae; Sønderby, Casper Kaae; Maaløe, Lars;

    2015-01-01

    We integrate the recently proposed spatial transformer network (SPN) [Jaderberg et. al 2015] into a recurrent neural network (RNN) to form an RNN-SPN model. We use the RNN-SPN to classify digits in cluttered MNIST sequences. The proposed model achieves a single digit error of 1.5% compared to 2...

  13. Diffuse alveolar hemorrhage emerging one week after starting high-dose corticosteroid therapy for granulomatosis with polyangiitis (GPA) with systemic lupus erythematosus (SLE).

    Science.gov (United States)

    Fukui, Shoichi; Iwamoto, Naoki; Tsuji, Sosuke; Umeda, Masataka; Nishino, Ayako; Nakashima, Yoshikazu; Suzuki, Takahisa; Horai, Yoshiro; Koga, Tomohiro; Kawashiri, Shin-Ya; Ichinose, Kunihiro; Hirai, Yasuko; Tamai, Mami; Nakamura, Hideki; Origuchi, Tomoki; Kawakami, Atsushi

    2015-01-01

    A 69-year-old man was diagnosed with granulomatosis with polyangiitis (GPA) based on the presence of skin granuloma, refractory otitis media, renal insufficiency and myeloperoxidase-antineutrophil cytoplasmic antibody positivity and slight lung opacity. He was treated with high-dose corticosteroid therapy. Despite the initial improvement of his renal function and a decrease in his C-reactive protein level, he suffered from an alveolar hemorrhage one week after the start of corticosteroid therapy. An anti-dsDNA antibody test was positive and the patient had hypocomplementemia. Elements of both GPA and systemic lupus erythematosus were thought to have affected his clinical course.

  14. Cardiac tamponade leading to the diagnosis of eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome): a case report and review of the literature.

    Science.gov (United States)

    Yano, Toshiyuki; Ishimura, Shutaro; Furukawa, Tetsuaki; Koyama, Masayuki; Tanaka, Marenao; Shimoshige, Shinya; Hashimoto, Akiyoshi; Miura, Tetsuji

    2015-11-01

    Eosinophilic granulomatosis with polyangiitis (EGPA), which was previously called Churg-Strauss syndrome, is a necrotizing systemic vasculitis of unknown cause accompanied by prominent eosinophilia. Cardiovascular complications, including eosinophilic myocarditis, are a major cause of mortality in this disorder. Acute pericarditis with slight pericardial effusion is a typical manifestation in EGPA, though hemodynamically significant pericardial effusion has been reported in a few cases. We report a case that initially presented with isolated cardiac tamponade, which was followed by systemic manifestations of EGPA over 3 weeks. Including the present case, previous EGPA cases with cardiac tamponade are reviewed to delineate its clinical characteristics.

  15. Coping with Fear of Recurrence

    Science.gov (United States)

    ... What Comes Next After Finishing Treatment Coping With Fear of Recurrence Having a Baby After Cancer: Pregnancy ... treatment and preparing for the future. Coping With Fear of Recurrence Learn ways to manage the fear ...

  16. Chaotic diagonal recurrent neural network

    Institute of Scientific and Technical Information of China (English)

    Wang Xing-Yuan; Zhang Yi

    2012-01-01

    We propose a novel neural network based on a diagonal recurrent neural network and chaos,and its structure andlearning algorithm are designed.The multilayer feedforward neural network,diagonal recurrent neural network,and chaotic diagonal recurrent neural network are used to approach the cubic symmetry map.The simulation results show that the approximation capability of the chaotic diagonal recurrent neural network is better than the other two neural networks.

  17. Increased frequency of circulating IL-21 producing Th-cells in patients with granulomatosis with polyangiitis (GPA).

    Science.gov (United States)

    Abdulahad, Wayel H; Lepse, Nikola; Stegeman, Coen A; Huitema, Minke G; Doornbos-van der Meer, Berber; Tadema, Henko; Rutgers, Abraham; Limburg, Pieter C; Kallenberg, Cees G M; Heeringa, Peter

    2013-01-01

    The present study aimed to explore a possible role for IL-21 producing Th-cells in the immunopathogenesis of granulomatosis with polyangiitis (GPA). Peripheral blood from 42 GPA patients in remission and 29 age-matched healthy controls (HCs) were stimulated in vitro, and the frequencies of IL-21 producing Th-cells were determined by flow cytometry. Since Th17-cells produce a low level of IL-21, IL-17 was also included in the analysis. Given that IL-21 is a hallmark cytokine for T follicular helper cells (T(FH)), we next evaluated the expression of their key transcription factor BCL-6 by RT-PCR and flow cytometry. To investigate the effect of IL-21 on autoantibody-production, PBMCs from GPA patients were stimulated in vitro with BAFF/IL-21 and total IgG and ANCA levels were measured in supernatants. In addition, the expression of IL-21-receptor on B-cells was analyzed. Percentages of IL-21 producing Th-cells were significantly elevated in GPA-patients compared to HCs, and were restricted to ANCA-positive patients. The expression of BCL-6 was significantly higher in ANCA-positive GPA-patients, as compared with ANCA-negative patients and HCs. IL-21 enhanced the production of IgG and ANCA in vitro in stimulated PBMCs from GPA patients. No difference was found in the expression of the IL-21-receptor on B-cells between ANCA-negative patients, ANCA-positive patients, and HCs. The increased frequency of circulating IL-21 producing Th-cells in ANCA-positive GPA patients and the stimulating capacity of IL-21 on ANCA-production suggest a role for these cells in the immunopathogenesis of GPA. Blockade of IL-21 could constitute a new therapeutic strategy for GPA.

  18. Immunomodulators to treat recurrent miscarriage

    NARCIS (Netherlands)

    Prins, Jelmer R.; Kieffer, Tom E.C.; Scherjon, Sicco A.

    2014-01-01

    Recurrent miscarriage is a reproductive disorder affecting many couples. Although several factors are associated with recurrent miscarriage, in more than 50% of the cases the cause is unknown. Maladaptation of the maternal immune system is associated with recurrent miscarriage and could explain part

  19. Imaging of recurrent prostate cancer

    NARCIS (Netherlands)

    Futterer, J.J.

    2012-01-01

    Approximately 30\\% of patients who underwent radical prostatectomy or radiation therapy will develop biochemical recurrent disease. Biochemical recurrent disease is defined as an increase in the serum value of prostate-specific antigen (PSA) after reaching the nadir. Prostate recurrence can present

  20. Immunomodulators to treat recurrent miscarriage

    NARCIS (Netherlands)

    Prins, Jelmer R.; Kieffer, Tom E. C.; Scherjon, Sicco A.

    2014-01-01

    Recurrent miscarriage is a reproductive disorder affecting many couples. Although several factors are associated with recurrent miscarriage, in more than 50% of the cases the cause is unknown. Maladaptation of the maternal immune system is associated with recurrent miscarriage and could explain part

  1. Training Recurrent Networks

    DEFF Research Database (Denmark)

    Pedersen, Morten With

    1997-01-01

    Training recurrent networks is generally believed to be a difficult task. Excessive training times and lack of convergence to an acceptable solution are frequently reported. In this paper we seek to explain the reason for this from a numerical point of view and show how to avoid problems when tra...... training. In particular we investigate ill-conditioning, the need for and effect of regularization and illustrate the superiority of second-order methods for training......Training recurrent networks is generally believed to be a difficult task. Excessive training times and lack of convergence to an acceptable solution are frequently reported. In this paper we seek to explain the reason for this from a numerical point of view and show how to avoid problems when...

  2. Spatial recurrence plots.

    Science.gov (United States)

    Vasconcelos, D B; Lopes, S R; Viana, R L; Kurths, J

    2006-05-01

    We propose an extension of the recurrence plot concept to perform quantitative analyzes of roughness and disorder of spatial patterns at a fixed time. We introduce spatial recurrence plots (SRPs) as a graphical representation of the pointwise correlation matrix, in terms of a two-dimensional spatial return plot. This technique is applied to the study of complex patterns generated by coupled map lattices, which are characterized by measures of complexity based on SRPs. We show that the complexity measures we propose for SRPs provide a systematic way of investigating the distribution of spatially coherent structures, such as synchronization domains, in lattice profiles. This approach has potential for many more applications, e.g., in surface roughness analyzes.

  3. Modularity promotes epidemic recurrence

    CERN Document Server

    Jesan, T; Sinha, Sitabhra

    2016-01-01

    The long-term evolution of epidemic processes depends crucially on the structure of contact networks. As empirical evidence indicates that human populations exhibit strong community organization, we investigate here how such mesoscopic configurations affect the likelihood of epidemic recurrence. Through numerical simulations on real social networks and theoretical arguments using spectral methods, we demonstrate that highly contagious diseases that would have otherwise died out rapidly can persist indefinitely for an optimal range of modularity in contact networks.

  4. Recurrent Gallstone Ileus

    OpenAIRE

    Hayes, Nicolas; Saha, Sanjoy

    2012-01-01

    Mechanical small bowel obstructions caused by gallstones account for 1% to 3% of cases. In these patients, 80% to 90% of residual gallstones in these patients will pass through a remaining fistula without consequence. Recurrent gallstone ileus has been reported in 5% of patients. We report the case of a woman, aged 72 years, who presented with mechanical small bowel obstruction caused by gallstone ileus. After successful surgical therapy for gallstone ileus, the patient's symptoms recurred, a...

  5. EULAR/PRINTO/PRES criteria for Henoch-Schönlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part I: Overall methodology and clinical characterisation

    DEFF Research Database (Denmark)

    Ruperto, Nicolino; Ozen, Seza; Pistorio, Angela;

    2010-01-01

    To report methodology and overall clinical, laboratory and radiographic characteristics for Henoch-Schönlein purpura (HSP), childhood polyarteritis nodosa (c-PAN), c-Wegener granulomatosis (c-WG) and c-Takayasu arteritis (c-TA) classification criteria....

  6. Pseudotumor pulmonar como apresentação inicial de granulomatose de Wegener Lung pseudotumor as the initial presentation of Wegener's granulomatosis

    Directory of Open Access Journals (Sweden)

    Marcelo Fouad Rabahi

    2009-04-01

    Full Text Available A granulomatose de Wegener é uma doença inflamatória multissistêmica de etiologia desconhecida, que se caracteriza por vasculite de pequeno e médio calibre. As manifestações clínicas mais comuns envolvem o trato respiratório superior, pulmões e rins e as alterações encontradas nos exames de imagem são geralmente opacidades pulmonares ou nódulos múltiplos, bilaterais e, em 50% dos casos, cavitações. O tratamento é feito com corticoides e imunossupressores. Descreve-se um caso atípico de um homem de 61 anos cuja investigação inicial de um tumor pulmonar unilateral evidenciou, na verdade, Granulomatose de Wegener.Wegener's granulomatosis is a multisystemic inflammatory illness of unknown etiology, characterized by vasculitis of small and medium caliber vessels. The most common clinical manifestations involve the upper respiratory tract, lungs and kidneys. Common alterations in imaging studies include pulmonary opacities and bilateral multiple nodules, cavitations occurring in 50% of the cases. Treatment includes corticosteroids and immunosuppressants. We describe an atypical case of a 61-year-old man initially investigated due to suspicion of a unilateral lung tumor, which proved to be, in fact, a case of Wegener's granulomatosis.

  7. Multiscale recurrence quantification analysis of order recurrence plots

    Science.gov (United States)

    Xu, Mengjia; Shang, Pengjian; Lin, Aijing

    2017-03-01

    In this paper, we propose a new method of multiscale recurrence quantification analysis (MSRQA) to analyze the structure of order recurrence plots. The MSRQA is based on order patterns over a range of time scales. Compared with conventional recurrence quantification analysis (RQA), the MSRQA can show richer and more recognizable information on the local characteristics of diverse systems which successfully describes their recurrence properties. Both synthetic series and stock market indexes exhibit their properties of recurrence at large time scales that quite differ from those at a single time scale. Some systems present more accurate recurrence patterns under large time scales. It demonstrates that the new approach is effective for distinguishing three similar stock market systems and showing some inherent differences.

  8. Recurrent Pregnancy Loss

    Directory of Open Access Journals (Sweden)

    Véronique Piroux

    1997-01-01

    Full Text Available Antiphospholipid antibodies (APA are associated with thrombosis, thrombocytopenia and fetal loss but they occur in a variety of diseases. Despite many efforts, a correlation between the specificity of particular subgroups of APA and particular clinical situations remains to be established. The antigens at the origin of APA remain to be identified. We discuss here the possible links between cell apoptosis or necrosis, leading to plasma membrane alterations, and the occurrence of APA in response to sustained stimulation. The pathogenic potential of APA is also considered with respect to recurrent pregnancy loss.

  9. Recurrent Miller Fisher syndrome.

    Science.gov (United States)

    Madhavan, S; Geetha; Bhargavan, P V

    2004-07-01

    Miller Fisher syndrome (MFS) is a variant of Guillan Barre syndrome characterized by the triad of ophthalmoplegia, ataxia and areflexia. Recurrences are exceptional with Miller Fisher syndrome. We are reporting a case with two episodes of MFS within two years. Initially he presented with partial ophthalmoplegia, ataxia. Second episode was characterized by full-blown presentation characterized by ataxia, areflexia and ophthalmoplegia. CSF analysis was typical during both episodes. Nerve conduction velocity study was fairly within normal limits. MRI of brain was within normal limits. He responded to symptomatic measures initially, then to steroids in the second episode. We are reporting the case due to its rarity.

  10. Recurrence Relations and Determinants

    CERN Document Server

    Janjic, Milan

    2011-01-01

    We examine relationships between two minors of order n of some matrices of n rows and n+r columns. This is done through a class of determinants, here called $n$-determinants, the investigation of which is our objective. We prove that 1-determinants are the upper Hessenberg determinants. In particular, we state several 1-determinants each of which equals a Fibonacci number. We also derive relationships among terms of sequences defined by the same recurrence equation independently of the initial conditions. A result generalizing the formula for the product of two determinants is obtained. Finally, we prove that the Schur functions may be expressed as $n$-determinants.

  11. Mycophenolate Mofetil and Cyclosporine in Reducing Graft-Versus-Host Disease in Patients With Hematologic Malignancies or Metastatic Kidney Cancer Undergoing Donor Stem Cell Transplant

    Science.gov (United States)

    2017-02-27

    Lymphoma; Noncontiguous Stage II Adult Lymphoblastic Lymphoma; Noncontiguous Stage II Grade 3 Follicular Lymphoma; Noncontiguous Stage II Mantle Cell Lymphoma; Noncutaneous Extranodal Lymphoma; Peripheral T-cell Lymphoma; Post-transplant Lymphoproliferative Disorder; Previously Treated Myelodysplastic Syndromes; Recurrent Adult Acute Lymphoblastic Leukemia; Recurrent Adult Acute Myeloid Leukemia; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Grade III Lymphomatoid Granulomatosis; Recurrent Adult Hodgkin Lymphoma; Recurrent Adult Immunoblastic Large Cell Lymphoma; Recurrent Adult Lymphoblastic Lymphoma; Recurrent Adult T-cell Leukemia/Lymphoma; Recurrent Childhood Acute Lymphoblastic Leukemia; Recurrent Childhood Acute Myeloid Leukemia; Recurrent Childhood Anaplastic Large Cell Lymphoma; Recurrent Childhood Grade III Lymphomatoid Granulomatosis; Recurrent Childhood Large Cell Lymphoma; Recurrent Childhood Lymphoblastic Lymphoma; Recurrent Childhood Small Noncleaved Cell Lymphoma; Recurrent Cutaneous T-cell Non-Hodgkin Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Mycosis Fungoides/Sezary Syndrome; Recurrent Renal Cell Cancer; Recurrent Small Lymphocytic Lymphoma; Recurrent/Refractory Childhood Hodgkin Lymphoma; Refractory Anemia; Refractory Anemia With Ringed Sideroblasts; Refractory Chronic Lymphocytic Leukemia; Refractory Hairy Cell Leukemia; Refractory Multiple Myeloma; Relapsing Chronic Myelogenous Leukemia; Splenic Marginal Zone Lymphoma; Stage I Adult Burkitt Lymphoma; Stage I Adult Diffuse Large Cell Lymphoma; Stage I Adult Diffuse Mixed Cell Lymphoma; Stage I Adult Immunoblastic Large Cell Lymphoma; Stage I Adult Lymphoblastic Lymphoma; Stage I Adult T-cell Leukemia

  12. Recurrent correlation associative memories.

    Science.gov (United States)

    Chiueh, T D; Goodman, R M

    1991-01-01

    A model for a class of high-capacity associative memories is presented. Since they are based on two-layer recurrent neural networks and their operations depend on the correlation measure, these associative memories are called recurrent correlation associative memories (RCAMs). The RCAMs are shown to be asymptotically stable in both synchronous and asynchronous (sequential) update modes as long as their weighting functions are continuous and monotone nondecreasing. In particular, a high-capacity RCAM named the exponential correlation associative memory (ECAM) is proposed. The asymptotic storage capacity of the ECAM scales exponentially with the length of memory patterns, and it meets the ultimate upper bound for the capacity of associative memories. The asymptotic storage capacity of the ECAM with limited dynamic range in its exponentiation nodes is found to be proportional to that dynamic range. Design and fabrication of a 3-mm CMOS ECAM chip is reported. The prototype chip can store 32 24-bit memory patterns, and its speed is higher than one associative recall operation every 3 mus. An application of the ECAM chip to vector quantization is also described.

  13. [Treatment of recurrent posterior epistaxis].

    Science.gov (United States)

    Bro, Søren Pauli; Bille, Jesper; Petersen, Kristian Bruun

    2017-08-21

    30% of the patients presenting with epistaxis at emergency wards and otorhinolaryngeal specialist departments have posterior bleeding. Traditional treatment with packing often leads to initial treatment failure, and many patients experience recurrent bleeding within the following month. Recurrent posterior epistaxis should be treated with local electrocautery or endoscopic ligation of the sphenopalatine artery to reduce patient discomfort, hospital stay, risk of treatment failure and recurrence.

  14. Psychosocial adjustment to recurrent cancer.

    Science.gov (United States)

    Mahon, S M; Cella, D F; Donovan, M I

    1990-01-01

    This descriptive study of the perceptions and needs of people with recurrent malignancies asks three questions: How do patients describe the meaning of a recurrence of cancer? Do individuals perceive the diagnosis of recurrence and the initial diagnosis of cancer differently? What are the key psychosocial problems associated with recurrent cancer? The theoretical framework was based on Lazarus and Folkman's theory of stress, appraisal, and coping. Subjects completed the Impact of Event Scale (IES), the Psychosocial Adjustment to Illness Scale--Self-Report (PAIS), and a semistructured qualitative interview. The interview elicited perceptions of the event of recurrence and differences between the diagnosis of recurrence and the initial diagnosis. The convenience sample included 40 patients diagnosed with recurrent cancer within the last 30 days. Many subjects (78%) reported that the recurrence was more upsetting than the initial diagnosis. Scores on both the IES and the PAIS were high when compared to normative samples of patients with cancer suggesting that this sample of patients experienced a lot of psychological distress as well as problems at home, work, and in their social lives. These concerns often were unknown to caregivers. Although more research is needed, the authors propose that, with more accurate assessment, more effective intervention could be implemented and the quality of life improved for patients with recurrent cancer.

  15. Epidemiology of recurrent venous thrombosis

    Directory of Open Access Journals (Sweden)

    D.D. Ribeiro

    2012-01-01

    Full Text Available Venous thrombosis, including deep vein thrombosis and pulmonary embolism, is a common disease that frequently recurs. Recurrence can be prevented by anticoagulants, but this comes at the risk of bleeding. Therefore, assessment of the risk of recurrence is important to balance the risks and benefits of anticoagulant treatment. This review briefly outlines what is currently known about the epidemiology of recurrent venous thrombosis, and focuses in more detail on potential new risk factors for venous recurrence. The general implications of these findings in patient management are discussed.

  16. Recurrent pregnancy loss

    DEFF Research Database (Denmark)

    Egerup, P; Kolte, A M; Larsen, E C

    2016-01-01

    STUDY QUESTION: Is there a different prognostic impact for consecutive and non-consecutive early pregnancy losses in women with secondary recurrent pregnancy loss (RPL)? SUMMARY ANSWER: Only consecutive early pregnancy losses after the last birth have a statistically significant negative prognostic...... impact in women with secondary RPL. WHAT IS KNOWN ALREADY: The risk of a new pregnancy loss increases with the number of previous pregnancy losses in patients with RPL. Second trimester losses seem to exhibit a stronger negative impact than early losses. It is unknown whether the sequence of pregnancy...... losses plays a role for the prognosis in patients with a prior birth. STUDY DESIGN, SIZE, DURATION: This retrospective cohort study of pregnancy outcome in patients with unexplained secondary RPL included in three previously published, Danish double-blinded placebo-controlled trials of intravenous...

  17. Recurrent vulvovaginal candidiasis.

    Science.gov (United States)

    Sobel, Jack D

    2016-01-01

    Recurrent vulvovaginal candidiasis (RVVC) is a common cause of significant morbidity in women in all strata of society affecting millions of women worldwide. Previously, RVVC occurrence was limited by onset of menopause but the widespread use of hormone replacement therapy has extended the at-risk period. Candida albicans remains the dominant species responsible for RVVC, however optimal management of RVVC requires species determination and effective treatment measures are best if species-specific. Considerable progress has been made in understanding risk factors that determine susceptibility to RVVC, particularly genetic factors, as well as new insights into normal vaginal defense immune mechanisms and their aberrations in RVVC. While effective control of RVVC is achievable with the use of fluconazole maintenance suppressive therapy, cure of RVVC remains elusive especially in this era of fluconazole drug resistance. Vaccine development remains a critical challenge and need.

  18. Serum, liver, and lung levels of the major extracellular matrix components at the early stage of BCG-induced granulomatosis depending on the infection route.

    Science.gov (United States)

    Kim, L B; Shkurupy, V A; Putyatina, A N

    2015-01-01

    Experiments on the model of mouse BCG-induced granulomatous showed that the content of glycosaminoglycans and proteoglycans in the extracellular matrix of the liver and lungs are changed at the early stages of inflammation (days 3 and 30 postinfection) before cell destruction in the organs begins. This is related to degradation of extracellular matrix structures. Their high content in the blood and interstitium probably contributes to the formation of granulomas, fibroblast proliferation and organ fibrosis. These processes depend on the infection route that determines different conditions for generalization of the inflammation process. Intravenous method of vaccine injection is preferable to use when designing the experiments simulating tuberculosis granulomatosis, especially for the analysis of its early stages.

  19. Poliangeitis amb Granulomatosis eosinofílica i asma al·lèrgica; dues monedes amb una mateixa cara?

    OpenAIRE

    Ramentol Sintas, Marc

    2013-01-01

    La síndrome de Churg-Strauss o Poliangeitis amb Granulomatosis eosinofílica (EGPA), clàssicament s'ha vinculat a un substrat asmàtic d'origen al·lèrgic. No obstant, l'evidència en aquest sentit és equívoca. L'objectiu d'aquest treball és identificar diferències clíniques i serològiques entre pacients amb EGPA i pacients asmàtics al·lèrgics, i determinar el perfil al·lèrgic d'un grup de pacients diagnosticats d'EGPA. Es comparen dos grups de pacients: un grup de diagnosticats d'EGPA, i un altr...

  20. Nocturnal lagophthalmos and recurrent erosion.

    Science.gov (United States)

    Sturrock, G. D.

    1976-01-01

    The symptoms and corneal changes caused by sleeping with one or both eyes open are described in 102 patients. The clinical picture is identical to that of the microform recurrent erosion. The close relationship between the micro- and macro-forms of recurrent corneal erosion suggests that the latter condition is also precipitated by nocturnal lagophthalmos. Images PMID:1268178

  1. Recurrent dermatitis from jellyfish envenomation.

    OpenAIRE

    Menahem, S; Shvartzman, P.

    1994-01-01

    Jellyfish envenomation can cause an immediate local skin reaction, which is usually a painful linear vesiculourticarial eruption. Persistent, delayed, or recurrent dermatitis is less common. Because jellyfish sting reactions and their management are unfamiliar to family physicians, we describe a case of recurrent local dermatitis after jellyfish envenomation and suggest appropriate treatment.

  2. Fludarabine Phosphate, Melphalan, Total-Body Irradiation, Donor Stem Cell Transplant in Treating Patients With Hematologic Cancer or Bone Marrow Failure Disorders

    Science.gov (United States)

    2016-05-05

    ; Noncontiguous Stage II Marginal Zone Lymphoma; Noncontiguous Stage II Small Lymphocytic Lymphoma; Paroxysmal Nocturnal Hemoglobinuria; Previously Treated Myelodysplastic Syndromes; Primary Myelofibrosis; Recurrent Adult Acute Lymphoblastic Leukemia; Recurrent Adult Acute Myeloid Leukemia; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Grade III Lymphomatoid Granulomatosis; Recurrent Adult Hodgkin Lymphoma; Recurrent Adult Immunoblastic Large Cell Lymphoma; Recurrent Adult Lymphoblastic Lymphoma; Recurrent Adult T-cell Leukemia/Lymphoma; Recurrent Childhood Acute Lymphoblastic Leukemia; Recurrent Childhood Acute Myeloid Leukemia; Recurrent Childhood Anaplastic Large Cell Lymphoma; Recurrent Childhood Grade III Lymphomatoid Granulomatosis; Recurrent Childhood Large Cell Lymphoma; Recurrent Childhood Lymphoblastic Lymphoma; Recurrent Childhood Small Noncleaved Cell Lymphoma; Recurrent Cutaneous T-cell Non-Hodgkin Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Mycosis Fungoides/Sezary Syndrome; Recurrent Small Lymphocytic Lymphoma; Recurrent/Refractory Childhood Hodgkin Lymphoma; Refractory Chronic Lymphocytic Leukemia; Refractory Multiple Myeloma; Relapsing Chronic Myelogenous Leukemia; Secondary Acute Myeloid Leukemia; Secondary Myelodysplastic Syndromes; Splenic Marginal Zone Lymphoma; Stage III Adult Diffuse Small Cleaved Cell Lymphoma; Stage III Adult Immunoblastic Large Cell Lymphoma; Stage III Adult Lymphoblastic Lymphoma; Stage III Grade 1 Follicular Lymphoma; Stage III Grade 2 Follicular Lymphoma; Stage III Grade 3 Follicular Lymphoma; Stage III Mantle Cell Lymphoma; Stage III Marginal Zone Lymphoma; Stage III Small Lymphocytic Lymphoma; Stage IV Adult Burkitt Lymphoma; Stage

  3. Tacrolimus and Mycophenolate Mofetil in Preventing Graft-Versus-Host Disease in Patients Who Have Undergone Total-Body Irradiation With or Without Fludarabine Phosphate Followed by Donor Peripheral Blood Stem Cell Transplant for Hematologic Cancer

    Science.gov (United States)

    2016-01-25

    Stage II Adult Diffuse Mixed Cell Lymphoma; Noncontiguous Stage II Adult Diffuse Small Cleaved Cell Lymphoma; Noncontiguous Stage II Adult Immunoblastic Large Cell Lymphoma; Noncontiguous Stage II Adult Lymphoblastic Lymphoma; Noncontiguous Stage II Grade 1 Follicular Lymphoma; Noncontiguous Stage II Grade 2 Follicular Lymphoma; Noncontiguous Stage II Grade 3 Follicular Lymphoma; Noncontiguous Stage II Mantle Cell Lymphoma; Noncontiguous Stage II Marginal Zone Lymphoma; Noncontiguous Stage II Small Lymphocytic Lymphoma; Noncutaneous Extranodal Lymphoma; Peripheral T-cell Lymphoma; Polycythemia Vera; Post-transplant Lymphoproliferative Disorder; Previously Treated Myelodysplastic Syndromes; Primary Myelofibrosis; Prolymphocytic Leukemia; Recurrent Adult Acute Lymphoblastic Leukemia; Recurrent Adult Acute Myeloid Leukemia; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Grade III Lymphomatoid Granulomatosis; Recurrent Adult Hodgkin Lymphoma; Recurrent Adult Immunoblastic Large Cell Lymphoma; Recurrent Adult Lymphoblastic Lymphoma; Recurrent Adult T-cell Leukemia/Lymphoma; Recurrent Childhood Acute Lymphoblastic Leukemia; Recurrent Childhood Acute Myeloid Leukemia; Recurrent Childhood Anaplastic Large Cell Lymphoma; Recurrent Childhood Grade III Lymphomatoid Granulomatosis; Recurrent Childhood Large Cell Lymphoma; Recurrent Childhood Lymphoblastic Lymphoma; Recurrent Childhood Small Noncleaved Cell Lymphoma; Recurrent Cutaneous T-cell Non-Hodgkin Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Mycosis Fungoides/Sezary Syndrome; Recurrent Small Lymphocytic Lymphoma; Recurrent/Refractory Childhood Hodgkin Lymphoma; Refractory Chronic Lymphocytic Leukemia; Refractory Hairy Cell

  4. Comprometimento da árvore respiratória na granulomatose de Wegener Laryngeal and tracheobronchial involvement in Wegener's granulomatosis

    Directory of Open Access Journals (Sweden)

    Ascedio Jose Rodrigues

    2012-04-01

    Full Text Available INTRODUÇÃO: A granulomatose de Wegener (GW é uma forma de vasculite sistêmica que envolve primariamente as vias aéreas superiores e inferiores e os rins. As manifestações mais frequentes nas vias aéreas são estenose subglótica e inflamações, estenoses da traqueia e dos brônquios. A visualização endoscópica das vias aéreas é a melhor ferramenta para avaliação, diagnóstico e manejo dessas alterações. OBJETIVOS: Descrever as alterações endoscópicas encontradas na mucosa das vias aéreas de um grupo de pacientes com GW submetido à broncoscopia no Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo (HC-FMUSP e relatar as intervenções terapêuticas broncoscópicas utilizadas em alguns casos. MÉTODOS: Foram estudados 15 pacientes com diagnóstico de GW provenientes do Ambulatório de Vasculites da Disciplina de Pneumologia do HC-FMUSP, encaminhados para a realização de broncoscopia no serviço de Endoscopia Respiratória do HC-FMUSP no período de 2003 a 2007. RESULTADOS: Dos 15 pacientes avaliados, 11 eram mulheres (73,33% com idade média de 34 ± 11,5 anos. Foram encontradas alterações das vias aéreas em 80% dos pacientes, e o achado endoscópico mais frequente foi estenose subglótica (n = 6. Realizou-se broncoscopia terapêutica em três pacientes com estenose subglótica e em outros três com estenose brônquica, todos apresentando bons resultados. CONCLUSÃO: A broncoscopia permite diagnóstico, acompanhamento e tratamento das lesões de vias aéreas na GW, constituindo-se um recurso terapêutico pouco invasivo em casos selecionados.INTRODUCTION: Wegener's granulomatosis (WG is a form of systemic vasculitis that involves primarily the upper and lower airways and the kidneys. The most frequent airway manifestations include subglottic stenosis and inflammation, and tracheal and bronchial stenoses. The endoscopic visualization of the airways is the best tool for assessing, diagnosing and

  5. Recurrence plots and recurrence quantification analysis of human motion data

    Science.gov (United States)

    Josiński, Henryk; Michalczuk, Agnieszka; Świtoński, Adam; Szczesna, Agnieszka; Wojciechowski, Konrad

    2016-06-01

    The authors present exemplary application of recurrence plots, cross recurrence plots and recurrence quantification analysis for the purpose of exploration of experimental time series describing selected aspects of human motion. Time series were extracted from treadmill gait sequences which were recorded in the Human Motion Laboratory (HML) of the Polish-Japanese Academy of Information Technology in Bytom, Poland by means of the Vicon system. Analysis was focused on the time series representing movements of hip, knee, ankle and wrist joints in the sagittal plane.

  6. [Chronic recurrent multifocal osteomyelitis].

    Science.gov (United States)

    Schilling, F; Eckardt, A; Kessler, S

    2000-01-01

    The aim of this paper is to give a detailed description of the so-called "chronic recurrent multifocal osteomyelitis" (CRMO). The clinical, radiological and histopathological results of an analysis of 29 cases (15 children/adolescents and 14 adults) are presented and correlated to current data from the literature. We could delinate the following points: 1. CRMO is a systemic aseptic inflammation of the bone marrow (Osteitis), it can occur polytopically and association with pustulous dermatologic symptoms is possible. 2. It is not a rare disease 3. Osteomyelitis is probably "reactive" and a plasma-cell sclerotic process with ist own characteristic histologic three-phase course. 4. We could observe 5 specific types of localization which can be documented by X-ray or bone scan. 5. Accompanying arthritis os often present, especially "sympathetic coxitis". 6. The use of drugs in treatment of CRMO (i.e. azithromycin, calcitonin, and bisphosphonates) is discussed. In conclusion we want to point out, that 1. 99mTC bone scan should always be performed when there is suspicion for CRMO to reveal the pattern of affection, 2. the rheumatologist and dermatologist should be contacted, 3. operation is normally not necessary for treatment of the mostly self-limitin disease, and 4. the term "SAPHO syndrome" should be avoided, further differentiation of the diagnosis is necessary.

  7. Chronic recurrent multifocal osteomyelitis.

    Science.gov (United States)

    Roderick, Marion R; Ramanan, Athimalaipet V

    2013-01-01

    Chronic recurrent multifocal osteomyelitis (CRMO) is an autoinflammatory bone disease occurring primarily in children and adolescents. Episodes of systemic inflammation occur due to immune dysregulation without autoantibodies, pathogens or antigen-specific T cells. CRMO is characterised by the insidious onset of pain with swelling and tenderness over the affected bones. Clavicular involvement was the classical description; however, the metaphyses and epiphyses of long bones are frequently affected. Lesions may occur in any bone, including vertebrae. Characteristic imaging includes bone oedema, lytic areas, periosteal reaction and soft tissue reaction. Biopsies from affected areas display polymorphonuclear leucocytes with osteoclasts and necrosis in the early stages. Subsequently, lymphocytes and plasma cells predominate followed by fibrosis and signs of reactive new bone forming around the inflammation. Diagnosis is facilitated by the use of STIR MRI scanning, potentially obviating the need for biopsy and unnecessary long-term antibiotics due to incorrect diagnosis. Treatment options include non-steroidal anti-inflammatory drugs and bisphosphonates. Biologics have been tried in resistant cases with promising initial results. Gene identification has not proved easy although research in this area continues. Early descriptions of the disease suggested a benign course; however, longer-term follow up shows that it can cause significant morbidity and longer-term disability. Although it has always been thought of as very rare, the prevalence is likely to be vastly underestimated due to poor recognition of the disease.

  8. Recurrent neural collective classification.

    Science.gov (United States)

    Monner, Derek D; Reggia, James A

    2013-12-01

    With the recent surge in availability of data sets containing not only individual attributes but also relationships, classification techniques that take advantage of predictive relationship information have gained in popularity. The most popular existing collective classification techniques have a number of limitations-some of them generate arbitrary and potentially lossy summaries of the relationship data, whereas others ignore directionality and strength of relationships. Popular existing techniques make use of only direct neighbor relationships when classifying a given entity, ignoring potentially useful information contained in expanded neighborhoods of radius greater than one. We present a new technique that we call recurrent neural collective classification (RNCC), which avoids arbitrary summarization, uses information about relationship directionality and strength, and through recursive encoding, learns to leverage larger relational neighborhoods around each entity. Experiments with synthetic data sets show that RNCC can make effective use of relationship data for both direct and expanded neighborhoods. Further experiments demonstrate that our technique outperforms previously published results of several collective classification methods on a number of real-world data sets.

  9. Recurrent Novae in M31

    CERN Document Server

    Shafter, A W; Rector, T A; Schweizer, F; Hornoch, K; Orio, M; Pietsch, W; Darnley, M J; Williams, S C; Bode, M F; Bryan, J

    2014-01-01

    The reported positions of 964 suspected nova eruptions in M31 recorded through the end of calendar year 2013 have been compared in order to identify recurrent nova candidates. To pass the initial screen and qualify as a recurrent nova candidate two or more eruptions were required to be coincident within 0.1', although this criterion was relaxed to 0.15' for novae discovered on early photographic patrols. A total of 118 eruptions from 51 potential recurrent nova systems satisfied the screening criterion. To determine what fraction of these novae are indeed recurrent the original plates and published images of the relevant eruptions have been carefully compared. This procedure has resulted in the elimination of 27 of the 51 progenitor candidates (61 eruptions) from further consideration as recurrent novae, with another 8 systems (17 eruptions) deemed unlikely to be recurrent. Of the remaining 16 systems, 12 candidates (32 eruptions) were judged to be recurrent novae, with an additional 4 systems (8 eruptions) b...

  10. Vulvovaginitis candidiasis recurrence during pregnancy.

    Science.gov (United States)

    Fardiazar, Z; Ronaci, F; Torab, R; Goldust, M

    2012-04-15

    Vulvovaginitis is the most common gynecologic condition seen by practitioners rendering primary care to women. Vulvovaginitis Candidiasis (VVC) is the most common type of vaginitis and this study aimed at specifying VVC recurrence during pregnancy. In this prospective study, 150 pregnant women suffering from vaginal excretion, morsus and itching were studied. Initially, the patients were treated using clotrimazole local cream (5 g) for 7 successive days. After initial treatment, the patients were freely visited once a month until delivery considering vaginitis symptoms and VVC recurrence was examined during pregnancy. Mean age of the understudy mothers was 27.26 +/- 3.76. Mean of recurrence number was 0.17 +/- 0.48 during the first trimester. Mean of recurrence number was 0.92 +/- 0.76 during the second trimester. Mean of recurrence number was 2.16 +/- 0.63 during the third trimester. Statistically significant difference was between recurrences during three trimesters of pregnancy (p < 0.001). There is statistically significant difference between mean number of recurrences during three trimesters of pregnancy.

  11. Imaging for Prostate Cancer Recurrence.

    Science.gov (United States)

    Maurer, Tobias; Eiber, Matthias; Fanti, Stefano; Budäus, Lars; Panebianco, Valeria

    2016-06-01

    Correct identification of metastatic sites in recurrent prostate cancer (PCa) is of crucial importance because it leads to further treatment decisions. To provide an overview on current imaging procedures and their performance in recurrent PCa. Medline search via PubMed was performed with the keywords imaging, recurrent, and prostate cancer as well as more detailed searches including the keywords bone scan, bone scintigraphy, computed tomography, magnetic resonance imaging, positron emission tomography, PET, choline, FDG, prostate-specific membrane antigen, and PSMA, with emphasis on recent literature from 2010 to the present. Non-English published literature was excluded. Abstracts and full-text articles were reviewed and assessed for relevant content. In diagnostic imaging and particularly with newer technologies like positron emission tomography (PET), a profound lack of prospectively designed studies in recurrent PCa has to be noted. In most studies histologic validation has only been performed in a subset of patient cohorts. Heterogeneity of included patient cohorts, lack of standardized assessment, as well as diverging end points, hamper systematic comparison of different image modalities. Thus evidence for currently used imaging in recurrent PCa is only presented descriptively. Computed tomography and magnetic resonance imaging (MRI) as well as bone scintigraphy still represent the standard imaging for recurrent PCa; however, particularly for detection of local recurrence, multiparametric MRI is a valuable imaging modality. PET using choline and particularly tracers against prostate-specific membrane antigen might improve visualization of metastatic lesions. These findings need to be validated in prospective trials. Imaging of recurrent prostate cancer (PCa) is important to guide further treatment. Computed tomography, magnetic resonance imaging, and bone scintigraphy represent the current standard. Positron emission tomography, especially with cancer

  12. Granulomatosis with Polyangiitis (Wegener's)

    Science.gov (United States)

    ... Cryopyrin-Associated Autoinflammatory Syndrome (CAPS) (Juvenile) Dermatomyositis (Juvenile) Familial Mediterranean Fever (Juvenile) Fibromyalgia Giant Cell Arteritis Glucocorticoid-induced Osteoperosis ...

  13. Granulomatosis with Polyangiitis (Wegener's)

    Science.gov (United States)

    ... Pharyngitis, Adenitis Syndrome (Juvenile) Polymyalgia Rheumatica Psoriatic Arthritis Raynaud's Phenomenon Reactive Arthritis Rheumatoid Arthritis Scleroderma Sjogren's Syndrome Spinal Stenosis Spondyloarthritis Systemic Lupus Erythematosus (Juvenile) Takayasu's ...

  14. STDP in recurrent neuronal networks

    Directory of Open Access Journals (Sweden)

    Matthieu Gilson

    2010-09-01

    Full Text Available Recent results about spike-timing-dependent plasticity (STDP in recurrently connected neurons are reviewed, with a focus on the relationship between the weight dynamics and the emergence of network structure. In particular, the evolution of synaptic weights in the two cases of incoming connections for a single neuron and recurrent connections are compared and contrasted. A theoretical framework is used that is based upon Poisson neurons with a temporally inhomogeneous firing rate and the asymptotic distribution of weights generated by the learning dynamics. Different network configurations examined in recent studies are discussed and an overview of the current understanding of STDP in recurrently connected neuronal networks is presented.

  15. Recurrent Wheezing in Infants

    Science.gov (United States)

    Belhassen, Manon; De Blic, Jacques; Laforest, Laurent; Laigle, Valérie; Chanut-Vogel, Céline; Lamezec, Liliane; Brouard, Jacques; Fauroux, Brigitte; de Pouvourville, Gérard; Ginoux, Marine; Van Ganse, Eric

    2016-01-01

    Abstract Recurrent wheezing (RW) has a significant impact on infants, caregivers, and society, but morbidity and related medical resource utilization (MRU) have not been thoroughly explored. The burden of RW needs to be documented with population-based data. The objective was to assess the characteristics, medical management, and MRU of RW infants identified from national claims data. Infants aged from 6 to 24 months, receiving ≥2 dispensations of respiratory drugs within 3 months, and presenting a marker of poor control (index date), were selected. During the 6 months after index date, MRU was described in the cohort and among 3 subgroups with more severe RW, defined as ≥4 dispensations of respiratory drugs, ≥3 dispensations of oral corticosteroids (OCS), or ≥1 hospitalization for respiratory symptoms. A total of 115,489 infants had RW, corresponding to 8.2% of subjects in this age group. During follow-up, 68.7% of infants received inhaled corticosteroids, but only 1.8 U (unit) were dispensed over 6 months, suggesting discontinuous use. Control was mostly inadequate: 61.7% of subjects received OCS, 80.2% antibiotics, and 71.2% short-acting beta-agonists, and medical/paramedical visits were numerous, particularly for physiotherapy. Severe RW concerned 39.0% of the cohort; 32.8% and 11.7% of infants had repeated use of respiratory drugs and OCS, respectively, and 5.5% were hospitalized for respiratory symptoms. In this real-life nation-wide study, RW was common and infants had poor control and high MRU. Interventions are needed to support adequate use of controller therapy, and to improve medical care. PMID:27082618

  16. Opioids and breast cancer recurrence

    DEFF Research Database (Denmark)

    Cronin-Fenton, Deirdre P; Heide-Jørgensen, Uffe; Ahern, Thomas P

    2015-01-01

    BACKGROUND: Opioids may alter immune function, thereby potentially affecting cancer recurrence. The authors investigated the association between postdiagnosis opioid use and breast cancer recurrence. METHODS: Patients with incident, early stage breast cancer who were diagnosed during 1996 through...... 2008 in Denmark were identified from the Danish Breast Cancer Cooperative Group Registry. Opioid prescriptions were ascertained from the Danish National Prescription Registry. Follow-up began on the date of primary surgery for breast cancer and continued until breast cancer recurrence, death......, emigration, 10 years, or July 31, 2013, whichever occurred first. Cox regression models were used to compute hazard ratios and 95% confidence intervals associating breast cancer recurrence with opioid prescription use overall and by opioid type and strength, immunosuppressive effect, chronic use (≥6 months...

  17. Tumoral calcinosis mimicking recurrent osteosarcoma

    National Research Council Canada - National Science Library

    Petscavage, Jonelle M; Richardson, Michael L

    2009-01-01

    We report a case of a tumoral calcinosis mimicking the appearance of recurrent osteosarcoma of the left femur and tibia in a 29-year-old woman with a history of osteosarcoma and chronic renal failure...

  18. Recurrent networks for wave forecasting

    Digital Repository Service at National Institute of Oceanography (India)

    Mandal, S.; Prabaharan, N.

    , merchant vessel routing, nearshore construction, etc. more efficiently and safely. This paper presents an application of the Artificial Neural Network, namely Backpropagation Recurrent Neural Network (BRNN) with rprop update algorithm for wave forecasting...

  19. Recurrence for random dynamical systems

    CERN Document Server

    Marie, Philippe

    2009-01-01

    This paper is a first step in the study of the recurrence behavior in random dynamical systems and randomly perturbed dynamical systems. In particular we define a concept of quenched and annealed return times for systems generated by the composition of random maps. We moreover prove that for super-polynomially mixing systems, the random recurrence rate is equal to the local dimension of the stationary measure.

  20. Recurrence risks in mental retardation.

    OpenAIRE

    Crow, Y J; Tolmie, J L

    1998-01-01

    Despite improvements in diagnostic techniques and progress made in mapping genes associated with syndromal mental handicap, the estimation of recurrence risks in non-syndromal mental retardation is still dependent on empirical data. Unfortunately, few studies are available to guide the clinician and their results differ significantly. For example, recurrence risks to all sibs of a male index patient with severe mental retardation vary between 3.5% and 14% in commonly quoted series. The presen...

  1. The recurrent carpal tunnel syndrome.

    Science.gov (United States)

    Kern, B C; Brock, M; Rudolph, K H; Logemann, H

    1993-01-01

    Sixteen out of 720 patients with carpal tunnel syndrome who had undergone surgery since 1979 were reoperated for a "recurrence" (2.2%). Twelve of these patients had been originally operated on in our department. Thus, our own recurrence rate is 1.7%. Three patients deteriorated following surgery, 6 had an unsatisfactory improvement, and in 7 the symptoms recurred after initial improvement. Eight of the reoperated patients had a predisposing disease (terminal renal insufficiency, insulin-dependent diabetes mellitus, acromegaly). In 10 of the 16 cases the initial operation had been carried out by surgeons in the first three years of training. Reoperation revealed incomplete splitting of the transverse carpal ligament in 10 cases, compression of the median nerve by the scar in 4, injury of the muscular branch in 1, and an anatomical variant as cause of incomplete decompression in 1 patient. "Recurrences" after carpal tunnel surgery are predominantly due to inadequacies of the first procedure. A remarkable number of patients (50%) has predisposing diseases. Interfascicular or epineural neurolysis and complete exposure and neurolysis of the median nerve and its branches is necessary only in cases of recurrence. Their omission at the first surgery does not result in an increased recurrence rate. Our observations indicate that the number of operations for recurrent carpal tunnel syndrome can probably be reduced when the first operation is performed with care and experience. Patients with carpal tunnel syndrome secondary to a systemic disease are particularly at risk.

  2. Fludarabine Phosphate, Cyclophosphamide, Tacrolimus, Mycophenolate Mofetil, Total-Body Irradiation, and Donor Bone Marrow Transplant in Treating Patients With High-Risk Hematologic Cancer

    Science.gov (United States)

    2014-02-27

    Accelerated Phase Chronic Myelogenous Leukemia; Adult Acute Lymphoblastic Leukemia in Remission; Adult Acute Myeloid Leukemia in Remission; Adult Acute Myeloid Leukemia With 11q23 (MLL) Abnormalities; Adult Acute Myeloid Leukemia With Del(5q); Adult Acute Myeloid Leukemia With Inv(16)(p13;q22); Adult Acute Myeloid Leukemia With t(15;17)(q22;q12); Adult Acute Myeloid Leukemia With t(16;16)(p13;q22); Adult Acute Myeloid Leukemia With t(8;21)(q22;q22); Adult Nasal Type Extranodal NK/T-cell Lymphoma; Anaplastic Large Cell Lymphoma; Angioimmunoblastic T-cell Lymphoma; Childhood Acute Lymphoblastic Leukemia in Remission; Childhood Acute Myeloid Leukemia in Remission; Childhood Burkitt Lymphoma; Childhood Chronic Myelogenous Leukemia; Childhood Myelodysplastic Syndromes; Childhood Nasal Type Extranodal NK/T-cell Lymphoma; Cutaneous B-cell Non-Hodgkin Lymphoma; de Novo Myelodysplastic Syndromes; Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Hematopoietic/Lymphoid Cancer; Hepatosplenic T-cell Lymphoma; Intraocular Lymphoma; Nodal Marginal Zone B-cell Lymphoma; Peripheral T-cell Lymphoma; Post-transplant Lymphoproliferative Disorder; Previously Treated Myelodysplastic Syndromes; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Grade III Lymphomatoid Granulomatosis; Recurrent Adult Hodgkin Lymphoma; Recurrent Adult Immunoblastic Large Cell Lymphoma; Recurrent Adult Lymphoblastic Lymphoma; Recurrent Adult T-cell Leukemia/Lymphoma; Recurrent Childhood Anaplastic Large Cell Lymphoma; Recurrent Childhood Grade III Lymphomatoid Granulomatosis; Recurrent Childhood Large Cell Lymphoma; Recurrent Childhood Lymphoblastic Lymphoma; Recurrent Childhood Small Noncleaved Cell Lymphoma; Recurrent Cutaneous T-cell Non-Hodgkin Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma

  3. Clinical analysis of 10 children with Wegener granulomatosis%儿童韦格纳肉芽肿10例临床分析

    Institute of Scientific and Technical Information of China (English)

    袁林; 陈植; 张桂菊; 沈颖

    2009-01-01

    Objective To investigate the characteristics of Wegener granulomatosis in children and to improve its recognition. Methods Ten pediatric patients with Wegener granulomatosis were studied retrospectively by clinical manifestations, serum examination, pathological data, treatment, etc. Results All children had clinical evidence of upper respiratory tract and lungs" involvement; kidney lesion occurred in 6 cases(6/10); joints, skin, eyes and nervous system were also involved with different degree, cANCA (PR3) showed positive in 8 cases; Pathological examination (1 kidney sample, 2 nasal mucosa samples, 2 skin samples) showed granulomatous vasculitis and crescents were found in renal biopsy; 7 cases were treated with corticosteroid plus cyclophosphamide, 1 case was treated with corticosteroid plus methotrexate, and improvement were seen in all of them. Conclusions Wegener granulomatosis in children have diverse clinical manifestations and clinical diagnosis is difficult. The upper and lower respiratory tract and kidney are most commonly involved organs. ANCA inspection is characteristic. Treatment with corticosteroid and immunosuppressive can achieve good outcome.%目的 分析儿童韦格纳肉芽肿的特点,提高对该病的认识.方法 对10例韦格纳肉芽肿患儿的临床表现、辅助检查、病理结果、治疗等资料进行总结分析.结果 10例患儿中上呼吸道、肺脏受累10例,肾脏受累6例,关节、皮肤、眼及神经系统等也有不同程度受累.实验室检查胞浆型抗中性粒细胞胞浆抗体(cANCA)阳性8例;5例行病理检查(肾脏1例,鼻黏膜2例,皮肤2例),均表现血管炎和(或)肉芽肿性改变,肾活检有新月体形成.7例患儿给予糖皮质激素(甲基泼尼松龙冲击)联合环磷酰胺治疗,1例进行糖皮质激素联合氨甲蝶呤治疗,患儿临床表现均有不同程度的改善.结论 儿童韦格纳肉芽肿临床表现多样,误诊率高;主要累及呼吸道及肾脏,ANCA检查有特

  4. Recurrence of anxiety disorders and its predictors

    NARCIS (Netherlands)

    Scholten, Willemijn D.; Batelaan, Neeltje M.; van Balkom, Anton J. L. M.; Penninx, Brenda; Smit, Johannes H.; van Oppen, Patricia

    Background: The chronic course of anxiety disorders and its high burden of disease are partly due to the recurrence of anxiety disorders after remission. However, knowledge about recurrence rates and predictors of recurrence is scarce. This article reports on recurrence rates of anxiety disorders

  5. Recurrence of anxiety disorders and its predictors

    NARCIS (Netherlands)

    Scholten, Willemijn D.; Batelaan, Neeltje M.; van Balkom, Anton J. L. M.; Penninx, Brenda; Smit, Johannes H.; van Oppen, Patricia

    2013-01-01

    Background: The chronic course of anxiety disorders and its high burden of disease are partly due to the recurrence of anxiety disorders after remission. However, knowledge about recurrence rates and predictors of recurrence is scarce. This article reports on recurrence rates of anxiety disorders an

  6. An Undecidable Nested Recurrence Relation

    CERN Document Server

    Celaya, Marcel

    2012-01-01

    Roughly speaking, a recurrence relation is nested if it contains a subexpression of the form ... A(...A(...)...). Many nested recurrence relations occur in the literature, and determining their behavior seems to be quite difficult and highly dependent on their initial conditions. A nested recurrence relation A(n) is said to be undecidable if the following problem is undecidable: given a finite set of initial conditions for A(n), is the recurrence relation calculable? Here calculable means that for every n >= 0, either A(n) is an initial condition or the calculation of A(n) involves only invocations of A on arguments in {0,1,...,n-1}. We show that the recurrence relation A(n) = A(n-4-A(A(n-4)))+4A(A(n-4)) +A(2A(n-4-A(n-2))+A(n-2)). is undecidable by showing how it can be used, together with carefully chosen initial conditions, to simulate Post 2-tag systems, a known Turing complete problem.

  7. RECURRENT NOVAE IN M31

    Energy Technology Data Exchange (ETDEWEB)

    Shafter, A. W. [Department of Astronomy, San Diego State University, San Diego, CA 92182 (United States); Henze, M. [European Space Astronomy Centre, P.O. Box 78, E-28692 Villanueva de la Cañada, Madrid (Spain); Rector, T. A. [Department of Physics and Astronomy, University of Alaska Anchorage, 3211 Providence Dr., Anchorage, AK 99508 (United States); Schweizer, F. [Carnegie Observatories, 813 Santa Barbara St., Pasadena, CA 91101 (United States); Hornoch, K. [Astronomical Institute, Academy of Sciences, CZ-251 65 Ondřejov (Czech Republic); Orio, M. [Astronomical Observatory of Padova (INAF), I-35122 Padova (Italy); Pietsch, W. [Max Planck Institute for Extraterrestrial Physics, P.O. Box 1312, Giessenbachstr., D-85741, Garching (Germany); Darnley, M. J.; Williams, S. C.; Bode, M. F. [Astrophysics Research Institute, Liverpool John Moores University, Liverpool L3 5RF (United Kingdom); Bryan, J., E-mail: aws@nova.sdsu.edu [McDonald Observatory, Austin, TX 78712 (United States)

    2015-02-01

    The reported positions of 964 suspected nova eruptions in M31 recorded through the end of calendar year 2013 have been compared in order to identify recurrent nova (RN) candidates. To pass the initial screen and qualify as a RN candidate, two or more eruptions were required to be coincident within 0.′1, although this criterion was relaxed to 0.′15 for novae discovered on early photographic patrols. A total of 118 eruptions from 51 potential RN systems satisfied the screening criterion. To determine what fraction of these novae are indeed recurrent, the original plates and published images of the relevant eruptions have been carefully compared. This procedure has resulted in the elimination of 27 of the 51 progenitor candidates (61 eruptions) from further consideration as RNe, with another 8 systems (17 eruptions) deemed unlikely to be recurrent. Of the remaining 16 systems, 12 candidates (32 eruptions) were judged to be RNe, with an additional 4 systems (8 eruptions) being possibly recurrent. It is estimated that ∼4% of the nova eruptions seen in M31 over the past century are associated with RNe. A Monte Carlo analysis shows that the discovery efficiency for RNe may be as low as 10% that for novae in general, suggesting that as many as one in three nova eruptions observed in M31 arise from progenitor systems having recurrence times ≲100 yr. For plausible system parameters, it appears unlikely that RNe can provide a significant channel for the production of Type Ia supernovae.

  8. Nivolumab With or Without Varlilumab in Treating Patients With Relapsed or Refractory Aggressive B-cell Lymphomas

    Science.gov (United States)

    2017-03-13

    Activated B-Cell-Like Diffuse Large B-Cell Lymphoma; ALK-Positive Large B-Cell Lymphoma; Atypical Burkitt/Burkitt-Like Lymphoma; Diffuse Large B-Cell Lymphoma Associated With Chronic Inflammation; Diffuse Large B-Cell Lymphoma, Not Otherwise Specified; Epstein-Barr Virus Positive Diffuse Large B-Cell Lymphoma of the Elderly; Epstein-Barr Virus-Positive Mucocutaneous Ulcer; Germinal Center B-Cell-Like Diffuse Large B-Cell Lymphoma; High-Grade B-Cell Lymphoma With MYC and BCL2 and/or BCL6 Rearrangements; Human Herpesvirus-8-Positive Neoplastic Cells Present; Intravascular Large B-Cell Lymphoma; MYC-Negative B-Cell Lymphoma With 11q Aberration Resembling Burkitt Lymphoma; Plasmablastic Lymphoma; Primary Cutaneous Diffuse Large B-Cell Lymphoma; Primary Cutaneous Diffuse Large B-Cell Lymphoma, Leg Type; Primary Diffuse Large B-Cell Lymphoma of the Central Nervous System; Primary Effusion Lymphoma; Recurrent Adult Burkitt Lymphoma; Recurrent Diffuse Large B-Cell Lymphoma; Recurrent Lymphomatoid Granulomatosis; Recurrent Mediastinal (Thymic) Large B-Cell Cell Lymphoma; Refractory Burkitt Lymphoma; Refractory Diffuse Large B-Cell Lymphoma; Refractory Mediastinal (Thymic) Large B-Cell Cell Lymphoma; Skin Ulcer; Small Intestinal B-Cell Lymphoma, Unclassifiable, With Features Intermediate Between Diffuse Large B-Cell Lymphoma and Burkitt Lymphoma; T-Cell/Histiocyte-Rich Large B-Cell Lymphoma

  9. Recurrence Statistics of Great Earthquakes

    CERN Document Server

    Ben-Naim, E; Johnson, P A

    2013-01-01

    We investigate the sequence of great earthquakes over the past century. To examine whether the earthquake record includes temporal clustering, we identify aftershocks and remove those from the record. We focus on the recurrence time, defined as the time between two consecutive earthquakes. We study the variance in the recurrence time and the maximal recurrence time. Using these quantities, we compare the earthquake record with sequences of random events, generated by numerical simulations, while systematically varying the minimal earthquake magnitude Mmin. Our analysis shows that the earthquake record is consistent with a random process for magnitude thresholds 7.0<=Mmin<=8.3, where the number of events is larger. Interestingly, the earthquake record deviates from a random process at magnitude threshold 8.4<=Mmin<= 8.5, where the number of events is smaller; however, this deviation is not strong enough to conclude that great earthquakes are clustered. Overall, the findings are robust both qualitat...

  10. Retreatment of recurrent nasopharyngeal carcinoma

    Energy Technology Data Exchange (ETDEWEB)

    McNeese, M.D.; Fletcher, G.H.

    1981-01-01

    Thirty patients with recurrent primary nasopharyngeal carcinoma were reirradiated between 1949 and 1976. Twenty eventually demonstrated recurrence at or near the primary site, involving the nasopharynx in 4 and the central nervous system in 16. Long-term palliation was often achieved, and there were no severe complications except for one patient who died of necrosis of the base of the skull. The most frequent problems were hearing loss and trismus; necrosis of the nasopharynx was seen in only 2 patients. With therapy in the range of 18-25 MeV, significant palliation and an occational cure can be achieved without excessive risk. Recurrent disease involving the skull may be controlled for several years using current techniques.

  11. Recurrence in coined quantum walks

    Energy Technology Data Exchange (ETDEWEB)

    Kiss, T; Kecskes, L [Research Institute for Solid State Physics and Optics, Hungarian Academy of Sciences, Konkoly-Thege M. u. 29-33, H-1121 Budapest (Hungary); Stefanak, M; Jex, I [Department of Physics, FJFI CVUT v Praze, Brehova 7, 115 19 Praha 1-Stare Mesto (Czech Republic)], E-mail: tkiss@szfki.hu

    2009-07-15

    Recurrence of quantum walks on lattices can be characterized by the generalized Polya number. Its value reflects the difference between a classical and a quantum system. The dimension of the lattice is not a unique parameter in the quantum case; both the coin operator and the initial quantum state of the coin influence the recurrence in a nontrivial way. In addition, the definition of the Polya number involves measurement of the system. Depending on how measurement is included in the definition, the recurrence properties vary. We show that in the limiting case of frequent, strong measurements, one can approach the classical dynamics. Comparing various cases, we have found numerical indication that our previous definition of the Polya number provides an upper limit.

  12. Recurrent Novae — A Review

    Directory of Open Access Journals (Sweden)

    K. Mukai

    2015-02-01

    Full Text Available In recent years, recurrent nova eruptions are often observed very intensely in wide range of wavelengths from radio to optical to X-rays. Here I present selected highlights from recent multi-wavelength observations. The enigma of T Pyx is at the heart of this paper. While our current understanding of CV and symbiotic star evolution can explain why certain subset of recurrent novae have high accretion rate, that of T Pyx must be greatly elevated compared to the evolutionary mean. At the same time, we have extensive data to be able to estimate how the nova envelope was ejected in T Pyx, and it turns to be a rather complex tale. One suspects that envelope ejection in recurrent and classical novae in general is more complicated than the textbook descriptions. At the end of the review, I will speculate that these two may be connected.

  13. [Therapy of recurrent cervical cancer].

    Science.gov (United States)

    Sekiba, K; Hayase, R

    1990-09-01

    1,122 uterine cancer patients above FIGO stage I a were treated at our hospital in the decade from 1980 to 1989. Total 69 patients diagnosed as recurrent cervical cancer had 82 lesions in 11 sites. The most frequent recurrent sites were uterus, vaginal stump and vaginal wall; the second sites were parametrium and pelvic wall. Radiotherapy and hyperthermia were done to patients with these lesions. Chemotherapy and radiotherapy were used to patients with distant metastatic lesions to left supraclavicular lymph nodes and lung. But there has been no good results.

  14. Galactosemia presenting as recurrent sepsis.

    Science.gov (United States)

    Rathi, Narendra; Rathi, Akanksha

    2011-12-01

    Galactosemia is a treatable metabolic disorder caused by the deficiency of enzyme galactose-1-phosphate uridyl transferase (GALT) and inherited as an autosomal recessive trait. A case of neonate manifesting with recurrent Escherichia coli sepsis is presented here which turned out to be a classic galactosemia. No other common presenting features were observed in this infant except cataract on slit lamp examination. To the best of our knowledge, there is no case of galactosemia reported in literature which presented with recurrent neonatal sepsis without hepatomegaly, hyperbilirubinemia, bleeding disorder, vomiting, diarrhea, failure to thrive, hypoglycemia, coagulopathy, hemolysis or renal tubular acidosis.

  15. Sunitinib Malate in Treating HIV-Positive Patients With Cancer Receiving Antiretroviral Therapy

    Science.gov (United States)

    2014-03-14

    -transplant Lymphoproliferative Disorder; Previously Treated Myelodysplastic Syndromes; Primary Myelofibrosis; Primary Systemic Amyloidosis; Progressive Hairy Cell Leukemia, Initial Treatment; Prolymphocytic Leukemia; Recurrent Adult Acute Lymphoblastic Leukemia; Recurrent Adult Acute Myeloid Leukemia; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Grade III Lymphomatoid Granulomatosis; Recurrent Adult Hodgkin Lymphoma; Recurrent Adult Immunoblastic Large Cell Lymphoma; Recurrent Adult Lymphoblastic Lymphoma; Recurrent Adult T-cell Leukemia/Lymphoma; Recurrent Cutaneous T-cell Non-Hodgkin Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Mycosis Fungoides/Sezary Syndrome; Recurrent Renal Cell Cancer; Recurrent Small Lymphocytic Lymphoma; Refractory Chronic Lymphocytic Leukemia; Refractory Hairy Cell Leukemia; Refractory Multiple Myeloma; Relapsing Chronic Myelogenous Leukemia; Stage IV Renal Cell Cancer; T-cell Large Granular Lymphocyte Leukemia; Testicular Lymphoma; Unspecified Adult Solid Tumor, Protocol Specific; Waldenström Macroglobulinemia

  16. Oncolytic HSV-1716 in Treating Younger Patients With Refractory or Recurrent High Grade Glioma That Can Be Removed By Surgery

    Science.gov (United States)

    2016-05-26

    Recurrent Childhood Anaplastic Astrocytoma; Recurrent Childhood Anaplastic Oligoastrocytoma; Recurrent Childhood Anaplastic Oligodendroglioma; Recurrent Childhood Giant Cell Glioblastoma; Recurrent Childhood Glioblastoma; Recurrent Childhood Gliomatosis Cerebri; Recurrent Childhood Gliosarcoma

  17. Evaluation of automated multi-parametric indirect immunofluorescence assays to detect anti-neutrophil cytoplasmic antibodies (ANCA) in granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA).

    Science.gov (United States)

    Csernok, Elena; Damoiseaux, Jan; Rasmussen, Niels; Hellmich, Bernhard; van Paassen, Pieter; Vermeersch, Pieter; Blockmans, Daniel; Cohen Tervaert, Jan-Willem; Bossuyt, Xavier

    2016-07-01

    The aim of this multicenter EUVAS study was to evaluate the diagnostic performance of multi-parametric indirect immunofluorescence (IIF) assays to detect anti-neutrophil cytoplasmic antibodies (ANCA) in granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA). The study included 912 samples from diseased controls and 249 diagnostic samples from GPA (n=183) and MPA (n=66) patients. The performance of two automated multi-parametric assays [Aklides (Medipan/Generic Assays) and EuroPattern (Euroimmun)] combining IIF on cellular and purified antigen substrates was compared with two manual IIF analyses and with commercially available ELISAs for MPO- and PR3-ANCA (Euroimmun). The area under the curve (AUC) of the receiver operating characteristics (ROC) curve to discriminate AAV from controls was 0.925, 0.848, 0.855 and 0.904 for, respectively, the two manual analyses, Aklides and EuroPattern, and 0.959, 0.921 and 0.886 for, respectively, antigen-specific ELISA, antigen-coated beads, and microdot, respectively. Variation in pattern assignment between IIF methods was observed. The performance of IIF depends on the substrate used and the definition of IIF patterns. The performance of automated IIF is improved by multi-parameter testing (combined IIF and antigen-specific testing). Given the variability between IIF methods, the diagnostic importance of this technique is questioned. Copyright © 2016. Published by Elsevier B.V.

  18. Eosinophilic Granulomatosis With Polyangiitis With Thrombotic Microangiopathy: Is Simultaneous Systemic Lupus Erythematosus Associated With Clinical Manifestations?: A Case Report and Review of the Literature.

    Science.gov (United States)

    Fukui, Shoichi; Iwamoto, Naoki; Tsuji, Sosuke; Umeda, Masataka; Nishino, Ayako; Nakashima, Yoshikazu; Suzuki, Takahisa; Horai, Yoshiro; Koga, Tomohiro; Kawashiri, Shin-Ya; Ichinose, Kunihiro; Hirai, Yasuko; Tamai, Mami; Nakamura, Hideki; Origuchi, Tomoki; Kinoshita, Naóe; Kawakami, Atsushi

    2015-11-01

    Eosinophilic granulomatosis with polyangiitis (EGPA) is one of the antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis, which is characterized by vasculitis of the small to medium-sized vessels. On the contrary, thrombotic microangiopathy (TMA) is a life-threatening condition which can cause ischemic organ injury. Although several case reports have described patients with TMA associated with ANCA-associated vasculitis except for EGPA, there are no previous case reports of EGPA associated with TMA.A 71-year-old Japanese man was diagnosed with EGPA based on his asthma, eosinophilia, lung opacity, refractory sinusitis, and positive myeloperoxidase-ANCA. He was also diagnosed with TMA based on peripheral schizocytes and hemolytic anemia. We performed plasmapheresis and started high-dose corticosteroid therapy; thereafter, he improved promptly. His case also fulfilled the classification criteria of systemic lupus erythematosus (SLE) based on the pleural effusion, renal disorder, anemia, thrombocytopenia, positive antidouble-stranded DNA antibody, and low complement. Elements of SLE were thought to affect his clinical course.We reviewed 11 patients with EGPA or hypereosinophilic syndrome (HES) associated with SLE, including our case. Patients with EGPA or HES associated with SLE had more heart complications than patients with simple EGPA or simple HES did. Patients with EGPA or HES associated with SLE had more pleural effusion than patients with simple SLE did.Clinical manifestations of eosinophilia with SLE or SLE with eosinophilia may differ from simple SLE or simple eosinophilia.

  19. 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.

  20. Recurrence quantification analysis of chimera states

    Energy Technology Data Exchange (ETDEWEB)

    Santos, M.S. [Pós-Graduação em Ciências/Física, Universidade Estadual de Ponta Grossa, 84030-900, Ponta Grossa, PR (Brazil); Szezech, J.D., E-mail: jdanilo@gmail.com [Departamento de Matemática e Estatística, Universidade Estadual de Ponta Grossa, 84030-900, Ponta Grossa, PR (Brazil); Batista, A.M., E-mail: antoniomarcosbatista@gmail.com [Departamento de Matemática e Estatística, Universidade Estadual de Ponta Grossa, 84030-900, Ponta Grossa, PR (Brazil); Caldas, I.L. [Instituto de Física, Universidade de São Paulo, 05315-970, São Paulo, SP (Brazil); Viana, R.L.; Lopes, S.R. [Departamento de Física, Universidade Federal do Paraná, 81531-990, Curitiba, PR (Brazil)

    2015-10-02

    Chimera states, characterised by coexistence of coherence and incoherence in coupled dynamical systems, have been found in various physical systems, such as mechanical oscillator networks and Josephson-junction arrays. We used recurrence plots to provide graphical representations of recurrent patterns and identify chimera states. Moreover, we show that recurrence plots can be used as a diagnostic of chimera states and also to identify the chimera collapse. - Highlights: • Chimera states have been found in various physical systems. • Recurrence plots is a graphical method useful to locate recurring patterns. • We used recurrence plots to identify the chimera states. • We show also the recurrence plots can identify the chimera collapse.

  1. [Local recurrence of paratesticular rhabdomyosarcoma].

    Science.gov (United States)

    Rabii, R; Moufid, K; Fekak, H; Dassouli, B; Joual, A; Bennani, S; el Mrini, M; Benjelloun, S

    2002-10-01

    We report an uncommon case of scrotal recurrence of embryonal paratesticular rhabdomyosarcoma in 19 year old man after 3 years later. The diagnosis was suspected clinically and confirmed by histopathology study after resection of the scrotal tumor. About this case, the authors discuss the diagnosis and the management of this tumor.

  2. [Recurrent pigmented villonodular synovitis (PVNS)].

    Science.gov (United States)

    Neuss, M; Hermanns, B; Wirtz, D C

    2001-01-01

    The pigmented vilionodular synovitis (PVNS) is a tumour like disease of unknown origin that often shows recurrence. The pathogenesis is still unknown and therefore the question of the right therapy is not resolved. With a case report of a patient with recurrence after two arthroscopic synovectomies, PVNS is discussed against the background of the clinical, histological, and radiological features. We performed an open synovectomy and cystic lesions in both condyles of the femur and proximal tibia were filled with homologous and autologous cancellous bone. Three months later the patient had no pain and the bone density in the former cystic lesions was appropriate. The pathogenesis is still unknown. Diagnosis often is obtained much too late due to missing specific symptoms. PVNS occurs in local forms as well as in a diffuse growth pattern. Recurrence rates of up to 78% are very high. Besides arthroscopic and open synovectomy, the treatment with radiosynoviorthesis must be considered. Depending on the growth pattern, the tumour masses, and the affected joint, the therapy has to be chosen very carefully and sometimes different forms have to be combined if a recurrence--free result is to be achieved.

  3. Interpretation of Recurrent Neural Networks

    DEFF Research Database (Denmark)

    Pedersen, Morten With; Larsen, Jan

    1997-01-01

    This paper addresses techniques for interpretation and characterization of trained recurrent nets for time series problems. In particular, we focus on assessment of effective memory and suggest an operational definition of memory. Further we discuss the evaluation of learning curves. Various nume...

  4. Relativistic phase space dimensional recurrences

    CERN Document Server

    Delbourgo, Robert

    2003-01-01

    We derive recurrence relations between phase space expressions in different dimensions by confining some of the coordinates to tori or spheres of radius $R$ and taking the limit as $R \\to \\infty$. These relations take the form of mass integrals, associated with extraneous momenta (relative to the lower dimension), and produce the result in the higher dimension.

  5. Recurrence Formulas for Fibonacci Sums

    CERN Document Server

    Brandao, Adilson J V

    2008-01-01

    In this article we present a new recurrence formula for a finite sum involving the Fibonacci sequence. Furthermore, we state an algorithm to compute the sum of a power series related to Fibonacci series, without the use of term-by-term differentiation theorem

  6. Clostridium difficile recurrences in Stockholm.

    Science.gov (United States)

    Sandell, Staffan; Rashid, Mamun-Ur; Jorup-Rönström, Christina; Ellström, Kristina; Nord, Carl Erik; Weintraub, Andrej

    2016-04-01

    Sixty-eight hospital-admitted patients with a first episode of Clostridium difficile infection (CDI) were included and followed up during 1 year. Faeces samples were collected at 1, 2, 6 and 12 months after inclusion and analyzed for the presence of C. difficile toxin B, genes for toxin A, toxin B, binary toxin and TcdC deletion by PCR. All strains were also PCR-ribotyped and the MICs of the isolates were determined against eight antimicrobial agents. In 68 patients initially included, antibiotics, clinical signs and co-morbidities were analyzed and 56 were evaluable for recurrences. The mean number of different antibiotics given during 3 months prior to inclusion was 2.6 (range 0-6). Six patients had not received any antibiotics and three of them had diagnosed inflammatory bowel disease. Thirty-two patients (57%) had either a microbiological or clinical recurrence, 16 of whom had clinical recurrences that were confirmed microbiologically (13, 23%) or unconfirmed by culture (3, 5%). Twenty-nine patients were positive in at least one of the follow-up tests, 16 had the same ribotype in follow-up tests, i.e. relapse, and 13 a different ribotype, i.e., reinfection. Most common ribotypes were 078/126, 020, 023, 026, 014/077, 001 and 005. No strain of ribotype 027 was found. Strains ribotype 078/126 and 023 were positive for binary toxin and were the strains most prone to cause recurrence. All strains were sensitive to vancomycin and metronidazole. Patients with recurrences were significantly older (p = 0.02) and all patients had a high burden of comorbidities, which could explain the high fatality rate, 26 (38%) patients died during the 1-year follow-up. Copyright © 2016 Elsevier Ltd. All rights reserved.

  7. Recurrent periductal mastitis: Surgical treatment.

    Science.gov (United States)

    Taffurelli, Mario; Pellegrini, Alice; Santini, Donatella; Zanotti, Simone; Di Simone, Domenico; Serra, Margherita

    2016-12-01

    Recurrent periductal mastitis is a benign breast disorder that often features a mammary fistula that runs between periareolar skin and the ductal mammary system. Due to the high recurrence rate of this disease, its management is controversial. This study was designed to assess the efficacy of fistulectomy (Hadfield operation), particularly with regard to its long-term outcome. We reviewed all women with recurrent periductal mastitis who underwent the Hadfield operation in the Breast Center in S.Orsola-Malpighi Hospital (Bologna University) from 2005 to 2015. All but one of the patients were heavy smokers and presented with a recurrent periareolar abscess and a periareolar mammary fistula. Eighteen women underwent the Hadfield surgical treatment. Mean age at the time of presentation was 42 years; 17 of 18 women smoked >10 cigarettes/d. All patients had a breast ultrasonography or mammography. Half of the patients had undergone antibiotic therapy with one or more prior abscess drainages or another form of operative treatment. All patients who underwent operative treatment had no postoperative events and were satisfied with the cosmetic results. Squamous metaplasia was always present in the specimens. After a median follow-up of 36 months, 2 patients developed a recurrence after a few months; neither had stopped smoking. Based on our review of the literature and taking into account the results of this study, it seems clear that the best treatment involves a combined total excision of the affected duct and the fistulous tract. Due to the important role of smoking in this disease, it is important to encourage patients to stop smoking. Copyright © 2016 Elsevier Inc. All rights reserved.

  8. [Thoracoscopic treatment of recurrent pneumothorax].

    Science.gov (United States)

    Suter, M; Berner, M; Vandoni, R; Cuttat, J F

    1994-04-01

    Spontaneous pneumothorax (PNO) is usually due to rupture of a small subpleural bleb into the pleural cavity and affects mainly young men. After simple drainage, recurrence occurs in about 50% of cases. The risk of recurrence increases after each new PNO. Secondary PNO complicates an underlying pulmonary disease, especially chronic obstructive pulmonary disease with emphysema. A new form of secondary PNO has emerged in the recent years in AIDS patients with pneumocystis carinii pneumonia. We have shifted to a thoracoscopic therapy of PNO since May 1991. 25 PNO in 24 patients (1 bilateral) have been treated since that time up to April 1993. 19 PNO were primary, whereas 6 were secondary, included 3 iatrogenic PNO. Resection of the leaking parenchymal area was performed in 20 patients, and parietal partial pleurectomy was done in 20 cases. In the remaining cases, fibrin glue was applied on the lesion and in 3 cases, chemical pleurodesis was attempted using silver nitrate or talc. 1 AIDS patient died of ARDS. 3 patients had recurrent PNO and had thoracotomy without complication. 21 patients did well. Partial PNO recurred in one of them 4 months later, and was treated by simple needle aspiration. Thoracoscopy is a useful method to treat recurrent or persistent spontaneous PNO. After only 25 cases, our success rate in primary PNO is 90%. There should be a learning curve. On the basis of our experience, we believe that recognition of the lesion and its resection as well as apical parietal pleurectomy are necessary to obtain good results and a low recurrence rate.

  9. Giant recurrent retroperitoneal liposarcoma presenting as a recurrent inguinal hernia

    Directory of Open Access Journals (Sweden)

    Ajay H. Bhandarwar

    2011-11-01

    Full Text Available Retroperitoneal liposarcoma presenting as an inguinal hernia is a rare entity. We present the first case of Giant recurrent liposarcoma presenting as a recurrent inguinal hernia in a 40-year-old male. Physical examination showed an irreducible lump in the right inguinal region and a scar in the right lumbar and right inguinal region. Computed tomography (CT scan of abdomen revealed it to be a retro peritoneal mass extending into the right inguinal region along and involving the cord structures. Wide local excision of the tumour with right orchidectomy and inguinal hernioplasty was performed. Histo-pathology confirmed it to be a liposarcoma. Patient received postoperative radio therapy. Follow up of two years has shown him to be disease free. Retroperitoneal liposarcoma can grow along cord structures into the inguinal canal and mimic an irreducible indirect inguinal hernia.

  10. Temperature, age, and recurrence of febrile seizure

    NARCIS (Netherlands)

    M. van Stuijvenberg (Margriet); E.W. Steyerberg (Ewout); G. Derksen-Lubsen (Gerarda); H.A. Moll (Henriëtte)

    1998-01-01

    textabstractOBJECTIVE: Prediction of a recurrent febrile seizure during subsequent episodes of fever. DESIGN: Study of the data of the temperatures, seizure recurrences, and baseline patient characteristics that were collected at a randomized placebo controlled trial of ibuprofen s

  11. Prevention of postoperative recurrence of Crohn's disease

    NARCIS (Netherlands)

    van Loo, E. S.; Dijkstra, G.; Ploeg, R. J.; Nieuwenhuijs, V. B.

    2012-01-01

    Background: Up to 75% of patients with Crohn's disease (CD) will have intestinal resection during their life. Most patients will, however, develop postoperative recurrence (endoscopic, clinical or surgical). Several medical and surgical strategies have been attempted to prevent postoperative recurre

  12. Temperature, age, and recurrence of febrile seizure

    NARCIS (Netherlands)

    M. van Stuijvenberg (Margriet); E.W. Steyerberg (Ewout); G. Derksen-Lubsen (Gerarda); H.A. Moll (Henriëtte)

    1998-01-01

    textabstractOBJECTIVE: Prediction of a recurrent febrile seizure during subsequent episodes of fever. DESIGN: Study of the data of the temperatures, seizure recurrences, and baseline patient characteristics that were collected at a randomized placebo controlled trial of ibuprofen

  13. Recurrent facial urticaria following herpes simplex labialis.

    Science.gov (United States)

    Zawar, Vijay; Godse, Kiran

    2012-03-01

    We describe recurrent acute right-sided facial urticaria associated with herpes labialis infection in a middle-aged female patient. Antiviral medications and antihistamines not only successfully cleared the herpes infection and urticaria but also prevented further recurrences.

  14. Recurrent facial urticaria following herpes simplex labialis

    OpenAIRE

    Vijay Zawar; Kiran Godse

    2012-01-01

    We describe recurrent acute right-sided facial urticaria associated with herpes labialis infection in a middle-aged female patient. Antiviral medications and antihistamines not only successfully cleared the herpes infection and urticaria but also prevented further recurrences.

  15. Sjogren's Syndrome: Can It Cause Recurrent UTIs?

    Science.gov (United States)

    ... it cause recurrent UTIs? Since being diagnosed with Sjogren's syndrome last year, I've had three urinary tract infections. Is there any evidence that Sjogren's syndrome causes recurrent UTIs? Answers from April Chang-Miller, ...

  16. Fifth International Symposium on Recurrence Plot

    CERN Document Server

    Riley, Michael; Giuliani, Alessandro; Webber, Charles; Jr, Jr; Translational Recurrences : From Mathematical Theory to Real-World Applications

    2014-01-01

    This book features 13 papers presented at the Fifth International Symposium on Recurrence Plots, held August 2013 in Chicago, IL. It examines recent applications and developments in recurrence plots and recurrence quantifi cation analysis (RQA) with special emphasis on biological and cognitive systems and the analysis of coupled systems using cross-recurrence methods. Readers will discover new applications and insights into a range of systems provided by recurrence plot analysis and new theoretical and mathematical developments in recurrence plots. Recurrence plot based analysis is a powerful tool that operates on real-world complex systems that are nonlinear, non-stationary, noisy, of any statistical distribution, free of any particular model type, and not particularly long. Quantitative analyses promote the detection of system state changes, synchronized dynamical regimes, or classifi cation of system states. Th e book will be of interest to an interdisciplinary audience of recurrence plot users and researc...

  17. Recurrent femoral hernia and associated ovarian pathology.

    Science.gov (United States)

    Gately, Ryan Patrick; Concannon, Elizabeth Sarah; Hogan, A; Ryan, R S; O'Leary, M; Barry, K

    2012-08-27

    The following case describes an ovarian tumour presenting in a highly unusual manner-in the form of a recurrent femoral hernia. Recurrent femoral herniae are unusual and should prompt awareness of underlying pathology causing increased intra-abdominal pressure.

  18. Recurrent anterior uveitis in Henoch Schonlein's vasculitis.

    Science.gov (United States)

    Erer, Burak; Kamali, Sevil; Cingu, Kursat; Kilicaslan, Isin; Gul, Ahmet; Inanc, Murat; Aral, Orhan; Tugal-Tutkun, Ilknur

    2010-08-01

    Uveitis is an important component of many rheumatic diseases. The main causes of recurrent uveitis are seronegative spondylarthropathies and Behçet's disease. We describe a rare case of Henoch Schönlein vasculitis (HSV) along with multiple recurrences of acute anterior uveitis. In cases of skin rash and recurrent anterior uveitis, HSV should be considered in the differential diagnosis.

  19. Recurrent cervical cancer : detection and prognosis

    NARCIS (Netherlands)

    Duyn, A; Van Eijkeren, M; Kenter, G; Zwinderman, K; Ansink, A

    2002-01-01

    Background. Only a small proportion of cervical cancer recurrences is detected during routine follow-up. We investigated which percentage of recurrences is detected during follow-up, which diagnostic tools are helpful to detect recurrent disease and which factors are of prognostic significance once

  20. 14 CFR 135.351 - Recurrent training.

    Science.gov (United States)

    2010-01-01

    ... 14 Aeronautics and Space 3 2010-01-01 2010-01-01 false Recurrent training. 135.351 Section 135.351... AND ON DEMAND OPERATIONS AND RULES GOVERNING PERSONS ON BOARD SUCH AIRCRAFT Training § 135.351 Recurrent training. (a) Each certificate holder must ensure that each crewmember receives recurrent...

  1. 14 CFR 121.427 - Recurrent training.

    Science.gov (United States)

    2010-01-01

    ... 14 Aeronautics and Space 3 2010-01-01 2010-01-01 false Recurrent training. 121.427 Section 121.427..., FLAG, AND SUPPLEMENTAL OPERATIONS Training Program § 121.427 Recurrent training. (a) Recurrent training must ensure that each crew member or dispatcher is adequately trained and currently proficient...

  2. 14 CFR 91.1107 - Recurrent training.

    Science.gov (United States)

    2010-01-01

    ... 14 Aeronautics and Space 2 2010-01-01 2010-01-01 false Recurrent training. 91.1107 Section 91.1107... Management § 91.1107 Recurrent training. (a) Each program manager must ensure that each crewmember receives recurrent training and is adequately trained and currently proficient for the type aircraft and...

  3. Segmentalliverincarcerationthrougha recurrent incisional lumbar hernia

    Institute of Scientific and Technical Information of China (English)

    Nikolaos S. Salemis; Konstantinos Nisotakis; Stavros Gourgiotis; Efstathios Tsohataridis

    2007-01-01

    BACKGROUND: Lumbar hernia is a rare congenital or acquired defect of the posterior abdominal wall. The acquired type is more common and occurs mainly as an incisional defect after lfank surgery. Incarceration or strangulation of hernia contents is uncommon. METHOD: Segmental liver incarceration through a recurrent incisional lumbar defect was diagnosed in a 58 years old woman by magnetic resonance imaging. RESULTS: The patient underwent an open repair of the com-plicated hernia. An expanded polytetralfouoroethylene (e-PTFE) mesh was fashioned as a sublay prosthesis. She had an uncomplicated postoperative course. Follow-up examinations revealed no evidence of recurrence. CONCLUSIONS: Although lumbar hernia rarely results in incarceration or strangulation, early repair is necessary because of the risks of complications and the increasing dififculty in repairment as it enlarges. Surgical repair is often dififcult and challenging.

  4. Multicentric synchronous recurrent aggressive fibromatosis

    Directory of Open Access Journals (Sweden)

    Kavita Kohli

    2012-01-01

    Full Text Available Extra-abdominal desmoid tumors are known as aggressive fibromatosis (AFM. Synchronous and metachronous multicentric aggressive fibromatosis are rare lesions and pose dilemma in diagnosis and management. A rare and interesting case of recurrent multicentric, synchronous AFM is presented which to the best of our knowledge has not been reported earlier. A young male presented with well defined, hard, fixed swelling on the thigh. Resected tumor mass on histopathology was diagnosed as an extra abdominal fibromatosis. He presented again after two months with swelling at the same site; and two more swellings on the foot. Fine needle aspiration cytology (FNAC from all three sites was performed; and was suggestive of benign spindle cell lesion of fibrogenic origin with the possibility of multicentric synchronous recurrent aggressive fibromatosis.

  5. Clopidogrel-Induced Recurrent Polyarthritis

    Directory of Open Access Journals (Sweden)

    Sahil Agrawal MD

    2013-08-01

    Full Text Available Clopidogrel is an oral thienopyridine and together with aspirin is a component of dual antiplatelet therapy for the prevention of stent thrombosis after intracoronary stent placement. The common adverse effects from its use are an increased risk of bleeding, neutropenia, and rash. Arthralgia and backache are also known to occur with its use. There have been case reports linking arthritis with the use of clopidogrel. We describe the case of a 64-year-old man who reported symptoms of fever and joint pains following initiation of therapy with clopidogrel. Acute-phase reactants were elevated. Laboratory and radiologic testing were unremarkable. Incidentally, he reported experiencing a similar arthritis after he received a loading dose of clopidogrel prior to a diagnostic coronary angiography in the past. The symptoms improved dramatically on discontinuation of clopidogrel. There was no recurrence of symptoms with prasugrel. This describes possibly the second incidence of recurrent arthritis with clopidogrel therapy.

  6. Training recurrent networks by Evolino.

    Science.gov (United States)

    Schmidhuber, Jürgen; Wierstra, Daan; Gagliolo, Matteo; Gomez, Faustino

    2007-03-01

    In recent years, gradient-based LSTM recurrent neural networks (RNNs) solved many previously RNN-unlearnable tasks. Sometimes, however, gradient information is of little use for training RNNs, due to numerous local minima. For such cases, we present a novel method: EVOlution of systems with LINear Outputs (Evolino). Evolino evolves weights to the nonlinear, hidden nodes of RNNs while computing optimal linear mappings from hidden state to output, using methods such as pseudo-inverse-based linear regression. If we instead use quadratic programming to maximize the margin, we obtain the first evolutionary recurrent support vector machines. We show that Evolino-based LSTM can solve tasks that Echo State nets (Jaeger, 2004a) cannot and achieves higher accuracy in certain continuous function generation tasks than conventional gradient descent RNNs, including gradient-based LSTM.

  7. Clopidogrel-Induced Recurrent Polyarthritis

    Science.gov (United States)

    Harburger, Joseph; Stallings, Gary; Agrawal, Nikhil; Garg, Jalaj

    2013-01-01

    Clopidogrel is an oral thienopyridine and together with aspirin is a component of dual antiplatelet therapy for the prevention of stent thrombosis after intracoronary stent placement. The common adverse effects from its use are an increased risk of bleeding, neutropenia, and rash. Arthralgia and backache are also known to occur with its use. There have been case reports linking arthritis with the use of clopidogrel. We describe the case of a 64-year-old man who reported symptoms of fever and joint pains following initiation of therapy with clopidogrel. Acute-phase reactants were elevated. Laboratory and radiologic testing were unremarkable. Incidentally, he reported experiencing a similar arthritis after he received a loading dose of clopidogrel prior to a diagnostic coronary angiography in the past. The symptoms improved dramatically on discontinuation of clopidogrel. There was no recurrence of symptoms with prasugrel. This describes possibly the second incidence of recurrent arthritis with clopidogrel therapy. PMID:26425581

  8. Recurrent bleeding after perimesencephalic hemorrhage.

    Science.gov (United States)

    Kauw, Frans; Velthuis, Birgitta K; Kizilates, Ufuk; van der Schaaf, Irene C; Rinkel, Gabriel J E; Vergouwen, Mervyn D I

    2017-08-31

    Perimesencephalic hemorrhage (PMH) is a type of subarachnoid hemorrhage with excellent long-term outcomes. Only one well-documented case of in-hospital rebleeding after PMH is described in the literature, which occurred after initiating antithrombotic treatment because of myocardial ischemia. In this case report we describe a patient with PMH without antithrombotic treatment who had two episodes of recurrent bleeding on the day of ictus. In order to validate the radiological findings we conducted a case-control study. Six neuroradiologists and two neuroradiology fellows performed a blinded assessment of serial unenhanced head CT scans of eight patients with a perimesencephalic bleeding pattern (1 index patient, 6 patients with PMH, 1 patient with a perimesencephalic bleeding pattern and basilar artery aneurysm) to investigate a potential increase in amount of subarachnoid blood. A 56-year-old woman with a perimesencephalic bleeding pattern and negative CT angiography had after the onset headache two episodes with a sudden increase of the headache. Blinded assessment of serial head CTs of eight patients with a perimesencephalic bleeding pattern identified the patient who was clinically suspected to have two episodes of recurrent bleeding to have an increased amount of subarachnoid blood on two subsequent CT scans. Recurrent bleeding after PMH may also occur in patients not treated with antithrombotics. Even after early rebleeding, prognosis of PMH is excellent. Copyright © 2017 Elsevier Inc. All rights reserved.

  9. Unfavorably Altered Fibrin Clot Properties in Patients with Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome: Association with Thrombin Generation and Eosinophilia.

    Directory of Open Access Journals (Sweden)

    Lucyna Mastalerz

    Full Text Available Given reports on the increased prevalence of thromboembolic incidents in patients with eosinophilic granulomatosis with polyangiitis (EGPA; Churg-Strauss syndrome, we investigated whether fibrin clot properties are unfavorably altered in EGPA.Ex vivo plasma fibrin clot characteristics, including clot permeability, turbidimetry and efficiency of fibrinolysis using two assays, were investigated in 34 consecutive patients with remission in EGPA according to the Birmingham Vasculitis Activity Score version 3 (23 female, 11 male, aged 48 (range, 21-80 years. The control group comprised 34 age- and sex- matched volunteers.Compared with controls, patients with EGPA were characterized by denser fiber clots (estimated pore size, Ks, 7.30±0.93 vs 10.14±1.07 10-9 cm2, faster fibrin polymerization (lag phase in a turbidimetric curve, 41.8±3.6 vs 47.4±2.9 s, thicker fibrin fibers (maximum absorbance, ΔAbs, 0.87±0.09 vs 0.72±0.07, higher maximum levels of D-dimer released from clots (DDmax 4.10±0.46 vs 3.54±0.35 mg/L, and prolonged clot lysis time (t50%; 9.50±1.45 vs 7.56±0.87 min; all p<0.0001. Scanning electron microscopy images confirmed denser plasma fibrin networks composed of thinner fibers formed in EGPA. Antineutrophil cytoplasmic antibody status and C-reactive protein did not affect clot variables. Multivariate analysis adjusted for fibrinogen showed that Ks was predicted by eosinophil count, peak thrombin generation, factor VIII, and soluble CD40 ligand, whereas eosinophil count, peak thrombin generation and antiplasmin predicted t50%.This study is the first to show that EGPA is associated with prothrombotic plasma fibrin clot phenotype, which may contribute to thromboembolic manifestations reported in this disease.

  10. Paquimeningite hipertrófica e parotidite: manifestações raras da granulomatose de Wegener Hypertrophic pachymeningitis and parotiditis: unusual ocurrences of the Wegener's granulomatosis

    Directory of Open Access Journals (Sweden)

    Ana Paula Monteiro Gomides

    2004-08-01

    Full Text Available A granulomatose de Wegener é uma vasculite sistêmica dos vasos de médio e pequeno calibre. Classicamente, há formação de granulomas com necrose no trato respiratório e glomerulonefrite necrosante. Embora seu acometimento mais comum envolva o trato respiratório superior, pulmões e rins, uma vasta gama de manifestações em vários órgãos e tecidos é descrita. Relatamos o caso de um paciente que, paralelamente às manifestações típicas da doença, desenvolveu algumas alterações raras como a parotidite e a paquimeningite hipertrófica. Diante de quadros graves, atípicos e/ou refratários ao tratamento convencional, torna-se necessário o aprofundamento no estudo e uso de novas armas terapêuticas.The wegener's granulomatosis is a systemic angiitis of small and medium caliber vessels. Normally, it is characterized by the formation of granulomas with necrosis in the respiratory system and necrosing glomerulonephritis. even though it is more common in the upper respiratory system, in the lung and in the kidney, a great range of manifestation in other different organs and tissues can be described. we report the case of a patient who, in parallel with the typical symptoms of this disease, developed some rare alteration such as the parotiditis and the hypertrophic pachymeningitis. in face of serious situations, severe disease, atypical or non responsive to conventional treatments, it is necessary a more detailed study of the case and the use of new therapeutic drugs.

  11. Erlotinib and Temozolomide in Treating Young Patients With Recurrent or Refractory Solid Tumors

    Science.gov (United States)

    2013-06-04

    Previously Treated Childhood Rhabdomyosarcoma; Recurrent Childhood Brain Tumor; Recurrent Childhood Cerebellar Astrocytoma; Recurrent Childhood Cerebral Astrocytoma; Recurrent Childhood Ependymoma; Recurrent Childhood Malignant Germ Cell Tumor; Recurrent Childhood Medulloblastoma; Recurrent Childhood Rhabdomyosarcoma; Recurrent Childhood Soft Tissue Sarcoma; Recurrent Childhood Supratentorial Primitive Neuroectodermal Tumor; Recurrent Neuroblastoma; Recurrent Osteosarcoma

  12. Perda auditiva neurossensorial em pacientes com granulomatose de Wegener: relato de três casos e revisão de literatura Neurosensory hearing loss in patients with Wegener's granulomatosis: a report of three cases and literature review

    Directory of Open Access Journals (Sweden)

    Ana Paula Monteiro Gomides

    2006-06-01

    Full Text Available A granulomatose de Wegener é uma vasculite sistêmica que acomete vasos de pequeno e médio calibres. As manifestações clássicas ocorrem no trato respiratório superior, inferior e rins, mas outros órgãos podem ser envolvidos. O sistema auditivo pode ser freqüentemente acometido nas suas várias porções (orelha externa, média e/ou interna com manifestações diversas. A perda auditiva neurossensorial pode ser conseqüência de alterações na orelha interna e deve ser precocemente reconhecida. O tratamento rápido e efetivo pode evitar danos irreversíveis. Descrevemos três casos de granulomatose de Wegener e hipoacusia sensorial, com respostas diferentes ao tratamento.Wegener's granulomatosis is a primary systemic vasculitis that affects small and medium-sized vessels. The classical manifestations occur in the upper and lower respiratory tract and in the kidneys, but other organs may be involved. The auditory system can be frequently affect in its various portions (outer, middle and/or inner ear, showing different manifestations. Neurosensory hearing loss might be a consequence of changes in the inner ear and should be recognized early. Rapid and effective treatment may prevent irreversible damage. We report herein three cases of Wegener's granulomatosis and sensory hypoacusis, which responded differently to treatment.

  13. Sperm DNA fragmentation, recurrent implantation failure and recurrent miscarriage

    Directory of Open Access Journals (Sweden)

    Carol Coughlan

    2015-01-01

    Full Text Available Evidence is increasing that the integrity of sperm DNA may also be related to implantation failure and recurrent miscarriage (RM. To investigate this, the sperm DNA fragmentation in partners of 35 women with recurrent implantation failure (RIF following in vitro fertilization, 16 women diagnosed with RM and seven recent fathers (control were examined. Sperm were examined pre- and post-density centrifugation by the sperm chromatin dispersion (SCD test and the terminal deoxynucleotidyl transferase dUTP nick end labeling (TUNEL assay. There were no significant differences in the age of either partner or sperm concentration, motility or morphology between three groups. Moreover, there were no obvious differences in sperm DNA fragmentation measured by either test. However, whilst on average sperm DNA fragmentation in all groups was statistically lower in prepared sperm when measured by the SCD test, this was not seen with the results from the TUNEL assay. These results do not support the hypothesis that sperm DNA fragmentation is an important cause of RIF or RM, or that sperm DNA integrity testing has value in such patients. It also highlights significant differences between test methodologies and sperm preparation methods in interpreting the data from sperm DNA fragmentation tests.

  14. Sperm DNA fragmentation, recurrent implantation failure and recurrent miscarriage.

    Science.gov (United States)

    Coughlan, Carol; Clarke, Helen; Cutting, Rachel; Saxton, Jane; Waite, Sarah; Ledger, William; Li, Tinchiu; Pacey, Allan A

    2015-01-01

    Evidence is increasing that the integrity of sperm DNA may also be related to implantation failure and recurrent miscarriage (RM). To investigate this, the sperm DNA fragmentation in partners of 35 women with recurrent implantation failure (RIF) following in vitro fertilization, 16 women diagnosed with RM and seven recent fathers (control) were examined. Sperm were examined pre- and post-density centrifugation by the sperm chromatin dispersion (SCD) test and the terminal deoxynucleotidyl transferase dUTP nick end labeling (TUNEL) assay. There were no significant differences in the age of either partner or sperm concentration, motility or morphology between three groups. Moreover, there were no obvious differences in sperm DNA fragmentation measured by either test. However, whilst on average sperm DNA fragmentation in all groups was statistically lower in prepared sperm when measured by the SCD test, this was not seen with the results from the TUNEL assay. These results do not support the hypothesis that sperm DNA fragmentation is an important cause of RIF or RM, or that sperm DNA integrity testing has value in such patients. It also highlights significant differences between test methodologies and sperm preparation methods in interpreting the data from sperm DNA fragmentation tests.

  15. Recurrent Hodgkin's disease after bone marrow transplantation.

    Science.gov (United States)

    Shahab, I; Greer, J P; Beeker, T A; Wolff, S N; Collins, R D; Cousar, J B

    1997-01-01

    Histologic features of recurrent Hodgkin's disease (HD) after conventional therapy are well known, but few studies describe HD after bone marrow transplantation (BMT). Histologic material from 63 patients who underwent BMT performed to treat recurrent nodular sclerosing HD (NSHD) between 1985 and 1994 was examined; 13 of the 63 patients had histologically proved recurrent disease after BMT. Histologic material and clinical findings from the original diagnostic biopsy specimen and pre-BMT and post-BMT specimens were available from our study population of eight patients (five male, three female; age range, 16 to 38 years; median age, 27.5 years). Seven patients had recurrent NSHD after BMT; sites of recurrence included lymph nodes only (four patients), and lymph nodes and lung, lung and liver, and lung only (one patient each). In one patient, a high-grade non-Hodgkin's B-cell lymphoma developed in the large intestine 5 years after BMT. In another, disease progressed from grade 1 in the original biopsy specimen to grade 2 in both the pre-BMT and post-BMT recurrent HD biopsy specimens. Post-BMT biopsy specimens of recurrent HD with lung involvement revealed a substantial increase in sclerosis and fibroblastic features. Paraffin immunoperoxidase studies in seven patients demonstrated substantial change in phenotype of Reed-Stemberg cell variants in only one post-BMT recurrent HD specimen, which showed a +2 reaction with CD30 (Ki-1). No substantial differences in the reactive component were noted between the original biopsy specimen and pre-BMT and post-BMT specimens of recurrent disease. In summary, histologic findings of post-BMT recurrent NSHD do not differ significantly from those of the original diagnostic biopsy or pre-BMT recurrent HD specimens. The lung is the most common site of extranodal post-BMT recurrence. In one patient, high-grade non-Hodgkin's B-cell lymphoma developed after BMT performed to treat recurrent HD.

  16. Recurrent glomerular disease after kidney transplantation.

    Science.gov (United States)

    Blosser, Christopher D; Bloom, Roy D

    2017-08-21

    With improving short-term kidney transplant outcomes, recurrent glomerular disease is being increasingly recognized as an important cause of chronic allograft failure. Further understanding of the risks and pathogenesis of recurrent glomerular disease enable informed transplant decisions, along with the development of preventive and treatment strategies. Multiple observational studies have highlighted differences in rates and outcomes for various recurrent glomerular diseases, although these rates have not markedly improved over the last decade. Emerging evidence supports use of rituximab to treat recurrent primary membranous nephropathy and possibly focal segmental glomerulosclerosis (FSGS), whereas eculizumab is effective in glomerular diseases associated with complement dysregulation [C3 glomerulopathy (C3G) and atypical hemolytic uremic syndrome (aHUS)]. Despite the potential for recurrence in the allograft, transplant remains the optimal therapy for patients with advanced chronic kidney disease (CKD) secondary to primary glomerular disease. Biomarkers and therapeutic options necessitate accurate pretransplant diagnoses with opportunities for improved surveillance and treatment of recurrent glomerular disease posttransplant.

  17. Recurrence of hypertensive disorders of pregnancy

    DEFF Research Database (Denmark)

    van Oostwaard, Miriam F; Langenveld, Josje; Schuit, Ewoud

    2015-01-01

    OBJECTIVE: We performed an individual participant data (IPD) metaanalysis to calculate the recurrence risk of hypertensive disorders of pregnancy (HDP) and recurrence of individual hypertensive syndromes. STUDY DESIGN: We performed an electronic literature search for cohort studies that reported...... that are included in our IPD, the recurrence rate of a HDP was 20.7% (95% CI, 20.4-20.9%). Recurrence manifested as preeclampsia in 13.8% of the studies (95% CI,13.6-14.1%), gestational hypertension in 8.6% of the studies (95% CI, 8.4-8.8%) and hemolysis, elevated liver enzymes and low platelets (HELLP) syndrome...... delivery, and perinatal death. Normotensive women experienced chronic hypertension after pregnancy more often after experiencing recurrence (odds ratio, 3.7; 95% CI, 2.3-6.1). CONCLUSION: Among women that experience hypertension in pregnancy, the recurrence rate in a next pregnancy is relatively low...

  18. Recurrent, giant subcutaneous leiomyosarcoma of the thigh

    Directory of Open Access Journals (Sweden)

    Gao Chuanping, MD

    2015-10-01

    Full Text Available We present a case of recurrent, massive subcutaneous leiomyosarcoma involving the left thigh in a 29-year-old male from Madagascar. The patient had earlier undergone local resection of subcutaneous leiomyosarcoma a half year before. After surgical intervention, local recurrence developed at this site and was rapidly growing. The patient was surgically treated with a 2-cm-wide margin local excision in our hospital. The patient has remained recurrence free at 1-year follow-up.

  19. Is acute recurrent pancreatitis a chronic disease?

    OpenAIRE

    Mariani, Alberto; Testoni, Pier Alberto

    2008-01-01

    Whether acute recurrent pancreatitis is a chronic disease is still debated and a consensus is not still reached as demonstrated by differences in the classification of acute recurrent pancreatitis. There is major evidence for considering alcoholic pancreatitis as a chronic disease ab initio while chronic pancreatitis lesions detectable in biliary acute recurrent pancreatitis (ARP) seem a casual association. Cystic fibrosis transmembrane conductance regulator (CFTR) gene mutation, hereditary a...

  20. Recurrence of gestational diabetes in primiparous women

    DEFF Research Database (Denmark)

    Kruse, Anne R; Darling, Mette S; Hansen, Mia K L

    2015-01-01

    Introduction Gestational diabetes mellitus (GDM) increases the risk for diabetes in the next pregnancy and later in life. Thus, estimating the risk of GDM in further pregnancies provides a time frame for possible preventive measures. We aimed to calculate the recurrence rate of GDM in primiparous...... and the subsequent pregnancy. Conclusions Recurrence of diet-treated GDM was 47.2% in primiparous women with previous GDM and the recurrence was associated with weight gain between pregnancies....

  1. Generalised Recurrence Plot Analysis for Spatial Data

    OpenAIRE

    Marwan, Norbert; Kurths, Juergen; Saparin, Peter

    2006-01-01

    Recurrence plot based methods are highly efficient and widely accepted tools for the investigation of time series or one-dimensional data. We present an extension of the recurrence plots and their quantifications in order to study recurrent structures in higher-dimensional spatial data. The capability of this extension is illustrated on prototypical 2D models. Next, the tested and proved approach is applied to assess the bone structure from CT images of human proximal tibia. We find that the ...

  2. Recurrence quantification analysis of chimera states

    Science.gov (United States)

    Santos, M. S.; Szezech, J. D.; Batista, A. M.; Caldas, I. L.; Viana, R. L.; Lopes, S. R.

    2015-10-01

    Chimera states, characterised by coexistence of coherence and incoherence in coupled dynamical systems, have been found in various physical systems, such as mechanical oscillator networks and Josephson-junction arrays. We used recurrence plots to provide graphical representations of recurrent patterns and identify chimera states. Moreover, we show that recurrence plots can be used as a diagnostic of chimera states and also to identify the chimera collapse.

  3. Faithfulness of Recurrence Plots: A Mathematical Proof

    Science.gov (United States)

    Hirata, Yoshito; Komuro, Motomasa; Horai, Shunsuke; Aihara, Kazuyuki

    It is practically known that a recurrence plot, a two-dimensional visualization of time series data, can contain almost all information related to the underlying dynamics except for its spatial scale because we can recover a rough shape for the original time series from the recurrence plot even if the original time series is multivariate. We here provide a mathematical proof that the metric defined by a recurrence plot [Hirata et al., 2008] is equivalent to the Euclidean metric under mild conditions.

  4. Unique double recurrence of cerebral arteriovenous malformation.

    Science.gov (United States)

    Nagm, Alhusain; Horiuchi, Tetsuyoshi; Ichinose, Shunsuke; Hongo, Kazuhiro

    2015-09-01

    Surgically treated patients with arteriovenous malformations (AVMs) are considered cured when the postoperative angiogram proves complete resection. However, despite no residual nidus or early draining vein on postoperative angiogram, rare instances of AVM recurrence have been reported in adults. In this paper, the authors present a case of a 24-year-old woman with asymptomatic double recurrence of her cerebral AVM after angiographically proven complete resection. To the authors' knowledge, this patient represents the first case with double de novo asymptomatic recurrence of Spetzler-Martin grade I AVM. Also, she represents the first case with unique AVM criteria in each recurrence.

  5. Generalised recurrence plot analysis for spatial data

    Energy Technology Data Exchange (ETDEWEB)

    Marwan, Norbert [Institute of Physics, University of Potsdam, 14415 Potsdam (Germany)]. E-mail: marwan@agnld.uni-potsdam.de; Kurths, Juergen [Institute of Physics, University of Potsdam, 14415 Potsdam (Germany); Saparin, Peter [Department of Biomaterials, Max Planck Institute of Colloids and Interfaces, 14424 Potsdam-Golm (Germany)

    2007-01-08

    Recurrence plot based methods are highly efficient and widely accepted tools for the investigation of time series or one-dimensional data. We present an extension of the recurrence plots and their quantifications in order to study recurrent structures in higher-dimensional spatial data. The capability of this extension is illustrated on prototypical 2D models. Next, the tested and proved approach is applied to assess the bone structure from CT images of human proximal tibia. We find that the spatial structures in trabecular bone become more recurrent during the bone loss in osteoporosis.

  6. Recurrent odontogenic keratocyst within the masticatory space

    Energy Technology Data Exchange (ETDEWEB)

    Lim, Su Yeon; Huh, Kyung Hoe; Yi, Won Jin; Choi, Hyun Bae; Choi, Soon Chul [School of Dentistry, Seoul National University, Seoul (Korea, Republic of)

    2008-06-15

    The odontogenic keratocyst (OKC) is a developmental odontogenic cyst typically occurring in the jaws. Since the first description of OKC was published in 1956, the lesion has been of particular interest because of its specific histopathologic features, high recurrence rate, and aggressive behavior. Recurrences most commonly arise within bone at the site of the original cyst. However, as lining cells may find their way into surrounding tissues either from implantation during surgery or from cortical perforation recurrences may arise at a distance from the original cyst. Here, we report a rare case of recurrent OKC which was first developed in mandible and recurred within the masticatory space.

  7. Treatment of a recurrent ameloblastic fibroma.

    Science.gov (United States)

    Manzon, Steven; Philbert, Rawle F; Bush, Benjamin F; Zola, Malcolm B; Solomon, Marshall

    2015-01-01

    Ameloblastic fibroma (AF), a slow-growing, benign tumor of odontogenic origin, represents 2% of all odontogenic tumors. Jaw expansion is among the most common symptoms, with diagnosis often made through routine radiographs. AFs have a recurrence rate of 18% to 43.5% after conservative enucleation. Long-term follow-up by both the surgeon and referring dentist is recommended, since recurrence may be due to regrowth of residual tumor undergoing malignant transformation. Aggressive management is recommended for local tumor recurrence. En bloc excision with bone grafting, followed by implant reconstruction, can be curative and preservative of function. Treatment of a recurrent AF is described.

  8. Recurrence and Relapse in Bipolar Mood Disorder

    Directory of Open Access Journals (Sweden)

    S Gh Mousavi

    2004-06-01

    Full Text Available Background: Despite the effectiveness of pharmacotherapy in acute phase of bipolar mood disorder, patients often experience relapses or recurrent episodes. Hospitalization of patients need a great deal of financial and humanistic resources which can be saved through understanding more about the rate of relapse and factors affecting this rate. Methods: In a descriptive analytical study, 380 patients with bipolar disorder who were hospitalized in psychiatric emergency ward of Noor hospital, Isfahan, Iran, were followed. Each patient was considered for; the frequency of relapse and recurrence, kind of pharmachotherapy, presence of psychotherapeutic treatments, frequency of visits by psychiatrist and the rank of present episode. Results: The overall prevalence of recurrence was 42.2%. Recurrence was lower in patients using lithium carbonate or sodium valproate or combined therapy (about 40%, compared to those using carbamazepine (80%. Recurrence was higher in patients treated with only pharmacotherapy (44.5% compared to those treated with both pharmacotherapy and psychotherapy (22.2%. Patients who were visited monthy by psychiatrist had lower rate of recurrence compared to those who had irregular visits. Conclusion: The higher rate of recurrence observed in carbamazepine therapy may be due to its adverse reactions and consequently poor compliance to this drug. Lower rates of recurrence with psychotherapy and regular visits may be related to the preventive effects of these procedures and especially to the effective management of stress. Keywords: Bipolar Mood Disorder, Recurrence, Relapse.

  9. Higher antineutrophil cytoplasmic antibody (C-ANCA) titers are associated with increased overall healthcare use in patients with sinonasal manifestations of granulomatosis with polyangiitis (GPA).

    Science.gov (United States)

    Janisiewicz, Agnieszka M; Klau, Marc H; Keschner, David B; Lehmer, Randy R; Venkat, Kumar V; Medhekar, Swati S; Chang, Parke K; Badran, Karam; Leary, Ryan; Garg, Rohit; Nguyen, Andrew A; Lee, Jivianne T

    2015-01-01

    Granulomatosis with polyangiitis (GPA) is an autoimmune disease characterized by necrotizing granulomatous airway inflammation and vasculitis. Sinonasal involvement occurs in more than 80% cases, with antineutrophil cytoplasmic antibody (C-ANCA) titers used as a marker of disease severity. The purpose of this study was to determine whether C-ANCA levels impact radiographic findings and healthcare use in patients with sinonasal GPA. A retrospective review was performed on GPA patients evaluated in a multidisciplinary rheumatologic/otolaryngologic clinic from 2008 to 2013. Data were collected with respect to age, gender, clinical presentation, C-ANCA titers, Lund-Mackay (LM) scores, surgical interventions, and healthcare use, the latter of which were determined by assessing the number of rheumatology/otolaryngology clinic visits, computed tomography (CT) scans, and email/telephone encounters. A total of 44 patients were identified, 11 male and 33 female. Sinonasal manifestations were evident in 70.4%, with chronic rhinosinusitis (CRS) (41.9%), septal perforation (38.7%), and crusting (32.2%) the most common findings. No significant differences in number of CT scans (p = 0.10) or mean LM scores (p = 0.47) were found between patients with more than or equal to 1:80 and less than 1:80 C-ANCA titers, respectively. However, overall healthcare use was increased in the more than or equal to 1:80 C-ANCA group (n = 28) compared with less than 1:80 (n = 16), with a significantly greater number of rheumatologic/otolaryngologic encounters (mean 121 versus 69.2, p = 0.03) noted. When otolaryngologic healthcare use was specifically examined, the average number of encounters was also higher in more than or equal to 1:80 C-ANCA patients (31.9 versus 22.9), but this difference was not statistically significant (p = 0.16). Sinonasal GPA patients with presenting C-ANCA titers more than or equal to 1:80 demonstrated significantly greater overall healthcare use than their lower C

  10. Comparing Presenting Clinical Features in 48 Children With Microscopic Polyangiitis to 183 Children Who Have Granulomatosis With Polyangiitis (Wegener's): An ARChiVe Cohort Study.

    Science.gov (United States)

    Cabral, David A; Canter, Debra L; Muscal, Eyal; Nanda, Kabita; Wahezi, Dawn M; Spalding, Steven J; Twilt, Marinka; Benseler, Susanne M; Campillo, Sarah; Charuvanij, Sirirat; Dancey, Paul; Eberhard, Barbara A; Elder, Melissa E; Hersh, Aimee; Higgins, Gloria C; Huber, Adam M; Khubchandani, Raju; Kim, Susan; Klein-Gitelman, Marisa; Kostik, Mikhail M; Lawson, Erica F; Lee, Tzielan; Lubieniecka, Joanna M; McCurdy, Deborah; Moorthy, Lakshmi N; Morishita, Kimberly A; Nielsen, Susan M; O'Neil, Kathleen M; Reiff, Andreas; Ristic, Goran; Robinson, Angela B; Sarmiento, Angelyne; Shenoi, Susan; Toth, Mary B; Van Mater, Heather A; Wagner-Weiner, Linda; Weiss, Jennifer E; White, Andrew J; Yeung, Rae S M

    2016-10-01

    To uniquely classify children with microscopic polyangiitis (MPA), to describe their demographic characteristics, presenting clinical features, and initial treatments in comparison to patients with granulomatosis with polyangiitis (Wegener's) (GPA). The European Medicines Agency (EMA) classification algorithm was applied by computation to categorical data from patients recruited to the ARChiVe (A Registry for Childhood Vasculitis: e-entry) cohort, with the data censored to November 2015. The EMA algorithm was used to uniquely distinguish children with MPA from children with GPA, whose diagnoses had been classified according to both adult- and pediatric-specific criteria. Descriptive statistics were used for comparisons. In total, 231 of 440 patients (64% female) fulfilled the classification criteria for either MPA (n = 48) or GPA (n = 183). The median time to diagnosis was 1.6 months in the MPA group and 2.1 months in the GPA group (ranging to 39 and 73 months, respectively). Patients with MPA were significantly younger than those with GPA (median age 11 years versus 14 years). Constitutional features were equally common between the groups. In patients with MPA compared to those with GPA, pulmonary manifestations were less frequent (44% versus 74%) and less severe (primarily, hemorrhage, requirement for supplemental oxygen, and pulmonary failure). Renal pathologic features were frequently found in both groups (75% of patients with MPA versus 83% of patients with GPA) but tended toward greater severity in those with MPA (primarily, nephrotic-range proteinuria, requirement for dialysis, and end-stage renal disease). Airway/eye involvement was absent among patients with MPA, because these GPA-defining features preclude a diagnosis of MPA within the EMA algorithm. Similar proportions of patients with MPA and those with GPA received combination therapy with corticosteroids plus cyclophosphamide (69% and 78%, respectively) or both drugs in combination with

  11. Palbociclib Isethionate in Treating Younger Patients With Recurrent, Progressive, or Refractory Central Nervous System Tumors

    Science.gov (United States)

    2016-10-19

    Childhood Choroid Plexus Tumor; Childhood Ependymoblastoma; Childhood Grade III Meningioma; Childhood High-grade Cerebellar Astrocytoma; Childhood High-grade Cerebral Astrocytoma; Childhood Medulloepithelioma; Recurrent Childhood Anaplastic Astrocytoma; Recurrent Childhood Anaplastic Oligoastrocytoma; Recurrent Childhood Anaplastic Oligodendroglioma; Recurrent Childhood Brain Stem Glioma; Recurrent Childhood Cerebellar Astrocytoma; Recurrent Childhood Cerebral Astrocytoma; Recurrent Childhood Giant Cell Glioblastoma; Recurrent Childhood Glioblastoma; Recurrent Childhood Gliomatosis Cerebri; Recurrent Childhood Gliosarcoma; Recurrent Childhood Medulloblastoma; Recurrent Childhood Pineoblastoma; Recurrent Childhood Supratentorial Primitive Neuroectodermal Tumor

  12. Bortezomib and Filgrastim in Promoting Stem Cell Mobilization in Patients With Non-Hodgkin Lymphoma or Multiple Myeloma Undergoing Stem Cell Transplant

    Science.gov (United States)

    2016-04-19

    Adult Grade III Lymphomatoid Granulomatosis; B-cell Chronic Lymphocytic Leukemia; Contiguous Stage II Adult Burkitt Lymphoma; Contiguous Stage II Adult Diffuse Large Cell Lymphoma; Contiguous Stage II Adult Diffuse Mixed Cell Lymphoma; Contiguous Stage II Adult Diffuse Small Cleaved Cell Lymphoma; Contiguous Stage II Adult Immunoblastic Large Cell Lymphoma; Contiguous Stage II Adult Lymphoblastic Lymphoma; Contiguous Stage II Grade 1 Follicular Lymphoma; Contiguous Stage II Grade 2 Follicular Lymphoma; Contiguous Stage II Grade 3 Follicular Lymphoma; Contiguous Stage II Mantle Cell Lymphoma; Contiguous Stage II Marginal Zone Lymphoma; Contiguous Stage II Small Lymphocytic Lymphoma; Cutaneous B-cell Non-Hodgkin Lymphoma; Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Intraocular Lymphoma; Nodal Marginal Zone B-cell Lymphoma; Noncontiguous Stage II Adult Burkitt Lymphoma; Noncontiguous Stage II Adult Diffuse Large Cell Lymphoma; Noncontiguous Stage II Adult Diffuse Mixed Cell Lymphoma; Noncontiguous Stage II Adult Diffuse Small Cleaved Cell Lymphoma; Noncontiguous Stage II Adult Immunoblastic Large Cell Lymphoma; Noncontiguous Stage II Adult Lymphoblastic Lymphoma; Noncontiguous Stage II Grade 1 Follicular Lymphoma; Noncontiguous Stage II Grade 2 Follicular Lymphoma; Noncontiguous Stage II Grade 3 Follicular Lymphoma; Noncontiguous Stage II Mantle Cell Lymphoma; Noncontiguous Stage II Marginal Zone Lymphoma; Noncontiguous Stage II Small Lymphocytic Lymphoma; Progressive Hairy Cell Leukemia, Initial Treatment; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Grade III Lymphomatoid Granulomatosis; Recurrent Adult Immunoblastic Large Cell Lymphoma; Recurrent Adult Lymphoblastic Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular

  13. Recurrence of neuroleptic malignant syndrome.

    Science.gov (United States)

    González-Blanco, Leticia; García-Prada, Hilario; Santamarina, Susana; Jiménez-Treviño, Luis; Bobes, Julio

    2013-01-01

    Neuroleptic malignant syndrome (NMS) is a rare idiosyncratic reaction associated with the use of neuroleptics that has an incidence of 0.02 to 3% among patients taking these drugs. This is a very serious complication with a mortality rate that reaches 10-20%. It is therefore very important to have high clinical suspicion and use appropriate criteria to objectify this clinical picture early, stopping the medication causing the picture and to avoid the subsequent complications as much as possible that would be responsible for both its mortality and sequels. We present that case of an 81-year old woman who was admitted to the Psychiatric Hospitalization Unit (PHU) for a depressive episode with psychotic symptoms who developed a neuroleptic malignant syndrome (NMS) when Haloperidol was introduced. After its suspension and subsequent clinical recovery, antipsychotic treatment with Risperidone was reintroduced and she suffered a recurrence of NMS. Finally, significant improvement was achieved with several sessions of electroshock therapy (EST).

  14. Recurrent pregnancy loss and obesity.

    Science.gov (United States)

    Sugiura-Ogasawara, Mayumi

    2015-05-01

    Recurrent pregnancy loss (RPL) was defined as two or more miscarriages. Antiphospholipid syndrome, uterine anomalies, and parental chromosomal abnormalities, particularly translocation and abnormal embryonic karyotype, are identifiable causes of RPL. Obesity may increase the risk of sporadic miscarriage in pregnancies conceived spontaneously. Obesity with body mass index (BMI)>30 kg/m2 is an independent risk factor for further miscarriage with odds ratio 1.7-3.5 in patients with early RPL. Obesity is associated with euploid miscarriage. Unexplained RPL with euploid embryo might be a common disease caused by both polymorphisms of multiple susceptibility genes and lifestyle factors such as women's age, obesity, and smoking. Patients with a history of RPL were found to have a higher risk of cardiovascular disease, celiac disease, gastric ulcer, gastritis, and atopic dermatitis. No study has examined the effect of weight loss on the prevention of further miscarriage in patients with RPL.

  15. Placental apoptosis in recurrent miscarriage

    Directory of Open Access Journals (Sweden)

    Tarek A. Atia

    2017-09-01

    Full Text Available Apoptosis is an interactive and dynamic biological process involved in all phases of embryogenesis. We aimed to study the effect of placental apoptosis on recurrent miscarriage (RM. Placental tissue samples were collected from 40 women with RM (study group and 30 women with sporadic spontaneous abortion (control group. Samples were prepared and stained immunohistochemically with markers for both the apoptotic protein (p53 and anti-apoptotic Bcl-2 antibodies. Our results showed that expression of the apoptotic (p53 protein was significantly increased in the placental tissues of the RM group (p = 0.003. By contrast, the expression of anti-apoptotic (Bcl-2 antibodies was significantly increased in the placental tissues of the control group (p = 0.025. We concluded that placental apoptosis plays a crucial role in pregnancy continuation. However, increased p53 expression in placental tissue in early pregnancy could negatively affect pregnancy continuation.

  16. Recurrent Glioblastoma: Where we stand

    Directory of Open Access Journals (Sweden)

    Sanjoy Roy

    2015-01-01

    Full Text Available Current first-line treatment regimens combine surgical resection and chemoradiation for Glioblastoma that provides a slight increase in overall survival. Age on its own should not be used as an exclusion criterion of glioblastoma multiforme (GBM treatment, but performance should be factored heavily into the decision-making process for treatment planning. Despite aggressive initial treatment, most patients develop recurrent diseases which can be treated with re-resection, systemic treatment with targeted agents or cytotoxic chemotherapy, reirradiation, or radiosurgery. Research into novel therapies is investigating alternative temozolomide regimens, convection-enhanced delivery, immunotherapy, gene therapy, antiangiogenic agents, poly ADP ribose polymerase inhibitors, or cancer stem cell signaling pathways. Given the aggressive and resilient nature of GBM, continued efforts to better understand GBM pathophysiology are required to discover novel targets for future therapy.

  17. Recurrent Primary Spinal Hydatid Cyst

    Directory of Open Access Journals (Sweden)

    Okan Turk

    2015-03-01

    Full Text Available Primary hydatid disease of spine is rare and spinal hydatitosis constitute only 1% of all hydatitosis. We report a case of recurrent primary intraspinal extradural hydatid cyst of the thoracic region causing progressive paraparesis. The patient was operated 16 years ago for primary spinal hydatid disease involvement and was instrumented dorsally for stabilization. The magnetic resonance imaging (MRI of thoracic spine showed a cystic lesion at T11-12 level and compressed spinal cord posterolaterally. Intraspinal cyst was excised through T11-12 laminectomy which made formerly. The early postoperative period showed a progressive improvement of his neurological deficit and he was discharged with antihelmintic treatment consisting of albendazole and amoxicillin-sulbactam combination. [Cukurova Med J 2015; 40(Suppl 1: 84-89

  18. Atypical recurrence of rheumatic chorea

    Directory of Open Access Journals (Sweden)

    Gunjan Pankaj Kumar

    2015-05-01

    Full Text Available Syndenhams Chorea in acute rheumatic fever is reported to occur in 20-30% of patients. It is usually late onset, occurring upto 6 months after acute infection but may occasionally be present as presenting symptom of rheumatic fever. It is a self-limiting condition with spontaneous remission lasting from 1 week to 6 months. The risk of recurrence is present in 1st 1-2 years in about 20% of cases. Most of children (two thirds with rheumatic fever are of school age (5-15 years of age. It is common in India and the incidence has not shown the declining trends seen in the developing countries. We report the clinical findings, investigations and the course of clinical development of a 14-year-old girl, who presented with Rheumatic chorea which recurred 3 years after the initial episode. [Int J Res Med Sci 2015; 3(5.000: 1272-1273

  19. Pharmacotherapy of recurrent aphthous ulcers

    Directory of Open Access Journals (Sweden)

    J P Angeline Archana

    2011-01-01

    Full Text Available Aphthous ulcer is the most common type of ulcer affecting the oral cavity and is considered to be one of the most painful conditions. Treatment is often unsatisfactory. Newer treatment modalities are therefore being tried. Amlexanox and rebamipide are the approved drugs for painful aphthous ulcers and have been used in painful symptoms of acid peptic disease as prostaglandin enhancers. Safety and efficacy of the drugs used in the treatment of recurrent aphthous ulcers were evaluated and being used widely by most of the treating physicians choosing a modality of treatment of their experience. There is no proper treatment modality available till date. Various drugs and their efficacy with least adverse drug effects while treating the various aphthous ulcers are discussed.

  20. Current management options for recurrent adrenocortical carcinoma

    Directory of Open Access Journals (Sweden)

    Glover AR

    2013-06-01

    Full Text Available Anthony R Glover,1 Julian C Y Ip,1 Jing Ting Zhao,1 Patsy S H Soon,1,4 Bruce G Robinson,1,3 Stan B Sidhu1,2 1Kolling Institute of Medical Research, Cancer Genetics Laboratory, 2Endocrine Surgical Unit, 3Department of Endocrinology, Royal North Shore Hospital and University of Sydney, St Leonards, 4Department of Surgery, Bankstown Hospital and University of New South Wales, Bankstown, NSW, Australia Abstract: Adrenal cortical carcinoma (ACC is a rare cancer that poses a number of management challenges due to the limited number of effective systemic treatments. Complete surgical resection offers the best chance of long-term survival. However, despite complete resection, ACC is associated with high recurrence rates. This review will discuss the management of recurrent ACC in adults following complete surgical resection. Management should take place in a specialist center and treatment decisions must consider the individual tumor biology of each case of recurrence. Given the fact that ACC commonly recurs, management to prevent recurrence should be considered from initial diagnosis with the use of adjuvant mitotane. Close follow up with clinical examination and imaging is important for early detection of recurrent disease. Locoregional recurrence may be isolated, and repeat surgical resection should be considered along with mitotane. The use of radiotherapy in ACC remains controversial. Systemic recurrence most often involves liver, pulmonary, and bone metastasis and is usually managed with mitotane, with or without combination chemotherapy. There is a limited role for surgical resection in systemic recurrence in selected patients. In all patients with recurrent disease, control of excessive hormone production is an important part of management. Despite intensive management of recurrent ACC, treatment failure is common and the use of clinical trials and novel treatment is an important part of management. Keywords: recurrence, surgery, chemotherapy

  1. Risk factors for idiopathic optic neuritis recurrence.

    Directory of Open Access Journals (Sweden)

    Yi Du

    Full Text Available Approximately 30-50% of idiopathic optic neuritis (ION patients experience one or multiple episodes of recurrence. The aim of this study was to search for risk factors for ION recurrence.Clinical data on hospitalized patients diagnosed with ION between January 2003 and January 2011 at the First Affiliated Hospital of Guangxi Medical University were retrospectively collected. Univariate and multivariate analyses were performed on factors that might cause ION recurrence. In total, 115 ION cases (32 recurrent and 83 non-recurrent cases with complete data were analyzed. The length of the follow-up period ranged from 12 to 108 months (median: 42 months.The univariate analysis showed that the recurrence rate for unilateral ION was higher than that for bilateral ION (40% vs. 12%, p=0.001. Underlying diseases had a significant impact on recurrence (p<0.001: the recurrence rates due to neuromyelitis optica (NMO, multiple sclerosis (MS, demyelinating lesions alone of the central nervous system, and unknown causes were 89%, 70%, 41%, and 8.7%, respectively. The multivariate analysis showed that the factors causing relatively high recurrence rates included NMO (odds ratio [OR], 73.5; 95% confidence interval [CI], 7.3 to 740.9, MS (OR, 33.9; 95% CI, 5.2 to 222.2, and demyelinating lesions alone (OR, 8.9; 95% CI, 2.3 to 34.4, unilateral involvement (OR, 5.7; 95% CI, 1.5 to 21.3, relatively low initial glucocorticoid dosage (equivalent to ≤ 100 mg prednisone/day (OR, 4.3; 95% CI, 1.0 to 17.9.Underlying diseases, laterality (unilateral or bilateral, and initial glucocorticoid dosage are important risk factors of ION recurrence. Clinical physicians are advised to treat ION patients with a sufficient dose of glucocorticoid in the initial treatment stage to reduce the recurrence risk.

  2. Recurrence in Major Depression: A Conceptual Analysis

    Science.gov (United States)

    Monroe, Scott M.; Harkness, Kate L.

    2011-01-01

    Theory and research on major depression have increasingly assumed a recurrent and chronic disease model. Yet not all people who become depressed suffer recurrences, suggesting that depression is also an acute, time-limited condition. However, few if any risk indicators are available to forecast which of the initially depressed will or will not…

  3. Forequarter amputation for recurrent breast cancer

    Directory of Open Access Journals (Sweden)

    Krishna N. Pundi

    2015-01-01

    Conclusion: Forequarter amputation can be judiciously used for patients with recurrent or metastatic breast cancer. Patients with recurrent disease without evidence of distant metastases may be considered for curative amputation, while others may receive palliative benefit; disappointingly our patient achieved neither of these outcomes. In the long term, these patients may still have significant psychological problems.

  4. Recurrent takotsubo cardiomyopathy in a child.

    Science.gov (United States)

    Srivastava, Nayan T; Parent, John J; Hurwitz, Roger A

    2016-02-01

    Takotsubo cardiomyopathy or transient apical ballooning syndrome very rarely presents in children. In all patients with takotsubo, it is estimated that only 3.5% will have recurrence. In this study, we describe a case of recurrent takotsubo cardiomyopathy in a child, likely triggered by status epilepticus.

  5. Recurrent respiratory papillomatosis : From diagnosis to treatment

    NARCIS (Netherlands)

    Tjon-Pian-Gi, Robin Edward Adrianus

    2016-01-01

    Recurrent respiratory papillomatosis (RRP) is a rare, benign, recurrent disease of the airway. Its warty lesions are associated with the Human papilloma virus (HPV) types 6 and 11, with a predisposition in the larynx. Because no curative treatment exists, some patients may require more than 100 surg

  6. Examestane in advanced or recurrent endometrial carcinoma

    DEFF Research Database (Denmark)

    Lindemann, Kristina; Malander, Susanne; Christensen, René dePont;

    2014-01-01

    We evaluated the efficacy and safety of the aromatase inhibitor exemestane in patients with advanced, persistent or recurrent endometrial carcinoma.......We evaluated the efficacy and safety of the aromatase inhibitor exemestane in patients with advanced, persistent or recurrent endometrial carcinoma....

  7. Recurrent facial urticaria following herpes simplex labialis

    Directory of Open Access Journals (Sweden)

    Vijay Zawar

    2012-01-01

    Full Text Available We describe recurrent acute right-sided facial urticaria associated with herpes labialis infection in a middle-aged female patient. Antiviral medications and antihistamines not only successfully cleared the herpes infection and urticaria but also prevented further recurrences.

  8. MRCP findings in recurrent pyogenic cholangitis

    Energy Technology Data Exchange (ETDEWEB)

    Jain, Manoj [Department of Radiology, AMRI Hospitals, Salt Lake, JC-16 and 17 Salt Lake City, Kolkata 700091 (India)], E-mail: jainmanoj70@hotmail.com; Agarwal, Ajay [Department of Radiology, AMRI Hospitals, Salt Lake, JC-16 and 17 Salt Lake City, Kolkata 700091 (India)], E-mail: doctorajay2002@yahoo.co.in

    2008-04-15

    Objective: In this essay, we present the spectrum of intrahepatic and extrahepatic bile duct abnormalities seen on MRCP in patients with recurrent pyogenic cholangitis. Conclusion: MRCP is a promising, noninvasive alternative to more invasive direct cholangiography for evaluating the intrahepatic and extrahepatic bile ducts in patients with recurrent pyogenic cholangitis.

  9. ACQUIRED CUTIS LAXA WITH RECURRENT URTICARIA

    Directory of Open Access Journals (Sweden)

    Ganaparthi

    2015-05-01

    Full Text Available A 30 year old male patient presented with progressive laxity and wrinkling of skin over the face for past 10 years, patient also gives history of recurrent urticaria since 12 years. Skin biopsy using Verhoff Van Gieson stain suggestive of cutis laxa. We are reporting a rare case of acquired cutis laxa with recurrent urticaria

  10. 6th International Symposium on Recurrence Plots

    CERN Document Server

    Jr, Jr; Ioana, Cornel; Marwan, Norbert

    2016-01-01

    The chapters in this book originate from the research work and contributions presented at the Sixth International Symposium on Recurrence Plots held in Grenoble, France in June 2015. Scientists from numerous disciplines gathered to exchange knowledge on recent applications and developments in recurrence plots and recurrence quantification analysis. This meeting was remarkable because of the obvious expansion of recurrence strategies (theory) and applications (practice) into ever-broadening fields of science. It discusses real-world systems from various fields, including mathematics, strange attractors, applied physics, physiology, medicine, environmental and earth sciences, as well as psychology and linguistics. Even readers not actively researching any of these particular systems will benefit from discovering how other scientists are finding practical non-linear solutions to specific problems. The book is of interest to an interdisciplinary audience of recurrence plot users and researchers interested in time...

  11. Characterization of stickiness by means of recurrence.

    Science.gov (United States)

    Zou, Yong; Thiel, Marco; Romano, M Carmen; Kurths, Jürgen

    2007-12-01

    We propose recurrence plots (RPs) to characterize the stickiness of a typical area-preserving map with coexisting chaotic and regular orbits. The difference of the recurrence properties between quasiperiodic and chaotic orbits is revisited, which helps to understand the complex patterns of the corresponding RPs. Moreover, several measures from the recurrence quantification analysis are used to quantify these patterns. Among these measures, the recurrence rate, quantifying the percentage of black points in the plot, is applied to characterize the stickiness of a typical chaotic orbit. The advantage of the recurrence based method in comparison to other standard techniques is that it is possible to distinguish between quasiperiodic and chaotic orbits that are temporarily trapped in a sticky domain, from very short trajectories.

  12. Recurrent well-differentiated thyroid carcinoma.

    Science.gov (United States)

    Magarey, Matthew J R; Freeman, Jeremy L

    2013-07-01

    The incidence of Well-differentiated Thyroid Carcinoma (WDTC) has been increasing over the past several decades. Consequently, so has the incidence of recurrence, which ranges from 15% to 30%. Factors leading to increased risk of recurrence are well described. However, the impact of local and regional recurrence is not well understood, but distant recurrence dramatically reduces 10-year survival to 50%. Recurrent WDTC has several established options for treatment; Observation, Radioactive Iodine (RAI), Surgery and External Beam Radiotherapy (EBRT). Novel treatments such as radiofrequency ablation (RFA) and percutaneous ultrasound-guided ethanol injection (PUEI) are beginning to gain popularity and have promising early results. A review of the current literature, outcome measurements and a strategy for revision surgery within the central neck compartment are discussed within this manuscript.

  13. Calcaneal chondroblastoma with pathologic fracture and recurrence.

    Science.gov (United States)

    Dutt, Laksha; Schade, Valerie L; Manoso, Mark W

    2015-01-01

    Chondroblastomas account for 80% of cases. Local recurrence rates of ≤38% have been reported, most often because of inadequate resection, and have been associated with malignant conversion and metastasis. Adjuvant therapies can help minimize the incidence of local recurrence. Long-term follow-up examinations are recommended, given the protracted interval that can exist between recurrence and the potential for malignant conversion and metastasis. We present the case of a young, healthy, active male with a calcaneal chondroblastoma and associated pathologic fracture whose initial treatment consisted of curettage, hydrogen peroxide lavage, and allogeneic bone grafting. Recurrence developed at 15 months postoperatively and was treated with repeat curettage, high-speed burring, and reconstruction with steel Steinman pins and polymethylmethacrylate, resulting in no pain or recurrence at the 5-month follow-up point.

  14. Recurrence of autoimmune liver diseases after livertransplantation

    Institute of Scientific and Technical Information of China (English)

    2015-01-01

    Liver transplantation (LT) is the most effective treatmentmodality for end stage liver disease caused by manyetiologies including autoimmune processes. That said,the need for transplantation for autoimmune hepatitis(AIH) and primary biliary cirrhosis (PBC), but not forprimary sclerosing cholangitis (PSC), has decreasedover the years due to the availability of effective medicaltreatment. Autoimmune liver diseases have superiortransplant outcomes than those of other etiologies. WhileAIH and PBC can recur after LT, recurrence is of limitedclinical significance in most, but not all cases. RecurrentPSC, however, often progresses over years to a stagerequiring re-transplantation. The exact incidence andthe predisposing factors of disease recurrence remaindebated. Better understanding of the pathogenesis andthe risk factors of recurrent autoimmune liver diseasesis required to develop preventive measures. In thisreview, we discuss the current knowledge of incidence,diagnosis, risk factors, clinical course, and treatmentof recurrent autoimmune liver disease (AIH, PBC, PSC)following LT.

  15. Interstitial Photodynamic Therapy in Treating Patients With Recurrent Head and Neck Cancer

    Science.gov (United States)

    2017-09-11

    Recurrent Laryngeal Squamous Cell Carcinoma; Recurrent Laryngeal Verrucous Carcinoma; Recurrent Lip and Oral Cavity Squamous Cell Carcinoma; Recurrent Metastatic Squamous Cell Carcinoma in the Neck With Occult Primary; Recurrent Oral Cavity Verrucous Carcinoma; Recurrent Oropharyngeal Squamous Cell Carcinoma; Tongue Carcinoma

  16. Alisertib in Treating Young Patients With Recurrent or Refractory Solid Tumors or Leukemia

    Science.gov (United States)

    2016-07-20

    Hepatoblastoma; Previously Treated Childhood Rhabdomyosarcoma; Recurrent Childhood Acute Lymphoblastic Leukemia; Recurrent Childhood Acute Myeloid Leukemia; Recurrent Childhood Kidney Neoplasm; Recurrent Childhood Malignant Germ Cell Tumor; Recurrent Childhood Rhabdomyosarcoma; Recurrent Childhood Soft Tissue Sarcoma; Recurrent Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Recurrent Neuroblastoma; Recurrent Osteosarcoma

  17. Recurrent seizures after lidocaine ingestion.

    Science.gov (United States)

    Aminiahidashti, Hamed; Laali, Abolghasem; Nosrati, Nazanin; Jahani, Fatemeh

    2015-01-01

    Lidocaine has a concentration-dependent effect on seizures. Concentrations above 15 μg/mL frequently result in seizures in laboratory animals and human. We report a case of central nervous system (CNS) lidocaine toxicity and recurrent seizure after erroneous ingestion of lidocaine solution. A 4-year-old boy presented to the Emergency Department of Imam Hospital of Sari in December 2013 due to tonic-clonic generalized seizures approximately 30 min ago. 3 h before seizure, his mother gave him 2 spoons (amount 20-25 cc) lidocaine hydrochloride 2% solution instead of pediatric gripe by mistake. Seizure with generalized tonic-clonic occurred 3 times in home. Neurological examination was essentially unremarkable except for the depressed level of consciousness. Personal and medical history was unremarkable. There was no evidence of intracranial ischemic or hemorrhagic lesions in computed tomography scan. There were no further seizures, the condition of the patient remained stable, and he was discharged 2 days after admission. The use of viscous lidocaine may result in cardiovascular and CNS toxicity, particularly in children. Conservative management is the best option for treatment of lidocaine induced seizure.

  18. Recurrent seizures after lidocaine ingestion

    Directory of Open Access Journals (Sweden)

    Hamed Aminiahidashti

    2015-01-01

    Full Text Available Lidocaine has a concentration-dependent effect on seizures. Concentrations above 15 μg/mL frequently result in seizures in laboratory animals and human. We report a case of central nervous system (CNS lidocaine toxicity and recurrent seizure after erroneous ingestion of lidocaine solution. A 4-year-old boy presented to the Emergency Department of Imam Hospital of Sari in December 2013 due to tonic-clonic generalized seizures approximately 30 min ago. 3 h before seizure, his mother gave him 2 spoons (amount 20-25 cc lidocaine hydrochloride 2% solution instead of pediatric gripe by mistake. Seizure with generalized tonic-clonic occurred 3 times in home. Neurological examination was essentially unremarkable except for the depressed level of consciousness. Personal and medical history was unremarkable. There was no evidence of intracranial ischemic or hemorrhagic lesions in computed tomography scan. There were no further seizures, the condition of the patient remained stable, and he was discharged 2 days after admission. The use of viscous lidocaine may result in cardiovascular and CNS toxicity, particularly in children. Conservative management is the best option for treatment of lidocaine induced seizure.

  19. Determinants of recurrence after intended curative resection for colorectal cancer

    DEFF Research Database (Denmark)

    Wilhelmsen, Michael; Kring, Thomas; Jorgensen, Lars N;

    2014-01-01

    in development of recurrence. It is well established that emergency surgery is a major determinant of recurrence. Moreover, anastomotic leakages, postoperative bacterial infections, and blood transfusions increase the recurrence rates although the exact mechanisms still remain obscure. From pathology studies...

  20. Tumor vaccine against recurrence of hepatocellular carcinoma

    Institute of Scientific and Technical Information of China (English)

    Bao-Gang Peng; Li-Jiang Liang; Qiang He; Ming Kuang; Jia-Ming Lia; Ming-De Lu; Jie-Fu Huang

    2005-01-01

    AIM: To investigate the effects of autologous tumor vaccine on recurrence of hepatocellular carcinoma (HCC).METHODS: Sixty patients with HCC who had undergone curative resection, were randomly divided into HCC vaccine group and control group. Three vaccinations at 2-wk intervals were performed after curative hepatic resection. Delayedtype- hypersensitivity (DTH) test was performed before and after vaccination. Primary endpoints were the time of recurrence.RESULTS: Four patients in control group and 6 patients in HCC vaccine group were withdrawn from the study. The vaccine containing human autologous HCC fragments showed no essential adverse effect in a phase Ⅱ clinical trial and 17 of 24 patients developed a DTH response against the fragments. Three of 17 DTH-positive response patients and 5 of 7 DTH- negative response patients had recurrences after curative resection. After the operation,1-, 2- and 3-year recurrence rates of HCC vaccine groupwere 16.7%, 29.2% and 33.3%, respectively. But, 1-, 2- and3-year recurrence rates of the control group were 30.8%,53.8% and 61.5%, respectively. The time before the first recurrence in the vaccinated patients was significantly longer than that in the control patients (P<0.05).CONCLUSION: Autologous tumor vaccine is of promise in decreasing recurrence of human HCC.

  1. Tea and Recurrent Clostridium difficile Infection.

    Science.gov (United States)

    Oman Evans Ii, Martin; Starley, Brad; Galagan, Jack Carl; Yabes, Joseph Michael; Evans, Sara; Salama, Joseph John

    2016-01-01

    Background and Aims. Studies have shown effects of diet on gut microbiota. We aimed to identify foods associated with recurrent Clostridium difficile infection (CDI). Methods. In this cross-sectional survey, consecutive patients diagnosed with CDI were identified by electronic medical records. Colitis symptoms and positive Clostridium difficile assay were confirmed. Health-care onset-health-care facility associated CDI was excluded. Food surveys were mailed to 411 patients. Survey responses served as the primary outcome measure. Spearman's rank correlation identified risk factors for CDI recurrence. Results. Surveys were returned by 68 patients. Nineteen patients experienced CDI recurrence. Compared to patients without CDI recurrence, patients with CDI recurrence had more antibiotics prescribed preceding their infection (p = 0.003). Greater numbers of the latter also listed tea (p = 0.002), coffee (p = 0.013), and eggs (p = 0.013), on their 24-hour food recall. Logistic regression identified tea as the only food risk factor for CDI recurrence (adjusted OR: 5.71; 95% CI: 1.26-25.89). Conclusion. The present results indicate a possible association between tea and CDI recurrence. Additional studies are needed to characterize and confirm this association.

  2. Inguinal hernia recurrence: Classification and approach

    Directory of Open Access Journals (Sweden)

    Campanelli Giampiero

    2006-01-01

    Full Text Available The authors reviewed the records of 2,468 operations of groin hernia in 2,350 patients, including 277 recurrent hernias updated to January 2005. The data obtained - evaluating technique, results and complications - were used to propose a simple anatomo-clinical classification into three types which could be used to plan the surgical strategy:Type R1: first recurrence ′high,′ oblique external, reducible hernia with small (< 2 cm defect in non-obese patients, after pure tissue or mesh repairType R2: first recurrence ′low,′ direct, reducible hernia with small (< 2 cm defect in non-obese patients, after pure tissue or mesh repairType R3: all the other recurrences - including femoral recurrences; recurrent groin hernia with big defect (inguinal eventration; multirecurrent hernias; nonreducible, linked with a controlateral primitive or recurrent hernia; and situations compromised from aggravating factors (for example obesity or anyway not easily included in R1 or R2, after pure tissue or mesh repair.

  3. Recurrence Risk of Febrile Seizures in Children.

    Science.gov (United States)

    Agrawal, J; Poudel, P; Shah, G S; Yadav, S; Chaudhary, S; Kafle, S

    2016-09-01

    Identifying children with febrile seizure who are at risk for recurrence is important so that special attention can be given to them. The objective of this study was to identify the risk factors for recurrence of febrile seizures in children. This prospective hospital based study was conducted from July 2013 to August 2014 'among children of 6 months to 6 years of age at Bishweshwar Prasad Koirala Institute of Health Sciences (BPKIHS), Nepal. Children meeting the selection criteria were enrolled in study. Clinical, investigation, treatment and outcome parameters were analyzed. A total of 92 children with febrile seizure were enrolled in study. Males accounted for 70% and females 30%. Simple febrile seizure was present in 48% and complex febrile seizures were seen in 52%. Recurrence of seizure was seen in one third of cases. Loss of consciousness was most common post-ictal phenomenon followed by confusion and lethargy. Upper respiratory infection was the most common precipitating factor. Generalized Tonic Clonic Seizure was the most common seizure type present in 79% of cases. Significant risk factors for recurrence occurred in males (p=0.088), age less than 1 year (p=0.003). Most of the recurrence occurred within one year of first seizure. Febrile Seizure is common in males. Almost one third of children with febrile seizure are at risk for recurrence. The significant risk factors for recurrences are male gender and age <1year.

  4. Recurrent acute pancreatitis and its relative factors

    Institute of Scientific and Technical Information of China (English)

    Wei Zhang; Hong-Chao Shan; Yan Gu

    2005-01-01

    AIM: To evaluate the causes and the relative factors of recurrent acute pancreatitis.METHODS: From 1997 to 2000, acute pancreatitis relapsed in 77 of 245 acute pancreatitis patients. By reviewing the clinical treatment results and the follow-up data, we analyzed the recurrent factors of acute pancreatitis using univariate analysis and multivariate analysis.RESULTS: Of the 245 acute pancreatitis patients, 77 were patients with recurrent acute pancreatitis. Of them, 56 patients relapsed two times, 19 relapsed three times, each patient relapsed three and four times. Forty-seven patients relapsed in hospital and the other 30 patients relapsed after discharge. Eighteen patients relapsed in 1 year, eight relapsed in 1-3 years, and four relapsed after 3 years. There were 48 cases of biliary pancreatitis, 3 of alcohol pancreatitis, 5 of hyperlipidemia pancreatitis, 21 of idiopathic pancreatitis. Univariate analysis showed that the patients with local complications of pancreas, obstructive jaundice and hepatic function injury were easy to recur during the treatment period of acute pancreatitis (P = 0.022<0.05, P = 0.012<0.05 and P = 0.002<0.05, respectively). Multivariate analysis showed that there was no single factor related to recurrence. Of the 47 patients who had recurrence in hospital, 16 had recurrence in a fast period, 31 after refeeding. CONCLUSION: Acute pancreatitis is easy to recur even during treatment. The factors such as changes of pancreas structure and uncontrolled systemic inflammatory reaction are responsible for the recurrence of acute pancreatitis. Early refeeding increases the recurrence of acute pancreatitis. Defining the etiology is essential for reducing the recurrence of acute pancreatitis.

  5. Percutaneous needle fasciotomy for recurrent Dupuytren disease.

    Science.gov (United States)

    van Rijssen, Annet L; Werker, Paul M N

    2012-09-01

    Increasing options to treat Dupuytren disease include percutaneous needle fasciotomy (PNF), a minimally invasive technique that has proven to be effective for the treatment of primary disease. However, its effect on recurrent disease is not clear. We studied 30 patients with recurrent Dupuytren disease in 40 fingers, with a mean follow-up of 4.4 years. Primary outcome measures were total passive extension deficit reduction and interval to a second recurrence, defined as an increase of more than 30° compared with the result at the end of the previous treatment. We noted complications. Total passive extension reduction was 76%. Percutaneous needle fasciotomy was especially effective for the metacarpophalangeal joint, with an average reduction of 93%, whereas the average reduction in the proximal interphalangeal joint was 57%. A total of 50% of patients did not develop a secondary recurrence during follow-up. The other 50% did, and we treated recurrence within an average of 1.4 years after PNF. By means of PNF, we postponed tertiary treatment an average of 2.9 years starting from the initial treatment for Dupuytren disease. We successfully treated all secondary recurrences by limited fasciectomy, according to patients' wishes. We noted no major adverse effects. Percutaneous needle fasciotomy can be applied effectively for recurrent disease; 50% of patients remain free of recurrence for a mean of 4.4 years. If a secondary recurrence occurs, it does so relatively early after treatment. Patients must therefore be willing to accept this uncertainty in the context of the advantages of PNF, such as fast recovery, low complication rate, and minimal invasiveness. Copyright © 2012 American Society for Surgery of the Hand. Published by Elsevier Inc. All rights reserved.

  6. Mastoidite e paralisia facial como manifestações iniciais de Granulomatose de Wegener Mastoiditis and facial paralysis as initial manifestations of Wegener's Granulomatosis

    Directory of Open Access Journals (Sweden)

    André Souza de Albuquerque Maranhão

    2012-04-01

    Full Text Available A Granulomatose de Wegener (GW é caracterizada por granulomas necrotizantes e vasculite. Sem tratamento a doença tem prognóstico pobre com índice de mortalidade de 90% em 2 anos. Diversos autores citam as manifestações otológicas no curso da GW, entretanto não é especificado em que momento da doença elas apareceram, isto é, se como manifestação inicial ou subsequente a outros achados. Objetivo: Descrever três casos confirmados de GW que apresentaram inicialmente mastoidite e evoluíram com paralisia facial periférica (PFP. Material e Método: Estudo de série de casos. Pacientes diagnosticados com GW que apresentaram inicialmente manifestações otológicas são descritos. Resultados: Os três casos descritos abriram o quadro com otalgia, otorreia e hipoacusia unilateral, associada a paralisia facial periférica ipsilateral. Tiveram resposta inadequada aos tratamentos instituídos o que motivou uma investigação de outras doenças associadas. Nessas circunstâncias, detectaram-se títulos positivos de ANCA-C em todos pacientes, confirmando-se o diagnóstico de GW, após período variável de investigação. Institui-se o tratamento para GW observando-se melhora do quadro clínico, regressão da PFP e melhora parcial dos limiares auditivos. Conclusão: Complicações de otites médias agudas (mastoidite e PFP refratárias as terapêuticas habituais impõem a investigação de doenças associadas e a GW deverá ser pesquisada para que se possa fazer o diagnóstico o mais precocemente possível, alterando desta forma o prognóstico destes pacientes.Wegener's Granulomatosis (WG is characterized by necrotizing granulomas and vasculitis. If left untreated, the prognosis is poor - a 90% mortality rate within 2 years. Several authors have described the otologic manifestations of WG; these authors, however, have not mentioned the stage of the disease in which these findings present - whether as initial manifestations or subsequent to

  7. Recurrence of parasite in epigastric heteropagus

    Directory of Open Access Journals (Sweden)

    Takeshi Shirai

    2017-10-01

    Full Text Available Epigastric heteropagus (EH refers to asymmetrically conjoined twins in whom an incomplete parasite is attached to the autosite's epigastric region from the xiphisternum to the umbilicus. We herein report an extremely rare case of EH in which unexpected rapid recurrence occurred 22 months after excision of the parasite. The recurrent mass comprised the intestinal wall with peristalsis-like movement, urogenital tissues, and hepatic tissue of the parasite. The recurrence was caused by a remnant of the parasite that extended from the autosite's xiphisternum to intra-abdominal cavity.

  8. Pain recurrence after shaving of rectovaginal endometriosis

    DEFF Research Database (Denmark)

    Heide, Mathias Gottschalck; Forman, Axel

    on the bowel with risk of recurrence. This could motivate a change into more radical surgery. In the present study we therefore assessed recurrence of pain after shaving of rectovaginal endometriosis performed 2001-2009. Methods: Retrospective follow-up study. Questionnaires were sent to 212 women of whom 174...... women (82%) responded. Outcomes were correlated to the involvement of the anterior rectal wall and postoperative hormonal treatment. Results: Recurrence (pain unchanged or worse) of menstrual pain was found in 26 %, intermenstrual pain in 29 %, dyspareunia in 42 % and dyschezia in 41 %. Postoperative...

  9. RECURRENT STROKE: WHAT HAVE WE LEARNT?

    Directory of Open Access Journals (Sweden)

    EM Khoo

    2007-01-01

    Full Text Available Stroke is the third leading cause of death, a major cause of disability in adults, and is frequently more disabling than fatal. With a decline in mortality from initial cerebral infarction and an increase in the life expectancy of the population, the number of patients with recurrent stroke and ensuing cardiovascular events will become greater. Thus it is important to find out those patients at high risk of stroke recurrence. This case report illustrates the process of recurrent stroke and the resulting disabilities and morbidities in a 42-year- old man. The role of integrated stroke rehabilitation programme is described.

  10. Massage Therapy Given by Caregiver in Treating Quality of Life of Young Patients Undergoing Treatment for Cancer

    Science.gov (United States)

    2017-01-17

    Accelerated Phase Chronic Myelogenous Leukemia; Acute Undifferentiated Leukemia; Angioimmunoblastic T-cell Lymphoma; Atypical Chronic Myeloid Leukemia, BCR-ABL1 Negative; Blastic Phase Chronic Myelogenous Leukemia; Burkitt Lymphoma; Childhood Acute Lymphoblastic Leukemia in Remission; Childhood Acute Myeloid Leukemia in Remission; Childhood Chronic Myelogenous Leukemia; Childhood Diffuse Large Cell Lymphoma; Childhood Grade III Lymphomatoid Granulomatosis; Childhood Immunoblastic Large Cell Lymphoma; Childhood Myelodysplastic Syndromes; Childhood Nasal Type Extranodal NK/T-cell Lymphoma; Chronic Eosinophilic Leukemia; Chronic Myelomonocytic Leukemia; Chronic Neutrophilic Leukemia; Chronic Phase Chronic Myelogenous Leukemia; Contiguous Stage II Mantle Cell Lymphoma; Cutaneous B-cell Non-Hodgkin Lymphoma; Essential Thrombocythemia; Extramedullary Plasmacytoma; Intraocular Lymphoma; Isolated Plasmacytoma of Bone; Juvenile Myelomonocytic Leukemia; Mast Cell Leukemia; Meningeal Chronic Myelogenous Leukemia; Noncontiguous Stage II Mantle Cell Lymphoma; Polycythemia Vera; Post-transplant Lymphoproliferative Disorder; Primary Myelofibrosis; Primary Systemic Amyloidosis; Progressive Hairy Cell Leukemia, Initial Treatment; Prolymphocytic Leukemia; Recurrent Childhood Acute Lymphoblastic Leukemia; Recurrent Childhood Acute Myeloid Leukemia; Recurrent Childhood Anaplastic Large Cell Lymphoma; Recurrent Childhood Grade III Lymphomatoid Granulomatosis; Recurrent Childhood Large Cell Lymphoma; Recurrent Childhood Lymphoblastic Lymphoma; Recurrent Childhood Small Noncleaved Cell Lymphoma; Recurrent Cutaneous T-cell Non-Hodgkin Lymphoma; Recurrent Mycosis Fungoides/Sezary Syndrome; Recurrent/Refractory Childhood Hodgkin Lymphoma; Refractory Chronic Lymphocytic Leukemia; Refractory Hairy Cell Leukemia; Refractory Multiple Myeloma; Relapsing Chronic Myelogenous Leukemia; Secondary Acute Myeloid Leukemia; Stage 0 Chronic Lymphocytic Leukemia; Stage I Childhood Anaplastic Large Cell

  11. Discontinuities in recurrent neural networks.

    Science.gov (United States)

    Gavaldá, R; Siegelmann, H T

    1999-04-01

    This article studies the computational power of various discontinuous real computational models that are based on the classical analog recurrent neural network (ARNN). This ARNN consists of finite number of neurons; each neuron computes a polynomial net function and a sigmoid-like continuous activation function. We introduce arithmetic networks as ARNN augmented with a few simple discontinuous (e.g., threshold or zero test) neurons. We argue that even with weights restricted to polynomial time computable reals, arithmetic networks are able to compute arbitrarily complex recursive functions. We identify many types of neural networks that are at least as powerful as arithmetic nets, some of which are not in fact discontinuous, but they boost other arithmetic operations in the net function (e.g., neurons that can use divisions and polynomial net functions inside sigmoid-like continuous activation functions). These arithmetic networks are equivalent to the Blum-Shub-Smale model, when the latter is restricted to a bounded number of registers. With respect to implementation on digital computers, we show that arithmetic networks with rational weights can be simulated with exponential precision, but even with polynomial-time computable real weights, arithmetic networks are not subject to any fixed precision bounds. This is in contrast with the ARNN that are known to demand precision that is linear in the computation time. When nontrivial periodic functions (e.g., fractional part, sine, tangent) are added to arithmetic networks, the resulting networks are computationally equivalent to a massively parallel machine. Thus, these highly discontinuous networks can solve the presumably intractable class of PSPACE-complete problems in polynomial time.

  12. Pulmonary recurrence in patients with endometrial cancer

    Directory of Open Access Journals (Sweden)

    Taner Turan

    2016-04-01

    Conclusion: Advanced stage is associated with PR. If recurrence is only in the lung, survival is better. Systemic treatment after PR is associated with improved survival. However, multi-center studies are required to standardize the treatment for PR.

  13. Evaluation for postoperative recurrence of Crohn disease.

    Science.gov (United States)

    Swoger, Jason M; Regueiro, Miguel

    2012-06-01

    Disease recurrence following resective surgery for Crohn disease remains a challenging clinical problem, and more studies are needed to better define risk stratification and treatment recommendations in the postoperative setting. Endoscopy remains the gold standard for the assessment of postoperative disease recurrence, and all Crohn disease patients who undergo surgery should undergo ileocolonoscopy within 6 to 12 months of surgery. The degree of endoscopic recurrence in the neoterminal ileum during this procedure provides prognostic information regarding the severity of the future disease course. WCE, MRE, and SICUS are all promising noninvasive modalities to assess for postoperative Crohn disease activity. However, further studies are needed to better define scoring systems, operating characteristics and variability, and prognostic data for each of these modalities. In patients at risk for early disease recurrence, more aggressive prophylactic therapy should be considered, in hopes of delivering true “top-down” therapy that may offer maximum impact in altering the natural history of Crohn disease.

  14. Ocean wave forecasting using recurrent neural networks

    Digital Repository Service at National Institute of Oceanography (India)

    Mandal, S.; Prabaharan, N.

    , merchant vessel routing, nearshore construction, etc. more efficiently and safely. This paper describes an artificial neural network, namely recurrent neural network with rprop update algorithm and is applied for wave forecasting. Measured ocean waves off...

  15. Recurrence Within 24 Hours of Unprovoked Seizures

    Directory of Open Access Journals (Sweden)

    J Gordon Millichap

    2006-09-01

    Full Text Available The incidence and risk factors of acute recurrence of unprovoked seizures within 24 hours of admission to an Emergency Department (ED were analyzed by a retrospective chart review at Schneider Children’s Hospital, New York.

  16. Recurrence risk for germinal mosaics revisited

    NARCIS (Netherlands)

    van der Meulen, M A; te Meerman, G J

    1995-01-01

    A formula to calculate recurrence risk for germline mosaicism published by Hartl in 1971 has been updated to include marker information. For practical genetic counselling new, more elaborate tables are given.

  17. Is acute recurrent pancreatitis a chronic disease?

    Institute of Scientific and Technical Information of China (English)

    Alberto Mariani; Pier Alberto Testoni

    2008-01-01

    Whether acute recurrent pancreaUtis is a chronic disease is still debated and a consensus is not still reached as demonstrated by differences in the classification of acute recurrent pancreatitis.There is major evidence for considering alcoholic pancreatitis as a chronic disease ab initio while chronic pancreatitis lesions detectable in biliary acute recurrent pancreatitis (ARP) seem a casual association.Cystic fibrosis transmembrane con ductance regulator (CFTR) gene mutation,hereditary and obstructive pancreatitis seem an acute disease that progress to chronic pancreatitis,likely as a consequence of the activation and proliferation of pancreatic stellate cells that produce and activate collagen and therefore fibrosis.From the diagnostic point of view,in patients with acute recurrent pancreatitis Endoscopic ultrasound (EUS) seems the more reliable technique for an accurate evaluation and follow-up of some ductal and parenchymal abnormalities suspected for early chronic pancreatitis.

  18. Remote Recurrence of Benign Multicystic Peritoneal Mesothelioma.

    Science.gov (United States)

    Lee, Caroline E; Agrawal, Anita

    2017-07-18

    Benign peritoneal cystic mesothelioma (BPCM) is a rare disease entity that arises from mesothelioma cells. We describe a rare case of BPCM recurrence 36 years after its initial presentation. A 62-year-old woman was referred to an outpatient gynaecologic oncology clinic with an incidental finding of multiple pelvic cysts. She had a preceding history of known BPCM treated with extensive debulking surgery. She presented after 36 years of clinical remission. A repeat laparotomy for a debulking surgical procedure confirmed a recurrence of BPCM. Our current case represents a woman with a remote recurrence of BPCM after initial optimal debulking surgery. Her clinical presentation of recurrence after 36 years illustrates the need for long-term follow-up and clinical suspicion in symptomatic patients with previously diagnosed BPCM. Crown Copyright © 2017. Published by Elsevier Inc. All rights reserved.

  19. Recurrent acute pancreatitis: From the editor

    Institute of Scientific and Technical Information of China (English)

    Pier Alberto Testoni

    2008-01-01

    @@ This special issue of World Journal of Gastroenterology contains a number of articles focusing on acute recurrent pancreatitis,a clinical entity that still remains a complex diagnostic and therapeutic challenge in clinical practice.

  20. Multiple nodal locoregional recurrence of pheochromocytoma

    Directory of Open Access Journals (Sweden)

    César Pablo Ramírez-Plaza

    2015-01-01

    Conclusion: Isolated lymph nodal recurrence is very rare in malignant PCC, with only 7 cases previously published. The role of surgery is essential to get long-term survival because provides clinical and functional control of the disease.

  1. Association of recurrent pregnancy loss with chromosomal ...

    African Journals Online (AJOL)

    EB

    Results: Parental chromosomal abnormality was detected in 28 cases (2.8% of all cases, 5.7% of the couples) most of which ... Key words: chromosomal abnormality, recurrent pregnancy loss, thrombophilia ..... significant role in infertility.

  2. Recurrent Omental Hemangiopericytoma: A Therapeutic Challenge

    Directory of Open Access Journals (Sweden)

    Sara Jaber

    2016-01-01

    Full Text Available Hemangiopericytomas are vascular tumors with a susceptibility to arise anywhere in the human body. We present a case of a 68-year-old female with primary omental hemangiopericytoma and a two-time recurrence managed with surgery and close follow-up. The first recurrence was at 52 months and the second at 37 months following the prior presentation. No adjuvant chemotherapy or radiation therapy was administered. Given the widespread nature of the cell of origin, routine follow-up postoperatively with interval imaging in order to detect recurrences is imperative. Pathologic tumor characteristics may determine potential for recurrence and may also assist in determining whether adjuvant treatment modalities should be included in the management plan. Review of the English literature reveals a total of 24 cases of omental hemangiopericytomas inclusive of the current report.

  3. Genetics Home Reference: benign recurrent intrahepatic cholestasis

    Science.gov (United States)

    ... All Close All Description Benign recurrent intrahepatic cholestasis (BRIC) is characterized by episodes of liver dysfunction called ... a lack of appetite. A common feature of BRIC is the reduced absorption of fat in the ...

  4. Recurrence quantification analysis theory and best practices

    CERN Document Server

    Jr, Jr; Marwan, Norbert

    2015-01-01

    The analysis of recurrences in dynamical systems by using recurrence plots and their quantification is still an emerging field.  Over the past decades recurrence plots have proven to be valuable data visualization and analysis tools in the theoretical study of complex, time-varying dynamical systems as well as in various applications in biology, neuroscience, kinesiology, psychology, physiology, engineering, physics, geosciences, linguistics, finance, economics, and other disciplines.   This multi-authored book intends to comprehensively introduce and showcase recent advances as well as established best practices concerning both theoretical and practical aspects of recurrence plot based analysis.  Edited and authored by leading researcher in the field, the various chapters address an interdisciplinary readership, ranging from theoretical physicists to application-oriented scientists in all data-providing disciplines.

  5. Fluoxetin-induced pulmonary granulomatosis.

    Science.gov (United States)

    de Kerviler, E; Trédaniel, J; Revlon, G; Groussard, O; Zalcman, G; Ortoli, J M; Espié, M; Hirsch, A; Frija, J

    1996-03-01

    A patient treated with fluoxetin for a manic depressive disorder developed pulmonary inflammatory nodules with noncaseating giant cell granulomas, interstitial pneumonia and non-necrotizing vasculitis, whilst remaining asymptomatic. A progressive resolution of pulmonary nodules occurred after withdrawal of the offending agent, and the chest radiograph returned to normal in 9 months. The diagnosis was assessed by an open lung biopsy.

  6. Solving recurrence relations supported by Wolfram Mathematica

    Directory of Open Access Journals (Sweden)

    Enrique Vílchez-Quesada

    2015-01-01

    Full Text Available This paper introduces some algorithms for solving linear relationships, homogeneous and non-homogeneous recurrence with constant and non-constant coefficients, using software as the main resource in solving processes. The Mathematica commercial application has provided the technical support necessary for the implementation of the methods used. It also presents other examples of recurrence relations, showing the effectiveness and limitations of the algorithms created by the author and programmed in Mathematica environment that provides.

  7. Recurrent congenital heart block in neonatal lupus.

    Science.gov (United States)

    Escobar, Maria C; Gómez-Puerta, José A; Albert, Dimpna; Ferrer, Queralt; Girona, Josep

    2007-07-01

    Congenital heart block (CHB) is the main complication of neonatal lupus (NL) and is strongly associated with the presence of anti-SSA/Ro and anti-SSB/La antibodies. The recurrence of CHB in subsequent pregnancies in mothers with these antibodies is uncommon, occurring in approximately 15% of cases. We describe here a case of recurrent CHB in a previously asymptomatic mother with Sjögren syndrome and discuss the current strategies for the prevention and treatment of CHB in NL.

  8. Management of Recurrent Rhinosinusitis in Children

    OpenAIRE

    2012-01-01

    Rhinosinusitis is the most common complication of viral upper respiratory tract infections and the fifth most common indication for the prescription for antimicrobial agents in childhood period. In clinical evaluation a persistant nasal discharge or cough (or both) lasting more than ten days are generally seen. Acute rhinosinusitis lasts less than four weeks and chronic rhinosinusitis persists more than 12 weeks. The common causes of recurrent acute and chronic rhinosinusitis are recurrent vi...

  9. Current surgery of retinal detachment recurrence. Review

    Directory of Open Access Journals (Sweden)

    V. D. Zakharov

    2012-01-01

    Full Text Available this review presents a detailed analysis and an experience of surgical treatment of retinal detachment recurrence associated with light silicone oil tamponade of vitreous cavity. Approaches and variants of treatment were described in the historical aspect and till now. there are considered general and particular issues in case of retinal detachment recurrence appearance, expediency and volume of intraoperative manipulations, time of operation and choice of temporary substitute of vitreous body for a purpose of postoperative tamponade of vitreous cavity.

  10. Current surgery of retinal detachment recurrence. Review

    Directory of Open Access Journals (Sweden)

    V. D. Zakharov

    2014-07-01

    Full Text Available this review presents a detailed analysis and an experience of surgical treatment of retinal detachment recurrence associated with light silicone oil tamponade of vitreous cavity. Approaches and variants of treatment were described in the historical aspect and till now. there are considered general and particular issues in case of retinal detachment recurrence appearance, expediency and volume of intraoperative manipulations, time of operation and choice of temporary substitute of vitreous body for a purpose of postoperative tamponade of vitreous cavity.

  11. Forequarter amputation for recurrent breast cancer

    OpenAIRE

    Pundi, Krishna N.; AlJamal, Yazan N.; Ruparel, Raaj K.; Farley, David R.

    2015-01-01

    Introduction: Localized excision combined with radiation and chemotherapy represents the current standard of care for recurrent breast cancer. However, in certain conditions a forequarter amputation may be employed for these patients. Presentation of case: We present a patient with recurrent breast cancer who had a complicated treatment history including multiple courses of chemotherapy, radiation, and local surgical excision. With diminishing treatment options, she opted for a forequarter...

  12. Family history and recurrence of febrile seizures.

    OpenAIRE

    1994-01-01

    To determine the value of a detailed family history for the assessment of the risk of recurrence of febrile seizures, 115 children who visited the emergency room of an academic children's hospital were studied prospectively. The recurrence risk of febrile seizures was analysed in relation to the child's family history and the proportion of relatives affected by febrile seizures using Kaplan-Meier estimates and Cox proportional hazard models. A first degree family history positive for febrile ...

  13. Recurrence risk of ictal asystole in epilepsy.

    Science.gov (United States)

    Hampel, Kevin G; Thijs, Roland D; Elger, Christian E; Surges, Rainer

    2017-08-22

    To determine the recurrence risk of ictal asystole (IA) and its determining factors in people with epilepsy. We performed a systematic review of published cases with IA in 3 databases and additionally searched our local database for patients with multiple seizures simultaneously recorded with ECG and EEG and at least one IA. IA recurrence risk was estimated by including all seizures without knowledge of the chronological order. Various clinical features were assessed by an individual patient data meta-analysis. A random mixed effect logistic regression model was applied to estimate the average recurrence risk of IA. Plausibility of the calculated IA recurrence risk was checked by analyzing the local dataset with available information in chronological order. Eighty patients with 182 IA in 537 seizures were included. Recurrence risk of IA amounted to 40% (95% confidence interval [CI] 32%-50%). None of the clinical factors (age, sex, type and duration of epilepsy, hemispheric lateralization, duration of IA per patient) appeared to have a significant effect on the short-term recurrence risk of IA. When considering the local dataset only, IA recurrence risk was estimated to 30% (95% CI 14%-53%). Information whether IA coincided with symptoms (i.e., syncope) or not was given in 60 patients: 100 out of 142 IAs were symptomatic. Our data suggest that in case of clinically suspected IA, the recording of 1 or 2 seizures is not sufficient to rule out IA. Furthermore, the high short-term recurrence risk favors aggressive treatment, including pacemaker implantation if seizure freedom cannot be achieved. Copyright © 2017 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology.

  14. Laboratory constraints on models of earthquake recurrence

    Science.gov (United States)

    Beeler, Nicholas M.; Tullis, Terry; Junger, Jenni; Kilgore, Brian D.; Goldsby, David L.

    2014-01-01

    In this study, rock friction ‘stick-slip’ experiments are used to develop constraints on models of earthquake recurrence. Constant-rate loading of bare rock surfaces in high quality experiments produces stick-slip recurrence that is periodic at least to second order. When the loading rate is varied, recurrence is approximately inversely proportional to loading rate. These laboratory events initiate due to a slip rate-dependent process that also determines the size of the stress drop [Dieterich, 1979; Ruina, 1983] and as a consequence, stress drop varies weakly but systematically with loading rate [e.g., Gu and Wong, 1991; Karner and Marone, 2000; McLaskey et al., 2012]. This is especially evident in experiments where the loading rate is changed by orders of magnitude, as is thought to be the loading condition of naturally occurring, small repeating earthquakes driven by afterslip, or low-frequency earthquakes loaded by episodic slip. As follows from the previous studies referred to above, experimentally observed stress drops are well described by a logarithmic dependence on recurrence interval that can be cast as a non-linear slip-predictable model. The fault’s rate dependence of strength is the key physical parameter. Additionally, even at constant loading rate the most reproducible laboratory recurrence is not exactly periodic, unlike existing friction recurrence models. We present example laboratory catalogs that document the variance and show that in large catalogs, even at constant loading rate, stress drop and recurrence co-vary systematically. The origin of this covariance is largely consistent with variability of the dependence of fault strength on slip rate. Laboratory catalogs show aspects of both slip and time predictability and successive stress drops are strongly correlated indicating a ‘memory’ of prior slip history that extends over at least one recurrence cycle.

  15. Recurrence relation for relativistic atomic matrix elements

    CERN Document Server

    Martínez y Romero, R P; Salas-Brito, A L

    2000-01-01

    Recurrence formulae for arbitrary hydrogenic radial matrix elements are obtained in the Dirac form of relativistic quantum mechanics. Our approach is inspired on the relativistic extension of the second hypervirial method that has been succesfully employed to deduce an analogous relationship in non relativistic quantum mechanics. We obtain first the relativistic extension of the second hypervirial and then the relativistic recurrence relation. Furthermore, we use such relation to deduce relativistic versions of the Pasternack-Sternheimer rule and of the virial theorem.

  16. Dupuytren contracture recurrence project: reaching consensus on a definition of recurrence.

    Science.gov (United States)

    Felici, N; Marcoccio, I; Giunta, R; Haerle, M; Leclercq, C; Pajardi, G; Wilbrand, S; Georgescu, A V; Pess, G

    2014-12-01

    The aim of this study was to determine a definition of recurrence of Dupuytren disease that could be utilized for the comparison of the results independently from the treatment used. 24 hand surgeons from 17 countries met in an international consensus conference. The participants used the Delphi method to evaluate a series of statements: (1) the need for defining recurrence, (2) the concept of recurrence applied to the Tubiana staging system, (3) the concept of recurrence applied to each single treated joint, and (4) the concept of recurrence applied to the finger ray. For each item, the possible answer was given on a scale of 1-5: 1=maximum disagreement; 2=disagreement; 3=agreement; 4=strong agreement; 5=absolute agreement. There was consensus on disagreement if 1 and 2 comprised at least 66% of the recorded answers and consensus on agreement if 3, 4 and 5 comprised at least 66% of the recorded answers. If a threshold of 66% was not reached, the related statement was considered "not defined". A need for a definition of recurrence was established. The presence of nodules or cords without finger contracture was not considered an indication of recurrence. The Tubiana staging system was considered inappropriate for reporting recurrence. Recurrence was best determined by the measurement of a specific joint, rather than a total ray. Time 0 occurred between 6 weeks and 3 months. Recurrence was defined as a PED of more than 20° for at least one of treated joint, in the presence of a palpable cord, compared to the result obtained at time 0. This study determined the need for a standard definition of recurrence and reached consensus on that definition, which we should become the standard for the reporting of recurrence. If utilized in subsequent publications, this will allow surgeons to compare different techniques and make is easier to help patients make an informed choice.

  17. FSGS Recurrence in Adults after Renal Transplantation

    Directory of Open Access Journals (Sweden)

    Michael Rudnicki

    2016-01-01

    Full Text Available Recurrence of focal segmental glomerulosclerosis (FSGS in the allograft occurs in 30–50% of patients, and it is associated with poor renal allograft survival. Major risk factors for recurrence are younger age at diagnosis, rapid progression to end-stage renal disease, white race, and the loss of previous allografts due to recurrence. Recent data support the hypothesis that circulating permeability factors play a crucial role in podocyte injury and progression of FSGS. Due to lack of controlled trials, the management of recurrent FSGS is inconsistent and highly empirical. Prophylactic and perioperative treatment with plasmapheresis and high-dose (intravenous cyclosporine represent the main cornerstones of immunosuppressive therapy. In recent years, therapy with rituximab has shown promising results. Despite evidence of activation of the renin-angiotensin system (RAS in recurrent FSGS and its association with progression, only limited data exist on the renoprotective role of RAS blockade in this setting. Further well designed studies are needed on pathogenesis risk factors and therapeutical options in FSGS and its recurrence after transplantation.

  18. Recurrence interval analysis of trading volumes.

    Science.gov (United States)

    Ren, Fei; Zhou, Wei-Xing

    2010-06-01

    We study the statistical properties of the recurrence intervals τ between successive trading volumes exceeding a certain threshold q. The recurrence interval analysis is carried out for the 20 liquid Chinese stocks covering a period from January 2000 to May 2009, and two Chinese indices from January 2003 to April 2009. Similar to the recurrence interval distribution of the price returns, the tail of the recurrence interval distribution of the trading volumes follows a power-law scaling, and the results are verified by the goodness-of-fit tests using the Kolmogorov-Smirnov (KS) statistic, the weighted KS statistic and the Cramér-von Mises criterion. The measurements of the conditional probability distribution and the detrended fluctuation function show that both short-term and long-term memory effects exist in the recurrence intervals between trading volumes. We further study the relationship between trading volumes and price returns based on the recurrence interval analysis method. It is found that large trading volumes are more likely to occur following large price returns, and the comovement between trading volumes and price returns is more pronounced for large trading volumes.

  19. Management of recurrent postoperative fungal endophthalmitis

    Directory of Open Access Journals (Sweden)

    Anand Vinekar

    2014-01-01

    Full Text Available Aim: To report the management of recurrent postoperative fungal endophthalmitis (POFE after failed pars plana vitrectomy (PPV and antifungal therapy. Settings and Design: Tertiary Care Referral Centre in North India. Retrospective, single institution, interventional case-series. Materials and Methods: Six patients with microbiologically proven recurrent post-operative fungal endophthalmitis refractory to conventional management were included. The final recurrence was managed with intraocular lens (IOL explantation and re-PPV. Main outcome measures included preserved globe anatomy, visual acuity and retinal status. ′Anatomical success′ was defined as preserved anatomy of the globe, and absence of signs of inflammation. ′Functional success′ was defined as an attached retina and a best corrected visual acuity of better than 20/400. Results: Of the six cases of POFE, five were culture positive [Aspergillus flavus (1, Aspergillus fumigatus (2, Candida albicans (1 and Candida glabrata (1] and one was smear positive for yeast. All recurred (mean recurrences, 4 despite a mean of 2.17 PPVs and intravitreal amphotericin B. No recurrences were observed after IOL explantation with re - PPV (median follow-up, 37 months. Pre-study defined criteria for successful ′anatomical′ and ′functional′ outcomes were achieved in 83.3% and 50% respectively. Conclusion: This report highlights the effective role of combined IOL explantation with PPV in managing recurrent POFE.

  20. An evaluation of factors predicting breast recurrence and prognosis after recurrence, on distinguishing intramammary and extramammary recurrence, in breast-conserving surgery

    Energy Technology Data Exchange (ETDEWEB)

    Nishimura, Reiki; Nagao, Kazuharu; Miyayama, Haruhiko [Kumamoto City Hospital (Japan)] (and others)

    2001-06-01

    Recurrence of cancer in the breast is an important problem in breast-conserving therapy. We evaluated risk factors for recurrence from the viewpoint of recurrence type and outcome after recurrence. Of 533 cases of breast cancer treated with breast-conserving surgery from April 1989 through July 2000, disease in 66 recurred (12.4%) and were classified as 23 cases of breast recurrence only, 16 cases of both breast recurrence and distant metastasis, and 27 cases of distant metastasis only. The clinical factors examined included age, lymphatic invasion, nodal status, extensive intraductal component (EIC), proliferative activity, and estrogen receptor (ER) status. Of the 39 cases of breast recurrence, 19 had intramammary tumors and 20 had extramammary tumors of the skin, subcutaneous tissue, or muscle, including 8 cases with inflammatory breast recurrence. Multivariate analysis showed that factors correlated with breast recurrence were age, ER status, proliferative activity, and surgical margin. EIC-comedo was related to intramammary recurrence, whereas lymphatic invasion and nodal status were related to extramammary recurrence. Postoperative irradiation was an effective treatment for tumors in young women and tumors with positive margins or a comedo component. Outcome after breast recurrence depended on nodal status at primary operation, and survival rates were worst in patients with inflammatory breast recurrence. In conclusion, age, EIC-comedo status, the surgical margin, and negative ER status were correlated with breast recurrence. Countermeasures against these factors should be investigated. (author)

  1. FEBRILE SEIZURE: RECURRENCE AND RISK FACTORS

    Directory of Open Access Journals (Sweden)

    A. TALEBIAN

    2006-06-01

    Full Text Available Background:Febrile Convulsion is the most common convulsive disorder in children,occurring in 2 to 4% of the pediatric population and recurring in 30-50% of cases. Considering the varying recurrence rates reported, thisstudy was conducted at the pediatric ward of the Shaheed BeheshtiGeneral Hospital, between 2000-2001 to determine the frequencyof recurrence and related risk factors in children presenting with theirfirst episode of febrile convulsionMaterials & Methods:A two–year cohort study was performed on 50 children presentingwith the first attack of febrile convulsion. Patient demographic dataincluding age, sex, type and duration of seizure, family history offebrile seizure or epilepsy and the interval between fever onset andoccurrence of seizure were recorded in questionnaires. Those patients,for whom prophylactic medication was not administered, werefollowed at three–month intervals for up to one year. Findings werestatistically analyzed using Fisher’s exact testResults:Recurrence was observed in twelve children (24% out of the fifty,being most common in patients aged less than one year (54.4%.Recurrence rates among children with a positive family history offebrile convulsion, presence of complex febrile seizure and positivefamily history of epilepsy were 42.1%, 42.8% and 25% respectively.From among those children with a “less than one hour” intervalbetween fever onset and occurrence of seizure, recurrence occurredin 43-7% of cases, while in those with a “more than one hourinterval”, 14.7% experienced recurrence.Conclusion:Recurrence rates are increased by certain factors including age-belowone year-, positive family history of febrile convulsion, and a “lessthan one hour” interval between time of fever onset and seizureoccurrence.

  2. Finding recurrence networks' threshold adaptively for a specific time series

    Science.gov (United States)

    Eroglu, D.; Marwan, N.; Prasad, S.; Kurths, J.

    2014-11-01

    Recurrence-plot-based recurrence networks are an approach used to analyze time series using a complex networks theory. In both approaches - recurrence plots and recurrence networks -, a threshold to identify recurrent states is required. The selection of the threshold is important in order to avoid bias of the recurrence network results. In this paper, we propose a novel method to choose a recurrence threshold adaptively. We show a comparison between the constant threshold and adaptive threshold cases to study period-chaos and even period-period transitions in the dynamics of a prototypical model system. This novel method is then used to identify climate transitions from a lake sediment record.

  3. Primary Recurrence in the Lung is Related to Favorable Prognosis in Patients with Pancreatic Cancer and Postoperative Recurrence.

    Science.gov (United States)

    Zheng, Biao; Ohuchida, Kenoki; Yan, Zilong; Okumura, Takashi; Ohtsuka, Takao; Nakamura, Masafumi

    2017-06-20

    The pattern of recurrence affects the clinical outcome in tumor patients. However, the clinical significance of lung metastasis as the primary recurrence site after resection in patients with pancreatic cancer remains unclear. This study aimed to clarify the clinical significance of the primary recurrence site in patients with pancreatic cancer, in terms of prognosis and clinicopathological features. This retrospective cohort study included 220 patients with postoperative recurrence after pancreatectomy for pancreatic cancer and classified by primary site of recurrence. We focused on patients with lung metastasis as the primary recurrence and investigated its correlation with prognosis and clinicopathological factors. Twenty-four (11%) patients had lung metastasis as the primary recurrence. This recurrence pattern had the best prognosis among all recurrence patterns, including liver metastasis and local recurrence. Patients with lung metastasis as the primary recurrence had favorable overall survival and survival from the date of recurrence compared with patients with other primary recurrence sites in both univariate (P = 0.0008 and P = 0.0005) and multivariate analyses (P = 0.0051 and P = 0.0068). In terms of clinicopathological features of resected pancreatic tumors, lung metastasis as the primary recurrence was associated with lower tumor stage and histologic grade, and less vascular invasion and residual tumor volume than liver metastasis. Pancreatic cancer patients with lung metastasis as the primary recurrence after pancreatectomy have a better prognosis than those with other recurrence patterns.

  4. Recurrent Falls in Parkinson’s Disease: A Systematic Review

    OpenAIRE

    Allen, Natalie E.; Allison K. Schwarzel; Canning,Colleen G.

    2013-01-01

    Most people with Parkinson’s disease (PD) fall and many experience recurrent falls. The aim of this review was to examine the scope of recurrent falls and to identify factors associated with recurrent fallers. A database search for journal articles which reported prospectively collected information concerning recurrent falls in people with PD identified 22 studies. In these studies, 60.5% (range 35 to 90%) of participants reported at least one fall, with 39% (range 18 to 65%) reporting recurr...

  5. Volumetric Growth Rate of Recurrent Pleomorphic Adenoma.

    Science.gov (United States)

    Naunheim, Molly; Wu, Xin; Ryan, William R; Wang, Steven J; Heaton, Chase M

    2017-07-01

    Surgery for recurrent pleomorphic adenoma (PA) can be challenging and may increase the risk of operative complications, particularly facial nerve weakness. As observation may be a viable alternative to surgery for slow-growing tumors, our objective was to assess the growth rate of recurrent PAs. This study is a case series of patients at our tertiary academic medical center with recurrent PA. Two magnetic resonance images (MRI) were compared; total volume (TV) of recurrent tumor on both studies was calculated to obtain our main outcomes of percent change in TV and tumor growth rate. Fourteen patients with recurrent PA had a median interval time between MRI of 12.8 months. Though growth rates were variable, the median continuous compound growth per year was 10.2%. Notably, 3 patients (21%) had no growth, and 2 patients (14%) had a reduction in TV. The median growth rate for enlarging tumors is estimated at 10.2% per year. Due to variability, tumor growth rate should be estimated on an individual patient basis. For slow-growing tumors, physicians may weigh the risk of this slow growth with the morbidity of reoperation.

  6. Management of postoperative recurrence of Crohn's disease.

    Science.gov (United States)

    van Lent, Anja U; D'Haens, Geert R

    2013-01-01

    The course of Crohn's disease (CD) is unpredictable and potentially destructive. The percentage of patients requiring surgery at some stage in their disease accumulates to over 70%. After resection of the affected intestine, reappearance of CD occurs in the majority of patients. Prophylactic medical therapy to reduce the rate of postoperative recurrence has been proven to be effective, yet the incidence of recurrence remains high. Patient profiling (risk stratification) is important in this postoperative setting. High-risk patients (associated with e.g. smoking, the need of repetitive surgery and penetrating disease) require strong immunosuppressive treatment, which should be commenced immediately after surgery, when recurrent disease activity begins. Additionally, early screening endoscopy should be performed to monitor treatment effect. The efficacy of thiopurines is shown to be higher than mesalazine or imidazole antibiotics alone for preventing and ameliorating endoscopic recurrence of CD postoperatively; however, anti-tumor necrosis factors (anti-TNFs) are increasingly considered the most potent agents. In patients with a risk factor for early postoperative recurrence, the first line of treatment is 6-mercaptopurine, in combination with imidazole antibiotics if tolerated, followed by anti-TNFs. When lesions are found at colonoscopy, therapy should be upscaled. We propose a treatment algorithm to direct therapeutic management of CD postoperatively. © 2013 S. Karger AG, Basel.

  7. Management of recurrent tonsillitis in children.

    Science.gov (United States)

    El Hennawi, Diaa El Din; Geneid, Ahmed; Zaher, Salah; Ahmed, Mohamed Rifaat

    To compare azithromycin (AZT) and benzathine penicillin (BP) in the treatment of recurrent tonsillitis in children. The study comprised of 350 children with recurrent streptococcal tonsillitis, 284 of whom completed the study and 162 children received conventional surgical treatment. The rest of the children, 122, were divided randomly into two equal main groups. Group A children received a single intramuscular BP (600,000IU for children≤27kg and 1,200,000IU for ≥27kg) every two weeks for six months. Group B children received single oral AZT (250mg for children≤25kg and 500mg for ≥25kg) once weekly for six months. Both groups showed marked significant reduction in recurrent tonsillitis that is comparable to results of tonsillectomy. There were no statistical differences between group A and B regarding the recurrence of infections and drug safety after six-month follow-up. Group B showed better compliance. AZT proved to be good alternative to BP in the management of recurrent tonsillitis with results similar to those obtained after tonsillectomy. Copyright © 2017 Elsevier Inc. All rights reserved.

  8. H pylori recurrence after successful eradication

    Institute of Scientific and Technical Information of China (English)

    Yaron Niv

    2008-01-01

    Recurrence of H pylori after eradication is rare in developed countries and more frequent in developing countries.Recrudescence(recolonization of the same strain within 12 mo after eradication)rather than reinfection(colonization with a new strain,more than 12 mo after eradication)is considered to be responsiblefor most of the cases.This observation was confirmed only in developed countries,while in developing countries a recent meta-analysis demonstrated a high rate of reinfection.The proportion of H pylori annual recurrence was 2.67% and 13.00% in developed and developing countries,respectively.Nested meta-analysis(only cases with a longer follow-up and a negative 13CUBT a year after eradication)revealed annual recurrence rate of 1.45%[relative risk(RR),0.54]and 12.00%(RR,0.92)in developed and developing countries,respectively.These findings support the notion that in developed countries many cases of recurrence are due to recrudescence within the first year after eradication,with a 46% drop in the recurrence rate after the first year post eradication,while in developing countries reinfection is more pronounced,and continue at the same rate since eradication.A different approach for follow-up after H pylori eradication is probably needed in patients of developing countries,since reinfection is highly prevalent.

  9. Recurrence of bronchioloalveolar carcinoma in transplanted lungs.

    Science.gov (United States)

    Garver, R I; Zorn, G L; Wu, X; McGiffin, D C; Young, K R; Pinkard, N B

    1999-04-08

    Bronchioloalveolar carcinoma is a distinctive subtype of typical adenocarcinoma of the lung that tends to metastasize widely throughout the lungs but less commonly elsewhere. Because conventional therapies for intrapulmonary metastatic bronchioloalveolar carcinoma are generally ineffective, we treated seven patients who had intrapulmonary metastatic bronchioloalveolar carcinoma with lung transplantation. Seven patients with biopsy-proved bronchioloalveolar carcinoma and no evidence of extrapulmonary disease received transplants of either one or two cadaveric lungs. At transplantation, all native lung tissue was removed and replaced with a donor lung or lungs. The patients received the usual post-transplantation care given at the institution. Four of the seven patients had recurrent bronchioloalveolar carcinoma within the donor lungs; the recurrences appeared from 10 to 48 months after transplantation. All recurrences were limited to the donor lungs. Histologic and molecular analyses showed that the recurrent tumors in three patients originated from the recipients of the transplants. Lung transplantation for bronchioloalveolar carcinoma is technically feasible, but recurrence of the original tumor within the donor lungs up to four years after transplantation was common.

  10. Clinical analysis of patients with Wegener's granulomatosis complicated with pulmonary infection%韦格纳肉芽肿病合并肺部感染的临床分析

    Institute of Scientific and Technical Information of China (English)

    曾平; 张国华; 苏金梅; 张文; 曾小峰

    2011-01-01

    目的 分析韦格纳肉芽肿病合并肺部感染的临床特点和危险因素.方法 回顾性分析北京协和医院1998-2009年收治的韦格纳肉芽肿病合并肺部感染患者的临床资料.采用t检验和Fisher检验进行统计分析.结果 92例韦格纳肉芽肿病患者中合并肺部感染27例(29%).其中首次就诊即合并肺部感染7例占26%,确诊韦格纳肉芽肿病0~6个月内发生肺部感染12例(44%),确诊韦格纳肉芽肿病6个月以上发生肺部感染8例(30%).肺部感染表现为咳嗽、咯痰(89%),咯血(63%),发热、乏力(56%),胸闷、胸痛(33%).主要为细菌感染(59%),真菌感染(37%),结核分枝杆菌感染(37%).WG合并肺部感染组有鼻窦感染( P=0.01)、低蛋白血症(P=0.03)、低免疫球蛋白血症(P=0.007)、接受激素冲击治疗(P=0.002)的比例高于无肺部感染组.结论 韦格纳肉芽肿病合并肺部感染0~6个月内发生率高,临床上表现为咳嗽、咯痰,病原学检查细菌、真菌、结核感染多见,与鼻窦感染、低蛋白血症、低免疫球蛋白血症、接受激素冲击治疗有关.%Objective To investigate the clinical features and risk factors of patient with Wegener's granulomatosis complicated with pulmonary infection.Methods Patients with Wegener's granulomatosis admitted to our hospital in the past 11 years were retrospectively analyzed.Comparisons between groups were performed by t tests or Fisher test.Results Pulmonary infection occurred in 27 cases with an incidence rate of 29%.Twenty-six percent of pulmonary infections occurred at the initial diagnosis,and 44% occurred within 6 months,while 30% occurred later than 6 months.The clinical manifestations of pulmonary infection were productive cough (89%),hemoptysis (63%),fever and fatigue (56%),chest pain and pactoralgia (33%).The most common causative pathogen were bacteria(59% ),fungi(37% ),and tubercle bacillus(37% ).Sinus infection (P=0

  11. Group G Streptococcus bacteremia in recurrent cellulitis.

    Science.gov (United States)

    di Meo, Nicola; Stinco, Giuseppe; Gubertini, Nicoletta; Patriarca, Maria Martina; Trevisan, Giusto

    2014-01-01

    In recent years, group G Streptococcus has been reported with increasing frequency as the cause of a variety of human infections. Underlying host factors such as immunosuppression, malignancy, diabetes mellitus, and rheumatoid arthritis may be predisposing conditions leading to infection. Toxic involvement and post-streptococcal sequalae, once believed to be exclusive to infections caused by group A Streptococcus, are now known to occur following acute group G Streptococcus and group C Streptococcus infections. We report on a case of group G Streptococcus bacteremia and recurrent cellulitis with toxic involvement. Patient blood cultures were always negative for β-hemolytic Streptococci in all the recurrences, except during the last one. Antibiotic therapy based on antibiogram quickly resolved the infection. A regimen of intramuscular injection of 1.2 million units of benzathine penicillin every 15 days for one year prevented recurrences of cellulitis.

  12. Laparoscopic Surgery for Recurrent Crohn's Disease

    Directory of Open Access Journals (Sweden)

    Antonino Spinelli

    2012-01-01

    Full Text Available In spite of the recent improvements in drug therapy, surgery still represents the most frequent treatment for Crohn's disease (CD complications. Laparoscopy has been widely applied over the last twenty years in colorectal surgery and was associated with lower postoperative pain, shorter hospitalization, faster return to daily activities, and better cosmetic results. Laparoscopy experienced a slower diffusion in inflammatory bowel disease surgery than in oncologic colorectal surgery, but proved to be safe and effective, and is currently considered the gold standard for the treatment of primary uncomplicated ileocolic CD. Indications for laparoscopy in CD have recently been widened to embrace more complicated or recurrent CD. This paper reviews the available data on the subset of recurrent CD patients. The reported results indicate that laparoscopy may be safely applied even in selected recurrent CD cases in hands of IBD surgeons with broad laparoscopic experience.

  13. Recurrence Metrics and Time Varying Light Cones

    CERN Document Server

    Singh-Modgil, M

    2005-01-01

    It is shown by explicit construction of new metrics, that General Relativity can solve the exact Poinc$\\acute{a}$re recurrence problem. In these solutions, the light cone, flips periodically between past and future, due to a periodically alternating arrow of the proper time. The geodesics in these universes show periodic Loschmidt's velocity reversion $v \\to -v$, at critical points, which leads to recurrence. However, the matter tensors of some of these solutions exhibit unusual properties - such as, periodic variations in density and pressure. While this is to be expected in periodic models, the physical basis for such a variation is not clear. Present paper therefore can be regarded as an extension of Tipler's "no go theorem for recurrence in an expanding universe", to other space-time geometries.

  14. Recurrent intramedullary epidermoid cyst of conus medullaris.

    LENUS (Irish Health Repository)

    Fleming, Christina

    2011-01-01

    Spinal intramedullary epidermoid cyst is a rare condition. Recurrent epidermoid cyst in the spine cord is known to occur. The authors describe a case of recurrent conus medullaris epidermoid cyst in a 24-year-old female. She initially presented at 7 years of age with bladder disturbance in the form of diurnal enuresis and recurrent urinary tract infection. MRI lumbar spine revealed a 4 cm conus medullaris epidermoid cyst. Since the initial presentation, the cyst had recurred seven times in the same location and she underwent surgical intervention in the form of exploration and debulking. This benign condition, owing to its anatomical location, has posed a surgical and overall management challenge. This occurrence is better managed in a tertiary-care centre requiring multi-disciplinary treatment approach.

  15. Recurrence quantification analysis of global stock markets

    Science.gov (United States)

    Bastos, João A.; Caiado, Jorge

    2011-04-01

    This study investigates the presence of deterministic dependencies in international stock markets using recurrence plots and recurrence quantification analysis (RQA). The results are based on a large set of free float-adjusted market capitalization stock indices, covering a period of 15 years. The statistical tests suggest that the dynamics of stock prices in emerging markets is characterized by higher values of RQA measures when compared to their developed counterparts. The behavior of stock markets during critical financial events, such as the burst of the technology bubble, the Asian currency crisis, and the recent subprime mortgage crisis, is analyzed by performing RQA in sliding windows. It is shown that during these events stock markets exhibit a distinctive behavior that is characterized by temporary decreases in the fraction of recurrence points contained in diagonal and vertical structures.

  16. RECURRENT SUBCLITORAL ABSCESS TREATED BY MARSUPIALIZATION

    Directory of Open Access Journals (Sweden)

    HORMOZ DABIRASHRAFI

    1986-05-01

    Full Text Available This is a report of a very rare case of recurrent subclitoral abscess. Its etiology and the best treatment of the disease is here in discussed. We, the same as Sur, believe that marsupialization is the most promising treatment. Recurrent periclitoral abscess has been described previously5. s ome of the authors believe that it is part of the pilonidal disease. The first pilonidal cyst in 7 the clitoral region was introduced by Palmer (1957."nAnother case of pilonidal sinus of clitoris was repor-2 ted by Betson . All of the researchers are not in this opinion that the disease is necessarily a pilonidal sinus 1 3,and, sometimes, there is not any hair in the epithelium lining of the cyst. One case of recurrent subclitoral abscess treated by marsupialization is presented here.

  17. Management of Recurrent Aphthous Stomatitis in Children.

    Science.gov (United States)

    Montgomery-Cranny, Jodie A; Wallace, Ann; Rogers, Helen J; Hughes, Sophie C; Hegarty, Anne M; Zaitoun, Halla

    2015-01-01

    Recurrent oral ulceration is common and may present in childhood. Causes of recurrent oral ulceration are numerous and there may be an association with underlying systemic disease. Recurrent aphthous stomatitis (RAS) is the most common underlying diagnosis in children. The discomfort of oral ulcers can impact negatively on quality of life of a child, interfering with eating, speaking and may result in missed school days. The role of the general dental practitioner is to identify patients who can be treated with simple measures in primary dental care and those who require assessment and treatment in secondary care. Management may include topical agents for symptomatic relief, topical corticosteroids and, in severe recalcitrant cases, systemic agents may be necessary.

  18. Recurrent Admissions for Diabetic Foot Complications

    Directory of Open Access Journals (Sweden)

    Ang CL

    2013-07-01

    Full Text Available Diabetic foot complications are a significant source of morbidity and mortality. Patients who undergo recurrent admissions for the same diabetic foot problems represent a difficult subgroup to treat. From July 2007 to June 2008, there were 38 such patients who were admitted recurrently. Eighteen patients (47% were re-admitted because of previous refusal of surgical treatment. Eighteen patients (47% received treatment as necessary but were still readmitted for recurrent infection at the same wound site. Assessment of patients’ compliance to outpatient treatment was found to be generally lacking. As a significant proportion were re-admitted because of previous refusal of surgery, a trained counselor may be suitable in counselling patients for debridement or amputation surgery.

  19. Recurrent otorrhea in chronic suppurative otitis media

    DEFF Research Database (Denmark)

    Jensen, Ramon Gordon; Johansen, Helle Krogh; Bjarnsholt, Thomas

    2017-01-01

    of otorrhea in CSOM. In a prospective case series at a primary healthcare clinic in Nuuk, Greenland, patients with more than 14 days of otorrhea were included consecutively. Samples for culturing and biofilm analysis were taken at enrollment and at any subsequent episode with otorrhea. Participants were...... treated with daily saline irrigation and Ciprofloxacin eardrops for 7–14 days. Biofilm was identified in otorrhea in 81% (17/21) of participants at enrollment. Multispecies infections dominated with Non-typeable Haemophilus Influenzae (NTHI), Staphyloccocus aureus, and anaerobes being the most frequent...... pathogens. After the initial treatment, 19 (90%) had dry ears. Median observation period was 140 days (range 14–280) where 13 participants had one or more recurrences. Median time to first recurrence was 60 days (range 14–197). Among the 13 with recurrence, three individuals had the same genotype...

  20. Recurrent conjunctival atypical fibroxanthoma in Pigmentosum Xeroderma.

    Science.gov (United States)

    Cerdà-Ibáñez, M; Barreiro-González, A; Barranco González, H; Aviñó Martínez, J; Évole-Buselli, M; Harto-Castaño, M Á

    2017-08-23

    A 7 year-old boy with Xeroderma Pigmentosum (XP) and who presents a recurrent conjunctival atypical fibroxanthoma after two surgeries. This is the third procedure and the patient is treated with a surgical excision of the tumour and cryotherapy at the surgical bed. Due to the risk of recurrence, topical Mitomycin C 0,02% was added at post-operative care achieving a good clinical outcome. Surgical exeresis with cryotherapy and topical Mitomycin C is an effective treatment for a case of an atypical fibroxanthoma with a high potential for recurrence and invasion. An ophthalmologic follow-up is required for these patients, as well as general paediatric care and support aids. Copyright © 2017 Sociedad Española de Oftalmología. Publicado por Elsevier España, S.L.U. All rights reserved.

  1. CHROMOSOMAL ABNORMALITIES IN PATIENTS WITH RECURRENT MISCARRIAGE

    Directory of Open Access Journals (Sweden)

    Daniela Mierla

    2012-06-01

    Full Text Available Chromosomal abnormalities are involved in the etiology of recurrent spontaneous pregnancy loss and sub-fertility. The purpose of this study was to determine the frequency and contribution of chromosomal abnormalities in recurrent miscarriages. The results obtained and literature review are helpful in understanding the importance of cytogenetics analysis of female infertility. To investigate the distribution of chromosomal abnormalities in the Romanian population with recurrent miscarriage, karyotype analysis by G-banding was performed from peripheral blood in 967 women infertility. Results: Chromosomal abnormalities were found to 79 women (8,17%. The percentage of chromosomal abnormalities in the studied population correlates with the data in the literature. Chromosomal abnormalities could play the important role in etiology of infertility and are more frequently detected in this group of patients compared to general population. In the infertile couples balanced chromosomal abnormalities are the main cause of spontaneous abortions.

  2. Recurrence for discrete time unitary evolutions

    CERN Document Server

    Grünbaum, F A; Werner, A H; Werner, R F

    2012-01-01

    We consider quantum dynamical systems specified by a unitary operator U and an initial state vector \\phi. In each step the unitary is followed by a projective measurement checking whether the system has returned to the initial state. We call the system recurrent if this eventually happens with probability one. We show that recurrence is equivalent to the absence of an absolutely continuous part from the spectral measure of U with respect to \\phi. We also show that in the recurrent case the expected first return time is an integer or infinite, for which we give a topological interpretation. A key role in our theory is played by the first arrival amplitudes, which turn out to be the (complex conjugated) Taylor coefficients of the Schur function of the spectral measure. On the one hand, this provides a direct dynamical interpretation of these coefficients; on the other hand it links our definition of first return times to a large body of mathematical literature.

  3. Acute recurrent pancreatitis: An autoimmune disease?

    Institute of Scientific and Technical Information of China (English)

    Raffaele Pezzilli

    2008-01-01

    In this review article,we will briefly describe the main characteristics of autoimmune pancreatitis and then we will concentrate on our aim,namely,evaluating the clinical characteristics of patients having recurrence of pain from the disease.In fact,the open question is to evaluate the possible presence of autoimmune pancreatitis in patients with an undefined etiology of acute pancreatitis and for this reason we carried out a search in the literature in order to explore this issue.In cases of recurrent attacks of pain in patients with "idiopathic"pancreatitis,we need to keep in mind the possibility that our patients may have autoimmune pancreatitis.Even though the frequency of this disease seems to be quite low,we believe that in the future,by increasing our knowledge on the subject,we will be able to diagnose an ever-increasing number of patients having acute recurrence of pain from autoimmune pancreatitis.

  4. Mining Recurrent Pattern Identification on Large Database

    Directory of Open Access Journals (Sweden)

    Shivangi Srivastava

    2014-04-01

    Full Text Available Recurrent pattern mining is an important problem in the context of data mining. In this paper data mining algorithms have been discussed and compared. Recurrent pattern mining has been an important area in data mining research and it is the first step in the analysis of data rising in a broad range of applications. The algorithms are compared with respect to the items like methodology and its basic principles in terms of the elements user like support, and scan of the database (full or partial.

  5. Harris Recurrence and MCMC: A Simplified Approach

    DEFF Research Database (Denmark)

    Asmussen, Søren; Glynn, Peter W.

    A key result underlying the theory of MCMC is that any η-irreducible Markov chain having a transition density with respect to η and possessing a stationary distribution is automatically positive Harris recurrent. This paper provides a short self-contained proof of this fact.......A key result underlying the theory of MCMC is that any η-irreducible Markov chain having a transition density with respect to η and possessing a stationary distribution is automatically positive Harris recurrent. This paper provides a short self-contained proof of this fact....

  6. Immunologic Abnormalities, Treatments, and Recurrent Pregnancy Loss

    DEFF Research Database (Denmark)

    Wang, Nathalie F; Kolte, Astrid M; Larsen, Elisabeth C

    2016-01-01

    Recurrent pregnancy loss, depending on the definition, affects 1% to 3% of women aiming to have a child. Little is known about the direct causes of recurrent pregnancy loss, and the condition is considered to have a multifactorial and complex pathogenesis. The aim of this review was to summarize ...... the evaluation and the management of the condition with specific emphasis on immunologic biomarkers identified as risk factors as well as current immunologic treatment options. The review also highlights and discusses areas in need of further research....

  7. Animal models of recurrent or bipolar depression.

    Science.gov (United States)

    Kato, T; Kasahara, T; Kubota-Sakashita, M; Kato, T M; Nakajima, K

    2016-05-03

    Animal models of mental disorders should ideally have construct, face, and predictive validity, but current animal models do not always satisfy these validity criteria. Additionally, animal models of depression rely mainly on stress-induced behavioral changes. These stress-induced models have limited validity, because stress is not a risk factor specific to depression, and the models do not recapitulate the recurrent and spontaneous nature of depressive episodes. Although animal models exhibiting recurrent depressive episodes or bipolar depression have not yet been established, several researchers are trying to generate such animals by modeling clinical risk factors as well as by manipulating a specific neural circuit using emerging techniques.

  8. [Recurrent vulvovaginitis: diagnostic assessment and therapeutic management].

    Science.gov (United States)

    Ramírez-Santos, A; Pereiro, M; Toribio, J

    2008-04-01

    Recurrent vulvovaginitis is a common problem in clinical practice. Management is often complicated by a long history of inappropriate treatments based on tentative diagnoses after an incomplete diagnostic workup. We review the most common causes of recurrent vulvovaginitis; the appropriate steps with which to establish a diagnosis, from the medical history through to the additional tests needed; and, finally, the best therapeutic options. We will focus on infectious, irritant, allergic, and hormonal causes as the ones of most interest to the dermatologist. Given that infection is the most frequent cause of these processes and also a common reason for inopportune treatment, we will pay particular attention to infectious etiologies and their differential diagnosis.

  9. Managing recurrent genital herpes with acyclovir

    Directory of Open Access Journals (Sweden)

    Bedi T

    1995-01-01

    Full Text Available Seventy five patients of recurrent genital herpes (RGH treated with oral or topical acyclovir and placebo were compared and followed for periods ranging 4 to 8 years in a prospective study. Oral acyclovir definitely helps RGH patients; it shortens healing time; postpones recurrences and instills confidence in the patients. There is sufficient evidence that RGH dies a natural death with time as seen after 8 years follow up in placebo group patients. Topical use of acyclovir cream is not as useful as believed.

  10. The emotive causes of recurrent international conflicts.

    Science.gov (United States)

    Long, William J; Brecke, Peter

    2003-03-01

    Many international conflicts are recurrent, and many of these are characterized by periods of violence, including wars, that are hard to describe as planned products of rational decision-making. Analysis of these conflicts according to rational-choice international-relations theory or constructivist approaches has been less revealing than might have been hoped. We consider the possibility that emotive causes could better explain, or at least improve the explanation of, observed patterns. We offer three emotive models of recurrent conflict and we outline a method by which the reliability of emotive explanations derived from these models could be tested prospectively.

  11. Cancer immunology and colorectal cancer recurrence.

    Science.gov (United States)

    Vannucci, Luca

    2011-06-01

    The recurrence of a cancer - local or distant (metastasis) - is manifested by the persistence of cancer cells in the organism after the ablation of the primary lesion, an ineffective anticancer immune response, and by the activity of biological/immunological factors that can stimulate and sustain its development. This review focuses on colorectal carcinoma and discusses some aspects of cancer immunology regarding cancer development and its recurrence. It is addressed also to the clinician to provide new insights helpful for designing better therapeutic strategies and patient's follow up. Therapeutic approaches used during and after surgical treatments, found capable of modulating immunity (differently affecting disease outcome), will also be described.

  12. Approach to the child with recurrent infections

    Directory of Open Access Journals (Sweden)

    Suzan A AlKhater

    2009-01-01

    Full Text Available Children with a history of recurrent, severe, or unusual infections present a diagnostic challenge. It is important to maintain a high index of suspicion for the diagnosis of immunodeficiency, for early diagnosis and treatment can improve outcome. Differentiation between infections caused by common risk factors, or immune dysfunction should be based on a detailed history and physical examination and, if indicated, followed by appropriate laboratory studies. This paper aims at providing guidelines for the evaluation of children with recurrent infections. It provides an overview of the diagnostic approach including important details required from the history, physical examination, and an appropriate choice of screening test to be ordered.

  13. Recurrent breast sparganosis: Clinical and radiological findings

    Energy Technology Data Exchange (ETDEWEB)

    Park, Ji Yoon; Woo, Ok Hee [Dept. of Radiology, Korea University Guro Hospital, Seoul (Korea, Republic of); Cho, Kyu Ran; Seo, Bo Kyoung [Korea University Anam Hospital, Seoul (Korea, Republic of)

    2015-09-15

    We report a case of recurrent sparganosis of the breast within 6 months following surgical removal of worms from the breast. The patient was referred to our hospital with a palpable mass in the right breast. On admission, breast ultrasonography revealed a tortuous tubular hypoechoic lesion with indistinct margins within a surrounding hyperechoic area, which strongly suggested sparganosis. We performed surgical excision and confirmed sparganosis. After 6 months, the patient detected a new mass in her right breast and visited our hospital. Breast ultrasonography revealed similar features in a different area of the same breast. We confirmed recurrent sparganosis surgically.

  14. Recurrent disease following liver transplantation for nonalcoholic steatohepatitis cirrhosis.

    Science.gov (United States)

    Malik, Shahid M; Devera, Michael E; Fontes, Paulo; Shaikh, Obaid; Sasatomi, Eizaburo; Ahmad, Jawad

    2009-12-01

    Recurrence of the original disease following liver transplantation is not uncommon and can lead to graft failure. There are limited data on recurrent fatty liver disease following liver transplantation. The aim of this study was to determine the incidence of recurrent fatty liver disease in patients with biopsy-proven nonalcoholic steatohepatitis, its effect on survival, and whether there are any predictive factors for recurrence. We analyzed patients undergoing liver transplantation for nonalcoholic steatohepatitis cirrhosis from 1997 to 2008 at a single center. Patients undergoing transplantation for cholestatic disease, alcohol, hepatitis C, or cryptogenic cirrhosis were controls. Ninety-eight patients underwent transplantation for nonalcoholic steatohepatitis cirrhosis. Recurrent fatty liver disease was seen in 70%, 25% had recurrent nonalcoholic steatohepatitis, and 18% had stage II/IV or greater fibrosis at a mean of 18 months. No patients with recurrent nonalcoholic steatohepatitis developed graft failure or required retransplantation at a follow-up of 3 years. No recipient or donor factors were associated with disease recurrence, although patients with recurrent nonalcoholic steatohepatitis had a higher incidence of diabetes, weight gain, and dyslipidemia at the time of diagnosis of recurrence. One-third of patients with recurrent nonalcoholic steatohepatitis had normal liver enzymes at the time of diagnosis post-transplantation. In conclusion, recurrent fatty liver disease is common following liver transplantation for nonalcoholic steatohepatitis cirrhosis but does not lead to early allograft failure. Recurrent nonalcoholic steatohepatitis can occur despite normal liver enzymes, and features of metabolic syndrome are associated with disease recurrence.

  15. Use of recurrence plot and recurrence quantification analysis in Taiwan unemployment rate time series

    Science.gov (United States)

    Chen, Wei-Shing

    2011-04-01

    The aim of the article is to answer the question if the Taiwan unemployment rate dynamics is generated by a non-linear deterministic dynamic process. This paper applies a recurrence plot and recurrence quantification approach based on the analysis of non-stationary hidden transition patterns of the unemployment rate of Taiwan. The case study uses the time series data of the Taiwan’s unemployment rate during the period from 1978/01 to 2010/06. The results show that recurrence techniques are able to identify various phases in the evolution of unemployment transition in Taiwan.

  16. Surgical management of primary and recurrent melanoma.

    Science.gov (United States)

    Farma, Jeffrey M; Kulkarni, Nandini; Hsu, Cary

    2015-04-01

    Melanoma accounts for less than 2% of skin cancer cases but causes most skin cancer-related deaths. Surgery continues to be the cornerstone of treatment of melanoma and surgical principles are guided by data derived from clinical research. This article examines the evolution of surgical techniques for the diagnosis and treatment of primary and locally recurrent melanoma.

  17. Recurrence of gestational diabetes in primiparous women

    DEFF Research Database (Denmark)

    Kruse, Anne R; Darling, Mette S; Hansen, Mia K L

    2015-01-01

    Introduction Gestational diabetes mellitus (GDM) increases the risk for diabetes in the next pregnancy and later in life. Thus, estimating the risk of GDM in further pregnancies provides a time frame for possible preventive measures. We aimed to calculate the recurrence rate of GDM in primiparous...

  18. [Multifocus or recurrent carcinoma adenoides cysticum].

    Science.gov (United States)

    Wójtowicz, P; Sujkowska, U; Kukwa, A; Sobczyk, G; Misztela, T

    1995-01-01

    Authors present the case of carcinoma adenoides cysticum, which was located in small salivary glands of palatum. After surgical treatment and radiotherapy during 3 years observation of the patient two new ca adenoides cysticum focus were noticed. It can give evidence of cancer multifocus of cancer recurrents.

  19. Recurrent Cellulitis: How Can I Prevent It?

    Science.gov (United States)

    ... M.D. To help prevent recurrent episodes of cellulitis — a bacterial infection in the deepest layer of skin — keep skin ... www.merckmanuals.com/professional/dermatologic_disorders/bacterial_skin_infections/cellulitis.html?qt=cellulitis&alt=sh. Accessed Dec. 20, ...

  20. A comprehensive profile of recurrent glioblastoma

    DEFF Research Database (Denmark)

    Campos, B.; Olsen, Lars Rønn; Urup, T.

    2016-01-01

    In spite of relentless efforts to devise new treatment strategies, primary glioblastomas invariably recur as aggressive, therapy-resistant relapses and patients rapidly succumb to these tumors. Many therapeutic agents are first tested in clinical trials involving recurrent glioblastomas. Remarkab...... 2016; doi:10.1038/onc.2016.85....