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Chow Eva W C - - 2011
Individuals with 22q11.2 deletion syndrome are known to be at high risk of developing schizophrenia. Previous imaging studies have provided limited data on the relation of schizophrenia expression in 22q11.2 deletion syndrome to specific regional brain volumetric changes. The authors hypothesized that the main structural brain finding associated with schizophrenia ...
Akdoğan Mehmet Fatih - - 2011
A 30-year-old woman was diagnosed with ulcerative colitis in January 2006. One year later, she presented because of severe back pain and was diagnosed with ankylosing spondylitis (AS). In February 2008, the patient, while still under standard treatment for ulcerative colitis and AS, was admitted because of massive proteinuria and ...
Ogo Atsushi - - 2011
We herein describe the case of a 47-year-old woman with pre-clinical Cushing's syndrome caused by a left adrenal adenoma, which was diagnosed 6 years after trans-sphenoidal selective removal of a pituitary adenoma for acromegaly at age 35. The patient was started on bromocriptine and then somatostatin analogues after the surgery; ...
Tuţă Liliana Ana - - 2011
Introduction: Large villous adenomas or adenocarcinomas of the rectum can determine secretory diarrhea, associated with a depleting syndrome of prerenal acute renal failure, hyponatremia, hypokalemia, and hypoproteinemia, with favorable prognosis if early detected and properly treated. The syndrome is rare, with approximately 50 cases reported in the literature. Aim: Acute ...
Liu Fuyi - - 2011
McCune-Albright syndrome (MAS) is a clinical syndrome with low incidence, and its concurrence with pituitary GH adenoma is rare. Little of the history, treatment and outcome has been studied. Follow-up of a 37-year-old male patient of MAS associated with pituitary GH adenoma was performed continuously recording the disease development and ...
Awad Amer - - 2010
Sjögren syndrome (SS) has been known to manifest with neurological complications, most frequently of the peripheral nervous system, and often in advance of xerostomia and xerophthalmia. There has been one case report of a patient with SS presenting with acute motor neuropathy similar to Guillain-Barré syndrome (GBS). We report the ...
Ter Borg Evert-Jan - - 2011
Extraglandular manifestations (EGM) are often seen in patients with primary Sjögren's syndrome and are probably due to a (more) disturbed immune system. Their relation to systemic autoantibodies remains controversial. We hypothesized that positive serology as reflected by the presence of 1 of more systemic autoantibodies is related to the number ...
Kokosi Maria - - 2010
Sjögren syndrome is a slowly progressing autoimmune disease. Pulmonary manifestations are frequent in primary Sjögren syndrome but often not clinically significant; the most common are xerotrachea, interstitial lung diseases, and small airway obstruction. Pulmonary manifestations in Sjögren syndrome have a slow progression and favorable prognosis, with the exception of primary ...
Ferraccioli Gianfranco - - 2010
Sjögren's syndrome (SS) is a chronic, inflammatory, autoimmune disease characterized by lymphocytic infiltration of the exocrine glands leading to qualitatively altered and diminished or absent salivary and lachrymal secretion, and by marked B-cell hyperreactivity. Many efforts have been made to define a panel of salivary and lachrymal markers helpful to ...
Saraux Alain - - 2010
Conventionnal therapy (moisturizers, pilocarpine, Cevimeline, local Cyclosporine, and hydroxychloroquine) remains the basis for the treatment of primary Sjögren's syndrome (pSS) but they do not modify the course of the disease. Rituximab is currently the most fully evaluated biologics in pSS. Open-label studies suggest that Rituximab is well tolerated (although infusion-related ...
Rizzo William B - - 2010
Sjögren-Larsson syndrome is a genetic disease characterized by ichthyosis, mental retardation, spasticity and mutations in the ALDH3A2 gene coding for fatty aldehyde dehydrogenase, an enzyme necessary for oxidation of fatty aldehydes and fatty alcohols. We investigated the cutaneous abnormalities in 9 patients with Sjögren-Larsson syndrome to better understand how the ...
Szturmowicz Monika - - 2010
Primary Sjögren's Syndrome (pSS) is a chronic, slowly progressive inflammatory autoimmune disorder, characterised by lymphocytic infiltration of the exocrine glands, leading to decrease of glandular secretion. In 40-60% of pSS patients, extraglandular disease develops. We present the case of a patient with two extraglandular sites involvement in the course of ...
Schrader S - - 2010
Aqueous tear deficiency due to lacrimal gland insufficiency is one of the major causes of dry eye. In severe cases, such as Sjoegren's syndrome, Stevens-Johnson syndrome or ocular cicatricial pemphigoid, therapy with artificial tears can be insufficient to relieve severe discomfort. Engineering a lacrimal gland construct may offer a suitable ...
Zhao Lihong - - 2010
Endoplasmic reticulum (ER) stress has been linked to the onset and progression of many diseases. SIL1 is an adenine nucleotide exchange factor of the essential ER lumen chaperone HSPA5/BiP that senses ER stress and is involved in protein folding. Mutations in the Sil1 gene have been associated with Marinesco-Sjögren syndrome, ...
Witte Torsten - - 2010
Establishing a diagnosis of Sjögren's syndrome (SS) has been difficult without sensitive laboratory markers, and in light of the non-specificity of the symptoms of dry eyes and mouth. Rather than complaints about dry eyes or dry mouth, objective symptoms and extraglandular manifestations should raise suspicion of SS. This evaluation requires ...
Obinata Kenichi - - 2010
OBJECTIVE: The present study examined the reliability and correlation of sialography, salivary gland biopsy, and ultrasonography for Sj?gren syndrome (SS) and evaluated the usefulness of ultrasonography as a diagnostic tool for SS compared with sialography and histopathology. STUDY DESIGN: Seventy-three patients who underwent sialography, ultrasonography, and salivary gland biopsy were ...
Whitcher John P - - 2010
To describe, apply, and test a new ocular grading system for assessing keratoconjunctivitis sicca (KCS) using lissamine green and fluorescein. Prospective, observational, multicenter cohort study. The National Institutes of Health-funded Sjögren's Syndrome International Registry (called Sjögren's International Collaborative Clinical Alliance [SICCA]) is developing standardized classification criteria for Sjögren syndrome (SS) ...
Mavragani Clio P - - 2010
Sjogren's syndrome (SS) is a slowly progressing autoimmune disease, affecting predominantly middle-aged women, with a female to male ratio reaching 9:1. It is characterized by lymphocytic infiltration of the exocrine glands, mainly the lacrimal and salivary glands, resulting in reduced secretory functions and oral and ocular dryness. The syndrome can ...
Chiorini J A - - 2009
Sjögren syndrome is an autoimmune disease characterized by hyposecretion of the lacrimal and salivary glands, resulting in dryness of the eyes and mouth. Individuals may experience primary Sjögren syndrome or a secondary form accompanying another rheumatic autoimmune disease, such as rheumatoid arthritis or systemic lupus erythematosus. The pathogenic mechanisms of ...
Nikolov Nikolay P - - 2009
To summarize recent developments in our understanding of the pathogenesis of Sjögren's syndrome with a focus on the relationship between inflammation and exocrine dysfunction. Animal models demonstrated the complex interactions between immunologic and nonimmunologic mechanisms in Sjögren's syndrome. Activation of the innate immune system can lead to exocrine dysfunction before ...
Palaniswamy Chandrasekar - - 2009
A middle-aged woman presented with insidious onset swelling of bilateral lacrimal and parotid glands. Mikulicz syndrome is a symptom complex caused by a variety of systemic disorders like lymphoma, sarcoidosis, amyloidosis, human immunodeficiency virus infection, tuberculosis, etc. Biopsy from her lacrimal glands revealed mantle cell lymphoma. Although involvement of salivary ...
Serefhanoglu Songul - - 2010
Autoimmune diseases are defined as specific, adapted immune reactions against self antigens. Immune mechanism deficiency is a causal factor for B-cell lymphoma in primary Sjögren's syndrome and autoimmune thyroid disease. Thyroid mucosa-associated lymphoid tissue (MALT) lymphoma is a 67-fold and parotid lymphoma is a 44-fold increased risk in Sjögren's syndrome ...
Margaix-Muñoz Maria - - 2009
Sjögren's syndrome is one of the most frequent autoimmune diseases. It is a chronic and systemic disorder predominantly found in women, and is characterized by the appearance of a lymphocytic inflammatory infiltrate, with dryness of the oral cavity and eyes, secondary to involvement of the salivary and lacrimal glands. The ...
Iwata Naomi - - 2009
The therapeutic efficacy of corticosteroids and immunosuppressants on secretary glands of children with Sjögren syndrome was investigated examining the lip-biopsy specimen on both lymphocyte infiltration and fibrosis. Six children with primary Sjögren syndrome and two children with lupus-associated secondary Sjögren syndrome were evaluated according to the intensity of therapy. The ...
Dölen Gül - - 2009
Autism is an umbrella diagnosis with several different etiologies. Fragile X syndrome (FXS), one of the first identified and leading causes of autism, has been modeled in mice using molecular genetic manipulation. These Fmr1 knockout mice have recently been used to identify a new putative therapeutic target, the metabotropic glutamate ...
Kruszka Paul - - 2009
Sjögren syndrome is a systemic autoimmune disease characterized by dry eyes and dry mouth. Other organ systems are affected in many patients. Sjogren syndrome is classified as primary or secondary. In primary disease, Sjogren syndrome is a solitary process, whereas secondary disease accompanies another autoimmune disease--often rheumatoid arthritis. Sjögren syndrome ...
Vinagre F - - 2009
Salivary gland scintigraphy (SGS) is a non invasive method of salivary gland function assessment. This technique is easy to perform, reproducible and well tolerated by patients. Additionally, an abnormal salivary gland scintigraphy result is accepted by the American-European consensus group as a criterion for the diagnosis of Sjögren's syndrome. Scintigraphic ...
Buonuomo P S - - 2009
A 12-year-old girl presented at the Oncoemato-logic Department with an acute onset of generalized lymphadenopathy. Lymphoproliferative disorders were highly suspected. Biopsied cervical and inguinal lymph node disclosed neither malignant cells nor monoclonal proliferation of lymphocytes. Revaluating the diagnosis, anamnestic data revealed multiple episodes of bilateral parotid swelling since age one, ...
Perlat Antoinette - - 2009
Sjogren syndrome is associated with lymphoproliferative disease in 7% of cases; however, association with AL amyloidosis is uncommon. We present a patient who presented simultaneously with Sjogren syndrome (supported by dry mouth, positive Schirmer's test, anti-Ro/SSA antibodies, and a lower lip salivary gland biopsy) and AL amyloidosis revealed by heart ...
Quartuccio Luca - - 2009
Sjögren's syndrome (SS) is a systemic autoimmune disease characterized by chronic inflammation of salivary and lachrymal glands, and frequently accompanied by systemic symptoms. A subgroup of SS patients develops malignant B cell non-Hodgkin's lymphoma (NHL), usually of the mucosa-associated lymphoid tissue (MALT) type and very often located in the major ...
Delaleu Nicolas - - 2008
Sjögren's syndrome (SS) is a rheumatic disease in which the salivary and lacrimal glands are the principal targets of a pathologic autoimmune reaction. SS is manifested by xerostemia and keratoconjunctivitis sicca and marked by persistent focal mononuclear cell infiltration within the salivary glands, often accompanied by glandular atrophy and fibrosis. ...
Sato Makito - - 2008
Postmenopausal frontal fibrosing alopecia (PFFA) is a rare alopecia that develops in the frontoparietal scalp of postmenopausal women. Etiology of PFFA is unknown. Most of cases of PFFA have been reported in European and North American countries. Herein, we report a Japanese case of PFFA associated with Sjögren's syndrome. A ...
Voulgarelis Michael - - 2008
Sjögren's syndrome is a chronic inflammatory disease primarily affecting the exocrine glands. Its association with lymphoma is well documented, with salivary extranodal marginal zone lymphomas of the mucosa-associated lymphoid tissue type being the most common and constituting a major disease complication. These neoplasms are antigen-stimulated B-cell lymphomas characterized by localized ...
Schlattmann P - - 2008
Background: In order to analyze the prevalence of Raynaud's syndrome in diseases such as scleroderma and Sjögren's syndrom - a meta-analysis of published data was performed. Methods: The PubMed data base of the National Library of Medicine was used for studies dealing with Raynaud's syndrome and scleroderma or Raynaud's syndroem ...
Parke Ann L - - 2008
Sjögren's syndrome (SS) is a systemic disease with a predilection for the exocrine glands. It also is considered to be an autoimmune epitheliitis, and, as the respiratory system is lined throughout with epithelial cells, it should not be surprising that patients who have SS may develop pulmonary disease. This article ...
Koike Kentaro - - 2008
A 49-year-old man was admitted to our hospital with mild proteinuria. Prior to admission, he had been diagnosed as having Sjögren's syndrome in association with primary biliary cirrhosis. Examination of a renal biopsy under light microscopy revealed diffuse and global mesangial cell proliferation and a spike and/or bubbling formation of ...
Mirshahi Ahmad - - 2009
Sjögren-Larsson syndrome (SLS) is a rare autosomal recessive neurocutaneous disorder characterized by the triad of intellectual disability, spastic diplegia or tetraplegia, and congenital ichthyosis with associated ocular features, which include macular glistening dots. Herein, two cases of SLS are presented and their fundus autofluorescence changes, which have not been reported ...
Gálvez José - - 2009
OBJECTIVES: Our objective is to carry out a clinical study of the performance of the preliminary European classification criteria for Sjögren Syndrome and that of the criteria proposed by the American European Consensus Group. METHODS: Eighty-eight patients who had undergone a biopsy of the salivary gland on suspicion that they ...
Stefanidis I - - 2008
Sjögren syndrome (SS) is a chronic systemic autoimmune disease characterized by lymphocytic infiltration of exocrine glands, especially lacrimal and salivary. The immunologic process which occurs in this syndrome is B cell hyperactivity, which results in production of autoantibodies and immune complexes. SS can exist as a primary disorder or in ...
Béjot Y - - 2008
INTRODUCTION: The central nervous system involvement has been reported in 20% of cases of primary Sjogrën's syndrome (SS), a chronic autoimmune disease characterized by a disorder of the exocrine glands secondary to progressive lymphocyte infiltration. Classically described neurological manifestations include sensorimotor deficits, aseptic meningitis or meningoencephalitis, multiple sclerosis-like syndromes and ...
Ou Tsan-Teng - - 2008
INTRODUCTION: To investigate the association of IkBalpha promoter polymorphisms with the development of primary Sjögren's syndrome in Taiwan, 98 patients with primary Sjögren's syndrome and 110 unrelated healthy controls were enrolled in this study. MATERIALS AND METHODS: The IkappaBalpha -881 A/G, IkappaBalpha -826 C/T, IkappaBalpha -550 A/T, IkappaBalpha -519 C/T, ...
Liu, Jingmei
Primary biliary cirrhosis(PBC) is a slowly progressive autoimmune disease of the liver which mainly affects women aged between 35 and 45 years.Prolonged liver inflammation can cause scarring, leading to cirrhosis. Although 50 to 60 percent of patients are asymptomatic at diagnosis, they will develop symptoms later. PBC can be associated ...
Szanto Antonia - - 2008
Presence of autoantibodies to alfa-fodrin was investigated in patients with Sjögren's syndrome (n = 61), Hashimoto thyroiditis (n = 27), Sjögren's syndrome associated with Hashimoto thyroiditis (n = 31) and in healthy persons (n = 77). In each group, level of alfa-fodrin antibodies was higher than in the controls. There ...
Mathews S A - - 2008
Sjögren's syndrome is a common autoimmune rheumatic disease. The most common symptoms of Sjögren's syndrome are extreme tiredness, along with dry eyes (keratoconjunctivitis sicca) and dry mouth (xerostomia). Saliva plays an essential role in numerous functions of the mouth. Xerostomia can be caused by medications, chronic diseases like Sjögren's syndrome, ...
Shimizu Mayumi - - 2008
OBJECTIVE: To evaluate the usefulness of the vascularity in parotid glands in sonographic diagnosis for Sjögren syndrome. STUDY DESIGN: Sonographic images of 72 cases of previously suspected Sjögren syndrome (including 43 actual cases) were analyzed retrospectively for the abnormal vascularity in the parotid gland parenchyma. The relationships between the vascularity ...
Comer D M - - 2008
A 43-year-old woman presented with a sudden onset of hypokalaemic paralysis requiring intubation and ventilatory support. Subsequent biochemical and clinical assessments established a diagnosis of distal renal tubular acidosis (RTA) in association with underlying Sjögren's syndrome as the aetiology of her profound hypokalaemia. Distal RTA is rare, but Sjögren's syndrome ...
Katsikas Georgios A - - 2008
We describe a 62-year-old female with primary Sjögren syndrome and myopathy, severe osteoporosis, and vertebral fractures that were attributed to celiac disease. A year after the diagnosis, she developed a skin nodule on the extensor surface of her right elbow, which was due to an amyloid deposit of AA type. ...
El-Gamal Yehia - - 2008
The rate of reporting of childhood Churg-Strauss syndrome (CSS) has increased lately because of either increased awareness to the disease or a real increase in incidence. It is defined as one of the antineutrophil cytoplasmic antibody-associated vasculitides, but the antineutrophil cytoplasmic antibody positivity is less reported in pediatric cases. The ...
Rigante Donato - - 2008
The aberrant induction of salivary/lacrimal proteins is considered to be crucial in the pathogenesis of sicca-symptoms related to primary Sjögren syndrome (SS). We report the case of an 11-year-old boy who was admitted to hospital due to recurrent bilateral parotid gland enlargement and keratoconjunctivitis, which were diagnosed as primary SS ...
Sahlin Sven - - 2008
The injection of botulinum toxin into the medial lower eyelid causes a local paralysis of the orbicularis oculi muscle. The paralysis leads to a decreased action of the lacrimal pump and an improved lubrication of the ocular surface. The injection reduces the discomfort in eyes of patients with Sjögren's syndrome.
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