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Suzuki Yuji Y Department of Cardiology, Iwate Prefectural Ninohe Hospital, - - 2014
A 42-year-old woman with a history of aspirin-induced asthma was admitted with severe chest pain. Emergency coronary angiography revealed coronary artery spasms. The administration of vasodilators did not suppress the anginal symptoms, and the differential white blood cell count continued to show eosinophilia. The patient's symptoms of aspirin-induced asthma, eosinophilia ...
Gräni Christoph - - 2012
The present article reports a case involving a 58-year-old man without cardiovascular risk factors who was admitted to hospital with acute coronary syndrome. Coronary angiography revealed high-grade arteriosclerotic stenosis of the mid-left anterior descending coronary artery and 80% proximal intermediate branch stenosis, which were treated using three drug-eluting stents. Churg-Strauss ...
Ota Mineto - - 2012
We describe 3 siblings who suffered from marked eosinophilia with organ involvement. One sibling, who experienced cervical lymphadenopathy and peripheral neuropathy with eosinophilia (5,834 cells/μL) following bronchial asthma, was diagnosed with Churg-Strauss syndrome (CSS) according to the criteria of the American College of Rheumatology. Another sibling, who suffered from severe ...
Celik Ozlem - - 2012
Current guidelines recommend the use of inhaled corticosteroids (ICS) for suppression of airway inflammation in patients with asthma. Although it is well known that ICS cause dose-related adrenocortical suppression, it is less known that they can lead to iatrogenic Cushing's syndrome (CS). Fluticasone propionate (FP) is an ICS more potent ...
Kroesen Michiel - - 2012
We present the case of a 2-year-old asthmatic boy with atrioventricular (AV)-reentry tachycardia following albuterol inhalation, who was later diagnosed with Wolff-Parkinson-White (WPW) syndrome. The Naranjo adverse drug reaction probability scale score for this adverse event was 7, indicating that the association between his AV-reentry tachycardia and inhalation of albuterol ...
- - 2011
To the Editor: According to Herrera et al. (Sept. 1 issue),(1) the cord colitis syndrome is distinct from graft-versus-host disease (GVHD). We think that this conclusion lacks the evidence needed for defining a new disease entity and that its manifestations are concordant with a delayed GVHD reaction. Late colitis associated ...
Masuda Shigeo - - 2011
To the Editor: According to Herrera et al. (Sept. 1 issue),(1) the cord colitis syndrome is distinct from graft-versus-host disease (GVHD). We think that this conclusion lacks the evidence needed for defining a new disease entity and that its manifestations are concordant with a delayed GVHD reaction. Late colitis associated ...
Cid Joan - - 2011
To the Editor: According to Herrera et al. (Sept. 1 issue),(1) the cord colitis syndrome is distinct from graft-versus-host disease (GVHD). We think that this conclusion lacks the evidence needed for defining a new disease entity and that its manifestations are concordant with a delayed GVHD reaction. Late colitis associated ...
Matuchansky Claude - - 2011
To the Editor: According to Herrera et al. (Sept. 1 issue),(1) the cord colitis syndrome is distinct from graft-versus-host disease (GVHD). We think that this conclusion lacks the evidence needed for defining a new disease entity and that its manifestations are concordant with a delayed GVHD reaction. Late colitis associated ...
van Bekkum Dirk - - 2011
To the Editor: According to Herrera et al. (Sept. 1 issue),(1) the cord colitis syndrome is distinct from graft-versus-host disease (GVHD). We think that this conclusion lacks the evidence needed for defining a new disease entity and that its manifestations are concordant with a delayed GVHD reaction. Late colitis associated ...
Baranowski Elizabeth - - 2011
AbstractThe association in young females of long standing primary hypothyroidism, isosexual precocious pseudopuberty and multi-cystic enlarged ovaries was first described in 1960 by Van Wyk and Grumbach. Since then, sporadic case reports have contributed to clarifying the key features of this syndrome.The unique elements that lead to this diagnosis are ...
Dworakowska Dorota - - 2011
Eur J Clin Invest 2011 ABSTRACT: Background  The pathogenesis of tumour formation in the anterior pituitary including adrenocorticotropic hormone (ACTH)-secreting tumours has been intensively studied, but the causative mechanisms involved in pituitary cell transformation and tumourigenesis remain unclear. Materials and methods  We searched PubMed on any paper related with molecular ...
Pastor-Idoate S - - 2011
A 28-year-old man with bilateral central serous chorioretinopathy (CSCR) and body weight gain was diagnosed with Cushing's syndrome secondary to an adrenocortical adenoma. The patient had high levels of free cortisol and the tumor was confirmed by histopathology. After surgery, cortisol levels decreased and the CSCR spontaneously resolved. This case ...
Seretis C - - 2011
Irritable bowel syndrome (IBS) is considered to be a physical disorder that mainly affects the bowel and is clinically characterized by lower abdominal pain or discomfort, diarrhea, constipation (or alternating diarrhea/constipation), gas, bloating, and nausea. According to recent studies, it appears that there is an association with increased prolactin levels ...
Ioachimescu Adriana Gabriela - - 2011
The pituitary stalk transection syndrome was characterized after introducing the MRI scan in the evaluation of children with hypopituitarism. Its prevalence and natural history into adulthood have not been clearly established. We present 4 cases of stalk transection syndrome diagnosed by the adult endocrinologist that reflect its pleiotropic manifestations. In ...
Dacha Sunil - - 2011
We report a case of 27 year old female patient who was admitted to the hospital with an acute flare up of ulcerative colitis. The patient presented with complaints of persistent abdominal pain and bloody diarrhea despite aggressive therapy for her ulcerative colitis. A CT scan of the abdomen on ...
Tanaka Satoshi - - 2011
Objective: To describe the unique association of corticotropin-independent Cushing syndrome caused by cortisol- and androgen-secreting black adrenal cortical adenomas with myelolipomatous change.Methods: We report the clinical, laboratory, radiologic, and pathologic findings from 2 patients who presented with androgen excess and typical signs and symptoms of Cushing syndrome.Results: Endocrine investigations showed ...
Martínez-Fernández Raul - - 2011
Deep brain stimulation remains an experimental treatment for patients with Gilles de la Tourette syndrome. Currently, a major controversial issue is the choice of brain target that leads to optimal patient outcomes within a presumed network of basal ganglia and cortical pathways involved in tic pathogenesis. This report describes our ...
Haidinger Michael - - 2011
Dysregulation and hyperactivation of the mammalian target of rapamycin (mTOR) pathway define the molecular basis of the hamartoma syndromes, including Cowden syndrome, tuberous sclerosis complex (TSC)/lymphangioleiomyomatosis, and Peutz-Jeghers syndrome. Loss of the tumor suppressors phosphatase and tensin homolog (PTEN), TSC1, TSC2, and LKB1 results in uncontrolled growth of usually benign ...
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 ...
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 ...
Zhao Lihong L Howard Hughes Medical Institute, Bar Harbor, ME 04609, - - 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, ...
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. ...
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