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Results 301 - 350 of 798
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Fujimoto T - - 2001
We report a case of hypokalaemic, flaccid quadriparesis with sudden respiratory arrest in a 27-year-old Japanese woman in whom the discovery of distal renal tubular acidosis led to the diagnosis of primary Sjögren's syndrome (SS). Hypokalaemic periodic paralysis as the initial manifestation of primary SS is rare, but when it ...
Hamburger J - - 2001
This article provides a brief overview of the various orofacial presentations of Sjögren's syndrome. The paper summarises the clinical features of patients with xerostomia, and alludes to differential diagnosis, investigative procedures and therapeutic modalities for the oral component of Sjögren's syndrome.
Panchovska M - - 2001
The primary Sjögren's syndrome (pSS) is an autoimmune exocrinopathy which is presenting with progressive dryness of the eyes and the mouth, changes in the parotid glands and not rarely with systemic (extraglandular) manifestations. The development of the immunogenetics proved that the course of the disease is genetically determined. HLA-antigens, associated ...
Asmussen K - - 2001
Primary Sjögren's syndrome is a chronic systemic rheumatic disease characterized as an autoimmune exocrinopathy or autoimmune epithelitis thereby suggesting a pathogenesis leading to tissue specific autoimmune lesion. The development of internationally approved criteria for the classification and diagnosis of Sjögren's syndrome has been a major scientific task for nearly two ...
Okubo H - - 2001
Sjögren's syndrome (SS) is known to be associated with pseudolymphoma in several organs. We describe a patient with SS complicated by a hepatic pseudolymphoma. Although the development of a hepatic pseudolymphoma is extremely rare, this disorder should be taken into consideration in the differential diagnosis of space occupying lesions related ...
Brayer J B - - 2001
Sjögren's syndrome is a chronic autoimmnune disorder characterized primarily by the discomforts od dry eyes and dry mouth due to the progressive loss of exocrine gland function. Development of a number of animal models to study Sjögren's syndrome, especially the NOD mouse and its congenic partner strains, has permitted a ...
Alvarez-Linera J - - 2000
Article abstract-The authors analyzed whether the pituitary gland enlarges in intracranial hypotension syndrome by studying 11 consecutive patients. Initial MRI scans showed pituitary gland enlargement with a convex superior margin. Follow-up MRI studies revealed that the size of the gland invariably diminished in all patients. The authors hypothesize that pituitary ...
Tabbara K F - - 2000
Sjögren syndrome is a chronic systemic disease characterized by polyglandular tissue destruction leading to keratoconjunctivitis sicca (KCS) and xerostomia. Patients with primary Sjögren syndrome show evidence of KCS and xerostomia, whereas patients with secondary Sjögren syndrome suffer from KCS, xerostomia, and an autoimmune disease, most commonly rheumatoid arthritis. Certain factors ...
Schattner A - - 2000
A diagnostic delay of several years in primary Sjögren's syndrome is common, even in patients who present with sicca symptoms. It is much more likely in cases with prominent symptomatic extraglandular involvement. We report on three such patients who presented as Coomb's positive haemolytic anaemia, systemic symptoms with agranulocytosis and ...
van Blokland S C - - 2000
Sjögren's syndrome is an autoimmune disease in which lymphocytic infiltrates develop in the salivary and lacrimal glands. We have shown that dendritic cells (DC) infiltrate the submandibular gland of the nonobese diabetic (NOD) mouse, a mouse model for Sjögren's syndrome, before lymphocytic infiltration, suggesting that these antigen-presenting cells (APC) may ...
Konttinen Y T - - 2000
Sjögren's syndrome (SS) is a condition characterized by sicca symptoms and by autoimmune features. We describe two SS patients with otitis externa fibroticans/sicca. One of these 2 patients developed a lesion of the tympanic membrane making it necessary to perform a tympantomy and meatoplasty. Our findings suggest firstly that the ...
Carpenter G H - - 2000
Sjögren's syndrome is an autoimmune condition affecting the lacrimal and salivary glands and can be associated with rheumatoid arthritis and primary biliary cirrhosis. Parotid salivas collected from patients and normal controls were analysed for lactoferrin, IgA and beta2-microglobulin (measured by ELISA), and cystatin (measured by a enzyme inhibition assay). Output ...
Stiller M - - 2000
Sjögren's syndrome is a chronic inflammatory systemic autoimmune disease mainly affecting the exocrine and, particularly, the salivary and lacrimal glands. The condition usually occurs in adults. In 1994, the criteria for this syndrome were redefined in a multicenter European study. In children, Sjögren's syndrome is a rare and probably underdiagnosed ...
Izumi M - - 2000
OBJECTIVE: We correlated abnormalities on MR imaging with the plasma lipid profiles of patients with hyperlipidemia and symptoms of sicca syndrome. SUBJECTS AND METHODS: MR imaging features of the salivary glands, salivary function, immunologic abnormalities, and plasma lipid profiles were analyzed in 24 patients with hyperlipidemia and symptoms of sicca ...
Gál I - - 2000
OBJECTIVE: To measure serum levels of anti-Ro52-kD/SSA, anti-Ro60-kD/SSA and anti-La/SSB autoantibodies in patients with primary and secondary Sjögren's syndrome. To examine if there is any connection between the disease and the subtype-spectrum of these antibodies. METHODS: We measured serum levels of anti-Ro52-kD/SSA, anti-Ro60-kD/SSA and anti-La/SSB autoantibodies by ELISA, in the ...
Baccaglini L - - 2000
Sjögren's syndrome is a chronic systemic disease that primarily affects the salivary and lacrimal glands. The pathogenesis of Sjögren's syndrome is unknown. We hypothesize that reduced somatostatin activity is an important factor in promoting immune dysregulation in patients affected by Sjögren's syndrome. Somatostatin is a multifunctional peptide with potent immunomodulatory ...
Chu K - - 2000
Sjögren's syndrome (SS) is a systemic lymphoproliferative, autoimmune disease, which is characterized by dryness of the eyes, mouth, and other mucous membranes. The nervous system may be affected in up to 20% of the cases of primary or secondary SS. We present a case of a 54-year-old woman with trochlear ...
Saatci O A - - 2000
A number of ichthyosis syndromes may have retinal abnormalities such as the retinitis pigmentosa-like diffuse rod-cone dystrophy in Refsum's syndrome and the maculopathy in Sjögren-Larsson syndrome. We present two sisters who have an unusual, almost identical, bilaterally symmetric central retinal dystrophy associated with ichthyosis vulgaris in the absence of other ...
Yoshimura M - - 2000
We report here a patient with mucosa associated lymphoid tissue (MALT)-lymphoma of the breast complicated with Sjögren syndrome. It is speculated that Ga-67 could accumulate not only in lymphoma lesions but also in benign lymphoproliferative locations of Sjögren syndrome. Gallium-67 scintigraphy might be useful for the diagnosis and therapeutic monitoring ...
Vale D L - - 2000
Sjögren's syndrome is an autoimmune disorder characterized by lymphocytic infiltration of salivary and lacrimal glands. The systemic production of autoantibodies leads to dry eyes, dry mouth, and other symptoms related to decreased salivary and lacrimal gland function in patients with Sjögren's syndrome. This article discusses origins, epidemiology, contributing factors, symptoms, ...
Aguglia U - - 2000
We report on 2 brothers (aged 19 and 12 years) with Marinesco-Sjögren syndrome who also had very low serum vitamin E concentrations with an absence of postprandial chylomicrons. The molecular study ruled out ataxia with isolated vitamin E deficiency, abetalipoproteinemia, and hypobetalipoproteinemia. The electron microscopy of the intestinal mucosa was ...
Henriksson G - - 2000
OBJECTIVE: The purpose of this study was to examine whether antibodies against CD4 are present in patients with primary Sjögren's syndrome, and to explore the possible correlation between these antibodies and the CD4+ T lymphocyte depletion that is seen in some Sjögren patients. METHODS: Sera from 214 patients with primary ...
Aung W - - 2000
This study compared the quantitative parameters of salivary gland scintigraphy and the sialographic stages in patients with Sjögren's syndrome. METHODS: One hundred sixteen patients suspected of having Sjögren's syndrome were examined with salivary gland scintigraphy and contrast sialography. When contrast sialography was used as the gold standard, Sjögren's syndrome was ...
Jung S M - - 2000
Sjögren's syndrome is a chronic autoimmune disorder characterized by lymphocytic infiltration of the lacrimal and salivary glands, leading to dryness of eyes (kerato-conjunctivitis sicca) and mouth (xerostomia). The skin lesions in Sjögren's syndrome are usually manifested as xeroderma, but sometimes appear as annular erythema or vasculitis. Central nervous system symptoms ...
Sutcliffe N - - 2000
We report two patients with rheumatoid arthritis and secondary Sjögren's syndrome whose disease was complicated by a mucosa-associated lymphoid tissue (MALT) lymphoma. Although this particular type of lymphoma is associated with primary Sjögren's syndrome, it has not been described, to our knowledge, in the context of rheumatoid arthritis and Sjögren's ...
Willemsen M A - - 2000
In 6 patients with Sjögren-Larsson syndrome (SLS), the urinary excretion of leukotriene B(4) (LTB(4)) and omega-hydroxy-LTB(4) was found to be highly elevated, whereas omega-carboxy-LTB(4) was absent. This abnormal pattern of urinary excretion of LTB(4) and its metabolites appears to be unique to patients with SLS and offers a new approach ...
Persaud N A - - 2000
Gustatory sweating syndrome involving the submandibular gland is rare. We present a case of a patient who experienced this syndrome 5 years after undergoing submandibular gland resection. Our patient was satisfied simply with an explanation of the disorder and reassurance. But in cases where further intervention is sought, medical and ...
Young C - - 2000
A case is described involving Sjögren's syndrome, high sustained levels of antibodies to serine proteinase 3, and cavitating lung disease. Possible diagnoses accounting for this unusual combination include a novel association of Sjögren's syndrome and Wegener's granulomatosis (suggested by the high and sustained levels of antibodies to serine proteinase 3) ...
Nakajima H - - 2000
We report a 79-year-old female with anaphylactoid purpura on her legs and unusual large annular purpura on the trunk. Histopathological characteristics of leukocytoclastic vasculitis were observed in the upper and middle dermis of both types of skin lesions. She was complicated by Sjögren's syndrome and advanced cervical cancer. The annular ...
Jonsson R - - 2000
Sjögren's syndrome is a chronic autoimmune and rheumatic disorder. Most patients have mild to moderate complaints and this may explain the great discrepancy in prevalence found in population studies compared to studies performed in the clinic. However, there is no straightforward and simple diagnostic test for Sjögren's syndrome, although several ...
Tumiati B - - 2000
Heart rate variability (HRV) gives information about sympathetic parasympathetic autonomic balance. Our purpose was to determine whether HRV is abnormal in patients with Sjögren's syndrome. In 16 patients with Sjögren's syndrome and 30 matched controls, a short time analysis of HRV was performed for both the frequency and the time ...
Hermann G A - - 1999
An abnormal salivary scintigram is an accepted objective criterion in the diagnosis of primary and secondary Sjögren's syndrome, an immune-mediated disorder characterized by xerostomia and kerato-conjunctivitis sicca. However, chronic sialadenitis constitutes a major differential diagnostic consideration in the xerostomic population. We investigated 39 cases of biopsy-confirmed chronic sialadenitis and 152 ...
Lacomis D - - 1999
We report a 63-year-old woman with common variable immunodeficiency (CVID) and an indolent distal sensory neuropathy. Despite having negative serologic tests for Sjögren's syndrome, evaluation of the neuropathy led to a new diagnosis of Sjögren's syndrome based on the presence of sicca symptoms, an abnormal Schirmer's test, and histologic evidence ...
Hashimoto S - - 1999
A 67-year-old woman developed severe sicca manifestations after initial treatment of Parkinson's disease with an anti-cholinergic drug, which prompted us to look for the presence of Sjögren's syndrome. The results of sialography, labial salivary gland biopsy, Rose-Bengal test as well as the presence of antinuclear antibody were consistent with the ...
Beby-Defaux A - - 1999
Human T-cell lymphotropic virus type I (HTLV-I) is associated with adult T-cell leukemia (ATL) and tropical spastic paraparesis/HTLV-I-associated myelopathy (TSP/HAM). Other inflammatory disorders may occur in HTLV-I-infected patients, such as sicca syndrome resembling Sjögren's syndrome. The sicca syndrome may be the unique clinical manifestation of HTLV-I infection, but is associated ...
Rhodus N L - - 1999
Sjögren's syndrome (SS) is a progressive autoimmune rheumatic disorder. Its precise etiology is unknown, although several contributing factors have been identified. One theory is that the condition results from complications related to infection with the Epstein-Barr virus. Primary exposure to or reactivation of Epstein-Barr virus elicits expression of the human ...
Haddad F S - - 1999
Sjögren-Larsson syndrome is a rare autosomal recessive neurocutaneous disorder in which the combination of spasticity, ichthyosis, and mental retardation commonly result in patients being wheelchair bound. We reviewed a cohort of children with Sjögren-Larsson syndrome who were successfully managed with early physiotherapy and later soft-tissue surgery. The favourable outcome seen ...
Sorajja P P Department of Internal Medicine, Mayo Clinic Rochester, Minn. 55905, - - 1999
Autonomic failure and proximal skeletal myopathy are rare features of the Sjögren syndrome (SS). We describe a 51-year-old woman with primary SS who had development of esophageal dysmotility, urinary retention, severe orthostatism, and skeletal myopathy during a 3-month period after the diagnosis of SS. Her symptoms and signs responded well ...
Sullivan D A - - 1999
Sjögren's syndrome is an extremely complex and currently incurable autoimmune disorder, which occurs primarily in females, and is associated with lacrimal gland inflammation, meibomian gland dysfunction, and severe dry eye. We hypothesize that androgen deficiency, which reportedly occurs in primary and secondary Sjögren's syndrome (e.g., systemic lupus erythematosus, rheumatoid arthritis), ...
Takabatake N - - 1999
We experienced a rare case of lung adenocarcinoma associated with lymphocytic interstitial pneumonitis caused by primary Sjögren's syndrome. A 78-year-old woman was referred to our hospital because of progressive sicca syndrome and nodular opacities in the right lower lobe on chest radiograph. This patient was diagnosed as primary Sjögren's syndrome ...
Fox P C - - 1999
Microdissection of biopsy material from labial minor salivary glands followed by RT-PCR demonstrates extensive production of cytokines IL-2, 1L-6, IL-10, TNF-alpha, TGF-beta, and IFN-gamma in both normal glands and in glands affected by Sjögren's syndrome. Continuation of these studies should expand our knowledge of normal salivary gland function and the ...
Kalk W W - - 1999
A case of primary sialoangiectasia, which in this case was initially misdiagnosed as Sjögren's syndrome, is described. Other diseases, including HIV infection, psoriatic arthritis, and acute parotitis, may cause glandular changes similar to the changes found in the syndrome. Therefore, sialography must be combined with other methods of assessment of ...
Iltanen S - - 1999
OBJECTIVE: Many autoimmune diseases occur concomitantly with celiac disease. We investigated prospectively the occurrence of celiac disease and small-bowel mucosal inflammation in patients with primary Sjögren's syndrome. METHODS: A total of 34 patients with primary Sjögren's syndrome and 28 controls underwent small bowel biopsy. Villous morphology, jejunal intraepithelial lymphocytes, and ...
Tishler M - - 1999
Salivary and serum concentrations of soluble interleukin-2 receptor (sIL-2R) were studied in a group of patients with Sjögren's syndrome and a group suffering from dry mouth. Salivary sIL-2R levels was significantly higher (57.9+/-15.1 vs 16.7+/-4.7 pg/ml) (p < 0.05) in the group of 26 patients with Sjögren's syndrome than in ...
Hermann G A - - 1999
PURPOSE: Three decades of work to enhance the diagnostic accuracy of salivary scintigraphy have generated various plausible decision criteria. This study evaluates four commonly cited numeric indices in studies of xerostomic populations and how accurately they identify Sjögren's syndrome, chronic sialadenitis, radiation sialadenitis, and drug effects and distinguish each from ...
Franquet T - - 1999
PURPOSE: The purpose of our study was to correlate the extent of air trapping on expiratory CT scans with results of pulmonary function tests (PFTs) in a large group of patients with primary Sjögren syndrome and to determine whether these techniques may be complementary in assessing small airways obstruction. METHOD: ...
al-Jubouri M A - - 1999
A 73 year old white man presented with life threatening hypokalaemic paralysis requiring admission to an intensive care unit. Biochemical investigations showed severe hypokalaemia with hyperchloraemic metabolic acidosis, a spot urine pH of 6.5, and a positive urinary anion gap, establishing the diagnosis of distal renal tubular acidosis. Autoimmune tests ...
Bjerrum K B - - 1999
PURPOSE: To study and compare the glycoprotein composition of tear fluid from patients with primary Sjögren's syndrome, patients with other connective tissue diseases and control individuals. METHODS: SDS polyacrylamide gel electrophoresis followed by Coomassie staining or Western blotting and overlaying with ten different lectins. RESULTS: The frequency of many glycoprotein ...
Wright R A - - 1999
The authors present a patient with Sjögren's syndrome with a fluctuating and then progressive myelopathic syndrome, and optic nerve involvement. Treatment with chlorambucil and prednisone improved the patient's function from being wheelchair bound to walking unaided. Spinal MRI showed multiple, extensive intraparenchymal areas of abnormal T2-weighted signal intensity, gadolinium enhancement, ...
Pigg M - - 1999
Sjögren-Larsson syndrome is an autosomal recessive disorder characterized by congenital ichthyosis, spastic di- or tetraplegia and mental retardation. In 1994 Sjögren-Larsson syndrome was mapped to chromosome 17, close to the genetic marker D17S805 in a study of 24 Swedish families. We have analysed 12 microsatellite markers in 10 additional non-Swedish ...
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