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ID PMID Title PublicationDate abstract
16490754 Incidence of cancer in a cohort of patients with primary Sjogren's syndrome. 2006 Aug OBJECTIVE: To compare the incidence of subsequent cancers in a cohort of patients with primary Sjögren's syndrome (pSS) with that of the general population in the same region of England. METHODS: A retrospective analysis was carried out on 112 patients who had attended the out-patients department at University College Hospital, London, from 1979 onwards. Patients were followed up from diagnosis of pSS to diagnosis of first subsequent cancer, death, loss to follow-up or 31 December 2003 (the censoring date) to determine the person-years at risk for each individual. The expected numbers of subsequent cancers were calculated from sex-/age-/period-specific rates for the general population of southeast England, derived from registrations at the Thames Cancer Registry. Standardized incidence ratios (SIRs) were then calculated as the ratio of observed to expected numbers of cancers, along with 95% confidence intervals (CIs). Separate analyses were performed for all malignant cancers combined, lymphomas and non-lymphoid cancers. RESULTS: Among the 112 patients with pSS, 25 developed cancer (either before or after development of pSS), with lymphoma occurring in 11 cases. Nine patients had two cancers. There was a significantly elevated incidence of lymphomas in pSS patients compared with the general population (SIR 37.5, 95% CI 20.7-67.6). For non-lymphoid cancer, the observed increase in incidence was small and not statistically significant (SIR 1.5, 95% CI 0.9-2.6). CONCLUSION: This study confirms that there is an increased incidence of lymphoma in patients with pSS. An increase in the incidence of other cancers was not proven but the observation that some patients developed more than one cancer raises the possibility that there may be a subgroup of patients who are at greater risk of developing cancer.
16870094 A community-based cohort of 201 consecutive patients with primary Sjögren's syndrome in I 2006 May OBJECTIVE: To determine the spectrum and prevalence of the varied manifestations, associated conditions and laboratory abnormalities of patients with primary Sjögren's syndrome in Israel and compare them between individuals of Sephardic and Ashkenazi descent and with data from the literature. METHODS: A retrospective study of a cohort of 201 consecutive patients diagnosed and followed at a single academic medical center. All cases were diagnosed using stringent criteria according to the American European Concensus Group including a labial minor salivary gland biopsy in all cases. RESULTS: Patients' mean age was 57 years and 84% were women. Overall, more than 98% of patients had sicca symptoms of dry eyes and mouth. About 35% of the cohort had hematological manifestations--primarily immune cytopenias, protein immunoelectrophoresis abnormalities and lymphoma. About 20% had associated neurological conditions (not only peripheral but often central nervous system) and 15% had pulmonary involvement. In addition, thyroid disease, liver disease, vascular or cutaneous manifestations, synovitis, ocular and renal disease could be found. In fact, the presenting manifestation was extraglandular or an abnormal test result in 39% of the patients. CONCLUSION: No significant differences were found in glandular or extraglandular manifestations or laboratory test results between Ashkenazi and Sephardic patients, despite their genetic differences. A negative history of sicca symptoms effectively rules out primary Sjögren's syndrome in this cohort. These symptoms may not be volunteered by patients and the large variety of extraglandular involvement patterns and associated conditions observed may dominate the patient's presentation, and mandate physicians' awareness and a high index of suspicion for a timely diagnosis.
16821274 Epitope mapping of anti-alpha-fodrin autoantibody in juvenile Sjögren's syndrome: differe 2006 Jul OBJECTIVE: Juvenile Sjögren's syndrome (SS) is an early-onset type of SS. Autoantibody against the N-terminal 120 kDa form of a-fodrin is a specific and sensitive disease marker for both juvenile and adult SS. We investigated the initial and major determinants of a-fodrin in SS. METHODS: Sera were obtained from patients with juvenile SS, 10 with primary SS and 10 with secondary SS. Epitope specificities of IgG antibodies were examined by dot-blot analyses using overlapping fusion proteins of the N-terminal part (561 amino acid residues) of a-fodrin as antigens. RESULTS: All sera from patients with primary SS reacted with amino acid residues 1 to 98 and 36 to 150, but not with 91 to 199. Epitope mapping using fusion proteins with subfragments, each consisting of about 50 amino acid residues, showed reactivity with amino acid residues 27-80 and 79-132, suggesting that at least 2 epitopes are contained in the first 150 amino acid residues. All 3 cases with neurological complications had additional epitope specificities. Sera from patients with secondary SS showed more diversified specificities and strongly reacted with amino acid residues 1-98 and 334-432, whereas the reactivities to 36-150, a major epitope in primary SS, were minimal. CONCLUSION: Major and initial B cell epitopes specifically reside in N-terminal amino acids 36-132 and could be used as a diagnostic tool for primary SS. The epitope subsequently expands to other regions of a-fodrin in association with the development of neurological complications or disease progression. Secondary SS has distinct epitope specificities.
15653590 Imaging of intraarticular masses. 2005 Jan Intraarticular masses can be classified as noninfectious synovial proliferative processes (lipoma arborescens, synovial osteochondromatosis, pigmented villonodular synovitis, rheumatoid arthritis), infectious granulomatous diseases (tuberculous arthritis, coccidioidomycosis arthritis), deposition diseases (gout, amyloid arthropathy), vascular malformations (synovial hemangioma, arteriovenous malformations), malignancies (synovial chondrosarcoma, synovial sarcoma, synovial metastases), and miscellaneous (cyclops lesion). Knowledge of articular anatomy aids the radiologist in localizing masses to the joint space. Some joints have complex anatomy with contiguous or adjacent bursae, recesses, and tendinous connections from which masses may originate or into which masses may extend. Many of the diseases causing intraarticular masses have specific imaging characteristics, especially on magnetic resonance images, and knowledge of these characteristics will allow for a more confident diagnosis.
17162369 Increased Fas and Bcl-2 expression on peripheral blood T and B lymphocytes from juvenile-o 2006 Jun Defective regulation of apoptosis may play a role in the development of autoimmune diseases. Fas and Bcl-2 proteins are involved in the control of apoptosis. The aims of this study were to determine the expression of Fas antigen and Bcl-2 protein on peripheral blood T and B lymphocytes from patients with juvenile-onset systemic lupus erythematosus (JSLE), juvenile rheumatoid arthritis (JRA) and juvenile dermatomyositis (JDM). Thirty-eight patients with JSLE, 19 patients with JRA, 10 patients with JDM and 25 healthy controls entered the study. Freshly isolated peripheral blood mononuclear cells (PBMC) were stained for lymphocyte markers CD3, CD4, CD8, CD19 and for Fas and Bcl-2 molecules. Expressions were measured by three-color flow cytometry. Statistical analysis was performed using Kruskal-Wallis test. Percentages of freshly isolated T lymphocytes positively stained for Fas protein from JSLE patients were significantly increased compared to healthy controls, patients with JRA and patients with JDM. Percentages of B lymphocytes positive for Fas from JSLE patients were higher than healthy controls and JRA patients. In addition, Fas expression on T cells from patients with JRA was increased compared to JDM patients. Otherwise, Fas expression on T and B cells from JRA and JDM patients were similar to healthy controls. MFI of Bcl-2 positive T lymphocytes from JSLE patients were significantly increased compared to healthy controls and JRA patients. MFI of Bcl-2 protein on B lymphocytes from JSLE patients was similar to healthy controls and patients with JRA and JDM. Bcl-2 expression did not differ between JRA and JDM patients and healthy controls. In conclusion, increased expression of Fas and Bcl-2 proteins observed in circulating T and B lymphocytes from patients with JSLE, but not from patients with JRA and JDM, suggests that abnormalities of apoptosis may be related to the pathogenesis of JSLE and probably are not a result of chronic inflammation.
15378386 Ocular manifestations of juvenile rheumatoid arthritis in Olmsted County, Minnesota: a pop 2005 Mar BACKGROUND: Juvenile rheumatoid arthritis (JRA) is the most common systemic cause of uveitis in Europe and North America. The cumulative incidence of uveitis in JRA has been reported at between 8.5% and 25% in series from referral centers in the USA. There have been no population-based studies of the cumulative incidence of uveitis in JRA in the USA. METHODS: We performed a population-based, retrospective cohort study of patients residing in Olmsted County, Minnesota between 1 January 1960 and 31 December 2000 who met American College of Rheumatology diagnostic criteria for JRA. The patients were identified using the Rochester Epidemiology Project (REP), a surveillance and medical records-linkage system which provides access to medical records of residents of Olmsted County. Patient histories were reviewed and information regarding rheumatic and ocular disease was extracted and analyzed. The main outcome measures were: cumulative incidence of uveitis, of complications of uveitis, of keratoconjunctivitis sicca (KCS) and of adverse visual outcome. RESULTS: Of the 88 patients identified, three patients developed uncomplicated uveitis [3.4%; 95% confidence intervals [CI] 0.7-9.6%), all with pauciarticular onset JRA. Two patients developed KCS (2.3%; 95% CI 0.3-8.0%). The visual acuity of these five patients at last follow-up (mean length of follow-up 22.6 years, range 8-36 years) was 20/20. There were no patients with visual loss attributable to JRA. CONCLUSIONS: In a population-based study of JRA in the United States, uveitis occurred at a lower frequency than expected. In the limited number of cases in this cohort with JRA-associated ophthalmologic complications there was no resulting loss of visual acuity.
16783460 Antigen-specific expansion of TCR Vbeta3+ CD4+ T cells in the early stage of collagen-indu 2006 May To investigate type II collagen (CII)-specific CD4+ T cell receptors involving in Collagen-induced arthritis (CIA) in DBA/1J mice as a model of rheumatoid arthritis in humans, TCR Vbeta usage in draining lymph nodes (dLNs) was assessed by flow cytometric analysis at 3, 5, and 8 weeks after bovine CII immunizations. In the early stage of CIA, the draining lymph node CD4+ T cells from CIA mice showed a higher proportion of CD4+ Vbeta3+ subsets compared with those from control mice. The CD4+ Vbeta3+ T cells were specifically and primarily expanded by antigen-specific stimulation in in vitro culture of dLNs lymphocytes and splenocytes from CIA mice. In addition, CII-reactive response was observed when CD4+ Vbeta3+ T cells were added to a non-responding T cell population. The adoptive transfer of CD4+ Vbeta3+ T cells produced exaggerated arthritis compared with that in the control group. Our results indicate that CD4+ Vbeta3+ T cells, which were selectively expanded in dLN of CIA mice, play a pivotal role in CIA pathogenesis.
15921024 Methotrexate treatment suppresses local cytokine and chemokine production in rat adjuvant 2005 Rheumatoid arthritis (RA) is a disease that is still insufficiently controlled by current treatments. Methotrexate (M7X), a small molecular weight compound, has been the gold standard in the treatment of RA. It has several anti-inflammatory activities, but their contribution to its antiarthritic effects has not yet been established. We conducted a rat adjuvant arthritis study, in which we investigated the effect of MTX on local cytokine and chemokine production in arthritic paws. Our data demonstrate that MTX was able to significantly suppress cytokine and chemokine release in the inflamed joints in a dose-dependent fashion, accompanied by amelioration of the disease as indicated by reduced paw swelling and arthritic scores. These findings prompt further studies to clarify whether these suppressive effects of MTX on local cytokine and chemokine release are direct or whether they are a result of other preceding anti-inflammatory activities of the compound.
16380757 [Psoriatic arthritis: epidemiological and clinical aspects in a cohort of 1.306 Italian pa 2005 Dec Because there is the impression that psoriatic arthritis is a composite disorder with mild forms close to more severe and aggressive ones, we conducted a multicenter study with the aim of characterizing disease expression in a large cohort of Italian patients. One-thousand-three-hundred-six patients fulfilled inclusion criteria and were analyzed in this study. Psoriasis antedated the onset of arthritis in the majority of the cases (67.7%). More rare was inverse or simultaneous onset which occurred in 17.3% and 15.0% of the cases, respectively. Peripheral articular involvement (mono-oligo or polyarthritis) was recorded in 88.7% of the cases while spondylitis occurred in 11.3%. Peripheral enthesopathies were found in 28.1% of the cases with a marked occurrence in patients with axial involvement (64.5% vs 35.5% in oligo or polyarthritis). Abnormal levels of ESR and CRP respectively occurred in 52.2% and in 52.6% of the cases, while rheumatoid factor was detected in 5.0% of the cases. On the basis of distribution of joint involvement, symmetry and presence of peripheral enthesopathies we recognized three clusters of arthritis. Patients included in Cluster 1 and Cluster 2 showed a severe form of polyarthritis in most of the cases (82.9%), with increased serum levels of inflammatory indices in more than 85% of the cases. Almost all the hospitalized patients (97.1%) were included in this two clusters. They markedly assumed steroids and methotrexate or another DMARD. About half of the patients (51.1%) included in Cluster 3 showed mono-oligo articular involvement. Serum inflammatory indices were increased in 20.8% of the cases while hospitalization occurred only in 2.9% of the cases and NSAIDs were the treatment of choice. The evidence in our country of a large prevalence of severe forms of arthritis needing specific and aggressive approach outlines the requirement of an intense educational action aimed at increasing the awareness of this condition.
16704744 Inhibition of macropinocytosis blocks antigen presentation of type II collagen in vitro an 2006 Professional antigen-presenting cells, such as dendritic cells, macrophages and B cells have been implicated in the pathogenesis of rheumatoid arthritis, constituting a possible target for antigen-specific immunotherapy. We addressed the possibility of blocking antigen presentation of the type II collagen (CII)-derived immunodominant arthritogenic epitope CII259-273 to specific CD4 T cells by inhibition of antigen uptake in HLA-DR1-transgenic mice in vitro and in vivo. Electron microscopy, confocal microscopy, subcellular fractionation and antigen presentation assays were used to establish the mechanisms of uptake, intracellular localization and antigen presentation of CII by dendritic cells and macrophages. We show that CII accumulated in membrane fractions of intermediate density corresponding to late endosomes. Treatment of dendritic cells and macrophages with cytochalasin D or amiloride prevented the intracellular appearance of CII and blocked antigen presentation of CII259-273 to HLA-DR1-restricted T cell hybridomas. The data suggest that CII was taken up by dendritic cells and macrophages predominantly via macropinocytosis. Administration of amiloride in vivo prevented activation of CII-specific polyclonal T cells in the draining popliteal lymph nodes. This study suggests that selective targeting of CII internalization in professional antigen-presenting cells prevents activation of autoimmune T cells, constituting a novel therapeutic strategy for the immunotherapy of rheumatoid arthritis.
17041993 An exploratory survey of the practice of rheumatology nurses addressing the sexuality of p 2005 BACKGROUND: Rheumatoid arthritis (RA) is a chronic progressive inflammatory condition primarily affecting the joints. It is characterized by pain, stiffness and fatigue. Research has demonstrated that the symptoms of RA can negatively impact on a patient's sexuality and patients would welcome the opportunity to discuss their sexual needs with a health professional. AIMS: To identify current perceived practice, skills, and knowledge of rheumatology nurses in addressing the sexuality of patients with RA. METHODS: A postal questionnaire was sent to 132 rheumatology nurses identified from the British Health Professionals in Rheumatology Handbook. The questionnaire sought to identify the nurse's role in the assessment and management of RA patients' sexuality. RESULTS: Seventy six questionnaires were returned (response rate of 57.5%). Sixty nine respondents stated that sexuality should be included within the nursing assessment. The main factors that influenced whether sexuality was discussed was privacy, the level of knowledge and skills and time. Nurses felt that while contraceptive advice for patients treated with cytotoxic drugs was discussed in depth, the patient's sexual relationship was not discussed at all. The majority of respondents (83%) had never received any training in this area and would undergo training if they had the opportunity. CONCLUSION: While rheumatology nurses acknowledge the importance of including sexuality in the care management of patients with RA, in practice the impact of the condition on a patient's sexuality is only discussed briefly. Rheumatology nurses have identified the need for further training in this complex area.
17029076 Follow-up study of ankle arthrodesis in severe hind foot deformity in patients with rheuma 2005 We report herein a retrospective study of 25 cases of ankle arthrodesis performed in 23 patients with rheumatoid arthritis (RA) using an intramedullary nail with fins, developed in 1994. Surgical treatment, postoperative management, and clinical evaluation are described. Clinical evaluation, at an average follow-up period of 7 years 1 month, was based on foot disease scores from the Japanese Orthopedic Association; we compared these scores pre- and postoperatively, and during follow-up. These parameters showed a significant difference between preoperation and the follow-up period. However, instability only significantly improved when compared between pre- and postoperation. Arthrodesis using an intramedullary nail with fins was effective for the treatment of severe deformity of the hind foot. Nonunion was not observed and no remarkable changes of the Chopart joint were recognized between preoperation and the follow-up period. In our series, delayed wound healing was recognized in 6 of 25 joints. However, infection or neuropathy and other complications were not found. Arthrodesis using an intramedullary nail with fins is a viable treatment option for severe deformity of the hind foot in RA patients, because nonunion was not recognized and the clinical results over an average 7-year follow-up period were good or satisfactory.
17029059 Analysis for the major contributor of collagenase to the primary cleavage of type II colla 2005 Degradation of type II collagen is a central process in cartilage destruction seen in osteoarthritis and rheumatoid arthritis. Primary cleavage of type II collagen at the collagenase site is rate-limiting and is, therefore, a critical step for its degradation. The major contributor to this cleavage was identified in three isozymes of collagenase in human cartilage. Primary cultured human chondrocytes were used for the study. The production of collagenase-1 was major in total production for three isozymes of collagenase after stimulations with any concentration of tumor necrosis factor-alpha and/or interleukin-1 at 48 and 72 h, comprising 98% or greater of the total collagenase. When the production of collagenase-1 was specifically suppressed by the transfection with duplexes of 21-nucleotide small interfering ribonucleic acid into the cells, the activity of type II collagen cleavage was linearly decreased at neutral pH after activation. The relative contribution of collagenase-1 to the primary cleavage of type II collagen was determined to be 85%-93%. These findings suggest that collagenase-1 is a major contributor to the primary cleavage of type II collagens in human cartilage and is a potential therapeutic target for osteoarthritis and rheumatoid arthritis.
17029044 Malnutrition and disease progression in patients with rheumatoid arthritis. 2005 To examine the changes in nutritional status during the progression of rheumatoid arthritis (RA), we studied anthropometric and biochemical variables in 97 Japanese patients with RA. Anthropometric data included body mass index (BMI), triceps skinfold thickness (TSF), and arm muscle area (AMA). Levels of albumin and cholesterol in serum, and lymphocyte count were studied as biochemical variables. The prevalence of malnutrition defined as hypoalbuminemia less than 3.4 g/dl was 24.7%, similar to the reports in other countries. Analysis of the data according to disease stage showed that malnutrition in RA was characterized by a progressive reduction in body protein. Body mass index and TSF were increased in patients with stage 1 disease, whereas serum albumin and AMA were within normal range. Stage 2 patients had normal BMI with decreased body protein, albumin, and AMA. Progression to stages 3 and 4 was associated with a stepwise decrease in AMA; serum albumin and BMI remained in the same range as stage 2. Albumin values and AMA were significantly lower in patients with poor functional class and high C-reactive protein. The characteristic progression of malnutrition in RA is attributed to excessive protein catabolism evoked by inflammatory cytokines and by disuse atrophy due to functional impairment.
17029087 Therapeutic effects of the combination of methotrexate and bucillamine in early rheumatoid 2005 Disease-modifying antirheumatic drug (DMARD) combination therapies are used widely, but there have been few reports clearly demonstrating that combination therapy is more effective than DMARD monotherapy. We conducted a multicenter, double-blind controlled trial in order to clarify that the combination of methotrexate and bucillamine is more effective than either alone. The subjects of this study were 71 patients with active rheumatoid arthritis within 2 years of onset. Dosages were 8 mg methotrexate with 5 mg folic acid per week (MTX group), 200 mg bucillamine per day (BUC group), or both MTX and BUC (combination group). Clinical effects and adverse reactions were observed for 96 weeks. The ACR 20 response rate was 79.2% in the combination group, significantly higher than the rates of 43.5% for the MTX group (P = 0.008) and 45.8% for the BUC group (P = 0.0178). The cumulative survival curve of maintaining the ACR 20 response was significantly higher in the combination group than in the MTX and BUC groups (P = 0.0123 and P = 0.0088, respectively). The mean increase in the total Sharp score over 96 weeks was 12.6 +/- 9.0 in the combination group, significantly lower (P = 0.0468) than the value of 28.0 +/- 28.3 for the single DMARD (combined MTX and BUC) group. The incidence of adverse reactions did not differ significantly between the three groups. It was concluded that the combination therapy with MTX and BUC showed significantly higher clinical efficacy than either of the single DMARD therapies.
16539828 Tumor necrosis factor alpha promoter polymorphisms in patients with juvenile idiopathic ar 2006 Jan OBJECTIVE: To investigate the potential association of tumor necrosis factor-alpha (TNF) promoter alleles within subtypes of juvenile idiopathic arthritis (JIA) compared to healthy controls in a Caucasian population. METHODS: TNF-alpha promoter polymorphisms at positions -163, -238, -244, -308, -376 were determined in 228 patients with JIA and 196 healthy individuals. Genomic DNA was isolated and a PCR fragment of about 500 base pairs of the TNF gene promoter were amplified by PCR. Detection of polymorphisms was achieved by a single sequencing procedure. RESULTS: The TNF -238A allele was more frequent in the psoriatic arthritis JIA subgroup compared to healthy controls as well as to non-psoriatic JIA patients (p < 0.001, chi-square-test) and was associated with the more frequent occurrence of joint erosion (p < 0.05, chi-square-test). The frequency of the TNF -308A allele was significantly lower in patients with rheumatoid factor negative polyarthritis JIA patients compared to healthy controls, respectively (p < 0.05, chi-square-test). Joint erosions were detectable more often in rheumatoid factor negative polyarthritis JIA patients with the G/A genotype (80%) than in those with the G/G genotype (45%) (p = 0.20). The rare alleles at position -376 or at positions -163 and -244 were found very infrequently. CONCLUSION: TNF promoter polymorphisms may play a role in the pathogenesis of JIA. The TNF-238A allele seems to be associated with juvenile psoriatic arthritis. The TNF-308A allele is less frequently found in rheumatoid factor negative but not in rheumatoid factor positive polyarthritis and may therefore be associated with a more severe disease, while the more common TNF-308G allele may be protective.
16237655 Macrophage migration inhibitory factor: gene polymorphisms and susceptibility to inflammat 2005 Nov 15 The cytokine macrophage migration inhibitory factor (MIF) is a constitutive element of the host antimicrobial defenses and stress response that promotes proinflammatory function of the innate and acquired immune systems. MIF plays an important role in the pathogenesis of acute and chronic inflammatory or autoimmune disorders, such as sepsis, acute respiratory distress syndrome, asthma, rheumatoid arthritis, and inflammatory bowel diseases. Polymorphisms of the human MIF gene (that is, guanine-to-cytosine transition at position -173 or CATT-tetranucleotide repeat at position -794) have been associated with increased susceptibility to or severity of juvenile idiopathic and adult rheumatoid arthritis, ulcerative colitis, atopy, or sarcoidosis. Whether these MIF polymorphisms affect the susceptibility to and outcome of sepsis has not yet been examined. Analyses of MIF genotypes in patients with sepsis may help to classify patients into risk categories and to identify those patients who may benefit from anti-MIF therapeutic strategies.
17118290 Advances in the management of septic arthritis. 2006 Dec Septic arthritis still continues to be a common and serious problem at major urban medical centers and is one of the most rapidly destructive forms of acute arthritis. The yearly incidence of bacterial arthritis varies from 2 to 10 per 100,000 in the general population to 30 to 70 per 100,000 in patients with rheumatoid arthritis and in patients with joint prostheses. Irreversible loss of joint function may develop in up to 50% of the patients. Despite better antimicrobial agents and improved hospital care, the fatality rate for this medical problem has not changed substantially during the past 30 years. An understanding of the risk factors and the pathogenesis of nongonoccocal bacterial arthritis and other forms of infectious arthritis, primarily in the context of a differential diagnosis and treatment, are important to avoid the delay in making a correct diagnosis and to improve the prognosis.
17274865 [Macrophage activation syndrome in Chinese children with systemic onset juvenile idiopathi 2006 Nov OBJECTIVE: To review and analyze the clinical features, treatment, and outcome of macrophage activation syndrome (MAS) in children with systemic onset juvennil rheumatoid arthritis (SOJRA). METHOD: Retrospective review and analysis were performed on cases with MAS from a prospectively collected database of children with SOJRA from the year of 2003 to 2006 in the Hospital. RESULTS: Twenty four patients (21 boys, 3 girls) were diagnosed as having MAS with SOJRA. Mean age of the patients with MAS at diagnosis was 7 years, and the duration prior to diagnosis of MAS was 12 months. No trigger factors were found except in one case whose MAS was triggered by use of methotrexate and in another by parvovirus B19 infection. High grade fever, new onset hepatosplenomegaly and lymphadenopathy, pancytopenia, liver dysfunction were common clinical features in all the 24 cases (100%). Bleeding from skin, mucous membrane and gastrointestinal tract were noted in 9 cases (38%). Twelve (50%) cases had CNS dysfunction (high intracranial pressure, seizure and coma). Six cases (25%) developed ARDS. One patient suffered from renal damage. The laboratory test revealed elevated live enzymes and ferritin, decreased value of ESR, albumin, complete blood count and fibrinogen in all the 24 cases. Bone marrow examination supported the diagnosis of definite hemophagocytosis in the 24 cases. Lymph node biopsy was done for one case and histopathological examination showed that the node was full of activated macrophage. As to treatment, five cases only received high dose steroids (three of them died), 14 cases were treated with high dose steroids plus cyclosporine (one died), two were treated with steroids plus cyclosporine and etoposide (none died). The causes of deaths were ARDS and CNS involvement. In three of the cases who died, treatment was given up by their parents. CONCLUSIONS: MAS is a rare and potentially fatal complication of SOJRA. Most of our patients were male. Bone marrow studies support the diagnosis. CNS involvement and ARDS were poor prognostic signs. Early diagnosis and aggressive therapy are essential.
16783831 Optimal nocturnal humidification for xerostomia. 2006 Sep BACKGROUND: Xerostomia is associated with a difficulty of maintaining oral hygeine and with symptoms of daytime oral discomfort and nocturnal sleep disturbance. METHODS: A pilot study was conducted to test the efficacy of a new method of delivering heated humidified air (37 degrees C, 100% water vapor saturated, at 20-25 L/min flow rate via specially designed nasal prongs) overnight for 2 weeks to 10 volunteers with Sjögrens Syndrome. Effectiveness was assessed by means of questionnaire, including the validated Xerostomia Inventory together with specific questions related to quality of sleep and oral comfort. RESULTS: Nine subjects completed the study. Daytime xerostomia symptoms were significantly reduced in participants (p = .004). Night-time oral comfort was significantly improved (p = .005) as was early morning mouth comfort (p = .008). Frequency of dry cough was also significantly reduced over the study period (p < .001). CONCLUSIONS: The body temperature, pressure saturated, delivery system was well tolerated and succeeded in alleviating the major oral and pharyngeal symptoms produced by xerostomia. These results have encouraged us to pursue further research in the application of this method of providing heated humidification.