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Laboratory Features (laboratory + feature)
Selected AbstractsHigh HDL-cholesterol in women with rheumatoid arthritis on low-dose glucocorticoid therapyEUROPEAN JOURNAL OF CLINICAL INVESTIGATION, Issue 9 2008C. García-Gómez ABSTRACT Background, Dyslipidaemia has been described in non-treated rheumatoid arthritis (RA), and improves after therapy with disease modifying anti-rheumatic drugs or glucocorticoids; however, it has generally been perceived that glucocorticoids adversely affect lipid metabolism. The association of low dose glucocorticoid therapy with plasma lipid levels was evaluated in female RA patients. Materials and methods, A cross-sectional study was conducted in 78 female RA patients [mean age: 60 (12) years; mean disease duration: 13 (9) years]. Sixty-five (83%) were on glucocorticoid therapy [total equivalent mean prednisone dose: 5·1 (1·7) mg d,1]. Each patient was assessed through a self-reported questionnaire, structured interview and physical examination. Blood samples were obtained for routine biochemistry, lipid profile and haematological tests. Lipid profiles of RA patients who were and were not on glucocorticoid therapy were compared. Results, Clinical and laboratory features of the two groups of patients were similar, except for the Health Assessment Questionnaire and body mass index, which were significantly higher in the patients on glucocorticoid therapy. These patients had 14·7% higher serum high-density lipoprotein cholesterol (HDL-c) levels than untreated patients (P = 0·043), mainly at the expense of HDL2 subfraction, which was 24·4% higher (P < 0·039), whereas HDL3-c was only 7·4% higher (P = 0·219). Serum levels of glucose and total cholesterol, triglyceride, low-density lipoprotein cholesterol (LDL -c), very low-density lipoprotein cholesterol, apolipoproteins A-I and B were not increased in patients on glucocorticoid therapy. Conclusions, Low dose glucocorticoid therapy in RA patients is associated with an increase in HDL-c, without increasing LDL-c or triglyceride. These lipid changes may overall be considered favourable. [source] Disseminated intravascular coagulation in acute leukemia: clinical and laboratory features at presentationEUROPEAN JOURNAL OF HAEMATOLOGY, Issue 4 2006Masamitsu Yanada Abstract:,Background:,Although there are two major scoring systems for the clinical diagnosis of disseminated intravascular coagulation (DIC), the validity of these systems for leukemia-associated DIC remains to be confirmed. Methods:,By analyzing 125 newly diagnosed acute leukemia patients, we investigated clinical and laboratory features of leukemia-associated DIC, and determined the validity of the two established criteria. Results:,A total of 36 patients (29%) were diagnosed with DIC according to expert opinion, a method regarded as the de facto gold standard. Leukemia-associated DIC is characterized by rare manifestation of organ failure because of thrombosis and no relevance of the platelet count for the diagnosis. The results of receiver operating characteristics analysis favored fibrin degradation product (FDP) rather than D-dimer as the fibrin-related marker test. Although prothrombin time, plasma fibrinogen, and serum FDP levels were significantly different for patients with and without DIC, multivariate analysis identified FDP levels to be the only factor associated with DIC diagnosis. The cut-off level of 15 ,g/mL for FDP was found to be the most effective to differentiate DIC from non-DIC, resulting in diagnostic sensitivity and specificity of 92% and 96%, respectively. The diagnostic results for our patients produced with this FDP-based system were at least comparable with or superior to those obtained with the two currently available scoring systems. Conclusions:,Our findings suggest that an FDP-based criterion may be applicable for the diagnosis of leukemia-associated DIC. Although it appears to be simple and practicable enough for clinical use, prospective validation of this criterion is needed. [source] Multiple myeloma in elderly patients: prognostic factors and outcomeEUROPEAN JOURNAL OF HAEMATOLOGY, Issue 5 2005Athanasios Anagnostopoulos Abstract:,Objectives:,Purpose of this study was to compare prognostic factors and outcome of patients with multiple myeloma (MM) aged >70 yr at diagnosis with those of younger patients. We also applied the recently proposed International Staging System (ISS) for MM in these patients. Patients and methods:,Among 1,162 newly diagnosed, symptomatic MM patients included in our database, 357 (31%) were >70 yr of age. Clinical and laboratory variables were evaluated in patients >70 yr and in younger patients and were assessed for possible correlation with survival in patients >70 yr of age. Results:,Most clinical and laboratory features were similar in the two groups of patients but older patients presented more frequently with advanced ISS (P = 0.02). Despite similar response rates to primary treatment, younger patients survived longer than patients >70 yr of age (40 vs. 28 months, P = 0.001). There was a longer survival of younger patients than that of older patients diagnosed with ISS stage 1 (median 71 vs. 54 months, P = 0.007) and ISS stage-2 patients (median: 38 vs. 26 months, P = 0.0008) but for patients with ISS stage 3 median survival was similarly poor in the younger and older age group (21 and 20 months, P = 0.283). Other variables associated with impaired prognosis were severe anemia, extensive bone marrow plasmacytosis and elevated serum LDH. Conclusions:,Older patients with MM present more often with advanced ISS and have significantly shorter survival than younger patients. The ISS retained its prognostic significance within the group of elderly patients. [source] Clinical, laboratory and therapeutic aspects of platelet-type von Willebrand diseaseINTERNATIONAL JOURNAL OF LABORATORY HEMATOLOGY, Issue 2 2008M. FRANCHINI Summary Platelet-type von Willebrand disease (PT-VWD), or pseudo-VWD, is a rare inherited platelet disorder characterized by an increased affinity of the platelet membrane glycoprotein Ib, receptor for normal von Willebrand factor leading to characteristic platelet hyperaggregability. As PT-VWD shares most of the clinical and laboratory features of subtype 2B VWD, the differential diagnosis between these two inherited bleeding disorders requires either platelet-mixing or molecular genetic studies. In this review, the main clinical, laboratory and therapeutic characteristics of PT-VWD are concisely reported. [source] Prolactin and macroprolactin in patients with systemic lupus erythematosusINTERNATIONAL JOURNAL OF RHEUMATIC DISEASES, Issue 3 2008Mohammadhassan JOKAR Abstract Aim: The aim of this study was to evaluate plasma levels of prolactin and macroprolactin in a group of systemic lupus erythematosus (SLE) patients and to determine if prolactin and macroprolactin concentrations were related to disease activity, clinical features or serological abnormalities. Methods: Ninety consecutive Iranian patients with SLE were tested for serum prolactin and macroprolactin levels. Total prolactin was measured directly in serum samples by radioimmunoassay. Free prolactin was extracted from the serum using polyethylene glycol. Clinical manifestation and SLE disease activity index (SLEDAI) were recorded. Auto antibodies were determined by standard techniques. Results: There were 90 patients (7 male, 83 female) with a mean age of 27.6 ± 9.1 (range 14,52). The mean disease duration was 27.6 ± 9.1 months. The frequency of high prolactin and macroprolactin, respectively, was 10% (9/90) and 5.6% (5/90) in patients with SLE. Macroprolactinemia was found in 55.55% (5/9) of hyperprolactinemic patients. Lupus activity was present in 63.3% (57/90) of patients without a significant difference in the frequency of high serum prolactin and macroprolactin levels when compared to inactive lupus. There were no statistically significant differences regarding demographic, clinical and laboratory characteristics between the group of patients with macroprolactinemia and the group without macroprolactinemia. Conclusion: Our results suggest that a subgroup of SLE patients have hyperprolactinemia and macroprolactinemia but they do not seem to have positive or negative correlation to clinical and laboratory features and disease activity. [source] The ZiReal Post: A New Ceramic Implant AbutmentJOURNAL OF ESTHETIC AND RESTORATIVE DENTISTRY, Issue 1 2003URS BRODBECK DMD ABSTRACT Restorations in the anterior esthetic zone present significant challenges in both the surgical and prosthetic phases of implant dentistry. Titanium has been established as the material of choice for endosseous implants, resulting in a high degree of predictability. Many types of implants require transmucosal abutments to retain implant restorations. Ceramics may be the ideal material to replace natural teeth, but most transmucosal abutments are made of titanium. However, ceramics may also be used as abutments in implant restorations. This combination of ceramics for abutment and crown provides better translucency for the implant restoration than is available with metal abutments and porcelain-fused-to-metal crowns. Ceramic abutments and implant restorations also minimize the gray color associated with metal components that is transmitted through the peri-implant tissues. Customized emergence profiles also may be obtained with ceramic abutments; this generally improves the predictability and consistency of the esthetics obtainable in implant restorations. Zirconia as a ceramic material offers not only outstanding material properties but also a well-documented biocompatibility. CLINICAL SIGNIFICANCE This article discusses the clinical and laboratory features of a new ceramic abutment, ZiRealÔ Post (Implant Innovations, Inc., Palm Beach Gardens, Florida). [source] Comparison between the clinical and laboratory features of enterovirus and West Nile virus infectionsJOURNAL OF MEDICAL VIROLOGY, Issue 7 2008Joanna Middleton Abstract The seasonality and clinical features of enterovirus (EV) infections overlap with those of West Nile virus (WNV). The purpose of this study was to determine the frequency of EV detection in patients being tested for WNV and to look for features that could be used to distinguish between infections with these two viruses. Nucleic acid amplification testing (NAT) for EV was performed on all plasma samples submitted for WNV testing in 2003 and 2004. Demographics, clinical features, and laboratory results for patients with documented EV viremia were compared with those for patients with confirmed WNV infection (as diagnosed by NAT and/or serology). NAT for EV was positive on 50 of 1,784 serum or plasma samples submitted for WNV testing (2.8%). Clinical information was compared for 45 patients with EV viremia and 214 patients with WNV infection. Patients with EV viremia were younger and less likely to have heart disease or a travel history (P,<,0.05). The EV viremia cases were distributed throughout the whole province while the WNV cases were predominantly in the southern part of the province. Symptoms were remarkably similar, although patients with WNV infection were more likely to have anorexia, dizziness, rash, and cranial nerve palsy (P,<,0.05). There are no consistent differences in the features of WNV infection and enteroviral viremia so diagnostic tests for both viruses should be performed when WNV is present in local mosquitoes. J. Med. Virol. 80: 1252,1259, 2008. © 2008 Wiley-Liss, Inc. [source] Epidemiologic aspects and laboratory features of enterovirus infections in Western Germany, 2000,2005JOURNAL OF MEDICAL VIROLOGY, Issue 7 2007Bernhard Roth Abstract From 2000 to 2005, a total of 1,096 enterovirus infections were diagnosed either by isolation of virus from cell culture or by RT-PCR (5,non-coding region (NCR)). Typing of viruses (n,=,674) was carried out by immunofluorescence with monoclonal antibodies, neutralization test or molecular methods. Seasons with high enterovirus activity were characterized by high prevalence of echovirus 30 (62.2% in 2000, 25.5% in 2001) and echovirus 13 (34.5% in 2001). In contrast, in the 2003 season, which had very low enterovirus activity, these types were rare. During this season, cell culture sensitivity (human colonic carcinoma cells and human embryonic lung fibroblasts (HEL)) was exceptionally low. In order to determine the type of "non-cultivable" enteroviruses, purified RNA from selected stool samples was subjected to direct molecular typing. VP1/2A-specific fragments were amplified by RT-PCR, cloned and sequenced. The predominant virus identified was coxsackie A. Consequently, rhabdomyosarcom cells were introduced into the daily routine, which improved the isolation of enteroviruses. Echovirus 30 was again most commonly isolated during seasons 2004 and 2005 with increasing enterovirus activity. In conclusion, high prevalence of echovirus 30 and 13 is indicative of seasons with high enterovirus activity. The type of circulating enteroviruses may influence isolation of enterovirus from cell culture. RT-PCR (VP1/2A) combined with cloning and sequencing of amplicons is a useful tool for viral typing directly from stool samples. In cases of severe enterovirus infection, virological diagnosis should not solely rely on virus isolation from cell culture. J. Med. Virol. 79:956-962, 2007. © 2007 Wiley-Liss, Inc. [source] Mucosal vulval lichen planus: outcome, clinical and laboratory featuresJOURNAL OF THE EUROPEAN ACADEMY OF DERMATOLOGY & VENEREOLOGY, Issue 4 2005G Kirtschig [source] Infectious Mononucleosis,Like Syndromes in Febrile Travelers Returning From the TropicsJOURNAL OF TRAVEL MEDICINE, Issue 4 2006Emmanuel Bottieau MD Background Infectious mononucleosis (IM), resulting from Epstein,Barr virus (EBV) infection, and IM-like syndromes, mainly due to cytomegalovirus (CMV), Toxoplasma gondii, or human immunodeficiency virus (HIV), have been occasionally reported in travelers returning from the tropics. Our objective was to investigate the prevalence, outcome, and diagnostic predictors of these syndromes in febrile travelers. Methods Between April 2000 and March 2005, all febrile travelers and migrants presenting at our referral centers within 12 months after a tropical stay were prospectively included. We identified all patients serologically diagnosed with IM or IM-like syndrome and compared them with the rest of the cohort. Results During the 5-year period, 72/1,842 patients (4%) were diagnosed with an IM-like syndrome, including 36 CMV, 16 T gondii, 15 EBV, and 5 HIV primary infections. All patients were western travelers or expatriates. Mean delay before consultation was 2 weeks. Most patients had consulted other practitioners and/or received presumptive treatment. A minority of patients presented with IM clinical features. Lymphocytosis ,40% of the white blood cells (WBC) and reactive/atypical lymphocyte morphology were observed in 60 and 30% of the patients. The four diseases were indistinguishable. Protracted fever and asthenia were common but complications rarely occurred. IM-like syndromes were independently associated with fever >7 days, lymphadenopathy, elevated liver enzymes, and lymphocytosis ,40% of WBC. Diagnostic probability increased to >20% if at least three of these predictors were present. Conclusions Diagnosis of IM and IM-like syndrome is not uncommon in febrile travelers, with a higher proportion of primary CMV, T gondii, and HIV infections than in nonimported series. Consequently, classic IM clinical and laboratory features are often lacking. All four pathogens should be systematically considered because early diagnosis should avoid unnecessary investigations and treatment and allow early intervention in case of primary HIV infection. [source] Clinical outcome and virological characteristics of hepatitis B-related acute liver failure in the United StatesJOURNAL OF VIRAL HEPATITIS, Issue 2 2005C.-T. Wai Summary., The role of hepatitis B virus (HBV) genotypes in the outcome of acute HBV infection is unclear. In this study, we aimed to evaluate the clinical and virological features of patients with hepatitis B-related acute liver failure (HBV-ALF) in the US. Clinical and laboratory features of consecutive patients with HBV-ALF from the US ALF Study Group were analysed. Prevalence of HBV genotypes, precore stop (G1896A) and core promoter dual (T1762A, A1764T) variants among patients with HBV-ALF were compared with a cohort of 530 patients with chronic HBV infection. Thirty-four HBV-ALF patients were studied: mean age 41 years, 56% men, 25 had detectable HBV-DNA. HBV genotypes A, B, C and D were found in 36, 24, 8 and 32% patients, respectively. Precore stop and core promoter dual variants were detected in 32 and 44% of patients, respectively. Twenty-three (68%) patients survived: 14 after liver transplant, nine without transplant. Older age was the only independent factor associated with poor outcome. Compared with patients with chronic HBV infection, patients with ALF were more likely to be non-Asians (88%vs 44%, P = 0.005) and to have genotype D (32%vs 10%, P < 0.01). A higher prevalence of HBV genotype D persisted even after matching for race and HBeAg status (32%vs 16%, P = 0.007). We concluded that HBV genotype D was more frequently found in patients with HBV-ALF than those with chronic HBV infection in the US. Further studies are needed to determine if HBV genotypes play a role in the outcome of acute HBV infection. [source] Occult hepatitis B virus infection in patients with autoimmune liver diseasesLIVER INTERNATIONAL, Issue 3 2009Sarah P. Georgiadou Abstract Background: Occult hepatitis B virus (HBV) infection is characterized by undetectable serum HBV surface antigen (HBsAg) but detectable HBV-DNA in serum or liver. Aims: To determine the prevalence and clinical impact of occult HBV in autoimmune liver diseases as similar data are missing. Methods: One hundred and ninety-six sera samples from HBsAg-negative patients, including 66 autoimmune hepatitis (AIH), 93 primary biliary cirrhosis (PBC) and 37 primary sclerosing cholangitis (PSC), were investigated for HBV-DNA using the polymerase chain reaction (PCR) before treatment initiation. One hundred and three serial samples from 38 AIH patients under immunosuppression and 282 selected blood donors (HBsAg negative; antibodies to HBV-core antigen positive) were also investigated. Fourteen available paraffin-embedded AIH liver samples were also investigated for HBV-DNA by nested-PCR. Results: Hepatitis B virus DNA was detected in the serum of 24/196 patients (12.2%) and 0/282 donors (P=0.0000). Nine patients had AIH (13.6%), eight had PBC (8.6%) and seven had PSC (18.9%) (P=0.0000 vs healthy). HBV-DNA detection in AIH livers was higher than in serum. HBV-DNA was associated neither with HBV markers nor with epidemiological, laboratory and clinical data. Serial testing of AIH patients revealed two HBV-DNA-negative patients before treatment becoming positive during treatment, while all HBV-DNA-positive patients before immunosuppression became negative. Conclusion: Based mainly on serum HBV-DNA, we found a significant proportion of autoimmune liver disease patients with occult HBV compared with donors. However, taking into account our results in a small number of liver tissues, it should be emphasized that occult HBV could be even higher when both serum and liver specimens are investigated. Occult HBV does not seem to affect the clinical and laboratory features of the diseases, while AIH patients with occult HBV under immunosuppression do not deteriorate during follow-up. [source] Pneumocystis jiroveci thyroiditis: report of 15 cases in the literatureMYCOSES, Issue 6 2007Alexandre P. Zavascki Summary The authors review the epidemiology, clinical manifestations, diagnosis and treatment of Pneumocystis jiroveci thyroiditis of 15 cases reported in the medical literature. Patients with acquired immunodeficiency disease syndrome were particularly at risk. P. jiroveci thyroiditis was diagnosed at autopsy as a part of disseminated infection in a substantial number of patients without clinical manifestations and laboratory evidence of thyroid dysfunction. Local signs and symptoms of infection were indistinguishable from other infectious thyroiditis and included neck enlargement with or without cervical pain, sometimes associated with dysphagia and dysphonia, and clinical and laboratory features of hypothyroidism. Antemortem diagnosis of fungal thyroiditis was made by direct microscopy and culture of a fine-needle aspirate in most cases. As most patients with P. jiroveci thyroiditis had disseminated Pneumocystis infection with a delay in diagnosis and treatment, the overall mortality was high. Pneumocystis jiroveci thyroiditis is rare but should be suspected in HIV-infected patients with CD4 count lower than 200 cells ,,1 on prophylatic inhalatory pentamidine who present with neck enlargement with or without pain, and clinical and laboratory evidence of hypothyroidism. [source] Hypertension, erythrocyturia and proteinuria in childhood non-Hodgkin's lymphomaNEPHROLOGY, Issue 3 2006WASIU A OLOWU SUMMARY: Aim: The objectives were to determine the prevalence and outcome of hypertension, significant microerythrocyturia and proteinuria among children with acute renal failure (ARF) due to Burkitt-type non-Hodgkin's lymphoma (BNHL). Methods: A retrospective analysis of clinical and laboratory data of children with BNHL/ARF was undertaken. Results: Nine of 23 (39.13%) BHNL/ARF children aged 5,14 years were found to have significant microerythrocyturia and proteinuria as urinary markers of glomerulonephritis (GN). Eight of nine were hypertensive with hypertensive encephalopathy (HTE) in three, and congestive heart failure (CCF)/pulmonary oedema in six. Three of nine patients (33.3%) died from these complications; two from CCF and one from a combination of CCF and HTE. A fourth death was due to uraemia. Treatments with cytotoxic drugs and anti-tumour lysis syndrome therapy resulted in normotension, improved clinical outcome and normalisation of laboratory features of ARF and GN in all five (55.6%) survivors. Conclusion: We conclude that all the children with BNHL/ARF had enlarged kidneys and evidence of glomerular disease. The mechanism of the glomerular disease is unclear. It is associated with a high mortality rate. [source] Carotid intima,media thickness in children and young adults with renal transplant: Internal carotid artery vs. common carotid arteryPEDIATRIC TRANSPLANTATION, Issue 8 2007Yelda Bilginer Abstract:, Cardiovascular diseases are the main causes of morbidity and mortality following renal transplantation. Atherosclerotic structural changes, which can be detected by high-resolution B-mode ultrasonography, begin before clinical findings. However, little is known about the extent of these abnormalities in children after renal transplantation. We aimed to determine early structural changes of large arteries in renal transplant recipients without cardiovascular disease and to evaluate the role of clinical and laboratory features on IMT of carotid arteries. IMT and hemoglobin, serum levels of creatinine, acute phase proteins, lipid profile, and homocysteine were examined in 24 asymptomatic renal transplant recipients (median age 16.5 yr; range 8,25), and 20 healthy controls (median age 16 yr; range 9,24). CCA and ICA were evaluated in patients and controls with a high-resolution B-mode ultrasonography in multiple projections to optimize detection of carotid IMT. Measurement of IMT of both CCA [0.36 mm (range 0.16,0.48) vs. 0.28 mm (range 0.21,0.35), p < 0.001] and ICA [0.27 mm (range 0.16,0.48) vs. 0.22 mm (range 0.1,0.26), p < 0.001] were significantly higher in renal recipients than in healthy controls. Among several parameters assessed, only significant correlations were found between duration of CRF, duration of dialysis prior to transplantation and ICA-IMT (p = 0.06 and p = 0.02, respectively) and between mean past serum calcium,phosphorus ion product and CCA-IMT (p = 0.002). In conclusion, our observations indicate that vascular changes begin early in the course of CRF and are directly related to time on CRF and dialysis. These changes can be detected by measuring CCA/ICA-IMT ultrasonographically. We suggest that early renal transplantation can potentially avoid long-term cardiovascular events in children with end stage kidney disease. [source] Original Article: Clinical management of short children with low serum immunoglobulin but no immunodeficiency featuresPEDIATRICS INTERNATIONAL, Issue 4 2010Cristina Meazza Abstract Background:, In children of different ages investigated for failure to thrive, low (below the cut-off for age) immunoglobulin (Ig) values can be detected, without any clinical evidence of humoral immunodeficiencies. To better characterize infants presenting with diminished immunoglobulin levels, we studied IgG subclasses, in vitro Ig production and B cell subpopulation. Methods:, We monitored 17 children (12 boys and five girls, age range 1,18 years) with low serum levels of one or more Ig isotypes but without any clinical or laboratory features of immunodeficiency. Results:, Low IgM levels were frequent (52.9%). During the follow up, six of 17 cases (35.3%) normalized their immunoglobulin levels. Frequently, in the observed patients, low levels of immunoglobulins were not limited to the period of infancy. In all patients, in vitro Ig production and B lymphocyte subpopulations were within normal ranges. Conclusions:, We suggest a quantification of serum Ig levels in children who fail to thrive in order to identify patients with low Ig levels. These children should be monitored until Ig levels normalize to exclude any immunodeficiency status. Early recognition of children with persistent hypogammaglobulinemia would allow prompt and appropriate clinical interventions. [source] Juvenile idiopathic arthritis profile in Turkish childrenPEDIATRICS INTERNATIONAL, Issue 2 2008Mustafa Yilmaz Abstract Background: Juvenile idiopathic arthritis (JIA) is a heterogeneous group of disorders. Publications from different countries point to differences in the disease manifestation of JIA among different populations. The aim of the present paper was to evaluate the clinical and laboratory features of JIA in Turkish children. Methods: A total of 196 JIA patients who fulfilled International League of Associations for Rheumatology (ILAR) diagnostic criteria were included in this retrospective study. The data collected were age, gender, age at disease onset and at diagnosis, and follow-up duration. Antinuclear antibody (ANA), rheumatoid factor (RF), and human leukocyte antigen B-27 were evaluated for each patient. Results: There were 102 boys and 94 girls with a mean duration of disease of 4.1 years. The mean age at the first visit was 8.8 years, and the mean age at onset of disease was 6.8 years (range, 8 months,15 years). Polyarticular JIA was the most frequent onset type (37.2%). Other subtypes included oligoarthritis (34.2%), systemic arthritis (15.3%), psoriatic arthritis (1%), enthesitis-related arthritis (9.7%), and other arthritis (2.2%). ANA was positive in 28 patients (14.2%). Chronic uveitis occurred in two patients with oligoarthritis; and two patients with enthesitis-related arthritis had acute uveitis. Three patients (1.4%) developed amyloidosis. Conclusion: Compared to reports from Western countries, remarkably different features of JIA were found in Turkish children, which included higher frequency of polyarticular JIA, higher prevalence among boys, lower rate of ANA positivity and uveitis. Further studies are required to understand how genetic and environmental differences affect JIA expression. [source] Macrophage activation syndrome in juvenile systemic lupus erythematosus: A multinational multicenter study of thirty-eight patientsARTHRITIS & RHEUMATISM, Issue 11 2009Alessandro Parodi Objective To describe the clinical and laboratory features of macrophage activation syndrome as a complication of juvenile systemic lupus erythematosus (SLE). Methods Cases of juvenile SLE,associated macrophage activation syndrome were provided by investigators belonging to 3 pediatric rheumatology networks or were found in the literature. Patients who had evidence of macrophage hemophagocytosis on bone marrow aspiration were considered to have definite macrophage activation syndrome, and those who did not have such evidence were considered to have probable macrophage activation syndrome. Clinical and laboratory findings in patients with macrophage activation syndrome were contrasted with those of 2 control groups composed of patients with active juvenile SLE without macrophage activation syndrome. The ability of each feature to discriminate macrophage activation syndrome from active disease was evaluated by calculating sensitivity, specificity, and area under the receiver operating characteristic curve. Results The study included 38 patients (20 with definite macrophage activation syndrome and 18 with probable macrophage activation syndrome). Patients with definite and probable macrophage activation syndrome were comparable with regard to all clinical and laboratory features of the syndrome, except for a greater frequency of lymphadenopathy, leukopenia, and thrombocytopenia in patients with definite macrophage activation syndrome. Overall, clinical features had better specificity than sensitivity, except for fever, which was highly sensitive but had low specificity. Among laboratory features, the best sensitivity and specificity was achieved using hyperferritinemia, followed by increased levels of lactate dehydrogenase, hypertriglyceridemia, and hypofibrinogenemia. Based on the results of statistical analysis, preliminary diagnostic guidelines for macrophage activation syndrome in juvenile SLE were developed. Conclusion Our findings indicate that the occurrence of unexplained fever and cytopenia, when associated with hyperferritinemia, in a patient with juvenile SLE should raise the suspicion of macrophage activation syndrome. We propose preliminary guidelines for this syndrome in juvenile SLE to facilitate timely diagnosis and correct classification of patients. [source] Predicting the course of juvenile dermatomyositis: Significance of early clinical and laboratory featuresARTHRITIS & RHEUMATISM, Issue 11 2008Elizabeth Stringer Objective Juvenile dermatomyositis (DM) is a rare chronic inflammatory disease of childhood. The clinical course of juvenile DM appears to be variable, and little is known about predictors of the disease course. The aims of this study were to describe the clinical course of juvenile DM and to determine whether early clinical and laboratory features can be used to predict the time to remission and/or the disease course. Methods Clinical and laboratory data from a cohort of 84 patients with juvenile DM were prospectively entered into a database (1990,2005). Remission was defined as a clinical state of no active skin rash, weakness, or elevated muscle enzyme levels for 6 months off medication. The disease course was defined as monophasic, polyphasic, or chronic. Data were reviewed at the time of diagnosis and at 3 months and 6 months after the diagnosis to determine predictors of the time to remission and/or the disease course. Results The median time to remission was 4.67 years. Sixty percent of patients had a chronic course, 37% a monophasic course, and 3% a polyphasic course. The presence of rash (most strongly indicated by Gottron's papules) at 3 months was the earliest predictor of a longer time to remission (relative risk [RR] 0.55 [95% confidence interval (95% CI) 0.37,0.81], P = 0.002). At 6 months, the presence of nailfold abnormalities and rash also predicted a longer time to remission (RR 0.35 [95% CI 0.14,0.74], P = 0.003). We were unable to determine a prediction model of disease course. Conclusion The majority of patients in our cohort had a chronic disease course. The persistence of Gottron's papules and nailfold abnormalities early in the disease course was associated with a longer time to remission. [source] Impaired lymphangiogenesis due to excess vascular endothelial growth factor-D/Flt-4 signalling in the skin of patients with systemic sclerosisBRITISH JOURNAL OF DERMATOLOGY, Issue 4 2010N. Honda Summary Background, Vascular abnormalities are one of the primary pathological components of systemic sclerosis (SSc). However, it has not been determined if there are also abnormalities in the formation of lymphatic vessels in SSc. Objective, To evaluate lymphangiogenic activity in SSc skin. Methods, The numbers of D2-40-positive lymphatic vessels in skin specimens from healthy control subjects and patients with SSc were counted and compared. Quantitative real-time polymerase chain reaction (PCR) was performed to determine mRNA levels of vascular endothelial growth factor (VEGF)-D and Flt-4 (fms-related tyrosine kinase 4, VEGFR-3, one of the receptors for VEGF-D) in the skin. Serum VEGF-D levels were measured with specific enzyme-linked immunosorbent assays. Results, The number of lymphatic vessels in patients with SSc was significantly decreased compared with healthy control subjects. Mean relative transcript levels of FIGF (VEGF-D) and FLT4 (Flt-4) in skin tissue from patients with SSc were significantly increased compared with healthy control subjects. By the analysis of the association between serum VEGF-D levels and the clinical or laboratory features, we found that patients with SSc with higher serum VEGF-D levels more frequently have skin ulcers than those with normal VEGF-D levels. Conclusions, A systemic increase of VEGF-D, as well as local overexpression of FIGF and FLT4, may be the cause of disturbed lymphangiogenesis in SSc skin and play a role in the pathogenesis of SSc. We showed the possibility that regulation of VEGF-D/Flt-4 signalling could lead to new treatment of skin ulcers in SSc by controlling the formation of lymphatic vessels. [source] Inherited and de novo von Willebrand disease ,Vicenza' in UK families with the R1205H mutation: diagnostic pitfalls and new insightsBRITISH JOURNAL OF HAEMATOLOGY, Issue 1 2006William A. Lester Summary von Willebrand disease (VWD) caused by the R1205H mutation has distinct and reproducible clinical and laboratory features. This report describes the phenotypic and molecular investigation of seven kindreds with VWD Vicenza R1205H. All affected individuals have historically been diagnosed with moderate to severe type 1 VWD. Amongst all families with highly penetrant type 1 VWD investigated at our centre, heterozygosity for the R1205H mutation was found to be the most common underlying molecular defect. A severe laboratory phenotype associated with a bleeding history that was milder than expected was commonly observed, consistent with previous published case reports; however, abnormal ultralarge high molecular weight multimers were not detected in resting plasma samples. We also provide evidence that the R1205H mutation may arise de novo, evidence that a common genetic origin for this mutation is unlikely. [source] Acute encephalitis with refractory, repetitive partial seizures (AERRPS): a peculiar form of childhood encephalitisACTA NEUROLOGICA SCANDINAVICA, Issue 4 2010H. Sakuma Sakuma H, Awaya Y, Shiomi M, Yamanouchi H, Takahashi Y, Saito Y, Sugai K, Sasaki M. Acute encephalitis with refractory, repetitive partial seizures (AERRPS): a peculiar form of childhood encephalitis. Acta Neurol Scand: 2010: 121: 251,256. © 2009 The Authors Journal compilation © 2009 Blackwell Munksgaard. Objective,,, We conducted a nationwide multicenter study in Japan to elucidate the clinical and laboratory characteristics of acute encephalitis with refractory, repetitive partial seizures (AERRPS). Materials and methods,,, Clinical and laboratory features, treatment, and outcome were assessed using a structured questionnaire. Results,,, Twenty-nine children were enrolled in the study. Refractory and repetitive partial seizures accompanied by fever were the cardinal clinical features. Partial seizures consisted principally of eye deviation or facial twitching, being periodically repeated during the acute phase. These seizures were refractory to conventional anticonvulsants and were only suppressed by high-dose intravenous barbiturate administration. Rhythmic activities on electroencephalography and non-specific cerebral atrophy on neuroimaging were common. Serum or cerebrospinal antibodies against GluR,2 were positive in six patients. General prognosis was unfavorable due to intractable epilepsy and cognitive deficits. Conclusion,,, Based on the peculiar and homogenous features, AERRPS can be regarded as a distinct clinical entity. [source] CA-125 change after chemotherapy in prediction of treatment outcome among advanced mucinous and clear cell epithelial ovarian cancers,CANCER, Issue 7 2009A Gynecologic Oncology Group study Abstract BACKGROUND: There are limited data regarding unique clinical or laboratory features associated with advanced clear cell (CC) and mucinous (MU) epithelial ovarian cancers (EOC), particularly the relationship between CA-125 antigen levels and prognosis. METHODS: A retrospective review of 7 previously reported Gynecologic Oncology Group phase 3 trials in patients with stage III/IV EOC was conducted. A variety of clinical parameters were examined, including the impact of baseline and changes in the CA-125 level after treatment of CC and MU EOC on progression-free (PFS) and overall survival (OS). RESULTS: Clinical outcomes among patients with advanced CC and MU EOC were significantly worse when compared with other cell types (median PFS, 9.7 vs 7.0 vs 16.7 months, respectively, P < .001; median OS, 19.4 vs 11.3 vs 40.5 months, respectively, P < .001). Suboptimal debulking was associated with significantly decreased PFS and OS among both. Although baseline CA-125 values were lower in CC (median, 154 ,/mL) and MU (100 ,/mL), compared with other cell types (275 ,/mL), this level did not appear to influence outcome among these 2 specific subtypes of EOC. However, an elevated level of CA-125 at the end of chemotherapy was significantly associated with decreased PFS and OS (P < .01 for all). CONCLUSIONS: Surgical debulking status is the most important variable at prechemotherapy predictive of prognosis among advanced CC and MU EOC patients. Changes in the CA-125 levels at the end treatment as compared with baseline can serve as valid indicators of PFS and OS, and likely the degree of inherent chemosensitivity. Cancer 2009. © 2009 American Cancer Society. [source] Paraneoplastic neurological syndromes , patients' cohort profile in the Czech RepublicACTA NEUROLOGICA SCANDINAVICA, Issue 2 2001toura Reported paraneoplastic neurological syndromes (PNS) are rare disabling neurological diseases with supposed autoimmune pathogenesis. The aims of this study were to evaluate frequency, clinical course and therapeutic response in the cohort of PNS positive patients (n=10) in the Czech Republic for the first time. Second, we determined the presence and distribution of oligoclonal IgG bands (OB IgG) in PNS and compared the clinical and laboratory features of OB IgG positive and negative patients. A total of 2355 suspicious serum and/or CSF samples were screened by immunofluorescence and immunohistochemistry with definite confirmation by Western blot. OB IgG were detected by isoelectric focusing and immunoenzymatic staining and clinical status was scored according to modified Rankin scale (RS). Four patients had anti-Yo antibody, ovarian cancer and the score in range (2,5) on RS. Five patients had anti-Hu antibody, small cell lung cancer (SCLC), prostate cancer and the score between 1,4 grade on RS. One patient with SCLC and anti-Ri antibody had grade 2. Five of 10 patients with PNS had positive OB IgG and average value 4.2 on RS comparing with negative OB IgG patients with average value 2.6. Finally, we add well-defined cohort of PNS patients to emerging European profile of PNS and conclude that the presence of OB IgG in PNS seems to reflect enhanced immune response with more severe neurological damage and clinical course. [source] Clinical characteristics of patients with non-specific and non-categorized mitochondrial diseasesACTA PAEDIATRICA, Issue 11 2009Jeong Tae Kim Abstract Aim:, Mitochondrial disease is a heterogeneous disorder entity induced by defects in mitochondrial respiratory chain complex (MRC). A significant portion of patients with MRC defect will not conform to a specific, known syndrome. We have analysed the clinical features of 108 Korean paediatric patients with non-specific and non-categorized mitochondrial disease. Methods:, We retrospectively reviewed the clinical and laboratory features of 108 paediatrics patients with non-specific and non-categorized mitochondrial diseases who showed defects in MRC activity, confirmed by spectrophotometric biochemical enzyme assay of their muscles. Results:, Neuromuscular involvement was noted in all patients, with developmental delay and seizure accounting for 92.6% and 77.8% of total patients respectively. Various extraneurological symptoms were observed. Most patients exhibited MRC I defect, accounting for 100 (92.6%) patients. The most common brain magnetic resonance imaging (MRI) finding was diffuse cerebral atrophy. However, in 23.1% of patients, no notable changes were visible on MRI. Conclusions:, Mitochondrial respiratory chain complex I defect was the most common finding in this study. Though neuromuscular symptoms predominated, with presence of numerous extraneurological findings, we could not find any novel symptoms that might be unique to this category of mitochondrial disease. But, comparatively, more patients presented with unremarkable birth histories and normal brain MRI findings. [source] | |||||||||||||||||||||||||