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Sample records for seropositivity clinical disease

  1. Increased inflammatory response in cytomegalovirus seropositive patients with Alzheimer's disease.

    Gabriel Westman

    Full Text Available Alzheimer's disease (AD has been associated with increased local inflammation in the affected brain regions, and in some studies also with elevated levels of proinflammatory cytokines in peripheral blood. Cytomegalovirus (CMV is known to promote a more effector-oriented phenotype in the T-cell compartment, increasing with age. The aim of this study was to investigate the inflammatory response of peripheral blood mononuclear cells (PBMCs from AD patients and non-demented (ND controls. Using a multiplex Luminex xMAP assay targeting GM-CSF, IFN-γ, IL-1β, IL-2, IL-4, IL-5, IL-6, IL-8, IP-10 and TNF-α, cytokine profiles from PBMCs were analysed after stimulation with anti-CD3/CD28 beads, CMV pp65 peptide mix or amyloid β (Aβ protofibrils, respectively. CMV seropositive AD subjects presented with higher IFN-γ levels after anti-CD3/CD28 and CMV pp65 but not after Aβ stimulation, compared to CMV seropositive ND controls. When analysing IFN-γ response to anti-CD3/CD28 stimulation on a subgroup level, CMV seropositive AD subjects presented with higher levels compared to both CMV seronegative AD and CMV seropositive ND subjects. Taken together, our data from patients with clinically manifest AD suggest a possible role of CMV as an inflammatory promoter in AD immunology. Further studies of AD patients at earlier stages of disease, could provide better insight into the pathophysiology.

  2. Disease flare of ankylosing spondylitis presenting as reactive arthritis with seropositivity: a case report

    Manoj EM

    2012-02-01

    Full Text Available Abstract Introduction Concurrent rheumatoid factor seropositivity is occasionally detected in ankylosing spondylitis and often causes confusion in clinical routine. Overlap between various seronegative arthritides is a known but uncommon association. Differentiation of spondyloarthropathy from rheumatoid arthritis is important, since the natural history, complications, treatments and prognosis of the two diseases differ significantly. Case presentation Here, we report the case of a 47-year-old Sri Lankan man who had a long history of intermittent joint pains worsening following a recent episode of self-resolving non-bloody diarrhea. Subsequently, he developed a skin rash suggestive of keratoderma blenorrhagica and circinate balanitis. He had classical radiological evidence of ankylosing spondylosis (previously undiagnosed associated with human leukocyte antigen B27 antigen, but was positive for rheumatoid factor. Conclusions A disease flare of ankylosing spondylitis prompted by a minor diarrheal illness showing well documented features of reactive arthritis is remarkable. The prognostic implications of seropositivity in spondyloarthritis are discussed.

  3. Omega 3 Fatty Acids Supplementation and Oxidative Stress in HIV-Seropositive Patients. A Clinical Trial.

    Norma Amador-Licona

    Full Text Available HIV-seropositive patients show high incidence of coronary heart disease and oxidative stress has been described as relevant key in atherosclerosis development. The aim of this study was to assess the effect of omega 3 fatty acids on different markers of oxidative stress in HIV-seropositive patients. We performed a randomized parallel controlled clinical trial in The Instituto Mexicano del Seguro Social, a public health hospital. 70 HIV-seropositive patients aged 20 to 55 on clinical score A1, A2, B1 or B2 receiving highly active antiretroviral therapy (HAART were studied. They were randomly assigned to receive omega 3 fatty acids 2.4 g (Zonelabs, Marblehead MA or placebo for 6 months. At baseline and at the end of the study, anthropometric measurements, lipid profile, glucose and stress oxidative levels [nitric oxide catabolites, lipoperoxides (malondialdehyde plus 4-hydroxialkenals, and glutathione] were evaluated. Principal HAART therapy was EFV/TDF/FTC (55% and AZT/3TC/EFV (15% without difference between groups. Treatment with omega 3 fatty acids as compared with placebo decreased triglycerides (-0.32 vs. 0.54 mmol/L; p = 0.04, but oxidative stress markers were not different between groups.

  4. Comparison of seronegative and seropositive rheumatoid arthritis with regard to some clinical characteristics.

    Sahatçiu-Meka, Vjollca; Izairi, Remzi; Rexhepi, Sylejman; Manxhuka-Kerliu, Suzana

    2009-01-01

    The aim of this study is to establish a scientific comparative analysis between seronegative and seropositive rheumatoid arthritis (RA), with regard to some clinical characteristics. The studied group consisted of RA seronegative patients with titters lower then 1:64 defined by Rose-Waaler test, while the control group consisted of RA seropositive patients with titters of 1:64 or higher. Examinees all belonged to the 2nd and 3rd functional classes according to ARA criteria, were between 25-60 years of age (Xb = 49.96), with disease duration between 1-27 years (Xbox = 6.41). In the disease onset most frequently affected joints were metacarpophalangeal (MCP) and proximal interphalangeal (PIP) joint of the hands, almost equally represented with regard to sero-status and sex. During the examination seropositive patients showed a higher presence of inflamation of peripheral joints of hand and foot, but only the presence of PIP of the hands was statistically significant (chi2 = 15.63, p < 0.01). Knees, talocrural joints and elbows were more frequently affected in seropositive patients, whereas humeroscapular, coxofemoral and sacroiliacal joints were more frequently affected in seronegative patients, but without significant statistical difference with regard to sero-status. The presence of affected PIP of the hands (chi2 = 9.96, p < 0.01) and knees (chi2 = 4.17, p < 0.05) with regard to sex was statistically significant in seropositive female patients, as well as the presence of atacked PIP of the hands (chi2 = 6.08, p < 0.05), and cervical vertebrae (chi2 = 6.00, p < 0.05) in seropositive male patients. There were some differences between groups with regard to sex in metatarsophalangealjoints (MTP), PIP of the foot, and other joints, but without any statistical significance. In both subsets statistically significant domination was found in affected second (chi2 = 20.85, p < 0.01) and third (chi2 = 15.70, p < 0.01) fingers of the PIP level of hands and third finger (chi2

  5. Clinical presentation and opportunistic infections in HIV-1, HIV-2 and HIV-1/2 dual seropositive patients in Guinea-Bissau

    Sørensen, Allan; Jespersen, Sanne; Katzenstein, Terese L

    2016-01-01

    HIV-2 is prevalent. In this study, we aimed to characterize the clinical presentations among HIV-1, HIV-2 and HIV-1/2 dual seropositive patients. Methods: In a cross-sectional study, newly diagnosed HIV patients attending the HIV outpatient clinic at Hospital Nacional Sim~ao Mendes in Guinea......-Bissau were enrolled. Demographical and clinical data were collected and compared between HIV-1, HIV-2 and HIV-1/2 dual seropositive patients. Results: A total of 169 patients (76% HIV-1, 17% HIV-2 and 6% HIV 1/2) were included in the study between 21 March 2012 and 14 December 2012. HIV-1 seropositive...... antigen. Conclusion: HIV-1 and HIV-1/2 seropositive patients have lower CD4 cell counts than HIV-2 seropositive patients when diagnosed with HIV with only minor clinical and demographic differences among groups. Few patients were diagnosed with TB and cryptococcal disease was not found to be a major...

  6. Neuromyelitis optica spectrum disorders: comparison of clinical and magnetic resonance imaging characteristics of AQP4-IgG versus MOG-IgG seropositive cases in the Netherlands.

    van Pelt, E D; Wong, Y Y M; Ketelslegers, I A; Hamann, D; Hintzen, R Q

    2016-03-01

    Neuromyelitis optica spectrum disorders (NMOSDs) are a group of rare inflammatory demyelinating disorders of the central nervous system. The identification of specific antibodies directed to aquaporin 4 (AQP4-IgG) led to the distinction from multiple sclerosis. However, up to 25% of the clinically diagnosed NMO patients are seronegative for AQP4-IgG. A subgroup of these patients might be identified by antibodies directed to myelin oligodendrocyte glycoprotein (MOG-IgG). Our objective was to investigate whether the clinical characteristics of these patients differ. Using a cell-based assay, samples of 61 AQP4-IgG seronegative patients and 41 AQP4-IgG seropositive patients with clinically NMOSD were analysed for the presence of MOG-IgG. Clinical characteristics of the AQP4-IgG, MOG-IgG seropositive and double seronegative NMOSD patients were compared. Twenty of the 61 AQP4-IgG seronegative patients tested MOG-IgG seropositive (33%). MOG-IgG seropositive patients were more frequently males in contrast to AQP4-IgG seropositive patients (55% vs. 15%, P < 0.01) and Caucasians (90% vs. 63%, P = 0.03). They more frequently presented with coincident optic neuritis and transverse myelitis (40% vs. 12%, P = 0.02) and had a monophasic disease course (70% vs. 29%, P < 0.01). AQP4-IgG seropositive patients were 2.4 times more likely to suffer from relapses compared with MOG-IgG seropositive patients (relative risk 2.4, 95% confidence interval 1.2-4.7). AQP4-IgG seropositive patients had higher Expanded Disability Status Scale levels at last follow-up (P < 0.01). Antibodies directed to MOG identify a subgroup of AQP4-IgG seronegative NMO patients with generally a favourable monophasic disease course. © 2015 EAN.

  7. Clinical presentation and aetiologies of acute or complicated headache among HIV-seropositive patients in a Ugandan clinic.

    Katwere, Michael; Kambugu, Andrew; Piloya, Theresa; Wong, Matthew; Hendel-Paterson, Brett; Sande, Merle A; Ronald, Allan; Katabira, Elly; Were, Edward M; Menten, Joris; Colebunders, Robert

    2009-09-19

    We set out to define the relative prevalence and common presentations of the various aetiologies of headache within an ambulant HIV-seropositive adult population in Kampala, Uganda. We conducted a prospective study of adult HIV-1-seropositive ambulatory patients consecutively presenting with new onset headaches. Patients were classified as focal-febrile, focal-afebrile, non-focal-febrile or non-focal-afebrile, depending on presence or absence of fever and localizing neurological signs. Further management followed along a pre-defined diagnostic algorithm to an endpoint of a diagnosis. We assessed outcomes during four months of follow up. One hundred and eighty patients were enrolled (72% women). Most subjects presented at WHO clinical stages III and IV of HIV disease, with a median Karnofsky performance rating of 70% (IQR 60-80).The most common diagnoses were cryptococcal meningitis (28%, n = 50) and bacterial sinusitis (31%, n = 56). Less frequent diagnoses included cerebral toxoplasmosis (4%, n = 7), and tuberculous meningitis (4%, n = 7). Thirty-two (18%) had other diagnoses (malaria, bacteraemia, etc.). No aetiology could be elucidated in 28 persons (15%). Overall mortality was 13.3% (24 of 180) after four months of follow up. Those without an established headache aetiology had good clinical outcomes, with only one death (4% mortality), and 86% were ambulatory at four months. In an African HIV-infected ambulatory population presenting with new onset headache, aetiology was found in at least 70%. Cryptococcal meningitis and sinusitis accounted for more than half of the cases.

  8. Vaccine Induced Antibody Response to Foot and Mouth Disease in Infectious Bovine Rhinotracheitis Seropositive Cattle

    Murat Şevik

    2014-01-01

    Full Text Available Foot and mouth disease (FMD and infectious bovine rhinotracheitis (IBR are two important infectious diseases of cattle. Inactivated FMD vaccines are the most powerful tools to protect animals against FMD. Previous studies showed that recombinant IBR-FMD viruses protected cattle from virulent BHV-1 challenge and induced protective levels of anti-FMDV antibodies. FMD is considered to be endemic in Turkey and inactivated oil adjuvanted vaccines are used for the immunization of cattle. Previous studies showed that seroprevalence of IBR in the Turkey’s dairy herd more than 50%. In this study, antibody response in IBR seropositive cattle following vaccination against FMD was investigated. IBR seropositive (n=208 and IBR seronegative (n=212 cattle were vaccinated with oil-adjuvanted bivalent vaccine (containing O1 Manisa, A22 Iraq FMDV strains. Solid-phase competitive ELISA (SPCE was used to measure antibodies produced in cattle. Protective level of antibody against serotype O was detected in 77.4% and serotypes A in 83.6% of IBR seropositive cattle. Protective level of antibody against serotype O antibody was detected in 49% and serotypes A in 66.9% of IBR seronegative cattle. The differences between the protection rates against both serotype O (P=0.0001 and serotype A (P=0.0001 in IBR seropositive and seronegative animals were statistically important (Fisher’s exact test, P<0.01. Results showed that after FMD vaccination, IBR seropositive animals produced high titres of antibodies than seronegative animals.

  9. Latiglutenase Improves Symptoms in Seropositive Celiac Disease Patients While on a Gluten-Free Diet.

    Syage, Jack A; Murray, Joseph A; Green, Peter H R; Khosla, Chaitan

    2017-09-01

    Celiac disease (CD) is a widespread condition triggered by dietary gluten and treated with a lifelong gluten-free diet (GFD); however, inadvertent exposure to gluten can result in episodic symptoms. A previous trial of latiglutenase (clinicaltrials.gov; NCT01917630), an orally administered mixture of two recombinant gluten-specific proteases, was undertaken in symptomatic subjects with persistent injury. The primary endpoint for histologic improvement was not met, presumably due to a trial effect. In this post hoc analysis, we investigated the efficacy of latiglutenase for reducing symptoms in subgroups of the study participants based on their seropositivity. The study involved symptomatic CD patients following a GFD for at least one year prior to randomization. Patients were treated for 12 weeks with latiglutenase or placebo. Of 398 completed patients, 173 (43%) were seropositive at baseline. Symptoms were recorded daily, and weekly symptom scores were compiled. p values were calculated by analysis of covariance. A statistically significant, dose-dependent reduction was detected in the severity and frequency of symptoms in seropositive but not seronegative patients. The severity of abdominal pain and bloating was reduced by 58 and 44%, respectively, in the cohort receiving the highest latiglutenase dose (900 mg, n = 14) relative to placebo (n = 54). Symptom improvement increased from week 6 to week 12. There was also a trend toward greater symptom improvement with greater baseline symptom severity. Seropositive CD patients show symptomatic improvement from latiglutenase taken with meals and would benefit from the availability of this treatment.

  10. Survival time and effect of selected predictor variables on survival in owned pet cats seropositive for feline immunodeficiency and leukemia virus attending a referral clinic in northern Italy.

    Spada, Eva; Perego, Roberta; Sgamma, Elena Assunta; Proverbio, Daniela

    2018-02-01

    Feline immunodeficiency virus (FIV) and feline leukemia virus (FeLV) are among the most important feline infectious diseases worldwide. This retrospective study investigated survival times and effects of selected predictor factors on survival time in a population of owned pet cats in Northern Italy testing positive for the presence of FIV antibodies and FeLV antigen. One hundred and three retrovirus-seropositive cats, 53 FIV-seropositive cats, 40 FeLV-seropositive cats, and 10 FIV+FeLV-seropositive cats were included in the study. A population of 103 retrovirus-seronegative age and sex-matched cats was selected. Survival time was calculated and compared between retrovirus-seronegative, FIV, FeLV and FIV+FeLV-seropositive cats using Kaplan-Meier survival analysis. Cox proportional-hazards regression analysis was used to study the effect of selected predictor factors (male gender, peripheral blood cytopenia as reduced red blood cells - RBC- count, leukopenia, neutropenia and lymphopenia, hypercreatininemia and reduced albumin to globulin ratio) on survival time in retrovirus-seropositive populations. Median survival times for seronegative cats, FIV, FeLV and FIV+FeLV-seropositive cats were 3960, 2040, 714 and 77days, respectively. Compared to retrovirus-seronegative cats median survival time was significantly lower (P<0.000) in FeLV and FIV+FeLV-seropositive cats. Median survival time in FeLV and FIV+FeLV-seropositive cats was also significant lower (P<0.000) when compared to FIV-seropositive cats. Hazard ratio of death in FeLV and FIV+FeLV-seropositive cats being respectively 3.4 and 7.4 times higher, in comparison to seronegative cats and 2.3 and 4.8 times higher in FeLV and FIV+FeLV-seropositive cats as compared to FIV-seropositive cats. A Cox proportional-hazards regression analysis showed that FIV and FeLV-seropositive cats with reduced RBC counts at time of diagnosis of seropositivity had significantly shorter survival times when compared to FIV and FeLV-seropositive

  11. Cytomegalovirus disease in lung transplantation: impact of recipient seropositivity and duration of antiviral prophylaxis.

    Hammond, S P; Martin, S T; Roberts, K; Gabardi, S; Fuhlbrigge, A L; Camp, P C; Goldberg, H J; Marty, F M; Baden, L R

    2013-04-01

    A recent randomized trial demonstrated that 1 year of antiviral prophylaxis for cytomegalovirus (CMV) after lung transplantation is superior to 3 months of treatment for prevention of CMV disease. However, it is uncertain if a shorter duration of prophylaxis might result in a similar rate of CMV disease among select lung transplant (LT) recipients who are at lower risk for CMV disease, based on baseline donor (D) and recipient (R) CMV serologies. We retrospectively assessed incidence, cumulative probability, and predictors of CMV disease and viremia in LT recipients transplanted between July 2004 and December 2009 at our center, where antiviral CMV prophylaxis for 6-12 months is standard. Of 129 LT recipients, 94 were at risk for CMV infection based on donor CMV seropositivity (D+) or recipient seropositivity (R+); 14 developed CMV disease (14.9%): 11 with CMV syndrome, 2 with pneumonitis, and 1 with gastrointestinal disease by the end of follow-up (October 2010); 17 developed asymptomatic CMV viremia (18.1%). The cumulative probability of CMV disease was 17.4% 18 months after transplantation. CMV D+/R- recipients who routinely received 1 year of prophylaxis were more likely to develop CMV disease compared with D+/R+ or D-/R+ recipients, who routinely received 6 months of prophylaxis (12/45 vs. 2/25 vs. 0/24, P = 0.005). Recipients who stopped CMV prophylaxis before 12 months (in D+/R- recipients) and 6 months (in R+ recipients) tended to develop CMV disease more than those who did not (9/39 vs. 3/41, P = 0.06). On a 6-month CMV prophylaxis protocol, few R+ recipients developed CMV disease in this cohort. In contrast, despite a 12-month prophylaxis protocol, D+/R- LT recipients remained at highest risk for CMV disease. © 2012 John Wiley & Sons A/S.

  12. Clinical Correlates of Diarrhea and Gut Parasites among Human Immunodeficiency Virus Seropositive Patients

    Elvis Bisong

    2017-09-01

    Full Text Available Cluster differentiation 4 (CD4 count estimation, which is not readily available in most resource poor settings in Nigeria, is an important indexdetermining commencement of antiretroviral therapy (ART. It is imperative for physicians who come in contact with these patients in such settings to recognize other parameters to evaluate these patients. The clinical correlates of diarrhea and gut parasites among human immunodeficiency virus (HIV-seropositive patients attending our special treatment clinic were studied. Three hundred and forty consenting HIV-positive adult subjects were enrolled. Their stool and blood specimens were collected for a period of three months. Stool samples were analyzed for the presence of diarrhea and gut parasites. The patients were clinically evaluated by physical examination for the presence of pallor, dehydration, oral thrush, wasting lymphadenopathy, dermatitis, skin hyperpigmentation, and finger clubbing. Participants with diarrhea represented 14.1% of the population, while 21.5% harbored one or more parasites. In the subjects with diarrhea, 14.6% harbored gut parasites. The presence of diarrhea was associated with a low CD4 count. Clinically, oral thrush, wasting, and rashes were more reliable predictors of low CD4 count levels; whereas, the presence of pallor, dehydration, wasting, and rashes correlated with the presence of diarrhea. HIV patients presenting with pallor, dehydration, wasting, and rashes should be evaluated for the presence of diarrhea. The clinical variables associated with low CD4 count in this study may guide commencing antiretroviral therapy in resource poor settings.

  13. Familial aggregation of arthritis-related diseases in seropositive and seronegative rheumatoid arthritis: a register-based case-control study in Sweden.

    Frisell, Thomas; Hellgren, Karin; Alfredsson, Lars; Raychaudhuri, Soumya; Klareskog, Lars; Askling, Johan

    2016-01-01

    Our objective was to estimate the risk of developing rheumatoid arthritis (RA) associated with a family history of non-RA arthritis-related diseases. This familial co-aggregation is of clinical interest since it is often encountered when assessing family history of RA specifically, but also informative on the genetic overlap between these diseases. Since anticitrullinated peptide antibodies/rheumatoid factor (RF)-positive and RF-negative RA have both specific and shared genetic factors, the familial co-aggregation was assessed separately for seropositive and seronegative disease. Nested case-control study in prospectively recorded Swedish total population data. The Multi-Generation Register identified first-degree relatives. RA and arthritis-related diseases were ascertained through the nationwide patient register. RA serology was based on International Classification of Diseases tenth revision coded diagnoses, mainly reflecting RF. Familial risks were calculated using conditional logistic regression. Results were replicated using the Swedish rheumatology register. Familial co-aggregation was found between RA and every studied arthritis-related disease, but the magnitude varied widely, from juvenile idiopathic arthritis (JIA) (seropositive RA OR=3.98 (3.01 to 5.26); seronegative RA OR=5.70 (3.47 to 9.36)) to osteoarthritis (seropositive RA OR=1.03 (1.00 to 1.06); seronegative RA OR=1.05 (1.00 to 1.09)). The familial co-aggregation pattern of non-RA arthritis-related diseases was overall similar for seropositive and seronegative RA. Among those with family history of RA, relatives' other arthritis-related diseases conferred little or no additional risk. Although family history of several arthritis-related diseases may be useful to predict RA (eg, lupus and JIA), others (eg, osteoarthritis and arthralgia) are less useful. Seropositive and seronegative RA had rather similar familial co-aggregation patterns with arthritis-related diseases, suggesting that the two RA

  14. Chronic genital ulcer disease with subsequent development of methicillin-resistant Staphylococcus aureus (MRSA urethritis and bacteraemia in an HIV-seropositive person – a case observation

    Christine Katusiime

    2012-03-01

    Full Text Available HIV-seropositive persons are at increased risk of methicillin-resistant Staphylococcus aureus (MRSA. Genital ulcerative disease and sexually transmitted infection with subsequent MRSA infection in HIV-seropositive persons have been documented only once. We report a case of a 44-year-old man who presented to the Infectious Diseases Institute, Kampala, Uganda, with chronic genital ulcer disease and who subsequently developed MRSA urethritis and bacteraemia. This case also demonstrates that persistent genital ulcer disease in HIV-seropositive persons may be as a result of concurrent MRSA infection.

  15. Relationships between certain metabolic diseases and selected serum biochemical parameters in seropositive dairy cows against Neospora caninum infection in different stages of lactation

    Alekish, Myassar O.; Talafha, Abdelsalam Q; Alshehabat, Musa A; Ismail, Zuhair A Bani

    Neospora caninum is an important cause of abortion in dairy cattle. The general health of affected cows has not been investigated before. Therefore, the main objective of this study was to identify possible relationships between certain metabolic diseases and selected serum biochemical parameters in seropositive dairy cows against N. caninum antibodies in different stages of lactation. The study was carried out using 72 N. caninum seropositive cows and 61 seronegative dairy cows (control). Serum from all cows was tested to determine their N. caninum status (seropositive vs seronegative) using commercially available indirect enzyme-linked immunosorbent assay test kit (iELISA). In addition, serum biochemical parameters including beta-hydroxybutyrate (BHB), glucose, creatinine, blood urea nitrogen, total protein, albumin, alkaline phosphatase (ALP), aspartate aminotransferase (AST), alanine transaminase (ALT), lactate dehydrogenase (LDH) and gamma-glutamyltranspeptidase (GGT) were determined using routine laboratory methods. The stage of lactation was obtained at the time of sampling from farm records. Student independent t-test showed that there was a significant difference in the serum concentrations of BHB, AST, ALT, and LDH between seropositive and seronegative cows. There was no significant association between seropositivity and the stage of lactation. However, multivariable logistic regression analysis showed that there was a strong association between seropositivity and BHB concentrations. Results of this study indicate a possible relationship between N. caninum seropositivity and certain metabolic diseases such as ketosis and fatty liver syndrome in dairy cows.

  16. Assessing Nutrient Intake and Nutrient Status of HIV Seropositive Patients Attending Clinic at Chulaimbo Sub-District Hospital, Kenya

    Agatha Christine Onyango

    2012-01-01

    Full Text Available Background. Nutritional status is an important determinant of HIV outcomes. Objective. To assess the nutrient intake and nutrient status of HIV seropositive patients attending an AIDS outpatient clinic, to improve the nutritional management of HIV-infected patients. Design. Prospective cohort study. Setting. Comprehensive care clinic in Chulaimbo Sub-District Hospital, Kenya. Subjects. 497 HIV sero-positive adults attending the clinic. Main Outcome Measures. Evaluation of nutrient intake using 24-hour recall, food frequency checklist, and nutrient status using biochemical assessment indicators (haemoglobin, creatinine, serum glutamate pyruvate (SGPT and mean corpuscular volume (MCV. Results. Among the 497 patients recruited (M : F sex ratio: 1.4, mean age: 39 years ± 10.5 y, Generally there was inadequate nutrient intake reported among the HIV patients, except iron (10.49 ± 3.49 mg. All the biochemical assessment indicators were within normal range except for haemoglobin 11.2 g/dL (11.4 ± 2.60 male and 11.2 ± 4.25 female. Conclusions. Given its high frequency, malnutrition should be prevented, detected, monitored, and treated from the early stages of HIV infection among patients attending AIDS clinics in order to improve survival and quality of life.

  17. A study of HIV seropositivity with various clinical manifestation of herpes zoster among patients from Karnataka, India.

    Naveen, Kikkeri Narayanashetty; Tophakane, R S; Hanumanthayya, Keloji; Pv, Bhagawat; Pai, Varadraj V

    2011-12-15

    To study the various clinical presentations of herpes zoster and to find out the proportion of HIV positivity in these patients. A time bound study was conducted from November 2004 to October 2005. All clinically diagnosed cases of herpes zoster were tested for HIV infection with ELISA and confirmed by Tridot and Coomb AID. Total numbers of 90 zoster cases were recorded. Mean duration of pre herpetic neuralgia was 2.134 (standard deviation=1.424, F=8.951, Psacral (6.66%) nerves. A substantial proportion, 34 (37.77%) out of 90 cases, were found to be HIV positive. Of these, 64.7 percent of the HIV seropositive herpes zoster patients belonged to the age group of 21-40 years. Out of 39 who had a risk of exposure to STDs and whose ages were less than 50 years, 31 (79.48%) tested positive for HIV infection. The occurrence of zoster in the young age group in patients who report a history of risk factors for HIV, may need testing. Herpes zoster serves as a clinical indicator of HIV seropositivity and one of the earliest manifestations.

  18. CLINICAL AND LABORATORY PROFILE OF SPUTUM POSITIVE PULMONARY TUBERCULOSIS AMONG HIV SEROPOSITIVE AND HIV SERONEGATIVE PATIENTS- A CROSS-SECTIONAL STUDY

    Amit Govind Kamat

    2017-05-01

    Full Text Available BACKGROUND The global impact of the converging dual epidemics of TB and HIV is one of the major public health challenges. The increasing rate of HIV infection in many countries has had an impact on TB epidemiology. As the prevalence of pulmonary tuberculosis is increasing among HIV seropositive patients with a wide range of immune status and clinical presentations, the present study was undertaken to assess the clinical and laboratory profile of sputum positive pulmonary tuberculosis among HIV seropositive and HIV seronegative patients. MATERIALS AND METHODS The present one year cross-sectional study was conducted in the Department of Medicine, KLES Dr. Prabhakar Kore Hospital and Medical Research Centre, Belgaum on 104 patients with sputum positive pulmonary tuberculosis patients during the period of January 2009 to December 2009. Routine investigations such as blood group, haemogram that is haemoglobin, total count, differential count, erythrocyte sedimentation rate, sputum smears for AFB and chest x-ray were done. RESULTS Seroprevalence of HIV among pulmonary tuberculosis patients was 23.08%. On examination anaemia, undernourishment, lymphadenopathy and the presence of opportunistic infections like oral candidiasis, herpes zoster stain and genital lesions were more predominant among HIV seropositives compared to HIV seronegatives. Mean Hb and TLC were significantly low among HIV seropositives compared to HIV seronegatives. Chest x-ray showed varied presentation. Upper zone infiltration, cavitation and fibrosis were more commonly involved among HIV seronegatives compared to HIV seropositives. CONCLUSION HIV seropositive PTB patients commonly present with fever, weight loss and loss of appetite, while cough with expectoration, haemoptysis, breathlessness were more common with HIV seronegative patients. Cavitation, fibrosis and fibrocavitary lesions were predominantly seen among HIV seronegatives, while infiltration and miliary mottling was

  19. Retrospective analysis of the clinical behavior of oral hairy leukoplakia in 215 HIV-seropositive patients

    Daniela Assis do VALE

    Full Text Available Abstract Oral manifestations are common findings in human immunodeficiency virus (HIV infected patients and frequently influence the overall health. Oral hairy leukoplakia (OHL is strongly associated with HIV infection demonstrating its relationship with the individual’s immune status and progression of immunosuppression. This study aims to retrospectively evaluate OHL in HIV patients, analyzing its incidence, demographic aspects and possible changes in clinical and epidemiological profile of the disease over 17 years. The records of 1600 HIV-infected patients were reviewed. The data were correlated and analyzed, considering HIV exposure category, age, gender, harmful habits, CD4 level, use and type of antiretroviral. OHL was observed in 215 (13.4% patients. Most were men in the fourth decade of life, 171 (79.5% and 112 (52,1% respectively, but an increase in the incidence of OHL among female patients and those in the fifth decade of life was observed. Tobacco smoking was the most frequent harmful habit reported by 114 (68% patients. OHL occurred mostly in patients with CD4 counts between 200 and 500 cells/mm3 35 (55.5%. The lower incidence of OHL was found among patients using at least one non-nucleoside reverse transcriptase inhibitor (NNRTI. OHL is related to CD4 count, use of ARVT and tobacco smoking and is also more prevalent in men in the fourth decade of life. These characteristics were recognized in absolute values, but when verifying the behavior over the years we noticed that the incidence of OHL is decreasing and its epidemiological characteristics changing.

  20. Seronegative and seropositive autoimmune autonomic ganglionopathy (AAG): Same clinical picture, same response to immunotherapy.

    Tijero, Beatriz; Del Pino, Rocio; Pérez-Concha, Tomás; Acera, Maria Angeles; Gabilondo, Iñigo; Berganzo, Koldo; Graus, Frances; Martinez-Alday, Jesus Daniel; Barcena, Joseba; Gómez-Esteban, Juan Carlos

    2018-06-15

    Two patients with a syndrome of pandisautonomia with clinical criteria of AAG are provided. Both patients present a similar clinical picture and response to immunosuppressive treatment. One of them has positive antibodies against the ganglionic nicotinic acetylcholine (gAChr) and the other does not. This brief article serves to reflect the spectrum of AAG, at a clinical level, in laboratory tests and in the response to immunotherapy, independently of the presence of positive gAChr antibodies. Copyright © 2018 Elsevier B.V. All rights reserved.

  1. Course and prognosis in seropositive and seronegative rheumatoid arthritis.

    Sahatçiu-Meka, Vjollca; Rexhepi, Sylejman; Kukeli, Anton; Manxhuka-Kërliu, Suzana; Pallaskas, Kelmend; Murtezani, Ardiana; Rexhepi, Mjellma; Rexhepi, Blerta

    2013-01-01

    Long since it have been suggested that a subpopulation of patients with rheumatoid arthritis (RA), diagnosed with negative rheumatoid factor (RF) tests, represents a clinical entity quite distinct from that of seropositive rheumatoid arthritis. The aim of the study was to establish a scientific comparative analysis between RA seronegative and seropositive, regarding course and prognoses of the disease. Two hundred fifty patients with rheumatoid arthritis according to the (American College of Rheumatology) criteria were retrospectively studied by analysis the course and prognoses of disease. All examinees were between 25-60 years of age (Xb=49.9, SD=10.3) with disease duration between 1-27 years (Xbox=6.41, SD=6.47). Course of the disease with "remissions and exacerbations", progressive continual course and bad prognoses, were more presented in seropositive group ofpatients. Partial remission was more common in seronegative patients but according to serostatus and gender has not shown statistically significant difference. Duration of the disease was a specific prognostic sign for both subsets [(r=0.32, p0.05) seronegative, (r=0.18, p<0.05) seropositive]. Seropositive and seronegative RA distinguish in course and prognostic feature, but not enough to differentiate them in two different forms of the disease. Regarding the sero-status, differences within sex, with some exceptions, are not relevant.

  2. Association of serum anti-rotavirus immunoglobulin A antibody seropositivity and protection against severe rotavirus gastroenteritis: analysis of clinical trials of human rotavirus vaccine.

    Cheuvart, Brigitte; Neuzil, Kathleen M; Steele, A Duncan; Cunliffe, Nigel; Madhi, Shabir A; Karkada, Naveen; Han, Htay Htay; Vinals, Carla

    2014-01-01

    Clinical trials of the human rotavirus vaccine Rotarix™ (RV1) have demonstrated significant reductions in severe rotavirus gastroenteritis (RVGE) in children worldwide. However, no correlate of vaccine efficacy (VE) has yet been established. This paper presents 2 analyses which aimed to investigate whether serum anti-RV IgA measured by ELISA 1 or 2 mo post-vaccination can serve as a correlate of efficacy against RVGE: (1) In a large Phase III efficacy trial (Rota-037), the Prentice criteria for surrogate endpoints was applied to anti-RV IgA seropositivity 1 mo post-vaccination. These criteria determine whether a significant vaccine group effect can be predicted from the surrogate, namely seropositivity (anti-RV IgA concentration>20 U/mL); (2) Among other GSK-sponsored RV1 VE studies, 8 studies which assessed immunogenicity at 1 or 2 mo post-vaccination in all or a sub-cohort of enrolled subjects and had at least 10 RVGE episodes were included in a meta-analysis to measure the regression between clinical VE and VE predicted from immunogenicity (VE1). In Rota-037, anti-RV IgA seropositivity post-vaccination was associated with a lower incidence of any or severe RVGE, however, the proportion of vaccine group effect explained by seropositivity was only 43.6% and 32.7% respectively. This low proportion was due to the vaccine group effect observed in seronegative subjects. In the meta-analysis, the slope of the regression between clinical VE and VE1 was statistically significant. These two independent analyses support the hypothesis that post-vaccination anti-RV IgA seropositivity (antibody concentration ≥20 U/mL) may serve as a useful correlate of efficacy in clinical trials of RV1 vaccines.

  3. Clinical utility of seropositive voltage-gated potassium channel-complex antibody.

    Jammoul, Adham; Shayya, Luay; Mente, Karin; Li, Jianbo; Rae-Grant, Alexander; Li, Yuebing

    2016-10-01

    Antibodies against voltage-gated potassium channel (VGKC)-complex are implicated in the pathogenesis of acquired neuromyotonia, limbic encephalitis, faciobrachial dystonic seizure, and Morvan syndrome. Outside these entities, the clinical value of VGKC-complex antibodies remains unclear. We conducted a single-center review of patients positive for VGKC-complex antibodies over an 8-year period. Among 114 patients positive for VGKC-complex antibody, 11 (9.6%) carrying the diagnosis of limbic encephalitis (n = 9) or neuromyotonia (n = 2) constituted the classic group, and the remaining 103 cases of various neurologic and non-neurologic disorders comprised the nonclassic group. The median titer for the classic group was higher than the nonclassic group ( p 0.25 nM) VGKC-complex antibody levels ( p VGKC-complex antibody titers are more likely found in patients with classically associated syndromes and other autoimmune conditions. Low-level VGKC-complex antibodies can be detected in nonspecific and mostly nonautoimmune disorders. The presence of VGKC-complex antibody, rather than its level, may serve as a marker of malignancy.

  4. Clinical utility of seropositive voltage-gated potassium channel–complex antibody

    Jammoul, Adham; Shayya, Luay; Mente, Karin; Li, Jianbo; Rae-Grant, Alexander

    2016-01-01

    Abstract Background: Antibodies against voltage-gated potassium channel (VGKC)–complex are implicated in the pathogenesis of acquired neuromyotonia, limbic encephalitis, faciobrachial dystonic seizure, and Morvan syndrome. Outside these entities, the clinical value of VGKC-complex antibodies remains unclear. Methods: We conducted a single-center review of patients positive for VGKC-complex antibodies over an 8-year period. Results: Among 114 patients positive for VGKC-complex antibody, 11 (9.6%) carrying the diagnosis of limbic encephalitis (n = 9) or neuromyotonia (n = 2) constituted the classic group, and the remaining 103 cases of various neurologic and non-neurologic disorders comprised the nonclassic group. The median titer for the classic group was higher than the nonclassic group (p 0.25 nM) VGKC-complex antibody levels (p VGKC-complex antibody titers are more likely found in patients with classically associated syndromes and other autoimmune conditions. Low-level VGKC-complex antibodies can be detected in nonspecific and mostly nonautoimmune disorders. The presence of VGKC-complex antibody, rather than its level, may serve as a marker of malignancy. PMID:27847683

  5. State estimation and optimal long period clinical treatment of HIV seropositive patients

    Juliana M. Grégio

    2009-03-01

    Full Text Available Optimal control theory provides a very interesting quantitative method that can be used to assist the decision making process in several areas of application, such as engineering, biology, economics and sociology. The main idea is to determine the values of the manipulated variables, such as drug doses, so that some cost function is minimized, subject to physical constraints. In this work, the cost function reflects the number of CD4+T cells, viral particles and the drug doses. It is worth noticing that high drug doses are related to more intense side-effects, apart from the impact on the actual cost of the treatment. In a previous paper by the authors, the LQR - Linear Quadratic Regulator approach was proposed for the computation of long period maintenance doses for the drugs, which turns out to be of state feedback form. However, it is not practical to determine all the components of the state vector, due to the fact that infected and uninfected CD4+T cells are not microscopically distinguishable. In order to overcome this difficulty, this work proposes the use of Extended Kalman Filter to estimate the state, even though, because of the nonlinear nature of the involved state equations, the separation principle may not be valid. Extensive simulations were then carried out to investigate numerically if the control strategy consisting of the feedback of estimated states yielded satisfactory clinical results.A teoria de controle ótimo apresenta um método quantitativo muito interessante que pode ajudar no processo de tomada de decisão em algumas áreas de aplicação, tais como engenharia, biologia, economia e sociologia. A principal idéia é determinar os valores das variáveis controladas, tais como doses de medicamentos, onde alguma função-custo é minimizada, sujeito às restrições físicas. Neste trabalho, a função-custo reflete o número de células CD4+T, partículas virais e doses de medicamentos. É fato que altas dosagens de

  6. A randomized clinical trial comparing cervical dysplasia treatment with cryotherapy vs loop electrosurgical excision procedure in HIV-seropositive women from Johannesburg, South Africa.

    Smith, Jennifer S; Sanusi, Busola; Swarts, Avril; Faesen, Mark; Levin, Simon; Goeieman, Bridgette; Ramotshela, Sibongile; Rakhombe, Ntombiyenkosi; Williamson, Anna L; Michelow, Pam; Omar, Tanvier; Hudgens, Michael G; Firnhaber, Cynthia

    2017-08-01

    Mortality associated with cervical cancer is a public health concern for women, particularly in HIV-seropositive women in resource-limited countries. HIV-seropositive women are at a higher risk of high-grade cervical precancer, which can eventually progress to invasive carcinoma as compared to HIV-seronegative women. It is imperative to identify effective treatment methods for high-grade cervical precursors among HIV-seropositive women. Randomized controlled trial data are needed comparing cryotherapy vs loop electrosurgical excision procedure treatment efficacy in HIV-seropositive women. Our primary aim was to compare the difference in the efficacy of loop electrosurgical excision procedure vs cryotherapy for the treatment of high-grade cervical intraepithelial neoplasia (grade ≥2) among HIV-seropositive women by conducting a randomized clinical trial. HIV-seropositive women (n = 166) aged 18-65 years with histology-proven cervical intraepithelial neoplasia grade ≥2 were randomized (1:1) to cryotherapy or loop electrosurgical excision procedure treatment at a government hospital in Johannesburg. Treatment efficacy was compared using 6- and 12-month cumulative incidence posttreatment of: (1) cervical intraepithelial neoplasia grade ≥2; (2) secondary endpoints of histologic cervical intraepithelial neoplasia grade ≥3 and grade ≥1; and (3) high-grade and low-grade cervical cytology. The study was registered (ClinicalTrials.govNCT01723956). From January 2010 through August 2014, 166 participants were randomized (86 loop electrosurgical excision procedure; 80 cryotherapy). Cumulative cervical intraepithelial neoplasia grade ≥2 incidence was higher for cryotherapy (24.3%; 95% confidence interval, 16.1-35.8) than loop electrosurgical excision procedure at 6 months (10.8%; 95% confidence interval, 5.7-19.8) (P = .02), although by 12 months, the difference was not significant (27.2%; 95% confidence interval, 18.5-38.9 vs 18.5%; 95% confidence interval, 11

  7. Prospective evaluation of human immunodeficiency virus-seropositive patients

    Post, M.J.D.; Berger, J.R.; Quencer, R.M.

    1989-01-01

    As part of a prospective longitudinal study of individuals who are human immunodeficiency virus (HIV) positive, cranial MR imaging was performed on 89 HIV-seropositive patients and correlated with clinical data. MR results were asymptomatics: MR images normal-58, abnormal-16; myelopathics: normal-seven, abnormal-four; encephalopathics: normal-three, abnormal-two. In asymptomatics, neurologic examination was positive in all with positive MR results but positive in only some with negative MR results. The authors concluded that MR imaging can show indirect evidence of HIV infection early in the disease, but abnormalities will be minor and seen only in the minority (21%) of symptomatics; these minor abnormalities may antedate clinical symptoms but not signs; an increase in severity of clinical disease correlates with increasingly severe atrophy and demyelination; and in some seropositives, whether asymptomatic or symptomatic, MR results remain normal

  8. Radiographic estimation in seropositive and seronegative rheumatoid arthritis.

    Sahatçiu-Meka, Vjollca; Rexhepi, Sylejman; Manxhuka-Kërliu, Suzana; Rexhepi, Mjellma

    2011-08-01

    Long since it have been suggested that a subpopulation of patients with rheumatoid arthritis, diagnosed with negative rheumatoid factor tests, represents a clinical entity quite distinct from that of seropositive rheumatoid arthritis (RA). Our aim was to establish a scientific comparative analysis between seronegative and seropositive rheumatoid arthritis, regarding some radiological and clinical parameters, applied for the first time on patients from Kosovo. Two hundred fifty patients with rheumatoid arthritis according to the American College of Rheumatology criteria were retrospectively studied by analysis the radiographic damage and clinical parameters of the disease, using a data base. All examinees were between 25-60 years of age (Xb=49.96, SD=10.37) with disease duration between 1-27 years (Xb = 6.41, SD=6.47). All patients underwent a standardised evaluation radiographs. Baseline standardised poster anterior radiographs of hands and feet and radiographs of other joints, depending on indications, were assessed. Erythrocyte sedimentation rate values correlated with the radiological damages and statistical difference was found for seronegative subset (r=0.24, p<0.01). Longer duration of the disease resulted in the increase of radiological changes in both subsets (r=0.66, p<0.01) seronegative, (r=0.49, p<0.01) seropositive. Anatomic changes of IInd and IIIrd level were nearly equally distributed in both subsets, 76 (60.8%) seronegative, 75 (60%) seropositive. Radiological damages are nearly equal in both subsets, elevate in relation to the duration of the disease and correlate with ESR values. Regarding the sero-status, differences within sex, with some exceptions, are not relevant. Although there are some definite quantitative and qualitative differences regarding sero-status, obviously there is a great deal of overlap between the two groups.

  9. Radiographic estimation in seropositive and seronegative rheumatoid arthritis

    Vjollca Sahatçiu-Meka

    2011-08-01

    Full Text Available Long since it have been suggested that a subpopulation of patients with rheumatoid arthritis, diagnosed with negative rheumatoid factor tests, represents a clinical entity quite distinct from that of seropositive rheumatoid arthritis (RA. Our aim was to establish a scientific comparative analysis between seronegative and seropositive rheumatoid arthritis, regarding some radiological and clinical parameters, applied for the first time on patients from Kosovo. Two hundred fifty patients with rheumatoid arthritis according to the American College of Rheumatology criteria were retrospectively studied by analysis the radiographic damage and clinical parameters of the disease, using a data base. All examinees were between 25-60 years of age (Xb=49.96, SD=10.37 with disease duration between 1-27 years (Xb = 6.41, SD=6.47. All patients underwent a standardised evaluation radiographs. Baseline standardised poster anterior radiographs of hands and feet and radiographs of other joints, depending on indications, were assessed. Erythrocyte sedimentation rate values correlated with the radiological damages and statistical difference was found for seronegative subset (r=0.24, p<0.01. Longer duration of the disease resulted in the increase of radiological changes in both subsets (r=0.66, p<0.01 seronegative, (r=0.49, p<0.01 seropositive. Anatomic changes of IInd and IIIrd level were nearly equally distributed in both subsets, 76 (60.8% seronegative, 75 (60% seropositive. Radiological damages are nearly equal in both subsets, elevate in relation to the duration of the disease and correlate with ESR values. Regarding the sero-status, differences within sex, with some exceptions, are not relevant. Although there are some definite quantitative and qualitative differences regarding sero-status, obviously there is a great deal of overlap between the two groups.

  10. Celiac disease: clinical observations

    Yu. A. Emel’yanova

    2016-01-01

    Full Text Available Presented clinical cases of patients with a diagnosis of gluten enteropathy in treatment in the department of gastroenterology Regional Clinical Hospital. The case is of interest to doctors of different specialties for the differential diagnosis of anemia and malabsorption syndrome, demonstrate both the classic version, and atypical forms of the disease course. Diagnosis of celiac disease is based on three key positions: clinical findings, histology and serological markers. The clinical picture of celiac disease is characterized by pronounced polymorphism, by going beyond the a gastroenterological pathology. For screening of gluten sensitive celiac typically used an antibody to tissue transglutaminase. Morphological research of the mucous membrane of the small intestine is the determining criterion in the diagnosis of celiac disease. The use of specific gluten-free diet leads to the positive dynamics of the disease and improve the quality of life of patients.

  11. Seroprevalences of feline leukemia virus and feline immunodeficiency virus infection in cats in the United States and Canada and risk factors for seropositivity.

    Burling, Amie N; Levy, Julie K; Scott, H Morgan; Crandall, Michael M; Tucker, Sylvia J; Wood, Erin G; Foster, Jessie D

    2017-07-15

    OBJECTIVE To estimate seroprevalences for FeLV antigen and anti-FIV antibody and risk factors for seropositivity among cats in the United States and Canada. DESIGN Cross-sectional study. ANIMALS 62,301 cats tested at 1,396 veterinary clinics (n = 45,406) and 127 animal shelters (16,895). PROCEDURES Blood samples were tested with a point-of-care ELISA for FeLV antigen and anti-FIV antibody. Seroprevalence was estimated, and risk factors for seropositivity were evaluated with bivariate and multivariable mixed-model logistic regression analyses adjusted for within-clinic or within-shelter dependencies. RESULTS Overall, seroprevalence was 3.1% for FeLV antigen and 3.6% for anti-FIV antibody. Adult age, outdoor access, clinical disease, and being a sexually intact male were risk factors for seropositivity for each virus. Odds of seropositivity for each virus were greater for cats tested in clinics than for those tested in shelters. Of 1,611 cats with oral disease, 76 (4.7%) and 157 (9.7%) were seropositive for FeLV and FIV, respectively. Of 4,835 cats with respiratory disease, 385 (8.0%) were seropositive for FeLV and 308 (6.4%) were seropositive for FIV. Of 1,983 cats with abscesses or bite wounds, 110 (5.5%) and 247 (12.5%) were seropositive for FeLV and FIV, respectively. Overall, 2,368 of 17,041 (13.9%) unhealthy cats were seropositive for either or both viruses, compared with 1,621 of 45,260 (3.6%) healthy cats. CONCLUSIONS AND CLINICAL RELEVANCE Seroprevalences for FeLV antigen and anti-FIV antibody were similar to those reported in previous studies over the past decade. Taken together, these results indicated a need to improve compliance with existing guidelines for management of feline retroviruses.

  12. Feline Toxoplasmosis: Tumor Necrosis Factor, Nitric Oxide, and Free Radicals in Seropositive Cats.

    Faria, Joice L M; Couto, Caroline do; Wierzynski, Sheron L; Bottari, Nathieli B; Baldissera, Matheus D; Pereira, Wanderson A B; Da Silva, Aleksandro S

    2018-02-01

    Toxoplasma gondii is a cosmopolitan protozoan that causes disease in several species, including humans. In cats, these infections are usually asymptomatic, but in other species they can lead to high levels of inflammatory and cell damage markers, causing cellular damage. Therefore, the aim of this study was to measure levels of tumor necrosis factor (TNF-α), reactive oxygen species (ROS), and nitric oxide (nitrite/nitrate-NO x ) in the serum of cats seropositive for T. gondii. Initially, we investigated the presence of antibodies against T. gondii in cats in the city of Concordia, Santa Catarina, Brazil, with the use of indirect immunofluorescence (IFA), and found 30 cats seropositive for T. gondii and 30 seronegative cats. In this study, seropositive cats showed higher levels of TNF-α, ROS, and NO x compared to seronegative cats. Although cats do not show clinical signs of disease, constant inflammatory response can cause cell damage, which over time may adversely affect the animal.

  13. Kidney Disease in Human Immunodeficiency Virus-seropositive Patients: Absence of Human Immunodeficiency Virus-associated Nephropathy was a Characteristic Feature.

    Prakash, J; Ganiger, V; Prakash, S; Sivasankar, M; Sunder, S; Singh, U

    2017-01-01

    Human immunodeficiency virus (HIV) infection can cause a broad spectrum of renal diseases. However, there is paucity of Indian data on the patterns of renal lesions in HIV-seropositive patients. The aim of the present study was to delineate the spectrum of renal lesions in HIV/acquired immunodeficiency syndrome patients. In this prospective study, all HIV-positive patients of both genders aged >18 years were screened for renal disease. Patients with proteinuria of more than 1 g/24 h were subjected to renal biopsy. A total of 293 HIV-positive patients were screened; of these, 136 (46.4%) patients found to have renal involvement. Dipstick-positive proteinuria of 1+ or more was observed in 112 (38.2%) patients, and 16 (14.2%) patients had proteinuria of more than 1 g/24 h. Renal biopsy in 14 cases revealed glomerulonephritis (GN) in 12 (85.7%) (isolated GN in 4 [28.5%] and GN mixed with chronic TIN in 8 [57.1%]) patients. These include mesangioproliferative GN in 5 (35.7%), membranoproliferative GN in 2 (14.2%), focal segmental glomerulosclerosis in 2 (14.2%), diffuse proliferative GN in 2 (14.2%), and diabetic nephropathy in 1 (7.1%) patients. Chronic interstitial nephritis was noted in 10 (71.42%) (superimposed on GN in 8 [57.1%], isolated in 2 [14.2%]) patients. Granulomatous interstitial nephritis was seen in 3 (24.1%) cases. GN and chronic interstitial nephritis were noted in 85.7% and 71.42% of patients, respectively, mostly superimposed on each other. Mesangioproliferative GN was the most common glomerular lesion, but classical HIV-associated nephropathy was not observed.

  14. Seropositivity for Enterocytozoon bieneusi, Czech Republic

    Sak, Bohumil; Kučerová, Z.; Kváč, Martin; Květoňová, Dana; Rost, M.; Secor, W. E.

    2010-01-01

    Roč. 16, č. 2 (2010), s. 335-337 ISSN 1080-6040 R&D Projects: GA AV ČR KJB500960701; GA ČR GP523/07/P117 Institutional research plan: CEZ:AV0Z60220518 Keywords : Enterocytozoon bieneusi * INTESTINAL MICROSPORIDIOSIS * seropositivity * human * DIARRHEA * SEROPREVALENCE Subject RIV: GJ - Animal Vermins ; Diseases , Veterinary Medicine Impact factor: 6.859, year: 2010

  15. The severity of the articular deformities of the hands depending on the seropositivity in rheumatoid arthritis

    Oprea Doiniţa

    2017-11-01

    Full Text Available Background. Rheumatoid arthritis (RA, met in the literature also as chronic evolving polyarthritis, is a chronic inflammatory disease, which typically involves the small and medium joints, with a symmetric distribution. Clinical studies shown that a proportion of patients with RA, without measurable rheumatoid factors are a distinct entity from those patients with seropositive RA. Around one third of patients with RA are seronegative for those two serological markers, the rheumatoid factors (RF and anti-citrullinated peptide antibodies (Anti-CCP. Hands are the most frequently affected in both seropositive and seronegative RA and the first joints involved in the inflammatory process are the metacarpo-phalangeal joints, IFP joints and the radiocarpian joint. The big joints become later symptomatic, after the small joints are affected. Materials and methods: 66 patients files and the personal records of the patients consecutively admitted in the Rheumatology Department of the Clinical Emergency County Hospital Constanta. Patients from county Constanta were included in the study, with diagnosis of rheumatoid arthritis (diagnosis confirmed at least 2 years ago, including both forms (seropositive and seronegative of the disease. The complete clinical exam was done for the patients included in the study, following the identification of the active joint disease. The mechanical status of the joint was observed by a single evaluator for each of those 66 patients with RA, with special focus on the presence of the deformities / ankyloses of the hands joints. Therefore, there were evaluated: the mobility (the number of the joints with limited movements, deformities, ankyloses (the number of the joints without mobility. The joint mobility was assessed by counting the hand joints with limited movements and the worsening of the prehension function. The presence of the following hand deformities was followed: fingers in “swan neck”, “fusiform fingers

  16. Association between Helicobacter pylori seropositivity and Hepatic Encephalopathy

    Behroozian, R.; Faramarzpur, M.; Rahimi, E.

    2010-01-01

    Objective: The knowledge on Helicobacter pylori (H. pylori) contribution in the pathology of the liver and biliary tract diseases in human is very limited. The aim of this study was to assess the probable association between H. pylori seropositivity and hepatic encephalopathy. Methodology: This is a case control study conducted through three groups, cirrhotics with hepatic encephalopathy (HE), cirrhotics without HE and healthy controls. All subjects were examined serologically for determination of IgG class antibodies to H. pylori based on ELISA technique. Results: H. pylori seropositivity was present in 88% cirrhotic patients with hepatic encephalopathy, 86% cirrhotics without hepatic encephalopathy and 66% healthy controls. Conclusion: According to our results, H. pylori seropositivity rate in cirrhotic patients with or without hepatic encephalopathy was higher than healthy controls. But H. pylori seropositivity rate was not significantly different among cirrhotics with hepatic encephalopathy and those without it.

  17. Clinical patterns in Parkinson's disease

    Rooden, Stephanie Maria van

    2012-01-01

    The clinical heterogeneity of Parkinson’s disease (PD) patients may reflect the existence of subtypes of the disease. PD subtypes have often been defined by a classification according to researcher-specified criteria, such as age-at-onset or predominant clinical motor features. The general objective

  18. LEBER'S DISEASE. CLINICAL CASE

    N. N. Maslova

    2013-01-01

    Full Text Available Violation of visual functions and oculomotor frustration develop at 90% of patients with MS. More than a half of patients are transferred by the numerous recurrence of an optical neuritis which is coming to an end with a partial atrophy of optic nerve. For well-timed purpose of pathogenetic treatment differential diagnostics with other diseases, being accompanied an atrophy of an optic nerve, in particular with Leber's disease

  19. Clinical neurogenetics: huntington disease.

    Bordelon, Yvette M

    2013-11-01

    Huntington disease (HD) is an autosomal dominant, adult-onset, progressive neurodegenerative disease characterized by the triad of abnormal movements (typically chorea), cognitive impairment, and psychiatric problems. It is caused by an expanded CAG repeat in the gene encoding the protein huntingtin on chromosome 4 and causes progressive atrophy of the striatum as well as cortical and other extrastriatal structures. Genetic testing has been available since 1993 to confirm diagnosis in affected adults and for presymptomatic testing in at-risk individuals. This review covers HD signs, symptoms, and pathophysiology; current genetic testing issues; and current and future treatment strategies. Copyright © 2013 Elsevier Inc. All rights reserved.

  20. Clinical evaluation of Morgellons disease in a cohort of North American patients.

    Fesler, Melissa C; Middelveen, Marianne J; Stricker, Raphael B

    2018-04-23

    Morgellons disease (MD) is a dermatological condition characterized by aberrant production of keratin and collagen fibers in skin. Although infection with Borrelia burgdorferi , the causative agent of Lyme disease (LD), has been associated with MD, relatively few studies have hitherto provided epidemiological evidence regarding this association. A cohort of 1000 seropositive North American LD patients was evaluated for the presence of MD. Patients were diagnosed with MD based on detection of microscopic fibers in skin lesions or under unbroken skin. Demographic and clinical features of MD patients were analyzed, and laboratory testing for tickborne coinfections and other infectious agents, was performed. Subjective and objective features of MD were analyzed using statistical methods. Of 1000 seropositive LD patients, 60 (6%) were diagnosed with MD. Of these 60 patients, 75% were female and 78% presented in the late disseminated stage of MD. All 60 patients (100%) were seropositive for B. burgdorferi infection. Tickborne coinfections in these patients included Babesia spp (62%), Bartonella and Rickettsia (25% each), Ehrlichia (15%) and Anaplasma (10%). Helicobacter pylori was detected in 12% of MD patients. In all, 77% of MD patients had one or more coinfections. This study confirms recent findings that MD occurs in a limited subset of LD patients. The clinical and genetic determinants of MD in LD patients require further study.

  1. Seropositivity for Trypanosoma cruzi in domestic dogs from Sonora, Mexico.

    Arce-Fonseca, Minerva; Carrillo-Sánchez, Silvia C; Molina-Barrios, Ramón M; Martínez-Cruz, Mariana; Cedillo-Cobián, Jesús R; Henao-Díaz, Yuly A; Rodríguez-Morales, Olivia

    2017-09-05

    Chagas disease is an important health problem in Latin America due to its incapacitating effects and associated mortality. Studies on seropositivity for Trypanosoma cruzi in Mexican dogs have demonstrated a direct correlation between seropositivity in humans and dogs, which can act as sentinels for the disease in this region. The objective of this study was to determine the seropositivity for T.cruzi infection in dogs from Sonora, a northern borderstate of Mexico. Responsible pet owners were selected at random from an urban area of Empalme municipality, Sonora, Mexico, and from there, 180 dog samples were collected. Anti-T. cruzi antibodies were determined using the enzyme-linked immunosorbent assay (ELISA) method. Reactive ELISA sera were processed by indirect immunofluorescence to confirm the presence of anti-T. cruzi antibodies. For the statistical analysis, chi-square tests were conducted. Dogs' sera showed a seropositivity rate of 4.44%. The rate of seropositivity was not associated with the dogs' age, sex, or socioeconomics pertaining to the geographical area. One sample (1/180, 0.55%) showed the acute state of the disease. The study found a presence of anti-T. cruzi antibodies in dogs in this area, which suggests vector transmission. There is a need for active surveillance programs throughout the state of Sonora and vector control strategies should also be implemented in endemic regions.

  2. Clinical heterogeneity in Fabry disease

    G. N. Salogub

    2015-01-01

    Full Text Available Fabry disease is an X-linked, lysosomal storage disease (OMIM: 301500, caused by α-galactosidase A deficiency, resulting in accumulation of its substrates, glycosphingolipids, primarily – globotriaosylceramide, in the lysosomes of multiple cell types with multi-system clinical manifestations, even within the same family, including abnormalities of the central and peripheral nervous system, kidneys, heart, gastrointestinal tract, lungs, organ of vision. Clinical heterogeneity is often the reason of the delayed diagnosis. Nowadays enzyme replacement therapy has proved its efficiency in the treatment of Fabry disease. Including Fabry disease in the differential diagnosis of a large range of disorders is important because of its wide clinical heterogeneity and the possibility of an earlier intervention with a beneficial treatment.

  3. Extra-Articular Manifestations of Seronegative and Seropositive Rheumatoid Arthritis

    Vjollca Sahatçiu-Meka

    2010-02-01

    Full Text Available Although considered a “joint disease,” rheumatoid arthritis is associated with the involvement of extra-articular manifestations. The aim of the study is the investigation and comparison of frequency and type of extra-articular manifestations in a well defined community based cohort of patients with seropositive and seronegative rheumatoid arthritis. Using the ACR (1987 criteria for rheumatoid arthritis, patients have been classified into the 2nd and 3rd functional class (ARA. The studied group consisted of 125 seronegative patients with titters lower than 1:64 as defined by Rose-Waaler test, whereas the control group consisted of 125 seropositive patients with titters of 1:64 or higher. All patients were between 25-60 years of age (Xb=49,96, with disease duration between 1-27 years (Xb=6,41. In order to present the findings of the study, the structure, prevalence, arithmetic mean (Xb, standard deviation (SB, variation quotient (QV% and variation interval (Rmax-Rmin have been used. Probability level has been expressed by p<0,01 and p<0,05. Correlation between the number of extra-articular manifestations and duration of the disease has been calculated by means of Pearson linear correlation. Higher presence of diffuse lung fibrosis, central and peripheral nervous system damages have been confirmed in the seropositive group, and osteoporosis in the seronegative; however, no statistical difference has been found. In extra-articular manifestations, “rheumatoid core” in the seropositive subset (χ2=4,80, p<0,05 presented significant statistical difference. Rheumatoid nodules were more frequent in seropositive subset (12%:16%, in both sexes; however, they were not of significant statistical difference. Neuropathy and lung diseases were also frequently present in seropositive group, but no statistical difference has been found regarding the statistical difference. Longer duration of the disease resulted in an increase of the number of extra

  4. Extra-articular manifestations of seronegative and seropositive rheumatoid arthritis.

    Sahatciu-Meka, Vjollca; Rexhepi, Sylejman; Manxhuka-Kerliu, Suzana; Rexhepi, Mjellma

    2010-02-01

    Although considered a "joint disease," rheumatoid arthritis is associated with the involvement of extra-articular manifestations. The aim of the study is the investigation and comparison of frequency and type of extra-articular manifestations in a well defined community based cohort of patients with seropositive and seronegative rheumatoid arthritis. Using the ACR (1987) criteria for rheumatoid arthritis, patients have been classified into the 2nd and 3rd functional class (ARA). The studied group consisted of 125 seronegative patients with titters lower than 1:64 as defined by Rose-Waaler test, whereas the control group consisted of 125 seropositive patients with titters of 1:64 or higher. All patients were between 25-60 years of age (Xb=49,96), with disease duration between 1-27 years (Xb=6,41). In order to present the findings of the study, the structure, prevalence, arithmetic mean (Xb), standard deviation (SB), variation quotient (QV%) and variation interval (Rmax-Rmin) have been used. Probability level has been expressed by p<0,01 and p<0,05. Correlation between the number of extra-articular manifestations and duration of the disease has been calculated by means of Pearson linear correlation. Higher presence of diffuse lung fibrosis, central and peripheral nervous system damages have been confirmed in the seropositive group, and osteoporosis in the seronegative; however, no statistical difference has been found. In extra-articular manifestations, "rheumatoid core" in the seropositive subset (chi2=4,80, p<0,05) presented significant statistical difference. Rheumatoid nodules were more frequent in seropositive subset (12%:16%), in both sexes; however, they were not of significant statistical difference. Neuropathy and lung diseases were also frequently present in seropositive group, but no statistical difference has been found regarding the statistical difference. Longer duration of the disease resulted in an increase of the number of extra

  5. Incidence of seropositive myasthenia gravis in Cape Town and ...

    Background. Myasthenia gravis (MG) is a treatable autoimmune disease characterised by fatiguable weakness of skeletal muscles. More than 85% of MG patients have antibodies to the acetylcholine receptor (AChR) at the neuromuscular junction or are seropositive for MG (SPMG). In the developed world the incidence of ...

  6. Periodontal status and dental care in HIV seropositive patients

    Maria Sueli Marques Soares

    2009-12-01

    Full Text Available Objective: The aim of the study was to evaluate the correlation between CD4+ cell count and viral load with periodontal and dental status in HIV seropositive patient. Methods: Forty HIV seropositive patients were selected from the dental clinic of the Clementino Fraga Hospital in João Pessoa, Paraíba, Brazil. The Community Periodontal Index, Simplified Oral Hygiene Index and Decayed, Missing or Filled Teeth Index were determined. The values of CD4+ lymphocyte count and viral load were collected from the medical records. Descriptive analysis was made and Spearman’s correlation test was applied, with values of p<0.05 being significant. Results: HIV seropositive patients were aged 33 to 47 years (mean = 40 years, 26 were men and 14 women. The mean CD4+ lymphocyte count was 507.82±306.4 cells/mm3, with 41% being a high, 38.5% a medium and 20.5% a low level. Sixty-five percent of the patients had undetectable viral load, 12.5% had a high, 5% a low and 5% a medium viral load. Most patients (87.5% presented with periodontal disease, calculus was the most frequent condition (40%. The mean Decayed, Missing or Filled Tooth Index was 19.7±6.8, with prevalence of the missing component. There was significant statistical correlation between the CD4+ count and the periodontal condition, p=0.046. There was no significant correlation between the CD4+ count and the Decayed, Missing or Filled Teeth Index (p=0.469 and the Simplified Oral Hygiene Index (p=0.189 indexes, and between viral load and Decayed, Missing or Filled Teeth Index (p=0.452, the Simplified Oral Hygiene Index (p=0.158 and Community Periodontal Index (p=0.216. Conclusion: The periodontal condition was influenced to a greater by the CD4+ cell count than the viral load, while there was no correlation between the decayed condition and the CD4+ cell count or the viral load condition.

  7. Trichinella infection and clinical disease

    Clausen, M R; Meyer, C N; Krantz, T

    1996-01-01

    Trichinellosis is caused by ingestion of insufficiently cooked meat contaminated with infective larvae of Trichinella species. The clinical course is highly variable, ranging from no apparent infection to severe and even fatal disease. We report two illustrative cases of trichinellosis. Returning....... Life-threatening cardiopulmonary, renal and central nervous system complications developed. The patient recovered after several months. Her husband, who also ate the pork, did not have clinical symptoms, but an increased eosinophil count and a single larva in a muscle biopsy confirmed infection....... The epidemiology, clinical manifestations, diagnosis, treatment and prevention of trichinellosis are reviewed....

  8. Comparison of polysomnographic data in age-, sex- and Axis I psychiatric diagnosis matched HIV-seropositive and HIV-seronegative insomnia patients.

    Low, Yinghui; Goforth, Harold W; Omonuwa, Toma; Preud'homme, Xavier; Edinger, Jack; Krystal, Andrew

    2012-12-01

    There is a high prevalence of insomnia in HIV-seropositive patients. Insomnia is associated with poorer disease outcomes, cognitive impairment and HIV-associated dementia. However there is limited data characterizing the type of sleep disturbances, and the cause. Previous studies report conflicting results, and observed changes in the distribution of REM and SWS were hypothesized to result from co-morbid mood disorders, although this is not established. We carried out this study to determine if there are differences in polysomnographic (PSG) sleep data in age-, sex- and Axis I diagnoses- matched HIV-seropositive and HIV-seronegative patients. Eighteen HIV-seropositive insomniacs were matched to HIV-seronegative insomniacs based on age, sex and Axis I diagnoses. Participants spent 2 consecutive nights in a sleep lab recording of PSG data. Multivariate analysis revealed an overall significant match-by-variable interaction (p=0.0126). Follow-up analysis show that compared to HIV-seronegative insomnia controls, HIV-seropositive insomniacs have significantly longer SOL, 8% decreased sleep efficiency, and 8-10% decreased time spent in REM sleep (p'saccounting for differences in age, sex and psychiatric diagnoses, HIV-seropositive patients with insomnia have significantly worse sleep than HIV-seronegative patients with insomnia. Unlike what previous authors have proposed, our results do not support the view that comorbid psychiatric disorders like depression are responsible for the observed differences in PSG findings and the greater incidence of insomnia, in HIV-seropositive patients when compared with other groups of insomnia patients. This suggests the presence of other etiologies including neuronal damage, psychosocial stressors, or comorbid medical conditions. Further studies are needed to determine the extent to which these play a role in insomnia in the HIV-seropositive population. Copyright © 2012 International Federation of Clinical Neurophysiology. Published by

  9. Neuro degenerative diseases: clinical concerns

    Ibanez, V.

    2005-01-01

    Idiopathic Parkinson's disease (PD) and Alzheimer's disease (AD) are the main neuro-degenerative diseases (NDDs) seen clinically. They share some common clinical symptoms and neuro-pathological findings. The increase of life expectancy in the developed countries will inevitably contribute to enhance the prevalence of these diseases. Behavioral disorders, common in NDDs, will produce major care management challenges. Idiopathic Parkinson's disease corresponds to a histopathological diagnosis, based on the observation of a de-pigmentation and a neuronal loss in the substantia nigra, as well as on the presence of intra-neuronal inclusion bodies. AD is insidious with slowly progressive dementia in which the decline in memory constitutes the main complaint. The diagnosis of definite AD requires the presence of clinical criteria as well as the histopathological confirmation of brain lesions. The two main lesions are the presence of senile plaques and neuro-fibrillary tangles. Positron emission tomography (PET) explores cerebral metabolism and neurotransmitter kinetics in NDDs using principally [ 18 F]-deoxyglucose and [ 18 F]-dopa. Nigrostriatal dopaminergic function is altered in PD, as evidenced by the low uptake of [ 18 F]-dopa in the posterior putamen as compared to anterior putamen and caudate nucleus. In contrast, [ 18 F]-dopa uptake is equally depressed in all striatal structures in progressive supra-nuclear palsy. Regional glucose metabolism at rest is preserved in elderly once cerebral atrophy is taken into account. On the contrary, glucose metabolism is globally reduced in AD, with marked decrease in the parietal and temporal regions. PET has proved to be useful to study in vivo neurochemical processes in patients suffering from NDDs. The potential of this approach is still largely unexploited, and depends on new ligand production to establish early diagnosis and treatment follow-up. (author)

  10. Huntington's disease: a clinical review

    Roos Raymund AC

    2010-12-01

    Full Text Available Abstract Huntington disease (HD is a rare neurodegenerative disorder of the central nervous system characterized by unwanted choreatic movements, behavioral and psychiatric disturbances and dementia. Prevalence in the Caucasian population is estimated at 1/10,000-1/20,000. Mean age at onset of symptoms is 30-50 years. In some cases symptoms start before the age of 20 years with behavior disturbances and learning difficulties at school (Juvenile Huntington's disease; JHD. The classic sign is chorea that gradually spreads to all muscles. All psychomotor processes become severely retarded. Patients experience psychiatric symptoms and cognitive decline. HD is an autosomal dominant inherited disease caused by an elongated CAG repeat (36 repeats or more on the short arm of chromosome 4p16.3 in the Huntingtine gene. The longer the CAG repeat, the earlier the onset of disease. In cases of JHD the repeat often exceeds 55. Diagnosis is based on clinical symptoms and signs in an individual with a parent with proven HD, and is confirmed by DNA determination. Pre-manifest diagnosis should only be performed by multidisciplinary teams in healthy at-risk adult individuals who want to know whether they carry the mutation or not. Differential diagnoses include other causes of chorea including general internal disorders or iatrogenic disorders. Phenocopies (clinically diagnosed cases of HD without the genetic mutation are observed. Prenatal diagnosis is possible by chorionic villus sampling or amniocentesis. Preimplantation diagnosis with in vitro fertilization is offered in several countries. There is no cure. Management should be multidisciplinary and is based on treating symptoms with a view to improving quality of life. Chorea is treated with dopamine receptor blocking or depleting agents. Medication and non-medical care for depression and aggressive behavior may be required. The progression of the disease leads to a complete dependency in daily life, which

  11. Oxidative stress in dairy cows seropositives for Neospora caninum.

    Glombowsky, Patrícia; Bottari, Nathieli B; Klauck, Vanderlei; Fávero, Juscivete F; Soldá, Natan M; Baldissera, Matheus D; Perin, Gessica; Morsch, Vera M; Schetinger, Maria Rosa C; Stefani, Lenita M; Da Silva, Aleksandro S

    2017-10-01

    Bovine neosporosis is caused by the protozoan Neospora caninum and is one of the major causes of abortion in cows. Cattle are intermediate hosts of this parasite and may have asymptomatic or symptomatic infections. Therefore, the aim of this study was to evaluate oxidative stress marker reactive oxygen species (ROS), thiobarbituric reactive acid substances (TBARS) levels, glutathione S-transferase (GST), adenosine deaminase (ADA), and butyrylcholinesterase (BChE) activities in dairy cows seropositives for N. caninum (asymptomatic or symptomatic). Dairy cows (n=90) were tested by immunofluorescent antibody assay (IFA) for N. caninum and divided accordingly into three groups: the group A (seronegatives, n=30), the group B (seropositives and asymptomatic, n=30), and the group C (seropositives and symptomatic, n=30). It was observed increased levels of TBARS and reduced (Pcows (the group C) compared to seronegatives dairy cows (the group A). Based on these results, it was observed that seropositive animals showed cell damage associated with oxidative stress and inflammation, mainly in those with symptomatic infections. Increased seric ROS levels and BChE activity may have influenced N. caninum pathogenesis in symptomatic animals due to increased cell damage and exacerbated inflammatory response, leading to the development of clinical signs. Copyright © 2017 Elsevier Ltd. All rights reserved.

  12. Clinical investigation in Wilson's disease

    Mizutani, Naoki; Maehara, Mitsuo; Negoro, Tamiko; Watanabe, Kazuyoshi

    1983-01-01

    Wilson's disease of cerebral type with a chief complaint of tremor occurred in 3 brothers and sisters of a certain family line. Treatment with D-penicillamine produced remarkable clinical improvement as shown by the disappearance of tremor and Kayser-Fleischer ring. Cranial CT scans made before the treatment revealed abnormal findings such as low density areas in the (bilateral) thalamus and the lenticular nucleus, atrophy of the cerebral cortex, and enlargement of the ventricles. After the treatment, cranial CT revealed that the low density areas in the thalamus and the lenticular nucleus disappeared corresponding to the clinical improvement. However, the atrophy of the cerebral cortex and the enlargement of the ventricles were not ameliorated. (Ueda, J.)

  13. Clinical studies on thyroid diseases

    Eskes, S.A.

    2014-01-01

    This thesis focuses on some aspects of thyroid disease: prevention of autoimmune thyroid disease (AITD), diagnosis of related conditions as autoimmune hypophysitis in autoimmune hypothyroidism (Hashimoto’s disease), and treatment of amiodarone-induced thyrotoxicosis (AIT).

  14. Leptospira seropositivity as a risk factor for Mesoamerican Nephropathy.

    Riefkohl, Alejandro; Ramírez-Rubio, Oriana; Laws, Rebecca L; McClean, Michael D; Weiner, Daniel E; Kaufman, James S; Galloway, Renee L; Shadomy, Sean V; Guerra, Marta; Amador, Juan José; Sánchez, José Marcel; López-Pilarte, Damaris; Parikh, Chirag R; Leibler, Jessica H; Brooks, Daniel R

    2017-01-01

    Leptospirosis is postulated as a possible cause of Mesoamerican Nephropathy (MeN) in Central American workers. Investigate job-specific Leptospira seroprevalence and its association with kidney disease biomarkers. In 282 sugarcane workers, 47 sugarcane applicants and 160 workers in other industries, we measured anti-leptospiral antibodies, serum creatinine, and urinary injury biomarkers, including neutrophil gelatinase-associated lipocalin (NGAL), interleukin-18 (IL-18), and N-acetyl-D-glucosaminidase (NAG). Leptospira seroprevalence differed among job categories and was highest among sugarcane cutters (59%). Seropositive sugarcane workers had higher NGAL concentrations (relative mean: 1.28; 95% CI: 0.94-1.75) compared to those who were seronegative, with similar findings among field and non-field workers. Leptospira seroprevalence varied by job category. There was some indication that seropositivity was associated with elevated biomarker levels, but results were inconsistent. Additional studies may help establish whether Leptospira infection plays any role in MeN among Central American workers.

  15. Infectious Disease Clinical Research Program (IDCRP)

    Federal Laboratory Consortium — Our mission is to conduct infectious disease clinical research of importance to the military through a unique, adaptive, and collaborative network, to inform health...

  16. Lymph nodes cytology in HIV seropositive cases with haematological alterations

    Neelima Tirumalasetti

    2014-01-01

    Full Text Available Background & objectives: Lymphadenopathy and haematological alterations are the earliest manifestations with other associated opportunistic infections and malignancies. Hence, there is a need for simple investigations like fine needle aspiration cytology (FNAC for evaluation of HIV lymphadenopathy and a haemogram to interpret the haematological alterations. This study was undertaken to analyze the cytological patterns of lymph node lesions in HIV/AIDS patients, to compare with available clinico-pathological and haematological parameters to segregate lymphadenopathy cases for further evaluation. Methods: In the present study, 129 HIV seropositive patients were included. Lymph node aspirates were stained routinely with hematoxylin and eosin and Ziehl-Neelsen (Z-N stains. Special stains and cultures were done in selected patients. Peripheral smears were taken from all the patients and CD4 counts were recorded. Tuberculous lymphadenitis was further categorized. Acid fast bacilli (AFB grading was done on Z-N positive smears. Each lesion was compared with CD4 counts, WHO clinical staging and haematological picture. Results: Cytological diagnosis in 129 patients included tuberculous (n=54, 41.9%, reactive lymphadenopathy (n=46, 35.6%, suppurative (n=16, 12.4% lymphadenitis, non-Hodgkin′s lymphoma (n=4, 3.1%, and Hodgkin′s lymphoma, secondary deposits, other granulomatous lesions, and cryptoccocal lymphadenitis in one patient each. The predominant cytomorphological pattern in tuberculous lymphadenitis was caseous necrosis + epithelioid granuloma formation (51.85%. Grade 2+ Z-N grading was noted in 62.96 per cent of AFB positive smears. CD4 counts showed a descending pattern with progression of WHO clinical staging. Cytopenia was more common in WHO clinical stage IV disease. Interpretation & conclusions: Lymph node cytology was found to be a useful tool for segregating lymphadenopathy cases for further evaluation and for identification of

  17. Seropositivity of Dengue Antibodies during Pregnancy

    Nor Azlin Mohamed Ismail

    2014-01-01

    Full Text Available Purpose. Malaysia a dengue endemic country with dengue infections in pregnancy on the rise. The present study was aimed at determining dengue seroprevalence (IgG or IgM during pregnancy and its neonatal transmission in dengue seropositive women. Methods. Maternal with paired cord blood samples were tested for dengue antibodies (IgG and IgM using an enzyme-linked immunosorbent assay (ELISA. Maternal age, parity, occupation, ethnic group, and gestational age were recorded. Data on neonatal Apgar score and admissions to the Neonatal Intensive Care Unit (NICU were analyzed. Results. Out of 358 women recruited, about 128 (35.8% patients were seropositive. Twelve patients (3.4% had recent infections (IgM positive and another 116 women (32.4% were with past infections (IgG positive. All babies born to seropositive mothers had positive IgG paired cord blood; however, no IgM seropositivity was observed. All neonates had good Apgar scores and did not require NICU admission. Conclusion. In this study, 35.8% pregnant women were found to be dengue seropositive. However, transplacental transfer of IgG antibodies had no detrimental effect on the neonatal outcomes.

  18. Clinical observation of radiation urinary bladder disease

    Jin Yuke; Liu Libo; Zhang Haiying; Liang Shuo; Chen Dawei; Wu Zhenfeng; Dong Lihua; Lu Xuejun

    2004-01-01

    Objective: Clinical characteristic, diagnosis and treatment of radiation urinary bladder disease induced by radiation therapy for cancers in the pelvis were inquired into for providing diagnostic basis. Methods: Statistical analysis for the clinical cases was carried out. Results: The incidence of radiation bladder diseases induced by radiation therapy of cervix cancer are about 0.8%-2.96%, with an average of 2.14%. Radiation bladder disease is divided into acute radiation cystitis, chronic radiation cystitis and radiation vesical fistula. Chronic radiation cystitis is seen most often in the clinic and its main clinical symptom is painless macroscopic hematuria, which is again subdivided into slight and severe degrees. Diagnosis should include history of exposure to radiation, which dose exceed the dose threshold, and typical clinical characteristics. Conclusion: The characteristics, types and diagnostic basis of radiation urinary bladder disease analyzed in this study can provide the reference for drawing up diagnostic standard

  19. Clinical presentation of juvenile Huntington disease

    Ruocco Heloísa H.

    2006-01-01

    Full Text Available OBJECTIVE: To describe the clinical presentation a group of patients with juvenile onset of Huntington disease. METHOD: All patients were interviewed following a structured clinical questioner. Patients were genotyped for the trinucleotide cytosine-adenine-guanine (CAG repeat in the Huntington Disease gene. High resolution brain MRI was performed in all patients. RESULTS: We identified 4 patients with juvenile onset of disease among 50 patients with Huntington disease followed prospectively in our Neurogenetics clinic. Age at onset varied from 3 to 13 years, there were 2 boys, and 3 patients had a paternal inheritance of the disease. Expanded Huntington disease allele sizes varied from 41 to 69 trinucleotide repeats. The early onset patients presented with rigidity, bradykinesia, dystonia, dysarthria, seizures and ataxia. MRI showed severe volume loss of caudate and putamen nuclei (p=0.001 and reduced cerebral and cerebellum volumes (p=0.01. CONCLUSION: 8% of Huntington disease patients seen in our clinic had juvenile onset of the disease. They did not present with typical chorea as seen in adult onset Huntington disease. There was a predominance of rigidity and bradykinesia. Two other important clinical features were seizures and ataxia, which related with the imaging findings of early cortical atrophy and cerebellum volume loss.

  20. Seropositivity diagnosis for hantavirus in Jataí, Goiás State, Brazil

    Marcos Lázaro Moreli

    Full Text Available Abstract INTRODUCTION: Emerging diseases are of great interest, especially those associated with high mortality rates such as hantaviruses. We aimed to conduct a seroepidemiological survey to determine the levels of hantavirus infection. METHODS: In-house enzyme-linked immunosorbent assay (ELISA was used to detect specific antibodies. RESULTS: Of the 429 samples collected, seropositivity of 3.9% to anti-hantavirus immunoglobulin G (IgG was observed (CI 95%: 2.3-5.7. Moreover, in three cases, immunoglobulin M (IgM was detected, of which two were diagnosed as hantavirus cardiopulmonary syndrome (HCPS. CONCLUSIONS: Our data indicate the considerable occurrence of previous hantavirus infections, highlighting occurrences from sub-clinical cases to HCPS.

  1. Occurrence of oxidative stress in dairy cows seropositives for Brucella abortus.

    Perin, Géssica; Fávero, Juscivete F; Severo, Diego R T; Silva, Anielen D; Machado, Gustavo; Araújo, Hugo L; Lilenbaum, Walter; Morsch, Vera M; Schetinger, Maria Rosa C; Jordão, Ricardo S; Stefani, Lenita M; Bottari, Nathieli B; Da Silva, Aleksandro S

    2017-09-01

    Bovine brucellosis is an important zoonotic disease caused by the bacterium Brucella abortus that leads to economic losses due to animal discard and commercial restrictions. Since positive animals for brucellosis are culled, little is known about the pathogenesis of this disease. Therefore, the aims of this study were to evaluate possible changes in the activity of deaminase adenosine (ADA) and the oxidative stress in cows seropositives for brucellosis (Experiment I), and to evaluate the seroprevalence of B. abortus in dairy cows from the Western state of Santa Catarina, Southern Brazil (Experiment II). The Experiment I evaluated 20 pregnant cows: ten seropositives for B. abortus and ten seronegatives that were used as controls. The ADA activity and markers of oxidative stress (TBARS, catalase (CAT) and superoxide dismutase (SOD)) were evaluated in these animals. A reduction in the activity of ADA and catalase enzymes in seropositive animals was observed (p cows infected by B. abortus (p cows of 69 herds. The serodiagnosis was performed using two tests: buffered acidified antigen and 2-mercaptoethanol. However, none of the serum samples were positive for B. abortus. Although we did not find seropositive animals for brucellosis in our study, the disease still requires continued surveillance, due to its economic impact, and to the oxidative stress caused by it, which may have contributed to cases of abortion in three seropositive cows (Experiment I) in the final third of the gestation. Copyright © 2017 Elsevier Ltd. All rights reserved.

  2. Clinical and Histologic Mimickers of Celiac Disease.

    Kamboj, Amrit K; Oxentenko, Amy S

    2017-08-17

    Celiac disease is an autoimmune disorder of the small bowel, classically associated with diarrhea, abdominal pain, and malabsorption. The diagnosis of celiac disease is made when there are compatible clinical features, supportive serologic markers, representative histology from the small bowel, and response to a gluten-free diet. Histologic findings associated with celiac disease include intraepithelial lymphocytosis, crypt hyperplasia, villous atrophy, and a chronic inflammatory cell infiltrate in the lamina propria. It is important to recognize and diagnose celiac disease, as strict adherence to a gluten-free diet can lead to resolution of clinical and histologic manifestations of the disease. However, many other entities can present with clinical and/or histologic features of celiac disease. In this review article, we highlight key clinical and histologic mimickers of celiac disease. The evaluation of a patient with serologically negative enteropathy necessitates a carefully elicited history and detailed review by a pathologist. Medications can mimic celiac disease and should be considered in all patients with a serologically negative enteropathy. Many mimickers of celiac disease have clues to the underlying diagnosis, and many have a targeted therapy. It is necessary to provide patients with a correct diagnosis rather than subject them to a lifetime of an unnecessary gluten-free diet.

  3. Risk Factors for Enterovirus A71 Seropositivity in Rural Indigenous Populations in West Malaysia.

    NikNadia, Nmn; Sam, I-Ching; Khaidir, Nasibah; Ngui, Romano; Lim, Yvonne A L; Goh, Xiang Ting; Choy, Seow Huey; Chan, Yoke Fun

    2016-01-01

    Enterovirus A71 (EV-A71), which is transmitted by the fecal-oral route, causes hand, foot and mouth disease and, rarely, severe neurological complications. In Malaysia, the indigenous rural community (Orang Asli) has a high prevalence of parasitic diseases due to poor sanitation, water supply and hygiene practices. This cross-sectional study compared the seroepidemiology of EV-A71 among rural Orang Asli and urban Kuala Lumpur populations in West Malaysia, and determined the risk factors associated with EV-A71 seropositivity in rural Orang Asli. Seropositive rates were determined by neutralization assay. EV-A71 seropositivity was strongly associated with increasing age in both populations. Rural Orang Asli children ≤12 years had significantly higher EV-A71 seropositivity rates than urban Kuala Lumpur children (95.5% vs 57.6%, P water (adjusted OR 6.2, 95% CI 2.3-16.6, P water may reduce the risk of EV-A71 infection. With significantly higher EV-A71 seropositive rates, younger rural children should be a priority target for future vaccination programs in Malaysia.

  4. Cytomegalovirus seropositivity is associated with herpes zoster

    Ogunjimi, Benson; Hens, Niel; Pebody, Richard; Jansens, Hilde; Seale, Holly; Quinlivan, Mark; Theeten, Heidi; Goossens, Herman; Breuer, Judy; Beutels, Philippe

    2015-01-01

    Herpes zoster (HZ) is caused by VZV reactivation that is facilitated by a declined immunity against varicella-zoster virus (VZV), but also occurs in immunocompetent individuals. Cytomegalovirus (CMV) infection is associated with immunosenescence meaning that VZV-specific T-cells could be less responsive. This study aimed to determine whether CMV infection could be a risk factor for the development of HZ. CMV IgG serostatus was determined in stored serum samples from previously prospectively recruited ambulatory adult HZ patients in the UK (N = 223) in order to compare the results with those from UK population samples (N = 1545) by means of a logistic regression (controlling for age and gender). Furthermore, we compared the UK population CMV seroprevalence with those from population samples from other countries (from Belgium (N1 = 1741, N2 = 576), USA (N = 5572) and Australia (N = 2080)). Furthermore, CMV IgG titers could be compared between UK HZ patients and Belgium N2 population samples because the same experimental set-up for analysis was used. We found UK ambulatory HZ patients to have a higher CMV seroprevalence than UK population samples (OR 1.56 [1.11 2.19]). CMV IgG seropositivity was a significant risk factor for HZ in the UK (OR 3.06 [1.32 7.04]. Furthermore, high CMV IgG titers (exceeding the upper threshold) were less abundant in CMV-seropositive Belgian N2 population samples than in CMV-seropositive UK HZ patients (OR 0.51 [0.31 0.82]. We found CMV-seroprevalence to increase faster with age in the UK than in other countries (P < 0.05). We conclude that CMV IgG seropositivity is associated with HZ. This finding could add to the growing list of risk factors for HZ. PMID:25905443

  5. Candida Species Prevalence Profile in HIV Seropositive Patients from a Major Tertiary Care Hospital in New Delhi, India

    Monika Maheshwari

    2016-01-01

    Full Text Available Candida is a common opportunistic pathogen during the course of human immunodeficiency virus (HIV disease progression. Changes in the clinical severity of candidiasis and the Candida species prevalence profile may be a reflection of immunological changes in HIV positive patients. The aim of this study was to document the changing pattern of Candida species prevalence profile in HIV seropositive patients from a tertiary care hospital in North India. One hundred and twenty HIV seropositive subjects were recruited for Candida microbial screening. Clinical specimens including blood, oral swabs, expectorated or induced sputum/bronchoalveolar lavage specimens, and urine were collected depending on the patient’s symptoms. A total of 128 Candida isolates were obtained from 88 cases and 7 different Candida species were identified. C. albicans (50% was the most common species isolated followed by C. glabrata (17% and C. dubliniensis (12.5%. Other species isolated were C. parapsilosis (7.8%, C. krusei, C. tropicalis (4.6% each, and C. kefyr (3%. Strong clinical suspicion along with optimal sampling of an accurate diagnosis of Candida species involved would go a long way in decreasing the morbidity associated with non-albicans Candida species.

  6. Lyme disease: clinical diagnosis and treatment

    Hatchette, TF; Davis, I; Johnston, BL

    2014-01-01

    Background Lyme disease is an emerging zoonotic infection in Canada. As the Ixodes tick expands its range, more Canadians will be exposed to Borrelia burgdorferi, the bacterium that causes Lyme disease. Objective To review the clinical diagnosis and treatment of Lyme disease for front-line clinicians. Methods A literature search using PubMed and restricted to articles published in English between 1977 and 2014. Results Individuals in Lyme-endemic areas are at greatest risk, but not all tick bites transmit Lyme disease. The diagnosis is predominantly clinical. Patients with Lyme disease may present with early disease that is characterized by a “bull’s eye rash”, fever and myalgias or with early disseminated disease that can manifest with arthralgias, cardiac conduction abnormalities or neurologic symptoms. Late Lyme disease in North America typically manifests with oligoarticular arthritis but can present with a subacute encephalopathy. Antibiotic treatment is effective against Lyme disease and works best when given early in the infection. Prophylaxis with doxycyline may be indicated in certain circumstances. While a minority of patients may have persistent symptoms, evidence does not demonstrate that prolonged courses of antibiotics improve outcome. Conclusion Clinicians need to be aware of the signs and symptoms of Lyme disease. Knowing the regions where Borrelia infection is endemic in North America is important for recognizing patients at risk and informing the need for treatment. PMID:29769842

  7. Toxoplasma gondii seroprevalence in pet cats in Norway and risk factors for seropositivity.

    Sævik, Bente Kristin; Krontveit, Randi Ingebjørg; Eggen, Kristine P; Malmberg, Nina; Thoresen, Stein I; Prestrud, Kristin W

    2015-12-01

    The aims of the study were to estimate Toxoplasma gondii seroprevalence in pet cats in Norway and to evaluate risk factors for seropositivity. Additionally, serum biochemistry and haematological variables for T gondii seropositive and seronegative cats were compared. A convenience sample of surplus sera submitted to the Central Laboratory, Norwegian University of Life Sciences, was collected. The samples were from healthy cats and cats with a variety of diseases. Analyses for IgG antibodies to T gondii were performed with a commercial direct agglutination test, with 1:40 as the threshold value. For risk factor analysis a logistic regression model of the relationship between predictors and the outcome was applied. One hundred and ninety-six of 478 cats were seropositive for T gondii, and the estimated seroprevalence in the study sample was 41.0% (95% confidence interval 36.6-45.4). Compared with domestic cats, pedigree cats had reduced risk for Toxoplasma seropositivity (odds ratio [OR] 0.42). Males had increased risk (OR 1.63) compared with females. The effect of age was highly significant, and an increase in the cats' age across the interquartile range (IQR; 52-160 months/4-13 years of age) doubled the risk of Toxoplasma seropositivity (OR 2.11). The risk for Toxoplasma seropositivity among cats living in Oslo was significantly reduced (OR 0.51) when compared with the rest of Norway. Pet cats in Norway appear to be commonly exposed to T gondii. Signalment and geographical region influenced the odds of Toxoplasma seropositivity, whereas health status did not. © ISFM and AAFP 2015.

  8. Clinical presentation of adult coeliac disease.

    Tajuddin, T

    2012-02-01

    The mode of presentation of coeliac disease has been changing to more atypical or silent disease. Few studies described the clinical presentation of adult coeliac disease in Ireland in recent years. We retrospectively collected the clinical data for all patients who had a diagnosis of coeliac disease made in our centre between January 07 and December 08. Forty seven adults, predominantly females (n = 30), had a confirmed diagnosis of coeliac disease made during the study period. In our patient cohort, the presenting symptom was diarrhoea in 19 (40%) patients, while 16 patients (34%) did not have any G.I. symptoms, 10 (21%) presented with anaemia. Females presented at a significantly younger age compared to males, with median ages at diagnosis of 44.5 and 57 years, respectively (p = 0.04). Females also presented more commonly with non G.I. symptoms (p = 0.07). The reasons behind this gender difference need further study.

  9. [Lyme disease--clinical manifestations and treatment].

    Stock, Ingo

    2016-05-01

    Lyme disease (Lyme borreliosis) is a systemic infectious disease that can present in a variety of clinical manifestations. The disease is caused by a group of spirochaetes--Borrelia burgdorferi sensu lato or Lyme borrelia--that are transmitted to humans by the bite of Ixodes ticks. Lyme disease is the most common arthropode-borne infectious disease in many European countries including Germany. Early localized infection is typically manifested by an erythema migrans skin lesion, in rarer cases as a borrelial lymphocytoma. The most common early disseminated manifestation is (early) neuroborreliosis. In adults, neuroborreliosis appears typically as meningoradiculoneuritis. Neuroborreliosis in children, however, is typically manifested by meningitis. In addition, multiple erythema migrans lesions and Lyme carditis occur relatively frequently. The most common manifestation oflate Lyme disease is Lyme arthritis. Early manifestations (and usually also late manifestations) of Lyme disease can be treated successfully by application of suitable antibacterial agents. For the treatment of Lyme disease, doxycycline, certain penicillins such as amoxicillin and some cephalosporins (ceftriaxone, cefotaxime, cefuroxime axetil) are recommended in current guidelines. A major challenge is the treatment of chronic, non-specific disorders, i. e., posttreatment Lyme disease syndrome and "chronic Lyme disease". Prevention of Lyme disease is mainly accomplished by protecting against tick bites. Prophylactic administration of doxycycline after tick bites is generally not recommended in Germany. There is no vaccine available for human beings.

  10. Assessment of local carotid stiffness in seronegative and seropositive rheumatoid arthritis.

    Aslan, Abdullah Nabi; Şirin Özcan, Ayşe Nur; Erten, Şükran; Alsancak, Yakup; Durmaz, Tahir

    2017-10-01

    Rheumatoid arthritis (RA) is a chronic, inflammatory disease associated with increased risk of cardiovascular (CV) disease. Arterial stiffness (AS) is an independent predictor of CV events. This study aimed to analyse local carotid AS parameters in seronegative and seropositive RA patients. Of 347 consecutive RA patients, we selected specifically those who were free of established CV diseases and risk factors. As a result, 140 patients (126 women, 52.2 ± 10 years) and 140 healthy controls (122 women, 52.7 ± 8.0 years) were enrolled into this study. The common carotid AS was evaluated using radio frequency echo-tracking system to determine the local carotid pulse wave velocity (cPWV) and carotid intima-media thickness (cIMT). Based on rheumatoid factor (RF) and/or anti-citrullinated protein antibody (ACPA) positivity, RA patients were categorized into seronegative and seropositive subgroups. Carotid PWV was determined to be significantly higher in all patients and subgroups than controls (p < .001 for all). Although cIMT was similar between the patients, controls and seropositive subgroup, seronegative patients had significantly higher cIMT compared to controls (p = .035) and seropositive group (p = .010). Moreover, a significant positive correlation was found between cPWV and age (r: 0.603, p < .001), ESR (r: 0.297, p = .004), ACPA (r: 0.346, p = .001) and cIMT (r: 0.290, p = .005) in seropositive patients. RA per se is sufficient to cause arteriosclerosis in the absence of classical CV risk factors. However, arterial hypertrophy is only increased in seronegative patients but not in seropositive group.

  11. Adenovirus-36 Seropositivity and Its Relation with Obesity and Metabolic Profile in Children

    Isela Parra-Rojas

    2013-01-01

    Full Text Available The human adenovirus 36 (Ad-36 is causally and correlatively associated in animals and humans, respectively, with increased adiposity and altered metabolic profile. In previous studies, the relationship between Ad-36 seropositivity with obesity was established in adults and children. We evaluated the association of positive antibodies to Ad-36 with obesity and metabolic profile in Mexican children. Seventy-five children with normal-weight and 82 with obesity were studied in this research. All children had a clinic assessment which included weight, height, body circumferences, and skinfold thickness. Laboratory analyzes included triglycerides, total cholesterol, high-density lipoprotein, low-density lipoprotein, and glucose and insulin levels. An enzyme-linked immunosorbent assay (ELISA was used to determine the antibodies to Ad-36 in the serum samples. The overall Ad-36 seroprevalence was 73.9%. Ad-36 seropositivity had a higher prevalence in obese children than in normal weight group (58.6 versus 41.4%, P=0.007. Ad-36 seropositivity was associated with obesity (OR=2.66, P=0.01 and high-density lipoprotein <40 mg/dL (OR=2.85, P=0.03. The Ad-36 seropositive group had greater risk of 4 metabolic abnormalities compared with those children without none alteration. In summary, Ad-36 seropositivity was associated with obesity and low HDL-c levels in the sample of children studied.

  12. Paediatric Inflammatory Bowel Disease: Clinical Presentation and Disease Location.

    Aziz, Danish Abdul; Moin, Maryum; Majeed, Atif; Sadiq, Kamran; Biloo, Abdul Gaffar

    2017-01-01

    To determine different clinical presentationsand disease location demarcatedby upper and lower gastrointestinal endoscopyand relevant histopathologyin children diagnosed with inflammatory bowel disease (IBD). This is 5 years (2010 to 2015) retrospective studyconducted at the Aga Khan University Hospitalenrolling65admitted children between 6 months to 15years from either gender, diagnosed with IBD on clinical presentation, endoscopy and biopsy. Different clinical presentations at the time of diagnosis were noted in different categories of the disease. All patients underwent upper and lower (up to the terminal ileum) endoscopy with multiple punch biopsies and histologic assessment of mucosal specimens. All endoscopies were done by paediatric gastroenterologists at endoscopy suite of the hospital and all specimens were reported by the pathology department. ESPGHAN revised criteria for the diagnosis of inflammatory bowel disease in children and an adolescent was used to standardize our diagnosis. Extent of disease on endoscopy and relevant histopathology of the biopsy samples were noted at the time of diagnosis. Data was summarized using mean, standard deviation, numbers and percentages for different variables. Total 56 children were enrolled according to inclusion criteria. There were 34children (61.53%) diagnosed with ulcerative colitis (UC), 10 patients (16.92%) had Crohn'sDisease (CD) and 11 (21.53%) patients were labeled as Indeterminate colitis (IC). Mean age at onset of symptoms was10.03±2.44 and mean age at diagnosis was11.10±2.36. Abdominal pain (80%) and chronic diarrhea (70%) were common symptoms in CD whereas bloody diarrhea (79.41%) and rectal bleeding(64.70%)were common presentation in UC. Patients diagnosed with indeterminate colitis(IC) had similar clinical features as in UC patients. Only 7% patients had some extra-intestinal features in the form of joint pain and/or uveitis. Aspartate aminotransferase level (95.18 ±12.89) was relatively high in

  13. Bilingualism delays clinical manifestation of Alzheimer's disease

    Woumans, Evy; Santens, Patrick; Sieben, Anne; Versijpt, Jan; Stevens, Michaël; Duyck, Wouter

    2015-01-01

    The current study investigated the effects of bilingualism on the clinical manifestation of Alzheimer's disease (AD) in a European sample of patients. We assessed all incoming AD patients in two university hospitals within a specified timeframe. Sixty-nine monolinguals and 65 bilinguals diagnosed with probable AD were compared for time of clinical AD manifestation and diagnosis. The influence of other potentially interacting variables was also examined. Results indicated a significant delay f...

  14. Seroprevalence of Trypanosoma cruzi in rural Ecuador and clustering of seropositivity within households.

    Black, Carla L; Ocaña-Mayorga, Sofía; Riner, Diana K; Costales, Jaime A; Lascano, Mauricio S; Arcos-Terán, Laura; Preisser, John S; Seed, J Richard; Grijalva, Mario J

    2009-12-01

    We performed a cross-sectional study of Trypanosoma cruzi seroprevalence in 14 communities in three provinces of Ecuador and estimated the magnitude of the association of seropositive individuals within households. A total of 3,286 subjects from 997 households were included. Seroprevalence was 5.7%, 1.0%, and 3.6% in subjects in the Manabí, Guayas, and Loja provinces, respectively. Seroprevalence increased with increasing age in Manabí and Guayas, whereas in Loja, the highest prevalence occurred in children Loja, the odds of seropositivity were more than two times greater for an individual living in a household with another seropositive person. Our results indicate that transmission of T. cruzi is ongoing in Ecuador, although intensity of transmission and mechanisms of interaction between humans and the insect vectors of disease vary between geographic regions.

  15. Comparison of seropositivity of HCV between oral lichen planus and healthy control group in Hamedan province (west of Iran

    Ahmad Reza Mobaien

    2011-10-01

    Full Text Available Background: Lichen planus is an idiopathic inflammatory disease of the skin, nail, hair and mucous membranes. Oral lichen planus (LP is a chronic inflammatory condition that affects the oral mucous membranes with a variety of clinical presentations. Various etiologies include HCV suggested for LP, and the aim of this study was comparison of seropositivity of HCV in LP patients and control group. Methods: All oral LP patients that were referred to dermatology clinic of farshchian hospitalwere entered in the study. Five cc of clot blood was taken from each patient and tested for anti-HCVand when anti-HCV tested positive another 2cc clot bloodwas taken for HCV-Rt-PCR test. The results were analyzed with SPSS 16. Results: This prospective cross-sectional study was conducted on 30 oral lichen planus patients [males 13(43.3% females 17(56.7%] with mean ages of 46±13.7years and 60 healthy individual [males 26(43.3% females 34(56.7%]. There was no oral lichen planus patients who had anti-HCV positive whiles 2 males(3.3% of healthy group had anti-HCV positive which was confirmed by HCV-Rt-PCR. Conclusions: This study showed that there is no correlation between seropositivity of HCV and oral lichen planus in our patients in the west of Iran.

  16. A clinical approach to Lyme disease.

    Nadelman, R B; Wormser, G P

    1990-05-01

    Lyme disease (also known as Lyme borreliosis) is an emerging, newly described infectious disease with diverse clinical manifestations. The disease is caused by the spirochetal agent Borrelia burgdorferi, which is transmitted to humans by the bite of certain species of Ixodes ticks harboring the organism. The most readily identifiable clinical feature is the distinctive skin lesion, erythema migrans. If recently infected patients go untreated, approximately 15% will develop neurologic conditions (most commonly facial nerve palsy), 8% will develop myocarditis (typically with heart block), and 60% will develop migratory mono- or pauci-articular arthritis. Diagnosis depends on clinical suspicion, recognition of the characteristic signs and symptoms, and appropriate testing for antibody to B. burgdorferi. Serology for Lyme disease, although in need of better standardization, is most useful in diagnosing patients with manifestations of Lyme disease other than erythema migrans. All manifestations of Lyme disease are potentially treatable with either a beta-lactam antibiotic (for instance penicillin, amoxicillin, or ceftriaxone) or a tetracycline preparation. However, the optimal antimicrobial regimen, including choice of drug, drug dose, route of administration, and length of therapy, is unknown. Other important areas for future research include Ixodes biology and control, improved laboratory tests for diagnosis and for assessing response to therapy, and vaccine development.

  17. Clinical pattern of heart diseases in children

    Ejaz, M.S.; Billoo, A.G.

    2000-01-01

    This study was done to determine various causes and clinical presentation of heart disease in children. It was a prospective hospital study conducted in Department of Pediatrics Civil Hospital, Karachi from August 1995 to February 1996. In this study, 70 patients of heart disease upto 12 years of age were inducted. There were 33 (47.14%) cases of congenital heart diseases and 37 (52.85%) cases of acquired heart diseases. The age distribution showed that heart disease was more frequent between 0-11 months of age (41.42%). Congenital heart diseases were also frequent between 0-11 months (28.57%). On the other hand acquired heart diseases were more common between 6-12 years (22.85%). In this study the males were predominantly involved, the male to female ratio was 1.05:1. In congenital heart disease it was 1.3:1 and in acquired heart diseases it was 0.85:1. Ventricular septal defect was the commonest congenital lesion reported (20%). Rheumatic fever and viral myocarditis were two frequently occurring acquired heart-diseases 17.14% each. The common presentation of heart diseases were respiratory distress (94.28%), fever (90%), feeding difficulty (57.14%) and failure to thrive (34.28%). In case of rheumatic fever, chorea was present in 8.57%, arthritis in 11.42% and S/C nodules (2.85%) cases respectively. The early management of the problem may help in decreasing morbidity and mortality due to these disease in children. Prenatal detection of congenital cardiac lesions by fetal echocardiography in high risk pregnancies, early intervention in neonatal period and counseling of the parents may help in prevention of congenital heart diseases in children. Primary prevention of rheumatic fever can be achieved by early diagnosis and treatment of streptococcal throat infection. (author)

  18. Clinical heterogeneity in newly diagnosed Parkinson's disease

    Post, Bart; Speelman, Johannes D.; de Haan, Rob J.

    2008-01-01

    OBJECTIVE: To determine clinical heterogeneity in newly diagnosed Parkinson's disease using cluster analysis and to describe the subgroups in terms of impairment, disability, perceived quality of life, and use of dopaminergic therapy. METHODS: We conducted a k-means cluster analysis in a prospective

  19. Dopaminreceptorscintigraphy in Parkinson's disease - Clinical correlation

    Riklund Aahlstroem, K.E.; Hietala, S.-O.; Johansson, F.

    2002-01-01

    Parkinson's disease is a severe, progressive neuro degenerative disorder which is characterised by a degeneration of the dopamine containing cells and loss of dopamine transporters (DA) in substantia nigra. Earlier 123 I-β-CIT SPECT studies have demonstrated this loss of DA content in Parkinson's disease. Recently a new radioligand 123 I-FP-CIT, with faster kinetics than b-CIT became available for imaging of the DA transporter. The applicability of this radioligand was tested in a large clinical material with early and advanced Parkinson's disease using a one day protocol. 123 I-FP-CIT uptake was decreased in patients with Parkinson's disease and this was seen three hours after injection of the radioligand. In the Parkinson's disease group the uptake in the putamen was reduced more than in the caudate nucleus. Specific to non-specific striatal uptake ratios correlated with the Hoehn and Yahr stage. It appeared that 123 I-FP-CIT SPECT allows a significant discrimination between patients with Parkinson's disease and other movement disorders. The scintigraphic observations were correlated to clinical findings. The results will be presented and discussed

  20. Comparative analysis of seronegative and seropositive rheumatoid arthritis regarding some epidemiological and anamnestic characteristics.

    Sahatçiu-Meka, Vjollca; Izairi, Remzi; Rexhepi, Sylejman; Manxhuka-Kerliu, Suzana

    2007-01-01

    Classifying patients into two subsets of the disease--seronegative RA and seropositive RA--has been the subject of many studies which aim to clarify this phenomenon--without any conclusive or acceptable answer so far. The aim of this prospective study was to establish a scientific comparative analysis between seronegative and seropositive rheumatoid arthritis (RA) regarding some epidemiological and anamnestic characteristics. The studied group consisted of seronegative patients with titers lower than 1:64 as defined by Rose-Waaler test, while the control group consisted of seropositive patients with titers of 1:64 or higher. All patients belonged to 2nd and 3rd functional class according to the ARA criteria, were between 25-60 years of age (Xb=49.96), with disease duration between 1-27 years (Xb=6.41). Education, residence, economic and living conditions did not show any significant statistical difference regarding serostatus. Familial clustering of RA confirmed higher susceptibility in the seropositive group (chi2=7.02; p<0.01). In both subsets banal diseases, psychic and physical trauma, weakness, and numbness of hands and legs dominated, without any statistical differenceregarding serostatus. Some differences between groups regarding sex were noticed, but were not statistically significant, except regarding physical trauma, which was more present in seronegative females (chi2=8.05; p<0.01).

  1. Comparison of prevalence factors in horses with and without seropositivity to Neospora hughesi and/or Sarcocystis neurona.

    Pusterla, Nicola; Tamez-Trevino, Eva; White, Alexandria; Vangeem, Joshua; Packham, Andrea; Conrad, Patricia A; Kass, Philip

    2014-05-01

    Equine protozoal myeloencephalitis is a commonly diagnosed neurological disease of horses in North America and is caused by infection with Sarcocystis neurona or Neospora hughesi. The aim of this study was to compare prevalence factors among horses seropositive or seronegative to N. hughesi and/or S. neurona. A total of 3123 submissions were included in the study, with horses originating from 49 States. Thirty-eight animals from 21 States tested seropositive for N. hughesi only, 840 horses from 40 States were seropositive for S. neurona only, 25 horses from 14 States were seropositive for both protozoa, and 2220 horses from 49 States tested seronegative for both parasites. Significant associations were found between geographical location (State), month of submission, breed and serological status. Copyright © 2014 Elsevier Ltd. All rights reserved.

  2. The Psychosocial Integration of Seropositive Persons

    Matei Raluca

    2017-05-01

    Full Text Available We can assume without fail that the psychosocial implications in case of HIV/AIDS are as overwhelming as the medical aspects. For the positive person as well as for those who assist the seropositive people, the uncertainty frames the medical prognosis. All the effects that occur are entirely funded: the loss of self-trust as well as mistrusting others, stigmatization, isolation, getting abandoned by the family, friends, neighbors and school mates or coworkers. The current study aims to evaluate this aspect, the fact that the living environment of the seropositive person does not lead to significant differences as far as adjusting to HIV/AIDS diagnosis is concerned, but the adjusting differences are more related to the personality structure of the infected person, to the way this person identifies strategies of coping with the new situations he / she has to deal with (cognitive, emotional and behavioral strategies. In the applicative part of the study, we analyze the adjustment to the diagnosis, as well as the adherence to the treatment, by applying certain specific work instruments.

  3. Delayed LGI1 seropositivity in voltage-gated potassium channel (VGKC)-complex antibody limbic encephalitis.

    Sweeney, Michael; Galli, Jonathan; McNally, Scott; Tebo, Anne; Haven, Thomas; Thulin, Perla; Clardy, Stacey L

    2017-04-20

    We utilise a clinical case to highlight why exclusion of voltage-gated potassium channel (VGKC)-complex autoantibody testing in serological evaluation of patients may delay or miss the diagnosis. A 68-year-old man presented with increasing involuntary movements consistent with faciobrachial dystonic seizures (FBDS). Initial evaluation demonstrated VGKC antibody seropositivity with leucine-rich glioma-inactivated 1 (LGI1) and contactin-associated protein-like 2 (CASPR2) seronegativity. Aggressive immunotherapy with methylprednisolone and plasmapheresis was started early in the course of his presentation. Following treatment with immunotherapy, the patient demonstrated clinical improvement. Repeat serum evaluation 4 months posthospitalisation remained seropositive for VGKC-complex antibodies, with development of LGI1 autoantibody seropositivity. VGKC-complex and LGI1 antibodies remained positive 12 months posthospitalisation. Our findings suggest that clinical symptoms can predate the detection of the antibody. We conclude that when suspicion for autoimmune encephalitis is high in the setting of VGKC autoantibody positivity, regardless of LGI1 or CASPR2 seropositivity, early immunotherapy and repeat testing should be considered. 2017 BMJ Publishing Group Ltd.

  4. Recent clinical trials in valvular heart disease.

    Kiss, Daniel; Anwaruddin, Saif

    2017-07-01

    With widespread adoption of transcatheter aortic valve replacement, there has been a change in the approach to management of valvular heart disease. New interest has taken hold in transcatheter therapies for valvular heart disease, as well as research into pathophysiology and progression of disease. Additionally, several key trials have further refined our understanding of surgical management of valvular heart disease. This review will elucidate recent clinical trial data leading to changes in practice. There have been several landmark trials expanding the indications for transcatheter aortic valve replacement. Additionally, although still early, trials are beginning to demonstrate the feasibility and safety of transcatheter mitral valves. Options for transcatheter management of right-sided valvular disease continue to evolve, and these are areas of active investigation. The emergence of novel therapies for valvular heart disease has expanded the management options available, allowing physicians to better individualize treatment of patients with valvular heart disease. This review will focus on the recent (within 2 years) trials in this field of interest.

  5. Meningococcal disease, a clinical and epidemiological review.

    Batista, Rodrigo Siqueira; Gomes, Andréia Patrícia; Dutra Gazineo, Jorge Luiz; Balbino Miguel, Paulo Sérgio; Santana, Luiz Alberto; Oliveira, Lisa; Geller, Mauro

    2017-11-01

    Meningococcal disease is the acute infection caused by Neisseria meningitidis, which has humans as the only natural host. The disease is widespread around the globe and is known for its epidemical potential and high rates of lethality and morbidity. The highest number of cases of the disease is registered in the semi-arid regions of sub-Saharan Africa. In Brazil, it is endemic with occasional outbreaks, epidemics and sporadic cases occurring throughout the year, especially in the winter. The major epidemics of the disease occurred in Brazil in the 70's caused by serogroups A and C. Serogroups B, C and Y represent the majority of cases in Europe, the Americas and Australia. However, there has been a growing increase in serogroup W in some areas. The pathogen transmission happens for respiratory route (droplets) and clinically can lead to meningitis and sepsis (meningococcemia). The treatment is made with antimicrobial and supportive care. For successful prevention, we have some measures like vaccination, chemoprophylaxis and droplets' precautions. In this review, we have described and clarify clinical features of the disease caused by N. meningitidis regarding its relevance for healthcare professionals. Copyright © 2017 Hainan Medical University. Production and hosting by Elsevier B.V. All rights reserved.

  6. Risk of HPV-16/18 Infections and Associated Cervical Abnormalities in Women Seropositive for Naturally Acquired Antibodies: Pooled Analysis Based on Control Arms of Two Large Clinical Trials.

    Safaeian, Mahboobeh; Castellsagué, Xavier; Hildesheim, Allan; Wacholder, Sholom; Schiffman, Mark H; Bozonnat, Marie-Cécile; Baril, Laurence; Rosillon, Dominique

    2018-06-05

    Studies on the role of antibodies produced after infection with human papillomavirus 18 (HPV-18) and subsequent protection from HPV-18 infection have been conflicting, mainly due to inadequate sample size. We pooled data from the control arms of the Costa Rica Vaccine Trial and the PATRICIA trial. Using Poisson regression we compared the risk of newly detected 1-time HPV-18 infection, HPV-18 1-year persistent infection (12MPI), and HPV-18-associated atypical squamous cells of undetermined significance or greater (ASC-US+) lesions between HPV-18 seropositive and seronegative women. High HPV-18 antibodies at enrollment was associated with reduced subsequent HPV-18 detection (P trend = 0.001; relative rate [RR] = 0.69; 95% confidence interval [CI], 0.47-1.01 for the third quartile; RR = 0.63; 95% CI, 0.43-0.94 for the fourth quartile, compared to seronegative). The risk of 12MPI showed a decreasing trend with increasing antibodies (P trend = 0.06; RR = 0.72; 95% CI, 0.29-1.77; RR = 0.42; 95% CI, 0.13-1.32 for the third and fourth quartiles, respectively). Lastly, we observed a significant decreased risk of HPV-18 ASC-US+ with increasing antibody (P trend = 0.01; RR = 0.46; 95% CI, 0.21-0.97 for the fourth quartile). We also observed a significant decreased risk of HPV-16 infection, 12MPI, and ASC-US+ with increasing HPV-16 antibody level. High HPV-18 naturally acquired antibodies were associated with partial protection from future HPV-18 infections and associated lesions. NCT00128661 and NCT001226810.

  7. Clinical course and outcome of early rheumatoid arthritis.

    Papadopoulos, I A; Katsimbri, P; Katsaraki, A; Temekonidis, T; Georgiadis, A; Drosos, A A

    2001-07-01

    We studied whether patients with seropositivity in early rheumatoid arthritis (RA) comprise a different clinical group than those with seronegativity. Four hundred seventeen patients with early RA according to the American College of Rheumatology criteria (disease duration less than 1 year) were retrospectively studied by analysis of demographic, clinical, laboratory, radiological, and therapeutic disease characteristics from the time of diagnosis until the end of the study period (1981 1999) using a data base. There were 248 seropositive patients and 169 seronegative patients with RA. No statistically significant differences were seen between the two groups before commencement of the study period in relation to age of disease onset, male:female ratio, and disease duration. However, seropositive patients showed longer medical follow-up. In addition, at disease onset, seropositive RA patients presented more frequently with symmetrical polyarthritis and small joint involvement than seronegative patients. The seropositive group also had more tender and swollen joints, weaker grip strength, and higher erythrocyte sedimentation and C-reactive protein rates during the follow-up period. In contrast, the seronegative group had less severe radiological findings and greater functional ability at the end of the study. In Greek patients with early RA, rheumatoid factor seems to be a predictor of more severe disease activity.

  8. Nosocomial Legionnaires’ Disease: Clinical and Radiographic Patterns

    Thomas J Marrie

    1992-01-01

    Full Text Available From 1981 to 1991, 55 patients (33 males, 22 females, mean age 58.6 years with nosocomial Legionnaires’ disease were studied. The mortality rate was 64%. One-half of the patients developed nosocomial Legionnaires’ disease within three weeks of admission. A surprising clinical feature was the low rate of findings of consolidation on physical examination, despite the fact that 52% of patients had this finding on chest radiograph. More than one-half of patients had pre-existing lung disease, rendering a radiographic diagnosis of pneumonia due to Legionella pneumophila impossible in 16% of cases despite microbiological confirmation. Nineteen per cent of patients who had blood cultures done had a pathogen other than L pneumophila isolated, suggesting dual infection in at least some of the patients. When the clinical and radiographic findings were combined it was noted that 40% of patients had one of three patterns suggestive of nosocomial Legionnaires’ disease: rapidly progressive pneumonia, lobar opacity and multiple peripheral opacities. However, in 60% of patients there were no distinctive features.

  9. Eco-epidemiological analysis of rickettsial seropositivity in rural areas of Colombia: A multilevel approach.

    Juan C Quintero V

    2017-09-01

    Full Text Available Rickettsiosis is a re-emergent infectious disease without epidemiological surveillance in Colombia. This disease is generally undiagnosed and several deadly outbreaks have been reported in the country in the last decade. The aim of this study is to analyze the eco-epidemiological aspects of rickettsial seropositivity in rural areas of Colombia where outbreaks of the disease were previously reported. A cross-sectional study, which included 597 people living in 246 households from nine hamlets in two municipalities of Colombia, was conducted from November 2015 to January 2016. The survey was conducted to collect sociodemographic and household characteristics (exposure data. Blood samples were collected to determine the rickettsial seropositivity in humans, horses and dogs (IFA, cut-off = 1/128. In addition, infections by rickettsiae were detected in ticks from humans and animals by real-time PCR targeting gltA and ompA genes. Data was analyzed by weighted multilevel clog-log regression model using three levels (person, household and hamlets and rickettsial seropositivity in humans was the main outcome. Overall prevalence of rickettsial seropositivity in humans was 25.62% (95%CI 22.11-29.12. Age in years (PR = 1.01 95%CI 1.01-1.02 and male sex (PR = 1.65 95%CI 1.43-1.90 were risk markers for rickettsial seropositivity. Working outdoors (PR = 1.20 95%CI 1.02-1.41, deforestation and forest fragmentation for agriculture use (PR = 1.75 95%CI 1.51-2.02, opossum in peridomiciliary area (PR = 1.56 95%CI 1.37-1.79 and a high proportion of seropositive domestic animals in the home (PR20-40% vs 40% vs <20% = 3.14 95%CI 2.43-4.04 were associated with rickettsial seropositivity in humans. This study showed the presence of Rickettsia antibodies in human populations and domestic animals. In addition, different species of rickettsiae were detected in ticks collected from humans and animals. Our results highlighted the role of domestic animals as sentinels of

  10. Strongyloides stercoralis infestation in HIV seropositive patients in ...

    Background: A contemporary surge in diarrhoeal illnesses due to parasitic infestations is believed to be a synergy between endemicity and HIV seropositivity. Aim: To determine the prevalence of Strongyloides stercoralis infestation among HIV seropositive patients at the University of Port Harcourt Teaching Hospital.

  11. Comparing Clinical Profiles in Alzheimer's Disease and Parkinson's Disease Dementia

    Martin R. Farlow

    2013-09-01

    Full Text Available Background: Greater understanding of differences in baseline impairment and disease progression in patients with Alzheimer's disease (AD and Parkinson's disease dementia (PDD may improve the interpretation of drug effects and the design of future studies. Methods: This was a retrospective analysis of three randomized, double-blind rivastigmine databases (one in PDD, two in AD. Impairment on the Alzheimer's Disease Assessment Scale-cognitive subscale (ADAS-cog, Alzheimer's Disease Cooperative Study-Activities of Daily Living (ADCS-ADL scale, 10-item Neuropsychiatric Inventory (NPI-10 and the ADCS-Clinical Global Impression of Change (CGIC was compared [standardized difference (Cohen's d, similar if Results: Patients with AD or PDD had similar levels of impairment on the ADAS-cog and NPI-10. Scores on the ADCS-ADL scale (standardized difference = 0.47 and the ADAS-cog memory domain (total, 0.33; items, 0.10-0.58 were higher in AD; PDD patients were more impaired in the language (0.23 and praxis (0.34 domains. AD patients receiving placebo showed greater deterioration on the ADAS-cog (0.14 and improvement on the NPI-10 (0.11 compared with patients with PDD. Conclusion: Differing patterns of impairment occur in AD and PDD.

  12. Comparing clinical profiles in Alzheimer's disease and Parkinson's disease dementia.

    Farlow, Martin R; Schmitt, Frederick; Aarsland, Dag; Grossberg, George T; Somogyi, Monique; Meng, Xiangyi

    2013-01-01

    Greater understanding of differences in baseline impairment and disease progression in patients with Alzheimer's disease (AD) and Parkinson's disease dementia (PDD) may improve the interpretation of drug effects and the design of future studies. This was a retrospective analysis of three randomized, double-blind rivastigmine databases (one in PDD, two in AD). Impairment on the Alzheimer's Disease Assessment Scale-cognitive subscale (ADAS-cog), Alzheimer's Disease Cooperative Study-Activities of Daily Living (ADCS-ADL) scale, 10-item Neuropsychiatric Inventory (NPI-10) and the ADCS-Clinical Global Impression of Change (CGIC) was compared [standardized difference (Cohen's d), similar if <0.1]. Patients with AD or PDD had similar levels of impairment on the ADAS-cog and NPI-10. Scores on the ADCS-ADL scale (standardized difference = 0.47) and the ADAS-cog memory domain (total, 0.33; items, 0.10-0.58) were higher in AD; PDD patients were more impaired in the language (0.23) and praxis (0.34) domains. AD patients receiving placebo showed greater deterioration on the ADAS-cog (0.14) and improvement on the NPI-10 (0.11) compared with patients with PDD. Differing patterns of impairment occur in AD and PDD.

  13. Blood transfusion and iatrogenic risks in Mexico city: anti-Trypanosoma cruzi seroprevalence in 43,048 blood donors, evaluation of parasitemia, and electrocardiogram findings in seropositive

    Nidia Hernández-Becerril

    2005-04-01

    Full Text Available Iatrogenous transmission of Trypanosoma cruziby blood transfusion was suggested as a potential risk by Pellegrino (1949. Seropositive blood donors in Mexico were first reported in 1978, however, limited information is available due to small sampling, the use of heterogeneous serologic assays, and geographically limited studies. A wide survey carried out in 18 out of the 32 states of Mexico, showed a national mean of 1.6% seropositive among 64,969 donors, ranging from 0.2 to 2.8%. In the present study, we have screened 43,048 voluntary blood donors in a period of five years at the Instituto Nacional de Cardiología I. Chávez, a concentration hospital located in Mexico city which serves mainly the metropolitan area and accepts from all over the country. Standardized ELISA and IIF were used to identify seropositive individuals in addition to hemoculture, PCR and standard 12 lead ECG tests that were applied to a group of seropositive patients (29/161. The result showed a seropositivity of 0.37% (161/43,048. From the group of seropositive individuals 40% (12/29 were potential carriers of T. cruzi at the donation time and 5/29 had subclinical ECG abnormalities. Parasitological tests performed in 70 erythrocyte and platelet fractions from seropositive units (70/161 showed negative results. Our findings strongly support T. cruzi screening in the transfusion medicine practice and identify subclinical heart disease among seropositive blood donors.

  14. Clinical neurorestorative progress in Parkinson's disease

    Chen L

    2015-06-01

    Full Text Available Lin Chen,1,2 Hongyun Huang,3–5 Wei-Ming Duan,6 Gengsheng Mao3 1Department of Neurosurgery, Yuquan Hospital, Tsinghua University, 2Department of Neurosurgery, Medical Center, Tsinghua University, 3Department of Neurosurgery, General Hospital of Chinese People's Armed Police Forces, 4Center of Cell Research, Beijing Rehabilitation Hospital of Capital Medical University, 5Beijing Hongtianji Neuroscience Academy, 6Department of Anatomy, Capital Medical University, Beijing, People's Republic of China Abstract: Parkinson’s disease (PD is one of the common neurodegenerative diseases. Besides the symptomatic therapies, the increasing numbers of neurorestorative therapies have shown the potential therapeutic value of reversing the neurodegenerative process and improving the patient's quality of life. Currrently available novel clinical neurorestorative strategies include pharmacological managements (glial cell-line derived neurotrophic factor, selegiline, recombinant human erythropoietin, neuromodulation intervention (deep brain stimulation, repetitive transcranial magnetic stimulation, transcranial direct current stimulation, tissue and cell transplantation (fetal ventral mesencephalic tissue, sympathetic neurons, carotid body cells, bone marrow stromal cells, retinal pigment epithelium cells, gene therapy, and neurorehabilitative therapy. Herein, we briefly review the progress in this field and describe the neurorestorative mechanisms of the above-mentioned therapies for PD. Keywords: Parkinson’s disease, clinical study, neurorestorative treatment, cell transplantation, neuromodulation

  15. Analysis of serum immune markers in seropositive and seronegative rheumatoid arthritis and in high-risk seropositive arthralgia patients

    Chalan, Paulina; Bijzet, Johan; van den Berg, Anke; Kluiver, Joost; Kroesen, Bart-Jan; Boots, Annemieke M. H.; Brouwer, Elisabeth

    2016-01-01

    Presence of autoantibodies precedes development of seropositive rheumatoid arthritis (SP RA) and seropositive arthralgia patients (SAP) are at risk of developing RA. The aims of the study are to identify additional serum immune markers discriminating between SP and seronegative (SN) RA, and markers

  16. Clinical epidemiology and disease burden of nonalcoholic fatty liver disease

    Perumpail, Brandon J; Khan, Muhammad Ali; Yoo, Eric R; Cholankeril, George; Kim, Donghee; Ahmed, Aijaz

    2017-01-01

    Nonalcoholic fatty liver disease (NAFLD) is defined as the presence of hepatic fat accumulation after the exclusion of other causes of hepatic steatosis, including other causes of liver disease, excessive alcohol consumption, and other conditions that may lead to hepatic steatosis. NAFLD encompasses a broad clinical spectrum ranging from nonalcoholic fatty liver to nonalcoholic steatohepatitis (NASH), advanced fibrosis, cirrhosis, and finally hepatocellular carcinoma (HCC). NAFLD is the most common liver disease in the world and NASH may soon become the most common indication for liver transplantation. Ongoing persistence of obesity with increasing rate of diabetes will increase the prevalence of NAFLD, and as this population ages, many will develop cirrhosis and end-stage liver disease. There has been a general increase in the prevalence of NAFLD, with Asia leading the rise, yet the United States is following closely behind with a rising prevalence from 15% in 2005 to 25% within 5 years. NAFLD is commonly associated with metabolic comorbidities, including obesity, type II diabetes, dyslipidemia, and metabolic syndrome. Our understanding of the pathophysiology of NAFLD is constantly evolving. Based on NAFLD subtypes, it has the potential to progress into advanced fibrosis, end-stage liver disease and HCC. The increasing prevalence of NAFLD with advanced fibrosis, is concerning because patients appear to experience higher liver-related and non-liver-related mortality than the general population. The increased morbidity and mortality, healthcare costs and declining health related quality of life associated with NAFLD makes it a formidable disease, and one that requires more in-depth analysis. PMID:29307986

  17. Clinical manifestations and management of Gaucher disease.

    Linari, Silvia; Castaman, Giancarlo

    2015-01-01

    Gaucher disease is a rare multi-systemic metabolic disorder caused by the inherited deficiency of the lysosomal enzyme β-glucocerebrosidase, which leads to the accumulation of its normal substrate, glucocerebroside, in tissue macrophages with damage to haematological, visceral and bone systems. Anaemia, thrombocytopenia, enlargement of liver and/or spleen, skeletal abnormalities (osteopenia, lytic lesions, pathological fractures, chronic bone pain, bone crisis, bone infarcts, osteonecrosis and skeletal deformities) are typical manifestations of the most prevalent form of the disease, the so-called non-neuronopathic type 1. However, severity and coexistence of different symptoms are highly variable. The determination of deficient β-glucocerebrosidase activity in leukocytes or fibroblasts by enzymatic assay is the gold standard for the diagnosis of Gaucher disease. Comprehensive and reproducible evaluation and monitoring of all clinically relevant aspects are fundamental for the effective management of Gaucher disease patients. Enzyme replacement therapy has been shown to be effective in reducing glucocerebroside storage burden and diminishing the deleterious effects caused by its accumulation. Tailored treatment plan for each patient should be directed to symptom relief, general improvement of quality of life, and prevention of irreversible damage.

  18. Clinical disease registries in acute myocardial infarction.

    Ashrafi, Reza; Hussain, Hussain; Brisk, Robert; Boardman, Leanne; Weston, Clive

    2014-06-26

    Disease registries, containing systematic records of cases, have for nearly 100 years been valuable in exploring and understanding various aspects of cardiology. This is particularly true for myocardial infarction, where such registries have provided both epidemiological and clinical information that was not readily available from randomised controlled trials in highly-selected populations. Registries, whether mandated or voluntary, prospective or retrospective in their analysis, have at their core a common study population and common data definitions. In this review we highlight how registries have diversified to offer information on epidemiology, risk modelling, quality assurance/improvement and original research-through data mining, transnational comparisons and the facilitation of enrolment in, and follow-up during registry-based randomised clinical trials.

  19. Smart Technology in Lung Disease Clinical Trials.

    Geller, Nancy L; Kim, Dong-Yun; Tian, Xin

    2016-01-01

    This article describes the use of smart technology by investigators and patients to facilitate lung disease clinical trials and make them less costly and more efficient. By "smart technology" we include various electronic media, such as computer databases, the Internet, and mobile devices. We first describe the use of electronic health records for identifying potential subjects and then discuss electronic informed consent. We give several examples of using the Internet and mobile technology in clinical trials. Interventions have been delivered via the World Wide Web or via mobile devices, and both have been used to collect outcome data. We discuss examples of new electronic devices that recently have been introduced to collect health data. While use of smart technology in clinical trials is an exciting development, comparison with similar interventions applied in a conventional manner is still in its infancy. We discuss advantages and disadvantages of using this omnipresent, powerful tool in clinical trials, as well as directions for future research. Published by Elsevier Inc.

  20. Alzheimer's disease: Cerebrovascular dysfunction, oxidative stress, and advanced clinical therapies

    Marlatt, M.W.; Lucassen, P.J.; Perry, G.; Smith, M.A.; Zhu, X.

    2008-01-01

    Many lines of independent research have provided convergent evidence regarding oxidative stress, cerebrovascular disease, dementia, and Alzheimer's disease (AD). Clinical studies spurred by these findings engage basic and clinical communities with tangible results regarding molecular targets and

  1. Advanced and controlled drug delivery systems in clinical disease management

    Brouwers, JRBJ

    1996-01-01

    Advanced and controlled drug delivery systems are important for clinical disease management. In this review the most important new systems which have reached clinical application are highlighted. Microbiologically controlled drug delivery is important for gastrointestinal diseases like ulcerative

  2. CDKD: a clinical database of kidney diseases

    Singh Sanjay

    2012-04-01

    Full Text Available Abstract Background The main function of the kidneys is to remove waste products and excess water from the blood. Loss of kidney function leads to various health issues, such as anemia, high blood pressure, bone disease, disorders of cholesterol. The main objective of this database system is to store the personal and laboratory investigatory details of patients with kidney disease. The emphasis is on experimental results relevant to quantitative renal physiology, with a particular focus on data relevant for evaluation of parameters in statistical models of renal function. Description Clinical database of kidney diseases (CDKD has been developed with patient confidentiality and data security as a top priority. It can make comparative analysis of one or more parameters of patient’s record and includes the information of about whole range of data including demographics, medical history, laboratory test results, vital signs, personal statistics like age and weight. Conclusions The goal of this database is to make kidney-related physiological data easily available to the scientific community and to maintain & retain patient’s record. As a Web based application it permits physician to see, edit and annotate a patient record from anywhere and anytime while maintaining the confidentiality of the personal record. It also allows statistical analysis of all data.

  3. Long-term MRI findings in neuromyelitis optica: seropositive versus seronegative patients.

    Kıyat-Atamer, A; Ekizoğlu, E; Tüzün, E; Kürtüncü, M; Shugaiv, E; Akman-Demir, G; Eraksoy, M

    2013-05-01

    Neuromyelitis optica (NMO) is a severe demyelinating inflammatory disorder associated with serum antibodies against aquaporin 4 (AQP4-Ab). A significant number of patients with NMO remain seronegative over time. Long-term observational magnetic resonance imaging (MRI) studies of the CNS in patients with NMO are rare or of limited duration. The objective of this study is to determine long-term MRI characteristics of seropositive and seronegative patients, and assess possible overlap with multiple sclerosis (MS). Clinical and radiological characteristics of 28 patients with NMO at onset and of 17 patients after an average follow-up time of 9 years were recorded. Fifty percent of patients were seropositive for AQP4-Ab. Onset and final brain/spinal MRI scans were retrospectively analysed and compared. Significantly more patients in the seronegative group had brain lesions at onset. Spinal lesions of seropositive patients were longer and showed increased cord swelling at onset MRI scans. After the follow-up time the differences between both groups disappeared. Patients in the seropositive group tended to develop brain lesions over time. No patient fulfilled Barkhof's or McDonald's radiological criteria for MS at onset or over time. Brain MRI features show differences between seropositive and seronegative patients at time of onset in NMO, but differences between groups vanish over time. None of the AQP4-negative patients fulfill radiological MS criteria on a long-term basis, suggesting that seronegative NMO constitutes an independent entity. © 2012 The Author(s) European Journal of Neurology © 2012 EFNS.

  4. Polyarthropathy in a cat seropositive for feline synctial-forming virus and feline immunodeficiency virus

    Becker, K.M.; Brown, N.O.; Denardo, G.

    1994-01-01

    A four-year-old, neutered male, domestic shorthair cat presented witha polyarthropathy. Indirect immunofluorescence assays revealed seropositive results for both feline synctial-forming virus (FeSFV) and feline immunodeficiency virus (FIV). Direct relationships between viral infections and polyarthropathy are not confirmed, however, possible correlations are discussed. Mechanisms of lentivirus infections and polyarthropathy in the cat are reviewed in order to theorize a potential relationship among these disease processes

  5. Features of anti-aquaporin 4 antibody-seropositive Chinese patients with neuromyelitis optica spectrum optic neuritis.

    Li, Hongyang; Wang, Yanling; Xu, Quangang; Zhang, Aidi; Zhou, Huanfen; Zhao, Shuo; Kang, Hao; Peng, Chunxia; Cao, Shanshan; Wei, Shihui

    2015-10-01

    The detection of anti-aquaporin-4 autoantibody (AQP-4 Ab) is crucial to detect patients who will develop neuromyelitis optica (NMO); however, there are few studies on the AQP-4 Ab serostatus of patients with neuromyelitis optica spectrum ON. We analyzed the clinical and paraclinical features of neuromyelitis optica spectrum ON patients in China according to the patients' AQP4-Ab serostatus. 125 patients with recurrent and bilateral ON with simultaneous attacks were divided into AQP-4 Ab-seropositive and -seronegative groups. Demographic, clinical, serum autoantibody data, connective tissue disorders (CTDs), visual performance were compared. A Visual Acuity (VA) of less than 0.1 during acute ON attacks occurred more frequently in the seropositive group (p = 0.023); however, there was not a significant difference between groups on VA recovery after the first attack. The seropositive group experienced the worst outcome during the last attack (p = 0.017). Other co-existing autoimmunity antibodies (p optica spectrum ON.

  6. Motivators for Alzheimer's disease clinical trial participation.

    Bardach, Shoshana H; Holmes, Sarah D; Jicha, Gregory A

    2018-02-01

    Alzheimer's disease (AD) research progress is impeded due to participant recruitment challenges. This study seeks to better understand, from the perspective of individuals engaged in clinical trials (CTs), research motivations. Participants, or their caregivers, from AD treatment and prevention CTs were surveyed about research motivators. The 87 respondents had a mean age of 72.2, were predominantly Caucasian, 55.2% were male, and 56.3% had cognitive impairment. An overwhelming majority rated the potential to help themselves or a loved one and the potential to help others in the future as important motivators. Relatively few respondents were motivated by free healthcare, monetary rewards, or to make others happy. Recruitment efforts should focus on the potential benefit for the individual, their loved ones, and others in the future rather than free healthcare or monetary rewards.

  7. A Rare Clinical Presentation of Darier's Disease

    Ferizi, Mybera; Begolli-Gerqari, Antigona; Luzar, Bostjan; Kurshumliu, Fisnik; Ferizi, Mergita

    2013-01-01

    Darier's disease, also known as keratosis follicularis or dyskeratosis follicularis, is a rare disorder of keratinization. It is an autosomal dominant genodermatosis with high penetrance and variable expressivity. Its manifestation appears as hyperkeratotic papules, primarily affecting seborrheic areas on the head, neck, and thorax and less frequently on the oral mucosa. When oral manifestations are present, the palatal and alveolar mucosae are primarily affected. They are usually asymptomatic and are discovered in routine dental examination. Histologically, the lesions are presented as suprabasal clefts in the epithelium with acantholytic and dyskeratotic cells represented by “corps ronds and grains”. This paper reports a case of a 53-year-old woman that was admitted to our clinic with more than 10-year history of keratotic papules, presented on the hands and feet, nose, ears, genitalia, and whitish lesions on palatal mucosae. PMID:23573430

  8. Seroprevalence and Risk Factors for Leptospira Seropositivity in Beef Cattle, Sheep and Deer Farmers in New Zealand.

    Sanhueza, J M; Heuer, C; Wilson, P R; Benschop, J; Collins-Emerson, J M

    2017-08-01

    Leptospirosis is a global zoonosis that in New Zealand affects primarily people occupationally exposed to livestock. The objective of this study was to estimate the seroprevalence of five Leptospira serovars in farmers working on cattle, sheep and deer farms that had the serological status of animals previously assessed and to identify risk factors for farmer seropositivity. A total of 178 farmers from 127 properties participated in the study. Blood samples were tested using the microscopic agglutination test (MAT) for the presence of antibodies to Leptospira. Samples with a MAT titre ≥48 were considered seropositive. Using Bayesian statistical analysis, the median seroprevalence of Leptospira, all serovars combined, was estimated to be 6.6% (95% probability interval (PI) 3.6-10.9%). Risk factors associated with seropositivity were assisting deer or cattle calving, farming deer, having ≥25% of flat terrain and high abundance of wild deer on farm, while high possum abundance on farm was negatively associated with seropositivity. No association was observed between farmer serostatus and previously recorded livestock serology. Leptospira seropositivity was associated with influenza-like illness of farmers (RR = 1.7; 95% PI 1.0-2.5). Assuming a causal relationship, this suggested an annual risk of 1.3% (95% PI 0.0-3.0%) of influenza-like illnesses due to Leptospira infection in the population of farmers. The association between seropositivity and disease can be used to estimate the public health burden of leptospirosis in New Zealand. Identifying and understanding risk factors for Leptospira seropositivity can inform preventive measures, hence contributing to the reduction of leptospirosis incidence in farmers. © 2016 Blackwell Verlag GmbH.

  9. Delayed LGI1 seropositivity in voltage-gated potassium channel (VGKC)-complex antibody limbic encephalitis

    Sweeney, Michael; Galli, Jonathan; McNally, Scott; Tebo, Anne; Haven, Thomas; Thulin, Perla; Clardy, Stacey L

    2017-01-01

    We utilise a clinical case to highlight why exclusion of voltage-gated potassium channel (VGKC)-complex autoantibody testing in serological evaluation of patients may delay or miss the diagnosis. A 68-year-old man presented with increasing involuntary movements consistent with faciobrachial dystonic seizures (FBDS). Initial evaluation demonstrated VGKC antibody seropositivity with leucine-rich glioma-inactivated 1 (LGI1) and contactin-associated protein-like 2 (CASPR2) seronegativity. Aggress...

  10. Hepatitis C: frequency and risk factors associated with sero-positivity among adults in Larkana city

    Shaikh, F.H.; Abro, H.A.; Abbasi, P.A.; Buriro, A.

    2009-01-01

    Viral hepatitis is a major public health problem. Infection with Hepatitis C virus (HCV) leads to chronicity and there are about 170 million people infected with HCV. Up to 70% of chronically infected individuals develop active liver disease. The Objective of the study was to find out the frequency of Anti-HCV seropositivity and risk factors of Hepatitis C Virus (HCV) transmission in people of Larkana city. The study was conducted at Larkana from April 2006 to April 2007. Camps were established in the main general practitioners' clinics. A questionnaire about knowledge of HCV and risk factors of its transmission was administered to subjects. Descriptive statistics were done by SPSS-10. Total 450 cases were enrolled into this study. They were divided into 3 age groups. Eighty-nine (19.8%) cases were in age group 40 years. There were 353 (78.4%) male and 97 (21.6%) female subjects, out of these 450 cases 30 (6.6%) were positive for Anti-HCV. A number of risk factors of HCV transmission were present in these cases. History of therapeutic injections was present in 72 cases and 35 cases used public barber services. History of therapeutic injections and use of barber services for shave were the most frequent risk factors in our study. (author)

  11. Veno occlusive disease: Update on clinical management

    Senzolo, M; Germani, G; Cholongitas, E; Burra, P; Burroughs, AK

    2007-01-01

    Hepatic veno-occlusive disease is a clinical syndrome characterized by hepatomegaly, ascites, weight gain and jaundice, due to sinusoidal congestion which can be caused by alkaloid ingestion, but the most frequent cause is haematopoietic stem cell transplantation (STC) and is also seen after solid organ transplantation. The incidence of veno occlusive disease (VOD) after STC ranges from 0 to 70%, but is decreasing. Survival is good when VOD is a mild form, but when it is severe and associated with an increase of hepatic venous pressure gradient > 20 mmHg, and mortality is about 90%. Prevention remains the best therapeutic strategy, by using non-myeloablative conditioning regimens before STC. Prophylactic administration of ursodeoxycholic acid, being an antioxidant and antiapoptotic agent, can have some benefit in reducing overall mortality. Defibrotide, which has pro-fibrinolytic and antithrombotic properties, is the most effective therapy; decompression of the sinusoids by a transjugular intrahepatic portosystemic shunt (TIPS) can be tried, especially to treat VOD after liver transplantation and when multiorgan failure (MOF) is not present. Liver transplantation can be the last option, but can not be considered a standard rescue therapy, because usually the concomitant presence of multiorgan failure contraindicates this procedure. PMID:17663504

  12. Diagnostic value of anti-annexin A5 antibodies in seropositive versus seronegative antiphospholipid syndrome patients

    Gihan Omar

    2018-04-01

    Full Text Available Background: Current laboratory criteria for antiphospholipid syndrome (APS classification recommend testing positive for antiphospholipid (aPL antibodies. However, there appears to be a subset of patients with classical APS manifestations who test negative. Aim of the work: To analyze the potential clinical usefulness of testing for anti-annexin A5 antibodies in patients with APS and to study the effectiveness of testing for non-criteria aPLs in an attempt to increase the diagnostic yield, particularly in seronegative APS. Patients and methods: 60 APS patients were divided into two groups; 30 seropositive (SP-APS (group I and 30 age and sex matched seronegative (sN-APS testing negative for aPL antibodies. Serum assay for detection of isotypes of anti-annexin A5 antibodies (IgG and IgM were conducted. Results: The mean age of the patients was 32.9 ± 5.8 years, female:male 57:3 and disease duration in SP-APS versus sN-APS (10.17 ± 4.9 years versus 9.6 ± 5.5 years respectively. Secondary APS was present in 16(53.3% patients in group I compared to 3(10% in group II (p < 0.0001. The mean anti-AnxA5 IgG level was 10.7 ± 5.6 U/ml and IgM was 11.2 ± 7.1 U/ml and were comparable between the 2 groups. The obstetric and thrombotic morbidity had no significant differences between SP and sN-APS. The IgG and IgM levels significantly correlated with the pregnancy morbidity, venous and arterial thrombosis events and showed reasonable sensitivities in their prediction (IgG:71.2%,72.8% and 75.8%; IgM: 68%,67.8% and 71.4% respectively and specificities (IgG:75.9%,77.8% and 81.5%; IgM: 70.9%,73.1% and 73.7% respectively. Conclusion: anti-annexinA5 antibodies are promising for detecting obstetric and thrombotic morbidity in both SP- and sN-APS patients. Keywords: Antiphospholipid syndrome, Seropositive APS (SP-APS, Seronegative APS (sN-APS, Anti-annexin A5 antibodies

  13. Calcium pyrophosphate deposition disease: clinical manifestations

    M.A. Cimmino

    2012-01-01

    Full Text Available Calcium pyrophosphate deposition (CPPD disease is an arthropathy caused by calcium pyrophosphate dihydrate (CPP crystal deposits in articular tissues, most commonly fibrocartilage and hyaline cartilage. According to EULAR, four different clinical presentations can be observed: 1 asymptomatic CPPD; 2 osteoarthritis (OA with CPPD; 3 acute CPP crystal arthritis; 4 chronic CPP inflammatory crystal arthritis. Acute CPP crystal arthritis is characterized by sudden onset of pain, swelling and tenderness with overlying erythema, usually in a large joint, most often the knee, wrist, shoulder, and hip. Occasionally, ligaments, tendons, bursae, bone and the spine can be involved. CPPD of the atlanto-occipital joint (crowned dens syndrome can cause periodic acute cervico-occipital pain with fever, neck stiffness and laboratory inflammatory syndrome. Chronic inflammatory arthritis is characterized by joint swelling, morning stiffness, pain, and high ESR and CRP. The relationship between OA and CPPD is still unclear. The main problem is whether such crystals are directly involved in the pathogenesis of OA or if they are the result of joint degeneration. Diagnosis is based on evaluation of history and clinical features, conventional radiology, and synovial fluid examination. Non-polarized light microscopy should be used initially to screen for CPPD crystals based upon their characteristic morphology, and compensated polarized light microscopy, showing the crystals to be weakly positive birefringent, is recommended for definitive identification, although this last pattern only occurs in about 20% of samples. The main goals of CPPD therapy are control of the acute or chronic inflammatory reaction and prevention of further episodes.

  14. Association of HCV Core Antigen Seropositivity with Long-Term Mortality in Patients on Regular Hemodialysis

    Akihiko Kato

    2012-03-01

    Full Text Available Anti-hepatitis C virus (HCV antibody seropositivity is independently associated with poor prognosis in hemodialysis (HD patients. However, anti-HCV antibody cannot distinguish between patients with active infection and those who have recovered from infection. We therefore aimed in this study to examine the association of HCV core antigen (HCVcAg seropositivity with mortality in HD patients. We first measured serum HCVcAg using an immunoradiometric assay and anti-HCV antibody in 405 patients on regular HD, and followed them for 104 months. There were 82 patients (20.2% who had been positive for anti-HCV antibodies; 57 (69.5% of these were positive for HCVcAg. During the follow-up, 29 patients were excluded, so we tested the association of HCVcAg seropositivity with all-cause, cardiovascular (CV and non-CV mortalities in 376 patients. A total of 209 patients (55.6% had expired during the observational period, 92 out of them due to CV causes. After adjusting for comorbid parameters, HCVcAg was independently associated with overall mortality (HR 1.61, 95% CI 1.05–2.47, p < 0.05. HCV infection was significantly related to liver disease-related mortality. Past HCV infection also contributed to CV mortality (HR 2.63, 95% CI 1.27–5.45, p < 0.01. In contrast, anti-HCV antibody and HCVcAg seropositivities did not associate with infectious disease-related and cancer-related (expect for hepatocellular carcinoma mortality. It follows from these findings that HCVcAg serology is associated with all-cause and CV mortality in HD patients.

  15. Seroprevalence and factors associated with seropositivity to equine arteritis virus in Spanish Purebred horses in Spain.

    Cruz, F; Fores, P; Mughini-Gras, L; Ireland, J; Moreno, M A; Newton, R

    2016-09-01

    Equine viral arteritis (EVA), a disease caused by infection with the equine arteritis virus (EAV), is present in many European countries. In Spain, the last confirmed outbreak was reported in 1992 and there is a paucity of seroprevalence studies. The disease has a major impact on the equine breeding industry, which is mainly represented by Spanish Purebred (SP) horses in Spain. To estimate the seroprevalence of EAV in the breeding SP horse population in central Spain and identify potential horse and studfarm level factors associated with seropositivity to EAV. Cross-sectional study. Individual serum samples from 555 SP horses, collected between September 2011 and November 2013 at 35 studfarms, were tested using a commercially available EAV antibody ELISA and seroneutralisation as the World Organisation for Animal Health reference confirmation test for samples with positive and equivocal results. Data on factors putatively associated with seropositivity to EAV were collected via a questionnaire and examined using random effects logistic regression for analysis of clustered data. Equine arteritis virus seroprevalence in the SP breeding population in central Spain standardised for the sex distribution of the reference horse population, was estimated to be 16.8% (95% confidence interval 5.2-28.5%). Increasing numbers of breeding mares on the studfarm and increasing percentage of mares with reproductive problems during the last 12 months were identified as being positively associated with EAV seropositivity. Mares vaccinated against Equine herpesvirus-1 (EHV-1) and/or -4 (EHV-4) were also positively associated with EAV seropositivity. These findings are of importance to ensure appropriate biosecurity measures for studfarms are carried out and may help facilitate the development of an EVA surveillance programme in the SP breeding horse population. © 2015 EVJ Ltd.

  16. Clinically Unsuspected Prion Disease Among Patients With Dementia Diagnoses in an Alzheimer's Disease Database.

    Maddox, Ryan A; Blase, J L; Mercaldo, N D; Harvey, A R; Schonberger, L B; Kukull, W A; Belay, E D

    2015-12-01

    Brain tissue analysis is necessary to confirm prion diseases. Clinically unsuspected cases may be identified through neuropathologic testing. National Alzheimer's Coordinating Center (NACC) Minimum and Neuropathologic Data Set for 1984 to 2005 were reviewed. Eligible patients had dementia, underwent autopsy, had available neuropathologic data, belonged to a currently funded Alzheimer's Disease Center (ADC), and were coded as having an Alzheimer's disease clinical diagnosis or a nonprion disease etiology. For the eligible patients with neuropathology indicating prion disease, further clinical information, collected from the reporting ADC, determined whether prion disease was considered before autopsy. Of 6000 eligible patients in the NACC database, 7 (0.12%) were clinically unsuspected but autopsy-confirmed prion disease cases. The proportion of patients with dementia with clinically unrecognized but autopsy-confirmed prion disease was small. Besides confirming clinically suspected cases, neuropathology is useful to identify unsuspected clinically atypical cases of prion disease. © The Author(s) 2015.

  17. Predictive markers of survival in HIV-seropositive and HIV-seronegative Tanzanian patients with extrapulmonary tuberculosis

    Richter, C.; Koelemay, M. J.; Swai, A. B.; Perenboom, R.; Mwakyusa, D. H.; Oosting, J.

    1995-01-01

    Prediction of survival in Tanzanian patients with extrapulmonary tuberculosis (TB). To evaluate the prognostic value of clinical and laboratory parameters on survival in human immunodeficiency virus (HIV) seropositive and HIV seronegative patients with extrapulmonary TB. Over an 8-month period 192

  18. Clinical manifestations of small vessel disease

    Kooistra, M.

    2014-01-01

    Cardiovascular disease is a major health problem worldwide. However, the mortality risk in patients with cardiovascular disease has decreased due to early detection of the disease and improved treatment possibilities. The downside of increased survival rates are higher rates of long-term functional

  19. Spectral analysis of HIV seropositivity among migrant workers entering Kuwait

    Mohammad Hameed GHH

    2008-03-01

    Full Text Available Abstract Background There is paucity of published data on human immunodeficiency virus (HIV seroprevalence among migrant workers entering Middle-East particularly Kuwait. We took advantage of the routine screening of migrants for HIV infection, upon arrival in Kuwait from the areas with high HIV prevalence, to 1 estimate the HIV seroprevalence among migrant workers entering Kuwait and to 2 ascertain if any significant time trend or changes had occurred in HIV seroprevalence among these migrants over the study period. Methods The monthly aggregates of daily number of migrant workers tested and number of HIV seropositive were used to generate the monthly series of proportions of HIV seropositive (per 100,000 migrants over a period of 120 months from January 1, 1997 to December 31, 2006. We carried out spectral analysis of these time series data on monthly proportions (per 100,000 of HIV seropositive migrants. Results Overall HIV seroprevalence (per 100,000 among the migrants was 21 (494/2328582 (95% CI: 19 -23, ranging from 11 (95% CI: 8 – 16 in 2003 to 31 (95% CI: 24 -41 in 1998. There was no discernable pattern in the year-specific proportions of HIV seropositive migrants up to 2003; in subsequent years there was a slight but consistent increase in the proportions of HIV seropositive migrants. However, the Mann-Kendall test showed non-significant (P = 0.741 trend in de-seasonalized data series of proportions of HIV seropositive migrants. The spectral density had a statistically significant (P = 0.03 peak located at a frequency (radians 2.4, which corresponds to a regular cycle of three-month duration in this study. Auto-correlation function did not show any significant seasonality (correlation coefficient at lag 12 = – 0.025, P = 0.575. Conclusion During the study period, overall a low HIV seroprevalence (0.021% was recorded. Towards the end of the study, a slight but non-significant upward trend in the proportions of HIV seropositive

  20. Helicobacter pylori seropositivity and risk of lung cancer.

    Jill Koshiol

    Full Text Available Lung cancer is the leading cause of cancer mortality worldwide. Helicobacter pylori (H. pylori is a risk factor for distal stomach cancer, and a few small studies have suggested that H. pylori may be a potential risk factor for lung cancer. To test this hypothesis, we conducted a study of 350 lung adenocarcinoma cases, 350 squamous cell carcinoma cases, and 700 controls nested within the Alpha-Tocopherol, Beta-Carotene Cancer Prevention Study (ATBC cohort of male Finnish smokers. Controls were one-to-one matched by age and date of baseline serum draw. Using enzyme-linked immunosorbent assays to detect immunoglobulin G antibodies against H. pylori whole-cell and cytotoxin-associated gene (CagA antigens, we calculated odds ratios (ORs and 95% confidence intervals (95% CIs for associations between H. pylori seropositivity and lung cancer risk using conditional logistic regression. H. pylori seropositivity was detected in 79.7% of cases and 78.5% of controls. After adjusting for pack-years and cigarettes smoked per day, H. pylori seropositivity was not associated with either adenocarcinoma (OR: 1.1, 95% CI: 0.75-1.6 or squamous cell carcinoma (OR: 1.1, 95% CI: 0.77-1.7. Results were similar for CagA-negative and CagA-positive H. pylori seropositivity. Despite earlier small studies suggesting that H. pylori may contribute to lung carcinogenesis, H. pylori seropositivity does not appear to be associated with lung cancer.

  1. [Clinical overview of auto-inflammatory diseases].

    Georgin-Lavialle, S; Rodrigues, F; Hentgen, V; Fayand, A; Quartier, P; Bader-Meunier, B; Bachmeyer, C; Savey, L; Louvrier, C; Sarrabay, G; Melki, I; Belot, A; Koné-Paut, I; Grateau, G

    2018-04-01

    Monogenic auto-inflammatory diseases are characterized by genetic abnormalities coding for proteins involved in innate immunity. They were initially described in mirror with auto-immune diseases because of the absence of circulating autoantibodies. Their main feature is the presence of peripheral blood inflammation in crisis without infection. The best-known auto-inflammatory diseases are mediated by interleukines that consisted in the 4 following diseases familial Mediterranean fever, cryopyrinopathies, TNFRSF1A-related intermittent fever, and mevalonate kinase deficiency. Since 10 years, many other diseases have been discovered, especially thanks to the progress in genetics. In this review, we propose the actual panorama of the main known auto-inflammatory diseases. Some of them are recurrent fevers with crisis and remission; some others evaluate more chronically; some are associated with immunodeficiency. From a physiopathological point of view, we can separate diseases mediated by interleukine-1 and diseases mediated by interferon. Then some polygenic inflammatory diseases will be shortly described: Still disease, Schnitzler syndrome, aseptic abscesses syndrome. The diagnosis of auto-inflammatory disease is largely based on anamnesis, the presence of peripheral inflammation during attacks and genetic analysis, which are more and more performant. Copyright © 2018 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier SAS. All rights reserved.

  2. Household Socioeconomic and Demographic Correlates of Cryptosporidium Seropositivity in the United States.

    Daniel J Becker

    Full Text Available Cryptosporidium are parasitic protozoa that infect humans, domestic animals, and wildlife globally. In the United States, cryptosporidiosis occurs in an estimated 750,000 persons annually, and is primarily caused by either of the Cryptosporidium parvum genotypes 1 and 2, exposure to which occurs through ingestion of food or water contaminated with oocytes shed from infected hosts. Although most cryptosporidiosis cases are caused by genotype 1 and are of human origin, the zoonotic sources of genotype 2, such as livestock, are increasingly recognized as important for understanding human disease patterns. Social inequality could mediate patterns of human exposure and infection by placing individuals in environments where food or water contamination and livestock contact is high or through reducing the availability of educational and sanitary resources required to avoid exposure.We here analyzed data from the National Health and Nutritional Examination Survey (NHANES between 1999 and 2000, and related seropositivity to Cryptosporidium parvum to correlates of social inequality at the household and individual scale. After accounting for the complex sampling design of NHANES and confounding by individual demographics and household conditions, we found impaired household food adequacy was associated with greater odds of Cryptosporidium seropositivity. Additionally, we identified individuals of non-white race and ethnicity and those born outside the United States as having significantly greater risk than white, domestic-born counterparts. Furthermore, we provide suggestive evidence for direct effects of family wealth on Cryptosporidium seropositivity, in that persons from low-income households and from families close to the poverty threshold had elevated odds of seropositivity relative to those in high-income families and in households far above the poverty line.These results refute assertions that cryptosporidiosis in the United States is independent of

  3. Seropositivity of Toxoplasma gondii and Toxocara spp. in Children with Cryptogenic Epilepsy, Benha, Egypt.

    Eraky, Maysa Ahmad; Abdel-Hady, Soha; Abdallah, Karim Fetouh

    2016-06-01

    The present study aimed to investigate the possible association of Toxoplasma gondii and Toxocara spp. infections with cryptogenic epilepsy in children. The study was carried out between June 2014 and March 2015. Total 90 children (40 with cryptogenic epilepsy, 30 with non-cryptogenic epilepsy, and 20 healthy control children) were evaluated to determine the anti-Toxocara and anti-T. gondii IgG seropositivity using ELISA kits. Epileptic cases were selected from those attending the pediatrics outpatient clinic of Benha University Hospital, Pediatrics Neurology Unit, and from Benha Specialized Hospital of children. The results showed that the level of anti-T. gondii IgG seropositivity was significantly higher among children with cryptogenic epilepsy (20%) than among children with non-cryptogenic children (0%). In healthy controls (10%), there was no association between toxocariasis seropositivity and cryptogenic epilepsy (only 5.7%; 4 out of 70 cases) among cases and 10% (2 out of 20) among controls. Among toxocariasis IgG positive cases, 3 (7.5%) were cryptogenic, and only 1 (3.3%) was non-cryptogenic. These statistically significant results support the association between T. gondii infection and cryptogenic epilepsy while deny this association with toxocariasis.

  4. An in vitro study of antifungal drug susceptibility of Candida species isolated from human immunodeficiency virus seropositive and human immunodeficiency virus seronegative individuals in Lucknow population Uttar Pradesh.

    Dar, Mohammad Shafi; Sreedar, Gadiputi; Shukla, Abhilasha; Gupta, Prashant; Rehan, Ahmad Danish; George, Jiji

    2015-01-01

    Candidiasis is the most common opportunistic infection in human immunodeficiency virus (HIV) seropositive patients, starting from asymptomatic colonization to pathogenic forms and gradual colonization of non-albicans in patients with advanced immunosuppression leads to resistance for azole group of antifungal drugs with high rate of morbidity and mortality. To isolate the Candida species and determine of antifungal drug susceptibility against fluconazole, itraconazole, nystatin, amphotericin B, and clotrimazolein HIV seropositive and control individuals, with or without clinical oropharyngeal candidiasis (OPC). Includes samples from faucial region of 70 subjects with and without clinical candidiasis in HIV seropositive and controls were aseptically inoculated onto Sabaraud's Dextrose Agar media and yeasts were identified for the specific species by Corn Meal Agar, sugar fermentation and heat tolerance tests. Antifungal drug susceptibility of the isolated species was done against above-mentioned drugs by E-test and disc diffusion method. The commonly isolated species in HIV seropositive and controls were Candida albicans, Candida glabrata and Candida tropicalis Candida guilliermondii and Candida dubliniensis isolated only in HIV seropositive patients. Susceptibility against selected antifungal drugs was observed more in HIV-negative individuals whereas susceptible dose-dependent and resistance were predominant in HIV-positive patients. Resistance is the major problem in the therapy of OPC, especially in HIV seropositive patients due to aggressive and prolonged use of antifungal agents, therefore, our study emphasizes the need for antifungal drug susceptibility testing whenever antifungal treatment is desired, especially in HIV-infected subjects.

  5. Chlamydia trachomatis IgG3 seropositivity is a predictor of reproductive outcomes in infertile women with patent fallopian tubes

    Steiner, Anne Z.; Diamond, Michael P.; Legro, Richard S.; Schlaff, William D.; Barnhart, Kurt T.; Casson, Peter R.; Christman, Gregory M.; Alvero, Ruben; Hansen, Karl R.; Geisler, William M.; Thomas, Tracey; Santoro, Nanette; Zhang, Heping; Eisenberg, Esther

    2015-01-01

    Objective To determine if Chlamydia trachomatis (Ct) seropositivity as detected by the Ct elementary body (EB)-based enzyme linked immunosorbent assay (Ct EB ELISA) predicts pregnancy and pregnancy outcome among infertile women with documented tubal patency. Design Cohort study Setting Outpatient clinics participating in the reproductive medicine network Patients 1250 infertile women with documented tubal patency enrolled in one of two randomized controlled trials: PPCOSII and AMIGOS Intervention Sera were analyzed for anti-Ct IgG1 and IgG3 antibodies using a research Ct EB ELISA. OD405 readings ≥0.35 and ≥0.1 were considered positive for IgG1 and IgG3, respectively. Main Outcome Measures Primary outcomes included pregnancy, live birth, and ectopic pregnancy. Log linear regression was used to determine the relative risk after adjusting for age, race, treatment medication, smoking status, and current alcohol use. Results 243 (19%) women were seropositive for anti-Ct IgG3. They tended to be non-White and smokers. Anti-Ct IgG3 seropositive women were significantly less likely to conceive (RR 0.65, 95% CI 0.52-0.83) or to have a live birth (RR 0.59, 95% 0.43-0.80); these associations were weakened after adjusting for number of HSG-documented patent tubes (RR 0.73, 95% CI 0.56-0.97) and (0.73, 95% CI: 0.50-1.04), respectively. Anti-Ct IgG3 seropositive women who conceived had 2.7 (95% CI: 1.40-5.34) times the risk of ectopic pregnancy. Conclusions Even in the presence of tubal patency, anti-Ct IgG3 seropositivity is associated with lower likelihood of pregnancy. Anti-Ct IgG3 seropositive women have up to 3 times the risk of ectopic pregnancy. PMID:26413816

  6. Clinical Manifestations and Treatment of Lyme Disease.

    Sanchez, Joyce L

    2015-12-01

    Lyme disease is the most common tick-borne illness in the United States and is also seen in areas of Europe and Asia. The growing deer and Ixodes species tick populations in many areas underscore the importance of clinicians to properly recognize and treat the different stages of Lyme disease. Controversy regarding the cause and management of persistent symptoms following treatment of Lyme disease persists and is highlighted in this review. Copyright © 2015 Elsevier Inc. All rights reserved.

  7. Recruitment of subjects into clinical trials for Alzheimer disease.

    Knebl, Janice A; Patki, Deepti

    2010-09-01

    Alzheimer disease is a devastating neurodegenerative disorder affecting millions of Americans. It reduces the ability of the individual to remain independent, places a burden on caregivers, and substantially increases healthcare costs. New treatments are being tested in numerous clinical trials with the goal of preventing or delaying the onset of Alzheimer disease, slowing or modifying the disease's course, or finding a cure for patients with the disease. Alzheimer disease research can successfully proceed only if individuals who have this illness are willing to participate in clinical trials. However, recruitment and retention of subjects in clinical trials for Alzheimer disease is a challenging task. Furthermore, because of reductions in decision-making capacities of individuals with Alzheimer disease, clinical trials also need to involve caregivers. The present article delineates unique hurdles encountered in the recruitment process for Alzheimer disease clinical trials. The article also identifies strategies for effective recruitment of subjects in Alzheimer disease clinical trials, including guidelines to help principal investigators and clinical research coordinators reach recruitment goals.

  8. Wilson disease : from clinical to molecular

    Houwen, Roderick Henk Johan

    1991-01-01

    Wilson disease is an autosomal recessive copper storage disease. It is characterized by an inability of the liver to; excrete copper into bile and to incorporate copper into ceruloplasmin. This results in a gradual accumulation of copper in the liver and subsequently in the brain and other organs,

  9. Liver involvement in Gaucher disease - Review and clinical approach.

    Adar, Tomer; Ilan, Yaron; Elstein, Deborah; Zimran, Ari

    2018-02-01

    Gaucher disease (GD), one of the most prevalent lysosomal storage diseases, is associated with glucocerebroside accumulation in cells of the monocyte-macrophage system in various organs, including the liver. Evaluating and managing liver disease in patients with Gaucher disease may be challenging. While hepatic involvement is common in Gaucher disease, its severity, and clinical significance span a wide spectrum, ranging from sub-clinical involvement to liver cirrhosis with its associated complications including portal hypertension. Apart from liver involvement in Gaucher disease, patients with may also suffer from other comorbidities involving the liver. That Gaucher disease itself can mimic hepatic lesions, affect laboratory tests used to characterize liver disease, and may be associated with non-cirrhotic portal hypertension, complicates the diagnostic approach even more. Better understanding of liver involvement in Gaucher disease can spare patients unnecessary invasive testing, and assist physicians in decision making when evaluating patients with Gaucher disease suspected for significant liver disease. This review describes the various clinical manifestations, laboratory and imaging abnormalities that may be encountered when following patients with Gaucher disease for liver involvement. The mechanism for liver disease are discussed, as well as the possible hepato-protective effect of glucocerebroside, and the a diagnostic and treatment approaches. Copyright © 2016 Elsevier Inc. All rights reserved.

  10. Clinical significance of adrenal computed tomography in Addison's disease

    Sun, Zhong-Hua; Nomura, Kaoru; Toraya, Shohzoh; Ujihara, Makoto; Horiba, Nobuo; Suda, Toshihiro; Tsushima, Toshio; Demura, Hiroshi; Kono, Atsushi

    1992-01-01

    Adrenal computed tomographic (CT) scanning was conducted in twelve patients with Addison's disease during the clinical course. In tuberculous Addison's disease (n=8), three of four patients examined during the first two years after disease onset had bilaterally enlarged adrenals, while one of four had a unilaterally enlarged one. At least one adrenal gland was enlarged after onset in all six patients examined during the first four years. Thereafter, the adrenal glands was atrophied bilaterally, in contrast to adrenal glands in idiopathic Addison's disease which was atrophied bilaterally from disease onset (n=2). Adrenal calcification was a less sensitive clue in tracing pathogenesis, i.e., adrenal calcification was observed in five of eight patients with tuberculous Addison's disease, but not idiopathic patients. Thus, adrenal CT scanning could show the etiology of Addison's disease (infection or autoimmunity) and the phase of Addison's disease secondary to tuberculosis, which may be clinically important for initiating antituberculous treatment. (author)

  11. Human Immunodeficiency Virus (HIV) Seropositivity In African ...

    A seroprevalence study of Human immunodeficiency virus (HIV) infection in new patients attending the eye clinic of LAUTECH Teaching Hospital in Osogbo, Osun State, Nigeria showed that twenty-nine patients 2.7%) were positive to HIV1. No patient was positive to HIV 2. There were 21 males (72.4%) and 8 females ...

  12. Cultural Influences on Hepatitis B Surface Antigen Seropositivity in ...

    Objective: To determine the role of cultural influences, namely: circumcision, ear piercing and traditional scarification, on HbsAg seropositivity among primary school children in Nnewi. Subjects and Method: Two hundred and thirty seven randomly selected primary school children aged 5-12 years, were screened for HbsAg.

  13. Seropositivity and determinants of immunoglobulin-G (IgG ...

    Background: This study is the first documented prevalence of IgG antibody against HSV-1&-2 in Port Harcourt, Nigeria and thus provides baseline data for future in-depth studies on HSV infection in South-South, Nigeria. Objective: This study determined the seropositivity and determinants of serum IgG antibody against ...

  14. Isoniazid prophylactic therapy for tuberculosis in HIV-seropositive ...

    hypothetical cohort of 100 000 HIV-seropositive people in. South Africa over a ... health sector resources can be utilised in an optimal manner. This article ... second part we estimate the costs and benefits of isoniazid preventive ... The cohort was stratified into ... population risk of dying from any cause, the number of dual.

  15. Phenotype and Clinical Course of Inflammatory Bowel Disease with Co-Existent Celiac Disease.

    Tse, Chung Sang; Deepak, Parakkal; De La Fuente, Jaime; Bledsoe, Adam C; Larson, Joseph J; Murray, Joseph A; Papadakis, Konstantinos A

    2018-05-07

    Inflammatory bowel diseases, principally Crohn's disease and ulcerative colitis, and celiac disease are among the most common immune-mediated gastrointestinal diseases. We aim to elucidate the clinical course and outcomes of patients with concomitant inflammatory bowel disease and celiac disease, a unique population that remains scarcely studied to date. A retrospective matched case-control study of adults with coexistent inflammatory bowel disease and celiac disease was performed at a tertiary referral institution in North America. Logistic regression and Kaplan-Meier curves compared disease characteristics and clinical outcomes of the two groups. A total of 342 inflammatory bowel disease patients were included in this study, of which 114 had coexistent celiac disease and 228 did not. Patients with coexistent inflammatory bowel disease and celiac disease had higher rates of primary sclerosing cholangitis (19.3% vs 5.7%; odds ratio, 4.4; 95% confidence interval, 2.1-9.4; pceliac disease (10.5% vs 3.5%; odds ratio 3.2; 95% confidence interval 1.3-8.2; p=0.01), compared to patients without concomitant celiac disease. Patients with inflammatory bowel disease with concomitant celiac disease have unique phenotypic features compared to non-celiac inflammatory bowel disease, with higher risks for colitis-related hospitalizations, extensive colitis, and primary sclerosing cholangitis. Increased recognition of coexistent IBD and celiac disease can prompt clinicians to investigate for concomitant disease sooner, particularly in patients with seemingly refractory disease.

  16. Inherited Retinal Degenerative Disease Clinical Trial Network

    2012-10-01

    diseases and dry AMD; • Established patient databases at the University of Utah and University of Medicine and Dentistry of New Jersey CTECs, classified...Scholl: Emily Fletcher, Rupert Strauss, Yulia Wolfson, Stacey Seabrook Wilmer Biostatistics Center: Ann Ervin, Beatriz Munoz Reading Center

  17. Clinical aspects of rheumatic diseases in children

    Saenger, L.

    1978-01-01

    Rheumatic fever is no longer the most problematic of rheumatic diseases as it poses some diagnostic, but no radiological problems. The most problematic are chronic inflammatory processes of the joints. Here, the fate in life of the child patients does indeed depend upon an exact diagnosis. (orig./AJ) [de

  18. Clinical outcome of Crohn's disease according to the Vienna classification: disease location is a useful predictor of disease course

    Oostenbrug, Liekele E.; van Dullemen, Hendrik M.; te Meerman, Gerard J.; Jansen, Peter L. M.; Kleibeuker, Jan H.

    2006-01-01

    OBJECTIVES: Crohn's disease (CD) is a complex genetic disease with multiple clinical patterns. Clinical classifications may help to identify subgroups of patients that have a distinct pattern of disease, and they are also a prerequisite for the conduction of genetic and therapeutic studies. The aim

  19. Clinical outcome of Crohn's disease according to the Vienna classification : disease location is a useful predictor of disease course

    Oostenbrug, Liekele E.; van Dullemen, Hendrik M.; te Meerman, Gerard J.; Jansen, Peter L. M.; Kleibeuker, Jan H.

    Objectives Crohn's disease (CD) is a complex genetic disease with multiple clinical patterns. Clinical classifications may help to identify subgroups of patients that have a distinct pattern of disease, and they are also a prerequisite for the conduction of genetic and therapeutic studies. The aim

  20. Current clinical research of immunoglobulin G4-related orbital disease

    Yang Wang

    2016-05-01

    Full Text Available Immunoglobulin G4-related disease(IgG4-related diseasehas received lots of attention in medical community as a recently recognized fibro-inflammatory condition. It is characterized by infiltration of IgG4-immunopositive plasmacytes and concentration of elevated serum IgG4. IgG4-related disease shows organ enlargement or nodular/hyperplastic lesions in various organs including the pancreas, hepatobiliary tract and orbit, which is called IgG4-related orbital disease. The diagnostic criteria for IgG4-related disease and IgG4-related orbital disease has recently been established, which is based on clinical, imaging and histopathologic features of the orbital lesions. Besides, attention should be drawn to the differentiation from other diseases. The treatment is empirical including corticosteroids, immunosuppressive drugs, radiotherapy, and rituximab. This article reviews clinical progression of IgG4-related orbital disease.

  1. Mitochondrial Diseases: Clinical Features- Management of Patients

    Filiz Koc

    2003-02-01

    Full Text Available Mitochondria are unique organells which their own DNA in cells. Human mitochondrial DNA is circular, double-stranded molecule and small. Because all mitochondria are contributed by the ovum during the formation of the zygote, the mitochondrial genom is transmitted by maternal inheritance. Multisystem disorders such as deafness, cardiomyopathy, miyopathy can be seen in mitochondrial diseases. [Archives Medical Review Journal 2003; 12(0.100: 14-31

  2. A New Resource for STD Clinical Providers: The Sexually Transmitted Diseases Clinical Consultation Network.

    Caragol, Laura A; Wendel, Karen A; Anderson, Teri S; Burnside, Helen C; Finkenbinder, Allison; Fitch, John D; Kelley, Destiny H; Stewart, Terry W; Thrun, Mark; Rietmeijer, Cornelis A

    2017-08-01

    An online consultation tool, the Sexually Transmitted Diseases Clinical Consultation Network is a new resource for sexually transmitted disease clinicians and clinic managers. An initial evaluation shows that most requests (29%) were from medical doctors, followed by nurse practitioners (22%). Syphilis queries comprised 39% of consults followed by gonorrhea (12%) and chlamydia (11%).

  3. Seroprevalence of Epstein-Barr Virus, Cytomegalovirus, and Polyomaviruses in Children with Inflammatory Bowel Disease.

    Hradsky, Ondrej; Copova, Ivana; Zarubova, Kristyna; Durilova, Marianna; Nevoral, Jiri; Maminak, Miroslav; Hubacek, Petr; Bronsky, Jiri

    2015-11-01

    Young age and thiopurine therapy are risk factors for lymphoproliferative disease among patients with inflammatory bowel disease (IBD). The aims of this study were to evaluate the prevalence of seropositivity for the Epstein-Barr virus (EBV) and human cytomegalovirus (CMV) among children and adolescents with IBD, to assess the viral load of EBV, CMV, and BK and JC polyomaviruses (BKV, JCV) in these patients, and to assess the influence of different therapeutic regimens on seroprevalence and viral load. Children who had been followed in our center were tested for EBV, CMV, BKV, and JCV in a cross-sectional study. One hundred and six children were included who had Crohn's disease (68%), ulcerative colitis (29%), and unclassified IBD (3%). We found that 64% of patients were EBV seropositive. The proportion of EBV seropositive patients increased during childhood. Azathioprine therapy (p = 0.003) was associated with EBV seropositivity in a multiple logistic regression model, after adjusting for gender, age, and disease activity at determination. We found a significant association between the number of polymerase chain reaction copies and infliximab dose (p = 0.023). We did not find any significant association between CMV serology and CMV, BKV, or JCV viral load, or any other therapeutic regimen or clinical characteristics. Treatment with azathioprine appears to be a risk factor for early EBV seropositivity in children with IBD, and the infliximab dose was associated with a higher EBV viral load.

  4. [Inflammatory brain diseases in clinical practice].

    Lungwitz, J; Voigt, W

    1984-06-01

    Over a period of twenty years all cases of inflammatory diseases treated in a neurological intensive-care unit were analysed in retrospective. For 90 cases of purulent bacterial meningitis, letality amounted to 30%. The need for agent-oriented, intensive early treatment is discussed. At a reduced incidence of tuberculosis, meningitis tuberculosa is generally neglected. In the majority of cases patients suffering from "encephalitic syndrome" make high demands on intensive-therapeutic concepts, including interdisciplinary cooperation, due to high complication-rates and lack of causal therapy. In 95 cases, letality also amounted to 30%.

  5. Radiation induced liver disease: A clinical update

    Benson, R.; Madan, R.; Chander, S.; Kilambi, R.

    2016-01-01

    Radiation-induced liver disease (RILD) or radiation hepatitis is a sub-acute form of liver injury due to radiation. It is one of the most dreaded complications of radiation which prevents radiation dose escalation and re irradiation for hepatobiliary or upper gastrointestinal malignancies. This complication should be kept in mind whenever a patient is planned for irradiation of these malignancies. Although, incidence of RILD is decreasing due to better knowledge of liver tolerance, improved investigation modalities and modern radiation delivery techniques, treatment options are still limited. In this review article, we have focussed on pathophysiology, risk factors, prevention and management of RILD

  6. Periodontal disease in diabetic patients - clinical and histopathological aspects.

    Corlan Puşcu, Dorina; Ciuluvică, Radu Constantin; Anghel, Andreea; Mălăescu, Gheorghe Dan; Ciursaş, Adina Nicoleta; Popa, Gabriel Valeriu; Agop Forna, Doriana; Busuioc, Cristina Jana; Siloşi, Izabela

    2016-01-01

    Periodontal disease is one of the most frequent diseases affecting people all over the world. The relation between periodontal disease and diabetes mellitus raised the interest both of dentists and doctors treating metabolic diseases, as the two conditions influence one another. In our study, we analyzed a number of 75 patients with diabetes mellitus and periodontal disease that presented to the medical consultory for conditions of the dental maxillary system. The clinical study showed that periodontal disease and diabetes may affect young adults as well, still this pathological association more frequently appears after the age of 50. The disease was identified especially in the women living in urban area. The clinical examination of the dental maxillary system identified the presence of gingival ulcerations, dental calculus, gingival bleeding, radicular leftovers with anfractuous margins, fixed prostheses with an inappropriate cervical adjustment. Of the systemic diseases associated to periodontal disease and diabetes mellitus, there was observed that 66.66% of the patients also suffered from cardiovascular diseases (high blood pressure, ischemic cardiopathy, heart failure), and 37.33% suffered from obesity. The histopathological and immunohistochemical tests highlighted the presence of an inflammatory chronic, intense reaction, mainly formed of lymphocytes, plasmocytes, macrophages and granulocytes, heterogeneously disseminated and alteration of the structure of marginal and superficial periodontium. The inflammatory reaction in the patients with periodontal disease and diabetes was more intense than in the patients with periodontal disease without diabetes.

  7. Graves Disease And Down Sindrome : Clinical Case

    Scrinic Olesea

    2015-08-01

    Full Text Available Introduction: Pacients with Down’s syndrome present an increase revalence of autoimune endocrine disorders. We communicate the case of 14 years and 6 months old pacient known with Down syndrome admitted in Endocrinology department with suspicion of hyperthyroidism, the diagnosis being confirmed by hormonal dosage. The particularity of the case consists in: symptomatology onset during puberty, clinical evolution with mild symptoms, without ocular involvement, morphological and functional remission obtained relatively soon after the initiation of antithyroid therapy, lack of posttherapy side effects, favorabile evolution under the “block and replace” therapy

  8. [Clinical microbiology laboratory and imported parasitic diseases].

    Martín-Rabadán, Pablo; Martínez-Ruiz, Rocío; Cuadros, Juan; Cañavate, Carmen

    2010-12-01

    Imported parasitosis represents an increasingly frequent diagnostic challenge for microbiology laboratories. A surge in immigration and international travel has led to a rise in the number of imported cases of parasitosis, and this trend is expected to continue in the future. The present article addresses this challenge by reviewing recommended diagnostic approaches and tests. Currently, microscopy is always recommended when analysing blood samples for parasites. If malaria is suspected, rapid antigen testing (including at least HRP2 antigen) should also be performed. The work-up for suspected leishmaniasis should include serology, culture, and in selected cases detection of antigen in urine. In suspected Chagas disease, two different serological tests should be performed. PCR for blood protozoa is highly sensitive, although it cannot be used to rule out Chagas disease, since this condition may be present without parasitemia. Accurate diagnosis of intestinal amebiasis usually requires PCR or antigen detection tests. In helminthiasis, traditional microscopy may need to be complemented with other tests, such as agar plate culture for strongyloidiasis, Og4C3 antigen detection for bancroftian filariasis, and antibody detection test for filariasis and schistosomiasis. Copyright © 2010 Elsevier España, S.L. All rights reserved.

  9. The clinical profile of idiopathic Parkinson's disease in a South ...

    The clinical profile of idiopathic Parkinson's disease in a South African hospital complex - the influence of ethnicity and gender. Marcelle Smith, Girish Modi. Abstract. Background Idiopathic Parkinson's Disease (IPD) has not been well studied in Black African populations. Data on the demographics, phenotype differences ...

  10. Infections Disease in the Dental Clinical Nigerian Dentists ...

    The dental clinic is a potential source of transmission of infectious disease due to the invasive nature of many dental procedures. Infectious disease therefore constitutes serious occupational hazards to all oral health care workers. This descriptive study aims to assess the knowledge of dentists on common transmissible ...

  11. Clinical evaluation of SPECT in cerebrovascular disease

    Oshibuchi, Masao; Satoh, Mitsutaka; Kanda, Tetsuro; Nishi, Fumiaki; Yamane, Kanji; Fujimatsu, Masahiko; Edamitsu, Satoshi; Anno, Yasuro; Ohtake, Hisashi.

    1989-01-01

    In 131 patients with cerebrovascular disease, regional cerebral blood flow were determined by 123 I-IMP (N-isopropyl ( 123 I)-iodoamphetamine) or 99m Tc-HM-PAO ( 99m Tc (d, 1)-hexamethyl propyleneamine oxime) SPECT and findings were compared with those of X-CT or MRI. The perfusion deficit detected by SPECT was larger than the deficit by X-CT or MRI in every case. The perfusion deficit area was more clearly demonstrated by SPECT than by X-CT or MRI in patients with acute cerebral infarction. The hypoperfusion area determined by 123 I-IMP SPECT was wider than that by 99m Tc-HM-PAO SPECT. The crossed cerebellar diaschisis was observed in 56 out of 131 cases (43%). The results of operation were quantitatively evaluated by 123 I-IMP SPECT in 25 patients. (author)

  12. ETIOLOGY, PATHOGENESIS AND CLINICAL DIAGNOSIS OF PEYRONIE’S DISEASE

    Тарас Валерьевич Шатылко

    2017-03-01

    Full Text Available Peyronie’s disease remains an understudied progressing disease being  one of the relevant problems of modern urology and andrology. This condition may cause erectile dysfunction in men of fertile age and its negative impact on sexual function adversely affects patients’ quality of life. This article reviews epidemiology, pathophysiology and specifics of recording history and clinical diagnosis of Peyronie’s disease, that includes questionnaires, physical examination, evaluation of erectile function and penile deformity.

  13. Early diagnosis of Alzheimer's disease. Clinical significance and future perspectives

    Buerger, K.; Teipel, S.J.; Hampel, H.

    2000-01-01

    Early diagnosis of Alzheimer's disease describes the recognition and diagnosis in patients with very mild dementia. Internationally accepted diagnostic criteria support the diagnosis based on clinical evaluation. Recent advances in structural and functional neuroimaging as well as studies on specific proteins in the cerebro-spinal fluid that are related to distinct pathophysiological disease processes are most promising approaches to defining biological markers of Alzheimer's disease. (orig.) [de

  14. Clinical Course of Acute Pancreatitis in Chronic Kidney Disease ...

    Introduction: The aim of this study was to assess the clinical course, etiology and complications of acute pancreatitis among chronic kidney disease (CKD) patients in a tertiary care renal center in Karachi. Methods: We retrospectively evaluated the clinical course of CKD patients who presented to our emergency room with ...

  15. Clinical and genetic data of Huntington disease in Moroccan patients

    Background: Huntington's disease (HD) occurs worldwide with prevalence varying from 0.1 to 10 /100,000 depending of the ethnic origin. Since no data is available in the Maghreb population, the aim of this study is to describe clinical and genetic characteristics of Huntington patients of Moroccan origin. Methods: Clinical ...

  16. Clinical Features and Pattern of Presentation of Breast Diseases in ...

    Objective: To characterize the clinical features and pattern of presentation of breast diseases as observed in our practice. Materials and Methods: A prospective study of 121 consecutive patients with breast complaints presenting in our Surgical Outpatient Clinics. The relevant data were collected by two surgeons using the ...

  17. Influence of cerebrovascular disease on brain networks in prodromal and clinical Alzheimer's disease.

    Chong, Joanna Su Xian; Liu, Siwei; Loke, Yng Miin; Hilal, Saima; Ikram, Mohammad Kamran; Xu, Xin; Tan, Boon Yeow; Venketasubramanian, Narayanaswamy; Chen, Christopher Li-Hsian; Zhou, Juan

    2017-11-01

    Network-sensitive neuroimaging methods have been used to characterize large-scale brain network degeneration in Alzheimer's disease and its prodrome. However, few studies have investigated the combined effect of Alzheimer's disease and cerebrovascular disease on brain network degeneration. Our study sought to examine the intrinsic functional connectivity and structural covariance network changes in 235 prodromal and clinical Alzheimer's disease patients with and without cerebrovascular disease. We focused particularly on two higher-order cognitive networks-the default mode network and the executive control network. We found divergent functional connectivity and structural covariance patterns in Alzheimer's disease patients with and without cerebrovascular disease. Alzheimer's disease patients without cerebrovascular disease, but not Alzheimer's disease patients with cerebrovascular disease, showed reductions in posterior default mode network functional connectivity. By comparison, while both groups exhibited parietal reductions in executive control network functional connectivity, only Alzheimer's disease patients with cerebrovascular disease showed increases in frontal executive control network connectivity. Importantly, these distinct executive control network changes were recapitulated in prodromal Alzheimer's disease patients with and without cerebrovascular disease. Across Alzheimer's disease patients with and without cerebrovascular disease, higher default mode network functional connectivity z-scores correlated with greater hippocampal volumes while higher executive control network functional connectivity z-scores correlated with greater white matter changes. In parallel, only Alzheimer's disease patients without cerebrovascular disease showed increased default mode network structural covariance, while only Alzheimer's disease patients with cerebrovascular disease showed increased executive control network structural covariance compared to controls. Our

  18. IMPULSIVE-COMPULSIVE DISORDERS IN PARKINSON'S DISEASE. CLINICAL CASES

    N. V. Fedorova; A. V. Nikitina

    2015-01-01

    Objective – a description of clinical cases of impulsive­compulsive disorders in Parkinson,s disease. The first clinical case. Patient N., 75 years old, suffering for 15 years from Parkinson,s disease, akinetic­rigid form of the disease, stage 4 by Hyun–Yar. Since 2009, he received levodopa/carbidopa 250/25 mg 5 times/day (daily dose of 1250 mg of levodopa); pramipexole 3.5 mg per day (daily dose 3.5 mg), amantadine sulfate 100 mg 5 times/day (daily dose 500 mg). While taking antiparkinsonian...

  19. Clinical and imaging diagnosis for heredodegenerative diseases.

    Boddaert, Nathalie; Brunelle, Francis; Desguerre, Isabelle

    2013-01-01

    Clinical features (progressive psychomotor retardation, seizures, movement disorders and motor signs in both central and peripheral systems, sensorineural defects, and psychiatric symptoms) and brain imaging are the keys to diagnosis. CT is indicated for the detection of calcifications and blood, and for angiography. MRI in all three axes requires T1, T2, FLAIR (from 1 year on), eventually T2* or contrast administration, and diffusion in any acute condition. MR spectroscopy allows the dectection of lactate and creatine deficiency, elevated choline in high membrane turnover, and low NAA in neuronal death. The normal sequence of myelination needs to be taken into account. Pre- and neonatal anomalies include cystic and basal ganglia lesions, gyral and myelin anomalies, callosal agenesis, and large subdural spaces. Anomalies disclosed after 3 months of age include basal ganglia appearing hyper- or hypointense on T2, hypointense on T2*, or calcified white matter anomalies mainly periventricular or subcortical, or with contrast enhancement, associated with macrocephaly and/or large or very small cysts, and hypomyelination; there may be "vascular" or pseudostroke disorders, cortical atrophy, hypoplasia, or abnormal signal of the brainstem and/or cerebellum. Spectroscopy should investigate basal ganglia, white matter, and the cerebellum. MRI may reveal typical alterations of the brain at the preclinical stage in siblings of affected children. Copyright © 2013 Elsevier B.V. All rights reserved.

  20. Ethical clinical translation of stem cell interventions for neurologic disease

    Cote, David J; Bredenoord, Annelien L; Smith, Timothy R

    2017-01-01

    The application of stem cell transplants in clinical practice has increased in frequency in recent years. Many of the stem cell transplants in neurologic diseases, including stroke, Parkinson disease, spinal cord injury, and demyelinating diseases, are unproven-they have not been tested...... in prospective, controlled clinical trials and have not become accepted therapies. Stem cell transplant procedures currently being carried out have therapeutic aims, but are frequently experimental and unregulated, and could potentially put patients at risk. In some cases, patients undergoing such operations...... are not included in a clinical trial, and do not provide genuinely informed consent. For these reasons and others, some current stem cell interventions for neurologic diseases are ethically dubious and could jeopardize progress in the field. We provide discussion points for the evaluation of new stem cell...

  1. Molecular genotyping of HCV infection in seropositive blood donor

    Zarin, Siti Noraziah Abu; Ibrahim, Nazlina

    2013-11-01

    This study is to investigate the prevalence of hepatitis C virus infection in seropositive blood donor. RNA was extracted from 32 positive samples in National Blood Centre and Melaka Hospital. The core and NS5B sequences were obtained from 23 samples. Genotype 3a is most prevalent in this study followed by genotype 1a. Evidence of mixed-genotypes (3a and 1b) infections was found in 5 subjects.

  2. Is there a link between seropositivity to Helicobacter pylori and hepatitis A virus? A systematic review.

    BinSaeed, Abdulaziz A

    2010-07-01

    Since hepatitis A virus (HAV) is acquired primarily through the fecal-oral pathway, several investigators have used HAV seropositivity as a proxy for exposure to this pathway. This paper is a critical review of the evidence relevant to the association between seropositivity to HAV and Helicobacter pylori, and considers the validity of comparisons for testing the hypothesis that H. pylori spreads by the fecal-oral route. A Medline search identified reports of all types published in the English language literature that were linked to the keywords 'Campylobacter pylori', 'hepatitis A', or 'Helicobacter pylori', cross-referenced with 'seroepidemiology', 'seroprevalence', or 'seropositivity'. Studies identified by the search were included in the review if they used specific IgG antibodies to classify the serostatus of subjects for both HAV and H. pylori infection and provided an estimate of the magnitude of the association between HAV and H. pylori or information that permitted calculation of an odds ratio (OR). Out of the 21 studies identified, 15 met the inclusion criteria. The studies showed ORs for an association of HAV and H. pylori that ranged from 0.81 to 8.4. After adjustment for potential confounders, ORs shifted toward the null. They also showed that HAV seroprevalence is lower than H. pylori seroprevalence in early life and then becomes higher in later life. Thus in most populations, the trends cross over at some point. The observed associations between the two infections are generally overestimated by the confounding effects of age and socio-economic status-related factors, and when these factors are controlled, the association becomes weak. Moreover, HAV infection elicits a long-term antibody response, while H. pylori infection does not. Consequently, serostatus comparison does not constitute a convincing test of the fecal-oral transmission hypothesis for H. pylori. Copyright 2009 International Society for Infectious Diseases. Published by Elsevier Ltd. All

  3. Treatment of clinically diagnosed laryngopharyngeal reflux disease.

    Youssef, Tarek Fouad; Ahmed, Mohamed Rifaat

    2010-11-01

    To determine the incidence of Helicobacter pylori (HP) stool antigen (HPSA) in patients with laryngopharyngeal reflux disease (LPRD), and to make a comparison of 2 treatment regimens that have been used based on the presence or absence of HPSA positivity in patients with LPRD. Randomized controlled study. Suez Canal University Hospital, Ismalia, Egypt. A total of 212 patients with symptoms of LPRD. Patients were evaluated by laryngoscopy, ambulatory pH monitoring for 24 hours, and HPSA testing. Esomeprazole magnesium as a monotherapy was evaluated vs triple therapy in patients with HP infection. To determine the incidence of HPSA in patients with LPRD, and to make a comparison of 2 treatment regimens that have been used based on the presence or absence of HPSA positivity in patients with LPRD. Persistent dry cough and a feeling of a lump in the throat (globus sensation) were the most frequent symptoms of LPRD, while posterior laryngeal inflammation was the main laryngoscopic finding. Results from the HPSA test were positive in 57% of the studied group. Patients with negative HPSA were treated with esomeprazole as single modality with a reported improvement score of 96.6%. Patients with positive HPSA test results were divided into 2 groups: 1 received only esomeprazole, with reported improvement in 40%, whereas the second group was treated with esomeprazole, plus amoxicillin sodium and clarithromycin (triple therapy) and reported a 90% incidence of symptom improvement. The incidence of HP infection in patients with LPRD in our study was 57%. Triple therapy showed a higher cure rate in patients with HPSA-positive test results.

  4. [Clinical Tests Testing New Therapies for Stargardt Disease].

    Kousal, B; Ďuďáková, Ľ; Hlavatá, L; Lišková, P

    2016-02-01

    To provide information on currently ongoing clinical trials for Stargardt disease. We have searched the clinical trial register (www.clinicaltrials.gov) for the keyword "Stargardt" and list active ongoing studies. There are currently eight registered clinical trials enrolling patients with Stargardt disease; all in phase I or II aiming at four mechanisms of action: inhibition of the production of vitamin A toxic dimers, gene therapy restoring wild type transcription of the ABCA4 gene, neuroprotection preventing retinal cells from oxidative damage, and replacement of the damaged retinal pigment epithelium using stem cell therapy. The basic prerequisite for enrolment in the vast majority of clinical trials is confirmation of the clinical diagnosis by mutational analysis. The wide variety of therapies that are registered as clinical trials for Stargardt disease significantly raises the possibility that effective treatments will be available in the near future for this currently incurable condition and that molecular genetic testing should be increasingly considered. Stargardt disease, clinical trial, ABCA4, mutation.

  5. Antibody response against gastrointestinal antigens in demyelinating diseases of the central nervous system

    Banati, M; Csecsei, P; Koszegi, E

    2013-01-01

    TG), intrinsic factor (IF), parietal cells (PC) and Saccharomyces cerevisiae (ASCA) were screened in the sera of 45 patients with AQP4-seropositive neuromyelitis optica (NMO) and NMO spectrum diseases (NMO/NMO-SD), 17 patients with AQP4-seronegative NMO, 85 patients with clinically definite multiple sclerosis...

  6. The Rate of Helicobacter pylori Seropositivity in a Group of Korean Patients with HLA-B27-Associated Acute Anterior Uveitis.

    Jeong Hun Bae

    Full Text Available To investigate an association between Helicobacter pylori seropositivity and HLA-B27-positive acute anterior uveitis (AAU in Korean patients.Retrospective analysis was performed with data from 106 patients previously diagnosed with AAU without clinical evidence of spondyloarthropathy. Serum immunoglobulin G antibodies to H. pylori were measured by enzyme-linked immunosorbent assay, and HLA typing was performed using polymerase chain reaction of DNA amplification. We included 72 non-uveitis patients and 35 age- and sex-matched healthy controls in the study.Of the 106 patients with AAU, 41 (38.7% were HLA-B27-positive, and 45 (42.5% were seropositive for H. pylori. Patients with HLA-B27-positive AAU had a significantly lower prevalence of H. pylori seropositivity compared to those with HLA-B27-negative AAU and healthy controls (24.4% vs. 53.8%, p = 0.003; 24.4% vs. 57.1%, p = 0.004, respectively. In the non-uveitis group, however, HLA-B27-positive patients exhibited similar H. pylori seropositivity prevalence to HLA-B27-negative patients and healthy controls (45.5% vs. 55.7%, p = 0.529; 45.5% vs. 57.1%, p = 0.497, respectively. In multivariate analysis, a low prevalence of H. pylori seropositivity was significantly associated with HLA-B27-positive AAU (odds ratio = 0.340, 95% confidence interval 0.135-0.855, p = 0.022.Our results suggest an inverse association between H. pylori seropositivity and HLA-B27-positive AAU. Further investigation of this association is needed, given the low prevalence of H. pylori seropositivity observed in patients with HLA-B27-positive AAU.

  7. Oxidative stress treatment for clinical trials in neurodegenerative diseases.

    Ienco, Elena Caldarazzo; LoGerfo, Annalisa; Carlesi, Cecilia; Orsucci, Daniele; Ricci, Giulia; Mancuso, Michelangelo; Siciliano, Gabriele

    2011-01-01

    Oxidative stress is a metabolic condition arising from imbalance between the production of potentially reactive oxygen species and the scavenging activities. Mitochondria are the main providers but also the main scavengers of cell oxidative stress. The role of mitochondrial dysfunction and oxidative stress in the pathogenesis of neurodegenerative diseases is well documented. Therefore, therapeutic approaches targeting mitochondrial dysfunction and oxidative damage hold great promise in neurodegenerative diseases. Despite this evidence, human experience with antioxidant neuroprotectants has generally been negative with regards to the clinical progress of disease, with unclear results in biochemical assays. Here we review the antioxidant approaches performed so far in neurodegenerative diseases and the future challenges in modern medicine.

  8. Five different tests of reaction time evaluated in HIV seropositive men.

    Dunlop, O; Bjørklund, R A; Abdelnoor, M; Myrvang, B

    1992-09-01

    In an attempt to develop a short neuropsychological test battery five different tests of reaction time were assessed according to their ability to discriminate between HIV seropositive men and healthy controls. In all tests a patient group with clinical symptoms was slower than the control group. In the complex reaction time test, which has a large cognitive aspect, even a clinically "asymptomatic" group was slower than the control group. The movement test, a new test with a large motor component, identified most slow responders, defining approximately half of the patients with clinical symptoms and one third of the "asymptomatic" patients as such. A test battery consisting of three tests is suggested for serial assessment and screening.

  9. Parkinsonian syndroms: Clinical phenotype, differential diagnosis and disease progression

    Storch, A.

    2002-01-01

    Parkinsonian syndromes include idiopathic Parkinson's disease (IPD), other neurodegenerative diseases with parkinsonism, the so-called atypical parkinsonian syndromes, and symptomatic parkinsonian syndromes, such as Wilson's disease. IPD is the most frequent disease with parkinsonism as the main clinical feature and is responsible for approx. 80% of all parkinsonian syndromes. Atypical parkinsonian syndromes are the most important differential diagnoses of IPD. The two most frequent types are multiple system atrophy (MSA) and progressive supranuclear palsy (PSP). For clinical diagnosis it is essential to take a careful medical history and to examine the patients physically in regular intervals. However, various clinico-pathological studies have shown that approx. 25% of patients with clinical diagnosis of IPD may have other causes of parkinsonism. Selected technical investigations, in particular functional imaging of the central dopaminergic system using PET or SPECT, may help to make clinical diagnosis more secure. This paper reviews the clinical features and diagnostic findings in diseases with parkinsonism and summarises the difficulties in establishing early and differential diagnoses. (orig.) [de

  10. Frequency of hepatitis B and C seropositivity in prisoners

    Fayyaz, M.; Qazi, M.A.

    2006-01-01

    This study was conducted on prisoners of Central Jail, Bahawalpur and on blood donors at Blood Bank Bahawal Victoria Hospital (BVD), Bahawalpur from 1st August 2005 to 30th November 2005. The intension was to study the sero prevalence of hepatitis Band C in prisoners and to note risk factor if any predisposing to the infection. All the prisoners of central jail Bahawalpur present during the study period, aged more than 18 years of either sex were included as the study group and a comparative group was blood donors presenting to blood bank BVH, Bahawalpur during this period. All the cases in study group (2086) as well as control group (9714) were screened for hepatitis B (HBs Ag) and hepatitis C (anti-HCV) infection detected on serum by One Step Test Device. All the positive cases were confirmed by Enzyme Linked Immunosorbent Assay. After an explanation, verbal consent was taken and a questionnaire was filled before taking samples from all the study group cases. On the basis of screening, prisoners were decided into seropositive group and sero-negative group. The different variable applied on both the groups were age, sex, occupation, residence, marital status, self shaving / by barber, family history of (H/o) jaundice, H/o blood trans-fusion, operation, tattooing and body piercing, drug abuse, same sex / extramarital sex and hepatitis B vaccination status. Total prisoners screened were 2086. Out of them 2072 were males (99.3%). Mean age was 34 years. Total seropositive cases were 249 (11.9%) While during the same period seropositivity in blood donors (control group) was 5.9%. Seropositivity of HBV was 5.6% in study group and 3.1% in control group (p<0.001), HCV was 6.3% Vs 2.8% (p<0.001), statistically very significant difference. Risk factors analysis among prisoners revealed that drug abuse was significantly associated with seropositivity (27% Vs 12%, p value <0.001). Among drug abusers, very high percentage of intravenous drug users was positive (89.2%, 25 out

  11. Pharmacogenetics in Neurodegenerative Diseases: Implications for Clinical Trials.

    Tortelli, Rosanna; Seripa, Davide; Panza, Francesco; Solfrizzi, Vincenzo; Logroscino, Giancarlo

    2016-01-01

    Pharmacogenetics has become extremely important over the last 20 years for identifying individuals more likely to be responsive to pharmacological interventions. The role of genetic background as a predictor of drug response is a young and mostly unexplored field in neurodegenerative diseases. Mendelian mutations in neurodegenerative diseases have been used as models for early diagnosis and intervention. On the other hand, genetic polymorphisms or risk factors for late-onset Alzheimer's disease (AD) or other neurodegenerative diseases, probably influencing drug response, are hardly taken into account in randomized clinical trial (RCT) design. The same is true for genetic variants in cytochrome P450 (CYP), the principal enzymes influencing drug metabolism. A better characterization of individual genetic background may optimize clinical trial design and personal drug response. This chapter describes the state of the art about the impact of genetic factors in RCTs on neurodegenerative disease, with AD, frontotemporal dementia, Parkinson's disease, amyotrophic lateral sclerosis, and Huntington's disease as examples. Furthermore, a brief description of the genetic bases of drug response focusing on neurodegenerative diseases will be conducted. The role of pharmacogenetics in RCTs for neurodegenerative diseases is still a young, unexplored, and promising field. Genetic tools allow increased sophistication in patient profiling and treatment optimization. Pharmaceutical companies are aware of the value of collecting genetic data during their RCTs. Pharmacogenetic research is bidirectional with RCTs: efficacy data are correlated with genetic polymorphisms, which in turn define subjects for treatment stratification. © 2016 S. Karger AG, Basel.

  12. Hematologic manifestations of Crohn's disease: two clinical cases

    O. V. Taratina

    2017-01-01

    Full Text Available Inflammatory bowel diseases (IBD are commonly associated with extraintestinal manifestations, hematological disorders being the most special among them. In some cases, they dominate the clinical picture masking the intestinal manifestations of the underlying disease. Aplastic anemia is an extremely rare extraintestinal IBD manifestation. There are only two clinical cases of aplastic anemia associated with ulcerative colitis and non with Crohn's disease reported in the literature. Combination of Crohn's disease and В₁₂-deficient anemia is more prevalent, but is seen usually only after more than 20 cm of the ileus has been resected. The first clinical case presented in this paper is a  combination of severe fistula-forming Crohn's disease with a constriction in the terminal part of the ileus and profound pancytopenia as an outcome of aplastic anemia. This profound pancytopenia is associated with an extremely high risk of life-threatening complications both of surgical treatment, as well as of several chemotherapeutic agents, which made the management of this patient difficult. The second clinical case demonstrates the manifestation of Crohn's disease as ileocolitis starting from the symptoms of cobalamin deficiency: severe В₁₂-deficient anemia, funicular myelosis and sensory ataxia, with blunted intestinal symptoms. This made the initial diagnosis and timely treatment difficult. Replacement therapy with cobalamin injections and treatment with glucocorticoids and antibacterials led to endoscopically confirmed remission of Crohn's disease and normalization of hematological parameters, with persistent polyneuropathy. Thus, management of patients with Crohn's disease should be multidisciplinary. In the case of anemia, leucopenia and/or thrombocytopenia in IBD patients it is necessary to exclude potential myelodysplasia and bone marrow aplasia. In the event of megaloblastic anemia and/or progressive polyneuropathy one should bear in mind

  13. Refractory coeliac disease in a country with a high prevalence of clinically-diagnosed coeliac disease.

    Ilus, T; Kaukinen, K; Virta, L J; Huhtala, H; Mäki, M; Kurppa, K; Heikkinen, M; Heikura, M; Hirsi, E; Jantunen, K; Moilanen, V; Nielsen, C; Puhto, M; Pölkki, H; Vihriälä, I; Collin, P

    2014-02-01

    Refractory coeliac disease (RCD) is thought to be a rare disorder, but the accurate prevalence is unknown. We aimed to identify the prevalence of and the risk factors for developing RCD in a Finnish population where the clinical detection rate of coeliac disease is high. The study involved 11 hospital districts in Finland where the number of treated RCD patients (n = 44), clinically diagnosed coeliac disease patients (n = 12 243) and adult inhabitants (n = 1.7 million) was known. Clinical characteristics at diagnosis of coeliac disease between the RCD patients and patients with uncomplicated disease were compared. The prevalence of RCD was 0.31% among diagnosed coeliac disease patients and 0.002% in the general population. Of the enrolled 44 RCD patients, 68% had type I and 23% type II; in 9% the type was undetermined. Comparing 886 patients with uncomplicated coeliac disease with these 44 patients that developed RCD later in life, the latter were significantly older (median 56 vs 44 years, P coeliac disease. Patients with evolving RCD had more severe symptoms at the diagnosis of coeliac disease, including weight loss in 36% (vs. 16%, P = 0.001) and diarrhoea in 54% (vs. 38%, P = 0.050). Refractory coeliac disease is very rare in the general population. Patients of male gender, older age, severe symptoms or seronegativity at the diagnosis of coeliac disease are at risk of future refractory coeliac disease and should be followed up carefully. © 2014 John Wiley & Sons Ltd.

  14. Review article. Predicting disease onset in clinically healthy people

    Zeliger . Harold I.

    2016-06-01

    Full Text Available Virtually all human disease is induced by oxidative stress. Oxidative stress, which is caused by toxic environmental exposure, the presence of disease, lifestyle choices, stress, chronic inflammation or combinations of these, is responsible for most disease. Oxidative stress from all sources is additive and it is the total oxidative stress from all sources that induces the onset of most disease. Oxidative stress leads to lipid peroxidation, which in turn produces Malondialdehyde. Serum malondialdehyde level is an additive parameter resulting from all sources of oxidative stress and, therefore, is a reliable indicator of total oxidative stress which can be used to predict the onset of disease in clinically asymptomatic individuals and to suggest the need for treatment that can prevent much human disease.

  15. AACE/ACE Disease State Clinical Review: Medical Management of Cushing Disease.

    Hamrahian, Amir H; Yuen, Kevin C J; Hoffman, Andrew R

    2014-07-01

    To review available medical therapies for patients with Cushing disease and to provide a roadmap for their use in clinical practice. PubMed searches were performed to identify all of the available published data on medical management of Cushing disease. Medical therapy is usually not the first-line treatment for patients with Cushing disease but may be used to improve clinical manifestations of Cushing disease in patients who are not suitable candidates for surgery, following unsuccessful surgery or recurrence, or as a "bridge therapy" in those who have undergone radiotherapy. Medical therapy may also be used in preoperative preparation of patients with severe disease. Current available medical options for patients with Cushing disease include centrally acting agents, steroidogenesis inhibitors, and a glucocorticoid receptor antagonists. At present, there are no head-to-head studies comparing the efficacy, tolerability, and safety of different U.S. Food and Drug Administration (FDA)- and non-FDA-approved drugs in patients with Cushing disease. With the initiation of new studies and the completion of ongoing clinical trials, the number of FDA-approved drugs for medical treatment of Cushing disease is expected to increase. Medical therapy has an important adjunctive role in the management of patients with Cushing disease. The decision to initiate medical treatment depends on many factors, including patient characteristics and preference. Long-term studies are needed to better define the clinical efficacy, safety, and tolerability of medical treatment of Cushing disease, including the role of combination therapies.

  16. [Advanced Parkinson's disease: clinical characteristics and treatment (part 1)].

    Kulisevsky, J; Luquin, M R; Arbelo, J M; Burguera, J A; Carrillo, F; Castro, A; Chacón, J; García-Ruiz, P J; Lezcano, E; Mir, P; Martinez-Castrillo, J C; Martínez-Torres, I; Puente, V; Sesar, A; Valldeoriola-Serra, F; Yañez, R

    2013-10-01

    A large percentage of patients with Parkinson's disease (PD) develop motor fluctuations, dyskinesias, and severe non-motor symptoms within 3 to 5 years of starting dopaminergic therapy, and these motor complications are refractory to treatment. Several authors refer to this stage of the disease as advanced Parkinson's disease. To define the clinical manifestations of advanced PD and the risk factors for reaching this stage of the disease. This consensus document has been prepared by using an exhaustive literature search and by discussion of the contents by an expert group on movement disorders of the Sociedad Española de Neurología (Spanish Neurology Society), coordinated by two of the authors (JK and MRL). Severe motor fluctuations and dyskinesias, axial motor symptoms resistant to levodopa, and cognitive decline are the main signs in the clinical phenotype of advanced PD. Copyright © 2013 Sociedad Española de Neurología. Published by Elsevier Espana. All rights reserved.

  17. Clinical application of SPECT and PET in cerebrovascular disease

    Ra, Young Shin

    2003-01-01

    Single photon emission computed tomography(SPECT) and positron emission tomography(PET) are modern imaging techniques that allow for both qualitative are quantitative assessment of hemodynamic changes in cerebrovascular diseases. SPECT has been becoming an indispensable method to investigate regional cerebral blood flow because equipment and isotope are easily available in most general hospitals. Acetazolamide stress SPECT has also been proved to be useful to evaluate the cerebrovascular reserve of occlusive cerebrovascular diseases and to select surgical candidate. PET has gained wide spread clinical use in the evaluation of the hemodynamic and metabolic consequences of extracranial or intracranial arterial obstructive disease despite its complexity and limited availability. PET has been established as an invaluable tool in the pathophysilogy investigation of acute ischemic stroke. The potentials, limitations, and clinical applications of SPECT and PET in various cerebrovascular diseases will be discussed in this article with reviews of literatures

  18. Clinical application of SPECT and PET in cerebrovascular disease

    Ra, Young Shin [Ulsan University College of Medicine, Seoul (Korea, Republic of)

    2003-02-01

    Single photon emission computed tomography(SPECT) and positron emission tomography(PET) are modern imaging techniques that allow for both qualitative are quantitative assessment of hemodynamic changes in cerebrovascular diseases. SPECT has been becoming an indispensable method to investigate regional cerebral blood flow because equipment and isotope are easily available in most general hospitals. Acetazolamide stress SPECT has also been proved to be useful to evaluate the cerebrovascular reserve of occlusive cerebrovascular diseases and to select surgical candidate. PET has gained wide spread clinical use in the evaluation of the hemodynamic and metabolic consequences of extracranial or intracranial arterial obstructive disease despite its complexity and limited availability. PET has been established as an invaluable tool in the pathophysilogy investigation of acute ischemic stroke. The potentials, limitations, and clinical applications of SPECT and PET in various cerebrovascular diseases will be discussed in this article with reviews of literatures.

  19. Whipple's disease. Report of five cases with different clinical features

    FERRARI,Maria de Lourdes de Abreu; VILELA,Eduardo Garcia; FARIA,Luciana Costa; COUTO,Claudia Alves; SALGADO,Célio Jefferson; LEITE,Virgínia Rios; BRASILEIRO FILHO,Geraldo; BAMBIRRA,Eduardo Alves; MENDES,Claudia Maria de Castro; CARVALHO,Silas de Castro; OLIVEIRA,Celso Affonso de; CUNHA,Aloísio Sales da

    2001-01-01

    Whipple's disease (WD) is a rare systemic disease of infectious etiology which involves the small intestine but can virtually affect any organ. We present here five cases (four males and one female) ranging in age from 20 to 59 years. All patients had intestinal involvement associated or not with clinical manifestations linked to this organ. Vegetation in the tricuspid valve was observed in one patient, suggesting endocarditis caused by Tropheryma whippelii, with disappearance of the echocard...

  20. Clinical Syndromes Associated with Cardiovascular Diseases: A Review

    Xing Sheng Yang, MD, PhD, FACC, FAHA

    2017-02-01

    Full Text Available In clinical practice, a variety of syndromes are associated with cardiovascular disease and have characteristic findings. Most of them are an autosomal dominant genetic disorder and have different types of cardiovascular abnormalities, including electrocardiographic conduction defects, arrhythmias, cardiomyopathy, vascular and valvular diseases, cardiac septal defects, and pulmonary problems. There is a growing need for physicians to pay more attention to these syndromes.

  1. Evaluation of medication treatment for Alzheimer's disease on clinical evidence

    Meng-qiu LI

    2014-03-01

    Full Text Available Objective To formulate the best treatment plan for Alzheimer's disease patients by evaluating the therapeutic efficacy and side effect of various evidence-based programs. Methods Alzheimer's disease, donepezil, rivastigmine, galantamine, memantine, rosiglitazone, etc. were defined as retrieval words. PubMed, Cochrane Library, Wanfang Data and China National Knowledge Infrastructure (CNKI databases were used with applying of manual searching. Systematic reviews, randomized controlled trials (RCT, controlled clinical trials and case-observation studies were collected and evaluated by Jadad Scale. Results After screening, 33 selected resources included 14 systematic reviews, 14 randomized controlled trials, 4 controlled clinical trials and 1 case-observation study. According to Jadad Scale, total 28 articles were evaluated to be high quality (12 with score 4, 10 score 5, 6 score 7, and 5 were low quality with score 3. It was summarized as follows: 1 Alzheimer's disease is a progressive neurodegenerative disease for which no cure exists. To date, only symptomatic treatments with cholinesterase inhibitors (donepezil, rivastigmine, galantamine and an N-methyl-D-aspartate (NMDA receptor noncompetitive antagonist (memantine, are effective and well tolerated to counterbalance the neurotransmitter disturbance, but cannot limit or impact on disease progression. 2 Disease modifying drug is an potential agent, with persistent effect on slowing the progression of structural damage, and can be detected even after withdrawing the treatment. Many types of disease modifying drugs are undergoing clinical trials. Conclusions Using evidence-based medicine methods can provide best clinical evidence on Alzheimer's disease treatment. doi: 10.3969/j.issn.1672-6731.2014.03.009

  2. Motor outcome measures in Huntington disease clinical trials.

    Reilmann, Ralf; Schubert, Robin

    2017-01-01

    Deficits in motor function are a hallmark of Huntington disease (HD). The Unified Huntington's Disease Rating Scale Total Motor Score (UHDRS-TMS) is a categoric clinical rating scale assessing multiple domains of motor disability in HD. The UHDRS-TMS or subsets of its items have served as primary or secondary endpoints in numerous clinical trials. In spite of a well-established video-based annual online certification system, intra- and interrater variability, subjective error, and rater-induced placebo effects remain a concern. In addition, the UHDRS-TMS was designed to primarily assess motor symptoms in manifest HD. Recently, advancement of technology resulted in the introduction of the objective Q-Motor (i.e., Quantitative-Motor) assessments in biomarker studies and clinical trials in HD. Q-Motor measures detected motor signs in blinded cross-sectional and longitudinal analyses of manifest, prodromal, and premanifest HD cohorts up to two decades before clinical diagnosis. In a multicenter clinical trial in HD, Q-Motor measures were more sensitive than the UHDRS-TMS and exhibited no placebo effects. Thus, Q-Motor measures are currently explored in several multicenter trials targeting both symptomatic and disease-modifying mechanisms. They may supplement the UHDRS-TMS, increase the sensitivity and reliability in proof-of-concept studies, and open the door for phenotype assessments in clinical trials in prodromal and premanifest HD. Copyright © 2017 Elsevier B.V. All rights reserved.

  3. MDS clinical diagnostic criteria for Parkinson's disease in China.

    Li, Jun; Jin, Miao; Wang, Li; Qin, Bin; Wang, Kang

    2017-03-01

    The Movement Disorder Society Clinical Diagnostic Criteria for Parkinson's disease (MDS-PD Criteria) was introduced by the Movement Disorder Society in 2015 for research purposes. However, its use for clinical diagnosis of Parkinson disease still needs further revision. This study compares the UK-Criteria versus MDS-PD Criteria in the clinical diagnosis of Parkinson disease referred to the China-Japan Friendship Hospital of Beijing, China. To compare the MDS-PD Criteria with the UK-Criteria and discuss the feasibility of the clinical application of MDS-PD Criteria as a general guide to clinical diagnosis of PD in Chinese PD patients. 150 patients of neurology clinic of China-Japan Friendship Hospital of Beijing were recruited in our research. They were divided into three groups: UK-Criteria group, MDS-PD Criteria group and a combined group of UK and MDS-PD Criteria. Clinical history was collected while physical and auxiliary examinations were done by a trained neurologist according to the corresponding criteria. An interrater reliability analysis using the Kappa statistic claimed substantial agreement (κ = 0.626) between the MDS-PD Criteria and the UK-Criteria. The differences between the diagnostic results of these two criteria were statistically significant by paired Chi-square test (p = 0.000). It was found that levodopa-induced dyskinesia had a good positive predictive value, while early bulbar impairment and inspiratory dysfunction presented a negative predictive value. The MDS-PD Criteria emphasize the importance of non-motor symptoms, keeping the motor symptoms as the core for the clinical diagnosis of PD, and establish categories of diagnosis features and levels of certainty which are more complete and organized to be used and replicated by non specialized physicians to evaluated patients with Parkinsonism. The higher sensitivity of MDS-PD Criteria compared with UK-Criteria is worth being widely used in clinical work.

  4. Genetics of liver disease: From pathophysiology to clinical practice.

    Karlsen, Tom H; Lammert, Frank; Thompson, Richard J

    2015-04-01

    Paralleling the first 30 years of the Journal of Hepatology we have witnessed huge advances in our understanding of liver disease and physiology. Genetic advances have played no small part in that. Initial studies in the 1970s and 1980s identified the strong major histocompatibility complex associations in autoimmune liver diseases. During the 1990 s, developments in genomic technologies drove the identification of genes responsible for Mendelian liver diseases. Over the last decade, genome-wide association studies have allowed for the dissection of the genetic susceptibility to complex liver disorders, in which also environmental co-factors play important roles. Findings have allowed the identification and elaboration of pathophysiological processes, have indicated the need for reclassification of liver diseases and have already pointed to new disease treatments. In the immediate future genetics will allow further stratification of liver diseases and contribute to personalized medicine. Challenges exist with regard to clinical implementation of rapidly developing technologies and interpretation of the wealth of accumulating genetic data. The historical perspective of genetics in liver diseases illustrates the opportunities for future research and clinical care of our patients. Copyright © 2015 European Association for the Study of the Liver. Published by Elsevier B.V. All rights reserved.

  5. Clinical research of bone scan characteristics for metabolic bone diseases

    Zhu Ruisen; Luo Qiong; Lu Haikui; Chen Libo; Luo Quanyong

    2009-01-01

    Characteristic images of 99m Tc-MDP bone scintigraphy in patients with metabolic bone diseases (MBD) were analyzed and compared, in an attempt to improve the capability of differential diagnosis in this aspect. A total of 142 cases, clinically confirmed as (MBD), were categorized into six groups: hyperparathyroidism (117), renal osteodystrophy (4), Paget's disease (16), hypophosphatemic osteomalacia (2), Albers-Schonberg disease (2), and Brittle bone disease (1). They were diagnosed clinically or pathologically, and scanned with 99m Tc-MDP bone scintegraphy, from which the 142 MBD cases were classified into 4 types. The cases of Type I had increased amount of 99m Tc-MDP uptake in whole body bones, including hyperparathyroidism, Albers-Schonberg disease, brittle bone disease and renal osteodystrophy. The cases of Type II had high uptake of 99m Tc-MDP in local region of bones, including paget's disease, hypophosphatemic osteomalacia and hyperparathyroidism. A Type I case with pathological fracture or secondary osteopathy was classified as Type III. Type IV cases were in early stage of hyperparathyroidism, with normal bone scan image. Analysis of the characteristics of 99m Tc-MDP bone scintigraphic findings (locations, morphology and intensities) in patients with MBD may be helpful in the differential diagnosis of MBD, in association with the patient's history and X-ray data altogether. (authors)

  6. Periodontal disease in pregnant patients with rheumatic valvular disease: clinical and microbiological study.

    Avila, Walkiria Samuel; Timerman, Lilia; Romito, Giuseppe Alexandre; Marcelino, Sílvia Linard; Neves, Itamara Lúcia Itagiba; Zugaib, Marcelo; Grinberg, Max

    2011-04-01

    The periodontal disease during pregnancy of women with rheumatic valve disease imply infective endocarditis risks and higher rate of preterm birth and low birth weight. To study the periodontal disease rate of women with rheumatic valve disease during pregnancy. We studied 140 pregnant women who included 70 patients with rheumatic valve disease and 70 healthy women. The periodontal examination included: 1) periodontal clinical exam regard the follow variables: a) probing depth; b) gingival margin; c) clinical attachment level; d) bleeding on probing; e) plaque index and f) gingival index; and 2) microbiological test was performed in samples serum and gingival crevicular fluid and considered positive controls to Porphyromonas gingivalis, Tannerella forsithia e Aggregobacter actinomycetemcomitans. Age and parity were similar between groups; as single or combined the mitral valve disease was prevalent among the rheumatic valve lesion in 45 (32.1%) e 20 (28.5%) cases, respectively. Among the periodontal variables gingival margin (p=0.01) and plaque index (p=0.04) were different between groups. The periodontal disease was identified in 20 (14,3%) pregnant women, seven (10%) of them were patients with valve rheumatic disease and the remain 13 (18,6%) were healthy women, its percentual was not different between groups (p=0,147). Microbiological analyses of oral samples showed higher percentual of P. gingivalis in healthy pregnant women (p=0.004). The clinical and microbiological study during pregnancy showed comparable incidence of periodontal disease between women with rheumatic valve disease and healthy women.

  7. Celiac disease in non-clinical populations of Japan.

    Fukunaga, Mai; Ishimura, Norihisa; Fukuyama, Chika; Izumi, Daisuke; Ishikawa, Nahoko; Araki, Asuka; Oka, Akihiko; Mishiro, Tomoko; Ishihara, Shunji; Maruyama, Riruke; Adachi, Kyoichi; Kinoshita, Yoshikazu

    2018-02-01

    Celiac disease is a chronic autoimmune enteropathy caused by gluten ingestion. While its prevalence in Western countries is reported to be as high as 1%, the prevalence has not been evaluated in a large-scale study of a Japanese population. The aim of our study was to clarify the possible presence of celiac disease in a Japanese non-clinical population as well as in patients showing symptoms suggestive of the disease. Serum samples were collected from 2008 non-clinical adults and 47 patients with chronic unexplained abdominal symptoms between April 2014 and June 2016. The anti-tissue transglutaminase (TTG) immunoglobulin A antibody titer was determined as a screening test for celiac disease in all subjects, and individuals with a value of >2 U/mL subsequently underwent testing for the presence of serum endomysial IgA antibody (EMA) as confirmation. Those testing positive for EMA or with a high concentration (>10 U/mL) of TTG were further investigated by histopathological examinations of duodenal mucosal biopsy specimens and HLA typing tests. Of the 2008 non-clinical adults from whom serum samples were collected, 161 tested positive for TTG, and all tested negative for EMA. Four subjects who had a high TTG titer were invited to undergo confirmatory testing, and the histopathological results confirmed the presence of celiac disease in only a single case (0.05%). Of the 47 symptomatic patients, one (2.1%) was found to have a high TTG titer and was diagnosed with celiac disease based on duodenal histopathological findings. The presence of celiac disease in a non-clinical Japanese population was low at 0.05% and was rarely found in patients with unexplained chronic abdominal symptoms.

  8. Can we Predict Disease Course with Clinical Factors?

    Vegh, Zsuzsanna; Kurti, Zsuzsanna; Golovics, Petra A; Lakatos, Peter L

    2018-01-01

    The disease phenotype at diagnosis and the disease course of Crohn's disease (CD) and ulcerative colitis (UC) show remarkable heterogeneity across patients. This review aims to summarize the currently available evidence on clinical and some environmental predictive factors, which clinicians should evaluate in the everyday practice together with other laboratory and imaging data to prevent disease progression, enable a more personalized therapy, and avoid negative disease outcomes. In recent population-based epidemiological and referral cohort studies, the evolution of disease phenotype of CD and UC varied significantly. Most CD and severe UC patients still require hospitalization or surgery/colectomy during follow-up. A change in the natural history of inflammatory bowel diseases (IBD) with improved outcomes in parallel with tailored positioning of aggressive immunomodulator and biological therapy has been suspected. According to the currently available literature, it is of major importance to refer IBD cases at risk for adverse disease outcomes as early during the disease course as possible. Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.org.

  9. Chlamydia trachomatis immunoglobulin G3 seropositivity is a predictor of reproductive outcomes in infertile women with patent fallopian tubes.

    Steiner, Anne Z; Diamond, Michael P; Legro, Richard S; Schlaff, William D; Barnhart, Kurt T; Casson, Peter R; Christman, Gregory M; Alvero, Ruben; Hansen, Karl R; Geisler, William M; Thomas, Tracey; Santoro, Nanette; Zhang, Heping; Eisenberg, Esther

    2015-12-01

    To determine if Chlamydia trachomatis (C. trachomatis) seropositivity, as detected by the C. trachomatis elementary body (EB)-based enzyme-linked immunosorbent assay [EB ELISA] predicts pregnancy and pregnancy outcome among infertile women with documented tubal patency. Cohort study. Outpatient clinics. In all, 1,250 infertile women with documented tubal patency enrolled in 1 of 2 randomized controlled trials: Pregnancy in Polycystic Ovary Syndrome II; and the Assessment of Multiple Intrauterine Gestations From Ovarian Stimulation. Sera were analyzed for anti-C. trachomatis immunoglobulin G (IgG)1 and IgG3 antibodies, using a research C. trachomatis EB ELISA. The optical density (OD)405 readings of ≥ 0.35 and ≥ 0.1 were considered positive for IgG1 and IgG3, respectively. Primary outcomes included pregnancy, live birth, and ectopic pregnancy. Log-linear regression was used to determine the relative risk after adjusting for age, race, treatment medication, smoking status, and current alcohol use. A total of 243 (19%) women were seropositive for anti-C. trachomatis IgG3. They tended to be nonwhite and smokers. Anti-C. trachomatis IgG3 seropositive women were significantly less likely to conceive (risk ratio [RR] 0.65, 95% confidence interval [CI] 0.52-0.83) or to have a live birth (RR 0.59, 95% CI 0.43-0.80); these associations were weakened after adjusting for number of hysterosalpingography-documented patent tubes (RR 0.73, 95% CI 0.56-0.97) and (RR 0.73, 95% CI 0.50-1.04), respectively. Anti-C. trachomatis IgG3 seropositive women who conceived had a ×2.7 risk (95% CI 1.40-5.34) of ectopic pregnancy. Even in the presence of tubal patency, anti-C. trachomatis IgG3 seropositivity is associated with a lower likelihood of pregnancy. Anti-C. trachomatis IgG3 seropositive women have as high as 3 times the risk of ectopic pregnancy. PPCOSII: NCT00719186 and AMIGOS: NCT01044862. Copyright © 2015 American Society for Reproductive Medicine. All rights reserved.

  10. NON-COMMUNICABLE DISEASE PROGRAM IN AMPANGAN HEALTH CLINIC

    MASTURA I

    2010-01-01

    Full Text Available Non-communicable diseases (NCDs represent among the most common and debilitating conditions seen in primary care. Patients’ care will often involves multiple providers and follow-up requires persistence by patients and clinicians alike, therefore ideal outcomes are often difficult to achieve. The need for better disease management policies and practice is growing. This is due to the changing demographic profile of the population, the increasing cost of managing people in acute care hospitals and the availability of new technologies and services. All these changes enable a different care paradigm which is more cost effective and provides people with chronic conditions an improved quality of life. Management of the NCDs therefore offers an excellent opportunity to practice chronic disease management - a systems approach designed to ensure excellent care. The NCD team has developed a comprehensive approach to chronic disease care. We would like to describe the NCD Program in Ampangan Health Clinic which represents many typical government health clinics in Malaysia and the processes by which it was developed. Included are specific examples of the tools and how they can be used by individual clinicians incaring for patients. The integration of Chronic Disease Management Services into health care systems is the direction being undertaken to tackle the burden of chronic disease. Disease management supports the shift in healthcare from an emphasis on managing the acute episode to managing the entire disease course, highlighting both prevention and maintenance of wellbeing for patients with chronic diseases. Disease management promotes better integration and coordination of care across all aspects of the health sector.

  11. Quantitation of autoantibodies in systemic autoimmune diseases : clinically useful?

    Kallenberg, C. G. M.; Stegeman, C. A.; Bootsma, H.; Biji, M.; Limburg, P. C.

    2006-01-01

    Serial assessment of levels of autoantibodies has been proposed as being clinically useful in certain systemic autoimmune diseases. In particular, attention has been given to anti-dsDNA antibodies in systemic lupus erythematosus (SLE) and ANCA in the ANCA-associated vasculitides (AAV). Much

  12. DNA-abzymes in autoimmune diseases in clinic and experiment

    T E Naumova

    2003-01-01

    Full Text Available DNA-abzymes enzymes in autoimmune diseases in clinic and experiment T.E. Naumova, O.M. Durova, A.G. Gabibov, Z.S. Alekberova, S. V. Suchkov DNA-hydrolyzing autoantibodies (AAB or DNA-abzymes can be found in autoimmune diseases in clinic and experiment. Technology of serum express screening for presence of DNA abzymes is described. Comparative study of DNA-hydrolising activity in patients with different forms of systemic and organ-specific autoimmune diseases was performed. Blood of clinically healthy donors was usually free of IgG DNA-abzymes. DNA-abzymes were most often revealed in patients with systemic lupus erythematosus (SLE and rheumatoid arthritis (RA less often in patients with organ-specific forms of autoimmune disturbances. The results of the study confirm the hypothesis of autoimmune origin of IgG DNA abzymes and demonstrate the possibility to use them in clinical practice for monitoring to disease activity in SLE and RA.

  13. Clinical surgical and pathological characterization of nodular thyroid disease

    Gonzalez Mendoza, Dayanis Ceila; Rodriguez Valdes, Adriana; Bejerano Garcia, Ramiro Julio

    2012-01-01

    Nodular thyroid disease is a worrisome endocrine problem due to its association with cancer. To characterize patients hospitalized with this condition according to clinical surgical and pathological aspects, as well as to determine the effectiveness of the cytology by fine-needle aspiration biopsy for diagnosis

  14. Childhood Pompe disease: clinical spectrum and genotype in 31 patients

    Capelle, C.I. van; Meijden, J.C. van der; Hout, J.M. van den; Jaeken, J.; Baethmann, M.; Voit, T.; Kroos, M.A.; Derks, T.G.; Rubio-Gozalbo, M.E.; Willemsen, M.A.A.P.; Lachmann, R.H.; Mengel, E.; Michelakakis, H.; Jongste, J.C. de; Reuser, A.J.; Ploeg, A.T. van der

    2016-01-01

    BACKGROUND: As little information is available on children with non-classic presentations of Pompe disease, we wished to gain knowledge of specific clinical characteristics and genotypes. We included all patients younger than 18 years, who had been evaluated at the Pompe Center in Rotterdam, the

  15. Childhood Pompe disease: Clinical spectrum and genotype in 31 patients

    C.I. van Capelle (Carine); J.C. van der Meijden (Chris); J.M.P. van den Hout (Johanna); J. Jaeken; M. Baethmann; T. Voit; M.A. Kroos (Marian); T.G.J. Derks (Terry G J); M.E. Rubio-Gozalbo (Estela); M.A. Willemsen (Michél); R. Lachmann (Robin); E. Mengel; H. Michelakakis (Helen); J.C. de Jongste (Johan); A.J.J. Reuser (Arnold); A.T. van der Ploeg (Ans)

    2016-01-01

    textabstractBackground: As little information is available on children with non-classic presentations of Pompe disease, we wished to gain knowledge of specific clinical characteristics and genotypes. We included all patients younger than 18 years, who had been evaluated at the Pompe Center in

  16. Childhood Pompe disease : clinical spectrum and genotype in 31 patients

    van Capelle, C I; van der Meijden, J C; van den Hout, J M P; Jaeken, J; Baethmann, M; Voit, T; Kroos, M A; Derks, T G J; Rubio-Gozalbo, M E; Willemsen, M A; Lachmann, R H; Mengel, E; Michelakakis, H; de Jongste, J C; Reuser, A J J; van der Ploeg, A T

    2016-01-01

    Background: As little information is available on children with non-classic presentations of Pompe disease, we wished to gain knowledge of specific clinical characteristics and genotypes. We included all patients younger than 18 years, who had been evaluated at the Pompe Center in Rotterdam, the

  17. Clinical presentation and mutations in Danish patients with Wilson disease

    Møller, Lisbeth Birk; Horn, Nina; Jeppesen, Tina Dysgaard

    2011-01-01

    This study describes the clinical presentation and diagnosis in all Danish patients (49, 41 unrelated) with Wilson disease (WND). On the basis of the number of diagnosed patients from 1990-2008, the prevalence was estimated to be 1:49¿500. Among routinely used diagnostic tests, none were...

  18. Clinical presentation and mutations in Danish patients with Wilson disease

    Møller, Lisbeth Birk; Horn, Nina; Jeppesen, Tina Dysgaard

    2011-01-01

    This study describes the clinical presentation and diagnosis in all Danish patients (49, 41 unrelated) with Wilson disease (WND). On the basis of the number of diagnosed patients from 1990-2008, the prevalence was estimated to be 1:49 500. Among routinely used diagnostic tests, none were...

  19. Variability in the clinical expression of Parkinson's disease

    Wolters, E.C.

    2008-01-01

    Parkinsonism is a clinical syndrome characterized by bradykinesia, hypo-/akinesia, muscular rigidity, and resting tremor, mainly caused by Parkinson's disease (PD). Symptoms of PD are due to a progressive loss of nigral neurons causing striatal dopaminergic denervation. However, nigral degeneration

  20. The clinical use of structural MRI in Alzheimer disease

    Frisoni, G.B.; Fox, N.C.; Jack, C.R.; Scheltens, P.; Thompson, P.M.

    2010-01-01

    Structural imaging based on magnetic resonance is an integral part of the clinical assessment of patients with suspected Alzheimer dementia. Prospective data on the natural history of change in structural markers from preclinical to overt stages of Alzheimer disease are radically changing how the

  1. Sickle cell disease clinical phenotypes in children from South ...

    2014-07-20

    Jul 20, 2014 ... Background:The clinical phenotypes of children with sickle cell disease (SCD) are poorly described in many sub-Saharan countries ..... World Health Organization. ... apps.who.int/gb/ebwha/pdf_files/WHA59/A59_9‑en.pdf.

  2. Updated clinical diagnostic criteria for sporadic Creutzfeldt-Jakob disease

    I. Zerr; K. Kallenberg; D.M. Summers; C. Romero; A. Taratuto; U. Heinemann; M. Breithaupt; D. Varges; B. Meissner; A. Ladogana (Anna); M. Schuur (Maaike); S. Haik; S.J. Collins (Steven); G.H. Jansen (Gerard); G.B. Stokin; J. Pimentel; E. Hewer; D. Collie; P. Smith; H. Roberts; J.P. Brandel; P. Tikka-Kleemola (Päivi); M. Pocchiari (Maurizio); C. Begue; P. Cras (Patrick); R.G. Will; P. Sanchez-Juan (Pascual)

    2009-01-01

    textabstractSeveral molecular subtypes of sporadic Creutzfeldt-Jakob disease have been identified and electroencephalogram and cerebrospinal fluid biomarkers have been reported to support clinical diagnosis but with variable utility according to subtype. In recent years, a series of publications

  3. Correlation of clinical data, anatomical site and disease stage in ...

    Objective: To evaluate the colorectal cancer clinical data with respect to the anatomical location and stage of disease. Design: Retrospective observational study. Setting: Kenyatta National Hospital (KNH), Nairobi, Kenya. Subjects: Two hundred and fifty three tumours were categorised as right colonic (RCC), left colonic ...

  4. Hotspots in clinical management of severe liver diseases

    LYU Jiayu

    2017-09-01

    Full Text Available Severe liver diseases such as liver failure and acute decompensated cirrhosis have critical conditions and high mortality rates, and the prognosis of such patients is closely associated with early warning, timely dynamic assessment, and comprehensive and effective therapy. The patients require a series of effective clinical management measures for elimination of causative factors, organ support, and prevention and treatment of complications. Medical treatment-artificial liver-liver transplantation is an important modality for severe liver diseases. Granulocyte colony-stimulating factor, stem cell therapy, and bioartificial liver have a promising future, while there are still controversies over non-selective β-blocker. This article reviews the hotspots in the clinical management of severe liver diseases.

  5. Autoinflammatory diseases in adults. Clinical characteristics and prognostic implications.

    González García, A; Patier de la Peña, J L; Ortego Centeno, N

    2017-03-01

    Autoinflammatory diseases are clinical conditions with inflammatory manifestations that present in a periodic or persistent manner and are caused by acquired or hereditary disorders of the innate immune response. In general, these diseases are more common in childhood, but cases have been reported in adults and are therefore important for all specialists. There are few references on these diseases in adults due to their low prevalence and underdiagnosis. The aim of this study is to review the scientific literature on these disorders to systematise their clinical, prognostic and treatment response characteristics in adults. Copyright © 2016 Elsevier España, S.L.U. and Sociedad Española de Medicina Interna (SEMI). All rights reserved.

  6. Safety and Immunogenicity of a Recombinant Adenovirus Serotype 35-Vectored HIV-1 Vaccine in Adenovirus Serotype 5 Seronegative and Seropositive Individuals.

    Fuchs, Jonathan D; Bart, Pierre-Alexandre; Frahm, Nicole; Morgan, Cecilia; Gilbert, Peter B; Kochar, Nidhi; DeRosa, Stephen C; Tomaras, Georgia D; Wagner, Theresa M; Baden, Lindsey R; Koblin, Beryl A; Rouphael, Nadine G; Kalams, Spyros A; Keefer, Michael C; Goepfert, Paul A; Sobieszczyk, Magdalena E; Mayer, Kenneth H; Swann, Edith; Liao, Hua-Xin; Haynes, Barton F; Graham, Barney S; McElrath, M Juliana

    2015-05-01

    Recombinant adenovirus serotype 5 (rAd5)-vectored HIV-1 vaccines have not prevented HIV-1 infection or disease and pre-existing Ad5 neutralizing antibodies may limit the clinical utility of Ad5 vectors globally. Using a rare Ad serotype vector, such as Ad35, may circumvent these issues, but there are few data on the safety and immunogenicity of rAd35 directly compared to rAd5 following human vaccination. HVTN 077 randomized 192 healthy, HIV-uninfected participants into one of four HIV-1 vaccine/placebo groups: rAd35/rAd5, DNA/rAd5, and DNA/rAd35 in Ad5-seronegative persons; and DNA/rAd35 in Ad5-seropositive persons. All vaccines encoded the HIV-1 EnvA antigen. Antibody and T-cell responses were measured 4 weeks post boost immunization. All vaccines were generally well tolerated and similarly immunogenic. As compared to rAd5, rAd35 was equally potent in boosting HIV-1-specific humoral and cellular immunity and responses were not significantly attenuated in those with baseline Ad5 seropositivity. Like DNA, rAd35 efficiently primed rAd5 boosting. All vaccine regimens tested elicited cross-clade antibody responses, including Env V1/V2-specific IgG responses. Vaccine antigen delivery by rAd35 is well-tolerated and immunogenic as a prime to rAd5 immunization and as a boost to prior DNA immunization with the homologous insert. Further development of rAd35-vectored prime-boost vaccine regimens is warranted.

  7. Clinical presentation of late haemorrhagic disease of newborn

    Majeed, R.; Memon, Y.; Majeed, F.

    2008-01-01

    To observe the clinical presentation of late haemorrhagic disease of the newborn (LHDNB), and clinical improvement after the administration of vitamin K/sub 1/. This is a prospective descriptive study. All the children older than seven days who presented with bleeding were admitted in pediatrics ward of Isra University Hyderabad from April 2006 to April 2007 were included. Data collection was done by means of detailed proforma. Analysis was done on SPSS version 11. Thirty five cases were included. Commonest site of bleeding was subcutaneous followed by oral and injection site. Mean age of late haemorrhagic disease of newborn was 109 days and minimum age of presentation was 28 days. Common clinical presentations were irritability, convulsions, poor reflexes and poor feeding. Mostly recovery was within 24 hours after vit K. Late HDN results in severe hemorrhage especially hemorrhage in the central nervous system. Administration of Vitamin K (1mg, 1M) at birth can present these severe complications. (author)

  8. Cranial Paget's disease - clinical case of symptomatic secondary basilar impression

    Gagov, E.; Gabrovsky, N.; Gabrovsky, S.

    2010-01-01

    A clinical case of 52 years old woman with history of periodic headaches for many years. The headache became more intensive and constant during the last 4-6 months. Instability by walking and stagger occurred as well as weakness in all 4 extremities, difficult swallowing and speech changes. Bulbar, quadripyramidal and archicerebellar symptoms were in hand. Pagets disease was ascertained engaging the skull with secondary basilar impression and compression of the cerebellum and the brain-stem leading to the above described clinical signs. Decompressive median suboccipital craniectomy was performed with laminectomy of C1. Occipital squama was thickened and highly vascularized.. Secondary basilar impression could occur in cranial Pagets disease with clinical symptoms resulting from the compression of the cerebellum and the brain-stem

  9. Clinical evaluation of oropharyngeal dysphagia in Machado-Joseph disease

    Sabrina Mello Alves Corrêa

    2010-12-01

    Full Text Available CONTEXT: In Machado-Joseph disease, poor posture, dystonia and peripheral neuropathy are extremely predisposing to oropharyngeal dysphagia, which is more commonly associated with muscular dystrophy. OBJECTIVE: To evaluate the clinical characteristics of oropharyngeal dysphagia in Machado-Joseph disease patients. METHOD: Forty individuals participated in this study, including 20 with no clinical complaints and 20 dysphagic patients with Machado-Joseph disease of clinical type 1, who were all similar in terms of gender distribution, average age, and cognitive function. The medical history of each patient was reviewed and each subject underwent a clinical evaluation of deglutition. At the end, the profile of dysphagia in patients with Machado-Joseph disease was classified according to the Severity Scale of Dysphagia, as described by O'Neil and collaborators. RESULTS: Comparison between dysphagic patients and controls did not reveal many significant differences with respect to the clinical evaluation of the oral phase of deglutition, since afflicted patients only demonstrated deficits related to the protrusion, retraction and tonus of the tongue. However, several significant differences were observed with respect to the pharyngeal phase. Dysphagic patients presented pharyngeal stasis during deglutition of liquids and solids, accompanied by coughing and/or choking as well as penetration and/or aspiration; these signs were absent in the controls. CONCLUSIONS: Oropharyngeal dysphagia is part of the Machado-Joseph disease since the first neurological manifestations. There is greater involvement of the pharyngeal phase, in relation to oral phase of the deglutition. The dysphagia of these patients is classified between mild and moderate.

  10. Clinical trial network for the promotion of clinical research for rare diseases in Japan: muscular dystrophy clinical trial network.

    Shimizu, Reiko; Ogata, Katsuhisa; Tamaura, Akemi; Kimura, En; Ohata, Maki; Takeshita, Eri; Nakamura, Harumasa; Takeda, Shin'ichi; Komaki, Hirofumi

    2016-07-11

    Duchenne muscular dystrophy (DMD) is the most commonly inherited neuromuscular disease. Therapeutic agents for the treatment of rare disease, namely "orphan drugs", have recently drawn the attention of researchers and pharmaceutical companies. To ensure the successful conduction of clinical trials to evaluate novel treatments for patients with rare diseases, an appropriate infrastructure is needed. One of the effective solutions for the lack of infrastructure is to establish a network of rare diseases. To accomplish the conduction of clinical trials in Japan, the Muscular dystrophy clinical trial network (MDCTN) was established by the clinical research group for muscular dystrophy, including the National Center of Neurology and Psychiatry, as well as national and university hospitals, all which have a long-standing history of research cooperation. Thirty-one medical institutions (17 national hospital organizations, 10 university hospitals, 1 national center, 2 public hospitals, and 1 private hospital) belong to this network and collaborate to facilitate clinical trials. The Care and Treatment Site Registry (CTSR) calculates and reports the proportion of patients with neuromuscular diseases in the cooperating sites. In total, there are 5,589 patients with neuromuscular diseases in Japan and the proportion of patients with each disease is as follows: DMD, 29 %; myotonic dystrophy type 1, 23 %; limb girdle muscular dystrophy, 11 %; Becker muscular dystrophy, 10 %. We work jointly to share updated health care information and standardized evaluations of clinical outcomes as well. The collaboration with the patient registry (CTSR), allows the MDCTN to recruit DMD participants with specific mutations and conditions, in a remarkably short period of time. Counting with a network that operates at a national level is important to address the corresponding national issues. Thus, our network will be able to contribute with international research activity, which can lead to

  11. Study on clinical symptoms in canine cardiac diseases

    F. Karlette Anne

    Full Text Available Cardiac diseases in canines are an extensively studied phenomenon all over the world but meagre information has been reported in India. Certain problems, including historical, physical, and laboratory abnormalities, are associated with cardiovascular or pulmonary disease. In India however, the recognition of canine cardiac diseases has been delayed, and ignored on account of lack of awareness and knowledge by the owner and inadequate diagnostic facility to a field veterinarian. Considering the above facts, the present study was undertaken in Gujarat to survey the prevalence of common cardiac diseases in hospital population of dogs along with the clinical symptoms which often goes undetected due to lack of proper diagnostic techniques to be implied and the most forms of heart disease may be present for many years before any evidence of failure develops. In the present study most of the clinical cases of cardiac diseases were presented with a history of nocturnal coughing (seven cases; 2.55%, exercise intolerance (five cases; 1.82%, partial or complete anorexia (five cases; 1.82%, swelling in abdominal area (four cases; 1.45%, dullness and depression (two cases; 0.72%, cachexia and hepatojugular pulsation (one case each; 0.36% each at times. [Vet World 2009; 2(8.000: 307-309

  12. IMPULSIVE-COMPULSIVE DISORDERS IN PARKINSON'S DISEASE. CLINICAL CASES

    N. V. Fedorova

    2015-01-01

    Full Text Available Objective – a description of clinical cases of impulsive­compulsive disorders in Parkinson,s disease. The first clinical case. Patient N., 75 years old, suffering for 15 years from Parkinson,s disease, akinetic­rigid form of the disease, stage 4 by Hyun–Yar. Since 2009, he received levodopa/carbidopa 250/25 mg 5 times/day (daily dose of 1250 mg of levodopa; pramipexole 3.5 mg per day (daily dose 3.5 mg, amantadine sulfate 100 mg 5 times/day (daily dose 500 mg. While taking antiparkinsonian drugs the patient developed behavioral disorders such as dopamine disregulatory syndrome combined with punding, hypersexuality and compulsive shopping accompanied by visual hallucinations. The total equivalent dose of levodopa was 1600 mg per day. The second clinical case. Patient R., 52 years old, suffers from Parkinson,s disease about 5 years, a mixed form. She complained of slowness of movement, tremor in her left hand, sleep disturbances, poor mood. The clinic was appointed piribedil 50 mg 3 times per day. Despite the fact that the patient took only one of dopaminergic drugs in a therapeutic daily dose, she developed impulsive­compulsive disorder as hyper­ sexuality, compulsive shopping and binge eating. Results. In the first clinical case for correction of behavioral disorders in patients with Parkinson,s disease levodopa/carbidopa dose was reduced to 750 mg per day (3/4 Tab. 4 times a day; added to levodopa/benserazide dispersible 100 mg morning and levodopa/benserazide 100 mg before sleep (total dose of levodopa of 950 mg per day. Amantadine sulfate and pramipexole were canceled. It was added to the therapy of atypical neuroleptic clozapine dose 6,25 mg overnight. After 3 months marked improvement, regressed visual hallucina­ tions, improved family relationships, background mood became more stable. The patient continue to sing karaoke, but this hobby has be­ come less intrusive. In the second clinical event correction impulsive

  13. Clinical Prediction Models for Cardiovascular Disease: Tufts Predictive Analytics and Comparative Effectiveness Clinical Prediction Model Database.

    Wessler, Benjamin S; Lai Yh, Lana; Kramer, Whitney; Cangelosi, Michael; Raman, Gowri; Lutz, Jennifer S; Kent, David M

    2015-07-01

    Clinical prediction models (CPMs) estimate the probability of clinical outcomes and hold the potential to improve decision making and individualize care. For patients with cardiovascular disease, there are numerous CPMs available although the extent of this literature is not well described. We conducted a systematic review for articles containing CPMs for cardiovascular disease published between January 1990 and May 2012. Cardiovascular disease includes coronary heart disease, heart failure, arrhythmias, stroke, venous thromboembolism, and peripheral vascular disease. We created a novel database and characterized CPMs based on the stage of development, population under study, performance, covariates, and predicted outcomes. There are 796 models included in this database. The number of CPMs published each year is increasing steadily over time. Seven hundred seventeen (90%) are de novo CPMs, 21 (3%) are CPM recalibrations, and 58 (7%) are CPM adaptations. This database contains CPMs for 31 index conditions, including 215 CPMs for patients with coronary artery disease, 168 CPMs for population samples, and 79 models for patients with heart failure. There are 77 distinct index/outcome pairings. Of the de novo models in this database, 450 (63%) report a c-statistic and 259 (36%) report some information on calibration. There is an abundance of CPMs available for a wide assortment of cardiovascular disease conditions, with substantial redundancy in the literature. The comparative performance of these models, the consistency of effects and risk estimates across models and the actual and potential clinical impact of this body of literature is poorly understood. © 2015 American Heart Association, Inc.

  14. Prevalence of HCV, HBV, and HIV Seropositivity among Cadavers Referred to Autopsy Hall of Legal Medicine Bureau of Tehran, Iran

    Jaber Gharehdaghi

    2017-01-01

    Full Text Available A large number of dead bodies are referred to forensic autopsy halls for medicolegal examination. They can be a source of transmission of infectious diseases through direct contact or autopsy tools. The main aim of this study was to estimate the virus infection rates in the dead bodies. One thousand consecutive dead bodies that had been referred to autopsy hall of Legal Medicine Bureau of Tehran, Iran, during 2016, were included. The blood samples were analyzed in the laboratory for detection of HBs Ag, HBs Ab, HIV Ab, and HCV Ab, after providing informed consent from legal next of kin of the dead bodies. The general characteristics of the dead bodies were also collected by a checklist. Forty-seven cases of HIV seropositivity, 80 cases of HBs Ag seropositivity, and 97 cases for HCV Ab seropositivity were found. Among them, 27 cases of HIV, 40 cases of anti-HBC positive, and 94 cases of RIBA testing positive for HCV were proved through confirmatory tests. In other words, 2.6% of the dead bodies were infected with HIV, 3.8% with HBV, and 9% with HCV. The total infection rate was 15.5%. This is a worrying risk for pathologist and autopsy technicians.

  15. Prevalence of HCV, HBV, and HIV Seropositivity among Cadavers Referred to Autopsy Hall of Legal Medicine Bureau of Tehran, Iran.

    Gharehdaghi, Jaber; Abedi Khorasgani, Mohammad Hassan; Ghadiani, Mohammad Hassan; Kazemifar, Amir Mohammad; Solhi, Hassan; Solhi, Sadra

    2017-01-01

    A large number of dead bodies are referred to forensic autopsy halls for medicolegal examination. They can be a source of transmission of infectious diseases through direct contact or autopsy tools. The main aim of this study was to estimate the virus infection rates in the dead bodies. One thousand consecutive dead bodies that had been referred to autopsy hall of Legal Medicine Bureau of Tehran, Iran, during 2016, were included. The blood samples were analyzed in the laboratory for detection of HBs Ag, HBs Ab, HIV Ab, and HCV Ab, after providing informed consent from legal next of kin of the dead bodies. The general characteristics of the dead bodies were also collected by a checklist. Forty-seven cases of HIV seropositivity, 80 cases of HBs Ag seropositivity, and 97 cases for HCV Ab seropositivity were found. Among them, 27 cases of HIV, 40 cases of anti-HBC positive, and 94 cases of RIBA testing positive for HCV were proved through confirmatory tests. In other words, 2.6% of the dead bodies were infected with HIV, 3.8% with HBV, and 9% with HCV. The total infection rate was 15.5%. This is a worrying risk for pathologist and autopsy technicians.

  16. Characterization of clinical-imaging characteristics of the binswanger's disease

    Rodriguez Mutuberria, Livan; Serra Valdes, Yusimi

    2002-01-01

    A review was made to go deep into the understanding of vascular dementias that behave as the second cause of dementia in practice. Binswanger's disease is one of the most important among them. Its detection has progressively increased with the continual improvement of the radiological diagnostic tools that allow to identify the ischemic damage of the hemispherical cerebral white matter and the presence of lacunar infarctions. It is a disease of chronic course and inexorably progressive that is characterized by the association of subcortical cognitive dysfunction, evidence of cerebrovascular disease, Parkinsonian rigidity and vesicle dysfunction with a characteristic imaging picture. The clinical picture and the main imaging characteristics are explained in this paper and the pathogens of the disease is briefly described

  17. Reproduction in porcine circovirus type 2 (PCV2 seropositive gilts inseminated with PCV2b spiked semen

    Sarli Giuseppe

    2012-08-01

    Full Text Available Abstract Background Since 1999, field evidence of transplacental infection by porcine circovirus type 2 (PCV2 and reproductive failure has been reported in pigs. The objective of this study was to evaluate the clinical and pathological consequences of PCV2 infection in conventional PCV2-seropositive gilts by insemination with PCV2b-spiked semen. Results Six PCV2 seropositive gilts were inseminated with PCV2b-supplemented semen (infected and three animals with semen and cell culture medium (controls. Only three out of the six infected animals were pregnant by ultrasonography on day 29 after insemination, while two out of the three controls were pregnant. One control gilt aborted on day 23 after insemination but not due to PVC2. Viraemia was demonstrated in four out of six infected and in one control gilt that became infected with PCV2a. Anti-PCV2 antibody titres showed dynamic variations in the infected group throughout the study. Among infected gilts, the animal with the lowest anti-PCV2 titre (1/100 at the beginning of the experiment and another that reached a similar low value during the experiment showed evident seroconversion over time and had also PCV2 positive foetuses. One placenta displayed mild focal necrosis of the chorionic epithelium positively stained by immunohistochemistry for PCV2 antigen. Conclusions PCV2-seropositive gilts can be infected with PCV2 after intrauterine exposure and low maternal antibody titre may increase the probability of a foetal infection.

  18. Pilonidal sinus disease - Etiological factors, pathogenesis and clinical features

    Kazim Duman

    2016-12-01

    Full Text Available and lsquo;Pilonidal sinus' disease, which is most commonly seen in reproductive populations, such as young adults - mostly in males who are in their twenties - is actually a controversial disease in that there is no consensus on its many facets. It is sometimes seen as an infected abscess draining from an opening or a lesion extending to the perineum. It may also present as a draining fistula opening to skin. In terms of etiological factors, various theories (main theories being congenital and acquired have been established since it was first described, no universal understanding achieved. A long and significant post-operative care period with different lengths of recovery depending on the type of operation are quite prevalent with regards to recurrence and complication status. In order to prevent recurrence and improve the quality of life, etiological and predisposing factors as well as clinical features of sacrococcygeal pilonidal disease should be well known, a detailed differential diagnosis should be made, and a suitable and timely intervention should be performed. It was aimed here to explain the etiological factors, pathogenesis and clinical features of the disease that may present with various clinical symptoms. [Arch Clin Exp Surg 2016; 5(4.000: 228-232

  19. Wilson's disease: cranial MRI observations and clinical correlation

    Sinha, S.; Taly, A.B.; Prashanth, L.K.; Venugopal, K.S.; Arunodaya, G.R.; Swamy, H.S.; Ravishankar, S.; Vasudev, M.K.

    2006-01-01

    Study of MRI changes may be useful in diagnosis, prognosis and better understanding of the pathophysiology of Wilson's disease (WD). We aimed to describe and correlate the MRI abnormalities of the brain with clinical features in WD. MRI evaluation was carried out in 100 patients (57 males, 43 females; mean age 19.3±8.9 years) using standard protocols. All but 18 patients were on de-coppering agents. Their history, clinical manifestations and scores for severity of disease were noted. The mean duration of illness and treatment were 8.3±10.8 years and 7.5±7.1 years respectively. MRI of the brain was abnormal in all the 93 symptomatic patients. The most conspicuous observations were atrophy of the cerebrum (70%), brainstem (66%) and cerebellum (52%). Signal abnormalities were also noted: putamen (72%), caudate (61%), thalami (58%), midbrain (49%), pons (20%), cerebral white matter (25%), cortex (9%), medulla (12%) and cerebellum (10%). The characteristic T2-W globus pallidal hypointensity (34%), ''Face of giant panda'' sign (12%), T1-W striatal hyperintensity (6%), central pontine myelinosis (7%), and bright claustral sign (4%) were also detected. MRI changes correlated with disease severity scores (P<0.001) but did not correlate with the duration of illness. MRI changes were universal but diverse and involved almost all the structures of the brain in symptomatic patients. A fair correlation between MRI observations and various clinical features provides an explanation for the protean manifestations of the disease. (orig.)

  20. Parthenium dermatitis severity score to assess clinical severity of disease

    Kaushal K Verma

    2017-01-01

    Full Text Available Background: Parthenium dermatitis is the most common type of airborne contact dermatitis in India. It is a chronic disease of a remitting and relapsing course with significant morbidity and distress, but there is no scoring system to assess its severity. Aim: To design a scoring system for the assessment of clinical severity of disease in Parthenium dermatitis and to use this scoring system in various studies to determine its sensitivity, specificity, and reproducibility. Methods and Results: In our first few studies on Parthenium dermatitis, we designed and used a basic clinical severity scoring system based on itching, morphology of the lesions, and areas involved. However, in subsequent studies, we modified it to the present scoring system as Parthenium dermatitis severity score (PDSS. Our studies showed the high sensitivity of PDSS in characterization of the disease severity at the given point of time, as well as to determine the efficacy of a prescribed treatment modality which was reliable and reproducible. Conclusion: Thus, PDSS may be used by clinicians for appropriate scoring of the clinical severity of Parthenium dermatitis and in monitoring the disease response to therapy.

  1. Design and Evaluation of a Bacterial Clinical Infectious Diseases Ontology

    Gordon, Claire L.; Pouch, Stephanie; Cowell, Lindsay G.; Boland, Mary Regina; Platt, Heather L.; Goldfain, Albert; Weng, Chunhua

    2013-01-01

    With antimicrobial resistance increasing worldwide, there is a great need to use automated antimicrobial decision support systems (ADSSs) to lower antimicrobial resistance rates by promoting appropriate antimicrobial use. However, they are infrequently used mostly because of their poor interoperability with different health information technologies. Ontologies can augment portable ADSSs by providing an explicit knowledge representation for biomedical entities and their relationships, helping to standardize and integrate heterogeneous data resources. We developed a bacterial clinical infectious diseases ontology (BCIDO) using Protégé-OWL. BCIDO defines a controlled terminology for clinical infectious diseases along with domain knowledge commonly used in hospital settings for clinical infectious disease treatment decision-making. BCIDO has 599 classes and 2355 object properties. Terms were imported from or mapped to Systematized Nomenclature of Medicine, Unified Medical Language System, RxNorm and National Center for Bitechnology Information Organismal Classification where possible. Domain expert evaluation using the “laddering” technique, ontology visualization, and clinical notes and scenarios, confirmed the correctness and potential usefulness of BCIDO. PMID:24551353

  2. Updated clinical diagnostic criteria for sporadic Creutzfeldt-Jakob disease

    Kallenberg, K.; Summers, D. M.; Romero, C.; Taratuto, A.; Heinemann, U.; Breithaupt, M.; Varges, D.; Meissner, B.; Ladogana, A.; Schuur, M.; Haik, S.; Collins, S. J.; Jansen, Gerard H.; Stokin, G. B.; Pimentel, J.; Hewer, E.; Collie, D.; Smith, P.; Roberts, H.; Brandel, J. P.; van Duijn, C.; Pocchiari, M.; Begue, C.; Cras, P.; Will, R. G.; Sanchez-Juan, P.

    2009-01-01

    Several molecular subtypes of sporadic Creutzfeldt–Jakob disease have been identified and electroencephalogram and cerebrospinal fluid biomarkers have been reported to support clinical diagnosis but with variable utility according to subtype. In recent years, a series of publications have demonstrated a potentially important role for magnetic resonance imaging in the pre-mortem diagnosis of sporadic Creutzfeldt–Jakob disease. Magnetic resonance imaging signal alterations correlate with distinct sporadic Creutzfeldt–Jakob disease molecular subtypes and thus might contribute to the earlier identification of the whole spectrum of sporadic Creutzfeldt–Jakob disease cases. This multi-centre international study aimed to provide a rationale for the amendment of the clinical diagnostic criteria for sporadic Creutzfeldt–Jakob disease. Patients with sporadic Creutzfeldt–Jakob disease and fluid attenuated inversion recovery or diffusion-weight imaging were recruited from 12 countries. Patients referred as ‘suspected sporadic Creutzfeldt–Jakob disease’ but with an alternative diagnosis after thorough follow up, were analysed as controls. All magnetic resonance imaging scans were assessed for signal changes according to a standard protocol encompassing seven cortical regions, basal ganglia, thalamus and cerebellum. Magnetic resonance imaging scans were evaluated in 436 sporadic Creutzfeldt–Jakob disease patients and 141 controls. The pattern of high signal intensity with the best sensitivity and specificity in the differential diagnosis of sporadic Creutzfeldt–Jakob disease was identified. The optimum diagnostic accuracy in the differential diagnosis of rapid progressive dementia was obtained when either at least two cortical regions (temporal, parietal or occipital) or both caudate nucleus and putamen displayed a high signal in fluid attenuated inversion recovery or diffusion-weight imaging magnetic resonance imaging. Based on our analyses, magnetic

  3. α-Synuclein oligomers and clinical implications for Parkinson disease

    Kalia, Lorraine V.; Kalia, Suneil K.; McLean, Pamela J.; Lozano, Andres M.; Lang, Anthony E.

    2012-01-01

    Protein aggregation within the central nervous system has been recognized as a defining feature of neurodegenerative diseases since the early 20th century. Since that time, there has been a growing list of neurodegenerative disorders, including Parkinson disease, which are characterized by inclusions of specific pathogenic proteins. This has led to the long-held dogma that these characteristic protein inclusions, which are composed of large insoluble fibrillar protein aggregates and visible by light microscopy, are responsible for cell death in these diseases. However, the correlation between protein inclusion formation and cytotoxicity is inconsistent suggesting another form of the pathogenic proteins may be contributing to neurodegeneration. There is emerging evidence implicating soluble oligomers, smaller protein aggregates not detectable by conventional microscopy, as potential culprits in the pathogenesis of neurodegenerative diseases. The protein α-synuclein is well recognized to contribute to the pathogenesis of Parkinson disease and is the major component of Lewy bodies and Lewy neurites. However, α-synuclein also forms oligomeric species with certain conformations being toxic to cells. The mechanisms by which these α-synuclein oligomers cause cell death are being actively investigated as they may provide new strategies for diagnosis and treatment of Parkinson disease and related disorders. Here we review the possible role of α-synuclein oligomers in cell death in Parkinson disease and discuss the potential clinical implications. PMID:23225525

  4. Measures of rheumatoid arthritis disease activity in Australian clinical practice.

    Taylor, Andrew; Bagga, Hanish

    2011-01-01

    Objectives. To investigate which rheumatoid arthritis (RA) disease activity measures are being collected in patients receiving glucocorticoids, non-biologic or biologic disease-modifying antirheumatic drugs (DMARDs) in Australian rheumatology practice. Methods. A retrospective audit of medical records was conducted from eight rheumatology practices around Australia. Each rheumatologist recruited 30 consecutive eligible patients into the review, 10 of whom must have been receiving a biological agent for rheumatoid arthritis. Disease activity measures and radiographic assessments were collected from each patient's last consultation. For biologic patients, disease activity measures were also collected from when the patient was first initiated on the biological agent. Results. At last consultation, the disease measures that were recorded most often were ESR (89.2%), haemoglobin (87.5%), and CRP (84.2%). DAS28 was infrequently recorded (16.3%). The rate of recording disease activity measures for patients receiving biologic DMARDs decreased over time (mean 27 months). Conclusion. This review has shown inconsistency of RA activity measures being recorded in Australian rheumatology clinical practice. An accurate assessment of the disease process is necessary to effectively target rheumatoid arthritis patients to treat in order to achieve optimal outcomes.

  5. Clinical-histopathological correlation in a case of Coats' disease

    Zajdenweber Moyses E

    2006-08-01

    Full Text Available Abstract Background Coats' disease is a non-hereditary ocular disease, with no systemic manifestation, first described by Coats in 1908. It occurs more commonly in children and has a clear male predominance. Most patients present clinically with unilateral decreased vision, strabismus or leukocoria. The most important differential diagnosis is unilateral retinoblastoma, which occurs in the same age group and has some overlapping clinical manifestations. Case presentation A 4 year-old girl presented with a blind and painful right eye. Ocular examination revealed neovascular glaucoma, cataract and posterior synechiae. Although viewing of the fundus was impossible, computed tomography disclosed total exsudative retinal detachment in the affected eye. The eye was enucleated and subsequent histopathological evaluation confirmed the diagnosis of Coats' disease. Conclusion General pathologists usually do not have the opportunity to receive and study specimens from patients with Coats' disease. Coats' disease is one of the most important differential diagnoses of retinoblastoma. Therefore, It is crucial for the pathologist to be familiar with the histopathological features of the former, and distinguish it from the latter.

  6. The influence of smoking on clinical periodontal disease

    Ina Hendiani

    2009-07-01

    Full Text Available Periodontal disease has very complex and multi factor etiology. Plaque bacteria is the main cause of periodontal disease and another risk factor that also plays a role is a smoking habit. Cigarette product such as nicotine can influence the development of periodontal disease that can directly and systemically damage the function of PMN cell. The research was conducted by taking a clinical examination on the smoking influence that covers the number of cigarettes and the period of smoking, and kind of cigarette to the worse of periodontal disease, and by measuring the epithelial attachment loss and the bleeding index. The research was conducted to 152 male aged 20-45 years old, comprised 80 smokers and 72 nonsmokers at the Clinic of Faculty of Dentistry Universitas Indonesia, Jakarta. The result of the research showed that smoking gave influence on the worse of the periodontal disease. There was a profound relationship between the smoking period and the number of cigarettes consumed everyday indicated by the epithelial attachment loss. Smoking did not enhance gingival bleeding. The relationship between kinds of cigarette and the gingival bleeding score and the epithelial attachment loss did not show a significant bleeding.

  7. Kikuchi-Fujimoto disease: Clinical and laboratory characteristics and outcome

    P S Rakesh

    2014-01-01

    Full Text Available Introduction: Kikuchi-Fujimoto disease is an uncommon disorder with worldwide distribution, characterized by fever and benign enlargement of the lymph nodes, primarily affecting young adults. Awareness about this disorder may help prevent misdiagnosis and inappropriate investigations and treatment. The objective of the study was to evaluate the clinical and laboratory characteristics of histopathologically confirmed cases of Kikuchi′s disease from a tertiary care center in southern India. Materials and Methods: Retrospective analysis of all adult patients with histopathologically confirmed Kikuchi′s disease from January 2007 to December 2011 in a 2700-bed teaching hospital in South India was done. The clinical and laboratory characteristics and outcome were analyzed. Results: There were 22 histopathologically confirmed cases of Kikuchi′s disease over the 5-year period of this study. The mean age of the subjects′ was 29.7 years (SD 8.11 and majority were women (Male: female- 1:3.4. Apart from enlarged cervical lymph nodes, prolonged fever was the most common presenting complaint (77.3%. The major laboratory features included anemia (54.5%, increased erythrocyte sedimentation rate (31.8%, elevated alanine aminotransferase (27.2% and elevated lactate dehydrogenase (LDH (31.8%. Conclusion: Even though rare, Kikuchi′s disease should be considered in the differential diagnosis of young individuals, especially women, presenting with lymphadenopathy and prolonged fever. Establishing the diagnosis histopathologically is essential to avoid inappropriate investigations and therapy.

  8. Uncontrolled chronic disease: patient non-compliance or clinical mismanagement?

    Javors, Jonathan R; Bramble, Judith E

    2003-01-01

    A study group of 30 individuals was randomly chosen from 1,379 beneficiaries predicted to be at risk for health care complications at a large, Midwest, industrial company currently experiencing increased health care costs. All 30 individuals had one or more chronic illness, primarily diabetes, cardiovascular disease, or asthma. Through analysis of medical records, a self-reporting health risk assessment survey, and personal contact with both patients and clinicians, each study individual was assessed as to whether his disease(s) was under control, the individual was compliant with his treatment protocol, and whether the supervising clinician was following nationally accepted standards of care. Fewer than 50% of the individuals in the study group had their chronic illness(es) under control. Those individuals whose treatment adhered to national guidelines were significantly more likely to have their disease under control (p Behavioral (external) barriers were most often cited as the reason a clinical practitioner did not follow the appropriate national standard of care. Most clinicians were aware of and familiar with the guidelines; a few either did not agree with or misunderstood the guidelines. The results of this study suggest that changing clinical practice behaviors to better ensure compliance to national standards of care may make a substantial difference in chronic disease control.

  9. [Clinical and morphological variants of diverticular disease in colon].

    Levchenko, S V; Lazebnik, L B; Potapova, V B; Rogozina, V A

    2013-01-01

    Our own results of two-stage research are presented in the article. The first stage contains the retrospective analysis of 3682 X-ray examining of large bowel which were conducted in 2002-2004 to define the structure of colon disease and to determine gender differences. The second stage is prospective research which took place from 2003 to 2012 and 486 patients with diverticular disease were regularly observed. Following parameters were estimated: dynamics of complaints, life quality, clinical symptoms. Multiple X-ray and endoscopic examining were done with estimation of quantity and size of diverticula, changes of colon mucosa, comparison of X-ray and endoscopic methods in prognosis of complications. Two basic clinical morphological variants of diverticular disease (DD) of colon are made out as a result of our research. There are IBD-like and DD with ischemic component. The variants differ by pain characteristics, presence of accompanying diseases, life quality parameters and description of colon mucosa morphological research. We suppose that different ethiopathogenetic factors of development of both variants mentioned above influence the disease prognosis and selection of treatment.

  10. Semantic Memory in the Clinical Progression of Alzheimer Disease.

    Tchakoute, Christophe T; Sainani, Kristin L; Henderson, Victor W

    2017-09-01

    Semantic memory measures may be useful in tracking and predicting progression of Alzheimer disease. We investigated relationships among semantic memory tasks and their 1-year predictive value in women with Alzheimer disease. We conducted secondary analyses of a randomized clinical trial of raloxifene in 42 women with late-onset mild-to-moderate Alzheimer disease. We assessed semantic memory with tests of oral confrontation naming, category fluency, semantic recognition and semantic naming, and semantic density in written narrative discourse. We measured global cognition (Alzheimer Disease Assessment Scale, cognitive subscale), dementia severity (Clinical Dementia Rating sum of boxes), and daily function (Activities of Daily Living Inventory) at baseline and 1 year. At baseline and 1 year, most semantic memory scores correlated highly or moderately with each other and with global cognition, dementia severity, and daily function. Semantic memory task performance at 1 year had worsened one-third to one-half standard deviation. Factor analysis of baseline test scores distinguished processes in semantic and lexical retrieval (semantic recognition, semantic naming, confrontation naming) from processes in lexical search (semantic density, category fluency). The semantic-lexical retrieval factor predicted global cognition at 1 year. Considered separately, baseline confrontation naming and category fluency predicted dementia severity, while semantic recognition and a composite of semantic recognition and semantic naming predicted global cognition. No individual semantic memory test predicted daily function. Semantic-lexical retrieval and lexical search may represent distinct aspects of semantic memory. Semantic memory processes are sensitive to cognitive decline and dementia severity in Alzheimer disease.

  11. Imaging of cystic fibrosis lung disease and clinical interpretation

    Wielpuetz, M.O.; Eichinger, M.; Kauczor, H.U. [Heidelberg University Hospital (Germany). Dept. of Diagnostic and Interventional Radiology; Translational Lung Research Center Heidelberg (TLRC) (Germany); Heidelberg University Hospital (Germany). Dept. of Diagnostic and Interventional Radiology with Nuclear Medicine; Biederer, J. [Heidelberg University Hospital (Germany). Dept. of Diagnostic and Interventional Radiology; Translational Lung Research Center Heidelberg (TLRC) (Germany); Gross-Gerau Community Hospital (Germany). Radiologie Darmstadt; Wege, S. [Heidelberg University Hospital (Germany). Dept. of Pulmonology and Respiratory Medicine; Stahl, M.; Sommerburg, O. [Translational Lung Research Center Heidelberg (TLRC) (Germany); Heidelberg University Hospital (Germany). Div. of Pediatric Pulmonology and Allergy and Cystic Fibrosis Center; Mall, M.A. [Translational Lung Research Center Heidelberg (TLRC) (Germany); Heidelberg University Hospital (Germany). Div. of Pediatric Pulmonology and Allergy and Cystic Fibrosis Center; Heidelberg University Hospital (Germany). Dept. of Translational Pulmonology; Puderbach, M. [Heidelberg University Hospital (Germany). Dept. of Diagnostic and Interventional Radiology; Translational Lung Research Center Heidelberg (TLRC) (Germany); Heidelberg University Hospital (Germany). Dept. of Diagnostic and Interventional Radiology with Nuclear Medicine; Hufeland Hospital, Bad Langensalza (Germany). Dept. of Diagnostic and Interventional Radiology

    2016-09-15

    Progressive lung disease in cystic fibrosis (CF) is the life-limiting factor of this autosomal recessive genetic disorder. Increasing implementation of CF newborn screening allows for a diagnosis even in pre-symptomatic stages. Improvements in therapy have led to a significant improvement in survival, the majority now being of adult age. Imaging provides detailed information on the regional distribution of CF lung disease, hence longitudinal imaging is recommended for disease monitoring in the clinical routine. Chest X-ray (CXR), computed tomography (CT) and magnetic resonance imaging (MRI) are now available as routine modalities, each with individual strengths and drawbacks, which need to be considered when choosing the optimal modality adapted to the clinical situation of the patient. CT stands out with the highest morphological detail and has often been a substitute for CXR for regular severity monitoring at specialized centers. Multidetector CT data can be post-processed with dedicated software for a detailed measurement of airway dimensions and bronchiectasis and potentially a more objective and precise grading of disease severity. However, changing to CT was inseparably accompanied by an increase in radiation exposure of CF patients, a young population with high sensitivity to ionizing radiation and lifetime accumulation of dose. MRI as a cross-sectional imaging modality free of ionizing radiation can depict morphological hallmarks of CF lung disease at lower spatial resolution but excels with comprehensive functional lung imaging, with time-resolved perfusion imaging currently being most valuable.

  12. Clinical management of pain and fatigue in Parkinson's disease.

    Del Sorbo, Francesca; Albanese, Alberto

    2012-01-01

    Pain and fatigue are part of the phenomenological spectrum of Parkinson's disease (PD). These non-motor symptoms can be as troublesome as motor symptoms, impact activities of daily living, and are often underdiagnosed. The recognition of pain and fatigue requires a high degree of clinical suspicion and is facilitated by the use of specific questionnaires and ancillary tests. This workup is highly valuable particularly considering that pain and fatigue in PD may be treatable. We review here the clinical manifestations and management of these non-motor symptoms. Their resolution can be challenging, as there is insufficient evidence concerning effective treatment options. Copyright © 2011 Elsevier Ltd. All rights reserved.

  13. ACG clinical guidelines: diagnosis and management of celiac disease.

    Rubio-Tapia, Alberto; Hill, Ivor D; Kelly, Ciarán P; Calderwood, Audrey H; Murray, Joseph A

    2013-05-01

    clinical trials, but are not yet approved for use in practice. Given the incomplete response of many patients to a GFD-free diet as well as the difficulty of adherence to the GFD over the long term, development of new effective therapies for symptom control and reversal of inflammation and organ damage are needed. The prevalence of celiac disease is increasing worldwide and many patients with celiac disease remain undiagnosed, highlighting the need for improved strategies in the future for the optimal detection of patients.

  14. Sub-isotypic differences in the immunoglobulin G response to Lawsonia intracellularis in vaccinated, seropositive, and equine proliferative enteropathy-affected horses.

    Page, Allen E; Stills, Harold F; Horohov, David W

    2014-12-15

    In the horse, Lawsonia intracellularis infection results in equine proliferative enteropathy (EPE). While upwards of 100% of weanlings on an endemic farm may seroconvert, only a small percentage (approximately 5%) will develop clinical disease. Cell-mediated immune mechanisms likely play a role in resistance to L. intracellularis and the absence of a L. intracellularis-specific IFN-γ response has been associated with the development of EPE. The goal of this study was to determine whether protection from clinical EPE is associated with the induction of a systemic IgG sub-isotypic response consistent with a Th1-type cytokine response. To describe their L. intracellularis/EPE status, horses enrolled in this study were placed into one of three categories: seropositive-only, vaccinated, and presumptive clinical EPE. An existing ELISA method was modified to detect L. intracellularis-specific IgG(a), IgG(b), and IgG(t) antibodies using the mouse anti-equine hybridomas CVS-48, CVS-39, and CVS-40, respectively. Additionally, the existing ELISA method was used to quantify total IgG antibodies specific for L. intracellularis for comparison between the groups. Total L. intracellularis-specific IgG was found to be significantly higher (pequine IgG sub-isotypes may provide additional information once these become commercially available. Copyright © 2014 Elsevier B.V. All rights reserved.

  15. Clinical observation of cerebrovascular diseases current in Chernobyl accident liquidators

    Golovchenko, Yu.Yi.; Usatenko, O.G.; Romanenko, N.Yi.

    1999-01-01

    The results of the clinical follow up study (1993-1997) of cerebrovascular diseases development in the Chernobyl accident liquidators are presented. The syndrome of autonomous nervous system dysfunction following to an exposure to the Chernobyl accident consequences factors promotes to fast development of atherosclerosis and arterial hypertension. On the base of an analysis of the data obtained it was established that the primary diencephalic structures damage resulted in severe changes of different metabolic system, particularly in the cerebrovascular disorders development

  16. Clinical Holistic Medicine: Chronic Infections and Autoimmune Diseases

    Ventegodt, Søren; Merrick, Joav

    2005-01-01

    The consciousness-based (holistic) medical toolbox might be useful in general practice and in cases of recurrent infections and chronic infection or inflammation. From our clinical experiences, there is hope for improvement from a number of diseases caused by disorders affecting the regulation of the immune system when the physician includes the holistic medical approach.Our scientific understanding of the connection between consciousness and cellular order is still limited. Consciousness-bas...

  17. Management of celiac disease: from evidence to clinical practice

    Tiziana M. Attardo

    2017-11-01

    Full Text Available Celiac disease (CD is a complex polygenic disorder, which involves genetic factors human leukocyte complex (HLA and non-HLA genes, environmental factors, innate and adoptive immunity, and a robust chronic T-mediated autoimmune component. The main goal of the present monograph is to define a methodological approach for the disease, characterized by frequent late diagnosis, in order for the physician to become aware of the disease management, the diversity of the clinical presentation itself and in different patients. A unique attention is payed to the specific diagnostic tests to define a correct and accurate application of them, and in addition, to disease follow-up and possible complications. Moreover, a dedicated space is assigned to refractory CD, to potential CD and non-celiac gluten sensitivity. Legislative aspects of the celiac disease in Italy are addressed, too. The celiac disease guidelines and their evaluation by means of Appraisal of Guidelines, Research and Evaluation II instrument allow us to classify the different recommendations and to apply them according to the stakeholders’ involvement, pertinence, methodological accuracy, clarity and publishing independence. Finally, the most current scientific evidence is taken into account to create a complete updated monograph.

  18. Norrie disease: extraocular clinical manifestations in 56 patients.

    Smith, Sharon E; Mullen, Thomas E; Graham, Dionne; Sims, Katherine B; Rehm, Heidi L

    2012-08-01

    Norrie disease (ND) is an X-linked recessive disorder characterized by congenital blindness, progressive sensorineural hearing loss and cognitive impairment. The ocular phenotype has been well described, while the extraocular manifestations of the disorder are not well understood. We present the data from the Norrie Disease Registry, which consists of 56 patients with detailed clinical histories and genotype data. This study represents the largest, detailed investigation into the phenotypic spectrum of ND to date and more importantly expands knowledge of the extraocular clinical manifestations. We identify several novel aspects of the syndrome that will improve the management of these patients. In particular, we expand our understanding of the neurologic manifestations in ND and identify a chronic seizure disorder in approximately 10% of all patients. In addition, details of the hearing phenotype are described including the median age of onset (12 years of age) and how genotype affects onset. Moreover, we find vascular disease to be a significant component of ND; and vascular health should be, in the future, a component of patient clinical care. In summary, the results expand our understanding of the phenotypic variability and genotypic heterogeneity in ND patients. Copyright © 2012 Wiley Periodicals, Inc.

  19. Analysis of neurodegenerative Mendelian genes in clinically diagnosed Alzheimer Disease.

    Maria Victoria Fernández

    2017-11-01

    Full Text Available Alzheimer disease (AD, Frontotemporal lobar degeneration (FTD, Amyotrophic lateral sclerosis (ALS and Parkinson disease (PD have a certain degree of clinical, pathological and molecular overlap. Previous studies indicate that causative mutations in AD and FTD/ALS genes can be found in clinical familial AD. We examined the presence of causative and low frequency coding variants in the AD, FTD, ALS and PD Mendelian genes, in over 450 families with clinical history of AD and over 11,710 sporadic cases and cognitive normal participants from North America. Known pathogenic mutations were found in 1.05% of the sporadic cases, in 0.69% of the cognitively normal participants and in 4.22% of the families. A trend towards enrichment, albeit non-significant, was observed for most AD, FTD and PD genes. Only PSEN1 and PINK1 showed consistent association with AD cases when we used ExAC as the control population. These results suggest that current study designs may contain heterogeneity and contamination of the control population, and that current statistical methods for the discovery of novel genes with real pathogenic variants in complex late onset diseases may be inadequate or underpowered to identify genes carrying pathogenic mutations.

  20. The prevalence and clinical characteristics of punding in Parkinson's disease.

    Spencer, Ashley H; Rickards, Hugh; Fasano, Alfonso; Cavanna, Andrea E

    2011-03-01

    Punding (the display of stereotyped, repetitive behaviors) is a relatively recently discovered feature of Parkinson's disease (PD). Little is known about the prevalence and clinical characteristics of punding in PD. In this review, four large scientific databases were comprehensively searched for literature in relation to punding prevalence and clinical correlates in the context of PD. Prevalence was found to vary greatly (between 0.34 to 14%), although there were large disparities in study populations, assessment methods, and criteria. We observed an association between punding, dopaminergic medications, and impulse control disorder. Other characteristics, which may be more common among punders, include a higher severity of dyskinesia, younger age of disease onset, longer disease duration, and male gender. More research in large clinical datasets is required in many areas before conclusions are drawn. The pathophysiology behind the punding phenomenon is also poorly understood at present, rendering it difficult to develop targeted therapy. The current mainstay of treatment is the reduction in the dose of dopaminergic medications, the evidence for other suggested therapies being purely empirical.

  1. Rethinking dry eye disease: a perspective on clinical implications.

    Bron, Anthony J; Tomlinson, Alan; Foulks, Gary N; Pepose, Jay S; Baudouin, Christophe; Geerling, Gerd; Nichols, Kelly K; Lemp, Michael A

    2014-04-01

    Publication of the DEWS report in 2007 established the state of the science of dry eye disease (DED). Since that time, new evidence suggests that a rethinking of traditional concepts of dry eye disease is in order. Specifically, new evidence on the epidemiology of the disease, as well as strategies for diagnosis, have changed the understanding of DED, which is a heterogeneous disease associated with considerable variability in presentation. These advances, along with implications for clinical care, are summarized herein. The most widely used signs of DED are poorly correlated with each other and with symptoms. While symptoms are thought to be characteristic of DED, recent studies have shown that less than 60% of subjects with other objective evidence of DED are symptomatic. Thus the use of symptoms alone in diagnosis will likely result in missing a significant percentage of DED patients, particularly with early/mild disease. This could have considerable impact in patients undergoing cataract or refractive surgery as patients with DED have less than optimal visual results. The most widely used objective signs for diagnosing DED all show greater variability between eyes and in the same eye over time compared with normal subjects. This variability is thought to be a manifestation of tear film instability which results in rapid breakup of the tearfilm between blinks and is an identifier of patients with DED. This feature emphasizes the bilateral nature of the disease in most subjects not suffering from unilateral lid or other unilateral destabilizing surface disorders. Instability of the composition of the tears also occurs in dry eye disease and shows the same variance between eyes. Finally, elevated tear osmolarity has been reported to be a global marker (present in both subtypes of the disease- aqueous-deficient dry eye and evaporative dry eye). Clinically, osmolarity has been shown to be the best single metric for diagnosis of DED and is directly related to

  2. Clinical association: Lyme disease and Guillain-Barre syndrome.

    Patel, Kinner; Shah, Siddharth; Subedi, Dinesh

    2017-10-01

    Guillain-Barre Syndrome (GBS) is a life-threatening condition in which patients may present to the Emergency Department in respiratory distress leading to death. The early identification and treatment of such a condition is paramount in preventing mortality. While there are many infections associated with GBS, the association with Lyme disease is uncommon. Through our case we aim to highlight Borrelia burgdorferi as an important antecedent infection associated with the development of GBS. In this case we report a 31-year-old male who was diagnosed with Lyme disease and GBS with relevant clinical presentation including progressive numbness and weakness in bilateral hands and feet for the past 1week along with areflexia. Initiation of medical therapy with intravenous immunoglobulin and parenteral ceftriaxone resulted in resolution of his symptoms. The treatment of both diseases early can help prevent further central nervous complications leading to high morbidity and mortality. Copyright © 2017 Elsevier Inc. All rights reserved.

  3. Update on the clinical management of Wilson's disease

    Hedera P

    2017-01-01

    Full Text Available Peter Hedera Department of Neurology, Vanderbilt University Medical Center, Nashville, TN, USA Abstract: Wilson’s disease (WD, albeit relatively rare, is an important genetic metabolic disease because of highly effective therapies that can be lifesaving. It is a great imitator and requires a high index of suspicion for correct and timely diagnosis. Neurologic, psychiatric and hepatologic problems in WD are very nonspecific, and we discuss the most common clinical phenotypes. The diagnosis remains laboratory based, and here we review the most important challenges and pitfalls in laboratory evaluation of WD, including the emerging role of genetic testing in WD diagnosis. WD is a monogenic disorder but has very high allelic heterogeneity with >500 disease-causing mutations identified, and new insights into phenotype–genotype correlations are also reviewed. The gold standard of therapy is chelation of excessive copper, but many unmet needs exist because of possible clinical deterioration in treated patients and potential adverse effects associated with currently available chelating medications. We also review the most promising novel therapeutic approaches, including chelators targeting specific cell types, cell transplantation and gene therapy. Keywords: Wilson’s disease, copper, ATP7B, chelation, gene therapy

  4. Personality in Parkinson's disease: Clinical, behavioural and cognitive correlates.

    Santangelo, Gabriella; Piscopo, Fausta; Barone, Paolo; Vitale, Carmine

    2017-03-15

    Affective disorders and personality changes have long been considered pre-motor aspects of Parkinson's disease (PD). Many authors have used the term "premorbid personality" to define distinctive features of PD patients' personality characterized by reduced exploration of new environmental stimuli or potential reward sources ("novelty seeking") and avoidance behaviour ("harm avoidance") present before motor features. The functional correlates underlying the personality changes described in PD, implicate dysfunction of meso-cortico-limbic and striatal circuits. As disease progresses, the imbalance of neurotransmitter systems secondary to degenerative processes, along with dopamine replacement therapy, can produce a reversal of behaviours and an increase in reward seeking, laying the foundations for the emergence of the impulse control disorders. Personality disorders can be interpreted, therefore, as the result of individual susceptibility arising from intrinsic degenerative processes and individual personality features, in combination with extrinsic factors such as lifestyle, PD motor dysfunction and drug treatment. For a better understanding of personality disorders observed in PD and their relationship with the prodromal stage of the disease, prospective clinical studies are needed that correlate different personality profiles with other disease progression markers. Here, we review previous studies investigating the clinical, cognitive and behavioural correlates of personality traits in PD patients. Copyright © 2017 Elsevier B.V. All rights reserved.

  5. Chronic Liver Diseases in Children: Clinical Profile and Histology.

    Dhole, Sachin Devidas; Kher, Archana S; Ghildiyal, Radha G; Tambse, Manjusha P

    2015-07-01

    The main aim of the study is to study the clinical profile of disorders of the liver and hepatobiliary system in paediatric patients and to correlate the histopathology findings of liver biopsy in chronic liver disease. Another aim being to assess the prognosis and to know the outcome and the effects of treatment in chronic liver diseases in paediatric age group. It was a prospective study, included the clinical profile of Chronic Liver Diseases (CLD) in children and the histopathological correlation. A total of 55 children were thoroughly investigated by doing relevant investigations and liver biopsy. A male predominance (60%) was noted with maximum incidence in the age group of 6-12 years. The incidence of CLD was 1.1% of total admissions. The most common presenting complaint was jaundice and abdominal distension. Hepatic encephalopathy was noted in 29% patients. Hepatomegaly was seen in 63% patients and spleenomegaly was seen in 60% patients. The incidence of cirrhosis on liver biopsy was 42% (23cases) in CLD patients. The most common diagnosis on histopathology was Wilson's disease (22%), followed by hepatitis and autoimmune hepatitis. The predominant spectrum of CLD was metabolic liver disease and also the predominant cause of death. As the incidence of CLD is quite low, a very high index of suspicion is required for its diagnosis. Some uncommon causes of CLD in children were seen in our study like neutral lipid storage disease, α1-Antitrypsin deficiency disease, lupus hepatitis, Alagille syndrome and Budd-Chiari syndrome. A patient of CLD with jaundice and hepatomegaly should be treated aggressively as those are the poor prognostic indicators of the disease. Hepatic encephalopathy and cirrhosis are also associated with poor outcome in patients with CLD. Liver biopsy histopathology by an expert and its correlation with laboratory investigations plays an important role in the diagnosis of CLD. The major cause of deaths in patients with CLD is due to end stage

  6. Alzheimer biomarkers and clinical Alzheimer disease were not associated with increased cerebrovascular disease in a memory clinic population.

    Spies, Petra E; Verbeek, Marcel M; Sjogren, Magnus J C; de Leeuw, Frank-Erik; Claassen, Jurgen A H R

    2014-01-01

    Preclinical and post-mortem studies suggest that Alzheimer disease (AD) causes cerebrovascular dysfunction, and therefore may enhance susceptibility to cerebrovascular disease (CVD). The objective of this study was to investigate this association in a memory clinic population. The AD biomarkers CSF amyloid β42, amyloid β40 and APOE-ε4 status have all been linked to increased CVD risk in AD, and therefore the first aim of this study was to analyze the association between these biomarkers and CVD. In 92 memory clinic patients the cross-sectional association between AD biomarkersand the severity of CVD was investigated with linear regression analysis. Additionally, we studied whether AD biomarkers modified the relation between vascular risk factors and CVD. CVD was assessed on MRI through a visual rating scale.Analyses were adjusted for age. The second aim of this study was to investigate the association between clinical AD and CVD, where 'clinical AD' was defined as follows: impairment in episodic memory, hippocampal atrophy and an aberrant concentration of cerebrospinal fluid (CSF) biomarkers. 47 of the 92 patients had AD. No association between CSF amyloid β42, amyloid β40 or APOE-ε4 status and CVD severity was found, nor did these AD biomarkers modify the relation between vascular risk factors and CVD. Clinical AD was not associated with CVD severity (p=0.83). Patients with more vascular risk factors had more CVD, but this relationship was not convincingly modified by AD (p=0.06). In this memory clinic population, CVD in patients with AD was related to vascular risk factors and age, comparable to patients without AD. Therefore, in our study, the preclinical and post-mortem evidence that AD would predispose to CVD could not be translated clinically. Further work, including replication of this work in a different and larger sample, is warranted.

  7. Infertility etiologies are genetically and clinically linked with other diseases in single meta-diseases.

    Tarín, Juan J; García-Pérez, Miguel A; Hamatani, Toshio; Cano, Antonio

    2015-04-15

    The present review aims to ascertain whether different infertility etiologies share particular genes and/or molecular pathways with other pathologies and are associated with distinct and particular risks of later-life morbidity and mortality. In order to reach this aim, we use two different sources of information: (1) a public web server named DiseaseConnect ( http://disease-connect.org ) focused on the analysis of common genes and molecular mechanisms shared by diseases by integrating comprehensive omics and literature data; and (2) a literature search directed to find clinical comorbid relationships of infertility etiologies with only those diseases appearing after infertility is manifested. This literature search is performed because DiseaseConnect web server does not discriminate between pathologies emerging before, concomitantly or after infertility is manifested. Data show that different infertility etiologies not only share particular genes and/or molecular pathways with other pathologies but they have distinct clinical relationships with other diseases appearing after infertility is manifested. In particular, (1) testicular and high-grade prostate cancer in male infertility; (2) non-fatal stroke and endometrial cancer, and likely non-fatal coronary heart disease and ovarian cancer in polycystic ovary syndrome; (3) osteoporosis, psychosexual dysfunction, mood disorders and dementia in premature ovarian failure; (4) breast and ovarian cancer in carriers of BRCA1/2 mutations in diminished ovarian reserve; (5) clear cell and endometrioid histologic subtypes of invasive ovarian cancer, and likely low-grade serous invasive ovarian cancer, melanoma and non-Hodgkin lymphoma in endometriosis; and (6) endometrial and ovarian cancer in idiopathic infertility. The present data endorse the principle that the occurrence of a disease (in our case infertility) is non-random in the population and suggest that different infertility etiologies are genetically and clinically

  8. Clinical utility of ustekinumab in Crohn’s disease

    Kotze PG

    2018-02-01

    Full Text Available Paulo Gustavo Kotze,1,2 Christopher Ma,1 Abdulelah Almutairdi,1,3 Remo Panaccione1 1Inflammatory Bowel Disease Unit, Division of Gastroenterology and Hepatology, Cumming School of Medicine, University of Calgary, Calgary, AB, Canada; 2Inflammatory Bowel Disease Outpatient Clinics, Colorectal Surgery Unit, Catholic University of Paraná, Curitiba, Brazil; 3Department of Medicine, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia Abstract: The introduction of anti-tumor necrosis factor (TNF therapy marked an important milestone in the management of moderate-to-severe Crohn’s disease (CD. However, there remains a pressing demand for alternative therapeutic options for patients with primary nonresponse, secondary loss of response, or intolerable side effects to conventional treatment and TNF antagonists. Ustekinumab (UST is a fully human IgG1κ monoclonal antibody that inhibits the p40 subunit shared by the proinflammatory cytokines, the interleukin (IL-12 and -23. This blockade leads to dampening of the inflammatory cascade and differentiation of inflammatory T cells. The clinical development program for UST in CD includes dose finding Phase II (Crohn’s Evaluation of Response to Ustekinumab Anti-Interleukin-12/23 for Induction [CERTIFI] and the pivotal Phase III (UNITI trials that demonstrated both the clinical efficacy and safety in anti-TNF-naive and anti-TNF-exposed patients. Real-world evidence has further defined the role of UST in CD management. In this review, we discuss the mechanism of action of UST, describe the results of the randomized controlled trials with this agent, and review the real-world efficacy and safety data from observational cohorts. Finally, we identify areas of future research in the IL-12/23 inflammatory pathway and discuss the positioning of this novel therapeutic option in CD treatment algorithms. Keywords: ustekinumab, Crohn’s disease, interleukin

  9. Clinical profile of parkinson's disease: Experience of niger

    Hamid Assadeck

    2018-01-01

    Full Text Available Background: Parkinson's disease (PD is a chronic neurodegenerative pathology with unknown etiology. It is characterized clinically by the classic triad that associated tremors, bradykinesia, and rigidity. In Niger, there are no data on PD. Aims: We aimed to provide the demographic and clinical profile of PD in patients from Niger to create a database on PD in Niger. Patients and Methods: We conducted a retrospective study at the Neurology Outpatient Clinic of the Hôpital National de Niamey (HNN, Niger over a period of 4.42 years from February 2009 to July 2013 collecting all cases of PD. The demographic and clinical features of all patients were collected and analyzed. Results: During the period of the study, 1695 patients consulted at the Neurology Outpatient Clinic of the HNN, among which 76 patients (4.48% had secondary parkinsonism and 25 patients (1.47% had features compatible with PD. Only patients with PD were included in this study. The mean age at onset of symptoms was 58 years (range: 42–74 years. The male sex was predominant (60% with a sex ratio of 1.5. The mean time interval from the onset of symptoms to diagnosis of PD was 1.8 years (range: 1–5 years. The tremor was the most common symptom (84%. Bradykinesia represented 64% of the symptoms and rigidity 20%. At the time of the diagnosis of PD, 8 patients (32% were in Stage I of the classification of Hoehn and Yahr, 16 patients (64% in Stage II, and 1 patient (4% in Stage III. The levodopa/carbidopa combination was the most used antiparkinsonian drug in our patients (88%. The mean time of follow-up of the patients was 2.5 years (range: 1–4.42 years. During the course of the disease, 9 patients (36% were in Stage II of the classification of Hoehn and Yahr, 13 patients (52% in Stage III, and 3 patients (12% in Stage IV. Conclusion: Our study provides demographic and clinical data of PD in patients from Niger and shows that the hospital frequency of this disease is low (1

  10. Occupational Lung Disease: Clinical-Pathological-Radiological Correlation

    Carrillo Bayona, Jorge Alberto; Rivera Bernal, Aura Lucia; Ojeda Paulina; Paez Garcia, Diana Sofia

    2008-01-01

    People are exposed to hundreds of substances daily, some of which may induce pulmonary injury. Occupational Lung Disease diagnosis requires 4 elements: Exposure to the harmful agent, adequate latency between exposure and beginning of the symptoms, syndrome with post-exposure abnormalities, and exclusion of other conditions which may otherwise explain signs and symptoms. Several occupational lung disease classifications based on structural or functional injury, type of agent, or both have been proposed. Generally, 5 groups are considered: Pneumoconiosis, hypersensitivity pneumonitis, toxic fumes exposure, asthma, and occupational lung infections. Conventional radiographs and in specific situations, CT, are crucial elements for the diagnosis of Occupational Lung Disease. In the patient with respiratory symptoms and altered imaging studies, the possibility of Occupational Lung Disease should be considered. Radiologist should be familiar the variety of substances that cause these entities and their radiological features. In this article Occupational Lung diseases are reviewed, including diagnostic criteria, classification, physiopathology, clinical and radiological manifestations as well as their corresponding histopathological features.

  11. Rare diseases in clinical endocrinology: a taxonomic classification system.

    Marcucci, G; Cianferotti, L; Beck-Peccoz, P; Capezzone, M; Cetani, F; Colao, A; Davì, M V; degli Uberti, E; Del Prato, S; Elisei, R; Faggiano, A; Ferone, D; Foresta, C; Fugazzola, L; Ghigo, E; Giacchetti, G; Giorgino, F; Lenzi, A; Malandrino, P; Mannelli, M; Marcocci, C; Masi, L; Pacini, F; Opocher, G; Radicioni, A; Tonacchera, M; Vigneri, R; Zatelli, M C; Brandi, M L

    2015-02-01

    Rare endocrine-metabolic diseases (REMD) represent an important area in the field of medicine and pharmacology. The rare diseases of interest to endocrinologists involve all fields of endocrinology, including rare diseases of the pituitary, thyroid and adrenal glands, paraganglia, ovary and testis, disorders of bone and mineral metabolism, energy and lipid metabolism, water metabolism, and syndromes with possible involvement of multiple endocrine glands, and neuroendocrine tumors. Taking advantage of the constitution of a study group on REMD within the Italian Society of Endocrinology, consisting of basic and clinical scientists, a document on the taxonomy of REMD has been produced. This document has been designed to include mainly REMD manifesting or persisting into adulthood. The taxonomy of REMD of the adult comprises a total of 166 main disorders, 338 including all variants and subtypes, described into 11 tables. This report provides a complete taxonomy to classify REMD of the adult. In the future, the creation of registries of rare endocrine diseases to collect data on cohorts of patients and the development of common and standardized diagnostic and therapeutic pathways for each rare endocrine disease is advisable. This will help planning and performing intervention studies in larger groups of patients to prove the efficacy, effectiveness, and safety of a specific treatment.

  12. High seropositivity of IgG and IgM antibodies against ...

    Objective: This study reports on the high seropositivity of immunoglobulin (Ig) G and M antibodies against CMV and the risk factors for CMV ... sex, highly active antiretroviral therapy (HAART) were not statistically associated with CMV seropositivity in this study. ... are infected with HIV have detectable IgG antibodies to CMV ...

  13. Clinical Practice Guidelines for Rare Diseases: The Orphanet Database.

    Sonia Pavan

    Full Text Available Clinical practice guidelines (CPGs for rare diseases (RDs are scarce, may be difficult to identify through Internet searches and may vary in quality depending on the source and methodology used. In order to contribute to the improvement of the diagnosis, treatment and care of patients, Orphanet (www.orpha.net has set up a procedure for the selection, quality evaluation and dissemination of CPGs, with the aim to provide easy access to relevant, accurate and specific recommendations for the management of RDs. This article provides an analysis of selected CPGs by medical domain coverage, prevalence of diseases, languages and type of producer, and addresses the variability in CPG quality and availability. CPGs are identified via bibliographic databases, websites of research networks, expert centres or medical societies. They are assessed according to quality criteria derived from the Appraisal of Guidelines, REsearch and Evaluation (AGREE II Instrument. Only open access CPGs and documents for which permission from the copyright holders has been obtained are disseminated on the Orphanet website. From January 2012 to July 2015, 277 CPGs were disseminated, representing coverage of 1,122 groups of diseases, diseases or subtypes in the Orphanet database. No language restriction is applied, and so far 10 languages are represented, with a predominance of CPGs in English, French and German (92% of all CPGs. A large proportion of diseases with identified CPGs belong to rare oncologic, neurologic, hematologic diseases or developmental anomalies. The Orphanet project on CPG collection, evaluation and dissemination is a continuous process, with regular addition of new guidelines, and updates. CPGs meeting the quality criteria are integrated to the Orphanet database of rare diseases, together with other types of textual information and the appropriate services for patients, researchers and healthcare professionals in 40 countries.

  14. Asymptomatic HLA-A*02:01–Restricted Epitopes from Herpes Simplex Virus Glycoprotein B Preferentially Recall Polyfunctional CD8+ T Cells from Seropositive Asymptomatic Individuals and Protect HLA Transgenic Mice against Ocular Herpes

    Dervillez, Xavier; Qureshi, Huma; Chentoufi, Aziz A.; Khan, Arif A.; Kritzer, Elizabeth; Yu, David C.; Diaz, Oscar R.; Gottimukkala, Chetan; Kalantari, Mina; Villacres, Maria C.; Scarfone, Vanessa M.; McKinney, Denise M.; Sidney, John; Sette, Alessandro; Nesburn, Anthony B.; Wechsler, Steven L.; BenMohamed, Lbachir

    2014-01-01

    Evidence from C57BL/6 mice suggests that CD8+ T cells, specific to the immunodominant HSV-1 glycoprotein B (gB) H-2b–restricted epitope (gB498–505), protect against ocular herpes infection and disease. However, the possible role of CD8+ T cells, specific to HLA-restricted gB epitopes, in protective immunity seen in HSV-1–seropositive asymptomatic (ASYMP) healthy individuals (who have never had clinical herpes) remains to be determined. In this study, we used multiple prediction algorithms to identify 10 potential HLA-A*02:01–restricted CD8+ T cell epitopes from the HSV-1 gB amino acid sequence. Six of these epitopes exhibited high-affinity binding to HLA-A*02:01 molecules. In 10 sequentially studied HLA-A*02:01–positive, HSV-1–seropositive ASYMP individuals, the most frequent, robust, and polyfunctional CD8+ T cell responses, as assessed by a combination of tetramer, IFN-γ-ELISPOT, CFSE proliferation, CD107a/b cytotoxic degranulation, and multiplex cytokine assays, were directed mainly against epitopes gB342–350 and gB561–569. In contrast, in 10 HLA-A*02:01–positive, HSV-1–seropositive symptomatic (SYMP) individuals (with a history of numerous episodes of recurrent clinical herpes disease) frequent, but less robust, CD8+ T cell responses were directed mainly against nonoverlapping epitopes (gB183–191 and gB441–449). ASYMP individuals had a significantly higher proportion of HSV-gB–specific CD8+ T cells expressing CD107a/b degranulation marker and producing effector cytokines IL-2, IFN-γ, and TNF-α than did SYMP individuals. Moreover, immunization of a novel herpes-susceptible HLA-A*02:01 transgenic mouse model with ASYMP epitopes, but not with SYMP epitopes, induced strong CD8+ T cell–dependent protective immunity against ocular herpes infection and disease. These findings should guide the development of a safe and effective T cell–based herpes vaccine. PMID:24101547

  15. Identification of Candida dubliniensis in a study of HIV-seropositive pediatric dental patients.

    Brown, D M; Jabra-Rizk, M A; Falkler, W A; Baqui, A A; Meiller, T F

    2000-01-01

    The combination of an immature immune system and suppressed cellular immunity in children with HIV infections provides optimal conditions for rapid disease progression. As a result, pediatric AIDS has become a major epidemiological challenge. Oral fungal colonization remains one of the most common opportunistic infections observed in both adult and pediatric HIV infected patients. Although Candida albicans is the most frequently isolated opportunistic fungal species, a recently characterized Candida species, C. dubliniensis, has gained considerable attention due to its almost exclusive association with HIV-seropositive individuals. The purpose of this study was to prospectively screen for the presence of C. dubliniensis among pediatric HIV+ patients. Oral samples taken from twenty-seven children were cultured for the presence of yeast. All positive yeast isolates obtained were screened for the presence of C. dubliniensis by use of tests for germ tube and chlamydospore production, detection of inability to grow at 45 degrees C, by colony color on CHROMagar Candida medium, coaggregation with Fusobacterium nucleatum ATCC 49256 and by the results of sugar assimilation testing with the API 20C AUX yeast identification system. Among the 27 patients tested, 3 patients were found to harbor C. dubliniensis, one of which also grew C. glabrata; 12 patients were colonized with C. albicans, while the remaining 12 patients were negative for yeast. Identification of the three C. dubliniensis isolates was genetically confirmed by electrophoretic karyotyping. All three C. dubliniensis isolates were found to be susceptible to fluconazole (MIC pediatric HIV seropositive population and support the need for further investigation into the prevalence and pathogenesis of C. dubliniensis.

  16. The Seropositivity Rate of Atypical Agents in Patients with Community-Acquired Pneumonia

    Ruhan Karakoc Gunes

    2007-08-01

    Full Text Available The aim of this study was to investigate the IgM antibody positivities of atypical pneumonia agents in patients with community-acquired pneumonia (CAP, and to compare the results with controls. The serum samples which were collected from 87 adult patients and 21 healthy controls have been investigated by a commercial ELISA (Pneumobact ELISA IgM, Vircell, Spain in which four different atypical pneumonia agents were fixed onto a slide. In the patients group, IgM positivity rates for the agents were as follows, respectively; 2.3% for Legionella pneumophila, 56.3% Chlamydia pneumoniae, 33.3% for Mycoplasma pneumoniae, 9.2% for Coxiella burnetii. The rates of IgM positivities in the control group varied 7% for all of the agents except M. Pneumoniae and C. Pneumoniae and 2 of these controls were positive for L. Pneumophila IgM, one was positive for C. Burnetii IgM. According to the statistical evaluation, there were significant differences for IgM seropositivities to Mycoplasma pneumoniae and Chlamydia Pneumoniae,between the patient and the control groups (p0.05. We showed that the seropositivity rate of atypical agents in patients with CAP was significantly higher when compared to healthy control group. This result suggests us, atypical agents might be responsible in CAP patients in a great amount. Furthermore, our study also suggests that clinical and radiological findings are not useful for discriminating atypical from typical pneumonia. [TAF Prev Med Bull 2007; 6(4.000: 279-284

  17. Idiopathic Canalicular Inflammatory Disease: New Disease Description of Clinical Patterns, Investigations, Management, and Outcomes.

    Ali, Mohammad Javed

    2018-01-25

    The objective of this perspective is to present a separate disease description of "idiopathic canalicular inflammatory disease" and outline the diagnostic criteria and early experiences with its investigations and management. Retrospective case series of 44 canaliculi of 22 eyes of 11 patients presenting at a tertiary care Dacryology service over a period of 2 years with typical clinical patterns of inflammatory canaliculitis and its outcomes were studied. All the patients underwent microbiological work-up with culture and sensitivity, dacryoendoscopy imaging, serial Fourier domain ocular coherence tomography, and collagen vascular profiles. Stages in the evolution of the disease were studied. All patients were treated initially with topical steroids followed by punctal dilatation and placement of mini-monoka stents. Five patients in addition had a small biopsy from the inflamed portion of the vertical canaliculus. Stents were extubated at 6 weeks. Forty-four canaliculi were diagnosed to have idiopathic canalicular inflammatory disease during the study period. There was a female preponderance (81.8%, 9/11) and the mean age at presentation was 57 years. All patients presented with unilateral epiphora without any discharge, pain, or swelling. Collagen vascular profiles and screening for autoimmune diseases were negative. Clinical picture ranged from stages 1 to 5, consisting of edema, progressive centripetal vascularization, pouting of vascularized mucosa, membrane formation, and progressive scarring. The presentation begins in 1 eye and usually involves the other eye at a mean of 6 months. Ocular coherence tomography and dacryoendoscopy were of adjunctive value in the diagnosis. Histopathological examination was suggestive of a chronic inflammation. All patients had relentless progression to end-stage disease, although delayed significantly by steroids and monoka intubation. Idiopathic canalicular inflammatory disease has a distinct and typical clinical behavior and

  18. Wilson's disease in children: clinical and diagnostic features

    Ayesha, H.; Choudhry, A.A.; Javed, M.T.; Javed, F.

    2002-01-01

    Objective: To study the clinical and diagnostic laboratory features of Wilsons disease in children and adolescents. Design: A prospective cohort study. Place and Duration of study: The study included patients diagnosed as Wilson s disease at the Department of Pediatrics Allied Hospital, Punjab medical College, Faisalabad from May 1997 to June 2001. Patients and methods: Patients presenting with liver or suggestive neurological disease were investigated. Others were diagnosed as a result of family screening. Diagnosis of neurologic disease was made if two of the following were present: Typical neurological findings, Kayser Fleischer corneal rings and low serum ceruloplasmin ( 100 mu gm) free serum copper (>10 mu gm/dl). In other forms and for family screening, 24 hours. Urinary copper (> 100 mu gm), free serum copper (>10 mu gm/dl), and wherever possible liver biopsy for histopathology and cytochemical staining by rubeanic acid was also done. Results: Twenty-seven patients with a mean age of 10.2 years were diagnosed as suffering from Wilson disease. Mean age for hepatic and neurological disease was 9 years and 11.5 years respectively. Youngest patient (neurologic) was 6 years old. 48% cases presented with neurological, 41% with hepatic and 4% with skeletal manifestations while 7 % were asymptomatic. Mean duration of symptoms before diagnosis was 6.1 months. Dysarthria (84.6%), tremors (69.2%), rigidity and poor school performance and hand writing (61.5%), dysphagia (46.1%) and dystonia (38.5%), were the most common neurologic findings. Chronic liver disease was seen in 73 % while acute forms were seen in 27 % cases. Two cases presented with fulminant hepatic failure. Consanguineous marriage of the parents was found in 70 % and family history of disease was present in 65 % cases. K-F (Kayser Fleischer) rings and low serum ceruloplasmin(<20 mg/dl) was found in 85% of all patients. In non neurologic types other tests of copper metabolism were done. Elevated urinary

  19. The clinical use of structural MRI in Alzheimer disease

    Frisoni, Giovanni B.; Fox, Nick C.; Jack, Clifford R.; Scheltens, Philip; Thompson, Paul M.

    2010-01-01

    Structural imaging based on magnetic resonance is an integral part of the clinical assessment of patients with suspected Alzheimer dementia. Prospective data on the natural history of change in structural markers from preclinical to overt stages of Alzheimer disease are radically changing how the disease is conceptualized, and will influence its future diagnosis and treatment. Atrophy of medial temporal structures is now considered to be a valid diagnostic marker at the mild cognitive impairment stage. Structural imaging is also included in diagnostic criteria for the most prevalent non-Alzheimer dementias, reflecting its value in differential diagnosis. In addition, rates of whole-brain and hippocampal atrophy are sensitive markers of neurodegeneration, and are increasingly used as outcome measures in trials of potentially disease-modifying therapies. Large multicenter studies are currently investigating the value of other imaging and nonimaging markers as adjuncts to clinical assessment in diagnosis and monitoring of progression. The utility of structural imaging and other markers will be increased by standardization of acquisition and analysis methods, and by development of robust algorithms for automated assessment. PMID:20139996

  20. [Adult congenital heart disease--between guidelines and clinical practice].

    Chessa, Massimo

    2011-01-01

    Advances in medical and surgical management of congenital heart disease have changed the prognosis of infants and children with cardiac defects, so that an increasing number of patients reach adolescence and adult life, even those with complex defects. Recent data suggest that the number of adults with congenital heart disease, either repaired or not, approaches the number of children with the disorder. A cure is rarely achieved and ongoing surveillance and management in conjunction with specialists in this highly specialized field is mandatory to provide optimal care for patients. The profile of this patient population is going to change over the next few decades. Ideally specialist units should be established in appropriate geographic locations; patients need to be concentrated for expertise, experience, and optimal management. Less specialized regional centers and outpatient clinics in districts in connection with grown-up congenital heart disease units should be created. Specialist units should accept responsibility for educating the professionals, training the specialists, and sharing particular skills between each other. Guidelines and recommendations should help physicians to make decision in their daily practice. However, the final judgment regarding the care of an individual patient must be made by his/her physician. This article will briefly discuss some aspects of these dedicated guidelines and how they influence the clinical daily practice.

  1. Disease models of chronic inflammatory airway disease : applications and requirements for clinical trials

    Diamant, Zuzana; Clarke, Graham W.; Pieterse, Herman; Gispert, Juan

    Purpose of reviewThis review will discuss methodologies and applicability of key inflammatory models of respiratory disease in proof of concept or proof of efficacy clinical studies. In close relationship with these models, induced sputum and inflammatory cell counts will be addressed for

  2. Glycogen storage disease type I: clinical and laboratory profile

    Berenice L. Santos

    2014-12-01

    Full Text Available OBJECTIVES: To characterize the clinical, laboratory, and anthropometric profile of a sample of Brazilian patients with glycogen storage disease type I managed at an outpatient referral clinic for inborn errors of metabolism. METHODS: This was a cross-sectional outpatient study based on a convenience sampling strategy. Data on diagnosis, management, anthropometric parameters, and follow-up were assessed. RESULTS: Twenty-one patients were included (median age 10 years, range 1-25 years, all using uncooked cornstarch therapy. Median age at diagnosis was 7 months (range, 1-132 months, and 19 patients underwent liver biopsy for diagnostic confirmation. Overweight, short stature, hepatomegaly, and liver nodules were present in 16 of 21, four of 21, nine of 14, and three of 14 patients, respectively. A correlation was found between height-for-age and BMI-for-age Z-scores (r = 0.561; p = 0.008. CONCLUSIONS: Diagnosis of glycogen storage disease type I is delayed in Brazil. Most patients undergo liver biopsy for diagnostic confirmation, even though the combination of a characteristic clinical presentation and molecular methods can provide a definitive diagnosis in a less invasive manner. Obesity is a side effect of cornstarch therapy, and appears to be associated with growth in these patients.

  3. Whipple's disease. Report of five cases with different clinical features.

    Ferrari, M de L; Vilela, E G; Faria, L C; Couto, C A; Salgado, C J; Leite, V R; Brasileiro Filho, G; Bambirra, E A; Mendes, C M; Carvalho, S de C; de Oliveira, C A; da Cunha, A S

    2001-01-01

    Whipple's disease (WD) is a rare systemic disease of infectious etiology which involves the small intestine but can virtually affect any organ. We present here five cases (four males and one female) ranging in age from 20 to 59 years. All patients had intestinal involvement associated or not with clinical manifestations linked to this organ. Vegetation in the tricuspid valve was observed in one patient, suggesting endocarditis caused by Tropheryma whippelii, with disappearance of the echocardiographic alterations after treatment. In one of the male patients the initial clinical manifestation was serologically negative spondylitis, with no diarrhea occurring at any time during follow-up. Ocular involvement associated with intestinal malabsorption and significant weight loss were observed in one case. In the other two cases, diarrhea was the major clinical manifestation. All patients were diagnosed by histological examination of the jejunal mucosa and, when indicated, of extraintestinal tissues by light and electron microscopy. After antibiotic treatment, full remission of symptoms occurred in all cases. A control examination of the intestinal mucosa performed after twelve months of treatment with sulfamethoxazole-trimethoprim revealed the disappearance of T. whippelii in four patients. The remaining patient was lost to follow-up.

  4. Whipple's disease. Report of five cases with different clinical features

    FERRARI Maria de Lourdes de Abreu

    2001-01-01

    Full Text Available Whipple's disease (WD is a rare systemic disease of infectious etiology which involves the small intestine but can virtually affect any organ. We present here five cases (four males and one female ranging in age from 20 to 59 years. All patients had intestinal involvement associated or not with clinical manifestations linked to this organ. Vegetation in the tricuspid valve was observed in one patient, suggesting endocarditis caused by Tropheryma whippelii, with disappearance of the echocardiographic alterations after treatment. In one of the male patients the initial clinical manifestation was serologically negative spondylitis, with no diarrhea occurring at any time during follow-up. Ocular involvement associated with intestinal malabsorption and significant weight loss were observed in one case. In the other two cases, diarrhea was the major clinical manifestation. All patients were diagnosed by histological examination of the jejunal mucosa and, when indicated, of extraintestinal tissues by light and electron microscopy. After antibiotic treatment, full remission of symptoms occurred in all cases. A control examination of the intestinal mucosa performed after twelve months of treatment with sulfamethoxazole-trimethoprim revealed the disappearance of T. whippelii in four patients. The remaining patient was lost to follow-up.

  5. Mental Health of HIV-Seropositive Women During Pregnancy and Postpartum Period: A Comprehensive Literature Review

    Dass-Brailsford, Priscilla; Nora, Diana; Talisman, Nicholas

    2014-01-01

    With growing numbers of HIV-seropositive (HIV+) women of child-bearing age and increased access to effective clinical protocols for preventing mother-to-child transmission (MTCT) of HIV, mental health-related factors have become increasingly relevant due to their potential to affect the women’s quality of life, obstetric outcomes and risk of MTCT. This review synthesizes evidence from 53 peer-reviewed publications examining mental health-related variables in pregnant and postpartum HIV+ women. The presentation of results is organized by the level of socioeconomic resources in the countries where studies were conducted (i.e., high-, middle-, and low-income countries). It is concluded that psychiatric symptoms, particularly depression, and mental health vulnerabilities (e.g., inadequate coping skills) are widespread among pregnant HIV+ women globally and have a potential to affect psychological well-being, quality of life and salient clinical outcomes. The current body of evidence provides rationale for developing and evaluating clinical and structural interventions aimed at improving mental health outcomes and their clinical correlates in pregnant HIV+ women. PMID:24584458

  6. Moyamoya Disease Clinical Course and Severity in Childhood

    Ayse Kacar Bayram

    2016-01-01

    Full Text Available Aim: Moyamoya disease (MMD is a rare, progressive and oclusive cerebrovascular disorder, predominantly affecting the terminal segment of the internal carotid arteries (ICA and its main branches. The purpose of this study is to evaluate the clinical course and severity of MMD in pediatric patients. Material and Method: We examined 5 consecutive pediatric patients with MMD, focusing on clinical and radiological features, the therapy and outcome over the 58-month follow-up period. Results: The study population consisted of 3 boys and 2 girls. The mean age at diagnosis of patients was 7.2 ± 3.4 years (age range: 3-10 years. The mean duration of follow-up was 30.4 ± 17.4 months (follow-up interval: 12-58 months. Neurological findings at presentation included: motor deficit in 4 patients (80.0%, epileptic seizures in 2 patients (40.0%, movement disorders in 3 patients (60.0%, and headache in 1 patients (20.0%. There was areas of infarction on brain MRI in all patients. Angiographic findings included: internal carotid artery stenosis in all patients, anterior cerebral artery stenosis in 3 patients, middle cerebral artery stenosis in 3 patients, posterior cerebral artery stenosis in 2 patients, and vertebral artery stenosis in 1 patient. Enoxaparine therapy was started to all patients. Subdural hematoma developed in 1 patient during follow-up. Cerebral infarctions recurred despite medical treatment in 4 patients. Discussion: Although this disease is rare, it is an important cause of pediatric stroke. MMD shows different clinical course and disease severity in childhood. Early diagnosis and appropriate treatment are crucial.

  7. Sarcopenia in Alcoholic Liver Disease: Clinical and Molecular Advances.

    Dasarathy, Jaividhya; McCullough, Arthur J; Dasarathy, Srinivasan

    2017-08-01

    Despite advances in treatment of alcohol use disorders that focus on increasing abstinence and reducing recidivism, alcoholic liver disease (ALD) is projected to be the major cause of cirrhosis and its complications. Malnutrition is recognized as the most frequent complication in ALD, and despite the high clinical significance, there are no effective therapies to reverse malnutrition in ALD. Malnutrition is a relatively imprecise term, and sarcopenia or skeletal muscle loss, the major component of malnutrition, is primarily responsible for the adverse clinical consequences in patients with liver disease. It is, therefore, critical to define the specific abnormality (sarcopenia) rather than malnutrition in ALD, so that therapies targeting sarcopenia can be developed. Skeletal muscle mass is maintained by a balance between protein synthesis and proteolysis. Both direct effects of ethanol (EtOH) and its metabolites on the skeletal muscle and the consequences of liver disease result in disturbed proteostasis (protein homeostasis) and consequent sarcopenia. Once cirrhosis develops in patients with ALD, abstinence is unlikely to be effective in completely reversing sarcopenia, as other contributors including hyperammonemia, hormonal, and cytokine abnormalities aggravate sarcopenia and maintain a state of anabolic resistance initiated by EtOH. Cirrhosis is also a state of accelerated starvation, with increased gluconeogenesis that requires amino acid diversion from signaling and substrate functions. Novel therapeutic options are being recognized that are likely to supplant the current "deficiency replacement" approach and instead focus on specific molecular perturbations, given the increasing availability of small molecules that can target specific signaling components. Myostatin antagonists, leucine supplementation, and mitochondrial protective agents are currently in various stages of evaluation in preclinical studies to prevent and reverse sarcopenia, in cirrhosis in

  8. Clinical evaluation of MRI for lumbar spinal disease

    Moriwaka, Yosuke; Nakayama, Sachiko; Murai, Nobuko; Watanabe, Katsushi; Kawano, Keiichiro; Tajima, Naoya; Kihara, Yasushi; Okada, Akihiko; Fujimoto, Toshiro.

    1985-01-01

    Thirty-two patients were examined to determine the clinical usefulness of magnetic resonance imaging (MRI) of the lumbar spine. MRI using a resistive magnet at 0.1 Tesla was compared with computed tomography (CT) and myelography. A saturation recovery (SR) technique with repetition time (TR) of 500 msec was used in all cases. There were 19 cases with disk herniation, 5 with canal stenosis, 2 with spondylosis deformans and 6 with other suspected disease. MRI showed the protrusion of disk, displacement of epidural fat and degenerative change of disk more clearly than CT and myelography. Of the 12 herniated disks, 8 disks were clearly shown and one was not detected by MRI. In some cases, herniated disk could not be differentiated from disk bulging. With further technical advance, MRI is likely to become the initial procedure for evaluation of lumbar spinal disease. (author)

  9. Clinical effect of Dilazep on ischemic heart disease

    Tsuda, Takashi; Hayashi, Senji; Shibata, Akira; Hama, Hitoshi; Mitani, Tohru.

    1982-01-01

    Dilazep tablets (300 mg/day) were administered to 9 patients with ischemic heart disease for more than 2 months. Stress myocardial scintigraphy was performed before and after the treatment to examine the clinical effect of this drug on the heart. The improvement rate of subjective symptoms was 57% (4/7 cases). No significant difference was observed in double product by the ergometer before and after the treatment, nor were any significant changes observed in ST by Master's two-step exercise test in any patient. The pre- to posttreatment improvement rate of myocardial uptake, demonstrated by stress myocardial scintigraphy, was 89% (8/9 cases). Thus, Dilazep tablets seemed to increase the blood flow in the ischemic area of the myocardium during exercise in ischemic heart disease. (Chiba, N.)

  10. Clinical evaluation of echography in diagnosis of thyroid disease

    Fritzsche, H.; Braendle, J.

    1983-01-01

    In 63 patients echography of thyroid was performed additionally to case history, palpation, scintigraphy and hormone tests for evaluating clinical significance of this method. The benefit of this technique is rapid measurement of thyroid size, demonstration of nodules in palpable diffuse goiters and differentiating of solid or cystic nodules of the thyroid. For diagnosis of autonomous areas in the thyroid scintigrahy remains the method of choice. Also there is no correlation of ultrasound findings and thyroid function. In routine diagnostic procedure of thyroid disease echography may replace scintigraphy only in diffuse goiter and if radionuclide imaging is not possible. Nevertheless ultrasonic evaluation of the thyroid is an important additional method in diagnostic of thyroid diseases. (Author)

  11. Epidemiology and introduction to the clinical presentation of Wilson disease.

    Lo, Christine; Bandmann, Oliver

    2017-01-01

    Our understanding of the epidemiology of Wilson disease has steadily grown since Sternlieb and Scheinberg's first prevalence estimate of 5 per million individuals in 1968. Increasingly sophisticated genetic techniques have led to revised genetic prevalence estimates of 142 per million. Various population isolates exist where the prevalence of Wilson disease is higher still, the highest being 885 per million from within the mountainous region of Rucar in Romania. In Sardinia, where the prevalence of Wilson disease has been calculated at 370 per million births, six mutations account for around 85% of Wilson disease chromosomes identified. Significant variation in the patterns of presentation may however exist, even between individuals carrying the same mutations. At either extremes of presentation are an 8-month-old infant with abnormal liver function tests and individuals diagnosed in their eighth decade of life. Three main patterns of presentation have been recognized - hepatic, neurologic, and psychiatric - prompting their presentation to a diverse range of specialists. Deviations in the family history from the anticipated autosomal-recessive mode of inheritance, with apparent "pseudodominance" and mechanisms of inheritance that include uniparental isodisomy (the inheritance of both chromosomal copies from a single parent), may all further cloud the diagnosis. It can therefore take the efforts of an astute clinician with a high clinical index of suspicion to clinch the diagnosis of this eminently treatable condition. © 2017 Elsevier B.V. All rights reserved.

  12. Acute Psychosis as Major Clinical Presentation of Legionnaires’ Disease

    Ricardo Coentre

    2016-01-01

    Full Text Available We report a case of a 61-year-old woman who presented with acute psychosis as a major manifestation of Legionnaires’ disease in the absence of other neuropsychiatric symptoms. Clinical history revealed dry cough and nausea. Observation showed fever and auscultation crackles in the lower lobe of the right lung. Laboratory testing demonstrated elevated C-reactive protein and lung chest radiograph showed patchy peribronchial and right lower lobe consolidation. Soon after admission, she started producing purulent sputum. Epidemiological data suggested Legionella pneumophila as possible cause of the clinical picture that was confirmed by urinary antigen detection and polymerase chain reaction of the sputum. She was treated with levofloxacin 750 mg/day for 10 days with complete remission of pulmonary and psychiatric symptoms. She has not had further psychotic symptoms.

  13. Significance of mammographic signs in clinically occult disease

    Waal, J.C. de; Steil, B.; Baltzer, J.; Vaillant, W.; Zander, J.

    1987-10-01

    The significance of various radiographic signs in 183 patients with clinically occult breast disease is described. 30.6% hat a carcinoma of the breast or a carcinoma in situ. The radiological features have varying predictive values and there is variation in the incidence of lymph node metastases. It is considered useful to classify the radiological appearances under the headings of round foci, star-shaped opacities, diffuse opacities, opacities with calcification and groups of micro-calcification. Despite the early diagnosis, 24% of patients already had lymph node metastases.

  14. Imaging and clinical characteristics of sporadic Creutzfeldt-Jakob disease

    HAN Shun-chang

    2013-04-01

    Full Text Available Five patients with sporadic Creutzfeldt-Jakob disease (sCJD presented rapidly progressive dementia which were subacute onset from 1 to 4 months. Among these cases, periodic synchronous discharge (PSD of electroencephalography (EEG was seen in 2 patients. Besides, 4 patients obtained positive results in cerebrospinal fluid (CSF analysis for 14-3-3 protein. The cranial MRI examination showed symmetrical or asymmetrical colored-ribbon-shaped high signals in cerebral cortex or basal ganglia by diffusion weighted imaging (DWI, suggesting that DWI had high sensitivity and specificity for the diagnosis of sCJD as a preferred method in the clinical examination of sCJD.

  15. Clinical neurogenetics: behavioral management of inherited neurodegenerative disease.

    Wexler, Eric

    2013-11-01

    Psychiatric symptoms often manifest years before overt neurologic signs in patients with inherited neurodegenerative disease. The most frequently cited example of this phenomenon is the early onset of personality changes in "presymptomatic" Huntington patients. In some cases the changes in mood and cognition are even more debilitating than their neurologic symptoms. The goal of this article is to provide the neurologist with a concise primer that can be applied in a busy clinic or private practice. Copyright © 2013 Elsevier Inc. All rights reserved.

  16. A Combination of CD28 (rs1980422 and IRF5 (rs10488631 Polymorphisms Is Associated with Seropositivity in Rheumatoid Arthritis: A Case Control Study.

    Lucia Vernerova

    Full Text Available The aim of the study was to analyse genetic architecture of RA by utilizing multiparametric statistical methods such as linear discriminant analysis (LDA and redundancy analysis (RDA.A total of 1393 volunteers, 499 patients with RA and 894 healthy controls were included in the study. The presence of shared epitope (SE in HLA-DRB1 and 11 SNPs (PTPN22 C/T (rs2476601, STAT4 G/T (rs7574865, CTLA4 A/G (rs3087243, TRAF1/C5 A/G (rs3761847, IRF5 T/C (rs10488631, TNFAIP3 C/T (rs5029937, AFF3 A/T (rs11676922, PADI4 C/T (rs2240340, CD28 T/C (rs1980422, CSK G/A (rs34933034 and FCGR3A A/C (rs396991, rheumatoid factor (RF, anti-citrullinated protein antibodies (ACPA and clinical status was analysed using the LDA and RDA.HLA-DRB1, PTPN22, STAT4, IRF5 and PADI4 significantly discriminated between RA patients and healthy controls in LDA. The correlation between RA diagnosis and the explanatory variables in the model was 0.328 (Trace = 0.107; F = 13.715; P = 0.0002. The risk variants of IRF5 and CD28 genes were found to be common determinants for seropositivity in RDA, while positivity of RF alone was associated with the CTLA4 risk variant in heterozygous form. The correlation between serologic status and genetic determinants on the 1st ordinal axis was 0.468, and 0.145 on the 2nd one (Trace = 0.179; F = 6.135; P = 0.001. The risk alleles in AFF3 gene together with the presence of ACPA were associated with higher clinical severity of RA.The association among multiple risk variants related to T cell receptor signalling with seropositivity may play an important role in distinct clinical phenotypes of RA. Our study demonstrates that multiparametric analyses represent a powerful tool for investigation of mutual relationships of potential risk factors in complex diseases such as RA.

  17. A Combination of CD28 (rs1980422) and IRF5 (rs10488631) Polymorphisms Is Associated with Seropositivity in Rheumatoid Arthritis: A Case Control Study.

    Vernerova, Lucia; Spoutil, Frantisek; Vlcek, Miroslav; Krskova, Katarina; Penesova, Adela; Meskova, Milada; Marko, Andrea; Raslova, Katarina; Vohnout, Branislav; Rovensky, Jozef; Killinger, Zdenko; Jochmanova, Ivana; Lazurova, Ivica; Steiner, Guenter; Smolen, Josef; Imrich, Richard

    2016-01-01

    The aim of the study was to analyse genetic architecture of RA by utilizing multiparametric statistical methods such as linear discriminant analysis (LDA) and redundancy analysis (RDA). A total of 1393 volunteers, 499 patients with RA and 894 healthy controls were included in the study. The presence of shared epitope (SE) in HLA-DRB1 and 11 SNPs (PTPN22 C/T (rs2476601), STAT4 G/T (rs7574865), CTLA4 A/G (rs3087243), TRAF1/C5 A/G (rs3761847), IRF5 T/C (rs10488631), TNFAIP3 C/T (rs5029937), AFF3 A/T (rs11676922), PADI4 C/T (rs2240340), CD28 T/C (rs1980422), CSK G/A (rs34933034) and FCGR3A A/C (rs396991), rheumatoid factor (RF), anti-citrullinated protein antibodies (ACPA) and clinical status was analysed using the LDA and RDA. HLA-DRB1, PTPN22, STAT4, IRF5 and PADI4 significantly discriminated between RA patients and healthy controls in LDA. The correlation between RA diagnosis and the explanatory variables in the model was 0.328 (Trace = 0.107; F = 13.715; P = 0.0002). The risk variants of IRF5 and CD28 genes were found to be common determinants for seropositivity in RDA, while positivity of RF alone was associated with the CTLA4 risk variant in heterozygous form. The correlation between serologic status and genetic determinants on the 1st ordinal axis was 0.468, and 0.145 on the 2nd one (Trace = 0.179; F = 6.135; P = 0.001). The risk alleles in AFF3 gene together with the presence of ACPA were associated with higher clinical severity of RA. The association among multiple risk variants related to T cell receptor signalling with seropositivity may play an important role in distinct clinical phenotypes of RA. Our study demonstrates that multiparametric analyses represent a powerful tool for investigation of mutual relationships of potential risk factors in complex diseases such as RA.

  18. Clinical value of natriuretic peptides in chronic kidney disease.

    Santos-Araújo, Carla; Leite-Moreira, Adelino; Pestana, Manuel

    2015-01-01

    According to several lines of evidence, natriuretic peptides (NP) are the main components of a cardiac-renal axis that operate in clinical conditions of decreased cardiac hemodynamic tolerance to regulate sodium homeostasis, blood pressure and vascular function. Even though it is reasonable to assume that NP may exert a relevant role in the adaptive response to renal mass ablation, evidence gathered so far suggest that this contribution is probably complex and dependent on the type and degree of the functional mass loss. In the last years NP have been increasingly used to diagnose, monitor treatment and define the prognosis of several cardiovascular (CV) diseases. However, in many clinical settings, like chronic kidney disease (CKD), the predictive value of these biomarkers has been questioned. In fact, it is now well established that renal function significantly affects the plasmatic levels of NP and that renal failure is the clinical condition associated with the highest plasmatic levels of these peptides. The complexity of the relation between NP plasmatic levels and CV and renal functions has obvious consequences, as it may limit the predictive value of NP in CV assessment of CKD patients and be a demanding exercise for clinicians involved in the daily management of these patients. This review describes the role of NP in the regulatory response to renal function loss and addresses the main factors involved in the clinical valorization of the peptides in the context of significant renal failure. Copyright © 2015 The Authors. Published by Elsevier España, S.L.U. All rights reserved.

  19. Targeting Prodromal Alzheimer Disease With Avagacestat: A Randomized Clinical Trial.

    Coric, Vladimir; Salloway, Stephen; van Dyck, Christopher H; Dubois, Bruno; Andreasen, Niels; Brody, Mark; Curtis, Craig; Soininen, Hilkka; Thein, Stephen; Shiovitz, Thomas; Pilcher, Gary; Ferris, Steven; Colby, Susan; Kerselaers, Wendy; Dockens, Randy; Soares, Holly; Kaplita, Stephen; Luo, Feng; Pachai, Chahin; Bracoud, Luc; Mintun, Mark; Grill, Joshua D; Marek, Ken; Seibyl, John; Cedarbaum, Jesse M; Albright, Charles; Feldman, Howard H; Berman, Robert M

    2015-11-01

    Early identification of Alzheimer disease (AD) is important for clinical management and affords the opportunity to assess potential disease-modifying agents in clinical trials. To our knowledge, this is the first report of a randomized trial to prospectively enrich a study population with prodromal AD (PDAD) defined by cerebrospinal fluid (CSF) biomarker criteria and mild cognitive impairment (MCI) symptoms. To assess the safety of the γ-secretase inhibitor avagacestat in PDAD and to determine whether CSF biomarkers can identify this patient population prior to clinical diagnosis of dementia. A randomized, placebo-controlled phase 2 clinical trial with a parallel, untreated, nonrandomized observational cohort of CSF biomarker-negative participants was conducted May 26, 2009, to July 9, 2013, in a multicenter global population. Of 1358 outpatients screened, 263 met MCI and CSF biomarker criteria for randomization into the treatment phase. One hundred two observational cohort participants who met MCI criteria but were CSF biomarker-negative were observed during the same study period to evaluate biomarker assay sensitivity. Oral avagacestat or placebo daily. Safety and tolerability of avagacestat. Of the 263 participants in the treatment phase, 132 were randomized to avagacestat and 131 to placebo; an additional 102 participants were observed in an untreated observational cohort. Avagacestat was relatively well tolerated with low discontinuation rates (19.6%) at a dose of 50 mg/d, whereas the dose of 125 mg/d had higher discontinuation rates (43%), primarily attributable to gastrointestinal tract adverse events. Increases in nonmelanoma skin cancer and nonprogressive, reversible renal tubule effects were observed with avagacestat. Serious adverse event rates were higher with avagacestat (49 participants [37.1%]) vs placebo (31 [23.7%]), attributable to the higher incidence of nonmelanoma skin cancer. At 2 years, progression to dementia was more frequent in the PDAD

  20. Measurement of leukocyte rheology in vascular disease: clinical rationale and methodology. International Society of Clinical Hemorheology.

    Wautier, J L; Schmid-Schönbein, G W; Nash, G B

    1999-01-01

    The measurement of leukocyte rheology in vascular disease is a recent development with a wide range of new opportunities. The International Society of Clinical Hemorheology has asked an expert panel to propose guidelines for the investigation of leukocyte rheology in clinical situations. This article first discusses the mechanical, adhesive and related functional properties of leukocytes (especially neutrophils) which influence their circulation, and establishes the rationale for clinically-related measurements of parameters which describe them. It is concluded that quantitation of leukocyte adhesion molecules, and of their endothelial receptors may assist understanding of leukocyte behaviour in vascular disease, along with measurements of flow resistance of leukocytes, free radical production, degranulation and gene expression. For instance, vascular cell adhesion molecule (VCAM-1) is abnormally present on endothelial cells in atherosclerosis, diabetes mellitus and inflammatory conditions. Soluble forms of intercellular adhesion molecule (ICAM-1) or VCAM can be found elevated in the blood of patients with rheumatoid arthritis or infections disease. In the second part of the article, possible technical approaches are presented and possible avenues for leukocyte rheological investigations are discussed.

  1. Atypical Clinical Manifestations of Graves' Disease: An Analysis in Depth

    Hegazi, Mohamed Osama; Ahmed, Sherif

    2012-01-01

    Over the past few decades, there has been an increase in the number of reports about newly recognized (atypical or unusual) manifestations of Graves' disease (GD), that are related to various body systems. One of these manifestations is sometimes the main presenting feature of GD. Some of the atypical manifestations are specifically related to GD, while others are also similarly seen in patients with other forms of hyperthyroidism. Lack of knowledge of the association between these findings and GD may lead to delay in diagnosis, misdiagnosis, or unnecessary investigations. The atypical clinical presentations of GD include anemia, vomiting, jaundice, and right heart failure. There is one type of anemia that is not explained by any of the known etiological factors and responds well to hyperthyroidism treatment. This type of anemia resembles anemia of chronic disease and may be termed GD anemia. Other forms of anemia that are associated with GD include pernicious anemia, iron deficiency anemia of celiac disease, and autoimmune hemolytic anemia. Vomiting has been reported as a presenting feature of Graves' disease. Some cases had the typical findings of hyperthyroidism initially masked, and the vomiting did not improve until hyperthyroidism has been detected and treated. Hyperthyroidism may present with jaundice, and on the other hand, deep jaundice may develop with the onset of overt hyperthyroidism in previously compensated chronic liver disease patients. Pulmonary hypertension is reported to be associated with GD and to respond to its treatment. GD-related pulmonary hypertension may be so severe to produce isolated right-sided heart failure that is occasionally found as the presenting manifestation of GD. PMID:22132347

  2. Clinical spectrum of Castleman disease-associated neuropathy.

    Naddaf, Elie; Dispenzieri, Angela; Mandrekar, Jay; Mauermann, Michelle L

    2016-12-06

    To define the peripheral neuropathy phenotypes associated with Castleman disease. We conducted a retrospective chart review for patients with biopsy-proven Castleman disease evaluated between January 2003 and December 2014. Patients with associated peripheral neuropathy were identified and divided into 2 groups: those with Castleman disease without POEMS syndrome (CD-PN) and those with Castleman disease with POEMS syndrome (CD-POEMS). We used a cohort of patients with POEMS as controls. Clinical, electrodiagnostic, and laboratory characteristics were collected and compared among patient subgroups. There were 7 patients with CD-PN, 20 with CD-POEMS, and 122 with POEMS. Patients with CD-PN had the mildest neuropathy characterized by predominant sensory symptoms with no pain and mild distal sensory deficits (median Neuropathy Impairment Score of 7 points). Although both patients with CD-POEMS and patients with POEMS had a severe sensory and motor neuropathy, patients with CD-POEMS were less affected (median Neuropathy Impairment Score of 33 and 66 points, respectively). The degree of severity was also reflected on electrodiagnostic testing in which patients with CD-PN demonstrated a mild degree of axonal loss, followed by patients with CD-POEMS and then those with POEMS. Demyelinating features, defined by European Federation of Neurologic Societies/Peripheral Nerve Society criteria, were present in 43% of the CD-PN, 78% of the CD-POEMS, and 86% of the POEMS group. There is a spectrum of demyelinating peripheral neuropathies associated with Castleman disease. CD-PN is sensory predominant and is the mildest phenotype, whereas CD-POEMS is a more severe sensory and motor neuropathy. Compared to the POEMS cohort, those with CD-POEMS neuropathy have a similar but less severe phenotype. Whether these patients respond differently to treatment deserves further study. © 2016 American Academy of Neurology.

  3. Clinical, psychophysiological and psychological aspects of risk factors of periodontal disease development in clinically healthy persons

    I.N. Nikulina

    2009-12-01

    Full Text Available The research goal is to determine risk factors of periodontal disease development, psychophysiological personal types and their interrelations in clinically healthy persons. 47 first-year cadets of St.-Petersburg Military School of radio electronics have been examined. This group of respondents has been chosen by presence of such social stressor as change of place of living (97,9% cadets have arrived in St.-Petersburg from other cities and republics of the Russian Federation and strict disciplinary conditions. The research has revealed a low level of oral hygiene, cases of mild gingivitis in most respondents. The general mental state of group under study is characterized by raised level of personal anxiety and low indices of reactive anxiety. The examined group has demonstrated anxiety, tension, indecision and lowered stress stability. Clinically healthy persons are more liable to develop inflammatory and inflammatory-destructive periodontal diseases. It was possible to determine psychophysiological features correlated with physiological parameters of risk degree of periodontal diseases. It may have a great significance in defining of periodontal disease etiology and pathogenesis

  4. Perceptions of dry eye disease management in current clinical practice.

    Williamson, Jennifer F; Huynh, Kyle; Weaver, Mark A; Davis, Richard M

    2014-03-01

    To assess the perceptions of eye care providers regarding the clinical management of dry eye. Invitations to complete a 17-question online survey were mailed to 400 members of the North Carolina Ophthalmology and Optometry Associations including community optometrists, comprehensive ophthalmologists, and cornea specialists. The survey was completed by 100 eye care providers (25% response rate). Providers reported burning (46.5%) as the most frequent symptom described by patients, followed by foreign body sensation (30.3%) and tearing (17.2%). Most respondents (80.8%) listed artificial tears as the recommended first-line treatment, even though providers reported high failure rates for both artificial tears and cyclosporine A (Restasis). Rheumatoid arthritis, Sjögren syndrome, affective disorders such as anxiety and depression, history of photorefractive surgery, smoking, and thyroid disease were acknowledged as common comorbid conditions. The survey provided an informative snapshot into the preferences of eye care providers concerning the diagnosis and management of dry eye disease. Overall, burning was the most common symptom reported by patients. Providers relied more on patient history in guiding their clinical decisions than objective signs. The survey underscores the incongruence when comparing subjective symptoms with objective signs, thereby highlighting the urgent need for the development of reliable metrics to better quantify dry eye symptoms and also the development of a more sensitive and specific test that can be used as the gold standard to diagnose dry eye.

  5. AMERICAN ASSOCIATION OF CLINICAL ENDOCRINOLOGISTS AND AMERICAN COLLEGE OF ENDOCRINOLOGY DISEASE STATE CLINICAL REVIEW: DIAGNOSIS OF RECURRENCE IN CUSHING DISEASE.

    Fleseriu, Maria; Hamrahian, Amir H; Hoffman, Andrew R; Kelly, Daniel F; Katznelson, Laurence

    2016-12-01

    Recurrence of hypercortisolemia after initial treatment of Cushing disease (CD) is more common than previously thought, with a third of patients suffering a recurrence over their lifetime. Awareness of this high rate and delayed timeline (sometimes decades) of potential recurrence is critical and patients with CD should be monitored at regular intervals throughout their lives. In this manuscript, we review the complex evaluation needed for defining CD remission versus persistent disease after surgery, and focus on challenges in diagnosing early recurrent hypercortisolemia. Late night salivary cortisol appears to be an earlier predictor of recurrence when compared with urinary free cortisol (UFC) excretion. We also review the criteria suggested to define recurrence of hypercortisolemia in patients treated with medical therapy. Further research is needed to determine the optimal way to evaluate a patient with CD recurrence as well as the riskbenefit ratio of treatment in early, mild recurrent disease. ACTH = adrenocorticotropic hormone AI = adrenal insufficiency CD = Cushing disease CDDT = coupled dexamethasone desmopressin test CR = circadian rhythm CRH = corticotropin-releasing hormone GC = glucocorticoid GCR = global clinical response HPA = hypothalamic-pituitary-adrenal LDDST = low-dose dexamethasone suppression test LNSC = late-night salivary cortisol ODST = overnight dexamethasone suppression test TSS = trans-sphenoidal surgery.

  6. Nutritional Risk, Micronutrient Status and Clinical Outcomes: A Prospective Observational Study in an Infectious Disease Clinic

    Oguzhan Sıtkı Dizdar

    2016-02-01

    Full Text Available Malnutrition has been associated with increased morbidity and mortality. The objective of this study was to determine the nutritional status and micronutrient levels of hospitalized patients in an infectious disease clinic and investigate their association with adverse clinical outcomes. The nutritional status of the study participants was assessed using the Nutritional Risk Screening 2002 (NRS 2002 and micronutrient levels and routine biochemical parameters were tested within the first 24 h of the patient’s admission. The incidence of zinc, selenium, thiamine, vitamin B6, vitamin B12 deficiency were 66.7% (n = 40, 46.6% (n = 29, 39.7% (n = 27, 35.3% (n = 24, 14.1% (n = 9, respectively. Selenium levels were significantly higher in patients with urinary tract infections, but lower in soft tissue infections. Copper levels were significantly higher in patients with soft tissue infections. In the Cox regression models, lower albumin, higher serum lactate dehydrogenase levels and higher NRS-2002 scores were associated with increased death. Thiamine, selenium, zinc and vitamin B6 deficiencies but not chromium deficiencies are common in infectious disease clinics. New associations were found between micronutrient levels and infection type and their adverse clinical outcomes. Hypoalbuminemia and a high NRS-2002 score had the greatest accuracy in predicting death, systemic inflammatory response syndrome and sepsis on admission.

  7. [Clinical stages of patients with Alzheimer disease treated in specialist clinics in Spain. The EACE study].

    Alom Poveda, J; Baquero, M; González-Adalid Guerreiro, M

    2013-10-01

    The diagnostic paradigm of Alzheimer disease (AD) is changing; there is a trend toward diagnosing the disease in its early stages, even before the complete syndrome of dementia is apparent. The clinical stage at which AD is usually diagnosed in our area is unknown. Therefore, the purpose of this study is to describe the clinical stages of AD patients at time of diagnosis. Multicentre, observational and cross-sectional study. Patients with probable AD according to NINCDS-ARDRA criteria, attended in specialist clinics in Spain, were included in the study. We recorded the symptom onset to evaluation and symptom onset to diagnosis intervals and clinical status of AD (based on MMSE, NPI questionnaire, and CDR scale). Participants in this study included 437 specialists representing all of Spain's autonomous communities and a total of 1,707 patients, of whom 1,694 were included in the analysis. Mean MMSE score was 17.6±4.8 (95% CI:17.4-17.9). Moderate cognitive impairment (MMSE between 10 and 20) was detected in 64% of the patients, and severe cognitive impairment (MMSEde Neurología. Published by Elsevier Espana. All rights reserved.

  8. Specific clinical signs and symptoms are predictive of clinical course in sporadic Creutzfeldt-Jakob disease.

    Nakatani, E; Kanatani, Y; Kaneda, H; Nagai, Y; Teramukai, S; Nishimura, T; Zhou, B; Kojima, S; Kono, H; Fukushima, M; Kitamoto, T; Mizusawa, H

    2016-09-01

    Akinetic mutism is thought to be an appropriate therapeutic end-point in patients with sporadic Creutzfeldt-Jakob disease (sCJD). However, prognostic factors for akinetic mutism are unclear and clinical signs or symptoms that precede this condition have not been defined. The goal of this study was to identify prognostic factors for akinetic mutism and to clarify the order of clinical sign and symptom development prior to its onset. The cumulative incidence of akinetic mutism and other clinical signs and symptoms was estimated based on Japanese CJD surveillance data (455 cases) collected from 2003 to 2008. A proportional hazards model was used to identify prognostic factors for the time to onset of akinetic mutism and other clinical signs and symptoms. Periodic synchronous discharges on electroencephalography were present in the majority of cases (93.5%). The presence of psychiatric symptoms or cerebellar disturbance at sCJD diagnosis was associated with the development of akinetic mutism [hazard ratio (HR) 1.50, 95% confidence interval (CI) 1.14-1.99, and HR 2.15, 95% CI1.61-2.87, respectively]. The clinical course from cerebellar disturbance to myoclonus or akinetic mutism was classified into three types: (i) direct path, (ii) path via pyramidal or extrapyramidal dysfunction and (iii) path via psychiatric symptoms or visual disturbance. The presence of psychiatric symptoms or cerebellar disturbance increased the risk of akinetic mutism of sCJD cases with probable MM/MV subtypes. Also, there appear to be sequential associations in the development of certain clinical signs and symptoms of this disease. © 2016 The Authors. European Journal of Neurology published by John Wiley & Sons Ltd on behalf of European Academy of Neurology.

  9. Subjective cognitive decline: The first clinical manifestation of Alzheimer's disease?

    Adalberto Studart Neto

    Full Text Available ABSTRACT Background: Mild cognitive impairment is considered as the first clinical manifestation of Alzheimer's disease (AD, when the individual exhibits below performance on standardized neuropsychological tests. However, some subjects before having a lower performance on cognitive assessments already have a subjective memory complaint. Objective: A review about subjective cognitive decline, the association with AD biomarkers and risk of conversion to dementia. Methods: We performed a comprehensive non-systematic review on PubMed. The keywords used in the search were terms related to subjective cognitive decline. Results: Subjective cognitive decline is characterized by self-experience of deterioration in cognitive performance not detected objectively through formal neuropsychological testing. However, various terms and definitions have been used in the literature and the lack of a widely accepted concept hampers comparison of studies. Epidemiological data have shown that individuals with subjective cognitive decline are at increased risk of progression to AD dementia. In addition, there is evidence that this group has a higher prevalence of positive biomarkers for amyloidosis and neurodegeneration. However, Alzheimer's disease is not the only cause of subjective cognitive decline and various other conditions can be associated with subjective memory complaints, such as psychiatric disorders or normal aging. The features suggestive of a neurodegenerative disorder are: onset of decline within the last five years, age at onset above 60 years, associated concerns about decline and confirmation by an informant. Conclusion: These findings support the idea that subjective cognitive complaints may be an early clinical marker that precedes mild cognitive impairment due to Alzheimer's disease.

  10. Clinical characteristics of sleep disorders in patients with Parkinson's disease.

    Mao, Zhi-Juan; Liu, Chan-Chan; Ji, Su-Qiong; Yang, Qing-Mei; Ye, Hong-Xiang; Han, Hai-Yan; Xue, Zheng

    2017-02-01

    In order to investigate the sleep quality and influencing factors in patients with Parkinson's disease (PD), 201 PD patients were enrolled and underwent extensive clinical evaluations. Subjective sleep evaluation was assessed using the Pittsburgh Sleep Quality Index (PSQI), and the Epworth Sleepiness Scale (ESS). It was found that poor sleep quality (77.11%) and excessive daytime sleepiness (32.34%) were commonly seen in PD patients and positively correlated with disease severity. Then 70 out of the 201 PD patients and 70 age- and sex-matched controls underwent a polysomnographic recording. The parameters were compared between PD group and control group and the influencing factors of sleep in PD patients were analyzed. The results showed that sleep efficiency (SE) was significantly decreased (Psleep latency (SL) and the arousal index (AI) were increased (Psleep time (TST) were positively correlated with the Hoehn and Yahr (H&Y) stage. There was significant difference in the extent of hypopnea and hypoxemia between the PD group and the control group (Psleep quality and a high prevalence of sleep disorder, which may be correlated with the disease severity. Respiratory function and oxygen supply are also affected to a certain degree in PD patients.

  11. Urate predicts rate of clinical decline in Parkinson disease

    Ascherio, Alberto; LeWitt, Peter A.; Xu, Kui; Eberly, Shirley; Watts, Arthur; Matson, Wayne R.; Marras, Connie; Kieburtz, Karl; Rudolph, Alice; Bogdanov, Mikhail B.; Schwid, Steven R.; Tennis, Marsha; Tanner, Caroline M.; Beal, M. Flint; Lang, Anthony E.; Oakes, David; Fahn, Stanley; Shoulson, Ira; Schwarzschild, Michael A.

    2009-01-01

    Context The risk of Parkinson disease (PD) and its rate of progression may decline with increasing blood urate, a major antioxidant. Objective To determine whether serum and cerebrospinal fluid (CSF) concentrations of urate predict clinical progression in patients with PD. Design, Setting, and Participants 800 subjects with early PD enrolled in the DATATOP trial. Pre-treatment urate was measured in serum for 774 subjects and in CSF for 713. Main Outcome Measures Treatment-, age- and sex-adjusted hazard ratios (HRs) for clinical disability requiring levodopa therapy, the pre-specified primary endpoint. Results The HR of progressing to endpoint decreased with increasing serum urate (HR for 1 standard deviation increase = 0.82; 95% CI = 0.73 to 0.93). In analyses stratified by α-tocopherol treatment (2,000 IU/day), a decrease in the HR for the primary endpoint was seen only among subjects not treated with α-tocopherol (HR = 0.75; 95% CI = 0.62 to 0.89, versus those treated HR = 0.90; 95% CI = 0.75 to 1.08). Results were similar for the rate of change in the United Parkinson Disease Rating Scale (UPDRS). CSF urate was also inversely related to both the primary endpoint (HR for highest versus lowest quintile = 0.65; 95% CI: 0.54 to 0.96) and to the rate of change in UPDRS. As with serum urate, these associations were present only among subjects not treated with α-tocopherol. Conclusion Higher serum and CSF urate at baseline were associated with slower rates of clinical decline. The findings strengthen the link between urate and PD and the rationale for considering CNS urate elevation as a potential strategy to slow PD progression. PMID:19822770

  12. Seropositivity to non-vaccine incorporated genotypes induced by the bivalent and quadrivalent HPV vaccines: A systematic review and meta-analysis.

    Bissett, Sara L; Godi, Anna; Jit, Mark; Beddows, Simon

    2017-07-13

    Human papillomavirus vaccines have demonstrated remarkable efficacy against persistent infection and disease associated with vaccine-incorporated genotypes and a degree of efficacy against some genetically related, non-vaccine-incorporated genotypes. The vaccines differ in the extent of cross-protection against these non-vaccine genotypes. Data supporting the role for neutralizing antibodies as a correlate or surrogate of cross-protection are lacking, as is a robust assessment of the seroconversion rates against these non-vaccine genotypes. We performed a systematic review and meta-analysis of available data on vaccine-induced neutralizing antibody seropositivity to non-vaccine incorporated HPV genotypes. Of 304 articles screened, 9 were included in the analysis representing ca. 700 individuals. The pooled estimate for seropositivity against HPV31 for the bivalent vaccine (86%; 95%CI 78-91%) was higher than that for the quadrivalent vaccine (61%; 39-79%; p=0.011). The pooled estimate for seropositivity against HPV45 for the bivalent vaccine (50%; 37-64%) was also higher than that for the quadrivalent vaccine (16%; 6-36%; p=0.007). Seropositivity against HPV33, HPV52 and HPV58 were similar between the vaccines. Mean seropositivity rates across non-vaccine genotypes were positively associated with the corresponding vaccine efficacy data reported from vaccine trials. These data improve our understanding of vaccine-induced functional antibody specificity against non-vaccine incorporated genotypes and may help to parameterize vaccine-impact models and improve patient management in a post-vaccine setting. Crown Copyright © 2017. Published by Elsevier Ltd. All rights reserved.

  13. Genetic testing in congenital heart disease:A clinical approach

    Marie A Chaix; Gregor Andelfinger; Paul Khairy

    2016-01-01

    Congenital heart disease(CHD) is the most common type of birth defect. Traditionally, a polygenic model defined by the interaction of multiple genes and environmental factors was hypothesized to account for different forms of CHD. It is now understood that the contribution of genetics to CHD extends beyond a single unified paradigm. For example, monogenic models and chromosomal abnormalities have been associated with various syndromic and non-syndromic forms of CHD. In such instances, genetic investigation and testing may potentially play an important role in clinical care. A family tree with a detailed phenotypic description serves as the initial screening tool to identify potentially inherited defects and to guide further genetic investigation. The selection of a genetic test is contingent upon the particular diagnostic hypothesis generated by clinical examination. Genetic investigation in CHD may carry the potential to improve prognosis by yielding valuable information with regards to personalized medical care, confidence in the clinical diagnosis, and/or targeted patient followup. Moreover, genetic assessment may serve as a tool to predict recurrence risk, define the pattern of inheritance within a family, and evaluate the need for further family screening. In some circumstances, prenatal or preimplantation genetic screening could identify fetuses or embryos at high risk for CHD. Although genetics may appear to constitute a highly specialized sector of cardiology, basic knowledge regarding inheritance patterns, recurrence risks, and available screening and diagnostic tools, including their strengths and limitations, could assist the treating physician in providing sound counsel.

  14. Genetic testing in congenital heart disease: A clinical approach

    Chaix, Marie A; Andelfinger, Gregor; Khairy, Paul

    2016-01-01

    Congenital heart disease (CHD) is the most common type of birth defect. Traditionally, a polygenic model defined by the interaction of multiple genes and environmental factors was hypothesized to account for different forms of CHD. It is now understood that the contribution of genetics to CHD extends beyond a single unified paradigm. For example, monogenic models and chromosomal abnormalities have been associated with various syndromic and non-syndromic forms of CHD. In such instances, genetic investigation and testing may potentially play an important role in clinical care. A family tree with a detailed phenotypic description serves as the initial screening tool to identify potentially inherited defects and to guide further genetic investigation. The selection of a genetic test is contingent upon the particular diagnostic hypothesis generated by clinical examination. Genetic investigation in CHD may carry the potential to improve prognosis by yielding valuable information with regards to personalized medical care, confidence in the clinical diagnosis, and/or targeted patient follow-up. Moreover, genetic assessment may serve as a tool to predict recurrence risk, define the pattern of inheritance within a family, and evaluate the need for further family screening. In some circumstances, prenatal or preimplantation genetic screening could identify fetuses or embryos at high risk for CHD. Although genetics may appear to constitute a highly specialized sector of cardiology, basic knowledge regarding inheritance patterns, recurrence risks, and available screening and diagnostic tools, including their strengths and limitations, could assist the treating physician in providing sound counsel. PMID:26981213

  15. Seropositive abdominal and thoracic donor organs are largely underutilized.

    Taylor, R M; Pietroski, R E; Hagan, M; Eisenbrey, A B; Fontana, R J

    2010-12-01

    The aim of this study was to describe the epidemiology and utilization of anti-hepatitis B core protein(+) and anti-hepatitis C virus(+) organ donor referrals in a large organ procurement organization. Between 1995 and 2006, 3,134 deceased organ donor referrals were tested for anti-HBc and anti-HCV using commercial assays. The prevalence of anti-HCV(+) organ donor referrals significantly increased from 3.4% in 1994-1996 to 8.1% in 2003-2005 (P organ donor referrals remained unchanged at 3%-4% (P = .20). The 112 anti-HBc(+) (3.5%) and 173 anti-HCV(+) (5.5%) organ donor referrals were significantly older and more likely to be noncaucasian than seronegative organ donor referrals (P donor organs were significantly lower compared with seronegative organ donors (P donors over time (21% vs 46%; P = .026), whereas utilization of anti-HCV(+) liver donors remained unchanged over time (5% vs 18%; P = .303). In summary, the proportion of anti-HCV(+) organ donor referrals has significantly increased and the proportion of anti-HBc(+) organ donor referrals has remained stable. Both thoracic and abdominal organs from seropositive donors are largely underutilized. Copyright © 2010 Elsevier Inc. All rights reserved.

  16. Kawasaki disease in Sicily: clinical description and markers of disease severity

    Maggio, M.; Corsello, G.; Prinzi, E.; Cimaz, R.

    2016-01-01

    Background Kawasaki disease (KD) is an acute systemic vasculitis of small and middle size arteries; 15-25 % of untreated patients and 5 % of patients treated with intravenous immunoglobulin (IVIG) develop coronary artery lesions (CAL). Many studies tried to find the most effective treatment in the management of resistant KD and to select the risk factors for CAL. Our data are assessed on children from west Sicily, characterized by a genetic heterogeneity. Methods We studied the clinical data ...

  17. Male breast disease: clinical, mammographic, and ultrasonographic features

    Guenhan-Bilgen, Isil; Bozkaya, Halil; Uestuen, Esin Emin; Memis, Aysenur

    2002-01-01

    Purpose: To describe and quantitate the radiological (mammographic and ultrasonographic) characteristics of male breast disease and to report the clinical and pathological findings. Materials and methods: Two-hundred-thirty-six male patients with different male breast diseases, diagnosed at our institution between January 1990 and July 2001, were retrospectively evaluated. The history, physical examination, mammographic and ultrasonographic findings were analyzed. Results: The spectrum of the disease in 236 male patients were gynecomastia (n=206), primary breast carcinoma (n=14), fat necrosis (n=5), lipoma (n=3), subareolar abscess (n=2), epidermal inclusion cyst (n=1), sebaceous cyst (n=1), hematoma (n=1), myeloma (n=1), and metastatic carcinoma (n=2). The distribution of patterns of gynecomastia were; 34% (n=71) nodular, 35% (n=73) dendritic and 31% (n=62) diffuse glandular. Gynecomastia was unilateral in 55% (n=113) and bilateral in 45% (n=93) of the patients. Male breast cancer presented as a mass without microcalcifications in 86% (n=12) and with microcalcifications in 7% (n=1) of patients. The mass was obscured by gynecomastia, partially in two, totally in one patient. The location of the mass was retroareolar in 46% (n=6) and eccentric to the nipple in 54% (n=7) of patients. On ultrasonography (US), the contours were well-circumscribed in 20% (n=3) and irregular in 80% (n=12) of the masses. Conclusion: Male breast has a wide spectrum of diseases, some of which have characteristic radiological appearances that can be correlated with their pathologic diagnosis. In the evaluation of the male breast, mammography and US are essential and should be performed along with physical examination

  18. Male breast disease: clinical, mammographic, and ultrasonographic features

    Guenhan-Bilgen, Isil E-mail: isilbilgen@hotmail.com; Bozkaya, Halil; Uestuen, Esin Emin; Memis, Aysenur

    2002-09-01

    Purpose: To describe and quantitate the radiological (mammographic and ultrasonographic) characteristics of male breast disease and to report the clinical and pathological findings. Materials and methods: Two-hundred-thirty-six male patients with different male breast diseases, diagnosed at our institution between January 1990 and July 2001, were retrospectively evaluated. The history, physical examination, mammographic and ultrasonographic findings were analyzed. Results: The spectrum of the disease in 236 male patients were gynecomastia (n=206), primary breast carcinoma (n=14), fat necrosis (n=5), lipoma (n=3), subareolar abscess (n=2), epidermal inclusion cyst (n=1), sebaceous cyst (n=1), hematoma (n=1), myeloma (n=1), and metastatic carcinoma (n=2). The distribution of patterns of gynecomastia were; 34% (n=71) nodular, 35% (n=73) dendritic and 31% (n=62) diffuse glandular. Gynecomastia was unilateral in 55% (n=113) and bilateral in 45% (n=93) of the patients. Male breast cancer presented as a mass without microcalcifications in 86% (n=12) and with microcalcifications in 7% (n=1) of patients. The mass was obscured by gynecomastia, partially in two, totally in one patient. The location of the mass was retroareolar in 46% (n=6) and eccentric to the nipple in 54% (n=7) of patients. On ultrasonography (US), the contours were well-circumscribed in 20% (n=3) and irregular in 80% (n=12) of the masses. Conclusion: Male breast has a wide spectrum of diseases, some of which have characteristic radiological appearances that can be correlated with their pathologic diagnosis. In the evaluation of the male breast, mammography and US are essential and should be performed along with physical examination.

  19. Pattern of breast diseases: preliminary report of breast clinic

    Siddiqui, K.; Rasool, I.

    2001-01-01

    Objective: To find out the pattern of breast disease in this part of the county and create public awareness about breast diseases especially cancer. Design: Ac cross sectional and cohort study. Place and Duration of Study: The study was conducted at Department of Surgery, Jinnah Hospital /Allama Iqbal Medical College, Lahore from March 1999 to July 2000. Subjects and Methods: All the female patients reported were included in this study. They were diagnosed by history, physical examination and rel event investigations like ultrasonography, mammography, FNAS and biopsy. Appropriate medical and surgical management was carried out. The breast cancer was treated according to TNA staging system by multidisciplinary approach. Method of breast self examination (BSE) was taught with the help of charts and brochures. Results: The age ranged from 10 years to 75 years. Maximum number of patients (30%) was seen between 20-29 years of age while 15 (1%) cases did not suffer from any disease. Among 1485 patients the common conditions were non cyclical mastalgia in 362 (24.37%), fibroadenoma in 289 (19.46%), fibrocystic disease in 276(17.98%) breast abscess in 149 (10%) and breast cancer (6.19%). Other diseases were puberty mastitis 49(3.2%), galactocele 40(2.69%), accessory breast 45(3%) and nipple discharge 28(1.88%). Among the palpable lumps, breast caner accounted for 11.75%. The commonest age of presentation of breast cancer was 5th decade (31%) followed by 4th decade (26%). Majority of cancer patients (45%) presented in stage III. All the women with beast abscesses were lactating. Non cyclical mastalgia was commonly seen in 4th decade (30.66%) while 44.63% patients of fibroadenoma reported in the 2nd decade. Fibrocystic disease was reported between 3rd and 4th decade (62.17%). Conclusion: Commonest being conditions were non cyclical mastalgia followed by fibroadenoma while breast cancer contributed a significant percentage of palpable lumps. Due to effective public awareness

  20. Clinical evaluation of guidelines and two-test approach for lyme disease

    Blaauw, A. A.; van Loon, A. M.; Schellekens, J. F.; Bijlsma, J. W.

    1999-01-01

    The diagnosis of Lyme disease should be based on objective clinical signs and symptoms. In a clinical study, we have evaluated whether the recommended two-step approach for serodiagnosis of Lyme disease is useful in daily clinical practice and can influence clinical decision making. The signs and

  1. Household prevalence of seropositivity for Trypanosoma cruzi in three rural villages in northwest Argentina: environmental, demographic, and entomologic associations.

    Gürtler, R E; Chuit, R; Cécere, M C; Castañera, M B; Cohen, J E; Segura, E L

    1998-11-01

    Environmental, demographic, and entomologic variables were analyzed by logistic multiple regression analysis for their association with the likelihood of being seropositive for Trypanosoma cruzi in three highly infested rural villages of northwest Argentina. The prevalence of seropositivity for T. cruzi, as determined by the composite results of three serologic tests, was 34% among 338 persons in 1992. The strongest positive predictors of the adjusted odds of being infected were the household number of dogs, the density of T. cruzi-infected Triatoma infestans in bedroom areas, and each person's age. Dwellers from houses with roofs made completely or partly with a grass called simbol, or which used insecticides rudimentarily and nonsystematically, had a significantly lower odds of being seropositive for T. cruzi than residents from other types of dwellings. The adjusted odds of infection also increased with the number of T. cruzi-infected dogs or cats and the presence of chickens in bedroom areas. No significant effects on the adjusted odds of infection of a community-wide deltamethrin spraying carried out in one of the villages seven years before were detected. Socioeconomic indicators, such as domiciliary area, and numbers of corrals and livestock, were inversely related to being infected. Our study identified several manageable variables suitable for control actions, most of them not examined before in univariate or multivariate analyses. Environmental management based on low-cost housing with appropriate local materials and removal of domestic animals from domiciliary areas have a crucial role to play in the control of Chagas' disease in rural areas.

  2. Disease activity indices in coeliac disease: systematic review and recommendations for clinical trials.

    Hindryckx, Pieter; Levesque, Barrett G; Holvoet, Tom; Durand, Serina; Tang, Ceen-Ming; Parker, Claire; Khanna, Reena; Shackelton, Lisa M; D'Haens, Geert; Sandborn, William J; Feagan, Brian G; Lebwohl, Benjamin; Leffler, Daniel A; Jairath, Vipul

    2018-01-01

    Although several pharmacological agents have emerged as potential adjunctive therapies to a gluten-free diet for coeliac disease, there is currently no widely accepted measure of disease activity used in clinical trials. We conducted a systematic review of coeliac disease activity indices to evaluate their operating properties and potential as outcome measures in registration trials. MEDLINE, EMBASE and the Cochrane central library were searched from 1966 to 2015 for eligible studies in adult and/or paediatric patients with coeliac disease that included coeliac disease activity markers in their outcome measures. The operating characteristics of histological indices, patient-reported outcomes (PROs) and endoscopic indices were evaluated for content and construct validity, reliability, responsiveness and feasibility using guidelines proposed by the US Food and Drug Administration (FDA). Of 19 123 citations, 286 studies were eligible, including 24 randomised-controlled trials. Three of five PROs identified met most key evaluative criteria but only the Celiac Disease Symptom Diary (CDSD) and the Celiac Disease Patient-Reported Outcome (CeD PRO) have been approved by the FDA. All histological and endoscopic scores identified lacked content validity. Quantitative morphometric histological analysis had better reliability and responsiveness compared with qualitative scales. Endoscopic indices were infrequently used, and only one index demonstrated responsiveness to effective therapy. Current best evidence suggests that the CDSD and the CeD PRO are appropriate for use in the definition of primary end points in coeliac disease registration trials. Morphometric histology should be included as a key secondary or co-primary end point. Further work is needed to optimise end point configuration to inform efficient drug development. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/.

  3. A case-control study of risk factors for bovine brucellosis seropositivity in Peninsular Malaysia.

    Anka, Mukhtar Salihu; Hassan, Latiffah; Khairani-Bejo, Siti; Zainal, Mohamed Abidin; Mohamad, Ramlan Bin; Salleh, Annas; Adzhar, Azri

    2014-01-01

    Bovine brucellosis was first reported in Peninsular Malaysia in 1950. A subsequent survey conducted in the country revealed that the disease was widespread. Current knowledge on the potential risk factors for brucellosis occurrence on cattle farms in Malaysia is lacking. Therefore, we conducted a case-control study to identify the potential herd-level risk factors for bovine brucellosis occurrence in four states in the country, namely Kelantan, Pahang, Selangor and Negeri Sembilan. Thirty-five cases and 36 controls of herds were selected where data on farm management, biosecurity, medical history and public health were collected. Multivariable logistic regression identified that Brucella seropositive herds were more likely to; have some interaction with wildlife (OR 8.9, 95% CI = 1.59-50.05); originated from farms where multiple species such as buffalo/others (OR 41.8, 95% CI = 3.94-443.19) and goat/sheep (OR 8.9, 95%Cl = 1.10-71.83) were reared, practice extensive production system (OR 13.6, 95% CI 1.31-140.24) and have had episodes of abortion in the past (OR 51.8, 95% CI = 4.54-590.90) when compared to seronegative herds. Considering the lack of information on the epidemiology of bovine brucellosis in peninsular Malaysia and absence of information on preventing the inception or spread of the disease, this report could contribute to the on-going area-wise national brucellosis eradication program.

  4. Clinical implications of shared genetics and pathogenesis in autoimmune diseases

    Zhernakova, Alexandra; Withoff, Sebo; Wijmenga, Cisca

    2013-01-01

    Many endocrine diseases, including type 1 diabetes mellitus, Graves disease, Addison disease and Hashimoto disease, originate as an autoimmune reaction that affects disease-specific target organs. These autoimmune diseases are characterized by the development of specific autoantibodies and by the

  5. Impulse control disorders in Parkinson's disease: clinical characteristics and implications.

    Leeman, Robert F; Potenza, Marc N

    2011-04-01

    Impulse control disorders (ICDs), specifically those related to excessive gambling, eating, sex and shopping, have been observed in a subset of people with Parkinson's disease (PD). Although some initial case reports claimed that dopamine replacement therapies, particularly dopamine agonists, cause ICDs, more recent, larger and better controlled studies indicate a more complicated picture. While dopamine replacement therapy use is related to ICDs, other vulnerabilities, some related to PD and/or its treatment directly and others seemingly unrelated to PD, have also been associated with ICDs in PD. This suggests a complex etiology with multiple contributing factors. As ICDs occur in a sizable minority of PD patients and can be associated with significant distress and impairment, further investigation is needed to identify factors that can predict who may be more likely to develop ICDs. Clinical implications are discussed and topics for future research are offered.

  6. A Rare Clinical Presentation of Darier’s Disease

    Mybera Ferizi

    2013-01-01

    Full Text Available Darier’s disease, also known as keratosis follicularis or dyskeratosis follicularis, is a rare disorder of keratinization. It is an autosomal dominant genodermatosis with high penetrance and variable expressivity. Its manifestation appears as hyperkeratotic papules, primarily affecting seborrheic areas on the head, neck, and thorax and less frequently on the oral mucosa. When oral manifestations are present, the palatal and alveolar mucosae are primarily affected. They are usually asymptomatic and are discovered in routine dental examination. Histologically, the lesions are presented as suprabasal clefts in the epithelium with acantholytic and dyskeratotic cells represented by “corps ronds and grains”. This paper reports a case of a 53-year-old woman that was admitted to our clinic with more than 10-year history of keratotic papules, presented on the hands and feet, nose, ears, genitalia, and whitish lesions on palatal mucosae.

  7. Characteristics of pulmonary tuberculosis in HIV seropositive and seronegative patients in a Northeastern region of Brazil

    Liberato Isabella Ramos de Oliveira

    2004-01-01

    Full Text Available The aim of this study was to analyse the clinical, epidemiological and bacteriological features present in 60 pulmonary tuberculosis patients who were also infected with human immunodeficiency virus (HIV and to compare these with 120 TB patients who were not infected with HIV. The patients with pulmonary tuberculosis and HIV coinfection were mostly male (p = 0.001, showed a higher frequency of weight loss >10 kilos (p <0.001, had a higher rate of non-reaction result to the tuberculin skin test (p <0.001, a higher frequency of negative sputum smear examination for acid-fast bacilli (p = 0.001 and negative sputum culture for Mycobacterium tuberculosis (p = 0.001. Treatment failure was more common in those who were HIV positive (p <0.000. No higher frequency of resistance to antituberculosis drugs was found to be associated with TB/HIV coinfection (p = 0.407. Association between extrapulmonary and pulmonary tuberculosis was more frequent in those seropositive to HIV than those without HIV virus, 30% and 1.6% respectively. These findings showed a predominance of atypical clinical laboratory features in co-infected patients, and suggest that health care personnel should consider the possibility this diagnosis.

  8. Possible interaction between myxomatosis and calicivirosis related to rabbit haemorrhagic disease affecting the European rabbit.

    Marchandeau, S; Bertagnoli, S; Peralta, B; Boucraut-Baralon, C; Letty, J; Reitz, F

    2004-11-06

    Serological data on myxoma virus, rabbit haemorrhagic disease (RHD) virus and RHD-like viruses in juvenile rabbits (Oryctolagus cuniculus) trapped in 1995, 1996 and 1997 in two areas of France were analysed. For each disease, the effects of bodyweight, year, month and seropositivity for the other disease were modelled by using logistic regressions. In one area, a model including RHD seropositivity was selected to explain the myxoma virus seropositivity. Models including myxoma virus seropositivity were selected to explain the RHD seropositivity in both areas, and the odds of a rabbit being seropositive to both viruses were 5.1 and 8.4 times higher than the odds of a rabbit being seronegative to myxoma virus and seropositive to RHD. The year and bodyweight had significant effects for myxomatosis in one area and for RHD in both areas.

  9. Dopaminergic and clinical correlates of pathological gambling in Parkinson's disease

    Callesen, Mette Buhl; Hansen, K V; Gjedde, A

    2013-01-01

    Dopaminergic medication for motor symptoms in Parkinson's disease (PD) recently has been linked with impulse control disorders, including pathological gambling (PG), which affects up to 8% of patients. PG often is considered a behavioral addiction associated with disinhibition, risky decision-mak...... decision-making. Overall, the findings are consistent with the hypothesis of medication-related PG in PD and underscore the importance of taking clinical variables, such as age and personality, into account when patients with PD are medicated, to reduce the risk of PG.......Dopaminergic medication for motor symptoms in Parkinson's disease (PD) recently has been linked with impulse control disorders, including pathological gambling (PG), which affects up to 8% of patients. PG often is considered a behavioral addiction associated with disinhibition, risky decision-making......, and altered striatal dopaminergic neurotransmission. Using [(11)C]raclopride with positron emission tomography, we assessed dopaminergic neurotransmission during Iowa Gambling Task performance. Here we present data from a single patient with PD and concomitant PG. We noted a marked decrease in [(11)C...

  10. Potential and clinical utility of stem cells in cardiovascular disease

    Korff Krause

    2010-03-01

    Full Text Available Korff Krause, Carsten Schneider, Kai Jaquet, Karl-Heinz KuckHanseatic Heart Center Hamburg, Department of Cardiology, Asklepios Hospital St. Georg, Hamburg, GermanyAbstract: The recent identification of bone marrow-derived adult stem cells and other types of stem cells that could improve heart function after transplantation have raised high expectations. The basic mechanisms have been studied mostly in murine models. However, these experiments revealed controversial results on transdifferentiation vs transfusion of adult stem cells vs paracrine effects of these cells, which is still being debated. Moreover, the reproducibility of these results in precisely translated large animal models is still less well investigated. Despite these weaknesses results of several clinical trials including several hundreds of patients with ischemic heart disease have been published. However, there are no solid data showing that any of these approaches can regenerate human myocardium. Even the effectiveness of cell therapy in these approaches is doubtful. In future we need in this important field of regenerative medicine: i more experimental data in large animals that are closer to the anatomy and physiology of humans, including data on dose effects, comparison of different cell types and different delivery routes; ii a better understanding of the molecular mechanisms involved in the fate of transplanted cells; iii more intensive research on genuine regenerative medicine, applying genetic regulation and cell engineering.Keywords: stem cells, cardiovascular disease

  11. Clinical relevance of sarcopenia in chronic kidney disease

    Moorthi, Ranjani N.; Avin, Keith G.

    2018-01-01

    Purpose of review In this article, we review sarcopenia in chronic kidney disease (CKD). We aim to present how definitions of sarcopenia from the general population may pertain to those with CKD, its assessment by clinicians and emerging therapies for sarcopenia in CKD. For this review, we limit our description and recommendations to patients with CKD who are not on dialysis. Recent findings Poorer parameters of lean mass, strength and physical function are associated with worsening patient-centered outcomes such as limiting mobility, falls and mortality in CKD; however, the magnitude of these associations are different in those with and without CKD. Sarcopenia in CKD is a balance between skeletal muscle regeneration and catabolism, which are both altered in the uremic environment. Multiple pathways are involved in these derangements, which are briefly reviewed. Differences between commonly used terms cachexia, frailty, protein-energy wasting, dynapenia and sarcopenia are described. Therapeutic options in predialysis CKD are not well studied; therefore, we review exercise options and emerging pharmacological therapies. Summary Sarcopenia, now with its own International Classification of Diseases, 10th Revision (ICD-10) code, is of importance clinically and should be accounted for in research studies in patients with CKD. Multiple therapies for sarcopenia are in development and will hopefully be available for our patients in the future. PMID:28198733

  12. Graves' disease: cost-effectiveness of clinical and radioiodine treatments

    Cruz junior, Antonio F.; Takahashi, Miriam H.; Albino, Claudio C.

    2005-01-01

    Full text: In this study, we set out to evaluate the costs and effectiveness of the two most used therapies in Graves' disease: antithyroid drugs (ATD) and radioiodine (RAI). Twenty-tree patients, 7 men and 16 women, with a mean age of 35.4 years, treated with ATD and 35 patients, 5 men and 30 women, mean age of 39.4 years, treated with RAI were studied. After 2 years receiving ATD, 21 patients achieved euthyroidism and 2 remained hyperthyroid. In the RAI group, 21 patients presented hypothyroidism and 13 became euthyroid. To calculate the costs of each therapy, we analysed the number of visits during this period, the laboratory data and the drugs needed, such as tiamazol and/or thyroxine. The group treated only with ATD needed a higher number of visits and laboratory measurements, with the mean total cost of U$ 791.65, while the RAI group spent a mean amount of U$ 366.44. Therefore, the costs of the RAI treatment were 53,7 % lower than clinical therapy with ATD. Conclusion: The present study demonstrates that RAI treatment has a lower cost than ATD, being very effective in controlling the hyperthyroidism of Graves' disease. (author)

  13. Clinical Applications of Mesenchymal Stem Cells in Chronic Diseases

    Andrea Farini

    2014-01-01

    Full Text Available Extraordinary progress in understanding several key features of stem cells has been made in the last ten years, including definition of the niche, and identification of signals regulating mobilization and homing as well as partial understanding of the mechanisms controlling self-renewal, commitment, and differentiation. This progress produced invaluable tools for the development of rational cell therapy protocols that have yielded positive results in preclinical models of genetic and acquired diseases and, in several cases, have entered clinical experimentation with positive outcome. Adult mesenchymal stem cells (MSCs are nonhematopoietic cells with multilineage potential to differentiate into various tissues of mesodermal origin. They can be isolated from bone marrow and other tissues and have the capacity to extensively proliferate in vitro. Moreover, MSCs have also been shown to produce anti-inflammatory molecules which can modulate humoral and cellular immune responses. Considering their regenerative potential and immunoregulatory effect, MSC therapy is a promising tool in the treatment of degenerative, inflammatory, and autoimmune diseases. It is obvious that much work remains to be done to increase our knowledge of the mechanisms regulating development, homeostasis, and tissue repair and thus to provide new tools to implement the efficacy of cell therapy trials.

  14. The Clinical and Immunologic Features of Patients With Combined Anti-GBM Disease and Castleman Disease.

    Gu, Qiu-Hua; Jia, Xiao-Yu; Hu, Shui-Yi; Wang, Su-Xia; Zou, Wan-Zhong; Cui, Zhao; Zhao, Ming-Hui

    2018-06-01

    Patients with both anti-glomerular basement membrane (anti-GBM) disease and Castleman disease have been rarely reported. In this study, we report 3 patients with this combination. They had immunologic features similar to patients with classic anti-GBM disease. Sera from the 3 patients recognized the noncollagenous (NC) domain of the α3 chain of type IV collagen (α3(IV)NC1) and its 2 major epitopes, EA and EB. All 4 immunogloblin G (IgG) subclasses against α3(IV)NC1 were detectable, with predominance of IgG1. In one patient with lymph node biopsy specimens available, sporadic plasma cells producing α3(IV)NC1-IgG were found, suggesting a causal relationship between the 2 diseases. One patient, who achieved remission with antibody clearance and normalization of serum creatinine and interleukin 6 concentrations after plasma exchange and 3 cycles of chemotherapy, experienced recurrence of anti-GBM antibodies and an increase in interleukin 6 concentration after chemotherapy discontinuation because of adverse effects, but both returned to normal after another cycle of chemotherapy. This clinical course and the pathologic findings support the hypothesis that the Castleman disease-associated tumor cells are the source of the anti-GBM autoantibodies. Copyright © 2018 National Kidney Foundation, Inc. Published by Elsevier Inc. All rights reserved.

  15. Clinical Characteristics and Associated Systemic Diseases in Patients With Esophageal "Absent Contractility"-A Clinical Algorithm.

    Laique, Sobia; Singh, Tavankit; Dornblaser, David; Gadre, Abhishek; Rangan, Vikram; Fass, Ronnie; Kirby, Donald; Chatterjee, Soumya; Gabbard, Scott

    2018-01-19

    This study was carried out to assess the clinical characteristics and associated systemic diseases seen in patients diagnosed with absent contractility as per the Chicago Classification version 3.0, allowing us to propose a diagnostic algorithm for their etiologic testing. The Chicago Classification version 3.0 has redefined major and minor esophageal motility disorders using high-resolution esophageal manometry. There is a dearth of publications based on research on absent contractility, which historically has been associated with myopathic processes such as systemic sclerosis (SSc). We conducted a retrospective, multicenter study. Data of patients diagnosed with absent contractility were pooled from Cleveland Clinic, Cleveland, OH (January 2006 to July 2016) and Metrohealth Medical Center, Cleveland, OH (July 2014 to July 2016) and included: age, gender, associated medical conditions, surgical history, medications, and specific antibody testing. A total of 207 patients, including 57 male individuals and 150 female individuals, with mean age of 56.1 and 60.0 years, respectively, were included. Disease distribution was as follows: SSc (diffuse or limited cutaneous) 132, overlap syndromes 7, systemic lupus erythematosus17, Sjögren syndrome 4, polymyositis 3, and dermatomyositis 3. Various other etiologies including gastroesophageal reflux disease, postradiation esophagitis, neuromuscular disorders, and surgical complications were seen in the remaining cohort. Most practitioners use the term "absent contractility" interchangeably with "scleroderma esophagus"; however, only 63% of patients with absent contractility had SSc. Overall, 20% had another systemic autoimmune rheumatologic disease and 16% had a nonrheumatologic etiology for absent contractility. Therefore, alternate diagnosis must be sought in these patients. We propose an algorithm for their etiologic evaluation.

  16. Clinical Holistic Medicine: The Patient with Multiple Diseases

    Søren Ventegodt

    2005-01-01

    Full Text Available In clinical practice, patients can present with many different diseases, often both somatic and mental. Holistic medicine will try to see the diseases as a whole, as symptoms of a more fundamental imbalance in the state of being. The holistic physician must help the patient to recover existence and a good relationship with self. According to the life mission theory, theory of character, and holistic process theory of healing, recovering the purpose of life (the life mission is essential for the patient to regain life, love, and trust in order to find happiness and realize the true purpose of life. We illustrate the power of the holistic medical approach with a case study of an invalidated female artist, aged 42 years, who suffered from multiple severe health problems, many of which had been chronic for years. She had a combination of neurological disturbances (tinnitus, migraine, minor hallucinations, immunological disturbances (recurrent herpes simplex, phlegm in the throat, fungal infection in the crotch, hormonal disturbances (14 days of menstruation in each cycle, muscle disturbances (neck tensions, mental disturbances (tendency to cry, inferiority feeling, mild depression, desolation, anxiety, abdominal complaints, hemorrhoids, and more. The treatment was a combined strategy of improving the general quality of life, recovering her human character and purpose of life (“renewing the patients life energy”, “balancing her global information system”, and processing the local blockages, thus healing most of her many different diseases in a treatment using 30 h of intense holistic therapy over a period of 18 months.

  17. Clinical utility of carotid and transcranial ultrasound in cerebrovascular diseases

    Figueiredo L

    2014-08-01

    Full Text Available Lívia Figueiredo, Viviane F Zétola, Marcos C Lange Neurology Division, Hospital de Clínicas, Universidade Federal do Paraná, Curitiba, Brazil Abstract: Carotid and transcranial (CTU ultrasound is a useful tool in a number of clinical settings, particularly in cerebrovascular diseases. CTU is the only method that provides real-time determination of velocity and the spectral waveform of blood flow in the extracranial and basal intracranial arteries, and is effective in the detection of stenosis and occlusion. When transcranial ultrasound is considered, CTU is the only method that allows visualization of microembolic signals in the intracranial arteries. CTU makes a rapid differential diagnosis possible, improving therapeutic decision-making in acute stroke and determining the risk of recurrence and prognosis based on its findings. It is also the standard of care in children with sickle cell disease, when selecting patients for chronic blood transfusion, and for reducing the risk of ischemic stroke in these patients. CTU has some advantages, ie, relative simplicity in terms of interpretation and performance, and affordability, noninvasiveness, and portability. The main concern with ultrasound is that it is an operator-dependent tool and requires a high level of expertise and knowledge of three-dimensional cerebrovascular anatomy for correct interpretation of sonograms. The most significant limitation of intracranial evaluation by transcranial ultrasound is the absence of a suitable bone window in approximately 10% of patients. This paper gives an overview of the current utility and importance of CTU in the prevention and evaluation of ischemic cerebrovascular disease. Keywords: transcranial Doppler ultrasonography, Doppler ultrasonography duplex, cerebrovascular disorders, stroke

  18. Clinical aspects of impulsive compulsive behaviours in Parkinson's disease.

    Djamshidian, Atbin; Averbeck, Bruno B; Lees, Andrew J; O'Sullivan, Sean S

    2011-11-15

    Impulsive-compulsive behaviours (ICBs) are an increasingly well-recognised adverse-effect of dopaminergic medications used to treat Parkinson's disease. ICBs include pathological gambling, compulsive sexual behaviour, compulsive buying, and binge eating, together with punding and the addiction-like compulsive use of dopamine replacement therapy, or dopamine dysregulation syndrome. The prevalence of ICBs was approximately 14% in a large study undertaken in specialist movement disorder clinics. Dopamine dysregulation syndrome is more associated with compulsive l-dopa use, whereas other ICBs are more linked with oral dopamine agonist use. Other mechanisms implicated in the development and perpetuation of ICBs in PD include aberrant learning from reward-related situations, including decreased learning from negative feedback, increased measures of impulsivity or sensation seeking, and strong preference for immediate over future rewards. Treatment options for impulsive-compulsive behaviours include pharmacological, surgical and psychological interventions. The early recognition and prevention of ICBs, coupled with awareness of clinical risk factors for the development of these behaviours is of paramount importance, given the lack of specific treatments for these sometimes debilitating behaviours. Copyright © 2011 Elsevier B.V. All rights reserved.

  19. Controlled clinical trial of cannabidiol in Huntington's disease.

    Consroe, P; Laguna, J; Allender, J; Snider, S; Stern, L; Sandyk, R; Kennedy, K; Schram, K

    1991-11-01

    Based on encouraging preliminary findings, cannabidiol (CBD), a major nonpsychotropic constituent of Cannabis, was evaluated for symptomatic efficacy and safety in 15 neuroleptic-free patients with Huntington's Disease (HD). The effects of oral CBD (10 mg/kg/day for 6 weeks) and placebo (sesame oil for 6 weeks) were ascertained weekly under a double-blind, randomized cross-over design. A comparison of the effects of CBD and placebo on chorea severity and other therapeutic outcome variables, and on a Cannabis side effect inventory, clinical lab tests and other safety outcome variables, indicated no significant (p greater than 0.05) or clinically important differences. Correspondingly, plasma levels of CBD were assayed by GC/MS, and the weekly levels (mean range of 5.9 to 11.2 ng/ml) did not differ significantly over the 6 weeks of CBD administration. In summary, CBD, at an average daily dose of about 700 mg/day for 6 weeks, was neither symptomatically effective nor toxic, relative to placebo, in neuroleptic-free patients with HD.

  20. Technical and clinical view on ambulatory assessment in Parkinson's disease.

    Hobert, M A; Maetzler, W; Aminian, K; Chiari, L

    2014-09-01

    With the progress of technologies of recent years, methods have become available that use wearable sensors and ambulatory systems to measure aspects of--particular axial--motor function. As Parkinson's disease (PD) can be considered a model disorder for motor impairment, a significant number of studies have already been performed with these patients using such techniques. In general, motion sensors such as accelerometers and gyroscopes are used, in combination with lightweight electronics that do not interfere with normal human motion. A fundamental advantage in comparison with usual clinical assessment is that these sensors allow a more quantitative, objective, and reliable evaluation of symptoms; they have also significant advantages compared to in-lab technologies (e.g., optoelectronic motion capture) as they allow long-term monitoring under real-life conditions. In addition, based on recent findings particularly from studies using functional imaging, we learned that non-motor symptoms, specifically cognitive aspects, may be at least indirectly assessable. It is hypothesized that ambulatory quantitative assessment strategies will allow users, clinicians, and scientists in the future to gain more quantitative, unobtrusive, and everyday relevant data out of their clinical evaluation and can also be designed as pervasive (everywhere) and intensive (anytime) tools for ambulatory assessment and even rehabilitation of motor and (partly) non-motor symptoms in PD. © 2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  1. Idiopathic granulomatous mastitis: a heterogeneous disease with variable clinical presentation.

    Baslaim, Muna M; Khayat, Hind A; Al-Amoudi, Shefaa A

    2007-08-01

    Idiopathic granulomatous mastitis (IGM) is a rare benign inflammatory breast disease that presents with variable local manifestations. We describe here the different management protocols based on the clinical presentation of these patients. A retrospective review of 20 histopathologic confirmed cases of IGM seen over a period of 10 years was performed. The median age was 34 years (age range: 21-45 years). All were married, parous with history of breast feeding. Ill-defined mass mimicking carcinoma was the commonest presentation (70%); however, with the presence of signs of inflammation like pain (55%), redness (40%), and peau d'orange (40%), an inflammatory process appeared more likely. Axillary lymph node enlargement was infrequently seen (40%). Radiologic findings (mammography and ultrasound) were nonspecific. Histopathology showed the characteristic lobular distribution of granulomatous inflammation in all cases. Surgically, 7 patients had abscess drainage with open biopsy, and 7 patients had lumpectomy. Six patients with diffuse breast involvement were diagnosed by core needle biopsy only. Microbial cultures showed no growth. Antibiotics were given empirically when signs of inflammation where present. Two patients needed further abscess drainage followed by persistent sinus excision 3-6 weeks later. The median follow-up was 24 months (range: 15-42 months). Seventeen patients (85%) were recurrence-free, and 3 patients (15%) were lost to follow-up. Management of IGM cases needs to be tailored according to the clinical presentation. Precise radiologic and pathologic data interpretation by a multidisciplinary breast team will facilitate diagnosis and minimize unnecessary intervention.

  2. Understanding complex clinical reasoning in infectious diseases for improving clinical decision support design.

    Islam, Roosan; Weir, Charlene R; Jones, Makoto; Del Fiol, Guilherme; Samore, Matthew H

    2015-11-30

    Clinical experts' cognitive mechanisms for managing complexity have implications for the design of future innovative healthcare systems. The purpose of the study is to examine the constituents of decision complexity and explore the cognitive strategies clinicians use to control and adapt to their information environment. We used Cognitive Task Analysis (CTA) methods to interview 10 Infectious Disease (ID) experts at the University of Utah and Salt Lake City Veterans Administration Medical Center. Participants were asked to recall a complex, critical and vivid antibiotic-prescribing incident using the Critical Decision Method (CDM), a type of Cognitive Task Analysis (CTA). Using the four iterations of the Critical Decision Method, questions were posed to fully explore the incident, focusing in depth on the clinical components underlying the complexity. Probes were included to assess cognitive and decision strategies used by participants. The following three themes emerged as the constituents of decision complexity experienced by the Infectious Diseases experts: 1) the overall clinical picture does not match the pattern, 2) a lack of comprehension of the situation and 3) dealing with social and emotional pressures such as fear and anxiety. All these factors contribute to decision complexity. These factors almost always occurred together, creating unexpected events and uncertainty in clinical reasoning. Five themes emerged in the analyses of how experts deal with the complexity. Expert clinicians frequently used 1) watchful waiting instead of over- prescribing antibiotics, engaged in 2) theory of mind to project and simulate other practitioners' perspectives, reduced very complex cases into simple 3) heuristics, employed 4) anticipatory thinking to plan and re-plan events and consulted with peers to share knowledge, solicit opinions and 5) seek help on patient cases. The cognitive strategies to deal with decision complexity found in this study have important

  3. Rationale and clinical data supporting nutritional intervention in Alzheimer's disease.

    Engelborghs, S; Gilles, C; Ivanoiu, A; Vandewoude, M

    2014-01-01

    Adequate nutrition plays an important role in the maintenance of cognitive function, particularly during aging. Malnutrition is amongst the risk factors for developing mild cognitive impairment (MCI) and Alzheimer's disease (AD). Epidemiological studies have associated deficiencies in some nutrients with a higher risk of cognitive dysfunction and/or AD. Cognitive decline in AD is correlated with synaptic loss and many of the components required to maintain optimal synaptic function are derived from dietary sources. As synapses are part of the neuronal membrane and are continuously being remodelled, the availability of sufficient levels of nutritional precursors (mainly uridine monophosphate, choline and omega-3 fatty acids) to make the phospholipids required to build neuronal membranes may have beneficial effects on synaptic degeneration in AD. In addition, B-vitamins, phospholipids and other micronutrients act as cofactors to enhance the supply of precursors required to make neuronal membranes and synapses. Despite this, no randomized controlled trial has hitherto provided evidence that any single nutrient has a beneficial effect on cognition or lowers the risk for AD. However, a multi-target approach using combinations of (micro)nutrients might have beneficial effects on cognitive function in neurodegenerative brain disorders like AD leading to synaptic degeneration. Here we review the clinical evidence for supplementation, based on a multi-target approach with a focus on key nutrients with a proposed role in synaptic dysfunction. Based on preclinical evidence, a nutrient mixture, Souvenaid(®) (Nutricia N.V., Zoetermeer, The Netherlands) was developed. Clinical trials with Souvenaid(®) have shown improved memory performance in patients with mild AD. Further clinical trials to evaluate the effects of nutritional intervention in MCI and early dementia due to AD are on-going.

  4. A systematic literature review of evidence-based clinical practice for rare diseases

    Rath, Ana; Salamon, Valérie; Peixoto, Sandra

    2017-01-01

    diseases comprise the difficulty to recruit participants because of rarity, scattering of patients, limited knowledge on natural history of diseases, difficulties to achieve accurate diagnosis and identify patients in health information systems, and difficulties choosing clinically relevant outcomes....... CONCLUSIONS: Evidence-based clinical practice for rare diseases should start by collecting clinical data in databases and registries; defining measurable patient-centred outcomes; and selecting appropriate study designs adapted to small study populations. Rare diseases constitute one of the most paradigmatic...

  5. Glycogen storage disease type I: clinical and laboratory profile

    Berenice L. Santos

    2014-11-01

    Full Text Available Objectives: To characterize the clinical, laboratory, and anthropometric profile of a sample of Brazilian patients with glycogen storage disease type I managed at an outpatient referral clinic for inborn errors of metabolism. Methods: This was a cross-sectional outpatient study based on a convenience sampling strategy. Data on diagnosis, management, anthropometric parameters, and follow-up were assessed. Results: Twenty-one patients were included (median age 10 years, range 1–25 years, all using uncooked cornstarch therapy. Median age at diagnosis was 7 months (range, 1–132 months, and 19 patients underwent liver biopsy for diagnostic confirmation. Overweight, short stature, hepatomegaly, and liver nodules were present in 16 of 21, four of 21, nine of 14, and three of 14 patients, respectively. A correlation was found between height-for-age and BMI-for-age Z-scores (r = 0.561; p = 0.008. Conclusions: Diagnosis of glycogen storage disease type I is delayed in Brazil. Most patients undergo liver biopsy for diagnostic confirmation, even though the combination of a characteristic clinical presentation and molecular methods can provide a definitive diagnosis in a less invasive manner. Obesity is a side effect of cornstarch therapy, and appears to be associated with growth in these patients. Resumo: Objetivos: Caracterizar o perfil clínico, laboratorial e antropométrico de uma amostra de pacientes brasileiros com doença de depósito de glicogênio tipo I tratados em um ambulatório de referência para erros inatos do metabolismo. Métodos: Este foi um estudo ambulatorial transversal com base em uma estratégia de amostragem de conveniência. Foram avaliados os dados com relação ao diagnóstico, tratamento, parâmetros antropométricos e acompanhamento. Resultados: Foram incluídos 21 pacientes (idade média de 10 anos, faixa 1-25 anos de idade, e todos se encontravam em terapia de amido de milho cru. A idade média na época do diagn

  6. Minimal residual disease in breast cancer. Clinical significance

    Kvalheim, G.

    2004-01-01

    Previously we have reported our results on minimal residual disease in breast cancer. Briefly, B M-aspirates were collected from 817 patients at primary surgery. Tumor cells in B M were detected by immunocytochemistry using anticytokeratin-antibodies (A E1/ A E3). Analyses of the primary tumor included histological grading, vascular invasion and immunohistochemical detection of cerbB2, cathepsin D, p53 and ER/PgRexpression. These analyses were compared to clinical outcome. Median follow-up was 49 months. ITC were detected in 13.2% of the patients. The detection rate rose with increasing tumor size (p=0.011) and lymph node involvement (p<0.001). Systemic relapse and death from breast cancer occurred in 31.7% and 26.9% of the B M-positive versus 13.7% and 10.9% of B M-negative patients, respectively (p<0.001). Analyzing node-positive and node-negative patients separately, ITC-positivity was associated with poor prognosis in the node-positive group and in node-negative patients not receiving adjuvant therapy (T1N0). In multivariate analysis, ITC in B M was an independent prognostic factor together with N-, T-, ER/Pg R-status, histological grade and vascular invasion. Combination of several independent prognostic factors can classify subgroups of patients into excellent and high-risk prognosis groups. Like other groups we have investigated the clinical role of monitoring minimal residual disease before and after adjuvant therapy in breast cancer. One hundred and eighteen high risk stage II breast cancer patients entering the Scandinavian Study Group multicenter trial were randomized to 9 cycles of dose escalated and tailored FEC (5-flurouracil, epirubicin, cyclophosphamide) or standard FEC followed by high dose chemotherapy. B M samples at diagnosis and 6 months after completion of chemotherapy were assessed for the presence of I CT. Median observation time for patients was 68 months. ITC positivity in bone marrow was evaluated as a prognostic and predictive marker and

  7. Poor Positive Predictive Value of Lyme Disease Serologic Testing in an Area of Low Disease Incidence.

    Lantos, Paul M; Branda, John A; Boggan, Joel C; Chudgar, Saumil M; Wilson, Elizabeth A; Ruffin, Felicia; Fowler, Vance; Auwaerter, Paul G; Nigrovic, Lise E

    2015-11-01

    Lyme disease is diagnosed by 2-tiered serologic testing in patients with a compatible clinical illness, but the significance of positive test results in low-prevalence regions has not been investigated. We reviewed the medical records of patients who tested positive for Lyme disease with standardized 2-tiered serologic testing between 2005 and 2010 at a single hospital system in a region with little endemic Lyme disease. Based on clinical findings, we calculated the positive predictive value of Lyme disease serology. Next, we reviewed the outcome of serologic testing in patients with select clinical syndromes compatible with disseminated Lyme disease (arthritis, cranial neuropathy, or meningitis). During the 6-year study period 4723 patients were tested for Lyme disease, but only 76 (1.6%) had positive results by established laboratory criteria. Among 70 seropositive patients whose medical records were available for review, 12 (17%; 95% confidence interval, 9%-28%) were found to have Lyme disease (6 with documented travel to endemic regions). During the same time period, 297 patients with a clinical illness compatible with disseminated Lyme disease underwent 2-tiered serologic testing. Six of them (2%; 95% confidence interval, 0.7%-4.3%) were seropositive, 3 with documented travel and 1 who had an alternative diagnosis that explained the clinical findings. In this low-prevalence cohort, fewer than 20% of positive Lyme disease tests are obtained from patients with clinically likely Lyme disease. Positive Lyme disease test results may have little diagnostic value in this setting. © The Author 2015. Published by Oxford University Press on behalf of the Infectious Diseases Society of America. All rights reserved. For Permissions, please e-mail: journals.permissions@oup.com.

  8. Analysis of metabolic changes of brain in HIV-1 seropositive patients with proton magnetic resonance spectroscopy

    Koltowska, A.; Hendrich, B.; Sokolska, V.; Mis, M.; Lubkowska, K.; Szewczyk, P.; Sasiadek, M.; Furdal, M.; Knysz, B.; Gasiorowski, J.; Gladysz, A.

    2010-01-01

    Background: Asymptomatic central nervous system involvement may occur in the early stages of the HIV infection. The aim of the study was to evaluate early brain metabolic changes by means of proton MR spectroscopy (H1MRS) in the HIV-1 seropositive patients without neurological deficits or significant abnormalities in the plain MR study. Material/Methods: The H1MRS examinations were performed with the use of a MR GE Signa 1,5 T system. There were 39 subjects examined, aged 21 to 57 years (mean age 35 years) were examined, including 25 patients infected with HIV-1 and 14 healthy volunteers who constituted a control group. The examinations were performed using the Single Voxel Spectroscopy technique with the PRESS sequence, with following parameters: TR=1500 ms, TE=35 ms, number of acquisitions =128, time of acquisition =3 min. 43 sec. Voxels of 8 cm 3 (20 x 20 x 20 mm) in size were located in the following 5 regions: posterior cingulate gyrus, grey matter of the frontal area, left basal ganglia, white matter of the left parietal area and white matter of the frontal area. The NAA/Cr, Cho/Cr, mI/Cr ratios in the defined regions of interest were statistically analyzed. Results: There was a statistically significant decrease (p < 0.05) in the NAA/Cr ratios in the posterior cingulate area and white matter of the left parietal area in HIV-1 seropositive patients, as compared to the control group. Other metabolite ratios in all the above mentioned locations showed no statistically significant differences, as was also the case for NAA/Cr ratios in grey matter of the frontal area, left basal ganglia and white matter of the frontal area. Conclusions: The reduction of NAA/Cr values revealed in H1MRS studies suggests loss of neurons/neuronal activity in the posterior cingulate area and white matter of the left parietal area, in patients with HIV-1 at the stage before clinical manifestations of retroviral infection and structural changes in the plain MR study. This may reflect a

  9. Clinical application of MR susceptibility weighted imaging in cerebrovascular diseases

    Zhu Wenzhen; Qi Jianpin; Shen Hao; Wang Chengyuan; Xia Liming; Hu Junwu; Feng Dingyi

    2007-01-01

    Objective: To assess clinical application value of susceptibility weighted imaging (SWI) in cerebrovascular diseases. Method: Twenty-three patients with cerebrovascular disease were investigated, including 7 cases of cavernoma, 4 of venous hemangioma, 3 of small AVM, 1 of Sturge-Weber Syndrome, 2 of cerebral venous sinus thrombosis and 6 of chronic cerebral infarction. All patients underwent standard Mill and SWI, and most of them also underwent enhanced T 1 WI and MRA. The corrected phase (CP) values were obtained at the lesions and control areas. Results: The average CP values of the lesions and the control areas were -0.112±0.032 and -0.013±0.004, respectively (t=2.167, P 2 WI. The cavemoma could be differentiated from the hemorrhage within lesions. Moreover, multiple microcavernomas were detected on SWI. In 4 cases of venous hemangioma, SWI detected spider-like lesions with more hair-thin pulp veins adjacent to the dilated draining vein than contrast MRI. In 3 cases of small AVM, SWI was more advantageous than MRA in clearly detecting the small feeding artery. In 1 case of Sturge-Weber Syndrome, SWI demonstrated large areas of calcification and the abnormal vessels on the cerebral surface and the deep part of the cerebrum at the same time. In 2 cases of cerebral venous sinus thrombosis, the deep draining veins and superficial venous rete were generally dilated and winding, and the hemorrhagic lesions could be detected earlier than conventional MR images in one case. In 6 eases of cerebral infarction, old hemorrhage was clearly displayed within the lesions. Conclusion: SWI has more predominant advantages than conventional MRI and MRA in detecting the low-flow cerebral vascular malformations, identifying microbleeds and cerebral infarction accompanying hemorrhage, and the dilation of cerebral deep or superficial veins in patients with cerebral venous sinus thrombosis. Moreover, SWI can show the phase contrast between the lesions and the control areas. (authors)

  10. 77 FR 59930 - Clinical Development Programs for Disease-Modifying Agents for Peripheral Neuropathy; Public...

    2012-10-01

    ...] Clinical Development Programs for Disease-Modifying Agents for Peripheral Neuropathy; Public Workshop... to the clinical development of disease-modifying agents for the treatment of peripheral neuropathy... disease-modifying products for the management of peripheral neuropathy. Date and Time: The public workshop...

  11. Variability of CSF Alzheimer's disease biomarkers: implications for clinical practice.

    Stephanie J B Vos

    Full Text Available BACKGROUND: Cerebrospinal fluid (CSF biomarkers are increasingly being used for diagnosis of Alzheimer's disease (AD. OBJECTIVE: We investigated the influence of CSF intralaboratory and interlaboratory variability on diagnostic CSF-based AD classification of subjects and identified causes of this variation. METHODS: We measured CSF amyloid-β (Aβ 1-42, total tau (t-tau, and phosphorylated tau (p-tau by INNOTEST enzyme-linked-immunosorbent assays (ELISA in a memory clinic population (n = 126. Samples were measured twice in a single or two laboratories that served as reference labs for CSF analyses in the Netherlands. Predefined cut-offs were used to classify CSF biomarkers as normal or abnormal/AD pattern. RESULTS: CSF intralaboratory variability was higher for Aβ1-42 than for t-tau and p-tau. Reanalysis led to a change in biomarker classification (normal vs. abnormal of 26% of the subjects based on Aβ1-42, 10% based on t-tau, and 29% based on p-tau. The changes in absolute biomarker concentrations were paralleled by a similar change in levels of internal control samples between different assay lots. CSF interlaboratory variability was higher for p-tau than for Aβ1-42 and t-tau, and reanalysis led to a change in biomarker classification of 12% of the subjects based on Aβ1-42, 1% based on t-tau, and 22% based on p-tau. CONCLUSIONS: Intralaboratory and interlaboratory CSF variability frequently led to change in diagnostic CSF-based AD classification for Aβ1-42 and p-tau. Lot-to-lot variation was a major cause of intralaboratory variability. This will have implications for the use of these biomarkers in clinical practice.

  12. Symptomatic Congenital Cytomegalovirus Infection in Children of Seropositive Women

    Ines Mack

    2017-06-01

    Full Text Available Cytomegalovirus (CMV is the most frequent congenital virus infection worldwide. The risk of congenital CMV (cCMV transmission is highest in seronegative women who acquire primary CMV infection during pregnancy. A growing body of evidence indicates that secondary CMV infections in pregnant women with preconceptual immunity (either through reactivation of latent virus or re-infection with a new strain of CMV contribute to a much greater proportion of symptomatic cCMV than was previously thought. Here, we describe a case of symptomatic cCMV infection in the newborn of a woman with proven immunity prior to pregnancy. Diagnosis was confirmed by CMV PCR from amniotic fluid and fetal MR imaging. The newborn presented with typical cCMV symptoms including jaundice, hepatosplenomegaly, cholestasis, petechiae, small head circumference, and sensorineural hearing loss, the most common neurologic sequela. CMV was detected in infant blood and urine by PCR, and intravenous ganciclovir was initiated and continued orally for 6 weeks totally. Apart from persisting right-sided deafness, the child exhibited normal neurological development up through the last follow-up at 4.5 years. To date, the most effective strategy to prevent vertical CMV transmission is hygiene counseling for women of childbearing age, which, in our case, and in concordance with recent literature, applies to seronegative, as well as seropositive, women. Once an expecting mother shows seroconversion or signs of an active CMV infection, there are no established procedures to reduce the risk of transmission, or therapeutic options for the fetus with signs of infection. After birth, symptomatic infants can be treated with ganciclovir to inhibit viral replication and improve hearing ability and neurodevelopmental outcome. A comprehensive review of the literature, including our case study, reveals the most current and significant diagnostic and treatment options available. In conclusion, the triad

  13. Human Immunodeficiency Virus Seropositivity in Children Aged Six-Eighty-four Months at Kenyatta National Hospital

    Mugo, J.W.; Wafula, D.M.; Ngacha, D.M.; Musia, J.; Plummer, F.M.; Moses, S.; Ndinya-Achola, J.; Mugo, R.W.; Waiyaki, P.

    1992-01-01

    To determine the prevalence of the HIV sero positivity in general paediatric emergency admissions at Kenyatta National Hospital, 552 children were studied systematically for four months in 1991. A clinical history was taken and physical examination conducted. The HIV status was determined by use of organon Teknika's Vironostika viral lysate assay for initial two screenings and then a Behring's enzygnost and anti HIV synthetic peptide assay as a confirmatory assay. Seventy (12.7%) of all the children studied were HIV positive, the mean age of the HIV -positive children was 17.5 months while that of total study population was 23.5 months. HIV seropositivity was not associated with history or parental injections. No haemophilia patient was recruited during the study period, and of all the 18 sicklers recruited, none was HIV positive despite multiple blood transfusions. The WHO paediatric Aids Case definition criteria had moderately low sensitivity (55.7%), high specificity (85.9%) and low positive predictive value (36.4%). This was in agreement with observations noted in earlier studies in East and Central Africa

  14. Moyamoya disease and syndromes: from genetics to clinical management

    Guey S

    2015-02-01

    Full Text Available Stéphanie Guey,1,3 Elisabeth Tournier-Lasserve,1,2 Dominique Hervé,1,3 Manoelle Kossorotoff4 1Inserm UMR-S1161, Université Paris 7 Denis Diderot, Sorbonne Paris Cité, Paris, France; 2AP-HP, Groupe hospitalier Lariboisière-Saint-Louis, Service de génétique neurovasculaire, Paris, France; 3Service de Neurologie, Centre de Référence des maladies Vasculaires Rares du Cerveau et de l'Œil (CERVCO, Groupe Hospitalier Saint-Louis Lariboisière-Fernand Widal, Assistance Publique-Hôpitaux de Paris, Paris, France; 4Pediatric Neurology Department, French Center for Pediatric Stroke, University Hospital Necker-Enfants Malades, AP-HP Assistance publique-Hôpitaux de Paris, Paris, France Abstract: Moyamoya angiopathy is characterized by a progressive stenosis of the terminal portion of the internal carotid arteries and the development of a network of abnormal collateral vessels. This chronic cerebral angiopathy is observed in children and adults. It mainly leads to brain ischemic events in children, and to ischemic and hemorrhagic events in adults. This is a rare condition, with a marked prevalence gradient between Asian countries and Western countries. Two main nosological entities are identified. On the one hand, moyamoya disease corresponds to isolated moyamoya angiopathy, defined as being “idiopathic” according to the Guidelines of the Research Committee on the Pathology and Treatment of Spontaneous Occlusion of the Circle of Willis. This entity is probably multifactorial and polygenic in most patients. On the other hand, moyamoya syndrome is a moyamoya angiopathy associated with an underlying condition and forms a very heterogeneous group with various clinical presentations, various modes of inheritance, and a variable penetrance of the cerebrovascular phenotype. Diagnostic and evaluation techniques rely on magnetic resonance imaging (MRI, magnetic resonance angiography (MRA conventional angiography, and cerebral hemodynamics measurements

  15. Chronic Disease Management in Family Practice: Clinical Note.

    1998-03-01

    disease management in the family practice selling. This paper discusses chronic disease management in the family practice selling....Chronic disease management is the process of evaluating and treating a medical condition or disease state which can not be readily cured so as to...minimize it’s negative impact on the individual. Examples of chronic disease management include the treatment of hypertension, diabetes, osteoporosis

  16. Clinical spectrum of impulse control disorders in Parkinson's disease.

    Weintraub, Daniel; David, Anthony S; Evans, Andrew H; Grant, Jon E; Stacy, Mark

    2015-02-01

    Impulse control disorders (ICDs), including compulsive gambling, buying, sexual behavior, and eating, are a serious and increasingly recognized psychiatric complication in Parkinson's disease (PD). Other impulsive-compulsive behaviors (ICBs) have been described in PD, including punding (stereotyped, repetitive, purposeless behaviors) and dopamine dysregulation syndrome (DDS; compulsive PD medication overuse). ICDs have been most closely related to the use of dopamine agonists (DAs), perhaps more so at higher doses; in contrast, DDS is primarily associated with shorter-acting, higher-potency dopaminergic medications, such as apomorphine and levodopa. Possible risk factors for ICDs include male sex, younger age and younger age at PD onset, a pre-PD history of ICDs, and a personal or family history of substance abuse, bipolar disorder, or gambling problems. Given the paucity of treatment options and potentially serious consequences, it is critical for PD patients to be monitored closely for development of ICDs as part of routine clinical care. © 2014 International Parkinson and Movement Disorder Society.

  17. Ivabradine in stable coronary artery disease without clinical heart failure

    Fox, Kim; Ford, Ian; Steg, Philippe Gabriel

    2014-01-01

    minute or more. METHODS: We conducted a randomized, double-blind, placebo-controlled trial of ivabradine, added to standard background therapy, in 19,102 patients who had both stable coronary artery disease without clinical heart failure and a heart rate of 70 beats per minute or more (including 12......,049 patients with activity-limiting angina [class ≥II on the Canadian Cardiovascular Society scale, which ranges from I to IV, with higher classes indicating greater limitations on physical activity owing to angina]). We randomly assigned patients to placebo or ivabradine, at a dose of up to 10 mg twice daily......, with the dose adjusted to achieve a target heart rate of 55 to 60 beats per minute. The primary end point was a composite of death from cardiovascular causes or nonfatal myocardial infarction. RESULTS: At 3 months, the mean (±SD) heart rate of the patients was 60.7±9.0 beats per minute in the ivabradine group...

  18. Diffusion abnormality maps in demyelinating disease: Correlations with clinical scores

    Onu, Mihaela; Roceanu, Adina; Sboto-Frankenstein, Uta; Bendic, Robert; Tarta, Eugen; Preoteasa, Florentin; Bajenaru, Ovidiu

    2012-01-01

    Background and purpose: Magnetic resonance imaging (MRI) has been explored as a noninvasive tool to assess pathology in multiple sclerosis (MS) patients. However, the correlation between classical MRI measures and physical disability is modest in MS. The diffusion tensor imaging (DTI) MRI technique holds particular promise in this regard. The present study shows brain regions where FA and individual diffusivities abnormalities are present and check their correlations with physical disability clinical scores. Methods: Eight patients and 12 matched healthy controls were recruited. The Multiple Sclerosis Functional Composite was administered. For MR-DTI acquisitions, a Genesis Signa 1.5T MR system, an EP/SE scanning sequence, 25 gradient directions were used. Results: Tract Based Spatial Statistics (TBSS) group comparisons showed reduced FA and increased individual diffusivities in several brain regions in patients. Significant correlations were found between FA and: EDSS, 9-HPT(NON)DOM and 25FW score; between λ 2 and: P100 (r and l), 9-HPT(NON)DOM and 25FW; between λ 3 and: 9-HPT(NON)DOM and 25FW score. Conclusions: Fractional anisotropy and individual radial diffusivities proved to be important markers of motor disabilities in MS patients when the disease duration mean and the disability scores values range are relatively high.

  19. Heterogeneous pattern of bone disease in adult type 1 Gaucher disease: clinical and pathological correlates.

    van Dussen, L; Lips, P; van Essen, H W; Hollak, C E M; Bravenboer, N

    2014-09-01

    Gaucher disease (GD) is a lysosomal storage disorder characterized by accumulation of glucosylceramide in macrophages, so-called Gaucher cells, as a result of a deficiency of the lysosomal enzyme glucocerebrosidase. Bone complications are an important cause of morbidity of GD and are thought to result from imbalance in bone remodeling. Bone manifestations among GD patients demonstrate a large variation including increased osteoclastic bone resorption, low bone formation and osteonecrosis. The purpose of the current case series is to describe the histological features observed in undecalcified bone samples, obtained from three GD patients, and evaluate the relationship with clinical features in these patients. Bone fragments were obtained from three adult type 1 GD patients with variable degrees of bone disease during orthopedic surgery. Specimens were embedded without prior decalcification in methylmethacrylate and prepared for histology according to standardized laboratory procedures. Histology revealed a heterogeneous pattern of bone involvement. High cellularity of bone marrow, abundant presence of Gaucher cells (GCs) and high turnover were observed in a patient with a history of multiple bone complications, while minimal bone turnover and few GCs were detected in the mildest affected patient in this series. An intermediate picture with relatively low bone turnover and a substantial amount of Gaucher cells was demonstrated in the third, moderately affected patient. No gross abnormalities in three biochemical markers of bone turnover (osteocalcin, N-terminal propeptide of type 1 procollagen and type 1 collagen C-terminal telopeptide) were noted. Plastic embedding and subsequent Goldner and TRAP staining offered a unique possibility to study bone histological findings in GD. Our data show that bone manifestations in GD may vary both clinically as well as histologically and bone disease in GD will likely require a personalized approach. Copyright © 2014 Elsevier

  20. The Infectious Diseases Society of America emerging infections network: bridging the gap between clinical infectious diseases and public health.

    Pillai, Satish K; Beekmann, Susan E; Santibanez, Scott; Polgreen, Philip M

    2014-04-01

    In 1995, the Centers for Disease Control and Prevention granted a Cooperative Agreement Program award to the Infectious Diseases Society of America to develop a provider-based emerging infections sentinel network, the Emerging Infections Network (EIN). Over the past 17 years, the EIN has evolved into a flexible, nationwide network with membership representing a broad cross-section of infectious disease physicians. The EIN has an active electronic mail conference (listserv) that facilitates communication among infectious disease providers and the public health community, and also sends members periodic queries (short surveys on infectious disease topics) that have addressed numerous topics relevant to both clinical infectious diseases and public health practice. The article reviews how the various functions of EIN contribute to clinical care and public health, identifies opportunities to further link clinical medicine and public health, and describes future directions for the EIN.

  1. A Review of the Centers for Disease Control and Prevention's Guidelines for the Clinical Laboratory Diagnosis of Lyme Disease.

    Miraglia, Caterina M

    2016-12-01

    The purpose of this paper is to review information regarding the current guidelines for the clinical laboratory diagnosis of Lyme disease as set forth by the Centers for Disease Control and Prevention (CDC) to chiropractic physicians and to discuss the clinical utility of this testing. The CDC's website was reviewed to determine what their current recommendations are for the clinical laboratory testing of Lyme disease. The CDC's established guidelines recommend the use of a 2-tiered serologic testing algorithm for the evaluation of patients with suspected Lyme disease. This review provides doctors of chiropractic with information to remain current with the CDC's recommended guidelines for Lyme disease testing because patients may present to their office with the associated signs and symptoms of Lyme disease.

  2. Automated interpretable computational biology in the clinic: a framework to predict disease severity and stratify patients from clinical data

    Soumya Banerjee

    2017-10-01

    Full Text Available We outline an automated computational and machine learning framework that predicts disease severity and stratifies patients. We apply our framework to available clinical data. Our algorithm automatically generates insights and predicts disease severity with minimal operator intervention. The computational framework presented here can be used to stratify patients, predict disease severity and propose novel biomarkers for disease. Insights from machine learning algorithms coupled with clinical data may help guide therapy, personalize treatment and help clinicians understand the change in disease over time. Computational techniques like these can be used in translational medicine in close collaboration with clinicians and healthcare providers. Our models are also interpretable, allowing clinicians with minimal machine learning experience to engage in model building. This work is a step towards automated machine learning in the clinic.

  3. Vascular care in patients with Alzheimer's disease with cerebrovascular lesions-a randomized clinical trial

    Richard, Edo; Kuiper, Roy; Dijkgraaf, Marcel G. W.; van Gool, Willem A.

    2009-01-01

    OBJECTIVES: To investigate whether vascular care slows dementia progression in patients with Alzheimer's disease with cerebrovascular lesions on neuroimaging. DESIGN: Multicenter randomized controlled clinical trial with 2-year follow-up. SETTING: Neurological and geriatric outpatient clinics in 10

  4. Clinical Considerations of Preimplantation Genetic Diagnosis for Monogenic Diseases.

    Xiaokun Hu

    Full Text Available The aim of this study was to explore factors contribute to the success of PGD cycles for monogenic diseases.During a 3-year period (January 2009 to December 2012, 184 consecutive ICSI-PGD cycles for monogenic diseases reaching the ovum pick-up and fresh embryo-transfer stage performed at the Reproductive Medicine Center of The First Affiliated Hospital Of Sun Yat-sen University were evaluated.ICSI was performed on 2206 metaphase II oocytes, and normal fertilization and cleavage rates were 83.4% (1840/2206 and 96.2% (1770/1840, respectively. In the present study, 60.5% (181/299 of day 3 good-quality embryos developed into good-quality embryos on day 4 after biopsy. Collectively, 42.9% clinical pregnancy rate (79/184 and 28.5% implantation rate (111/389 were presented. In the adjusted linear regression model, the only two significant factors affecting the number of genetically unaffected embryos were the number of biopsied embryos (coefficient: 0.390, 95%CI 0.317-0.463, P = 0.000 and basal FSH level (coefficient: 0.198, 95%CI 0.031-0.365, P = 0.021. In the adjusted binary logistic regression model, the only two significant factors affecting pregnancy outcome were the number of genetically available transferable embryos after PGD (adjusted OR 1.345, 95% CI 1.148-1.575, P = 0.000 and number of oocyte retrieved (adjusted OR 0.934, 95% CI 0.877-0.994, P = 0.031.There should be at least four biopsied embryos to obtain at least one unaffected embryos in a PGD system for patients with single gene disorder and under the condition of basal FSH level smaller than 8.0mmol/L. Moreover, if only a low number (< 4 of biopsied embryos are available on day 3, the chance of unaffected embryos for transfer was small, with poor outcome.

  5. An Update on Renal Artery Denervation and Its Clinical Impact on Hypertensive Disease

    Bhat, Aditya; Kuang, Ye Min; Gan, Gary C. H.; Burgess, David; Denniss, Alan Robert

    2015-01-01

    Hypertension is a globally prevalent condition, with a heavy clinical and economic burden. It is the predominant risk factor for premature cardiovascular and cerebrovascular disease, and is associated with a variety of clinical disorders including stroke, congestive cardiac failure, ischaemic heart disease, chronic renal failure, and peripheral arterial disease. A significant subset of hypertensive patients have resistant hypertensive disease. In this group of patients, catheter-based renal a...

  6. Impact of PGL-I seropositivity on the protective effect of BCG vaccination among leprosy contacts: a cohort study.

    Nádia C Düppre

    Full Text Available BACKGROUND: Contacts of leprosy patients are at increased risk of developing leprosy and need to be targeted for early diagnosis. Seropositivity to the phenolic glycolipid I (PGL-I antigen of Mycobacterium leprae has been used to identify contacts who have an increased risk of developing leprosy. In the present study, we studied the effect of seropositivity in patient contacts, on the risk of developing leprosy, stratified by Bacille Calmette Guerin (BCG vaccination after index case diagnosis. METHODOLOGY/PRINCIPAL FINDINGS: Leprosy contacts were examined as part of the surveillance programme of the Oswaldo Cruz Institute Leprosy Outpatient Clinic in Rio de Janeiro. Demographic, social, epidemiological and clinical data were collected. The presence of IgM antibodies to PGL-I in sera and BCG vaccination status at the time of index case diagnosis were evaluated in 2,135 contacts. During follow-up, 60 (2.8%; 60/2,135 leprosy cases were diagnosed: 41 among the 1,793 PGL-I-negative contacts and 19 among the 342 PGL-I-positive contacts. Among PGL-I-positive contacts, BCG vaccination after index case diagnosis increased the adjusted rate of developing clinical manifestations of leprosy (Adjusted Rate Ratio (aRR = 4.1; 95% CI: 1.8-8.2 compared with the PGL-I-positive unvaccinated contacts (aRR = 3.2; 95% CI: 1.2-8.1. The incidence density was highest during the first year of follow-up for the PGL-I-positive vaccinated contacts. However, all of those contacts developed PB leprosy, whereas most MB cases (4/6 occurred in PGL-I-positive unvaccinated contacts. CONCLUSION: Contact examination combined with PGL-I testing and BCG vaccination remain important strategies for leprosy control. The finding that rates of leprosy cases were highest among seropositive contacts justifies targeting this specific group for close monitoring. Furthermore, it is recommended that PGL-I-positive contacts and contacts with a high familial bacteriological index

  7. Impact of PGL-I Seropositivity on the Protective Effect of BCG Vaccination among Leprosy Contacts: A Cohort Study

    Düppre, Nádia C.; Camacho, Luiz Antonio B.; Sales, Anna M.; Illarramendi, Ximena; Nery, José Augusto C.; Sampaio, Elizabeth P.; Sarno, Euzenir N.; Bührer-Sékula, Samira

    2012-01-01

    Background Contacts of leprosy patients are at increased risk of developing leprosy and need to be targeted for early diagnosis. Seropositivity to the phenolic glycolipid I (PGL-I) antigen of Mycobacterium leprae has been used to identify contacts who have an increased risk of developing leprosy. In the present study, we studied the effect of seropositivity in patient contacts, on the risk of developing leprosy, stratified by Bacille Calmette Guerin (BCG) vaccination after index case diagnosis. Methodology/Principal Findings Leprosy contacts were examined as part of the surveillance programme of the Oswaldo Cruz Institute Leprosy Outpatient Clinic in Rio de Janeiro. Demographic, social, epidemiological and clinical data were collected. The presence of IgM antibodies to PGL-I in sera and BCG vaccination status at the time of index case diagnosis were evaluated in 2,135 contacts. During follow-up, 60 (2.8%; 60/2,135) leprosy cases were diagnosed: 41 among the 1,793 PGL-I-negative contacts and 19 among the 342 PGL-I-positive contacts. Among PGL-I-positive contacts, BCG vaccination after index case diagnosis increased the adjusted rate of developing clinical manifestations of leprosy (Adjusted Rate Ratio (aRR) = 4.1; 95% CI: 1.8–8.2) compared with the PGL-I-positive unvaccinated contacts (aRR = 3.2; 95% CI: 1.2–8.1). The incidence density was highest during the first year of follow-up for the PGL-I-positive vaccinated contacts. However, all of those contacts developed PB leprosy, whereas most MB cases (4/6) occurred in PGL-I-positive unvaccinated contacts. Conclusion Contact examination combined with PGL-I testing and BCG vaccination remain important strategies for leprosy control. The finding that rates of leprosy cases were highest among seropositive contacts justifies targeting this specific group for close monitoring. Furthermore, it is recommended that PGL-I-positive contacts and contacts with a high familial bacteriological index, regardless of

  8. Advances in clinical application of optical coherence tomography in vitreomacular interface disease

    Xiao-Li Xing

    2013-08-01

    Full Text Available Vitreous macular interface disease mainly includes vitreomacular traction syndrome, idiopathic macular epiretinal membrane and idiopathic macular hole. Optical coherence tomography(OCTas a new tool that provides high resolution biopsy cross section image non traumatic imaging inspection, has a unique high resolution, no damage characteristics, and hence clinical widely used, vitreous macular interface for clinical disease diagnosis, differential diagnosis and condition monitoring and quantitative evaluation, treatment options, etc provides important information and reference value. Vitreous macular interface disease in OCT image of anatomical morphology characteristics, improve the clinical on disease occurrence and development of knowledge. We reviewed the advances in the application of OCT in vitreomacular interface disease.

  9. Ebola Virus Disease Candidate Vaccines Under Evaluation in Clinical Trials

    2016-06-02

    evidence that oral vaccines fail in populations with disturbed microbiota, poor nutrition , and high intestinal inflammation [102-104]. Additionally...countermeasure development against Ebola virus disease becoming a global public- health priority. This review summarizes the status quo of candidate...members of the mononegaviral family Filoviridae) cause two diseases recognized by the World Health Organization (WHO): Ebola virus disease (EVD) can be

  10. Precision medicine in airway diseases: moving to clinical practice

    Agustí, Alvar; Bafadhel, Mona; Beasley, Richard; Bel, Elisabeth H.; Faner, Rosa; Gibson, Peter G.; Louis, Renaud; McDonald, Vanessa M.; Sterk, Peter J.; Thomas, Mike; Vogelmeier, Claus; Pavord, Ian D.

    2017-01-01

    On February 21, 2017, a European Respiratory Society research seminar held in Barcelona discussed how to best apply precision medicine to chronic airway diseases such as asthma and chronic obstructive pulmonary disease. It is now clear that both are complex and heterogeneous diseases, that often

  11. Neuroinflammation and Alzheimer disease: clinical and therapeutic implications

    Eikelenboom, P.; Rozemuller, A. J.; Hoozemans, J. J.; Veerhuis, R.; van Gool, W. A.

    2000-01-01

    In Alzheimer disease brains, the amyloid plaques are closely associated with a locally induced, nonimmune-mediated, chronic inflammatory response without any apparent influx of leukocytes from the blood. The present findings indicate that in cerebral A beta diseases (Alzheimer disease, Down

  12. Lyme disease in Wisconsin: epidemiologic, clinical, serologic, and entomologic findings.

    Davis, J P; Schell, W L; Amundson, T E; Godsey, M S; Spielman, A; Burgdorfer, W; Barbour, A G; LaVenture, M; Kaslow, R A

    1984-01-01

    In 1980-82, 80 individuals (71 Wisconsin residents) had confirmed Lyme disease (LD-c) reported; 39 additional patients had probable or possible LD. All cases of LD-c occurred during May-November; 73 percent occurred during June-July; 54 (68 percent) occurred in males. The mean age was 38.7 years (range, 7-77 years). Among LD-c patients, likely exposure to the presumed vector Ixodes dammini (ID) occurred in 22 different Wisconsin counties. Antibodies to the ID spirochete that causes LD occurred in 33 of 49 LD-c cases versus 0 of 18 in ill controls (p less than .001) and in 13 of 26 LD-c cases treated with penicillin or tetracycline versus 16 of 19 LD-c cases not treated. Early antibiotic therapy appears to blunt the antibody response to the ID spirochete. Regional tick surveys conducted in Wisconsin during each November in 1979-82 have demonstrated regions of greater density of ID. Utilizing comparable tick collection in these surveys, increases were noted in the percentage of deer with ID from 24 percent (31/128) in 1979 to 38 percent (58/152) in 1981, in the standardized mean value of ID/deer from 1.0 in 1979 to 2.2 in 1981, in the percentage of ID of the total ticks collected from 13 percent in 1979 to 71 percent in 1981, or in the ratio of ID to Dermacentor albipictus ticks from 0.14 in 1979 to 2.44 in 1981. However, a reduction in the density of ID/deer was noted generally throughout Wisconsin in 1982 when compared to 1981. LD is widespread in Wisconsin, with ecologic and clinical features similar to those occurring along the eastern seaboard.

  13. Pharmacogenetics: progress, pitfalls and clinical potential for coronary heart disease.

    Humphries, Steve E; Hingorani, Aroon

    2006-02-01

    Much has been written about the potential of pharmacogenetic testing to inform therapy based on an individual's genetic makeup, and to decide the most effective choice of available drugs, or to avoid dangerous side effects. Currently, there is little hard data for either in the field of cardiovascular disease. The usual approach has been opportunistic use of drug trials in unrelated patients, and to look for differences in response or outcome by "candidate gene" genotype, for example genes coding for drug metabolising enzymes (activators and metabolisers), and enzymes and receptors involved in lipid metabolism, adrenergic response, etc. As with all association studies, initially promising results have often failed the test of replication in larger studies, and the relationship between the CETP Taq-I variant and response to statins has now been disproved. The strongest data to date is the report [Chasman, D.I., Posada, D., Subrahmanyan, L., Cook, N.R., Stanton Jr., V.P., Ridker, P.M., 2004. Pharmacogenetic study of statin therapy and cholesterol reduction. J. Am. Med. Assoc. 291, 2821-2827] of a poorer cholesterol-lowering response to Pravastatin in the 7% of patients carrying a certain haplotype of the HMG CoA reductase gene (14% fall versus 19%), but if this is overcome simply by a higher dose, it is of little clinical relevance. Currently, the best example of avoiding side effects is determining genotype at the CYP2C9 locus with respect of warfarin treatment, since carriers for functional variants (>20% of the population) require lower doses for optimal anticoagulation, and homozygotes, although rare, may well experience serious bleeding if given a usual dose. The full potential of this field will only be realised with much further work.

  14. Reiter′s Disease - Clinical Profile of Epidemic Form

    H S Girgia

    1980-01-01

    Full Text Available Absence of urethritis need not exclude the possiblity of Reiter′s disease in young males where conjunctivitis and polyarthritis are cardinal features. Appearance of cutaneous lesion early in the course of the disease heralds a poor prognosis specially in the rare epidemic form of the disease. Two cases of Reiter′s disease are reported. Both belonged to the dysentric type of the disease; sometimes ref to as the epidemic form. Relatively high dose of steroids necessary to control symptoms.

  15. A Case-Control Study of Risk Factors for Bovine Brucellosis Seropositivity in Peninsular Malaysia

    Anka, Mukhtar Salihu; Hassan, Latiffah; Khairani-Bejo, Siti; Zainal, Mohamed Abidin; Mohamad, Ramlan bin; Salleh, Annas; Adzhar, Azri

    2014-01-01

    Bovine brucellosis was first reported in Peninsular Malaysia in 1950. A subsequent survey conducted in the country revealed that the disease was widespread. Current knowledge on the potential risk factors for brucellosis occurrence on cattle farms in Malaysia is lacking. Therefore, we conducted a case-control study to identify the potential herd-level risk factors for bovine brucellosis occurrence in four states in the country, namely Kelantan, Pahang, Selangor and Negeri Sembilan. Thirty-five cases and 36 controls of herds were selected where data on farm management, biosecurity, medical history and public health were collected. Multivariable logistic regression identified that Brucella seropositive herds were more likely to; have some interaction with wildlife (OR 8.9, 95% CI = 1.59–50.05); originated from farms where multiple species such as buffalo/others (OR 41.8, 95% CI = 3.94–443.19) and goat/sheep (OR 8.9, 95%Cl = 1.10–71.83) were reared, practice extensive production system (OR 13.6, 95% CI 1.31–140.24) and have had episodes of abortion in the past (OR 51.8, 95% CI = 4.54–590.90) when compared to seronegative herds. Considering the lack of information on the epidemiology of bovine brucellosis in peninsular Malaysia and absence of information on preventing the inception or spread of the disease, this report could contribute to the on-going area-wise national brucellosis eradication program. PMID:25265020

  16. Association of conjunctival and corneal calcification with vascular calcification among hepatitis-C-seropositive hemodialysis patients.

    AbouSeif, Khaled; Sany, Dawlat; Elshahawy, Yasser; Seddik, Ayman; Rahman, Khedr; Gaber, Moustapha

    2016-01-01

    Disorders associated with the hepatitis C virus (HCV) have been reported including cardiovascular, metabolic, and central nervous system diseases. Since chronic HCV infections may be curable, their identification as causal contributors to cardiovascular risk could offer new perspectives in the prevention of cardiovascular disease. The aim of this study is to investigate the association between HCV and aortic arch calcification (AAC) and corneal and conjunctival calcification (CCC) in maintenance hemodialysis (MHD) patients; further, we assessed the correlation of CCC with vascular calcification. A total of 100 patients undergoing hemodialysis (HD) in our hospital were included in this study. Patients underwent a complete ocular examination including intraocular pressure, and CCC was looked for by slit lamp and fundoscopy. CCC was graded according to modified Porter and Crombie classification system described by Tokuyama et al. Helical computerized tomographic chest examination was used to evaluate the grading of AAC. Demographic, hematological, biochemical, and dialysis-related data were obtained. There was significant difference between seropositive (n = 51) and seronegative patients (n = 49) regarding grading of AAC and CCC (P <0.001). Significant positive correlation was found between grading of CCC, AAC, age (P <0.001), duration on HD (P <0.001), HCV-antibody positivity (P <0.001), serum calcium level (P <0.001), serum phosphorus level (P <0.001), calcium × phosphorus product (P <0.001), and i-parathormone level (P < 0.001). In addition, CCC grading positively correlated with AAC. Our results suggest that patients undergoing HD infected with the HCV have high degree of CCC, AAC, and mineral metabolism disorder. The strong correlation between CCC and AAC indicates that CCC evaluation is an easy, fast, non-invasive method, and might be used as an indirect indicator to detect vascular calcification in patients undergoing MHD.

  17. Association of conjunctival and corneal calcification with vascular calcification among hepatitis-C-seropositive hemodialysis patients

    Khaled AbouSeif

    2016-01-01

    Full Text Available Disorders associated with the hepatitis C virus (HCV have been reported including cardiovascular, metabolic, and central nervous system diseases. Since chronic HCV infections may be curable, their identification as causal contributors to cardiovascular risk could offer new perspectives in the prevention of cardiovascular disease. The aim of this study is to investigate the association between HCV and aortic arch calcification (AAC and corneal and conjunctival calcification (CCC in maintenance hemodialysis (MHD patients; further, we assessed the correlation of CCC with vascular calcification. A total of 100 patients undergoing hemodialysis (HD in our hospital were included in this study. Patients underwent a complete ocular examination including intraocular pressure, and CCC was looked for by slit lamp and fundoscopy. CCC was graded according to modified Porter and Crombie classification system described by Tokuyama et al. Helical computerized tomographic chest examination was used to evaluate the grading of AAC. Demographic, hematological, biochemical, and dialysis-related data were obtained. There was significant difference between seropositive (n = 51 and seronegative patients (n = 49 regarding grading of AAC and CCC (P <0.001. Significant positive correlation was found between grading of CCC, AAC, age (P <0.001, duration on HD (P <0.001, HCV-antibody positivity (P <0.001, serum calcium level (P <0.001, serum phosphorus level (P <0.001, calcium × phosphorus product (P <0.001, and i-parathormone level (P < 0.001. In addition, CCC grading positively correlated with AAC. Our results suggest that patients undergoing HD infected with the HCV have high degree of CCC, AAC, and mineral metabolism disorder. The strong correlation between CCC and AAC indicates that CCC evaluation is an easy, fast, non-invasive method, and might be used as an indirect indicator to detect vascular calcification in patients undergoing MHD.

  18. THE CLINICAL PRESENTATION OF AUTOIMMUNE THYROID DISEASE IN MEN IS ASSOCIATED WITH IL12B GENOTYPE

    Walsh, John P; Berry, Jemma; Liu, Shu

    2011-01-01

    hypothesized that IL12B genotype may influence the clinical presentation of autoimmune thyroid disease. Objective.  We tested for differences in IL12B genotype between Graves' disease and Hashimoto's disease. Patients.  We studied a discovery cohort of 203 Australian women and 37 men with autoimmune thyroid......' disease (P=0.005) and Hashimoto's disease (P=0.029). Conclusion.  In men with autoimmune thyroid disease, a common variant located upstream of the IL12B coding region may influence whether patients present with Graves' disease or Hashimoto's disease....

  19. IgG4-RELATED DISEASE. CLINICAL NOTES

    Vladimir Ivanovich Vasilyev

    2013-01-01

    Full Text Available IgG4-related diseases are a new nosological entity that encompasses a few previously known diseases. IgG4-related systemic disease is diagnosed if two or more affected organs are detected. This group of diseases has two similar signs: serological (elevated serum IgG4 subclass concentrations and histological (organ and tissue infiltration from plasmo-cytes secreting IgG4, and eosinophils, and the development of fibrosclerosis and phlebitis obliterans. The paper describes two cases. In one case, a multisystemic disease was observed virtually at its onset whereas in the other this lesion was diagnosed several years after the natural course of the disease.

  20. Periodontal disease and pregnancy hypertension: a clinical correlation.

    Pralhad, Swati; Thomas, Betsy; Kushtagi, Pralhad

    2013-08-01

    Periodontal disease is thought to be associated with increased risk of systemic diseases and adverse pregnancy outcomes, including pregnancy hypertension (PH). The aim of the present study is to find the prevalence of periodontal disease in females with PH in a rural-based medical institute. The present case control study was performed with 200 females, 100 with PH and 100 without PH. Antenatal periodontal screening was performed within 72 hours of their hospital admission for delivery. The periodontal parameters assessed were oral hygiene index-simplified, gingival index, mean probing depth, and loss of attachment. Prevalence of periodontal disease was 65.5% and was significantly higher (P periodontal disease and PH on bivariate multiple logistic regression analysis. Nulliparous females were at higher odds to develop periodontal disease and PH (odds ratio = 1.7; 95% CI = 0.5 to 6.1). As the severity of periodontal disease increased from moderate to severe, the severity of hypertension also increased (r(2) = 0.8 and 0.5 for moderate and severe periodontal disease, respectively). Periodontal disease is more prevalent in females with PH.

  1. Harnessing Cerebrospinal Fluid Biomarkers in Clinical Trials for Treating Alzheimer's and Parkinson's Diseases: Potential and Challenges

    Kim, Dana; Kim, Young-Sam; Shin, Dong Wun; Park, Chang-Shin; Kang, Ju-Hee

    2016-01-01

    No disease-modifying therapies (DMT) for neurodegenerative diseases (NDs) have been established, particularly for Alzheimer's disease (AD) and Parkinson's disease (PD). It is unclear why candidate drugs that successfully demonstrate therapeutic effects in animal models fail to show disease-modifying effects in clinical trials. To overcome this hurdle, patients with homogeneous pathologies should be detected as early as possible. The early detection of AD patients using sufficiently tested bio...

  2. The Prevalence of Chagas Heart Disease in a Central Bolivian Community Endemic for Trypanosoma Cruzi

    Yager, Jessica E.; Lozano Beltran, Daniel F.; Torrico, Faustino; Gilman, Robert H.; Bern, Caryn

    2015-01-01

    Background Though the incidence of new Trypanosoma cruzi infections has decreased significantly in endemic regions in the Americas, medical professionals continue to encounter a high burden of resulting Chagas disease among infected adults. The current prevalence of Chagas heart disease in a community setting is not known; nor is it known how recent insecticide vector control measures may have impacted the progression of cardiac disease in an infected population. Objectives and Methods Nested within a community serosurvey in rural and periurban communities in central Bolivia, we performed a cross-sectional cardiac substudy to evaluate adults for historical, clinical, and electrocardiographic evidence of cardiac disease. All adults between the ages of 20 and 60 years old with T. cruzi infection and those with a clinical history, physical exam, or ECG consistent with cardiac abnormalities were also scheduled for echocardiography. Results and conclusions Of the 604 cardiac substudy participants with definitive serology results, 183 were seropositive for infection with T. cruzi (30.3%). Participants who were seropositive for T. cruzi infection were more likely to have conduction system defects (1.6% versus 0 for complete right bundle branch block and 10.4% versus 1.9% for any bundle branch block; p=0.008 and p<0.001, respectively). However, there was no statistically significant difference in the prevalence of bradycardia among seropositive versus seronegative participants. Echocardiogram findings were not consistent with a high burden of Chagas cardiomyopathy: valvulopathies were the most common abnormality, and few participants were found to have low ejection fraction or left ventricular dilatation. No participants had significant heart failure. Though almost one third of adults in the community were seropositive for T. cruzi infection, few had evidence of Chagas heart disease. PMID:26407509

  3. [HCV and HBV seropositivity in university students of the State of Nuevo Leòn, Mexico].

    Flores-Castañeda, M S; García-Méndez, B L; Tijerina-Menchaca, R

    1996-01-01

    Viral hepatitis is a contagious disease. Patients infected with hepatitis B virus (HBV) or hepatitis C virus (HCV), may be either chronically symptomatic or asymptomatic, and suffer cirrhosis and high risk of hepatic carcinoma. Asymptomatic carriers of HBV surface antigen (HBs-Ag) or with anti-HCV antibodies are potentially infectious, and therefore a risk to public health. This work seeks to establish the frequency of seropositivity for HBs-Ag and anti-HCV antibodies in a population of 774 newly accepted students of the Medical School of the Autonomous University of Nuevo Leon, whose average age was 18 years. Second generation ELISA test were used to screen for HBs-Ag and anti-HCV antibodies. HBs-Ag was confirmed by a neutralization test and anti-HCV antibodies were confirmed by a RIBA test. Three sera were positive for HBs-Ag by ELISA and only one serum (0.13% of analyzed samples) was confirmed by the neutralization technique. On the other hand 12 sera were positive for anti-HCV antibodies by ELISA, and eight of these were confirmed by RIBA (1.03% of the analyzed samples). Intensive reactivity bands were found in two sera, and weak reactivity bands were found in six sera. ELISA screening for anti-HCV antibodies showed 0.5% of false positives. This study shows that the frequency of anti-HCV antibodies is 7.95% times higher than that found for HBs-Ag. All seropositive patients were asymptomatic and potentially infective. This demonstrates the need to routinely screen for HBs-Ag and anti-HCV antibodies to establish the prevalence of these diseases in our area.

  4. Diseases of poultry in Akure, Nigeria: a ten year analysis of clinical ...

    An analysis of poultry diseases diagnosed at the avian clinic of Veterinary Hospital of the Ministry of Agriculture and Natural Resources, Akure, Ondo-State, was carried out using data from 22,927 cases reported at the clinic over a period of ten years. The commonest diseases reported were Newcastle, 9.8%, chronic ...

  5. CHRONIC OBSTRUCTIVE PULMONARY DISEASE: DEFINITION, EPIDEMIOLOGY, PATHOPHYSIOLOGY, CLINICAL PICTURE AND TREATMENT (GOLD 2013

    M. T. Vatutin

    2015-01-01

    Full Text Available Chronic obstructive pulmonary disease: definition, epidemiology, pathophysiology, clinical picture (GOLD 2013. Vatutin M.T., Smyrnova G.S., Taradin G.G. The represented translation of the new international guidelines (GOLD 2013 reflected the epidemiology, pathophysiology, clinical picture and treatment of chronic obstructive pulmonary disease.

  6. Prevention of cervical cancer in HIV-seropositive women from developing countries: a systematic review protocol.

    Mapanga, Witness; Elhakeem, Ahmed; Feresu, Shingairai A; Maseko, Fresier; Chipato, Tsungai

    2017-04-24

    Over 85% of cervical cancer cases and deaths occur in developing countries. HIV-seropositive women are more likely to develop precancerous lesions that lead to cervical cancer than HIV-negative women. However, the literature on cervical cancer prevention in seropositive women in developing countries has not been reviewed. The aim of this study is to systematically review cervical cancer prevention modalities available for HIV-seropositive women in developing countries. This protocol was developed by following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses Protocols (PRISMA-P) statement, and the systematic review will be reported in accordance with the PRISMA guidelines. Embase, MEDLINE, PubMed, CINAHL and Cochrane Library will be searched from inception up to date of final search, and additional studies will be located through citation and reference list tracking. Eligible studies will be randomised controlled trials, prospective and retrospective cohort studies, case-control and cross-sectional studies carried out in developing countries. Studies will be included if they are published in English and examine cervical cancer prevention modalities in HIV-seropositive women. Results will be summarised in tables and, where appropriate, combined using meta-analysis. This review will address the gap in evidence by systematically reviewing the published literature on the different prevention modalities being used to prevent cervical cancer in HIV-seropositive women in developing countries. The findings may be used to inform evidence-based guidelines for prevention of cervical cancer in seropositive women as well as future research. PROSPERO CRD42017054678 .

  7. Coxiella burnetii seropositivity and associated risk factors in goats in Ontario, Canada.

    Meadows, S; Jones-Bitton, A; McEwen, S; Jansen, J; Menzies, P

    2015-10-01

    Coxiella burnetii is a zoonotic bacterium, and infection in goats with this bacterium can result in abortion, stillbirth or birth of non-viable kids. A cross-sectional study was conducted to identify the seroprevalence and risk factors for C. burnetii exposure in Ontario goats. Sera were collected between August 2010 and February 2012, and tested for C. burnetii specific antibodies using an enzyme-linked immunosorbent assay (IDEXX). Overall, 63.2% (48/76, 95% CI=51.9-73.4) of farms had one or more seropositive goats. A higher farm-level seroprevalence of 78.6% (33/42) was found on dairy goat farms, compared to 44.1% (15/34) on meat goat farms (pgoats were seropositive. Similarly, a higher individual-level seroprevalence was identified for dairy goats (43.7%, 633/1447) compared to meat goats (10.8%, 81/748) (pfarm-level clustering identified risk factors associated with seropositivity (pgoat farms were located in a 5-km radius, goats had 5.6 times (95% CI=1.01-30.8) times the odds of seropositivity compared to those that were not. Relative to goats from farms where all kidding pen hygiene was practiced (adding bedding, removing birth materials and disinfection after kidding), goats from farms which only added bedding and removed birth materials had a higher odds of seropositivity (OR=19.3, 95% CI=1.1-330.4), as did goats from farms which practiced none of these measures (OR=161.0, 95% CI=2.4-10822.2). An interaction term revealed kidding outdoors when there were no swine on farm had a protective effect on seropositivity compared to kidding indoors, or kidding outdoors with swine on the farm. These results can inform strategies to mitigate exposure to C. burnetii in Ontario. Copyright © 2015 Elsevier B.V. All rights reserved.

  8. Risk Factors Associated with Brucella Seropositivity in Sheep and Goats in Duhok Province, Iraq

    Ali. G. Alhamada

    2017-12-01

    Full Text Available Sera from 432 small ruminants (335 sheep and 97 goats from 72 farms in Duhok Province, northern Iraq, were collected to investigate risk factors associated with brucellosis seropositivity. Serum samples were tested using the Rose Bengal test (RBT and an indirect enzyme-linked immunosorbent assay (iELISA. Using parallel interpretation, RBT and iELISA results showed that 31.7% (95% confidence interval (CI: 26.1, 36.3 of sheep and 34.0% (95% CI: 24.7, 44.3 of goats had antibodies against Brucella in the study area. A random-effects multivariable logistic regression model indicated that a higher chance of being seropositive (odds ratio (OR = 1.7; 95% 1.4; 2.2 was associated with an increase in the age of animals. The odds of Brucella seropositivity in flocks where sheep and goats grazed together was 2.0 times higher (95% CI: 1.08; 3.9 compared to flocks where sheep and goats grazed separately. The odds of Brucella seropositivity in small ruminants was 2.2 higher (95% CI: 1.2; 4.3 for animals originating from farms with a history of goat abortion in the preceding 12 months. In contrast, for every 1000 Iraqi Dinars (~0.85 US Dollar spent by the farmers on control of Brucella in their flocks, the odds of Brucella seropositivity decreased significantly (OR = 0.9, p-value = 0.021. The final model also indicated significant differences in Brucella seropositivity between the different districts of Duhok Province. This study provides a contribution to the epidemiology of brucellosis in small ruminants in northern Iraq.

  9. Development, validation and clinical assessment of a short questionnaire to assess disease-related knowledge in inflammatory bowel disease patients.

    Keegan, Denise

    2013-02-01

    Only two inflammatory bowel disease (IBD) knowledge scales are available, both primarily aimed at evaluating the effectiveness of clinical education programs. The aim of this study was to develop and validate a short knowledge questionnaire for clinical and academic research purposes.

  10. The association of neuroleptic sensitivity in Lewy body disease with a false positive clinical diagnosis of Creutzfeldt-Jakob disease

    Lemstra, A. W.; Schoenmaker, N.; Rozemuller-Kwakkel, A. J. M.; van Gool, W. A.

    2006-01-01

    BACKGROUND: Dementia with Lewy bodies (DLB) and Creutzfeldt-Jakob disease (CJD) share clinical features like cognitive decline, motor disturbances en psychiatric symptoms. Overlapping symptoms may cause physicians to mistake DLB for CJD. METHODS: Clinical data of 12 patients with autopsy-confirmed

  11. Advanced Methods for Clinical Outcome Prediction in Acquired Heart Disease

    L.C. Battes (Linda)

    2014-01-01

    markdownabstract__Abstract__ Acquired heart disease, which includes conditions such as coronary artery disease (CAD) and heart failure, continues to pose a large impediment on the individuals that suffer from it as well as on society in general. CAD is the leading cause of death in the

  12. Prions And Prion Diseases | Obi | African Journal of Clinical and ...

    Patients also may experience involuntary jerking movements called myoclonus, unusual sensation, insomnia, and confusion or memory problems. In the later stages of the disease, patients may have severe mental impairment (dementia) and may lose the ability to move or speak. Well known prion diseases include scrapie ...

  13. Clinical impact of exercise in patients with peripheral arterial disease.

    Novakovic, Marko; Jug, Borut; Lenasi, Helena

    2017-08-01

    Increasing prevalence, high morbidity and mortality, and decreased health-related quality of life are hallmarks of peripheral arterial disease. About one-third of peripheral arterial disease patients have intermittent claudication with deleterious effects on everyday activities, such as walking. Exercise training improves peripheral arterial disease symptoms and is recommended as first line therapy for peripheral arterial disease. This review examines the effects of exercise training beyond improvements in walking distance, namely on vascular function, parameters of inflammation, activated hemostasis and oxidative stress, and quality of life. Exercise training not only increases walking distance and physiologic parameters in patients with peripheral arterial disease, but also improves the cardiovascular risk profile by helping patients achieve better control of hypertension, hyperglycemia, obesity and dyslipidemia, thus further reducing cardiovascular risk and the prevalence of coexistent atherosclerotic diseases. American guidelines suggest supervised exercise training, performed for a minimum of 30-45 min, at least three times per week, for at least 12 weeks. Walking is the most studied exercise modality and its efficacy in improving cardiovascular parameters in patients with peripheral arterial disease has been extensively proven. As studies have shown that supervised exercise training improves walking performance, cardiovascular parameters and quality of life in patients with peripheral arterial disease, it should be encouraged and more often prescribed.

  14. Clinical radiodiagnosis of collagen diseases. Klinicheskaya rentgenodiagnostika kollagenozov

    Spasskaya, P A; Bartusevichene, A S

    1988-01-01

    General information about collagen diseases, short history of their theory development are presented; classification is discussed. Pathomorphology of connective tissue and vessels in these diseases is described. Clinicoroentgenologic diagnosis of internal organ and locomotor system injuries in systemic lupus erythemotosus, in sclerodermia systematica, in dermatomyositis in combination with morphological and clinicolabaratory data is analysed in detail. 166 refs.; 85 figs.

  15. Clinical evaluation of chemokine and enzymatic biomarkers of Gaucher disease

    Deegan, Patrick B.; Moran, Mary Teresa; McFarlane, Ian; Schofield, J. Paul; Boot, Rolf G.; Aerts, Johannes M. F. G.; Cox, Timothy M.

    2005-01-01

    Purpose: Gaucher disease is an exemplary orphan disorder. Enzyme replacement therapy with imiglucerase is effective, but very expensive. To improve the assessment of severity of disease and responses to this costly treatment, we have evaluated several enzymatic biomarkers and a newly-described

  16. Chronic kidney disease: sonographic/clinical findings at the ...

    Introduction: Kidney disease arises from various causes which can lead to death, especially if it progresses to chronic renal disease. Some of these patients can be managed by the use of conservative management, drugs, dialysis or renal transplantation depending on several factors. Amongst several investigative methods ...

  17. Clinical approach to kidney disease in kidney recipients in Spain

    Josep M. Campistol

    2015-05-01

    Conclusions: Secondary markers and factors resulting in CKD progression, particularly anemia, are still frequently uncontrolled after kidney transplantation. Only about 2% of patients benefit from a therapeutic intervention based on a biopsy. Clinical perception differs from objective measures, which results in an obvious clinical inertia regarding risk factor control in such patients.

  18. Clinical pharmacology in leishmaniasis: treatment optimization of a neglected disease

    Dorlo, T.P.C.

    2013-01-01

    This thesis presents various novel applications of clinical pharmacokinetics and pharmacodynamics in the treatment of leishmaniasis, by which diverse clinically relevant issues, mainly related to the efficacy and safety of miltefosine, could be elucidated. Throughout this thesis, the added value of

  19. Iron deficiency and neurologic disease in children | Chiabi | Clinics ...

    Iron deficiency is a frequent disorder and a public health problem especially in children and pregnant women. The clinical manifestations are varied, and the most dreaded are neurologic. These neurologic manifestations are often missed as differential diagnosis in current clinical practice. The authors review iron ...

  20. Clinical features of Parkinson’s disease with and without rapid eye movement sleep behavior disorder

    Liu, Ye; Zhu, Xiao-Ying; Zhang, Xiao-Jin; Kuo, Sheng-Han; Ondo, William G.; Wu, Yun-Cheng

    2017-01-01

    Background Rapid eye movement sleep behavior disorder (RBD) and Parkinson’s disease (PD) are two distinct clinical diseases but they share some common pathological and anatomical characteristics. This study aims to confirm the clinical features of RBD in Chinese PD patients. Methods One hundred fifty PD patients were enrolled from the Parkinson`s disease and Movement Disorders Center in  Department of Neurology, Shanghai General Hospital from January 2013 to August 2014. This study examined P...

  1. Canavan disease - unusual imaging features in a child with mild clinical presentation

    Nguyen, Ho V.; Ishak, Gisele E.

    2015-01-01

    Canavan disease is a rare hereditary leukodystrophy that manifests in early childhood. Associated with rapidly progressive clinical deterioration, it usually results in death by the third year of life. The predominant MRI appearance is diffuse and symmetrical white matter disease. We discuss an atypical, late presentation of Canavan disease with a benign clinical course and uncharacteristic imaging features. This case introduces a previously unreported pattern of diffuse cortical abnormality without significant white matter involvement. (orig.)

  2. Canavan disease - unusual imaging features in a child with mild clinical presentation

    Nguyen, Ho V.; Ishak, Gisele E. [University of Washington, Department of Radiology, Seattle Children' s Hospital, Seattle, WA (United States)

    2015-03-01

    Canavan disease is a rare hereditary leukodystrophy that manifests in early childhood. Associated with rapidly progressive clinical deterioration, it usually results in death by the third year of life. The predominant MRI appearance is diffuse and symmetrical white matter disease. We discuss an atypical, late presentation of Canavan disease with a benign clinical course and uncharacteristic imaging features. This case introduces a previously unreported pattern of diffuse cortical abnormality without significant white matter involvement. (orig.)

  3. The behavioural/dysexecutive variant of Alzheimer's disease: clinical, neuroimaging and pathological features

    Ossenkoppele, R.; Pijnenburg, Y.A.L.; Perry, D.C.; Cohn-Sheehy, B.I.; Scheltens, N.M.E.; Vogel, J.W.; Kramer, J.H.; van der Vlies, A.E.; La Joie, R.; Rosen, H.J.; van der Flier, W.M.; Grinberg, L.T.; Rozemuller, A.J.M.; Huang, E.J.; van Berckel, B.N.M.; Miller, B.L.; Barkhof, F.; Jagust, W.J.; Scheltens, P.; Seeley, W.W.; Rabinovici, G.D.

    2015-01-01

    A 'frontal variant of Alzheimer's disease' has been described in patients with predominant behavioural or dysexecutive deficits caused by Alzheimer's disease pathology. The description of this rare Alzheimer's disease phenotype has been limited to case reports and small series, and many clinical,

  4. EDITORIAL Clinical issues in genetic testing for multifactorial diseases

    Given the importance of MDs, detection of genetic predisposition is potentially attractive ... burden for affected individuals and families: disease is often early onset and ... to detection of BRCA-related breast cancer in a local public health sector.

  5. Sternoclavicular Arthritis as a Clinical Presentation for Lyme Disease.

    Ramgopal, Sriram; Rosenkranz, Margalit; Nowalk, Andrew J; Zuckerbraun, Noel S

    2018-04-01

    Lyme disease is caused by Borrelia burgdorferi and can lead to dermatologic, neurologic, cardiac, and musculoskeletal manifestations. The arthritis of Lyme disease is typically monoarticular, with the knee being most commonly involved. Lyme arthritis of small joints has not previously been well described. We report 3 children who presented with sternoclavicular joint swelling and who were found to have Lyme disease based on enzyme-linked immunosorbent assay and Western blot. This description of sternoclavicular Lyme arthritis highlights the importance of considering Lyme disease in the differential and diagnostic workup of new onset, small joint arthritis in patients presenting from or with travel to Lyme endemic regions. Copyright © 2018 by the American Academy of Pediatrics.

  6. The treatment of Alzheimer's disease using Chinese medicinal plants: from disease models to potential clinical applications.

    Su, Yang; Wang, Qiuhong; Wang, Changfu; Chan, Kelvin; Sun, Yanping; Kuang, Haixue

    2014-03-28

    Alzheimer's disease (AD) is characterized by the sustained higher nervous disorders of the activities and functions of the brain. Due to its heavy burden on society and the patients' families, it is urgent to review the treatments for AD to provide basic data for further research and new drug development. Among these treatments, Chinese Material Medica (CMM) has been traditionally clinical used in China to treat AD for a long time with obvious efficacy. With the further research reports of CMM, new therapeutic materials may be recovered from troves of CMM. However, So far, little or no review work has been reported to conclude anti-AD drugs from CMM in literature. Therefore, a systematic introduction of CMM anti-AD research progress is of great importance and necessity. This paper strives to systematically describe the progress of CMM in the treatment of AD, and lays a basis data for anti-AD drug development from CMM, and provides the essential theoretical support for the further development and utilization of CMM resources through a more comprehensive research of the variety of databases regarding CMM anti-AD effects reports. Literature survey was performed via electronic search (SciFinder®, Pubmed®, Google Scholar and Web of Science) on papers and patents and by systematic research in ethnopharmacological literature at various university libraries. This review mainly introduces the current research on the Chinese Material Medica (CMM) theoretical research on Alzheimer's disease (AD), anti-AD active constituent of CMM, anti-AD effects on AD models, anti-AD mechanism of CMM, and anti-AD effect of CMM formula. Scholars around the world have made studies on the anti-AD molecular mechanism of CMM from different pathways, and have made substantial progress. The progress not only enriched the anti-AD theory of CMM, but also provided clinical practical significance and development prospects in using CMM to treat AD. Western pure drugs cannot replace the advantages of

  7. Inherited Retinal Degenerative Disease Clinical Trial Network. Addendum

    2010-10-01

    Stargardt disease, and Usher syndrome represent the predominant forms of inherited orphan retinal degenerative diseases and are estimated to affect...working with Oxford Biomedica and a separate project with academic investigators on gene therapy for Usher lb syndrome (deaf-blindness due to a gene...s. The NEER Network will also develop standard protocols for data collection, mainta i n and expand patient databases, classified by genotype and

  8. Measures of Rheumatoid Arthritis Disease Activity in Australian Clinical Practice

    Taylor, Andrew; Bagga, Hanish

    2011-01-01

    Objectives. To investigate which rheumatoid arthritis (RA) disease activity measures are being collected in patients receiving glucocorticoids, non-biologic or biologic disease-modifying antirheumatic drugs (DMARDs) in Australian rheumatology practice. Methods. A retrospective audit of medical records was conducted from eight rheumatology practices around Australia. Each rheumatologist recruited 30 consecutive eligible patients into the review, 10 of whom must have been receiving a biological...

  9. A Review of Gaucher Disease Pathophysiology, Clinical Presentation and Treatments.

    Stirnemann, Jérôme; Belmatoug, Nadia; Camou, Fabrice; Serratrice, Christine; Froissart, Roseline; Caillaud, Catherine; Levade, Thierry; Astudillo, Leonardo; Serratrice, Jacques; Brassier, Anaïs; Rose, Christian; Billette de Villemeur, Thierry; Berger, Marc G

    2017-02-17

    Gaucher disease (GD, ORPHA355) is a rare, autosomal recessive genetic disorder. It is caused by a deficiency of the lysosomal enzyme, glucocerebrosidase, which leads to an accumulation of its substrate, glucosylceramide, in macrophages. In the general population, its incidence is approximately 1/40,000 to 1/60,000 births, rising to 1/800 in Ashkenazi Jews. The main cause of the cytopenia, splenomegaly, hepatomegaly, and bone lesions associated with the disease is considered to be the infiltration of the bone marrow, spleen, and liver by Gaucher cells. Type-1 Gaucher disease, which affects the majority of patients (90% in Europe and USA, but less in other regions), is characterized by effects on the viscera, whereas types 2 and 3 are also associated with neurological impairment, either severe in type 2 or variable in type 3. A diagnosis of GD can be confirmed by demonstrating the deficiency of acid glucocerebrosidase activity in leukocytes. Mutations in the GBA1 gene should be identified as they may be of prognostic value in some cases. Patients with type-1 GD-but also carriers of GBA1 mutation-have been found to be predisposed to developing Parkinson's disease, and the risk of neoplasia associated with the disease is still subject to discussion. Disease-specific treatment consists of intravenous enzyme replacement therapy (ERT) using one of the currently available molecules (imiglucerase, velaglucerase, or taliglucerase). Orally administered inhibitors of glucosylceramide biosynthesis can also be used (miglustat or eliglustat).

  10. A Review of Gaucher Disease Pathophysiology, Clinical Presentation and Treatments

    Jérôme Stirnemann

    2017-02-01

    Full Text Available Gaucher disease (GD, ORPHA355 is a rare, autosomal recessive genetic disorder. It is caused by a deficiency of the lysosomal enzyme, glucocerebrosidase, which leads to an accumulation of its substrate, glucosylceramide, in macrophages. In the general population, its incidence is approximately 1/40,000 to 1/60,000 births, rising to 1/800 in Ashkenazi Jews. The main cause of the cytopenia, splenomegaly, hepatomegaly, and bone lesions associated with the disease is considered to be the infiltration of the bone marrow, spleen, and liver by Gaucher cells. Type-1 Gaucher disease, which affects the majority of patients (90% in Europe and USA, but less in other regions, is characterized by effects on the viscera, whereas types 2 and 3 are also associated with neurological impairment, either severe in type 2 or variable in type 3. A diagnosis of GD can be confirmed by demonstrating the deficiency of acid glucocerebrosidase activity in leukocytes. Mutations in the GBA1 gene should be identified as they may be of prognostic value in some cases. Patients with type-1 GD—but also carriers of GBA1 mutation—have been found to be predisposed to developing Parkinson’s disease, and the risk of neoplasia associated with the disease is still subject to discussion. Disease-specific treatment consists of intravenous enzyme replacement therapy (ERT using one of the currently available molecules (imiglucerase, velaglucerase, or taliglucerase. Orally administered inhibitors of glucosylceramide biosynthesis can also be used (miglustat or eliglustat.

  11. The clinical management of Type 2 Gaucher disease.

    Weiss, Karin; Gonzalez, Ashley; Lopez, Grisel; Pedoeim, Leah; Groden, Catherine; Sidransky, Ellen

    2015-02-01

    Gaucher disease, the inherited deficiency of the enzyme glucocerebrosidase, is the most common of the lysosomal storage disorders. Type 2 Gaucher disease, the most severe and progressive form, manifests either prenatally or in the first months of life, followed by death within the first years of life. The rarity of the many lysosomal storage disorders makes their diagnosis a challenge, especially in the newborn period when the focus is often on more prevalent illnesses. Thus, a heightened awareness of the presentation of these rare diseases is necessary to ensure their timely consideration. This review, designed to serve as a guide to physicians treating newborns and infants with Gaucher disease, discusses the presenting manifestations of Type 2 Gaucher disease, the diagnostic work-up, associated genotypes and suggestions for management. We also address the ethical concerns that may arise with this progressive and lethal disorder, since currently available treatments may prolong life, but do not impact the neurological manifestations of the disease. Published by Elsevier Inc.

  12. The ADAS-cog and clinically meaningful change in the VISTA clinical trial of galantamine for Alzheimer's disease.

    Rockwood, Kenneth; Fay, Sherri; Gorman, Mary

    2010-02-01

    A minimum 4-point change at 6 months on the Alzheimer's disease assessment scale-cognitive subscale (ADAS-cog) is deemed clinically important, but this cut-point has been little studied in relation to clinical meaningfulness. In an investigator-initiated, clinical trial of galantamine, we investigated the extent to which a 4-point change classifies goal attainment by individual patients. Secondary analysis of the video imaging synthesis of treating Alzheimer's disease (VISTA) study: a 4-month, multi-centre, parallel-group, double-blind, placebo-controlled, trial of galantamine in 130 mild-moderate Alzheimer's disease patients (4-month open-label follow-up). ADAS-cog responses at 6 months were compared with outcomes on three clinical measures: clinician's interview based impression of change-plus caregiver input (CIBIC+), patient/carer-goal attainment scaling (PGAS) and clinician-GAS (CGAS). Thirty-seven of 99 patients improved by > or = 4 points on the ADAS-cog at 6 months, and 16/99 showed > or = 4-point worsening. ADAS-cog change scores correlated notionally to modestly with changes on the CGAS (r = -0.31), the PGAS (r = -0.29) and the CIBIC+ (r = 0.31). As a group, patients with ADAS-cog improvement were significantly more likely to improve on the clinical measures; those who worsened showed non-significant clinical decline. Individually, about half were misclassified in relation to each clinical measure; often when the ADAS-Cog detected 'no change', clinically meaningful effects could be detected. Even so, no ADAS-Cog cut-point optimally classified patients' clinical responses. A 4-point ADAS-cog change at 6 months is clinically meaningful for groups. Substantial individual misclassification between the ADAS-cog and clinical measures suggests no inherent meaning to a 4-point ADAS-cog change for a given patient.

  13. Implementation and evaluation of Parkinson disease management in an outpatient clinical pharmacist-run neurology telephone clinic.

    Stefan, Teodora Cristina; Elharar, Nicole; Garcia, Guadalupe

    2018-05-01

    Parkinson disease (PD) is a progressive, debilitating neurodegenerative disease that often requires complex pharmacologic treatment regimens. Prior to this clinic, there was no involvement of a clinical pharmacy specialist (CPS) in the outpatient neurology clinic at the West Palm Beach Veterans Affairs Medical Center. This was a prospective, quality-improvement project to develop a clinical pharmacist-run neurology telephone clinic and evaluate pharmacologic and nonpharmacologic interventions in an effort to improve the quality of care for patients with PD. Additionally, the CPS conducted medication education groups to 24 patients with PD and their caregivers, if applicable, at this medical center with the purpose of promoting patient knowledge and medication awareness. Medication management was performed via telephone rather than face to face. Only patients with a concomitant mental health diagnosis for which they were receiving at least one psychotropic medication were included for individual visits due to the established scope of practice of the CPS being limited to mental health and primary care medications. Data collection included patient and clinic demographics as well as pharmacologic and nonpharmacologic interventions made for patients enrolled from January 6, 2017, through March 31, 2017. A total of 49 pharmacologic and nonpharmacologic interventions were made for 10 patients. We successfully implemented and evaluated a clinical pharmacist-run neurology telephone clinic for patients with PD. Expansion of this clinic to patients with various neurological disorders may improve access to care using an innovative method of medication management expertise by a CPS.

  14. Clinical trials of new drugs for the treatment of rheumatoid arthritis: focus on early disease

    Smolen, Josef S.; Collaud Basset, Sabine; Boers, Maarten; Breedveld, Ferdinand; Edwards, Christopher J.; Kvien, Tore K.; Miossec, Pierre; Sokka-Isler, Tuulikki; van Vollenhoven, Ronald F.; Abadie, Eric C.; Bruyère, Olivier; Cooper, Cyrus; Mäkinen, Heidi; Thomas, Thierry; Tugwell, Peter; Reginster, Jean-Yves

    2016-01-01

    The European Society for Clinical and Economic Aspects of Osteoporosis, Osteoarthritis and Musculoskeletal Diseases convened a task force of experts in rheumatoid arthritis (RA) and clinical trial methodology to comment on the new draft 'Guideline on clinical investigation of medicinal products for

  15. Clinical value of natriuretic peptides in chronic kidney disease

    Carla Santos-Araújo

    2015-05-01

    This review describes the role of NP in the regulatory response to renal function loss and addresses the main factors involved in the clinical valorization of the peptides in the context of significant renal failure.

  16. Clinical profile of cyanotic congenital heart disease in neonatal ...

    Ashraf Abou-Taleb

    2016-02-01

    Feb 1, 2016 ... rate was 100% in cases of pulmonary atresia and hypoplastic left heart syndrome ..... [10] Patton C, Hey E. How effectively can clinical examination pick up .... [33] Jacobs EGJ, Leung MP, Karlberg J. Distribution of symptomatic.

  17. Ebola Virus Diseases | Elisha | African Journal of Clinical and ...

    African Journal of Clinical and Experimental Microbiology. Journal Home · ABOUT THIS JOURNAL · Advanced Search · Current Issue · Archives · Journal Home > Vol 15, No 3 (2014) >. Log in or Register to get access to full text downloads.

  18. Kawasaki disease in Sicily: clinical description and markers of disease severity.

    Maggio, Maria Cristina; Corsello, Giovanni; Prinzi, Eugenia; Cimaz, Rolando

    2016-11-02

    Kawasaki disease (KD) is an acute systemic vasculitis of small and middle size arteries; 15-25 % of untreated patients and 5 % of patients treated with intravenous immunoglobulin (IVIG) develop coronary artery lesions (CAL). Many studies tried to find the most effective treatment in the management of resistant KD and to select the risk factors for CAL. Our data are assessed on children from west Sicily, characterized by a genetic heterogeneity. We studied the clinical data of 70 KD Sicilian children (36 males: 51 %; 34 females: 49 %), analysed retrospectively, including: demographic and laboratory parameters; echocardiographic findings at diagnosis, at 2, 6 and 8 weeks, and at 1 year after the onset of the illness. Forty-seven had Typical KD, three Atypical KD and twenty Incomplete KD. Age at the disease onset ranged from 0.1 to 8.9 years. IVIG were administered 5 ± 2 days after the fever started. Defervescence occurred 39 ± 26 hours after the first IVIG infusion. Fifty-six patients (80 %) received 1 dose of IVIG (responders); 14 patients (20 %) had a resistant KD, with persistent fever after the first IVIG dose (non responders). Ten (14 %) non responders responded to the second dose, 4 (5 %) responded to three doses; one needed treatment with high doses of steroids and Infliximab. Cardiac involvement was documented in twenty-two cases (eighteen with transient dilatation/ectasia, fifteen with aneurysms). Pericardial effusion, documented in eleven, was associated with coronaritis and aneurysms, and was present earlier than coronary involvement in seven. Hypoalbuminemia, D-dimer pre-IVIG, gamma-GT pre-IVIG showed a statistically significant direct correlation with IVIG doses, highlighting the role of these parameters as predictor markers of refractory disease. The persistence of elevated CRP, AST, ALT levels, a persistent hyponatremia and hypoalbuminemia after IVIG therapy, also had a statistical significant correlation with IVIG doses. Non responders

  19. Analysis of Published Criteria for Clinically Inactive Disease in a Large Juvenile Dermatomyositis Cohort Shows That Skin Disease Is Underestimated

    Almeida, Beverley; Campanilho‐Marques, Raquel; Arnold, Katie; Pilkington, Clarissa A.; Wedderburn, Lucy R.; Armon, Kate; Briggs, Vanja; Ellis‐Gage, Joe; Roper, Holly; Watts, Joanna; Baildam, Eileen; Hanna, Louise; Lloyd, Olivia; McCann, Liza; Roberts, Ian; McGovern, Ann; Riley, Phil; Al‐Abadi, Eslam; Ryder, Clive; Scott, Janis; Southwood, Taunton; Thomas, Beverley; Amin, Tania; Burton, Deborah; Jackson, Gillian; Van Rooyen, Vanessa; Wood, Mark; Wyatt, Sue; Browne, Michael; Davidson, Joyce; Ferguson, Sue; Gardner‐Medwin, Janet; Martin, Neil; Waxman, Liz; Foster, Helen; Friswell, Mark; Jandial, Sharmila; Qiao, Lisa; Sen, Ethan; Smith, Eve; Stevenson, Vicky; Swift, Alison; Wade, Debbie; Watson, Stuart; Crate, Lindsay; Frost, Anna; Jordan, Mary; Mosley, Ellen; Satyapal, Rangaraj; Stretton, Elizabeth; Venning, Helen; Warrier, Kishore; Almeida, Beverley; Arnold, Katie; Beard, Laura; Brown, Virginia; Campanilho‐Marques, Raquel; Enayat, Elli; Glackin, Yvonne; Halkon, Elizabeth; Hasson, Nathan; Juggins, Audrey; Kassoumeri, Laura; Lunt, Sian; Maillard, Sue; Nistala, Kiran; Pilkington, Clarissa; Simou, Stephanie; Smith, Sally; Varsani, Hemlata; Wedderburn, Lucy; Murray, Kevin; Ioannou, John; Suffield, Linda; Al‐Obaidi, Muthana; Leach, Sam; Lee, Helen; Smith, Helen; Inness, Emma; Kendall, Eunice; Mayers, David; Wilkinson, Nick; Clinch, Jacqui; Pluess‐Hall, Helen

    2015-01-01

    Objective The Pediatric Rheumatology International Trials Organisation (PRINTO) recently published criteria for classification of patients with juvenile dermatomyositis (DM) as having clinically inactive disease. The criteria require that at least 3 of 4 conditions be met, i.e., creatine kinase level ≤150 units/liter, Childhood Myositis Assessment Scale score ≥48, Manual Muscle Testing in 8 muscles score ≥78, and physician's global assessment of overall disease activity (PGA) ≤0.2. The present study was undertaken to test these criteria in a UK cohort of patients with juvenile DM. Methods We assessed 1,114 patient visits for the 4 items in the PRINTO criteria for clinically inactive disease. Each visit was analyzed to determine whether skin disease was present. The Disease Activity Score (DAS) for juvenile DM was determined in 59 patients. Results At 307 of the 1,114 visits, clinically inactive disease was achieved based on the 3 muscle criteria (but with a PGA of >0.2); rash was present at 65.8% of these visits and nailfold capillary abnormalities at 35.2%. When PGA ≤0.2 was one of the 3 criteria that were met, the frequency of skin signs was significantly lower (rash in 23.1% and nailfold capillary abnormalities in 8.7%). If PGA was considered an essential criterion for clinically inactive disease (P‐CID), patients with active skin disease were less likely to be categorized as having clinically inactive disease (a median DAS skin score of 0 [of a possible maximum of 9] in visits where the PGA was ≤0.2, versus a median DAS skin score of 4 in patients meeting the 3 muscle criteria [with a PGA of >0.2]; P < 0.001). Use of the P‐CID led to improvements in the positive predictive value and the positive likelihood ratio (85.4% and 11.0, respectively, compared to 72.9% and 5.1 with the current criteria). Conclusion There was a high frequency of skin disease among patients with juvenile DM who did not meet the PGA criterion for inactive disease but met

  20. Harnessing Cerebrospinal Fluid Biomarkers in Clinical Trials for Treating Alzheimer's and Parkinson's Diseases: Potential and Challenges.

    Kim, Dana; Kim, Young Sam; Shin, Dong Wun; Park, Chang Shin; Kang, Ju Hee

    2016-10-01

    No disease-modifying therapies (DMT) for neurodegenerative diseases (NDs) have been established, particularly for Alzheimer's disease (AD) and Parkinson's disease (PD). It is unclear why candidate drugs that successfully demonstrate therapeutic effects in animal models fail to show disease-modifying effects in clinical trials. To overcome this hurdle, patients with homogeneous pathologies should be detected as early as possible. The early detection of AD patients using sufficiently tested biomarkers could demonstrate the potential usefulness of combining biomarkers with clinical measures as a diagnostic tool. Cerebrospinal fluid (CSF) biomarkers for NDs are being incorporated in clinical trials designed with the aim of detecting patients earlier, evaluating target engagement, collecting homogeneous patients, facilitating prevention trials, and testing the potential of surrogate markers relative to clinical measures. In this review we summarize the latest information on CSF biomarkers in NDs, particularly AD and PD, and their use in clinical trials. The large number of issues related to CSF biomarker measurements and applications has resulted in relatively few clinical trials on CSF biomarkers being conducted. However, the available CSF biomarker data obtained in clinical trials support the advantages of incorporating CSF biomarkers in clinical trials, even though the data have mostly been obtained in AD trials. We describe the current issues with and ongoing efforts for the use of CSF biomarkers in clinical trials and the plans to harness CSF biomarkers for the development of DMT and clinical routines. This effort requires nationwide, global, and multidisciplinary efforts in academia, industry, and regulatory agencies to facilitate a new era.

  1. Clinical and radiological correlations in patients with gestational trophoblastic disease

    Lana de Lourdes Aguiar Lima

    Full Text Available Abstract Gestational trophoblastic disease is an abnormality of pregnancy that encompasses a group of diseases that differ from each other in their propensity for regression, invasion, metastasis, and recurrence. In the past, it was common for patients with molar pregnancy to present with marked symptoms: copious bleeding; theca lutein cysts; uterus larger than appropriate for gestational age; early preeclampsia; hyperemesis gravidarum; and hyperthyroidism. Currently, with early diagnosis made by ultrasound, most patients are diagnosed while the disease is still in the asymptomatic phase. In cases of progression to trophoblastic neoplasia, staging-typically with Doppler flow studies of the pelvis and chest X-ray, although occasionally with computed tomography or magnetic resonance imaging-is critical to the choice of an appropriate antineoplastic therapy regimen. Because it is an unusual and serious disease that affects women of reproductive age, as well as because its appropriate treatment results in high cure rates, it is crucial that radiologists be familiar with gestational trophoblastic disease, in order to facilitate its early diagnosis and to ensure appropriate follow-up imaging.

  2. Clinical and radiological correlations in patients with gestational trophoblastic disease

    Lima, Lana de Lourdes Aguiar; Parente, Raphael Camara Medeiros; Amim Junior, Joffre; Rezende Filho, Jorge Fonte de; Montenegro, Carlos Antonio Barbosa; Braga, Antonio; Maesta, Izildinha

    2016-01-01

    Gestational trophoblastic disease is an abnormality of pregnancy that encompasses a group of diseases that differ from each other in their propensity for regression, invasion, metastasis, and recurrence. In the past, it was common for patients with molar pregnancy to present with marked symptoms: copious bleeding; theca lutein cysts; uterus larger than appropriate for gestational age; early preeclampsia; hyperemesis gravidarum; and hyperthyroidism. Currently, with early diagnosis made by ultrasound, most patients are diagnosed while the disease is still in the asymptomatic phase. In cases of progression to trophoblastic neoplasia, staging-typically with Doppler flow studies of the pelvis and chest X-ray, although occasionally with computed tomography or magnetic resonance imaging-is critical to the choice of an appropriate antineoplastic therapy regimen. Because it is an unusual and serious disease that affects women of reproductive age, as well as because its appropriate treatment results in high cure rates, it is crucial that radiologists be familiar with gestational trophoblastic disease, in order to facilitate its early diagnosis and to ensure appropriate follow-up imaging. (author)

  3. Clinical and radiological correlations in patients with gestational trophoblastic disease

    Lima, Lana de Lourdes Aguiar; Parente, Raphael Camara Medeiros; Amim Junior, Joffre; Rezende Filho, Jorge Fonte de; Montenegro, Carlos Antonio Barbosa; Braga, Antonio, E-mail: lanalima@hotmail.com [Universidade Federal do Rio de Janeiro (UFRJ), RJ (Brazil); Maesta, Izildinha [Universidade Estadual Paulista Julio de Mesquita Filho (UNESP), Botucatu, SP (Brazil). Faculdade de Medicina

    2016-07-15

    Gestational trophoblastic disease is an abnormality of pregnancy that encompasses a group of diseases that differ from each other in their propensity for regression, invasion, metastasis, and recurrence. In the past, it was common for patients with molar pregnancy to present with marked symptoms: copious bleeding; theca lutein cysts; uterus larger than appropriate for gestational age; early preeclampsia; hyperemesis gravidarum; and hyperthyroidism. Currently, with early diagnosis made by ultrasound, most patients are diagnosed while the disease is still in the asymptomatic phase. In cases of progression to trophoblastic neoplasia, staging-typically with Doppler flow studies of the pelvis and chest X-ray, although occasionally with computed tomography or magnetic resonance imaging-is critical to the choice of an appropriate antineoplastic therapy regimen. Because it is an unusual and serious disease that affects women of reproductive age, as well as because its appropriate treatment results in high cure rates, it is crucial that radiologists be familiar with gestational trophoblastic disease, in order to facilitate its early diagnosis and to ensure appropriate follow-up imaging. (author)

  4. Alzheimer's Disease: Lessons Learned from Amyloidocentric Clinical Trials.

    Soejitno, Andreas; Tjan, Anastasia; Purwata, Thomas Eko

    2015-06-01

    Alzheimer's disease (AD) is one of the most debilitating neurodegenerative diseases and is predicted to affect 1 in 85 people by 2050. Despite much effort to discover a therapeutic strategy to prevent progression or to cure AD, to date no effective disease-modifying agent is available that can prevent, halt, or reverse the cognitive and functional decline of patients with AD. Several underlying etiologies to this failure are proposed. First, accumulating evidence from past trials suggests a preventive as opposed to therapeutic paradigm, and the precise temporal and mechanistic relationship of β-amyloid (Aβ) and tau protein should be elucidated to confirm this hypothesis. Second, we are in urgent need of revised diagnostic criteria to support future trials. Third, various technical and methodological improvements are required, based on the lessons learned from previous failed trials.

  5. Clinical implications of the microbiome in urinary tract diseases.

    Hiergeist, Andreas; Gessner, André

    2017-03-01

    The purpose of this review is to outline and evaluate the most recent literature on the role of the microbiome in urinary tract diseases. High throughput molecular DNA sequencing of bacterial 16S rRNA genes enabled the analysis of complex microbial communities inhabiting the human urinary tract. Several recent studies have identified bacterial taxa of the urinary microbiome to impact urinary tract diseases including interstitial cystitis, urgency urinary incontinence or calcium oxalate stone formation. Furthermore, treatment of urinary tract infections by antibiotics globally impacts community profiles of the intestinal microbiota and might indirectly influence human health. Alternative treatment options like application of probiotics for the treatment of urinary tract infections are currently under investigation. The urinary microbiome and its relationship to urinary tract diseases is currently under comprehensive investigation. Further studies are needed to shed light on the role of commensal microbiota for urinary tract infections.

  6. Syphilis sero-positivity in recently admitted and long-term psychiatric inpatients: Screening, prevalence and diagnostic profile

    Maria P Henning

    2012-12-01

    Full Text Available Background. Syphilis research has neglected the prevalence of the disease among psychiatric patients, and traditional syphilis screening has been reported as inadequate. Objectives. (i To assess the syphilis prevalence among psychiatric patients; (ii to compare psychiatric diagnoses of syphilis-infected and -uninfected patients; (iii to assess self-reported high-risk sexual behaviour; (iv to establish syphilis/HIV co-morbidity; and (v to investigate the performance of the rapid plasma reagin (RPR test in syphilis screening, compared with the Treponema pallidum haemagglutination (TPHA test. Methods. Psychiatric inpatients at Weskoppies Hospital, Pretoria, who consented to participate in the study (N=195 were categorised according to gender and length of admission (long-term or recent. Non-treponemal RPR, confirmatory TPHA, HIV-rapid and HIV enzyme-linked immunosorbent assay (ELISA tests were performed. A reactive TPHA test was used to diagnose syphilis. Results. The estimated prevalence of syphilis was 11.7%. There was no significant association between TPHA sero-positivity and primary psychiatric diagnosis or self-reported high-risk sexual behaviour. Significant co-morbidity existed between syphilis and HIV (p=0.012. Compared with the TPHA test, the RPR test performed poorly, identifying only 2/23 patients who had a sero-positive TPHA test (8.7% sensitivity and 100% specificity. Conclusions. The prevalence of syphilis was higher than anticipated, supporting the need for routine testing. The significant co-morbidity and alarming prevalence of HIV and syphilis warrant testing for both conditions in all psychiatric admissions. Current syphilis screening with a single RPR test is inadequate; both RPR and TPHA tests should be performed.

  7. Association of seropositivity for influenza and coronaviruses with history of mood disorders and suicide attempts.

    Okusaga, Olaoluwa; Yolken, Robert H; Langenberg, Patricia; Lapidus, Manana; Arling, Timothy A; Dickerson, Faith B; Scrandis, Debra A; Severance, Emily; Cabassa, Johanna A; Balis, Theodora; Postolache, Teodor T

    2011-04-01

    Anecdotal reports of mood disorder following infection with common respiratory viruses with neurotropic potential have been in existence since the last century. Nevertheless, systematic studies on the association between these viruses and mood disorders are lacking. Influenza A, B and coronavirus antibody titers were measured in 257 subjects with recurrent unipolar and bipolar disorder and healthy controls, by SCID. Pearson's χ² tests and logistic regression models were used to analyze associations between seropositivity for coronaviruses, influenza A and B viruses and the following: a) history of recurrent mood disorders b) having attempted suicide in the past c) uni- vs. bi-polarity and d) presence of psychotic symptoms during mood episodes. Seropositivity for influenza A (p=0.004), B (pmood disorders but not with the specific diagnosis of unipolar or bipolar depression. Seropositivity for influenza B was significantly associated with a history of suicide attempt (p=0.001) and history of psychotic symptoms (p=0.005). The design was cross-sectional. Socioeconomic factors, inflammatory markers, and axis II psychopathology were not assessed. The association of seropositivity for influenza and coronaviruses with a history of mood disorders, and influenza B with suicidal behavior require replication in larger longitudinal samples. The need for these studies is additionally supported by the high incidence of these viral infections, the high prevalence of mood disorders, and resilience of suicide epidemics. Copyright © 2010 Elsevier B.V. All rights reserved.

  8. HTLV-I/II and blood donors: determinants associated with seropositivity in a low risk population

    Bernadette Catalan Soares

    2003-08-01

    Full Text Available OBJECTIVE: Blood donors in Brazil have been routinely screened for HTLV-I/II since 1993. A study was performed to estimate the prevalence of HTLV-I/II infection in a low risk population and to better understand determinants associated with seropositivity. METHODS: HTLV-I/II seropositive (n=135, indeterminate (n=167 and seronegative blood donors (n=116 were enrolled in an open prevalence prospective cohort study. A cross-sectional epidemiological study of positive, indeterminate and seronegative HTLV-I/II subjects was conducted to assess behavioral and environmental risk factors for seropositivity. HTLV-I/II serological status was confirmed using enzyme-linked immunosorbent assay (EIA and Western blot (WB. RESULTS: The three groups were not homogeneous. HTLV-I/II seropositivity was associated to past blood transfusion and years of schooling, a marker of socioeconomic status, and use of non-intravenous illegal drugs. CONCLUSIONS: The study results reinforce the importance of continuous monitoring and improvement of blood donor selection process.

  9. Serotonin modulates immune function in T cells from HIV-seropositive subjects

    Eugen-Olsen, J; Afzelius, P; Andresen, L

    1997-01-01

    We have shown earlier increased intracellular levels of cAMP in peripheral lymphocytes from HIV-seropositive subjects and that a chemically induced decrease in this level increases cell proliferation and cytotoxicity. Others have shown that serotonin indirectly decreases intracellular cAMP levels...

  10. Prevention for HIV-Seropositive Persons: Successive Approximation Toward a New Identity

    Lightfoot, Marguerita; Rotheram-Borus, Mary Jane; Milburn, Norweeta G.; Swendeman, Dallas

    2005-01-01

    This article presents a three-module intervention based on social action theory that focuses on health promotion and social identity formation for seropositive youth. The modules are designed to reduce transmission of HIV by reducing sexual and substance abuse acts, increasing healthy acts and adherence to care, and maintaining positive behavioral…

  11. Individual and environmental risk factors for dengue and chikungunya seropositivity in North-Eastern Tanzania

    Kajeguka, Debora C.; Msonga, Maulid; Schiøler, Karin L.

    2017-01-01

    was associated with G6PD status (p = 0.03) while there was no apparent association between genetic factors (G6PD, HbB or alpha-thalassemia) and chikungunya seropositivity. Conclusion: Public health education on environmental management practices is needed to eliminate the identified risks such as simple removal...

  12. CMV seropositivity determines epoetin dose and hemoglobin levels in patients with CKD

    M.G.H. Betjes (Michiel); W. Weimar (Willem); N.H.R. Litjens (Nicolle)

    2009-01-01

    textabstractCytomegalovirus (CMV)-seropositive patients with ESRD may have more CD4+T cells lacking the co-stimulatory molecule CD28 (CD4+CD28null) than CMV-seronegative patients. Increased numbers of CD28null T cells associates with epoetin nonresponsiveness in patients with ESRD, but whether

  13. Staphylococcus aureus nasal colonization in HIV-seropositive and HIV-seronegative drug users

    K.A. Holbrook; R.S. Klein; D. Hartel; D.A. Elliott; T. B. Barsky; L. H. Rothschild; F. D. Lowy

    1997-01-01

    textabstractNasal colonization plays an important role in the pathogenesis of Staphylococcus aureus infections. To identify characteristics associated with colonization, we studied a cross-section of a well-described cohort of HIV-seropositive and -seronegative active and former drug users

  14. Clinical course of Crohn's disease first diagnosed at surgery for acute abdomen.

    Latella, G; Cocco, A; Angelucci, E; Viscido, A; Bacci, S; Necozione, S; Caprilli, R

    2009-04-01

    The severity of clinical activity of Crohn's disease is high during the first year after diagnosis and decreases thereafter. Approximately 50% of patients require steroids and immunosuppressants and 75% need surgery during their lifetime. The clinical course of patients with Crohn's disease first diagnosed at surgery has never been investigated. To assess the clinical course of Crohn's disease first diagnosed at surgery for acute abdomen and to evaluate the need for medical and surgical treatment in this subset of patients. Hospital clinical records of 490 consecutive Crohn's disease patients were reviewed. Patients were classified according to the Vienna criteria. Sex, extraintestinal manifestations, family history of inflammatory bowel diseases, appendectomy, smoking habit and medical/surgical treatments performed during the follow-up period were assessed. Kaplan-Meier survival method and Cox proportional hazards regression model. Of the 490 Crohn's disease patients, 115 had diagnosis of Crohn's disease at surgery for acute abdomen (Group A) and 375 by conventional clinical, radiological, endoscopic and histologic criteria (Group B). Patients in Group A showed a low risk of further surgery (Log Rank test pacute abdomen showed a low risk for reintervention and less use of steroids and immunosuppressants during follow-up than those not operated upon at diagnosis. Early surgery may represent a valid approach in the initial management of patients with Crohn's disease, at least in the subset of patients with ileal and complicated disease.

  15. Influence of sociodemographic and clinical characteristics at the impact of valvular heart disease.

    dos Anjos, Daniela Brianne Martins; Rodrigues, Roberta Cunha Matheus; Padilha, Kátia Melissa; Pedrosa, Rafaela Batista dos Santos; Gallani, Maria Cecília Bueno Jayme

    2016-01-01

    to analyze the sociodemographic and clinical characteristics of patients with valvular heart disease and to verify the influence of these variables on the impact of valve disease in daily life. the study involved 86 outpatients. Data collection was performed in two stages - face-to-face interview for sociodemographic and clinical characterization and through telephone contact for the application of the Instrument to Measure the Impact of Valvular Heart Disease on Patient's Everyday Life (IDCV). Data were analyzed through descriptive statistics and multiple regression analysis. it was noticed that the total score of IDCV and its domains were influenced by age, schooling, presence or absence of symptoms, use or not of diuretic. The impact of the disease was influenced by sociodemographic and clinical variables. The results provide subsidies for the design of nursing interventions aimed at reducing the impact of the disease on the patient's daily life with valve disease.

  16. Early-onset Coronary Artery Disease: Clinical and Hereditary Aspects

    Christiansen, Morten Krogh

    2017-01-01

    ), and to characterize and quantify subclinical atherosclerosis in their relatives. Furthermore, the aim was to explore the impact of common genetic risk variants on the age of onset, familial clustering and disease severity. In study I, 143 patients with early-onset CAD were recruited from the Western Denmark Heart...

  17. Clinical evaluation of stress thallium spect in ischemic heart disease

    Sui, Osamu; Kimura, Nazuna; Soeki; Takeshi; Takeichi, Naoki; Shinohara, Hisanori; Tamura, Yoshiyuki; Fukuda, Nobuo

    1997-01-01

    Thallium SPECT was performed in patients with significant coronary artery stenosis, 67 cases were after maximal exercise and 74 cases were during coronary vasodilation induced by ATP (adenosine triphosphate) infusion. In patients suspected of angina pectoris, the sensitivity, specificity and predictive accuracy for detection of coronary artery disease (CAD) were 88%, 78% and 82% for exercise SPECT, and 100%, 72% and 84% for ATP SPECT studies, respectively. In patients with old myocardial infarction, these were 73%, 100% and 88% for exercise SPECT and 71%, 100% and 81% for ATP SPECT. These were 75%, 49% and 60% for treadmill exercise test in the patient group including both angina and myocardial infarction. For detection of diseased vessels, the diagnostic accuracy for left anterior descending artery and right coronary artery lesions was almost equal for ATP and exercise SPECT study, but ATP SPECT study was more sensitive than exercise SPECT study in detection of left circumflex artery lesions. ATP as well as exercise SPECT studies occasionally gave false positive results in patients with single-vessel disease. ATP as well as exercise SPECT studies underestimated the severity of multi-vessel disease. In general, the results of ATP SPECT imaging were highly concordant with the results of exercise SPECT imaging. ATP stress thallium SPECT imaging provided a safe and highly accurate diagnostic tool for detection of CAD. (author)

  18. Clinical Studies in Risk Stratification & Therapy of Thoracic Aortic Disease

    Kamman, AV

    2017-01-01

    For this thesis we aimed to summarize outcomes and optimal treatment modality for thoracic aortic disease, discuss new imaging techniques and improve the use of current imaging modalities. Furthermore, we aimed to improve risk stratification for uncomplicated type B aortic dissection (TBAD) and

  19. Cerebrospinal Fluid Biomarkers in Diagnosing Alzheimer's Disease in Clinical Practice

    Slats, Diane; Spies, Petra E; Sjögren, Magnus J C

    2010-01-01

    Analysis of the brain specific biomarkers amyloid beta(42) (Abeta(42)) and total tau (t-tau) protein in cerebrospinal fluid (CSF) has a sensitivity and specificity of more than 85% for differentiating Alzheimer's Disease (AD) from non-demented controls. International guidelines are contradictory...

  20. Memory in neurodegenerative disease: biological, cognitive, and clinical perspectives

    Tröster, Alexander I

    1998-01-01

    ... disease: contributions from comparative neuropsychology  -    3 2 Nonprimate animal models of motor and cognitive dysfunction...

  1. HANSENS DISEASE : STUDY OF CLINICAL, NEUROPATHOLOGICAL, NEUROPHYSIOLOGICAL PATTERN OF LEPROUS NEUROPATHY

    Vijay Kumar; Ajay Kumar

    2015-01-01

    A need still exists to determine the clinical and neurophysiological characteristics of leprosy neuropathy at distinct times of the disease by different methods that measure the various nerve fiber functions. A prospective clinical study was performed 100 patients of clinically proven Hansen’s will take in study and given diagnosis is made by dermatologist and neurologist. For Study of Clinical, Neuropathological , Neurophysiological Pattern of leprous neuropathy and r...

  2. A clinical perspective of obesity, metabolic syndrome and cardiovascular disease

    Thang S Han

    2016-02-01

    Full Text Available The metabolic syndrome is a condition characterized by a special constellation of reversible major risk factors for cardiovascular disease and type 2 diabetes. The main, diagnostic, components are reduced HDL-cholesterol, raised triglycerides, blood pressure and fasting plasma glucose, all of which are related to weight gain, specifically intra-abdominal/ectopic fat accumulation and a large waist circumference. Using internationally adopted arbitrary cut-off values for waist circumference, having metabolic syndrome doubles the risk of cardiovascular disease, but offers an effective treatment approach through weight management. Metabolic syndrome now affects 30–40% of people by age 65, driven mainly by adult weight gain, and by a genetic or epigenetic predisposition to intra-abdominal/ectopic fat accumulation related to poor intra-uterine growth. Metabolic syndrome is also promoted by a lack of subcutaneous adipose tissue, low skeletal muscle mass and anti-retroviral drugs. Reducing weight by 5–10%, by diet and exercise, with or without, anti-obesity drugs, substantially lowers all metabolic syndrome components, and risk of type 2 diabetes and cardiovascular disease. Other cardiovascular disease risk factors such as smoking should be corrected as a priority. Anti-diabetic agents which improve insulin resistance and reduce blood pressure, lipids and weight should be preferred for diabetic patients with metabolic syndrome. Bariatric surgery offers an alternative treatment for those with BMI ≥ 40 or 35–40 kg/m 2 with other significant co-morbidity. The prevalence of the metabolic syndrome and cardiovascular disease is expected to rise along with the global obesity epidemic: greater emphasis should be given to effective early weight-management to reduce risk in pre-symptomatic individuals with large waists.

  3. Clinical pharmacy activities in chronic kidney disease and end-stage renal disease patients: a systematic literature review

    Stemer Gunar

    2011-07-01

    Full Text Available Abstract Background Chronic kidney disease (CKD and end-stage renal disease (ESRD represent worldwide health problems with an epidemic extent. Therefore, attention must be given to the optimisation of patient care, as gaps in the care of CKD and ESRD patients are well documented. As part of a multidisciplinary patient care strategy, clinical pharmacy services have led to improvements in patient care. The purpose of this study was to summarise the available evidence regarding the role and impact of clinical pharmacy services for these patient populations. Methods A literature search was conducted using the Medline, Embase and International Pharmaceutical Abstracts databases to identify relevant studies on the impact of clinical pharmacists on CKD and ESRD patients, regarding disease-oriented and patient-oriented outcomes, and clinical pharmacist interventions on drug-related problems. Results Among a total of 21 studies, only four (19% were controlled trials. The majority of studies were descriptive (67% and before-after studies (14%. Interventions comprised general clinical pharmacy services with a focus on detecting, resolving and preventing drug-related problems, clinical pharmacy services with a focus on disease management, or clinical pharmacy services with a focus on patient education in order to increase medication knowledge. Anaemia was the most common comorbidity managed by clinical pharmacists, and their involvement led to significant improvement in investigated disease-oriented outcomes, for example, haemoglobin levels. Only four of the studies (including three controlled trials presented data on patient-oriented outcomes, for example, quality of life and length of hospitalisation. Studies investigating the number and type of clinical pharmacist interventions and physician acceptance rates reported a mean acceptance rate of 79%. The most common reported drug-related problems were incorrect dosing, the need for additional

  4. The causation and clinical management of pelvic radiation disease

    Schofield, P.; Lupton, E.

    1989-01-01

    This book describes the principles and methods of radiotherapy for pelvic tumours, pathological features and radiological analysis of bowel and urinary tract changes. Topics covered include clinical assessment and management, where the need for a multi-disciplinary approach is emphasized

  5. Ebola Virus Diseases | Elisha | African Journal of Clinical and ...

    African Journal of Clinical and Experimental Microbiology ... The current outbreak and a single case reported in 1994 in Ivory Coast are the only two outbreaks in West Africa (7). However, the current outbreak, which stared in Guinea (Bissau) in March 2014, remains the deadliest today and the epidemic is still ongoing.

  6. Seropositive pigs to Toxoplasma gondii (Apicomplexa: Toxoplasmatinae sent to slaughter and destined for human consumption

    Wanderley M. de Almeida

    2015-12-01

    Full Text Available ABSTRACT. de Almdeida W., Miranda Z.B., Flausino W., Coelho C.D., Fonseca A.B.M. & Lopes C.W.G. [Seropositive pigs to Toxoplasma gondii (Apicomplexa: Toxoplasmatinae sent to slaughter and destined for human consumption.] Suínos sororreagentes a Toxoplasma gondii (Apicomplexa: Toxoplasmatinae enviados para abate e destinados ao consumo humano. Revista Brasileira de Medicina Veterinária, 37(4:397-400, 2015. Curso de Pós-Graduação em Medicina Veterinária - Higiene Veterinária e Processamento Tecnológico de Produtos de Origem Animal, Faculdade de Veterinária, Universidade Federal Fluminense, Rua Vital Brasil Filho, 64, Vital Brazil, Niterói, RJ 24230-340, Brasil. E-mail: wanderleyma@yahoo.com.br This study aimed to determine the frequency of pigs seropositive to Toxoplasma gondii by indirect immunofluorescence assay with cutoff 1:16. Of blood samples collected at slaughter, 250 of them were from animals raised in the State of Minas Gerais; while 181 were from the State of Rio de Janeiro with a total of 431 examined samples. Of these, 19/250 (4.41% of MG and 39/181 (9.04% of RJ, determining a total of 13.45% seropositive animals. Regardless of slaughter be under the control of the inspection service, the precedence of the animals was highly significant (p = 0.0001 in the number of seropositive animals. Although the percentage of seropositive animals to T. gondii from both regions studied in this work, and from the point of view of a public health problem, pork and its frescal type products may continue to be considered as a source of infection of this coccidia for humans, if they are consumed as raw or undercooked.

  7. Clinical significance of determination of serum TRAb levels in patients with thyroid diseases

    Wu Lijian; Tang Bing; Yang Chunying; Lin Jun

    2005-01-01

    Objective: To investigate the clinical Significance of determination of serum thyroid stimulating hormone receptor antibody (TRAb) levels in patients with thyroid diseases, especially in patients with Graves' disease. Methods: Serum TRAb levels were determined with radio-receptor assay (RRA) in 302 patients with various thyroid diseases and 52 controls. Results: In patients with Graves' disease before treatment (n=62) the positive rate of TRAb was 86.3%. In patients with Graves' diseases improved after treatment (n=60), the positive rate was 74.5%; in those clinically cured (n=68) the positive rate was 32.1%. In 58 patients with Graves' disease who relapsed after apparently cure, the positive rate of TRAb rose to 90.3 %. However, there were no positive TRAb cases in 23 patients with simple goiter and 31 patients with benign thyroid adenomas. Also, there were no positive TRAb cases in the controls. Conclusion: TRAb levels correspond well with the severity of Graves' disease. (authors)

  8. The Affordability of Providing Sexually Transmitted Disease Services at a Safety-net Clinic.

    Dean, Lorraine T; Montgomery, Madeline C; Raifman, Julia; Nunn, Amy; Bertrand, Thomas; Almonte, Alexi; Chan, Philip A

    2018-04-01

    Sexually transmitted diseases continue to increase in the U.S. There is a growing need for financially viable models to ensure the longevity of safety-net sexually transmitted disease clinics, which provide testing and treatment to high-risk populations. This micro-costing analysis estimated the number of visits required to balance cost and revenue of a sexually transmitted disease clinic in a Medicaid expansion state. In 2017, actual and projected cost and revenues were estimated from the Rhode Island sexually transmitted disease clinic in 2015. Projected revenues for a hypothetical clinic offering a standard set of sexually transmitted disease services were based on Medicaid; private ("commercial") insurance; and institutional ("list price") reimbursement rates. The number of visits needed to cover clinic costs at each rate was assessed. Total operating cost for 2,153 clinic visits was estimated at $255,769, or $119 per visit. Laboratory testing and salaries each accounted for 44% of operating costs, medications for treatment 7%, supplies 5%, and 28% of visits used insurance. For a standard clinic offering a basic set of sexually transmitted disease services to break even, a projected 73% of visits need to be covered at the Medicaid rate, 38% at private rate, or 11% at institutional rate. Sexually transmitted disease clinics may be financially viable when a majority of visits are billed at a Medicaid rate; however, mixed private/public models may be needed if not all visits are billed. In this manner, sexually transmitted disease clinics can be solvent even if not all visits are billed to insurance, thus ensuring access to uninsured or underinsured patients. Copyright © 2018 American Journal of Preventive Medicine. Published by Elsevier Inc. All rights reserved.

  9. Clinical image: Hydatid disease of the chest wall

    Graham, R.J.; Berlin, J.W.; Ghahremani, G.G. [Northwestern Univ., Evanston, IL (United States)

    1996-05-01

    Hydatid disease is rarely encountered among the population of the United States, but it affects several million people in sheep-raising regions of the world. Human infestation with Echinococcus granulosus begins following ingestion of its ova, which are excreted into the contaminated water during the usual dog-sheep cycle. Hydatid cysts will then develop most frequently in the liver (75% of cases) and lungs (15%) of the human host. Skeletal involvement has been reported to occur in only 0.5-4.0% of patients in the endemic areas. Because of the rarity and perplexing imaging features of hydatid disease involving the chest wall, we wish herein to present a case evaluated recently at our institution. 5 refs., 1 fig.

  10. Advanced Parkinson's disease: clinical characteristics and treatment. Part II.

    Kulisevsky, J; Luquin, M R; Arbelo, J M; Burguera, J A; Carrillo, F; Castro, A; Chacón, J; García-Ruiz, P J; Lezcano, E; Mir, P; Martinez-Castrillo, J C; Martínez-Torres, I; Puente, V; Sesar, A; Valldeoriola-Serra, F; Yañez, R

    2013-01-01

    Many patients who have had Parkinson's disease (PD) for several years will present severe motor fluctuations and dyskinesias which require more aggressive therapies. The different approaches which are now available include deep brain stimulation of the subthalamic nucleus or medial globus pallidus, subcutaneous infusion of apomorphine, and intestinal infusion of levodopa-carbidopa. To define the indications and results for the 3 available therapies for advanced PD. Exhaustive review of the literature concerning the indications and results of deep brain stimulation, subcutaneous apomorphine infusion and duodenal infusion of levodopa/carbidopa gel to treat patients with advanced Parkinson disease. Although numerous studies have confirmed the efficacy of the 3 different therapies in advanced PD, there are no comparative studies that would allow us to define the best candidate for each technique. Copyright © 2013 Sociedad Española de Neurología. Published by Elsevier Espana. All rights reserved.

  11. Ebola Virus Disease in Pregnancy: Clinical, Histopathologic, and Immunohistochemical Findings.

    Muehlenbachs, Atis; de la Rosa Vázquez, Olimpia; Bausch, Daniel G; Schafer, Ilana J; Paddock, Christopher D; Nyakio, Jean Paul; Lame, Papys; Bergeron, Eric; McCollum, Andrea M; Goldsmith, Cynthia S; Bollweg, Brigid C; Prieto, Miriam Alía; Lushima, Robert Shongo; Ilunga, Benoit Kebela; Nichol, Stuart T; Shieh, Wun-Ju; Ströher, Ute; Rollin, Pierre E; Zaki, Sherif R

    2017-01-01

    Here we describe clinicopathologic features of Ebola virus disease in pregnancy. One woman infected with Sudan virus in Gulu, Uganda, in 2000 had a stillbirth and survived, and another woman infected with Bundibugyo virus had a live birth with maternal and infant death in Isiro, the Democratic Republic of the Congo in 2012. Ebolavirus antigen was seen in the syncytiotrophoblast and placental maternal mononuclear cells by immunohistochemical analysis, and no antigen was seen in fetal placental stromal cells or fetal organs. In the Gulu case, ebolavirus antigen localized to malarial parasite pigment-laden macrophages. These data suggest that trophoblast infection may be a mechanism of transplacental ebolavirus transmission. Published by Oxford University Press for the Infectious Diseases Society of America 2016. This work is written by (a) US Government employee(s) and is in the public domain in the US.

  12. [Neuromuscular disease: respiratory clinical assessment and follow-up].

    Martínez Carrasco, C; Villa Asensi, J R; Luna Paredes, M C; Osona Rodríguez de Torres, F B; Peña Zarza, J A; Larramona Carrera, H; Costa Colomer, J

    2014-10-01

    Patients with neuromuscular disease are an important group at risk of frequently suffering acute or chronic respiratory failure, which is their main cause of death. They require follow-up by a pediatric respiratory medicine specialist from birth or diagnosis in order to confirm the diagnosis and treat any respiratory complications within a multidisciplinary context. The ventilatory support and the cough assistance have improved the quality of life and long-term survival for many of these patients. In this paper, the authors review the pathophysiology, respiratory function evaluation, sleep disorders, and the most frequent respiratory complications in neuromuscular diseases. The various treatments used, from a respiratory medicine point of view, will be analyzed in a next paper. Copyright © 2013 Asociación Española de Pediatría. Published by Elsevier Espana. All rights reserved.

  13. Memory in neurodegenerative disease: biological, cognitive, and clinical perspectives

    Tröster, Alexander I

    1998-01-01

    ... of memory dysfunction in neurodegenerative disease  . ,  . ,     .  100 6 Functional neuroimaging correlates...

  14. Cardiac autonomic testing and treating heart disease. 'A clinical perspective'

    Nicholas L. DePace

    2014-12-01

    Full Text Available Background Coronary heart disease (CHD is a major health concern, affecting nearly half the middle-age population and responsible for nearly one-third of all deaths. Clinicians have several major responsibilities beyond diagnosing CHD, such as risk stratification of patients for major adverse cardiac events (MACE and treating risks, as well as the patient. This second of a two-part review series discusses treating risk factors, including autonomic dysfunction, and expected outcomes. Methods Therapies for treating cardiac mortality risks including cardiovascular autonomic neuropathy (CAN, are discussed. Results While risk factors effectively target high-risk patients, a large number of individuals who will develop complications from heart disease are not identified by current scoring systems. Many patients with heart conditions, who appear to be well-managed by traditional therapies, experience MACE. Parasympathetic and Sympathetic (P&S function testing provides more information and has the potential to further aid doctors in individualizing and titrating therapy to minimize risk. Advanced autonomic dysfunction (AAD and its more severe form cardiovascular autonomic neuropathy have been strongly associated with an elevated risk of cardiac mortality and are diagnosable through autonomic testing. This additional information includes patient-specific physiologic measures, such as sympathovagal balance (SB. Studies have shown that establishing and maintaining proper SB minimizes morbidity and mortality risk. Conclusions P&S testing promotes primary prevention, treating subclinical disease states, as well as secondary prevention, thereby improving patient outcomes through (1 maintaining wellness, (2 preventing symptoms and disorder and (3 treating subclinical manifestations (autonomic dysfunction, as well as (4 disease and symptoms (autonomic neuropathy.

  15. Clinical and genetic data of Huntington disease in Moroccan patients

    1. Equipe de Recherche sur les Maladies Neurodégénératives, Faculté de Médecine et de Pharmacie, Université ... Service de Neurologie et de Neurogénétique, Hôpital des Spécialités de Rabat, Morocco. 3. ... ed person have a 50% risk of inheriting the disease. It ... deficit, loss of motivation, concentration problems, dif-.

  16. A Rare Clinical Presentation of Darier’s Disease

    Ferizi, Mybera; Begolli-Gerqari, Antigona; Luzar, Bostjan; Kurshumliu, Fisnik; Ferizi, Mergita

    2013-01-01

    Darier’s disease, also known as keratosis follicularis or dyskeratosis follicularis, is a rare disorder of keratinization. It is an autosomal dominant genodermatosis with high penetrance and variable expressivity. Its manifestation appears as hyperkeratotic papules, primarily affecting seborrheic areas on the head, neck, and thorax and less frequently on the oral mucosa. When oral manifestations are present, the palatal and alveolar mucosae are primarily affected. They are usually asymptomat...

  17. Preferred retinal locus in macular disease: characteristics and clinical implications.

    Greenstein, Vivienne C; Santos, Rodrigo A V; Tsang, Stephen H; Smith, R Theodore; Barile, Gaetano R; Seiple, William

    2008-10-01

    To investigate the location and fixation stability of preferred retinal locations (PRLs) in patients with macular disease, and the relationship among areas of abnormal fundus autofluorescence, the PRL and visual sensitivity. Fifteen patients (15 eyes) were studied. Seven had Stargardt disease, 1 bull's eye maculopathy, 5 age-related macular degeneration, 1 Best disease, and 1 pattern dystrophy. All tested eyes had areas of abnormal fundus autofluorescence. The PRL was evaluated with fundus photography and the Nidek microperimeter. Visual field sensitivity was measured with the Nidek microperimeter. Of the 15 eyes, 4 had foveal and 11 had eccentric fixation. Eccentric PRLs were above the atrophic lesion and their stability did not depend on the degree of eccentricity from the fovea. Visual sensitivity was markedly decreased in locations corresponding to hypofluorescent areas. Sensitivity was not decreased in hyperfluorescent areas corresponding to flecks but was decreased if hyperfluorescence was in the form of dense annuli. Eccentric PRLs were in the superior retina in regions of normal fundus autofluorescence. Fixation stability was not correlated with the degree of eccentricity from the fovea. To assess the outcomes of treatment trials it is important to use methods that relate retinal morphology to visual function.

  18. Clinical effects of CO2 laser on equine diseases

    Lindholm, Arne; Svensson, Ulf; Collinder, Eje

    2002-10-01

    CO2 lasers has been used for five years at Malaren Equine Hospital, as an alternative treatment of some equine diseases. The application of CO2 laser has been studied for evaluation of its appropriateness for treatment of the equine diseases sarcoids, lameness in fetlock joints or pulmonary haemorrhage. During the last five years, above 100 equine sarcoids have been removed by laser surgery (CO2 laser) and so far resulting in significantly few recurrences compared with results from usual excision surgery. In one study, acute traumatic arthritis in fetlock joints was treated three times every second day with defocalised CO2 laser. The therapeutic effectiveness of CO2 laser in this study was better than that of the customary therapy with betamethasone plus hyaluronan. During one year, chronic pulmonary bleeders, namely exercise induced pulmonary haemorrhage, has been treated with defocalised CO2 laser. Six race horses have been treated once daily during five days. Until now, three of these horses have subsequently been successfully racing and no symptoms of pulmonary haemorrhage have been observed. These studies indicate that CO2 laser might be an appropriate therapy on sarcoids and traumatic arthritis, and probably also on exercise induced pulmonary haemorrhage. Other treatments for this pulmonary disease are few.

  19. Gastroesophageal reflux disease: A clinical overview for primary care physicians.

    Pandit, Sudha; Boktor, Moheb; Alexander, Jonathan S; Becker, Felix; Morris, James

    2018-03-01

    GERD is among the most common outpatient disease processes encountered by clinicians on a daily basis. This review provides insights about how to approach GERD in terms of disease management and treatment. Review articles were searched using PUBMED and MEDLINE using criteria that included English language articles published in the last 5 years concerning studies carried out only in humans. The key words used in the searches were GERD, PPI, and erosive esophagitis. Recommendations from the American College of Gastroenterology are also included in this manuscript. The search resulted in ∼260 articles. The manuscript brings together and presents the results of recent recommendations from professional societies and recently published review articles on GERD. GERD is one of the most common diagnoses made by gastroenterologists and primary care physicians. It is important to recognize the typical and atypical presentations of GERD. This paper helps primary care physicians understand the disease's pathophysiology, and when, how, and with what to treat GERD before referring patients to gastroenterologists or surgeons. Copyright © 2017 Elsevier B.V. All rights reserved.

  20. Mesoglycan: Clinical Evidences for Use in Vascular Diseases

    Antonella Tufano

    2010-01-01

    Full Text Available Vascular glycosaminoglycans (GAG are essential components of the endothelium and vessel wall and have been shown to be involved in several biologic functions. Mesoglycan, a natural GAG preparation, is a polysaccharide complex rich in sulphur radicals with strong negative electric charge. It is extracted from porcine intestinal mucosa and is composed of heparan sulfate, dermatan sulfate, electrophoretically slow-moving heparin, and variable and minimal quantities of chondroitin sulfate. Data on antithrombotic and profibrinolytic activities of the drug show that mesoglycan, although not indicated in the treatment of acute arterial or venous thrombosis because of the low antithrombotic effect, may be useful in the management of vascular diseases, when combined with antithrombotics in the case of disease of cerebral vasculature, and with antithrombotics and vasodilator drugs in the case of chronic peripheral arterial disease. The protective effect of mesoglycan in patients with venous thrombosis and the absence of side effects, support the use of GAG in patients with chronic venous insufficiency and persistent venous ulcers, in association with compression therapy (zinc bandages, multiple layer bandages, etc., elastic compression stockings, and local care, and in the prevention of recurrences in patients with previous DVT following the standard course of oral anticoagulation treatment.

  1. Encephalopathy associated with autoimmune thyroid disease in patients with Graves' disease: clinical manifestations, follow-up, and outcomes

    Tamagno, Gianluca

    2010-04-28

    Abstract Background The encephalopathy associated with autoimmune thyroid disease (EAATD) is characterized by neurological\\/psychiatric symptoms, high levels of anti-thyroid antibodies, increased cerebrospinal fluid protein concentration, non-specific electroencephalogram abnormalities, and responsiveness to the corticosteroid treatment in patients with an autoimmune thyroid disease. Almost all EAATD patients are affected by Hashimoto\\'s thyroiditis (HT), although fourteen EAATD patients with Graves\\' disease (GD) have been also reported. Methods We have recorded and analyzed the clinical, biological, radiological, and electrophysiological findings and the data on the therapeutic management of all GD patients with EAATD reported so far as well as the clinical outcomes in those followed-up in the long term. Results Twelve of the fourteen patients with EAATD and GD were women. The majority of GD patients with EAATD presented with mild hyperthyroidism at EAATD onset or shortly before it. Active anti-thyroid autoimmunity was detected in all cases. Most of the patients dramatically responded to corticosteroids. The long term clinical outcome was benign but EAATD can relapse, especially at the time of corticosteroid dose tapering or withdrawal. GD and HT patients with EAATD present with a similar clinical, biological, radiological, and electrophysiological picture and require an unaffected EAATD management. Conclusions GD and HT equally represent the possible background condition for the development of EAATD, which should be considered in the differential diagnosis of all patients with encephalopathy of unknown origin and an autoimmune thyroid disease, regardless of the nature of the underlying autoimmune thyroid disease.

  2. Encephalopathy associated with autoimmune thyroid disease in patients with Graves' disease: clinical manifestations, follow-up, and outcomes.

    Tamagno, Gianluca

    2012-02-01

    BACKGROUND: The encephalopathy associated with autoimmune thyroid disease (EAATD) is characterized by neurological\\/psychiatric symptoms, high levels of anti-thyroid antibodies, increased cerebrospinal fluid protein concentration, non-specific electroencephalogram abnormalities, and responsiveness to the corticosteroid treatment in patients with an autoimmune thyroid disease. Almost all EAATD patients are affected by Hashimoto\\'s thyroiditis (HT), although fourteen EAATD patients with Graves\\' disease (GD) have been also reported. METHODS: We have recorded and analyzed the clinical, biological, radiological, and electrophysiological findings and the data on the therapeutic management of all GD patients with EAATD reported so far as well as the clinical outcomes in those followed-up in the long term. RESULTS: Twelve of the fourteen patients with EAATD and GD were women. The majority of GD patients with EAATD presented with mild hyperthyroidism at EAATD onset or shortly before it. Active anti-thyroid autoimmunity was detected in all cases. Most of the patients dramatically responded to corticosteroids. The long term clinical outcome was benign but EAATD can relapse, especially at the time of corticosteroid dose tapering or withdrawal. GD and HT patients with EAATD present with a similar clinical, biological, radiological, and electrophysiological picture and require an unaffected EAATD management. CONCLUSIONS: GD and HT equally represent the possible background condition for the development of EAATD, which should be considered in the differential diagnosis of all patients with encephalopathy of unknown origin and an autoimmune thyroid disease, regardless of the nature of the underlying autoimmune thyroid disease.

  3. Encephalopathy associated with autoimmune thyroid disease in patients with Graves' disease: clinical manifestations, follow-up, and outcomes

    Lee Byung I

    2010-04-01

    Full Text Available Abstract Background The encephalopathy associated with autoimmune thyroid disease (EAATD is characterized by neurological/psychiatric symptoms, high levels of anti-thyroid antibodies, increased cerebrospinal fluid protein concentration, non-specific electroencephalogram abnormalities, and responsiveness to the corticosteroid treatment in patients with an autoimmune thyroid disease. Almost all EAATD patients are affected by Hashimoto's thyroiditis (HT, although fourteen EAATD patients with Graves' disease (GD have been also reported. Methods We have recorded and analyzed the clinical, biological, radiological, and electrophysiological findings and the data on the therapeutic management of all GD patients with EAATD reported so far as well as the clinical outcomes in those followed-up in the long term. Results Twelve of the fourteen patients with EAATD and GD were women. The majority of GD patients with EAATD presented with mild hyperthyroidism at EAATD onset or shortly before it. Active anti-thyroid autoimmunity was detected in all cases. Most of the patients dramatically responded to corticosteroids. The long term clinical outcome was benign but EAATD can relapse, especially at the time of corticosteroid dose tapering or withdrawal. GD and HT patients with EAATD present with a similar clinical, biological, radiological, and electrophysiological picture and require an unaffected EAATD management. Conclusions GD and HT equally represent the possible background condition for the development of EAATD, which should be considered in the differential diagnosis of all patients with encephalopathy of unknown origin and an autoimmune thyroid disease, regardless of the nature of the underlying autoimmune thyroid disease.

  4. Encephalopathy associated with autoimmune thyroid disease in patients with Graves' disease: clinical manifestations, follow-up, and outcomes.

    Tamagno, Gianluca

    2010-01-01

    BACKGROUND: The encephalopathy associated with autoimmune thyroid disease (EAATD) is characterized by neurological\\/psychiatric symptoms, high levels of anti-thyroid antibodies, increased cerebrospinal fluid protein concentration, non-specific electroencephalogram abnormalities, and responsiveness to the corticosteroid treatment in patients with an autoimmune thyroid disease. Almost all EAATD patients are affected by Hashimoto\\'s thyroiditis (HT), although fourteen EAATD patients with Graves\\' disease (GD) have been also reported. METHODS: We have recorded and analyzed the clinical, biological, radiological, and electrophysiological findings and the data on the therapeutic management of all GD patients with EAATD reported so far as well as the clinical outcomes in those followed-up in the long term. RESULTS: Twelve of the fourteen patients with EAATD and GD were women. The majority of GD patients with EAATD presented with mild hyperthyroidism at EAATD onset or shortly before it. Active anti-thyroid autoimmunity was detected in all cases. Most of the patients dramatically responded to corticosteroids. The long term clinical outcome was benign but EAATD can relapse, especially at the time of corticosteroid dose tapering or withdrawal. GD and HT patients with EAATD present with a similar clinical, biological, radiological, and electrophysiological picture and require an unaffected EAATD management. CONCLUSIONS: GD and HT equally represent the possible background condition for the development of EAATD, which should be considered in the differential diagnosis of all patients with encephalopathy of unknown origin and an autoimmune thyroid disease, regardless of the nature of the underlying autoimmune thyroid disease.

  5. Rheumatoid arthritis disease activity measures: American College of Rheumatology recommendations for use in clinical practice.

    Anderson, Jaclyn; Caplan, Liron; Yazdany, Jinoos; Robbins, Mark L; Neogi, Tuhina; Michaud, Kaleb; Saag, Kenneth G; O'Dell, James R; Kazi, Salahuddin

    2012-05-01

    Although the systematic measurement of disease activity facilitates clinical decision making in rheumatoid arthritis (RA), no recommendations currently exist on which measures should be applied in clinical practice in the US. The American College of Rheumatology (ACR) convened a Working Group (WG) to comprehensively evaluate the validity, feasibility, and acceptability of available RA disease activity measures and derive recommendations for their use in clinical practice. The Rheumatoid Arthritis Clinical Disease Activity Measures Working Group conducted a systematic review of the literature to identify RA disease activity measures. Using exclusion criteria, input from an Expert Advisory Panel (EAP), and psychometric analysis, a list of potential measures was created. A survey was administered to rheumatologists soliciting input. The WG used these survey results in conjunction with the psychometric analyses to derive final recommendations. Systematic review of the literature resulted in identification of 63 RA disease activity measures. Application of exclusion criteria and ratings by the EAP narrowed the list to 14 measures for further evaluation. Practicing rheumatologists rated 9 of these 14 measures as most useful and feasible. From these 9 measures, the WG selected 6 with the best psychometric properties for inclusion in the final set of ACR-recommended RA disease activity measures. We recommend the Clinical Disease Activity Index, Disease Activity Score with 28-joint counts (erythrocyte sedimentation rate or C-reactive protein), Patient Activity Scale (PAS), PAS-II, Routine Assessment of Patient Index Data with 3 measures, and Simplified Disease Activity Index because they are accurate reflections of disease activity; are sensitive to change; discriminate well between low, moderate, and high disease activity states; have remission criteria; and are feasible to perform in clinical settings. Copyright © 2012 by the American College of Rheumatology.

  6. Nutrition-Related Cardiovascular Disease Risk Factors In Chronic Kidney Disease: Relationship With Clinical Outcome

    Emma McMahon

    2012-06-01

    Traditional CV-risk factors in this CKD population were not associated with clinical outcome. Despite being within clinical reference range, serum phosphate and albumin were independently associated with clinical outcome. This may highlight a potential therapeutic target for risk management to delay or prevent renal end-points in CKD.

  7. Clinical Polymorphism of Stargardt Disease in a Large Consanguineous Tunisian Family; Implications for Nosology

    Leila El Matri

    2013-01-01

    Full Text Available Purpose: To describe the polymorphic expression of Stargardt disease in a large Tunisian family with clinical intra- and interfamilial variation of the condition. Methods: Twelve subjects from two related families with autosomal recessive Stargardt disease were enrolled. A detailed clinical examination including visual acuity and visual field measurement, fundus photography, fluorescein angiography, electroretinography (ERG and color vision testing was performed for all subjects. Results: The youngest child from family A manifested typical Stargardt disease while her two brothers presented with Stargardt disease-fundus flavimaculatus (STGD-FFM and her two sisters demonstrated a peculiar phenotype overlapping Stargardt disease and cone-rod dystrophy; their phenotypic manifestation corresponded well with ERG groups I, II and III, respectively. This uncommon occurrence of an age-related decline in ERG amplitude and worsening of fundus changes is suggestive of a grading pattern in Stargardt disease. Their two cousins in family B, displayed the STGD-FFM phenotype. Despite clinically similar STGD-FFM patterns in both families, age of onset and progression of the phenotype in family B differed from family A. Conclusion: This is the first report on phenotypic variation of Stargardt disease in a large Tunisian family. Regarding phenotype and severity of visual symptoms, family A demonstrated Stargardt disease at various stages of progression. In addition, STGDFFM appeared to be an independent clinical entity in family B. These findings imply that further parameters are required to classify Stargardt′s disease.

  8. Omega-3 polyunsaturated fatty acid (fish oil) supplementation and the prevention of clinical cardiovascular disease

    Multiple randomized controlled trials (RCTs) have assessed the effects of supplementation with eicosapentaenoic acid plus docosahexaenoic acid (omega-3 polyunsaturated fatty acids, commonly called fish oils) on the occurrence of clinical cardiovascular diseases. Although the effects of supplementati...

  9. Clinical outcome and radiographic results after operative treatment of Scheuermann's disease

    Poolman, R. W.; Been, H. D.; Ubags, L. H.

    2002-01-01

    The aim of this prospective study was to evaluate radiographic findings, patient satisfaction and clinical outcome, and to report complications and instrumentation failure after operative treatment of Scheuermann's disease using a combined anterior and posterior spondylodesis. The loss of sagittal

  10. Clinical Application of 18F-FDG PET in Alzheimer's Disease

    Ryu, Young Hoon

    2008-01-01

    PET of the cerebral metabolic rate of glucose is increasingly used to support the clinical diagnosis in the examination of patients with suspected major neurodegenerative disorders, such as Alzheimer's disease. 18 F-FDG PET has been reported to have high diagnostic performance, especially, very high sensitivity in the diagnosis and clinical assessment of therapeutic efficacy. According to clinical research data hitherto, 18 F-FDG PET is expected to be an effective diagnostic tool in early and differential diagnosis of Alzheimer's disease. Since 2004, Medicare covers 18 F-FDG PET scans for the differential diagnosis of fronto-temporal dementia (FTD) and Alzheimer's disease (AD) under specific requirements; or, its use in a CMS approved practical clinical trial focused on the utility of 18 F-FDG PET in the diagnosis or treatment of dementing neurodegenerative diseases

  11. The clinical, biochemical and genetic features associated with RMND1-related mitochondrial disease

    Ng, Yi Shiau; Alston, Charlotte L; Diodato, Daria

    2016-01-01

    BACKGROUND: Mutations in the RMND1 (Required for Meiotic Nuclear Division protein 1) gene have recently been linked to infantile onset mitochondrial disease characterised by multiple mitochondrial respiratory chain defects. METHODS: We summarised the clinical, biochemical and molecular genetic in...

  12. Lyme disease risk in dogs in New Brunswick

    Bjurman, Natalie K.; Bradet, Gina; Lloyd, Vett K.

    2016-01-01

    This study assessed the seroprevalence of anti-Borrelia burgdorferi antibodies in New Brunswick dogs. Testing of 699 serum samples from dogs across the province revealed a 6% province-wide seropositivity, more than 6 times higher than that found in 2008. The rapid increase in seropositivity indicates increased Lyme disease risk to both canine and human health.

  13. C9orf72-related disorders: expanding the clinical and genetic spectrum of neurodegenerative diseases

    Paulo Victor Sgobbi de Souza

    2015-03-01

    Full Text Available Neurodegenerative diseases represent a heterogeneous group of neurological conditions primarily involving dementia, motor neuron disease and movement disorders. They are mostly related to different pathophysiological processes, notably in family forms in which the clinical and genetic heterogeneity are lush. In the last decade, much knowledge has been acumulated about the genetics of neurodegenerative diseases, making it essential in cases of motor neuron disease and frontotemporal dementia the repeat expansions of C9orf72 gene. This review analyzes the main clinical, radiological and genetic aspects of the phenotypes related to the hexanucleotide repeat expansions (GGGGCC of C9orf72 gene. Future studies will aim to further characterize the neuropsychological, imaging and pathological aspects of the extra-motor features of motor neuron disease, and will help to provide a new classification system that is both clinically and biologically relevant.

  14. Gastroesophageal reflux disease: exaggerations, evidence and clinical practice.

    Ferreira, Cristina Targa; Carvalho, Elisa de; Sdepanian, Vera Lucia; Morais, Mauro Batista de; Vieira, Mário César; Silva, Luciana Rodrigues

    2014-01-01

    there are many questions and little evidence regarding the diagnosis and treatment of gastroesophageal reflux disease (GERD) in children. The association between GERD and cow's milk protein allergy (CMPA), overuse of abdominal ultrasonography for the diagnosis of GERD, and excessive pharmacological treatment, especially proton-pump inhibitors (PPIs) are some aspects that need clarification. This review aimed to establish the current scientific evidence for the diagnosis and treatment of GERD in children. a search was conducted in the MEDLINE, PubMed, LILACS, SciELO, and Cochrane Library electronic databases, using the following keywords: gastroesophageal reflux; gastroesophageal reflux disease; proton-pump inhibitors; and prokinetics; in different age groups of the pediatric age range; up to May of 2013. abdominal ultrasonography should not be recommended to investigate gastroesophageal reflux (GER). Simultaneous treatment of GERD and CMPA often results in unnecessary use of medication or elimination diet. There is insufficient evidence for the prescription of prokinetics to all patients with GER/GERD. There is little evidence to support acid suppression in the first year of life, to treat nonspecific symptoms suggestive of GERD. Conservative treatment has many benefits and with low cost and no side-effects. there have been few randomized controlled trials that assessed the management of GERD in children and no examination can be considered the gold standard for GERD diagnosis. For these reasons, there are exaggerations in the diagnosis and treatment of this disease, which need to be corrected. Copyright © 2013 Sociedade Brasileira de Pediatria. Published by Elsevier Editora Ltda. All rights reserved.

  15. On clinics and therapy of acute radiation disease at the recovery period

    Alekseev, G.I.; Ivanov, I.A.; Nikiforov, A.M.

    1990-01-01

    Main features of clinics and therapy of acute radiation disease at the recovery period are described. Search for ways of activization of recovery to improve the efficiency of treatments and to accelerate patients rehabilitation is of great interest for clinical medicine. It is outlined that correction of post-irradiation disorders of energy and plastic metabolism by means of influence on cell metabolism must begin as early as possible already at the initial period of disease

  16. Coronary artery anomalies and clinically important anatomy in patients with congenital heart disease: multislice CT findings

    Goo, Hyun Woo; Seo, Dong-Man; Yun, Tae-Jin; Park, Jeong-Jun; Park, In-Sook; Ko, Jae Kon; Kim, Young Hwee

    2009-01-01

    In patients with congenital heart disease, coronary artery anomalies are common and have different clinical importance from individuals with structurally normal hearts. Visibility of the coronary arteries by CT has markedly improved due to high temporal resolution and ECG-synchronized data acquisition. In this article we describe current multislice CT techniques for coronary artery imaging and illustrate coronary artery anomalies and clinically important coronary artery anatomy from the point of view of congenital heart disease. (orig.)

  17. Coronary artery anomalies and clinically important anatomy in patients with congenital heart disease: multislice CT findings

    Goo, Hyun Woo [University of Ulsan College of Medicine, Department of Radiology and Research Institute of Radiology, Asan Medical Center, Seoul (Korea); Seo, Dong-Man; Yun, Tae-Jin; Park, Jeong-Jun [University of Ulsan College of Medicine, Department of Pediatric Cardiac Surgery, Asan Medical Center, Seoul (Korea); Park, In-Sook; Ko, Jae Kon; Kim, Young Hwee [University of Ulsan College of Medicine, Department of Pediatric Cardiology, Asan Medical Center, Seoul (Korea)

    2009-03-15

    In patients with congenital heart disease, coronary artery anomalies are common and have different clinical importance from individuals with structurally normal hearts. Visibility of the coronary arteries by CT has markedly improved due to high temporal resolution and ECG-synchronized data acquisition. In this article we describe current multislice CT techniques for coronary artery imaging and illustrate coronary artery anomalies and clinically important coronary artery anatomy from the point of view of congenital heart disease. (orig.)

  18. Stargardt disease: clinical features, molecular genetics, animal models and therapeutic options

    Tanna, P.; Strauss, R. W.; Fujinami, K.; Michaelides, M.

    2017-01-01

    Stargardt disease (STGD1; MIM 248200) is the most prevalent inherited macular dystrophy and is associated with disease-causing sequence variants in the gene ABCA4 Significant advances have been made over the last 10 years in our understanding of both the clinical and molecular features of STGD1, and also the underlying pathophysiology, which has culminated in ongoing and planned human clinical trials of novel therapies. The aims of this review are to describe the detailed phenotypic and genot...

  19. Stargardt disease: clinical features, molecular genetics, animal models and therapeutic options

    Tanna, Preena; Strauss, Rupert W; Fujinami, Kaoru; Michaelides, Michel

    2016-01-01

    Stargardt disease (STGD1; MIM 248200) is the most prevalent inherited macular dystrophy and is associated with disease-causing sequence variants in the gene ABCA4. Significant advances have been made over the last 10?years in our understanding of both the clinical and molecular features of STGD1, and also the underlying pathophysiology, which has culminated in ongoing and planned human clinical trials of novel therapies. The aims of this review are to describe the detailed phenotypic and geno...

  20. Cognitive impairment and dementia in Parkinson’s disease: clinical features, diagnosis, and management

    Joana eMeireles

    2012-05-01

    Full Text Available Parkinson’s disease (PD is a common, disabling, neurodegenerative disorder. In addition to classical motor symptoms, nonmotor features are now widely accepted as part of the clinical picture, and cognitive decline is a very important aspect of the disease, as it brings an additional significant burden for the patient and caregivers. The diagnosis of cognitive decline in PD, namely mild cognitive impairment and dementia, can be extremely challenging, remaining largely based on clinical and cognitive assessments. Diagnostic criteria and methods for PD dementia and mild cognitive impairment have been recently issued by expert work groups. This manuscript has synthesized relevant data in order to obtain a pragmatic and updated review regarding cognitive decline in PD, from milder stages to dementia. This text will summarize clinical features, diagnostic methodology, and therapeutic issues of clinical decline in Parkinson’s disease. Relevant clinical genetic issues, including recent advances, will also be approached.

  1. Clinical and molecular genetic features of Hb H and AE Bart's diseases in central Thai children.

    Traivaree, Chanchai; Boonyawat, Boonchai; Monsereenusorn, Chalinee; Rujkijyanont, Piya; Photia, Apichat

    2018-01-01

    α-Thalassemia, one of the major thalassemia types in Thailand, is caused by either deletion or non-deletional mutation of one or both α-globin genes. Inactivation of three α-globin genes causes hemoglobin H (Hb H) disease, and the combination of Hb H disease with heterozygous hemoglobin E (Hb E) results in AE Bart's disease. This study aimed to characterize the clinical and hematological manifestations of 76 pediatric patients with Hb H and AE Bart's diseases treated at Phramongkutklao Hospital, a tertiary care center for thalassemia patients in central Thailand. Seventy-six unrelated pediatric patients, 58 patients with Hb H disease and 18 patients with AE Bart's disease, were enrolled in this study. Their clinical presentations, transfusion requirement, laboratory findings, and mutation analysis were retrospectively reviewed and analyzed. A total of 76 pediatric patients with Hb H and AE Bart's diseases who mainly lived in central Thailand were included in this study. The clinical severities of patients with non-deletional mutations were more severe than those with deletional mutations. Eighty-six percent of patients with non-deletional AE Bart's disease required more blood transfusion compared to 12.5% of patients with deletional AE Bart's disease. Non-deletional AE Bart's disease also had a history of urgent blood transfusion with the average of 6±0.9 times compared to 1±0.3 times in patients with deletional Hb H disease. The difference was statistically significant. This study revealed the differences in clinical spectrum between patients with Hb H disease and those with AE Bart's disease in central Thailand. The differentiation of α-thalassemia is essential for appropriate management of patients. The molecular diagnosis is useful for diagnostic confirmation and genotype-phenotype correlation.

  2. National Priority Setting of Clinical Practice Guidelines Development for Chronic Disease Management.

    Jo, Heui-Sug; Kim, Dong Ik; Oh, Moo-Kyung

    2015-12-01

    By November 2013, a total of 125 clinical practice guidelines (CPGs) have been developed in Korea. However, despite the high burden of diseases and the clinical importance of CPGs, most chronic diseases do not have available CPGs. Merely 83 CPGs are related to chronic diseases, and only 40 guidelines had been developed in the last 5 yr. Considering the rate of the production of new evidence in medicine and the worsening burden from chronic diseases, the need for developing CPGs for more chronic diseases is becoming increasingly pressing. Since 2011, the Korean Academy of Medical Sciences and the Korea Centers for Disease Control and Prevention have been jointly developing CPGs for chronic diseases. However, priorities have to be set and resources need to be allocated within the constraint of a limited funding. This study identifies the chronic diseases that should be prioritized for the development of CPGs in Korea. Through an objective assessment by using the analytic hierarchy process and a subjective assessment with a survey of expert opinion, high priorities were placed on ischemic heart disease, cerebrovascular diseases, Alzheimer's disease and other dementias, osteoarthritis, neck pain, chronic kidney disease, and cirrhosis of the liver.

  3. Comparison of risk factors for seropositivity to feline immunodeficiency virus and feline leukemia virus among cats: a case-case study.

    Chhetri, Bimal K; Berke, Olaf; Pearl, David L; Bienzle, Dorothee

    2015-02-10

    Feline immunodeficiency virus (FIV) and feline leukemia virus (FeLV) are reported to have similar risk factors and similar recommendations apply to manage infected cats. However, some contrasting evidence exists in the literature with regard to commonly reported risk factors. In this study, we investigated whether the known risk factors for FIV and FeLV infections have a stronger effect for either infection. This retrospective study included samples from 696 cats seropositive for FIV and 593 cats seropositive for FeLV from the United States and Canada. Data were collected during two cross sectional studies, where cats were tested using IDEXX FIV/FeLV ELISA kits. To compare the effect of known risk factors for FIV infection compared to FeLV, using a case-case study design, random intercept logistic regression models were fit including cats' age, sex, neuter status, outdoor exposure, health status and type of testing facility as independent variables. A random intercept for testing facility was included to account for clustering expected in testing practices at the individual clinics and shelters. In the multivariable random intercept model, the odds of FIV compared to FeLV positive ELISA results were greater for adults (OR = 2.09, CI: 1.50-2.92), intact males (OR = 3.14, CI: 1.85-3.76), neutered males (OR = 2.68, CI: 1.44- 3.14), cats with outdoor access (OR = 2.58, CI: 1.85-3.76) and lower for cats with clinical illness (OR = 0.60, 95% CI: 0.52-0.90). The variance components obtained from the model indicated clustering at the testing facility level. Risk factors that have a greater effect on FIV seropositivity include adulthood, being male (neutered or not) and having access to outdoors, while clinical illness was a stronger predictor for FeLV seropositivity. Further studies are warranted to assess the implications of these results for the management and control of these infections.

  4. Clinical application of pelvic MR angiography for gynecologic diseases

    Ohnishi, Takeshi [Saint Marianna Univ., Kawasaki, Kanagawa (Japan). School of Medicine

    1996-08-01

    Magnetic resonance (MR) imaging is the second most important method next to the ultrasound for the diagnosis of gynecological disorders. MR angiography provides a new noninvasive method for evaluating vascular occlusive disease of the lower extremities and the branches of abdominal aorta. To our knowledge, no determinant of efficacy of MR angiography and images of gynecological diseases have yet been made. Maximum intensity projection image was obtained from the data set by the three dimensional contrast enhanced MR angiography (3D-CE MRA) with newly designed fat suppression method. MR angiogram reveals well visualization of lower abdominal aorta and its branches. Furthermore, uterine arteries were identified in 49.3% of the cases, branching from the internal iliac artery. Leiomyoma of the uterus showed an enhanced uterus in 53.7% of the eases. All cases of endometriosis demonstrated a homogeneous, high signal intensity being a specific finding. In conclusion, maximum intensity projection image of pelvis showed a good visualization of the lower abdominal aorta and its branches. Uterine artery and, in a few cases, ovarian artery can be demonstrated on MR angiography. All cases of endometriosis showed a significant, high signal intensity. This technique is noninvasive and be a supplementary method for diagnosis and follow up of vasculature of gynecological disorders. (author)

  5. Clinical application of pelvic MR angiography for gynecologic diseases

    Ohnishi, Takeshi

    1996-01-01

    Magnetic resonance (MR) imaging is the second most important method next to the ultrasound for the diagnosis of gynecological disorders. MR angiography provides a new noninvasive method for evaluating vascular occlusive disease of the lower extremities and the branches of abdominal aorta. To our knowledge, no determinant of efficacy of MR angiography and images of gynecological diseases have yet been made. Maximum intensity projection image was obtained from the data set by the three dimensional contrast enhanced MR angiography (3D-CE MRA) with newly designed fat suppression method. MR angiogram reveals well visualization of lower abdominal aorta and its branches. Furthermore, uterine arteries were identified in 49.3% of the cases, branching from the internal iliac artery. Leiomyoma of the uterus showed an enhanced uterus in 53.7% of the eases. All cases of endometriosis demonstrated a homogeneous, high signal intensity being a specific finding. In conclusion, maximum intensity projection image of pelvis showed a good visualization of the lower abdominal aorta and its branches. Uterine artery and, in a few cases, ovarian artery can be demonstrated on MR angiography. All cases of endometriosis showed a significant, high signal intensity. This technique is noninvasive and be a supplementary method for diagnosis and follow up of vasculature of gynecological disorders. (author)

  6. Diabetic Kidney Disease: From Epidemiology to Clinical Perspectives

    Cheol Whee Park

    2014-08-01

    Full Text Available With worldwide epidemic of diabetes mellitus, diabetic nephropathy which is one of the major causes of microvascular complication has become a serious concern in Korea as well as the rest of the world. In view of its significance, there is an urgent and paramount need for proper managements that could either deter or slow the progression of diabetic nephropathy. Despite advances in care, ever increasing number of patients suffering from diabetic kidney disease and from end-stage renal disease implies that the current management is not adequate in many aspects. The reasons for these inadequacies compromise lack of early diagnosis, failure to intervene with timely and aggressive manner, and lack of understanding on the kind of interventions required. Another issue equally important for the adequate care of patients with diabetic nephropathy is an understanding of past, present and future epidemiology of diabetic nephropathy which serves, especially in Korea, as a material determining standard diagnosis and treatment and a national health-policy decision.

  7. Emerging clinical experience with vaccines against group B meningococcal disease.

    Wilkins, A L; Snape, M D

    2017-08-01

    The prevention of paediatric bacterial meningitis and septicaemia has recently entered a new era with the availability of two vaccines against capsular group B meningococcus (MenB). Both of these vaccines are based on sub-capsular proteins of the meningococcus, an approach that overcomes the challenges set by the poorly immunogenic MenB polysaccharide capsule but adds complexity to predicting and measuring the impact of their use. This review describes the development and use of MenB vaccines to date, from the use of outer membrane vesicle (OMV) vaccines in MenB outbreaks around the world, to emerging evidence on the effectiveness of the newly available vaccines. While recent data from the United Kingdom supports the potential for protein-based vaccines to provide direct protection against MenB disease in immunised children, further research is required to understand the breadth and duration of this protection. A more detailed understanding of the impact of immunisation with these vaccines on nasopharyngeal carriage of the meningococcus is also required, to inform both their potential to induce herd immunity and to preferentially select for carriage of strains not susceptible to vaccine-induced antibodies. Although a full understanding of the potential impact of these vaccines will only be possible with this additional information, the availability of new tools to prevent the devastating effect of invasive MenB disease is a significant breakthrough in the fight against childhood sepsis and meningitis. Copyright © 2017 Elsevier Ltd. All rights reserved.

  8. Motor activation SPECT for the neurosurgical diseases. Clinical application

    Noguchi, Hiroshi; Kawaguchi, Shoichiro; Sakaki, Toshisuke; Imai, Teruhiko; Ohishi, Hajime

    1999-01-01

    We evaluated and analyzed the motor activation single photon emission computed tomography (M-SPECT) findings on patients with ischemic cerebrovascular diseases (CVD). The M-SPECT studies were carried out on 91 patients with ischemic cerebrovascular diseases. The M-SPECT study was performed using the finger opposition task in each case. The SPECT images were superimposed on the magnetic resonance images (MRIs) for each case using Image Fusion Software. The result of the M-SPECT was expressed as positive or negative. The cases with a marked increase of blood flow in the sensorio-motor cortex after the finger opposition task were categorized as positive, and those cases showing no marked increase of blood flow were categorized as negative. Among the 91 cases examined, 53 (58%) were categorized as positive in the M-SPECT study. Among the negative M-SPECT cases treated with revascularization surgery, there were some cases showing positive M-SPECT results postoperatively. The cases without any revascularization surgery did not change the M-SPECT findings in each during the follow-up period. The M-SPECT procedure for examining intracranial lesions could provide the cortical localization of the motor function. The M-SPECT procedure in the ischemic CVDs contributes to knowledge about the choices of treatment and the evaluation of the treatment result. (author)

  9. Clinical epidemiology of Alzheimer's disease: assessing sex and gender differences.

    Mielke, Michelle M; Vemuri, Prashanthi; Rocca, Walter A

    2014-01-01

    With the aging of the population, the burden of Alzheimer's disease (AD) is rapidly expanding. More than 5 million people in the US alone are affected with AD and this number is expected to triple by 2050. While men may have a higher risk of mild cognitive impairment (MCI), an intermediate stage between normal aging and dementia, women are disproportionally affected with AD. One explanation is that men may die of competing causes of death earlier in life, so that only the most resilient men may survive to older ages. However, many other factors should also be considered to explain the sex differences. In this review, we discuss the differences observed in men versus women in the incidence and prevalence of MCI and AD, in the structure and function of the brain, and in the sex-specific and gender-specific risk and protective factors for AD. In medical research, sex refers to biological differences such as chromosomal differences (eg, XX versus XY chromosomes), gonadal differences, or hormonal differences. In contrast, gender refers to psychosocial and cultural differences between men and women (eg, access to education and occupation). Both factors play an important role in the development and progression of diseases, including AD. Understanding both sex- and gender-specific risk and protective factors for AD is critical for developing individualized interventions for the prevention and treatment of AD.

  10. Clinical availability of cholescintigraphy in evaluating diffuse liver parenchymal diseases

    Itoh, Hisao; Shimono, Reiko; Hamamoto, Ken; Ohshima, Kanji; Akamatsu, Koichi

    1988-01-01

    Technetium-99m N-pyridoxyl-5-methyltryptophan (PMT) cholescintigraphy has been performed in 46 consecutive patients with diffuse liver parenchymal diseases, including acute hepatitis (9), chronic hepatitis (17), and liver cirrhosis (20), and 18 controls. Blood clearance rate, liver uptake rate, liver excretion rate, and half time (T1/2) were determined from cardiac and hepatic time-activity curves. Regarding the four parameters, there were statistically significant differences between the control group and the groups of acute hepatitis and liver cirrhosis. Both blood clearance rate and liver uptake rate were well correlated with ICG-k values (r = 0.874 and r = 0.791, respectively). Liver excretion rate was most highly correlated with total serum bilirubin levels (r = 0.763), followed by ICG-k values. T1/2 was well correlated as well with total serum bilirubin levels. During the process where liver excretory ability was lowered in association with elevated serum bilirubin levels, threshold values for liver excretion rate appeared to be established. Cholescintigraphy may be of value in evaluating the pathophysiology of diffuse liver parenchymal diseases in that it is capable of quantitatively determining excretory function of hepatic cells. (Namekawa, K.)

  11. Motor activation SPECT for the neurosurgical diseases. Clinical application

    Noguchi, Hiroshi; Kawaguchi, Shoichiro; Sakaki, Toshisuke; Imai, Teruhiko; Ohishi, Hajime [Nara Medical Univ., Kashihara (Japan)

    1999-08-01

    We evaluated and analyzed the motor activation single photon emission computed tomography (M-SPECT) findings on patients with ischemic cerebrovascular diseases (CVD). The M-SPECT studies were carried out on 91 patients with ischemic cerebrovascular diseases. The M-SPECT study was performed using the finger opposition task in each case. The SPECT images were superimposed on the magnetic resonance images (MRIs) for each case using Image Fusion Software. The result of the M-SPECT was expressed as positive or negative. The cases with a marked increase of blood flow in the sensorio-motor cortex after the finger opposition task were categorized as positive, and those cases showing no marked increase of blood flow were categorized as negative. Among the 91 cases examined, 53 (58%) were categorized as positive in the M-SPECT study. Among the negative M-SPECT cases treated with revascularization surgery, there were some cases showing positive M-SPECT results postoperatively. The cases without any revascularization surgery did not change the M-SPECT findings in each during the follow-up period. The M-SPECT procedure for examining intracranial lesions could provide the cortical localization of the motor function. The M-SPECT procedure in the ischemic CVDs contributes to knowledge about the choices of treatment and the evaluation of the treatment result. (author)

  12. Unexpected MRI findings in clinically suspected Legg-Calve-Perthes disease

    Lobert, Philip F.; Dillman, Jonathan R.; Strouse, Peter J.; Hernandez, Ramiro J. [University of Michigan Health System, Department of Radiology, Section of Pediatric Radiology, C.S. Mott Children' s Hospital/F3503, Ann Arbor, MI (United States)

    2011-03-15

    In the setting of clinically suspected Legg-Calve-Perthes (LCP) disease and negative/equivocal radiographs, contrast-enhanced MRI can be performed to confirm the diagnosis. To determine the frequency of unexpected causes of hip pain as identified by MRI in children with clinically suspected LCP disease and negative/equivocal radiographs. All pediatric contrast-enhanced MRI examinations of the pelvis and hips performed between January 2000 and February 2009 to evaluate for possible LCP disease in the setting of negative/equivocal radiographs were identified. MRI examinations performed to evaluate for secondary avascular necrosis were excluded. Imaging reports were retrospectively reviewed for unexpected clinically important causes of hip pain. Thirty-six pediatric patients underwent contrast-enhanced MRI examinations for clinically suspected LCP disease in the setting of negative/equivocal radiographs. Twenty-two (61%) imaging studies were normal, while four (11%) imaging studies demonstrated findings consistent with LCP disease. Ten (28%) imaging studies revealed unexpected clinically important causes of hip pain, including nonspecific unilateral joint effusion and synovitis (n = 7, juvenile chronic arthritis was eventually diagnosed in 3 patients), sacral fracture (n = 1), apophyseal injury (n = 1), and femoral head subluxation (n = 1). MRI frequently reveals unexpected clinically important causes of hip pain in children with suspected LCP disease and negative/equivocal radiographs. (orig.)

  13. Clinical considerations in the management of inflammatory periodontal diseases in children and adolescents.

    Cabanilla, Leyvee; Molinari, Gail

    2009-01-01

    Periodontal diseases and conditions, as defined by The 1999 International Workshop for Classification of Periodontal Diseases and Conditions presented some significant paradigm shifts based on evidence that the transition from plaque-associated reversible gingival diseases to periodontitis can occur in children and adolescents with characteristics which were previously thought to be typical of adult periodontitis. The purposes of this paper are to present the periodontal diseases and conditions described in the 1999 workshop sponsored by the American Academy of Periodontology, review the risk factors for the development of periodontal diseases in the pediatric and adolescent populations, and present appropriate clinical periodontal assessment and management for these age groups.

  14. Long-term pruritus as the initial and sole clinical manifestation of occult Hodgkin's disease.

    Omidvari, Shapour H; Khojasteh, Habib Noorani; Mohammadianpanah, Mohammad; Monabati, Ahmad; Mosalaei, Ahmad; Ahmadloo, Niloofar

    2004-06-01

    Pruritus or itch is a frequent symptom of patients with Hodgkin's disease. It often occurs during the clinical course of the disease and rarely may precede the diagnosis of underlying disease. In this report, we present a 16-year-old patient who had history of generalized pruritus without any skin rash for 4 years before the diagnosis of Hodgkin's disease. Within that period, she had received symptom-oriented medications, with no significant effect. After the first cycle of chemotherapy, her pruritus resolved completely. This case suggests that long-term generalized pruritus may be indicative of a significant underlying problem like Hodgkin's disease.

  15. Bridging the Gap between Research and Clinical Practice in Asymptomatic Alzheimer's Disease.

    Downing, A M; Yaari, R; Ball, D E; Selzler, K J; Devous, M D

    2016-01-01

    Due to the growing global health impact of Alzheimer's disease (AD), there is a greater need for interventions that prevent or delay the onset of clinical symptoms of this debilitating disease. Clinical trials for disease-modifying compounds in AD have shifted towards earlier stages in the spectrum of illness, including the stage prior to cognitive symptoms. A population of specific interest for clinical research includes individuals with evidence of Alzheimer's disease pathology who are asymptomatic (ADPa). The challenges and barriers regarding medical treatment of ADPa must be identified and addressed prior to the completion of a positive clinical trial in order to accelerate the translation of research findings to clinical practice. This report applies an existing public health impact model from Spencer and colleagues (2013) to evaluate the readiness of the clinical practice environment to treat ADPa individuals if a disease-modifying agent achieves approval. We contrast the current clinical practice environment with a potential future state through investigating the effectiveness, reach, feasibility, sustainability, and transferability of the practice of treating ADPa individuals.

  16. Clinical outcomes for young people with screening-detected and clinically-diagnosed rheumatic heart disease in Fiji.

    Engelman, Daniel; Mataika, Reapi L; Ah Kee, Maureen; Donath, Susan; Parks, Tom; Colquhoun, Samantha M; Carapetis, Jonathan R; Kado, Joseph H; Steer, Andrew C

    2017-08-01

    Echocardiographic screening is under consideration as a disease control strategy for rheumatic heart disease (RHD). However, clinical outcomes of young people with screening-detected RHD are unknown. We aimed to describe the outcomes for a cohort with screening-detected RHD, in comparison to patients with clinically-diagnosed RHD. A retrospective cohort study included all young people with screening-detected RHD in the Central Division of Fiji in the primary cohort. Screen-negative and clinically-diagnosed comparison groups were matched 1:1 to the primary cohort. Data were collected on mortality, clinical complications and healthcare utilisation from the electronic and paper health records and existing databases. Seventy participants were included in each group. Demographic characteristics of the groups were similar (median age 11years, 69% female, median follow-up 7years). There were nine (12.9%) RHD-related deaths in the clinically-diagnosed group and one (1.4%) in the screening-detected group (Incident Rate Ratio: 9.6, 95% CI 1.3-420.6). Complications of RHD were observed in 39 (55.7%) clinically-diagnosed cases, four (20%) screening-detected cases and one (1.4%) screen-negative case. There were significant differences in the cumulative complication curves of the groups (pFiji. The prognosis of clinically-diagnosed RHD remains poor, with very high mortality and complication rates. Further studies in other settings will inform RHD screening policy. Comprehensive control strategies are required for disease prevention. Copyright © 2017 Elsevier B.V. All rights reserved.

  17. HLA-A02:01-restricted epitopes identified from the herpes simplex virus tegument protein VP11/12 preferentially recall polyfunctional effector memory CD8+ T cells from seropositive asymptomatic individuals and protect humanized HLA-A*02:01 transgenic mice against ocular herpes.

    Srivastava, Ruchi; Khan, Arif A; Spencer, Doran; Vahed, Hawa; Lopes, Patricia P; Thai, Nhi Thi Uyen; Wang, Christine; Pham, Thanh T; Huang, Jiawei; Scarfone, Vanessa M; Nesburn, Anthony B; Wechsler, Steven L; BenMohamed, Lbachir

    2015-03-01

    The HSV type 1 tegument virion phosphoprotein (VP) 11/12 (VP11/12) is a major Ag targeted by CD8(+) T cells from HSV-seropositive individuals. However, whether and which VP11/12 epitope-specific CD8(+) T cells play a role in the "natural" protection seen in seropositive healthy asymptomatic (ASYMP) individuals (who have never had clinical herpes disease) remain to be determined. In this study, we used multiple prediction computer-assisted algorithms to identify 10 potential HLA-A*02:01-restricted CD8(+) T cell epitopes from the 718-aa sequence of VP11/12. Three of 10 epitopes exhibited high-to-moderate binding affinity to HLA-A*02:01 molecules. In 10 sequentially studied HLA-A*02:01-positive and HSV-1-seropositive ASYMP individuals, the most frequent, robust, and polyfunctional effector CD8(+) T cell responses, as assessed by a combination of tetramer frequency, granzyme B, granzyme K, perforin, CD107(a/b) cytotoxic degranulation, IFN-γ, and multiplex cytokines assays, were predominantly directed against three epitopes: VP11/1266-74, VP11/12220-228, and VP11/12702-710. Interestingly, ASYMP individuals had a significantly higher proportion of CD45RA(low)CCR7(low)CD44(high)CD62L(low)CD27(low)CD28(low)CD8(+) effector memory CD8(+) T cells (TEMs) specific to the three epitopes, compared with symptomatic individuals (with a history of numerous episodes of recurrent ocular herpetic disease). Moreover, immunization of HLA-A*02:01 transgenic mice with the three ASYMP CD8(+) TEM cell epitopes induced robust and polyfunctional epitope-specific CD8(+) TEM cells that were associated with a strong protective immunity against ocular herpes infection and disease. Our findings outline phenotypic and functional features of protective HSV-specific CD8(+) T cells that should guide the development of an effective T cell-based herpes vaccine. Copyright © 2015 by The American Association of Immunologists, Inc.

  18. Clinical Features and Outcome of Ebola Virus Disease in Pediatric Patients

    Damkjær, Mads; Rudolf, Frauke; Mishra, Sharmistha

    2016-01-01

    Clinical and outcome data on pediatric Ebola virus disease are limited. We report a case-series of 33 pediatric patients with Ebola virus disease in a single Ebola Treatment Center in 2014-2015. The case-fatality rate was 42%, with the majority of deaths occurring within 10 days of admission....

  19. Clinical and biochemical observations in a patient with combined Pompe disease and cblC mutation

    Wijburg, F. A.; Rosenblatt, D. S.; Vos, G. D.; Oorthuys, J. W.; van't Hek, L. G.; Poorthuis, B. J.; Sanders, M. K.; Schutgens, R. B.

    1992-01-01

    Metabolic studies are described in a patient who presented at 3 weeks of age with severe anaemia, hyperbilirubinaemia and hypotonicity. Clinically, glycogen storage disease type II (Pompe disease) was suspected because of a massively enlarged heart and hepatosplenomegaly. This was confirmed

  20. The hidden Niemann-Pick type C patient : Clinical niches for a rare inherited metabolic disease

    Hendriksz, Christian J.; Anheim, Mathieu; Bauer, Peter; Bonnot, Olivier; Chakrapani, Anupam; Corvol, Jean-Christophe; de Koning, Tom J.; Degtyareva, Anna; Dionisi-Vici, Carlo; Doss, Sarah; Duning, Thomas; Giunti, Paola; Iodice, Rosa; Johnston, Tracy; Kelly, Dierdre; Kluenemann, Hans-Hermann; Lorenzl, Stefan; Padovani, Alessandro; Pocovi, Miguel; Synofzik, Matthis; Terblanche, Alta; Bergh, Florian Then; Topcu, Meral; Tranchant, Christine; Walterfang, Mark; Velten, Christian; Kolb, Stefan A.

    2017-01-01

    Background: Niemann-Pick disease type C (NP-C) is a rare, inherited neurodegenerative disease of impaired intracellular lipid trafficking. Clinical symptoms are highly heterogeneous, including neurological, visceral, or psychiatric manifestations. The incidence of NP-C is under-estimated due to

  1. Clinical significance of determination of serum TRAb levels in patients with relapsing graves' disease

    Guo Chunlei; Zhou Jiaqiang; Li Wenpeng

    2004-01-01

    Objective: To explore the clinical significance of changes of serum TRAb levels in patients with relapsing Graves' disease. Methods: Serum TRAb (with RRA) and several other thyroid-related hormones (TT 4 , TT 3 , TSH, FT 3 , with CLIA) were determined in the following subjects: 1. 25 cases of relapsing Graves' disease after previous successful treatment; 2. 18 cases of recently diagnosed Graves' disease; 3. 31 cases of successfully treated Graves' disease; 4. 15 cases of simple goiter; 5. 10 cases of nodular goiter; 6. 18 cases of hypothyroidism due to Hashimoto disease. Results: Positive rate of TRAb was 76.00% in patients with relapsing Graves' disease and 77.78% in recently diagnosed Graves' disease cases, both being significantly higher than that in all the other sets of patients studied (P<0.01). Conclusion: Determination of serum TRAb levels was helpful for the diagnosis of relapse in Graves' disease

  2. Evolocumab and Clinical Outcomes in Patients with Cardiovascular Disease

    Sabatine, Marc S; Giugliano, Robert P; Keech, Anthony C

    2017-01-01

    -blind, placebo-controlled trial involving 27,564 patients with atherosclerotic cardiovascular disease and LDL cholesterol levels of 70 mg per deciliter (1.8 mmol per liter) or higher who were receiving statin therapy. Patients were randomly assigned to receive evolocumab (either 140 mg every 2 weeks or 420 mg...... death, myocardial infarction, or stroke. The median duration of follow-up was 2.2 years. RESULTS: At 48 weeks, the least-squares mean percentage reduction in LDL cholesterol levels with evolocumab, as compared with placebo, was 59%, from a median baseline value of 92 mg per deciliter (2.4 mmol per liter......) to 30 mg per deciliter (0.78 mmol per liter) (P

  3. Fatigue in Parkinson's disease: concepts and clinical approach.

    Nassif, Daniel V; Pereira, João S

    2018-03-01

    Parkinson's disease (PD) is a progressive neurological disorder characterized by a large number of motor and non-motor features. Fatigue is one of the most common and most disabling symptoms among patients with PD, and it has a significant impact on their quality of life. Although fatigue has been recognized for a long time, its pathophysiology remains poorly understood, and there is no evidence to support any therapeutic approach in PD patients. Expert consensus on case definition and diagnostic criteria for PD-related fatigue have been recently published, and although they still need to be adequately validated, they provide a great step forward in the study of fatigue. The goal of this article is to provide relevant information for the identification and management of patients with fatigue. © 2018 Japanese Psychogeriatric Society.

  4. Von Willebrand's disease in the German shepherd dog : clinical communication

    R.G. Lobetti

    2000-07-01

    Full Text Available Two litters of Germanshepherd dogs were evaluated for a haemorrhagic tendency that was characterised by excessive bleeding from the umbilicus at birth, haemorrhage and haematoma formation at vaccination, excessive bruising, and lameness due to haemarthrosis. Platelet counts, clotting times and Von Willebrand's factor (VWF assays were assessed in all dogs. Factor VIII determination was performed in 1 puppy and its parents. Based on the clotting times and VWF assay, 6 puppies (4 male and 2 female showed type I Von Willebrand's disease (VWD, 5 (4 male and 1 female possible type II VWD, and 4 were unaffected. One puppy with possible type II VWD had very low factor VIII activity; its sire had a normal factor activity, whereas the dam was in the low-normal range. This article reports type I and possible type II VWD in 2 related litters of German shepherd dogs, the latter being rare in German shepherd dogs.

  5. Late-onset Pompe disease: first clinical description in Russia

    S. S. Nikitin

    2014-01-01

    Full Text Available Late-onset Pompe-disease (LOPD is an adult form of the glycogenosis type II. The age of onset ranges from 1 till 75 y.o. and older. The diagnosis of LOPD is based on the presence of trunk and limb-girdle muscle weakness with hyperlordosis, respiratory failure, ocasionally accompanied by cardiomyopathy, persistent mild elevation of creatine kinase, dry blood spot test of the enzyme activity and DNA-analysis of GAA-gene. Early recognition of the LOPD and beginning of the enzyme replacement therapy is important in preventing severe motor and respiratory deficit, the patient disability and in increasing the survival in those patients.

  6. [Psychocardiology: clinically relevant recommendations regarding selected cardiovascular diseases].

    Albus, C; Ladwig, K-H; Herrmann-Lingen, C

    2014-03-01

    Psychosocial risk factors (work stress, low socioeconomic status, impaired social support, anger, anxiety and depression), certain personality traits (e.g. hostility) and post-traumatic stress disorders may negatively influence the incidence and course of multiple cardiovascular disease conditions. Systematic screening for these factors may help to adequately assess the psychosocial risk pattern of a given patient and may also contribute to the treatment of these patients. Recommendations for treatment are based on current guidelines. The physician-patient interaction should basically follow the principle of a patient centered communication and should gender and age specific aspects into consideration. Integrated biopsychosocial care is an effective, low threshold option to treat psycho-social risk factors and should be offered on a regular basis. Patients with high blood pressure may profit from relaxation programs and biofeedback procedures (however with moderate success). An individually adjusted multimodal treatment strategy should be offered to patients with coronary heart disease, heart failure and after heart surgery. It may incorporate educational tools, exercise therapy, motivational modules, relaxation and stress management programs. In case of affective comorbidity, psychotherapy may be indicated. Anti-depressant pharmacotherapy with selective serotonin reuptake inhibitors (SSRIs) in the first line should only be offered to patients with at least moderate severe depressive episodes. Psychotherapy and SSRIs, particularly sertraline, have been proven to be safe and effective with regard to improvements of the patient's quality of life. A prognostic benefit has not been clearly proven so far. Patients with an implanted cardioverter/defibrillator (ICD) should receive psychosocial support on a regular basis. Concomitant psychotherapy and/or psychopharmacotherapy (SSRIs) should be offered in case of a severe mental comorbidity. Generally, tricyclic

  7. Norrie disease resulting from a gene deletion: clinical features and DNA studies.

    Donnai, D; Mountford, R C; Read, A P

    1988-01-01

    We describe a family in which two boys with Norrie disease have a deletion of the DXS7 locus. The deletion does not extend as far distally as the OTC or DXS84 loci. A full clinical description of the patients is given to help establish the range of manifestations of Norrie disease. There is no evidence of any other X linked disease in our patients.

  8. Norrie disease resulting from a gene deletion: clinical features and DNA studies.

    Donnai, D; Mountford, R C; Read, A P

    1988-02-01

    We describe a family in which two boys with Norrie disease have a deletion of the DXS7 locus. The deletion does not extend as far distally as the OTC or DXS84 loci. A full clinical description of the patients is given to help establish the range of manifestations of Norrie disease. There is no evidence of any other X linked disease in our patients.

  9. Extramammary Paget disease: review of patients seen in a non-melanoma skin cancer clinic.

    Pang, J; Assaad, D; Breen, D; Fialkov, J; Antonyshyn, O; Balogh, J; Tsao, M; Kamra, J; Czarnota, G; Barnes, E A

    2010-10-01

    Extramammary Paget disease (EMPD) is a rare skin disease commonly found in the anogenital region. In this study, we aimed to identify EMPD patients seen in the non-melanoma skin cancer clinic at Odette Cancer Centre and to describe the treatments delivered and outcomes achieved. From 2000 to 2009, 14 patients were seen. Initial treatment recommendations included imiquimod and surgical excision, although half the patients required more than one treatment modality, highlighting the difficulty of achieving complete eradication of this disease.

  10. Development and clinical course of diseases accompanied by connective tissue dysplasia in children of puberty age

    Elizarova S.Yu.

    2011-03-01

    Full Text Available The risk of development and clinical course of somatic diseases have been analyzed in the research work. 111 adolescents suffering from connective tissue dysplasia have been under the study. It has been stated that the frequency of somatic diseases among adolescents with connective tissue dysplasia is higher than this frequency among adolescents without such disease. Phenotypic signs of connective tissue dysplasia have been revealed. They are responsible for the development of bronchial asthma and severe stomach ulcer

  11. Power analysis to detect treatment effects in longitudinal clinical trials for Alzheimer's disease.

    Huang, Zhiyue; Muniz-Terrera, Graciela; Tom, Brian D M

    2017-09-01

    Assessing cognitive and functional changes at the early stage of Alzheimer's disease (AD) and detecting treatment effects in clinical trials for early AD are challenging. Under the assumption that transformed versions of the Mini-Mental State Examination, the Clinical Dementia Rating Scale-Sum of Boxes, and the Alzheimer's Disease Assessment Scale-Cognitive Subscale tests'/components' scores are from a multivariate linear mixed-effects model, we calculated the sample sizes required to detect treatment effects on the annual rates of change in these three components in clinical trials for participants with mild cognitive impairment. Our results suggest that a large number of participants would be required to detect a clinically meaningful treatment effect in a population with preclinical or prodromal Alzheimer's disease. We found that the transformed Mini-Mental State Examination is more sensitive for detecting treatment effects in early AD than the transformed Clinical Dementia Rating Scale-Sum of Boxes and Alzheimer's Disease Assessment Scale-Cognitive Subscale. The use of optimal weights to construct powerful test statistics or sensitive composite scores/endpoints can reduce the required sample sizes needed for clinical trials. Consideration of the multivariate/joint distribution of components' scores rather than the distribution of a single composite score when designing clinical trials can lead to an increase in power and reduced sample sizes for detecting treatment effects in clinical trials for early AD.

  12. Clinical management of ebola virus disease in the United States and Europe

    Uyeki, Timothy M.; Mehta, Aneesh K.; Davey, Richard T.; Liddell, Allison M.; Wolf, Timo; Vetter, Pauline; Schmiedel, Stefan; Grünewald, Thomas; Jacobs, Michael; Arribas, Jose R.; Evans, Laura; Hewlett, Angela L.; Brantsaeter, Arne B.; Ippolito, Giuseppe; Rapp, Christophe; Hoepelman, Andy I M; Gutman, Julie

    2016-01-01

    Background Available data on the characteristics of patients with Ebola virus disease (EVD) and clinical management of EVD in settings outside West Africa, as well as the complications observed in those patients, are limited. METHODS We reviewed available clinical, laboratory, and virologic data

  13. Granulomatous prostatitis: clinical and histomorphologic survey of the disease in a tertiary care hospital

    Prakriti Shukla

    2017-03-01

    Conclusion: Despite present-day advances in imaging modalities and serological investigations, it is virtually impossible to identify granulomatous prostatitis clinically. Histopathology remains the gold standard in diagnosing the disease. However, assigning an etiologic cause to the wide spectrum of granulomas in granulomatous prostatitis requires a pathologist’s expertise and proper clinical correlation for appropriate patient management.

  14. Methodology of clinical measures of healthcare quality delivered to patients with cardiovascular diseases

    Posnenkova O.M.

    2014-03-01

    Full Text Available The results of implementation the methodology proposed by American Colleague of Cardiology and American Heart Association (ACC/AHA for development of Russian clinical quality measures for patients with arterial hypertension, coronary heart disease and chronic heart failure. Created quality measures cover the key elements of medical care influencing directly on clinical outcomes of treatment.

  15. Effectiveness of donepezil in reducing clinical worsening in patients with mild-to-moderate alzheimer's disease

    Wilkinson, David; Schindler, Rachel; Schwam, Elias

    2009-01-01

    BACKGROUND: Therapeutic endpoints based on reduced clinical worsening represent clinically relevant and realistic goals for patients suffering from progressive neurodegenerative disorders such as Alzheimer's disease (AD). METHODS: Data from 906 patients (388 receiving placebo; 518 receiving...... of declining were significantly reduced for donepezil-treated versus placebo patients (p treatment...

  16. Two Babinski signs in seropositive (HAM and seronegative tropical spastic paraparesis Dos signos de Babinski en pacientes con paraparesia espástica tropical seropositiva (HAM y seronegativa

    Fidias E. Leon-Sarmiento

    2008-01-01

    Full Text Available Tropical spastic paraparesis (TSP may or may not be associated to HTLV-I antibodies and is usually characterized by clinical and pathological spinal cord abnormalities at thoracic levels. We present here five Brazilian patients who had typical chronic idiopatic spastic paraparesis; two of them were HTLV-I seropositive (HAM and three HTLV-I seronegative (TSP - associated-myelopathy. Three out of these five patients also displayed clinical supraspinal involvement, indeed, platysma muscle hypotrophy or atrophy (the Babinski plus sign. These findings support the view that clinical involvement in HAM and TSP is wider than the spinal cord abnormalities usually considered. Possible non-infectious co-factors (e.g., mycotoxins may be involved in disease pathogenesis in a multistep process of viruses, toxins and environment which may account for serological differences found in this group of patients.La paraparesia espástica tropical (PET, puede o no estar asociada con anticuerpos contra el HTLV-I y se caracteriza, usualmente, por alteraciones clínicas y patológicas a nivel de region dorso-lumbar de la medula espinal. Presentamos cinco pacientes brasileros, quienes tuvieron hallazgos típicos de paraparesia espástica crónica idiopática; dos de ellos tuvieron (HAM y tres no tuvieron (TSP anticuerpos, en el suero, contra el HTLV-I. En tres pacientes se encontró hipotrofia o atrofia del músculo platisma (signo de Babinski plus, demostrando que el compromiso clínico en pacientes con HAM y TSP se extiende más allá de la médula espinal torácica. Cofactores (por ejemplo, micotoxinas podrían estar involucrados en la patogénesis de esta enfermedad, en una interacción compleja de virus, toxinas y medio ambiente, lo cual explicaría las diferencias serológicas encontradas en este grupo de pacientes.

  17. miRNAs in inflammatory skin diseases and their clinical implications

    Løvendorf, Marianne B; Skov, Lone

    2015-01-01

    biological processes. The clinical implications of miRNAs are intriguing, both from a diagnostic and a therapeutic perspective. Accordingly, there is emerging evidence for the clinical potential of miRNAs as both biomarkers and possible therapeutic targets in skin diseases. Future studies will hopefully...... incomplete; however, it is known that miRNAs are implicated in various cellular processes of both normal and diseased skin. Some miRNAs appear to be consistently deregulated in several different inflammatory skin diseases, including psoriasis and atopic dermatitis, indicating a common role in fundamental...

  18. Stargardt disease: clinical features, molecular genetics, animal models and therapeutic options

    Tanna, Preena; Strauss, Rupert W; Fujinami, Kaoru; Michaelides, Michel

    2017-01-01

    Stargardt disease (STGD1; MIM 248200) is the most prevalent inherited macular dystrophy and is associated with disease-causing sequence variants in the gene ABCA4. Significant advances have been made over the last 10 years in our understanding of both the clinical and molecular features of STGD1, and also the underlying pathophysiology, which has culminated in ongoing and planned human clinical trials of novel therapies. The aims of this review are to describe the detailed phenotypic and genotypic characteristics of the disease, conventional and novel imaging findings, current knowledge of animal models and pathogenesis, and the multiple avenues of intervention being explored. PMID:27491360

  19. Clinical significance of measurements of serum IL-6 levels in patients with cardiovascular and cerebrovascular diseases

    Wu Cuihua; Luo Nanping; Zhang Daojie; Wei Hong

    2004-01-01

    Objective: To explore the clinical significance of changes of serum IL-6 levels in patients with cardiovascular and cerebrovascular diseases. Methods: Serum IL-6 levels were determined with RIA in 35 patients with coronary heart disease, 20 patients with essential hypertension, 28 patients with cerebral infarction and 30 controls. Results: Serum IL-6 levels in patients with coronary heart disease and cerebral infarction were significantly higher than those in the controls (P 0.05). Conclusion: Serum IL-6 levels changes could reflect the severity of the inflammatory process and would be helpful in clinical assessment. (authors)

  20. IgG4-Related Disease: Baseline clinical and laboratory features in 125 patients with biopsy-proven disease

    Wallace, Zachary S.; Deshpande, Vikram; Mattoo, Hamid; Mahajan, Vinay S.; Kulikova, Maria; Pillai, Shiv; Stone, John H.

    2015-01-01

    Purpose IgG4-related disease (IgG4-RD) is an immune-mediated fibroinflammatory condition that can affect nearly any organ. No detailed clinical and laboratory assessments have been reported in large numbers of patients with IgG4-RD diagnoses established by strict clinicopathological correlation. Methods We reviewed the baseline features of 125 patients with biopsy-proven disease. The diagnosis was confirmed by pathology review according to consensus diagnostic criteria. Disease activity and damage were assessed by the IgG4-RD Responder Index (RI). Flow cytometry was used to assess levels of circulating plasmablasts. Results Of the 125 patients, 103 had active disease and 86 were on no treatment. Only 51% of the patients with active disease had elevated serum IgG4 concentrations. However, patients with active disease and elevated serum IgG4 concentrations were older, had a higher RI, a greater number of organs involved, lower complement levels, higher absolute eosinophil counts, and higher IgE levels compared to those with active disease but normal serum IgG4 (PIgG4+ plasmablast level and RI (R=0.45, P=0.003) was stronger than that of total plasmablasts and RI. Seventy-six (61%) of the patients were male, but no significant differences according to gender were observed with regard to disease severity, organ involvement, or serum IgG4 concentrations. Glucocorticoids failed to produce sustained remission in the majority of patients. Conclusion Nearly 50% of this patient cohort with biopsy-proven, clinically-active IgG4-RD had normal serum IgG4 concentrations. Serum IgG4 elevation identify a subset with more inflammatory features. IgG4+ plasmablasts correlate well with disease activity. PMID:25988916