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Sample records for systemic onset juvenile

  1. Experience of the Tocilizumab Application in Systemic Onset Juvenile Arthritis

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    A. V. Krasnopol’skaja

    2015-01-01

    Full Text Available The article provides information on the unfavourable course of systemic onset juvenile arthritis, resistant to immunosuppressive therapy with methotrexate in combination with cyclosporine, and pulse therapy with methylprednisolone and methotrexate. We describe the successful use of genetically engineered biological drug tocilizumab in the patient with systemic onset juvenile arthritis. After the first injection, pain was already significantly reduced; after the second, fever was relieved and non-steroid anti-inflammatory drugs were cancelled; after the third, lymphadenopathy and splenomegaly disappeared and the child’s functional activity improved significantly. After 12 months of treatment, an inactive phase of the disease was achieved, the joints’ kinetics (with the exception of the right hip were almost entirely restored and the patient’s quality of life had significantly improved. At the same time, metabolic disorders and changes in the cardiovascular system were reversed. This example demonstrated the high effectiveness of interleukin-6 antagonist tocilizumab in systemic arthritis, which allowed arresting joint affection as well as extra-articular manifestations of the disease, providing normal puberty, the restoration of growth and sexual development.

  2. A comparison between nailfold capillaroscopy patterns in adulthood in juvenile and adult-onset systemic sclerosis

    DEFF Research Database (Denmark)

    Ingegnoli, Francesca; Boracchi, Patrizia; Gualtierotti, Roberta

    2015-01-01

    OBJECTIVE: Qualitative capillaroscopy patterns in juvenile- and adult-onset systemic sclerosis (SSc) were studied in adulthood using data from the EULAR Scleroderma Trials and Research (EUSTAR) database. METHODS: Data collected between June 2004 and April 2013 were examined with focus on capillar......OBJECTIVE: Qualitative capillaroscopy patterns in juvenile- and adult-onset systemic sclerosis (SSc) were studied in adulthood using data from the EULAR Scleroderma Trials and Research (EUSTAR) database. METHODS: Data collected between June 2004 and April 2013 were examined with focus....... Juvenile-onset SSc showed scleroderma pattern more frequently than adult-onset SSc (93.3% and 88%). The OR was 2.44 and 95% CI 0.57-10.41. An active scleroderma pattern was present in 58% of juvenile- and 61% of adult-onset SSc. The OR was 0.91 and 95% CI 0.28-2.93. The late scleroderma pattern was present...... in 61% of juvenile- and 55.5% of adult-onset SSc. The OR was 1.06 and 95% CI 0.34-3.56. CONCLUSION: This is the first exploratory study on the comparison of capillaroscopy between juvenile- and adult-onset SSc in adulthood. Juvenile-onset SSc had an increase prevalence of scleroderma pattern...

  3. Macrophage activation syndrome in a patient with systemic onset of the juvenile idiopathic arthritis.

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    Jain, Deepak; Aggarwal, Hari K; Rao, Avinash; Mittal, Anshul; Jain, Promil

    2016-01-01

    Systemic onset juvenile idiopathic arthritis (sJIA) is defined as arthritis affecting one or more joint usually in the juvenile age group (< 16 years of age) with or preceded by fever of at least 2 weeks duration that is documented to be daily ("quotidian") for at least 3 days which may be associated with evanescent (non-fixed) erythematous rash or generalized lymph node enlargement or hepatomegaly/splenomegaly/both or serositis. Macrophage activation syndrome (MAS) is a life-threatening complication of sJIA marked by sudden onset of non-remitting high fever, profound depression in all three blood cell lines (i.e. leukopenia, anemia, and thrombocytopenia), hepatosplenomegaly, lymphadenopathy, and elevated serum liver enzyme levels. In children with systemic juvenile idiopathic arthritis, the clinical picture may mimic sepsis or an exacerbation of the underlying disease. We report a case of a 16-year-old female patient presenting with high grade fever with joint pains and generalized weakness which proved to be systemic onset juvenile idiopathic arthritis with macrophage activation syndrome after ruling out all other differential diagnoses and responded well to intravenous steroids.

  4. Mucocutaneous manifestations in juvenile-onset systemic lupus erythematosus: a review of literature

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    Chiewchengchol, Direkrit; Murphy, Ruth; Edwards, Steven W; Beresford, Michael W

    2015-01-01

    Patients diagnosed with juvenile-onset systemic lupus erythematosus (JSLE) often have skin and oral lesions as part of their presentation. These mucocutaneous lesions, as defined by the American College of Rheumatology (ACR) in 1997, include malar rash, discoid rash, photosensitivity and oral ulcers. It is therefore essential to recognize mucocutaneous lesions to accurately diagnose JSLE. The mucocutaneous lesions can be divided into those with classical histological features (LE specific) an...

  5. Oral Ulcers in Juvenile-Onset Systemic Lupus Erythematosus: A Review of the Literature

    OpenAIRE

    Rodsaward, Pongsawat; Prueksrisakul, Titipong; Deekajorndech, Tawatchai; Edwards, Steven W.; Beresford, Michael W.; Chiewchengchol, Direkrit

    2017-01-01

    Oral ulcers are the most common mucosal sign in juvenile-onset systemic lupus erythematosus (JSLE). The ulcers are one of the key clinical features; however, the terminology of oral ulcers, especially in JSLE patients, is often vague and ill-defined. In fact, there are several clinical manifestations of oral ulcers in JSLE, and some lesions occur when the disease is active, indicating that early management of the disease should be started. Oral ulcers are classified as lupus erythematosus (LE...

  6. Juvenile systemic lupus erythematosus onset patterns in Vietnamese children

    DEFF Research Database (Denmark)

    Dung, Nguyen Thi Ngoc; Loan, Huynh Thoai; Nielsen, Susan

    2013-01-01

    to have systemic lupus erythematosus (f/m = 4/1) were referred to the Ho Chi Minh City Children's Hospital No.1 during a 12-month period in 2009. RESULTS: The mean age at diagnosis was 12.8 years (SD = 2.5). Thirty-seven (82%) fulfilled criteria for lupus nephritis (LN). At diagnosis, impressively high...... No. 1 during a16 month period from 2008-2009. These patients had a strikingly high prevalence of Coombs positive anaemia, a high prevalence of lupus nephritis, and very high SLEDAI and ECLAM scores at the time of diagnosis. While there may be referral biases, our Vietnamese SLE patients appear...

  7. Late Onset Juvenile Xanthogranuloma

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    Punithwavathy K

    1999-01-01

    Full Text Available A 19 year old female was seen with multiple skin coloured and hyperpigmented macules, discrete as well as grouped papules and nodules of varying sizes distributed over the face, neck, extensor and flexor aspects of both upper and lower extremities including joints. The trunk was spared. Some of the lesions showed features of spontaneous regression. Investigations confirmed the diagnosis of juvenile xanthogranuloma. Lesions regressed satisfactorily with liquid nitrogen cryotherapy.

  8. Oral Ulcers in Juvenile-Onset Systemic Lupus Erythematosus: A Review of the Literature.

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    Rodsaward, Pongsawat; Prueksrisakul, Titipong; Deekajorndech, Tawatchai; Edwards, Steven W; Beresford, Michael W; Chiewchengchol, Direkrit

    2017-12-01

    Oral ulcers are the most common mucosal sign in juvenile-onset systemic lupus erythematosus (JSLE). The ulcers are one of the key clinical features; however, the terminology of oral ulcers, especially in JSLE patients, is often vague and ill-defined. In fact, there are several clinical manifestations of oral ulcers in JSLE, and some lesions occur when the disease is active, indicating that early management of the disease should be started. Oral ulcers are classified as lupus erythematosus (LE) specific, where the lesional biopsy shows a unique pattern of mucosal change in LE, and LE nonspecific, where the ulcers and their histopathological findings can be found in other oral diseases. Here, the clinical manifestations, diagnosis and management of oral ulcers in JSLE patients are reviewed.

  9. Mucocutaneous manifestations in juvenile-onset systemic lupus erythematosus: a review of literature.

    Science.gov (United States)

    Chiewchengchol, Direkrit; Murphy, Ruth; Edwards, Steven W; Beresford, Michael W

    2015-01-01

    Patients diagnosed with juvenile-onset systemic lupus erythematosus (JSLE) often have skin and oral lesions as part of their presentation. These mucocutaneous lesions, as defined by the American College of Rheumatology (ACR) in 1997, include malar rash, discoid rash, photosensitivity and oral ulcers. It is therefore essential to recognize mucocutaneous lesions to accurately diagnose JSLE. The mucocutaneous lesions can be divided into those with classical histological features (LE specific) and those strongly associated with and forming part of the diagnostic spectrum, but without the classical histological changes of lupus (LE nonspecific). A malar rash is the most commonly associated LE specific dermatological presentation. This skin manifestation is an acute form and also correlates with disease activity. Subacute (polycyclic or papulosquamous lesions) and chronic (discoid lesions) forms, whilst showing classical histological changes supportive of lupus, are less commonly associated with systemic lupus and do not correlate with disease activity. The most commonly associated skin lesions without classical lupus changes are cutaneous vasculitis, oral ulcers and diffuse non-scarring alopecia. These signs frequently relate to disease activity. An understanding of cutaneous signs and symptoms of lupus in children is important to avoid delay in diagnosis. They will often improve as lupus is adequately controlled and their reappearance is often the first indicator of a disease flare.

  10. Coronary artery abnormalities in children with systemic-onset juvenile idiopathic arthritis.

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    Lefèvre-Utile, Alain; Galeotti, Caroline; Koné-Paut, Isabelle

    2014-05-01

    Still's disease (Systemic-onset Juvenile Idiopathic Arthritis: SoJIA) is characterised by high-spiking daily fevers, arthritis and evanescent rashes. Diagnosis of Still's disease is often challenging. Infectious diseases and other inflammatory conditions, especially in young children, Kawasaki disease may look similar. Clinicians often rely on echocardiographic evidence of coronary artery abnormalities to differentiate between Kawasaki disease and Still's disease. Coronary artery dilation would typically favour the diagnosis of Kawasaki disease. We present four children with Still's disease and coronary artery abnormalities who were initially misdiagnosed as Kawasaki disease. The first patient had pericarditis and an irregular wall of the left coronary artery, without dilation on echocardiography. The second patient had a left coronary artery dilatation and a pericarditis. The third patient had thickened left coronary artery walls, and the fourth patient had a hyperechogenicity of the left and right coronary arteries. They received IVIG without success. The diagnosis of Still's disease was made secondary with evidence of persistent arthritis. All but one patient finally needed biologic treatments. Coronary abnormalities may be observed during various febrile conditions and do not exclude the diagnosis of Still's disease. Copyright © 2013 Société française de rhumatologie. Published by Elsevier SAS. All rights reserved.

  11. Comparison of clinical and serological differences among juvenile-, adult-, and late-onset systemic lupus erythematosus in Korean patients.

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    Choi, J H; Park, D J; Kang, J H; Yim, Y R; Lee, K E; Lee, J W; Wen, L; Kim, T J; Park, Y W; Lee, J K; Lee, S S

    2015-10-01

    We investigated whether systemic lupus erythematosus (SLE) patients could be distinguished based on the time of disease onset and, if so, whether the groups differed in their clinical and laboratory features in ethnically homogeneous Korean patients. We enrolled 201 SLE patients with available clinical data at the time of onset of SLE from the lupus cohort at Chonnam National University Hospital. Sociodemographic, clinical, and laboratory data, including autoantibodies, and concomitant diseases were found at the time of diagnosis of SLE by reviewing patient charts. We divided SLE patients according to age at SLE diagnosis into three groups: juvenile-onset SLE (JSLE, diagnosed at ≤ 18 years), adult-onset SLE (ASLE, diagnosed at 19-50 years), and late-onset SLE (LSLE, diagnosed at >50 years), and compared baseline demographic, clinical, and relevant laboratory findings. Of the 201 patients, 27 (14.4%), 149 (74.1%), and 25 (12.4%) were JSLE, ASLE, and LSLE patients, respectively. Fever, oral ulcers, nephritis, anemia, and thrombocytopenia were more common in JSLE patients than ASLE or LSLE patients (p manifestations according to age at disease onset. JSLE patients have more severe disease activity and more frequent renal involvement and LSLE patients have milder disease activity, more commonly accompanied by Sjögren's syndrome, at disease onset. © The Author(s) 2015.

  12. JUVENILE-ONSET SYSTEMIC LUPUS ERYTHEMATOSUS: CLINICAL FEATURES AND CURRENT DIAGNOSTIC APPROACHES

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    M. I. Kaleda

    2017-01-01

    Full Text Available Systemic lupus erythematosus (SLE is an autoimmune disease with various organs and systems involved in the pathological process, with an unpredictable course, a risk for life-threatening conditions, and an onset from childhood through adolescence in 10–20% of cases. The onset of SLE prior to adult age affects its clinical manifestations, course, and prognosis. Based on the data available in the literature, the paper analyzes the features of the onset and clinical presentations of SLE in children and adolescents and discusses the aspects of verifying its diagnosis, by taking into account the need to use early effective personalized therapy and to improve prognosis.

  13. Validation of the systemic lupus erythematosus responder index for use in juvenile-onset systemic lupus erythematosus.

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    Mina, Rina; Klein-Gitelman, Marisa S; Nelson, Shannen; Eberhard, B Anne; Higgins, Gloria; Singer, Nora G; Onel, Karen; Tucker, Lori; O'Neil, Kathleen M; Punaro, Marilynn; Levy, Deborah M; Haines, Kathleen; Martini, Alberto; Ruperto, Nicolino; Lovell, Daniel; Brunner, Hermine I

    2014-02-01

    This study tested the concurrent validity of the systemic lupus erythematosus responder index (SRI) in assessing improvement in juvenile-onset systemic lupus erythematosus (jSLE). The SRI considers changes in the SELENA-SLEDAI, BILAG and a 3-cm visual analogue scale of physician-rated disease activity (PGA) to determine patient improvement. Using prospectively collected data from 760 unique follow-up visit intervals of 274 jSLE patients, we assessed the sensitivity and specificity of the SRI using these external standards: physician-rated improvement (MD-change), patient/parent-rated major improvement of wellbeing (patient-change) and decrease in prescribed systemic corticosteroids (steroid-change). Modifications of the SRI that considered different thresholds for the SELENA-SLEDAI, BILAG and 10-cm PGA were explored and agreement with the American College of Rheumatology/PRINTO provisional criteria for improvement of jSLE (PCI) was examined. The sensitivity/specificity in capturing major improvement by the MD-change were 78%/76% for the SRI and 83%/78% for the PCI, respectively. There was fair agreement between the SRI and PCI (kappa=0.35, 95% CI 0.02 to 0.73) in capturing major improvement by the MD-change. Select modified versions of the SRI had improved accuracy overall. All improvement criteria tested had lower sensitivity when considering patient-change and steroid-change as external standards compared to MD-change. The SRI and its modified versions based on meaningful changes in jSLE have high specificity but at most modest sensitivity for capturing jSLE improvement. When used as an endpoint of clinical trials in jSLE, the SRI will provide a conservative estimate regarding the efficacy of the therapeutic agent under investigation.

  14. Juvenile systemic lupus erythematosus onset patterns in Vietnamese children: a descriptive study of 45 children

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    Dung Nguyen Thi Ngoc

    2012-11-01

    Full Text Available Abstract Background Incidence and disease pattern of childhood-onset SLE is reported to differ among ethnic groups. Methods To describe disease pattern and 6 month follow-up in a referral based cohort of 45 Vietnamese children with SLE. Forty-five children who were subsequently diagnosed to have systemic lupus erythematosus (f/m = 4/1 were referred to the Ho Chi Minh City Children’s Hospital No.1 during a 12-month period in 2009. Results The mean age at diagnosis was 12.8 years (SD = 2.5. Thirty-seven (82% fulfilled criteria for lupus nephritis (LN. At diagnosis, impressively high SLEDAI and ECLAM scores were recorded (mean and SD, 23.8 (11.6 and 6 (2.3, respectively. The mean renal SLEDAI score was 8.2. The mean haemoglobin (g/dL, SD was 8.5 (2.1. The Coombs test was positive in 30 of 36 children (83%. The mean plasma creatinine was 0.98 (SD 1.2 and mean Westergren sedimentation rate was 83.6 (SD 37.4. The patient age at diagnosis was positively correlated to the SLEDAI (p = 0.034 and ECLAM (p = 0.022. At 6 month follow-up of the 45 children, 15 patients were in complete remission, 5 were in partial remission, 6 had stable disease, 3 had relapsed, 3 had evolving disease, 2 had ongoing resistant disease and 4 had died. Seven patients were lost to follow-up. A second renal biopsy showed an improved ISN class in 13 of 15; in 2 cases the ISN class remained unchanged. Conclusions Forty-five Vietnamese children with SLE were referred to Ho Chi Minh Children’s Hospital No. 1 during a16 month period from 2008–2009. These patients had a strikingly high prevalence of Coombs positive anaemia, a high prevalence of lupus nephritis, and very high SLEDAI and ECLAM scores at the time of diagnosis. While there may be referral biases, our Vietnamese SLE patients appear to have severe disease upon presentation but do reasonably well in the short-term.

  15. Increased Fas and Bcl-2 Expression on Peripheral Blood T and B Lymphocytes from Juvenile-Onset Systemic Lupus Erythematosus, but not from Juvenile Rheumatoid Arthritis and Juvenile Dermatomyositis

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    Bernadete L. Liphaus

    2006-01-01

    Full Text Available Defective regulation of apoptosis may play a role in the development of autoimmune diseases. Fas and Bcl-2 proteins are involved in the control of apoptosis. The aims of this study were to determine the expression of Fas antigen and Bcl-2 protein on peripheral blood T and B lymphocytes from patients with juvenile-onset systemic lupus erythematosus (JSLE, juvenile rheumatoid arthritis (JRA and juvenile dermatomyositis (JDM. Thirty-eight patients with JSLE, 19 patients with JRA, 10 patients with JDM and 25 healthy controls entered the study. Freshly isolated peripheral blood mononuclear cells (PBMC were stained for lymphocyte markers CD3, CD4, CD8, CD19 and for Fas and Bcl-2 molecules. Expressions were measured by three-color flow cytometry. Statistical analysis was performed using Kruskal–Wallis test. Percentages of freshly isolated T lymphocytes positively stained for Fas protein from JSLE patients were significantly increased compared to healthy controls, patients with JRA and patients with JDM. Percentages of B lymphocytes positive for Fas from JSLE patients were higher than healthy controls and JRA patients. In addition, Fas expression on T cells from patients with JRA was increased compared to JDM patients. Otherwise, Fas expression on T and B cells from JRA and JDM patients were similar to healthy controls. MFI of Bcl-2 positive T lymphocytes from JSLE patients were significantly increased compared to healthy controls and JRA patients. MFI of Bcl-2 protein on B lymphocytes from JSLE patients was similar to healthy controls and patients with JRA and JDM. Bcl-2 expression did not differ between JRA and JDM patients and healthy controls. In conclusion, increased expression of Fas and Bcl-2 proteins observed in circulating T and B lymphocytes from patients with JSLE, but not from patients with JRA and JDM, suggests that abnormalities of apoptosis may be related to the pathogenesis of JSLE and probably are not a result of chronic inflammation.

  16. Juvenile-onset hypothyroidism in a dog

    International Nuclear Information System (INIS)

    Greco, D.S.; Peterson, M.E.; Cho, D.Y.; Markovits, J.E.

    1985-01-01

    Juvenile-onset hypothyroidism was diagnosed in an adult mixed-breed dog examined because of quadraparesis. Unusual clinical signs attributable to juvenile-onset or congenital hypothyroidism included disproportionate dwarfism; enlarged, protruding tongue; mental dullness; and retention of a 'puppy' coat, which was soft and fluffy, without guard hairs. Radiography of the vertebral column and long bones revealed multiple areas of delayed epiphyseal closure and epiphyseal dysgenesis. Myelography demonstrated several intervertebral disk protrusions in the cervical and lumbar regions. Hypothyroidism was confirmed on the basis of a low basal serum thyroxine concentration that failed to increase after the administration of thyroid stimulating hormone. Other laboratory abnormalities included nonregenerative, normocytic, normochromic anemia; mild hypercalcemia; and an impaired growth hormone (GH) secretory response after xylazine administration. At necropsy, the thyroid gland was small and weighed only 0.2g. Microscopic examination of the thyroid gland revealed a loss of glandular tissue, which was replaced by adipose tissue along its periphery. Gross or microscopic abnormalities were not noted in the pituitary gland, and immunohistochemical staining of the pituitary gland revealed a normal number of GH-containing acidophils. This suggests that primary hypothyroidism may result in an impaired secretion of growth hormone, and that pituitary dwarfism or GH deficiency may be difficult to differentiate from hypothyroid dwarfism on the basis of provocative GH testing alone

  17. The Myeloid-Related Proteins 8 and 14 Complex, a Novel Ligand of Toll-Like Receptor 4, and Interleukin-1 beta Form a Positive Feedback Mechanism in Systemic-Onset Juvenile Idiopathic Arthritis

    NARCIS (Netherlands)

    Frosch, Michael; Ahlmann, Martina; Vogl, Thomas; Wittkowski, Helmut; Wulffraat, Nico; Foell, Dirk; Roth, Johannes

    Objective. Fever of unknown origin is a diagnostic challenge in children, especially for differentiation of systemic-onset juvenile idiopathic arthritis (systemic-onset JIA) and infectious diseases. We undertook this study to analyze the relevance of myeloid-related proteins (MRPs) 8 and 14,

  18. Social maturation in juvenile onset diabetes.

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    Kokkonen, J; Lautala, P; Salmela, P

    1994-03-01

    We re-examined a group of 82 (36F, 46M) patients with juvenile onset diabetes at the age of 19-25 years and compared their social outcome in early adulthood with that of a group of 211 randomly selected controls. Their basic school achievements and vocational or higher education were covered in detail in an interview which also included data on their employment status. Various descriptors of social situation and social maturation assessed in a separate interview formed a social maturation index. Relations between factors probably affecting social maturation and this index were analysed. The average marks scored by the diabetics in Finnish comprehensive schools were significantly lower than those of the controls. High school, vocational and commercial schools were discontinued more often by the patient group. Diabetics (27%) and controls (35%) continued their studies equally often in various vocational high schools or universities. Presently, 17% of the diabetics and 11% of the controls had no vocational education or were not on their way to gaining it. Working experience, employment status and unemployment were similar in both groups, but diabetics were more often employed in public service and commerce. At the time of the study the diabetics were significantly more often unmarried and living in the same household as their parents compared with the controls. Other parameters also indicated difficulties in the diabetic group in separating from parents. The overall social maturation index showed poor social maturation in diabetics more often than in the controls. Neither social background, education nor sex were related to poor social maturation. It is concluded that having diabetes delays social maturation.(ABSTRACT TRUNCATED AT 250 WORDS)

  19. Juvenile systemic lupus erythematosus: onset patterns and short-term outcome in Egyptian children, a single-center experience.

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    Abdel-Hafez, M A; Abdel-Nabi, H

    2015-11-01

    The objective of this article is to define disease onset pattern and understand the response to therapy in children with systemic lupus erythematosus (SLE) in Egypt. A prospective cohort of 41 Egyptian children diagnosed with SLE was analyzed. SLE Disease Activity Index (SLEDAI) score was used to record disease activity at onset, and renal biopsy was performed to define the stage of lupus nephritis. Response to therapy over a follow-up period ranging from 10 to 50 months was evaluated. The mean age at diagnosis was 12.12 ± 3.45 years. Thirty-six (87.8%) patients were females. Most patients had multiple manifestations at onset. The most common presenting symptoms were pallor and fever (51.2% and 43.9%, respectively). Lupus nephritis was found in 27 (65.9%) children. International Society of Nephrology (ISN) classes I and III were the most common findings on renal biopsy. Neuropsychiatric manifestations were present at disease onset in 19 patients (46.3%) with a bad prognostic course. At diagnosis, high SLEDAI scores were recorded (mean: 29.95 ± 2.06). The mean renal SLEDAI score was 10.2 ± 4. At follow-up 16 (39.02%) patients were in complete remission, 10 (24.39%) were in partial remission, two (4.87%) had active disease, five (12.9%) had relapsed, four (9.75%) had died and four (9.75%) patients were lost to follow-up. Egyptian children with SLE appear to have severe disease on presentation with high SLEDAI scores and high prevalence of lupus nephritis, but respond well to therapy with a favorable short-term prognosis. © The Author(s) 2015.

  20. Demographic and clinical profile of patients with juvenile onset open ...

    African Journals Online (AJOL)

    Objective: To document the demographic characteristics, clinical features and treatment outcome of the patients diagnosed with JOAG. Materials ... [1] The juvenile onset open angle glaucoma (JOAG) is characterized by an early age of onset, ... in 1 in 50,000 individuals,[2] while Ellong et al.[3] reported a prevalence of 0.4% ...

  1. Comparison of Disease Characteristics, Organ Damage, and Survival in Patients with Juvenile-onset and Adult-onset Systemic Lupus Erythematosus in a Combined Cohort from 2 Tertiary Centers in Turkey.

    Science.gov (United States)

    Artim-Esen, Bahar; Şahin, Sezgin; Çene, Erhan; Şahinkaya, Yasemin; Barut, Kenan; Adrovic, Amra; Özlük, Yasemin; Kılıçaslan, Işın; Omma, Ahmet; Gül, Ahmet; Öcal, Lale; Kasapçopur, Özgür; İnanç, Murat

    2017-05-01

    Age at onset has been shown to affect the clinical course and outcome of systemic lupus erythematosus (SLE). Herein, we aimed to define the differences in clinical characteristics, organ damage, and survival between patients with juvenile-onset (jSLE) and adult-onset SLE (aSLE). For the study, 719 patients (76.9%) with aSLE and 216 (23.1%) with jSLE were examined. Comparisons between the groups were made for demographic characteristics, clinical features, auto-antibody profiles, damage, and survival rates. These results were significantly more frequent in jSLE: photosensitivity, malar rash, oral ulcers, renal involvement, neuropsychiatric (NP) manifestations, and autoimmune hemolytic anemia (AIHA). Of the autoantibodies, a higher frequency of anti-dsDNA and anticardiolipin IgG and IgM were observed in the jSLE group. A significant proportion of patients with aSLE had anti-Sm positivity and pleuritis. The proportion of patients with jSLE who developed organ damage was comparable to that of patients with aSLE (53% vs 47%) and the mean damage scores were similar in both groups. Renal damage was significantly more frequent in jSLE while musculoskeletal and cardiovascular system damage and diabetes mellitus were more prominent in aSLE. Comparison of survival rates of the 2 groups did not reveal any significant differences. We report a higher frequency in the jSLE group of renal involvement, cutaneous symptoms, oral ulcers, NP manifestations, AIHA, and anti-dsDNA positivity. A significant proportion of patients in the jSLE group had damage, most prominently in the renal domain. Our findings might support different genetic/environmental backgrounds for these 2 subgroups.

  2. Depression Risk in Young Adults With Juvenile- and Adult-Onset Lupus: Twelve Years of Followup.

    Science.gov (United States)

    Knight, Andrea M; Trupin, Laura; Katz, Patricia; Yelin, Edward; Lawson, Erica F

    2018-03-01

    To compare major depression risk among young adults with juvenile-onset and adult-onset systemic lupus erythematosus (SLE), and to determine demographic and health-related predictors of depression. Young adults with SLE ages 18-45 years (n = 546) in the Lupus Outcomes Study completed annual telephone surveys from 2002-2015, including assessment of depression using the Center for Epidemiologic Studies Depression Scale (CES-D), and self-report measures of sociodemographics and health characteristics. Juvenile-onset SLE was defined as age adult-onset SLE. Older age, lower educational attainment, and physical function, higher disease activity, and a history of smoking were associated with an increased depression risk. Juvenile-onset SLE patients had a higher risk of major depression across all educational groups. Young adults with SLE, particularly those with juvenile-onset disease, are at high risk for major depression, which is associated with increased disease activity, poorer physical functioning, and lower educational attainment. Early depression intervention in young adults with SLE has the potential to improve both medical and psychosocial outcomes. © 2017, American College of Rheumatology.

  3. Prospective Prediction of Juvenile Homicide/Attempted Homicide among Early-Onset Juvenile Offenders.

    Science.gov (United States)

    Baglivio, Michael T; Wolff, Kevin T

    2017-02-16

    While homicide perpetrated by juveniles is a relatively rare occurrence, between 2010 and 2014, approximately 7%-8% of all murders involved a juvenile offender. Unfortunately, few studies have prospectively examined the predictors of homicide offending, with none examining first-time murder among a sample of adjudicated male and female youth. The current study employed data on 5908 juvenile offenders (70% male, 45% Black) first arrested at the age of 12 or younger to prospectively examine predictors of an arrest for homicide/attempted homicide by the age of 18. Among these early-onset offenders, males, Black youth, those living in households with family members with a history of mental illness, those engaging in self-mutilation, and those with elevated levels of anger/aggression (all measured by age 13) were more likely to be arrested for homicide/attempted homicide by age 18. These findings add to the scant scientific literature on the predictors of homicide, and illustrate potential avenues for intervention.

  4. Prospective Prediction of Juvenile Homicide/Attempted Homicide among Early-Onset Juvenile Offenders

    Directory of Open Access Journals (Sweden)

    Michael T. Baglivio

    2017-02-01

    Full Text Available While homicide perpetrated by juveniles is a relatively rare occurrence, between 2010 and 2014, approximately 7%–8% of all murders involved a juvenile offender. Unfortunately, few studies have prospectively examined the predictors of homicide offending, with none examining first-time murder among a sample of adjudicated male and female youth. The current study employed data on 5908 juvenile offenders (70% male, 45% Black first arrested at the age of 12 or younger to prospectively examine predictors of an arrest for homicide/attempted homicide by the age of 18. Among these early-onset offenders, males, Black youth, those living in households with family members with a history of mental illness, those engaging in self-mutilation, and those with elevated levels of anger/aggression (all measured by age 13 were more likely to be arrested for homicide/attempted homicide by age 18. These findings add to the scant scientific literature on the predictors of homicide, and illustrate potential avenues for intervention.

  5. Age of crime onset and psychopathic traits in female juvenile delinquents.

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    Pechorro, Pedro; Gonçalves, Rui Abrunhosa; Marôco, João; Nunes, Cristina; Jesus, Saul Neves

    2014-09-01

    The aim of this study was to analyze the role of psychopathic traits in the age of crime onset of female juvenile delinquents. Using a sample of 132 young females from the Juvenile Detention Centers of the Portuguese Ministry of Justice and from schools in the Lisbon region, a group of early crime onset (n = 44), a group of late crime onset (n = 44), and a nondelinquent school group (n = 44) were formed. Results showed that early crime onset participants score higher on psychopathy measures, self-reported delinquency, and crime seriousness than late crime onset participants and school participants. Psychopathic-traits scores were significantly associated with age of crime onset, age at first trouble with the law, and frequency and seriousness of crime. © The Author(s) 2013.

  6. Clinical Features in Juvenile-Onset Ankylosing Spondylitis Patients Carrying Different B27 Subtypes

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    Yikun Mou

    2015-01-01

    Full Text Available Background. Ankylosing spondylitis (AS is a common rheumatic disease and is characterized by inflammation of the axial skeleton. HLA-B27 is strongly associated with AS. Juvenile-onset AS (JAS with disease onset before 16 years of age differs from adult-onset AS (AAS in many respects. Objective. To compare the clinical features in JAS with different B27 subtypes and analyze the differences between JAS and AAS. Methods. 145 JAS and 360 AAS patients were included. The demographic data, clinical manifestations, laboratory markers, Bath AS indices, and B27 subtypes were recorded. Results. Peripheral arthritis, enthesitis, BASDAI, ESR, and CRP were significantly higher in JAS patients with HLA-B*2704 than those with B27-negative. Enthesitis and ESR were significantly higher in patients with HLA-B*2705 than those with B27-negative. The onset age of HLA-B*2715 group was much earlier than the other groups. The peripheral arthritis, enthesitis, and hip joint involvement in JAS with HLA-B*2704 were significantly higher than those in AAS with HLA-B*2704. Conclusion. JAS with different B27 subtypes had similar features in most of manifestations; JAS and AAS patients with the same subtype could have distinctive courses. Early diagnosis, hip detection, and control of systemic active inflammation in JAS patients will be helpful for improving the prognosis.

  7. Demographic and clinical profile of patients with juvenile onset open ...

    African Journals Online (AJOL)

    Background: This was a non-comparative, retrospective review of patients diagnosed with juvenile open angle glaucoma (JOAG) in the eye clinic of a tertiary hospital in southwestern Nigeria. Objective: To document the demographic characteristics, clinical features and treatment outcome of the patients diagnosed with ...

  8. Up regulation of serum tumor necrosis factor-related apoptosis inducing ligand in juvenile-onset systemic lupus erythematosus: relations with disease activity, antibodies to double -stranded DNA, nephritis and neutropenia.

    Science.gov (United States)

    Ezzat, Mohamed H M; El-Gammasy, Tarek M A; Shaheen, Kareem Y A; El-Mezdawi, Ramzi A M; Youssef, Mervat S M

    2013-06-01

    Apoptosis is induced by binding of death receptor ligands, members of the tumor necrosis factor (TNF) superfamily, to their cognate receptors. It is suggested that TNF-related apoptosis inducing ligand (TRAIL) is involved in pathogenesis of juvenile-onset systemic lupus erythematosus (JSLE). This study aimed to assess TRAIL concentrations in sera of JSLE children and to determine their potential relationship with disease activity, anti-double-stranded DNA (anti-dsDNA) levels, neutropenia and renal involvement. Circulating levels of TRAIL were measured by enzyme-linked immunosorbent assay (ELISA) in serum samples obtained from 40 JSLE patients (20 with active and 20 with inactive disease) and 20 controls. The mean (SEM) serum TRAIL concentration in JSLE was 1750.7 (440.2) pg/mL. Serum TRAIL concentrations in patients were higher than those in controls (P nephritis compared to classes I and II nephritis (1970 [512] vs. 1330 [331] pg/mL; P lupus nephritis. © 2013 The Authors International Journal of Rheumatic Diseases © 2013 Asia Pacific League of Associations for Rheumatology and Wiley Publishing Asia Pty Ltd.

  9. Clinical and genetic study of a juvenile-onset Huntington disease

    Directory of Open Access Journals (Sweden)

    HAO Ying

    2012-06-01

    Full Text Available Background Huntington's disease (HD is an autosomal dominant hereditary progressive neurodegenerative disorder with a distinct phenotype characterized by chorea, dementia, cognitive and affective impairment. There are selective neural cell loss and atrophy in the caudate and putamen. Dr. George Huntington firstly described the disease accurately and insightfully, which led to a widespread recognition of the inherited chorea that now bears his name. Huntington disease gene (IT15 locus on chromosome 4p16.3, and encompasses 67 exons with a trinucleotide repeat (CAG in the first exon. The CAG repeat length is highly polymorphic in the population and expanded on at least one chromosome of individuals with HD. Clinically, patient with HD are often onset in adulthood. Juvenile-onset HD is relatively rare. Adult-onset HD patients usually have a CAG expansion from 40 to 55 whereas those with juvenile-onset greater than 60 which are often inherited from the father. We investigated the clinical features of a juvenile-onset case with Huntington disease and dynamic mutation of his family. Methods The CAG repeats of IT15 gene were detected using polymerase chain reaction and capillary electrophoresis in 115 individuals with preliminary diagnosis as Huntington disease. The repeat numbers of some samples carried expanded or intermediate alleles were verified by the pMD18-T vector clone sequencing. Results Fragment analysis showed that one juvenile-onset case presenting with cognitive dysfunction and hypokinesis carried 15/68 CAG repeats of IT15. His father carried 17/37 and mother carried 15/17. Conclusion 1 The juvenile-onset case of HD presented with different clinical features compared with adult-onset cases. The typical signs of adult-onset cases include progressive chorea, rigidity and dementia. The most common sign of juvenile-onset Huntington disease is cognitive decline. 2 The dynamic mutation of IT15 gene expansion of the CAG repeats in the

  10. REFORMATIONS IN ZIMBABWE'S JUVENILE JUSTICE SYSTEM

    African Journals Online (AJOL)

    Mugumbate

    1996-05-23

    May 23, 1996 ... inconsistent parenting practices, poverty, school-related problems and exposure to delinquent peers and community and societal problems. This paper discusses the recent reforms in the juvenile justice system in Zimbabwe and elaborates on the role of social work practice in the field of juvenile justice.

  11. Reformations in Zimbabwe's juvenile justice system | Ruparanganda ...

    African Journals Online (AJOL)

    Children in conflict with the law are often stigmatized and shunned by society as they are perceived as a threat to society. Historically, Zimbabwe's juvenile justice system has been retributive and focused on punishing the juvenile offender. As a result, it has been criticised from a number of viewpoints, including the need to ...

  12. Sex differences in metabolic and adipose tissue responses to juvenile-onset obesity in sheep.

    Science.gov (United States)

    Bloor, Ian D; Sébert, Sylvain P; Saroha, Vivek; Gardner, David S; Keisler, Duane H; Budge, Helen; Symonds, Michael E; Mahajan, Ravi P

    2013-10-01

    Sex is a major factor determining adipose tissue distribution and the subsequent adverse effects of obesity-related disease including type 2 diabetes. The role of gender on juvenile obesity and the accompanying metabolic and inflammatory responses is not well established. Using an ovine model of juvenile onset obesity induced by reduced physical activity, we examined the effect of gender on metabolic, circulatory, and related inflammatory and energy-sensing profiles of the major adipose tissue depots. Despite a similar increase in fat mass with obesity between genders, males demonstrated a higher storage capacity of lipids within perirenal-abdominal adipocytes and exhibited raised insulin. In contrast, obese females became hypercortisolemic, a response that was positively correlated with central fat mass. Analysis of gene expression in perirenal-abdominal adipose tissue demonstrated the stimulation of inflammatory markers in males, but not females, with obesity. Obese females displayed increased expression of genes involved in the glucocorticoid axis and energy sensing in perirenal-abdominal, but not omental, adipose tissue, indicating a depot-specific mechanism that may be protective from the adverse effects of metabolic dysfunction and inflammation. In conclusion, young males are at a greater risk than females to the onset of comorbidities associated with juvenile-onset obesity. These sex-specific differences in cortisol and adipose tissue could explain the earlier onset of the metabolic-related diseases in males compared with females after obesity.

  13. [Epidemiologic profile of juvenile-onset compared to adult-onset spondyloarthritis in a large Brazilian cohort].

    Science.gov (United States)

    Duarte, Angela P; Marques, Cláudia D L; Bortoluzzo, Adriana B; Gonçalves, Célio R; da Silva, José Antonio Braga; Ximenes, Antonio Carlos; Bértolo, Manoel B; Ribeiro, Sandra Lúcia E; Keiserman, Mauro; Skare, Thelma L; Carneiro, Sueli; Menin, Rita; Azevedo, Valderilio F; Vieira, Walber P; Albuquerque, Elisa N; Bianchi, Washington A; Bonfiglioli, Rubens; Campanholo, Cristiano; Carvalho, Hellen M S; Costa, Izaias P; Kohem, Charles L; Leite, Nocy; Lima, Sonia A L; Meirelles, Eduardo S; Pereira, Ivânio A; Pinheiro, Marcelo M; Polito, Elizandra; Resende, Gustavo G; Rocha, Francisco Airton C; Santiago, Mittermayer B; Sauma, Maria de Fátima L C; Valim, Valéria; Sampaio-Barros, Percival D; Barros, Percival D Sampaio

    2014-01-01

    To analyze the clinical and epidemiologic characteristics of juvenile-onset spondyloarthritis (SpA) (< 16 years) and compare them with a group of adult-onset (≥ 16 years) SpA patients. Prospective, observational and multicentric cohort with 1,424 patients with the diagnosis of SpA according to the European Spondyloarthropathy Study Group (ESSG) submitted to a common protocol of investigation and recruited in 29 reference centers participants of the Brazilian Registry of Spondyloarthritis (RBE - Registro Brasileiro de Espondiloartrites). Patients were divided in two groups: age at onset<16 years (JOSpA group) and age at onset ≥ 16 years (AOSpA group). Among the 1,424 patients, 235 presented disease onset before 16 years (16.5%). The clinical and epidemiologic variables associated with JOSpA were male gender (p<0.001), lower limb arthritis (p=0.001), enthesitis (p=0.008), anterior uveitis (p=0.041) and positive HLA-B27 (p=0.017), associated with lower scores of disease activity (Bath Ankylosing Spondylitis Disease Activity Index - BASDAI; p=0.007) and functionality (Bath Ankylosing Spondylitis Functional Index - BASFI; p=0.036). Cutaneous psoriasis (p<0.001), inflammatory bowel disease (p=0.023), dactylitis (p=0.024) and nail involvement (p=0.004) were more frequent in patients with adult-onset SpA. Patients with JOSpA in this large Brazilian cohort were characterized predominantly by male gender, peripheral involvement (arthritis and enthesitis), positive HLA-B27 and lower disease scores. Copyright © 2014 Elsevier Editora Ltda. All rights reserved.

  14. CD8-positive juvenile onset mycosis fungoides: an immunohistochemical and genotypic analysis of six cases.

    Science.gov (United States)

    Whittam, L R; Calonje, E; Orchard, G; Fraser-Andrews, E A; Woolford, A; Russell-Jones, R

    2000-12-01

    Childhood cases of cytotoxic T-cell lymphoma have not been well described. We have undertaken an immunohistochemical and genotypic analysis of patients presenting with juvenile onset mycosis fungoides (MF). Of 10 patients presenting over a 3-year period, six exhibited a CD8-positive phenotype. These six patients were also CD2, CD3 and TIA1 positive, but CD56 negative. Apart from the cytotoxic phenotype, these patients had clinicopathological features that were indistinguishable from ordinary cases of MF, with slowly evolving patches and plaques. Three patients were staged as 1A and three as 1B, with no evidence of nodal or systemic disease. Patients responded well to conventional therapy, with no evidence of disease progression after 3 years follow-up. Epidermotropism was a prominent feature in four of the six cytotoxic cases. In two patients with an equivocal histology the diagnosis was confirmed by the finding of a clonal population, using polymerase chain reaction/single strand conformational polymorphism analysis of the T-cell receptor gamma gene in lesional skin. The same technique revealed that all blood samples analysed were polyclonal. These data show that cytotoxic T-cell lymphoma can pursue an indolent course and that cases of CD8-positive MF may be over-represented in childhood.

  15. Comparison of Children With Onset of Juvenile Dermatomyositis Symptoms Before or After Their Fifth Birthday in a UK and Ireland Juvenile Dermatomyositis Cohort Study

    Science.gov (United States)

    Martin, N; Krol, P; Smith, S; Beard, L; Pilkington, C A; Davidson, J; Wedderburn, L R

    2012-01-01

    Objective To compare 2 groups of children with juvenile dermatomyositis (DM), those with onset of symptoms before their fifth birthday versus those whose disease begins either on or after their fifth birthday, and to assess whether age at onset is associated with differences in disease presentation, treatments received, or outcomes 2 years after diagnosis. Methods Data were analyzed on children recruited to a UK juvenile DM cohort study with a diagnosis of probable or definite juvenile DM and less than 12 months between diagnosis and recruitment. Results Fifty-five (35%) of 157 children had onset of symptoms before their fifth birthday. At diagnosis, cutaneous ulceration was found in 32.7% of the younger group versus 11.8% of the older group (P = 0.003). Facial or body swelling was reported more often in the younger group, whereas headaches, alopecia, and Raynaud's phenomenon were all more frequently reported in the older group. At followup 2 years later, there were no important differences in outcomes between the groups. More than 90% of patients in both groups received both methotrexate and steroids. Twenty-three percent of both groups remained on steroids 2 years after diagnosis. Conclusion Our study showed that children with juvenile DM with disease onset at age <5 years are more likely to present with ulcerative skin disease and edema. There were no clinically significant differences in outcomes between the 2 groups. PMID:22674907

  16. Juvenile Myasthenia Gravis in Korea: Subgroup Analysis According to Sex and Onset Age.

    Science.gov (United States)

    Lee, Ha Neul; Kang, Hoon-Chul; Lee, Joon Soo; Kim, Heung Dong; Shin, Ha Young; Kim, Seung Min; Sunwoo, Il Nam; Lee, Young-Mock

    2016-12-01

    Juvenile myasthenia gravis presents before 18 years of age with different characteristics according to racial background and pubertal development. The authors aimed to determine the clinical characteristics of children and adolescents of Korean ethnicity with myasthenia gravis, and evaluate the presentation and clinical outcomes according to the sex and onset age of the patients. The authors recruited 88 Korean juvenile myasthenia gravis patients between September 2005 and August 2015. Worse clinical severity from presentation, more aggressive treatment strategies, and worse final treatment outcomes were noted in girls with postpubertal onset than in the other patients. The symptoms were milder (pure ocular presentation in 96.6% [85/88]) and the disease course was more benign (94.3% [83/88]) in this study than in the literature. The homogenous racial background might have contributed to these results. These findings highlight the influence of pubertal development and the need for timely and appropriate active treatment, including thymectomy, to improve prognosis. © The Author(s) 2016.

  17. Autoimmune hepatitis and juvenile systemic lupus erythematosus

    NARCIS (Netherlands)

    Deen, M. E. J.; Porta, G.; Fiorot, F. J.; Campos, L. M. A.; Sallum, A. M. E.; Silva, C. A. A.

    Juvenile systemic lupus erythematosus (JSLE) and autoimmune hepatitis (AIH) are both autoimmune disorders that are rare in children and have a widespread clinical manifestation. A few case reports have shown a JSLE-AIH associated disorder. To our knowledge, this is the first study that

  18. Radiographic abnormalities of the wrist in adult-onset still disease: Comparison with juvenile chronic arthritis and rheumatoid arthritis

    International Nuclear Information System (INIS)

    Bjorkengren, A.G.; Pathria, M.N.; Terkeltaub, R.; Esdaile, J.; Weisman, M.; Sartoris, D.J.; Resnick, D.

    1987-01-01

    Pericapitate involvement of the wrist has been described as characteristic of adult-onset Still disease, a relatively rare disorder that is often diagnosed by exclusion after extensive and frequently invasive tests. To evaluate the diagnostic value of carpal radiography in cases of adult-onset Still disease, a retrospective blinded analysis of 48 patients, 16 each with adult-onset Still disease, juvenile chronic arthritis, and rheumatoid arthritis, was performed. Pericapitate articular alterations without radiocarpal involvement were found to be frequent in the setting of adult-onset Still disease but distinctly unusual among patients with rheumatoid arthritis. In juvenile chronic arthritis, severe pericapitate involvement was frequent, but generally occurred in conjunction with radiocarpal joint abnormalities

  19. Condyloma in pregnancy is strongly predictive of juvenile-onset recurrent respiratory papillomatosis

    DEFF Research Database (Denmark)

    Silverberg, Michael J.; Thorsen, Poul; Lindeberg, Henning

    2003-01-01

    OBJECTIVE: To assess the risk of juvenile-onset recurrent respiratory papillomatosis conferred by a maternal history of genital warts in pregnancy, and to identify additional cofactors such as the method of delivery (cesarean versus vaginal) and procedures or complications during pregnancy. METHODS......: A retrospective cohort design was used to evaluate maternal and infant characteristics associated with respiratory papillomatosis among Danish births between 1974 and 1993. Using data from Danish registries, we identified 3033 births with a maternal history of genital warts during pregnancy. Fifty......-seven respiratory papillomatosis cases were identified by review of medical records from ear, nose, and throat departments. RESULTS: Seven of every 1000 births with a maternal history of genital warts resulted in disease in the offspring, corresponding to a 231.4 (95% confidence interval 135.3, 395.9) times higher...

  20. Early onset bilateral juvenile myasthenia gravis masquerading as simple congenital ptosis

    Directory of Open Access Journals (Sweden)

    Alam, Md. Shahid

    2017-03-01

    Full Text Available Myasthenia gravis is an autoimmune disorder affecting the neuromuscular junction. Ocular myasthenia gravis presents as ptosis with extraocular motility restriction and is prone to be misdiagnosed as third nerve palsy or congenital or aponeurotic ptosis. Juvenile ocular myasthenia gravis in very young children is difficult to diagnose and can be easily labeled as a case of congenital ptosis, the more so when the condition is bilateral. We present a case of a two-year-old child who presented with bilateral ptosis and was diagnosed as a case of simple congenital ptosis elsewhere with the advice to undergo tarsofrontalis sling surgery. The child was diagnosed with juvenile myasthenia gravis on thorough history, examination, and systemic evaluation and was started on anti-myasthenic treatment.

  1. Early- versus Late-Onset Systemic Sclerosis

    Science.gov (United States)

    Alba, Marco A.; Velasco, César; Simeón, Carmen Pilar; Fonollosa, Vicent; Trapiella, Luis; Egurbide, María Victoria; Sáez, Luis; Castillo, María Jesús; Callejas, José Luis; Camps, María Teresa; Tolosa, Carles; Ríos, Juan José; Freire, Mayka; Vargas, José Antonio; Espinosa, Gerard

    2014-01-01

    Abstract Peak age at onset of systemic sclerosis (SSc) is between 20 and 50 years, although SSc is also described in both young and elderly patients. We conducted the present study to determine if age at disease onset modulates the clinical characteristics and outcome of SSc patients. The Spanish Scleroderma Study Group recruited 1037 patients with a mean follow-up of 5.2 ± 6.8 years. Based on the mean ± 1 standard deviation (SD) of age at disease onset (45 ± 15 yr) of the whole series, patients were classified into 3 groups: age ≤30 years (early onset), age between 31 and 59 years (standard onset), and age ≥60 years (late onset). We compared initial and cumulative manifestations, immunologic features, and death rates. The early-onset group included 195 patients; standard-onset group, 651; and late-onset, 191 patients. The early-onset group had a higher prevalence of esophageal involvement (72% in early-onset compared with 67% in standard-onset and 56% in late-onset; p = 0.004), and myositis (11%, 7.2%, and 2.9%, respectively; p = 0.009), but a lower prevalence of centromere antibodies (33%, 46%, and 47%, respectively; p = 0.007). In contrast, late-onset SSc was characterized by a lower prevalence of digital ulcers (54%, 41%, and 34%, respectively; p < 0.001) but higher rates of heart conduction system abnormalities (9%, 13%, and 21%, respectively; p = 0.004). Pulmonary hypertension was found in 25% of elderly patients and in 12% of the youngest patients (p = 0.010). After correction for the population effects of age and sex, standardized mortality ratio was shown to be higher in younger patients. The results of the present study confirm that age at disease onset is associated with differences in clinical presentation and outcome in SSc patients. PMID:24646463

  2. Prednisone versus prednisone plus ciclosporin versus prednisone plus methotrexate in new-onset juvenile dermatomyositis: a randomised trial.

    Science.gov (United States)

    Ruperto, Nicolino; Pistorio, Angela; Oliveira, Sheila; Zulian, Francesco; Cuttica, Ruben; Ravelli, Angelo; Fischbach, Michel; Magnusson, Bo; Sterba, Gary; Avcin, Tadej; Brochard, Karine; Corona, Fabrizia; Dressler, Frank; Gerloni, Valeria; Apaz, Maria T; Bracaglia, Claudia; Cespedes-Cruz, Adriana; Cimaz, Rolando; Couillault, Gerard; Joos, Rik; Quartier, Pierre; Russo, Ricardo; Tardieu, Marc; Wulffraat, Nico; Bica, Blanca; Dolezalova, Pavla; Ferriani, Virginia; Flato, Berit; Bernard-Medina, Ana G; Herlin, Troels; Trachana, Maria; Meini, Antonella; Allain-Launay, Emma; Pilkington, Clarissa; Vargova, Veronika; Wouters, Carine; Angioloni, Simona; Martini, Alberto

    2016-02-13

    Most data for treatment of dermatomyositis and juvenile dermatomyositis are from anecdotal, non-randomised case series. We aimed to compare, in a randomised trial, the efficacy and safety of prednisone alone with that of prednisone plus either methotrexate or ciclosporin in children with new-onset juvenile dermatomyositis. We did a randomised trial at 54 centres in 22 countries. We enrolled patients aged 18 years or younger with new-onset juvenile dermatomyositis who had received no previous treatment and did not have cutaneous or gastrointestinal ulceration. We randomly allocated 139 patients via a computer-based system to prednisone alone or in combination with either ciclosporin or methotrexate. We did not mask patients or investigators to treatment assignments. Our primary outcomes were the proportion of patients achieving a juvenile dermatomyositis PRINTO 20 level of improvement (20% improvement in three of six core set variables at 6 months), time to clinical remission, and time to treatment failure. We compared the three treatment groups with the Kruskal-Wallis test and Friedman's test, and we analysed survival with Kaplan-Meier curves and the log-rank test. Analysis was by intention to treat. Here, we present results after at least 2 years of treatment (induction and maintenance phases). This trial is registered with ClinicalTrials.gov, number NCT00323960. Between May 31, 2006, and Nov 12, 2010, 47 patients were randomly assigned prednisone alone, 46 were allocated prednisone plus ciclosporin, and 46 were randomised prednisone plus methotrexate. Median duration of follow-up was 35.5 months. At month 6, 24 (51%) of 47 patients assigned prednisone, 32 (70%) of 46 allocated prednisone plus ciclosporin, and 33 (72%) of 46 administered prednisone plus methotrexate achieved a juvenile dermatomyositis PRINTO 20 improvement (p=0.0228). Median time to clinical remission was 41.9 months in patients assigned prednisone plus methotrexate but was not observable in the

  3. Juvenile myoclonic epilepsy 25 years after seizure onset: a population-based study.

    Science.gov (United States)

    Camfield, Carol S; Camfield, Peter R

    2009-09-29

    To document the long-term evolution of juvenile myoclonic epilepsy (JME) in a population-based cohort. All patients developing JME by 16 years of age in Nova Scotia between 1977 and 1985 were contacted in 2006-2008. Twenty-four patients (17 women) had JME, 3.5% of all childhood-onset epilepsy. Age at first seizure was 10.4 +/- 4.3 years. We contacted 23 of 24 (96%) at a mean age of 36 +/- 4.8 years. All were initially treated with antiepileptic drugs (AEDs). At the end of a 25.8 +/- 2.4-year follow-up, 11 (48%) had discontinued treatment: 6 were seizure-free (without AEDs for 5-23 years), 3 had myoclonic seizures only (without AEDs for >18 years), and 2 continued with rare seizures. Convulsive status epilepticus occurred in 8 (36%) and 3 had intractable epilepsy. About 70% reported good satisfaction with their health, work, friendships, and social life (Likert scales). Despite 87% high school graduation, 31% were unemployed. Sixteen live with a partner, 7 alone. Nine received antidepressant medications. Ten women had > or =1 pregnancy and 4 men fathered a child. Eleven pregnancies (80%) were unplanned, outside of a stable relationship. At least 1 major unfavorable social outcome was noted in 76%. Our sample size is modest but the long follow-up and population-based sampling is unique. All seizure types in juvenile myoclonic epilepsy (JME) resolved in 17% and for 13%, only myoclonus persisted. Therefore, one-third of people with JME have troublesome seizures vanish and antiepileptic drug treatment is no longer needed. Depression, social isolation, unemployment, and social impulsiveness complicate the lives of many patients.

  4. Are Indian patients with juvenile-onset ankylosing spondylitis taller than reference population ?

    Science.gov (United States)

    Sandhya, Pulukool; Danda, Debashish; Jeyaseelan, Lakshmanan

    2015-04-01

    Paucity of growth retardation has been observed by us in patients with juvenile-onset ankylosing spondylitis (JAS) in a tertiary care health centre in south India. We, therefore, undertook this pilot study to assess and compare anthropometry of patients with JAS who were 15 yr and older with that of adult onset ankylosing spondylitis (AAS) and matching Indian reference population. Consecutive male patients (December 2009- October 2012) with JAS and AAS fulfilling Modified New York Criteria were selected after applying inclusion and exclusion criteria. Demography and anthropometry were noted. Height of both patient groups as well as their parents and siblings were compared with that of the reference population. Mid-parental height and delta height were derived. Those with delta height of >8.5 cm were compared with the remaining. Multivariate logistic regression was done for variables that were found to be significant by chi-square in bivariate analysis. Similar analysis was done for BMI also. There was no significant difference in anthropometric variables between JAS and AAS groups. Twenty eight of the 30 (93.33%) JAS patients were taller as compared to the reference population. Twenty six (86.67%) AAS patients were taller than the reference population. The mean heights of JAS (170.67 ± 6.94 cm) and AAS (168.2 ± 5.94 cm) patients were significantly higher than the reference value of 163.11 cm; both p0 growth retardation was seen in patients with JAS in our study. Majority of patients with JAS and AAS were taller than reference population. The difference between mean height of JAS and AAS was not significant. Larger studies involving different populations are required to confirm these findings.

  5. Neuropsychological Symptoms of Juvenile-Onset Batten Disease: Experiences From 2 Studies

    OpenAIRE

    Adams, Heather R.; Kwon, Jennifer; Marshall, Frederick J.; de Blieck, Elisabeth A.; Pearce, David A.; Mink, Jonathan W.

    2007-01-01

    Juvenile neuronal ceroid lipofuscinosis (Batten disease) is a progressive and fatal autosomal-recessive inherited lysosomal storage disorder of childhood. Core symptoms include vision loss, seizures, and mental and motor decline. This article presents data from 2 studies of neuropsychological function in juvenile neuronal ceroid lipofuscinosis. In the first cross-sectional pilot study, 15 children with genetic or clinicopathologic confirmation of juvenile neuronal ceroid lipofuscinosis comple...

  6. A Survey of Conventional and Complementary Therapies Used by Youth with Juvenile-Onset Fibromyalgia

    Science.gov (United States)

    Verkamp, Emily K.; Flowers, Stacy R.; Lynch-Jordan, Anne M.; Taylor, Janalee; Ting, Tracy V.; Kashikar-Zuck, Susmita

    2013-01-01

    Little is known regarding treatment choices of youth diagnosed with juvenile-onset fibromyalgia (JFM) as they move into young adulthood. Additionally, there is little empirical evidence to guide youth with FM into appropriate treatment options, leading to a variety of therapies used to manage FM symptoms. The purpose of this descriptive study was to examine all therapies used by individuals with JFM as they entered young adulthood and the perceived effectiveness of these treatments. As part of a larger follow-up study, participants completed a web-based survey of all current and past treatments received for FM symptoms 2 years after their initial presentation and diagnosis at a pediatric rheumatology clinic. One hundred ten out of 118 eligible patients participated in the follow-up assessment as young adults (mean age 18.97 years; 93.6% female). A majority of participants reported use of conventional medications (e.g., antidepressants, anti-convulsants) and nondrug therapies (e.g., psychotherapy). Currently and within the past 2 years, antidepressant medications were the most commonly used to manage FM. Complementary treatments were used less often, with massage being the most popular choice. Although currently used treatments were reported as being effective, past treatments, especially medications, were viewed as being more variably effective. This is a potential reason why young adults with JFM might try more complementary and alternative approaches to managing their symptoms. More controlled studies are needed to investigate the effectiveness of these complementary methods to assist treatment providers in giving evidence-based treatment recommendations. PMID:24315277

  7. Long-term results after Boston brace treatment in late-onset juvenile and adolescent idiopathic scoliosis

    Directory of Open Access Journals (Sweden)

    Gunderson Ragnhild

    2011-08-01

    Full Text Available Abstract Background It is recommended that research in patients with idiopathic scoliosis should focus on short- and long-term patient-centred outcome. The aim of the present study was to evaluate outcome in patients with late-onset juvenile or adolescent idiopathic scoliosis 16 years or more after Boston brace treatment. Methods 272 (78% of 360 patients, 251 (92% women, responded to follow-up examination at a mean of 24.7 (range 16 - 32 years after Boston brace treatment. Fifty-eight (21% patients had late-onset juvenile and 214 had adolescent idiopathic scoliosis. All patients had clinical and radiological examination and answered a standardised questionnaire including work status, demographics, General Function Score (GFS (100 - worst possible and Oswestry Disability Index (ODI (100 - worst possible, EuroQol (EQ-5D (1 - best possible, EQ-VAS (100 - best possible, and Scoliosis Research Society - 22 (SRS - 22 (5 - best possible. Results The mean age at follow-up was 40.4 (31-48 years. The prebrace major curve was in average 33.2 (20 - 57°. At weaning and at the last follow-up the corresponding values were 28.3 (1 - 58° and 32.5 (7 - 80°, respectively. Curve development was similar in patients with late-onset juvenile and adolescent start. The prebrace curve increased > 5° in 31% and decreased > 5° in 26%. Twenty-five patients had surgery. Those who did not attend follow-up (n = 88 had a lower mean curve at weaning: 25.4 (6-53°. Work status was 76% full-time and 10% part-time. Eighty-seven percent had delivered a baby, 50% had pain in pregnancy. The mean (SD GFS was 7.4 (10.8, ODI 9.3 (11.0, EQ-5D 0.82 (0.2, EQ-VAS 77.6 (17.8, SRS-22: pain 4.1 (0.8, mental health 4.1 (0.6, self-image 3.7 (0.7, function 4.0 (0.6, satisfaction with treatment 3.7 (1.0. Surgical patients had significantly reduced scores for SRS-physical function and self-image, and patients with curves ≥ 45° had reduced self-image. Conclusion Long-term results were

  8. Juvenile systemic lupus erythematosus in Nigeria.

    Science.gov (United States)

    Adelowo, O O; Olaosebikan, B H; Animashaun, B A; Akintayo, R O

    2017-03-01

    Juvenile systemic lupus erythematosus (JSLE) is a complex multisystemic autoimmune disorder of unknown cause. It accounts for about one in five cases of SLE. The tendency for SLE to run a fulminant course when it starts in childhood has made JSLE a potentially more severe disease than adult SLE. Reports of JSLE from sub-Saharan Africa are scanty in spite of the increasing reports of adult SLE. We conducted a 4-year retrospective study of JSLE cases seen at the Lagos State University Teaching Hospital between January 2010 and December 2014. Out of the 12 patients studied, eight were girls and four were boys. All patients had positive antinuclear antibody and extractable nuclear antibody tests. Anti-dsDNA antibody was positive in 10 patients. Eight patients had renal disease while four patients had neuropsychiatric manifestations. Haematological abnormalities and constitutional symptoms were present in all patients. Patients were treated with pulse methylprednisolone, oral prednisolone, hydroxychloroquine and azathioprine. Three patients also received rituximab. In conclusion, JSLE exists in Nigeria and exhibits clinical and immunological characteristics similar to its pattern in other parts of the world. It is, however, diagnosed late and is possibly being underdiagnosed as there is no paediatric rheumatologist in the country.

  9. Prevalence of mutations and functional analyses of melanocortin 4 receptor variants identified among 750 men with juvenile-onset obesity

    DEFF Research Database (Denmark)

    Larsen, Lesli H; Echwald, Søren Morgenthaler; Sørensen, Thorkild I A

    2005-01-01

    Mutations in the gene encoding the melanocortin 4 receptor (MC4R) are associated with the most common monogenic form of obesity. We examined 750 Danish men with juvenile-onset obesity (body mass index 33.3 +/- 2.4 kg/m(2)) and 706 control subjects (body mass index 21.4 +/- 2.1 kg/m(2)) for mutati...... in the Danish population. This study shows a carrier frequency of 2.5% of pathogenic mutations in the MC4R gene in a population-based study of obese men. Thus, variation in this gene is the most common known specific genetic cause of obesity among Scandinavian men.......Mutations in the gene encoding the melanocortin 4 receptor (MC4R) are associated with the most common monogenic form of obesity. We examined 750 Danish men with juvenile-onset obesity (body mass index 33.3 +/- 2.4 kg/m(2)) and 706 control subjects (body mass index 21.4 +/- 2.1 kg/m(2...

  10. Increased frequency of HLA-DPw2 in pauciarticular onset juvenile chronic arthritis

    DEFF Research Database (Denmark)

    Ødum, Niels; Morling, Niels; Friis, J

    1986-01-01

    Thirty-six unrelated Danish patients with pauciarticular Juvenile Chronic Arthritis (PJCA) and 120 controls were typed for HLA-DPw1-w6 and the local specificity CDPHEI with bulk-expanded Primed Lymphocyte Typing (PLT) cells. The frequency of HLA-DPw2 was 52.8% in PJCA patients and 16.7% in contro...

  11. Childhood-onset bullous systemic lupus erythematosus.

    Science.gov (United States)

    Lourenço, D M R; Gomes, R Cunha; Aikawa, N E; Campos, L M A; Romiti, R; Silva, C A

    2014-11-01

    Bullous systemic lupus erythematosus has rarely been described in pediatric lupus population and the real prevalence of childhood-onset bullous systemic lupus erythematosus has not been reported. From January 1983 to November 2013, 303 childhood-onset SLE (c-SLE) patients were followed at the Pediatric Rheumatology Unit of the Childreńs Institute of Hospital das Clínicas da Faculdade de Medicina Universidade da Universidade de São Paulo, three of them (1%) diagnosed as childhood-onset bullous systemic lupus erythematosus. All three cases presented tense vesiculobullous lesions unassociated with lupus erythematosus lesions, with the median duration of 60 days (30-60). All patients fulfilled bullous systemic lupus erythematosus criteria. Two had nephritis and serositis and presented specific autoantibodies. The histological pattern demonstrated subepidermal blisters with neutrophils-predominant infiltrates within the upper dermis. Direct immunofluorescence (DIF) showed deposits of IgG and complement along the epidermal basement membrane, in the presence or absence of IgA and/or IgM. A positive indirect immunofluorescence on salt-split skin demonstrating dermal binding was observed in two cases. All of them had moderate/severe disease activity at diagnosis with median Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) of 18 (14-24). Two patients received dapsone and one with severe nephritis received immunosuppressive drugs. In conclusion, in the last 30 years the prevalence of bullous lupus in childhood-onset lupus population was low (1%) in our tertiary University Hospital. A diagnosis of SLE should always be considered in children with recurrent tense vesiculobullous lesions with or without systemic manifestations. © The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav.

  12. An ARHGEF10 deletion is highly associated with a juvenile-onset inherited polyneuropathy in Leonberger and Saint Bernard dogs.

    Directory of Open Access Journals (Sweden)

    Kari J Ekenstedt

    2014-10-01

    Full Text Available An inherited polyneuropathy (PN observed in Leonberger dogs has clinical similarities to a genetically heterogeneous group of peripheral neuropathies termed Charcot-Marie-Tooth (CMT disease in humans. The Leonberger disorder is a severe, juvenile-onset, chronic, progressive, and mixed PN, characterized by exercise intolerance, gait abnormalities and muscle atrophy of the pelvic limbs, as well as inspiratory stridor and dyspnea. We mapped a PN locus in Leonbergers to a 250 kb region on canine chromosome 16 (Praw = 1.16×10-10, Pgenome, corrected = 0.006 utilizing a high-density SNP array. Within this interval is the ARHGEF10 gene, a member of the rho family of GTPases known to be involved in neuronal growth and axonal migration, and implicated in human hypomyelination. ARHGEF10 sequencing identified a 10 bp deletion in affected dogs that removes four nucleotides from the 3'-end of exon 17 and six nucleotides from the 5'-end of intron 17 (c.1955_1958+6delCACGGTGAGC. This eliminates the 3'-splice junction of exon 17, creates an alternate splice site immediately downstream in which the processed mRNA contains a frame shift, and generates a premature stop codon predicted to truncate approximately 50% of the protein. Homozygosity for the deletion was highly associated with the severe juvenile-onset PN phenotype in both Leonberger and Saint Bernard dogs. The overall clinical picture of PN in these breeds, and the effects of sex and heterozygosity of the ARHGEF10 deletion, are less clear due to the likely presence of other forms of PN with variable ages of onset and severity of clinical signs. This is the first documented severe polyneuropathy associated with a mutation in ARHGEF10 in any species.

  13. Onset of Systemic Lupus Erythematosus During Pregnancy

    Directory of Open Access Journals (Sweden)

    Ming-Jie Yang

    2006-03-01

    Full Text Available When systemic lupus erythematosus (SLE is first suspected during pregnancy, though rare, the diagnostic criteria are not different from those for nonpregnant women. The pregnancy outcome is good if treatment with adequate immunosuppressive agents starts as soon as the diagnosis is made. There are 4 cases in this report who had SLE onset during pregnancy. Although 2 of them suffered from preeclampsia, all 4 pregnancies resulted in favorable outcomes after the lupus was controlled by medical treatment.

  14. American juvenile justice system: history in the making.

    Science.gov (United States)

    Meng, Aaron; Segal, Roland; Boden, Eric

    2013-01-01

    The original theory behind separating juvenile offenders from adult offenders was to provide care and direction for youngsters instead of isolation and punishment. This idea took hold in the 19th century and became mainstream by the early 20th century. In the 1950s and 1960s, public concern grew because of a perceived lack of effectiveness and lack of rights. The Supreme Court made a series of rulings solidifying juvenile rights including the right to receive notice of charges, the right to have an attorney and the right to have charges proven beyond a reasonable doubt. In the 1980s, the public view was that the juvenile court system was too lenient and that juvenile crimes were on the rise. In the 1990s, many states passed punitive laws, including mandatory sentencing and blanket transfers to adult courts for certain crimes. As a result, the pendulum is now swinging back toward the middle from rehabilitation toward punishment.

  15. Neuropsychological symptoms of juvenile-onset batten disease: experiences from 2 studies.

    Science.gov (United States)

    Adams, Heather R; Kwon, Jennifer; Marshall, Frederick J; de Blieck, Elisabeth A; Pearce, David A; Mink, Jonathan W

    2007-05-01

    Juvenile neuronal ceroid lipofuscinosis (Batten disease) is a progressive and fatal autosomal-recessive inherited lysosomal storage disorder of childhood. Core symptoms include vision loss, seizures, and mental and motor decline. This article presents data from 2 studies of neuropsychological function in juvenile neuronal ceroid lipofuscinosis. In the first cross-sectional pilot study, 15 children with genetic or clinicopathologic confirmation of juvenile neuronal ceroid lipofuscinosis completed a brief test of attention (mean age = 14.3 +/- 2.9 years, range = 8.75-18.74 years; 7 males, 8 females). Average attention performances were significantly below age-expected normative data. A second longitudinal study was then initiated to study neuropsychological function in greater depth, including change in function over time. The authors have enrolled 18 children to date (mean age = 12.88 +/- 3.59 years, range = 6.26-18.65; 11 males, 7 females). Of these, 5 children have completed a second (annual) re-evaluation. Results thus far indicate significant impairment in domains of auditory attention, memory, estimated verbal intellectual function, and verbal fluency. Neuropsychological impairment was significantly correlated with disease duration and with motor function as assessed by a disease-specific clinical neurologic rating scale. There was no significant difference between males and females in neuropsychological test performance. Neuropsychological function was worse among children with a positive seizure history. Juvenile neuronal ceroid lipofuscinosis-affected children exhibited significant and pervasive impairments on tests of auditory attention, verbal memory and repetition, verbal fluency, and an estimate of verbal intellectual ability. Preliminary follow-up data from an annual reassessment showed progressive declines in cognitive function, in particular on a task of working memory. Neuropsychological deficits are pervasive and progressive. Future research will

  16. Adult-onset juvenile xanthogranuloma of the external auditory canal: A case report

    Energy Technology Data Exchange (ETDEWEB)

    Hur, Joon Ho; Kim, Jae Kyun; Seo, Gi Young; Choi, Woo Sun; Byun, Jun Soo; Lee, Woong Jae; Lee, Tae Jin [Chung-Ang University College of Medicine, Chung-Ang University Hospital, Seoul (Korea, Republic of); Kim, Na Ra [Dept. of Radiology, Samsung Medical Center, Sungkyunkwan University College of Medicine, Seoul (Korea, Republic of)

    2016-05-15

    Juvenile xanthogranuloma (JXG) is a benign, spontaneously regressing lesion that usually occurs during the first year of life, but may also occur in adulthood. Although the most common presentation of JXG is the cutaneous lesion, it can also manifest in various visceral organs. JXG of the external auditory canal is extremely rare, and there have been only a few reports of those cases in the English literature. In this study, we present a case of pathologically proven JXG that occurred in the external auditory canal with a symptomatic clinical presentation.

  17. Adult-onset juvenile xanthogranuloma of the external auditory canal: A case report

    International Nuclear Information System (INIS)

    Hur, Joon Ho; Kim, Jae Kyun; Seo, Gi Young; Choi, Woo Sun; Byun, Jun Soo; Lee, Woong Jae; Lee, Tae Jin; Kim, Na Ra

    2016-01-01

    Juvenile xanthogranuloma (JXG) is a benign, spontaneously regressing lesion that usually occurs during the first year of life, but may also occur in adulthood. Although the most common presentation of JXG is the cutaneous lesion, it can also manifest in various visceral organs. JXG of the external auditory canal is extremely rare, and there have been only a few reports of those cases in the English literature. In this study, we present a case of pathologically proven JXG that occurred in the external auditory canal with a symptomatic clinical presentation

  18. Associations of clinical features and prognosis with age at disease onset in patients with systemic lupus erythematosus.

    Science.gov (United States)

    Feng, X; Zou, Y; Pan, W; Wang, X; Wu, M; Zhang, M; Tao, J; Zhang, Y; Tan, K; Li, J; Chen, Z; Ding, X; Qian, X; Da, Z; Wang, M; Sun, L

    2014-03-01

    The objective of this study is to evaluate the association of clinical features and prognosis with age at disease onset in patients with systemic lupus erythematosus (SLE) in a large, multicenter Chinese cohort. Medical records of 1898 SLE inpatients from 15 hospitals were reviewed and classified into three groups according to their ages at disease presentation. Categorical data were analyzed by chi-square test and potentially associated factors were tested by multinomial logistic regression. Among the patients studied, 259 (13.6%) were juvenile onset (≤18 years), 1444 (76.1%) were early onset (>18 and ≤45 years) and 195 (10.3%) were late onset (>45 years). Whenever manifestations occurred, most patients (>80%) were diagnosed within two years. Juvenile-onset patients were more likely to be untreated before admission (p lupus. Interestingly, our data showed that more patients with late-onset disease had a SLEDAI score change of >7 at discharge. In conclusion, age at onset has an impact on SLE disease status, and infection is the main cause of death in those with late-onset lupus. Considering that the late-onset patients had simultaneously easily controllable diseases and high incidence of comorbidities, a different treatment strategy from younger patients should be considered.

  19. HLA-B27 predicts a more extended disease with increasing age at onset in boys with juvenile idiopathic arthritis

    DEFF Research Database (Denmark)

    Berntson, Lillemor; Damgård, Michael; Andersson-Gäre, Boel

    2008-01-01

    OBJECTIVE: Juvenile idiopathic arthritis (JIA) is a heterogeneous condition with very few clinical and laboratory signs that can help predict the course and severity of the disease in the individual patient. The cell-surface antigen HLA-B27 is well known to be associated with spondyloarthropathies...... to a population-based study as possible. METHODS: We studied an incidence-based cohort of 305 patients collected prospectively in 3 Nordic countries (Sweden, Norway, Denmark). Clinical and serological data of the first 3 years of the disease were collected. RESULTS: HLA-B27 was found to be positive in 25.......5% of the patients, and we found a higher proportion of HLA-B27-positive boys with older age at disease onset (p=0.034). Regression analysis showed a correlation of 0.7 in the HLA-B27-positive boys, pointing to a higher risk of more joint involvement with older age at disease onset. By Fisher's exact test...

  20. Neurological soft signs in juvenile patients with Asperger syndrome, early-onset psychosis, and healthy controls.

    Science.gov (United States)

    Mayoral, María; Merchán-Naranjo, Jessica; Rapado, Marta; Leiva, Marta; Moreno, Carmen; Giráldez, Marisa; Arango, Celso; Parellada, Mara

    2010-11-01

    The study of neurological soft signs (NSS) in patients with Asperger syndrome may help us to elucidate the neurological basis of this disorder and to clarify its relationship with other neurodevelopmental disorders. The goal of this study was to compare the prevalence of NSS in a sample of patients with Asperger syndrome, early-onset psychosis and healthy controls. NSS were assessed by means of the Neurological Evaluation Scale in a sample of 29 patients with Asperger syndrome (mean age = 12.86 ± 2.58 years), 30 patients with first-episode early-onset psychoses (mean age 14.17 ± 1.02 years) and 30 healthy controls (mean age 12.33 ± 2.69 years). Significant group differences were found between Asperger syndrome patients and healthy controls both in all the Neurological Evaluation Scale subscales and in the Neurological Evaluation Scale total score. There were no significant differences between both groups of patients in any of the Neurological Evaluation Scale scores. NSS are more prevalent in Asperger syndrome than in healthy controls. The NSS profile was not disorder-specific in our samples of patients with Asperger syndrome and early-onset psychoses. © 2010 Blackwell Publishing Asia Pty Ltd.

  1. Calcinosis in juvenile dermatomyositis is influenced by both anti-NXP2 autoantibody status and age at disease onset.

    Science.gov (United States)

    Tansley, Sarah L; Betteridge, Zoe E; Shaddick, Gavin; Gunawardena, Harsha; Arnold, Katie; Wedderburn, Lucy R; McHugh, Neil J

    2014-12-01

    Calcinosis is a major cause of morbidity in JDM and has previously been linked to anti-NXP2 autoantibodies, younger age at disease onset and more persistent disease activity. This study aimed to investigate the clinical associations of anti-NXP2 autoantibodies in patients with JDM stratified by age at disease onset. A total of 285 patients with samples and clinical data were recruited via the UK Juvenile Dermatomyositis Cohort and Biomarker Study. The presence of anti-NXP2 was determined by both immunoprecipitation and ELISA. Logistic regression analysis was performed to assess the age-dependent relationship between anti-NXP2 and the development of calcinosis and disease activity measures. We identified anti-NXP2 autoantibodies in 56 patients (20%). While in all patients younger age at disease onset was associated with an increased risk of calcinosis and this relationship was nearly linear, anti-NXP2 autoantibodies substantially increased the risk of calcinosis across all ages (P = 0.025) and were detectable prior to calcinosis development. Children with anti-NXP2 autoantibodies had a greater degree of weakness (median lowest ever Childhood Myositis Assessment Score 29.6 vs 42) and were less likely to be in remission at 2 years post-diagnosis. No difference in disease activity was seen 4 years post-diagnosis. Children diagnosed at a young age have a high risk of calcinosis regardless of autoantibody status. However, the presence of anti-NXP2 autoantibodies substantially increases the risk of calcinosis across all ages and is associated with disease severity. © The Author 2014. Published by Oxford University Press on behalf of the British Society for Rheumatology.

  2. Metabolic factors in the development of retinopathy of juvenile-onset type I diabetes mellitus

    Directory of Open Access Journals (Sweden)

    Khosla P

    1994-01-01

    Full Text Available Thirty-five patients of insulin-dependent diabetes mellitus (IDDM were investigated for the effect of various metabolic factors on retinopathy. The severity of retinopathy increased with duration and age of onset of IDDM. Degree of glycaemia (fasting blood sugar, FBS was similar in patients with or without retinopathy. All IDDM patients as a group showed severe carbohydrate intolerance with lower basal and post glucose serum immunoreactive insulin (IRI levels and serum C-peptide radioimmunoreactivity (CPR as compared to controls. The insulin secretory response was similar in no retinopathy, mild retinopathy and severe retinopathy groups. Patients with retinopathy had higher incidence of hyperlipidemia but mean serum levels of cholesterol and triglyceride were similar. This study does not suggest a direct relationship between the various metabolic factors studied and retinopathy due to IDDM

  3. Time outdoors, visual activity, and myopia progression in juvenile-onset myopes.

    Science.gov (United States)

    Jones-Jordan, Lisa A; Sinnott, Loraine T; Cotter, Susan A; Kleinstein, Robert N; Manny, Ruth E; Mutti, Donald O; Twelker, J Daniel; Zadnik, Karla

    2012-10-01

    To investigate the association between myopia progression and time spent outdoors and in various visual activities. Subjects were 835 myopes (both principal meridians -0.75 diopters [D] or more myopia by cycloplegic autorefraction) in the Collaborative Longitudinal Evaluation of Ethnicity and Refractive Error (CLEERE) Study with both progression data and at least one measure of activity associated with a progression interval. Activity data were collected by parental survey. Average activity level (mean of the activity at the beginning and the end of a 1-year progression interval) was the primary predictor in a repeated-measures mixed model. The model controlled for age, sex, ethnicity, refractive error at the beginning of the progression interval, clinic site, and type of autorefractor used. Effects were scaled based on performing an additional 10 hours per week of an activity. In the multivariate model, the number of hours of reading for pleasure per week was not significantly associated with annual myopia progression at an a priori level of P ≤ 0.01, nor were the other near activities, the near-work composite variable diopter-hours, or outdoor/sports activity. The magnitude of effects was clinically small. For example, the largest multivariate effect was that each additional 10 hours of reading for pleasure per week at the end of a progression interval was associated with an increase in average annual progression by -0.08 D. Despite protective associations previously reported for time outdoors reducing the risk of myopia onset, outdoor/sports activity was not associated with less myopia progression following onset. Near work also had little meaningful effect on the rate of myopia progression.

  4. Update on differences between childhood-onset and adult-onset systemic lupus erythematosus

    Science.gov (United States)

    2013-01-01

    Systemic lupus erythematosus (SLE) is a complex autoimmune disease and occurs worldwide in both children and adults. The estimated annual incidence among children is 2.22/100,000 and among adults is 23.2/100,000 in the United States. There is increasing understanding about differences in disease manifestations, medication use, and disease severity between those with childhood-onset SLE as compared with adult-onset SLE. Children have a more fulminant disease onset and course than adults with SLE, resulting in two to three times higher mortality. In future years, we anticipate more insight into the genetics between childhood-onset SLE and adult-onset SLE to help delineate the best therapies for both subsets of patients. PMID:23998441

  5. Nymphal RNAi: systemic RNAi mediated gene knockdown in juvenile grasshopper

    Directory of Open Access Journals (Sweden)

    Dong Ying

    2005-10-01

    Full Text Available Abstract Background Grasshopper serves as important model system in neuroscience, development and evolution. Representatives of this primitive insect group are also highly relevant targets of pest control efforts. Unfortunately, the lack of genetics or gene specific molecular manipulation imposes major limitations to the study of grasshopper biology. Results We investigated whether juvenile instars of the grasshopper species Schistocerca americana are conducive to gene silencing via the systemic RNAi pathway. Injection of dsRNA corresponding to the eye colour gene vermilion into first instar nymphs triggered suppression of ommochrome formation in the eye lasting through two instars equivalent to 10–14 days in absolute time. QRT-PCR analysis revealed a two fold decrease of target transcript levels in affected animals. Control injections of EGFP dsRNA did not result in detectable phenotypic changes. RT-PCR and in situ hybridization detected ubiquitous expression of the grasshopper homolog of the dsRNA channel protein gene sid-1 in embryos, nymphs and adults. Conclusion Our results demonstrate that systemic dsRNA application elicits specific and long-term gene silencing in juvenile grasshopper instars. The conservation of systemic RNAi in the grasshopper suggests that this pathway can be exploited for gene specific manipulation of juvenile and adult instars in a wide range of primitive insects.

  6. Differences in clinical features observed between childhood-onset versus adult-onset systemic lupus erythematosus

    Science.gov (United States)

    Bundhun, Pravesh Kumar; Kumari, Alka; Huang, Feng

    2017-01-01

    Abstract Background: Systemic lupus erythematosus (SLE) affects people in childhood (childhood onset) or in adulthood (adult onset). Observational studies that have previously compared childhood-onset versus adult-onset SLE were often restricted to 1 ethnic group, or to a particular area, with a small sample size of patients. We aimed to systematically compare childhood-onset versus adult-onset SLE through a meta-analysis. Methods: Electronic databases were searched for relevant publications comparing childhood-onset with adult-onset SLE. Adverse clinical features were considered as the endpoints. The Newcastle Ottawa Scale (NOS) was used to assess the methodological quality of the studies and RevMan software (version 5.3) was used to carry out this analysis whereby risk ratios (RRs) and 95% confidence intervals (95% CIs) were used as the statistical parameters. Results: A total number of 10,261 participants (1560 participants with childhood-onset SLE and 8701 participants with adult-onset SLE) were enrolled. Results of this analysis showed that compared with childhood-onset SLE, pulmonary involvement was significantly higher with adult-onset SLE (RR: 1.51, 95% CI: 1.18–1.93; P = .001), whereas renal involvement was significantly higher with childhood-onset SLE (RR: 0.65, 95% CI: 0.55–0.77; P = .00001). Raynaud phenomenon and photosensitivity were significantly higher in adult-onset SLE (RR: 1.29, 95% CI: 1.04–1.60; P = .02) and (RR: 1.08, 95% CI: 1.01–1.17; P = .03), respectively. Malar rash significantly favored adult-onset SLE (RR: 0.84, 95% CI: 0.75–0.94; P = .002). Childhood-onset SLE was associated with significantly higher hemolytic anemia, thrombocytopenia, leukocytopenia, and lymphopenia. Seizure and ocular manifestations were significantly higher with childhood-onset SLE (RR: 0.57, 95% CI: 0.47–0.70; P = .00001) and (RR: 0.34, 95% CI: 0.21–0.55; P = .00001), respectively, whereas pleuritis was significantly higher

  7. Police restorative approach in the juvenile justice system

    Directory of Open Access Journals (Sweden)

    Stefanovska Vesna

    2010-01-01

    Full Text Available Restorative justice is a new, different response to crime, response that offers and tries to establish justice again. Not going into aims, impact and basic principles of restorative justice, as well as into substance of different restorative practices, in this article we will concentrate on restorative interventions that lead to avoidance of the formal justice system. Concretely, we will analyze the role of the police in applying restorative interventions in the juvenile justice system. Particular emphasis will be put on the meaning and the aim of diversion procedures towards juvenile offenders that have committed minor offences and more serious ones for which they come in conflict with the law. In the foreign expert literature the concept of restorative policing is recognized (restorative approach in police conduct, as an attempt to introduce a new reform in performing police affairs. This subject should be approached very carefully and fundamentally, if we want consistent implementation of the new tendency and practices in the juvenile justice systems in accordance with the international standards.

  8. Just Learning: The Imperative to Transform Juvenile Justice Systems into Effective Educational Systems. A Study of Juvenile Justice Schools in the South and the Nation. Special Summary

    Science.gov (United States)

    Southern Education Foundation, 2014

    2014-01-01

    This brief summarizes the findings of the larger study, "Just Learning: The Imperative to Transform Juvenile Justice Systems into Effective Educational Systems. A Study of Juvenile Justice Schools in the South and the Nation." With awareness growing that schools are disciplining and suspending minority students at alarming rates, the…

  9. Integration of HPV6 and downregulation of AKR1C3 expression mark malignant transformation in a patient with juvenile-onset laryngeal papillomatosis.

    Directory of Open Access Journals (Sweden)

    Christian Ulrich Huebbers

    Full Text Available Juvenile-onset recurrent respiratory papillomatosis (RRP is associated with low risk human papillomavirus (HPV types 6 and 11. Malignant transformation has been reported solely for HPV11-associated RRP in 2-4% of all RRP-cases, but not for HPV6. The molecular mechanisms in the carcinogenesis of low risk HPV-associated cancers are to date unknown. We report of a female patient, who presented with a laryngeal carcinoma at the age of 24 years. She had a history of juvenile-onset RRP with an onset at the age of three and subsequently several hundred surgical interventions due to multiple recurrences of RRP. Polymerase chain reaction (PCR or bead-based hybridization followed by direct sequencing identified HPV6 in tissue sections of previous papilloma and the carcinoma. P16(INK4A, p53 and pRb immunostainings were negative in all lesions. HPV6 specific fluorescence in situ hybridization (FISH revealed nuclear staining suggesting episomal virus in the papilloma and a single integration site in the carcinoma. Integration-specific amplification of papillomavirus oncogene transcripts PCR (APOT-PCR showed integration in the aldo-keto reductase 1C3 gene (AKR1C3 on chromosome 10p15.1. ArrayCGH detected loss of the other gene copy as part of a deletion at 10p14-p15.2. Western blot analysis and immunohistochemistry of the protein AKR1C3 showed a marked reduction of its expression in the carcinoma. In conclusion, we identified a novel molecular mechanism underlying a first case of HPV6-associated laryngeal carcinoma in juvenile-onset RRP, i.e. that HPV6 integration in the AKR1C3 gene resulted in loss of its expression. Alterations of AKR1C gene expression have previously been implicated in the tumorigenesis of other (HPV-related malignancies.

  10. Juvenile Onset HD

    Science.gov (United States)

    ... to meet the Compassionate Allowance listing. The preferable sources of this information are the clinical records from the treating primary physician, neurologist, or psychiatrist including: Treating physician records ...

  11. EFFECTIVENESS OF INFLIXIMAB IN PATIENT WITH SYSTEMIC TYPE OF JUVENILE RHEUMATOID ARTHRITIS

    Directory of Open Access Journals (Sweden)

    E.S. Zholobova

    2011-01-01

    Full Text Available The article presents the case report of a child with juvenile rheumatoid arthritis, systemic type. The efficacy of infliximab (Remicade in treatment of systemic types of juvenile rheumatoid arthritis (JRA is analyzed. Treatment of JRA with high doses of prednisolone is inacceptable due to the risk of severe adverse events.Key words: juvenile rheumatoid arthritis, systemic type, infliximab, prednisolone, treatment.(Voprosy sovremennoi pediatrii — Current Pediatrics. 2011; 10 (2: 201–204

  12. A novel KCNQ1 missense mutation identified in a patient with juvenile-onset atrial fibrillation causes constitutively open IKs channels.

    Science.gov (United States)

    Hasegawa, Kanae; Ohno, Seiko; Ashihara, Takashi; Itoh, Hideki; Ding, Wei-Guang; Toyoda, Futoshi; Makiyama, Takeru; Aoki, Hisaaki; Nakamura, Yoshihide; Delisle, Brian P; Matsuura, Hiroshi; Horie, Minoru

    2014-01-01

    Atrial fibrillation (AF) is one of the most common cardiac arrhythmias. In some patients, the disease is inheritable; however, hereditary aspects of AF remain not fully elucidated. The purpose of this study was to identify genetic backgrounds that contribute to juvenile-onset AF and to define the mechanism. In 30 consecutive juvenile-onset AF patients (onset age KCNQ1, KCNH2, KCNE1-3, KCNE5, KCNJ2, SCN5A). We analyzed the function of mutant channels using whole-cell patch-clamp techniques and computer simulations. Among the juvenile-onset AF patients, we identified three mutations (10%): SCN5A-M1875T, KCNJ2-M301K, and KCNQ1-G229D. Because KCNQ1 variant (G229D) identified in a 16-year-old boy was novel, we focused on the proband. The G229D-IKs was found to induce a large instantaneous activating component without deactivation after repolarization to -50 mV. In addition, wild-type (WT)/G229D-IKs (WT and mutant coexpression) displayed both instantaneous and time-dependent activating currents. Compared to WT-IKs, the tail current densities in WT/G229D-IKs were larger at test potentials between -130 and -40 mV but smaller at test potentials between 20 and 50 mV. Moreover, WT/G229D-IKs resulted in a negative voltage shift for current activation (-35.2 mV) and slower deactivation. WT/G229D-IKs conducted a large outward current induced by an atrial action potential waveform, and computer simulation incorporating the WT/G229D-IKs results revealed that the mutation shortened atrial but not ventricular action potential. A novel KCNQ1-G229D mutation identified in a juvenile-onset AF patient altered the IKs activity and kinetics, thereby increasing the arrhythmogenicity to AF. © 2013 Heart Rhythm Society Published by Heart Rhythm Society All rights reserved.

  13. Pathogenic Variant in ACTB, p.Arg183Trp, Causes Juvenile-Onset Dystonia, Hearing Loss, and Developmental Delay without Midline Malformation

    Directory of Open Access Journals (Sweden)

    Erin Conboy

    2017-01-01

    Full Text Available ACTB encodes the β-actin, and pathogenic variations in this gene have typically been associated with Baraitser-Winter cerebrofrontofacial syndrome, a congenital malformation syndrome characterized by short stature, craniofacial anomalies, and cerebral anomalies. Here, we describe the third case with the p.Arg183Trp variant in ACTB causing juvenile-onset dystonia. Our patient has severe, intractable dystonia, developmental delay, and sensorineural hearing loss, besides hyperintensities in the caudate nuclei and putamen on the brain MRI, which is a distinct but overlapping phenotype with the previously reported case of identical twins with the same alteration in ACTB.

  14. Superior oblique tendon (Brown’s syndrome as the presenting finding in childhood onset HLA-B27-related enthesitis and juvenile idiopathic oligoarticular arthritis

    Directory of Open Access Journals (Sweden)

    C. Pham

    2014-11-01

    Full Text Available We report two patients who presented with Brown’s syndrome. The first is a 7-year-old boy who at the time of his diagnosis was also found to have enthesitis and HLA-B27 positivity. The second patient was diagnosed with bilateral Brown’s syndrome at 13 months of age. At age 7 she developed a persistent oligoarticular arthritis and unilateral anterior iritis consistent with the oligoarticular Juvenile Idiopatic Arthritis (JIA phenotype. These cases highlight ophthalmologic findings and diagnostic considerations with respect to Brown’s syndrome and associated childhood onset rheumatologic disease.

  15. [Macrophage activation syndrome in a patient with systemic juvenile idiopathic arthritis].

    Science.gov (United States)

    Tavares, Anna Carolina Faria Moreira Gomes; Ferreira, Gilda Aparecida; Guimarães, Luciano Junqueira; Guimarães, Raquel Rosa; Santos, Flávia Patrícia Sena Teixeira

    2015-01-01

    Machrophage activation syndrome (MAS) is a rare and potentially fatal disease, commonly associated with chronic rheumatic diseases, mainly juvenile idiopathic arthritis. It is included in the group of secondary forms of haemophagocytic syndrome, and other causes are lymphoproliferative diseases and infections. Its most important clinical and laboratorial manifestations are non-remitting fever, splenomegaly, bleeding, impairment of liver function, cytopenias, hypoalbuminemia, hypertriglyceridemia, hypofibrinogenemia and hyperferritinemia. The treatment needs to be started quickly, and the majority of cases have a good response with corticosteroids and cyclosporine. The Epstein-Barr virus is described as a possible trigger for many cases of MAS, especially in these patients in treatment with tumor necrosis factor (TNF) blockers. In these refractory cases, etoposide (VP16) should be administered, associated with corticosteroids and cyclosporine. Our objective is to describe a rare case of MAS probably due to EBV infection in a subject with systemic-onset juvenile idiopathic arthritis, which achieved complete remission of the disease after therapy guided by 2004-HLH protocol. Copyright © 2014 Elsevier Editora Ltda. All rights reserved.

  16. Are Bicipital Synovial Cysts in Children with Systemic Juvenile Idiopathic Arthritis still a Significant Clinical Challenge?

    DEFF Research Database (Denmark)

    Kyvsgaard, Nini; Herlin, Troels

    2015-01-01

    BACKGROUND: Large synovial cysts are rarely seen in juvenile idiopathic arthritis. When they do appear, they usually appear in the popliteal space (Baker’s cyst). Less commonly, they occur in the antecubital area or as bicipital synovial cysts. Bicipital synovial cysts present as a sudden-onset p...

  17. Mental Health and the Juvenile Justice System: Issues Related to Treatment and Rehabilitation

    Science.gov (United States)

    Hovey, Katrina A.; Zolkoski, Staci M.; Bullock, Lyndal M.

    2017-01-01

    Children and youth with mental health issues and learning difficulties are common in the juvenile justice system and finding ways to effectively rehabilitate, treat, and educate them is complicated, yet imperative. In this article, we examine the prevalence rates of mental health disorders in youth involved in the juvenile justice system, discuss…

  18. Seizure and Psychosocial Outcomes of Childhood and Juvenile Onset Generalized Epilepsies: Wolf in Sheep's Clothing, or Well-Dressed Wolf?

    Science.gov (United States)

    Nickels, Katherine

    2015-01-01

    Studies of generalized electroclinical syndromes can provide guidance regarding long-term seizure, cognitive, and psychosocial outcomes. Childhood absence epilepsy, juvenile absence epilepsy, juvenile myoclonic epilepsy, and idiopathic generalized epilepsy with generalized tonic-clonic seizures alone are electroclinical syndromes typically associated with normal intellect and good response to antiseizure medications. However, studies have demonstrated significantly poorer psychosocial outcomes than expected for these syndromes, regardless of seizure control. Potential causes for this include underlying abnormalities in social skills, social stigma, and underlying abnormalities in brain development and maturation.

  19. Juvenile technologies as a system of organisation of social partnership of the state and society

    Directory of Open Access Journals (Sweden)

    Portnyagina E.V.

    2016-01-01

    Full Text Available The article presents the analysis of different approaches to the definition of "juvenile technologies", presented both in scientific publications and in the activities of institutions engaged in the work with minors, including those who have found themselves in difficult situations and/or in conflict with the law. The analysis of the organization of interdepartmental cooperation between governmental bodies of Omsk Region and social institutions, as well as educational organizations on the implementation of juvenile technologies in Omsk region has been conducted. The study allowed posing the problem of inconsistencies in the understanding of juvenile technologies, which does not allow establishing an effective juvenile system. The authors propose an alternative vision of the concepts. It is proposed to increase the efficiency of the use of juvenile technologies at the expense of the organization of continuous monitoring of the implementation of juvenile technologies in the region, where pedagogical, psychological, legal, financial valuation criteria would be presented.

  20. Síndrome de ativação macrofágica associada com artrite idiopática juvenil sistêmica Macrophage activation syndrome associated with systemic juvenile idiopathic arthritis

    Directory of Open Access Journals (Sweden)

    Clovis Artur A. Silva

    2004-12-01

    Full Text Available OBJETIVO: Descrever as características da síndrome de ativação macrofágica associada a artrite idiopática juvenil. DESCRIÇÃO DOS CASOS: Foram analisados retrospectivamente os prontuários de 462 pacientes com artrite idiopática juvenil. Destes, sete (1,5% pacientes desenvolveram síndrome de ativação macrofágica; todos tinham a forma sistêmica da doença. A mediana de idade de início da artrite idiopática juvenil foi de 3 anos e 10 meses, e a mediana do tempo de duração da artrite idiopática juvenil antes da síndrome de ativação macrofágica foi de 8 anos e 4 meses. Todos os pacientes apresentaram febre, icterícia, hepatoesplenomegalia, sangramentos, pancitopenia e elevação das enzimas hepáticas e dos tempos de coagulação e bilirrubina direta. Três casos apresentaram infecções associadas e um caso desenvolveu a síndrome de ativação macrofágica 2 semanas após a introdução de sulfasalazina. Três pacientes morreram. Proliferação macrofágica e hemofagocitose foram evidenciadas em cinco. A terapêutica da síndrome de ativação macrofágica incluiu pulsoterapia com metilprednisolona em todos, ciclosporina em três, plasmaférese em dois e gamaglobulina endovenosa em dois. COMENTÁRIOS: A síndrome de ativação macrofágica é uma complicação da artrite idiopática juvenil sistêmica com alta morbidade e mortalidade.OBJECTIVE: To describe the characteristics of macrophage activation syndrome associated with juvenile idiopathic arthritis. DESCRIPTION: This is a retrospective study involving 462 patients with juvenile idiopathic arthritis. Seven (1.5% of those patients suffered from systemic onset juvenile idiopathic arthritis and developed macrophage activation syndrome. The median age of the juvenile idiopathic arthritis onset was 3 years and 10 months and the median duration of juvenile idiopathic arthritis before macrophage activation syndrome was 8 years and 4 months. All of them presented with fever

  1. Baby with neonatal systemic juvenile xanthogranuloma born within a cross-cousin marriage

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    Hikmet Tekin Nacaroglu

    2015-12-01

    Full Text Available Juvenile xanthogranuloma is a non-Langerhans cell histiocytosis seen most commonly in childhood and adolescence. Extracutaneous involvement is rare. We report an interesting and extremely rare case of systemic (skin, lung, spleen, and colon “juvenile xanthogranuloma” in the neonatal period. Our case was the first ever reported case born to a cross-cousin marriage.

  2. New-onset systemic lupus erythematosus during pregnancy.

    Science.gov (United States)

    Zhao, Chunmei; Zhao, Jijun; Huang, Yuefang; Wang, Zilian; Wang, Hongyue; Zhang, Hui; Xu, Hanshi; Yang, Niansheng

    2013-06-01

    Few studies have been published focusing on the clinical features of new-onset systemic lupus erythematosus (SLE) during pregnancy. This study examined the clinical characteristics of SLE during pregnancy or puerperium. The clinical characteristics and serological parameters of 48 patients with onset of SLE during pregnancy were retrospectively compared with those of age-matched new-onset SLE patients who were diagnosed in a period of more than 12 months without pregnancy (n = 65) and age-matched preeclampsia patients (n = 48). SLE tended to occur during the first and second trimesters (33 and 42 %, respectively). Lupus nephritis (LN) and severe thrombocytopenia were more commonly seen in new-onset SLE during pregnancy than in patients without pregnancy (68.8 vs 35.4 % and 25 vs 9.2 %, respectively, p pregnancy (n = 23), LN patients with pregnancy (n = 33) had more prominent proteinuria and nephrotic syndrome (p pregnancy had early onset of symptoms during gestation and were characterized by presence of fever, malar lesion, autoantibodies, hypocomplementemia, hyperuricemia, active urinary sediment, and multi-organ involvement. In conclusion, patients with their first onset of lupus during pregnancy generally have more severe disease with higher prevalence of renal and platelet involvement.

  3. Pregnancy outcomes in women with childhood-onset and adult-onset systemic lupus erythematosus: a comparative study.

    Science.gov (United States)

    Saavedra, Miguel Ángel; Miranda-Hernández, Dafhne; Sánchez, Antonio; Morales, Sara; Cruz-Domínguez, Pilar; Medina, Gabriela; Jara, Luis Javier

    2016-10-01

    To compare the maternal and fetal outcomes between childhood-onset and adult-onset systemic lupus erythematosus (SLE), we reviewed the medical records of SLE pregnant women treated from January 2005 to August 2013. For comparison, patients were allocated to one of the two groups, those pregnant patients with SLE onset before 18 years of age (childhood-onset) and ≥18 years (adult-onset). The patients were evaluated at least once in each trimester and postpartum. Relevant maternal and fetal outcomes were extracted, such as lupus flare, preeclampsia/eclampsia, rate of liveborns, fetal loss (spontaneous abortion and stillbirth), term delivery, preterm birth, neonatal death, low birth weight, low birth weight at term, and congenital malformations. We studied 186 pregnancies (in 180 women), 58 of them had childhood-onset SLE, and the remaining 128 had adult-onset SLE. The rate of maternal and fetal complications was similar in both groups. Multivariate analysis showed that active SLE before pregnancy, primigravida, renal flare, preeclampsia, lupus flare, anticardiolipin antibodies, and low serum complement were associated with an increased risk of poor maternal and fetal outcomes. The diagnosis of childhood-onset had no impact on maternal-fetal outcome. The maternal and fetal outcome in women with childhood-onset SLE is similar to that reported in women with adult-onset SLE. Pregnancy in women with childhood-onset SLE should not be contraindicated if the disease is well controlled.

  4. Bone mineral density in juvenile systemic lupus erythematosus

    Directory of Open Access Journals (Sweden)

    Castro T.C.M.

    2002-01-01

    Full Text Available We evaluated spine bone mineral density (BMD in Brazilian children with juvenile systemic lupus erythematosus (JSLE in order to detect potential predictors of reduction in bone mass. A cross-sectional study of BMD at the lumbar spine level (L2-L4 was conducted on 16 female JSLE patients aged 6-17 years. Thirty-two age-matched healthy girls were used as control. BMD at the lumbar spine was measured by dual-energy X-ray absorptiometry. Weight, height and pubertal Tanner stage were determined in patients and controls. Disease duration, mean daily steroid doses, mean cumulative steroid doses and JSLE activity measured by the systemic lupus erythematosus disease activity index (SLEDAI were determined for all JSLE patients based on their medical charts. All parameters were used as potential determinant factors for bone loss. Lumbar BMD tended to be lower in the JSLE patients, however, this difference was not statistically significant (P = 0.10. No significant correlation was observed in JSLE girls between BMD and age, height, Tanner stage, disease duration, corticosteroid use or disease activity. We found a weak correlation between BMD and weight (r = 0.672. In the JSLE group we found no significant parameters to correlate with reduced bone mass. Disease activity and mean cumulative steroid doses were not related to BMD values. We did not observe reduced bone mass in female JSLE.

  5. EXPERIENCE OF TREATMENT WITH RITUXIMAB IN PATIENT WITH SYSTEMIC TYPE OF JUVENILE RHEUMATOID ARTHRITIS

    Directory of Open Access Journals (Sweden)

    K.B. Isaeva

    2009-01-01

    Full Text Available The article gives a case report on patient with severe systemic type of juvenile arthritis, refractory to treatment with classical immuno suppressants and blocker of tumor necrotizing factor Successive application of biological agent — rituximab was described. In 9 weeks, extra articular symptoms of disease and acute inflammatory lesions in joints were stopped, and functional activity of child increased. Presented case report demonstrates high effectiveness of rituximab: patient with severe systemic type of juvenile arthritis has clinical and laboratory remission of disease during 12 months.Key words: children, juvenile rheumatoid arthritis, rituximab.(Voprosy sovremennoi pediatrii — Current Pediatrics. 2009;8(3:132-138

  6. Chest Abnormalities in Juvenile-Onset Mixed Connective Tissue Disease: Assessment with High-Resolution Computed Tomography and Pulmonary Function Tests

    International Nuclear Information System (INIS)

    Aaloekken, T.M.; Mynarek, G.; Kolbenstvedt, A.; Lilleby, V.; Foerre, Oe.; Soeyseth, V.; Pripp, A.H.; Johansen, B.

    2009-01-01

    Background: Mixed connective tissue disease (MCTD) is associated with several chest manifestations. Only a few studies have focused on chest manifestations in juvenile-onset MCTD (jMCTD), and the true prevalence of pulmonary abnormalities on high-resolution computed tomography (HRCT) in these patients is unknown. Purpose: To investigate the occurrence of pulmonary abnormalities in jMCTD with particular reference to interstitial lung disease (ILD), and to evaluate a possible association between pulmonary findings and disease-related variables. Material and Methods: Twenty-four childhood-onset MCTD patients with median disease duration of 10.5 years (range 1-21 years) were investigated in a cross-sectional study by means of HRCT, pulmonary function tests (PFT), and clinical assessment. Results: Discrete ILD was identified in six patients (25%). Median extent of ILD was 2.0%, and all except one of the patients had very mild disease in which 5% or less of the parenchyma was affected. The CT features of fibrosis were mainly microcystic and fine intralobular. The most frequently abnormal PFT was carbon monoxide uptake from the lung, which was abnormal in 33% of the patients. PFT and disease duration were not significantly associated with HRCT findings of ILD. Conclusion: The prevalence of ILD in childhood-onset MCTD patients was lower than previously believed. In most of the patients with ILD, the findings were subtle and without clinical correlation. The results suggest a low extent of ILD in childhood-onset MCTD, even after long-term disease duration

  7. Juvenile Salmon Acoustic Telemetry System Transmitter Downsize Assessment

    Energy Technology Data Exchange (ETDEWEB)

    Carlson, Thomas J.; Myjak, Mitchell J.

    2010-04-30

    At the request of the U.S. Army Corps of Engineers, Portland District, researchers from Pacific Northwest National Laboratory investigated the use of an application-specific integrated circuit (ASIC) to reduce the weight and volume of Juvenile Salmon Acoustic Telemetry System (JSATS) transmitters while retaining current functionality. Review of the design of current JSATS transmitters identified components that could be replaced by an ASIC while retaining the function of the current transmitter and offering opportunities to extend function if desired. ASIC design alternatives were identified that could meet transmitter weight and volume targets of 200 mg and 100 mm3. If alternatives to the cylindrical batteries used in current JSATS transmitters can be identified, it could be possible to implant ASIC-based JSATS transmitters by injection rather than surgery. Using criteria for the size of fish suitable for surgical implantation of current JSATS transmitters, it was concluded that fish as small as 70 mm in length could be implanted with an ASIC-based transmitter, particularly if implantation by injection became feasible.

  8. Transportation of juvenile tambaqui (Colossoma macropomum in a closed system

    Directory of Open Access Journals (Sweden)

    L. C. Gomes

    Full Text Available The objective of this study was to investigate the effect of density, duration and the use of additives to the water during the transportation of juvenile tambaqui (Colossoma macropomum and use of this data to establish a safe transportation protocol for the species. The tested products and dosages were: salt (1000, 2000 and 3000 mg/L, gypsum (100, 300 and 500 mg/L and benzocaine (10, 20 and 30 mg/L. Fish were transported in closed systems (plastic bag at different densities and time periods of up to 24 h. Fish survival (FS and water quality parameters were monitored immediately after transportation. The remaining fish were kept in floating cages in order to evaluate mortality which occurred up to 96 h after transportation (S96. The best fish density, additives dosages and time period of the transportation was estimated with a general linear model. The effect of the condition factor on FS and S96 was also evaluated. As expected, FS and S96 were significantly related to time and density. FS but not S96, were also were significantly related to treatment. FS with gypsum treatment was not different from controls and FS with table salt and benzocaine treatments were significantly reduced. The condition factor was not related to either FS or S96. FS was inversely correlated with carbon dioxide concentration. It was concluded that the additives did not improve fish transportation survival. Linear models were developed to predict the best transportation densities as a function of time.

  9. Transportation of juvenile tambaqui (Colossoma macropomum) in a closed system.

    Science.gov (United States)

    Gomes, L C; Araujo-Lima, C A R M; Chippari-Gomes, A R; Roubach, R

    2006-05-01

    The objective of this study was to investigate the effect of density, duration and the use of additives to the water during the transportation of juvenile tambaqui (Colossoma macropomum) and use of this data to establish a safe transportation protocol for the species. The tested products and dosages were: salt (1000, 2000 and 3000 mg/L), gypsum (100, 300 and 500 mg/L) and benzocaine (10, 20 and 30 mg/L). Fish were transported in closed systems (plastic bag) at different densities and time periods of up to 24 h. Fish survival (FS) and water quality parameters were monitored immediately after transportation. The remaining fish were kept in floating cages in order to evaluate mortality which occurred up to 96 h after transportation (S96). The best fish density, additives dosages and time period of the transportation was estimated with a general linear model. The effect of the condition factor on FS and S96 was also evaluated. As expected, FS and S96 were significantly related to time and density. FS but not S96, were also were significantly related to treatment. FS with gypsum treatment was not different from controls and FS with table salt and benzocaine treatments were significantly reduced. The condition factor was not related to either FS or S96. FS was inversely correlated with carbon dioxide concentration. It was concluded that the additives did not improve fish transportation survival. Linear models were developed to predict the best transportation densities as a function of time.

  10. CLINICAL CASE OF TOCILIZUMAB THERAPY IN A PATIENT WITH SYSTEMIC JUVENILE IDIOPATHIC ARTHRITIS

    Directory of Open Access Journals (Sweden)

    E. I. Alexeeva

    2013-01-01

    Full Text Available The article presents a case of successful application of a monoclonal antibodies drug to interleukin 6 receptors (tocilizumab at severe systemic juvenile idiopathic arthritis with the development of secondary hemophagocytic syndrome. Tocilizumab treatment secured a decrease in clinical and laboratory parameters of the disease activity, life quality improvement, systemic juvenile idiopathic arthritis and hemophagocytic syndrome remission and allowed avoiding the per os prescription of glucocorticoids.

  11. A Critical Appraisal of the Juvenile Justice System under Cameroon's 2005 Criminal Procedure Code: Emerging Challenges

    Directory of Open Access Journals (Sweden)

    S Tabe

    2012-03-01

    Full Text Available The objective of this article is to examine the changes introduced by the 2005 Cameroonian Criminal Procedure Code on matters of juvenile justice, considering that before this Code, juvenile justice in Cameroon was governed by extra-national laws. In undertaking this analysis, the article highlights the evolution of the administration of juvenile justice 50 years after independence of Cameroon. It also points out the various difficulties and shortcomings in the treatment of juvenile offenders in Cameroon since the enactment of the new Criminal Procedure Code. The article reveals that the 2005 Code is an amalgamation of all hitherto existing laws in the country that pertained to juvenile justice, and that despite the considerable amount of criticism it has received, the Code is clearly an improvement of the system of juvenile justice in Cameroon, since it represents a balance of the due process rights of young people, the protection of society and the special needs of young offenders. This is so because the drafters of the Code took a broad view of the old laws on juvenile justice. Also a wide range of groups were consulted, including criminal justice professionals, children’s service organisations, victims, parents, young offenders, educators, advocacy groups and social-policy analysts. However, to address the challenges that beset the juvenile justice system of Cameroon, the strategy of the government should be focussed on three areas: the prevention of youth crime, the provision of meaningful consequences for the actions of young people, and the rehabilitation and reintegration of young offenders. Cameroonian law should seek educative solutions rather than to impose prison sentences or other repressive measures on young offenders. Special courts to deal with young offenders should be established outside the regular penal system and should be provided with resources that are adequate for and appropriate to fostering their understanding of

  12. Systemic Besnoitiosis in a Juvenile Roe Deer (Capreolus capreolus).

    Science.gov (United States)

    Arnal, M C; Gutiérrez-Expósito, D; Martínez-Durán, D; Regidor-Cerrillo, J; Revilla, M; Fernández de Luco, D; Jiménez-Meléndez, A; Ortega-Mora, L M; Álvarez-García, G

    2017-10-01

    Herein, we report the first incidence of systemic besnoitiosis in a male juvenile roe deer Capreolus capreolus. The animal was found dead in an area where bovine besnoitiosis is endemic and showed cachexia and multiple skin erosions in the metacarpal and metatarsal areas. Moreover, round and elevated white structures suggestive of Besnoitia spp. tissue cysts were also present. Twenty-eight tissue samples from different anatomical locations were collected for microscopic lesion and parasite detection through histopathology and PCR. Immunohistochemistry was performed to confirm Besnoitia-positive reaction in the tissue cysts. In addition, the identity of Besnoitia spp. in PCR-positive tissue samples was also investigated using microsatellite (MS) markers, and the comparison of protein disulphide isomerase gene sequences (BbPDI) of B. besnoiti and B. tarandi isolated from cattle and reindeer, respectively. Besnoitia cysts were detected in the skin (several parts), respiratory and upper digestive tracts, eyes, kidney, liver, testicle, cardiac muscle and lymphoid tissue. Remarkably, the presence of tissue cysts in the brain confirmed the capacity of Besnoitia spp. to form tissue cysts in the central nervous system (CNS). Finally, the Besnoitia species detected showed the same MS genotype as B. besnoiti, and BbPDI sequences from roe deer and two B. besnoiti isolates were genetically identical throughout multiple sequence alignment. Thus, for the first time, there is evidence that roe deer might act as an intermediate host of B. besnoiti. Further molecular analyses and parasite isolations are needed to corroborate these findings. © 2016 Blackwell Verlag GmbH.

  13. JUVENILE RHEUMATOID ARTHRITIS

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    I N Sartika

    2012-11-01

    Full Text Available Juvenile rheumatoid arthritis (JRA is the most common rheumatic condition in children. JRA is defined as persistent arthritis in 1 or more joints for at least 6 weeks, with the onset before age 16 years. The etiology of JRA is unknown. Antigen activated CD4+ T cell stimulate monocytes, macrophages, and synovial fibroblasts to produce the cytokines Interleukin-1 (IL-1, IL-6, and tumor necrosis factor ? (TNF-? and to secrete matrix metalloproteinases, which lead to chronic inflammation due to infiltration of inflammatory cell, angiogenesis, destruction of cartilage and bone with pannus formation. The 3 major subtypes of JRA are based on the symptoms at disease onset and are designated systemic onset, pauciarticular onset, and polyarticular onset. For all patients, the goals of therapy are to decrease chronic joint pain and suppress the inflammatory process. Poor prognostic have been observed in patients with polyarticular onset, rheumatoid factor, persistent morning stiffness, tenosynovitis, involvement of the small joints, rapid appearance of erosions, active late onset childhood, subcutaneous nodules, or antinuclear antibody.

  14. Wheel running from a juvenile age delays onset of specific motor deficits but does not alter protein aggregate density in a mouse model of Huntington's disease

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    Spires Tara L

    2008-04-01

    Full Text Available Abstract Background Huntington's disease (HD is a neurodegenerative disorder predominantly affecting the cerebral cortex and striatum. Transgenic mice (R6/1 line, expressing a CAG repeat encoding an expanded polyglutamine tract in the N-terminus of the huntingtin protein, closely model HD. We have previously shown that environmental enrichment of these HD mice delays the onset of motor deficits. Furthermore, wheel running initiated in adulthood ameliorates the rear-paw clasping motor sign, but not an accelerating rotarod deficit. Results We have now examined the effects of enhanced physical activity via wheel running, commenced at a juvenile age (4 weeks, with respect to the onset of various behavioral deficits and their neuropathological correlates in R6/1 HD mice. HD mice housed post-weaning with running wheels only, to enhance voluntary physical exercise, have delayed onset of a motor co-ordination deficit on the static horizontal rod, as well as rear-paw clasping, although the accelerating rotarod deficit remains unaffected. Both wheel running and environmental enrichment rescued HD-induced abnormal habituation of locomotor activity and exploratory behavior in the open field. We have found that neither environment enrichment nor wheel running ameliorates the shrinkage of the striatum and anterior cingulate cortex (ACC in HD mice, nor the overall decrease in brain weight, measured at 9 months of age. At this age, the density of ubiquitinated protein aggregates in the striatum and ACC is also not significantly ameliorated by environmental enrichment or wheel running. Conclusion These results indicate that enhanced voluntary physical activity, commenced at an early presymptomatic stage, contributes to the positive effects of environmental enrichment. However, sensory and cognitive stimulation, as well as motor stimulation not associated with running, may constitute major components of the therapeutic benefits associated with enrichment

  15. Treating Juvenile Crime.

    Science.gov (United States)

    Gelber, Seymour

    1983-01-01

    Although juvenile crime rates have not changed significantly in the last five years, the juvenile courts' ability to handle crime has deteriorated. To treat the problem of juvenile crime effectively requires intervention at the earliest sign of delinquency and an assessment of the juvenile courts and school system. (AM)

  16. Children within the Juvenile Justice System in Nigeria ...

    African Journals Online (AJOL)

    Background: Many children in Nigeria face a life of poverty, family instability, inadequate educational opportunities and poor physical and mental health which hinder their ability to develop into healthy adults, live an improved quality of life or fulfil their life aspirations. These factors have also been associated with juvenile ...

  17. A Critical Appraisal of the Juvenile Justice System under ...

    African Journals Online (AJOL)

    This is so because the drafters of the Code took a broad view of the old laws on juvenile justice. Also a wide range of groups were consulted, including criminal justice professionals, children's service organisations, victims, parents, young offenders, educators, advocacy groups and social-policy analysts. However, to ...

  18. Motor system hyperconnectivity in juvenile myoclonic epilepsy: a cognitive functional magnetic resonance imaging study.

    Science.gov (United States)

    Vollmar, Christian; O'Muircheartaigh, Jonathan; Barker, Gareth J; Symms, Mark R; Thompson, Pamela; Kumari, Veena; Duncan, John S; Janz, Dieter; Richardson, Mark P; Koepp, Matthias J

    2011-06-01

    Juvenile myoclonic epilepsy is the most frequent idiopathic generalized epilepsy syndrome. It is characterized by predominant myoclonic jerks of upper limbs, often provoked by cognitive activities, and typically responsive to treatment with sodium valproate. Neurophysiological, neuropsychological and imaging studies in juvenile myoclonic epilepsy have consistently pointed towards subtle abnormalities in the medial frontal lobes. Using functional magnetic resonance imaging with an executive frontal lobe paradigm, we investigated cortical activation patterns and interaction between cortical regions in 30 patients with juvenile myoclonic epilepsy and 26 healthy controls. With increasing cognitive demand, patients showed increasing coactivation of the primary motor cortex and supplementary motor area. This effect was stronger in patients still suffering from seizures, and was not seen in healthy controls. Patients with juvenile myoclonic epilepsy showed increased functional connectivity between the motor system and frontoparietal cognitive networks. Furthermore, we found impaired deactivation of the default mode network during cognitive tasks with persistent activation in medial frontal and central regions in patients. Coactivation in the motor cortex and supplementary motor area with increasing cognitive load and increased functional coupling between the motor system and cognitive networks provide an explanation how cognitive effort can cause myoclonic jerks in juvenile myoclonic epilepsy. The supplementary motor area represents the anatomical link between these two functional systems, and our findings may be the functional correlate of previously described structural abnormalities in the medial frontal lobe in juvenile myoclonic epilepsy.

  19. Radiologic findings in late-onset systemic lupus erythematosus

    Energy Technology Data Exchange (ETDEWEB)

    Braunstein, E.M.; Weissman, B.N.; Sosman, J.L.; Schur, P.H.

    1983-03-01

    Systemic lupus erythematosus in the elderly has a different clinical and serologic course from that in young patients. Radiographic findings in patients in whom the diagnosis was made after age 50 were compared with findings in younger patients to see if the radiologic patterns are also different. The only significant radiographic difference between the two groups was that the older group had a greater incidence of soft-tissue swelling of the hands and wrists (p < 0.001). There was no significant difference in osteopenia, erosion, soft-tissue calcification, alignment abnormalities, or intrathoracic findings. Of 24 patients over age 50, two developed lymphoma and another developed multiple myeloma. The data agree with clinical observations that there is a higher incidence of arthritis in late-onset lupus, but clinical findings of increased incidence of pleuropericardial disease are not confirmed radiographically. The coincidence of hematologic malignancy with late-onset lupus in this series is noteworthy.

  20. Radiologic findings in late-onset systemic lupus erythematosus

    International Nuclear Information System (INIS)

    Braunstein, E.M.; Weissman, B.N.; Sosman, J.L.; Schur, P.H.

    1983-01-01

    Systemic lupus erythematosus in the elderly has a different clinical and serologic course from that in young patients. Radiographic findings in patients in whom the diagnosis was made after age 50 were compared with findings in younger patients to see if the radiologic patterns are also different. The only significant radiographic difference between the two groups was that the older group had a greater incidence of soft-tissue swelling of the hands and wrists (p < 0.001). There was no significant difference in osteopenia, erosion, soft-tissue calcification, alignment abnormalities, or intrathoracic findings. Of 24 patients over age 50, two developed lymphoma and another developed multiple myeloma. The data agree with clinical observations that there is a higher incidence of arthritis in late-onset lupus, but clinical findings of increased incidence of pleuropericardial disease are not confirmed radiographically. The coincidence of hematologic malignancy with late-onset lupus in this series is noteworthy

  1. Gastrointestinal system manifestations in juvenile systemic lupus erythematosus.

    Science.gov (United States)

    Sönmez, Hafize Emine; Karhan, Asuman Nur; Batu, Ezgi Deniz; Bilginer, Yelda; Gümüş, Ersin; Demir, Hülya; Yüce, Aysel; Özen, Seza

    2017-07-01

    Systemic lupus erythematosus (SLE) is an autoimmune disease which may involve gastrointestinal system (GIS). The aim of this study was to present GIS manifestations of pediatric SLE patients. The medical files of 69 children with SLE followed between January 2011 and January 2016 were reviewed. All fulfilled the Systemic Lupus International Collaborating Clinics criteria. All patients (≤18 years of age) with GIS manifestations were included. GIS manifestations were observed in 19 (27.5%) out of 69 SLE patients and present at the time of SLE diagnosis in 13 (68.4%). The GIS manifestations due to SLE were autoimmune hepatitis (AIH) (n = 8) and lupus enteritis (n = 1). Manifestations associated with SLE were hepatomegaly and hypertransaminasemia due to macrophage activation syndrome (MAS) (n = 3) and hepatic steatosis (n = 1). GIS manifestations as a result of the adverse events of drugs were as follows: toxic hepatitis (n = 3; associated with methotrexate and nonsteroidal anti-inflammatory drugs in one, methotrexate in another, and azathioprine in another patient), azathioprine-induced cholestatic hepatitis (n = 1), and gastritis associated with corticosteroid (n = 1). In one patient, acute appendicitis occurred as a coincidence. In this study, one of every five pediatric SLE patients had GIS-related manifestations. GIS involvement may occur as an initial manifestation of the disease.

  2. Being (almost invisible: Victims of crime in the Italian juvenile criminal justice system

    Directory of Open Access Journals (Sweden)

    Vezzadini Susanna

    2014-01-01

    Full Text Available From 2008 to 2013 the author has been a Special Judge in the Juvenile Criminal Court of the Emilia Romagna Region. From that privileged perspective, it was possible to observe the dynamics of how victims of underage offenders were considered before the law, no differences if they are adults or minors, too. The reflections presented will first consider EU and UN provision on victims of crime; then, the normative framework supporting the Italian criminal juvenile justice system will be considered by an examining of the difficulties victims meet in that peculiar context. The implementation of juvenile criminal law shows the paradox victims of crime have to cope with. The Juvenile Criminal Court in Bologna recently started to promote a wide use of restorative justice measures as an attempt to correct the unfair consequences in the application of law, with judicial discretion interpreted as an instrument to favour victims’ harm recognition and to protect their dignity as persons.

  3. Zinc transporter-8 autoantibodies can replace IA-2 autoantibodies as a serological marker for juvenile onset type 1 diabetes in India

    Directory of Open Access Journals (Sweden)

    C Shivaprasad

    2014-01-01

    Full Text Available Introduction: Zinc transporter-8 (ZnT8 is an islet cell secretory granule membrane protein recently identified as an autoantigen in type 1 diabetes (T1D. The aim of this study was to estimate the prevalence of antibodies to ZnT8 (ZnT8A in juvenile onset T1D and to determine the utility of ZnT8A as an independent marker of autoimmunity either alone in antibody-negative subjects or in conjunction with glutamic acid decarboxylase (GAD and insulinoma-2 antigen antibodies (GADA and IA2A. Research Design: ZnT8A, GADA, and IA2A were measured in sera of consecutive T1D patients (n = 88, age range 2-18 years within 4 years of diagnosis and 88 sex-matched controls. Results: The prevalences of GADA, ZnT8a, and IA2A were 64.7%, 31.8% and 19.3%, respectively. In newly diagnosed patients, the frequency of ZnT8A was 45%. ZnT8A were positive in 26% of patients negative for both GADA and IA2A. IA2A were positive only in two patients who were negative for other two antibodies. Combined use of ZnT8A and GADA could detect 97% of antibody positive patients. In receiver operating characteristic (ROC analysis, the performances of GADA and ZnT8As were better than that of IA2A; and AUCs of GADA, ZnT8A, and IA2A for the prediction of T1D were 0.8, 0.65, and 0.59, respectively. Conclusions: ZnT8A complements GADA and increases the diagnostic sensitivity for detection of autoimmunity in juvenile-onset T1D. Inclusion of ZnT8A increases the proportion of patients with antibody positivity to nearly 80%. ZnT8A can replace IA2A as a serological marker for autoimmunity in Indian T1D patients without loss of sensitivity and specificity.

  4. RESEARCHES REGARDING THE GROWTH OF HYPOPTHALMICHTHYS MOLITRIX AND CTENOPHARYNGODON IDELLA JUVENILES IN AN INTENSIVE SYSTEM

    Directory of Open Access Journals (Sweden)

    ALINA RODICA ANI

    2008-10-01

    Full Text Available The intensive net cage system is based on the superior capitalization of theaccumulation lakes or other different basins through the use of different types anddimensions, stationary or floating net cages, depending on categories like age,species and destination. This study is about the use of net cage intensive system typein the growth of juveniles Ctenopharyngodon idella and Hypopthalmichthys molitrixspecies. From the experimental point of view, the objectives were represented by theconstruction of a net cage mini-system and the surveillance of the biologicalmaterial inside this system. The experiment was unfolded on the length of 38 days atthe Mǎrtinesti Fish Farm from Cluj County. A net cage system designed andconceived by the research collective was populated with 4000 juveniles fish fromeach species. At the beginning of this experiment the juveniles fish had a mediumweight of 2 grams/sample. At the end of this experiment the fish had a mediumweight of 9 grams.

  5. Pathophysiological characteristics of preproinsulin-specific CD8+ T cells in subjects with juvenile-onset and adult-onset type 1 diabetes: A 1-year follow-up study.

    Science.gov (United States)

    Paul, Mahinder; Badal, Darshan; Jacob, Neenu; Dayal, Devi; Kumar, Rakesh; Bhansali, Anil; Bhadada, Sanjay Kumar; Sachdeva, Naresh

    2018-02-01

    Among the beta-cell associated antigens, preproinsulin (PPI) has been shown to play a key role in the pathogenesis of type 1 diabetes (T1D). PPI-specific autoreactive CD8+ T cells emerge early during beta-cell destruction and persist in peripheral circulation during diabetes progression. However, the influence of insulin therapy on phenotype of autoreactive CD8+ T cells in T1D including, juvenile-onset T1D (JOT1D), and adult-onset T1D (AOT1D) is not yet known. We followed the time course of PPI-specific CD8+ T cells in JOT1D and AOT1D subjects that achieved glycemic control after 1 year of insulin therapy, using major histocompatibility complex-I (MHC-I) dextramers by flow cytometry. At follow-up, PPI-specific CD8+ T cells could be detected consistently in peripheral blood of all T1D subjects. Proportion of PPI-specific effector memory (T EM ) subsets decreased, while central memory T (T CM ) cells remained unchanged in both groups. Expression of granzyme-B and perforin in PPI-specific CD8+ T cells also remained unchanged. Further, on analysis of B-chain and signal peptide (SP) specific CD8+ T cell responses separately, we again observed decrease in T EM subset in both the groups, while increase in naive (T N ) subset was observed in B-chain specific CD8+ T cells only. Our study shows that PPI-specific CD8+ T cells can be detected in both JOT1D and AOT1D subjects over a period of time with reliable consistency in frequency but variable pathophysiological characteristics. Insulin therapy seems to reduce the PPI-specific T EM subsets; however, the PPI-specific T CM cells continue to persist as attractive targets for immunotherapy. © 2017 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  6. The incidence and prevalence of juvenile-onset recurrent respiratory papillomatosis in the Free State province of South Africa and Lesotho.

    Science.gov (United States)

    Seedat, R Y

    2014-12-01

    Although the estimated incidence and prevalence of juvenile-onset recurrent respiratory papillomatosis (JORRP) has been determined in countries in North America and Europe and in Australia, no studies have attempted to determine the incidence or prevalence of JORRP in African countries. The aim of this study is to determine the incidence and prevalence of JORRP in the Free State province of South Africa and Lesotho. This was a retrospective study in which the records of all patients with JORRP from the Free State province of South Africa or Lesotho treated at Universitas Academic Hospital or by otorhinolaryngologists in private practice between 1 January 2011 and 31 December 2013 were reviewed. The estimated incidence and prevalence of JORRP in the Free State were 1.34 and 3.88 per 100,000 population respectively while the estimated incidence and prevalence in Lesotho were 0.49 and 1.04 per 100,000 population respectively. However, these figures are probably an underestimation. The incidence and prevalence calculated for the Free State were generally higher than those found in other studies, while those calculated for Lesotho was similar to those obtained in other studies. Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.

  7. Enhanced Apoptosis of Monocytes from Complication-Free Juvenile-Onset Diabetes Mellitus Type 1 May Be Ameliorated by TNF-α Inhibitors

    Directory of Open Access Journals (Sweden)

    Jolanta Myśliwska

    2014-01-01

    Full Text Available Diabetes mellitus type 1 is associated with an enhanced apoptosis of different cells and tissues, accelerating occurrence of diabetic microvascular complications. The aim of our study was to determine spontaneous apoptotic potential of the monocyte subsets in juvenile-onset complication-free diabetes mellitus type 1 and to compare them with the corresponding values of the healthy. Moreover, we wanted to assess effects of TNF-R1 blocking agents and those of general TNF-α blocker (Infliximab on spontaneous apoptosis of monocytes. Sixty randomly selected DM1 patients (14.5 ± 3.2 years and 30 healthy (13.5 ± 2.8 years volunteers were enrolled in the study. Our results indicate that three monocyte subsets are distinguishable in the groups of young diabetic patients and the healthy, similarly to in the blood of adults. DM1 patients were characterized by higher values of apoptotic monocytes than the healthy. The manipulation with drugs inhibiting TNF-R1 expression diminished the pool of CD16+ apoptotic monocytes. Infliximab reduced the apoptotic CD16− cells. In conclusion, diabetes mellitus type 1 is associated with greater apoptosis of three monocyte subsets which may contribute to the development of microvascular complications. TNF-α modifiers appear to ameliorate monocyte apoptosis. They may be useful for controlling excessive monocyte apoptosis in diabetic patients.

  8. Juvenile Justice System in Italy. Research and interventions / Sistema de Justicia Juvenil en Italia: investigaciones e intervenciones

    Directory of Open Access Journals (Sweden)

    Patrizia Meringolo

    2012-12-01

    Full Text Available This paper talks about the juvenile justice system in Italy. The author describes the interventions done with minors, boys and girls aged from 14 until 18 years, who have committed offenses of the civil or penal code, by the New Code of Criminal Procedure for Minors (1988. The Procedures have had some positive psychological aspects, aimed to avoid detention,thanks to alternative measures and strategies for inclusion, including also the minors living in the South, that are often involved in mafia-crimes. Nonetheless there are more negative psychological issues, because alternative punishments are not often applied to minors that lack social networks, particularly to foreign ones. Three examples of participatory researcheswill be shown, promoted by the Municipality of Florence, Department of Psychology and Third Sector Associations, aimed to promote psychological and social inclusion of minors (particularly those coming from abroad, with the commitment of active citizenship organizations, with an evaluation oftheir strengths and weaknesses.

  9. Suicidal Ideation and Behavior in Youth in the Juvenile Justice System: A Review of the Literature.

    Science.gov (United States)

    Stokes, Marquita L; McCoy, Kathleen P; Abram, Karen M; Byck, Gayle R; Teplin, Linda A

    2015-07-01

    Suicide is prevalent among youth, especially those involved in the juvenile justice system. Although many studies have examined suicidal ideation and behavior in delinquent youth, prevalence rates vary widely. This article reviews studies of suicidal ideation and behavior in youth in the juvenile justice system, focusing on the point of contact: incarceration status and stage of judicial processing. Suicidal ideation and behavior are prevalent and increase with greater involvement in the juvenile justice system. Depression, sexual abuse, and trauma were the most commonly identified predictors of suicidal ideation and behavior. Prevalence rates of suicidal ideation and behavior vary by gender and race/ethnicity, indicating the need for gender-specific and culturally relevant interventions. © The Author(s) 2015.

  10. Delinquent-Victim Youth-Adapting a Trauma-Informed Approach for the Juvenile Justice System.

    Science.gov (United States)

    Rapp, Lisa

    2016-01-01

    The connection between victimization and later delinquency is well established and most youth involved with the juvenile justice system have at least one if not multiple victimizations in their history. Poly-victimized youth or those presenting with complex trauma require specialized assessment and services to prevent deleterious emotional, physical, and social life consequences. Empirical studies have provided information which can guide practitioners work with these youth and families, yet many of the policies and practices of the juvenile justice system are counter to this model. Many youth-serving organizations are beginning to review their operations to better match a trauma-informed approach and in this article the author will highlight how a trauma-informed care model could be utilized to adapt the juvenile justice system.

  11. Salivary proteomics: A new adjuvant approach to the early diagnosis of familial juvenile systemic lupus erythematosus.

    Science.gov (United States)

    Abrão, Aline Lauria P; Falcao, Denise Pinheiro; de Amorim, Rivadávio Fernandes Batista; Bezerra, Ana Cristina B; Pombeiro, Gilson Augusto N M; Guimarães, Luciano Junqueira; Fregni, Felipe; Silva, Luciano Paulino; da Mota, Licia Maria Henrique

    2016-04-01

    Systemic lupus erythematosus (SLE) is a chronic multisystemic disease characterized by autoimmune inflammatory disturbance. Pleomorphic manifestations are present and a potentially progressive and debilitating course can be detected. SLE rarely manifests before age 5, and its onset peaks is around puberty. Although clinical manifestations, immunological alterations and treatment do not differ between juvenile and adult SLE, children tend to present with a more aggressive disease course than adults. Hence, autoimmune rheumatic diseases are the most common cause of morbidity and mortality in pediatric populations. Blood serum analysis plays an especially important role in the detection and monitoring of autoantibodies in SLE. However, since blood sampling is an uncomfortable procedure, especially in children, novel less invasive techniques and approaches are of utmost importance to evaluate pediatric subjects. In this regard, saliva samples have several advantages, such as: easy access, fast collection, painless and riskless procedure. Saliva has antimicrobial, immunomodulatory and anti-inflammatory properties, as well as several other relevant features. The whole saliva is a complex mixture of major and minor salivary gland secretion, gingival crevicular fluid, transudates plasma protein, keratinocyte products and oral microbiota. This biological fluid reflects the physiological state of the body, including the emotional condition, and endocrine, nutritional and metabolic changes. Therefore, salivary proteomics is becoming increasingly used for the early diagnosis of several diseases such as breast cancer, oral cancer, Sjögren's syndrome, diffuse systemic sclerosis, rheumatoid arthritis, among others. Considering the detection of some potential markers related to SLE in serum and urine, this study aims to conduct an initial evaluation of the possible presence of such biomarkers in saliva. Furthermore, it is expected to track down new salivary proteins that could be

  12. Clinical Case of Tocilizumab Use in a Patient with Systemic Juvenile Idiopathic Arthritis

    Directory of Open Access Journals (Sweden)

    Y. M. Spivakovskiy

    2015-01-01

    Full Text Available The article presents a case of using genetically engineered biopharmaceutical tocilizumab in a child with systemic juvenile idiopathic arthritis. On the initial stage, the treatment was characterized by resistance to high doses of glucocorticoids and cytostatic drugs. Successful termination of visceral and articular manifestations of systemic juvenile idiopathic arthritis and normalization of laboratory indicators of disease activity in the setting of use of interleukin 6 receptor blocker were described. We observed stable improvement of the child’s condition during a year-long follow-up in the setting of the selected anti-inflammatory therapy pattern. 

  13. Skeletal manifestations of juvenile hypothyroidism and the impact of treatment on skeletal system

    Directory of Open Access Journals (Sweden)

    Manish Gutch

    2013-01-01

    Full Text Available Thyroid hormone mediates growth and development of the skeleton through its direct effects and through its permissive effects on growth hormone. The effect of hypothyroidism on bone is well described in congenital hypothyroidism, but the impact of thyroid hormone deficiency on a growing skeleton, as it happens with juvenile hypothyroidism, is less defined. In addition, the extent to which the skeletal defects of juvenile hypothyroidism revert on the replacement of thyroid hormone is not known. A study was undertaken in 29 juvenile autoimmune hypothyroid patients to study the skeletal manifestations of juvenile hypothyroidism and the impact of treatment of hypothyroidism on the skeletal system of juvenile patients. Hypothyroidism has a profound impact on the skeletal system and delayed bone age, dwarfism, and thickened bands at the metaphyseal ends being the most common findings. Post treatment, skeletal findings like delayed bone age and dwarfism improved significantly, but there were no significant changes in enlargement of sella, presence of wormian bones, epihyseal dysgenesis, vertebral changes and thickened band at the metaphyseal ends. With the treatment of hypothyroidism, there is an exuberant advancement of bone age, the catch up of bone age being approximately double of the chronological age advancement.

  14. Youth with Disabilities in the Corrections System: Prevalence Rates and Identification Issues. Monograph Series on Education, Disability and Juvenile Justice.

    Science.gov (United States)

    Rutherford, Robert B., Jr.; Bullis, Michael; Anderson, Cindy Wheeler; Griller-Clark, Heather M.

    This monograph, one of a series on youth with disabilities and the juvenile justice system, reviews current data on disabilities requiring special education and related supports. Statistics on the prevalence of juvenile crime are followed by statistics on the prevalence of special education disabilities in the system, specifically specific…

  15. Osteonecrosis of the jaw on imaging exams of patients with juvenile systemic lupus erythematosus.

    Science.gov (United States)

    Fernandes, Elisabeth Gonzaga Canova; Guissa, Vanessa Ramos; Saviolli, Cynthia; Siqueira, José Tadeu Tesseroli; Valente, Marcelo; da Silva, Clovis Artur Almeida

    2010-01-01

    The objective of the present study was to evaluate radiographic changes of the temporomandibular joint (TMJ) in patients with juvenile systemic lupus erythematosus (JSLE) and a control group. Panoramic radiographies of the TMJ of 26 JSLE patients and 28 healthy individuals were evaluated. Multislice computed tomography (MCT) was performed on those patients who presented flattening and/or destruction of mandibular condyles. Demographic data, oral health indices, clinical manifestations, laboratory exams, and treatment were evaluated. Important radiographic changes consistent with osteonecrosis of the mandible, confirmed by MCT of the TMJ, were observed in 2/26 (8%) JSLE patients versus 0% in the control group (P = 0.22). Mild clinical dysfunction and abnormal TMJ mobility were observed in 67% and 54% of the patients, respectively. Age of onset, disease duration, and current age were similar in JSLE patients with and without severe radiographic changes of TMJ (9.3 versus 10.8 years, P = 0.77; 3.3 versus 2 years, P = 0.63; 12.6 versus 13.5 years, P = 0.74, respectively). Significant differences in gender, socioeconomical status, oral health indices, clinical manifestations, laboratory exams, and treatment were not observed between both subgroups (P 0.05). Both JSLE patients with osteonecrosis of the mandible had active chronic disease, used corticosteroids for a prolonged period, and had mild TMJ dysfunction. Antiphospholipid antibodies were not observed in those two patients, and neither one had been treated with bisphosphonate. Osteonecrosis of the mandible with mild TMJ dysfunction was observed in some of the patients, demonstrating the importance of odontological assessment during clinical follow-up.

  16. Juvenile systemic lupus erythematosus in Portugal: clinical and immunological patterns of disease expression in a cohort of 56 patients.

    Science.gov (United States)

    Cabral, Marta; Escobar, Carlos; Conde, Marta; Ramos, Margarida; Melo Gomes, José A

    2013-01-01

    To define the pattern of disease expression and to gain better understanding in patients with juvenile onset systemic lupus erythematosus (SLE) in Portugal. The features of unselected patients with systemic lupus erythematosus who had disease onset before the age of 18 years were retrospectively analysed in three Portuguese centres with Pediatric Rheumatology Clinic over a 24-year period (1987-2011). Demographic, clinical and laboratory manifestations, therapy and outcome were assessed. A cohort of 56 patients with a mean age at disease onset of 12.6 ± 4.04 years (mean ± 1SD) (range, 1.0-17.0 years) and a mean period of follow-up of 5.5 ± 5.4 years. Forty six (82.1%) patients were female. The most common disease manifestations were musculoskeletal (87.5%), mucocutaneous (80.3%) and haematological abnormalities (75%). Lupus nephritis was diagnosed in 46.4% of patients and consisted of glomerular nephritis in all cases. Neuropsychiatric manifestations occurred in 21.4% but severe central nervous system complications were uncommon, as brain infarcts and organic brain syndrome in 4 (7.1%) patients. Antinuclear antibodies and anti-double stranded DNA were positive in most patients in (98.2% and 71.4% respectively), as well as low C3 and/or C4 were observed frequently (85.7%). Generally, most patients had a good response to therapy as demonstrated by a significant decreasing of SLEDAI score from disease presentation to the last evaluation. The SLEDAI at diagnosis, the maximum SLEDAI and the incidence of complications were significantly higher in patients with neurolupus and/or lupus nephritis. Therapy included oral steroids (87.5%), hydroxychloroquine (85.7%), azathioprine (55.4%), IV cyclophosphamide (28.6%) along with other drugs. Six (10.7%) patients were treated with rituximab. Long-term remission was achieved in 32%, disease was active in 68%, adverse reactions to therapy occurred in 53.6% and complications/severe manifestations in 23.2%. Two patients died, being

  17. Obsessive fears about harm to self or others and overt aggressive behaviors in youth diagnosed with juvenile-onset bipolar disorder.

    Science.gov (United States)

    Papolos, Demitri; Hennen, John; Cockerham, Melissa

    2005-12-01

    Obsessive fear-of-harm, either fear of doing harm or fear of harm coming to self, may be closely associated with aggressive behaviors in juvenile-onset bipolar disorder. We analyzed parent-report data on the Yale-Brown Obsessive Compulsive Scale (YBOCS) and Overt Aggression Scale (OAS) for 1601 children/adolescents with a clinician-assigned diagnosis of bipolar disorder. The summing of 6 YBOCS items rated "often" or "very often or almost constant" yielded a biphasic distribution of scores. Median-split was used to define meaningful subgroups contrasting high vs. low "fear-of-harm", which were then compared on parent-reported severe injury to self and others and on parent-reported suicide threats. High fear-of-harm was strongly associated with parent-reported severe injury to self and others. For self-injury, the estimated risk ratio for high vs. low fear-of-harm subgroups was 2.68 (95% confidence interval 1.87-3.86), indicating greater than doubling of risk associated with high fear-of-harm. For severe injury to others, the estimated risk ratio was 7.97 (95% confidence interval 4.19-15.2), suggesting a nearly eight-fold increased risk associated with high fear-of-harm. High fear-of-harm subjects were reported to make serious suicide threats much more frequently than low fear-of-harm subjects (odds ratio, estimated by ordinal logistic regression modeling methods, was 2.42 (95% CI 2.00 to 2.92; z=9.12, pObsessive fears about harm to self or others in a sample of children with a clinician-assigned diagnosis of bipolar disorder were found to be positively related to increased behavioral aggression towards self and others, as well as to frequent suicide threats.

  18. School Personnel Perceptions of Youth with Disabilities Returning to High School from the Juvenile Justice System

    Science.gov (United States)

    Sinclair, James S.; Unruh, Deanne K.; Griller Clark, Heather; Waintrup, Miriam G.

    2017-01-01

    Little is known about the perceptions of teachers of their students returning from the juvenile justice system, which can influence student relationships and student engagement, both of which are critical to reduce recidivism rates. This study utilized an online survey to examine the perceptions of a convenience sample of 283 school personnel…

  19. Special Education Advocacy in the Juvenile Justice System: Perspectives from Probation Officers

    Science.gov (United States)

    Burke, Meghan M.; Dalmage, Heather

    2016-01-01

    Although students with disabilities are over-represented in the juvenile justice system and frequently receive poor educational services, few studies have examined strategies to increase compliance with student needs and individualized education programs. In this study, we conducted interviews with eight probation officers in the Advocacy Unit of…

  20. Cluster-Derived Groupings of the Behavior Assessment System for Children among Male Juvenile Offenders

    Science.gov (United States)

    Scarborough, Zane T.; Glaser, Brian A.; Calhoun, Georgia B.; Stefurak, Tres; Petrocelli, John V.

    2004-01-01

    Examined the cluster-derived typologies of males in a juvenile offender sample. Adolescent males, age 12-17, in a regional youth detention center were given the Behavior Assessment System for Children-Self Report-Adolescent (BASC-SRP-A; C. Reynolds &R. Kamphaus, 1992) and the Millon Adolescent Clinical Inventory (MACI; T. Millon, 1993).…

  1. Disease activity patterns in juvenile systemic lupus erythematosus and its relation to early aggressive treatment

    NARCIS (Netherlands)

    Otten, M. H.; Cransberg, K.; van Rossum, M. A. J.; Groothoff, J. W.; Kist-van Holthe, J. E.; ten Cate, R.; van Suijlekom-Smit, L. W. A.

    2010-01-01

    This study aimed to determine disease activity patterns in juvenile systemic lupus erythematosus (jSLE) and its relation to early treatment. All jSLE patients who visited the outpatient departments of three Dutch university hospitals for at least 6 months were included. Data were retrospectively

  2. Hormone profile in juvenile systemic lupus erythematosus with previous or current amenorrhea

    NARCIS (Netherlands)

    Silva, Clovis A.; Deen, Maria E. J.; Febronio, Marilia V.; Oliveira, Sheila K.; Terreri, Maria T.; Sacchetti, Silvana B.; Sztajnbok, Flavio R.; Marini, Roberto; Quintero, Maria V.; Bica, Blanca E.; Pereira, Rosa M.; Bonfa, Eloisa; Ferriani, Virginia P.; Robazzi, Teresa C.; Magalhaes, Claudia S.; Hilario, Maria O.

    To identify the underlying mechanism of amenorrhea in juvenile systemic lupus erythematosus (JSLE) patients, thirty-five (11.7%) JSLE patients with current or previous amenorrhea were consecutively selected among the 298 post-menarche patients followed in 12 Brazilian pediatric rheumatology centers.

  3. Relative size of the eye and orbit: an evolutionary and craniofacial constraint model for examining the etiology and disparate incidence of juvenile-onset myopia in humans.

    Science.gov (United States)

    Masters, Michael P

    2012-05-01

    within the confines of the orbital walls. The results of this study indicate that future research examining the etiology of juvenile-onset myopia, and particularly its correlation with ancestry, sex, age, and intelligence, should consider how the eye interacts with the matrix of structural and functional components of the skull during both ontogenetic and evolutionary morphogenesis. Copyright © 2012 Elsevier Ltd. All rights reserved.

  4. EFFICACY OF RECURRENT RITUXIMAB TREATMENT IN PATIENT WITH SEVERE REFRACTORY SYSTEMIC JUVENILE RHEUMATOID ARTHRITIS

    Directory of Open Access Journals (Sweden)

    E.I. Alexeeva

    2011-01-01

    Full Text Available The article contains clinical case description of a severe systemic juvenile rheumatoid arthritis, that was refractory to classic immunosuppressant therapy. The disease was characterized by such extraarticular manifestations as fever, lymphadenopathy,  hepatosplenomegaly, polyserositis, generalized joint involvement and high activity in lab tests. As a result of severe clinical course of the disease, patients develop bilateral aseptic bone necrosis in coxofemoral joints and coxarthrosis. Against the background of glucocorticosteroid treatment the patient has developed hormone-dependency and hormone resistance. Inclusion into the treatment of anti-CD20 monoclonal antibodies (rituximab has stopped systemic manifestations of the disease, inflammation in the joints, normalized lab activity rates. The positive therapeutic effect allowed to perform surgery due to bilateral coxarthrosis. These results show that rituximab is highly effective in children with systemic juvenile rheumatoid arthritis, that is resistant to classic immunosupressants and glucocorticoides. Key words: children, systemic juvenile rheumatoid arthritis, rituximab, recurrent treatment, prosthetics, hip joint. (Voprosy sovremennoi pediatrii — Current Pediatrics. — 2011; 10 (5: 157–163.

  5. A Research Framework for Understanding the Practical Impact of Family Involvement in the Juvenile Justice System: The Juvenile Justice Family Involvement Model.

    Science.gov (United States)

    Walker, Sarah Cusworth; Bishop, Asia S; Pullmann, Michael D; Bauer, Grace

    2015-12-01

    Family involvement is recognized as a critical element of service planning for children's mental health, welfare and education. For the juvenile justice system, however, parents' roles in this system are complex due to youths' legal rights, public safety, a process which can legally position parents as plaintiffs, and a historical legacy of blaming parents for youth indiscretions. Three recent national surveys of juvenile justice-involved parents reveal that the current paradigm elicits feelings of stress, shame and distrust among parents and is likely leading to worse outcomes for youth, families and communities. While research on the impact of family involvement in the justice system is starting to emerge, the field currently has no organizing framework to guide a research agenda, interpret outcomes or translate findings for practitioners. We propose a research framework for family involvement that is informed by a comprehensive review and content analysis of current, published arguments for family involvement in juvenile justice along with a synthesis of family involvement efforts in other child-serving systems. In this model, family involvement is presented as an ascending, ordinal concept beginning with (1) exclusion, and moving toward climates characterized by (2) information-giving, (3) information-eliciting and (4) full, decision-making partnerships. Specific examples of how courts and facilities might align with these levels are described. Further, the model makes predictions for how involvement will impact outcomes at multiple levels with applications for other child-serving systems.

  6. Processes of constitution of the uruguayan juvenile criminal system. ¿hybrids paradigms?

    OpenAIRE

    López Gallego, Laura

    2017-01-01

    In this paper, the analysis focuses on adolescence as a particularized space of social criminal control   through devices that have historically constituted the Uruguayan juvenile criminal system. Social criminal control practices make up a heterogeneous field composed of a multiplicity of vectors which show the ways in which people are tried and/or treated according to their departures from the prevailing criminal law in a particularly socio-historical context . They set concrete social prac...

  7. PROCESSES OF CONSTITUTION OF THE URUGUAYAN JUVENILE CRIMINAL SYSTEM. ¿HYBRIDS PARADIGMS?

    OpenAIRE

    López Gallego, Laura

    2017-01-01

    In this paper, the analysis focuses on adolescence as a particularized space of social criminal control   through devices that have historically constituted the Uruguayan juvenile criminal system. Social criminal control practices make up a heterogeneous field composed of a multiplicity of vectors which show the ways in which people are tried and/or treated according to their departures from the prevailing criminal law in a particularly socio-historical context . They set concrete social prac...

  8. Miliary tuberculosis: a severe opportunistic infection in juvenile systemic lupus erythematosus patients

    OpenAIRE

    Freire, Priscilla S.; Montoni, João D.; Ribeiro, Aline S.M.; Marques, Heloísa H.; Mauad, Thais; Silva, Clovis A.

    2016-01-01

    Abstract Introduction One of the main issues in juvenile systemic lupus erythematosus (JSLE) patients is infection, such as tuberculosis (TB). Of note, SLE patients are susceptible to pulmonary and extrapulmonary TB. However, to our knowledge, this contagious disease was rarely reported in pediatric lupus population, particularly diffuse or miliary TB. Therefore, from January 1983 to December 2011, 5,635 patients were followed-up at our Pediatric Rheumatology Unit and 285 (5%) of them met th...

  9. Juvenile idiopathic arthritis (JIA): a screening study to measure class II skeletal pattern, TMJ PDS and use of systemic corticosteroids.

    LENUS (Irish Health Repository)

    Mandall, Nicky A

    2010-03-01

    To screen patients with oligoarticular and polyarticular forms of Juvenile Idiopathic Arthritis (JIA) to determine (i) the severity of their class II skeletal pattern; (ii) temporomandibular joint signs and symptoms and (iii) use of systemic corticosteroids.

  10. Trauma, Delinquency, and Substance Use: Co-occurring Problems for Adolescent Girls in the Juvenile Justice System

    OpenAIRE

    Smith, Dana K.; Saldana, Lisa

    2013-01-01

    Girls in the juvenile justice system are known to have high rates of co-occurring childhood abuse, trauma, and substance abuse. Girls with this constellation of problems are at high risk for serious adverse outcomes, including problems with drug dependence and abuse. The relationship between childhood sexual abuse, childhood physical abuse, other types of childhood trauma, and rates of substance use during adolescence were examined for girls in the juvenile justice system. As expected, childh...

  11. Youth, Guns, and the Juvenile Justice System. Research Report.

    Science.gov (United States)

    Butts, Jeffrey; Coggeshall, Mark; Gouvis, Caterina; Mears, Daniel; Travis, Jeremy; Waul, Michelle; White, Ruth

    This report documents trends in youth gun violence and the response within the justice system, noting the growing variety of data resources available to investigate the effect of new gun laws on youth, communities, and public safety. The first section reviews recent trends, examining the major wave of gun violence in the United States during the…

  12. Juvenile Arrests, 2007. Juvenile Justice Bulletin

    Science.gov (United States)

    Puzzanchera, Charles

    2009-01-01

    This Bulletin summarizes 2007 juvenile crime and arrest data reported by local law enforcement agencies across the country and cited in the FBI report, "Crime in the United States 2007." The Bulletin describes the extent and nature of juvenile crime that comes to the attention of the justice system. It serves as a baseline for comparison for…

  13. Oral health and the masticatory system in juvenile systemic lupus erythematosus.

    Science.gov (United States)

    Fernandes, E G C; Savioli, C; Siqueira, J T T; Silva, C A A

    2007-01-01

    Our objectives were to evaluate the oral health and the masticatory system of 48 juvenile systemic lupus erythematosus (JSLE) patients and to compare them with 48 healthy children and adolescents. Demographic data, clinical manifestations and therapies of JSLE were reviewed. The DMFT index (DMFTI), the plaque (PI) and the gingival bleeding (GI) indices, dental relationship, facial profile, clinical dysfunction and mandibular mobility indices were evaluated. The two groups were homogeneous regarding age, gender, Brazilian social-economic class and dental decay index (P > 0.05). Of note, the medians of the PI and the GI were higher in JSLE patients than in controls (61.5 versus 38.10, P = 0.003 and 26.0 versus 15.95, P = 0.014; respectively). Likewise, a linear statistical correlation was evidenced between the JSLE duration and the GI (P = 0.017, r = 0.11), cumulative dose of prednisone and the PI (P = 0.01, r = 0.385) and cumulative dose of prednisone and the GI ( P = 0.001, r = 0.471). The clinical dysfunction and mandibular mobility indices were higher in JSLE patients versus controls (P = 0.002, P = 0.025). Moreover, the median of the mandibular mobility index was higher in JSLE patients who used at least one immunosuppressive than on those who did not use this medication (P = 0.0001). These results suggest that JSLE patients had an inadequate oral hygiene, higher incidence of gingivitis and temporomandibular joint dysfunction.

  14. A radiographic classification system in juvenile rheumatoid arthritis applied to the knee

    International Nuclear Information System (INIS)

    Dale, K.; Paus, A.C.; Laires, K.

    1994-01-01

    A new radiographic grading system for evaluation of juvenile rheumatoid arthritis (JRA) for the knee is presented. The classification is based on known arthritic criteria in childhood. Joints with erosion are given a higher score than growth disturbances alone. Signs of osteoarthrosis including joint space narrowing were excluded from the classification. The femorotibial and patello-femoral joints are assessed together. Verbal definitions are used for the classification, but, regarding the erosions, standard reference films are used. The intra- and inter-observer variations of the method were low. (P < 0.01) (orig.)

  15. Juvenile amyotrophic lateral sclerosis.

    Science.gov (United States)

    Aggarwal, Anju; Shashiraj

    2006-03-01

    Juvenile amytrophic lateral sclerosis (JALS) is a type of motor neuron disease presenting before 25 years of age. It is characterized by a combination of upper and lower motor signs. It may be familial or sporadic. We are reporting a sporadic case of JALS with onset of symptoms at 4 years of age. Diagnostic criteria and a brief review of literature are presented.

  16. Naevoxanthoendothelioma (Synonym: Juvenile Xanthogranuloma

    Directory of Open Access Journals (Sweden)

    F Handa

    1978-01-01

    Full Text Available A case of naevoxanthoendothelioma juvenile xanthogranuloma is reported with rare features like late onset of the disease, involvement of liver and diffuse cutaneous lesions including cafe au lait spots and pigmented naevus. Final diagnosis could be achieved only on histopathology report.

  17. Social Justice for Crossover Youth: The Intersection of the Child Welfare and Juvenile Justice Systems.

    Science.gov (United States)

    Kolivoski, Karen M; Goodkind, Sara; Shook, Jeffrey J

    2017-10-01

    Social workers are critical to promoting racial and social justice. "Crossover youth," a term used to describe youths who have contact with both the child welfare and juvenile justice systems, are an especially vulnerable but often overlooked population with whom social workers engage. A disproportionate number of crossover youth are African American. Empirical research on crossover youth is growing, but such scholarship rarely engages with a human rights and social justice perspective. African American children and youths have a distinct place within the history and current context of the child welfare and juvenile justice systems. These systems have historically excluded them or treated them differently; now, African American youths are overrepresented in each of them, and evidence suggests they are more likely to cross over. The purpose of this article is to describe the historical and current context of crossover youth, with a particular focus on African American youths, to provide the foundation for a discussion of what social workers can do to promote racial and social justice for crossover youth, including specific implications for practice and policy, as well as broader implications for human and civil rights. © 2017 National Association of Social Workers.

  18. Accountability in Teenage Dating Violence: A Comparative Examination of Adult Domestic Violence and Juvenile Justice Systems Policies

    Science.gov (United States)

    Zosky, Diane L.

    2010-01-01

    Unlike in the adult criminal justice system, where domestic violence policies hold perpetrators accountable for their violence, the juvenile justice system rarely addresses teenage dating violence. Although the adult criminal justice system has pursued policies toward intimate partner violence grounded on a "zero tolerance" ideology, the juvenile…

  19. Perceived parental security profiles in African American adolescents involved in the juvenile justice system.

    Science.gov (United States)

    Andretta, James R; Ramirez, Aaron M; Barnes, Michael E; Odom, Terri; Roberson-Adams, Shelia; Woodland, Malcolm H

    2015-12-01

    Many researchers have shown the importance of parent attachment in childhood and adolescence. The present study extends the attachment literature to African Americans involved in the juvenile justice system (N = 213), and provides an initial inquiry using person-oriented methods. The average age was 16.17 years (SD = 1.44), and the sample was predominantly male (71%). Results of a confirmatory factor analysis of Inventory of Parent and Peer Attachment-Short Form (IPPA-S) scores supported a 3-factor model: (a) Communication, (b) Trust, and (c) Alienation. Model-based clustering was applied to IPPA-S scores, and results pointed to 4 perceived parental security profiles: high security, moderately high security, moderately low security, and low security. In keeping with our hypotheses, IPPA-S profiles were associated with prosocial behaviors, depression, anxiety, and oppositional defiance. Contrary to hypotheses, IPPA-S profiles were not associated with perspective taking, emotional concern, or behaviors characteristic of a conduct disorder. Results also showed that gender, age, family member with whom the participant resides, charge severity, and offense history did not have an effect on IPPA-S clustering. Implications for therapeutic jurisprudence in African Americans involved with the juvenile justice system are provided. (c) 2015 APA, all rights reserved).

  20. Idiopathic Pulmonary Hemosiderosis in a Child with Recurrent Macrophage Activation Syndrome Secondary to Systemic Juvenile Idiopathic Arthritis

    Directory of Open Access Journals (Sweden)

    Kenan Barut

    2017-01-01

    Full Text Available Macrophage activation syndrome, a severe complication of systemic juvenile idiopathic arthritis and other inflammatory diseases, represents one of the most important rheumatological emergencies. Delayed diagnosis could lead to life-threatening complications. Pulmonary hemosiderosis has been classically characterized by a triad of anemia, hemoptysis, and lung infiltrates on chest radiogram. Although the majority of patients of pulmonary hemosiderosis are considered idiopathic, secondary hemosiderosis associated with known diseases could be seen. In this case report, we aimed to present gradually increased pulmonary manifestations due to pulmonary hemosiderosis with recurrent macrophage activation syndrome attacks in a child with systemic juvenile idiopathic arthritis.

  1. Explaining Ethnic Inequality in the Juvenile Justice System, an analysis of the outcomes of Dutch Prosecutorial Decision Making

    NARCIS (Netherlands)

    Weenink, D.

    2009-01-01

    Most studies of the treatment of minorities in criminal justice systems show that ethnic minorities are punished more harshly. This paper aims to explain ethnic inequality in prosecutorial decision making in the Dutch juvenile justice system. Based on statistical analyses of 409 case files, it

  2. Power, Process, and Protection: Juveniles as Defendants in the Justice System.

    Science.gov (United States)

    Woolard, Jennifer L; Henning, Kristin; Fountain, Erika

    The juvenile court was created in 1899 in part to remedy the unfairness of trying youth in the adult criminal justice system, but its success at rectifying those problems is unclear. One concern is that the vast majority of youth who are adjudicated delinquent are adjudicated after waiving their right to trial and entering a guilty plea. Fairness and equity in the plea bargaining process are premised on the assumption that youth have the capacity to understand and elect between available options and will be given a meaningful opportunity to choose without coercion and deception. In legal terms, the Constitution will only sanction a plea when the defendant makes a knowing, voluntary, and intelligent waiver of her right to trial. In this chapter, we briefly describe the juvenile court process and explain the circumstances of a plea bargain, which constitutes both a waiver of Constitutional rights and an agreement to certain conditions. Then we evaluate the research and practice knowledge regarding the legal components of a valid waiver-that it must be knowing and voluntary. We consider how information, capacity, and circumstance contribute to a knowing waiver. Then we examine how procedural justice, paternalism, and coercion may affect a voluntary waiver. Throughout, we consider whether the people, policies, and practices meant to assess and safeguard that waiver decision fulfill their intended purpose. © 2016 Elsevier Inc. All rights reserved.

  3. Serum interleukin-17 levels are associated with nephritis in childhood-onset systemic lupus erythematosus.

    Science.gov (United States)

    Peliçari, Karina de Oliveira; Postal, Mariana; Sinicato, Nailú Angelica; Peres, Fernando Augusto; Fernandes, Paula Teixeira; Marini, Roberto; Costallat, Lilian Tereza Lavras; Appenzeller, Simone

    2015-05-01

    To determine the serum interleukin-17 (IL-17) levels in childhood-onset systemic lupus erythematosus patients and to evaluate the association between IL-17 and clinical manifestations, disease activity, laboratory findings and treatment. We included 67 consecutive childhood-onset systemic lupus erythematosus patients [61 women; median age 18 years (range 11-31)], 55 first-degree relatives [50 women; median age 40 years (range 29-52)] and 47 age- and sex-matched healthy controls [42 women; median age 19 years (range 6-30)]. The childhood-onset systemic lupus erythematosus patients were assessed for clinical and laboratory systemic lupus erythematosus manifestations, disease activity [Systemic Lupus Erythematosus Disease Activity Index (SLEDAI)], cumulative damage [Systemic Lupus International Collaborating Clinics/American College of Rheumatology (ACR) Damage Index] and current drug use. Serum IL-17 levels were measured by an enzyme-linked immunosorbent assay using commercial kits. The median serum IL-17 level was 36.3 (range 17.36-105.92) pg/mL in childhood-onset systemic lupus erythematosus patients and 29.47 (15.16-62.17) pg/mL in healthy controls (p=0.009). We observed an association between serum IL-17 levels and active nephritis (p=0.01) and migraines (p=0.03). Serum IL-17 levels were not associated with disease activity (p=0.32), cumulative damage (p=0.34), or medication use (p=0.63). IL-17 is increased in childhood-onset systemic lupus erythematosus and may play a role in the pathogenesis of neuropsychiatric and renal manifestations. Longitudinal studies are necessary to determine the role of IL-17 in childhood-onset systemic lupus erythematosus.

  4. Serum interleukin-17 levels are associated with nephritis in childhood-onset systemic lupus erythematosus

    Directory of Open Access Journals (Sweden)

    Karina de Oliveira Peliçari

    2015-05-01

    Full Text Available OBJECTIVES: To determine the serum interleukin-17 (IL-17 levels in childhood-onset systemic lupus erythematosus patients and to evaluate the association between IL-17 and clinical manifestations, disease activity, laboratory findings and treatment. METHODS: We included 67 consecutive childhood-onset systemic lupus erythematosus patients [61 women; median age 18 years (range 11-31], 55 first-degree relatives [50 women; median age 40 years (range 29-52] and 47 age- and sex-matched healthy controls [42 women; median age 19 years (range 6-30]. The childhood-onset systemic lupus erythematosus patients were assessed for clinical and laboratory systemic lupus erythematosus manifestations, disease activity [Systemic Lupus Erythematosus Disease Activity Index (SLEDAI], cumulative damage [Systemic Lupus International Collaborating Clinics/American College of Rheumatology (ACR Damage Index] and current drug use. Serum IL-17 levels were measured by an enzyme-linked immunosorbent assay using commercial kits. RESULTS: The median serum IL-17 level was 36.3 (range 17.36-105.92 pg/mL in childhood-onset systemic lupus erythematosus patients and 29.47 (15.16-62.17 pg/mL in healthy controls (p=0.009. We observed an association between serum IL-17 levels and active nephritis (p=0.01 and migraines (p=0.03. Serum IL-17 levels were not associated with disease activity (p=0.32, cumulative damage (p=0.34, or medication use (p=0.63. CONCLUSION: IL-17 is increased in childhood-onset systemic lupus erythematosus and may play a role in the pathogenesis of neuropsychiatric and renal manifestations. Longitudinal studies are necessary to determine the role of IL-17 in childhood-onset systemic lupus erythematosus.

  5. Immune system dysregulation in first-onset postpartum psychosis

    NARCIS (Netherlands)

    Bergink, V.; Burgerhout, K.M.; Weigelt, K.; Pop, V.J.M.; de Wit, H.; Drexhage, R.C.; Kushner, S.A.; Drexhage, H.A.

    2013-01-01

    Background Accumulating evidence suggests that dysregulation of the immune system represents an important vulnerability factor for mood disorders. Postpartum psychosis (PP) is a severe mood disorder occurring within 4 weeks after delivery, a period of heightened immune responsiveness and an altered

  6. Expression of immune system-related genes during ontogeny in experimentally wounded common carp (Cyprinus carpio) larvae and juveniles

    DEFF Research Database (Denmark)

    Schmidt, Jacob; Nielsen, Michael Engelbrecht

    2014-01-01

    they were microscopically indistinguishable from normal tissue by day 3 post-wounding in all but the juvenile carp wounded on day 49 post-fertilization. In these juveniles the wounded area was still visible even 7days post-wounding. On the transcriptional level a very limited response was observed......We investigated the effect of full-thickness incisional wounding on expression of genes related to the immune system in larvae and juveniles of common carp (Cyprinus carpio). The wounds were inflicted by needle puncture immediately below the anterior part of the dorsal fin on days 7, 14, 28 and 49...... after fertilization. We followed the local gene expression 1, 3 and 7days after wounding by removing head and viscera before extracting RNA from the remaining part of the fish, including the wound area. In addition, we visually followed wound healing. Overall the wounds had regenerated to a point where...

  7. Nervous system development in lecithotrophic larval and juvenile stages of the annelid Capitella teleta.

    Science.gov (United States)

    Meyer, Néva P; Carrillo-Baltodano, Allan; Moore, Richard E; Seaver, Elaine C

    2015-01-01

    Reconstructing the evolutionary history of nervous systems requires an understanding of their architecture and development across diverse taxa. The spiralians encompass diverse body plans and organ systems, and within the spiralians, annelids exhibit a variety of morphologies, life histories, feeding modes and associated nervous systems, making them an ideal group for studying evolution of nervous systems. We describe nervous system development in the annelid Capitella teleta (Blake JA, Grassle JP, Eckelbarger KJ. Capitella teleta, a new species designation for the opportunistic and experimental Capitella sp. I, with a review of the literature for confirmed records. Zoosymposia. 2009;2:25-53) using whole-mount in situ hybridization for a synaptotagmin 1 homolog, nuclear stains, and cross-reactive antibodies against acetylated α-tubulin, 5-HT and FMRFamide. Capitella teleta is member of the Sedentaria (Struck TH, Paul C, Hill N, Hartmann S, Hosel C, Kube M, et al. Phylogenomic analyses unravel annelid evolution. Nature. 2011;471:95-8) and has an indirectly-developing, lecithotrophic larva. The nervous system of C. teleta shares many features with other annelids, including a brain and a ladder-like ventral nerve cord with five connectives, reiterated commissures, and pairs of peripheral nerves. Development of the nervous system begins with the first neurons differentiating in the brain, and follows a temporal order from central to peripheral and from anterior to posterior. Similar to other annelids, neurons with serotonin-like-immunoreactivity (5HT-LIR) and FMRFamide-like-immunoreactivity (FMRF-LIR) are found throughout the brain and ventral nerve cord. A small number of larval-specific neurons and neurites are present, but are visible only after the central nervous system begins to form. These larval neurons are not visible after metamorphosis while the rest of the nervous system is largely unchanged in juveniles. Most of the nervous system that forms during

  8. Use of single photon emission computed tomography and magnetic resonance to evaluate central nervous system involvement in patients with juvenile systemic lupus erythematosus

    Directory of Open Access Journals (Sweden)

    Prismich G.

    2002-01-01

    Full Text Available The objective of the present study was to identify the single photon emission computed tomography (SPECT and magnetic resonance (MR findings in juvenile systemic lupus erythematosus (JSLE patients with CNS involvement and to try to correlate them with neurological clinical history data and neurological clinical examination. Nineteen patients with JSLE (16 girls and 3 boys, mean age at onset 9.2 years were submitted to neurological examination, electroencephalography, cerebrospinal fluid analysis, SPECT and MR. All the evaluations were made separately within a period of 15 days. SPECT and MR findings were analyzed independently by two radiologists. Electroencephalography and cerebrospinal fluid analysis revealed no relevant alterations. Ten of 19 patients (53% presented neurological abnormalities including present or past neurological clinical history (8/19, 42%, abnormal neurological clinical examination (5/19, 26%, and abnormal SPECT or MR (8/19, 42% and 3/19, 16%, respectively. The most common changes in SPECT were cerebral hypoperfusion and heterogeneous distribution of blood flow. The most common abnormalities in MR were leukomalacia and diffuse alterations of white matter. There was a correlation between SPECT and MR (P<0.05. We conclude that SPECT and MR are complementary and useful exams in the evaluation of neurological involvement of lupus.

  9. NDTAC Practice Guide: Quality Education Services Are Critical for Youth Involved with the Juvenile Justice and Child Welfare Systems

    Science.gov (United States)

    Gonsoulin, Simon; Clark, Heather Griller; Rankin, Victoria E.

    2015-01-01

    This National Evaluation and Technical Assistance Center for the Education of Children and Youth Who are Neglected, Delinquent, or At-Risk (NDTAC) practice guide examines the principle that quality education services are critical for youth involved with the juvenile justice and child welfare systems. This principle asserts that, to address the…

  10. Trends in nitrogen isotope ratios of juvenile winter flounder reflect changing nitrogen inputs to Rhode Island, USA estuarine systems

    Science.gov (United States)

    Nitrogen isotope ratios (d 15N) in juvenile winter flounder, Pseudopleuronectes americanus, were used to examine changes in nitrogen inputs to several Rhode Island, USA estuarine systems. Fish were collected over two three-year periods with a ten-year interval between sampling pe...

  11. Idiopathic Pulmonary Hemosiderosis in a Child with Recurrent Macrophage Activation Syndrome Secondary to Systemic Juvenile Idiopathic Arthritis

    OpenAIRE

    Barut, Kenan; Sahin, Sezgin; Adrovic, Amra; Sen, Velat; Kasapcopur, Ozgur

    2017-01-01

    Macrophage activation syndrome, a severe complication of systemic juvenile idiopathic arthritis and other inflammatory diseases, represents one of the most important rheumatological emergencies. Delayed diagnosis could lead to life-threatening complications. Pulmonary hemosiderosis has been classically characterized by a triad of anemia, hemoptysis, and lung infiltrates on chest radiogram. Although the majority of patients of pulmonary hemosiderosis are considered idiopathic, secondary hemosi...

  12. Research-Based Practices for Reintegrating Students with Emotional and Behavioral Disorders from the Juvenile Justice System

    Science.gov (United States)

    Mathur, Sarup R.; Griller Clark, Heather; LaCroix, Leslie; Short, James

    2018-01-01

    This article discusses the unique and influential role of school teachers in the rehabilitation and reintegration of students with emotional and behavioral disorders who have been involved with the juvenile justice (JJ) system. By adopting evidenced-based practices, highlighted within this article, teachers can prevent further escalation of…

  13. [Clinical features and adverse pregnancy outcomes of new onset systemic lupus erythematosus during pregnancy].

    Science.gov (United States)

    Zhan, Z P; Yang, Y; Zhan, Y F; Chen, D Y; Liang, L Q; Yang, X Y

    2016-11-08

    Objective: To investigate the clinical characteristics and adverse pregnancy outcomes in pregnant women with new onset systemic lupus erythematosus (SLE) during pregnancy. Methods: The clinical data of 263 pregnancies with SLE in the First Affiliated Hospital of Zhongshan University from 2001 to 2015 were collected and analyzed retrospectively. Results: Of all the 263 pregnancies, 188 were diagnosed before pregnancy and 75 were newly diagnosed during pregnancy. Among the 75 new onset SLE, 27, 31, 14 and 3 cases were diagnosed during first trimester, second trimester, third trimester and puerperium, respectively. Active lupus was noted in 81.3% of the patients with new onset SLE. The main clinical manifestations of new onset SLE were lupus nephritis (57.3%) and thrombocytopenia (38.7%). SLEPDAI scores as well as the prevalence of lupus nephritis, and thrombocytopenia in patients with new onset SLE was higher than those in the previously diagnosed ones ( P pregnancies, adverse pregnancy outcomesoccurred in 53 patients, including 34 with pregnancy loss, 15with premature, 8with intrauterine growth restriction, 5with fetal distress and5 with neonatal lupus. Compared with patients withnon-newonset SLE, patients with newonset SLEhad a higher prevalence of adverse pregnancy outcomes (56.4% vs 70.7%, P pregnancy loss (21.8% vs 45.3%, P lupus nephritis and thrombocytopenia. Patients with new onset SLE were more prone to active lupus, lupus nephritis and thrombocytopenia, as well as more adverse pregnancy outcomes and pregnancy loss.

  14. Inadequate dietary intake of children and adolescents with juvenile idiopathic arthritis and systemic lupus erythematosus.

    Science.gov (United States)

    Caetano, Michelle C; Ortiz, Thaís T; Terreri, Maria Teresa S R L A; Sarni, Roseli O S; Silva, Simone G L; Souza, Fabíola I S; Hilário, Maria Odete E

    2009-01-01

    To evaluate the dietary intake of children and adolescents with juvenile idiopathic arthritis (JIA) and juvenile systemic lupus erythematosus (JSLE) using a 24-hour diet recall and relating it to the patients' clinical and anthropometric characteristics and to the drugs used in their treatment. By means of a cross-sectional study, we assessed the 24-hour diet recalls of outpatients. Their nutritional status was classified according to the CDC (2000). The computer program NutWin UNIFESP-EPM was used for food intake calculation. The Recommended Dietary Allowances and the Brazilian food pyramid were used for quantitative and qualitative analysis. Median age was 12 years for JIA patients and 16.5 years for JSLE patients. Among the JIA patients, 37.5% had active disease, and among the JSLE patients, 68.2% showed Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) > 4. Malnutrition was found in 8.3 and 4.5% of the JIA and JSLE patients, respectively, and obesity was present in 16.7 and 18.2%. For JIA patients, the excessive intake of energy, protein, and lipids was 12.5, 75, and 31.3%, respectively. For JSLE patients, the excessive intake of energy, protein, and lipids was 13.6, 86.4, and 36.4%, respectively. Low intake of iron, zinc, and vitamin A was found in 29.2 and 50, 87.5 and 86.4, and 87.5 and 95.2% of the JIA and JSLE patients, respectively. There was not a significant association between intake, disease activity, and nutritional status. Patients with rheumatic diseases have inadequate dietary intake. There is excessive intake of lipids and proteins and low intake of micronutrients.

  15. Understanding Child Maltreatment: Juvenile Delinquency. From Research to Effective Program, Practice, and Systemic Solutions.

    Science.gov (United States)

    Wiig, Janet; Widom, Cathy Spatz

    This monograph details the growing body of research showing the connection between child maltreatment and juvenile delinquency. In 2000, nearly 879,000 children were victims of child abuse and neglect. Although juvenile crime has declined recently, the level of crime committed by youth remains high. This monograph describes an array of program,…

  16. A Marked Response to Immunosuppressive Intervention for Abruptly Occurring Cardiac Complications in a Case of Juvenile Systemic Sclerosis Overlapped with Dermatomyositis

    Directory of Open Access Journals (Sweden)

    Tsunehisa Nagamori

    2017-01-01

    Full Text Available Juvenile-onset systemic sclerosis (jSSc is a rare condition, having unique characteristic features compared to adult-onset SSc. Although cardiac involvement (CI is known as a leading cause of mortality overall in SSc, the importance of CI in jSSc has not been emphasized. Here we present a 13-year-old female with jSSc overlapped with dermatomyositis (DM complicated CI. She developed skin thickness and induration, Raynaud’s phenomenon, digital pitting scars in fingertips, and skeletal myositis. Oral prednisolone and pulse methotrexate treatment led to the improvement of skin findings; however two weeks after the initiation she suddenly presented with muscle pain and dyspnea within a few days. Cardiac investigations then showed pericardiac effusion and diastolic dysfunction due to significant biventricular hypertrophy causing heart failure. As pericardiac effusion and exacerbation of skeletal myositis were evident, steroid pulse therapy was initiated. Unexpectedly, not only the myositis but also the CI including diastolic dysfunction was improved. She thereafter followed a favorable clinical course without reactivation of the CI or cardiac fibrosis. As a conclusion, close attention to CI must be paid in jSSc patients, especially when skeletal muscle involvement is evident and immunosuppressive therapy may be effective for CI in jSSc in cases where it occurs abruptly.

  17. Cytokines in relation to autoantibodies before onset of symptoms for systemic lupus erythematosus.

    Science.gov (United States)

    Eriksson, C; Rantapää-Dahlqvist, S

    2014-06-01

    A number of cytokines and chemokines were analysed and related to autoantibodies in blood samples pre-dating the onset of symptoms of systemic lupus erythematosus. Thirty-five patients with systemic lupus erythematosus (American College of Rheumatology criteria) were identified as having donated blood samples, prior to symptom onset, to the Biobank of northern Sweden. Altogether, 140 age- and sex-matched controls were also identified. The concentrations of interferon-α, interleukin-4, interleukin-9, interleukin-10, interferon inducible protein-10 and monocyte chemotactic protein-1 were analysed using multiplex technology and related to autoantibodies (ANA, ENA, anti-dsDNA and anti-histone antibodies) analysed from the same blood sample. The interferon-γ inducible protein-10 levels were higher in the pre-symptomatic individuals than in controls (p lupus erythematosus. An increased concentration of interferon-γ inducible protein-10 pre-dated the onset of systemic lupus erythematosus and was related to autoantibodies before the onset of disease. The levels of interferon-γ inducible protein-10 and interferon-α were correlated. These findings support the proposal that the interferon system is important early in the pathogenesis of systemic lupus erythematosus and autoantibody formation. © The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav.

  18. Juvenil idiopatisk arthritis

    DEFF Research Database (Denmark)

    Herlin, Troels

    2002-01-01

    The new classification of juvenile idiopathic arthritis (JIA) is described in this review. Clinical characteristics divide JIA in to subtypes: systemic, oligoarticular (persistent and extended type), RF-positive and--negative polyarticular, enthesitis-related arthritis and psoriatic arthritis...

  19. Bypass system modification at Bonneville Dam on the Columbia River improved the survival of juvenile salmon

    Science.gov (United States)

    Ferguson, J.W.; Sandford, B.P.; Reagan, R.E.; Gilbreath, L.G.; Meyer, E.B.; Ledgerwood, R.D.; Adams, N.S.

    2007-01-01

    From 1987 to 1992, we evaluated a fish bypass system at Bonneville Dam Powerhouse 2 on the Columbia River. The survival of subyearling Chinook salmon Oncorhynchus tshawytscha released into the system ranged from 0.774 to 0.911 and was significantly lower than the survival of test fish released into turbines and the area immediately below the powerhouse where bypass system flow reentered the river. Yearling and subyearling Chinook salmon and yearling coho salmon O. kisutch released into the bypass system were injured or descaled. Also, levels of blood plasma cortisol and lactate were significantly higher in yearling and subyearling Chinook salmon that passed through the bypass system than in fish released directly into a net located over the bypass exit. This original system was then extensively modified using updated design criteria, and the site where juvenile fish reentered the river was relocated 2.8 km further downstream to reduce predation on bypassed fish by northern pikeminnow Ptychocheilus oregonensis. Based on studies conducted from 1999 to 2001, the new bypass system resulted in high fish survival, virtually no injuries to fish, fish passage times that were generally similar to water travel times, and mild stress responses from which fish recovered quickly. The mean estimated survival of subyearling Chinook salmon passing through the new bypass system was 0.946 in 2001, which was an usually low-flow year. Survival, physical condition, passage timing, and blood physiological indicators of stress were all useful metrics for assessing the performance of both bypass systems and are discussed. The engineering and hydraulic criteria used to design the new bypass system that resulted in improved fish passage conditions are described.

  20. Disturbance of the muscoloskeletal system in juvenile ankylosing spondylitis and disease developed in the adulthood (involvement of spine and sacroiliac joints

    Directory of Open Access Journals (Sweden)

    Yehudina Ye.D.

    2018-03-01

    Full Text Available Background. Two forms of ankylosing spondylitis (AS are distinguished: juvenile and adult, depending on debut age of the disease. The diagnosis of juvenile AS (JAS is one of the most urgent problems in a pediatric rheumatology. The peculiarities of AS course that onsets in childhood and adulthood are manifested by differences in the nature of a spinal column disturbance. At the same time, the evolution of JAS in adulthood remains unexplored. The goals and objectives of research: to study clinical and X-ray symptoms of spondylopathy and sacroiliitis course, to assess their characteristics in the disease that onset in childhood and adulthood. Material and methods. 217 patients with AS (193 men and 24 women with an average age of 38 years were examined. The fast-progressing course of the disease was detected in 21% of cases, moderate and high degree of activity – in 79% of cases, the ІІ-ІІІ stage in 82%, and polyarthritis – in 65%. JAS was detected in 16% of cases (all boys, among them the third stage occurred twice more likely than among the other patients. Results. The clinical and radiologic signs of spondylopathy and sacroiliitis are observed in 95% and 97% of the total number of AS cases, respectively, among all patients with JAS lumbago was detected 4,3 times more frequently, sciatic muscles hypotrophy – 7,8 times, "the string symptom" - 2,9 times", the calcification of the spinal cord - 2,3 times, whereas the prevalence of spinal column injury, the severity of cervico-spondylopathy and sacroiliitis among patients with the disease debut in the adulthood is significantly greater, and the involvement in the process of the lumbar and thoracic spine are detected correspondingly twice as often and by 19%, occurrence of dorsalgia is 4 times as often, the limitation of body lateral bendover by 59%, while there are ambiguous dispersion-correlation links with extraarticular (systemic manifestations of the disease, and the high prevalence of

  1. Association between periphyton and bioflocs systems in intensive culture of juvenile Nile tilapia

    Directory of Open Access Journals (Sweden)

    Davi de Holanda Cavalcante

    2016-05-01

    Full Text Available The present work aimed at determining the effects of the association between the periphyton-based system with the bioflocs-based system in the intensive culture of juvenile Nile tilapia (1.56 ± 0.07 g; 72 fish m-3, on variables of water quality, growth performance and effluent quality after 10 weeks. The experiment was arranged in a 2 x 2 factorial randomized block design with four treatments and five repetitions each. The factors tested were the following: ‘underwater structure’ (absence and presence and ‘adjustment of the C: N ratio of water’ (no and yes. The final fish body weight, specific growth rate and yield were higher (p < 0.05 in the C: N-adjusted tanks. The presence of submerged structures in the tanks had no significant influence on those same variables. It was concluded that the periphyton-based system is not indicated for intensive farming of Nile tilapia, in which there is a high allowance of artificial feed to fish.

  2. Late-onset systemic lupus erythematosus in Latin Americans: a distinct subgroup?

    Science.gov (United States)

    Catoggio, L J; Soriano, E R; Imamura, P M; Wojdyla, D; Jacobelli, S; Massardo, L; Chacón Díaz, R; Guibert-Toledano, M; Alvarellos, A; Saurit, V; Manni, J A; Pascual-Ramos, V; Silva de Sauza, A W; Bonfa, E; Tavares Brenol, J C; Ramirez, L A; Barile-Fabris, L A; De La Torre, I Garcia; Alarcón, G S; Pons-Estel, B A

    2015-07-01

    To examine the characteristics of patients who developed late onset systemic lupus erythematosus (SLE) in the GLADEL (Grupo Latino Americano de Estudio del Lupus) cohort of patients with SLE. Patients with SLE of less than two years of disease duration, seen at 34 centers of nine Latin American countries, were included. Late-onset was defined as >50 years of age at time of first SLE-related symptom. Clinical and laboratory manifestations, activity index (SLEDAI), and damage index (SLICC/ACR- DI) were ascertained at time of entry and during the course (cumulative incidence). Features were compared between the two patient groups (lupus, adjusting for other variables. Of the 1480 patients included, 102 patients (6.9 %) had late-onset SLE, 87% of which were female. Patients with late-onset SLE had a shorter follow-up (3.6 vs. 4.4 years, p  0.05). In multivariable analysis, late onset was independently associated with higher odds of ocular (OR = 3.66, 95% CI = 2.15-6.23), pulmonary (OR = 2.04, 95% CI = 1.01-4.11), and cardiovascular (OR = 1.76, 95% CI = 1.04-2.98) involvement and lower odds of cutaneous involvement (OR = 0.41, 95% CI = 0.21-0.80), number of cumulative SLE criteria (OR = 0.79, 95% CI = 0.64-0.97), use of cyclophosphamide (OR = 0.47, 95% CI = 0.24-0.95), and anti-RNP antibodies (OR = 0.43, 95% CI = 0.20-0.91). A Cox regression model revealed a higher risk of dying in older onset than the younger-onset SLE (OR = 2.61, 95% CI = 1.2-5.6). Late-onset SLE in Latin Americans had a distinct disease expression compared to the younger-onset group. The disease seems to be mild with lower cumulative SLE criteria, reduced renal/mucocutaneous involvements, and less use of cyclophosphamide. Nevertheless, these patients have a higher risk of death and of ocular, pulmonary, and cardiovascular involvements. © The Author(s) 2014.

  3. Comparison of estimates of body fat content in childhood-onset systemic lupus erythematosus.

    Science.gov (United States)

    Sinicato, N A; Peres, F A; de Oliveira Peliçari, K; de Oliveira Santos, A; Ramos, C D; Marini, R; Appenzeller, S

    2017-04-01

    Objective We aimed to compare estimates of body fat content with respect to their ability to predict the percentage of body fat, confirmed by dual-energy X-ray absorptiometry scans in childhood-onset systemic lupus erythematosus. Methods We included 64 consecutive childhood-onset systemic lupus erythematosus patients and 64 healthy age and sex-matched controls in a cross-sectional study. Anthropometric data, body mass index and body adiposity index were calculated for all subjects. Childhood-onset systemic lupus erythematosus patients were further assessed for clinical and laboratory childhood-onset systemic lupus erythematosus manifestations and fat mass, lean mass and percentage of body fat evaluated by dual-energy X-ray absorptiometry. Results Elevated waist/hip ratio was observed in childhood-onset systemic lupus erythematosus patients when compared to controls ( p lupus erythematosus patients and controls. Using dual-energy X-ray absorptiometry as gold standard we observed that all indirect estimates of body fat were correlated with whole body fat mass. We observed a correlation between height and cumulative corticosteroid dose adjusted by weight ( r = 0.429, p = 0.005) in childhood-onset systemic lupus erythematosus. On whole body analysis we observed a correlation between lean mass and ACR Damage Index scores ( r = -0.395; p = 0.019); percentage of body fat and adjusted Systemic Lupus Erythematosus Disease Activity Index ( r = 0.402; p = 0.008), disease duration ( r = -0.370; p = 0.012). On trunk analysis we observed a correlation between lean mass and ACR Damage Index ( r = -0.319; p = 0.042); percentage of body fat with adjusted Systemic Lupus Erythematosus Disease Activity Index ( r = 0.402; p = 0.005), disease duration ( r = -0.408; p = 0.005). Conclusions This is the first study analyzing body adiposity index in childhood-onset systemic lupus erythematosus patients. We observed that all indirect

  4. An unusual presentation of juvenile lupus nephritis

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    Malleshwar Bottu

    2016-01-01

    Full Text Available The incidence of juvenile lupus varies widely ranging between 4 and 250 per 100,000 population. Most common organ involvement in juvenile lupus is kidney. Neurological, cutaneous and hematological involvements are also involved. Skeletal muscle involvement in the form of myositis is rare. Myositis as presenting manifestation in juvenile lupus is also unusual. Herein, we report one such case wherein myositis preceded the onset of lupus nephritis

  5. Elevated levels of peripheral blood CD14(bright) CD16+ and CD14(dim) CD16+ monocytes may contribute to the development of retinopathy in patients with juvenile onset type 1 diabetes.

    Science.gov (United States)

    Ryba-Stanisławowska, Monika; Myśliwska, Jolanta; Juhas, Ulana; Myśliwiec, Małgorzata

    2015-09-01

    The study aimed to analyze the CD14(bright) CD16(+) and CD14(dim) CD16(+) monocyte subsets in juvenile-onset complication-free diabetes mellitus type 1 in the context of their association with microvascular complications. 61 children with type 1 diabetes and 30 healthy individuals were enrolled in a study. CD14(bright) CD16(+) and CD14(dim) CD16(+) monocytes were quantified in peripheral blood by means of flow cytometry. At the time of sampling blood glucose concentration was taken along with biochemical measurement of renal function, CRP and glycosylated hemoglobin. The Spearman's correlations were used to compare the relationship between CD16(+) monocyte subsets and the clinical parameters that can predict the development of microangiopathies. The flow cytometric analysis of monocyte subsets in peripheral blood of analyzed subjects revealed that the numbers of CD14(bright) CD16(+) and CD14(dim) CD16(+) monocytes were significantly higher in patients with type 1 diabetes than in the healthy individuals. As to the relationship between CD16(+) monocyte subsets and the clinical parameters that can predict development of microangiopathies, it was shown that both CD16(+) subsets were associated with increased risk of retinopathy development, defined as retinopathy development value. Elevated levels of intermediate CD14(bright) CD16(+) and non-classical CD14(dim) CD16(+) monocytes predict development of diabetic retinopathy in patients with type 1 diabetes. © 2015 APMIS. Published by John Wiley & Sons Ltd.

  6. A critical appraisal of radiographic scoring systems for assessment of juvenile idiopathic arthritis

    International Nuclear Information System (INIS)

    Doria, Andrea S.; Babyn, Paul S.; Feldman, Brian

    2006-01-01

    Assessing structural damage to joints over time is essential for evaluating the effectiveness of therapeutic interventions for patients with inflammatory arthritis. Although radiography is able to quantify joint damage, the changes found with conventional radiography early in the disease course are nonspecific, and late radiographic changes are often irreversible. Although many clinical trials on drug development for children still use radiographic scales as endpoints for the study, more specific therapies have been developed for juvenile idiopathic arthritis (JIA) that would enable imaging to ''fine-tune'' patients to placement into specific treatment algorithms. As a result, new imaging scales to identify early abnormalities are clearly needed. Many pediatric rheumatology centers around the world persistently apply adult-designed radiographic scoring systems to evaluate the progression of JIA. Few pediatric-targeted radiographic scales are available for assessment of progression of JIA in growing joints, and the clinimetric and psychometric properties of such scales have been poorly investigated. We present a critique to the evaluative, discriminative, and predictive roles of the van der Heijde modification of Sharp's radiographic method, a scale originally designed to assess damage to joints of adults with rheumatoid arthritis, when it is applied to a pediatric population. We discuss the advantages and drawbacks of this radiographic scoring system for assessing growing joints and the ability of MRI to overcome inadequacies of conventional radiography. (orig.)

  7. The association between initial manifestations of childhood-onset systemic lupus erythematosus and the survival.

    Science.gov (United States)

    Fatemi, Alimohammad; Matinfar, Mohammad; Saber, Mina; Smiley, Abbas

    2016-10-01

    Childhood-onset systemic lupus erythematosus (cSLE) comprises 15-20% of patients with SLE. Although several studies have reported the outcomes of adult-onset SLE, few investigations have been conducted on cSLE in the Middle East. In a retrospective study, all children with SLE admitted to our tertiary referral center between 1992 and 2011 were recruited. The clinical and laboratory data at the time of onset were recorded and analyzed. Kaplan-Meier analysis was used to calculate the survival rates. Cox regression analysis was applied to assess the predictors of mortality. One hundred and eighty-eight children diagnosed with SLE were enrolled during the study period. Nine patients were censored due to loss to follow-up (6) and incomplete data (3 cases). Mean age of patients at the time of onset was 14.4 (3.05) years. Only 22 (11.8%) children were younger than 10 years at the time of disease onset. In total, 20 patients (11%) died, all after the first decade of life. The most common cause of death was lupus nephritis (10 patients, 50% of deaths) followed by infections (35%), cerebrovascular accidents (10%) and alveolar hemorrhage (5%). Cumulative survival rate after 5, 10, 15 and 20 years was 91, 87, 85, and 79%, respectively. Having hematuria or pleurisy at the time of SLE onset had a negative effect on survival in multivariate analysis. cSLE survival in Iran was comparable to that in other developing countries. Baseline presentation with hematuria predominantly increased the mortality rate in cSLE. Prospective and larger studies in future may unfold other aspects of cSLE. © 2015 Asia Pacific League of Associations for Rheumatology and Wiley Publishing Asia Pty Ltd.

  8. Juvenile Arthritis

    Science.gov (United States)

    Juvenile arthritis (JA) is arthritis that happens in children. It causes joint swelling, pain, stiffness, and loss of motion. It can affect any joint, but ... of JA that children get is juvenile idiopathic arthritis. There are several other forms of arthritis affecting ...

  9. Juvenile Justice in Mexico

    Directory of Open Access Journals (Sweden)

    Martha Frías Armenta

    2014-08-01

    Full Text Available The first tribunal in Mexico was established in the central state of San Luis Potosi in 1926. The Law Regarding Social Prevention and Juvenile Delinquency for the Federal District and Mexican territories was promulgated in 1928. In 2005, Article 18 of the Mexican Constitution was modified to establish a comprehensive system (“Sistema Integral de justicia” in Spanish of justice for juveniles between 12 and 18 years old who had committed a crime punishable under criminal law. Its objective was to guarantee juveniles all the due process rights established for adults, in addition to the special ones recognized for minors. The constitutional reform also provides a framework that includes special tribunals as well as alternative justice options for juveniles. With these reforms, institutionalization of minors was to be considered an extreme measure applicable only to felonies and to juveniles older than 14. In 2006, all states within the Mexican federation enacted the “Law of justice for adolescents”. This system, at both the federal and state levels, formalizes a new global paradigm with regard to the triangular relationship between children, the State and the Law. It recognizes that children are also bearers of the inherent human rights recognized for all individuals, instead of simply objects in need of protection. However, despite formally aligning Mexican juvenile justice law with the Convention on the Rights of the Child (CRC, issues of actual substantive rights remained and new ones have appeared. For example, juveniles younger than 14 who have not committed a felony are released from institutions without any rehabilitation or treatment options, and alternative forms of justice were included without evaluating their possibilities of application or their conditions for success. In addition, the economic status of most juvenile detainees continues to be one of the most important determining factors in the administration of justice

  10. Steps towards improving the system of records, collection and monitoring of data relevant to statistics within the system of juvenile justice in the Republic of Serbia

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    Stevanović Ivana

    2012-01-01

    Full Text Available The paper emphasises the importance of „good statistics of juvenile justice“ as one of the basis for a clearer overview of the juvenile crime situation, in order to create unique policies at local and national levels for the suppression and prevention of this phenomenon, and to create appropriate areas for action in terms of improving the system reform. The author particularly gives a review of the „Global Indicators in Juvenile Justice“ which present the basic set of data and comparative tool that provides a basis for the assessment and evaluation of services and policies in the field of juvenile justice, and highlights the importance of compatibility of „national indicators“ with them. Particular attention in the paper has been devoted to the overview and analysis of the necessary measures to improve this field, that were prepared and delivered to the relevant ministries and institutions by the Council for Monitoring and Promoting the work of Bodies Engaged in Criminal Proceeding and Enforcement of Juvenile Criminal Sanctions Involving Juveniles - Juvenile Justice Council (hereinafter: the Council. It was pointed, first of all, at the suggestions made by the Council to the Ministry of Justice with the aim to improve the Program for automated record keeping, at the necessary changes of the Court rules, and certain amendments to Forms SK- 3 and SK- 4 of the Statistical Office of the Republic of Serbia were presented. [Projekat Ministarstva nauke Republike Srbije, br. 47011: Kriminal u Srbiji - fenomenologija, rizici i mogućnosti socijalne intervencije

  11. Algorithm development for corticosteroid management in systemic juvenile idiopathic arthritis trial using consensus methodology

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    Ilowite Norman T

    2012-08-01

    Full Text Available Abstract Background The management of background corticosteroid therapy in rheumatology clinical trials poses a major challenge. We describe the consensus methodology used to design an algorithm to standardize changes in corticosteroid dosing during the Randomized Placebo Phase Study of Rilonacept in Systemic Juvenile Idiopathic Arthritis Trial (RAPPORT. Methods The 20 RAPPORT site principal investigators (PIs and 4 topic specialists constituted an expert panel that participated in the consensus process. The panel used a modified Delphi Method consisting of an on-line questionnaire, followed by a one day face-to-face consensus conference. Consensus was defined as ≥ 75% agreement. For items deemed essential but when consensus on critical values was not achieved, simple majority vote drove the final decision. Results The panel identified criteria for initiating or increasing corticosteroids. These included the presence or development of anemia, myocarditis, pericarditis, pleuritis, peritonitis, and either complete or incomplete macrophage activation syndrome (MAS. The panel also identified criteria for tapering corticosteroids which included absence of fever for ≥ 3 days in the previous week, absence of poor physical functioning, and seven laboratory criteria. A tapering schedule was also defined. Conclusion The expert panel established consensus regarding corticosteroid management and an algorithm for steroid dosing that was well accepted and used by RAPPORT investigators. Developed specifically for the RAPPORT trial, further study of the algorithm is needed before recommendation for more general clinical use.

  12. Common Variable Immunodeficiency Associated with Hepatosplenic T-Cell Lymphoma Mimicking Juvenile Systemic Lupus Erythematosus

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    A. A. Jesus

    2011-01-01

    Full Text Available Common variable immunodeficiency (CVID is a heterogeneous disorder with susceptibility to infections, autoimmune manifestations, and cancer. To our knowledge, CIVD with T-cell lymphoma mimicking juvenile systemic lupus erythematosus (JSLE was not described in the literature, and one case was reported herein. An 8-year-old female was admitted in our Pediatric Immunology Unit with a clinical history of hypogammaglobulinemia, recurrent upper respiratory infections, and pneumonias. She had a marked decrease of three serum immunoglobulin isotypes, and the diagnosis of CVID was established. At the age of 17 years, she presented with oral ulceration, nonerosive arthritis, nephritis, serositis, cytopenia, positive antiphospholipid antibodies, and positive antinuclear antibody fulfilling the American College of Rheumatology (ACR criteria for SLE. She was treated with intravenous methylprednisolone for three consecutive days, and intravenous immunoglobulin, and maintenance therapy of chloroquine, azathioprine and prednisone 40 mg/day. Two months later, she died of septic shock secondary to acute pneumonia. The necropsy showed hepatosplenic T-cell lymphoma with diffuse involvement of bone marrow, spleen, liver, and lungs. The lymphoma cells were positive for CD3 immunostaining and negative for CD20 and lysozyme. In conclusion, the association of CVID and hepatosplenic T-cell lymphoma may simulate JSLE diagnosis.

  13. LAW ENFORCEMENT PREPAREDNESS FOR THE IMPLEMENTATION OF INDONESIA’S LAW ON JUVENILE JUSTICE SYSTEM

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    Dani Krisnawati

    2014-03-01

    Full Text Available Paradigmatic changes stipulated in Law Number 11 of 2012 on Juvenile Justice System, including regulations concerning restorative justice and diversion require the competency and skills of the law enforcement officers. This research identifies measures that have been taken and the existing barriers in preparing for the implementation of this Law. The research demonstrates that the readiness of the investigators and child prosecutors are merely limited to the outreach of Law Number 11 of 2012, whilst knowledge of the court judges only covers the draft Law. The number of officers receiving outreach is limited and should be increased. Negative perception on the officers due to the risk of a bribery accusation is feared to hamper the implementation of diversion regulation based on a restorative justice. Perubahan paradigmatik yang termuat dalam Undang-Undang Nomor 11 Tahun 2012 tentang Sistem Peradilan Pidana Anak, termasuk diantaranya ketentuan mengenai keadilan restoratif dan diversi, memerlukan kompetensi dan keahlian aparat penegak hukum. Penelitian ini mengidentifikasi langkah yang telah dilakukan dan kendala persiapan implementasi Undang-Undang tersebut. Hasil penelitian memperlihatkan bahwa kesiapan penyidik dan penuntut umum anak masih terbatas pada partisipasi sosialisasi Undang-Undang Nomor 11 Tahun 2012, sedangkan hakim anak memiliki pengetahuan hanya pada Rancangan Undang-Undang. Jumlah aparat yang menerima sosialisasi Undang-Undangmasih terbatas dan perlu ditingkatkan jumlahnya. Pandangan negatif terhadap aparat karena bisa menimbulkan dugaan suap dikhawatirkan menjadi penghambat diterapkannya ketentuan diversi dengan pendekatan keadilan restoratif.

  14. Common variable immunodeficiency associated with hepatosplenic T-cell lymphoma mimicking juvenile systemic lupus erythematosus.

    Science.gov (United States)

    Jesus, A A; Jacob, C M A; Silva, C A; Dorna, M; Pastorino, A C; Carneiro-Sampaio, M

    2011-01-01

    Common variable immunodeficiency (CVID) is a heterogeneous disorder with susceptibility to infections, autoimmune manifestations, and cancer. To our knowledge, CIVD with T-cell lymphoma mimicking juvenile systemic lupus erythematosus (JSLE) was not described in the literature, and one case was reported herein. An 8-year-old female was admitted in our Pediatric Immunology Unit with a clinical history of hypogammaglobulinemia, recurrent upper respiratory infections, and pneumonias. She had a marked decrease of three serum immunoglobulin isotypes, and the diagnosis of CVID was established. At the age of 17 years, she presented with oral ulceration, nonerosive arthritis, nephritis, serositis, cytopenia, positive antiphospholipid antibodies, and positive antinuclear antibody fulfilling the American College of Rheumatology (ACR) criteria for SLE. She was treated with intravenous methylprednisolone for three consecutive days, and intravenous immunoglobulin, and maintenance therapy of chloroquine, azathioprine and prednisone 40  mg/day. Two months later, she died of septic shock secondary to acute pneumonia. The necropsy showed hepatosplenic T-cell lymphoma with diffuse involvement of bone marrow, spleen, liver, and lungs. The lymphoma cells were positive for CD3 immunostaining and negative for CD20 and lysozyme. In conclusion, the association of CVID and hepatosplenic T-cell lymphoma may simulate JSLE diagnosis.

  15. Columbia River System Operation Review : Final Environmental Impact Statement, Appendix C: Anadromous Fish and Juvenile Fish Transportation.

    Energy Technology Data Exchange (ETDEWEB)

    Columbia River System Operation Review (U.S.)

    1995-11-01

    This Appendix C of the Final Environmental Impact Statement for the Columbia River System discusses impacts on andromous fish and juvenile fish transportation. The principal andromous fish in the Columbia basin include salmonid species (Chinook, coho, and sockeye salmon, and steelhead) and nonsalmoinid andromous species (sturgeon, lamprey, and shad). Major sections in this document include the following: background, scope and process; affected environment for salmon and steelhead, shaded, lamprey, sturgeon; study methods; description of alternatives: qualitative and quantitative findings.

  16. Age of onset influences on clinical and laboratory profile of patients with systemic lupus erythematosus.

    Science.gov (United States)

    Sassi, Rafael Hennemann; Hendler, Jordana Vaz; Piccoli, Giovana Fagundes; Gasparin, Andrese Aline; da Silva Chakr, Rafael Mendonça; Brenol, João Carlos Tavares; Monticielo, Odirlei André

    2017-01-01

    The present study aims to evaluate differences in clinical and laboratory manifestations and medication use in the different ages of disease onset in patients with systemic lupus erythematosus (SLE). This cross-sectional study consisted of 598 SLE patients (550 female and 48 male), who attended the Rheumatology Clinic of the Hospital de Clínicas de Porto Alegre between 2003 and 2015. Demographic, clinical and laboratory data were collected. The patients were classified into three groups according to their ages at disease diagnosis. Mean age of diagnosis was 33.6 ± 14.3 years, and the median (25th-75th percentile) disease duration was 13 (7-20) years. Among the patients studied, 419 (70%) were adult-onset (aSLE), 90 (14.8%) were late-onset (lSLE) and 89 (14.8%) were childhood-onset (cSLE). The female to male ratio was higher in aSLE (18:1) compared to the other groups (p = 0.001). Arthritis was predominantly found in aSLE (78.5%) when compared with lSLE (57.7%) (p Nephritis was more common in cSLE (60.6%) than in lSLE (26.6%) (p nephritis and needed a more aggressive treatment with immunosuppressive drugs.

  17. A Cabled Acoustic Telemetry System for Detecting and Tracking Juvenile Salmon: Part 1. Engineering Design and Instrumentation

    Science.gov (United States)

    Weiland, Mark A.; Deng, Z. Daniel; Seim, Tom A.; LaMarche, Brian L.; Choi, Eric Y.; Fu, Tao; Carlson, Thomas J.; Thronas, Aaron I.; Eppard, M. Brad

    2011-01-01

    In 2001 the U.S. Army Corps of Engineers, Portland District (OR, USA), started developing the Juvenile Salmon Acoustic Telemetry System, a nonproprietary sensing technology, to meet the needs for monitoring the survival of juvenile salmonids through eight large hydroelectric facilities within the Federal Columbia River Power System (FCRPS). Initial development focused on coded acoustic microtransmitters and autonomous receivers that could be deployed in open reaches of the river for detection of the juvenile salmonids implanted with microtransmitters as they passed the autonomous receiver arrays. In 2006, the Pacific Northwest National Laboratory began the development of an acoustic receiver system for deployment at hydropower facilities (cabled receiver) for detecting fish tagged with microtransmitters as well as tracking them in two or three dimensions for determining route of passage and behavior as the fish passed at the facility. The additional information on route of passage, combined with survival estimates, is used by the dam operators and managers to make structural and operational changes at the hydropower facilities to improve survival of fish as they pass the facilities through the FCRPS. PMID:22163918

  18. Púrpura trombocitopênica trombótica na apresentação de pacientes com lúpus eritematoso sistêmico juvenil Thrombotic thrombocytopenic purpura at presentation of juvenile systemic lupus erythematosus patients

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    Lucia M. A. Campos

    2013-02-01

    Full Text Available Púrpura trombocitopênica trombótica (PTT é uma alteração hematológica rara e com risco de morte, caracterizada por trombocitopenia, anemia hemolítica microangiopática e alterações neurológicas e/ou renais. A PTT foi descrita em raros pacientes com lúpus eritematoso sistêmico juvenil (LESJ e, até onde se sabe, a prevalência dessa manifestação em uma população de lúpus pediátrico ainda não foi estudada. Assim, entre janeiro de 1983 e dezembro de 2010, revisamos os prontuários de 5.508 pacientes acompanhados na Unidade de Reumatologia Pediátrica do nosso hospital universitário. Foram identificados 279 (5,1% casos de LESJ que preencheram os critérios de classificação do American College of Rheumatology. Dois destes (0,7% apresentavam PTT, ambos no início do LESJ, e foram aqui descritos. Os dois pacientes tinham febre, anemia hemolítica microangiopática (com esquizócitos no sangue periférico e trombocitopenia. O paciente do gênero masculino apresentava hemiparesia e proteinúria, e a paciente do gênero feminino tinha cefaleia persistente e hematúria. Ambos foram tratados com metilprednisolona endovenosa e plasmaferese quando do diagnóstico de PPT. Após tratamento, não houve recidiva da PTT, e hematócritos, contagens de plaquetas e níveis de desidrogenase lática permaneceram normais. Em conclusão, a PTT é uma rara e grave manifestação no início do LESJ. Os casos relatados reforçam a importância de um diagnóstico precoce e de uma terapia agressiva em pacientes com PTT, devido à sua alta morbidade.Thrombotic thrombocytopenic purpura (TTP is a rare and life-threatening hematological abnormality characterized by thrombocytopenia and microangiopathic hemolytic anemia, with neurological abnormalities and/or renal disease. TTP has been rarely reported in juvenile systemic lupus erythematosus (JSLE patients and, to our knowledge, its prevalence in a paediatric lupus population has not been studied. Therefore

  19. Migration depth and residence time of juvenile salmonids in the forebays of hydropower dams prior to passage through turbines or juvenile bypass systems: implications for turbine-passage survival.

    Science.gov (United States)

    Li, Xinya; Deng, Zhiqun D; Brown, Richard S; Fu, Tao; Martinez, Jayson J; McMichael, Geoffrey A; Skalski, John R; Townsend, Richard L; Trumbo, Bradly A; Ahmann, Martin L; Renholds, Jon F

    2015-01-01

    Little is known about the three-dimensional depth distributions in rivers of individually marked fish that are in close proximity to hydropower facilities. Knowledge of the depth distributions of fish approaching dams can be used to understand how vulnerable fish are to injuries such as barotrauma as they pass through dams. To predict the possibility of barotrauma injury caused by pressure changes during turbine passage, it is necessary to understand fish behaviour relative to acclimation depth in dam forebays as they approach turbines. A guiding study was conducted using high-resolution three-dimensional tracking results of salmonids implanted with Juvenile Salmon Acoustic Telemetry System transmitters to investigate the depth distributions of subyearling and yearling Chinook salmon (Oncorhynchus tshawytscha) and juvenile steelhead (Oncorhynchus mykiss) passing two dams on the Snake River in Washington State. Multiple approaches were evaluated to describe the depth at which fish were acclimated, and statistical analyses were performed on large data sets extracted from ∼28 000 individually tagged fish during 2012 and 2013. Our study identified patterns of depth distributions of juvenile salmonids in forebays prior to passage through turbines or juvenile bypass systems. This research indicates that the median depth at which juvenile salmonids approached turbines ranged from 2.8 to 12.2 m, with the depths varying by species/life history, year, location (which dam) and diel period (between day and night). One of the most enlightening findings was the difference in dam passage associated with the diel period. The amount of time that turbine-passed fish spent in the immediate forebay prior to entering the powerhouse was much lower during the night than during the day. This research will allow scientists to understand turbine-passage survival better and enable them to assess more accurately the effects of dam passage on juvenile salmon survival.

  20. Arbitrary arbitration: diverting juveniles into the justice system--a reexamination after 22 years.

    Science.gov (United States)

    Béchard, Stephanie; Ireland, Connie; Berg, Bruce; Vogel, Brenda

    2011-06-01

    This article reports on a study of a juvenile diversion program based in California. It replicates a similar 1986 study of a Florida-based juvenile diversion program. Both studies examine the appropriateness of the actual juvenile population serviced in comparison with the declared and described programs' target populations. Findings in the current research support earlier findings that the diversion program services self-serving, low-risk populations, rather than their intended "first-time nonviolent," more seriously at risk of continued delinquent behavior, target population. This finding is examined in terms of its relationship to issues of labeling and net widening, and suggests how the current study diversion program manages to create a façade that more seriously at-risk youth are being properly targeted by the program.

  1. Detection of nitrogen deficiency QTL in juvenile wild barley introgression lines growing in a hydroponic system

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    Hoffmann Astrid

    2012-10-01

    Full Text Available Abstract Background In this report we studied the genetic regulation of juvenile development of wild barley introgression lines (S42ILs under two contrasting hydroponic nitrogen (N supplies. Ten shoot and root related traits were examined among 42 S42ILs and the recurrent parent ‘Scarlett’. The traits included tiller number, leaf number, plant height, leaf and root length, leaf to root length ratio, shoots and root dry weight, shoot to root weight ratio, and chlorophyll content. Our aims were (1 to test the suitability of a hydroponic system for early detection of favourable S42ILs, (2 to locate quantitative trait loci (QTL that control the examined traits, (3 to identify favourable wild barley alleles that improve trait performances in regard to N treatment and, finally, (4 to validate the identified QTL through comparison with previously reported QTL originating from the same parental cross. Results The phenotypic data were analysed in a mixed model association study to detect QTL. The post-hoc Dunnett test identified 28 S42ILs that revealed significant (P Hsp effects for tiller number, leaf number, leaf length, plant height and leaf to root ratio on the long arm of chromosome 7H. These QTL correspond to QTL for ears per plant and plant height that were previously detected in field trials conducted with the same S42ILs or with the S42 population. Conclusion Our results suggest that the QTL we identified under hydroponic N cultivation partly correspond to QTL detected in field experiments. Due to this finding, screening of plants in early developmental stages grown in a hydroponic system may be a fast and cost effective method for early QTL detection and marker-assisted allelic selection, potentially speeding up elite barley breeding programs.

  2. Detection of nitrogen deficiency QTL in juvenile wild barley introgression lines growing in a hydroponic system.

    Science.gov (United States)

    Hoffmann, Astrid; Maurer, Andreas; Pillen, Klaus

    2012-10-20

    In this report we studied the genetic regulation of juvenile development of wild barley introgression lines (S42ILs) under two contrasting hydroponic nitrogen (N) supplies. Ten shoot and root related traits were examined among 42 S42ILs and the recurrent parent 'Scarlett'. The traits included tiller number, leaf number, plant height, leaf and root length, leaf to root length ratio, shoots and root dry weight, shoot to root weight ratio, and chlorophyll content. Our aims were (1) to test the suitability of a hydroponic system for early detection of favourable S42ILs, (2) to locate quantitative trait loci (QTL) that control the examined traits, (3) to identify favourable wild barley alleles that improve trait performances in regard to N treatment and, finally, (4) to validate the identified QTL through comparison with previously reported QTL originating from the same parental cross. The phenotypic data were analysed in a mixed model association study to detect QTL. The post-hoc Dunnett test identified 28 S42ILs that revealed significant (P hydroponic N study corresponded to QTL that were also detected in field trials with adult plants of a similar S42IL set or of the original S42 population. For instance, S42IL-135, -136 and -137, revealed increasing Hsp effects for tiller number, leaf number, leaf length, plant height and leaf to root ratio on the long arm of chromosome 7H. These QTL correspond to QTL for ears per plant and plant height that were previously detected in field trials conducted with the same S42ILs or with the S42 population. Our results suggest that the QTL we identified under hydroponic N cultivation partly correspond to QTL detected in field experiments. Due to this finding, screening of plants in early developmental stages grown in a hydroponic system may be a fast and cost effective method for early QTL detection and marker-assisted allelic selection, potentially speeding up elite barley breeding programs.

  3. Increased IgE serum levels are unrelated to allergic and parasitic diseases in patients with juvenile systemic lupus erythematosus

    Directory of Open Access Journals (Sweden)

    Bernadete L. Liphaus

    2012-11-01

    Full Text Available OBJECTIVE: The aim of this study was to assess the IgE serum levels in juvenile systemic lupus erythematosus patients and to evaluate possible associations with clinical and laboratory features, disease activity and tissue damage. METHODS: The IgE serum concentrations in 69 consecutive juvenile systemic lupus erythematosus patients were determined by nephelometry. IgG, IgM and IgA concentrations were measured by immunoturbidimetry. All patients were negative for intestinal parasites. Statistical analysis methods included the Mann-Whitney, chi-square and Fisher's exact tests, as well as the Spearman rank correlation coefficient. RESULTS: Increased IgE concentrations above 100 IU/mL were observed in 31/69 (45% juvenile systemic lupus erythematosus patients. The mean IgE concentration was 442.0 ± 163.4 IU/ml (range 3.5-9936.0 IU/ml. Fifteen of the 69 patients had atopic disease, nine patients had severe sepsis and 56 patients presented with nephritis. The mean IgE level in 54 juvenile systemic lupus erythematosus patients without atopic manifestations was 271.6 ± 699.5 IU/ml, and only nine of the 31 (29% patients with high IgE levels had atopic disease. The IgE levels did not statistically differ with respect to the presence of atopic disease, severe sepsis, nephritis, disease activity, or tissue damage. Interestingly, IgE concentrations were inversely correlated with C4 levels (r = -0.25, p = 0.03 and with the SLICC/ACR-DI score (r = -0.34, p = 0.005. The IgE concentration was also found to be directly correlated with IgA levels (r = 0.52, p = 0.03. CONCLUSIONS: The present study demonstrated for the first time that juvenile systemic lupus erythematosus patients have increased IgE serum levels. This increase in IgE levels was not related to allergic or parasitic diseases. Our results are in line with the hypothesis that high IgE levels can be considered a marker of immune dysregulation.

  4. Causal Attributions about Disease-Onset and Relapse in Patients with Systemic Vasculitis

    Science.gov (United States)

    Grayson, Peter C.; Amudala, Naomi A.; McAlear, Carol A.; Leduc, Renée L.; Shereff, Denise; Richesson, Rachel; Fraenkel, Liana; Merkel, Peter A.

    2014-01-01

    Objectives Patients vary in their beliefs related to the cause of serious illness. The impact of these beliefs among patients with systemic vasculitis is not known. This study aimed to describe causal attributions about disease-onset and relapse in systemic vasculitis and to examine whether causal beliefs a) differ by type of vasculitis; and b) are associated with negative health outcomes. Methods Patients with vasculitis were recruited to complete an online questionnaire. Categories of causal beliefs were assessed with the Revised Illness Perception Questionnaire (IPQ-R). Differences in beliefs about disease-onset versus relapse were compared across different forms of vasculitis. Causal beliefs were assessed in association with several health outcomes including fatigue, functional impairments, and personal understanding of the condition. Results 692 patients representing 9 forms of vasculitis completed the questionnaire. The majority (90%) of patients had beliefs about the cause of their illness. Causal attributions were highly variable, but altered immunity and stress were the most commonly agreed upon causal beliefs. Frequencies of causal beliefs were strikingly similar across different forms of vasculitis, with few notable exceptions primarily in Behçet’s disease. Beliefs differed about causes of disease-onset versus relapse. Specific beliefs about disease-onset and relapse were weakly associated with fatigue, functional impairments, and understanding of the condition. Conclusion Patient beliefs related to the cause of systemic vasculitis are highly variable. Patterns of causal beliefs are associated with important negative health outcomes. Clinicians who care for patients with vasculitis should be mindful of these associations and consider asking about patients’ causal beliefs. PMID:24634202

  5. Juveniles in criminal procedure

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    Lukić Tatjana

    2012-01-01

    Full Text Available Taking into consideration the importance and role of children in modern society, as well as their position, this article has as its focus juveniles in criminal procedure. The existence of a separate juvenile justice system independent of the criminal law applicable to the adult offenders and general criminal procedure, as well as the periodic changes of the dominant approach in theory and practice reflects the ascendancy of different theoretical perspectives in the juvenile justice. In this paper, the authors scrutinize the models of responding to juvenile crime - justice and welfare model - as two models of the greatest importance in the present reaction of the society to the crimes conducted by the youngest delinquents at the beginning of the new century and millennium. Furthermore, the paper deals with a matter of international legal standards which, to a large extent, give shape to the legal framework for juvenile offenders and provide their rights and position in the criminal procedure. The authors refer to the internationally accepted documents on several levels. From the (almost universally accepted multilateral conventions on human rights, through the field of recommendations, rules and guidelines which are obeyed and enforced in practice of the juvenile justice although they are of non-binding nature, via the regional European legislative to the national provisions in a particular number of countries. On all the levels mentioned above the rights of the juveniles are regulated having in mind their possible role in the criminal procedure as a perpetrator of a criminal act, as a victim or as a witness. This paper also analyzes the criminal procedure with respect to juvenile perpetrators of the criminal acts in the Republic of Serbia and compliance of the provisions currently in vigor with the international legal standards contained in the international conventions and other internationally accepted and recognized instruments.

  6. Multiple system atrophy with prolonged survival: is late onset of dysautonomia the clue?

    Science.gov (United States)

    Calandra-Buonaura, Giovanna; Guaraldi, Pietro; Sambati, Luisa; Lopane, Giovanna; Cecere, Annagrazia; Barletta, Giorgio; Provini, Federica; Contin, Manuela; Martinelli, Paolo; Cortelli, Pietro

    2013-10-01

    Multiple system atrophy (MSA) is a neurodegenerative disease characterised by cardiovascular autonomic failure and/or urinary dysfunctions, associated with parkinsonism, cerebellar and/or corticospinal signs, usually leading to death after an average of 7 years. We describe the disease course of five patients diagnosed with probable MSA (4 with predominant parkinsonism and 1 with predominant cerebellar ataxia) who survived for more than 15 years and were followed throughout the disease course at our department. Cardiovascular autonomic dysfunction of any severity occurred late (mean latency from disease onset 9.4 ± 5 years) in this subgroup of MSA patients. The time of involvement of the urogenital system was more variable (from 0 to 14 years after disease onset) and manifested with symptoms of storage disorders (urinary urgency, frequency and incontinence) and erectile dysfunction in men. Conversely complains suggestive of urinary voiding dysfunction (incomplete bladder emptying and urinary retention) were not recorded and patients required catheterization only late in the disease course. In conclusion, our study showed that late onset of both cardiovascular autonomic failure and urinary voiding disorders may be positive prognostic factors in MSA irrespective of the MSA subtype.

  7. Late onset autosomal dominant cerebellar ataxia a family description and linkage analysis with the hla system

    Directory of Open Access Journals (Sweden)

    Walter O. Arruda

    1991-09-01

    Full Text Available A family suffering an autosomal dominant form of late onset hereditary cerebellar ataxia is described. Eight affected family members were personally studied, and data from another four were obtained through anamnesis. The mean age of onset was 37.1±5.4 years (27-47 years. The clinical picture consisted basically of a pure ataxic cerebellar syndrome. CT-scan disclosed diffuse cerebellar atrophy with relative sparing of the brainstem (and no involvement of supratentorial structures. Neurophysiological studies (nerve conduction, VEP and BAEP were normal. Twenty-six individuals were typed for HLA histocompatibility antigens. Lod scores were calculated with the computer program LINKMAP. Close linkage of the ataxia gene with the HLA system in this family could be excluded - 0==0,02, z=(-2,17 - and the overall analysis of the lod scores suggest another chromossomal location than chromosome 6.

  8. Juvenile Prostitution.

    Science.gov (United States)

    Csapo, Marg

    1986-01-01

    Recent research and Canadian government committee reports concerning juvenile prostitution are reviewed. Proposals are made in the realms of law and social policy; and existing programs are described. (DB)

  9. Hormone profile in juvenile systemic lupus erythematosus with previous or current amenorrhea.

    Science.gov (United States)

    Silva, Clovis A; Deen, Maria E J; Febrônio, Marilia V; Oliveira, Sheila K; Terreri, Maria T; Sacchetti, Silvana B; Sztajnbok, Flavio R; Marini, Roberto; Quintero, Maria V; Bica, Blanca E; Pereira, Rosa M; Bonfá, Eloisa; Ferriani, Virginia P; Robazzi, Teresa C; Magalhães, Claudia S; Hilário, Maria O

    2011-08-01

    To identify the underlying mechanism of amenorrhea in juvenile systemic lupus erythematosus (JSLE) patients, thirty-five (11.7%) JSLE patients with current or previous amenorrhea were consecutively selected among the 298 post-menarche patients followed in 12 Brazilian pediatric rheumatology centers. Pituitary gonadotrophins [follicle-stimulating hormone (FSH) and luteinizing hormone (LH)] and estradiol were evaluated in 32/35 patients, and prolactin and total testosterone in 29/35 patients. Patient's medical records were carefully reviewed according to demographic, clinical and therapeutic findings. The mean duration of amenorrhea was 7.2 ± 3.6 months. Low FSH or LH was observed in 7/32 (22%) JSLE patients and normal FSH or LH in 25 (78%). Remarkably, low levels of FSH or LH were associated with higher frequency of current amenorrhea (57% vs. 0%, P = 0.001), higher median disease activity (SLEDAI) and damage (SLICC/ACR-DI) (18 vs. 4, P = 0.011; 2 vs. 0, P = 0.037, respectively) and higher median current dose of prednisone (60 vs. 10 mg/day, P = 0.0001) compared to normal FSH or LH JSLE patients. None of them had decreased ovarian reserve and premature ovarian failure. Six of 29 (21%) patients had high levels of prolactin, and none had current amenorrhea. No correlations were observed between levels of prolactin and SLEDAI, and levels of prolactin and SLICC/ACR-DI scores (Spearman's coefficient). We have identified that amenorrhea in JSLE is associated with high dose of corticosteroids indicated for active disease due to hypothalamic-pituitary-ovary axis suppression.

  10. Bone mineral density in children with systemic lupus erythematosus and juvenile rheumatoid arthritis

    International Nuclear Information System (INIS)

    Kashef, S.; Saki, F.; Karamizadeh, Z.; Kashef, Mohammed Amin

    2007-01-01

    Although there is increasing interest in bone metabolism in patients with rheumatic disorders, few data exist on bone mineral density (BMD) in children with rheumatic disorders or on the association of BMD with disease-related variables. We determined BMD in Iranian children with systemic lupus erythematosus (SLE) and juvenile rheumatoid arthritis (JRA) to evaluate the relationship between disease related variables and BMD. Twenty patients (13 girls and 7 boys) with SLE (n=5) and JRA (n=5) with a mean age of 13.10+-3.29 years (range, 6-17 years), attending a pediatric rheumatology clinic and 20 healthy controls (matched for age and sex with each patient) were enrolled in a cross-sectional study between 2001 and 2003. BMD (g/cm) of the femoral neck (BMD-F) and lumbar vertebrae (BMD-L) were measured by dual energy x-ray absorptiometry (DEXA). The correlation between BMD and cumulative dose of steroids, daily dose of steroid, disease duration, disease activity, height, weight and age was investigated. BMD in the patients (BMD-F=0.72+-0.15, BMD-L=0.70+-0.19) was significantly lower than controls (BMD-F=0.95+-0.17, BMD-L=0.98+-0.20, P<0.001). The severity of decreased BMD was more prominent in lumbar vertebrae than the femoral neck (P=0.04). None of the variables were consistently related to decrease in BMD. BMD was significantly lower in patients compared with controls. It was more prominent in lumbar vertebrae (trabecular bone). Although cumulative doses of steroids and disease duration appeared to have some influence on BMD, none were independently correlated with BMD. (author)

  11. Determination of juvenile hormone titers by means of LC-MS/MS/MS and a juvenile hormone-responsive Gal4/UAS system in Aedes aegypti mosquitoes.

    Science.gov (United States)

    Zhao, Bo; Hou, Yuan; Wang, Jianjun; Kokoza, Vladimir A; Saha, Tusar T; Wang, Xue-Li; Lin, Ling; Zou, Zhen; Raikhel, Alexander S

    2016-10-01

    In anautogenous mosquitoes, juvenile hormone III (JH) plays an essential role in female post-eclosion (PE) development, preparing them for subsequent blood feeding and egg growth. We re-examined the JH titer during the reproductive cycle of female Aedes aegypti mosquitoes. Using liquid chromatography coupled with triple tandem mass spectrometry (LC-MS/MS/MS), we have shown that it reaches its peak at 48-54 h PE in the female hemolymph and at 72 h PE in whole body extracts. This method represents an effective assay for determination of JH titers. The 2.1-kb 5' promoter region of the Early Trypsin (ET) gene, which is specifically expressed in the female midgut under the control of JH during the PE phase, was utilized to genetically engineer the Ae. aegypti mosquito line with the ET-Gal4 activator. We then established the ET-GAL4>UAS-enhanced green fluorescent protein (EGFP) system in Ae. aegypti. In ET-Gal4>UAS-EGFP female mosquitoes, the intensity of the midgut-specific EGFP signal was observed to correspond to the ET gene transcript level and follow the JH titer during the PE phase. The EGFP signal and the EGFP transcript level were significantly diminished in midguts of transgenic female mosquitoes after RNA interference depletion of the JH receptor Methoprene-tolerant (Met), providing evidence of the control of ET gene expression by Met. Topical JH application caused premature enhancement of the EGFP signal and the EGFP transcript level in midguts of newly eclosed ET-Gal4>UAS-EGFP female mosquitoes, in which endogenous JH titer is still low. Hence, this novel ET-Gal4>UAS system permits JH-dependent gene overexpression in the midgut of Ae. aegypti female mosquitoes prior to a blood meal. Copyright © 2016 Elsevier Ltd. All rights reserved.

  12. Organ involvement other than lupus nephritis in childhood-onset systemic lupus erythematosus.

    Science.gov (United States)

    Huggins, J L; Holland, M J; Brunner, H I

    2016-07-01

    In this review we critically analyze pulmonary, gastrointestinal and cardiac manifestations of childhood-onset systemic lupus erythematosus (cSLE). Clinical manifestations of these organ systems may be the initial manifestation of cSLE; frequently occur with very active cSLE; and are potential life-threatening manifestations often presenting to the emergency department and requiring admission to the intensive care unit. Early recognition and treatment of the pulmonary, gastrointestinal and cardiac manifestations of cSLE will result in improved prognosis and better outcomes. © The Author(s) 2016.

  13. A new onset of systemic lupus erythematosus developed after bee venom therapy.

    Science.gov (United States)

    Rho, Young Hee; Woo, Jin-Hyun; Choi, Seong Jae; Lee, Young Ho; Ji, Jong Dae; Song, Gwan Gyu

    2009-09-01

    Lupus is a systemic autoimmune disease of an unknown origin, and systemic lupus erythematosus (SLE) can be triggered by numerous stimuli. Bee venom therapy is an alternative therapy that is believed to be effective for various kinds of arthritis. We present here a case of a 49-year-old female who experienced a new onset lupus after undergoing bee venom therapy, and this looked like a case of angioedema. The patient was successfully treated with high dose steroids and antimalarial drugs. We discuss the possibility of bee venom contributing to the development of SLE, and we suggest that such treatment should be avoided in patients with lupus.

  14. Early Lupus Project - A multicentre Italian study on systemic lupus erythematosus of recent onset.

    Science.gov (United States)

    Sebastiani, G D; Prevete, I; Piga, M; Iuliano, A; Bettio, S; Bortoluzzi, A; Coladonato, L; Tani, C; Spinelli, F R; Fineschi, I; Mathieu, A

    2015-10-01

    Systemic lupus erythematosus (SLE) is an autoimmune disease with a high degree of variability at onset that is problematic for a correct and prompt diagnosis. We undertook this project with the purpose of collecting an inception cohort of Italian patients with recent-onset SLE, in order to obtain information on the main clinical and serological characteristics at the beginning of the disease. In this first report we describe the characteristics of this cohort at study entry. All patients with a diagnosis of SLE (1997 ACR criteria) and a disease duration less than 12 months were consecutively enrolled between 1 January 2012 and 31 December 2013 in a multicentre prospective study. Information on clinical and serological characteristics at study entry and then every six months was collected into a specific electronic database. Statistical analysis was performed by means of the Openstat program. Among 122 patients enrolled (103 F) 94.3% were Caucasians. Mean age (SD) of patients at study entry was 37.3 (14.3) years, mean age at disease onset was 34.8 (14.3) years, mean age at diagnosis was 36.9 (14.3) years, and mean disease duration was 2.9 (3.9) months. The frequency of the manifestations included in the 1997 ACR criteria was as follows: ANA 97.5%, immunologic disorders (anti-dsDNA, anti-Sm, antiphospholipid antibodies) 85.2%, arthritis 61.8%, haematologic disorders 55.7%, malar rash 31.1%, photosensitivity 29.5%, serositis 27%, renal disorders 27%, oral/nasal ulcers 11.5%, neurologic disorders 8.2%, and discoid rash 5.7%. The cumulative frequency of mucocutaneous symptoms was 77.8%. At enrolment, autoantibody frequency was: ANA 100%, anti-dsDNA 83.6%, anti-SSA 28%, anticardiolipin 24.5%, anti-nRNP 20.4%, anti-beta2GPI 17.2%, lupus anticoagulant 16.3%, anti-Sm 16%, and anti-SSB 13.1%. In this paper we describe the main clinical and serological characteristics of an Italian inception cohort of patients with recent-onset SLE. At disease onset, mucocutaneous

  15. The Onset of Chaos via Asymptotically Period-Doubling Cascade in Fractional Order Lorenz System

    Science.gov (United States)

    Lin, Xiaofang; Liao, Binghui; Zeng, Caibin

    2017-12-01

    Little seems to be known about the chaotification problem in the framework of fractional order nonlinear systems. Based on the negative damping instability mechanism and fractional calculus technique, this paper reports the onset of chaos in fractional order Lorenz system with periodic system parameters via asymptotically period-doubling cascade. To further understand the complex dynamics of the system, some basic properties such as the largest Lyapunov exponents, bifurcation diagram, routes to chaos, asymptotically periodic windows, possible chaotic and asymptotically periodic window parameter regions, and the compound structure of the system are analyzed and demonstrated with careful numerical simulations. Of particular interest is a striking finding that fractional derivative can chaotify the globally stable periodic system without feedback control.

  16. Epidemiology of juvenile violence.

    Science.gov (United States)

    Farrington, D P; Loeber, R

    2000-10-01

    It is difficult to review the epidemiology of juvenile violence because few studies focus specifically on this topic as opposed to childhood aggression or delinquency in general. More research is needed specifically on juvenile violence, which is generally measured using official records or self-reports. Self-report research shows that a substantial fraction of the male juvenile population commits violence, and that very few violent acts are followed by arrests or convictions. Racial differences in violence may be explainable by reference to racial differences in community contexts. There is a great deal of versatility in juvenile violence. Juveniles who commit one type of violent offense also tend to commit other types and nonviolent offenses. Violent offenders tend to be persistent or frequent offenders, and there is little difference between violent offenders and nonviolent but equally frequent offenders. Nevertheless, there is some degree of specialization in violence. More research is needed to investigate whether risk factors exist for violence that are not risk factors for serious nonviolent delinquency (e.g., biologic factors). Violent juveniles tend to have co-occurring problems such as victimization, substance abuse, and school failure. Often, they might be described as multiple-problem youth. There is considerable continuity from childhood aggression to juvenile violence. An early age of onset of violence predicts a large number of violent offenses. The major long-term risk factors for juvenile violence are individual (high impulsiveness and low intelligence, possibly linked to the executive functions of the brain), family (poor supervision, harsh discipline, child physical abuse, a violent parent, large family size, poverty, a broken family), peer delinquency, gang membership, urban residence, and living in a high-crime neighborhood (characterized by gangs, guns, and drugs in the United States). More research is needed on interactions among risk factors

  17. Serum Neuroinflammatory Disease-Induced Central Nervous System Proteins Predict Clinical Onset of Experimental Autoimmune Encephalomyelitis

    Directory of Open Access Journals (Sweden)

    Itay Raphael

    2017-07-01

    Full Text Available There is an urgent need in multiple sclerosis (MS patients to develop biomarkers and laboratory tests to improve early diagnosis, predict clinical relapses, and optimize treatment responses. In healthy individuals, the transport of proteins across the blood–brain barrier (BBB is tightly regulated, whereas, in MS, central nervous system (CNS inflammation results in damage to neuronal tissues, disruption of BBB integrity, and potential release of neuroinflammatory disease-induced CNS proteins (NDICPs into CSF and serum. Therefore, changes in serum NDICP abundance could serve as biomarkers of MS. Here, we sought to determine if changes in serum NDICPs are detectable prior to clinical onset of experimental autoimmune encephalomyelitis (EAE and, therefore, enable prediction of disease onset. Importantly, we show in longitudinal serum specimens from individual mice with EAE that pre-onset expression waves of synapsin-2, glutamine synthetase, enolase-2, and synaptotagmin-1 enable the prediction of clinical disease with high sensitivity and specificity. Moreover, we observed differences in serum NDICPs between active and passive immunization in EAE, suggesting hitherto not appreciated differences for disease induction mechanisms. Our studies provide the first evidence for enabling the prediction of clinical disease using serum NDICPs. The results provide proof-of-concept for the development of high-confidence serum NDICP expression waves and protein biomarker candidates for MS.

  18. Growth of juvenile Atlantic cod Gadus morhua in land-based recirculation systems: Effects of feeding regime, photoperiod and diet

    DEFF Research Database (Denmark)

    Fülberth, Michael; Moran, Damian; Jarlbæk, Henrik

    2009-01-01

    feeding duration. In each RAS unit three diets (A, B and C) were tested, which were broadly similar in composition but from different manufacturers. Water exchange rate averaged 10–19% in the three recirculation systems, and key water quality parameters such as NH4+ and CO2 remained at low effect......The combined effect of feeding regime and photoperiod on the growth of juvenile Atlantic cod Gadus morhua in land-based recirculating aquaculture systems (RAS) was examined using three different commercial diets. Fish of 8–10 g were reared in 1 m3 tanks at an initial density of 10 kg m− 3 for 78 d...

  19. RESTORATIVE JUSTICE DALAM SISTEM PERADILAN PIDANA ANAK / Restorative Justice In Juvenile Justice System

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    Randy Pradityo

    2016-11-01

    Full Text Available Anak sebagai generasi penerus bangsa sudah selayaknya mendapatkan perhatian khusus. Hal tersebut bertujuan dalam rangka pembinaan anak untuk mewujudkan sumber daya manusia yang berkualitas. Maka dari itu, diperlukan pula sarana dan prasarana hukum yang mengantisipasi segala permasalahan yang timbul. Sarana hukum ini bertujuan untuk mengantisipasi stigma atau cap jahat yang ditimbulkan ketika anak berhadapan dengan hukum, sekaligus memulihkan dan memasyarakatkan kembali anak tersebut. Salah satu solusinya adalah dengan mengalihkan atau menempatkan pelaku tindak pidana anak keluar dari sistem peradilan pidana serta memberikan alternatif bagi penyelesaian dengan pendekatan keadilan demi kepentingan terbaik bagi anak, yang kemudian dikenal dengan pendekatan restorative justice. Restorative justice yang merupakan implementasi konsep dari diversi telah dirumuskan dalam sistem peradilan pidana anak, namun sistem yang baik haruslah diiringi dengan suatu sikap yang dijiwai kehendak untuk memandang dan berkeyakinan bahwa dunia ini selalu menjadi lebih baik. Selain itu, hendaknya prinsip the best interest of the children selalu diutamakan ketika menangani anak yang berhadapan dengan hukum.   Children as the nation's next generation is already deserve special attention. It aims in order to develop the child to realize the quality of human resources. Therefore, it is also necessary legal infrastructure to anticipate any problems that arise. The legal means to anticipate stigma or stamp evil inflicted when the child against the law, as well as restoring and re-socialize the child. One solution is to divert or placing the offender children out of the criminal justice system as well as providing an alternative to the settlement with justice approach in the best interests of the child, who was then known as restorative justice approach. Restorative justice which is the implementation of the concept of diversion has been formulated in the juvenile justice system

  20. Initial manifestations and clinical course of systemic onset juvenile idiopathic arthritis: A ten-year retrospective study

    Directory of Open Access Journals (Sweden)

    Hu-Yuan Tsai

    2012-10-01

    Conclusion: In SoJIA, extra-articular features such as fever, rash, and lymphadenopathy are most prominent. Leukocytosis and polyarticular pattern on presentation may indicate a refractory clinical course.

  1. Restorative Discipline as an Alternative to Retributive Discipline within the Juvenile Court System: An Analysis of the Metro County Juvenile Court Community Restorative Board

    Science.gov (United States)

    Banjoko, Ajamu A.

    2009-01-01

    Giroux (2003) indicated that the prison industry has become a major economic industry with many states spending more money on prison reforms than on educational reforms. Juvenile delinquent behavior should be punished but fair treatment and equal rights for all human beings under the rule of law is paramount to punishment. Casella (2001) indicated…

  2. Herpes zoster em pacientes com lúpus eritematoso sistêmico juvenil Herpes zoster in patients with juvenile systemic lupus erythematosus

    Directory of Open Access Journals (Sweden)

    Paula da Silva Neves

    2007-04-01

    Full Text Available Infecção pelo vírus varicela zoster (VVZ em pacientes com lúpus eritematoso sistêmico juvenil (LESJ tem sido pouco descrita. Durante um período de 12 anos, ocorreram 195 internações em 77 pacientes com LESJ e estas foram acompanhadas pela Unidade de Reumatologia Pediátrica do Instituto da Criança do Hospital das Clínicas da Universidade de São Paulo. Onze pacientes (14%, dez do sexo feminino, apresentaram 14 internações (7,1% pelo VVZ. Nesses pacientes, a média de idade foi de 16 anos e 5 meses e a média do tempo de duração do LESJ até a primeira infecção devido ao VVZ foi de 4 anos. Todos os episódios das infecções estavam associados com atividade da doença, que se apresentaram como lesões vesicobolhosas seguindo trajeto nervoso. As regiões do tórax e membros foram mais comumente afetadas. Todos haviam utilizado prednisona e quatro usaram ciclofosfamida EV. Todos receberam aciclovir EV por 7 a 10 dias. Nenhum paciente apresentou neuralgia pós-herpética, infecção bacteriana secundária ou evoluiu para óbito. Entretanto, uma paciente em uso de aciclovir apresentou amaurose aguda por vasculite necrosante retiniana bilateral associado ao VVZ, necessitando de duas aplicações de ganciclovir intravítreo e gamaglobulina EV (2 g/kg/dose, com recuperação parcial da acuidade visual. Assim sendo, infecção por VVZ em pacientes com LESJ foi infreqüente, habitualmente associada à atividade da doença e à corticoterapia. Essa infecção foi controlada com aciclovir, e os pacientes raramente apresentaram complicações.Varicella zoster virus (VZV infection in patients with juvenile systemic lupus erythematosus (JSLE has been rarely described. 195 hospitalizations of 77 JSLE patients occurred in a period of 12 years and were followed at the Pediatric Rheumatology Unity of the Instituto da Criança - Hospital das Clínicas - Universidade de São Paulo. Eleven patients (14%, 10 female, had 14 hospitalizations (7.1% due to

  3. Discharge Onset Voltage Prediction for a Gas-Insulated System Via the Figure-of-Merit Concept

    DEFF Research Database (Denmark)

    Crichton, George C; Vibholm, Svend

    1987-01-01

    The accuracy of discharge onset prediction via thefigur figure-of-merit concept for a strongly electronegative gas is examined. A coaxial system is employed, for which the inner electrode possesses a surface roughness of Ra=35 ¿m. With SF6 as the insulating medium a reference discharge-onset cha......The accuracy of discharge onset prediction via thefigur figure-of-merit concept for a strongly electronegative gas is examined. A coaxial system is employed, for which the inner electrode possesses a surface roughness of Ra=35 ¿m. With SF6 as the insulating medium a reference discharge...

  4. Effect of systemic medications on onset and progression of diabetic retinopathy.

    Science.gov (United States)

    Silva, Paolo S; Cavallerano, Jerry D; Sun, Jennifer K; Aiello, Lloyd M; Aiello, Lloyd Paul

    2010-09-01

    Diabetic retinopathy remains a leading cause of visual loss worldwide. Patients with diabetes mellitus commonly have multiple comorbidities treated with a wide variety of medications. Systemic medications that target glycemic control and coexisting conditions may have beneficial or deleterious effects on the onset or progression of diabetic retinopathy. In addition, data is accumulating to suggest that the use of systemic therapy primarily to address ocular complications of diabetic retinopathy may be a promising therapeutic approach. This article reviews our current understanding of the ocular-specific effects of systemic medications commonly used by patients with diabetes mellitus, including those directed at control of hyperglycemia, dyslipidemia, hypertension, cardiac disease, anemia, inflammation and cancer. Current clinical evidence is strongest for the use of angiotensin-converting enzyme inhibitors and angiotensin-2 receptor blockers in preventing the onset or slowing the progression of early diabetic retinopathy. To a more limited extent, evidence of a benefit of fibrates for diabetic macular edema exists. Numerous other agents hold considerable promise or potential risk. Thus, these compounds must undergo further rigorous study to determine the actual clinical efficacy and adverse effects before definitive therapeutic care recommendations can be offered.

  5. Onset of liquid droplet entrainment on a direct vessel injection system for APR1400

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Han-sol; Lee, Jae-Young [Handong Global University, Pohang (Korea, Republic of); Kim, Jong-Rok; Euh, Dong-Jin [Korea Atomic Energy Research Institute, Daejeon (Korea, Republic of)

    2016-10-15

    In this research, a series of visualization works was conducted to understand droplet entrainment of the flow pattern generated in direct vessel injection system(DVI) of Korea nuclear power plant, APR 1400. In the emergency situation of a nuclear power plant, reliability of DVI cooling can be an important issue. It is known that, the amount and the rate of entrainment during the DVI cooling process can significantly affect the total heat removal. To visualize the film Reynolds number closely related with onset of droplet entrainment induced by falling film flow and lateral air flow in a small gap, confocal chromatic sensing method for measuring accurately film thickness and depth averaging particle image velocimetry for film velocity were used. The results have been post processed 4G Insight software. By measuring two dimensional film Reynolds number, we can predict the onset of droplet entrainment and obtain visible breakup region intuitively. To visualize the droplet entrainment induced by falling film flow and lateral air flow in a small gap, shadowgraph method with CCD camera (2200fps, 1280 pixel X 800 pixel, ) on coated plate with super water-repellent agent was used. The results have been post processed using 4G Insight software. By measuring two dimensional film Reynolds number, we can predict the onset of droplet entrainment and obtain visible breakup region intuitively. By adopting both super hydrophobic coating method and shadowgraph method, entrainment in a narrow gap was successfully visualized that has rarely performed before and meaningful results for DVI system research fields have been made.

  6. CLINICAL SIGNIFICANCE OF ANTIPHOSPHOLIPID ANTIBODIES AND GENE MUTATIONS IN HEMOSTASIS OF CHILDREN WITH SYSTEMIC LUPUS ERYTHEMATOSUS AND JUVENILE DERMATOMYOSITIS

    Directory of Open Access Journals (Sweden)

    O.A. Solntseva

    2006-01-01

    Full Text Available Thrombophilia in children with diffuse connective tissue disorders as systemic lupus erythematosus (SLE and juvenile dermatomyositis (JDM could arise from various causes including peripherial blood circulation of antiphospholipid antibodies (APH and genetic mutations in the system of hemostasis. Thrombosis is a serious and prognostically unfavorable complication that has negative impact on the underlying disease course. The study included 96 children, 65 of them had diagnosed SLE and the other 31 had JDM. The Elisa method was used to detect antiphospholipid antibodies, coagulation method was used to detect lupus anticoagulant (LAC and antibodies to cardiolipins (anticl, ?2:glycoprotein 1 (anti ? 2 gp 1 and prothrombin (APT. The PCR method (DNA diagnostics was used to detect DNA mutations as factor resistance to of activated protein c (Leiden 5,10 methylen tetrahydrofolate reductase (MTHFR gene polymorphism. The incidence of APL antibodies was registered in 61.5% patients with SLE and in 32.2% of patients with JDM. Ac ligg, anti ?2 gp 1 Igg were clinically significant in thrombotic events in patients with SLE and JDM, and so was LAC in patients with SLE. The prevalence of the hemostasis system mutations is concordant with reported data. Conclusion thrombophilia is frequently associated with APH antibodies or combination of APH antibodies with genetic abnormalities. Sole genetic mutations are salient in patients with JDM.Key words: thrombophilia, systemic lupus erythematosus, juvenile dermatomyositis, antiphospholipid antibodies, lupus anticoagulant, leiden, prothrombin, methylentet rahydrofolate reductase.

  7. A critical examination of "being Black" in the juvenile justice system.

    Science.gov (United States)

    Peck, Jennifer H; Jennings, Wesley G

    2016-06-01

    The current study examined the role of race in juvenile court outcomes across 3 decision-making stages. This analysis was conducted with a random sample of all delinquent referrals in a Northeast state from January 2000 through December 2010 (N = 68,188). In addition to traditional logistic regression analysis, a propensity score matching (PSM) approach was utilized to create comparable samples of Black and White youth and provide a more rigorous methodological test of the relationship between race and juvenile court processing. Results indicated that even after the use of PSM techniques, race was still found to influence the likelihood of intake (OR = 1.54; 95% C.I. = 1.48-1.62, p social control. They also reaffirm the noticeable role that selection bias can play in the research surrounding race differences in juvenile court outcomes, and highlight the importance of utilizing a more stringent statistical model to control for selection bias. (PsycINFO Database Record (c) 2016 APA, all rights reserved).

  8. Congenital eyelid ptosis: onset and prevalence of amblyopia, associations with systemic disorders, and treatment outcomes.

    Science.gov (United States)

    Stein, August; Kelly, John P; Weiss, Avery H

    2014-10-01

    To determine the age at onset of amblyopia, the response to occlusion therapy, and the association with systemic disorders in children with congenital eyelid ptosis. Retrospective chart review of children seen at Seattle Children's Hospital with moderate or severe congenital ptosis. Assessments were longitudinal visual acuity development using objective methods, definition of ptosis severity by eyelid margin to pupillary light reflex distance (margin reflex distance [MRD]), age at amblyopia diagnosis, correlation between amblyopia and MRD, and associated systemic disorders. Eighty-four children with moderate-to-severe congenital ptosis met inclusion criteria; the mean longitudinal follow-up was 49.1 months. Fifteen (18%) of these children had amblyopia, of which 9 had deprivation amblyopia (mean age 17.3 months ± 11.2) and 6 had anisometropic or strabismic amblyopia (mean age 60 months ± 11.8). Eleven (73%) of the children with amblyopia were successfully treated with occlusion therapy. Amblyopia was not correlated with MRD. A systemic disorder was identified in 29 (35%) of the children, the most common being genetic, chromosomal, or neurologic conditions. Patients with systemic disorders and developmental delay have significantly lower visual acuity bilaterally compared with patients without systemic disorders (P ≤ .003). Using longitudinal and objective visual acuity assessments, the incidence of amblyopia was 18% in children with moderate to severe congenital ptosis. Visual deprivation was the predominant risk factor that was reliably distinguished by its earlier onset in young children. The best indicator of amblyopia in children is visual acuity rather than MRD measurements. Systemic disorders are frequent in children with moderate to severe congenital ptosis. Copyright © 2014 Elsevier Inc. All rights reserved.

  9. Effects of dietary oxidized fish oil supplementation on oxidative stress and antioxidant defense system in juvenile rainbow trout (Oncorhynchus mykiss).

    Science.gov (United States)

    Fontagné-Dicharry, Stéphanie; Larroquet, Laurence; Dias, Karine; Cluzeaud, Marianne; Heraud, Cécile; Corlay, Dominique

    2018-03-01

    The objective of the study was to characterize the response of the antioxidant defense system against dietary prooxidant conditions in rainbow trout juveniles. Fish (initial mean weight: 62 ± 1 g) were fed three fishmeal and plant-derived protein-based diets supplemented with 15% fresh fish oil (CTL diet), 15% fresh fish oil from tuna by-products (BYP diet) or 15% autooxidized fish oil (OX diet) over a 12-week growth trial at 17.5 ± 0.5 °C. No significant differences in growth performance were recorded between dietary groups. Muscle lipid content was reduced and n-6 PUFA levels were increased in rainbow trout fed diets BYP and OX compared to CTL. After 12 weeks of feeding, the level of lipid peroxidation products in muscle was not affected whereas the 8-isoprostane content in liver was increased in fish fed diet OX as well as plasma total and oxidized glutathione contents. The hepatic and muscle contents for α-tocopherol were decreased in fish fed BYP and OX. Hepatic antioxidant enzyme activities and mRNA levels were not affected after 12 weeks of feeding, except for catalase and glutathione peroxidase 1b2 mRNA levels that were decreased in trout fed diet OX. Fish fed diet OX and BYP displayed also reduced cytosolic Nrf2 and both cytosolic and nuclear NF-κB protein levels in liver. The present work indicates that feeding rainbow trout juveniles with fresh fish oil from by-products or moderately oxidized lipid appears not to be detrimental to the growth performance of fish. The mechanisms beyond the control of the antioxidant defense system by moderately oxidized lipid require further investigations in rainbow trout juveniles. Copyright © 2017 Elsevier Ltd. All rights reserved.

  10. The family journey-to-diagnosis with systemic juvenile idiopathic arthritis: a cross-sectional study of the changing social media presence

    Directory of Open Access Journals (Sweden)

    Modica RF

    2016-05-01

    Full Text Available Renee F Modica,1 Kathleen Graham Lomax,2 Pamela Batzel,3 Leah Shapardanis,3 Kimberly Compton Katzer,3 Melissa E Elder1 1Division of Pediatric Rheumatology, Immunology and Infectious Diseases, University of Florida, Gainesville, FL, USA; 2Immunology and Dermatology Medical Affairs, Novartis Pharmaceuticals Corporation, East Hanover, USA; 3Treato, Princeton, NJ, USA Background: Children with systemic juvenile idiopathic arthritis (SJIA often encounter a delay between symptom onset and disease diagnosis, partly due to the broad differential of fever and lack of symptom recognition by providers. Families often seek multiple medical opinions and post on social media about their frustrations. This linguistic analysis observed the changing language patterns and social media posting behaviors of parents in the time leading to, during, and after SJIA diagnosis. Methods: Public social media sites were manually reviewed by a linguistic team to evaluate posts about SJIA from US-based parents. Results: A total of 3,979 posts between July 2001 and January 2015 were reviewed from 108 sites. Pre-SJIA diagnosis parents sought answers and shared status updates on social media, focusing primarily on the following three site types: alternative/natural lifestyle forums (39%, Facebook (27%, and disease-specific forums (17%. Posts during early prediagnosis phases were characterized by expressive language showing confidence in health care providers and trust in parental instincts. At later prediagnosis stages, parents continued to use social media, but the posts demonstrated increased frustration with delays in diagnosis and gaps in communication with providers. More objective symptom descriptions and a greatly reduced child-centered emotional focus were observed as parents shifted into caregiving roles. Once the diagnosis of SJIA was confirmed, parents used straightforward, less expressive language, and Facebook (47% to make "announcement" posts and increased their

  11. Autoantibodies to IL-1 alpha in sera from umbilical cords, children, and adults, and from patients with juvenile chronic arthritis

    DEFF Research Database (Denmark)

    Müller, K; Hansen, M B; Zak, M

    1996-01-01

    umbilical cords (n = 11), children (n = 45), and adults (n = 20), as well as in 51 patients with juvenile chronic arthritis (JCA) of pauciarticular (n = 34), polyarticular (n = 8), or systemic onset type (n = 9). RESULTS. The frequency of positive sera was significantly lower in children than in cord blood...

  12. The influence of intrauterine exposure to immunosuppressive treatment on changes in the immune system in juvenile Wistar rats.

    Science.gov (United States)

    Kabat-Koperska, Joanna; Kolasa-Wołosiuk, Agnieszka; Wojciuk, Bartosz; Wojciechowska-Koszko, Iwona; Roszkowska, Paulina; Krasnodębska-Szponder, Barbara; Paczkowska, Edyta; Safranow, Krzysztof; Gołembiewska, Edyta; Machaliński, Bogusław; Ciechanowski, Kazimierz

    2016-01-01

    In our study, we assessed the impact of immunosuppressive drug combinations on changes in the immune system of juvenile Wistar rats exposed to these drugs during pregnancy. We primarily concentrated on changes in two organs of the immune system - the thymus and the spleen. The study was conducted on 40 (32+8) female Wistar rats administered full and half dose of drugs, respectively, subjected to regimens commonly used in therapy of human kidney transplant recipients ([1] cyclosporine A, mycophenolate mofetil, and prednisone; [2] tacrolimus, mycophenolate mofetil, and prednisone; [3] cyclosporine A, everolimus, and prednisone). The animals received drugs by oral gavage 2 weeks before pregnancy and during 3 weeks of pregnancy. There were no statistically significant differences in the weight of the thymus and spleen, but changes were found in the results of blood hematology, cytometry from the spleen, and a histologic examination of the examined immune organs of juvenile Wistar rats. In the cytokine assay, changes in the level of interleukine 17 (IL-17) after increasing amounts of concanavaline A were dose-dependent; the increase of IL-17 was blocked after administration of higher doses of immunosuppressive drugs. However, after a reduction of doses, its increase resumed. Qualitative, quantitative, and morphological changes in the immune system of infant rats born to pharmacologically immunosuppressed females were observed. Thymus structure, spleen composition, and splenocyte IL-17 production were mostly affected in a drug regimen-dependent manner.

  13. Retinoschisis (Juvenile)

    Science.gov (United States)

    ... of central and peripheral (side) vision due to degeneration of the retina. What are the symptoms? Juvenile retinoschisis, also known ... compounds that can preserve vision. Future stem cell treatments might also ... dorzolamide may improve retinal health and restore some vision in people with ...

  14. Juvenile angiofibromer

    DEFF Research Database (Denmark)

    Thuesen, Anne Daugaard; Jakobsen, John; Nepper-Rasmussen, Jørgen

    2005-01-01

    Juvenile angiofibroma is a rare, benign, rich vascular tumor, and approximately one new case is diagnosed in Denmark each year. It sits in the foramen sphenopalatinum and occurs in boys from 14 to 25 years of age. The most frequent initial symptoms are nasal obstruction and epistaxis. Through...

  15. Learning and memory deficits in male adult mice treated with a benzodiazepine sleep-inducing drug during the juvenile period

    Directory of Open Access Journals (Sweden)

    Yusuke Furukawa

    2016-07-01

    Full Text Available Gamma-aminobutyric acid (GABA, the major inhibitory neurotransmitter in the mammalian central nervous system, is also known to be important for brain development. Therefore, disturbances of GABA receptor (GABA-R mediated signaling (GABA-R signal during brain development may influence normal brain maturation and cause late-onset brain malfunctions. In this study, we examined whether the temporal stimulation of the GABA-R signal during brain development induces late-onset adverse effects on the brain in adult male mice. To stimulate the GABA-R signal, we used either the benzodiazepine sleep-inducing drug triazolam (TZ or the non-benzodiazepine drug zolpidem (ZP. We detected deficits in learning and memory in mice treated with TZ during the juvenile period, as seen in the fear conditioning test. On the other hand, ZP administration during the juvenile period had little effect. In addition, decreased protein expression of GluR1 and GluR4, which are excitatory neurotransmitter receptors, was detected in the hippocampi of mice treated with TZ during the juvenile period. We measured mRNA expression of the immediate early genes (IEGs, which are neuronal activity markers, in the hippocampus shortly after the administration of TZ or ZP to juvenile mice. Decreased IEG expression was detected in mice with juvenile TZ administration, but not in mice with juvenile ZP administration. Our findings demonstrate that TZ administration during the juvenile period can induce irreversible brain dysfunction in adult mice. It may need to take an extra care for the prescription of benzodiazepine sleep-inducing drugs to juveniles because it might cause late onset learning and memory defects.

  16. Evaluation of quality indicators and disease damage in childhood-onset systemic lupus erythematosus patients.

    Science.gov (United States)

    Harris, Julia G; Maletta, Kristyn I; Kuhn, Evelyn M; Olson, Judyann C

    2017-02-01

    The aim of this study was to describe compliance with select quality indicators and assess organ-specific dysfunction in a childhood-onset systemic lupus erythematosus population by using a validated damage index and to evaluate associations between compliance with quality indicators and disease damage. A retrospective chart review was performed on patients diagnosed with systemic lupus erythematosus prior to age 18 followed at a single center in the USA from 1999 to 2012 (n = 75). Data regarding quality indicators and outcome variables, including the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index, were collected. The median disease duration was 3.8 years. The proportion of patients or patient-years in which care complied with the proposed quality measures was 94.4% for hydroxychloroquine use, 84.3% for vitamin D recommendation,75.8% for influenza vaccination (patient-years), 67.2% for meningococcal vaccination, 49.0% for ophthalmologic examination (patient-years), 31.7% for pneumococcal vaccination, and 28.6% for bone mineral density evaluation. Disease damage was present in 41.3% of patients at last follow-up, with an average damage index score of 0.81. Disease damage at last follow-up was associated with minority race/ethnicity (p = 0.008), bone mineral density evaluation (p = 0.035), and vitamin D recommendation (p = 0.018). Adherence to quality indicators in a childhood-onset systemic lupus erythematosus population is varied, and disease damage is prevalent. This study highlights the importance of quality improvement initiatives aimed at optimizing care delivery to reduce disease damage in pediatric lupus patients.

  17. Diagnosing early onset dementia and then what? A frustrating system of aftercare resources

    Directory of Open Access Journals (Sweden)

    Chemali Z

    2012-01-01

    Full Text Available Z Chemali1–3, S Schamber2, EC Tarbi2, D Acar1,2, M Avila-Urizar21Harvard Medical School, 2Departments of Neurology and Psychiatry, Division of Cognitive and Behavioral Neurology, Brigham and Women’s Hospital, 3Departments of Psychiatry and Neurology, Massachusetts General Hospital, Boston, MA, USAAbstract: Recent studies indicate that the prevalence of early onset dementia (EOD is more common than it was once presumed. As such, and considering the substantial challenges EOD presents to the patient, caregivers, and health care providers, this study sought to investigate the mechanism of care delivered to these patients. A medical record chart review was conducted for 85 patients attending a memory disorder unit who initially presented to rule out EOD as a working diagnosis. The results suggest that while the majority of these patients received an extensive work-up and were heavily medicated, they remained at home, where they lacked adequate age-related services and could not be placed, despite the crippling caregiver burden. This manuscript is a platform to discuss our current system limitations in the care of these patients with an eye on new opportunities for this challenging group.Keywords: early onset dementia, social work, services, caregiving

  18. Association studies of serotonin system candidate genes in early-onset obsessive-compulsive disorder.

    Science.gov (United States)

    Dickel, Diane E; Veenstra-VanderWeele, Jeremy; Bivens, Nancy Chiu; Wu, Xiaolin; Fischer, Daniel J; Van Etten-Lee, Michelle; Himle, Joseph A; Leventhal, Bennett L; Cook, Edwin H; Hanna, Gregory L

    2007-02-01

    Family-based evidence for association at serotonin system genes SLC6A4, HTR1B, HTR2A, and brain-derived neurotrophic factor (BDNF) has been previously reported in obsessive-compulsive disorder (OCD). Early-onset OCD is a more familial form of the disorder. We used the transmission-disequilibrium test of association at common polymorphisms in each of these genes in 54 parent-child trios ascertained through probands with early-onset OCD. No evidence for association was detected at any of the polymorphisms in the entire set of subjects. Nominally significant association was found at the HTR2A rs6311 polymorphism in subjects with tic disorder and OCD (p = .05), replicating a previous finding in Tourette syndrome and OCD. Nominally significant association was also found for the SLC6A4 HT transporter gene-linked polymorphic region (5-HTTLPR) polymorphism for female subjects (p = .03). Neither association would remain significant after statistical correction for multiple testing. Despite no individual study reporting replication, a pooled analysis of five replication studies of the SLC6A4 5-HTTLPR polymorphism supports association (p = .02). Low power across individual association studies in OCD may lead to a false acceptance of the null hypothesis. Accumulation of evidence from multiple studies will be necessary to evaluate the potential role for these genes in contributing to susceptibility to OCD.

  19. Lifetime exposure to traumatic events among adolescents in contact with the Nigerian juvenile justice systems compared with a comparison group of secondary school students.

    Science.gov (United States)

    Atilola, Olayinka; Omigbodun, Olayinka; Bella-Awusah, Tolulope

    2014-05-01

    There are some knowledge gaps in what is known about pre-contact exposure to traumatic events among adolescents within the juvenile justice system. Data often focus on psychological sequelae without describing the traumatic events. In addition, there are few data from sub-Saharan Africa where juvenile justice inmates are often minor offenders and may themselves have been victims of abuse and neglect. To present detailed data on the lifetime prevalence rate and pattern of traumatic events among a cohort of adolescents in juvenile justice custody in Nigeria and to compare inmates who are 'offenders' with those who are 'victims'. Inmates of a borstal and a remand home comprised the study group and age- and gender-matched adolescents from two government schools were the secondary comparison group. The trauma-checklist of the Current and Lifetime Version of the Kiddies Schedule for Affective Disorders and Schizophrenia was used as a guide in assessing traumatic events. Of a total of 408 adolescents, 204 were recruited from the two juvenile justice institutions and 204 from secondary schools. Ninety per cent of participants were male and the mean (SD) age was 15·9 (2·8) years. The prevalence rate of lifetime exposure to traumatic events among the juvenile justice offenders was 88·7% compared with 48·5% of the comparison group (P = 0·001). The most commonly reported specific lifetime traumatic event was physical abuse (52·8%). The institutionalised adolescents were significantly more likely to report lifetime exposure to almost all the traumatic events assessed. Apart from the perpetrators of violent crime, there was no statistically significant difference in the prevalence and pattern of lifetime exposure to traumatic events between the offenders and the victims. This study provides further evidence that exposure to traumatic events is a fact of life for inmates of juvenile institutions, irrespective of whether they are offenders or victims. The implications

  20. Púrpura trombocitopênica e anemia hemolítica auto-imune em pacientes internados com lúpus eritematoso sistêmico juvenil Trombocytopenic purpura and autoimmune hemolytic anemia in hospitalized patients with juvenile systemic lupus erythematosus

    Directory of Open Access Journals (Sweden)

    Jochebed Kyoung Kim

    2007-02-01

    esplenectomia. CONCLUSÕES: o CHE isolado foi uma manifestação grave em pacientes internados com LESJ, habitualmente associado a uma doença ativa e sistêmica.OBJECTIVE: to evaluate the hematological involvement (HI in hospitalized patients with juvenile systemic lupus erythematosus (JSLE. METHODS: from 1994 to 2005, 195 admissions occurred in 77 JSLE patients (American College of Rheumatology criteria and were followed by the Pediatric Rheumatology Unit of the Instituto da Criança - University of São Paulo. These admissions were evaluated according to the presence of HI at onset or during the evolution of the disease: autoimmune hemolytic anemia (AHA or thrombocytopenic purpura. All patients performed at least two complete blood counts. AHA was defined by a fall in hemoglobin levels (beyond 2 g/dl, reticulocytosis, increase in lactate dehydrogenase (LDH and indirect bilirubin levels, and a positive Coombs test. The hematologic manifestations associated with infection, neoplasia and aplastic anemia were excluded. RESULTS: HI occurred in 14 patients (18.9%, with 15 admissions. Among these patients, 11 were female, 7 had trombocytopenic purpura, 5 AHA and 2 Evans syndrome. HI as onset and single manifestation of JSLE was observed in three patients. All the patients with trombocytopenic purpura presented cutaneous bleeding (petechia and/or ecchymosis. All had disease activity and simultaneously presented other manifestations of JSLE, particularly nephritis and vasculitis. Initially, all patients received pulsetherapy with methylprednisolone and prednisone later. In three patients the treatment aimed predominantly the control of hematologic manifestations, with intravenous gammaglobulin. The most used immunossupressive therapies were intravenous cyclophosphamide, cyclosporine and azathioprine. One patient died of central nervous system bleeding. No patient needed splenectomy. CONCLUSIONS: isolated HI was a severe manifestation in hospitalized patients with JSLE, generally

  1. Implementation of a real-time automatic onset time detection for surface electromyography measurement systems using NI myRIO

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    Lersviriyanantakul Chaiwat

    2016-01-01

    Full Text Available For using surface electromyography (sEMG in various applications, the process consists of three parts: an onset time detection for detecting the first point of movement signals, a feature extraction for extracting the signal attribution, and a feature classification for classifying the sEMG signals. The first and the most significant part that influences the accuracy of other parts is the onset time detection, particularly for automatic systems. In this paper, an automatic and simple algorithm for the real-time onset time detection is presented. There are two main processes in the proposed algorithm; a smoothing process for reducing the noise of the measured sEMG signals and an automatic threshold calculation process for determining the onset time. The results from the algorithm analysis demonstrate the performance of the proposed algorithm to detect the sEMG onset time in various smoothing-threshold equations. Our findings reveal that using a simple square integral (SSI as the smoothing-threshold equation with the given sEMG signals gives the best performance for the onset time detection. Additionally, our proposed algorithm is also implemented on a real hardware platform, namely NI myRIO. Using the real-time simulated sEMG data, the experimental results guarantee that the proposed algorithm can properly detect the onset time in the real-time manner.

  2. Alterações oftalmológicas decorrentes do tratamento do lúpus eritematoso sistêmico juvenil Ocular changes due to the treatment of juvenile systemic lupus erythematosus

    Directory of Open Access Journals (Sweden)

    Melissa Mariti Fraga

    2011-12-01

    Full Text Available OBJETIVO: Avaliar retrospectivamente as alterações oftalmológicas de crianças e adolescentes com lúpus eritematoso sistêmico juvenil (LESJ em um serviço de reumatologia pediátrica terciário. MÉTODOS: Avaliamos 117 pacientes com LESJ (85,5% do gênero feminino, 60,7% não caucasoides com média de idade de 10,4 anos e média de tempo de evolução da doença de 5,4 anos que preenchiam no mínimo quatro critérios de classificação do LES de acordo com o American College of Rheumatology de 1997. Aplicamos um protocolo que continha dados clínicos e demográficos, queixas e alterações oftalmológicas, idade do início, tempo de uso e dose cumulativa das medicações. RESULTADOS: Dos 117 pacientes, 24 (20,5% apresentaram alterações oftalmológicas. Destes, 16 apresentaram alteração de fundo de olho associada a hipertensão arterial sistêmica e/ou uso de cloroquina, quatro apresentaram catarata, dois apresentaram glaucoma e dois apresentaram catarata e glaucoma. A média de idade do aparecimento das alterações oftalmológicas foi de 14,1 anos. Os pacientes com alterações oftalmológicas receberam, estatisticamente, maiores doses e tempos de pulsoterapia de glicocorticoide em relação aos pacientes sem alterações oftalmológicas [1,5 (0,4-1,6 versus 1 (0,2-1,6 mg/kg, P = 0,003; 25,7 (2-99 versus 17,8 (1-114 meses, P = 0,0001; respectivamente]. CONCLUSÃO: Verificamos alta prevalência de alterações oftalmológicas relacionadas principalmente ao tratamento do LESJ, o que demonstra a necessidade de avaliações regulares mesmo em pacientes assintomáticos, visando ao diagnóstico e intervenção precoces e à diminuição da morbidade ocular relacionada a essa doença.OBJECTIVE: To assess retrospectively the ocular changes in children and adolescents with juvenile systemic lupus erythematosus (JSLE in a tertiary pediatric rheumatology service. METHODS: This study assessed 117 JSLE patients (85.5% female, 60.7% non

  3. A systematic review and meta-analysis of cutaneous manifestations in late- versus early-onset systemic lupus erythematosus.

    Science.gov (United States)

    Medlin, Jennifer L; Hansen, Karen E; Fitz, Sara R; Bartels, Christie M

    2016-06-01

    Although systemic lupus erythematosus (SLE) most commonly occurs in reproductive-age women, some are diagnosed after the age of 50. Recognizing that greater than one-third of SLE criteria are cutaneous, we undertook a systematic review and meta-analysis to evaluate differences in cutaneous manifestations in early- and late-onset SLE patients. We searched the literature using PubMed, CINAHL, Web of Science, and Cochrane Library. We excluded studies that did not include ACR SLE classification criteria, early-onset controls, that defined late-onset SLE as manifestations by age. Study heterogeneity was assessed using I(2). Overall, 35 studies, representing 11,189 early-onset and 1727 late-onset patients with SLE, met eligibility criteria. The female:male ratio was lower in the late-onset group (5:1 versus 8:1). Most cutaneous manifestations were less prevalent in the late-onset group. In particular, malar rash [OR = 0.43 (0.35, 0.52)], photosensitivity [OR = 0.72 (0.59, 0.88)], and livedo reticularis [OR = 0.33 (0.17, 0.64)] were less common in late-onset patients. In contrast, sicca symptoms were more common [OR = 2.45 (1.91, 3.14)]. The mean Newcastle Ottawa Quality Scale score was 6.3 ± 0.5 (scale: 0-9) with high inter-rater reliability for the score (0.96). Overall, cutaneous manifestations are less common in late-onset SLE patients, except sicca symptoms. Future studies should investigate etiologies for this phenomenon including roles of immune senescence, environment, gender, and immunogenetics. Copyright © 2016 Elsevier Inc. All rights reserved.

  4. Association of neopterin as a marker of immune system activation and juvenile rheumatoid arthritis activity

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    Mones M. Abu Shady

    2015-08-01

    Full Text Available OBJECTIVE: To evaluate neopterin plasma concentrations in patients with active juvenile idiopathic arthritis (JIA and correlate them with disease activity.METHODS: Sixty patients diagnosed as active JIA, as well as another 60 apparently healthy age- and gender-matched children as controls, were recruited from the Pediatrics Allergy and Immunology Clinic, Ain Shams University. Disease activity was assessed by the Juvenile Arthritis Disease Activity Score 27 (JADAS-27. Laboratory investigations were performed for all patients, including determination of hemoglobin concentration (Hgb, erythrocyte sedimentation rate (ESR, and C-reactive protein. Serum concentrations of tumor necrosis factor-alpha (TNF-a, interleukin-6 (IL-6, monocyte chemoattractant protein-1 (MCP-1, and neopterin were measured.RESULTS: Significant differences were found between JIA patients and controls with regard to the mean levels of Hgb, ESR, TNF-a, IL-6, and MCP-1 (p 0.05. Multiple linear regression analysis showed that JADAS- 27 and ESR were the main variables associated with serum neopterin in JIA patients (p < 0.05.CONCLUSION: The elevation of plasma neopterin concentrations in early JIA patients may indicate stimulation of immune response. Serum neopterin can be used as a sensitive marker for assaying background inflammation and disease activity score in JIA patients.

  5. Association of neopterin as a marker of immune system activation and juvenile rheumatoid arthritis activity.

    Science.gov (United States)

    Shady, Mones M Abu; Fathy, Hanan A; Ali, Alaa; Youness, Eman R; Fathy, Gihan A

    2015-01-01

    To evaluate neopterin plasma concentrations in patients with active juvenile idiopathic arthritis (JIA) and correlate them with disease activity. Sixty patients diagnosed as active JIA, as well as another 60 apparently healthy age- and gender-matched children as controls, were recruited from the Pediatrics Allergy and Immunology Clinic, Ain Shams University. Disease activity was assessed by the Juvenile Arthritis Disease Activity Score 27 (JADAS-27). Laboratory investigations were performed for all patients, including determination of hemoglobin concentration (Hgb), erythrocyte sedimentation rate (ESR), and C-reactive protein. Serum concentrations of tumor necrosis factor-alpha (TNF-α), interleukin-6 (IL-6), monocyte chemoattractant protein-1 (MCP-1), and neopterin were measured. Significant differences were found between JIA patients and controls with regard to the mean levels of Hgb, ESR, TNF-α, IL-6, and MCP-1 (p0.05). Multiple linear regression analysis showed that JADAS- 27 and ESR were the main variables associated with serum neopterin in JIA patients (p<0.05). The elevation of plasma neopterin concentrations in early JIA patients may indicate stimulation of immune response. Serum neopterin can be used as a sensitive marker for assaying background inflammation and disease activity score in JIA patients. Copyright © 2015 Sociedade Brasileira de Pediatria. Published by Elsevier Editora Ltda. All rights reserved.

  6. Bifurcation onset delay in magnetic bearing systems by time varying stiffness

    Science.gov (United States)

    Ghazavi, M. R.; Sun, Q.

    2017-06-01

    We study the nonlinear dynamics behaviours of a rigid rotor supported by magnetic bearings. In particular, we consider the effect of rotor unbalanced mass and geometric coupling. Existing works in literature have mostly focused on a single value of parameter or a smaller range of the nonlinearities introduced by rotor imbalance and geometric coupling. This is partly due to the use of a linear PD controller which limits the system performance. In this paper, we use a nonlinear PD controller by adopting a time varying stiffness term. The control gains are chosen according to the stability chart for a Mathieu's equation. Consequently, we observe a delay in the onset of bifurcation indicating an improved rotor performance.

  7. Detecting singular weak-dissipation limit for flutter onset in reversible systems

    Science.gov (United States)

    Bigoni, Davide; Misseroni, Diego; Tommasini, Mirko; Kirillov, Oleg N.; Noselli, Giovanni

    2018-02-01

    A "flutter machine" is introduced for the investigation of a singular interface between the classical and reversible Hopf bifurcations that is theoretically predicted to be generic in nonconservative reversible systems with vanishing dissipation. In particular, such a singular interface exists for the Pflüger viscoelastic column moving in a resistive medium, which is proven by means of the perturbation theory of multiple eigenvalues with the Jordan block. The laboratory setup, consisting of a cantilevered viscoelastic rod loaded by a positional force with nonzero curl produced by dry friction, demonstrates high sensitivity of the classical Hopf bifurcation onset to the ratio between the weak air drag and Kelvin-Voigt damping in the Pflüger column. Thus, the Whitney umbrella singularity is experimentally confirmed, responsible for discontinuities accompanying dissipation-induced instabilities in a broad range of physical contexts.

  8. Tocilizumab: The evidence for its place in the treatment of juvenile idiopathic arthritis

    DEFF Research Database (Denmark)

    Herlin, Troels

    2010-01-01

    INTRODUCTION: Juvenile idiopathic arthritis (JIA) is one of the most common chronic diseases with childhood onset. It comprises different subtypes of which the systemic onset subtype is often resistant to treatment. With the advent of biological treatment with tumor necrosis factor-alpha (TNFalpha......)-inhibitors, the clinical outcome of JIA has improved considerably, but only for subtypes other than systemic JIA. Substantial evidence shows that the proinflammatory cytokine interleukin-6 (IL-6) plays a pivotal role in systemic JIA. The blockage of IL-6 action by tocilizumab, a humanized anti-IL-6-receptor monoclonal...

  9. Comparison of ethnicity, gender, age of onset and outcome in South Africans with systemic lupus erythematosus.

    Science.gov (United States)

    Budhoo, A; Mody, G M; Dubula, T; Patel, N; Mody, P G

    2017-04-01

    Ethnicity, gender and age of onset are reported to influence the expression and outcome of systemic lupus erythematosus. We studied a multi-ethnic cohort of 408 South Africans (91.2% females) comprising 237 (58.1%) Indians, 137 (33.6%) African Blacks, 17 (4.2%) Mixed ethnicity and 17 (4.2%) Whites. The most common manifestations were arthritis (80.6%), photosensitivity (67.2%), oral ulcers (50.0%), malar rash (49.0%) and renal (39.2%). The common laboratory findings were positive anti-nuclear factor (96.8%), haematological (74.8%) and anti-dsDNA antibodies (45.3%). Serositis ( p = 0.002), nephritis ( p = 0.039), leucopaenia ( p = 0.001), haemolytic anaemia ( p = 0.026), anti-dsDNA antibodies ( p = 0.028) and anti-Sm antibodies ( p = 0.050) were more common in African Blacks compared to Indians. Males had increased prevalence of discoid rash ( p = 0.006) and anti-Sm antibodies ( p = 0.016). Discoid rash ( p = 0.018), renal involvement ( p lupus erythematosus. On multivariate analysis, the independent predictors of death were renal involvement, anti-dsDNA antibodies and seizures. There were 53 (13%) deaths and the five- and 10-year survival was 90.8% and 85.7% respectively, with no differences related to ethnicity or age of onset. In conclusion, we report on the spectrum and outcome of systemic lupus erythematosus in a large South African multi-ethnic cohort.

  10. Outcomes of 847 childhood-onset systemic lupus erythematosus patients in three age groups.

    Science.gov (United States)

    Lopes, S R M; Gormezano, N W S; Gomes, R C; Aikawa, N E; Pereira, R M R; Terreri, M T; Magalhães, C S; Ferreira, J C; Okuda, E M; Sakamoto, A P; Sallum, A M E; Appenzeller, S; Ferriani, V P L; Barbosa, C M; Lotufo, S; Jesus, A A; Andrade, L E C; Campos, L M A; Bonfá, E; Silva, C A

    2017-08-01

    Objective The objective of this study was to assess outcomes of childhood systemic lupus erythematosus (cSLE) in three different age groups evaluated at last visit: group A early-onset disease (Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SLICC/ACR-DI) (0 (0-9) vs 0 (0-6) vs 0 (0-7), p = 0.065) was comparable in the three groups. Further analysis of organ/system damage revealed that frequencies of neuropsychiatric (21% vs 10% vs 7%, p = 0.007), skin (10% vs 1% vs 3%, p = 0.002) and peripheral vascular involvements (5% vs 3% vs 0.3%, p = 0.008) were more often observed in group A compared to groups B and C. Frequencies of severe cumulative lupus manifestations such as nephritis, thrombocytopenia, and autoimmune hemolytic anemia were similar in all groups ( p > 0.05). Mortality rate was significantly higher in group A compared to groups B and C (15% vs 10% vs 6%, p = 0.028). Out of 69 deaths, 33/69 (48%) occurred within the first two years after diagnosis. Infections accounted for 54/69 (78%) of the deaths and 38/54 (70%) had concomitant disease activity. Conclusions This large multicenter study provided evidence that early-onset cSLE group had distinct outcomes. This group was characterized by higher mortality rate and neuropsychiatric/vascular/skin organ damage in spite of comparable frequencies of severe cumulative lupus manifestations. We also identified that overall death in cSLE patients was an early event mainly attributed to infection associated with disease activity.

  11. Psammomatoid juvenile ossifying fibroma of the jaws

    OpenAIRE

    Malathi, N; Radhika, T; Thamizhchelvan, H; Ravindran, C; Ramkumar, S; Giri, GVV; Gopal, Deepika

    2011-01-01

    Juvenile ossifying fibroma is an uncommon, benign, bone-forming neoplasm with aggressive local growth that is distinguished from other fibro-osseous lesions primarily by its age of onset, clinical presentation and aggressive behavior. Although a benign entity, juvenile ossifying fibroma is known to be locally aggressive and has a high tendency to recur. Two distinctive microscopic patterns have been described: A trabecular variant and a psammomatoid variant. This latter variant is predominant...

  12. Clinical and serological manifestations associated with interferon-α levels in childhood-onset systemic lupus erythematosus

    Directory of Open Access Journals (Sweden)

    Mariana Postal

    2012-01-01

    Full Text Available OBJECTIVE: To determine the serum levels of interferon alpha in childhood-onset systemic lupus erythematosus patients, their first-degree relatives and healthy controls and to evaluate the associations between serum interferon alpha and disease activity, laboratory findings and treatment features. METHODS: We screened consecutive childhood-onset systemic lupus erythematosus patients in a longitudinal cohort at the pediatric rheumatology unit of the State University of Campinas between 2009 and 2010. All patients demonstrated disease onset before the age of 16. Disease status was assessed according to the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI and Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI. Interferon alpha levels were measured using an enzyme-linked immunoabsorbent assay. RESULTS: We included 57 childhood-onset systemic lupus erythematosus patients (mean age 17.33±4.50, 64 firstdegree relatives (mean age 39.95±5.66, and 57 healthy (mean age 19.30±4.97 controls. Serum interferon alpha levels were significantly increased in childhood-onset systemic lupus erythematosus patients compared to their firstdegree relatives and healthy controls. Interferon alpha levels were significantly increased in patients with positive dsDNA antibodies, patients with cutaneous vasculitis, patients with new malar rash and patients who were not receiving medication. Interferon alpha levels correlated with C3 levels and systemic lupus erythematosus Disease Activity Index scores. In addition, we observed an inverse correlation between patient age and interferon alpha levels. CONCLUSION: Interferon alpha may play a role in the pathogenesis of childhood-onset systemic lupus erythematosus, especially in cutaneous manifestations and dsDNA antibody formation. The observation that interferon alpha levels are increased in patients who are not taking medication should be investigated in

  13. Macrophage Activation Syndrome as Onset of Systemic Lupus Erythematosus: A Case Report and a Review of the Literature

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    Guido Granata

    2015-01-01

    Full Text Available Macrophage activation syndrome (MAS is a potentially fatal condition. It belongs to the hemophagocytic lymphohistiocytosis group of diseases. In adults, MAS is rarely associated with systemic lupus erythematosus, but it also arises as complication of several systemic autoimmune disorders, like ankylosing spondylitis, rheumatoid arthritis, and adult-onset Still’s disease. Several treatment options for MAS have been reported in the literature, including a therapeutic regimen of etoposide, dexamethasone, and cyclosporine. Here we report a case of 42-year-old woman in whom MAS occurred as onset of systemic lupus erythematosus.

  14. Is late-onset OCD a distinct phenotype? Findings from a comparative analysis of "age at onset" groups.

    Science.gov (United States)

    Sharma, Eesha; Sundar, A Shyam; Thennarasu, Kandavel; Reddy, Y C Janardhan

    2015-10-01

    Significant differences in clinical profile and comorbidity patterns have been observed between "juvenile-onset" and "adult-onset" obsessive-compulsive disorder (OCD). There is little systematic research on onset of OCD after the fourth decade. The current study aims to compare the demographic, clinical, and comorbidity patterns of patients with "juvenile-onset" (OCD. Eight hundred two consecutive patients who consulted a specialty OCD clinic at a tertiary care hospital in India were evaluated with the Mini International Neuropsychiatric Interview, the Yale-Brown Obsessive-Compulsive Scale, and the Clinical Global Impression scale. 37.4%, 57.4%, and 5.2% of patients had juvenile-, adult-, and late-onset OCD, respectively. Late-onset OCD was associated with female gender (χ2=42, pOCD in first-degree relatives (χ2=20.4, pOCD was significantly associated with female gender, collecting compulsions, and less aggressive obsessions, in comparison with adult-onset OCD. In comparison with juvenile-onset, late-onset OCD was significantly associated with female gender, presence of precipitating factors, and less aggressive obsessions, sexual obsessions, and repeating compulsions. Late-onset OCD is characterized by female gender, lesser familial loading for OCD, and presence of precipitating factors, suggesting that it may have a distinct pathophysiology compared to juvenile- and adult-onset OCD. Systematic research is required to understand the family-genetic, neuropsychological, and neurobiological correlates of late-onset OCD.

  15. Clinical, laboratory and neuroimage findings in juvenile systemic lupus erythematosus presenting involvement of the nervous system Achados clínicos, laboratoriais e de imagem no lupus eritematoso sistêmico juvenil com comprometimento do sistema nervoso

    Directory of Open Access Journals (Sweden)

    Mônica Jaques Spinosa

    2007-06-01

    Full Text Available OBJECTIVE: To characterize neurological involvement in juvenile systemic lupus erythe-matosus. METHOD: The charts of all patients with the diagnosis of systemic lupus erythematosus before the age of 16 years, followed at the Rheumatology Unit of Pequeno Príncipe Hospital, from January 1992 to January 2006, were retrospectively reviewed, highlighting neuropsychiatric aspects. RESULTS: Forty-seven patients were included. Neuropsychiatric syndromes were found 29 (61.7%: seizures (17 / 36.2%, intractable headache (7 / 14.9%, mood disorders (5 / 10.6%, cerebrovascular disease (4 / 8.5%, acute confusional state (3 / 6.4%, aseptic meningitis (3 / 6.4%, psychosis (3 / 6.4%, chorea (3 / 6.4%, Guillain-Barré syndrome (2 / 4.3% and cranial neuropathy (1 / 2.1%. Morbidity indexes (SLEDAI and SLICC were higher among patients with neuropsychiatric manifestations (pOBJETIVO: Caracterizar o comprometimento neurológico no lupus eritematoso sistêmico juvenil. MÉTODO: Os prontuários dos pacientes com o diagnóstico de lupus eritematoso sistêmico antes dos 16 anos de idade, em acompanhamento na Unidade de Reumatologia do Hospital Pequeno Príncipe, de janeiro de 1992 a janeiro de 2006, foram revisados retrospectivamente enfatizando aspectos neuropsiquiátricos. RESULTADOS: Quarenta e sete pacientes foram incluídos. Síndromes neuropsiquiátricas foram encontradas em 29 (61,7%: crises convulsivas (17 / 36,2%, cefaléia intratável (7 / 14,9%, distúrbios do humor (5 / 10,6%, doença cerebrovascular (4 / 8,5%, estado confusional agudo (3 / 6,4%, meningite asséptica (3 / 6,4%, psicose (3 / 6,4%, coréia (3 / 6,4%, síndrome de Guillain-Barré (2 / 4,3% e neuropatia craniana (1 / 2,1%. Índices de morbidade (SEDAI e SLICC foram maiores em pacientes com manifestações neuropsiquiátricas (p<0,05. CONCLUSÃO: Síndromes neuropsiquiátricas são um achado freqüente que acrescenta morbidade significativa ao lupus eritematoso sistêmico juvenil.

  16. High disease activity: an independent factor for reduced immunogenicity of the pandemic influenza a vaccine in patients with juvenile systemic lupus erythematosus.

    Science.gov (United States)

    Campos, Lucia M A; Silva, Clovis A; Aikawa, Nadia E; Jesus, Adriana A; Moraes, Julio C B; Miraglia, Joao; Ishida, Maria A; Bueno, Cleonice; Pereira, Rosa M R; Bonfa, Eloisa

    2013-07-01

    Recent findings demonstrated a reduced immunogenicity of the influenza A H1N1/2009 vaccine in juvenile rheumatic diseases. However, a point of concern is whether the vaccine could induce disease flares. The aim of this study was to assess the disease safety of and the possible influence of disease parameters and therapy on nonadjuvant influenza A H1N1 vaccine response of juvenile systemic lupus erythematosus (SLE) patients. One hundred eighteen juvenile SLE patients and 102 healthy controls of a comparable age were vaccinated. Seroprotection rate, seroconversion rate, and factor increase in geometric mean titer (GMT) were calculated and effective immune response was defined by the Food and Drug Administration and the European Committee for Proprietary Medicinal Products vaccine immunologic standards. Disease parameters, treatment, and adverse events were evaluated. Age was comparable in juvenile SLE patients and controls (mean ± SD 16.0 ± 3.5 versus 15.9 ± 4.5 years; P = 0.26). Three weeks after immunization, seroprotection rate (73.7% versus 95.1%; P vaccine adverse events were observed. The present study demonstrated adequate disease safety and is the first to discriminate that high disease activity impairs influenza A H1N1/2009 vaccine antibody production in juvenile SLE, in spite of an overall immune response within recommended levels. Copyright © 2013 by the American College of Rheumatology.

  17. Features of renal dysfunction in children with systemic lupus erythematosus and juvenile idiopathic arthritis

    Directory of Open Access Journals (Sweden)

    L.F. Bogmat

    2017-09-01

    Full Text Available Background. In systemic autoimmune diseases of the connective tissue, kidney damage in some cases is a syndrome of the disease, and in others, it is a consequence of the underlying process. Objective: to establish the incidence and nature of violations of kidney function in children and adolescents with juvenile idiopathic arthritis (JIA and systemic lupus erythematosus (SLE, depending on the duration and activity of the disease. Materials and methods. 113 children aged 7 to 18 years were examined, of which 70 patients with JIA and 43 — with SLE. Distribution to the groups was carried out depending on the duration and activity of the disease. The status of kidney function was evaluated using hemorrhagic tests (serum creatinine level, determination of glomerular filtration rate. We have evaluated the level of proteinuria in the daily urine, as well as studied the concentration function of the kidneys by means of urine analysis according to Zimnytsky. Statistical processing was carried out using the statistical software package Statgra­phics 16.0. Results. Thus, in children with JIA, with the duration of the disease more than 3 years, there was a change in the renal function characterized as proteinuria, and a violation of glomerular filtration rate and a decrease in the concentration function of the kidneys. When studying the incidence of violations depending on the activity of the pathological process, it was established that in the group with 0 degree of activity, both glomerular filtration rate (25.0 % and concentration function of the kidneys (12.5 % decreased. In the group with I degree of activity, there was detected microproteinuria (4.4 %, violation of glomerular filtration rate (12.3 % and decreased renal concentration (6.6 %. In the group with II degree of activity, microproteinuria (14.2 % and a certain reduction in glomerular filtration rate (14.2 % were detected, and in the group with III degree of activity, almost all patients

  18. The family journey-to-diagnosis with systemic juvenile idiopathic arthritis: a cross-sectional study of the changing social media presence.

    Science.gov (United States)

    Modica, Renee F; Lomax, Kathleen Graham; Batzel, Pamela; Shapardanis, Leah; Katzer, Kimberly Compton; Elder, Melissa E

    2016-01-01

    Children with systemic juvenile idiopathic arthritis (SJIA) often encounter a delay between symptom onset and disease diagnosis, partly due to the broad differential of fever and lack of symptom recognition by providers. Families often seek multiple medical opinions and post on social media about their frustrations. This linguistic analysis observed the changing language patterns and social media posting behaviors of parents in the time leading to, during, and after SJIA diagnosis. Public social media sites were manually reviewed by a linguistic team to evaluate posts about SJIA from US-based parents. A total of 3,979 posts between July 2001 and January 2015 were reviewed from 108 sites. Pre-SJIA diagnosis parents sought answers and shared status updates on social media, focusing primarily on the following three site types: alternative/natural lifestyle forums (39%), Facebook (27%), and disease-specific forums (17%). Posts during early prediagnosis phases were characterized by expressive language showing confidence in health care providers and trust in parental instincts. At later prediagnosis stages, parents continued to use social media, but the posts demonstrated increased frustration with delays in diagnosis and gaps in communication with providers. More objective symptom descriptions and a greatly reduced child-centered emotional focus were observed as parents shifted into caregiving roles. Once the diagnosis of SJIA was confirmed, parents used straightforward, less expressive language, and Facebook (47%) to make "announcement" posts and increased their use of SJIA websites (30%). With treatment initiation, the posts demonstrated a slow return of expressive language and an increased parental understanding of the "new normal". Parents use different language styles, frames of reference, and websites before and after SJIA diagnosis. Gaps in parent-provider communication, especially before diagnosis, and their new roles as caregivers lead to parental use of social

  19. Childhood-onset systemic lupus erythematosus in Singapore: clinical phenotypes, disease activity, damage, and autoantibody profiles.

    Science.gov (United States)

    Tan, J H T; Hoh, S F; Win, M T M; Chan, Y H; Das, L; Arkachaisri, T

    2015-08-01

    Childhood-onset systemic lupus erythematosus (cSLE) is a multisystem autoimmune disease characterized by immune dysregulation affecting patients less than 18 years old. One-fifth of SLE cases are diagnosed during childhood. cSLE presents differently from adults and has a more severe and aggressive course. We describe the clinical and antibody profiles in our cSLE Singapore cohort. All cSLE patients who satisfied the 1997 American College of Rheumatology diagnostic criteria were captured in our lupus registry from January 2009 to January 2014. Data including demographic, cumulative clinical, serologic data, and damage indices were collected. Adjusted mean SLEDAI-2K (AMS) was used to summarize disease activity over multiple visits. Cluster analysis using non-hierarchical K-means procedure was performed on eight selected antibodies. The 64 patients (female:male ratio 5:1; Chinese 45.3%, Malay 28.1%, Indian 9.4%, and other races 17.2%) had a mean onset age of 11.5 years (range 2.1-16.7) and mean age at diagnosis was 11.9 years (range 2.6-18.0). Our study demonstrated differences in clinical manifestations for which hematologic involvement was the most common manifestation with less renal disease and uncommon neurologic manifestation as compared to other cSLE cohorts reported in our region. Antibody clusters were identified in our cohort but their clinical association/discrimination and outcome prediction required further validation study. Outcomes of our cohort in regard to disease activity after therapy and organ damages were comparable if not better to other cSLE cohorts elsewhere. Steroid-related damage, including symptomatic multifocal avascular necrosis and cataract, were not uncommon locally. Infection remains the major cause of death for the continent. Nevertheless, the five year survival rate of our cohort (98.4%) was high. © The Author(s) 2015.

  20. Juvenile systemic lupus erythematosus in a adolescent with acquired immunodeficiency syndrome

    OpenAIRE

    Sacilotto, Nathália de Carvalho; Yamashiro, Cintia Yukimi; Nishimoto, Teresa Maria Isaac

    2010-01-01

    O lúpus eritematoso sistêmico juvenil (LESJ) é uma doença inflamatória crônica, multissistêmica e autoimune. Algumas manifestações clínicas dessa condição são semelhantes às encontradas na síndrome da imunodeficiência adquirida (SIDA). A coexistência da SIDA com o LESJ é rara, especialmente na população pediátrica, sendo descritos na literatura pesquisada apenas cinco casos de pacientes com infecção congênita por HIV que desenvolveram essa enfermidade reumatológica, tendo como manifestação in...

  1. Thrombotic thrombocytopenic purpura at presentation of juvenile systemic lupus erythematosus patients

    OpenAIRE

    Campos, Lucia M. A.; Spadoni, Maria Silvia; Michelin, Cintia M.; Jesus, Adriana A.; Carneiro, Jorge D. A.; Silva, Clovis Artur Almeida da

    2013-01-01

    Púrpura trombocitopênica trombótica (PTT) é uma alteração hematológica rara e com risco de morte, caracterizada por trombocitopenia, anemia hemolítica microangiopática e alterações neurológicas e/ou renais. A PTT foi descrita em raros pacientes com lúpus eritematoso sistêmico juvenil (LESJ) e, até onde se sabe, a prevalência dessa manifestação em uma população de lúpus pediátrico ainda não foi estudada. Assim, entre janeiro de 1983 e dezembro de 2010, revisamos os prontuários de 5.508 pacient...

  2. Systemic lupus erythaematosus in a multiethnic US cohort (LUMINA) LIII: disease expression and outcome in acute onset lupus.

    Science.gov (United States)

    Bertoli, A M; Vilá, L M; Reveille, J D; Alarcón, G S

    2008-04-01

    To determine the features associated with acute onset systemic lupus erythaematosus (SLE). A total of 631 SLE patients from LUMINA (for "lupus in minority populations: nature vs nurture"), a multiethnic (Hispanics, African-Americans and Caucasians) cohort, were studied. Acute disease onset was defined as the accrual of > or = 4 American College of Rheumatology (ACR) criteria for the classification of SLE in < or = 4 weeks. Socioeconomic demographic features, clinical manifestations, disease activity, damage accrual, mortality, autoantibodies, HLA class II and FCGR alleles, behavioural/psychological variables were compared between patients with acute and insidious disease onset by univariable (chi(2) and Student t test) and multivariable (stepwise logistic regression) analyses. A total of 94 (15%) patients had acute disease onset. In the multivariable analysis, patients with acute onset lupus had more renal involvement (odds ratio (OR) = 1.845, 95% CI 1.076-3.162; p = 0.026) and higher disease activity (OR = 1.057, 95% CI 1.005-1.112; p = 0.030). By contrast, age (OR = 0.976, 95% CI 0.956-0.997; p = 0.025), education (OR = 0.901, 95% CI 0.827-0.983, p = 0.019), health insurance (OR = 0.423, 95% CI 0.249-0.718; p = 0.001) and skin involvement (OR = 0.346, 95% CI 0.142-0.843; p = 0.019) were negatively associated with acute onset lupus. No differences were found regarding the serological, genetic and behavioural/psychological features; this was also the case for damage accrual and mortality. Patients with acute onset lupus seem to be younger, have a lower socio-economic status and display more severe disease in terms of clinical manifestations and disease activity. However, intermediate (damage) and long-term (mortality) outcomes appear not to be influenced by the type of disease onset in SLE.

  3. Juvenile rheumatoid arthritis

    Science.gov (United States)

    ... joints. This form of JIA may turn into rheumatoid arthritis. It may involve 5 or more large and ... no known prevention for JIA. Alternative Names Juvenile rheumatoid arthritis (JRA); Juvenile chronic polyarthritis; Still disease; Juvenile spondyloarthritis ...

  4. A Pediatric Case of Systemic Lupus Erythematosus Developed 10 Years after Cord Blood Transplantation for Juvenile Myelomonocytic Leukemia

    Directory of Open Access Journals (Sweden)

    Masayuki Nagasawa

    2012-01-01

    Full Text Available Allogeneic hematopoietic stem cell transplantation (allo-HSCT is a most powerful immunotherapy for hematological malignancies. However, the impact of immunological disturbances as a result of allo-HSCT is not understood well. We experienced an 11-year-old boy who presented with systemic lupus erythemathosus (SLE 10 years after unrelated cord blood transplantation of male origin for juvenile myelomonocytic leukemia (JMML with monosomy 7. Bone marrow examination showed complete remission without monosomy 7. Genetic analysis of peripheral blood revealed mixed chimera with recipient cells consisting of <5% of T cells, 50–60% of B cells, 60–75% of NK cells, 70–80% of macrophages, and 50–60% of granulocytes. Significance of persistent mixed chimera as a cause of SLE is discussed.

  5. EVALUATION OF THICKNESS OF INTIMA-MEDIA COMPLEX OF COMMON CAROTID ARTERIES IN CHILDREN WITH JUVENILE ARTHRITIS AND SYSTEMIC LUPUS ERYTHEMATOSUS

    Directory of Open Access Journals (Sweden)

    A.B. Sugak

    2010-01-01

    Full Text Available Rheumatic diseases in adults are associated with accelerated atherosclerosis, and its early signs can be stated by the thickening of intima-media complex of common carotid arteries (CCA. This symptom is detected during ultrasound examination in 49% of children with systemic lupus erythematosus, in 24% of patients with juvenile rheumatoid arthritis and in 13% of children with juvenile spondylarthritis. Besides, 36% of children with systemic lupus erythematosus and 17% — with systemic type of juvenile rheumatoid arthritis had structure changes of CCA wall. A dependence of these disorders on cholesterol and glucose levels in blood serum, overweight and Cushing syndrome, age, duration and activity of a disease, levels of ESR, C-reactive protein and white blood cells was not showed. Authors detected a correlation between the thickness of intima-media complex of CCA and hemostasis parameters.Key words: children, juvenile arthritis, systemic lupus erythematosus, intima-media complex, ultrasound diagnostics.(Voprosy sovremennoi pediatrii — Current Pediatrics. 2010;9(2:64-69

  6. Iatrogenic effect of juvenile justice.

    Science.gov (United States)

    Gatti, Uberto; Tremblay, Richard E; Vitaro, Frank

    2009-08-01

    The present study uses data from a community sample of 779 low-SES boys to investigate whether intervention by the juvenile justice system is determined, at least in part, by particular individual, familial and social conditions, and whether intervention by the juvenile courts during adolescence increases involvement in adult crime. The study considers self-reported crime in childhood and adolescence, and introduces individual, familial and social variables into its analysis. The results show that youths who are poor, impulsive, poorly supervised by their parents, and exposed to deviant friends are more likely, for the same degree of antisocial behavior, to undergo intervention by the Juvenile Court, and that this intervention greatly increases the likelihood of involvement with the penal system in adulthood. The results also show that the various measures recommended by the Juvenile Court exert a differential criminogenic effect; those that involve placement have the most negative impact.

  7. Dermatomiositis juvenil

    OpenAIRE

    Goldaracena, Pablo; Pérez, Federico

    2008-01-01

    La dermatomiositis juvenil (DMJ) es una enfermedad multi sistémica de etiología desconocida, caracterizada por una vasculitis que ocasiona una inflamación no supurativa del músculo estriado y lesiones cutáneas distintivas. La cobertura de los criterios de Bohan y Peter establece el diagnóstico: exantema patognomónico junto a debilidad muscular proximal simétrica, elevación sérica de enzimas musculares, s...

  8. Effects of juvenile host density and food availability on adult immune response, parasite resistance and virulence in a Daphnia-parasite system.

    Directory of Open Access Journals (Sweden)

    Corine N Schoebel

    Full Text Available Host density can increase infection rates and reduce host fitness as increasing population density enhances the risk of becoming infected either through increased encounter rate or because host condition may decline. Conceivably, potential hosts could take high host density as a cue to up-regulate their defence systems. However, as host density usually covaries with food availability, it is difficult to examine the importance of host density in isolation. Thus, we performed two full-factorial experiments that varied juvenile densities of Daphnia magna (a freshwater crustacean and food availability independently. We also included a simulated high-density treatment, where juvenile experimental animals were kept in filtered media that previously maintained Daphnia at high-density. Upon reaching adulthood, we exposed the Daphnia to their sterilizing bacterial parasite, Pasteuria ramosa, and examined how the juvenile treatments influenced the likelihood and severity of infection (Experiment I and host immune investment (Experiment II. Neither juvenile density nor food treatments affected the likelihood of infection; however, well-fed hosts that were well-fed as juveniles produced more offspring prior to sterilization than their less well-fed counterparts. By contrast, parasite growth was independent of host juvenile resources or host density. Parasite-exposed hosts had a greater number of circulating haemocytes than controls (i.e., there was a cellular immune response, but the magnitude of immune response was not mediated by food availability or host density. These results suggest that density dependent effects on disease arise primarily through correlated changes in food availability: low food could limit parasitism and potentially curtail epidemics by reducing both the host's and parasite's reproduction as both depend on the same food.

  9. Hypoxia tolerance and antioxidant defense system of juvenile jumbo squids in oxygen minimum zones

    Science.gov (United States)

    Trübenbach, Katja; Teixeira, Tatiana; Diniz, Mário; Rosa, Rui

    2013-10-01

    Jumbo squid (Dosidicus gigas) is a large oceanic squid endemic off the Eastern Tropical Pacific that undertakes diel vertical migrations into mesopelagic oxygen minimum zones. One of the expected physiological effects of such migration is the generation of reactive oxygen species (ROS) at the surface, promoted by the transition between hypoxia and reoxygenation states. The aim of this study was to investigate the energy expenditure rates and the antioxidant stress strategies of juvenile D. gigas under normoxia and hypoxia, namely by quantifying oxygen consumption rates, antioxidant enzyme activities [including superoxide dismutase (SOD), catalase (CAT) and glutathione-S-transferase (GST)], heat shock protein expression (Hsp70/Hsc70), and lipid peroxidation [malondialdehyde (MDA) levels]. A high significant decrease (68%) in squid's metabolic rates was observed during hypoxia (p0.05), with the latter indicating no enhancement of lipid peroxidation (i.e. cellular damage) at the warmer and normoxic surface waters. The understanding of such physiological strategies that are linked to oxygen deprivation and reoxygenation phases may provide valuable information about how this species is quickly responding to the impacts of environmental stressors coupled with global climate change.

  10. Update on the pathogenesis and treatment of childhood-onset systemic lupus erythematosus.

    Science.gov (United States)

    Couture, Julie; Silverman, Earl D

    2016-09-01

    This article will provide an update of studies published in the last year regarding epidemiology, pathogenesis, major disease manifestations and outcomes, and therapies in childhood-onset systemic lupus erythematosus (cSLE). Recent studies on cSLE epidemiology supported previous findings that cSLE patients have more severe disease and tend to accumulate damage rapidly. Lupus nephritis remains frequent and is still a significant cause of morbidity and mortality. In the past year unfortunately there were no new reproducible, biomarker studies to help direct therapy of renal disease. However, some progress was made in neuropsychiatric disease assessment, with a new and promising automated test to screen for cognitive dysfunction reported. There were no prospective interventional treatment trials designed for patients with cSLE published in the last year, but some studies involving children are currently active and might improve the therapeutic options for patients with cSLE. There is a need to get a better understanding of pathogenesis and identify new biomarkers in cSLE to more accurately predict outcomes. New insights into characterization of different clinical manifestations may enable to optimize individual interventions and influence the prognosis.

  11. Herpes zoster infection in childhood-onset systemic lupus erythematosus patients: a large multicenter study.

    Science.gov (United States)

    Ferreira, J C O A; Marques, H H; Ferriani, M P L; Gormezano, N W S; Terreri, M T; Pereira, R M; Magalhães, C S; Campos, L M; Bugni, V; Okuda, E M; Marini, R; Pileggi, G S; Barbosa, C M; Bonfá, E; Silva, C A

    2016-06-01

    The aim of this multicenter study in a large childhood-onset systemic lupus erythematosus (cSLE) population was to assess the herpes zoster infection (HZI) prevalence, demographic data, clinical manifestations, laboratory findings, treatment, and outcome. A retrospective multicenter cohort study (Brazilian cSLE group) was performed in ten Pediatric Rheumatology services in São Paulo State, Brazil, and included 852 cSLE patients. HZI was defined according to the presence of acute vesicular-bullous lesions on erythematous/edematous base, in a dermatomal distribution. Post-herpetic neuralgia was defined as persistent pain after one month of resolution of lesions in the same dermatome. Patients were divided in two groups for the assessment of current lupus manifestations, laboratory findings, and treatment: patients with HZI (evaluated at the first HZI) and patients without HZI (evaluated at the last visit). The frequency of HZI in cSLE patients was 120/852 (14%). Hospitalization occurred in 73 (61%) and overlap bacterial infection in 16 (13%). Intravenous or oral aciclovir was administered in 113/120 (94%) cSLE patients at HZI diagnosis. None of them had ophthalmic complication or death. Post-herpetic neuralgia occurred in 6/120 (5%). After Holm-Bonferroni correction for multiple comparisons, disease duration (1.58 vs 4.41 years, p Nephritis (37% vs 18%, p treatment seem to be major factors underlying this complication in spite of a benign course. © The Author(s) 2016.

  12. Initial digital vasculitis in a large multicenter cohort of childhood-onset systemic lupus erythematosus

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    Ana Paula Sakamoto

    Full Text Available Abstract Objectives: To assess clinical digital vasculitis (DV as an initial manifestation of childhood-onset systemic lupus erythematosus (cSLE within a large population. Methods: Multicenter cross-sectional study including 852 cSLE patients (ACR criteria followed in ten Pediatric Rheumatology centers in São Paulo State, Brazil. Results: DV was observed in 25/852 (3% cSLE patients. Periungual hemorrhage was diagnosed in 12 (48%, periungual infarction in 7 (28%, tip finger ulceration in 4 (16%, painful nodules in 1 (4% and gangrene in 1 (4%. A poor outcome, with digital resorption, occurred in 5 (20%. Comparison of patients with and without DV revealed higher frequency of malar rash (80% vs. 53%, p = 0.008, discoid rash (16% vs. 4%, p = 0.017, photosensitivity (76% vs. 45%, p = 0.002 and other cutaneous vasculitides (80% vs. 19%, p 0.05. SLEDAI-2K median, DV descriptor excluded, was significantly lower in patients with DV compared to those without this manifestation [10 (0-28 vs. 14 (0-58, p = 0.004]. Visceral vasculitis or death were not observed in this cSLE cohort. The frequency of cyclophosphamide use (0% vs. 18%, p = 0.014 was significantly lower in the DV group. Conclusion: Our large multicenter study identified clinical DV as one of the rare initial manifestation of active cSLE associated with a mild multisystemic disease, in spite of digital resorption in some of these patients.

  13. Fighting Juvenile Gun Violence. Juvenile Justice Bulletin.

    Science.gov (United States)

    Sheppard, David; Grant, Heath; Rowe, Wendy; Jacobs, Nancy

    This bulletin describes the Office of Juvenile Justice and Delinquency Prevention's efforts to fight juvenile gun violence. The Office awarded four community demonstration grants to implement "Partnerships To Reduce Juvenile Gun Violence." Partnership goals include increasing the effectiveness of existing strategies by enhancing and…

  14. Seizure Onset Detection based on a Uni- or Multi-modal Intelligent Seizure Acquisition (UISA/MISA) System

    DEFF Research Database (Denmark)

    Conradsen, Isa; Beniczky, Sándor; Wolf, Peter

    2010-01-01

    An automatic Uni- or Multi-modal Inteligent Seizure Acquisition (UISA/MISA) system is highly applicable for onset detection of epileptic seizures based on motion data. The modalities used are surface electromyography (sEMG), acceleration (ACC) and angular velocity (ANG). The new proposed automatic...

  15. European evidence-based recommendations for diagnosis and treatment of childhood-onset systemic lupus erythematosus : The SHARE initiative

    NARCIS (Netherlands)

    Groot, Noortje; De Graeff, Nienke; Avcin, Tadej; Bader-Meunier, Brigitte; Brogan, Paul A.; Dolezalova, Pavla; Feldman, Brian M.; Kone-Paut, Isabelle; Lahdenne, Pekka; Marks, Stephen D; McCann, Liza J.; Ozen, Seza; Pilkington, Clarissa; Ravelli, Angelo; Royen-Kerkhof, Annet Van; Uziel, Yosef; Vastert, Bas; Wulffraat, Nico; Kamphuis, Sylvia; Beresford, Michael W.

    2017-01-01

    Childhood-onset systemic lupus erythematosus (cSLE) is a rare, multisystem and potentially life-Threatening autoimmune disorder with significant associated morbidity. Evidence-based guidelines are sparse and management is often based on clinical expertise. SHARE (Single Hub and Access point for

  16. Responses of earthworms (Lumbricus rubellus) to copper and cadmium as determined by measurement of juvenile traits in a specifically designed test system.

    Science.gov (United States)

    Spurgeon, D J; Svendsen, C; Kille, P; Morgan, A J; Weeks, J M

    2004-01-01

    In this study, the effects of two metals, copper and cadmium, on the growth and development of juvenile Lumbricus rubellus were measured in a toxicity test in which individuals were grown in isolation. This design had a number of advantages over traditional test systems for earthworms. Importantly, the test is specifically designed to measure two juvenile traits (survival over and length of the juvenile period) that have been shown to have a high sensitivity for determining population growth rate. The test system also maximizes replication, while allowing time-series-based monitoring of individual growth. For both metals, significant exposure-dependent effects on survival, growth, development time, and (less certainly) maturation weight were observed. Comparisons of the relative toxicity of the two metals indicated different concentration-response relationships. For copper, hormesis was found at low levels, while only at the highest soil concentration tested (10.07 micromol g(-1)) were (severe) toxic effects present. For cadmium, hormesis was also evident at the lowest concentration tested; however, at soil levels above this, a graded concentration-dependent toxic effect was apparent. These differences in the exposure response patterns can be (tentatively) explained in terms of the mechanisms for handling copper (an essential metal for earthworms) and cadmium (a putative nonessential element). The applicability of the test for routine measurement of chemical effects on ecologically relevant juvenile traits is also outlined and future developments are discussed.

  17. Osteonecrose de mandíbula em pacientes com lúpus eritematoso sistêmico juvenil observada em exame de imagem Osteonecrosis of the jaw on imaging exams of patients with juvenile systemic lupus erythematosus

    Directory of Open Access Journals (Sweden)

    Elisabeth Gonzaga Canova Fernandes

    2010-02-01

    joint (TMJ in patients with juvenile systemic lupus erythematosus (JSLE and a control group. PATIENTS AND METHODS: Panoramic radiographies of the TMJ of 26 JSLE patients and 28 healthy individuals were evaluated. Multislice computed tomography (MCT was performed on those patients who presented flattening and/or destruction of mandibular condyles. Demographic data, oral health indices, clinical manifestations, laboratorial exams, and treatment were evaluated. RESULTS: Important radiographic changes consistent with osteonecrosis of the mandible, confirmed by MCT of the TMJ, were observed in 2/26 (8% JSLE patients versus 0% in the control group (P = 0.22. Mild clinical dysfunction and abnormal TMJ mobility were observed in 67% and 54% of the patients, respectively. Age of onset, disease duration, and current age were similar in JSLE patients with and without severe radiographic changes of TMJ (9.3 versus 10.8 years, P = 0.77; 3.3 versus 2 years, P = 0.63; 12.6 versus 13.5 years, P = 0.74, respectively. Significant differences in gender, socioeconomical status, oral health indices, clinical manifestations, laboratorial exams, and treatment were not observed between both subgroups (P 0.05. Both JSLE patients with osteonecrosis of the mandible had active chronic disease, used corticosteroids for a prolonged period, and had mild TMJ dysfunction. Antiphospholipid antibodies were not observed in those two patients, and neither one had been treated with bisphosphonate. CONCLUSIONS: Osteonecrosis of the mandible with mild TMJ dysfunction was observed in some of the patients, demonstrating the importance of odontological assessment during clinical follow-up.

  18. Risk Factors for Symptomatic Avascular Necrosis in Childhood-onset Systemic Lupus Erythematosus.

    Science.gov (United States)

    Yang, Yelin; Kumar, Sathish; Lim, Lily Siok Hoon; Silverman, Earl D; Levy, Deborah M

    2015-12-01

    To examine the frequency and risk factors for symptomatic avascular necrosis (AVN) in childhood-onset systemic lupus erythematosus (cSLE). A single-center, nested, matched, case-control design was used. There were 617 patients with cSLE followed at the Hospital for Sick Children (SickKids) Lupus Clinic between July 1982 and June 2013 included in the study. The AVN cohort consisted of 37 patients identified with clinical findings of symptomatic AVN and diagnosis was confirmed by 1 or more imaging modalities. Three controls were matched to each patient with AVN by date and age at diagnosis. Baseline clinical, laboratory, and treatment characteristics were compared between patients with AVN and controls by univariable analyses and if statistically significant, were included in a multivariable logistic regression model. A total of 37/617 patients (6%) developed symptomatic AVN in 91 joints during followup at SickKids. The mean duration to disease was 2.3 years. The hip was the most commonly involved joint (26/37, 70%). Compared with the matched non-AVN cohort, patients with AVN had a higher incidence of central nervous system (CNS) involvement and nephritis, required greater cumulative prednisone (PRED) from cSLE diagnosis to AVN, received a greater maximal daily PRED dose, and had more frequent use of pulse methylprednisolone therapy. Multivariable regression analysis confirmed major organ involvement (CNS disease and/or nephritis) and maximal daily PRED dose as significant predictors of symptomatic AVN development. Patients with cSLE with severe organ involvement including nephritis and CNS disease and higher maximal daily dose of PRED are more likely to develop symptomatic AVN.

  19. S100β is associated with cognitive impairment in childhood-onset systemic lupus erythematosus patients.

    Science.gov (United States)

    Lapa, A T; Postal, M; Sinicato, N A; Bellini, B S; Fernandes, P T; Marini, R; Appenzeller, S

    2017-04-01

    Objective To investigate serologic S100β protein levels in childhood-onset SLE patients (cSLE) and to elucidate their association with disease activity and neuropsychiatric (NP) manifestations. Methods We included 71 cSLE patients (67 females; median age 18 years; range 9-37 and 53 (47 females; median age of 20 years; range 6-29) age and sex matched healthy controls. Neurological manifestations were analysed according to the American College of Rheumatology (ACR) criteria. Cognitive evaluation was performed in all participants using Wechsler Intelligence Scale for Children (WISC-III) and Wechsler Adult Intelligence Scale (WAIS), according to age, and validated in Portuguese. SLE patients were further assessed for clinical and laboratory SLE manifestations, disease activity (SLE Disease Activity Index (SLEDAI)), damage (Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI)) and current drug exposures. Sera S100β protein levels were measured by enzyme-linked immunosorbent assay using commercial kits. Results The median S100β protein level was 116.55 pg/mL (range 1.53-468.50) in cSLE and 54.98 pg/mL (range 0.69-181.00) in healthy controls ( p manifestations ( p = 0.03). The S100β protein levels was associated with cognitive impairment in cSLE patients ( p = 0.006). Conclusions S100β protein levels are increased in cSLE with cognitive impairment. S100β may be considered a potential biomarker that underlies central nervous system (CNS) dysfunction, especially cognitive impairment.

  20. Evaluation of dietary soybean meal as fish meal replacer for juvenile whiteleg shrimp, Litopenaeus vannamei reared in biofloc system

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    Hyeonho Yun

    2017-01-01

    Full Text Available Abstract Different levels of dietary soybean meal (SBM as a fish meal (FM replacer, with and without amino acid supplementation, for whiteleg shrimp, Litopenaeus vannamei reared in the biofloc system was examined in eight weeks of feeding trial. Eight experimental diets consisted of a basal diet with 0% FM replacement by SBM provided in clear sea water without biofloc system (S0SW, four diets replacing FM at 0% (S0, 33% (S33, 67% (S67 and 100% (S100 by SBM, and three diets replacing FM at 33% (S33A, 67% (S67A and 100% (S100A by SBM supplemented with amino acids (methionine and lysine in the seawater biofloc system. Results of water quality analyses showed significantly lower total suspended solids and nitrate for S0SW group than all other treatments. Diets S0 and S33A resulted in higher weight gain and specific growth rate among all groups, with no significant differences with S33 group. In addition, whole-body protein and amino acid compositions of shrimp fed S0SW were lower than most biofloc groups. Haemolymph parameters showed significant differences in total protein, cholesterol and triglyceride between groups S0 and S0SW. Also, superoxide dismutase activity showed a decreasing trend with increasing replacement level. In conclusion, based on these results, SBM could replace up to 33% of FM with or without amino acid supplementation in juvenile whiteleg shrimp diets reared in the biofloc system.

  1. Cultivable intestinal microbiota of yellowtail juveniles (Seriola lalandi in an aquaculture system

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    Eduardo Aguilera

    2013-07-01

    Full Text Available The yellowtail (Seriola lalandi has been farmed for many years and is becoming a promising aquaculture species. Knowledge of the intestinal microbiota of this species is very limited. Thus, the aim of this study is to describe the bacterial populations associated with the intestinal tract of Seriola lalandi reared in Chile. The microbiota composition was analyzed at two growth stages distinguished by weight and parameters such as Specific Growth Rate (SGR and Feed Conversion Ratio (FCR. Juveniles (mean initial weight 7.33 ± 0.30 g and pre-adults (81.7 ± 19.0 g were fed with commercial diet for 33 and 50 days, respectively. The first intestinal samples were collected at the end of Trial 1 from specimens weighing approximately 50 g while the second samples were obtained at the end of Trial 2 from specimens weighing approximately 370 g. The microbiota composition was examined using conventional isolation in Tryptic Soy Agar (TSA followed by 16S rRNA sequencing and identification. In total, 16 genera were identified. Pseudomonas, Vibrio and Staphylococcus were the predominant genera in fish at the 50 g stage, whereas Microbacterium and Francisella were the predominant genera in the 370 g stage. The microbiota composition showed different assemblages, depending on host size, with Bacillus and Vibrio being the only genera that were shared. Knowledge of the intestinal microbiota of Seriola lalandi is the first step in the exploration of microbiota management and the development of probiotics, as well as in the identification of the bacterial populations in healthy fish under cultured conditions.

  2. The influence of intrauterine exposure to immunosuppressive treatment on changes in the immune system in juvenile Wistar rats

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    Kabat-Koperska J

    2016-07-01

    Full Text Available Joanna Kabat-Koperska,1 Agnieszka Kolasa-Wołosiuk,2 Bartosz Wojciuk,3 Iwona Wojciechowska-Koszko,3 Paulina Roszkowska,3 Barbara Krasnodębska-Szponder,3 Edyta Paczkowska,4 Krzysztof Safranow,5 Edyta Gołembiewska,1 Bogusław Machaliński,4 Kazimierz Ciechanowski1 1Department of Nephrology, Transplantology and Internal Medicine, 2Department of Histology and Embryology, 3Department of Microbiology and Immunological Diagnostics, 4Department of General Pathology, 5Department of Biochemistry and Medical Chemistry, Pomeranian Medical University, Szczecin, Poland Background: In our study, we assessed the impact of immunosuppressive drug combinations on changes in the immune system of juvenile Wistar rats exposed to these drugs during pregnancy. We primarily concentrated on changes in two organs of the immune system – the thymus and the spleen. Methods: The study was conducted on 40 (32+8 female Wistar rats administered full and half dose of drugs, respectively, subjected to regimens commonly used in therapy of human kidney transplant recipients ([1] cyclosporine A, mycophenolate mofetil, and prednisone; [2] tacrolimus, mycophenolate mofetil, and prednisone; [3] cyclosporine A, everolimus, and prednisone. The animals received drugs by oral gavage 2 weeks before pregnancy and during 3 weeks of pregnancy. Results: There were no statistically significant differences in the weight of the thymus and spleen, but changes were found in the results of blood hematology, cytometry from the spleen, and a histologic examination of the examined immune organs of juvenile Wistar rats. In the cytokine assay, changes in the level of interleukine 17 (IL-17 after increasing amounts of concanavaline A were dose-dependent; the increase of IL-17 was blocked after administration of higher doses of immunosuppressive drugs. However, after a reduction of doses, its increase resumed. Conclusion: Qualitative, quantitative, and morphological changes in the immune system of infant

  3. Optimization of Concurrent Deployments of the Juvenile Salmon Acoustic Telemetry System and Other Hydroacoustic Equipment at John Day Dam

    Energy Technology Data Exchange (ETDEWEB)

    Ploskey, Gene R.; Hughes, James S.; Khan, Fenton; Kim, Jina; Lamarche, Brian L.; Johnson, Gary E.; Choi, Eric Y.; Faber, Derrek M.; Wilberding, Matthew C.; Deng, Zhiqun; Weiland, Mark A.; Zimmerman, Shon A.; Fischer, Eric S.; Cushing, Aaron W.

    2008-09-01

    The purpose of this report is to document the results of the acoustic optimization study conducted at John Day Dam during January and February 2008. The goal of the study was to optimize performance of the Juvenile Salmon Acoustic Telemetry System (JSATS) by determining deployment and data acquisition methods to minimize electrical and acoustic interference from various other acoustic sampling devices. Thereby, this would allow concurrent sampling by active and passive acoustic methods during the formal evaluations of the prototype surface flow outlets at the dam during spring and summer outmigration seasons for juvenile salmonids. The objectives for the optimization study at John Day Dam were to: 1. Design and test prototypes and provide a total needs list of pipes and trolleys to deploy JSATS hydrophones on the forebay face of the powerhouse and spillway. 2. Assess the effect on mean percentage decoded of JSATS transmissions from tags arrayed in the forebay and detected on the hydrophones by comparing: turbine unit OFF vs. ON; spill bay OPEN vs. CLOSED; dual frequency identification sonar (DIDSON) and acoustic Doppler current profiler (ADCP) both OFF vs. ON at a spill bay; and, fixed-aspect hydroacoustic system OFF vs. ON at a turbine unit and a spill bay. 3. Determine the relationship between fixed-aspect hydroacoustic transmit level and mean percentage of JSATS transmissions decoded. The general approach was to use hydrophones to listen for transmissions from JSATS tags deployed in vertical arrays in a series perpendicular to the face of the dam. We used acoustic telemetry equipment manufactured by Technologic and Sonic Concepts. In addition, we assessed old and new JSATS signal detectors and decoders and two different types of hydrophone baffling. The optimization study consisted of a suite of off/on tests. The primary response variable was mean percentage of tag transmissions decoded. We found that there was no appreciable adverse effect on mean percentage

  4. Trends in nitrogen isotope ratios of juvenile winter flounder reflect changing nitrogen inputs to Rhode Island, USA estuarine systems.

    Science.gov (United States)

    Pruell, Richard J; Taplin, Bryan K; Miller, Kenneth M

    2017-05-15

    Nitrogen isotope ratios (δ 15 N) in juvenile winter flounder, Pseudopleuronectes americanus, were used to examine changes in nitrogen inputs to several Rhode Island, USA estuarine systems. Fish were collected over two three-year periods with a ten-year interval between sampling periods (2002-2004 and 2012-2014). During that interval numerous changes to nutrient management practices were initiated in the watersheds of these estuarine systems including the upgrade of several major wastewater treatment facilities that discharge to Narragansett Bay, which significantly reduced nitrogen inputs. Following these reductions, the δ 15 N values of flounder in several of the systems decreased as expected; however, isotope ratios in fish from upper Narragansett Bay significantly increased. We believe that low δ 15 N values measured in 2002-2004 were related to concentration-dependent fractionation at this location. Increased δ 15 N values measured between 2012 and 2014 may indicate reduced fractionation or that changes in wastewater treatment processes altered the nitrogen isotopic ratios of the effluents. Published by Elsevier Ltd.

  5. Experience in the use of tocilizumab in patient with systemic juvenile idiopathic arthritis and type 1 diabetes

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    E. A. Ligostaeva

    2016-01-01

    Full Text Available The article describes the experience of using tocilizumab in a patient with systemic juvenile idiopathic arthritis (JIA refractory to therapy with classical immunosuppressants, coupled with type 1 diabetes. Already after the first injection of tocilizumab there was a decrease in the severity of the disease’s systemic manifestations, pain in the affected joints and functional disorders. A 30% improvement by JADAS index, ACRPedi was reached. By the 8th week of therapy, proliferative changes in wrist joints and arthralgias reduced and the duration of morning stiffness decreased. After 3 months, JIA’s activity decreased (DAS 28 scale; erythrocyte sedimentation rate and serum concentrations of C-reactive protein decreased; hemoglobin concentration and the number of erythrocytes increased. After 6 months of treatment, clinical and laboratory remission (DAS 28 < 2.6 started. On the background of tocilizumab treatment, systemic manifestations of the disease stopped, laboratory and articular JIA’s activity decreased and there was no need for the introduction of glucocorticoids. There also has been a positive dynamics of glycemia, which allowed reducing the dose of insulin. There were no adverse events during treatment with tocilizumab. The disease’s activity on a visual analog scale has also decreased, as well as functional insufficiency by Children Heals Assessment Questionnaire.

  6. Clinical presentation of juvenile Huntington disease

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    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.

  7. Catch-up growth during tocilizumab therapy for systemic juvenile idiopathic arthritis: results from a phase III trial.

    Science.gov (United States)

    De Benedetti, Fabrizio; Brunner, Hermine; Ruperto, Nicolino; Schneider, Rayfel; Xavier, Ricardo; Allen, Roger; Brown, Diane E; Chaitow, Jeffrey; Pardeo, Manuela; Espada, Graciela; Gerloni, Valeria; Myones, Barry L; Frane, James W; Wang, Jianmei; Lipman, Terri H; Bharucha, Kamal N; Martini, Alberto; Lovell, Daniel

    2015-03-01

    To investigate the impact of tocilizumab treatment on growth and growth-related laboratory parameters in patients with systemic juvenile idiopathic arthritis (JIA) enrolled in a phase III clinical trial. Patients with systemic JIA ages 2-17 years (n = 112) received tocilizumab in a 12-week, randomized, placebo-controlled period and a long-term open-label extension. Height velocity and standard deviation (SD) score; levels of insulin-like growth factor 1 (IGF-1), osteocalcin (OC), and C-telopeptide of type I collagen (CTX-I); and Juvenile Arthritis Disease Activity Score in 71 joints (JADAS-71) were measured in a post hoc analysis of 83 patients who never received growth hormone and did not reach Tanner stage 5 by the end of the first year of treatment. Patients had stunted growth at baseline (mean height SD score -2.2). During tocilizumab treatment, males (73%) and females (83%) experienced above-normal mean height velocities of 6.6 cm/year (P < 0.0001 versus World Health Organization norms). Mean height SD score increases during year 1 (0.29) and year 2 (0.31) were significant (both P < 0.0001). The mean SD score for IGF-1 levels increased significantly (-0.2 for year 1 and -0.1 for year 2 versus -1.0 at baseline; both P < 0.0001). Mean OC and CTX-I levels (both P < 0.0001) and the OC:CTX-I ratio (P = 0.014) significantly increased from baseline to year 2. In multiple regression analysis, first-year height velocity had a significant inverse relationship to JADAS-71 at year 1, age, mean glucocorticoid dosage during the year, and height SD score at baseline. Our findings indicate that during treatment with tocilizumab, patients with systemic JIA experience significant catch-up growth, normalization of IGF-1 levels, and bone balance improvement favoring bone formation. Copyright © 2015 by the American College of Rheumatology.

  8. Neurocognitive impairment in childhood-onset systemic lupus erythematosus: measurement issues in diagnosis.

    Science.gov (United States)

    Williams, Tricia S; Aranow, Cynthia; Ross, Gail S; Barsdorf, Alexandra; Imundo, Lisa F; Eichenfield, Andrew H; Kahn, Philip J; Diamond, Betty; Levy, Deborah M

    2011-08-01

    To assess the prevalence of neurocognitive impairment (NCI) in childhood-onset systemic lupus erythematosus (cSLE) by comparing published classification criteria, and to examine associations between NCI, disease characteristics, psychosocial well-being, and intelligence. cSLE patients and ethnicity- and age-matched healthy controls completed a neuropsychological research battery, and results were categorized by 3 different NCI classification criteria with different cutoff scores (e.g., >2, 1.5, or 1 SD below the mean) and the number of required abnormal tests or domains. Forty-one cSLE subjects and 22 controls were included. Subjects were predominantly female (~70%) and Hispanic (∼70%). Executive functioning, psychomotor speed, and fine motor speed were most commonly affected. Method 1 classified 34.1% of cSLE subjects with NCI compared to method 2 (14.6% with decline and 7.3% with NCI) and method 3 (63.4% with NCI). The prevalence of NCI was not significantly different between the controls and patients using any of the categorization methods. NCI was not associated with SLE disease activity or characteristics or with depression. Using method 3, patients in the cognitive impairment group reported significantly lower quality of life estimates (69.7 versus 79.3; P = 0.03). Below average intellectual functioning (intelligence quotient 1 and >1.5 SDs, but not >2 SDs below the mean. NCI was prevalent in cSLE, but varied according to the chosen categorization method. A similar proportion of cSLE patients and controls had NCI, reinforcing the importance of studying an appropriate control group. Categorical classification (i.e., impaired/nonimpaired) may oversimplify the commonly observed deficits in cSLE. Copyright © 2011 by the American College of Rheumatology.

  9. Macrophage activation syndrome at the onset of glucocorticoid-resistant systemic lupus erythematosus: a case report.

    Science.gov (United States)

    Tulbă, Delia; Balea, Marius; Băicuş, Cristian

    2018-03-01

    Macrophage activation syndrome (MAS) is a life-threatening hyperinflammatory state mediated by uncontrolled cytokine storm and haemophagocytosis. Although rarely reported, MAS might occur in systemic lupus erythematosus (SLE), notably as an inaugural manifestation. Glucocorticoids (GCs) are the cornerstone of SLE therapy. However, in some cases high doses of GCs are required to achieve remission (i.e. glucocorticoid-resistance), leading to significant side effects. A 28-year-old Romani male was admitted to our hospital for polyarthralgia, polyserositis and fatigability. The patient had high-grade fever, jaundice and generalized lymphadenopathy. Laboratory tests revealed severe mixed hemolytic autoimmune anemia, leukopenia, hepatocytolysis, coagulation abnormalities, hypertriglyceridemia, biological inflammatory syndrome, hyperferritinemia and persistent proteinuria of nephritic pattern. Imaging studies showed pleuropericardial effusion, hepatosplenomegaly and polysynovitis. Additional blood tests revealed hypocomplementemia and positive ANA, anti-dsDNA and anti-Sm antibodies. Haemophagocytosis was not identified either on bone marrow or axillary lymph node biopsy specimens. However, SLE-associated MAS seemed to fit this set-up. High-dose corticotherapy (6.5 g methylprednisolone followed by prednisone, 1.5 mg/kg/day after discharge) and intravenous cyclophosphamide were necessary to induce and sustain remission. MAS is a potentially severe manifestation that should be considered at SLE onset whenever high fever and elevated serum levels of aspartate aminotransferase, lactate dehydrogenase, C-reactive protein, ferritin and procalcitonin are noted. Early diagnosis and prompt treatment lead to remission in two thirds of cases. Glucocorticoid-resistance leads to the use of high-dose corticotherapy or immunosuppressive agents that could elicit serious side effects. New insights into the molecular mechanisms of glucocorticoid-resistance are needed in order to conceive

  10. A Practical Approach to Juvenile Dermatomyositis and Juvenile Scleroderma.

    Science.gov (United States)

    McCann, Liza J; Pain, Clare E

    2016-02-01

    Juvenile dermatomyositis and juvenile scleroderma are rare multisystem autoimmune disorders. Although they share some pathognomonic hallmarks with adult onset myositis or scleroderma, there are significant differences in presentation, characteristics and associated features when the diseases present in childhood. In view of this, and the rarity of the conditions, it is important for care to be led by teams with expertise in pediatric rheumatology conditions. Prognosis has improved significantly in the West; likely due to early diagnosis and aggressive treatment with immunosuppressive medications. However, this trend is not replicated in the developing world. Early recognition of these diseases is crucial to achieve rapid and sustained remission and prevent disease or medication associated complications. This article aims to provide a practical overview for recognition, diagnosis and treatment of these conditions.

  11. Influence of age at disease onset in the outcome of paediatric systemic lupus erythematosus.

    Science.gov (United States)

    Descloux, Elodie; Durieu, Isabelle; Cochat, Pierre; Vital-Durand, Denis; Ninet, Jacques; Fabien, Nicole; Cimaz, Rolando

    2009-07-01

    The aim of this study was to investigate the influence of age at disease onset in the outcome of paediatric SLE (pSLE). Fifty-six patients with pSLE, divided into three groups (pre-pubertal, peripubertal and post-pubertal onset), were studied. The SDI (SLICC/ACR Damage Index for SLE), patients' characteristics, disease manifestations and treatments were compared using Fisher's exact test and Kruskal-Wallis test. Kaplan-Meier curves were constructed to compare the risk of damage occurrence. The risk of damage (SDI >or=1) significantly decreased when age at disease onset increased (89% in pre-pubertal pSLE, 57% in peripubertal pSLE and 38% in post-pubertal pSLE). This excess of risk was found in all disease duration intervals studied (1-3, 3-5, 5-8, 8-10, >10 years) and at the end of follow-up. Kaplan-Meier curves indicated a higher and earlier risk of damage in younger patients. Young children showed higher frequency of autoimmune family history. The frequency of neuropsychiatric disorders and damages decreased with age at disease onset (P 0.5 mg/kg/day) and number of immunosuppressive drugs used that seem to contribute to damage significantly increased when age at disease onset decreased. The risk of damage is inversely correlated with age at disease onset in pSLE. The poorer outcome observed in younger children may be explained by a more severe disease expression, may be a higher infectious susceptibility, and a more aggressive therapy, particularly within the first 6 months of disease course.

  12. Púrpura trombocitopênica imunológica como manifestação inicial de lúpus eritematoso sistêmico juvenil Idiopathic thrombocytopenic purpura as initial manifestation of juvenile systemic lupus erythematosus

    Directory of Open Access Journals (Sweden)

    Josefina Aparecida Pellegrini Braga

    2003-12-01

    salientar a importância da determinação de auto-anticorpos para lúpus eritematoso sistêmico nas crianças com a forma crônica desta patologia.Patients with Idiopathic Thrombocytopenic Purpura (ITP present a high trend to develop Systemic Lupus Erythematosus (SLE, especially those with chronic presentation. Some authors observed that female gender, older patients and familial history of autoimmune disease in patients with Idiopathic Thrombocytopenic Purpura are factors that lead to increased susceptibility for the development of Systemic Lupus Erythematosus. Based on these facts, we decided to study 5 children with chronic Idiopathic Thrombocytopenic Purpura and late Systemic Lupus Erythematosus. In this paper, we describe the clinical and laboratorial features of 5 female children with Idiopathic Thrombocytopenic Purpura that later developed Systemic Lupus Erythematosus. All patients were girls, 3 Caucasian, with Idiopathic Thrombocytopenic Purpura onset age ranged between 6 years and 3 months and 12 years and 1 month (mean - 9 years and 2 months. The age at Systemic Lupus Erythematosus diagnosis ranged between 8 years and 13 years and 8 months (mean - 10 years and 10 months. Though, the gap between Idiopathic Thrombocytopenic Purpura and Systemic Lupus Erythematosus diagnosis ranged between 11 months and 2 years and 9 months (mean - 1 year and 10 months. All patients presented chronic Idiopathic Thrombocytopenic Purpura (thrombocytopenia lasts longer than 6 months. The Systemic Lupus Erythematosus classification criteria were (in decreasing frequency: malar erythema and positivity of ANA in 5 patients; arthritis, hematological (thrombocytopenia and immunological alterations (positivity of anti-DNA in 4 patients; photosensitivity and positivity of anti-cardiolipin in 3 patients. Other manifestations included oral ulcers, renal involvement, leukopenia and auto-immune hemolytic anemia, serositis (pericarditis, neurological involvement and positivity of anti-Sm antibody

  13. Conversion (dissociative) symptoms as a presenting feature in early onset bipolar disorder: a case series

    OpenAIRE

    Ghosal, Malay Kumar; Guha, Prathama; Sinha, Mausumi; Majumdar, Debabrata; Sengupta, Payel

    2009-01-01

    We present three cases of early onset bipolar disorder where dissociative (conversion) symptoms preceded the onset of mania. This case series underscores the significance of dissociative/conversion symptoms as an early atypical presentation in juvenile bipolar disorder.

  14. Development of a new multiple sampling trawl with autonomous opening/closing net control system for sampling juvenile pelagic fish

    Science.gov (United States)

    Oozeki, Yoshioki; Hu, Fuxiang; Tomatsu, Chiaki; Kubota, Hiroshi

    2012-03-01

    A new multiple layer sampling trawl with an autonomous net opening/closing control system was developed to sample pelagic juvenile fish quantitatively. The new trawl system, based on the Matsuda-Oozeki-Hu Trawl (MOHT), has a rigid-frame 3.3 m high and 2.35 m wide and five nets of 11.0 m length with a rectangular mouth of 2.22 m×1.81 m (4 m2 mouth area; large-scale prototype). A cambered V-shape depressor is hung below the frame and two bridles are attached at the midpoint of the side frames. A net-release controller is used, which not only controls the net release mechanism but also records the net depth, temperature and flow rate during net towing. The controller sends stored command signals to the net release mechanism as depth settings and/or time settings and does not require any commands from the surface through a conducting cable or by acoustic signals. Two other models were constructed before the construction of the large-scale prototype, which are a small-scale prototype (2 m2 mouth area) for testing the net release mechanism and a 1/4-scale model of the large-scale prototype for flume tank tests. Flume tank tests with the 1/4-scale model showed that the frame leaned forward at a tilt angle from 5 to 15 degrees at towing speeds from 0.8 to 1.4 m s-1. Opened nets closed smoothly and sequentially nets were completely opened when the trigger was released by the command. Net depth rarely changed even during changes in towing speed. Sea trials both by the small-scale and the large-scale prototype demonstrated the same towing characteristics expected from the flume tank tests. The newly developed multiple layer opening/closing MOHT (MOC-MOHT) is considered to be suitable for quantitative layer sampling of juvenile fish.

  15. Carpal erosions in children with juvenile idiopathic arthritis: repeatability of a newly devised MR-scoring system

    Energy Technology Data Exchange (ETDEWEB)

    Boavida, Peter [Great Ormond Street Hospital for Children, Department of Radiology, London (United Kingdom); Lambot-Juhan, Karen [Hospital Necker Enfants Malades, Department of Radiology, Paris (France); Ording Mueller, Lil-Sofie [Oslo University Hospital, Department of Radiology, Oslo (Norway); Damasio, Beatrice; Malattia, Clara [Ospedale Pediatrico Gaslini, Department of Rheumatology, Genoa (Italy); Tanturri de Horatio, Laura [Ospedale Pediatrico Bambino Gesu, Department of Radiology, Rome (Italy); Owens, Catherine M. [Great Ormond Street Hospital for Children, Department of Radiology, London (United Kingdom); UCL, Institute of Child Health, London (United Kingdom); Rosendahl, Karen [Haukeland University Hospital, Department of Radiology, Bergen (Norway); University of Bergen, Department of Clinical Medicine, Bergen (Norway)

    2015-12-15

    Juvenile idiopathic arthritis (JIA) is characterized by synovial inflammation, with potential risk of developing progressive joint destruction. Personalized state-of-the-art treatment depends on valid markers for disease activity to monitor response; however, no such markers exist. To evaluate the reliability of scoring of carpal bone erosions on MR in children with JIA using two semi-quantitative scoring systems. A total of 1,236 carpal bones (91 MR wrist examinations) were scored twice by two independent pediatric musculoskeletal radiologists. Bony erosions were scored according to estimated bone volume loss using a 0-4 scale and a 0-10 scale. An aggregate erosion score comprising the sum total carpal bone volume loss was calculated for each examination. The 0-4 scoring system resulted in good intra-reader agreement and moderate to good inter-observer agreement in the assessment of individual bones. Fair and moderate agreement were achieved for inter-reader and intra-reader agreement, respectively, using the 0-10 scale. Intra- and particularly inter-reader aggregate score variability were much less favorable, with wide limits of agreement. Further analysis of erosive disease patterns compared with normal subjects is required, and to facilitate the development of an alternative means of quantifying disease. (orig.)

  16. Carpal erosions in children with juvenile idiopathic arthritis: repeatability of a newly devised MR-scoring system

    International Nuclear Information System (INIS)

    Boavida, Peter; Lambot-Juhan, Karen; Ording Mueller, Lil-Sofie; Damasio, Beatrice; Malattia, Clara; Tanturri de Horatio, Laura; Owens, Catherine M.; Rosendahl, Karen

    2015-01-01

    Juvenile idiopathic arthritis (JIA) is characterized by synovial inflammation, with potential risk of developing progressive joint destruction. Personalized state-of-the-art treatment depends on valid markers for disease activity to monitor response; however, no such markers exist. To evaluate the reliability of scoring of carpal bone erosions on MR in children with JIA using two semi-quantitative scoring systems. A total of 1,236 carpal bones (91 MR wrist examinations) were scored twice by two independent pediatric musculoskeletal radiologists. Bony erosions were scored according to estimated bone volume loss using a 0-4 scale and a 0-10 scale. An aggregate erosion score comprising the sum total carpal bone volume loss was calculated for each examination. The 0-4 scoring system resulted in good intra-reader agreement and moderate to good inter-observer agreement in the assessment of individual bones. Fair and moderate agreement were achieved for inter-reader and intra-reader agreement, respectively, using the 0-10 scale. Intra- and particularly inter-reader aggregate score variability were much less favorable, with wide limits of agreement. Further analysis of erosive disease patterns compared with normal subjects is required, and to facilitate the development of an alternative means of quantifying disease. (orig.)

  17. THE STUDY OF FEATURES OF GUILT OF JUVENILE OFFENDERS IN THE CONTEXT OF JUVENILE JUSTICE

    Directory of Open Access Journals (Sweden)

    Natalija Vladimirovna Galkina

    2015-08-01

    Full Text Available The article is devoted to the results of empirical studies of the experiences of guilt of juvenile offenders in the context of juvenile justice where a minor appears as the subject of legal relations. Restorative approach of juvenile justice is based on an admission of guilt to the victim. In connection with it, the research of features of the guilt of minors who have committed an offence and the conditions for the development of the subjectivity will enhance understanding of the possibilities of restorative juvenile justice system in the prevention of juvenile delinquency.Thus, the results of empirical research presented in the article are important for determining of the psychological bases of realization of rehabilitation programs in the context of juvenile justice. In particular, the results are important for the organization and conduct of psychological work to overcome the psychological barriers in the behavior of juveniles having inherently maladaptive guilt and destructive psychological defense mechanisms.

  18. Disease activity and transition outcomes in a childhood-onset systemic lupus erythematosus cohort.

    Science.gov (United States)

    Son, M B; Sergeyenko, Y; Guan, H; Costenbader, K H

    2016-11-01

    Objective The chronicity and severity of childhood-onset systemic lupus erythematosus (cSLE) necessitate effective transition from pediatric to adult providers. We studied transition outcomes in a cSLE cohort. Methods We identified patients at an adult lupus clinic diagnosed with SLE ≤ 18 years who had been followed by a pediatric rheumatologist. Data extracted from the first three years in adult care ("post-transition period") included: sociodemographics, depression, anxiety, SLE manifestations, SLE Disease Activity Index (SLEDAI) and Systemic Lupus International Collaborating Clinics/ACR Damage Index for SLE (SLICC) scores, non-adherence, and gaps in care (no appointments in the recommended time frame). Multivariable logistic regression analyses for predictors of: (1) time between pediatric and adult providers, (2) gaps in care, (3) unscheduled utilization (emergency department visits and admissions) (4) depression and/or anxiety were performed, as was a multivariable Poisson regression analysis for number of missed appointments. Results In 50 patients, SLEDAI scores were stable (mean 5.7 ± 5.0 at start vs. 4.7 ± 4.8 at year 3, p = 0.2), but SLICC scores increased (0.46 ± 0.84, vs. 0.78 ± 1.25, p = 0.01). Depression and anxiety increased significantly (10% vs. 26%, p = 0.02). Mean time from last pediatric to first adult provider visit was almost nine months (253 ± 392 days). Nearly 75% of patients had ≥ 1 gap in care. White race, low education level and non-adherence were significantly associated with missed appointments. Conclusion Despite moderate disease activity in this cSLE transition cohort, prolonged time between pediatric and adult providers and gaps in care in the post-transition period occurred. Anxiety and depression were frequently reported. Future work should identify methods to improve transition.

  19. Effects of birthweight on reproductive system development and onset of puberty in gilts

    NARCIS (Netherlands)

    Almeida, F.R.C.L.; Laurenssen, B.F.A.; Pereira, L.X.; Teerds, K.J.; Soede, N.M.

    2017-01-01

    The aim of the present study was to investigate the effects of birthweight on bodyweight development, development of the genital tract, onset of puberty and their associations with insulin-like growth factor (IGF) 1 and leptin concentrations. Pairs of littermate gilts from 51 litters were selected:

  20. Clinical and immunological aspects and outcome of a Brazilian cohort of 414 patients with systemic lupus erythematosus (SLE): comparison between childhood-onset, adult-onset, and late-onset SLE.

    Science.gov (United States)

    das Chagas Medeiros, M M; Bezerra, M Campos; Braga, F N Holanda Ferreira; da Justa Feijão, M R Melo; Gois, A C Rodrigues; Rebouças, V C do Rosário; de Carvalho, T M Amorim Zaranza; Carvalho, L N Solon; Ribeiro, Át Mendes

    2016-04-01

    The clinical expression of systemic lupus erythematosus (SLE) is influenced by genetic and environmental factors and therefore varies between ethnicities. Information on the epidemiology of SLE in Brazil is scarce and practically limited to studies conducted in socioeconomically developed regions (South and Southeast). The objective of this study was to describe the clinical and immunological aspects and outcome of a cohort of patients with SLE treated at a university hospital in northeastern Brazil and compare patterns related to age at onset: childhood (cSLE), adult (aSLE), and late (lSLE). A random sample of 414 records (women: 93.5%) were reviewed. The mean age at SLE onset and the mean disease duration were 28.9 ± 10.9 years and 10.2 ± 6.6 years, respectively. Most patients had aSLE (n = 338; 81.6%), followed by cSLE (n = 60; 14.5%) and lSLE (n = 16; 3.9%). The female/male ratio was 6.5:1 in cSLE and 16.8:1 in aSLE; in lSLE, all patients were female (p = 0.05). During follow-up, the cSLE group presented higher rates of nephritis (70% vs. 52.9% vs. 12.5%; p = 0.0001) and leuko/lymphopenia (61.7% vs. 43.8% vs. 56.2%; p = 0.02). No significant differences were found for anti-dsDNA, anti-Sm, and antiphospholipid antibodies. Treatment with immunosuppressants was significantly more common, and higher doses of prednisone were used, in cSLE. The prevalence of cardiovascular diseases were more frequent in lSLE (p = 0.03). No significant differences were found between the three groups with regard to mean damage accrual (SDI), remission, and mortality. Although cSLE presented higher rates of nephritis and leuko/lymphopenia, more frequent use of immunosuppressants and higher prednisone doses than aSLE and lSLE, the three groups did not differ significantly with regard to damage accrual, remission, and mortality. © The Author(s) 2015.

  1. Anti-ribosomal P antibody: a multicenter study in childhood-onset systemic lupus erythematosus patients.

    Science.gov (United States)

    Valões, C C M; Molinari, B C; Pitta, A C G; Gormezano, N W S; Farhat, S C L; Kozu, K; Sallum, A M E; Appenzeller, S; Sakamoto, A P; Terreri, M T; Pereira, R M R; Magalhães, C S; Ferreira, J C O A; Barbosa, C M; Gomes, F H; Bonfá, E; Silva, C A

    2017-04-01

    Objectives Anti-ribosomal P protein (anti-P) autoantibodies are highly specific for systemic lupus erythematosus (SLE). However, the evaluation of this autoantibody in childhood-onset SLE (cSLE) populations has been limited to a few small series, hampering the interpretation of the clinical and laboratorial associations. Therefore, the objective of this multicenter cohort study was to evaluate demographic, clinical/laboratorial features, and disease damage score in cSLE patients with and without the presence of anti-P antibody. Methods This was a retrospective multicenter study performed in 10 pediatric rheumatology services of São Paulo state, Brazil. Anti-P antibodies were measured by ELISA in 228 cSLE patients. Results Anti-P antibodies were observed in 61/228 (27%) cSLE patients. Frequencies of cumulative lymphadenopathy (29% vs. 15%, p = 0.014), acute confusional state (13% vs. 5%, p = 0.041), mood disorder (18% vs. 8%, p = 0.041), autoimmune hemolytic anemia (34% vs. 15%, p = 0.001), as well as presence of anti-Sm (67% vs. 40%, p = 0.001), anti-RNP (39% vs. 21%, p = 0.012) and anti-Ro/SSA antibodies (43% vs. 25%, p = 0.016) were significantly higher in cSLE patients with anti-P antibodies compared to those without these autoantibodies. A multiple regression model revealed that anti-P antibodies were associated with autoimmune hemolytic anemia (odds ratio (OR) = 2.758, 95% confidence interval (CI): 1.304-5.833, p = 0.008) and anti-Sm antibody (OR = 2.719, 95% CI: 1.365-5.418, p = 0.004). The SLICC/ACR damage index was comparable in patients with and without anti-P antibodies ( p = 0.780). Conclusions The novel association of anti-P antibodies and autoimmune hemolytic anemia was evidenced in cSLE patients and further studies are necessary to determine if anti-P titers may vary with this hematological manifestation.

  2. [Determination of 25(OH)D serum levels in children with systemic lupus erythematosus and juvenile idiopathic arthritis].

    Science.gov (United States)

    Rosiles, Vanessa Hernández; Salazar, Carolina Duarte; Velazquez, Rocío Maldonado; Ruiz, Rodolfo Rivas; Clark, Patricia

    It is well recognized that vitamin D has a direct effect in bone and muscle and has been associated as well with some rheumatologic diseases. Reports in children are scarce. The aim of this study was to determine the concentration level of 25(OH)D in a group of patients with systemic lupus erythematosus (SLE) and juvenile idiopathic arthritis (JIA) and compare them with healthy controls. Vitamin D (25(OH)D) was measured with isotope-dilution liquid chromatography-tandem mass spectrometry (ID-LC-MS/MS), PTH with immunoradiometric assay (IRMA), calcium, phosphorus and alkaline phosphatase by colorimetric assay in 37 patients with SLE, 37 patients with JIA and 79 healthy controls. Mean 25(OH)D concentration levels were as follows: SLE 18.9±7.92ng/ml, JIA 21.97±5.55ng/ml and 23.6±3.07ng/ml in healthy controls. There was a significant difference between SLE patients vs. healthy controls (p <0.05); 29.7% of SLE patients, 35.1% of JIA patients and 31.6% of healthy controls had deficient levels of vitamin D. One third of the total sample of children in this study had deficient levels of vitamin D. Patients with SLE presented a significant difference compared with healthy controls. Copyright © 2015 Hospital Infantil de México Federico Gómez. Publicado por Masson Doyma México S.A. All rights reserved.

  3. Urinary interleukin 22 binding protein as a marker of lupus nephritis in Egyptian children with juvenile systemic lupus erythematosus.

    Science.gov (United States)

    Badr, Ahmed Mohamed Mahmoud; Farag, Yomna; Abdelshafy, Maie; Riad, Nermine Magdi

    2018-02-01

    Juvenile systemic lupus erythematosus (JSLE) is a multi-system autoimmune inflammatory disease. Generally, 60% of patients will develop lupus nephritis (LN); thus, early recognition and treatment is associated with better outcome. Interleukin 22 (IL-22) is involved in tissue inflammation and is regulated by interleukin 22 binding protein (IL-22BP). This study aimed to use IL-22BP as a non-invasive marker for disease activity in JSLE and LN. This is a cross-sectional study conducted on 82 subjects: 51 JSLE patients and 31 healthy controls of matched age and gender. Urinary IL-22BP was measured using enzyme-linked immunosorbent assay, and its level was correlated with different clinical and laboratory data in JSLE as well as Systemic Lupus Erythematous Disease Activity Index 2000 (SLEDAI-2k), renal SLEDAI-2k, and Systemic Lupus International Collaborating Clinics (SLICC) renal activity score which were used to assess overall disease and renal activity. Our results showed that urinary IL-22BP level was significantly higher in JSLE patients with mean level of 4.13 ± 1.10, as compared to controls 1.63 ± 0.61 (P value < 0.001); also, patients with active LN had urinary levels of IL-22BP (5.47 ± 1.03) higher than patients with active JSLE without LN (4.23 ± 0.72) and patients with non-active JSLE/LN (3.5 ± 0.65) with a highly significant P value < 0.001. There was a positive correlation with SLEDAI-2k, renal SLEDAI, and renal activity scores (P < 0.001). Urinary IL-22BP may be used as a non-invasive marker for assessment of disease activity in children with JSLE and LN.

  4. Decreased serotonin level during pregnancy alters morphological and functional characteristics of tonic nociceptive system in juvenile offspring of the rat

    Directory of Open Access Journals (Sweden)

    Mikhailenko Victor A

    2003-11-01

    Full Text Available Abstract Serotonin (5-HT contributes to the prenatal development of the central nervous system, acting as a morphogen in the young embryo and later as a neurotransmitter. This biologically active agent influences both morphological and biochemical differentiation of raphe neurons, which give rise to the descending serotonergic paths that regulate the processing of acutely evoked nociceptive inputs. The involvement of 5-HT in the prenatal development of tonic nociceptive system has not been studied. In the present study we evaluated the effects of a single injection (400 mg/kg, 2 ml, i.p. of the 5-HT synthesis inhibitor, para-chlorophenylalanine (pCPA, given to pregnant rats during the critical period fetal serotonin development. The functional integrity of the tonic nociceptive response was investigated in 25 day old rats using the classic formalin test. Morphological analysis of brain structures involved in formalin-induced pain and 5-HT levels in the heads of 12-day embryos were also evaluated. Embryonic levels of 5-HT were significantly lowered by the treatment. The juvenile rats from pCPA-treated females showed altered brain morphology and cell differentiation in the developing cortex, hippocampus, raphe nuclei, and substantia nigra. In the formalin test, there were significant decreases in the intensity and duration of the second phase of the formalin-induced response, characterizing persistent, tonic pain. The extent of impairments in the brain structures correlated positively with the level of decrease in the behavioral responses. The data demonstrate the involvement of 5-HT in the prenatal development of the tonic nociceptive system. The decreased tonic component of the behavioral response can be explained by lower activity of the descending excitatory serotonergic system originating in the raphe nuclei, resulting in decreased tonic pain processing organized at the level of the dorsal horn of the spinal cord.

  5. Towards Establishing a Standardized Magnetic Resonance Imaging Scoring System for Temporomandibular Joints in Juvenile Idiopathic Arthritis

    DEFF Research Database (Denmark)

    Tolend, Mirkamal A; Twilt, Marinka; Cron, Randy Q

    2017-01-01

    of a MRI scoring system for TMJs has important clinical utility in timely improvement of diagnosis, and serving as an outcome measure. We report on a multi-institutional collaboration towards developing a TMJ MRI scoring system for JIA. METHODS: Seven readers independently assessed MRI scans from 21...... patients (42 TMJs, age range 6-16y) using three existing MRI scoring systems from American, German, and Swiss institutions. Reliability scores, scoring system definitions and items were discussed among 10 JIA experts through two rounds of Delphi surveys, nominal group voting, and subsequent consensus...... meetings to create a novel TMJ MRI scoring system. RESULTS: Average-measure intraclass correlation coefficients (avICC) for the total scores of all three scoring systems were highly reliable at 0.96 each. Osteochondral items showed higher reliability than inflammatory items. An additive system was deemed...

  6. Forensic aspects of juvenile violence.

    Science.gov (United States)

    Haller, L H

    2000-10-01

    The juvenile justice system was created because it was recognized that youthful offenders needed to be managed differently from adults. They were to receive habilitation services instead of punishment. It is now more than a century since the creation of the first juvenile court. After 67 years, the US Supreme Court, in Kent v United States stated that the model was not working because juveniles in the criminal justice system received no treatment and they had no rights. Because the issue that had been appealed was the lack of rights (not lack of treatment), the Court mandated that juveniles, like adults, be given certain rights. The following year, in In re Gault, the Court expanded these rights. Subsequent Supreme Court cases have dealt with these kinds of issues--that is, whether juvenile offenders are entitled to the same rights as adults and subject to the same penalties. The Supreme Court has never heard a "right to treatment" case, which is the other part of the juvenile court system. Cases have been brought in lower courts (e.g., Nelson v. Heyne, 1972) alleging inadequate treatment services, but no national impact has resulted. Thus, in general, children in the juvenile court system do not have an enforceable right to treatment and can obtain only what services are available in their jurisdictions. The services often are woefully inadequate. Sentencing a youth to probation, with the requirement that he or she participate in counseling or mental health treatment, is meaningless if services are not available. Community-based, model programs that provide effective treatment do exist. They are, as yet, the rare exception rather than the norm and, therefore, are not available to most youthful offenders. Incarcerated juveniles, obviously, cannot avail themselves of community programs. Litigation to give these youth the same rights as adults in penal institutions is not the answer because incarcerated adults don't have a right to treatment, only a right to be free

  7. SYSTEMIC ADMINISTRATION OF BORDETELLA PERTUSSIS ENHANCES PULMONARY SENSITIZATION TO HOUSE DUST MITE IN JUVENILE RATS

    Science.gov (United States)

    The incidence of allergies and asthma has increased significantly in the past few decades. The objectives of this study were to establish an allergy model in weanling rats to more closely reflect the developing immune system of children, and to determine whether systemic administ...

  8. Involvement of Galectin-9/TIM-3 pathway in the systemic inflammatory response in early-onset preeclampsia.

    Directory of Open Access Journals (Sweden)

    Eva Miko

    Full Text Available Preeclampsia is a common obstetrical disease affecting 3-5% of pregnancies and representing one of the leading causes of both maternal and fetal mortality. Maternal symptoms occur as an excessive systemic inflammatory reaction in response to the placental factors released by the oxidatively stressed and functional impaired placenta. The T-cell immunoglobulin domain and mucin domain (TIM family is a relatively newly described group of molecules with a conserved structure and important immunological functions. Identification of Galectin-9 as a ligand for TIM-3 has established the Galectin-9/TIM-3 pathway as an important regulator of Th1 immunity and tolerance induction.The aim of our study was to investigate the expression and function of Galectin-9 and TIM-3 molecules by peripheral blood mononuclear cells and the possible role of Galectin-9/TIM-3 pathway in the immunoregulation of healthy pregnancy and early-onset preeclampsia. We determined TIM-3 and Gal-9 expression and cytotoxicicty of peripheral lymphocytes of early-onset preeclamptic women and healthy pregnant woman using flow cytometry.Investigating peripheral lymphocytes of women with early-onset preeclampsia, our results showed a decreased TIM-3 expression by T cells, cytotoxic T cells, NK cells and CD56(dim NK cells compared to healthy pregnant women. Interestingly, we found a notably increased frequency of Galectin-9 positive cells in each investigated lymphocyte population in the case of early-onset preeclamptic patients. We further demonstrated increased cytotoxic activity by cytotoxic T and CD56(dim NK cells in women with early-onset preeclampsia. Our findings showed that the strongest cellular cytotoxic response of lymphocytes occurred in the TIM-3 positive subpopulations of different lymphocytes subsets in early-onset preeclampsia.These data suggest that Gal-9/TIM-3 pathway could play an important role in the immune regulation during pregnancy and the altered Galectin-9 and TIM-3

  9. Síndrome de ativação macrofágica em paciente com lúpus eritematoso sistêmico juvenil Macrophage activation syndrome in a patient with juvenile systemic lupus erythematosus

    Directory of Open Access Journals (Sweden)

    Simone Manso de Carvalho

    2008-08-01

    Full Text Available A hemofagocitose reativa ou síndrome de ativação macrofágica (SAM é uma complicação das doenças inflamatórias sistêmicas, causada por expansão de células T e macrófagos, com produção maciça de citocinas pró-inflamatórias, ocorrendo mais freqüentemente na artrite idiopática juvenil sistêmica e raramente no lúpus eritematoso sistêmico juvenil (LESJ. OBJETIVO: Relatar um caso de LESJ que evoluiu com SAM precipitada por infecção e infarto esplênico, com desfecho fatal. RELATO DE CASO: Uma menina de 7 anos, com diagnóstico de LESJ desde os 5 anos, evoluiu com artrite em atividade, alopecia intensa, citopenias, cefaléia, infecções respiratórias recorrentes e elevação intermitente de transaminases. Os anticorpos anti-DNA e anticardiolipina IgG e IgM foram identificados e a biópsia renal evidenciou glomerulonefrite lúpica de classe III. A paciente foi tratada com pulso de metilprednisolona, prednisona, azatioprina e hidroxicloroquina. Após dois anos, na vigência de pneumonia apresentou abdome agudo e convulsões, evoluindo para o choque hemorrágico fatal após esplenectomia, que evidenciou infarto esplênico e infiltração maciça por macrófagos hemofagocíticos CD163+. CONCLUSÃO: A revisão do desfecho sugere a SAM precipitada por infecção e sobreposta a atividade inflamatória do lúpus com febre persistente, citopenias, disfunção hepática, hepatomegalia e esplenomegalia, como efeitos do excesso de produção de citocinas. Os anticorpos anticardiolipina podem ter tido papel precipitante na coagulopatia, que resultou infarto esplênico e choque hemorrágico.Reactive haemophagocytosis or macrophage activation syndrome (MAS is a complication of systemic inflammatory disorders, caused by expansion of T cells and haemophagocytic macrophages, with cytokine overproduction. It has been described most often in systemic juvenile idiopathic arthritis and rarely in juvenile systemic lupus erythematosus (JSLE

  10. Parenting and juvenile delinquency

    NARCIS (Netherlands)

    Hoeve, M.

    2008-01-01

    Juvenile delinquency is a noteworthy problem. This thesis addressed the association between parenting and juvenile delinquency by analyzing the concepts of parenting adopted in family research in relation to criminological concepts and measures of delinquent behavior. Four studies were conducted.

  11. Challenges and prospects of the juvenile justice administration in ...

    African Journals Online (AJOL)

    Juvenile justice administration in Nigeria is weak and has been given very little priority, despite Nigeria being signatory to the major international instruments relevant to the administration of juvenile justice. This is attributable to the history of the penal system of Nigeria, with laws guiding juvenile justice administration having ...

  12. Juvenile Arrests, 2000. Juvenile Justice Bulletin.

    Science.gov (United States)

    Snyder, Howard N.

    This bulletin examines the national and state juvenile arrest rate in 2000 using data reported annually by local law enforcement agencies nationwide to the FBI's Uniform Crime Reporting program. Results indicate that the murder rate in 2000 was the lowest since 1965; juvenile arrests for violence in 2000 were the lowest since 1988; few juveniles…

  13. Professional Development: A Capacity-Building Model for Juvenile Correctional Education Systems

    Science.gov (United States)

    Mathur, Sarup R.; Clark, Heather Griller; Schoenfeld, Naomi A.

    2009-01-01

    Youth in correctional facilities experience a broad range of educational, psychological, medical, and social needs. Professional development, a systemic process that improves the likelihood of student success by enhancing educator abilities, is a powerful way to positively affect student outcomes in correctional settings. This article offers a…

  14. Concepts Shaping Juvenile Justice

    Science.gov (United States)

    White, Rob

    2008-01-01

    Rob White's paper explores ways in which community building can be integrated into the practices of juvenile justice work. He provides a model of what can be called "restorative social justice", one that builds upon the juvenile conferencing model by attempting to fuse social justice concerns with progressive juvenile justice practices.

  15. Juvenile Residential Facility Census, 2010: Selected Findings. Juvenile Offenders and Victims: National Report Series. Bulletin NCJ 241134

    Science.gov (United States)

    Hockenberry, Sarah; Sickmund, Melissa; Sladky, Anthony

    2013-01-01

    This bulletin is part of the "Juvenile Offenders and Victims National Report Series." The "National Report" offers a comprehensive statistical overview of the problems of juvenile crime, violence, and victimization and the response of the juvenile justice system. During each interim year, the bulletins in the "National…

  16. Clinical characteristics of children with Juvenile Systemic Sclerosis: follow-up of 23 patients in a single tertiary center

    Directory of Open Access Journals (Sweden)

    Katsicas María M

    2007-05-01

    Full Text Available Abstract Background Juvenile systemic sclerosis (JSS is a multisystem connective tissue disease characterized by skin fibrosis and internal organ involvement. It has a low prevalence, even in a tertiary facility setting. The purpose of the present study is to describe and analyze the clinical and laboratory characteristics of a group of children with JSS followed in a single center. Methods Clinical charts of children with a diagnosis of JSS who were seen at a tertiary referral center between 1995 and 2005 were reviewed. Clinical features were recorded and analysed. Results Twenty-three patients who met preliminary classification criteria for JSS were included. Age at first symptom attributable to JSS was 6 (1–14 years, The first symptom attributable to JSS was Raynaud's phenomenon in 14 cases. Proximal sclerosis (23 patients, 100%, sclerodactyly (21, 91%, Raynaud's phenomenon (19, 83%, and periungual capillaropathy (17, 74% were the most consistent clinical findings during follow-up. Respiratory involvement occurred in two thirds of our patients, and it manifested as dyspnea as well as abnormal imaging and/or pulmonary function tests; pulmonary hypertension was an infrequent finding. Dysphagia was the commonest gastrointestinal symptom (9 patients, 39%. The most frequent musculoskeletal symptom was arthralgia (14 children, 6%; symmetrical arthritis was found in 8 (35% patients. Periungual capillary abnormalities were evident during physical examination in 17 children; capillaroscopy revealed abnormalities in all 19 examined patients. ANA were present in 17 (74% children: homogeneous pattern was the most frequent (8 patients, nucleolar (5 and speckled (4 were less common. Conclusion Raynaud's phenomenon heralds the beginning of the disease. Capilaroscopy is a major adjuvant in the diagnosis, since autoantibody determination may not offer sensitive and specific markers. Skin and vascular manifestations are the most common clinical features

  17. The effect of carnitine on ketogenesis in perfused livers from juvenile visceral steatosis mice with systemic carnitine deficiency.

    Science.gov (United States)

    Nakajima, T; Horiuchi, M; Yamanaka, H; Kizaki, Z; Inoue, F; Kodo, N; Kinugasa, A; Saheki, T; Sawada, T

    1997-07-01

    Juvenile visceral steatosis (JVS) mice have been reported to have systemic carnitine deficiency, and the carnitine concentration in the liver of JVS mice was markedly lower than that of controls (11.6 +/- 2.6 versus 393.5 +/- 56.4 nmol/g of wet liver). To evaluate the role of carnitine in mitochondrial beta-oxidation in liver, we examined the effects of carnitine on ketogenesis in perfused liver from control and JVS mice. In control mice, ketogenesis was increased by the infusion of 0.3 mM oleate, but not by L-carnitine. In contrast, although ketogenesis in JVS mice was not increased by the infusion of oleate, it was increased 2.5-fold by the addition of 1000 microM L-carnitine. Addition of 50, 100, and 200 microM L-carnitine increased ketogenesis in a dose-dependent manner. The infusion of 0.3 mM octanoate or butyrate increased ketogenesis in a carnitine-independent fashion in both control and JVS mice. These findings suggest that endogenous long chain fatty acids from accumulated triglycerides may be used as substrates in the presence of carnitine in JVS mice. The relationship between ketogenesis and free carnitine concentration was examined in livers from JVS mice. Ketogenesis increased as free carnitine levels increased until concentrations exceeded about 100 nmol/g of wet liver (340 microM). The free carnitine concentration required for half-maximal ketone body production in liver of JVS mice was 45 microM (13 nmol/g of wet liver), which corresponds to a K(m) value of carnitine palmitoyltransferase I. We conclude that carnitine is a rate-limiting factor for beta-oxidation in liver only when the carnitine level in liver is very low.

  18. Effects of birthweight on reproductive system development and onset of puberty in gilts.

    Science.gov (United States)

    Almeida, F R C L; Laurenssen, B; Pereira, L X; Teerds, K J; Soede, N M

    2017-02-01

    The aim of the present study was to investigate the effects of birthweight on bodyweight development, development of the genital tract, onset of puberty and their associations with insulin-like growth factor (IGF) 1 and leptin concentrations. Pairs of littermate gilts from 51 litters were selected: one piglet with the highest birthweight (HW; 1.5±0.2kg) and the other with the lowest birthweight (LW; 1.0±0.2kg). Gilt pairs were killed at either fixed ages (80.8±1.2 days; AG; 16 pairs), fixed bodyweight (35.2±1.4kg; WG; 16 pairs) or after first oestrus (EG; 19 pairs). In the AG group, HW gilts were 5.6kg heavier at the time of death than LW gilts. In the WG group, LW gilts were 5.9 days older at the time of death (Pgilts, but bodyweight at time of death was greater for HW gilts (P<0.05). Birthweight did not affect the development of the genital tract, ovulation rate or hormone plasma concentrations. These results suggest that birthweight does not affect the development of the genital tract before puberty and puberty onset.

  19. Efficacy and safety of biological agents for systemic juvenile idiopathic arthritis

    DEFF Research Database (Denmark)

    Tarp, Simon; Amarilyo, Gil; Foeldvari, Ivan

    2016-01-01

    OBJECTIVE: To define the optimal biologic agent for systemic JIA (sJIA) based on safety and efficacy data from a randomized controlled trial (RCT). METHODS: Through a systematic literature search, sJIA RCTs evaluating biologic agents were identified. The primary efficacy outcome was defined as a 30......, this meta-analysis of short-term RCTs presents empirical evidence that canakinumab and tocilizumab are more effective than rilonacept. Biologic agents in sJIA seem safe and comparable with respect to SAE risk in the short term....

  20. Evolution of Juvenile Ankylosing Spondylitis

    Directory of Open Access Journals (Sweden)

    Ye.V. Prohorov

    2013-02-01

    Full Text Available Evolution of juvenile ankylosing spondylitis tend to follow a more frequent involvement in the pathological process of elbow and ankle joints, development of enthesiopathies, changes of intraarticular meniscal horns, forming of Baker’s cysts, cartilage flaps and systemic osteoporosis, and total value of all these signs 13 times exceeds thereof in patients with with the debut of disease in adulthood, but for juvenile ankylosing spondylitis vertebral lesion is less common. Age dimorphism of the use of certain groups of drugs and physiotherapy facilities is observed.

  1. The role of genetic variants in genes regulating the oxytocin-vasopressin neurohumoral system in childhood-onset aggression.

    Science.gov (United States)

    Malik, Ayesha I; Zai, Clement C; Berall, Laura; Abu, Zihad; Din, Farah; Nowrouzi, Behdin; Chen, Sheng; Beitchman, Joseph H

    2014-10-01

    The genetic etiology of aggressive behaviors remains elusive, but growing evidence suggests that they are heritable, and certain genetic variants have been implicated as contributing factors. The oxytocin-vasopressin (OXT-AVP) neurohumoral system has recently been implicated in social behaviors. Oxytocin, especially, has been linked to prosocial behaviors such as trust and social bonds. Hence, the aim of this study was to determine whether genes regulating this system were also associated with childhood-onset aggressive behaviors. Our sample included 182 White children showing extreme, persistent, and pervasive aggressive behavior. These cases were matched with 182 White controls on the basis of sex and age. We used PCR to determine the genotype for 28 single nucleotide polymorphisms within eight genes regulating the OXT-AVP system, including CD38 polymorphisms. Genotypic analyses were carried out using STATA, whereas differences in haplotypic and allelic frequencies were analyzed using Unphased. None of the results reached significance after correction for multiple testing. However, nominally significant allelic effects were observed for OXTR rs6770632T (P=0.028) and AVPR1A rs11174811G (P=0.040) in females, and OXTR rs237898A (P=0.006), rs237902C (P=0.007), and AVP rs3761249A (P=0.008) in males. Genetic variants regulating the OXT-AVP system may be associated with childhood-onset aggression.

  2. Effect of stocking density on performances of juvenile turbot ( Scophthalmus maximus) in recirculating aquaculture systems

    Science.gov (United States)

    Li, Xian; Liu, Ying; Blancheton, Jean-Paul

    2013-05-01

    Limited information has been available about the influence of loading density on the performances of Scophthalmus maximus, especially in recirculating aquaculture systems (RAS). In this study, turbot (13.84±2.74 g; average weight±SD) were reared at four different initial densities (low 0.66, medium 1.26, sub-high 2.56, high 4.00 kg/m2) for 10 weeks in RAS at 23±1°C. Final densities were 4.67, 7.25, 14.16, and 17.47 kg/m2, respectively, which translate to 82, 108, 214, and 282 percent coverage of the tank bottom. Density had both negative and independent impacts on growth. The final mean weight, specific growth rate (SGR), and voluntary feed intake significantly decreased and the coefficient of variation (CV) of final body weight increased with increase in stocking density. The medium and sub-high density groups did not differ significantly in SGR, mean weight, CV, food conversion rate (FCR), feed intake, blood parameters, and digestive enzymes. The protease activities of the digestive tract at pH 7, 8.5, 9, and 10 were significantly higher for the highest density group, but tended to be lower (not significantly) at pH 4 and 8.5 for the lowest density group. The intensity of protease activity was inversely related to feed intake at the different densities. Catalase activity was higher (but not significantly) at the highest density, perhaps because high density started to induce an oxidative effect in turbot. In conclusion, turbot can be cultured in RAS at a density of less than 17.47 kg/m2. With good water quality and no feed limitation, initial density between 1.26 and 2.56 kg/m2 (final: 7.25 and 14.16 kg/m2) would not negatively affect the turbot cultured in RAS. For culture at higher density, multi-level feeding devices are suggested to ease feeding competition.

  3. Disruptions to the cerebellar GABAergic system in juvenile guinea pigs following preterm birth.

    Science.gov (United States)

    Shaw, Julia C; Palliser, Hannah K; Dyson, Rebecca M; Berry, Mary J; Hirst, Jonathan J

    2018-04-01

    Children that are born preterm are at an increased risk of developing cognitive problems and behavioural disorders, such as attention deficit hyperactivity disorder (ADHD). There is increasing interest in the role of the cerebellum in these processes and the potential involvement of GABAergic pathways in neurodevelopmental disorders. We propose that preterm birth, and the associated loss of the trophic intrauterine environment, alters the development of the cerebellum, contributing to ongoing neurobehavioral disorders. Guinea pigs were delivered preterm (GA62) or spontaneously at term (GA69), and tissues collected at corrected postnatal day (PND) 28. Neurodevelopmental and GABAergic markers myelin basic protein (MBP), neuronal nuclei (NeuN), calbindin (Purkinje cells), and GAD67 (GABA synthesis enzyme) were analysed in cerebellar lobules IX and X by immunohistochemistry. Protein expression of GAD67 and GAT1 (GABA transporter enzyme) were quantified by western blot, whilst neurosteroid-sensitive GABA A receptor subunits were measured by RT-PCR. MBP immunostaining was increased in lobule IX of preterm males, and reduced in lobule X of preterm females when compared to their term counterparts. GAD67 staining was decreased in lobule IX and X of the preterm males, but only in lobule X of the preterm females compared to term cohorts for each sex. Internal granule cell layer width of lobule X was decreased in preterm cohorts of both sexes compared to terms. There were no differences between gestational age groups for NeuN staining, GAD67 and GAT1 protein expression as measured by western blotting, or GABA A receptor subunits as measured by RT-PCR between preterm and term for either sex. The present findings suggest that components of the cerebellar GABAergic system of the ex-preterm cerebellum are disrupted. The higher expression of myelin in the preterm males may be due to a deficit in axonal pruning, whereas females have a deficit in myelination at 28 corrected days of

  4. Psammomatoid juvenile ossifying fibroma of the jaws.

    Science.gov (United States)

    Malathi, N; Radhika, T; Thamizhchelvan, H; Ravindran, C; Ramkumar, S; Giri, Gvv; Gopal, Deepika

    2011-09-01

    Juvenile ossifying fibroma is an uncommon, benign, bone-forming neoplasm with aggressive local growth that is distinguished from other fibro-osseous lesions primarily by its age of onset, clinical presentation and aggressive behavior. Although a benign entity, juvenile ossifying fibroma is known to be locally aggressive and has a high tendency to recur. Two distinctive microscopic patterns have been described: A trabecular variant and a psammomatoid variant. This latter variant is predominantly a craniofacial lesion and occurs rarely in the jaws. We present herein two cases of psammomatoid juvenile ossifying fibroma involving the jaws. The first case was a mandibular lesion in a 31-year-old female whereas the second case presented with maxillary involvement in a 46-year-old female. In addition, the pathology of the lesion was analyzed with confocal laser scanning microscopy.

  5. A case report of juvenile Huntington disease

    Directory of Open Access Journals (Sweden)

    Anita Choudhary

    2017-09-01

    Full Text Available Huntington disease (HD is a progressive neurodegenerative disorder, characterized by autosomal dominant inheritance, movement disorder, dementia, and behavioural disturbances. It is caused by a mutation in IT15 gene on chromosome 4p16.3, which leads to unstable CAG trinucleotide repeat expansion. The onset of juvenile HD occurs before the 2nd decade of life and comprises approximately 10% of total HD patients. Juvenile HD differs in symptomatology and is usually transmitted from paternal side with genetic anticipation phenomenon. Magnetic resonance imaging (MRI of the brain shows specific changes of early affection of caudate nucleus and putamen. Multidisciplinary approach with symptomatic treatment of specific symptoms is the current available management. Gene editing and gene silencing treatment are under trial. Hereby, we introduce a case of an 8-year-old boy, who presented with typical symptoms of juvenile HD, positive family history with genetic anticipation phenomenon and characteristic MRI findings.

  6. DNAJC6 Mutations Associated with Early-Onset Parkinson's Disease

    NARCIS (Netherlands)

    S. Olgiati (Simone); M. Quadri (Marialuisa); M. Fang (Mingyan); J.P.M.A. Rood (Janneke P.M.A.); J.A. Saute (Jonas A.); H.F. Chien (Hsin Fen); C.G. Bouwkamp (Christian); J. Graafland (Josja); M. Minneboo (Michelle); G.J. Breedveld (Guido J.); J. Zhang (Jianguo); F.W. Verheijen (Frans W.); A.J.W. Boon (Agnita); A.J.A. Kievit (Anneke J.A.); L.B. Jardim (L.); W.J. Mandemakers (Wim); E.R. Barbosa (Egberto Reis); C.R.M. Rieder (Carlos); K.L. Leenders (Klaus L.); J. Wang (Jinxia); V. Bonifati (Vincenzo)

    2016-01-01

    markdownabstract_Objective_ DNAJC6 mutations were recently described in two families with autosomal recessive juvenile parkinsonism (onset age < 11), prominent atypical signs, poor or absent response to levodopa, and rapid progression (wheelchair-bound within ∼10 years from onset). Here, for the

  7. Onset of quantum chaos in one-dimensional bosonic and fermionic systems and its relation to thermalization

    Science.gov (United States)

    Santos, Lea; Rigol, Marcos

    2010-03-01

    By means of exact diagonalization, we study level statistics and the structure of the eigenvectors of one-dimensional gapless bosonic and fermionic systems across the transition from integrability to quantum chaos. These systems are integrable in the presence of only nearest-neighbor terms, whereas the addition of next-nearest neighbor hopping and interaction may lead to the onset of chaos. We show that the strength of the next-nearest neighbor terms required to observe clear signatures of nonintegrability is inversely proportional to the system size. The transition to chaos is also seen to depend on particle statistics, bosons responding first to the integrability breaking terms. In addition, we discuss the use of delocalization measures as main indicators for the crossover from integrability to chaos. The analysis and findings described in this work footnotetextL. F. Santos and M. Rigol, arXiv:0910.2985 are intimately reflected by studies of thermalization.

  8. JUVENILE DERMATOMIYOSITIS: PENEGAKAN DIAGNOSIS

    Directory of Open Access Journals (Sweden)

    Flora Ramona Sigit Prakoeswa

    2015-06-01

    Full Text Available Juvenile dermatomiyositis (JDM merupakan suatu penyakit autoimun infl amatif yang jarang dijumpai dan secara khas ditandai oleh adanya lesi-lesi kulit tipikal serta kelemahan otot proksimal yang simetris. Etiologi penyakit tersebut belum diketahui dengan pasti, namun demikian patogenesisnya diketahui bersifat multifaktorial meliputi faktor genetik, paparan sinar ultra violet (UV serta infeksi oleh berbagai mikroba seperti virus Coxsackie atau Borrelia burgdorferi. Penegakan diagnosis JDM adalah berdasarkan kriteria Bohan-Peter meliputi: 1 kelemahan otot proksimal simetris, 2 peningkatan enzim otot, 3 infl amasi miopati pada hasil biopsi otot 4 gambaran miyopati pada hasil pemeriksaan EMG, dan 5 lesi kulit tipikal. Ditemukannya papul-papul Gottron dan heliotrope rash di kulit, serta adanya kelemahan otot proksimal yang simetris, merupakan manifestasi klinis yang khas untuk JDM. Terapi lini pertama penatalaksanaan JDM adalah kortikosteroid sistemik. Dilaporkan satu kasus JDM pada seorang anak perempuan berusia 6 tahun. Diagnosis ditegakkan berdasarkan adanya lesi kulit tipikal, kelemahan otot proksimal simetris dan peningkatan kadar enzim penanda infl amasi jaringan muskuloskeletal. Pasien berespon baik terhadap pemberian kortikosteroid adekuat serta tabir surya. Kata Kunci: Juvenile dermatomiyositis, systemic corticosteroid

  9. Adult-onset asthma associated with periocular xanthogranuloma: new diagnostic and therapeutic approaches in a very rare systemic disease.

    Science.gov (United States)

    London, Jonathan; Soussan, Michael; Gille, Thomas; Badelon, Isabelle; Warzocha, Ursula; Galatoire, Olivier; Martin, Antoine; Dhote, Robin; Abad, Sébastien

    2013-01-01

    To report some novel findings concerning the systemic manifestations and treatment of adult-onset asthma and periocular xanthogranuloma, a rare type of non-Langerhans histiocytosis that can lead to important visual dysfunction. A retrospective case series of 2 patients was evaluated for orbital and systemic manifestations using fluorodeoxyglucose positron emission tomography/CT and/or orbital MRI. Histological specimens were reviewed in all patients. Oral prednisone was initiated at 1 mg/kg daily and gradually tapered to a minimum effective dose. Efficacy was assessed on the basis of an objective observation of decreased swelling. One patient displayed original uptake foci involving intrathoracic lymphadenopathies, the trajectory of the paraumbilical vein and perirectal fat. Low-dose prednisone was able to induce a durable response in the authors' patients. Fluorodeoxyglucose positron emission tomography/CT may be useful for the diagnostic workup and follow-up assessment of patients with adult-onset asthma and periocular xanthogranuloma. Oral corticosteroids can be used successfully as first-line treatment in such patients.

  10. Juvenile Justice

    OpenAIRE

    International Child Development Centre

    1998-01-01

    The third Innocenti Digest deals with the main issues connected with children and young people coming into conflict with the law and contact with the justice system. It looks at standards and problems from arrest through to the court hearing and sentencing, use of custodial measures and ways of avoiding the child’s unnecessary and counter-productive involvement with the formal justice system. It also covers prevention questions. Like previous publications in the series, it contains practical ...

  11. Intravenous immunoglobulin therapy leading to dramatic improvement in a patient with systemic juvenile idiopathic arthritis and severe pericarditis resistant to steroid pulse therapy.

    Science.gov (United States)

    Aizawa-Yashiro, Tomomi; Oki, Eishin; Tsuruga, Kazushi; Nakahata, Tohru; Ito, Etsuro; Tanaka, Hiroshi

    2012-05-01

    A 7-year-old Japanese boy with a 4-month history of systemic juvenile idiopathic arthritis (s-JIA) experienced disease flare with spiking fever, exanthema and arthralgia. He then developed progressive dyspnea due to severe pericarditis, and proinflammatory hypercytokinemia was suspected. Methylprednisolone pulse therapy was ineffective and echocardiography showed massive pericardial effusion had persisted. Alternatively, subsequent intravenous immunoglobulin (IVIG) therapy resulted in dramatic resolution of the pericardial effusion, and his general condition significantly improved within a few days. This case report may lend further support the use of IVIG for selected patients with s-JIA and severe pericarditis.

  12. Adult-onset offenders: Is a tailored theory warranted?

    Science.gov (United States)

    Beckley, Amber L.; Caspi, Avshalom; Harrington, Honalee; Houts, Renate M.; Mcgee, Tara Renae; Morgan, Nick; Schroeder, Felix; Ramrakha, Sandhya; Poulton, Richie; Moffitt, Terrie E.

    2016-01-01

    Purpose To describe official adult-onset offenders, investigate their antisocial histories and test hypotheses about their origins. Methods We defined adult-onset offenders among 931 Dunedin Study members followed to age 38, using criminal-court conviction records. Results Official adult-onset offenders were 14% of men, and 32% of convicted men, but accounted for only 15% of convictions. As anticipated by developmental theories emphasizing early-life influences on crime, adult-onset offenders’ histories of antisocial behavior spanned back to childhood. Relative to juvenile-offenders, during adolescence they had fewer delinquent peers and were more socially inhibited, which may have protected them from conviction. As anticipated by theories emphasizing the importance of situational influences on offending, adult-onset offenders, relative to non-offenders, during adulthood more often had schizophrenia, bipolar disorder, and alcohol-dependence, had weaker social bonds, anticipated fewer informal sanctions, and self-reported more offenses. Contrary to some expectations, adult-onset offenders did not have high IQ or high socioeconomic-status families protecting them from juvenile conviction. Conclusions A tailored theory for adult-onset offenders is unwarranted because few people begin crime de novo as adults. Official adult-onset offenders fall on a continuum of crime and its correlates, between official non-offenders and official juvenile-onset offenders. Existing theories can accommodate adult-onset offenders. PMID:27134318

  13. Disruption of the thyroid system by the thyroid-disrupting compound Aroclor 1254 in juvenile Japanese flounder (Paralichthys olivaceus.

    Directory of Open Access Journals (Sweden)

    Yifei Dong

    Full Text Available Polychlorinated biphenyls (PCBs are a group of persistent organochlorine compounds that have the potential to disrupt the homeostasis of thyroid hormones (THs in fish, particularly juveniles. In this study, thyroid histology, plasma TH levels, and iodothyronine deiodinase (IDs, including ID1, ID2, and ID3 gene expression patterns were examined in juvenile Japanese flounder (Paralichthys olivaceus following 25- and 50-day waterborne exposure to environmentally relevant concentrations of a commercial PCB mixture, Aroclor 1254 (10, 100, and 1000 ng/L with two-thirds of the test solutions renewed daily. The results showed that exposure to Aroclor 1254 for 50 d increased follicular cell height, colloid depletion, and hyperplasia. In particular, hypothyroidism, which was induced by the administration of 1000 ng/L Aroclor 1254, significantly decreased plasma TT4, TT3, and FT3 levels. Profiles of the changes in mRNA expression levels of IDs were observed in the liver and kidney after 25 and 50 d PCB exposure, which might be associated with a reduction in plasma THs levels. The expression level of ID2 mRNA in the liver exhibited a dose-dependent increase, indicating that this ID isotype might serve as sensitive and stable indicator for thyroid-disrupting chemical (TDC exposure. Overall, our study confirmed that environmentally relevant concentrations of Aroclor 1254 cause significant thyroid disruption, with juvenile Japanese flounder being suitable candidates for use in TDC studies.

  14. A fuzzy-based system reveals Alzheimer's Disease onset in subjects with Mild Cognitive Impairment.

    Science.gov (United States)

    Tangaro, Sabina; Fanizzi, Annarita; Amoroso, Nicola; Bellotti, Roberto

    2017-06-01

    Alzheimer's Disease (AD) is the most frequent neurodegenerative form of dementia. Although dementia cannot be cured, it is very important to detect preclinical AD as early as possible. Several studies demonstrated the effectiveness of the joint use of structural Magnetic Resonance Imaging (MRI) and cognitive measures to detect and track the progression of the disease. Since hippocampal atrophy is a well known biomarker for AD progression state, we propose here a novel methodology, exploiting it as a searchlight to detect the best discriminating features for the classification of subjects with Mild Cognitive Impairment (MCI) converting (MCI-c) or not converting (MCI-nc) to AD. In particular, we define a significant subdivision of the hippocampal volume in fuzzy classes, and we train for each class Support Vector Machine SVM classifiers on cognitive and morphometric measurements of normal controls (NC) and AD patients. From the ADNI database, we used MRI scans and cognitive measurements at baseline of 372 subjects, including 98 subjects with AD, and 117 NC as a training set, 86 with MCI-c and 71 with MCI-nc as an independent test set. The accuracy of early diagnosis was evaluated by means of a longitudinal analysis. The proposed methodology was able to accurately predict the disease onset also after one year (median AUC=88.2%, interquartile range 87.2%-89.0%). Besides its robustness, the proposed fuzzy methodology naturally incorporates the uncertainty degree intrinsically affecting neuroimaging features. Thus, it might be applicable in several other pathological conditions affecting morphometric changes of the brain. Copyright © 2017 Associazione Italiana di Fisica Medica. Published by Elsevier Ltd. All rights reserved.

  15. Climate change is advancing spring onset across the U.S. national park system

    Science.gov (United States)

    Monahan, William B.; Rosemartin, Alyssa; Gerst, Katharine L.; Fisichelli, Nicholas A.; Ault, Toby R.; Schwartz, Mark D.; Gross, John E.; Weltzin, Jake F.

    2016-01-01

    Many U.S. national parks are already at the extreme warm end of their historical temperature distributions. With rapidly warming conditions, park resource management will be enhanced by information on seasonality of climate that supports adjustments in the timing of activities such as treating invasive species, operating visitor facilities, and scheduling climate-related events (e.g., flower festivals and fall leaf-viewing). Seasonal changes in vegetation, such as pollen, seed, and fruit production, are important drivers of ecological processes in parks, and phenology has thus been identified as a key indicator for park monitoring. Phenology is also one of the most proximate biological responses to climate change. Here, we use estimates of start of spring based on climatically modeled dates of first leaf and first bloom derived from indicator plant species to evaluate the recent timing of spring onset (past 10–30 yr) in each U.S. natural resource park relative to its historical range of variability across the past 112 yr (1901–2012). Of the 276 high latitude to subtropical parks examined, spring is advancing in approximately three-quarters of parks (76%), and 53% of parks are experiencing “extreme” early springs that exceed 95% of historical conditions. Our results demonstrate how changes in climate seasonality are important for understanding ecological responses to climate change, and further how spatial variability in effects of climate change necessitates different approaches to management. We discuss how our results inform climate change adaptation challenges and opportunities facing parks, with implications for other protected areas, by exploring consequences for resource management and planning.

  16. Anti-chromatin antibodies in juvenile rheumatoid arthritis

    Directory of Open Access Journals (Sweden)

    V. Gerloni

    2011-09-01

    Full Text Available Objective: to evaluate the prevalence and clinical significance of anti-chromatin antibodies (Abs in juvenile rheumatoid arthritis (JRA. Methods: IgG anti-chromatin Abs were detected by an enzyme-linked immunosorbent assay (ELISA, in sera of 94 children with JRA (10 children with systemic, 38 with polyarticular and 46 with oligoarticular disease onset. As control group, 33 age- and-sex-matched healthy children (HC were also examined. Results: Abs to chromatin were detected in 24/94 (25,5% of children suffering from JRA. Particularly, the higher prevalence of anti-chromatin Abs has been found in children with oligoarticular (30,4% and polyarticular (23,7% onset JRA. In these groups Abs titers were significantly higher compared to systemic JRA and HC (p=0.003. Anti-chromatin Abs were observed more frequently in patients with oligoarticular disease and chronic uveitis (21,7%. Furthermore, higher levels of anti-chromatin Abs has been found in all the patients treated with anti-TNFα therapy (p<0.0001. Conclusions: our results confirm previous data about the prevalence of anti-chromatin Abs in JRA. These Abs were significantly higher in the group of patients with oligoarticular onset with past or present hystory of ocular involvement and in the group with polyarticular JRA treated with biologic therapy. A long-term follow-up study could be useful to evaluate the potential utility of these autoantibodies.

  17. Mycophenolate mofetil as maintenance therapy for childhood-onset systemic lupus erythematosus patients with severe lupus nephritis.

    Science.gov (United States)

    Kizawa, Toshitaka; Nozawa, Tomo; Kikuchi, Masako; Nagahama, Kiyotaka; Okudela, Koji; Miyamae, Takako; Imagawa, Tomoyuki; Nakamura, Tomoko; Mori, Masaaki; Yokota, Shumpei; Tsutsumi, Hiroyuki

    2015-03-01

    We evaluated histological changes occurring in renal biopsy specimens, between the time before initial induction therapy and after 12 months' maintenance therapy, as well as changes in laboratory parameters, SLE disease activity (SLEDAI), and dosage of corticosteroid (CS) in childhood-onset systemic lupus erythematosus (SLE) patients treated with mycophenolate mofetil (MMF). A retrospective analysis was performed on nine patients diagnosed with childhood-onset SLE and lupus nephritis. They were treated with pulsed mPSL and intravenous cyclophosphamide as induction therapy and MMF (500-1500 mg/day) plus CS as maintenance therapy. Renal biopsy was performed before the initial induction therapy and after 12 months' maintenance therapy. Pathological findings at second biopsy were improved in eight of nine patients (89%). The findings of SLEDAI, urinalysis, and blood tests also showed improvement. CS doses could be tapered satisfactorily. Adverse events were observed in two patients. No patients treated with MMF experienced any disease flares during maintenance therapy. MMF as maintenance therapy might be useful in that not only the histological findings of lupus nephritis were improved, but also CS doses could be beneficially tapered. Nonetheless, this is a retrospective report of only nine cases and further prospective multicenter studies are necessary.

  18. 494 Skin Sensitization to Carmine Before Onset of Systemic Allergy to Ingested Carmine

    Science.gov (United States)

    Katada, Yoshinori; Harada, Yoshinori; Azuma, Naoto; Hashimoto, Jun; Saeki, Yukihiko; Tanaka, Toshio

    2012-01-01

    Background Allergic sensitization to food can occur through skin exposure. We investigated anaphylactic cases due to carmine, a food additive extracted from Dactylopius coccus. Methods Screening all patients, who visited our department from January 2000 to December 2009, we identified 2 new such cases. Both had history of rash induced by certain cosmetics containig carmine. We further investigated previous case reports of carmine allergy, whether skin sensitization antedated food allergy or not. Results Case 1: A 26-year-old woman visited our hospital because of anaphylaxis occurred within 5 minutes after ingesting a Japanese YOKAN (sweetened and jellied bean paste). IgE antibodies against common food allergens including beans and wheat were all negative. As the paste contains carmine, we tested specific IgE antibody, which was positive. She had been avoiding using certain cheeks and lips for 2 years, since they cause erythema. These cosmetics emerged as containing carmine. Abstaining from the food additive made her free from anaphylaxis. Case 2: A 30-year-old woman came to our hospital for dyspnea, uriticaria, and bilateral blepharedema, immediately after drinking Campari soda. Her past history was prominent, as she had 4 episodes of anaphylaxis in 4 years, requiring emergency transport twice. All anaphylactic episodes occurred in Italian restaurants when she drank cocktails, which might contain carmine in Campari soda. She had been also sensitive to certain rouges since several years before the first onset of anaphylaxis. It became clear that the rouges contained carmine. In literatures, we found 22 cases with allergy to ingested carmine. It is surprising that all cases were women (aged 25 to 52), while occupationally sensitized patients are predominantly men. As far as we could know, 85.7 % of (6/7) mentioned cases had previous history of sensitization to cosmetics containing carmine. Conclusions In many cases with allergy against ingested carmine, the route of

  19. In juvenile dermatomyositis, heart rate variability is reduced, and associated with both cardiac dysfunction and markers of inflammation: a cross-sectional study median 13.5 years after symptom onset.

    Science.gov (United States)

    Barth, Zoltan; Nomeland Witczak, Birgit; Schwartz, Thomas; Gjesdal, Knut; Flatø, Berit; Koller, Akos; Sanner, Helga; Sjaastad, Ivar

    2016-03-01

    Low heart rate variability (HRV) is a well-established predictor of cardiac death. The aim of this study was to investigate arrhythmias and HRV in patients with JDM, and associations between HRV and inflammatory markers, echocardiographic measurements and disease parameters. Fifty-five patients with JDM were examined 2-34 years (median 13.5 years) after disease onset, and compared with 55 age and sex matched controls. Holter ECG monitoring and echocardiography were analysed blinded to patient information. Arrhythmia and HRV (six parameters) were analysed by standard software, finally adjudicated by an experienced cardiologist. Markers of inflammation (ESR, high sensitivity (hs)CRP and cytokines) were analysed. Disease activity and organ damage were assessed by clinical examination at follow-up and retrospectively by chart review. In two out of six HRV parameters, JDM patients had lower values than controls. No difference in arrhythmias was found between the groups. In patients, but not in controls, there were significant negative correlations between five out of six HRV parameters, and ESR and hsCRP (Spearman correlation coefficient, -0.306 to -0.470; P, 0.023 to markers, active disease and reduced myocardial function. This suggests reduced vagal control of the heart; further studies are needed to determine whether this is also associated with cardiac morbidity or mortality. © The Author 2015. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

  20. Long-term outcome in patients with juvenile dermatomyositis

    DEFF Research Database (Denmark)

    Mathiesen, P; Hegaard, H; Herlin, Troels

    2012-01-01

    To evaluate a group of 53 patients with juvenile dermatomyositis (JDM), on average 13.9 years after disease onset, in order to describe the long-term disease outcome and to identify disease-related parameters associated with poor disease outcome.......To evaluate a group of 53 patients with juvenile dermatomyositis (JDM), on average 13.9 years after disease onset, in order to describe the long-term disease outcome and to identify disease-related parameters associated with poor disease outcome....

  1. Juvenile Dermatomyositis: Key Roles of Muscle Magnetic Resonance Imaging and Early Aggressive Treatment.

    Science.gov (United States)

    Corral-Magaña, O; Bauzá-Alonso, A F; Escudero-Góngora, M M; Lacruz, L; Martín-Santiago, A

    2017-09-11

    Juvenile dermatomyositis is a rare systemic connective tissue disease with onset during childhood. It presents clinically with proximal muscle weakness and characteristic skin involvement. Diagnosis is based on the Bohan and Peter criteria, though many authors are now substituting biopsy with muscle magnetic resonance imaging (MRI) for both diagnosis and follow-up. Without intensive early treatment, complications such as calcinosis cutis and lipodystrophy can develop in the chronic phases of the disease. Early recognition is therefore key to management. We present a series of 5 patients who were diagnosed with Juvenile dermatomyositis on muscle MRI without undergoing muscle biopsy and who received early treatment. We draw attention to the usefulness of muscle MRI for the diagnosis of muscle involvement and to the importance of early initiation of intensive treatment to prevent complications. Copyright © 2017 AEDV. Publicado por Elsevier España, S.L.U. All rights reserved.

  2. Developmental Psycho- Neurological Research Trends and Their Importance for Reassessing Key Decision-Making Assumptions for Children, Adolescents, and Young Adults in Juvenile/Youth and Adult Criminal Justice Systems

    Directory of Open Access Journals (Sweden)

    Raymond Corrado

    2014-12-01

    Full Text Available One of the underlying foundations of Western criminal justice is the notion that human behavior is the product of rational choice. The creation of separate justice systems for juveniles and adults is based on the idea that fundamental differences in rationality exist between these two groups. Since its inception, the establishment of upper and lower boundaries demarking the juvenile justice system has been a highly contentious issue, both scientifically and politically. Critically, this debate stems from the largely arbitrary nature of the boundaries. Over the last thirty years a sufficiently large body of psychological and neurological empirical work has examined the development of decision-making and rational choice in late childhood, adolescents, and adulthood. The current article discusses the implications of this research on the establishment of upper and lower age jurisdictions for the juvenile justice system, as well as how adolescent decision-making influences other key aspects of the justice process such as competency to stand trial.

  3. Juvenile generalized pustular psoriasis is a chronic recalcitrant disease: an analysis of 27 patients seen in a tertiary hospital in Johor, Malaysia.

    Science.gov (United States)

    Lau, Bi-Wen; Lim, Dee-Zhen; Capon, Francesca; Barker, Jonathan N; Choon, Siew-Eng

    2017-04-01

    Limited information exists regarding juvenile generalized pustular psoriasis (GPP). We aim to determine the clinical profile and outcome of Malaysians with juvenile GPP. Review of hospital case notes on patients with juvenile GPP. Twenty-seven patients with juvenile GPP were identified. Female to male ratio was 1.4:1. The median age at onset of GPP was 6.5 years. Ten patients had prior psoriasis with a median pre-pustular duration of 2.7 years. Onset of GPP was earlier in patients without prior psoriasis (5.1 years vs. 12.0 years, P = 0.002). Precipitating factors identified included stress, upper respiratory tract infection, systemic steroid use, vaccination, and pregnancy. A positive family history of psoriasis and GPP was present in six and one patient(s), respectively. Twenty-one patients had acute, five annular, and one localized variant of GPP. Arthritis was present in 22.2%. Fever, leukocytosis, and transaminitis were mainly seen in patients with acute GPP at 80.9, 72.2, and 11.1%, respectively. Among 20 patients screened, eight carry IL36RN variants and one has CARD14 mutation. IL36RN-positive patients have more severe disease characterized by early onset, low prevalence of prior plaque psoriasis, high prevalence of systemic inflammation, and need for continuous long-term systemic therapy. Acitretin and cyclosporine were effective in aborting acute GPP in 100% of 16 and 66.7% of six patients treated, respectively. However, relapses were common. Only three of the 17 patients whose initial acute GPP was controlled with systemic agents were successfully weaned off treatment. Juvenile GPP is a chronic recalcitrant disease. IL36RN-positive patients have more severe disease. © 2017 The International Society of Dermatology.

  4. [Influence of toxin botulin on walk stereotype of children with juvenile cerebral palsy. The functional examination performed by BTS, comprehensive movement analysis system. A preliminary report].

    Science.gov (United States)

    Kwiecień-Czerwieniec, Ilona; Krukowska, Jolanta; Woldańska-Okońska, Marta

    2014-01-01

    Juvenile Cerebral Palsy--is caused by damage of the motor control centers of the developing brain (cerebral refers to the cerebrum, which is the affected area of the brain, although the disorder probably involves connections between the cortex and other parts of the brain and palsy refers to disorder of movement). The clinical symptoms of juvenile cerebral palsy are very diversified and include gross and fine motor-coordination disorders, manual ability, locomotion, perception and response, speech, psychomotor retardation, emotional disorders. The primary therapeutic problem in children with cerebral palsy is learning to move in a lower position and learning to walk. The aim of this research is evaluation the action of the botulinum toxin on gait pattern of children with cerebral palsy. Application of a comprehensive BTS analysis of gait will get accurate, consistent EBM (Evidence Base Medicine) results. The children with pyramidal syndrome of juvenile cerebral palsy have been included in the examinations. The children have been divided into 2 random groups: group I--children treated with standard therapy a neurodevelopmental rehabilitation and classic kinesiotherapy, group II--hildren treated with standard therapy --eurodevelopmental rehabilitation, classic kinesiotherapy and a botulinum toxin. The children were examined three times: before the therapy, after 6 weeks of treatment and after 3 months of treatment. In the research BTS comprehensive motion analysis system have been used where influence of toxin botulin on walk stereotype of children was assessed. The treatment connected with standard rehabilitation and using botulinum toxin brings quicker walk improvement. Comparing the above treatment methods of influence on the walk stereotype of children with juvenile cerebral palsy, we can confirm, that standard treatment brings regular improvement, still, it requires longer period of time, often even 3 months. Using botulinum toxin brings quicker walk improvement

  5. Onset of magnetic order in strongly-correlated systems from ab initio electronic structure calculations: application to transition metal oxides

    Science.gov (United States)

    Hughes, I. D.; Däne, M.; Ernst, A.; Hergert, W.; Lüders, M.; Staunton, J. B.; Szotek, Z.; Temmerman, W. M.

    2008-06-01

    We describe an ab initio theory of finite temperature magnetism in strongly-correlated electron systems. The formalism is based on spin density functional theory, with a self-interaction corrected local spin density approximation (SIC-LSDA). The self-interaction correction is implemented locally, within the Kohn-Korringa-Rostoker (KKR) multiple-scattering method. Thermally induced magnetic fluctuations are treated using a mean-field 'disordered local moment' (DLM) approach and at no stage is there a fitting to an effective Heisenberg model. We apply the theory to the 3d transition metal oxides, where our calculations reproduce the experimental ordering tendencies, as well as the qualitative trend in ordering temperatures. We find a large insulating gap in the paramagnetic state which hardly changes with the onset of magnetic order.

  6. Pattern-recognition software detecting the onset of failures in complex systems

    International Nuclear Information System (INIS)

    Mott, J.; King, R.

    1987-01-01

    A very general mathematical framework for embodying learned data from a complex system and combining it with a current observation to estimate the true current state of the system has been implemented using nearly universal pattern-recognition algorithms and applied to surveillance of the EBR-II power plant. In this application the methodology can provide signal validation and replacement of faulty signals on a near-real-time basis for hundreds of plant parameters. The mathematical framework, the pattern-recognition algorithms, examples of the learning and estimating process, and plant operating decisions made using this methodology are discussed. The entire methodology has been reduced to a set of FORTRAN subroutines which are small, fast, robust and executable on a personal computer with a serial link to the system's data acquisition computer, or on the data acquisition computer itself

  7. Effects of sulfasalazine treatment on serum immunoglobulin levels in children with juvenile chronic arthritis

    NARCIS (Netherlands)

    van Rossum, MAJ; Fiselier, TJW; Franssen, MJAN; ten Cate, R; van Suijlekom-Smit, LWA; Wulffraat, NM; van Luijk, WHJ; Oostveen, JCM; Kuis, W; Dijkmans, BAC; van Soesbergen, RM

    2001-01-01

    This article describes the effects of sulfasalazine (SSZ) treatment on serum immunoglobulin (Ig) levels in 6 children with oligoarticular- or polyarticular onset juvenile chronic arthritis (JCA). None of the children who developed dysimmunoglobulinemia during treatment showed clinical symptoms of

  8. Identification and regulation of the juvenile hormone esterase gene in the Colorado potato beetle

    NARCIS (Netherlands)

    Vermunt, A.M.W.

    1999-01-01

    A number of important physiological processes in insects is controlled by the titer of juvenile hormone (JH). The juvenile (larval) stage is maintained at a high JH titer, whereas the onset of metamorphosis is induced by a low JH titer. Reproduction by adults requires often a high JH titer.

  9. Juvenile giant fibroadenoma

    Directory of Open Access Journals (Sweden)

    Vipul Yagnik

    2011-07-01

    Full Text Available Fibroadenomas are benign solid tumor associated with aberration of normal lobular development. Juvenile giant fibroadenoma is usually single and >5 cm in size /or >500 gms in weight. Important differential diagnoses are: phyllodes tumor and juvenile gigantomastia. Simple excision is the treatment of choice.

  10. Juvenile Confinement in Context

    Science.gov (United States)

    Mendel, Richard A.

    2012-01-01

    For more than a century, the predominant strategy for the treatment and punishment of serious and sometimes not-so-serious juvenile offenders in the United States has been placement into large juvenile corrections institutions, alternatively known as training schools, reformatories, or youth corrections centers. America's heavy reliance on…

  11. The Age of Onset of Personal Authority in the Family System.

    Science.gov (United States)

    Lawson, David; And Others

    1993-01-01

    Explored position that relational patterns indicative of Personal Authority in Family System occur during fourth and early fifth decades of life. Participants were 232 university students. Canonical correlations revealed that, as age increases, amount of variance accounted for by nuclear family triangulation, intergenerational intimidation, and…

  12. Angrite meteorites record the onset and flux of water to the inner solar system

    Science.gov (United States)

    Sarafian, Adam R.; Nielsen, Sune G.; Marschall, Horst R.; Gaetani, Glenn A.; Hauri, Erik H.; Righter, Kevin; Sarafian, Emily

    2017-09-01

    Earth and the other rocky bodies that make up the inner solar system are systematically depleted in hydrogen (H) and other cosmochemically volatile elements (e.g., carbon (C), fluorine (F), chlorine (Cl), and thallium (Tl)) relative to primitive undifferentiated meteorites known as carbonaceous chondrites. If we are to understand how and when Earth gained its life-essential elements, it is critical to determine the timing, flux, and nature of the delivery of condensed volatiles into the presumed hot and dry early inner solar system. Here we present evidence preserved in ancient basaltic angrite meteorites for an addition of volatiles to the hot and dry inner solar system within the first two million years of solar system history. Our data demonstrate that the angrite parent body was enriched in highly volatile elements (H, C, F, and Tl) relative to those predicted on the basis of the angrite parent body's overall volatile depletion trend (e.g., H is enriched by up to a factor of 106). This relative enrichment is best explained by mixing of extremely volatile-depleted material, located well inside the snow line, with volatile-rich material derived from outside the snow line.

  13. Systems biology of seeds: deciphering the molecular mechanisms of seed storage, dormancy and onset of germination.

    Science.gov (United States)

    Sreenivasulu, Nese

    2017-05-01

    Seeds are heterogeneous storage reserves with wide array of storage compounds that include various soluble carbohydrates, starch polymer, storage proteins and lipids. These stored reserves comprise 70% of the world's caloric intake in the form of food and animal feed produced through sustainable agriculture, which contributes to food and nutritional security. Seed systems biology remains an enigmatic subject in understanding seed storage processes, maturation and pre-germinative metabolism. The reviews and research articles covered in this special issue of Plant Cell Reports highlight recent advances made in the area of seed biology that cover various systems biology applications such as gene regulatory networks, metabolomics, epigenetics and the role of micro-RNA in seed development.

  14. A CASE OF EPSTEIN-BARR VIRAL INFECTION PROCEEDED UNDER THE MASK OF THE SYSTEMIC VARIANT OF THE JUVENILE RHEUMATOID ARTHRITIS

    Directory of Open Access Journals (Sweden)

    T.M. Bzarova

    2007-01-01

    Full Text Available The article describes the treatment of Epstein–Barr viral infection under the mask of the systemic variant of the juvenile rheumatoid arthritis. The clinical presentations of the disease included fever, rash, lymphadenopathy, hepatomegaly, polyarticular syndrome and high lab activity indices. the serologic research uncovered the antibodies to the Epstein–Barr virus in diagnostic titers, which allowed the researchers to verify the diagnosis. A child underwent the treatment with the immunoglobulin of a man with the high concentration of antibodies to cytomegalovirus, which induced the remission of the systemic representations, articular syndrome accompanied B normalization of the lab activity indices and reduction of the antibody titers towards the Epstein–Barr virus.Key words: children, treatment, immune globulin intravenous, septic syndrome, epstein–barr virus.

  15. Multiscale Immune System Simulator for the Onset of Type-2 Diabetes for use in online decision support and coaching tools

    Directory of Open Access Journals (Sweden)

    Albert De Graaf

    2015-10-01

    Full Text Available Background: To empower patients to optimize lifestyle factors and compliance in intervention strategies, predictive personalised simulation models such as developed in the framework of the Virtual Physiological Human initiative (http://www.vph-institute.org/ are a potential important asset. Aim:The FP7 EU project MISSION-T2D aims to develop and validate a computer model incorporating genetic, metabolic, and nutritional data for the simulation and prediction of the onset and progression of T2D. The model should include immunological/inflammatory processes contributing to insulin resistance and beta cell function loss, functionally interacting with energy intake/expenditure-associated metabolic regulatory mechanisms. Methods: The overall architecture of the model interconnects and integrates an already established immune system simulator with newly developed and/or adapted discrete and continuous mathematical simulation models for relevant processes as detailed below. The model is programmed in ANSI/C. Results: Model sections are as follows: 1 GUT MICROBIOTA: predicts how different dietary components affect the relative abundance of important gut bacteria, thus influencing butyrate production, and thereby modulating TNF, IL-6 and IL-1β; 2 PANCREATIC BETA CELL employs mTOR signaling as a central mechanism and predicts the beta cell survival and –functionality as a function of concentration of macrophages, acivated macrophages, and beta cell antigenic proteins; 3 WHOLE BODY METABOLISM describes seven tissue compartments: brain, heart, liver, gastrointestinal tract, skeletal muscle, and adipose tissue, by dynamic mass balances for major cellular metabolic reactions involving glucose, lactate, pyruvate, alanine, free fatty acids, triglycerides, glycerol and ATP as driven by macronutrient meal intake and hormonal control by insulin, glucagon and epinphrine; 4 PHYSICAL ACTIVITY predicts epinephrine concentration and IL-6 as a function of exercise

  16. Validation of the Lupus Nephritis Clinical Indices in Childhood-Onset Systemic Lupus Erythematosus.

    Science.gov (United States)

    Mina, Rina; Abulaban, Khalid; Klein-Gitelman, Marisa S; Eberhard, Barbara A; Ardoin, Stacy P; Singer, Nora; Onel, Karen; Tucker, Lori; O'neil, Kathleen; Wright, Tracey; Brooks, Elizabeth; Rouster-Stevens, Kelly; Jung, Lawrence; Imundo, Lisa; Rovin, Brad; Witte, David; Ying, Jun; Brunner, Hermine I

    2016-02-01

    To validate clinical indices of lupus nephritis activity and damage when used in children against the criterion standard of kidney biopsy findings. In 83 children requiring kidney biopsy, the Systemic Lupus Erythematosus Disease Activity Index renal domain (SLEDAI-R), British Isles Lupus Assessment Group index renal domain (BILAG-R), Systemic Lupus International Collaborating Clinics (SLICC) renal activity score (SLICC-RAS), and SLICC Damage Index renal domain (SDI-R) were measured. Fixed effects and logistic models were calculated to predict International Society of Nephrology/Renal Pathology Society (ISN/RPS) class; low-to-moderate versus high lupus nephritis activity (National Institutes of Health [NIH] activity index [AI]) score: ≤10 versus >10; tubulointerstitial activity index (TIAI) score: ≤5 versus >5; or the absence versus presence of lupus nephritis chronicity (NIH chronicity index) score: 0 versus ≥1. There were 10, 50, and 23 patients with ISN/RPS class I/II, III/IV, and V, respectively. Scores of the clinical indices did not differentiate among patients by ISN/RPS class. The SLEDAI-R and SLICC-RAS but not the BILAG-R differed with lupus nephritis activity status defined by NIH-AI scores, while only the SLEDAI-R scores differed between lupus nephritis activity status based on TIAI scores. The sensitivity and specificity of the SDI-R to capture lupus nephritis chronicity was 23.5% and 91.7%, respectively. Despite being designed to measure lupus nephritis activity, SLICC-RAS and SLEDAI-R scores significantly differed with lupus nephritis chronicity status. Current clinical indices of lupus nephritis fail to discriminate ISN/RPS class in children. Despite its shortcomings, the SLEDAI-R appears best for measuring lupus nephritis activity in a clinical setting. The SDI-R is a poor correlate of lupus nephritis chronicity. © 2016, American College of Rheumatology.

  17. Diffuse Bullous Eruptions in an Elderly Woman: Late-Onset Bullous Systemic Lupus Erythematosus

    Directory of Open Access Journals (Sweden)

    Prajwal Boddu

    2016-10-01

    Full Text Available Vesiculobullous eruptions in the elderly represent a diverse range of varying pathophysiologies and can present a significant clinical dilemma to the diagnostician. Diagnosis requires a careful review of clinical history, attention to detail on physical and histomorphological examination, and appropriate immunofluorescence testing. We describe the case of a 73-year-old female who presented to our hospital with a painful blistering skin rash developed over 2 days. Examination of the skin was remarkable for numerous flaccid hemorrhagic bullae on a normal-appearing nonerythematous skin involving both the upper and lower extremities. Histopathology of the biopsy lesion showed interface change at the epidermo-dermal region with subepidermal blister formation, mild dermal fibrosis, and sparse interstitial neutrophilic infiltrate. Immunohistological analysis was significant for positive IgG basement membrane zone antibodies with a dermal pattern of localization on direct immunofluorescence and positive IgG antinuclear antibodies on indirect immunofluorescence. Evidence of antibodies to type VII collagen suggested the diagnosis of epidermolysis bullosa acquisita versus bullous systemic lupus erythematosus (BSLE. A diagnosis of BSLE was made based on positive American College of Rheumatology criteria, acquired vesiculo-bullous eruptions with compatible histopathological and immunofluorescence findings. This case illustrates one of many difficulties a physician encounters while arriving at a diagnosis from a myriad of immunobullous dermatoses. Also, it is important for internists and dermatologists alike to be aware of and differentiate this uncommon and nonspecific cutaneous SLE manifestation from a myriad of disorders presenting with vesiculobullous skin eruptions in the elderly.

  18. Swift onset of central nervous system depression and asystole followingan overdose of Guaifenesin.

    Science.gov (United States)

    Okic, Merisa; Johnson, Tom; Crifasi, Joseph A; Long, Christopher; Mitchell, Erik K

    2013-06-01

    Guaifenesin is an over-the-counter expectorant used for chest congestion and is available both in single-ingredient formulations and in combination with antihistamines, cough suppressants and decongestants. The documented side-effects of guaifenesin are generally mild. We present the case of a 23-year-old female who committed suicide by ingestion of guaifenesin along with small amounts of cetirizine, ethanol and sertraline. Approximately 2 h after ingestion, the patient experienced central nervous system depression followed by asystole. No anatomic cause of death could be determined at autopsy. The initial toxicology detected only ethanol, which was found at a concentration insufficient to cause death. Upon further analysis, guaifenesin was detected in femoral blood at 25.0 μg/mL, urine at >50.0 μg/mL, vitreous fluid at 9.2 μg/mL, brain at 17.0 μg/g and liver at 25.0 μg/g. This is the first reported human case that can be considered a death to which guaifenesin was the significant pharmacologic contributor. Guaifenesin is not detected by the primary screening methods employed by some labs and may be missed in toxicological analyses of overdoses unless specifically suspected.

  19. Late-onset Radiologic Findings of Respiratory System Following Sulfur Mustard Exposure

    Directory of Open Access Journals (Sweden)

    Mahnaz Amini

    2013-06-01

    Full Text Available Background: Sulfur mustard (SM as a chemical warfare agent, increases permeability of bronchial vessels and damages airway epithelium. SM exposure causes debilitating respiratory complications. This study was designed to evaluate clinical respiratory manifestations, and to compare chest X ray (CXR and high resolution computed tomography (HRCT scan of chest in SM exposed patients with respiratory complaints. Methods:All patients with history of SM exposure who visited Imam Reza Specialized Clinic of Respiratory Diseases from September 2001 to March 2011 were included. Patients with other comorbidities which affect respiratory system were excluded. CXR and chest HRCT scan were performed on the same day and were repeated after 5 years. Clinical and radiologic findings were collected and were compared with each other. Results: In total, 62 male patients with mean age of 53 (6.9, 41-65 were studied. Dyspnea (61 cases; 100%, dry cough (40 cases; 66%, hemoptysis (21 cases; 35% and productive cough (20 cases; 33% were the most common respiratory manifestations. Pulmonary infiltration (51; 83%, pleural thickening (25; 40% and emphysema (16; 26% were the most common findings on CXR. According to HRCT scan, pulmonary infiltration (53; 85%, bronchiolitis obliterans (38; 61% and pleural thickening (36; 58% were the most common findings (Table 2. Repeated radiologic assessments after 5 years showed a few additional findings in HRCT scan, while in about one fifth of CXRs, new pathologic findings were found. Conclusion: Patients with SM exposure experience debilitating respiratory disorders in long term. Repeating CXR in patients who present with subjective symptoms may show new findings; however, repeating HRCT scan is probably not necessary.

  20. The role of central nervous system development in late-onset neurodegenerative disorders.

    Science.gov (United States)

    Palubinsky, Amy M; Martin, Jacob A; McLaughlin, Bethann

    2012-01-01

    The human brain is dependent upon successfully maintaining ionic, energetic and redox homeostasis within exceptionally narrow margins for proper function. The ability of neurons to adapt to genetic and environmental perturbations and evoke a 'new normal' can be most fully appreciated in the context of neurological disorders in which clinical impairments do not manifest until late in life, although dysfunctional proteins are expressed early in development. We now know that proteins controlling ATP generation, mitochondrial stability, and the redox environment are associated with neurological disorders such as Parkinson's disease and amyotrophic lateral sclerosis. Generally, focus is placed on the role that early or long-term environmental stress has in altering the survival of cells targeted by genetic dysfunctions; however, the central nervous system undergoes several periods of intense stress during normal maturation. One of the most profound periods of stress occurs when 50% of neurons are removed via programmed cell death. Unfortunately, we have virtually no understanding of how these events proceed in individuals who harbor mutations that are lethal later in life. Moreover, there is a profound lack of information on circuit formation, cell fate during development and neurochemical compensation in either humans or the animals used to model neurodegenerative diseases. In this review, we consider the current knowledge of how energetic and oxidative stress signaling differs between neurons in early versus late stages of life, the influence of a new group of proteins that can integrate cell stress signals at the mitochondrial level, and the growing body of evidence that suggests early development should be considered a critical period for the genesis of chronic neurodegenerative diseases. Copyright © 2012 S. Karger AG, Basel.

  1. Young-Onset Parkinson's

    Science.gov (United States)

    ... What Is Parkinson's? › Young Onset Parkinson's Young-Onset Parkinson's 1. Symptoms 2. How Is Young-Onset PD ... of the foot Why Is Distinguishing Young-Onset Parkinson's Important? Socially, people who are affected by PD ...

  2. Penetration of Treosulfan and its Active Monoepoxide Transformation Product into Central Nervous System of Juvenile and Young Adult Rats.

    Science.gov (United States)

    Romański, Michał; Baumgart, Joachim; Böhm, Sonja; Główka, Franciszek K

    2015-12-01

    Treosulfan (TREO) is currently investigated as an alternative treatment of busulfan in conditioning before hematopoietic stem cell transplantation. The knowledge of the blood-brain barrier penetration of the drug is still scarce. In this paper, penetration of TREO and its active monoepoxide (S,S-EBDM) and diepoxide (S,S-DEB) into the CNS was studied in juvenile (JR) and young adult rats (YAR) for the first time. CD rats of both sexes (n = 96) received an intravenous dose of TREO 500 mg/kg b.wt. Concentrations of TREO, S,S-EBDM, and S,S-DEB in rat plasma, brain, and cerebrospinal fluid (CSF, in YAR only) were determined by validated bioanalytical methods. Pharmacokinetic calculations were performed in WinNonlin using a noncompartmental analysis and statistical evaluation was done in Statistica software. In male JR, female JR, male YAR, and female YAR, the brain/plasma area under the curve (AUC) ratio for unbound TREO was 0.14, 0.17, 0.10, and 0.07 and for unbound S,S-EBDM, it was 0.52, 0.48, 0.28, and 0.22, respectively. The CSF/plasma AUC ratio in male and female YAR was 0.12 and 0.11 for TREO and 0.66 and 0.64 for S,S-EBDM, respectively. Elimination rate constants of TREO and S,S-EBDM in all the matrices were sex-independent with a tendency to be lower in the JR. No quantifiable levels of S,S-DEB were found in the studied samples. TREO and S,S-EBDM demonstrated poor and sex-independent penetration into CNS. However, the brain exposure was greater in juvenile rats, so very young children might potentially be more susceptible to high-dose TREO-related CNS exposure than young adults. Copyright © 2015 by The American Society for Pharmacology and Experimental Therapeutics.

  3. The Evolution of Juvenile Schönlein-Henoch Purpura

    Directory of Open Access Journals (Sweden)

    O.E. Chernyshova

    2016-10-01

    Full Text Available Background. Hemorrhagic vasculitis, or Schönlein-Henoch purpura (SHP, is the most common type of systemic vasculitis in childhood, and peculiarities of the further evolution of the pathological process in adult patients remain unexplored. Objective: to study the evolution of juvenile SHP, comparing the nature of lesions of the skin, joints, heart and kidneys in patients in childhood and adulthood. Material and methods. The study included 92 patients (61 men and 31 women with the average age of 27 years, and the average age of disease onset — 11 years. I degree of the activity of the pathological process is determined in 40 % of cases, II — in 35 %, III — in 25 %. Seropositivity by hyperimmunoglobulinemia A occurred in 27 % of cases, by the presence of rheumatoid factor — in 21 %. At the time of the survey, cutaneous syndrome was diagnosed in 55 % of patients, joint one — in 45 %, kidney one — in 71 %. Renal biopsy was performed in 15 cases. Results. The cutaneous, cutaneous-joint-abdominal and cutaneous-abdominal-renal forms of the disease, lesions of the skin, gastrointestinal tract, wrist, ankle and knee joints become rarer in the process of juvenile SHP evolution, but exceptionally renal variant of the pathological process, changes of skeletal muscles, liver, spleen and the heart are revealed more often, chronic kidney disease with the renal failure progression is developed in 12 % of patients (in 17 % of cases of nephropathy, sacroiliitis, spondylopathy, tendovaginitis, enthesopathies, epiphyseal osteoporosis and meniscitis of the knee joints are arisen, II, III, VI and IV morphological classes of Henoch glomerulonephritis are formed in a ratio of 8 : 4 : 2 : 1 with tubulointerstitial component in all cases, and lymphohistiocytic infiltration of the vascular wall is the unfavorable sign for the prognosis of the disease. Conclusions. In cases of transition of juvenile SHP into the chronic adult form, the disease often obtain

  4. Late-Onset Asthma

    DEFF Research Database (Denmark)

    Ulrik, Charlotte Suppli

    2017-01-01

    the risk of systemic effects. However, most recommendations are based on extrapolation from findings in younger patients. Comorbidities are very common in patients with late-onset asthma and need to be taken into account in the management of the disease. In conclusion, late-onset asthma is poorly......Late-onset asthma is common, associated with poor outcome, underdiagnosed and undertreated, possibly due to the modifying effect of ageing on disease expression. Although the diagnostic work-up in elderly individuals suspected of having asthma follows the same steps as in younger individuals (case...... history and spirometry), it is important to acknowledge that elderly individuals are likely to have diminished bronchodilator reversibility and some degree of fixed airflow obstruction. Elderly individuals, therefore, often require further objective tests, including bronchial challenge testing...

  5. Integrated Systems Approach Reveals Sphingolipid Metabolism Pathway Dysregulation in Association with Late-Onset Alzheimer’s Disease

    Directory of Open Access Journals (Sweden)

    John Stephen Malamon

    2018-02-01

    Full Text Available Late-onset Alzheimer’s disease (LOAD and age are significantly correlated such that one-third of Americans beyond 85 years of age are afflicted. We have designed and implemented a pilot study that combines systems biology approaches with traditional next-generation sequencing (NGS analysis techniques to identify relevant regulatory pathways, infer functional relationships and confirm the dysregulation of these biological pathways in LOAD. Our study design is a most comprehensive systems approach combining co-expression network modeling derived from RNA-seq data, rigorous quality control (QC standards, functional ontology, and expression quantitative trait loci (eQTL derived from whole exome (WES single nucleotide variant (SNV genotype data. Our initial results reveal several statistically significant, biologically relevant genes involved in sphingolipid metabolism. To validate these findings, we performed a gene set enrichment analysis (GSEA. The GSEA revealed the sphingolipid metabolism pathway and regulation of autophagy in association with LOAD cases. In the execution of this study, we have successfully tested an integrative approach to identify both novel and known LOAD drivers in order to develop a broader and more detailed picture of the highly complex transcriptional and regulatory landscape of age-related dementia.

  6. Performance of the 2012 Systemic Lupus International Collaborating Clinics classification criteria versus the 1997 American College of Rheumatology classification criteria in adult and juvenile systemic lupus erythematosus. A systematic review and meta-analysis.

    Science.gov (United States)

    Hartman, Esther A R; van Royen-Kerkhof, Annet; Jacobs, Johannes W G; Welsing, Paco M J; Fritsch-Stork, Ruth D E

    2018-03-01

    To evaluate the performance in classifying systemic lupus erythematosus by the 2012 Systemic Lupus International Collaborating Clinics criteria (SLICC'12), versus the revised American College of Rheumatology criteria from 1997 (ACR'97) in adult and juvenile SLE patients. A systematic literature search was conducted in PubMed and Embase for studies comparing SLICC'12 and ACR'97 with clinical diagnosis. A meta-analysis was performed to estimate the sensitivity and specificity of SLICC'12 and ACR'97. To assess classification earlier in the disease by either set, sensitivity and specificity were compared for patients with disease duration <5years. Sensitivity and specificity of individual criteria items were also assessed. In adult SLE (nine studies: 5236 patients, 1313 controls), SLICC'12 has higher sensitivity (94.6% vs. 89.6%) and similar specificity (95.5% vs. 98.1%) compared to ACR'97. For juvenile SLE (four studies: 568 patients, 339 controls), SLICC'12 demonstrates higher sensitivity (99.9% vs. 84.3%) than ACR'97, but much lower specificity (82.0% vs. 94.1%). SLICC'12 classifies juvenile SLE patients earlier in disease course. Individual items contributing to diagnostic accuracy are low complement, anti-ds DNA and acute cutaneous lupus in SLICC'12, and the immunologic and hematologic disorder in ACR'97. Based on sensitivity and specificity SLICC'12 is best for adult SLE. Following the view that higher specificity, i.e. avoidance of false positives, is preferable, ACR'97 is best for juvenile SLE even if associated with lower sensitivity. Our results on the contribution of the individual items of SLICC'12 and ACR´97 may be of value in future efforts to update classification criteria. Copyright © 2018 The Authors. Published by Elsevier B.V. All rights reserved.

  7. Ventilation onset prior to umbilical cord clamping (physiological-based cord clamping improves systemic and cerebral oxygenation in preterm lambs.

    Directory of Open Access Journals (Sweden)

    Graeme R Polglase

    Full Text Available As measurement of arterial oxygen saturation (SpO2 is common in the delivery room, target SpO2 ranges allow clinicians to titrate oxygen therapy for preterm infants in order to achieve saturation levels similar to those seen in normal term infants in the first minutes of life. However, the influence of the onset of ventilation and the timing of cord clamping on systemic and cerebral oxygenation is not known.We investigated whether the initiation of ventilation, prior to, or after umbilical cord clamping, altered systemic and cerebral oxygenation in preterm lambs.Systemic and cerebral blood-flows, pressures and peripheral SpO2 and regional cerebral tissue oxygenation (SctO2 were measured continuously in apnoeic preterm lambs (126±1 day gestation. Positive pressure ventilation was initiated either 1 prior to umbilical cord clamping, or 2 after umbilical cord clamping. Lambs were monitored intensively prior to intervention, and for 10 minutes following umbilical cord clamping.Clamping the umbilical cord prior to ventilation resulted in a rapid decrease in SpO2 and SctO2, and an increase in arterial pressure, cerebral blood flow and cerebral oxygen extraction. Ventilation restored oxygenation and haemodynamics by 5-6 minutes. No such disturbances in peripheral or cerebral oxygenation and haemodynamics were observed when ventilation was initiated prior to cord clamping.The establishment of ventilation prior to umbilical cord clamping facilitated a smooth transition to systemic and cerebral oxygenation following birth. SpO2 nomograms may need to be re-evaluated to reflect physiological management of preterm infants in the delivery room.

  8. Ventilation onset prior to umbilical cord clamping (physiological-based cord clamping) improves systemic and cerebral oxygenation in preterm lambs.

    Science.gov (United States)

    Polglase, Graeme R; Dawson, Jennifer A; Kluckow, Martin; Gill, Andrew W; Davis, Peter G; Te Pas, Arjan B; Crossley, Kelly J; McDougall, Annie; Wallace, Euan M; Hooper, Stuart B

    2015-01-01

    As measurement of arterial oxygen saturation (SpO2) is common in the delivery room, target SpO2 ranges allow clinicians to titrate oxygen therapy for preterm infants in order to achieve saturation levels similar to those seen in normal term infants in the first minutes of life. However, the influence of the onset of ventilation and the timing of cord clamping on systemic and cerebral oxygenation is not known. We investigated whether the initiation of ventilation, prior to, or after umbilical cord clamping, altered systemic and cerebral oxygenation in preterm lambs. Systemic and cerebral blood-flows, pressures and peripheral SpO2 and regional cerebral tissue oxygenation (SctO2) were measured continuously in apnoeic preterm lambs (126±1 day gestation). Positive pressure ventilation was initiated either 1) prior to umbilical cord clamping, or 2) after umbilical cord clamping. Lambs were monitored intensively prior to intervention, and for 10 minutes following umbilical cord clamping. Clamping the umbilical cord prior to ventilation resulted in a rapid decrease in SpO2 and SctO2, and an increase in arterial pressure, cerebral blood flow and cerebral oxygen extraction. Ventilation restored oxygenation and haemodynamics by 5-6 minutes. No such disturbances in peripheral or cerebral oxygenation and haemodynamics were observed when ventilation was initiated prior to cord clamping. The establishment of ventilation prior to umbilical cord clamping facilitated a smooth transition to systemic and cerebral oxygenation following birth. SpO2 nomograms may need to be re-evaluated to reflect physiological management of preterm infants in the delivery room.

  9. The Pediatric Rheumatology International Trials Organization/American College of Rheumatology provisional criteria for the evaluation of response to therapy in juvenile systemic lupus erythematosus: prospective validation of the definition of improvement

    NARCIS (Netherlands)

    Ruperto, Nicolino; Ravelli, Angelo; Oliveira, Sheila; Alessio, Maria; Mihaylova, Dimitrina; Pasic, Srdjan; Cortis, Elisabetta; Apaz, Maria; Burgos-Vargas, Ruben; Kanakoudi-Tsakalidou, Florence; Norambuena, Ximena; Corona, Fabrizia; Gerloni, Valeria; Hagelberg, Stefan; Aggarwal, Amita; Dolezalova, Pavla; Saad, Claudia Magalhaes; Bae, Sang-Cheol; Vesely, Richard; Avcin, Tadej; Foster, Helen; Duarte, Carolina; Herlin, Troels; Horneff, Gerd; Lepore, Loredana; van Rossum, Marion; Trail, Lucia; Pistorio, Angela; Andersson-Gäre, Boel; Giannini, Edward H.; Martini, Alberto

    2006-01-01

    To use the Pediatric Rheumatology International Trials Organization (PRINTO) core set of outcome measures to develop a validated definition of improvement for the evaluation of response to therapy in juvenile systemic lupus erythematosus (SLE). Thirty-seven experienced pediatric rheumatologists from

  10. INFLIXIMAB IN TREATMENT OF REFRACTORY JUVENILE ARTHRITIS

    Directory of Open Access Journals (Sweden)

    I.P. Nikishina

    2010-01-01

    Full Text Available Inclusion to the clinical practice of genetically engineered biological medications opens new opportunities in treatment of juvenile arthritis. The article summarizes an experience of treatment of juvenile arthritis with infliximab in children’s department of Scientific Center of Rheumatology, Russian Academy of Medical Sciences. The analysis included 55 patients (16 children had systemic type, 23 — polyarticular type of juvenile arthritis, and 16 patients had juvenile spondylarthritis, treated with infliximab in 2002–2009 years. Infliximab was administrated in patients with high activity of the disease refractory to the modern basic therapy. Patients received intravenous infliximab 3–5 mg/kg daily according to the standard scheme (on 0, 2, 6 weeks and further every 8 weeks. Therapy with this drug was estimated as effective (improvement on 30% and more according ACRpedi in 80% of patients: 16% achieved ACR30, 29% — ACR50, 26% — ACR70, and 9% — ACR90. Unfavorable effects (infusion reactions were detected in 16% of cases. Infections, including one case of disseminated tuberculosis, developed in 20% of patients. Thus, treatment with infliximab is effective and has good «risk–benefit» ratio in treatment of patients with refractory juvenile arthritis. Key words: children, juvenile arthritis, infliximab, treatment.(Voprosy sovremennoi pediatrii — Current Pediatrics. 2010;9(1:142-149

  11. Juvenile Sex Offenders.

    Science.gov (United States)

    Ryan, Eileen P; Otonichar, Joseph M

    2016-07-01

    Sexual offending by juveniles accounts for a sizable percentage of sexual offenses, especially against young children. In this article, recent research on female juvenile sex offenders (JSOs), risk factors for offending in juveniles, treatment, and the ways in which these youth may differ from general delinquents will be reviewed. Most JSOs do not go on to develop paraphilic disorders or to commit sex offenses during adulthood, and as a group, they are more similar to nonsexual offending juvenile delinquents than to adult sex offenders. Recent research has elucidated some differences between youth who commit sex offenses and general delinquents in the areas of atypical sexual interests, the use of pornography, and early sexual victimization during childhood.

  12. Juvenile Rockfish Recruitment Cruise

    Data.gov (United States)

    National Oceanic and Atmospheric Administration, Department of Commerce — In 1983, the groundfish analysis project began a series of yearly cruises designed to assess the annual abundance of juvenile rockfish along the central California...

  13. Parenting and juvenile delinquency

    OpenAIRE

    Hoeve, M.

    2008-01-01

    Juvenile delinquency is a noteworthy problem. This thesis addressed the association between parenting and juvenile delinquency by analyzing the concepts of parenting adopted in family research in relation to criminological concepts and measures of delinquent behavior. Four studies were conducted. The first study addressed a meta-analysis on parenting characteristics and styles in relation to delinquency. In this meta-analysis, previous manuscripts were systematically analyzed, computing mean ...

  14. Juvenile polyposis syndrome

    OpenAIRE

    Hsiao, Yi-Han; Wei, Chin-Hung; Chang, Szu-Wen; Chang, Lung; Fu, Yu-Wei; Lee, Hung-Chang; Liu, Hsuan-Liang; Yeung, Chun-Yan

    2016-01-01

    Abstract Background: Juvenile polyposis syndrome, a rare disorder in children, is characterized with multiple hamartomatous polyps in alimentary tract. A variety of manifestations include bleeding, intussusception, or polyp prolapse. In this study, we present an 8-month-old male infant of juvenile polyposis syndrome initially presenting with chronic anemia. To the best of our knowledge, this is the youngest case reported in the literature. Methods: We report a rare case of an 8-month-old male...

  15. Functional and structural changes throughout the auditory system following congenital and early-onset deafness: implications for hearing restoration

    Science.gov (United States)

    Butler, Blake E.; Lomber, Stephen G.

    2013-01-01

    The absence of auditory input, particularly during development, causes widespread changes in the structure and function of the auditory system, extending from peripheral structures into auditory cortex. In humans, the consequences of these changes are far-reaching and often include detriments to language acquisition, and associated psychosocial issues. Much of what is currently known about the nature of deafness-related changes to auditory structures comes from studies of congenitally deaf or early-deafened animal models. Fortunately, the mammalian auditory system shows a high degree of preservation among species, allowing for generalization from these models to the human auditory system. This review begins with a comparison of common methods used to obtain deaf animal models, highlighting the specific advantages and anatomical consequences of each. Some consideration is also given to the effectiveness of methods used to measure hearing loss during and following deafening procedures. The structural and functional consequences of congenital and early-onset deafness have been examined across a variety of mammals. This review attempts to summarize these changes, which often involve alteration of hair cells and supporting cells in the cochleae, and anatomical and physiological changes that extend through subcortical structures and into cortex. The nature of these changes is discussed, and the impacts to neural processing are addressed. Finally, long-term changes in cortical structures are discussed, with a focus on the presence or absence of cross-modal plasticity. In addition to being of interest to our understanding of multisensory processing, these changes also have important implications for the use of assistive devices such as cochlear implants. PMID:24324409

  16. Síndrome de ativação macrofágica em paciente com lúpus eritematoso sistêmico juvenil Macrophage activation syndrome in a patient with juvenile systemic lupus erythematosus

    OpenAIRE

    Simone Manso de Carvalho; João Henrique do Amaral e Silva; Juliana de Oliveira Sato; Maria Aparecida Custódio Domingues; Claudia Saad Magalhães

    2008-01-01

    A hemofagocitose reativa ou síndrome de ativação macrofágica (SAM) é uma complicação das doenças inflamatórias sistêmicas, causada por expansão de células T e macrófagos, com produção maciça de citocinas pró-inflamatórias, ocorrendo mais freqüentemente na artrite idiopática juvenil sistêmica e raramente no lúpus eritematoso sistêmico juvenil (LESJ). OBJETIVO: Relatar um caso de LESJ que evoluiu com SAM precipitada por infecção e infarto esplênico, com desfecho fatal. RELATO DE CASO: Uma menin...

  17. Mechanisms of diabetic autoimmunity: I--the inductive interface between islets and the immune system at onset of inflammation.

    Science.gov (United States)

    Askenasy, Nadir

    2016-04-01

    The mechanisms of autoimmune reactivity onset in type 1 diabetes (T1D) remain elusive despite extensive experimentation and discussion. We reconsider several key aspects of the early stages of autoimmunity at four levels: islets, pancreatic lymph nodes, thymic function and peripheral immune homeostasis. Antigen presentation is the islets and has the capacity to provoke immune sensitization, either in the process of physiological neonatal β cell apoptosis or as a consequence of cytolytic activity of self-reactive thymocytes that escaped negative regulation. Diabetogenic effectors are efficiently expanded in both the islets and the lymph nodes under conditions of empty lymphoid niches during a period of time coinciding with a synchronized wave of β cell apoptosis surrounding weaning. A major drive of effector cell activation and expansion is inherent peripheral lymphopenia characteristic of neonates, though it remains unclear when is autoimmunity triggered in subjects displaying hyperglycemia in late adolescence. Our analysis suggests that T1D evolves through coordinated activity of multiple physiological mechanisms of stimulation within specific characteristics of the neonate immune system.

  18. The reality of teenage driving: the results of a driving educational experience for teens in the juvenile court system.

    Science.gov (United States)

    Manno, Mariann; Maranda, Louise; Rook, Allison; Hirschfeld, Ryan; Hirsh, Michael

    2012-10-01

    In the United States, one third of all deaths in teens are a result of motor vehicle crashes, accounting for 6,000 deaths annually. Injury Free Coalition for Kids-Worcester in collaboration with Worcester Juvenile Court has developed an interactive program for first-time teenaged driving offenders, Reality Intensive Driver Education (Teen RIDE). This full-day program at the trauma center provides a realistic exposure to the consequences of risky driving behaviors. This article examined the driving offense recidivism rates for Teen RIDE participants versus a comparison group (CG). The intervention group (IG) consists of teenagers between 13 years and 17 years who have been arrested for the first time for a serious driving offense and are sentenced by a Worcester Juvenile Court Judge or Magistrate to the Teen RIDE program. They are required to attend the program as a condition of probation, so attendance is mandatory. Each participant in the IG completed the program and was tracked for driving reoffenses for 6 months after completion of the course. The CG consists of also first-time driving offenders. The CG was matched with the IG with respect to age (13-17 years), sex, and offense type. Springfield, Massachusetts, serves as the site for recruitment of the CG, since it is demographically similar to Worcester but 60 mi away. Students in the CG had no exposure to this program. Each CG member was also tracked for 6 months after arrest. The recidivism rate for Teen RIDE participants 6 months after the course is 6% with 0% reoffending more than once. The CG has a recidivism rate of 56% 6 months after the arrest and 14% have more than one reoffense. The CG is 13.062 (4.296-39.713) times more likely to reoffend, and this is significant (p Teen RIDE program provides an impactful exposure of the consequences of risky driving behaviors to teenaged participants. In addition, Teen RIDE participants are significantly less likely to reoffend after completion of the course

  19. Very late-onset group B Streptococcus meningitis, sepsis, and systemic shigellosis due to interleukin-1 receptor-associated kinase-4 deficiency.

    Science.gov (United States)

    Krause, Jens C; Ghandil, Pegah; Chrabieh, Maya; Casanova, Jean-Laurent; Picard, Capucine; Puel, Anne; Creech, C Buddy

    2009-11-01

    We describe a child with very late-onset group B Streptococcus sepsis and meningitis, systemic shigellosis, and chronic osteomyelitis. Peripheral blood cells obtained from the patient and her brother did not respond to stimulation with either interleukin-1beta or lipopolysaccharide. Sequencing of the interleukin-1 receptor-associated kinase-4 gene revealed 2 novel mutations.

  20. History of Juvenile Arrests and Vocational Career Outcomes for At-Risk Young Men

    Science.gov (United States)

    Wiesner, Margit; Kim, Hyoun K.; Capaldi, Deborah M.

    2009-01-01

    This study used longitudinal data from the Oregon Youth Study (OYS) to examine prospective effects of juvenile arrests, and of early versus late onset of juvenile offending, on two labor market outcomes by age 29/30 years. It was expected that those with more juvenile arrests and those with an early onset of offending would show poorer outcomes on both measures, controlling for propensity factors. Data were available for 203 men from the OYS, including officially recorded arrests and self-reported information on the men's work history across 9 years. Analyses revealed unexpected specificity in prospective effects: Juvenile arrests and mental health problems predicted the number of months unemployed; in contrast, being fired from work was predicted by poor child inhibitory control and adolescent substance use. Onset age of offending did not significantly predict either outcome. Implications of the findings for applied purposes and for developmental taxonomies of crime are discussed. PMID:20448840

  1. Metric Characters and Body Indices of Juvenile Sterlet Acipenser ruthenus Raised in a Recirculating Aquaculture System (RAS

    Directory of Open Access Journals (Sweden)

    Mărioara Nicula

    2015-05-01

    Full Text Available Our research was performed on juvenile sterlets (Acipenser ruthenus, aged between 346-431 days, maintained in five round pools (Ø=3 m, V=6 m3, h-100 cm and fed with a commercial extruded pellet (3 mm granulation, 48% CP, and 10% EE. Body weight and total body length on 30 individuals from each tank were determined every two weeks. Weighing and somatic measurements achieved at the end of experimental period (standard length-l, body weight-G, maximum body high–H, maximum body perimeter (Pmax, head length–lh,, caudal peduncle length-lp allowed us to calculate the following body indices: profile index, thickness index, Fulton index, Kiselev index, meat index. Statistical analysis was performed by using the SPPS IBM 22 and Minitab 16 software. Data were reported as Mean±Sx at a significant level of p<0.05. Testing differences between means was realized by ANOVA completed with post-hoc Tukey test. Results obtained led to conclude that specimens analyzed had a good maintenance and feeding, an adequate development, a high proportion of meat, an elongated body and a straight back, and they are eligible for selection.

  2. Smoking-specific parenting and smoking onset in adolescence: the role of genes from the dopaminergic system (DRD2, DRD4, DAT1 genotypes.

    Directory of Open Access Journals (Sweden)

    Marieke Hiemstra

    Full Text Available Although only few studies have shown direct links between dopaminergic system genes and smoking onset, this does not rule out the effect of a gene-environment interaction on smoking onset. Therefore, the aim of this study was to examine the associations between smoking-specific parenting (i.e., frequency and quality of communication and house rules and smoking onset while considering the potential moderating role of dopaminergic system genes (i.e., DRD2, DRD4, and DAT1 genotypes. Data from five annual waves of the 'Family and Health' project were used. At time 1, the sample comprised 365 non-smoking adolescents (200 younger adolescents, mean age = 13.31, SD = .48; 165 older adolescents, mean age = 15.19, SD = .57. Advanced longitudinal analyses were used (i.e., logistic regression analyses, (dual latent growth curves, and cross-lagged path models. The results showed a direct effect of quality of communication on smoking onset. No direct effects were found for frequency of communication and house rules. Furthermore, no direct and moderating effects of the DRD2, DRD4, or DAT1 genotypes were found. In conclusion, the findings indicated that the effects of smoking-specific parenting on smoking are similar for adolescent carriers and non-carriers of the dopaminergic system genes.

  3. Fetal and juvenile radiotoxicity

    International Nuclear Information System (INIS)

    Sikov, M.R.

    1983-01-01

    Comparative information on the deposition, distribution, retention, and toxicity of radionuclides in the prenatal and juvenile mammal is reported. Emphasis is toward establishing patterns, phenomenologic interactions, and relationships which will be useful in determining appropriate exposure levels for the rapidly growing infant or child and for pregnant women. Recent results have shown that injection of pregnant rats with 239 Pu increases the incidence and severity of adenomatous hyperplasia of the liver in the offspring; the magnitude of these effects is relatd to dose and prenatal age at exposure. Analysis of combined data from several experiments leads to the conclusion that perinatal rats are more sensitive to bone tumor induction by 239 Pu alpha-particle irradiation than are adults. Further histopathologic evaluations of material from earlier experiments have demonstrated that most of the increased incidence of thyroid tumors following 131 I exposure is attributable to follicular tumors. An analysis of the literature led to the conclusion that prenatal irradiation can lead to an increased or decreased incidence of tumors, depending on the specific details of the experimental design and system

  4. [Bone mineral density in juvenile-onset diabetes mellitus].

    Science.gov (United States)

    Viña Simón, E; Bueno Lozano, G; Armadá Maresca, M I; Ruibal Francisco, J L; Fernández Pérez, C; Lozano Tonkin, C; Casado de Frías, E

    2000-06-01

    To determine bone mineral density (BMD) at axial and appendicular sites in patients with type 1 diabetes mellitus and evaluate its relationship with metabolic control and disease duration. BMD was measured by dual-energy X-ray absorptiometry (Hologic QDR-1000) in the lumbar spine (L1-L4) and at the distal third forearm in 246 healthy non-diabetic children and adolescents (111 boys, 135 girls, aged 2.8-20.8 years) and in 45 diabetic patients (18 boys, 27 girls, aged 5.2-19.4 years). The results were expressed as the mean and standard deviation. The differences were tested by analysis of variance or Students t-test, as appropriate. The relationship between BMD and the remaining variables was studied by simple Pearsons coefficient and partial correlation coefficient. Significance was defined as p pubertal spurt. The greatest differences were found in males and in the trabecular bone. No relationship was found between metabolic control (mean glycosylated hemoglobin, insulin requirement) and duration of diabetes and the BMD in any region studied (p > 0.05). Pediatric patients with type 1 diabetes mellitus appear to constitute a population at risk of osteoporosis in adult-hood. Diagnosis of osteopenia diagnosis should be established according to densitometric criteria. In this study, metabolic control parameters and duration of diabetes did not enable predetermination of diabetic patients at risk of osteoporosis in adulthood.

  5. Influence of Environmental Conditions in Ichthyophthiriasis Trigger to the Europeans Catfish Juveniles (Silurus Glanis Stocked into a Production System with Partially Reused Water

    Directory of Open Access Journals (Sweden)

    Sandita (Placinta Ion

    2011-10-01

    Full Text Available Currently, in Romanian aquaculture, the rearing of the European catfish into intensive production systems raises a growing interest determined by its higher technological performances. In comparison with pond traditional aquaculture, the new farming systems are characterized by modified environmental conditions that may lead, in case of exceeding thresholds of tolerability, to occurrence of stress factors, thus setting the disease conditions. From this perspective, the purpose of this paper is to present the association circumstances of the predisposed environmental conditions that have weakened the fish body resistance promoting the pathological action of Ichthyophthirius multifiliis after the transfer of European catfish juveniles (G=33.6±1.7 g, L=17.5±2.9 cm from a pond system into a tank culture system with partially reused water. During a 21-day period, some restrictive parameters of water quality have registered values far beyond the optimum interval for wells juveniles growth (t≤16oC, CmaxN-NH4+=3.62mg/l, CmaxNO2-=9.18mg/l, CmaxCl2=0.1mg/l which caused the appearance of some characteristic symptoms, such as the gills brown coloration, masking the first symptoms of ichthyophthiriasis and contributing to the subclinical evolution of the disease. The concentrations of water chemical parameters were determined by spectrophotometer methods using Merck kits, analogous to standard methods. Subsequently, the “ich” diagnosis was confirmed by microscopic examination of the gills and skin scrapings highlighting the presence of parasites. In order to alleviate methaemoglobinaemia and parasite treatment, sodium chloride was gradually added in water till 1 ppt concentration. Maintaining the salinity of the system led to a reduction of gills parasitic intensity, while mature trophonts encapsulated in the skin were protected by mucus hypersecretion. Finally, the destruction of gill epithelium seriously affected the osmoregulation

  6. Postpartum Circulating Markers of Inflammation and the Systemic Acute-Phase Response After Early-Onset Preeclampsia.

    Science.gov (United States)

    van Rijn, Bas B; Bruinse, Hein W; Veerbeek, Jan H; Post Uiterweer, Emiel D; Koenen, Steven V; van der Bom, Johanna G; Rijkers, Ger T; Roest, Mark; Franx, Arie

    2016-02-01

    Preeclampsia is an inflammatory-mediated hypertensive disorder of pregnancy and seems to be an early indicator of increased cardiovascular risk, but mechanisms underlying this association are unclear. In this study, we identified levels of circulating inflammatory markers and dynamic changes in the systemic acute-phase response in 44 women with a history of severe early-onset preeclampsia, compared with 29 controls with only uneventful pregnancies at 1.5 to 3.5 years postpartum. Models used were in vivo seasonal influenza vaccination and in vitro whole-blood culture with T-cell stimulants and the toll-like receptor-4 ligand lipopolysaccharide. Outcome measures were C-reactive protein, interleukin-6 (IL-6), IL-18, fibrinogen, myeloperoxidase, and a panel of 13 cytokines representative of the innate and adaptive inflammatory response, in addition to established cardiovascular markers. The in vivo acute-phase response was higher for women with previous preeclampsia than that for controls without such a history, although only significant for C-reactive protein (P=0.04). Preeclampsia was associated with higher IL-1β (Ppreeclampsia: an adaptive response cluster associated with increased C-reactive protein and IL-6 before and after vaccination, increased weight, and low high-density lipoprotein cholesterol; and a toll-like receptor-4 mediated the cluster associated with increased IL-18 before and after vaccination but not associated with other cardiovascular markers. Furthermore, we found interactions between previous preeclampsia, common TLR4 gene variants, and the IL-18 response to vaccination. In conclusion, preeclampsia is associated with alterations in the inflammatory response postpartum mostly independent of other established cardiovascular risk markers. © 2015 American Heart Association, Inc.

  7. Stevens-Johnson syndrome and toxic epidermal necrolysis in childhood-onset systemic lupus erythematosus patients: a multicenter study

    Directory of Open Access Journals (Sweden)

    Ana Paula Sakamoto

    2017-07-01

    Full Text Available Objective: To assess Stevens-Johnson syndrome (SJS and toxic epidermal necrolysis (TEN in a large population of childhood-onset systemic lupus erythematosus (cSLE patients. Methods: Multicenter study including 852 cSLE patients followed in Pediatric Rheumatology centers in São Paulo, Brazil. SJS was defined as epidermal detachment below 10% of body surface area (BSA, overlap SJS-TEN 10-30% and TEN greater than 30% of BSA. Results: SJS and TEN was observed in 5/852 (0.6% cSLE female patients, three patients were classified as SJS and two patients were classified as overlap SJS-TEN; TEN was not observed. The mean duration of SJS and overlap SJS-TEN was 15 days (range 7-22 and antibiotics induced four cases. Regarding extra-cutaneous manifestations, hepatomegaly was observed in two cSLE patients, nephritis in two and neuropsychiatric involvement and conjunctivitis were observed respectively in one patient. Hematological involvement included lymphopenia in four, leucopenia in three and thrombocytopenia in two patients. The mean SLEDAI-2K score was 14.8 (range 6-30. Laboratory analysis showed low C3, C4 and/or CH50 in two patients and the presence of anti-dsDNA autoantibody in two patients. One patient had lupus anticoagulant and another one had anticardiolipin IgG. All patients were treated with steroids and four needed additional treatment such as intravenous immunoglobulin in two patients, hydroxychloroquine and azathioprine in two and intravenous cyclophosphamide in one patient. Sepsis was observed in three cSLE patients. Two patients required intensive care and death was observed in one patient. Conclusion: Our study identified SJS and overlap SJS-TEN as rare manifestations of active cSLE associated with severe multisystemic disease, with potentially lethal outcome.

  8. European evidence-based recommendations for diagnosis and treatment of childhood-onset systemic lupus erythematosus: the SHARE initiative.

    Science.gov (United States)

    Groot, Noortje; de Graeff, Nienke; Avcin, Tadej; Bader-Meunier, Brigitte; Brogan, Paul; Dolezalova, Pavla; Feldman, Brian; Kone-Paut, Isabelle; Lahdenne, Pekka; Marks, Stephen D; McCann, Liza; Ozen, Seza; Pilkington, Clarissa; Ravelli, Angelo; Royen-Kerkhof, Annet van; Uziel, Yosef; Vastert, Bas; Wulffraat, Nico; Kamphuis, Sylvia; Beresford, Michael W

    2017-11-01

    Childhood-onset systemic lupus erythematosus (cSLE) is a rare, multisystem and potentially life-threatening autoimmune disorder with significant associated morbidity. Evidence-based guidelines are sparse and management is often based on clinical expertise. SHARE (Single Hub and Access point for paediatric Rheumatology in Europe) was launched to optimise and disseminate management regimens for children and young adults with rheumatic diseases like cSLE. Here, we provide evidence-based recommendations for diagnosis and treatment of cSLE. In view of extent and complexity of cSLE and its various manifestations, recommendations for lupus nephritis and antiphospholipid syndrome will be published separately. Recommendations were generated using the EULAR (European League Against Rheumatism) standard operating procedure. An expert committee consisting of paediatric rheumatologists and representation of paediatric nephrology from across Europe discussed evidence-based recommendations during two consensus meetings. Recommendations were accepted if >80% agreement was reached. A total of 25 recommendations regarding key approaches to diagnosis and treatment of cSLE were made. The recommendations include 11 on diagnosis, 9 on disease monitoring and 5 on general treatment. Topics included: appropriate use of SLE classification criteria, disease activity and damage indices; adequate assessment of autoantibody profiles; secondary macrophage activation syndrome; use of hydroxychloroquine and corticosteroid-sparing regimens; and the importance of addressing poor adherence. Ten recommendations were accepted regarding general diagnostic strategies and treatment indications of neuropsychiatric cSLE. The SHARE recommendations for cSLE and neuropsychiatric manifestations of cSLE have been formulated by an evidence-based consensus process to support uniform, high-quality standards of care for children with cSLE. © Article author(s) (or their employer(s) unless otherwise stated in the

  9. Poor muscle strength and function in physically inactive childhood-onset systemic lupus erythematosus despite very mild disease

    Directory of Open Access Journals (Sweden)

    Ana Jéssica Pinto

    Full Text Available ABSTRACT Objective: To compare muscle strength (i.e. lower- and upper-body strength and function between physically inactive childhood-onset systemic lupus erythematosus patients (C-SLE and healthy controls (CTRL. Methods: This was a cross-sectional study and the sample consisted of 19 C-SLE (age between 9 and 18 years and 15 CTRL matched by age, sex, body mass index (BMI, and physical activity levels (assessed by accelerometry. Lower- and upper-body strength was assessed by the one-repetition-maximum (1-RM test. Isometric strength was assessed through a handgrip dynamometer. Muscle function was evaluated by the timed-stands test (TST and the timed-up-and-go test (TUG. Results: When compared with CTRL, C-SLE showed lower leg-press and bench-press 1-RM (p = 0.026 and p = 0.008, respectively, and a tendency toward lower handgrip strength (p = 0.052. C-SLE showed lower TST scores (p = 0.036 and a tendency toward higher TUG scores (p = 0.070 when compared with CTRL. Conclusion: Physically inactive C-SLE patients with very mild disease showed reduced muscle strength and functionality when compared with healthy controls matched by physical activity levels. These findings suggest C-SLE patients may greatly suffer from a physically inactive lifestyle than healthy controls do. Moreover, some sub-clinical “residual” effect of the disease or its pharmacological treatment seems to affect C-SLE patients even with a well-controlled disease.

  10. Effects of Juvenile Court Exposure on Crime in Young Adulthood

    Science.gov (United States)

    Petitclerc, Amelie; Gatti, Uberto; Vitaro, Frank; Tremblay, Richard E.

    2013-01-01

    Background: The juvenile justice system's interventions are expected to help reduce recidivism. However, previous studies suggest that official processing in juvenile court fails to reduce adolescents' criminal behavior in the following year. Longer term effects have not yet been investigated with a rigorous method. This study used propensity…

  11. Juvenile Crime and Criminal Justice: Resolving Border Disputes

    Science.gov (United States)

    Fagan, Jeffrey

    2008-01-01

    Rising juvenile crime rates during the 1970s and 1980s spurred state legislatures across the country to exclude or transfer a significant share of offenders under the age of eighteen to the jurisdiction of the criminal court, essentially redrawing the boundary between the juvenile and adult justice systems. Jeffrey Fagan examines the legal…

  12. Suicidal Ideation and Behaviors among Youths in Juvenile Detention

    Science.gov (United States)

    Abram, Karen M.; Choe, Jeanne Y.; Washburn, Jason J.; Teplin, Linda A.; King, Devon C.; Dulcan, Mina K.

    2008-01-01

    An epidemiological study used data from the Northwestern Juvenile Project to examine the association between psychiatric diagnosis and suicide risk among newly detained youths in the US juvenile system. Results concluded that psychiatric disorders were associated with suicide attempts, and females were found at a higher risk than males.

  13. Young Adult Outcomes of Juvenile Court-Involved Girls.

    Science.gov (United States)

    Bright, Charlotte Lyn; Jonson-Reid, Melissa

    2010-01-01

    The recent increase in the number of girls involved in the juvenile justice system has resulted in increased academic and public attention. Thus far, this attention has focused on entry into the juvenile justice system rather than longer-term consequences. This research helps fill this gap by examining a sample of 700 maltreated and/or impoverished juvenile court-involved females. Competing risks models were used to control for time from juvenile-court entry to adult outcomes: criminal justice system involvement, use of public mental health or substance use services, and income maintenance use. Results indicate that there are distinct predictors associated with the different outcomes, although learning disability and adolescent parenthood were associated with higher risk of both mental health/substance use services and income maintenance. Individualized services for juvenile court-involved girls are suggested. Prospective, longitudinal research is needed to investigate intrapsychic and behavioral dynamics associated with females' young adult outcomes.

  14. Late-Onset Radial Nerve Palsy Associated with Conservatively Managed Humeral Fracture. A Case Report and Suggested Classification System

    OpenAIRE

    Abdelgawad, Amr Atef; Wassef, Andrew; Ebraheim, Nabil A.

    2009-01-01

    Radial nerve palsy can occur with humerus fracture, either at the time of injury (primary) or during reduction (secondary). Late-onset radial nerve palsy (not immediately related to injury or reduction) has been very seldom reported in the English literature. We describe a case of late-onset radial nerve palsy, which developed 9 weeks after an attempted closed management of a midshaft humerus fracture. Exploration of the nerve was performed. The radial nerve was found to be stretched over the...

  15. Bilateral, independent juvenile nasopharyngeal angiofibroma

    DEFF Research Database (Denmark)

    Mørkenborg, Marie-Louise; Frendø, M; Stavngaard, T

    2015-01-01

    BACKGROUND: Juvenile nasopharyngeal angiofibroma is a benign, vascular tumour that primarily occurs in adolescent males. Despite its benign nature, aggressive growth patterns can cause potential life-threatening complications. Juvenile nasopharyngeal angiofibroma is normally unilateral, originati...

  16. Cerebrospinal Fluid Cytokines Correlate With Aseptic Meningitis and Blood-Brain Barrier Function in Neonatal-Onset Multisystem Inflammatory Disease: Central Nervous System Biomarkers in Neonatal-Onset Multisystem Inflammatory Disease Correlate With Central Nervous System Inflammation.

    Science.gov (United States)

    Rodriguez-Smith, Jackeline; Lin, Yen-Chih; Tsai, Wanxia Li; Kim, Hanna; Montealegre-Sanchez, Gina; Chapelle, Dawn; Huang, Yan; Sibley, Cailin H; Gadina, Massimo; Wesley, Robert; Bielekova, Bibiana; Goldbach-Mansky, Raphaela

    2017-06-01

    To evaluate proinflammatory cytokines and leukocyte subpopulations in the cerebrospinal fluid (CSF) and blood of patients with neonatal-onset multisystem inflammatory disease (NOMID) after treatment, and to compare inflammatory cytokines in the CSF and blood in 6 patients treated with 2 interleukin-1 (IL-1) blockers-anakinra and canakinumab. During routine follow-up visits between December 2011 and October 2013, we immunophenotyped the CSF of 17 pediatric NOMID patients who were treated with anakinra, and analyzed CSF cytokine levels in samples obtained at baseline and at 3-5-year follow-up visits and compared them to samples from healthy controls. CSF levels of IL-6, interferon-γ-inducible 10-kd protein (IP-10/CXCL10), and IL-18 and monocyte and granulocyte counts significantly decreased with anakinra treatment but did not normalize to levels in the controls, even in patients fulfilling criteria for clinical remission. CSF IL-6 and IL-18 levels significantly correlated with measures of blood-brain barrier function, specifically CSF protein (r = 0.75 and r = 0.81, respectively) and albumin quotient (r = 0.79 and r = 0.68, respectively). When patients were treated with canakinumab versus anakinra, median CSF white blood cell counts and IL-6 levels were significantly higher with canakinumab treatment (10.2 cells/mm 3 versus 3.7 cells/mm 3 and 150.7 pg/ml versus 28.5 pg/ml, respectively) despite similar serum cytokine levels. CSF leukocyte subpopulations and cytokine levels significantly improve with optimized IL-1 blocking treatment, but do not normalize. The correlation of CSF IL-6, IP-10/CXCL10, and IL-18 levels with clinical laboratory measures of inflammation and blood-brain barrier function suggests that they may have a role as biomarkers in central nervous system (CNS) inflammation. The difference in inhibition of CSF biomarkers between 2 IL-1 blocking agents, anakinra and canakinumab, suggests differences in efficacy in the intrathecal

  17. Extending juvenility in grasses

    Energy Technology Data Exchange (ETDEWEB)

    Kaeppler, Shawn; de Leon Gatti, Natalia; Foerster, Jillian

    2017-04-11

    The present invention relates to compositions and methods for modulating the juvenile to adult developmental growth transition in plants, such as grasses (e.g. maize). In particular, the invention provides methods for enhancing agronomic properties in plants by modulating expression of GRMZM2G362718, GRMZM2G096016, or homologs thereof. Modulation of expression of one or more additional genes which affect juvenile to adult developmental growth transition such as Glossy15 or Cg1, in conjunction with such modulation of expression is also contemplated. Nucleic acid constructs for down-regulation of GRMZM2G362718 and/or GRMZM2G096016 are also contemplated, as are transgenic plants and products produced there from, that demonstrate altered, such as extended juvenile growth, and display associated phenotypes such as enhanced yield, improved digestibility, and increased disease resistance. Plants described herein may be used, for example, as improved forage or feed crops or in biofuel production.

  18. Miastenia gravis juvenil Juvenile myasthenia gravis

    OpenAIRE

    Oscar Papazian; Israel Alfonso; Nayle Araguez

    2009-01-01

    La miastenia gravis juvenil (MGJ) es un trastorno crónico auto inmune en el cual existen anticuerpos séricos que al unirse a los receptores de acetilcolin nicotínicos de la membrana muscular de la placa motora alteran la transmisión neuromuscular. El resultado es fatiga muscular precoz con progresión a la parálisis durante estados de contracción muscular iterativos (movimientos) o sostenidos (posturas) y más raramente parálisis permanente durante el reposo. Los músculos inervados por los nerv...

  19. Fully automatic diagnostic system for early- and late-onset mild Alzheimer's disease using FDG PET and 3D-SSP

    International Nuclear Information System (INIS)

    Ishii, Kazunari; Kono, Atsushi K.; Sasaki, Hiroki; Miyamoto, Naokazu; Fukuda, Tetsuya; Sakamoto, Setsu; Mori, Etsuro

    2006-01-01

    The purpose of this study was to design a fully automatic computer-assisted diagnostic system for early- and late-onset mild Alzheimer's disease (AD). Glucose metabolic images were obtained from mild AD patients and normal controls using positron emission tomography (PET) and 18 F-fluorodeoxyglucose (FDG). Two groups of 20 mild AD patients with different ages of onset were examined. A fully automatic diagnostic system using the statistical brain mapping method was established from the early-onset (EO) and late-onset (LO) groups, with mean ages of 59.1 and 70.9 years and mean MMSE scores of 23.3 and 22.8, respectively. Aged-matched normal subjects were used as controls. We compared the diagnostic performance of visual inspection of conventional axial FDG PET images by experts and beginners with that of our fully automatic diagnostic system in another 15 EO and 15 LO AD patients (mean age 58.4 and 71.7, mean MMSE 23.6 and 23.1, respectively) and 30 age-matched normal controls. A receiver operating characteristic (ROC) analysis was performed to compare data. The diagnostic performance of the automatic diagnostic system was comparable with that of visual inspection by experts. The area under the ROC curve for the automatic diagnostic system was 0.967 for EO AD patients and 0.878 for LO AD patients. The mean area under the ROC curve for visual inspection by experts was 0.863 and 0.881 for the EO and LO AD patients, respectively. The mean area under the ROC curve for visual inspection by beginners was 0.828 and 0.717, respectively. The fully automatic diagnostic system for EO and LO AD was able to perform at a similar diagnostic level to visual inspection of conventional axial images by experts. (orig.)

  20. Gene polymorphism and HLA-G expression in patients with childhood-onset systemic lupus erythematosus: A pilot study.

    Science.gov (United States)

    Cavalcanti, A; Almeida, R; Mesquita, Z; Duarte, A L B P; Donadi, E A; Lucena-Silva, N

    2017-10-01

    Human leukocyte antigen-G (HLA-G) presents inhibitory functions in immune cells and is located in a chromosomal region associated with systemic lupus erythematosus (SLE) susceptibility. Polymorphisms in 3' untranslated region (3'UTR) of HLA-G gene may influence protein expression. To date, no study analyzing HLA-G polymorphism and expression in childhood-onset systemic lupus erythematosus (cSLE) has been conducted. Therefore, we investigated the influence of HLA-G 3'UTR polymorphisms in 50 cSLE patients and 144 healthy controls. For the expression analysis, the control group included 26 healthy individuals. No significant difference in allele, genotype, and haplotype frequencies was observed between patients and control group. However, both the 14 bp deletion allele (odds ratio [OR] = 2.76, 95% confidence interval [CI] = 1.17-6.52, P = .028) and the 14 bp deletion-deletion genotype (OR = 8.00, 95% CI = 1.57-40.65, P = .006) showed an association with lupus nephritis. After Bonferroni correction, none P-value remained statistically significant. Regarding HLA-G expression, no significant difference was observed between plasma levels of cSLE patients (56.02 U/mL, interquartile range [IQR] = 37.54-75.41) and control group (49.2 U/mL, IQR = 27.84-154.4, P = .952). However, when the patients were stratified according to clinical manifestations, patients with hematological manifestations showed a lower plasma concentration of soluble HLA-G (sHLA-G) (47.08 U/mL, IQR = 34.15-61.56) than patients with no hematological manifestations (65.26 U/mL, IQR = 47.69-102.60, P = .013). These results suggest that HLA-G polymorphism has small effect on cSLE susceptibility and that sHLA-G may be involved in the pathogenesis of the disease. © 2017 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  1. Gender Differences in Delinquency and Juvenile Justice Processing: Evidence from National Data

    Science.gov (United States)

    Tracy, Paul E.; Kempf-Leonard, Kimberly; Abramoske-James, Stephanie

    2009-01-01

    This article traces the historical coverage of the gender issue in the criminological literature. It also provides contemporary empirical evidence about differences and similarities between girls and boys with respect to juvenile crime and to processing by the juvenile justice system, by analyzing several national juvenile crime data series, all…

  2. Immunization With a Pneumococcal Polysaccharide Vaccine in Children With Juvenile Idiopathic Arthritis Without Systemic Manifestations: a Prospective Study

    Directory of Open Access Journals (Sweden)

    Ekaterina I. Alexeeva

    2017-01-01

    Full Text Available Background. Patients with juvenile idiopathic arthritis (JIA have an increased risk of being infected. Approximately half of all serious infections in children with JIA are associated with airway involvement.Objective. Our aim was to study the efficacy and safety of the pneumococcal 13-valent conjugate vaccine (PCV in children with JIA.Methods. In a prospective cohort study, 5 groups were formed:  children with JIA in the remission phase on methotrexate therapy  (group 1 or etanercept (group 2, with JIA in the active phase prior  to the appointment of methotrexate (group 3 or etanercept (group  4, control group (conditionally healthy children. 0.5 ml of the 13-valent PCV was administered once subcutaneously during therapy in patients in the remission phase or 3 weeks before the appointment  of methotrexate or etanercept in patients in the active phase. The  main study outcome was the proportion of patients with a protective  ( 40 mg/L level of specific anti-pneumococcal antibodies (anti-SPP IgG to Streptococcus pneumoniae 4 weeks after vaccination. In  addition, we assessed the incidence of infectious events before and  after vaccination as well as changes in the content of a high-sensitivity C-reactive protein, S100 protein, and post-vaccination period.Results. The study included 125 children. Four weeks after  vaccination, the protective level of anti-SPP IgG was established in  21 (84% patients in the 1st, 23 (92% in the 2nd, 22 (88% in the  3rd, 24 (96% in the 4th and 5th groups (p =1.0. Increase in the  concentration of S100 protein and high-sensitivity C-reactive protein  after vaccination was not noted. JIA exacerbation episodes were not  recorded in any patient. After immunization, the total number of infectious events decreased in all observed groups (p 0.001. Serious adverse events were not registered during the study.Conclusion. Vaccination with the 13-valent PCV in children with JIA  is highly effective

  3. [Clinical features of patients with juvenile and adult dermatomyositis].

    Science.gov (United States)

    Szalmás, Orsolya; Nagy-Vince, Melinda; Dankó, Katalin; Farkas, Flóra

    2015-09-13

    Juvenile and adult dermatomysitis are chronic, immune-mediated inflammatory myopathies characterized by progressive proximal muscle weakness and typical skin symptoms. To compare the symptoms, laboratory and serological findings, treatment and disease course in children and adults suffering from dermatomyositis. In this retrospective study, juvenile and adult dermatomyositis groups were formed. There were 27 patients with juvenile dermatomyositis (mean age, 8.7 years; mean follow-up time: 104.6 months) and 30 adult patients (mean age, 50.3; mean follow-up time: 58.1 months). In patients with juvenile dermatomyositis, treatment with intravenous immunoglobulin and cyclosporine A were more frequent as compared to adult patients. Acute onset of the disease was more frequent in adult patients than in those with juvenile disease. In children symptoms of the disease developed gradually. The findings confirm previously published data showing that there are differences between juvenile and adult patients with dermatomyositis. The authors recommend to follow the patients regularly after reaching remission to avoid bad patient compliance and decrease the number and severity of relapses.

  4. Delinquência juvenil

    OpenAIRE

    Pais, Marta Filipa Xavier

    2012-01-01

    Trabalho apresentado à Universidade Fernando Pessoa, como parte dos requisitos para a obtenção da avaliação do grau de Licenciada em Criminologia O presente projecto intitula-se de “Delinquência juvenil: Ausência de Vínculos e Adopção de Comportamentos Desviantes”. Divide-se em duas etapas, inicialmente possui uma pesquisa bibliográfica em que se pretende dar-se a conhecer algumas das teorias explicativas da delinquência juvenil e simultaneamente compreender-se de que for...

  5. DERMATOMIOSITIS JUVENIL Y EMBARAZO

    OpenAIRE

    Evans M,Gregorio; Poulsen R,Ronald; Blanco R,Romiely; Luna V,Viviana

    2002-01-01

    La dermatomiositis juvenil es un desorden inflamatorio crónico multisistémico del tejido conectivo. Tiene una incidencia de 2-3/100.000/año. Con la disminución en la mortalidad experimentada en los últimos decenios, la atención está cifrada en la morbilidad a largo plazo y en las alteraciones funcionales. Con un tratamiento agresivo los niños con dermatomiositis juvenil generalmente tienen un futuro promisorio, sin incapacidad o con incapacidad mínima. La mortalidad actualmente se estima cerc...

  6. The impact of disease and antirheumatic therapy factors on growth retardation in children suffering from juvenile rheumatoid arthritis

    Directory of Open Access Journals (Sweden)

    T.M. Bzarova

    2006-01-01

    Full Text Available THE STUDY EVALUATED THE IMPACT OF REFRACTORY JUVENILE IDIOPATHIC ARTHRITIS (JIA AND INTERFERENCE OF ANTIRHEUMATIC THERAPY ON PHYSICAL STATURE AND GROWTH AND PHYSICAL DEVELOPMENT IN CHILDREN. WE OBSERVED 133 PATIENTS WITH JIA WITH AGE FROM 4.6 TO 18 YEARS. WE ASSESSED PATIENTS' PHYSICAL STATURE (DOCUMENTING HEIGHT AND WEIGHT AND FURTHER GROWTH AND DEVELOPMENT STARING FROM BIRTH, BEFORE THE ONSET OF JIA, AT THE FIRST YEAR FROM THE ONSET OF THE DISB EASE AND LATER ON. PHYSICAL RETARDATION WAS MARKED IN ALL CHILDREN IN THE FIRST YEAR FROM THE ONSET OF JIA. SYSTEMIC JIA, WHICH IS KNOWN FOR HIGH ACTIVITY AND POLYARTICULAR OR GENERALIZED JOINT LESION, WAS LINKED TO SIGNIFICANT PHYSICAL RETARDATION IN ALL CHILDREN WITH SYSBTEMIC JIA. LONGBTERM GLUCOCORTICOID ADMINISTRATION, EVEN AT LOW DOSES, ACCENTUATED THE NEGATIVE IMPACT OF JIA ON GROWTH RESULTING IN MARKED GROWTH DELAY OR ITS' TOTAL ARREST. WE HAVE ELABORATED RISK FACTORS FOR SHORT STATURE IN CHILDREN WITH JIA, WHICH SUGGEST THERAPY GUIDELINES, PRESENTED IN THIS ARTICLE.KEY WORDS: JUVENILE IDIOPATHIC ARTHRITIS, СHILDREN.

  7. Delincuencia y responsabilidad penal juvenil en Colombia

    Directory of Open Access Journals (Sweden)

    Cristina Montalvo Velásquez

    2011-01-01

    England in 1815, “Juvenile delinquency is defined as the set of crimes, misdemeanors or socially reprehensible conduct, committed by young people considered by the law”. Each state is subject to its own legal system, for some it is the adolescent juvenile who commits sanctioned by the law regardless of their severity, other states only consider the youth as a juvenile offender who commits a serious criminal act.The phenomenon of juvenile delinquency is something that fits in the space of a society in which its material structure, and its consequent social formation, is in deep crisis. That younger as organized criminal gangs are telling us that result in the same general crime that has gripped society in perspective to survive materially. Capitalism is not only accumulation of wealth, but concentration of the very few hands, and all the legal and institutional system tends to favor this phenomenon because it is the structure above the capitalist mode of production. Just as adults are organized to commit crimes, do children and young people from an age in which they can see that society is not healthy and have no human future on it. Abandoned and subject to the violence that begets the system, they simply respond in a demonstration of conditioned reflexes that sustain survival in an instinctive way, “children do not know about laws but about ways to survive such a situation, the survival instinct does not ages or the regulations is liable to affect the.Key WordsJuvenile Delinquency, Youth Crime, Family Factors, criminal act, criminal liability.

  8. Therapeutic Potential of Interferon-γ and Its Antagonists in Autoinflammation: Lessons from Murine Models of Systemic Juvenile Idiopathic Arthritis and Macrophage Activation Syndrome

    Directory of Open Access Journals (Sweden)

    Anneleen Avau

    2015-11-01

    Full Text Available Interferon-γ (IFN-γ affects immune responses in a complex fashion. Its immunostimulatory actions, such as macrophage activation and induction of T helper 1-type responsiveness, are widely acknowledged, however, as documented by a large body of literature, IFN-γ has also the potential to temper inflammatory processes via other pathways. In autoimmune and autoinflammatory disorders, IFN-γ can either play a disease-enforcing role or act as protective agent, depending on the nature of the disease. In animal models of any particular autoimmune disease, certain changes in the induction procedure can reverse the net outcome of introduction or ablation of IFN-γ. Here, we review the role of endogenous IFN-γ in inflammatory disorders and related murine models, with a focus on systemic juvenile idiopathic arthritis (sJIA and macrophage activation syndrome (MAS. In particular, we discuss our recent findings in a mouse model of sJIA, in which endogenous IFN-γ acts as a regulatory agent, and compare with results from mouse models of MAS. Also, we elaborate on the complexity in the activity of IFN-γ and the resulting difficulty of predicting its value or that of its antagonists as treatment option.

  9. Juvenile prison: Remarks on the specific characteristics of regular sentencing

    Directory of Open Access Journals (Sweden)

    Miladinović-Stefanović Dušica

    2015-01-01

    Full Text Available The system of the juvenile criminal law in the Republic of Serbia includes different mechanisms of social response to juvenile delinquency, including corrective orders, corrective measures and juvenile prison. This paper deals with the issue of determining a relevant sentence for juvenile offenders in trial proceedings. The legislator has provided a number of guidelines for these proceedings: the specific range of the juvenile prison sentence, the purpose of punishment, the degree of maturity of a juvenile offender, the time needed for his/her educational and vocational training, and all relevant circumstances envisaged in Article 54 of the Criminal Code. The previous statement reveals the focal points of examination and structural organization in this article, whose purpose is twofold: first, to explain the effect of the subject-specific legal provisions (aggravating and mitigating circumstances envisaged in the Act on Juvenile Criminal Offenders and Protection of Juveniles in Criminal Law and, second, to establish how the general provisions from the Criminal Code are applied in this case, with special emphasis on their specific manifestations.

  10. Setting a minimum age for juvenile justice jurisdiction in California

    Science.gov (United States)

    Barnert, Elizabeth S.; Abrams, Laura S.; Maxson, Cheryl; Gase, Lauren; Soung, Patricia; Carroll, Paul; Bath, Eraka

    2018-01-01

    Purpose Despite the existence of minimum age laws for juvenile justice jurisdiction in 18 US states, California has no explicit law that protects children (i.e. youth less than 12 years old) from being processed in the juvenile justice system. In the absence of a minimum age law, California lags behind other states and international practice and standards. The paper aims to discuss these issues. Design/methodology/approach In this policy brief, academics across the University of California campuses examine current evidence, theory, and policy related to the minimum age of juvenile justice jurisdiction. Findings Existing evidence suggests that children lack the cognitive maturity to comprehend or benefit from formal juvenile justice processing, and diverting children from the system altogether is likely to be more beneficial for the child and for public safety. Research limitations/implications Based on current evidence and theory, the authors argue that minimum age legislation that protects children from contact with the juvenile justice system and treats them as children in need of services and support, rather than as delinquents or criminals, is an important policy goal for California and for other national and international jurisdictions lacking a minimum age law. Originality/value California has no law specifying a minimum age for juvenile justice jurisdiction, meaning that young children of any age can be processed in the juvenile justice system. This policy brief provides a rationale for a minimum age law in California and other states and jurisdictions without one. Paper type Conceptual paper PMID:28299968

  11. Two-year seizure reduction in adults with medically intractable partial onset epilepsy treated with responsive neurostimulation: final results of the RNS System Pivotal trial.

    Science.gov (United States)

    Heck, Christianne N; King-Stephens, David; Massey, Andrew D; Nair, Dileep R; Jobst, Barbara C; Barkley, Gregory L; Salanova, Vicenta; Cole, Andrew J; Smith, Michael C; Gwinn, Ryder P; Skidmore, Christopher; Van Ness, Paul C; Bergey, Gregory K; Park, Yong D; Miller, Ian; Geller, Eric; Rutecki, Paul A; Zimmerman, Richard; Spencer, David C; Goldman, Alica; Edwards, Jonathan C; Leiphart, James W; Wharen, Robert E; Fessler, James; Fountain, Nathan B; Worrell, Gregory A; Gross, Robert E; Eisenschenk, Stephan; Duckrow, Robert B; Hirsch, Lawrence J; Bazil, Carl; O'Donovan, Cormac A; Sun, Felice T; Courtney, Tracy A; Seale, Cairn G; Morrell, Martha J

    2014-03-01

    To demonstrate the safety and effectiveness of responsive stimulation at the seizure focus as an adjunctive therapy to reduce the frequency of seizures in adults with medically intractable partial onset seizures arising from one or two seizure foci. Randomized multicenter double-blinded controlled trial of responsive focal cortical stimulation (RNS System). Subjects with medically intractable partial onset seizures from one or two foci were implanted, and 1 month postimplant were randomized 1:1 to active or sham stimulation. After the fifth postimplant month, all subjects received responsive stimulation in an open label period (OLP) to complete 2 years of postimplant follow-up. All 191 subjects were randomized. The percent change in seizures at the end of the blinded period was -37.9% in the active and -17.3% in the sham stimulation group (p = 0.012, Generalized Estimating Equations). The median percent reduction in seizures in the OLP was 44% at 1 year and 53% at 2 years, which represents a progressive and significant improvement with time (p < 0.0001). The serious adverse event rate was not different between subjects receiving active and sham stimulation. Adverse events were consistent with the known risks of an implanted medical device, seizures, and of other epilepsy treatments. There were no adverse effects on neuropsychological function or mood. Responsive stimulation to the seizure focus reduced the frequency of partial-onset seizures acutely, showed improving seizure reduction over time, was well tolerated, and was acceptably safe. The RNS System provides an additional treatment option for patients with medically intractable partial-onset seizures. © 2014 The Authors. Epilepsia published by Wiley Periodicals, Inc. on behalf of International League Against Epilepsy.

  12. The Pediatric Rheumatology International Trials Organization/American College of Rheumatology provisional criteria for the evaluation of response to therapy in juvenile systemic lupus erythematosus: prospective validation of the definition of improvement

    DEFF Research Database (Denmark)

    Ruperto, N; Ravelli, A; Oliviera, S

    2006-01-01

    on the product of the content validity score multiplied by its kappa statistic. RESULTS: The definition of improvement with the highest final score was at least 50% improvement from baseline in any 2 of the 5 core set measures, with no more than 1 of the remaining worsening by more than 30%. CONCLUSION: PRINTO......OBJECTIVE: To use the Pediatric Rheumatology International Trials Organization (PRINTO) core set of outcome measures to develop a validated definition of improvement for the evaluation of response to therapy in juvenile systemic lupus erythematosus (SLE). METHODS: Thirty-seven experienced pediatric...... proposes a valid and reproducible definition of improvement that reflects well the consensus rating of experienced clinicians and that incorporates clinically meaningful change in core set measures in a composite end point for the evaluation of global response to therapy in patients with juvenile SLE...

  13. Generation of a novel mouse model that recapitulates early and adult onset glycogenosis type IV

    Science.gov (United States)

    Akman, H. Orhan; Sheiko, Tatiana; Tay, Stacey K.H.; Finegold, Milton J.; DiMauro, Salvatore; Craigen, William J.

    2011-01-01

    Glycogen storage disease type IV (GSD IV) is a rare autosomal recessive disorder caused by deficiency of the glycogen branching enzyme (GBE). The diagnostic feature of the disease is the accumulation of a poorly branched form of glycogen known as polyglucosan (PG). The disease is clinically heterogeneous, with variable tissue involvement and age of disease onset. Absence of enzyme activity is lethal in utero or in infancy affecting primarily muscle and liver. However, residual enzyme activity (5–20%) leads to juvenile or adult onset of a disorder that primarily affects muscle as well as central and peripheral nervous system. Here, we describe two mouse models of GSD IV that reflect this spectrum of disease. Homologous recombination was used to insert flippase recognition target recombination sites around exon 7 of the Gbe1 gene and a phosphoglycerate kinase-Neomycin cassette within intron 7, leading to a reduced synthesis of GBE. Mice bearing this mutation (Gbe1neo/neo) exhibit a phenotype similar to juvenile onset GSD IV, with wide spread accumulation of PG. Meanwhile, FLPe-mediated homozygous deletion of exon 7 completely eliminated GBE activity (Gbe1−/−), leading to a phenotype of lethal early onset GSD IV, with significant in utero accumulation of PG. Adult mice with residual GBE exhibit progressive neuromuscular dysfunction and die prematurely. Differently from muscle, PG in liver is a degradable source of glucose and readily depleted by fasting, emphasizing that there are structural and regulatory differences in glycogen metabolism among tissues. Both mouse models recapitulate typical histological and physiological features of two human variants of branching enzyme deficiency. PMID:21856731

  14. Subpopulations Within Juvenile Psoriatic Arthritis: A Review of the Literature

    Directory of Open Access Journals (Sweden)

    Matthew L. Stoll

    2006-01-01

    Full Text Available The presentation of juvenile psoriatic arthritis (JPsA has long been recognized to be clinically heterogeneous. As the definition of JPsA expanded to accommodate atypical manifestations of psoriasis in young children, studies began to reflect an increasingly clear biphasic distribution of age of onset, with peaks in the first few years of life and again in early adolescence. These two subpopulations differ in gender ratio, pattern of joint involvement, laboratory findings and potentially response to therapy. Intriguingly, a similar distribution of age of onset has been observed in juvenile rheumatoid arthritis (JRA, and correlates with patterns of HLA association. While a secure classification of subpopulations within JPsA awaits improved pathophysiologic understanding, future research must consider the possibility that different disease mechanisms may be operative in distinct subsets of patients with this disorder.

  15. Influenza vaccination can induce new onset anticardiolipins but not β2-glycoprotein-I antibodies among patients with systemic lupus erythematosus

    Science.gov (United States)

    Vista, Evan S.; Crowe, Sherry R.; Thompson, Linda F.; Air, Gillian M.; Robertson, Julie M.; Guthridge, Joel M.; James, Judith A.

    2012-01-01

    Summary Background Antiphospholipid syndrome is characterized by autoantibodies against cardiolipins (aCL), lupus anticoagulant, and independent β2-glycoprotein (β2GPI). Controversy exists as to whether vaccination triggers the development of anti-phospholipid antibodies (aPL) in systemic lupus erythematosus (SLE) patients. Methods SLE patients (101) and matched controls (101) were enrolled from 2005 to 2009 and received seasonal influenza vaccinations. Sera were tested by ELISA for aCL at baseline, 2, 6, and 12 weeks after vaccination. Vaccine responses were ranked according to an overall anti-influenza antibody response index. Individuals with positive aCL were further tested for β2GPI antibodies. Results SLE patients and healthy controls developed new onset aCL post-vaccination (12/101 cases and 7/101 controls, OR 1.81, p=0.34). New onset moderate aCL are slightly enriched in African American SLE patients (5/36 cases; p=0.094). The optical density (OD) measurements for aCL reactivity in patients were significantly higher than baseline at 2 weeks (pvaccination. No new β2GPI antibodies were detected among patients with new aCL reactivity. Vaccine response was not different between patients with and without new onset aCL reactivity (p=0.43). Conclusions This study shows transient increases in aCL, but not anti-β2GPI responses, after influenza vaccination. PMID:22235049

  16. Influenza vaccination can induce new-onset anticardiolipins but not β2-glycoprotein-I antibodies among patients with systemic lupus erythematosus.

    Science.gov (United States)

    Vista, E S; Crowe, S R; Thompson, L F; Air, G M; Robertson, J M; Guthridge, J M; James, J A

    2012-02-01

    Antiphospholipid syndrome is characterized by autoantibodies against cardiolipins (aCL), lupus anticoagulant, and independent β2-glycoprotein (β2GPI). Controversy exists as to whether vaccination triggers the development of antiphospholipid antibodies (aPL) in patients with systemic lupus erythematosus (SLE). Patients with SLE (101) and matched controls (101) were enrolled from 2005-2009 and received seasonal influenza vaccinations. Sera were tested by ELISA for aCL at baseline, 2, 6, and 12 weeks after vaccination. Vaccine responses were ranked according to an overall anti-influenza antibody response index. Individuals with positive aCL were further tested for β2GPI antibodies. Patients with SLE and healthy controls can develop new-onset aCL post vaccination, although at rates which do not differ between patients and controls (12/101 cases and 7/101 controls, OR 1.81, p = 0.34). New-onset moderate aCL are slightly enriched in African American SLE patients (5/36 cases; p = 0.094). The optical density measurements for aCL reactivity in patients were significantly higher than baseline at 2 weeks (p vaccination. No new β2GPI antibodies were detected among patients with new aCL reactivity. Vaccine response was not different between patients with and without new-onset aCL reactivity (p = 0.43). This study shows transient increases in aCL, but not anti-β2GPI responses, after influenza vaccination.

  17. Syn-volcanic cannibalisation of juvenile felsic crust: Superimposed giant 18O-depleted rhyolite systems in the hot and thinned crust of Mesoproterozoic central Australia

    Science.gov (United States)

    Smithies, R. H.; Kirkland, C. L.; Cliff, J. B.; Howard, H. M.; Quentin de Gromard, R.

    2015-08-01

    Eruptions of voluminous 18O-depleted rhyolite provide the best evidence that the extreme conditions required to produce and accumulate huge volumes of felsic magma can occur in the upper 10 km of the crust. Mesoproterozoic bimodal volcanic sequences from the Talbot Sub-basin in central Australia contain possibly the world's most voluminous accumulation of 18O-depleted rhyolite. This volcanic system differs from the better known, but geochemically similar, Miocene Snake River Plain - Yellowstone Plateau of North America. Both systems witnessed 'super' sized eruptions from shallow crustal chambers, and produced 18O-depleted rhyolite. The Talbot system, however, accumulated over a much longer period (>30 Ma), at a single depositional centre, and from a magma with mantle-like isotopic compositions that contrast strongly with the isotopically evolved basement and country-rock compositions. Nevertheless, although the Talbot rhyolites are exclusively 18O-depleted, the unavoidable inference of an 18O-undepleted precursor requires high-temperature rejuvenation of crust in an upper-crustal chamber, and in this respect the evolution of the Talbot rhyolites and 18O-depleted rhyolites of the Snake River Plain - Yellowstone Plateau is very similar. However, instead of older crustal material, the primary upper-crustal source recycled into Talbot rhyolites was comagmatic (or nearly so) felsic rock itself derived from a contemporaneous juvenile basement hot-zone. Whereas giant low δ18O volcanic systems show that voluminous melting of upper crust can occur, our studies indicate that felsic magmas generated at lower crustal depths can also contribute significantly to the thermal and material budget of these systems. The requirement that very high-temperatures be achieved and sustained in the upper crust means that voluminous low δ18O magmatism is rare, primarily restricted to bimodal tholeiitic, high-K rhyolite (A-type) magmatic associations in highly attenuated lithosphere. In the

  18. Syndromes that simulate a juvenile systemic arthritis; on purpose of a case of NOMID/CINCA syndrome

    International Nuclear Information System (INIS)

    Gamarra Iglesias, Antonio; Rojas, Adriana; Calvo P, Enrique; Restrepo S, Jose Felix

    2004-01-01

    We report a years old female with NOMID/CINCA syndrome, and we reviewed of this rare pathology. We discuss the differential diagnosis of this disease with all of the syndromes in the childhood that resemble a systemic arthritis, specially when this appears before the two years of age

  19. Early onset cannabis use and psychosocial adjustment in young adults.

    Science.gov (United States)

    Fergusson, D M; Horwood, L J

    1997-03-01

    The relationships between early onset (prior to 16 years) cannabis use and later psychosocial adjustment was examined in a birth cohort of New Zealand children studied to age 18 years. Early onset users had significantly higher rates of later substance use, juvenile offending, mental health problems, unemployment and school dropout. The linkages between early onset cannabis use and later outcomes were largely explained by two routes that linked cannabis use to later adjustment. First, those electing to use cannabis were a high risk population characterized by social disadvantage, childhood adversity, early onset behavioural difficulties and adverse peer affiliations. Secondly, early onset cannabis use was associated with subsequent affiliations with delinquent and substance using peers, moving away from home and dropping out of education with these factors in turn, being associated with increased psychosocial risk. The implications of these results are examined.

  20. Depressive symptom trajectories among girls in the juvenile justice system: 24-month outcomes of an RCT of Multidimensional Treatment Foster Care.

    Science.gov (United States)

    Harold, Gordon T; Kerr, David C R; Van Ryzin, Mark; DeGarmo, David S; Rhoades, Kimberly A; Leve, Leslie D

    2013-10-01

    Youth depression is a significant and growing international public health problem. Youth who engage in high levels of delinquency are at particularly high risk for developing problems with depression. The present study examined the impact of a behavioral intervention designed to reduce delinquency (Multidimensional Treatment Foster Care; MTFC) compared to a group care intervention (GC; i.e., services as usual) on trajectories of depressive symptoms among adolescent girls in the juvenile justice system. MTFC has documented effects on preventing girls' recidivism, but its effects on preventing the normative rise in girls' depressive symptoms across adolescence have not been examined. This indicated prevention sample included 166 girls (13-17 years at T1) who had at least one criminal referral in the past 12 months and who were mandated to out-of-home care; girls were randomized to MTFC or GC. Intent-to-treat analyses examined the main effects of MTFC on depression symptoms and clinical cut-offs, and whether benefits were greatest for girls most at risk. Depressive symptom trajectories were specified in hierarchical linear growth models over a 2 year period using five waves of data at 6 month intervals. Depression clinical cut-off scores were specified as nonlinear probability growth models. Results showed significantly greater rates of deceleration for girls in MTFC versus GC for depressive symptoms and for clinical cut-off scores. The MTFC intervention also showed greater benefits for girls with higher levels of initial depressive symptoms. Possible mechanisms of effect are discussed, given MTFC's effectiveness on targeted and nontargeted outcomes.

  1. The effect of nutritional intervention on the lipid profile and dietary intake of adolescents with juvenile systemic lupus erythematosus: a randomized, controlled trial.

    Science.gov (United States)

    da Silva, S G L; Terreri, M T; Abad, T T O; Machado, D; Fonseca, F L A; Hix, S; Suano-Souza, F I; Sarni, R O S; Len, C A

    2018-04-01

    Objective This study sought to evaluate the effects of a nutritional intervention on the lipid metabolism biomarkers associated with cardiovascular risk, and their variation over time, in juvenile systemic lupus erythematosus (JSLE) patients. This study also investigated the relationships between these biomarkers and dietary intake, nutritional status, disease variables, and medication used. Methods A total of 31 10- to 19-year-old female adolescents with JSLE for at least six months were analyzed. The participants were randomly allocated to two groups: nutritional intervention or control. The intervention group received verbal and printed nutritional instructions once per month over nine months. Before and after the intervention, the participants underwent assessments of anthropometry; dietary intake; physical activity; socioeconomic status; total cholesterol and fractions; triglycerides; apolipoprotein A (Apo A-I); apolipoprotein B (Apo B); paraoxonase (PON) activity (a) and amount (q); myeloperoxidase (MPO); and small, dense LDL-c (sdLDL) particles. Results After nine months, we found significant reductions in the calorie, carbohydrate, total fat, saturated fat, and trans fat intakes in the intervention compared with the control group over time. The PONa/HDL-c ratio increased by 3.18 U/ml/mg/dl in the intervention group and by 0.63 U/ml/mg/dl in the control group ( p = 0.037). Unlike the intervention group, the sdLDL levels of the control group worsened over time ( p = 0.018). Conclusion The present study detected a reduction in calorie and fat intake, which indicates an improvement of HDL-c function and possible protection against cardiovascular risk for the intervention group.

  2. Multidimensional Treatment Foster Care for Girls in the Juvenile Justice System: 2-Year Follow-Up of a Randomized Clinical Trial

    Science.gov (United States)

    Chamberlain, Patricia; Leve, Leslie D.; DeGarmo, David S.

    2007-01-01

    This study is a 2-year follow-up of girls with serious and chronic delinquency who were enrolled in a randomized clinical trial conducted from 1997 to 2002 comparing multidimensional treatment foster care (MTFC) and group care (N = 81). Girls were referred by juvenile court judges and had an average of over 11 criminal referrals when they entered…

  3. Predictive validity of the Washington State Juvenile Court Pre-Screen Assessment in the Netherlands: the development of a new scoring system

    NARCIS (Netherlands)

    van der Put, C.E.; Stams, G.J.J.M.; Deković, M.; van der Laan, P.H.

    2014-01-01

    This study examined the predictive validity of the Washington State Juvenile Pre-Screen Assessment (WSJCA pre-screen) in the Netherlands. Previous research conducted in the United States showed the predictive validity of the WSJCA pre-screen to be modest, as is the case with the predictive validity

  4. Predictive Validity of the Washington State Juvenile Court Pre-Screen Assessment in the Netherlands : The Development of a New Scoring System

    NARCIS (Netherlands)

    van der Put, Claudia E.; Stams, Geert Jan J. M.; Dekovic, Maja; van der Laan, Peter H.

    This study examined the predictive validity of the Washington State Juvenile Pre-Screen Assessment (WSJCA pre-screen) in the Netherlands. Previous research conducted in the United States showed the predictive validity of the WSJCA pre-screen to be modest, as is the case with the predictive validity

  5. [Role of oxidative stress in the maintenance of inflamation in patients with juvenile rheumatoid arthritis

    Science.gov (United States)

    Ramos, V A; Ramos, P A; Dominguez, M C

    2000-01-01

    OBJECTIVE: To determine the level of cellular oxidative stress blood markers and the enzymatic system of antioxidant defense establishing the oxidative profile in patients with Juvenile Rheumatoid Arthritis. METHODS: Case-control study that included 64 patients (46 of female sex) with Juvenile Rheumatoid Arthritis (JRA) following clinical control in the Pediatric Rheumatology Service of the Vall dacute;Hebron Hospital, Barcelona, Spain. The patients were separated in three subtypes based on the pattern of onset within the first six months of disease: polyarticular, pauciarticular and systemic. The control group included 60 patients (38 of female sex) following clinical control to diseases of non inflammatory nature, in the same hospital. The plasmatic levels of malondialdehyde (MDA), lipoperoxide (LPO), hydroperoxide (HPX), carbonile groups (CG) of proteins and gluthathione and the enzymatic activities of Superoxide dismutase (SOD), gluthathione peroxidase (GSH-Px) and gluthathione reductase were determined. RESULTS: The group of patients with JRA presented high concentrations of lipid peroxidation products, evaluated by determining the plasmatic levels of MDA, LPO, and HPX; oxidative damage of the circulate protein, determined by CG contents of plasma proteins; elevation of enzymatic activity of SOD and GSH-Red; decrease of GSH-Px activity and GSH levels. CONCLUSIONS: Our results show the presence of molecular damage determined by oxygen free radicals in the JRA patients. The SOD activity and the changes of gluthathione redox enzymatic cycle confirm the decrease of capacity of cellular defense system against the induced toxicity of oxidative stress in these patients.

  6. Relationship between health-related quality of life, disease activity and disease damage in a prospective international multicenter cohort of childhood onset systemic lupus erythematosus patients

    DEFF Research Database (Denmark)

    Moorthy, L N; Baldino, M E; Kurra, V

    2017-01-01

    Previously, we described associations between health-related quality of life (HRQOL) and disease-related factors among childhood onset systemic lupus erythematosus (cSLE) patients. Here we determined the relationship between HRQOL, disease activity and damage in a large prospective international...... cohort of cSLE. We compared HRQOL, disease activity and disease damage across different continents and examined the relationship between children's and parents' assessments of HRQOL. Patients with cSLE and their parents completed HRQOL measures at enrollment and ≥4 follow-up visits. Physicians assessed...

  7. Genetics Home Reference: juvenile myoclonic epilepsy

    Science.gov (United States)

    ... Home Health Conditions Juvenile myoclonic epilepsy Juvenile myoclonic epilepsy Printable PDF Open All Close All Enable Javascript ... view the expand/collapse boxes. Description Juvenile myoclonic epilepsy is a condition characterized by recurrent seizures (epilepsy). ...

  8. Genetics Home Reference: juvenile idiopathic arthritis

    Science.gov (United States)

    ... Home Health Conditions Juvenile idiopathic arthritis Juvenile idiopathic arthritis Printable PDF Open All Close All Enable Javascript ... view the expand/collapse boxes. Description Juvenile idiopathic arthritis refers to a group of conditions involving joint ...

  9. Time-to-Onset Analysis of Drug-Induced Long QT Syndrome Based on a Spontaneous Reporting System for Adverse Drug Events.

    Directory of Open Access Journals (Sweden)

    Sayaka Sasaoka

    Full Text Available Long QT syndrome (LQTS is a disorder of the heart's electrical activity that infrequently causes severe ventricular arrhythmias such as a type of ventricular tachycardia called torsade de pointes (TdP and ventricular fibrillation, which can be fatal. There have been no previous reports on the time-to-onset for LQTS based on data from spontaneous reporting systems. The aim of this study was to assess the time-to-onset of LQTS according to drug treatment. We analyzed the association between 113 drugs in 37 therapeutic categories and LQTS including TdP using data obtained from the Japanese Adverse Drug Event Report database. For signal detection, we used the reporting odds ratio (ROR. Furthermore, we analyzed the time-to-onset data and assessed the hazard type using the Weibull shape parameter. The RORs (95% confidence interval for bepridil, amiodarone, pilsicainide, nilotinib, disopyramide, arsenic trioxide, clarithromycin, cibenzoline, donepezil, famotidine, sulpiride, and nifekalant were 174.4 (148.6-204.6, 17.3 (14.7-20.4, 52.0 (43.4-62.4, 13.9 (11.5-16.7, 69.3 (55.3-86.8, 54.2 (43.2-68.0, 4.7 (3.8-5.8, 19.9 (15.9-25.0, 8.1 (6.5-10.1, 3.2 (2.5-4.1, 7.1 (5.5-9.2, and 254.8 (168.5-385.4, respectively. The medians and quartiles of time-to-onset for aprindine (oral and bepridil were 20.0 (11.0-35.8 and 18.0 (6.0-43.0 days, respectively. The lower 95% confidence interval of the shape parameter β of bepridil was over 1 and the hazard was considered to increase over time.Our study indicated that the pattern of LQTS onset might differ among drugs. Based on these results, careful long-term observation is recommended, especially for specific drugs such as bepridil and aprindine. This information may be useful for the prevention of sudden death following LQTS and for efficient therapeutic planning.

  10. Effects of natal departure and water level on survival of juvenile snail kites (Rostrhamus sociabilis) in Florida

    Science.gov (United States)

    Dreitz, V.J.; Kitchens, W.M.; DeAngelis, D.L.

    2004-01-01

    Survival rate from fledging to breeding, or juvenile survival, is an important source of variation in lifetime reproductive success in birds. Therefore, determining the relationship between juvenile survival and environmental factors is essential to understanding fitness consequences of reproduction in many populations. With increases in density of individuals and depletion of food resources, quality of most habitats deteriorates during the breeding season. Individuals respond by dispersing in search of food resources. Therefore, to understand the influence of environmental factors on juvenile survival, it is also necessary to know how natal dispersal influences survival of juveniles. We examined effects of various environmental factors and natal dispersal behavior on juvenile survival of endangered Snail Kites (Rostrhamus sociabilis) in central and southern Florida, using a generalized estimating equations (GEEs) approach and model selection criteria. Our results suggested yearly effects and an influence of age and monthly minimum hydrologic levels on juvenile Snail Kite survival. Yearly variation in juvenile survival has been reported by other studies, and other reproductive components of Snail Kites also exhibit such variation. Age differences in juvenile survival have also been seen in other species during the juvenile period. Our results demonstrate a positive relationship between water levels and juvenile survival. We suggest that this is not a direct linear relationship, such that higher water means higher juvenile survival. The juvenile period is concurrent with onset of the wet season in the ecosystem we studied, and rainfall increases as juveniles age. For management purposes, we believe that inferences suggesting increasing water levels during the fledging period will increase juvenile survival may have short-term benefits but lead to long-term declines in prey abundance and possibly wetland vegetation structure.

  11. Miastenia gravis juvenil Juvenile myasthenia gravis

    Directory of Open Access Journals (Sweden)

    Oscar Papazian

    2009-01-01

    Full Text Available La miastenia gravis juvenil (MGJ es un trastorno crónico auto inmune en el cual existen anticuerpos séricos que al unirse a los receptores de acetilcolin nicotínicos de la membrana muscular de la placa motora alteran la transmisión neuromuscular. El resultado es fatiga muscular precoz con progresión a la parálisis durante estados de contracción muscular iterativos (movimientos o sostenidos (posturas y más raramente parálisis permanente durante el reposo. Los músculos inervados por los nervios craneales, especialmente los extraoculares y elevadores de los párpados, tienen más tendencia a la debilidad muscular persistente que los inervados por otros pares craneales y las extremidades. Las formas clínicas de presentación son generalizadas, oculares y respiratorias. El diagnóstico se sospecha mediante la anamnesia, la fatiga anormal se comprueba mediante el examen físico y la estimulación eléctrica iterativa del nervio que inerva al músculo afectado pero no paralizado. Se corrobora mediante la administración de inhibidores de la acetilcolin esterasa (IACE que al aumentar la cantidad de acetilcolin en la hendidura sináptica, corrigen la fatiga o la debilidad muscular transitoriamente. Se hace el diagnóstico de certeza mediante la demostración sérica de anticuerpos contra los receptores de acetilcolin (ACRA. El tratamiento es a largo plazo sintomático con IACE y etiopatogénico con inmunosupresores, plasmaféresis, gamma globulina endovenosa y timectomía. El curso es crónico. La remisión espontánea o después de tratamiento sintomático o etiopatogénico ocurre entre 1-10 años respectivamente. La mortalidad es prácticamente nula aun durantes las crisis miastenias gracias a la educación de padres, pacientes y público en general sobre el tema, al desarrollo del sistema de respuesta rápida de auxilio domiciliario y las unidades de cuidados intensivos y el empleo de la ventilación asistida profiláctica, plasmaféresis y

  12. New solutions in the juvenile criminal law in the light of the restorative justice

    Directory of Open Access Journals (Sweden)

    Jovašević Dragan

    2007-01-01

    Full Text Available New criminal legislation got into force in Serbia at the beginning of 2006. In that way, Serbia got unique Criminal Code which includes all provisions of material criminal law except provisions related to the criminal position of juveniles. System of criminal sanctions for juvenile off enders, procedure for their imposition and the way, procedure and terms for their execution are regulated by the provisions of the separate law - the Law on juvenile off enders and criminal protection of juveniles. Some of the most important novelties introduced by new juvenile criminal law are system of diversion, i.e. system of diversion orders, which aim at excluding the imposition of criminal sanctions in the cases when criminal sanction is not necessary from the perspective of crime suppression. Bearing that in mind, this paper is dedicated to forms of diversion orders as a form of measures that lead to more efficient system of restorative justice within our new juvenile criminal legislation. .

  13. Boundaries between familial Mediterranean fever and juvenile spondyloarthritis: Analysis of three French retrospective cohorts.

    Science.gov (United States)

    Cherqaoui, Bilade; Rossi-Semerano, Linda; Georgin-Lavialle, Sophie; Dusser, Perrine; Galeotti, Caroline; Piram, Maryam; Hentgenb, Véronique; Touitou, Isabelle; Koné-Paut, Isabelle

    2018-02-13

    Children with Familial Mediterranean fever may suffer from musculoskeletal involvement, somewhat difficult to distinguish from juvenile spondyloarthritis. The association of these two diseases has been scarcely reported in children. Objective of this work was to define the association of familial Mediterranean fever and juvenile spondyloarthritis in France. Three cohorts of children with familial Mediterranean fever, juvenile spondyloarthritis, familial Mediterranean fever related juvenile spondyloarthritis, were retrospectively identified in the French reference center of auto-inflammatory diseases. Familial Mediterranean fever was defined according to Tel-Hashomer or Turkish pediatric criteria with at least one exon-10 MEFV-gene mutation. Juvenile spondyloarthritis was defined according to ILAR criteria. Patients with familial Mediterranean fever or juvenile spondyloarthritis were respectively compared to familial Mediterranean fever related juvenile spondyloarthritis patients. Sixteen children were identified as having familial Mediterranean fever related juvenile spondyloarthritis. The male/female-ratio was 0.6, with median age at spondyloarthritis onset of 7.5years (3-16years). All carried at least one M694V variant in MEFV gene; 16.7% were HLA-B27-carriers. Compared to 83 familial Mediterranean fever patients, familial Mediterranean fever related juvenile spondyloarthritis patients had less frequently fever (PMediterranean fever related juvenile spondyloarthritis patients less often received non-steroidal anti-inflammatory drugs (PMediterranean fever may be associated with typical pattern of juvenile spondyloarthritis. These patients, with less response to colchicine, should be diagnosed earlier and treated as for jSpA. Copyright © 2018 Société française de rhumatologie. Published by Elsevier SAS. All rights reserved.

  14. Juvenile Dermatomyositis in Pregnancy

    Directory of Open Access Journals (Sweden)

    Anthony Emeka Madu

    2013-01-01

    Full Text Available Juvenile dermatomyositis has variable clinical presentations both in and outside of pregnancy. A literature review indicated that optimal maternal and fetal outcomes can be anticipated when the pregnancy is undertaken while the disease is in remission. Poorer outcomes are associated with flare-up of the disease in early pregnancy compared with exacerbation in the second or third trimester, when fetal prognosis is usually good. We present a case of JDM in pregnancy with disease exacerbation late in pregnancy and review of the relevant literature.

  15. The incidence, diagnostic clinical manifestations and severity of juvenile systemic lupus erythematosus in New Zealand Maori and Pacific Island children: the Starship experience (2000-2010).

    Science.gov (United States)

    Concannon, A; Rudge, S; Yan, J; Reed, P

    2013-10-01

    To describe the incidence, diagnostic clinical manifestations and severity of juvenile systemic lupus erythematosus (jSLE) in a cohort of New Zealand Maori and Pacific Island children compared to European children. A chart review was conducted of children with jSLE seen by the Starship paediatric rheumatology and/or renal services between January 2000 and November 2010. Diagnostic clinical data and lupus nephritis data at anytime were collated while classic British Isles Lupus Assessment Group (BILAG) and Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) scores were derived retrospectively. Thirty-two children were diagnosed with jSLE with an annual incidence of 0.52 per 100,000 per year. Compared with European children (0.31 per 100,000 per year) the incidence of jSLE was higher among Maori and Pacific (0.67 per 100,000 per year, p=0.06) and significantly higher among Asian children (1.17 per 100,000 per year, p=0.01). Compared with European children, Maori and Pacific children were more frequently diagnosed with lupus nephritis (80% vs 40%, p=0.09) and severe (WHO class 4 or 5) renal lesions (60% vs 40%, p=0.43) at presentation. Similarly, at any time during the study, lupus nephritis (100% vs 40%, p=0.001) and severe (WHO class 4 or 5) renal lesions (73.3% vs 40%, p=0.12) were more frequent among Maori and Pacific compared with European children. Furthermore, retrospective BILAG assessment of diagnostic disease severity demonstrated that Maori and Pacific children experienced the majority of severe "Category A" disease (56.8% vs 22.7%, p=0.17) which was predominantly renal (73.3% vs 40%, p=0.12) in nature. This is the first description of the incidence and clinical manifestations of jSLE in a cohort of New Zealand children. Although limited by the small numbers involved it confirmed anecdotal suspicions that the incidence of jSLE among Maori, Pacific and Asian children is higher than European children. Lupus nephritis is also more frequent and severe

  16. Interactions of juvenile Lumbricus terrestris with adults and their burrow systems in a two-dimensional microcosm Interações de juvenis de Lumbricus terrestris com adultos e seus sistemas de galerias em um microcosmo bidimensional

    Directory of Open Access Journals (Sweden)

    Niki Grigoropoulou

    2009-08-01

    Full Text Available The objective of this work was to evaluate interactions of Lumbricus terrestris juveniles with adults and with inherited burrow systems. An experiment was set up using a two dimensional Evans' boxes microcosm. Adult L. terrestris were added to 16 boxes (one individual per box and kept in darkness at 17ºC along with eight unoccupied boxes for two months. The adult L. terrestris were removed from eight randomly selected boxes, and L. terrestris juveniles were added (one juvenile per box, composing three treatments with eight replicates: 1, with an adult in an inherited burrow (ABJ; 2, alone in an inherited burrow (BJ; and 3, alone in a previously uninhabited box (J. The proportion of juveniles occupying adult burrows observed was significantly different in treatments ABJ (48% and BJ (75%. The mean mass of juveniles at experimental termination differed significantly among treatments and was greater in treatment J (4.04±0.39 g in comparison to the BJ (3.09±0.93 g and ABJ treatments (2.13±0.64 g. Results suggest a negative influence of both the presence of an adult and its burrow system on juvenile growth. Intraspecific competition partially explained this, but further investigation is required to examine how an inherited environment (i.e. burrow could negatively affect the growth of juveniles.O objetivo deste trabalho foi avaliar as interações de juvenis de Lumbricus terrestris com indivíduos adultos e com sistemas de galerias herdados. O experimento foi realizado usando microcosmos bidimensionais de Evans como unidades experimentais. Adultos de L. terrestris foram colocados em 16 unidades experimentais (um indivíduo por unidade e mantidos no escuro a 17ºC juntamente com oito unidades experimentais inabitadas, por dois meses. Os adultos foram removidos de oito unidades selecionadas aleatoriamente e juvenis foram adicionados a todas as unidades experimentais (um indivíduo por unidade, em três tratamentos, com oito repetições: 1, com um

  17. [From gene to disease; mutations in the WFS1-gene as the cause of juvenile type I diabetes mellitus with optic atrophy (Wolfram syndrome)

    NARCIS (Netherlands)

    Pennings, R.J.E.; Dikkeschei, L.D.; Cremers, C.W.R.J.; Ouweland, J.M.W. van den

    2002-01-01

    Wolfram syndrome patients are mainly characterised by juvenile onset diabetes mellitus and optic atrophy. A synonym is the acronym DIDMOAD: diabetes insipidus, diabetes mellitus, optic atrophy, deafness. Diabetes insipidus and sensorineural high-frequency hearing impairment are important additional

  18. Predictors of disability in a childhood-onset systemic lupus erythematosus cohort: results from the CARRA Legacy Registry.

    Science.gov (United States)

    Hersh, A O; Case, S M; Son, M B

    2018-03-01

    Objective Few descriptions of physical disability in childhood-onset SLE (cSLE) exist. We sought to describe disability in a large North American cohort of patients with cSLE and identify predictors of disability. Methods Sociodemographic and clinical data were obtained from the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Legacy Registry for patients with cSLE enrolled between May 2010 and October 2014. The Childhood Health Assessment Questionnaire (CHAQ) was used to assess disability and physical functioning. Chi-square tests were used for univariate analyses, and multivariate logistic regression was used to assess predictors of disability. Results We analyzed data for 939 patients with cSLE. The median and mean CHAQ scores were 0 and 0.25, respectively, and 41% of the cohort had at least mild disability. Arthritis and higher pain scores were significantly associated with disability as compared to those without disability ( p disability at baseline. Conclusions Disability as measured by baseline CHAQ was fairly common in cSLE patients in the CARRA Legacy Registry, and was associated with low household income, arthritis, and higher pain scores. In addition to optimal disease control, ensuring psychosocial supports and addressing pain may reduce disability in cSLE. Further study is needed of disability in cSLE.

  19. Juvenile Justice in Australia 2009-10. Juvenile Justice Series. Number 8

    Science.gov (United States)

    Aalders, Rachel; Morgan, Kirsten

    2011-01-01

    In Australia, the state and territory governments are responsible for dealing with young people who are involved in crime. One major aspect of the juvenile justice system is the supervision of children and young people who have committed or are alleged to have committed an offence. This report presents information on the young people under…

  20. Juvenile Crime. Opposing Viewpoints Series.

    Science.gov (United States)

    Sadler, A. E., Ed.

    Books in the Opposing Viewpoints Series present debates about current issues that can be used to teach critical reading and thinking skills. The variety of opinions expressed in this collection of articles and book excerpts explores many aspects of juvenile crime. It is a commonly held view that the number of crimes committed by juveniles is…

  1. Juvenile Justice Paradigms and Improvement of Realization of the Constitutional Rights and Freedoms of Man and Citizen in Russiay

    Directory of Open Access Journals (Sweden)

    Arpentieva M. R.

    2015-07-01

    Full Text Available The article is devoted to the problems of implementation and procedures of reconstructing model of juvenile justice system, it considers foreign and domestic experience (problems of juvenile justice. The author compares the doctrines of juvenile justice, including the doctrine of punitive and understanding justice.

  2. HIV-Risk Reduction with Juvenile Offenders on Probation

    OpenAIRE

    Donenberg, Geri R.; Emerson, Erin; Mackesy-Amiti, Mary Ellen; Udell, Wadiya

    2014-01-01

    Youth involved in the juvenile justice system are at elevated risk for HIV as a result of high rates of sexual risk taking, substance use, mental health problems and sexually transmitted infections. Yet few HIV prevention programs exist for young offenders. This pilot study examined change in juvenile offenders’ sexual activity, drug/alcohol use, HIV testing and counseling, and theoretical mediators of risk taking following participation in PHAT Life, an HIV-prevention progr...

  3. Paracoccidioidomycosis: acute-subacute clinical form, juvenile type*

    Science.gov (United States)

    Marques, Silvio Alencar; Lastória, Joel Carlos; de Camargo, Rosangela Maria Pires; Marques, Mariangela Esther Alencar

    2016-01-01

    The authors report aspects of paracoccidioidomycosis, acute-subacute clinical form, juvenile type, in a 19-year-old female patient. Paracoccidioidomycosis, juvenile type, classically occurs in young patients, both sexes, with lymphoma-like aspects as initial presentation. However, following the natural history of the disease the lymph nodes assume patterns of infectious disease, as an abscess and fistulae. Systemic dissemination of the disease can occur and lethality and morbidity are significant in this clinical presentation. PMID:27438214

  4. Paracoccidioidomycosis: acute-subacute clinical form, juvenile type*

    OpenAIRE

    Marques, Silvio Alencar; Last?ria, Joel Carlos; de Camargo, Rosangela Maria Pires; Marques, Mariangela Esther Alencar

    2016-01-01

    Abstract: The authors report aspects of paracoccidioidomycosis, acute-subacute clinical form, juvenile type, in a 19-year-old female patient. Paracoccidioidomycosis, juvenile type, classically occurs in young patients, both sexes, with lymphoma-like aspects as initial presentation. However, following the natural history of the disease the lymph nodes assume patterns of infectious disease, as an abscess and fistulae. Systemic dissemination of the disease can occur and lethality and morbidity a...

  5. Paracoccidioidomycosis: acute-subacute clinical form, juvenile type.

    Science.gov (United States)

    Marques, Silvio Alencar; Lastória, Joel Carlos; Camargo, Rosangela Maria Pires de; Marques, Mariangela Esther Alencar

    2016-01-01

    The authors report aspects of paracoccidioidomycosis, acute-subacute clinical form, juvenile type, in a 19-year-old female patient. Paracoccidioidomycosis, juvenile type, classically occurs in young patients, both sexes, with lymphoma-like aspects as initial presentation. However, following the natural history of the disease the lymph nodes assume patterns of infectious disease, as an abscess and fistulae. Systemic dissemination of the disease can occur and lethality and morbidity are significant in this clinical presentation.

  6. [Morphea or juvenile localised scleroderma: Case report].

    Science.gov (United States)

    Strickler, Alexis; Gallo, Silvanna; Jaramillo, Pedro; de Toro, Gonzalo

    2016-01-01

    Morphea or juvenile localised scleroderma (JLS) is an autoimmune, inflammatory, chronic, slowly progressive connective tissue disease of unknown cause that preferably affects skin and underlying tissues. To report a case of Juvenil Localised scleroderma in an 8-year old girl, contributing to an early diagnosis and treatment. The case is presented of an 8 year-old girl who presented with indurated hypopigmented plaques, of linear distribution in the right upper extremity of two years onset, together with papery texture hyperpigmented indurated plaques with whitish areas of thinned skin in right lower extremity, and leg and ankle swelling. The clinical features and diagnostic tests, including histology were compatible with linear and pansclerotic JLS. She started with immunosuppressive therapy, physiotherapy, and occupational therapy. We report a case of linear and pansclerotic ELJ type, in which there was a 2 year delay in diagnosis, however the response to treatment was positive as expected. Copyright © 2016 Sociedad Chilena de Pediatría. Publicado por Elsevier España, S.L.U. All rights reserved.

  7. Biological stress systems, adverse life events and the onset of chronic multisite musculoskeletal pain : a six-year cohort study

    NARCIS (Netherlands)

    Generaal, E.; Vogelzangs, N.; Macfarlane, G.J.; Geenen, R.; de Geus, E.; Smit, J.H.; Penninx, B.W.; Dekker, J.

    2015-01-01

    Background Dysregulated biological stress systems and adverse life events, both independently and in interaction, have been hypothesized to initiate chronic pain. Objectives We examine whether (i) function of biological stress systems, (ii) adverse life events, and (iii) their combination predict

  8. Treatment of Juvenile Idiopathic Arthritis-Associated Uveitis

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    İlknur Tuğal-Tutkun

    2016-04-01

    Full Text Available Pediatric uveitis may be a serious health problem because of the lifetime burden of vision loss due to severe complications if the problem is not adequately treated. Juvenile idiopathic arthritis (JIA-associated uveitis is characterized by insidious onset and potentially blinding chronic anterior uveitis. Periodic ophthalmologic screening is of utmost importance for early diagnosis of uveitis. Early diagnosis and proper immunomodulatory treatment are essential for good visual prognosis. The goal of treatment is to achieve enduring drug-free remission. The choice of therapeutic regimen needs to be tailored to each individual case. One must keep in mind that patients under immunomodulatory treatment should be monitored closely due to possible side effects. Local and systemic corticosteroids have long been the mainstay of therapy; however, long-term corticosteroid therapy should be avoided due to serious side effects. Steroid-sparing agents in the treatment of JIA-associated uveitis include antimetabolites and biologic agents in refractory cases. Among the various immunomodulatory agents, methotrexate is generally the first choice, as it has a well-established safety and efficacy profile in pediatric cases and does not appear to increase the risk of cancer. Other classic immunomodulators that may also be used in combination with methotrexate include azathioprine, mycophenolate mofetil, and cyclosporin A. Biologic agents, primarily tumor necrosis factor alpha inhibitors including infliximab or adalimumab, should be considered in cases of treatment failure with classic immunomodulatory agents.

  9. Magnetic resonance imaging assessment of the ventricular system in the brains of adult and juvenile beagle dogs treated with posaconazole IV Solution.

    Science.gov (United States)

    Hines, C D G; Song, X; Kuruvilla, S; Farris, G; Markgraf, C G

    2015-01-01

    Noxafil® (posaconazole; POS) is a potent, selective triazole antifungal approved for use in adults as an oral suspension, oral tablet and intravenous (IV) Solution. In support of pediatric administration of POS IV Solution to childrentwo years of age, two studies were undertaken using magnetic resonance imaging (MRI) to monitor brain ventricle size longitudinally during three months administration of POS IV in adult and juvenile dogs. Necropsy was performed on all animals at the end of the studies. From the baseline MRI images, great variability in ventricle size was noted in both the adult and juvenile dogs; these images were used to distribute differently sized ventricles between treatment and vehicle groups as to not skew group means during the course of the study. POS IV Solution had no effect on ventricle volume at any timepoint during dosing in either the adult or the juvenile dogs. Further, no gross or histomorphologic differences between groups were observed in either study. Compared to juvenile dogs, MRI analysis showed that adult dogs had larger ventricles, lower variability in all ventricle volumes, and a greater rate of increase in total ventricle volume. Information on growth and development of brains is one of the few areas in which more detailed information is available about humans than about the standard laboratory animals used to model disease and predict toxicities. The use of MRI helped elucidate large natural variabilities in the dog brain, which could have altered the interpretation of this de-risking study, and provided a valuable noninvasive means to monitor the brain ventricles longitudinally. Copyright © 2015 Elsevier Inc. All rights reserved.

  10. Effects of partial replacement of fish meal by yeast hydrolysate on complement system and stress resistance in juvenile Jian carp (Cyprinus carpio var. Jian).

    Science.gov (United States)

    Yuan, Xiang-Yang; Liu, Wen-Bin; Liang, Chao; Sun, Cun-Xin; Xue, Yun-Fei; Wan, Zu-De; Jiang, Guang-Zhen

    2017-08-01

    A 10-week feeding trial was carried out to investigate the effects of dietary fish meal replacement by yeast hydrolysate (YH) on growth performance, complement system and stress resistance of juvenile Jian carp (Cyprinus carpio var. Jian) (initial average weight 19.44 ± 0.06 g). In the study, there were five groups: one control group was fed with a basal diet (YH0), and four treatment groups were fed with dietary fish meal replaced by 1% YH (YH1), 3% (YH3), 5% (YH5) and 7% (YH7), respectively. Each group had four replicates. At the end of feeding trial, twelve fish from each group (three fish per replicate) were randomly selected for assessing the growth and immunity. Meanwhile, 20 fish per replicate were injected by Aeromonas hydrophila. The results showed that (1) Replacement levels of YH significantly affected the growth of the fish with the highest values of weight gain (WG) occurred in fish fed YH3 diet. However, no significant difference in feed conversion ratios (FCR) was observed among all groups. (2) Pre-stressed plasma lysozyme activity, total protein and albumin contents and complement component 3 (C3) and complement component 4 (C4) levels of fish fed YH3 diet were significantly higher than those of fish fed YH0 diet. However, post-stressed immune parameters of fish in all groups were significantly lower. (3) There was a trend that the expression levels of the complement-related genes (c1r/s-A, c4-1, c3-H1, c5-1, fb/c2-A, mbl-2 and masp) initially increased and then decreased except mbl-2 and masp, with the maximum values observed in fish fed YH3 diet. Before stress, the expression levels of the inflammation-related genes (alp, il-1β and tnf-α) in the hepatopancreas and spleen of fish fed YH1 diet and YH7 diet were significant higher than that of fish fed YH0 diet. After stress, no significant difference in the expression levels of those genes was observed among all groups. These results indicated that FM replacement by YH could improve growth

  11. Tocilizumab in systemic juvenile idiopathic arthritis in a real-world clinical setting: results from 1 year of postmarketing surveillance follow-up of 417 patients in Japan.

    Science.gov (United States)

    Yokota, Shumpei; Itoh, Yasuhiko; Morio, Tomohiro; Origasa, Hideki; Sumitomo, Naokata; Tomobe, Minako; Tanaka, Kunihiko; Minota, Seiji

    2016-09-01

    To evaluate the safety and effectiveness of tocilizumab (TCZ) in patients with systemic juvenile idiopathic arthritis (sJIA) in real-world clinical settings in Japan. Paediatric patients with sJIA initiating TCZ between April 2008 and February 2012 and those previously enrolled in clinical trials who initiated TCZ before April 2008 were enrolled in a Japanese registry surveillance programme. Safety and effectiveness parameters were collected for 52 weeks. Of 417 patients enrolled, mean age was 11.2 years and 48.0% were female. TCZ exposure was 407.0 patient-years (PYs). Baseline corticosteroid use was higher than in clinical trials. Rates of total adverse events (AEs) and serious AEs (SAEs) were 224.3/100 PYs and 54.5/100 PYs, respectively, with SAEs higher than previously reported. The most frequent AEs and SAEs were infections and infestations (69.8/100 PYs and 18.2/100 PYs, respectively). 74 serious infections occurred in 55 patients (18.2/100 PYs); higher than previously reported. 26 macrophage activation syndrome events were reported in 24 patients (6.4/100 PYs). Fever and rash symptoms improved from baseline to week 52 (54.6% to 5.6% and 43.0% to 5.6%, respectively). At 4 weeks, 8 weeks and 52 weeks, 90.5%, 96.2% and 99.0% of patients achieved normal C reactive protein levels (<0.3 mg/dL), respectively. These first real-world data demonstrated that TCZ was well tolerated, with acceptable safety and effectiveness in patients with sJIA. Higher incidences of SAEs and serious infections may be due to differences, such as corticosteroid use and concomitant diseases, between patient populations enrolled in previously reported clinical trials and this study. 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/

  12. Juvenile dermatomyositis: a tertiary center experience.

    Science.gov (United States)

    Barut, Kenan; Aydin, Pinar Ozge Avar; Adrovic, Amra; Sahin, Sezgin; Kasapcopur, Ozgur

    2017-02-01

    Juvenile dermatomyositis (JDM) is a rare chronic inflammatory disease of unknown etiology and primarily involves muscle and skin. It is the most common idiopathic inflammatory myopathy of childhood. This study aimed to evaluate demographic and clinical features, laboratory data, treatment modalities, and outcome of patients with JDM at a referral pediatric rheumatology center in Turkey. We retrospectively reviewed medical records of patients diagnosed with JDM between the years 2003-2016 at the Pediatric Rheumatology Department Cerrahpasa Medical Faculty. A total of 50 patients (35 females), median age at the onset 6.1 ± 4.1 years, were identified. Mean follow-up period was 74.5 ± 49.7 months. Presenting clinical symptoms included heliotrope rash (100%), Gottron papule (96%), muscle weakness (90%), erythroderma (88%), and calcinosis (38%). All patients had elevated muscle enzymes at the disease onset. Sixty-eight percent of the patients had anti-nuclear antibody positivity. Electromyography on 27 patients and muscle biopsy on 14 patients were performed, and all of them showed signs of juvenile dermatomyositis. Early aggressive treatment with corticosteroids mostly in combination with methotrexate was used. Cyclosporine was added to 48% of the patients' treatment regimen in case of severe or refractory disease. All patients except two cases, who were referred to our clinic after long disease duration with widespread calcinosis, achieved remission. Early diagnosis and early initiation of intensive therapy are important in reducing JDM complications. International collaboration is needed in order to better understanding and management of the disease.

  13. Design of an electronic medical record (EMR-based clinical decision support system to alert clinicians to the onset of severe sepsis

    Directory of Open Access Journals (Sweden)

    Fountain S

    2016-04-01

    Full Text Available Background: The aim of our study was to design an electronic medical record ­based alert system to detect the onset of severe sepsis with sensitivity and positive predictive value (PPV above 50%. Methods: The PPV for each of seven potential criteria for suspected infection (white blood cell count (WBCC >12 or 0.1 K/uL or immature granulocyte % >1%, temperature >38 C. or 50%, the charts of sixty consecutive patients who met CMS criteria for severe sepsis were reviewed to calculate the sensitivity of organ dysfunction plus any one of the suspected infection criteria. Results: Four proposed criteria for suspected infection had PPV >50%: WBCC >12 x 10 9 /L (69%; 95%CI:53­84%, Temperature >38C. (84%; 95%CI:68­100%, Temperature <36C. (57% 95%CI:36­78%, and initiation of antibiotics (70% 95%CI:56­84%. These four criteria were present in 53/60 of the patients with severe sepsis by CMS criteria, yielding a sensitivity of 88.3% (95%CI: 80.2­96.4%. Alert criteria were satisfied before the onset of severe sepsis in 25/53 cases, and within 90 minutes afterwards in 28/53 cases. Conclusions: Our criteria for suspected infection plus organ dysfunction yields reasonable sensitivity and PPV for the detection of severe sepsis in real­time.

  14. Utility of the systemic inflammatory response syndrome (SIRS) criteria in predicting the onset of septic shock in hospitalized patients with hematologic malignancies.

    Science.gov (United States)

    Mato, Anthony; Fuchs, Barry D; Heitjan, Daniel F; Mick, Rosemarie; Halpern, Scott D; Shah, Payal D; Jacobs, Samamtha; Olson, Erin M; Schuster, Stephen J; Ujjani, Chaitra; Chong, Elise A; Loren, Alison W; Miltiades, Andrea N; Luger, Selina

    2009-06-01

    The systemic inflammatory response syndrome (SIRS) criteria have not been validated in patients with hematologic malignancies (HM). To determine whether daily assessment of SIRS criteria allows early identification of HM patients who will develop septic shock (SS). Observational, single-center,nested case-control study. Oncology unit of a tertiary care center. 547 consecutive, hospitalized, HM subject were enrolled. Using incidence-density sampling, 184 controls were matched to 46 SS cases. The study exposure was the SIRS score. The study outcome was the development of SS during the hospitalization. 8.4% of subjects developed SS. SIRS scores measured 24 hours prior to SS were significantly higher in cases than in controls (2.1 vs. 1.4,pSIRS cutpoints, fever, tachypnea and tachycardia were each associated with the onset of SS. Population-specific SIRS criteria were empirically derived. Single-center study. Further validation is warranted. SIRS can identify HM patients at risk for SS at least 24 hours before SS onset. These data may lead to evidence-based guidelines using routine vital signs to risk-stratify HM patients for SS.

  15. Juvenile delinquency in Russia: Ccriminal justice, trends, key issues

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    Kovačević Milica

    2015-01-01

    Full Text Available In the literature, as well as in international statistical surveys, we can rarely find more detailed information on juvenile delinquency in the Russian Federation, as well as on the criminal reaction towards juvenile offenders. Due to the turbulent conditions and great social turmoil which took place in the last few decades in this country, there should be a greater interest in the problem of juvenile delinquency. For Serbia, the experience of the Russian Federation could be especially important if one bears in mind that our country is still going through a transition, population stratification and through economic crisis, and also that Serbia and the Russian Federation share some cultural and religious similarities. Therefore, the aim of this paper is to present, in the summary way, the basic features of juvenile delinquency and the criminal justice system, and thereby build a basis for future research and comparison.

  16. Juvenile prison in parallel legislation

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    Lutovac Mitar

    2016-01-01

    Full Text Available The need for punishment of juveniles occurred from the time when there was no clear line separating them from the adult criminal population. At the same time, the evolution of the juvenile punishment is not in itself involve substantial changes to their criminal status. On the contrary, the status of minors in society did not show serious differences regarding the status of young adults, as well as the adult elderly. On the other hand, on the ground of their punishment is recorded deviations that go in the direction of application of mild corporal punishment. Closing the minor was performed in a physically separate parts of the general penal institutions with the use of a lower degree of restrictions while serving juvenile prison. Due to the different treatment of minors during the evolution of their criminal status leads to their different treatment in comparative law. That is why we are witnessing the existence of numerous differences in the juvenile punishment in some countries in the world. On the European continent there is a wide range of different legal solutions when it comes to punishing juveniles. There are considerable differences in the procedure pronouncing juvenile prison and in particular penal treatment of juveniles in penitentiary institutions. For these reasons, the author has decided to show the basic statutory provisions in the part that relates to the issue of punishment of minors in the legislation of individual countries.

  17. Recurrent giant juvenile fibroadenoma

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    Kathryn S. King

    2017-11-01

    Full Text Available Breast masses in children, though rare, present a difficult clinical challenge as they can represent a wide variety of entities from benign fibroadenomas to phyllodes tumors. Rapidly growing or recurrent masses can be particularly concerning to patients, families and physicians alike. Clinical examination and conventional imaging modalities are not efficacious in distinguishing between different tumor types and surgical excision is often recommended for both final diagnosis and for treatment of large or rapidly growing masses. While surgical excision can result in significant long-term deformity of the breast there are some surgical techniques that can be used to limit deformity and/or aid in future reconstruction. Here we present a case of recurrent giant juvenile fibroadenoma with a review of the clinical presentation, diagnostic tools and treatment options.

  18. Clinical and Biochemical Characteristics of Children with Juvenile Idiopathic Arthritis

    International Nuclear Information System (INIS)

    Ahmed, S.; Ali, S. R.; Ishaque, S.

    2014-01-01

    Objective: To determine the clinical and biochemical characteristics of children with Juvenile Idiopathic Arthritis (JIA) at a tertiary care centre in Karachi, Pakistan. Study Design: A descriptive study. Place and Duration of Study: Paediatric Rheumatology Clinic of The Aga Khan University Hospital (AKUH), Karachi, from January 2008 to December 2011. Methodology: Clinical and laboratory profile and outcome of children less than 15 years of age attending the Paediatric Rheumatology Clinic of the Aga Khan University, Karachi with the diagnosis of Juvenile Idiopathic Arthritis according to International League against Rheumatism were studied. These children were classified into different types of JIA; their clinical and laboratory characteristics, response to therapy and outcome was evaluated. Results: Sixty eight patients satisfying the criteria of International League against Rheumatism (ILAR) for Juvenile Idiopathic Arthritis were enrolled during the study period of four consecutive years, their age ranged from 9 months to 15 years. Mean age at onset was 6.45 +- 4.03 years while mean age at diagnosis was 7.60 +- 3.93 years. Polyarticular was the most predominant subtype with 37 (54%) patients, out of these, 9 (24%) were rheumatoid factor positive. An almost equal gender predisposition was observed. Fever and arthritis were the most common presenting symptoms, with only 2 patients presenting with uveitis. Conclusion: The clinico-biochemical characteristics of JIA at the study centre showed a pattern distinct with early onset of disease, high frequency of polyarticular type and a higher rheumatoid factor (QRA) and ANA positivity in girls. (author)

  19. Juvenile homosexual homicide.

    Science.gov (United States)

    Myers, Wade C; Chan, Heng Choon Oliver

    2012-01-01

    Limited information exists on juvenile homosexual homicide (JHH), that is, youths who perpetrate sexual homicides against same-sex victims. Only a handful of cases from the United States and internationally have been described in the literature. This study, the first of its kind, examines the epidemiology, victimology, victim-offender relationship, and weapon-use patterns in JHH offenders using a large U.S. database on homicide spanning three decades. The data for this study were derived from the Federal Bureau of Investigation's Supplementary Homicide Reports (SHRs) for the years 1976 through 2005. A total of 93 cases of JHH were identified. On average, three of these crimes occurred annually in the U.S., and there was a marked decline in its incidence over the study period. Ninety-five percent were male offender-male victim cases and 5% were female offender-female victim cases. JHH offenders were over-represented amongst all juvenile sexual murderers, similar to their adult counterparts. The majority of these boys were aged 16 or 17 and killed adult victims. They were significantly more likely to kill adult victims than other age groups, to be friends or acquaintances of the victims, and to use contact/edged weapons or firearms. Most offenders killed same-race victims, although Black offenders were significantly more likely than White offenders to kill interracially. A case report is provided to illustrate JHH. Further research is needed to promote our understanding of the pathogenesis, etiology, and associated risk factors for this aberrant form of murder by children. Copyright © 2012 John Wiley & Sons, Ltd.

  20. Analysis of the Juvenile Idiopathic Arthritis Immunization Schedule

    Directory of Open Access Journals (Sweden)

    L. S. Namazova-Baranova

    2016-01-01

    Full Text Available Background: The connection between vaccination and autoimmune diseases (and rheumatic pathology in particular is still a subject of discussions. When discussing the possibility of vaccinating rheumatic patients we should take into account the ultra high dangers that infectious diseases pose for such patients, including those that can be prevented by vaccination. We should also take into account the experience of using various vaccine types in rheumatic patients, which illustrates of their high safety profile.Objective: Our aim was to study the immunization schedule in children with juvenile idiopathic arthritis.Methods: The evaluation of vaccine history and other anamnestic data in juvenile idiopathic arthritis patients was based on individual medical records (individual child’s card/preventive vaccination certificate, as well as questionnaires filled by mothers.Results: It has been determined that a significant proportion of children with vaccination schedule deviations are juvenile idiopathic arthritis patients. Almost one in four children with a confirmed rheumatic diagnosis has not been immunized against the major vaccine-preventable diseases. In one non-vaccinated group, there was a case of juvenile arthritis onset after recovering from measles. A small number of patient mothers connects the manifestation of rheumatic diseases with vaccination.Conclusion: Violations of vaccination status in JIA patients require corrections according to the results of clinical studies and the recommendations of international experts.

  1. Evolution of recidivism risk, using the YLS/CMI Inventory in a population of juvenile offenders

    Directory of Open Access Journals (Sweden)

    Keren Cuervo Gómez

    2017-05-01

    Full Text Available Risk assessment in juvenile recidivism allows understanding the specific factors that drives the juvenile to the commission of offences. Most of these juveniles will have a punctual relation with the justice system and only a small percentage will persist in this type of conducts. However, it seems that society perceives these juveniles as high risk offenders with high rates of recidivism. Hence, with the aim to clarify this topic, the objective of this paper is to explore the general risk of recidivism and the areas with higher risk, examining the risk of recidivism in a follow up period. Participants in this research were juveniles with a criminal record in the Juvenile Court of Castellón (N = 210. The Youth Level of Service/Case Management Inventory (YLS/CMI was administered to the juveniles along the follow up period of two years. Results show a majoritary profile of low risk juveniles, and a punctual relation with justice, rejecting the belief of dangerousness on juvenile offenders. On the contrary, juvenile recidivists with long criminal trajectories are in fact characterized by a high risk that would increase throughout the follow up period.

  2. THE CURRENT STATE OF LEGAL INSTITUTIONS PROTECTING THE RIGHTS OF JUVENILES IN RUSSIA

    OpenAIRE

    Yulia Hafizovna DAVYDOVA

    2015-01-01

    The article highlights the current state of the legislation on protection of the rights of under-aged. Much attention is paid to the formation of the juvenile justice system in the Russian Federation. We present problems related to law infringements by teenagers in various fields and their solutions, highlight the work and the development of juvenile courts in Russia, sum up the results of the implementation of juvenile technologies and their significance, gives the definition of the term «co...

  3. Early onset of disc degeneration in SM/J mice is associated with changes in ion transport systems and fibrotic events.

    Science.gov (United States)

    Zhang, Ying; Xiong, Chi; Kudelko, Mateusz; Li, Yan; Wang, Cheng; Wong, Yuk Lun; Tam, Vivian; Rai, Muhammad Farooq; Cheverud, James; Lawson, Heather A; Sandell, Linda; Chan, Wilson C W; Cheah, Kathryn S E; Sham, Pak C; Chan, Danny

    2018-04-09

    Intervertebral disc degeneration (IDD) causes back pain and sciatica, affecting quality of life and resulting in high economic/social burden. The etiology of IDD is not well understood. Along with aging and environmental factors, genetic factors also influence the onset, progression and severity of IDD. Genetic studies of risk factors for IDD using human cohorts are limited by small sample size and low statistical power. Animal models amenable to genetic and functional studies of IDD provide desirable alternatives. Despite differences in size and cellular content as compared to human intervertebral discs (IVDs), the mouse is a powerful model for genetics and assessment of cellular changes relevant to human biology. Here, we provide evidence for early onset disc degeneration in SM/J relative to LG/J mice with poor and good tissue healing capacity respectively. In the first few months of life, LG/J mice maintain a relatively constant pool of notochordal-like cells in the nucleus pulposus (NP) of the IVD. In contrast, chondrogenic events are observed in SM/J mice beginning as early as one-week-old, with progressive fibrotic changes. Further, the extracellular matrix changes in the NP are consistent with IVD degeneration. Leveraging on the genomic data of two parental and two recombinant inbred lines, we assessed the genetic contribution to the NP changes and identified processes linked to the regulation of ion transport systems. Significantly, "transport" system is also in the top three gene ontology (GO) terms from a comparative proteomic analysis of the mouse NP. These findings support the potential of the SM/J, LG/J and their recombinant inbred lines for future genetic and biological analysis in mice and validation of candidate genes and biological relevance in human cohort studies. The proteomic data has been deposited to the ProteomeXchange Consortium via the PRIDE [1] partner repository with the dataset identifier PXD008784. Copyright © 2017. Published by

  4. Prodromal Symptoms and Atypical Affectivity as Predictors of Major Depression in Juveniles: Implications for Prevention

    Science.gov (United States)

    Kovacs, Maria; Lopez-Duran, Nestor

    2010-01-01

    Background: Given the long-term morbidity of juvenile-onset major depressive disorder (MDD), it is timely to consider whether more effort should be dedicated to its primary and secondary prevention. Methods: We reviewed studies of prodromal symptoms that may herald a first episode pediatric MDD and considered whether that literature has made an…

  5. Aerobic and anaerobic exercise capacity in children with juvenile idiopathic arthritis

    NARCIS (Netherlands)

    van Brussel, M.; Lelieveld, O. T. H. M.; van der Net, J.; Engelbert, R. H. H.; Helders, P. J. M.; Takken, T.

    2007-01-01

    To compare the aerobic and anaerobic exercise capacity of children with juvenile idiopathic arthritis (JIA) with healthy controls, to determine if there were differences based on disease onset type, and to examine the relationship between aerobic and anaerobic exercise capacity in children with JIA.

  6. Aerobic and anaerobic exercise capacity in children with juvenile idiopathic arthritis

    NARCIS (Netherlands)

    van Brussel, Marco; Lelieveld, O T H M; van der Net, J; Engelbert, R H H; Helders, P J M; Takken, T

    2007-01-01

    Objective. To compare the aerobic and anaerobic exercise capacity of children with juvenile idiopathic arthritis (JIA) with healthy controls, to determine if there were differences based on disease onset type, and to examine the relationship between aerobic and anaerobic exercise capacity in

  7. Developing and Evaluating JIApp: Acceptability and Usability of a Smartphone App System to Improve Self-Management in Young People With Juvenile Idiopathic Arthritis.

    Science.gov (United States)

    Cai, Ran A; Beste, Dominik; Chaplin, Hema; Varakliotis, Socrates; Suffield, Linda; Josephs, Francesca; Sen, Debajit; Wedderburn, Lucy R; Ioannou, Yiannakis; Hailes, Stephen; Eleftheriou, Despina

    2017-08-15

    Flare-ups in juvenile idiopathic arthritis (JIA) are characterized by joint pain and swelling and often accompanied with fatigue, negative emotions, and reduced participation in activities. To minimize the impact of JIA on the physical and psychosocial development and well-being of young people (YP), it is essential to regularly monitor disease activity and side effects, as well as to support self-management such as adherence to treatment plans and engagement in general health-promoting behaviors. Smartphone technology has the potential to engage YP with their health care through convenient self-monitoring and easy access to information. In addition, having a more accurate summary of self-reported fluctuations in symptoms, behaviors, and psychosocial problems can help both YP and health care professionals (HCPs) better understand the patient's condition, identify barriers to self-management, and assess treatment effectiveness and additional health care needs. No comprehensive smartphone app has yet been developed in collaboration with YP with JIA, their parents, and HCPs involved in their care. The objective of this study was to design, develop, and evaluate the acceptability and usability of JIApp, a self-management smartphone app system for YP with JIA and HCPs. We used a qualitative, user-centered design approach involving YP, parents, and HCPs from the rheumatology team. The study was conducted in three phases: (1) phase I focused on developing consensus on the features, content, and design of the app; (2) phase II was used for further refining and evaluating the app prototype; and (3) phase III focused on usability testing of the app. The interview transcripts were analyzed using qualitative content analysis. A total of 29 YP (aged 10-23, median age 17) with JIA, 7 parents, and 21 HCPs were interviewed. Major themes identified as the ones that helped inform app development in phase I were: (1) remote monitoring of symptoms, well-being, and activities; (2

  8. System-wide significance of predation on juvenile salmonids in Columbia and Snake River reservoirs and evaluation of predation control measures. Annual report 1993

    International Nuclear Information System (INIS)

    Gadomski, D.M.; Poe, T.P.

    1994-01-01

    This project had three major goals. The first was to assist the Oregon Department of Fish and Wildlife with predation indexing as part of an effort to estimate the relative magnitude of juvenile salmonid losses to northern squawfish Ptychocheilus oregonensis in reservoirs throughout the Columbia River Basin. The second goal was to evaluate the northern squawfish control program and test critical assumptions about mid-reservoir predation processes. The final goal was to determine mechanisms underlying northern squawfish recruitment and factors affecting year-class strength

  9. Systemic Akt1 Deletion after Tumor Onset in p53−/− Mice Increases Lifespan and Regresses Thymic Lymphoma Emulating p53 Restoration

    Directory of Open Access Journals (Sweden)

    Wan-Ni Yu

    2015-07-01

    Full Text Available Akt is frequently activated in human cancers. However, it is unknown whether systemic inhibition of a single Akt isoform could regress cancer progression in cancers that are not driven by Akt activation. We systemically deleted Akt1 after tumor onset in p53−/− mice, which develop tumors independently of Akt activation. Systemic Akt1 deletion regresses thymic lymphoma in p53−/− mice emulating p53 restoration. Furthermore, pharmacological inhibition of Akt selectively kills thymic lymphoma cells and not primary thymocytes. Mechanistically, Akt1 inhibition in p53−/− thymic lymphoma inhibits Skp2 expression and induces FasL, which is the primary cause of cell death. Skp2 exerts resistance to cell death by antagonizing the induction of FasL and reducing FAS expression, which is linked to cyclin D1 expression. The results established a paradigm whereby systemic Akt1 inhibition is sufficient to regress tumors that are not driven by Akt activation and a mechanism of cell survival by Skp2.

  10. Initiation of stimulant and antidepressant medication and clinical presentation in juvenile bipolar I disorder.

    Science.gov (United States)

    Pagano, Maria E; Demeter, Christine A; Faber, Jon E; Calabrese, Joseph R; Findling, Robert L

    2008-03-01

    The primary purpose of this study was to examine the extent to which the initiation of stimulant and antidepressant medication was associated with the subsequent onset of juvenile bipolar I disorder (BP I). Another aim was to investigate differences in clinical presentation between youths prescribed stimulant or antidepressant medication before and after the onset of juvenile BP I disorder. Youths between the ages of 5 and 17 years meeting full, unmodified DSM-IV diagnostic symptom criteria for BP were included in this study. Data regarding the age of onset of BP I, psychiatric comorbidities, and current symptoms of mania and depression were obtained. Medication history was recorded as part of the assessment interview with parents and youths. Of the 245 youths with BP I, 65% (n = 160) were treated with stimulant medication; 32% (56/173) were treated after the onset of BP I, and 19% (32/173) were treated before the onset of BP I. Forty-six percent (113/245) were treated with antidepressant medication; 33% (67/206) were treated after the onset of BP I, and 3% (7/206) were treated before the onset of BP I. Patients who were treated with stimulants after the onset of BP I were significantly more likely to be younger (p presenting symptoms of depression or manic symptoms.

  11. JUVENILE DELINQUENCY: TRENDS (REGIONAL ASPECT

    Directory of Open Access Journals (Sweden)

    I. G. Selezneva

    2017-01-01

    Full Text Available he article analyzes the nature and internal structure of various types of crimes in which involved minors. Describes the main social factors contributing to this anomaly in the period of development of society. Investigated the motivation, the system and the types of crimes of minors in the Volgograd region, are the main trends of development of this phenomenon. The study also discusses the theoretical basis of the problem of the influence of economic stability on the species structure of juvenile delinquency. In this study the analysis of various types of deviance minors in different areas of the city of Volgograd. In the process of rapid modernization of communication processes most of today’s youth have not been able to quickly rebuild their behavior. Currently, the value-perception of the adolescents focused on the material benefits in terms of expanded economic interactions. In these conditions, social processes become increasingly removed from humane and spiritual orientations. The effective functioning of society in its interaction based on cooperation and understanding is of great importance to stimulate the positive trends in social sphere in modern Russia. The modern period of development, coupled with a drastic breaking of the foundations of life, the formation of new social relations and institutions and the destruction of the old, inevitably contributes to social tension, the reassessment of social and moral values and development of deviant behavior of minors. The advantages of this study are the involvement of local archives regional committees on Affairs of minors, was first introduced to active scientific revolution, as well as logical structuring and grouping of the main issues related to the dynamics and changes in the species structure of juvenile crime, which allowed us to perform a fairly extensive archive of statistical material. Based on this analysis, the authors made a

  12. Juvenile Fibromyalgia: Different from the Adult Chronic Pain Syndrome?

    Science.gov (United States)

    Kashikar-Zuck, Susmita; King, Christopher; Ting, Tracy V; Arnold, Lesley M

    2016-04-01

    While a majority of research has focused on adult fibromyalgia (FM), recent evidence has provided insights into the presence and impact of FM in children and adolescents. Commonly referred as juvenile fibromyalgia (JFM), youths, particularly adolescent girls, present with persistent widespread pain and cardinal symptoms observed in adult FM. A majority of youth with JFM continue to experience symptoms into adulthood, which highlights the importance of early recognition and intervention. Some differences are observed between adult and juvenile-onset FM syndrome with regard to comorbidities (e.g., joint hypermobility is common in JFM). Psychological comorbidities are common but less severe in JFM. Compared to adult FM, approved pharmacological treatments for JFM are lacking, but non-pharmacologic approaches (e.g., cognitive-behavioral therapy and exercise) show promise. A number of conceptual issues still remain including (1) directly comparing similarities and differences in symptoms and (2) identifying shared and unique mechanisms underlying FM in adults and youths.

  13. Does early onset of criminal behavior differentiate for whom serious mental illness has a direct or indirect effect on recidivism?

    Science.gov (United States)

    Matejkowski, Jason; Conrad, Aaron; Ostermann, Michael

    2017-02-01

    The involvement of people with serious mental illness (SMI) with the justice system may be a direct result of their disruptive/unsafe expression of psychiatric symptoms being responded to by law enforcement. SMI may also indirectly contribute to justice involvement, through exposure to environmental and social learning processes that place people with SMI at risk for criminal behavior. This study addresses the question: For whom does SMI directly or indirectly relate to criminal behavior? Mediation and conditional effects testing were used to examine the potential of early onset of criminal behavior to distinguish those groups for whom SMI displays a direct effect or an indirect effect on criminal recidivism. This study utilized a disproportionate random sample of 379 inmates released from New Jersey Department of Corrections; 190 of whom had SMI and 189 of whom did not have SMI. Data were collected from clinical and administrative records. Results indicate that criminal risk mediated the relationship between SMI and recidivism. This indirect effect was conditioned by whether the individual had a juvenile conviction. Specifically, for early start offenders, criminal risk was positively related to recidivism while this relationship was not observed for late start offenders. Juvenile criminal onset did not condition the direct effects of SMI on recidivism. A juvenile history of criminal involvement may signal the presence of heightened criminogenic need among adults with SMI. This simple indicator could function to differentiate for clinicians those adults who are good candidates for exploring further, and targeting for amelioration, criminogenic needs to reduce further criminal involvement. (PsycINFO Database Record (c) 2017 APA, all rights reserved).

  14. Fetal and juvenile radiotoxicity

    International Nuclear Information System (INIS)

    Sikov, M.R.

    1985-01-01

    This project is directed at obtaining detailed comparative information on the deposition, distribution, retention, and toxicity of radionuclides in the prenatal and juvenile mammal. Because quantitative data cannot necessarily be extrapolated to man, emphasis is also directed toward establishing patterns, phenomenologic interactions, and relationships which will be useful in determining appropriate exposure levels for rapidly growing infants or children and for pregnant women. Further dosimetry for an experiment to evaluate the effects of foster-rearing of newborn rats on the lifetime effects of 239 Pu exposure has demonstrated that most of the lifetime burden is derived from prenatal exposure and that milk contributes little in addition. Other measurements have confirmed a tentative observation that the lifetime burden in offspring is greater with near-term exposure than with exposure earlier in gestation. Additional results from a comparison of the embryotoxicity of 239 Pu and 241 Am have confirmed that, on the basis of dose administered to the dam, the former has a greater effect on the conceptus. Pilot studies indicate that 233 U is teratogenic, acting as a chemical rather than as a radiological teratogen. Studies with 239 Pu-exposed pregnant rabbits have shown that maternal distribution differs from that in rodents; concentration patterns in the placenta and membranes also differed. 4 figures, 1 table

  15. Juvenile osteochondritis dissecans.

    Science.gov (United States)

    Polousky, John D

    2011-03-01

    Juvenile osteochondritis dissecans (JOCD) has been a recognized entity for more than 100 years. Despite our long recognition of OCD, the natural history and most effective therapies are poorly understood. Although conclusive evidence of an exact cause is lacking, there is widespread agreement that JOCD is related to repetitive trauma. Patients with JOCD present with vague pain and occasionally, mechanical symptoms. The diagnosis of JOCD can be confirmed on plain radiographs. Magnetic resonance imaging has emerged as the study of choice to evaluate the stability of the lesion and integrity of the overlying articular cartilage. Treatment decisions are based on the stability of the lesion. Stable JOCD lesions should be treated initially with activity modification and possibly, immobilization. Unstable lesions and stable lesions not responding to an initial course of nonoperative therapy should be surgically treated. Surgical treatment is based on the radiographic and arthroscopic characteristics of the lesion. Multiple techniques from simple arthroscopic drilling and fixation to salvage techniques for cartilage restoration are discussed.

  16. Adult onset tic disorders

    OpenAIRE

    Chouinard, S.; Ford, B.

    2000-01-01

    BACKGROUND—Tic disorders presenting during adulthood have infrequently been described in the medical literature. Most reports depict adult onset secondary tic disorders caused by trauma, encephalitis, and other acquired conditions. Only rare reports describe idiopathic adult onset tic disorders, and most of these cases represent recurrent childhood tic disorders.
OBJECTIVE—To describe a large series of patients with tic disorders presenting during adulthood, to compare cl...

  17. Measurement of blood calprotectin (MRP-8/MRP-14) levels in patients with juvenile idiopathic arthritis.

    Science.gov (United States)

    Bojko, Jaryna

    2017-01-01

    The aim of the investigation was to compare blood calprotectin (MRP8/14, S100A 8/9) levels in patients with systemic-onset, polyarticular, RF-negative and oligoarticular subtypes of juvenile idiopathic arthritis (JIA), and to explore links between blood calprotectin levels and clinical and laboratory markers of JIA activity. Measurement of calprotectin in blood serum was performed in 160 patients with JIA followed up at Lviv Regional Council Public Institution "Western-Ukrainian Specialised Children's Medical Centre". Seventeen patients with systemic-onset JIA (sJIA) and 49 patients with other JIA subtypes (RF-negative polyarthritis and oligoarthritis) in the active phase of the disease were included in this study. Determination of calprotectin levels in blood serum was performed using EK-MRP8/14 Buhlmann Calprotectin reagents (Buhlmann, Switzerland) by the ELISA method. The results of the investigations showed that blood calprotectin levels were higher in patients with systemic-onset subtype of the disease (median 13,800 ng/ml), and differed significantly from levels in healthy children (median 1,800 ng/ml, p = 0.00002), levels in patients with articular subtypes of JIA (median 2,700 ng/ml, p = 0.000008), and patients with RF-negative polyarthritis (median 3,800 ng/ml, p = 0.003226) and oligoarthritis (median 2,500 ng/ml, p = 0.000009). The highest blood calprotectin levels were found in patients with newly diagnosed sJIA, the median being 32,500 ng/ml (range: 13,800-177,000 ng/ml). Direct correlations were found between blood calprotectin and JADAS 27 activity score ( p = 0.000009), ESR ( p = 0.000079) and CRP ( p = 0.000058). Blood calprotectin level is one of the measures that can be used to confirm the diagnosis of sJIA and to monitor the disease activity and therapy effectiveness.

  18. Clinical Prediction Making: Examining Influential Factors Related to Clinician Predictions of Recidivism among Juvenile Offenders

    Science.gov (United States)

    Calley, Nancy G.; Richardson, Emily M.

    2011-01-01

    This study examined factors influencing clinician predictions of recidivism for juvenile offenders, including youth age at initial juvenile justice system involvement, youth age at discharge, program completion status, clinician perception of strength of the therapeutic relationship, and clinician perception of youth commitment to treatment.…

  19. Best Implementation Practices: Disseminating New Assessment Technologies in a Juvenile Justice Agency

    Science.gov (United States)

    Young, Douglas; Moline, Karl; Farrell, Jill; Bierie, David

    2006-01-01

    Much has been written in recent years about advances in assessment technologies designed to aid decision making in the juvenile justice system. Adoption and implementation of this latest generation of actuarial tools, however, have lagged behind their development. Assessment in juvenile justice exemplifies the "science-practice gap" that…

  20. A Cost-Benefit Study of a Breaking the Cycle Program for Juveniles

    Science.gov (United States)

    Cowell, Alexander J.; Lattimore, Pamela K.; Krebs, Christopher P.

    2010-01-01

    The authors present a cost-benefit analysis of a Juvenile Breaking the Cycle (JBTC) program in Oregon designed to provide juvenile justice system monitoring and coordinated treatment and services to youth who are assessed as at high risk for recidivism and substance use. Detailed cost analyses are presented for youth in the JBTC program and a…

  1. Traumatic Experiences and Juvenile Amenability: The Role of Trauma in Forensic Evaluations and Judicial Decision Making

    Science.gov (United States)

    Riggs Romaine, Christina L.; Goldstein, Naomi E. Sevin; Hunt, Elizabeth; DeMatteo, David

    2011-01-01

    The legal files of 144 juveniles charged as adults in one Pennsylvania county were reviewed to investigate whether trauma-related information was included in evaluations of amenability to treatment and how that information related to legal decisions to keep youth in criminal court or decertify them to the juvenile system. Potentially traumatic…

  2. Completed Suicides Among Quebec Adolescents Involved with Juvenile Justice and Child Welfare Services

    Science.gov (United States)

    Farand, Lambert; Chagnon, Francois; Renaud, Johanne; Rivard, Michele

    2004-01-01

    In the Province of Quebec (Canada), adolescents involved with the child welfare and juvenile justice systems committed at least one third of all completed suicides in their age group in 1995 and 1996. Their risk of suicide, standardized for age and sex, was five times that of the general adolescent population, and female juvenile delinquents had…

  3. The Importance of Juvenile Root Traits for Crop Yields

    Science.gov (United States)

    White, Philip; Adu, Michael; Broadley, Martin; Brown, Lawrie; Dupuy, Lionel; George, Timothy; Graham, Neil; Hammond, John; Hayden, Rory; Neugebauer, Konrad; Nightingale, Mark; Ramsay, Gavin; Thomas, Catherine; Thompson, Jacqueline; Wishart, Jane; Wright, Gladys

    2014-05-01

    Genetic variation in root system architecture (RSA) is an under-exploited breeding resource. This is partly a consequence of difficulties in the rapid and accurate assessment of subterranean root systems. However, although the characterisation of root systems of large plants in the field are both time-consuming and labour-intensive, high-throughput (HTP) screens of root systems of juvenile plants can be performed in the field, glasshouse or laboratory. It is hypothesised that improving the root systems of juvenile plants can accelerate access to water and essential mineral elements, leading to rapid crop establishment and, consequently, greater yields. This presentation will illustrate how aspects of the juvenile root systems of potato (Solanum tuberosum L.) and oilseed rape (OSR; Brassica napus L.) correlate with crop yields and examine the reasons for such correlations. It will first describe the significant positive relationships between early root system development, phosphorus acquisition, canopy establishment and eventual yield among potato genotypes. It will report the development of a glasshouse assay for root system architecture (RSA) of juvenile potato plants, the correlations between root system architectures measured in the glasshouse and field, and the relationships between aspects of the juvenile root system and crop yields under drought conditions. It will then describe the development of HTP systems for assaying RSA of OSR seedlings, the identification of genetic loci affecting RSA in OSR, the development of mathematical models describing resource acquisition by OSR, and the correlations between root traits recorded in the HTP systems and yields of OSR in the field.

  4. Juvenile dermatomyositis: a 20-year retrospective analysis of treatment and clinical outcomes.

    Science.gov (United States)

    Sun, Chi; Lee, Jyh-Hong; Yang, Yao-Hsu; Yu, Hsin-Hui; Wang, Li-Chieh; Lin, Yu-Tsan; Chiang, Bor-Luen

    2015-02-01

    Juvenile dermatomyositis is a rare childhood multisystem autoimmune disease involving primarily the skin and muscles, and it may lead to long-term disability. This study aimed to describe the clinical course of juvenile dermatomyositis and determine if any early clinical or laboratory features could predict outcome. Medical charts of patients aged ≤18 years and diagnosed with juvenile dermatomyositis (according to the criteria of Bohan and Peter) at the Pediatric Department, National Taiwan University Hospital, between 1989 and 2009 were reviewed. The endpoints for disease assessment were complete clinical response and complete clinical remission. Cox's proportional hazards model was fitted to identify important predictors of complete clinical remission. A total of 39 patients with juvenile dermatomyositis were reviewed. Two-thirds were females, and the mean age at disease onset was 81.97 ± 46.63 months. The most common initial presentations were Gottron's papule (82.1%) and muscle weakness (82.1%). After excluding one patient with an incomplete record, the remaining 31 patients who had muscle weakness were analyzed; among them, 22 (70.97%) achieved complete clinical response, but only six (19.4%) achieved complete clinical remission. Multivariate analysis showed that female sex, negative Gowers' sign at disease onset, and positive photosensitivity at disease onset were favorable factors to achieve complete clinical remission. Moreover, covariate-adjusted survival curves were drawn for making predictions of complete clinical remission. Only 13 (33.33%) patients were symptom free at the end of follow up, whereas the other 26 suffered from different kinds of complications. None of them developed malignancy, but two (5.13%) patients died during the follow-up period. Factors such as male sex and Gowers' sign were unlikely to favor the achievement of complete clinical remission in juvenile dermatomyositis. Certain complications cannot be avoided, and thus more

  5. 76 FR 54978 - Special Immigrant Juvenile Petitions

    Science.gov (United States)

    2011-09-06

    ... Violence Against Women and Department of Justice Reauthorization Act of 2005 (VAWA 2005), Public Law 109... support and to juvenile court proceedings. The juvenile could bring a trusted adult (who is familiar with...

  6. 27 August 2001 substorm: Preonset phenomena, two main onsets, field-aligned current systems, and plasma flow channels in the ionosphere and in the magnetosphere

    Science.gov (United States)

    Mishin, V. M.; Mishin, V. V.; Lunyushkin, S. B.; Wang, J. Y.; Moiseev, A. V.

    2017-05-01

    We supplement the results of the 27 August 2001 substorm studied earlier in the series of papers. Described is the plasma flow in the nightside ionosphere from the near-polar region from the polar cap to the auroral oval during the substorm preonset phase and two expansion onsets, EO1 and EO2, produced by reconnection in the closed tail (magnetic reconnection (MR1) and in the open tail lobes (MR2), respectively. We discuss the location of the MR2 region (is it near, middle, and/or distant tail?) and the EO2 trigger mechanism. The upward substorm current wedge field-aligned current (FAC) and the downward FAC in the polar cap dusk sector that were both produced by different types of magnetosphere-ionosphere feedback instability are found to provide the main contribution to the system of FACs during EO1 and EO2. Also, we obtain the estimates for the EO1 and EO2 power and energy. Addressed are the variations in the tail lobe magnetic flux and their (variations) association with EO2. In addition, we describe a 3-D system of mesoscale cells, each of which involves a plasma vortex and a local FAC maximum. The cells of this system in the inner magnetosphere and in the tail lobes intensify one after other within 2 min interval. At last, we substantiate the assumption that the fast plasma flow recorded by the Cluster satellites 7 min prior to EO1 was a bursty bulk flow from the most distant tail.

  7. Early onset breast cancer in a registry-based sample of African-american women: BRCA mutation prevalence, and other personal and system-level clinical characteristics.

    Science.gov (United States)

    Pal, Tuya; Bonner, Devon; Kim, Jongphil; Monteiro, Alvaro N A; Kessler, Lisa; Royer, Robert; Narod, Steven A; Vadaparampil, Susan T

    2013-01-01

    Young Black women are disproportionately afflicted with breast cancer, a proportion of which may be due to BRCA1 and BRCA2 (BRCA) gene mutations. In a sample of Black women with early onset breast cancer, we evaluated BRCA mutations and explored personal and system-level clinical characteristics. Black women diagnosed with invasive breast cancer (age ≤50) were recruited through the state cancer registry. Participants completed a questionnaire, genetic counseling and BRCA testing. Of the 48 women who consented to study participation, 46 provided a usable biologic specimen for BRCA testing. The overall prevalence of BRCA mutations and variants of uncertain significance (VUS) in participants was 6.5% and 34.8%, respectively. Of these, only 14 were referred for genetic counseling prior to study enrollment. Overall, those participants who chose to undergo bilateral mastectomy had a higher number of relatives with breast and ovarian cancer (p = 0.024) and a higher household income (p = 0.009). BRCA mutation prevalence and the high prevalence of VUS in participants are consistent with prior studies. Furthermore, clinical factors such as family history and financial means may influence type of surgery recommended and chosen, at both the provider and patient level, respectively. Finally, the limited number of patients referred for genetic counseling prior to surgical treatment for breast cancer may represent a missed clinical opportunity to inform surgical decisions. © 2013 Wiley Periodicals, Inc.

  8. The effect of a high-carbohydrate meal on postprandial thermogenesis and sympathetic nervous system activity in boys with a recent onset of obesity.

    Science.gov (United States)

    Nagai, Narumi; Sakane, Naoki; Hamada, Taku; Kimura, Tetsuya; Moritani, Toshio

    2005-04-01

    The purpose of the present study was to investigate the thermic effect of food (TEF) and sympathetic nervous system (SNS) activity in obese boys. Ten obese (9.2+/-0.4 years) and 13 lean boys (8.8+/-0.4 years) were examined for energy expenditure and fat oxidation measured via indirect calorimetry for 3 hours after a high-carbohydrate (HC; 70% carbohydrate, 20% fat, and 10% protein) or a high-fat (HF; 20% carbohydrate, 70% fat, and 10% protein) meal served on 2 different days at random. The activity of the SNS was assessed by means of a power spectral analysis of the heart rate variability. The TEF, expressed as a percentage of the consumed energy, was significantly lower in obese boys than in lean boys after the HC meal; however, such a difference was not observed after the HF meal. Multiple regression analysis revealed that obesity was a significant variable contributing to the variances in the TEF induced by the HC meal. Moreover, after the HC meal, the boys with a recent onset of obesity (duration, frequency component of the heart rate variability, an index of thermoregulatory SNS functions, compared with the remaining obese and lean boys. In conclusion, obese boys possessed normal metabolic and sympathetic responses to the HF meal but showed a diminished thermogenic response to the HC meal, especially during the early phase of obesity.

  9. Acoustic Telemetry Evaluation of Juvenile Salmonid Passage and Survival at John Day Dam with Emphasis on the Prototype Surface Flow Outlet, 2008

    Energy Technology Data Exchange (ETDEWEB)

    Weiland, Mark A.; Ploskey, Gene R.; Hughes, James S.; Deng, Zhiqun; Fu, Tao; Monter, Tyrell J.; Johnson, Gary E.; Khan, Fenton; Wilberding, Matthew C.; Cushing, Aaron W.; Zimmerman, Shon A.; Faber, Derrek M.; Durham, Robin E.; Townsend, Richard L.; Skalski, John R.; Kim, Jina; Fischer, Eric S.; Meyer, Matthew M.

    2009-12-01

    The main purpose of the study was to evaluate the performance of Top Spill Weirs installed at two spillbays at John Day Dam and evaluate the effectiveness of these surface flow outlets at attracting juvenile salmon away from the powerhouse and reducing turbine passage. The Juvenile Salmonid Acoustic Telemetry System (JSATS) was used to estimate survival of juvenile salmonids passing the dam and also for calculating performance metrics used to evaluate the efficiency and effectiveness of the dam at passing juvenile salmonids.

  10. Skeletal muscle major histocompatibility complex class I and II expression differences in adult and juvenile dermatomyositis.

    Science.gov (United States)

    Shinjo, Samuel Katsuyuki; Sallum, Adriana Maluf Elias; Silva, Clovis Artur; Marie, Suely Kazue Nagahashi

    2012-08-01

    To analyze major histocompatibility complex expression in the muscle fibers of juvenile and adult dermatomyositis. In total, 28 untreated adult dermatomyositis patients, 28 juvenile dermatomyositis patients (Bohan and Peter's criteria) and a control group consisting of four dystrophic and five Pompe's disease patients were analyzed. Routine histological and immunohistochemical (major histocompatibility complex I and II, StreptoABComplex/HRP, Dakopatts) analyses were performed on serial frozen muscle sections. Inflammatory cells, fiber damage, perifascicular atrophy and increased connective tissue were analyzed relative to the expression of major histocompatibility complexes I and II, which were assessed as negatively or positively stained fibers in 10 fields (200X). The mean ages at disease onset were 42.0±15.9 and 7.3±3.4 years in adult and juvenile dermatomyositis, respectively, and the symptom durations before muscle biopsy were similar in both groups. No significant differences were observed regarding gender, ethnicity and frequency of organ involvement, except for higher creatine kinase and lactate dehydrogenase levels in adult dermatomyositis (pmajor histocompatibility complex I (96.4% vs. 50.0%, pmajor histocompatibility complex II expression (14.3% vs. 53.6%, p=0.004) was observed in juvenile dermatomyositis. Fiber damage (p=0.006) and increased connective tissue (pmajor histocompatibility complex I was an important finding for the diagnosis of both groups, particularly for juvenile dermatomyositis, whereas there was lower levels of expression of major histocompatibility complex II than major histocompatibility complex I. This finding was particularly apparent in juvenile dermatomyositis.

  11. Juvenile technologies in foreign publications

    Directory of Open Access Journals (Sweden)

    Shpagina E.M.

    2012-09-01

    Full Text Available The article provides the review of foreign publications, concerning the juvenile technologies used in France, Canada, Germany and Switzerland. The paper presents legal, social and psychotherapeutic aspects of juvenile judiciary in foreign countries. The authors paid special attention to the complexity of approaches to young children and teenagers who found themselves in complicated life circumstances or got into trouble with the law. The article gives examples of using the following techniques: cognitive-behavioral intervention, mediation, family therapy (including family background and family history, relations theory, narrative practices, utilization of «emotional intelligence» resources.

  12. On the Prevention of Juvenile Crime

    Science.gov (United States)

    Lelekov, V. A.; Kosheleva, E. V.

    2008-01-01

    Crimes committed by juveniles are among the most urgent social problems. Juvenile crime is as prevalent as crime itself is, and it has not been solved completely in any society and cannot be solved through law enforcement measures alone. In this article, the authors discuss the dynamics and structure of juvenile crime in Russia and present data…

  13. Visual Disability Among Juvenile Open-angle Glaucoma Patients.

    Science.gov (United States)

    Gupta, Viney; Ganesan, Vaitheeswaran L; Kumar, Sandip; Chaurasia, Abadh K; Malhotra, Sumit; Gupta, Shikha

    2018-04-01

    Juvenile onset primary open-angle glaucoma (JOAG) unlike adult onset primary open-angle glaucoma presents with high intraocular pressure and diffuse visual field loss, which if left untreated leads to severe visual disability. The study aimed to evaluate the extent of visual disability among JOAG patients presenting to a tertiary eye care facility. Visual acuity and perimetry records of unrelated JOAG patients presenting to our Glaucoma facility were analyzed. Low vision and blindness was categorized by the WHO criteria and percentage impairment was calculated as per the guidelines provided by the American Medical Association (AMA). Fifty-two (15%) of the 348 JOAG patients were bilaterally blind at presentation and 32 (9%) had low vision according to WHO criteria. Ninety JOAG patients (26%) had a visual impairment of 75% or more. Visual disability at presentation among JOAG patients is high. This entails a huge economic burden, given their young age and associated social responsibilities.

  14. The Sequential Intercept Model and Juvenile Justice: Review and Prospectus.

    Science.gov (United States)

    Heilbrun, Kirk; Goldstein, Naomi E S; DeMatteo, David; Newsham, Rebecca; Gale-Bentz, Elizabeth; Cole, Lindsay; Arnold, Shelby

    2017-07-01

    Behavioral health needs in justice-involved adolescents are an increasing concern, as it has been estimated that two-thirds of youths in the juvenile justice system now meet the criteria for one or more psychological disorders. This article describes the application of the Sequential Intercept Model (SIM), developed to describe five "points of interception" from standard prosecution into rehabilitation-oriented alternatives for adults (Munetz & Griffin, 2006), to juvenile justice. The five SIM intercepts are: (1) first contact with law enforcement or emergency services; (2) initial hearings and detention following arrest; (3) jails and courts (including problem-solving courts); (4) re-entry from jails, prisons and forensic hospitals; and (5) community corrections and community support, including probation and parole. Modifying the SIM for application with justice-involved adolescents, this article describes three examples of interventions at different intercepts: Intercept 1 (the Philadelphia Police School Diversion Program), Intercept 3 (problem-solving courts for juveniles), and Intercept 5 (juvenile probation). Relevant research evidence for each example is reviewed, and the further application of this model to juveniles is described. Copyright © 2017 John Wiley & Sons, Ltd. Copyright © 2017 John Wiley & Sons, Ltd.

  15. Long-term outcome in patients with juvenile idiopathic arthritis.

    Science.gov (United States)

    Minden, Kirsten; Niewerth, Martina; Listing, Joachim; Biedermann, Thomas; Bollow, Matthias; Schöntube, Monika; Zink, Angela

    2002-09-01

    To describe the long-term outcome of juvenile idiopathic arthritis (JIA). All patients with JIA referred to a pediatric rheumatology center between 1978 and 1988 were identified and invited to undergo an assessment. Patients with JIA from a population-based cohort from East Berlin were included. The outcome assessment considered changes in body function and structure (e.g., mortality, joint abnormalities, disease activity), activities at the individual level (Health Assessment Questionnaire), and participation in society (e.g., mobility, educational and vocational background). Of 260 eligible patients, 215 (83%) were evaluated. Subtypes of JIA at disease onset included oligoarthritis (40%), polyarthritis (14%), systemic arthritis (14%), psoriatic arthritis (1%), enthesitis-related arthritis (15%), and other arthritis (16%). Followup was conducted after a median of 16.5 years. No deaths occurred in this cohort. At followup, approximately half of the patients had active disease and/or changes in body structures to a variable extent. Approximately one-third of patients rated themselves as being functionally limited. Patients demonstrated good social integration: few mobility problems were reported, and the educational achievements of patients were higher and their rate of unemployment was lower compared with the age-matched population. No significant differences in outcome were found between the population-based and the referral-based cohorts. Even though approximately half of the JIA patients had more or less distinctive changes in body function and/or structure after a disease duration of >15 years, fewer than 10% were severely disabled or handicapped. Because JIA often persists into adulthood, long-term followup and care are necessary.

  16. Síndrome de ativação macrofágica em pacientes com artrite idiopática juvenil Macrophage activation syndrome in patients with juvenile idiopathic arthritis

    Directory of Open Access Journals (Sweden)

    Rogério do Prado

    2004-10-01

    Full Text Available A síndrome de ativação macrofágica (SAM é uma complicação rara das doenças reumáticas crônicas, particularmente a artrite idiopática juvenil (AIJ de início sistêmico. Este processo pode ser desencadeado por agentes infecciosos virais e bacterianos, neoplásicos, drogas antiinflamatórias não esteroidais ou drogas modificadoras da doença, mudanças abruptas das medicações e doenças reumáticas. O quadro clínico inicia-se com irritação do sistema nervoso central, acompanhado de falências hepática e renal, além de pancitopenia. Relatamos três casos de pacientes com AIJ do nosso serviço que desenvolveram SAM com descrição das características clínicas, evolutivas e de tratamento.The macrophage activation syndrome (MAS is an uncommon complication of chronic rheumatic diseases, specially systemic onset juvenile idiopathic arthritis (JIA. It can be triggered by infectious (viral or bacterial or malignant diseases, non-steroidal anti-inflammatory or disease modified anti-rheumatic drugs, changes in the therapy and rheumatic diseases. The clinical features present at the onset are related mainly with central nervous system involvement, hepatic and renal failure and pancytopenia. We describe the clinical, evolutive features and treatment of three patients with JIA that developed MAS.

  17. Measurements of fireball onset

    Science.gov (United States)

    Scheiner, Brett; Barnat, Edward V.; Baalrud, Scott D.; Hopkins, Matthew M.; Yee, Benjamin T.

    2018-04-01

    Laser-based measurements of the characteristic features of fireball onset and stabilization in response to a stepped voltage applied to an anode immersed in a low pressure (100 mTorr) helium afterglow are reported. These include spatial and temporal evolution of metastable species, electron density, and electric field magnitude as measured by planar laser induced fluorescence, laser-collision induced fluorescence, and laser-induced fluorescence-dip spectroscopy, respectively. These measurements are found to be in qualitative agreement with recent particle-in-cell simulations and theoretical models [Scheiner et al., Phys. Plasmas 24, 113520 (2017)]. The measurements validate the simulations and models in which fireball onset was predicted to follow from the trapping of electrons born from electron impact ionization within a potential well created by a buildup of ions in the sheath. The experimental measurements also demonstrate transient features following the onset that were not present in previous simulations. New simulation results are presented which demonstrate that these features are associated with the abruptness of the voltage step used to initiate fireball onset. An abrupt step in the anode bias causes rapid displacement of ions and an associated plasma potential response following the sheath and fireball expansion.

  18. Juvenile myopia progression, risk factors and interventions.

    Science.gov (United States)

    Myrowitz, Elliott H

    2012-07-01

    The development and progression of early onset myopia is actively being investigated. While myopia is often considered a benign condition it should be considered a public health problem for its visual, quality of life, and economic consequences. Nearly half of the visually impaired population in the world has uncorrected refractive errors, with myopia a high percent of that group. Uncorrected visual acuity should be screened for and treated in order to improve academic performance, career opportunities and socio-economic status. Genetic and environmental factors contribute to the onset and progression of myopia. Twin studies have supported genetic factors and research continues to identify myopia genetic loci. While multiple myopia genetic loci have been identified establishing myopia as a common complex disorder, there is not yet a genetic model explaining myopia progression in populations. Environmental factors include near work, education levels, urban compared to rural location, and time spent outdoors. In this field of study where there continues to be etiology controversies, there is recent agreement that children who spend more time outdoors are less likely to become myopic. Worldwide population studies, some completed and some in progress, with a common protocol are gathering both genetic and environmental cohort data of great value. There have been rapid population changes in prevalence rates supporting an environmental influence. Interventions to prevent juvenile myopia progression include pharmacologic agents, glasses and contact lenses. Pharmacological interventions over 1-2 year trials have shown benefits. Peripheral vision defocus has been found to affect the emmetropization process and may be affected by wearing glasses or contacts. Accommodation accuracy also has been implicated in myopia progression. Further research will aim to assess both the role and interaction of environmental influences and genetic factors.

  19. Juvenile European anchovy otolith microstructure

    Directory of Open Access Journals (Sweden)

    Pablo Cermeño

    2006-09-01

    Full Text Available Juvenile European anchovy (Engraulis encrasicolus has a complex incremental growth pattern that was studied using scanning electron microscope (SEM and optical microscope observations. Daily increments were identified and related to rhythmic growth patterns while double-band structures were identified as one increment. The causes of these growth patterns are discussed.

  20. Juvenile Courts. Creation and development

    Directory of Open Access Journals (Sweden)

    Montserrat GONZÁLEZ FERNÁNDEZ

    2013-11-01

    Full Text Available This paper studies the creation of Juvenile or Children's Courts in Spain, analysing their reasons and aims, as well as the ethical and political connotations present on their way of acting. Their history and the one of the institutions that complement them is built from the legislation, writings and ideas of their promoters.

  1. Novel FUS deletion in a patient with juvenile amyotrophic lateral sclerosis.

    Science.gov (United States)

    Belzil, Veronique V; Langlais, Jean-Sébastien; Daoud, Hussein; Dion, Patrick A; Brais, Bernard; Rouleau, Guy A

    2012-05-01

    Juvenile amyotrophic lateral sclerosis (JALS) refers to a form of amyotrophic lateral sclerosis (ALS) in which a progressive upper and lower motor neuron degeneration begins before 25 years of age. It is generally associated with slow disease progression. During the past decade, a number of genes have been reported to cause JALS. Mutations in the ALSIN gene cause JALS type 2 (ALS2) as well as juvenile primary lateral sclerosis and infantile-onset ascending spastic paralysis. Mutations in the SETX gene can also sometimes lead to JALS. Conversely, mutations in SOD1, TARDBP, and FUS typically cause pure ALS, with adult onset between 46 and 56 years of age and usually rapid progression over 3 to 5 years. Recently, a few mutations in FUS have been associated with juvenile-onset of ALS characterized by a very rapid progression. To investigate the genetics of a patient with juvenile-onset ALS. We sequenced all the coding exons of SOD1, TARDBP, and FUS in a 19-year-old patient experiencing rapid degeneration of upper and lower motor neurons. A novel 1-base pair deletion was detected in exon 14 of the FUS gene, leading to a frameshift and the integration of 33 new amino acids. The variant p.R495QfsX527 is located in the highly conserved, extreme C terminal of the FUS protein, where most of the mutations in FUS have been identified. The variant was also identified in the unaffected 47-year-old mother of the patient, who remains asymptomatic. Our finding, along with other research, further confirms that FUS mutations can lead to an early-onset malignant form of ALS. In addition, our data lend additional support to the notion that disruption of the conserved C terminal of FUS is critical for developing ALS.

  2. Desempenho diagnóstico e associações clínicas dos anticorpos contra componentes da cromatina no lúpus eritematoso sistêmico juvenil Diagnostic performance and clinical associations of antibodies to the chromatin antigenic system in juvenile systemic lupus erythematosus

    Directory of Open Access Journals (Sweden)

    Silene Peres Keusseyan

    2012-10-01

    Full Text Available OBJETIVOS: Determinar a frequência de anticorpos contra componentes da cromatina no lúpus eritematoso sistêmico juvenil (LESJ e correlacionar a presença desses autoanticorpos com manifestações clínicas e atividade da doença. MÉTODOS: Os anticorpos anticromatina (anti-CHR, antinucleossomo (anti-NCS e anti-dsDNA foram medidos em 175 indivíduos, incluindo 37 pacientes com LESJ ativo e 41 com doença inativa, 47 com doenças autoimunes não lúpicas, e 50 crianças saudáveis. Um teste ELISA in house foi desenvolvido com nucleossomos purificados a partir de timo de bezerro para determinar os anticorpos IgG e IgG3 anti-NCS. Anti-CHR e anti-dsDNA foram detectados por kits comerciais de ELISA (INOVA. RESULTADOS: Anticorpos anti-NCS e anti-CHR exibiram não só uma alta especificidade para LESJ, mas também uma frequência semelhante em LESJ ativo e inativo. Os níveis séricos de anti-CHR e IgG/IgG3 anti-NCS não diferiram entre LESJ ativo e inativo. Houve correlação entre o SLEDAI e os anticorpos anti-dsDNA, mas não com os anticorpos contra outros componentes da cromatina. Houve associação de anticorpos anti-dsDNA, anti-CHR e IgG/IgG3 anti-NCS com proteinúria e baixos níveis séricos de C4. Foram observados anticorpos anti-NCS em 14% dos pacientes com LESJ na ausência de anticorpos anti-dsDNA. CONCLUSÕES: Nossos dados indicam que os anticorpos anti-NCS e anti-CHR são marcadores diagnósticos relevantes para LESJ e parecem estar correlacionados com a atividade da nefrite lúpica no LESJ. O anticorpo IgG3 anti-NCS não parece ser mais relevante como marcador de atividade da doença ou nefrite ativa no LESJ em comparação ao anticorpo IgG anti-NCS.OBJECTIVES: To determine the frequency of antibodies to chromatin components in juvenile systemic lupus erythematosus (JSLE, and to correlate the presence of these autoantibodies with clinical manifestations and disease activity. METHODS: Anti-chromatin (anti-CHR, anti-nucleosome core

  3. Adolescent Neglect, Juvenile Delinquency and the Risk of Recidivism

    Science.gov (United States)

    Ryan, Joseph P.; Williams, Abigail B.; Courtney, Mark E.

    2013-01-01

    Victims of child abuse and neglect are at an increased risk of involvement with the juvenile justice and adult correctional systems. Yet, little is known about the continuation and trajectories of offending beyond initial contact with law enforcement. Neglect likely plays a critical role in continued offending as parental monitoring, parental…

  4. Restorative Justice: New Horizons in Juvenile Offender Counseling

    Science.gov (United States)

    Ryals, John S. Jr.

    2004-01-01

    Treatment strategies of the juvenile justice system focus singularly on rehabilitation of offenders, and victims and communities are excluded from the rehabilitative process. Restorative justice views victims and communities as essential components in rehabilitative efforts. In this article, the principles and practices of restorative justice,…

  5. HIV testing among non-incarcerated substance-abusing juvenile offenders.

    Science.gov (United States)

    Tolou-Shams, Marina; Conrad, Selby; Louis, Alaina; Shuford, Sarah Hart; Brown, Larry K

    2015-11-01

    Juvenile offenders are a subgroup of adolescents at particular risk for HIV/STI infection. Although HIV prevalence among these youth is low (justice system, which is known to have an extremely high rate of HIV infection. US constitutional mandates provide HIV/STI testing for incarcerated juveniles, but close to 80% of juvenile arrestees are never detained. Moreover, although they engage in similar HIV risk behaviors as those detained, they have limited access to available HIV/STI testing services. Thus, our study examined rates of lifetime HIV testing among a pilot sample of 60 court-involved, substance-using juveniles monitored in the community to explore rates of testing and the reasons related to lifetime testing among a high-risk, yet understudied US juvenile population.

  6. Juvenile Treatment with a Novel mGluR2 Agonist/mGluR3 Antagonist Compound, LY395756, Reverses Learning Deficits and Cognitive Flexibility Impairments in Adults in A Neurodevelopmental Model of Schizophrenia

    Science.gov (United States)

    Li, Meng-Lin; Gulchina, Yelena; Monaco, Sarah A.; Xing, Bo; Ferguson, Brielle R.; Li, Yan-Chun; Li, Feng; Hu, Xi-Quan; Gao, Wen-Jun

    2018-01-01

    Schizophrenia (SCZ) is a neurodevelopmental psychiatric disorder, in which cognitive function becomes disrupted at early stages of the disease. Although the mechanisms underlying cognitive impairments remain unclear, N-methyl-D-aspartate receptors (NMDAR) hypofunctioning in the prefrontal cortex (PFC) has been implicated. Moreover, cognitive symptoms in SCZ are usually unresponsive to treatment with current antipsychotics and by onset, disruption of the dopamine system, not NMDAR hypofunctioning, dominates the symptoms. Therefore, treating cognitive deficits at an early stage is a realistic approach. In this study, we tested whether an early treatment targeting mGluR2 would be effective in ameliorating cognitive impairments in the methylazoxymethanol acetate (MAM) model of SCZ. We investigated the effects of an mGluR2 agonist/mGluR3 antagonist, LY395756 (LY39), on the NMDAR expression and function in juveniles, as well as cognitive deficits in adult rats after juvenile treatment. We found that gestational MAM exposure induced a significant decrease in total protein levels of the NMDAR subunit, NR2B, and a significant increase of pNR2BTyr1472 in the juvenile rat PFC. Treatment with LY39 in juvenile MAM-exposed rats effectively recovered the disrupted NMDAR expression. Furthermore, a subchronic LY39 treatment in juvenile MAM-exposed rats also alleviated the learning deficits and cognitive flexibility impairments when tested with a cross-maze based set-shifting task in adults. Therefore, our study demonstrates that targeting dysfunctional NMDARs with an mGluR2 agonist during the early stage of SCZ could be an effective strategy in preventing the development and progression in addition to ameliorating cognitive impairments of SCZ. PMID:28213064

  7. Juvenile Crime, Juvenile Justice. Panel on Juvenile Crime: Prevention, Treatment, and Control.

    Science.gov (United States)

    McCord, Joan, Ed.; Widom, Cathy Spatz, Ed.; Crowell, Nancy A., Ed.

    This book discusses patterns and trends in crimes committed by children and adolescents, analyzing youth crime as a subset of general crime and studying the impact of race and gender. It evaluates different approaches to forecasting future crime rates. Data come from a national panel that examined what is known about juvenile crime and its…

  8. High rate of serious infection in juvenile idiopathic arthritis patients under biologic therapy in a real-life setting.

    Science.gov (United States)

    Brunelli, Juliana Barbosa; Schmidt, Ana Renata; Sallum, Adriana Maluf Elias; Goldenstein-Schainberg, Claudia; Bonfá, Eloisa; Silva, Clovis A; Aikawa, Nádia Emi

    2018-03-01

    To assess the rate of serious and/or opportunistic infections in juvenile idiopathic arthritis (JIA) patients from a single tertiary center under biologic therapy and to identify possible risk factors associated to these complications. A total of 107 JIA patients followed at the biologic therapy center of our tertiary university hospital using a standardized electronic database protocol including demographic data, clinical and laboratorial findings and treatment at baseline and at the moment of infection. Opportunistic infections included tuberculosis, herpes zoster and systemic mycosis. A total of 398 patient-yrs(py) were included. The median time of biologic exposure was 3.0 years (0.15-11.5). We observed 35 serious/opportunistic infectious events in 27 (25%) patients: 31(88.6%) were serious infections and four (11.4%) opportunistic infections. Serious/opportunistic infections rates were 10.6/100py for ETN, 10.9/100py for ADA, 2.6/100py for ABA and 14.8/100py for TCZ. Comparison of 27 patients with and 80 without infection showed a higher frequency of systemic-onset JIA, lower age at biologic therapy initiation and a history of previous serious infection (p biologic therapy in a real-life setting. Systemic-onset JIA, lower age at biologic therapy start and history of previous serious infections were important risk factors for these complications. Also, higher rates of severe infections comparing to the former studies was possibly due to elevated MTX doses in our patients.

  9. MRI and 2D-CSI MR spectroscopy of the brain in the evaluation of patients with acute onset of neuropsychiatric systemic lupus erythematosus

    International Nuclear Information System (INIS)

    Sundgren, P.C.; Jennings, J.; Gebarski, S.; Pang, Y.; Maly, P.; Attwood, J.T.; McCune, W.J.; Nan, B.

    2005-01-01

    MRI and 2D-CSI spectroscopy were performed in eight patients with systemic lupus erythematosus who presented with acute onset of neuropsychiatric lupus (NP-SLE), and in seven normal controls to evaluate for differences in metabolic peaks and metabolic ratios between the two groups. Also, the interval change of the metabolic peaks and their ratios during treatment in the NP-SLE patient group was evaluated. Metabolic peaks for N-acetyl-aspartate (NAA), choline (Cho), creatine (Cr), and lactate/lipids (LL) and their ratios (NAA/Cr, NAA/Cho, Cho/Cr, LL/Cr) were determined at initial presentation and 3 and 6 months later. In the eight lupus patients compared to the seven normal controls, NAA/Cho ratios were lower at presentation (1.05 vs 1.25; p = 0.004) and decreased even further at the three month follow-up (0.92 vs 1.05; p = 0.008). In contrast, both Cho/Cr (1.42 vs 1.26; p = 0.026) and LL/Cr ratios (0.26 vs 0.19; p = 0.002) were higher in the lupus patients at presentation compared to the controls and did not significantly change at three and six months follow-up. The NAA/Cr ratios were lower in the lupus patients compared to the controls at presentation but the difference was not statistically significant. However, the mean NAA/Cr significantly decreased from the initial examination to the three month follow-up (1.42 vs 1.32; p = 0.049) but did not significantly change from the three to the six month follow-up examinations. The NAA/Cr, Cho/Cr, and NAA/Cho ratios varied significantly (p < 0.05, p < 0.05, p < 0.05, respectively) between the 17 different locations measured in the brain in all eight patients and seven controls. Both the NAA/Cr ratios and the Cho/Cr ratios were also significantly lower in the gray matter than in the white matter (p < 0.0001) in both patients and controls, whereas the LL/Cr and NAA/Cho ratios were not significantly different. In conclusion, 2D-CSI MR spectroscopy may be useful in the early detection of metabolic CNS changes in NP

  10. The DSM-5 Limited Prosocial Emotions subtype of Conduct Disorder in incarcerated male and female juvenile delinquents.

    Science.gov (United States)

    Pechorro, Pedro; Jiménez, Lucía; Hidalgo, Victoria; Nunes, Cristina

    2015-01-01

    The aim of the present study was to analyze the relevance of the DSM-5's Conduct Disorder new Limited Prosocial Emotions (CD LPE) specifier in incarcerated juvenile delinquents. A sample of 201 males and 98 females from the Juvenile Detention Centers managed by the Portuguese Ministry of Justice diagnosed with Conduct Disorder (CD) was used. Results showed that male juvenile delinquents with the CD LPE specifier scored higher on callous-unemotional traits (CU), general psychopathic traits, psychopathy taxon membership, self-reported delinquency, and crime seriousness, and lower on prosocial behavior and social desirability, while female juvenile delinquents with the CD LPE specifier scored higher on callous-unemotional traits (CU) and general psychopathic traits, and lower on prosocial behavior. Significant associations for both genders were found between the CD LPE specifier and age of crime onset and first problems with the law. Copyright © 2015 Elsevier Ltd. All rights reserved.

  11. Using a Creative Intervention to Increase Self-Disclosure among Mandated Juveniles with Co-Occurring Disorders

    Science.gov (United States)

    Drew, Faith; Bitar, George W.; Gee, Robert; Graff, Chad; Springer, Paul

    2007-01-01

    Counselors providing treatment within the juvenile justice system encounter numerous challenges that are inherent in working with this population. One of the challenges includes providing treatment to adolescents who are entering the juvenile justice system with co-occurring mental health and substance use disorders. Given the challenges, creative…

  12. Lung lobe torsion in seven juvenile dogs.

    Science.gov (United States)

    Latimer, Christian R; Lux, Cassie N; Sutton, Jessie S; Culp, William T N

    2017-12-15

    CASE DESCRIPTION 7 juvenile (< 12 months old) dogs with lung lobe torsion were evaluated. CLINICAL FINDINGS All patients were male; breeds included Pug (n = 5), Chinese Shar-Pei (1), and Bullmastiff (1). Dyspnea and lethargy were the most common initial complaints, with a duration of clinical signs ranging from 1 to 10 days. A CBC showed leukocytosis and neutrophilia in all dogs. Anemia was present in 6 dogs, 2 of which received packed RBC transfusions. The diagnosis was made on the basis of results of thoracic radiography, CT, ultrasonography, or a combination of modalities. The left cranial lung lobe was most commonly affected (n = 4), followed by the right middle lung lobe (2) and the right cranial lung lobe (1). TREATMENT AND OUTCOME A lateral intercostal thoracotomy with lobectomy of the affected lobe was performed in all patients. All dogs survived to be discharged between 1 and 2 days postoperatively. Six of 7 owners contacted for follow-up information 7 to 170 months after discharge reported satisfaction with the treatment and no apparent signs of recurrence of disease. CLINICAL RELEVANCE The juvenile patients of this report were successfully treated surgically with no apparent complications. Clinicians should be aware of the possibility of lung lobe torsion when evaluating young dogs with clinical signs related to the respiratory system, including those with vague signs, to avoid undue delays in treatment.

  13. Juvenile offender as a crime victim

    Directory of Open Access Journals (Sweden)

    Vasiljević-Prodanović Danica

    2017-01-01

    Full Text Available There is a stereotype of an ideal victim that deserves attention and sympathy of the public because it is helpless and has not done anything to cause harm or damage inflicted on her. In real life, a victim can often have quite the opposite attributes and identities. Recent studies deal with the phenomenon of victim-offender overlap, or the circumstance in which an individual is the victim and the offender at the same time. Numerous authors state that offenders are more likely to become crime victims than non-offenders, while on the other hand, crime victims are more likely to become offenders than non-victims. This article provides an overview of some of the characteristics of the victim-offender overlap phenomenon in the context of adolescents, aiming to point out the theoretical determinants and practical implications of the relationship between offending and victimization of juveniles. Studying this relationship is a current topic in criminology and victimology, which also has applicative importance for making various decisions in juvenile justice system.

  14. Very Early-onset Schizophrenia with Secondary Onset Tic Disorder.

    Science.gov (United States)

    Telgote, Shilpa A; Pendharkar, Shreyas Shrikant; Kelkar, Amol D; Bhojane, Sachin

    2017-01-01

    Very early-onset schizophrenia (defined as an onset of psychosis before 13 years of age) is a rare and severe form of the disorder which is clinically and neurobiologically continuous with the adult-onset disorder. It is rarely reported tic disorder.

  15. Very early-onset schizophrenia with secondary onset tic disorder

    OpenAIRE

    Shilpa A Telgote; Shreyas Shrikant Pendharkar; Amol D Kelkar; Sachin Bhojane

    2017-01-01

    Very early-onset schizophrenia (defined as an onset of psychosis before 13 years of age) is a rare and severe form of the disorder which is clinically and neurobiologically continuous with the adult-onset disorder. It is rarely reported

  16. Challenges of the reform of the position of juvenile victims of crime in the Republic of Serbia

    Directory of Open Access Journals (Sweden)

    Mirić Filip

    2016-01-01

    Full Text Available The paper offers an analysis of the possible directions of the reform of juvenile criminal legislation with regard to the position of juvenile victims of crime in Serbia. The paper analyses provisions of national and international legal acts, relevant and important for the legal protection of juvenile victims. The aim is to point out the challenges and problems in the process of reform of juvenile law, which are directed towards building a “child-friendly justice” system. In this way, as the author points, it is possible to get valuable information on which direction the reform of juvenile criminal legislation should go in order to provide basis for prevention of secondary victimization of juvenile victims of crime in the criminal procedure.

  17. ALTERED HISTOLOGY OF THE THYMUS AND SPLEEN IN CONTAMINANT-EXPOSED JUVENILE AMERICAN ALLIGATORS

    Science.gov (United States)

    Morphological difference in spleen and thymus are closely related to functional immune differences. Hormonal regulation of the immune system has been demonstrated in reptilian splenic and thymic tissue. Spleens and thymus were obtained from juvenile alligators at two reference si...

  18. The outcome of patients with renal involvement in pediatric-onset systemic lupus erythematosus--a 20-year experience in Asia.

    Science.gov (United States)

    Lee, P-Y; Yeh, K-W; Yao, T-C; Lee, W-I; Lin, Y-J; Huang, J-L

    2013-12-01

    Systemic lupus erythematosus (SLE) predominantly affects women of childbearing age, but 15-20% of cases are diagnosed during childhood. It is important for physicians to understand the epidemiology and clinical presentation for early detection and diagnosis of this disease in difference races. The aim of this retrospective review was to provide a 20-year experience for initial clinical and laboratory manifestations and outcomes in pediatric-onset SLE (pSLE) in a medical center in Asia. We reviewed medical records between April 1990 and June 2012 of patients with a diagnosis of International Classification of Diseases, Ninth Revision (ICD-9) code 710.0 (SLE), who admitted or received follow-up in the Department of Pediatrics at Chang Chung Memorial Hospital. Patients with a diagnosis of SLE prior to their 18th birthday and followed up at our hospital were eligible for inclusion in this study. Medical records regarding age, gender, date of birth and diagnosis, clinical manifestations at diagnosis, laboratory results, image studies and the classification criteria were reviewed. Patients received regular outpatient department follow-up and laboratory survey every 1-6 months. The study cohort consisted of 189 patients; 164 females (86.87%) and 25 males (13.23%). The overall mean age at pSLE diagnosis was 12.62 ± 2.77 years. The most common clinical symptom was malar rash, followed by arthritis and oral ulcers. There was no significant difference in clinical and laboratory manifestations between females and males. More than half of the patients presented with renal involvement initially. The most common histological finding was Class IV lupus nephritis (LN), especially in males (p = 0.034) and young age. Even with severe LN, the rate of end-stage renal disease (ESRD) was low if adequate treatment was initiated. The 5, 10 and 15-year ESRD-free survival rates were 95.4%, 94.0% and 89.9% in patients with biopsy-proven LN. However, infection was the leading cause of

  19. Adult-onset cerebello-brainstem dominant form of X-linked adrenoleukodystrophy presenting as multiple system atrophy: case report and literature review.

    Science.gov (United States)

    Ogaki, Kotaro; Koga, Shunsuke; Aoki, Naoya; Lin, Wenlang; Suzuki, Kinuko; Ross, Owen A; Dickson, Dennis W

    2016-02-01

    X-linked adrenoleukodystrophy (X-ALD) is the most common peroxisomal disorder and is caused by ABCD1 mutations. A cerebello-brainstem dominant form that mainly involves the cerebellum and brainstem is summarized in a review of the literature, with autopsy-confirmed cases exceedingly rare. We report a 69-year-old White man who was diagnosed with this rare disorder and describe neuropathologic, ultrastructural and genetic analyses. He did not have adrenal insufficiency or a family history of X-ALD or Addison's disease. His initial symptom was temporary loss of eyesight at age 34 years. His major symptoms were chronic and progressive gait disorder, weakness in his lower extremities and spasticity, as well as autonomic failure and cerebellar ataxia suggesting possible multiple system atrophy (MSA). He also had seizures, hearing loss and sensory disturbances. His brain MRI showed no obvious atrophy or significant white matter pathology in cerebrum, brainstem or cerebellum. He died at age 69 years with a diagnosis of MSA. Microscopic analysis showed mild, patchy myelin rarefaction with perivascular clusters of PAS-positive, CD68-positive macrophages in the white matter most prominent in the cerebellum and occipital lobe, but also affecting the optic tract and internal capsule. Electron microscopy of cerebellar white matter showed cleft-like trilamellar cytoplasmic inclusions in macrophages typical of X-ALD, which prompted genetic analysis that revealed a novel ABCD1 mutation, p.R163G. Given the relatively mild pathological findings and long disease duration, it is likely that the observed pathology was the result of a slow and indolent disease process. We described a patient who had sporadic cerebello-brainstem dominant form of X-ALD with long clinical course, mild pathological findings, and an ABCD1 p.R163G substitution. We also review a total of 34 cases of adult-onset cerebello-brainstem dominant form of X-ALD. Although rare, X-ALD should be considered in the

  20. Feasibility and impact of a computerised clinical decision support system on investigation and initial management of new onset chest pain: a mixed methods study.

    Science.gov (United States)

    Johnson, Rachel; Evans, Maggie; Cramer, Helen; Bennert, Kristina; Morris, Richard; Eldridge, Sandra; Juttner, Katy; Zaman, Mohammed J; Hemingway, Harry; Denaxas, Spiros; Timmis, Adam; Feder, Gene

    2015-08-26

    Clinical decision support systems (CDSS) can modify clinician behaviour, yet the factors influencing their effect remain poorly understood. This study assesses the feasibility and acceptability of a CDSS supporting diagnostic and treatment decisions for patients with suspected stable angina. Intervention The Optimising Management of Angina (OMA) programme includes a CDSS guiding investigation and medication decisions for clinicians managing patients with new onset stable angina, based on English national guidelines, introduced through an educational intervention. Design and participants A mixed methods study i. A study of outcomes among patients presenting with suspected angina in three chest pain clinics in England before and after introduction of the OMA programme. ii. Observations of clinic processes, interviews and a focus group with health professionals at two chest pain clinics after delivery of the OMA programme. Medication and cardiovascular imaging investigations undertaken within six months of presentation, and concordance of these with the recommendations of the CDSS. Thematic analysis of qualitative data to understand how the CDSS was used. Data were analysed for 285 patients attending chest pain clinics: 106 before and 179 after delivery of the OMA programme. 40 consultations were observed, 5 clinicians interviewed, and a focus group held after the intervention. The proportion of patients appropriate for diagnostic investigation who received one was 50 % (95 CI 34-66 %) of those before OMA and 59 % (95 CI 48-70 %) of those after OMA. Despite high use of the CDSS (84 % of consultations), observations and interviews revealed difficulty with data entry into the CDSS, and structural and practical barriers to its use. In the majority of cases the CDSS was not used to guide real-time decision making, only being consulted after the patient had left the room. The OMA CDSS for the management of chest pain is not feasible in its current form. The CDSS was