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1.
Artigo em Inglês | MEDLINE | ID: mdl-31507092

RESUMO

OBJECTIVES: To study growth and puberty in a multi-national longitudinal prospective cohort of juvenile dermatomyositis (JDM). METHODS: Children with JDM ≤18 years in active phase from 31 countries were studied, analyzing height, weight and pubertal development in children with available anthropometric data and follow-up visits over two years. RESULTS: A total of 196/275 (71%) children were included. We found a significant reduction in parent-adjusted height z score over time in females (p<0.0001) and males (p=0.001), but with catch-up growth at the final study visit. Median BMI z score peaked at 6 months (p<0.0001) and was still significantly above baseline at the final study visit at a median of 26 months (p=0.007) after baseline with no gender difference. Females with a disease duration ≥12 months after onset had significantly lower parent-adjusted height z score (p=0.002) and no 2-year catch-up growth. Growth failure was seen in 20 (21%) of the females and 11 (15%) of the males, at the final study visit. Height deflection (∆height z-score <-0.25/year) was observed in 29 (25%) of the females and 25 (31%) of the males. Delayed puberty was seen in 20/55 (36.4%) of the females and in 11/31 (35.5%) of the males. Children in early pubertal stage at baseline had the highest risk of growth failure. CONCLUSIONS: JDM and/or its treatment has a significant impact on growth and puberty in the active phase in affected children. Children with recent onset of puberty or previous growth failure, have the highest risk of delayed pubertal development and further growth retardation.

2.
Pediatr Rheumatol Online J ; 16(1): 40, 2018 Jun 25.
Artigo em Inglês | MEDLINE | ID: mdl-29940960

RESUMO

BACKGROUND: Juvenile dermatomyositis (JDM) is the most common inflammatory myopathy in childhood and a major cause of morbidity among children with pediatric rheumatic diseases. The management of JDM is very heterogeneous. The JDM working group of the Society for Pediatric Rheumatology (GKJR) aims to define consensus- and practice-based strategies in order to harmonize diagnosis, treatment and monitoring of JDM. METHODS: The JDM working group was established in 2015 consisting of 23 pediatric rheumatologists, pediatric neurologists and dermatologists with expertise in the management of JDM. Current practice patterns of management in JDM had previously been identified via an online survey among pediatric rheumatologists and neurologists. Using a consensus process consisting of online surveys and a face-to-face consensus conference statements were defined regarding the diagnosis, treatment and monitoring of JDM. During the conference consensus was achieved via nominal group technique. Voting took place using an electronic audience response system, and at least 80% consensus was required for individual statements. RESULTS: Overall 10 individual statements were developed, finally reaching a consensus of 92 to 100% regarding (1) establishing a diagnosis, (2) case definitions for the application of the strategies (moderate and severe JDM), (3) initial diagnostic testing, (4) monitoring and documentation, (5) treatment targets within the context of a treat-to-target strategy, (6) supportive therapies, (7) explicit definition of a treat-to-target strategy, (8) various glucocorticoid regimens, including intermittent intravenous methylprednisolone pulse and high-dose oral glucocorticoid therapies with tapering, (9) initial glucocorticoid-sparing therapy and (10) management of refractory disease. CONCLUSION: Using a consensus process among JDM experts, statements regarding the management of JDM were defined. These statements and the strategies aid in the management of patients with moderate and severe JDM.


Assuntos
Dermatomiosite/tratamento farmacológico , Antirreumáticos/uso terapêutico , Áustria , Criança , Consenso , Dermatomiosite/diagnóstico , Gerenciamento Clínico , Alemanha , Glucocorticoides/uso terapêutico , Humanos , Inquéritos e Questionários
3.
Int J Rheum Dis ; 2018 Apr 17.
Artigo em Inglês | MEDLINE | ID: mdl-29664210

RESUMO

OBJECTIVES: To assess demographical and clinical data in a Middle-European cohort of patients with Adamantiades-Behçet's disease (ABD), together with the use of medication in adherence to international guidelines. METHODS: In a retrospective cohort study, in- and outpatients of an Austrian secondary and tertiary university hospital center were analyzed independent from the medical discipline involved. After ethics approval, screening for ABD-patients in the clinical information system resulted in 1821 documents from 1997 to 2016. Patients fulfilling the International Criteria for Behçet's Disease were included, and ABD symptoms and signs together with medical interventions for immunosuppression, anticoagulation and pain management were identified by individual chart reviews and evaluated for conformity with international recommendations. RESULTS: A total of 76 ABD patients were identified with 39.1% Austrian and 37.0% Turkish origin. Genital aphthae and skin manifestations were more frequent, neurological, gastrointestinal and vascular manifestations less frequent in ABD patients of Turkish origin living in Austria compared to those living in Turkey (each P < 0.05). The male-to-female ratio averaged 0.86 (0.39 in patients with Austrian and 1.43 with Turkish backgrounds), and was 3.3 in patients with venous manifestations. Out of 174 medical interventions, 55.2% fully matched the European League Against Rheumatism recommendations of 2008, and 93.7% were considered at least as equal to the recommendations. Indications for tumor necrosis factor inhibition were in line with the 2007 Sfikakis recommendations. CONCLUSIONS: In this Middle-European ABD cohort clinical presentations between patients of Austrian and Turkish origin do not strongly vary, whereas Turkish patients from the non-endemic Innsbruck cohort present differently compared to patients living in Turkey. The role of such cohort analyses will increase, from the epidemiological as well as the management perspective.

4.
Orphanet J Rare Dis ; 12(1): 167, 2017 10 18.
Artigo em Inglês | MEDLINE | ID: mdl-29047407

RESUMO

BACKGROUND: Hereditary recurrent fevers (HRF) are a group of rare monogenic diseases leading to recurrent inflammatory flares. A large number of variants has been described for the four genes associated with the best known HRF, namely MEFV, NLRP3, MVK, TNFRSF1A. The Infevers database ( http://fmf.igh.cnrs.fr/ISSAID/infevers ) is a large international registry collecting variants reported in these genes. However, no genotype-phenotype associations are provided, but only the clinical phenotype of the first patient(s) described for each mutation. The aim of this study is to develop a registry of genotype-phenotype associations observed in patients with HRF, enrolled and validated in the Eurofever registry. RESULTS: Genotype-phenotype associations observed in all the patients with HRF enrolled in the Eurofever registry were retrospectively analyzed. For autosomal dominant diseases (CAPS and TRAPS), all mutations were individually analyzed. For autosomal recessive diseases (FMF and MKD), homozygous and heterozygous combinations were described. Mean age of onset, disease course (recurrent or chronic), mean duration of fever episodes, clinical manifestations associated with fever episodes, atypical manifestations, complications and response to treatment were also studied. Data observed in 751 patients (346 FMF, 133 CAPS, 114 MKD, 158 TRAPS) included in the Eurofever registry and validated by experts were summarized in Tables. A total of 149 variants were described: 46 TNFRSF1A and 27 NLRP3 variants, as well as various combinations of 48 MVK and 28 MEFV variants were available. CONCLUSIONS: We provide a potentially useful tool for physicians dealing with HRF, namely a registry of genotype-phenotype associations for patients enrolled in the Eurofever registry. This tool is complementary to the Infevers database and will be available at the Eurofever and Infevers websites.


Assuntos
Estudos de Associação Genética , Doenças Hereditárias Autoinflamatórias/epidemiologia , Doenças Hereditárias Autoinflamatórias/genética , Sistema de Registros , Bases de Dados Genéticas , Europa (Continente)/epidemiologia , Humanos , Estudos Retrospectivos
5.
Pediatr Int ; 58(1): 56-8, 2016 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-26541246

RESUMO

We report the case of a 13-year-old girl who presented with fever, headache, nausea and pain behind the right ear. Cerebrospinal fluid (CSF; leukocytes 227/µL), electroencephalogram and cerebral magnetic resonance imaging were indicative of meningoencephalitis. Despite intensive therapy the general condition worsened and the patient was admitted to the intensive care unit. Serological analysis of CSF and serum indicated acute tick-borne encephalitis virus (TBEV) infection (IgG and IgM positive). TBEV infection has been reported after incomplete and complete vaccination. TBEV vaccination breakthrough in childhood has been shown to cause severe disease. It has been suggested that immunized patients develop more severe disease due to altered immune response, but the exact mechanism is unknown. In the presence of typical symptoms and a history of vaccination, possible vaccination breakthrough or missing booster vaccination should be considered.


Assuntos
Anticorpos Antivirais/sangue , Vírus da Encefalite Transmitidos por Carrapatos/imunologia , Encefalite Transmitida por Carrapatos/diagnóstico , Vacinação , Adolescente , Eletroencefalografia , Encefalite Transmitida por Carrapatos/virologia , Ensaio de Imunoadsorção Enzimática , Feminino , Humanos , Imagem por Ressonância Magnética
6.
Muscle Nerve ; 52(3): 437-9, 2015 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-26111941

RESUMO

INTRODUCTION: Lipin 1 gene (LPIN1) mutations lead to cellular energy deficiency and cause up to 50% of the rhabdomyolysis episodes seen in pediatric patients. These episodes are associated with poor prognosis, as treatment options have been limited. We propose a novel therapeutic strategy based on prevention and early treatment of catabolism. METHODS: Five patients were diagnosed with LPIN1 mutations. They were instructed to maintain high caloric intake in situations possibly leading to catabolism such as viral infections or excessive physical activity. When an episode of rhabdomyolysis occurred, patients were treated with intravenous high-concentration glucose at first symptoms. RESULTS: The therapeutic strategies described limited the number of rhabdomyolyis episodes, and the duration of episodes was reduced from 7-10 days, as reported in the literature, to 5 days. CONCLUSION: In this small series, patients with LPIN1 mutations appear to have benefited from prevention and early treatment of catabolism.


Assuntos
Dietoterapia/métodos , Ingestão de Energia , Hidratação/métodos , Glucose/uso terapêutico , Rabdomiólise/prevenção & controle , Edulcorantes/uso terapêutico , Anestesia Geral/efeitos adversos , Áustria , Criança , Pré-Escolar , Feminino , Humanos , Masculino , Atividade Motora , Mutação , Fosfatidato Fosfatase/genética , Rabdomiólise/etiologia , Rabdomiólise/terapia , Resultado do Tratamento , Viroses/complicações
7.
Artigo em Inglês | MEDLINE | ID: mdl-25705138

RESUMO

BACKGROUND: Rheumatic diseases in children are associated with significant morbidity and poor health-related quality of life (HRQOL). There is no health-related quality of life (HRQOL) scale available specifically for children with less common rheumatic diseases. These diseases share several features with systemic lupus erythematosus (SLE) such as their chronic episodic nature, multi-systemic involvement, and the need for immunosuppressive medications. HRQOL scale developed for pediatric SLE will likely be applicable to children with systemic inflammatory diseases. FINDINGS: We adapted Simple Measure of Impact of Lupus Erythematosus in Youngsters (SMILEY©) to Simple Measure of Impact of Illness in Youngsters (SMILY©-Illness) and had it reviewed by pediatric rheumatologists for its appropriateness and cultural suitability. We tested SMILY©-Illness in patients with inflammatory rheumatic diseases and then translated it into 28 languages. Nineteen children (79% female, n=15) and 17 parents participated. The mean age was 12±4 years, with median disease duration of 21 months (1-172 months). We translated SMILY©-Illness into the following 28 languages: Danish, Dutch, French (France), English (UK), German (Germany), German (Austria), German (Switzerland), Hebrew, Italian, Portuguese (Brazil), Slovene, Spanish (USA and Puerto Rico), Spanish (Spain), Spanish (Argentina), Spanish (Mexico), Spanish (Venezuela), Turkish, Afrikaans, Arabic (Saudi Arabia), Arabic (Egypt), Czech, Greek, Hindi, Hungarian, Japanese, Romanian, Serbian and Xhosa. CONCLUSION: SMILY©-Illness is a brief, easy to administer and score HRQOL scale for children with systemic rheumatic diseases. It is suitable for use across different age groups and literacy levels. SMILY©-Illness with its available translations may be used as useful adjuncts to clinical practice and research.


Assuntos
Cooperação Internacional , Linguagem , Qualidade de Vida/psicologia , Projetos de Pesquisa , Doenças Reumáticas/psicologia , Tradução , Adolescente , Antirreumáticos/uso terapêutico , Criança , Pré-Escolar , Estudos de Viabilidade , Feminino , Humanos , Imunossupressores/uso terapêutico , Masculino , Psicometria , Doenças Reumáticas/tratamento farmacológico , Inquéritos e Questionários , Resultado do Tratamento
8.
PLoS One ; 8(7): e67523, 2013.
Artigo em Inglês | MEDLINE | ID: mdl-23874425

RESUMO

The deep ocean is the largest and least known ecosystem on Earth. It hosts numerous pelagic organisms, most of which are able to emit light. Here we present a unique data set consisting of a 2.5-year long record of light emission by deep-sea pelagic organisms, measured from December 2007 to June 2010 at the ANTARES underwater neutrino telescope in the deep NW Mediterranean Sea, jointly with synchronous hydrological records. This is the longest continuous time-series of deep-sea bioluminescence ever recorded. Our record reveals several weeks long, seasonal bioluminescence blooms with light intensity up to two orders of magnitude higher than background values, which correlate to changes in the properties of deep waters. Such changes are triggered by the winter cooling and evaporation experienced by the upper ocean layer in the Gulf of Lion that leads to the formation and subsequent sinking of dense water through a process known as "open-sea convection". It episodically renews the deep water of the study area and conveys fresh organic matter that fuels the deep ecosystems. Luminous bacteria most likely are the main contributors to the observed deep-sea bioluminescence blooms. Our observations demonstrate a consistent and rapid connection between deep open-sea convection and bathypelagic biological activity, as expressed by bioluminescence. In a setting where dense water formation events are likely to decline under global warming scenarios enhancing ocean stratification, in situ observatories become essential as environmental sentinels for the monitoring and understanding of deep-sea ecosystem shifts.


Assuntos
Eutrofização/fisiologia , Fluorescência , Oceanos e Mares , Animais , Contagem de Células , Medições Luminescentes/métodos , Região do Mediterrâneo , Estações do Ano
9.
Ann Rheum Dis ; 72(10): 1628-33, 2013 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-23100606

RESUMO

BACKGROUND: Rare chronic childhood vasculitides lack a reliable disease activity assessment tool. With emerging new treatment modalities such a tool has become increasingly essential for both clinical practice and therapeutic trials to reproducibly quantify change in disease state. OBJECTIVE: To develop and validate a paediatric vasculitis activity assessment tool based on modification of the Birmingham Vasculitis Activity Score (BVASv.3). METHODS: A paediatric vasculitis registry was reviewed to identify clinical features missing in the BVASv.3. A modified nominal group technique was used to develop a working version of the Paediatric Vasculitis Activity Score (PVAS). Prospective validation provided tool reliability, reproducibility and responsiveness to change. Training of assessors was done according to the BVAS principles. RESULTS: BVAS items were redefined (n=22) and eight paediatric items added in Cutaneous (n=4), Cardiovascular (n=3) and Abdominal (n=1) sections. The final PVAS has 64 active items in nine categories. The principles of new/worse and persistently active disease were retained as were the overall score and weighting of categories. The median PVAS in 63 children with systemic vasculitis was 4/63 (0-38/63). There was a high interobserver agreement for the overall as well as for subsystem scores (linear-weighted-κ ≥0.87). PVAS correlated with physician's global assessment (p<0.01); treatment decision (p=<0.01) and erythrocyte sedimentation rate (ESR) (p=0.01). In response to treatment, 15/19 patients assessed demonstrated a significant fall in PVAS (p=0.002), with good agreement among assessors for this change. CONCLUSIONS: The PVAS validity in children with systemic vasculitis was demonstrated. Like the BVAS, we anticipate that the PVAS will provide a robust tool to objectively define disease activity for clinical trials and future research.


Assuntos
Índice de Gravidade de Doença , Vasculite/diagnóstico , Adolescente , Criança , Pré-Escolar , Doença Crônica , Feminino , Glucocorticoides/uso terapêutico , Humanos , Masculino , Variações Dependentes do Observador , Reprodutibilidade dos Testes , Resultado do Tratamento , Vasculite/tratamento farmacológico
10.
Ann Rheum Dis ; 69(5): 798-806, 2010 May.
Artigo em Inglês | MEDLINE | ID: mdl-20413568

RESUMO

OBJECTIVES: To validate the previously proposed classification criteria for Henoch-Schönlein purpura (HSP), childhood polyarteritis nodosa (c-PAN), c-Wegener granulomatosis (c-WG) and c-Takayasu arteritis (c-TA). METHODS: Step 1: retrospective/prospective web-data collection for children with HSP, c-PAN, c-WG and c-TA with age at diagnosis

Assuntos
Granulomatose com Poliangiite/classificação , Poliarterite Nodosa/classificação , Púrpura de Schoenlein-Henoch/classificação , Arterite de Takayasu/classificação , Adolescente , Criança , Métodos Epidemiológicos , Granulomatose com Poliangiite/diagnóstico , Humanos , Cooperação Internacional , Poliarterite Nodosa/diagnóstico , Púrpura de Schoenlein-Henoch/diagnóstico , Arterite de Takayasu/diagnóstico , Terminologia como Assunto
11.
Arthritis Rheum ; 58(7): 2153-62, 2008 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-18576332

RESUMO

OBJECTIVE: Juvenile idiopathic arthritis (JIA) is an autoimmune disease of the young. The pathogenesis is not completely understood. Premature aging, associated thymic involution, and compensatory autoproliferation could play important roles in the pathogenesis of autoimmunity. We undertook this study to determine whether patients with JIA demonstrate premature immunosenescence. METHODS: To test this hypothesis, we measured 3 indicators of aging: the percentages and total counts of peripheral blood naive T cells, the frequency of T cell receptor excision circles (TRECs) in naive T cells, and telomeric erosion and Ki-67 expression as estimates of the replicative history of homeostatic proliferation. RESULTS: JIA patients showed an accelerated loss of CD4+,CD45RA+,CD62L+ naive T cells with advancing age and a compensatory increase in the number of CD4+,CD45RO+ memory T cells. JIA patients demonstrated a significantly decreased frequency of TRECs in CD4+,CD45RA+ naive T cells compared with age-matched healthy donors (P = 0.002). TREC numbers correlated with age only in healthy donors (P = 0.0001). Telomeric erosion in CD4+,CD45RA+ naive T cells was increased in JIA patients (P = 0.01). The percentages of Ki-67-positive CD4+,CD45RA+ naive T cells were increased in JIA patients (P = 0.001) and correlated with disease duration (P = 0.003), which was also an independent factor contributing to telomeric erosion (P = 0.04). CONCLUSION: Our findings suggest that age-inappropriate T cell senescence and disturbed T cell homeostasis may contribute to the development of JIA. In patients with JIA, dysfunction in the ability to reconstitute the T cell compartment should be considered when exploring new therapeutic strategies.


Assuntos
Envelhecimento/imunologia , Artrite Juvenil/imunologia , Antígeno Ki-67/biossíntese , Linfócitos T/imunologia , Estudos de Casos e Controles , Criança , Feminino , Expressão Gênica , Humanos , Contagem de Linfócitos , Masculino
12.
Rheumatol Int ; 28(9): 949-50, 2008 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-18324378

RESUMO

Juvenile idiopathic arthritis (JIA) is an inflammatory joint disease of unknown etiology. The pathogenesis is driven by T and B cells. The role of macrophages remains unclear. Chitotriosidase belongs to the chitinase protein family and is secreted by activated macrophages. The chitinases are able to catalyze the hydrolysis of chitin or chitin-like substrates such as 4-methylumbelliferyl chitotrioside. Chitotriosidase activity was determined using the substrate 4-methylumbelliferyl beta-DNN'N''-triacetylchitotrioside (4-MU-TCT, SIGMA Chemical Co.). The substrate and serum were incubated with the serum in a citrate/phosphate buffer. The reaction was stopped by adding a buffer (Na2CO3). The fluorescence of 4-methylumbelliferone was evaluated by fluorimeter at excitation 360 nm and emission 450 nm. We report about chitotriosidase measurements in patients with JIA. The chitotriosidase level in synovial fluid was up to approximately 1,000 nmol/(h ml) at disease onset before therapy. The level in the sera was below 600 nmol/(h ml). Serum chitotriosidase levels could represent the activity of macrophages in the synovial fluid in JIA.


Assuntos
Artrite Juvenil/enzimologia , Hexosaminidases/sangue , Líquido Sinovial/enzimologia , Artrite Juvenil/sangue , Estudos de Casos e Controles , Humanos
13.
Arthritis Rheum ; 59(1): 4-13, 2008 Jan 15.
Artigo em Inglês | MEDLINE | ID: mdl-18163404

RESUMO

OBJECTIVE: To validate a core set of outcome measures for the evaluation of response to treatment in patients with juvenile dermatomyositis (DM). METHODS: In 2001, a preliminary consensus-derived core set for evaluating response to therapy in juvenile DM was established. In the present study, the core set was validated through an evidence-based, large-scale data collection that led to the enrollment of 294 patients from 36 countries. Consecutive patients with active disease were assessed at baseline and after 6 months. The validation procedures included assessment of feasibility, responsiveness, discriminant and construct ability, concordance in the evaluation of response to therapy between physicians and parents, redundancy, internal consistency, and ability to predict a therapeutic response. RESULTS: The following clinical measures were found to be feasible, and to have good construct validity, discriminative ability, and internal consistency; furthermore, they were not redundant, proved responsive to clinically important changes in disease activity, and were associated strongly with treatment outcome and thus were included in the final core set: 1) physician's global assessment of disease activity, 2) muscle strength, 3) global disease activity measure, 4) parent's global assessment of patient's well-being, 5) functional ability, and 6) health-related quality of life. CONCLUSION: The members of the Paediatric Rheumatology International Trials Organisation, with the endorsement of the American College of Rheumatology and the European League Against Rheumatism, propose a core set of criteria for the evaluation of response to therapy that is scientifically and clinically relevant and statistically validated. The core set will help standardize the conduct and reporting of clinical trials and assist practitioners in deciding whether a child with juvenile DM has responded adequately to therapy.


Assuntos
Dermatomiosite/diagnóstico , Guias de Prática Clínica como Assunto , Criança , Feminino , Humanos , Masculino , Estudos Prospectivos
14.
Isr J Psychiatry Relat Sci ; 45(4): 257-62, 2008.
Artigo em Inglês | MEDLINE | ID: mdl-19439831

RESUMO

BACKGROUND: Johannes Heinrich Schultz (1884-1970) established the set of techniques known as "autogenic training." From 1936 until 1945 he worked as assistant director of the Göring Institute. His role during National Socialism has been underestimated in our opinion. METHOD: We considered Schultz's academic publications and his "autobiography" from 1964. RESULTS: Schultz publicly advocated compulsory sterilization as well as the "annihilation of life unworthy of life" and developed a diagnostic scheme which distinguished between the neurotic/curable and the hereditary/ incurable. In fact, this classification was then employed to decide between life and death. In order to justify the "New German Psychotherapy" alongside eugenic psychiatry, Schultz carried out degrading and inhuman "treatments" of homosexual prisoners of concentration camps who were in mortal danger. LIMITATIONS: This study was based on written documents. We were not able to interview contemporary witnesses. CONCLUSION: By advocating compulsory sterilization and the "annihilation of life unworthy of life" and by the abuse of homosexuals as research objects Schultz violated fundamental ethical principles of psychiatry.


Assuntos
Treinamento Autógeno/história , Campos de Concentração/história , Eugenia (Ciência)/história , Holocausto/história , Homossexualidade Masculina/história , Experimentação Humana/história , Judeus/história , Socialismo Nacional/história , Psicoterapia/história , Esterilização Involuntária/história , Alemanha , História do Século XIX , História do Século XX , Humanos , Masculino
15.
Rheumatol Int ; 27(12): 1171-2, 2007 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-17534622

RESUMO

Sarcoidosis is a chronic granulomatous inflammation. The clinical spectrum in childhood is heterogeneous. Angiotensin converting enzyme (ACE) activity is used as a marker for disease activity. Human chitotriosidase is produced in macrophages. In this study serum chitotriosidase levels were significantly higher in active sarcoidosis, than in inactive disease or healthy controls. Serum chitotriosidase concentrations may be a useful marker for monitoring disease activity in sarcoidosis.


Assuntos
Biomarcadores/sangue , Hexosaminidases/sangue , Sarcoidose/diagnóstico , Adolescente , Feminino , Humanos , Peptidil Dipeptidase A/sangue , Sarcoidose/sangue , Sarcoidose/enzimologia
16.
Rheumatol Int ; 27(12): 1185-6, 2007 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-17252260

RESUMO

Sarcoidosis is a chronic granulomatous inflammation. The clinical spectrum in childhood is heterogeneous. Angiotensin converting enzyme activity is used as a marker for disease activity. Human chitotriosidase is produced in macrophages. In this study, serum chitotriosidase levels were significantly higher in active sarcoidosis than in inactive disease or healthy controls. Serum chitotriosidase concentrations may be a useful marker for monitoring disease activity in sarcoidosis.


Assuntos
Hexosaminidases/sangue , Sarcoidose/diagnóstico , Sarcoidose/enzimologia , Adolescente , Biomarcadores/sangue , Criança , Feminino , Hexosaminidases/análise , Humanos , Masculino , Peptidil Dipeptidase A/sangue , Sarcoidose/sangue
17.
Mod Rheumatol ; 16(6): 372-5, 2006.
Artigo em Inglês | MEDLINE | ID: mdl-17164999

RESUMO

Anticyclic citrullinated peptide (anti-CCP) antibodies have been detected in patients with juvenile idiopathic arthritis (JRA), particularly in those with polyarticular JIA. We analyzed the presence of anti-CCP antibodies of the IgG class in sera of patients with defined juvenile idiopathic arthritis (JIA) of various subgroups. One hundred and fifty-nine serum samples were investigated. Forty-five patients were diagnosed with JIA (15 male and 30 female) aged 1.9-17.3 years (median 12.9, mean 11.0). Thirty-eight samples were taken from patients suffering from other autoimmunopathies and 34 patients with other underlying diseases were taken at different time points in their disease course. Under 42 samples were taken from patients with noninflammatory diseases. Enzyme-linked immunosorbent assay (ELISA) was used for the detection of anti-CCP antibodies. Anti-CCP antibodies were found in 6.9% of all samples and in 4.4% patients with JIA. Disease duration and medication did not differ significantly between anti-CCP positive and negative patients. A review of the literature and our own results shows that anti-CCP antibodies can be detected in the sera of only some patients with JIA. Routine determination of anti-CCP cannot be recommended.


Assuntos
Artrite Juvenil/imunologia , Autoanticorpos/sangue , Peptídeos Cíclicos/imunologia , Adolescente , Artrite Juvenil/fisiopatologia , Biomarcadores/sangue , Criança , Pré-Escolar , Estudos de Coortes , Ensaio de Imunoadsorção Enzimática , Feminino , Humanos , Imunoglobulina G/sangue , Lactente , Masculino
18.
Eur J Pediatr ; 165(6): 398-401, 2006 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-16547728

RESUMO

A 12-year-old Turkish girl suffered from abdominal pain located in the right lower abdomen for 3 weeks. Ultrasound revealed palisade-like swelling of the mucosa in the ileum. Gastrointestinal biopsy showed incipient granulomas in the stomach and moderate fibrosis of the terminal ileum. Subsequently, bilateral hilar adenopathy and an abnormal level of serum angiotensin-converting enzyme were detected. The relevance of paediatric sarcoidosis mimicking Crohn's disease is discussed.


Assuntos
Doença de Crohn/diagnóstico , Sarcoidose/diagnóstico , Criança , Doença Crônica , Doença de Crohn/patologia , Diagnóstico Diferencial , Feminino , Gastrite/etiologia , Granuloma/etiologia , Humanos , Linfonodos/patologia , Peptidil Dipeptidase A/sangue , Sarcoidose/complicações , Sarcoidose/patologia , Tomografia Computadorizada por Raios X
19.
J Affect Disord ; 91(1): 57-62, 2006 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-16412522

RESUMO

BACKGROUND: Abnormalities in the serotonergic (5-HT) system have been implicated in the pathogenesis of suicidal behavior. Studies on peripheral serotonergic parameters as a measure for central serotonergic function in suicidal patients appear to be promising, yet failed to show a clear association with suicidality. The objective of this study was to elucidate the role of serotonergic blood parameters in depressed suicidal patients and to examine their usefulness as a potential biological marker for suicidality. A number of personality traits were assessed in order to provide a basis for a psychobiological model of suicidal behavior. METHODS: Depressed patients with a recent suicide attempt (SA; n = 59) were compared to those without history of suicide attempts (NSA; n = 28). 5-HT2A receptor binding in platelets and tryptophan/amino acid ratio in plasma were measured. Acute psychopathology and personality traits as well as characteristics of suicide attempts were assessed. RESULTS: There was no significant difference between SA and NSA in terms of peripheral serotonergic parameters as well as personality traits. However, the whole sample showed associations between certain personality traits and serotonergic platelet parameters. Furthermore, we observed a relation between suicidal ideation, lethality of suicide attempts and peripheral serotonergic markers. LIMITATIONS: The number of cases with data on peripheral markers is relatively low. The potential influence of antidepressant medication previous to study inclusion has to be taken into account. The study focussed on depressed patients only. CONCLUSIONS: Low serotonergic function is involved in the pathogenesis of suicidality, whereas the use of platelet 5-HT2A receptor activity and tryptophan availability as biological markers for suicidality in depressed patients could not be proven an appropriate tool. Alterations in the serotonergic system are associated with trait aggression and other character dimensions.


Assuntos
Plaquetas/metabolismo , Transtorno Depressivo/sangue , Determinação da Personalidade/estatística & dados numéricos , Receptor 5-HT2A de Serotonina/sangue , Tentativa de Suicídio/psicologia , Triptofano/sangue , Adulto , Aminoácidos/sangue , Biomarcadores/sangue , Transtorno Depressivo/psicologia , Feminino , Alemanha , Humanos , Masculino , Pessoa de Meia-Idade , Psicometria , Ensaio Radioligante , Risco , Estatística como Assunto
20.
Arch Suicide Res ; 10(1): 1-9, 2006.
Artigo em Inglês | MEDLINE | ID: mdl-16287691

RESUMO

Low cholesterol concentrations and cholesterol-lowering therapies have been suggested to be associated with increased suicidality. This article examined the association of cholesterol, triglycerides, and body-mass index (BMI) with suicidal ideation and suicide attempts. Findings are based on a nationally representative community sample of n = 4,181 subjects (18-65 years) examined with a standardized diagnostic interview (CIDI) for (DSM-IV) mental disorders. Controlling for age and gender the study revealed a moderate positive association between cholesterol, triglycerides, BMI, and suicide attempts in subjects with depressive symptoms during the past 12 months (n = 1,205). The results of this study are compatible with two recent epidemiological cohort studies showing a positive association between cholesterol and completed suicide.


Assuntos
Índice de Massa Corporal , Colesterol/sangue , Transtorno Depressivo/epidemiologia , Tentativa de Suicídio/estatística & dados numéricos , Triglicerídeos/sangue , Adolescente , Adulto , Idoso , Transtorno Depressivo/sangue , Alemanha/epidemiologia , Humanos , Modelos Logísticos , Pessoa de Meia-Idade , Fatores de Risco
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