RESUMEN
OBJECTIVE: To compare unenhanced versus enhanced knee joint magnetic resonance imaging (MRI) to assess disease activity of juvenile idiopathic arthritis (JIA). METHODS: Fifty-three knee joint MRI examinations were performed on a 3-Tesla system in 27 patients (age: 11.40 ± 3.61 years; 21 females, 6 males). MRI protocols comprised PD-weighted sequences in addition to the widely used standard protocol. JIA subgroups comprised oligoarticular arthritis (n = 16), extended oligoarthritis (n = 6), rheumatoid factor-negative polyarticular arthritis (n = 3), enthesitis-related arthritis (n = 1), and psoriatic arthritis (n = 1). MR images were retrospectively analyzed by 3 experienced radiologists in two readings, using JAMRIS (juvenile arthritis MRI scoring) system and a modified IPSG (international prophylaxis study group) classification. In the first reading session, only unenhanced MR images were evaluated. In a second reading session, all images before and after contrast medium application were included. In order to avoid bias, an interval of at least 2 weeks was set between the two readings. The clinical JADAS10 (juvenile arthritis disease activity score) was calculated including clinical assessment and laboratory workup and correlated with MRI scores. Statistical analysis comprised Pearson's correlation for correlating two scoring results of unenhanced and the enhanced MRI, intra-class correlation coefficient (ICC) for inter- and intra-reader agreement. Diagnostic accuracy was calculated using ROC (receiver operating characteristics) curve analysis. RESULTS: Inter-reader agreement determined by ICC for unenhanced and enhanced MRI scores for IPSG was moderate (0.65, 95% CI 0.51-0.76, and 0.62, 95% CI 0.48-0.75) and high for JAMRIS (0.83, 95% CI 0.75-0.89, and 0.82, 95% CI 0.74-0.89). Intra-reader agreement was good to very good for JAMRIS (0.85 95% CI 0.81-0.88, 0.87 95% CI 0.83-0.89 and 0.96 95% CI 0.92-0.98) and IPSG (0.76 95% CI 0.62-0.86, 0.86 95% CI 0.77-0.92 and 0.92 95% CI 0.86-0.96). Scores of unenhanced MRI correlated with contrast-enhanced MRI: JAMRIS (r = 0.97, R2 = 0.93, p < 0.01), modified IPSG (r = 0.95, R2 = 0.91, p < 0.01). When using JADAS10 as a reference standard, moderate accuracy for both unenhanced and enhanced MRI scores was noted: JAMRIS (AUC = 0.68, 95% CI 0.51-0.85, and AUC = 0.66, 95% 0.49-0.82), IPSG score (AUC = 0.68, 95% 0.50-0.86, and AUC = 0.61, 95% 0.41-0.81). CONCLUSIONS: Our results suggest that contrast agent application could be omitted in JIA patients with an augmented knee MRI protocol comprising PD-weighted sequence. KEY POINTS: ⢠Unenhanced MRI can detect disease activity of the knee joint in patients with JIA with equally high accuracy compared to contrast-enhanced MRI. ⢠The intra- and inter-reader agreement was high for unenhanced and enhanced MRI JAMRIS scores, which indicate relatively good applicability of the scoring system, even for less experienced readers. ⢠When using the clinical JADAS10 as a reference standard for the detection of disease activity, moderate accuracy for both unenhanced and enhanced MRI scores, both JAMRIS and IPSG, was noted, which might be caused by the fact that the majority of patients had either no or minimal clinical disease activity.
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Artritis Juvenil , Masculino , Femenino , Humanos , Niño , Adolescente , Artritis Juvenil/diagnóstico por imagen , Estudios Retrospectivos , Imagen por Resonancia Magnética/métodos , Articulación de la Rodilla/diagnóstico por imagen , Curva ROC , Medios de Contraste/farmacologíaRESUMEN
BACKGROUND: Oligoarticular juvenile idiopathic arthritis (oligoJIA) is the most commonly diagnosed category of chronic arthritis in children. Nevertheless, there are no evidence- based guidelines for its treatment, in particular for the use of methotrexate (MTX). The primary objective of this analysis is to evaluate the outcomes in patients with persistent oligoJIA compared to those with extended oligoJIA and rheumatoid factor (RF) negative polyarthritis treated with methotrexate. METHODS: Patients with persistent or extended oligoJIA or RF negative PA recorded in the Biologics in Pediatric Rheumatology Registry (BiKeR), receiving methotrexate for the first time were included in the analyses. Efficacy was determined using the Juvenile Arthritis Disease Activity Score 10 (JADAS 10). Safety assessment included the documentation of adverse and serious adverse events. RESULTS: From 2005 through 2011, 1056 patients were included: 370 patients with persistent oligoJIA, 221 patients with extended oligoJIA and 467 patients with RF negative PA. Therapeutic efficacy was observed following the start of methotrexate. Over a period of 24 months JADAS-minimal disease activity (JADAS ≤2) was reached in 44% of patients with persistent oligoJIA, 38% with extended oligoJIA, 46% with RF negative PA, JADAS-remission defined as JADAS ≤1 was reached in 33% of patients with persistent oligoJIA, 29% with extended oligoJIA and 35% (RF negative PA). Patients with extended oligoJIA achieved JADAS remission significantly later and received additional biologic disease-modifying drugs significantly more often than patients with persistent oligoJIA or RF negative PA (p < 0.001). Tolerability was comparable. New onset uveitis occurred in 0.3 to 2.2 per 100 patient years. CONCLUSIONS: Patients with persistent oligoJIA taking methotrexate are at least as likely to enter remission as patients with extended oligo JIA or polyarticular JIA. Patients with extended oligoJIA achieved JADAS remission significantly later. Within 2 years, almost half of the patients with persistent oligoJIA achieved JADAS-minimal disease activity.
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Antirreumáticos/uso terapéutico , Artritis Juvenil/tratamiento farmacológico , Artritis/tratamiento farmacológico , Metotrexato/uso terapéutico , Niño , Preescolar , Femenino , Alemania , Humanos , Masculino , Sistema de Registros , Resultado del TratamientoRESUMEN
In the healthy heart, physiological heterogeneities in structure and in electrical and mechanical activity are crucial for normal, efficient excitation and pumping. Alterations of heterogeneity have been linked to arrhythmogenesis in various cardiac disorders such as long QT syndrome (LQTS). This inherited arrhythmia disorder is caused by mutations in different ion channel genes and is characterized by (heterogeneously) prolonged cardiac repolarization and increased risk for ventricular tachycardia, syncope and sudden cardiac death. Cardiac electrical and mechanical function are not independent of each other but interact in a bidirectional manner by electromechanical and mechano-electrical coupling. Therefore, changes in either process will affect the other. Recent experimental and clinical evidence suggests that LQTS, which is primarily considered an "electrical" disorder, also exhibits features of disturbed mechanical function and heterogeneity, which in turn appears to correlate with the risk of arrhythmia in the individual patient. In this review, we give a short overview of the current knowledge about physiological and pathological, long QT-related electrical and mechanical heterogeneity in the heart. Also, their respective roles for future risk prediction approaches in LQTS are discussed.
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Electrocardiografía , Síndrome de QT Prolongado/fisiopatología , Fenómenos Biomecánicos/genética , Fenómenos Biomecánicos/fisiología , Análisis Mutacional de ADN , Muerte Súbita Cardíaca/etiología , Fenómenos Electrofisiológicos/genética , Fenómenos Electrofisiológicos/fisiología , Humanos , Canales Iónicos/genética , Canales Iónicos/fisiología , Síndrome de QT Prolongado/genética , Contracción Miocárdica/genética , Contracción Miocárdica/fisiología , Medición de Riesgo , Síncope/genética , Síncope/fisiopatología , Taquicardia Ventricular/genética , Taquicardia Ventricular/fisiopatologíaRESUMEN
These guidelines summarize the current evidence for diagnosis and treatment of Lyme arthritis and the most frequent skin manifestations of Borrelia burgdorferi infections. Lyme arthritis is a monoarticular or oligoarticular form of arthritis that typically involves the knee. A positive enzyme-linked immunosorbent assay (ELISA) for IgG antibodies should be followed by an IgG immunoblot. A positive PCR test from synovial fluid adds increased diagnostic certainty. Serum positivity for antibodies to Borrelia burgdorferi without typical symptoms does not justify antibiotic treatment. Oral antibiotic treatment for erythema migrans is recommended using doxycycline, 200 mg once per day for 10-21 days, alternative choices are amoxicillin, cefuroxime and azithromycin. For children below 8 years of age, amoxicillin is recommended.Lyme arthritis can usually be successfully treated with orally administered antimicrobial agents. Doxycycline, 1 × 200 or 2 × 100 mg for 30 days is the antibiotic agent of choice. Amoxicillin (3 × 500-1000 mg) can be alternatively chosen. Patients who have persistent or recurrent joint swelling after a recommended course of oral antibiotic therapy should be treated intravenously. In this situation, ceftriaxone at 2 g per day for 14-21 days is recommended. There is no evidence to recommend long-term and combined treatments.
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Antibacterianos/administración & dosificación , Artritis Infecciosa/diagnóstico , Artritis Infecciosa/tratamiento farmacológico , Enfermedad de Lyme/diagnóstico , Enfermedad de Lyme/tratamiento farmacológico , Guías de Práctica Clínica como Asunto , Reumatología/normas , Artritis Infecciosa/sangre , Alemania , Humanos , Enfermedad de Lyme/sangreRESUMEN
Familial Mediterranean fever (FMF) is the most inherited common autoinflammatory disease (AID) with mutations in the MEFV (MEditerraneanFeVer) gene.The Mor- and Pras-Score modified for children and C-reactive protein (CRP) were used to assess FMF disease severity in Germany. We evaluate the applicability of the 2 severity scores and the correlations between ethnic origin, phenotype, and genotype.Among 242 children (median 5 age at diagnosis), we detected 431 pyrin mutations and 22 different sequence variants, including one new mutation (p.Gly488Asp). The 5 most -frequent alterations were p.Met694Val (55.2%), p.Met680lle (11.8%), p.Val726Ala (10%), p.Glu148Gln (7.9%) and p.Met694IIe (2.3%). The prevailing ancestries of 223 cases were Turkish (82.5%) and Lebanese (8.1%). Homozygous p.Met694Val substitution (30.2%) was associated with a more severe disease activity by Mor-Score, as well as with a higher mean CRP (74 mg/l) compared to patients with other mutations. Indeed, Mor- and Pras-Score were inconsistent with each other. A typical distribution of mutations in different ethnic populations was obvious, but not statistically verifiable due to the low number of cases.The homozygous p.Met694Val substitution was associated with a more severe disease activity in our German cohort. The common severity scores were inconsistent in -children.
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Fiebre Mediterránea Familiar/diagnóstico , Fiebre Mediterránea Familiar/genética , Genotipo , Fenotipo , Adolescente , Alelos , Sustitución de Aminoácidos/genética , Proteína C-Reactiva/metabolismo , Niño , Preescolar , Estudios de Cohortes , Proteínas del Citoesqueleto/genética , Análisis Mutacional de ADN , Fiebre Mediterránea Familiar/etnología , Femenino , Frecuencia de los Genes/genética , Alemania , Homocigoto , Humanos , Lactante , Líbano/etnología , Masculino , Metionina/genética , Pirina , Sistema de Registros , Turquía/etnología , Valina/genéticaRESUMEN
In a nation-wide registration project 38 incident cases of juvenile dermatomyositis were collected in Germany over a 2-year-period. Diagnostic methods as well as the primary treatment for these patients were recorded. Detailed information was available for 25 of these patients. Diagnostic as well as therapeutic decisions varied widely. Steroids were used in almost all of the 25 patients either as oral or as parenteral pulse therapy, additional immunosuppressive drugs were used in 52%. We plan to establish national consensus recommendations for diagnostic and therapeutic standards in JDM. Due to the rarity of JDM clinical trials will have to be performed on an international basis.
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Dermatomiositis/diagnóstico , Dermatomiositis/epidemiología , Sistema de Registros , Administración Oral , Adolescente , Corticoesteroides/administración & dosificación , Niño , Preescolar , Consenso , Estudios Transversales , Dermatomiositis/tratamiento farmacológico , Quimioterapia Combinada , Femenino , Alemania , Humanos , Inmunosupresores/administración & dosificación , Incidencia , Infusiones Intravenosas , Masculino , Guías de Práctica Clínica como Asunto , Quimioterapia por PulsoRESUMEN
Lyme arthritis is one of the manifestations of Lyme disease and is caused by infection with Borrelia burgdorferi sensu lato. This article reviews the current knowledge regarding the epidemiology, etiology and pathogenesis as well as the clinical manifestations, the diagnosis, treatment and prognosis with special emphasis on children and adolescents.
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Artritis Juvenil/diagnóstico , Artritis Juvenil/terapia , Enfermedad de Lyme/diagnóstico , Enfermedad de Lyme/terapia , Adolescente , Artritis Juvenil/epidemiología , Niño , Preescolar , Humanos , Lactante , Recién Nacido , Enfermedad de Lyme/complicaciones , Enfermedad de Lyme/epidemiologíaRESUMEN
In all subgroups of juvenile idiopathic arthritis (JIA), a pathologic loss of bone or the lack of increase in bone mass has been described in a high percentage of cases, even with new therapeutic approaches. The decrease in bone mass is correlated with the duration of active disease and the number of affected joints (cytokines, inactivity). In several studies, muscle mass was the strongest predictor of bone mass. A standardized diagnostic approach to the musculoskeletal system including measures of prophylaxis and therapy therefore seems to be mandatory for all children with JIA who do not achieve rapid remission. In this review, the diagnostic and therapeutic options are described and summarized in an algorithm.
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Artritis Juvenil/diagnóstico , Osteoporosis/diagnóstico , Absorciometría de Fotón , Adolescente , Artritis Juvenil/tratamiento farmacológico , Artritis Juvenil/terapia , Calcio/administración & dosificación , Niño , Difosfonatos/administración & dosificación , Terapia por Ejercicio , Glucocorticoides/efectos adversos , Glucocorticoides/uso terapéutico , Humanos , Inmunosupresores/efectos adversos , Inmunosupresores/uso terapéutico , Metotrexato/efectos adversos , Metotrexato/uso terapéutico , Fuerza Muscular/fisiología , Atrofia Muscular/inducido químicamente , Atrofia Muscular/tratamiento farmacológico , Atrofia Muscular/terapia , Osteoporosis/inducido químicamente , Osteoporosis/terapia , Factores de Riesgo , Factor de Necrosis Tumoral alfa/antagonistas & inhibidores , Vitamina D/administración & dosificaciónRESUMEN
Juvenile dermatomyositis (JDM) is an inflammatory multi-system disease of unknown etiology with classic involvement of the skin and striated muscles. Following a prodromal period, patients develop a progressive proximal muscle weakness. Typical skin involvement includes heliotrope rash, facial erythema, Gottron's sign and nailfold capillary abnormalities. For the diagnosis of JDM, modified Bohan and Peter criteria are used including clinical skin and muscle signs plus elevated muscle enzymes and typical findings from electromyography, muscle biopsy and - more recently - also on magnetic resonance imaging. Steroids are administered classically as high-dose oral treatment. Intravenous pulse therapy with intermittent lower dose oral treatment and other immunosuppressive drugs such as methotrexate may reduce steroid side-effects. Prognosis in JDM has improved, and most patients eventually make a full functional recovery. However, a few patients still die from their disease, and in a minority significant sequelae with muscle atrophy or severe calcinosis ensue.
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Dermatomiositis/diagnóstico , Adolescente , Biopsia , Niño , Preescolar , Terapia Combinada , Dermatomiositis/mortalidad , Dermatomiositis/patología , Diagnóstico Diferencial , Quimioterapia Combinada , Humanos , Inmunosupresores/administración & dosificación , Imagen por Resonancia Magnética , Metilprednisolona/administración & dosificación , Músculo Esquelético/patología , Prednisolona/administración & dosificación , Prednisona/administración & dosificación , Pronóstico , Tasa de SupervivenciaRESUMEN
OBJECTIVES: Inflammation plays a role in prematurity, in neonatal disorders of the brain, lung, eye, bowel, and in developmental disability among preterm infants. We initiated a pilot study in preterm children to determine the prevalence of single nucleotide polymorphisms (SNPs) in the infection/inflammation-associated genes for interleukin (IL)-10 (- 1082 G/A), IL-1beta (+ 3953 C/T), tumor necrosis factor (TNF)-alpha (- 308 G/A) and toll-like receptor 4 (TLR-4) (Asp299Gly) and whether these SNPs affect the risk for neonatal disorders. STUDY DESIGN: We genotyped 73 children >/= 2 years of age whose gestational age at birth was < 32 weeks, and explored the associations between genotypes and neonatal disorders and developmental status at age 2 + years. RESULTS: Infants homozygous for the high IL-10 producer - 1082 G-allele (n = 15) were significantly less likely to develop ultrasound-defined periventricular echodensities. A non-significant, but prominent, risk reduction for bronchopulmonary dysplasia, high-grade retinopathy, cerebral palsy, and developmental delay at age 2 + years was present. Polymorphisms in the IL-1beta, TNF-alpha, and TLR-4 genes were too infrequent in our pilot sample to allow for reasonable analysis. CONCLUSION: Infants homozygous for the IL-10 high producer - 1082 G allele might be at reduced risk for prematurity-associated disorders.
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Encéfalo/anomalías , Interleucina-10/metabolismo , Nacimiento Prematuro , Encéfalo/patología , Ventrículos Cerebrales/anomalías , Ventrículos Cerebrales/patología , Preescolar , Femenino , Genotipo , Humanos , Interleucina-1/genética , Interleucina-1/metabolismo , Interleucina-10/genética , Masculino , Proyectos Piloto , Polimorfismo de Nucleótido Simple/genética , Embarazo , Nacimiento Prematuro/diagnóstico por imagen , Nacimiento Prematuro/metabolismo , Nacimiento Prematuro/patología , Estudios Retrospectivos , Receptores Toll-Like/genética , Receptores Toll-Like/metabolismo , Factor de Necrosis Tumoral alfa/genética , Factor de Necrosis Tumoral alfa/metabolismo , Ultrasonografía/métodosRESUMEN
AIMS: To explore the information needs of adolescents with juvenile chronic arthritis (JCA) with respect to patient education and other measures to promote self-management. METHODS: Standardized cross-sectional inquiry concerning disease-related knowledge, perceived importance of information giving, unmet needs as well as perceived attractiveness of a range of services (lecture, structured patient education, support group, self-help group) to promote self-management. SAMPLE: N = 48 adolescents (68% of all adolescents with JCA of our outpatient clinic); mean age x = 14.9 (+/- 2.1) years; 56% female; 17% had the oligoarthritis form of JCA, 40% juvenile spondylarthritis, 25% polyarthritis and systemic form, 19% other rheumatic diseases. RESULTS: The majority of adolescents considered themselves as sufficiently well-informed and voted in favour of detailed information giving. However, 30% were unsatisfied with their current information and knowledge. Information needs predominantly related to the prognosis, course, and treatment of JCA, whereas the psychosocial impact (except sports and job matters) were judged as less important. Adolescents with a lower level of education were generally more interested than those with a high level of education. As for the attractiveness of services nearly half of the adolescents judged all of them as not very attractive. CONCLUSIONS: The majority of adolescents is interested in detailed information giving and some of them point to unmet needs, but nearly half of them is hesitant towards services which are delivered in a group format (such as structured patient education or support groups).
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Artritis Juvenil/psicología , Artritis Juvenil/terapia , Adolescente , Niño , Estudios Transversales , Femenino , Alemania , Conocimientos, Actitudes y Práctica en Salud , Humanos , Masculino , Relaciones Padres-Hijo , Educación del Paciente como Asunto , Grupos de Autoayuda , Encuestas y CuestionariosRESUMEN
We report the results of the cross-cultural adaptation and validation into the German language of the parent's version of two health related quality of life instruments. The Childhood Health Assessment Questionnaire (CHAQ) is a disease specific health instrument that measures functional ability in daily living activities in children with juvenile idiopathic arthritis (JIA). The Child Health Questionnaire (CHQ) is a generic health instrument designed to capture the physical and psychosocial well-being of children independently from the underlying disease. The German CHAQ was fully validated with 3 forward and 3 backward translations, while the CHQ has already been published and therefore it was revalidated. A total of 197 subjects were enrolled: 142 patients with JIA (5% systemic onset, 13% polyarticular onset, 8% extended oligoarticular subtype, and 74% persistent oligoarticular subtype) and 55 healthy children. The CHAQ clinically discriminated between healthy subjects and JIA patients, with the polyarticular and extended oligoarticular subtypes having a higher degree of disability, pain, and a lower overall well-being when compared to their healthy peers. Also the CHQ clinically discriminated between healthy subjects and JIA patients, with the polyarticular onset and extended oligoarticular subtypes having a lower physical and psychosocial well-being when compared to their healthy peers. In conclusion the German versions of the CHAQ-CHQ are reliable, and valid tools for the functional, physical and psychosocial assessment of children with JIA.
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Artritis Juvenil/diagnóstico , Comparación Transcultural , Estado de Salud , Encuestas y Cuestionarios , Adolescente , Niño , Características Culturales , Evaluación de la Discapacidad , Femenino , Alemania , Humanos , Lenguaje , Masculino , Psicometría , Calidad de Vida , Reproducibilidad de los ResultadosRESUMEN
OBJECTIVES: Cystatin C and beta(2)-microglobulin are established serum markers of renal function in children and adults. In contrast to creatinine, diaplacental exchange is minimal. The aim of the study was to establish reference values in fetal serum and to test their efficiency in predicting postnatal kidney function. STUDY DESIGN: This was a prospective noninterventional study measuring cystatin C and beta(2)-microglobulin by particle-enhanced immunoturbidimetry in excess serum from 129 cordocenteses performed in 84 fetuses. Reference intervals (mean +/- 1.96 SD) were calculated in a subgroup of 54 fetuses without evidence of kidney disease, and these reference values were evaluated in 75 sera from 55 fetuses. RESULTS: Mean cystatin C was 1.66 +/- 0.202 mg/L (upper limit 2.06), and mean beta(2)-microglobulin was 4.25 +/- 0.734 mg/L. Unlike cystatin C, beta(2)-microglobulin decreased significantly with gestational age so that the upper reference limit was 7.19-0.052 x gestational age in weeks. beta(2)-Microglobulin had higher sensitivity (90.0% vs 63.6%) and cystatin C a higher specificity (91.8% vs. 85.5%) for the prediction of impaired renal function; diagnostic efficiency was equal (87.6% vs. 86.1%). Fetuses with impaired renal function at birth or who were aborted for renal malformations had higher cystatin C concentrations than those in a control group. beta(2)-Microglobulin was increased only in fetuses who were aborted. CONCLUSION: Fetal serum cystatin C and beta(2)-microglobulin concentrations may be useful predictors of postnatal kidney function.
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Cistatinas/sangre , Sangre Fetal/química , Enfermedades Renales/diagnóstico , Diagnóstico Prenatal , Microglobulina beta-2/sangre , Cordocentesis , Creatinina/sangre , Cistatina C , Femenino , Edad Gestacional , Humanos , Enfermedades Renales/sangre , Embarazo , Valores de ReferenciaRESUMEN
OBJECTIVE: The purpose of this study was to evaluate the effects of opioid addiction on pregnancy, perinatal period, and the long-term development of mother and child. MATERIAL AND METHODS: Records of 44 opioid-dependent women and their children were analyzed. Thirty-three patients were enrolled in methadone maintenance treatment (MMT) programs or received codeine. RESULTS: Problems during pregnancy were premature rupture of membranes (n = 8), premature labor (n = 21), premature delivery (n = 9), abruption placentae (n = 2), cesarean section (n = 10), and fetal growth retardation (n = 15). Whereas MMT improved fetal growth, no influence was seen on other problems during pregnancy. Thirty newborns had significant withdrawal symptoms. One child became HIV-positive and two children required treatment for suspected congenital syphilis. One child died of sudden infant death syndrome. CONCLUSIONS: The major goal has to be the avoidance of an unwanted pregnancy. Does it still happen, the patient needs special support. If an opioid-dependent woman presents herself the first time, an anamnesis concerning drugs in addition to a general anamnesis should be arisen.
RESUMEN
Following the recommendations of the United States National Institutes of Health Consensus Conference in 1993, otoacoustic emissions (OAE) are now used internationally for hearing screening. The use of recording systems as well as the interpretation of results requires specially trained personnel although measurements are easier to perform than other recording methods available. To date, no objective method for general hearing screening, has been introduced in Germany, for neonates or children at risk, even though it would be desirable to detect and rehabilitate children with congenital hearing loss as early as possible (incidence, 1-6 per 1000). The Echosensor provides the means for carrying out OAE recordings within a short period of time and includes an automatic evaluation of results. Recordings can also be made by trained personnel. As a comparison test, audiologically trained staff recorded otoacoustic emissions in 111 new-born children in order to compare the results of the Echosensor with the results of a conventional OAE measurements device (the ILO88). The aim of this pilot study was to determine the reliability and validity of the recordings in comparison with available standards in brainstem audiometry. Our study showed that the results of the Echosensor corresponded well with the ILO88 results. Consequently, an OAE measuring method is now available is also provides high sensitivity and specificity and is easy to use. Our findings show that the Echosensor can meet the demands of systematic hearing screening in Germany.
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Sordera/congénito , Tamizaje Neonatal/instrumentación , Emisiones Otoacústicas Espontáneas/fisiología , Procesamiento de Señales Asistido por Computador/instrumentación , Pruebas de Impedancia Acústica/instrumentación , Audiometría de Respuesta Evocada/instrumentación , Sordera/diagnóstico , Sordera/fisiopatología , Diseño de Equipo , Femenino , Humanos , Recién Nacido , Masculino , Variaciones Dependientes del Observador , Sensibilidad y EspecificidadRESUMEN
This paper reviews the current literature on the clinical aspects of juvenile rheumatoid arthritis (JRA) and the juvenile spondyloarthropathies. The classification of the juvenile arthritides remains controversial. A sibling pair registry established a role for genetic influences on the onset and course types of JRA. Even in the absence of steroid treatment, children with JRA demonstrated decreased bone mineral density and an impairment of linear growth. Magnetic resonance imaging was found to be helpful in detecting subtalar or sacroiliac involvement. Studies were published on the use of azathioprine, cyclosporine, and cyclopyhosphamide in the treatment of severe JRA. The lack of severe liver toxicity was shown in patients having received high total doses of methotrexate. An international agreement was reached on defining improvement in JRA. Several studies found an improved long-term outcome in patients with JRA or the juvenile spondyloarthropathies.
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Artritis Juvenil , Enfermedades de la Columna Vertebral , Artritis Juvenil/clasificación , Artritis Juvenil/diagnóstico , Artritis Juvenil/terapia , Humanos , Enfermedades de la Columna Vertebral/clasificación , Enfermedades de la Columna Vertebral/diagnóstico , Enfermedades de la Columna Vertebral/terapia , Resultado del TratamientoRESUMEN
BACKGROUND: The presence of an abnormal late-peaking left ventricular velocity contour, detected by Doppler echocardiography, has been reported in patients with hypertrophic cardiomyopathy, left ventricular hypertrophy, and aortic stenosis. METHODS AND RESULTS: To evaluate the clinical and prognostic significance of this characteristic Doppler finding, we studied a group of patients (n = 57) with isolated AS undergoing aortic valve replacement. Patients were divided into two groups according to the presence (n = 28) or absence (n = 29) of a hypertrophic late-peaking left ventricular velocity pattern. There were no differences between the groups with respect to age, sex, and presence of coronary artery disease. Peak preoperative aortic valve gradients were similar in both groups (70 vs. 67 mm Hg; p = not significant). The postoperative course of patients with abnormal late-peaking ventricular velocity contour was complicated by a higher incidence of arrhythmias (80% vs. 38%; p = 0.0002). Hypotension and the use of inotropic support were significantly more common in the group with abnormal late-peaking ventricular velocity contour (79% vs. 24%; p = 0.0001 and 76% vs. 26%; p = 0.0001, respectively). Hemodynamically, patients with a late-peaking velocity pattern exhibited a higher pulmonary diastolic pressure (16+/-3 vs 12+/-2 mm Hg; p = 0.0003), wedge pressure (12+/-4 vs 10+/-2; p < 0.05), and systemic vascular resistance (2126+/-459 vs 1553+/-199; p = 0.0001) and lower cardiac index (2.4+/-0.3 vs 2.9+/-0.4; p = 0.0001). CONCLUSIONS: An abnormal late-peaking ventricular velocity contour pattern appears to be a high risk marker for postoperative complications in patients undergoing aortic valve replacement. Routine Doppler evaluation in patients undergoing valve replacement for AS may therefore identify these patients.
Asunto(s)
Estenosis de la Válvula Aórtica/fisiopatología , Implantación de Prótesis de Válvulas Cardíacas , Disfunción Ventricular Izquierda/fisiopatología , Adulto , Anciano , Estenosis de la Válvula Aórtica/diagnóstico por imagen , Estenosis de la Válvula Aórtica/cirugía , Velocidad del Flujo Sanguíneo , Cateterismo Cardíaco , Ecocardiografía Doppler , Femenino , Humanos , Masculino , Persona de Mediana Edad , Pronóstico , Estudios Retrospectivos , Disfunción Ventricular Izquierda/diagnóstico por imagen , Disfunción Ventricular Izquierda/etiologíaRESUMEN
OBJECTIVES: We sought to determine the influence of plaque morphology and warfarin anticoagulation on the risk of recurrent emboli in patients with mobile aortic atheroma. BACKGROUND: An epidemiologic link between aortic atheroma and systemic emboli has been described both in pathologic and transesophageal studies. Likewise, a few studies have found an increased incidence of recurrent emboli in these patients. The therapeutic implications of these findings has not been studied. METHODS: Thirty-one patients presenting with a systemic embolic event and found to have mobile aortic atheroma were studied. The height, width and area of both immobile and mobile portions of atheroma were quantitated. The dimensions of the mobile component was used to define three groups: small, intermediate and large mobile atheroma. The patients were followed up by means of telephone interview and clinical records, with emphasis on anticoagulant use and recurrent embolic or vascular events. RESULTS: Patients not receiving warfarin had a higher incidence of vascular events (45% vs. 5%, p = 0.006). Stroke occurred in 27% of these patients and in none of those treated with warfarin. The annual incidence of stroke in patients not taking warfarin was 0.32. Myocardial infarction occurred in 18% of patients also in this group. Taken together, the risk of myocardial infarction or stroke was significantly increased in this group (p = 0.001). Forty-seven percent of patients with small, mobile atheroma did not receive warfarin. Recurrent stroke occurred in 38% of these patients, representing an annual incidence of 0.61. There were no strokes in patients with small, mobile atheroma treated with warfarin (p = 0.04). Likewise, none of the patients with intermediate or large mobile atheroma had a stroke during follow-up. Only three of these patients had not been taking warfarin. CONCLUSIONS: Patients presenting with systemic emboli and found to have mobile aortic atheroma on transesophageal echocardiography have a high incidence of recurrent vascular events. Warfarin is efficacious in preventing stroke in this population. The dimension of the mobile component of atheroma should not be used to determine the need for anticoagulation.