Vitrectomy for juvenile uveitis: prognostic factors for the long-term functional outcome

PURPOSE: This study evaluated the long-term effect of pars plana vitrectomy (PPV) in children and adolescents with chronic uveitis on visual function, anatomical outcome, and the requirement of systemic treatment. Further, predictive preoperative factors associated with a beneficial visual outcome were assessed. METHODS: Retrospective review of 29 eyes of 23 consecutive paediatric and juvenile patients below 20 years of age with chronic uveitis who underwent a PPV for visually significant opacities in 25 eyes, vitreous haemorrhage in three eyes, and retinal detachment in one eye. The clinical diagnosis was chronic intermediate uveitis in 22 eyes and retinal vasculitis of different origin in seven eyes. RESULTS: LogMAR visual acuity improved from an average of 0.91 to 0.33 (P<0.001). Cystoid macular oedema (CME) was significantly reduced in eight of 10 eyes postoperatively (P=0.021). In the multiple regression analysis, a low preoperative logMAR visual acuity and the presence of a CME had a negative influence on the final logMAR visual acuity. Furthermore, the appearance of chronic uveitis relapses was significantly reduced from 15 eyes before to seven eyes after surgery (P=0.042). CONCLUSIONS: PPV has a beneficial effect on the course and the complications of chronic uveitis in paediatric and juvenile patients with respect to the anatomical and visual outcome. Preoperative logMAR visual acuity and clinically significant CME were the most accurate predictors for the functional outcome.

Limited value of cyclosporine A for the treatment of patients with uveitis associated with juvenile idiopathic arthritis

AIMS: Juvenile idiopathic arthritis (JIA) is often associated with severe chronic anterior uveitis (CAU), and immunosuppressive therapy may be required. In this study, the value of cyclosporine A (CsA) as monotherapy or as combination therapy for treating uveitis was studied in a large cohort of JIA children. METHODS: Multicentre retrospective study including 82 JIA children (girls n=60) suffering from unilateral or bilateral (n=55) CAU. The indication for CsA was active uveitis, although patients were on topical or systemic corticosteroids, MTX, or other immunosuppressive drugs. RESULTS: Inactivity of uveitis during the entire treatment period (mean 3.9 years) was obtained with CsA monotherapy in 6 of 25 (24%) patients, but more often when CsA was combined with the immunosuppressives (35/72 patients; 48.6%, P=0.037), or MTX (18/37 patients, 48.6%, P=0.065), which had already been given. With CsA (mean dosage 2.9 mg/kg), systemic immunosuppressive drugs and steroids could be reduced by >or=50% (n=19) or topical steroids reduced to

Early diagnosis of temporomandibular joint involvement in juvenile idiopathic arthritis: a pilot study comparing clinical examination and ultrasound to magnetic resonance imaging

OBJECTIVES: To study the validity of both rheumatological and orthodontic examinations and ultrasound (US) as screening methods for early diagnosis of TMJ arthritis against the gold standard MRI. METHODS: Thirty consecutive juvenile idiopathic arthritis (JIA) patients were included in this pilot study. Rheumatological and orthodontic examinations as well as US were performed within 1 month of the MRI in a blinded fashion. Joint effusion and/or increased contrast enhancement of synovium or bone were considered signs of active arthritis on MRI. RESULTS: A total of 19/30 (63%) patients and 33/60 (55%) joints had signs of TMJ involvement on MRI. This was associated with condylar deformity in 9/19 (47%) patients and 15/33 (45%) joints. Rheumatological, orthodontic and US examinations correctly diagnosed 11 (58%), 9 (47%) and 6 (33%) patients, respectively, with active TMJ arthritis, but misdiagnosed 8 (42%), 10 (53%) and 12 (67%) patients, respectively, as having no signs of inflammation. The best predictor for active arthritis on MRI was a reduced maximum mouth opening. CONCLUSION: None of the methods tested was able to reliably predict the presence or absence of MRI-proven inflammation in the TMJ in our cohort of JIA patients. US was the least useful of all methods tested to exclude active TMJ arthritis.

Juvenile myoclonic epilepsy with photosensitivity in a female with Velocardiofacial syndrome (del(22)(q11.2))--causal relationship or coincidence?

We report on an adolescent female with Velocardiofacial syndrome (del(22)(q11.2)) and an epilepsy phenotype resembling juvenile myoclonic epilepsy (JME). Clinically, the patient has characteristic signs of both disorders. JME has been linked to several chromosomes, but has not been related to 22q11.2 and is rarely observed in other genetic syndromes. We discuss possible explanations for a relationship between the chromosomal aberration and epilepsy as well as the importance of precise delineation of both epilepsy phenotypes and genetic defects in chromosomal disorders.

[Eye complications in chronic juvenile arthritis]

BACKGROUND: The relationship between uveitis anterior in childhood and juvenile chronic arthritis (JCA, respectively JRA) has been known since 1950. In a review, the clinical picture of uveitis anterior, its prevalence, pathogenesis, prognosis and current therapy of ocular complications are presented. In addition, we will report our results of a clinical study. PATIENTS AND METHODS: In a cross-sectional study, 64 patients with juvenile chronic arthritis (JCA) had an ophthalmological screening for eye complications either from the disease itself or from the treatment. RESULTS: In 16% of the patients, an iridocyclitis was found, in one case acute, in 9 cases chronic. The cases of chronic uveitis anterior showed in 43% a combination with the classic risk factors (ANA-positive, oligoarticular, female). At the beginning of uveitis, the patients had a mean age of 81 months, at the beginning of JCA disease a mean age of 37 months. Four of 10 patients (= 40%) had eye complications from uveitis (cataract, posterior synechiae, glaucoma). Complications from therapy were found in 27%, mostly cataract as a complication of systemic and topical steroid treatment. Eighteen % had a visual acuity of 0.4 or less. CONCLUSIONS: Because of the often asymptomatic progression of chronic uveitis anterior, the risk of severe undetected eye complications is high. Therefore, an intensive interdisciplinary cooperation between rheumatologists, pediatrics and ophthalmologists is required.

[Juvenile chronic arthritis: a rare disease with potentially serious consequences]

The term juvenile chronic arthritis covers several nosological entities with very different clinical presentation, course and prognosis. This review addresses non-rheumatologists and non-pediatricians. Diagnostic criteria, clinical presentation of the most common nosological subgroups and relevant investigations are spotlighted. Therapeutic guidelines are presented, bearing in mind, that for each child an individual interdisciplinary plan has to be elaborated. This would involve close cooperation between the pediatrician, the pediatric rheumatologist, the family doctor, parents and, if necessary, the physiotherapist, ergotherapist, social worker, ophthalmologist, orthopedic surgeon and school teachers. It is essential that each member of this therapeutic team reports regularly.

[Juvenile chronic arthritis: therapeutic strategy 1990]

Therapeutic strategies based on experience with 119 patients with juvenile chronic arthritis are reviewed. Therapeutic goals are formulated and the means of attaining them (NSAIDs, the so-called disease modifying drugs gold, chloroquine and penicillamine, the antimetabolite methotrexate, intra-articular and systemic corticosteroids, physio- and ergotherapy, technical and orthopedic measures, as well as vocational and medicosocial aspects) are discussed. As the individual prognosis normally depends less on drugs than on preventive and rehabilitative measures, the outcome is largely determined by the quality of a well-coordinated inter-disciplinary team approach.

Anomic Crime in Post-Welfarist Societies. Cult of the Individual, Integration Patterns and Delinquency

Post-Fordist economies come along with post-welfarist societies marked by intensified cultural individualism and increased structural inequalities. These conditions are commonly held to be conducive to relative deprivation and, thereby, anomic crime. At the same time, post-welfarist societies develop a new ‘balance of power’ between institutions providing for welfare regulation, such as the family, the state and the (labour) market – and also the penal system. These institutions are generally expected to improve social integration, ensure conformity and thus reduce anomic crime. Combining both perspectives, we analyse the effects of moral individualism, social inequality, and different integration strategies on crime rates in contemporary societies through the lenses of anomie theory. To test our hypotheses, we draw on time-series cross-section data compiled from different data sources (OECD, UN, WHO, WDI) for twenty developed countries in the period 1970-2004, and run multiple regressions that control for country-specific effects. Although we find some evidence that the mismatch between cultural ideal (individual inclusion) and structural reality (stratified exclusion) increases the anomic pressure, whereas conservative (i. e. family-based), social-democratic (i. e. state-based) and liberal (i. e. market-based) integration strategies to a certain extent prove effective in controlling the incidence of crime, the results are not very robust. Moreover, reservations have to be made regarding the effects of “market” income inequality as well as familialist, unionist and liberalist employment policies that are shown to have reversed effects in our sample: the former reducing, the latter occasionally increasing anomic crime. As expected, the mismatch between cultural ideal (individual inclusion) and structural reality (stratified exclusion) increases the anomic pressure, whereas conservative (i. e. family-based), social-democratic (i. e. state-based) and liberal (i. e. market-based) integration strategies generally prove effective in controlling the incidence of crime. Nevertheless, we conclude that the new cult of the individual undermines the effectiveness of conservative and social-democratic integration strategies and drives societies towards more “liberal” regimes that build on incentive as well as punitive elements.

Pseudomonas chlororaphis strain JF3835 reduces mortality of juvenile perch, Perca fluviatilis L., caused by Aeromonas sobria

Motile aeromonad septicaemia caused by Aeromonas sobria is a cause of disease in farmed perch, Perca fluviatilis L., in Switzerland. We have evaluated the potential of a Pseudomonas chlororaphis isolate, obtained from perch intestine, to control A. sobria infection. Inoculation of juvenile perch with P. chlororaphis strain JF3835 prior to infection with A. sobria caused a reduction in A. sobria associated mortalities. Infection of perch with xylE-labelled P. chlororaphis indicated the bacterium is able to transiently colonize juvenile fish and fingerlings.

Juvenile onset central nervous system folate deficiency and rheumatoid arthritis.

Isolated cerebral folate deficiency was detected in a 13-year-old girl with cognitive and motor difficulties and juvenile rheumatoid arthritis. Her serum contains autoantibodies that block membrane-bound folate receptors that are on the choroid plexus and diminish the uptake of folate into the spinal fluid. Whereas her serum folate exceeded 21 ng/mL, her spinal fluid contained 3.2 ng/mL of 5-methyltetrahydrofolate as a consequence of the autoantibodies diminishing the uptake of this folate.

Temperature and Resource Availability May Interactively Affect Over-Wintering Success of Juvenile Fish in a Changing Climate

The predicted global warming may affect freshwater systems at several organizational levels, from organism to ecosystem. Specifically, in temperate regions, the projected increase of winter temperatures may have important effects on the over-winter biology of a range of organisms and especially for fish and other ectothermic animals. However, temperature effects on organisms may be directed strongly by resource availability. Here, we investigated whether over-winter loss of biomass and lipid content of juvenile roach (Rutilus rutilus) was affected by the physiologically relatively small (2-5°C) changes of winter temperatures predicted by the Intergovernmental Panel on Climate Change (IPCC), under both natural and experimental conditions. This was investigated in combination with the effects of food availability. Finally, we explored the potential for a correlation between lake temperature and resource levels for planktivorous fish, i.e., zooplankton biomass, during five consecutive winters in a south Swedish lake. We show that small increases in temperature (+2°C) affected fish biomass loss in both presence and absence of food, but negatively and positively respectively. Temperature alone explained only a minor part of the variation when food availability was not taken into account. In contrast to other studies, lipid analyses of experimental fish suggest that critical somatic condition rather than critical lipid content determined starvation induced mortality. Our results illustrate the importance of considering not only changes in temperature when predicting organism response to climate change but also food-web interactions, such as resource availability and predation. However, as exemplified by our finding that zooplankton over-winter biomass in the lake was not related to over-winter temperature, this may not be a straightforward task.

An exploratory investigation of America's most costly delinquents: Juvenile firesetters

This study focused on the instruments that are currently being used by fire department personnel to identify and classify juvenile firesetters, these instruments, as published by the Federal Emergency Management Agency (F.E.M.A.) have never been empirically validated as to their ability to discriminate between first time and multiple firesetters and to predict the degree of risk for future firesetting by juveniles that come to the attention of authorities for firesetting behaviors. The study was descriptive in nature and not designed to test the validity of these instruments. The study was designed to test the ability of the instruments to discriminate between first time and multiple firesetters and to categorize known firesetters, based on the motive for firesetting, as to their degree or risk for future firesetting.^ The results suggest that the F.E.M.A. instruments are of little use in discriminating between first time and multiple firesetters. The F.E.M.A. instruments were not able to categorize juvenile firesetters as to their potential risk for future firesetting. A subset of variables from the F.E.M.A. instruments was identified that may be useful in discriminating between youth that are troubled firesetters and those that are not. ^