Registries in rheumatological and musculoskeletal conditions. Paediatric Behçet's disease: an international cohort study of 110 patients. One-year follow-up data.

OBJECTIVE: To set-up an international cohort of patients suspected with Behçet's disease (BD). The cohort is aimed at defining an algorithm for definition of the disease in children. METHODS: International experts have defined the inclusion criteria as follows: recurrent oral aphthosis (ROA) plus one of following-genital ulceration, erythema nodosum, folliculitis, pustulous/acneiform lesions, positive pathergy test, uveitis, venous/arterial thrombosis and family history of BD. Onset of disease is <16 years, disease duration is ≤3 years, future follow-up duration is ≥4 years and informed consent is obtained. The expert committee has classified the included patients into: definite paediatric BD (PED-BD), probable PED-BD and no PED-BD. Statistical analysis is performed to compare the three groups of patients. Centres document their patients into a single database. RESULTS: At January 2010, 110 patients (56 males/54 females) have been included. Mean age at first symptom: 8.1 years (median 8.2 years). At inclusion, 38% had only one symptom associated with ROA, 31% had two and 31% had three or more symptoms. A total of 106 first evaluations have been done. Seventeen patients underwent the first-year evaluation, and 36 had no new symptoms, 12 had one and 9 had two. Experts have examined 48 files and classified 30 as definite and 18 as probable. Twenty-six patients classified as definite fulfilled the International Study Group criteria. Seventeen patients classified as probable did not meet the international criteria. CONCLUSION: The expert committee has classified the majority of patients in the BD group although they presented with few symptoms independently of BD classification criteria.

Sistema d'adquisició de temperatures de 2 canals via R.F.

Poder mesurar i enregistrar diferents tipus de magnituds com pressió, força, temperatura etc. s’ha convertit en una necessitat per moltes aplicacions actuals. Aquestes magnituds poden tenir procedències molt diverses, tals com l’entorn, o poden ser generades per sistemes mecànics, elèctrics, etc. Per tal de poder adquirir aquestes magnituds, s’utilitzen els sistemes d’adquisició de dades. Aquests sistemes, prenen mostres analògiques del món real, i les transformen en dades digitals que poden ser manipulades per un sistema electrònic. Pràcticament qualsevol magnitud es pot mesurar utilitzant el sensor adient. Una magnitud molt utilitzada en sistemes d’adquisició de dades, és la temperatura. Els sistemes d’adquisició de temperatures estan molt generalitzats, i podem trobar-los com a sistemes, on l’objectiu és mostrar les dades adquirides, o podem trobar-los formant part de sistemes de control, aportant uns inputs necessaris per el seu correcte funcionament, garantir-ne l’estabilitat, seguretat etc. Aquest projecte, promogut per l’empresa Elausa, s’encarregarà d’adquirir, el senyal d’entrada de 2 Termoparells. Aquests mesuraran temperatures de circuits electrònics, que es trobaran dintre la càmera climàtica de Elausa, sotmesos a diferents condicions de temperatura, per tal de rebre l’homologació del circuit. El sistema haurà de poder mostrar les dades adquirides en temps real, i emmagatzemar-les en un PC que estarà ubicat en una oficina, situada a uns 30 m de distància de la sala on es farà el test. El sistema constarà d’un circuit electrònic que adquirirà, i condicionarà el senyal de sortida dels termoparells, per adaptar-lo a la tensió d’entrada d’un convertidor analògic digital, del microcontrolador integrat en aquesta placa. Seguidament aquesta informació, s’enviarà a través d’un mòdul transmissor de radiofreqüència, cap al PC on es visualitzaran les dades adquirides. Els objectius plantejats són els següents: - Dissenyar el circuit electrònic d’adquisició i condicionament del senyal. - Dissenyar, fabricar i muntar el circuit imprès de la placa d’adquisició. - Realitzar el programa de control del microcontrolador. - Realitzar el programa per presentar i desar les dades en un PC. - El sistema ha d’adquirir 2 temperatures, a través de Termoparells amb un rang d’entrada de -40ºC a +240ºC - S’ha de transmetre les dades via R.F. Els resultats del projecte han estat satisfactoris i s’han complert els objectius plantejats.

Efficiency and safety of bilateral contemporaneous pallidal stimulation (deep brain stimulation) in levodopa-responsive patients with Parkinson's disease with severe motor fluctuations: a 2-year follow-up review.

OBJECT: The aim of this study was to evaluate the long-term safety and efficacy of bilateral contemporaneous deep brain stimulation (DBS) in patients who have levodopa-responsive parkinsonism with untreatable motor fluctuations. Bilateral pallidotomy carries a high risk of corticobulbar and cognitive dysfunction. Deep brain stimulation offers new alternatives with major advantages such as reversibility of effects, minimal permanent lesions, and adaptability to individual needs, changes in medication, side effects, and evolution of the disease. METHODS: Patients in whom levodopa-responsive parkinsonism with untreatable severe motor fluctuations has been clinically diagnosed underwent bilateral pallidal magnetic resonance image-guided electrode implantation while receiving a local anesthetic. Pre- and postoperative evaluations at 3-month intervals included Unified Parkinson's Disease Rating Scale (UPDRS) scoring, Hoehn and Yahr staging, 24-hour self-assessments, and neuropsychological examinations. Six patients with a mean age of 55 years (mean 42-67 years), a mean duration of disease of 15.5 years (range 12-21 years), a mean "on/off' Hoehn and Yahr stage score of 3/4.2 (range 3-5), and a mean "off' time of 40% (range 20-50%) underwent bilateral contemporaneous pallidal DBS, with a minimum follow-up period lasting 24 months (range 24-30 months). The mean dose of levodopa in these patients could not be changed significantly after the procedure and pergolide was added after 12 months in five patients because of recurring fluctuations despite adjustments in stimulation parameters. All but two patients had no fluctuations until 9 months. Two of the patients reported barely perceptible fluctuations at 12 months and two at 15 months; however, two patients remain without fluctuations at 2 years. The mean improvements in the UPDRS motor score in the off time and the activities of daily living (ADL) score were more than 50%; the mean off time decreased from 40 to 10%, and the mean dyskinesia and complication of treatment scores were reduced to one-third until pergolide was introduced at 12 months. No significant improvement in "on" scores was observed. A slight worsening after 1 year was observed and three patients developed levodopa- and stimulation-resistant gait ignition failure and minimal fluctuations at 1 year. Side effects, which were controlled by modulation of stimulation, included dysarthria, dystonia, and confusion. CONCLUSIONS: Bilateral pallidal DBS is safe and efficient in patients who have levodopa-responsive parkinsonism with severe fluctuations. Major improvements in motor score, ADL score, and off time persisted beyond 2 years after the operation, but signs of decreased efficacy started to be seen after 12 months.

Customer Focus Newsletter, March/April, 2009, Vol. 6, no. 2

A bi-monthly bulletin to keep the department/agency management teams of state government better informed. We hope to consolidate most of the service update messages we send throughout the month and keep you updated about the work of the Customer Councils. If yours is one of the many departments who participated in the second annual DAS customer satisfaction survey recently, we thank you for taking the time to give us this important feedback. We look forward to sharing survey results with you, and pledge to consider responses carefully as we work to determine benchmarks and set future priorities.

Change in defense mechanisms and coping patterns during the course of 2-year-long psychotherapy and psychoanalysis for recurrent depression: a pilot study of a randomized controlled trial.

Very little research has been conducted so far to study the potential mechanisms of change in long-term active psychological treatments of recurrent depression. The present pilot randomized controlled trial aimed to determine the feasibility of studying the change process occurring in patients during the course of 2-year-long dynamic psychotherapy, psychoanalysis, and cognitive therapy, as compared with clinical management. In total, eight outpatients presenting with recurrent depression, two patients per treatment arm, were included. All patients were randomly assigned to one of the four treatment conditions. Defense mechanisms and coping patterns were assessed using validated observer-rated methodology based on transcribed, semistructured follow-along independent dynamic interviews. The results indicated that, whereas some patients in the active treatments changed on the symptomatic levels, some others remained unchanged during the course of their 2-year-long treatment. However, with regard to potential mechanisms of change in these patients, changes in defense mechanisms and coping patterns were revealed to be important processes over time in successful therapies and, to a lesser extent, in less successful treatments. No change was found either on outcome or on the process measure for the control condition, that is, clinical management. These results are discussed along with previous data comparing change in defense mechanisms and coping during the course of treatments.

Business Sphere, Vol. 17, no.2

Anadvanced foods economy is under development in the state of Iowa that builds on Iowa’s established strengths in plant and animal sciences,production agriculture, food science, nutrition, and processing technology. This new pathway was identified when Battelle Institute studied Iowa’s strengths in the biosciences and recommended six technology platforms as opportunities to use these strengths for growing the economy. One of those technology platforms is functional foods, sometimes called nutraceuticals.

Business Sphere, Vol. 21, no.2

In the fall of 2007, a group of individuals representing a wide range of aspects of the wind industry gathered together and voted to establish the Iowa Wind Energy Association (IWEA). Now in its second year and operated out of Iowa Lakes Community College in Estherville, our association’s increasing membership rolls parallel the phenomenal growth of the wind energyindustry in our state. IWEA has just concluded our second annual meeting which brought together a host of wind energy experts to share their knowledge and expertise. Attending were wind energy companies, wind developers, agricultural landowners, large- and small-scale wind farm producers, construction companies, energy companies, educators and students. This broad range of interests pursuing common goals has made the Iowa WindEnergy Association one of the largest state wind organizations in the nation.

Customer Focus Newsletter, March-April, 2010, Vol. 7, no.2

A bi-monthly bulletin to keep the department/agency management teams of state government better informed. We hope to consolidate most of the service update messages we send throughout the month and keep you updated about the work of the Customer Councils. If yours is one of the many departments who participated in the second annual DAS customer satisfaction survey recently, we thank you for taking the time to give us this important feedback. We look forward to sharing survey results with you, and pledge to consider responses carefully as we work to determine benchmarks and set future priorities.

Business Sphere, Vol. 20, no.2

With its central U.S. location, access to a plethora of agricultural raw materials, a highly educated and skilled workforce, and a supportive state government; food and ingredient manufacturers find many advantages to locating in Iowa. Another major plus for Iowa’s food makers is access to one of the strongest food science and human nutrition programs in the nation, located on the campus of Iowa State University (ISU). At ISU, you will find scientists who will assist your organization in bringing food related innovations in plant, animal and microbial products to commercialization. The Department of Food Sciences and Human Nutrition (FSHN) is jointly administered by the Colleges of Agriculture and Life Sciences and Human Sciences. Our mission is to generate new knowledge around food and human nutrition and to promote health through food.

Business Sphere, Vol. 19, no.2

The first commercial quantities of a soybean oil with about three percent linolenic acid oil were produced in Iowa in 1994 through a collaboration with Pioneer Hi-Bred International, Inc. Continued to breed new soybean varieties with a focus on a even lower linolenic acid content of one percent. I was interested in understanding whether the oil would be stable enough to eliminate the need for chemical hydrogenation. During the process of increasing one percent linolenic acid seed varieties to obtain oil for testing, the Food and Drug Administration announced it would require labeling for trans fat beginning in 2006. Instead of using the seed of the new varieties to obtain oil for testing, it was used for further seed production so that commercial quantities of the one percent linolenic acid oil could be available as quickly as possible.

Glomerular hyperfiltration and increased proximal sodium reabsorption in subjects with type 2 diabetes or impaired fasting glucose in a population of the African region.

BACKGROUND. Glomerular hyperfiltration (GHF) is a well-recognized early renal alteration in diabetic patients. As the prevalence of GHF is largely unknown in populations in the African region with respect to normal fasting glucose (NFG), impaired fasting glucose (IFG) and type 2 diabetes [diabetes mellitus (DM)], we conducted a cross-sectional study in the Seychelles islands among families including at least one member with hypertension. METHODS. The glomerular filtration rate (GFR), effective renal plasma flow (ERPF) and proximal tubular sodium reabsorption were measured using inulin, p-aminohippurate (PAH) and endogenous lithium clearance, respectively. Twenty-four-hour urine was collected on the preceding day. RESULTS. Of the 363 participants (mean age 44.7 years), 6.6% had IFG, 9.9% had DM and 63.3% had hypertension. The prevalence of GHF, defined as a GFR >140 ml/min, was 17.2%, 29.2% and 52.8% in NFG, IFG and DM, respectively (P trend <0.001). Compared to NFG, the adjusted odds ratio for GHF was 1.99 [95% confidence interval (CI) 0.73-5.44] for IFG and 5.88 (2.39-14.45) for DM. Lithium clearance and fractional excretion of lithium were lower in DM and IFG than NFG (P < 0.001). CONCLUSION. In this population of African descent, subjects with impaired fasting glucose or type 2 diabetes had a high prevalence of GHF and enhanced proximal sodium reabsorption. These findings provide further insight on the elevated incidence of nephropathy reported among African diabetic individuals.

p53-induced protein with a death domain (PIDD) isoforms differentially activate nuclear factor-kappaB and caspase-2 in response to genotoxic stress

Cells respond to DNA damage in a complex way and the fate of damaged cells depends on the balance between pro- and antiapoptotic signals. This is of crucial importance in cancer as genotoxic stress is implied both in oncogenesis and in classical tumor therapies. p53-induced protein with a death domain (PIDD), initially described as a p53-inducible gene, is one of the molecular switches able to activate a survival or apoptotic program. Two isoforms of PIDD, PIDD (isoform 1) and LRDD (isoform 2), have already been reported and we describe here a third isoform. These three isoforms are differentially expressed in tissues and cell lines. Genotoxic stress only affects PIDD isoform 3 mRNA levels, whereas isoforms 1 and 2 mRNA levels remain unchanged. All isoforms are capable of activating nuclear factor-kappaB in response to genotoxic stress, but only isoform 1 interacts with RIP-associated ICH-1/CED-3 homologous protein with a death domain and activates caspase-2. Isoform 2 counteracts the pro-apoptotic function of isoform 1, whereas isoform 3 enhances it. Thus, the differential splicing of PIDD mRNA leads to the formation of at least three proteins with antagonizing/agonizing functions, thereby regulating cell fate in response to DNA damage

Lessons learned after 2 full scale disaster exercises in a Swiss pediatric hospital

Introduction: Following a disaster, up to 50% of mass casualties are children. The number of disaster increases worldwide, including in Switzerland. Following national order, the mapping of the various risks of disaster in Switzerland will be completed by the end of 2012. Pre-hospital disaster drills and plans are well established and regularly tested. In-hospital disaster plans are much less frequently tested, if only available. Pediatric in-hospital full scale disaster exercises have never been reported in Switzerland. Based on our local constraints, we set up and evaluated a disaster plan during two full scale exercises. Methods: In a university hospital treating more than 35 000 pediatric emergencies per year, two exercises involving mock victims of a disaster aged 9 to 14 years old were successively set up over a period of 3 years. The exercises were planned during the day, without modification of the normal emergency room activities. The hospital staff was informed and trained in advance. Variables such as the alarm timing and transmission, triage set-up and function, special disaster medical records utilization, communication and victims' identification were assessed. Family members participated in the second exercise. An evaluation team observed and record exercises activities, identifying strength and weaknesses. Results: On two separate occasions, a total of 44 mock patients participated, were triaged, admitted and treated in the hospital according to usual standards of care. Alarm transmission was not appropriate during the first exercise. Triage overload occurred on one occasion. In-hospital communication needed readjustment. Identification and in-hospital tracking of the children remained problematic. Hospital employees showed great enthusiasm and stressed the positive effect of full scale exercises on their knowledge of the hospital disaster plan. Conclusions: Performing real life disaster exercises in a pediatric hospital was very beneficial. The disaster plan was adapted to local needs and updated accordingly. An alarm transmission protocol was elaborated and tested. Triage set-up was adapted and tested. A hospital identification plan for injured children was created and tested. Full scale hospital exercises evaluating disaster plans revealed several weaknesses in the system. Practice readjustments based on local experience were made. A tested pediatric disaster plan adapted to local constraints could minimize chaos, optimize care and support in the event of a real disaster. Children's identification and family reunification following a disaster remains a challenge.

Whole-exome sequencing detects somatic mutations of IDH1 in metaphyseal chondromatosis with D-2-hydroxyglutaric aciduria (MC-HGA).

We used exome sequencing of blood DNA in four unrelated patients to identify the genetic basis of metaphyseal chondromatosis with urinary excretion of D-2-hydroxy-glutaric acid (MC-HGA), a rare entity comprising severe chondrodysplasia, organic aciduria, and variable cerebral involvement. No evidence for recessive mutations was found; instead, two patients showed mutations in IDH1 predicting p.R132H and p.R132S as apparent somatic mosaicism. Sanger sequencing confirmed the presence of the mutation in blood DNA in one patient, and in blood and saliva (but not in fibroblast) DNA in the other patient. Mutations at codon 132 of IDH1 change the enzymatic specificity of the cytoplasmic isocitrate dehydrogenase enzyme. They result in increased D-2-hydroxy-glutarate production, α-ketoglutarate depletion, activation of HIF-1α (a key regulator of chondrocyte proliferation at the growth plate), and reduction of N-acetyl-aspartyl-glutamate level in glial cells. Thus, somatic mutations in IDH1 may explain all features of MC-HGA, including sporadic occurrence, metaphyseal disorganization, and chondromatosis, urinary excretion of D-2-hydroxy-glutaric acid, and reduced cerebral myelinization.