This talk was given by Dr. Hermine Brunner of Cincinnati Childrens Hospital to a group of patient families, at Systemic Juvenile Idiopathic Arthritis (or SJIA) Family Day on July 22nd, 2017.
is an umbrella term referring to a group of disorders characterized by chronic arthritis. JIA is the most common chronic rheumatic illness in children and is a significant cause of short-and long-term disability.
It is a clinical diagnosis made in a child less than16 years of age with arthritis (defined as swelling or limitation of motion of the joint accompanied by heat, pain, or tenderness) for at least 6 weeks’ duration with other identifiable causes of arthritis excluded.
is an umbrella term referring to a group of disorders characterized by chronic arthritis. JIA is the most common chronic rheumatic illness in children and is a significant cause of short-and long-term disability.
It is a clinical diagnosis made in a child less than16 years of age with arthritis (defined as swelling or limitation of motion of the joint accompanied by heat, pain, or tenderness) for at least 6 weeks’ duration with other identifiable causes of arthritis excluded.
Tmj Ankylosis In Still’s Disease – A Case ReportQUESTJOURNAL
ABSTRACT: Rheumatoid arthritis (RA) is a chronic inflammatory disease characterized by joint swelling, joint tenderness, and destruction of synovial joints, leading to severe disability and premature mortality. TMJ complaints are present in about more than 50% of patients of RA. TMJ is usually among the last joint to be involved and is associated with many clinical signs and symptoms of which pain is a major problem later leading to inflammation, limited movements, swelling (joint stiffness) and muscle spasm. If it occurs in early age it may result in mandibular growth disturbance, facial deformity, and ankylosis and in adult these can vary from mild joint stiffness to total joint disruption with occlusal-facial deformity. The diagnosis and management of TMJ involvement in RA is exclusionary based on history, physical findings, radiographic study, and lab testing. Hence a multidisciplinary approach is necessary. The present paper reports a case of RA with bilateral TMJ involvement with its classical radiographic findings.
the presentation gives a detail information about the seronegative spondyloarthropathy. this ppt also provide recent evidences to frame the rehab protocol.
Leading Quality Improvements in Pediatric Rheumatology Care - Dr. Esi MorganSystemic JIA Foundation
This talk was given by Dr. Esi Morgan of Cincinnati Childrens Hospital to a group of patient families, at Systemic Juvenile Idiopathic Arthritis (or SJIA) Family Day on July 22nd, 2017.
This talk was given by Dr. Daniel Lovell of Cincinnati Children's Hospital to a group of patient families, at Systemic Juvenile Idiopathic Arthritis (or SJIA) Family Day on July 22nd, 2017.
This talk was given by Dr. Grant Schulert Cincinnati Children's Hospital to a group of patient families, at Systemic Juvenile Idiopathic Arthritis (or SJIA) Family Day on July 22nd, 2017.
This talk was given by Dr. Alexei Grom of Cincinnati Childrens Hospital to a group of patient families, at Systemic Juvenile Idiopathic Arthritis (or SJIA) Family Day on July 22nd, 2017.
This talk was given by Dr. Jennifer Huggins of Cincinnati Childrens Hospital, at the Systemic Juvenile Idiopathic Arthritis (or SJIA) Family Day, on July 22nd, 2017.
Rare Pulmonary Diseases in Systemic JIA. This presentation tracks the increased use of biologics to treat SJIA and observes the trends in rare pulmonary diseases.
Nucleophilic Addition of carbonyl compounds.pptxSSR02
Nucleophilic addition is the most important reaction of carbonyls. Not just aldehydes and ketones, but also carboxylic acid derivatives in general.
Carbonyls undergo addition reactions with a large range of nucleophiles.
Comparing the relative basicity of the nucleophile and the product is extremely helpful in determining how reversible the addition reaction is. Reactions with Grignards and hydrides are irreversible. Reactions with weak bases like halides and carboxylates generally don’t happen.
Electronic effects (inductive effects, electron donation) have a large impact on reactivity.
Large groups adjacent to the carbonyl will slow the rate of reaction.
Neutral nucleophiles can also add to carbonyls, although their additions are generally slower and more reversible. Acid catalysis is sometimes employed to increase the rate of addition.
ANAMOLOUS SECONDARY GROWTH IN DICOT ROOTS.pptxRASHMI M G
Abnormal or anomalous secondary growth in plants. It defines secondary growth as an increase in plant girth due to vascular cambium or cork cambium. Anomalous secondary growth does not follow the normal pattern of a single vascular cambium producing xylem internally and phloem externally.
ESR spectroscopy in liquid food and beverages.pptxPRIYANKA PATEL
With increasing population, people need to rely on packaged food stuffs. Packaging of food materials requires the preservation of food. There are various methods for the treatment of food to preserve them and irradiation treatment of food is one of them. It is the most common and the most harmless method for the food preservation as it does not alter the necessary micronutrients of food materials. Although irradiated food doesn’t cause any harm to the human health but still the quality assessment of food is required to provide consumers with necessary information about the food. ESR spectroscopy is the most sophisticated way to investigate the quality of the food and the free radicals induced during the processing of the food. ESR spin trapping technique is useful for the detection of highly unstable radicals in the food. The antioxidant capability of liquid food and beverages in mainly performed by spin trapping technique.
Remote Sensing and Computational, Evolutionary, Supercomputing, and Intellige...University of Maribor
Slides from talk:
Aleš Zamuda: Remote Sensing and Computational, Evolutionary, Supercomputing, and Intelligent Systems.
11th International Conference on Electrical, Electronics and Computer Engineering (IcETRAN), Niš, 3-6 June 2024
Inter-Society Networking Panel GRSS/MTT-S/CIS Panel Session: Promoting Connection and Cooperation
https://www.etran.rs/2024/en/home-english/
Deep Behavioral Phenotyping in Systems Neuroscience for Functional Atlasing a...Ana Luísa Pinho
Functional Magnetic Resonance Imaging (fMRI) provides means to characterize brain activations in response to behavior. However, cognitive neuroscience has been limited to group-level effects referring to the performance of specific tasks. To obtain the functional profile of elementary cognitive mechanisms, the combination of brain responses to many tasks is required. Yet, to date, both structural atlases and parcellation-based activations do not fully account for cognitive function and still present several limitations. Further, they do not adapt overall to individual characteristics. In this talk, I will give an account of deep-behavioral phenotyping strategies, namely data-driven methods in large task-fMRI datasets, to optimize functional brain-data collection and improve inference of effects-of-interest related to mental processes. Key to this approach is the employment of fast multi-functional paradigms rich on features that can be well parametrized and, consequently, facilitate the creation of psycho-physiological constructs to be modelled with imaging data. Particular emphasis will be given to music stimuli when studying high-order cognitive mechanisms, due to their ecological nature and quality to enable complex behavior compounded by discrete entities. I will also discuss how deep-behavioral phenotyping and individualized models applied to neuroimaging data can better account for the subject-specific organization of domain-general cognitive systems in the human brain. Finally, the accumulation of functional brain signatures brings the possibility to clarify relationships among tasks and create a univocal link between brain systems and mental functions through: (1) the development of ontologies proposing an organization of cognitive processes; and (2) brain-network taxonomies describing functional specialization. To this end, tools to improve commensurability in cognitive science are necessary, such as public repositories, ontology-based platforms and automated meta-analysis tools. I will thus discuss some brain-atlasing resources currently under development, and their applicability in cognitive as well as clinical neuroscience.
BREEDING METHODS FOR DISEASE RESISTANCE.pptxRASHMI M G
Plant breeding for disease resistance is a strategy to reduce crop losses caused by disease. Plants have an innate immune system that allows them to recognize pathogens and provide resistance. However, breeding for long-lasting resistance often involves combining multiple resistance genes
Toxic effects of heavy metals : Lead and Arsenicsanjana502982
Heavy metals are naturally occuring metallic chemical elements that have relatively high density, and are toxic at even low concentrations. All toxic metals are termed as heavy metals irrespective of their atomic mass and density, eg. arsenic, lead, mercury, cadmium, thallium, chromium, etc.
hematic appreciation test is a psychological assessment tool used to measure an individual's appreciation and understanding of specific themes or topics. This test helps to evaluate an individual's ability to connect different ideas and concepts within a given theme, as well as their overall comprehension and interpretation skills. The results of the test can provide valuable insights into an individual's cognitive abilities, creativity, and critical thinking skills
Observation of Io’s Resurfacing via Plume Deposition Using Ground-based Adapt...Sérgio Sacani
Since volcanic activity was first discovered on Io from Voyager images in 1979, changes
on Io’s surface have been monitored from both spacecraft and ground-based telescopes.
Here, we present the highest spatial resolution images of Io ever obtained from a groundbased telescope. These images, acquired by the SHARK-VIS instrument on the Large
Binocular Telescope, show evidence of a major resurfacing event on Io’s trailing hemisphere. When compared to the most recent spacecraft images, the SHARK-VIS images
show that a plume deposit from a powerful eruption at Pillan Patera has covered part
of the long-lived Pele plume deposit. Although this type of resurfacing event may be common on Io, few have been detected due to the rarity of spacecraft visits and the previously low spatial resolution available from Earth-based telescopes. The SHARK-VIS instrument ushers in a new era of high resolution imaging of Io’s surface using adaptive
optics at visible wavelengths.
This presentation explores a brief idea about the structural and functional attributes of nucleotides, the structure and function of genetic materials along with the impact of UV rays and pH upon them.
Phenomics assisted breeding in crop improvementIshaGoswami9
As the population is increasing and will reach about 9 billion upto 2050. Also due to climate change, it is difficult to meet the food requirement of such a large population. Facing the challenges presented by resource shortages, climate
change, and increasing global population, crop yield and quality need to be improved in a sustainable way over the coming decades. Genetic improvement by breeding is the best way to increase crop productivity. With the rapid progression of functional
genomics, an increasing number of crop genomes have been sequenced and dozens of genes influencing key agronomic traits have been identified. However, current genome sequence information has not been adequately exploited for understanding
the complex characteristics of multiple gene, owing to a lack of crop phenotypic data. Efficient, automatic, and accurate technologies and platforms that can capture phenotypic data that can
be linked to genomics information for crop improvement at all growth stages have become as important as genotyping. Thus,
high-throughput phenotyping has become the major bottleneck restricting crop breeding. Plant phenomics has been defined as the high-throughput, accurate acquisition and analysis of multi-dimensional phenotypes
during crop growing stages at the organism level, including the cell, tissue, organ, individual plant, plot, and field levels. With the rapid development of novel sensors, imaging technology,
and analysis methods, numerous infrastructure platforms have been developed for phenotyping.
What is SJIA - How is it different than other diseases - Dr. Hermine Brunner
1. What is SJIA - how is it different
than other diseases
HERMINE BRUNNER, MD MSC MBA
PROFESSOR OF PEDIATRICS
CHIEF, DIVISION OF RHEUMATOLOGY
2. Sir George Frederic Stills
(27 February 1868 – 28 June 1941)
England’s first professor of childhood medicine
First described 22 children with SJIA in 1897
…also the first to describe attention deficit hyperactivity disorder
Still G F. On a form of
chronic joint disease in
children.Med-Chir Trans
1897; 80: 47-59.
Systemic-onset
juvenile chronic
arthritis
Systemic-onset
juvenile rheumatoid
arthritis
Systemic juvenile
idiopathic arthritis
Still’s disease
2
3. 7. Shenoi S, Wallace CA. J Pediatr. 2016;177:19-26.
3
4. Rash
Nonpruritic, evanescent, urticarial-like,
maculopapular, salmon colored
Koebner phenomenon
Histologic evidence of sparse: Cellular
perivascular infiltrate
Just as in other inflammatory processes (such as psoriasis,
lichen planus, cutaneous lupus or wound healing)
Activated keratinocytes expressing proinflammatory S100-
proteins
M. Frosch, et al., “Expression of myeloidrelated proteins 8 and
14 in SJRA. A&R vol. 48, no. 9, pp. 2622–2626, 2003
4
5. Pericardial effusion > pleural effusion > peritoneal fluid
Pericarditis
Typical anterior chest pain, shortness of breath, friction
rub on auscultation
Diagnosed by x-ray, EKG, echocardiography
Myocarditis much less common
4% in one study
Serositis
J. et al., “Symptomatic cardiac involvement in juvenile
rheumatoid arthritis,” International Journal of Cardiology, vol. 34,
no. 1, pp. 57–62, 1992.
5
6. Fever
(Double) quotidian fever
(2) fever spike(s) per day, not induced by antipyretic medications
About the same time every day
DD: Malaria, Leishmaniasis
The classic pattern with 1 spike in the evening is only
seen in 37% of the patients during initial presentation
Morning fevers (12%),
Twice daily fevers (15%)
Intermittent fevers (27%)
Unremitting fevers (5%)
E. M. Behrens et al: Evaluation of the presentation of sJRA . J Rheumatol, vol. 35, no. 2, pp. 343–348, 2008.
6
8. 7. Shenoi S, Wallace CA. J Pediatr. 2016;177:19-26.
8. Petty RE et al. J Rheumatol. 2004;31:390-392.
8
9. SJIA – One of the Types of juvenile
idiopathic Arthritis
Group of Diseases
Shared features
Chronic Arthritis of unknown etiology
Presentation by 16th birthday
Petty RE, Southwood TR, Manners P, Baum J, Glass DN, Goldenberg J, He X, Maldonado-Cocco J, Orozco-Alcala J, Prieur AM, Suarez-Almazor ME, Woo P: International League of Associations for
Rheumatology classification of juvenile idiopathic arthritis: second revision, Edmonton, 2001. J Rheumatol 2004, 31(2):390–392. Stoll, Matthew L., and Randy Q. Cron. "Treatment of juvenile idiopathic arthritis:
a revolution in care." Pediatric rheumatology 12.1 (2014): 13.
9
SJIA is mostly a disease
starting in childhood
13. Systemic JIA – Not A Single Gene
Disease
More than a monogenic inflammatory disease
SJIA rarely runs in families
Polygenic auto-inflammatory disease
1. No auto-reactive T cells or antibodies at onset
2. No strong human leucocyte antigen (HLA) associations
3. Microarray studies innate immune activity involving a cellular
structures called “inflammasomes”
S. Vastert, B. Prakken 232 / Best Practice & Research Clinical Rheumatology 28 (2014) 229-246
13
14. Non-systemic Forms of JIA – Adaptive
Immune System Activation
T cell subsets (Th17) and different Treg cells are very important
for the development/course of other JIA forms
RNA-containing immune complexes (IC) against citrullinated peptide
antigens (collagen, fibrinogen) induce netosis
B cell activation perpetuates IC formation
Downstream ↑ Syk signaling, esp. with certain genetic mutations (e.g.
Zap-70, low affinity Fc receptor)
S. Vastert, B. Prakken 232 / Best Practice & Research Clinical Rheumatology 28 (2014)
Chauhan AK, Moore TL, Bi Y, Chen C. J Biol Chem (2016) 291:1368–86.
14
16. Window of Opportunity – Basic
Science Evidence
High IL-1, IL6, TNF levels facilitate differentiation of
pathogenic Th17 cells
Esp. with genetic (environment) predisposition
Shift to chronicity of Th17 response with sJIA (and other
forms of JIA)
Th17 cell pool expands with arthritis progression
Th17 depleted arthritis mice do not get joint diseases
Peter Nigrovic; ARTHRITIS & RHEUMATOLOGY; Vol. 66, No. 6, June 2014, pp 1405–1413;
S. Vastert, B. Prakken 232 / Best Practice & Research Clinical Rheumatology 28 (2014) 229-246
16
17. Prognosis of SJIA
has markedly
improved with the
approval of IL1 and
IL6 blocking
medications
17
19. SJIA Poor prognostic Clinical Indicators
Early age at onset
Cumulative active disease
Platelets > 600K at 6 months,
Hip involvement by 6 months
Generalized lymphadenopathy
Toronto Cohort
19
20. In the past: progressive joint destruction in approximately 1/3 of the
children
More common in patients with polyarthritis
Ankylosis in cervical spine, carpal &tarsal areas
Joint space narrowing & erosions
Apophyseal fusion of
C2-C4 and undergrowth
of adjacent vertebrae
20
21. Growth Delay
Generalized - Localized
Intensified by corticosteroid therapy
Likely due to IL-6 effects
Growth hormone therapy possible
in some patients
Need to control inflammation
22
22. Mortality
In SJIA mostly due to disease
complications:
Infection
MAS
Myocarditis
Amyloidosis
Systemic
64%
Poly
18%
Pauci
12%
Other
6%
23
23. Amyloidosis
Very rare in North America
Biopsy required for diagnosis
Deposited in kidneys, liver, gastro-
intestinal tract, heart, peripheral nerves
Proteinuria, diarrhea, hepato-
splenomegaly, unexplained anemia
Amyloid A deposition in kidneys detected by
staining with Congo Red dye
24