This presentation details Osteogenic Imperfecta in its varying clinical manifestations in the population and offers a variety of adjunctive treatments not commonly used in OI management across the lifespan in order to decrease fracture, pain, and disability.
Description : Osteogenesis Imperfecta/
Brittle bone disease :
It is disorder of type I collagen synthesis that affects all connective tissue in the body.
Musculoskeletal involvement is diffuse and includes osteoporosis with excessive fracture even at birth, bowing of long bone, spinal deformities, muscle weakness and ligamentous laxity.
Key words :
Osteogenesis Imperfecta, Brittle bone disease, Genetic disorder, Pathophysiology, Types of OI, Denetinogenesis Imperfecta, Bluish sclera, Frequent fractures, fractures, Hearing loss, Management, orthopedic, Rehabilitation
Physiotherapy, pediatrics, physiotherapist, pediatric orthopedic surgery.
This presentation includes four major topics:
1- reviews the essentials of osteoporosis including definition, pathophysiology, etiology, epidemiology, and prognosis
2- talks about the presentation of osteoporosis, including risk factors, symptoms and signs, radiologic manifestations, and complications
3- reviews the workup process to diagnose and define the severity of osteoporosis, including the lab. and radiologic procedures
4- reviews management tools of osteoporosis, including pharmacologic and non pharmacologic methods, with brief description for each pharmacologic or non pharmacologic tool.
Finally, some statements about the education and prevention of osteoporosis.
This presentation details Osteogenic Imperfecta in its varying clinical manifestations in the population and offers a variety of adjunctive treatments not commonly used in OI management across the lifespan in order to decrease fracture, pain, and disability.
Description : Osteogenesis Imperfecta/
Brittle bone disease :
It is disorder of type I collagen synthesis that affects all connective tissue in the body.
Musculoskeletal involvement is diffuse and includes osteoporosis with excessive fracture even at birth, bowing of long bone, spinal deformities, muscle weakness and ligamentous laxity.
Key words :
Osteogenesis Imperfecta, Brittle bone disease, Genetic disorder, Pathophysiology, Types of OI, Denetinogenesis Imperfecta, Bluish sclera, Frequent fractures, fractures, Hearing loss, Management, orthopedic, Rehabilitation
Physiotherapy, pediatrics, physiotherapist, pediatric orthopedic surgery.
This presentation includes four major topics:
1- reviews the essentials of osteoporosis including definition, pathophysiology, etiology, epidemiology, and prognosis
2- talks about the presentation of osteoporosis, including risk factors, symptoms and signs, radiologic manifestations, and complications
3- reviews the workup process to diagnose and define the severity of osteoporosis, including the lab. and radiologic procedures
4- reviews management tools of osteoporosis, including pharmacologic and non pharmacologic methods, with brief description for each pharmacologic or non pharmacologic tool.
Finally, some statements about the education and prevention of osteoporosis.
Paget’s disease of bone is a condition characterized by abnormal and anarchic resorption and deposition of bone, resulting in distortion and weakening of the affected bones
2024.06.01 Introducing a competency framework for languag learning materials ...Sandy Millin
http://sandymillin.wordpress.com/iateflwebinar2024
Published classroom materials form the basis of syllabuses, drive teacher professional development, and have a potentially huge influence on learners, teachers and education systems. All teachers also create their own materials, whether a few sentences on a blackboard, a highly-structured fully-realised online course, or anything in between. Despite this, the knowledge and skills needed to create effective language learning materials are rarely part of teacher training, and are mostly learnt by trial and error.
Knowledge and skills frameworks, generally called competency frameworks, for ELT teachers, trainers and managers have existed for a few years now. However, until I created one for my MA dissertation, there wasn’t one drawing together what we need to know and do to be able to effectively produce language learning materials.
This webinar will introduce you to my framework, highlighting the key competencies I identified from my research. It will also show how anybody involved in language teaching (any language, not just English!), teacher training, managing schools or developing language learning materials can benefit from using the framework.
The French Revolution, which began in 1789, was a period of radical social and political upheaval in France. It marked the decline of absolute monarchies, the rise of secular and democratic republics, and the eventual rise of Napoleon Bonaparte. This revolutionary period is crucial in understanding the transition from feudalism to modernity in Europe.
For more information, visit-www.vavaclasses.com
Honest Reviews of Tim Han LMA Course Program.pptxtimhan337
Personal development courses are widely available today, with each one promising life-changing outcomes. Tim Han’s Life Mastery Achievers (LMA) Course has drawn a lot of interest. In addition to offering my frank assessment of Success Insider’s LMA Course, this piece examines the course’s effects via a variety of Tim Han LMA course reviews and Success Insider comments.
June 3, 2024 Anti-Semitism Letter Sent to MIT President Kornbluth and MIT Cor...Levi Shapiro
Letter from the Congress of the United States regarding Anti-Semitism sent June 3rd to MIT President Sally Kornbluth, MIT Corp Chair, Mark Gorenberg
Dear Dr. Kornbluth and Mr. Gorenberg,
The US House of Representatives is deeply concerned by ongoing and pervasive acts of antisemitic
harassment and intimidation at the Massachusetts Institute of Technology (MIT). Failing to act decisively to ensure a safe learning environment for all students would be a grave dereliction of your responsibilities as President of MIT and Chair of the MIT Corporation.
This Congress will not stand idly by and allow an environment hostile to Jewish students to persist. The House believes that your institution is in violation of Title VI of the Civil Rights Act, and the inability or
unwillingness to rectify this violation through action requires accountability.
Postsecondary education is a unique opportunity for students to learn and have their ideas and beliefs challenged. However, universities receiving hundreds of millions of federal funds annually have denied
students that opportunity and have been hijacked to become venues for the promotion of terrorism, antisemitic harassment and intimidation, unlawful encampments, and in some cases, assaults and riots.
The House of Representatives will not countenance the use of federal funds to indoctrinate students into hateful, antisemitic, anti-American supporters of terrorism. Investigations into campus antisemitism by the Committee on Education and the Workforce and the Committee on Ways and Means have been expanded into a Congress-wide probe across all relevant jurisdictions to address this national crisis. The undersigned Committees will conduct oversight into the use of federal funds at MIT and its learning environment under authorities granted to each Committee.
• The Committee on Education and the Workforce has been investigating your institution since December 7, 2023. The Committee has broad jurisdiction over postsecondary education, including its compliance with Title VI of the Civil Rights Act, campus safety concerns over disruptions to the learning environment, and the awarding of federal student aid under the Higher Education Act.
• The Committee on Oversight and Accountability is investigating the sources of funding and other support flowing to groups espousing pro-Hamas propaganda and engaged in antisemitic harassment and intimidation of students. The Committee on Oversight and Accountability is the principal oversight committee of the US House of Representatives and has broad authority to investigate “any matter” at “any time” under House Rule X.
• The Committee on Ways and Means has been investigating several universities since November 15, 2023, when the Committee held a hearing entitled From Ivory Towers to Dark Corners: Investigating the Nexus Between Antisemitism, Tax-Exempt Universities, and Terror Financing. The Committee followed the hearing with letters to those institutions on January 10, 202
Model Attribute Check Company Auto PropertyCeline George
In Odoo, the multi-company feature allows you to manage multiple companies within a single Odoo database instance. Each company can have its own configurations while still sharing common resources such as products, customers, and suppliers.
The Roman Empire A Historical Colossus.pdfkaushalkr1407
The Roman Empire, a vast and enduring power, stands as one of history's most remarkable civilizations, leaving an indelible imprint on the world. It emerged from the Roman Republic, transitioning into an imperial powerhouse under the leadership of Augustus Caesar in 27 BCE. This transformation marked the beginning of an era defined by unprecedented territorial expansion, architectural marvels, and profound cultural influence.
The empire's roots lie in the city of Rome, founded, according to legend, by Romulus in 753 BCE. Over centuries, Rome evolved from a small settlement to a formidable republic, characterized by a complex political system with elected officials and checks on power. However, internal strife, class conflicts, and military ambitions paved the way for the end of the Republic. Julius Caesar’s dictatorship and subsequent assassination in 44 BCE created a power vacuum, leading to a civil war. Octavian, later Augustus, emerged victorious, heralding the Roman Empire’s birth.
Under Augustus, the empire experienced the Pax Romana, a 200-year period of relative peace and stability. Augustus reformed the military, established efficient administrative systems, and initiated grand construction projects. The empire's borders expanded, encompassing territories from Britain to Egypt and from Spain to the Euphrates. Roman legions, renowned for their discipline and engineering prowess, secured and maintained these vast territories, building roads, fortifications, and cities that facilitated control and integration.
The Roman Empire’s society was hierarchical, with a rigid class system. At the top were the patricians, wealthy elites who held significant political power. Below them were the plebeians, free citizens with limited political influence, and the vast numbers of slaves who formed the backbone of the economy. The family unit was central, governed by the paterfamilias, the male head who held absolute authority.
Culturally, the Romans were eclectic, absorbing and adapting elements from the civilizations they encountered, particularly the Greeks. Roman art, literature, and philosophy reflected this synthesis, creating a rich cultural tapestry. Latin, the Roman language, became the lingua franca of the Western world, influencing numerous modern languages.
Roman architecture and engineering achievements were monumental. They perfected the arch, vault, and dome, constructing enduring structures like the Colosseum, Pantheon, and aqueducts. These engineering marvels not only showcased Roman ingenuity but also served practical purposes, from public entertainment to water supply.
Unit 8 - Information and Communication Technology (Paper I).pdfThiyagu K
This slides describes the basic concepts of ICT, basics of Email, Emerging Technology and Digital Initiatives in Education. This presentations aligns with the UGC Paper I syllabus.
A Strategic Approach: GenAI in EducationPeter Windle
Artificial Intelligence (AI) technologies such as Generative AI, Image Generators and Large Language Models have had a dramatic impact on teaching, learning and assessment over the past 18 months. The most immediate threat AI posed was to Academic Integrity with Higher Education Institutes (HEIs) focusing their efforts on combating the use of GenAI in assessment. Guidelines were developed for staff and students, policies put in place too. Innovative educators have forged paths in the use of Generative AI for teaching, learning and assessments leading to pockets of transformation springing up across HEIs, often with little or no top-down guidance, support or direction.
This Gasta posits a strategic approach to integrating AI into HEIs to prepare staff, students and the curriculum for an evolving world and workplace. We will highlight the advantages of working with these technologies beyond the realm of teaching, learning and assessment by considering prompt engineering skills, industry impact, curriculum changes, and the need for staff upskilling. In contrast, not engaging strategically with Generative AI poses risks, including falling behind peers, missed opportunities and failing to ensure our graduates remain employable. The rapid evolution of AI technologies necessitates a proactive and strategic approach if we are to remain relevant.
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This presentation provides a briefing on how to upload submissions and documents in Google Classroom. It was prepared as part of an orientation for new Sainik School in-service teacher trainees. As a training officer, my goal is to ensure that you are comfortable and proficient with this essential tool for managing assignments and fostering student engagement.
2. Osteogenesis Imperfacta
Hereditary condition resulting from a decrease
in the amount of normal Type I collagen
Type I collagen ( important for )
Bone
Ligaments
Teeth
White Sclera
Skin
3. Type I collagen deficiency can result from
decreased collagen secretion
production of abnormal collagen
Manifest by
increase bone fragility
low bone mass ( Osteopenia )
Both Autosomal dominant and Autosomal recessive forms
Can be severe or mild (Tarda)
Osteogenesis Imperfecta
4. Orthopaedic manifestations
Bone fragility and fractures
fractures heal in normal fashion initially
but the bone is does not remodel
can lead to progressive bowing
ligamentous laxity
Short stature
Scoliosis
Codfish vertebrae (compressionfx)
Basilar invagination
Olecranon apophyseal avulsion fx
5. Non-Orthopaedic manifestations
Blue sclera
Hearing loss
lessfrequentthangenerallysuspected
Dentinogenesis imperfecta
brownishopalescentteeth
Wormian skull bones
(puzzlepieceintrasuturalskullbones)
6. Symptoms
Mild cases
multiple fractures during childhood
Severe cases
present with fractures at birth and can be fatal
Number of fractures typically decreases as patient ages and
usually stops after puberty
But deformity persist.
Basilar invagination
Brain Stem dysfunction
apnea, altered consciousness, ataxia, or myelopathy
usually in third or fourth decade of life, but can be as early as teenage
years
8. Sillence Classification of Osteogenes Imperfecta
(simplified)
Type I
Mildest form. Presents at preschool age (Tarda).
Autosomal dominant
blue sclera
Hearing deficit in 50%.
Divided into type A and B based on tooth involvement
Type II
Autosomal recessive
Blue sclera
Lethal in perinatal period
9. Type III
Autosomal recessive
Normal sclerea
Fractures at birth.
Progressively short stature.
Most severe survivable form
Type IV
Autosomal dominant normal
Moderate severity.
Bowing bones and vertebral fractures are common.
Hearing normal.
Divided into type A and B based on tooth involvement
10. Type V
Hypertrophic callus after fracture.
Ossification of IOM ( radius/ulna and tibia/fibula )
Type VI
Moderate severity. Similar to type IV
Type VII
Associated with rhizomelia and coxa vara
Type V, VI, VII
Added to the original classification system .
No Type I collagen mutation
But have abnormal bone on microscopy and a similar phenotype
11. Radiographs
Thin cortices
Generalized osteopenia
Long bone thin and bowed
Pelvis may show acetabular protrusion
Fractures that are at different stages of healing
The vertebra maybe biconcave.
12. Diagnosis
Diagnosis is based on family history associated
with typical radiographic and clinical features
No commercially available diagnostic test(varietyofgeneticmutations)
laboratory values are typically within normal range
Possible methods include
Fibroblast culturing to analyze type I collagen (positivein80%of
typeIV) can be used for confirmation of diagnosis in equivocal
cases
Collagen analysis of a punch biopsy
Iliac crest biopsy which shows a decrease in cortical widths and
cancellous bone volume, with increased bone remodeling.
14. Treatment of Fractures
Fracture prevention
Early bracing
Decrease deformity.
Stabilize lax joints.
Decrease fractures incidence.
Bisphosphonates
Growth hormone
Clinical studies showed no increase in bone mass
Bone marrow transplantation
15. Bisphosphonates
Prevent bone mass loss and decrease bone resorption by suppressing the activity of
osteoclasts.
Indications
Osteoporosis
Metastatic bone disease
Multiple myeloma
Paget's disease
Polyostotic fibrous dysplasia
Total joint arthroplasty to prevent osteolysis
Early stage avascular necrosis
Osteogenesis imperfecta
Contraindications
Severe renal disease
Lumbar fusion decreased spinal fusion rates
Hypersensitivity.
Pregnancy.
Side Effects & Complications
Jaw osteonecrosis
Atypical subtrochanteric and femoral stress fractures
Radiographic changes consistent with osteopetrosis
Bisphosphonates in O I
Indicated in most cases of OI to reduces fracture rate and
pain
Combined with calcium and vitamin D
Increase cortical thickness by inhibiting osteoclasts
Does not affect development of scoliosis
Treatment is less effective after completion of growth.
16. Pamidronate
Injectable bisphosphonate
(Cyclic Intravenous )
Increases cortical bone thickness
Increase bone mass and density.
Decreases the incidence of fractures.
Relieves chronic bone pain.
Increases activity levels.
Decreases the reliance on mobility aids.
Increases the height of the collapsed
vertebral bodies.
BUT
Notdecreasetheincidenceofscoliosis.
Zebralines
Radiographically Pamidronate therapy creates growth lines in the bone
17. Bone marrow transplantation
Used with some success
Introduces normal marrow stem cells that could
potentially differentiate into normal osteoblasts,
Problems of graft rejection and graft versus host
reactions limit this approach.
18. Fracture treatment
Nonoperative
child is less than 2 years
treataschildwithoutOI
Operative
Fixation with Telescoping rodes
patients > 2 years
allow continued growth
19. Treatment of Long Bone Deformities
Realignment Osteotomy with rod fixation
(Sofield-Miller procedure)
Indicated in severe deformity to
Correct the deformity
Reduce fracture rates
Techniques include
Nontelescopic devices
Telescopic devices
20. Treatment of Scoliosis
Observation
Curve less than 45 °
Bracing is ineffective
Operative
posterior spinal fusion
Indications
for curves > 45 ° in mild forms and
> 35 ° in severe forms
Technique
Challenging due to fragility of bones
Use allograft instead of iliac crest autograft
Large blood loss
Prolapse of the upper cervical spine in to the base of skull3 rd and 4 th decade but maybe earlyBrain Stem Dysfunction
Genatic analysis of collagen from dermal fibroblast identify abnormality in type I collagen
Bisphosphonates decrease the resorption of bone by suppressing the activity of osteoclasts.human growth hormone has been used in the past because of its anabolic effects on bone; however, clinical studies showed no increase in bone mass or change in natural historyexercise as much as possible to promote muscle and bone strength, which can help prevent fractures
severe renal disease primary mode of excretion is renalfollowing lumbar fusion decreased spinal fusion rates in lab animal models (increased fusion mass size, but decreases the actual fusion rate)Treatment is less effective after completion of growth
These radiodense areas of bone probably represent the inhibition of osteoclastic resorption,whereas the clear areas between the lines represent the interval growth between treatment cycles.Radiographic zebra lines are a manifestation of the administration of cyclic bisphosphonate therapy in children before closure of the epiphyseal growth platesCyclical intravenouspamidronate administration reduces bone pain, and increases bone mass and density
ASF only indicated in very young children to prevent crankshaft
