This document summarizes information about haemophilia, including:
- It is a genetic bleeding disorder caused by deficiencies in coagulation factors VIII or IX.
- It leads to recurrent bleeding episodes into joints and muscles.
- Repeated bleeding can cause chronic joint damage and deformities over time.
- Treatment involves replacing the deficient clotting factor, managing pain, and physical therapy to prevent joint damage. Early intervention and splinting can help correct deformities and maintain range of motion.
Charcot joint or neuropathic joint are destructed joint occurs in Diabetes, syphilis, syringomyelia , leprosy, AMLS, Peripheral neuropathy and any condition leads to impair sensation of peripheral part of body
Charcot joint or neuropathic joint are destructed joint occurs in Diabetes, syphilis, syringomyelia , leprosy, AMLS, Peripheral neuropathy and any condition leads to impair sensation of peripheral part of body
This presentation deals with the causes, signs, symptoms and management of compartment syndrome. It also briefly deals with abdominal comaprtment syndrome
This is short Presentation on avascular necrosis of femoral head.This presentation gives brief description of introduction, causes investigation and treatment for AVN of hip.
Post Polio Residual Palsy: Pathophysiology & Principles of RxAnisuddin Bhatti
Prof. Anisuddin Bhatti, Paeds Orthop Surgeon delivered lecture on Post Polio paralysis and deformities Part 1 on Pathophysio and principles of treatment, through Dr. Ziauddin University Hospital Clifton Karachi webinar on googel.meet, on 3rd April 2021. Acknowledge for material taken from Research papers, slideshare and books as referred in reference list.
PHYSIOTHERAPY IN COMMON VASCULAR CONDITIONS.pptxKunjalPardeshi1
Vascular disease includes any condition that affects your circulatory system, or system of blood vessels. This ranges from diseases of your arteries, veins and lymph vessels to blood disorders that affect circulation.
Blood vessels are elastic-like tubes that carry blood to every part of your body. Blood vessels include:
Arteries that carry blood away from your heart.
Veins that return blood back to your heart.
Capillaries, your tiniest blood vessels, which link your small veins and arteries, deliver oxygen and nutrients to your tissues and take away their waste.
Spinal stenosis is a degenrative spine disorder in which the AP and transverse diameter are decreased causing neural compression and symptoms of chronic & acute nerve compression
Patients with spinal cord injury face a number of challenges, with continence being a top priority. For those affected by neurogenic bladder and bowel, there are various management options available. To help understand these options, study notes in this area can be useful. These notes, which are similar to index cards, can highlight key information related to the management of neurogenic bladder and bowel in spinal cord injury patients.
presentation about relation between posture and pain. there is lot of talk and research regarding bad posture and chronic pain. but posture, disease along with physical activity intervention should be done to manage.
Cancer Rehabilitation. integrating rehabilitation with oncology. a model of care. cancer survivorship. rehabilitation care in low resource area. Mrinal Joshi. Rehabilitation Research Center. Jaipur.
Title: Sense of Smell
Presenter: Dr. Faiza, Assistant Professor of Physiology
Qualifications:
MBBS (Best Graduate, AIMC Lahore)
FCPS Physiology
ICMT, CHPE, DHPE (STMU)
MPH (GC University, Faisalabad)
MBA (Virtual University of Pakistan)
Learning Objectives:
Describe the primary categories of smells and the concept of odor blindness.
Explain the structure and location of the olfactory membrane and mucosa, including the types and roles of cells involved in olfaction.
Describe the pathway and mechanisms of olfactory signal transmission from the olfactory receptors to the brain.
Illustrate the biochemical cascade triggered by odorant binding to olfactory receptors, including the role of G-proteins and second messengers in generating an action potential.
Identify different types of olfactory disorders such as anosmia, hyposmia, hyperosmia, and dysosmia, including their potential causes.
Key Topics:
Olfactory Genes:
3% of the human genome accounts for olfactory genes.
400 genes for odorant receptors.
Olfactory Membrane:
Located in the superior part of the nasal cavity.
Medially: Folds downward along the superior septum.
Laterally: Folds over the superior turbinate and upper surface of the middle turbinate.
Total surface area: 5-10 square centimeters.
Olfactory Mucosa:
Olfactory Cells: Bipolar nerve cells derived from the CNS (100 million), with 4-25 olfactory cilia per cell.
Sustentacular Cells: Produce mucus and maintain ionic and molecular environment.
Basal Cells: Replace worn-out olfactory cells with an average lifespan of 1-2 months.
Bowman’s Gland: Secretes mucus.
Stimulation of Olfactory Cells:
Odorant dissolves in mucus and attaches to receptors on olfactory cilia.
Involves a cascade effect through G-proteins and second messengers, leading to depolarization and action potential generation in the olfactory nerve.
Quality of a Good Odorant:
Small (3-20 Carbon atoms), volatile, water-soluble, and lipid-soluble.
Facilitated by odorant-binding proteins in mucus.
Membrane Potential and Action Potential:
Resting membrane potential: -55mV.
Action potential frequency in the olfactory nerve increases with odorant strength.
Adaptation Towards the Sense of Smell:
Rapid adaptation within the first second, with further slow adaptation.
Psychological adaptation greater than receptor adaptation, involving feedback inhibition from the central nervous system.
Primary Sensations of Smell:
Camphoraceous, Musky, Floral, Pepperminty, Ethereal, Pungent, Putrid.
Odor Detection Threshold:
Examples: Hydrogen sulfide (0.0005 ppm), Methyl-mercaptan (0.002 ppm).
Some toxic substances are odorless at lethal concentrations.
Characteristics of Smell:
Odor blindness for single substances due to lack of appropriate receptor protein.
Behavioral and emotional influences of smell.
Transmission of Olfactory Signals:
From olfactory cells to glomeruli in the olfactory bulb, involving lateral inhibition.
Primitive, less old, and new olfactory systems with different path
Ozempic: Preoperative Management of Patients on GLP-1 Receptor Agonists Saeid Safari
Preoperative Management of Patients on GLP-1 Receptor Agonists like Ozempic and Semiglutide
ASA GUIDELINE
NYSORA Guideline
2 Case Reports of Gastric Ultrasound
ARTIFICIAL INTELLIGENCE IN HEALTHCARE.pdfAnujkumaranit
Artificial intelligence (AI) refers to the simulation of human intelligence processes by machines, especially computer systems. It encompasses tasks such as learning, reasoning, problem-solving, perception, and language understanding. AI technologies are revolutionizing various fields, from healthcare to finance, by enabling machines to perform tasks that typically require human intelligence.
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MANAGEMENT OF ATRIOVENTRICULAR CONDUCTION BLOCK.pdfJim Jacob Roy
Cardiac conduction defects can occur due to various causes.
Atrioventricular conduction blocks ( AV blocks ) are classified into 3 types.
This document describes the acute management of AV block.
Lung Cancer: Artificial Intelligence, Synergetics, Complex System Analysis, S...Oleg Kshivets
RESULTS: Overall life span (LS) was 2252.1±1742.5 days and cumulative 5-year survival (5YS) reached 73.2%, 10 years – 64.8%, 20 years – 42.5%. 513 LCP lived more than 5 years (LS=3124.6±1525.6 days), 148 LCP – more than 10 years (LS=5054.4±1504.1 days).199 LCP died because of LC (LS=562.7±374.5 days). 5YS of LCP after bi/lobectomies was significantly superior in comparison with LCP after pneumonectomies (78.1% vs.63.7%, P=0.00001 by log-rank test). AT significantly improved 5YS (66.3% vs. 34.8%) (P=0.00000 by log-rank test) only for LCP with N1-2. Cox modeling displayed that 5YS of LCP significantly depended on: phase transition (PT) early-invasive LC in terms of synergetics, PT N0—N12, cell ratio factors (ratio between cancer cells- CC and blood cells subpopulations), G1-3, histology, glucose, AT, blood cell circuit, prothrombin index, heparin tolerance, recalcification time (P=0.000-0.038). Neural networks, genetic algorithm selection and bootstrap simulation revealed relationships between 5YS and PT early-invasive LC (rank=1), PT N0—N12 (rank=2), thrombocytes/CC (3), erythrocytes/CC (4), eosinophils/CC (5), healthy cells/CC (6), lymphocytes/CC (7), segmented neutrophils/CC (8), stick neutrophils/CC (9), monocytes/CC (10); leucocytes/CC (11). Correct prediction of 5YS was 100% by neural networks computing (area under ROC curve=1.0; error=0.0).
CONCLUSIONS: 5YS of LCP after radical procedures significantly depended on: 1) PT early-invasive cancer; 2) PT N0--N12; 3) cell ratio factors; 4) blood cell circuit; 5) biochemical factors; 6) hemostasis system; 7) AT; 8) LC characteristics; 9) LC cell dynamics; 10) surgery type: lobectomy/pneumonectomy; 11) anthropometric data. Optimal diagnosis and treatment strategies for LC are: 1) screening and early detection of LC; 2) availability of experienced thoracic surgeons because of complexity of radical procedures; 3) aggressive en block surgery and adequate lymph node dissection for completeness; 4) precise prediction; 5) adjuvant chemoimmunoradiotherapy for LCP with unfavorable prognosis.
Flu Vaccine Alert in Bangalore Karnatakaaddon Scans
As flu season approaches, health officials in Bangalore, Karnataka, are urging residents to get their flu vaccinations. The seasonal flu, while common, can lead to severe health complications, particularly for vulnerable populations such as young children, the elderly, and those with underlying health conditions.
Dr. Vidisha Kumari, a leading epidemiologist in Bangalore, emphasizes the importance of getting vaccinated. "The flu vaccine is our best defense against the influenza virus. It not only protects individuals but also helps prevent the spread of the virus in our communities," he says.
This year, the flu season is expected to coincide with a potential increase in other respiratory illnesses. The Karnataka Health Department has launched an awareness campaign highlighting the significance of flu vaccinations. They have set up multiple vaccination centers across Bangalore, making it convenient for residents to receive their shots.
To encourage widespread vaccination, the government is also collaborating with local schools, workplaces, and community centers to facilitate vaccination drives. Special attention is being given to ensuring that the vaccine is accessible to all, including marginalized communities who may have limited access to healthcare.
Residents are reminded that the flu vaccine is safe and effective. Common side effects are mild and may include soreness at the injection site, mild fever, or muscle aches. These side effects are generally short-lived and far less severe than the flu itself.
Healthcare providers are also stressing the importance of continuing COVID-19 precautions. Wearing masks, practicing good hand hygiene, and maintaining social distancing are still crucial, especially in crowded places.
Protect yourself and your loved ones by getting vaccinated. Together, we can help keep Bangalore healthy and safe this flu season. For more information on vaccination centers and schedules, residents can visit the Karnataka Health Department’s official website or follow their social media pages.
Stay informed, stay safe, and get your flu shot today!
2. • Coagulation disorders represent disruption of the body's
ability to control bleeding
• Genetic deficiencies of plasma coagulation factors - life-
long recurrent bleeding episodes
• Von Willebrand disease (VWD) - most common type of
bleeding disorder
• Most common inherited factor deficiencies - Hemophilias
3. • Deficiency of factor VIII (hemophilia A) or factor IX
(hemophilia B) – XR (1/3 cases – spontaneous mutation)
• Annual incidence 1/5000 & 1/30000, male births of hemophilia
A & B
• FII, FV, FVII, FX, FXI, FXIII, and fibrinogen –AR
• Hemophilics do not bleed any faster, but for a longer time
• 500 different mutations identified in F8 or F9 genes
• Inversion of intron 22 sequence, MC mutation
• Present in 40% of cases of severe hemophilia A
5. Possible
diagnosis
PT aPTT BT Platelet
count
Normal N N N N
Haemophila
A/B
N Inc N N
VWD N N or Inc N or Inc N or Dec
Platelet defect N N N or Inc N or Dec
DIAGNOSIS
Screening
test
Definitive
diagnosis
Factor assay
Severity Factor level Bleeding episode
Severe < 1 IU/dl (< 0.01 IU/ml)
or
< 1 % of normal
Spontaneous bleeding in
muscles and joints
Moderate 1-5 IU/dl (0.01-0.05
IU/ml) or
1-5% of normal
Occasional spontaneous
bleeding
Mild 5-40 IU/dl (0.05-0.40
IU/ml) or
5-<40% of normal
spontaneous bleeding rare,
severe bleed with major
trauma
6. Mother is a carrier Father has hemophilia
Mother is a carrier AND
Father has hemophilia
8. ACUTE HEMARTHROSES
• Generally first occur when child begins to walk
• Continue, usually cyclically, into adulthood, when frequency diminishes
• Intense pain due to rapid joint capsule distension
• Accompanied by objective clinical findings of
• Warmth
• Tense effusion
• Tenderness
• Limitation of motion
• Joint often held in flexed position
9. ACUTE HEMARTHROSES
• Joint pain responds rapidly to replacement
of deficient clotting factor
• Hemostasis
• achieved early
• full joint function may be regained
within 12 to 24 hours
• Hemorrhage - more advanced
• blood is resorbed slowly over 5 to 7
days
• full joint function is regained within 10
to 14 days
10. Muscle and soft tissue hemorrhage
• More insidious than hemarthrosis
• Lack of premonitory symptoms
• Iliopsoas hemorrhage
• Acute groin pain
• Marked pain on hip extension
• Hip flexion contracture
• Rotation is preserved, in contrast to
intra-articular hemorrhage
11. Muscle and soft tissue hemorrhage
• If untreated, mass may compress femoral nerve - signs and
symptoms of neuropathy
• Bleeding into gastrocnemius - equinus deformity from heel
cord contracture
• Hemorrhage into closed compartments - acute muscle necrosis
and nerve compression
• Of particular importance - volar compartment of forearm,
which can cause flexion contracture
12. ABNORMAL POSTURE FOLLOWING BLEEDS
Joint bleeds Position of comfort
Knee Flexion
Elbow Flexion
Ankle Planter flexion
Hip Flexion, ER
Shoulder Adduction, IR
Wrist and fingers Flexion
Toes Dorsi flexion
Muscle bleeds Position of
comfort
Hamstrings Knee Flexion, Hip
extension
Biceps brachii Elbow flexion,
Shoulder IR
Gastrocnemius Planter flexion,
Knee flexion
Iliopsoas HF, some ER &
increased lumbar
lordosis
Wrist and finger
flexors
Wrist and finger
flexion, elbow
flexion
Quadriceps Knee extension
Hip extensors Hip extension
13. MANAGEMENT - ACUTE BLEEDING
• Treated preferably in 2 hours- If in doubt, treat
• RICE- therapy
Factor VIII concentrates
1 IU/kg increases plasma level by
2 IU /dl
Dosage = Wt.(kg) x desired
factor level x 0.5
Factor IX concentrates
1 IU/kg increases plasma level by 1
IU /dl
Dosage = Wt.(kg) x desired
factor level
14. MANAGEMENT...
• Fresh Frozen Plasma (FFP) – all coagulation factors
• 1 ml FFP contains 1 unit of factor activity
• Starting dose is 15-20 ml/kg
• Cryoprecipitate- contains F VIII( 3-5 IU/ml), VWF but not F IX
and F XI
• Contain 70-80 units of FVIII in 30-40 ml
15. MANAGEMENT...
• Desmopressin- synthetic analog of vasopressin – increases plasma
level of F VIII & VWF
• In mild and moderate hemophilia
• Not used in pregnancy
• Route & Dosage – sc, iv, nasal spray
• 0.3 microgram/kg increases F VIII level by 3-6 times
16. Hemophilia A Hemophilia B
Type of
hemorrhage
Desired level Duration (days) Desired level Duration (days)
Joint 40-60 1-2 40-60 1-2
Superficial
muscle/ no NV
compromise
40-60 2-3 40-60 2-3
Iliopsoas and deep
muscle with NV
injury
Initial 80-100 1-2 60-80 1-2
Maintenence 30-60 3-5 30-60 3-5
CNS/GI
Initial 80-100 1-7 60-80 1-7
Maintenence 50 8-21 30 8-21
17. SUBACUTE OR CHRONIC ARTHRITIS
• Recurrent hemarthroses, may lead to a self-perpetuating condition
• Joint abnormalities persist in intervals between bleeding episodes
• Involved joint is chronically swollen although painless and only
slightly warm
• Chronic synovitis, including prominent synovial proliferation with
or without effusion, may be present
• Mild limitation of motion often with a flexion deformity
• Factor replacement does not modify these findings
18. END-STAGE HEMOPHILIC ARTHROPATHY
• Features in common with DJD and advanced RA
• Joint appears enlarged and “knobby” - osteophytic bone overgrowth
• Synovial thickening and effusion - not prominent
• Range of motion is severely restricted
• Fibrous ankylosis is common
• Subluxation, joint laxity, and malalignment - frequently present
• Hemarthroses decreases in frequency
19. HAEMOPHILIC ARTHROPATHY
• Joint that has had recurrent
bleeding episodes -“target
joint”
• 4 bleeds into the same joint
in a 6-month time period
• Most common are - knees,
ankles, and elbows
21. RADIOLOGIC MANIFESTATIONS OF
CHRONIC HEMOPHILIC ARTHROPATHY
Characteristic ALSO SEEN IN
Periarticular soft tissue swelling RA
Periarticular demineralization RA
Marginal erosions RA
Subchondral irregularity and cyst formation RA, OA
Decreased joint space OA
Osteophyte formation OA
Chondrocalcinosis CPPD
23. ARNOLD HILL GARTNER STAGING- XRAY
• Stage 0: normal joint
• Stage I: no skeletal abnormalities, soft-tissue swelling is present
• Stage II: osteoporosis and overgrowth of the epiphysis, no cysts, no narrowing of the cartilage
space
• Stage III: early subchondral bone cysts, squaring of the patella, widened notch of the distal femur
or humerus, preservation of the cartilage space
24. ARNOLD HILL GARTNER STAGING- XRAY
• Stage IV: findings of stage III, but more advanced; narrowed cartilage space
• Stage V: fibrous joint contractures, loss of the joint cartilage space, extensive
enlargement of the epiphyses with substantial disorganization of the joint
26. PAIN MANAGEMENT
• Patients with chronic hemophilic arthropathy
• difficulty distinguishing arthritic pain from acute pain of
hemarthrosis
• No relief of pain following aggressive factor replacement,
• pain may be related to arthritic changes
• NSAIDs controversial as they inhibit platelet function
• COX-2 inhibitors - safer
• More debilitating cases - narcotics may be needed
28. EARLY INTERVENTION - PHYSICAL THERAPY
• Integral part of hemophilia team
• Helpful in management of target joints or those with hemophilic
arthropathy
• Exercise is important to counteract the long-term effects of bleeding in
the key joints and muscle groups
• Exercises progress from simple to more difficult levels
29. PHYSICAL THERAPY -PRINCIPALS
• Slow and steady
• Start slowly and progress gradually
• Isometric exercises
• contracting a muscle without producing movement at the joint
• excellent if muscles are very weak, or if joints are very painful with
movement
• No gain with pain
• if new pain begins during exercising - analysed carefully
• Is this muscle fatigue, or the sign of a new bleed?
• exercise placing too much strain on a damaged joint
30. EXERCISE THERAPY- PRINCIPALS
• Each situation is unique
• number of exercises and repetitions depends on the situation of each individual
• after a bleed, two or three repetitions may be all that can be tolerated
• also depend on which type of muscle is being exercised
• Stabilizer muscles should be trained with low resistance but many repetitions
• Use gravity and body weight as resistance to start
• Think about the other joints
• weight-bearing exercises to strengthen knee may be difficult and ill advised if the
ankle on the same leg is damaged
31. PHYSICAL THERAPY- PRINCIPALS
• Remember functional requirements
• Don’t forget proprioception
• Normal range of motion and muscle length
• Everyday activities are important too
• Everyday activities, such as walking, riding a bicycle, climbing stairs, or can have
beneficial effects too
• Swimming and non contact sports are advised
32. EARLY INTERVENTION - SPLINTING, ORTHOTICS,
AND ASSISTIVE DEVICES
Splints
• To immobilize a bleeding or painful joint
• To prevent deformity in chronic bleeding joints and/or to prevent postsurgical
deformity
• To correct joint deformity if a contracture is present
• To maintain range of motion
• To improve joint function
• To provide support for weakness or instability
36. EARLY INTERVENTION - SPLINTING, ORTHOTICS,
AND ASSISTIVE DEVICES
Assistive devices include
• Crutches
• Cane
• Walker
Care should be taken to make sure that they have been fitted properly for the patient
and that the patient has had proper instruction in the use of these devices
37. EARLY INTERVENTION - SYNOVECTOMY
• Chronic hemophilic hemarthrosis is incited by a hypertrophic and highly vascular
synovium
• Removal of this synovium may be the key to prevention of further joint damage
• Accomplished through surgical and non-surgical procedures
• Radionuclide synovectomy
• Chemical synovectomy – Rifampacin/oxytetracycline
• Arthroscopic/open synovectomy