Hemophilia is a common hereditary coagulation disorder due to deficiency or reduce activity of clotting factor VIII or clotting factor IX.
This disorder is a X- linked recessive disorder.
Types:
Hemophilia A- deficiency of clotting factors VIII
Hemophilia B- deficiency of clotting factors IX
Hemophilia C- deficiency of clotting factors XI
Parahaemophilia- deficiency of clotting factor V
Causes of hemophilia
Hemophilia has a sex-linked recessive inheritance.
In most cases Hemophilia caused by a mutation in a gene that encodes for one of the clotting factors .
Since the hemophilia gene is located on the X chromosome, Hemophilia usually occurs in males, and Female is the carrier of hemophilia.
Diagnosis
Complete blood cell count
Coagulation studies
FVIII assay
Normal values for FVIII assays are 50-150%. Values in hemophilia are as follows:
Mild: >5%
Moderate: 1-5%
Severe: <1%
Treatment of Hemophilia
Other Types of Treatment
Desmopressin (DDAVP)
Antifibrinolytic Medicines
Vaccinations- hepatitis A and B.
Gene Therapy
Gene Therapy
New Drugs for Hemophilia treatment
New Drugs for Hemophilia treatment
Bangladesh perspectives
Bangladesh would have 10800 hemophiliacs.
But, initially the patients does not concern about hemophilia.
Patients are usually diagnosed only after bleeding episode and sometimes the episode are causes serious consequences.
Conclusion
Primary diagnosis with the success of gene therapy and availability of the new bioengineered products the prospect of the hemophiliacs will be brighter in near future.
Megaloblastic anaemia is a red blood cell disorder due to the inhibition of DNA synthesis during erythropioesis.
Mitotically, the inhibition of the DNA synthesis impaires the progression of the cell cycle development from G2 to (M) stage.
Hemophilia is a common hereditary coagulation disorder due to deficiency or reduce activity of clotting factor VIII or clotting factor IX.
This disorder is a X- linked recessive disorder.
Types:
Hemophilia A- deficiency of clotting factors VIII
Hemophilia B- deficiency of clotting factors IX
Hemophilia C- deficiency of clotting factors XI
Parahaemophilia- deficiency of clotting factor V
Causes of hemophilia
Hemophilia has a sex-linked recessive inheritance.
In most cases Hemophilia caused by a mutation in a gene that encodes for one of the clotting factors .
Since the hemophilia gene is located on the X chromosome, Hemophilia usually occurs in males, and Female is the carrier of hemophilia.
Diagnosis
Complete blood cell count
Coagulation studies
FVIII assay
Normal values for FVIII assays are 50-150%. Values in hemophilia are as follows:
Mild: >5%
Moderate: 1-5%
Severe: <1%
Treatment of Hemophilia
Other Types of Treatment
Desmopressin (DDAVP)
Antifibrinolytic Medicines
Vaccinations- hepatitis A and B.
Gene Therapy
Gene Therapy
New Drugs for Hemophilia treatment
New Drugs for Hemophilia treatment
Bangladesh perspectives
Bangladesh would have 10800 hemophiliacs.
But, initially the patients does not concern about hemophilia.
Patients are usually diagnosed only after bleeding episode and sometimes the episode are causes serious consequences.
Conclusion
Primary diagnosis with the success of gene therapy and availability of the new bioengineered products the prospect of the hemophiliacs will be brighter in near future.
Megaloblastic anaemia is a red blood cell disorder due to the inhibition of DNA synthesis during erythropioesis.
Mitotically, the inhibition of the DNA synthesis impaires the progression of the cell cycle development from G2 to (M) stage.
Hemophilia is a rare disorder in which your blood doesn't clot normally because it lacks sufficient blood-clotting proteins (clotting factors). If you have hemophilia, you may bleed for a longer time after an injury than you would if your blood clotted normally. Small cuts usually aren't much of a problem.
Megaloblastic anaemia is a type of anaemia characterized by the formation of unusually large, abnormal and immature red blood cells called as megaloblasts by the bone marrow, which are released into the blood. To know more visit here: www.lazoi.com
Hemophilia is a genetic bleeding disorder in which body loses the ability to stop bleeding due to low levels or absence of proteins known as ‘’clotting factors’’ which are necessary for clotting of blood. Hemophilia leads to excessive bleeding.
Heart failure (HF) is a common cardiovascular condition with increasing incidence and prevalence. Unlike western countries where heart failure is predominantly a disease of elderly, in India it affects younger age group. Heart failure is a chronic condition in which the heart cannot pump enough blood and oxygen to support other organs in your body.
Hemophilia is a rare disorder in which your blood doesn't clot normally because it lacks sufficient blood-clotting proteins (clotting factors). If you have hemophilia, you may bleed for a longer time after an injury than you would if your blood clotted normally. Small cuts usually aren't much of a problem.
Megaloblastic anaemia is a type of anaemia characterized by the formation of unusually large, abnormal and immature red blood cells called as megaloblasts by the bone marrow, which are released into the blood. To know more visit here: www.lazoi.com
Hemophilia is a genetic bleeding disorder in which body loses the ability to stop bleeding due to low levels or absence of proteins known as ‘’clotting factors’’ which are necessary for clotting of blood. Hemophilia leads to excessive bleeding.
Heart failure (HF) is a common cardiovascular condition with increasing incidence and prevalence. Unlike western countries where heart failure is predominantly a disease of elderly, in India it affects younger age group. Heart failure is a chronic condition in which the heart cannot pump enough blood and oxygen to support other organs in your body.
Demands for Haemophilia tratment centres to fullfull universal health access...SEJOJO PHAAROE
Haemophilia ia a rare disease that affect 1: 10 000 people. Demands for services, medication and access to cheap effective clotting factors is a human right for people living with haemophilia. A haemophilia registry is a demand from the Government at large .. Essential drug list should also contain haemophilia medication.
we need to be contacted for more information
www.thinktankent.com
Mechanical Ventilation Cheat Book for Internal Medicine ResidentsThe Medical Post
This short cheat book talks about basic concepts and physiology of artificial ventilation and also elaborates on point guided approach in maneuvering different modes of mechanical ventilation. Consider this as a basic overview and is intended for all internal medicine residents.
Salient features of the book are -
- The book provides a shortcut to understand and remember certain specific formulae and points you require to interpret the 12-lead ECG.
- Treatment protocols (in green boxes) for most of the important conditions are also included.
- View sample ECGs as you read along the topics.
- The content is explained in a very simple language to provide good conceptions, written from a student’s point of view.
- People can gain their belief in the book after going through sample ECGs which would be available at www.themedicalpost.net/ecg
- The book competes with the other books available in the market in simplicity, summaries, treatment protocols, live diagrams and regularly updated sample ECGs on the website.
Pulmonary Thromboembolism - etilogy, types, medical- Surgical and nursing man...VarunMahajani
Disruption of blood supply to lung alveoli due to blockage of one or more pulmonary blood vessels is called as Pulmonary thromboembolism. In this presentation we will discuss its causes, types and its management in depth.
Ethanol (CH3CH2OH), or beverage alcohol, is a two-carbon alcohol
that is rapidly distributed in the body and brain. Ethanol alters many
neurochemical systems and has rewarding and addictive properties. It
is the oldest recreational drug and likely contributes to more morbidity,
mortality, and public health costs than all illicit drugs combined. The
5th edition of the Diagnostic and Statistical Manual of Mental Disorders
(DSM-5) integrates alcohol abuse and alcohol dependence into a single
disorder called alcohol use disorder (AUD), with mild, moderate,
and severe subclassifications (American Psychiatric Association, 2013).
In the DSM-5, all types of substance abuse and dependence have been
combined into a single substance use disorder (SUD) on a continuum
from mild to severe. A diagnosis of AUD requires that at least two of
the 11 DSM-5 behaviors be present within a 12-month period (mild
AUD: 2–3 criteria; moderate AUD: 4–5 criteria; severe AUD: 6–11 criteria).
The four main behavioral effects of AUD are impaired control over
drinking, negative social consequences, risky use, and altered physiological
effects (tolerance, withdrawal). This chapter presents an overview
of the prevalence and harmful consequences of AUD in the U.S.,
the systemic nature of the disease, neurocircuitry and stages of AUD,
comorbidities, fetal alcohol spectrum disorders, genetic risk factors, and
pharmacotherapies for AUD.
These simplified slides by Dr. Sidra Arshad present an overview of the non-respiratory functions of the respiratory tract.
Learning objectives:
1. Enlist the non-respiratory functions of the respiratory tract
2. Briefly explain how these functions are carried out
3. Discuss the significance of dead space
4. Differentiate between minute ventilation and alveolar ventilation
5. Describe the cough and sneeze reflexes
Study Resources:
1. Chapter 39, Guyton and Hall Textbook of Medical Physiology, 14th edition
2. Chapter 34, Ganong’s Review of Medical Physiology, 26th edition
3. Chapter 17, Human Physiology by Lauralee Sherwood, 9th edition
4. Non-respiratory functions of the lungs https://academic.oup.com/bjaed/article/13/3/98/278874
Explore natural remedies for syphilis treatment in Singapore. Discover alternative therapies, herbal remedies, and lifestyle changes that may complement conventional treatments. Learn about holistic approaches to managing syphilis symptoms and supporting overall health.
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
Tom Selleck Health: A Comprehensive Look at the Iconic Actor’s Wellness Journeygreendigital
Tom Selleck, an enduring figure in Hollywood. has captivated audiences for decades with his rugged charm, iconic moustache. and memorable roles in television and film. From his breakout role as Thomas Magnum in Magnum P.I. to his current portrayal of Frank Reagan in Blue Bloods. Selleck's career has spanned over 50 years. But beyond his professional achievements. fans have often been curious about Tom Selleck Health. especially as he has aged in the public eye.
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Introduction
Many have been interested in Tom Selleck health. not only because of his enduring presence on screen but also because of the challenges. and lifestyle choices he has faced and made over the years. This article delves into the various aspects of Tom Selleck health. exploring his fitness regimen, diet, mental health. and the challenges he has encountered as he ages. We'll look at how he maintains his well-being. the health issues he has faced, and his approach to ageing .
Early Life and Career
Childhood and Athletic Beginnings
Tom Selleck was born on January 29, 1945, in Detroit, Michigan, and grew up in Sherman Oaks, California. From an early age, he was involved in sports, particularly basketball. which played a significant role in his physical development. His athletic pursuits continued into college. where he attended the University of Southern California (USC) on a basketball scholarship. This early involvement in sports laid a strong foundation for his physical health and disciplined lifestyle.
Transition to Acting
Selleck's transition from an athlete to an actor came with its physical demands. His first significant role in "Magnum P.I." required him to perform various stunts and maintain a fit appearance. This role, which he played from 1980 to 1988. necessitated a rigorous fitness routine to meet the show's demands. setting the stage for his long-term commitment to health and wellness.
Fitness Regimen
Workout Routine
Tom Selleck health and fitness regimen has evolved. adapting to his changing roles and age. During his "Magnum, P.I." days. Selleck's workouts were intense and focused on building and maintaining muscle mass. His routine included weightlifting, cardiovascular exercises. and specific training for the stunts he performed on the show.
Selleck adjusted his fitness routine as he aged to suit his body's needs. Today, his workouts focus on maintaining flexibility, strength, and cardiovascular health. He incorporates low-impact exercises such as swimming, walking, and light weightlifting. This balanced approach helps him stay fit without putting undue strain on his joints and muscles.
Importance of Flexibility and Mobility
In recent years, Selleck has emphasized the importance of flexibility and mobility in his fitness regimen. Understanding the natural decline in muscle mass and joint flexibility with age. he includes stretching and yoga in his routine. These practices help prevent injuries, improve posture, and maintain mobilit
These lecture slides, by Dr Sidra Arshad, offer a quick overview of physiological basis of a normal electrocardiogram.
Learning objectives:
1. Define an electrocardiogram (ECG) and electrocardiography
2. Describe how dipoles generated by the heart produce the waveforms of the ECG
3. Describe the components of a normal electrocardiogram of a typical bipolar leads (limb II)
4. Differentiate between intervals and segments
5. Enlist some common indications for obtaining an ECG
Study Resources:
1. Chapter 11, Guyton and Hall Textbook of Medical Physiology, 14th edition
2. Chapter 9, Human Physiology - From Cells to Systems, Lauralee Sherwood, 9th edition
3. Chapter 29, Ganong’s Review of Medical Physiology, 26th edition
4. Electrocardiogram, StatPearls - https://www.ncbi.nlm.nih.gov/books/NBK549803/
5. ECG in Medical Practice by ABM Abdullah, 4th edition
6. ECG Basics, http://www.nataliescasebook.com/tag/e-c-g-basics
micro teaching on communication m.sc nursing.pdfAnurag Sharma
Microteaching is a unique model of practice teaching. It is a viable instrument for the. desired change in the teaching behavior or the behavior potential which, in specified types of real. classroom situations, tends to facilitate the achievement of specified types of objectives.
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.
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Hemophilia
1. Hemophilia
Dr. Kalpana Malla
MD Pediatrics
Manipal Teaching Hospital
Download more documents and slide shows on The Medical Post [ www.themedicalpost.net ]
2. Introduction:HAEMOPHILIA
• Commonest inherited bleeding
disorder
• Bleeding due to deficiency of FVIII /
IX / XI coagulant activity
• Severity of bleeding is related to
FVIII / IX /XI concentration in blood
3. INCIDENCE
• 1 per 5,000 male births
• 1 per 10,000 population
• 85 % - F VIII deficiency
• 10- 15 % - F IX deficiency
• Haemophilia A: B= 7:1
6. INHERITANCE
• Father with Haemophilia:
Daughters are carriers Sons normal
• Mother with haemophilia gene (carrier)
Sons 50:50 normal or affected
Daughters 50:50 normal or carriers
7. FEMALES AFFECTED ONLY WHEN:
1) TURNERS SYNDROME
2) MOSAICISM/LYONISATION
3) MOTHER TO DAUGHTER
TRANSMISSION (POSSIBLE
THEORETICALLY BUT EXTREMLY
RARE)
8. Types:
• Haemophilia A – deficiency of Factor VIII
• Haemophilia B – deficiency of Factor IX
• Haemophilia C – deficiency of Factor XI
9. HAEMOPHILIA A & B:
• Basic abnormality :
1. Reduction in amount of protein in
factor
2. Dysfunctional protein
5-10 % Haemophilia A
40-50 % ” ” B
10. Severity of haemophilia
• 1 ml of normal plasma contains 1 unit (U) of each
factor
100 ml plasma contains 100 U/dl (100 % activity)
Severity depends on factor level in blood:
● Severe haemophilia: < 1 U/dl (%)
● Moderate haemophilia: 1-5 U/dl (%)
● Mild haemophilia: 5-30 U/dl (%)
11. Severity of haemophilia
• Haemostatic level of factor VIII: 30-40 U/dl
• ” ” of factor IX: 25-30 U/dl
12. Severity of Haemophilia
Severity Factor Type of presentation
level
iu/dl (%)
Severe <1 Spontaneous bleeds, Severe bleeding
Moderate 1-5 Few bleeds, Haemathrosis - traumatic
Mild 5-30 Few bleeds, Post-traumatic
Post-dental surgery
14. CLINICAL FEATURES – SUBTLE
• Bleeding as a baby rare
• Prolonged bleed from umbilical cord
• Muscle hematoma during immunisation
• Bleeding during circumcision
15. CLINICAL FEATURES – SUBTLE
• Toddlers - Large and prolonged bleed to
trivial injury/cuts/abrasions
• Lip bleeds (hematomas)
• First bleeding in childhood
Tooth extraction
Trauma with walking
Gum bleeds while brushing teeth
16. SIGNIFICANT HEMORRHAGES
• RETROPERITONEAL H’GE: severe abd
pain, anemia and shock
• HEMATURIA
• GI BLEED : difficult to control, severe
• INTRACRANIAL:
extradural, subdural, intracerebral
- Headache, vomiting, altered sensorium ->
coma
• May be seen in neonates
18. CLINICAL FEATURES - FRANK
HEMARTHROSIS (joint bleed) – hallmark of
hemophilia
• Joints affected:
• in toddlers - ankle (most common) – earliest jt
involved due to lack of stability as they assume
upright posture
• Older child – knee, elbow (most common)
** Target joint – recurrent bleeding at a same
joint
• LL > UL
19. CLINICAL FEATURES - FRANK
• Later : all joints
• Contact sports can provoke
• Recurrent – may be unprovoked,
spontaneous
LARGE HEMATOMAS & EXTENSIVE
ECCHYMOSES
20. BLEEDING INTO JOINTS
• First haemorrhage : Swelling >> Pain
• Subsequent haemorrhages: Pain >>
Swelling
23. C/F OF ACUTE HAEMARTHROSIS
• Abnormal sensation
• Pain and swelling of joint
• Limitation of movement - especially
flexion
• Tenderness and heat in the joint
Acute symptoms last 3-4 days; full recovery
takes weeks
24. STAGES
• Initial bleed into joint - haemarthrosis
• Inflammatory stage affecting
Synovium (synovial hypertrophy)
Cartilage - Bone
• Final Stage
Permanent joint changes
Erosion & destruction Cartilage, Bone -
Knees** ,Ankles** , Elbows*
Wrists
Shoulders less common
Hips
25. Chronic Haemophilic Arthropathy
• Repeated bleeds - many years
Chronic degenerative changes
• Chronic haemophilic arthritis → Loss
of joint movement → Fixed flexion
contractures → Severe muscle wasting
→ Muscle action imbalance →Valgus
deformities → Crippling deformities →
Wheel chair-bound
26. Chronic Haemophilic Arthropathy – Radiological
Changes
• Epiphyseal overgrowth
• Enlargement of bone ends
• Loss of cartilage (joint space)
• Gross irregularity articular surface
–Subchondral collapse
–Subchondral cysts
–Osteophyte formation
–Osteoporosis
–Changes in joint alignment
27. HAEMOPHILIC PSEUDO TUMOURS (BLOOD
CYSTS)
• Cysts within the fascial muscle envelope
• Cysts arising in muscles
• Cysts arising from sub-periosteal
haemorrhage
• Pseudo tumours in bone
• Gross destruction of normal architecture
of bone
• Large bone cysts
• Pathological fracture
29. Lab findings:
• Hb low – proportional to blood loss
• Platelets- normal
• Bleeding time -normal
• PT normal
• APTT prolonged > 2-3 times ULN
• CT prolonged
• Low levels of F VIII / IX
• Factor VIII and IX assay : mixing studies
• Genetic analysis
30. MIXING STUDIES
• To determine if prolonged PT or PTT is due to
a factor deficiency or an inhibitor
• Normal plasma : all Clotting factors-V,VIII,
IX,X,XI,XII
• Method: add patient plasma to equal volume
of normal plasma and repeat PT & PTT
• Correction of PT & PTT – suggests- deficiency
of clotting factors
• Assays for factors
31. MIXING STUDIES
• Remains prolonged after mixing study:
indicates inhibitor - most common is
lupus anticoagulant
- therapeutic anticoagulant
- rarely ,inhibitors to factors VIII, IX, XI
32. MIXING STUDIES
• With normal /adsorbed plasma/aged plasma
• Normal plasma : all factor present
• Adsorbed plasma : FIX- deficient
• Aged plasma - FVIII- deficient
• Mix patient’s plasma with normal /aged/
adsorbed plasma –
• Correction of APTT with normal & aged
plasma/ not with adsorbed plasma F IX
deficiency
33. • Correction of APTT with normal /
adsorbed plasma & not with aged
plasma F VIII deficiency
• If correction does not occur suspect
inhibitor
34. Inhibitors:
• Antibodies against factors blocks clotting
activity
• 14-25 % patients who receive factors (VIII/ IX)
• Failure of a bleeding episode to respond to
appropriate replacement therapy 1st sign of
inhibitor
• Others develop higher titres
desensitisation- higher doses of factors given
39. FFP AND CRYOPPT
• FFP contains F VIII and IX
• CRYOPPT contains F-VIII, fibrinogen, VWF
• 1 unit FFP = 200 units factor VIII/IX
• 1 unit cryoppt = 100 units factor VIII
• FVIII 1u/kg Increase plasma factor VIII by 2% (
2 iu/dl) - t ½ = 8 hrs
• FIX 1u/kg Increase plasma factor IX by 1%
(1iu/dl) - t ½ = 18-20 hrs
• Risk of HIV, HBV, HCV, CMV transmission
40. FACTOR CONCENTRATES
• Prothrombin complex concentrates
(PCC) - Contain F IX & Vitamin K
dependent factors I.e. II, VII, IX, X – high
cost
• Pure factor IX concentrates available
– Currently high cost
41. THERAPY FOR HAEMOPHILIA
• FACTOR RECOVERY AFTER I.V.
INFUSION
Factor VIII 100%
Factor IX30-50%
• Raise factor levels to:
100 U/dl – life threatening bleeds
35- 40 U/dl – other bleeds
42. DOSAGE CALCULATION
• Dose of F VIII (U):
desired rise in plasma F VIII (U/dl) X body wt (kg) X
0.5
• Dose of F IX (U):
desired rise in plasma F IX (U/dl) X body wt (kg) X
1.4
43. Factor
FVIII
level FIX Dose
Indication or Site of Bleeding Dose,
Desired, IU/kg
IU/kg*
%
Severe epistaxis; mouth, lip,
20-50 10-25 20-50
tongue, or dental work
Joint (hip or groin) - Repeat in 24-
48 h 40 20 40
Soft tissue or muscle 20-40 10-20 40
Muscle (calf and forearm) 30-40 15-20 40
Muscle deep (thigh, hip, iliopsoas)
40-60 20-30 40-60
Transfuse, repeat at 24 h
Neck or throat 50-80 25-40 50-80
44. Hematuria 40 20 40
Laceration 40 20 40
GI or retroperitoneal
60-80 30-40 60-80
bleeding
Head trauma (no evidence of
50 25 50
CNS bleeding)
Head trauma (probable or
definite CNS bleeding, eg,
100 50 100
headache, vomiting,
neurologic signs)
Trauma with bleeding,
80-100 50 100
surgery†
45. Other measures:
• General supportive measures:
bed rest
hospitalize for severe bleeds
analgesics
Local haemostasis
Immobilisation
Physiotherapy
46. HEMARTHOSIS MANAGEMENT
• 25 U F-VIII/kg q12h
• Prompt rx : prevent early sequalae
• Check APTT
• Joint immobilisation - 48hrs
• Early ambulation and physio
• NSAIDS : Aspirin, indomethacin – with
caution- may induce GI bleed
• Paracetamol, pethidine, diazepam – can be
used
• Home infusions : train for early administration
48. PREVENTION
• Immunisation : SC not IM
• Avoid IM injections-
apply pressure 5 minutes
• Avoid contact sports
49. PREVENTION
• Orthopaedic care- traction, splinting,
reconstructive surgery
• Regular dental exam and hygiene
• Prophylactic immunization- hep B
• Counselling
50. DRUGS
• EACA - aminocaproic acid
• Tranexemic acid: 25mg/kg/day
• C/I in hematuria- ppt renal failure
• Desmopressin acetate: increases levels for
first 2 days from body stores
• Danazol
• Fibrin glue : tooth extraction
51. Other agents used:
• Tranexamic acid
Used for external bleeding
eg. teeth extraction
Not for internal bleedings
Inhibits fibrinolysis
decreases F VIII requirement
52. • DDAVP D-amino D-arginine Vasopressin
Found to raise FVIII levels
Mild haemophilia- releases F VIII from storage
sites
Moderate to severe cases- no endogenous
stores treatment ineffective
Mild von Willebrand’s disease
Not effective in Haemophilia B
53. • Danazol:
Androgen
Elevates levels of protein in factors
Increases levels of F VIII & F IX
• Prednisolone
Acute synovitis
56. ACQUIRED HEMOPHILIA
• 5-20% of hemophiliacs develop antibodies against
factor VIII/IX with time - alloantibodies
• Hemophilia A > Hemophilia B
• Suspect when failure of therapy
• Manage:
1) porcine FVIII
2) activated prothrombin complex
3) activated FVII
4) plasmapheresis
5) immunosuppresants
6) recombinant FVIII/IX
57. THERAPY FOR HAEMOPHILIA
• DEMAND THERAPY Hospital
Home
Long standing approach to haemophilia
Patient treats when bleeds
Arrest acute bleed
No arrest, long term sequelae
• PROPHYLACTIC THERAPY
58. PROPHYLACTIC THERAPY
• Initiated with the 1st bleed
• Home - self or patient infused
• Small dose FVIII (FIX) 2-3 X /week ( 20%-
30%)
Prevents bleeds
Prevents long term sequelae
THERAPY FOR SURGERY/ DENTISTRY
• Prophylactic
• Enables major procedures - 10 days cover
59. Thank you
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