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    There are three components of blood coagulationThere are three components of blood coagulation

    system:system:

    HAEMOSTASISHAEMOSTASIS

    1.1.

    CapillariesCapillaries

    2.2.

    PlateletsPlatelets

    3.3.

    PlasmaPlasma

    coagulationcoagulation

    factorsfactors

    1. 2: Primary haemostasis1. 2: Primary haemostasis (it is enough to stop bleeding from(it is enough to stop bleeding fromsmall injuries)small injuries)

    3: Secundary haemostasis3: Secundary haemostasis (it is necessary to stop bleeding(it is necessary to stop bleedingdefiniteldefinitel

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    Primary haemostasis I:Primary haemostasis I:

    CapillariesCapillaries and larger blood vessels react to injury byand larger blood vessels react to injury by

    an immediate local temporaryan immediate local temporary vasoconstrictionvasoconstriction (a reflex nervous(a reflex nervousmechanism) to reduce the amount of blood lost.mechanism) to reduce the amount of blood lost.

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    Primary haemostasis II:Primary haemostasis II:

    Platelets:Platelets:-- adhereadhere to the site of injuryto the site of injury

    -- aggregationaggregation

    -- releaserelease substances from their cytoplasms to initiatesubstances from their cytoplasms to initiateblood coagulationblood coagulation haemostatic plug is formed.haemostatic plug is formed.

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    Disorders of the haemostatic mechanism areDisorders of the haemostatic mechanism are

    devided into three main groups:

    Disorders of the vesselsDisorders of the vessels

    Disorders of the plateletsDisorders of the platelets

    Disorders of the coagulation mechanismDisorders of the coagulation mechanism(coagulopathies)(coagulopathies)

    purpuricpurpuric

    diseasesdiseases

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    The investigation of a patient with aThe investigation of a patient with a

    suspected disorder of haemostasissuspected disorder of haemostasis

    case history (personal details, familycase history (personal details, family

    history)history)

    inspection (type of bleeding)inspection (type of bleeding)

    physical examinationphysical examination

    other known diseasesother known diseases

    drugs and medicationsdrugs and medicationslaboratory testslaboratory tests

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    Certain signs and symptoms are virtually diagnostic ofCertain signs and symptoms are virtually diagnostic of

    disordered haemostasis.disordered haemostasis.

    The main symptom of all diseases is theThe main symptom of all diseases is the bleeding:bleeding:

    in thein the purpuric disorderspurpuric disorders cutaneous and mucosalcutaneous and mucosal

    bleeding usually is prominentbleeding usually is prominent

    in different types ofin different types ofcoagulopathiescoagulopathies hemarthroses,hemarthroses,

    haematomas are the characteristic bleeding manifestations.haematomas are the characteristic bleeding manifestations.

    The onset of bleeding following trauma frequently isThe onset of bleeding following trauma frequently is

    delayeddelayed (recur in a matter of hours)(recur in a matter of hours)(the temporary hemostatic adequacy of the platelet plug may explain this(the temporary hemostatic adequacy of the platelet plug may explain this

    phenomenon).phenomenon).

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    Petechiae, purpuras:Petechiae, purpuras:small capillary haemorrhages ranging from the size of a pinhead to much largersmall capillary haemorrhages ranging from the size of a pinhead to much larger

    http://www.jyi.org/articleimages/294/originals/img0.jpghttp://www.jyi.org/articleimages/294/originals/img0.jpghttp://en.wikipedia.org/wiki/Image:Purpura.jpg
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    Petechiae, purpurasPetechiae, purpuras

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    Haematomas:Haematomas:

    may be spontaneous (in a serious hemorrhagic disease) or may occurmay be spontaneous (in a serious hemorrhagic disease) or may occur

    after trauma (in a mild hemorrhagic disease).after trauma (in a mild hemorrhagic disease).

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    HaematomasHaematomas

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    Intramuscular injection may be very dangerous toIntramuscular injection may be very dangerous to

    the patient with a bleeding disorderthe patient with a bleeding disorder

    Venipuncture (if skilfullyVenipuncture (if skilfully

    performed) is without dangerperformed) is without danger

    becouse the elasticity of the venousbecouse the elasticity of the venous

    walls.walls.

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    Screening tests of blood coagulationScreening tests of blood coagulation Disorders of vessels:Disorders of vessels:

    Rumpel-Leede testRumpel-Leede test

    Disorders of platelets:Disorders of platelets:

    Platelet count and morphologyPlatelet count and morphology

    Bleeding time (Ivy)Bleeding time (Ivy)

    Coagulopathies:Coagulopathies:

    Coagulation timeCoagulation time

    Aktivated partial thromboplastinAktivated partial thromboplastin

    time (APTT)time (APTT)

    Prothrombin (INR)Prothrombin (INR)

    Thrombin time (TT)Thrombin time (TT)

    http://images.google.hu/imgres?imgurl=http://www.sld.cu/fotos/foto.php%3Fid%3D906%26key%3Ddz%26width%3D0%26height%3D230&imgrefurl=http://www.sld.cu/sitios/reumatologia/galeria.php%3Fid_esp%3D109%26action%3D2&h=230&w=243&sz=9&hl=hu&start=22&tbnid=G3pjokhDLSMeuM:&tbnh=104&tbnw=110&prev=/images%3Fq%3DLeede%2BRumpel%26start%3D20%26gbv%3D2%26ndsp%3D20%26svnum%3D10%26hl%3Dhu%26sa%3DN
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    Laboratory diagnosis of the coagulopathiesLaboratory diagnosis of the coagulopathies

    Contact activationContact activation Tissue thromboplastin (TF)Tissue thromboplastin (TF)

    XIIXII

    XIXI

    IXIX

    VIIIVIII

    VIIVII

    XX

    VV

    IIII

    II

    INTRINSICINTRINSIC EXTRINSICEXTRINSIC

    COMMONCOMMON

    BloodBlood

    coagulationcoagulation

    timetime

    APTTAPTT

    Prothrom-Prothrom-

    binbin

    FibrinFibrin

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    PlateletPlateletcountcount

    BleedingBleedingtimetime

    APTTAPTT Prothrom-Prothrom-binbin

    Presumptive diagnosisPresumptive diagnosis

    DecreasedDecreased ProlongedProlonged Norm.Norm. Norm.Norm. ThrombocytopeniaThrombocytopenia

    Norm.Norm. ProlongedProlonged ProlongedProlonged Norm.Norm. von Willebrands diseasevon Willebrands disease

    Norm./Norm./

    increasedincreased

    ProlongedProlonged Norm.Norm. Norm.Norm. ThrombocytopathiaThrombocytopathia

    Norm.Norm. Norm.Norm. ProlongedProlonged Norm.Norm. intrinsic pathwayintrinsic pathway

    abnormalityabnormality

    (FVIII. IX. XI. XII)(FVIII. IX. XI. XII)

    Norm.Norm. Norm.Norm. Norm.Norm. ProlongedProlonged extrinsicpathwayextrinsicpathwayabnormalityabnormality (FVII)(FVII)

    Norm.Norm. Norm.Norm. ProlongedProlonged ProlongedProlonged common pathwaycommon pathway

    abnorm.abnorm. (FI. II. V. X.)(FI. II. V. X.)

    Norm.Norm. Norm.Norm. Norm.Norm. Norm.Norm. - /FXIII deficiency/ milde- /FXIII deficiency/ milde

    bleeding disorderbleeding disorder

    Diagnosis of bleeding disorders by the screening testsDiagnosis of bleeding disorders by the screening tests

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    CoagulopathiesCoagulopathies

    Aquired:Aquired: generallygenerallyseveral coagulationseveral coagulation

    abnormalitiesabnormalities are present.are present.

    Clinical picture is complicatedClinical picture is complicated

    by signs and symptoms ofby signs and symptoms ofthethe

    underlying diseaseunderlying disease..

    Deficiencies of the vitamin KDeficiencies of the vitamin K

    dependent coagulation factors (FII,dependent coagulation factors (FII,

    VII, IX, X)VII, IX, X) HepaticHepatic

    disordersdisorders AcceleratedAccelerated

    destruction of blood coagulation (DIC)destruction of blood coagulation (DIC)

    Inhibitors ofInhibitors of

    coagulationcoagulation Others (massiveOthers (massive

    transfusion, extracorporal circulation)transfusion, extracorporal circulation)

    Hereditary:Hereditary:

    deficiency or abnormality ofdeficiency or abnormality ofaa single coagulation factor.single coagulation factor.

    Hemofilia A (FVIII)Hemofilia A (FVIII)

    Hemofilia B (FIX)Hemofilia B (FIX)

    Von Willebrands diseaseVon Willebrands disease

    Rare coagulopathiesRare coagulopathies

    (FI. II. V. VII. X. XI. XIII)(FI. II. V. VII. X. XI. XIII)

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    HaemophiliaHaemophilia

    A bleeding disorder in which clotting factor VIIIA bleeding disorder in which clotting factor VIII

    (eight)(eight) /Haemophilia A//Haemophilia A/or IX (nine)or IX (nine)/Haemophilia B//Haemophilia B/

    in a person's blood plasma is missing or is at a lowin a person's blood plasma is missing or is at a low

    level.level.

    Prevalence:Prevalence:

    haemophilia A: 105/million menhaemophilia A: 105/million men

    haemophilia B: 28/million menhaemophilia B: 28/million men

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    In haemophilia, VIIIIn haemophilia, VIII

    or IX clotting factor isor IX clotting factor ismissing, or the level ofmissing, or the level ofthat factor is low.that factor is low.

    This makes it difficultThis makes it difficultfor the blood to form afor the blood to form aclot, so bleedingclot, so bleedingcontinues longer thancontinues longer than

    usual.usual.

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    The hemophilia gene is carried on theThe hemophilia gene is carried on the X chromosomeX chromosome

    in males who lack a normal allele, the defect is manifested byin males who lack a normal allele, the defect is manifested by

    clinical haemophilia. Women may be carriers.clinical haemophilia. Women may be carriers.

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    Haemophilia is aHaemophilia is a lifelong diseaselifelong disease

    A person born withA person born with

    haemophilia will havehaemophilia will have

    it for life.it for life. The level of factor VIIIThe level of factor VIII

    or IX in his bloodor IX in his blood

    usually stays the sameusually stays the same

    throughout his life.throughout his life.

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    Clinical manifestationsClinical manifestations

    The most dramatic manifestation of haemophilia isThe most dramatic manifestation of haemophilia is

    extensiveextensive bleedingbleeding into the soft tissue and musclesinto the soft tissue and muscles afterafter

    only negligible trauma, or even no known trauma.only negligible trauma, or even no known trauma.

    The frequency and severity of bleeding generally isThe frequency and severity of bleeding generally is

    related to the blood level of FVIII or FIX.related to the blood level of FVIII or FIX.

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    Haemophilia can be mild, moderate, or severe,Haemophilia can be mild, moderate, or severe,

    depending on the level of clotting factor.depending on the level of clotting factor.

    Three category of severity:Three category of severity:

    Severe:Severe: FVIII/FIXFVIII/FIX < 1 %< 1 % Repeated and severe hemarthroses and spontaneousRepeated and severe hemarthroses and spontaneous

    bleeding, crippling common.bleeding, crippling common.

    Moderate:Moderate: FVIII/FIX:FVIII/FIX: 1-5 %1-5 % Spontaneous bleeding and hemarthroses infrequent.Spontaneous bleeding and hemarthroses infrequent.

    Serious bleeding from trivial injuries.Serious bleeding from trivial injuries.

    Milde:Milde: FVIII/FIX:FVIII/FIX: 5-40 %5-40 % Spontaneous bleeding manifestations may be absent,Spontaneous bleeding manifestations may be absent,

    although serious bleeding may follow surgicalalthough serious bleeding may follow surgical

    procedures or traumatic injury.procedures or traumatic injury.

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    Joint bleedingJoint bleeding

    As blood fills the capsule, the jointAs blood fills the capsule, the joint

    swells and becomes painful and hard toswells and becomes painful and hard to

    move.move.

    The most common joint bleeds happenThe most common joint bleeds happen

    in ankles, knees, and elbows.in ankles, knees, and elbows.

    Bleeds into other joints can also happen.Bleeds into other joints can also happen.

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    The long-term effects of jointThe long-term effects of joint

    bleeds:bleeds:Repeated bleeding into a joint causes theRepeated bleeding into a joint causes the

    synovium to swell and bleed very easily.synovium to swell and bleed very easily.

    Some blood remains in the joint after eachSome blood remains in the joint after each

    bleed. The synovium stops producing thebleed. The synovium stops producing the

    slippery, oily fluid that helps the joint move.slippery, oily fluid that helps the joint move.

    This damages the smooth cartilage thatThis damages the smooth cartilage that

    covers the ends of the bones. The jointcovers the ends of the bones. The jointbecomes stiff, painful to move, and unstable.becomes stiff, painful to move, and unstable.

    It becomes more unstable as muscles aroundIt becomes more unstable as muscles around

    the joint weaken.the joint weaken.

    With time, most of the cartilage breaks downWith time, most of the cartilage breaks downand some bone wears away. Sometimes theand some bone wears away. Sometimes the

    joint cannot move at all.joint cannot move at all.

    The whole process is called:The whole process is called:

    hemophilic arthritis.hemophilic arthritis.

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    Haemophilic arthropathy (radiographs)Haemophilic arthropathy (radiographs)

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    Other types of bleeding:Other types of bleeding:subcutaneous, intramuscular hematomas, gastrointestinalsubcutaneous, intramuscular hematomas, gastrointestinal

    bleeding, hematuria, cerebral hemorrhagebleeding, hematuria, cerebral hemorrhage

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    Volkmanns contractureVolkmanns contracture

    Large haematoma of theLarge haematoma of the

    cerebellumcerebellum(computer tomography)(computer tomography) PseudotumorPseudotumor

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    Life-threatening bleeding:Life-threatening bleeding:- bleeding within the head is a- bleeding within the head is a

    major cause of death inmajor cause of death inhaemophiliahaemophilia

    -Bleeding into the throat mayBleeding into the throat may

    cause swelling, as well as difficultycause swelling, as well as difficulty

    swallowing and breathingswallowing and breathing- Gastrointestinal bleeding (oftenGastrointestinal bleeding (often

    due to peptic ulceration)due to peptic ulceration)

    Serious, but usually notSerious, but usually notlife-threatening bleeding:life-threatening bleeding:- bleeds into the eyes, spine and- bleeds into the eyes, spine and

    psoas musclepsoas muscle

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    TherapyTherapy

    The only mode of treatment isThe only mode of treatment is replacement therapy:replacement therapy:toto

    injectinjectthe missing clotting factor into a vein.the missing clotting factor into a vein.

    Clotting factor cannot be given by mouth.Clotting factor cannot be given by mouth.

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    Factor substitutionFactor substitution

    On demand:On demand:

    in the event of bleeding episodesin the event of bleeding episodes

    Profilaxis:Profilaxis: to preventto preventbleedings and their consequencesbleedings and their consequences

    primaryprimary

    secundarysecundary

    Home treatment:Home treatment: the patient or his relatives arethe patient or his relatives are

    taught to give iv. injection of thetaught to give iv. injection of the

    factor concentrate immediatelyfactor concentrate immediately

    when there are symptoms ofwhen there are symptoms of

    bleeding.bleeding.

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    Calculation of the dose of factor replacementCalculation of the dose of factor replacement

    Haemophilia A:Haemophilia A:

    (desired level FVIII % - patient FVIII level %) x bodyweightkg/2(desired level FVIII % - patient FVIII level %) x bodyweightkg/2

    Haemophilia B:Haemophilia B:

    (desired level FIX % - patient FIX level %) x bodyweightkg(desired level FIX % - patient FIX level %) x bodyweightkg

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    Recommended doses of FVIII/FIX for variousRecommended doses of FVIII/FIX for various

    types of haemorrhagetypes of haemorrhage

    SSiittee ooffhheemmoorrrrhhaaggee:: DDeessiirreedd FFVVIIIIII//FFIIXXlleevveell ((%%))::

    DDuurraattiioonn ((ddaayyss))::

    Hemarthroses,superficial,

    intramuscular

    hematoma, im. inj.

    1100--2200 %% 11--33

    Deep intramuscular

    haematomas2200--3355 %% 33--44

    Tooth extraction,

    intraabdominal,intrathoracal bleeds,

    epistaxis, minor surgery

    4400--5500 %% 44--1144

    uunnttiill hheeaalliinngg

    Central nervous system,

    major surgery5500--110000 %% 1144--2211

    uunnttiill hheeaalliinngg

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    Factor replacementFactor replacement

    at the consultingat the consultingroomroom

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    Home therapy:Home therapy:

    is infusion with clotting factoris infusion with clotting factor

    replacement away from the hospital.replacement away from the hospital.

    A person with haemophilia can infuseA person with haemophilia can infuseat home, school, work, or elsewhere.at home, school, work, or elsewhere.

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    sharps containersharps container

    disposable wipesdisposable wipes

    alcohol wipealcohol wipe

    bandagebandage

    cotton ballscotton balls

    tapetape

    tourniquettourniquetbutterfly needlebutterfly needle

    syringesyringe

    transfer needle/transfer needle/

    filter needlefilter needle

    factor concentratefactor concentratelatex gloveslatex gloves

    diluent (sterile water) supplieddiluent (sterile water) supplied

    with the concentratewith the concentrate

    Supplies needed for treatment with factor concentrate:Supplies needed for treatment with factor concentrate:

    A written recordA written record

    of all treatmentsof all treatments

    must be kept.must be kept.

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    Medical treatment is only one part of good health.Medical treatment is only one part of good health.

    People with hemophilia should:People with hemophilia should:

    - Exercise and stay fit.- Exercise and stay fit.

    - Wear protection that is appropriate for the sport or- Wear protection that is appropriate for the sport or

    activity.activity.

    - Get regular check-ups that include joint and muscle- Get regular check-ups that include joint and muscle

    examination.examination.

    - Get all vaccinations recommended, including hepatitis A- Get all vaccinations recommended, including hepatitis A

    and hepatitis B protection.and hepatitis B protection.

    - Maintain a healthy body weight. People who do not exercise- Maintain a healthy body weight. People who do not exerciseare more likely to put on extra weight. A person withare more likely to put on extra weight. A person with

    hemophilia needs to control his weight so that he does nothemophilia needs to control his weight so that he does not

    put extra stress on his joints, especially if he has arthritis.put extra stress on his joints, especially if he has arthritis.

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    Dental healthDental healthis very important in haemophilia:is very important in haemophilia:

    - Healthy teeth and gums reduce the need for haemophilia- Healthy teeth and gums reduce the need for haemophilia

    treatment.treatment.

    - Regular dental care reduces the need for injections and- Regular dental care reduces the need for injections and

    surgery.surgery.

    - Dental care should include brushing, flossing, and check-ups byDental care should include brushing, flossing, and check-ups by

    a dentist.a dentist.

    - Cooperation between hematologists (hemostaseologists) andCooperation between hematologists (hemostaseologists) and

    dentists is necessary.dentists is necessary.

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    Regular dental visitsRegular dental visits usually every 6 months usually every 6 months will help identify problems early.will help identify problems early.

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    I.I. Prevention:Prevention:

    It is an essential component of oral care.It is an essential component of oral care.

    It will reduce the need of treatment and the numberIt will reduce the need of treatment and the numberof emergency visit.of emergency visit.

    With prevention Without preventionWith prevention Without prevention

    O l h i i i

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    Oral hygiene is very important:Oral hygiene is very important:

    BrushingBrushingtwice daily with a fluoride toothpaste.twice daily with a fluoride toothpaste.

    Proper brushing is essential for cleaning teeth and gumsProper brushing is essential for cleaning teeth and gumseffectively.effectively.

    It removesIt removes plaqueplaquefrom the surfaces of teeth.from the surfaces of teeth.PlaquePlaquedevelops into unhealthydevelops into unhealthy calculus.calculus.

    EnamelEnamel

    PlaquePlaque

    CalculusCalculus

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    Brushing must begin atBrushing must begin at

    childhood.childhood.

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    The toothbrush should have medium texture bristlesThe toothbrush should have medium texture bristles

    (- hard bristles can cause abrasion of the teeth(- hard bristles can cause abrasion of the teeth

    - soft bristles are inadequate).- soft bristles are inadequate).

    Interdental cleaning aids (floss, tape, interdentalInterdental cleaning aids (floss, tape, interdental

    brushes) should be used to prevent the formation ofbrushes) should be used to prevent the formation of

    dental caries and periodontal disease.dental caries and periodontal disease.

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    Recommended Fluoride Supplementation:

    AGEAGE

    FLUORIDE ION LEVEL IN DRINKING WATERFLUORIDE ION LEVEL IN DRINKING WATER (ppm)

    Less than 0.3ppm

    0.3 to 0.6 ppm More than 0.6ppm

    Birth - 6 months NONE NONE NONE

    6 months - 3 years 0.25 mg / day NONE NONE

    3 - 6 years 0.50 mg / day 0.25 mg / day NONE

    6 - 16 years 1.0 mg / day 0.50 mg / day NONE

    Fluoride supplementsFluoride supplements(fluorid drops, tablets, topical application of fluorid(fluorid drops, tablets, topical application of fluorid

    mouthrinses)mouthrinses) are recommended if the water supply isare recommended if the water supply is 0,6 ppm0,6 ppm

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    Dietary CounselingDietary Counseling

    The consumption of food andThe consumption of food and

    drinks with a high sugar and aciddrinks with a high sugar and acid

    content should be limited tocontent should be limited to

    mealtimesmealtimes

    (the aim: food and drink does not(the aim: food and drink does not

    cause the pH level of the oralcause the pH level of the oral

    cavity to fall below the criticalcavity to fall below the critical

    level of pH 5,5).level of pH 5,5).

    Artificial sweeteners (aspartame,Artificial sweeteners (aspartame,

    sorbitol, acesulfamate) can besorbitol, acesulfamate) can be

    used as an alternative to sugars.used as an alternative to sugars.

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    Orthodontic treatment:Orthodontic treatment:

    Fixed and removable orthodontic appliences may be used.Fixed and removable orthodontic appliences may be used.

    Special care should be taken when treating patients with aSpecial care should be taken when treating patients with asevere bleeding disorder to ensure that the gingiva is notsevere bleeding disorder to ensure that the gingiva is not

    damaged when fitting the applience.damaged when fitting the applience.

    P i d t l t t tP i d t l t t t

    http://ipj.quintessenz.de/index.php?content=issues&doc=zoomimg&select=84&file=abb13gr.jpg
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    Periodontal treatment:Periodontal treatment:

    GUM DISEASE IS THEGUM DISEASE IS THEMAIN CAUSE OF TOOTHMAIN CAUSE OF TOOTH

    LOSS!LOSS!

    Mouth tissues reflectMouth tissues reflect

    symptoms of other problems.symptoms of other problems.

    80 % or more of adults have80 % or more of adults have

    some form of periodontalsome form of periodontal

    disease anddisease and

    99% of those have no signs99% of those have no signsthat indicate they have athat indicate they have a

    problem.problem.

    P i d titi ( di )P i d titi ( di )

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    Periodontitis (gum disease)Periodontitis (gum disease)Healthy gumsHealthy gums

    Severely advanced periodontalSeverely advanced periodontal

    (gum) disease, with receded(gum) disease, with receded

    gums, massive stains fromgums, massive stains from

    smoking cigarettes andsmoking cigarettes and

    inadequate oral hygiene.inadequate oral hygiene.

    http://images.google.hu/imgres?imgurl=http://www.mkdentalcare.com/Pictures/MV110118.JPG&imgrefurl=http://www.mkdentalcare.com/GumDisease.htm&h=600&w=800&sz=39&hl=hu&start=2&tbnid=--TW_pQTOr2AtM:&tbnh=107&tbnw=143&prev=/images%3Fq%3Dgum%2Bdisease%2B%26gbv%3D2%26svnum%3D10%26hl%3Dhu%26sa%3DG
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    In case of periodontal disease treatment must start as soon asIn case of periodontal disease treatment must start as soon as

    possible.possible.

    Factor replacement therapy is not necessary.Factor replacement therapy is not necessary.Bleeding can be controlled locally: with direct pressureBleeding can be controlled locally: with direct pressure

    and topical antifibrinolytic agents.and topical antifibrinolytic agents.

    Clorhexidine gluconate mouthwash can be used toClorhexidine gluconate mouthwash can be used to

    control periodontal problems.control periodontal problems.

    Antibiotics may be required to help reduce the initialAntibiotics may be required to help reduce the initial

    inflammation.inflammation.

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    Dental cariesDental caries

    If the oral hygiene is bad,If the oral hygiene is bad,

    certain bacteria have overgrowncertain bacteria have overgrown

    on certain parts of the toothon certain parts of the toothsurface and have produced sosurface and have produced so

    much acid that the toothmuch acid that the tooth

    mineral has dissolved ormineral has dissolved or

    decayed, forming a cavity.decayed, forming a cavity.

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    Carious lesionsCarious lesions

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    Restorative treatment:Restorative treatment:

    Filling a cavityFilling a cavity cancan

    be undertakenbe undertaken

    routinely withroutinely with

    protection theprotection the

    mucosa.mucosa.

    In the case of localIn the case of local

    anesthesia factoranesthesia factorreplacementreplacement

    therapy istherapy is

    necessary.necessary.

    E d d i ( l h )E d d i ( l h )

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    Endodontic treatment (root canal therapy):Endodontic treatment (root canal therapy):

    It is generally low risk for patients with bleeding disorders.It is generally low risk for patients with bleeding disorders.

    Pulpectomy be carried out carefully (the instrument do notPulpectomy be carried out carefully (the instrument do notpass through the apex of the root canal).pass through the apex of the root canal).

    Tooth extraction dentalTooth extraction dental

    http://hu.wikipedia.org/wiki/K%C3%A9p:DentalSurgicalExtraction.jpg
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    Tooth extraction, dentalTooth extraction, dental

    surgery:surgery:

    Extraction of even a single toothExtraction of even a single tooth

    requires replacement therapyrequires replacement therapy

    (recommended FVIII/IX level:(recommended FVIII/IX level:

    40-50 %).40-50 %).

    Multiple extractions may save timeMultiple extractions may save time

    and expense but create a majorand expense but create a major

    bleeding hazard.bleeding hazard.

    The suturing of bleeding toothThe suturing of bleeding tooth

    sockets should be avoided.sockets should be avoided.Antifibrinolytics (tranexamic acidAntifibrinolytics (tranexamic acid

    = Exacyl) may diminish bleeding= Exacyl) may diminish bleeding

    in patients with coagulationin patients with coagulation

    disorders.disorders.

    A h i d iA th i d i t

    http://hu.wikipedia.org/wiki/K%C3%A9p:DentalSurgicalExtraction.jpg
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    Anesthesia and pain management:Anesthesia and pain management:

    - Minor analgesics:- Minor analgesics:

    dental pain can be controlled with a minordental pain can be controlled with a minoranalgesic, such as paracetamol. Aspirin should not beanalgesic, such as paracetamol. Aspirin should not be

    used due to its inhibitory effect on platelet function!used due to its inhibitory effect on platelet function!

    - Local anesthesia:- Local anesthesia:No haemostatic cover:No haemostatic cover: Haemostatic cover required:Haemostatic cover required:

    Buccal infiltrationBuccal infiltration Inferior dental blockInferior dental block

    Intra-papillary injectionIntra-papillary injection Lingual infiltrationLingual infiltration

    Intraligamentary injectionsIntraligamentary injections

    Guidelines for Dental Treatment of Patients with Inherited Bleeding Disorders

    (WFH, Dental Committee, 2006)

    Inferior alveolar nerve block:Inferior alveolar nerve block:

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    Inferior alveolar nerve block:Inferior alveolar nerve block:

    It is a risk of bleeding into the muscles along withIt is a risk of bleeding into the muscles along with

    potential airway compromise due to a haematoma inpotential airway compromise due to a haematoma in

    the retromolar or pterygoid space.the retromolar or pterygoid space.

    A lingual infiltration:A lingual infiltration:

    There is a risk of a significant airway obstruction inThere is a risk of a significant airway obstruction in

    the event a bleed.the event a bleed.

    Dental prosthesisDental prosthesis

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    Dental prosthesisDental prosthesis

    Patients with bleeding disorders can be givenPatients with bleeding disorders can be given

    dentures.dentures.

    If a partial denture is provided it is important that theIf a partial denture is provided it is important that the

    periodontal health of the remaining teeth isperiodontal health of the remaining teeth is

    maintained.maintained.

    The earliest known dental prosthesisThe earliest known dental prosthesis

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    The earliest known dental prosthesisThe earliest known dental prosthesis

    from Romefrom Rome (date from the 1st to the(date from the 1st to the

    2nd century A.D.)2nd century A.D.)resulted fromresulted frommulti-karat gold wire, which wasmulti-karat gold wire, which was

    used to string together "artificialused to string together "artificial

    teeth."teeth."

    They were found in the mouth ofThey were found in the mouth ofanan

    unidentified womanunidentified woman who was buriedwho was buriedin an elaborate mausoleum within ain an elaborate mausoleum within a

    Roman necropolis.Roman necropolis.

    It is believed the unidentifiedIt is believed the unidentified

    Roman's bridgework was made fromRoman's bridgework was made fromthe woman's own teeth that probablythe woman's own teeth that probably

    fell out due to periodontal disease.fell out due to periodontal disease.

    Gold wire bound the teeth together.Gold wire bound the teeth together.

    In dental health of a patient with bleedingIn dental health of a patient with bleeding

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    In dental health of a patient with bleedingIn dental health of a patient with bleeding

    disorder team work is very importantdisorder team work is very important

    (patient- dentist- haematologist)(patient- dentist- haematologist)

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