Presentation on theme: "Practical approach to a bleeding child"— Presentation transcript:
1 Practical approach to a bleeding child Peri Kamalakar,MDDirectorThe Valerie Fund Children’s CentersFor Cancer &Blood DisordersAtSaint Barnabas Health Care SystemAssociate Director, Hemophilia CenterNewark Beth israel Medical Center
2 Practical approach to a bleeding child OBJECTIVES:Overview of hemostasisClinical approach in making a diagnosisReview the most common bleeding conditionsDiscuss the current treatment strategies
3 Note the central location of red blood cells and the peripheral margination of platelets. This radial distribution of cells results from a fluid dynamic effect called rheophoresis. The location of plasma and platelets at the vessel surface is important for efficient repair of the vessel injury.
4 With an injury to the endothelial lining of the vessel at least two parameters are changed. First collagen is exposed to the platelets that are spatially approximated to the vessel surface. Secondly, rheologic factors further enhance platelet activation.
5 Overview of Haemostasis INJURYCollagen ExposureTissue FactorVASOCONSTRICTIONPlatelet Adhesion and release reactionSerotoninPlatelet PhospolipidCoagulationThromboxane A2 ADPPlatelet aggregationThrombinPrimary haemostatic plugFibrinStable haemostatic plugFibrinolysis
6 Once thrombin is present fibrin can be formed Once thrombin is present fibrin can be formed. Thrombin leads to numerous other important events, including activation and recruitment of other platelets to the injury site.
7 This scanning electron micrograph shows the consequences of exposure of the sub endothelial basement membrane. Once collagen is presented to platelets under mechanical shear, platelet activation and adhesion occur. Fibrin assembly will shortly follow.
8 Overview of Haemostasis INJURYCollagen ExposureTissue FactorVASOCONSTRICTIONPlatelet Adhesion and release reactionSerotoninPlatelet PhospolipidCoagulationThromboxane A2 ADPPlatelet aggregationThrombinPrimary haemostatic plugFibrinStable haemostatic plugFibrinolysis
10 Within a few minutes the entire injury site is covered by confluent activated platelets.
11 PRACTICAL APPROACH TO A CHILD WITH BLEEDING HISTORY HISTORY – HISTORY – HISTORY>AGE OF ONSET> SEX>FREQUENCY>LOCATION / TYPE OF BLEEDING>DURATION OF BLEEDING> MEDICATIONS> ASSOCIATED SYMPTOMS> REVIEW OF SYSTEMS
12 Approach to a bleeding patient What is the type of bleeding disorder?Primary hemostasis – Vascular causesPlatelets-Number vs.FunctionFibrin formation – clotting factorsPremature clot dissolution- post clot formation
13 . Approach to a bleeding patient Is a bleeding tendency present?Easy BruisingMucosal bleedingMenorrhagiaSurgical Hemorrhage – Procedure vs.DiathesisPostpartum HemorrhageJoint and Muscle bleed –Severity of trauama
14 . Approach to a bleeding patient Is the disorder Familial or Acquired?Family history – MOTHER & OTHERFEMALE MEMBERS IN THE IMMEDIATE FAMILY – Detailed Menstrual history
15 Vascular causes –First and foremost rule out infectious causes – “Meningococcemia”Vasculitis – Henoch-Schonlein PurpuraHemangiomas- Kassalback-Merritt syndrome
16 Petechiae and Purpura Infectious– Meningococcemia– Rocky mountain spottedfever– Group A strep– Atypical measles– Echovirus 9, 4, 7– Epstein-Barr virus– Coxsackie virus A9
18 PRACTICAL APPROACH TO A CHILD WITH BLEEDING HISTORY PHYSICAL EXAMINATION-> PETICHEAE>ECHYMOSES>JOINT BLEED &DEEPSEATED HEMATOMAS> HEPATOSPLENOMEGALY>SIGNIFICANT LYMPHADENOPATHY> ACTIVE AND PLAYFUL VS. ILL LOOKING> DYSMORPHIC FEATURES> CAFÉ-AU-LAIT SPOTS>TELANGIECATIC VESSELS>HEMANGIOMAS
19 PRACTICAL APPROACH TO A CHILD WITH BLEEDING HISTORY LABORATORY WORK UP-P.M.D -> C.B.C./PLATELET COUNT>PERIPHERAL SMEAR- MORPHOLOGY> P.T. [Prothrombin time]> a.P.T.T. [ Activated partial thromboplastin time]Hemophilia service --> T.T. [Thrombin time]> Bleeding time>Platelet aggregation studies> Factor assay
20 Pandora’s box: coagulation test The results are as good as the sample is.Standards: Time from sample to test: PT 24 hours ,PTT 4 hours.Blood/citrate ratio: 9 :1.
30 Functions of vWFBinds to platelet receptor GP Ib and to subendothelial structures such as collagen serving as bridge between platelets and subendothelium in damaged vesselsActs as bridge between adjacent platelets in vessels with high shear (arterioles) forming small platelet aggregatesBinds to circulating factor VIII protecting it and prolonging FVIII t1/2 in the circulation from 2 to 8-12 hours
31 Symptoms of vWD Easy bruisability Epistaxis or gingival bleeding MenorrhagiaPost-partum hemorrhagePost-surgical bleedingBleeding post-dental extraction
32 Classification of vWD Sub types of VW Type Partial quantitative deficiency of vWFType 2 Qualitative variants of vWFA Absence of HMW vWF multimersB Same as 2A and increased affinity for platelet gp IbM Abnormal function not caused by absence of HMW multimersN Decreased affinity for factor VIIIType 3 Complete deficiencey of vWF & Behave as SevereHemophilia AClassification of vWD
33 Treatment Guidelines in VWD TYPE12A2B2M2N3TREATMENTDDAVPDDAVP/FVIII-VWFFVIII-VWF
34 DDAVP (1-desamino-8-D-arginine vasopressin) Parenteral form: DDAVP (for IV or SC use, 0.3 ug/kg)Highly concentrated intranasal spray form: Stimate nasal spray ( ug )
43 Minor Bleeding Episodes Early joint bleedsSoft tissue & muscle bleedsNose & gum bleeding not responding to local measuresTreatment of minor bleeding episodes% correctionFVIII : 25 units / kgFIX : 50 units / kg
44 Major Bleeding Episodes Advanced soft tissue & muscle bleedsHead & neck injuriesGastrointestinal bleedingAdvanced joint bleedingTreatment of major bleeding episodes80 – 100 % correctionFVIII : 50 units / kgFIX : 100 units / kg
45 Current ProductsPlasma Products: plasma-derived factor VIII concentratePorcine Factor:Use was halted due to parvovirus/retrovirus sequences discoveredRecombinant products:First Generation: Recombinate, Kogenate, HelixateSecond Generation: Kogenate FS, Helixate FSThird Generation: AdvateDDAVP:Causes release of factor VIII/vWFIncreased factor activity in 30-60”For mild hemophiliacs and mild bleeding symptoms2nd generation has no B domain – shorter molecule
51 Hemophilia Treatment Center Team Members Patient / FamilyHematologistNurseSocial WorkerPhysical TherapistOrthopedistPrimary CareInfectious DiseaseGeneticsPharmacyDentalHepatologist
52 Basis for Comprehensive Care HematologistAssumes overall careMusculoskeletalOrthopedic Surgeon, Physical therapistNursingCoordination of home/clinic care for rapid treatment at the earliest symptoms suggestive of a bleedDentalGenetic CounselingInfectious DiseasePsychosocialsocial worker
53 Role of Hemophilia Treatment Centers State-of-the-art medical treatment for persons with hemophilia through out the life spanEducationResearchOutreachModel of comprehensive care for chronic disease