Coagulopathy in children

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Coagulopathy in children

Dr. Mohammed Saiful IslamResident ( Phase:A),

Department of Paediatric Surgery, BSMMU.Bangladesh.

20/09/2016, Venue: Paediatric surgery class room.

Objectives• To define & understand the extent of

coagulopathy.• A review of coagulation pathways.• Discussions on Platelet disorders.• Key points discussions associated with

coagulation factor disorders.• Perioperative strategies to meet the

challenges of coagulopathy in case of children.

Definition:Group of medical

conditions resulting from deficiency of clotting factors, inhibition of coagulation process or excessive activity of the fibrinolytic system.

Reff: de Gruchy 5th

Bleeding disorders

Coagulopathy

Extent of Coagulopaty:

• VESSEL WALL ABNORMALITIES:

• PLATELETS DISORDER:

• COAGULATION DISORDER:

INTRINSIC EXTRINSIC

PROTHROMBIN(II) THROMBIN(III)

FIBRINOGEN

FIBRIN

(I)V

X

Tissue ThromboplastinCollagen

XIIXI

IXVIII

VII

PTPTT Vit.K, Liver

CLOTTING MECHANISM (Easy to keep in mind)

PLATELETS DISORDERS:

• QUANTITATIVE PLATELETS DISORDERs.

• QUALITATIVE PLATELET DYSFUNCTION.

QUANTITATIVE PLATELETS DISORDER (Thrombocytopenia)

Mechanisms:1. Failure of megakaryocytic

maturation.2. Excessive platelets consumption

after their release into circulation i.e ITP, DIC etc.

3. Platelets sequestration in enlarged spleen i.e HYPERSPLEENISM.

QUANTITATIVE PLATELETS DISORDER (Continued…)

S/S:• Petechial cutaneous bleeding,• Intracranial bleeding,• Oozing from mucus membrane &

skin surface. Lab: Decreased platelets count and prolong bleeding time.

(Thrombocytopenia) Causes:

Marrow Disorder• Aplastic anemia.• Haematological

malignancy.• Myelodysplastic

disorder.• B12 defficiency.

Non Marrow Disorder• Immune disorders.• ITP, Drug induced.• Sec: ALL, SLE.• Post transfusion.• DIC.• Human immunodeficiency syndrome. • Hyperspleenism.• Haemangiomas.• Sepsis.• Viral infection.

Thrombocytopenia (Continued…)

• Management:– Rx Underlying cause.– Platelet transfusion.

IDIOPATHIC THROMBOCYTOPENIC PURPURA(ITP)

• An autoimmune antibody IgG is formed against unknown antigen of platelets membrane/surface.

• Antipletelet antibody binds to complement, but platelets are not destroyed by direct lysis.

• Destruction takes place in spleen, where spleenic macrophages destroyes antibody coated platelets.

IDIOPATHIC THROMBOCYTOPENIC PURPURA. (Continued…)

Clinical Features: Often precipitated by viral infection

and usually self limiting. Asymptomatic, not febrile. Present with mucosal/skin bleeding,

mennorrhagia, purpura, petechiae. Females are more affected (2:1

male/female ratio).

IDIOPATHIC THROMBOCYTOPENIC PURPURA. (Continued…) Δ LAB:Platelets below 10,000 /ml.Bone marrow will appear normal.RxPREDNISONONE: 1-2 mg/kg/day. Immunoglobulin: 1g/kg/day 2-3 days.DANAZOLE: 600mg/day response rate is 50%IMMUNOSUPPERESSIVE DRUGS: i-e vincristine,

vinblastine, azathioprine, cyclosporin, cyclophosphomide.

SPLEENECTOMY.Prognosis:

Good, if disease is initially controlled with prednisolone, Spleenectomy is definite Rx.

QUALITATIVE PLATELET DISORDER

CONGENITAL:• Glanzmann’s

thrombasthenia.• Bernard souliar

syndrome.• Von Willibrand’s

disease.

ACQUIRED • Myeloproliferative disorder.• Uremia.• Drugs i-e NSAIDS Aspirin.• Autoantibody.• Fibrin degradation products.

QUALITATIVE PLATELET DISORDERGLANZMANN’s THROMBASTHENIA:Autosomal recessive disorder.Lack of receptors (glycoprotein Ib & IIIa)

for fibrinogen on platelets.Platelets fails to aggregate in respons to

ADP, collagen, thrombin.Clinical Features: Mucosal bleedingLAB:Platelets no’s and morphology are normal B.T is prolonged Rx:Platelet transfusion

QUALITATIVE PLATELET DISORDERBERNARD SOULIER SYNDROME:Autosomal recessive intrinsic platelets disorder.Due to lack of glycoprotein (Gp1b), receptor for

vonWillibrand’s factor.Clinical Features:Presents with mucosal bleeding and post

operative oozes.LAB:Thrombocytopenia may be present, and Platelets

are abnormally large in size.BT is prolonged Von Willibrand’s factor Normal .Rx:Platelet transfusion

QUALITATIVE PLATELET DISORDERVON-WILLIBRAND’S DISEASE:

• Autosomal dominant (gene for vWF is located on chromosome 12.)

• vWF is synthesized by endothelial cells and megakaryocytes.

• It acts as carrier protein for factor VIII by non-covalent bond.

• A defect therefore leads to decreased plasma factor VIII level.

VON-WILLIBRAND’S DISEASE (Continued….)

• It also forms bridges between platelets and sub endothelium.

• There fore defect of vWF leads to prolonged bleeding.


Figure: Structure and function of factor VIII-von Willebrand factor (vWF) complex.


Clinical Features:• Mucosal bleeding (mild-massive)LAB:• Reduced level of vWF.• Secondary reduction in factor VIII.• Prolonged bleeding time (B.T).


Rx:• MILD HAEMORRHAGES:

Desmopressin 0.3 μg/kg, after which vWF levels usually raise 3 units in 30-90 minutes.

• MASSIVE HAEMORRHAGES:Factor VIII transfusion.

COAGULATION DISORDER: Coagulation factor disorder can be either due to single factor def., i.e. a “congenital deficiency”, e.g factor VIII resulting in HAEMOPHILIA-A or due to multiple factor defficiency which is an ‘’acquired condition” e.g Sec: to liver disease or warfarin therapy.

• HEAMOPHILIA – A (CLASSIC / TRUE HAEMOPHILIA)

• HAEMOPHILLIA – B (CHRISTMAS DISEASE).

• Other specific factor deficiency (e.g. Factor І, ІІ, ІІІ etc. deficiency).

COAGULATION DISORDER:CONGENITAL BLEEDING DISORDER:

COAGULATION DISORDERACQUIRED BLEEDING DISORDER

• DIC• Liver disease • Renal disease• Vitamin K deficiency• Massive blood transfusion• Anticoagulant drugs.

HAEMOPHILIA• X-linked recessive disorder. • Due to deficiency of factor VIII (Haemophiia A)

or factor IX (Haemophiia B) .

HAEAMOPHILIA (Continued….)

C/F:• Bleeding occurs as bruising at the age of 6

months.• Trauma results in excessively bleeding.• Recurrent bleeding /hemorrhage in knee, elbow,

ankle, and hip. (Hemarthrosis).• Mucus membrane /internal bleeding of mouth,

lips, gums, brain and kidney also occur.• Muscle haematoma esp. calf and Psoas muscle .Rx• Cryoprecipitate or Factor VIII infusion ( in

Haemophiia A) & Factor IX infusion (in Haemophilia B)


LONG TERM COMPLICATIONsCOMPLICATION due to repeated

hemorrhage:– Arthropathy of large joints eg knee, elbow– Muscle atrophy due to haematoma– Mononeuropathy due to pressure of

haematoma.COMPLICATION due to therapy

– Antifactor VIII antibody develops.– Virus transmission Hepatitis B,C + HIV etc.


Figure: Haemarthrosis & Arthropathy.

SURGERY IN A CHILD WITH HAEMOPHILLIA:

• In minor Surgery:Factor VIII should be at least 50% of the normal predicted value.

• In Major Surgery: Factor VIII should be 100%. Half life of Factor VIII is 8 to 12 hours. Factor VIII should be substituted just

before surgery & 12 hours after the initial dose ( Target is to pass the time of reactionary haemorrhage).

Calculation of Factor VІІІ

% Desired Χ Wt in Kg

• Amount of Factor VІІІ = K

Here, value of K is 1.5 for Haemophilia A & 0.75 for Haemophilia B.

DISSAMINATED INTRAVASCULAR COAGULATION

Disseminated Intravascular Coagulation (DIC) is a thrombo-haemorrhagic disorder appearing as a secondary complication of several diseases, where there is consumption of platelets and clotting factors in the circulation as well as secondary activation of plasminogen fibrinolytic system.

DIC:CAUSES

A Infection: 1. Gram negative

septicaemia 2.

Meningococcaemia3.

Pneumococcaemia4.Malaria

B. Massive injury:

1. Massive trauma

2. Extensive burn (severe burn)

3. Extensive surgery (brain, lung, prostate, etc.)

C. Carcinoma: 1. Pancreas 2. Lungs 3. Stomach 4. Prostate, etc.

D. Others: 1. Transfusion

reaction 2. Fat embolism 3. Snake bite 4. Shock, etc.

Fig: Pathophysiology of disseminated intravascular coagulation.

DIC (Continued….)CLINICAL FEATURES:• Bleeding & thrombosis, bleeding is more

than thrombosis.• Features of complications.LAB:• Thrombocytopenia• Prolong PT• APTT may be normal/increased• Low fibrinogen• Increased level FDP/D-dimmer

Treatment of DICRx. Underlying cause.General Measures: Correction & prevention of :• Dehydration• Renal failure• Acidosis and • ShockReplacement:• Platelets transfusion if platelets counts below

10,000/ml• Cryoprecipitate to maintain plasma fibrinogen level

above 150 mg/dl • FFP• Heparin, if there is DVT, Pulmonary thrombosis.

WARCRAFT FOR SURGERY IN A CHILD HAVING

ANTICOAGULANT THERAPY

• If patient on oral anticoaguant:Dose adjustment for INR within 2.5 to 3.5.During surgery INR should be 1.5 or less.

• If patient on Parenteral anticoaguant:Per operative APTT should be within 1.5 to

2.5 times of normal range.

WARCRAFT FOR SURGERY (Continued….)

• During emergency surgery: Inj. Vitamin K should be administered.

FFP & Factor concentrate of ІІ, VІІ, ІX & X should be kept in hand.

Per operative & Post operative monitoring of PT, INR.

WARCRAFT FOR SURGERY (Continued….)

• During elective surgery:• Minor surgery:

» Oral anticoagulant should be stopped 2 to 3 days prior to surgery.

• Major surgery:» Oral anticoagulant should be stopped 4

to 5 days prior to surgery.» Then low molecular weight heparin

should be started.» Heparin should be stopped 4 hours prior

to surgery & restarted 12 hours after surgery.

THANK YOU

Coagulopathy in children

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