SlideShare a Scribd company logo
HAEMORRHAGIC
DISORDERS IN
NEWBORN
PRESENTED
BY :
VRUTI PATEL,
HAEMORRHAGIC DISEASES
OF THE NEWBORN
It is a syndrome characterized by
spontaneous internal or external bleeding. In
neonates, there is decreased activity of
several clotting factors and diminished
platelet function.
CAUSES
• Abnormalities of clotting factors
• Deficiencies of vitamin k dependent factors –
II, VII, IX, X. it usually occurs between D2 and
D5 and common in preterm and breast fed
babies. Deficiency of protein C is also
responsible
• Drugs – received by mother during pregnancy
– phenytoin, coumarin compounds,
salicylates (affects vit K function).
• DIC – due to infection, Anoxia, shock.
2. Disturbances of clotting
- Related to DIC due to infection, shock,
anoxia, NEC, renal vein thrombosis, use
of IV canula.
3. Inherited abnormalities of C.F.
a. X-LINKED RECESSIVE DISEASES-
i. Hemophilia-A : Factor VIII deficiency.
ii. Hemophilia-B : Factor IX deficiency.
b. AUTOSOMAL DOMINANT DISEASES:
i. Von Willebrand disease – Deficiency of VWF
which is a carrier of factor VIII & as a platelet
aggregation agent.
c. AUTOSOMAL RECESSIVE DISEASES:
I. Severe factor VII & factor XIII deficiency –
intracranial hemorrhage in neonates
II.Factor XI deficiency – unpredictable bleeding
during surgery/trauma.
III. VWD Type III
B. Platelet problems:
1. Qualitative disorders:
- Glanzman’s thrombasthenia.
- Bernard-Soulier syndrome
- Platelet type VWD
2. Quantitive disorders:
- Immune thrombocytipenia
- Matrnal Preeclampsia, HELLP syndrome
or severe uteroplacental insuffuciency.
- DIC due to infection or asphyxia.
- Inherited bone marrow failure syndromes :
Fanconi anemia & congenital
amegakaryocytic thrombocytopenia
- Congenital leukemia
- Inherited thrombocytopenia syndromes :
gray platelet syndrome
- Macrothrombocytopenias : May-Hegglin
syndr.
- Platelet consumption in clots/ vascular
disorders eg. Vascular malformations,
NEC.
C. Vascular origin:
- Pulmonary haemorrhage
- A-V malformations
- CNS haemorrhage
- Hemangiomas.
DIAGNOSTIC WORKUP
A.History
- Family h/o bleeding disorders
- Maternal medications
- Pregnancy & birth history
- Maternal h/o infant with bleeding disorder
- Any medications, procedures, anomalies
in infant
B. Examination:
First diagnose whether the infant is Sick or Well
1.Sick infant:
- DIC
- Bacterial/ viral infections.
2. Well infant:
- Vit K deficiency
- Isolated C.F. deficiencies
- Immune thrombocytopenia
- Maternal blood in infant’s GIT.
3. Patchiae, ecchymosis, mucosal bleeding:
Platelet problem
4. Large bruises: DIC, C.F deficiencies, liver
diseases
5. Enlarged spleen : Possible congenital
infections or erythroblastosis.
6. Jaundice : Sepsis, liver diseases,
resorption of large hematoma.
C. Laboratory tests:
1.Apt test :
- To rule out maternal blood in infant’s GIT
- Done in otherwise well infant with only GI
bleeding.
2. PBS :
- DIC- fragmented RBCs
- Congenital macrothrombocytopenias –
large platelets.
3. PT
4. APTT
5. D-Dimer assays: Measure fibrin
degradation products in DIC & Liver
diseases causing defective clearing of
fibrin split products.
6. Specific factor assays & Von Willebrand
assay: For patients with + ve family h/o.
TREATMENT OF
BLEEDING
A. Inj Vitamin K1 (Aquaminophyton)
- 1 mg IV or IM if not given at birth.
- Infants on TPN
- Infants on Antibiotics > 2 weeks: at least
0.5mg Vit K weekly.
- Preferred rather than FFP for prolonged
PT & PTT, FFP should be reserved for
emergencies.
B. FFP:
- 10ml/kg IV for active bleeding
- Repeated 8-12 hrly as needed.
- Replaces C.F. immediately.
C. Platelets:
- 1 Unit of platelet raises count by 50,000-
10,000/mm3.
- Platelet count slowly decreases if stores 3-
5 days.
D. Fresh whole blood:
- 10ml/kg
- Can be repeated after 6-8 hrs as needed.
E. Clotting factor concetrates
- Severe VWD :
- VWF containing plasma derived factor VIII
concetrate.
- Known deficiency of factor VIII or IX :
Recombinent DNA derived factor VIII and
IX concetrate
F. Disorders due to problems other than
hemostatic proteins :
- Rule out the underlying possibilities
- eg. Infection, Liver rupture, catheter, NEC.
G. T/t of specific disorders :
1.DIC :
- Treat the underlying cause i.e. sepsis, NEC
- Make sure that Vit K1 has been given.
- Platelets/ FFP to keep platelet counts >
50,000/ml and to stop bleeding.
- If bleeding persists,
i. Exchange transfusion with fresh whole
blood /Packed RBC/Platelets/FFP
ii. Continuous transfusion with platelets,
packed RBCs or FFP as needed.
iii. For hypofibrinogenemia : Cryoprecipitate
(10ml/kg)
Haemorrhagic disorders

More Related Content

What's hot

Nephrotic And Nephritic Syndrome 2008
Nephrotic And Nephritic Syndrome 2008Nephrotic And Nephritic Syndrome 2008
Nephrotic And Nephritic Syndrome 2008
Dang Thanh Tuan
 
Thrombocytopenia
ThrombocytopeniaThrombocytopenia
Thrombocytopenia
fitango
 
Nephrotic syndrome
Nephrotic syndrome Nephrotic syndrome
Nephrotic syndrome
Abhay Mange
 

What's hot (20)

Anemia And Its Classification
Anemia And Its ClassificationAnemia And Its Classification
Anemia And Its Classification
 
Nephrotic And Nephritic Syndrome 2008
Nephrotic And Nephritic Syndrome 2008Nephrotic And Nephritic Syndrome 2008
Nephrotic And Nephritic Syndrome 2008
 
Thalassemia.
Thalassemia.Thalassemia.
Thalassemia.
 
Thrombocytopenia
ThrombocytopeniaThrombocytopenia
Thrombocytopenia
 
Hemophilia : causes, symptoms, diagnosis and treatment
Hemophilia : causes, symptoms, diagnosis and treatmentHemophilia : causes, symptoms, diagnosis and treatment
Hemophilia : causes, symptoms, diagnosis and treatment
 
Hemophilia
HemophiliaHemophilia
Hemophilia
 
Nephrotic syndrome
Nephrotic syndrome Nephrotic syndrome
Nephrotic syndrome
 
Pernicious anemia
Pernicious anemia Pernicious anemia
Pernicious anemia
 
Leukemia
LeukemiaLeukemia
Leukemia
 
Vitamin k deficiency of newborn
Vitamin k deficiency of newbornVitamin k deficiency of newborn
Vitamin k deficiency of newborn
 
Polycythemia
PolycythemiaPolycythemia
Polycythemia
 
Laboratory diagnosis of anemia
Laboratory diagnosis of anemiaLaboratory diagnosis of anemia
Laboratory diagnosis of anemia
 
Hemophilia
HemophiliaHemophilia
Hemophilia
 
Itp
ItpItp
Itp
 
Von willebrand disease
Von willebrand diseaseVon willebrand disease
Von willebrand disease
 
Hemolytic anemia
Hemolytic anemiaHemolytic anemia
Hemolytic anemia
 
Thrombocytopenia
ThrombocytopeniaThrombocytopenia
Thrombocytopenia
 
Heart failure in children
Heart failure in childrenHeart failure in children
Heart failure in children
 
Thrombocytopenia
Thrombocytopenia Thrombocytopenia
Thrombocytopenia
 
Pathophysiology of leukemia
Pathophysiology of leukemiaPathophysiology of leukemia
Pathophysiology of leukemia
 

Viewers also liked

Approach to gynaecology patient
Approach to gynaecology patientApproach to gynaecology patient
Approach to gynaecology patient
obsgynhsnz
 
Clinical Approach to Gynecological Patient (part-1)
Clinical Approach to Gynecological Patient (part-1)Clinical Approach to Gynecological Patient (part-1)
Clinical Approach to Gynecological Patient (part-1)
drmcbansal
 

Viewers also liked (11)

The ALTERNATIVE
The ALTERNATIVEThe ALTERNATIVE
The ALTERNATIVE
 
Fscpp
FscppFscpp
Fscpp
 
Mekanisme pembekuan darah
Mekanisme pembekuan darahMekanisme pembekuan darah
Mekanisme pembekuan darah
 
Approach to gynaecology patient
Approach to gynaecology patientApproach to gynaecology patient
Approach to gynaecology patient
 
Neonatal jaundice(reference msia cpg)
Neonatal jaundice(reference msia cpg)Neonatal jaundice(reference msia cpg)
Neonatal jaundice(reference msia cpg)
 
Clinical Approach to Gynecological Patient (part-1)
Clinical Approach to Gynecological Patient (part-1)Clinical Approach to Gynecological Patient (part-1)
Clinical Approach to Gynecological Patient (part-1)
 
Neonatal convulsion
Neonatal convulsionNeonatal convulsion
Neonatal convulsion
 
Vitamin k
Vitamin kVitamin k
Vitamin k
 
Haemorrhagic and Haemolytic of Newborn Diseases
Haemorrhagic and Haemolytic of Newborn DiseasesHaemorrhagic and Haemolytic of Newborn Diseases
Haemorrhagic and Haemolytic of Newborn Diseases
 
Approach to bleeding disorder (coagulation defects) in children
Approach to bleeding disorder (coagulation defects) in childrenApproach to bleeding disorder (coagulation defects) in children
Approach to bleeding disorder (coagulation defects) in children
 
Coagulation
CoagulationCoagulation
Coagulation
 

Similar to Haemorrhagic disorders

Bleedingneonate sandip1
Bleedingneonate sandip1Bleedingneonate sandip1
Bleedingneonate sandip1
Sandip Gupta
 
Hematology lect 3
Hematology lect 3Hematology lect 3
Hematology lect 3
Miami Dade
 
medicine.Bleeding disorders.(dr.sabir) (new powerpoint)
medicine.Bleeding disorders.(dr.sabir) (new powerpoint)medicine.Bleeding disorders.(dr.sabir) (new powerpoint)
medicine.Bleeding disorders.(dr.sabir) (new powerpoint)
student
 
Medicine.Bleeding disorders.(dr.sabir)
Medicine.Bleeding disorders.(dr.sabir)Medicine.Bleeding disorders.(dr.sabir)
Medicine.Bleeding disorders.(dr.sabir)
student
 

Similar to Haemorrhagic disorders (20)

Bleeding neonate
Bleeding neonateBleeding neonate
Bleeding neonate
 
Bleedingneonate sandip1
Bleedingneonate sandip1Bleedingneonate sandip1
Bleedingneonate sandip1
 
Bleedingneonate sandip1
Bleedingneonate sandip1Bleedingneonate sandip1
Bleedingneonate sandip1
 
Approach to bleeding neonate final
Approach to bleeding neonate finalApproach to bleeding neonate final
Approach to bleeding neonate final
 
Approach to a bleedinf neonate Dr.Rashmi..pptx
Approach to a bleedinf neonate Dr.Rashmi..pptxApproach to a bleedinf neonate Dr.Rashmi..pptx
Approach to a bleedinf neonate Dr.Rashmi..pptx
 
hemorrhagic disease of newborn
hemorrhagic disease of newbornhemorrhagic disease of newborn
hemorrhagic disease of newborn
 
hemorrhagic disease of newborn
hemorrhagic disease of newbornhemorrhagic disease of newborn
hemorrhagic disease of newborn
 
myseminar-210817133841.pdf
myseminar-210817133841.pdfmyseminar-210817133841.pdf
myseminar-210817133841.pdf
 
HDM newborn.pptx
HDM newborn.pptxHDM newborn.pptx
HDM newborn.pptx
 
Vitamin k & its applied aspects
Vitamin k & its applied aspectsVitamin k & its applied aspects
Vitamin k & its applied aspects
 
Haemorrhagic disease of newborn
Haemorrhagic disease of newbornHaemorrhagic disease of newborn
Haemorrhagic disease of newborn
 
Coagulation Disorders
Coagulation DisordersCoagulation Disorders
Coagulation Disorders
 
Hematology lect 3
Hematology lect 3Hematology lect 3
Hematology lect 3
 
Hemorrhagic Disease of the newborn 2018.ppt
Hemorrhagic Disease of the newborn 2018.pptHemorrhagic Disease of the newborn 2018.ppt
Hemorrhagic Disease of the newborn 2018.ppt
 
Neonatal hematological disorders
Neonatal hematological disordersNeonatal hematological disorders
Neonatal hematological disorders
 
Anuupam ppt for bleeding child
Anuupam ppt for bleeding childAnuupam ppt for bleeding child
Anuupam ppt for bleeding child
 
medicine.Bleeding disorders.(dr.sabir) (new powerpoint)
medicine.Bleeding disorders.(dr.sabir) (new powerpoint)medicine.Bleeding disorders.(dr.sabir) (new powerpoint)
medicine.Bleeding disorders.(dr.sabir) (new powerpoint)
 
Medicine.Bleeding disorders.(dr.sabir)
Medicine.Bleeding disorders.(dr.sabir)Medicine.Bleeding disorders.(dr.sabir)
Medicine.Bleeding disorders.(dr.sabir)
 
Infective endocarditis-Neonate
 Infective endocarditis-Neonate Infective endocarditis-Neonate
Infective endocarditis-Neonate
 
Bleeding in newborns
Bleeding in newbornsBleeding in newborns
Bleeding in newborns
 

More from vruti patel (14)

Handbook of neonatology
Handbook of neonatologyHandbook of neonatology
Handbook of neonatology
 
Calculation of fluid
Calculation of fluidCalculation of fluid
Calculation of fluid
 
Unit 5 nursing process
Unit   5 nursing processUnit   5 nursing process
Unit 5 nursing process
 
Unit 1 introdution to health
Unit 1   introdution to healthUnit 1   introdution to health
Unit 1 introdution to health
 
Breast complications
Breast complications  Breast complications
Breast complications
 
Nicu ppt
Nicu pptNicu ppt
Nicu ppt
 
menopause
menopausemenopause
menopause
 
Menstrual abnormalities
Menstrual abnormalitiesMenstrual abnormalities
Menstrual abnormalities
 
Material management
Material managementMaterial management
Material management
 
fiscal & Financial planning
fiscal & Financial planningfiscal & Financial planning
fiscal & Financial planning
 
Budget and financialaudit
Budget and financialauditBudget and financialaudit
Budget and financialaudit
 
Puerperal sepsis
Puerperal sepsisPuerperal sepsis
Puerperal sepsis
 
Diagnosis of pregnancy
Diagnosis of pregnancyDiagnosis of pregnancy
Diagnosis of pregnancy
 
Physiological changes during pregnancy
Physiological changes during pregnancyPhysiological changes during pregnancy
Physiological changes during pregnancy
 

Recently uploaded

Cell structure slideshare.pptx Unlocking the Secrets of Cells: Structure, Fun...
Cell structure slideshare.pptx Unlocking the Secrets of Cells: Structure, Fun...Cell structure slideshare.pptx Unlocking the Secrets of Cells: Structure, Fun...
Cell structure slideshare.pptx Unlocking the Secrets of Cells: Structure, Fun...
ananyagirishbabu1
 
Benefits of Dentulu's Salivary Testing.pptx
Benefits of Dentulu's Salivary Testing.pptxBenefits of Dentulu's Salivary Testing.pptx
Benefits of Dentulu's Salivary Testing.pptx
Dentulu Inc
 
Integrated Mother and Neonate Childwood Illness Health Care
Integrated Mother and Neonate Childwood Illness  Health CareIntegrated Mother and Neonate Childwood Illness  Health Care
Integrated Mother and Neonate Childwood Illness Health Care
ASKatoch1
 
Urinary Elimination BY ANUSHRI SRIVASTAVA.pptx
Urinary Elimination BY ANUSHRI SRIVASTAVA.pptxUrinary Elimination BY ANUSHRI SRIVASTAVA.pptx
Urinary Elimination BY ANUSHRI SRIVASTAVA.pptx
AnushriSrivastav
 
Production.pptx\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\
Production.pptx\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\Production.pptx\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\
Production.pptx\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\
DanielOliver74
 
BOWEL ELIMINATION BY ANUSHRI SRIVASTAVA.pptx
BOWEL ELIMINATION BY ANUSHRI SRIVASTAVA.pptxBOWEL ELIMINATION BY ANUSHRI SRIVASTAVA.pptx
BOWEL ELIMINATION BY ANUSHRI SRIVASTAVA.pptx
AnushriSrivastav
 
ASSISTING WITH THE USE OF BED PAN BY ANUSHRI SRIVASTAVA.pptx
ASSISTING WITH THE USE OF BED PAN BY ANUSHRI SRIVASTAVA.pptxASSISTING WITH THE USE OF BED PAN BY ANUSHRI SRIVASTAVA.pptx
ASSISTING WITH THE USE OF BED PAN BY ANUSHRI SRIVASTAVA.pptx
AnushriSrivastav
 

Recently uploaded (20)

Cell structure slideshare.pptx Unlocking the Secrets of Cells: Structure, Fun...
Cell structure slideshare.pptx Unlocking the Secrets of Cells: Structure, Fun...Cell structure slideshare.pptx Unlocking the Secrets of Cells: Structure, Fun...
Cell structure slideshare.pptx Unlocking the Secrets of Cells: Structure, Fun...
 
Benefits of Dentulu's Salivary Testing.pptx
Benefits of Dentulu's Salivary Testing.pptxBenefits of Dentulu's Salivary Testing.pptx
Benefits of Dentulu's Salivary Testing.pptx
 
Integrated Mother and Neonate Childwood Illness Health Care
Integrated Mother and Neonate Childwood Illness  Health CareIntegrated Mother and Neonate Childwood Illness  Health Care
Integrated Mother and Neonate Childwood Illness Health Care
 
Valle Egypt Illustrates Consequences of Financial Elder Abuse
Valle Egypt Illustrates Consequences of Financial Elder AbuseValle Egypt Illustrates Consequences of Financial Elder Abuse
Valle Egypt Illustrates Consequences of Financial Elder Abuse
 
Urinary Elimination BY ANUSHRI SRIVASTAVA.pptx
Urinary Elimination BY ANUSHRI SRIVASTAVA.pptxUrinary Elimination BY ANUSHRI SRIVASTAVA.pptx
Urinary Elimination BY ANUSHRI SRIVASTAVA.pptx
 
Virtual Health Platforms_ Revolutionizing Patient Care.pdf
Virtual Health Platforms_ Revolutionizing Patient Care.pdfVirtual Health Platforms_ Revolutionizing Patient Care.pdf
Virtual Health Platforms_ Revolutionizing Patient Care.pdf
 
💃Joint ❤89011-83002❤ #ℂALL #gIRLS Ludhiana Escorts by ✔️🍑💃Hotel #cALL #gIRLS...
💃Joint ❤89011-83002❤ #ℂALL #gIRLS Ludhiana Escorts  by ✔️🍑💃Hotel #cALL #gIRLS...💃Joint ❤89011-83002❤ #ℂALL #gIRLS Ludhiana Escorts  by ✔️🍑💃Hotel #cALL #gIRLS...
💃Joint ❤89011-83002❤ #ℂALL #gIRLS Ludhiana Escorts by ✔️🍑💃Hotel #cALL #gIRLS...
 
Digital Healthcare: The Future of Medical Consultations
Digital Healthcare: The Future of Medical ConsultationsDigital Healthcare: The Future of Medical Consultations
Digital Healthcare: The Future of Medical Consultations
 
Call Girls in Jaipur (Rajasthan) call me [🔝89011-83002🔝] Escort In Jaipur ℂal...
Call Girls in Jaipur (Rajasthan) call me [🔝89011-83002🔝] Escort In Jaipur ℂal...Call Girls in Jaipur (Rajasthan) call me [🔝89011-83002🔝] Escort In Jaipur ℂal...
Call Girls in Jaipur (Rajasthan) call me [🔝89011-83002🔝] Escort In Jaipur ℂal...
 
What can we really do to give meaning and momentum to equality, diversity and...
What can we really do to give meaning and momentum to equality, diversity and...What can we really do to give meaning and momentum to equality, diversity and...
What can we really do to give meaning and momentum to equality, diversity and...
 
Breaking Down Oppositional Defiant Disorder Treatments
Breaking Down Oppositional Defiant Disorder TreatmentsBreaking Down Oppositional Defiant Disorder Treatments
Breaking Down Oppositional Defiant Disorder Treatments
 
Mental Health Startup Pitch Deck Presentation
Mental Health Startup Pitch Deck PresentationMental Health Startup Pitch Deck Presentation
Mental Health Startup Pitch Deck Presentation
 
Production.pptx\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\
Production.pptx\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\Production.pptx\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\
Production.pptx\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\\
 
Homeopathy Medicine for Diabetes_ Balancing Blood Sugar .pdf
Homeopathy Medicine for Diabetes_ Balancing Blood Sugar .pdfHomeopathy Medicine for Diabetes_ Balancing Blood Sugar .pdf
Homeopathy Medicine for Diabetes_ Balancing Blood Sugar .pdf
 
BOWEL ELIMINATION BY ANUSHRI SRIVASTAVA.pptx
BOWEL ELIMINATION BY ANUSHRI SRIVASTAVA.pptxBOWEL ELIMINATION BY ANUSHRI SRIVASTAVA.pptx
BOWEL ELIMINATION BY ANUSHRI SRIVASTAVA.pptx
 
Storage_of _Bariquin_Components_in_Storage_Boxes.pptx
Storage_of _Bariquin_Components_in_Storage_Boxes.pptxStorage_of _Bariquin_Components_in_Storage_Boxes.pptx
Storage_of _Bariquin_Components_in_Storage_Boxes.pptx
 
pathology seminar presentation best ppt by .pptx
pathology seminar presentation best ppt by  .pptxpathology seminar presentation best ppt by  .pptx
pathology seminar presentation best ppt by .pptx
 
Master the Art of Yoga with Joga Yoga Training
Master the Art of Yoga with Joga Yoga TrainingMaster the Art of Yoga with Joga Yoga Training
Master the Art of Yoga with Joga Yoga Training
 
CHAPTER- 1 SEMESTER - V NATIONAL HEALTH PROGRAMME RELATED TO CHILD.pdf
CHAPTER- 1 SEMESTER - V NATIONAL HEALTH PROGRAMME RELATED TO CHILD.pdfCHAPTER- 1 SEMESTER - V NATIONAL HEALTH PROGRAMME RELATED TO CHILD.pdf
CHAPTER- 1 SEMESTER - V NATIONAL HEALTH PROGRAMME RELATED TO CHILD.pdf
 
ASSISTING WITH THE USE OF BED PAN BY ANUSHRI SRIVASTAVA.pptx
ASSISTING WITH THE USE OF BED PAN BY ANUSHRI SRIVASTAVA.pptxASSISTING WITH THE USE OF BED PAN BY ANUSHRI SRIVASTAVA.pptx
ASSISTING WITH THE USE OF BED PAN BY ANUSHRI SRIVASTAVA.pptx
 

Haemorrhagic disorders

  • 2. HAEMORRHAGIC DISEASES OF THE NEWBORN It is a syndrome characterized by spontaneous internal or external bleeding. In neonates, there is decreased activity of several clotting factors and diminished platelet function.
  • 3. CAUSES • Abnormalities of clotting factors • Deficiencies of vitamin k dependent factors – II, VII, IX, X. it usually occurs between D2 and D5 and common in preterm and breast fed babies. Deficiency of protein C is also responsible • Drugs – received by mother during pregnancy – phenytoin, coumarin compounds, salicylates (affects vit K function). • DIC – due to infection, Anoxia, shock.
  • 4. 2. Disturbances of clotting - Related to DIC due to infection, shock, anoxia, NEC, renal vein thrombosis, use of IV canula. 3. Inherited abnormalities of C.F. a. X-LINKED RECESSIVE DISEASES- i. Hemophilia-A : Factor VIII deficiency. ii. Hemophilia-B : Factor IX deficiency.
  • 5. b. AUTOSOMAL DOMINANT DISEASES: i. Von Willebrand disease – Deficiency of VWF which is a carrier of factor VIII & as a platelet aggregation agent. c. AUTOSOMAL RECESSIVE DISEASES: I. Severe factor VII & factor XIII deficiency – intracranial hemorrhage in neonates II.Factor XI deficiency – unpredictable bleeding during surgery/trauma. III. VWD Type III
  • 6. B. Platelet problems: 1. Qualitative disorders: - Glanzman’s thrombasthenia. - Bernard-Soulier syndrome - Platelet type VWD
  • 7. 2. Quantitive disorders: - Immune thrombocytipenia - Matrnal Preeclampsia, HELLP syndrome or severe uteroplacental insuffuciency. - DIC due to infection or asphyxia. - Inherited bone marrow failure syndromes : Fanconi anemia & congenital amegakaryocytic thrombocytopenia
  • 8. - Congenital leukemia - Inherited thrombocytopenia syndromes : gray platelet syndrome - Macrothrombocytopenias : May-Hegglin syndr. - Platelet consumption in clots/ vascular disorders eg. Vascular malformations, NEC.
  • 9. C. Vascular origin: - Pulmonary haemorrhage - A-V malformations - CNS haemorrhage - Hemangiomas.
  • 10. DIAGNOSTIC WORKUP A.History - Family h/o bleeding disorders - Maternal medications - Pregnancy & birth history - Maternal h/o infant with bleeding disorder - Any medications, procedures, anomalies in infant
  • 11. B. Examination: First diagnose whether the infant is Sick or Well 1.Sick infant: - DIC - Bacterial/ viral infections. 2. Well infant: - Vit K deficiency - Isolated C.F. deficiencies - Immune thrombocytopenia - Maternal blood in infant’s GIT.
  • 12. 3. Patchiae, ecchymosis, mucosal bleeding: Platelet problem 4. Large bruises: DIC, C.F deficiencies, liver diseases 5. Enlarged spleen : Possible congenital infections or erythroblastosis. 6. Jaundice : Sepsis, liver diseases, resorption of large hematoma.
  • 13. C. Laboratory tests: 1.Apt test : - To rule out maternal blood in infant’s GIT - Done in otherwise well infant with only GI bleeding. 2. PBS : - DIC- fragmented RBCs - Congenital macrothrombocytopenias – large platelets.
  • 14. 3. PT 4. APTT 5. D-Dimer assays: Measure fibrin degradation products in DIC & Liver diseases causing defective clearing of fibrin split products. 6. Specific factor assays & Von Willebrand assay: For patients with + ve family h/o.
  • 15.
  • 16.
  • 17. TREATMENT OF BLEEDING A. Inj Vitamin K1 (Aquaminophyton) - 1 mg IV or IM if not given at birth. - Infants on TPN - Infants on Antibiotics > 2 weeks: at least 0.5mg Vit K weekly. - Preferred rather than FFP for prolonged PT & PTT, FFP should be reserved for emergencies.
  • 18. B. FFP: - 10ml/kg IV for active bleeding - Repeated 8-12 hrly as needed. - Replaces C.F. immediately. C. Platelets: - 1 Unit of platelet raises count by 50,000- 10,000/mm3. - Platelet count slowly decreases if stores 3- 5 days.
  • 19. D. Fresh whole blood: - 10ml/kg - Can be repeated after 6-8 hrs as needed. E. Clotting factor concetrates - Severe VWD : - VWF containing plasma derived factor VIII concetrate. - Known deficiency of factor VIII or IX : Recombinent DNA derived factor VIII and IX concetrate
  • 20. F. Disorders due to problems other than hemostatic proteins : - Rule out the underlying possibilities - eg. Infection, Liver rupture, catheter, NEC. G. T/t of specific disorders : 1.DIC : - Treat the underlying cause i.e. sepsis, NEC - Make sure that Vit K1 has been given.
  • 21. - Platelets/ FFP to keep platelet counts > 50,000/ml and to stop bleeding. - If bleeding persists, i. Exchange transfusion with fresh whole blood /Packed RBC/Platelets/FFP ii. Continuous transfusion with platelets, packed RBCs or FFP as needed. iii. For hypofibrinogenemia : Cryoprecipitate (10ml/kg)