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Introduction
Blood volume and red cell mass at birth and in neonatal period depend on
Volume of placental transfusion and, Subsequent readjustments of blood volume
Placental Transfusion
Occurs within 3 min. of delivery Contributes 25% of total neonatal blood volume. This amount will be increased in:
Elevated maternal blood pressure Use of oxytocic drugs Late clamping or milking of the cord Infant held in a low dependent position.
Placental Transfusion
Average blood volume 85-90ml/kg. Ranges between 75-100 ml/kg. Readjustment of blood volume
Occur the first 3-4 hours after birth with heamoconcentration to compensate for expansion of intravascular volume.
Neonatal Anaemia
Neonatal Anaemia
Defined as: Hb. Less than 13 g%
Haemorrhage (3)
Causes of haemorrhage
Haemorrhage before and during delivery
Placental praevia Cord rupture or torn vessels Fetal fetomaternal, twin twin transfusion
Neonatal haemorrhage
Bleeding into brain, lung or bowel Haemorrhagic disease of newborn
Management of Haemorrhage
Clinical assessment for shock and hypovolaemia. Investigations include: Haemoglobin and haematocrit Blood group Cross match blood Kleihauers test Coagulation studies Ultrasound cranium, abdomen, stools for blood.
History
Townsend in Boston (1864) described 50 cases of hemorrhagic disease of the newborn during first 2 weeks of life In 1929, Vitamin K isolated from alfalfa by Dam and Doisy (Nobel Prize, 1942), and conducted clinical trials showing Vitamin K protects against HDN 1961, Am Acad Pediatrics and Am College Obstetrics and Gynecology recommended routine prophylaxis with Vit K for all newborns Controversy in Britain in 1990s resolved to satisfaction of AAP, ACOG, Canada, Australia, New Zealand and others
Primary HDN
Often fatal condition Diffuse hemorrhage in otherwise healthy infant During the first week of life Particularly in low birth weight babies Results of low levels of prothrombin and other vitamin K dependent clotting factors, (Factors II, VII, IX and X) caused by vitamin K deficiency An exaggerated of physiologic deficiency of clotting factors normal in the first few days of life Incidence between 2.5 to 17.0 per thousand newborns not given vitamin K prophylactically
Late HDN
Between 2-12 weeks of life, Especially in breast-fed babies. Immaturity of liver affects production of clotting factors Late HDN primarily in breast fed infants without or inadequate vitamin K rates of 4.4-7.2/100,000 live births
Clinical features:
Spontaneous bleeding usually GIT. Umbilical bleeding or postcircumcision. Occurs late in first week of life esp to breastfed infant. DD from bleeding due to swallow blood.
Differentiate by APTs Test.
Investigations:
The diagnosis is confirmed by a prolonged prothrombin time (PT) but normal partial thromboplastin time (PTT). Apts test resistance of Fetal RBCs to denaturation by sodium hydroxide.
Treatment
Vitamin K1, 1 mg im or iv Blood transfusion if indicated.
Other Countries
Still not routine in Japan, Germany, UK Routine prophylactic Vitamin K for newborns adopted in
Canada Australia New Zealand Croatia, 1988
Summary
Deficiency of Vit K remains a significant worldwide cause of neonatal morbidity and mortality Routine prophylactic use of vitamin K should always be used to prevent HDN (good public health practice) Administration by intramuscular injection (0.5-1.0 mgm) within 6 hours of birth is preferable May be given orally as 3 doses spread over the first 4 weeks of life Vit K showing up in literature on osteoporosis A safe, inexpensive preventive procedure that should be mandatory component of newborn care.
Haemolysis (4)
(Hemolytic Disease of Newborn, HDN)
Non-Immune haemolysis
Congenital infection DIC G6PD def Pyruvate kinase Alpha Thal
What is HDN?
Destruction of the RBCs of the fetus and newborn by antibodies produced by the mother Only IgG antibodies are involved because it can cross the placenta (not IgA or IgM)
+
Mothers antibodies
Fetal RBC
destruction
Pathophysiology
Although transfer of maternal antibodies is good, transfer of antibodies involved in HDN are directed against antigens on fetal RBCs inherited by the father Most often involves antigens of the Rh and ABO blood group system, but can result from any blood group system Remember: The fetus is POSITIVE for an antigen and the mother is NEGATIVE for the same antigen
Pathophysiology
HDN develops in utero The mother is sensitized to the foreign antigen present on her childs RBCs usually through some seepage of fetal RBCs (fetomaternal hemorrhage) or a previous transfusion HDN occurs when these antibodies cross the placenta and react with the fetal RBCs
Rh HDN
Mother is D negative (d/d) and child is D positive (D/d) Most severe form of HDN 33% of HDN is caused by Rh incompatibility Sensitization usually occurs very late in pregnancy, so the first Rh-positive child is not affected
Bleeds most often occur at delivery Mother is sensitized Subsequent offspring that are D-positive will be affected
FetoMaternal Hemorrhage
Sensitization occurs as a result of seepage of fetal cells into maternal circulation as a result of a fetomaternal hemorrhage
Placental membrane rupture (7%) Trauma to abdomen Delivery (>50%) Amniocentesis Abortion
Pathogenesis
Maternal IgG attaches to antigens on fetal cells
Sensitized cells are removed by macrophages in spleen Destruction depends on antibody titer and number of antigen sites IgG has half-life of 25 days, so the condition can range from days to weeks
RBC destruction and anemia cause bone marrow to release erythroblasts, hence the name erythroblastosis fetalis)
Pathogenesis
When erythroblasts are used up in the bone marrow, erythropoiesis in the spleen and liver are increased
Hepatosplenomegaly (enlarged liver & spleen) Hypoproteinemia (from decreased liver function) leads to cardiac failure edema, etc called Hydrops fetalis
Bilirubin
Hemoglobin is metabolized to bilirubin
Before birth, indirect bilirubin is transported across placenta and conjugated in maternal liver (direct) where it is excreted After birth, the newborn liver is unable to conjugate the bilirubin
Unconjugated (indirect) bilirubin can reach toxic levels (18-20 mg/dL) This is called kernicterus and can lead to permanent brain damage
Dose
Each vial of RhIg contains enough anti-D to protect against a FMH of 30 mL
One vial contains 300 g of anti-D Given intramuscularly of intravenously Massive fetomaternal hemorrhage (>30 mL) requires more than one vial To assess a FMH, a maternal sample is screened within 1 hour of delivery (rosette test)
Liley graph
The OD is plotted on the Liley graph according to gestational age Three zones estimate the severity of HDN
Lower: mildly or unaffected fetus (Zone 1) Midzone: moderate HDN, repeat testing (Zone 2) Upper: severe HDN and fetal death (Zone 3)
Liley graph
Management of the rhesus immunized infant The infant should be assessed for maturity,pallor, jaundice,ascites Placenta send for pathology examination. Cord blood taken for HB.,DCT, Hb. And platelet and total Serum Bilirubin.
Cord haemoglobin < 8 g/dl. Hydrops fetalis Cord bilirubin > 85umol/L Rapidly rising bilirubin crossing the level for exchange transfusion. Strong positive Coombs test.
ABO HDN
ABO HDN
ABO incompatibilities are the most common cause of HDN but are less severe
About 1 in 5 pregnancies are ABO-incompatible 65% of HDN are due to ABO incompatibility
Usually, the mother is type O and the child has the A or B antigenWhy?
Group O individuals have a high titer of IgG antiA,B in addition to having IgM anti-A and anti-B
ABO Incompatibility
Mother
O A B
Infant
A or B B or AB A or AB
Frequency
Common Rare Rare
ABO HDN
ABO HDN can occur during the FIRST pregnancy b/c prior sensitization is not necessary ABO HDN is less severe than Rh HDN because there is less RBC destruction
Fetal RBCs are less developed at birth, so there is less destruction by maternal antibodies When delivered, infants may present with mild anemia or normal hemoglobin levels Most infants will have hyperbilirubinemia and jaundice within 12 to 48 hours after birth
Clinical features
Jaundice first 24 hours of life No hepatosplenomegaly Kernicterus unusual. Hydrops occasionally being reported. Late anemia is seldom a problem.
Investigations:
Suspected in mother who is blood group O and infant either group A or less commonly group B. DCT usually negative but indirect coombs test may be positive. Blood smear may show features of haemolysis. Immune anti-A or anti-B may be elicated from fetal RBCs or cord blood.
Phototherapy
Exchange transfusion
Hydrops Fetalis
This term is used to describe an infant who shows severe and generalized oedema, ascites and pleural effusions at birth.
Polycythaemia
Polycythaemia
Common and is defined as a venous haemotocrit of 65% or more (= Hb 22g/dl), during the first week. Not equal to hyperviscous. Blood viscosity depends largely on packed cell volume. Viscosity much more greater in small vessels than large vessels. Daignosed on free flowing venous specimen and not from heel prick sample.
Clinical features
Infants look plethoric. Neurological
Jitteriness,apnoea,convulsions.
Cardiovascular
Congestive heart failure,pulmonary hypertension with Right to left shunting and cyanosis.
Gastrointestinal - NEC Renal renal vein thrombosis Others: hypoglycaemia, thrombocytopenia, jaundice,hypocalcemia.
Management
Anticipation At risk infants haemotocrit should be measured. Infants without symptoms but haematocrit > 70% should have a dilutional exchange transfusion. Symptomatics infants with venous PCV of 65-70% may require dilutional exchange transfusion.
Clinical features
Bleeding from umbilical or venepuncture sites. Bruising, purpura or petechial haemorrhages.
Causes of thrombocytopenia
Infection
Bacterial infection TORCH infections
Isoimmune thrombocytopenia
Rare condition. Analogous to Rh isoimmunization. Transfusion of fetal A1 antigen-positive platelets into maternal circulation may produce maternal IgG antibodies if the mother is platelet A1 antigen negative. Mother has normal platelet count. Newborn may have severe thrombocytopenia but is transient. Platelets transfusion or exchange transfusion may be needed in severe cases.
Maternal ITP
Due to transplacental maternal antibodies. Mother may have thrombocytopenia and the lower mum platelets count the more severe affected the infant is. Steroids and IVIG had been advocated for treatment. Blood transfusion or platelet may be required if PC < 20,000. Intracerebral bleed may occur before onset of labour.
Causes of thrombocytopenia
Neonatal drug exposure
Thiazide diuretics Quinine Sulphonamides
DIC TAR syndrome Kasabach Merritt syndrome Fanconis anaemia Leukaemia Pancytopenias
Treatment
Very complex Treat underlying disease process Treatment of the haematological abnormality.