Newborn Hemorrhagic Diseases

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Questions and Answers

Why are neonates, especially preterm infants, more susceptible to bleeding problems?

Because coagulation proteins are able to freely cross the placenta.
Because they have very high platelet counts compared to adults.
Because they often have inherited bleeding disorders since birth.
Because most bleeding problems in neonates are acquired secondary to perinatal complications. (correct)

What is the significance of vitamin-K dependent factors (FII, FVII, FIX, FX, FXI and FXII) in neonates?

They are crucial for platelet aggregation in response to thrombin.
They are primarily synthesized by the mother and transferred through breast milk.
They are low in neonates (less than 40% of adult values) during the first month of life, impacting neonatal hemostasis. (correct)
Their levels in neonates are higher than adult values due to placental transfer.

How reliable are bleeding times as screening tools for bleeding disorders in neonates?

Generally unhelpful and platelet function investigations are often deferred. (correct)
Especially reliable in preterm infants for detecting platelet aggregation issues.
The most useful screening tests due to their ability to detect platelet disorders in neonates.
Highly reliable due to the precise measurement of platelet function.

Why is it recommended to test both parents for coagulation abnormalities when a neonate presents with a suspected inherited bleeding disorder?

To identify potential carriers and understand the inheritance pattern of the disorder. (B) Signup and view all the answers

What distinguishes Type 2B von Willebrand disease (vWD) from Type 3 vWD in neonates?

Type 2B vWD presents with thrombocytopenia and bleeding, while Type 3 vWD has a clinical phenotype similar to haemophilia. (C) Signup and view all the answers

If a neonate is suspected of having Factor XIII deficiency, what diagnostic approach confirms this condition?

A specific FXIII assay following a clot solubility test in 5 mol/L urea solution is used. (C) Signup and view all the answers

What is the primary difference between afibrinogenemia and dysfibrinogenemia?

Afibrinogenemia is characterized by the plasma level of the protein being either not measurable or very low, whereas dysfibrinogenemia involves low clotable fibrinogen with normal or reduced fibrinogen antigen. (B) Signup and view all the answers

Why are neonates susceptible to Vitamin K deficiency despite the presence of vitamin-K dependent factors?

Due to limited placental transfer of vitamin K, low vitamin K stores at birth, low vitamin K in breastmilk, and the lack of bacterial vitamin K synthesis is the sterile neonatal gut. (C) Signup and view all the answers

What is the purpose of using assays of decarboxyprothrombin (PIVKA II) when diagnosing Vitamin K deficiency bleeding (VKDB)?

To confirm VKDB when doubt remains after initial tests, by identifying inactive forms of vitamin K-dependent clotting factors. (B) Signup and view all the answers

What are the main triggers of Disseminated Intravascular Coagulation (DIC) in neonates?

Severe hypoxia, acidosis, sepsis, perinatal Coxsackie virus infection, and placental abruption. (B) Signup and view all the answers

What is the primary pattern of coagulation abnormalities observed in neonatal Disseminated Intravascular Coagulation (DIC)?

Prolongation of PT, APTT, and TT along with low platelets and fibrinogen. (A) Signup and view all the answers

In the management of Disseminated Intravascular Coagulation (DIC) in neonates, what hematological parameters are typically targeted for maintenance with blood product replacement?

Maintain platelet count above 30 x $10^9$/L and fibrinogen greater than 1 g/L. (B) Signup and view all the answers

What are the most frequent causes of early onset thrombocytopenia in preterm infants?

Intrauterine growth restriction and maternal hypertension or diabetes. (B) Signup and view all the answers

What maternal condition leads to neonatal autoimmune thrombocytopenia?

Maternal ITP and Systemic Lupus Erythematosus (SLE). (A) Signup and view all the answers

What is the established approach for the treatment of severe thrombocytopenia in neonates?

Intravenous IgG (400mg/kg/day) given over 2-4 hours for 5 days or 1 g/kg/day for 2 days, total dose 2 g/kg. (B) Signup and view all the answers

Under what circumstances is platelet transfusion not indicated in a term neonate?

If the neonate has platelet counts >30 × $10^9$/L and no evidence of bleeding. (D) Signup and view all the answers

Early VKDB typically presents with which symptom?

Severe gastrointestinal and intracranial haemorrhage. (B) Signup and view all the answers

Early VKDB is often associated with which risk factor?

Maternal medication that interferes with vitamin K. (D) Signup and view all the answers

What are the clinical signs that are included for inherited FVIII deficiency?

intracranial haemorrhage, cephalohaematomas and bleeding after circumcision or from venous or arterial puncture sites (C) Signup and view all the answers

What are the diagnostic results in adults, that diagnose FVIII deficiency?

Prolonged APTT and reduced FVIII clotting activity by factor assay test (A) Signup and view all the answers

How is bleeding in neonates treated with recombinant FIX concentrate monitored?

To achieve a FIX level of 1.0 IU/mL. (D) Signup and view all the answers

What is the diagnostic method for von Willebrand disease?

Measuring VWF, FVIII and pattern of VWF multimers (A) Signup and view all the answers

What is the screening test used to diagnose Factor XIII deficiency?

The diagnosis is made by measuring clot solubility in 5 mol/L urea solution. (A) Signup and view all the answers

How is bleeding treated with FXIII deficiency?

FXIII concentrate. (B) Signup and view all the answers

What occurs if there is dysfibrinogenemia?

Low clotabl fibrinogen with normal or moderately reduced fibrinogen antigen. (A) Signup and view all the answers

What is the conventional treatment plan for fibrinogen deficiency?

Effective long-term secondary prophylaxis with administration of fibrinogen every 7-14 days. (D) Signup and view all the answers

What causes Acquired disorders of coagulation?

Vitamin K deficiency. (D) Signup and view all the answers

What are the levels like in vitamin-K-dependent procoagulant factors?

They are low at birth because of poor placental transfer of vitamin K. (C) Signup and view all the answers

Classical VKDB presents at how many days?

2-7 days (C) Signup and view all the answers

When does late VKDB occur?

Between 2 and 8 weeks after birth. (B) Signup and view all the answers

What is used to treat VKDB?

Vitamin K 1 mg intravenously (D) Signup and view all the answers

What are the main triggers of DIC in neonates?

Severe hypoxia (B) Signup and view all the answers

What is a common cause of DIC in neonates?

Acute perinatal hypoxia. (A) Signup and view all the answers

What are the signs that DIC is present in newborns?

Generalized bleeding, pulmonary haemorrhage and oozing from venepuncture sites (D) Signup and view all the answers

What is the usual pattern of coagulation abnormalities in neonatal?

Prolongation of the PT, APTT and TT. (D) Signup and view all the answers

What are frequent causes of early onset thrombocytopenia?

Intrauterine growth restriction (B) Signup and view all the answers

What is a frequent cause of late onset thrombocytopenia?

Neonatal alloimmune thrombocytopenia (NAITP) (A) Signup and view all the answers

When does platelet count usually fall for all thrombocytopenic neonates with NAITP?

The first 4-7 days of life (B) Signup and view all the answers

What does platelet transfusion cause in term neonates with platelet counts less than >30 × $10^9$/L?

Platelet transfusion is not indicated. (D) Signup and view all the answers

Flashcards

Neonatal Bleeding

Bleeding problems are relatively common, especially in sick or preterm neonates.

Causes of Neonatal Bleeding

Most neonatal bleeding problems are acquired and secondary to perinatal complications like asphyxia or infection.