Nonimmune hydrops hemorrhagic diseases of the newborn

2. William 2001 Nonimmunehydropshemorrhagic diseases of the newborn

3. Hyperbilirubinemia • Nonimmunehydrops • Cardiac arrhythmias • Hemorrhagic disease of the newborn • Thrombocytopenia • Polycythemia • Necrotizing entrocolitis

4. Unconjugatedbilirubin: • Not excreted in bile and urine • Pass the placenta to the mother Conjugated bilirubin: • Water soluble • Excreted in bile and urine Kernicterus: ↑ unconjugatedbilirubin > 18 – 20 mg/dL - < 18 in preterm hyperbilirubinemia

5. Clinical picture: • Spasticity • MR • Muscle incoordination Causes of kernicterus: • Hypoxia • Hypoglycemia • hypothermia • Acidosis • sepsis

6. Drugs: Furosemide Gentamicin Salicylates Sulfonamides Diazepam Na benzoate ↑ vitamin K1

7. Brest milk jaundice: - Due to excretion of: pregnane – 3 α, 20 β–diol in the milk  inhibit conjugation of bilirubin by inhibiting glucuronyltransferase activity - Jaundice starts 4th to 15th day - No encephalopathy

8. Physiological jaundice: Starts 3rd to 4th day Bilirubin level < 10 mg/dL Phototherapy: For treatment of hyperbilirubinemia Mechanism: Ligh oxidation of bilirubin  ↓  ↑ peripheral blood flow  ↑ photooxidation

9. Method: • Eyes covered • Skin exposed • Appropriate fluorescent wavelength • Baby turned /2 hours • Bilirubin measured after 24 hours • Monitor temperature to prevent dehydration

10. Definition: Abnormal fluid accumulation in ≥ sites Incidence: 0.6 %  77% of them are known 1.7 %  95% of them are known Incidence of hydrops: 13% immune 1.3% extrinsic 21% idiopathic 64% intrinsic Nonimmunehydropsfetalis

11. Intrinsic causes: 41% cystic hygroma 27% cardiac anomalies 21% multiple malformations 11% others Causes of nonimmunehydrops: 1 – Cardiac: = 20 – 45%

12. ½ structural anomalies ½ cardiac arrhythmia 2 – Chromosomal anomalies: = 35% - earlier - extensive  space suite hydrops 87% with anencephaly 3 – Severe anemia: Parvovirus Acute fetal - maternal Hg α - thalassemia

13. 4 – Twin-to-twin transfusion: Recipient  HF Donor  hydrops after the death of the recipient 5 - Inborn errors of metabolism: - Gaucher disease - GM 1 gangliosidosis - Sialidosis All  recurrent hydrops

14. 6 – Lymph system anomalies: - Chylothorax - Chylousascites Prognosis: < 24 weeks  95% mortality ≥ 24 weeks  80% mortality Diagnosis: Maternal tests – cordocentesis - US

15. Maternal tests: • Hb electrophoresis • Indirect Coombs test • Kleihauer – Batke test • Serological tests for: Rubella Toxoplasmosis Syphilis Cytomegalovirus Parvovirus B - 19

16. Cordocentesis: • karyotyping • Hb% • Hb electrophoresis • Direct Coombs test • Liver transaminases • Serological test for Ig M specific Abs

17. Most important predictor tests for prognosis: • Karyotyping • Fetal ECG Management: - Blood transfusion for anemia - Amniocentesis for twin-to-twin transfusion may  spontaneous cure If persistent  exclude cardiac anomalies and anencephaly

18. Deliver if near term Expectant treatment if very preterm Maternal complications: • Mirror syndrome: Edema and preeclampsia due to vascular changes in the fetus • Others: Overdistension  PTL – PP Hg -- retained placenta

19. Usually transient and benign Some tacchycardia if sustained may  hydrops, HF and fetal death Sustained bradicardia is caused by: • Congenital anomalies • Myocarditis And is less often associated with hydrops Cardiac arrhythmias

20. Isolated extrasystoles: • Atrialextrasystoles • Ventricular extrasystoles Sustained arrhythmias: • Supraventriculartacchycardia • Ventricular tacchycardia • Complete heart block • 2 degree heart block • Atrial flatter, fibrillation • Sinus bradicardia Types of arrhythmias

21. Premature atrial contractions: = 64% of cardiac arrhythmia Usually benign and transient Rarely  supraventricular tacchycardia and if > 200 b/m may  HF Bradicardia:  Poor prognosis Caused by:

22. Structure anomalies as A-V canal • Heart block Congenital heart block: - Caused by Abs against fetal myometrium in 50% of the cases - Most common Abs: Anti-SS-A (Anti Ro) Abs -  Inflammation and permanent damage to the myocardial tissue

23. - Neonate may require pacemaker - Only 1 : 20 of the cases are affected - Mothers usually have: SLE or other CT disease or subsequently develop it Fetotherapy : By corticosteroids to the mother

24. Characterized by: • Hypoprothrombinemia • ↓ factor V, VII, IX, X • ↑ prothrombin time • ↑ PTT  Spontaneous internal or ext Hgs May occur at any time Usually delayed 1 – 2 days Hemorrhagic disease of the neonate

25. Causes: • ↓ vit k1 • Hemophilia • Sepsis • Syphilis • Thrombocytopenia • Erythroblastosis • ICH

26. Vit K1: • ↓ during pregnancy # nonpregnant • ↓ placental transmission • ↓ in milk Anticonvulsive drugs prevent hepatic synthesis of factor VII, IX, X  ↓ vit K1  A phenotype similar to Chondrodysplasia punctata = Conradi – Hunermann syndrome =inherited disease characterized by bone dystrophy and facial anomalies

27. Types: 1 – Immune thrombocytopenia: - Maternal antiplateletIg G  fetal/ neonatal thrombocytopenia - Usually associated with maternal autoimmune disease and maternal thrombocytopenia - Corticosteroid therapy  ↑ maternal platelet count but does not improve fetal condition thrombocytopenia

28. 2 -- Alloimmune thrombocytopenia (ATP): - Fetal platelet Ag  pass the placenta to the mother  isoimmunization - Usually discovered after the delivery of an affected child - May  IC Hg - 98% of the population are HPA 1a +ve 2 % of the population are HPA 1a –ve - % = 1 : 5000 – 10000 live birth - 1 : 50 of pregnancies are at risk

29. - Significant fetal – maternalHg must occur provoke immune respond - Affect offspring of women with HLA type DR - 3 or B - 8 Diagnosis: - Maternal platelet count normal +no autoimmune disease - Fetal platelets count ↓ + no other autoimmune D

30. - IV injection of Ig in a large dose to the mother  recurrent fetal thrombocytopenia by cordocentesis Recurrence = 70 – 90%  More severe and earlier in subsequent pregnancies

31. Predisposing factors: • Chronic hypoxia • Placentaltransfusion ( maternal or twin) Clinical picture: • Plethora • Cyanosis • Neurological impairment polycythemia

32. Laboratory: • ↑ bilirubin • ↓ platelet • Hypoglycemia • Fragmented RBCs Treatment: plasma

33. Bowel disorder affects mainly premature neonates due to intestinal immaturity Clinical picture: • Distension • Illus • Bloody stools X ray: Gas in intestine = pneumatosisintestinalis May  perforation Necrotizing enterocolitis

34. % 5.7of preterm infants Causes: • Perinatal hypotension • hypoxia • Sepsis • Umbilical catheters • Exchange transfusion • Hypertonic fluids

35. Cow milk • Coronovirusinfection Treatment: Ig administration orally