NONIMMUNE HYDROPSHEMORRHAGIC DISEASES OF THE NEWBORNWilliam 2001
Hyperbilirubinemia Nonimmune hydrops Cardiac arrhythmias Hemorrhagic disease of the
newborn Thrombocytopenia Polycythemia Necrotizing entrocolitis
HYPERBILIRUBINEMIA
Unconjugated bilirubin: Not excreted in bile and urine Pass the placenta to the mother
Conjugated bilirubin: Water soluble Excreted in bile and urine
Kernicterus: ↑unconjugated bilirubin
> 18 – 20 mg/dL - < 18 in preterm
Clinical picture: Spasticity MR Muscle incoordination
Causes of kernicterus: Hypoxia Hypoglycemia hypothermia Acidosis sepsis
Drugs: Furosemide Gentamicin
Salicylates Sulfonamides
Diazepam Na benzoate
↑vitamin K1
Brest milk jaundice: -Due to excretion of :
pregnane – 3 α, 20 β–diol in the milk inhibit conjugation of bilirubin by
inhibiting glucuronyl transferase activity
-Jaundice starts 4th to 15th day -No encephalopathy
Physiological jaundice:Starts 3rd to 4th dayBilirubin level < 10 mg/dLPhototherapy:For treatment of hyperbilirubinemiaMechanism:
Ligh oxidation of bilirubin ↓ ↑ peripheral blood flow
↑ photooxidation
Method: Eyes covered Skin exposed Appropriate fluorescent wavelength Baby turned /2 hours Bilirubin measured after 24 hours Monitor temperature to prevent
dehydration
NONIMMUNE HYDROPS FETALIS
Definition:Abnormal fluid accumulation in ≥ sitesIncidence:
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
Intrinsic causes: 41% cystic hygroma
27% cardiac anomalies 21% multiple malformations
11% othersCauses of nonimmune hydrops:
1 – Cardiac: = 20 – 45%
½structural anomalies ½cardiac arrhythmia
2 – Chromosomal anomalies: = 35% - earlier - extensive
space suite hydrops 87% with anencephaly
3 – Severe anemia: Parvovirus
Acute fetal - maternal Hg α - thalassemia
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
6 – Lymph system anomalies: - Chylothorax
- Chylous ascitesPrognosis:
< 24 weeks 95% mortality ≥24 weeks 80% mortality
Diagnosis:Maternal tests – cordocentesis - US
Maternal tests: Hb electrophoresis Indirect Coombs test Kleihauer – Batke test Serological tests for:
Rubella Toxoplasmosis Syphilis Cytomegalovirus
Parvovirus B - 19
Cordocentesis: karyotyping Hb% Hb electrophoresis Direct Coombs test Liver transaminases Serological test for Ig M
specific Abs
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
Deliver if near termExpectant treatment if very pretermMaternal complications:
Mirror syndrome: Edema and preeclampsia due to
vascular changes in the fetus Others:
Overdistension PTL – PP Hg-- retained placenta
CARDIAC ARRHYTHMIAS
Usually transient and benignSome tacchycardia if sustained may hydrops, HF and fetal deathSustained bradicardia is caused by:
Congenital anomalies Myocarditis
And is less often associated with hydrops
TYPES OF ARRHYTHMIASIsolated extrasystoles :
Atrial extrasystoles Ventricular extrasystoles
Sustained arrhythmias: Supraventricular tacchycardia Ventricular tacchycardia Complete heart block 2 degree heart block Atrial flatter, fibrillation Sinus bradicardia
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:
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
-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
HEMORRHAGIC DISEASE OF THE NEONATE
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
Causes: ↓ vit k1 Hemophilia Sepsis Syphilis Thrombocytopenia Erythroblastosis ICH
Vit K1: ↓ during pregnancy # nonpregnant ↓ placental transmission ↓ in milkAnticonvulsive drugs prevent hepatic synthesis of factor VII, IX, X ↓ vit K1 A phenotype similar to Chondrodysplasiapunctata = Conradi – Hunermann syndrome =inherited disease characterized by bone dystrophy and facial anomalies
THROMBOCYTOPENIA
Types:1 – Immune thrombocytopenia: - Maternal antiplatelet Ig G fetal/
neonatal thrombocytopenia - Usually associated with maternal
autoimmune disease and maternal thrombocytopenia
- Corticosteroid therapy ↑ maternal platelet count but does not improve
fetal condition
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
-Significant fetal – maternal Hg 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
-IV injection of Ig in a large dose to the mother recurrent fetal
thrombocytopenia by cordocentesisRecurrence = 70 – 90%
More severe and earlier in subsequent pregnancies
POLYCYTHEMIA
Predisposing factors: Chronic hypoxia Placental transfusion
( maternal or twin)Clinical picture:
Plethora Cyanosis Neurological impairment
Laboratory: ↑ bilirubin ↓ platelet Hypoglycemia Fragmented RBCs
Treatment:plasma
NECROTIZING ENTEROCOLITISBowel disorder affects mainly prematureneonates due to intestinal immaturityClinical picture:
Distension Illus Bloody stools
X ray:Gas in intestine = pneumatosis intestinalisMay perforation
%5.7 of preterm infantsCauses:
Perinatal hypotension hypoxia Sepsis Umbilical catheters Exchange transfusion Hypertonic fluids
Cow milk Coronovirus infectionTreatment :
Ig administration orally