Download Cardiac arrhythmias

NONIMMUNE HYDROPS
HEMORRHAGIC
DISEASES OF THE
NEWBORN
William 2001
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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
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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 day
Bilirubin level < 10 mg/dL
Phototherapy:
For treatment of hyperbilirubinemia
Mechanism:
Ligh  oxidation of bilirubin  ↓
 ↑ peripheral blood flow
 ↑ photooxidation
Method:
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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 ≥ 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
Intrinsic causes:
41% cystic hygroma
27% cardiac anomalies
21% multiple malformations
11% others
Causes of nonimmune hydrops:
1 – Cardiac:
= 20 – 45%
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
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 ascites
Prognosis:
< 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 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
CARDIAC ARRHYTHMIAS
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
TYPES OF ARRHYTHMIAS
Isolated 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
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- 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 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
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 cordocentesis
Recurrence = 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 ENTEROCOLITIS
Bowel disorder affects mainly premature
neonates due to intestinal immaturity
Clinical picture:
 Distension
 Illus
 Bloody stools
X ray:
Gas in intestine = pneumatosis intestinalis
May  perforation
% 5.7 of preterm infants
Causes:
 Perinatal hypotension
 hypoxia
 Sepsis
 Umbilical catheters
 Exchange transfusion
 Hypertonic fluids
Cow milk
 Coronovirus infection
Treatment:
Ig administration orally
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