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