Nonimmune hydrops hemorrhagic diseases of the newborn

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