Dr.Abdulaziz Alsoumali Intern Alyamamh hospital Pediatric rotation Jaundice Dr.Abdulaziz Alsoumali Intern Alyamamh hospital Pediatric rotation

Content Definition of jaundice Background & Epidemiology Bilirubin metabolism Classifications of jaundice Causes of neonatal jaundice Diagnosis Treatment

Jaundice Definition:

Definition Yellow discoloration of : - The skin - The conjunctival membrane (sclera) Jaundice is not a disease Bilirubin Over the sclera But a sign of increases bilirubin level

Background & Epidemiology Over 50% of all newborn infants become visibly jaundiced. About 80% of pre-term newborn infants become jaundiced. The red cell half life span of newborn infants is (70 days) Hepatic bilirubin metabolism is less efficient in the first few days of life.

Bilirubin metabolism

Classification A) Unconjugated Hyperbilirubinemia Hemolysis & Recticuloycytosis No Hemolysis (+) Coombs test ABO & Rh incompatibility Autoimmune SLE Idiopathic acquired hemolytic anemia Coombs test RBC enzyme defect (G6PD) RBC membrane defect (spherocytosis) Gilbert syndrome Physiologic jaundice Breast milk jaundice Breast feeding Crigler-Najjar syndrome Hypothyrodisim Pyloric stenosis SLE: systemic lupus erthematous

Classification B) Conjugated Hyperbilirubinemia Obstructive Infectious Metabolic Biliary atresia Choledochal cyst Cholelithiasis Bile duct stenosis Tumor/neoplasia Spontanoeus bile duct perforation Bile-mucus plug Hepatitis Cytomegalovirus Herpes simplex 1,2,6 Epstein-Barr virus Measles Varicella Bacterial sepsis Cholecystitis Wilson disease Alpha-1 antitrypsin deficiency Galactosemia Cystic fibrosis Dubin-Johnson Rotor syndrome D.D. of jaundice in childhood !!

Classification B) Conjugated Hyperbilirubinemia Idiopathic Autoimmune e.g. Idiopathic neonatal hepatitis e.g. Autoimmune chronic hepatitis Sclerosing cholangitis

Causes of neonatal jaundice

Diagnosis (Clinical Assessment) Jaundice appears clinically --> the bilirubin level reaches about 80 μmol/L Kramer recognised the cephalocaudal progression of jaundice with increasing total serum bilirubin levels and divided the baby into 5 zones, with a total serum bilirubin level measurement associated with each zone. This is known as Kramer’s rule (see Figure 1) and has traditionally been used to visually assess the severity of jaundice Kramer’s rule is inaccurate on a baby who has already commenced phototherapy.1 Visual estimation of bilirubin levels can lead to errors,2,3 especially in darkly pigmented babies.

Complications Permanent damage --> Kernicterus - Athetoid cerebral palsy - developmental delay - hearing deficit - dental dysplasia - Permanent upward gaze (Parinaud’s sign) Acute bilirubin encephalopathy refers to the acute manifestations of bilirubin toxicity1 seen in the first few weeks after birth.2 Initial signs include1 : • lethargy • hypotonia and poor suck progressing to: o hypertonia (opisthotonos and retrocollis) o high pitched cry and eventually to: ƒ seizures and coma Kernicterus is the pathogenic diagnosis characterised by bilirubin staining of the brain stem nuclei and cerebellum, but has also come to refer to chronic bilirubin encephalopathy.1 Clinical findings include1 : • athetoid cerebral palsy with or without seizures • developmental delay • hearing deficit • oculomotor disturbances including paralysis of upward gaze (Parinaud’s sign) • dental dysplasia • intellectual impairment

Complications Initial signs include: - lethargy Reversible damage --> Acute bilirubin encephalopathy Initial signs include: - lethargy - hypotonia - poor suck, progressing to - hypertonia (opisthotonos&retrocollis) - High pitched cry Postmortem brainstem and cerebellum showing kernicterus with yellow bilirubin staining of There is no bilirubin level known to be safe or which will definitely cause kernicterus. In rhesus haemolytic disease, it was found that kernicterus could be prevented if the bilirubin was kept below 340 μmol/L (20 mg/dl brainstem nuclei (arrows). , kernicterus is the yellow staining of specific areas of brain tissue in the neonate secondary to accumulation of unconjugated bilirubin. Bilirubin staining is seen most commonly in the basal ganglia (particularly the globus pallidus), subthalamic nuclei, and hippocampus and multiple cranial nerve nuclei (facial, cochlear, oculomotor, and vestibular nuclei). http://www.mayoclinic.org/diseases-conditions/infant-jaundice/basics/complications/con-20019637

Treatment Phototherapy Exchange transfusion Complications: Loose stools Erythematous macular rash Overheating --> leading to dehydration Bronze baby syndrome Exchange transfusion - no response with phototherapy - reaches the threshold of the transfusion Light (wavelength 450 nm) from the blue–green band of the visible spectrum converts unconjugated bilirubin into a harmless water­soluble pigment excreted pre­ dominantly in the urine. (light (wavelength 450 nm) from the blue-green band)

NICE guidelines ....

Take home message

Literatures Up to date Tom Lissauer, Graham Clayden. Illustrated textbook of Pediatrics, 4th edition NELSON, Essentials of pediatrics Queensland Maternity and Neonatal Clinical guideline NICE guidelines for neonatal jaundice

Questions !!