Persistent Neonatal Jaundice on Neonates and Childern

(persistent ) Prolonged neonatal jaundice (PNNJ)is defined as a serum bilirubin level higher than 85 μ mol/L (5 mg/dl) with yellowish discoloration of the skin ,sclera and conjunctiva , which persists at postnatal 14 days in full term infants and 21 days following the birth in preterm infants .

Etiologically it is important to distinguish jaundice type , is it unconjugated (indirect) or conjugated (direct)hyperbilirubinemia. A prolonged unconjugated hyperbilirubinemia is mostly caused by breastfeeding(which is the most common identified cause for prolonged unconjugated hyperbilirubinemia , It is known that breastmilk jaundice is seen at a rate of 1.3% in newborn infants and 2.4-25% in infants fed with breastmilk ) or to some pathological conditions such as hemolytic diseases (due to hereditary spherocytosis ,elliptocytosis or G6PD deficiency), congenital hypothyroidism, urinary infection, Crigler-Najjar or Gilbert syndromes ,pyloric stenosis ,sepsis.

Conjugated hyperbilirubinemia (Cholestatic jaundice ) is never physiologic. It affects 1/2,500 live births and it should be suspected in all jaundiced infants with light stools and dark urine , The differential diagnosis of cholestasis is extensive and a step-wise approach based on the initial history and physical examination is useful to rapidly identify the underlying etiology, Early recognition of neonatal cholestasis is essential to ensure timely treatment and optimal prognosis. (causes Include infections, anatomic abnormalities of the biliary system, endocrinopathies, genetic disorders ,cystic fibrosis, metabolic abnormalities, toxin and drug exposures, vascular abnormalities, neoplastic processes, and other miscellaneous causes , the most commonly identifiable causes are biliary atresia (BA) (25%-35%), genetic disorders (25%), metabolic diseases (20%), and a1-antitrypsin (A1AT) deficiency (10%) ; other pathological causes of prolonged conjugated hyperbilirubinemia are TORCH-S infections, Neonatal hepatitis syndrome, Choledochal cyst , Inspissated bile syndrome, Galactosemia ,Alagille syndrome \& Hereditary bile acid synthesis disorders.

Diagnosis : is made according to the physical examination(skin ,stool \& urine color, organomegaly) , clinical presentation , investigations \&imaging findings.

The clinical presentation of(PNNJ) include yellowish discoloration of skin ,sclera \&conjunctiva with change in color of stool \&urine with or without organomegally persistent for more than 14 days in full term infants \&21 days in preterm infants.

investigations: include CBC, serum total \&direct Bilirubin , urine analysis \&culture , Coombs' test, thyroid function tests, G6PD level, LFT, blood film, GAL1PUT, alpha - 1 antitrypsin test , screening for TORCH ,Ultrasonography of the abdomen.

Investigations for cholestatic jaundice :fine needle or true cut needle liver biopsy , magnetic resonance cholangiopacreatography (MRCP),endoscopic retrograde cholangiopancreatography (ERCP) and hepatobiliary scintigraphy.

Mortality/morbidity:

• Kernicterus or bilirubin encephalopathy, typically in infants It occurs when the unconjugated bilirubin levels cross 25 mg/dL in the blood it gets deposited in the brain tissue. The neurotoxicity leads to various neurologic sequelae as poor feeding ,irritability, a high-pitched cry ,no startle reflex ,lethargy , apnea , seizures ,cerebral palsy ,hearing loss ,learning disabilities.
• Liver cell failure \&cirrhosis with portal hypertension are complications of neonatal cholestasis.

Treatment Phototherapy, intravenous immune globulin (IVIG), and exchange transfusion are the most widely used therapeutic modalities in certain instance as Gilbert syndrome medications as Phenobarbital an inducer of hepatic bilirubin metabolism a new therapy currently under development consist of inhibition of bilirubin production through blockage of heme oxygenase by using mesoporphyrins \& protoporphyins .

Treatment of Cholestatic jaundice depend mainly on cause of cholestasis ,all neonates \& children should receive fat soluble vitamins (A,K,E,D),surgical intervention (kasai procedure)or even liver transplantation may be done.