Revival of Landing's Concept of Infantile Obstructive Cholangiopathy – A Plea to Hand Over the 2 Week Old Jaundiced Full Term Neonate to the Surgeon!

Abstract

:The concept of infantile obstructive cholangiopathy started in 1974 by Landing and remained till early 1980s. However, the concept has not been used much since then as nothing new has developed over the last 40 years sufficient enough to change the course of the disease or identify its etiology. This review article is an attempt to consolidate evidence on review of literature pointing towards a common etiopathological process leading to the spectrum of anomalies constituting neonatal cholestasis. The importance of early referral of any case with neonatal jaundice in a full term neonate, preferably at 2 weeks age is highlighted. This will help to identify and treat more cases within the correctable range and prevent the disease from progressing to a life threatening situation especially in developing countries where the resources for liver transplantation are meagre. Introduction:Newborn babies are at increased propensity for developing hyperbilirubinemia due to increased bilirubin production or its decreased excretion. The increased production is attributed to a greater red cell mass per kg as compared to adults and a shorter life span of red blood cells. The decreased excretion is attributed to defective uptake of bilirubin due to hepatic immaturity and decreased ligandin, defective conjugation due to decreased uridine diphospho glucuronyl transferase (UDPG-T) activity, decreased hepatic excretion of bilirubin and increased enterohepatic circulation due to higher levels of glucuronidase Review Article REVIVAL OF LANDING'S CONCEPT OF INFANTILE Physiological Jaundice:The physiologic jaundice seen in newborn babies appears on the third day of life and usually lasts till the tenth day in full term babies. It appears slightly earlier in preterm babies and may last upto two weeks. However, the serum bilirubin levels do not exceed 12 mg % in full term babies and 15 mg % in preterm babies. The normal range of total and conjugated bilirubin are <1 mg/dl and <0.25 mg/dl respectively, although jaundice can only be appreciated in newborn babies only when the total bilirubin is greater than 5 mg/dL Physiological jaundice may be accentuated by immaturity, birth asphyxia, acidosis, hypothermia, hypoglycemia, septicemia, blood group incompatibilities, drugs like salicylates, gentamycin, sulpha drugs, cephalhematoma, polycythemia, hypothyroidism intrauterine infections and breast milk jaundice. At high levels, unconjugated bilirubin can cross the blood-brain barrier and cause brain injury (kernicterus). The usual therapy for unconjugated jaundice is phototherapy and occasionally exchange transfusion. Neonatal Cholestasis :Infants with the persistence of jaundice beyond the physiologic limits, in the absence of an apparent medical cause, need to be investigated without much delay. The causes for Conjugated Hyperbilirubinemia (neonatal cholestasis) include obstructive and nonobstructive disorders. These are broadly called extrahepatic biliary obstruction and neonatal hepatitis. Cholestasis thus results from either to s...