Neonatal Cholestasis: A Pandora's Box

Abstract

Neonatal cholestasis (NC) is a diagnostic dilemma frequently countered in a neonatal care unit. Early diagnosis is vital for achieving an optimal patient outcome as many causes of cholestasis such as biliary atresia are time-sensitive and amenable to treatment if analyzed and treated early. Nonetheless, it is not generally simple to analyze these cases right on time as some of them are regularly missed due to the presence of pigmented stools, lack of newborn metabolic screening, and named as instances of prolonged jaundice. In this manner, we prescribe to explore all reasons for prolonged jaundice stretching out past 14 days in neonates. Besides, we suggest that stool card ought to be a piece of release rundown for all newborn children being released from the nursery. This is of most extreme significance in the nation like India where guaranteeing customary follow-up is as yet a tough assignment. These stool cards will help in the early determination of patients with NC particularly biliary atresia and guarantee their auspicious cure. Another reason which needs exceptional say is parenteral nutrition-associated liver illness, as the proportion of preterm babies is getting greater and greater with better neonatal care. These extreme preterm infants are in the requirement for prolonged (>14 days) total parenteral nourishment because of which they are at high hazard for NC contrasted with their more developed peers. In this survey, we will give an understanding of clinical approach, differential diagnosis, and clinical review of NC.

Keywords: biliary atresia; hepatoportoenterostomy; liver biopsy; neonatal cholestasis; neonatal jaundice; radionuclide scan.

Figure 1.

Acholic stools due to inspissated bile syndrome in a 2-month-old neonate with Rh hemolytic disease.

Figure 2.

A schematic diagram to work up a patient with neonatal cholestasis syndrome. *Workup includes sweat chloride, Pi typing, serum cortisol, urine GCMS, TMS, acyl carnitine profile, ANA, anti-Ro/SSA, anti-La/SSB, anti-U1RNP antibodies, urinary bile acid analysis, genetic analysis for PFIC (↑ liver enzymes and ↓ GGT) and bone marrow for hemophagocytic lymphohistiocytosis (HLH). Diagnostic criteria for HLH includes fever (>7 days), hepatosplenomegaly with liver dysfunction, pancytopenia, sCD25 (>2400 μg/mL), ferritin (>500 μg/L), triglycerides (>3 mmol/L), hypofibrinogenemia (<150 mg/dL), and serum cytokine levels of both IFNγ (>75 pg/mL) and IL-10 (>60 pg/mL) ↑.^,,,