Cholestasis: Unfamiliar, but don't take it lightly

Maria Endah Hulupi, The Jakarta Post, Jakarta

Few are familiar with cholestasis, but this fatal disorder can affect people from different age groups, even infants.

Symptoms of cholestasis include yellowish skin and the whites of eyes along with dark urine and pale stools.

"When parents notice yellow discoloration in their babies, don't take it lightly. See a doctor right away for proper diagnosis," said Jose L. Batubara, chairman of the Indonesian Society of Pediatricians during a recent seminar held by baby food manufacturer Nutricia.

So, what exactly is cholestasis? It's an organic disorder that leads to the failure to excrete toxic substances out of the body through the liver and the bile. When this happens, the toxic substances accumulate in the liver -- poisoning liver cells and eventually leading to chronic liver disease causing cirrhosis (a condition where the liver slowly shrinks, hardens and loses its ability to perform its vital functions.

In babies, cholestasis is usually marked by jaundiced skin, and is also known as infant jaundice.

The yellow discoloration occurs due to excessive bilirubin (or bile pigment) in the blood stream that dissolves in the subcutaneous fat. This causes a yellowish appearance of the skin and the whites of the eyes (excessive intake of beta carotene can also cause yellow discoloration but the whites of the eyes remain white).

Cholestasis itself is separated into intrahepatic cholestasis and extrahepatic cholestasis.

"Each disorder requires different treatment and failure to provide early treatment for the disorder can be fatal," said pediatrician Asril Aminullah, director of the Cipto Mangunkusumo General Hospital (RSCM)'s pediatric division.

Intrahepatic cholestasis can be triggered by viral and bacterial infections. All serious infections in which microorganisms have entered the bloodstream can cause cholestasis because they also enter the liver thus damaging the liver cells.

Intrahepatic cholestasis in newborn babies is mostly caused by viral infections, which can pass over time, while those caused by sepsis (a toxic condition caused by the infection) require proper antibiotic treatment.

Other noninfectious causes include irregularities in fat, protein or carbohydrate metabolism, congenital disorder, drug- induced liver damage, Down's Syndrome and hormonal disorders (like hypothyroid and panhypopituitarism).

Extrahepatic cholestasis can be caused by the blocking of the bile duct (also called biliary atresia) and cystic duct. Biliary atresia results in an ability to excrete toxic substances out of the body through fecal discharge, while the cystic duct variety can cause enlargement of the bile duct that can in turn lead to various complications, and even death.

Data from the University of Indonesia's School of Medicine and the RSCM shows that 60 percent of cholestasis in babies recorded by the hospital is intrahepatic with the infection taking place during the prenatal period. It also reveals that most of the extrahepatic cases are caused by biliary atresia.

Asril explained that both types of cholestasis are accompanied by jaundice. However, the color of the stool can help identify which type of extrahepatic cholestasis a baby has.

Babies with biliary atresia, for example, usually excrete pale- or whitish-colored stools. The weight of the baby is usually normal at birth, and normal development continues for about three weeks.

Treatment for babies with intrahepatic cholestasis is differentiated based on the causes. Viral infection can slowly lessen as time goes by, and those with metabolic disorders can also be treated based on the type of the disorder.

However, babies with extrahepatic cholestasis, both biliary atresia or cystic duct, have to be operated on. During the operation, the surgeon makes a new drainage system using a segment of intestine.

Jose said early surgery helped ensure satisfactory results. However, some patients with this progressive disease could experience a worsening condition at a slower pace.

"It is advisable that the jaundice be immediately checked by a doctor as it may indicate a serious disorder that requires immediate treatment, and even after the treatment a jaundiced baby should always be monitored periodically," he said.