Cholestasis: Unfamiliar, but don't take it lightly

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.