An experimental screening program aimed at identifying an estimated
one-in-14,000 newborns suffering from the potentially deadly gastroenterological
condition called biliary atresia has been launched at Shaare Zedek Medical
Center (SZMC) in Jerusalem.
The condition involves an ongoing blockage in
the bile ducts that begins around the time of birth. Bile ducts carry bile from
the liver to the small intestine, removing wastes from the liver and carrying
salts that help the small intestine digest fat.
screening is being headed by pediatric gastroenterologist Dr. Oren Ledder, who
came on aliya from Australia and joined SZMC a year ago. He works under Dr. Dan
Turner, chief of pediatric gastroenterology.
Shaare Zedek delivers more
babies than any other hospital in the country – 16,000 a year – and with its
recent takeover of the capital’s Bikur Cholim Hospital this will rise to about
21,000. Thus, the disease could be detected in one or two babies annually just
with the screening.
“Pediatricians know about biliary atresia and can
diagnose the disease, but since the symptoms are very subtle and often
nonspecific, it might not be for some time,” Ledder told The Jerusalem Post
“Whether a virus or genetics or the immune system is responsible
is still not clear.”
The Health Ministry has not initiated national
screening of infants for biliary atresia. As some 160,000 babies are born in the
country each year, a national program could save the lives of 10 infants, not to
mention the costs of treatment resulting from late diagnosis.
was received from the ministry spokeswoman.
The symptoms for biliary
atresia usually show up during the first two months of life, and if there is no
treatment irreversible cirrhosis of the liver could result. This can lead to the
infant’s death if a liver transplant is not performed, and such donor organs are
hard to obtain.
Newborns with biliary atresia may seem normal and be at
normal weight after delivery, but they might develop jaundice – a yellow tinge
on their skin and mucous membranes – lose weight and become irritable within a
few weeks. The condition can usually be detected from pale or clay-colored
stools (instead of the normal “mustard-colored” ones, especially when
breastfed), and possibly dark urine. Often, an astute pediatrician who
investigates ongoing jaundice detects the problem.
Ledder said that an
operation called the Kasai procedure, developed in Japan, can be performed to
connect the liver to the small intestine, bypassing abnormal bile ducts.
However, a liver transplant might still be needed.
He explained that the
procedure does not cure the condition.
Rather, it is palliative, meaning
it provides relief and can limit a worsening of the condition for a long time.
The earlier the condition is diagnosed and the procedure performed, the better
“During the operation, performed by specially trained
surgeons, the bile ducts are removed and the liver is attached directly to the
small intestine,” he said. “It’s a very intricate, complicated procedure – most
successful if performed before the baby is eight weeks old, but doing so by 45
days is preferable.”
As pale stools during the baby’s first weeks of life
are easiest for parents to notice, the SZMC screening program is based on the
distribution of a printed card in color given to every new mother, with
explanations from a senior hospital nurse.
“Parents may not be sure about
the actual color of the stools that signal biliary atresia,” Ledder said, “so we
have printed eight-by-18-centimeter cards in Hebrew and Arabic with explanations
of what is normal and abnormal. We hope to produce cards in other languages as
SZMC director-general Prof. Jonathan Halevy has approved
the program, which will mean minimal costs to the hospital for the parental
briefing and cards. Explanations about the program will be posted on the
Such cards have been issued in Taiwan, Germany and
elsewhere for the past few years.
The main issue the experimental SZMC
program still has to settle is whether after studying the card and observing
their child, parents will become overcautious and call the hospital, creating
false positives that lead to more tests than the medical system can
“If the screening does not overwhelm the health system, it should
be implemented, as catching problems early is preferable to more complicated
treatment and death,” Ledder added.
False positives have so far been
proven to be very low in the Far East.
Ledder added that if his program
is successful, Tipat Halav (well-baby center) nurses should be trained starting
in a year to discuss screening with parents.
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