One in every 14,000 babies suffers from potentially deadly gastroenterological condition called biliary atresia.
By JUDY SIEGEL-ITZKOVICHOren Ledder 370(photo credit: Shaare Zedek)
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.The experimental 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 Monday.“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.No comment 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 the prognosis.“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 well.”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 hospital’s website.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 handle.“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.
