Selected Podcast
Race Against Time: Diagnosing Biliary Atresia
Dr. Leina Alrabadi discusses Biliary Atresia.
Featuring:
Leina Alrabadi, MD
Leina Alrabadi, MD is a pediatric transplant hepatologist at Lucile Packard Stanford Children’s hospital. She works within a multidisciplinary team and cares for patients who have complex liver disease and for children who require or have undergone liver transplantation. She is a clinical researcher, with her focus on finding improved therapies for autoimmune and cholestatic liver diseases since an ideal therapy currently does not exist with the aim of giving children a better future. Transcription:
Scott Webb: Today, we are discussing biliary atresia. Most people never heard of biliary atresia. And although it is a rare condition in children, it is the number one indication of pediatric liver transplants worldwide. And joining me today to educate all of us about this condition, including red flags for parents like jaundice after two weeks is Dr. Leina Alrabadi. She's a pediatric transplant hepatologist with Stanford Children's Health.
This is Health Talks from Stanford Children's Health. I'm Scott Webb. And doctor, thanks so much for your time and your expertise today. As we get rolling here, what is biliary atresia?
Dr. Leina Alrabadi: So biliary atresia is a pediatric liver condition that is what we call progressive obliterative disease of the biliary tree. So that means that there's obliteration or destruction of the bile ducts, which are the channels that carry bilirubin in and out of the liver. And then that destruction of the bile ducts is what causes bilirubin to build up and become toxic in the liver.
Scott Webb: And what causes biliary atresia to occur and who gets it?
Dr. Leina Alrabadi: Unfortunately, the cause of the disease is unknown. But there's a lot of research worldwide to try to understand this disease process. But what we do know is it's not a hereditary disease, so it's not a disease that's passed on from a parent to a child, nor is it a preventable disease. So it's not something that expectant mothers could do anything to avoid, but the cause is what a lot of researchers are trying to understand currently.
Scott Webb: Yeah, and doing a little bit of research myself just to kind of prep myself for this. You know, I watched a couple of videos and did some reading and it does seem like understanding the cause is something that researchers are still working on. But at least we know how it manifests and how to treat it. And specifically, what are the symptoms to suspect biliary atresia in a child?
Dr. Leina Alrabadi: The clinical symptoms that you would suspect occur around two to six weeks of life in a child. I would say that most of these children are born full term, go home a few days after discharge from the hospital from birth, and do well. But then at two weeks, what we say is jaundice persists, so the yellowing of the skin. Again, as we know, yellowing of the skin can be very common in newborn children. But really, past two weeks of life warrants a workup with biliary atresia being on the differential for why that jaundice is still there.
So jaundice is one of the concerns, but the other one would be what we call acholic stools, which means that the stools, the poops for the baby, they don't have that yellow seedy, green or brown color that newborn stools should look like. These stools are gray, white, very light yellow, pale in color and that's what acholic means. And most families don't know that this is abnormal or know to look out for this. So this is a lot of education towards families and providers give families to look out at the color of the stool.
So I would say that acholic stools and jaundice are the big red flags for thinking of biliary atresia. But then other things are just poor weight gain, not growing as appropriately as they should are other things that the pediatrician looks for.
Scott Webb: Yeah, and it's so good as I have two kids myself, it's so good to know what the warning signs are because, you know, those first few weeks are magical and wonderful and worrisome. And so being on the lookout for jaundice after two weeks, looking out for abnormal stools, and I'm sure this all kind of feeds into exactly how do you diagnose biliary atresia?
Dr. Leina Alrabadi: Recognition by the family and what is discussed by the pediatrician also during well-child checks usually prompts the evaluation and then that workup usually will involve, initially it will be blood work. So you're looking at specific bilirubin numbers and and something we call the GGT number and those will be abnormally elevated in a patient with biliary atresia. And that workup will continue on to ultrasound imaging and then, in most scenarios, a liver biopsy.
And then liver biopsy does not diagnose biliary atresia, but it furthers the suspicion for biliary atresia if that liver biopsy shows what we call duct obstruction. So those bile ducts that we talked about earlier are obstructed, and you're kind of seeing that in the tissue of the liver. And then that usually will prompt clinical context, the lab workup to be abnormal, the liver biopsy to be abnormal for a patient to make the gold standard diagnosis of biliary atresia with what we call an intraop cholangiogram.
Scott Webb: Yeah. And I know Stanford Children's Health, we're always looking for the gold standard, which, you know, all parents want for their children. And when we talk about management and treatment, what are the options when it comes to biliary atresia?
Dr. Leina Alrabadi: So we mentioned the intraop cholangiogram, so what that is is that means a child is taken into surgery and a surgeon is able to visualize those bile ducts. And then insert a dye and then take pictures, and then we're able to see where does that dye go? Does that dye just kind of sit there because those ducts are not formed? Or does that dye appropriately go into the intestine? And that was said to be unlikely biliary atresia in that scenario.
But let's say that the patient goes into an intraop cholangiogram and there's no ducts, the ducts are all atretic, that's the diagnosis of biliary atresia. And that prompts what comes next for a patient, which is a Kasai procedure.
The Kasai procedure, it's known as the hepatoportoenterostomy. And the surgery was named after Dr. Morio Kasai. He's a Japanese surgeon who invented it. And what happens during a Kasai is that the surgeon takes part of the baby's intestines and brings it up to the dome of the liver. And this allows for a free-flowing channel. So they're creating a channel that allows the bile that was building up and becoming toxic in the liver to escape through this new duct out of the liver. So creating this channel in place of the atretic bile ducts that were previously unable to kind of get that bile out of the liver.
Scott Webb: It sounds really fascinating. You know, a little bit like science fiction when you're dealing with tiny little babies. It's really amazing. Why is the timing so essential for biliary atresia?
Dr. Leina Alrabadi: The timing is so essential because we talk about the Kasai procedure, but unfortunately with biliary atresia, there's no cure. The treatment that we do with the Kasai is helping the flow of the bilirubin come out of the liver. And the timing is so essential because better outcomes occur when bile flow is restored earlier through the Kasai procedure.
And what that means is it prevents accumulation of that toxic bile in the liver, which is only more damaging when it builds up and causes stiffness, liver scarring, and then what we would say, fibrosis, cirrhosis in a child. And really saying early is really not enough. It really comes down to a matter of days.
Scott Webb: Yeah. And doctor, you mentioned outcomes, and I'm sure for parents who would unfortunately have a child diagnosed with biliary atresia, they're going to want to know. As you say, it's really not so much treatment or cure rather, it's really more management of this. So what are the outcomes?
Dr. Leina Alrabadi: Yeah. So outcomes all relate to the timing of the Kasai. So that's why we say an early Kasai is really important and it really comes down to a matter of days. Always, you want to get a Kasai in before 90 days. But really, the push is now to get it below 60 days, even below 45 days and if possibly by 30 days because really those patients have the most success.
And then again, the goal for the Kasai is to continue to function and allow for appropriate bile flow so that the patient can keep what we say their native liver for as long as possible without needing a liver transplant.
And in terms of outcomes, what we look for is at three months post the Kasai, so after patient has had a Kasai, there's a clear difference in the two years native liver survival of a patient with what their total bilirubuin is at three months. And it's been shown that those who have a total bilirubin less than 2 compared to those with greater than 6, really have much better outcomes. And if the jaundice clears by three months after the Kasai, the 10-year transplant free survival is between 75% to 95%.
And if the jaundice persists after the Kasai, the three-year transplant-free survival is only 20%. So that's why it all comes down to the timing.
Scott Webb: Yeah, as you say, timing's not only important, I mean, it is absolutely critical and it can be a matter of days in terms of the outcomes for these children, these patients. Doctor, as we get close to wrapping up here, and this has been a really interesting conversation, really educational, we've been over this, but let's go back through it again. Why is biliary atresia so difficult to recognize and, ultimately, why is awareness so important?
Dr. Leina Alrabadi: Biliary atresia is so hard to recognize because newborn jaundice is so common. However, as we mentioned, jaundice that occurs after two weeks of life should always be investigated carefully. And again, biliary atresia is rare. It occurs between one and 8,000 to one in 18,000 cases per year according to the American Liver Foundation. But it's important to get this message out there and increase awareness as it is currently the leading diagnosis for liver transplant in children, and definitely something that affects children and families worldwide.
And the last point I would mention is getting a patient to a Kasai is extremely important. Doing that timely is critical, but what the procedure fails to do is prevent some of the biliary cirrhosis that will progress on its own. So really getting that Kasai in timely will prolong the patient with their native liver and decrease their need for liver transplant early in life.
Scott Webb: Yeah. So doctor, obviously the goal is to, you know, have patients have their native livers for as long as possible. But I guess the question that pops into my head is ultimately are all patients who are diagnosed with biliary atresia as children, are they going to need a transplant at some point?
Dr. Leina Alrabadi: Really the answer is yes. And it's very unlikely to make it into their 20s or 30s without needing a liver transplant. But the message is so important because there are patients who what we call have a missed biliary atresia, or this is not diagnosed early enough. And then these kiddos are going to transplant within six months of life.
Scott Webb: Well, yeah, I think you've emphasized that point today, both the red flags, you know, for parents, early diagnosis, early treatment, the procedure that you've mentioned, all good stuff. Doctor, thanks so much for your time today and you stay well.
Dr. Leina Alrabadi: Thank you.
Scott Webb: For more information on biliary atresia, go to stanfordchildrens.org and search biliary atresia. And if you've found this podcast to be helpful, please be sure to tell a friend and subscribe, rate, and review this podcast and check out our entire podcast library for additional topics of interest. This is Health Talks from Stanford Children's Health. I'm Scott Webb. Stay well, and we'll talk again next time.
Scott Webb: Today, we are discussing biliary atresia. Most people never heard of biliary atresia. And although it is a rare condition in children, it is the number one indication of pediatric liver transplants worldwide. And joining me today to educate all of us about this condition, including red flags for parents like jaundice after two weeks is Dr. Leina Alrabadi. She's a pediatric transplant hepatologist with Stanford Children's Health.
This is Health Talks from Stanford Children's Health. I'm Scott Webb. And doctor, thanks so much for your time and your expertise today. As we get rolling here, what is biliary atresia?
Dr. Leina Alrabadi: So biliary atresia is a pediatric liver condition that is what we call progressive obliterative disease of the biliary tree. So that means that there's obliteration or destruction of the bile ducts, which are the channels that carry bilirubin in and out of the liver. And then that destruction of the bile ducts is what causes bilirubin to build up and become toxic in the liver.
Scott Webb: And what causes biliary atresia to occur and who gets it?
Dr. Leina Alrabadi: Unfortunately, the cause of the disease is unknown. But there's a lot of research worldwide to try to understand this disease process. But what we do know is it's not a hereditary disease, so it's not a disease that's passed on from a parent to a child, nor is it a preventable disease. So it's not something that expectant mothers could do anything to avoid, but the cause is what a lot of researchers are trying to understand currently.
Scott Webb: Yeah, and doing a little bit of research myself just to kind of prep myself for this. You know, I watched a couple of videos and did some reading and it does seem like understanding the cause is something that researchers are still working on. But at least we know how it manifests and how to treat it. And specifically, what are the symptoms to suspect biliary atresia in a child?
Dr. Leina Alrabadi: The clinical symptoms that you would suspect occur around two to six weeks of life in a child. I would say that most of these children are born full term, go home a few days after discharge from the hospital from birth, and do well. But then at two weeks, what we say is jaundice persists, so the yellowing of the skin. Again, as we know, yellowing of the skin can be very common in newborn children. But really, past two weeks of life warrants a workup with biliary atresia being on the differential for why that jaundice is still there.
So jaundice is one of the concerns, but the other one would be what we call acholic stools, which means that the stools, the poops for the baby, they don't have that yellow seedy, green or brown color that newborn stools should look like. These stools are gray, white, very light yellow, pale in color and that's what acholic means. And most families don't know that this is abnormal or know to look out for this. So this is a lot of education towards families and providers give families to look out at the color of the stool.
So I would say that acholic stools and jaundice are the big red flags for thinking of biliary atresia. But then other things are just poor weight gain, not growing as appropriately as they should are other things that the pediatrician looks for.
Scott Webb: Yeah, and it's so good as I have two kids myself, it's so good to know what the warning signs are because, you know, those first few weeks are magical and wonderful and worrisome. And so being on the lookout for jaundice after two weeks, looking out for abnormal stools, and I'm sure this all kind of feeds into exactly how do you diagnose biliary atresia?
Dr. Leina Alrabadi: Recognition by the family and what is discussed by the pediatrician also during well-child checks usually prompts the evaluation and then that workup usually will involve, initially it will be blood work. So you're looking at specific bilirubin numbers and and something we call the GGT number and those will be abnormally elevated in a patient with biliary atresia. And that workup will continue on to ultrasound imaging and then, in most scenarios, a liver biopsy.
And then liver biopsy does not diagnose biliary atresia, but it furthers the suspicion for biliary atresia if that liver biopsy shows what we call duct obstruction. So those bile ducts that we talked about earlier are obstructed, and you're kind of seeing that in the tissue of the liver. And then that usually will prompt clinical context, the lab workup to be abnormal, the liver biopsy to be abnormal for a patient to make the gold standard diagnosis of biliary atresia with what we call an intraop cholangiogram.
Scott Webb: Yeah. And I know Stanford Children's Health, we're always looking for the gold standard, which, you know, all parents want for their children. And when we talk about management and treatment, what are the options when it comes to biliary atresia?
Dr. Leina Alrabadi: So we mentioned the intraop cholangiogram, so what that is is that means a child is taken into surgery and a surgeon is able to visualize those bile ducts. And then insert a dye and then take pictures, and then we're able to see where does that dye go? Does that dye just kind of sit there because those ducts are not formed? Or does that dye appropriately go into the intestine? And that was said to be unlikely biliary atresia in that scenario.
But let's say that the patient goes into an intraop cholangiogram and there's no ducts, the ducts are all atretic, that's the diagnosis of biliary atresia. And that prompts what comes next for a patient, which is a Kasai procedure.
The Kasai procedure, it's known as the hepatoportoenterostomy. And the surgery was named after Dr. Morio Kasai. He's a Japanese surgeon who invented it. And what happens during a Kasai is that the surgeon takes part of the baby's intestines and brings it up to the dome of the liver. And this allows for a free-flowing channel. So they're creating a channel that allows the bile that was building up and becoming toxic in the liver to escape through this new duct out of the liver. So creating this channel in place of the atretic bile ducts that were previously unable to kind of get that bile out of the liver.
Scott Webb: It sounds really fascinating. You know, a little bit like science fiction when you're dealing with tiny little babies. It's really amazing. Why is the timing so essential for biliary atresia?
Dr. Leina Alrabadi: The timing is so essential because we talk about the Kasai procedure, but unfortunately with biliary atresia, there's no cure. The treatment that we do with the Kasai is helping the flow of the bilirubin come out of the liver. And the timing is so essential because better outcomes occur when bile flow is restored earlier through the Kasai procedure.
And what that means is it prevents accumulation of that toxic bile in the liver, which is only more damaging when it builds up and causes stiffness, liver scarring, and then what we would say, fibrosis, cirrhosis in a child. And really saying early is really not enough. It really comes down to a matter of days.
Scott Webb: Yeah. And doctor, you mentioned outcomes, and I'm sure for parents who would unfortunately have a child diagnosed with biliary atresia, they're going to want to know. As you say, it's really not so much treatment or cure rather, it's really more management of this. So what are the outcomes?
Dr. Leina Alrabadi: Yeah. So outcomes all relate to the timing of the Kasai. So that's why we say an early Kasai is really important and it really comes down to a matter of days. Always, you want to get a Kasai in before 90 days. But really, the push is now to get it below 60 days, even below 45 days and if possibly by 30 days because really those patients have the most success.
And then again, the goal for the Kasai is to continue to function and allow for appropriate bile flow so that the patient can keep what we say their native liver for as long as possible without needing a liver transplant.
And in terms of outcomes, what we look for is at three months post the Kasai, so after patient has had a Kasai, there's a clear difference in the two years native liver survival of a patient with what their total bilirubuin is at three months. And it's been shown that those who have a total bilirubin less than 2 compared to those with greater than 6, really have much better outcomes. And if the jaundice clears by three months after the Kasai, the 10-year transplant free survival is between 75% to 95%.
And if the jaundice persists after the Kasai, the three-year transplant-free survival is only 20%. So that's why it all comes down to the timing.
Scott Webb: Yeah, as you say, timing's not only important, I mean, it is absolutely critical and it can be a matter of days in terms of the outcomes for these children, these patients. Doctor, as we get close to wrapping up here, and this has been a really interesting conversation, really educational, we've been over this, but let's go back through it again. Why is biliary atresia so difficult to recognize and, ultimately, why is awareness so important?
Dr. Leina Alrabadi: Biliary atresia is so hard to recognize because newborn jaundice is so common. However, as we mentioned, jaundice that occurs after two weeks of life should always be investigated carefully. And again, biliary atresia is rare. It occurs between one and 8,000 to one in 18,000 cases per year according to the American Liver Foundation. But it's important to get this message out there and increase awareness as it is currently the leading diagnosis for liver transplant in children, and definitely something that affects children and families worldwide.
And the last point I would mention is getting a patient to a Kasai is extremely important. Doing that timely is critical, but what the procedure fails to do is prevent some of the biliary cirrhosis that will progress on its own. So really getting that Kasai in timely will prolong the patient with their native liver and decrease their need for liver transplant early in life.
Scott Webb: Yeah. So doctor, obviously the goal is to, you know, have patients have their native livers for as long as possible. But I guess the question that pops into my head is ultimately are all patients who are diagnosed with biliary atresia as children, are they going to need a transplant at some point?
Dr. Leina Alrabadi: Really the answer is yes. And it's very unlikely to make it into their 20s or 30s without needing a liver transplant. But the message is so important because there are patients who what we call have a missed biliary atresia, or this is not diagnosed early enough. And then these kiddos are going to transplant within six months of life.
Scott Webb: Well, yeah, I think you've emphasized that point today, both the red flags, you know, for parents, early diagnosis, early treatment, the procedure that you've mentioned, all good stuff. Doctor, thanks so much for your time today and you stay well.
Dr. Leina Alrabadi: Thank you.
Scott Webb: For more information on biliary atresia, go to stanfordchildrens.org and search biliary atresia. And if you've found this podcast to be helpful, please be sure to tell a friend and subscribe, rate, and review this podcast and check out our entire podcast library for additional topics of interest. This is Health Talks from Stanford Children's Health. I'm Scott Webb. Stay well, and we'll talk again next time.