Ahmad Anouti, MD, is a pediatric resident at the University of Texas Southwestern in the Physician Scientist Training Program.

Ahmad Anouti, MD

Recognizing the Signs and Rewriting the Story Around Biliary Atresia

On Rare Disease Day, attention turns to conditions that may be uncommon, but profoundly life-altering for the families they touch. Among them is biliary atresia, a rare, progressive cholestatic liver disease affecting approximately 1 in 10,000 to 20,000 infants.

Though babies often appear healthy in the first weeks of life, the clock is ticking, with early recognition potentially meaning the difference between preserving a child’s native liver and facing the need for transplantation.

Biliary atresia is characterized by inflammation and obstruction or absence of the bile ducts. Without a pathway to drain bile, the liver becomes progressively damaged, leading to cirrhosis and liver failure.

The earliest sign is often jaundice, a common finding in newborns. While physiologic or breast milk jaundice accounts for most cases, a subset of infants have elevated direct (conjugated) bilirubin—a red flag that requires urgent evaluation. Fractionated bilirubin testing is critical, and missing that distinction can delay diagnosis beyond the ideal window for intervention.

The primary surgical treatment, hepatoportoenterostomy, also known as the Kasai procedure, connects the liver directly to the intestine to restore bile flow. Outcomes are closely tied to timing, with success rates declining significantly after 60 days of life. As Ahmad Anouti, MD, a pediatric resident at UT Southwestern, explains, every day of delay matters. When the Kasai fails or diagnosis comes too late, liver transplantation becomes the only definitive therapy, bringing lifelong immunosuppression and its associated risks.

For Ashley and Taylor Rhoades, their son’s biliary atresia diagnosis triggered an immediate shift from parent to caregiver, forcing rapid decisions under extraordinary emotional strain. Their story underscores the need for clearer communication, coordinated multidisciplinary care, and empathy tailored to each family’s journey.

Encouragingly, Samar Ibrahim, MD, medical director of the pediatric liver transplant program within the Transplant Center at Mayo Clinic, highlights advances in noninvasive biomarkers, genetic cholestasis panels, and improved imaging techniques that are helping streamline diagnosis. Emerging therapies may further extend native liver survival and improve quality of life.

This Rare Disease Day spotlight underscores a central truth: rare does not mean invisible. Biliary atresia demands vigilance from frontline clinicians, advocacy from families, and sustained innovation from researchers. Through stories like these, awareness becomes action, and action creates the possibility of brighter futures.

Mayo Clinic. Biliary Atresia. February 24, 2026. Accessed February 28, 2026. 

https://www.mayoclinic.org/diseases-conditions/biliary-atresia/symptoms-causes/syc-20591855

Anouti A. Advancing Prognostic Tools in Biliary Atresia Care, With Ahmad Anouti, MD. HCPLive. February 18, 2026. Accessed February 28, 2026. 

https://www.hcplive.com/view/advancing-prognostic-tools-in-biliary-atresia-care-with-ahmad-anouti-md

Brooks A. New Biliary Atresia Guidance Released by American Academy of Pediatrics. HCPLive. February 21, 2025. Accessed February 28, 2026. 

https://www.hcplive.com/view/new-biliary-atresia-guidance-released-american-academy-pediatrics

Videos

Families and clinicians highlight the race against time in biliary atresia, where early recognition can change the course of a child’s life.

Rare but Critical: Recognizing the Signs and Rewriting the Story Around Biliary Atresia

Advancing Prognostic Tools in Biliary Atresia Care

Early recognition remains the single most important determinant of outcomes in biliary atresia, a rare but life-threatening cholestatic liver disease of infancy.

Hepatoportoenterostomy, also known as a Kasai procedure, can effectively treat biliary atresia and delay, or in some cases prevent, the need for a liver transplant, especially when performed early. While several serum markers have been suggested to better predict post-hepatoportoenterostomy outcomes, emerging research is shedding light on the potential utility of serum predictors of native liver survival in these patients.

In a recent interview with HCPLive, Ahmad Anouti, MD, a pediatric resident at UT Southwestern, underscored how easily biliary atresia can be overlooked and how costly delays can be.

Biliary atresia is characterized by absent, collapsed, or nonfunctional bile ducts, preventing bile from draining from the liver into the intestine. As bile accumulates, progressive inflammation and fibrosis lead to cirrhosis. Clinically, infants present with jaundice driven by rising direct bilirubin levels.

“The most common causes of jaundice are physiologic: breastfeeding, breast milk, just natural jaundice, and so and the majority of babies are jaundice, more than 60%,” Anouti explained. “Most of the time, it is not biliary atresia, but if it is biliary atresia, and we're saying ‘Oh, this is normal jaundice, this is physiological, we're not fractionating,’ and we're missing the direct bilirubin, then the kids are going to get worse.”

Timing is critical for the primary surgical intervention, hepatoportoenterostomy, which connects the intestine directly to the liver in an attempt to restore bile flow. Outcomes decline significantly after 60 days of life, with each day of delay associated with increased mortality risk. If the Kasai fails, liver transplantation becomes the only definitive option.

Against this clinical backdrop, Anouti and colleagues conducted a meta-analysis, published in the 

Journal of Pediatric Gastroenterology and Nutrition

, examining serum biomarkers as potential prognostic tools in biliary atresia. While direct bilirubin and other markers help establish diagnosis, reliable predictors of long-term outcomes remain elusive. The team systematically reviewed existing literature to identify serum values that may correlate with prognosis.

Their findings suggest several potential thresholds. Elevated direct bilirubin > 1 mg/dL within the first 48 to 72 hours of life may signal biliary atresia. Beyond diagnosis, persistent direct bilirubin levels > 1 mg/dL at 12 months may indicate a poorer prognosis. Total bile acids also show promise as longer-term prognostic indicators.

However, as Anouti noted, the analysis is limited by the quality and heterogeneity of the underlying studies. Additionally, he pointed out much of the existing literature focuses on diagnosis rather than risk stratification.

Ultimately, the study highlights both progress and gaps. While early detection remains paramount, advancing prognostic tools could help clinicians better counsel families, tailor surveillance, and identify patients most likely to require transplant, moving the field beyond diagnosis toward precision management.

Anouti A, Dahshi H, Cotter TG, Rabbani TA, Corbitt N, Hassan S. Serum predictors of native liver survival post-Kasai: Systematic review and meta-analysis. 

. Published online January 29, 2026. doi:10.1002/jpn3.70355

NIDDK. Treatment for Biliary Atresia. September 2017. Accessed February 18, 2026. 

https://www.niddk.nih.gov/health-information/liver-disease/biliary-atresia/treatment

Anouti outlines why early bilirubin testing and emerging serum markers are key to improving prognosis in biliary atresia.

Advancing Prognostic Tools in Biliary Atresia Care, With Ahmad Anouti, MD

Diagnostic Challenges and Transplant Outcomes in Pediatric Cholestatic Liver Disease, With Ahmad Anouti, MD

New research is shedding light on racial disparities in post-liver transplant outcomes among children with cholestatic liver disease.

The analysis of United Network for Organ Sharing (UNOS) data identified greater post-transplant mortality among non-Hispanic Black children with cholestatic liver disease compared with non-Hispanic White patients, even after adjustment for clinical factors. Although the study could not determine causality, the findings raise concern that delays in recognition and diagnosis may contribute to these disparities.

“Anecdotally, you hear a lot of patients say jaundice was detected too late,” study author Ahmad Anouti, MD, a pediatric resident at UT Southwestern, told 

. “To be honest with you, many clinicians and practitioners have a difficult time realizing jaundice in patients of different skin color tones. We're not well trained or well versed on that.”

Inspired by uncertainty regarding whether delays in post-transplant outcomes are based on race and ethnicity, Anouti and colleagues retrospectively examined UNOS data for 3501 pediatric patients ≤ 18 years of age who were listed or received a liver transplant for cholestatic liver disease between 2010 and 2024. 

showed non-Hispanic White recipients were more likely to have private insurance (56.7% vs 18.2%; 

 <.01) and experienced shorter waitlist times than Hispanic patients (175.9 vs 189.4 days; 

 <.01). Of note, the 1-year survival rate was lower in non-Hispanic Black than in non-Hispanic White recipients (94.8% vs 96.8%; 

 = .04), with no significant difference at 5 years (93.7% vs 95.4%; 

While Hispanic patients had similar 1-year (95.9%; 

 = .06) survival rates to non-Hispanic White patients, non-Hispanic Black recipients had a significantly increased risk of mortality with (hazard ratio [HR], 1.53; 95% CI, 1.05–2.22). Predictive modeling using extreme gradient boosting showed a relative likelihood of increased mortality in non-Hispanic Black (3.91%) and Hispanic (3.14%) recipients.

“We feel that this might [suggest] that maybe patients of different ethnicities and backgrounds are seen at a later time due to more challenging diagnosis and not being taken as seriously as other patients,” Anouti explained, describing the importance of always starting a clinical work up whenever jaundice is suspected.

Anouti additionally emphasized the need to look beyond the skin alone. Although he says jaundice is the most commonly discussed sign of pediatric cholestatic liver disease in medical education, he describes the importance of also considering things like the eyes, gums, and fingernails, which may offer better insight than skin examination alone.

“I think it's sad that in this day and age, we have kids actively dying or having worse outcomes just because we're delaying care,” he said.

Editors’ Note: Anouti has no relevant disclosures.

Anouti A, Dahshi H, Rady ED, et al. Racial Disparities in Pediatric Patients With Cholestatic Liver Disease Undergoing Liver Transplant: A UNOS Database Study. 

. 2026;30(1):e70261. doi:10.1111/petr.70261

Anouti describes clinical uncertainties regarding signs of cholestatic liver disease, like jaundice, in patients with skin of color, and how this may impact transplant outcomes.

Simplifying, Expediting Biliary Atresia Diagnosis, with Ahmad Anouti, MD

Recent guidance from the American Academy of Pediatrics (AAP) is helping facilitate the early identification of 

 in infants, providing pediatric primary care providers with a novel strategy to simplify and expedite the process.

Intended to be used between 2 and 4 weeks of life at the “By 1 month” well-child visit in the Bright Futures/AAP “Recommendations for Preventive Pediatric Health Care,” the strategy involves examining infants’ eye color, stool color, and prior laboratory results to determine whether measurement of a direct or conjugated bilirubin level is warranted.

Biliary atresia is estimated to impact 1 in 8,000 to 1 in 18,000 live births worldwide, with symptoms typically appearing between 2 and 6 weeks after birth. The trapped bile can quickly cause liver damage and cirrhosis, eventually leading to liver failure, but outcomes can be improved with early diagnosis and surgery.

“The key here is to catch it early and catch it fast,” Ahmad Anouti, MD, a pediatric resident at the University of Texas Southwestern in the Physician Scientist Training Program, explained to 

. “If we do, the success and the outcomes have been proven time and time again to be better."

Anouti emphasized the importance of the strategy provided by the recent AAP guidance, citing frequent overcomplication in medicine as a whole when it comes to diagnoses and testing. For biliary atresia, he noted the step-by-step guide provided by AAP can simplify the diagnostic process for any primary pediatrician, ideally expediting linkage to a hepatologist.

In addition to being a medical doctor, Anouti is also a patient advocate. Having been diagnosed with biliary atresia as a child and eventually requiring a liver transplant after receiving a late Kasai procedure when he was about 77 days old, Anouti told 

 he wishes the AAP guideline had existed when he needed it as a child.

“With a guideline like this and with an approach like this, the answers come quicker and the treatment comes quicker,” he explained. “As a patient and as a resident in training, it's really eye-opening to see how much these minutes or days or months impact the patient's life, decrease the anxiety, make the answers come quicker, and really make the patient feel like this is being taken seriously and not dragging on.”

Anouti went on to describe how he considers himself to be a patient before a doctor, and the unique perspective this equips him with during his research and clinical work.

“It's very humbling and very exciting to see guidelines like this come out, because it shows me how the field is pushing forward,” he said.

Editors’ note: Anouti has no relevant disclosures.

Brooks A. New Biliary Atresia Guidance Released by American Academy of Pediatrics. 

. February 21, 2025. Accessed March 4, 2025. 

Anouti discusses the new AAP biliary atresia guidance and what it means to him as a patient-advocate who had biliary atresia as an infant.

Ahmad Anouti, MD

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