Ultrasound Shear Wave Elastography Evaluation of Suspected and Known Biliary Atresia

Study Overview
=================
Brief Summary
-----------------
The investigators plan to investigate the use of US shear wave elastography (SWE), a newly available imaging technology, in children with suspected/known BA.

Detailed Description
-----------------
The investigators plan a prospective, multi-center study to determine if liver ultrasound shear wave speed measurements acquired at clinical presentation can discriminate biliary atresia (BA) from other causes of liver disease in the setting of neonatal cholestasis. The investigators also plan to determine if liver ultrasound shear wave speed measurements acquired at clinical presentation or longitudinally during the first one-year period after Kasai can predict important long-term outcomes in BA patients.

Official Title
-----------------
Ultrasound Shear Wave Elastography Evaluation of Suspected and Known Biliary Atresia

Conditions
-----------------
Biliary Atresia

Intervention / Treatment
-----------------
* Device: Ultrasound Shear Wave Elastography


Participation Criteria
=================
Eligibility Criteria
-----------------
Inclusion Criteria: neonatal cholestasis with concern for BA vs. other liver disease direct bilirubin >20% of total or ≥2 mg/dL age <3 months Exclusion Criteria: inability to obtain parent/guardian consent. inability to return for follow up visits as required by the study (these patients will be included in aim 1, but will be unable to participate in aim 2). no other exclusion criteria.

Ages Eligible for Study
-----------------
Maximum Age: 3 Months

Sexes Eligible for Study
-----------------
All

Accepts Healthy Volunteers
-----------------
No

Study Plan
=================
How is the study designed?
-----------------

Arms and Interventions

| Intervention/Treatment |
| --- |
|Device: Ultrasound Shear Wave Elastography|The investigators have recently published a very small study in neonates/infants with suspected BA showing that US liver shear wave speed (i.e. - liver stiffness) can differentiate BA from other causes of cholestasis, potentially with a very high degree of accuracy. Additional research is needed to confirm these very preliminary results and determine if liver shear wave speed measurements either before or after (including longitudinally) Kasai can predict key outcomes.|

What is the study measuring?
-----------------
Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Discrimination of biliary atresia from other liver diseases using ultrasound liver shear wave speed (Aim 1) | The primary outcome measure will be ultrasound shear wave speed of the liver. The investigators will compare the mean liver shear wave speed measurements in children with biliary atresia to other liver diseases. Sensitivity and specificity of ultrasound for diagnosing biliary atresia will be determined using multiple shear wave speed cut-off values. | 2 years | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Predicting long term clinical outcomes in biliary atresia patients using ultrasound liver shear wave speed (aim 2) | Change in shear speed liver over time will be compared between children with biliary atresia versus other liver diseases in order to determine if differences predict important clinical outcomes. | 6 years |