Part II: Granulocyte-Colony Stimulating Factor Adjunct Therapy for Biliary Atresia (BA_GCSF2b)
Biliary Atresia
About this trial
This is an interventional treatment trial for Biliary Atresia
Eligibility Criteria
Inclusion criteria
- preliminary work up for cholestasis suspected or inconclusive diagnosis of BA.
- Serum Direct bilirubin > 2 mg/dl,GGT> 100 U/L
- Male or female infants with a gestational age> 36 weeks
- Admission weight > 2 kg
- Age > 14 days - 180 days at diagnosis
- For Kasai operated subjects, Type 3 or 4 anatomy of BA
- For Kasai operated subjects, cholangiogram (if performed) diagnostic of BA
- Liver biopsy supporting BA diagnosis
Exclusion criteria
- Patients having access to liver transplantation for immediate liver failure
- Prior Kasai patients
- Major cardiac, renal, central nervous system (CNS) malformations
- Intracranial hemorrhage
- History of recent total parenteral nutrition (TPN) use within the last 2 weeks
Gl tract obstruction
For Kasai-operated subjects: Type 1 or 2 biliary atresia anatomy
- Current systemic infection
- WBC > 20,000 cells/uL
- Platelet count < 20,000 cells/uL or >1 million cells/uL
- Concurrent respiratory, metabolic, neurological, cardiovascular, metabolic, and renal illness
- Elevated serum creatinine > 1 mg/dL
- Purpura fulminans or unexplained vascular thrombosis
Sites / Locations
- Oregon Health & Science University (OHSU)
- Aga Khan University
- Nation Children's HospitalRecruiting
- Children Hospital 1
Arms of the Study
Arm 1
Arm 2
Arm 3
Arm 4
Experimental
No Intervention
Experimental
No Intervention
Kasai GCSF
Kasai no GCSF
No Kasai GCSF
No Kasai No GCSF
The Kasai GCSF group will receive the standard of care PLUS 3 consecutive daily doses of 10 ug/kg of GCSF to be administered subcutaneously by day 3 post Kasai surgery
The no GCSF group will not receive GCSF and receives the standard of care
The No Kasai GCSF group will receive the standard of care PLUS 3 consecutive daily doses of 10 ug/kg of GCSF to be administered subcutaneously once the diagnosis of BA is established
The No Kasai No GCSF group will receive the standard of care and will not receive GCSF