Evaluation of the Use of Granulocyte Colony Stimulating Factor (GCSF) in Post Kasai Type 3 Biliary Atresia

NCT ID: NCT06708572

Last Updated: 2024-12-11

Study Results

Results pending

The study team has not published outcome measurements, participant flow, or safety data for this trial yet. Check back later for updates.

Basic Information

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Recruitment Status

RECRUITING

Clinical Phase

NA

Total Enrollment

40 participants

Study Classification

INTERVENTIONAL

Study Start Date

2024-12-31

Study Completion Date

2026-10-30

Brief Summary

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The aim of the study is to evaluate the use of Granulocyte Colony Stimulating Factor (GCSF) on the clinical and biochemical outcome of type 3 biliary atresia post kasai.

Detailed Description

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Biliary atresia (BA) is a devastating disease manifest early in infancy characterized by bile duct injury and extrahepatic biliary obstruction, leading to cirrhosis in the majority of infants.

Although BA is a rare disease, occurring in \~1 in 5600 to 1 in 18,000 infants worldwide, it is considered the most common indication for liver transplantation in children.

However, despite a 50-60% rate of initial jaundice clearance, liver transplantation by 2 years of age is necessary for long term survival in many of the post-Kasai patients.

GCSF cytokine that stimulates neutrophil and hematopoietic stem cell (HSC) production and mobilization from the bone marrow, and has served as a complementary agent to bone marrow stem cell therapy for patients with congenital or acquired diseases of bone marrow suppression.

Granulocyte colony-stimulating factor (G-CSF) mobilizes CD34+(cluster of differentiation34) cells, these CD34+ cells increase hepatocyte growth factor inducing the proliferation of hepatic progenitor cells within 7 days.

In experimental liver diseases of toxin-induced or bile duct ligation-induced liver injury, GCSF-based stem cell therapy has the same effects as direct HSC transplantation on improving liver regeneration and suppressing the inflammatory and fibrotic responses to hepatic injury. The cellular and molecular mechanisms are unknown but are postulated to be derived from the many paracrine actions of GCSF.

Conditions

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Granulocyte Colony-stimulating Factor Biliary Atresia

Study Design

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Allocation Method

NON_RANDOMIZED

Intervention Model

PARALLEL

Protocol of cohort study will be conducted on 40 infants with the final diagnosis of biliary atresia type 3 (supported by the liver histology and intra-operative findings) divided into two groups one of them is for GCSF after Kasai operation, and the other group will be without GCSF therapy and their data will be collected retrospectively.
Primary Study Purpose

PREVENTION

Blinding Strategy

NONE

Study Groups

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biliary atresia without GCSF

20 infants with the final diagnosis of biliary atresia type 3 (supported by the liver histology and intra-operative finding) for GCSF after Kasai operation will not receive GCSF.

Group Type NO_INTERVENTION

No interventions assigned to this group

biliary atresia with GCSF

20 infants with the final diagnosis of biliary atresia type 3 (supported by the liver histology and intra-operative finding) for GCSF after Kasai operation will receive GCSF.

Group Type EXPERIMENTAL

Granulocyte Colony-Stimulating Factor

Intervention Type DRUG

20 patients will receive subcutaneous GCSF at a daily dose of 10ug/kg for 3 consecutive days. It is administered within 3-4 days after the Kasai procedure.

Interventions

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Granulocyte Colony-Stimulating Factor

20 patients will receive subcutaneous GCSF at a daily dose of 10ug/kg for 3 consecutive days. It is administered within 3-4 days after the Kasai procedure.

Intervention Type DRUG

Eligibility Criteria

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Inclusion Criteria

* Infants with initial diagnosis of biliary atresia with biliary atresia score \> 23.927 will be allocated for Kasai porto-enterostomy with intra-operative cholangiogram reaching type 3 biliary atresia anatomy as a final diagnosis.

Exclusion Criteria

* Major cardiac, renal, pulmonary, neurological malformations or illnesses.
* Hemoglobinopathies, such as sickle cell anemia
* Active systemic infection.
* White blood cell count \> 20,000 cells/mm3.
* Platelet count \< 40,000 cells/mm3 or ≥ 800,000 cells/mm3.
* Purpura fulminans or unexplained vascular thrombotic conditions.
Minimum Eligible Age

20 Days

Maximum Eligible Age

1 Year

Eligible Sex

ALL

Accepts Healthy Volunteers

Yes

Sponsors

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National Liver Institute, Egypt

OTHER

Sponsor Role lead

Responsible Party

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Responsibility Role SPONSOR

Locations

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National Liver Institute

Cairo, Menofia Governorate, Egypt

Site Status RECRUITING

Countries

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Egypt

Central Contacts

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Marwa Narwa Foad Asker, master

Role: CONTACT

Phone: +201008913103

Email: [email protected]

Behairy Behairy El Sayed Behairy, MD

Role: CONTACT

Phone: +201001847754

Email: [email protected]

Facility Contacts

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Behairy El Sayed El Sayed Behairy, MD

Role: primary

Gasser Gasser El-Azab, MD

Role: backup

References

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Bezerra JA, Wells RG, Mack CL, Karpen SJ, Hoofnagle JH, Doo E, Sokol RJ. Biliary Atresia: Clinical and Research Challenges for the Twenty-First Century. Hepatology. 2018 Sep;68(3):1163-1173. doi: 10.1002/hep.29905.

Reference Type BACKGROUND
PMID: 29604222 (View on PubMed)

Overi D, Carpino G, Cardinale V, Franchitto A, Safarikia S, Onori P, Alvaro D, Gaudio E. Contribution of Resident Stem Cells to Liver and Biliary Tree Regeneration in Human Diseases. Int J Mol Sci. 2018 Sep 25;19(10):2917. doi: 10.3390/ijms19102917.

Reference Type BACKGROUND
PMID: 30257529 (View on PubMed)

Corrado MM, Mack CL. Diagnostic Tools for Early Detection of Biliary Atresia: Is a Newborn Screen Attainable? Clin Liver Dis (Hoboken). 2022 Jan 24;19(1):25-28. doi: 10.1002/cld.1165. eCollection 2022 Jan.

Reference Type BACKGROUND
PMID: 35106146 (View on PubMed)

Other Identifiers

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GCSFin biliary atresia

Identifier Type: -

Identifier Source: org_study_id