The Saudi Journal of Gastroenterology (Jan 2024)

Clinical and laboratory features of biliary atresia and patterns of management practices: Saudi national study (2000–2018)

  • Homoud Alhebbi,
  • Mohammed El-Edreesi,
  • Mohammed Abanemai,
  • Omar Saadah,
  • Maher Alhatlani,
  • Hana Halabi,
  • Razan Bader,
  • Ahmed Al Sarkhy,
  • Ahmed Aladsani,
  • Sami Wali,
  • Talal Alguofi,
  • Nawaf Alkhathran,
  • Amira NasserAllah,
  • Muhammed Salman Bashir,
  • Abdulrahman Al-Hussaini

DOI
https://doi.org/10.4103/sjg.sjg_151_23
Journal volume & issue
Vol. 30, no. 2
pp. 89 – 95

Abstract

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Background: We utilized the data from the Saudi national biliary atresia (BA) study (2000–2018) to describe the clinical, biochemical, imaging, and histopathological features of BA and the perioperative clinical practices among local pediatric gastroenterologists. Methods: This is a retrospective, multicenter, nationwide study that included 10 tertiary care governmental hospitals including the four liver transplant (LT) centers in different regions across Saudi Arabia. Results: BA was diagnosed in 204 infants (106 females; 10% preterm). The median age at referral was 65 days. Congenital anomalies were present in 68 patients (33%); 22 were splenic malformation (10.8%). The medians of laboratory investigations were total bilirubin (189 μmol/l), direct bilirubin (139 μmol/l), ALT (164 u/l), and GGT (472 u/l). The level of serum GGT was normal in 26 cases (12.7%). The ultrasound findings included hypoplastic or atrophic gall bladder (GB) (65%), normal GB (30%), and cord sign (5%). A HIDA scan was performed in 99 cases (48.52%). Magnetic resonance cholangiopancreatography (MRCP) was performed in 27 cases (13%). A total of 179 liver biopsies (88%) were obtained. The most common histopathologic findings were bile duct proliferation (92%), canalicular cholestasis (96%), bile plugs (84%), and portal fibrosis (95%). Cholangiography was performed in 139 cases (68%): operative in 122 (60%) and percutaneous in 17 (8%). A total of 143 children (70%) underwent Kasai portoenterostomy (KPE) at a median age of 70 days. After KPE, steroid was used in 37% of the cases and 100 cases (70%) were prescribed prophylactic antibiotics for variable duration (ranging between 3 and 12 months). Conclusion: Our data show marked variation in the diagnostic evaluation and perioperative management of BA cases among the different tertiary centers. There is a need to establish a national BA registry in Saudi Arabia aiming to standardize pre- and postoperative clinical practices. Additionally, normal serum GGT level, normal GB size on ultrasound, and being a premature baby should not preclude the diagnostic workup for BA.

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