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Clinical Characteristics and Outcome of Infants with Biliary Atresia in Bahrain
Hasan M Isa1,2, Zainab Irshad1
1Pediatric Department, Salmaniya Medical Complex, Manama, Bahrain.
Insights
Biliary atresia (BA) is uncommon in Bahrain, but late diagnosis leads to poor outcomes. Early detection through infant stool color screening and timely treatment are crucial for improving survival rates in infants with BA.
Area of Science:
- Pediatric Hepatology
- Gastroenterology
- Public Health
Background:
- Biliary atresia (BA) presents a significant challenge in pediatric liver disease, despite its low prevalence in the Middle East.
- Early diagnosis and intervention are critical for improving outcomes in infants with BA.
Purpose of the Study:
- To determine the prevalence, clinical features, diagnostic methods, and surgical outcomes of biliary atresia in Bahrain.
- To analyze prognostic factors influencing survival rates in infants diagnosed with BA.
Main Methods:
- A retrospective cohort study of infants diagnosed with BA between 1993 and 2021 at Salmaniya Medical Complex, Bahrain.
- Analysis of demographic, clinical, laboratory, imaging, and histopathological data.
- Outcomes included 5-year native liver survival (NLS) and overall liver survival (OLS) rates.
Main Results:
- The study identified 26 infants with BA, with an overall prevalence of 0.007%.
- Key clinical signs included clay-colored stools (86.4%) and hepatomegaly (81.8%).
- Native liver survival was 53.8% and overall liver survival was 65.4%, with a 34.6% mortality rate. Lower birth weight and older age at diagnosis were associated with better survival.
Conclusions:
- Biliary atresia, while uncommon in Bahrain, is associated with significant morbidity and mortality, often due to delayed diagnosis.
- Implementing infant stool color screening could enhance early detection and parental awareness.
- Improved early detection and novel therapeutic approaches are essential for enhancing survival rates in BA patients.
Objectives:
Though the prevalence of biliary atresia (BA) in the Middle East is low, its role in causing life-threatening liver diseases is disproportionately high. This study aimed to assess the prevalence, clinical presentations, diagnosis, surgical interventions, and long-term outcomes including survival rate, and to analyze prognostic factors affecting the survival of infants with BA in Bahrain.
Methods:
A retrospective cohort review of cases of infants diagnosed with BA between January 1993 and December 2021 at Salmaniya Medical Complex, Bahrain, was conducted. Demographic, clinical, laboratory, imaging, and histopathological data were analyzed. Outcomes measured included 5-year native liver survival (NLS) and overall liver survival (OLS) rates.
Results:
Out of 27 infants diagnosed with BA, 26 were included in the study. The overall prevalence was 0.007% and was significantly higher among Bahraini (0.006%) compared to the non-Bahraini population (0.001%), p< 0.001. Most patients had clay-colored stool (19 of 22; 86.4%) and hepatomegaly (18; 81.8%). Kasai procedure was performed in 23 (88.5%) patients and primary liver transplant (LT) in three. Ten (38.5%) infants required LT after the Kasai procedure. The median age at diagnosis was 56 days with interquartile range (IQR) of 30-90, and the median age at the Kasai procedure was 61 days (IQR = 56-90). NLS and OLS rates were 53.8% and 65.4%, respectively. The overall mortality rate was 34.6%. Six of the 13 (46.2%) patients who were subjected to Kasai procedure alone died awaiting LT donors. Infants with lower birth weight and older age at diagnosis had significantly better odds of survival (p< 0.029 and p< 0.044, respectively).
Conclusions:
BA is uncommon among infants in Bahrain. Despite effective treatment options, late diagnosis can lead to significant morbidity and mortality. Our study found a relatively low prevalence of NLS (53.8%) and OLS (65.4%). Since the majority of the diagnosed patients had presented with clay-colored stools, implementing an infant stool card for screening may be beneficial in raising parental awareness and detecting BA early. Early detection and treatment using novel therapies are likely to improve the survival rates.
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