Biliary atresia in adolescents and young adults

Mclin, Valérie Anne

doi:10.1002/cld.252

Cited by 2 publications

(2 citation statements)

References 20 publications

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“…Early diagnosis is pivotal for the best outcomes from the surgical intervention known as Kasai portoenterostomy (KPE). The outcome following this surgery is variable, with the national success rate—assessed as clearance of jaundice at 6 months, being 57% in the UK 42. In children with failed KPE, mortality is high without liver transplantation.…”

Section: Common Liver Diseases In the Adolescentmentioning

confidence: 99%

Liver disease in adolescents

2022

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In this article, we discuss common liver diseases in the adolescent population. We describe the initial evaluation of an adolescent presenting with new-onset liver enzyme abnormalities, based on the clinical history and physical examination. The management approach to the adolescent with liver disease is exemplified, including monitoring for adherence, risk-taking behaviours and focusing on psychosocial aspects of their care. Finally, we highlight the challenges of caring for the adolescent patient and the importance of addressing not only the liver disease but, more importantly, the holistic approach towards their management.

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Section: Common Liver Diseases In the Adolescentmentioning

confidence: 99%

Liver disease in adolescents

2022

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“…Despite being a rare pediatric disease with a prevalence of about one per 20 000 live births, biliary atresia (BA) is the most frequent reason for surgery in infants with persistent jaundice and the leading cause of pediatric liver transplants (LTs). 1 , 2 BA is an idiopathic inflammatory liver disease that manifests in the neonatal period, and characterized by progressive fibrosis and obstruction of both intrahepatic and extrahepatic bile ducts. 3 It presents with symptoms of cholestatic jaundice, which, if left untreated, results in cirrhosis and death in 50–80% of patients within a year and 90–100% at the age of three.…”

Section: Introductionmentioning

confidence: 99%

Clinical Characteristics and Outcome of Infants with Biliary Atresia in Bahrain

Isa¹,

Irshad²

2023

Oman Med J

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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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Biliary atresia in adolescents and young adults

Cited by 2 publications

References 20 publications

Liver disease in adolescents

Liver disease in adolescents

Clinical Characteristics and Outcome of Infants with Biliary Atresia in Bahrain

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