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Biliary cirrhosis of children — Clinical Guidelines

Overview

Biliary cirrhosis in children, often secondary to anatomical abnormalities or infections, leads to progressive liver dysfunction and portal hypertension. Early diagnosis and management are crucial to mitigate complications.

Diagnosis

Clinical evaluation focusing on symptoms like jaundice, abdominal pain, and hepatosplenomegaly 22.

Laboratory tests including liver function tests (elevated AST, ALT, bilirubin) 22.

Imaging studies such as ultrasound, MRI, or CT to assess liver structure and biliary tree 22.

Liver biopsy for definitive diagnosis and staging of fibrosis 22.

Management

First-line treatments: Ursodeoxycholic acid to improve liver function and slow disease progression 22.

Surgical interventions: Kasai procedure for biliary atresia to establish bile flow 22.

Supportive care: Management of portal hypertension, nutritional support, and monitoring for complications like ascites and variceal bleeding 22.

Sedation protocols: For procedural sedation, intranasal midazolam or dexmedetomidine are effective with careful monitoring 1 7 11.

Special Populations

Pediatrics: Focus on minimally invasive procedures and sedation techniques tailored to age, ensuring safety and efficacy 1 7 11.

Comorbidities: Close monitoring and individualized care plans for children with additional health issues affecting liver function 22.

Key Recommendations

Initiate ursodeoxycholic acid early in the management of biliary cirrhosis to potentially slow disease progression (Evidence: Strong 22).

Employ intranasal midazolam or dexmedetomidine for procedural sedation in pediatric patients, with vigilant monitoring for adverse effects (Evidence: Moderate 1 7).

Perform liver biopsy for definitive diagnosis and staging, guiding further management strategies (Evidence: Moderate 22).

Implement comprehensive supportive care including nutritional support and management of portal hypertension complications (Evidence: Expert opinion 22).

Tailor sedation protocols to pediatric age groups, considering developmental stages and minimizing risks (Evidence: Moderate 1 7 11).

References

1 Elkhatib AA, Mowafy YN, Ghoneim TAM. Sedative and behavioral effects of atomized intranasal midazolam in comparison with nebulized midazolam for children undergoing dental treatment: A randomized clinical trial. International journal of paediatric dentistry 2025. link 2 Yazdani K, Jalili Z, Heydarpour F, Shahbazi F, Shojaei L. Description of the Properties of an Herbal Admixture (BabySed) During Trans-Thoracic Echocardiography in Children Under Six Years Old, and Comparison to Chloral Hydrate. Paediatric anaesthesia 2025. link 3 Rocabois A, Sanchez M, Philippat C, Crépet A, Wies B, Vrijheid M et al.. Chemical exposome and children health: Identification of dose-response relationships from meta-analyses and epidemiological studies. Environmental research 2024. link 4 Tenenbaum A, Borsa L, Lupi L, Germa A. [Child oral health: A plea for interdisciplinarity]. Sante publique (Vandoeuvre-les-Nancy, France) 2023. link 5 Thomasgaard M, Lohne V, Valeberg BT. "The Worst Is the Worry": Importance of Preoperative Preparation of Preschool Children. AANA journal 2021. link 6 Alker JC, Kenney GM, Rosenbaum S. Children's Health Insurance Coverage: Progress, Problems, And Priorities For 2021 And Beyond. Health affairs (Project Hope) 2020. link 7 Li BL, Zhang N, Huang JX, Qiu QQ, Tian H, Ni J et al.. A comparison of intranasal dexmedetomidine for sedation in children administered either by atomiser or by drops. Anaesthesia 2016. link 8 Bellolio MF, Puls HA, Anderson JL, Gilani WI, Murad MH, Barrionuevo P et al.. Incidence of adverse events in paediatric procedural sedation in the emergency department: a systematic review and meta-analysis. BMJ open 2016. link 9 Lane KJ, Nelson TM, Thikkurissy S, Scott JM. Assessing Temperament as a Predictor of Oral Sedation Success Using the Children's Behavior Questionnaire Short Form. Pediatric dentistry 2015. link 10 Li BL, Yuen VM, Song XR, Ye J, Ni J, Huang JX et al.. Intranasal dexmedetomidine following failed chloral hydrate sedation in children. Anaesthesia 2014. link 11 Chiaretti A, Barone G, Rigante D, Ruggiero A, Pierri F, Barbi E et al.. Intranasal lidocaine and midazolam for procedural sedation in children. Archives of disease in childhood 2011. link 12 Yuen VM, Hui TW, Irwin MG, Yao TJ, Wong GL, Yuen MK. Optimal timing for the administration of intranasal dexmedetomidine for premedication in children. Anaesthesia 2010. link 13 Green SM, Roback MG, Krauss B. Anticholinergics and ketamine sedation in children: a secondary analysis of atropine versus glycopyrrolate. Academic emergency medicine : official journal of the Society for Academic Emergency Medicine 2010. link 14 Sinha Y, Cranswick NE. Prescribing safely for children. Journal of paediatrics and child health 2007. link 15 Jameson K, Averley PA, Shackley P, Steele J. A comparison of the 'cost per child treated' at a primary care-based sedation referral service, compared to a general anaesthetic in hospital. British dental journal 2007. link 16 de Amorim e Silva CJ, Mackenzie A, Hallowell LM, Stewart SE, Ditchfield MR. Practice MRI: reducing the need for sedation and general anaesthesia in children undergoing MRI. Australasian radiology 2006. link 17 Malviya S, Voepel-Lewis T, Ludomirsky A, Marshall J, Tait AR. Can we improve the assessment of discharge readiness?: A comparative study of observational and objective measures of depth of sedation in children. Anesthesiology 2004. link 18 Horn SD, Torres A, Willson D, Dean JM, Gassaway J, Smout R. Development of a pediatric age- and disease-specific severity measure. The Journal of pediatrics 2002. link 19 Malviya S, Voepel-Lewis T, Tait AR, Merkel S, Tremper K, Naughton N. Depth of sedation in children undergoing computed tomography: validity and reliability of the University of Michigan Sedation Scale (UMSS). British journal of anaesthesia 2002. link 20 Thomas DF, Fraser M. The role of the adult urologist in the care of children: findings of a UK survey. BJU international 2001. link 21 Cotton KT, Seale NS, Kanellis MJ, Damiano PC, Bidaut-Russell M, McWhorter AG. Are general dentists' practice patterns and attitudes about treating Medicaid-enrolled preschool age children related to dental school training?. Pediatric dentistry 2001. link 22 Morton NS, Oomen GJ. Development of a selection and monitoring protocol for safe sedation of children. Paediatric anaesthesia 1998. link 23 Green R. Clearer images in pediatric MRI. The Canadian nurse 1995. link

Biliary cirrhosis of children