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Paeds Vivasgastroenterology-hepatology-and-nutrition

Paeds Vivas · gastroenterology-hepatology-and-nutrition

Chronic liver disease, cirrhosis and portal hypertension: Viva

Branching clinical structured oral on chronic liver disease, cirrhosis and portal hypertension in children: recognising the presentation of portal hypertension, the classification of causes, the acute variceal bleeding management bundle, primary prophylaxis, spontaneous bacterial peritonitis, and hepatopulmonary syndrome.

branching clinical structured oral3 min readVerification in progress

Target exams

RACP DWERACP DCEMRCPCH Clinical
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Target exams

RACP DWERACP DCEMRCPCH Clinical
Prompt
A general practitioner refers a seven-year-old girl with biliary atresia who had a Kasai portoenterostomy in infancy. Her routine blood tests show a platelet count of 78,000 per cubic millimetre, and her mother reports that her abdomen looks more swollen. The examiner asks how you would assess and manage this child.

Branch 1: Assessing portal hypertension

The candidate should recognise that a child with biliary atresia and a Kasai portoenterostomy is at high risk for progressive fibrosis and portal hypertension, and that the thrombocytopenia of 78,000 per cubic millimetre is a strong clue to clinically significant portal hypertension from hypersplenism. The key is to interpret the platelet count not as an isolated abnormality but as a marker of splenic sequestration from portal venous congestion. [1]

A strong candidate would explain that the hepatic venous pressure gradient of 10 mmHg or more defines clinically significant portal hypertension, which is the threshold at which varices form and the risk of bleeding becomes real. The candidate should describe the focused examination looking for splenomegaly, ascites with shifting dullness, spider naevi, palmar erythema, and signs of chronic liver disease, and should request an abdominal ultrasound with Doppler to assess portal vein patency, flow direction, splenomegaly, and the presence of collaterals. [1]

If the examiner presses on the next step, the candidate should name upper gastrointestinal endoscopy as the gold standard for diagnosing and characterising varices, and should explain that the finding of large varices or red wale signs would prompt consideration of primary prophylaxis with either non-selective beta-blockers or endoscopic variceal ligation. [1]

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References3ShowHide
  1. [1]de Franchis R, Bosch J, Garcia-Tsao G, Reiberger T, et al Baveno VII - Renewing consensus in portal hypertension. J Hepatol, 2022.PMID 35120736
  2. [2]Cifuentes LI, Gattini D, Torres-Robles R, Gana JC Beta-blockers versus placebo or no intervention for primary prophylaxis of oesophageal variceal bleeding in children with chronic liver disease or portal vein thrombosis. Cochrane Database Syst Rev, 2021.PMID 33498095
  3. [3]Krowka MJ, Fallon MB, Kawut SM, Fuhrmann V, et al International Liver Transplant Society Practice Guidelines: Diagnosis and Management of Hepatopulmonary Syndrome and Portopulmonary Hypertension. Transplantation, 2016.PMID 27326810
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