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Paeds SAQsgastroenterology-hepatology-and-nutrition

Paeds SAQs · gastroenterology-hepatology-and-nutrition

Non-alcoholic fatty liver disease in children — formative SAQs

Two formative SAQs on paediatric fatty liver disease, now named metabolic dysfunction-associated steatotic liver disease: the overweight eleven-year-old boy found on screening to have a mildly raised alanine aminotransferase who needs the NASPGHAN sex-specific thresholds and family-based lifestyle therapy, and the obese adolescent with persistent enzyme elevation who needs exclusion of mimics, fibrosis staging, and a discussion of pharmacotherapy and bariatric surgery.

20 marks30 min4 min readVerification in progress

Target exams

RACP General PaediatricsRACP DWEMRCPCH TheoryABP General Pediatrics
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Study tools

Target exams

RACP General PaediatricsRACP DWEMRCPCH TheoryABP General Pediatrics
Prompt
Paediatric fatty liver disease (MASLD)

SAQ 1 — The overweight child with a mildly raised ALT (20 marks, ~15 minutes)

An eleven-year-old boy is reviewed at a routine visit. His body mass index is on the ninety-second percentile. He drinks two sugar-sweetened cans of soft drink a day, plays no organised sport, and spends most evenings on a screen. His alanine aminotransferase is 34 units per litre; the laboratory reports its normal range as up to 55 units per litre. His father has type 2 diabetes. [1]

Questions

  1. Give the most likely diagnosis and explain why the laboratory reference range may be misleading. (4 marks) [1]
  2. State the NASPGHAN 2017 sex-specific upper limits of normal for alanine aminotransferase and the age at which screening should begin. (4 marks) [1]
  3. Outline how you would confirm the diagnosis and the threshold for formal evaluation and referral. (4 marks) [6]
  4. Describe your first-line management and the weight-loss target associated with histological improvement. (5 marks) [1]
  5. Summarise the new nomenclature and why it matters. (3 marks) [4]

Model answer (must-hit)

  1. The most likely diagnosis is paediatric fatty liver disease, now named metabolic dysfunction-associated steatotic liver disease, given the overweight body mass index, the central adiposity pattern, the insulin-resistance family history, and the sedentary high-sugar lifestyle. The laboratory reference range is misleading because it was derived from populations that included people with undiagnosed fatty liver disease and is far higher than the metabolically healthy ninety-fifth percentile, so a value that looks in-range to the laboratory is in fact abnormal by the NASPGHAN thresholds. [1]
  2. The NASPGHAN 2017 upper limits of normal are 22 units per litre for girls and 26 units per litre for boys, and screening with alanine aminotransferase should begin between ages nine and eleven years for all children with obesity and for children with overweight who have additional cardiometabolic risk factors. [1]
  3. Confirm the elevation is persistent by repeating the alanine aminotransferase, and pursue formal evaluation when it remains above twice the sex-specific upper limit of normal for more than three months, or exceeds 80 units per litre at any time. The evaluation excludes viral, autoimmune, Wilson, alpha-1-antitrypsin, drug and metabolic causes, confirms steatosis and stages fibrosis non-invasively with elastography, and refers to paediatric gastroenterology or hepatology. [6]
  4. First-line management is family-based lifestyle change: a calorie-appropriate diet with a sharp reduction in sugar-sweetened beverages and fructose, increased fruit, vegetables and fibre, portion control, at least one hour of moderate to vigorous activity daily, and reduced sedentary screen time, engaging the whole household. A weight reduction of around 7 to 10 per cent is associated with histological improvement of steatohepatitis. [1]
  5. The 2023 multisociety Delphi consensus renamed non-alcoholic fatty liver disease to metabolic dysfunction-associated steatotic liver disease and non-alcoholic steatohepatitis to metabolic dysfunction-associated steatohepatitis, defining the disease by steatosis plus at least one cardiometabolic criterion rather than by exclusion of alcohol. This matters because it ties the liver disease to the cardiometabolic risk it travels with and aligns paediatric and adult practice. [4]
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References5ShowHide
  1. [1]Vos MB, Abrams SH, Barlow SE, Caprio S, Daniels SR, Kohli R, et al NASPGHAN Clinical Practice Guideline for the Diagnosis and Treatment of Nonalcoholic Fatty Liver Disease in Children: Recommendations from the Expert Committee on NAFLD (ECON) and the North American Society of Pediatric Gastroenterology, Hepatology and Nutrition (NASPGHAN). J Pediatr Gastroenterol Nutr, 2017.PMID 28107283
  2. [3]Lavine JE, Schwimmer JB, Van Natta ML, Molleston JP, Murray KF, Rosenthal P, et al Effect of vitamin E or metformin for treatment of nonalcoholic fatty liver disease in children and adolescents: the TONIC randomized controlled trial. JAMA, 2011.PMID 21521847
  3. [4]Rinella ME, Lazarus JV, Ratziu V, Francque SM, Sanyal AJ, Kanwal F, et al A multisociety Delphi consensus statement on new fatty liver disease nomenclature. J Hepatol, 2023.PMID 37364790
  4. [6]Newsome PN, Cramb R, Davison SM, Dillon JF, Foulerton M, Godfrey EM, et al Guidelines on the management of abnormal liver blood tests. Gut, 2018.PMID 29122851
  5. [8]Sood V, Alam S, Nagral A, Srivastava A, Deshmukh A, Bavdekar A, et al Practice Recommendations for Metabolic Dysfunction-Associated Steatotic Liver Disease by the Indian Society of Pediatric Gastroenterology, Hepatology and Nutrition (ISPGHAN). Indian Pediatr, 2024.PMID 39297398
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